•Pathogenesis•Classification•Complications

Vishav

Yadav 1

Keratin-producing squamous epithelium in the middle ear, mastoid or petrous apex

Johannes Müller (1838):German Physiologist coined cholesteatoma i.e. layered paerly tumpour of fat

Schuknecht : keratoma

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Abramson3D epithelial and connective tissue

structure usually in form of sac , mostly confined to middle ear spaces , with tendency of independent growth on cost of underlying bone , with tendency of recurrence despite complete removal .

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Histologically made up of :Cystic content – anucleate keratin squamesMatrix – keratinizing squamous epitheliumPerimatrix – granulation tissue in contact

with bone (produces proteolytic enzymes)

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Congenital Acquired

Primary acquired (retraction pocket)Secondary acquiredTertiary acquired

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The incidence of CC - 1% to 5% of all cholesteatomas

Congenital cholesteatoma of the middle ear was first described by Howard House

Location (AS,PS , petrous pyramid, mastoid and middle ear cleft , CP Angle)

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Levenson criteria (Modified Derlacki & Clemis)

White mass medial to normal TM Normal pars flaccida and tensa No history of otorrhea or perforations No prior otologic procedures Prior bouts of otitis media not grounds for

exclusion

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The accepted cause of CC remains controversialTeed’s epithelial cell rest theoryFriedberg’s implantation theoryRuedi’s invagination theoryAimi’s epithelial migration theoryMichael’s epidermoid formation theory

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Most commonly accepted and quoted theory Teed An epidermal structure : these rests as

ectodermal implants in the fusion plates between the first and second branchial arches that appear around 10 weeks

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

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Friedberg observed viable squamous cells in the amniotic fluid present in the middle ear of neonates.

Could they be a cause for congenital cholesteatoma?

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Ruedi Inflammatory injury to an intact

tympanic membrane Microperforations in the basal layer that

lead to invasion of the squamous epithelium by proliferating epithelial cones through a macroscopically intact but microscopically injured tympanic membrane.

Epithelial cones fuse and expand forming a middle ear cholesteatoma

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First proposed by Aimi He suggested that ectoderm of external

canal managed to grow / migrate in to the middle ear cavity somehow overcoming the restraining influence of the annulus.

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First proposed by Michael He observed nests of squamous

epithelium in the lateral wall of tympanic cavity below the level of pars flaccida

These nests normally involute Failure of this involution process can

possibly cause cholesteatoma

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(1) aid the clinician in preoperative planning of treatment

(2) Indicate prognosis(3) facilitate exchange of information

between different clinicians(4) evaluate the results of treatment.

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Petrous Pyramid Mastoid Tympanic

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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

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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

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Clinical Stage

Recurrence Rates

TYPE 1 15 0

TYPE 2 59 34

TYPE 3 26 55

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Invagination theory of Wittmaack Eustachian tube dysfunction Poor aeration of the epitympanic space Retraction of the pars flaccida Normal migratory pattern altered Accumulation of keratin, enlargement of

sac

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Basal cell hyperplasia (Ruedi theory) Inflammatory injury to an intact

tympanic membrane Microperforations in the basal layer

that lead to invasion of the squamous epithelium by proliferating epithelial cones through a macroscopically intact but microscopically injured tympanic membrane.

Epithelial cones fuse and expand forming a middle ear cholesteatoma

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Squmaous metaplasia (Sade theory) transformation of cuboidal epithelium

to squamous epithelium from chronic infection

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Presence of preexisting perforation in pars tensa

Often associated with posterosuperior marginal perforation or large central perforation

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Immigration & Invasion: Habermann medial migration along permanent perforation

of TM Implantation & invasion –foreign body,

blast injuryMetaplasia – transformation of cuboidal

epithelium to squamous epithelium from chronic infection

Papillary ingrowth – intact pars flaccida, inflammation in Prussack’s space, break in the basal membrane, cords of epithelium migrate inward

