Ramsay Hunt Syndrome

What is Ramsay Hunt Syndrome?

Ramsay Hunt syndrome, also known as herpes zoster oticus, is a long-term complication of a virus called varicella-zoster virus (VZV), this is the same virus that causes chickenpox. The syndrome is named after James Ramsay Hunt, an American neurologist. It causes inflammation in a particular region of a nerve in your head.

When you first get the VZV infection, you might have a fever and a rash, typically known as chickenpox. After this, the virus often goes to sleep in your body. However, sometimes it can wake up and cause problems. We call this a “zoster” or “herpes zoster” event. This causes pain and a rash along the path of the affected nerve. However, Ramsay Hunt syndrome is quite rare, affecting less than 1% of people with herpes zoster.

Ramsay Hunt syndrome usually has three symptoms: facial paralysis on one side, ear pain, and a rash. However, not everyone gets all these symptoms. Some people might find their face becomes paralyzed before they get a rash. Some people might not get a rash at all, just severe ear pain and facial weakness. This is known as zoster sine herpete, and it can be hard to tell apart from another condition called Bell’s palsy. If there is a rash, it can look like small blisters or raised red spots, and it could spread to one side of the face, scalp, palate, and tongue.

Other symptoms can include a change in taste, dry eye, excessive tearing, sensitivity to loud noises, blocked nose, and difficulty with speaking. Some people may experience hearing loss, ringing in the ears, and dizziness if the virus also affects another nerve that controls hearing and balance. Voice changes or difficulties with swallowing might signal the involvement of the nerve that controls voice and swallowing.

What Causes Ramsay Hunt Syndrome?

Ramsay Hunt syndrome is caused by a virus called varicella-zoster virus. This virus is a part of the herpesvirus family, specifically from the subgroup known as alphaherpesvirinae. Other viruses like herpes simplex viruses 1 and 2 are also part of this group. The varicella-zoster virus is a type of DNA virus, also known as human alphaherpesvirus 3.

After the body fights off the initial infection caused by this virus, usually referred to as chickenpox, the virus doesn’t completely leave the body. Instead, it hides in some part of the nerves, specifically in parts called the cranial nerves or dorsal root ganglia. The virus stays inactive or ‘latent’ in these nerves, and could wake up and reactivate if the body is under stress or if the immune system becomes weak. When it reactivates, it can cause a condition known as “shingles.”

Shingles can happen anywhere in the body. But when the reactivated virus causes facial paralysis, it is known as “Ramsay Hunt syndrome”. Since the introduction of the varicella-zoster virus vaccine in 1995, the number of chickenpox and shingles cases reduced greatly. However, Ramsay Hunt syndrome and shingles still appear in people who have been vaccinated against the virus but haven’t had chickenpox.

Risk Factors and Frequency for Ramsay Hunt Syndrome

Ramsay Hunt syndrome is a condition that can affect anyone, regardless of whether their immune system is strong (immunocompetent) or weak (immunocompromised). About 5 out of every 100,000 people get Ramsay Hunt syndrome each year. However, Bell palsy, a similar condition, is much more common, affecting 15 to 30 out of every 100,000 people each year. Of all the cases of acute facial paralysis, Ramsay Hunt syndrome represents about 7%, and in roughly 30% of those cases, the person may have a variation of this condition known as zoster sine herpete.

People with weaker immune systems may experience a more severe form of the disease and may not recover completely. Ramsay Hunt syndrome can occur at any age. It’s been reported in people as young as 3 months and as old as 82 years. However, people in their 70s and 80s are at the highest risk.

There are several factors that can increase the risk of getting Ramsay Hunt syndrome. These include:

• Stress
• Chemotherapy
• Being immunocompromised
• Infections
• Malnutrition

Essentially, anything that weakens the immune system can increase the likelihood of getting this condition.

