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Updated: Dec 17 2021


  • Snapshot
    • A 19-year-old woman presents to her primary care physician due to progressive left ear hearing loss. She is otherwise healthy with no significant past medical history. Her father began wearing a hearing aid around the age of 23. Tympanic membranes are normal on otoscopy. A tuning fork is placed at the center of the forehead and showed lateralization to the left ear.
  • Introduction
    • An autosomal dominant disease of the middle ear
      • stapedial bony overgrowth → stapedial stiffness → stapedial fixation → conductive hearing loss
        • normal bone of the stapes is replaced with sclerotic or spongiotic bone
        • small percentage of patients may experience cochlear otosclerosis → sensorineural hearing loss, or mixed hearing loss
      • most cases are bilateral (~85%)
  • Presentation
    • Progressive painless hearing loss
    • Patients may experience tinnitus
    • Tympanic membrane is usually normal
    • Conductive hearing loss pattern on Weber and Rinne testing
      • Weber
        • vibration is louder on affected ear
      • Rinne
        • bone conduction is greater than air conduction
  • Evaluation
    • Progressive conductive hearing loss in the setting of family history
    • Tone audiometry (most useful)
  • Differential
    • Cerumen impaction
    • Otitis externa and media
    • Cholesteatoma
  • Treatment
    • Hearing aid
    • Surgical stapectomy
    • Cochlear implantation
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