Vestibulopathy in Patients Presenting With Ramsay Hunt Syndrome

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.