Ten cases of apparent congenital cholesteatoma of the middle ear and mastoid are presented. Five of the cholesteatomas were in the middle ear, two involved principally the attic region, one was isolated in the mastoid process, and the last two showed extensive attic and mastoid involvement. The surgical management of the 10 cases resulted in substantial hearing improvement in 4 cases, no change in the hearing loss in 4 cases, and normal hearing was preserved in 2 cases. A historical review of the pathogenesis of cholesteatoma provides: 1) a classification of the theories of cholesteatoma genesis; 2) a classification of cholesteatoma of the temporal bone. The salient clinical features of intratemporal congenital cholesteatomata are compared with those of congenital cholesteatoma in the middle ear and mastoid.
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