Congenital cholesteatoma in a case of congenital aural atresia

Objective: A congenital cholesteatoma arising in the setting of congenital aural atresia is described. This represents the first case reported in the literature. Study Design: Case report. Setting: Tertiary otologic referral center, University of Virginia Health System, Charlottesville, Virginia. Patient: Eight-year-old patient with unilateral congenital aural atresia. Interventions: Preoperative computer tomographic scanning and surgical atresia repair with removal of cholesteatoma. Main Outcome Measures: Recurrence of cholesteatoma; epithelialized ear canal; hearing improvement. Results: A congenital cholesteatoma located medial to the ossicular mass in a child with congenital aural atresia was removed, with no evidence of recurrent disease at a revision operation 1 year later. Hearing was improved to a speech reception threshold of 25 dB. Conclusion: Congenital cholesteatoma can arise medial to the ossicular mass in congenital aural atresia. This finding lends support to the epidermoid rest theory for the cause of congenital cholesteatoma.