The Ramsay Hunt Syndrome

 

The Ramsay Hunt Syndrome

  

What is Ramsay Hunt Syndrome?

It is a neurological disorder, a rare one that is characterized by the partial paralysis of the facial nerves (facial palsy). An erythematous vesicular rash of the skin affecting the ear or mouth may also be present.

Also accompanying the disorder could be ringing in the ears (tinnitus) and hearing loss. The disorder is also sometimes known as herpes Zoster oticus due to the rash around the ears that accompanies the disorder.

 

Discovery of Ramsay Hunt Syndrome

The disorder is named after James Ramsay Hunt, a physician, who first described it in 1907, through observations of a patient presenting mucosal and cutaneous rashes.

 

What causes Ramsey Hunt Syndrome?

Ramsay Hunt syndrome is caused by a virus, the same type that causes the chickenpox virus, known as the Varicella-Zoster Virus (VZV). After the chicken pox has cleared up, the virus are still present in the nerves in a dormant state. 

Years later, the virus may be reactivated causing shingles (herpes zoster) and infecting the facial nerves and leading to irritation, swelling, and partial paralysis.

 

Signs and Symptoms of Ramsay Hunt Syndrome

The symptoms can vary from individual to individual, but usually, only one side of the face is affected. The main symptoms of the disorder are -

·    Red painful blisters that may be fluid filled breaking out in and around either ear.

· Paralysis or facial weakness on the side of the face of the affected ear (unilateral paralysis).

·        Facial drooping

·        Sensorineural hearing loss (where sound is not properly transmitted to the brain) in the affected ear

·        Ringing in the ear (tinnitus)

·        Difficulty closing one eye (usually the eye on the affected face side

·        Develops a crooked smile

·        Dryness of the mouth and eyes

·        A sensation that you or your surrounding is in motion  or you are spinning (vertigo)

·        Painful reddish rash on affected side of face

·        Blisters on the tongue and ear canal

·        Dizziness

·        Ear ache

·        Change in taste or lose of

·        Nausea and vomiting

·        Hyperacusis - condition where sounds appear louder than they really are

·        Slurred speach

 

 

How to diagnose Ramsay Hunt Syndrome

There is a possibility of misdiagnoses of this disorder, more so as it not very common, like some ailments that Doctors are familiar with. In fact most doctors will never come across it in the course of their practice.

Ways in which Ramsay Hunt Syndrome can be diagnosed include

·  Taking a sample of the fluid in the blisters that usually forms around the ear on one side of the face

·   A sample of the patient’s  blood can also be taken and analyzed

· A magnetic resonance imaging scan is also used to detect inflammation in the facial nerves, indicative of the syndrome

·  Otoscope ear examination to check foe inflammation in the ear canal

·  All the above coupled with physical examination.

 

Risk factors for Ramsay Hunt Syndrome

· Anyone that had previously had chicken pox infection can become afflicted with the syndrome. Adults, usually above 50 years are at a higher risk that children.

·  Ramsay Hunt syndrome in not contagious, however anyone that has not been previously infected with the chicken pox virus or been vaccinated can contract it from someone with an active Ramsay Hunt Syndrome because the virus has become reactivated.

·   During the blister stage, babies, young children and pregnant women should avoid contact with an infected person. People with a weak or compromised immune system should also stay away.

 

Treatment for Ramsay Hunt Syndrome

Beginning treatment as soon as possible after the onset of the disorder is important, so as to minimize the risk of permanent nerve damage. Medications used include-

· Antiviral drugs – Famciclovir (famvir), Zovirax or Valacyclovir (Valtex), can be used to fight the disorder for between 7 to 10 days.

·  Corticosteroids – Like prednisone help increase the efficacy of the antiviral drugs used, for 3 to 5 days.

· Pain relievers- these are used to alleviate the pain, such as seizures associated with the syndrome. Medication for these include carbamazepine, an anti-seizure medicine.

· Anti-anxiety drugs – antihistamines and anticholinergics can help to suppress vertigo.

·   Because the patient may find it difficult to close their affected eye properly, dryness and irritation (from dust) can cause injury to the cornea. Artificial tears and eye ointment should be used to minimize this risk.

 

Prevention of Ramsey Hunt Syndrome

Since it is a reactivation the chickenpox virus that had remained dormant in the nervous system after the infection and treatment of the virus, probably due to a compromised immune system, vaccination against the chicken pox virus in the first place will help to prevent the Ramsay Hunt syndrome.

 

Prognosis

Treatment just after the onset of symptoms gives a good chance of recovery. If the damage to the nerves is minimal, then a high degree of recovery is expected. More serious damage to the nerves reduces the chance of full recovery even months after treatment.

The younger the patient, the better the chances of making a full recovery with little or no complications. Complications include synkinesis, a situation where the damaged nerves grow and connect back to the wrong areas. This can lead to inappropriate responses to certain actions.

 

Related disorders

Bell’s palsy is a neurological disorder that presents symptoms similar with Ramsay Hunt syndrome. It start with a fever, stiffness on the affected face side, and a stiff neck.

 

Additional information

·   How long does Ramsay Hunt syndrome last – with proper treatment, the disorder lasts between 6 weeks and 3months before recovery.

·  Can Ramsay Hunt syndrome relapse or re-occur? – No, it does not usually re-occur.  

 

Take Away

The Ramsay Hunt Syndrome can cause pain, discomfort and anxiety in affected persons. To greatly reduce the chance of developing the disorder, vaccination against the chickenpox Virus is key. This is so because the chickenpox virus that goes dormant after the treatment of and recovery from the virus getting reactivated is the precursor to the development of the Ramsay Hunt Syndrome.