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, infects 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 without any symptoms or problems. The varicella-zoster virus, however, does not always dormant. In some people, the virus can become active again, infecting the facial nerve.

The disorder was named after James Ramsay Hunt, the first person to describe it. Ramsay Hunt was an American neurologist and Army officer serving in World War I. In addition to an ear or mouth rash, this condition is marked by the paralysis of the facial nerve. 

What are the symptoms of Ramsay Hunt Syndrome?

Symptoms might differ greatly between affected individuals. People with this condition usually have facial nerve paralysis and an ear rash. Sometimes these two signs don’t happen at the same time. Most people only have it on one side of their face.

Nerve palsy can cause facial muscle weakness or stiffness, leading to a “saggy face” from the inability to smile or furrow the brow. Facial weakness is typically at its worst within a week of the commencement of symptoms. Some individuals may exhibit asymmetric muscular tone and have drooping mouths. The inability to close the eye frequently arises and results in irritation. Occasionally, the cornea might get injured, resulting in impaired vision.

Most people with the condition have a red, painful, blistering rash that is filled with fluid and spreads to the outside of the ear and often the outside of the ear canal. The painful blisters and rash may also affect the top of the throat, mouth, eardrum, and soft palate, especially on the side where the nerve is affected.

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 the illness. If you had chickenpox as a kid, the virus might still be lurking in your system decades later. When the varicella-zoster virus becomes active again, it causes shingles and, in rare cases, Ramsay Hunt syndrome when it affects the facial nerves. 

Additionally, 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, which can happen due to many different factors, including aging, disease, and stress. 

Who is at risk of getting Ramsay Hunt Syndrome?

According to statistics, 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 the disease in the general population is unknown because it often goes undetected or is misdiagnosed.

Both sexes are equally affected by Ramsay Hunt syndrome. Although an infected person cannot spread the disorder itself, they can spread chickenpox to healthy individuals who have never had the disease before. A higher incidence of the disorder is seen in people over 60 who had previously experienced chickenpox. Ramsay Hunt Syndrome in children is highly unusual but has been documented.

The CDC recommends that the infected avoid coming into touch with the immunocompromised, 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?

Prompt medical attention for Ramsay Hunt syndrome can alleviate symptoms and lessen the likelihood of permanent damage. Different drugs can be used to treat the illness. Your doctor might recommend that you take the following:

• Antiviral drugs. The chickenpox virus can be treated with antiviral drugs, including acyclovir (Zovirax), famciclovir (Famvir), and valacyclovir (Valtrex).
• Corticosteroids. Antiviral medicines for Ramsay Hunt syndrome may be more effective when combined with a brief course of high-dose prednisone.
• Anti-anxiety medications. Even though antidepressants are typically prescribed to treat depression, they can be helpful when used to treat Ramsay Hunt syndrome. Damage to the neurological system can cause excruciating pain known as neuropathic pain, which can be alleviated with this treatment. Particularly, tricyclic antidepressants have demonstrated efficacy in treating Ramsay Hunt syndrome.
• Pain relievers. The pain associated with Ramsay Hunt Syndrome can be treated with over-the-counter or prescription pain medicines. Doctors sometimes administer opioid medicine for patients experiencing severe pain from herpes zoster.

One of the more non-invasive ways to deal with the discomfort of Ramsay Hunt syndrome is to use a cold compress. You could also cover the eye with a patch. You can also use artificial tears or eye drops that keep the eyes moist to keep them from drying out. Your doctor may recommend surgical intervention if you have a total facial paralysis. The operation has the potential to lessen the strain on your facial nerves.

What is the recovery time for Ramsay Hunt syndrome?

How long it takes to regain your facial movement after suffering from Ramsay Hunt syndrome is quite individual. In cases when nerve damage is minimal, and the patient is generally healthy, signs of improvement may be noticed in as little as two to four months. Complete healing, however, could take as long as a year. There is less probability of a full recovery if the infection damages more of the facial nerve.

Antiviral medications can help, and if you start taking them within three days of your symptoms emerging, you have a greater chance of making a full recovery. The long-term prognosis is better if the disease is diagnosed early and treated quickly with antiviral therapy. With treatment, over 70% of those the infected will regain full or nearly full use of their faces. Despite quick medical attention, some cases might cause lifelong facial paralysis or hearing loss.

What are its complications?

One of the potential complications of Ramsay Hunt syndrome is a reconnection of nerves to the inappropriate facial tissues. Affected individuals may respond erratically to physical stimuli. For example, if you smile too widely, you might find that one of your eyes closes. Additional difficulties that can arise from the condition include the following:

• Permanent hearing loss and facial weakness. The facial paralysis and acute hearing loss 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 occurs, 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.
• Postherpetic neuralgia. When a herpes zoster virus infection affects nerve fibers, it can cause this excruciating disease. Pain can persist long after other Ramsay Hunt syndrome symptoms have subsided because of the miscommunication and exaggeration of pain signals provided by these nerve fibers.

Confusion, tiredness, back discomfort, weakness in the arms and legs, and headaches may be symptoms of the virus’s spread to other nerves, including the brain and spinal cord. In such circumstances, a spinal tap may be necessary to identify which parts of the patient’s neurological system have been compromised.

How is Ramsay Hunt Syndrome Diagnosed?

Diagnosing the disease requires a physical examination. They will inquire as to your health background and current symptoms. Ramsay Hunt syndrome is difficult to diagnose since the three main symptoms — earache, facial paralysis, and the characteristic rash — do not usually appear simultaneously. Consequently, your doctor may order a laboratory test to double-check the diagnosis. 

Depending on the situation, the physician may take a saliva, blood, or blister fluid sample from you. The specimen will be examined by a pathologist who will be looking for the varicella-zoster virus. Magnetic resonance imaging (MRI) scans are another type of imaging test that your doctor may recommend. The examination may help your doctor rule out the possibility that your symptoms are due to something else.

The lowdown

The effects of the illness tend to be temporary. However, if you wait too long to get treatment, you risk lifelong muscle weakening in your face and even hearing loss. It’s important to see a doctor as soon as possible after experiencing symptoms to get prompt treatment. 

According to the CDC, you can prevent the varicella-zoster virus with either the chickenpox vaccine in children or the shingles vaccine in adults 50 and older. The vaccines significantly lower the risk of developing the varicella-zoster virus and, by extension, Ramsay Hunt syndrome.