Justin Bieber’s Ramsay Hunt Syndrome: A Rare Neurological Disorder

Ramsay Hunt syndrome, which suffers from singer Justin Bieber who has just canceled two concert dates, is a rare disorder that paralyzes one side of his face, caused by a reactivation of the chickenpox virus.

• Read also: Justin Bieber announces that he is paralyzed in half of his face

Discovered in 1907 by neurologist Ramsay Hunt for whom it is named, the syndrome is a rare neurological disorder characterized by facial nerve paralysis (facial paralysis) and a rash affecting the ear or mouth.

They differ from case to case. Those affected usually suffer from paralysis of the facial nerve and a rash affecting the ear. These two symptoms do not always occur simultaneously. In most cases, only one side of the face is affected (on one), and the American organization NORD, which aims to provide support for people with rare diseases, indicates on its website.

The affected facial muscles may be weak or stiff, which may cause affected individuals to be unable to smile, to wrinkle their foreheads, or to close their eyes on the affected side. In some cases, speech may become slurred.

In most cases, a painful red (erythema) rash (vesicular) appears that affects the outer part of the ear (the pinna) and most often the external auditory canal.

“The diagnosis is often made because of this vesicular eruption around the ear or within the ear canal,” says Benjamin Davido, an infectious disease specialist at Jarchis Hospital (AP-HP Hôpitaux de Paris).

In some cases, rashes, including painful blisters, may also affect the mouth, palate, and upper throat.

Other symptoms such as ringing in the ears (tinnitus), earache (earache), sensorineural hearing loss, and hyperacusis (increased sounds) are possible.

Sometimes sufferers also experience vomiting or dizziness.

Ramsay Hunt syndrome is caused by the varicella zoster virus, the same virus that causes chickenpox and herpes zoster.

The virus can lie dormant for decades in a person who had chickenpox as a child. Reactivation of the varicella-zoster virus leads to the appearance of herpes zoster and in some cases it develops into Ramsay Hunt syndrome. Why the virus activates and affects the facial nerve in Ramsay Hunt syndrome is unknown.

This syndrome affects men and women in equal numbers. It is estimated that five out of every 100,000 people develop this syndrome each year in the United States, according to NORD. Some researchers believe, however, that cases are underdiagnosed or misdiagnosed, making it difficult to determine the true frequency of the disorder in the general population.

Anyone who has had chickenpox can develop Ramsay Hunt syndrome. However, most cases occur in the elderly, especially those over 60 years of age or those who are immunocompromised.

“It’s amazing to be stalking Ramsey at Justin Bieber’s age,” says Benjamin Davido. But “an imperfect lifestyle or an overworking context can play a role in that, because we are then more susceptible to viral infections,” he continues.

Treatment for Ramsay Hunt syndrome usually includes antiviral medications, such as acyclovir or famciclovir, along with corticosteroids, such as prednisone.

Rehabilitation, which is “essential to start early,” generally allows for a good recovery, according to Benjamin Davido. But the consequences persist in “about 30% of cases”.

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