Congenital cholesteatoma involving the Eustachian tube

Abstract Cholesteatomas, classified as acquired or congenital, are non-neoplastic, keratinizing squamous epithelial lesions that can affect many different areas of the temporal bone. We present a case of a six-month-old female with chronic otorrhea who was found to have a congenital cholesteatoma originating from the Eustachian tube. Chronic pressure from cholesteatoma expansion turned the Eustachian tube into a large cavity approximately double the size of the middle ear space. This case was unusual not only in the location of the cholesteatoma but also in respect to the unusual presentation of chronic middle ear infection due to Eustachian tube obstruction.