Ramsay Hunt Syndrome

1 What is Ramsay Hunt Syndrome?

Ramsay Hunt syndrome is defined as an acute peripheral facial neuropathy associated with erythematous vesicular rash of the skin of the ear canal, auricle (also termed herpes zoster oticus), and/or mucous membrane of the oropharynx.

This syndrome is also known as geniculate neuralgia or nervus intermedius neuralgia. Ramsay Hunt syndrome can also occur in the absence of a skin rash, condition known as zoster sine herpete.

Ramsay Hunt syndrome was first described in 1907 by James Ramsay Hunt in a patient who had otalgia associated with cutaneous and mucosal rashes, which he ascribed to infection of the geniculate ganglion by human herpesvirus 3 (ie,varicella-zoster virus [VZV]).

The following may be observed:

  • VZV auricularis,
  • VZV in any of the zoster zones of the head and neck (herpes auricularis, herpes facialis, and herpes occipito-collairs) with facial palsy,
  • VZV in any of the zoster zones with facial palsy and auditory symptoms (eg, tinnitus, deafness, vertigo, nystagmus, ataxia).

2 Symptoms

Signs and symptoms of Ramsay Hunt syndrome include:

  • Painful rash on the eardrum, ear canal, earlobe, tongue, and roof of the mouth on the side where there is weakness of the face
  • Hearing loss on one side
  • Sensation of things spinning (vertigo)
  • Weakness on one side of the face that causes difficulty closing one eye, eating (food falls out of the weak corner of the mouth), making expressions, and making fine movements of the face, as well as facial droop and paralysis on one side of the face.

3 Causes

Infection by herpes zoster of facial nerve near ear is believed to be the main cause.

This leads to irritation and swelling of facial nerve and hence the symptoms.

At times our immune system becomes depressed and is less able to fight off infection. The body then becomes vulnerable to reactivation of the chickenpox virus.

Stress is often a trigger. 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. For this reason, it is believed that stress can be linked to outbreaks of shingles, and thus RHS could result.

4 Making a Diagnosis

A diagnosis of Ramsay Hunt syndrome is often difficult because symptoms vary from person to person and symptoms of RHS are similar to other causes of facial weakness.

The following examinations and investigations may be used in the diagnosis of RHS:

  • Thorough history taking by your GP or doctor.
  • Examination of the facial nerve functions (such as eye closure).
  • A full physical examination including the ear.
  • Blood tests to measure the presence of the antibodies to the varicella zoster virus responsible for causing RHS.
  • Hearing tests.
  • Magnetic Resonance Imaging (MRI) to identify areas of inflammation along the track of the facial nerve and exclude any other cause of your symptoms.
  • Nerve conduction studies can assess facial nerve function. It is not diagnostic but indicates the degree of facial nerve damage.
  • Electromyography can also show if the facial nerve is damaged but again this cannot give a specific diagnosis or reason for the damage.

5 Treatment

Corticosteroids and oral acyclovir are commonly used in the treatment of Ramsay Hunt syndrome.

In one review, combined therapy using corticosteroids plus intravenous acyclovir did not show benefit over corticosteroids alone in promoting facial nerve recovery after 6 months.

However, randomized clinical trials evaluating both therapies are required.

Another study concluded that controlled-release oxycodone was safe and generally well tolerated in patients experiencing acute pain due to herpes zoster.

Vestibular suppressants may be helpful if vestibular symptoms are severe.

As with Bell palsy, care must be taken to prevent corneal irritation and injury.

Temporary relief of otalgia may be achieved by applying a local anesthetic or cocaine to the trigger point, if in the external auditory canal.

Carbamazepine may be helpful, especially in cases of idiopathic geniculate neuralgia.

6 Prevention

Vaccination against chickenpox helps to prevent Ramsay Hunt syndrome from occurring.

Also building up proper immunity is thought to prevent the disease from occurring.

7 Lifestyle and Coping

Lifestyle modifications are necessary in order to cope with Ramsay Hunt syndrome.

The following can help reduce the discomfort of Ramsay Hunt syndrome:

  • Keep areas affected by the rash clean.
  • Apply cool, wet compresses to the rash to ease pain.
  • Take an over-the-counter pain reliever or anti-inflammatory drug, such as ibuprofen (Advil, Motrin IB, others).

If facial weakness makes it difficult for you to close one of your eyes, take the following steps to protect your vision:

  • Use moisturizing eyedrops throughout the day if your eye becomes dry.
  • At night, apply ointment to the eye and tape your eyelid shut or wear an eye patch.

8 Risks and Complications

There are several complications associated with Ramsay Hunt syndrome, which include:

  • Changes to the appearance of the face (disfigurement) from loss of movement
  • Changes to 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

Occasionally, the virus may spread to other nerves, or even to the brain and spinal cord. This can cause:

If these symptoms occur, a hospital stay may be needed. A spinal tap may help determine whether other areas of the nervous system have been infected.

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