What is Ramsay Hunt syndrome?
Ramsay Hunt syndrome (RHS) is a complication of shingles. It is the name given to describe the symptoms of a shingles infection affecting the facial nerve. Shingles is caused by the same virus that causes chickenpox (varicella zoster virus, or VZV). As a result of this infection, the facial nerve becomes inflamed and irritated.
If you develop RHS, you will have had chickenpox as a child but once the spots heal and you recover the virus continues to live in the nerves that it has infected by the virus. It is harmless unless it is reactivated and should this happen new symptoms will appear. This cluster of symptoms is called RHS.
What causes the shingles virus (VZV) to reactivate?
- At times our immune system becomes depressed and is less able to fight off infection. The body then becomes vulnerable to reactivation of the chickenpox virus.
- Stress is often a trigger. Many studies have shown that stress can weaken the immune system, and that people under significant stress are more likely to suffer from infections than those who are not. For this reason, it is believed that stress can be linked to outbreaks of shingles, and thus RHS could result.
What are the symptoms of Ramsay Hunt syndrome?
- A rash or blisters in or around the ear, scalp or hair line. The blisters may also appear inside the mouth.
- The rash/blisters are often painful with a generalised sensation of burning over the affected area.
- Weakness on the affected side of your face which causes the facial muscles to droop.
- Difficulty closing the eye or blinking on the affected side.
- Altered taste on the affected half of the tongue.
- Loss of facial expression on the affected side.
- Difficulty eating, drinking and speaking as a result of weakness in the lip and cheek on the affected side.
- Ear, face or head pain.
- Hearing loss on the affected side
- Tinnitis (ringing in the ear) on the affected side.
- In some cases the throat may also be affected directly or indirectly via the vagus nerve.
You may not have all the symptoms or some symptoms may develop later than others. A small number of people may not have a rash. This is called Zoster sine herpete (ZSH). Alert your doctor if you have chronic facial pain and have been diagnosed with Bell’s palsy.
Can you catch Ramsay Hunt syndrome (RHS) from an infected person?
- You cannot catch RHS from an infected person.
- People with no immunity to chickenpox can develop chickenpox from contact with the open rash or blister on a person who has RHS.
How is Ramsay Hunt syndrome diagnosed?
A diagnosis is often difficult because symptoms vary from person to person and symptoms of RHS are similar to other causes of facial weakness. Patients with RHS may experience severe pain which often differentiates it from other causes of sudden onset facial paralysis. The following examinations and investigations may be used in the diagnosis of RHS:
- Thorough history taking by your GP or doctor.
- Examination of the facial nerve function (such as eye closure).
- A full physical examination including the ear.
- Blood tests to measure the presence of the antibodies to the varicella zoster virus responsible for causing RHS.
- Hearing tests.
- Magnetic Resonance Imaging (MRI) to identify areas of inflammation along the track of the facial nerve and exclude any other cause of your symptoms.
- Nerve conduction studies can assess facial nerve function. It is not diagnostic but indicates the degree of facial nerve damage.
- Electromyography can also show if the facial nerve is damaged but again this cannot give a specific diagnosis or reason for the damage.
What is the treatment for Ramsay Hunt syndrome?
- Prompt treatment (ideally within three days of onset of symptoms) with antiviral medication (e.g. acyclovir).
- Prompt treatment with a short-course of high dose steroids is also recommended.
- Eye lubrication for the unblinking eye (preservative-free if used more than four times per day).
Find out more about steroids and antivirals.
What sort of recovery can be expected?
- If antiviral treatment is given within 72 hours of developing symptoms approximately 70% of people will experience a virtually full recovery.
- If antiviral medication is not given within this time frame then the likelihood of making a full recovery reduces to 50%.
- The more severe the damage, the longer it will take to recover, and the lower the chance that you will completely regain normal function.
- If damage to the nerve is mild then recovery should take place within a few weeks.
- Recovery follows a similar pattern to Bell’s palsy. However symptoms in RHS tend to be more severe than with Bell’s palsy.
The video below has tips how to manage your facial palsy while your face is floppy. Please note this is a series of 7 short videos in one.
If your eye still does not close properly after 2-3 weeks you should be referred to ophthalmology. If you are struggling to care for your eye with tape and eye drops then ask your GP to make an earlier referral.
If the paralysis shows no sign of improvement after one month you should be referred to a facial palsy specialist, this could be a plastic surgeon, neurologist or ENT consultant. Ideally you should be referred to a specialist facial therapist as well. Studies show that botulinum toxin injections can help with residual facial pain, spasms and involuntary facial movements.
Mnemonic for Health Professionals
A quick reference guide aimed at health professionals for differentiating Ramsay Hunt syndrome/Varicella Zoster Virus/Zoster Sine Herpete from Bell’s palsy.
Treatment and prognosis of facial palsy on Ramsay Hunt syndrome: Results based on a review of the literature.
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Last reviewed: 13-03-2023 || Next review due: 01-09-2023