Canadian singer Justin Bieber in a file photo Image Credit: AFP

Dubai: Even as friends and fans rally around Justin Bieber following his recent diagnosis with Ramsay Hunt syndrome, not much is known about the rare medical condition that affects five out of every 100,000 people annually in the United States, according to The National Organization for Rare Disorders (NORD).

The 28-year-old Canadian singer has been forced to cancel concerts from his Justice World Tour — although his UAE gigs remain unaffected as of now — with the condition paralysing one side of his face and hindering Bieber from even blinking effectively, as revealed in his Instagram post over the weekend. The ‘Peaches’ singer has said he’s been doing facial exercises and taking time to “rest and relax and get back to 100 per cent so I can do what I was born to do.”

While he hasn’t given an estimated timeline for his recovery, we breakdown the medical condition to understand how Ramsay Hunt syndrome affects people and what causes it.

What is Ramsay Hunt syndrome, the condition Justin Bieber is suffering from?

In his own words, Bieber explained in the video to his legion of fans that his condition stems “from this virus that attacks the nerve in my ear and my facial nerves, and has caused my face to have paralysis.”

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NORD describes Ramsay Hunt syndrome as a rare neurological disorder Image Credit: Shutterstock

NORD describes Ramsay Hunt syndrome as a rare neurological disorder that can occur in people who were previously infected with chickenpox. If the dormant varicella-zoster virus (the same virus that causes chickenpox) is reactivated, leading to shingles, it can affect the facial nerve and may lead to facial paralysis and hearing loss.

What are the symptoms of this medical condition?

Ramsay Hunt syndrome
Ramsay Hunt syndrome as a rare neurological disorder that can occur in people who were previously infected with chickenpox Image Credit: Shutterstock

Aside from facial palsy, other symptoms can include a rash affecting the ear or mouth. Ear abnormalities such as ringing in the ears (tinnitus) and hearing loss may also occur. In most cases, only one side of the face is affected. Additional symptoms that may be present include nausea, vomiting and vertigo, according to NORD.

Does the condition affect everyone?

Older adults are more likely to be affected. According to NORD, most cases affect those over 60. Ramsay Hunt syndrome affecting children is extremely rare.

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Justin Bieber has been forced to cancel his world tour after his diagnosis Image Credit: Reuters

How does one diagnose Ramsay Hunt syndrome?

According to the Mayo Clinic, to confirm the diagnosis, a doctor might take a sample of fluid from one of the rash blisters in the ear for testing. But in some cases, a rash may be completely absent and makes it difficult to diagnose.

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For illustrative purposes only. Image Credit: Agency

How does one recover from the condition?

Depending on the patient’s condition, Mayo Clinic says antiviral drugs, medications used to combat the chickenpox virus can be used, supplemented by medicines to help relieve vertigo and pain relievers, which can be severe in some cases.

How severe is Ramsay Hunt syndrome?

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Justin Bieber in a screengrab of his video posted on Instagram Image Credit: Instagram.com/justinbieber

While Ramsay Hunt syndrome isn’t contagious, according to the medical research, the reactivation of the varicella-zoster virus can cause chickenpox in people who haven’t previously had it. The infection can also be serious for people who have immune system problems.

In Bieber’s case, the condition has affected one side of his face. In the video posted on June 10, Bieber broke the news to his millions of fans, saying: “As you can see, this eye is not blinking, I can’t smile on this side of my face, this nostril will not move. So, there’s full paralysis on this side of my face. So for those who are frustrated by my cancellations of the next shows, I’m just physically, obviously, not capable of doing them. This is pretty serious, as you can see.”

Early detection is key to recovering from Ramsay Hunt syndrome

Dr Bobby Jose Specialist Neurosurgeon, NMC, Al Zahra Hospital, Sharjah.

Early detection, diagnosis and treatment is key to getting the symptoms of Ramsay Hunt syndrome under control.

