Autoimmune inflammation of the inner ear may occur in isolation as an organ-specific process or may be part of a systemic autoimmune syndrome (20). Evidence is mounting to support the existence of an organ-specific cochlear autoimmune disease. The presence of antibodies to cochlear antigens, such as the 68 kD inner-ear antigen, as well as an impressive response to immunosuppressive medications, implicate autoimmunity as the underlying pathologic process in some patients with "idiopathic" sensorineural hearing loss. These patients with autoimmune inner-ear disease (more accurately termed immune-mediated inner-ear disease, since proof for autoimmunity is still lacking) have a fluctuating progressive sensorineural hearing loss, often associated with vertigo and tinnitus. Generally, hearing loss progresses rapidly, within a period of weeks and months, to an irreversible end-stage disease. While at any given moment, one ear may be more significantly affected, the process eventually affects both ears.
Cogan's syndrome (Chapter 6) is a rare disease characterized by nonsyphilitic interstitial keratitis associated with vertigo, tinnitus, and hearing loss (21). If the same labyrinthine complaints are associated with other forms of ocular inflammation (e.g., uveitis and episcleritis), the condition is known as atypical Cogan's disease. The auditory and vestibular dysfunction resembles that seen in Meniere's disease and, untreated, progresses to profound deafness within weeks or months. The interval between ocular and otologic disease varies from a few weeks to a year; either organ may be affected first. The etiology of Cogan's syndrome is unknown. While the relatively focal inflammation suggests organ-specific autoimmunity, this has not been proven. Like other vasculitides, the disease may represent a hypersensitive immune reaction to a viral infection.
The systemic vasculitides may involve the labyrinth. Wegener's granulomatosis (Chapter 8) is a systemic vasculitis of medium and small blood vessels resulting in a triad of necrotizing granulomas of the upper airway, necrotizing glomerulonephritis, and systemic necrotizing angiitis. Chronic otitis media is the most common otologic manifestation of Wegener's, but sensorineural hearing loss will occur in a significant number of patients. Immune-mediated inner-ear disease can also be a component of polyarteritis nodosa (PAN). Postmortem temporal bone histopathologic studies on patients with PAN have demonstrated vasculitic changes within the labyrinth. Systemic lupus erythematosus, rheumatoid arthritis, Sjogren's syndrome, and relapsing polychondritis have also been associated with cases of sudden hearing loss and histopathologic evidence of labyrinthitis.
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