[Congenital cholesteatoma of the ear in the child. Clinical, follow-up and therapeutic analysis of a series of 34 cases]

Abstract

Long a subject of debate, congenital cholesteatomas of the middle ear appear to be a specific clinical entity different from the much more frequent classical acquired cholesteoma. Characteristic features of congenital cholesteatomas are young age at diagnosis, typical peroperative presentation, satisfactory mastoid air cells in almost all cases, and associated congenital malformations, which may involve the otology system or not. Diagnosis is a difficult task due to the long latency period with no clinical manifestations. These congenital cholesteatomas appear to be more aggressive in a mastoid with functioning air cells. Thus open excision does not appear to be appropriate and should be reserved for selected cases. For us, the closed technique with two procedures is more adapted but requires good cooperation with the family. The risk of recurrence is however significant and at least comparable to that of acquired cholesteatomas in children. Follow-up should be persuade as long as possible. Functional results have been encouraging even though ossicular destruction is frequent. The quality of the auditory tube appears to be a determining factor.