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孤立的感觉神经听力损失是多炎的粒状瘤病的唯一表现

Alejandro Arango¹, Sehreen Mumtaz², Andy Abril²

¹Department of Internal Medicine, Pontifical Bolivarian University, Medellín, Colombia.

The American journal of case reports

|September 12, 2025

概括

带有多叶膜炎 (GPA) 的粒状瘤症很少只能呈现为渐进的双侧神经神经听力损失 (SNHL). 早期识别和治疗这种ANCA相关的血管炎对于保护听力至关重要.

科学领域:

类风湿病学类风湿病学
耳鼻喉科耳鼻喉科耳鼻喉科
腎臟病學 (nephrology) 是一種醫學專業.

背景情况:

带有多炎的粒状瘤炎 (GPA) 是一种罕见的系统性血管炎,影响小血管,通常涉及呼吸道和脏.
在GPA中,耳部疾病的发生很常见,但作为最初和唯一的表现,感觉神经听力损失 (SNHL) 非常罕见.
进展性双边SNHL作为GPA的唯一症状,特别是在蛋白酶3-ANCA (PR3-ANCA) 阳性的情况下,很少报告.

研究的目的:

报告一种罕见的GPA病例,该病例仅表现为渐进的双边SNHL.
强调在患者中考虑ANCA相关血管炎的重要性. 无法解释的SNHL.
突出早期免疫抑制疗法在非典型的GPA呈现中保持听觉功能的潜力.

主要方法:

一个36岁的男子突然发病SNHL的病例报告.
诊断工作包括听力测量,实验室测试 (PR3-ANCA,ANA,炎症标志物) 和头部MRI.
治疗包括皮质类固醇和rituximab.

主要成果:

患者出现了渐进的双边SNHL,耳和听觉充实.
实验室测试证实了PR3-ANCA阳性,炎症标志物略高;其他器官参与不存在.
确立了GPA与孤立的内耳干扰的诊断.
用皮质类固醇和rituximab实现了症状稳定.

结论:

这个案例说明了GPA与PR3-ANCA阳性和孤立的渐进双边SNHL的异常呈现.
早期发现非典型的GPA对于预防不可逆转的听力损失至关重要.
临床医生应该怀疑ANCA相关的血管炎在无法解释的双边SNHL中,即使没有全身症状,以实现及时的免疫抑制治疗和潜在的听力保护.

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