Ramsay Hunt Syndrome

Ramsay Hunt Syndrome is a rare complication of varicella-zoster virus infection. It presents as an ear and mouth rash, pain in the ears, and facial paralysis.

Early diagnosis and treatment appear to improve the long-term outlook for those suffering from Ramsay Hunt Syndrome. About 70% of patients recover completely or nearly completely, though some may experience permanent facial paralysis or hearing loss.

Symptoms

Ramsay Hunt Syndrome is a rare neurological disorder caused by the varicella zoster virus, which causes chickenpox and shingles, reactivating in the brain and spinal cord. Generally affecting adults over 60 years of age, but can also affect children and young adults.

Ramsay Hunt Syndrome is usually identified by a painful rash around one ear or on the face and mouth. This rash often contains fluid-filled blisters known as vesicles and typically appears on one side of the body but may cover both sides simultaneously.

If you have Ramsay Hunt Syndrome, it is imperative to see your doctor as soon as possible. Doing so may increase the chance of recovery and help avoid serious complications like permanent facial paralysis or hearing loss.

Your doctor can perform tests to determine if varicella zoster virus is causing your symptoms. These could include a blood test, skin biopsy or an MRI of the head and neck.

A test called direct immunofluorescent assay analysis can help confirm if the vesicles on your skin are caused by varicella zoster virus. A second, more accurate method called PCR may also be utilized to confirm the diagnosis.

Ramsay Hunt syndrome may present with eye damage, tinnitus (ringing in the ears), and hearing loss due to nerve damage to eye, nose, and mouth muscles due to rash and facial weakness.

You may feel pain or tingling in your face, as well as a sense of pressure. Additionally, you could develop a rash on the tongue and have difficulty closing or opening your mouth.

Your doctor may prescribe antiviral medicine or corticosteroids depending on the severity of your symptoms. Medication such as acyclovir (Zovirax), famciclovir (Famvir) and valacyclovir (Valtrex) can help alleviate chickenpox and shingles symptoms. You may also take corticosteroids if there is excessive swelling or pain from the rash.

Ramsay Hunt syndrome can usually be managed successfully if caught early. In fact, the sooner you begin antiviral treatment for this condition, the higher your chances are of recovery.

Diagnosis

Ramsay Hunt syndrome can be difficult to identify due to the fact that it doesn't always present at once and may be mistaken for other conditions or diseases like poison ivy rash or shingles. Your healthcare provider may use a sample of fluid from one of the blisters as confirmation for diagnosis.

Your doctor may take a sample of skin on your affected ear, an ear drum or canal to look for varicella-zoster virus infection. If the sample shows signs of infection, it's likely you have Ramsay Hunt Syndrome.

Ramsay Hunt Syndrome is typically characterized by a painful rash on both the outside and inside of your ear, filled with fluid that looks like "chickenpox".

Ramsay Hunt Syndrome may present with facial weakness or stiffness depending on which branch of your facial nerve is affected. This could make it difficult to close your eye, wrinkle your forehead or make facial expressions; additionally, these abnormalities may cause social anxiety due to social inadequacies.

Your doctor may suggest taking a test to check for varicella-zoster vaccine or another condition causing your symptoms. The test could be as straightforward as taking blood or saliva samples, or it could involve more complex procedures like an imaging scan.

In some instances, the shingles virus can spread to other parts of the body and lead to meningitis or encephalitis. If not treated promptly, these infections have the potential to be fatal.

Vaccinating against the chicken pox virus (VZV) is highly recommended, as it helps reduce the risk of getting shingles, Ramsay Hunt syndrome, and other serious illnesses that could be caused by VZV.

Ramsay Hunt Syndrome is treated with antiviral medication such as acyclovir or valacyclovir. Some patients may also receive a steroid to relieve the pain caused by shingles infection. In most cases, symptoms will clear up within a few weeks but full recovery may take months or years.

