Primary and secondary autoimmune disorders and hearing loss

Autoimmune inner ear disease (AIED) is a rare inner ear disorder characterised by fluctuating, progressive, bilateral sensorineural hearing loss that is responsive to immunomodulatory medications. Due to a lack of widely accepted diagnostic criteria its diagnosis remains elusive. Consequently, little is known about its precise aetiology.

A novel classification system for "immune-mediated inner ear disease" (IMIED) has been proposed, which incorporates additional ear pathologies with identified immune components. This acknowledges the breadth of ear conditions that may respond to immune modulation. A range of diagnostic biomarkers are identified with varied clinical efficacy. This is appraised within the full context of a clinical setting to provide insight into practical utility. The efficacy of traditional steroid therapy is re-appraised and remains first-line therapy., The indication for steroid spring therapies is identified, and modern immunomodulatory medications are discussed.

AIED should be discussed within the spectrum of "immune-mediated inner ear disease", and therapeutic efficacy may be translated along the spectra. Diagnostic biomarkers must be appraised with caution, and diagnosis still relies on responses to steroids. Steroid sparring agents are efficacious for long-term management, and modern biologics show promise, but further investigation is needed.