About Alice
Alice was born at the end of March 2007. Alice had an uneventful birth and early life. She was born at 39 weeks by elective caesarian, she had APGARs of 9 and 10 with no jaundice or any other problems. She had an OAE (otoacoustic emission) test shortly thereafter which proved normal. As Alice passed the year mark, it became apparent to us that Alice was not developing any speech and seemed to intermittently respond to sounds and prompts. After 2 or 3 months arguing with health professionals that she wasn’t just ‘ slower than her brother’ we were finally referred for hearing tests in July 2008. In the behavioural tests Alice provided inconsisitent results and was then referred for further OAE and ABR (auditory brainstem response) tests.
Alice’s OAE was, again, normal but she had an absent ABR up to 90db. She was then referred to the Audiology department at St. George’s Hospital in London. In between times, we chose to seek the advice of one of St. George’s Consultant ENT Surgeons who runs their Cochlear Implant programme, Mr. David Selvadurai, who diagnosed Alice with Auditory Neuropathy Spectrum Disorder (ANSD).
For her 2nd birthday, Alice received a cochlear implant on her right ear and shortly after her 4th birthday was implanted on the left side. Since switch on we have been working with her every day to improve her access to speech and language. Alice is a bright little girl and now communicates wonderfully with her peer group through speech . She spends sessions every fortnight at Auditory Verbal UK in Oxford and attends mainstream school and is now beyond age appropriate in her language.
I have enjoyed reading about Alice! Thank you for sharing your story.
I am an audiology student at Utah State University, USA. I am giving a presentation about auditory neuropathy to my fellow classmates. I was so impressed with your blog that I decided to add Alice’s story to my presentation. I thought you might like to know, that in another part of the world, a small group of people will be hearing about Alice’s story.
Becky
Becky,
Thank you so much for your kind note – your post has reminded us that it has been a very long time (far too long) since we last updated the blog. Greetings as well to your colleagues from USU – I hope the blog gives an insight into living with ANSD and a CI.
Chris
Thank you for taking the time to put together this website. My daughter was just diagnosed last week with AN. It happened just before she received her cochlear implant. I am trying to find out as much information about this as possible. As you have said most of the information is for scholars. It has been a bit difficult to get my head wrapped around all of this. I look forward to reading all of your site.
Sincerely,
Yvette
Thank you so much for your kind words – it seems our site has developed a momentum and reputation of its own. We really ought to update it more often, but if there is any help or info we can offer, do just drop us a line.
Regards
Chris
Hi
I love your website! I have recently set one up for my little boy Calum. Calum is profoundly deaf due to CMV (cytomegalovirus). From information I have read 30% of childhood deafness is caused by CMV but noone has heard of it. I am working with the CMV support group to raise awareness and to offer support to families. We also attend AVUK and have found them to be invaluable. I would love to speak with you.
If you could contact me at: info@calum-reece.co.uk it would be much appreciated.
Regards,
Gaye Tomlinson
Gaye, I have emailed you.
Chris
Hi Chris
I saw your email thankyou very much, I’ve been so busy I’ve not had chance to call you back. Had appointments with Calum all week – I’m sure you know how it is!
I’ve seen on the website that Alice has her operation for her second implant today. I hope it goes really well and I’ll speak with you in a week or so.
Thanks
Gaye
Wow Alice bless her!! I’ve come across your website as we too have a late developer, third child which you wish to call her we prefer Myla muffin!! Lol who is two on Friday. Myla was born at 39 weeks by elcs also with great apgars but had minimal startling however passed oae we got to 11 mths and still no babbling, Myla was then referred for a vra and failed then re-tested oae and passed that was it. In march I pushed again as mam ma hasn’t left her lips yet!! However she does dance to music anyway appointment was end of June myla went onto to fail two more vra’s at 60 dcb we had abr yesterday which passed!?!?! Myself and my husband are so confused and Myla bless still has no form of communication other than sign which I’m teaching and let’s say it’s a battle of wills on most days.
