What is Ramsay Hunt Syndrome: Causes, Symptoms, Treatment

What is Ramsay Hunt Syndrome?

Ramsay Hunt Syndrome is a rare disorder in which the facial nerve gets infected. This infection is caused by the varicella zoster virus when it attacks certain nerves in the brain. It is the same virus that causes chickenpox. People who have a history of chickenpox carry this dormant virus in their body which makes them vulnerable to Ramsay Hunt Syndrome if the virus becomes active after some years. The primary presenting features of Ramsay Hunt Syndrome includes development of rashes and weakness of the facial muscles. It is often at times confused with stroke.[1,2,3]

There is treatment available for Ramsay Hunt Syndrome but in some cases the damage caused by it may become permanent and some people may even end up having hearing loss due to it. If a correct diagnosis is made on time and prompt treatment is given, then the patient can be protected from any complications that may arise due to Ramsay Hunt Syndrome. If the treatment is delayed then the chances of complete recovery from Ramsay Hunt Syndrome drops significantly.[1,2,3]

As stated, Ramsay Hunt Syndrome is a rare disease and has an incidence rate of approximately 5 in 100,000 people in the United States. Ramsay Hunt Syndrome can affect both people with a healthy immune system or people with a compromised immune system; however, the severity of the disease is more in people who are immunocompromised.[1,2,3]

Females are believed to be affected more than males by about 20%. Around 12% of all facial nerve paralysis cases occur due to Ramsay Hunt Syndrome. This condition has been noted to affect people from age range of 5 to 80. There are also some risk factors associated with Ramsay Hunt Syndrome which include stress, history of chemotherapy, and as stated a history of chickenpox.[1,2,3]

What Causes Ramsay Hunt Syndrome?

As mentioned above, Ramsay Hunt Syndrome is a viral infection caused by the varicella zoster virus. This virus is also associated with conditions like chickenpox and shingles. The virus may remain dormant for a very long time in a person who has a history of chickenpox in childhood. If the virus becomes active again it results in shingles and rarely when certain nerves in the brain get affected causes Ramsay Hunt Syndrome. The reason behind why the virus all of a sudden gets activated and affects the facial nerve is not yet known.[2,3]

What are the Symptoms of Ramsay Hunt Syndrome?

The symptoms of Ramsay Hunt Syndrome are varied and differ from person to person. The primary symptom however of this condition is facial nerve paralysis which is seen in almost all the cases. There is also development of a rash around the ears. These two symptoms may occur together but not always and in majority of the cases it is unilateral. The person will find it tough to smile, close the eyes, and wrinkle the forehead. Speech also gets affected in some cases.[2,3]

When speaking of the rash seen with Ramsay Hunt Syndrome, they are painful blisters that are erythematous. They usually are present on the outer part of the ear.

Sometimes, the mouth and the top of the throat also can have these rashes. People with Ramsay Hunt Syndrome also complain of tinnitus and ear pain as well which at times can be severe. This pain may also radiate to the neck.[2,3]

Some people also have sensorineural hearing loss as a result of Ramsay Hunt Syndrome. This hearing loss is temporary but if proper and timely treatment is not given then it may become permanent. Hyperacusis is yet another symptom of Ramsay Hunt Syndrome. In such people, normal sounds may appear to louder as a result of which the daily life of the person gets severely affected. Some of the other symptoms of Ramsay Hunt Syndrome include nausea, vomiting, vertigo, and rarely dry mouth, loss of taste, and dry eyes.[2,3]

How is Ramsay Hunt Syndrome Treated?

As Ramsay Hunt Syndrome is a viral infection, the primary mode of treatment is by way of antiviral medications. Acyclovir is the most preferred medication to treat Ramsay Hunt Syndrome. This medication is often given in combination with corticosteroids. For best results, it is necessary to start treatment within three days of the onset of symptoms which makes it even more important for a prompt diagnosis and treatment. However, there are some cases in which a person ends up having permanent deficits despite treatment but such cases are quite few.[2,3]

More treatments are required for calming down the symptoms of Ramsay Hunt Syndrome. These include medications to control neuralgic pain and medications for vertigo. It should be noted here that people with Ramsay Hunt Syndrome should take proper care of their eyes, especially the cornea, and prevent them from any injury as due to this condition such people are not able to close their eyes properly and this makes them vulnerable for injuries. For this, the patient might be given lubricants or ointments to protect the cornea and prevent any injuries due to Ramsay Hunt Syndrome.[2,3]

References:

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