Ramsay Hunt Syndrome, also known as herpes zoster oticus, is a VZV infection involving the ear and auditory canal with associated pain and ipsilateral facial paralysis. The VZV virus lies dormant in the geniculate ganglion, and when it is reactivated, Ramsay Hunt Syndrome may occur.
Pain precedes the rash, from hours to even days, which is important when giving follow up instructions to patients with pain in this distribution. The most common cranial nerve involved is CN VII, followed by CN VIII, IX, V, and VI. The paralysis will include the forehead, as this is a lower motor neuron being affected. However, you must always keep this diagnosis in mind when evaluated patients for CVA. The major differential diagnoses are Bell’s Palsy and CVA. The facial paralysis reaches its maximum around 1 week after the onset of symptoms. Also, 50% of people with Ramsay Hunt Syndrome will have ipsilateral hearing loss.
Other than paralysis, the most obvious physical exam finding is a vesicular rash that usually involves the pinna and the auditory canal. The actual rash appears the same as zoster on other parts of the body. Lab studies are usually not indicated, as this is a clinical diagnosis. However, if the patient is ill, they should have a workup initiated, including basic labs.
Treatment has usually consisted of oral steroids and antivirals. Prednisone is usually the steroid of choice, while acyclovir or valcyclovir are given as antivirals. A cochrane review showed no evidence that adding the antivirals to the steroids provides any benefit at 6 months, although most providers still use them. Pain control is usually accomplished with oral opioids such as hydrocodone or oxycodone in the short term, with carbamazepine being used for neuropathic pain in the long term. If the patient has vestibular symptoms, you should treat this symptomatically as well.
The take home message is the following: If you see some facial paralysis and are going down the Bell’s Palsy or CVA track, check the ear first!
Albrecht M. Clinical manifestations of varicella-zoster virus infection: Herpes zoster. Uptodate.com. Updated Oct 30, 2011.
Miravalle A. Ramsay Hunt Syndrome. Emedicine.com. Updated Feb 6, 2012.
Uscategui T, Doree C, et al. Antiviral therapy for Ramsay Hunt Syndrome (herpes zoster oticus with facial palsy) in adults. The Cochrane Collaboration. Published online Jan 21, 2009.