Abstract

PurposeTo explore the characteristics, medical treatments, and long-term facial palsy outcome in Ramsay Hunt syndrome.MethodsPatient questionnaire including self-assessment of long-term facial palsy outcome and retrospective chart review. Initial facial palsy grade was compared to self-assessed or patient record stated palsy outcome. Occurrence of different characteristics (blisters, hearing loss, vertigo, etc.) of the syndrome were assessed.ResultsAltogether 120 patients were included of which 81 answered the questionnaire. All but one patient had received virus medication (aciclovir, valaciclovir), and half received simultaneous corticosteroids. If the medication was started within 72 h of Ramsay Hunt diagnosis, facial palsy recovered totally or with only slight sequelae in over 80% of the patients. Only a minority of the patients experienced varicella blisters simultaneously with facial palsy, blisters more often preceded or followed the palsy. Approximately 20% of the patients had their blisters in hidden places in the ear canal or mouth.ConclusionsThe long-term outcome of facial palsy in medically treated Ramsay Hunt syndrome was approaching the outcome of Bell’s palsy. It is crucial to ask and inform the patient about the blisters and look for them since, more often than not, the blisters precede or follow the palsy and can be in areas not easily seen.

Highlights

  • Ramsay Hunt syndrome (RHS) consists of peripheral facial palsy (FP) of the VII cranial nerve (CN) and herpes blisters in the head and neck area

  • All nerves that communicate with the facial nerve can be involved e.g. CNs V, VIII, IX, and X and cervical nerves C2, C3, and C4 [1]

  • RHS is caused by reactivation of latent varicella-zoster virus (VZV) [2, 3]

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Summary

Introduction

Ramsay Hunt syndrome (RHS) consists of peripheral facial palsy (FP) of the VII cranial nerve (CN) and herpes blisters in the head and neck area. All nerves that communicate with the facial nerve can be involved e.g. CNs V, VIII, IX, and X and cervical nerves C2, C3, and C4 [1]. Even though cranial polyneuropathy including many additional CNs (e.g. III, XI, XII) was not included in the original definition of RHS, investigators have reported such patients as rare cases of RHS [1, 4, 5]. Peitersen [6] studied idiopathic peripheral FP and gathered all cases throughout 25 years in Copenhagen, Denmark. In this group of 2570 FP patients, there were 116 cases of RHS. Estimates of incidence based on mostly retrospective studies of individual centers have ranged 4–12% of all FPs and around 5/100,000/year [7, 8]

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