Ramsay Hunt syndrome is a complication of shingles that occurs when the varicella-zoster virus becomes active again and affects the facial nerve near the ear. It can cause weakness or paralysis on one side of the face, often together with severe ear pain and a blistering rash around the ear or inside the mouth.
Other symptoms can include hearing loss, ringing in the ear, dizziness, changes in taste and difficulty closing one eye. In some cases, the rash develops after facial weakness begins or does not appear at all, which can make Ramsay Hunt syndrome more difficult to recognize.
Treatment commonly involves antiviral medicines and corticosteroids, although the available research has not clearly established how much these treatments improve recovery. Medical assessment is important when sudden facial weakness, severe ear pain, a new ear rash, dizziness or hearing changes occur, and vaccination against shingles can help prevent the infection that causes the syndrome.
Main symptoms
The symptoms of Ramsay Hunt syndrome can vary depending on which nerves are affected. Common symptoms include:
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Weakness or paralysis on one side of the face;
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Severe pain in or around one ear;
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A blistering or vesicular rash in or around the ear;
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Blisters or sores inside the mouth;
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Difficulty closing the eye on the affected side;
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Hearing loss;
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Ringing in the affected ear, also known as tinnitus;
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Dizziness or vertigo;
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Changes in taste.
The classic combination associated with Ramsay Hunt syndrome is facial paralysis on one side, ear pain and a blistering rash. However, not everyone develops all three symptoms, and the rash may appear after other symptoms have already started.
In some cases, facial paralysis occurs without a visible rash. This presentation is known as zoster sine herpete and can make the condition harder to distinguish from other causes of facial paralysis.
Common causes
Ramsay Hunt syndrome is caused by reactivation of the varicella-zoster virus, or VZV, which is the same virus that causes chickenpox and shingles.
After a person has chickenpox, the virus does not completely leave the body. Instead, it remains inactive in nerve tissue and can become active again later in life. Ramsay Hunt syndrome develops when this reactivation occurs in or near the geniculate ganglion of the facial nerve, leading to inflammation and problems with nerve function.
Because the facial nerve controls muscles on one side of the face, inflammation can cause facial weakness or paralysis. Nearby nerves involved in hearing and balance may also be affected, which can explain symptoms such as hearing loss, tinnitus and vertigo.
Is Ramsay Hunt syndrome contagious?
Ramsay Hunt syndrome itself is not passed from one person to another. It develops when varicella-zoster virus that is already present in the body becomes active again.
However, a person with active shingles blisters can spread the varicella-zoster virus to someone who does not have immunity to it. Transmission can occur through direct contact with fluid from the blisters or through aerosols containing material from the lesions.
A person who becomes infected in this way can develop chickenpox, not Ramsay Hunt syndrome or shingles directly. Localized shingles is considered contagious from the time the rash appears until the lesions have crusted over, and keeping the lesions covered can help reduce transmission.
Confirming a diagnosis
The diagnosis of Ramsay Hunt syndrome is mainly based on symptoms and a medical examination. Doctors look for characteristic findings such as sudden facial weakness or paralysis on one side, ear pain and fluid-filled blisters around the ear or in the mouth.
Diagnosis can be more difficult when symptoms do not occur at the same time. Facial weakness or severe ear pain may develop before the rash appears, while some people may never develop a visible rash.
Because other conditions can also cause facial paralysis, the pattern and combination of symptoms are important when evaluating the condition. Hearing symptoms, vertigo, the location of skin lesions and the severity of facial nerve involvement can also help support the diagnosis.
Treatment options
Treatment for Ramsay Hunt syndrome is aimed at managing the viral reactivation, inflammation and complications caused by facial nerve problems. Although antiviral medicines and corticosteroids are commonly used, high-quality clinical trial evidence specifically showing their benefit in Ramsay Hunt syndrome remains limited.
Treatment may include:
- Antiviral medicines: Antiviral medicines are commonly included in treatment for Ramsay Hunt syndrome because the condition is caused by reactivation of the varicella-zoster virus.
- Corticosteroids: Corticosteroids may be prescribed to help reduce inflammation affecting the facial nerve. They are often used together with antiviral medicines in clinical practice.
- Intratympanic corticosteroids: In some specialist settings, corticosteroid medicine may be administered directly into the middle ear. This approach has been studied in people with facial paralysis caused by Ramsay Hunt syndrome and Bell's palsy.
- Eye care: Facial paralysis can make it difficult or impossible to fully close the eye on the affected side. Eye care and protection may therefore form part of treatment to help prevent problems associated with incomplete eye closure.
The specific care required depends on the degree of facial weakness and should be determined during medical assessment.
Recovery and prognosis
Recovery from Ramsay Hunt syndrome varies according to the severity and extent of nerve involvement. More severe facial paralysis is generally associated with a less favorable recovery than milder facial weakness.
Hearing problems and involvement of additional nerves can also influence the clinical course. Because outcomes vary considerably, treatment cannot guarantee complete recovery.
When to seek medical care
Sudden weakness or paralysis on one side of the face should receive prompt medical assessment, particularly when it occurs together with severe ear pain or a new blistering rash around the ear or mouth.
Medical evaluation is also important when facial weakness causes difficulty closing an eye or when symptoms such as dizziness, vertigo, tinnitus or new hearing loss occur. The rash may appear later than other symptoms or may be absent, so a visible rash is not required for medical assessment to be appropriate.
Prevention measures
Preventing shingles can reduce the risk of complications caused by reactivation of the varicella-zoster virus, including Ramsay Hunt syndrome.
In the United States, the CDC recommends 2 doses of the recombinant zoster vaccine, Shingrix, for adults aged 50 years and older. Two doses are also recommended for adults aged 19 years and older who are or will be immunodeficient or immunosuppressed because of a disease or treatment.
The CDC reports that Shingrix is 97% effective against shingles in immunocompetent adults between 50 and 69 years old and 91% effective in immunocompetent adults aged 70 years and older. Effectiveness among people who are immunocompromised varies according to the underlying condition.
For people who develop active shingles lesions, keeping the affected area covered can help reduce transmission of the varicella-zoster virus until the lesions have crusted.