Congenital cholesteatomas of the middle ear: a different experience.

A retrospective analysis is presented of 44 cases of congenital cholesteatoma of the middle ear. Twenty-one patients had cholesteatoma localized to the posterosuperior mesotympanum in complete contrast to the commonly reported anterosuperior location seen in only two cases. The remaining 21 patients had cholesteatoma involving either the entire mesotympanum or epitympanum or both. The posteriorly located congenital cholesteatomas might represent a completely different entity and originate from the trapped epithelial cell rests in the posterior mesotympanum during the development of temporal bone. All but one patient were treated by using a closed tympanoplasty. Eight patients underwent a single-stage surgery. A preplanned second-stage procedure was performed in 33 patients, and three are awaiting their second stage. Residual disease was seen in 19 (57%) patients undergoing second-stage surgery. No patient has had recurrent disease so far. Thirty-eight (85%) patients had a preoperative air-bone gap of > or = 30 dB. Of the 33 patients considered for hearing results, 16 (48%) had a postoperative gap within 10 db.