Lessons from a rare case of shingles and a pop icon

Justin Bieber, the 28-year-old pop icon, announced Friday that he was suffering from Ramsay Hunt Syndrome, a rare condition involving the facial nerve which can cause one-sided facial paralysis, loss of hearing, and is generally separated out from more common Bell’s Palsy because of a characteristic blistery painful red rash on the outside of the ear or another part of the face. Of course, Ramsay Hunt can lack the rash, and it is estimated that close to 20 percent of all Bell’s Palsy are really misdiagnosed and are actually due to Ramsay Hunt Syndrome.

The condition is a form of shingles and involves reactivation of the chickenpox virus (varicella-zoster). It generally occurs over the age of 60, likely because the immune system isn’t as strong as in younger people and the virus is more easily able to reemerge from being dormant. The varicella-zoster (chickenpox) vaccine almost entirely prevents it, and what is particularly relevant in Bieber’s case is that he was born in Canada (London, Ontario) in 1994. While the chickenpox vaccine became available in 1995, it wasn’t universally utilized in Canada until 2000.

I don’t know if Justin Bieber had chickenpox or not, but it is very likely he did if this is truly Ramsey Hunt Syndrome he is suffering from. The treatment, namely the antiviral drug acyclovir as well as steroids, is generally effective (close to 70 percent) in causing a complete recovery, especially in conjunction with rehabilitation and facial exercises that he says he is getting. Full recovery can take several weeks.

For those of us who had chickenpox and are over the age of 50, the best thing we can do is take a vaccine; in this case, the Shingrix protein vaccine which has been available since 2017. I recommend this vaccine for all my patients over 60, and for most over 50 and I find it to be highly successful and safe. In fact, studies show it to be well over 90 percent effective at preventing shingles, including Ramsay Hunt Syndrome. 

The varicella-zoster vaccine has decreased the rate of chickenpox cases dramatically, though there are still 350,000 cases every year according to the Centers for Disease Control and Prevention, and 90 percent of all cases occur in young children. Children receive the first dose at ages 12 to 15 months (15 to 20 percent still get chickenpox but it’s a much milder case) and most receive the second dose at ages 4 to 6 years, before attending kindergarten. Two doses of the vaccine are over 90 percent effective at preventing the disease. This is why 45 states mandate two shots before attending school and five states at least one shot. I think this practice makes good public health sense. The CDC estimates that each year more than 3.5 million cases of chickenpox and 9,000 hospitalizations are prevented by the vaccine. 

And if you do develop chickenpox anyway, when you get older, you have the highly effective Shingrix vaccine to protect you. Stress has been shown to induce herpes outbreaks (including shingles) by hampering the immune system. Bieber is clearly under stress, which could help explain his rare outbreak, though if this is indeed Ramsay Hunt Syndrome, it is occurring at an uncharacteristically young age. 

Celebrities frequently use their own illnesses to promote awareness not just of their problem but also of a solution. Justin Bieber could do a big public service for all his fans by spotlighting not just Ramsay Hunt Syndrome, but also the great public health value of the vaccines that prevent both chickenpox and shingles.

Marc Siegel, M.D., is a professor of medicine and medical director of Doctor Radio at NYU Langone Health. He is a Fox News medical correspondent and author of the new book, “COVID; the Politics of Fear and the Power of Science.”