Autoimmune Inner Ear Disease (AIED): Causes, Treatment, and More – Healthline

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.

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:

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.

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. …….

Source: https://www.healthline.com/health/autoimmune-inner-ear-disease