Cochlear immunoglobulin in the C3H/lpr mouse model for autoimmune hearing loss.

Immunoglobulin staining was conducted in cochlear tissue from the C3H/lpr autoimmune strain mouse to better understand the local immune processes underlying autoimmune inner ear disease. This mouse is a model for spontaneous systemic lupus erythematosus with coincident elevated cochlear thresholds. Cochleas were examined from C3H/lpr mice at 2 months of age, before disease onset, and at 8 months of age, when systemic disease and hearing loss are manifested. Sections of these cochleas, along with cochlear sections from age-matched C3H/HeJ nonautoimmune controls, were immunocytochemically stained for IgG and IgM to identify areas of abnormal immunoglobulin activity. IgM immunoreactivity was similar in control and autoimmune cochlear tissue and did not appear to vary with disease progression. Staining was limited to the inside of capillaries in the stria vascularis and other areas within the cochlea. Similar staining patterns were seen in control animals stained for IgG. However, C3H/lpr mice with autoimmune disease showed extensive IgG immunoreactivity spreading out from the stria vascularis capillaries into the extracapillary spaces. This increased permeability suggested that breakdown of the blood labyrinth barrier was coincident with systemic autoimmune disease.