When fibrous dysplasia affects the temporal bone, it most often presents with conductive hearing loss attributable to stenosis of the external auditory canal. Sensorineural hearing loss has usually been attributed to involvement of the otic capsule. We present a patient with bilateral fibrous dysplasia of the temporal bones who complained of unilateral hearing loss, facial tingling, and facial twitching. The audiogram showed severe sensorineural hearing loss. The hearing markedly improved and facial twitching and tingling ceased after decompression of the internal auditory canal via a middle fossa approach. This is the only case of which we are aware showing reversal of sensorineural hearing loss caused by fibrous dysplasia.
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