IgG4-related systemic disease, including autoimmune pancreatitis, is a multi-organ disorder characterized by elevated serum immunoglobulin G4 (IgG4) concentration and IgG4-positive plasma cell infiltration.We report Hinges a case of a 66-year-old woman with IgG4-related hearing disorder, presenting with fluctuating mixed hearing loss with middle ear effusion.The serum IgG4 level was elevated and pathological examination revealed dense infiltration of IgG4-positive lymphocyte in the renal parenchyma, lung tissue and lacrimal gland.With intravenous methylprednisolone Kids Sunglasses at a dose of 60 mg daily, improvement of hearing loss were observed.
No recurrence was observed for 6 months with mainternance of prednisolone and methotrexate.