Sorry to give life story but you know how it is you just want to gather as much research as possible to help your little ones. Have you come across this with anyone you have met?
Regards
Sam Maguire
Would just like you to know as a mother of a 7 month old baby boy – Finn, I have found Alice’s Blog an invaluable reading experience. Like Alex, Finn is diagnosed as having ANSD. At birth he by chance failed to pass the OAE screening and was sent to Audiology for testing. Since he has passed every OAE and so far he has had 3 ABRS to all of which he has had no measurable response and has been tested up to 90db. We were actually at Audiology today and met Finns consultant for the first time. His name is Dr Banerjee at James Cook. The next step to us is to trial hearing aids, he gets them fitted early September so it is so encouraging to hear of Alices improvement with CI’s. If possible I would love to know if Alice had any benefit from Hearing Aids?
Regards
Natalie Nixon (Finns Mum)
Natalie,
Thank you so much for your note. Alice had to wear aids as part of the assessment process for both her implants. She wore two before her first implant and one (obviously) before her second. They gave her no benefit at all – they just amplified the fuzz she was hearing which she found quite distressing the first time round (to the point that she wouldn’t wear them at all) and it became distracting the second time round (it actually made her hearing worse). There is evidence that children with ANSD perform as well as children with other forms of hearing loss, so you never know. That said, Alice’s progress with her CIs has been fantastic – you really wouldn’t know these days.
Chris
Thanks Chris,
Its reall encouraging to hear of Alice’s improvement! I really hope Finn is put forward for CI’s as we are seeing no improvement at the moment with his aids. Have Alice’s doctors managed to pinpoint where throughout the hearing structure the problem was? I know this is relatively hard to determine in most cases, but I know in a slight few it is possible.
Natalie
Like you say, it’s very tricky. The best guess they had with Alice, owing to the wild fluctuations in her hearing and apparent decrease in her hearing throughout the course of the day, is that there was a problem with the neurotransmitter at the synaptic junction with the auditory nerve. This depletion of the neurotransmitter would account for her hearing tailing off. This was only a guess though. Either way, the CI’s bypass this junction and, so far at least, we haven’t seen any problems with Alice’s hearing fluctuating.
Chris
Like you say, it’s very tricky. The best guess they had with Alice, owing to the wild fluctuations in her hearing and apparent decrease in her hearing throughout the course of the day, is that there was a problem with the neurotransmitter at the synaptic junction with the auditory nerve. This depletion of the neurotransmitter would account for her hearing tailing off. This was only a guess though. Either way, the CI’s bypass this junction and, so far at least, we haven’t seen any problems with Alice’s hearing fluctuating.
Hi,
I am a mother of a child initally diagnosed with ansd. He’s 1 yr old now and upon the MRI scan, it appears he does not have a hearing nerve (even though he responded with hearing aids by turning to sounds above 70 decibals) . From your experience, can you pls send me Dr Berlin’s contacts or the contacts of key specialist that could help us – either with second opinions or has had success with CI even with cochlear nerve deficiency? We are based in Singapore where there are very limited access to signing.
much appreciated,
cy
Thank you so much for your note and for visiting the blog. I’m not really in a position to recommend health professionals – I can only speak for those individuals we have encountered along the way. I guess that from Singapore, you are better looking to Australia for help – there are a number of experts there who have experience of ANSD and auditory nerve problems. The Sydney Cochlear Implant Centre being the most obvious. We did meet Catherine McMahon from the macquarie University who alos has (i think) an affiliation to SCIC and she is something of a world expert on ANSD – http://www.hearingcrc.org/users/catherine-mcmahon
Charles (Chuck) can be contacted here http://experts.martindale.com/Charles-I-%28Chuck%29-Berlin-PhD/975672800010012-Expert.html .
He is usually very good at coming back to you.
Let me know if I can be of any further help.
Chris
Dear Chris,
Thank you for your speedy response. It appears the email for Dr Berlin does not work anymore. I have however contacted Dr Mcmahon. You are right indeed that Australia would be a good place to tap resources.
