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Novel Association of GAD68-Positive Autoimmune Inner Ear Disease with Autoimmune Urticaria
Monday, March 7, 2016
South Exhibit Hall H (Convention Center)
Jack G. Ghably, MD, Sara Atwater, MD, Mark Guido, MD, Aman Nasir, MD, Guha Krishnaswamy, MD, FAAAI, CC-D, ABIHM
Rationale: Acute autoimmune sensorineural loss or autoimmune inner ear disease (AIED) is characterized by bilateral, progressively worsening asymmetric hearing loss that usually improves with early immunosuppressive therapy. The disease shares a T-cell mediated pathology with Muckle-Wells Syndrome (MWS), a rare autosomal disorder characterized by urticarial rash and steroid-resistant deafness.  AIED was postulated to be an autoimmune disorder, and a large subset of patients with this disease express antibodies to the 68kD antigen/HSP70.   Other associated autoantibodies can be directed against collagen, endothelial, or cochlear antigens.    

Methods: We evaluated a 56 year old female with acute onset of progressive hearing loss in the left ear and fluctuant loss in the right ear.  Audiograms demonstrated compatible losses.  Serology was negative for ANA but was positive for antibodies to 68kD antigen/HSP 70.  The patient also developed recurrent bouts of urticaria and angioedema, with very high titers of antibody to the high affinity IgE receptor anti-FceRI.  The hearing loss responded to intratympanic steroids, while the urticaria improved with histamine and leukotriene inhibition.

Results: This patient demonstrates a novel association of autoimmune urticaria with autoimmune sensorineural deafness.  AIED has not been described in association with chronic autoimmune urticarial (characterized by detectable antibodies targeting FceRI on mast cells and basophils).  However both disorders tend to occur with increased frequency in patients with autoimmune “proclivity”.  Several autoantibodies may coexist is these patients- such as ANA, RF, ANCA, and cardiolipin and thyroid antibodies.

Conclusions: The pathogenesis and roles of IgE and TNF-alpha in these diseases and novel approaches to therapy need further evaluation.