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 condition is caused by a viral infection of the facial nerve, which can damage the nerve and lead to these symptoms. Treatment for this syndrome typically involves antiviral medications and steroids to reduce inflammation. In some cases, surgery may be necessary to repair the damage to the facial nerve.
The syndrome is caused by the varicella-zoster virus – the same virus that causes chickenpox. 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. The condition is most common in people over the age of 50. In fact, the average age of people diagnosed with Ramsay Hunt is 60. Less than 1% of people infected with chicken pox develop thissyndrome.
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 symptoms — a painful rash/sores in or near the ear and facial weakness/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
- 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. Most estimates claim that 0.005% of the American population develops the syndrome. 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.