After Willow failed her newborn hearing screenings, her family sought further testing with hearing health professionals. During this process, they discovered that Willow had hearing loss in both ears due to Waardenburg syndrome, a genetic condition. Following the recommendation of their hearing health professional, Willow received cochlear implants. Now, she is thriving as a child, embracing the world of sound. Below is Willow’s inspiring hearing journey:
In 2019, our worlds changed forever. Our beautiful baby girl was born. We named her Willow. For months we had talked about how our lives were going to change and imagined how wonderfully different life would be. She was so beautiful and so perfect.
Every screening at the hospital indicated that she was healthy, but she failed her newborn hearing screening. We were told that it was likely just fluid in her ears, with a plan to return in two weeks for one final try.
After failing once again, we decided to have her referred for an auditory brainstem response test (ABR). At that point we weren’t too worried and the technician still believed it was just fluid. However, my husband had some hearing loss due to a buildup of fluid in his ears as a child and I had profound hearing loss in one ear for unknown reasons, so we knew it was for the best.
We were scheduled to have Willow’s ABR done in March 2020, when boom, the world shut down. Thanks COVID! We ended up rescheduling later in May. During the time leading up to that appointment, I considered canceling as Willow seemed responsive – she was always so focused and (visually) tuned into her surroundings.
Suspicions of a potential diagnosis for our daughter
Thankfully, we kept the appointment. A few days before the ABR, I was holding Willow in the sunlight when I noticed that her eyes appeared to be different colors. At 5-months-old, it still was subtle. I had never heard of such a thing and I’m not one to research symptoms online but my curiosity got the best of me. Most results lead to Waardenburg syndrome but I didn’t read much into it.
The day of the test had finally come and they only allowed one parent to come in with Willow; that happened to be me. We went in, met the audiologist, I rocked Willow to sleep and we proceeded with the ABR test. The audiologist finished, looked at me and said ‘she has profound hearing loss in both ears.’
My heart dropped, and I felt a feeling of shock fall over me. She immediately brought up Waardenburg syndrome and suggested that we have genetic testing done, because not only did Willow have hearing loss, but she had the most beautiful white forelock at that time and heterochromia, which are three big indications of Waardenburg syndrome. Meanwhile, as I’m having a nervous breakdown on the inside, my husband is waiting outside and has no clue.
The audiologist started discussing how Willow would likely need cochlear implants around her 1st birthday but first she would need to have a hearing aid trial. That day and the days following were ones of heartbreak, anxiety and denial. We were grieving all of those things we thought Willow might not ever be able to experience, some of our favorite sounds such as waves breaking on the coastline, rain, the loud booms of a thunderstorm, the trickle of a stream, bacon sizzling in a pan, deep belly laughs, music and the words ‘I love you.’
Early intervention helped us all
I remember my mom telling me ‘We are going to become better people because of Willow.’ Little did I know how right she would be. We began working with an amazing speech language pathologist/ teacher of the deaf and hard of hearing through Early Start, an early intervention program. She showed us so much empathy, compassion and support and she was incredibly knowledgeable. She was everything we needed during that time.
We first started out by learning and teaching Willow American Sign Language (ASL). She picked up on it very quickly, especially for how young she was. At 6-months-old, Willow received her hearing aids. I remember the audiologist and our speech pathologist stressing the importance of keeping them on during all waking hours, even if we weren’t noticing much of a difference. The purpose was to get her used to wearing something on her ears and head and to help stimulate the auditory nerves.
We took it very seriously and we did notice Willow was hearing some things. She was now 7 months old and we felt it was time to get a second opinion from a more reputable facility that specialized in hearing health. We first met with the audiologist. She blew us away and we instantly knew we made the right choice.
We got Willow in a sound booth to see how well she could hear with the hearing aids versus without. It was something we had never done before. The hearing aids put her in the medium to severe hearing loss range and without them, she still had profound loss. It warmed my heart to confirm that when we thought Willow heard a particular sound, she had been hearing it, but it still wasn’t enough for her to be able to understand speech.
The next step: cochlear implants
The audiologist suggested cochlear implants as soon as possible. We immediately got the process going, meeting with the ear, nose and throat doctor (ENT) that would be implanting Willow. We also had to decide which company we wanted to use for the cochlear implants.
