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What To Know About Autoimmune Inner Ear Disease (AIED)

Autoimmune diseases occur when the body’s immune system mistakenly attacks its own healthy cells. With Autoimmune Inner Ear Disease (AIED), the immune system targets normal cells in the ear as if they were harmful viruses or bacteria. This often results in hearing loss. AIED is a rare condition, making up only 1% of the 28 million cases of hearing loss in the U.S. It tends to affect women more than men and affects individuals between the ages of 30 and 60.


Signs of AIED

AIED usually results in sensorineural hearing loss (SNHL), or hearing loss caused by damage to the inner ear. The severity of hearing loss can vary dramatically and may change rapidly. About 20% of people with AIED also have other autoimmune disorders like lupus or rheumatoid arthritis.


Additional Signs of AIED may include:

  1. A sensation of fullness in the ears accompanied by tinnitus (ringing or buzzing in one or both ears)

  2. Issues with balance or motion sensitivity

  3. Walking difficulties or vertigo

  4. Conductive hearing loss (hearing loss in the outer or middle ear)


If you’ve recently found it challenging to understand your barista repeat your order at CoffeeHead, it could be due to poor word recognition. This is a symptom of AIED. Even if AIED isn’t the cause, poor word recognition could be a sign of other forms of hearing loss and you should consider scheduling a hearing test.


Diagnosis and Treatments

The effects of AIED can often be reversed if treated promptly. The primary form of treatment is a course of corticosteroids lasting four weeks. The treatment can be extended up to six months before being gradually reduced.


For those who don’t respond to steroid treatments, immunosuppressants might be suggested. These drugs work by reducing the body’s immune response. Diagnosing AIED can be challenging due to the lack of established diagnostic criteria. Methods such as lab tests, immune screenings and steroid trials are used to diagnose AIED, with other potential causes for SHNL needing to be ruled out.


In some cases, AIED can lead to irreversible hearing loss, in which case assistive hearing devices could be beneficial. However, if detected early enough, the hearing loss can be treated.


The hearing specialists at Topeka Ear Nose & Throat can help you determine the most appropriate treatment for you.

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