Ramsay Hunt syndrome

Medical quality assurance by Dr. Albrecht Nonnenmacher, MD at June 30, 2016
StartDiseasesRamsay Hunt syndrome

A direct result of being infected with the virus that causes chickenpox, Ramsay Hunt syndrome is a form of shingles. It is caused by a viral infection of the facial nerve and its symptoms include a painful rash accompanied by weakness in the facial muscles and hearing loss. The rashes associated with this syndrome are usually large, red, painful, and present on the ear. While these symptoms are usually temporary, if left untreated, they can become permanent. Ramsay Hunt syndrome is sometimes referred to as Hunt’s syndrome. 

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Definition & Facts

First discovered in 1907 by James Ramsay Hunt, this condition is most commonly found in people over the age of 60. It occurs when the facial nerve has the same viral infection that causes chickenpox and shingles. The nerve is infected near the inner ear, which causes the nerve to become irritated and swollen. This leads to many of the symptoms associated with Ramsay Hunt syndrome, such as ear ache and vertigo.

One of the most common symptoms of this disease is facial nerve paralysis. Because of this, patients with Ramsay Hunt syndrome sometimes confuse the condition with the symptoms of a stroke. Categorized as a very rare condition, it is estimated that only 5 out of every 100,000 Americans are diagnosed with Ramsay Hunt syndrome each year. 

Symptoms & Complaints

Because Ramsay Hunt syndrome is a type of shingles, one symptom is the identifiable red rash that comes with shingles. The rash forms around, on, or in one ear. Both the roof of the mouth and the tongue can also show signs of the red rash.

The rash is painful and usually has blisters filled with fluid. A person will experience facial paralysis on the same side of the face the rash appears. Typically, people will experience both the rash and the paralysis at the same time, but occasionally one symptom will come before the other. Either the rash will form before facial paralysis or vice-versa.

In a few cases, the only physical symptom of Ramsay Hunt syndrome is facial paralysis with the rash never fully forming. Other symptoms may include hearing loss, ear pain, difficulty closing the eye on the infected side of the face, loss of sense of taste, and a sensation of spinning or moving.

In some cases where Ramsay Hunt syndrome has been allowed to progress without treatment, hearing loss and facial weakness may become permanent. Or, when the Ramsay Hunt syndrome is particularly bad, a patient will never fully recover from the symptoms. In the most serious complications, the infection can spread to other nerves within the body in places such as the brain and spinal cord.

Causes

Ramsay Hunt syndrome is a specific medial case directly caused by the same virus that causes shingles and chickenpox. The virus, varicella zoster virus, which causes chickenpox, can lay dormant in a person’s system even after they have recovered from the initial condition. The virus can lay dormant for several years or even decades, and can reemerge at a later time causing shingles.

Diagnosis & Tests

A combination of a review of a person’s medical history and a physical examination by a medical doctor is all that is usually required to diagnose Ramsay Hunt syndrome. Having chickenpox previously is an indicator for a positive shingles diagnosis later in life. Because the rash that forms from the shingles virus has a very distinguishable appearance and pattern, the doctor may also be able to diagnose from visual observation of symptoms.

For confirmation in some cases, a doctor my want to take a sample of the fluid that fills the blisters in the rash. They may run a PCR (polymerase chain reaction) test to see if the sample tests positive for the varicella zoster virus. A doctor might also take a blood test to detect the presence of the virus. An evaluation of the nerve damage the patient has sustained may be necessary to determine the appropriate treatment and likelihood of a full recovery. 

Treatment & Therapy

There are several ways a doctor can choose to treat Ramsay Hunt syndrome. One common way is to prescribe antiviral drugs that can help fight the virus causing the infection. These drugs may be acyclovir, famciclovir, or valacyclovir. Often, a corticosteroid will be prescribed in conjunction with an antiviral drug. Studies have found that when taken together, the corticosteroid can help increase the effectiveness of the antiviral drug.

If a patient is experiencing vertigo, anti-anxiety drugs may be prescribed to help lessen the symptoms. Because the rash associated with Ramsay Hunt syndrome can be very painful, opioid painkillers such as oxycodone or hydrocodone may be prescribed though are highly addictive substances and carry side effects.

There are also several at home remedies that can be used for treatment. Many over-the-counter pain relievers can help ease the patient’s suffering. Cold compresses applied directly to the rash can also help with the discomfort. If a patient is having difficulty closing his/her eyelid on the affected side of the face, re-wetting eye drops can help ease dry eyes.

With any treatment, it is very important to begin medication very early. Most doctors recommend seeking medical attention and starting medication within three days of the onset of symptoms. By seeking treatment quickly, the chances of having the effects of Ramsay Hunt syndrome become only temporary greatly increases. If left untreated, facial paralysis and hearing loss can become permanent. 

Prevention & Prophylaxis

At the earliest stage of prevention, young children are now commonly vaccinated against the virus that causes chickenpox. Without the virus, shingles and thus Ramsay Hunt syndrome, cannot develop at a later age. For people over 60, there is also a Zoster vaccine available.

While Ramsay Hunt syndrome itself is not contagious, the virus that causes the condition is, and thus it can spread chickenpox to those that have not had it in their lifetime. An infected person should always avoid contact with the very young, women who are pregnant, or those with a weakened immune system.