An Overview of Ramsay Hunt Syndrome

A rare cause of facial paralysis and rash

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Ramsay Hunt syndrome (type II), also called herpes zoster oticus, is a rare neuro-dermatologic condition that causes a rash and facial paralysis. It is caused by the same virus that causes chickenpox and shingles (varicella-zoster virus). The condition was identified and named after neurologist James Ramsey Hunt in 1907.

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, Bell's Palsy is usually temporary and is caused by infection with herpes simplex virus (HSV). The facial paralysis caused by RHS tends to be more severe than it is in patients with Bell's Palsy. In some cases, the paralysis from RHS may be permanent.

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)

Causes

Ramsay Hunt syndrome is caused by reactivation of the varicella-zoster virus—the same virus that causes chickenpox.

This means that any person who has had chickenpox at some point in their lifetime can develop RHS.

A person who has had chickenpox can also develop a painful rash called shingles (herpes zoster) if the virus is reactivated after many years of being inactive (dormant). Once a person develops shingles, they may go on to develop RHS.

When the reactivated virus begins to affect one or more cranial nerves it causes the symptoms of facial paralysis. Researchers aren't sure why the reactivated varicella-zoster virus specifically affects the facial nerves in RHS, but the condition is the second most common cause of atraumatic peripheral facial paralysis.

Diagnosis

Each year in the United States approximately five out of every 100,000 people develop RHS, though researchers have pointed out that frequent missed or misdiagnosis of the condition may mean it's more common than statistics suggest. It appears to affect men and women equally.

RHS is most often diagnosed in people over the age of 60 and those who have compromised immune systems, usually as the result of chronic illness or undergoing treatments that suppress the immune system (such as those for cancer or HIV/AIDS).

Patients with RHS don't always develop the hallmark "triad" of symptoms (rash, ear pain, and facial paralysis) at the same time—or even all three symptoms at all. Therefore, correctly diagnosing the condition can be challenging. When the rash is present, since it is very distinct it is often enough for a doctor to make the diagnosis after performing a thorough clinical exam and taking a careful medical history.

Sometimes blood, saliva, or ear swab samples are taken and tested for the presence of the virus that causes RHS, but this isn't a strict requirement for diagnosing the condition. These "viral studies" can be useful if a person has another dermatologic condition that causes a rash or lesions on the skin that make it difficult to ascertain if the diagnosis of RHS is accurate. Since the symptoms and signs of RHS can sometimes look like other conditions, lab tests can also help doctors determine which virus (herpes simplex or varicella-zoster) is responsible for a patient's symptoms.

Treatment

After a person is diagnosed with RHS, their doctor will want to start treatment as soon as possible. Ideally, treatment with antiviral medications (acyclovir) and sometimes other drugs like steroids (prednisone) should be started within the first three days after symptoms appear.

Getting treated within that three-day timeframe seems to help prevent more serious symptoms or complications, such as severe pain and permanent facial paralysis, from developing. However, even with timely treatment, some people with RHS will have permanent facial paralysis or hearing loss.

Additional treatment will be prescribed depending on the other symptoms, if any, a person with RHS experiences. Dizziness or the sensation of vertigo can often be treated with medications such as antihistamines. Antiemetics can be prescribed to help alleviate nausea and vomiting.

If a person is experiencing pain, opioid painkillers may be prescribed. There are several other types of medications (gabapentin, carbamazepine) that can be prescribed specifically to treat nerve pain. For severe pain, nerve blocks or local anesthetic may be used.

To help prevent RHS, older adults may be advised to receive the shingles vaccine.

A Word From Verywell

Ramsay Hunt syndrome causes a painful rash on the ear and sometimes the mouth, as well as facial paralysis, all of which can affect day-to-day living. You can seek out testimonies of how other individuals have coped with the condition, whether through online forums or someone you may know personally. The symptoms of Ramsay Hunt syndrome may feel debilitating, but they are usually manageable.

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Article Sources
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