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Facial nerve

Ramsay Hunt syndrome is an infection of your facial nerve that's accompanied by a painful rash and facial muscle weakness, among other signs and symptoms.

The cause of Ramsay Hunt syndrome is varicella-zoster virus, the same virus that causes chickenpox. After chickenpox heals, the virus lies dormant in your nerves. Years later, it may reactivate. If the virus reactivates and infects your facial nerve, the result is Ramsay Hunt syndrome.

The onset of Ramsay Hunt syndrome can be frightening. The symptoms may cause some people to fear they're having a stroke. But, there's often effective treatment for Ramsay Hunt syndrome. Sometimes, Ramsay Hunt syndrome can lead to permanent facial muscle weakness and hearing loss. Prompt treatment can reduce your risk of complications.

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Facial weakness

Signs and symptoms of Ramsay Hunt syndrome include:

• A painful red rash with fluid-filled blisters on your eardrum, external ear canal, the outside of your ear, the roof of your mouth (palate) or your tongue
• Facial weakness (palsy) on the same side as the affected ear
• Difficulty closing one eye
• Ear pain
• Hearing loss
• Ringing in your ears (tinnitus)
• A sensation of spinning or moving (vertigo)
• A change in taste perception or loss of taste

When to see a doctor
If you experience weakness on one side of your face or if you develop a rash in or around your ear or in your mouth along with facial weakness, call your doctor for an urgent evaluation. Ramsay Hunt syndrome isn't a condition that requires emergency care. But, diagnosis and treatment within seven days of the start of signs and symptoms may help prevent long-term complications.

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Facial nerve

The cause of Ramsay Hunt syndrome is a reactivation of the varicella-zoster virus — the same virus that causes chickenpox.

Varicella-zoster is part of a family of viruses called herpes viruses, which includes the viruses that cause cold sores and genital herpes. Many of these viruses can lie hidden in your nervous system after the first infection and remain dormant for years before causing another infection.

If your immune system doesn't destroy all the varicella-zoster virus during the initial infection, the remaining virus may reactivate later and infect your facial nerve — producing Ramsay Hunt syndrome. Reactivation of this virus can also cause other disorders, including shingles (herpes zoster). Researchers don't clearly understand how or why the varicella-zoster virus re-emerges.

Ramsay Hunt syndrome is also called herpes zoster oticus.

Anyone who has had chickenpox can develop Ramsay Hunt syndrome. But, it's more common in older adults, typically affecting people older than 60. Ramsay Hunt syndrome is rare in children.

Ramsay Hunt syndrome isn't contagious. However, reactivation of the varicella-zoster virus can cause chickenpox in people you come in contact with if they haven't previously had chickenpox. The infection can be serious for people with immune system deficiencies.

Until the rash blisters scab over, avoid physical contact with:

• Anyone who's never had chickenpox
• Anyone who has a weak immune system
• Newborns
• Pregnant women (because chickenpox infection can be dangerous for a developing baby)

If treated within the first seven days, most people with Ramsay Hunt syndrome don't have long-term complications. However in some cases, even with prompt treatment, Ramsay Hunt may result in permanent hearing loss and facial weakness.

Other possible complications of Ramsay Hunt syndrome include:

• Unusual facial movements. A condition called synkinesis may develop if the infection severely damages your facial nerve and the nerve grows back to the wrong place. This can cause inappropriate nerve responses, such as blinking or tears when you're talking, laughing or chewing.
• Eye damage. The facial weakness caused by Ramsay Hunt syndrome may make it difficult for you to close your eyelid on the side of your face that's affected. Incomplete eyelid closure can lead to damage of the protective dome of clear tissue over the front of your eye (cornea). This damage can cause eye pain and blurred vision.
• Effects on other parts of your body. In rare cases, the varicella-zoster virus may spread to other nerves, or to your brain or spinal cord, causing headaches, back pain, confusion, lethargy and limb weakness.
• Postherpetic neuralgia. Postherpetic neuralgia may develop as a result of nerve fiber damage. Damaged fibers aren't able to send messages from your skin to your brain, as they normally do. Instead, the messages become confused and exaggerated, causing pain that may persist long after other signs and symptoms of Ramsay Hunt syndrome have faded.

You're likely to start by first seeing your family doctor. However, in some cases when you call to set up an appointment, you may be referred to a doctor who specializes in nervous system disorders (neurologist) or to an ear, nose and throat surgeon (otolaryngologist).

Because appointments can be brief, and because there's often a lot of ground to cover, it's a good idea to be well prepared for your appointment. Here's some information to help you get ready and what to expect from the doctor.

