Sound sense: Treating babies with hearing disabilities

There was no reason for Jane*, a 33-year-old American, to suspect  that there was anything wrong with her unborn baby’s hearing.

Like all women, she was advised to have an anomaly scan at 20 weeks, which also included a hearing test. But when the foetus didn’t respond, the doctor wasn’t worried and told  Jane it was sleeping.

The mother-to-be didn’t think anymore about it until her baby, Patty, was born. Just 48 hours old, the newborn failed a routine hearing test and US-based Jane and her husband David began to worry. 

The audiologist reassured them that a follow-up examination at ten weeks may eliminate the possibility of any aural disorder. Jane and David, both 35, have normal hearing and Patty responded to very loud noises so the couple dismissed their fears.

But they were devastated when the follow-up tests revealed their only child had been born deaf. “We couldn’t believe it,” says Jane. “But rather than giving up, we were determined to give her all the help we could to make her life as normal as possible.’’ Patty’s deafness could have been genetic or a result of prenatal infection, though the cause was never established.

After numerous tests to decide the best course of action, doctors suggested that Patty have a cochlear implant when she was a year old. The electronic device is surgically implanted and provides a sense of sound to a person who is profoundly deaf or severely hard of hearing.

They are often referred to as a bionic ear. Patty is two years old now and amazingly she can communicate just as well as any other child. “She loves singing along with her favourite characters from Sesame Street,” Jane smiles. “As soon as the programme comes on TV, she joins in.”

But the most meaningful moment for Jane came when Patty first said ‘Mama’ a year ago. “When she said it I really wept with joy,” says Jane. “My daughter, who previously could barely mouth a few words incoherently, was actually saying a proper word."

David too is visibly elated when he talks about how his daughter reacts to sounds and pronounces simple words. “It just fills my heart to see her talk,’’ he says. “What I’ve learnt along the way is that if detected early enough, hearing loss needn’t be an issue for any child.”

But that’s just the problem, says Paige Stringer, founder and executive director of the Global Foundation For Children with Hearing Loss. Many parents don’t realise that hearing tests – like the otoacoustic emission test – have such high sensitivity and produce accurate results for even newborn babies.

The OAE test measures an acoustic response produced by the inner ear (cochlea), which in essence bounces back out of the ear in response to a sound stimulus. The test is performed by placing a small probe that contains a microphone and speaker into the infant’s outer ear.

As the infant rests quietly, sounds are generated in the probe and the responses that come back from the cochlea are recorded. Once the cochlea processes the sound, an electrical stimulus is sent to the brainstem.

In addition, there is a second and separate sound that does not travel up the nerve, but comes back out into the infant’s ear canal – this is the otoacoustic emission. The emission is recorded with the microphone probe and represented pictorially on a computer screen. The audiologist can determine which sounds yielded a response and the strength of those responses.

If there is an emission present for the sounds that are critical to speech comprehension, then the infant has passed the test. Experts advise the test should be performed when the child is six weeks old for early intervention. Paige agrees, and she should know. She was born with permanent hearing loss and essentially couldn’t hear anything at all.

“I was a beneficiary of early intervention, thanks to an audiologist who spotted my condition right from the outset,” says Paige, who was in Dubai recently at the invitation of Yvonne Caldwell, an Abu Dhabi-based mother who has a son with a hearing condition.

Early intervention has not always been possible, in fact, in years gone by medical experts struggled to make a diagnosis until a child started talking, which was often too late.

“Prior to the 1970s, identifying children with hearing loss was extremely difficult until they started talking,” says Paige. “That’s the reason I started the Global Foundation – to help children in other parts of the world who don’t have the chance that I had.”

Catch it early

A baby diagnosed with deafness by the time it’s six months old can immediately be fitted with hearing aids, and can be on a par with their peers by their second birthday. If the diagnosis is delayed, it takes the child longer to match their peers’ hearing ability.

Indeed, delayed intervention carries a significant impact. Worldwide, there are around 20 million deaf children, 80 per cent of whom live in developing countries.

In India alone there are three million deaf children, according to Deaf Child Worldwide, the international development agency of the National Deaf Children’s Society (NDCS), UK.

