This study was designed to investigate the role of immunologic mechanisms in sensorineural hearing disorders and the relationship between inner ear and renal disorders. Autoimmune strain (NZB/kl) mice, in which autoimmune disease can be spontaneously induced, were used in this study. The mice were tested for acoustic brain stem responses, cochlear and renal disorders, and circulating immune complex serology by means of enzyme immunoassay. Pathologic studies consisted of observation of tissue changes following hematoxylin and eosin staining and indirect immunofluorescence staining under light microscopy. Compared to controls, the hearing was impaired in NZB/kl mice. Indirect fluorescence staining showed immunoglobulin G deposits in the stria vascularis. There was a correlation between the degree of hearing impairment and the severity of stria vascularis lesions. The NZB/kl mouse can provide a model for sensorineural hearing disorders secondary to immunologic disorders.