Ramsay Hunt Syndrome is a varicella-zoster virus infection of your facial nerve. This causes one sided facial paralysis and a painful rash in and around your ear. The close proximity to your vestibulocochlear nerve leads to hearing loss, tinnitus and vertigo. It is named after American neurologist, Dr James Ramsay Hunt, who first described it in 1907, but brought to public attention in 2022 when Justin Bieber was diagnosed. Ramsay Hunt Syndrome treatment includes physiotherapy, not only for strengthening of your facial muscles, but addressing your balance and stability because of the asymmetry caused by the virus to your vestibular system.
Ramsay Hunt Syndrome has a prevalence of 5 out of 100 000 people and is the second most common cause of peripheral, non traumatic facial paralysis. 100% of people suffering from Ramsay Hunt Syndrome will have facial paralysis, while only 50% will experience hearing loss and vertigo.
The nerves that supply your face & ear
Your nervous system is like your electrical supply. It is a uniquely complex designed system that not only allows such precise movement of a surgeon saving a brain or a pianist playing Chopin, but also receiving information from your environment, like a ballerina on point doing pirouettes. Your facial and vestibulocochlear nerves are team members of this complex system.
Facial nerve CN VII
You have two facial nerves, one for each side. Your facial nerve is the seventh cranial nerve (CN VII) and communicates directly with your brain. It begins in your brain stem and runs through the facial canal in your temporal bone. The facial canal is 3 cm, the longest bony canal in your body, and Z-shaped. Your facial nerve and stylomastoid artery both exit through a tiny hole, the stylomastoid foramen. This foramen is about 0.7 mm wide.
After exiting through the stylomastoid foramen your facial nerve divides into 5 branches so supply your facial muscles, part of your tongue, glands of your mouth and eye and sensation from your ear.
Vestibulocochlear nerve CV VIII
Your vestibulocochlear nerves, one on either side, send information from your vestibular system and cochlea to your brain about balance and sound. Deep inside your temporal bone lies your vestibular apparatus, your gyroscope, and cochlea, that convert vibration into impulses that is registered as sound. You have five organs that sense movement and body position on each side, three semicircular canals (SCC) and two otoliths that communicate with your brain via your vestibulocochlear nerve.
What do these nerves do?
Facial nerve CN VII
Your facial nerve is a mixed nerve, meaning it sends and receives information. 70% of your facial nerve’s fibres are myelinated and supply your 30 facial muscles, while the other 30% have mixed sensory fibres. This sensory part of your facial nerve is responsible for the front 2/3 of taste sensation from your tongue, like when you lick ice cream, and the lubrication function of your lacrimal, submandibular and sublingual glands. These glands make tears to lubricate your eye and saliva to help digest the food you eat.
We take for granted how subtle changes in facial expression are part of communication. Remember when we were all wearing masks during the pandemic? When you cannot see someone’s face you can’t read the change of expression when they talk of a painful experience, hint at a sarcastic pun or busy taking you for a fool with a silly joke.
On a more practical note, you need your facial muscles to talk, chew, contain your sip of coffee to the inside of your mouth, whistle and close your eyes. This is very important to protect your eye from flying insects and keep your eye lubricated and cornea protected.
Vestibulocochlear nerve CV VIII
Balance is dependent on your eyes, body and ears, like a tripod. How the brain interprets the sensory information it receives from sight, proprioception and the vestibular apparatus determines if you can pirouette, land steady from a back flip or navigate the supermarket aisles without bumping into other customers.
Your semicircular canals detect changes in velocity, while the otoliths detect changes in linear acceleration and gravity. Your utricle relays the change of linear speed, like the movement in a car. While your saccule detects gravitational change in an elevator.
Your cochlea analysis different frequencies, “translate” frequencies into nerve impulses that your brain then interprets as sound.
What happens with Ramsay Hunt Syndrome?
With a varicella-zoster infection of your facial nerve your immune system will attempt to attack the virus. This causes inflammation, which is occupying space through those tiny canals and can lead to pressure on the nerve and vasculature. Poor blood supply then worsens the nerve damage. That is why it is so crucial to intervene quickly. Your vestibulocohlear nerve is in close proximity to your facial nerve, about 1-2mm apart, in the cerebellopontine angle, making it susceptible to the virus’s spread. The virus can also spread via your blood vessels.
