Guest blog: Parents give daughter with Treacher Collins Syndrome the gift of hearing through bone conduction

Six months before writing this, father Emre, 31, and mother Öznur, 30, welcomed “miracle baby” Elif into their new family. Emre wrote Elif’s story from Istanbul, Turkey, where he works as a customer manager in a private company, and Öznur works as a banker. Emre’s story has been translated and edited from its original form in Turkish.


Learning about our baby’s syndrome and making an important decision

Elif sits with her parents while wearing her first Baha device, a Baha Intenso sound processor.

Our daughter Elif came to the world with Treacher Collins Syndrome, a rare genetic disease. Treacher Collins is a genetic condition that causes deformities of the face and head, often characterized by low eyelids, full ears and an underdeveloped jaw. In some cases people with Treacher Collins Syndrome may also have cleft lip and palate. One baby in every 50,000 births has Treacher Collins Syndrome.

We first learned about this syndrome during my wife’s pregnancy. During the routine examination in the 18th week of pregnancy, the doctor found out Elif had a double-sided cleft lip. One week later, the doctors detected double-sided cleft palate and a double-sided microtia (absence of external ears). Additionally, it turned out that the baby’s jaw was small and pushed back. The doctors suspected some of the possible explanations, including Treacher Collins Syndrome, and mentioned the risks at the same time. Later on, we discussed our opinions on whether to continue the pregnancy. We asked ourselves many questions at this point, and the answers we gave to these questions showed us that Elif should come to the world.

Can happiness be defined?

There is a general assumption that an individual who has physical deformaties will suffer and will be unhappy. Can happiness be defined? We believe that happiness is not in the physical world but in the heart. On the other hand, we believe that every life is sacred. We considered every effort to provide a real life for a physically impaired individual to live, first of all, as a human being. Life evolves in meaningful way, for us and our daughter, and the struggle itself is meaningful. We started to wait for our miracles.

Elif is born and begins to hear

Elif could not breathe on her own, because her jaw was small and pushed back, so she was immediately connected to the respirator and stayed in intensive care for 62 days. At the end of this process we came home with the breathing apparatus. In the second month of her development, Elif was completely free from the respiratory device for life-long use.

Doctors discovered that while Elif has no problem in her inner ear, she has hearing loss in the middle ear. She first began to hear with the help of a Baha Intenso sound processor, brought to us from the United States. Elif showed a noticeable improvement in her perceptions and reaction to her surroundings wearing her Baha device on a Softband. She now wears a Baha 5 Sound Processor.

Solidarity with parents and individuals with Treacher Collins Syndrome

Elif wears her first Baha hearing device, the Baha Intenso sound processor, on a Softband.

After our daughter came to this world with this syndrome, we wanted to find people who have similar situations and to learn from them. I have communicated with various associations and groups abroad on social media. We received very useful information and positive support messages, and we saw the power of the love beyond boundaries.

While Elif was in intensive care, when we were not with her, we found other affected families in our country through our own efforts and visited a significant number of them. Later, we began a meeting with some of the families with Treacher Collins living in Turkey. In the name of starting something new, we have built solidarity among ourselves.

The biggest problem facing people living with this syndrome is others’ negative attitudes and behaviors exposed to them by society. However, the only expectation these individuals have is to live like normal individuals in society and to not be exposed to others’ attitudes because of their external appearance.

Elif’s journey on social media

We share the processes of Elif’s journey in Turkish on the social media accounts that I set up in Elif’s name. Anyone who wants to support us or contact us can reach us at these links on Facebook or Instagram. We are also excited to announce that in September we will publish a book about Elif’s journey.

Every individual is valuable

We are beginning our awareness work with an enthusiastic amateur spirit, and our goal is to raise awareness that every human being is valuable just because they are human, to be able to feel that every person can overcome their challenges and that their value is not based on their appearance to others.

The opinions expressed in this blog are my own views and not those of Cochlear.


Click here to learn more about the importance of early intervention in a child’s hearing loss. Looking to take action on hearing loss? Click here to find a clinic near you.


Want to share your story, hearing tips or Baha advice with The Baha Blog? Let us know! Find us on Twitter at @TheBahaBlog, on Facebook at our page The Baha Blog or via email at bahablog@cochlear.com.

