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