2006, Vol.39, Issues 6, Cholesteatoma

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Preface

Guest Editor

Even though cholesteatoma is not a new entity in the specialty of otology,

it can present certain management challenges. Recurrence and recidivismcan befuddle even the most experienced surgeon. Subsequently, our spe-

cialty is constantly trying to devise new approaches to increase visualization

and effectively eradicate disease while also maximize hearing and create

a maintenance-free ear. As these nuances in surgical and medical treatment

continue to evolve, it is helpful to pause and review how our current tech-

niques have matured and to share what we have learned with colleagues.

We are fortunate to have the contributions of several prominent surgeons

in our specialty presenting information covering the entire scope of choles-

teatoma from history and pathophysiology to diagnosis and management.The contributors and guest editor are grateful to the Otolaryngologic

Clinics of North America for providing a forum in which these topics can

be presented. I hope that readers find the content both enjoyable and

informative.

Christopher J. Danner, MD

Otology/Neurotology/Skull Base Surgery

Tampa Bay Hearing and Balance Center

Harbourside Medical Tower, Suite 6104 Columbia Drive

Tampa, FL 33606, USA

E-mail address: [email protected]

Christopher J. Danner, MD

0030-6665/06/$ - see front matter 2006 Elsevier Inc. All rights reserved.

doi:10.1016/j.otc.2006.09.003 oto.theclinics.com

Otolaryngol Clin N Am

39 (2006) xi

mailto:[email protected]




Congenital Cholesteatoma: Theories,

Facts, and 53 Patients

Marc Bennett, MD*, Frank Warren, MD,Gary C. Jackson, MD, David Kaylie, MD

The Otology Group, Otolaryngology Head & Neck Surgery,

Vanderbilt University, 300 20th Avenue North,

Suite 502, Nashville, TN 37203, USA

Although the first written report of congenital cholesteatoma (CC) was

over 100 years ago in 1885, its true incidence, etiology, and pathogenesis still

remains a controversial. CC of the temporal bone can be found intradural,

most commonly at the cerebropontine angle, or extradural in the middle ear

or mastoid. Congenital cholesteatoma of the middle ear was first describedby Howard House [1] in 1953. Later, Derlacki and Clemis [2] described six

cases of CC and established the clinical criteria for the diagnosis. These in-

clude a pearly white mass medial to an intact tympanic membrane, a normal

pars tensa and flaccida, and no history of otorrhea, perforation, or previous

otologic procedure. Levenson revised the criteria by adding that previous

bouts of otitis media or effusion should not be exclusion criteria.

Once thought to be relatively rare, CC of the middle ear is now thought

to be on the rise, and accounts for 2% to 5% of all cholesteatomas [3]. There

are multiple theories to the pathophysiology of congenital cholesteatomas of the middle ear. Proposed mechanisms are inclusion, migration, or invasion

of squamous epithelium, epithelial rests from faulty embryogenesis, or meta-

plasia of normal epithelium. Patients with CC may present with a variety of

complaints including conductive hearing loss, but the most common presen-

tation is an asymptomatic white mass medial to an intact tympanic

membrane. Early detection of CC is critical, limiting the size of the retro-

tympanic mass and reducing the risks and complications from surgery.

Treatment remains surgical removal. This study will discuss the diagnosis,

classification, treatment, and theories of pathogenesis, as well as reportthe 53 cases of CC treated at our institution.

* Corresponding author.

E-mail address: [email protected] (M. Bennett).




39 (2006) 1081–1094





Incidence

The true incidence of CC is difficult to determine. Initially thought to be

rare, the incidence seems to be on the rise [4]. The incidence of CC of the mid-

dle ear is estimated to be between 1% to 5% of all cholesteatomas in most

published series [4–6]. Earlier treatment of otitis media and allergies is reduc-

ing the number of acquired cholesteatomas, and consequently increasing the

percentage of CC. There are also many reasons that the number of reported

cases of CC has increased over the last 30 years. Heightened awareness of the

condition by pediatricians and otolaryngologists has lead to earlier diagnosis

and intervention, avoiding the tympanic membrane perforation, which

would eventually occur as the natural progression of the disease [7]. This

would preclude the diagnosis of CC. Routine audiologic screening has also

identified children with conductive hearing losses at early ages. Workup of

these children with better otoscopic equipment may identify masses medial

to the tympanic membrane that previously would have been unseen. In ad-

dition, incidental masses are occasionally seen on CT scans of the temporal

bone ordered in children with a conductive hearing loss.

Presentation and growth

The clinical presentation of any ear mass depends on its size, location,

and histology. Earlier diagnosis decreases the overall size of the CC and re-

duces the likelihood of ossicular erosion. The most common presentation of

a CC is a white retrotympanic middle ear mass [5], although they may be

discovered incidentally during the time of routine otologic evaluation or

during a myringotomy. Improved American health care and preventive

medicine for children has lead to a substantially earlier diagnosis of CC at

an average age of 4 to 5 years old [5,7].

The most common location of CCs is the anterior superior quadrant of the tympanic membrane, followed by the posterior–superior quadrant

[5,7]. Lesions discovered at later ages are more likely to be located in the

posterior mesotympanum, as continued growth of the cholesteatoma is gen-

erally in a posterior direction of spread. The growth follows a natural course

as described by Koltai [8]. Although growth can proceed inferiorly toward

the hypotympanum, it more commonly extends posteriorly along the medial

surface of the ossicles. Once in the posterior superior mesotympanum, the

expansion can involve the incudostapedial joint or stapes superstructure,

but usually spares the footplate. Continued expansion proceeds towardthe facial recess, sinus tympani, and eventually the mastoid air cells. It is

rare for the cholesteatoma to invade the otic capsule bone or labyrinth.

As growth continues, symptoms become more common. Both large ante-

rior and posterior lesions present with conductive hearing loss for different

lesions. Posterior located lesions affect the ossicles much more frequently,

causing a conductive hearing loss from ossicular movement impairment or

1082 BENNETT et al



discontinuity. Anterior lesions may compromise the function of the Eusta-

chian tube and cause a conductive hearing loss through middle ear effusion.

Otalgia and otorrhea are rare, but nearly 50% of patients describe episodesof previous otitis media [9]. If cholesteatoma extends to invade the laby-

rinth, patients may suffer from vertigo or sensorineural hearing loss. Despite

early reports describing a high percentage of facial nerve palsy at presenta-

tion, facial nerve dysfunction is relatively uncommon from middle ear CC

[10]. In fact, facial paresis mandates exclusion of malignancies, metastases,

facial nerve neuromas, and other diagnoses.

Histology

CC or epidermoid cyst is a stratified squamous epithelial lined cyst filled

with keratin debris. Like acquired cholesteatoma, the cyst forms as the result

of progressive desquamation of the epithelium. The congenital form of choles-

teatoma is indistinguishable by histology from the acquired form; therefore, it

is the clinical picture that is important in distinguishing the two entities.

Imaging

Pediatric patients with conductive hearing loss and a normal otomicro-scopic examination require radiologic evaluation to evaluate for the presence

of middle ear anomalies like CC. Because plain radiographs are nonspecific,

high-resolution CT and MRI are the most commonly used imaging modalities

[11]. CT is generally used as the first imaging modality because of its superior

bony definition. CT cannot only confirm the location of a middle ear mass, but

can accurately determine the size of the lesion. CC is usually seen as a hypo-

dense expansile lesion, round to oval in shape, with well-defined margins

that do not enhance with contrast. The lack of enhancement helps distinguish

the cholesteatoma from other lesions like neuromas, glomus tumors, sarco-mas, or meningiomas. Unlike patients with chronic ear disease, the mastoid

air cells are usually well aerated and nonsclerotic [12].

MRI further enhances the evaluation by distinguishing the cholesteatoma

from other middle ear masses such as neuromas, adenomas, schwannomas,

or metastasis. On T1 weighted images, CC appears as a homogenous lesion

that is hypointense to brain, but can also appear isointense [12]. On T2

weighted images, signal intensity is usually high like cerebral spinal fluid

(CSF). There is usually no enhancement with gadolinium. Diffusion

weighted imaging can be used to help distinguish cholesteatoma from othercystic masses as well [11].

Staging

CC of the middle ear is often staged by its location and relationship with

surrounding structures. Derlacki and Clemis [13] are credited with the first

1083CONGENITAL CHOLESTEATOMA



classification system for CC. They classified the lesions into petrous pyra-

mid, mastoid, or tympanic. Recently, two staging systems for CC of the

middle ear have been suggested. The first, by Potsic [14], suggested thefollowing stages:

Stage 1: Single quadrant with no ossicular or mastoid involvement

Stage 2: Multiple quadrants with no ossicular or mastoid involvement

Stage 3: Ossicular involvement but no mastoid involvement

Stage 4: Mastoid extension

In their experience, 40% of the cases were stage I, 14% were stage II,

23% were stage III, and 23% were stage IV. There was a statistically signif-

icant association between the stage, hearing outcomes, and residual disease.Nelson suggested classification of CC into three categories [15]:

Type 1: Mesotympanum with no incus or stapes erosion

Type 2: Mesotympanum or attic with ossicular erosion but no mastoid

extension

Type 3: Mesotympanum with mastoid extension

In their experience, 15% were type I, 59% were type II, and 26% were

type III. Again, recurrence rates were correlated with the clinical stage.

Although no type I patients recurred, 34% and 55% of patients with typeII and III lesions respectively recurred.

The purpose of staging systems should be to (1) aid the clinician in

preoperative planning of treatment, (2) I ndicate prognosis, (3) facilitate

exchange of information between different clinicians, and (4) evaluate the

results of treatment.

Both Potnic’s and Nelson’s staging systems accomplish these goals. The

major difference between systems is that Potnic’s system separates Nelson’s

type I lesions into two categories, depending on whether it occupies more

than one quadrant of the tympanic membrane. Both systems have excellentcorrelation between stage and recurrence.

Surgery

Surgical management of the CC requires complete removal of the matrix

or exteriorization to prevent recurrence. The goal of surgery is complete

removal of disease with optimal hearing outcomes. For complete removal,

intraoperative dissection must be complete around the matrix, oftenmandating a mastoidectomy for better visualization.

As expected, the majority of patients with isolated anterior mesotympa-

num lesions can be adequately approached through a standard tympano-

plasty. Posterior lesions involving the ossicles often require a mastoidectomy

for circumferential visualization of the cholesteatoma. Most surgeons advo-

cate an intact canal wall mastoidectomy because it allows for better hearing

1084 BENNETT et al



reconstruction and eliminates both water restrictions and the periodic

debridements that can be necessary after a canal wall down procedure [16].

However, a canal wall down mastoidectomy is performed if the patienthad [17]:

(1) unreconstructible external auditory canal defects

(2) labyrinthine fistulas

(3) poor health

(4) poor compliance

In CC patients, it is rarely indicated as the patients are usually in good

health, the cholesteatoma by definition cannot erode into the external audi-

tory canal, and the lesions rarely affect the otic capsule bone. Middle earreconstruction is used if ossicles are eroded or removed to allow complete

excision of the lesion. Cholesteatoma matrix is frequently found close to

the facial nerve, but rarely is mobilization of the nerve needed for complete

excision of matrix. Middle cranial fossa approaches may be required for

perigeniculate lesions [18].

There is some controversy over whether there should be a planned second

look in CC. Limited anterior CC not involving the ossicles has an extremely

low rate of residual disease and, therefore, a single stage is frequently suffi-

cient. However, if there is any concern about residual disease, a ‘‘secondlook’’ is certainly warranted. For more extensive lesions the indications

for a second-look procedure are less clear and must be individualized for

each patient. Recurrence in a canal wall down mastoidectomy is fairly con-

spicuous, and therefore serial exams are all that is necessary. Long-term fol-

low-up is important for all patients. A routine second look is no longer the

general policy at our institution. Patients are reexplored for worsening hear-

ing or obvious recurrence.

Pathophysiology

The accepted cause of CC remains controversial. The competing theories

of pathogenesis fall into four categories: implantation, invagination or inva-

sion, metaplasia, and epithelial rest formation.

Epithelial rest

The most commonly accepted and quoted theory on the origin of CC is

the epithelial rest theory. This theory is based on Teed’s initial observationof an epidermal structure found in a 5-month human fetus in ‘‘the dorsal

lateral pole of the tympanum, just medial to the neck of the malleus’’ [19].

Paparella [20] explained these rests as ectodermal implants in the fusion

plates between the first and second branchial arches that appear around

10 weeks at the junction of the first branchial cleft and pouch systems in

the anterior mesotympanum near the geniculate ganglion. The structure is




distinct from surrounding tissue, and is located at the transition from simple

cuboidal epithelium of the tympanic cavity to the pseudostratified epithe-

lium of the anterior mesotympanum and Eustachian tube [21]. The exactfunction of the rests is unknown, but Levenson [21] postulated that it aided

in middle ear and tympanic membrane development. Initially dormant, it

undergoes rapid proliferation before resorption around 33 weeks’ gestation.

CC is thought to form if resorption is incomplete. Levenson [21] proposed

that the epidermal rests fail to undergo involution because of chronic

irritation.

Michaels [22] histologically confirmed these rests of epithelial cells in

54% of the fetal temporal bones examined and claimed their persistence

led to CCs.McGill [23] further confirmed their presence, but suggested that interme-

diate forms of the epithelial rests between 33 weeks and early childhood

must be found. To date, there is no documentation of epithelial rests beyond

33 weeks of gestation up to birth.

Although the middle ear epidermoid provides a satisfactory source for

the CC, it does not explain the existence of CCs found outside the anterior

superior quadrant of the tympanic membrane where these rests are tradi-

tionally found. However, recent studies have found that the locations of ep-

ithelial rests vary. Although most are found in the anterosuperior annularregion, they can also be in seen posterosuperior, posteroinferior, and ante-

roinferior regions of the lateral wall of the tympanic cavity.

Invaginaton

Aimi was the first to suggest migration of normal squamous epithelium

from the external canal through the tympanic ring and into the middle

ear as the source of CC. His theory holds that small inflammatory injury

of the tympanic membrane near the neck of the malleus causes invaginationof the epithelium that progresses to form a CC. This event may occur in

utero or during childhood development. The retracted tympanic membrane

is adherent to the malleus or incus. The eardrum is loosed from the ossicles

and torn, leaving a small remnant of keratinized epithelium adherent to the

bone along with or without a small perforation. The perforation heals but

the retained epithelium forms a cholesteatoma over time. Alternatively,

Reudi [24] postulated that external auditory canal ectoderm may penetrate

the tympanic membrane in utero and migrate into the middle ear. Small in-

flammatory injuries to the tympanic membrane produce small perforationsin the epithelium through which squamous epithelium invades into the mid-

dle ear.

Although there is no histopathologic evidence of this theory, it provides

a plausible hypothesis for CC. It would help explain lesions not located in

the anterior–superior quadrant of the tympanic membrane as the invagina-

tion could occur anywhere in the tympanic membrane.

1086 BENNETT et al



Implantation

Friedberg and Sheehy were the first to suggest that CC was the result of

implantation of squamous epithelium to the middle ear either from trauma

or an unrecognized and subsequently healed tympanic membrane perfora-

tion. This may account for some cases of cholesteatoma that appear to

meet the criteria to be classified as CC. This theory also helps explain

why lesions can be isolated in many different sites. However, because this

theory requires an insult to the tympanic membrane, it by definition

excludes the classification of CC.

Metaplasia

Squamous metaplasia of the inflamed middle ear epithelium is not un-

common, and can occasionally be seen even in nonpathologic ears [25]. Re-

cently, Sade [25] has found squamous metaplasia in cases of chronic otitis

media. In addition, other studies have shown that retinoic acid depletion

can induce squamous differentiation of middle ear epithelium in cultures

[26]. If metaplastic squamous epithelium becomes keratinizing, a cholestea-

toma may form from the accumulation of keratin. Serial sections of middle

ear mucosa have revealed keratin production in cells not connected to sur-

face epithelium. However, Friedberg noted the location of metaplasia

would be random and not explain the high frequency of lesions in the

anterior superior tympanic cavity; however, this area near the eustacian

tube may be more prone to inflammation and therefore have a higher

rate of CC.

Current series

Material and methods

Chart review

Between March 1971 and December 2003, over 3000 chronic ear surgeries

were performed at our institution. A computerized otologic database was

used to identify 53 patients who had a history of CC. Cholesteatoma was

considered to be congenital if there was no history of otologic surgery,

otorrhea, or perforation, and no tympanic membrane abnormality on

examination.

Charts of these patients were reviewed, and patients were classified ac-cording to the following data: age of patient, location and extent of choles-

teatoma, type of surgery performed, audiologic outcomes, development of

recurrent perforation or cholesteatoma, and intraoperative complications.

Disease was defined by size as occupying the middle ear, Eustachian tube, ep-

itympanum, ossicles, or mastoid. Recurrence was defined visible disease or

cholesteatoma visualization at subsequent surgical procedures or clinic visits.




Demographics

Ninety patients were identified in the database with the diagnosis of CC.

Unfortunately, 37 had previous procedures performed elsewhere before be-ing evaluated at our institution. They were excluded because it could not be

determined conclusively whether they had a CC. Fig. 1 shows the number of

patients in each pediatric age group. The average age of presentation was

4.7 3.1 years old. There were 22 female patients and 31 males. Thirty-two

were left ears and 21 right ears. No patient had bilateral CC. In our series,

CC was 2.48% of all primary cholesteatomas, 1.80% of all cholesteatoma

surgeries, and 8.31% of cholesteatomas in patients less than 18 years old.

Disease locationTable 1 lists the general location of our patients. Twenty-nine patients

had disease limited to the middle ear or epitympanum without mastoid in-

volvement. Twenty-four patients had cholesteatoma extension into the mas-

toid, while 12 patients had disease extension into the Eustachian tube.

Twelve patients had ossicular erosion requiring reconstruction. Only two

of these patients had disease extension into the mastoid. We classified our

patients according to Nelson’s staging and found 17 patients with type I dis-

ease, 12 patients with type II, and 24 patients with type III.

Surgeries performed

Surgeries were directed at removing cholesteatoma and optimizing hear-

ing for all patients. Table 2 lists the procedures performed and the relative

percentages. Twenty-four patients with extension into the mastoid under-

went a complete mastoidectomy. In addition, 23 patients with posterior

mesotympanic disease with inadequate transcanal exposure underwent

0

2

4

6

8

10

12

14

1 2 3 4 5 6 7 8 9 10 11 >12

Age

N u m b e r o f P a t i e n t s

Series1

Fig. 1. Age at presentation.

1088 BENNETT et al



mastoidectomy procedures for better visualization. Twelve patients had dis-

ease involving the middle ear ossicles and requiring reconstruction. Seven

patients had erosion of the incus that required partial ossicular chain recon-

struction prosthesis (PORP) with cartilage overlay. Two of these patients

each underwent partial ossicular chain prosthesis reconstruction with

a Black PORP, hydroxyapatite PORP, or Kurz prosthesis. One patienthad an incus interposition. Five patients had eroded stapes superstructures

requiring total ossicular chain reconstruction prosthesis (TORP) with carti-

lage overlay. Two patients underwent successful revision surgery for tym-

panic membrane perforation.

Audiologic data

Audiologic data was analyzed for the patients using preoperative and

postoperative, and long-term (O1 year) pure-tone average air–bone gaps

(PTA-ABG) obtained from four frequencies (500, 1000, 2000, and 3000Hz) according to AAO-HNS guidelines and listed in Table 3. Hearing re-

sults were also described by the stage of the cholesteatoma. For all patients,

regardless of the stage or surgery type, the average preoperative, postoper-

ative, and last PTA-ABG were compared. The results showed the difference

Table 1

Location of cholesteatoma

Site of cholesteatomaNelsonstage

Number of patients Percentage

Middle ear and eustacian tube I 0 0.0

Eptiympanum only I 0 0.0

Tympanic membrane I 1 1.9

Mastoid only I 2 3.8

Middle ear, epitympanum, eustacian tube I 2 3.8

Middle ear and epitympanum I 4 7.5

Middle ear only I 8 17.0

Middle ear and epitympanum with ossicular invt II 4 7.5

Middle ear with ossicular involvement II 8 17.0

Mastoid, middle ear, epitympanum, eust. tube III 14 18.9

Mastoid, middle ear, epitympanum III 10 22.6

Table 2

Procedures performed

Procedures performed Number of patients Percentage

Tympanoplasty no OCR 1 1.9

Tympanoplasty with OCR 1 1.9

Tympanoplasty with mastoidectomy and OCR 11 20.8

Tympanoplasty with mastoidectomy no OCR 36 67.9

Tympanoplasty with modified radical mastoidectomy 4 7.5

Abbreviation: OCR, ossicular chain reconstruction.




between preoperative and both postoperative and last PTA-ABG was statis-tically worse using the standard paired t-test (P ¼ 0.001). The hearing results

of patients undergoing an ossicular chain reconstruction were also analyzed.

There was no significant difference between preoperative, postoperative, and

last PTA-ABG using the standard paired t-test (P ¼ 1.1). For patients with

intact ossicular chains, the postoperative and last PTA-ABG were statisti-

cally better than preoperative PTA-ABG using the standard paired t-test

(P ¼ 0.001). Twenty-one patients did not have long-term follow-up or serial

audiograms. The hearing results by stages is shown in Table 3. Patients with

stage 3 disease had worse hearing than stages 1 or 2.

Surgical findings

Thirteen patients had intraoperative findings listed in Table 4. Although

eight patients had evidence of facial nerve exposure, no patients had injuries

to the facial nerve. In addition, facial nerve exposure was not related to dis-

ease extent. Three patients with mastoid extension of cholesteatoma had du-

ral exposure from the cholesteatoma, but no CSF leaks were encountered.

There was one case of oval window fistula, but no other labyrinthine fistu-

las. There was one dehiscent carotid artery seen intraoperatively withoutcomplication.

Table 3

Hearing outcomes

Preoperative Postoperative Most recentAll patients 24.8 13.4 30.3 15.5 31.6 16.3

No OCR 24.0 14.0 30.5 16.1 31.0 17.4

Stage I 16.6 12.9 25.0 12.7 27.2 16.0

Stage II 30.6 15.6 27.1 16.0 35.9 12.3

Stage III 32.8 12.8 32.6 15.3 35.1 17.0

Abbreviation: OCR, ossicular chain reconstruction.

Table 4

Intraoperative complications

Intraoperative complications Number of patients Percentage

Facial nerve injury 0 0.0

Dural exposure 3 5.7

Oval window fistula 1 1.9

Facial nerve exposure 8 13.2

Semicircular canal fistula 0 0.0

Dehiscent carotid artery 1 1.9

Death 0 0.0

CSF Leak 0 0.0

Fixed footplate 0 0.0

Abbreviation: CSF, cerebral spinal fluid.

1090 BENNETT et al



Long-term complications

Only 6 of the 53 patients had long-term complications from the CC sur-

gery as listed in Table 5. Four patients had tympanic membrane perforationin the operated ear. Two elected to undergo revision surgery and were suc-

cessfully closed. One patient had delayed facial paresis (House-Brackmann

VI), which resolved with steroids. One patient had a prosthesis extrusion

with healed tympanic membrane. No patients had recurrence of their

cholesteatoma.

Discussion

Although considerable controversy exists over the existence of CC, thereexists a set of pediatric patients with normal eustachian tube function and

normal tympanic membranes with a retrotympanic cholesteatoma. Congen-

ital and acquired cholesteatomas share many similarities, but key differences

in the pathophysiology and location of these lesions mandate separate treat-

ment protocols.

Preoperative workup includes a thorough examination and audiogram.

Because these lesions develop behind a normal appearing tympanic mem-

brane, the physical examination may be completely normal or reveal a retro-

tympanic white mass. Audiologic evaluation usually reveals a conductivehearing loss. Because CT shows the size and location of the CC, it is often

helpful in determining the extent of disease and surgical intervention that

will be required; however, it is not absolutely necessary in the evaluation

of patients with a clearly visible retrotympanic mass. MRI adds little to

the evaluation of the middle ear CC unless the diagnosis is unclear.

Unlike acquired cholesteatomas, which develop from posterior tympanic

membrane retraction pockets, most CCs start in the anterior mesotympa-

num. This anterior location often allows for cholesteatoma dissection

Table 5

Postoperative complications

Postoperative complications Number of patients Percentage

Recurrent cholesteatoma 0 0.0

Recurrent perforation 4 7.5

Prosthesis extrusion 1 1.9

Otorrhea 1 1.9

Delayed facial paresis 1 1.9

Mastocutaneous fistula 0 0.0

Granuloma 0 0.0

Meningitis 0 0.0

Abscess 0 0.0

Profound hearing loss 0 0.0

Perichondritis 0 0.0

Hematoma 0 0.0

Wound infection 0 0.0




without disarticulation of the incudostapedial joint, therefore, improving

chances of postoperative hearing results. We start each case via a postauric-

ular tympanoplasty approach. Lesions found to extend into the ossicularmass can usually be approached using this approach. Increased exposure

can be created with gentle curetting of the anterior medial scutum. If poste-

rior exposure is still inadequate, an intact canal wall mastoidectomy can be

performed. The little additional morbidity and improved exposure of an

intact canal wall mastoidectomy is favorable to a transcanal atticotomy.

McGrew [27] noted no increase in postoperative complications of facial pa-

resis, CSF leak, or meningitis in patients undergoing a tympanoplasty with

mastoidectomy as opposed to those undergoing a tympanoplasty alone. In

addition, our experience is that the need for canal wall down procedures isrelatively rare and should only be performed if there is a labyrinthine fistula

or children with either poor health or likely to have poor follow-up. When

performed, the basic principles of a wide meatoplasty, low facial ridge, and

complete eradication of mastoid air cells apply [28]. Often, these cavities

become self-cleaning and do not require life-long care.

Like other chronic ear disease, surgery for CC is relatively safe. The ideal

timing of surgery remains unclear. Early detection and surgical intervention

reduces the risk of ossicular involvement and improves hearing outcomes.

Although there is no lower age limit to chronic ear surgery, generally thechildren should be at least 10- to 12-months-old for both anesthetic and sur-

gical consideration. At this age, the external auditory canal and middle ear

cleft are large enough to accommodate surgery and the risk of anesthesia is

minimal. The two most likely unexpected intraoperative findings are dural

exposure and facial nerve exposure. Unplanned facial nerve injury is a dev-

astating complication in these young patients. Care must be exercised when

dissecting around the facial nerve to ensure there is a healthy bony covering.

Although Hough observed nearly 30% of patients with CC had a dehiscence

in the tympanic segment of the facial nerve, our rates were much lower.The pathophysiology of CCs does not involve Eustacian tube dysfunc-

tion; therefore, recurrence rates should be lower than acquired forms of

the disease. In fact, although typical rates of recurrence for cholesteatomas

are around 10% to 40% for children, we did not experience a recurrence in

the 32 patients with long-term follow-up. Because of low recurrence rates,

we do not routinely stage these patients and only reexplore these patients

for obvious recurrence, worsening or poor hearing.

There are several explanations for why the hearing results do not appear

more favorable. Over half of the patients had air–bone gaps less than 20 dBPTA preoperatively. If preoperative hearing is not that bad, it is hard to

make significant improvement postoperatively. Patients with stage II or

III disease had slight improvements in their hearing after surgery. This

also underscores the fact that it is more likely to improve hearing in a patient

that has poorer hearing preoperatively. The patients with stage 1 disease had

significantly better hearing than those with stage II or III disease. There are

1092 BENNETT et al



inherent difficulties with serial audiograms in children, especially those un-

der 4 or 5 years old. They are prone to inconsistencies in their audiograms,

which may account for some inaccuracies. A significant difference could alsobe accounted for by lack of follow-up. As a tertiary otology referral center,

nearly half of 53 patients sought follow-up care with referring physicians. It

is likely that these patients had better outcomes or revision surgery or fol-

low-up would have been arranged. In addition, our local patients without

complaints and good hearing often skip follow-up appointments, and there-

fore would not have postoperative audiograms.

Further readings

Doyle K, Luxford W. Congenital aural cholesteatoma: results of surgery in 60 cases. Laryngo-

scope 1995;105:263–7.

Eavey RD. Abnormalities of the neonatal ear: otoscopic observations, histologic observations,

and a model for contamination of the middle ear by cellular contents of amniotic fluid.

Laryngoscope 1993;103(1 Pt 2 Suppl 58):1–31.

Grundfast KM, et al. The inferiorly based superior tympanomeatal flap for removal of congenital

cholesteatoma. Laryngoscope 1990;100:1341–3.

Karmody C, et al. The origin of congenital cholesteatoma. Am J Otolaryngol 1998;19:292–7.

Nelson M, et al. Congenital cholesteatoma. Arch Otolaryngol Head Neck Surg 2002;128:810–4.

Piza J, et al. Meconium contamination fo the neonatal ear. J Pediatr 1989;115:910–4.

Robert Y. Congenital cholesteatoma of the temporal bone: MR findings and comparison withCT. Am J Neurorad 1995;19:755–61.

Selesnick SH, Lynn-Macrae AG. The incidence of facial nerve dehisence at surgery for cholestea-

toma. Otolaryngol Neurotol 2001;22(2):129–32.

Thakkar K, et al. Congenital cholesteatoma Isolated to the mastoid. Otolaryngol Neurotol 2006;

27:282–3.

Tos M. A new pathogenesis of mesotympanic cholesteatoma. Laryngoscope 2000;110:1890–7.

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[1] House HP. An apparent primary cholesteatoma. A case report. Laryngoscope 1953;63(3):

712–3.

[2] Derlacki EL, Clemis JD. Congenital cholesteatoma of the middle ear and mastoid. Ann Otol

Rhinol Laryngol 1965;74(3):706–27.

[3] Paparella M, Rybak L. Congenital cholesteatoma. Otolaryngol Clin North Am 1978;11:

113–20.

[4] Kazahaya K, Potsic WP. Congenital cholesteatoma. Curr Opin Otol Head Neck Surg 2004;

12:398–403.

[5] Darrouzet V, et al. Congenital middle ear cholesteatomas in children: our experience in 34

cases. Otolaryngol Head Neck Surg 2002;126:34–9.[6] House JW, Sheehy JL. Cholesteatoma with intact tympanic membrane: a report of 41 cases.

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[7] Potsic W, et al. CongenitalcCholesteatoma: 20 years experience at the Children’s Hospital of

Philadelphia. Otolaryngol Head Neck Surg 2002;126:409–13.

[8] Koltai PJ, et al. The natural history of congenital cholesteatoma. Arch Otolarygol Head

Neck Surg 2002;128:804–9.

[9] Friedberg J. Congenital cholesteatoma. Laryngoscope 1994;104:1–23.




[10] Cannoni M. Congenital cholesteatoma of the petrous bone. Rev Laryngol Otol Rhinol

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[11] Jackler RK, Parker DA. Radiographic differential diagnosis of petrous apex lesions. Am J

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[12] Peron DL, Schuknecht HF. Congenital cholesteatoma with other anomalies. Arch Otolar-

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[13] Derlacki EL, Harrison WH, Clemis JD. Congenital cholesteatoma of the middle ear and

mastoid. A 2nd report presenting 7 additional cases. Laryngoscope 1968;78:1050–78.

[14] Potsic WP, et al. A staging system for congenital cholesteatoma. Arch Otolaryngol Head

Neck Surg 2002;128:1009–12.

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[16] Dodson EE, Hashisaki GT, et al. Intact canal wall mastoidectomy with tympanoplasty for

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[17] Jackson CG, Glasscock ME, Nissen AJ. Open mastoid procedures: contemporary indica-

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[19] Teed RW. Cholesteatoma verum tympani: its relationship to the first epibrachial placode.

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[20] Paparella MM. Congenital cholesteatoma. Otolaryngol Clin North Am 1978;11:113–20.

[21] Levenson MJ, et al. Congenital cholesteatoma in children. Laryngoscope 1988;98:949–55.

[22] Michaels L. Origin of congenital cholesteatoma from a normally occurring epidermoid rest

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[23] Liang J, et al. Immunohistochemical characterization of the epidermoid formation in themiddle ear. Laryngoscope 2003;113:1007–14.

[24] Ruedi L. Cholesteatoma formation in the middle ear in animal experiments. Acta Otolaryngol

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[25] Sade J, BaBiacki A, Pinkus G. The metaplastic and congenital origin of cholesteatoma. Acta


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[28] Jackson CG, et al. A surgical solution for the difficult chronic ear. Am J Otolaryngol 1996;

17(1):7–14.

1094 BENNETT et al



Indications and Technique

in Mastoidectomy

Marc Bennett, MD*, Frank Warren, MD,David Haynes, MD

The Otology Group, Otolaryngology Head & Neck Surgery, Vanderbilt University,

300 20th Avenue North, Suite 502, Nashville, TN 37203, USA

Approximately 350 years have passed since the first published report of

a mastoidectomy by Riolan the Younger. Many changes have occurred

over the subsequent years, especially since the advent of the operating

microscope 50 years ago. This report focuses on mastoid surgery as it relates

to chronic ear disease as well as providing access for a variety of other sur-

gical procedures. We reflect on the current status and indications of theprocedure as well as common complications.

History

Chronic and suppurative infections of the mastoid have been described as

long ago as ancient Greece. However, it was not until mid 17th century

when Riolan the Younger described the first trephination procedure of

the mastoid. The subsequent 200 years did not produce many significant ad-vances until Fielitz and Petit reported multiple cases of mastoid trephina-

tions for acute abscesses in the late 18th century. These procedures fell

out of favor for more than 100 years until Schwartze and Eysell [1] popular-

ized the cortical mastoidectomy in 1873. It was effective for draining acute

infections; however, it did little to treat chronic infections of the ear. In

1890, Zaufal [2] described the first radical mastoidectomy removing the

superior and posterior ear canal, tympanic membrane, and ossicles in an at-

tempt to eliminate infection, externalize disease, and create a dry ear. Bondy

revised the technique by leaving the uninvolved middle ear alone and exte-riorizing the epitympanum [3].


E-mail address: [email protected] (M. Bennett).




39 (2006) 1095–1113





The introduction of the Zeiss otologic operating scope in 1953 made precise

dissection possible. Soon thereafter, Wullstein described the first attempts at

reconstruction of the tympanic membrane via tympanoplasty [4]. Five yearslater, William House introduced intact canal wall mastoidectomy [5]. Since

then, there have been multiple variations of the mastoidectomy described.

Indications

The goals of any chronic ear surgery are to create a dry, safe ear and pre-

serve or restore hearing as much as possible. Although there are some abso-

lute and relative indications for a mastoidectomy, the type of mastoidectomy

is based on the extent of disease, preoperative health of the patient, the statusof the opposite ear, and both the surgeon’s and patient’s preference. For

chronic ear surgery, a mastoidectomy is performed to help eradicate disease

and gain access to the antrum, attic, or middle ear. It also increases the air-

containing space in continuity with the middle ear, allowing the middle ear

to better accommodate changes in pressure without tympanic membrane re-

traction. Absolute indications include cholesteatomas or tumors with exten-

sion into the mastoid bone. Relative indications include [6]:

History of profuse otorrhea

Previous tympanoplasty failure

Secondary acquired cholesteatoma

Tympanic membrane perforations no correctable without the further ex-

posure provided by a mastoidectomy

Although surgeons remain divided on the utility of the mastoidectomy in

primary cholesteatoma surgery and tympanic membrane perforation re-

pairs, most agree to its utility in revision cases after graft failure. Generally,

imaging and cholesteatoma size are not a determinate of what procedure is

performed.

Simple mastoidectomy

A simple or cortical mastoidectomy involves removing the mastoid cortex

and some of the underlying air cells. Dissection may be superficial or pro-

ceed to the mastoid antrum. It is used to unroof the mastoid cortex and

drain a coalescent mastoiditis with subperiosteal abscess.

Intact canal wall or complete mastoidectomy

The canal wall up mastoidectomy involves removing the mastoid air cells

lateral to the facial nerve and otic capsule bone while preserving the poste-

rior and superior external auditory canal walls. This technique affords

access to the epitympanum while maintaining the natural barrier between

the external auditory canal and mastoid cavity. In pediatric patients, this ap-

proach is preferred generally to avoid the long-term problems associated

1096 BENNETT et al



with canal wall down procedures. This approach can be combined with a fa-

cial recess dissection for:

Removal of disease in the recess

Better exposure of the posterior mesotympanum around the oval and

round windows

Better visualization of the tympanic segment of the facial nerve

Better middle ear aeration postoperatively

For increased exposure, the facial recess can be extended inferiorly or su-

periorly to gain complete access to the hypotympanum and epitympanum. If

cholesteatoma or tumor cannot be resected via this approach, the surgery

needs to be converted to a canal wall down procedure. Occasionally, a mas-toidectomy may be used to identify and repair an injured facial nerve.

Modified radical mastoidectomy

Although the classic description of a modified radical mastoidectomy is

the atticotomy described by Bondy, most surgeons currently use the term

to describe a canal wall down mastoidectomy with tympanic membrane

grafting. There are both preoperative and intraoperative indications to re-

move the auditory canal. Preoperative indications for a modified radical

mastoidectomy include [5] (1) disease in an only hearing ear, (2) patients

with poor general health making them an anesthetic risk, and (3) patients

in whom follow-up is problematic.

Some surgeons advocate a canal wall down after multiple failed attempts

at canal wall intact surgery [7]. The decision to remove the canal wall is

made intraoperatively when one of the following is encountered [8]: (1) un-

reconstructible posterior external auditory canal defect, (2) labyrinthine

fistula where the matrix cannot be resected primarily, and (3) obstructing

low-lying middle fossa dura limiting epitympanic access. Again, cholestea-

toma size is not a determining factor.

Radical mastoidectomy

A radical mastoidectomy is performed in patients with severe eustachian

tube dysfunction, irreversible middle ear disease, or unresectable cholestea-

toma or tumors. The procedure leaves middle ear and mastoid air cells ex-

teriorized as a single cavity with no attempt at reconstruction. The

eustachian tube is occluded and both the malleus and incus are removed. Be-

cause the middle ear is not reconstructed, the expectation is that surround-ing squamous epithelium will overgrow the middle ear and mastoid cavity.

Mastoid obliteration

Mastoid obliteration involves overclosing the external auditory canal in

blind sac fashion and obliterating the cavity with autologous bone, bone

1097MASTOIDECTOMY



pate, vascularized flaps, or abdominal fat. It is used in advanced cases in

which the ear continues to drain despite multiple prior attempts at canal

wall down surgery. Obliteration may also be indicated in cases of chronicsuppurative otitis media in which there is extensive dural dehiscence with

or without cerebrospinal fluid leakage.

