Abstract
To report a contemporary review from a single-institution series on Paget's disease of the temporal bone (PDTB). Retrospective chart review of patients evaluated from 1998 to 2016. Quaternary referral center. Patients with radiographically confirmed PDTB. Clinical, audiological, and radiological features and management strategies of PDTB. A total of 50 temporal bones in 27 patients (15 men) were diagnosed with PDTB. Symptoms at presentation included hearing loss (n = 23, 85%), headache (n = 18, 67%), dizziness (n = 14, 52%), tinnitus (n = 5, 19%), chronic otitis media (n = 2, 7%), hemifacial spasm without facial paralysis (n = 1, 4%), multiple cranial neuropathies (n = 1, 4%), and neoplastic transformation (n = 1, 4%). Of the 23 ears with audiometric data available for review, 65% exhibited sensorineural hearing loss, and 35% mixed hearing loss. Long-term audiometric follow-up was available on two patients, both of whom demonstrated hearing loss at a rate greater than would be expected for normal aging. Two patients underwent successful cochlear implantation, achieving open-set speech recognition. Radiographic features of temporal bone involvement are reviewed and illustrated. This is the largest single-institution clinical ser...Continue Reading
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