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بسم هللا الرحمن الرحيم OTOSCLEROSIS DEFINITION A primary disease of the otic capsule characterized pathologically by abnormal resorption and deposition of bone HISTOPATHOLOGY • Resorption of bone by osteocytes • Formation of new vascular spongy bone • Formation of dense sclerotic bone AREAS OF PREDILECTION Fissula ante fenestram (80% to 90%) OTHER AREAS • Round window, the apex of the cochlea, the cochlear aqueduct, the semicircular canals, and the stapes footplate itself COCHLEAR INVOLVEMENT 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 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 Involvement of footplate and cochlea 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 PHYSICAL EXAMINATION • Normal tympanic membrane • Schwartze sign (Flamingo flush) PHYSICAL EXAMINATION • Normal tympanic membrane • Schwartze sign (Flamingo flush) • Tuning fork tests PURE TONE AUDIO 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 AUDIOMETRY • Pure tone audiogram • Speech discrimination AUDIOMETRY • Pure tone audiogram • Speech discrimination • Impedence & tympanometry CT SCAN Double ring cochlea or Halo’s sign COCHLEAR OTOSCLEROSIS Isolated pure sensorineural hearing loss without a conductive component 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 DIFFERENTIAL DIAGNOSIS • Congenital fixation of the stapes • Middle ear effusion • Chronic OM and ossicular discontinuity • Tympanosclerosis • Malleus head fixation • Systemic diseases SYSTEMIC DISEASES • Osteogenesis imperfecta – Stapes fixation – Blue sclera – Fractures SYSTEMIC DISEASES • Osteogenesis imperfecta – Stapes fixation – Blue sclera – Fractures • Pagets disease – Crowding in epitympanum – Elevated alkaline phosphatase – Skeletal bone involvement TREATMENT • Observation • Hearing aid • Medical treatment • Surgical treatment OBSERVATION INDICATIONS OF OBSERVATION • Unilateral • Mild CHL • Young age HEARING AID INDICATIONS OF HEARING AID • Refuse surgery • Poor surgical candidate • Following improvement of CHL MEDICAL TREATMENT AIM OF MEDICAL TREATMENT • Stabilize the disease by reduction of the osteoclastic bone resorption and increase osteoblastic bone formation MEDICAL MANAGEMENT • Sodium fluoride: 50-75 mg /day/2years followed by 25 mg for life • Vitamin D • Calcium carbonate INDICATIONS • Cochlear otosclerosis • Patients with confirmed otosclerosis but having progressive SNHL disproportionate to age CONTRAINDICATIONS • Chronic nephritis • Rheumatoid arthritis • Pregnancy and lactation • Children SURGICAL TREATMENT PATIENT SELECTION FOR SURGICAL TREATMENT Socially unacceptable conductive mixed hearing loss Good speech discrimination Age Lifestyle and occupation or ABSOLUTE CONTRAINDICATION OF SURGERY The better or the only functioning ear OTHER CONTRAINDICATIONS • ? Patients experience frequent changes in barometric pressure • “Malignant” otosclerosis • Endolymphatic hydrops • TM perforation • Infections STAPES SURGERY Stapedectomy Stapedotomy STAMP (STApedotomy Minus Prosthesis) or Stapedioplasty 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 STAPEDOTOMY • Equal or better results vestibulocochlear side effects with less COMPARISON 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 SURGICAL PROCEDURE The Incision Permeatal (Transcanal) Endaural STAPEDOTOMY LASER STAPEDOTMY STAMP OPERATIVE PROBLEMS & COMPLICATIONS TM PERFORATION • Proceed and then repair CHORDA TYMPANI INJURY • 30% of cases • Metallic taste • Symptoms usually resolves in 3-4 months • More symptoms if bilateral OBTRUSIVE FACIAL NERVE • 0.5 % • Stapedotomy is usually possible BLEEDING • Mucosal trauma • Active phase • Persistent stapedial artery Persistent stapedial artery ROUND WINDOW OTOSCLEROSIS • About 1% complete (Shuknecht) • If complete: Abandon surgery • If incomplete or not sure: Do not remove bone and proceed OBLITERATIVE OTOSCLEROSIS OF THE OVAL WINDOW • A total stapedectomy is contraindicated because of high risk of surgically induced SNHL INCUS PROBLEMS • Subluxation: Proceed • Dislocation: Remove incus & use a malleus-grip prosthesis FLOATING FOOTPLATE • May be avoided if control holes are used or by using laser fenestration FLOATING FOOTPLATE • May be extracted by needles/hooks with hole inferior to the oval window FLOATING FOOTPLATE • In many cases should be left and surgery is completed with unpredictable results or use laser fenestration 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 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 PERILYMPH FISTULA • Primary or secondary 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 DIAGNOSIS OF PERILYMPH FISTULA • Drop or fluctuation in hearing • Vertigo & tinnitus • Audiometry • ENG • Fistula test • Radiology TREATMENT • Surgical closure 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 REPARATIVE GRANULOMA • Treatment is by emergency tympanotomy and excision SNHL • 0.2-10% • Serous labyrinthitis high frequencies • Surgical trauma PERSISTENCE OR RECURRENCE OF CHL • Prosthesis malfunction • Fibrous adhesion • Incus erosion PERSISTENCE OR RECURRENCE OF CHL • Prosthesis malfunction • Fibrous adhesion • Incus erosion • Missed pathology: e.g. malleus fixation, round window otosclerosis • Otosclerosis regrowth RARE COMPLICATIONS • Facial paralysis • Acute otitis media • Cholesteatoma THANK YOU