Clinical Characteristics and Treatment Outcomes of Congenital Cholesteatoma

OBJECTIVES: Since its introduction in 2002, the staging system for congenital cholesteatoma, as defined by Potsic, has been used widely owing to its simplicity and predictability. The aim of the present study was to analyze the clinical characteristics and surgical treatment outcomes of congenital cholesteatoma and to correlate them with postoperative recurrence. MATERIALS and METHODS: A retrospective chart review was performed for 38 patients who were diagnosed with congenital cholesteatoma and who underwent surgical resection between August 2007 and November 2014 at the Department of Otorhinolaryngology of our hospital. RESULTS: The mean age of the patients was 7.9 +/- 8.2 years, and the number of males and females was 20 and 18, respectively. The mean follow-up period was 30 +/- 26 months. Of the 38 patients, 9 (24%) had residual or recurrent cholesteatoma during follow-up examination after primary surgery. The results showed that the size (>= 4 mm) of the lesion was significantly correlated with residual or recurrent disease (p=0.026). The disease extent (single vs. multiple quadrant involvement), type (closed vs. open type), and stage; applied surgical method (with mastoidectomy vs. without mastoidectomy); and preoperative hearing level (normal vs. abnormal) were not significantly correlated with residual or recurrent disease. CONCLUSION: The disease extent, type, and stage; applied surgical methods; and preoperative hearing level were not significantly correlated with residual or recurrent disease. The size (>= 4 mm) of congenital cholesteatoma was significantly correlated with residual or recurrent disease.