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Ramsay Hunt Syndrome

Definition :
Ramsay Hunt syndrome — also called herpes zoster oticus — is an infection of your facial nerve that's accompanied by a painful rash and facial muscle weakness, among other signs and symptoms.

The cause of Ramsay Hunt syndrome is varicella-zoster virus, the same virus that causes chickenpox. After chickenpox heals, the virus lies dormant in your nerves. Years later, it may reactivate. If varicella-zoster virus reactivates and infects your facial nerve, the result is Ramsay Hunt syndrome.

The onset of Ramsay Hunt syndrome can be frightening. Facial weakness, pain and dizziness may cause some people to fear they're having a stroke. But, despite its unnerving symptoms, Ramsay Hunt syndrome often can be effectively treated. Sometimes, however, Ramsay Hunt syndrome can lead to permanent muscle facial weakness and hearing loss. Prompt treatment can reduce the risk of these and other long-term complications.

Causes:
The cause of Ramsay Hunt syndrome is a reactivation of the varicella-zoster virus — the same virus that causes chickenpox.

Varicella-zoster is part of a group of viruses called herpes viruses, which includes the viruses that cause cold sores and genital herpes. Many of these viruses can lie hidden in your nervous system after the first infection

and remain dormant for years before causing another infection.

If your immune system doesn't destroy all the varicella-zoster virus during the initial infection, the remaining virus may reactivate later and infect your facial nerve — producing Ramsay Hunt syndrome. Reactivation of this virus also can cause other disorders, including shingles (herpes zoster). Researchers don't clearly understand how or why the varicella-zoster virus re-emerges.

Risk Factors:
Anyone who has had chickenpox can develop Ramsay Hunt syndrome. But, it's more common in older adults, typically affecting people older than 40. Ramsay Hunt syndrome is rare in children.

Ramsay Hunt syndrome isn't contagious. However, reactivation of the varicella-zoster virus can cause chickenpox in people you come in contact with if they haven't had chickenpox before. The infection can be serious for people with immune system deficiencies.

Until the rash blisters scab over, avoid physical contact with :

  • Anyone who's never had chickenpox
  • Anyone who has a weak immune system
  • Newborns
  • Pregnant women (because chickenpox infection can be dangerous for a developing baby)

When to seek medical advice :
If you experience weakness on one side of your face or if you develop a rash in or around your ear or in your mouth along with facial weaknesses, call your doctor for an evaluation. Ramsay Hunt syndrome isn't a condition that requires emergency care. But, diagnosis and treatment within three days of the start of signs and symptoms may help prevent long-term complications.

Symptoms :
Signs and symptoms of Ramsay Hunt syndrome include :

  • A painful red rash with fluid-filled blisters on your eardrum, external ear canal, the outside of your ear, the roof of your mouth (palate) or your tongue
  • Facial weakness (palsy) on the same side as the affected ear
  • Difficulty closing one eye
  • Ear pain
  • Hearing loss
  • Ringing in your ears (tinnitus)
  • A sensation of spinning or moving (vertigo)
  • A change in taste perception or loss of taste

Diagnosis:
Doctors often can identify Ramsay Hunt syndrome based on medical history, a physical exam and the disorder's distinctive signs and symptoms. To confirm the diagnosis, your doctor may take a sample of fluid from one of the rash blisters in your ear and perform a polymerase chain reaction (PCR) test on it. This test can detect the varicella-zoster virus. A PCR test also can be done on a blood or tear sample. But, testing fluid from your ear offers more reliable results.

Complications :
If treated within the first three days, most people with Ramsay Hunt syndrome don't suffer long-term complications. However in some cases, even with prompt treatment, Ramsay Hunt may result in permanent hearing loss and facial weakness.

Other possible complications of Ramsay Hunt syndrome include :

  • Unusual facial movements. A condition called synkinesis may develop if the infection severely damages your facial nerve and the nerve grows back to the wrong place. This can cause inappropriate nerve responses, such as blinking or tears when you're talking, laughing or chewing.
  • Eye damage. The facial weakness caused by Ramsay Hunt syndrome may make it difficult for you to close your eyelid on the side of your face that's affected. Incomplete eyelid closure can lead to damage of the protective dome of clear tissue over the front of your eye (cornea). This damage can cause eye pain and blurred vision.
  • Effects on other parts of your body. In rare cases, the varicella-zoster virus may spread to other nerves, or to your brain or spinal cord, causing headaches, back pain, confusion, lethargy and limb weakness.
  • Postherpetic neuralgia. Postherpetic neuralgia may develop as a result of nerve fiber damage. Damaged fibers aren't able to send messages from your skin to your brain as they normally do. Instead, the messages become confused and exaggerated, causing pain that may persist long after others signs and symptoms of Ramsay Hunt syndrome have faded.

Treatment:
Prompt treatment of Ramsay Hunt syndrome can ease pain and decrease your risk of long-term complications. To treat Ramsay Hunt, your doctor may prescribe the following medications :

  • Antiviral medication, such as acyclovir (Zovirax), famciclovir (Famvir) or valacyclovir (Valtrex)
  • Corticosteroids, such as prednisone, to reduce swelling and pain
  • Diazepam (Valium), to relieve vertigo
  • Pain relievers

If facial weakness remains after treatment, physical therapy involving facial exercises may help you improve or regain control of your facial muscles.

If you have trouble closing one of your eyes because of facial weakness, your doctor may recommend an injection of botulinum toxin (Botox) into your upper eyelid, allowing it to close and protect your eye.

Depending on the extent of damage to your facial nerve, recovery from Ramsay Hunt syndrome can take from several weeks to several months. If damage is severe, or if treatment is delayed, full recovery may not be possible.

Prevention:
There's no way to prevent Ramsay Hunt syndrome once the varicella-zoster virus is in your body. But, the varicella virus vaccine (Varivax) can reduce your risk of getting the virus in the first place. This vaccine is a routine childhood immunization, given between ages 12 months and 18 months. The vaccine is also recommended for older kids and adults who've never had chickenpox. The varicella virus vaccine prevents chickenpox for most people. If chickenpox does develop after vaccination, it's typically less severe.

The Food and Drug Administration (FDA) 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.

 
Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.
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