Autoimmune Diseases and the Vestibular and Oculomotor System: Clinical Presentation, Diagnosis, and Treatment

Vertigo, dizziness, and oculomotor disturbances may occur as manifestations of immune-mediated disorders affecting the inner ear, central vestibular pathways, or multisystem autoimmune disease. Although uncommon, these conditions are clinically important because delayed recognition may lead to irreversible hearing loss, vestibular dysfunction, or neurological disability. This review summarizes the clinical presentation, diagnostic approach, and treatment of immune-mediated vestibular and oculomotor disorders. We suggest a practical classification into isolated immune-mediated inner ear disease, systemic autoimmune disorders with audio-vestibular involvement, and autoimmune disorders of the central or peripheral nervous system affecting balance and eye movements. Red flags for such conditions include bilateral or progressive symptoms, fluctuating audio-vestibular deficits, associated neurological signs, and accompanied autoimmune disease. Corticosteroids remain the main first-line treatment in many of these disorders, mainly due to missing data from controlled trials. Steroid-sparing immunosuppressants, biologics, and tumor-directed therapies are effective in many cases; however, because of the missing data, they are only used in selected entities without any other choice. A structured neuro-otological and immunological workup is essential to improve diagnostic accuracy and enable timely therapy.