Ramsay Hunt syndrome is an infection of a facial nerve. It is a neurological disorder in which the Varicella zoster virus infects specific nerves in the head. The condition is also known as Herpes zoster oticus.
The Varicella zoster virus can also cause chicken pox. 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, causing Ramsay Hunt syndrome.
The condition involves a painful rash and facial muscle weakness, which is sometimes confused with a stroke.
Ramsay Hunt syndrome is usually effectively treated, but some people may have permanent facial muscle weakness and hearing loss.
The risk of complications drops significantly with prompt and proper treatment. The longer the person has to wait for treatment, the smaller the chances of making a complete recovery.
The Office of Rare Diseases classifies Ramsay Hunt Syndrome as a rare disease, with an incidence of around
Early treatment improves the chance of a complete recovery.
The doctor is likely to prescribe an antiviral medication, such as acyclovir (Zovirax), famciclovir (Famvir), or valacyclovir (Valtrex), as well as a corticosteroid for the swelling and pain. The patient may have to take diazepam (Valium) for the symptoms of vertigo.
If facial weakness persists after the completion of medical treatment, the physician may recommend physical therapy to help regain proper use of the facial muscles.
An injection into the upper eyelid of botulinum toxin type A, more commonly known as Botox, 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.
Successful recovery from Ramsay Hunt syndrome is dependent on recognizing and treating symptoms in the first few days.
The best way to ensure the fullest possible recovery is to take the drugs acyclovir and prednisolone within 7 days of symptoms starting.
The damage caused by Ramsay Hunt syndrome is reversible.
Signs and symptoms of Ramsay Hunt syndrome include:
- loss of taste, or a considerable change in how taste is experienced
- an often painful red rash, with blisters on the tongue, palate, inner/outer ear, and eardrum
- one of the eyes may be harder to close
- a continuous earache
- facial weakness on the same side as the affected ear
- deafness in the affected ear
- facial expressions may be altered, such as a crooked smile
- facial drooping
Individuals over the age of 60 years who have already had chickenpox have a higher risk of developing this syndrome than other age groups. Ramsay Hunt syndrome has been known in extremely rare cases to affect children.
Ramsay Hunt syndrome is not contagious, but a person with the condition can pass chickenpox on people who have never before had the disease.
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.
Complications are rare if treatment starts within a few days of the onset of symptoms. A small percentage of patients may, however, experience permanent hearing loss and facial weakness even if their treatment was applied quickly and effectively.
If treatment is delayed, there is a risk of losing facial movement permanently, as well as uncontrolled movements, 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, in a condition known as 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.
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 the extent of damage to the facial nerve, as well as defining the likelihood of recovery.
Ramsay Hunt syndrome is commonly misdiagnosed, as many doctors have never encountered the condition.
A sample of the fluid in one of the blisters in the ear may be taken to confirm the diagnosis. The doctor may also use a tear or blood sample.
An imaging scan, such as an MRI, may reveal swelling of the facial nerves. Scans can also help the doctor determine whether the virus has reached the brain or other nerves.