Your deaf child with additional needs may be offered hearing aids. Hearing aids are only useful if:
For deaf children with additional needs, it may be more difficult to assess their hearing, to fit hearing aids and to measure their benefit. Your child may go through a phase of removing their hearing aids. It can be very frustrating to keep replacing them, especially if your child does not want them put back in. They may be removing their hearing aids because it is fun and they can do it, to attract attention, or because they are too quiet or too loud.
This is where it is important for you to identify when your child is happy to wear aids, when they are not, what sounds they prefer, and any sounds your child makes (not just speech), and where sounds seem too loud or not loud enough. This all helps your child’s audiologists to make sure hearing aids are programmed to best meet your child’s needs. If your child’s hearing aids constantly fall out or they use a wheelchair with head supports, it is important for their audiologist to know as the hearing aids can be adjusted to specific situations.
Children with a severe to profound hearing loss and additional disabilities are now being considered for cochlear implantation. The type and severity of the additional disability plays an important role in how successful an implant might be for the child. Although they may not be considered good candidates, deaf children with additional disabilities can become successful cochlear implant users.
An implant may give your child with disabilities in addition to their deafness access to the best listening possibilities for development and learning opportunities. Improved quality of life and family relationships may be important benefits for your child and family. A cochlear implant may help your child to hear your voices and develop positive emotional relationships with family and friends. Cochlear implantation may provide environmental sounds and help your child react better to potential dangerous situations.
Cochlear implantation in a deaf child with additional disabilities needs very careful consideration from both parents and professionals. A thorough multidisciplinary pre-implant assessment is important and the child’s suitability for implantation decided on a case-by-case basis.
Matthew’s story
Matthew was 6 months old when he was diagnosed as being profoundly deaf. Our paediatrician referred him almost straight away to the cochlear implant program where we were put on the waiting list.
In the meantime we were to find out that Matthew also suffered from a brain condition called pacchygyria that affects both his physical and mental development. As a result of various complications, Matthew was three by the time all the necessary checks had been completed to show that physically he was suitable for an implant.
At that time, the implant team had very minimal experience implanting children with learning difficulties and couldn’t say that Matthew would gain any more than environmental awareness from an implant. By this time we had been signing with Matthew at home for over 2 years, but he had barely picked up more than a dozen signs and our communication was very limited. We were having to work very hard with him to develop his sign language, and felt that if he was implanted, we would have to change our focus to develop his hearing. We were concerned that if we couldn’t develop communication through his implant, and if we didn’t continue to focus heavily on signing, then we might end up with no real communication with Matthew.
It was a difficult decision, but we felt in the end that it would be better not to go ahead with an implant. This meant that we could continue to focus on signing with Matthew without introducing what we felt would have been yet another complicating factor for Matthew. It did take quite some considerable time for Matthew to learn enough signing to communicate at his own level, but he has continued to progress well and can now hold quite a conversation and is never lost for ‘words’. We have no regrets about the decision we made, which was right for our son at that time.
Bethany’s story
Bethany was born 15 weeks early weighing only 1lb 10. Bethany was in hospital for 17 very very long weeks. The roller coaster ride we have had with her since then has taken many twists and turns.
At 6 months she had to have major surgery, at 9 months we found out she was profoundly deaf and at 18 months we found out she had athetoid cerebral palsy.
As you can imagine, when the teacher for the deaf came along and mentioned cochlear implant, my first reaction was ‘no way’. We have done hospitals, we have done surgery, for 2 long years we were everything to Bethany but parents.
We just wanted to look after our little girl without any more interference.
Maybe it sounds selfish, looking back it probably was. We also feel it wasn’t the time to make any more major decisions. We got over that though. We decided that if there was the slightest opportunity to better Bethany’s life in any way, we had to try it.
The negativity we received from a few professionals made us all the more determined. I don’t know why we worried. The implant team were fantastic from day one.
For many people the year it took for us to have Bethany assessed would have seemed too long. It gave us the chance to discuss any issues we had. To see for ourselves in the assessments if an implant would benefit. If you have a child that needs a wheelchair, get it sorted as soon as possible. It’s important because they need to be stable so that a response can be seen.
We could never see a response. For months we thought we were on a losing road. Bethany was never going to respond enough to warrant the implant. The team were great. They can see things that you don’t and they were so patient with Bethany and never gave up.
Then we took Bethany to a session where we were convinced she would be a little madam. She had been in a foul mood all morning. The little devil performed perfectly, you could actually see her responding. Everyone was delighted.
We went to a team meeting and it was decided implantation would go ahead. No, life never runs smoothly in our household. Bethany got a temperature an hour before she should have been in hospital. Operation cancelled.
On Thursday June 12th 2003 Bethany had her operation. Everything went well and after the xray the next morning we went home. Bethany’s implant was turned on on the 7thJuly. You could see the wonder in her eyes. It was amazing. We have gone through spates of not wearing it which was put down to her threshold going down but she loves it now.
It was by far the right decision for us to have made for her. She is more tolerant, she watches a wider range of television and plays with a wider range of toys. She even tries to put the magnet back on herself, a joy to watch considering she doesn’t have full control of her arms.
She can hear me sneeze and shout and she can hear us call her name. Just recently she has started to become very vocal, very loudly. All the assessments, the tests, the MRI. the hurt of watching her just pull it off because she doesn’t want it on. It was all worth it when I see my child turn when she hears a noise. We made the right decision, no one will ever convince me otherwise.
