Experimental autoimmune inner ear disease: an electrocochleographic and histophysiologic study

Systemic immunization with swine inner ear antigens in complete Freund's adjuvant induces functional disturbances in the cochlea. Morphometric data indicate that an endolymphatic hydrops develops within 2 weeks. It diminishes 6 weeks after immunization. A progressive decrease in the compound action potential amplitude is observed from 2 to 6 weeks after immunization. Enhancement of the amplitude of the summating potential is present without a clear overall correlation to the presence of endolymphatic hydrops. The amplitude of the cochlear microphonics shows no significant changes after immunization. Western blot analysis of the sera performed 2 and 6 weeks after immunization shows enhanced reactivity at 68, 50, 45, and 27 kd molecular weights, as compared to controls. The same spectrum of cross-reacting antibodies is believed to be instrumental in immune-mediated sensorineural hearing loss in patients. Apparently, cross-reacting antibodies and released mediators disturb cochlear homeostasis, resulting in the observed changes in the electrophysiological responses. However, these changes are not clearly related to structural changes at the light and electron microscopic levels.