Autoimmune Inner Ear Disease (AIED)

What AIED is

The inner ear contains the cochlea, which converts sound into nerve signals, and the vestibular system, which controls balance. In AIED, immune cells or antibodies attack inner ear tissue, damaging the sensory hair cells. It accounts for well under 1% of hearing loss, but it matters because it is potentially treatable.

About 15% to 30% of people with AIED have an autoimmune disease elsewhere in the body.

Symptoms

• Hearing loss in both ears, usually beginning in one ear and then the other, developing over weeks to months. It is faster than age-related hearing loss and slower than sudden hearing loss.
• Fluctuating hearing, with good and bad days
• Tinnitus (ringing, roaring or hissing)
• A feeling of fullness in the ears
• Dizziness, imbalance or vertigo in about half of patients
• difficulty understanding speech, especially in noise

Symptoms can resemble Ménière's disease, which usually affects one ear at first. AIED often looks like bilateral Ménière's.

Associated conditions

AIED can occur on its own (primary) or with systemic autoimmune disease (secondary):

• rheumatoid arthritis
• systemic lupus erythematosus
• granulomatosis with polyangiitis, which can also cause sinus, lung and kidney disease
• Cogan syndrome, with eye inflammation and inner ear damage
• polyarteritis nodosa and other vasculitis
• ulcerative colitis and Crohn's disease
• relapsing polychondritis
• Sjögren's syndrome

Diagnosis

There is no single test for AIED. It is a diagnosis based on the pattern of hearing loss, exclusion of other causes, and response to steroids.

• Audiogram (hearing test): shows sensorineural hearing loss, often in both ears. Repeated tests over weeks show progression.
• Speech discrimination testing.
• Vestibular tests for balance involvement.
• MRI of the brain and inner ear: to rule out an acoustic neuroma, multiple sclerosis or other causes.
• Blood tests: inflammatory markers, antinuclear antibodies, rheumatoid factor, ANCA, and tests for syphilis and Lyme disease, which can mimic AIED.
• Response to steroids: improvement of hearing on high-dose steroids supports the diagnosis.

Treatment

• High-dose oral corticosteroids (such as prednisone) for about 4 weeks, followed by a slow taper, are first-line. A good response is a strong sign of AIED.
• Intratympanic steroids: injected through the eardrum when oral steroids cannot be used or as an add-on.
• Steroid-sparing medicines for those who relapse when steroids are reduced or need long-term treatment: methotrexate, biologics such as TNF inhibitors or rituximab, and others, usually with a rheumatologist.
• Treating the underlying autoimmune disease in secondary AIED.
• Hearing aids for remaining hearing loss.
• Cochlear implants for severe to profound loss in both ears, which give good results in AIED.
• Vestibular rehabilitation for balance problems.

Outlook

About half to two thirds of people respond to steroids at first, but many relapse as the dose is reduced and need longer treatment. Without treatment, hearing loss tends to progress and can become profound. Early diagnosis improves the chance of preserving useful hearing. Cochlear implants restore hearing access for those who lose most of their hearing.

Frequently asked questions

What is autoimmune inner ear disease?

AIED is a rare condition in which the immune system attacks the inner ear, causing progressive sensorineural hearing loss, usually in both ears, over weeks to months. Tinnitus, ear fullness and dizziness often occur. It is one of the few treatable causes of nerve-type hearing loss.

What are the symptoms of AIED?

The main symptom is hearing loss in both ears that worsens over weeks to months and may fluctuate. Other symptoms include ringing in the ears, a feeling of fullness, and dizziness or imbalance. Symptoms often begin in one ear before affecting the other.

How is AIED treated?

High-dose steroids are the first treatment, usually for about a month, followed by a taper. Steroid injections through the eardrum and steroid-sparing medicines such as methotrexate or biologics are used for relapses. Hearing aids and cochlear implants help with remaining hearing loss.

Can hearing loss from AIED be reversed?

Partly, in many cases. Hearing often improves with early steroid treatment, especially if started before damage becomes permanent. Some people relapse and need longer treatment. If hearing becomes severely damaged, cochlear implants can restore access to sound.

Is AIED related to other autoimmune diseases?

Sometimes. A minority of people with AIED have another autoimmune disease, such as rheumatoid arthritis, lupus, vasculitis, Cogan syndrome or inflammatory bowel disease. Many people have AIED alone. Blood tests help look for associated conditions.