What are the other Names for this Condition? (Also known as/Synonyms)

• Herpes Zoster Oticus
• RHS (Ramsay Hunt Syndrome)

What is Ramsay Hunt Syndrome? (Definition/Background Information)

• Ramsay Hunt Syndrome (RHS) is a condition that occurs when shingles infection leads to paralysis of the facial nerve as well as painful rashes near one of the ears. It is the second most common cause of atraumatic peripheral facial paralysis
• Shingles is a viral infection that results in painful skin rashes, tingling and burning sensation, and itching. Ramsay Hunt Syndrome is caused by the varicella zoster virus (VZV), the virus responsible for childhood chickenpox and shingles in adults
• After a case of chickenpox, VZV remains in a state of dormancy within the nerves. The virus can be re-activated years later and harm the facial nerve. In addition to causing shingle rashes and facial paralysis, Ramsay Hunt Syndrome can also lead to hearing loss and vertigo, and a spinning sensation that
• The treatment of the condition involves the administration of antiviral medications. With early diagnosis and treatment, the outcomes are usually excellent, especially for mild cases of Ramsay Hunt Syndrome

Who gets Ramsay Hunt Syndrome? (Age and Sex Distribution)

• Any individual who has acquired chickenpox in the past can be at risk of developing Ramsay Hunt Syndrome (RHS), as the varicella zoster virus responsible for RHS is also the cause of chickenpox
• Males and females have an equal risk, and the majority of RHS patients are typically aged 60 and above. Children are rarely affected by RHS

It is estimated that every year, about 1 in 20,000 individuals in the U.S. acquire Ramsay Hunt Syndrome, which is typically more common in older adults.

What are the Risk Factors for Ramsay Hunt Syndrome? (Predisposing Factors)

• One risk factor associated with Ramsay Hunt Syndrome (RHS) is having had chickenpox in the past
• The varicella zoster virus responsible for chickenpox can lie dormant in one’s nerves even after recovery from chickenpox; the potential re-activation of the virus can ultimately affect the facial nerve and lead to symptoms characteristic of RHS

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Ramsay Hunt Syndrome? (Etiology)

The cause of Ramsay Hunt Syndrome (RHS) is the varicella zoster virus (VZV), which is responsible for causing both chickenpox and RHS.

• A recovery from a case of chickenpox does not necessarily suggest the complete elimination of the infecting virus from the body; instead, the VZV can stay dormant in the nerves for years after the initial case of chickenpox
• If re-activated, the VZV can result in shingles, a viral infection characterized by painful rashes. The shingles infection can then develop into RHS, which is when the virus has spread to the facial nerve. Even so, the exact mechanism of re-activation of the varicella zoster virus is yet unestablished

Ramsay Hunt Syndrome is not a contagious disease. However, re-activation of the virus in an individual with RHS can lead to chickenpox in those who have not previously had the infection or been vaccinated against chickenpox.

What are the Signs and Symptoms of Ramsay Hunt Syndrome?

The signs and symptoms of Ramsay Hunt Syndrome (RHS) can vary from one individual to the other. However, there are two major symptoms typically associated with RHS:

• Facial paralysis and weakness
• Painful, blistering rash on external ear canal and outer region of the ear (pinna)

In most cases, facial paralysis and skin rash occur simultaneously. Sometimes, however, the paralysis can develop before the rash or after. Also, these two presentations usually affect only one side of the face.

Other signs and symptoms associated with RHS include:

• Painful, blistering rash in the mouth, soft palate, and throat
• Hearing loss (typically transient)
• Ear pain
• Difficulty smiling or closing eye on the affected side
• Ringing in the ears (tinnitus)
• Vertigo (sensation that the surrounding environment is spinning), nausea, vomiting
• Loss of taste 
• Over-sensitivity to sounds (hyperacusis)

Individuals with Ramsay Hunt Syndrome may have paralysis in the face, but no rash or other skin irregularities. In these cases, individuals are known to have a condition termed zoster sine herpete. 

How is Ramsay Hunt Syndrome Diagnosed?

• Ramsay Hunt Syndrome (RHS) is typically diagnosed after a detailed evaluation of the patient’s history and evaluation of symptoms unique to RHS, including facial paralysis and painful rash near the ear
• Moreover, blood tests and fluid samples from rash blisters can be examined to reveal the presence of varicella zoster virus (VZV)
• At times, diagnosing RHS can be difficult, especially if the distinctive symptoms do not develop simultaneously

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Ramsay Hunt Syndrome?

• Although facial paralysis and hearing loss is temporary for most people with Ramsay Hunt Syndrome (RHS), complications can lead to permanent paralysis and loss of hearing.
• In addition, the difficulty in closing one’s eye can result in injury to the cornea, which covers the front of the eye. Corneal damage can cause blurred vision or eye pain
• RHS can also lead to postherpetic neuralgia, or persistent pain, caused by damage of nerve fibers due to a shingles infection

How is Ramsay Hunt Syndrome Treated?

Antiviral medications are commonly prescribed for individuals with Ramsay Hunt Syndrome (RHS), typically for 7-10 days.

• It is recommended to start antiviral medications within 3 days of onset of RHS, which improves the chance of recovery and potentially lead to the greatest benefit
• Antiviral drugs are often used together with corticosteroids, such as prednisone, which have been known to enhance the effect of the antiviral drugs against symptoms of RHS. These medications are typically prescribed for 5-7 days

Symptomatic treatments are available to help manage specific signs and symptoms in individuals with RHS.

• Anti-anxiety medications, including diazepam, can be used to manage vertigo (a feeling that the environment is spinning)
• Pain medications are prescribed to reduce the pain associated with the blistering rash often experienced by RHS patients
• Moreover, individuals who find it difficult to completely close their eye can be at risk of corneal damage due to dryness or entry of foreign debris. They may be prescribed lubricating eye ointments or artificial tears to help protect the cornea

How can Ramsay Hunt Syndrome be Prevented?

• Vaccination against chickenpox for children can help reduce the risk of acquiring the infectious viral infection
• Moreover, a vaccine against shingles is available for adults over the age of 60.

Both of these measures can be taken to prevent the spread of the varicella zoster virus to the facial nerve, which results in Ramsay Hunt Syndrome.

What is the Prognosis of Ramsay Hunt Syndrome? (Outcomes/Resolutions)

• It has been suggested that a full recovery from Ramsay Hunt Syndrome (RHS) can occur in about a few weeks if the damage to the facial nerve is not severe
• On the other hand, if the damage is serious, complete recovery may not occur even after many months. Overall, more severe damage results in not only a longer recovery time, but also in reduced chances of even achieving a full recovery
• In addition, there is a better chance of recovery if treatment is started within 3 days of the first appearance of symptoms

It is estimated that about 70% of the RHS patients fully recover if treated within this period of time; while if more than 3 days have passed, this number reduces to about 50% of the patients.

Additional and Relevant Useful Information for Ramsay Hunt Syndrome:

• Chickenpox and shingles test may be performed to detect and diagnose a current or past chickenpox and/or shingles (herpes zoster) infection. The infections are caused by the varicella zoster virus (VZV)

The following resource link provides more information about the test:

https://www.dovemed.com/common-procedures/procedures-laboratory/chickenpox-and-shingles-test/