Management
Treatment & prognosis
Two goals: suppress the immune attack to halt or reverse the damage, and support hearing and balance while it settles. The earlier the first goal is met, the more there is to save.
Corticosteroids — first line
The first treatment is steroids, which calm the immune attack. Given early, they can stabilise or even improve hearing. They can be taken as tablets or, in some cases, injected through the eardrum to act locally with fewer whole-body side effects.
Systemic corticosteroids are the mainstay: prednisone ~60 mg/day, with response rates of 50–70% when started early, gauged by hearing, vertigo and audiometric thresholds, then tapered over 4–6 weeks.1 Intratympanic corticosteroid (dexamethasone 4 mg/mL) gives high local concentration with low systemic toxicity — useful for partial responders, unilateral disease, or when systemic steroids are contraindicated (e.g. diabetes, osteoporosis).2
The steroid trial doubles as a diagnostic test: a clear response supports the diagnosis and identifies who will benefit from ongoing immunomodulation. Non-response, or steroid-dependence, is the trigger to escalate to steroid-sparing therapy rather than to persist with high-dose steroids and their toxicity.1
Steroid-sparing agents & biologics
For steroid-dependent, refractory or steroid-intolerant patients, methotrexate is the best-studied steroid-sparing agent (weekly low dose), maintaining remission and reducing steroid exposure.3 Azathioprine and cyclophosphamide are reserved for severe or systemic vasculitic disease, the latter for aggressive cases given its toxicity. Biologics — TNF-α inhibitors (etanercept, infliximab) and, in refractory cases, IL-1/IL-6 inhibitors (anakinra, tocilizumab) — are promising but evidence is limited and largely from coexisting systemic autoimmune disease.4,5
Symptomatic & rehabilitative care
During acute vertigo, vestibular suppressants (meclizine, betahistine, short-course benzodiazepines) relieve symptoms but should be brief, to avoid impeding central compensation. For persistent imbalance, vestibular rehabilitation improves balance, reduces falls and restores confidence.6 For irreversible hearing loss, hearing aids help milder loss and cochlear implantation is indicated for bilateral severe-to-profound SNHL, with generally favourable outcomes once disease is stable and the cochlea is patent.7
Prognosis
Outcome tracks the timing of treatment. Early intervention preserves function; delay allows irreversible loss. Hearing improvement is more likely than vestibular recovery, but rehabilitation mitigates imbalance even when physiological recovery is incomplete. Relapse is common — up to ~40% — so long-term audiovestibular monitoring and follow-up are essential.
Emerging directions
The field is moving toward precision: characterising inner-ear autoantigens (cochlin, β-tectorin), multiplex immunoassays to detect activity, targeted biologics (IL-1/IL-6 inhibitors),8 gene-expression profiling for personalised therapy, and — most ambitiously — regenerative approaches generating hair cells from pluripotent stem cells.9 The goal is to shift AIED care from non-specific suppression toward disease modification and, eventually, repair.
Key points
- Corticosteroids are first-line (≈60 mg/day prednisone, 50–70% respond); intratympanic steroid is an alternative/adjunct.
- The steroid response is both treatment and a diagnostic clue.
- Escalate to methotrexate (steroid-sparing) or, in severe/refractory disease, other immunosuppressants and biologics.
- Support with brief vestibular suppressants, rehabilitation, hearing aids and cochlear implantation.
- Early treatment preserves function; relapse (~40%) demands long-term monitoring.