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iatrogenic – surgery, foreign body, blast injury

TympanoplastyOssiculoplastyStapedotomyTypmpanostomy tubes

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Epitympanic cholesteatoma patterns of spread from Prussack’s spacePosterior

epitympanumPosterior

mesotympanumAnterior

epitympanum

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Medially :neck of malleus laterally :Shrapnell’s membrane Sup : lat malleal fold Anteriorly anterior malleolar fold & anterior

ligament

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Posterior epitympanum – through superior incudal space to mastoid antrum

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Posterior mesotympanum – inferiorly through posterior pouch of Von Troeltsch to stapes, round window, sinus tympani and facial recess

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Anterior epitympanum – anterior to head of malleus, may gain access to supratubal recess

To ant mesotympanum via anterior pouch of von Troeltsch

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*Medially – Medial Wall Of Middle Ear with Oval & Round Window Niches

*Laterally – Pyramid & Facial Nv

*Superiorly– Ponticulus *Inferiorly – Subiculum

-can extend as far as 9mm into the mastoid

-probably the most inaccessible site in middle ear & mastoid

Molecular modelsPreneoplastic transformation eventsDefective wound-healing processCollision between host inflammatory response,

normal middle ear epithelium, and bacterial infection

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Hyperproliferative keratinocytes Increased proliferationDecreased terminal differentiation

Expression of epithelial markers in the basal and suprabasal layers (cytokeratins –10,13,16, filaggrin, involucrin); confirm they arise from pars flaccida and overlying EAC skin

High expression of epidermal growth factor receptor, transforming growth factor

Upregulation of p5336

Chronic inflammatory response around matrix (granulation/perimatrix)

Infiltration of activated T-cells and macrophages

Production of cytokines (TGF,TNF,IL-1,IL-2,FGF,PDGF)

Causes increased migration and invasion of cholesteatoma epithelium and fibroblasts

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Bacterial related antigens producing host inflammatory response may stimulate the migrating epithelium’s uncoordinated proliferation

Granulation induces invasion of keratinocytes

Granulation – contains proteases, acid phosphatases, bone resorption proteins, osteoclast-activating factors, prostaglandins

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Intracranial

Extradural abscess Subdural abscess

(empyema) Sigmoid sinus

thrombophlebitis Meningitis Brain abscess Otitic

hydrocephalus

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Intratemporal Facial paralysis Labrynthine infections Labyrinthine fistula Petrositis Mastoiditis Other rare

complications: Abscess:

Bezold’s abscess Luc’s abscess Citeli’s abscess

Distant pyaemic abscess

Extratemporal complications: Subperiosteal abscess Migrating

thrombophlebitis of IJV

Migrating thrombophlebitis of IJV

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Possible routes of spread:

Extension through bone Infected clot within small

veins Normal anatomical

pathways Non anatomical bony

defects Surgical defects Into brain tissue along periarteriolar spaces of

Robin Virchow

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General principles: The complications are multiple in about 1/3rd of

the cases The symptoms of intracranial spread of infection

are those of infection and those of brain tissue compression

Otalgia is never a symptom of uncomplicated cholesteatoma

Investigation and treatment must run concurrently The principles of treatment :

Systemic antibiotic therapyLocal neurosurgical attention to the

complication(s)Treatment of the ear lesion

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Intracranial complication:G-ve organismsStaphylococci-

beta lactamase producing

Obligate anaerobes Bacteroides fragilis

Extratemporal and temporal complications:Pseudomonas

Proteus

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High grade fever Headache with vomiting Copious ear discharge Gait changes Visual alterations Seizure activity Neck stiffness

Unusual persistent pain

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Etiology :Bone erosion

Sigmoid perisinus abscess

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Clinical features: Site and size Duration and rate Incidental finding

Headache with malaise Intermittent relief from pain during episodes of

aural discharge

Diagnosis : Operative findings CT scan

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Management : Surgical exploration

