[Congenital medially invasive cholesteatoma of the temporal bone: supralabyrinthine dissemination and possible origin in the area of the geniculate ganglion].

Congenital cholesteatoma appears in about 2% of all cholesteatomas. The possibility of deep extension producing a medially invasive petrous bone cholesteatoma is very uncommon because congenital cholesteatomas are not as aggressive as acquired ones. The onset of facial palsy in a patient with a history of several years of hearing loss must suggest this possibility. We reviewed in this report the main concepts on this subject after bibliographic review and a case report.