Justin Bieber Suffering From New Facial Paralysis, What Is Ramsay Hunt Syndrome?

When you sing in a concert, you kind of need the ability to move your face, unless you happen to part of a ventriloquist act. That’s why an onset of partial facial paralysis has prompted Grammy Award winner Justin Bieber to postpone three shows on his current “Justice World” tour. On Friday, Bieber revealed on Instagram that he’s suffering from Ramsay Hunt syndrome. The following tweet from RapTV included a clip of Bieber’s Instagram video:

Yeah, having such symptoms while trying to perform wouldn’t do anyone “justice” on stage, including Bieber. In the video, Bieber pointed out, “As you can see this eye is not blinking. I can’t smile on this side of my face. This nostril will not move.” He added, “So there’s full paralysis on this side of my face.” Leave it to Bieber to be so forthcoming about his diagnosis to raise more needed awareness about Ramsay Hunt syndrome.

Now, Ramsay Hunt syndrome may sound like a search for celebrity chef Gordon Ramsay or Ramsay having some issues with a bottle of ketchup. But it is actually a neurological disorder caused by reactivation of the varicella-zoster virus (VZV) in your body. You may think that you’ve never gotten down with VZV. But in actuality, it’s probably a “yeah, you know me” virus. VZV is what causes chickenpox. So if you’ve ever had chickenpox, your body knows VZV.

The trouble with VZV is that it can be a bit like someone who caused a ruckus while holding a high political office and subsequently retreated to a resort, only to later resurface to cause more problems. After giving you chickenpox, VZV can actually have some nerve, so to speak. It may hide out in some of your nerve cells, specifically either your cranial nerves or your dorsal root ganglia, remaining dormant for years until one day some type of stress or weakening of your immune system allows the virus to reactivate and cause what’s called herpes zoster. Although herpes zoster may sound like a sexually transmitted infection (STI), it’s actually quite different from herpes simplex. So when you tell your friend that his or her upcoming blind date is suffering from herpes zoster, make sure that you include the word zoster. Otherwise, that may lead to some misunderstanding.

A more common name for herpes zoster is “shingles,” which sounds a bit like what you would say when you have too many potato chips in your mouth and try to mention the name of that 1992 rom-com movie starring Bridget Fonda and Matt Dillon. As I recently described for Forbes, shingles can result in a very painful rash affecting one side of your body. When herpes zoster specifically affects your cranial nerves V, VII, VIII, IX, X, or XII and results in paralysis of your face, you can refer to it as Ramsay Hunt syndrome. That’s why Ramsay Hunt syndrome is otherwise known as herpes zoster oticus or geniculate ganglion herpes zoster, just in case you want to employ more difficult-to-pronounce words at a cocktail party or need to use a “z” in a game of Scrabble. Ramsey Hunt syndrome is named after James Ramsay Hunt, MD, a neurologist who was the first person on record to describe it back in 1907.

Again a key distinguishing feature of Ramsey Hunt syndrome is that symptoms affect only one side of your face and head. So if food falls out of both side of your mouth while you’re eating, you can’t simply claim that you have Ramsey Hunt syndrome. The classic triad of symptoms for Ramsey Hunt syndrome consists of:

• Ipsilateral facial paralysis: This is a fancy way of saying one side of your face can’t move. The paralysis may vary in severity of what portion of the face is affected. Such weakness may manifest as difficulty closing one eye, food falling out of one weaker corner of your mouth, difficult making expressions when you haven’t had any Botox, or drooping of one side of your face.
• Otalgia: This is an even more fancy way of saying that you have severe ear pain.
• Vesicles on the auricle: This may sound like a Shakespearean play but really means fluid-filled lesions on your ear lobe.

Specific symptoms may vary from person to person, though. Typically, the syndrome will begin with a one-to-three day period of fever, fatigue, and worsening pain on one side of your face. (Yes, there is a good chance that Bieber has had Bieber fever.) This first set of “prodromal” symptoms will then progress to some degree of facial paralysis. Like shingles in general, a rash will appear at some point, beginning as reddish bumps that subsequently transform into fluid-filled lesions (vesicles). Unlike shingles in general, this rash will be on one side of your face, such as in and around one of your ear or one side of your mouth. Within a week, these vesicles will rupture, crust over, and eventually fall off as scabs. These lesions can last for two to three weeks.

Other possible symptoms reflect the variety of functions controlled by your cranial nerves V, VII, VIII, IX, X, XII. This may include hearing issues, nasal blockage, problems with taste, excessive watering or dryness of your eye, drooling, uneven smiling, vertigo, hoarseness, problems swallowing, or facial numbness. Ramsay Hunt syndrome can be mistaken for other conditions such as Bell’s Palsy. Therefore. two things that need to be present to make the diagnosis are the one-sided facial paralysis and severe pain.

Like political figures and reality TV show characters, these symptoms may go away or in some cases persist long after the initial kerfuffle. Those with more severe paralysis initially may be more likely to suffer longer term issues like continuing facial weakness or pain, otherwise known as post-herpetic neuralgia. In fact, some problems such as involuntary mouth twitching, eye winking, and other such movements may not even appear until four to six months after the paralysis first came about.

Therefore, although many cases of Ramsay Hunt syndrome may resolve on their own with time, treatment with antiviral medications and steroids may help reduce the risk of such persistent problems. The most common course of antiviral medications has been seven to 10 days of acyclovir, valacyclovir, or famciclovir, although some studies have suggested that a longer 21-day course may be more effective. It’s not clear whether Bieber has been taking medications but he may want to do so “Justin” case.

Before you start to think about Ramsay Hunt syndrome every time a curly fry accidentally drops out of your mouth, keep in mind that this syndrome is not super common. It affects only about five out of every 100,000 people in the overall population per year. The syndrome most frequently affects people in their 60’s and 70’s. Now you may claim that Bieber doesn’t look like your typical 60 or 70 year-old. After all, oversized hoodies and distressed jeans aren’t the most common outfits for people in that age range. Bieber is actually only 28 years of age, underscoring the fact that Ramsay Hunt syndrome can appear in nearly anyone of any age as long as the VZV is present.

The good news is that there is a vaccine that can reduce your risk of getting any type of herpes zoster. It is the shingles vaccine. The Centers for Disease Control and Prevention (CDC) recommends that “adults 50 years and older get two doses of the shingles vaccine called Shingrix (recombinant zoster vaccine) to prevent shingles and the complications from the disease. Adults 19 years and older who have weakened immune systems because of disease or therapy should also get two doses of Shingrix, as they have a higher risk of getting shingles and related complications.”

Clearly, Ramsay Hunt syndrome is not a fun thing to get. The words “facial paralysis” and “severe pain” are rarely followed with words like “that’s awesome”, “yummy”, or “stay.” Being younger than 50 years of age does make Bieber more likely to fully recover. But as can be seen, the syndrome can not only cause problems in the short term but also in the much longer term as well. Therefore, not treating this condition seriously could end up being a rather one-sided view.