The management of congenital cholesteatoma: surgical results of 42 cases.

Radical surgery for congenital cholesteatoma leaves the patient, usually a child, with an ear that requires care for life. At the Otologic Medical Group, the intact canal wall technique has been used for many patients with cholesteatoma. To determine results of this management in the treatment of congenital cholesteatoma, we reviewed records of all patients treated between 1974 and 1985. Forty-two ears were studied in 41 patients. A closed middle-ear space was achieved in all cases, and an intact canal wall was maintained in 41 (98%). Follow-up averaged 4.3 years. Of the 41 cases with serviceable hearing before surgery, 28 (68%) had postoperative hearing within 10 dB of the best preoperative bone conduction threshold. Thirty-eight of the 41 achieved a final hearing result within 20 dB of the best bone conduction threshold.