When Marley was diagnosed with enlarged vestibular aqueduct syndrome in her right ear, her hearing loss proved to be progressive. After she developed hearing loss in her left ear, her hearing aids alone were no longer providing enough benefit. Now that she has bilateral cochlear implants, Marley is mainstreamed, loves to swim and dance and has a bright future ahead of her:


“We first found out Marley had hearing loss in her right ear at birth and it was confirmed a month later with an auditory brainstem response (ABR) test with our audiologist. Marley saw an ear, nose and throat doctor (ENT) shortly after and had an MRI done. With the MRI, we learned that Marley has enlarged vestibular aqueduct (EVA) syndrome present in her right ear only. It was with this MRI that we knew a cause for her hearing loss.

Marley, who has enlarged vestibular aqueduct syndrome, with her sound processorMarley’s hearing loss would prove to be very progressive. She was fitted for a hearing aid on the right side at 4 months old, and only a few short months later, would develop hearing loss in her left ear. Just about the time she was receiving her left hearing aid, Marley’s right side progressed quickly, and her hearing aid was no longer providing proper amplification. This is when we started to discuss cochlear implants with her audiologist.

Choosing the Nucleus 7 Sound Processor

Marley, who has enlarged vestibular aqueduct syndrome, with her momWe knew quickly that we would choose Cochlear as her brand. I love the (then) new behind-the-ear Cochlear™ Nucleus® 7 Sound Processor because it seemed to have everything: a sleek design, lightweight feel, rechargeable batteries, Bluetooth®1 capabilities and an Aqua+ kit2 accessory option.

Marley was less than a year old at the time and I needed to do my research. I was also able to connect with a local mom who was going through the implant process with her son and happened to be a cochlear recipient as well. She spoke so highly of the Cochlear brand — the technology, customer service, product life and so much more. Having a review from someone we knew and trusted just made our decision that much easier. So, Marley received her first implant in November 2018 and her second would come about nine months later.

Marley, who has enlarged vestibular aqueduct syndrome, with her dollLike all things, we had a few bumps in the road. Marley fell pretty hard shortly after her first cochlear implant surgery. The internal device shifted and over time irritated her insides. She developed an infection and eventually had to have that right implant removed. It was a very hard time for us, but the infection cleared up, and three months later she had the right side reimplanted. We went through some very rough months leading up to these surgeries, but honestly seeing her progress and knowing where she is today — it was all worth it. I still have no doubt that her care team was AMAZING and that choosing Cochlear was the right decision for us.

Growth and progress by going bilateral

Marley with her sound processor, who has enlarged vestibular aqueduct syndromeWhen Marley went bilateral, we noticed tremendous growth and progress. She did weekly speech therapy from 1.5 years old until 3.5 years old when she graduated! Marley is now 4 years old and has average to above-average speech and language for a child her age. She attends a mainstream preschool and is known as the leader of the classroom; she has had no problem keeping up with the students in her class. Marley will continue mainstream schooling, just like her brothers. She has an amazing teacher of the deaf and hard of hearing, and they are working with her on self-advocacy skills. We are already seeing her progress shine through and are so proud!

Since Marley was implanted as a toddler, she has grown to have a strong love for her cochlear implants. She even falls asleep with them on. We’ve always relied heavily on the Hugfit™ retention loop and she still uses it today. Some recent goals she has accomplished are alerting an adult when/if her batteries die, and she has also started to master putting her sound processors on her own ear with the Hugfit loop. She has been able to put her  own magnet back on for some time now, but these little milestones make a big difference! The full-day wear time and independence with her sound processors wasn’t always the case and certainly wasn’t always easy but seeing how far she has come has been so worth it!

Marley’s favorite activities

Marley, in her swimming gear, who has enlarged vestibular aqueduct syndromeMarley loves to dance, play with her baby dolls, is learning to sign, sing  and play with her brothers and neighbors. Marley also enjoys swimming with her brothers in a nearby lake, so naturally, our most used accessory is the Aqua+ kit. For me, having multiple kits is a MUST. Who knows exactly what the future will hold for Marley—she is so young still (secretly I hope she decides to be an audiologist or a speech-language pathologist!). But no matter what her plan is, I know she will rock it!”

Was your child diagnosed with enlarged vestibular aqueduct syndrome? To learn more about helping your child receive the gift of sound, please click here for information.

  1. 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.
  2. The Nucleus 7 Sound Processor with Aqua+ is water resistant to level IP68 of the International Standard IEC60529. This water protection rating means that the sound processor with the Aqua+ can be continuously submerged under water to a depth of 3 m (9 ft and 9 in) for up to 2 hours. This water protection only applies when you use a Cochlear Standard Rechargeable Battery Module or Cochlear Compact Rechargeable Battery Module.
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.