title: Otologic Manifestation in IgG4-Related Systemic Disease.
abstract: 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 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 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.
authors: Department of Otorhinolaryngology, National Medical Center, Seoul, Korea.
authors: Han-Kyu
authors: HK
authors: Cho
authors: Yun Jong
authors: YJ
authors: Lee
authors: Jin-Haeng
authors: JH
authors: Chung
authors: Ja-Won
authors: JW
authors: Koo