Guest blog: How the Baha System helped one mom take early action to treat her infant daughter’s hearing loss

Guest blog: How the Baha System helped one mom take early action to treat her infant daughter’s hearing loss

Hearing loss can be challenging for a newborn trying to reach developmental milestones, but mom Ashton M. decided to treat her daughter’s hearing loss, due to microtia and atresia, right away. Hazel, who just turned one year old, wears her Baha 5 Sound Processor as she begins to grow up like any other baby.

 Ashton wrote to the Baha Blog to share Hazel’s story, and to provide a few tips for other parents who are considering treating their children’s hearing loss with bone conduction and the Baha System.


At one month old, Hazel has an Auditory Brainstem Response (ABR) test performed to measure her hearing.

On June 6th, 2017 at 2:00 in the morning, we were truly blessed with a perfect little girl who we named Hazel. Her dad Mike and I were over the moon to finally see her after nine long months.

With all the excitement of the delivery, there were a lot moments that seemed like a blur to me. After they weighed and measured her I finally got to hold her in my arms. I was just overcome with happiness.

In that moment I will never forget when Mike asked me if I had looked at Hazel’s left ear. I immediately lifted the cap that newborns wear and began to examine her. The nurse in me started to run down a list of possible diagnoses in my head, and then the worried mother began to set in as well. I didn’t see an opening in her ear canal, and I noticed her ear was not fully formed. Those first few days of Hazel’s life were both exciting and scary for Mike and me.

We learned that Hazel was born with a congenital condition called microtia with atresia, affecting her left ear. Microtia means she has a small outer ear, and atresia means she has no external ear canal. We were then told she was not able to hear out of her affected ear, but she tested perfect with her right ear during a screening.

Hazel is fitted with her first Baha 5 Sound Processor at 6 months old.

The day we walked out of the hospital to take Hazel home, I could hear the birds chirping and all the noise around me – and it had never seemed so loud. I covered my left ear and tried to put myself in my brand new daughter’s shoes and I began to cry. I wondered and asked myself if she will ever hear like I do.

Then came many appointments with an ENT (Ear, Nose and Throat clinician) and an audiologist. The moment we met our audiologist was the first time that I felt, “Hey, Hazel is going to be just fine!” Our audiologist performed another hearing test on both of Hazel’s ears called an Auditory Brainstem Response (ABR). Hazel’s right ear showed normal hearing sensitivity and her left ear showed moderate to severe conductive hearing loss. Our audiologist began to tell us our options from that point and educated us on a bone conduction hearing device that will allow Hazel to experience bilateral hearing.

We gave the OK to start the process of getting her fitted for her Cochlear Baha 5 Sound Processor, a bone conduction hearing device. Then the day finally came! When she was about 6 months old, she received her sound processor, which she wears on a Baha Softband headband.

She was a little overwhelmed and frightened, hearing for the first time out of her microtia ear. Until then all she ever knew was being able to hear from one side, so it took a few days for her to get used to the change. It has been a challenge adapting to the milestones of growing up as she wears her Baha sound processor, but we could not be more thankful for the brilliant minds behind the development of this device.

Hazel wears her Baha 5 Sound Processor on a Softband, adorned with a small bow.

As an infant wearing a Baha sound processor, Hazel faces a few challenges that we have to navigate, and throughout this process we’ve found some useful tips for parents like us. Here are a few tips for parents of infants wearing a Baha device:

  • Rolling on the floor sometimes leads to feedback issues. When Hazel is on her back or when she wants to roll around, we rotate the Softband so that the sound processor is on her forehead, preventing feedback.
  • Since she was very young we have put headbands on her, which has helped her get used to wearing something on her head. This has definitely been useful as she adjusts to wearing the Softband for extended periods of time.
  • Gaining more self-awareness means she wants to play with her clothes, or even pull the Softband off whenever she gets a chance. Once we put the Baha 5 Sound Processor on her, we immediately do something to distract her. When it’s out of sight, out of mind, she gets better at wearing her processor for longer periods.
  • When she is not wearing her sound processor, we also use a glasses case to store the processor while it is attached to the Softband. The white storage box that comes with the Baha 5 Sound Processor is the best solution for storing the processor on its own, but the glasses case allows more room for both the sound processor and the Softband while they are attached. As an added bonus, Hazel’s hands aren’t yet strong enough to open the case on her own.
  • So that you can easily open the Baha 5 Sound Processor’s tamper-proof battery door, designed to prevent children like Hazel from accidentally removing the battery on her own, we recommend you also store the tamper-proof tool provided in the sound processor box, or a small hairpin, together in your storage case.
  • It is also a good idea to remove the battery and store your child’s sound processor overnight in Cochlear’s Dry Aid Kit or a drying box like The Breeze by Dry & Store. Infants are messy, and doing this helps to keep moisture out of the sound processor.
  • For more tips on living with the Baha System, click here.

