Ramsay Hunt Syndrome

Ramsay Hunt syndrome, also known as herpes zoster oticus or geniculate ganglion herpes zoster, is a late complication of varicella-zoster virus (VZV) infection, resulting in inflammation of the geniculate ganglion of cranial nerve VII. The syndrome is named after James Ramsay Hunt (1872-1937), an American neurologist and Army officer in World War I who described three different syndromes, the most famous of which is the second, which is discussed herein as "Ramsay Hunt syndrome." Early stages of VZV infection cause fever and diffuse vesicular rash, a condition that is commonly referred to as chickenpox. After the initial infection, the virus will often remain dormant in the body. Subsequent reactivation of the virus causes a "zoster" or "herpes zoster" phenomenon. This syndrome consists of pain and a vesicular rash along the involved nerve's distribution, typically corresponding to a single dermatome. The distribution and associated symptoms depend on the nerve involved. Less than 1% of zoster cases involve the facial nerve and result in Ramsay Hunt syndrome.

Although the classic triad of Ramsay Hunt syndrome is ipsilateral facial paralysis, otalgia, and a vesicular rash, there is significant variability in clinical presentation, with some patients demonstrating facial paralysis before the rash or, sometimes, no rash at all. In the latter, the patient's chief complaints are severe ear pain and facial weakness; this variant is known as zoster sine herpete and can be very difficult to clinically distinguish from Bell's palsy. Zoster sine herpete has been reported to comprise up to 30% of Ramsay Hunt cases. If a rash is present, it may be frankly vesicular or maculopapular, and can involve the affected side of the face, scalp, palate, and tongue. Additional symptoms that may be reported include a change in taste sensation, dry eye, tearing, hyperacusis, nasal obstruction, and dysarthria. Hearing loss, tinnitus, and vertigo can be seen with involvement of the vestibulocochlear nerve, and hoarseness or aspiration may indicate involvement of the vagus nerve.