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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December 31, 2012
Cochlear (Retrofenestral) Otosclerosis
- Genetically mediated metabolic bone disease. Mode of inheritance: autosomal dominant; penetrance and expressivity vary.
- Women more commonly affected
- Disease is bilateral in 80% of cases.
- Two separate pathologic phases: otospongiotic and otosclerotic phases
- Two categories: fenestral and retrofenestral otosclerosis
- Key Diagnostic Features: Cochlear otosclerosis: Ring of lucency likened to the "fourth turn" sign or "double ring" sign is seen around cochlea. Subsequent sclerotic phase follows. PET-CT trials quantifying ostoblastic activity based on 18F-FDG uptake to monitor disease progression are currently being evaluated.
- DDx: labyrinthitis ossificans; Paget's disease; osteogenesis imperfecta; fibrous dysplasia
- Rx: cochlear implantation
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