What Is Ramsay Hunt Syndrome? What Causes Ramsay Hunt Syndrome?

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Main Category: Neurology / Neuroscience
Also Included In: Infectious Diseases / Bacteria / Viruses
Article Date: 12 Jun 2010 - 0:00 PST

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Ramsay Hunt syndrome, or Herpes Zoster Oticus is a neurological disorder in which the Varicella-zoster virus infects specific nerves in the head - it is an infection of the facial nerve. The Varicella-zoster virus can also cause chicken pox. Patients who have had chicken pox carry the dormant virus in their nerves. Some years later it may become active again, infecting the facial nerve, causing Ramsay Hunt syndrome.

The patient typically has a painful rash and facial muscle weakness. Some people may become alarmed during the onset of symptoms, and wonder whether they have had a stroke.

Ramsay Hunt syndrome is usually effectively treated. In some cases, however, patients may have permanent facial muscle weakness and hearing loss. The risk of complications is significantly reduced if the individual receives prompt and proper treatment. The longer the person has to wait for treatment, the smaller are his/her chances of making a complete recovery.

According to Medilexicon's medical dictionary, Herpes Zoster Oticus is:

"a painful varicella virus infection presenting with a vesicular eruption on the pinna, with or without facial nerve paralysis."

The syndrome was first described in detail by James Ramsay Hunt, an American from Philadelphia in 1907.

The Office of Rare Disease of th National Institutes of Health, USA, classifies Ramsay Hunt Syndrome as a rare disease (fewer than 200,000 cases in the USA). The majority of doctors will never come across a case of Ramsay Hunt syndrome during their careers.

What are the signs and symptoms of Ramsay Hunt syndrome?

A symptom is something the patient senses and describes, while a sign is something other people, such as the doctor notice. For example, drowsiness may be a symptom while dilated pupils may be a sign.

Signs and symptoms of Ramsay Hunt syndrome include:

• Loss of taste, or a considerable alteration in how taste is perceived
• A red rash, often painful with blisters on the tongue, palate, inner/outer ear, and eardrum
• Vertigo
• One of the eyes may be harder to close
• Continuous earache
• Palsy (facial weakness) on the same side as the affected ear
• Deafness in the affected ear
• Tinnitus
• Facial expressions may be altered, such as a crooked smile/grin
• The face drops

What are the risk factors for Ramsay Hunt syndrome?

A risk factor is something which increases the likelihood of developing a condition or disease. For example, obesity significantly raises the risk of developing diabetes type 2. Therefore, obesity is a risk factor for diabetes type 2.

Individuals over the age of 60 who have had chicken pox have a higher risk of developing this syndrome than other age groups. Ramsay Hunt syndrome has been known to affect children, but extremely rarely.

Ramsay Hunt syndrome is not in itself contagious, but an affected person can be the source of chicken pox for those who have never had it (chicken pox).

People with Ramsay Hunt syndrome should wait until their blisters scab over before being in contact with individuals with weakened immune systems, those who have never had chicken pox, infants and pregnant mothers.

What are the complications of Ramsay Hunt syndrome?

Complications are rare if treatment starts within a few days of the onset of symptoms. Unfortunately, a small percentage of patients may experience permanent hearing loss and facial weakness even if their treatment was prompt and proper.

If treatment is delayed there is a risk of permanent loss of facial movement, as well as uncontrolled movement, such as blinking. Some people may find it hard to close one eye, which can eventually damage the cornea, leading to eye pain and vision problems. Some patients may experience pain long after other symptoms have gone (postherpetic neuralgia).

The virus may spread to other nerves, such as the brain and spinal cord, causing confusion, lethargy, back pain, weakness in the arms and legs, and headaches. In such cases the patient may require a spinal tap to determine which areas of the nervous system have become infected.

How is Ramsay Hunt syndrome diagnosed?

A doctor will probably make a diagnosis if the hallmark signs of facial weakness and a blister-like rash are detected. A nerve conduction study may be performed to determine how severe facial nerve damage is, as well as determining prognosis (chances of a recovery).

Ramsay Hunt syndrome is commonly diagnosed because so many doctors have never come across it.

A sample of the fluid in one of the blisters in the ear may be taken to confirm the diagnosis (Polymerase chain reaction, or PCR). A tear or blood sample may also be used.

A neuroimaging scan, such as an MRI (magnetic resonance imaging) may reveal swelling of the facial nerves. Scans can help the doctor determine whether the virus has reached the brain or other nerves.

What are the treatment options of Ramsay Hunt syndrome?

The earlier a patient is treated, the less severe his/her symptoms will be, and the better are his/her chances of making a complete recovery. The doctor will probably prescribe an antiviral medication, such as acyclovir (Zovirax), famciclovir (Famvir) or valacyclovir (Valtrex). For the swelling and pain a corticosteroid, such as prednisone may also be prescribed. The patient may have to take Diazepam (Valium) for the symptoms of vertigo.

If facial weakness persists after medical treatment has been completed, the physician may recommend physical therapy (physiotherapy) to help regain proper use of the facial muscles.

An injection of botulinum toxin type A (Botox) into the upper eyelid may help those who have trouble closing one eye. An eye patch may help protect the affected eye.

Patients may take from a few weeks to several months to recover from Ramsay Hunt syndrome. If treatment is delayed and nerve damage is severe, a full recovery is much less likely.

Written by Christian Nordqvist


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