June 21, 2022
Years published: 2005, 2011, 2022
NORD gratefully acknowledges Camey Calzolano and Kathleen Narloch, NORD Editorial Interns from the University of Notre Dame and Sombat Muengtaweepongsa, MD, Associate Professor, Associate Dean for Research Affairs, Faculty of Medicine, Thammasat University, for assistance in the preparation for this report.
Ramsay Hunt syndrome is a rare neurological disorder that typically affects adults over 60 years of age. The disorder is characterized by facial weakness or paralysis of the facial nerve (facial palsy) and a rash affecting the ear or mouth. Symptoms are usually on one side of the face (unilateral). Ringing in the ears (tinnitus) and hearing loss may also be present. Ramsay Hunt syndrome is caused by the varicella zoster virus (VZV), the same virus that causes chickenpox in children and shingles (herpes zoster) in adults. In Ramsay Hunt syndrome, previously inactive (dormant) varicella-zoster virus is reactivated and spreads to affect the facial nerve.
Treatment for Ramsay Hunt syndrome includes anti-inflammatory drugs (steroids) to reduce pain and swelling of the nerves. There is usually a good prognosis when treatment is started within three days of the onset of symptoms. However, some patients may have permanent facial paralysis or hearing loss.
The symptoms of Ramsay Hunt syndrome vary from person to person. Affected individuals usually experience paralysis of the facial nerve and a rash affecting the ear. These two symptoms do not always occur at the same time. In most people, only one side of the face is affected.
Facial muscles affected by nerve palsy may be weak or feel stiff and result in the inability to smile or wrinkle the forehead, creating the appearance of a “saggy face”. After the onset of symptoms, facial weakness is usually most severe within one week. Asymmetric muscle tone may be visible and some people may have a drooping mouth and drool saliva.The inability to close the eye usually occurs and results in irritation. In rare cases, the clear, front part of the eye (cornea) can become damaged and blur vision.
Most patients with Ramsay Hunt syndrome have a reddish (erythematous), painful, fluid-filled blistering (vesicular) rash that affects the outer portion of the ear (pinna) and often the external ear canal. Vesicular rashes of the ear and mouth have been reported in as many as 80% of patients. The rash, including painful blisters, may also affect the eardrum, mouth, soft palate and top portion of the throat, notably on the side with the affected nerve. Additional symptoms affecting the ear include ringing in the ear (tinnitus) and ear pain (otalgia). In some patients, ear pain may be intense. Some affected individuals develop sensorineural hearing loss, a condition in which sound vibrations are not properly transmitted to the brain due to a defect of the inner ear or the auditory nerve, resulting in hearing loss on the symptomatic side of the body (ipsilateral). Hearing loss is usually temporary and occurs in as many as 50% of patients. Rarely, hearing loss may become permanent. Some affected individuals may experience hyperacusis, a condition in which sounds seem louder (often dramatically) than normal due to an abnormality of the stapedius muscle in the eardrum, causing tremendous discomfort. In certain people, pain may spread to affect the neck.
Additional symptoms that may be present include nausea, vomiting and a sensation that one’s surroundings are spinning (vertigo).
Possible complications in rare cases of Ramsay Hunt syndrome include a change in taste perception, loss of vision caused by eye damage from corneal ulcers and infections, abnormal reactions to facial movements caused by nerves growing back to the wrong muscles, persistent pain (postherpetic neuralgia) and facial weakness. In rare cases, the virus may spread to other nerves or to the brain and spinal cord, causing confusion, drowsiness, limb weakness, headaches and nerve pain.
Ramsay Hunt syndrome is not contagious, but reactivation of the varicella-zoster virus can cause chickenpox in people who have not had it or have not been vaccinated. Patients should avoid contact with these individuals as well as people with a weakened immune system until there is scabbing over the blistered rash.
Some individuals with Ramsay Hunt syndrome may have facial palsy with evidence of varicella-zoster virus through testing (e.g., blood tests), but without the associated skin abnormalities. These cases may be referred to as zoster sine herpete.
Ramsay Hunt syndrome is caused by the varicella-zoster virus, which is the same virus that causes chickenpox and shingles. The virus can remain dormant for decades in a person who has had chickenpox as a child. Reactivation of the varicella-zoster virus results in a shingles outbreak and, in cases where the varicella-zoster virus spreads to the facial nerves, develops into Ramsay Hunt syndrome. The reason why the virus reactivates and affects the facial nerve in Ramsay Hunt syndrome is unknown.
According to one estimate, 5 out of every 100,000 people develop Ramsay Hunt syndrome each year in the United States. Ramsay Hunt syndrome affects males and females in equal numbers. Anyone who has previously had chickenpox can potentially develop Ramsay Hunt syndrome. However, most patients are adults over the age of 60. Ramsay Hunt syndrome is extremely rare in children.
Ramsay Hunt syndrome may go undiagnosed or misdiagnosed making it difficult to determine the true frequency of this condition in the general population.
Diagnosis of Ramsay Hunt syndrome can be difficult because the symptoms of the disorder (earache, facial paralysis and the distinctive rash) do not always develop at the same time.
Diagnosis is based on a thorough clinical evaluation, detailed patient history and identification of characteristic symptoms (i.e., one-sided facial palsy and/or a rash around the ear). A sample from the fluid-filled blistering rash surrounding the ear can be used to confirm the diagnosis. This rash is a good indicator that the disease is Ramsay Hunt syndrome and not Bell’s palsy, acoustic neuroma or trigeminal neuralgia.
Viral studies can detect varicella-zoster virus in saliva, tears, and blood but are not necessary to establish a diagnosis of Ramsay Hunt syndrome.
The treatment of Ramsay Hunt syndrome involves antiviral medications like acyclovir or famciclovir, as well as corticosteroids like prednisone. The drugs work together to boost effectiveness in combating the varicella zoster virus. Specifically, anti-inflammatory drugs, or steroids, may help diminish pain by reducing inflammation of the nerves. However, it should be noted that the effectiveness and benefits of antiviral medications have not been confirmed. Despite therapy, some degree of facial paralysis and hearing loss may become permanent in some people.
Further treatment is directed towards specific symptoms that are apparent in each individual. This includes pain medication, carbamazepine, an anti-seizure medicine which may help reduce neuralgic pain and vertigo suppressants like antihistamines and anticholinergics. Anti-anxiety medications such as diazepam (Valium) can also be helpful in treatment of pain and vertigo.
Capsaicin has been approved by the U.S. Food and Drug Administration (FDA) to manage neuropathic pain associated with postherpetic neuralgia.
Beginning treatment within three days of the onset of symptoms is important to have the greatest benefit.
Individuals with Ramsay Hunt syndrome need to take special care to prevent corneal injury because they have difficulty closing one eye. This can expose the cornea to abnormal drying and foreign body irritation. Artificial tears and lubricating ointments may be prescribed to protect the cornea. Some patients may be recommended to wear an eye patch.
Prevention against the varicella zoster virus is available through a chickenpox vaccine in children and a shingles vaccine for people 50 years of age and older. These vaccinations can greatly reduce the chance of becoming infected with the varicella zoster virus, which reduces the chances of contracting Ramsay Hunt syndrome.
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