Ramsay Hunt Syndrome

OVERVIEW

Ramsay Hunt syndrome is a complication of varicella-zoster virus infections, including chickenpox or shingles (herpes zoster). Other names for the condition include herpes zoster oticus and Ramsay Hunt syndrome type 2. It got its name when an American army officer and neurologist named James Ramsay Hunt first described the disorder in 1907.

If you had chickenpox as a child, the virus that caused it (varicella-zoster virus) can remain inactive (dormant) in your nerves for years. Shingles occurs when the previously dormant varicella-zoster virus reactivates. The reactivation of the varicella-zoster virus in your facial nerve is called Ramsay Hunt syndrome.

Ramsay Hunt syndrome causes facial nerve paralysis (facial palsy) and intense ear pain. In addition, it may cause a painful rash on your ear, face or mouth.

Ramsay Hunt syndrome can affect anyone who’s had chickenpox or shingles. Most cases occur in people age 60 and older. The condition rarely affects children but has been diagnosed as young as three years old. People who are immunocompromised are more likely to be affected and generally experience more severe symptoms. They’re also less likely to have a complete recovery.

Ramsay Hunt syndrome affects about 5 out of every 100,000 people each year in the U.S.

However, some researchers think the condition is sometimes undiagnosed or misdiagnosed. This makes its true frequency more difficult to determine.

The disorder is the second most common cause of facial nerve paralysis, accounting for about 7% of all cases. Bell’s palsy is the most common cause of facial nerve paralysis.

Symptoms

• Facial paralysis
• Facial paralysis Open pop-up dialog box
• The two main signs and symptoms of Ramsay Hunt syndrome are:
• A painful red rash with fluid-filled blisters on, in and around one ear
• Facial weakness or paralysis on the same side as the affected ear
• Usually, the rash and the facial paralysis occur at the same time. Sometimes one can happen before the other. Other times, the rash never occurs.

If you have Ramsay Hunt syndrome, you might also experience:

• Ear pain
• Hearing loss
• Ringing in your ears (tinnitus)
• Difficulty closing one eye
• A sensation of spinning or moving (vertigo)
• A change in taste perception or loss of taste
• Dry mouth and eyes

When to see a doctor

Call your doctor if you experience facial paralysis or a shingles rash on your face. Treatment that starts within three days of the start of signs and symptoms may help prevent long-term complications.

Causes

Ramsay Hunt syndrome occurs when the varicella-zoster virus reactivates in the facial nerve, which is the cranial nerve that affects your face, tongue and inner ear. The varicella-zoster virus is the same virus that causes chickenpox and shingles.

If you had chickenpox as a child, the virus can remain inactive (dormant) in your nerves for years. Shingles occurs when the previously dormant varicella-zoster virus reactivates. The virus travels down your nerve fibers into your skin, causing painful sores. The virus sometimes develops into Ramsay Hunt syndrome, affecting your facial nerve. Researchers aren't sure why the virus sometimes involves the facial nerve.

Is Ramsay Hunt syndrome contagious?

Ramsay Hunt syndrome isn’t contagious. But if you have the condition, it means you have the varicella-zoster virus. The virus can spread if someone comes in contact with the fluid from blisters that form in the affected person. It’s most likely to cause chickenpox in people who’ve never had the disease or gotten the chickenpox vaccine. Ramsay Hunt syndrome can cause serious health issues in people with compromised immune systems. It’s a good idea to avoid certain groups of people until your blisters scab over. These people include:

• People who’ve never had chickenpox or gotten the chickenpox vaccine.
• People with weakened immune systems.
• Newborns.
• Pregnant people.

Diagnosis

Doctors often can identify Ramsay Hunt syndrome based on medical history, a physical exam, and the disorder's distinctive signs and symptoms. To confirm the diagnosis, your doctor might take a sample of fluid from one of the rash blisters in your ear for testing.

Treatment

Prompt treatment of Ramsay Hunt syndrome can ease pain and decrease the risk of long-term complications. Medications may include:

Antiviral drugs. Medications such as acyclovir (Zovirax), famciclovir (Famvir) or valacyclovir (Valtrex) often help combat the chickenpox virus.

Corticosteroids. A short regimen of high-dose prednisone appears to boost the effect of antiviral drugs in Ramsay Hunt syndrome.

Anti-anxiety medications. Drugs such as diazepam (Valium) can help relieve vertigo.

Pain relievers. The pain associated with Ramsay Hunt syndrome can be severe. Prescription pain medications may be needed.

Lifestyle and home remedies

• The following can help reduce the discomfort of Ramsay Hunt syndrome:
• Keep areas affected by the rash clean.
• Apply cool, wet compresses to the rash to ease pain.
• Take an over-the-counter pain reliever or anti-inflammatory drug, such as ibuprofen (Advil, Motrin IB, others).
• If facial weakness makes it difficult for you to close one of your eyes, take the following steps to protect your vision:
• Use moisturizing eyedrops throughout the day if your eye becomes dry.
• At night, apply ointment to the eye and tape your eyelid shut or wear an eye patch.

Preparing for your appointment

You're likely to start by first seeing your family doctor. He or she may refer you to a doctor who specializes in nervous system disorders (neurologist) or to an ear, nose and throat specialist (otolaryngologist).

What you can do

Before your appointment, you may want to write a list of answers to the following questions:

• What are your symptoms? When did they start?
• Have you had the sensation that the room is spinning (vertigo)?
• Has your hearing been affected?
• Have you noticed a change in your sense of taste?
• Have you had the chickenpox (varicella) vaccine? When?
• Have you ever had chickenpox? When?
• Are you being treated for any chronic health conditions? If so, what treatments are you receiving?
• Are you pregnant?

What to expect from your doctor

During the physical exam, your doctor will closely examine your face to check for evidence of one-sided paralysis or a shingles rash on, in or around your ear.