Autoimmune Inner Ear Disease (AIED)
© December 2001, latest revision August 2007 by Neil Bauman, Ph.D.
Question: There seems to be a lot of talk about autoimmune inner ear disease now. What exactly is this? How do I know if this caused my hearing loss? What can I do about it?—P. R.
Answer: Good questions! Autoimmune Inner Ear Disease (AIED) is one of those mystery conditions that bedevil and baffle so many doctors. Even today, not much is definitely understood about AIED. It is still the subject of much controversy and debate. The good news is that researchers are busy learning more about AIED so they can find effective ways of preventing and/or treating it.
What is AIED?
The short answer is, "Autoimmune inner ear disease (AIED) results when your body’s out-of-control immune system attacks your inner ear tissues."
God designed your extremely complex immune system to ward off infections. Normally your immune system fights invaders such as bacteria, viruses and cells from other organisms. However, sometimes something goes wrong and the antibodies and immune cells in your immune system fail to distinguish the "bad guys" from the "good guys." As a result, your immune system attacks various cells in your body. This results in one of the more than 80 identified autoimmune diseases. If this attack takes place in your inner ears, you end up with AIED.
AIED consists of a syndrome that may include sudden or progressive hearing loss in both ears, tinnitus, dizziness and a feeling of fullness in your ears.
Characteristics of AIED
Here are some things we now know about AIED.
1. The hearing loss is sensorineural (the old term was "nerve deafness") and normally occurs in both ears (79%) at the same time. The actual hearing loss often is somewhat different in each ear however (asymmetric).
2. When AIED only occurs in one ear, generally it will show up in your other ear at a later date.
3. When AIED occurs, your hearing loss progresses rapidly and gets steadily worse over a period of weeks or months and even up to a year or more. In contrast, Sudden Sensorineural Hearing Loss (SSHL) occurs over a matter of minutes to hours to days.
4. While you are losing your hearing, your hearing may fluctuate (so you may have "good days" and "bad days"), then stabilize at a certain level, or it may continue to get worse without any fluctuation.
5. AIED generally impairs your ability to understand speech. This may even be a bigger problem than the resulting hearing loss. If you have AIED, your speech discrimination test scores will often be poor.
6. About 50% of the people with AIED have balance (vestibular) problems. These symptoms can include vertigo (a spinning sensation), dizziness, imbalance (disequilibrium), ataxia (staggering gait) and motion intolerance.
7. Between 25% and 50% of the people with AIED have tinnitus (ringing, roaring, hissing...