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بسم الله الرحمن الرحيم
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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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Fissula ante fenestram (80% to 90%)
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 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 Pagets disease
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: 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 STAMP (STApedotomy Minus Prosthesis) or Stapedioplasty
Stapedectomy Stapedotomy
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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 No prosthesis complications
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 dB CHL Remove malleus head and the incus and use malleus grip prosthesis
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CSF GUSHER Introduce spinal catheter and proceed Or
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 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
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