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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. Patients should avoid contact with these individuals as well as people with a weakened immune system until there is scabbing over the blistered rash. 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. 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. 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 certain people, pain may spread to affect the neck.Īdditional symptoms that may be present include nausea, vomiting and a sensation that one’s surroundings are spinning (vertigo). 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. Rarely, hearing loss may become permanent. Hearing loss is usually temporary and occurs in as many as 50% of patients. 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). In some patients, ear pain may be intense. Additional symptoms affecting the ear include ringing in the ear (tinnitus) and ear pain (otalgia). 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. Vesicular rashes of the ear and mouth have been reported in as many as 80% of patients. 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. In rare cases, the clear, front part of the eye (cornea) can become damaged and blur vision. 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.
After the onset of symptoms, facial weakness is usually most severe within one week. In most people, only one side of the face is affected.įacial 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”. These two symptoms do not always occur at the same time. Affected individuals usually experience paralysis of the facial nerve and a rash affecting the ear. The symptoms of Ramsay Hunt syndrome vary from person to person. However, some patients may have permanent facial paralysis or hearing loss. There is usually a good prognosis when treatment is started within three days of the onset of symptoms. Treatment for Ramsay Hunt syndrome includes anti-inflammatory drugs (steroids) to reduce pain and swelling of the nerves. In Ramsay Hunt syndrome, previously inactive (dormant) varicella-zoster virus is reactivated and spreads to affect the facial nerve. 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. Ringing in the ears (tinnitus) and hearing loss may also be present. Symptoms are usually on one side of the face (unilateral). The disorder is characterized by facial weakness or paralysis of the facial nerve (facial palsy) and a rash affecting the ear or mouth. Ramsay Hunt syndrome is a rare neurological disorder that typically affects adults over 60 years of age. Stay Informed With NORD’s Email Newsletter.Find a Rare Disease Patient Organization.Find Clinical Trials & Research Studies.Launching Registries & Natural History Studies.A Podcast For The Rare Disease Community.
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