Canal wall up versus down

The controversy over canal wall up versus down surgery has been on-

going for nearly half a century. Although there are multiple indications

to remove the canal wall, the decision is usually individualized. Most sur-

geons prefer to avoid a cavity if possible. The primary advantage of a ca-

nal wall down procedure is increased visibility and access to the

mesotympanum and epitympanum, which allows disease resection and re-

construction to be accomplished in a single stage. This increased exposure

accounts for reduced rates of recurrences versus intact canal wall proce-

dures [9]. However, postoperative care is more intense in the canal wall

down surgery both in the immediate postoperative period and long-

term. Serial debridements of the cavity and frequently antibiotic drop

irrigation are often required. In contrast, the intact canal wall mastoidec-

tomy maintains the natural anatomy and heals more quickly than the

modified radical mastoidectomy. Canal wall intact procedures do not re-

quire regular debridements, and hearing outcomes tend to be slightly im-

proved over canal wall down procedures [10]. However, poorer

intraoperative exposure and the recreation of a middle ear space increase

the potential for recurrent or residual disease after intact canal wall

procedures.

Preoperative evaluation

Preoperative planning includes a comprehensive head and neck exami-

nation with an otomicroscopic examination. Active infections are treated

aggressively with topical antibiotic drops before surgery. Bilateral full au-

diometric evaluation is performed in all cases. Although computed tomog-

raphy scans can help delineate the bony anatomy of the temporal bone,

this evaluation is not necessary in most patients. They are especially useful

in revision surgery and in patients with symptoms consistent with a labyrin-thine fistula. All patients should be encouraged to stop smoking because it

increases recurrence rates over nonsmokers [9]. Sinonasal disease is treated

aggressively. Adult patients with significant symptoms are tested and

treated for seasonal allergies. In children, preoperative adenoid assessment

may be necessary and when appropriate, adenoidectomy should be per-

formed 1 month before ear surgery.

1098 BENNETT et al



Preparation

Anesthesia is given without paralytic agents. Facial nerve bipolar elec-

trodes are placed into the orbicularis oculi and oris muscles for monitoring

of the facial nerve throughout the case. The tragus and the area just behind

the postauricular sulcus are injected with 2% lidocaine with 1:100,000 epi-

nephrine about 10 minutes before the start of the case to allow proper he-

mostasis. The periauricular hair is cleansed with a hibiclens shampoo and

the patient’s ear is prepped and draped in the usual sterile fashion. Antibi-

otics are routinely given preoperatively to reduce infection risks [11]. Ste-

roids are also often used to reduce postoperative nausea.

Surgical incisions

Canal incisions

Each case starts with a detailed examination of the tympanic membrane.

With the exception of cochlear implantation, temporal bone resection, and

skull base procedures, transcanal injection of the posterior ear canal with

2% lidocaine and 1:50,000 epinephrine is performed. The ear is copiously

irrigated with saline solution impregnated with antibiotic and desquamateddebris in the external auditory canal is removed. Fig. 1 shows the vascular

strip incisions. A radial incision is made in the tympanomastoid and then

tympanosquamous suture lines. The dependent or inferior cut is always per-

formed first to avoid blood obscuring future incisions. These incisions are

then connected by a medial incision approximately 1 to 2 mm lateral to

the annulus. Just medial to the bony-cartilaginous junction, a radial incision

is made from the tympanomastoid suture line to the inferior aspect of the

external auditory canal.

Fig. 1. Vascular strip incisions. (A) tympanomastoid suture line, (B) tympanosquamous suture

line, (C ) medial incision, (D) radial incision.

1099MASTOIDECTOMY



Mastoid incisions

The standard postauricular incision and the endaural incision are the two

basic incisions for access to the mastoid.

Postauricular incision

A postauricular incision as shown in Fig. 2 is the method most widely used

to gain access to the mastoid. The incision spans from the helical rim to the

mastoid tip and is well hidden in the postauricular region. It rarely causes any

visible scarring. The incision should be about 1 cm behind the postauricular

crease to avoid unsightly deepening of the sulcus, which can occur when in-

cisions are placed directly in the crease. The incision is more posterior inyoung children to avoid a superficial facial nerve near the mastoid tip.

The incision is made through the skin with a scalpel. An avascular plane

is elevated anteriorly toward the external auditory canal just below the sub-

dermal fat, leaving a layer of loose areolar tissue on the temporalis fascia.

This plane is developed down to the mastoid tip. The attachments of the

sternocleidomastoid muscle can be separated from the mastoid tip for in-

creased exposure during skull base cases, but usually these attachments

are left intact to reduce postoperative discomfort.

A self retaining retractor is spread over the temporalis muscle. As shown inFig. 3, a large graft is harvested with a scalpel and scissors. This tissue often is

scarce in revision surgery and if not present, a true temporalis fascial graft can

be harvested. For proper healing, this graft must be thinned of all muscle and

fat attachments. If the temporalis fascia is unavailable, tragal perichondrium

or periosteum medial to the temporalis muscle may be harvested for grafting.

Autologous veins or alloderm may also be used for grafting in rare cases [12].

As shown by the dashed lines in Fig. 3, ‘‘T’’ shaped incisions are then

made through the mastoid periosteum with electrocautery. The horizontal

incision is made just below the temporalis muscle in the linea temporalis.A second incision is made perpendicular to the first in the middle of the

Fig. 2. Postauricular incision.

1100 BENNETT et al



mastoid extending from the temporalis muscle to the mastoid tip. A Lem-

pert elevator is then used to elevate the periosteum posteriorly over the sig-

moid sinus, superiorly over the tegmen, and anteriorly to the suprameatal

spine of Henle where the vascular strip is identified and reflected laterally.

Two self retaining retractors are then placed in orthogonal directions as

shown in Fig. 4. In revision surgery, careful palpation of the underlying

bone will often identify a potentially unprotected sigmoid sinus or dura.The incision is also modified to a ‘‘C’’ shaped incision at the posterior aspect

of the previously dissected mastoid cavity. In younger children, elevation in-

ferior to the external auditory canal can potentially injure a lateralized facial

nerve near the stylomastoid foramen.

Lempert incision

Endaural incisions have been used for more than 100 years. Lempert pop-

ularized this approach in the mid 1930s. An incision is made down to the

Fig. 3. Fascial graft harvest and periosteal incisions.

Fig. 4. Mastoid surface anatomy.

1101MASTOIDECTOMY



mastoid bone in the lateral external auditory canal between the tragus and

the helical crus. Because exposure can only be obtained of the anterior su-

perior part of the mastoid, these incisions have fallen out of favor for mas-toidectomies; however, some surgeons continue to use these incisions to

facilitate exposure of the middle ear in transcanal surgery.

Techniques

Basics

All drilling is done under the microscope with binocular vision. Constant

irrigation is critical to prevent thermal damage from the drill bits. A varietyof surgical drills exist for mastoidectomy, but a high-speed, comfortable,

and reliable drill system is crucial. In the past, air-powered systems were

the norm, but recently the development of high-speed electrical systems offer

easier setup, efficiency, and less noise than the air powered systems.

A variety of burs exist, ranging from those that aggressively remove bone

to those used for fine polishing of structures like the facial nerve. Larger bits

are always preferred as they offer better control and easier removal of bone;

however, drill bits should not be so large as to obstruct visualization during

the dissection. Initially, cutting burs are used to removed bone and identifyimportant landmarks. Diamond burrs are then used for more delicate pro-

cedures like removing the last layer of bone over sigmoid sinus or facial

nerve. As dissection continues, smaller burrs will be required as space

becomes limited. Periodic irrigation of the surgical field with saline solution

reduces bleeding and washes squamous debris from wound.

Surface anatomy

An understanding of the temporal bone anatomy is important to avoid

injuring vital structures. The surface landmarks of the mastoid bone shownin Fig. 4 not only define the boundaries of the mastoid bone, but approxi-

mate important deep structures. The spine of Henle is the anterior extent

of dissection. This protuberance extends superficially from the posterior su-

perior bony ear canal and approximates the location of the underlying mas-

toid antrum. Superiorly, the linea temporalis, the inferior border of the

temporalis muscle, approximates the lowest level of the tegmen or floor of

the middle fossa. The mastoid tip is the inferior limit of dissection.

Complete mastoidectomy

The key to a safe dissection is identifying key structures. Identifying the

tegmen, external auditory canal, sigmoid sinus, middle ear ossicles, and

facial nerve is the easiest and safest way to ensure their preservation.

As indicated by Fig. 5, dissection starts high in the mastoid cortex, re-

moving bone along the linea temporalis until a thin layer of tegmen bone

1102 BENNETT et al



is left over the middle fossa dura, remembering that tegmen height is vari-

able depending on mastoid pneumatization. Next, a cut perpendicular to

the first and tangential to the external auditory canal is made from the zy-gomatic root to the mastoid tip. Finally, a cut is made from the mastoid tip

to the sinodural angle. Dissection is continued along these three planes, sau-

cerizing the lateral surface of the temporal bone from the middle fossa teg-

men to the mastoid tip and from the ear canal to the sigmoid, keeping the

deepest part of the dissection in the anterior superior mastoid directly

over the mastoid antrum. There is no attempt to keep the mastoid small.

The next structure visualized deep in the mastoid cavity is Ko ¨ rner’s septum,

the remnant of the petrosquamous suture line. Once through Ko ¨ rner’s sep-

tum, the lateral semicircular canal is visible on the medial side of the antrumas shown in Fig. 6. The otic capsule bone is easily distinguished from the

mastoid air cells by its smooth glistening appearance.

For proper exposure, it is critical at this point to thin the posterior exter-

nal auditory canal. The lateral external auditory canal is thinned from be-

hind to the base of the spine of Henle. This thickness is carried medially

to the level of the mastoid antrum. The superior external auditory canal is

thinned similarly, and the bone between the middle fossa tegmen and supe-

rior ear canal is removed to open the zygomatic root. As dissection is con-

tinued medially, the epitympanum is opened widely and both the incus andmalleus are visualized. Air cells lateral to the labyrinth are exenterated down

the mastoid tip where the digastric ridge is identified. At the completion of

the procedure, the middle and posterior fossa plates, sigmoid sinus, poste-

rior external auditory canal, and bony labyrinth are all skeletonized.

For endolymphatic sac procedures, the sigmoid sinus is decompressed

and the jugular bulb identified. The labyrinth is skeletonized, and the

Fig. 5. Drill cuts used in start of mastoidectomy. (A) Thin layer of tegmen bone is left over the

middle fossa dura, remembering that tegmen height is variable depending on mastoid pneuma-

tization. Cut (B) perpendicular to the first and tangential to the external auditory canal is made

from the zygomatic root to the mastoid tip. Cut ( C ) is made from the mastoid tip to the

sinodural angle.

1103MASTOIDECTOMY



dura between Donaldson’s line, a line drawn as the posterior extension of

the lateral semicircular canal, and the jugular bulb is exposed. The endolym-

phatic sac and duct are identified and decompressed carefully or opened

over the underlying dura.At the completion of the procedure, the mastoid periosteum is reapproxi-

mated with several interrupted 3-0 Vicryl sutures, and the skin is closed with

interrupted subcuticular 4-0 Vicryl sutures. The wound is then covered with

a piece of telfa, several 4 4s, and a Glasscock dressing. The mastoid defect

rarely causes any aesthetic concerns, but recently surgeons have attempted

to reconstruct the mastoid cortex with titanium mesh [13].

Facial recess or posterior tympanotomy

As seen in Fig. 7, the facial recess is an inverted triangle bounded poste-

riomedially by the facial nerve, anterolaterally by the chorda tympani nerve,

and superiorly by the incus buttress. The first step in safely performing a fa-

cial recess is to ensure that the posterior external auditory canal is thinned

appropriately at the end of a complete mastoidectomy. The next step is iden-

tification of the facial nerve using previously found landmarks including the

lateral semicircular canal, short process of the incus, and digastric ridge. The

facial nerve is always found inferomedial to the lateral semicircular canal.

As shown in Fig. 7, a line drawn as the extension of the short process of the incus approximates the facial recess.

Using a large diamond burr and copious amounts of irrigation, the facial

nerve is identified throughout its entire mastoid course, from the second

genu just inferior to the lateral semicircular canal to the stylomastoid fora-

men. Using strokes parallel to the direction of the nerve, the nerve is traced

out, leaving a thin layer of the fallopian canal bone intact over the nerve.

Fig. 6. Complete mastoidectomy in cholesteatoma dissection. Asterisk indicates lateral semicir-

cular canal.

1104 BENNETT et al



The surgeon must be wary of a lack of bony covering, or dehiscent, facial

nerve in the mastoid. Invariably, there are several small vessels around

the facial nerve that bleed during dissection near the nerve that usually

can be controlled by the diamond burr or bipolar cautery. Next, the chorda

tympani nerve is identified as the anterior branch of the facial nerve 4 to 5mm proximal to the stylomastoid foramen. Dissection proceeds between the

medial facial nerve and lateral chorda tympani nerve superiorly where the

recess is the widest until the middle ear is entered. A short bridge of bone,

the incus buttress, is left in the superior part of the facial recess to protect

the incus from the drill and maintain the support for the incus.

Extended facial recess

The facial recess can be extended after a complete mastoidectomy witha facial recess both inferiorly and superiorly. Superiorly, the incus buttress

can be removed with a small diamond burr. After removal of the incus

and head of the malleus, the entire epitympanum can be accessed. Dissection

can proceed anteriorly to the temporomandibular joint.

Inferiorly, an extended facial recess can expose the entire hypotympanum

as shown in Fig. 8. The chorda tympani nerve is skeletonized and sacrificed

sharply to avoid retrograde trauma to the facial nerve. As shown in Fig. 8,

dissection proceeds between the facial nerve and the tympanic membrane

annulus as far anteriorly as the parotid fascia. Identification of the jugularbulb in this approach often helps avoid inadvertent injury.


The goal in creating a modified radical mastoidectomy is to create

a smooth, self-cleaning cavity with no corners, edges, or depressions in

Fig. 7. Facial recess. (dashed line) Short process of the incus helps identify the facial recess.

1105MASTOIDECTOMY



which debris can accumulate. As shown in Fig. 9, the keys to the procedure

include [7]:

Aggressive saucerization of the mastoid

Eliminating irregularities or overhangs in the bone

Removing the posterior bony external auditory canal down to the level

of the facial nerve

Creating a large meatus

The modified radical mastoidectomy procedure starts after a complete

mastoidectomy and identification of the mastoid segment of the facial nerve.

The incudostapedial joint is separated, and both the incus and malleus are

removed. The external auditory canal is then removed completely to the

level of the fallopian canal, first with a large cutting burr and later with a di-

amond burr. If the air cells in the mastoid tip are diseased, they are com-

pletely exenterated to avoid dependent tip infections. If the mastoid is

Fig. 8. Inferior extended facial recess. Asterisks indicate sacrificed chorda tympani nerves.

Fig. 9. Modified radical mastoidectomy. Asterisk indicates low facial ridge. Arrowheads

indicate smooth junction of ear canal plus mastoid cavity.

1106 BENNETT et al



well aerated, it is often helpful to reduce the size of the cavity by removing

the lateral aspect of the mastoid tip, allowing the soft tissue to ‘‘cave in’’ and

auto-obliterate some of the cavity. As shown by the arrowheads in Fig. 9,both the floor and roof of the medial ear canal are then drilled flush to

the anterior ear canal. This creates a smooth transition between the ear ca-

nal and tegmen superiorly and mastoid tip inferiorly. Care must be used in-

feriorly to avoid injury to a high jugular bulb in the hypotympanum. The

ossicular chain may be reconstructed and a large fascial graft is used to rec-

reate the tympanic membrane.

A large meatoplasty is necessary for epithelialization of the cavity and

easier postoperative care. A postauricular approach is used to remove

nearly 30% to 40% of the conchal cartilage as shown by the trapezoidalwedge of cartilage between lines A and B in Fig. 10. This allows posterior

reflection of the Ko ¨ rner’s flap without deforming the auricle. Electrocautery

is used to divide the subcutaneous tissues of the auricle in a half-moon

shape until the conchal cartilage is encountered. The cartilage is then ex-

posed medially to about the bony cartilaginous junction. A curvilinear in-

cision is made through the cartilage as shown in Fig. 11. Retrograde

elevation of the deep perichondrium with a freer elevator is then performed,

and a crescent-shaped wedge of cartilage is removed. A small portion of the

Fig. 10. Meatoplasty. (A) Superior canal cut. (B) Inferior canal cut. Dashed line indicates area

of cartilage removed. The lower image shows Koerner’s flap reflected posteriorly.

1107MASTOIDECTOMY



cartilage is cut to the appropriate size and thinned for use in the ossicular

chain reconstruction.

A shown by the dashed lines in Fig. 10, meatal incisions A and B are

then made to enlarge the opening of the external auditory canal. A 15blade is placed in the ear canal and under direct vision an incision is

made through skin and subcutaneous tissue at 12 o’clock in the external

auditory canal. As shown by line A, this incision is made from the bony

cartilaginous junction to the incisura notch. An incision is made in the in-

ferior aspect of the ear canal as depicted by line B. These incisions are

made through the skin and subcutaneous tissues in continuity with the

postauricular Koerner’s flap. As shown in Fig. 12, three subdermal sutures

are placed between the Koerner’s flap and periosteum to reflect the Koern-

er’s flap posteriorly. Tension in the sutures is adjusted to optimize the con-figuration of the meatus. Generally, the meatus initially should be made

about the size of the mastoid cavity because it will undergo about 25%

contraction over time. A good approximation of this size is the surgeon’s

thumb.

The postauricular skin is then closed using several interrupted subcuticu-

lar 4-0 Vicryl sutures. The mastoid and meatus are then filled with bactro-

ban ointment. The wound is then covered with a telfa, several 4 4s and

a Glasscock dressing or formal mastoid wrap.


The radical mastoidectomy is an operation performed to eliminate all

middle ear and mastoid disease through complete removal of mucosa, tym-

panic membrane, annulus, malleus, and incus. Dissection is performed in

a fashion similar to the modified radical mastoidectomy, but there is no

Fig. 11. Conchal incisions.

1108 BENNETT et al



attempt at reconstruction or tympanic membrane grafting. In addition, the

eustachian tube is occluded with a fascial plug.

Alternative procedures

Recently, several alternative procedures to the standard mastoidectomy

have been described. Dornhoffer [9] has described an intact canal wall mas-

toidectomy in which removal of the posterior superior external auditory ca-

nal provides better epitympanic exposure. It also allows for dissection of

cholesteatoma sacs in continuity without the obstruction of the ear canal.

The canal defect is then reconstructed with conchal cartilage to maintain

the natural barrier between the external auditory canal and mastoid cavity.The cartilage appears to remain stable over time, and there is a low rate of

postoperative complications or recurrences.

A recent variation of the modified radical mastoidectomy has been pro-

posed recently by Gantz and Hansen [14] in which the posterior ear canal is

removed en bloc. This creates exposure for cholesteatoma dissection similar

to a canal wall down mastoidectomy. Once dissection is complete, the poste-

rior external auditory canal is replaced, and several large bone chips are used

to seal off the epitympanum. The mastoid cavity is then obliterated with bone

pate, obviating the need for serial mastoid cavity care. Patients require hospi-talization for at least 2 days of intravenous antibiotics postoperatively [14].

Cholesteatoma dissection

For the sake of simplicity, this article will not address cholesteatoma dis-

section in the middle ear. However, cholesteatoma sacs often extend into the

Fig. 12. Koerner’s flap. Suture tension can be varied to optimize meatus.

1109MASTOIDECTOMY



mastoid air cells. Before dissection, bone is removed circumferentially

around the cholesteatoma sac avoiding direct contact with the sac. Once

fully exposed, the cholesteatoma sac is opened and the squamous debris re-moved to facilitate dissection.

Because labyrinthine fistulas are difficult to assess preoperatively, careful

examination of medial surface of the cholesteatoma sac is performed, look-

ing for flattening of the lateral semicircular canal or defects in the medial

wall of the cholesteatoma, which may indicate an underlying fistula. Areas

of suspected fistula can also be palpated carefully with blunt instruments.

Leaving a small matrix on the fistula preserves labyrinthine function in

93% of patients as opposed to only 80% if the matrix is removed [15]. If

less than 2 mm of matrix is left, a canal wall intact procedure can be per-formed if a second stage is planned. A canal wall down procedure should

be performed if a large cholesteatoma matrix is left in the mastoid [16].

Postoperative care

Both immediate and long-term care are important in the mastoidectomy

patient. Both nausea and pain are treated aggressively to make the patient

comfortable. Facial nerve function is tested and recorded. Patients are dis-charged with their dressings in place and are allowed to remove the dressing

after 24 hours. Patients are instructed to change cotton balls in their ear and

keep the postauricular incision clean. Follow-up is scheduled for 3 weeks at

which time their ears are lightly debrided and the patient is started on anti-

biotic drops. Gentian violet is often used on granulation tissue in liberal

fashion in canal wall down cavities. Water precautions are maintained for

2 months or until the ear drum is noted to be fully healed.

Complications

Facial nerve injury

Other than death, facial nerve injury is the most disturbing complication

of ear surgery. We monitor all otologic cases to aid in preservation of the

facial nerve; however, monitoring is not a substitute for the thorough

knowledge of the anatomy of the nerve. In primary surgery, surgical land-

marks are usually present and identification of the nerve is easier. In revision

surgery and congenital ears, normal landmarks may be absent, making iden-tification of the nerve more difficult.

If nerve injury is suspected intraoperatively, identification of the nerve is

performed. It is important to remember that the injury often extends beyond

the visible injury several millimeters in both directions, and 3 to 4 mm of nerve

should be exposed both proximal and distal to the suspected site of injury us-

ing a diamond burr. Injury to the epineurium or nerve sheath usually has no

1110 BENNETT et al



long-term consequences [17]. If less than 40% of the nerve is injured and facial

muscle contraction can be elicited with small milliamp (!0.1) stimulation of

the proximal segment of the nerve, no further treatment is necessary otherthan the decompression already performed, postoperative steroids and close

follow-up. If more than 50% of the nerve is injured, superior results may be

achieved through nerve grafting [18]. This is often a difficult decision, and

a consultation from a colleague is useful in prompt evaluation of the nerve.

Primary reanastamosis through simple reapproximation in the fallopian ca-

nal or several 9-0 sutures through the epineurium should be performed if there

is enough length of nerve present. If there is a segment of nerve missing, mo-

bilization of the nerve may obtain the extra length needed for anastomosis. If

more length is still needed, a cable graft using the great auricular or sural nervemay be used.

Immediate facial paralysis in the postoperative period also requires

prompt evaluation. Several hours may pass to ensure paresis is not the result

of overzealous use of local anesthetic at the beginning of the case. If paral-

ysis persists beyond 4 hours, prompt operative exploration of the nerve is

warranted. Postoperative care depends on intraoperative circumstances

and common sense; if the nerve was already decompressed in the operating

room, observation may be appropriate. If the operative team has gone home

and an inexperienced team is present, it may be advisable to observe the pa-tient until the regular team is back in the morning. Referral may also be the

best option in these difficult cases depending on the experience and expertise

of the surgeon. Conservative management with steroids, antibiotics, and

antivirals is warranted in all cases of delayed facial paralysis [19].

Hearing loss

Iatrogenic hearing loss may occur after mastoid surgery. Sensorineural

hearing loss (SNHL) may be the result of removal of cholesteatoma over

labyrinthine fistulas or inadvertent contact between the drill and ossicularchain during dissection. Labyrinthitis may also lead to SNHL as inflamma-

tory cells enter the inner ear via the round or oval windows. Drill injuries

usually result in a high-frequency sensorineural hearing loss. Conductive

hearing losses are usually observed. They can be owing to multiple etiologies

including middle ear adhesions, tympanic membrane perforation, middle

ear effusions, ossicular fixation, or failed ossicular chain reconstruction.

Infection

Postoperative infections occur in 2% to 5% of mastoidectomies. Infec-

tion may be the result of wound infection or continued chronic ear disease.

Routine prophylaxis may not necessarily reduce postoperative infection

rates [20]. Perichondritis occurs in approximately 1% of canal wall down

procedures; therefore, perioperative antibiotics are used routinely in these

procedures. Aggressive intervention with debridement and topical

1111MASTOIDECTOMY



antibiotics will limit overall disfigurement. Infections in a mastoid with

exposed dura may predispose the patient to meningitis and brain abscesses.

Vertigo

Labyrinthine fistulas and injuries during mastoid surgery may alter the

vestibular responses of an ear. Chronic infection may also be a source of re-

duced vestibular function. Although unilateral loss of vestibular function

may occur, chronic disequilibrium is rare.

Intracranial injury

Exposure of dura generally is avoided but is not of consequence unlesslarge defects in the tegmen, dural abrasions, or cerebrospinal fluid are en-

countered. Repair is generally through layered closure with soft tissue sup-

port including muscle and fascia grafts with fibrin glue. Emergence from

anesthesia must be controlled without bucking or rises in intracranial

pressure.

Bleeding

Like any surgery, bleeding is a potential postoperative risk. In modifiedradical and radical mastoidectomies, postoperative bleeding is greater owing

to more soft tissue dissection; however, blood drains through the meatus

and there is little risk for hematoma formation. Injury to large vascular

structures like the sigmoid sinus, jugular bulb, or large emissary veins man-

dates immediate assessment. Bleeding often is controlled easily with gelfoam

and gentle pressure. Hematomas may form from uncontrolled bleeding or

more often from vessels in vasospasm during the procedure, which start

bleeding with coughing or straining in the postoperative period.

Canal defects

Small defects in the external auditory canal usually require no interven-

tion. Defects greater than 0.5 cm may be fixed with bone pate or cartilage

grafting often with overlying fascial grafts to prevent canal cholesteatoma

formation.

Further readings

Cass S. Mastoid surgery. In: Operative Otolaryngology Head and Neck Surgery. 1997.p. 1280–98.

Glasscock ME. Surgical technique for open mastoid procedures. Laryngoscope 1982;92:1440–2.

Glasscock ME III, Haynes DS, Storper IS, et al. Surgery for chronic ear disease. In: Hughes GB,

Pensak ML, editors. Clinical otology. New York: Thieme Medical Publishers; 1996.

p. 215–32.

Haynes DS, Harley DH. Surgical management of chronic otitis media: beyond tympanostomy

tubes. Otolaryngol Clin N Am 2002;35:827–39.

1112 BENNETT et al



Jackson CG, Glasscock ME, Nissen AJ, et al. Open mastoid procedures: contemporary

indications and surgical technique. Laryngoscope 1985;95:1037–43.

Kaylie DM, Jackson CG. Revision Chronic Ear Surgery. Otol Head Neck Surg 2006;134:443–50.

McGrew BM, Glasscock ME. Impact of mastoidectomy on simple tympanic membrane

perforation repair. Laryngoscope 2004;114:506–11.

Pillsbury HC III, Carrasco VN. Revision mastoidectomy. Arch Otolaryngol Head Neck Surg

1990;116:1019–22.

Smyth GD, Toner JG. Mastoidectomy: the canal wall down procedure. In: Otologic surgery.

p. 226–39.

Syms MJ, Luxford WM. Management of cholesteatoma: status of the canal wall. Laryngoscope

2003;113(3):443–8.

References

[1] Schwartze HH, Eysell CG. Ueber die Kunstliche eroffnung des warzenfortsatzes. Arch

Ohrenheilkd 1873;7:157.

[2] Zaufal E. Technik der Trepanationdes Proc. Mastoid. Nach Kuster‘‘schen Grundsatzen.

Arch Ohrenheilkd 1893;30:291.

[3] Shambaugh GE, Glasscck ME. Surgery of the ear. Philadelphia: WB Saunders; 1980.

[4] Wullstein HL. Tympanoplasty and its results. Arch Ohren Nasen Kehlkopfheilkd 1958;171:

84–90.

[5] House WF. Middle cranial fossa approach to the petrous pyramid. Report of 50 cases. Arch

Otol 1963;78:460–9.

[6] Haynes DS. Surgery for chronic ear disease. Ear Nose Throat J 2001;80:8–11.

[7] Jackson CG, Touma B. A Surgical solution for the difficult chronic ear. Am J Otol 1996;17:

7–14.

[8] Sheehy JL. Mastoidectomy: the intact canal wall procedure. In: Otologic surgery. Chapter

18. 212–24.

[9] Dornhoffer J. Retrograde mastoidectomy with canal wall reconstruction: a follow-up report.

Otol Neurotol 2004;25:653–60.

[10] Dodson EE, Lambert PR. Intact canal wall matoidectomy with tympanoplasty for choles-

teatoma in children. Laryngoscope 1998;108(7):977–83.

[11] Haynes DS. Perioperative antibiotics in chronic suppurative otitis media. Ear Nose Throat J

2002;81(1):13–5.

[12] Haynes DS, Vos JD, Labadie RF. Acellular allorgraft dermal matrix for tympanoplasty.

Curr Opin Otol Head Neck Surg 2005;13(5):283–6.

[13] Kim HH, Wilson DF. Titanium mesh for functional reconstruction of the mastoid cortex

after mastoidectomy. Otol Neurotol 2006;27(1):33–6.

[14] Gantz BJ, Hansen M. Canal wall reconstruction tympanomatoidectomy with mastoid oblit-

eration. Laryngoscope 2005;115:1734–40.

[15] Palva T, Ramsay H. Treatment of labyrinthine fistula. Arch Otolaryngol Head Neck Surg

1989;115(7):804–6.

[16] Glasscock ME, Poe D. Surgical management of cholesteatoma in an only hearing ear. Otol

Head Neck Surg 1990;102:246–50.[17] Lambert P. Mastoidectomy. In: Cummings otolaryngology head and neck surgery. 4th edi-

tion. 2005. p. 3075–86.

[18] Brackman DE. Tympanoplasty with mastoidectomy: canal wall up procedures. Am J Otol

1993;14:380.

[19] Vrabec JT. Delayed facial palsy after tympanomastoid surgery. Am J Otol 1999;20(1):26–30.

[20] Jackson CG. Antimicrobial prophylaxis in ear surgery. Laryngoscope. 1988 Oct;98(10):

1116–23.

1113MASTOIDECTOMY



Retrograde Mastoidectomy

John L. Dornhoffer, MDa,b,c,*aDivision of Neurotology, Department of Otolaryngology/Head and Neck Surgery,

University of Arkansas for Medical Sciences, 4301 West Markham,

Slot 543 Little Rock, AR 72205, USAb

ENT Clinic and Audiology Services, University of Arkansas for Medical Sciences,4301 West Markham, Slot 543 Little Rock, AR 72205, USA

cDepartment of Neurobiology and Developmental Sciences, University of Arkansas for

Medical Sciences, 4301 West Markham, Slot 543 Little Rock, AR 72205, USA

After nearly 5 decades of debate, the management of the posterior canal

wall in cholesteatoma surgery remains a controversial issue. The canal wall

up (CWU) tympanomastoidectomy preserves the normal anatomy of the ear

canal, thus avoiding the risk of bowl problems and the necessity for periodic

cleaning and leading to reduced healing time and, possibly, better hearingresults [1]. Likewise, one must consider that surgery for cholesteatoma,

which frequently involves complex ossicular reconstruction, may require

hearing rehabilitation, with the need for amplification postoperatively.

Hearing aid fitting after CWU surgery is generally straightforward and as-

sociated with fewer complications than that after canal wall down (CWD)

tympanomastoidectomy. However, a high recitative rate (cholesteatoma

left behind) and recurrence rate (recurrent retractions leading to cholestea-

toma formation) continue to be major drawbacks to CWU surgery.

At issue is the exposure of the critical anatomic regions for cholesteatomaremoval: the epitympanum and associated supratubal recess and the meso-

tympanum. These are the two areas associated with most recurrent disease

[2]. Primary acquired cholesteatomas typically begin as retraction pockets in

the pars flaccida (epitympanic cholesteatoma) or posterior pars tensa (mes-

otympanic cholesteatoma). In the former situation, the sac typically forms

in Prussak’s space, lateral to the malleus, and exits posteriorly or inferiorly,

with some evidence of erosion of the scutum. The sac typically egresses lat-

eral to the body of the incus into the antrum. With larger epitympanic cho-

lesteatomas, one should anticipate extensive involvement of the epitympanicspace and supratubal recess. The sinus tympani and facial recess can also be

problematic, but are involved secondarily through inferior extension of the






39 (2006) 1115–1127





cholesteatoma into the mesotympanum. Mesotympanic cholesteatomas, on

the other hand, arise from posterior retraction of the pars tensa and extend

toward the antrum medial to the incus. In these cases, involvement of thesinus tympani and facial recess is very frequent, with extension to the epi-

tympanum between the facial nerve and incus.

CWU surgery, especially in cases with poorly developed zygomatic root

cells and a low tegmun, can provide very limited exposure of the epitympa-

num and posterior mesotympanum, leading to blind and piecemeal removal

of the cholesteatoma, which contributes to the high recitative rate. Fre-

quently, the surgeon believes that the anterior epitympanic space has been

reached, while in actuality only the bony cog has been exposed and disease

is still present in the supratubal recess. Because of these short comings, sec-ond-stage surgery is frequently recommended 9 to 12 months after the initial

CWU tympanomastoidectomy [3–5]. A review of the literature shows a wide

variation in the reported recurrence rates with this technique. Sheehy and

Robinson [6] initially reported recurrence rates of 5%, whereas Glasscock

[7] reported a 14% recurrence rate. Recurrence rates up to 20% to 40%

have also been reported [3,4]. As pointed out so aptly by Smyth [2],

long-term results with CWU cholesteatoma can be very humbling, with

recurrence rates of between 15% and 50%, even after staging. Nyrop and

Bonding’s 10-year follow-up of CWU cases in Copenhagen showed that70% required a later CWD surgery [8].

The CWD technique creates an open cavity after removal of the bony

posterior canal wall, providing excellent exposure for cholesteatoma re-

moval. The epitympanum, cog, and supratubal recess are widely exposed,

facilitating complete removal of the cholesteatoma sac and lowering the re-

currence rate to 5% to 10% [9,10]. Taking the canal wall down to the level

of the facial ridge deals adequately with facial recess disease and affords im-

proved, but by no means complete, view of the sinus tympani. In cases with-

out sinus tympani involvement, this technique can also frequently beperformed as a single-stage surgery. The main disadvantages of this tech-

nique are related to the necessity for periodic cleaning of the mastoid cavity

[9,10]. Subsequent bowl infections can occur, especially when the cavity be-

comes wet, causing significant lifestyle changes, particularly in the pediatric

age group [9]. In addition, the hearing results are frequently worse due to the

shallow middle ear cleft [9,10]. Although bone-anchored hearing aids and

open-mold technology have offered some new possibilities for amplification

in this group, traditional hearing aid fitting can be very problematic due to

a wet cavity, or widened, distorted meatus.

Evolution of retrograde tympanomastoidectomy with canal reconstruction

There has been a trend toward the development of surgical techniques that

involve temporary removal of the canal wall, offering the exposure of CWD

surgery for cholesteatoma extirpation, followed by reconstitution of the canal

1116 DORNHOFFER



wall defect using autologous (bone, cartilage) or alloplastic (hydroxyapatite

cement, titanium) graft materials [11–17]. The retrograde tympanomastoidec-

tomy is one such technique that involves temporary removal of the upper canalwall, in association with a retrograde-type mastoidectomy, followed by recon-

struction of the canal defect using Cymba cartilage. The posterior tympanic

membrane is reconstructed with the cartilage palisade technique in close ap-

proximation with the canal reconstruction to inhibit recurrent retractions.

Our 15-year experience with this technique has shown that the exposure of

the epitympanum and upper mesotympanum is similar to CWD surgery, al-

lowing single-stage surgery in 90% of cases, with 10-year recurrence rates

very similar to those reported for CWD surgery. The primary indication for

second-look or staged surgery is involvement of the sinus tympani with uncer-tain removal, which continues to be a difficulty with all techniques. Primary

reconstruction of the ossicular chain is done in all cases, regardless of the

need for a planned second surgery, as it acts as a scaffolding for the primary

reconstruction. Before a detailed description of this technique is provided, it

is worthwhile to consider its evolution.

Conceptually, this technique seems to resemble an extended atticotomy;

however, it actually represents a union of two divergent approaches: the os-

teoplastic flap of Wu ¨ llstein [14] and the small-cavity technique of Smyth [2].

Wu ¨ llstein recognized the advantage of this concept when he describedosteoplastic flap surgery in 1974 [14]. A traditional CWU surgery was

performed, followed by removal of the canal wall for exposure of the

epitympanum, with subsequent reinsertion of the bony segment after choles-

teatoma extirpation. Others have modified this technique by rigidly fixing

the canal remnant with microplates and frequently using a vascularized ped-

icled flap for coverage [17]. Good preliminary results have been reported

with these modifications [17]. However, the problem of a technically chal-

lenging fit, coupled with late bony necrosis of the reinserted graft, limited

the routine acceptance of this technique [13].Likewise, we have found that after the bony segment was removed, it was

often necessary to drill anteriorly to further expose the supratubal recess, re-

sulting in the bony segment becoming too small to be reused. Smyth’s small

cavity surgery, on the other hand, described a technique that involved

a CWD surgery by starting from the canal side, gradually enlarging it until

complete exposure of the cholesteatoma was accomplished. This ended up

creating a much smaller cavity than traditional CWD techniques, which

involve a complete mastoidectomy, skeletonizing the sigmoid sinus and

tegmen, and removing the canal wall when deemed necessary. With Smyth’stechnique, it was found that when the mastoid was sclerotic, as is often the

case with adult cholesteatomas, the cholesteatoma extended only to the

antrum, resulting in a very small cavity.

Professor Jan Helms, who succeeded Wu ¨ llstein at the University of

Wu ¨ rzburg, performed an approach similar to Smyth’s, starting with a poste-

rior canaloplasty and widening the canal until the air cells were first visible

1117RETROGRADE MASTOIDECTOMY



through the bone. The drilling then turned superiorly, skeletonizing the teg-

mun to the anterior malleolar ligament and following the cholesteatoma

posteriorly into the antrum. The cholesteatoma frequently extended justto the antrum, in which case the canal wall defect was reconstructed with

cartilage from the conchal bowl. The defect was typically small enough

that the cartilage was supported superiorly at the zygomatic buttress, inferi-

orly at the posterior canal wall, and laterally at the mastoid cortex. If the

cholesteatoma was more extensive, ‘‘turning the corner’’ into the mastoid

tip, the canal could be taken down as described by Smyth [2].