????Don’t remove the granulation tissue attached to the dura

Appropriate antibiotics

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Spread of infection Loculated infection Thrombophlebitis Obliteration of subdural

space

Commonly associated organisms:

Strp. milleri H. influenzae

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Clinical features: Severe headache Fever Drowsiness Focal neurological deficit Seizure Paralysis

The course is much more rapid than brain abscess

Papilledema is uncommon, as are the cranial nerve palsy

Seizures and FND differentiates from meningitis

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Diagnosis CT scan MRI LP : helpful but risky

↑pressure Normal sugar content Cultures are sterile Marked pleocytosis

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Management : Removal of subdural fluid Massive antibiotics Treatment of ear disease Antiepileptics

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Lateral sinus :Sigmoid sinus Transverse sinus

Usually preceded by the development of extradural perisinus abscess

Spread of infection Torcula herophili (confluence of sinuses) Superior sagittal sinus Superior and inferior petrosal sinus Cavernous sinus Brain abscess Internal jugular vein Subclavian vein

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Commonly associated organisms: Beta hemolytic streptococci Staphylococus aureus Now a days : mixed flora :

Venezio et al in 1982 grew Proteus mirabilis, staphylococci, streptococcus pneumoniae and Bacteroides oralis

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Clinical features: Classical picture before

antibiotics: Severe pyrexial wasting

illness Develop over several

weeks Picket fence fever Headache and neck pain Emaciation with anaemia

Spread along the IJV: Perivenous

inflamation Perivenous edema Suppuration of the

lymph node Paralysis of the lower

cranial nerves ↑ICP Hydrocephalus

Superior extension Superior sagittal sinus Cavernous sinus

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Septic embolisation (sinus thrombophlebitis) Lung fields Large joints Subcutaneous tissue Other viscera Pleuroperitoneal cavity

Mastoid emissary vein: suboccipital abscess

Griesinger’s sign: oedema of the postauricular soft tissues

overlying the mastoid process as a result of thrombosis of the mastoid emissary vein

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Investigations : Hemogram Blood cultures Lumbar puncture :

Low WBC countNormal CSF pressure

Queckstedt or Tobey-Ayer test CT scan:

An empty triangle at the level of the sigmoid sinus, clot surrounded by a high-intensity rim of contrast-enhanced dura: delta sign

Angiography Venography MRI

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•The jugular vein on the side of the suspected thrombosis is compressed• A rise in spinal fluid pressure should occur•Its absence indicates presence of thrombosis.63

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Treatment : Administration of

antibiotics Exposure of the

sinus: incision and removal of contents Perisinus cells need

to be cleared: chances of delayed sinus thrombosis if not cleared

???? Anticoagulants : If in spite of the

treatment the infection is progressively involving the cortical veins

Role of IJV ligation: if the thrombus is

getting dislodged then the need to ligate IJV

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The commonest intracranial complicationChildhood otogenic meningitis is seen most

often as a complication of acute middle ear infection

In adults, it is now more commonly a complication of chronic disease

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Pathways of spread:Bone erosion Suppurative labrynthitis Rupture of established brain abscess

Organisms responsible for acute infection:

Hemophilus influenzae type B

Streptococcus pneumoniae type III

Organisms responsible in chronic infection:

G-ve enteric organisms

Proteus Pseudomonas Anaerobes such as

Bacteroides species

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Clinical features: Headache and neck stiffness Malaise Pyrexia mental hyperactivity Exaggerated Tendon reflexes Photophobia Vomiting

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Diagnisis : Lumbar puncture

Rise in fluid pressure above the normal 100-150 mm Hg Gross appearance of fluid: cloudy and then turbid On histological inspection PMN cells ranging from 0.1-10x109 /lBiochemical tests:

Protein content may rise from normal of 150-400mg/l to a raised level of 2-3g/l

Chloride content may fall from the normal 120mmol/l to 80mmol/l Fall in glucose level from the normal value of 1.7-3.0 mmol/l to

zero Bacteriological examination : gram staining and then culture D/d :

Rupture of brain abscess Rupture of subdural abscess

CT scan MRI PCR

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Treatment :Medical :

Repeated LP to lower the raised pressure Large doses of systemic antibiotics Systemic antibiotics must continue for 10 days

after apparent clinical recovery Dexamethasone??

