The Incidence and Prognosis of Positive Autoimmune Laboratory Markers in Idiopathic Sudden Sensorineural Hearing Loss: A National Database Study

Objective To identify the incidence of positive autoimmune laboratory markers in idiopathic sudden sensorineural hearing loss (iSSNHL) and its impact on hearing prognosis. Study Design Retrospective cohort database study Setting A collaborative national database (TriNetX) sourced from 79 large healthcare organizations in the United States. Patients Adults (≥18 years old) diagnosed with iSSNHL (ICD-10 H91.2) treated with systemic steroids. Interventions Autoimmune laboratory markers and salvage intratympanic (IT) steroids for SSNHL (CPT 69801). Main Outcome Measures 1) Positivity of autoimmune laboratory markers—rheumatoid factor (RF), ANCA, DNA double strand antibody (Ab), Sjogren syndrome A and B Abs, SCL-70 Ab, cardiolipin IgG Ab, Jo-1 Ab, ANA, mitochondria Ab. 2) Percent of patients that underwent salvage IT steroids, cochlear implantation, or hearing aid evaluation—all utilized as a proxy for hearing outcomes. Results Subjects with iSSNHL who had autoimmune testing (n = 17,413) were marginally more likely to be positive for at least one autoimmune laboratory marker compared to subjects without iSSNHL (n = 17,413; 23.0% vs. 21.4%, p = 0.0006). Statistical significance was lost after removing nonspecific autoimmune markers, however. Of those with iSSNHL who received systemic steroid treatment, subjects with positive autoimmune markers (n = 5,153) versus negative autoimmune markers (n = 5,153) underwent similar rates of salvage IT steroids (7.1% vs. 7.8%, p = 0.154), hearing aid evaluation (2.76% vs 2.47%, p = 0.354), and cochlear implantation (1.65% vs. 1.69%, p = 0.878). Conclusions Patients with iSSNHL have a marginally higher incidence of nonspecific positive autoimmune laboratory markers compared to those without iSSNHL; however, the presence of these markers does not predict treatment response or prognosis. Specifically, autoimmune markers did not predict the need for salvage IT steroids, nor CI and hearing aid use in iSSNHL. Autoimmune laboratory testing may be useful in iSSNHL patients with additional symptoms suspicious for an autoimmune disorder; however, a generalized screening is not recommended as it is unlikely to alter management or prognosis.