Autoimmune disease and cochlear pathology in the C3H/lpr strain mouse

The C3H/lpr autoimmune strain mouse is a model for spontaneous systemic lupus erythematosus. Inner ear structure and function were examined during systemic autoimmune disease progression to identify correlated auditory system pathology. Onset of the systemic disease occurred at 2-3 months of age and was characterized by elevated serum immune complexes, cryoglobulins, and antinuclear antibodies. Coincident with the onset of autoimmune disease was degeneration of the stria vascularis. Early edema of the stria occurred in the apex and progressed basalward with duration of the disease. By 10 months of age, stria vascularis area was smaller and auditory brainstem response thresholds were elevated. No degeneration of hair cells was seen at any age, suggesting that the stria vascularis may be the primary anatomic site of autoimmune auditory damage in this mouse model.