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Key Features

  • A progressive disease with a marked familial tendency that affects the bony otic capsule

Clinical Findings

  • Slowly progressive hearing loss that is usually bilateral and asymmetric, although unilateral disease may occur in up to 30% of patients

  • Normal tympanic membrane on otoscopic examination

  • Lesions involving the footplate of the stapes result in increased impedance to the passage of sound through the ossicular chain, producing conductive hearing loss

  • When otosclerotic lesions impinge on the cochlea ('cochlear otosclerosis'), permanent sensory hearing loss occurs

Diagnosis

  • Otoscopic examination is to exclude other causes of chronic hearing loss such as cholesteatoma and middle ear effusion

  • Audiogram to document conductive hearing loss

  • Weber test should lateralize to the ear with the greater conductive deficit

Treatment

  • The conductive hearing loss may be treated either through the use of a hearing aid or surgical replacement of the stapes with a prosthesis (stapedectomy)

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