A British girl has had her hearing restored after becoming the first person worldwide to take part in a pioneering new gene therapy trial.
Opal Sandy was born totally deaf due to auditory neuropathy, which disrupts nerve impulses from the inner ear to the brain.
But the 18-month-old’s hearing is now almost normal – and could improve further – after having the one-time treatment.
Opal was treated at Addenbrooke’s Hospital in Cambridge and the head of the trial, Professor Manohar Bance, said results were “better than I hoped or expected” and he hopes medics might be able to cure others with this type of deafness.
“We have results from (Opal) which are very spectacular – so close to normal hearing restoration. So we do hope it could be a potential cure,” he said.
Image: Opal (second left) with her parents and sister Nora, who has the same condition. Pic: PA
Auditory neuropathy can be caused by a fault in the OTOF gene, which makes a protein called otoferlin and enables cells in the ear to communicate with the hearing nerve.
Opal, from Oxfordshire, had an infusion of the working gene into her right ear during surgery in September – the treatment developed by biotech firm Regeneron.
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Her parents noticed changes in only four weeks and the improvements were especially noticeable 24 weeks later.
Jo and James Sandy, both 33, said they were “gobsmacked” when she responded to sound tests at home without her cochlear implant, the usual way to treat the condition.
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Image: Opal had her operation in September. Pic: Cambridge University NHS Trust
“I thought it was a fluke or like a change in light or something that had caught her eye, but I repeated it a few times,” said Mrs Sandy.
They say she now enjoys the sound of slamming her cutlery on the table and playing with toy drums and wooden blocks.
“We were told she had near normal hearing last time,” she added. “I think they got responses at sort of 25 to 30 decibels.
“I think normal hearing is classed at 20 decibels, so she’s not far off. Before, she had no hearing whatsoever.”
Opal’s surgery was very similar to fitting a cochlear implant, according to Prof Bance.
He said the inner ear (cochlea) was opened and the treatment infused using a catheter over 16 minutes.
“We have to make a release hole in another part of the ear to let the treatment out because it has to go all the way through the ear,” he said.
“And then we just repair and close up, so it’s actually a very similar approach to a cochlear implant, except we don’t put the implant in.”
Image: Experts say it could mark a ‘new era’ in treating deafness. Pic: PA
He said the gene therapy potentially “marks a new era in the treatment for deafness”.
“It was just the fact that we’ve been hearing about this for so long, and there’s been so much work, decades of work… to finally see something that actually worked in humans… It was quite spectacular and a bit awe-inspiring really,” he said.
The treatment was developed especially for children with OTOF mutations, and a second child who has had the same surgery is also seeing positive results.
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It was expected that the three-day state visit would take place in September after Mr Trump let slip earlier in April that he believed that was when his second “fest” was being planned for.
Windsor was also anticipated to be the location after the US president told reporters in the Oval Office that the letter from the King said Windsor would be the setting. Refurbishment works at Buckingham Palace also meant that Windsor was used last week for French President Emmanuel Macron’s visit.
This will be Mr Trump’s second state visit to the UK, an unprecedented gesture towards an American leader, having previously been invited to Buckingham Palace in 2019.
Image: Donald Trump and Melania Trump posing with Charles and Camilla in 2019. Pic: Reuters
He has also been to Windsor Castle before, in 2018, but despite the considerable military pageantry of the day, and some confusion around inspecting the guard, it was simply for tea with Queen Elizabeth II.
Further details of what will happen during the three-day visit in September will be announced in due course.
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On Friday, Sky News revealed it is now unlikely that the US president will address parliament, usually an honour given to visiting heads of state as part of their visit. Some MPs had raised significant concerns about him being given the privilege.
But the House of Commons will not be sitting at the time of Mr Trump’s visit as it will rise for party conference season on the 16 September, meaning the president will not be able to speak in parliament as President Macron did during his state visit this week. However, the House of Lords will be sitting.
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After reading it, Mr Trump said it was a “great, great honour”, adding “and that says at Windsor – that’s really something”.
Image: In February, Sir Keir Starmer revealed a letter from the King inviting Donald Trump to the UK. Pic: Reuters
In the letter, the King suggested they might meet at Balmoral or Dumfries House in Scotland first before the much grander state visit. However, it is understood that, although all options were explored, complexities in both the King and Mr Trump’s diaries meant it wasn’t possible.