We are so proud of our little Hazey and so excited to see what the future brings for her! As a family, we are excited to learn about these medical advances and be on this amazing journey with the Cochlear family.

I know now that she hears every little birdie tweet, and every outside noise there is!

The opinions expressed in this blog are my own views and not those of Cochlear.


Click here to learn more about treating hearing loss in children.


Want to share your story, hearing tips or Baha advice with The Baha Blog? Let us know! Find us on Twitter at @TheBahaBlog, on Facebook at our page The Baha Blog or via email at bahablog@cochlear.com.

Teacher Irene M. hears children more clearly in the classroom since upgrading to Baha 5 Power

Teacher Irene M. hears children more clearly in the classroom since upgrading to Baha 5 Power

Irene was diagnosed with hearing loss after a viral infection in 2010. After five years with the Baha Intenso sound processor, she upgraded to the Baha 5 Power and is thrilled she did.

Irene was diagnosed with hearing loss after a viral infection in 2010. After five years with the Baha Intenso sound processor, she upgraded to the Baha 5 Power and is thrilled she did.

Irene works teaching children, which can be a challenging task with a hearing loss. For anyone, trying to get children to speak slowly and clearly — and to be patient with the listener — can be tough. But now that Irene has upgraded to the Baha 5 Power sound processor, she said she can hear the children at her primary school more clearly.

Irene, 31, was diagnosed with hearing loss after a viral infection in 2010. After she got her implant surgery, she was fitted first with the Baha Intenso sound processor. Five years later, she upgraded to the Baha 5 Power and is thrilled she did.

Watch this video to see more of Irene’s story about why she chose to upgrade:

 


Want to share your story, hearing tips or Baha advice with The Baha Blog? Let us know! Find us on Twitter at @TheBahaBlog, on Facebook at our page The Baha Blog or via email at bahablog@cochlear.com.

Tips and tricks: How to wear and customise your Baha SoundArc

Tips and tricks: How to wear and customise your Baha SoundArc

SoundArc_colors2

The Baha SoundArc is the newest hearing solution for people who live with conductive hearing loss, mixed hearing loss or single-sided sensorineural deafness (SSD). It is the world’s first non-surgical, behind-the-head bone conduction hearing device, specially designed for children who are not ready for a bone conduction implant and adults who want to trial bone conduction in everyday situations.

Whether you’ve got a SoundArc already or you’re ready to learn more about it, these how-to videos give great tips on how you can get the most out of the SoundArc.

soundarc_topshot_all_colours3_PPTThe Baha SoundArc comes in a range of sizes and features your choice between a variety of coloured tips, where available. You can choose to match your clothing, your hair or your mood. Choose between black, grey or brown tips to discreetly match hair color — or opt instead for green, pink or turquoise tips to add style to an outfit. Watch the first video below to learn how to change the coloured tips.

Don’t worry, your hearing care professional will ensure your Baha SoundArc has the proper fit when you first try it on. Still, this next video is a useful reference for when you need to take it off and put it back on throughout your demo period, or if you or your child is wearing it for a longer term. Watch the video below to learn how to properly wear the SoundArc.

If you are wearing two sound processors on your SoundArc, your hearing care professional will set up your SoundArc for bilateral use. You can refer to this last video if you need to adjust or remove the connector discs for any reason during the period you will be wearing the device. Watch the video below to learn how to assemble the Baha SoundArc for bilateral use.


LEARN MORE: Click here to read more about the Baha SoundArc, ideal for use as a demo device, or for children not yet ready for an implant.


