Katy L. had never thought of hearing loss and its impact on her life before. She and her husband were hearing and her first child was born hearing. Then her next two children, Ledger and Ezrie Kate, were diagnosed as the first in their family to have hearing loss, later finding they had the Connexin 26 gene mutation. Thanks to their parents’ research and decision to go forward with cochlear implants, see how Ledger and Ezrie Kate love the new sounds around them:

Ledger with connexin 26 gene mutation“Our oldest child was born with perfect hearing, so the chance of having a child with hearing loss never crossed our minds. Our second son, Ledger, was born in November 2014, and he was diagnosed with bilateral sensorineural moderate hearing loss at 3 months old; he received his first pair of hearing aids at 5 months old.

When Ledger was around 16 months old, he had a sedated repeat auditory brainstem response test (ABR) and genetic testing. The ABR in August 2016 showed a hearing loss progression in his left ear, and genetic testing revealed that he was positive for the Connexin 26 gene mutation. We were told that this mutation is responsible for 10 percent of genetic hearing loss in children. The ABR showed that Ledger’s loss was not at the level to implant yet, but we were told to familiarize ourselves with cochlear implants and the different companies due to the progression of his loss. This is how we found Cochlear.

Impressed by Cochlear customer service

I was immediately contacted back by a representative for Cochlear. My husband and I were completely blown away with the customer service and support that Cochlear showed us to educate and provide us resources to early intervention materials as we were understanding this new world; we were sold.

Let us take a journey to about four months later when our third child, a beautiful baby girl, was born. Ezrie Kate failed her newborn hearing screening and a few retests when she was in the Neonatal Intensive Care Unit (NICU). At that point, we were seasoned pros though, and the hearing loss did not come as such a shock because of Ledger’s Cx26 diagnosis. At 6 weeks old, Ezrie Kate had a repeat ABR. At this appointment, we were informed she was profoundly deaf and had a Connexin 26 mutation too. Talk about a shot to the heart…I was prepared for her to have hearing loss, but not that she would be profoundly deaf. We were told Ezrie Kate would be eligible for bilateral cochlear implant surgery at 1-year-old.

An easy choice with Cochlear

After our daughter’s diagnosis, my husband and I decided to travel five hours from home to meet with an ENT-otolaryngologist and audiologist that came highly recommended to us. Ezrie Kate’s diagnosis was confirmed with a sedated ABR, and at that time, Ledger had showed even more hearing loss progression in his left ear. We were ready to schedule surgery. When scheduling surgery, we did not hesitate when asked which company we wanted to choose for our children’s cochlear implants.

Ezrie Kate with connexin 26 gene mutation

Cochlear had proven great to us over and over again, and the Nucleus® 7 Sound Processor was soon to be released. We loved the design of the Nucleus 7s, and the Bluetooth®* capability (as we have a deep love for music). On September 26, 2017, Ledger was unilaterally implanted on his left side, and on December 19, 2017, Ezrie Kate was bilaterally implanted.

Favorite features and sounds

Both Ledger and Ezrie Kate are doing amazing with their ‘new ears.’ One of our favorite features of the Nucleus 7 Sound Processor is the Nucleus® Smart App. The Nucleus Smart App has come in handy because we do not have to keep up with remotes to change volumes. The app is also great to check battery life.

[Ledger] pointed to his ears and said, ‘Mama, you hear that…’tweet tweet’…Birds.”

Since activation, Ledger’s speech is progressing beautifully, and he is thriving in his preschool class with the help of speech therapy. A few months after Ledger’s activation, when playing outside, he stopped suddenly with a confused look on his face. He pointed to his ears and said, ‘Mama, you hear that…’tweet tweet’…Birds.’ My baby boy heard birds chirping for the very first time, and my heart melted.

Ezrie Kate is starting to recognize her name, and she loves dancing to music. Our family is grateful for the opportunity Cochlear, our surgeon and our audiologist have extended to our sweet babies. We hope that due to the gift of hearing, Ledger and Ezrie Kate will be able to lead normal and successful lives where the sky is the limit.”

If you are a parent of a child with hearing loss and are looking for treatment options, visit IWantYouToHear.com.

*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.
Cara Lippitt
Cara Lippitt is the Senior Manager, Social Media Strategy at Cochlear Americas. Cara is inspired by the stories of the recipients that she is able to tell and the incredible journeys they have taken. Cara was born and raised in Colorado and adores the mountains, snow and the world of musical theatre.