Autoimmune diseases

Inner Ear Disease
by: Imran Khan, M.BBS |JUlY 08, 2020

Antiphospholipid inner ear syndrome Mouadeb DA,

Department of Otolaryngology, University of California-Davis, Sacramento, California, USA. OBJECTIVE: This study was initiated to clarify the possible association between antiphospholipid antibodies,  CONCLUSIONS: These data support the hypothesis that antiphospholipid antibodies are involved in the pathogenesis of some forms of inner ear dysfunction, presumably by causing microthrombus formation in the labyrinthine vasculature. Basic science studies are required to better understand the mechanisms by which antiphospholipid antibodies mediate inner ear dysfunction. Clinical studies to evaluate the efficacy of anticoagulation in this group of patients are also required.

Autoimmune inner ear disease (AIED) was first described in 1979 and the disease has become more widely recognized over the last decade. Limited information is available regarding clinical features of the disease, disease course, and response to treatment.
To analyze data from 42 patients with documented immune mediated inner ear disease to further define this syndrome.
A retrospective chart review was conducted on all patients considered to have AIED by the Otolaryngology division physicians and on all patients positive for antibody testing to inner ear antigens from 1990 to 1999. Patients who were antibody positive with a clinical diagnosis of AIED were included in this review.
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. In 4 of the patients the onset of vestibuloauditory complaints preceded the diagnosis of autoimmune disorder. Thirty-three of 42 demonstrated antibodies to inner ear antigens but other autoantibodies were infrequent except in patients with 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.
Immune-mediated inner ear disease is not a uniform disease with simple diagnosis or treatment. The course of the disease often results in significant long-term disability due to hearing loss and response to aggressive immunosuppression including corticosteroids is poor.


If the autoimmune disorder affects the blood, the person may need blood transfusions.

Measures to help with movement or other functions may be needed for autoimmune disorders that affect the bones, joints, or muscles.

Medicines are often prescribed to control or reduce the immune system's response. Such medicines may include corticosteroids and immunosuppressant drugs such as cyclophosphamide or azathioprine.

Before one starts on medication one should find the offending organism or allergy. The most common are food allergies and in particular celicac disease.

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