A silent world can be an isolated one, and without the ability to communicate their wants and needs, babies and toddlers swiftly become frustrated. This ultimately affects their self-esteem, and because they can’t communicate, their speech is affected, and this may become even more challenging if the diagnosis comes when the child is older than six months, say experts.

Yvonne has witnessed first-hand the difficulties that children with hearing problems experience. Her seven-year-old son, Hamid Abdulla Al Harmoudi “fell through the system", she says.

Hamid, who has three sisters and two brothers (one of his brothers is his twin and has normal hearing) was only diagnosed at around 11 months. Yvonne still remembers the moment she first spotted the symptoms. “Hamid didn’t react when a door of the room in which he was playing in slammed shut," says Yvonne.

He underwent several tests in hospitals in the UAE. But the time between tests and completion of certain medical procedures meant that he had to wait until he was two years old until he got his first cochlear implant and was left unable to speak coherently.

“A cochlear implant costs around Dh120,000," says Yvonne. His second implant was done much later, when he was six years old. “So his speech developed at a very late age. However, now he speaks pretty coherently. We just have to keep working with him over and over again,” she says. They are hoping he will be able to catch up with his peers within a year.

Hamid undergoes speech and audio therapy every day at a centre in Abu Dhabi. “But our friend’s son was identified early and is progressing beautifully. If a child is identified within six months and intervention and therapy is started by 12 months the child can start hearing sounds and develop normally.”

While comprehensive figures for the UAE are hard to come by, the number of babies with hearing problems that have been referred from Corniche Hospital to the Sheikh Khalifa Medical City (SKMC) are “quite high”, according to Almas Naseem, marketing manager of Corniche Hospital.

“Hearing [tests for newborns] are performed on every baby born at Corniche Hospital in Abu Dhabi,” says Almas. “The screening can be performed six hours after the birth. However it is generally done as part of the neonatal screening 24 hours after birth or when the child is older, along with the congenital heart disease screening.

“We refer the babies that do not pass the screening test to the SKMC. Further audiology tests to define the hearing deficit are performed at other facilities following a referral. The Audiology Department was started in our hospital in January 2010, and since then,  328 babies have been referred to the SKMC from our hospital alone,” says Almas.

And that high number of baby referrals doesn’t come as any surprise to Yvonne.
“A doctor told me she sees between 13 and 15 children with hearing loss every day,” she says.

Determined that other parents who have children with hearing disabilities should not undergo the trials and tribulations that she had to, Yvonne began to research more on the subject, which led her to Paige and her work with the hearing impaired.

She invited Paige to her home in Abu Dhabi to talk about doing something to help. Paige set up her foundation in 2008 in the US out of a desire to help children less fortunate than her.

“I went to a regular school and I was one of the first children [with my condition] to do that,” says Paige. “I learnt sign language, but never had to use it. I went on to get a university degree in forest resources conservation and a masters degree in management. I was extremely fortunate. That’s why I am so passionate about the cause. All children should have that support.”

You would never suspect that Paige is hearing impaired. “It has never been a detriment for me,” she says. “I worked with Amazon.com for many years, and did a lot of freelance writing. I began to consider what I could do to give back to society. Then I came across a volunteer-vacation programme that offered a working holiday for a month assisting at a school for hearing-impaired in Vietnam.”

The programme was to prove life-changing for Paige. “The school that I was assigned to had a director who’d just come back from Europe where she’d learned about early intervention and breakthrough technology in hearing aids,” she says.

“She was trying to educate people in Vietnam that they needed to start intervention and training early for children with hearing loss.” Usually, the children were six years old by the time they came to school.

“By then it was too late, they’d already lost six years of language development which is hard to make up,” says Paige. “Although I did teach English to the children, what the director of the centre did was showcase me as a case for early intervention – she made me talk to everybody who mattered to demonstrate why it was so necessary to get treatment early in life. I too learnt a lot from her – how to push forward despite challenges and how to educate people using real-life models.”

At the end of her trip, the director asked Paige to train the teachers in the 50 schools for the deaf in Vietnam in early intervention. “They could then train others so that education of children with hearing loss would improve,” she explains. “That was essentially the beginning of my project to bring back experts to train the people in Vietnam on how to do that. Our model, begun in 2009, is unique in that we don’t focus on only one thing but look at the whole picture.’’

