Ramsay Hunt syndrome, also known as herpes zoster oticus, is a rare neurological condition caused by the varicella-zoster virus – the same virus that causes chickenpox and shingles. People who have had chickenpox and recovered from it will still carry particles or the whole inactive virus in their nerves for decades.

Years later, the varicella zoster virus can be reactivated causing shingles in which a painful rash and blisters typically appear on the torso. However, when the reactivation of the varicella zoster virus infects a nerve in the head, the facial nerve, it causes Ramsay Hunt syndrome.

The condition was named after Dr. James Ramsay Hunt, the doctor who first described the disorder in 1907. Ramsay Hunt syndrome is characterized by paralysis of the facial nerve (facial palsy) and a painful shingles-like rash that typically appears in and around the ear on the same side as the paralysis. Other symptoms may include ringing in the ear (tinnitus), loss of balance, slurred speech, and extremely dry eyes due to the inability to blink caused by the facial paralysis.

In the United States, Ramsay Hunt syndrome affects approximately 5 out of every 100,000 people each year and anyone who has had chickenpox can potentially develop it.

Ramsay Hunt syndrome is caused by the varicella-zoster virus – the same virus that causes chickenpox and shingles. The varicella-zoster virus belongs to a group of viruses called herpes viruses. Theherpes virusesthat cause cold sores, fever blisters, and genital herpes also belongs in this family of viruses. This group of viruses can remain dormant in the nervous system after an initial infection and then travel down nerve cell fibers at a later time to cause a renewed infection, which can present with different symptoms from the initial infection. This is the case with the varicella-zoster virus causing chickenpox and later coming back as shingles, and/or Ramsay Hunt syndrome.

If a person has had chickenpox as a young child, they are at risk for developing shingles or Ramsay Hunt syndrome later in life. The initial exposure to varicella-zoster virus results in chickenpox. Once infected, some particles of the virus or the whole virus can settle into nerve cells (neurons) and remain dormant in the body for decades. The virus usually hides in a structure called the dorsal root ganglion, which is a collection of nerve cells next to the spinal cord.

Years later, the virus can be reactivated and cause an infection. Once reactivated, the varicell- zoster virus spreads down the long nerve fibers (axons) and causes inflammation of the surrounding nerves. As the virus replicates and multiplies, the telltale painful blister-like rash erupts. Reactivation of the varicella-zoster virus that attacks the nerves along the torso causes shingles. However, when the virus specifically attacks the facial nerve, it causes Ramsay Hunt syndrome.

Anatomically, the facial nerve leaves the brain to get to the face via a very narrow tunnel called the facial canal. There is a canal that leads to the left side of the face and one to the right side of the face. With Ramsay Hunt syndrome, the varicella-zoster virus infects the facial nerve and causes it to become inflamed. Because the facial canal is so narrow, the slightest inflammation of the facial nerve can lead to it being pinched, resulting in facial paralysis on one side of the face. This is another hallmark symptom of Ramsay Hunt syndrome.

Anyone who has previously had chickenpox is at risk for Ramsay Hunt syndrome. Meaning, a person cannot develop Ramsay Hunt syndrome unless they've had an early exposure to the varicella-zoster virus causing chickenpox.

In the United States, approximately 5 out of every 100,000 people develop Ramsay Hunt syndrome each year. Ramsay Hunt syndrome affects males and females equally. While anyone who has had a previous exposure to the varicella-zoster virus can develop Ramsay Hunt syndrome, most cases are in adults over the age of 60. Ramsay Hunt syndrome is extremely rare in children.

While the varicella-zoster virus is dormant, the virus is harmless and causes no symptoms. However, in some people, the virus can become reactivated. This can happen spontaneously, or it can be triggered by factors that lead to a weakened immune system, such as stress, natural aging, various medical conditions, certain medications (e.g., immunosuppressants), or another infection (e.g., COVID-19). As a result, the weakened immune system is less able to control and keep the varicella-zoster virus inactive, enabling it to spring back into action and cause disease. Thus, people with compromised immune systems are at increased risk of developing Ramsay Hunt syndrome.

Ramsay Hunt syndrome itself is not contagious. Meaning that a person with an active outbreak of Ramsay Hunt syndrome cannot pass Ramsay Hunt syndrome to another person. However, a person with an active case of Ramsay Hunt syndrome can pass the varicella-zoster virus on to people who have never been exposed to the virus before and cause a chickenpox outbreak.

The varicella-zoster virus spreads through direct contact with the fluid from the blisters because the fluid carries the active virus. The varicella-zoster virus in a person with an active case of Ramsay Hunt syndrome is most contagious when the rash is in the blister-phase. Once the blisters scab off, they are no longer infectious. Therefore, people with an active case of Ramsay Hunt syndrome should not come into close contact with people with weakened immune systems because they can more easily contract the virus and become infected with chickenpox.