All the best to Alice and your family. You have been truly inspirational. Do let me know when you hear of other similar cases where CI or ABI was adopted.
thanks
cy
Cy,
No problem. When we spoke to Chuck, he was working at the University of South Florida – you may be able to ask them. Try this instead http://csd.cbcs.usf.edu/faculty/bio.cfm?ID=86
Chris
Hi,
My little boy is called Rhys, and we had the fortunate opportunity of meeting Alice and her mummy at Legoland a few months back (totally by chance). I would like to say a big thank you for introducing us to AVUK – I am so grateful for this recommendation. Glad to hear Alice is doing well, Rhys is still on his CI assesment and we are hoping the future is bright for him.
Best wishes
Faye and Rhys
Dear Chris,
We are a couple from Denmark and or youngest son – Aaron – was just recently (3 months ago) diag-nosed with Auditory Neuropathy Spectrum Disorder.
Needless to say we were crushed by the news – as Aaron has already been through so much that we figured he already had been given his fair share of struggles in his young life.
I would just like to thank you so much for this site!!! – It really does allow us to regain hope for our son.
When we received the news, I think we – much like other parents – had a million questions – but most and foremost we would really just like to hear: ”don’t worry – he will be able to hear and develop speech”.
As you know it is just not that simple. In the search for answers we stumbled upon your site – and thank God for that – reading about Alice just makes us think that it will work out in the end, but the ride will be bumpy.
The problem with becoming involved in the ”medical system” in Denmark is that no one can really give you precise answers and you are left with questions and having to make judgments based on just that!
Aaron is turning 2 years old March 28th next year and is going for his second hearing/brain test in January to see if his condition has improved in any way.
If not we are going forward with hearings aids and ultimately a CI-operation.
I understand Alice got her first CI at this age and her second when she turned 4 years old. What did you do in terms of trying to cope with her at home? Did you start her of with sign/cued language?
Problem here is that everything has to be processed through the system and takes time and so getting help to sign/cued language will probably take some time yet.
We experience that Aaron is very intelligent but so frustrated – and we are thinking that months in this period of his life are crucial not to miss – in terms of teaching – but what did you do?
When Alice got her diagnose did you then put her in a ”special child care” together with a blend of impaired children and ”normal” children where cued language was taught to all? Now Aaron is attending “normal” child care but is experiencing children rejecting him – because he has difficulties playing with them and because he cannot communicate he chooses to bite a lot. So he is going through a lot of personal defeats which we are not sure is the best for him – but on the other hand we don’t want to make him more disabled than he is.
Sorry for the questions, but we are trying to make the right decisions and we are a lot of years behind your progress with Alice today – but really hope to see our son come through like Alice.
Fortunately – all say – he has a will like a lion and is highly intelligent – which we also suspect being the reason for his constant frustration. He has an older brother (Levi – almost 4 years old) to look up to – and he just wants to do whatever he does – and God have mercy if you try to help him – then you will shatter his whole world.
In his first year – doctors threw a mild autism or even ADHD at us, even though that is naturally all too early to diagnose – but we did recognize some of the symptoms. Now after his diagnose we have been told that it is very common that you see signs that may look like ADHD/mild autism with children with Auditory Neuropathy Spectrum Disorder due to their high level of frustration with background noise and not being able to communicate properly.
Did you have any of these issues with Alice?
We have read a lot about all this – and we understand that the medical profession is divided in the question about whether or not CI will help children with ANSD – some children do very well and other do not – and once you walk down the CI lane you cannot go back in terms of hoping for your child to develop a natural hearing.
But in Denmark – the condition is only diagnosed 5-10 times a year among all born children and so it is really hard to get in contact with other parents going through this or getting good answers – it seems like you are a bit ahead in the UK.
Have you maybe heard of an international network for parents having children with this condition?
Again sorry for the many questions – and thank you so much for sharing this site with us!
Best regards and a happy new year,
Alex and Camilla
Alex and Camilla.
