The non-classical presentation of congenital cholesteatoma: A case report

<p>Sri Wartati and Yarni Alimah</p>

The non-classical presentation of congenital cholesteatoma: A case report

Sri Wartati and Yarni Alimah

Hasanuddin University, Indonesia

: J Otol Rhinol

Abstract

Congenital cholesteatoma is a well-described anatomical and clinical entity. Adult forms are exceptional and rare but often diffuse. We describe a mastoid congenital cholesteatoma and compare this case to other infantile and adult forms described in the literature. We present a non-classical presentation of congenital cholesteatoma with post aural fistula in the right ear, intact tympanic membrane and normal hearing in a 23 year-old female. The temporal bone CT-scan showed a soft tissue density lesion of the middle ear with erosion in mastoid part. After investigation the patient underwent surgical management resulting in improvement of her symptoms. Existence of congenital cholesteatoma is well established. An extensive form is described in this case. Apparently, there are two types of congenital middle ear cholesteatoma; a closed keratotic cyst and an open matrix. Patient with open cholesteatoma may have a clinical presentation that is uniquely different from the classical description of congenital cholesteatoma. The specificity remains unknown. It is uncertain whether the adult and infantile forms have the same origin. A multifactorial or metaplastic mechanism could explain adult congenital cholesteatoma.