This author has modified the technique to suit the large number of pedi-

atric patients with cholesteatoma seen in his practice. These young patients

tend to have larger cholesteatomas in more developed mastoids, requiringCWD surgery using the technique of Helms. This technique was then

slightly modified to allow the cholesteatoma to be followed posteriorly,

but if the disease ‘‘turned the corner’’ invading the mastoid tip, it was pos-

sible to follow the disease while leaving the lower two thirds of the posterior

canal wall intact. This resulted in the need to perform more extensive groov-

ing at the zygomatic buttress as well as the posterior canal wall to facilitate

a cartilage reconstruction that would be supported only superiorly and infe-

riorly. This results in an optimized technique for cholesteatoma removal

that represents the best of what the CWU and CWD procedures have tooffer. It is described in detail below.

Surgical technique

Cholesteatoma removal

The mastoid cortex is widely exposed through a traditional postauricular

skin incision. A tympanomeatal flap is then developed, and the middle ear is

entered inferiorly, away from the cholesteatoma, to determine the extent of disease in the middle ear. As this technique involves extensive drilling in

close proximity and just lateral to the head of the malleus and the body

of the incus, it is necessary to visualize the incudostapedial joint and the in-

tegrity of the lenticular process of the incus as an initial step. Except in cases

of very limited retraction pockets, the incudostapedial joint is disarticulated

to avoid trauma to the inner ear from the subsequent drilling. A canalo-

plasty is performed posteriorly until air cells can just be visualized through

the intact cortical bone of the ear canal. This improves the view of the pos-

terior mesotympanum in cases of mesotympanic cholesteatomas and facili-tates subsequent drilling of the upper canal wall as the cholesteatoma is

followed up and back into the epitympanum and antrum. A prominent

tympanosquamous suture and spine are taken off at this point to increase

exposure of the pars flaccida.

The drilling for cholesteatoma removal begins on the canal side with a

3 mm to 4 mm cutting burr at the upper posterior quadrant of the posterior

1118 DORNHOFFER



The exposure for cholesteatoma removal is very similar to a CWD

situation and allows a nice progression of disease removal, with direct visu-

alization of the horizontal canal, facial nerve, and oval window/stapes area.

If necessary, one can drill the cog to follow disease in the supratubal recess.If disease extends anteriorly into the tubal orifice, it is possible to gain in-

creased exposure by cutting the tensor tympani tendon. If possible, the ten-

don is left intact to facilitate and secure the ossicular reconstruction.

Reconstruction

Although removal of the cholesteatoma is important to reduce residual

disease, reconstruction is equally important to prevent recurrence of

Fig. 1. Cholesteatoma is followed back posteriorly with exposure of entire sac at its origin from

the tympanic membrane.

Fig. 2. Sac is resected, including the incus and head of malleus.

1120 DORNHOFFER



retraction pockets. Primary ossicular reconstruction is performed in all ears,

even when second-look surgery is planned, because the prosthesis acts as

a scaffolding to support the cartilage reconstruction of the tympanic mem-brane and maintain the middle ear space during the healing phase.

To reconstruct the canal wall, a 1 mm to 2 mm diamond burr is used to

form grooves in the bone at the tegmen superiorly and in the inferior canal

wall. The superior groove at the zygomatic root is very important and must

be drilled properly to insure a good fit. The groove should be 1 mm to 2 mm

wide and 2 mm deep and should be oriented parallel to the inferior canal to

avoid torsion of the graft. Medially, the groove and subsequent reconstruc-

tion should extend down to and just posterior to the anterior malleolar spine

and ligament so that is will approximate the cut edge of the malleus pre-cisely. The inferior groove should be drilled carefully as the canal is fre-

quently thin due to a combination of the canaloplasty and subsequent

mastoidectomy. Often, a shelf, as opposed to a groove, is drilled here.

Although we have experimented with other materials, autologous carti-

lage appears to be the ideal material for canal wall reconstruction. A piece

of cartilage is harvested from the cymba of the conchal bowl to reconstruct

the posterior canal wall. Because of its appropriate curvature, cartilage from

the cymba is ideal for this purpose, and it generally has a uniform thickness

of 1 mm. Generally, the cartilage will be 1 mm to 2 mm wider than the bonydefect, allowing the canal wall reconstruction to snap into place under a little

tension. The medial fit of the canal wall reconstruction is the most critical,

and it is essential that a good fit between the cartilage and the residual bony

canal wall be achieved and that the superior edge of the cartilage reconstruc-

tion be placed medially so that it closely approximates the handle of the mal-

leus (Fig. 3).

For ossicular reconstruction, we typically use hydroxyapatite prostheses

and attempt to use the malleus in the reconstruction. It has been shown that

incorporation of the malleus in ossicular reconstruction provides an acousticgain, possibly due to its cantenary action in the tympanic membrane [19].

Fig. 3. Canal wall defect reconstructed with Cymba cartilage, with close proximity to the cut

edge of the manubrium.




Likewise, the presence of the malleus with an intact anterior malleolar liga-

ment offers improved prosthesis stability by allowing precise length adjust-

ments and ultimate fit, leading to optimal hearing results [20]. When themalleus handle and suspensory ligaments are present, we have found that

a partial ossicular replacement prosthesis (PORP), which is 2 mm in length,

and a total ossicular replacement prosthesis (TORP), cut to 4.0 mm to

4.5 mm, can be consistently used for precise reconstruction when the notch

of the prosthesis is placed just inferior to the insertion of the tensor tympani

(Fig. 4A and B).

After precise ossicular reconstruction is performed, the posterior half of

the tympanic membrane is reconstructed with pieces of cartilage using the

modified palisade technique. With this technique, the tympanic membraneis put together like a jigsaw puzzle: the half moon-shaped piece is placed

on top of the prosthesis first, abutting the malleus handle, followed by the

scutum piece; any spaces left between these two plates and the canal wall

are reconstructed with slivers of cartilage cut to fit precisely in these areas

(Fig. 5A and B). The trifurcation of the malleus handle, canal reconstruc-

tion, and tympanic membrane reconstruction has proven to be critical for

recurrence prevention, so small chips of cartilage are very important in

this area. Likewise, if the drilling proceeds superior to the anterior malleolar

ligament, care should be taken to reinforce this small area with cartilage.The reconstruction is then covered with perichondrium or fascia and the

tympanic membrane remnant, if available. No space is left between the ca-

nal wall and reconstructed tympanic membrane to prevent cholesteatoma or

retraction pocket recurrence. The anterior half of the tympanic membrane is

typically not reconstructed with cartilage to allow postoperative surveillance

and tube insertion, if necessary.

Fig. 4. (A) Schematic showing reconstruction of the ossicular chain after canal reconstruction.

(B) Interoperative picture demonstrating reconstruction.

1122 DORNHOFFER



The canal is then packed with antibiotic ointment, and a Pope Otowick

(Xomed Co., Jacksonville, Florida) is placed in the ear canal. The packing

is removed 10 days postsurgery, and the patient is instructed to perform

the Valsalva maneuver three times a day, starting 2 weeks after the surgery.

The Otovent (Abigo Medical, Sweden) has been used in children who are

unable to perform this maneuver. The first postoperative audiogram is ob-

tained 6 to 8 weeks postsurgery. If the patient is unable to perform the Val-

salva maneuver or if the postoperative period is complicated by an effusionor retraction of the reconstructed tympanic membrane, a ventilation tube is

placed 3 to 4 months after the surgery. Primary ventilation tube insertion is

generally avoided.

For the most part, the retrograde mastoidectomy allows removal of the

cholesteatoma sac in one piece, with direct exposure, so second-look surgery

is only performed in those cases where the cholesteatoma sac was violated

and had to be removed piecemeal. The area of the sinus tympani is the

most problematic region, as exposure here is difficult even with the retro-

grade mastoidectomy.

Results with the retrograde mastoidectomy

As of this writing, the retrograde technique has been used at our institu-

tion as the primary surgery for cholesteatoma removal for 13 consecutive

years, resulting in an experience with over 400 ears. Short-term (2-year)

and longer-term (8-year) results have been reported [21,22], indicating a dra-

matic reduction in recurrence rates and in need for a second surgery com-

pared with our experience with CWU surgery [23]. We no longer performCWD surgery as a primary surgery unless more than 50% of the canal

wall is destroyed by disease or exterioration is needed because of large lab-

yrinthine or cochlear fistulae, where the matrix cannot be safely removed.

In our longer term study [22], we examined the results of 46 patients

(representing 50 ears undergoing this procedure) who had an average 7-year

follow-up. Second-look surgery was performed in 4% for questionable

Fig. 5. (A) Schematic showing reconstruction of the posterior tympanic membrane with the

palisade technique. (B) Interoperative picture demonstrating precise fit between palisade

reconstruction of the tympanic membrane and canal wall reconstruction.




incomplete removal of disease in the sinus tympani or footplate area. One

of these patients had residual disease at the footplate. We found recurrent

cholesteatoma in eight ears (six adult and two pediatric), representing a re-currence rate of 16%. The site of the original cholesteatoma was evenly

divided between the epitympanum and the mesotympanum. However, of

the recurrent cholesteatomas, 63% had originally been mesotympanic

and 37% had originally been epitympanic, suggesting a higher recurrence

rate for mesotympanic cholesteatomas.

This finding is not totally unexpected. The exposure of the epitympanum

and supratubal recess is superior to the standard CWU approaches, and the

removal is quite direct. The reason some ears develop epitympanic disease as

opposed to mesotympanic cholesteatomas in the first place, from seeminglythe same pathophysiologic mechanism (ie, Eustachian tube dysfunction), is

unknown. It is possible that mesotympanic cholesteatomas result from more

pervasive Eustachian tube dysfunction compared with epitympanic choles-

teatomas, the latter of which may reflect anatomic or structural blockage

of the antrum, which could be more easily corrected with surgical removal

of the disease.

Likewise, it appears that a ‘‘stable retraction’’ after cholesteatoma

surgery may be an oxymoron. Both of the ears noted to have stable

epitympanic retractions at the short-term assessment eventually developedcholesteatomas at longer follow-up. Thus, earlier or more aggressive intuba-

tion may need to be performed in those ears with early signs of retraction.

The site of recurrence after reconstruction was typically in the epitym-

panic area (six of eight ears), just above the malleus handle, between the

canal wall reconstruction and the tympanic membrane reconstruction. All

six of these eight ears were revised, with removal of the previously inserted

canal wall cartilage to expose the recurrent retraction, removal of the

retraction, and reinsertion of the canal wall with reinforcement of the

epitympanum. This recurrence pattern points out a relative weakness of the retrograde technique. Removal of the canal wall allows excellent expo-

sure for complete removal of the cholesteatoma, leading to very low inci-

dence of residual disease with a single-stage approach like CWD surgery.

However, the reconstruction of the canal wall and posterior tympanic mem-

brane is challenging, with a definite learning curve. Most of the recurrences

in this series (four) were noted during the initial 2-year follow-up, and the

area between the canal wall and tympanic membrane cartilage reconstruc-

tion was identified as a high-risk site. Because of this, much greater care

is now taken to reinforce this area with chips of cartilage (Fig. 6). The effectof this modification is felt to be positive, but longer follow-up will be impor-

tant in this cohort of patients.

The negative impact of smoking on long-term results was apparent in our

follow-up study [22] and has changed the way we counsel and treat patients

who are smokers. If one includes as complications the presence of recurrent

disease, need to intubate the ear postoperatively, occurrence of a perforation,

1124 DORNHOFFER



and need for a second-look surgery due to poor hearing results, the long-

term complication rate of smokers was 79% (15/19 ears), compared with

16% (5/31 ears) for nonsmokers, making overall complications in smokers

five times that seen in nonsmokers. Postoperative intubation for persistent

effusion was necessary in nine individuals in this study, of which sevenwere smokers. Tobacco use has been reported to have a negative impact

on graft take and hearing results during tympanoplasty [24], but its effect

on long-term cholesteatoma recurrence has not been reported until recently.

All but two of the recurrent cholesteatomas in our series were in individuals

who routinely smoked cigarettes, making cholesteatoma recurrence almost

five times higher for individuals who used tobacco.

Long-term hearing results with the retrograde mastoidectomy technique

have been encouraging, and compare favorably with those reported in staged

ossiculoplasty [7]. In our longer term study, there was a slight trend towardworsening hearing compared with the earlier follow-up, especially in the pedi-

atric group, but the decrease was not significant, and the original gain from the

preoperative level was maintained (see Table 1) [22]. The type of ossicular re-

construction (PORP versus TORP) had no apparent impact on hearing re-

sults. Because there may have been a trend for those patients experiencing

Fig. 6. Postoperative picture after the retrograde technique. Area in ellipse demonstrates

‘‘high-risk’’ area reinforced with additional pieces of cartilage.

Table 1

Comparison of short- and long-term hearing results in 50 ears undergoing retrograde mastoid-

ectomy with canal wall reconstruction

Preoperative 2-Year postoperative 8-Year postoperative

Air–bone gap/dB Number % Number % Number %

0–10 (excellent) 5 10 24 48 23 46

11–20 (good) 10 20 23 46 21 42

21–30 (fair) 19 38 3 6 6 12

O30 (poor) 16 32 0 0 0 0




no complaints to disregard long-term follow-up (all but one of the 14 patients

who had not been recently seen were doing well), it is possible that our longer

term data are biased toward worse outcomes. However, whether patients didnot return for follow-up because they were doing well or because they were

doing poorly is impossible to know with any degree of certainty.

Summary

The single-stage technique for cholesteatoma removal and canal wall

reconstruction described here appears to be a valid procedure that can be

performed as a single-stage surgery in greater that 90% of patients, both pe-

diatric and adult. We found no difference between adults and children in ei-ther recurrence rates or hearing results. Nearly all primary cholesteatomas

can be dealt with using this technique, except when more than 50% of the

canal wall is destroyed with disease. Although our longer-term results

have been encouraging, the need for long-term follow-up and continued

management of Eustachian tube dysfunction is essential, as recurrences

can be quite delayed. However, because cholesteatoma can recur fairly rap-

idly between the canal wall and tympanic membrane reconstruction, rigor-

ous reconstruction with cartilage chips is advocated in this area. When

recurrences do occur, they can be managed with removal and reinsertionof the canal, avoiding a CWD procedure in greater than 90% of cases. It

appears that mesotympanic cholesteatomas may have a higher recurrence

rate compared with epitympanic disease. Likewise, tobacco use is associated

with a higher long-term complication rate, including recurrent cholestea-

toma, perforation, poor hearing, and need for middle ear intubation as a

second procedure.

References

[1] Arriaga MA. Cholesteatoma in children. Otolaryngol Clin North Am 1994;27(3):573–91.

[2] Smyth GD. Cholesteatoma surgery: the influence of the canal wall. Laryngoscope 1985;

95(1):92–6.

[3] Hirsch BE, Kamerer DB, Doshi S. Single-stage management of cholesteatoma. Otolaryngol

Head Neck Surg 1992;106(4):351–4.

[4] Austin DF. Single-stage surgery for cholesteatoma: an actuarial analysis. Am J Otol 1989;

10(6):419–25.

[5] Schuring AG, Lippy WH, Rizer FM, et al. Staging for cholesteatoma in the child, adolescent,

and adult. Ann Otol Rhinol Laryngol 1990;99(4 Pt 1):256–60.

[6] Sheehy JL, Robinson JV. Revision tympanoplasty: residual and recurrent cholesteatoma.Paper presented at the Second International Conference on Cholesteatoma and Mastoid

Surgery, Tel Aviv, Israel; 1982.

[7] Glasscock ME 3rd. Results in cholesteatoma surgery. Paper presented at the First Interna-

tional Conference on Cholesteatoma, Birmingham, Alabama; 1977.

[8] Nyrop M, Bonding P. Achievement of stable ears in cholesteatoma surgery: long-term results

of three surgical techniques. Paper presented at the Fourth International Conference on

Cholesteatoma and Mastoid Surgery, Niigata, Japan; 1993.

1126 DORNHOFFER



[9] Cody DT, McDonaldTJ. Mastoidectomy for acquired cholesteatoma: follow-up to 20 years.

Laryngoscope 1984;94(8):1027–30.

[10] Smyth GD. Surgical treatment of cholesteatoma: the role of staging in closed operations.

Ann Otol Rhinol Laryngol 1988;97(6 Pt 1):667–9.

[11] Black B. Mastoidectomy elimination: obliterate, reconstruct, or ablate? Am J Otol 1998;

19(5):551–7.

[12] Grote JJ. Reconstruction of the middle ear with hydroxylapatite implants: long-term results.

Ann Otol Rhinol Laryngol Suppl 1990;144:12–6.

[13] Magnan J, Chays A, Pencroffi E, et al. Canal wall prosthesisdnew devices. Rev Laryngol

Otol Rhinol (Bord) 1995;116(1):19–21.

[14] Wullstein SR. Osteoplastic epitympanotomy. Ann Otol Rhinol Laryngol 1974;83(5):663–9.

[15] Wiet RJ, Harvey SA, Pyle MG. Canal wall reconstruction: a newer implantation technique.

Laryngoscope 1993;103(6):594–9.

[16] Zini C, Quaranta N, Piazza F. Posterior canal wall reconstruction with titanium micro-mesh

and bone pate. Laryngoscope 2002;112(4):753–6.

[17] McElveen JT Jr, Chung AT. Reversible canal wall down mastoidectomy for acquired

cholesteatomas: preliminary results. Laryngoscope 2003;113(6):1027–33.

[18] Tos M. Mastoid surgery and reconstructive procedures. Vol. 2. New York: Thieme Pub-

lishers; 1995.

[19] Dornhoffer JL, Gardner E. Prognostic factors in ossiculoplasty: a statistical staging system.

Otol Neurotol 2001;22(3):299–304.

[20] Dornhoffer JL. Hearing results with the Dornhoffer ossicular replacement prostheses.

Laryngoscope 1998;108(4 Pt 1):531–6.

[21] Dornhoffer JL. Retrograde mastoidectomy with canal wall reconstruction: a single-stage

technique for cholesteatoma removal. Ann Otol Rhinol Laryngol 2000;109(11):1033–9.[22] Dornhoffer JL. Retrograde mastoidectomy with canal wall reconstruction: a follow-up

report. Otol Neurotol 2004;25(5):653–60.

[23] Stern SJ, Fazekas-May M. Cholesteatoma in the pediatric population: prognostic indicators

for surgical decision making. Laryngoscope 1992;102(12 Pt 1):1349–52.

[24] Becvarovski Z, Kartush JM. Smoking and tympanoplasty: implications for prognosis and

the Middle Ear Risk Index (MERI). Laryngoscope 2001;111(10):1806–11.




Mastoid Obliteration

Ritvik P. Mehta, MDa,*, Jeffrey P. Harris, MD, PhDb

aDepartment of Otology and Laryngology, Massachusetts Eye and Ear Infirmary/Harvard

Medical School, Boston, MA, USAbDivision of Otolaryngology/Head and Neck Surgery, Department of Surgery,

University of California San Diego School of Medicine, 200 W. Arbor Drive,San Diego, CA 92103, USA

The concept of mastoid obliteration was first introduced in 1911 by

Mosher to promote healing of a mastoidectomy defect [1]. Over the course

of this century, there have been numerous reports detailing a variety of tech-

niques of obliterating the mastoid cavity. The vast majority of obliteration

techniques consist of either local flaps (muscle, periosteum, or fascia) or free

grafts (bone, cartilage, hydroxyapatite, and so on) (Box 1). Mosher’s origi-nal description was that of a superiorly based postauricular soft tissue flap.

Kisch described the use of a pedicled temporalis muscle flap that was ex-

panded on by Rambo [2,3]. Popper [4] described the use of a periosteal

flap used to line, rather than obliterate, the mastoid cavity. Palva [5] went

on to describe a modification of Popper’s flap as a musculoperiosteal flap

to obliterate the mastoid bowl. Palva [6] further added the use of bone chips

and bone pate ´ in combination with a musculoperiosteal flap. In addition to

bone pate ´ , other materials that have been described as implants for mastoid

obliteration include fat grafts, diced cartilage, fascia, bone chips, and ce-ramic materials such as hydroxyapatite [7–11].

Indications and contraindications

The most common indication for mastoid obliteration is following canal

wall-down tympanomastoidectomy for chronic otitis media. A canal wall-

down mastoid cavity, if not obliterated, can result in persistent otorrhea

that can be difficult to control even with topical antibiotic therapy and fre-

quent cleaning of the cavity. Other problems associated with a mastoid


E-mail address: [email protected] (J.P. Harris).




39 (2006) 1129–1142





cavity may include the need for frequent cleaning, difficulty with the use of

a hearing aid, water intolerance due to a susceptibility to infection, and pro-

pensity to vertigo by a caloric stimulus such as warm/cold air or water [12].

Obliteration of the mastoid bowl is indicated to reduce the size of the cavity.It is ideally conducted as a primary procedure at the time of canal wall-

down mastoidectomy. However, for a problematic mastoid bowl with

chronic otorrhea and nonhealing, mastoid obliteration can be performed

as a secondary revision procedure.

Total tympanomastoid obliteration, or obliteration of the entire mastoid,

middle ear, and Eustachian tube, is used for the prevention as well as manage-

ment of cerebrospinal fluid leaks emanating through the temporal bone. The

most common complication following microsurgery for acoustic neuroma is

cerebrospinal fluid leak at a rate of 10.9% according to a recent metaanalysis[13]. Tympanomastoid obliteration can be performed primarily when the

translabyrinthine approach is used for acoustic neuroma resection. Alterna-

tively, it is used as a means of surgical treatment of cerebrospinal fluid

(CSF) leak that has failed to heal with conservative measures such as bed

rest, head elevation, and lumbar drainage. Tympanomastoid obliteration

can be used for other causes of CSF leak as well such as meningoencephalo-

celes with CSF otorrhea or rhinorrhea or severe temporal bone trauma.

Another indication for tympanomastoid obliteration includes recon-

struction following surgical treatment for malignancies of the temporalbone. The defect created by lateral, subtotal, or total temporal bone resec-

tion typically requires obliteration of the middle ear, Eustachian tube, and

mastoidectomy defect. This is especially important if there is a concomitant

CSF leak resulting from the malignancy or the resection. Large defects may

require the use of pedicled flaps or free tissue transfer for reconstruction

[14].

Box 1. Techniques of mastoid obliteration

Local flaps Meatally based musculoperiosteal flap (Palva flap)

Inferiorly based periosteal-pericranial flap

Superiorly based musculoperiosteal flap

Temporalis muscle flap

Temporoparietal fascial flap (TPFF)

Free grafts

Bone chips/bone pate

FatCartilage

Fascia

Hydroxyapatite

1130 MEHTA & HARRIS



A special situation that may require tympanomastoid obliteration is co-

chlear implantation in patients with a history of chronic otitis media. This

group of patients can be challenging because of the risks associated with for-eign body implantation in a potentially infected space. Tympanomastoid

obliteration has been used in this setting to provide complete eradication

of infection and inflammation as well as to provide a protective soft tissue

layer over the electrode array [15]. It can also be used in patients with

labyrinthitis ossificans in whom a drill-out of the cochlea is required with

removal of the posterior canal wall and tympanic membrane [16].

Tympanomastoid obliteration can be performed in cases of chronic otitis

media in patients with no usable hearing. It is important that complete erad-

ication of disease is achieved by the surgeon before tympanomastoid oblit-eration [3,17]. Box 2 lists the most common indications for mastoid and

tympanomastoid obliteration.

Relative contraindications to mastoid obliteration include persistent ac-

tive disease (cholesteatoma, malignancy, or active infection) within the tym-

panomastoid cavity. An exception to this is an extensive malignancy that

may require tympanomastoid obliteration following subtotal resection

in preparation for radiation therapy.

Techniques

Mastoid obliteration following chronic ear surgery

As mentioned above, a vast array of techniques have been described for

mastoid obliteration following canal wall-down mastoidectomy for chronic

otitis media. The authors prefer the Massachusetts Eye and Ear Infirmary

technique of mastoid obliteration using a combination of bone pate ´ and

an inferiorly based periosteal-pericranial flap [17,18]. This technique is

Box 2. Indications for mastoid/tympanomastoid obliteration

Canal wall-down mastoidectomy

Chronic otorrhea/nonhealing of mastoid bowl

Translabyrinthine acoustic neuroma resection

CSF leak

Extensive temporal bone traumaTemporal bone resection for malignancy

Cochlear implantation in patients with chronic otitis media

Cochlear implantation requiring extensive drill-out with removal

of posterior canal wall

Treatment of chronic otitis media in ears with no useful hearing

1131MASTOID OBLITERATION



outlined in detail. A postauricular incision 2- to 3-mm posterior to the post-

auricular crease is used (Fig. 1). Fig. 2 illustrates the site of harvest of the

flap. Superiorly, the plane of the temporalis fascia is identified. Inferiorly,the postauricular muscles are divided and the skin is elevated in a supraper-

iosteal plane both anteriorly as well as posteriorly (Fig. 3). The temporalis

muscle is then separated from the underlying pericranium at the level of

the temporal line. The muscle is retracted superiorly to expose 3- to 4-cm

of pericranium above the temporal line. The flap is then outlined with elec-

trocautery. The width of the flap is approximately 2- to 3-cm, with the an-

terior limit being just posterior the external auditory canal. The pericranial

portion of the flap consists of an extension 3- to 4-cm above the temporal

line deep to the temporalis muscle. The flap is elevated inferiorly using a peri-osteal elevator and electrocautery for any dense adhesions (Fig. 4). It is left

pedicled at the mastoid tip. A canal wall-down mastoidectomy is then per-

formed for eradication of the disease process. At the beginning of the mas-

toidectomy, bone pate ´ is collected from the lateral mastoid cortex using

a Sheehy Pate ´ Collector (OtoMed, Lake Havasu City, Arizona). The bone

pate ´ is kept moist and can be irrigated with antibiotic solution. At the

end of the canal wall-down procedure, the bone pate ´ is used to obliterate

the mastoid cavity (Fig. 5). The inferiorly pedicled periosteal-pericranial

flap is then used to cover the bone pate ´ (Fig. 6). The pericranial extensionof the flap allows coverage of the superior most aspect of the mastoidectomy

defect. It is critical to completely cover the bone pate ´ with the flap. Any

exposed areas of bone are covered with split thickness skin grafts. The

Koerner flap is replaced anterior to the obliterated mastoid cavity.

Surgical pearls for success with this technique include the following:

1) A well-saucerized mastoid cavity with no ridges or cavities

Fig. 1. Typical postauricular incision used for mastoid obliteration. (From Ramsey MJ,

Merchant SN, Mckenna MJ. Postauricular periosteal-pericranial flap for mastoid obliteration

and canal wall down tympanomastoidectomy. Otol Neurotol 2004;25:873–8; with permission.)

1132 MEHTA & HARRIS



2) Adequate meatoplasty

3) Split thickness skin grafting to enhance reepithelialization

4) Complete coverage of bone pate with the periosteal-pericranial flap or

fascia

5) Maximal lowering of facial ridge to level of facial nerve

6) Adequate canalplasty to remove anterior canal bulge

7) Drilling out the mastoid tip to allow the flap to smoothly lay into the

mastoidectomy defect

Although the aforementioned technique of mastoid obliteration is theworkhorse at our institution, many other techniques of mastoid obliteration

Fig. 2. Outline of the harvest site for the inferiorly based periosteal-pericranial flap. (From

Ramsey MJ, Merchant SN, Mckenna MJ. Postauricular periosteal-pericranial flap for mastoid

obliteration and canal wall down tympanomastoidectomy. Otol Neurotol 2004;25:873–8; with

permission.)

Fig. 3. Coronal section of postauricular soft tissue and bone. The postauricular incision is kept

lateral to the temporalis fascia and mastoid periosteum. (From Ramsey MJ, Merchant SN,

Mckenna MJ. Postauricular periosteal-pericranial flap for mastoid obliteration and canal

wall down tympanomastoidectomy. Otol Neurotol 2004;25:873–8; with permission.)




have been described. Palva’s original description describes a meatally based

musculoperiosteal flap in conjunction with the use of cortical bone chips and

bone pate ´

for mastoid obliteration [5,6]. Fig. 7 illustrates the use of an an-teriorly based musculoperiosteal flap. Moffat and colleagues [19] describe

the use of bone pate ´ and a superiorly based temporalis musculoperiosteal

flap for mastoid obliteration. Some authors even advocate the use of mas-

toid obliteration for canal wall-up mastoidectomy in an attempt to prevent

Fig. 4. The temporalis muscle is retracted superiorly. A periosteal elevator is used to elevate the

flap from a superior to inferior direction. (From Ramsey MJ, Merchant SN, Mckenna MJ. Post-

auricular periosteal-pericranial flap for mastoid obliteration and canal wall down tympanomas-

toidectomy. Otol Neurotol 2004;25:873–8; with permission.)

Fig. 5. Bone pate ´ is used to obliterate the mastoid; particular attention is paid to obliterate the

sinodural, retrofacial, and mastoid tip areas. (From Ramsey MJ, Merchant SN, Mckenna MJ.

Postauricular periosteal-pericranial flap for mastoid obliteration and canal wall down tympano-

mastoidectomy. Otol Neurotol 2004;25:873–8; with permission.)

1134 MEHTA & HARRIS



Fig. 6. The periosteal-pericranial flap is used to cover the bone pate ´ and line the remainder

of the cavity. The anterior aspect of the flap lies just over the facial ridge. (From Ramsey

MJ, Merchant SN, Mckenna MJ. Postauricular periosteal-pericranial flap for mastoid obliter-

ation and canal wall down tympanomastoidectomy. Otol Neurotol 2004;25:873–8; with

permission.)

Fig. 7. Schematic illustrating the use of an anteriorly based musculoperiosteal flap for obliter-

ation of the mastoid cavity. (From Smyth GDL, Toner JG. Mastoidectomy: canal wall down

techniques. In Brackman DE, Shelton C, Arriaga MA, editors. Otologic surgery. Philadelphia

(PA): WB Saunders; 1994. p. 235; with permission.)




retraction pockets and recurrent cholesteatoma [20,21]. Montandon and

colleagues [20] use cartilage to block the aditus and an abdominal fat graft

for the canal wall-up mastoidectomy cavity. Gantz and colleagues [22] de-scribe a ‘‘canal wall reconstruction’’ tympanomastoidectomy with mastoid

obliteration. Their technique consists of removal of the posterior bony canal

wall with a microsagittal saw. The mastoid cavity is obliterated with bone

pate ´ and bone chips followed by replacement of the posterior canal wall seg-

ment. An anteriorly based musculoperiosteal Palva flap is used to cover the

obliterated mastoid cavity.

Some authors describe the use of the TPFF based on the superficial tem-

poral artery for mastoid obliteration. East and colleagues and Cheney and

colleagues [23,24] describe the successful use of this robust flap for mastoidobliteration. It provides an excellent option when standard pedicled muscle

or periosteal flaps are not available as in revision cases with scar tissue or in

patients with previous irradiation. The TPFF is well vascularized, and ac-

cepts both full and split-thickness skin grafts. Figs. 8 through 10 illustrate

the use of the TPFF for mastoid obliteration.

There are numerous reports in the literature of the use of ceramic mate-

rials such as calcium phosphate ceramic granules and hydroxyapatite for

mastoid obliteration. Hartwein and colleagues [25] describe the use of hy-

droxyapatite to obliterate the mastoid bowl while reconstructing the poste-rior canal wall with autologous conchal cartilage. Yung and colleagues [26]

describe 34 cases of mastoid obliteration using hydroxyapatite granules and

Fig. 8. Dotted line outlines the TPFF supplied by the superficial temporal artery (A). (B) is the

frontal branch of the facial nerve. (From Cheney ML, Megerian CA, Brown MT, et al. Mastoid

obliteration and lining using the temporoparietal fascial flap. Laryngoscope 1995;105:1010–3;

with permission.)

1136 MEHTA & HARRIS



Fig. 9. (1) Skin/subcutaneous tissue; (2) TPFF; (3) loose areolar tissue; (4) temporalis muscle

fascia; (5) temporalis muscle. (From Cheney ML Megerian CA, Brown MT, et al. Mastoid

obliteration and lining using the temporoparietal fascial flap. Laryngoscope 1995;105:1010–3;

with permission.)

Fig. 10. Canal wall-down mastoidectomy defect filled with the TPFF with its vascular pedicle

(large arrowhead ). (From Cheney ML, Megerian CA, Brown MT, et al. Mastoid obliteration

and lining using the temporoparietal fascial flap. Laryngoscope 1995;105:1010–3; with

permission.)




an inferiorly based periosteal flap. Proponents of the use of synthetic mate-

rials such as hydroxyapatite point out the minimal resorption of these ma-

terials over time [27]. Mahendran and colleagues [28] describe the use of hydroxyapatite cement for mastoid obliteration. In their series, however,

there was a significant incidence of postoperative infection with 50% of

the patients requiring revision surgery and removal of the foreign material.

Total tympanomastoid obliteration

Total tympanomastoid obliteration consists of obliteration of the entire

mastoid, middle ear, and Eustachian tube. The indications for this proce-

dure are outlined above. Rambo [3] described a technique for total tympa-

nomastoid obliteration in cases in which there was no useful residual

hearing. He obliterated the mastoid and middle ear with temporalis muscle

followed by suturing shut the external auditory canal. As discussed later,

histopathologic studies have shown that muscle tends to atrophy with

time resulting in a cavity [29]. Our preferred technique for total tympano-

mastoid obliteration is outlined in detail. Following a canal wall-down mas-

toidectomy, this procedure involves transection of the external auditory

canal with the auricle reflected anteriorly (Figs. 11 and 12). Removal of car-

tilage on the auricular side of the external canal is then performed (Fig. 13).

As this cartilage is removed, care is taken not to buttonhole the skin. Ante-

riorly and posteriorly based skin flaps are then reflected onto the conchal

side and sutured together in an H pattern (Fig. 14). This closure is intended

to provide a water-tight seal effectively closing off the external auditory

Fig. 11. The external auditory canal is transected and the auricle is reflected anteriorly. (From

Nadol JB Jr. Chronic otitis media. In Nadol JB Jr, McKenna MJ, editors. Surgery of the ear

and temporal bone. 2nd edition. Philadelphia (PA): Lipincott Williams and Wilkins; 2005.

p. 214–6; with permission.)

1138 MEHTA & HARRIS



meatus. The meatal closure can be reinforced by additional closure of soft

tissue medially (Fig. 15). The Eustachian tube orifice is then identified in

the middle ear. It is cleaned of all mucosa and burnished with a small dia-

mond burr. The Eustachian tube is then obliterated with a plug of fascia fol-

lowed by bone wax. The tympanomastoidectomy defect is then filled with an

abdominal fat graft. If available, an inferiorly based periosteal flap, or alter-

natively, a TPFF, can be used to supplement the obliteration [17].

Fig. 12. The auricle is reflected anteriorly based on a small musculofascial pedicle. (From




Fig. 13. Cartilage is removed from the auricle side of the external auditory canal. (From







Fig. 14. The skin flaps are sutured together in an H pattern closing the external auditory me-

atus (From Nadol JB Jr. Chronic otitis media. In Nadol JB Jr, McKenna MJ, editors. Surgery

of the ear and temporal bone. 2nd edition. Philadelphia (PA): Lipincott Williams and Wilkins;

2005. p. 214–6; with permission.)

Fig. 15. The meatal closure is reinforced medially by closure of additional soft tissue (From




1140 MEHTA & HARRIS



Radiographic and histopathologic features of mastoid obliteration

Radiographic features

The radiographic features of mastoid obliteration have not been well

studied. Yung and colleagues [30] examined the value of high-resolution

CT in detecting recurrent cholesteatoma in ears that had previously under-

gone mastoid obliteration. Their study concluded that CT scanning was ef-

fective in detecting small epithelial pearls within a cavity obliterated with

hydroxyapatite. However, in cavities obliterated with muscle, CT scanning

was not as effective in detecting epithelial pearls. Further studies are needed

to evaluate the radiographic features of each of the various techniques of

mastoid obliteration and the ability to detect recurrent disease.

Histopathology

Palva [31] was the first to describe the histopathologic fate of tissues used

in mastoid obliteration. He reported on three temporal bones that had un-

dergone a meatally based musculoperiosteal flap for mastoid obliteration.

Each of the bones showed viability of the musculoperiosteal flap with

good vascularity. Linthicum [29] performed a more extensive histopatho-

logic study of 17 temporal bones that had undergone mastoid obliterationwith a variety of techniques. He concluded that fat and bone pate ´ appear

to maintain their original volume. However, muscle and subcutaneous tissue

tended to atrophy with time. Bone pate ´ used with a Palva type musculoper-

iosteal flap provided the best obliteration of a mastoid cavity.

References

[1] Mosher HP. A method of filling the excavated mastoid with a flap from the back of the

auricle. Laryngoscope 1911;21:1158–63.[2] Kisch J. Temporal muscle grafts in the radical mastoid operation. J Laryngol 1928;43:735.

[3] Rambo JHT. Primary closure of the radical mastoidectomy wound; a technique to eliminate

postoperative care. Laryngoscope 1958;68:1216–27.

[4] Popper O. Periosteal flap grafts in mastoid operations. S Afr Med J 1935;9:77.

[5] Palva T. Operative technique in mastoid obliteration. Acta Otolaryng 1973;75:289–90.

[6] Palva T. Mastoid obliteration. Acta Otolaryngol Suppl 1979;360:152–4.

[7] Dornhoffer JL. Surgical modification of the difficult mastoid cavity. Otolaryngol Head Neck

Surg 1999;120:361–7.

[8] Estrem SA, Highfill G. Hydroxyapatite canal wall reconstruction/mastoid obliteration.

Otolarngol Head Neck Surg 1999;120:345–9.

[9] D’arc MB, Daculsi G, Emam N. Biphasic ceramics and fibrin sealant for bone reconstructionin ear surgery. Ann Otol Rhinol Laryngol 2004;113:711–20.

[10] Mills RP. Surgical management of the discharging mastoid cavity. J Laryngol Otol Suppl

1988;16:1–6.

[11] Shea MC, Gardner G Jr, Simpson ME. Mastoid obliteration with bone. Otolaryngol Clin

North Am 1972;5(1):161–72.

[12] Roberson JB, Mason TP, Stidham KR. Mastoid obliteration: autogenous cranial bone pate

reconstruction. Otol Neurotol 2003;24:132–40.




[13] Kaylie DM, Horgan MJ, Delashaw JB, et al. A meta-analysis comparing outcomes of micro-

surgery and gamma knife radiosurgery. Laryngoscope 2000;110:1850–6.

[14] Gal TJ, Kerschner JE, Futran ND, et al. Reconstruction after temporal bone resection.

Laryngoscope 1998;108(4):476–81.

[15] Kim CS, Chang SO, Lee HJ, et al. Cochlear implantation in patients with a history of chronic

otitis media. Acta Otolaryngol 2004;124:1033–8.

