According to the American Academy of Otolaryngology-Head and Neck Surgery, Ramsay Hunt syndrome (RHS) is a rare but serious condition caused by reactivation of the herpes virus varicella-zoster, the same virus that causes chickenpox and shingles. When this virus gets into your body it can remain dormant in nerve cells and reactivate years later, usually causing shingles, but sometimes causing RHS. About one percent of varicella reactivation causes RHA, the rest of the infections become shingles.
Signs and Symptoms of Ramsay Hunt Syndrome
Varicella-zoster virus remains in nerve cells after chickenpox. When they reactivate, they follow the path of the nerve where they live. In shingles, it may be a nerve that wraps around your chest or belly. In RHS, they follow the facial nerve. On each side of your head, your facial nerve leaves your brain near your inner ear and comes out in front of your outer ear, where it branches out to supply the muscles of your face.
Because it is a reactivation of a viral infection, you may have early symptoms of fever and fatigue followed by weakness or complete loss of movement on one side of the face, a chicken pox-like rash on your ear or face, and pain one side of your face or ear. These signs and symptoms may appear all at once or in any order.
Facial weakness or paralysis makes your face droop. You will lose the wrinkles in your forehead and your eye will stop blinking. You may not be able to close your eye completely. The rash may be in your ear canal, outside your ear, or on your face. Over time, the rash develops tiny blisters, called vesicles (vesicular rash), that may burst, drain clear fluid, and scab over. The fluid contains live virus and can spread the virus to other people causing chickenpox if they have not had chickenpox or been vaccinated for varicella zoster. Like shingles, the pain of RHS can be severe and can last longer than the other signs.
In addition to supplying the muscles of your face, the facial nerve also has branches that supply parts of your inner ear and your tongue, so other symptoms may be sensitivity to loud noise and a change or loss of taste. The infection may also spread to the inner ear and cause hearing loss, ringing in the ear, and dizziness.
Diagnosis and Treatment of Ramsay Hunt Syndrome
The signs and symptoms of RHS – facial paralysis, vesicular rash, and pain – do not occur with any other diseases, so they are usually enough to make the diagnosis. An MRI imaging study may show the inflammation of the facial nerve, but it is rarely needed for diagnosis.
In about 70 percent of cases, signs and symptoms of RHS improve without treatment, but it may take weeks or even up to a year. Treatment is given to improve recovery and prevent long-term complications.
“It’s important to start treatment early so symptoms don’t become too severe and you minimize the risk of long-term complications. The first-line treatments include an antiviral, such as valacyclovir, and a steroid, such as prednisone,” says Dr. Yasser Hussain, Neurologist at Austin Neuromuscular Center.
Antiviral drugs and steroids may be given for up to 21 days. Another important treatment is treatment to protect the eye. Because you can’t close your eye with facial paralysis, dryness can damage the outer surface of the eye. To prevent this damage, the eye may be taped shut and artificial tears used frequently to keep it moist. Pain control is important, because the pain of RMS, like shingles can be severe. Medications may also be used to reduce dizziness.
Prognosis and Complications
About 30 percent of people with RHS will not have complete recovery of facial nerve function. There may be some residual weakness or abnormal facial nerve function called synkinesis. Synkinesis occurs when facial nerve fibers regenerate, but make abnormal connections. This may result in movement of the eye when moving the mouth, or flowing of tears when you produce saliva while eating. People with more severe paralysis at the beginning of RHS are more likely to have these long-term complications. Another complication is pain that continues long after other symptoms are gone, called post-herpetic neuralgia. This can also occur with shingles. Pain medications and physical therapy may be need for people with long-term complications.
RHS can occur at any age but is most common in people between the ages of 70 to 80. The reactivation of varicella-zoster virus may be triggered by physical stress, an infection, or a weakened immune system from cancer treatment or poor nutrition. Although it is not 100 percent effective, getting the shingles vaccine is the best way to prevent RHS. Treatment helps if started within the first week after symptoms start, so let your doctor know right away if you have one-sided facial weakness, pain, or a vesicular rash on your face or ear.