Treatment

Ramsay Hunt syndrome, caused by the shingles-Zoster virus (VZV), can be treated with antiviral medications to increase your chances of full recovery and protect you against complications like hearing loss or facial paralysis that may arise after VZV infection.

Diagnose varicella-zoster is done through examination of symptoms and physical examination. Your provider may collect a sample of saliva, blood or fluid from a blister to check for the virus; then they may order an MRI scan to confirm the diagnosis.

Functional Anatomy of the Facial Nerve: Special visceral efferent motor fibres exit from the brain stem through the internal acoustic meatus and pass through stylomastoid foramen to supply facial muscles. In Ramsay Hunt Syndrome, these fibers are disrupted as they pass through geniculate ganglion.

In some cases, facial paralysis may be accompanied by a rash on the affected area (including the ears and tongue). This rash is commonly described as maculopapular or vesicular in origin and typically begins on or around the ear. It can be difficult to differentiate Ramsay Hunt syndrome from Bell's palsy (facial paralysis without a rash) [6].

Even without a rash, patients with this rare condition may develop otalgia (ear pain). This disorder affects both immunocompetent and immunocompromised individuals alike.

Ramsay Hunt Syndrome is usually curable, though some individuals experience permanent facial paralysis and/or hearing loss after infection with the shingles-Zoster virus. Treatment options include taking an antiviral medication or having surgery to remove scar tissue that's pressing on a facial nerve.

At UT Southwestern, our esteemed facial paralysis team brings together experts in medicine, plastic surgery and other fields to assess your condition and provide personalized treatment plans. Our otolaryngologists / neurosurgeons, an ear, nose and throat specialist, plastic surgeon who specializes in skull base repair as well as psychotherapists or counselors who can help you cope with facial paralysis.

Most often, full recovery from symptoms takes place within weeks to months of the onset. If symptoms persist longer than this, antiviral therapy or surgery may be recommended. Steroid treatments or steroid patches can be helpful in controlling inflammation and relieving facial weakness; botulinum toxin injections are another option for treating inability to close one eye or excessive blinking.

Prevention

Ramsay Hunt Syndrome is an infection caused by the varicella zoster virus (VZV) that caused chickenpox, occurring years after its original exposure. Even people without previous exposure or those who are immunocompromised can become infected.

Vaccinating against chickenpox can reduce your risk of developing shingles and Ramsay Hunt syndrome. For best results, receive two doses of varicella vaccine 4 to 8 weeks apart, plus add on a shingles vaccine if you are 50 or older.

If you experience a shingles outbreak, make an appointment with your doctor right away to get treated with medication and avoid potential complications like permanent facial muscle weakness or hearing loss. Early treatment is key in avoiding long-term effects like these.

A shingles vaccine can also lower your risk of developing postherpetic neuralgia, a persistent pain that develops months to years after healing from shingles. This discomfort may be worse in the morning and persist for several hours after stopping taking pain medications.

Before deciding if you should get a shingles vaccine, consult your doctor about the different kinds of vaccines and when is best to receive them.

Some factors that may increase your chance of developing shingles or Ramsay Hunt Syndrome include having a family history of the disorder, being immunocompromised, and taking certain drugs such as steroids. If you are pregnant, getting a shingles vaccine may be beneficial in protecting your unborn child from harm.

Ramsay Hunt Syndrome can present as a rash of fluid-filled blisters on the face or around your ears, but may actually be another skin condition altogether. If this describes your experience, contact us for further evaluation and advice on managing this condition.

Rarely, one-sided facial paralysis and hearing loss in the affected ear may develop. This condition is usually painful and mistaken for Bell's palsy.

Ramsay Hunt Syndrome can be difficult to identify. If you experience sudden facial paralysis and a rash in or around your ear, seek medical assistance immediately.

Your doctor will likely conduct a physical examination and diagnostic test, such as an imaging scan. You may also receive blood or tear sample to confirm the diagnosis.