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.
Risk Factors for Getting AIED
There are several factors that increase your chances of getting AIED. Those already identified include:
How Common is AIED?
Autoimmune diseases of all kinds strike an estimated 14 to 50 million people in the US. Many people who ultimately get AIED come from this enormous "pool" of people with existing autoimmune diseases.
At present, no one seems to have a clue just how many people actually have AIED since researchers are still trying to refine and identify this disease. Furthermore, mild versions of AIED are probably never even reported.
The figure that is currently bandied about is that less than 1% of hard of hearing people have AIED. This figure seems much too low since approximately half of the people with Meniere's disease are thought to have AIED as the underlying cause. Meniere's disease affects approximately 6% of hard of hearing people. Therefore, the incidence of AIED from Meniere's sufferers alone would be about 3%.
Diagnosing AIED is a tricky business. This is because there is still no well-established means of diagnosing AIED although researchers are working on it. Doctors try to diagnose AIED based on medical history, findings of physical examination, blood tests, and the results of hearing and balance tests. Dr. Timothy Hain declares, "While specific tests for autoimmunity to the inner ear would be desirable, as of this writing, [Sep 2001], there are none that are commercially available and proven to be useful." As a result, doctors often look for evidence of other autoimmune diseases that may be present elsewhere in the body to indicate that AIED might also be present.
One of the chief ways doctors confirm their diagnosis of AIED is to try a course of immunosuppressant drugs. Researchers have found that if you have certain anti-bodies in your blood, you will very likely respond positively to steroid treatment. Therefore, if the patient responds positively, doctors assume this confirms their diagnosis that it must have been AIED!
The treatment goal in people severely affected with AIED is to improve their hearing/discrimination enough so that they can benefit from wearing hearing aids. In those with mild to moderate losses, the goal is to recover hearing to normal or near normal levels.
Doctors think that AIED is potentially reversible. Therefore they like to treat it with steroid drugs and see if they can stop the progress of AIED or make it retreat since steroids are the drug of choice for other autoimmune diseases. Sometimes it seems to work. Sometimes it doesn't do a thing. In fact, doctors don't have a clue what would happen if no treatment was given since, when patients come to them, they always "try" something. Dr. Shelley Broughton wrote, "The natural history of untreated immune-mediated inner ear disease is unknown at this time." In other words, doctors don't know what would happen if they kept their hands off and let nature take its course.
Because many doctors believe that AIED is caused by inflammation and altered immunity, they try the same immunosuppressive drugs they use to treat rheumatoid arthritis and cancer. According to the American Academy of Otolaryngology Head and Neck Surgery, Inc., they use high doses Prednisone, Methotrexate, and Cyclophosphamide, but with "little guidance about dosages or duration of treatment and with less than ideal proof of efficacy."
Reading between the lines, you can see that doctors at present don't know how much of which drugs to prescribe. They don't know how long they should prescribe these drugs. Finally, they don't have a clue whether these drugs work or not! As a result, you need to decide for yourself if you want to risk all the harmful side effects to your body from these dangerous drugs on the chance that it will help your hearing. They may, and then again, they may not.
If you are going to go the drug treatment route, you need to begin promptly. This is extremely important to have the best chance to recover or at least stop the hearing loss. However, aggressive treatment doesn't necessarily work. Look at Rush Limbaugh. In spite of the best treatment from the best doctors (in my opinion), he still lost every scrap of his hearing.
Not all people respond to steroid therapy the same way. Treatment in some people results in better hearing, or better discrimination or both. With others, hearing fluctuation or hearing loss progression stabilizes where it is without improving any. With the remainder, they continue to lose their hearing in spite of immunosuppressive therapy.
In cases of rapidly progressing bilateral hearing loss, doctors try steroids such as Prednisone (Deltasone) or Dexamethasone (Decadron) for 4 weeks. If the person responds to this treatment, then they often put them on long-term chemotherapy drugs such as Cyclophosphamide (Cytoxan), Methotrexate (Rheumatrex), or Azathioprine (Imuran).
In one study using Methotrexate, the patients had significant improvement in their discrimination scores, but did not have any improvement in their hearing.
Overall, steroid response rates are approximately 60%. Success is defined as a hearing improvement of just 10-15 dB, or a "significant" improvement in discrimination scores. For a severe loss, this is not all that great an improvement, but at least the hearing loss isn't progressing any more!
Plasmapheresis treatment may also be beneficial. This involves filtering the patient's blood, which removes antibody, antigen, and immune complexes and other immune mediators. 75% of the patients in one study regained some of their hearing after this treatment.
The anti-TNF drug, Etanercept (Enbrel) shows promise too.
Prednisone, although an effective immunosuppressant drug, has a host of negative side effects, especially in the long term. You want to be aware of them. You need to know that you can get both cataracts and glaucoma (among other things) from taking Prednisone. If the Prednisone treatment isn't successful in restoring your hearing, you could find yourself up a creek without a paddle, ending up both hard of hearing and without much sight.
You also want to be careful when taking any cytotoxic drugs because their side effects can also be serious. For example, Cyclophosphamide is often prescribed, yet it can cause increased risk of malignancy (cancer).
If you have (or think you have) AIED, talk with your doctor. Together decide on a course of treatment (if any) that fits your particular situation and takes into consideration the risks that are acceptable to you.
Neil Bauman, Ph.D., was born with a severe hereditary hearing
loss. He is a hearing loss coping skills specialist, researcher,
author, speaker and educator. He is not a medical doctor and does
not prescribe/endorse treatment for medical problems. This educational
article is for your information only. If you suspect that you
have a medical problem related to your hearing, please seek competent
medical help. Use the information here to help you make informed
decisions, not as a substitute for any treatment that your doctor
may prescribe. Send your questions to him at: firstname.lastname@example.org
or visit his web site at: www.hearinglosshelp.com.
BACK to Table of Contents