Want to share your story, hearing tips or Baha advice with The Baha Blog? Let us know! Find us on Twitter at @TheBahaBlog, on Facebook at our page The Baha Blog or via email at bahablog@cochlear.com.

Meet the team who helped create the new Baha SoundArc: videos

Meet the team who helped create the new Baha SoundArc: videos

Meet the team who helped to innovate the new Cochlear Baha SoundArc.

Meet the team who helped to innovate the new Cochlear Baha SoundArc.

Have you ever wondered what goes on behind the scenes as Cochlear develops the latest hearing tech? If you’re curious about the new Baha SoundArc, Cochlear’s newest non-surgical bone conduction solution, look no further. In the two videos below, the teams behind the SoundArc describe the challenges and successes of this totally new design.

In the first video, listen to Henrik Fyrlund (Senior Project Engineer Technology Development), Stefan Magnander (Senior Mechanical Engineer) and Fredrik Boivie (NPI Project Leader) describe the challenges of designing the SoundArc as well as key moments in its development.

 

In part two, watch Linnea Agostino (Product Manager) and Jenny Andersson (Clinical Research Audiologist) talk about some of the features that make the SoundArc special when compared to other non-surgical bone conduction solutions.


LEARN MORE: Click here to read more about the Baha SoundArc, ideal for use as a demo device, or for children not yet ready for an implant.


Want to share your story, hearing tips or Baha advice with The Baha Blog? Let us know! Find us on Twitter at @TheBahaBlog, on Facebook at our page The Baha Blog or via email at bahablog@cochlear.com.

‘A whole world opened up to him’: Celebrating a new life of sounds this Autism Awareness Month

Parents of children with autism can have unique challenges in diagnosing and treating their children’s hearing loss. For Autism Awareness Month, blogger Jen B. from Oregon, U.S. has written about her 3-year-old son Xander’s hearing journey, from difficult diagnoses to helping him learn to wear his new sound processor.

You can follow Jen’s blog here on WordPress or here on Facebook.

 


“Xander!” “Xander!” “Xander!?”

But no response did I ever get from my son.

Xander, now 3, plays at school.

Xander, now 3, plays at school.

He will turn on a dime if he hears his favourite theme song start up. He’ll sing along in perfect pitch. And yet, dogs barking right next to him never make him flinch.

My son was born three weeks early with a collapsed lung and rapidly dropping sugars. Very soon he was transferred to a hospital with a Neonatal Intensive Care Unit (NICU). When I first saw him it was obvious that there was a slight deformity of his right ear. I assumed he had lain on it wrong in utero, and that it would work itself out.

The hospital gave Xander a hearing screening, as was standard procedure for all NICU babies. The nurse came in while he was asleep on me and checked his left ear first, because that’s always, even now, the ear he has to have facing out while snuggling.

Everything was fine.

The nurse tried twice to check his right ear and still seemed perplexed, because she couldn’t get the readings she was used to. Ultimately, she marked it as a pass.

It was only later we learned Xander’s hearing wasn’t normal.


My son has Autism Spectrum Disorder. He was diagnosed at only 18 months old. He is now 3 years old and considered non-verbal. He speaks, but not necessarily with what professionals would call, “purpose.”

Autism affects all forms of communication. Where a neurotypical child with a hearing loss might pull at their ear, say they can’t hear, or make some sort of gesture – something — we got nothing. It gradually became clear he needed to have his hearing assessed.

At about 2 years old Xander finally saw an audiologist. Autism can make a child both seek out stimulations (sensory seeking) and feel overwhelmed by them (sensory overload), so diagnosing hearing loss can be a challenge. In a room with all sorts of things making noise and lighting up, Xander wasn’t very cooperative. His tubes were clear, but the audiologist scheduled him for a sedated Auditory Brainstem Response (ABR) hearing screening. All the worst-case scenarios came into my mind during the scan, which felt agonisingly long.

Jen poses with 3-year-old Xander, wearing his Baha 5 Sound Processor on a Softband.

Jen poses with 3-year-old Xander, wearing his Baha 5 Sound Processor on a Softband.

