Ramsay Hunt syndrome, also known as herpes zoster oticus, is a rare type of shingles that can affect people years after they’ve had the chickenpox virus. It affects part of the facial nerve, causing inflammation in a small area called the geniculate ganglion. Although the condition usually goes away on its own, treatment focuses on shortening how long it lasts, relieving pain, and preventing complications.
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What Is Ramsay Hunt Syndrome?
Ramsay Hunt syndrome (RHS), or herpes zoster oticus, is a sudden-onset, complete facial nerve paralysis. It is often associated with hearing loss, vertigo, or a characteristic rash involving the face or ear.
A rare peripheral facial palsy, RHS can affect people years after they’ve had the varicella-zoster (V-Z) virus, the virus that causes chickenpox and, later, shingles. RHS occurs when the V-Z virus, which has been lying dormant in the cell bodies of the facial nerve since the initial infection, is reactivated during a shingles outbreak.
RHS affects part of the seventh cranial nerve, also called the facial nerve, causing inflammation in a small area called the geniculate ganglion. Because the eighth cranial nerve, also known as the vestibulocochlear or auditory nerve, lies next to the sensory ganglion of the facial nerve within the facial canal, both nerves are usually affected.
Why does the virus reactivate?
There are times when our bodies are less able to fight off infections. When the immune system is weakened, it becomes vulnerable to reactivation of the chickenpox virus. If the virus is lying dormant in facial nerve cells, it could be triggered to reactivate in the form of shingles and RHS.
The biggest trigger for reactivation of the V-Z virus is stress. Studies have shown that stress can weaken the immune system and that people are more likely to suffer from infections if they are under stress. That’s why an outbreak of shingles often can be linked to periods of extreme stress.
What causes Ramsay Hunt syndrome?
Ramsay Hunt syndrome is a condition that can occur after a person has had chickenpox earlier in life. After they are infected, the virus stays dormant or inactive in their body. Years down the road, the virus can reactivate, resulting in shingles or herpes zoster.
Shingles causes pain and a blister-like rash called a dermatome that follows the path of the affected nerve, usually in one specific area of the skin. The location of the rash and symptoms depend on which nerve is affected. When it involves the seventh cranial or facial nerve, it may be Ramsay Hunt syndrome.
Ramsay Hunt syndrome risk factors
Risk factors for the reactivation of the dormant V-Z virus that causes shingles and RHS include:
- Age 60 and up.
- Being under emotional stress.
- Having an upper respiratory infection.
- Having cancer.
- Having chronic renal failure.
- Having diabetes.
- Smoking.
- Undergoing immunosuppressive therapy.
Complications of Ramsay Hunt syndrome
Untreated RHS can cause complications such as long-term facial weakness, paralysis, or uncontrolled movements.
Other complications can include:
- Eye problems — Dry eye, irritation, damage to the cornea, or vision loss if your ability to blink or close your eye is affected.
- Postherpetic neuralgia — Long-term pain from nerves damaged by shingles.
- Synkinesis — A condition in which voluntary muscle movements trigger other muscles to move involuntarily at the same time.
You can lessen the severity of your symptoms and help prevent complications by seeking treatment within the first few days of developing shingles symptoms.
Is Ramsay Hunt syndrome contagious?
RHS does not spread from person to person. However, if you have RHS and are around children or other people who have not had chickenpox, they can get chickenpox if they come in contact with your blisters.
How long does Ramsay Hunt syndrome last?
It usually takes about a week for the rash from RHS to scab over. At that point, you can no longer pass the V-Z virus on to others.
However, related symptoms might take longer to go away. Most people experience gradual improvements in their symptoms over several months to a year. Nearly everyone who experiences RHS will recover fully or almost fully. Even if symptoms don’t resolve completely, treatments can help make them more manageable.
How common is Ramsay Hunt syndrome?
Ramsay Hunt syndrome is a rare condition that affects approximately 5 people per 100,000 people per year.
What Are the Signs and Symptoms of Ramsay Hunt Syndrome?
Doctors diagnose Ramsay Hunt syndrome based on three key symptoms:
- Fluid-filled blisters near the ear or inside the ear canal.
- Hearing loss or vertigo.
