SULFOGLUCURONOSYL GLYCOLIPIDS AS PUTATIVE ANTIGENS FOR AUTOIMMUNE INNER-EAR DISEASE

Autoimmune inner ear disease is diagnosed based on clinical history of fluctuating but progressive sensorineural hearing loss (SNHL) with or without vestibular symptoms occurring over weeks to months. An initia l response to steroids or immunosuppressive drugs usually reverses the hearing loss. In search of specific diagnostic and therapeutic marker s for autoimmune inner ear diseases, we investigated serum anti-glycol ipid antibody activities in these patients by two different methods, H PTLC-immunoblotting and ELISA. We found that 37 out of 74 patients of clinically diagnosed autoimmune inner ear disease (30 of sensorineural hearing loss (SNHL) (group I), 14 of vestibular symptoms only (group II), 30 of Menieres symptoms (with both hearing loss and vestibular sy mptoms) (group III)) showed positive anti-sulfoglucuronosyl lactosamin yl paragloboside (SGLPG) antibody titers (p <0.001). On the other hand , anti-sulfoglucuronosyl paragloboside (SGPG) titers were not elevated in these conditions. In contrast, only 3 out of 56 pathological contr ol and 2 out of 28 healthy volunteers had measurable anti-SGLPG antibo dy titers. We further analyzed the localization of SGLPG in the audito ry pathway and found that the antigens existed exclusively in inner ea r and the eighth nerve, but not in pens, cerebellum, nor cerebrum. We conclude that the anti-SGLPG antibody represents a novel diagnostic ma rker for autoimmune inner ear disease and may participate in the patho genesis of this disease. (C) 1998 Published by Elsevier Science B.V.