They don’t look at the child in isolation but at the parents, caregivers, school and community and how all can come together to help children with hearing conditions.
 
“We’ve brought over 15 professionals from US, Hong Kong and Singapore to Vietnam. We work with teachers on therapy and education for children under six years of age.”

Since summer 2010, the foundation has trained over 190 teachers, 220 families, and 120 medical and other professionals. The foundation raises funds for its activities in Vietnam through donations. “We have fitted 246 hearing aids on young children in Vietnam,” says Paige. “Our efforts are impacting over 900 children with hearing loss.”

What makes the foundation’s Vietnam Deaf Education Program unique – besides its scale of 38 schools and two hospitals across 20 Vietnam provinces – is its multi-disciplinary and integrated approach.

“Our programme provides professional development and training across audiology, speech pathology, early intervention, and auditory-verbal practice for families and professionals who have or work with children with hearing loss,” she says. “We also provide hearing aids to young children.”

During the foundation’s summer training, Vietnamese professionals representing multiple disciplines attend the workshops at Thuan An Centre in Binh Duong province in Vietnam and families engage in consultations and lectures.

“During the year, we travel to various locations as part of our Mobile Mission series to provide audiology and auditory verbal practice coaching and support,” says Paige. “We work with the same group of schools, clinics, centres, and hospitals over time to ensure the participants build on their skills and that the children progress in their auditory development.”

Three years since they started work in Vietnam, people there are demonstrating a progression in understanding and expertise for helping children with hearing loss learn to communicate through listening and spoken language. And, because the participants are sharing their knowledge within their communities, the benefits are exponential and sustainable.

Now the global foundation has spread its activities to the Czech Republic and Guatemala. And if Yvonne’s plan to start an organisation in the UAE takes shape soon, Paige will be extending her activities here too.

Yvonne, who’s been working on the plan for two years now says, “The academy or centre will specialise in developing and delivering concept training and education and an educational path which will allow hearing-impaired children to eventually be integrated into the public school system in line with the Abu Dhabi government’s 2030 strategic vision.”

She envisions the centre working in coordination with recently introduced world-class medical facilities in Abu Dhabi at the Shaikh Khalifa Medical City, among others, to assist in providing early detection of hearing loss and a formal programme and information on individualised intervention at home.

“We also hope to provide education services through each developmental stage, instructor-led training, consulting, course design and development services to organisations,” says Yvonne.

The facility will also offer guidance, support and instruction for parents in the special skills necessary to help their children learn spoken language. “We also hope to eventually offer distance education serving parents and families throughout the Middle East and North Africa who are unable to relocate to Abu Dhabi,” says Yvonne.

With the right interventions, a deaf child can happily attend a mainstream school. “There is nothing wrong with their brain,” says Paige. “Making that investment is well worth it as the pay-off is tremendous. When they grow up they are going to be able to contribute to society fully.” Jane has the last word. “People often comment that Patty seems so normal. She is; she just can’t hear.”

Why are babies born deaf?

Deafness may be caused by any number of factors, say Dr Susan Waltzman, codirector of the NYU Cochlear Implant Center. “The cause, most often, is something that occurs during the intrauterine period.”

Viruses during pregnancy may play a role, as may maternal drinking or smoking or a number of syndromes, as well as premature birth or the parents’ family history.

Further risks may be posed by common childhood diseases and even certain medications. Recurring middle ear infection can cause temporary residual damage to the middle ear, so treatment and management options should be explored as soon as possible, says Dr Waltzman.

The problem is that if parents are unaware of these risk factors, they may not know their child’s hearing has been compromised. “Parents don’t realise that even if a child can hear a loud sound, like a door slamming, they may not be able to hear very soft sounds, like those present in speech,” she says.

“Hearing loss is an invisible challenge, so the early signs are easy to miss. Normally, you’d notice that the baby doesn’t startle at loud sounds, or may get a fright when you enter his visual range because he hasn’t heard your footsteps. Very young children may not be soothed by their parents’ voices.”

Very often, though, parents dismiss such signals as “a phase”, and worry only when their child does not start talking at the appropriate stage.