[16] Nadol JB Jr. Cochlear implantation and implantable hearing aids. In: Nadol JB Jr,

McKenna MJ, editors. Surgery of the ear and temporal bone. 2nd edition. Philadelphia

(PA): Lipincott Williams and Wilkins; 2005. p. 355–63.

[17] Nadol JB Jr. Chronic otitis media. In: Nadol JB Jr, McKenna MJ, editors. Surgery of the ear


p. 199–218.

[18] Ramsey MJ, Merchant SN, Mckenna MJ. Postauricular periosteal-pericranial flap for mas-

toid obliteration and canal wall down tympanomastoidectomy. Otol Neurotol 2004;25:

873–8.

[19] Moffat DA, Gray RF, Irving RM. Mastoid obliteration using bone pate ´ . Clin Otolaryngol

1994;19:149–57.

[20] Montandon P, Benchaou M, Guyot JP. Modified canal wall-up mastoidectomy with

mastoid obliteration for severe chronic otitis media. ORL 1995;57:198–201.

[21] Vartiainen E, Harma R. Mastoid obliteration in intact canal wall mastoidectomy. Clin


[22] Gantz BJ, Wilkinson EP, Hansen MR. Canal wall reconstruction tympanomastoidectomy

with mastoid obliteration. Laryngoscope 2005;115:1734–40.

[23] East CA, Brough MD, Grant HR. Mastoid obliteration with the temporoparietal fascial

flap. J Laryngol Otol 1991;105:417–20.[24] Cheney ML, Megerian CA, Brown MT, et al. Mastoid obliteration and lining using the

temporoparietal fascial flap. Laryngoscope 1995;105:1010–3.

[25] Hartwein J, Hormann K. A technique for the reconstruction of the posterior canal wall and

mastoid obliteration in radical cavity surgery. Am J Otol 1990;11(3):169–73.

[26] Yung MW. The use of hydroxyapatite granules in mastoid obliteration. Clin Otolaryngol

1996;21:480–4.

[27] Gyllencreutz T. Reconstruction of the ear canal wall using hydroxylapatite with and without

mastoid obliteration and by obliteration with bone chips. Acta Otolaryngol (Stock)

1992;(Suppl 492):144–6.

[28] Mahendran S, Yung MW. Mastoid obliteration with hydroxyapatite cement: the Ipswich

experience. Otol Neurotol 2004;25:19–21.[29] Linthicum FH. The fate of mastoid obliteration tissue: a histopathological study. Laryngo-

scope 2002;112:1777–81.

[30] Yung MW, Karia KR. Mastoid obliteration with hydroxyapatitedthe value of high resolu-

tion CT scanning in detecting recurrent cholesteatoma. Clin Otolaryngol 1997;22:553–7.

[31] Palva T, Karma P, Karja J, et al. Mastoid obliteration: histopathological study of three

temporal bones. Arch Otolaryngol 1975;101:271–5.

1142 MEHTA & HARRIS



The Pathophysiology of Cholesteatoma

Maroun T. Semaan, MD, Cliff A. Megerian, MD*

Department of Otolaryngology and Head and Neck Surgery, University Hospitals

of Cleveland, Case Western Reserve University, LKS 4500, 11100 Euclid Avenue,

Cleveland, OH 44106, USA

Cholesteatoma is a cystic lesion formed from keratinizing stratified squa-

mous epithelium, the matrix of which is composed of epithelium that rests

on a stroma of varying thickness, the perimatrix. The resulting hyperkerato-

sis and shedding of keratin debris usually results in a cystic mass with a

surrounding inflammatory reaction. It may present extradurally and intra-

durally. Extradurally, cholesteatoma most commonly involves the middle

ear cleft but can occur in all portions of the petrous bone including the

mastoid, petrous apex, and external auditory canal. Intradurally, cholestea-toma, also known as epidermoid , have been described in a variety of

anatomic locations, the most common being the cerebellopontine angle.

The history of cholesteatoma has been reviewed recently [1] and is sum-

marized briefly. In 1683, Duverney [2] published the first description of what

might correspond to a cholesteatoma. He described an abscess of the bone

originating from the auditory canal that opened behind the auricle, forming

a fistula above the mastoid process, shedding the small sheets composed of

what he describes as scales. The abscess described was accompanied by

a bad odor and gave rise to what was described as grave accidents. Healso mentioned that the same process easily enters the middle ear cleft

through the auditory canal, destroying its contents and resulting in deafness.

Nearly a century and a half after Duverney’s original description, Cruveilh-

ier [3] provided in 1829 a detailed description of what he thought was an

avascular tumor originating from the cells of the subarachnoid space. Inde-

pendently, Mu ¨ ller [4] in 1838 used the term cholesteatoma as he became

aware of the presence of cholesterin and fat in what he believed to be a tu-

mor. Although, he noted the resemblance between the squamae of choles-

teatoma and the cells of the stratum corneum he did not postulate theepidermal origin of these lesions. In 1855, Virchow [5] classified


E-mail address: [email protected] (C.A. Megerian).




39 (2006) 1143–1159





cholesteatoma among squamous cell carcinomas and atheromas. However,

because these lesions grew in bone, where epidermis does not exist, he con-

sidered them as heteroplastic tumors arising from mesenchymal cells thatundergo dedifferentiation and then redifferentiation into epithelial cells.

This postulation represents the first theory suggesting that cholesteatoma

arise from mesenchymal cells undergoing metaplasia. Despite being a misno-

mer, the term cholesteatoma is still used today.

Von Troeltsch [6,7] was the first to consider the epidermal origin of cho-

lesteatoma. He theorized that epidermal debris accumulating in the external

meatus are able to cause pressure-induced osteolysis of the bony wall of the

meatus and thus invasion of the mastoid and the middle ear with extension

if unchecked into the transverse sinus and brain. Gruber [8], Wendt [9] andRokitansky [10] considered that middle ear mucosa rather than bone under-

went malpighian metaplasia in response to chronic inflammation. The des-

quamated cells developed into cholesteatoma as the passage for squamae

elimination became narrower. The theory of metaplasia became well ac-

cepted among otologists in the 19th century. At the end of the century, by

studying two different pathologic entities, Bezold [11] and Habermann

[12] proved that cholesteatoma could originate from the skin of the external

auditory meatus, which migrates into the middle ear under the influence of

chronic inflammation. Similar to normal skin, the migrated skin desqua-mated, and as the drainage passages became too narrow to enable migra-

tion, cholesteatoma forms. Habermann based his findings on the studying

patients with marginal tympanic membrane perforation after acute necrotiz-

ing otitis; Bezold, however, studied cholesteatoma formation in patients

with attic or posterosuperior retraction pockets secondary to eustachian

tube dysfunction.

Middle ear cholesteatoma occurs as two principle different entities that

share many pathological resemblances: congenital and acquired. The latter

is divided further into the more common primary acquired or attic retrac-tion pocket cholesteatoma and the secondary acquired cholesteatoma as it

occurs secondary to epithelial migration into the middle ear at the site of

a tympanic membrane perforation or iatrogenically implanted during an

otologic procedure.

In this review, we limit our discussion to middle ear cholesteatoma and

provide an updated literature review on the pathophysiology of congenital

and acquired cholesteatoma. Emphasis will be placed on the pathophysiol-

ogy of congenital and primary acquired cholesteatoma, cytokine-mediated

inflammation and bony destruction.

Congenital cholesteatoma

The first published description of a congenital cholesteatoma appeared in

1885, by Lucae [13]. Ko ¨ rner’s initial criteria [14] to distinguish acquired

from congenital cholesteatoma were revived half a century later by Derlacki

1144 SEMAAN & MEGERIAN



and Clemis [15] who reintroduced the concept of congenital cholesteatoma

in 1965. They proposed that congenital cholesteatoma be defined as a pearly

white mass behind an intact tympanic membrane in the absence of history of otitis or otorrhea, tympanic membrane perforation, or previous otologic

procedures. In 1986, Levenson and coworkers [16] suggested that the pres-

ence of prior bouts of otitis media does not necessarily exclude the presence

of congenital cholesteatoma, because this inflammatory condition is very

common among children.

The incidence of congenital cholesteatoma is 0.12 per 100,000 [17]. There

has been a recent increase in the reported incidence of this disease likely sec-

ondary to an increased awareness among pediatricians and otolaryngolo-

gists along with improvement in office based tools used for otologicexamination (ie, otomicroscopy, halogen lightening, and photodocumenta-

tion). The pathogenesis of congenital cholesteatoma sparked an active de-

bate that continues to this day. In 1936 Teed [18] described the presence

of epithelial rests in fetal temporal bones that disappeared by 33 weeks of

gestation. He postulated that the persistence of these cells leads to formation

of congenital cholesteatoma. These rests were localized in the lateral wall of

the eustachian tube in proximity of the tympanic ring in the anterosuperior

quadrant of the middle ear. These findings were confirmed later by Michaels

in 1986 [19] but failed to prove their persistence after 33 weeks of gestation.In 1998, Karmody and colleagues [20] described histologic findings of squa-

mous epithelial rest in the temporal bones of two postpartum patients. This

was the first description of these epithelial rests persisting beyond 33 weeks

of gestation. In their first patient, they described the presence of a cup-

shaped elevation of squamous epithelium with a keratin cap noted in the an-

terosuperior quadrant of the middle ear. In their second patient, a small

mass of squamous epithelium was seen embedded in the mucosa of the ante-

rosuperior quadrant of the middle ear at the junction of the columnar and

cuboidal epithelia. In their clinical study of a series of 160 congenital choles-teatoma, Potsic and coauthors [21] found that in cases of isolated quadrant

involvement, 77% were anterosuperior and 22% were posterosuperior. The

number of quadrants involved increased with age. The incidence of isolated

posterosuperior quadrant involvement appears to be higher than initially

thought.

Many theories have been proposed to explain the origin of congenital

cholesteatoma. The Teed-Michaels’ epithelial rest theory has been well ac-

cepted among otologists. Ru ¨ edi [22,23] speculated that inflammatory injury

to an intact tympanic membrane results in microperforations in the basallayer that lead to invasion of the squamous epithelium by proliferating ep-

ithelial cones through a macroscopically intact but microscopically injured

tympanic membrane. These epithelial cones fuse and expand forming a mid-

dle ear cholesteatoma. Tos [17] recently questioned the epithelial rest theory

and proposed a different explanation for the pathogenesis of this disease. He

observed that anterosuperior cholesteatoma had a frequent attachment to

1145PATHOPHYSIOLOGY OF CHOLESTEATOMA



the anterior aspect of the malleus handle or neck and that posterosuperior

cholesteatoma had an attachment to the posterior aspect of the malleus han-

dle and to the incudostapedial joint. This location was far from the anteriortympanic annulus and the lateral wall of the eustachian tube where epithelial

rests are usually found. Furthermore, he speculated that if the site of origin

was the lateral eustachian tube wall and the area anterior to the tympanic

annulus, cholesteatoma would block the eustachian tube before extending

into the tympanic cavity and the area of the malleus handle, a finding

that has not been described previously. Therefore, he argued against the ep-

ithelial rest theory and explained the pathogenesis of congenital cholestea-

toma by the acquired inclusion theory (Fig. 1). This theory speculates that

keratinized squamous epithelium may be implanted or included into thetympanic cavity during one of many pathological events affecting the tym-

panic membrane and middle ear in childhood. According to Tos, viable

Fig. 1. ‘‘Acquired’’ inclusion theory suggested by Tos. (A1, 2) The tympanic membrane re-

tracted and adherent to the malleus handle, malleus neck, or long process of the incus is loos-

ened and torn leaving a small cuff of viable keratinized epithelium adherent to the ossicles with

a small residual tear in the tympanic membrane. As the tear heals, the included epithelium leads

to formation of an inclusion cholesteatoma. (B1, 2) A tangential tear is created as the retracted

and adherent tympanic membrane is loosened from the underlying structure resulting in a rem-

nant of epithelial cells without a perforation of the tympanic membrane that results in an inclu-sion cholesteatoma. (C1, 2) Microperforations of the traumatized retracted tympanic

membrane result in invasion of the basal membrane by epithelial cones. As the ear drum is sud-

denly loosened, these cones are left behind and included in the tympanic cavity. (D1, 2) Similar

to the previous mechanism, repeated inflammation of the tympanic membrane result in prolif-

erating epithelial cones that penetrate the basal membrane and proliferate into the subepithelial

space. These cones are included in the tympanic cavity as the drum is loosened and detached

from the underlying bony structures.




keratinized epithelial cells of the retracted and adherent tympanic mem-

brane to the malleus handle, malleus neck, or the long process of the incus

are left behind after loosening of the drum and are included into the tym-panic cavity.

Four mechanisms are thought to account for the inclusion of epithelial

cells into the tympanic cavity.

1. The tympanic membrane retracted and adherent to the malleus handle,

malleus neck, or long process of the incus is loosened and torn leaving

a small cuff of viable keratinized epithelium adherent to the ossicles with

a small residual tear in the tympanic membrane. As the tear heals, the

included epithelium leads to formation of an inclusion cholesteatoma.

2. A tangential tear is created as the retracted and adherent tympanic

membrane is loosened from the underlying structure resulting in a rem-

nant of epithelial cells without a perforation of the tympanic membrane

that results in an inclusion cholesteatoma.

3. Microperforations of the traumatized retracted tympanic membrane

result in invasion of the basal membrane by epithelial cones. As the

ear drum is suddenly loosened these cones are left behind and included

in the tympanic cavity.

4. Similar to the previous mechanism, repeated inflammation of the tym-

panic membrane results in proliferating epithelial cones that penetrate

the basal membrane and proliferate into the subepithelial space. These

cones are included in the tympanic cavity as the drum is loosened and

detached from the underlying bony structures.

In response to Tos’ observations, Liang and coauthors [24] performed an

immunohistochemical analysis of 36 temporal bones of 19 fetuses aged be-

tween 6 gestational weeks to 15 months postpartum. The investigators ob-

served in each of the 22 temporal bones aged 16 gestational weeks to 8

months postpartum at least one epidermoid formation with a total of 116.The majority were found in the middle ear epithelium in the anterosuperior

annular region of the tympanic cavity with a small number of epidermoid

formations seen in the posterosuperior, anteroinferior, and posteroinferior

region of the lateral wall in the vicinity of the annular zone. In addition,

Liang and colleagues [24] examined the differential expression of 34bE12,

a cytokeratin antigen expressed by the external ear epidermis and the pseu-

dostratified columnar epithelium at all gestational ages, and 35bH11, a cyto-

keratin antigen expressed by pseudostratified columnar and simple cuboidal

epithelium used to characterize the epidermoid formation seen in temporalbones histological sections. In addition, they used antibodies to antilym-

phoid enhancing factor-1 (LEF-1), a marker expressed by embryonic epi-

dermis, to analyze the epidermoid formation precursor previously

described by Michaels [19,25]. All epidermoid formations seen in their study

stained positive for epidermal cytokeratin. The epidermoid formation pre-

cursor found in both temporal bones of an embryo aged 6 gestational weeks




did not stain for LEF-1. Thus, they concluded that the epidermoid forma-

tion precursor initially reported is likely the result of a tangential cut arti-

fact of a thickened actively growing epithelial bud from the tip of thetubotympanic recess. Microscopically, they observed that as the anterosu-

perior tip of the meatal plate (precursor of the pars tensa) develops, by ges-

tational week 12, the epidermal interface becomes jagged, and by

gestational week 16, epidermal cells become encroached onto the fibroblasts

of the bilaminar collagen layer. As the fibroblasts become more condensed,

small clumps of epidermal cell become trapped within the condensed bila-

minar collagen layer.

Despite improvements in our understanding, the pathophysiology of con-

genital cholesteatoma continues to be controversial and actively debated.Furthermore, many questions remain unanswered. These questions pertain

to the biological factors that predict aggressiveness, growth, and recidivism

of middle ear congenital cholesteatoma (Fig. 2).

Acquired cholesteatoma

Primary acquired cholesteatoma

The pathophysiology of acquired cholesteatoma is similarly controver-sial. As previously eluded to, the precise pathogenesis of cholesteatoma

has been debated for more than two centuries. Four predominant theories

have fueled the debate: (1) invagination, (2) basal cell hyperplasia or papil-

lary ingrowth, (3) metaplasia, and (4) epithelial invasion.

The invagination theory is currently regarded as one of the primary

mechanism of the formation of primary acquired attic cholesteatoma.

Anatomic or pathological conditions that predispose to eustachian tube

Fig. 2. Site of origin and patterns of spread of congenital cholesteatoma according to (A) Tos

‘‘acquired’’ inclusion theory and (B) Teed-Michael’s epidermal rest theory.




dysfunction result in barometric perturbation of the middle ear space. Im-

paired ventilation secondary to a dysfunctional eustachian tube leads to neg-

ative middle ear pressure. The negative pressure is the culprit for structuralweakening of the tympanic membrane and development of retraction

pockets. The pars flaccida, having the weaker structural support, is the

most common site of formation of a retraction pocket. Sade [26] and

Sade and Halevy [27] described four stages of tympanic membrane retrac-

tion: stage I, retracted membrane; stage II, retraction onto the incus; stage

III middle ear atelectasis; and stage IV, adhesive otitis media. The geomet-

rical changes attributed to progressive retraction lead to narrowing of the

anatomic passages and impairment of the epithelial migration and cleaning

of the keratin debris. As the pocket deepens and insinuates between mucosalfolds and crevices, it becomes non–self cleaning and leads to accumulation

of keratin debris (Fig. 3) Bacterial proliferation and super-infection of the

accumulated debris form a biofilm that leads to chronic infection and

epithelial proliferation. The latter appears to be influenced by the cytokine-

mediated inflammatory response. Chole and Faddis [28], analyzed the pres-

ence of biofilm matrix in cholesteatoma debris of 22 surgically induced

Mongolian gerbils and 24 human specimens. The investigators detected the

amorphous polysaccharide matrix suggestive of biofilm formation in 21 of 22

animals and 16 of 24 human cholesteatoma. Recently, Wang and coworkers[29] found that otopathogenic strains of pseudomonas aeruginosa are

Fig. 3. Mucosal compartmentalization of the middle ear. The mucosal folds of the middle ear

cleft define the spaces that limit the boundaries of the retraction pockets. Knowledge of theiranatomy helps understand the formation and extension of primary acquired cholesteatoma

(black arrows). (1) superior incudal fold, (2) superior malleolar fold, (3) lateral incudal fold,

(4) anterior malleolar fold, (5) lateral malleolar fold, (6) posterior malleolar fold. ET, eustachian

tube orifice; HAC, hypotympanic air cells; RW, round window niche. Eustachian tube dysfunc-

tion results in formation of a retraction pocket. Often, a pars flaccida retraction pocket is

formed (star). As the pocket deepens and insinuates between folds, the self-cleaning mechanism

is altered and keratin accumulates.




capable of producing biofilm and become highly resistant to antimicrobial

therapy. These findings strongly suggest a role of bacterial biofilm in the

pathogenesis of cholesteatoma.The experimental model illustrating the implication of eustachian tube

dysfunction in the formation of retraction pockets and later cholesteatoma

was described by Kim and Chole [30]. By ligating the eustachian tube of

Mongolian gerbils, the investigators succeeded in creating an induced, sur-

gical model of primary acquired cholesteatoma (Fig. 4).

The exact mechanism and triggers that lead to development of an active

cholesteatoma in some patients with an attic retraction pocket while others

continue to have a quiescent and self-cleaning pocket remain unclear. It has

been shown recently that the combination of tympanic membrane retractionand basal cell proliferation is the hallmark for cholesteatoma formation and

development.

In a cohort of healthy children age 5 to 16 years, the prevalence of attic

retractions was between 14% and 25% of ears [31]. In a separate cohort of

children treated for secretory otitis with pressure equalization tube insertion

with or without adenoidectomy and followed up to 18 years, the incidence of

severe retractions (behind the scutum with some bone resorption) was 5% to

6% and attic cholesteatoma was 0.2% to 1.7%. Sudhoff and Tos [31] per-

formed immunohistochemical analysis of surgical specimens obtainedfrom 14 patients with middle ear cholesteatoma. In their clinical study,

they compared the expression of MIB-1, a marker of cellular proliferation,

between the cholesteatoma content and the normal external auditory canal

skin. In addition, the investigators analyzed the integrity of the basement

membrane by using avidin biotin complex peroxidase to stain collagen

type IV. At the level of the basement membrane, interruption in the

Fig. 4. Patterns of spread of primary acquired cholesteatoma from an attic retraction pocket

(D). (A) Antrum, most common; (B) posterior mesotympanum, second most common; and

(C ) anterior mesotympanum, least common.




continuity was seen at the cholesteatoma–lamina propria interface, whereas

the integrity was preserved in the adjacent normal auditory canal skin. They

also showed an increased expression of MIB-1 in the keratinocytic popula-tion of the basal cell layer. This increased expressivity was consistent with

proliferating keratinocytes localized primarily in small epithelial cones or

pseudopods growing into the subepithelial stroma through interruptions

of the basement membrane. Their observation provides experimental evi-

dence that support the implication of both the retraction and basal cell hy-

perplasia theories. They postulated that in the initial retraction pocket stage,

the epithelial migratory pattern is maintained until the pockets deepen and

the drainage pathways become small leading to keratin debris accumulation.

As the debris becomes infected, the bacterial proliferation and resultant in-flammation leads to an influx of inflammatory cells and production of cyto-

kines. This progression along with local release of collagenases created

breaks in the basement membrane allowing the formation of epithelial cones

that grow toward the stroma (papillary ingrowth theory). The combination

of subepithelial invasion and keratinocytic proliferation in the form of

microcholesteatoma is the hallmark of the precholesteatomatous stage of

cholesteatoma.

As the microcones expand and fuse together, an attic cholesteatoma is

formed. Using the normal postauricular skin as control, Albino and co-workers [32] found a nine- to 20-fold increase in the expression of p53 in

cholesteatoma tissue, trough all epithelial layers. The p53 proteins by acti-

vating downstream products (p21/WAF1, GADD45, and mdm2) appear

to have a role in the down-regulation of cellular proliferation and promo-

tion of apoptosis [33], a checkpoint control mechanism to protect the cell

from genetic alterations. Similarly, they noted a two-fold increase in the ex-

pression of Ki-67, a marker of cellular proliferation, in cholesteatoma tissue

compared with control normal postauricular skin. According to Albino

and coauthors [34], the increased p53 expression was a feedback negativeresponse to control an increased proliferative state as witnessed by the

increase expression of Ki-67.

Using immunohistochemistry, Kim and coworkers [35], analyzed the pat-

tern of cellular proliferation and epithelial migration in the Mongolian ger-

bil animal model. They showed an increase in the expression of cytokeratin

(CK) 13/16, markers of epidermal cell proliferation, in the expanding part of

the cholesteatoma and to a lesser degree an increase in the expression of CK

5/6 and CK 1/10, markers of epithelial migration. They concluded that cel-

lular migration (or invasion) and proliferation play a role in the expansionof cholesteatoma.

On the other hand, Olszewska and coauthors [36], by studying the expres-

sion of five different cytokeratin (CK 10, CK 14, CK 18, CK 19 and 34bE12)

concluded that congenital and acquired cholesteatoma exhibit a similar ex-

pression pattern. These findings suggested that the so-called ‘‘acquired’’ cho-

lesteatoma in children may be an advanced congenital cholesteatoma that




resulted in destruction of the tympanic membrane, erosion of the ossicular

chain, and invasion of the mastoid cavity.

Epithelial invasion by cholesteatoma appears to be an important charac-teristic of this disease. Cholesteatoma expand by invading into surrounding

middle ear soft tissue structures and bone. It remains unclear to what factors

predict the biologic behavior of these lesions, such as recidivism and a more

aggressive clinical course.

Mallet and colleagues [37] found a correlation between the aggressiveness

of the clinical behavior of cholesteatoma and the index of proliferation. In

their analysis of surgical specimen from 91 ears with cholesteatoma, MIB-1

was detected in 23% of the ears with moderate bony destruction (single os-

sicle affected) versus 56% of ears with severe bony destruction (two or moreossicles, meningeal exposure, denudation of the facial nerve or sigmoid

sinus, and erosion of the lateral semicircular canal). These findings were

statistically significant. Young age was found to be a predictor of aggressive-

ness as witnessed by a higher proliferative index in children.

Tokuriki and coworkers [38] performed gene expression analysis on hu-

man middle ear cholesteatoma using complementary DNA arrays. They

compared the expression pattern of eight cholesteatoma to normal postaur-

icular skin samples. They found an upregulation or induction in genes

involved in cellular proliferation and differentiation (calgranulin A, calgra-nulin B, psoriasin, thymosin b-10) and cell invasion (cathepsin C, cathepsin

D, cathepsin H, and matrix metalloproteinase 9 [MMP-9]). These results

were confirmed using reverse transcriptase-polymerase chain reaction

(RT-PCR) analysis.

Immunohistochemical analysis showed increased expression of calgranu-

lin A, calgranulin B, and calgranulin D in the cytoplasm of all cell layers of

the cholesteatoma epithelium. Calgranulin proteins belong to the S100 pro-

tein family. In epithelial cells they may be involved in Ca2þ- dependent re-

organization of cytoskeletal filaments [39]. Psoriasin, also a member of theS100 protein family, has been shown to be increased in hyperproliferative

and inflammatory skin conditions and are believed to play a role in kerati-

nocytic differentiation [40]. Upregulation and induction of these genes may

reflect an alteration in keratinocyte differentiation and migration leading to

keratin overproduction and accumulation as seen in cholesteatoma. The ca-

thepsin family is a group of lysosomal proteases that play a key role in the

degradation of intracellular and extracellular proteins in the epidermis and

have been shown to contribute to the invasive properties of some neoplasms

[41]. Cathepsin B has been shown to play a role in the osteolysis seen in cho-lesteatoma [42].

The increased keratinocyte proliferation is coupled with an increased cell

death resulting in the production of larger amount of keratin debris respon-

sible for the expansion and keratin accumulation seen in cholesteatoma. The

implication of apoptotic cell death has been demonstrated recently [43]. Cas-

pase-8 activation, a known effector of the extrinsic pathway of apoptosis, is




triggered by activation of the cell surface death receptors (tumor necrosis

factor [TNF] family, Fas-L/Fas-R). This activation results in the activation

of an end product of apoptosis, caspase-3, that induces the nuclear translo-cation of effector molecules that result in apoptosis and programmed cell

death. The transcription factor nuclear factor (NF)-kB is a known key me-

diator of the TNF-mediated cellular response. NF-kB proteins are intracy-

tosolic and are inactivated by IkB-a (an inhibitory protein). The inactivation

of IkB-a activates NF-kB and results in nuclear translocation of the tran-

scription factor. The activation of NF-kB suppresses apoptosis induced by

TNF-a. Miyao and coauthors [43] found an increased expression of cas-

pase-3 localized to the granular and spinous layers of the cholesteatoma ep-

ithelium and an increased expression of caspase-8 confined to the granularlayer. The retroauricular skin was used as a control. The NF-kB proteins

were localized in the perinuclear region suggesting that the mechanism of

negatively controlling apoptosis was inactivated leading to keratinocyte

cell death and keratin accumulation.

These findings strongly suggest differential properties inherent to choles-

teatoma compared with normal epidermal keratinocytes that may explain

their clinical aggressiveness and behavior responsible for the expansion,

bony destruction and recidivism. Numerous studies have confirmed the im-

plication of invagination, basal cell hyperplasia, and invasion in the patho-genesis of primary acquired cholesteatoma. The exact inciting events and

factors responsible for the genesis and progression of middle ear cholestea-

toma remain unclear, and further research is warranted to help elucidate

these missing links.

Secondary acquired cholesteatoma

Secondary acquired cholesteatoma has been described to occur as the

result of the migration of tympanic membrane epidermis into the middleear at the site of a marginal perforation or as the result of the implan-

tation of viable keratinocytes into the middle ear cleft. The implantation

occurs during a blast injury to the tympanic membrane leaving keratino-

cytes behind a healed perforation, at the site of a temporal bone fracture,

or as the result of an iatrogenic introduction of these cells. The latter

have been described to occur in various otologic surgeries such as stape-

dectomy, tympanoplasty, pressure equalization tube placement, and mid-

dle ear exploration.

Wolf and coauthors [44] described the otologic findings in 210 ears from147 soldier-patients that sustained blast injuries with perforation of tym-

panic membrane localized to the pars tensa. These investigators reported

an incidence of 4.8% of invasive cholesteatoma. Freeman [45] reported three

cases of cholesteatoma secondary to temporal bone fracture. The keratino-

cytes appear to have invaded into the middle ear cleft through the fracture

sites.




Golz and coauthors [46] performed a retrospective analysis of 2829 chil-

dren who underwent a ventilation tube placement between 1978 and 1997.

These investigators noted an incidence of 1.1% of middle ear cholesteatomaattributed to the insertion of the pressure equalization tube. The presence of

cholesteatoma around the tube site was a prerequisite to incriminate the

procedure as a cause of the cholesteatoma. They also noted a higher inci-

dence in children aged less than 5 years, those with placement of Goode

T-tubes, children with frequent reinsertions, patients with duration of place-

ment exceeding 12 months, and ears with history of frequent postoperative

otorrhea. Ferguson and coworkers [47] described the reasons for cholestea-

toma formation after a stapedectomy. The investigators described four

mechanisms: prosthesis extrusion independent of eustachian tube dysfunc-tion, inadvertent implantation of keratinocytes with the oval window fat

graft, malpositioned inverted tympanomeatal flap, and migration at the

site of a marginal tympanic membrane perforation.

Eavey and coworkers [48], and Camacho and colleagues [49] were able to

produce viable keratinocytes in the bulla of gerbils and chinchilla, respec-

tively, by implanting the mastoid space with autogenous keratinocytes ob-

tained from the conchal surface of the pinna. Production of new keratin

was observed up to 9 months postimplantation. Various histopathologic

changes ranging from granulation tissue to cholesteatoma formation weredescribed. The investigators concluded that neonatal aspiration of lanugo

and viable keratinocytes can result in middle ear inflammation that in the

chronic stage can lead to cholesteatoma formation. Bernal-Sprekelsen and

coworkers [50], argued against this model of implantation of keratinocytes

as an etiology for cholesteatoma and failed to find keratinizing epithelial

cells in 31 temporal bones of infants who died before 1 year of age and

27 temporal bones of preterm fetuses that succumbed to various conditions.

Despite the fact that the neonatal aspiration of viable keratinocytes may not

fully account for the development of congenital cholesteatoma, it providesa valuable experimental platform that the implantation of viable keratino-

cytes can lead to formation of middle ear or mastoid cholesteatoma. This

is observed frequently in revision middle ear surgery and described as

a ‘‘cholesteatomatous pearl’’ formation that is the result of a trapped viable

keratinocytic formation that leads to a small localized cholesteatoma.

Another experimental model recently described by Massuda and Oliveira

[51] provides physiopathologic evidence that supports epithelial migration at

the edges of a tympanic membrane perforation as a possible cause for cho-

lesteatoma development. By creating a tympanic membrane perforation andlatex with 50% propylene glycol, the investigators succeeded in producing

cholesteatoma in 90% and 80% of their animals, respectively. They con-

cluded that latex provides a biomembrane that favors neoangiogenesis

and forms a bridge for epithelial migration. This environment is enhanced

further by a cytokine-producing acute or chronic inflammatory milieu cre-

ated by the inciting material. This model may provide evidence that




epithelial migration of keratinizing epithelium at the site of a tympanic

membrane perforation, in the setting of recurrent inflammatory events,

may be the culprit for cholesteatoma formation.

Mechanism of bone destruction

The ongoing debate on the pathogenesis of cholesteatoma is paralleled by

the ongoing research to help elucidate the mechanism of expansion, bone

destruction and invasion seen in middle ear cholesteatoma. Two predomi-

nant mechanisms are believed to account for the osteolysis seen in middle

ear cholesteatoma: pressure-induced bone resorption and enzymatic dissolu-

tion of bone by cytokine-mediated inflammation. Pressure necrosis initiallydescribed by Steinbru ¨ gge in 1879 and Walsh in 1951, and direct bone resorp-

tion as described by Chole and coworkers [52] in 1985 have been proposed

as possible mechanisms of bone destruction. Chole and colleagues im-

planted silicone sheets in the middle ear of gerbils without cholesteatoma

and noted bone resorption at the pressure sites. They estimated that pres-

sures of 50 to 120 mm Hg resulted in osteoclastic-induced bone resorption.

The interaction of osteoclasts and osteoblasts to extrinsic biomechanical

factors is a well-documented biological response [53,54].

It is uncertain to what degree the pressure-induced activation of osteo-clasts play a role in the osteolysis seen in cholesteatoma. Enzymatic-induced

and cytokine-induced bone destruction has been studied in the last two de-

cades. Matrix metalloproteinases (MMP), a family of zinc metalloenzymes

that degrades unmineralized extracellular matrix, have been shown to be

present in the cholesteatoma [55]. MMP-2 (72 kD collagenase) and MMP-9

(92 kD collagenase) were expressed in suprabasal epithelial layers of

cholesteatoma.

Other investigators found the increased expression of MMP-9 but not

MMP-2 in cholesteatoma cells [56]. Schmidt and coworkers [56] analyzedthe in vivo significance of MMP-9 activity in relation to the production of cy-

tokines interleukin (IL)-1a, IL-1b, TNF-a, transforming growth factor

(TGF)-b, and epidermal growth factor (EGF) in tissue homogenates of 37

cholesteatoma and nine external ear skin specimens. IL-1a production was

found to be significantly elevated; however, no correlation was found between

MMP-9 activity and cytokine production. IL-1 and IL-8, important intercel-

lular mediators of osteoclastic activities have been shown to increase in cul-

tured cholesteatoma cells compared with normal external auditory canal skin.

The role of another important cytokine, TNF-a, has also been found.Yan and coauthors [57] found that by in vitro stimulating monocytes,

they were able to produce multinucleated cells with osteoclastlike activity

that produced acid phosphatase-induced bone demineralization. The

amount of osteolysis was increased by adding osteoblasts to the TNF-a –

treated osteoclasts containing medium, suggesting a cell to cell interaction

mediated by TNF-a. In addition, the latter enhanced the production of




collagenases by macrophages and osteoblasts. However, by performing en-

zyme-linked immunosorbent assay on tissue samples from 23 patients with

cholesteatoma and 16 patients with chronic otitis without cholesteatoma,the detection of IL-1a, TNF-a, and EGF was significantly higher in the cho-

lesteatoma samples [58].

Recent histopathologic evidence was obtained from the temporal bone of

two patients with ruptured cholesteatoma sac resulting in local inflamma-

tion and osteolysis [59]. These changes were associated with a small abscess

formation at the site of the rupture. They noted a marked inflammatory

cellular infiltrate surrounding the rupture site with evidence of epithelial

proliferation at the lining of the perforation site.

Recent work by Jung and coworkers [60] showed the possible role of ni-tric oxide as an important mediator of osteoclast function. Using in vivo

analysis of a murine model of cholesteatoma-induced bone resorption and

in vitro analysis of osteoclast culture, the investigators studied the gene ex-

pression of nitric oxide synthase (NOS) and the effect of aminoguanidine (an

inhibitor of cytokine mediated nitrite production). They showed a selective

upregulation of the inducible NOS or NOS II compared with NOS I and III

and a dose-dependent stimulation of osteoclastic activity (not proliferation)

using low concentration of nitric oxide donors (sodium nitroprusside and

S-nitro-N -acetyl-D, L-penicillamine). In vitro, only interferon (IFN)-g (notIL-1b or TNF-a) was able to generate nitrite. This nitrite production was

blocked in vitro by the addition of aminoguanidine (but not in vivo) and

was synergistically enhanced in the presence of IFN-g, IL-1b, and TNF-a.

These findings indicate a role for nitric oxide in the osteoclastic-mediated

bone resorption in cholesteatoma and suggest the implication of additional

cytokines in the in vivo osteoclastogenesis and bone resorption. In contrast

to the increased osteoclastic activity without increase in the number of oste-

oclasts seen by Jung and colleagues [60], in a separate study, Hamzei and co-

authors [61] found an increase in the number of the osteoclast precursor cells inthe perimatrix of 21 cholesteatoma surgically obtained. These studies high-

light the importance of osteolysis and its regulatory mechanisms in the bone

destruction seen in middle ear cholesteatoma that results in significant mor-

bidity and mortality.

Summary

The pathophysiology of cholesteatoma continues to be debated widely.

Cholesteatoma is classified as congenital or acquired. Recent studies appear

to favor a possible common origin and overlap in the pathophysiology be-tween both entities. Despite the growing evidence that the genesis, expan-

sion, and progression of cholesteatoma is a complex interaction between

anatomic, inflammatory, and regulatory factors of cellular proliferation

and differentiation, the exact mechanism responsible for the invasion, recid-

ivism, and destruction seen in this disease remains unknown.




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Cartilage Tympanoplasty

John L. Dornhoffer, MDa,b,c,*aDivision of Neurotology, Department of Otolaryngology/Head and Neck Surgery,

University of Arkansas for Medical Sciences, 4301 West Markham,

Slot 543 Little Rock, AR 72205, USAb

ENT Clinic and Audiology Services, University of Arkansas for Medical Sciences,4301 West Markham, Slot 543 Little Rock, AR 72205, USA

cDepartment of Neurobiology and Developmental Sciences, University of Arkansas

for Medical Sciences, 4301 West Markham, Slot 543 Little Rock, AR 72205, USA

The use of cartilage in middle ear surgery is not a new concept and has

been recommended on a limited basis to manage retraction pockets for

many years [1–5]. More recently, however, it has been described increasingly

for the reconstruction of large portions of the pars tensa of the tympanic

membrane in cases of recurrent perforation, atelectasis, and cholesteatoma[6–8]. Although one might anticipate a significant conductive hearing loss

with cartilage owing to its thickness and rigidity, several studies have

reported results to the contrary, suggesting hearing results with cartilage

to be no different than those for fascia. It has been shown in both

experimental and clinical studies that cartilage is well tolerated by the mid-

dle ear, and long-term survival is the norm [9–12]. It appears that cartilage

grafts are nourished largely by diffusion and become well incorporated in

the tympanic membrane (TM) [3]. Human and animal studies [13,14] have

found that although some softening occurs with time, the matrix of thecartilage remains intact, but with empty lacunae, showing degeneration of

the chondrocytes. Cartilage retains its rigid quality and resists resorption

and retraction, even in the milieu of continuous eustachian tube

dysfunction.

Two distinct techniques are described for cartilage reconstruction of the

tympanic membrane: the perichondrium/cartilage island flap, which uses

tragal cartilage, and the palisade technique, which uses cartilage from the

tragus or cymba. The choice of technique is dictated typically by the specific

* Department of Otolaryngology/Head and Neck Surgery, University of Arkansas for

Medical Sciences, 4301 West Markham, Slot 543 Little Rock, AR 72205.





