Congenital cholesteatoma clinical features and surgical results.

We analyzed congenital cholesteatomas in 63 ears of 63 patients who were operated on over a 24-year period. We investigated the presenting symptoms, tympanic membrane findings, cholesteatoma morphology and location, associated anomalies of the ossicles, surgical methods, and outcome of treatment. The age of the patients ranged from 2 to 51 years (mean, 13.3 years), and the closed type of cholesteatoma and the open type were found in 57 ears and 6 ears, respectively. Seventy-three percent of the patients were 15 years or younger. Hearing loss was complained of as a presenting symptom by 60.3% of the patients. In particular, all the patients with the open type of cholesteatoma reported hearing loss as a chief complaint, and most of them had a normal tympanic membrane. Many of the cholesteatomas were located at or around the posterosuperior part of the tympanic cavity. The lesion was confined to the tympanic cavity in 26 (41.3%) ears and to the petrous apex in 1 (1.6%) ear. Advanced cholesteatoma that extended from the tympanic cavity into the mastoid antrum was seen in 35 (55.6%) ears, and multiple cholesteatoma was present in 1 (1.6%) ear. Associated anomalies of the ossicles were suspected in 8 (12.7%) of the 63 ears. Among patients with the open type of cholesteatoma, such anomalies were found in 3 (50.0%) of 6 ears. The super structure of the stapes was destroyed in 63.5% of the patients, so the columella on the foot plate method was most frequently used for ossiculoplasty, followed by the columella on the stapes method and the Wullstein type I method.