Scleroderma and sudden sensorineural hearing loss

Abstract

Sudden sensorineural hearing loss (SNHL) is an emergency in otolaryngological practice. The exact cause, in most of the cases cannot be found. A triad of vascular, viral, and membrane rupture are the known theories for the causation of sudden SNHL. Now various autoimmune disorders have also been suggested to play a role in the pathogenesis. Autoimmune inner ear disease in human being is an uncommon, but a distinct clinical entity. The diagnosis of the inner ear autoimmune disorder depends mainly on relatively distinct clinical course, immune laboratory tests and treatment response. The clinical picture usually consists of bilateral SNHL, bilateral reduced vestibular loss and symptoms of pressure with tinnitus and rarely tissue destruction of middle and external ear. Systemic scleroderma is an aggressive multi-system and multistage connective tissue disorder with high mortality. It is characterized by proliferative vascular lesion and marked deposition of collagen in various tissues and organs. Responses to immunosuppressive drugs are cited as evidence of an autoimmune process.