بسم الله الرحمن الرحيم. OTOSCLEROSIS DEFINITION A primary disease of the otic...
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الرحيم الرحمن الله بسم
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OTOSCLEROSIS
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DEFINITION
A primary disease of the otic capsule
characterized pathologically by abnormal
resorption and deposition of bone
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HISTOPATHOLOGY
• Resorption of bone by osteocytes
• Formation of new vascular
spongy bone
• Formation of dense sclerotic bone
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AREAS OF PREDILECTION
Fissula ante fenestram (80% to 90%)
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OTHER AREAS
• Round window, the apex of the cochlea, the
cochlear aqueduct, the semicircular canals, and
the stapes footplate itself
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COCHLEAR INVOLVEMENT
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ETIOLOGY
• Unknown cause
• Positive family history in about 60%
• Inherited by autosomal dominant transmission with
incomplete penetration (60%)
• Persistent measles virus infection
– Detection of measles virus RNA in the affected bone
– Detection of measles virus-specific antibodies in the
perilymph
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PHYSIOLOGY• Conductive HL: due to fixation of the
stapedial footplate
• Mixed HL: due to– Liberation of toxic metabolites into the inner
ear
– Vascular compromise from sclerosis and narrowing of vascular channels
– Direct extension of lesions into the inner ear
• Cochlear otosclerosis
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Involvement of footplate and cochlea
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CLINICAL PRESENTATION
• Hearing loss of gradual onset at 15 - 45 years• Slowly progressive course• 70% are bilateral• Accelerates with pregnancy (30-40%)• Tinnitus• Paracusis Willisii• Change of the speech pattern • Vestibular symptoms
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PHYSICAL EXAMINATION
• Normal tympanic membrane
• Schwartze sign (Flamingo flush)
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PHYSICAL EXAMINATION
• Normal tympanic membrane
• Schwartze sign (Flamingo flush)
• Tuning fork tests
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PURE TONE AUDIO
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CARHART’S NOTCH
• Decrease in bone conduction thresholds• 5 dB at 500 Hz
• 10 dB at 1000 Hz
• 15 dB at 2000 Hz
• 5 dB at 4000 Hz
• Explanation is not known
• Reverses following successful surgery
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AUDIOMETRY
• Pure tone audiogram
• Speech discrimination
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AUDIOMETRY
• Pure tone audiogram
• Speech discrimination
• Impedence & tympanometry
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CT SCAN
Double ring cochlea or Halo’s sign
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COCHLEAR OTOSCLEROSIS
Isolated pure sensorineural hearing loss without
a conductive component
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CRITERIA FOR DIAGNOSIS OF COCHLEAR OTOSCLEROSIS
• Progressive pure cochlear loss beginning at the usual age of onset for otosclerosis
• Unilateral conductive hearing loss consistent with otosclerosis and bilateral symmetric SNHL
• Positive Schwartze’s sign• Positive family history• Excellent discrimination• Stapedial reflex demonstrating the “on-off effect”• CT: demineralization of the cochlea
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DIFFERENTIAL DIAGNOSIS
• Congenital fixation of the stapes
• Middle ear effusion
• Chronic OM and ossicular discontinuity
• Tympanosclerosis
• Malleus head fixation
• Systemic diseases
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SYSTEMIC DISEASES
• Osteogenesis imperfecta– Stapes fixation– Blue sclera– Fractures
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SYSTEMIC DISEASES
• Osteogenesis imperfecta– Stapes fixation– Blue sclera– Fractures
• Pagets disease– Crowding in epitympanum– Elevated alkaline phosphatase– Skeletal bone involvement
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TREATMENT
• Observation
• Hearing aid
• Medical treatment
• Surgical treatment
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OBSERVATION
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INDICATIONS OF OBSERVATION
• Unilateral
• Mild CHL
• Young age
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HEARING AID
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INDICATIONS OF HEARING AID
• Refuse surgery
• Poor surgical candidate
• Following improvement of CHL
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MEDICAL TREATMENT
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AIM OF MEDICAL TREATMENT
• Stabilize the disease by reduction of the
osteoclastic bone resorption and increase
osteoblastic bone formation
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MEDICAL MANAGEMENT
• Sodium fluoride: 50-75 mg /day/2years
followed by 25 mg for life
• Vitamin D
• Calcium carbonate
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INDICATIONS
• Cochlear otosclerosis
• Patients with confirmed otosclerosis but
having progressive SNHL disproportionate
to age
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CONTRAINDICATIONS
• Chronic nephritis
• Rheumatoid arthritis
• Pregnancy and lactation
• Children
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SURGICAL TREATMENT
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PATIENT SELECTION FOR SURGICAL TREATMENT
