Autoimmune Inner Ear Disease FAQ
(Frequently Asked Questions)
What is Autoimmune Inner Ear Disease?
Autoimmune Inner Ear Disease (AIED) is a poorly understood syndrome of potentially reversible progressive sensorineural hearing loss with or without dizziness. The classic clinical presentation of this disease is with bilateral fluctuating progressive sensorineural hearing loss occurring over several months. Tinnitus (noises in the ear) and aural fullness, "pressure", may also occur as well as dizziness or vertigo.It is felt that this clinical syndrome of potentially reversible sensorineural hearing loss is caused by autoimmune dysfunction. Autoimmune diseases are set off when an out of control immune system causes the body to attack its own tissues by failing to distinguish the body's own cells from invaders such as bacteria or viruses or cells from other organisms. Autoimmune disease forms a large family of disorders which according to the National Institute of Allergy and Infectious Diseases strike an estimated fourteen to fifty million Americans. AIED can also be seen in people with other autoimmune diseases. New research has pointed out that many people with one autoimmune disease can have more than one type of autoimmune disease.
How often does AIED occur?
AIED is uncommon, probably accounting for less than one percent of all cases of sensorineural hearing loss or dizziness. The exact incidence is unknown partially because this disease is in the process of being defined and identified. It is felt that a significant percentage of patients with Meniere's Disease, especially those with bilateral symptoms, may be due to autoimmune inner ear dysfunction.How is the diagnosis of AIED made?
Your physician can make the diagnosis based upon your history, physical examination findings, blood tests and results of hearing and vestibular test. A fairly specific test, Western Blot immunoassay for Heat Shock Protein-70 antibodies correlates with active disease and potential steroid responsiveness in patients with bilateral rapidly progressive sensorineural hearing loss. However this laboratory test does not demonstrate correlation in all cases. Therefore a therapeutic trial of medication should be undertaken as directed by your physician for this potentially reversible sensorineural hearing loss.Is there any way we can find out more about AIED and why is this important?
Physician scientists are actively investigating the cause and treatment of AIED. Because of its unique potential reversibility, intensive study of this sensorineural hearing loss may provide leads for understanding other forms of sensorineural hearing loss.There is a multicenter clinical trial funded by the National Institute of Deafness and Communication Disorders (NIDCD) to study the cause of AIED and to evaluate a response to Prednisone and an alternative drug, Methotrexate. These drugs have a long track record for treatment of other autoimmune diseases. Since the number of patients with AIED are few, it is imperative that this scarce population and its treatment be thoroughly investigated in centers involved with the clinical trial.
If you would like more information about AIED or the AIED clinical trail please contact Kathy O'Connor, RN by phone at 212-263-2411 or by email at koconnor@brany.com.
Paul E. Hammerschlag, MD, FACS
Department of Otolaryngology
New York University School of Medicine
550 First Avenue, New York, NY 10016
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