Learning Objectives:

Objectives: We investigated the clinical features and surgical results of congenital cholesteatoma according to Potsic's staging system. Potsic proposed a classification system comprising four stages to evaluate the extent of disease as follows: I, disease confined to a single quadrant; II, cholesteatoma in multiple quadrants, but without ossicular involvement or mastoid extension; III, ossicular involvement without mastoid extension; and IV, mastoid disease.

Methods: A total of 87 patients who had undergone surgery at our hospital were retrospectively analyzed for presenting symptoms, the location of cholesteatoma, and surgical results according to Potsic's staging system.

Results: Of the 87 patients, 25 were classified as Potsic stage I, 13 as stage II, 35 as stage III, and 14 as stage IV. More than half of the patients with early-stage congenital cholesteatoma (stages I and II) were diagnosed asymptomatically by a chance visit to a clinic or on ear screening. Others were diagnosed following a complaint of hearing loss, acute otitis media, or otitis media with effusion. The location of congenital cholesteatoma varied somewhat by stage. In stage I congenital cholesteatoma, the most frequent location was behind the anterior-superior quadrant of the tympanic membrane; however, in stage III congenital cholesteatoma, it was behind the posterior-superior quadrant. All patients were treated surgically. Recurrence was detected in 11 of the 87 patients (12.7%). Recurrent lesions were removed during revision surgery.

Conclusions: Potsic's staging system is a clinically useful procedure for evaluating the extent of congenital cholesteatoma. As the classification is simple, and the stage is easily determined based on otoscopic and CT findings.