An 18-month-old British girl has had her hearing restored after becoming the first person in the world to take part in a ground-breaking gene therapy trial.
Opal Sandy was born completely deaf due to auditory neuropathy, which is caused by the disruption of nerve impulses travelling from the inner ear to the brain.
Opal’s hearing is now almost perfect – and could even improve further thanks to a “one and done” gene therapy being trialled in the UK and worldwide.
The girl, from Oxfordshire, who has a genetic form of auditory neuropathy, was treated at Addenbrooke’s Hospital, which is part of Cambridge University Hospitals NHS Foundation Trust.
Prof Manohar Bance, an ear surgeon at the trust and chief investigator for the trial, told the Press Association the results were “better than I hoped or expected” and may cure patients 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.
Auditory neuropathy can be caused by a fault in the Otof gene, which is responsible for making a protein called otoferlin. This enables cells in the ear to communicate with the hearing nerve.
To overcome the fault, the “new-era” gene therapy – from biotech firm Regeneron – delivers a working copy of the gene to the ear.
In Opal’s case, she received an infusion containing the working gene to her right ear during surgery in September.
Her parents Jo and James, both 33, noticed improvements to her hearing after four weeks when Opal turned her head to loud clapping.
“When she first turned, I couldn’t believe it,” Ms Sandy told PA.
“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.
“I picked my phone up and texted James, and said ‘I think it’s working’. I was absolutely gobsmacked. I thought it was a fluke.”
But even more impressive results were on the horizon.
About 24 weeks after surgery, in February, tests in Cambridge showed Opal could also hear soft sounds such as a whisper.
“The audiologist played back some of the sounds that she was responding to and they were ridiculously quiet sort of sounds that in the real world wouldn’t catch your attention during a conversation,” Ms Sandy said.
“Certainly since February, we’ve noticed her sister [Nora[ waking her up in the morning because she’s running around on the landing, or someone rings on the door so her nap’s cut short.
“She’s definitely responding more to sort of what we would call functional sounds rather than just sounds that we use to test her.
“We were told she had near normal hearing last time – 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.”
Prof Bance said Opal’s hearing is now “close to normal”, adding: “We hope she’ll get back to normal by the next testing.”
He added that the treatment is “a one-and-done therapy, so hopefully you have your treatment and then you go back to your life”.
A second child has also received the gene therapy treatment at Cambridge University Hospitals, with positive results seen recently, six weeks after surgery.
The overall phase 1/2 Chord trial consists of three parts, with three deaf children, including Opal, receiving a low dose of gene therapy in one ear only.
A different set of three children will get a high dose on one side. Then, if that is shown to be safe, more children will receive a dose in both ears at the same time.
Up to 18 youngsters from the UK, Spain and the US are being recruited to the trial and will be followed up for five years.
“My entire life, gene therapy has been ‘five years away’, and I’ve been in practice about 30 years," Prof Bance said.
“So, for me, it was almost unreal that this moment had arrived.
“It felt very special.”
At the moment, the gold standard treatment for auditory neuropathy is cochlear implants.
Opal had one fitted to her left ear at the same time as she underwent gene therapy in her right ear, to ensure she got hearing as soon as possible.
The youngster is the first patient globally to receive the Regeneron therapy and “she’s the youngest globally that’s been done to date as far as we know,” Prof Bance said.
China has also been working on targeting the same gene, with positive results, though Prof Bance said theirs uses a different technology and slightly different mode of delivery.
Martin McLean, senior policy adviser at the National Deaf Children’s Society, welcomed the study, saying it would lead to learning regarding gene therapies for deafness with a specific genetic cause.
“We would like to emphasise that, with the right support from the start, deafness should never be a barrier to happiness or fulfilment,” he said.
“As a charity, we support families to make informed choices about medical technologies, so that they can give their deaf child the best possible start in life.”
Should late investors consider cryptocurrencies?
Wealth managers recommend late investors to have a balanced portfolio that typically includes traditional assets such as cash, government and corporate bonds, equities, commodities and commercial property.
