Chances are if you were born after 1995 and received the vaccine, chickenpox isn’t a big concern for you these days. Yet, entertainer Justin Bieber’s recent diagnosis of Ramsay Hunt syndrome (RHS) or herpes zoster oticus, has drawn concern — especially its similarity to Bell’s palsy.
RHS is a very rare viral condition linked to chickenpox and the shingles virus that causes facial weakness or paralysis. It left Bieber unable to blink or smile on one side of his face.
Read on to understand more about Ramsay Hunt, its symptoms, diagnosis and treatment, and how it differs from a common cause of facial paralysis, Bell’s palsy.
What is Ramsay Hunt syndrome?
“Ramsay Hunt is caused by the reactivation of the varicella-zoster virus, which is more commonly known as the chickenpox and shingles virus,” said Heather Coffman, MD, an ear, nose and throat specialist at Banner – University Medicine North in Tucson, AZ. “It lies dormant in your body after a previous exposure.”
For millions of Americans who had chickenpox in childhood or young adulthood, the virus may have been treated or gone away, but it remains and lives “sleeping” in the nerves. Then, for reasons that aren’t well understood yet, the virus can “reawaken” and affect the facial and hearing nerves — even the skin around the ear.
RHS is very rare, occurring in about 5 in 100,000 people, but anyone who has had chickenpox in the past could develop the syndrome if the virus is reactivated. If you’ve been vaccinated, you’re protected against it.
What are the symptoms of Ramsay Hunt syndrome?
Most often, RHS affects the facial nerve which controls your facial muscles that help you smile, frown, wrinkle your nose and raise your eyebrows. In addition to facial movement and facia expressions, it also partially controls the sensation of taste on the inside of your mouth.
“When the virus is reactivated, it can damage the facial nerve, causing unilateral facial paralysis, or paralysis on one side of the face, changes in sense of taste and a painful rash in the ear and/or tongue,” Dr. Coffman said.
RHS can also affect the cranial nerve responsible for hearing and balance. When this nerve is damaged, people with Ramsay Hunt syndrome can experience hearing loss, ringing in the ear (tinnitus), vertigo and nausea and vomiting.
“Hearing loss is usually temporary and occurs in about 50% of people,” Dr. Coffman said.
How does Ramsay Hunt syndrome differ from Bell’s palsy?
The symptoms of RHS can appear differently from person to person and can mimic another condition called Bell’s palsy. While both can cause facial paralysis, Bell’s palsy doesn’t have a tell-tale rash inside the ear.
“Bell’s palsy is a sudden or rapid onset of facial paralysis or weakness, which can develop over hours to a day or two,” Dr. Coffman said. “It tends to cause milder weakness of the face and improves faster than Ramsay Hunt.”
In comparison to Bell’s palsy, RHS is more likely to cause permanent facial asymmetry, where the face doesn’t look balanced.
The causes of facial nerve palsy and paralysis with Bell’s palsy are unknown, but they could be viral or autoimmune-related.
How is Ramsay Hunt diagnosed?
The diagnosis of RHS can be difficult because the symptoms don’t always develop at the same time and may mimic those of other conditions.
Your health care provider will usually diagnose RHS by looking for signs of weakness in your face and a blister-like rash. They may also do additional testing, including blood tests.
How is Ramsay Hunt syndrome treated?
RHS can cause severe problems and even long-term, permanent damage if it’s not treated quickly. “Treatment within three days is thought to have the most benefit,” Dr. Coffman said.
The treatment for RHS is similar to the treatment of Bell’s palsy and may include antiviral medications and corticosteroids. Steroids, such as prednisone, are used to reduce swelling and antivirals are given to reduce or minimize nerve damage.
Pain medication may be prescribed to help with the pain. If your eye on the affected side doesn’t close, you may need to see an ophthalmologist, or eye doctor, for additional care.
“If the eye does not close (due to paralysis), it is very important to keep the eye moist to prevent corneal injury,” Dr. Coffman said. “Treatment for the eye includes artificial tears (eye drops), lubricating ointments, an eye patch and taping the eye closed at night.”
Other treatment options may include physical therapy and Botox injections to relax overactive facial muscles and improve facial symmetry.
What is the outlook for Ramsay Hunt syndrome?
With early diagnosis and treatment, typically within 72 hours (three days), there is a high rate of complete to near complete recovery, around 70%,” Dr. Coffman said. “If treatment is delayed, there is less of a chance of a complete recovery.”
If you don’t have much damage to the nerves, you should get better within a few weeks. If damage is more severe, you may not fully recover – even after a few months.
Get vaccinated
Preventing the varicella-zoster virus is available through a chickenpox vaccine in children and a shingles vaccine for people 50 years of age and older. These vaccinations can greatly reduce your risk of getting this viral infection, which can reduce the chances of RHS.
Bottom line
Ramsay Hunt syndrome is a very rare but serious virally induced condition that can cause facial weakness and paralysis, rash and a host of other symptoms.
If you think you are experiencing symptoms of Bell’s palsy or RHS, don’t wait it out. See your health care provider right away. RHS can be dangerous if left untreated, but early care and treatment can greatly improve the chances of recovery.
To find a Banner Health specialist near you, visit bannerhealth.com.
