Corticosteroid therapy reverses clinical autoimmune sensorineural hearing l
oss, although little is known of how steroids restore normal auditory funct
ion. If suppression of systemic autoimmune processes underlies hearing rest
oration, then preventing autoimmune symptoms from developing should prevent
cochlear dysfunction. MRL.MpJ-Fas(lpr) autoimmune mice were used to test t
his potential mechanism by initiating oral prednisolone treatment at 6 week
s of age, prior to autoimmune disease and hearing loss onset. The steroid t
reatment group was given prednisolone in their drinking water, while untrea
ted controls were given tap water. Treatment continued for 7 months with pe
riodic evaluations of cochlear function with auditory brainstem response (A
BR) audiometry. Autoimmune mice given the steroid lived longer and did not
develop levels of serum immune complexes seen in their untreated controls.
Also, their ABR thresholds remained near normal throughout the 7 months of
treatment: while untreated controls showed progressive threshold elevations
typical for autoimmune disease. This correlation of suppressed systemic au
toimmune activity and maintenance of normal cochlear function identifies on
e potential mechanism for autoimmune hearing loss and hearing restoration w
ith steroid therapy. The autoimmune mouse should serve as a valuable model
for future studies of the cochlear mechanisms responsive to steroid treatme
nt in autoimmune hearing loss. (C) 1999 Elsevier Science B.V. All rights re
served.