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Autoimmune Inner Ear Disease (AIED)

Bilateral progressive sensorineural loss, steroid-responsive vertigo, immunosuppressive therapy, HSP-70 antibodies

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our KBB (Kulak Burun Boğaz) department. Book Appointment →

What is Autoimmune Inner Ear Disease (AIED)?

Autoimmune inner ear disease is an immune-mediated cochleovestibular disorder presenting as bilateral, asymmetric, fluctuating or progressive sensorineural hearing loss developing over weeks to months. About one-third of patients have vestibular symptoms and one-third have systemic autoimmune disease such as rheumatoid arthritis, Cogan syndrome, or systemic lupus.

Diagnosis is clinical and based on rapid bilateral progression, exclusion of alternative causes, and response to immunosuppression. Anti-heat shock protein 70 antibodies are not specific. Magnetic resonance imaging excludes mass lesions; audiometry, electrocochleography, and vestibular testing characterize the deficit.

First-line treatment is high-dose oral or intratympanic corticosteroids for several weeks. Steroid responders may transition to maintenance methotrexate, azathioprine, or biologics. Cochlear implantation is highly effective for nonresponders or end-stage disease. Untreated AIED progresses to bilateral profound deafness within months.

Symptoms

Progressive hearing loss in both ears
Fluctuating tinnitus and aural fullness
Recurrent vertigo or imbalance
Hearing decline over weeks to months
Associated systemic autoimmune symptoms

Risk Factors

Rheumatoid arthritis or systemic lupus
Cogan syndrome
Granulomatosis with polyangiitis
Female sex (3:1 predominance)
Family history of autoimmune disease

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • When hearing drops in both ears within months
  • For sudden hearing loss with vertigo
  • For hearing loss with autoimmune disease
  • For asymmetric progressive hearing loss
  • Before considering cochlear implant

Treatment Methods

01
High-dose oral prednisone trial
02
Intratympanic dexamethasone injections
03
Methotrexate or azathioprine maintenance
04
Biologic therapy for refractory cases
05
Cochlear implantation for end-stage loss
06
Multidisciplinary rheumatology-otology care

Which Department to Visit?

You can visit our KBB (Kulak Burun Boğaz) department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

Learn About KBB (Kulak Burun Boğaz) Department

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.