Neurologists explain Ramsay Hunt syndrome, and outline signs to watch out for

The singer at a perfomance. Pics/Instagram

Two weeks ago, Indian Beliebers were over the moon with the announcement of New Delhi being included in pop star Justin Bieber's Justice tour. Sadly, the tour now stands postponed. On June 10, Bieber shared that he has been suffering the effects of the Ramsay Hunt
syndrome paralysing one side of his face.

Before Bieber's dramatic revelation, there was no interest in the virus that also commonly causes chickenpox or shingles. A rare affliction, the history of the Ramsay Hunt syndrome dates back to the early 20th century. Named after American neurologist James Ramsay Hunt, it was first noted in 1906 before research commenced. After having chickenpox or shingles as a child, the virus can lay dormant in the human body. In some rare cases, it may reactivate in adulthood causing the syndrome. While scientists are still working out the exact cause for the virus to reactivate, neurologists explain to us signs to watch out for.

A moment from Bieber's video about his affliction

What is it?

Dr Pavan Pai, consultant neurologist, Wockhardt Hospitals, Mira Road, tells us that the syndrome is a rare disease and affects the facial nerve near the ear. "In this syndrome, the ear's pinna develops vesicles or red rashes. Since the facial nerve that controls the movement of the side of the face runs from behind the ear." The virus causes the nerve to swell and can cause facial paralysis.

Dr Kavita Barhate, consultant neurologist, SRV Mamta Hospital, Dombivli, tells us that not everybody who has chickenpox or shingles may be affected by Ramsay Hunt's syndrome. "While the ears are the most common part affected, the vesicles caused by the virus can also be found on the inside of the eyes."

Dr Kavita Barhate and Dr Pavan Pai

Effects of the syndrome

Originating near the facial nerve, the affliction impairs facial movement in its proximity. Dr Pai explains, "It affects both - the ear and the movement on the side of the face. The side develops weakness, causing an inability to shut the eye, sometimes the face twists and causes difficulty in chewing and swallowing." Bieber has already revealed that he suffers from facial paralysis preventing him from closing his eyes. Dr Barhate adds that in acute cases it can even cause deafness or visual damage depending on the affected organ. "The most prominent sign is the facial asymmetry," she adds. While it only has a minor effect on speech, mechanical movements will be hindered, she says.

"Activities like closing the eyelid, chewing or swallowing liquids - the mechanical things are affected," Dr Barhate explains. She adds that in some cases patients suffer from damaged nerves and severe pain along the distribution region. On his Instagram, Bieber had revealed that he could not blink his eye. "I can't smile on this side of my face. This nostril will not move. So full paralysis in this side of my face," he said, adding that he will be postponing the tour for recovery.

The solution

Both doctors agree that the key to treatment is early diagnosis. If the right treatment is not chosen, it can lead to secondary infections. An early protocol of antivirals and steroids can help bring down the inflammation of the nerve," Dr Pai says.

For Dr Barhate, it is often down to the ignorance of patients. "Many times people ignore the warning signs, particularly with the rash," she says adding, "If not treated promptly, it could rupture and there can be a secondary bacterial infection over that resulting in hearing impairment."

In addition to the medical treatment, doctors also recommend facial physiotherapy to rejuvenate the muscles. "Helping them to close their eyes, blinking or blowing out their cheeks are part of the physiotherapy exercises. Another activity is to try and blow balloons," Dr Pai elaborates.