Congenital cholesteatoma assessment based on staging and classification criteria for middle ear cholesteatoma proposed by the Japan Otological Society.

Objective: We aimed to assess the clinical characteristics of extent patterns in congenital cholesteatoma, based on the Japan Otological Society (JOS) staging system.

Methods: This was a retrospective chart review that included 80 ears of 80 patients with congenital cholesteatoma who underwent primary surgery at a tertiary academic medical center. The main characteristics and outcomes reviewed were sex, age, clinical background, surgical method, and stage classification according to two staging classifications: the criteria advocated by JOS and Potsic staging system.

Results: The age at the time of surgery ranged from 1 to 35 years (average 8.4 years), and there were 54 men and 26 women. According to the JOS staging system, 12 ears were Stage Ia (15%), 7 ears were Stage Ib (9%), 1 ear was Stage Ic (1%), 59 ears were Stage II (74%), and 1 ear was Stage III (1%). In the study of postoperative residual recurrence, there were 4 cases after the primary operation and 3 cases after the staged operation. All 3 ears with residual disease after planned surgery were cholesteatomas that extended to all the tympanomastoid space.

Conclusion: We consider the JOS staging system to be more suitable, in terms of anatomical classification and surgical procedure selection for comparison between Europe, the United States, and Asia. Specifically, it was advantageous that the PTAM classification and the S classification are associated with surgical procedure selection and postoperative course.