They do not usually recommend investing in Bitcoin or other cryptocurrencies due to the risk and volatility associated with them.
“It has produced eye-watering returns for some, whereas others have lost substantially as this has all depended purely on timing and when the buy-in was. If someone still has about 20 to 25 years until retirement, there isn’t any need to take such risks,” Rupert Connor of Abacus Financial Consultant says.
He adds that if a person is interested in owning a business or growing a property portfolio to increase their retirement income, this can be encouraged provided they keep in mind the overall risk profile of these assets.
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UAE currency: the story behind the money in your pockets
Company Profile
Company name: OneOrder
Started: October 2021
Founders: Tamer Amer and Karim Maurice
Based: Cairo, Egypt
Industry: technology, logistics
Investors: A15 and self-funded
The specs
AT4 Ultimate, as tested
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Premier League results
Saturday
Crystal Palace 1 Brighton & Hove Albion 2
Cardiff City 2 West Ham United 0
Huddersfield Town 0 Bournemouth 2
Leicester City 3 Fulham 1
Newcastle United 3 Everton 2
Southampton 2 Tottenham Hotspur 1
Manchester City 3 Watford 1
Sunday
Liverpool 4 Burnley 2
Chelsea 1 Wolverhampton Wanderers 1
Arsenal 2 Manchester United 0
The specs
Engine: 3.9-litre twin-turbo V8
Power: 620hp from 5,750-7,500rpm
Torque: 760Nm from 3,000-5,750rpm
Transmission: Eight-speed dual-clutch auto
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Price: From Dh1.05 million ($286,000)
WHAT IS A BLACK HOLE?
1. Black holes are objects whose gravity is so strong not even light can escape their pull
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3. Large black holes can also be formed when smaller ones collide and merge
4. The biggest black holes lurk at the centre of many galaxies, including our own
5. Astronomers believe that when the universe was very young, black holes affected how galaxies formed
Lexus LX700h specs
Engine: 3.4-litre twin-turbo V6 plus supplementary electric motor
Power: 464hp at 5,200rpm
Torque: 790Nm from 2,000-3,600rpm
Transmission: 10-speed auto
Fuel consumption: 11.7L/100km
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COMPANY PROFILE
Name: Almnssa
Started: August 2020
Founder: Areej Selmi
Based: Gaza
Sectors: Internet, e-commerce
Investments: Grants/private funding
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Labour dispute
The insured employee may still file an ILOE claim even if a labour dispute is ongoing post termination, but the insurer may suspend or reject payment, until the courts resolve the dispute, especially if the reason for termination is contested. The outcome of the labour court proceedings can directly affect eligibility.
- Abdullah Ishnaneh, Partner, BSA Law
Top tips
Create and maintain a strong bond between yourself and your child, through sensitivity, responsiveness, touch, talk and play. “The bond you have with your kids is the blueprint for the relationships they will have later on in life,” says Dr Sarah Rasmi, a psychologist.
Set a good example. Practise what you preach, so if you want to raise kind children, they need to see you being kind and hear you explaining to them what kindness is. So, “narrate your behaviour”.
Praise the positive rather than focusing on the negative. Catch them when they’re being good and acknowledge it.
Show empathy towards your child’s needs as well as your own. Take care of yourself so that you can be calm, loving and respectful, rather than angry and frustrated.
Be open to communication, goal-setting and problem-solving, says Dr Thoraiya Kanafani. “It is important to recognise that there is a fine line between positive parenting and becoming parents who overanalyse their children and provide more emotional context than what is in the child’s emotional development to understand.”