Your nerves work pretty much like any electrical appliance. If electricity can’t be sent to your kettle, it cannot boil the water. With Ramsay Hunt Syndrome, this is like having a cut in the kettle’s cord. Electricity or nerve impulses cannot be transported to and from your facial muscles, glands, cochlea, and vestibular apparatus. That means your muscles cannot move, glands cannot secrete, cochlea doesn’t hear and vestibular system can’t balance.
Cause of Ramsay Hunt Syndrome
Ramsay Hunt Syndrome is caused by the same virus that is responsible for chickenpox and shingles, varicella-zoster virus. Even if you had chickenpox as a child, the virus can remain dormant in your body. It may be reactivated years later. If this reactivation happens in your facial nerve, the resulting symptoms are grouped together and referred to as Ramsay Hunt Syndrome.
If you have a weakened immune system, you have a higher risk of developing Ramsay Hunt Syndrome. This may be because of your age or immune suppressant treatments. The following poses a greater risk of developing Ramsay Hunt Syndrome:
- over 60 years of age
- pregnant women
- infants
- biological treatment
- HIV infection
- oncology treatment
Corticosteroid medication is most effective if started within 72 hours of onset of symptoms.
How severe is my Ramsay Hunt Syndrome?
Facial paralysis, hearing loss, vertigo and pain will affect anybody’s quality of life. Young, healthy individuals tend to recover quicker than older patients with other comorbidities, like diabetes, vascular insufficiencies or people who smoke.
In 1943 Seddon classified nerve injuries into three groups:
I. Neuropraxia
The physical nerve remains intact, both the power cable and surrounding insolation, but the nerve’s ability to transport impulses is temporarily damaged. Where infection, inflammation and compression caused your Ramsay Hunt Syndrome, you can expect to recover within a few months.
II. Axonotmesis
The connective tissue surrounding the nerve (insolation) remains intact, but the axon (power cable) is damaged. This type of nerve damage is uncommon in Ramsay Hunt Syndrome, but trauma, tumours and fractures may cause axonal damage to your facial nerve. You can expect a longer recovery of 5 – 6 months, with your eyes and mouth regaining movement first and your chin and cheeks later.
III. Neurotmesis
Both surrounding connective tissue and axon are damaged or severed. This can happen as a result of trauma or surgical complications. Lengthy and partial recovery is expected. Nerves can regenerate about 1 mm a day under ideal circumstances.
Neuropraxia is what causes Ramsay Hunt symptoms. Timely intervention is therefor very important to minimise nerve damage. Unfortunately, some people have a partial recovery only, with persisting tinnitus, balance problems and facial weakness.
Sudden onset facial paralysis of the lower half of the face, that doesn’t involve the eye, indicated upper motor neuron injury like stroke or tumor.
SEEK IMMEDIATE MEDICAL CARE!
Diagnosing Ramsay Hunt Syndrome
Sinister, potentially life threatening, pathology needs to be ruled out if you have vascular risk factors. Your doctor will consider your history when deciding what tests or imaging to send you for. Your unique situation and comorbidities will determine the workup you receive.
Physiotherapy diagnosis
Our physiotherapists can then determine the extent of your facial palsy and give you an approximate time line for your recovery, while starting your rehabilitation. We can evaluate your balance, vestibular mismatch, facial sensation and muscle strength to customise your treatment program and home exercises. This will reduce your fall risk, facilitate vestibular recovery and hasten facial muscle function.
Blood tests
Blood tests that include complete blood count, erythrocyte sedimentation rate, thyroid hormones, liver enzymes and viral/bacterial load can be done to exclude other causes of your facial palsy and hearing loss. This will be done routinely in the ER by your doctor.
Audiogram/Caloric
Ramsay Hunt Syndrome causes hearing loss on your affected side. This can be objectively assessed by an audiologist to determine severity and frequencies lost/affected. Caloric testing can reveal vestibular deficit and can be considered.
If you need a hearing test, your physio will refer you.
