후천성 중이 진주종의 선천성 원인의 가능성에 대한 임상분석

Background and Objectives: Congenital cholesteatoma of the middle ear was a rare clinical entity that classically presents as a pearly epidermal cyst seen behind the normal tympanic membrane. In recent years, there has been an increasing number of case reports and an apparent increase in congenital cholesteatoma in the middle ear. The actual incidence of congenital cholesteatoma is substantially greater than the literature indicates. Several otologists have speculated on the pathogenesis of acquired cholesteatoma related to congenital origin. The issue may further be complicated by extensive cases of congenital cholesteatoma that become infected and eventually show perforation of the tympanic membrane and, therefore, those are classified as acquired cholesteatomas. The aim of this study is to analyse suspicious congenital choleteatomas and compare them with typical congenital cases, and to determine whether congenital cholesteatomas can be a source of acquired cholesteatoma.

Materials and Methods: We reviewed 11 cases of middle ear cholesteatoma including 8 typical congenital cholesteatoma by analysing patients’ history, preoperative myringoscopic findings, temporal bone computerized tomographies and operative findings.

Results: The diagnostic evidences which congenital cholesteatomas can cause acquired cholesteatomas are : (1) shorter duration of otorrhea than that of hearing impairment ; (2) relatively smaller perforation of the ear drum especially in the pars tensa area.; (3) relatively good pneumatization of the temporal bone ; (4) cholesteatoma matrices involved in mesotympanum ; and (5) much more damage of ossicular structures in the mesotympanum.

Conclusion: This review supports that some middle ear cholesteatoma may have the possibility of congenital origin.