안면 마비와 난청으로 진단된 면역글로불린 G4 연관질환 동반 육아종성 다발 혈관염 1예

Granulomatosis with polyangiitis (GPA) and Immunoglobulin (Ig) G₄-related disease (IgG₄-RD) are rare diseases and early diagnosis and proper management are imperative to prevent multi-organ damage. The authors present a case of a 60 years old woman who had facial paralysis and hearing loss. Lt intact canal wall tympanomastoidectomy, Lt facial nerve decompression and ossiculoplasty with partial ossicular replacement prosthesis (PORP) was done. During operation, middle ear tissue was biopsied and GPA with IgG₄-RD was diagnosed. After methyl prednisolone (MPD) pulse therapy and azathioprine therapy, the severity of paralysis was improved. We present this case because common otologic symptoms like facial palsy and hearing loss could be initial symptoms of rare systemic disease. (J Clinical Otolaryngol 2018;29:240-244)