Dec 2017 Colchester Daily Gazette

Deaf children not being able to enjoy and participate in music activities is a huge misconception.
Having a hearing impairment alters the sounds but it does not stop the child-like desire to thump on a drum, or to feel the sensations created by different instruments. At Lexden Primary School’s Unit for Hearing Impaired Pupils, music therapy is being used to help build confidence and resilience among its young people. A £500 donation from the Colchester and Tendring Deaf Children’s Society means its 14 pupils, who have between moderate and profound hearing loss, get to become rockstars for two days. Although the ethos of the primary school is centred on inclusivity so ordinarily the hearing impaired pupils are not segregated from their hearing peers, when it comes to learning and playing music, solo, in a sense, works best.

Music & deaf kids

Music & childrenDebbie Newbold, head of the enhanced provision, said: “Deaf children are unable to access speech and music through their ears alone, they need it through amplification.

“Hearing sounds through a cochlear implant produces quite a metallic sound and hearing aids don’t enable a person to know how loudly they’re producing sounds. In the context of a music lesson, it means our pupils can’t tell if they’re singing in tune, for example, or if they’re playing a piece of music off-beat and it becomes hard for them to follow the pace and rhythm. Because the sounds are being amplified in their personal aid, whether an implant or behind the ear hearing aids, it’s hard to follow a tune and how music fits into the bigger picture when performing with others”.

“Within a school situation, this can be quite a challenge for them so we tend to find deaf children are quite inhibited when it comes to music. Therefore holding sessions in small groups where they can have free reign to try, play and record their own music can be very beneficial.”

When the sessions start next term, it will be the second occasion the students, aged up to 11, have worked with a professional music therapist who is able to progress their learning better than most. Working together like a band, they have a chance to compose and play music before recording it and watching it back. This is a novelty compared to practising with hearing children who can naturally race through music.

Debbie said: “It works a lot on their listening skills, their ability to recognise different instruments and rhythms, and to feel the vibrations of things, particularly the drums. The therapist uses technology like iPads to compose with and can overlay different sounds, so the money from the charity will fund the expertise and equipment that the school doesn’t have access to. Parents of deaf children make up Colchester and Tendring Deaf Children’s Society. Some of their children were once pupils here or are still at the unit so they understand this is an area we want to develop. 

The children greatly enjoy it and talk about it for ages afterwards.”

Deaf kids with musicKids & musicNo child at the unit is completely dependent on sign language. Some were born without hearing and others have lost their hearing over time, but everyone is encouraged to make best use of aids to help them access the world around them. Hearing loss can be extremely isolating. Missing sounds hampers a person’s understanding of what is happening around them which can then lead to social exclusion and affects a child’s resilience, and self-esteem, early on in their development.

She said: “Music plays such an important part in people’s lives so we want to still give our pupils some of that although they can’t hear it properly. Music therapy can increase anyone’s capacity for learning and coping within a learning environment. Separating the children means the deaf pupils can take their time and really feel the music in an emotional way.”

Many of the people in north Essex are unaware the unit exists, according to Debbie, but it has been in existence for 50 years. It is staffed by three teachers of the deaf and seven teaching assistants, who treat the deaf pupils just the same as hearing pupils.

Dec 2017 Our Mail

Dr Dimity Dornan, has dedicated decades of her life to bringing sound to the lives of deaf children. If the senior years of life are supposed to be about doing nothing much except ticking off everything on your bucket list, Dr Dimity Dornan did not get the memo. There’ll be no lying back with a margarita on some exotic faraway beach for her. Instead, it’s business as usual for Queensland ­Senior of the Year 2018, the state’s entrant in Australian Senior of the Year, to be announced on January 25. Recognised worldwide for her groundbreaking work changing the lives of thousands of deaf children by helping them learn to listen and speak, Dornan was Queenslander of the Year in 2010, but admits to being slightly taken aback when approached to be Queensland’s Senior.

Pip Russell etc(left-right) Queensland Local Hero winner Pip Russell, Queensland Australian of the Year winner Johnathan Thurston, Queensland Senior Australian of the Year winner Dr Dimity Dornan, and Queensland Young Australian of the Year winner Phillip Thompson

“I suppose it’s really quite funny, I’ve now moved up to the senior section!” she says in our interview in one of the beautifully designed rooms at Hear and Say’s premises in Brisbane’s inner-west Ashgrove, which opened in 2015. “The Senior Australian of the Year people rang and asked if I’d like to accept it and I said, yes, I’d love to, but then I had second thoughts later on. (I told them) if I had to be Queensland’s ­Senior Australian of the Year, I’d really like it if you didn’t use my age if you can possibly do it.” But isn’t there a certain irony here? A person of senior years being feted for her achievements not wishing to mention her age? “I prefer to be treated as a professional, rather than have age define me,” she says. “I just feel that it’s such a competitive world, it’s much better to compete on your merits, rather than your age.”

