CONGENITAL CHOLESTEATOMA

Objective: To review the characteristics of congenital cholesteatomata . Design: Case series. Setting: Tertiary care (referral-based) private practice. Patients and Other Participants: Fourteen patients were inc luded in the study. The diagnosis of congenital cholesteatoma was base d on an intact tympanic membrane on physical examination; a history th at excluded tympanic membrane perforation, otorrhea, or previous otolo gic procedure; and a documented cholesteatoma at the time of surgical removal. Intervention: Surgical procedures including tympanotomy, atti cotomy, and tympanotomy with mastoidectomy were performed on all patie nts. Main Outcome Measures: Removal of cholesteatoma. Results: Four of the 14 patients had lesions isolated to the anterosuperior quadrant o f the tympanum; the remainder had more extensive disease with notable posterior tympanic involvement. Three of the patients underwent surger y for recidivism; none were from isolated anterior lesions. One of the se patients was referred at the time of recurrence, one had known resi dual cholesteatoma, and one had recurrence. Conclusions: Clinical pres entation and surgical findings enable the differentiation of two separ ate sites of congenital cholesteatoma formation: the anterosuperior an d posterior-superior regions of the tympanic cavity. Recidivism of the lesion appears more commonly with posterior-superior congenital chole steatomas.