[Cholesteatoma behind an intact ear drum--an overlooked disease?].

On the background of increasing international interest for early diagnosis, pathogenesis and treatment of cholesteatoma in children, a sub-group is described and termed primary cholesteatoma, congenital cholesteatoma in the middle ear or cholesteatoma in the middle ear behind an intact ear-drum. This review is based on a retrospective review of cholesteatoma in children submitted to operation in Hjørring Hospital during a period of five years from 1986 to 1991. All of the children were under the age of 16 years. Out of 22 cholesteatoma treated operatively in children, the authors found six cases (27%) of congenital cholesteatoma. The symptomless congenital cholesteatoma in the middle ear is recognized clinically by otoscopy where it appears as a white shadow, most frequently in the anterior upper quadrant, behind an intact ear-drum. Early diagnosis is important and may be established by general practitioners, paediatricians and otologists at routine otoscopy.