# Anyone have experience of Auditory Neuropathy



## Charliej101

My little angel Lydia was born at 29 weeks following a fetal haemorage. After 8 long hard weeks on neo natal we finally took her home 2 weeks ago.
Just over a week later she had a second hearing test and we were told she had auditory neuropathy and her brain showed no response to any sounds.

We have been told there may be some improvement on her next test but I cant stop crying at the thought she may never hear my voice or say 'mummy.' 
I have read about cochlear implants which seem to offer some hope but all the information is so confusing.

Just wondering if anyone has any experience of this condition or the effects of hearing aids and implants.


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## Lottie86

Marleysgirl has a gorgeous little boy called Andrew and he has hearing aids so I'm sure she'll be able to give you some good advice when she sees this :flower:


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## Marleysgirl

Thanks for thinking of me, Lottie!

Hi Charlie, and congratulations on the birth of your little girl Lydia! Let me tell you my story and where we are now ...

Andrew was born at 29+1 due to bloodflow failure, he was tiny (1lb 6oz) and came out of NICU two days after his original due date. 

His newborn hearing test on the unit was inconclusive, so we returned when he was 4m actual (1m corrected) for a further newborn hearing test. He showed no brain response to sounds played, and he was diagnosed with Auditory Neuropathy Spectrum Disorder (ANSD). They feared he would be severely, if not profoundly, deaf.

_First thing we did at this point was to claim Disability Living Allowance, on the grounds that we had to supervise him constantly and have face-to-face contact as much as possible. We were awarded Middle Rate care, which enabled us to claim Carers Allowance and funded one of us to be a stay-at-home parent (OH)._

We were meant to return for a further newborn-style hearing test a few months later, but Andrew started being more active and we felt we couldn't keep him still or asleep long enough for that kind of test, so we declined. We returned when he was 9m actual for the first attempt at a hearing level test.

_I don't like the hearing level tests, they rely on him responding to sound on one side by turning to that side. If he turns, he gets rewarded by the sight of a hidden toy or puppet. But he's not interested in that puppet even when placed in front of it!!! _

After two attempts at a hearing-level test, he was given hearing aids set at a low level, this was around his first birthday. He hated wearing them and showed no visible signs of improved response. We decided he was too young (given his prematurity), and put them away until he was 16m actual age. During this time, the Audiology department arranged regular visits from a Teacher Of The Deaf, who comes every couple of weeks to visit us and Andrew at home, and to work with us on assessing and improving his responses.

Since January this year, Andrew has had hearing aids. But I have to confess, we have not been very diligent about him wearing them, and he only wears them (even now) for medical visits because his various support team expect him to have them. He had them on today!

Our opinion is that, whilst Andrew does have some hearing loss, it is nowhere near as great as the Audiology team believe (they still cannot measure it because he won't behave in testing). He has "tuned out" most background noise, and that includes us - this may be a result of being on the NICU where there is so much background noise going on that gets ignored. He doesn't respond to his name, but does respond to a loud shout of "Eggy Bear" (his nickname). He loves to play with noisy toys, whether they have lights or not, we have a fantastic video of him playing with a xylophone and giggling at it. He can tell when his toys stop playing tunes, even with his back turned, and will go over and start them again. And (this is our favourite!) he hears the National Accident Helpline advert (Underdog!!) whenever it comes onto the TV, even if he's not facing that way, he will stop what he's doing and walk over and stand in front of the TV till it finishes.

During the last 8 months, Andrew has had hearing aids set at three different levels by Audiology, and doesn't show any improved response with them at any level. We have tried teaching him sign language, but he has developmental delay and focussing issues, so he has only learnt one sign so far - that for "more". He is not yet talking, but we are being advised not to worry yet about this, he may just be late developing that too. He had MRI & CT scans a couple of months ago just to check on the actual growth of his inner ear, cochlea & auditory nerve, and they all appear fine on the scans.

We return to Audiology next Thursday, and this time we have to take our own toys in (those that he responds to), and also video footage of him responding to noises (such as the TV advert). This may help to persuade them that he does have some level of hearing, as he refuses to co-operate in tests so they cannot measure it.

ANSD is a "spectrum disorder" because it covers such different levels of hearing loss. Until your baby is older, you won't be able to tell whether the hearing loss is low-level or severe, whether she learns to compensate or not. I've had support from the National Deaf Children's Society, and also from a couple of good online friends who themselves wear hearing aids; but the best support has been from Andrew's Teacher of the Deaf, and his Special Needs Support worker. 

The crying that I did when Andrew was diagnosed with ANSD was nothing compared to the crying that I did when he was firstborn, on NICU, and it was touch-and-go as to whether he was big enough to survive. We still have occasional emotional wobbles, such as when we hear other 2yo's beginning to talk. But we have a fantastic little boy who has overcome so much to be here with us, he's an absolute joy. 

If you want to know more, or fancy a chat sometime, or want to compare Audiology approaches, or even just want to moan ... drop me a Private Message on here and I'll let you have my FB details and my email address. 

:kiss:


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## Charliej101

Thank you for replying and telling me about your're experiences. Im trying to remain positive I know how lucky we are to have a beautiful baby girl. I just feel lost as to what will happen in the future.
I have so many questions in my head I don't know where to start but I guess most of them can only be answered over time as we watch Lydia develop. 

Did the doctors ever discuss cochlear implants? 

Did you go to any support groups?

