Autoimmune Inner Ear Disease

Created in Ears

Autoimmune inner ear disease (AIED) is an inflammatory condition caused by an uncontrolled immune system response that attacks the inner ear causing progressive sensorineural hearing loss (SNHL) that usually starts in one ear and then affects the other ear. The body thinks a part of the inner ear should not be there and makes antibodies and immune, also called antigen-antibody, complexes that attack the inner ear. This causes blood vessel inflammation, inner ear tissue damage, and hearing loss. Antigens are the molecules that cause an immune response and antibodies are proteins that protect the body against these antigens.

AIED is rare and diagnosed only when all other causes have been ruled out. The estimated prevalence of AIED is about 15 out of 100,000 people. AIED is felt to be responsible for less than one percent of all SNHL cases, realizing many AIED cases might not be diagnosed due to a lack of specific tests.

AIED is considered “primary” when the inner ear is the only organ affected. However, in 15 to 30 percent of cases, AIED is “secondary” when it occurs as part of a larger autoimmune disorder that affects the whole body, such as rheumatoid arthritis, lupus, scleroderma, ulcerative colitis, or Sjogren’s syndrome. AIED is more common among middle-aged women.

Diagnosing AIED is challenging because there are no definite blood or imaging criteria to measure. Timing the progression of hearing loss over weeks to months is an important diagnostic clue for AIED. If AIED is suspected, early corticosteroid and immunosuppressive treatment may prevent irreversible hearing loss. A multidisciplinary team approach between an ENT (ear, nose, and throat) specialist, or otolaryngologist, an audiologist, and a rheumatologist is recommended to manage the condition.

What Are the Symptoms of AIED?

Common symptoms of AIED can include:

What Causes AIED?

The causes of AIED can include:

What Are the Treatment Options?

If you are having hearing loss that is getting worse in both ears over weeks to months, you should see an ENT specialist who can make a diagnosis after reviewing your hearing tests and imaging scans. If your doctor suspects that you may have AIED, you may respond well to medical therapy—steroid and immunosuppressive medication—if started early.

Corticosteroid is the main treatment to suppress the immune response to reduce inner ear swelling and inflammation. Early detection of AIED and prompt steroid treatment may help reverse your SNHL. If you cannot tolerate steroid treatment, there are alternative medications, such as cyclophosphamide, methotrexate, azathiorprine, and rituximab.

Your doctor may also evaluate you for a hearing aid or other assistive listening devices. If hearing loss is substantial, your doctor may recommend a hearing device called a cochlear implant. Speak with your doctor about other specific treatment options.

What Questions Should I Ask My Doctor?

  1. Do I have a systemic autoimmune disorder?
  2. What tests should I have performed to evaluate my hearing loss?
  3. Do I need to get an MRI to rule out other inner ear disorders?
  4. What are my treatment options for AIED?
  5. What medications are available to me?
  6. What other healthcare professionals should I see for AIED?

Copyright 2021. American Academy of Otolaryngology–Head and Neck Surgery Foundation.

You Might Also Enjoy...

Sinusitis

Have you ever felt like you had a cold that wouldn’t go away? If symptoms of discolored nasal drainage and blockage hang around for more than 10 days, or worsen after they start getting better, there’s a good chance you have sinusitis...

Sore Throats

Everybody gets a sore throat now and then. When you have a sore throat, this can affect speaking, swallowing, or breathing. Infections from viruses or bacteria are the main cause of sore throats,...

Swimmer's Ear

Swimmer’s ear (also called acute otitis externa) is a painful condition that affects the outer ear and ear canal that is caused by infection, inflammation, or irritation.

Tinnitus

Over 50 million Americans have experienced tinnitus, or ringing in the ears, which is the perception of sound without an external source being present.

Tonsillitis

Tonsillitis, also described as pharyngitis, refers to inflammation of the pharyngeal tonsils, which are lymph glands located in the back of the throat that are visible through the mouth.

Turbinate Hypertrophy

Turbinate hypertrophy refers to an excessive growth or enlargement of the turbinates, which are bony structures located inside the nose. They are covered with a special skin called mucosa, and they help filter, warm, and humidify the air as you breathe.