Immune-mediated inner ear disease

Abstract

The incidence of autoimmune inner ear disease (AIED) is difficult to determine: probably it is a rare disease, accounting for <1% of all cases of hearing impairment or dizziness. Nevertheless, the diagnosis of AIED might be overlooked because of the lack of a specific diagnostic test. The hallmark of this clinically diagnosed condition is the presence of a rapidly progressive, often fluctuating, bilateral sensorineural hearing loss (SNHL) over a period of weeks to months. The progression of hearing loss is too rapid to be diagnostic for presbycusis and too slow to conclude a diagnosis of sudden SNHL. Vestibular symptoms, such as generalized imbalance, ataxia, positional vertigo and episodic vertigo may be present in almost 50% of patients. Occasionally only one ear is affected initially, but bilateral hearing loss occurs in most patients, with symmetric or asymmetric audiometric thresholds. Almost 25-50% of patients also have tinnitus and aural fullness, which can fluctuate. Systemic autoimmune diseases coexist in 15-30% of patients.

Cogan’s Syndrome in a Jordanian patient: A case report

“…Sometimes, there is an elevation in the level of C-reactive protein (CRP), but often no alteration in inflammation parameters is observed, therefore, no specific diagnostic test is performed. 2 The results of radiographic studies of CS cases, such as cranial CT and MRI, are often normal; which could be useful in the exclusion of other causes of vestibuloauditory symptoms such as stroke or neoplasm. 5 CS is a cause of progressive deafness; it is assumed to be an ''autoimmune disease''.…”

Sensorineural hearing loss in pediatric celiac patients

“…Type II and III immunological reactions would be causing inner ear pathology together in pediatric CD patients [28,29]. In type II reaction, an immunoglobulin directed against a tissue or organ is basic mechanism for injury of endolymphatic sac as in the pathogenesis of Meniere's disease and in type III reaction an immune complex deposition leads to inner ear pathology as in the pathogenesis of Wegeners granulomatosis [24].…”

Activation of TRAIL‐DR5 pathway promotes sensorineural degeneration in the inner ear

“…Many systemic autoimmune diseases are associated with hearing loss (Bovo et al ., 2006) while patients with seemingly isolated sensorineural hearing loss can have autoantibodies against inner ear antigens (Greco et al ., 2011). A comprehensive bioinformatic analysis has revealed that inner ear proteins share sequence similarity with many known immunogenic proteins, which may lead to cross‐reactivity and detrimental immune activation in the inner ear (Platt et al ., 2014).…”