Ramsay Hunt Syndrome (RHS)

What is Ramsay Hunt Syndrome (RHS)

Ramsay Hunt syndrome is a viral infection that affects the nerves in the face and the nerves near the inner ear. The infection is caused by the varicella zoster virus (VZV). This is the same virus that causes chickenpox and shingles.

After a person has chickenpox, this virus may become inactive. Years later, the virus can become active again and cause Ramsay Hunt syndrome. The trigger may be something that weakens the body’s defense system (immune system), like stress.

When VZV becomes activated, it moves up the facial nerve and causes a painful rash in or around the ear canal. It may also travel up the nerve that is responsible for hearing.

Ramsay Hunt syndrome is a herpetic infection of the geniculate ganglion of the facial nerve. Symptoms include ear and facial pain, lower motor neuron facial weakness, and vesicular eruption around the external auditory canal. Treatment comprises oral acyclovir and corticosteroids.

Ramsay Hunt syndrome cannot be passed from person to person (it is not contagious). However, if a person who has never had chickenpox comes in contact with fluid from someone’s skin blisters, he or she may develop chickenpox.

What are the causes?

This condition is caused by the varicella zoster virus.

What increases the risk?

You may be at risk for Ramsay Hunt syndrome if you have had chickenpox in the past.

What are the symptoms?

Symptoms of this condition include:

  • A rash with blisters that breaks out around the ear. The rash may be accompanied by:
    • A rash in the inner ear, along the side of the face, or up the scalp.
    • A rash inside the mouth.
    • Deep and severe pain in the ear.
    • Severe, burning pain wherever the rash develops.
  • Facial nerve weakness. This may cause:
    • Drooping on one side of the face.
    • Inability to close the eyelid on the affected side of the face.
    • Trouble eating.
    • Loss of ability to taste on the side of the tongue.

If RHS affects the inner ear nerve (auditory nerve), other symptoms may be present. These may include:

  • Hearing loss.
  • A spinning sensation (vertigo).
  • Clumsiness.
  • Ringing in the ear (tinnitus).

How is this diagnosed?

This condition may be diagnosed based on:

  • Your symptoms.
  • A physical exam.
  • Other tests to confirm the diagnosis. These may include:
    • Viral culture. This test is done by swabbing the rash or blister to check for VZV.
    • Blood tests to check for antibodies to VZV. Antibodies are proteins that your body produces in response to germs.
    • Nerve conduction studies (electroneurogram).
    • MRI scan.
    • Hearing tests (audiology).

How is this treated?

This condition may be treated with:

  • An antiviral medicine to treat the virus.
  • NSAIDs or prescription pain relievers to control pain.
  • An anti-inflammatory medicine (steroid) called prednisone.
  • Antibiotic medicine, if the rash becomes infected.

If treatment starts within the first 3 days of having symptoms, it will shorten the course of the pain and rash that is caused by RHS. Treatment will also prevent your facial nerve from continuing to weaken. Without treatment, it is possible that you may not recover full use of your facial nerve.

Follow these instructions at home:

Medicines

  • Take over-the-counter and prescription medicines only as told by your health care provider.
  • If you were prescribed an antibiotic or antiviral medicine, take or apply it as told by your health care provider. Do not stop using the antibiotic or antiviral medicine even if your condition improves.
  • Do not drive or use heavy machinery while taking prescription pain medicine.
  • If you are taking prescription pain medicine, take actions to prevent or treat constipation. Your health care provider may recommend that you:
    • Drink enough fluid to keep your urine pale yellow.
    • Eat foods that are high in fiber, such as fresh fruits and vegetables, whole grains, and beans.
    • Limit foods that are high in fat and processed sugars, such as fried or sweet foods.
    • Take an over-the-counter or prescription medicine for constipation.

General instructions

  • If told by your health care provider, use artificial tears and wear an eye patch to protect your eye until you can close your eyelid again.
  • Do not scratch or pick at the rash.
  • Put a cold, wet cloth (cold compress) on the itchy area as told by your health care provider.
  • If you have blisters in your mouth, do not eat or drink spicy, salty, or acidic things. Soft, bland, and cold foods and beverages are easiest to swallow.
  • Keep all follow-up visits as told by your health care provider. This is important.

Contact a health care provider if:

  • Your pain medicine is not helping.
  • You have chills or fever.
  • Your symptoms get worse.
  • Your symptoms have not gone away after 2 weeks.
  • You have any changes in vision.

Summary

  • Ramsay Hunt syndrome (RHS) is a viral infection that affects the nerves in the face and the nerves near the inner ear. The infection is caused by the varicella zoster virus (VZV), which also causes chicken pox and shingles.
  • After a person has chickenpox, this virus may become inactive. If the inactive VZV virus becomes activated, it moves up the facial nerve and causes a painful rash in or around the ear canal. It can also cause hearing loss and facial nerve weakness, with drooping on one side of the face.
  • If treatment starts within the first 3 days of having symptoms, it will shorten the course of the pain and rash that are caused by RHS. Treatment will also prevent your facial nerve from continuing to weaken.
  • Treatments may include antiviral, steroid, and pain medicines.
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