Ramsay Hunt syndrome (RHS): Symptoms, causes, treatments

2021-09-07 03:05 PM

Ramsay Hunt syndrome is caused by the varicella-zoster virus, the same virus that causes chickenpox. After chickenpox heals, the virus lies dormant in the nerves.

Ramsay Hunt syndrome (RHS): Symptoms, causes, treatments

Ramsay Hunt syndrome (RHS): Symptoms, causes, treatments

Define

Ramsay Hunt syndrome (RHS) is an infection of the facial nerve with a rash, some of the signs and symptoms of which include pain and weakness in the facial muscles.

Ramsay Hunt syndrome is caused by the varicella-zoster virus, the same virus that causes chickenpox. After chickenpox heals, the virus lies dormant in the nerves. Years later, it can reactivate. If the infecting virus reactivates the facial nerve, the result is Ramsay Hunt syndrome.

The onset of Ramsay Hunt syndrome can be scary. The symptoms can make some people fear they are having a stroke. But, often treatment is effective for Ramsay Hunt syndrome. Sometimes, Ramsay Hunt can lead to facial muscle weakness and hearing loss. Treatment can reduce the risk of complications.

Symptoms

Signs and symptoms of Ramsay Hunt syndrome include:

Painful, red, fluid-filled blisters on the eardrum, the outer ear canal, the outside of the ear, the roof of the mouth, or the tongue.

Facial paralysis on the same side of the affected ear.

Closing one eye is difficult.

Ear hurt.

Hearing poorly.

Tinnitus.

Feeling dizzy or moving.

Change in taste perception, loss of taste.

If you experience hemiplegia or if you develop a rash in or around your ears or in your mouth along with facial paralysis, call your doctor for an evaluation. Ramsay Hunt syndrome is not a problem that requires urgent care. However, diagnosis and treatment within seven days of the onset of signs and symptoms can help prevent long-term complications.

Causes

The cause of Ramsay Hunt syndrome is the activation of the varicella-zoster virus - the same virus that causes chickenpox.

Varicella-zoster belongs to the same strain of the herpes virus, which includes the viruses that cause cold sores and genital herpes. Many of these viruses can lie dormant in the nervous system after the first infection and remain dormant for years before causing another infection.

If the immune system does not destroy all of the varicella-zoster viruses in the initial infection, the virus can reactivate later and infect the facial nerve – causing Ramsay Hunt syndrome. This viral response can also cause other disorders, including shingles (herpes zoster). Researchers do not fully understand how or why the varicella-zoster virus emerged.

Ramsay Hunt syndrome is also called herpes zoster oticus.

Risk factors

Anyone who has chickenpox can develop Ramsay Hunt syndrome. However, it is more common in older adults, usually affecting adults over the age of 60. Ramsay Hunt syndrome is rare in children.

Ramsay Hunt syndrome is not contagious. However, activation of the varicella-zoster virus can cause chickenpox in people exposed to it if they have not had chickenpox before. The disease can be serious in people with weakened immune systems.

Until blisters crust over, avoid physical contact with:

Anyone who has never had chickenpox.

Anyone with a weak immune system.

Infant.

Pregnant women (because chickenpox infection can be dangerous to a developing baby).

Complications

If treated within seven days of the first day, most people with Ramsay Hunt syndrome have no long-term complications. However, in some cases, even with prompt treatment, Ramsay Hunt can cause permanent hearing loss and facial paralysis.

Other possible complications of Ramsay Hunt syndrome include:

Facial abnormalities. A problem called facial abnormality can develop if the infection severely damages the facial nerve and the nerve grows back out of place. This can cause nerves to react inappropriately, such as blinking or tearing up while talking, laughing, or chewing.

Injury to the eye. Facial paralysis caused by Ramsay Hunt syndrome can make it difficult to close the eyelid on the affected side of the face. Incomplete eyelid closure can lead to damage to the front of the eye (cornea). This damage can cause eye pain and blurred vision.

Injury to other parts of the body. In rare cases, the chickenpox zoster virus can spread to other nerves, or to the brain or spinal cord, causing headaches, back pain, lethargy, and limb weakness.

Nerve pain. Nerve pain following viral nerve damage can develop as a result of nerve fiber damage. Damaged nerve fibers cannot send messages from the skin to the brain as they normally do. Instead, the information becomes confusing and exaggerated, causing pain that can linger long after the signs and symptoms of Ramsay Hunt syndrome have subsided.

Testing and diagnosis

Doctors can usually identify Ramsay Hunt syndrome based on medical history, examination, and the distinguishing signs of this disorder and other symptoms. To confirm the diagnosis, the doctor may take a sample of fluid from the rash in the ear and test it for PCR. This test can detect the varicella-zoster virus. The PCR test can also be done on a blood or tear sample. However, the ear fluid test provides more reliable results.

Treatments and drugs

Treatment for Ramsay Hunt syndrome can be about reducing pain and reducing the risk of long-term complications. To treat Ramsay Hunt, your doctor may prescribe the following medicines:

Antiviral drugs, such as acyclovir (Zovirax), famciclovir (Famvir), or valacyclovir (Valtrex).

Corticosteroids, such as prednisone, to reduce swelling and pain.

Diazepam (Valium), to relieve dizziness.

Analgesic.

If facial paralysis persists after treatment, physical therapy with facial exercises can help improve or control the facial muscles.

If you have trouble closing your eyes because of facial paralysis, your doctor may recommend injecting botulinum type A (Botox) into the upper eyelid, allowing it to close and protect the eye.

Depending on the extent of the damage, facial nerve recovery from Ramsay Hunt syndrome can take anywhere from a few weeks to several months. If the damage is severe, or if treatment is slow, full recovery may not be as good.

Lifestyle and remedies

May help relieve the discomfort of Ramsay Hunt syndrome:

Keep the affected area (rash) clean.

Cool, wet compresses relieve pain.

Use an over-the-counter pain reliever or an anti-inflammatory such as ibuprofen (Advil, Motrin, others).

If facial paralysis, use the unaffected side of the mouth to chew.

Practice good oral hygiene, especially after eating.

Rest much.

If facial paralysis makes it difficult to close your eyes, take the following steps to preserve vision:

Use moisturizing eye drops throughout the day if your eyes are dry.

At night, apply ointment to the eyes and eyelids, or wear an eye patch.

Prevention

There is no way to prevent Ramsay Hunt syndrome when the varicella-zoster virus is in the body. However, the chickenpox virus vaccine (Varivax) can reduce the risk of getting the virus. This vaccine is a routine childhood immunization, given between 12 months and 18 months of age. This vaccine is also recommended for older children and adults who have never had chickenpox. The chickenpox virus vaccine prevents chickenpox for most people. If chickenpox develops after vaccination, it is usually less serious.

The U.S. Food and Drug Administration (FDA) has also approved a vaccine (Zostavax) to help prevent shingles and other problems associated with the varicella-zoster virus, such as Ramsay Hunt syndrome in adults 60 years or older. This vaccine is given as a single injection, preferably in the upper arm. The most common side effects are redness, pain, and itching at the injection site.