Ramsay Hunt syndrome (RHS) is a rare neurological disorder caused when the varicella zoster virus (VZV) reactivates in one of the facial nerves near the ear. Herpes zoster oticus is characterized by a painful unilateral rash of fluid-filled blisters affecting one ear and facial weakness or paralysis on the same side. Diagnosing the condition can be difficult because the rash and paralysis do not always appear together, and sometimes the rash is missing entirely (zoster sine herpete).
Named after James Ramsay Hunt, the physician who first described the disorder in 1907, RHS is the second most common cause of atraumatic peripheral facial paralysis after Bell’s palsy. The syndrome affects men and women equally, and it is estimated that 5 out of every 100,000 people develop RHS annually in the United States.
Ramsay Hunt syndrome patients usually exhibit the painful red and blistering rash associated with shingles on the outer part of the ear and external ear canal. The rash can also affect the mouth, soft palate, and upper portion of the throat.
Nerve palsy affecting the facial muscles can be weak or stiff in RHS patients. Some may experience difficulty with simple gestures like smiling, closing the eye on the affected side, and wrinkling their forehead. They may also slur their speech.
Other symptoms of RHS include tinnitus, hearing loss, and intense internal ear pain that spreads to the neck. Ear abnormalities like vertigo, sensorineural hearing loss, and hyperacusis can add to the patient’s discomfort. While hearing loss is usually temporary, it may become permanent in rare cases.
RHS can also cause nausea and vomiting, loss of taste, dry mouth, and dry eyes. Postherpetic neuralgia is a potential complication. Only about half of Ramsay Hunt patients recover completely.
As with other cases of shingles, prompt diagnosis and management of RHS is recommended to improve outcomes and prevent long-term complicationsthat include deafness and permanent facial muscle weakness. Antivirals like acyclovir (Zovirax), famciclovir (Famvir), and valacyclovir (Valtrex), along with corticosteroids like prednisone, should be initiated within 72 hours of the onset of symptoms.
Other RHS symptoms may require additional treatment, such as pain medication, carbamazepine to reduce neuralgic pain, and antihistamines and anticholinergics to suppress vertigo. Special care to prevent corneal injury is needed in patients who cannot close their eye, making it susceptible to foreign body irritation and abnormal drying. The use of an eye patch, lubricating ointments, and artificial tears are recommended in these cases.
The only way to protect against RHS is through vaccination against shingles. Since 2017, the CDC’s Advisory Committee on Immunization Practices has recommended Shingrix as the first line of defense against shingles in adults aged 50 and older. Alternatively, Zostavax may be used under certain circumstances, including if Shingrix is unavailable or if the person is allergic to Shingrix or prefers Zostavax.