Immunoglobulin G4: Cross Talk in Hearing Loss Manifestation

Abstract Immunoglobulin G4-related disease (IgG4-RD) is a systemic immune-mediated condition characterized by tissue infiltration with IgG4-positive plasma cells and elevated serum IgG4 levels. While IgG4-RD can affect multiple organs, its involvement in the auditory system, leading to hearing loss, is a less frequent but clinically significant manifestation. This review comprehensively examines the underlying pathophysiology, diagnostic techniques, and management options for hearing loss related to IgG4-RD. The pathogenesis involves a complex dysregulation of B- and T-cell responses, resulting in chronic inflammation and fibrosis of affected tissues. Diagnosis typically requires a combination of clinical presentation, elevated serum IgG4 levels, imaging studies, and histopathological findings. Treatment primarily consists of systemic corticosteroids, with immunosuppressive agents like rituximab considered for refractory cases. The review highlights the importance of early diagnosis and appropriate management to prevent long-term complications and improve patient outcomes. By increasing clinicians’ awareness of IgG4-related otological diseases, this review aims to enhance understanding and facilitate better care for affected patients.