Sjögren's syndrome is a chronic autoimmune disease characterized by systemic dysfunction of exocrine glands, mainly the salivary and lachrymal glands. Sjögren's syndrome consists of two forms: primary Sjögren's syndrome, which is characterized by dry eyes and dry mouth without autoimmune diseases; and secondary Sjögren's syndrome, which is characterized by symptoms associated with other autoimmune diseases, such as systemic lupus erythematosus. Disease severities vary considerably from mild glandular dryness to severe glandular involvement with numerous extraglandular and systemic features. Sensorineural hearing loss is sometimes observed in both primary and secondary Sjögren's syndrome. This review article consists of (1) Pathology of Sjögren's syndrome, (2) Clinical manifestation of Sjögren's syndrome, (3) Autoimmune inner ear disease, (4) Histoanatomical features of the inner ear, (5) Immunological characteristics of the inner ear, (6) Pathophysiology of autoimmune inner ear disease, (7) Treatment for sensorineural hearing loss in Sjögren's syndrome, and (8) Future direction. Finally, we introduce a recently developed disease model of salivary gland inflammation and discuss future expectations for the treatment of sensorineural hearing loss in Sjögren's syndrome.
Keywords: Sjögren’s syndrome; autoimmune mediated inner ear disease; high-frequency hearing loss; sensorineural hearing loss.