[Adult localized congenital cholesteatoma]

Congenital cholesteatoma is a well-described anatomical and clinical entity. Adult forms are rare. We describe a posterosuperior encapsulated cholesteatoma and compare this case to other infantile and adult forms described in the literature.

A 25-year-old patient with no medical history consulted for left conductive hearing loss. A flat tympanogram was obtained. The temporal bone computed tomographic scan showed a soft tissue density lesion of the middle ear and anterior stapes erosion. A congenital cholesteatoma was discovered during surgical exploration. The lesion was removed and the ossicular chain was reconstructed with a Shea piston.

The patient showed approximately 20dB conductive hearing improvement.

Existence of congenital cholesteatoma is well established. Adult forms are exceptional and often diffuse. A localized, encapsulated form is described in this article. 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.

Pathogenic hypothesis for adult forms of congenital cholesteatoma are different from infantile forms.