- Weakness or paralysis on one side of the face.
Although Ramsay Hunt syndrome shows up as these three symptoms, not everyone has all three symptoms. Some people experience facial paralysis before the rash appears, while others may never develop a rash at all.
When there’s no rash, the main symptoms are hearing loss/vertigo and facial weakness. This form is called zoster sine herpete, and it can be hard to distinguish from Bell’s palsy, another condition that causes facial paralysis.
If a rash does appear, it may look like blisters or red bumps and can affect the same side of the face, scalp, roof of the mouth, or tongue.
Other symptoms can include:
- Altered taste on the affected half of the tongue.
- Difficulty closing the eye or blinking on the affected side.
- Difficulty eating, drinking, and speaking due to weakness in the lip and cheek.
- Dizziness or vertigo.
- Ear, face, or head pain.
- Facial muscle weakness or drooping on one side.
- Hearing loss on the affected side.
- Loss of facial expression on the affected side.
- Painful rash or blisters around the ear, scalp, hairline, or inside the mouth.
- Tinnitus or ringing in the ear on the affected side.
When should I see a doctor about my Ramsay Hunt syndrome symptoms?
Patients experiencing new-onset Ramsay Hunt syndrome should seek medical attention from a primary care provider, an emergency care provider, or an otolaryngologist (ear, nose, and throat specialist).
How Do You Diagnose Ramsay Hunt Syndrome?
Diagnosis of Ramsay Hunt syndrome is made based on your health history, clinical findings, and the results of a neurological examination. This condition is often missed, misdiagnosed, or diagnosed late, which can lead to more serious long-term problems.
Other neurological conditions that can be mistaken for RHS include:
- Acoustic neuroma — A benign, slow-growing tumor in the Schwann cells of the myelin sheath of the eighth cranial nerve, the vestibulocochlear or acoustic nerve. This can be excluded with MRI.
- Bell's palsy — Sudden-onset facial palsy that can be differentiated from RHS by the absence of severe pain in the mastoid area, which is a symptom of RHS, and the lack of vesicles and other cranial nerve involvement.
- Postherpetic neuralgia — The most common nerve pain condition that can occur after shingles, especially in people aged 60 and up. It is not associated with facial palsy.
- Temporomandibular joint (TMJ) disorders — Jaw pain not accompanied by facial palsy.
- Trigeminal neuralgia — A facial pain syndrome not accompanied by facial palsy.
How Do You Treat Ramsay Hunt Syndrome?
Although symptoms usually subside on their own, Ramsay Hunt syndrome treatment focuses on reducing the duration of symptoms, alleviating pain, and preventing complications.
The recommended treatment includes a course of high-dose steroids and antiviral medication.
Treatment for RHS-related synkinesis
A frequent complication of Ramsay Hunt syndrome, synkinesis is a condition in which voluntary muscle movements trigger involuntary movements in other muscles at the same time.
Treatment options for synkinesis with Ramsay Hunt syndrome include:
Botulinum toxin (Botox®)
Botox may be used on synkinetic muscles alone or in combination with physical therapy/facial training therapy. Results typically last three to four months.
Physical therapy/facial training therapy
This type of facial synkinesis treatment focuses on retraining the coordination of appropriate facial muscle movements. Other related treatments include mirror therapy, relaxation exercises, and self-massage.
Surgery
Surgery is a second- or third-line option reserved for cases of severe synkinesis that have failed to respond to physical therapy and Botox.
Surgeries used may include:
- Gracilis free muscle transfer.
- Selective myectomy.
- Selective neurectomy.
How effective is treatment?
Up to 70% of patients return to baseline facial function after having Ramsay Hunt syndrome. A typical timeline ranges from one week to six months after onset. Up to 30% of patients may experience long-term synkinesis.
Why Choose UPMC for Ramsay Hunt Syndrome Care?
Some health care providers might miss a Ramsay Hunt syndrome diagnosis. At UPMC, you will be evaluated by a specialist experienced in treating Ramsay Hunt syndrome.
You may be given a hearing test or have a photo series taken to document your facial function over time. Your case will be carefully reviewed to ensure adequate treatment and rule out other causes of facial paralysis.
