The disease

Systemic autoimmune disease & the inner ear

AIED can be organ-specific — or the inner ear can be one front in a wider autoimmune war. Knowing which systemic diseases strike the labyrinth turns an unexplained vertigo into a diagnosis.

How systemic disease reaches the inner ear

Beyond isolated AIED, several systemic autoimmune and vasculitic diseases involve the inner ear — by direct immune injury, small-vessel vasculitis, microthrombosis, or granulomatous infiltration. Vertigo in these conditions is often under-recognised when overshadowed by the systemic picture, yet spotting it is what enables timely treatment.1 They share mechanisms — autoantibodies, T-cell inflammation and small-vessel vasculitis — with primary AIED.

The six to know

Select a disease to see its inner-ear mechanism, audiovestibular picture, the systemic clue that points to it, and how it is treated. The audiovestibular features often mimic Ménière’s disease; the systemic clue is the giveaway.

Cogan's syndrome

Mechanism
Autoimmune vasculitis of cochleovestibular vessels
Audiovestibular
Sudden bilateral SNHL, vertigo, tinnitus — Ménière-like but bilateral and progressive
Clue
Interstitial keratitis (red, painful eyes) + audiovestibular disease in a young adult; may have aortitis
Treatment
Early corticosteroids + immunosuppressants
These diseases reach the inner ear by direct immune injury, small-vessel vasculitis, microthrombosis or granulomatous infiltration. The audiovestibular picture often mimics Ménière’s — the systemic clue is what gives them away.

Two not to miss

Cogan’s syndrome pairs interstitial keratitis (red, painful eyes) with sudden audiovestibular disease in a young adult, and can carry life-threatening large-vessel vasculitis such as aortitis — early immunosuppression protects both hearing and heart.2 Granulomatosis with polyangiitis can destroy the otic capsule and temporal bone if untreated; positive ANCA serology and airway/renal disease point to it, and it needs glucocorticoids with cyclophosphamide or rituximab.3

Antiphospholipid syndrome deserves a special mention because its mechanism is thrombotic rather than inflammatory — recurrent or sudden hearing loss and vertigo from microthrombosis of the terminal labyrinthine circulation — and its treatment is anticoagulation, not immunosuppression.

Key points

  • The inner ear can be involved in Cogan’s, SLE, GPA, APS, RA and Behçet’s disease.
  • Mechanisms span vasculitis, immune complexes, microthrombosis and granulomatous infiltration.
  • Audiovestibular features often mimic Ménière’s — the systemic clue distinguishes them.
  • Cogan’s (with keratitis ± aortitis) and GPA (ANCA, bony destruction) must not be missed.
  • Antiphospholipid syndrome is treated with anticoagulation, not immunosuppression.