Ramsay Hunt Syndrome (Herpes Zoster Oticus)

Understanding the causes, symptoms and treatment options for Ramsay Hunt Syndrome.

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What is Ramsay Hunt Syndrome?

Ramsay-Hunt Syndrome , or Herpes Zoster Oticus, is a viral disease, also known as shingles in ear. It arises from the reactivation of the varicella zoster virus. Shingles is usually noticeable as a painful rash. The causes of the outbreak are often unknown, but the main cause is the weakening of the immune system.

What is varicella zoster virus?

The varicella-zoster virus (VZV) is the virus responsible for both chickenpox and shingles. After causing chickenpox, typically in childhood, the virus remains dormant in the body’s nerve cells. Years or even decades later, it can reactivate as shingles, especially in older adults or individuals with weakened immune systems. This reactivation can lead to a painful rash, nerve pain, and in some cases, complications such as facial paralysis, hearing loss, or balance issues if the virus affects the nerves near the ear.

How does Ramsay Hunt syndrome develop?

Ramsay-Hunt Syndrome, occurs when the herpes zoster virus affects in the facial and cranial nerves. It is the cranial nerve that controls hearing and balance. This particular virus rests in the nerve roots after chickenpox and can be reactivated if the immune system is weakened. It migrates along the nerve tracts and causes inflammation of the affected nerve tissue up to the skin, where painful wounds then develop. These wounds are caused by burst vesicles, which then form a crust and gradually heal. This rash with small blisters behind the ear and in the ear usually indicates shingles in the ear, a herpes zoster oticus.

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Symptoms of Ramsay Hunt Syndrome

The symptoms of Ramsay Hunt Syndrome are different but clear. The first sign that make you recognise shingles in the ear is a burning pain under the skin. You can feel this days before the familiar blisters form. In addition, itching and hypersensitivity can occur on the affected skin area and the affected person often feels exhausted and feverish. Herpes zoster oticus can also be recognised by the following symptoms:

Ramsay Hunt Syndrome earache

Often the first symptom, this pain is typically sharp, burning, or throbbing and localised deep within or around one ear. It may begin days before any visible rash appears and can radiate to the jaw, neck, or side of the head.

Ramsay Hunt Syndrome vesicles

These painful blisters usually appear:

On the outer ear (auricle)
Inside the ear canal
On the earlobe

Occasionally on the tongue, roof of the mouth, or neck

They are a hallmark of herpes zoster oticus and may crust over after a few days.

Ramsay Hunt Syndrome hearing loss

Hearing loss in the affected ear can range from mild to profound. It may be temporary or, in some cases, permanent. This occurs due to inflammation of the auditory nerve.

Ramsay Hunt Syndrome facial paralysis

This is a defining feature of Ramsay Hunt Syndrome. It typically affects one side of the face and may cause:

Drooping of the mouth or eyelid
Inability to smile, close the eye, or raise the eyebrow
Slurred speech or difficulty eating and drinking
Reduced tear and saliva production
Altered or lost sense of taste on the front two-thirds of the tongue

Ramsay Hunt Syndrome dizziness

Dizziness can be intense and disorienting, often accompanied by nausea or balance issues. It results from viral involvement of the vestibular nerve.

Ramsay Hunt Syndrome headaches

These may be generalised or localised around the ear and temple. They are often due to nerve inflammation and can persist throughout the infection.

Hyperacusis due to Ramsay Hunt Syndrome

Sounds may seem unusually loud or sharp in the affected ear. This sensitivity is due to dysfunction of the stapedius muscle, which normally dampens loud noises.

Ramsay Hunt Syndrome tinnitus

A persistent ringing, buzzing, or hissing sound in the ear may accompany hearing loss and can be distressing.

What are the causes of ramsay hunt syndrome?

Ramsay Hunt Syndrome (herpes zoster oticus) is caused by the reactivation of the varicella-zoster virus, the same virus that causes chickenpox and shingles. After a person recovers from chickenpox, the virus remains dormant in the nerve ganglia, clusters of nerve cell bodies near the spinal cord or cranial nerves. It can reactivate years later, particularly in the facial nerve near the ear, leading to Ramsay Hunt Syndrome.

Weakened immune system

A weakened immune system is the most common trigger for VZV reactivation. This can be due to:

Ageing: Immunity naturally declines with age, making older adults more susceptible.
Chronic illnesses: Conditions like diabetes, cancer, or autoimmune diseases can impair immune function.
Immunosuppressive treatments: Chemotherapy, radiation, or long-term corticosteroid use can reduce the body’s ability to control latent viruses.
HIV/AIDS: People with HIV have a significantly higher risk of shingles and related complications like Ramsay Hunt Syndrom.

Ramsay Hunt Syndrome due to stress and fatigue

High levels of physical or emotional stress can suppress immune responses, creating an opportunity for the virus to reactivate.

Exposure to radiation or UV light

Prolonged exposure to X-rays or ultraviolet (UV) radiation may contribute to viral reactivation by damaging skin and nerve tissues or weakening local immune defences.

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How do you diagnose Ramsay Hunt?

