Copyright 2001
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
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!
Treating 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: neil@hearinglosshelp.com
or visit his web site at: www.hearinglosshelp.com.
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