Autoimmune syndromes associated with hearing loss
| Condition/syndrome | Neuro-otologic syndrome | Associated findings | Epidemiology | Laboratory markers/diagnostic tests |
|---|---|---|---|---|
| ANA, antinuclear antibody; ANCA, antineutrophil cytoplasmic antibody; CRP, C reactive protein; ESR, erythrocyte sedimentation rate; MRI, magnetic resonance imaging; NSAIDs, non-steroidal anti-inflammatory drugs; SNHL, sensorineural hearing loss. | ||||
| Cogan’s syndrome | “Ménière-like”: attacks of vertigo, nausea, tinnitus, and hearing loss | Eye inflammation (keratitis, scleritis, conjunctivitis, uveitis, retinal vasculitis); systemic vasculitis in 10% | Young adults and older children (median 25 years) | Neutrophilia, raised ESR/CRP, MRI enhancement of vestibulocochlear structures |
| Wegener’s granulomatosis | Usually conductive hearing loss; often have otitis media; SNHL reported, but usually as part of mixed picture | Rhinorrhoea and sinusitis; pulmonary, renal, joint manifestations; peripheral nervous system involvement | 40 to 50 years; males and females equally affected | Raised ESR/CRP, raised ANCA (proteinase 3), granulomatous infiltration on MRI, biopsy |
| Polyarteritis nodosa | Rapid SNHL | Systemic vasculitis (kidney, gut); constitutional symptoms; mononeuritis multiplex | Male > female; older age of onset | Leucocytosis, raised ESR, visceral angiography, organ/nerve/muscle biopsy |
| Systemic lupus erythematosus | Subacute SNHL; may be subclinical (NSAIDs and antimalarials may complicate picture as both may cause SNHL) | Dermatologic, joint, renal, neuropsychiatric; constitutional symptoms | Female > male (5:1); age 15–40 years | Raised ESR, ANA, dsDNA antibodies, antiphospholipid antibodies, complement consumption |
| Sjogren’s syndrome | Often subclinical | Dry eyes, dry mouth; Raynauds and joint symptoms; neuropathies (axonal, sensory ataxic, trigeminal) | Female > male | ANA, Ro, La, Schirmer test, lip biopsy |