Overview of Autoimmune Inner Ear Disease

Signs and Symptoms

The first signs and symptoms of autoimmune inner ear disease is the reduction of hearing or tinnitus which is hissing or ringing in the ears. Some people will only lose hearing in one ear or the other, while many more have hearing loss in both ears. There are variations of AIED that appear medically much like Meniere’s disease, where dizziness is accompanying the other symptoms along with abnormal blood tests. More than half of all patients diagnosed with autoimmune inner ear disease have balance issues. Diseases that are closely related to or have been associated with AIED include Cogan’s disease, ulcerative colitis, psoriatic arthritis, Bechet’s disease, systemic lupus erythematosus and ankylosing spondylitis.

Diagnoses

All diagnoses are based on the patient’s history as well as physical exams along with blood tests and hearing or vestibular testing. Since hearing neuropathy can be found with many other diseases and has other causes, an ABR test should be done on those patients that have enough hearing for its use. For those patients who do not have enough hearing for the ABR testing, otoacoustic emission testing can be done.

Diagnosis of this disease is difficult and sometimes is even confused with otitis media so extensive testing is required for the diagnosis. One test in particular that is used with much success is the Western blot immunoassay. Audiometry, ECOG or electrocochleography and the rotary chair test are all additional tests used to help diagnose AIED. Blood tests for this disease includes ANA, raji-cell, erythrocyte sedimentation rates, thyroid tests, anti-gliadin antibodies, smooth muscle antibodies and even rheumatoid factors in the blood.

Treatment of Autoimmune Inner Ear Disease

For patients with a rapidly progressing case, steroid trials can be done using Prednisone or Decadron for a month. For those persons that respond to the steroids, Cytoxan or Methotrexate can be used for a long term treatment. In more recent studies, Enbrel or Etanercept has been used as an injection, twice a week.

Risks or Possible Complications

Potential complications or risks from autoimmune inner ear disease include balance problems and permanent hearing loss if treatment is not sought early enough. Since this is an autoimmune disorder, medication can be used to control the loss of hearing and can help with the vertigo that is felt from the loss. Causes or risks of complications due to AIED include sustaining damage to the inner ear, surgery or infection on the inner ear and genetics. If anyone in your family has ever had autoimmune inner ear disease, there is a high possibility of being diagnosed with AIED.

Resources

American Hearing Research Foundations - Autoimmune Inner Ear Disease last edited 6/10/08