Ash explains that Emilia was born with Enlarged Vestibular Aqueducts (EVA) in her left ear. While her right ear is currently 100% hearing she could lose hearing in this ear later on in life. Ultimately this means she is profoundly deaf in one ear. 

This condition was picked up through the universal hearing screening for newborns, Emilia was then referred to an audiologist who conducted some tests using electrodes. It was from these tests they were able to identify that her left ear was not reacting to sounds and that she was profoundly deaf in this ear. This meant that a normal hearing aid would not help with her condition. 

She was referred to a paediatrician who booked her in for a MRI, and this was when EVA was diagnosed. 

At this point Ash and his wife Lou had a diagnosis of baby Emilia, they could start to look at what the next steps were. 

At just seven months old, Emilia had surgery to have a cochlear implant  - pretty traumatic for both Emilia and her parents. Going forward the implant will not need to be replaced and it’s just the hardware on the outside that will need to be changed or updated. 

This surgery was also pretty significant as Emilia is only one of a few children in Aotearoa that was this young to have the operation. Although cochlear implants have been around for a few decades now, they are usually performed on children and adults not babies. 

The reason why such young children have not, until recently, undergone this surgery was that there was a lot of concern from a neurological perspective on how the brain would interpret receiving sound organically from one ear and artificially through the ear with the implant. It’s only recently been concluded that the brain will adapt accordingly.

As Emilia’s right ear is fine, the cost of the implant surgery was not covered by ACC, fortunately, Emilia was on the family health insurance policy so the cost of her surgery was covered (although the equipment that goes with it was not covered). 

When I ask Ash if Emilia’s balance has been affected, he says it’s hard to tell. They feel the surgery might have knocked her confidence in walking, but she’s getting more and more confident every day, so long term they don’t think this will be an issue. 

With regards to speaking, Emilia is normal (if not a little advanced) for her age. Alongside developing her vocabulary, the whole whānau is learning sign language. There is such a thing as ‘baby sign language’ as babies don’t have as much dexterity in their hands, baby sign is easier for them to use. Interesting fact, here are different sign languages across the world (300 according to Google!).  

At the moment, Ash, Lou and Emilia are learning sign language through a fully funded course with First Sign. Within the wider whānau, Ash's mum had also learned sign a year before as there were a few people in her church who were deaf and she wanted to communicate with them. Lou’s sister is a teacher and knows some sign language and Ash’s cousins and aunt are also fluent in sign. 

Emilia attends a home based centre (rather than day care centre), this was a more flexible option for Ash and Lou, but it also meant they only needed to teach one person, rather than multiple people, about Emilia’s implant. 

Teaching Emilia to sign from such a young age has been beneficial for all the family, as she was able to sign before she could say words. This meant she was able to communicate early on what she wanted before she could talk. 

There shouldn’t be a problem with Emilia going to a mainstream school. Her current development is tracking along like any normal child her age, so she’s expected to join her peers at school when she turns 5. 

Emilia, Ash and Lou have very much immersed themselves in the deaf and hard of hearing community. Emilia goes to a deaf playgroup, there’s a deaf kids group and last month they went to an event in Bethlehem called Deaf, Deaf World for students and users of NZSL. 

A big thank you to Ash and Lou for sharing the whānau’s journey and learnings with Emilia and what it means to raise a child who is profoundly deaf in one ear. 

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