39 (2006) 1161–1176





middle ear pathology or, in cases in which the TM reconstruction is in con-

junction with ossiculoplasty, the status of the ossicular chain. The palisade

technique is preferred in cases of cholesteatoma and when ossicular recon-struction is needed in the malleus-present situation. The perichondrium/car-

tilage island flap is preferred for management of the atelectatic ear and the

high-risk perforation. This chapter describes the two techniques in detail,

followed by descriptions of the modifications that should be taken in re-

sponse to specific surgical indications.

Techniques for reconstruction

The perichondrium/cartilage island flap

The general technique of reconstruction using the perichondrium/carti-

lage island flap begins with harvest of the cartilage from the tragal area

[15]. This cartilage is ideal because it is thin, flat, and in sufficient quantities

to permit reconstruction of the entire TM. The cartilage is used as a full-

thickness graft and is typically slightly less than 1 mm thick in most cases.

Although it has been suggested that a slight acoustical benefit could be ob-

tained by thinning the cartilage to 0.5 mm [16], this advantage is offset by

the unacceptable curling of the graft, which occurs when the cartilage isthinned and the perichondrium is left attached to one side.

An initial cut through skin and cartilage is made on the medial side of the

tragus, leaving a 2-mm strip of cartilage in the dome of the tragus for cosm-

esis (Fig. 1). The cartilage, with attached perichondrium, is dissected medi-

ally from the overlying skin and soft tissue by spreading a pair of sharp

scissors in a plane that is developed easily superficial to the perichondrium

on both sides. It is necessary to make an inferior cut as low as possible to

maximize the length of harvested cartilage. The cartilage is then grasped

and retracted inferiorly, which delivers the superior portion from the inci-sura area. The superior portion is then dissected out while retracting, which

Fig. 1. Harvest of cartilage, leaving small rim of cartilage in dome for cosmesis (right ear).

1162 DORNHOFFER



produces a piece of cartilage typically measuring 15 mm 10 mm in chil-

dren and somewhat larger in adults.

The perichondrium from the side of the cartilage furthest from the earcanal is dissected off, leaving the thinner perichondrium on the reverse

side. A perichondrium/cartilage island flap is then constructed as described

previously [15]. In short, using a round knife, cartilage is removed to pro-

duce an eccentrically located disc of cartilage about 7 mm to 9 mm in diameter

for total TM reconstruction. A flap of perichondrium is produced posteri-

orly that will eventually drape over the posterior canal wall. A complete

strip of cartilage 2 mm in width is then removed vertically from the center

of the cartilage to accommodate the entire malleus handle (Fig. 2). The cre-

ation of two cartilage islands in this manner is essential to enable the recon-structed TM to bend and conform to the normal conical shape of the TM.

When the ossicular chain is intact, an additional triangular piece of cartilage

is removed from the posterior-superior quadrant to accommodate the incus.

This excision prevents the lateral displacement of the posterior portion of

the cartilage graft that sometimes occurs because of insufficient space between

the malleus and incus.

The entire graft is placed in an underlay fashion, with the malleus fitting

in the groove and actually pressing down into and conforming to the peri-

chondrium, as shown in Fig. 3. The cartilage is placed toward the promon-tory, with the perichondrium immediately adjacent to the TM remnant,

both of which are medial to the malleus. Failure to remove enough cartilage

from the center strip will cause the graft to fold up at the center instead of

lying flat in the desired position. Likewise, if the strip is insufficient, the car-

tilage may be displaced medially instead of assuming a more lateral position

in the same plane as the malleus.

Gelfoam (Upjohn Laboratories, Kalamazoo, Michigan) is packed in the

middle ear space underneath the anterior annulus to support the graft in this

area, and the posterior flap of perichondrium is draped over the posterior

Fig. 2. Prepared perichondrium/cartilage island graft shows strip of cartilage removed to facil-

itate malleus.

1163CARTILAGE TYMPANOPLASTY



canal wall. Middle ear packing is avoided on the promontory and in the vi-

cinity of the ossicular chain. One piece of Gelfoam is placed lateral to the

reconstructed TM, and antibiotic ointment is placed in the ear canal (Fig. 4).

The Palisade technique

When the palisade technique is used for reconstruction of the TM, carti-

lage can be harvested from either the tragus or the cymba. Cartilage fromthe cymba area of the conchal bowl is used if the surgical approach involves

a postauricular incision. Tragal cartilage is used if the approach is transca-

nal or endaural. The cartilage of the cymba is similar to the tragus in that it

has an acceptable thickness of about 1 mm compared with other areas of the

concha, which are thicker and irregular. It is different, however, in that it is

curved, making it difficult to create a perichondrium/cartilage island flap

suitable for reconstruction of the entire TM.

Fig. 3. Lateral line drawing shows proper placement of graft.

Fig. 4. Postoperative ear with perichondrium/cartilage island graft (left ear).

1164 DORNHOFFER



The cartilage is cut into several slices that subsequently are pieced to-

gether, like the pieces of a jigsaw puzzle, to reconstruct the TM (Fig. 5). Be-

cause of the nature of the reconstruction, it is not necessary to have onelarge, flat piece of cartilage, and the more curved cymba cartilage is suitable

(Fig. 6). A large area of conchal eminence can be exposed by elevating the

subcutaneous tissue and postauricular muscle from the conchal perichon-

drium. The cymba cartilage is the prominent bulge at the superior aspect

of the concha (Fig. 7). A circumferential cut the size of the anticipated graft

is made through the perichondrium and cartilage but not through the ante-

rior skin. The perichondrium is removed from the postauricular side, and

the cartilage, with the perichondrium on the anterior aspect, is dissected

from the skin. This technique is also used for harvesting cartilage for canalwall reconstruction when the retrograde mastoidectomy technique is used

for cholesteatoma surgery.

The technique described here differs somewhat from the palisade tympa-

noplasty of Heermann and coworkers [17]. Instead of placing rectangular

strips of cartilage side to side, an attempt is made to cut one major piece

of cartilage in a semilunar fashion, which is placed directly against the mal-

leus on top of the prosthesis (Fig. 8A, B). This acts to reconstruct a major

portion of the posterior half of the TM and serves as a foundation for the

rest of the cartilage pieces. A second semilunar piece is placed betweenthis first piece and the canal wall to reconstruct the scutum precisely

(Fig. 8C). Any spaces that result between this cartilage and the canal wall

or scutum are filled in with small slivers of cartilage to prevent prosthesis

extrusion and recurrent retraction (Fig. 8D). The reconstruction is then cov-

ered with the previously harvested perichondrium draped over the posterior

canal wall (Fig. 9).

Although this technique can be used for TM reconstruction without os-

sicular reconstruction, it is favored when ossiculoplasty is performed in

Fig. 5. Schematic of palisade technique (right ear).




a malleus-present situation and is especially suitable for cholesteatoma sur-

gery. Because the prosthesis is placed before the cartilage reconstruction,

this technique allows direct visualization and contact of the notched pros-thesis to the manubrium handle, which has been shown to provide superior

hearing results [18]. The prosthesis acts as scaffolding on which the cartilage

is placed, which serves to reconstruct the TM as well as prevent prosthesis

extrusion. It likewise allows a precise and water-tight fit between the recon-

structed TM and the canal wall in the posterior area, where recurrent cho-

lesteatoma most frequently occurs. Typically, in these situations, the anterior

half of the TM is not altered or is grafted with conventional materials to allow

cholesteatoma surveillance and possible intubation in the postoperative

period if necessary.

Fig. 6. Schematic illustrating location of cymba cartilage (left ear).

Fig. 7. Harvesting of cymba cartilage with postauricular incision (right ear).

1166 DORNHOFFER



Postoperative care

At 1 to 2 weeks postsurgery, the packing material of Gelfoam and anti-

biotic ointment is completely suctioned from the external canal. Antibiotic

steroid-containing drops are used for an additional 2 weeks to clear the

Fig. 9. Postoperative appearance of TM after palisade reconstruction (right ear).

Fig. 8. Series shows sequence of palisade reconstruction (left ear). (A) TORP in place. (B) Ini-

tial cartilage placement. (C ) Reconstruction of the scutum. (D) Reconstruction of remainder of

posterior TM.




ear of residual ointment and Gelfoam, the latter of which can lead to gran-

ulation and fibrous tissue formation if inadequately removed from the TM.

The adult patient is instructed to begin the Valsalva maneuver, and childrenare instructed to use the Otovent (Invotec International, Jacksonville, Flor-

ida), three times a day beginning 2 to 3 weeks after the surgery.

A postoperative audiogram is obtained 6 to 8 weeks later, at which time the

TM is examined. It should be mentioned that impedance tympanometry is un-

reliable after cartilage tympanoplasty and will generally yield a low-volume,

type B tympanogram in most cases owing to the noncompliant nature of

the graft, despite normal hearing. It is necessary to check air and bone conduc-

tion after the surgery and use the hearing levels to determine whether effusion

is present. If the hearing result is good and the TM is clear, the ear is examinedat 6 months and again 1 year from the date of surgery. If effusion is present

based on observation or conductive hearing loss, nasal steroids are added,

the Valsalva (Otovent) is encouraged, and the ear is examined at 3 months.

If there is a question as to whether effusion is present in a case of postoperative

conductive hearing loss owing to the opacity of the TM, a computed tomog-

raphy scan is sometimes necessary to assess the status of the middle ear. If the

effusion is still present at that time, the ear is intubated. This can be difficult if

a total cartilage reconstruction was performed. In such a situation, a CO2 laser

myringotomy is performed, followed by insertion of a soft tube, such as theGoode T-tube (Xomed Surgical Products, Jacksonville, Florida).

Specific indications and subsequent modifications to the surgical technique

The high-risk perforation

The high-risk perforation comprises a revision surgery, a perforation an-

terior to the annulus, a perforation draining at the time of surgery, a perfo-

ration larger than 50%, or a bilateral perforation, all of which have beenshown to be associated with increased failure rates using traditional tech-

niques. In these cases, cartilage has proven extremely valuable for recon-

struction of the TM.

Revision tympanoplasty has been shown by many to be a risk factor for

subsequent failure in graft take and hearing [19,20], and drainage at the time

of surgery is considered by many to represent a negative prognostic factor

[19,21]. However, although every attempt is made to dry an ear before sur-

gical intervention, it is not considered a prerequisite for tympanoplasty.

Cartilage is used in this situation and has proven to yield successful results.Cartilage is likewise used for reconstruction when the size of the perforation

is larger than 50%, although the larger perforations fair less well [22].

Age as a prognostic factor in cartilage tympanoplasty of high-risk perfo-

rations is somewhat controversial. In previous studies, a young patient age

(!18 years) was not found to have a negative influence on success [15,23].

There is, however, an association between young age and other significant

1168 DORNHOFFER



factors, such as bilateral ear disease and drainage at the time of surgery,

which are associated with immature tubal function. The general approach

to pediatric patients is to avoid repairing the TM during the otitis-proneyears (!3 years). If the contralateral ear is normal, routine tympanoplasty

is performed at age 4 [24]. If the contralateral ear is abnormal at this time,

adenoidectomy is considered, and tympanoplasty is generally deferred until

age 7 [25,26]. If contralateral disease is still present at this time, cartilage tym-

panoplasty is performed on the worse ear because a perforation in the con-

tralateral ear has been shown to be associated with a high risk of failure [27].

Typically, the perichondrium/cartilage island flap is used for the high-risk

perforation, with the exception that the size of the cartilage is tailored to the

size of the perforation. For example, if the perforation is a 50% anteriorperforation, the flap is constructed with the posterior island of cartilage re-

moved to avoid the need to modify the normal TM posteriorly, which would

otherwise be necessary to facilitate this plate of cartilage. The perichon-

drium is typically left the same size as described above so that it still extends

under the posterior TM and drapes over the canal wall under the tympano-

meatal flap for enhanced stability. Gelfoam is placed anteriorly to hold the

graft against the annulus, but Gelfoam is avoided posteriorly around the os-

sicular chain. In revision surgery, fibrosis and disrupted mucosa are fre-

quently seen, and Gelfoam in this milieu should be avoided to alleviatefurther scarring and optimize hearing results.

In our experience with more than 1000 cartilage tympanoplasties [28], the

high-risk perforation was an indication for the use of cartilage in a third of

cases, and successful TM closure was seen in more than 95%, of which 60%

were children (!18 years of age). Effusion requiring intubation as a second

procedure was seen in approximately 5%. Hearing results were encouraging

and were no different than those results using perichondrium or fascia.

The ear requiring ossiculoplasty

Reconstruction or reinforcement of the TM with cartilage is performed

typically in conjunction with ossiculoplasty to prevent prosthesis extrusion,

recurrent retractions, or cholesteatoma. Although the usual surgical indica-

tion is cholesteatoma in these cases, the method of cartilage reconstruction

is not dependent so much on the middle ear pathology, but on the presence

or absence of the malleus manubrium.

Malleus presentWhen the malleus handle is present, the modification of the palisade tech-

nique is used. An acoustic benefit has been shown with the incorporation of

the malleus in ossicular reconstruction, possibly owing to the cantenary ac-

tion of the malleus in the TM [18]. Likewise, the presence of the malleus with

an intact anterior malleolar ligament offers improved prosthesis stability by

allowing precise length adjustments and ultimate fit, leading to optimal




hearing results [29]. This has led us to abandon the use of the tragal island

flap when the malleus is needed for ossicular reconstruction because prior

placement of the flap, using the underlay technique as described, obscuresthe malleus and makes the subsequent ossicular reconstruction less precise.

Likewise, it is frequently difficult to carve the cartilage with enough preci-

sion so that the island flap fits exactly against the canal wall, which is nec-

essary in cases involving cholesteatoma.

When the malleus handle and suspensory ligaments are present, a partial

ossicular replacement prosthesis (PORP) cut to 2 mm and a total ossicular

replacement prosthesis (TORP) cut to 4.0 mm to 4.5 mm can be used con-

sistently for precise reconstruction when the notch of the prosthesis is placed

just inferior to the insertion of the tensor tympani. After precise ossicularreconstruction is performed, the posterior half of the TM is reconstructed

with cartilage pieces. The TM is pieced together like a jigsaw puzzle: the

half moon–shaped piece is placed on top of the prosthesis first, abutting

the malleus handle, followed by the scutum piece. Because the usual indica-

tion for this technique is cholesteatoma, any spaces left between these two

plates and the canal wall are reconstructed with slivers of cartilage cut to

fit precisely in these areas. The reconstruction is then covered with perichon-

drium if available; however, this is not necessary in most cases if good fit is

achieved. No space is left between the canal wall and reconstructed TM toprevent cholesteatoma or retraction pocket recurrence. In addition, the an-

terior half of the TM typically is not reconstructed with cartilage to allow

postoperative surveillance and tube insertion, if necessary.

If reconstruction of the anterior TM is necessary owing to pathology,

perichondrium is used. The technique is the same, but after precise fitting

of the prosthesis to the malleus handle, the prosthesis is removed and the

perichondrium is placed as an underlay graft. The prosthesis is then rein-

serted, with palpation of the malleus handle through the graft to facilitate

precise fit. The posterior palisade technique is then performed.

Malleus absent

The malleus-absent situation represents one of the most useful indica-

tions for cartilage tympanoplasty but one of the more challenging situations

for ossicular reconstruction because there is no malleus enabling an exact fit

between two essentially stable, bony platforms. The cartilage tympanoplasty

technique has proven useful to alleviate this problem.

The perichondrium/cartilage island flap from tragal cartilage is used.

Even though the malleus is absent, a similar circular cartilage flap is con-structed, again removing the 1- to 2-mm strip of cartilage from the center

section to facilitate accurate placement of the prosthesis. The cartilage is in-

serted in an underlay technique medial to the anterior TM remnant, with the

perichondrium again toward the ear canal. Several pieces of Gelfoam are in-

serted to support the graft securely to the anterior annulus and the bony

ledge just lateral to the supratubal recess. With the anterior portion of the

1170 DORNHOFFER



cartilage graft held securely in place, the posterior half is folded out to ex-

pose the trailing edge of the anterior piece of cartilage, which acts, in effect,

as a neomalleus (Fig. 10). The distance between the stapes footplate or su-perstructure and this trailing edge of the anterior cartilage is measured, and

the prosthesis is cut to the appropriate length.

For ossicular reconstruction, a prosthesis specifically designed for use

with cartilage tympanoplasty techniques is used. The notched portion of

the prosthesis is hooked under the trailing edge of the anterior piece of car-

tilage, much in the same way the malleus would be used in conventional os-

siculoplasty, with the shaft placed on the stapes (Fig. 11A, B) [30]. The

posterior half of the cartilage is folded back and is supported by the broader

posterior head of the prosthesis. This technique allows accurate length mea-surement and placement of the prosthesis, with direct visualization of the

stapes superstructure or footplate. Once the freestanding prosthesis is posi-

tioned accurately, supporting Gelfoam is placed to provide extra security

during the postsurgical healing phase.

The atelectatic ear

Numerous reports have established the efficacy of cartilage in TM recon-

struction of the atelectatic ear [1–3]. Much of the confusion associated with

this disorder stems from a poor understanding of the underlying pathophys-

iologic conditions that ultimately lead to changes in the TM, resulting in at-

rophy, diffuse or local retractions, and cholesteatoma formation [31,32]. The

controversy over whether the patient should proceed with surgery is aug-

mented by the fact that, early in the course of the disease, and even in the

presence of incus necrosis, hearing loss is frequently minimal, and patients

for the most part are asymptomatic. Once a decision is made to proceed

with surgery (grade III with adhesions and grade IV), the perichondrium/

cartilage island flap is generally used to reconstruct the TM.

Fig. 10. Reconstruction technique when malleus is absent (right ear).




There are two potential pitfalls specific for the atelectatic ear with an in-

tact chain. The first is the medially rotated malleus, which can make inser-

tion of the flap quite difficult. This can be overcome in one of two ways. The

first is to remove 1 mm of the manubrium at the umbo with the malleus head

amputator (malleus ‘‘nippers’’). This does not affect hearing and allows me-

dial placement of the graft. Attempting to lateralize the malleus with an in-tact chain should be discouraged because of the possibility of acoustic

trauma. The second is to remove a slightly wider strip of cartilage (2 mm)

to facilitate the malleus handle. This technique allows the more medial mal-

leus to indent further into the perichondrium, allowing the cartilage plates

to move more laterally in the reconstruction and avoiding contact with

the promontory. This also allows the anterior island of cartilage more flex-

ibility in positioning, which is necessary to make good contact with the an-

terior annulus.

The other pitfall specific to the atelectatic ear concerns management of theatrophic TM. After elevating the atrophic TM off the promontory, it is tempt-

ing to insert the cartilage medial to the intact TM. It is important, however, to

remove at least a portion of the atrophic TM anterior and posterior to the mal-

leus to ensure that the cartilage flap is incorporated into the reconstructed TM.

In our 1000-case series reported in the literature [28], an atelectatic TM

was the surgical indication in 15%, of which 20% were revision cases,

and two thirds were in children. The success rate was more than 95% for

graft take, with good hearing results. Reformation of the retraction pocket

was extremely rare, but 7% of patients required postoperative tube insertionowing to persistent effusion.

Managing cholesteatoma

Cholesteatoma represents one of the most controversial but important

pathologic conditions in which cartilage is used. The primary purpose of

Fig. 11. (A) Placement of cartilage graft (left ear), with posterior piece of cartilage folded lat-erally, shows posterior edge of anterior cartilage (neomalleus). (B) Placement of TORP, notch-

ing the neomalleus (left ear).

1172 DORNHOFFER



cholesteatoma surgery is to eradicate disease and provide a safe, hearing ear.

The magnitude of the controversy with regard to optimal surgical manage-

ment is beyond the scope of this discussion, but cartilage should arguably beinvolved in each technique.

A technique involving partial canal wall removal for cholesteatoma extir-

pation, followed by cartilage reconstruction, is recommended and discussed

as a separate chapter [33]. For TM reconstruction, the cartilage palisade

technique is preferred because this allows precise placement of the prosthesis

against the malleus, with the prosthesis acting as a scaffold to support the

cartilage posteriorly. This also allows excellent approximation between the

canal wall and TM reconstruction, creating a water-tight fit, which has

greatly reduced recurrent disease. With this technique, it is also preferableto leave the anterior portion of the TM without cartilage to allow observa-

tion and possible tube placement should this be necessary in the postopera-

tive period. For reasons that are not totally understood, the posterior

portion of the TM tends to be much more prone to retraction than the an-

terior portion, so leaving this latter area without cartilage has not proven to

be a problem with recurrent cholesteatoma [34,35].

One serious disadvantage of cartilage in this scenario is that it creates an

opaque TM posteriorly, which could potentially hide residual disease. This

is a problem that should be recognized, and surgical discretion should beused. If major disruption of the cholesteatoma sac occurs at extirpation,

one must consider the advisability of performing a second-look surgery at

a later date. However, this also applies to cholesteatoma surgery in general,

not just in cases in which cartilage is used in the reconstruction. One must

also recognize that most residual disease occurs in the epitympanum, an

area that is hidden by the bony canal wall and scutum when canal wall-up

surgery of any type is performed [36]. Although posterior cartilage TM re-

construction can delay the diagnosis of residual cholesteatoma, the disease

will manifest either anteriorly or as a recurrence of a conductive hearingloss, and there should be no major complications as a result of this delay

in diagnosis [37,38].

Managing pervasive eustachian tube dysfunction

We have developed distinct criteria for primary intubation of cartilage

tympanoplasties that is made up of craniofacial abnormalities, including

Down syndrome; previous head and neck cancer involving the nasopharynx;

and a history of multiple ear surgeries with demonstrated eustachian tubedysfunction.

The perichondrium/cartilage island graft is harvested and prepared as de-

scribed previously. Using a round knife, a window that is large enough to

allow placement of a Xomed Modified Goode T-tube is cut into the anterior

cartilage island. A straight pick is then placed into the cartilage window to

dilate the perichondrium to allow tube placement. Using scissors, the tube is




remodeled by trimming the flanges to approximately 3 mm to 4 mm. Before

insetting the graft, the tube is placed into the cartilage window and brought

out through the perichondrial surface. If the malleus is present, the end of

the tube is first angled under the manubrium with small alligator forceps.

After hooking the tube under the malleus, the graft is slid forward into

place. If the malleus is absent, the graft/tube complex is slid directly into

its final position (Fig. 12).If the tube is removed either accidentally or purposely, the TM heals with

a monomeric membrane; however, the cartilage defect remains. If tube rein-

sertion is needed, a myringotomy is performed first through the monomeric

membrane. The tube is then reinserted at the original site by grasping the

end of the T-tube with alligator forceps and pushing the flanges through

the cartilage defect. Because the TM is relatively rigid from the cartilage re-

construction, it does not medialize when pressure is placed laterally. This in-

creased rigidity greatly facilitates secondary tube insertion. The procedure is

well tolerated by the patient because the island flap remains insensate afterhealing, possibly owing to the cartilage preventing reinnervation from the

carototympanic plexus.

Summary

Cartilage is proving to be a very effective material for the reconstruction

of the TM in cases of advanced middle ear pathology. It is it particularly

useful for the management of the atelectatic ear, cholesteatoma, andhigh-risk perforation and for reinforcement of the TM in conjunction

with ossiculoplasty. Although the need for postoperative tube insertion is

relatively rare, it can prove to be difficult when the entire TM is recon-

structed with cartilage, emphasizing the need to optimize tubal function

and continue research to better predict outcome based on preoperative

parameters.

Fig. 12. Technique of cartilage tympanoplasty with intraoperative placement of T-tube.

1174 DORNHOFFER



References

[1] Sheehy JL. Surgery of chronic otitis media. In: English G, editor. Otolaryngology. Volume 1.

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1989;10(6):447–50.

[8] Duckert LG, Muller J, Makielski KH, et al. Composite autograft ‘‘shield’’ reconstruction of

remnant tympanic membranes. Am J Otol 1995;16(1):21–6.

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[11] Kerr AG, Byrne JE, Smyth GD. Cartilage homografts in the middle ear: a long-term histo-

logical study. J Laryngol Otol 1973;87(12):1193–9.[12] Don A, Linthicum FH Jr. The fate of cartilage grafts for ossicular reconstruction in tympa-

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1094–9.

[16] Zahnert T, Huttenbrink KB, Murbe D, et al. Experimental investigations of the use of car-

tilage in tympanic membrane reconstruction. Am J Otol 2000;21(3):322–8.

[17] Heermann J Jr, Heermann H, Kopstein E. Fascia and cartilage palisade tympanoplasty.Nine years’ experience. Arch Otolaryngol 1970;91(3):228–41.


Otol Neurotol 2001;22(3):299–304.

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[20] Goldenberg RA. Hydroxylapatite ossicular replacement prostheses: preliminary results.

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[22] Jurovitzki I, Sade J. Myringoplasty: long-term followup. Am J Otol 1988;9(1):52–5.

[23] Dornhoffer JL. Surgical management of the atelectatic ear. Am J Otol 2000;21(3):

315–21.[24] Buchwach KA, Birck HG. Serous otitis media and type 1 tympanoplasties in children.

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[25] Strong MS. The eustachian tube: basic considerations. Otolaryngol Clin North Am 1972;

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[26] Bailey HA Jr. Symposium: contraindications to tympanoplasty. I. Absolute and relative

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[27] Raine CH, Singh SD. Tympanoplasty in children. A review of 114 cases. J Laryngol Otol

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patient series. Laryngoscope 2003;113(11):1844–56.

[29] Dornhoffer JL. Hearing results with the Dornhoffer ossicular replacement prostheses.

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Ossiculoplasty

Luv Ram Javia, MD,Michael J. Ruckenstein, MD, MSc*

Department of Otorhinolaryngology, Head and Neck Surgery, University of Pennsylvania

School of Medicine, 3400 Spruce Street, 5 Silverstein, Philadelphia, PA 19104, USA

Contemporary surgical techniques for treating various pathologies

affecting the middle ear address not only eradication of the underlying

disease process but also restoration of normal auditory function. Trauma,

neoplasms, inflammatory processes, and cholesteatomas can erode and alter

normal middle ear components and relationships vital for the transmission

of auditory energy to the inner ear. Restoration of normal tympanic mem-

brane and ossicle function has its roots in the 1950s when surgeons

performed and examined the role of tympanoplasty in the treatment of

chronic otitis media. Over the last five decades, various ossiculoplasty tech-

niques and prostheses have been studied and reported in the literature.

Unfortunately, the multitude of reconstructive techniques attests to the

fact that none of the currently available methods are ideal.

Historical perspective

There was a divide created in the early stages of ossiculoplasty betweensurgeons who were proponents of natural prostheses and those who advo-

cated using artificial materials. In the 1950s and 1960s, several otolaryngol-

ogists, including Hall and Rytzner [1], Farrior [2], Guilford [3], Austin and

Shea [4], and Sheehy [5] began publishing results on using autografts for re-

construction, whereas House and coworkers [6] were the first to publish in

1966 the use of homografts. Cartilage was another material used initially

by Jansen [7] in 1956 for reconstruction of eroded ossicles that subsequently

became popular with numerous other otologists. These natural materials

gave good results; however, several problems existed with their use. First,homograft stores had to be maintained to have them available for surgery.


E-mail address: [email protected] (M.J. Ruckenstein).




39 (2006) 1177–1189





This was not an insignificant undertaking and was not practical for those

otologists who were not part of a larger institution. Even with the advent

of commercial tissue banks, the transmission threat of HIV, Creutzfeldt-Jacob disease, and other infectious agents became a concern. Moreover,

the time needed for shaping and customizing these natural materials is a ris-

ing concern as health care costs increase and processes are made more

efficient. Others have noted that homograft materials may become adherent

to various middle ear structures, such as the stapes, and more difficult to

remove during revision surgery compared with allografts [8].

Synthetic prostheses

In 1952, Wullstein was the first to publish the use of an artificial material

in ossiculoplasty. He used a vinyl-acryl plastic called palavit as a prosthesis

placed between the tympanic membrane graft and the stapes footplate. Since

that time, numerous materials, including polyethylene, polytetrafluoroethy-

lene, silicone, stainless steel, proplast, plastipore, ceravital, bioglass,

aluminum oxide ceramic, hydroxylapatite, carbon, titanium, platinum, tan-

talum, and gold, have been reported and most largely abandoned [9,10]. A

survey of otologists and neurotologists conducted by Goldenberg and Em-

met [11] in 1999 shows an interesting shift in the use of prostheses for ossi-culoplasty. When those surveyed were questioned as to their prosthetic

preference for ossiculoplasty; hydroxylapatite, autograft/homograft bone,

and plastipore were reported in order of decreasing preference. In fact,

70% of those surveyed preferred synthetic materials to bone (25.1%) or

cartilage (4.4%). This was in sharp contrast to a similar survey reported

by Emmet [12] in 1989 in which the preference was bone, then cartilage,

and finally plastipore; hydroxylapatite was not in the top three. Clearly,

as otologic surgeons gain more experience with alloplastic prostheses and

examine their outcomes, artificial materials are gaining popularity. Thisreview examines some of the more commonly used alloplastic prostheses,

including hydroxylapatite and titanium.

Middle ear structure and physiology

The tympanic membrane, malleus, incus, and stapes are vital components

of the middle ear involved with the transmission of energy from the air of

the external auditory canal to the fluid of the inner ear. It has been long

known that there is an impedance mismatch involved with this transfer of energy from air to fluid, with only 0.1% of acoustic energy traveling through

air actually transferring into a water medium [13]. The middle ear in classic

descriptions functions as an impedance matching device through three ma-

jor mechanisms: a hydraulic lever, ossicular lever, and catenary lever [14].

The hydraulic lever concentrates acoustic energy at the oval window and re-

sults from the 17- to 20-fold difference in vibratory surface of the tympanic

1178 JAVIA & RUCKENSTEIN



membrane compared with the smaller area of the stapes footplate [15]. This

is thought to be the main impedance matching mechanism. The ossicular le-

ver results from the long axis of the malleus being 1.3 times the length of thelong process of the incus. A smaller contribution is made by the catenary

lever suggested by Helmholtz in 1868 [16]. Both the outward convexity of

the eardrum and radial orientation of collagen fibers in the tympanic mem-

brane lead to a twofold amplification of sound pressure onto the umbo

[17,18]. These unique characteristics of the tympanic membrane also protect

the inner ear from static pressure variations while remaining sensitive to

small pressure changes. An overall 20- to 30-dB sound pressure gain is

thus produced by the impedance matching function of the middle ear

[15,19]. Classical theory regarding these levers was thought to be indepen-dent of frequency.

More recent studies have yielded some modifications to this general

scheme [13,17]. Laser measuring techniques have shown that different

parts of the tympanic membrane move at different velocities, decreasing

the effective area and efficiency of acoustic energy transfer. These tech-

niques have also shown that the malleus does not vibrate about a fixed

rotational axis but instead moves in three dimensions comprised of fre-

quency-dependent translational and rotational movements. Moreover, la-

ser-Doppler vibrometers have shown that laxity in the incudo-malleal joint leads to slippage and reduced efficiency of acoustic energy transfer

in a frequency-dependent manner. The lever function becomes less effi-

cient above 1 kHz. The incudo-stapedial joint, however, is thought to

predominantly be pistonlike, although there is evidence for some flexibil-

ity in the joint. Recently, Nakajima and coauthors [20] reported data sup-

porting the notion of ossicular joint flexibility. Although this joint

flexibility can introduce a loss of sound energy in the middle ear, it

may provide a protective function for the annular ligament and inner

ear in the face of large tympanic membrane movements from static pres-sure effects [21,22]. Another modification to the classical lever impedance

matching theory is that sound energy gain transferred by the middle ear

is frequency dependent [13]. Below 1 kHz the gain averages 23 dB and is

relatively flat with a peak of 26 to 27 dB at 0.9 kHz. Gain decreases by 8

to 9 dB per octave above 1 kHz. A final modification is imparted by the

resonance of the mastoid air cells. A peak resonance at 3 kHz leads to

a 5- to 6-dB decrease in sound transmission at that frequency.

It should be noted that middle ear impedance matching depends on

mobility of the tympanic membrane, which, in turn, can be affected byfibrosis or scarring, perforations, fluid in the middle ear space, alterations

in middle ear pressure, and presence of infection or masses. Healthy eusta-

chian tube function helps equalize pressure in the middle ear, drain

secretions from the middle ear to the nasopharynx, and prevent reflux

of harmful infectious and chemical nasopharyngeal contents into the

middle ear.

1179OSSICULOPLASTY



Biomechanics of ossiculoplasty

There are a number of biologic and mechanical factors to be considered

in reconstructing the middle ear. Biologic factors include the biocompatibil-

ity of the prosthesis, environment of the postoperative middle ear, healing

process, and patient comorbidities. Mechanical elements present include

the prosthetic vector of motion, variations in remaining ossicular elements

and their effects, tension of reconstruction, impact of interposed cartilage,

and mechanical properties of the prosthesis.

Biocompatibility depends on a prosthetic device remaining inert and

stable in a biologic environment. If a material used in ossiculoplasty is

perceived as foreign by the human body, the prosthesis could become a nidus

for chronic inflammation resulting in possible further destruction,

morbidity, hearing loss, and ultimately a failure of treatment. Moreover,

the implanted material should not be measurable in the immediate environ-

ment or circulating in the body. Polyethylene 90 tubing used in reconstruc-

tion in the late 1950s to early 1960s had some good initial results; however,

this material was noted to induce an inflammatory reaction in animal studies

[23,24]. Additionally, plastipore implants were criticized by some owing to

studies that show frequent histologic evidence of multinucleated giant cells

on plastipore implants, even though there has not been a clear association

delineated between the presence of these cells and a foreign body reaction

[9,25]. Ceravital was a bioceramic first introduced in the early 1980s with

promising hearing results and low extrusion rates. However, the implant

was withdrawn from the market after its biostability was questioned by

studies reporting absorption rates up to 5% [26]. In fact, Brewis and

coauthors [27] later reported a 36% absorption rate at 14 years with only

16% of treated ears having a favorable audiologic result.

The middle ear environment

The environment of the reconstructed middle ear must be one that is

conducive to optimal sound transfer. This includes repairing perforations

and reconstructing a tympanic membrane that is capable of vibrating freely

in response to acoustic energy. Retraction of the eardrum from negative

pressure, fluid or granulation tissue in the middle ear, and scarring with teth-

ering of the tympanic membrane, prosthesis, or remaining ossicles can all be

detrimental to efficient middle ear impedance matching function. In fact, it is

likely that pathology in the middle ear is more significant than prostheticfactors when considering extrusions and reconstruction failure [9,28]. Dur-

ing healing, scar formation and resultant tethering of the prosthesis, tym-

panic membrane, remaining ossicles, or footplate can undo any acoustic

gain garnered from ossiculoplasty. Critical to the success of ossiculoplasty

is the ability of the middle ear to maintain normal aeration, which prevents

restriction of movement of both the tympanic membrane and the round




window [17,19,29,30]. In a clinical study of middle ear mechanics in type III

tympanoplasties, Merchant reported a 35- to 55-dB conductive hearing loss

in those with nonaerated middle ear spaces [31].

The status of the malleus

A prosthesis with a lateral surface that terminates on the tympanic mem-

brane functions like a piston. As such, the lever mechanism conferred by the

normal arrangement of the ossicular chain is lost. Running the prosthesis

from the malleus to the stapes or oval window may preserve some lever ef-

fect. Goldenberg and Driver [28] have reported a significantly higher inci-

dence of closure of the air bone gap (!20 dB) when the prosthesis

interfaces with the malleus as opposed to the tympanic membrane (67% ver-

sus 42%). A medialized malleus results in increased angulation of the pros-

thesis, decreasing the efficiency of energy transfer and the stability of the

reconstruction. Cutting the tensor tympani tendon and, less desirably, the

umbo, may allow for lateralization of the malleus. However, if the malleus

is significantly medialized, it is likely more prudent to run the prosthesis to

the tympanic membrane. It should be noted that Goldenberg generally did

not place a cartilage interface between the prosthesis and the tympanic

membrane, and other series, in which cartilage is used, report improved

results.

Tension

Another area that has been examined is the optimal tension for recon-

struction. The annular ligament of the stapes footplate provides 90% of

the stiffness of the middle ear. However, placing a prosthesis too tightly

can distend the stapes footplate and annular ligament, decreasing the sensi-tivity. Using human cadaveric temporal bones and Goldenberg HAPEX

PORP prostheses, Morris and colleagues [32] examined the acoustic energy

transmission of loose-, medium-, and tight-fitting prostheses. There was

a trend for the loose-fitting prosthesis to give better acoustic results than

the tighter fitting ones, especially in the lower frequencies. At higher fre-

quencies, the tighter fitting prostheses did better but much less markedly.

The ‘‘loose fit’’ prosthesis was more unstable than Morris and Colleagues

[32] would allow in a patient, but based on trends present in their data,

they recommend using the loosest ‘‘stable’’ prosthesis. Similar results werereported by Bance and coauthors [14] using Goldenberg HAPEX PORP

and incus replacement prostheses. One caveat expressed in both studies is

that scarring and healing that occurs in a patient after the placement of

prosthesis may affect results in ways that have not yet been delineated or un-

derstood. The more effective acoustic transmission of looser fitting prosthe-

ses needs to be balanced with the threat of markedly decreased acoustic

1181OSSICULOPLASTY



transmissions of a slipped prosthesis. Hu ¨ ttenbrink [17] reported that an un-

steady interface between a stapes head and prosthesis can lead to acoustic

energy loss.

Prosthesis characteristics

Additional mechanical factors examined include prosthetic physical

factors. Zenner and coworkers [33] attempted to delineate prosthetic prop-

erties using multibody systems mathematical modeling. These data showed

that the optimal prosthetic weight for low impedance and good acoustic

energy transfer over the 100-Hz to 10-kHz frequency range was less than

5 mg. This conclusion was disputed by Hu ¨ ttenbrink [17] who contendsthat the weight of a prosthesis may not play a major role in vivo, because

when the mass of a prosthesis is increased by eightfold, only a 4 dB compro-

mise is seen.

Morris and colleagues [34] examined the acoustical effects of having an

interposed material between a tympanic membrane and a Goldenberg HA-

PEX PORP in cadaveric human temporal bones. At lower frequencies, there

was little impact seen by changing the material rigidity (glass, cartilage, and

Merocel). However, Merocel (the least rigid) had the worst transfer of en-

ergy at higher frequencies. Furthermore, this study also found that the mostcommon size of cartilage used, being the diameter of the prosthetic head,

had little affect on acoustic transmission.

