Pop superstar Justin Bieber brought attention to a little known neurological disorder earlier this month when he shared news of his Ramsay Hunt syndrome diagnosis, telling fans and viewers in an Instagram video that a virus attacking the nerve in his ear and face is causing partial paralysis.
Ramsay Hunt syndrome is a rare neurological disorder, occurring in about five in 100,000 people. Ramsay Hunt syndrome (herpes zoster oticus) occurs when a shingles outbreak affects the facial nerve near one of your ears. In addition to the painful shingles rash, Ramsay Hunt syndrome can cause facial paralysis and hearing loss in the affected ear. Side effects can include ringing in the ears or noises sounding louder than they actually are.
Ramsay Hunt syndrome is caused by the same virus that causes chickenpox or shingles. If you’ve had chickenpox as a child, the virus lives dormant in your body.
Zara Khan, MD, a neurologist with Hartford HealthCare’s Ayer Neuroscience Institute, said the syndrome is named after a physician who diagnosed the disparate symptoms as one syndrome among soldiers during the First World War. And while it is considered rare, she said it is the second most common cause of facial weakness not caused by a stroke.
One risk factor for developing Ramsay Hunt is stress, which can “wake up” the virus that already exists in the body, Khan said.
Prompt treatment of Ramsay Hunt syndrome with steroids and antivirals can reduce the risk of complications, which can include permanent facial muscle weakness and deafness. Around 75 percent of Ramsay Hunt syndrome patients recover completely. Pain medications can be prescribed to alleviate the pain often associated with the skin blisters.
Complications of Ramsay Hunt syndrome may include:
- Permanent hearing loss and facial weakness. For most people, the hearing loss and facial paralysis associated with Ramsay Hunt syndrome is temporary. However, it can become permanent.
- Eye damage. The facial weakness caused by Ramsay Hunt syndrome may make it difficult for you to close your eyelid. When this happens, the cornea, which protects your eye, can become damaged. This damage can cause eye pain and blurred vision.
- Postherpetic neuralgia. This painful condition occurs when a shingles infection damages nerve fibers. The messages sent by these nerve fibers become confused and exaggerated, causing pain that may last long after other signs and symptoms of Ramsay Hunt syndrome have faded.