중이강 내에 동시에 발생한 독립된 2개의 폐쇄형 선천성 진주종 1예

Abstract

Middle ear cholesteatoma is considered to be congenital in origin when there is an intact tympanic membrane with no prior history of otorrhea, tympanic perforation or otologic procedure. However, congenital cholesteatoma is a relatively rare disease entity for which a variety of theories regarding its pathogenesis have been suggested as follows: epidermoid formation, migration of squamous epithelium, implantation, metaplasia, and acquired inclusion theory. However, the pathogenesis of congenital cholesteatoma remains unclear. There are two pathologic types of congenital cholesteatoma, “closed” and “open”. We present a rare case of congenital cholesteatoma arising from middle ear cavity in a condition of two completely isolated closed congenital cholesteatomas, which were successfully removed via transcanal approach. Also we review the literature and discuss the theories on the development of two separate congenital cholesteatomas.