Specific prosthetic materials

Hydroxylapatite (HA) is a calcium phosphate bioceramic that is similar

to the mineral matrix of bone. This is a popular implant material with

good biocompatibility properties. Hydroxylapatite prosthetic heads can be

placed directly adjacent to tympanic membranes without interposed carti-lage as is needed with plastipore and titanium implants; although many

authorities continue to advocate interposing cartilage between the tympanic

membrane and the prosthesis. The first HA prosthesis used in ossiculoplasty

was reported by Grote [35] in the early 1980s in which he used a prosthetic

implant made completely of dense hydroxylapatite. Grote [36], and subse-

quently Wehrs [37,38], published the first reports of the efficacy, biocompat-

ibility, and stability of solid HA middle ear implants.

To make these HA implants easier to manipulate and cut for sizing,

numerous hybrid implants were developed with heads made of HA andshafts made of various materials including plastipore, polycel, polytetra-

fluoroethylene, FLEX HA (50% HA and 50% Silastic [Dow Corning,

Midland, Michigan]), and HAPEX (HA reinforced polyethylene composite)

[9,11,28,30,39]. Long-term extrusion rates for HA are not reported fre-

quently, but the few reports that exist place the rate at 4% to 16%

[40,41]. Interposition of cartilage between the implant and tympanic




membrane has been shown to significantly decrease extrusion rate [25,42].

The hearing success rates (postoperative air-bone gap (ABG) %20 dB) are

summarized in Table 1 [25,28,40,42]. Of particular note are Goldenberg’srelatively poor results with total ossicular replacement prosthesis (TORP)

and partial ossicular replacement prosthesis (PORP) reconstructions com-

pared with the results obtained when the malleus is in place. Based on the

results of the other referenced studies, Goldenberg’s results are explainable,

at least in part, by his choice not to place a cartilage interface between the

tympanic membrane and the prosthesis.

Titanium

Although initial studies done by Palva and coworkers [43,44] working

with stainless steel implants did not show great promise for metal implants,

subsequent work with titanium shows otherwise. After titanium was shown

to be biocompatible by Branemark in the 1970s, Stupp in 1993 was the first

to use it in human ossicular reconstructions [10]. In addition to its biocom-

patibility, titanium has a number of favorable properties including light

weight, rigidity, greater versatility than HA in manufacturing of different

shapes and sizes, and an open head that allows for better visualization of

the distal end of the prosthesis shaft than hydroxylapatite’s solid head al-lows. Zenner reports that the low weight, mass, and rigidity of titanium

give it a theoretical favorable response at 2 and 3 kHz [45]. A similar nar-

rowing of the ABG is reported by Ho and colleagues at 2 kHz [46]. Cartilage

must be placed between the tympanic membrane and the prosthesis head to

prevent extrusion. Initial results indicate low extrusion rates with titanium

prostheses, ranging from 1% to 2% with follow-up periods of 3 years or

less [47–49]. Initial hearing results appear to be similar or slightly better

than those obtained with hydroxylapatite [46–49].

Bone cement ossiculoplasty

Distal erosion of the incus with an intact stapes superstructure represents

a challenging reconstruction for the otologist. A more recent technique de-

scribed in the literature involves the use of bone cements in ossiculoplasty,

usually in reconstructing the incudo-stapedial interface. Bone cement is

not a novel material in surgery or otology. It has been used in reconstructing

Table 1Percentage of patients with successful closure of ABG to %20 dB.

HA Incus Incus-stapes PORP TORP

Goldenberg and Driver [28] 75 50 32 57

House and Teufert [25] 57 39

Kobayaski and Colleagues [42] 84 62

Grote [35] 83 66

1183OSSICULOPLASTY



the cranium, tegmen, external auditory canal, and craniofacial defects.

There are a number of different types of bone cements studied for ossiculo-

plasty including Dahllite [50], hydroxylapatite [50–53], and glass ionomeric[54,55]. Glass ionomeric preparations (eg, OtoCem; Oto-Tech, Raleigh,

North Carolina) may provide good initial hearing results but are associated

with unfavorable toxicity and biocompatibility profiles [56]. Much better

biocompatibility results were obtained in animal models using Dahllite,

a calcium phosphate cement (Norian Craniofacial Repair System, Synthes

Corp, Paoli, Pennsylvania) and hydroxylapatite [50]. Hydroxylapatite prep-

arations include BoneSource (Stryker-Leibinger Corp, Kalamazoo, Michi-

gan) and OtoMimix (Walter Lorenz Surgical Inc, Jacksonville, Florida)

and form hydroxylapatite bone matrix upon setting. Hydroxylapatite ce-ments harden in 15 to 20 minutes and complete chemical reactions in 4

hours. Goebel reports that Mimix, a quick set formulation of HA cement,

hardens in 4 to 6 minutes [53]. All reports with HA cements have affirmed

its ease of preparation and application, rapid setting times, and minimal in-

flammation. It is critical to use gelfoam to protect areas surrounding the re-

construction, especially the footplate, during application. No extrusions

have been reported, which may be because of the absence of contact between

the cement and the tympanic membrane.

Good hearing results have been published using bone cement with betterthan 90% of the reconstructions showing an ABG closure of 20 dB or better

[51–54]. The versatility and ability to mold bone cement into any configura-

tion while preserving native ossicles, makes the use of bone cement very at-

tractive. Future studies involving bone cements are required that examine

the biomechanics, long-term results of hearing, extrusion, inflammation,

and comparison with more traditional prostheses.

Prognostic factors

Since the introduction of ossicular prostheses, there have been numerous

reports attempting to delineate prognostic factors important for predicting

good hearing results after ossiculoplasty. Significant factors include middle

ear pathology, ossicular chain status, staging of ossiculoplasty, external au-

ditory canal wall status, and type of prostheses. Yung [57] reported that

among disease, prosthesis, and surgeon-related causes of ossiculoplasty fail-

ure, disease-related factors accounted for 56% of failures. A significant por-

tion of these failures was caused by atelectasis with resultant prosthesis

extrusion or migration. In fact, in another study, atelectasis was found tobe the main reason for failure [58].

Status of middle ear mucosa

Mucosal status and presence of drainage are significant predictors of

postoperative hearing [59]. Fibrotic middle ear mucosa, including adhesions,




scar bands, and denuded mucosa during surgery, was detrimental to hearing

results compared with that of normal and reversibly thickened middle ear

mucosa. Drainage from the middle ear more than 50% of the time was sim-ilarly unfavorable with respect to hearing success. House and Teufert [25]

reported statistically significant poorer hearing results in patients with

chronic otitis media compared with otosclerosis, congenital ossicular abnor-

malities, ossicular fixations, and traumatic ossicular injuries grouped to-

gether. Although Yung [57] and Dornhoffer and Gardner [59] found that

the presence of a cholesteatoma did not impair the hearing result, Ron-

dini-Gilli [60] reported worse hearing in those ears with cholesteatoma.

Condition of the ossicles

The presence of a malleus or stapes can also affect the hearing outcome of

ossiculoplasty. Numerous reports indicate the importance of the stapes su-

perstructure for favorable hearing results. Rondini-Gilli [60] reported clo-

sure of the ABG to within 20 dB for 67% and 50% of ears with PORPs

and TORPs, respectively (P ! .05). A myriad of other studies also report

more favorable results with PORPs compared with TORPs

[25,26,42,46,48,49,58,61,62]. Some believe the difference between TORPs

and PORPs is attributable to a more secure connection afforded by the sta-pes head and less slippage compared with the footplate. Based on experi-

ences by numerous centers and surgeons with ossiculoplasty, Yung [57]

recently estimated the 5-year successful ABG closure rate for PORPs and

TORPs to be about 66% and 33%, respectively.

The malleus also has been of great interest as discussed in the biomechan-

ics section and is thought to provide better hearing results if incorporated in

the reconstruction by preservation of the catenary lever. Dornhoffer and

Gardner [59] felt that it was of such importance that malleus presence was

included as part of the Ossiculoplasty Outcome Parameter Staging Indexfor predicting hearing outcomes. Brackmann and coauthors [63], however,

did not report a clinical difference between malleus presence or absence.

Staged reconstruction

The issue of whether or when to delay (stage) an ossiculoplasty after pri-

mary middle ear surgery (eg, tympanomastoidectomy) has always been

somewhat controversial. A recent study lends support to the generally

held concept that a delayed ossicular reconstruction is the preferred optionwhen extensive middle ear/mastoid surgery is performed [64]. These investi-

gators suggest that a primary ossicular reconstruction may be most appro-

priate in cases of canal wall up tympanomastoidectomy with an intact stapes

superstructure. A delayed reconstruction may yield better results in cases re-

quiring a TORP, when a canal wall down procedure is performed, when re-

sidual cholesteatoma is suspected, or with extensive disease and resection of

1185OSSICULOPLASTY



mucosa. One exception to this premise is in the case of a canal wall down

tympanomastoidectomy with an intact stapes in which a small piece of car-

tilage is interfaced between the stapes and the fascia graft, forming a type 3tympanoplasty.

Canal wall up versus canal wall down mastoidectomy

Debate in the literature exists as to whether retention of the ear canal dur-

ing mastoidectomy confers an intrinsic advantage when reconstructing the

middle ear. This question has proven difficult to answer, as most otologists

perform both procedures, with more extensive middle ear/mastoid disease

resulting in a canal wall down procedure. In general, it is fair to concludethat the extent of the middle ear disease is a much more important predictor

of hearing outcome than is the type of mastoidectomy performed. In fact,

the originator of the canal wall up technique concedes that hearing results

are similar in both mastoidectomy techniques [63].

Summary

Ossiculoplasty is an effective surgical option for reconstruction of the dis-

eased middle ear that can very effectively treat conductive hearing loss.

However, techniques and prostheses used in reconstruction are still imper-

fect. Based on the data available today, the following conclusions can be

drawn pertaining to ossiculoplasty.

1. Autograft prostheses largely have been replaced with more readily avail-

able and durable allograft prostheses.

2. Titanium and hydroxylapatite prostheses are the most widely used and

successful implants available at this time and have good biomechanical

properties, low extrusion rates, and good hearing results. More prospec-tive trials will help delineate the differences between implants. Titanium

prostheses are somewhat more ‘‘user friendly’’ and have therefore be-

come more popular in recent years.

3. A prosthesis should be placed with as little tension as possible without

significant tenting of the tympanic membrane, while maintaining pros-

thesis stability.

4. An interface of cartilage should be placed between all of the currently

available prostheses and the tympanic membrane to minimize extrusion.

5. The axis of pistonlike movement should be kept in mind and angulationminimized to prevent slippage and inefficient sound energy transfer.

6. The tympanic membrane should be mobile with emphasis placed on

middle ear aeration and minimal surgical destruction of mucosa.

7. Bone cements are promising in selected applications and require further

study with more patients and investigation into the biomechanical prop-

erties after reconstruction.




References

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1189OSSICULOPLASTY



Virtuosity with the Mallet and Gouge:

The Brilliant Triumph of the

‘‘Modern’’ Mastoid Operation

Sarmela Sunder, MD, Robert K. Jackler, MD*

,Nikolas H. Blevins, MDDepartment of Otolaryngology - Head and Neck Surgery, Stanford University Medical

Center, 801 Welch Road, Stanford, CA 94305-5739, USA

As a life-saving measure few surgical procedures rival and none surpass

in efficiency the modern mastoid operation, the meritorious achievements

of which very properly entitle it to the approbation and esteem of the med-

ical profession and to the enduring gratitude and applause of the apprecia-

tive public.Brilliant as are the triumphs of surgery, no brighter page ornaments its

records than that which chronicles the recent remarkable progress in the di-

agnosis and treatment of mastoid and intracranial infective diseases in the

development of which the otologist, we are proud to say, has borne a by no

means inconspicuous part.

dFrederick Whiting, The Modern Mastoid Operation, Philadelphia:

P. Blakiston’s Son & Co., 1905.

Mastoid surgery has a rich and extraordinarily interesting history (Table 1).

Although performed only sporadically and tentatively before the mid-nine-teenth century, by the early 1870s it had become a major part of otologic prac-

tice. Barber-surgeons faced with a swollen, red, hot, and acutely painful

abscess behind the ear occasionally performed a simple incision and drainage.

This practice is magnificently illustrated in a drawing executed by Lucas van

Leyden in 1524 (Fig. 1).

By the early 1900s, still in the pre-antibiotic era, drainage of the acute

abscess became commonplace (Fig. 2). Dr. Francis A. Sooy (1915–1986), a

renowned San Francisco otologist, described that, as late as the 1940s, most

clinic days were followed by a trip across the street to the operating room todrain a couple of acute mastoids (Francis Sooy, MD, personal


E-mail address: [email protected] (R.K. Jackler).




39 (2006) 1191–1210

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communication, 1980). In the postantibiotic era, surgery for acute mastoiditis,

so prominent in the operation’s early history, had become a rarity. Although it

remains largely used as a method to control infection, it has been refined to be-

come a central component in a broad number otologic procedures. It serves as

the central access route to facilitate neurotologic exposure and provides access

for vestibular surgery and cochlear implantation, procedures never even

dreamed about by the operation’s early proponents.

Table 1

Major milestones in the history of mastoidectomy

1774 First description Jean-Louis Petit Paris1853 Postauricular incision William Wilde Dublin

1873 Simple mastoidectomy Hermann Schwartze Halle

1889–90 Radical mastoidectomy Ernst Ku ¨ ster

Ludwig Stacke

Emanuel Zaufal

Berlin

Erfurt

Prague

1905–10 Modified radical mastoidectomy Charles Heath

W. Sohier Bryant

Gustave Bondy

London

New York

Vienna

Fig. 1. Early mastoid surgery by a barber-surgeon. Incision to drain mature abscesses was

likely practiced since antiquity. Lucas van Leyden, 1494–1533; The surgeon, 1524. (From The

Clements C. Fry collection of Harvey Cushing/John Hay Whitney Medical Library at Yale

University, New Haven, CT.)

1192 SUNDER et al



Early suggestions

The mastoidectomy procedure has as its origin an attempt to drain pus

from an infected mastoid cavity. Several individuals throughout history sug-

gested the concept of drainage, although there are no documented cases of

them ever having performed the procedure.

The first century Greek physician Galen (130–200 AD) recognized the im-

portance of allowing drainage from an infected ear. In his writings, he rec-

ommended that otorrhea should be permitted and drainage encouraged,

rather than be obstructed with wool [1–4]. Dr. Adam Politzer’s interpreta-

tions of Galen’s works credit him with recommending that carious bonebe removed after making an incision behind the ear. However, in reviewing

Galen’s books, the authors of this article were unable to locate any such spe-

cific suggestion. Galen, considered to be a founder of experimental physiol-

ogy, based his work and knowledge of anatomy on animal dissections,

rather than human subjects. He, himself, made no record of performing

a postauricular incision on a human subject [1–6].

The earliest mention of draining an infected ear with a surgical incision in

a patient is attributed to the French physician Ambrose Pare ´ in the sixteenth

century [7–10], who is credited with having suggested the use of a drainageprocedure for King Francois II of France (in 1560) [9]. The king died from

his condition soon after, without ever having undergone the procedure be-

cause his mother, Catherine of Medici, reportedly denied him the operation

[10]. The details of this incident are varied, depending on the source. How-

ever, the authors’ review of Pare’s writings indicates no explicit suggestion

to drain the king’s abscess [9–12]. In fact, a student of Pare’s, Francis

Fig. 2. The Mastoid Club. A World War I era photograph illustrating the frequency of suppu-

rative mastoiditis before the introduction of antibiotics. (Courtesy of the National Library of

Medicine, Bethesda, MD.)

1193THE ‘‘MODERN’’ MASTOID OPERATION



Packard, claims that, ‘‘Pare does not anywhere state that he even suggested

operating on Francois II for his mastoid condition’’ [13,14].

Incision to drain pus

The first unequivocally documented recommendation at trephining the

mastoid bone can be found in the works of Johannes Riolanus the Younger

[15], in 1671. An anatomist and dissector at the University of Paris, Riola-

nus later became a professor of medicine. His father, who shared the same

name, was a dean of the Paris Medical Faculty. In several of his texts, Rio-

lanus makes references to opening the mastoid surgically, when such inter-

vention does not pose undue risk to the patient. ‘‘If an intolerableinflammatory and pulsating pain does occupy the hinder parts of the

head, and matter floes thither and there stops, the pain abiding, it will be

safe to bore a hole in the hinder part of the head, that egress may be given

to the quitter; when no great danger is like to follow the operation’’ [15]. In

his text Anthropographia, he describes the findings of the procedure as ‘‘a

small and narrow opening which, gradually enlarging passes into a caries

of considerable size, presenting everywhere little depressions, like the cells

of a honeycomb’’ [16].

Jean-Louis Petit is credited with developing the first successful operationon the mastoid for the purpose of evacuating pus. Unlike his predecessors,

Petit clearly established the procedure as a method to perforate bone with

the deliberate intent to search for and evacuate pus. Petit (1674–1750) was

an eighteenth century French surgeon, who obtained his master’s certificate

in surgery in 1700 and joined the Royal Academy of Sciences in 1715

(Fig. 3). Later, in 1731, the king of France created the Royal Academy of

Surgery, and appointed Petit as its first director. According to Petit’s eulogy,

presented to the Royal Academy of Surgery, he achieved great renown for

his experience with various surgical techniques, including the operative man-agement of bone diseases [17]. Employed in the military hospital as of 1692,

he taught army surgeons, who then extended his teachings all over Europe.

During this time, he developed a new type of tourniquet for which he was

well-known. The tourniquet, which was tightened by means of a screw,

was aptly named a screw tourniquet and was used to arrest hemorrhage be-

fore and during surgery. He is referred to as one of the founders of gall blad-

der surgery, for suggesting, in 1733, the removal of gallstones, drainage of

the gall bladder, and creation of a draining fistula in patients who had

gall bladder empyema, and then successfully performing the procedure in1743. It has been said that his general surgery training gave Petit a unique

approach to mastoid disease [18].

Petit advocated a deliberate search for pus within the mastoid bone,

rather than simply extending a persistent fistula tract. In 1774, in his Traites

des malaides chirurgicales, Petit described mastoid surgery, stating that the

‘‘pus is situated in bony cavities whose walls cannot be made to collapse;

1194 SUNDER et al



it is entirely shielded from compression, it lodges there, gives rise to caries of

the bone and this caries cannot be reached by any topical application. It is

even inaccessible to any efficient operation’’ [18,19]. He described originally

chipping away the edge of bone in a piecemeal fashion, along the pre-existing

fistula. He later recommended perforating an intact mastoid. Petit pro-

posed that if there are signs of inflammation suggesting the presence of an

underlying abscess of the mastoid, one should not hesitate to pierce the tabla

externa and open up the mastoid through trepanation. He felt that trepana-

tion was justified because surgical drainage could provide effective decom-pression well before untreated purulence would spontaneously drain by

perforating the tabla externa, thereby shortening the time course of the dis-

ease [19]. He reported immediate recovery ‘‘after the compact layer had been

taken away with gouge and mallet’’ to drain the purulent collection.

‘‘These abscesses in the diploe may persist for a long time before reaching

a stage at which they cause death; but from the very first days of formation

they ought to be opened, and we ought not to wait until they have destroyed

the bone, for the patient is always in danger, not only because of the pus

which does not escape, but because of other accidents which may superveneand render the disease infinitely complicated and fatal’’ [18,19]. This ap-

proach to treating mastoiditis was a significant deviation from the accepted

treatment of the time, which included medical management, consisting of

topical applications of various concoctions. Petit detailed how his proposal

was not readily accepted by his peers, describing how he was ‘‘met with

much opposition from certain consultants, who.could not consent to an

Fig. 3. Jean Louis Petit (1674–1750). Early French surgeon who was the first to formally

describe opening the mastoid, in a text posthumously publishedin 1774. (Courtesy of the National

Library of Medicine, Bethesda, MD.)




incision being made upon the skull at a spot where there was no external

lesion’’ [19].

As a treatment for tinnitus or deafness

Most mastoid operations were performed for infections; however, during

different pockets of history, opening of the mastoid was attempted for other

reasons, particularly as a cure for deafness and tinnitus. These periods, in

which the operation was applied to treat conditions for which it is not

clearly indicated, would eventually give rise to the mounting unpopularity

of mastoid surgery.

J.L. Jasser, an eighteenth century Prussian military surgeon, made a post-auricular incision down to bone on a soldier with swelling over the mastoid

and a history of copious otorrhea. Probing a region of bone that appeared

dark, he entered a deep opening into the mastoid air cells. On irrigating this

area using a syringe inserted into the opening, Jasser noticed that a signifi-

cant amount of pus was relieved from the meatus and through the nostril.

Three weeks later, the patient reported improved hearing [13,20]. Jasser ini-

tially performed the procedure for cases of infection, but was so encouraged

by the success of the mastoid perforation in the treatment of hearing loss

associated with mastoiditis that mastoid trephination came into use brieflyfor the treatment of deafness from any cause.

Sir Charles Ballance [18] credits Johannes Riolanus the Younger with rec-

ommending opening the mastoid as a treatment for tinnitus in his Anthro-

pographia. Riolanus states the following: ‘‘It happens that when this air,

which should always be still, is stirred up in the ear by the impact of a fresh

air current, that the ears buzz continually. But how can we give exit to this

air which is such a source of trouble? There is certainly no other way of

doing this except trephining the mastoid process.’’ However, there is no clear

evidence that Riolanus ever implemented his suggestion.The death in 1791 of Baron Johann von Berger, a Danish court physician,

was a critical point in temporarily halting the progress of mastoidectomy.

According to various authors, the 68-year- old physician von Berger had suf-

fered for many years with tinnitus and deafness when he requested that the

operation be performed on him to restore his hearing and eradicate his tinni-

tus. von Berger underwent the surgery and died of meningitis within 2 weeks1

[13,18,21]. This event drew great attention to the hazards of the operation and

was cited by opponents of the procedure for many years.

The combination of adverse results and questionable indications led toa loss of popularity of the mastoidectomy. However, despite its loss of favor,

several surgeons were advancing the application of mastoidectomy by bring-

ing greater understanding to the underlying pathophysiology that could be

addressed by this procedure. The recognition of mastoid surgery’s inherent

potential was summarized by the optimistic sentiments of Jasser, who said,

‘‘No good book or good thing of any sort shows its best face at first’’ [13].

1196 SUNDER et al



Postauricular incision

Interest in mastoid surgery did not completely fade, however, as several

surgeons continued to utilize it in certain situations and for specific indica-

tions. Ballance attributes J.E. Dezeimeris with defending the surgery in

1838. Dezeimeris argued that reports of complications from perforation of

the mastoid must be scrutinized before the procedure is abandoned alto-

gether. In his L’Experience in Journal de Medecine et de Chirurgie that

‘‘the opponenets of perforation of the mastoid process have not failed to

bring into prominence as an argument against the operation the danger

which ordinarily attends abscess with caries of this apophysis, but the ques-

tion should be to determine whether it is really the making of the opening

which constitutes the danger in theses cases, or whether the danger does

not really lie in the lesions which have time to occur in the inner ear and

often beyond it before a way out has been made for the pus by the efforts

of nature or, to discard this figure of speech, the progress of the disease.’’

Dezeimeris describes several cases wherein the operation was performed

either with successful results, or in cases with complications, he notes that

improper indications or the natural course of the disease, rather than the

operation itself, yielded the negative results [22,23].

The next era of the mastoid operation was inaugurated by Sir William

Wilde and the introduction of the postauricular incision that bears his

name (Fig. 4). William Wilde, born and raised in Ireland, followed in his

father’s footsteps in the practice of medicine (Fig. 5). As a young surgeon,

inspired by the lack of medical facilities for the care of the poor, Wilde es-

tablished a small hospital in Dublin that originally catered to the eye and ear

care needs of most of the poor population. News of his talents spread, as

individuals of the upper classes learned of his work from their servants

Fig. 4. Wilde’s mastoid incision. Wilde advocated postauricular incision to release pus, but did

not endeavor into the mastoid bone itself. (From Portmann G. A treatise on the surgical tech-

nique of otorhinolaryngology; Baltimore: William Wood; 1939. [English translation from the

original French.])




and maids. The hospital later moved to larger accommodations, and, for

a time, Wilde’s hospital was the only one in the United Kingdom where au-

ral surgery was taught. In 1853, he published Aural Surgery, a series of es-

says on the most common and serious ear diseases. Interestingly, although

he spoke his native Irish fluently, he did not have a thorough grasp of its

written form, often requiring him to write Irish words phonetically, rather

than in their correct spellings. Wilde continued to have a decorated profes-sional career, leading to his knighthood in 1864. Wilde’s personal life was

equally colorful, from his numerous mistresses and reportedly illegitimate

children, to his abrasive temper and eccentric behavior later in life [24,25].

Perhaps one of the great ironies about this pioneering surgeon was his dif-

ficult relationship with his famous son, Oscar Wilde, who suffered from

a chronic draining ear and eventually died of meningitis secondary to otitis

[25,26].

Instrumental to the development of mastoid surgery, Wilde [27] asserted

that mastoiditis resulted from ‘‘what was originally otorrhea from an in-flamed mucous and periosteal membrane spread from thence to the bone it-

self.’’ He advocated that ‘‘should the mastoid process become engorged, or

even an indistinct sense of fluctuation be discovered, we should not hesitate

to make a free incision at least an inch in length. The head should be placed

against some unyielding substance, and the blade of a stout scalpel inserted

steadily till the point reaches the bone, to secure complete division of the

Fig. 5. William Wilde (1815–1876). Irish surgeon who popularized the postauricular incision

(Wilde’s incision) as a means of draining abscess of the mastoid. (From Wilson, TG. Victorian

doctor: being the life of Sir William Wilde. New York: Fischer; 1946.)

1198 SUNDER et al



periosteum.From the swollen state of the parts, we are sometimes obliged

to introduce the instrument at a depth of nearly an inch. Immediate relief

follows, even if pus is not discovered.’’ Later, he cautions ‘‘as the success at-tending this procedure must be very doubtful, and the hazard very great, it is

never resorted to in the present day’’. This incision forms the basis of the

first step of the modern mastoid operation.

Wilde was not lauded universally by his contemporaries. Wilhelm Kramer

[28] of Berlin, also an author of a prominent mid-nineteenth textbook of ear

diseases, wrote a scathing review of Wilde’s 1853 book Aural Surgery, in

which he said: ‘‘ . . . I willingly confess that my unfavorable opinion of the

state of English aural surgery is not in the least changed by the publication

of his ‘Aural Surgery.’ ’’ Evidently, Kramer’s critique was at least in part aretaliation for Wilde’s harsh criticism of his own book 4 years prior, in the

Dublin Journal of Medical Sciences. It could be inferred that the Wilde–

Kramer controversy was a manifestation of competition between the German

and British otologic schools. However, the Lancet editor clearly was on

Kramer’s side, indicating that Wilde’s book should be consigned ‘‘to the

trunkmaker.’’ One might suppose the London-based editor was more con-

cerned about English superiority over the Irish than any continental rivalries.

During this same time, Joseph Toynbee, who also did not partake in

making an incision, retrospectively commented on the value of allowingthe escape of pus, when reflecting on a patient who had mastoiditis: ‘‘It

seems to me probable that if in any way a sufficient portion of the membrane

tympani had been removed at the beginning of the attack to permit of a thor-

ough evacuation of the contents of the mastoid cells, the bone would have

been free from disease’’ [13,29]. In another case, Toynbee supports trephina-

tion, stating, ‘‘perforation of the mastoid process also suggests itself, and the

operation may doubtless be performed in those cases where the matter is

pent up in the cavity of the ear and is causing such harmful and serious

symptoms as are likely, if not relieved, to terminate in death. I have neverperformed the operation, but I should not scruple to do so in a case where

the life of a patient was threatened’’ [29].

In an attempt at a different technique to relieve infection, Amedee Forget,

a French surgeon, reported a case in 1860, in which he opened the antrum,

passed a seton from the meatus into the antrum, and brought it through the

operation wound, so that drainage would be possible both ways. The seton

was replaced periodically, but the symptomatic relief was only temporary.

The patient underwent a second operation to remove dead tissue and

bone. After this procedure, the patient reported significant improvementin symptoms [13].

When the typical treatment of ‘‘general injunction of bacon fat twice

daily’’ and subsequent irrigations of the external auditory canal failed to re-

solve a patient’s symptoms, the German surgeon Anton Friedrich Baron

von Tro ¨ ltsch [21] opted to intervene by making a postauricular incision

down to bone. von Tro ¨ ltsch, a professor in aural medicine at the University




of Wurzburg as of 1864, studied diseases of the ear under Wilde in Dublin

and Toynbee in London [30]. In an 1861 paper, he discussed the case of a 16-

year-old girl with scarlet fever associated with otalgia, deafness, and mastoiderythema and edema. He commented that ‘‘immediately after the incision,

pus suddenly filled the meatus and as this was washed away fresh blood be-

came freely mingled with the last portion evacuated. No pus appeared in the

wound behind the mastoid.’’ The patient’s symptoms were reportedly

greatly relieved for the next 2 days. When symptoms of pain and increased

postauricular swelling recurred, Von Tro ¨ ltsch explored the previously made

incision with a blunt-ended probe, trying to ‘‘pass the probe in a forward

direction but did not succeed.’’ He placed a plug in the wound, with the in-

tent to try again the following day. However, soon after, he noticed ‘‘pusbegan to come away from behind the ear, at first thin and afterwards

thicker, while but now little came from the meatus.’’ He continued to ad-

vance the probe the next day, with persistent drainage of pus and subse-

quent improvement in the patient’s symptoms. In hindsight, Von Tro ¨ ltsch

relates, ‘‘if I had not succeeded by merely using the probe, I should hardly

have the courage to adopt more active measures with other instruments.

Now, however, in a similar case I should act with far stronger resolution

and should not dread to remove the external layer of bone with a gouge

or with gouge forceps if it appeared thickened or broken down’’ [21].Von Tro ¨ ltsch is credited with stimulating interest in mastoid operations

among German surgeons. He encouraged study of the operation, suggesting

‘‘if this operation has been forgotten, or has come into disrepute, it is

because of its abuse in the preceding century, as well as in the peculiar and

exceptional position of the literature of otology until a short time ago.’’ He

encouraged a methodical thought process to be applied to the operation

and its indications because ‘‘principles, which in other departments of med-

icine are accepted as rational, methods of treatment, which surgery views

as absolutely necessary, under exactly the same circumstances, were notapplied to the ear and its diseases’’ [21]. Specifically, Von Tro ¨ ltsch is credited

with encouraging his student Hermann Schwartze to focus attention on

methods and indications for the surgical opening of the mastoid.

Simple mastoidectomy

In 1873, Hermann Schwartze and Adolf Eysell [31] published an article

outlining surgical opening of the mastoid. It was the first systematic account

of the operation as a scientific procedure to be performed with a definiteplan and under specific conditions.

The paper described how in cases of destruction of the mastoid cortex,

curettage of the interior would hasten the healing process. Schwartze de-

scribed a case of mastoid suppuration where the cortex was so soft that it

could easily be penetrated with a probe, but suggested that cortex consisting

of hard, firm bone could be entered with the aid of trocars and trephines. He

1200 SUNDER et al



took the concept of mastoid surgery one step further, by recommending the

use of a chisel and hammer to remove bone adequately, so that the antrum

might be properly inspected and effectively drained.Schwartze laid down clear indications for surgery: ‘‘(a) As a cure for

chronic purulent discharges from the attic without taking the still existing

hearing capacity into consideration, in caries of the hammer and anvil, in

chronic purulent discharge in the tympanic cavity without positive signs

of caries of the hammer and anvil, or in cholesteatoma in the tympanic

cavity. (b) For the improvement of hearing and the cure of subjective noises,

when there is fixation of the hammer., in incurable obstruction of the Eu-

stachian tube., or in sclerosis of the tympanic cavity with no sign of nerve

deafness’’ [31]. The publication of these results by Schwartze, a professor atthe renowned Halle Clinic, was the impetus for the renaissance of mastoid

surgery (Fig. 6) [30,31].

The Schwartze mastoidectomy, as it came to be known, served as a means

of establishing a direct communication between the mastoid process and the

tympanic cavity, so that the cavity could be irrigated with antiseptic solution

(Fig. 7). Although initially used in cases of acute mastoiditis, it was later

used for treatment of chronic infection during times of acute exacerbations,

or in cases of fistula formation through infected bone, and finally extended

to cases of persistent suppurative otitis media [30].Hermann Hugo Rudolf Schwartze was born in Neuhof, Germany in

1837. His father was a farmer who descended from a lineage of clergymen

Fig. 6. Hermann Schwartze (1859–1919). German surgeon (University of Halle) whose influen-

tial work advocated anatomically thorough, simple mastoidectomy, including entry into the

antrum. (Courtesy of the National Library of Medicine, Bethesda, MD.)




[32]. Schwartze studied in Berlin and Wu ¨ rzburg, receiving his medical doc-

torate from the University of Berlin in 1859. He studied under Anton von

Tro ¨ ltsch, and served as an assistant at the Anatomopathologic Institute at

the University of Wurzburg. After receiving his medical degree in 1859, he

became professor and director of the otologic clinic at Halle University.

In 1863, with von Tro ¨ ltsch (1829–1890) and Adam Politzer (1835–1920),

Schwartze founded the journal Archiv fu ¨ r Ohrenheilkunde, the first journal

dedicated exclusively to the pathology and treatment of the ear. He later es-

tablished his own clinic for patients who had diseases of the ear [32]. In the

Franco-Prussian war, Schwartze served as a physician in a field hospital of

the IV Army and was awarded the Iron Cross, Second Class. In 1903, he be-

came the first ear physician in Germany to receive the honor of being named

professor ordinarius of his discipline. He was a member of several scientific

societies, and was an honorary member of the American Otological Society.

The University of Halle established the Hermann-Schwartze Medal to

honor his contributions to the field.

Near the end of his life, Schwartze reportedly suffered from a ‘‘nervous

condition of restlessness, vertigo and delusions’’ [33–35]. Interestingly, his

one-time mentor, von Tro ¨ ltsch, also endured a similar fate. Von Tro ¨ ltsch’s

obituary describes that ‘‘in his later years an insidious neurosis sapped his

powers and finally reduced him to helplessness’’ [30,36]. Schwartze eventu-

ally died of heart failure at the age of 73 at the Tecklenburg sanitarium [32].


The simple mastoidectomy or antrostomy was sufficient in cases of acute

mastoiditis; a more comprehensive procedure, the radical mastoidectomy,

Fig. 7. Schwartze’s mastoidectomy: Schwartze opened through the mastoid cortex until the

antrum was widely opened. (From Schwartze HH, Eysell CG. Uber die Kunstliche Eroffnung

des Warzenfortsatzes. Arch Ohrenheilkd 1873;7:178.)

1202 SUNDER et al



was developed as an answer to cases of chronic otitis or for acute cases with

intracranial complications. Ernst Ku ¨ ster and Ernst von Bergmann, both of

whom were general surgeons, are credited with developing the radical pro-cedure, which for some time was referred to as the Ku ¨ ster-Bergmann oper-

ation. Their combined operations allowed the conversion of the various

spaces of the attic, the antrum, the middle ear, and the external auditory

canal into a single larger cavity.

Ernst Ku ¨ ster [37], a German general surgeon, argued that Schwartze’s

operation should be extended further. In 1888, he made a presentation on

this topic at the German Medical Society, and later published a paper illus-

trating his belief that abscesses in hard bones should be exposed completely

by opening all the sources of pus, regardless of depth. Rather than justopening into the antrum, as suggested by Schwartze, Ku ¨ ster advocated

that bone be opened beyond the antrum, with removal of the posterior mea-

tal wall. He suggested, ‘‘open up freely.clear away all disease.fully expose

the source of suppuration.’’ He encouraged that ‘‘the rational treatment

must be based on the surgical principle that a diseased bony cavity should

be opened up extensively, all diseased tissue removed, and the source of sup-

puration brought clearly to the light. The pus must nowhere be hindered in

its outflow. Only when this is done are the surgical requirements fulfilled’’

[35]. Ku ¨ ster suggested that, whereas in less severe cases simply chiselingaway the mastoid process suffices, in cases of extensive disease a more thor-

ough approach should be undertaken. Specifically, ‘‘if the tympanic cavity is

filled with granulations, and if the drum-membrane and ossicles are in large

part or entirely destroyed, I try to get down and into the middle ear, so as to

be guided by my eye in removing with the sharp spoon everything that is

diseased’’ [37].

Von Bergmann [38,39] similarly presented to the German Medical Society

in 1889 and published his results suggesting the removal of the posterior and

upper wall of the canal. He stated, ‘‘Between the upper wall of the externalauditory canal and the lower and lateral border of the middle fossa of the

skull, lies a thick layer of bone into which the chisel can penetrate as far as

the bony frame of the drum-membrane, without opening the cranial cavity.’’

After a detailed description of how to arrive at, and chisel through, the pos-

terior wall, he stated that ‘‘by removing in this way the posterior and upper

wall of the external wall of the external meatus, we can form a funnel-shaped

hole, into which we can introduce the finger as far as the tympanic cavity, and

thus remove from the latter with the sharp spoon the remains of the ossicles,

the indurated granulations and the cheesy and stagnating pus’’ [38,39].Recommendations to chisel down through the posterior wall of the canal

were met with reservations because of concern about facial nerve injury.

Dr. Arthur Hartmann [40] of Berlin, quoting a visiting American otologist

in 1895: ‘‘I have been only two days in Berlin, and yet I have seen three facial

paralyses following the chiselling of the mastoid, although in America I

never saw one.’’




Emanual Zaufal [41,42] further advanced the radical procedure by work-

ing forward from the antrum toward the attic, and by developing the inci-

sion of the upper and posterior portions of the membranous meatus (Fig. 8).He modified the Schwartze procedure in 1890 by suggesting the removal of

the posterior canal wall. Presenting at the Twelfth Association of the Asso-

ciation of German Physicians (Nordberg) in April 1890 and, later, at the

Tenth Convention of South-German and Swiss Otologists (Prague) in May

1890, he explained his method of chiseling through the mastoid into the tym-

panum. He performed this procedure by placing one blade of a Luers ron-

geur into the auditory canal and the other blade through the antrostomy,

such that with a single closure of the forceps, he was able to remove the upper

posterior wall of the canal and the outer attic wall, simultaneously. This stepallows for proper exposure so that ‘‘the cavity in which the malleo-incudal

joint lies is exposed, and now we have the very important task of removing

the malleus and incus, or any remains that may be present, with the forceps

or with a sharp spoon’’ [40–42]. Zaufal would opt not to insert a drainage

tube, and instead would sew close the wound. His method was later practiced

using wide-angled, hollow chisels in place of the rongeur [30,41,42].

