Autoimmune Inner Ear Disease.ppt

Autoimmune Inner Ear Disease Introduction Inner ear is subject to the influences of immune response , inflammation, and autoimmunity, and hearing loss can occur If detected early enough, hearing loss can be restored through appropriate use of immunosuppressive drugs The inner ear immune response The CNS and the inner ear originally were thought to be immunoprivileged sites through the actions of the blood-brain barrier and blood-labyrinthine barrier, respectively. Immunoglobulin crosses the blood-labyrinthine barrier and is present in perilymph at a level of 1/1000 the concentration found in serum. Levels of IgG appear to predominate with lesser amounts of IgM and IgA present. Endolymphatic sac endolymphatic sac: 1. antigen access 2.initial site of the immune response. Horseradish peroxidase (HRP), does diffuse to the endolymphatic sac after scala tympani injection. (perilymph ? perisaccular tissue ? endolymphatic space) KLH Keyhole limpet hemocyanin (KLH), a potent stimulator of cellular immunity, was used to investigate the development of inner ear immunocompetent cells. Both macrophages with phagocytosed antigen and T-helper cells appeared in the endolymphatic sac before they appeared in the cochlea. The spiral modiolar vein The spiral modiolar vein (SMV) appears to play a key role in the cellular infiltration Egress of lymphocytes from the circulation into lymph nodes has long been known to occur at specialized postcapillary venules They have a unique morphology and histochemistry, earning them the name high endothelial venules (HEVs). SMV undergoes an HEV-like transformation during the inflammatoryresponse. A steady increase in extracellular matrix. Both suppurative and sterile labyrinthitis are known to result in the formation of a dense extracellular matrix and eventual ossification in the cochlea. Fibroblast and endosteal cells lining the scala tympani are proliferating The inner ear seems particularly incapable of clearing this extrac