Learning Objectives:

Background: The main goal of congenital cholesteatoma treatment is total eradication of the disease in order to prevent recurrence and preserve normal structure and function. This is usually achieved by a surgical method depending on the nature and extent of the disease. The authors aims to find out the proper surgical method for each stage by comparing possible surgical methods and their following prognoses depending on the stage.

Method: We retrospectively reviewed 55 patients from 2010 to 2015 who were diagnosed congenital cholesteatoma. The surgical treatment was performed by several different approaches determined by the location and extent of the disease and degree of adhesion to the surrounding structure. After 6 months of follow up, Recurrence was evaluated. Data was analyzed according to the patients age, stage by Potsic's classification, relationship between age and location of the lesion and also relationship between surgical methods and results.

Results: Age distribution was from 1 year to 14 years and among these patients, 25(45%) were under 2 years of age. 26 Patients(47%) were classified as Stage I with the highest number. Patients diagnosed at an elder age showed a tendancy of lesions being located more posteriorally and being found at more various locations such as the mastoid or attic. The result of surgical procedure was stage I with no recurrence, stage II with 1 case of recurrence, stage III with no recurrence, stage IV with 2 cases of recurrence. Recurrence was found in 3 cases among the total 55 cases.

Conclusion: Early diagnosed diseases with lower stage were treated with surgical approaches capable of removing the lesion and at the same time preserving normal function. And also in these cases rates of recurrence and complication revealed to be low. Therefore early diagnosis with minimal conservative surgery is the most important principle to achieve the main goal of congenital cholesteatoma treatment.