A HEARTWARMING video shows a little boy who was born deaf dancing for the first time after receiving an ‘astounding’ injection giving the ability to hear.
The boy was one of five deaf children to receive the treatment that restored hearing in both ears.
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As part of a clinical trial at the Eye & ENT Hospital of Fudan University in Shanghai, China – conducted in partnership with Mass General Brigham Eye and Ear researchers – the kids were given a novel gene therapy designed to target a form of inherited deafness called DFNB9.
After being treated in both their ears, the children were able to hear for the first time, perceiving speech and were sounds came from.
One of those was a little boy, who couldn’t hear anything before being given the injection.
A heartwarming video filmed 15 weeks after the injection shows the tot dancing to ‘Twinkle Twinkle Little Star’, waving his hands round in excitement.
The clip also shows the little boy responding to sounds from different directions, wheeling around and almost losing his balance.
The little boy also began to understand some words in the weeks after receiving his treatment, trying to say ‘Baba’ – the word for Daddy – and ‘Ayi’, the word for Aunty.
Senior author Zheng-Yi Chen, an associate scientist in the Eaton-Peabody Laboratories at Mass Eye and Ear, said: “The results from these studies are astounding.
“We continue to see the hearing ability of treated children dramatically progress and the new study shows added benefits of the gene therapy when administrated to both ears, including the ability for sound source localisation and improvements in speech recognition in noisy environments.”
Over 430 million people around the world are affected by disabling hearing loss, according to study authors, with 26 million of them born deaf.
Up to 60 percent of childhood deafness is caused by genetic factors, they added.
In the UK, one in five adults are deaf, have hearing loss or tinnitus, according to RNID.
And there are more than 50,000 deaf children, three born every day, according to the National Deaf Children’s Society.
Children with DFNB9 are born with mutations in the OTOF gene that prevent the production of functioning otoferlin protein, which is necessary for mechanisms in hearing.
In 2022, the research team treated six deaf children for DFNB9, injecting the gene therapy in one ear.
That trial, whose findings were published in The Lancet in January this year, showed five of six children gaining improvements to their hearing and speech.
This new study is the first to administer the gene therapy in both ears.
Lead study author Yilai Shu – director of Diagnosis and Treatment Center of Genetic Hearing Loss – injected copies of the human OTOF gene carried by a virus into the inner ears of the kids in minimally invasive surgery.
The five children were observed over a 13 and 26-week period.
All of them showed hearing recovery in both ears, with dramatic improvements in speech perception and sound localisation.
But two of the children gained an ability to appreciate music – a more complex auditory signal – and were seen dancing to music in videos captured for the study.
The trial remains ongoing and the children are continuing to be monitored.
Dr Shu said: “Restoring hearing in both ears of children who are born deaf can maximise the benefits of hearing recovery.
“These new results show this approach holds great promise and warrant larger international trials.”
Dr Chen added: “Our study strongly supports treating children with DFNB9 in both ears, and our hope is this trial can expand and this approach can also be looked at for deafness caused by other genes or non-genetic causes.
“Our ultimate goal is to help people regain hearing no matter how their hearing loss was caused.”
Currently, there are no drugs available to treat inherited deafness, which has made room for novel interventions like gene therapies.
But the study authors noted that more work is needed to further study and refine the treatment.
They said injecting the treatment in both ears rather than just one the course of one surgery doubles the surgical time.
On top of that, injecting two doses of a virus into the body could trigger a stronger immune response, with more adverse effects.
The authors intend to use the treatment on more patients and observe them for longer afterwards.
The study was published in Nature Medicine.
It comes after an 18-month-old girl born with damaged nerves between her inner ear and brain became able to hear for the first time thanks to experimental NHS gene therapy.