Signs and Symptoms of Ramsay Hunt Syndrome

Ramsay Hunt syndrome is a condition often characterized by three main symptoms: paralysis on one side of the face, ear pain, and painful blisters on the ear. However, these symptoms may not all be present at the beginning of the illness. Sometimes, a person may only feel pain without experiencing paralysis or developing a rash. This rash usually shows up in areas affected by the facial nerve, such as the ear, scalp, cheek, tongue, or palate. Generally, before these symptoms appear, individuals may experience a few days of non-specific symptoms like headache, fever, and fatigue.

The paralysis of the face usually develops over 1 to 3 days, and the rash often starts as red bumps before turning into blisters, which then burst and form crusty sores within a week. The stages of these symptoms can vary, and occasionally, the rash may appear after the paralysis. These sores can last for 2 to 3 weeks and usually leave behind red, flat scars.

While a rash isn’t always present, most patients with Ramsay Hunt syndrome report intense ear pain before the rash appears. This pain is usually severe, sharp, or stabbing in nature, can disrupt sleep, and lasts for many days. Additional symptoms that may be experienced during the acute phase of the syndrome include:

• Loudness sensitiveness
• Taste abnormalities
• Blocked nose
• Tearing
• Dry eyes
• Drooling
• Speech difficulties
• Asymmetrical smile
• Dizziness
• Ringing in the ears
• Hearing loss
• Voice changes
• Difficulty swallowing
• Facial numbness

In some cases, the syndrome may be diagnosed even when there isn’t a vesicular rash, particularly if severe pain precedes facial paralysis. This form represents up to 30% of all Ramsay Hunt syndrome presentations and can be difficult to distinguish from Bell’s palsy.

On physical examination, the most noticeable feature is usually unilateral facial paralysis. The facial analysis may reveal a myriad of abnormalities, including an absent skin fold between the nose and upper lip, a droopy eyelid, sagging of the mouth corner, among other features. The examination will assess facial symmetry and any involuntary facial movements, which often indicate abnormal nerve regeneration. This common symptom usually appears 4 to 6 months after the onset of paralysis, particularly in patients with severe initial paralysis.

Additionally, the facial nerve performance is also typically assessed using the House-Brackmann scale. This scale is straightforward and comprises six grades, ranging from I (normal facial function) to VI (no movement at all).

Patients who have difficulty closing their eyes should have their Bell’s phenomenon checked. This phenomenon entails the upward rotation of the eye when the eyelids close and can be lost as patients age. Assessments should also be conducted for corneal sensation, hearing or balance complaints, hoarseness or swallowing difficulty, and cranial nerve examination. The eyes, nose, and oral cavity should also be inspected for vesicular eruptions. In Ramsay Hunt syndrome, these areas do not typically have vesicles, but these can occur in other forms of zoster. Vesicles appearing near the tip of the nose, known as Hutchinson’s sign, can hint at ocular lesions.

Testing for Ramsay Hunt Syndrome

Ramsay Hunt syndrome is usually diagnosed based on symptoms, rather than laboratory tests. This is because tests to confirm the presence of the varicella-zoster virus (VZV), the virus that also causes chickenpox and shingles, are often not practical and can lack sensitivity.

While the virus can potentially be identified from fluid taken from a blister, or from blood, saliva, or tears, these tests are not always reliable. For instance, the Polymerase Chain Reaction (PCR) test, a type of DNA test, only identifies the virus about 58% of the time. Additionally, enzyme-linked immunoassays (ELISA), another type of blood test that can discover antibodies to specific viruses, have a higher success rate (82-99%), but are usually not useful in the early stages of the disease. This is because it takes our body a few weeks to create these antibodies. Also, recent vaccinations against the virus can affect the results of these tests.

Magnetic resonance imaging (MRI) often does show signs of inflammation near the affected facial nerve, but its use isn’t strictly necessary and a CT scan doesn’t generally help with diagnosis. Additional tests, like hearing tests, balance tests, and examinations of the throat with a flexible camera, can determine how many of the cranial nerves are involved. If the cause of facial paralysis isn’t clear from symptoms, then imaging and blood tests may be used, but most Ramsay Hunt syndrome cases are diagnosed from a detailed patient history and physician examination.

