Keywords: Cholesteatoma, middle ear

Abstract: Congenital cholesteatoma of the middle ear is a Tare entity that can occur in other sites in the cranial cavity: cerebellopontine angle, chiasma region, diploe of the skull, and rhomboid fossa. It is estimated to represent 0,2% to 1,5% of all intracranial tumors. Various pathogenetic mechanisms have been theorized, the hypothesis that Congenital cholesteatoma arises from a normally ocurring epidermoid cell rest in lhe developing middle ear, is the generally most accepted theory. Early diagnoses of lhes lesion, before the age of 3 years, during which time the cholesteatoma is confrned to the anterosuperior segment of lhe middle ear, is strongly advocated The autorrs reportseven cases of Cngnital cholesteatoma of the middle ear and ãe anatomic and functional results aftersurgery, as well as their follow-up.