Updated: 12/17/2021

Otosclerosis

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Topic
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  • 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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(M2.ET.17.4828) A 28-year-old Caucasian woman presents to her primary care physician due to decreased hearing of the right ear. She reports that her hearing has progressively worsened over the course of a year. In addition to her current symptoms, she has also noticed ear ringing of the affected ear, as well as dizziness. She states that her hearing is improved in a noisy environment and that her friends have noted that she usually speaks quietly during conversations. Medical history is significant for multiple episodes of acute otitis media and seasonal allergies. Family history is significant for her father having "ear surgery" when he was approximately 30-years-old. Otoscopy findings are seen in Figure A. Which of the following is most likely to be found on physical exam?

QID: 109462
FIGURES:

Air conduction is greater than bone conduction of the affected ear on Rinne testing

9%

(9/97)

Sound lateralizes to the unaffected ear on Weber testing

29%

(28/97)

Air conduction is less than bone conduction of the unaffected ear on Rinne testing

6%

(6/97)

Sound lateralizes to the affected ear on Weber testing

52%

(50/97)

Physical exam will be unremarkable

4%

(4/97)

M 6 E

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