[Congenital cholesteatoma and associated ossicular malformations]

15 cases of congenital cholesteatoma surgically treated from 1982 to 1992 are presented. Clinical diagnosis criteria, pathology, localisations and extensions to the middle ear are analyzed. Ossicular malformations were associated in one third of the patients of this series. These ossicular malformations underscore the congenital origin of such cholesteatoma. A review of the literature and pathogenesis of congenital cholesteatoma is discussed.