A temporal bone report is presented of a patient with progressive sensorineural hearing loss and advanced Paget’s disease involving the skull. The histopathology reveals Pagetic bone invading the internal auditory canal and compressing the cochlear division of the VIIIth cranial nerve resulting in severe neural degeneration. There are no other histopathologic abnormalities present in the cochlea to explain the hearing loss. A lowfreqnency conductive hearing loss was also present, but no histopathologic correlate could be identified.
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