MUSIC TO MY EARS
Watch the heartwarming moment a little boy dances for the first time after getting ‘astounding’ injection for deafness
Researchers injected the groundbreaking treatment into the boys ear using a virus
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.
2
2
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.
Read more on deafness
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.”
Most read in Health
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 per cent of childhood deafness is caused by genetic factors, they added.
Girl born deaf hears for the first time after experimental gene therapy
In the UK, one in five adults are deaf, have hearing loss or tinnitus, according to .
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, 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.
READ MORE SUN STORIES
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.
Are there cures for deafness?
Congenital hearing loss (that is present at birth) can be caused by infections, such as rubella or herpes simplex virus, premature birth, drug or alcohol use during pregnancy, maternal diabetes or high blood pressure, among other things.
It can also be genetic.
Acquired hearing loss (that develops later in childhood) can be caused by infectious diseases, such as meningitis or recurrent ear infections, as well as trauma and certain medications.
Treatment can include hearing aids, medication, surgery or cochlear implants
A cochlear implant is an electronic device that onsists of an external portion that sits behind the ear and a second portion that is surgically placed under the skin.
It is different from a hearing aid, which amplifies sound, making it possible to detected by those with damaged hearing.
Specialist may also perform surgery if there is a strucutral problem within the ear that obstructs hearing.
Topics