Long-term results of congenital cholesteatoma surgery including elimination of disease and restoration of function have been reported. Doyle and Luxford14 reported 60 ears in 59 patients followed during a 15-year period. In regard to elimination of disease, cholesteatoma was considered cured in 90%. No recurrent cholesteatoma was found at the second-stage operation in all patients with initial limited cholesteatoma, and in all patients after a second-stage operation removal of residual cholesteatoma with a greater than 10-year follow-up. In regard to preservation of function, postoperative air-bone gap of 10 dB or less was found in 63% and 20 dB or less in 91% of ears, with an average postoperative speech reception threshold of 20 dB HL. Friedberg11 reported that congenital cholesteatoma has a high rate of recurrence after removal. Substantial submucosal extension may explain the cause of recurrence. Appropriate surgical treatment, however, may reduce the residual and recurrence rates.
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