Medical Edge Newspaper Column

Ramsay Hunt Syndrome Results from Reactivation of Varicella-zoster Virus

November 13, 2009
Dear Mayo Clinic:
What causes Ramsay Hunt syndrome? Is it somehow connected to shingles?

Answer:
Ramsay Hunt syndrome — also called herpes zoster oticus — is an infection of the facial nerve. This infection causes a severe form of Bell's palsy, or paralysis of the facial muscles on one side. Ramsay Hunt syndrome is similar to shingles. Both result from reactivation of the varicella-zoster virus, which also causes chickenpox.

Varicella-zoster is part of a common group of viruses called herpes viruses, which include the viruses that cause cold sores, chickenpox and genital herpes. Many herpes viruses can lie hidden in the nervous system after the first infection and remain dormant for years before causing another infection.

Thus, years after a person has chickenpox, the varicella-zoster virus can reactivate. Researchers don't clearly understand how or why the varicella-zoster virus re-emerges.

If the virus reactivates and infects the facial nerve, the result is Ramsay Hunt syndrome. The onset of Ramsay Hunt syndrome can be frightening. Because its symptoms include severe weakness of one side of the face and pain, some people who have this syndrome fear they're having a stroke. Other symptoms of Ramsay Hunt may include a painful red rash with fluid-filled blisters on the eardrum, external ear canal, the outside of the ear, roof of the mouth or tongue, as well as ear pain, hearing loss and a sensation of spinning or moving (vertigo).

Shingles (herpes zoster) is caused by reactivation of the same virus. Shingles results when a spinal nerve is affected instead of the facial nerve. Symptoms of shingles usually affect only a small section on one side of the body. They can include pain, burning, numbness or tingling; a red rash with fluid-filled blisters that break open and crust over; and itching. The pain often starts a few days before the rash develops.

Anyone who had chickenpox can later develop shingles or Ramsay Hunt syndrome. These conditions are more common in older adults, typically affecting people older than 40.

Ramsay Hunt syndrome cannot be prevented once the varicella-zoster virus has been reactivated in the body. But, the varicella virus vaccine (Varivax) can reduce the risk of getting the virus. This vaccine is a routine childhood immunization, given between ages 12 months and 18 months. The vaccine is also recommended for older children and adults who've never had chickenpox. The varicella virus vaccine prevents chickenpox in most people. Chickenpox that might develop after vaccination is typically less severe.

The Food and Drug Administration has also approved a vaccine (Zostavax) to help prevent shingles and other conditions related to the varicella-zoster virus, such as Ramsay Hunt syndrome, in adults age 60 and older. This vaccine is given as a single injection, preferably in the upper arm. The most common side effects are redness, pain and swelling at the injection site; itching and headache.

Ramsay Hunt syndrome is often treated with medication to reduce inflammation and anti-viral agents to fight the virus. Sometimes, however, the syndrome can lead to permanent facial muscle weakness and hearing loss. Other complications can include unusual facial movements due to severe nerve damage; difficulty closing the eyelid on the side of the face that's affected; and postherpetic neuralgia, which is persistent pain in the area of sensation supplied by the damaged nerve fibers long after others signs and symptoms of Ramsay Hunt syndrome have faded.

If you experience symptoms of Ramsay Hunt syndrome, make an appointment to see your doctor as soon as possible. Prompt diagnosis and treatment may help prevent long-term complications.

— Eric Sorenson, M.D., Neurology, Mayo Clinic, Rochester, Minn.