title: [Primary sclerosing cholangitis and autoimmune cochlear deafness: a new syndrome?].
abstract: Association of autoimmune cochlear hearing loss with primary sclerosing cholangitis is reported in two patients. Endocochlear sensorineural hearing loss was associated with the presence of anti-cochlear antibodies in the serum directed against the walls of vessels in the stria vascularis. The hearing loss appeared at the same time or shortly after the diagnosis of cholangitis. This association, which has never been described, may reinforce the theory of the role of immunologic factors in the pathogenesis of primary sclerosing cholangitis, possibly linked to or initiated by vasculitis.
authors.affiliation: Clinique d'Hépato-Gastroentérologie, CHRU, Grenoble.
authors.forename: M
authors.initials: M
authors.lastname: Cohard
authors.forename: A
authors.initials: A
authors.lastname: Rolachon
authors.forename: D
authors.initials: D
authors.lastname: Vetter
authors.forename: J M
authors.initials: JM
authors.lastname: Lejeune
authors.forename: J P
authors.initials: JP
authors.lastname: Stierle
authors.forename: S
authors.initials: S
authors.lastname: Fratte
authors.forename: M
authors.initials: M
authors.lastname: Doffoël
authors.forename: R
authors.initials: R
authors.lastname: Charachon
authors.forename: J P
authors.initials: JP
authors.lastname: Zarski
mesh_terms: D000328:Adult; D015209:Cholangitis, Sclerosing / Q000150:complications* / Q000209:etiology; D003051:Cochlea / Q000276:immunology*; D015834:Cochlear Diseases / Q000150:complications* / Q000276:immunology; D003638:Deafness / Q000209:etiology* / Q000276:immunology; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000209:etiology* / Q000276:immunology; D006801:Humans; D008297:Male; D008856:Microscopy, Fluorescence; D013577:Syndrome; D014657:Vasculitis / Q000150:complications
vernacular_title: Cholangite sclérosante primitive et surdité endocochléaire auto-immune: nouveau syndrome?