Congenital Middle Ear Cholesteatoma in Children

Kim, Jong Hyung; Park, Kyung Ho; Park, Shi Nae; Park, Jun Ook; Hong, Sung Jin; Choi, Ji Young; Yeo, Sang Won

Abstract

BACKGROUND AND OBJECTIVES: Congenital middle ear cholesteatoma (CMEC) is a rare entity that may go undiagnosed for years. Aims of this study were to assess the characteristic features and recurrence of CMEC in pediatric patients of different stages and to determine the value of preoperative CT scan in CMEC.
SUBJECTS AND METHOD
Thirty cases of CMEC under 15 years old that had been treated at the hospitals of the Catholic University from 1995 through 2005 were reviewed retrospectively. The age range was from 2 to 13 with the mean age of 6.2. The main outcome measures were CT findings, surgical findings, recurrence rate and hearing assessment.
RESULTS
Preoperative CT scan accurately predicted the extent of the cholesteatoma seen during surgery in 25/30 (83.3%). The recurrence rate of CMEC was 6.7% (2/30) and all of recurrent cases were belonged to stage IV. In the recurrent cases, cholesteatomas were extended to sinus tympani and facial recess at revisional operation as well as at the initial operation.
CONCLUSION
Preoperative CT scan is essential in defining the extent of existing pathology. The intra-operative CMEC extension and location influence the outcome of surgery. In the higher stages, careful eradication of disease, particularly in the region of sinus tympani and facial recess, are recommended.