In 1890, Ludwig Stacke [43], a German otologist, proposed a relatively

novel approach to managing chronic inflammation of the middle ear. His

technique involved opening the mastoid from within outward, aiming toremove all diseased tissue. By operating initially within the canal, then

methodically removing successive layers of bone in the appropriate direction,

the necessary cavities would be exposed and all diseased tissue cleared.

His method described elevating the periosteum from the superior and

Fig. 8. Emanuel Zaufal (1837–1910) Czechoslovakian surgeon who, along with Stacke, is

credited with the introduction of the radical mastoidectomy operation. (From Politzer A.

Geschichte der Ohrenheilkunde, vol. 2. Stuggart (Germany): F. Enke; 1913.)

1204 SUNDER et al



posterior walls of the auditory canal, then, with a chisel, entering the tym-

panic cavity, followed by the attic and the antrum. His other contribution

to this technique was the creation of skin and periosteal flaps from theposterior and superior walls, which were then placed into the wound cavity

to facilitate wound healing and hasten epidermization.

Ludwig Stacke was born on April 14, 1859, in Rinteln, Germany. From

1877 to 1882, he studied in Wu ¨ rzburg, Halle, and Munich, receiving his doc-

torate in 1882. He was an assistant to Hermann Schwartze at Halle from

1882 to 1884. In 1886, he began working in Erfurt as a physician for the

care of ear and nose infections. He opened his own private clinic in 1887

and became a professor the same year. He died on January 13, 1918.

While reviewing the works attributed to Stacke, the authors noted thatthe article repeatedly cited as Stacke’s original paper describing his opera-

tion was, in fact, cited erroneously. Repeatedly cited in the literature as

Stacke’s original report was an early mis-citation: Stacke L. Stacke’s Oper-

ationsmethode. Archiv fur Ohrenheilkunde 1893;35:145. However, this refer-

ence correlates to an article by Professor K. Burkner in which a presentation

by Stacke is mentioned on page 145. This publication is in the proceedings

of the German Otological Society meeting in Frankfurt on May 20–21,

1893. The supposed paper was, in fact, merely a paragraph-long comment

by Stacke on a paper on chronic middle ear infection presented by ClausJansen. The title, Stacke’s Operationsmethode, was not used by Stacke,

but was applied by others, and certainly was not his article title. In his un-

titled comment, he refers to his original presentation at the Berlin conference

of 1890, and credits Zaufal for having accomplished the same operation

(with different methods) earlier. This error is an example of a commonly

observed phenomenon in medical history: an early mis-citation of a key his-

torical paper, followed by a cascade of repetitions by authors who neglected

to check the original reference. The correct article in which Stacke describes

his operation is an 1890 article in Archiv fur Ohrenheilkunde [43].As the radical mastoidectomy gained popularity, otologists published

their experiences and suggestions for improvement. In 1893, Sir William

MacEwan [44] outlined the suprameatal triangle, later to be called MacEwan’s

triangle. Opening of the mastoid antrum may be performed safely within

the triangle formed by the ‘‘posterior root of the zygoma above, the upper

and posterior segment of the osseous external meatus below, and an imagi-

nary perpendicular line uniting these two, extending from the most posterior

portion of the external osseous meatus to the zygomatic root.’’ MacEwan in-

troduced this technique to his European and American colleagues (Fig. 9).


The tide soon turned toward that of conservatism, with a goal of preserv-

ing hearing and middle ear function whenever reasonable. A quote by Tu-

markin [45] sums up the sentiments of many who condemned the surgery as




savage and unsuccessful: ‘‘Radical mastoidectomy is one of the most unsatis-factory operations in surgery. Before the operation the patient is more or less

deaf and has a troublesome otorrhoea; after the operation he finds himself just

as deaf, if not deafer, and only too often the discharge continues unabated.

Patients may well ask what is the justification for such a serious operation.’’

The credit for developing the procedure that is the predecessor of today’s

notion of the modified radical mastoidectomy (Fig. 10) has generally been

attributed to Gustave Bondy in 1910. However, Charles J. Heath, an En-

glish aurist, and William Soheir Bryant, an American otologist, reported

similar procedures during the same time frame [46–51].To exteriorize disease restricted to the antrum and attic, Bondy proposed

extending the bone excision to include the outer attic wall and bridge. He

intended to preserve pars tensa, and, whenever possible, the ossicles. Meatal

skin flaps were used to seal off the defect between the epitympanum and

mesotympanum, or, when the ossicles were preserved, the flaps would be

used to cover their exposed bodies [51–53].

Gustave Bondy was born in Austria in 1870 (Fig. 11) and received his

medical degree from the University of Vienna in 1894. In 1897, he began

working in the ear department of the General Poliklinic, where he remainedan assistant until 1907, when he became first assistant to Victor Urban-

tschitsch at the ear clinic, and several years later, a lecturer in otology.

Bondy became well-known for his 1907 work on the comparative anatomy

of the hearing organs, Beitra ¨ ge zur Vergleichenden Anatomie des Goho ¨ rogans

[54]. With an expertise in otologic surgery and the management of intracra-

nial otologic complications, he gained particular recognition for developing

Fig. 9. Sir William MacEwen (1848–1924). Scottish surgeon who described an eponymous tri-

angle useful in determining the safest route to the antrum. In this 1896 picture, shot during

a visit to San Francisco, he is demonstrating ‘‘MacEwen’s triangle’’ to Levi Cooper Lane,

for whom the Stanford Medical Library is named. (Courtesy of the Stanford University

Lane Medical Library, Stanford, CA.)

1206 SUNDER et al



criteria for the modified radical mastoidectomy procedure. He described us-ing curettes and chisels to carry out this surgery, which would eventually

carry his name. Bondy developed his criteria based on evaluation of the

postoperative results of his associate Erich Ruttin, who had performed

1000 radical mastoidectomies by 1910. Bondy noted that hearing worsened

after radical mastoidectomy in a subset of Ruttin patients. These patients

had normal tympanic membranes, intact ossicles, and relatively normal

hearing preoperatively, and had attic cholesteatomas that resulted from a de-

fect in the pars flaccida. Based on these observations, Bondy established in-

dications for performing the modified mastoidectomy, including intactossicles, attic cholesteatoma, and an intact tympanic membrane, except

for the pars flaccida [52,53,55]. In the years preceding World War II, Bondy

left Vienna for Australia, where he lived for the remainder of his life [55].

Although Bondy’s name has been steadfastly attached to the modified

mastoidectomy operation, several others, including American otologist

William Sohier Bryant in 1904 and British aurist Charles J. Heath in 1907,

described their experiences with similar procedures before Bondy.

William Sohier Bryant (1861–1956) graduated from Harvard University

in 1888, served on the faculties of Cornell University and New York Post-graduate Medical School, and was a member of the staff at the New York

Eye and Ear Infirmary [53]. At a meeting of the New York Otological

Society in late 1905, he detailed his experience with a modified radical mas-

toidectomy he had performed earlier that year [50,56].

In a 1904 Lancet article, Charles Heath first described an operation ‘‘with

a view of restoring hearing and arresting discharge in chronic suppurative

Fig. 10. Early illustration of modified radical mastoidectomy. (From Ballenger WL. The meato-

mastoid operation in chronic mastoiditis: the technique, rationale, and indications. JAMA

1908;51:1062–70.)




ear disease’’ [46]. In subsequent years, he presented the results of 400 cases

where the operation was performed [47,57]. Although his results demon-

strated the success of the modified radical operation, he had many critics

who suggested questionable ethics in operating on such a large number of

subjects in a short period of time, and questioned the indications [47]. Critics

suggested that many of Heath’s cases could have been handled equally well

with a simple mastoidectomy or with conservative management, without

surgical intervention.

The operations of Heath and Bryant failed to garner general acceptancebecause they failed to meet the requirements of the radical procedure

requiring that all areas of disease be exposed and exteriorized. In both op-

erations, the canal was partially removed to create an opening from the an-

trum to the meatus, but the ossicles and tympanic membrane, with a portion

of the superior canal wall, remained intact, potentially harboring disease. By

contrast, Bondy’s operation, by removing superior canal wall and exterior-

izing the attic, satisfied the requirements of the radical procedure, facilitat-

ing exposure of the site of chronic disease in the attic.

By the 1930s, the mastoidectomy had evolved into a generally acceptedotologic procedure. Many other surgeons, not reviewed in this article for

brevity’s sake, played a role in its development. The concepts and experi-

ences of the barber-surgeons, aurists, general surgeons, and otologists of

the preceding 4 centuries set the foundation for this one operation, the cor-

nerstone of otology. Endowed with a rich history, the future of mastoid sur-

gery promises to be equally momentous.

Fig. 11. Gustave Bondy (1870–1954) Austrian surgeon (University of Vienna) who popularized

(but was not the first to perform) a modified radical mastoidectomy in which part or all of the

middle ear structures were preserved in an effort to preserve hearing. (From The Institute for the

History of Medicine, Medical University, Vienna, with permission.)

1208 SUNDER et al



Acknowledgments

The authors appreciate Dr. Stefan Heller for his help with translation of

German reference material.

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Enke; 1913.

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[8] Cawthorne T. The surgery of the temporal bone. J Laryngol Otol 1953;67:377–91.

[9] Mudry A. Contributionof Ambroise Pare ´ (1510–1590) to otology.OtolNeurotol1999;20:809–13.

[10] Liston SL. Ambroide Pare and the king’s mastoiditis. In: International Society of the History

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[11] Singer DW. Selections from the works of Ambroise Pare. London: John Bale, Sons &

Danielsson, Ltd; 1924.[12] Pare A. The apologie and treatise of Ambroise Pare. New York: Dover Publications; 1968.

[13] Sonnenschein R. A brief consideration of the history of the development of the mastoidec-

tomy. Ann Med Hist 1935;8:500–10.

[14] Packard FR. Life and times of Ambroise Pare. 2nd edition. New York: Hoeber; 1926.

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[23] Dezeimeris JE. De la perforation de L’apophyse mastoide. Dans diverses affections de ses

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[46] Heath C. The restoration of hearing after removal of the drum and ossicles by a modification

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ossicles or the loss of hearing. Lancet 1907;169(4365):1146–9.

1210 SUNDER et al



Middle Ear Atelectasis: What Causes

It and How Is It Corrected?

Christopher J. Danner, MDOtology/Neurotology/Skull Base Surgery, Tampa Bay Hearing and Balance Center,

Tampa Bay, FL, USA

Pathogenesis

The three main mechanisms that regulate middle ear pressure are [1]:

(1) Gas diffusion through the middle ear mucosa

(2) Pressure buffer of the mastoid air cell system

(3) Gas exchange through the eustachian tube

Mucosal respiration

The middle ear mucosa exchanges gas in a similar fashion to alveoli in the

lung. The direction of gas exchange is predicated on the differences in partial

pressure of the component gases in the middle ear cleft and mucosa. Inflam-

matory processes that affect the thickness and blood flow of the middle ear

mucosa have a direct effect on the rate of diffusion. Generally, when the mu-

cosa is healthy, there is an equal rate of exchange because oxygen and nitro-

gen are absorbed by the mucosa [2] at the same rate at which carbon dioxideis expelled (Fig. 1) [3,4]. However, the more inflamed and vascular the mu-

cosa, the greater the rate of gas absorption [5].

Gas exchange does not occur uniformly throughout the middle ear. The

epithelium covering the middle ear space is heterogeneous, changing from

a respiratory epithelium in the anterior inferior part of the middle ear cleft

to a cuboidal, richly vascularized epithelium around the antrum [6]. It is

here, around the antrum, that most of the gas exchange occurs. Because

the convective forces in the middle ear are small, and the air in the middle

ear is relatively static, the concentrations of gases in the middle ear approachthe same concentrations as venous blood (Table 1) [7]. The more vascular-

ized the mucosa, the greater the rate of gas exchange.





39 (2006) 1211–1219





Because the gaseous exchange across the mucosa depends on the differ-

ence in partial pressures of each component gas between the blood and tym-

panic cavity, differences in blood flow can have dramatic effects on middle

ear respiration [8,9]. Fluctuations in blood flow based on body position

have a significant effect on the respiratory/diffusion rate [10,11]. Sade and

Luntz [7] serially examined 52 subjects who had completely atelectatic tym-

panic membranes. They found one half had inflated middle ear spaces

within 1 minute of awakening from a night’s sleep. After the subjects

were upright, the tympanic membranes quickly reverted back to their nor-

mal atelectatic position.

Fig. 1. The exchange of gas between the middle ear cleft and vascular space. The font size is

proportional to the partial pressure of the represented gas. Oxygen and nitrogen diffuse out

of the middle ear cleft into to the vascular space, and carbon dioxide does the reverse. Arrows

represent direction of diffusion.

Table 1

Different partial pressures of key component gases in the middle ear, venous system, and

environment

Gas Air (mm Hg) Venous blood (mm Hg) Middle ear (mm Hg)

H2O 3.7 47 47

CO2 0.3 46 40–58

O2 159.0 41 41–94

N2 597.0 574 560–632

Total 760.0 708 760

(Data from Sade J, Luntz M. Middle ear gases. Acta Oto Rhino Laryngologica Belgica

1992;46(4):355–360.)

1212 DANNER



Mastoid volume and pressure

Does the lack of aeration lead to the development of middle ear disease,

or does middle ear disease lead to lack of aeration? Regardless of the cause,

most would agree that lack of mastoid aeration is associated with middle ear

disease. The anatomic and physiologic properties of the mastoid air cell sys-

tem contribute to the homeostasis of the middle ear. The anatomic volume

of the aerated middle ear space affects how the ear drum behaves. This re-

lationship between volume and pressure is best represented by Boyle’s law,

which states that at a constant temperature, pressure multiplied by volume

equals a constant (P V ¼ C). Thus, pressure and volume are inversely

proportional.

Small mastoid volumes tend to cause greater changes in pressure. There-

fore, the volume of mastoid aeration plays a significant role in middle ear

compliance and the resultant forces imparted on the tympanic membrane

[12,13]. Fluctuations in middle ear pressure in the presence of a small mas-

toid cavity will result in greater forces applied to the tympanic membrane,

when compared with the same pressure changes in a larger, more aerated

mastoid. The drum is a compliant structure that will move medially or lat-

erally to compensate for these increases or decreases in middle ear pressure.

The physical properties of the mastoid, represented by Boyle’s law, are not

the only factors predisposing to atelectasis.

The physiologic properties of the mastoid air cell system are also impor-

tant in middle ear homeostasis. Mastoid aeration increases the mucosal sur-

face area. This increased surface area, caused by folds in the mastoid mucosa,

increases the respiratory properties of the middle ear, similar to the way the

alveoli increase gas transfer in the lung. These different folds in the mucosa

increase the amount of gas transfer across the mastoid mucosa [14,15].

The amount of mastoid aeration is important in regulating middle ear

pressure for two reasons. First, the physical properties of mastoid volume

affect compliance: the greater the volume, the more compliant the system.

Second, the surface area affects mucosal respiration: the greater the surface

area, the more efficient the middle ear respiration. Both are important in the

regulation of middle ear pressure [12,13].

In 1990, Avraham [16] studied the effect of mastoidectomy on middle ear

aeration. Two groups were followed, one with mastoidectomy and one with-

out. The groups did not differ regarding age or extent of disease. He fol-

lowed more than 100 ears for a minimum of 4 years and found that the

group that did not have a mastoidectomy had a more aerated middle ear.

Eustachian tube function

The eustachian tube connects the middle ear cleft with the outside envi-

ronment and helps keep the pressure on either side of the ear drum equal.

The eustachian tube is normally closed, opening only when the tensor

1213MIDDLE EAR ATELECTASIS



palatini contracts. When the eustachian tube does open, the volume of gas

exchange is around 1 mL with every swallow in nondiseased states [1]. Often,

the eustachian tube is hypofunctional in atelectatic ears. This decrease infunction leads to a proportional decrease in middle ear ventilation, causing

a negative pressure to form as the middle ear mucosa absorbs nitrogen from

the air in the middle ear cleft.

Although common, a hypofunctional eustachian tube is not the only

cause of middle ear atelectasis. A hyperfunctional/patulous eustachian

tube can produce disconcerting symptoms of autophony. Patients who

have a hyperfunctional eustachian tube will often sniff to create a negative

pressure and voluntarily close their eustachian tube. This sniffing can also

lead to the development of middle ear atelectasis [17].

Tympanic membrane

The tympanic membrane is composed of two main structures: the pars

tensa and the pars flaccida. These structures are histologically different.

The pars tensa has both circular and radial collagenous fibers that are

well organized and relatively thick [18]. The posterior portion of the pars

tensa has a thinner lamina propria than the anterior portion, and also has

increased vascular supply. The lamina propria of the posterior superiorportion of the pars tensa has thinner, more sparsely distributed circular

collagenous fibers. This increased vascular supply makes it more prone to

damage from an inflammatory process. The inflammatory changes disrupt

the collagenous support structures, making the ear drum weaker and

more prone to retraction [19]. The pars flaccida has a thin lamina propria

and the collagenous fibers are thinner, less organized and more sparsely dis-

tributed in the ground substance. The structural differences within the tym-

panic membrane cause the pars flaccida and the posterior portion of the pars

tensa to be inherently weaker and more susceptible to changes in middle earpressures [1,20]. The lack of a fibrocartilaginous ring at the notch of Rivinus

contributes to the inherent weakness of the pars flaccida.

The viscoelastic properties of the tympanic membrane allow it to com-

pensate and therefore change the volume of the tympanic cavity to buffer

sudden changes in middle ear pressure.

Luntz [20] stated that pars flaccida retractions are more common than

pars tensa retractions. If there was only one retraction present, there was

a more than 70% chance that it was a pars flaccida retraction, which makes

intuitive sense because the pars flaccida has less collagenous support and ismore compliant than the pars tensa.

Classification

Several classification systems exist for middle ear atelectasis. In 1976,

Sade [21] proposed the first classification system that graded both pars tensa

1214 DANNER



and pars flaccida retractions. In 1982, Tos [22] described a classification sys-

tem solely devoted to Shrapnell’s membrane. Ohnishi [23] had two different

classifications systems for atelectatic ears: one for draining ears and one for

ears that were dry.

In 2000, Dornhoffer [24] described a classification system for pars tensa

retractions that was similar to Sade’s but differed in its representation of

the worse atelectatic ears. Both Sade’s and Dornhoffer’s classification

schemes described the atelectatic ear, which has a drum that is retracted

but does not touch any middle ear structures, as grade one (Figs. 2 and 3).

In a grade two atelectatic ear, the drum is retracted and touches the incus

or stapes (Fig. 4). In a grade three retraction, the drum progresses inward

and touches the promontory (Fig. 5). The description of the grade four ate-

lectatic ear is the point where Sade’s and Dornhoffer’s classification systems

diverge. In a grade four atelectatic ear, the drum is retracted around the

edge of the scutum, and all aspects of the drum cannot be visualized

Fig. 2. Normal ear.

Fig. 3. Grade one atelectatic ear. All or a portion of the drum is retracted, but does not touch

any middle ear structures. All aspects of the drum are visible.




(Figs. 6 and 7). Sade uses the grade four classification to describe an atelec-

tatic ear that has a tympanic membrane adheased to the middle ear struc-

tures, but does not comment on whether the entire drum can be seen easily.

Dornhoffer, on the other hand, is less concerned about the adhesive prop-

erties of the tympanic membrane, but is more concerned that all aspects of

the drum can be visualized adequately. It is the author’s opinion that visu-alizing all aspects of the drum is more important than its adhesive proper-

ties. Adequate visualization enables the physician to monitor the ear

confidently. Once the drum retracts beyond what can be visualized, the phy-

sician has no way of adequately assessing the size or depth of the retraction.

The leading edge of a retraction may be just beyond the physician’s line of

sight, or the retraction may be quite large and fill the epitympanum and

mastoid (see Fig. 7). Depending on the inflammatory status of the middle

ear mucosa and the amount of debris accumulated in the retraction, even

Fig. 4. Grade two atelectatic ear. The drum is retracted and draped over the incus or stapes.

The drum is not touching the promontory. All aspects of the drum are visible.

Fig. 5. Grade three atelectatic ear. The same as grade two, but the drum is now touching the

promontory. All aspects of the drum are visible.

1216 DANNER



high-resolution CT scans of the temporal bone can be very misleading and

often misrepresent the extent of the atelectatic drum. By contrast, if all as-

pects of the drum are seen easily, but the drum is adherent to the middle ear,

the ear can be followed. Sade also explains a grade five atelectatic ear as an

ear that perforates spontaneously.

In 1980, Sade [25] reported on 308 ears that were followed for an averageof 3 years. He described the progression of the atelectatic ear. He found that

approximately one half of the ears either stayed the same or improved.

Smaller retractions, grade three or lower, were more likely not to progress.

He also felt that pressure equalization tubes were ineffective, stating that the

tubes did improve the retraction temporarily, but inevitably would extrude,

and the ear would retract to its original state.

The classification system is useful in documenting and following the ate-

lectatic ear and is helpful with determining management. The type of

Fig. 6. Grade four atelectatic ear. The same as grade three, but the drum is now retracted

around a corner. All aspects of the drum are not visible.

Fig. 7. Grade four atelectatic ear. The shaded area (A) represents the portion of the ear drum

that is not visualized. This area may be relatively shallow, as represented here, or can be quite

large and retract into the mastoid.




medical or surgical management is determined by the atelectatic grade of the

TM and whether the pathologic state is progressive or stable (Table 2).

Grade one or two retractions are often managed medically by encourag-

ing the patient to insufflate the middle ear space by attempting to blow

through a pinched nose. If the patient has difficulty accomplishing this

task, nasal steroid spray is recommended.

Grade three retractions are managed generally with Valsalva and nasal

steroids; usually, if the drum fails to improve, a pressure equalization tube

is recommended. During routine follow-up, if the drum has progressed to

a grade three with medical management, or has adhesive characteristics,

then a cartilage tympanoplasty is often recommended.

Grade four retractions are managed by performing a cartilage tympano-

plasty and a mastoidectomy for cholesteatoma, as needed. Typically, unless

the retraction pocket extends into the antrum and mastoid, a mastoidectomy

is not performed [26].

Summary

Many options are available to manage a patient who has atelectatic ears.Establishing normal middle ear ventilation and aeration is the cornerstone

to successful control of these ears. Often, medical management with nasal

steroids and decongestants is all that is needed. If recurrent infections

have weakened the tympanic membrane progressively, or the middle ear en-

vironment is so severe that medical management does not correct the prob-

lem, then surgical correction is often necessary.

References

[1] Ars B. Tympanic membrane. Retraction pocket. Acta Otorhinolaryngol Belg 1995;49(2):

163–71.

[2] Cantekin EI, Doyle WJ, Phillips DC, et al. Gas absorption in the middle ear. Ann Otol Rhi-

nol Laryngol Suppl 1980;89(3 Pt 2):71–5.

[3] Bylander A, Tjernstrom O, Ivarsson A, et al. Eustachian tube function and its relation to

middle ear pressure in children. Auris, Nasus, Larynx 1985;12(Suppl 1):S43–5.

Table 2

Classification and treatment of middle ear atelectasis

Atelectasis grade Treatment1 Observation, Valsalva, nasal steroids as needed

2 Observation, Valsalva, nasal steroids as needed

3 Observation, Valsalva, nasal steroids as needed

Consider pressure equalization tube if drum fails to improve

Consider tympanoplasty if drum is adherent cartilage

4 Cartilage tympanoplasty, mastoidectomy as needed

1218 DANNER



[4] Yamamoto Y. Gas exchange function through the middle ear mucosa in piglets: comparative

study of normal and inflamed ears. Acta Otolaryngol 1999;119(1):72–7.

[5] Doyle WJ, Seroky JT, Alper CM. Gas exchange across the middle ear mucosa in monkeys.

Estimation of exchange rate. Arch Otolaryngol Head Neck Surg 1995;121(8):887–92.

[6] Buch NH, Jorgensen MB. Eustachian tube and middle ear. Embryology and pathology.


[7] Sade J, Luntz M. Middle ear gases. Acta Otorhinolaryngol Belg 1992;46(4):355–60.

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Eustachian Tube Functionand the Middle Ear

John W. Seibert, MDa,Christopher J. Danner, MDb,*

aDepartment of Otolaryngology, Washington University School of Medicine,

St. Louis, MO, USAbOtology/Neurotology/Skull Base Surgery, Tampa Bay Hearing and Balance Center,

Tampa Bay, FL USA

The eustachian tube (ET) has three physiologic functions. These are (1)pressure regulation, (2) protection of the middle ear from pathogens/foreignmaterial in the nasopharynx, and (3) clearance of the middle ear space [1].

It is well known that eustachian tube dysfunction (ETD) is linked tochronic secretory otitis media [2]. Other more invasive diseases can also oc-cur with ETD. When the tubal mechanism fails, either in passive or activefunction, a series of events can occur in the middle ear space that variesfrom a mild retraction to fulminate cholesteatoma.

History

The first modern-era researcher of the ET was Bartolomeus Eustachius.He was a 16th century anatomist who taught at the Collegia della Sapienzain Italy. Eustachius, for whom the ET is named, studied several areas in thehuman body including the ET [3]. Further work was done by AntonioValsalva, (1666-1723). Valsalva was a Professor of Anatomy at Bolognaand is most recognized for his middle ear insufflating maneuver that bearshis name. Valsalva is credited with naming the auditory tube, the eustachian

tube, and describing its function [4].Adam Politzer is probably best known for his contribution to otology

and probably considered the greatest otologist of the 19th century. One of his legacies was a hand-held air bag that allowed insufflation of the middle

* Corresponding author.E-mail address: [email protected] (C.J. Danner).




39 (2006) 1221–1235





ear space or politzerization, as the inventor termed it. Further therapies de-veloped by Politzer included a primitive middle ear ventilation tube [5].

Embryonic development

The development of the eustachian tube and middle ear occurs as an outpouching of the pharynx that forms the tubotympanum and the pneumatizedtemporal bone. [6] The cartilaginous portion of the eustachian tube undergoesthe majority of the growth seen in utero. [1] During development the ET pro-vides a continuous sheet of epithelial cells to create the middle ear lining. [7]One difference between the middle ear mucosa and the ET mucosa is thatthe epithelium of the ET differentiates into respiratory epithelium (pseudos-

tratified ciliated columnar), whereas the middle ear epithelium does not.However, more posteriorly in the middle ear, away from the ET orifice,some simple nonciliated cuboidal epithelium is present [8]. This distinctionfound in ET mucosa provides a more effective, inherent protective compo-nent. Unique characteristics of the ET include the presence of more mucuscells and accessory glands when compared with the middle ear mucosa [9].

Compared with adults, the position of the infant ET is 10 from theFrankfort horizontal plane. This angle is different in adults whose tube ispositioned at a 45 [1]. Among other differences in adult and pediatric

ETs, a less angled ET in the pediatric population has been thought to beresponsible for the increased incidence of middle ear pathology. However,some researchers have found that active muscle function, rather than passiveclearance and impedance of the ET, is responsible for the decreased diseasestate seen in adults [10,11].

A smaller or partially obstructed ET does not necessarily correlate withthe risk of disease or even active disease. Using an in vivo model, Sadeand coworkers [12] in 2004 found that narrowing of the ET alone did notprohibit natural gas flow into the middle ear space with a swallowing

maneuver. Although the narrowed ET was open for a brief period, thiswas sufficient to overcome a negative pressure.

Anatomy

The length of the ET has been reported to be between 31 and 38 mm [13].The normal orientation of the ET is downward, anterior, and with a medialrotation. With this positioning, the ET creates an angle of about 45 and 30

to 40 with the sagittal and horizontal planes, respectively [9].

The ET is made up of bone, cartilage, and fibrous tissue. The bonycomponent is approximately 12 mm in length, whereas the cartilaginousis about 24 mm in length. This longer portion is described as a triangularplate of elastic fibrocartilage. The base of the tube forms the torus tubarius,which is posterior to the nasopharyngeal opening the eustachian tube [9].

Blood supply to the ET and its supporting structures originates from thedeep auricular branches of the internal maxillary artery, ascending

1222 SEIBERT & DANNER



pharyngeal artery, and the ascending palatine artery [14]. Sensory and mo-tor innervation of the ET is supplied by a branch from the otic ganglion,

sphenopalatine nerve, and the pharyngeal plexus from branches of the glos-sopharyngeal nerve. Sympathetic branches innervate the ET from the sphe-nopalatine ganglion, otic ganglion, glossopharyngeal nerve, petrosal nerves,and the carticotympanic nerve. Parasympathetic innervation is from thetympanic branch of the glossopharyngeal nerve [1,14].

Muscles of the ET

Four muscles are associated with the ET, which through a complex inter-

action, assist with equilibrating middle ear pressure. These muscles are: (1)tensor veli palatine, (2) levator veli palatine, (3) salpingopharyngeus, and (4)tensor tympani.

The tensor veli palatini (TVP) is a thin muscle lateral to the levator velipalatini. The medial portion of this muscle is the primary dilator of the ET.The origin of the muscle arises from three locations. One origin is at the baseof the medial pterygoid plate on the scaphoid fossa. The second is from thespina angularis of the sphenoid, and the third is from the lateral wall of thecartilaginous eustachian tube. The TVP muscle descends and inserts on

a tendon at the pterygoid hamulus [9,15].Levator veli palatini is a thicker muscle compared with the TVP and lies

lateral to the choanae. Its origin is from two anatomic sites. The first is theinferior surface of the apex of the petrous part of temporal bone. The secondorigin is the medial lamina of the cartilage of the ET. The muscle extendsabove the superior pharyngeal constrictor merging with the opposite levatormuscle at midline [9,15].

Salpingopharyngeus originates from the inferior portion of the ETextending downward joining the pharyngopalatinus muscle and assists

with elevation of the pharynx and opening of the ET with deglutition [9,15].Tensor tympani is a large muscle encased in a bony canal above the os-

seous portion of the ET. The tensor tympani origin involves three locations.The first is the cartilaginous portion of the ET, and the second is the greaterwing of the sphenoid. The third is attachments to the bony canal in whichthe muscle travels. The tensor tympani insertion is at the manubrium of the malleus [9,15].

Function and dysfunction of ET

Normally, the ET stays closed and opens when necessary to equalizepressure. Other functions include clearance of middle ear fluid while atthe same time preventing nasopharyngeal secretions refluxing into the mid-dle ear space.

Ghadiali and coworkers [16] looked at the physiologic function of the ETand found that the ET opening was highly sensitive to the applied muscle

1223ET FUNCTION AND THE MIDDLE EAR



forces and relatively insensitive to cartilage elastic properties. In their anal-ysis of the muscle forces (tensor and levator veli palatini) and soft tissue

elastic properties, luminal dilation of the ET was caused by muscle contractioncausing medial-superior rotation of the medial lamina, which in turn causeda deformation of fatty tissue surrounding the ET (Ostmann’s fat pad) [16].

Bluestone and colleagues [17] reported on the progression to cholestea-toma describing how acquired cholesteatoma can develop. This sequenceof pathogenesis (Fig. 1) involved functional failure of the ET, leading to in-creased negative middle ear pressure, atelectasis of the tympanic membrane,formation of a retraction pocket in attic or posterior-superior quadrant, andsubsequent adhesive otitis media [17]. Although otologic surgery generally

has been successful in clearing cholesteatomas, those patients with nonfunc-tioning or marginally functioning ETs traditionally have had less successfulresults. Reoccurrence rates in pediatric patients have been linked to poor ETfunction [18]. ET dysfunction has also been shown to adversely affect post-operative hearing results in children [19].

Although the normal physiologic state of the middle ear is to haveequal pressure between the middle and lateral sides of the tympanic mem-brane, some patients will prefer a negative pressure in their ear. Bunneand coworkers [20] in 1999 found that these patients complained of hyper-

acusis and autophony with the tympanic membrane in the normal position.Two groups with retracted tympanic membranes and sound disturbances

Functional failure of the

ET

Increased negative

middle ear pressure

Atelectasis of the

tympanic membrane

Retraction pocket in attic

or posterior-superior

quadrant

Adhesive otitis media

Fig. 1. Pathway of acquired cholesteatoma. (From Bluestone CD, Cantekin EI, Beery QC, et al.

Function of the eustachian tube related to surgical management of acquired aural cholesteato-

ma in children. Laryngoscope 1978;88(7 Pt 1):1155-64; with permission.)




smokers compared with nonsmokers. This finding, however, has beentempered by work by Coggins and colleagues [29] and Antonelli and co-

workers [30] who found passive tobacco smoke in the animal model tohave little effect on otitis media. According to Dubin and coworkers [31],passive smoke does affect the ET function, but may play only part of a role in causing middle ear disease.

Reflux

White and coworkers [32] determined that exposure to gastric contentsin the nasopharynx caused a significant ET dysfunction in an animal

model. Their experiment found that middle ear pressure regulation andmucociliary clearance of middle ear contents were disabled. Heavner andcoauthors [33] in 2001 had previously published similar results in ananimal model.

Cleft palate

Previous research has found that children with a cleft palate have an in-creased risk of middle ear pathology [34]. The incidence of ETD has been

quoted as high as 79% in patients with cleft palate and cleft lip/palate asfound by Goldman and coworkers [35]. Interestingly, in their cohort, only2 of 110 patients had acquired cholesteatoma.

Arnold and coworkers found patients with bilateral cleft palate to havea nearly horizontal course of the ET, possibly worsening symptoms. Al-though TVP muscle had a bony attachment on either side, the levator velipalatini muscle also showed an abnormal course. This finding led the inves-tigators to conclude that, during contraction, an aberrant obstruction of theET may result.

Unfortunately, some cleft patients continue to have ETD postoperativelyand as they growth into adulthood. One third of the adults in a 2006 studyby Gudziol and Mann [36] with cleft lip and palate had persistent ETD.

Radiation

Treatment of nasopharyngeal malignancies with external beam radiationhas detrimental effects on the surrounding structures, especially the ET.Multiple investigators have found patients with early and late middle ear pa-

thologies secondary to iatrogenic ET injury [37–39].

Reduced mastoid air cell system

The presence of a mastoid air cell system has been reported as an impor-tant criterion postoperatively to act as a pressure buffering system. How-ever, this function is dependent on having healthy mastoid mucosa [40].




Nitrous oxide

A study by Teixeira and coworkers [41] in 2005 found that approximatelyone half of patients who received 50% nitrous oxide under general anesthe-sia, had Type C tympanograms postoperatively, compared with a Type Atympanogram preoperatively. This change in middle ear pressure is likelysecondary to absorption of nitrous oxide, leaving a decrease in gaseous vol-ume in the middle ear space.

ET, mastoid, and cholesteatoma

The status of the mastoid has been found to affect the progression of cho-lesteatomas. In some instances, this factor was more important than thepresence of poor ET function. Hasebe and coworkers [42] in 2001 comparedthree groups with varying degrees of tympanic membrane retraction. Thisgrouping included patients with a severe attic retraction pocket, patientswith cholesteatoma but could be treated conservatively, and patients withcholesteatoma but needing surgery. All three groups had ETD, but no sig-nificant difference in the function of the ET was found among the threegroups. Progression of the cholesteatoma appeared to be related more to

the ventilatory condition of the mastoid rather than the ET function. Thisobservation was based on less aeration seen in the surgery group comparedwith the nonsurgical group [42].

When the ET fails to equalize pressure, a negative pressure in the middleear space occurs. The mastoid is seen by many researchers as a buffer zonefor the middle ear and tympanic membrane allowing some equalization of this abnormal pressure. Cinamon and Sade [43] developed a model to eval-uate how pressure homeostasis of the middle ear can be maintained. Theyfound that the worst "model" for adapting to these changes was a middle

ear space with a small mastoid. The investigators proposed that this ana-tomic finding may lead to patients developing compensatory bufferingmechanisms, such as retraction or fluid accumulation, which reduced middleear volume [43].

Retraction pockets are well known to result from ETD. Wolfman andChole [44] in 1986 found cauterized ETs of the Mongolian gerbil resultedin a progressive retraction in 75% of the animal in a 16-week period.

Examining the retracted tympanic membrane, Paparella and coworkers[45] described epithelial and subepithelial changes such as keratin accumu-

lation, papillary growth, mucosal adhesion, irregular epithelium, and bonedestruction. Although reasonable theories exist to describe why cholesteato-mas occur, it is not entirely known what allows retraction pockets to evolveinto cholesteatomas (Fig. 2).

Cholesteatomas are known to arise more commonly in the pars flaccida.The reason for this may be that there is poorer aeration in this area, espe-cially in the area of the tympanic isthmus. Kobayashi and colleagues [46]




in 1994 compared computed tomography scans of 53 patients with retrac-

tions in the pars flaccida, including those with cholesteatoma. Their resultsfound little association with this blockage and progression of a cholesteatoma.

Evaluation of the ET

Assessment can be started initially by taking a thorough history. A typ-ical ETD patient will complain of fullness or clogging of the ears, pain ordiscomfort, hearing loss, tinnitus, and dizziness. Most concerning to these

patients is when these symptoms cannot be relieved by swallowing, yawning,or chewing.

Physical examination

Using pneumatic otoscopy, an examiner can evaluate the mobility of thetympanic membrane. Stiffness or middle ear effusions are suggestive of ETD. Indirect nasopharyngoscopy, using a small dental mirror, representsanother manner to visually inspect the posterior nasopharynx and proximal

opening of the ET. Pathology such as adenoid hypertrophy or mucosaledema can be seen.It is well known that a rigid or flexible nasal endoscope allows the exam-

iner to visualize the nasopharyngeal opening of the ET. Usually 30 or 70

rigid Hopkins rod endoscopes provide the best visualization. Other re-searchers have advocated using 0.8-mm flexible fiberscopes to evaluatebeyond the isthmus of the ET and even into the middle ear cleft [47,48].

Pars Flaccida

Retraction with debris

Pars Tensa Retraction

Robert W. Seibert, MD

Fig. 2. Retracted tympanic membrane caused by ETD. (Courtesy of Robert W. Seibert, MD,

Little Rock, AK.)




ET testing

Some researchers have reported that tests for evaluating ET function arenot reliable [49]. However, most agree that there are objective and subjectiveassessments helpful in studying ETD.

A tympanogram plots a measure of how energy is transmitted throughthe middle ear. When pressures between the middle ear space and the earcanal are equal, a normal or ‘‘type A’’ tympanogram is recorded. Abnormaladmittance in the form of a retraction or stiffness will result in a plottedgraph know as a ‘‘type C’’ or ‘‘type B’’ tympanogram, respectively [50].