Let’s just say then that Dimity Dornan, PhD, AO, ­author, speech pathologist of more than 50 years’ standing, inspirational promoter of the largest paediatric cochlear implant program in Queensland, which has used Auditory-Verbal Therapy (AVT) to empower some 10,000 children to fulfil their potential in life, is older than 60 but younger than 100.

Hear and Say – the private, not-for-profit organisation she ­established in 1992, treating six young ­patients from rooms she shared with her physiotherapist husband Peter Dornan in the inner-western Brisbane suburb of Toowong – has just celebrated its 25th birthday. A new book cele­brating the history of the centre, Sounds of Hope: The Hear and Say Story written by Brisbane author Madonna King and published by University of Queensland Press, was launched by Governor Paul de Jersey.

Hear and Say now has six centres around Queensland, treating children and their families here, as well as around the world, using new telecommunications technology that allows face-to-face interaction. The acclaimed facility ­offers its services to any deaf baby or child no matter where they live. Currently two in every 1000 babies are born deaf in Australia each year and it is the most common disability in newborns. Hear and Say ­relies largely on fundraising on top of ­government grants, and part of Dornan’s job as its executive director is being its tireless advocate, a lobbyist par ­excellence, a passionate and moving speaker whose eyes still fill with tears when she speaks of her life’s work, or the moment – which never ­becomes stale – a child first hears the sounds of the world. Dornan was born Dimity Crist (pronounced as in “crisp”), the eldest of four girls and a boy born to Richard “Dick” Crist, a Shell sales rep, and his wife Marjorie, a full-time mother. Dick Crist was a World War II veteran and, before their marriage, Marjorie worked in US General Douglas MacArthur’s mapping department.

The Crists were of German descent (the name was spelt “Christ” but along the way the “h” disappeared) and were pioneers of the Toowoomba region. Dick’s mother, Alice Guerin, was of Irish origin. Alice died before Dimity was born but she was always intrigued by the mystery of her grandmother’s portrait, and by the fact that she was a ­writer. But it was her grandmother’s work as a teacher that lit up her impressionable granddaughter. “She came from Ireland with her parents in the early vanguard of teachers who were meant to upgrade the quality of education in Queensland … she travelled with her father to different state schools, eventually became a pupil teacher and then a teacher herself at a very young age.”

Dornan’s parents believed in the value of education, too. The family lived on a large block at Corinda in Brisbane’s southwest, and Dornan and her sisters attended Our Lady of the Sacred Heart Convent there. From an early age, Dornan knew she wanted some kind of career that involved helping people. The family knew a professor of psychology from UQ who lived nearby, who told her about a new speech pathology course. Dornan would become one of the first graduates in speech therapy at UQ.

Dr Dimity DormanDr Dimity Dornan of the Hear and Say Centre with Edward Mewing (2)

Her first jobs involved working with patients with head injuries. “It was wonderful work, but then I got the opportunity to work with children with hearing loss. I said to my prospective employer, ‘but I don’t know anything about hearing loss!’ And she said something that changed my life, something I like to pass on to other people. She said: ‘No, but you have the ability to find out’. So I’ve been finding out ever since – I’ve become a lifelong learner and that’s what I’ve been up to. People think they’ve got their qualifications, and then they sit back, but I’ll tell you what, life’s not like that.”

Which is why, besides her work as Hear and Say’s executive director, the fundraising and the meet-and-greets she ceaselessly undertakes every day, Dornan is ­increasingly absorbed in the area of bionics, founding ­Bionics Queensland and the Human Bionics ­Interface, an Australian research collaboration project to grow the ­bionics ­industry. She has also ­secured a manufacturer for a cutting-edge invention, a hearing aid that allows people to hear in noisy restaurants.
This is a woman who is constantly learning.

As if her days aren’t busy enough, there are now four grandchildren she enjoys with her husband whom she married in 1967. The couple has two children – Brisbane-based Melissa, 49, who runs a business helping mothers get over traumatic births, and Melbourne-based Roderick, 47, known as Rod, who works in IT for IBM.

“I came late to my career,” Dornan says. “I was always a speech pathologist but I didn’t start doing Hear and Say until my own kids were in uni, so I’m sort of a late starter, if you like, in that area.” She began working with deaf children when she had to learn sign language to work with hearing-impaired children at Zillmere North Special Education Unit. She became frustrated that parents weren’t involved in reinforcing their children’s learning, and eventually found her way to the Shepherd Centre in Sydney, which in turn led to a Churchill Fellowship to Canada to study AVT.

