What is AIED?
Autoimmune inner ear disease or "AIED"
consists of a syndrome of hearing loss or dizziness which is
caused by antibodies or immune cells which are attacking the
inner ear. It can be a sudden
hearing loss on one side or both ears accompanied by pain. In
the past was called Meniers disease.
The classic picture is reduction of
hearing accompanied by tinnitus (ringing, hissing, roaring)
which occurs over a few months. Variants are bilateral
attacks of hearing loss and tinnitus which resemble
, and attacks of dizziness accompanied by
abnormal blood tests for self-antibodies. About 50% of
patients with AIED have balance symptoms.
we have reversed this
disease with simple antibiotics.
As there are no specific tests for AIED, a common approach
is to look for other evidence for autoimmune involvement.
Blood tests for autoimmune
- Sed Rate,CRP
- Rheumatoid Factor
- Complement C1Q
- Thyroid (TSH, anti-microsomal
- anti-gliadin antibodies (for
Blood tests for conditions that
resemble autoimmune disorders include:
HBA1C (for diabetes, which is
often autoimmune mediated also)
HIV (HIV is associated with
What Are the Symptoms of AIED?
The symptoms of AIED are sudden hearing loss in one ear
progressing rapidly to the second ear. The hearing loss can
progress over weeks or months. Patients may feel fullness in
the ear and experience vertigo. In addition, a ringing,
hissing, or roaring sound in the ear may be experienced.
Diagnosis of AIED is difficult and is often mistaken for
otitis media until the patient develops a loss in the second
ear. One diagnostic test that is promising is the Western
blot immunoassay. Meniere's
disease and AIED seem to be closely intertwined. In fact,
immune dysfunction is increasingly being recognized as one
of the factors that causes Meniere's disease. Researchers
now feel that a "significant percentage" of the people with
bilateral Meniere's disease have AIED as the underlying
cause. One study indicated that immune system dysfunction
was responsible for 16% of people with bilateral Meniere's
disease and 6% of all people with any variety of Meniere's
disease. In another study, 96% of the patients with
Meniere's disease had elevated levels of circulating immune
complexes, compared to just 20% of the controls. In yet
another study, specific immune system antibodies were
detected in 47% of people with Meniere's disease. This all
shows that a large percentage of the people with Meniere's
disease have an underlying immune system problem.
What Is the Treatment for AIED?
Most patients with AIED respond to the initial treatment
of IVIgm steroids, prednisone, and methotrexate, a chemotherapy
agent. Some patients may benefit from the use of hearing
aids. If patients are unresponsive to drug therapy and
hearing loss persists, a cochlear implant maybe considered.
Until recently it was thought that the inner ear could
not be attacked by the immune system. Studies have shown
that the perisacular tissue surrounding the endolymphatic
sac contains the necessary components for an immunological
reaction. The inner ear is also capable of producing an
autoimmune response to sensitized cells that can enter the
cochlea through the circulatory system.