Antiphospholipid inner ear syndrome.

Objective: This study was initiated to clarify the possible association between antiphospholipid antibodies, mediators of microthrombus formation, and sensorineural hearing loss (SNHL) of unknown origin in a large cohort of patients.

Study Design: Prospective study.

Methods: The study cohort consists of 168 adult patients (>/=18 yr) referred to the University of Pennsylvania's Division of Neurotology and Balance Center for diagnosis and treatment of progressive hearing loss with or without vertigo. A comprehensive screening panel of blood tests for autoimmune and infectious diseases was applied to all patients, including testing for anticardiolipin antibodies, anti-B2 glycoprotein, and lupus anticoagulant.

Results: Forty-two patients (25%) had at least one elevated antiphospholipid antibody marker. Twenty patients had two or more positive test results. Of the 42 patients, 64% (n = 27) met the diagnostic criteria for Meniere disease, and the remainder were diagnosed with idiopathic SNHL. Within this group of patients, 24 patients (57%) had unilateral hearing loss, and 18 (44%) had bilateral hearing loss.

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.