Our clinic identified two companies we could choose from, so we came up with a long list of questions to ask and began interviewing each one. We ended up choosing Cochlear for a few reasons: The first one was the sales rep – she seemed to genuinely care about Willow and connected us to a mother, who had a daughter that was bilaterally implanted. They also didn’t make a big sales pitch; they simply stated the facts, connected with us and allowed us to make an informed decision.
Our ENT doctor had loads of experience with Cochlear, as did our audiologist. And a few other people we talked to raved about their customer service which we felt was important as we’d be dealing with them a lot over the years. All in all, they seemed to have a wonderful reputation.
At 9-months-old1, Willow was bilaterally implanted. She healed up beautifully and three weeks later we had her activation. The audiologist had prepared us for what kind of reaction we may or may not get so we didn’t go into it with expectations. However, to our surprise we got a few reactions! It was a moment we had been waiting months for and I immediately felt my worries melt away.
We knew they worked, now it was our turn to do the work to give our baby girl the best shot at success. Around that time we received confirmation from the geneticist that Willow did have Waardenburg Syndrome, as did I, which finally gave me an answer as to why I had a profound unilateral hearing loss. Not only that, but we were only the third family on record to have this gene mutation with the last diagnosis in 1994, so there was a lot unknown.
Sound processor features & benefits
We were allowed to pick two processors for each ear, so we went with the Cochlear™ Nucleus® 7 Sound Processor and the Cochlear Nucleus Kanso® 2 Sound Processor. The Nucleus 7 Sound Processors were going to be for everyday wear. We felt that they would be easier to keep on as there were lots of retention methods and as she got older, she could wear it over her ear. We thought the Kanso 2 Sound Processors were a nice option if at some point she would rather have her devices off her ears. We were also told that they would be great for bath time.
Both sound processors have great features such as Bluetooth® compatibility, so you can stream2 music, tv, and phone calls straight to the sound processors. They are water resistant, so you don’t have to worry about getting caught in the rain. They also have the Aqua+ Kit34 if you choose to go swimming with them.
They have multiple color options so you can choose to have them blend in with your hair, or not. The battery options for the Nucleus 7 Sound Processors aren’t too large and they do have a compact battery which is more discrete, while the Kanso 2 Sound Processors have a built-in battery that you don’t have to change. What I really like is being able to monitor and control everything from the Nucleus Smart App5.
Willow’s Progress with her Cochlear Implants
We began a new virtual speech therapy with another amazing teacher of the deaf/listening and spoken language specialist every Sunday morning. This woman knew her stuff and helped us immensely, especially in combination with the therapy we were already receiving. Pretty soon we were like sports commentators, narrating everything we were doing and we were always singing, making up jingles and reading.
Just two months after activation Willow was filling in the ‘quacks’ to the song ‘Five Little Ducks.’ I constantly worked on building her receptive language knowing that at some point there would be a language explosion. And then it happened, six months post activation she could say 20 different words and by 10 months she was up to 100 words!
Just after we celebrated Willow’s first ‘hearing birthday’ or the anniversary of when her cochlear implants were turned on, she told us ‘I love you.’ Those words we longed to hear, and in the beginning of our journey, they were the three words we thought we might not ever hear. Our hard work was paying off. The 3-4 days a week of therapy was beyond worth it and Willow was loving her ears. She was blossoming.
At 17 months old she was singing songs such as ‘Five Little Monkeys Jumping on the Bed,’ ‘Baby Shark,’ ‘The Muffin Man,’ and ‘Finger Family’ songs word for word. By then she had fully caught up and/or surpassed her typical hearing peers in all areas. To say we were proud was an understatement. Willow continued to flourish to the point that we mostly began to focus on her articulation.
We’ll be celebrating her 4th hearing birthday very soon. We’ve made a tradition of celebrating her hearing birthday every year by taking a picture of her by a tree we planted around the time she was implanted; we have a special dessert and I write a letter to her about all of her accomplishments she had that year.
The positive impact of Willow’s cochlear implants
Willow attended a deaf and hard of hearing preschool at a mainstream elementary school for one year until she recently began mainstream transitional kindergarten. She utilizes a Roger™ FM System while in class and currently wears Snugfits to keep her Nucleus 7 Sound Processor’s in place. One of her teachers recently told me that she is a poster child for early intervention as she sets such a wonderful example of what can happen when you are proactive on addressing a child’s hearing loss and getting them the right therapies. She loves to sing and has quite the repertoire of songs. Willow rides four wheelers and dirt bikes, she has the wildest imagination and is incredibly creative; she’s very interested in learning how to read and spell and she’s making friends.
