How BONEBRIDGE Helps My Son With CHARGE Syndrome: Experiences From a Mom

10-year-old Maximilian was born with CHARGE syndrome, a rare genetic disorder, and has multiple sensory impairments. For more than a year bilateral bone conduction implants have helped him hear. In today’s guest article, Maxi’s mother Susanne talks about their family life and how BONEBRIDGE supports Maxi at home and at school.

I live in Northern Germany with my husband Christian and our sons Mattes and Maximilian. We are a very active family and often go on bike tours with our tandem bike. Apart from that, we love spending time in our garden or playing cards.

Diagnosis: CHARGE Syndrome and Life With Hearing Aids

In 2010, our second son Maximilian—Maxi—was born. Doctors noticed multiple sensory impairments soon after his birth. Shortly afterwards, he was diagnosed with CHARGE syndrome, a rare genetic disorder. It usually includes some form of hearing loss, but the degree can vary. Tests have shown that Maxi has a moderate hearing loss. At the age of six weeks he got his first hearing aids with earmolds. He used to wear them regularly. But due to a malformation of his outer ear, the earmolds didn’t fit properly.

Maxi used his hearing aids for more than nine years and overall he got along well. But he needed help putting the hearing aids on and and couldn’t tell which direction sound was coming from. He also frequently had water in his ears canals, which meant he couldn’t wear hearing aids at all.

New Opportunities With Bone Conduction Implants

During a hospital stay in 2019 he had another hearing test—this time a bone conduction test. The result was surprisingly good and it turned out that Maxi’s inner ear works fine. Instead, his middle ear was causing issues. We were told it would be possible to fix my son’s middle ear with surgery. However, we decided against surgery because there was a small risk that his hearing could get worse after the procedure. We didn’t want to risk anything since our son is also affected by vision loss: he only has one eye with limited vision and a glass eye. The concern that his hearing would also deteriorate was just too big.

Another option Maxi’s doctors suggested was the BONEBRIDGE bone conduction implant. This would definitely improve his hearing. After reading more, we arranged surgery dates in February and March 2020. Due to the COVID-19 pandemic, the second surgery had to be postponed, but in June 2020 Maxi received his second bone conduction implant.

New Life With Hearing Implants

Both surgeries went smoothly and since summer 2020, Maxi has audio processors on both sides: first SAMBA and now SAMBA 2. He quickly got used to his new hearing. Only a few sounds at school were too loud. Once he asked his classroom aide to turn off “that beeping sound”. It turned out that Maxi had heard the birds sitting in the tree outside the open classroom window for the first time.

What’s really great is that Maxi can use his new hearing devices on his own. He puts SAMBA 2 on his head and knows how to change the battery and attach the clip. When the battery is empty, he notices immediately and asks for a new one. None of that was possible with his old hearing aids.

And you can tell that he hears better than with hearing aids. He notices the rustling of leaves and the sound of the neighbors’ lawn mover. Moreover, directional hearing is now possible, which is very precious and important in everyday life!

Because Maxi hears better now, his speech has also improved slightly. We are very happy about that. He doesn’t need earmolds anymore. And because his ears are not blocked anymore, he doesn’t have any liquid in his ears.

In the Classroom With SAMBA 2

In the summer of 2020, Maxi transferred to a secondary school and attends the local high school now. We put together a profile of Maxi before he started, just as we had for his nursery, kindergarten, and elementary schools. We’re very open about Maxi’s disability and we think it’s very important that other families know some basic information about Maxi: How he is, what he can or can’t do, and what’s important when interacting with him. For us this has proven to be the right way to go, because there is less fear of contact.

At school Maxi uses an FM system together with his SAMBA 2 audio processor. The teacher wears a microphone, that connects to Maxi’s audio processor via SAMBA 2 GO. Since November, we’ve also been testing microphones and loud speakers for Maxi’s classmates. Because of the COVID-19 pandemic, windows and doors are open most of the time and children have to sit facing the teacher at the front. With the assistive listening technology, Maxi can now also hear and understand his classmates.

Switching to BONEBRIDGE was the best thing that could have happened to us!

Thank you, Susanne!

Marie from Germany was born without hearing because of CMV. Thanks to bilateral cochlear implants, she can enjoy a world full of sounds today. Mother Michaela shares Marie’s hearing journey in her guest article.