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.


METHODS
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.
Mouadeb
Debbie Aviva
DA
Department of Otolaryngology, University of California-Davis, Sacramento, California, USA. debbie_mouadeb@yahoo.com
Ruckenstein
Michael J
MJ
D017153:Antibodies, Anticardiolipin / Q000276:immunology; D017152:Antibodies, Antiphospholipid / Q000276:immunology; D016736:Antiphospholipid Syndrome / Q000150:complications* / Q000276:immunology / Q000503:physiopathology*; D001301:Audiometry, Pure-Tone; D015331:Cohort Studies; D018450:Disease Progression; D007758:Ear, Inner / Q000503:physiopathology*; D006312:Hearing Loss, Bilateral / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D006801:Humans; D007118:Immunoassay; D007074:Immunoglobulin G / Q000276:immunology; D007075:Immunoglobulin M / Q000276:immunology; D008575:Meniere Disease / Q000175:diagnosis / Q000453:epidemiology / Q000503:physiopathology; D008875:Middle Aged; D011446:Prospective Studies; D012720:Severity of Illness Index; D014722:Vestibule, Labyrinth / Q000503:physiopathology
D017153:Antibodies, Anticardiolipin; D017152:Antibodies, Antiphospholipid; D007074:Immunoglobulin G; D007075:Immunoglobulin M