Autoimmune inner ear disease (AIED)

Rapidly progressive hearing loss and/or dizziness caused by antibodies attacking the inner ear, usually occurring suddenly and beginning in one ear before spreading to both. Hearing decline over the course of a few months is often accompanied by tinnitus. Irreversible hearing loss may be prevented if caught early enough but AIED can be difficult to diagnose.

Dizziness, tinnitus, hearing loss, ear fullness, fever, malaise, fatigue, vertigo, and difficulty with recognizing words in conversation.

Tinnitus, rheumatoid arthritis, Cogan’s syndrome, relapsing polychondritis, systemic lupus erythematosus, Wegener’s granulomatosis, polyarteritis nodosa, Sjogren’s syndrome, and Lyme disease.

Very few risk factors are known about AIED. Middle-aged females tend to be the most affected group and having other autoimmune conditions increases risk.

• Secondary autoimmune immune ear disease (AIED): a systematic review and meta-analysis on vestibular manifestations of systemic autoimmune and inflammatory disorders (2023)
• Role of Inner Ear Macrophages and Autoimmune/Autoinflammatory Mechanisms in the Pathophysiology of Inner Ear Disease (2022)
• Sex differences in autoimmune inner ear disease treatment and audiological outcomes (2023)
• Disease-Modifying Antirheumatic Drugs in the Treatment of Autoimmune Inner Ear Disease: A Systematic Review and Meta-Analysis of Auditory and Vestibular Outcomes (2023)
• Prevalence of immune-mediated inner ear disease in non-segmental vitiligo: A cross-sectional study (2023)