Hand to Hold's Official Blog: Written by Parents for Parents

Living with Auditory Neuropathy Spectrum Disorder

Waiting for a CI mapping

Waiting for a CI mapping

When my husband, Scott, and I made the decision to have children, we knew there was a strong possibility our children would have hearing loss once reaching adulthood. A hereditary hearing loss is prevalent in Scott’s paternal family. Scott, his twin sister, father, and grandmother are all hearing impaired.

When our twin boys, Joseph and Campbell, were born at 24 weeks gestation, we quickly learned that preemies can have hearing loss. Campbell passed away after 23 days of life and never had a hearing screening. Joseph failed both of his newborn hearing screenings. We were told not to be too concerned as many preemies often fail their hearing screenings at first but pass once they are older. We took this to be true and crossed this off the list of things to be worried about.

Although Joseph had not passed his newborn hearing screenings, we never had any concerns about his hearing as he always seemed to respond to our voices. When Joseph was five and a half months old, just days before discharge, he developed a late onset case of Necrotizing Enterocolitis (NEC). Joseph was subsequently left with 41 centimeters of small intestine. In order to treat the infection, Joseph was given strong doses of Gentamicin – an ototoxic drug. We were told that if Joseph didn’t have this drug, he could die but that if he did receive this drug, there was a good chance he would lose his hearing. There was no question that he should receive the drug in order to live.

Several weeks later, we had been given permission to take Joseph to the garden at the hospital without being accompanied by a nurse. Freedom. While we were enjoying time as a family in the garden, a helicopter flew overhead to land on the hospital. The sound was incredibly loud. As I reached down into the stroller to cover Joseph’s ears, I saw he was sound asleep and completely unphased by the helicopter flying very low above us. We call that moment our “Mr. Holland’s Opus moment” – we knew Joseph could not hear.

A few days later, Joseph was diagnosed with Auditory Neuropathy Spectrum Disorder (ANSD). The best way to describe ANSD is as a disconnect between what the ear hears and what the brain understands. I once had someone describe it to me as listening to the radio while driving through rural countryside and the radio keeps fading in and out due to poor reception. While someone with ANSD may technically “hear”, it doesn’t mean they can understand. If I had a dollar for every time I was told my child could hear, I would be one very rich woman right now. No, my child could not hear.

The ENT met with us several days later and told us that a future with ANSD is often uncertain. Hearing aids may work or they may not, he may ultimately need a cochlear implant(s), he may be dependent on sign language – he just didn’t know. Scott and I are both chorus teachers. As musicians, knowing we were going to have a hearing impaired child was a tough pill to swallow. Would Joseph be able to enjoy music like we do? Would he ever be able to sing? The questions were never ending.

One of Joseph's first CI mappings

One of Joseph’s first CI mappings

Joseph received his first pair of hearing aids days before his first birthday. We enrolled Joseph in auditory verbal therapy. While Joseph was making progress, it became evident around the age of two and a half that his progress had plateaued with his hearing aids and that he would need a cochlear implant (CI). While CI’s sometimes work and sometimes do not work with ANSD, we felt as if we needed to see if a CI would be successful for Joseph. Joseph received his CI three months after his third birthday. January 16, 2013 was the beginning of a new journey for Joseph. It took him down a new path – a path of being able to hear.

Joseph is now five and has made astounding progress. He attends an oral school for children who have hearing loss. He is in a full day class with six children (all who have hearing loss) and two teachers. His entire school day is focused around listening and spoken language. There were days when Joseph was a toddler that I would pray for him to just say one word. Now, he does not stop talking. He says something new every day. It is inspirational to watch his progress.

All the questions we had as musicians raising a hearing impaired child are slowly being answered. Yes, Joseph loves music. We will be listening to music while driving to school and I will often hear Joseph say, “Baby (referring to his 19 month old sister, Emily), do you hear the music? Do you hear the music, baby?” What is ironic is that my hearing impaired child is asking my hearing child if she can hear the music.

It makes me smile every time.

Laura Martin About Laura Martin

Laura B. Martin (GA) is the NICU Ambassador Director at Graham’s Foundation and keeps parents connected with their NICU by serving as a liaison between the NICU and the foundation. She is a former middle and high school teacher and is now mom to Joseph, Campbell, and Emily. Joseph and Campbell, fraternal twins, were born at 24 weeks gestation. Campbell lived for 23 beautiful days. Joseph spent 228 days in the NICU and also experienced a near fatal bout with NEC that left him with Short Bowel Syndrome. He also has a g-tube, auditory neuropathy, hypotonic cerebral palsy, asthma, vision impairment, hypothyroidism and multiple food allergies. Even still, he is a happy and active child. Laura can be reached via email or her personal blog.

Comments

  1. Torran has ANSD, although he’s only using hearing aids. I find that few people understand what hearing loss truly means, and it’s even worse with ANSD, where the translation of sound is more of a problem than it’s reception. People misunderstand that a device such as a hearing aid or cochlear implant fixes everything. It helps, certainly, but it doesn’t fix the problem. One high school student I saw in a “get ready for school” presentation said he preferred to watch movies with closed captions, even though he had implants. My son can’t read the screen fast enough (yet!). Have you explored that option?

  2. Laura, my son has unilateral ANSD with profound sensorineural loss. We’re currently using a BAHA for that side but he seems to have plauteaued. Did you find it hard to get a CI eval with the ANSD diagnosis? We’re meeting some resistance about it because he has one hearing ear but he’s very behind in language development (oral and sign).

    • Laura Martin says:

      Hi Christy-
      We did not face any resistance in getting the eval. The initial information submitted to the insurance company for the surgery itself was first denied. Once the surgeon sent more documentation, it was approved. Feel free to email me anytime!

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