Congenital cholesteatoma

Abstract

We present a British series of eleven patients with primary cholesteatoma, including one patient with bilateral disease. Eight children presented with a history of hearing loss, while one child had had recurrent otitis media and another had had earache. Operative findings were: in five ears, cholesteatoma confined to the antero-superior segment with intact ossicles, in a further four, cholesteatomas extending throughout the mesotympanum with ossicular erosion in one, and in two ears posterior disease throughout the middle ear and mastoid, which had eroded the ossicles in both cases. The five cases of antero-superior cholesteatoma lend most support to Michaels' concept of epidermoid formation as a possible source of congenital cholesteatoma. With a greater awareness of the problem and careful examination of the antero-superior quadrant of the tympanic membrane, earlier diagnosis may be possible enabling removal of small intact cholesteatoma sacs and preserving the structures of the middle ear and therefore the hearing. A screening programme for infants included as part of their routine examination which would be undertaken by examiners who are trained to be more aware of the problem and skilled at otoscopy, would help in the earlier detection of such cases as is shown by reports from the U.S.A.