Paget's disease and the temporal bone--a clinical and histopathological review of six temporal bones.

Paget's disease of bone occurs more commonly in the elderly and has been reported to involve the temporal bone in 30% of those afflicted. The clinical and histopathological features of six temporal bones from three patients with this disease are reported and the relevant literature reviewed. The effect of Paget's disease on the middle ear structures was more variable than its extension into the otic capsule. Pagetic involvement of the otic capsule was observed in five temporal bones. One patient had bilateral asymptomatic neurofibromas in the eighth cranial nerve. the potential mechanisms responsible for the conductive deafness, the sensorineural deafness and vestibular dysfunction associated with Paget's disease are discussed.