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Autoimmune Inner Ear Disease

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Some degree of hearing loss is common with age. Most types are irreversible, but autoimmune inner ear disease (AIED) is an exception.

Maria C. Buniel and colleagues at the Cleveland Clinic provided a review.

“AIED is one of the few treatable forms of sensorineural hearing loss and is diagnosed on clinical suspicion,” Buniel wrote. The hearing loss develops over the course of weeks to months, in both ears, often with one ear affected more than the other one.

Some patients experience temporary facial paralysis. Vertigo is less common. The primary treatment is corticosteroids.

Not everyone responds to corticosteroids. Shresh Pathak of the Feinstein Institute for Medical Research in New York and colleagues investigated factors that may determine which patients can benefit from treatment.

Pathak identified two subgroups of sensorineural hearing loss: AIED and sudden sensorineural hearing loss (SSNHL).

The SSNHL patients usually experience an isolated hearing loss event in only one ear. The cause may be autoimmune, but is more likely to be viral. “Nonetheless, the immunologic responses may be similar,” Pathak wrote.

Approximately 70 percent of patients initially respond to corticosteroids, but only 14 percent remain responsive after 34 months.

Pathak found that patients who do not respond to steroids have higher levels of the immune molecule IL-1beta in their blood. This finding offers guidance to researchers who are investigating new treatments.

“To date, no effective treatment has been identified for patients with either AIED or SSNHL who do not respond to steroids,” he concluded.

The Vestibular Disorders Association offers more information and support online. Their web site reports that other autoimmune disorders can also affect the ears:

1.Rheumatoid arthritis

2.Systematic lupus erythematosus

3.Ulcerative colitis

4.Sjogren's syndrome

5.Cogan's syndrome

6.Relapsing polychondritis

7.Polyarteritis nodosa


The Vestibular Disorders Association site reports that chemotherapy agents, anti-transplant rejection drugs, and anti-tumor necrosis factor drugs (TNF blockers) can treat some forms of autoimmune ear disease.

An ear doctor (otolaryngologist or neurotologist) may recommend a rheumatologist for treatment of hearing loss caused by autoimmune processes.

The American Hearing Research Foundation provides another website with AIED information.


Buniel MC et al, “Immunosuppressive therapy for autoimmune inner ear disease”, Immunotherapy. 2009; 1(3): 425-34. http://www.ncbi.nlm.nih.gov/pubmed/19885385

Pathak S et al, “IL-1beta is overexpressed and aberrantly regulated in corticosteroid nonresponders with autoimmune inner ear disease”, J immunol. 2011 Feb 1; 186(3): 1870-79.

Autoimmune Inner Ear Disease (AIED). Vestibular Disorders Association. Web. May 3, 2012.

American Hearing Research Foundation. Web. May 3, 2012.

Reviewed April 25, 2012
by Michele Blacksberg RN
Edited by Jody Smith

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EmpowHER Guest

I struggled with AIED for a number of years before finding what appears to be the cause and the cure. Jugular vein blockage. It's a plumbing problem. Blood doesn't drain from head, backs up around the ears, deposits excess iron triggering an autoimmune response. A non-invasive ballooning of the veins and voila, some symptoms disappear immediately, others slowly diminishing as the body processes slowly eliminate the deposit of excess iron. Google "Pablo Zamboni, CCSVI and MS and you can learn about the procedure. S.

April 29, 2012 - 6:41am
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