Understanding Autoimmune Ear disease a potentially treatable cause of Deafness

The endolymphatic sac (ELS) plays a significant role in the inner ear immune response. The sac contains a resident population of lymphocytes. 6 It has been shown that the immune response within the ear may be significantly reduced and cochlear damage decreased with the destruction of the ELS or the duct. 7. The lymphocytes responding to the antigenic stimulation in the inner ear. enter lu the systemic circulation, apparently via the spiral modiolar vein. The endothelial cells of the spiral modiolar vein undergo activation and express intracellular adhesion molecule during the secondary immune response. All four types of immune reactions described by Gell and Coombs have been hypothesized to contribute to inner ear disease. Type I immune reactions are mediated by IgE and characterized by the activation of sensitized mast cells with the release of histamine and other vasoactive substances. The type I immune response has been hypothesised as a cause of disruption of ionic transport within the inner ear due to histamine induced vasodilatation, resulting in endolymphatic hydrops.