Surgical : Certainly deterioration or failure of response

over 48hrs implies loculated infection in the mastoid, needing surgical drainage

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Failure of an adequate response to antimicrobial therapy may be a result of :

Organism resistant to chosen antibiotics Persistent leakage of infected material into the

CSF Persistence of a previously unidentified other

complication/Leakage into the CSF from an unrecognized brain abscess.

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It is a focal suppurative process within the brain parenchyma serrounded by a region of encephalitis. Bimodal age of distribution with peaks in the

paediatric age group and in the 4th decade of life.

Almost always develop in the temporal lobe or the cerebellum

Temporal lobe abscess is twice as common as cerebellum

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Various routes of spread: Through an osteitic tegmen tympani , with

middle fossa extradural abscess Local pachymeningitis followed by

thrombophlebitis Extension of infection along periarteriolar

Virchow-Robin spaces Cerebellar abscess are frequently preceded by

lateral sinus thrombophlebitis

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Bacteriology :polymicrobial (also extradural) Anaerobes Streptococcus & staphylococcus G- organism:

Escherichia coliProteus Klebsiella Pseudomonas

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Clinical presentation: Very toxic and drowsy Brain abscess is associated with the triad of:

Headache High grade fever Focal neurological deficits

Cerebellar abscess:Dizziness Ataxia Nystagmus Vomiting

Temporal lobe lesion:Seizure Visual field defects Nominal aphasia

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Diagnosis : Imaging :

Computed tomography Hypodense area by an area of edema, ring

sign MRI: superior to CT scan except for delineation

of temporal bone is poor.

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Other d/d: Meningitis Subdural abscess Lateral sinus thrombophlebitis Otitic hydrocephalus Other masses such as brain tumour.

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Management :High-dose antimicrobial medicationPatient should be first stabilized

neurologically: ? Aspiration with high dose of antibiotics ?Total excision ?parenteral antibiotics

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Syndrome (pseudotumour cerebrii) associated with otitis media with

Increased intracranial pressure Normal CSF findings Spontaneous recovery No abscess

No associated ventricular dilation: benign raised intracranial tension.

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Pathophysiology:Symonds: decreased absorption of CSF

secondary to blockage of arachnoid villi Sahs and Joynt: secondary to brain edema Weed and Flexner: disruption in venous

circulation as a cause .

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Symptoms : Headache Drowsiness Vomiting Blurring of vision Diplopia

Signs: Papilledema 6th cranial nerve palsy Optic disc atrophy

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Diagnosis : ↑CSF pressure Normal CSF biochemistry MRI is the modality of choice

Management: Lowering of the elevated intracranial pressure Medical therapy:

CorticosteroidsMannitolDiuretics Acetazolamide

Eradication of ear disease

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In CSOM ostietis and subsequent bone erosion likely expose the nerve to infection

This results in inflammation and ultimately facial nerve compression

The tympanic segment is the most common area of involvement

HRCT of the temporal bone Management :

Intravenous antibiotics Prompt surgical intervention:

Any attached granulation to the nerve should not be removed

Healthy bone should be removed from the nerve on either side of the diseased segment.

Concomitant corticosteroids

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It has two clinically distinguishable subtypes: Localised, circumscribed, or serous labyrinthitis

without total or permanent loss of function Diffuse, purulent or suppurative labyrinthitis with

permanent total destruction of the sensory elements within the labyrinth

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As the result of osteitis of the labyrinth capsule or of extension along preformed pathways

Potential pathway include: oval window Round window membrane Cholesteatomatous fistula

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Clinical features: Vestibular symptoms precede cochlear symptoms

by hours to days when the site of invasion is semicircular canal

Symptoms Profound vertigo Nausea Vomiting

Signs: Spontaneous nystagmus towards the unaffected

ear. Some degree of ataxia with pastpointing High frequency sensorineural hearing loss Distortion of hearing.