What you can do

• Be aware of any pre-appointment restrictions. At the time you make the appointment, be sure to ask if there's anything you need to do in advance.
• Write down any symptoms you're experiencing, including any that may seem unrelated to the reason for which you scheduled the appointment. For example, your doctor will want to know if you've noticed any changes in the way things taste.
• Write down key personal information, including any major stresses or recent changes in your life. Also note any recent travel abroad.
• Make a list of your key medical information, including other medical problems for which you've recently been treated and the names of any medications you're taking. Be sure to tell your doctor if you have been diagnosed with an immune system disorder or if you are taking any medications, such as prednisone, that suppress your immune system. If you have access to your vaccination history, bring a copy to your appointment.
• Take a family member or friend along, if possible. Sometimes it can be difficult to soak up all the information provided to you during an appointment. Someone who accompanies you may remember something that you missed or forgot.
• Write down questions to ask your doctor.

Your time with your doctor may be limited, so preparing a list of questions will help you make the most of your time together. List your questions from most important to least important in case time runs out.

For Ramsay Hunt syndrome, some basic questions to ask your doctor include:

• What is likely causing my symptoms or condition?
• Are there any other possible causes for these symptoms or this condition?
• What treatment do you recommend?
• What are the possible side effects of the medication you're prescribing?
• How soon after I begin treatment can I expect improvement in my symptoms?
• What is the risk that this condition will recur?
• Am I at risk of long-term complications? If so, what are they?
• Am I contagious? For how long?
• What steps should I take to reduce the risk of spreading this illness?
• Does this condition increase my risk of any other medical problems?
• I have these other health conditions. How can I best manage them together?
• Should I see a specialist? What will that cost, and will my insurance cover seeing a specialist?
• Are there any brochures or other printed material that I can take home with me? What Web sites do you recommend visiting?

In addition to the questions that you've prepared to ask your doctor, don't hesitate to ask questions during your appointment at any time that you don't understand something.

What to expect from your doctor
Your doctor is likely to ask you a number of questions. Being ready to answer them may reserve time to go over any points you want to talk about in-depth. Your doctor may ask:

• What are your symptoms?
• When did you first begin experiencing symptoms?
• Have your symptoms changed over time? If so, how?
• 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 your symptoms included a headache?
• Have you had the chickenpox (varicella) vaccine? When?
• Have you ever had chickenpox? When?
• Have you recently been sick with any short-term illness, such as a cold or the flu?
• Are you being treated for any chronic health conditions? If so, what treatments are you receiving?
• Have you recently undergone any surgical procedures?
• Have you traveled abroad recently?
• Are you pregnant?

What you can do in the meantime
While you wait for your appointment, try taking an over-the-counter pain reliever or anti-inflammatory drug, such as ibuprofen (Advil, Motrin, others), to relieve your discomfort. Cool, wet compresses also may ease pain. Take care to keep the skin around your rash clean, and rest as much as possible.

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 may take a sample of fluid from one of the rash blisters in your ear and perform a polymerase chain reaction (PCR) test on it. This test can detect the varicella-zoster virus. A PCR test can also be done on a blood or tear sample. But, testing fluid from your ear offers more-reliable results.

Prompt treatment of Ramsay Hunt syndrome can ease pain and decrease your risk of long-term complications. To treat Ramsay Hunt, your doctor may prescribe the following medications:

• Antiviral medication, such as acyclovir (Zovirax), famciclovir (Famvir) or valacyclovir (Valtrex)
• Corticosteroids, such as prednisone, to reduce swelling and pain
• Diazepam (Valium), to relieve vertigo
• Pain relievers

If facial weakness remains after treatment, physical therapy involving facial exercises may help you improve or regain control of your facial muscles.

If you have trouble closing one of your eyes because of facial weakness, your doctor may recommend an injection of botulinum toxin type A (Botox) into your upper eyelid, allowing it to close and protect your eye.

Depending on the extent of damage to your facial nerve, recovery from Ramsay Hunt syndrome can take from several weeks to several months. If damage is severe, or if treatment is delayed, full recovery may not be possible.

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, others).
• If you have facial weakness, use the unaffected side of your mouth for chewing.
• Practice good oral hygiene, especially after eating.
• Get plenty of rest.

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.

There's no way to prevent Ramsay Hunt syndrome once the varicella-zoster virus is in your body. But, the varicella virus vaccine (Varivax) can reduce your risk of getting the virus in the first place. This vaccine is a routine childhood immunization, given between ages 12 months and 18 months. The vaccine is also recommended for older kids and adults who've never had chickenpox. The varicella virus vaccine prevents chickenpox for most people. If chickenpox does develop after vaccination, it's typically less severe.

The Food and Drug Administration (FDA) has also approved a vaccine (Zostavax) to help prevent shingles and other conditions related to the varicella-zoster virus, such as Ramsay Hunt syndrome, in adults age 60 and older. This vaccine is given as a single injection, preferably in the upper arm. The most common side effects are redness, pain and swelling at the injection site, itching and headache.