Surgery for congenital cholesteatoma; Therapeutic strategy in the endoscopic era

Abstract

Most Congenital cholesteatoma (CC) which is associated with well-pneumatized temporal bone and good Eustachian tube function occurs in the mesotympanum. Therefore, CC is considered to be a good indication for transcanal endoscopic ear surgery (TEES) with preservation of the normal anatomy (i.e., the mastoid air cells). In this report, we present our CC cases treated by TEES and microscopic ear surgery (MES), and describe our therapeutic strategy for CC. Totally, we studied 29 ears of 28 patients of 19 male and 9 female patients who were operated on in our department. The age at operation raged from 1 to 13 years (median age, 3 years). The average follow-up period was 40.6 months. Of the 29 ears, 23 were operated on by TEES, and four of 23 ears underwent staged surgery. TEES group included 14 ears of stage Ia, one ear of stage Ib, three ears of stage Ic and five ears of stage II. Type I tympanoplasty for 18 ears, type III for three ears and type IV for two ears were performed. On the other hand, six ears were operated on by MES, of which all of six ears classified to stage II. All six ears underwent staged surgery (canal wall-up tympanoplasty). The postoperative hearing evaluation showed that 21 of 22 ears in TESS group were “success” by judging from the Criteria by Japan Otological Society 2010. In MES group, 5 of 6 ears showed “success.” No major complications occurred during intraoperative period. No recurrence developed among either the TEES or the MES group. Our experience suggested that CCs of stage I should be operated on by TEES and stage II CCs without extension into mastoid can also be operated using TEES. Introduction of TESS into CC will be possible to perform effective, safe and minimally invasive tympanoplasty.