In June, pop star Justin Bieber shared with fans via Instagram that he is experiencing facial paralysis due to Ramsay Hunt syndrome. The Well spoke with Dr. Matthew Miller, the director of the UNC Facial Nerve Center, about facial paralysis and what it means for Bieber and others who have it.
What is Ramsay Hunt syndrome, and how serious is it?
Ramsay Hunt syndrome is a reactivation of the varicella-zoster virus affecting the facial nerve, which results in facial paralysis. While typically Ramsay Hunt syndrome is seen in individuals who have had chickenpox — caused by primary varicella-zoster virus infection — Ramsay Hunt syndrome occasionally affects people who reportedly never had chickenpox.
The facial nerve starts in your brainstem and travels through the temporal bone, which is the bone where your ear sits. When the virus reactivates in the facial nerve, it causes swelling of the nerve. The nerve is compressed by the surrounding temporal bone, which is thought to cause the facial paralysis.
Other common symptoms of Ramsay Hunt syndrome include severe ear pain, a blistering rash in the ear, hearing loss and severe dizziness; the hearing and balance nerves travel next to the facial nerve in the temporal bone. Patients often describe Ramsay Hunt syndrome as the worst pain they have ever experienced.
Think of it as shingles of the facial nerve. One in three people will get shingles, whereas Ramsay Hunt syndrome is much less common — affecting about one in 300 people.
I read that you had a similar facial paralysis, but for a different reason. What was your experience like?
Back in 2008, I was a third-year at the University of Virginia and a triathlete. While I was on a bicycle training ride, I collided head-on with a car and suffered panfacial fractures, severe traumatic brain injury and complete left-sided facial paralysis. For about six or seven months, I had no movement on the left side of my face.
I can remember vividly just how hard it was to live with facial paralysis — not really wanting to go out in public, not wanting to smile. It’s devastating because you’re talking to some of your friends, some of your family members, people you don’t know, and they don’t listen to what you’re saying because they’re just staring at your face. They’re like, “Why did this guy have a stroke?” Of course, I didn’t have a stroke, and the vast majority of people with facial paralysis did not have a stroke. But that’s what people assume, and it’s frustrating to feel ignored. Usually, people don’t even ask because they’re trying to be polite. In a way, that makes it worse, because if they ask, you could actually explain what happened.
Did that experience influence your career choice?
Without a doubt. I went to medical school at the University of Pennsylvania. Then I went back to the University of Virginia for residency. During residency, as I saw some patients with facial paralysis and then put that together with my own experiences, I was like, “You know what? This is what I want to do with my life. I want to try to give people back their smile, their facial function, their facial symmetry.”
Harvard has perhaps the best fellowship in the world for sub-specialized training in caring for patients with facial paralysis. I spent two years in Boston learning the intricacies of facial paralysis management and the most cutting-edge treatments.
Caring for patients with facial paralysis is such a rewarding career. It is such a privilege for people to trust you with their faces, their facial symmetry, their facial function.
What are the main causes of facial paralysis?
Bell’s palsy is by far the most common cause. Bell’s palsy is a reactivation of the herpes virus in the facial nerve, which causes sudden-onset facial paralysis. Somewhere between 50% and two-thirds of patients with facial paralysis have it from Bell’s palsy, and one in 60 people will be affected with Bell’s palsy in their lifetime. People will often call all facial paralysis Bell’s palsy, but facial paralysis itself is a manifestation of many different disease processes. We’re at over 30 different causes of facial paralysis here at the UNC Facial Nerve Center.
Ramsay Hunt syndrome is one of the three most common causes of facial paralysis, accounting for roughly 7% of cases.
Other common causes include trauma, benign tumors, cancer, facial paralysis after surgery, and infections. About one in 1,000 children are born with facial paralysis. Lyme disease is a relatively common cause of facial paralysis in regions where it is endemic; fortunately, we don’t see Lyme disease a lot in North Carolina.
When evaluating patients with facial paralysis, it is critical to get the diagnosis correct. At least once every couple of weeks, someone’s coming into the UNC Facial Nerve Center with “Bell’s palsy” that’s not really Bell’s palsy. The last thing you want to do is miss somebody who might have cancer, a benign tumor or another cause of facial paralysis that’s treatable because you assume it’s Bell’s palsy. It’s an easy mistake to make. My career is largely dedicated to caring for patients with facial paralysis, so it’s relatively easy for me to identify what’s going on; that takes years of training.
How does Ramsay Hunt syndrome limit one’s ability to smile?
By the time you get toward the middle of your face, you have about 50 branches of the facial nerve. They’re less than one millimeter in size. I know them well because I frequently have to identify them during surgery when repairing the facial nerve or performing “selective denervation,” a procedure that can improve a patient’s smile and facial tightness.
In the healthy facial nerve, all these tiny facial nerve branches know exactly which muscle to go to. You have 43 muscles on each side of your face, so there’s this beautiful relationship between these tiny nerve branches and these multiple facial muscles. This relationship is why we can use facial expressions like smiling to communicate so effectively.
About 30% of Bell’s palsy patients and 40-50% of Ramsay Hunt syndrome patients do not make a complete recovery. This incomplete recovery happens because the face is miswired as the facial nerve regenerates. This can lead to facial tightness, smile asymmetry, general facial asymmetry and a phenomenon known as synkinesis. Synkinesis is an involuntary facial movement associated with a voluntary facial movement. The most common type of synkinesis patients notice is involuntary closing of their affected eye when they smile. Similar to smile asymmetry, this involuntary eye closing can be devastating, especially now that we wear masks so often and we rely even more on our eyes to communicate.
What else should people know about facial paralysis?
The last five or 10 years have seen an explosion of new treatment options for patients with facial paralysis. Before that, health care providers would often tell somebody with facial paralysis to “Just live with it; there’s nothing we can do.” Today we have a lot of options, especially at a specialized center like the UNC Facial Nerve Center.
I have patients who have lived with chronic facial paralysis for 20-30 years, after having been told there was nothing that could be done to help their facial function and symmetry. I am treating one patient who had Ramsay Hunt syndrome for 45 years prior to coming to the UNC Facial Nerve Center. These patients can become depressed, avoid going out in public and frequently avoid photographs.
Young people get affected by facial paralysis. Older patients can have facial paralysis. All races, all genders — no one is immune to facial paralysis. That’s something Justin Bieber shows. He’s young, he’s healthy and he got facial paralysis.
The bottom line is, I don’t care how long you’ve had facial paralysis or what has caused your facial paralysis, there is almost always something we can do. The problem is many health care providers do not know this because many of the treatments are relatively new and only offered at specialized centers such as the UNC Facial Nerve Center. I’m so thankful that Justin Bieber has talked openly about his experience, because I think it will help generate awareness for facial paralysis, both how devastating it can be and how many treatments are now available.
There are only a few dedicated facial nerve centers in the United States. One of the reasons I came to UNC is because the Southeast region of the U.S. had very limited options for facial paralysis patients seeking cutting-edge treatments. For me, it’s a dream job, because you have thousands of patients in the region living with facial paralysis, waiting for a center to be able to offer them the most innovative treatments and management for their facial paralysis. We now have this center at UNC.
How do you think this celebrity case will affect the public’s view of facial paralysis?
I think the biggest thing is that people are going to be aware of it. Until you know someone or until a public figure has had facial paralysis, like most things in life, it’s not on your mind. If a teenager sees Justin Bieber’s facial paralysis and has a relative or friend who’s had facial paralysis, I hope they’ll talk to them about it. I hope there will be an improved recognition of facial paralysis and, with that, an improved awareness of all the treatments we have now for facial paralysis.