Socially unacceptable conductive or
mixed hearing loss
Good speech discrimination
Age
Lifestyle and occupation
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ABSOLUTE CONTRAINDICATION OF SURGERY
The better or the only functioning ear
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OTHER CONTRAINDICATIONS
• ? Patients experience frequent changes in
barometric pressure
• “Malignant” otosclerosis
• Endolymphatic hydrops
• TM perforation
• Infections
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STAPES SURGERY
Stapedectomy Stapedotomy
STAMP (STApedotomy Minus Prosthesis) or Stapedioplasty
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STAPEDECTOMY• Results probably are the best
• More traumatic to the inner ear– Increased post-op vestibular symptoms
– Higher incidence of postoperative SNHL
• The operation is unavoidable in:– Comminuted fracture of the footplate
– Revision surgery
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STAPEDOTOMY
• Equal or better results with less
vestibulocochlear side effects
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COMPARISON
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STAMP
• Preservation of the stapedius
tendon– Reduction in hyperacusis
– Reduction in risk for long-term
postoperative inner ear injuries
• No prosthesis complications
• Very difficult technique
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SURGICAL PROCEDURE
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The Incision
Permeatal (Transcanal) Endaural
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STAPEDOTOMY
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LASER STAPEDOTMY
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STAMP
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OPERATIVE PROBLEMS & COMPLICATIONS
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TM PERFORATION
• Proceed and then repair
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CHORDA TYMPANI INJURY
• 30% of cases
• Metallic taste
• Symptoms usually resolves in
3-4 months
• More symptoms if bilateral
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OBTRUSIVE FACIAL NERVE
• 0.5 %
• Stapedotomy is usually possible
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BLEEDING
• Mucosal trauma
• Active phase
• Persistent stapedial artery
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Persistent stapedial artery
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ROUND WINDOW OTOSCLEROSIS
• About 1% complete (Shuknecht)
• If complete:
Abandon surgery
• If incomplete or not sure:
Do not remove bone and
proceed
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OBLITERATIVE OTOSCLEROSIS OF THE OVAL WINDOW
• A total stapedectomy
is contraindicated
because of high risk
of surgically induced
SNHL
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INCUS PROBLEMS
• Subluxation:
Proceed
• Dislocation:
Remove incus & use a
malleus-grip prosthesis
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FLOATING FOOTPLATE
• May be avoided if control
holes are used or by using
laser fenestration
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FLOATING FOOTPLATE
• May be extracted by needles/hooks with hole
inferior to the oval window
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FLOATING FOOTPLATE
• In many cases should be left
and surgery is completed
with unpredictable results or
use laser fenestration
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MALLEUS ANKYLOSIS
• About 0.5%
• May be congenital or acquired
• Causes about 15-20 dB CHL
Remove malleus head and the incus and
use malleus grip prosthesis
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CSF GUSHER
• Due to fundal defect of IAM or widened cochlear
aqueduct
• Introduce spinal catheter and proceed
Or
• Pack with fascia and gauze for 4-5 days with delayed
reconstruction that avoid reopening the fenestra
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PERILYMPH FISTULA
• Primary or secondary
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PREVENTION OF PERILYMPH FISTULA
• Stapedectomy < stapedotomy
• Oval window seal
• No fat or gel-foam for seal
• Prohibit nose blowing, flying, diving, &
lifting heavy objects postoperatively
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DIAGNOSIS OF PERILYMPH FISTULA
• Drop or fluctuation in hearing
• Vertigo & tinnitus
• Audiometry
• ENG
• Fistula test
• Radiology
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TREATMENT
• Surgical closure
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REPARATIVE GRANULOMA
• Granuloma formation around the prosthesis and incus
• 1-5%
• Gradual deterioration 5-15 days postoperativly
• Vertigo, tinnitus and deafness
• Otoscopy: reddish discoloration of the posterior TM
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REPARATIVE GRANULOMA
• Treatment is by emergency
tympanotomy and excision
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SNHL
• 0.2-10%
• Serous labyrinthitis -
high frequencies
• Surgical trauma
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PERSISTENCE OR RECURRENCE OF CHL
• Prosthesis malfunction
• Fibrous adhesion
• Incus erosion
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PERSISTENCE OR RECURRENCE OF CHL
• Prosthesis malfunction
• Fibrous adhesion
• Incus erosion
• Missed pathology: e.g. malleus fixation, round
window otosclerosis
• Otosclerosis regrowth
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RARE COMPLICATIONS
• Facial paralysis
• Acute otitis media
• Cholesteatoma
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THANK YOU