Dr Sharik Mustafa, Specialist Neurologist at Prime Medical Centre, Dubai added that in the case of Justin Bieber, one could expect a 95 per cent recovery with the immediate medical intervention, treatment and physiotherapy.

Seventh cranial nerve affected

Dr Jose elaborated: “Ramsay Hunt syndrome is caused by a flare up of the varicella-zoster virus that causes chickenpox and shingles. Actually, when an individual contracts the varicella-zoster virus.

“These days, most young children are vaccinated with live attenuated virus and protected from chickenpox and shingles. In case of those who contracted it earlier and recovered, the virus never goes away, but continues to live in the ganglions of the nerves. These ganglions are relay centres of our nerves in any part of the body.

“In the case of Ramsay Hunt syndrome, the virus affects the ganglions of the seventh cranial nerve that results in facial paralysis. In this case, the flare-up must have started with manifestation of a rash below or behind his [Bieber’s] ear. This rash is usually vesicles or liquid filled boils which goes on to affect the facial muscles causing paralysis.”

Different from Bell’s Palsy

Dr Mustafa highlighted the important difference between Ramsay Hunt syndrome and Bell’s Palsy, the more common type of facial paralysis from this condition.

Dr Sharik Mustafa, Specialist Neurologist at Prime Medical Centre

Ramsay Hunt syndrome can be differentiated from Bell’s Palsy or stroke symptoms as it is more severe with lasting impact of paralysis. It begins with the appearance of lesions or vesicles, which are fluid-filled tiny rashes. Usually shingles or flare up of dominant virus happens in case of older, or those with diabetes or other immunocompromised conditions.

“In the case of Bieber who had earlier been diagnosed with Lyme disease and indicates that his immunity has been compromised a bit and that is why he has been effected at such a young age.”

In physiotherapy, we focus on maintaining the muscle tone of the affected side and prevent any wasting of the muscle owing to its flaccidity. We usually alternate the treatment with galvanic stimulation, followed by ultrasonic treatment along the affected nerve track

- Deepa Chandran, Head of physiotherapy at Prime Healthcare
Types of treatment
Prognosis for patients who get an early diagnosis and begin treatment is very good, said both the doctors. They added that quick treatment for Ramsay Hunt syndrome included a strong antiviral course that could last up to a year. In addition, electrical stimulation of the facial nerve and other physiotherapy can help maintain the muscle tone to prevent muscles getting flaccid.

Dr Jose added that since Bieber was a vocalist, it was important to take care that his blowing capacity and sense of hearing was not affected and doctors could monitor this to prevent any deterioration. With antiviral, faradic face stimulation started the very same day the condition is diagnosed, one can expect a 95 per cent improvement within 3-6 weeks, added Dr Mustafa.

Physiotherapy Protocol

Patients with facial paralysis will experience weakness of muscles on the side impacted by the virus and will have difficulty in lifting the eyebrows, swallowing, chewing and have pain near the nerve track near the ear and neck pain as well.

Deepa Chandran, Head of physiotherapy at Prime Healthcare explained the physiotherapy protocol for Ramsay Hunt syndrome or any other kind of facial paralysis.
“In physiotherapy, we focus on maintaining the muscle tone of the affected side and prevent any wasting of the muscle owing to its flaccidity. We usually alternate the treatment with galvanic stimulation, followed by ultrasonic treatment along the affected nerve track,” said Chandra, adding: “We also provide a tailored programme for facial muscles that the individual can practice at home to keep the muscles supple. In addition, if the eye muscle is impacted we also provide strapping of the eyelid to prevent it from drooping. With a consistent exercise programmer the patient will see substantial benefits as the nerve generally takes 21 days or three weeks to regenerate.”

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Gigi Hadid Image Credit: Shutterstock

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Lady Gaga Image Credit: Shutterstock

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Jada Pinkett Smith Image Credit: AP

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Venus Williams Image Credit: AFP

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Gaten Matarazzo Image Credit: Shutterstock

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