Journal of Pediatric Neurological Disorders

Autoimmune Inner Ear Diseases

AIED occurs when the body's system attacks cells within the internal ear that are mistaken for an epidemic or bacteria. AIED may be a rare disease occurring in but 1% of the 28 million Americans with deafness. It can happen in isolation (as just labyrinthine disease) or as a part of other systemic autoimmune disorders. One-fifth of patients produce other autoimmune diseases, like atrophic arthritis or lupus.The exact clinical description can vary somewhat; however, most agree that the syndrome follows a reasonably typical course. AIED will manifest as rapidly progressing, sensorineural deafness that's most ordinarily bilateral. Typically, it begins unilaterally and ultimately affects the opposite ear, and it can occur suddenly. Word recognition is typically disproportionately poor. Vestibular symptoms can have a fluctuating course during the active phase of disease. Additionally, tinnitus, as a ringing, roaring, or hissing noise, could also be present. Diagnosis are often difficult because AIED can mimic other ear disorders. In any patient with presumed AIED, it's essential to rule out other causes of progressive sensorineural deafness, like acoustic neuroma, neurosyphilis, and Meniere's, also as other metabolic conditions. Because of the problem within the medical diagnosis of AIED, many have proposed the utilization of lab tests to help within the diagnosis. McCabe originally recommended an "immune screen" to incorporate.    

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