Batinović Franko, Martinić Marina Krnić, Durdov Merica Glavina, Sunara Davor
Department of Otorhinolaryngology with Head and Neck Surgery, University Hospital Center Split, Split, Croatia.
Department of Pathology, Forensic Medicine and Cytology, University Hospital Center Split, Split, Croatia.
J Audiol Otol. 2023 Jul;27(3):161-167. doi: 10.7874/jao.2022.00311. Epub 2023 Feb 16.
Granulomatosis with polyangiitis (GPA) is a chronic and systematic autoimmune condition characterized by granuloma formation and necrotizing vasculitis of small to medium-sized vessels. GPA initially presents itself as respiratory and renal symptoms. Although temporal bone involvement is not uncommon, an otologic disorder is rarely the initial symptom. We present a case of a 36-year-old man who presented with unilateral ear pain, hearing loss, and facial palsy. After a series of diagnostics and temporal bone and chest imaging, he was diagnosed with GPA with multiorgan involvement. Cyclophosphamide and methylprednisolone relieved the patient's ear pain and partially improved his hearing, facial palsy, and overall clinical condition. Although uncommon, systemic GPA may cause initial otologic symptoms and should not be dismissed as a possible cause of an otologic disease resistant to standard therapy.
肉芽肿性多血管炎(GPA)是一种慢性系统性自身免疫性疾病,其特征为肉芽肿形成以及中小血管的坏死性血管炎。GPA最初表现为呼吸道和肾脏症状。虽然颞骨受累并不罕见,但耳部疾病很少是首发症状。我们报告一例36岁男性病例,该患者出现单侧耳痛、听力丧失和面神经麻痹。经过一系列诊断以及颞骨和胸部影像学检查后,他被诊断为累及多器官的GPA。环磷酰胺和甲泼尼龙缓解了患者的耳痛,并部分改善了他的听力、面神经麻痹及整体临床状况。虽然不常见,但系统性GPA可能导致首发耳部症状,不应被排除为对标准治疗耐药的耳部疾病的可能病因。