LVHN is now proudly part of Jefferson Health
La Lehigh Valley Health Network se enorgullece en ser ahora parte de Jefferson Health.
Proudly Part of Jefferson Health
Learn about this condition that can occur years after having chickenpox
One of the world’s biggest pop stars, Justin Bieber, recently announced on Instagram that he was diagnosed with a rare neurological disease called Ramsay Hunt syndrome. The singer shared the news in a post on Saturday, June 11, that showed the effects, including partial facial paralysis.
“Obviously, as you can probably see from my face, I have this syndrome called Ramsay Hunt syndrome and it is from this virus that attacks the nerve in my ear and my facial nerves and has caused my face to have paralysis,” he said.
He went on to share, “As you can see, this eye is not blinking,” he said, showing that the other eye was able to. “I can’t smile with this side of my face, this nostril will not move, so there is full paralysis in this side of my face.”
Bieber announced that he would be taking time off to “rest and relax and get back to 100%.” He also shared that he has been doing “facial exercises to get my face back to normal.”
Ramsay Hunt syndrome, also known as herpes zoster oticus, is caused by the same virus that causes chickenpox and shingles (the varicella-zoster virus). Varicella-zoster virus can remain dormant in sensory nerves if you previously had chickenpox. Shingles is a reactivation of the varicella-zoster virus in those nerves. Shingles commonly causes painful blisters (vesicles) on the torso or limbs but also can affect the eyes or ears. Ramsay Hunt syndrome is when a shingles outbreak affects the face and ears.
The National Institute for Neurological Disorders and Stroke describes Ramsay Hunt syndrome as causing not only facial weakness or paralysis, but also a painful red rash that appears on, in and around one ear (including the eardrum, ear canal and earlobe) accompanied by fluid-filled blisters, ear and mouth pain (tongue, roof of mouth) and vertigo.
Ramsay Hunt syndrome is also very rare. According to the National Organization for Rare Disorders, only 5 out of every 100,000 people develop Ramsay Hunt syndrome each year in the United States, affecting both men and women equally. And in most cases it affects older adults, especially those over 60.
To diagnose Ramsay Hunt syndrome, Alex Benjamin, MD, Chief Infection Control and Prevention Officer, Lehigh Valley Health Network, says it starts with the patient’s description of symptoms. “Diagnosis is made by gathering history from the patient and looking for the typical distribution of the rash/blisters. In some cases, testing the fluid from an unruptured blister can confirm the presence of varicella-zoster virus,” he says.
Neurologist Jonathan Cheponis, MD, with Lehigh Valley Fleming Neuroscience Institute, says Ramsay Hunt syndrome resembles Bell’s palsy. “Bell’s palsy is a much more common cause of one-sided facial weakness, so identifying the typical rash and other signs of the varicella-zoster virus is crucial to make the correct diagnosis.”
In his post, Bieber shared that he was doing facial exercises to help rehabilitate movement in his face.
Besides physical rehabilitation, medication is also an important treatment. “Anti-viral medications including acyclovir, famciclovir or valacyclovir can be prescribed,” Benjamin says. “If given early enough in the course of the disease, anti-virals can reduce the duration of infection.”
Corticosteroids such as prednisone may also be given, according to the National Organization for Rare Disorders.
“The most important factor in managing Ramsay Hunt syndrome is starting treatment as early as possible,” Cheponis says. “Starting treatment within a few days of symptom onset enables most patients to make a complete or near-complete recovery. However, patients often don’t improve until weeks or months after the medications are given, so patience is also important.”
Ramsay Hunt syndrome may rarely result in permanent facial paralysis or hearing loss, regardless of therapy and treatments. It can also cause eye damage, specifically to the cornea, since Ramsay Hunt syndrome may prevent people from closing the eyelid (which Bieber also shared was a symptom he experienced). To prevent injury to the cornea, wearing an eye patch and using eye lubricant can help.
There is no set-in-stone recovery time for complete healing from Ramsay Hunt syndrome. This is often determined by how quickly treatment starts and how much nerve damage is done. Once treatment begins, symptoms typically resolve within a few weeks.
Symptoms of Ramsay Hunt syndrome are also similar to other disorders. This includes the neurological disorder Bell’s palsy, which can present with sudden onset facial paralysis, muscle weakness and/or pain in the ears and face.
If you have similar symptoms, talk to your doctor right away, who can help you determine if you have Ramsay Hunt syndrome and start you on treatment as soon as possible.
And for Justin Bieber, he is on the road to recovery. He ended his video on Instagram saying, “We don’t know how much time it’s gonna be but it’s gonna be OK and I have hope and I trust God and I trust that this is all gonna, it’s all for a reason and I’m not sure what that is right now but, in the meantime, I’m gonna rest. I love you guys, peace.”