Information on Autoimmune inner ear disease.
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.
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.
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.
IVIg has been used successfully as well as intratympanic steroids to reverse the disease.