Basic and clinical aspect of immune-mediated inner ear disorders

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A.Clinical Studies(1) The clinical features of steroid-responsive sensorineural hearing loss and acute sensorineural hearing loss with autoimmune diseases were investigated.(2) Using a RT-PCR technique, the measles virus was detected in perilymph taken during cochlear implant surgery in patients with profound hearing loss.(3) The frequency of HLA-Aw33 in otosclerosis patients was higher than that in the control subjects.B.Basic Studies(1) Secondary antigen challenge into the endolymphatic sac resulted in the cellular migration and ICAM-1 expression in the whole inner ear venules, suggesting that chemical mediators produced within the endolymphatic sac may spread into all compartments of the inner ear and lead to immunological defense and injury of the inner ear.(2) Inner ear responses to foreign cells induced activation and invasion of natural killer cells which occur relatively late compared with those in other organs.(3) Decreased immunoreactivity for Na, K-ATPase and connexin 26 was observed in the spiral ligament after immune injury.(4) Study of cell proliferation revealed that the epithelium of the endolymphatic sac can regenerate after immune injury.(5) Repeated antigen challenges into the inner ear caused an anaphylactic reaction that may disturb hearing and equilibrium.(6) MRL/prmouse and NZB mouse were studied electrophysiologically and histopathologically to clarify the mechanism by which autoimmune inner ear diseases occur.