What You Need To Know About Ramsay Hunt Syndrome And How To Treat It

Ramsay Hunt syndrome — also known as herpes zoster oticus — is a neurological disorder that occurs when the chickenpox virus, varicella-zoster, becomes active again later and affects the facial nerve. The chickenpox virus often stays dormant in certain nerve cells after an infected individual, usually a child, recovers from the disease. 

Many individuals can have the varicella virus latently present in their nerve cells for years without any symptoms or problems. The varicella-zoster virus, however, does not always stay dormant. In some people, the virus can become active again, affecting  the facial nerve, cranial nerve number 7, which extends along the ear and side of the face.

The disorder was named after James Ramsay Hunt, the first American neurologist to describe it. In addition to an ear or mouth rash, this condition is marked by unilateral paralysis of  a facial nerve, ear pain, and a rash around the ear. 

What are the symptoms of Ramsay Hunt syndrome?

Ramsay Hunt syndrome symptoms might differ between affected individuals. People with this condition usually have facial nerve paralysis, ear pain/ringing, and an ear rash. Sometimes these symptoms don't happen at the same time. The condition often only affects one side of the face.

Nerve paralysis can cause weak or stiff facial muscles, which can make it hard to smile or frown. This can give the affected person a "saggy face." Some individuals may exhibit asymmetric muscular tone and have drooping jaws. Constant irritation from an open eye is a common outcome of being unable to close it. Injuries to the cornea can cause temporary or permanent visual loss.

Most people with Ramsay Hunt syndrome have a red, painful, blistering rash that is filled with fluid. The rash typically extends around the ear and into the external ear canal. The top of the throat, mouth, eardrum, and soft palate may also be affected by the painful blisters and rash. This is most common on the side where the nerve is affected. However, the rash may not develop in about 30% of the cases, which can make this condition difficult to diagnose or differentiate from other issues that cause facial paralysis, such as Bell's palsy.

Additional symptoms include: 

• Pain in your neck and the affected ear

• Slightly slurred speech

• A ringing noise in your ear

• A feeling like the room is spinning

• A decreased sense of taste

What causes Ramsay Hunt syndrome?

The varicella-zoster virus — also responsible for chickenpox and shingles — is the culprit in the development of Ramsay Hunt syndrome. If you had chickenpox when you were younger, the virus might still be in your system decades later. When the varicella-zoster virus becomes active again, it causes shingles. In rare cases, the active virus may cause Ramsay Hunt syndrome when it affects the facial nerves. 

In Ramsay Hunt syndrome, it is unclear why the virus becomes active again and damages the facial nerve. However, it is believed that the virus can spread from the spinal cord to the facial nerve when the immune system is impaired. This can happen due to many different factors, including aging, disease, and stress.  

Who’s at risk of getting Ramsay Hunt syndrome?

Approximately 5 out of every 100,000 people in the United States are diagnosed with Ramsay Hunt syndrome each year. However, the true prevalence of Ramsay Hunt syndrome in the general population is unknown. This is because the disease often goes undetected or is misdiagnosed.¹

Both sexes are equally at risk of getting Ramsay Hunt syndrome. A person with Ramsay Hunt syndrome cannot spread the disorder itself. However, they can spread chickenpox to healthy individuals who have never had the disease before. A higher incidence of the disorder is seen in immunocompromised individuals and in people over 60 who have previously experienced chickenpox. Ramsay Hunt syndrome in children is very unusual, but it has been documented.

The CDC recommends that those with Ramsay Hunt syndrome avoid coming into touch with older adults or anyone with an impaired immune system. It also recommends avoiding contact with those who have never had chickenpox, newborns, and pregnant women until their blisters have scabbed over.

What is the optimal treatment for Ramsay Hunt syndrome?

If you receive prompt medical attention for Ramsay Hunt syndrome, it can alleviate symptoms and reduce the likelihood of permanent damage. Different drugs can be used to treat Ramsay Hunt syndrome. 

Your doctor could recommend that you take:

• Antiviral drugs. The chickenpox virus can be treated with antiviral drugs, including acyclovir (Zovirax), famciclovir (Famvir), and valacyclovir (Valtrex). Antiviral therapy is most effective when taken within 72 hours from the beginning of the symptoms. 

• Corticosteroids. Combining antiviral medications for Ramsay Hunt syndrome with a brief course of high-dose prednisone may increase their efficacy.

• Antidepressants. Even though antidepressants are typically prescribed to treat depression, they can be helpful when used to treat the neurological pain associated with Ramsay Hunt syndrome. Damage to the neurological system can cause excruciating pain known as neuropathic pain, which can last for a while even after the other symptoms disappear and may be alleviated with this treatment. 

