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Fibrous dysplasia involving the temporal bone is unusual. The most common initial findings are stenosis of the external auditory canal and conductive hearing loss. The frequency of sensorineural hearing loss and facial paresis as complications of fibrous dysplasia have been poorly documented in the past. Forty-three cases of fibrous dysplasia of the temporal bone previously published in the otolaryngologic literature are reviewed, and 10 new cases are reported. In addition to age, sex, and frequency of various presenting complaints, the audiometric, radiographic, and surgical data are evaluated in both groups to better describe the manifestations of disease, degree and type of hearing loss, and success of surgical intervention. In nearly 70% of cases in this study, fibrous dysplasia was monostotic. Although most patients had a conductive hearing loss, 17% of patients demonstrated profound sensorineural hearing …