Everything You Need to Know About Autoimmune Inner Ear Disease

If you’ve been fighting a hearing loss disorder that hasn’t responded to standard treatment, you might be fighting something more sinister.

Autoimmune Inner Ear Disease is a rare form of sensorineural hearing loss that causes permanent hearing damage. Here are the symptoms, causes, and treatments you need to know.

Out of 28 million Americans that experience hearing loss, about 1 percent of them suffer from a hearing disorder called Autoimmune Inner Ear Disease.

Autoimmune Inner Ear Disease is a relatively rare type of bilateral sensorineural hearing loss that occurs most frequently in middle-aged women.

It’s often difficult to diagnose as its symptoms are similar to other sensorineural hearing loss diseases, and there is no clear diagnostic test available.

However, identifying it early is imperative because permanent hearing loss will spread from one ear to the other ear if it’s left untreated.


What is Autoimmune Inner Ear Disease

Autoimmune hearing loss occurs when the immune system becomes confused and believes that cells in your inner ear are harmful viruses. The immune system attacks and kills the inner ear cells the same way that it would kill a virus.

Unfortunately, the Autoimmune Inner Ear Disease isn’t the only type of Autoimmune Disease. An Autoimmune Disease is any disease where the immune system attacks healthy parts of the body.

For example, Lupus is one of the more common Autoimmune Diseases where the immune system attacks various healthy tissues and organs throughout the body.

Roughly 20 percent of patients with Autoimmune Inner Ear Disease also suffer from other autoimmune diseases.


What Causes Autoimmune Inner Ear Disease

Scientists are still learning more about the exact cause of Autoimmune Inner Ear Disease. However, many predict that some drugs can lead the immune system to confuse healthy cells for harmful cells.

Many medical professionals believe that genetics also plays a role as the disease often occurs in families. Most family members have different types of Autoimmune Diseases, suggesting that the susceptibility to autoimmune conditions is genetic, but the individual disease is not.

One study showed that the Autoimmune Inner Ear Disease tends to occur twice as much in women than in men, though there isn’t a conclusion on why women may be more susceptible. The disease usually shows up between the ages of 15 and 44.


Autoimmune Inner Ear Disease Symptoms

The most obvious Autoimmune Inner Ear Disease symptom is hearing loss. It usually starts in one ear and eventually spreads to the other ear over the course of several weeks.

Other symptoms include tinnitus (ringing in your ears), vertigo, dizziness, and a fullness in your ear. Patients may also lose word recognition.

Unfortunately, Autoimmune Inner Ear Disease can be difficult to diagnose, as there is no diagnostic test. Its symptoms also mimic other more common hearing disorders such as a Middle Ear Infection or even Ménière’s disease.

Most doctors perform a series of blood and balancing tests if they suspect you have Autoimmune Inner Ear Disorder, but the results are often inconclusive. Therefore, most doctors immediately start patients on steroids such as dexamethasone, prednisone, and prednisolone to prevent hearing loss from spreading.

If the steroids are successful, the patient is then diagnosed with the disease.



Autoimmune Inner Ear Disease can be life-altering, though advancements in technology are helping patients continue to live normal lives. Most patients that begin treatment within the first two months of the disease still retain useful hearing. Hearing aids and implants have also greatly increased patient quality of life.

If you suspect that you may have symptoms of Autoimmune Inner Ear Disease, talk to your doctor immediately about how you can proceed with treatment options.