Ramsay Hunt syndrome

Alternative names

Hunt's syndrome; Herpes oticus

Definition

Ramsay Hunt syndrome is a neurologic disorder caused by a virus called varicella zoster, which can infect certain nerves of the head to produce facial paralysis and rash either in the ear, tongue, or palate.

Causes, incidence, and risk factors

The varicella zoster virus causes both Ramsay Hunt syndrome and another common cause of facial weakness, Bell's palsy.

This virus is a relative of human herpes viruses, which cause fever sores on the mouth and genital herpes. The varicella zoster virus also causes 2 other diseases - shingles (a nerve infection affecting the so-called "dorsal roots," the nerves of the sides of the trunk) and chickenpox (a disease characterized by an itchy rash, commonly affecting children).

The virus is believed to infect the facial nerve near the inner ear in this condition, which results in local inflammation (irritation and swelling). The extent of the symptoms may reflect the severity of the nerve inflammation.

Symptoms

Symptoms may include:

• Facial weakness with difficulty closing one eye - smile may look crooked
  
• Painful rash on the ear drum, tongue, or palate on the same side of the facial weakness
  
• Hearing loss on one side
  
• Sensation of things spinning around (vertigo)
  

Signs and tests

Diagnosis is generally determined by observation of evidence of facial weakness and a vesicular rash. Occasionally, a nerve conduction study will be done to determine the extent of damage to the facial nerve and potential for recovery.

The more severe the damage, the longer it will take to recover, and the lower the chance of complete return to normal function. Occasionally, blood tests are used to determine whether or not an infection with the varicella zoster virus has occurred.

There is a promising laboratory technique called PCR, which can detect very small amounts of viral DNA in the affected skin. However, it is mostly used for research nowadays.

The use of neuroimaging (pictures of the brain), particularly MRI (magnetic resonance imaging), may sometimes show inflammation of the facial nerves and determine whether the infection has spread to other nerves or the brain. A spinal tap is used in rare cases, especially when the diagnosis is not clear.

Treatment