Autoimmune inner ear disease (AIED) is a rare disease in which the body’s immune system attacks the inner ear. This often causes hearing loss and other complications.
While it’s not always clear why a person develops AIED, it’s common for someone with the disease to also have at least one other autoimmune disorder.
When you get treatment for AIED-related hearing loss early on, your hearing can often be restored. If hearing loss is significant, you may need a hearing device. Though AIED has no known cure, with ongoing care and regular monitoring it can often be a manageable condition.
An autoimmune disease is a condition in which your immune system mistakenly attacks healthy cells. In the case of AIED, the immune system goes after cochlin, a protein in the inner ear.
AIED can affect anyone. The reasons why it develops aren’t well understood.
It is often found in people with one or more autoimmune disorders, according to the American Academy of Otolaryngology–Head and Neck Surgery Foundation (AAO-HNS). These autoimmune disorders often develop in clusters of two or three diseases.
Most cases of AIED, however, are confined to the inner ear and occur without the presence of other autoimmune diseases.
How a healthy inner ear works
Sounds first enter the ear through the outer ear. Those sound waves hit the eardrum and cause it to vibrate. The vibrations cause tiny bones known as ossicles to amplify the sound in the middle ear.
The inner ear takes in sound waves from the middle ear and transmits them into the cochlea, which is largely composed of cochlin. The cochlea is a tiny fluid-filled organ in the inner ear that carries sound waves to thousands of nerve endings. Those nerve endings transform the sound waves into electrical impulses that travel to the brain for interpretation.
The inner ear is also the home of the vestibular organ, which helps control balance.
The main symptom of AIED is hearing loss that starts in one ear and then affects both ears. This can develop over a period of weeks or months. The degree of hearing loss is not always the same in both ears.
Other symptoms include:
- hearing that improves and worsens at random
- dizziness or poor balance (in about half of AIED cases)
- tinnitus, or ringing in the ears
- systemic autoimmune disease symptoms, such as fatigue and muscle aches
AIED is sometimes mistaken for other inner ear conditions that cause hearing loss, including Meniere’s disease. The two diseases have similar symptoms, like poor balance and tinnitus. The main difference between the two conditions is that Meniere’s disease usually affects only one ear.
When is hearing loss a medical emergency?
A 2018 study suggests AIED usually develops over a span of
There are currently no guidelines for diagnosing AIED, mostly because it’s so uncommon. The AAO-HNS estimates that AIED affects about 15 in 100,000 people. It’s associated with less than 1 percent of sensorineural hearing loss.
Doctors tend to diagnose AIED when other possible explanations for symptoms have been eliminated. If you’re experiencing unexplained hearing loss that is worsening in both ears over a period of weeks, see an ear, nose, and throat (ENT) specialist.
You will likely undergo several tests:
- Serial audiograms test your hearing over the course of several months.
- Imaging scans such as MRIs can reveal details of the inner ear structure and may help rule out other problems with the cochlea.
- Vestibular testing can test your balance. This test can take several hours.
One other important screening is a blood test. This test looks for elevated levels of T cells that respond to antigens in the inner ear.
The best treatment for AIED depends on a few key factors, including:
- severity of symptoms
- overall health
- medication tolerance
A variety of medications are available to tamp down your body’s immune system response and ease symptoms. You also have options when it comes to devices that improve hearing.
Your doctor may evaluate your response to corticosteroids to help diagnose AIED. If symptoms start to go away after starting treatment, your doctor may determine that AIED was the cause of your hearing loss and balance problems.
Side effects from corticosteroids can include:
AIED may present with sudden hearing loss. In this emergency situation, you’ll first be treated with a course of oral steroids. You may also receive steroid injections through the eardrum directly into the middle ear.
If you don’t respond well to corticosteroids, medications known as biologics may be helpful. Biologics are medications made from living organisms, and include rituximab and golimumab. A
Biologics may present a heightened risk of infection because of the way the drugs work on the immune system.
Another AIED treatment option is immunosuppressants. Immunosuppressants are drugs that slow or reduce the body’s immune system response. Immunosuppressants are taken for a variety of autoimmune diseases, as well as to help keep the body from rejecting organ transplants. A commonly used AIED immunosuppressant is methotrexate, which is also widely used in chemotherapy.
Potential side effects of immunosuppressants include:
To compensate for hearing loss related to AIED, you may benefit from auditory devices. These can amplify sound or otherwise improve your hearing.
A cochlear implant, for example, is a tiny electronic device that stimulates the cochlear nerve to improve hearing. The implant has an internal portion that reaches into the inner ear and an external component located behind the ear. Cochlear implants are usually reserved for cases of severe hearing loss or specific patterns of hearing loss.
Hearing aids are also effective options for many people with AIED. They are less invasive than a cochlear implant, and you can remove them when they’re not needed.
We don’t know much about risk factors for AIED. But we do know that people who have other autoimmune disorders are more likely to develop AIED compared to those who don’t.
When the impact is isolated to the inner ear, the condition is called primary AIED. When AIED is accompanied by other autoimmune disorders, the condition is known as secondary AIED. This occurs in as many as
Other autoimmune diseases that people may have along with AIED include:
Having an autoimmune disease is only one factor that raises your risk of developing AIED. A
A diagnosis of AIED may seem alarming at first, but it can be reassuring to know the reason for your hearing loss and to know that treatment is possible in many cases.
Once you have received a proper diagnosis and begun treatment, you may remain on medications designed to keep your immune system in check. In some cases, the types and dosages of medications may need to be adjusted based on factors such as resistance to a drug or side effects.
Monitoring your hearing will also be an ongoing part of living with AIED. Expect to have regular hearing tests, such as audiograms, which use tones to show how loud sounds need to be for you to hear them clearly.
Without treatment, AIED usually leads to complete hearing loss and balance difficulties. There isn’t a standard timeline for symptoms to progress. But the sooner you get an evaluation after symptoms begin, the greater the chance of limiting the impact of AIED.