OBJECTIVES: We described the characteristics of congenital cholesteatoma in Japanese patients and assessed whether the staging system is useful for predicting the rate of residual disease, the need for reexploration, and surgical outcomes. METHODS: We performed a retrospective chart analysis of 23 consecutive patients with congenital cholesteatoma. RESULTS: The proportion of cases with anterior-superior quadrant involvement was significantly lower in the Asian group than in Western patients. The total residual rate was 26%, and there was a positive association between stage and residual rate, ranging from 0% in stage I and II to 44% in stage IV. Canal wall-up tympanomastoidectomy was the most frequent procedure (57%), and 61% had reexploration. CONCLUSIONS: Congenital cholesteatoma in Asia is less likely to involve the anterior-superior quadrant than in Western patients. The 4-stage system was useful for predicting residual rates, even in patients in whom anterior-superior quadrant involvement was less common. Postoperative hearing was significantly related to the stages. A routine second-look procedure may be unnecessary in the early stages, whereas reexploration would be better performed in advanced stages. Endoscopy might reduce residual disease and the need for reexploration in the near future.
OBJECTIVES: We described the characteristics of congenital cholesteatoma in Japanese patients and assessed whether the staging system is useful for predicting the rate of residual disease, the need for reexploration, and surgical outcomes. METHODS: We performed a retrospective chart analysis of 23 consecutive patients with congenital cholesteatoma. RESULTS: The proportion of cases with anterior-superior quadrant involvement was significantly lower in the Asian group than in Western patients. The total residual rate was 26%, and there was a positive association between stage and residual rate, ranging from 0% in stage I and II to 44% in stage IV. Canal wall-up tympanomastoidectomy was the most frequent procedure (57%), and 61% had reexploration. CONCLUSIONS:Congenital cholesteatoma in Asia is less likely to involve the anterior-superior quadrant than in Western patients. The 4-stage system was useful for predicting residual rates, even in patients in whom anterior-superior quadrant involvement was less common. Postoperative hearing was significantly related to the stages. A routine second-look procedure may be unnecessary in the early stages, whereas reexploration would be better performed in advanced stages. Endoscopy might reduce residual disease and the need for reexploration in the near future.