Willow is witty and outspoken but is also empathetic and very nurturing. She’s a problem solver and as we like to say ‘where there’s a will(ow), there’s a way.’ Nothing holds her back!
Having cochlear implants allows her to do everything typical hearing children can do. Plus, she gets the best of both worlds because she can turn the sound off! When she rides her four-wheeler, dirt bike or bicycle we are able to still talk to her through her Mini Microphone that we can wear on our shirt. So even with all the noise, she can still hear us. We love the mini mic and use it frequently in noisy settings such as restaurants, gymnastics, at the park, or events where there may be a lot of people in attendance.
She currently does weekly swim lessons and wears her Kanso 2 Sound Processors with the Aqua+ Kits. She enjoys doing gymnastics and even with the noisy environment of a big, open gym, she is still able to follow along with her hearing peers.
She’s able to sing to her 21-month-old sister Sequoia, who loves to dance in return. In addition, Willow is teaching her sister lots of new words. She sets a beautiful example for language. Willow states that she would like to be an ENT doctor and an audiologist when she grows up so she can give others ‘super ears.’
She is thriving and we have her cochlear implants to thank for that. As I mentioned in the beginning, my mom once stated that we’d all become better people because of Willow. And we have.
Willow has blessed us in so many ways. We’ve grown to be more compassionate, patient, understanding and present and to try to be the best version of ourselves everyday. We feel incredibly lucky and blessed to have had such a great experience so far in Willow’s hearing journey and to be surrounded with so much love and support. I know that it may not always be smooth sailing, but I know our girl is tough and resilient and she can overcome anything.
Before Willow was born, I often prayed that she would grow to be someone that changes the world for the better and has a positive impact on many people’s lives. With this incredible gift she’s received, I can already see that she’s doing just that. So, if you asked me if it was all worth it, I’d say a million times over again.”
If your child has been diagnosed with Waardenburg syndrome and is experiencing hearing loss, a cochlear implant may help with early intervention. Learn about our solutions today!
6 7- In the United States, the Cochlear Nucleus 24 cochlear implant system is intended for use in children 9 months to 24 months of age who have bilateral profound sensorineural hearing loss and demonstrate limited benefit from appropriate bilateral hearing aids. Children 2 years of age or older may demonstrate severe to profound hearing loss bilaterally. In Canada, the Cochlear Nucleus Implant System (CI500 and CI600 Series) is intended for use in children 9 months to 24 months of age who have bilateral profound sensorineural hearing loss and demonstrate limited benefit from appropriate bilateral hearing aids. Children two years of age or older may demonstrate severe to profound hearing loss bilaterally.
- For a full list of smartphone and app compatible devices, visit: www.cochlear.com/compatibility.
- The Nucleus 7 Sound Processor with Aqua+ is water resistant to level IP68 of the International Standard IEC60529. This water protection means that the sound processor with the Aqua+ can be continuously submerged under water to a depth of 3 meters (9 ft and 9 in) for up to 2 hours. This water protection only applies when you use a Cochlear Standard Rechargeable Battery Module or Cochlear Compact Rechargeable Battery Module. The Nucleus 7 Sound Processor is water- resistant without the Nucleus Aqua+ accessory to level of IP57 of the International Standard IEC60529 when used with rechargeable batteries.
- The Kanso 2 Sound Processor is dust and water resistant to level of IP68 of the International Standard IEC60529. The Kanso 2 Sound Processor with Aqua+ is dust and water resistant to level of IP68 of the International Standard IEC60529. This water protection rating means that the sound processor with the Aqua+ can be continuously submerged under water to a depth of up to 3 meters (9 feet and 9 inches) for up to 2 hours. The Aqua+ accessory should be used when participating in prolonged water activities.
- Nucleus Smart App is available on App Store and Google Play. For compatibility information, visit www.Cochlear.com/compatibility.
- The Bluetooth® word mark and logos are registered trademarks owned by Bluetooth SIG, Inc. and any use of such marks by Cochlear is under license.
- Phonak – A Sonova brand
