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.

Sensorineural hearing loss - autoimmune inner ear disease - endolymphatic hydrops - dizziness

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