William’s story
What follows may prove uncomfortable reading. I have not written it seeking sympathy, neither have I written it to worry parents considering an implant, nor to criticise any of the medical and professional teams that have been so supportive over the last five years. I have no “deafness” agenda. All I ask is that with children like William, where deafness is just one of a complex web of issues, we do not lose sight of what best serves the child as a whole.
When a child is born every family sets out on a journey. When that child is disabled the road they travel is far from smooth. We started our journey with our first son, William, in March 1999. Within a few days it was clear that things were not exactly as they should be. We were fortunate to have an understanding and trusting team of GPs and health visitors who never once questioned our concerns or observations and over the following months helped us through the rounds of tests and referrals.
William was diagnosed as profoundly deaf, but there was more. He was slow to achieve the landmarks that most babies do, slow to lift his head, slow to sit and walk. Without boring you with the history of scans, tests and consultations, by the age of 18 months William was diagnosed with CHARGE syndrome.
In many ways it was good to have a name to put to William’s problems and we at last knew where the boundaries lay. Whilst the diagnosis identified a number of problems, we became absorbed by his hearing loss. We started to sign with him from early on and began investigating the possibility of an implant. After the usual assessment process and some pauses for other minor operations, the team at the hospital undertook the surgery in April 2002. We were no strangers to hospitals and surgery – in his three short years William had been anaesthetised at least seven times.
After switch-on William made slow progress, everyone taking hope from his happiness to wear his headset all day from the very beginning. Six months on and after a change in mapping strategy he made a step forward and started to turn to claps – a revelation to us. He no longer needed to be within waving, touching or stamping distance in order to get his attention.
In April 2003, just as we started to see a brighter future for William, it all stopped. What started as a slightly prominent implant site became a patch of red skin, and a cause for concern to the surgeon. This in turn became emergency surgery to reposition the package and ended in the discovery of a large infection necessitating the removal of the implant. The hospital had no hesitation in offering re-implantation on the other side.
Sitting with William in the recovery room at the hospital the questions started spinning around my head. As the next few days and weeks passed more questions kept coming. Could we put William through this again? What if a second implant failed or the infection recurred? What if he made better use of a second implant and then it failed and we had to take away this gift of sound? If we did re-implant what kind of life would we let him lead, worried that the slightest knock could cause damage? These are all the kinds of questions asked the first time around, but this time we knew that he had made slow and limited progress with the first implant and the question “How much benefit would William really get from an implant?” became central.
Thank goodness for my husband, a kind and loving man who nonetheless has the ability and strength not to let raw emotion cloud his judgment. He said, “Don’t try and make a decision, identify the questions we need answering, answer them and then the decision will have made itself.” He was right.
With the help of the hospital team we spent time looking more closely at CHARGE syndrome and the success of implants for children like William. Very little data exists, however, what soon became clear was that the children we had heard of remained reliant on signing; implants gave them environmental cues, which are of course helpful but they remained signers. Whilst the hospital put the infection down to nothing more than bad luck, we never stopped wondering whether there was something about William that made him more susceptible to it and we were not about to try again with an implant just to disprove that hunch.
The more we thought about it the more we realised that William was not just a deaf child. His CHARGE syndrome, the way his brain and body worked was at the heart of this issue. We needed to look at William as a whole, not just his deafness. We had been so focused on helping to give him some hearing that we had tended to ignore other issues like his general but significant developmental delays. We had come to understand that these had a greater impact on his ability to adapt to and process what the implant was giving him than we had previously realised.
By this time we were anticipating William’s move from preschool to school and were determined that it should be as smooth as possible for him. Without an implant we had a stable, immovable baseline for everyone involved in his care to start with.
Whilst we will never regret our initial decision to have an implant, with hindsight we realise it had been a huge distraction for everyone around him. Although we had continued signing with him, we had been pinning too much hope on the chance of him becoming oral. Without even realising it, we had stopped pushing ourselves to expand our own and his signing vocabulary – we were treading water. Family and friends at times seemed to forget he was deaf; it was as if by seeing him with the implant they thought it was just a matter of time before he would be talking like any hearing child.
The discovery of the infection really made us stop and reassess William’s situation in a way I think we might never otherwise have done and by doing so we reached a greater understanding of our child and his situation. In the past we used to say William was a deaf child who just happened to have CHARGE syndrome. Now we say William has CHARGE syndrome, which includes being profoundly deaf.
I will never forget my journey home from London after telling the hospital team that we would not be opting for another implant. I sat waiting for a feeling of sadness and panic to overwhelm me; instead I was enveloped by a wave of relief and determination to do all I could for William as he is now.
It may appear to some that we gave up too soon on the implant. We should have tried again for William’s sake. In truth, we were given the opportunity to stop and look closely at William as a four and a half year old. We were able to look back and realise how much more we understood about him, his abilities and our expectations than we had two years before.
More than a year on from the removal surgery and William has settled well into a mainstream school with a unit. He has signed support all the time and is making slow but steady progress. At home we are all happily signing, including his two year old brother, and continue to feel that we have made the right decision for William. The path may have changed direction, but our journey with William continues.
Note: The above Parent Stories are extracts from the National Deaf Children’s Society’s booklet “Cochlear Implants: A guide for families” (2005). The views expressed by parents are their own and not necessarily endorsed by the National Deaf Children’s Society.
References:
Information provided by the National Deaf Children’s Society. Reproduced with permission.
Disclaimer: The information contained on this website is not intended as a substitute for independent professional advice.
