Clinical Features and Natural Course of Anteriorly Located Congenital Cholesteatoma

Abstract

Objectives: Anteriorly located congenital cholesteatoma is a silent disease in the pediatric population. In most cases, they are diagnosed incidentally by endoscopic examination of tympanic membrane. The aims of this study were to evaluate the clinical features and natural courses of congenital cholesteatoma, especially anteriorly located group. Methods: From 2000 to 2012, 117 patients were diagnosed with congenital cholesteatoma. Retrospective chart review was done. Among them, 65 cases were anteriorly located congenital cholesteatoma. A total of 53 patients were exclusively located in the anterosuperior quadrant. Other cases were extended to posteriorly (n = 4) or inferiorly (n = 8). Age distribution, types (closed or open), volumes (measured by tetrahedron beam computed tomography), ossicular involvement, and possible origin of cholesteatoma were assessed. Results: Mean age was 3.07 years (1-6). Patients older than 6 years were not encountered. Closed type was 61.5% (n = 40) and open type was 38.5% (n = 25). Volume of cholesteatoma was 59.38 ± 6.53 mm3 (closed type: 33.02, open type: 98.44). The incidence of open type and volume increased as age increased. Hearing loss was found in 5 cases. Ossicular involvement was found in 4 cases. During surgery, in many cases, the attachment of cholesteatoma to anterior side of cochleariform process was found by endoscopic examination. Conclusions: Anteriorly located congenital cholesteatoma seems to originate from the anterior side of the cochleariform process. Its size grows with age and tends to rupture into open type. In addition, it might be washed out through E-tube as patients get older.