Community Spotlight: Three of four siblings share hearing loss gene

We love it when people share their hearing loss stories with us on social media. Our community often provides comfort, encouragement, inspiration and support for others in similar situations. Recently we connected with one of our Instagram friends who we think has a wonderful story to share.

I had the opportunity to chat with Yana, the mom of four beautiful children – three of whom have hearing loss. This is their story: 

Jill: Does hearing loss run in your family apart from your children?

Yana: Before our children our family had no history of hearing loss. Our children are still the only ones with a hearing loss.

Jill: How did you find out your first born had hearing loss? How did you cope or what steps did you first take?

Yana: Emily was given a ‘refer’ result from her newborn hearing screening in hospital. She was the referred to our local children’s hospital for further testing. An ABR diagnosed her, and her diagnosis was a huge shock to us and certainly not what we were expecting. After her diagnosis I did a lot of reading and research to understand what we were dealing with.

Jill: How did you find out that each of your other children also had hearing loss? 

Yana: Jayden and Maddison also received ‘refer’ results from their newborn screening and went on to be diagnosed by ABR at the children’s hospital. When Jayden was diagnosed we had to deal with the fact that we knew it was genetic and we faced the possibility of all of our future children being born with a hearing loss. But other than that, it was easier in terms of understanding what we were dealing with and knowing what happens next.

Jill: Did you go through a process to figure out that a hearing loss gene deletion was the cause? 

Yana: When Emily was diagnosed we were offered to test for Connexon 26 and Connexin 30. These were the most common genes to cause hearing loss and the only hearing loss gene test available in Australia. That test came back negative. We decided to expand the test to our family, which eventually proved the hearing loss was genetic. After Maddison’s diagnosis I found out about a test called ‘Otoscope’ genetic testing, where we had Emily’s DNA sent to a lab in the US. The results took 6 months to come back and revealed that Emily has a gene deletion of the OTOA gene. We are still awaiting results for the test of the family.

Jill: How have each of your children coped to wearing their hearing technology?

Yana: Overall they are good hearing aid wearers. They are at an age that they are aware that their hearing aids help them and they ask me to put them in each morning, and they know where their hearing aids belong when they aren’t in their ears. The same with their FM sytems. They bigger kids put them on charge each night and make sure they are ready for school. The older two are also very competent in asking their teachers to use the FM while at school.

Jill: Do you have any tips for parents of children who also wear hearing aids?

Yana: There is that frustrating period where getting kids to keep their hearing aids in is difficult, but it doesn’t last forever. We read books and watch TV shows with characters who wear hearing aids. We try to talk to other hearing device wearers when we are out in public. We welcome questions from strangers about their hearing aids. I do all of this to teach them to be proud to wear their hearing aids and not be ashamed of them.

Jill: Do you have any special memories of them experiencing special sounds or communicating with help from their hearing technology?

Yana: Apart from seeing their faces when they were first fitted, my next favourite experience has been the moments of realization that their hearing aids are there to help them hear – whether it is asking for their hearing aid to be put in, telling me they can’t hear without their hearing aids or explaining to someone why they wear hearing aids.

Thanks to Yana for sharing her story!

• Author Details

Author Details

Jill von Büren

Editor-in-chief of HearingLikeMe.com