MRI/CT
A brain MRI/CT scan can view all of the structures of your head, including vasculature and nerves. This will be done to exclude a cerebrovascular attack (CVA) if you went to the ER with your facial palsy and hearing loss and have certain risk factors for a stroke.
Why am I still dizzy?
Complete recovery from Ramsay Hunt Syndrome without intervention is uncommon. Young, healthy individuals, without bad habits like smoking, who can rely on fantastic circulation have a greater chance of making a speedy recovery without physiotherapy intervention. Older individuals with co-morbidities need extra assistance to hasten recovery.
It is not uncommon to be more concerned about your facial paralysis during your initial treatment, there is no vanity when it comes to looking like yourself on photos. But if you neglected your balance and vestibular recovery, your unilateral vestibular hypofunction remains. This means that after your facial and vestibulocochlear nerves have healed, your brain still perceives faulty information from the vestibular system, because it was never trained in the integration of information from the left and right sides. You may cope well with “easy” single focus tasks, like walking down your hallway. But you may have difficulty with tasks that require multi-sensory integration, like walking down the aisle of the grocery store, with the announcements blasting, other shoppers and the list of what you need. You need to retrain this integration we all take for granted with adaptation and habituation exercises.
Problems we see when patients come to us with Ramsay Hunt Syndrome
Waiting too long
Time is of the essence! The longer you wait, the greater the chance of delayed recovery, because the nerve is losing out on crucial circulation while you weigh the odds of a spontaneous recovery. Go to the ER, rule out any dangerous pathology and get your cortisone and antiviral prescription. Make an appointment to start your Ramsay Hunt treatment as soon as possible. We can get started while you are on oral medication.
Dry eye
It is crucial to assist eye closure regularly throughout your waking day. During the night you can tape your eye shut or make use of an eye patch. Your GP/pharmacist can recommend eye drops to use. If your eye isn’t lubricated you can develop a corneal ulcer that causes loss of vision.
Misdiagnosis
If your Ramsay Hunt syndrome is mistakenly diagnosed as Bell’s Palsy you will not address your unilateral vestibular hypofunction, which can lead to poor balance and increased fall risk in the future. If you experienced vertigo and dizziness with your facial palsy, get it checked out by your doctor.
Not completing your rehabilitation
Your Ramsay Hunt treatment is complete when you feel safe navigating unstable surfaces and obstacles and your facial expressions look and feel symmetrical. Stopping before the end can cause synkinesis and an asymmetrical apprearance.
Physiotherapy treatment for Ramsay Hunt Syndrome
Our physiotherapists are experts in rehabilitation and understand physiology. We have studied how a viral infection causes a cascade of inflammation, how nerves react to pressure and what effects that will have on your everyday life. Physiotherapy enhances function while promoting healing. Neural plasticity excites us, muscles are our friends, in that we understand how movement translates to facial expressions. Balance, control of movement and endurance are the commodities we deal with daily. There are no shortcuts to healing. It’s important to do the right exercise at the right time as your facial palsy and balance training treatment progresses. We will guide you every step of the way.
Phases of Ramsay Hunt treatment
1st Phase of Ramsay Hunt treatment: Fall risk awareness & facilitate a flicker (weeks 0 – 3)
Our first aim is to get the correct diagnosis, establish when you started oral medication and make sure you know how to take care of your eye. If you have more than a 2 line difference on your dynamic visual acuity, you should avoid driving for your own and other’s safety.
We will start your balance and vestibular exercises in a position (sitting/standing) and at a speed that you feel challenges, but comfortable. We use electrical muscle stimulation (EMS) to get a muscle flicker while facilitating some facial expressions. Laser can be used for pain and inflammation. Your physiotherapist will teach you self soft tissue mobilisation and taping techniques for home use.
2nd Phase of Ramsay Hunt treatment: Adaptation & facial strength through range (weeks 4 – 6)
As your brain adapts to your current vestibular input, we can make your exercises more challenging, by changing distance, background, speed and surface you are doing your exercise drills. We can add moving targets and cognitive dual tasking to access your brains neural plasticity capacity.
Once you have regained some movement of your facial muscles we also aim to strengthen that movement through the entire range. You can expect some hands on assists while you are doing your facial expressions with the EMS. Full, wide smiles, snarls, pouts and eye closure are our priority.