Research shows that the critical brain period for learning to speak is within the first three years of life, therefore early diagnosis is essential. Once a baby or child has been fitted with a cochlear implant, AVT successfully develops the listening and spoken language of kids with hearing loss by stimulating auditory brain development. The input of parents is critical to reinforce learning.
When Dornan speaks of giving the gift of sound, her eyes fill. “I feel very strongly that I have a purpose in life,” she says.

Dornan mentions the Future Hear Project, which she is working on with Queensland University of Technology, which aims to build ears for children born without them. It involves the 3D printing of a prosthetic ear and a bio­degradable scaffold built from a child’s cartilage and skin cells that will allow an ear to grow. It’s nothing short of ­miraculous. It’s also nothing short of all the little miracles ­Dornan has guided along the way, from teaching a child to distinguish an “f” sound from an “s” sound, to getting ­citizens to part with their money to help a deaf child. Do we care if Dr Dimity Dornan is classified as a senior, junior or middle-aged Australian? My guess is we care more that people like her keep doing what they’re doing, while the rest of us keep being grateful.

Dec 2017 Daily Mail Australia

Lewis, 15, has been profoundly deaf since birth, the result of a genetic condition he was diagnosed with at 13 months old. He was raised to be 'deaf and proud' and attends specialist deaf school Mary Hare, in Newbury, Berkshire.  But two years ago he decided to have the device fitted in his ear in the hope he would be able to hear his own name for the first time, admitting it was 'like he doesn't have one'. Lewis made the heartfelt confession in the Channel 4 documentary Extraordinary Teens: School of Life and Deaf, moving viewers to tears. 

LewisMary Hare Deaf SchoolExtraordinary Teens: School of Life and Deaf, which follows a year in their life at specialist deaf school Mary Hare, in Newbury

Dozens took to Twitter to praise the teenager, saying his story was 'so moving'. One tweeted today: 'Eyes still puffy today from crying them out last night for the boy who heard birdsong - and his name - for the first time aged 15.'

Speaking on the show, which followed three students at the school over the course of a year, Lewis explained he had been hesitant about having the implant fitted.  They are a sensitive issue among some parts of the deaf community, who regard them as an unwelcome attempt to 'cure' deafness. Lewis' mother Jaime recalled on the show how she watched her son wrestle with his decision. 'I think he felt disloyal to deaf culture by even contemplating it, because he'd been brought up to be deaf and proud - it was a massive step for him to say he was curious,' she said.  Lewis explained there was no instant, overnight change as a result of getting the device. Two years on from being fitted, he can still feel like he is 'hitting a wall', or that there is a 'barrier he can't get past’. But it has granted his wish to hear the sound of his parents saying his name - and the sound of birdsong.  Lewis says on the show he is keen to emphasise that in every other respect he is no different from any other teenager.  'I can be a bit cheeky and I can be naughty,' he admits. 'I'm just a normal teenager. And no-one wants to be perfect do they?'  His ambition is to become a photographer, and Lewis refuses to be held back by his disability. 'I hate it that people think deaf people are stupid and not able to achieve,' he says on the show.  'We are just like anyone else - we just can't hear, that's all.' 

Lewis is the only child of Colin Hunt, a courier, 42, and wife Jaime, hairdresser, and his diagnosis came as an immense shock to his parents, neither of whom had any history of hearing problems. Poignantly, he confides that there was one reason in particular that has also pushed him towards the decision to have an implant: he wanted to be able to hear his parents call his name.

'It's like I don't have a name,' he says 'as I've never heard it before.’ An implant is not a panacea though – while many people can hear some sounds straightaway for others it doesn't work at all at first. For some it makes little difference at all, or makes things worse. For Lewis it took time – two years after getting the implant he admits it is still a work in progress - and the documentary does not shy away from the upset and frustrations he experiences as he realises he will require months of intensive speech and language therapy and ongoing testing to see if he will ever hear a full range of sounds. 'I remember the implant being switched on,' he recalls now.  'All I could hear was "beep". I realised that it was training my brain to hear but I was confused and nervous. Some people think that the cochlear implant is going to work straight away after switch-on – you see this kind of thing on social media. I needed time to understand that this wasn't necessarily going to happen to me.'

