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Clin Exp Otorhinolaryngol. 2011 Mar;4(1):52-4. doi: 10.3342/ceo.2011.4.1.52. Epub 2011 Feb 07.

Otologic Manifestation in IgG4-Related Systemic Disease.

Clinical and experimental otorhinolaryngology

Han-Kyu Cho, Yun Jong Lee, Jin-Haeng Chung, Ja-Won Koo

Affiliations

  1. Department of Otorhinolaryngology, National Medical Center, Seoul, Korea.

PMID: 21461065 PMCID: PMC3062229 DOI: 10.3342/ceo.2011.4.1.52

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.

Keywords: Autoimmune disease; Hearing loss; Immunoglobulin G

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