Image Diagnosis: Ramsay Hunt Syndrome

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Perm J 2012 Fall;16(4):52

https://doi.org/10.7812/TPP/12-035

A 68-year-old woman presented to the Emergency Department with left facial weakness (Figure 1). She reported upper and lower motor facial weakness. She was diagnosed with left-sided Bell's palsy and treated with steroids. She returned two days later with chief complaint of left ear pain and swelling. She denied having fever or hearing loss. Ramsay Hunt syndrome (Herpes zoster oticus) by strict definition is a peripheral facial nerve palsy accompanied by an erythematous vesicular rash on the ear (zoster oticus) or in the mouth. The triad of ipsilateral facial paralysis, ear pain, and vesicles in the auditory canal and auricle is typically present (Figure 2).¹ Various clinical presentations of facial paralysis and rash such as tinnitus, hearing loss, nausea, vomiting, vertigo, and nystagmus can be present. This has been attributed to close proximity of the geniculation ganglion to the vestibulocochlear nerve within the bony facial canal. In a prospective study of patients with Ramsay Hunt syndrome, 14% developed vesicles after the onset of facial weakness. Thus, Ramsay Hunt syndrome may initially be indistinguishable from Bell's palsy.² The facial paralysis seen in Ramsay Hunt syndrome is typically more severe than Bell's palsy, attributed to herpes simplex virus with a decreased probability of complete recovery.³ Retrospective studies have shown earlier administration of steroids along with antivirals within 3 days of symptom onset have 75% rate of full recovery versus only 30% if combined therapy is started 7 days after onset of symptoms. The typical combined therapy involves a 7- to 10-day course of famciclovir (500 mg, 3 times daily) or acyclovir (800 mg, 5 times daily), along with oral prednisone (60 mg daily for 3 to 5 days).⁴

References

1.    Adour KK. Otological complications of herpes zoster. Ann Neurol 1994;35 Suppl:S62-4. DOI: https://doi.org/10.1002/ana.410350718
2.    Sweeney CJ, Gilden DH. Ramsay Hunt syndrome. J Neurol Neurosurgery Psychiatry 2001 Aug;71(2):149-54. DOI: https://doi.org/10.1136/jnnp.71.2.149
3.    Robillard RB, Hilsinger RL Jr, Adour KK. Ramsay Hunt facial paralysis: clinical analyses of 185 patients. Otolaryngol Head Neck Surg 1986 Oct;95(3 Pt 1):292-7.
4.    Murakami S, Hato N, Horiuchi J, Honda N, Gyo K, Yanagihara N. Treatment of Ramsay Hunt syndrome with acyclovir-prednisone: significance of early diagnosis and treatment. Ann Neurol 1997 Mar;41(3):353–7. DOI: https://doi.org/10.1002/ana.410410310