Autoimmune Inner Ear Disease

Pam Rangen
Assistant Director
Respiratory Therapy, St. Alexius Medical Center

Autoimmune Inner Ear DiseaseAutoimmune inner ear disease, or AIED, is an inflammatory condition of the inner ear.  It occurs when a person’s immune system attacks body cells rather than fights infection in the body.  Autoimmune attacks in the inner ear result in progressive hearing loss.

The most popular theory as to the cause of  AIED is that cells meant to fight disease and foreign materials accidentally damage the inner ear because they mistake inner ear cells to be a virus, bacteria or other harmful substance that are present in the body at the time.  Researchers also are exploring the possibility that the disease may have a genetic origin.

Hearing loss resulting from an autoimmune disorder is rare, representing less than one percent of all cases of hearing or balance problems.  However, it can be associated with a variety of autoimmune diseases, including lupus, scleroderma and rheumatoid arthritis.  Researchers also are beginning to think that Meniere’s disease may be an autoimmune disorder.  Meniere’s disease is a disorder of the inner ear that causes episodes of vertigo, ringing in the ears, a feeling of fullness or pressure in the ear and fluctuating hearing loss.

When a person has AIED, their ability to hear will fluctuate and then worsen in a progressive manner.  Problems with balance, dizziness and tinnitus (ringing in the ears) also may occur.  The disease may affect one or both ears.  Nearly 80 percent of patients experience hearing loss in both ears.

65 percent of all patients diagnosed with AIED are women.  When AIED is suspected, diagnosis is not considered until hearing worsens dramatically in the better ear.  When this occurs, medical professionals review the patient’s family history and results of hearing and balance tests.  In addition, blood tests and a physical are conducted.

Patients who are diagnosed with AIED may be helped with hearing aids.  A common treatment approach is the use of steroids, which often helps to stabilize or improve the patient’s health status.  Over the long run, however, a chemotherapy-type medication or immune modifying medication may be utilized.  Plasmapheresis, a process that cleans the blood of unwanted substances, also may be helpful in treating symptoms of the disease.

If you suspect you may have AIED, talk with your doctor, preferably one who specializes in ear, nose and throat conditions.  Together, you can decide on a course of treatment.