Vanstrum Erik B, Smith Eric, Weng Maxwell, Kim Minjung, Kita Ashley
Department of Head and Neck Surgery, Los Angeles, California.
David Geffen School of Medicine, Los Angeles, California.
Otol Neurotol Open. 2024 Dec 13;4(4):e064. doi: 10.1097/ONO.0000000000000064. eCollection 2024 Dec.
Ramsay Hunt syndrome (RHS) refers to a reactivation of the varicella-zoster virus in the distribution of the facial nerve, but it can involve other cranial nerves as well. In patients with polyneuropathy, the vestibulocochlear nerve is most involved after the facial nerve. The clinical manifestations and long-term vestibular outcomes in these patients remain unclear. This report aims to characterize the clinical course of this rare subset of RHS patients.
This study is a retrospective case series. The study was conducted in a tertiary care institution. Patients with RHS polyneuropathy presenting with vestibular deficit were reviewed over a 30-year period. Case details including initial presentation, House-Brackmann grade, treatment regimen, vestibular examination and testing, MRI findings, and follow-up time were extracted.
A total of 22 patients were identified. The mean age of diagnosis was 53 years and the average follow-up time was 23 months. Most patients demonstrated complete facial paralysis (88%) upon presentation. Nystagmus was rarely recorded during physical examination (18%, n = 4). For those patients who underwent MRI of the internal auditory canal (n = 17), 59% demonstrated enhancement of the facial nerve, with a notable absence of vestibulocochlear nerve abnormality. Of the patients who underwent vestibular testing, all demonstrated unilateral caloric weakness on videonystagmography (VNG; 66% ± 22%, n = 8).
Patients with RHS polyneuropathy and vestibular complaints do not consistently demonstrate objective vestibular physical examination or imaging findings on presentation. However, VNG consistently demonstrates significant unilateral weakness on caloric stimulation. Most patients in our sample continued to have vestibular complaints at latest follow-up.
拉姆齐·亨特综合征（RHS）是指水痘-带状疱疹病毒在面神经分布区域重新激活，但也可累及其他颅神经。在患有多发性神经病的患者中，前庭蜗神经在面神经之后最常受累。这些患者的临床表现和长期前庭预后仍不清楚。本报告旨在描述这一罕见的RHS患者亚组的临床病程。
本研究为回顾性病例系列研究。研究在一家三级医疗机构进行。对30年间出现前庭功能缺损的RHS多发性神经病患者进行了回顾。提取了病例详细信息，包括初始表现、House-Brackmann分级、治疗方案、前庭检查和测试、MRI结果以及随访时间。
共确定了22例患者。诊断时的平均年龄为53岁，平均随访时间为23个月。大多数患者初诊时表现为完全性面瘫（88%）。体格检查时很少记录到眼球震颤（18%，n = 4）。对于接受内耳道MRI检查的患者（n = 17），59%显示面神经强化，明显没有前庭蜗神经异常。在接受前庭测试的患者中，所有患者在视频眼震图（VNG）上均显示单侧冷热试验减弱（66%±22%，n = 8）。
患有RHS多发性神经病且有前庭症状的患者在初诊时并不总是表现出客观的前庭体格检查或影像学异常。然而，VNG始终显示在冷热刺激下有明显的单侧减弱。我们样本中的大多数患者在最近一次随访时仍有前庭症状。