The doctors learned Xander had conductive hearing loss in his right ear, and to learn more he would need a CT scan of his temporal bone. The doctors decided further diagnosis was not urgent and told me things like, “just speak to him on the left side” and, “in cars he probably won’t be able to hear you.”

About six months later Xander need an MRI for another issue, so we decided to have the CT scan done at the same time while he was sedated. We learned he is missing the third stapes, or stirrup – one of the bones of the ear – on his right side, a rare condition.

There is a surgery to implant a prosthetic stapes, but Xander’s major facial nerve runs where the bone should be, making the operation too risky. We decided instead to treat Xander’s hearing loss through bone conduction.


The sensory sensitivity caused by Xander’s autism made it hard for him to wear his first sound processor. The constant vibration against his head, the increase in noise and the strap around his head were difficult for him to tolerate. I would try bribing him, like I do with his glasses. “If you put it on, you can have a candy.” It didn’t help much.

When Xander’s trial was over, he received his Cochlear Baha 5 Sound Processor, and we both loved the way it looked. When I pointed out how cool he looked wearing it on his Softband, he beamed with pride and wore it for several days, for long stretches at a time – even to school!

Believe me when I tell you that I cried when I said, “Xander!” and he looked up at me.

He was able to hear the start of his favourite show in the whole world. He was mesmerized. He had never noticed all the nuances of it before he got his Baha processor. He could understand enunciations. He could hear his electronic toys, the ones that had never interested him before. A whole world opened up to him.

But for a child with autism, that can be really intimidating.

After an accident left him injured, Xander has become even more sensitive to stimulation, and sometimes getting him to wear his sound processor is nearly impossible.

Well, every day is a different day in the world of autism. While trying to take a photo of him wearing his sound processor, I was able to capture a whole video. He let it stay on while cruising around a department store today. I was so excited, and I hope tomorrow he’ll leave it on even longer.

For children with autism like Xander, treating hearing loss might not be a linear journey, but each day brings new opportunities for him to listen to his teacher at school, his toys, and even respond when I call him, “Xander.”

The opinions expressed in this blog are my own views and not those of Cochlear.


If your child is showing signs of hearing loss, use this tool to find a clinic near you.


Want to share your story, hearing tips or Baha advice with The Baha Blog? Let us know! Find us on Twitter at @TheBahaBlog, on Facebook at our page The Baha Blog or via email at bahablog@cochlear.com.

When losing something leads to something ‘wonderful’: Dwight’s experience upgrading to the Baha 5 Sound Processor

When losing something leads to something ‘wonderful’: Dwight’s experience upgrading to the Baha 5 Sound Processor

This blog was adapted from its original article on Hear and Now, a Cochlear Americas recipient blog. Read it here.


Dwight J. of Colorado streams a phone call directly into his Baha 5 Sound Processor.

Dwight J. of Colorado streams a phone call directly into his Baha 5 Sound Processor.

Sometimes losing something important to you turns out to be surprisingly “wonderful.” Just take it from Dwight J. in Colorado, U.S.

After a day of hiking, Dwight noticed he could not hear as well as he was used to. He reached up and discovered his BP100 Sound Processor was gone. He looked everywhere for it, but he realised he must have lost it while out on the hike.

Dwight acted quickly and called Cochlear to get a replacement. To his surprise, the representative told him Cochlear no longer offered the BP100. They introduced Dwight to the Baha 5 Sound Processor, and he knew he wanted an upgrade.

“It’s wonderful,” Dwight said after upgrading.

He said he especially likes being able to connect to his sound processor with Bluetooth, stream phone calls and stream radio directly to his sound processor while out on a walk.

Dwight also said he carries his Cochlear Wireless Mini Microphone with him wherever he goes. “I put it on whoever I’m talking to, if I’m in a loud situation, so that I’m able to hear them,” he said.

When it comes to continuing his journey as a part of the Baha family, Dwight seems thrilled.

“Cochlear culture oozes with a four-letter word: care,” he said. “They care about people.”

Dwight is a member of his local chapter of Cochlear Community, a Cochlear Americas program that connects Cochlear recipients in the U.S.


Want to share your story, hearing tips or Baha advice with The Baha Blog? Let us know! Find us on Twitter at @TheBahaBlog, on Facebook at our page The Baha Blog or via email at bahablog@cochlear.com.

 

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