Transcription:
Ramsay Hunt Syndrome

Melanie Cole, MS (Host): Welcome to Back to Health, your source for the latest in health, wellness, and medical care, keeping you informed. So you can make informed healthcare choices for yourself and your whole family. Back to Health features, conversations about trending health topics and medical breakthroughs from our team of world renowned physicians at Weill Cornell Medicine. I'm Melanie Cole joining me today is Dr. Michael Stewart. He's a professor and chairman in the department of otolaryngology, head and neck surgery at Weill Medical College of Cornell University, and otolaryngologist in chief at New York Presbyterian Hospital, Weill Cornell Medical Center. And he's here to tell us about Ramsay Hunt Syndrome.

Dr. Stewart. It's a pleasure to have you join us today. Can you tell us a little bit about Ramsay Hunt Syndrome? What is it and discuss this condition as it's recently been in the news with Justin Bieber's recent diagnosis.

Michael Stewart, MD, MPH: Yes, I'll be happy to thank you for having me. I'd like to tell a little bit of history about Ramsay Hunt, which is interesting most medical syndromes for example, Bell's Palsy, which is facial paralysis is named after sir, Charles Bell. This Ramsay Hunt syndrome is named after Dr. Ramsey Hunt. That was his name, his whole name. So it's a little unusual that both his first and last name were in the syndrome. It's also interesting that he discovered and reported this syndrome when he was a neurology professor at Cornell University's Medical College, back before it was named Weill Cornell.

He moved from Cornell's medical college to Columbia's Medical College in New York city a few years later. So, I'm sitting here at Cornell's Medical College now and reporting on what Dr. Ramsay Hunt did more than a hundred years. So Ramsay Hunt syndrome is a special type of shingles. Shingles is familiar to many people. It's a reactivation of the chicken pox virus called herpes zoster or vari cell zoster that lives dormant in a nerve until it is reactivated by something later in life and causes a painful nerve pain and rash with vesicles and little plus fuels that spread over the distribution of that nerve.

Ramsay Hunt affects the ear, the area right around the ear, and it affects the facial nerve causing facial paralysis or severe facial weakness. The classic findings of Ramsay Hunt Syndrome are vesicles and rash, severe ear pain and facial nerve paralysis. So it's a subtype of shingles caused by exactly the same virus.

Melanie Cole, MS (Host): Yikes. Shingles is scary enough. So tell us a little bit about who is at risk and you can speak as well about some of the way it presents itself.

Michael Stewart, MD, MPH: Yes. So, many people are at risk because if you ever had chicken pots you have the virus in you throughout your life. Even if you got a certain type of chickenpox vaccine, which was the killed virus, still in some patients, you're at risk. Now we do have vaccines for chickenpox and for shingles, which can reduce the frequency of this now, which are relatively recent. Who gets it? Anyone can get it. It's more common in the patients over age 60, it's more common in patients who have an immune compromise condition or who have another illness.

Something that sets off or weakens the immune system or causes stress to the body, which causes this virus to reactivate. However, it has been reported in young, healthy people frequently as well. The typical presentation is significant ear pain, followed by development of rash and blisters in the ear itself was called the Oracle, the external ear or in the skin surrounding the ear, followed by facial weakness or facial paralysis.

Melanie Cole, MS (Host): Dr. Stewart, does this come on fast or is it slow to develop? Because it sounds to me by what you've described, that it's some of those symptoms look similar to a stroke. And so facial paralysis and such and that would warrant a B fast and 911 call. Tell us a little bit about how this develops? Does it come on really fast or is it a little bit slower?

Michael Stewart, MD, MPH: It's a little bit slower. Typically you typically get one or two symptoms and then the others follow in a progression over a matter of say one to three days. So it doesn't all happen. Boom. All at once. Many people who have this, just like people who have Bell's Palsy, one of their first thoughts is. Was this a stroke and that's not an unreasonable concern, but there's often a progression of symptoms. Other symptoms that can occur are hearing loss because the facial nerve that moves the facial muscles also is right next to the hearing nerve and the balanced nerve.

So patients can also get hearing loss. They can get vertigo and the facial nerve also carries nerves that protect the ear from loud. So patients who have this will oftentimes suddenly find that loud sounds are extremely bothersome in that affected ear. It also carries nerve fibers that supply the tear glands. So people get a dry eye and also part of the taste of the tongue. So, Patients can notice a change in their taste or a funny taste in the mouth.

So if you develop over a period of one to two days, vesicles, pain, change in your hearing, dry eye, and then you develop a facial paralysis. That's not a stroke. Almost definitely that's this nerve inflammation, this shingles reactivation happening over the initial course.

Melanie Cole, MS (Host): So then how is it diagnosed? What tests do you do to determine that this is what's going.

Michael Stewart, MD, MPH: It is a fairly classical clinical picture. You can do an MRI scan, which will demonstrate enhancement in the nerve, but that's actually not necessary for the diagnosis with a classic presentation, the vesicles, the facial weakness, or total paralysis, etcetera. The diagnosis is often made clinicly. It's relatively rare. Ramsay Hunt for example, Bell's Palsy, which is just pure facial paralysis, which is not caused by any known cause.

