Ratmeyer Paul R, Johnson Benjamin R, Roldan Luis P, Kraai Tania L
University of New Mexico School of Medicine, Department of Surgery: Division of Otolaryngology and Head and Neck Surgery, Albuquerque, NM 87106, USA.
Case Rep Otolaryngol. 2021 Apr 20;2021:6632344. doi: 10.1155/2021/6632344. eCollection 2021.
Granulomatosis with polyangiitis (GPA) is a severe systemic vasculitis that commonly affects the paranasal sinuses, upper and lower respiratory tracts, and kidneys. GPA has also been associated with sensorineural hearing loss (SNHL), through inflammation of the cochlear apparatus. Early recognition, diagnostic laboratory evaluation, and appropriate treatment are essential to improve outcomes and achieve remission for patients with GPA. Here, we present a case of bilateral sudden sensorineural hearing loss (SSNHL) and distal symmetric polyneuropathy as the first presenting signs of GPA. A specific diagnostic work-up to rule out autoimmune inner-ear disease in patients with bilateral SSNHL is not clearly stated in the clinical practice guidelines from the American Academy of Otolaryngology-Head and Neck Surgery. The aim of this paper is to delineate an appropriate diagnostic work-up for patients with bilateral SSNHL when there is concern for autoimmune disease.
肉芽肿性多血管炎（GPA）是一种严重的系统性血管炎，通常累及鼻窦、上呼吸道和下呼吸道以及肾脏。GPA还与感音神经性听力损失（SNHL）有关，是通过耳蜗器官的炎症引起的。早期识别、诊断性实验室评估和适当治疗对于改善GPA患者的预后和实现缓解至关重要。在此，我们报告一例以双侧突发感音神经性听力损失（SSNHL）和远端对称性多发性神经病为GPA首发症状的病例。美国耳鼻咽喉-头颈外科学会的临床实践指南中并未明确指出针对双侧SSNHL患者排除自身免疫性内耳疾病的具体诊断检查方法。本文的目的是明确当怀疑存在自身免疫性疾病时，针对双侧SSNHL患者的适当诊断检查方法。
