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Ramsay Hunt syndrome is a rare condition caused by the varicella-zoster virus, the same virus that causes chickenpox and shingles.
The condition has been in the news since singer Justin Bieber announced that he has partial facial paralysis from the virus.
Chickenpox and shingles vaccines are recommended for children and adults and can protect against Ramsay Hunt syndrome.
You might not have heard of Ramsay Hunt syndrome until pop singer Justin Bieber announced last year that he was suffering partial facial paralysis from this condition. Bieber highlighted that his symptoms included not being able to blink one of his eyes, smile with half of his face or move one of his nostrils.
But what is this rare, neurological condition?
“Ramsay Hunt syndrome, or herpes zoster oticus, is a rare disease caused by the varicella-zoster virus – the same virus that causes chickenpox in children and shingles in adults,” says Michael Yong, M.D., ear, nose and throat (ENT) specialist at Providence’s Pacific Neuroscience Institute. “Even after your chickenpox or shingles clears up, the virus can still live in your nerves and can reactivate, causing neurological symptoms.”
Ramsay Hunt syndrome is rare in children and, similar to shingles, generally affects adults over age 60. It is different than Bell’s palsy, another kind of facial palsy. Patients with Ramsay Hunt syndrome generally have more severe facial paralysis and a lower likelihood of complete recovery. “It has a high incidence of permanent facial paralysis,” says Dr. Yong.
When the virus causes an outbreak of shingles near the facial nerve, which controls movement and sensing for many of the muscles of the face, it can cause Ramsay Hunt syndrome. The most telling signs of Ramsay Hunt syndrome are a painful rash with fluid-filled blisters around one ear (including the inner ear and ear canal) and facial paralysis or facial weakness on the same side. Other symptoms may include:
- Difficulty blinking or closing one eye
- Dry eyes and mouth
- Ear pain
- Hearing loss or tinnitus (ringing in the ears)
- Loss of taste
- Vertigo (a feeling of spinning)
Left untreated, Ramsay Hunt syndrome can cause permanent hearing loss, nerve damage and eye damage.
What options are available to prevent Ramsay Hunt syndrome?
Vaccination against the varicella virus is the best defense against Ramsay Hunt syndrome. The chickenpox vaccine was first available in 1995, with the first shingles vaccine following in 2006. Fortunately, this rare condition may become even rarer in the future: The Centers for Disease Control and Prevention estimates that more than 90% of children under age two have been vaccinated against chickenpox.
How is Ramsay Hunt syndrome diagnosed?
Ramsay Hunt syndrome is diagnosed based on medical history, including, chiefly, if you have ever had chickenpox. Because the symptoms of Ramsay Hunt syndrome can be so specific, your doctor may be able to complete a diagnosis with a physical exam and discussion of these signs and symptoms. They may also confirm your diagnosis with a sample of fluid from one of your blisters. Other tests may include:
- Blood tests for the varicella-zoster virus
- Magnetic resonance imaging (MRI) of the head
- Nerve conduction tests to check the extent of facial paralysis in the facial muscles
Treatment for Ramsay Hunt syndrome
While it may take weeks or months for Ramsay Hunt syndrome symptoms to go away, some treatments may help decrease the time to remission and ease symptoms:
- Antiviral medications (e.g., acyclovir or valacyclovir): help your immune system fight the varicella virus
- Corticosteroids (e.g., prednisone): help reduce inflammation
- Pain relievers: ease discomfort from Ramsay Hunt rash and facial nerve pain
“With treatment, we’re trying to target both the inflammation of the nerve, and the virus that’s underlying the problem,” says Dr. Yong.
Other remedies, including eye patches and eye drops to relieve eye dryness, or keeping your rash clean and cool, can help relieve symptoms.
Learn more about testing and treatment options for Ramsay Hunt syndrome and other rare neurological disorders at the Providence Neuroscience Institute.
Michael Yong, M.D., ear, nose and throat (ENT) specialist at Providence’s Pacific Neuroscience Institute
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