2. Department of Otolaryngology-head and neck surgery, Blegdamsvej 9B, 2100 Copenhagen East, Denmark.
3. Private practice Ear, nose and throat diseases Strandgade 41, 4800 Nykoebing Falster, Denmark.
4. Department of Otorhinolaryngology and audiology, Copenhagen University Hospital Rigshospitalet/Gentofte, Niels Andersens vej 65, 2900 Hellerup, Denmark.
Introduction: The Ramsay Hunt syndrome is characterized by herpetic lesions combined with peripheral facial nerve palsy. The disease is caused by a reactivation of the varicella zoster virus and can be deceiving since the herpetic lesions are not always present (zoster sine herpete) and might mimic other severe neurological illnesses. This article reviews the various forms of Ramsay Hunt syndrome and how they can give rise to diagnostic and therapeutic challenges.
Material and method: Studies on the assessment and treatment of Ramsay Hunt syndrome were found by conducting a thorough literature search in PubMed, Medline, The Cochrane Library database and Google Scholar using the search words «varicella», «zoster», «ramsay hunt», «oticus», «cranial nerve», «facial nerve» and combinations thereof. The bibliographies of substantial articles were subsequently assessed.
Results: About 12% of all peripheral facial nerve palsies are caused by varicella zoster virus. In more than 50% pain is the initial symptom making the diagnosis difficult. Female gender, and in general age above 50 years, renders patients more susceptible to Ramsay Hunt syndrome. The main prognostic factor is the severity of the initial symptoms. The occurrence rate of associated cranial polyneuropathy has been reported to be 1.8-3.2% and cranial nerves VII, VIII, IX are the ones most commonly affected. The full recovery rate is reported to be as low as 27.3% when multiple cranial nerves are involved. Combination therapy comprising of antiviral drugs and corticosteroids is recommened and should be initiated within 72 hours. Vaccination against varicella zoster virus is an interesting new development that might reduce the incidence of varicella zoster virus associated disease altogether.
Conclusion: Ramsay Hunt syndrome is a difficult and severe diagnosis with a low full recovery rate. Extensive randomized trials are urgently needed to verify the optimal treatment and the efficacy of varicella zoster virus vaccine in both naïve and herpes zoster patients.
Keywords: Ramsay Hunt syndrome, facial paralysis, herpes zoster, herpes zoster oticus, cranial nerve diseases