Castle Michael S, Carter Matthew M, Poulakis Alexander, Man Li-Xing, Schmale Isaac L
Department of Otolaryngology Head and Neck Surgery, University of Rochester Medical Center, Rochester, New York, USA.
University of Rochester School of Medicine and Dentistry, University of Rochester Medical Center, Rochester, New York, USA.
Case Rep Otolaryngol. 2025 Jul 28;2025:8773843. doi: 10.1155/crot/8773843. eCollection 2025.
To describe a rare case of granulomatosis with polyangiitis (GPA) initially presenting at the petrous apex, accompanied by a brief literature review. A detailed retrospective single-case study of a 29-year-old male diagnosed with GPA. A review of the scientific literature of GPA affecting the petrous apex, causing cranial neuropathies and/or ottorhrea was conducted. A 29-year-old male with a history of schizophrenia presented with right-sided otalgia, initially diagnosed as acute otitis media. Despite antibiotics, his symptoms persisted. Weeks later, he returned with cranial neuropathies and clear otorrhea. Imaging findings lead to a working diagnosis of skull base osteomyelitis despite noninfectious signs. His course was further complicated by his discharge against medical advice. Eventually, he was found to have a septal perforation and diffuse nasal inflammation. An autoimmune workup revealed c-ANCA-positive GPA. Subsequent kidney biopsy confirmed GPA, and appropriate therapy led to clinical improvement and near-complete resolution of skull base inflammation. This case underscores the diagnostic complexity of GPA. Initial misdiagnosis of infection delayed appropriate treatment. Awareness of the varied presentations of GPA, including rare manifestations like skull base involvement and symptoms such as otorrhea and conductive hearing loss, is valuable. Early consideration of autoimmune etiologies and timely serological and histopathological analyses can prevent diagnostic delays and unnecessary treatments, improving patient outcomes.
描述一例最初表现为岩尖部病变的肉芽肿性多血管炎（GPA）罕见病例，并进行简要文献复习。对一名诊断为GPA的29岁男性进行详细的回顾性单病例研究。对影响岩尖部、导致颅神经病变和/或耳漏的GPA科学文献进行了综述。一名有精神分裂症病史的29岁男性出现右侧耳痛，最初诊断为急性中耳炎。尽管使用了抗生素，其症状仍持续存在。数周后，他因颅神经病变和清亮耳漏再次就诊。影像学检查结果虽无感染迹象，但初步诊断为颅底骨髓炎。他违背医嘱出院，病情进一步复杂化。最终，发现他有鼻中隔穿孔和弥漫性鼻炎症。自身免疫检查显示c-ANCA阳性的GPA。随后的肾脏活检证实为GPA，适当的治疗使临床症状改善，颅底炎症几乎完全消退。该病例强调了GPA诊断的复杂性。最初误诊为感染导致治疗延误。认识到GPA的多种表现形式，包括像颅底受累这样的罕见表现以及耳漏和传导性听力损失等症状，很有价值。早期考虑自身免疫病因并及时进行血清学和组织病理学分析可防止诊断延误和不必要的治疗，改善患者预后。
