Hearing Loss in Systemic Lupus Erythematosus: a Systematic Review.

Abstract

Background: Hearing loss is an underrecognized manifestation of systemic lupus erythematosus (SLE). Proposed mechanisms include autoimmune inner ear injury, vascular occlusion related to antiphospholipid syndrome (APS) and ototoxicity from medications such as hydroxychloroquine (HCQ). Although multiple case reports and observational studies have described this complication, the overall burden and clinical significance remain unclear.

Methods: A systematic review of PubMed, Embase, Web of Science, Scopus and the Cochrane Library was conducted up to September 2025, following PRISMA guidelines. Eligible studies included cohort, case-control, cross-sectional studies, case series, case reports and temporal bone analyses reporting auditory outcomes in SLE patients. Data on prevalence, clinical presentation, pathophysiology and management were extracted. Risk of bias was assessed using validated tools appropriate to each study design.

Results: The search identified 236 records; after removal of duplicates and screening, 10 studies were included. These comprised case reports, small series, histopathology and larger observational studies. Reported prevalence of sensorineural hearing loss in SLE ranged from ~27% in pooled analyses to ~55-56% when extended high-frequency audiometry was used. Pathological studies demonstrated vasculitis, stria vascularis atrophy and hair cell loss in SLE patients. Case-based evidence consistently linked sudden hearing loss to antiphospholipid antibodies, supporting a vascular occlusive mechanism. Hydroxychloroquine ototoxicity was described in isolated cases, often reversible after discontinuation, but not consistently supported in larger studies.

Conclusion: Hearing loss in SLE is more prevalent than in the general population and arises through multifactorial mechanisms. Early recognition and tailored management, including corticosteroids for autoimmune-mediated cases, anticoagulation for APS and discontinuation of suspected ototoxic drugs, may improve outcomes. Routine audiological monitoring should be considered to reduce the risk of irreversible disability in this patient population.