Objective: An autoimmune pathogenesis has been suggested for idiopathic sensorineural hearing loss (iSHL). Specific tests have been developed to detect inner ear autoantibodies and have been shown to correlate with treatment outcome. However, the disease is rare, and specific tests are not easily available. We aimed to analyze the correlation between positive systemic autoimmune test results and steroid treatment outcome in patients with iSHL.
Study design: Prospective, single-center, open trial.
Setting: All patients younger than 60 years seen in the ENT department from 1999 to 2007 and fulfilling the criteria for iSHL were tested for systemic autoimmunity.
Patients: Patients were classified into 2 groups, according to the presence or absence of autoimmunity.
Intervention: : Clinical evaluation and audiologic tests.
Main outcome measures: The outcomes of steroid treatment were compared between these 2 groups.
Results: Forty-nine patients were included; the mean age at iSHL onset was 36.1 years. Hearing loss was often bilateral (89.8%). Tinnitus and vertigo were present in 75.5% and 51%, respectively. On audiograms, disease severity was correlated with disease duration. Nine patients (18.4%) had positive autoimmune tests: anti-neutrophil cytoplasmic antibody (n = 1), antinuclear antibody (n = 3), rheumatoid factor (n = 3), and antiphospholipid (n = 2). Twenty-five courses of oral steroids were evaluated after 1 month: 52% of cases experienced some improvement. There were no differences in outcome associated with autoimmune status (p = 0.85).
Conclusion: The results of this study suggest that positive autoimmune tests in patients with iSHL are not predictive of improvement after a 1-month steroid course. Therefore, systematic screening does not seem to be useful, and specific inner ear autoantibody tests need to be developed.