What Is Ramsay Hunt Syndrome?
Ramsay Hunt syndrome is a shingles outbreak that affects the facial nerve near one ear. It happens when the varicella-zoster virus, the virus that causes chickenpox, reactivates years later. The main signs are a painful, blistering rash in or around the ear (sometimes in the mouth) and weakness or paralysis on the same side of the face. It can also cause ear pain, hearing loss, ringing in the ear and vertigo. Doctors also call it herpes zoster oticus. According to the Mayo Clinic, treatment started within about three days of symptoms gives the best chance of recovery, so anyone with facial weakness and an ear rash should see a doctor promptly.
In June of 2022, pop star Justin Bieber published an Instagram video announcing he had Ramsay Hunt syndrome. In the video, he explained that half of his face is paralyzed and demonstrated that his right eye won’t blink and only half of his mouth can smile.
This prompted many people to look into this otherwise rarely discussed neurological disorder. Here’s what you should know about the syndrome.
Ramsay Hunt is a rare condition that can cause several symptoms, including paralysis on one side of the face, hearing loss, and pain. The symptoms happen because the reactivated virus inflames the facial nerve near the inner ear. Treatment usually involves antiviral medicine and steroids to reduce the inflammation, prescribed by a doctor.
The syndrome is caused by the varicella-zoster virus, the same virus that causes chickenpox and shingles. The condition gets its name from James Ramsay Hunt, who first described it in 1907. There are actually three separate neurological disorders named “Ramsay Hunt syndrome” because James Ramsay Hunt was the first to document them. The technical name for the condition described here is Ramsay Hunt syndrome type 2.
While most people who have had chickenpox will never develop this syndrome, the risk increases as people get older. It is most common in adults over 60 and is rare in children. Only a small share of people who have had chickenpox ever develop it.
Symptoms
Ramsay Hunt syndrome typically causes a sudden outbreak of a chickenpox-like rash with sores in the ear canal or on the face. This is accompanied by facial paralysis or weakness on the side of the face where the sores are located.
These two symptoms, a painful rash or sores in or near the ear and facial weakness or paralysis, are the main hallmarks of Ramsay Hunt. These two symptoms don’t always occur together at the same time. In most cases, only one side of the face is affected.
Other symptoms can include:
- Ear pain, loss of hearing, and/or tinnitus
- Vertigo
- Change or loss of taste
- Dry mouth
So what exactly does it feel like to have Ramsay Hunt?
When it comes to the nerve palsy affecting facial muscles, your face feels stiff or weak. As onset occurs, controlling your facial muscles feels like it requires an undue amount of strength. Once paralyzed, you can’t smile, blink, or move your eyebrow on the affected side of your face. Because part of your mouth is paralyzed, speaking may be difficult and slurred.
The rash usually spreads in and around your ear. This rash is red, itchy, painful, and often full of blisters. Sometimes this rash is also present in parts of the mouth.
Sometimes additional symptoms develop in the affected ear. This includes tinnitus, which is a ringing or buzzing noise that comes in waves. With tinnitus, the sound isn’t triggered by external noise, and can include ringing, buzzing, clicking, hissing, or roaring. (See: Can Ear Wax Cause Tinnitus?)
Some people affected with the syndrome experience hyperacusis, which is when noises sound much louder than normal, sometimes leading to extreme discomfort. Finally, those with Ramsay Hunt may lose their ability to hear in the affected ear altogether.
Nausea, vertigo, and dizziness are also symptoms that develop in people with this condition. Vertigo is the feeling that you or your surroundings are moving when there isn’t any actual movement taking place. It’s often described as a spinning sensation. This is caused by the varicella-zoster virus damaging the vestibular nerve in the inner ear, which is responsible for balance. Vertigo can make it difficult to maintain balance and may cause nausea or vomiting.
As with any neurological disorder, and especially those affecting your appearance, individuals who suffer from permanent symptoms of Ramsay Hunt are prone to depression, anxiety, and other mental illnesses.
How is Ramsay Hunt Syndrome Diagnosed?
This syndrome is diagnosed through a careful examination and by looking at the patient’s history. Clinicians look for specific symptoms, such as facial palsy and a rash. A virus can be found in saliva, tears, and blood, but it is not necessary to make a diagnosis of Ramsay Hunt syndrome.
Since specific symptoms of the syndrome (rash, pain, paralysis) don’t always occur at the same time, diagnosing the syndrome can be difficult. In some cases, facial paralysis is misdiagnosed as Bell’s palsy.
Recovery and Long-Term Effects
Ramsay Hunt is treated with antiviral drugs and corticosteroids. The best results occur when antiviral drugs are taken within three days of onset. But some people don’t fully recover, even with adequate and timely treatment. In those cases, they may have some permanent facial paralysis or hearing loss.
Depending on individual symptoms, further treatment may be required. This can include pain medication, anti-seizure medicine, and vertigo suppressants.
People with facial paralysis have to be careful to not injure their cornea. The inability to close the eye properly can leave the cornea exposed and dry. Artificial tears and other ointments may be needed to lubricate the cornea and protect it from damage.
How Pervasive is Ramsay Hunt Syndrome?
Ramsay Hunt may affect anybody who has had chickenpox. The National Organization for Rare Disorders estimates that it affects about 5 in every 100,000 people in the United States each year. It’s a rare condition, but it’s more common in older adults.
The medical community concedes that it’s difficult to know exactly how pervasive the syndrome is. In mild cases it may go undiagnosed, and in other cases it may be misdiagnosed. The research of neurologist James M. Gilchrist has led many to believe that Ramsay Hunt makes up 20% of cases diagnosed as Bell’s palsy.
According to additional research, Ramsay Hunt syndrome accounts for 16% of facial palsies in children and 18% in adults.
The Bottom Line
Ramsay Hunt is a rare condition that can have serious consequences, such as permanent facial paralysis and hearing loss. Caused by the varicella-zoster virus, which also causes chickenpox and shingles, this medical condition continues to be researched to develop a better understanding of its prevalence, long-term effects, and treatment. Early diagnosis and treatment is critical for the best possible outcome.
Frequently Asked Questions
Does Ramsay Hunt syndrome go away?
For many people, yes. Most people recover some or all of their facial movement and hearing, and the chances are better when treatment starts within a few days of symptoms. Some people are left with lasting facial weakness or hearing loss, especially when treatment is delayed.
Can you die from Ramsay Hunt syndrome?
Ramsay Hunt syndrome is not usually life-threatening. The main risks are lasting facial paralysis, hearing loss, eye damage from not being able to close the eyelid, and long-lasting nerve pain. Anyone with symptoms should be seen by a doctor promptly.
Is Ramsay Hunt syndrome contagious?
The syndrome itself does not spread, but the virus in the blisters can give chickenpox to someone who has never had chickenpox or the chickenpox vaccine. Until the blisters crust over, the Mayo Clinic advises avoiding contact with people at higher risk, such as newborns, pregnant people and anyone with a weakened immune system.
How is Ramsay Hunt syndrome different from Bell’s palsy?
Both cause sudden weakness on one side of the face. Ramsay Hunt syndrome is caused by the shingles virus and usually comes with a painful rash in or around the ear, and it is more likely to affect hearing and balance. Bell’s palsy has no rash. Because the rash can appear after the facial weakness, a doctor may need to examine the ear more than once.
Can Ramsay Hunt syndrome be prevented?
There is no specific way to prevent it, but the shingles vaccine lowers the risk of shingles, which is what Ramsay Hunt syndrome is. The CDC recommends shingles vaccination for adults 50 and older and for some younger adults with weakened immune systems. Ask your doctor whether it is right for you.
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