Clinical study of congenital cholesteatoma of the middle ear

We studied 35 ears of 34 patients with congenital cholesteatoma who were operated between June 1992 and May 2003, focusing on the localization and progression of congenital cholesteatoma. Patients were 2 to 55 years of age. Congenital cholesteatoma was diagnosed based on two intraoperative findings: 1) no continuity between the tympanic membrane and cholesteatoma, 2) no presence of perforation, retraction, or granulation of the tympanic membrane. All patients had closed-type cholesteatoma, and none formed open-type cholesteatoma, which grows as a flat sheet of epidermis. The primary site of congenital cholesteatoma was classified into 3 types; 1) anterior-superior quadrant, 2) posterior-superior quadrant, and 3) epitympanic, and the origin of these three types of congenital cholesteatoma was difficult to explain by a single theory. We operated on 31 ears by canal wall up tympanoplasty and on 4 ears by canal wall down tympanoplasty. On all but 4 ears, we performed planned-staged operation, including second-look operations, in case of recurrence or residual cholesteatoma. Improvement in hearing after surgery was seen in 22 of the 26 ears treated and followed up for more than 6 months after surgery. By drawing all localization of congenital cholesteatoma in 35 ears, we studied the progression of congenital cholesteatoma and speculated on the original primary site. Congenital cholesteatoma in restricted areas consequently implies good results in hearing after surgery, making earlier diagnosis and treatment desirable.