Christy S.’ son, Dexter, was born with hearing loss from branchiootorenal syndrome. After trying hearing aids, Dexter’s speech was not developing, so Christy turned to cochlear implants. Now with the Cochlear™ Nucleus® Kanso® Sound Processors on both ears, Dexter lights up when he has access to sound:
“On Sept. 19, 2014, our son, Dexter, was born with a profound hearing loss. Thanks to the observations of our health nurse, by Dexter’s two-week pediatrician appointment we had a referral to an ear, nose and throat (ENT) doctor and audiologist for hearing tests.
With CT and MRI scans, we were informed that his middle ear bones were displaced and located in his cheek. We saw many doctors who informed us that he had all of the symptoms of branchiootorenal syndrome. This translates to him having pits and malformations of the neck and ears, one kidney that is smaller than the other (though both kidneys are fully functional) and profound hearing loss.
When we were given the diagnosis, we were devastated. We could not believe it, as he had seemed to be responsive to sound for the most part. However, one night my husband and I stood on opposite sides of our counter and I screamed as loud as I could; he did not even flinch.
By January 2015, Dexter was fitted with the strongest model of hearing aids to attempt to give him access to sound. If the seal on the hearing aids was not perfect, the feedback (high-pitched squealing) was terrible. The hearing aids certainly helped to a degree, as he never stopped making sounds. However, he did not receive enough sound to help develop his speech. After several months, a plan was put into place to get him cochlear implants.
Dexter went through four surgeries. The first was in November 2015 to remove the pits on his ears and neck. It was at this time, we found out those ear pits were actually tiny ear canals. He had his left cochlear implant surgery in April 2016 and was activated in May 2016.
His reaction at the t
ime of activation was amazing! He sat up and immediately started to make sounds. It is hard to describe, but there is a distinct sparkle in his eyes when he has access to sound and a blank, lost look when he does not.
We chose the Cochlear brand of cochlear implants because it provided us multiple electrode lengths for implantation, which we would potentially need with the complicated anatomy of his right ear. At the time of activation, we received the Nucleus® 6 Sound Processor. Since he was 1 and a half years old at the time, his ear was still very pliable and as the processor was larger, we used a bonnet on him at all times to make sure his processor stayed in place and we did not lose it.
Bonding to his ‘ears’
His right side cochlear implant was not as straightforward as the left side, and it required a two-part surgery. The first surgery took place in August 2016 and the other in November 2016. In December 2016, his right side cochlear implant was activated, roughly 8 months after his left. At that time, we switched both ‘ears’ to the Kanso Sound Processor and kept the Nucleus 6 as a backup.
On the morning of our one-week follow up, one of his processors fell off for whatever reason, and he picked it up and put it back on his head. My husband and I looked at each other in awe. He has bonded to his ‘ears’ in a way we never could have imagined. With the hair clips available for the Kansos, if the processors fall off, he can just pick them back up and connect them on his head by himself.
During his last booth test and individual testing on each side, Dexter was even responding to quiet sounds; I actually questioned if a sound had been played because I could not hear it. For the first time in three years, I am OK with saying he is hearing at a ‘normal’ hearing level. With no processors, a sound would need to be at 120 dB, the equivalent of an airplane engine, for Dexter to hear.
Kanso tips and tricks
In order to do our part for the environment, we like to recycle batteries at the library!
The Aqua+ for the Kanso* takes a bit of practice, but once you get comfortable with it, it is truly no problem! Unfortunately, I did break one set as I pushed the Aqua+ in the wrong place. If you look at the hard mould, you can see a subtle outline where the back of the processor lines up. Just make sure you take the clips off as well, before putting the processors in the Aqua+.
I also found that with Dexter having a level one magnet, it was not holding onto his head and we would have to put the bonnet on. I gave that feedback to our audiologist and she ordered us a level two magnet for when he uses the Aqua+. The level two magnets, headband and water clips worked well at the splash pad over the summer. Seeing your child hear water trickling for the first time is a very magical thing! He was running all over the place and they stayed on well. I even used the Cochlear™ True Wireless™ Mini Microphone 2 so he could hear me while he was playing rather than just turning up the sound; that worked well.
It has now been a year since Dexter had both ears activated. He is trying to say new things daily through repetition, and we could not be more grateful and blessed to live in a time where such a technology exists to give him this opportunity to experience all the sounds around him. Thank your Cochlear for this technology and all the support you provide our family!”
If your child has hearing loss from branchiootorenal syndrome, visit IWantYouToHear.com to find out if cochlear implants could help.
* For Cochlear’s latest Nucleus Sound Processors: The Kanso Sound Processor with the Kanso Aqua+ is water resistant to level IP68 of the International Standard IEC60529. This Kanso water protection only applies when used with LR44 alkaline or nickel metal hydride disposable batteries.
