Ramsay Hunt Syndrome

1 What is Ramsay Hunt syndrome?

Ramsay Hunt syndrome (also termed Hunt’s Syndrome and herpes zoster oticus) is an infection of a facial nerve. It is a complication of shingles which are caused by the same virus that causes chickenpox (varicella-zoster virus, or VZV). In fact, people who have had chicken pox carry the dormant virus in their nerves. Some years later it may become active again, infecting the facial nerve. This cluster of symptoms calls RHS.【1】

If you have RHS, the condition involves a painful rash and facial muscle weakness, which is sometimes confused with a stroke. As a matter of fact, Ramsay Hunt syndrome is rare. About 5 out of every 100,000 people in the U.S. get it each year.【2】

Ramsay Hunt syndrome is usually effectively treated, but some people may have permanent facial muscle weakness and hearing loss. Therefore, fast treatment is key. The sooner your doctor gets you to medicine for RHS, the better your chances are for a good recovery.

2 What Causes it?

RHS is caused by the reactivation of the herpes zoster virus. This virus has previously caused chickenpox in the patient. In people with this syndrome, the virus infects the facial nerve near the inner ear. This leads to irritation and swelling of the nerve.

Although the specific reason for virus reactivation is unknown, experts believe that stress triggers RHS. Many studies have shown that stress can weaken the immune system and that people under significant stress are more likely to suffer from infections than those who are not.【3】

3 What Are the Symptoms of Ramsay Hunt Syndrome?

The symptoms of Ramsay Hunt syndrome vary from case to case. 【4】 But there are two main signs and symptoms:

  • A painful red rash with fluid-filled blisters on, in, and around one ear.
  • Facial weakness or paralysis on the same side as the affected ear.

Usually, the rash and the facial paralysis occur simultaneously. But sometimes, only one side of the face is affected (unilateral). In some cases, there will be some other symptoms:【5】

  • Hearing loss on one side
  • Ringing in your ears (tinnitus)
  • Difficulty closing one eye
  • Dry mouth and eyes
  • Dizziness/vertigo
  • Loss of taste, or a considerable change in how taste is experienced
  • Difficulty eating and drinking

4 How Is Ramsay Hunt Syndrome Diagnosed?

Diagnosis of the syndrome is most often made by observing the symptoms described above. But usually, The diagnosis of Ramsay Hunt syndrome can be difficult because the specific symptoms of the disorder (otalgia, facial paralysis, and the distinctive rash) do not always develop at the same time and symptoms of RHS are similar to other causes of facial weakness. So, there will be some tests:【6】

  • Blood tests for varicella-zoster virus
  • Electromyography (EMG)
  • Hearing tests.
  • MRI of the head. To identify areas of inflammation along the track of the facial nerve and exclude any other cause of your symptoms.
  • Polymerase chain reaction test (PCR). 
  • Lumbar puncture (in rare cases)
  • Skin tests for varicella-zoster virus
  • Nerve conduction (to determine the amount of damage to the facial nerve)

5 Is Ramsay Hunt Syndrome Contagious?

The syndrome is not contagious. But the virus in the RHS blisters is the same virus that causes chickenpox. This virus will cause chickenpox in those who have never had chickenpox or unvaccinated.

If you with Ramsay Hunt syndrome, you should avoid contact with newborns, pregnant women, immunodepression individuals, and people with no history of chickenpox, at least until all the blisters chance to scab.

6 Is There any Difference between Ramsay Hunt Syndrome and Bell’s Palsy?

Bell’s palsy is the most common cause of sudden onset, non-traumatic facial palsy. It is a nonprogressive neurological disorder of the facial nerve. The symptoms may:【7】

  • Slight fever
  • Sudden onset of facial paralysis
  • Pain behind the ear on the affected side
  • Stiff neck

Same as Ramsay Hunt Syndrome, the exact cause of Bell’s palsy is not clear. There is no red rash associated with Bell’s palsy as well. However, Ramsay Hunt syndrome is commonly more painful, and symptoms in RHS tend to be more severe than Bell’s palsy.

7 What are Treatments for Ramsay Hunt Syndrome?

Treatment for RHS includes many aspects:【8】

  • Corticosteroids
  • Antiviral medicines, such as acyclovir or valacyclovir.
  • Anti-inflammatory medicines, such as prednisone.
  • Protection of eyes
  • Painkillers
  • Physical therapy
  • Vertigo suppressants

Patients may take from a few weeks to several months to recover from Ramsay Hunt syndrome. A full recovery is impossible if patients can not get any treatment, and nerve damage is severe.

8 What are Possible Complications?

Complications are rare if treatment starts within a few days. If there is no timely treatment, you are more likely to get complications.

Complications of Ramsay Hunt syndrome may include:

  • Disfigurement
  • Change in taste
  • Damage to the eye (corneal ulcers and infections), resulting in a loss of vision.
  • Persistent pain (postherpetic neuralgia)
  • Spasm of the face muscles or eyelids

The virus may spread to other nerves, such as the brain and spinal cord. That will cause:

  • Confusion
  • Lethargy
  • Back pain
  • Weakness in the arms and legs
  • Headaches

In such cases, the patient may require a spinal tap to determine which areas of the nervous system have become infected.

9 What Sort of Recovery can be Expected?

Infections usually clear up within a couple of weeks. The facial paralysis and hearing loss linked to RHS tend to be temporary. But it’s possible for these problems to become permanent.

Successful recovery from Ramsay Hunt syndrome is dependent on recognizing and treating symptoms in the first few days. If patients can get antiviral treatment within 72 hours of developing symptoms, approximately 70% of people will experience a virtually full recovery. If there is no treatment for more than 3 days, there is less of a chance of complete recovery.【9】

The more severe the damage, the longer it will take to recover, and the lower the chance that you will completely regain normal function.

10 How can I Prevent Ramsay Hunt Syndrome?

There is no known way to prevent Ramsay Hunt syndrome, but treating it with medicine soon after symptoms develop can improve recovery.【10】

Fortunately, there is a vaccine that is helpful in preventing viral reactivation. If you have the vaccination, there will be lighter symptoms. Usually, individuals over 60 who had chickenpox in childhood need this vaccine. The CDC (Centers for Disease Control and Prevention) suggests the vaccine be routinely given to individuals aged 60 or older, as about 90% of the population has been exposed to chickenpox, and about 20% of people had chickenpox are likely to get shingles without the vaccine.【11】