What is Ramsay Hunt syndrome?
Quick treatment of this rare condition can prevent complications, like permanent facial paralysis and hearing loss.
Sudden facial paralysis indicates a few conditions — some more serious than others. If it’s accompanied by other symptoms, like a rash in or around your ear, it’s most likely Ramsay Hunt syndrome. This rare neurological disorder is caused by the varicella zoster virus (the same one that causes chickenpox in children and shingles in adults).
“Ramsay Hunt syndrome is rare, and for most patients it’s not a serious illness,” says Mohammed Majeed, MD, a neurologist at Geisinger.
Symptoms of Ramsay Hunt syndrome
Ramsay Hunt syndrome is a form of shingles. Shingles typically causes a rash around your midsection, but with Ramsay Hunt syndrome, the rash can appear in or around your ear. The rash is usually painful, with red, fluid-filled sacs.
The condition can also cause facial paralysis or weakness in the facial muscles on the same side as the rash. It can be difficult to blink or smile, and sometimes it can cause slurred speech.
Other symptoms include:
- Ringing in the ear, or tinnitus
- Ear pain
- Hearing loss
Diagnosis can be tricky, especially since all the characteristic symptoms don’t always appear at the same time. Facial paralysis can be mistaken for Bell’s palsy, a sudden weakness of muscles on one side of the face. The actual cause for Bell’s palsy is unknown, but could be the result of a viral infection, too.
Facial paralysis can also be mistaken for stroke, which has more serious implications.
No matter the cause, if you’re having facial paralysis or facial weakness, seek care as soon as possible.
Causes of Ramsay Hunt syndrome
If you’ve had chickenpox, it’s possible to develop Ramsay Hunt syndrome, though the condition is rare. It affects 5 out of every 100,000 people every year in the United States, according to the National Organization for Rare Disorders.
Ramsay Hunt syndrome is caused by the reactivation of the dormant varicella zoster virus. The varicella zoster virus causes chickenpox, and the initial infection is highly contagious. Cases of chickenpox can range from mild to severe, and it can be dangerous for adults who have never had it.
Once you’ve recovered from chickenpox, the virus is still inside you, though dormant. If the virus reactivates years later, it causes shingles.
“Those who are immunocompromised or have a weakened immune system are especially at risk,” says Dr. Majeed. “Advanced age is also a contributing factor.”
Shingles itself isn’t contagious, so you can’t catch it from someone else. However, if you’ve never had chickenpox and you come into contact with someone’s open shingles blister, you can contract chickenpox.
Like shingles, someone can’t pass Ramsay Hunt syndrome to you. But they can pass the virus that causes chickenpox to you if you’ve never had it or haven’t been vaccinated against it.
Treatment for Ramsay Hunt syndrome
Treatment for Ramsay Hunt syndrome includes taking antiviral medications and steroids to reduce inflammation.
If you can’t close your affected eye, lubricate it to keep it from getting dry. And be sure to protect it from debris that can damage your cornea.
“Duration of the condition can vary,” says Dr. Majeed. “Some people recover quickly, within a few weeks, and others can have permanent complications. Getting treatment quickly at the onset of symptoms is key.”
Symptoms of Ramsay Hunt syndrome should eventually resolve for those who receive treatment early. Occasionally, the condition can cause permanent facial paralysis or hearing loss.
It’s possible, though extremely rare, for the virus to affect the brain covering (causing meningitis) or the brain tissue itself (causing encephalitis). And those can be fatal if not treated immediately. In other instances, the virus can affect the spinal cord, resulting in a condition called myelitis.
As always, even better than treatment is prevention. Getting vaccinated for varicella zoster as a child or if you’ve never had chickenpox, or getting the shingles vaccine as an adult (over age 50) can help prevent serious illness.
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