PRIMARY SCLEROSING CHOLANGITIS AND AUTOIMMUNE COCHLEAR HEARING-LOSS -A NEW SYNDROME

Association of autoimmune cochlear hearing loss with primary sclerosin g cholangitis is reported in two patients. Endocochlear sensorineural hearing loss was associated with the presence of anticochlear antibodi es in the serum directed against the walls of vessels in the stria vas cularis. The hearing loss appeared at the same time or shortly after t he diagnosis of cholangitis. This association, which has never been de scribed, may reinforce the theory of the role of immunologic factors i n the pathogenesis of primary sclerosing cholangitis, possibly linked to or initiated by vasculitis.