3rd Phase of Ramsay Hunt treatment: Habituation & Eccentric Control (weeks 7 – 8)
We can now add optokinetics to your vestibular recovery with moving visual targets on moving, complicated backgrounds. Functional balance drills and moving against gravity, think yoga/pilates type head on body movements, will also be part of your rehabilitation.
The EMS has been your friend until now. From this stage, you can expect to work against the machine, similar to Body 20 and Bodytec training. You should be more comfortable eating in public as you regain mouth closure.
4th Phase of Ramsay Hunt treatment: Isolated Specificity (weeks 9 – 12)
Walking with head turns, navigating obstacles, doing 360 degree turns, should feel comfortable at this stage. Now we work on the subtle nuances as you gain isolated control of your facial expressions. No more forcing your eyes shut, please wink. Whistling and blowing bubblegum bubbles and watching laugh out load sitcoms is part of your rehabilitation now.
5th Phase of Ramsay Hunt treatment: Clearance and synkinesis watch (months 3 – 6)
You should feel safe to drive, run up stairs (if that was part of your training before) and going dancing again. Your pre- and post-rehabilitation selfies should be difficult to distinguish. Even though we won’t be seeing each other often, keep an eye on those involuntary movements to ensure you don’t develop synkinesis.
Healing time for Ramsay Hunt Syndrome
When diagnosed correctly and a treatment protocol of corticosteroid and antiviral medication started immediately, recovery can take 8 – 12 weeks. The duration of nerve compression greatly influences your overall recovery period. Your general health and any comorbidities, like diabetes and vascular problems, will also influence the duration of your recovery. You can still expect changes for up to a year after your Ramsay Hunt Syndrome diagnosis.
Unfortunately, persistent symptoms can develop in some people. Delay in care, lack of early movement and a history of anxiety and depression increases your risk of developing chronic symptoms. This can include motion sensitivity, dizziness with exertion, vertigo or facial synkinesis.
Other forms of medical treatment for Ramsay Hunt Syndrome
- Your doctor (GP) will probably prescribe corticosteroids and antiviral medication. This is best done within 12 hours of symptom onset.
- Eye care is very important while you recover from Ramsay Hunt Syndrome. You need to protect your eye from drying out with drops, salve and and/or an eye patch while sleeping. Ask your doctor/pharmacist about different options to protect your eye. Damage to your cornea can lead to irreversible loss of sight.
- Hearing assessment to determine the extend of hearing loss and options, like an amplifier, during your recovery, can be done by an audiologist.
- Supplementation of vitamins and minerals can be considered during your recovery.
Is surgery an option for Ramsay Hunt Syndrome?
Surgery is not the standard of care for Ramsay Hunt Syndrome, but can be considered if facial paralysis is not improved with rehabilitation exercises. Facial nerve decompression can be done to relieve pressure from your facial nerve if compression is believed to be causing your facial paralysis. Facial reanimation surgery, with nerve grafts or muscle transfers, can be considered for aesthetic purposes and will look more symmetrical, but not necessarily restore your facial functions, like eating without leaking.
Even a surgical intervention will need to be followed up with rehabilitation exercises and sensory desensitising techniques to integrate and strengthen your facial muscles.
What else could it be?
Vertigo and balance dysfunction, caused by a viral infection of the vestibulocochlear nerve (CN VIII), may also cause facial pain and hearing loss, but no paralysis of your facial muscles.
One sided facial paralysis without any hearing loss or balance problems.
- Cerebrovascular Accident (CVA/stroke)
Hemorrhagic or ischeamic incidents can also cause facial palsy, but will be accompanied by sudden, sever headache, inability to talk, confusion, loss of balance and weakness in one or more limbs.
Cervicogenic headaches can cause facial and scalp pain, without paralysis.
Whiplash or other injury to your high cervical spine can cause referred pain to your face and jaw.
Ramsay Hunt Syndrome is also known as
- Herpes zoster oticus
- Facial Shingles
- Ramsay Hunt Syndrome Type 2
- Facial nerve palsy due to herpes zoster
- Geniculate ganglion herpes zoster
- Zoster Sine Herpete
