Hunt’s syndrome; Herpes oticus
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 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 with anti-viral medications, such as acyclovir or famciclovir is recommended for 7 - 10 days, along with strong anti-inflammatory drugs called steroids (such as prednisone) for 3 - 5 days.
The steroids are then tapered off in about 1 week. Sometimes strong pain killers are also needed if the pain persists despite the use of the steroids. During the period of facial weakness, an eye patch should be worn to prevent corneal abrasion and damage to the eye if it does not close completely.
If damage to the nerve is minimal, then a full recovery is usually expected within a few weeks. If damage is more severe, there may not be full recovery - even after several months.
Overall, chances of recovery are better if the treatment is started within 3 days of the onset of the symptoms. Complete recovery is achieved by 70% of patients if treatment is begun at this time.
However, when the treatment is delayed more than 3 days, the chances of complete recovery drop to about 50%. Children are more likely to have a complete recovery than adults.
Recovery may be complicated if the nerve grows back to the wrong areas (synkinesis) which may cause inappropriate responses, such as tears when laughing or chewing (croc’s tears). Some other people may experience blinking of the eye when talking or chewing food.
Severe paralysis will result in incomplete or inappropriate recovery with potential for permanent facial paralysis and synkinesis.
Damage to the cornea due to incomplete eyelid closure can occur, resulting in local eye pain and blurred vision.
Occasionally, the virus may spread to other nerves or even to the brain and spinal cord, causing headaches, back pain, confusion, lethargy, and limb weakness. This may prompt an admission to the hospital, where a spinal tap may help to determine whether other areas of the nervous system have been infected.
Calling your health care provider
Call your health care provider if you have facial paralysis or a rash on your face associated with facial weakness.
There is no known prevention of Ramsay Hunt syndrome, but recovery is improved with the use of medications early in the course of the symptoms. It is important to keep the eye protected if there is incomplete eye closure due to weakness.
by Simon D. Mitin, M.D.