Autoimmune Inner Ear Disease (AIED)

Autoimmune inner ear disease (AIED) is a rare inflammatory condition that happens when your immune system attacks your inner ear. Your inner ear manages hearing and balance. This disease causes hearing loss in one ear or both ears that quickly gets worse. It also affects your sense of balance.

We don’t always know why someone has AIED, but certain autoimmune disorders increase the chance of having it. Corticosteroids and other anti-inflammatory medications can improve and often temporarily restore your hearing and sense of balance. But people with hearing loss that doesn’t improve may need hearing aids or cochlear implants.

Types of this condition

Autoimmune inner ear disease can be primary or secondary:

• Primary AIED: In this type, your immune system mistakes inner ear cells for bacteria or viruses and sends antibodies in to destroy the cells. Experts aren’t sure what triggers this reaction. In this case, your inner ear is the only organ affected.
• Secondary AIED: This type happens when you have a systemic autoimmune disorder that affects your inner ear. About 30% of people with AIED have an autoimmune disorder affecting the rest of their bodies.

Symptoms of autoimmune inner ear disease

The most noticeable and serious symptom is hearing loss that quickly becomes worse. Many people have hearing loss in one ear first, then in the other. The change may develop within a few days to a few months. It may feel like your ability to hear comes and goes.

Other symptoms may include:

• Dizziness
• Ear fullness
• Tinnitus (ringing in the ears)
• Vertigo

Condition causes

Autoimmune inner ear disease happens because your immune system mistakenly attacks your inner ear. The attack causes inflammation that damages blood vessels and special hair cells in your inner ear. These special cells convert sound vibrations into electrical signals. Your auditory nerve forwards those signals to your brain. Your brain turns the signals into sound. AIED disrupts this process.

AIED can happen for no reason. But people with certain autoimmune disorders may also develop AIED. Autoimmune disorders that may cause this disease include:

• Cogan syndrome: This rare disorder causes inflammation in your ears and eyes.
• Granulomatosis with polyangiitis: This is chronic inflammation in your smaller blood vessels.
• Lupus: Your immune system causes inflammation throughout your body.
• Relapsing polychondritis: This condition affects cartilage in your ears, nose and other parts of your body.
• Sarcoidosis: Your immune system overreacts and makes lumps of white blood cells in different parts of your body.
• Sjögren’s syndrome: This condition affects glands that produce moisture, causing dry mouth and dry eyes.
• Rheumatoid arthritis: In this condition, you have inflammation in the tissue that lines your joints.

How doctors diagnose this condition

A healthcare provider may do blood tests, hearing tests and MRIs to diagnose AIED. Often, they’ll start treatment to see if it helps before they make a final diagnosis. Blood tests may include:

• Antibody test
• Antinuclear antibody
• C-reactive protein
• Sedimentation rate

How is it treated?

Healthcare providers treat AIED with corticosteroids. This medication reduces inflammation in your inner ear and prevents new attacks. Most people take steroid pills. But you may have a shot in your middle ear. There may be other medication options for people who can’t take steroids.

Treatment may start with high doses of steroids for two to four weeks. If your hearing improves, your healthcare provider may slowly reduce the dosage. You may take steroids for six months to a year. Your treatment may include immunosuppressants once you stop taking steroids. Like steroids, these medications help manage your immune system.

Medication often improves hearing loss. But sometimes, autoimmune inner ear disease causes severe hearing loss that treatment can’t fix. In that case, your provider may recommend hearing aids or a cochlear implant for the treatment of your hearing loss. A cochlear implant is an electronic device that improves your ability to hear more sounds and understand speech.

When should I seek care?

Talk to a healthcare provider right away if you have sudden hearing loss or hearing issues that don’t improve. The issue may be AIED. Early treatment can keep your immune system from doing more damage to your fragile inner ear.

What can I expect if I have autoimmune inner ear disease?

Treatment often restores hearing. But if it doesn’t, or the hearing loss comes back, there may be other treatment options. In some cases, you may need hearing aids or a cochlear implant.