유양동에 발생한 거대 선천성 진주종 1예

Congenital cholesteatoma is a rare lesion of the temporal bone. It accounts for only 2 to 5% of all cholesteatoma, though that number is throught to be on the rise due to improved imaging techniques. The diagnosis of a congenital cholesteatoma includes a pearly white mass behind an intact tympanic membrane without history of otitis media, otorrhea, or otological surgical procedure. Congenital cholesteatoma may develop in various temporal bone sites including the middle ear cavity, petrous apex, cerebellopontine angle, external auditory canal and mastoid process. The most frequent site of origin is the middle ear cavity, whereas the rarest is the mastoid. The clinical presentation is usually an symptomatic white mass behind an intact tympanic membrane. The congenital cholesteatoma that originates in the mastoid is more difficult to diagnose due to variable clinical presentation. We recently experienced a case of congenital cholesteatoma with facial palsy in 33 year old male patient who showed normal tympanic membrane without prior history of ear disease and otologic surgical procedure. We report this case with brief review of the literatures. (J Clinical Otolaryngol 2012;23:248–252)