Ramsay Hunt syndrome is named after its discoverer, Dr. James Ramsay Hunt, an American neurologist who first described it in 1907. In Ramsay Hunt syndrome the facial nerve becomes infected, which results in a painful rash and weakness of the facial muscles. Ramsay Hunt syndrome can appear upsetting, but if treatment is given in time, the most serious complications, like permanent facial paralysis and complete hearing loss, can be prevented.

Ramsay Hunt syndrome is a neurological disorder caused by the reactivation of the Varicella zoster virus, which is the same virus that causes chicken pox. After the initial infection occurs, the virus lies dormant in the nervous system for many years. This virus may be reactivated – no one understands exactly how – and infect the facial nerve, causing Ramsay Hunt syndrome.

People who have had chicken pox are at heightened risk of developing Ramsay Hunt syndrome. It is more common in people above the age of 60, and it is rarely found in children. People who have a weakened immune system, like HIV patients, are also at high risk, as are diabetics and pregnant women in their third trimester. The reactivation of Varicella zoster virus in a person can spread chicken pox to people who never had chicken pox before.

Ramsay Hunt syndrome is manifested by a weakness of the face on one side, known as palsy. As the facial nerve is affected, someone may experience difficulty making facial expressions and a pronounced droop on one side of the face.

Additional symptoms of Ramsay Hunt syndrome include:

• A painful red rash with fluid-filled blisters that appears on the outside and inside of the ear, on the roof of the mouth, and on the tongue

• Pain in the ears

• Difficulty in hearing

• Vertigo (a sensation of spinning)

• Difficulty closing one eye

• Loss of taste

• Difficulty in eating

The doctor will examine the rash and check for facial weakness. If damage to the facial nerve is detected, then a nerve test is performed to determine the extent of damage, and the potential recovery.

To check for lesions of Varicella zoster virus in the ear, a doctor will use an otoscope to look inside the ear. A lab test called the polymerase chain reaction (PCR) test is performed, in which blood, tears, or fluid from blisters is tested for the Varicella zoster virus. An MRI scan may be performed to check for swelling of the facial nerve. Electromyography (EMG) may be conducted by the doctor to check the health of the muscles and nerves. If diagnosis is not clear, a spinal tap may be conducted, and the cerebrospinal fluid tested for the Varicella zoster virus.

Because Ramsay Hunt syndrome is caused by a virus a doctor may prescribe a seven to ten day course of antiviral medications. For relief from vertigo, the doctor may prescribe diazepam. To reduce swelling, corticosteroids may be prescribed, and there may also be need for painkillers.

If facial muscle weakness causes difficulty in closing one eye, a Botox injection may be given into the upper eyelid, to allow the eye to close. Physical therapy may be recommended, to improve the condition of the facial muscles. In case of complete facial palsy (no facial movement at all), surgical relief of pressure on the facial nerve (caused by inflammation related to the infection) is possible. Recovery may take several weeks to several months; in severe cases complete recovery may not be possible.

Prevention

To prevent Ramsay Hunt syndrome, a Varicella virus vaccination (the “chicken pox vaccine”) is recommended for children between the ages of twelve and eighteen months. This vaccination can also be given to kids of other age groups, and also to adults who have never had chicken pox.

Another vaccination has been approved by the FDA for adults older than 60. This vaccination also helps to prevent other conditions that occur due to the Varicella zoster virus, like shingles.

Outlook

If treatment is started promptly, serious complications can be prevented, and patients have a good chance of a full recovery. If the treatment is started within three days after the symptoms appear, there is a 70 percent chance of a complete recovery. The recovery odds drop to 50 percent if the treatment is started later than that. The younger the patient, the better the chances become for a complete recovery.