Electrodiagnostic testing, like electroneuronography (ENoG) and electromyography (EMG), can also give more information about the extent of nerve damage. These tests can be especially helpful in severe cases of facial paralysis (classified as House-Brackmann grade VI), giving a more precise evaluation than a physical examination alone.

Treatment Options for Ramsay Hunt Syndrome

Herpes zoster, often known as shingles, typically gets better on its own over time. The main aim of treatment is to reduce the risk of later complications, including extreme facial tightness and post-shingles nerve pain. Research has shown that oral antiviral medication and steroid treatment can significantly decrease long-term complications. However, it’s unclear whether these treatments reduce the duration or severity of the immediate symptoms.

Medications such as acyclovir, valacyclovir, and famciclovir have all been studied and found effective for treating shingles. These medications are usually given for 7 to 10 days. Some studies suggest longer treatment, as facial nerve damage can take up to three weeks to recover from.

Along with antiviral medication, high-dose steroids are also given. These can be taken orally or by an intravenous drip. The dosage and length of treatment vary, but the dosage is usually high. This helps manage the inflammation caused by shingles. Some studies suggest that a treatment involving very high doses of steroids, followed by a gradual reduction in dosage, might be beneficial when combined with antiviral therapy.

An alternative treatment method involves injecting steroids into the eardrum, rather than taking them orally. This method could potentially avoid some of the side effects associated with systemic steroids. Some research suggests that this treatment, combined with systemic steroids, can increase recovery rates. However, more research is needed to confirm its effectiveness.

If a doctor prescribes high-dose steroids, they should discuss potential side effects with the patient. These can include changes in mood, difficulty sleeping, stomach issues, high blood pressure, and elevated blood sugar. Close monitoring is required for patients with diabetes who are prescribed high-dose steroids. Sometimes, temporary insulin use might be necessary.

Other treatments aim to manage symptoms like pain and dry eyes. Pain can be managed with various painkillers, anti-inflammatory drugs, and methods for managing nerve pain. Dizziness can be managed with medications like meclizine and benzodiazepines. Eye lubricants can help to prevent dryness and irritation, and some patients might need to use eye tapes at nighttime for protection.

Surgical intervention in such cases is generally considered controversial and typically only offered to patients predicted to have a poor recovery. The role of such surgical intervention in improving recovery in cases like this is still unclear. Long-term management can include physical therapy and injections to treat muscle tightness, as well as surgical procedures to improve facial movements.

What else can Ramsay Hunt Syndrome be?

There aren’t many health conditions that result in both a facial rash and paralysis. Some common reasons for a localized facial rash could be outbreaks of herpes simplex virus, a skin infection known as Staphylococcal impetigo, and contact dermatitis, which can be caused by things applied on the skin like neomycin or exposure to irritants like poison ivy.

On the other hand, acute facial paralysis is most often caused by Bell’s palsy, which comes up with symptoms similar to a condition called zoster sine herpete. But there are also other causes not related to injuries. These could involve:

• Lyme disease
• Non-cancerous tumors at the base of the skull
• Cancers outside the temporal bone
• Autoimmune diseases
• Diseases related to the ear
• Other viral infections
• Neurosyphilis, a nerve disease due to syphilis
• Stroke

Strokes in the cortex part of the brain typically don’t paralyze the forehead, but strokes in the brainstem can lead to paralysis of half the face. Regardless, a stroke that’s progressing should come with unstable vital signs and other neurological symptoms besides facial paralysis.

In the context of diseases affecting the central nervous system as well as viral infections, including SARS-CoV-2, multiple cranial neuropathies, or damage to the cranial nerves, can occur.

What to expect with Ramsay Hunt Syndrome

Everyone recovers from Ramsay Hunt syndrome, a condition that affects facial nerves and causes ear pain and facial paralysis, but the extent of recovery varies. Generally, the severity of facial paralysis matters the most in predicting how well a patient will recover.

