Sudden sensorineural hearing loss (SSHL) may be a manifestation of systemic vascular involvement in systemic lupus erythematosus (SLE) and may have an important impact on the health of patients with SLE. To investigate the risk of developing SSHL in patients with SLE, we conducted a population-based, retrospective cohort study from the Taiwan National Health Insurance Research Database. A total of 7168 patients diagnosed with SLE and 35840 control subjects without SLE were selected from claims made from 2001 to 2006. The incidence of SSHL was assessed and determined at the end of 2010. The incidence of SSHL was 2.22-fold higher in the SLE group than in the non-SLE group (6.52 vs. 2.93 per 10000 person-years), with an adjusted hazard ratio (HR) of 2.253 (95% confidence interval, CI = 1.407–3.608) calculated using a Cox proportional hazard regression model. Age was an independent risk factor for SSHL, with adjusted HRs of 2.103 for individuals aged ≥35 years compared with those 0–34 years. In the 0–34 age range, the incidence of developing SSHL was 4.27-fold (95% CI = 2.11–8.67) higher in the SLE group compared with the non-SLE group. In female patients, the incidence of developing SSHL was 2.19-fold (95% CI = 1.73–3.50) higher in the SLE group than in the non-SLE group. Systemic lupus erythematosus was significantly associated with an increased risk of developing SSHL. Scheduled auditory examinations for patients with SLE to assess the presence of chronic hearing impairment are advised to enable the early detection of SSHL.