Autoimmune Inner Ear Disease

Abstract

Autoimmune inner ear disease (AIED), first reported by McCabe in 1979, describes a disease process in which cochleovestibular is compromised by one's own immune system. Only indirect laboratory evidence exists regarding the underlying immune reaction, which can only be confirmed histopathologically in postmortem studies. Diagnosis involves a thorough history and complete head and neck, otomicroscopic, and audiometric evaluation. AIED classically presents with bilateral, fluctuating, or rapidly progressive asymmetric sensorineural hearing loss (SNHL), which typically occurs over weeks to months but can occur suddenly over a period of a few hours or days. Fifty percent of patients have vestibular symptoms as well, which can be unilateral or bilateral. A lengthy serological workup is unwarranted given the absence of a reliable diagnostic laboratory test. Antibodies to the 68-kDa protein (heat shock protein-70) is marker-specific but not sensitive for AIED and may correlate with steroid responsiveness. In cases where the diagnosis remains unclear, a prolonged course of steroids, with repeat audiometric testing at 1 month, can be undertaken. Treatment aims to inhibit the detrimental immune response using immunosuppressant drugs; however, no standardized treatment regimen has been found. Cochlear implantation remains a viable last resort for patients with progressive SNHL.KEYWORDS: Sensorineural hearing loss, autoimmune inner ear disease, endolymphatic hydrops, dizziness Learning Outcomes: As a result of this activity, the participant will be able to (1) list how to diagnose autoimmune inner ear disease and (2) describe how to treat autoimmune inner ear disease.

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

“…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 . Corticosteroids remain the sole proven treatment but only 70% of patients respond to therapy with this benefit curtailing over time . Additionally, the harmful side‐effect profile of Corticosteroids makes long‐term usage impractical.…”

Κοχλιακές Εκδηλώσεις Σε Ασθενείς Με Αυνοάνοσα Ρευματολογικά Νοσήματα

“…ανίχνευση αυτοαντισωμάτων, (δ) ανίχνευση αντισωμάτων έναντι αλλεργιογόνων και (ε) ανίχνευση αντισωμάτων έναντι αντιγόνων, της στην περίπτωση της λεισμανίασης(151,152,153,154). και εμβοές, ενώ μπορεί να σχετίζεται με άλλο συστηματικό αυτοάνοσο νόσημα σε ποσοστό 15-30% των περιπτώσεων(156,157). Είναι ο μόνος τύπος νευροαισθητηριακής βαρηκοΐας που κλινικά, βελτιώνεται άμεσα μετά από χορήγηση φαρμακευτικής αγωγή(158), όπως τα κορτικοστεροειδή, τα οποία είναι αποτελεσματικά χωρίς υποτροπή των Διαφορική διάγνωση Αυτοάνοσης νόσου του έσω ωτός Η πρόγνωση και οι μακροπρόθεσμες συνέπειες στην ακοή των ασθενών, εξαρτάται από της πρώιμη διάγνωση και έγκαιρη θεραπεία.…”