Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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June 18, 2012
Cochlear Otosclerosis
- Also known as otospongiosis
- One of the 2 types of otosclerosis: fenestral and cochlear.
- Etiology unknown
- Patients present with progressive mixed hearing loss, and, occasionally, tinnitus and vertigo. Otoscopy demonstrates the Schwartze sign, which refers to a vascular hue behind the tympanic membrane.
- Key Diagnostic Features: Foci of demineralization involving the otic capsule. When more widespread, a "halo" or "double-ring" sign will be seen surrounding the cochlea. PET-CT can demonstrate increased uptake and can be used to monitor disease progression.
- DDx: Osteogenesis imperfecta, Paget disease, fibrous dysplasia, otosyphilis
- Rx: Cochlear implantation
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