Autoimmune inner ear disease

Abstract

The known etiologies of acquired sensorineural hearing loss include acoustic trauma, physical trauma, ototoxicity, genetic predisposition, infections, Meniere's disease, aging, and autoimmune disease. Treatments are directed at eliminating or managing the underlying disease process and aiding hearing with amplification. Rarely is it possible to improve unaided hearing after sensorineural loss except when the severe to profound level of loss is reached and cochlear implantation becomes an option. Autoimmune inner ear disease, however, is a treatable cause of sensorineural hearing loss and it is important for physicians and hearing health professionals to recognize that proper early diagnosis and management strategies may result in stabilization and possibly improvement in hearing.

Current understanding of the pathogenesis of autoimmune inner ear disease: a review

“…The inner ear expresses many antigens that may be targets for immune surveillance, with their recognition by innate immune cells such as neutrophils, macrophages and dendritic cells (see Table ) stimulating the release of IL‐1 β that promote an ongoing adaptive immune response where activated circulating leucocytes are drawn into the inner ear by chemotaxis and cause irreversible tissue damage . Studies have proven that the endolymphatic sac contains immunocompetent tissues capable of immune responses . Immune challenge of a sensitised animal by delivery of antigen to the inner ear results in substantial inner ear inflammation and associated hearing loss .…”

“…Autoimmune inner ear disease (AIED) usually causes bilateral, asymmetric and progressive sensorineural hearing loss (SNHL), sometimes with vestibular symptoms, that is associated with a systemic autoimmune disease in 15–30% of patients . AIED is the only form of SNHL which responds to medical therapy but delays in treatment beyond three months lead to profound and permanent hearing decline that may necessitate the provision of hearing aids or cochlear implantation . Human and animal studies have variously implicated self‐reactive T cells and antibodies, circulating immune complexes, and vasculitis in its pathogenesis .…”

Doença Autoimune do Ouvido Interno: Revisão da Literatura

“…1,4 A história clínica permite avaliar determinadas características, nomeadamente o tempo de instalação da hipoacusia, sintomas otológicos concomitantes e fatores predisponentes (exposição a ruído, quimioterapia ou tratamento prévio com antibióticos ototóxicos, cirurgia otológica prévia, história de trauma, meningite ou histó-ria familiar de surdez). 1,4,8 Por vezes, o diagnóstico diferencial com surdez súbita pode ser desafiante. Enquanto a DAIOI é frequentemente bilateral e apresenta uma progressão durante semanas a meses, a surdez súbita é geralmente unilateral e desenvolve-se num período inferior ou igual a setenta e duas horas.…”

Mobile tablet audiometry in fluctuating autoimmune ear disease

“…Secondary AIED is associated with a wide array of systemic conditions including Behçet’s, Wegener’s, Hashimoto’s thyroiditis, rheumatoid arthritis, and lupus [2, 4, 5]. …”