Congenital cholesteatoma: 20 years' experience at The Children's Hospital of Philadelphia.

OBJECTIVE: We report our experience with congenital cholesteatoma over a span of 20 years with an emphasis on presenting characteristics and predictors of outcome.

METHODS: We conducted a retrospective review from 1981 through 2000.

RESULTS: One hundred seventy-two congenital cases were identified in 167 patients. Five patients had bilateral disease. The majority (72%) were found in boys, with an average age of 5.0 years. Hearing loss was slight to moderate. When confined to 1 quadrant, cholesteatoma was anterosuperior in 82% of cases; 47% had cholesteatoma in 2 or more quadrants. Ossicular chain involvement was found in 43% of all cases, and mastoid extension was evident in 23%. The rate of recurrent disease was directly related to the extent and number of quadrants involved.

CONCLUSION: To our knowledge, this is the largest series of congenital cholesteatomas to be reported. This review confirms the male predominance and predilection for the anterosuperior quadrant. The extent of cholesteatoma and its relation to residual disease should be used as a guide for planning a second-look procedure.