In the United States and Canada, children are now routinely vaccinated against the varicella-zoster virus with the chickenpox vaccine called Zostavax. This is recommended for all children between 18 months and adolescence, and can greatly reduce the risk of becoming infected with chickenpox. People who have been vaccinated against the varicella-zoster virus are probably less likely to get Ramsay Hunt syndrome as well because the weakened (attenuated) strain of the virus used in the vaccine is less likely to survive in the body over decades. However, in some rare instances, the attenuated strain of the varicella-zoster virus in the vaccine can remain dormant in the nerve cells and become reactivated causing vaccine-induced Ramsay Hunt syndrome.

Most people who develop Ramsay Hunt syndrome have only one episode in their lifetime. However, a person can get Ramsay Hunt syndrome more than once.

The two most visible symptoms of Ramsay Hunt syndrome are:

• Facial weakness or paralysis, usually affecting one side of the face
• A painful shingles-like rash in and around the ear on the same side of the face that is affected by the paralysis.

Facial weakness or paralysis associated with Ramsay Hunt syndrome can make it difficult to smile, frown, or chew food on the affected side of the face. Other symptoms include slurred speech, inability to blink the eye on the side of the face affected by paralysis, and changes in ones sense of taste.

The shingles-like rash is most distinguishable by its red, pus-filled blisters that can cause severe pain. The rash typically appears in and around the ear on the same side of the face affected by the paralysis. In some instances, the rash can also appear in the mouth, usually localized to the roof of the mouth or top of the throat.

Because the facial nerve also travels with the vestibulocochlear nerve, a nerve associated with the ear, hearing and balance can be affected in people with Ramsay Hunt syndrome. Some people may experience problems with balance and hearing, such as ringing in the ear (tinnitus) or temporary hearing loss.

Diagnosis of Ramsay Hunt syndrome can sometimes be difficult because the telltale symptoms of the condition, such as the distinctive shingles-like rash, facial paralysis, and earache, do not always develop at the same time.

However, there are several ways a doctor can diagnose Ramsay Hunt syndrome:

• Performing a physical examination for the hallmark signs of facial weakness or paralysis and the shingles-like rash in the ear or mouth.
• Taking a detailed account of a patient’s medical history to determine whether they have had chickenpox as a child or a shingles outbreak as an adult. This will inform the doctor that there was a prior exposure to the varicella-zoster virus.
• Sending a sample of saliva or blood to look for the varicella-zoster virus, which will confirm whether there was a prior exposure to the virus.
• A sample of the fluid within the blisters or tissue from the rash can be sent to a lab to confirm a diagnosis.

Currently, there is no cure for Ramsay Hunt syndrome. Meaning that if a person has been exposed to the varicella-zoster virus and has had chickenpox, the virus is in the body and Ramsay Hunt syndrome can develop at any time if the virus is reactivated.

While there is no cure for Ramsay Hunt syndrome, attacks can be made less severe and shorter in duration using drugs to treat the symptoms. Using prescription anti-viral drugs, such as acyclovir, valacyclovir, and famcyclovir, prevent the varicella-zoster virus from replicating and multiplying. This can reduce the duration and severity of the viral infection; however, these anti-viral medications do not prevent transmission of the virus.

Other medications are used to treat the symptoms of Ramsay Hunt syndrome, such as corticosteroids (e.g., prednisone) to reduce the inflammation of the nerve and analgesics (pain reliever; e.g., NSAIDs) to reduce the pain from the rash, blisters, and/or earache. A variety of possible medications may also be prescribed to those who experience symptoms such as vertigo (sensation of feeling off-balance).

Beginning treatment within three days of the onset of symptoms is imperative to have the greatest benefit.

Ramsay Hunt syndrome is not a life-threatening condition and complications are rare if treatment starts within three days of the onset of symptoms. However, in rare cases, Ramsay Hunt syndrome can cause complications, such as permanent hearing loss and facial weakness even if treatment was applied immediately and effectively. Thus, it is imperative to start treatment immediately after onset of symptoms to mitigate the possibility of long-term complications.

If treatment is delayed long enough, there is a risk of losing facial movement permanently, as well as uncontrolled movements, such as blinking. In some cases of Ramsay Hunt syndrome, there is the potential for damage to the cornea of the eye (where light passes through for vision) due to extreme dryness. One reason for the dryness is because of the inability to blink caused by the paralysis. Another reason for extremely dry eyes is that the lacrimal gland, the gland that produces the fluid to lubricate the eyes, can become temporary paralyzed during an active outbreak of Ramsay Hunt syndrome because the facial nerve also supplies this gland. Therefore, if the eye on the side of the face affected by the paralysis loses its ability to blink and the eye becomes extremely dry for a prolonged period, the cornea of that eye can become damaged. This can result in constant cornea irritation or permanent vision loss.

While about 75% of those who develop Ramsay Hunt syndrome will recover completely with treatment, approximately 25% of people will develop some type of long-term nerve damage. If Ramsay Hunt syndrome damages the facial nerve, the pain can persist, even long after the condition has subsided. This is known as postherpetic neuralgia. The pain that occurs is the result of the damaged nerve not being able to detect sensations correctly and therefore, sends the wrong signals to the brain.