Emma and Alastair White had been warned not to expect a "YouTube moment" when their 18-month-old, profoundly deaf daughter Sadbh had her cochlear implants switched on for the first time last month.
“She was on my knee playing with toys the audiologist had put out and there was no reaction, she didn’t move at all,” says Emma White. “But they told us from what they could see on the screen that everything was working really, really well, so we still came out feeling really positive.”
Like the vast majority of parents of the 100 or so babies born in the Republic every year with hearing loss, the Whites had no experience of the deaf world. And there was nothing during pregnancy or delivery to flag concerns about their first child.
So it was a big shock when they received the diagnosis at seven weeks. Sadbh had failed the newborn screening test and two follow-up outpatient tests before being referred to an audiologist.
“This was a whole culture we had no idea of,” says White. “And because I am a general nurse, in my head I went straight to ‘what else is there?’”
Some 30 to 40 per cent of children who are deaf or hard of hearing have an additional condition. “My husband, being a teacher, went straight to ‘how is she going to cope at school?’”
However, from the moment of diagnosis, the audiologist offered them good information, she says, including details of DeafHear, a charity that supports more than 32,500 deaf and hard-of-hearing people and their families, and the HSE had Sadbh’s care plan rolling straight away.
State provision of the technology is not followed up with adequate investment in the therapy needed to help children gain maximum benefit from the tools they have been given
“By 12 weeks Sadbh was fitted with hearing aids, just to try and keep her nerve stimulated and get whatever sound we could in to keep the brain trying to learn to listen.”
They were also referred to a paediatrician to make sure there were no other issues, as well as to the cochlear implant team in Beaumont Hospital, Dublin.
“Knowing that Sadbh was profoundly deaf,” her mothers says, “we knew the only way she would access any kind of hearing would be through cochlear implants.”
While hearing aids just amplify sounds, cochlear implants bypass damaged portions of the ear and directly stimulate the auditory nerve. Signals generated by the implant are sent by way of the auditory nerve to the brain, which recognises them as sound.
What the Whites found most beneficial in the early stages was going on a DeafHear weekend away. There they talked to other parents in a similar situation, met deaf people who had implants and also those who use sign language as their first language. All of Sadbh’s options were outlined.
“It just opened us up to that this isn’t the end of things, it’s actually the beginning of a slightly different journey,” says White. They don’t know what caused their daughter’s hearing loss, but any co-existing syndromes have been ruled out.
Suitable candidate
Once Sadbh was confirmed at a year old as a suitable candidate for the bilateral cochlear implant programme, it was a matter of waiting for a date for the surgery, to be done in Temple Street Children’s Hospital because she was under six.
The surgery was on April 3rd last, when Sadbh was 16 months, and the implants were switched on a month later. “The challenge now is help her to learn sounds and explain to her what she is experiencing,” says her mother.
Sadbh already has some sign language, as do her parents, because their daughter was entitled to one hour’s tuition a week from the time of diagnosis and the teacher who came out to their house taught it to all of them. “If all else fails, she has that to communicate with.”
White realises that opinions differ about the use of sign language when striving to teach the spoken word, but they have been told to say things to Sadbh three times, and then sign it afterwards to reinforce the message.
While they have been happy so far with their daughter's treatment within the health service, they are concerned about what will happen down the line
It’s early days, but at least Sadbh is keeping her technology on – “which is half the battle with a toddler”, her mother laughs. Adults would normally wear the external processor on the ear, which is connected by a wire to a metal coil on the scalp. But young children’s ears are too small, so Sadbh has her processor tucked into in a headband. The battery pack is worn separately, clipped on to her clothes.
White doesn’t deny there are days when she worries about Sadbh’s future. While they have been happy so far with their daughter’s treatment within the health service, they are concerned about what will happen down the line.
Parents of older children born with hearing loss would say they are right to be concerned. Although two major policy improvements have been made within the past decade in regard to early diagnosis and treatment, State provision of the technology is not followed up with adequate investment in the therapy needed to help children gain maximum benefit from the tools they have been given.
