Explained | What is Ramsay Hunt syndrome, the disorder affecting Justin Bieber?

Ramsay Hunt syndrome is a late complication of a varicella-zoster virus infection, which in its early stages causes chickenpox

June 13, 2022 01:10 pm | Updated June 15, 2022 12:34 pm IST

Justin Bieber shows his paralysed face in an Instagram video.

Justin Bieber shows his paralysed face in an Instagram video. | Photo Credit: Instagram/Justin Bieber

The story so far: In an Instagram post, Canadian singer Justin Bieber announced that he has been infected by a virus that causes the Ramsay Hunt syndrome. The 28-year-old singer posted a video to share his condition with his followers and show how half of his face has been paralysed by the virus. Mr. Bieber has cancelled a few of his upcoming concerts due to the diagnosis. “For those frustrated by my cancellations of the next shows, I'm just physically not capable of doing them," he said.

What is the Ramsay Hunt syndrome?

The Ramsay Hunt syndrome is a rare neurological disorder caused by the varicella-zoster virus of the human herpesvirus family. It is characterised by facial paralysis and may also present as ear abnormalities, including ringing in the ears, earache, or hearing loss.

The disorder, also called herpes zoster oticus, was first documented by neurologist James Ramsay Hunt.

According to the U.S. National Centre for Biotechnology Information, Ramsay Hunt syndrome is a late complication of the varicella-zoster virus infection and results in the inflammation of the facial nerve that controls expressions and movement of the face.

Early-stage infection of the varicella-zoster virus causes chickenpox in humans, but the virus often remains dormant in the body long after the condition has passed. Reactivation of the virus, usually caused due to physiological stress or immunocompromise, can cause a rash or shingles along the affected nerve. Less than one percent of zoster cases involve the facial nerve and result in Ramsay Hunt Syndrome. Additional symptoms of the disorder include a change in taste sensation, dry eyes, tearing, nasal obstruction, hyperacusis or noise sensitivity, and dysarthria or speaking difficulties.

Symptoms usually begin with pain in the ear on the affected side of the face in around 55 per cent of patients with the disorder. Facial paralysis and vesicles (small fluid-filled sacs or blisters) appear in 2-3 days. In 23 per cent of patients, facial paralysis is the first symptom hinting at the disorder.

The vestibulocochlear nerve that transfers impulses from the inner ear to the brain is located very close to the facial nerve, and can therefore cause hearing and balance-related complications like auditory loss, tinnitus (ringing sensation in the ears), and vertigo.

The disorder is easy to miss in the early stages and diagnosis can often be delayed, leading to long-term complications.

Ramsay Hunt syndrome accounts for approximately 7 per cent of acute facial paralysis cases, and patients who are immunocompromised have a higher chance of suffering from a more acute period of being infected and less complete recovery. There is no age bracket within which the virus affects humans – cases have been reported in babies as young as three months and adults as old as 82 years.

The condition r is reportedly self-limiting in nature, so treatment is mostly focussed on reducing pain, preventing later complications and decreasing the duration of the illness.

Is Ramsay Hunt Syndrome similar to Bell’s palsy?

Bell’s palsy is a case of facial muscle weakness or paralysis that can be set off suddenly. It is not considered to be a permanent condition, although in some rare cases, it can last for longer periods of time. Patients of Bell’s palsy usually show full recovery.

In comparison, patients of Ramsay Hunt syndrome may have a more severe paralysis at the onset of the disorder and may not recover completely. Only 70 per cent of patients with Ramsay Hunt syndrome regain normal or near-normal facial function compared to over 90 per cent in Bell’s palsy.

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