For instance, people facing moderate paralysis (known as House-Brackmann grade III) often recover back to normal function. However, patients who have more severe paralysis (House-Brackmann grade IV or V) might regain only some of their facial functions. Similarly, those with the most severe paralysis (House-Brackmann grade VI) may recover partially.

Patients who do not regain their original functions may develop synkinesis, where one movement leads to movements in other parts of the face. Significant long-term paralysis is extremely rare in Ramsay Hunt syndrome, and most people see improvement within a year.

Healthy younger patients with partial paralysis often recover more quickly, usually within a few weeks or months. Around 70% of Ramsay Hunt syndrome patients are likely to regain full or near-full function.

The prognosis for Ramsay Hunt syndrome is poorer than that for Bell’s palsy, another condition that causes facial paralysis. Ramsay Hunt syndrome is associated with a higher likelihood of developing synkinesis and postherpetic neuralgia – a condition where pain lasts for more than three months after the onset of the facial paralysis.

Certain factors can negatively influence recovery, including age over 50, more extensive nerve damage, presence of oral lesions, and diabetes. Interestingly, those who develop a defining rash before the onset of facial palsy (seen in about 25% of patients) seem to have a better prognosis.

Research is also being done to look for ways to predict the patient’s chances of recovery at an early stage by using neutrophil-to-lymphocyte ratio (NLR), a measure related to inflammation. Some studies show that patients with a higher pre-treatment NLR are less likely to recover fully, however, this is still under investigation.

Possible Complications When Diagnosed with Ramsay Hunt Syndrome

Ramsay Hunt syndrome has a range of symptoms including pain, a rash, facial paralysis, and changes to taste, hearing, balance, voice, and speech. Short-term complications of this condition can involve corneal abrasion (a scrape or scratch on your eye) and exposure keratopathy (damage to the eye due to drying). It may also lead to depressive symptoms and social anxiety. Additionally, it can result in the spread of chickenpox to others who are not vaccinated or have weak immune systems.

Long-term, patients typically don’t suffer from flaccid paralysis, where muscles become weak and soft, but synkinesis, where the muscles involuntarily move together, is very common. Other long-term complications of Ramsay Hunt syndrome can include postherpetic neuralgia, a condition causing pain and nerve damage, scars from the vesicles (small fluid-filled blisters), and ongoing depression and/or social anxiety due to facial dysfunction.

Common symptoms and complications:

• Pain
• Rash
• Facial paralysis
• Taste & hearing changes
• Balance, voice, & speech issues
• Corneal abrasion
• Exposure keratopathy
• Depression & social anxiety
• Procedure can transmit chickenpox
• Common occurence of synkinesis
• Postherpetic neuralgia
• Scarring from vesicles
• Ongoing depression and/or social anxiety due to facial dysfunction

Preventing Ramsay Hunt Syndrome

When talking to patients about Ramsay Hunt syndrome, it’s important to clarify that everyone will improve, but not everyone will fully recover. Here, “not fully getting better” can mean experiencing things like synkinesis (unintentional movement or muscle contractions) and postherpetic neuralgia (persistent nerve pain after shingles). One of the critical things for patients to understand is the need to protect their eyes during the time of facial muscle weakness. Even though this weakness is temporary, there’s still a risk of permanent eye damage during this period. For this reason, patients are advised to use artificial tears during the day, lubricating eye ointment at night, and techniques like stretching and taping their eyelids. These simple measures can significantly impact the long-term outcome of their condition.

Patients with active sores also need to avoid close contact with people who are unvaccinated or have weakened immune systems. This is because they can spread the virus causing their condition, the varicella-zoster virus, from their sores.

The shingles vaccine could also potentially prevent Ramsay Hunt syndrome, although it’s not 100% effective. It’s also worth noting that while most people will not get chickenpox or shingles more than once, there have been cases, especially in people with weaker immune systems.