Explained: What is Ramsay Hunt Syndrome — the rare facial condition affecting Justin Bieber?

What is Ramsay Hunt Syndrome?

Ramsay Hunt Syndrome is neurological disease in which a virus – Varicella Zoster – causes inflammation of the nerves involved in facial movements. When the nerves get inflamed, they lose their ability to function, leading to temporary facial palsy or paralysis. What it means is that facial muscles in the infected person cannot receive necessary signals to function properly.

“There are 12 cranial nerves in the body. Ramsay Hunt Syndrome is the viral infection impacting the 7^th cranial nerve that is involved in facial movements. The inflammation caused by the Varicella Zoster Virus makes the nerve ineffective,” says Dr (Col.) JD Mukherji, Principal Director and Head of Neurology, Max Healthcare at Saket, Delhi.

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What are the symptoms of Ramsay Hunt Syndrome?

The symptoms include painful, red rash and blisters in and around the ear, and facial paralysis on the same side.  Patients also report hearing loss in the ear that has been impacted apart from Tinnitus or ringing sounds. Inability to close the eye causes dryness.

“You can get a rash on the eardrum, the ear canal and the earlobe. Rashes can also appear inside the mouth, on the tongue. There is hearing loss, vertigo or spinning sensation. It causes weakness of one side of the face and causes difficulty in closing one eye and eating as food falls off from the affected side. There is drooping of the face,” said Dr Mukherji.

What is the virus that causes Ramsay Hunt Syndrome?

The Varicella Zoster Virus (VZV) is the same virus that causes chickenpox and shingles. According to the US Centre for Disease Control and Prevention, this virus belongs to the herpesvirus group and can stay in the body as a latent infection. The virus can stay dormant in a person’s body and can reawaken to attack the nerves. According to doctors, VZV can get reactivated due to weakening of the immune system. Stress is also considered a trigger, according to some studies, as it depresses the immune system. Some reports also suggest that T-cell dysfunction caused by coronavirus infection can reactivate VZV.

How does the disease progress?

Initially, the patient may complain of facial weakness and little difficulty in closing one eye. The disease fully manifests in a matter of two to three days. Prompt treatment after diagnosis can speed up the recovery and reduce chances of severe nerve damage.

How is Ramsay Hunt Syndrome treated?

It is treated using anti-viral drugs, steroids and physiotherapy. Steroids and anti-viral drugs are the cornerstone of the treatment, says Dr Mukherji.

“Facial exercises are very important. Patients need to protect their eyes. When one eye doesn’t close, there is a chance of developing corneal aberration. Patients must use tear drops and use eye protection. They should wear glasses when they go out, and tape the eye that has been impacted when they sleep at night,” he adds.

Is Ramsay Hunt Syndrome contagious?

The disease is not contagious but can lead to chickenpox in those not vaccinated for the disease. Till the blister scabs fall off, the patients are advised to avoid contact with those with low immunity and the ones who have not had chickenpox, or are not vaccinated against it.

Do patients recover fully from Ramsay Hunt Syndrome?

The disease is reversible. Patients get cured within 15 days to three months of starting the treatment.

“The take-home message is that it can be cured. Near-complete resolution happens. Even the hearing loss is reversible. Little bit of residual facial palsy may remain. There may be just a little bit of lag in the closure of the eye later on. That’s very minor,” Dr Mukherji pointed out.