Autoimmune Inner Ear Disease

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. While AIED specifically attacks the inner ear, other forms of autoimmune disease can affect the skin, nervous system, the joints and any of the other systems in the body.

Autoimmune disease can be systemic or organ specific such as AIED (inner ear), diabetes (pancreas), Graves disease (thyroid), joint (rheumatoid arthritis), skin (psoriasis) and multiple sclerosis (central nervous system). Examples of systemic autoimmune disease affecting several organ systems throughout the body are systemic lupus, erythematosis (SLE) involving skin, kidneys, etc or scleroderma (skin, esophagus). 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 frequently 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.

A multicenter clinical trial funded by the National Institute of Deafness and Communication Disorders (NIDCD) was recently completed 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 was imperative that this scarce population and its treatment be thoroughly investigated in centers involved with the clinical trial.

Please note that this clinical trial was completed. The main publication from this trial is:

Harris, J.P., Weisman, M.H., Derebery, J.M., Espeland, M.A., Gantz, B.J., Gulya, A.J., Hammerschlag, P.E., et al.: Treatment of Corticosteroid-Responsive Autoimmune Inner Ear Disease with Methotrexate: A Randomized Controlled Trial.  Journal of the American Medical Association, Vol. 290, No. 14: 1875-1883, October, 2003.

The centers that participated in this AIED are listed below:

Boystown National Research Hospital Clinic
Coordinator:Marjorie Brennan Phone Number: 402-498-6597 E-mail: brennanm@boystown.org Address: Boys Town National Research Hospital 555 N. 30th Street Omaha, NE 68131

UCSD Medical Center Clinic
Coordinator: Muppy Haigler Phone Number: 858-657-8057 E-mail: mhaigler@ucsd.edu Address: Division of Otolaryngology, Head & Neck Surgery 9350 Campus Point Dr. La Jolla, CA 92037-0970

Clinic Coordinator: Sandra Neal, RN, BSN Phone Number:214-648-9151 E-mail: sandra.neal@email.swmed.edu Address: UTSouthwestern Dept. of Otolaryngology 5323 Harry Hines Blvd. Dallas, TX 75390-9035

Cleveland Clinic
Clinic Coordinator: Nancy Nalepa, MA Phone Number: 216-444-4948 E-mail: nalepan@cesmtp.ccf.org Address: Dept. of Otolaryngology 9500 Euclid Ave., Desk A-71 Cleveland, OH 44195-5245

House Ear Institute
Clinic Coordinator:Ann Masuda, MS Phone Number: 213-273-8005 E-mail: amasuda@hei.org Address: 2100 West Third Street Los Angeles, CA 90057

House Ear Institute
Clinic Coordinator: Patty Johnson Phone Number: 213-353-7025 E-mail: pjohnson@hei.org Address: Clinical Studies Dept. 2100 West Third Street Los Angeles, CA 90057

University of Iowa School of Medicine
Clinic Coordinator: Rachel Hottel, RN Phone Number: 319-356-4851 Address: Department of Otolaryngology – Head & Neck Surgery 200 Hawkins Dr. Iowa City, IA 52242

Massachusetts Eye & Ear Infirmary

Clinic Coordinator:Pamela O’Brien, RN, MSN Phone Number:617-573-4123 E-mail: pamela_obrien@meei.harvard.edu Address: Dept. of Otolaryngology 243 Charles Street Boston, MA 02114

Johns Hopkins University
Clinic Coordinator: JoAnne Andresini, NP Phone Number:410-955-6554 E-mail: jandres@jhmi.edu Address: Dept. of Otolaryngology, Head & Neck Surgery 601 North Caroline Street Baltimore, MD 21287 33136

University Of Michigan School of Medicine
Clinic Coordinator:Donna Priano-English Phone Number:734-764-9122 E-mail: prieng@umich.edu Address: CCIT/Research Support Division 1310 E. Catherine St., Rm. 3201, Upjohn Center Ann Arbor, MI 48109-0504

New York University School of Medicine
Department of Otolaryngology Clinic Coordinator: Dorlene Jean, RN Phone Number:212-889-2600 E-mail: information@newyorkear.com Address: 650 First Ave., York NY 10016

If you are seeking more help about AIED, you can start with these centers distributed throughout the country.