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Ramsay Hunt syndrome

 

Definition

Ramsay Hunt syndrome is a disorder of the brain and nervous system (neurologic disorder) that occurs when a virus (varicella zoster) infects certain nerves in the head.

Alternative Names

Hunt syndrome; Herpes oticus

Causes

The varicella zoster virus causes both Ramsay Hunt syndrome and another common cause of facial weakness, Bell's palsy (in which there is no rash).

This virus is part of the family of human herpes viruses, which cause fever sores on the mouth and genital herpes. The varicella zoster virus also causes shingles and chickenpox.

In people with Ramsay Hunt syndrome, the virus is believed to infect the facial nerve near the inner ear. This leads to irritation and swelling.

Symptoms
  • Hearing loss on one side
  • Painful rash on the eardrum, tongue, or roof of the mouth (palate) on the same side as the facial weakness
  • Sensation of things spinning around (vertigo)
  • Weakness of the face with difficulty closing one eye -- smile may look crooked
Signs and tests

The doctor will usually make the diagnosis by looking for signs of weakness in the face and a blister-like (vesicular) rash. Some patients will have a nerve conduction study to find out how much damage has been done to the facial nerve and to see how well they will recover.

Blood tests can help determine whether you have been infected with the varicella zoster virus.

There is a promising laboratory technique called PCR, which can detect very small amounts of the virus's DNA in the skin. However, it is mostly used for research.

The use of pictures of the brain (neuroimaging), such as magnetic resonance imaging (MRI), may show swelling of the facial nerves and help the doctor find out whether the infection has spread to other nerves or the brain. A spinal tap is used in rare cases, especially when the diagnosis is not clear.

Support Groups

Expectations (prognosis)

The more severe the damage, the longer it will take to recover, and the lower the chance that you will completely regain normal function. If there is not much damage to the nerve, then you should get better completely within a few weeks. If damage is more severe, you may not fully recover-- even after several months.

Overall, chances of recovery are better if the treatment is started within 3 days of when the symptoms begin. If treatment is started at this time, 70% of patients make a full recovery.

However, when the treatment is delayed more than 3 days, the chances of complete recovery drop to about 50%. Children are more likely to have a complete recovery than adults.

Recovery may be complicated if the nerve grows back to the wrong areas (synkinesis), which may cause inappropriate responses, such as tears when laughing or chewing (crocodile tears). Some other people may experience blinking of the eye when they talk or chew food.

Calling your health care provider

Call your health care provider if you lose movement in your face, or you have a rash on your face that occurs with facial weakness.

Complications

Severe paralysis will lead to a poor recovery, possibly leaving you with permanent loss of movement in your face (facial paralysis) and uncontrolled movements (synkinesis).

If the eyelid doesn't close properly, damage to the cornea can occur. This can result in eye pain and blurred vision.

Occasionally, the virus may spread to other nerves, or even to the brain and spinal cord, causing:

  • Back pain
  • Confusion
  • Drowsiness (lethargy)
  • Headaches
  • Limb weakness

This may require a hospital stay, where a spinal tap may help find out whether other areas of the nervous system have been infected.

Treatments

Treatment with antiviral medications, such as acyclovir or famciclovir, is recommended for 7 - 10 days, along with strong anti-inflammatory drugs called steroids (such as prednisone) for 3 - 5 days.

The steroids are tapered off in about 1 week. Sometimes strong painkillers are also needed if the pain continues even with steroids. While you have weakness of the face, wear an eye patch to prevent injury to the cornea (corneal abrasion) and damage to the eye if it does not close completely.

Prevention

There is no known way to prevent Ramsay Hunt syndrome, but taking medication early in the course of the symptoms can improve recovery.


Review Date: 2/6/2008
Reviewed By: Daniel Kantor, MD, Director of the Comprehensive MS Center, Neuroscience Institute, University of Florida Health Science Center, Jacksonville, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
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