• Pain relievers. You can treat the pain associated with Ramsay Hunt Syndrome with over-the-counter or prescription pain medicines. 

Using a cold compress is one of the more non-invasive ways to deal with the discomfort of Ramsay Hunt syndrome. You could also use a patch to cover the eye. Artificial tears or other moisturizing eye drops can also be used to prevent the eyes from drying out. In the case of total facial paralysis, your doctor may recommend surgical intervention. The operation can lessen the pressure on your facial nerves, improving some symptoms.

How long does it take to recover from Ramsay Hunt syndrome?

The amount of time it takes to regain your facial movement after suffering from Ramsay Hunt syndrome depends on the individual. For generally healthy patients with minimal nerve damage, signs of improvement may be noticed in a few weeks to a few months. However, to completely heal, it could take as long as a year. If the infection damages more of the facial nerve, there is less chance of a full recovery.

Antiviral medications can help. If you start taking them early, you have a greater chance of making a full recovery. The long-term prognosis is better if diagnosis occurs early and the disease is treated quickly with antiviral therapy. With treatment, over 70% of those affectedby Ramsay Hunt syndrome will regain full or nearly full use of their faces. Even with quick medical attention, some cases might still suffer from lifelong facial paralysis or hearing loss.

What are the complications of Ramsay Hunt syndrome?

A potential complication of Ramsay Hunt syndrome is a reconnection of nerves to inappropriate facial tissues. Those affected may respond erratically to physical stimuli. For example, if you smile too widely, one of your eyes might close. 

Additional difficulties that can arise from the condition include:

• Permanent hearing loss and facial weakness. The facial paralysis and acute hearing issues that are hallmarks of Ramsay Hunt syndrome are usually temporary. However, the damage done by the infection can become permanent.

• Eye damage. You may have trouble closing your eyelids due to Ramsay Hunt syndrome-related facial paralysis. When this happens, there is a risk that the cornea, the outermost layer of the eye, will be injured. As a result, your eyes may start to hurt, and your vision may be impacted.

• Postherpetic neuralgia. When a herpes zoster virus infection impacts nerve fibers, it can cause this painful disease. Pain can persist long after other Ramsay Hunt syndrome symptoms have subsided.

Signs that the virus may have spread to other nerves, including the brain and spinal cord, are:

• Confusion

• Tiredness

• Back discomfort

• Weakness in the arms and legs

• Headaches

In these circumstances, a spinal tap may be required to identify which parts of the patient's neurological system have been compromised.

How is Ramsay Hunt syndrome diagnosed?

To diagnose Ramsay Hunt syndrome, your doctor will do a physical examination. They will ask about your health background and current symptoms. Ramsay Hunt syndrome can be difficult to diagnose. That is because the condition's three main symptoms don't always appear at the same time. Therefore, your doctor may order a laboratory test to double-check the diagnosis. 

Depending on the situation, the physician may take a blister fluid, blood, or saliva sample from you. A pathologist will examine the specimen to look for the varicella-zoster virus. Magnetic resonance imaging (MRI) scans are another imaging test your doctor may recommend. These tests may help your doctor rule out that your symptoms are due to something else.

The lowdown

The effects of Ramsay Hunt syndrome tend to be temporary in most cases. But if you wait too long to receive treatment, you risk lifelong muscle weakening in your face and possible hearing loss. It's important to consult a doctor immediately after experiencing symptoms to receive prompt treatment. 

According to the CDC, you can reduce the risk of infection and complications from the varicella-zoster virus in two ways. Firstly, with the chickenpox vaccine in children or the shingles vaccine in adults 50 and older. The vaccines greatly reduce the risk of developing infection and complications from varicella-zoster virus and, by extension, Ramsay Hunt syndrome.

Frequently asked questions

1. Does Ramsay Hunt syndrome go back to normal?

Yes. Complete recovery is typically achieved within a few weeks to months if there is no significant nerve damage.

2. Can stress cause Ramsay Hunt syndrome?

Yes. Since stress has been linked to shingles outbreaks, it stands to reason that it could also trigger RHS.

3. How many types of Ramsay Hunt syndrome are there?

The name "Ramsay Hunt syndrome" has been given to at least three distinct neurological disorders. The only tie they have is that they were first recorded by the renowned neurologist James Ramsay Hunt.

4. Which celebrities have battled facial paralysis?

Justin Bieber, Angelina Jolie, George Clooney, Pierce Brosnan, Sylvester Stallone, Katie Holmes, and more.