Matt Carney, a stage manager who lives in Bedford, was at work in 2017 when he experienced the first of a series of mysterious symptoms. “We’d just started a performance of Hairspray, and almost with the first beat of the overture an intense earache started – like no earache I’ve ever had before.” Painkillers had no effect and when he was driving home later, his face felt strange in a way he couldn’t put his finger on. Within two days, he says, “it was completely paralysed on the right side”.
Anyone who has seen the news recently might correctly guess that Carney’s condition was Ramsay Hunt syndrome – a complication from the chickenpox virus – thanks to Justin Bieber’s post on Instagram announcing that he has it. In a short video, Bieber demonstrated that he can only smile, nostril-twitch and blink on one side of his face. But before Bieber’s post, the syndrome was little known – it isn’t even mentioned on the NHS website. It is commonly misdiagnosed as Bell’s palsy, which also manifests as facial paralysis on one side, meaning that patients miss out on rapid treatment with antiviral drugs, which, if administered within the first 72 hours, could save them from chronic, life-changing symptoms .
For Carney, now 33, the worst part was being unable to blink. “I had to put drops in my eye every 20 minutes to stop it drying out, and to manually blink by pulling my eyelid down with my fingers. I was having to tape it closed at night to be able to sleep. Just walking outside, even on a still summer’s day, there was enough of a breeze to dry my eyes out within seconds.”
After an initial Bell’s palsy diagnosis, it wasn’t until day four that he had an attack of what doctors later suspected was vertigo, and went to hospital, where he was finally diagnosed with Ramsay Hunt syndrome – too late for the antiviral meds.
Charles Nduka, consultant plastic surgeon at Queen Victoria hospital in East Grinstead, West Sussex, and Guy’s and St Thomas’ hospital in London, founded the charity Facial Palsy UK in 2012 to raise awareness of the condition whose long-term effects his team see daily , but to little avail. While it’s hard to get accurate data because misdiagnosis is so common – the reported incidence of Bell’s palsy is 20-30 per 100,000, for Ramsay Hunt syndrome it is around five cases per 100,000 – he estimates that in the UK there are about 25,000 new cases of Ramsay Hunt syndrome per year, and maybe a third of those affected will be left with chronic symptoms. Ironically, he says, he even developed the condition himself during lockdown.
It is caused by the varicella zoster virus, which, after a bout of chickenpox, becomes dormant in the body – in the case of Ramsay Hunt syndrome, in nerve cells. It is, essentially, affecting a shingles infection the facial nerve after the dormant virus is activated, “when your immune system is down,” says Nduka. “Patients who we see will have some preceding physical or emotional stress, such as cancer, chemotherapy, immunotherapy, moving house or stress in their workplace.”
Nduka had been working hard during the pandemic, he says. “I was giving a talk on facial palsy when my face started twitching, and I had this horrible sensation on the side of my tongue and an awful taste, which is one of the early symptoms – altered taste because the facial nerve also supplies the front part of the tongue. I knew what it was and was able to get treatment early on, and managed to abort the onset.” He had another minor flare-up a year later, but again, he says, “I was able to hit it early.”
Unlike the sudden onset of a stroke, the facial paralysis with a palsy gradually develops. The key symptoms that make Ramsay Hunt syndrome stand out from Bell’s palsy are, Nduka says, ear, face or head pain, a rash or blisters – often painful – in or around the ear, scalp, hairline or inside the mouth, altered taste on half of the tongue, hearing loss or tinnitus on the affected side and dizziness or vertigo. Sometimes, however, the rash may be hidden internally, and the facial paralysis appears first, meaning that, even if the doctor is familiar with the syndrome, they might disregard that diagnosis. This is why Ndukas treating any new facial palsy with antivirals – which he says are cheap and have few side-effects – to be on the safe side.
Both forms of sudden-onset facial palsy – Bell’s and Ramsay Hunt – are treated with steroids, too. “The facial nerve runs through a bone tunnel in the skull, so if it gets inflamed there is no room for it to swell, so it will cut off its own blood supply and stop working,” says Nduka. “The steroids are there to reduce the inflammation and therefore reduce swelling and allow the nerve to recover earlier.” Early treatment with antivirals and steroids, he says, increases recovery rates in cases of Ramsay Hunt syndrome from about 50% to about 70%. “If patients do not receive prompt treatment with a combination of steroids and antivirals,” he says, “it’s a coin flip as to whether they will recover fully.” Nduka says he really wants doctors to be aware of the condition.
It’s nine years since Deborah Lack’s Ramsay Hunt syndrome was misdiagnosed as Bell’s palsy. Now 42 and based in Hertfordshire, she still has tinnitus and at her most recent checkup – the first in four years, thanks to Covid – found no improvements in her facial movement. Other than give her Botox to relieve tightness in her facial muscles, she says, “they said to me: ‘Nothing else can be done for you.’ They want to start the Botox again, to see where we are in another six months, and the next step would be to have reanimation surgery: they take some nerves out of one area of your body and then put them in your face to try to get the eye and the smile going again. Obviously, there’s no guarantees.”
Lack used to run a dance school, but she gave it up after she developed the condition. “I work with children, and children are so honest,” she says. “What’s wrong with your face? Why have you got a wonky face? Why is one eye smaller than the other? There’s only so much you can take.”
These days, she says, “at rest, if I wasn’t tired or stressed out, then you probably wouldn’t notice. I’ve even learned how to pluck my eyebrows in a different way to make sure that my eyebrows are level. I can’t even pucker my lips.”
This makes everyday things like spitting after brushing her teeth, or drinking from sports bottles, impossible. “You can dribble as well, and if I’m out to eat I often put my hand in front of my mouth because I’m aware that one side can be open,” she says. For family photographs, she always stands at an angle to hide her affected side.
Nduka says the condition can lead to blindness in the affected eye, and the long-term symptoms can have serious mental health consequences. “Fundamentally, your face is what makes you human,” he says. “It allows you to interact with strangers, which is unique to humans.”
Facial rehabilitation can include a combination of approaches, he says, “such as neuromuscular retraining, biofeedback training – learning how to sense the face. It’s a long process and it requires a lot of time and patience.” Patients with weakness on one side may experience exaggerated movements on the other as it tries to compensate for the lack of movement.
If therapy hasn’t been successful enough, surgery is an option – for instance weighting the eyelid with platinum to help it close properly, “releasing” overactive muscles, or, “if the smile muscles aren’t quite working,” Nduka says, “we can apply a nerve graft to those muscles..”
Carney considers himself lucky that he got all his movement back. After two or three months, he recalls, “it started to come back on its own, but I then realised that I had some strange movements and stiffness of the muscles.” He was referred to a specialist physio who helped, but, he says, “if I close my left eye, my right eyebrow goes up, and if I smile – a really big smile – then my right eye closes up a bit. And the bizarre one is, if I cough, my right eye cries, which was an absolute pain when I had Covid.”
He still has tinnitus, which comes and goes, but you probably wouldn’t notice he’s had the condition now. “It has taken a few years to get to that point,” he says. “There’s the chance that if I’d had the antivirals early on, I might have avoided the paralysis altogether.” It’s just unfortunate that we’ve had to wait for Justin Bieber, he says, “to be able to bring it to people’s attention”.