Thank you for your note – I find it hard to believe sometimes how Alice’s story has touched so many people around the world.
I am still on Christmas holidays with no proper Internet access, so will send you a proper note when I am back at my desk next week if that’s ok?
Chris
Hi Chris,
Thx. for your swift reply.
Of course it is ok with a proper note later on – I am just grateful for your time.
Hope you and your family had a nice christmas and happy new year.
All the best,
Alex, Camilla, Levi and Aaron
Alex & Camilla,
I just wanted to say that you are not alone in the confusing world of ANSD and which communication route to go down.
Since my last note we have continued to sign with Myla and this has really helped her frustrations as she is now 2yr 5mths and has no single words but will sign and is really going through a leap with it at the moment and wanting to copy every sign you do which is just amazing! We self taught through Cath Smith apps, LSL DVDs and books.
Myla is still going through diagnosis but is now under the umbrella of ANSD as her hearing fluctuates and has no acoustic reflexes and her abr hasn’t been repeated yet.
We get a lot of support from the elizabeth foundation which I would recommend you look at there website or contact them as they do home packages which are very helpful.
We also believe Myla is very intelligent and her anger and frustrations are purely down to not being able to communicate. Myla attends the Elizabeth foundation one session a week and visits another nursery for children with special needs two mornings a week as they have a higher care ratio and use makaton throughout the session where ever they can. Those mornings just give us both a breather Myla gets to socialise and I get the shopping done etc.
My plan in the new year is to get Mylas assisted listening device sorted as we are struggling with that at the moment,continue to sign, do more research and celebrate every step we make towards speech no matter how big or
small.
I hope this helps !!
Happy new year to you, Aaron and the rest of your family! X
Hi Sam,
Thx so much for your reply and interest.
It is so comforting to know that one is not alone with this ANSD diagnose.
I must say that my mood is ever changing at the time being – shifting from being positive and keeping faith to disbelief and sadness. I think our biggest concern is whether or not Aaron will develop his ability to speak!?
Right now we are applying for support from our local municipality and proper authorities, but boy is it frustrating to just sit and wait and having to argument, fight and shout like a mad man to get things going on.
As we speak 2 months have gone by and nothing has happened yet – no help to sign or cued language, nothing! I drives you mad because I sense that Aaron is really becoming more and more frustrated with his situation, and yet he seems eager and mature to learn alternative ways of communication.
Did you have major issues with Myla in terms of her being “hard to handle”. Aaron has always been a very energetic child – all over the place – having difficulties concentrating more than 30 seconds at the time, and having mood shifts from being in a good mood to being frustrated and crying a lot – and almost out of reach.
It has really been tough. But as I wrote doctors here say that dealing with ANSD can manifest itself in ADHD or even mild autism symptoms. Have you face any of this with Myla?
In childcare they describe him as having a lot of problems – having no friends (because he bites – as he has no other form of communication), dealing with personal defeats every day (because he knows he is different, but wants to be like everyone else) and having trouble being in a setting with too many children (properly due to him being frustrated by background noise I gather) etc. etc.
I understand you have choosen to put Myla in special child care – at least some of the weekdays – has this made a difference in Myla’s behaviour and her anger/frustration? Do you sense this being the right way to go about it? Sorry for my ignorance, but what is “makaton”?
At home Aaron is really a handful, but we believe he is a very loving child who is seeking a lot of comfort and security and really just wants to communicate but is not able to. We never taught him anything, but he is nodding his head “yes” and “no” and pointing at what he wants or coming to get us and bring us to whereever he wants to show us somehting.
So naturally we just want to start him off with some kind of alternative communication, but we are in doubt about which form to choose and should you just start of without professionals being offered by the “system” yet?
Thx again for your reply – it is so nice to be able to connect with parents dealing with this issue as well – no “outsider” really can comprehend what you have to deal with when facing ANSD.
All the best and I hope you and your family had a nice Christmas and a happy new year.
Alex, Camilla, Levi and Aaron
…Sorry for the misspelling – went a bit fast…
/Alex
Hi Alex, Camila and Aaron!