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Company profile
Company name: Dharma
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Founders: Charaf El Mansouri, Nisma Benani, Leah Howe
Based: Abu Dhabi
Sector: TravelTech
Funding stage: Pre-series A
Investors: Convivialite Ventures, BY Partners, Shorooq Partners, L& Ventures, Flat6Labs
Scoreline:
Everton 4
Richarlison 13'), Sigurdsson 28', Digne 56', Walcott 64'
Manchester United 0
Man of the match: Gylfi Sigurdsson (Everton)
BRIEF SCORES
England 228-7, 50 overs
N Sciver 51; J Goswami 3-23
India 219, 48.4 overs
P Raut 86, H Kaur 51; A Shrubsole 6-46
England won by nine runs
The%20stats%20and%20facts
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'Gold'
Director:Anthony Hayes
Stars:Zaf Efron, Anthony Hayes
Rating:3/5
If you go…
Emirates launched a new daily service to Mexico City this week, flying via Barcelona from Dh3,995.
Emirati citizens are among 67 nationalities who do not require a visa to Mexico. Entry is granted on arrival for stays of up to 180 days.
UAE currency: the story behind the money in your pockets
'Panga'
Directed by Ashwiny Iyer Tiwari
Starring Kangana Ranaut, Richa Chadha, Jassie Gill, Yagya Bhasin, Neena Gupta
Rating: 3.5/5
Evacuations to France hit by controversy
- Over 500 Gazans have been evacuated to France since November 2023
- Evacuations were paused after a student already in France posted anti-Semitic content and was subsequently expelled to Qatar
- The Foreign Ministry launched a review to determine how authorities failed to detect the posts before her entry
- Artists and researchers fall under a programme called Pause that began in 2017
- It has benefited more than 700 people from 44 countries, including Syria, Turkey, Iran, and Sudan
- Since the start of the Gaza war, it has also included 45 Gazan beneficiaries
- Unlike students, they are allowed to bring their families to France
Our legal consultant
Name: Hassan Mohsen Elhais
Position: legal consultant with Al Rowaad Advocates and Legal Consultants.
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F1 The Movie
Starring: Brad Pitt, Damson Idris, Kerry Condon, Javier Bardem
Director: Joseph Kosinski
Rating: 4/5
Most sought after workplace benefits in the UAE
- Flexible work arrangements
- Pension support
- Mental well-being assistance
- Insurance coverage for optical, dental, alternative medicine, cancer screening
- Financial well-being incentives
Banned items
Dubai Police has also issued a list of banned items at the ground on Sunday. These include:
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Political flags or banners
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Bikes, skateboards or scooters
How to get exposure to gold
Although you can buy gold easily on the Dubai markets, the problem with buying physical bars, coins or jewellery is that you then have storage, security and insurance issues.
A far easier option is to invest in a low-cost exchange traded fund (ETF) that invests in the precious metal instead, for example, ETFS Physical Gold (PHAU) and iShares Physical Gold (SGLN) both track physical gold. The VanEck Vectors Gold Miners ETF invests directly in mining companies.
Alternatively, BlackRock Gold & General seeks to achieve long-term capital growth primarily through an actively managed portfolio of gold mining, commodity and precious-metal related shares. Its largest portfolio holdings include gold miners Newcrest Mining, Barrick Gold Corp, Agnico Eagle Mines and the NewMont Goldcorp.
Brave investors could take on the added risk of buying individual gold mining stocks, many of which have performed wonderfully well lately.
London-listed Centamin is up more than 70 per cent in just three months, although in a sign of its volatility, it is down 5 per cent on two years ago. Trans-Siberian Gold, listed on London's alternative investment market (AIM) for small stocks, has seen its share price almost quadruple from 34p to 124p over the same period, but do not assume this kind of runaway growth can continue for long
However, buying individual equities like these is highly risky, as their share prices can crash just as quickly, which isn't what what you want from a supposedly safe haven.
MATCH INFO
Uefa Champions League semi-final, first leg
Tottenham v Ajax, Tuesday, 11pm (UAE).
Second leg
Ajax v Tottenham, Wednesday, May 8, 11pm
Games on BeIN Sports
UK-EU trade at a glance
EU fishing vessels guaranteed access to UK waters for 12 years
Co-operation on security initiatives and procurement of defence products
Youth experience scheme to work, study or volunteer in UK and EU countries
Smoother border management with use of e-gates
Cutting red tape on import and export of food
