Understanding Autoimmune Inner Ear Disease

Our immune system constantly works hard to protect our body from harm caused by alien substances like toxins, bacteria, and viruses. Blood proteins called antibodies attack and remove these foreign substances from our body to prevent them from causing illnesses and infections.

The immune system is one of those things that you don’t really notice as long as it’s doing its job right; but once it malfunctions, it’s hard to ignore. Sometimes, the human body mistakenly identifies its own as foreign and attacks itself. This is what we call an autoimmune reaction.

An autoimmune reaction can occur anywhere in the body, but today, we will focus on understanding autoimmune inner ear disease.

What is an autoimmune inner ear disease?

This disease, AIED in short, is a syndrome in which the patient suffers from progressive loss of hearing, which is often accompanied by dizziness or ringing in the ear (tinnitus). As the name suggests, it results from the immune system attacking the inner ear.

It can be caused by other autoimmune diseases such as systemic lupus erythematosus, Cogan’s disease, ulcerative colitis, Sjoegren’s syndrome, rheumatoid arthritis, and psoriatic arthritis. In fact, 30% of those who suffer from this disease also have another autoimmune disease that affects the rest or another part of their body.

Symptoms of AIED:

• Vertigo/dizziness and problems with balance or feeling like you are spinning
• Tinnitus/constant ringing or hissing in the ear
• Fullness and/or pressure in the ear

AIED shares the same symptoms with Meniere’s Disease, another condition affecting hearing, so it can be hard to diagnose immediately. It’s also often mistaken for a simple ear infection until it’s already too late.

If you suspect you have AIED, or are having trouble with hearing in general, you should go to the ENT doctor immediately. They will give you a physical exam, a hearing test, and a balance test to see if your inner ear is malfunctioning. They may also draw blood to have it checked. This will determine if you are having an autoimmune reaction, which will rule out other hearing diseases and identify AIED as the cause of your symptoms. Since AIED isn’t usually diagnosed early, your ENT doctor will most likely start you on a treatment that will at least prevent permanent damage to your hearing. Calcium and vitamin D supplements are helpful in slowing down hearing loss.

Once AIED has been properly diagnosed, treatments include high doses of steroids, medication for slowing down the immune system, and the use of a hearing aid to help the patient get around the hearing loss. Steroids work well for AIED, but they have shown to have many undesirable side effects.

Other treatments like cell and gene therapy and cochlear implantation are being looked into and laboratory tests are still being done using animal models.

If you think you suffer from a hearing problem and may need a hearing aid, make sure that you get a hearing aid prescription. For more details of our ENT services, please contact HK ENT Specialist.

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Hearing aids prescription,

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Sleep disordered breathing Management,

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