Would you like to e-mail me then I can answer all your questions as I feel my reply may be some long winded!! lol!
maguire765@hotmail.co.uk
It seems that Myla and Aaron have a lot in common, Myla is very hard to handle she hits out alot at anything if she is not understood.
The last six months since she passed her ABR amazingly ?? have been incredibly difficult for the whole family hence we have now got Private specialists involved and will be visiting AVUK this month.
It has been explained to us that Myla’s ABR could have been done on a good hearing day and the fact she has no acoustic reflxes points us back to ANSD. We also had a Private speech and laungage assessment which put her at 16mths old when she was actually 2yr 5mths they are also now looking for signs of dyspraxia but as you have already mentioned alot of these conditions appear to overlap.
We started baby sign before professionals got involved as we knew from already having two young daughters that Myla was not developing normal communication. (Makaton is sign langauge for special needs.)
Hope to hear form you soon
Kind Regards
Sam and Myla
(Chris Thank you So much for your amazing website!!)
Hello there!
We were given the link to your page by our Teacher of the Deaf. It’s good to share experiences and learn more about this condition. I’ve found out more information through talking to parents over the last 6 years, than I have talking to professionals, which is why I’m so grateful for this site. Thank you for putting it together.
My son James just had his 6th birthday before Christmas. He attends mainstream school and is thriving, although his language development isn’t fully formed, and his speech is sometimes confused, he is making great strides. We are very proud of him and what he’s acheived so far.
James’ was 6 weeks prem, low birth weight delivered by Emergency C-Section, and suffered from a malrotated intestine as a result of which he was in SCBU for 7 weeks. His hearing problem wasn’t picked up until week 7, through Newborn Screening and that’s when the ‘fun’ began. He had an ABR from an early age (at 9 months, from memory!) and again at 2 years. We introduced signing from a young age, first “Sing & Sign” and then Makaton, progressing onto a mix of Makaton and BSL. He doesn’t sign so much now; only when he and we are finding it difficult to communicate to each other which is thankfully, infrequent.
As all James hearing tests since birth have been consistent in their results, contrary to a fluctuating condition such as ANSD, we have asked for another ABR to be conducted which will help make the decision whether he has ANSD or not, or whether he has a speech delay/hearing problem. He is aided bi-laterally and this seems to be working. Grommets were inserted around his 4th birthday and subsequently fell out/removed although we’re still dealing with the aftermath of that.
James has also been assessed for additional speech problems and also dyspraxia which have both been dis-proved.
I’d love to hear from anyone who has similar experiences to us. I’ve not been fully conclusive here; I’d be here all day if I listed all of the things that we’ve been through!
Wishing you all well with your amazing kids!
Ange Chan
Dear Chris and family,
So our journey begins. Our daughter Ella was diagnosed with ANSD in Feb. of this year. She was born 7.5 weeks early. As I began my internet search soon after, your blog was the first thing I ever saw. I cannot tell you enough how appreciative I am that you created this. Reading about Alice, whom without even knowing I am so very proud of, has given my wife and I wonderful information and comfort. I know we have a long road ahead and a lot of questions to answer. Thank you again for taking the time to create this blog and I hope to stay in touch in the future.
Very Sincerely, Tom
Tom,
I’m so glad Alice’s story is of help and comfort. Do feel free to drop me a line directly if you’d like to talk further – we have made many new friends through the blog.
Regards
Chris
Chris,
Thanks again, I will keep in touch! We live in the U.S. in St. Louis, Missouri. Ella is doing great, she is 10 weeks old now. We have a second ABR test coming up April 11th. We have begun to meet with schools here. There are 3 schools in St. Louis which we will decide upon. What city do you live in? Congrats on the new baby, don’t know if you’ve had him/her yet? Have a fantastic weekend. Really appreciate everything. My direct email is tom2e@yahoo.com
Tom
Chris,
Congrats to you and your family, we are so very happy for you. He is beautiful!
Tom