The diagnosis of shingles in the ear is usually easy for the attending physician to make. A physical examination of the patient can determine the viral infection. The vesicles and redness on the ear and in the area of the ear are characteristic of herpes zoster oticus. Further diagnostics are only necessary in rare problem cases. If in doubt, the doctor will have the pathogen detected in the secretion of the vesicles or - if the brain is suspected of being involved - in the cerebrospinal fluid.

Ramsay Hunt Syndrome treatment

Antiviral drugs are used to treat shingles in the ear. These drugs stop the virus from multiplying and relieve pain and improve skin symptoms within two hours. In the case of herpes zoster oticus, anesthetic ear drops are also used to relieve the pain, and even antibiotic ear drops, as there is an additional risk of bacterial infection. Antiviral therapy should be started no later than 72 hours after the onset of the skin symptoms, so the person concerned should contact the doctor about this if he has any first suspicions.

Home remedies

Cold towels or compresses alleviate the discomfort
Powder and creams with local anesthetics have a pain-relieving effect both in acute shingles and in post-zoster neuralgia
Lots of rest and sleep so that the disease is overcome faster
Shingles patients are contagious - good hygiene is essential. Close contact with others, especially babies, pregnant women, young children and sick people, should also be avoided
Wear loose clothing as the affected areas of the skin are very sensitive to touch.

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Bell's palsy and ramsay hunt syndrome

Both Bell’s palsy and Ramsay Hunt Syndrome involve sudden facial paralysis, but they have different causes and symptoms. Bell’s palsy is often linked to a viral infection, commonly the herpes simplex virus, and usually appears without warning, often without pain or other symptoms. Most people recover fully within a few weeks.

Ramsay Hunt Syndrome, on the other hand, is caused by the reactivation of the varicella-zoster virus (the same virus that causes chickenpox and shingles). It typically presents with additional symptoms such as severe ear pain, a blistering rash around the ear, hearing loss, and dizziness. Recovery can take longer, and the condition is generally more complex to treat.

Long term effects of ramsay hunt syndrome

While many people recover well, especially with early treatment, some may experience long-term effects. These can include:

Persistent facial weakness: Some people may not regain full control of their facial muscles, leading to asymmetry or difficulty with expressions.
Chronic ear pain: Nerve damage can result in ongoing discomfort or sensitivity in and around the ear.
Permanent hearing loss: If the auditory nerve is affected, hearing may not fully return in the affected ear.
Tinnitus: A constant ringing or buzzing sound in the ear may continue even after the infection has cleared.
Balance issues: Damage to the inner ear can lead to long-term dizziness or unsteadiness.
Eye complications: Difficulty closing the eye on the affected side can lead to dryness or irritation if not properly managed.
Emotional impact: Living with visible facial changes or chronic symptoms can affect confidence and emotional wellbeing.

FAQs about the ramsay hunt syndrome

Is shingles in the ear contagious?

Yes, shingles in the ear is also contagious. Chickenpox pathogens are contained in the secretion of the vesicles and that is why a patient with shingles is contagious - until the last vesicle has opened and crusted over. People who have never had chickenpox in particular can get chickenpox through contact with shingles. Shingles is just not contagious for those who have already had chickenpox, because they already carry the pathogen in their body.

How long does ramsay hunt syndrome recovery take?

The rash and pain usually subside within three to five weeks, but about one in five patients develop a painful condition called postherpetic neuralgia, the most common complication of shingles, which is often difficult to manage.

What about complications and facial paralysis?

Failure to treat herpes zoster oticus can result in serious complications. This can result in hearing loss or even deafness. Peripheral fascial palsy (nerve paralysis) can also occur as a complication of herpes zoster oticus. This means that the muscles responsible for facial expressions are weak or completely paralyzed on one side. In about 80% of the cases, however, healing occurs.

Further complications can arise from the involvement of various nerves. If the vestibulochochlear nerve (inner ear) is affected, hearing loss or deafness can occur, but also ringing in ears , vertigo and nausea can occur. If other cranial nerves are affected, it can have effects such as:

Hiccups or difficulty swallowing
Mood disorders
Injury to the conjunctiva, cornea, or optic nerve
Meningitis

Does ramsay hunt syndrome recur?

Ramsay Hunt Syndrome is typically a one-time condition, but recurrence is possible, especially in people with weakened immune systems. The virus that causes it (varicella-zoster) remains in the body for life and can reactivate under certain conditions, though repeat episodes are uncommon

Can ramsay hunt syndrome affect the brain?

In rare cases, Ramsay Hunt Syndrome can lead to complications that affect the brain and central nervous system. If the virus spreads beyond the facial nerve, it may cause inflammation in nearby areas such as the brainstem, potentially leading to more serious neurological symptoms. Prompt diagnosis and treatment are essential to reduce this risk.

Can stress cause ramsay hunt syndrome?

Stress doesn’t directly cause Ramsay Hunt Syndrome, but it can weaken the immune system. This makes it easier for the varicella-zoster virus to reactivate, which can trigger the condition. Managing stress and maintaining a healthy immune system may help reduce the risk.

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