Cholesteatoma and fibrous dysplasia of the temporal bone: case report and review of the literature.

摘要

The term fibrous dysplasia was introduced by Lichtenstein and Jaffe to define a group of peculiar lesions histologically characterized by rearrangement of the bone structure. It is localized in the temporal bone in less than 10% of afflicted patients, with a male to female ratio of 2:1. It presents macroscopically like a painless, elastic mass of variable colour in the external auditory canal (EAC) or as a swelling of the squama and/or the mastoid or in the preauricular region. Histologically, it is represented by irregular trabeculae of mature bone with woven architecture dipped into a collagen stroma with fibrovascular and osteoblastic features. The disease is classified in three types1: (1) monostotic (type I), (2) polyostotic (type II), and (3) the McCune-Albright syndrome, the latter being characterized by polyostotic involvement, skin hyperpigmentation, and endocrine disturbances (type III). The usual clinical findings are conductive deafness, modification of temporal bone morphology, and stenosis of the EAC. It is seldom associated with cholesteatoma of the outer or middle ear and, rarely, with facial nerve paralysis. We present a case of an isolated temporal bone fibrous dysplasia with a cholesteatoma medial to the ear canal stenosis in a teenager and a review of the literature.