Ramsay Hunt syndrome is a rare neurological disorder characterized by facial nerve paralysis (facial palsy) and a rash that affects the ear or mouth. It is caused by the varicella-zoster virus, the same virus that causes chickenpox and shingles (herpes zoster).
Once a person has had chickenpox, the varicella-zoster virus remains dormant in the body. The virus can be reactivated and spread to affect the facial nerve.
Treatment for Ramsay Hunt syndrome includes:
- Antiviral medications
- Other medications
- Pain medication
- Carbamazepine, an anti-seizure medicine which may help reduce nerve pain
- Vertigo suppressants
- Prevention of corneal injury
- The inability to properly close the eye can expose the cornea to abnormal drying and foreign body irritation
- Eye patch to protect the eye
- Artificial tears and lubricating ointments are used to protect the cornea
If the nerve is not too damaged, complete recovery usually happens within a few weeks. For those who have more severe nerve damage, full recovery may not occur. Chances of recovery are improved if treatment begins within three days after symptoms start.
Is Ramsay Hunt Syndrome Serious?
Ramsay Hunt syndrome can be serious. Complications of Ramsay Hunt syndrome may include:
- Facial disfigurement caused by loss of movement
- Changes in taste
- Eye damage (corneal ulcers and infections) that may result in vision loss
- Persistent pain (postherpetic neuralgia)
- Face muscle or eyelid spasm
- Nerves growing back to the wrong structures causing abnormal reactions to a movement, for example, smiling causes the eye to close
- The virus can spread to other nerves or to the brain and spinal cord which can cause:
- Nerve pain
- Limb weakness
What Are Symptoms of Ramsay Hunt Syndrome?
Symptoms of Ramsay Hunt syndrome can vary. The characteristic symptoms of Ramsay Hunt syndrome include:
- Paralysis (palsy) of the facial nerve
- Muscle weakness
- Muscle stiffness
- Inability to smile, wrinkle the forehead, or close the eye on the affected side
- Speech may become slurred
- A rash affecting the ear
- Rash is reddish, painful, blistering
- Affects the outer portion of the ear and often the external ear canal
- Sometimes the rash and blisters may affect the mouth, soft palate, and top portion of the throat
These symptoms do not always occur at the same time and most of the time, only one side of the face is affected (unilateral).
Additional symptoms of Ramsay Hunt syndrome may include:
- Ringing in the ear (tinnitus)
- Ear pain (may be intense) that may spread to affect the neck
- Sounds may appear louder than normal (hyperacusis), causing discomfort
- Sensorineural hearing loss, which is usually temporary but in rare cases it may become permanent
- Spinning sensation (vertigo)
- Loss of taste
- Dry mouth
- Dry eyes
What Are the Types of Ramsay Hunt Syndrome?
There are three types of Ramsay Hunt syndrome. These syndromes are unrelated, other than they were all described by and named for neurologist Dr. James Ramsay Hunt.
- Ramsay Hunt syndrome type 1
- Ramsay Hunt syndrome type 2
- The type referred to in this article
- The reactivation of herpes zoster affects the facial nerve and can cause facial paralysis, ear pain, and rash near the ear
- Ramsay Hunt syndrome type 3
- Also called Hunt's disease or artisan's palsy
- Less common
- Occupationally induced nerve pain of the deep palmar branch of the ulnar nerve in the arm
What Causes Ramsay Hunt Syndrome?
Ramsay Hunt syndrome is caused by the varicella-zoster virus, the same virus that causes chickenpox and shingles. Once a person has had chickenpox, the varicella-zoster virus remains dormant in the body.
- The virus can be reactivated to result in shingles and, in some cases, it develops into Ramsay Hunt syndrome. It is not known why the virus reactivates and affects the facial nerve in Ramsay Hunt syndrome.
- Anyone who has had chickenpox could potentially develop Ramsay Hunt syndrome but most cases occur in adults over age 60 years.
How Is Ramsay Hunt Syndrome Diagnosed?
Ramsay Hunt syndrome is diagnosed with a patient history, physical examination, and identification of the characteristic symptoms (i.e., facial palsy and rash).
A diagnosis of Ramsay Hunt syndrome can be difficult because the specific symptoms of the disorder (facial paralysis and distinctive rash) do not always occur at the same time.
Tests may include:
- Blood tests for varicella-zoster virus
- Nerve conduction study to determine the amount of damage to the facial nerve
- Electromyography (EMG)
- Magnetic resonance imaging (MRI) scan of the head
- Skin tests for varicella-zoster virus
- Lumbar puncture (only performed in rare cases)
Health Solutions From Our Sponsors
Brain and Nervous System Resources
Health Solutions From Our Sponsors