Autoimmune Diseases Web

Autoimmune inner ear disease (AIED

Autoimmune inner ear disease (AIED) is an inflammatory condition of the inner ear. It occurs when the body's immune system attacks cells in the inner ear that are mistaken for a virus or bacteria. AIED is a rare disease occurring in less than one percent of the 28 million Americans with a hearing loss.

Ruckenstein MJ.

Department of Otorhinolaryngology, Head and Neck Surgery, University of Pennsylvania,


The role of the immune system in inner ear disease has received considerable attention over the past two decades.  To summarize recent basic science research into the pathogenesis of autoimmune inner ear disease (AIED), review the current diagnostic work-up for patients with suspected AIED, and delineate treatment strategies.


Basic science and clinical studies have been performed to delineate the mechanisms by which autoimmune processes may affect the ear and to develop treatment strategies to reverse this pathology.

AIED refers to a rapidly progressive (over a course of weeks to months) sensorineural hearing loss that responds to the administration of corticosteroids. In addition, systemic autoimmune diseases (eg, vasculitis, Lupus, Wegener) can secondarily affect the inner ear.

Although a number of diagnostic tests for AIED have been advocated, the diagnosis of this entity is still predicated on a positive therapeutic response to corticosteroid administration.

Alternate immunosuppressive regimens, designed to be used in patients who cannot be weaned off corticosteroids or whose disease becomes refractory to treatment, have proven to be difficult to develop. Methotrexate, which initially showed promise, has recently been shown to be ineffective in preventing progression of hearing loss. Further research is required to better delineate the pathophysiologic mechanisms involved in AIED and to establish more effective and better tolerated treatment regimens.

SUMMARY: The original enthusiasm that was generated by the concept that immune-mediated mechanisms may mediate reversible forms of inner ear pathology has been tempered by the realities conveyed by scientific research. The pathophysiology of AIED is still not well understood. Multiple potential mechanisms have been identified that can result in immune-mediated inner ear pathology. The diagnosis of AIED is based on clinical presentation and response to the administration of corticosteroids. Diagnostic testing may support the diagnosis, and the results of a recent multicenter study should clarify the role of immunologic testing in the diagnosis of this entity. Treatment options are limited, with corticosteroids being the only validated treatment option, and methotrexate offering no significant benefit to patients. The role of cyclophosphamide is restricted to patients willing to endure the attendant risks. With the advent of cochlear implants, the administration of toxic medications to preserve hearing at all costs is a less desirable

CIDPUSA has discovered that the disease is triggered by a virus & bacteria is very difficult to treat and by using electronic means the treatmentshould be limited. As the Herx reaction tend to make the attacks worse in the initial treatment period. Steroids in the ear are very effective.


Semin Arthritis Rheum. 2004 Oct;34(2):544-8.

Immune-mediated inner ear disease: 10-year experience.

Broughton SS,

Otolaryngology, University of Texas Southwestern Medical School, Dallas, Texas, USA.

 RESULTS: Patients with AIED presented with rapidly progressive, frequently bilateral (79%), often fluctuating sensironeural hearing loss. Mean age at presentation was 50 years (22-80) with no gender predilection. Tinnitus (83%), vestibular complaints (79%), and Menieres (50%) were common concomitant symptoms along with hearing loss. Seven of 42 (17%) of the patients had evidence for other systemic autoimmune disorders.  Twenty-three of 33 (70%) of patients treated with corticosteroids improved clinically, often short-term. Sixteen patients received treatment with other immunosuppressive drugs including methotrexate, cyclophosphamide, azathioprine, mycophenolic mofetil, and intravenous immunoglobulin (IVIg) generally with a limited modest response. Using clinical trial criteria for response, only 5/35 (14%) demonstrated improvement over a mean 34.4 months follow-up.

PMID: 15505770 [PubMed - indexed for MEDLINE]

IVIg has been used successfully as well as intratympanic steroids to reverse the disease.


Pathalogy in CIDP and autoimmune diseases