Universal newborn screening was introduced in the Republic in 2013. After intense lobbying by parents in a Happy New Ears campaign, bilateral cochlear implants for newly diagnosed children, instead of just one, became standard practice in 2014.
Cochlear implant programme
ENT consultant Laura Viani set up the cochlear implant programme in Beaumont Hospital more than 20years ago with a staff of two. She decided from the outset to make it a totally public service and not do any private practice.
They are still profoundly deaf people when they turn their implant off"
There is now a multidisciplinary team of 30, and about 200 implants are performed each year on small children, adolescents and adults, with priority given to children. Prof Viani would like to have more theatre access so that both children and adults can be operated on quicker.
“We always strive to improve the service,” she says. “My aim for implantation in children is to open up options for them. Not all children will learn to speak, but hopefully they will give them sound awareness. Children that can hear well with an implant can learn to speak and go to mainstream school.”
Still, it is no miracle cure, she stresses. “They are still profoundly deaf people when they turn their implant off.”
She acknowledges that some in the deaf culture don’t agree with implants, as they regard sign language as their language. But if you don’t give a child an implant early on, she adds, they won’t have a choice later because the auditory pathways can’t be stimulated beyond a certain age.
The Irish Deaf Society (IDS) has no objection to cochlear implants but it does believe that parents of newly diagnosed children should be given more balanced information. At the moment they are only getting the medical view of being deaf, says Elaine Grehan, manager of the IDS advocacy service "Deaforward".
Recent research suggests, she continues, that some medical professionals actively discourage parents from learning Irish Sign Language to communicate with their child.
“This denial of sign language doesn’t make sense,” Grehan says. “Hearing baby and toddler groups promote the use of baby-sign as it is shown to reduce the child’s frustration at not being able to express themselves. It has been suggested that learning sign language can delay speech, but this is refuted by experts who claim that in fact, it aids speech development.”
Deaf culture
Regardless of whether their deaf child has been fitted with a cochlear implant, she adds, “we would encourage parents to meet with people in the deaf community. It’s a good way to get advice and learn about ISL, deaf culture and our community.”
Laura Grant is chairwoman of Our New Ears, a parents’ group that evolved out of the previous campaigning group. Her six-year-old son James was diagnosed as profoundly deaf at age two. Born before the introduction of newborn screening, James also just missed out on the introduction of bilateral implants. He had his first implant three years ago and his second 1½ years ago.
Early diagnosis through newborn screening gave children a chance to develop age-appropriate spoken language before they started school
James began to learn spoken language from scratch at age two; now he is finishing junior infants in a mainstream school in Dún Laoghaire and is doing well. His mother attributes this rapid progress after the implants to Auditory Verbal Therapy (AVT), which she attended with him in London because it was not available in Ireland.
“It is a parent-coaching programme emphasising the listening skills because the children need to be taught to listen,” Grant explains. “Their brains don’t make sense of the sound immediately.”
After one visit to Auditory Verbal UK, a charity that provides AVT to children, to find out what it was all about, she resolved not only to do it with James, but also to work to make it available in Ireland in the future: “I felt it was the missing piece for a lot of kids.”
For a year, Grant travelled once a month to sessions that cost £250 (€287) each – parents living in the UK get them free. Along with air fares and other costs, she reckons it was costing €600-€700 each time. But the therapy proved “totally and absolutely revelatory”. Since they were trained in the techniques, James “has been a transformed child”.
In 2015, Our New Ears, along with DeafHear, organised part one of AVT training for professionals in Dublin. Only audiologists, speech and language therapists and teachers of the deaf are eligible for this further qualification. One of those who signed up for that course was Rosie Gardner from Banbridge, Co Down.
A teacher of the deaf for 30 years, Gardner went along wanting to learn a bit more about it, never envisaging that she would pursue this speciality full time. The combination of technological advances and new research on the brain had made her question more traditional approaches.
