Ramsay Hunt Syndrome

Ramsay Hunt syndrome may be triggered by conditions that weaken the immune system, such as COVID-19 or the shingles virus.

• Ramsay Hunt syndrome (also termed Hunt's Syndrome and herpes zoster oticus) is a herpes zoster virus infection of the geniculate ganglion of the facial nerve.
• It is caused by the reactivation of the herpes zoster virus that has previously caused chickenpox in the patient.
• Ramsay Hunt syndrome results in paralysis of the facial muscles on the same side of the face as the infection. So, the virus infects the facial nerve that normally controls the muscles on one side of the face.
• Ramsay Hunt syndrome is typically associated with a red rash and blisters (inflamed vesicles or tiny water-filled sacks in the skin) in or around the ear and eardrum and sometimes on the roof of the mouth or tongue.

The classic symptom that clinically distinguishes Ramsay Hunt syndrome is a red painful rash associated with blisters in the ears and facial paralysis (for example, eyelid or mouth) on one side of the face. Other symptoms are: 

• ear pain,
• hearing loss,
• headaches,
• speech difficulty,
• fever,
• dizziness (or vertigo),
• difficulty eating and drinking,
• dry eye, and
• changes in taste sensation may also occur.

Diagnosis of the syndrome is most often made by observing the symptoms described above (red painful rash with ear and or mouth blisters and one-sided facial paralysis). Also, a PCR test (polymerase chain reaction) can be performed on the fluid from the blisters to demonstrate the viral genetic material, but this test is not done routinely.

The syndrome is not contagious; however, the herpes zoster virus that can be found in the blisters of Ramsay Hunt syndrome can be transmitted to other people and cause chickenpox in those who are unvaccinated against chickenpox and who have never had chickenpox. Individuals with Ramsay Hunt syndrome should avoid contact with newborns, pregnant women, immunodepression individuals, and people with no history of chickenpox, at least until all the blisters chance to scabs.

Bell's palsy also is a result of injury to the facial nerve by a viral infection, but the suspected viral cause of Bell's palsy has not been identified. Ramsay Hunt syndrome is caused by the Varicella virus (Herpes zoster) that also causes chickenpox and shingles (a painful, blister-producing Herpes zoster reinfection that usually occurs on one side of the body). There is no red rash associated with Bell's palsy as there is with Ramsay Hunt syndrome. Additionally, Ramsay Hunt syndrome is commonly more painful than Bell's palsy. However, both can cause eyelid and mouth paralysis on one side of the face.

Dyssynergia cerebellaris myoclona is a rare degenerative disease of the nerves characterized by epilepsy, muscle spasms, and gradually increasing tremors. Like Bell's palsy, this disease complex mimics many symptoms of Ramsay Hunt syndrome. Some investigators term the disease complex Ramsay Hunt syndrome type 2.

Treatment consists of antiviral agents (for example, acyclovir, valacyclovir, or famciclovir) for about one week, steroids (prednisone), and pain medications. Best results are reported when treatment protocols are started within three days after symptoms appear.

Early treatment usually results in a better prognosis (see below). For children, the varicella vaccine can reduce the chance of getting chickenpox from which the syndrome comes (reactivation of the virus). However, once a person gets chickenpox, the person is susceptible to reactivation of the virus and thus can develop shingles and/or Ramsay Hunt syndrome.

Fortunately, there is another vaccine, Zostavax, which is helpful in preventing viral reactivation. Consequently, shingles and Ramsay Hunt syndrome can be either prevented or their symptoms reduced if the vaccine is administered. Usually, this vaccine is given to individuals that have had chickenpox as children and are now age 60 or older. The CDC (U.S. Centers for Disease Control and Prevention) suggests the vaccine be routinely given to individuals aged 60 or older, as about 90% of the population has been exposed to chickenpox, and about 20% of people had chickenpox are likely to get shingles without the vaccine.

• The prognosis for Ramsay Hunt syndrome is not as good as that for Bell's palsy.
• People with Ramsay Hunt syndrome may not recover completely, especially if treatment is not initiated within three days (72 hours) of symptom onset.
• Good clinical evidence suggests that treatment with steroids, pain medications, and antiviral agents (like acyclovir, valacyclovir, or famciclovir) improves recovery and lessens extreme facial discomfort. However, infrequent complications can develop, such as synkinesis (inappropriate nerve responses such as blinking or tear formation while trying to talk), eye damage, or rarely, viral spread to other nerves causing many other problems (for example, pain, confusion, weakness).
• Postherpetic neuralgia (pain due to nerve fiber damage by the virus) may also develop and persist for months to years.