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Autoimmune Inner Ear Disease

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 disorders include:

  • Sed Rate,CRP
  • ANA
  • Rheumatoid Factor
  • Complement C1Q
  • Thyroid (TSH, anti-microsomal antibodies)
  • anti-gliadin antibodies (for Celiac disease)

 Blood tests for conditions that resemble autoimmune disorders include:

  • RPR (for Syphilis)
  • Lyme titer
  • HBA1C (for diabetes, which is often autoimmune mediated also)
  • HIV (HIV is associated with auditory neuropathy)

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.

History

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.




 

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