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.

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.

How the Baha System helped Jamie pursue her love of music: guest blog

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


 

Jamie G. has conductive hearing loss, and the Baha System has helped her pursue her love of music.

Jamie G. has conductive hearing loss, and the Baha System has helped her pursue her love of music.

At the age of 14, I was asked by my otolaryngologist what I wanted to be when I grew up. Excitedly, I told him I wanted to be a singer. He then explained that music wouldn’t be a good choice for me.

I developed many ear infections and battled a cholesteatoma in my early years. I lost most of my hearing in my left ear even after numerous reconstruction surgeries. Because of this, my ability to sing should be out of the question. Shortly after that, I sent him a tape of my recordings. After a listen, my doctor wholeheartedly supported my decision to pursue music.

Hearing loss certainly didn’t make my life easy. It made me withdrawn, depressed and a shy girl in school. But music was one avenue that made me feel confident and secure. I loved feeling the sounds of the bass in my chest as I listened to my favorite artist, the drums vibrating in my feet, or the soprano’s high notes bringing my hearing to clarity. In order to perform, practicing music had to consume most of my time, but I didn’t mind. Reading music, counting rhythms and knowing chord progressions took time, but it was vital in order for me to be successful as a musician.

Thankfully, I outgrew the ugly battle with the cholesteatoma. Sadly, my hearing could never be restored. Hearing aids were not an option due to my ear’s inability to move any drainage or wax. So for 30 years I adjusted my life around my hearing loss.

In July 2015, I was referred to an ENT-otolaryngologist in Kansas City for a second opinion. That visit changed how I looked at my future. I was a strong candidate for the Baha® Implant System due to my conductive hearing loss. It didn’t take much thought to know that the Baha System was the right decision for me.

My switch-on date was December 2017. I slowly adjusted to this new, but amazing world. My world of quiet was replaced with new sounds. I could now hear that thumping bass line without having to just feel it in my chest. I could hear my voice not only when I sang those soprano notes, but I loved to hear the rich lower tones of my voice now. I didn’t have to make sure I performed in a certain spot on stage. I had the freedom to move wherever I needed to be. I still prefer to be near the drums, feeling the music in my feet and chest while I express my songs.

I chose Cochlear because of my musical lifestyle. I needed access to streaming without a separate device. But I also wanted to connect my device to any in-ear monitor system while performing. I knew I needed the best technology for my device to function in all areas of my life, so I choose the Baha 5 Sound Processor.

Jamie G. uses the Baha System to help explore her love of music.

Jamie G. uses the Baha System to help explore her love of music.

When I first got it, I made sure my settings were adjusted to my liking, and it took a few trips to the audiologist to get it just right. I love the ability to switch modes. When I listen or perform, I need my Baha 5 Sound Processor to adjust to the sound of the music. The tones I want when I sing or listen to music are certainly different that my daily mode.

I use my Cochlear True Wireless Mini Microphone every time I perform with my monitor system. Using a Y-adapter plugged into the monitor, I plug in my Mini Mic to one jack, my in-ear headphones to the other. Once I pair my Mini Mic to my Baha 5 Sound Processor, I hear every tone of the piano, the full sound of the acoustic guitar, the intricate bass line, my vocals moving in progression with the band…all in stereo – in both ears!

My musicianship has improved and excelled in a way I only could dream of a few years ago. I can now lead musically, knowing exactly what needs to be done to move the band to a more unified and excellent sound.

Because of my increased musical abilities with the Baha 5 Sound Processor, I got to hit the studio for my very first recording a year after my implant. While using my Baha System, I recorded a CD with a full live band. It was a beautiful experience to see and hear my own songs come alive and to be able to hear them fully and in true form.

Hearing loss made me appreciate my gift of music through expression. But the Baha System has given me the gift to hear that expression fully.

I look forward to more recording sessions and producing many more songs that have yet to be written! To hear a sample of my music or to purchase a download of my CD, you can visit jamiegroshart.com.

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


 

If you are dealing with constant ear infections or a cholesteatoma impacting your hearing, click here to find a solution to help you get better hearing.


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.

World Down Syndrome Day and hearing loss: Celebrating children like Chloe

The following is a re-post of a guest blog from 2014 about Chloe, who was born with Down syndrome and lived with hearing loss until she was implanted with the Baha System at 10 years old in 2010. Chloe was 14 when her mother Jane wrote this guest blog to share with us the affect Chloe’s Baha implant had on her life. Join us in marking World Down Syndrome Day today (21 March) and celebrating children like Chloe.


It’s been a while since you last heard about Chloe. She is now 14, in year nine at senior school and even more of a little madam than when we last met four years ago.

Unfortunately Chloe’s health hasn’t been very good the past two years. She has had some major operations which have left her bed bound for weeks, and in and out of hospitals. Chloe has shown incredible strength and determination, both because that’s the type of girl she is and because she was able to communicate effectively with hospital staff and myself.

This is all thanks to her Baha sound processor. Chloe’s language skills and attention skills have flourished over the last four years; her vocabulary has grown, she is learning new words each day and she is definitely more attentive to those talking to her (apart from when she chooses not to!). This meant the hospital staff was able talk her through what was going to happen and she could indicate her pain levels and talk to the doctors.

I dread to think how she would have coped six or seven years ago, when her hearing levels were so low, and she hardly had any language. The world would have been a very lonely and scary place for her.

Chloe still loves drama and attends a very good secondary school that specialises in drama so she can devote more time to her passion. She is still my drama queen and a budding actress, dancer and musician. Obviously she is becoming a young lady and her life is changing. Developing language and communication skills has played a big part in this and will continue to do so.

My one aim for my daughter has been to become as independent as her disability would allow her. The Baha [System] has contributed to the success of this aim. She can order her food in restaurants. She can shop at the supermarket. She is also very good at being a typical teenage and slamming doors on me!

So am I pleased with the Baha [System]? Yes, my only regret is that I didn’t push for it sooner. I would recommend it for any parent or young person who needs it. Read the literature, speak to the professionals and if you feel it right go for it! It opens many doors and not once have we looked back.”

Jane lives in Birmingham, UK. In 2013 she won the Birmingham Parent of the Year Award for her tireless work with a special needs support group, as well as caring for her own two children who both have special needs.


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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