Ramsay Hunt syndrome

Ramsay Hunt syndrome typically presents with sudden-onset (<72 hours) unilateral peripheral facial palsy, severe ear/facial pain, and a vesicular ear rash. Other presenting symptoms include vertigo, hearing loss, tinnitus, dry eye, altered taste, and oral lesions.

It is caused by reactivation of latent varicella zoster virus (VZV) and spread to the facial nerve. Previous exposure to VZV, age >50 years, and immunosuppression are key risk factors.

Diagnosis is typically clinical. Other serious causes of facial paralysis such as cerebrovascular accidents and tumors must be ruled out. If there is uncertainty regarding etiology, the vesicular lesions, if present, can be swabbed directly for confirmation by VZV polymerase chain reaction.

Prompt combination treatment (within 72 hours) with high-dose oral corticosteroids and antivirals is recommended in all patients. Patients with ocular symptoms should be given eye lubricants. In patients who can’t close their eyes, taping the eye shut is recommended to protect the cornea at night.

The majority of patients will recover their facial movement at least partially with treatment.

Ramsay Hunt syndrome (also known as herpes zoster oticus or auricular herpes zoster) is a rare neurologic condition characterized by reactivation of the varicella zoster virus involving the facial nerve, and less commonly other cranial nerves (e.g., V, IX, and X).[1]Gross GE, Eisert L, Doerr HW, et al. S2k guidelines for the diagnosis and treatment of herpes zoster and postherpetic neuralgia. J Dtsch Dermatol Ges. 2020 Jan;18(1):55-78. https://www.doi.org/10.1111/ddg.14013 http://www.ncbi.nlm.nih.gov/pubmed/31951098?tool=bestpractice.com Patients often present with a triad of symptoms: sudden-onset (<72 hours) ipsilateral peripheral facial palsy, severe ear/facial pain, and vesicular ear rash, including blisters.[1]Gross GE, Eisert L, Doerr HW, et al. S2k guidelines for the diagnosis and treatment of herpes zoster and postherpetic neuralgia. J Dtsch Dermatol Ges. 2020 Jan;18(1):55-78. https://www.doi.org/10.1111/ddg.14013 http://www.ncbi.nlm.nih.gov/pubmed/31951098?tool=bestpractice.com However, many patients do not present with all three classic features as these do not always develop at the same time; vesicles can precede, coexist with, or follow facial palsy. The most suggestive symptom is unilateral facial palsy. Ramsay Hunt syndrome is most common in people ages >50 years and is extremely rare in children. The condition is a diagnosis of exclusion. Patients typically see improvement in facial movement within a few weeks to months of symptom onset. However, changes in facial symmetry and function can continue for up to a year before a patient’s exam stabilizes. Recurrent Ramsay Hunt syndrome is exceedingly rare.[2]Sweeney CJ, Gilden DH. Ramsay Hunt syndrome. J Neurol Neurosurg Psychiatry. 2001 Aug;71(2):149-54. https://www.doi.org/10.1136/jnnp.71.2.149 http://www.ncbi.nlm.nih.gov/pubmed/11459884?tool=bestpractice.com