What Is Ramsay Hunt Syndrome?

The rare neurological disorder known as Ramsay Hunt syndrome affects a very small number of individuals worldwide, but is gaining significant attention after pop star Justin Bieber announced that he had been diagnosed with the condition.

Approximately 5 in 100,000 people are affected by Ramsay Hunt syndrome, according to the National Organization for Rare Disorders.

John Cullen, MD, is a neurologist with Rochester Regional Health and explains more about this syndrome.

What is Ramsay Hunt syndrome?

The name of the syndrome is derived from an American neurologist named James Ramsay Hunt, who was the first to describe the condition in the early 20^th century.

Caused by the varicella-zoster virus, the same virus that causes chickenpox in children and shingles in adults, Ramsay Hunt syndrome is a rare late-stage complication of the viral infection. In cases of Ramsay Hunt syndrome, the previously inactive or dormant varicella-zoster virus is reactivated and spreads to affect the facial nerve.

Symptoms

The symptoms of Ramsay Hunt syndrome vary from case to case. Affected individuals usually experience paralysis of the facial nerve and a rash around the ear. These two symptoms do not always occur simultaneously. In most cases, only one side of the face is affected.

Other symptoms may include:

• Ringing in the ear (tinnitus)
• Ear pain
• Changes in taste sensation
• Dry eye
• Vertigo
• Sensitivity to sounds
• Stuffy nose
• Slurred speech

Research suggests approximately 70 percent of individuals diagnosed with Ramsay Hunt syndrome regain their original facial nerve functions, although some deal with involuntary facial muscle movements due to the effects of the virus on facial nerves.

Prevention and treatment

There is no known method of prevention for Ramsay Hunt syndrome, although the recommended immunization schedule for children includes the varicella vaccine. If an individual has ever had chickenpox and is infected with the varicella-zoster virus, the virus can linger in the body for long periods of time, before reawakening and inflaming the nerves in the face.

Most of the treatment options for individuals with Ramsay Hunt syndrome are antiviral medications and steroids. Physicians are encouraged to start patients on medications as soon as possible to achieve the best possible effects.

Individuals with dry eye can use artificial tears and ocular lubricant ointment to help relieve this symptom.

Pain management can be achieved using acetaminophen, ibuprofen, and/or gabapentin as prescribed by an individual’s primary care provider.

If you start to notice some of the previously mentioned symptoms, seek medical attention as soon as possible. Facial drooping can be a sign of stroke or Bell’s Palsy, as well as Ramsay Hunt syndrome. The sooner that a medical professional can assess you, the better your odds of recovery.

“Most individuals who are diagnosed with Ramsay Hunt syndrome will regain function, but they may not return to the same level of health they had prior to their diagnosis,” Dr. Cullen said. “Focusing on improvement and not complete restoration to a prior state of function is a healthy way to approach this condition.”