Early diagnosis through newborn screening gave children a chance to develop age-appropriate spoken language before they started school. But Gardner observes that in the “rare cases” this was achieved, the parents were able to afford to go to London and even to the US to access therapy. That is what aroused her interest in AVT and made her determined to try to help provide it for parents here.
AVT focuses on activating the auditory part of the brain. We tend to think about hearing and hearing loss as being about ears, Gardner points out, but they are just the doorway to the brain. When no sound is getting to the brain, the brain starts to use that part for something else.
We now know from the research that between ages nought and 3½, the brain has the most plasticity so this time is crucial, she says. “There is a very, very short window to have the appropriate stimulation.”
Parents are key because an hour’s therapy in clinic once a week will not suffice. They are coached in how to work through daily activities to help a child’s brain begin to learn to listen – and to make sense of what they are listening to.
Last August, Gardner resigned as head of the service for hearing-impaired children in the NI Education Authority’s southern region, convinced that AVT and giving parents the skills was the way to go. She stresses she is still a trainee. “Parents feel empowered by it,” she says. “They can live the therapy, live the language.”
Gardner is currently working with eight or nine families in the North and a similar number in the Republic. One of them is the Glynn family in Mullingar, Co Westmeath.
Silent one
The first time Jean Glynn’s son William laughed was when his twin brother Nicholas laughed, at about 20 months old. “My heart burst with joy – he can hear!” Up to then, William had been very much the silent one of the pair. He was diagnosed at five months with auditory neuropathy spectrum disorder (ANSD), which has left him profoundly deaf.
The boys had twin-to-twin transfusion in the womb – an uneven distribution of the blood supply between identical twins sharing one placenta. William was the one whose organs were overloaded and was in most danger. He and Nicholas were delivered at 30 weeks in March 2015. They weighed three pounds and two pounds respectively.
There were no major issues with the smaller twin, and William’s problems seemed to be resolved after three months in neonatal care. Both had failed the newborn hearing screening, but Jean Glynn expected the follow-up would give them the all-clear. It did for Nicholas but not William.
Glynn was “gutted for a week” before setting about to see what would be the best path for her son. There was a slim chance the ANSD might resolve itself, so there was a delay, during which he had hearing aids, before he could be considered for cochlear implants.
Glynn knows that they are lucky to be able to pay for the therapy and, seeing its results in action, has no doubt of its value
Glynn was “lucky” that as a primary school teacher she had worked with children with speech and language disorders, including a child with bilateral cochlear implants. That child’s mother, along with other similar parents, were an invaluable source of information and empathy.
William did not receive his implants until he was 18 months but already his mother had identified AVT as an approach she wanted to follow. They had their first session at home with Gardner a month before his surgery.
Glynn knows that they are lucky to be able to pay for the therapy and, seeing its results in action, has no doubt of its value: “It has opened up a whole life for our child and accelerated his language.” The twins now have a six-month-old brother, Michael, and it is “fantastic” for William that he is now hearing all the early language stages he missed.
The morning we speak, Glynn had just asked him to pick up a tea-towel off the floor and put it in the laundry basket, and he had done just that. It is such accomplishments that give her the confidence that “all is going in the right direction”.
Sound Advice, an organisation founded by Caroline Carswell to promote the use of technology and digital tools for those with hearing loss, will present the short film 95 Decibels at the Irish Film Institute on June 10th. Starring Goran Visnijc of ER fame, it is based on a true story of how a family learned their toddler daughter was deaf and moved into a new world of cochlear implants. It will be followed by a Q&A with US producer Lisa Reznik and her 20-year-old daughter, who wears two cochlear implants, plus a panel of young adults who live in Ireland. Those who wish to attend must email hello@soundadvice.pro by June 8th.
swayman@irishtimes.com
In numbers
1-2 children per 1,000 are born with a hearing loss
90% of deaf children are born to hearing parents
4 years since universal auditory screening of newborns was introduced in the Republic