It has been, he admits, a 'bumpy' road since, and as the months ticked by with only minor improvements to his hearing Lewis struggled to contain his frustration.  'I just get so angry sometimes,' he admits at one point in the documentary.  It's like you want it but you can't get it - there's a barrier you can't reach and you feel like you've hit a wall.'  Yet there have been beautiful moments too – like the time he discovered he could hear birdsong. 'I recorded a sound and I thought "what is this sound?" and was shocked to hear that it was birds,' he grins. 'And I can hear my own name now which is great.’

Lewis acknowledges that accepting his lack of hearing has become harder as he has gotten older and begun to yearn for more independence. 'For me one of the main things is always having to say 'I'm deaf', when talking to hearing people. I would like not to have to say that.’ In particular he has always struggled with his speech: for many years, when in public environments like shops or transport hubs, Lewis largely communicated by handing over a note – the documentary shows him buying a rail ticket to his school by writing down his destination and handing it to the ticket office.

Lewis Hunt'If I compare my speech to my friends I'd say their speech is way better than mine,' he admits. 'I hate the word 'can't', I hate feeling like that - I like to be able to do things.’

At Mary Hare, sign language isn't a requirement – while many pupils use it to communicate amongst themselves, lessons are taught through speech – a deliberate decision that is itself the subject of some controversy.'There can be a lot of politics around deaf culture,' admits the school's head teacher Peter Gale.  'There are people who passionately believe that sign language is their natural language and that that is how communication should take place. We have no issue with sign language, but our view is we want our pupils to maximise their potential to speak, lip read and listen and understand and therefore develop good English literacy. It is not an issue that affects the pupils. There are no camps here in terms of friendships or social groups – you could have two best friends one of whom uses sign language a lot and the other of whom is a more typical hands-in-pockets teenager – they don't care which is why it is so frustrating that the adults can care so much.'

Dec 2017 The Guardian Australian Edition and Boston Business Journal

Deafness has been prevented in mice using gene editing for the first time, in an advance that could transform future treatment of genetic hearing loss. The study found that a single injection of a gene editing cocktail prevented progressive deafness in baby animals that were destined to lose their hearing. “We hope that the work will one day inform the development of a cure for certain forms of genetic deafness in people,” said Prof David Liu, who led the work at Harvard University and MIT.
Nearly half of all cases of deafness have a genetic root, but current treatment options are limited. However, the advent of new high-precision gene editing tools such as Crispr has raised the prospect of a new class of therapies that target the underlying problem.

Crispr

Treatment with Crispr was shown to preserve sound-detecting hairs in the inner ear of mice (white box, left). Without the treatment, these hairs disappear (white box, right).

Crispr, or to give it its full name, Crispr-Cas9, allows scientists to precisely target and edit pieces of the genome. Crispr is a guide molecule made of RNA, that allows a specific site of interest on the DNA double helix to be targeted. The RNA molecule is attached to Cas9, a bacterial enzyme that works as a pair of "molecular scissors" to cut the DNA at the exact point required. This allows scientists to cut, paste and delete single letters of genetic code. 

RNA moleculeThe study focused on a mutation in a gene called Tmc1, a single wrong letter in the genetic code, that causes the loss of the inner ear’s hair cells over time. The delicate hairs, which sit in a spiral-shaped organ called the cochlea, vibrate in response to sound waves. Nerve cells pick up the physical motion and transmit it to the brain, where it is perceived as sound. If a child inherits one copy of the mutated Tmc1 gene they will suffer progressive hearing loss, normally starting in the first decade of life and resulting in profound deafness within 10 to 15 years. However, since most people affected by the mutation will also have a healthy version of the gene, inherited from their other parent, the scientists wanted to explore whether deleting the faulty version worked as a treatment.

Liu and colleagues used gene editing technology known as Crispr-Cas9, which acts as a molecular scissors, snipping the genome to disable a target gene. The team injected the gene editing solution into the inner ears of baby mice with the hearing loss mutation. After eight weeks, hair cells in treated ears resembled those in healthy animals – densely packed and tufted with hairlike bundles. The hair cells of untreated mice, in contrast, looked damaged and sparse.
Then the researchers conducted a hearing test on the mice by placing electrodes on their heads and monitoring the activity of brain regions involved in hearing. Researchers needed more sound to spark brain activity in untreated mice compared with treated mice, the team found. On average, after four weeks, treated ears could hear sounds about 15 decibels lower than untreated ears. “That’s roughly the difference between a quiet conversation and a garbage disposal,” Liu said.
Simon Waddington, a reader in gene transfer technology at University College London, described the study as an elegant application of new gene editing tools. “Hitherto incurable and often even untreatable diseases are now within the scope of gene therapy,” he said.

The team plans to develop the therapy in larger animals to ensure the method is safe and effective, before moving closer to a patient trial.