Autoimmune inner-ear disease (AIED), a rare condition in which the body’s immune systems attack the inner ear, is called a rare disorder. This can lead to hearing loss or other complications.
Although it is not always possible to determine why someone develops AIEDs, it is common for them to have at least one other type of autoimmune disorder.
Your hearing may be restored if you seek treatment early for AIED-related hearing damage. A hearing aid may be necessary if your hearing loss is severe. AIED is a condition that has no cure. However, it can be managed with regular care and monitoring.
What causes AIED?
An autoimmune condition is when your immune system attacks healthy cells in an error. AIED is a condition in which the immune system attacks healthy cells by attacking cochlin (a protein found in the inner ear).
Anyone can be affected by an AIED. It is not well understood why it occurs.
According to the American Academy of Otolaryngology – Head and Neck Surgery Foundation (AAOHNS), it is common in patients with one or more autoimmune conditions. These autoimmune disorders are often found in groups of two or more diseases.
AIED cases are usually found in the inner ear, and do not involve any other autoimmune diseases.
What are the signs of an AIED?
AIED is characterized by hearing loss in one ear that then spreads to the other ear. This may occur over several weeks or months. It is possible for both ears to experience hearing loss at different levels.
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Some other symptoms include:
- Hearing that something improves or worsens randomly
- Poor balance or dizziness (in approximately half of AIED cases).
- Tinnitus or ringing in your ears
- Systemic autoimmune diseases symptoms include fatigue and muscle aches.
AIED can sometimes be confused with Meniere’s disease or other inner ear conditions that lead to hearing loss. Both diseases can cause tinnitus and poor balance. Meniere’s disease typically affects one ear. This is the main difference.
How can an AIED be diagnosed?
Because AIED is so rare, there are no current guidelines. AIED is estimated to affect approximately 15 out of 100,000 people according to the AAO-HNS. It is associated with less that 1 percent of sensorineural loss.
AIED is a condition that doctors diagnose when all other explanations have been exhausted. See an ENT specialist if you are experiencing undiagnosed hearing loss.
- It is likely that you will be subject to multiple tests.
- Serial audiograms are a way to test your hearing over a period of time.
- Imaging scans, such as MRIs, can show details about the inner ear structure. They may also help to rule out other problems in the cochlea.
- Balance testing can be done by your vestibular. This test may take up to several hours.
A blood test is another important screening. This test checks for high levels of T cells in the inner ear that are responsive to antigens.
How does AIED get treated?
The best treatment for AIED is dependent on several key factors:
- Severity of symptoms
- Overall health
- Tolerance to medication
There are many medications that can be used to reduce your immune system’s response and alleviate symptoms. There are many options available for devices that can improve your hearing.
2020 Review of AIED Treatment Options suggests that treatment with corticosteroids such as prednisone be initiated. They can reduce inflammation quickly.
To diagnose AIED, your doctor might examine how you respond to corticosteroids. Your doctor might diagnose AIED if your symptoms do not improve after you start treatment.
Corticosteroids may cause side effects such as:
- Fluid retention and weight gain
- High blood pressure
- Mood swings
Sudden hearing loss may be caused by AIED. You will first receive oral steroids in this situation. The steroid injections may be delivered through the eardrum into the middle of the ear.
Biologics are medications that can be used to treat a patient who doesn’t respond to corticosteroids. Biologics, which are medications made from living organisms and include golimumab and rituximab. These medications could be as effective as corticosteroids for reverse hearing loss. They may also be helpful in maintaining steroid dependency.
Because biologics work with the immune system, there may be a higher risk of infection for patients taking them.
Immunosuppressants are another option for AIED treatment. Immunosuppressants slow down or decrease the body’s immune response. The body can use immunosuppressants to treat a wide range of autoimmune conditions and prevent organ transplant rejection. Methotrexate is a commonly used AIED immunosuppressant. It is also used extensively in chemotherapy.
Side effects of immunosuppressants could include:
- High blood pressure
- Increased risk of infection
- Weight gain
Auditory devices can be used to compensate for hearing loss caused by AIED. These devices can amplify sound and improve your hearing.
For example, a cochlear implant is a small electronic device that stimulates cochlear nerves to improve hearing. An internal component extends into the inner ear, while an external part is located behind it. Cochlear implants should be reserved for severe hearing loss cases or those with specific hearing patterns.
Many people with AIED find hearing aids to be a good option. These are much less intrusive than cochlear implants and can be removed when not required.
What are the risks of an AIED?
Unfortunately, we don’t know much about the risk factors that could lead to AIED. However, we know that AIED is more common in people with other autoimmune conditions than those without.
Primary AIED is a condition where the damage is not confined to the inner ear. Secondary AIED occurs when AIED is associated with other autoimmune conditions. According to a 2018 study, this can occur in up to 30% of AIED cases.
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Additional autoimmune diseases people could have in addition to AIED include:
- rheumatoid arthritis
- Ulcerative colitis
An autoimmune disease is not the only factor that increases your chance of developing AIED. According to a 2018 study, AIED is more common among women than in men. Their risk is greatest in their 20s and 50s.