And then typically responds very well on its own over days to weeks and recovers. That's a more common condition. If you're at a cocktail party, you're more likely to meet someone who has had Bell's Palsy once in their life or someone in their family has Ramsay Hunt is more rare. It's not something that you'll typically meet or know someone who's had it, but it's not vanishingly rare. It, you know, we do see it in our practice certainly.

Melanie Cole, MS (Host): Does the shingles vaccine. You mentioned it a little bit earlier. Can you expand on that? Does that provide, I mean, I just got two of them last year as my mother lost the vision in her eye from shingles. And so did an uncle on the other side of the family. So both sides of my family had that in their head and around that area. Does that vaccine offer some protection, even though this is relatively rare, as you said?

Michael Stewart, MD, MPH: It absolutely does. Now vaccines are not perfect. It is possible for a person who has been vaccinated depending on your body's response to the vaccine. It's theoretically possible you could still develop shingles, but the vaccine dramatically reduces the frequency of shingles compared to unvaccinated patients. And because this is a subtype of shingles. Yes. If you've been vaccinated, with the Shingrix or there's another vaccine that's available as well. If you've been vaccinated, you're much less likely to have Ramsay Hunt.

Melanie Cole, MS (Host): So then speak about what available to treat it. Does it permanently damage the nerves? Are there treatments? What do you do for it?

Michael Stewart, MD, MPH: Yes. So it is important to treat this. And we typically treat this with an antiviral medication that's designed to be treated for the, herpes virus, which would be either Valcyclovir or acyclovir. Usually Valcyclovir or Valtrex is what's preferred. We also typically get steroids because inflammation is part of the problem. And it's actually important to treat with antivirals and steroids for several days. First of all, it helps the acute symptoms resolve quicker. But it also very likely prevents the development of what's called postherpetic neuralgia, which is persistent nerve pain after shingles, which is shingles are bad enough.

If you get persistent nerve pain in that area after shingles, it's even worse. So, treatment with antivirals and steroids helps the initial symptoms and it also reduces the risk of post herpetic neuralgia. So treatment is important. Also, we give pain medication many times some sort of analgesic because in fact, the pain associated with uh, Ramsay on ear pain can be quite significant and bothersome.

Melanie Cole, MS (Host): Are there any long term complications, if it is not treated quickly with antiviral and steroids, as you mentioned?

Michael Stewart, MD, MPH: Well, yes potentially a higher risk of the post, herpetic neuralgia that I mentioned, but also I should point out that Ramsay Hunt does have a generally poorer prognosis of total recovery of facial nerve function than like Bell's Palsy. Even if you do almost nothing has a greater than 90% chance of returning to normal. It's a rare patient who doesn't have a full recovery. With Ramsay Hunt, it's about a 70% chance of recovery to complete normal. So there's more patients with Ramsay Hunt who do not fully recover their facial nerve function .

Or they have, what's called a sin kinese or mass motion, which is where different parts of the face move together when they're not supposed to. So the eye blinks at the same time as your mouth smiles, for example that's an outcome that can happen after Ramsay Hunt which is more common than it is in other types of facial paralysis. Also the hearing loss, if it occurs and hearing loss only occurs in about. Patients, but if it occurs it can often recover just like other viral infections causing hearing loss can recover, but it may not. So some people develop a permanent hearing loss after Ramsay Hunt as well.

Melanie Cole, MS (Host): Well, I can attest that post hepatic neuralgia can be quite painful and disabling in so many ways. I'd like you to wrap it up Dr. Stewart, with your best advice, whether we can prevent this. About how rare it is. Maybe the importance of keeping up with our well visits and good lifestyle behaviors and vaccines for sure. Give us a summary of what you tell people when they're asking you about this now, as it's been in the news.

Michael Stewart, MD, MPH: Yes. So, this is caused by the virus that causes chickenpox. So getting vaccinated against chickenpox as a young person is very helpful. Following the appropriate guidelines for vaccination against shingles is also important, and those are based on age and underlying medical conditions. So I strongly recommend people get the shingles vaccine, not just Ramsay Hunt but for shingles in general, that's the other piece of advice. The third piece of advice is if you develop your pain sudden hearing loss or weakness of the face, facial weakness or paralysis definitely seek medical attention.

Because in fact, starting early treatment may, this hasn't really been proven definitively, but it's definitely believed that early treatment improves your outcome. And almost definitely reduces the chance of having a long term problem, like a post herpetic neuralgia.

Melanie Cole, MS (Host): Wow. So interesting. And doctor, thank you so much for joining us and telling us about this, which is so similar to shingles. but people don't really know what it is. So what a great education you've given us today, such an informative podcast. Thank you again. And Weill Cornell Medicine continues to see our patients in person, as well as through video visits. And you can be confident of the safety of your appointments at Weill Cornell Medicine. That concludes today's episode of Back to Health.

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