Other subjective testing includes the Valsalva test, which involves patientsholding their nose and blowing out with a closed mouth. The Toynbee test isa similar maneuver. In this test, patients hold their nose and swallow. Whilepatients swallow, the examiner can visually inspect the tympanic membraneand evaluate for movement. This exercise generates a positive pressurewithin the nasopharynx, followed by a negative pressure phase and isconsidered positive when there is an alteration in middle-ear pressure asassessed by pneumatic otoscopy before and after the maneuver. Negativemiddle ear pressure or temporary negative middle ear pressure followedby return to ambient pressure after the Toynbee test usually is indicativeof normal ET function [51].

In the Politzer test, one of the patient’s nostrils is occluded with a rubberballoon as the examiner pinches the other nostril tightly. The patient ele-vates the palate by swallowing or phonating. The examiner then forces airinto the closed nasal cavity from Politzer’s bag. Air can be heard goinginto the middle space with an auscultation device. The examiner can alsovisually compare the tympanic membrane before and after the procedureto determine its relative patency [52].

A final testing mechanism is sonotubometery. In this procedure, a soundsource is applied to the nostril as a microphone in the external auditorycanal records the transmitted sound. Sound levels are measured as the ETopens and closes. The advantage of this diagnostic test is the ability to eval-uate the ET with or without an intact membrane under physiologic condi-tions [53].

Medical treatment of ET dysfunction

A review of the literature finds that there is no clear consensus on oral/topical medications for the treatment of ETD. Some of the more pertinent

studies are mentioned. van Heerbeek and coworkers [54] studied outcomesof using pseudoephedrine in children already treated with pressure equaliza-tion tubes. Their findings showed there was no significant effect on ETfunction in children who used this topical decongestant.

In a double-blind, placebo-controlled, crossover study, Cantekin andcolleagues [55] in 1980 found that children without an upper respiratorytract infection had a lower closing pressure of the ET after taking




a decongestant-antihistamine combination compared with their controlgroup. In the presence of normal mucosa, this study confirmed that some

effect on the performance of the ET was possible.Dexamethasone has shown promising results. Silverstein and coworkers

[56] in 2003 published their findings in a small group of chronic ETD pa-tients that benefited from direct treatment of the ET with dexamethasone.This application, however, required that a pressure equalization tube beplaced first and that the medication be applied transtympanically. Shapiroand colleagues [57] in 1982 showed increased benefit in achieving normalmiddle ear pressure and tympanic membrane mobility with aerosolizednasal dexamethasone. This positive result was tempered by a concern raised

on cortisol levels, which were lowered in two of the study patients.This early success of steroids on ETD laid a foundation on which other

studies using less potent treatments could be built. Tracy and coauthors [58]found that intranasal beclomethasone may be a useful adjunct to prophylac-tic antibiotic treatment of chronic middle ear effusion. The investigators re-ported that patients had a more rapid improvement in the first 8 weekscompared with the antibiotic-alone treatment arm. Although this studydoes not address the functional status of the ET itself, one can inferimprovement with the resolution of the effusion.

A later study by Karlidag and coworkers [59] in 2002, failed to duplicatethe results. These researchers found no statistical difference in the nasal ste-roid treatment group compared with the antibiotic alone group. However,both groups were more effective than the control (no treatment). The inves-tigators blamed the lack of significance on the sample size. A previous studyby Ruohola and colleagues [60] in 2000 may weaken this hypothesis. Thislarger study evaluated 210 children. Those patients who received intranasalsteroids had no statistical difference in the development of acute otitis mediacompared with the placebo group. Again, although ET physiologic status

was not measured directly, failure to treat the targeted anatomy can beattributed to a poor-functioning ET.

Surgical treatment of ET dysfunction

Insertion of pressure equalization tubes (PET) had been the mainstay sur-gical treatment of ETD. Although the pressure difference between the mid-dle ear and the external auditory canal is resolved immediately with thisprocedure, little effect can be seen the ET itself. Several investigators have

found that active tubal function does not change after PET insertion and re-mains at the same poor level postoperatively [61,62]. However, van Heer-beek [61] and coworkers did identify changes to the ET seen in the formof passive tubal function. These patients had a significant increase openingpressure compared with preoperative measurements.

Fuldaer ventilation surgery proposed by Kaftan and Draft [63] in 2000combined different methods to improve ventilation of the middle ear. The




surgeon performed mastoidectomy, posterior tympanotomy, and removal of the incus and the head of the malleus with an interposition of the incus. Af-

ter the tympanic membrane was reconstructed with a cartilage-perichon-drium graft, a tube was placed into the middle ear orifice leading to thenasopharynx (Wright-tube). This aggressive approach showed some successin preventing disease. Specifically, approximately 26% of those patients withchronic ETD who have not responded to previous surgeries required subse-quent revision surgery for recurrent cholesteatoma. However, other investi-gators have gained similar results for preventing recurrent cholesteatoma inpatients with ETD by performing mastoid and epitympanic obliterationtechnique alone [64].

Once patients have been determined to have poor mastoid aeration in asso-ciation the ETD, little can be done to correct these areas surgically. This theoryis based on the fact that patients with poor ET function are more likely to haveretraction pockets and recurrent cholesteatoma. Mastoid obliteration hasshown promising in preventing this recurrent disease owing to malfunctioningET by eliminating the potential space for development [64,65].

One of the few procedures to treat proximal dysfunction of the ET is lasereustachian tuboplasty. This technique, proposed by Kujawski and Poe [66],provides a means to treat intractable ETD. In this procedure, a CO2 or

a 980-nm diode laser was used to obliterate mucosa and cartilage fromthe luminal posterior wall of the ET. A 65.21% success rate at 3 yearswas reported with minimal postoperative complications.

Adenoidectomy for ETD remains a controversial topic. Bluestone andcolleagues [67] in 1975 found that children with obstructive adenoids of the nasopharynx and proximal opening of the ET showed some benefit afteradenoidectomy in active opening and closing of the ET. Other investigatorshave found that adenoidectomy did not affect either passive or active open-ing or closing pressures of the ET [68–70].

ETD is a risk after cholesteatoma surgery. Chao and coworkers [71]1996 looked at a 5-year follow-up of postoperative tympanomastoidec-tomy patients. Sixty-six percent of these patients had a retraction pocket,most obviously seen near the scutum defect. Although the investigatorshad difficulty determining whether the ETD was the etiology or the se-quelae of a cholesteatoma, it is known that these sequelae likely increasesthe risk of reoccurrence.

Complications

Cholesteatoma is the most worrisome complication for patients withchronic ET dysfunction. Other possible sequelae include retraction, effusion,and atelectasis. Each of these pathologies can be associated with conductivehearing loss of varying degrees.

Aside from the complaints of discomfort with ETD, other traumatic eventscan occur in these patients in the form of otic barotraumas. Because of their




inability to equalize pressure normally, patients with ETD have an increasedrisk of injury to the tympanic membrane when involved in activities such as

descending and ascending in aircraft, scuba diving, and driving in higher ele-vations. Sade and colleagues [72] in 2003 reported that during a commercialplane flight, the middle ear has to equalize about 20% of its gas volumewith the ambient pressure. This equalization must take place within 15 to 20minutes of ascent and descent, otherwise otic barotraumas can result. An in-teresting note, chronic ear patients had a smaller mastoid air cell volume.When compared with patients with history of barotraumas, a less-developedmastoid routinely found in chronic ear patients was more protective of injury.Thus, chronic ear patients are at less risk for barotrauma.

Summary

There appears to be a correlation between ET dysfunction and thesubsequent development of a cholesteatoma. Although both a healthy mas-toid and adequate ET function seem to be instrumental in providing aera-tion to the middle ear, the physiologic state of the ET plays a larger role.Postoperative conductive hearing loss and the incidence of reoccurrenceof cholesteatoma can be reduced significantly with improved ET function.

Several etiologies exist that can cause ETD, which can lead to a widespectrum of middle ear disease from a mild retraction to an invasive choles-teatoma. However, there are little data to explain why some retractions arestable, while others progress to cholesteatoma.

Although some findings have suggested that the degree of aeration of themastoid is an important physical finding, the key to interrupting this evolu-tion appears to be treatment of the underlying cause of the negative pres-sure, in this case a poorly functioning ET. Medical intervention has hadsuccess in the preventing or treating ETD, whereas surgical treatment has

shown to be more effective in addressing the sequelae rather than the causeitself.

Acknowledgment

I would like to thank Chris Danner, MD, for inviting me into this projectand his perseverance to teach residents. I would also like to thank my wife,Shannon, and my three children for their continual support.

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Extratemporal (extracranial) complications

Subperiosteal abscess

Subperiosteal abscess is the most common extratemporal complication

that occurs with COM. This abscess occurs over the mastoid cortex when

the infectious process within the mastoid air cells extends into the subperios-

teal space. This extension most commonly occurs as a result of erosion of

the cortex secondary to acute or coalescent mastoiditis, but can also occur

as a result of vascular extension secondary to phlebitis of the mastoid veins

[3]. Subperiosteal abscesses are seen more commonly in young children with

AOM, but are also found in chronic otitis with and without cholesteatoma.

Cholesteatoma can block the aditus ad antrum, preventing communicationof the infected contents of the mastoid with the middle ear space and the eu-

stachian tube. This obstruction increases the possibility of infectious decom-

pression through the mastoid cortex, presenting clinically as a subperiosteal

abscess or Bezold’s abscess.

Diagnosis

Often, the diagnosis of a subperiosteal abscess is made on clinical

grounds. Commonly, the patient will present with systemic symptoms, in-

cluding fever and malaise, along with local signs, including a protruding au-ricle that is laterally and inferiorly displaced, and the presence of a fluctuant,

erythematous, tender area behind the ear. When the diagnosis is not certain

on clinical evaluation, a contrasted CT scan can demonstrate abscess and

possibly the cortical defect in the mastoid (Fig. 1) [4]. A case can be made

for a contrasted CT scan of the temporal bone in all patients presenting

with these symptoms, to aid in therapeutic planning and to rule out other

possible complications. Mastoiditis without abscess, lymphadenopathy,

superficial abscess, and an infected sebaceous cyst are other possibilities

that must be excluded.

Fig. 1. An axial CT scan (A) of a 5-month-old child, demonstrating opacification of the left ear

and mastoid with coalescence, and a coronal CT scan (B) of the left temporal bone in the same

patient, demonstrating a subperiosteal abscess.

1238 SMITH & DANNER



Management

The management of a postauricular subperiosteal abscess from otitis me-

dia without cholesteatoma is debatable. Conventional teaching and currenttexts advocate draining the abscess, in conjunction with a cortical mastoid-

ectomy [3,4]. In recent years, other, less invasive treatment options have

emerged. Patients have been treated with simple incision and drainage of

the abscess in conjunction with intravenous (IV) antibiotics and a myringot-

omy without sequelae [5]. One investigator advocates the use of IV antibi-

otics, myringotomy, and needle aspiration of the abscess without formal

drainage. In his experience, 14 of the 17 subjects treated in this manner re-

solved their abscesses without the need for further intervention, and were

discharged home significantly sooner than those subjects who were managedwith a mastoidectomy. The three subjects who failed needle aspiration went

on to require cortical mastoidectomy [6]. An argument for conservative

treatment modalities for subperiosteal abscesses from AOM is reasonable,

because the process likely will be self-limited; however, there is not yet a con-

sensus advocating these less aggressive approaches. The approach to a sub-

periosteal abscess resulting from chronic otitis in the presence of

a cholesteatoma is not as controversial. In this setting, the cholesteatoma

warrants surgical intervention, and therefore more conservative alternatives

are not reasonable. The skin incision for the mastoidectomy should be mod-ified to incorporate the abscess cavity for adequate drainage. Once the ab-

scess is drained, a mastoidectomy is performed and the cholesteatoma

matrix is removed in the standard manner.

Bezold’s abscess

A Bezold’s abscess is a cervical abscess that develops from pathology sim-

ilar to the subperiosteal abscess. In the presence of coalescent mastoiditis, if

the mastoid cortex is violated at its tip, as opposed to its lateral cortex, anabscess will develop in the neck, deep to the sternocleidomastoid. This ab-

scess will present as a tender, deep, poorly defined mass in level two of

the neck. Because the abscess develops from air cells at the tip of the mas-

toid, it is found in older children and adults, where pneumatization of the

mastoid has extended to the tip. Most of these abscesses result from direct

extension through the cortex, but transmission through an intact cortex by

way of mastoid vein phlebitis is known to occur [1]. Although Bezold’s ab-

scess is more commonly a complication of AOM with mastoiditis in chil-

dren, it is a known complication of COM with cholesteatoma [7].

Diagnosis

A contrasted CT scan of the neck and mastoid is recommended to make

the diagnosis of a Bezold’s abscess [8]. The presentation of an enlarged, ten-

der, deep neck mass must be differentiated from inflammatory cervical

lymphadenopathy, which is difficult on clinical grounds alone. CT scans

1239COMPLICATIONS OF CHRONIC OTITIS MEDIA AND CHOLESTEATOMA



of Bezold’s abscesses show a rim-enhancing abscess with surrounding in-

flammation, may demonstrate the bony dehiscence in the tip of the mastoid,

and can help in operative planning.

Management

The commonly recommended conventional approach for management of

a Bezold’s abscess is described as an open incision and drainage of the cer-

vical abscess through a transcervical approach, combined with a cortical

mastoidectomy to address the mastoiditis [3]. Abscess drainage combined

with myringotomy and IV antibiotics has not been advocated for Bezold’s

abscesses in the literature, as it has been for the subperiosteal abscess. How-

ever, time and investigation may prove that this complication can be man-aged by less aggressive surgical options.

Intratemporal (extracranial) complications

Labyrinthine fistulae

Labyrinthine fistulae continue to be among the most common complica-

tions of chronic otitis with associated cholesteatoma, and have been re-

ported in approximately 7% of cases [9–11]. Few circumstances are more

unsettling to an otologic surgeon than the presence of an open labyrinthfound at the time of cholesteatoma surgery. The risk of significant sensori-

neural hearing loss as a result of surgical manipulation makes the open lab-

yrinth and its management a highly controversial topic.

As a result of its location near the antrum, the horizontal semicircular ca-

nal is the most commonly involved portion of the labyrinth, and accounts

for approximately 90% of these fistulae (Fig. 2). Although the horizontal

canal is usually involved, fistulae have been described in both the superior

canal and posterior canal, and in the cochlea itself. Cochlear fistulae are as-

sociated with a much higher incidence of hearing loss encountered duringsurgical manipulation than are labyrinthine fistulae [10].

Fig. 2. Intraoperative picture of a horizontal canal fistula.

1240 SMITH & DANNER



Erosion of the bone of the otic capsule can occur through two distinct

processes. In the presence of a cholesteatoma, activated mediators from

the matrix, or pressure from the cholesteatoma itself, can lead to osteolysisand uncovering of the labyrinth. However, labyrinthine fistulae can occur

from resorption of the otic capsule due to inflammatory mediators in the ab-

sence of cholesteatoma, which typically occurs in COM with granulation [4].

One reason for the confusion and controversy in discussing these fistulae

is the lack of an accepted staging system. Multiple staging systems have been

proposed [9,12]. The system introduced by Dornhoffer and Milewski [9] is

the classification used in the authors’ department, and is used in this article

to discuss fistulae and their management (Fig. 3). This system stages fistulae

with respect to the involvement of the underlying labyrinth. Fistulae withbony erosion and intact endosteum are classified as type I fistulae. If the

endosteum is violated, but the perilymphatic space is preserved, the fistula

is staged as type IIa. When the perilymph is violated by disease or inadver-

tently suctioned, the fistula is labeled as type IIb. Type III fistula indicates

that the membranous labyrinth and endolymph have been disrupted by dis-

ease or surgical intervention [9].

Diagnosis

Patients who have significant erosion of the labyrinth classically presentwith subjective vertigo and a positive fistula test on examination. Unfortu-

nately, this classic picture is not sensitive in the preoperative identification of

a fistula. Periodic vertigo or significant disequilibrium is found in 62% to

64% of patients who have fistulae preoperatively. The fistula test is positive

in 32% to 50% of patients who are found ultimately to have fistulae during

surgical exploration. Although sensorineural hearing loss is found in most

of these patients (68%), it is not a sensitive indicator of fistula [9,10].

Although the presence of sensorineural hearing loss, vertigo, or a positive

fistula test in a patient who has a cholesteatoma should raise the suspicionfor a fistula, their absence does not guarantee an intact bony labyrinth. It is

for this reason that a prudent surgical approach is to assume the presence of

a fistula in every cholesteatoma case, to prevent unexpected complications.

Although universal imaging of all patients who have cholesteatoma has

not been the standard, review of the literature demonstrates that the use

Fig. 3. A fistula staging system. (Adapted from Dornhoffer JL, Milewski C. Management of the

open labyrinth. Otolaryngol Head Neck Surg 1995;112(3):410–4.)




of preoperative CT imaging is increasing in this country [10]. Because of the

inability to accurately diagnose fistulae preoperatively on clinical grounds,

the increase in imaging is likely an attempt to increase the detection of anexposed labyrinth, facial nerve, or dura, to aid in surgical planning

(Fig. 4). Unfortunately, the ability to detect fistulae accurately on preoper-

ative CT has been reported as 57% to 60% [10,13]. In one of these reports,

when subjects had fistulae detected intraoperatively, their preoperative CT

scan was reviewed retrospectively to look for radiologic evidence. Despite

the knowledge of a confirmed fistula, CT evidence could only be found in

60% of these cases [13]. In the labyrinth, where millimeters matter, CT scans

of the temporal bone with 1 mm cuts can miss a thin layer of cortical bone,

which falsely increases the concern for fistula. Also, a small fistula easily canbe missed between cuts on the CT images. Although the debate regarding

the need for preoperative imaging for cholesteatoma cases will continue,

in current reports CT scans are no more sensitive than history and physical

examination in detecting labyrinthine fistulae. The definitive diagnosis for

a fistula is only made intraoperatively, which reaffirms the need to approach

all cholesteatoma cases with caution.

Management

A tympanomastoidectomy is required for the treatment of the cholestea-toma, but the most appropriate management of the fistula remains an ongo-

ing debate. Some investigators believe that the most appropriate approach

to the fistula is to perform a canal wall down mastoidectomy, remove the

bulk of the cholesteatoma, and leave the fistula covered with the matrix ex-

teriorizing it into the cavity [14,15]. Advocates of this approach argue that

the complete removal of the matrix increases the risk of sensorineural hear-

ing loss, and that by removing the sac itself, the pressure from the cholestea-

toma is relieved and further bony erosion or infectious complications are

unlikely. Other investigators advocate the complete removal of the choles-teatoma over the fistula, with repair of the bony defect in all circumstances

Fig. 4. A coronal CT scan of a postoperative right ear, revealing a horizontal canal fistula.

1242 SMITH & DANNER



[12,16]. These investigators feel that by removing the matrix in its entirety,

the potential risk of continued bony erosion and infectious complications

such as labyrinthitis can be prevented. Furthermore, they argue the riskof significant sensorineural hearing loss with complete removal is minimal,

and the long-term risk of sensorineural hearing loss is greater if the matrix

is left intact. Complete removal can be performed in a single setting, or in

a staged manner, with a second-look procedure. A recent review of the liter-

ature found that hearing preservation for patients who underwent complete

removal was equivalent to patients where the matrix was left over the fistula

[10]. It is impossible to know if the extent of disease was similar in these two

groups because an accepted staging system was not used for comparison.

The size, extent, and location of the fistula should be considered when de-termining whether complete cholesteatoma removal should be attempted.

Multiple studies have demonstrated that larger fistulae have worse hearing

results postoperatively [17,18]. Dornhoffer evaluated hearing results after

single-stage, complete cholesteatoma removal, and compared results, based

on extent of disease [9]. No hearing loss resulted from the removal of type

IIa fistulae where the endosteum was violated but the perilymphatic space

was not disrupted or suctioned significantly. For type IIb and type III fistu-

lae, where the perilymph or endolymph was involved or violated signifi-

cantly, roughly one half of the subjects experienced significant hearingloss postoperatively (8 of 17). Subjects who had fistulae with significant

labyrinthine involvement (types IIb and III) who received intraoperative

steroids experienced stable or improved hearing 90% of the time (9 of 10);

and the one subject who had hearing loss sustained only moderate high fre-

quency hearing loss. The anatomic location of the fistula also impacts hearing

results significantly. A 35% rate of profound deafness has been reported with

fistulae that involve the promontory, compared with a 3% rate of deafness

with semicircular canal fistulae [10].

The debate over the most appropriate management of labyrinthine fistu-lae continues, but a review of the literature can help with some recommen-

dations. Small fistulae involving the labyrinth can be removed safely in

a primary setting. The use of corticosteroids at the time of cholesteatoma

removal from fistulae may have a protective effect on hearing. Large fistulae

involving the labyrinth can be treated by carefully removing the cholestea-

toma matrix and covering the defect with bone pate ´ and fascia (Fig. 5).

However, fistulae involving the cochlea should be approached with more

caution because of the greater risk for iatrogenic sensorineural hearing

loss, and exteriorization of matrix remaining on the fistula may be the bestoption.

Coalescent mastoiditis

Mastoiditis is a spectrum of disease that must be defined appropriately to

be treated adequately. Mastoiditis, defined as mucosal thickening or




a mastoid effusion, is common in the face of an acute or chronic otitis, and is

appreciated routinely on a CT scan performed in this setting. This entity isof little clinical significance. Clinical mastoiditis presenting with postauricu-

lar erythema, tenderness, and edema, with an inferiorly and posteriorly

displaced auricle, is a distinctly different scenario. In this setting, further

workup is indicated to determine the most appropriate treatment. Fig. 6

demonstrates the difference between coalescent mastoiditis and a mastoid

effusion, as seen on CT scan.

Diagnosis

In the presence of clinical mastoiditis, a CT scan should be performed toevaluate for an unappreciated subperiosteal abscess or coalescent mastoid-

itis (see Fig. 1). Coalescent mastoiditis is an acute, infectious process of

the mastoid bone, with characteristic loss of trabecular bone. It is a rare

complication, and is seen usually in young children with AOM. Classically,

coalescent mastoiditis is described as occurring in a well pneumatized mas-

toid with a significant, incompletely treated AOM, whereas chronic otitis

and cholesteatoma occur in a sclerotic temporal bone. However, as many

as 25% of cases of coalescent mastoiditis have been reported to occur in

a sclerotic temporal bone with COM and cholesteatoma [19].

Fig. 5. The repair of a labyrinthine fistula after removal of cholesteatoma.

Fig. 6. Coalescent mastoiditis in a sclerotic temporal bone with COM (A), as compared with

a mastoid effusion in a well-aerated temporal bone with AOM ( B).

1244 SMITH & DANNER



Management

Coalescent mastoiditis is a serious medical problem that requires aggres-

sive treatment, either surgical or medical. Classically, treatment included IVantibiotics and mastoidectomy with removal of necrotic, devitalized bone.

In recent years, the use of myringotomy and IV antibiotics has been advo-

cated as an alternative [3,20]. This medical management requires a CT scan

to confirm resolution of the infection and aeration of the mastoid. The pres-

ence of a cholesteatoma is a surgical indication, and therefore coalescent

mastoiditis in this setting is a surgical disease. In this case, a tympanomastoi-

dectomy is performed to remove devitalized bone and the cholesteatoma,

and to re-establish aeration to the mastoid and middle ear.

Petrous apicitis

The petrous apex comprises the anterior, medial portion of the temporal

bone, and has been reported to be pneumatized in 30% of individuals [3].

These air cells, when present, are in continuity with the middle ear and mas-

toid through well-described cell tracts around the labyrinth, allowing for in-

fection involving the mastoid and middle ear cleft to extend into the petrous

apex. Petrous apicitis is a spectrum of disease much like mastoiditis, and can

involve anything from an asymptomatic effusion to coalescence and abscess

formation. Infection of the petrous apex is a dangerous entity because of itsproximity to the middle and posterior cranial fossae and their contents.

Diagnosis

The classic symptomatology associated with petrous apicitis is a triad of

deep retro-orbital pain, aural discharge, and sixth nerve palsy, also known

as Gradenigo’s syndrome. Although these symptoms can be associated

with apicitis, they are by no means pathognomonic of this condition.

Retro-orbital pain and abducens palsy have been reported to occur in

50% and 25% of reported cases, respectively [21]. Petrous apicitis becomesevident only after failure to control chronic suppurative otomastoiditis with

prolonged medical and surgical management. When apicitis is suspected,

a CT scan should be performed to make the diagnosis and to evaluate sur-

rounding anatomy. A CT scan will also aid in the diagnosis of intracranial

complications that often accompany this condition. Some believe that once

the diagnosis is made with clinical evaluation and CT, an MRI of the brain

or a lumbar puncture should be performed to evaluate for intracranial com-

plications [4].

Management

Early in the twentieth century, before the widespread use of antibiotics,

surgical intervention for petrous apex abscesses and petrous apicitis was rel-

atively common. However, as the use of antibiotics has increased, the prev-

alence of apicitis has decreased significantly. The petrous apex is an area

that is not easily approached surgically because of its relationship with




the otic capsule and carotid artery. Because of the difficult surgical approach

and the response rate to antibiotics, IV antibiotics are often the first-line

treatment of petrous apicitis. IV antibiotics require a long duration of treat-ment. Serial C-reactive protein levels and erythrocyte sedimentation rates

have been used to monitor for response of bony infections to medical man-

agement elsewhere in the body [22], and are a reasonable option for follow-

ing a patient who has petrous apicitis to assess for response. In the presence

of abscess, necrotic bone, or persistent infection despite medical therapy,

surgical drainage is required. Air cell tracts extend to the apex below, above,

and anterior to the labyrinth. In a hearing ear, these three air cell tracts al-

low several possibilities for surgical approach including: infracochlear,

infralabyrinthine, retrolabyrinthine, subarcuate, and even middle fossa [4].In a nonhearing ear, the translabyrinthine or transcochlear approaches

are reasonable and give wide exposure to the affected area. One disadvan-

tage of these two approaches is that they could potentially expose the cere-

brospinal fluid (CSF) to the infectious process.

Facial paralysis

Otogenic causes of facial nerve paralysis include AOM, COM without

cholesteatoma, and cholesteatoma. The first usually occurs with a dehiscent

fallopian canal within its tympanic segment, allowing direct contact of in-

flammatory mediators with the facial nerve itself. COM with or without

cholesteatoma can result in facial paralysis through involvement of a dehis-

cent nerve, or through bony erosion. Facial paralysis secondary to AOM of-

ten presents in children with incomplete paresis that comes on abruptly and

is usually short-lived with appropriate treatment. On the other hand, paral-

ysis secondary to COM or cholesteatoma often presents with slowly pro-

gressive facial paralysis and has a worse prognosis [3].

Diagnosis

The diagnosis of otogenic facial paralysis is made on clinical grounds.

Facial paresis or paralysis in the presence of AOM, COM, or cholesteatoma

is not a difficult diagnosis to make by examination alone. The role of diag-

nostic CT imaging is questionable. Although a CT scan is not required, it

can be useful in therapeutic planning and patient counseling. When choles-

teatoma involves the fallopian canal, it may also erode structures such as the

labyrinth or tegmen. Furthermore, the extent of bony erosion of the fallo-

pian canal and degree of involvement is better appreciated on CT [23].

Management

Although facial paralysis secondary to AOM usually is treated with ap-

propriate antibiotics and myringotomy, the treatment of paralysis with

COM with or without cholesteatoma requires surgical intervention. When

facial paralysis is associated with cholesteatoma, a mastoidectomy is

1246 SMITH & DANNER



performed to remove the cholesteatoma or granulation tissue that is contact-

ing the facial nerve. The nerve is approached on both sides of the involve-

ment, and a diamond burr is used to expose the epineurium on either sideof the diseased segment. Once the proximal and distal segments of the fallo-

pian canal have been opened, blunt dissection is used to remove the disease

from the epineurium. The nerve sheath does not have to be incised unless the

cholesteatoma has invaded the nerve itself [4]. Although cholesteatoma can

involve the facial nerve at any point through its intratemporal course, the

tympanic segment and second genu are involved most commonly [24].

Intracranial complications

Meningitis

Meningitis is the most common intracranial complication of acute and

COM; conversely, AOM is the most common secondary cause of meningitis

[25,26]. In a recent series of COM complications, meningitis occurred in ap-

proximately 0.1% of subjects [1,4]. Although this remains a significant com-

plication, the mortality rate from otitic meningitis has declined significantly,

from 35% in the preantibiotic era to 5% in the postantibiotic era [27]. Men-

ingitis can arise from three distinct otogenic routes: hematogenous seeding

of the meninges and subarachnoid space; spread from the middle ear or

mastoid through preformed channels (Hyrtl’s fissures); or through bony ero-

sion and direct extension. Of these three possibilities, otogenic meningitis

most commonly results from hematogenous seeding [3,4].

Diagnosis

The prompt diagnosis of meningitis relies on the recognition of warning

signs by an astute clinician. Signs that should increase the suspicion of an

intracranial complication include persistent or intermittent fever; nauseaand vomiting; irritability; lethargy; or persistent headache. Ominous signs

virtually diagnostic of an intracranial process include visual changes; new

onset seizures; nuchal rigidity; ataxia; or decreased mental status [3]. If

any of these suspicious or ominous signs occur, immediate treatment and

further workup are critical. Broad-spectrum antibiotics, such as third-gener-

ation cephalosporins, should be administered while diagnostic tests are

ordered and arranged. A contrasted CT scan or MRI will show character-

istic meningeal enhancement and rule out additional intracranial complica-

tions known to occur in up to 50% of these cases [28]. In the absence of a significant mass effect on imaging, a lumbar puncture should be performed

to confirm the diagnosis and to allow for culture and sensitivity.

Management

The presence of symptoms suspicious for otogenic intracranial complica-

tions warrants the use of broad-spectrum IV antibiotic therapy while the




concomitant intracranial complications, or evidence of increased intracra-

nial pressure.

Management

Immediate initiation of broad-spectrum antibiotics that cover gram pos-

itives, gram negatives, and anaerobes is necessary because of the severity of

this infection and its polymicrobial nature. As in otitic meningitis, these an-

tibiotics should be initiated while further workup is being performed. Once

the diagnosis of a brain abscess is made, surgical intervention is required.

Drainage of the abscess requires neurosurgical intervention, but the patient

must be stabilized from a neurologic standpoint. IV steroids are often given

to decrease brain edema, and anticonvulsants are given to prophylax againstseizures. When the patient is stable, neurosurgical drainage is performed,

either through an open craniotomy with drainage or excision, or by stereo-

tactic aspiration through a burr hole. This procedure not only drains the

abscess, but provides a culture, enabling antibiotic therapy to be tailored.

Drainage of the brain abscess is paramount, and should be performed

within 24 hours of presentation, if the patient is stable [3].

However, the most appropriate way to treat the otologic component is

somewhat controversial. In the setting of AOM, myringotomy with evacu-

ation of the purulent effusion is sufficient. In the presence of COM withor without cholesteatoma, a mastoidectomy is required to eradicate the

source of infection. The most appropriate time to perform the mastoidec-

tomy is controversial. It has been conventional teaching that a mastoidec-

tomy is performed in a delayed manner after the patient recovers from

the abscess and neurosurgical drainage. Current recommendations, how-

ever, are to perform a mastoidectomy at the time of abscess drainage to

Fig. 7. An axial T1-weighted MRI (A) and a coronal T2-weighted MRI (B) demonstrating

a large right cerebellar brain abscess with resulting hydrocephalus.




remove the infectious focus, assuming the patient is stable enough to toler-

ate this additional surgery. However, specific parameters dictating staged

versus simultaneous surgery have not yet been reported or established.One recent report has advocated primary mastoidectomy with needle aspi-

ration of the abscess through the dura exposed in the mastoidectomy cavity

[30]. Regardless of the type of surgical intervention, when it has been com-

pleted, IV antibiotics should be continued for several weeks and serial CT

scans with contrast followed to assure resolution of the abscess [3].

Lateral sinus thrombosis

Sigmoid sinus or lateral sinus thrombosis is a well-known complication of

otitis media that compromises 17% to 19% of intracranial complications

[26,31]. The proximity of the middle ear and mastoid air cells to the dural

venous sinuses predisposes them to thrombosis and thrombophlebitis sec-

ondary to infection and inflammation in the middle ear and mastoid. In-

volvement of the sigmoid or lateral sinus can result from bony erosion

secondary to COM and cholesteatoma, with direct extension of the infec-

tious process to the perisinus space, or from the seeding of the space from

thrombophlebitis of mastoid emissary veins. Once the sinuses have become

involved, and an intramural thrombus develops, any number of serious

complications can result. Otitic hydrocephalus is known to complicate a sig-

nificant number of these cases. The infected clot can propagate proximally

to involve the confluence of sinuses (torcular herophili) and sagittal sinus,

causing life-threatening hydrocephalus, or propagate distally to involve

the internal jugular vein [3]. Involvement of the internal jugular vein in-

creases the risk of septic pulmonary emboli.

Diagnosis

The classic presentation of sigmoid or lateral sinus thrombosis is the pres-ence of high spiking fevers in a ‘‘picket fence’’ pattern, often seen with head-

aches and general malaise [2]. Like many of these complications, a high

degree of suspicion is required because the spiking fevers may be blunted by

concurrent antibiotic use. With the presence of high spiking fevers, or concern

for increased intracranial pressure, a contrasted CT scan should be performed

to screen for thrombophlebitis. The sinus wall will enhance brightly with con-

trast and produce the characteristic delta sign associated with sinus thrombo-

sis (Fig. 8). With the presence of significant sinus thrombosis, an MRI and

magnetic resonance venogram (MRV) are warranted, because they can beused serially to evaluate for clot propagation or resolution.

Management

Dural venous thrombosis in the presence of chronic otomastoiditis with

or without cholesteatoma is a surgical disease. At a minimum, a mastoidec-

tomy with removal of chronic infection, granulation, and cholesteatoma is

1250 SMITH & DANNER



cholesteatoma or from coalescent mastoiditis. The signs and symptoms do

not differ significantly from those found in COM. Occasionally, dural irrita-

tion can result in increased otalgia or headaches that serve as a concerningsign in the background of COM. Because this complication can be subtle in

presentation, it is often found incidentally at the time of cholesteatoma sur-

gery or CT scan for other purposes.

Diagnosis

Unlike other intracranial complications, there are no sensitive or specific

symptoms suggestive of this disease process. A high degree of clinical suspi-

cion is required to diagnose an epidural abscess preoperatively. The presence

of increased otalgia or headache should raise the suspicion for an intracra-nial complication, and warrants imaging. A contrasted CT scan or MRI is

sufficient to diagnose this abscess. Even with a careful evaluation, this diag-

nosis is often made at the time of surgery.

Management

When an epidural abscess is appreciated intraoperatively or on CT scan,

surgical drainage is necessary. A mastoidectomy is performed to treat the

underlying pathology, paying careful attention to thin the bony tegmen

and bone overlying the posterior fossa dura as much as possible, so epiduralpus or granulation can be appreciated. The bone overlying the dura is re-

moved to evacuate the pus and granulation until normal dura is encountered

[4]. Postoperative antibiotics are continued at least until the symptoms of

the abscess and otitis have resolved.

Otitic hydrocephalus

Otitic hydrocephalus is described as signs and symptoms indicative of in-

creased intracranial pressure with normal CSF studies on lumbar puncture,which can present as a complication of AOM, COM, or otologic surgery.

‘‘Otitic hydrocephalus’’ is somewhat of a misnomer, and its pathophysiol-

ogy is not understood completely. It is a misnomer because this condition

can be found in the absence of otitis, and patients do not have dilated ven-

tricles indicative of true hydrocephalus. Symonds [34], who coined the term

otitic hydrocephalus, felt that this condition developed from infection of the

lateral (transverse) sinus, with extension of thrombophlebitis into the con-

fluence of sinuses to involve the superior sagittal sinus. Inflammation or in-

fection of the superior sagittal sinus prevents CSF absorption through thearachnoid villi, resulting in increased intracranial pressure. This infectious

thrombophlebitis usually occurs as a result of otologic infection, but multi-

ple cases have been described in the absence of otitis or otologic surgery

[34,35]. Furthermore, although lateral sinus thrombosis is found usually

in the presence of otitic hydrocephalus, cases have been reported without

thrombosis of the dural sinuses [36].

1252 SMITH & DANNER



Diagnosis

The diagnosis of otitic hydrocephalus is one of exclusion, and requires

a high degree of suspicion to recognize the suggestive symptoms. The symp-toms found in these patients are a result of increased intracranial pressure

and include diffuse headaches, nausea, vomiting, visual changes, and leth-

argy. The presence of these symptoms requires a thorough examination

and imaging. A dilated fundoscopic examination should be conducted to

evaluate for papilledema as evidence of increased intracranial pressure.

An MRI and MRV should be performed to evaluate for ventricular enlarge-

ment, or coexisting intracranial complications, such as significant sinus

thrombosis with obstruction. Increased intracranial pressure with clinical

symptoms and papilledema in the absence of ventricular dilation or menin-gitis is enough to make this diagnosis. MRV will confirm the presence and

extent of dural sinus thrombosis, but is not required to make a diagnosis of

otitic hydrocephalus.

Management

The goal in the treatment of otitic hydrocephalus is to treat any underly-

ing infectious otitis, decrease intracranial pressure, and prevent the poten-

tially devastating complication of optic nerve atrophy. With COM (with

or without cholesteatoma) in the presence of dural sinus thrombosis, a mas-toidectomy should be performed to remove the infectious process and the

cholesteatoma, and to address the dural venous sinus. In the absence of

a surgical indication such as cholesteatoma or tumor, the treatment is med-

ical in nature, and should include acetazolamide, fluid restriction, and cor-

ticosteroids to decrease intracranial pressure and cerebral edema. Systemic

anticoagulation is not required unless an MRV shows sinus thrombophlebi-

tis involves the sagittal sinus. In these cases, the risk of neurologic sequelae

and death are significant enough to warrant anticoagulation [35]. If aggres-

sive medical management does not normalize the intracranial pressure, lum-bar drainage of CSF can be performed serially or by a lumbar drain. If

prolonged drainage is required because of recalcitrant papilledema, a shunt

may be required [4,35].

Summary

The incidence of extracranial and intracranial complications of COM and

cholesteatoma has decreased since the proliferation of antibiotics early in

the twentieth century. However, these complications continue to occur,and can be lethal if they are not identified and treated properly. Therapy

for the complications associated with COM, unlike those of AOM, usually

includes surgical intervention, in addition to medical therapy. As medical

(antibiotic) therapy continues to improve, and new imaging techniques are

introduced, less invasive treatment modalities may be shown to be as effec-

tive as the classic, time-tested, surgical options.




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2006, Vol.39, Issues 6, Cholesteatoma

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