Overview

Ramsay Hunt syndrome (herpes zoster oticus) occurs when a shingles outbreak affects the facial nerve near one of your ears. In addition to the painful shingles rash, Ramsay Hunt syndrome can cause facial paralysis and hearing loss in the affected ear.

Ramsay Hunt syndrome is caused by the same virus that causes chickenpox. After chickenpox clears up, the virus still lives in your nerves. Years later, it may reactivate. When it does, it can affect your facial nerves.

Prompt treatment of Ramsay Hunt syndrome can reduce the risk of complications, which can include permanent facial muscle weakness and deafness.

Symptoms

The two main symptoms of RHS are a painful rash on the ear and facial paralysis. These symptoms usually occur on just one side (unilateral) and they may not happen at the same time.

The rash appears red with blisters and is usually very painful. While it typically affects the inner and outer portion of the ear, it can also affect the mouth and throat. The pain a person feels in their ear canal may be very intense and may spread down into their neck.

With facial paralysis—a condition also known as palsy—the muscles of the face may feel stiff. A person may find that they are having a hard time making facial expressions, speaking, or closing the eye on the side that is affected. Sometimes when a person seeks medical attention for these symptoms they are mistakenly diagnosed with another similar condition called Bell’s Palsy.

While the two conditions may look and feel similar, the key difference is that RHS causes a painful rash. Some people with RHS get the ear rash without the facial paralysis. Doctors sometimes refer to these cases as zoster sine herpete.

People with RHS may experience other symptoms, including:

• Nausea and vomiting
• Dizziness or vertigo
• Hearing loss
• Facial pain that may be accompanied by a runny nose or watering eyes
• Ringing in the ears (tinnitus)
• Sounds seem much louder than normal (hyperacusis)
• If the rash affects the mouth and throat, symptoms such as dry mouth and loss of taste
• Dry eyes or involuntary movement of the eye (nystagmus)

Exams and Tests

A health care provider will usually diagnose Ramsay Hunt Syndrome by looking for signs of weakness in the face and a blister-like rash.

Tests may include:

• Blood tests for varicella-zoster virus
• Electromyography (EMG)
• Lumbar puncture (in rare cases)
• MRI of the head
• Nerve conduction (to determine the amount of damage to the facial nerve)
• Skin tests for varicella-zoster virus

Treatment

Strong anti-inflammatory drugs called steroids (such as prednisone) are usually given. Antiviral medicines, such as acyclovir or valacyclovir may be given.

Sometimes strong painkillers are also needed if the pain continues even with steroids. While you have weakness of the face, wear an eye patch to prevent injury to the cornea (corneal abrasion) and other damage to the eye if the eye does not close completely. Some people may use a special eye lubricant at night and artificial tears during the day to prevent the eye from drying out.

If you have dizziness, your provider can advise other medicines.

Outlook (Prognosis)

If there is not much damage to the nerve, you should get better completely within a few weeks. If damage is more severe, you may not fully recover, even after several months.

Overall, your chances of recovery are better if the treatment is started within 3 days after the symptoms begin. When treatment is started within this time, most people make a full recovery. If treatment is delayed for more than 3 days, there is less of a chance of complete recovery. Children are more likely to have a complete recovery than adults.

Possible Complications

Complications of Ramsay Hunt syndrome may include:

• Changes in the appearance of the face (disfigurement) from loss of movement
• Change in taste
• Damage to the eye (corneal ulcers and infections), resulting in a loss of vision
• Nerves that grow back to the wrong structures and cause abnormal reactions to a movement — for example, smiling causes the eye to close
• Persistent pain (postherpetic neuralgia)
• Spasm of the face muscles or eyelids

Prevention

There is no known way to prevent Ramsay Hunt syndrome, but treating it with medicine soon after symptoms develop can improve recovery.

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