Autoimmune Inner Ear Disease (AIED)

Suspect immune-mediated inner-ear disease when SNHL progresses or fluctuates over weeks to months, particularly when bilateral and asymmetric. AIED may be confined to the ear or accompany systemic inflammatory disease. It is a clinical working diagnosis after excluding alternatives, not the result of one blood test.

Hearing may improve with corticosteroids, but response is neither universal nor proof of autoimmunity. Record objective change and reassess the diagnosis and treatment burden. ^{Harris2003 Diaz-Menindez2025}

Arrange prompt otology, audiology and rheumatology assessment. New abrupt hearing loss still needs the urgent sudden-hearing-loss pathway; neurological, visual or systemic organ-threatening features change the urgency. ^{NICE NG98}