This week, it emerged that Police Scotland are planning for a summer visit from the US president, which is likely to see him visit one or both of his golf clubs in Aberdeenshire and Ayrshire, and require substantial policing resources and probably units to be called in from elsewhere in the UK.
Precedent for second-term US presidents, who have already made a state visit, is usually tea or lunch with the monarch at Windsor Castle, as was the case for George W Bush and Barack Obama.
A small plane has crashed at Southend Airport in Essex.
Essex Police said it was at the scene of a “serious incident”.
Images posted online showed huge flames and a large cloud of black smoke, with one witness saying they saw a “fireball”.
A police statement said: “We were alerted shortly before 4pm to reports of a collision involving one 12-metre plane.
“We are working with all emergency services at the scene now and that work will be ongoing for several hours.
“We would please ask the public to avoid this area where possible while this work continues.”
Image: A huge fireball near the airport. Pic: Ben G
It has been reported that the plane involved in the incident is a Beech B200 Super King Air.
According to flight-tracking service Flightradar, it took off at 3.48pm and was bound for Lelystad, a city in the Netherlands.
One man, who was at Southend Airport with his family around the time of the incident, said the aircraft “crashed headfirst into the ground”.
John Johnson said: “About three or four seconds after taking off, it started to bank heavily to its left, and then within a few seconds of that happening, it more or less inverted and crashed.
“There was a big fireball. Obviously, everybody was in shock in terms of witnessing it. All the kids saw it and the families saw it.”
Mr Johnson added that he phoned 999 to report the crash.
Southend Airport said the incident involved “a general aviation aircraft”.
Four flights scheduled to take off from Southend this afternoon were cancelled, according to its website.
Flightradar data shows two planes that had been due to land at Southend were diverted to nearby airports London Gatwick and London Stansted.
Image: Plumes of black smoke. Pic: UKNIP
Essex County Fire and Rescue Service said four crews, along with off-road vehicles, have attended the scene.
Four ambulances and four hazardous area response team vehicles are also at the airport, as well as an air ambulance, the East of England Ambulance Service said.
Its statement described the incident as “still developing”.
Image: Fire engines at the airport
David Burton-Sampson, the MP for Southend West and Leigh, posted on social media: “I am aware of an incident at Southend Airport. Please keep away and allow the emergency services to do their work.
“My thoughts are with everyone involved.”
Local councillor Matt Dent said on X: “At present all I know is that a small plane has crashed at the airport. My thoughts are with all those involved, and with the emergency services currently responding to the incident.”
This breaking news story is being updated and more details will be published shortly.
Another hint that tax rises are coming in this autumn’s budget has been given by a senior minister.
Speaking to Sunday Morning with Trevor Phillips, Transport Secretary Heidi Alexander was asked if Sir Keir Starmer and the rest of the cabinet had discussed hiking taxes in the wake of the government’s failed welfare reforms, which were shot down by their own MPs.
Trevor Phillips asked specifically if tax rises were discussed among the cabinet last week – including on an away day on Friday.
Tax increases were not discussed “directly”, Ms Alexander said, but ministers were “cognisant” of the challenges facing them.
Asked what this means, Ms Alexander added: “I think your viewers would be surprised if we didn’t recognise that at the budget, the chancellor will need to look at the OBR forecast that is given to her and will make decisions in line with the fiscal rules that she has set out.
“We made a commitment in our manifesto not to be putting up taxes on people on modest incomes, working people. We have stuck to that.”
Ms Alexander said she wouldn’t comment directly on taxes and the budget at this point, adding: “So, the chancellor will set her budget. I’m not going to sit in a TV studio today and speculate on what the contents of that budget might be.
“When it comes to taxation, fairness is going to be our guiding principle.”
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Afterwards, shadow home secretary Chris Philp told Phillips: “That sounds to me like a barely disguised reference to tax rises coming in the autumn.”
He then went on to repeat the Conservative attack lines that Labour are “crashing the economy”.
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10:43
Chris Philp also criticsed the government’s migration deal with France
Mr Philp then attacked the prime minister as “weak” for being unable to get his welfare reforms through the Commons.
Discussions about potential tax rises have come to the fore after the government had to gut its welfare reforms.
Sir Keir had wanted to change Personal Independence Payments (PIP), but a large Labour rebellion forced him to axe the changes.
With the savings from these proposed changes – around £5bn – already worked into the government’s sums, they will now need to find the money somewhere else.
The general belief is that this will take the form of tax rises, rather than spending cuts, with more money needed for military spending commitments, as well as other areas of priority for the government, such as the NHS.