Paget's disease of the temporal bone.

Osteitis deformans Paget is a fairly common, heritable, sometimes progressive disease of bone which affects primarily the axial skeleton and may lead to deformity and weakness. It affects 3% of the population over forty years of age and males more frequently than females. The skull and temporal bones become involved in about two-thirds of the patients. Progressive involvement of the temporal bones may lead to alteration of position, increase in size and change of architecture of the petrous pyramid, external canal, middle ear and inner ear capsule. These changes in turn may produce impairment of hearing (about 30-50% of cases) and vestibular function (20-25%). The clinical, radiological, and pathological manifestations of Paget's disease of the temporal bone are discussed in detail and explained with photomicrographs. Examples of tumor formations and vascular changes are presented.