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Treatment : Parenteral antibiotics

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Usually following the serous labyrinthitis Involvement of labyrinth secondary to generalized

meningitis Symptoms: same as serous labyrinthitis including

the lack of fever Key differences:

Symptoms are more rapid and intense The onset of severe vestibular symptoms is

accompanied by a complete loss of cochleovestibular response

A caloric response is conspicuously absent from the disease ear.

d/d: cerebellar abscess

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Treatment: Close and continuous monitoring for symptoms of

intracranial extension Bed rest with minimal head movements Antiemetics Antibiotics If meningeal signs are noted: LP Any signs of intracranial spread: labyrinthectomy Premature surgical trauma can promote

dissemination of infection: mastoid exploration to be deferred until acute symptoms subside.

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Bone erosion by cholesteatoma Most common site: dome of the lateral semicircular

canal. Symptoms :

Episodic vertigo Signs:

Fistula testNegative test does not rule out fistula

Tullio’s phenomenon Site of fistula:

The dome of the LSCC: deviation of eyes towards normal ear. LSCC fistula anterior to the ampulla: deviation towards the

affected ear. Erosion of the vestibule: rotatory horizontal deviation towards

the diseased ear. SSCC: rotatory movements towards the normal ear PSCC: vertical deviation of the eyes.

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Investigations: HRCT temporal bone

Treatment: Surgery :

Managed according to the site, size and status of hearing

The rate of total hearing loss is 8% to 56%

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It is the infected petrosal cells with inadequate drainage causing bone changes of coalescence in the cell walls and resulting in symptoms referable to the petrosa.

Symptoms: Gradneigo’s syndrome:

1.Deep-boring pain: depending upon the site of involvement:Posterior group of cells:

occipital, parietal, or temporal Discharge is from the mastoid

Anterior petrositis: Frontal or behind the eye Drainage is from the tympanum

2.Aural drainage 3,Diplopia : 6th cranial nerve palsy occurs when the

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Investigations: HRCT temporal bone Gallium 67 scan Technetium 99m scan

Management : Parenteral antibiotics Surgical drainage

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Can occur with: Long standing perforation Cholesteatoma

Once any type of mastoiditis causes continuous purulent drainage for 8 or more weeks, the likelihood of complete resolution with antibiotics significantly decreases.

Hallmark triad: Otalgia Postauricular pain Fever

Signs: Postauricular tenderness Protrusion of pinna Postauricular erythema Induration over the mastoid: impending

subperiosteal abscess.

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Investigaton : HRCT temporal bone

Treatment :Parenteral antibioticsMastoidectomy:

Significant bony destruction Poor response to up to 2 weeks of conservative

management.

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Direct destruction of cortical bone / hematogenous spread through small vascular channels.

Well pneumatized mastoids are more susceptible

The most common site of cortex breakdown is through the thin trabecular bone Macewen’s triangle

Signs and symptoms: Auricle is displaced forward and

outward Fluctuant mass can be palpated

behind the ear Bezold’s abscess Luc’s abscess

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Investigations: HRCT temporal bone Management:

Simple mastoidectomy Antibiotics

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Benzold abscess: Breach in the bony plate forming the inner

surface of mastoid tip. Erosion of inner or outer cortex of the mastoid if

periphlebitis or phlebitis propagate the infection.Pus track below the sternocleidomastoid or

even the layer of cervical fascia.d/d : inflamed lymph nodes Investigation : CT scan Treatment :

Complete excision of mastoid pathology Drainage of the abscess Removal of associated granulation tissue.

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