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Ramsay Hunt syndrome


Definition:

Ramsay Hunt syndrome is a painful rash around the ear that occurs when the varicella zoster virus infects a nerve in the head.

Alternative Names:

Hunt syndrome; Herpes oticus

Causes:

The varicella zoster virus that causes Ramsay Hunt syndrome is the same virus that causes chickenpox and shingles.

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 of the nerve.

Symptoms:
  • Painful rash on the eardrum, ear canal, earlobe, tongue, and roof of the mouth on the side where there is weakness of the face
  • Hearing loss on one side
  • Sensation of things spinning (vertigo)
  • Weakness on one side of the face that causes difficulty closing one eye, eating (food falls out of the weak corner of the mouth), making expressions, and making fine movements of the face, as well as facial droop and paralysis on one side of the face
Exams and Tests:

A doctor will usually diagnose Ramsay Hunt Syndrome by looking for signs of weakness in the face and a blister-like rash.

Tests may include:

Treatment:

Strong anti-inflammatory drugs called steroids (such as prednisone) are usually prescribed for 5 - 7 days. Antiviral medications, such as acyclovir or valacyclovir, can be given for 7 - 10 days, although the benefit of antiviral medications is uncertain.

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 other damage to the eye if the eye does not close completely. Some people may use a special eye lubricant at night and artificial tears during the day to prevent the eye from drying out.

If you have dizziness, your doctor can recommend other medications.

Outlook (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, you should get better completely within a few weeks. If damage is more severe, you may not fully recover, even after several months.

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

However, when the treatment is delayed for more than 3 days, the chances of a 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. When this happens, inappropriate responses, such as tears when laughing or chewing may occur. Other people may experience blinking of the eye when they talk or chew food.

Possible Complications:
  • Changes to the appearance of the face (disfigurement) from loss of movement
  • Changes to taste
  • Damage to the eye (corneal ulcers and infections), resulting in a loss of vision
  • Nerves that grow back to the wrong structures and cause abnormal reactions to a movement -- for example, smiling causes the eye to close
  • Persistent pain (postherpetic neuralgia)
  • Spasm of the face muscles or eyelids

Occasionally, the virus may spread to other nerves, or even to the brain and spinal cord. This can cause:

  • Confusion
  • Drowsiness
  • Headaches
  • Limb weakness
  • Nerve pain

If these symptoms occur, a hospital stay may be needed. A spinal tap may help determine whether other areas of the nervous system have been infected.

When to Contact a Medical Professional:

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

Prevention:

There is no known way to prevent Ramsay Hunt syndrome, but treating it with medication soon after symptoms develop can improve recovery.

References:

Baloh RW, Jen J. Hearing and equilibrium. In: Goldman L, Ausiello D, eds. Goldman's Cecil Medicine. 24th ed. Philadelphia, PA: Elsevier Saunders; 2011:chap 436.

Rucker JC. Cranial neuropathies. In: Daroff RB, Fenichel GM, Jankovic J, Mazziotta JC. Bradley's Neuropathy in Clinical Practice. 6th ed. Philadelphia, PA: Elsevier Saunders; 2012:chap 70.


Review Date: 5/28/2014
Reviewed By: Joseph V. Campellone, MD, Division of Neurology, Cooper University Hospital, Camden, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed physician should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. Copyright 2002 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.

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