[Adult localized congenital cholesteatoma].
OBJECTIVE
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.


METHODS
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.


RESULTS
The patient showed approximately 20dB conductive hearing improvement.


CONCLUSIONS
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.


CONCLUSIONS
Pathogenic hypothesis for adult forms of congenital cholesteatoma are different from infantile forms.
Mornet
E
E
Service d'ORL, hôpital Morvan, 29200 Brest, France.
Martins-Carvalho
C
C
Valette
G
G
Potard
G
G
Marianowski
R
R
D000328:Adult; D018424:Cholesteatoma, Middle Ear / Q000151:congenital*; D006309:Hearing / Q000502:physiology; D006314:Hearing Loss, Conductive / Q000209:etiology; D006801:Humans; D008297:Male; D019933:Ossicular Replacement; D014057:Tomography, X-Ray Computed