We received a letter today detailing her hearing test just wondering if you have any understanding of the numbers as to how severe her hearing loss is

Stimulus Click 1KHz 4KHz

Right AC >85 >95 >95

Left AC >90 >95 >100

Thank you so much for replying and offering you're support


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## Marleysgirl

I've just had a look back at the letters written after Andrew's various audiology appointments, and none of them tell us any figures in that kind of layout. You might be able to find answers about this in the information about newborn hearing tests on the NCDS website. In the narrative of one letter, they say that they tested Andrew up to 100dB and got no response, so it's possible that those figures represent dB volume tests, but of course it doesn't tell if that's the point where her brain showed response or not.

Cochlea implants haven't really been discussed for Andrew, they have concentrated on hearing aids for him. His recent scans showed that the cochlea & auditory nerves appear to have formed correctly, so his deafness seems to be an inability of the brain to interpret the nerve impulses as sounds. Cochlea implants are probably inappropriate in his case therefore, as even if they could bypass his ear and put those impulses directly into his brain (via implant) he wouldn't be any better at interpreting them. (Again, there's good information about cochlea implants etc on the NCDS website)

I say his deafness "seems" to be an inability to process, that's because we are convinced that he does have some hearing but that he is only just learning to listen to sounds and interpret them. Maybe his brain is learning to compensate, we really don't know. 

I get the impression from the hospital Audiology team and from his support workers that they try to prepare you for the absolute worst (profound deafness) from the outset, there was a keenness to fit him with hearing aids, sign language classes were recommended etc. Then as some level of hearing does manifest itself, it is a bonus and everybody breathes a sigh of relief. But this is just my opinion!!

We do go to one support group, but we've kinda been bullied into it :haha: Andrew started having regular (every 2-3 weeks) visits at home from a Teacher of the Deaf, who plays with him, assesses his hearing, chats with him and tries to teach him baby signing. The value of those sessions isn't very obvious, but we don't want to stop the sessions as he may need that level of support at some time in the future, so we want to stay on their books! That teacher works as part of the local Sensory Support group, and they run a fortnightly "music group" where they play with percussion instruments, sing nursery rhymes and do signing, and play with tactile toys. So OH reluctantly takes Andrew to these fortnightly groups, again because we want to appear keen. Andrew also receives fortnightly visits from a Special Needs Support worker (he has about 3m additional developmental delay as a result of a bout of epilepsy), and she has arranged sessions in a Sensory Room at the local SureStart centre, which he absolutely loves. (All of this kicked in around his first birthday)

I would suggest to you that if you feel a little isolated by your recent news, then you should go to support groups - but your daughter is young, and you don't necessarily have to go to support groups specially for deaf children, why not go to "normal" support groups? Baby & toddler sessions at your local community centre? Breastfeeding clubs? You don't have to separate yourself from other parents just because your child has a (non-obvious at this stage!) issue. 

I don't know where in the country you are, and different Councils have different support services, but every Council is meant to have a Family Information Service (you can google FIS to get contact details). They should be able to put you in touch with any sensory support workers/groups in your local area, if you feel that you would benefit from specialist support at this stage.

Keep asking the questions! And not just of me. If you can't find an answer to those figures, phone up the hospital and ask them. Make a nuisance of yourself. ;)


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## Charliej101

Thanks for answering my questions. Im starting to feel better it just came as such a shock especially as I had no knowledge or understanding of it. 

I read you're thread about Andrew's journey and it sounds like he is a fantastic little boy. 

I think the best thing for Lydia is for us to remain positive and see her as our beautiful baby girl before seeing anything else. Im hoping ill remain positive but Im sure theyll always be days of tears and fears for the future just like there was in NICU but we got through that

xxx


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## Marleysgirl

I would agree with you - although it is a scary diagnosis (particularly if you've no prior experience of deafness within your family), you still have a beautiful little girl to adore. 

They will probably offer a re-test in a few months, and if you think Lydia will stay still long enough, I'd encourage you to go along - I did read of a few cases where the auditory nerve continued to develop as the baby matured, and hearing levels improved. (It gave me hope at the time!)

We made a point of selecting more "sensory" toys for Andrew, a visible mobile dangling above the cot rather than one that just played sounds, that kind of thing. 

Think about contacting your FIS in the meantime, see if there are any groups you can join when Lydia is a little older. And consider the DLA thing - not everyone wants to claim, but you may well be entitled and every little helps!!


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## Marleysgirl

Charlie, just an FYI: the National Deaf Children's Society is participating in an online webchat on the NetMums website this Friday (21st October). Linky.


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## Charliej101

Thanks for letting me know Ill have a look x


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## daisii

Hi, I just found this thread. I am a Teacher of the Deaf and just wanted to say that if you want to ask any questions I'm happy to answer any. hope all is well with you and your beautiful LO's

x


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## Marleysgirl

I may take you up on that, when I've turned my random thoughts into some kind of sense.

Thanks.


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## daisii

please do, anytime.
x


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## Charliej101

Thanks
Its early days for us, Lydia should hopefully be getting hearing aids in December. Only thing I can think of now is if you can think of anything we can be doing now to help her (shes 5 weeks corrected) apart from treating her the same as we would a hearing baby x


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## daisii

Treat her the same as a hearing baby, but give her even more 'face to face' attention. Also when you are talking to her you could turn off background noise such as tv/radio so that she can focus on your voice. do you know what degree of hearing loss she has?


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## Charliej101

Thanks for the advice

The hearing test showed severe in one profound in the other ear but the audiologist said it is not a good test of hearing levels and they'll get a better idea from behavioural tests when shes 3-4 months corrected. We've not seem any startle reaction to any very loud sudden noises so I'm assuming the results are fairly accurate. 

Can she hear background noise? she's not got her hearing aids yet. Id hadnt thought about cutting out background noise as I assumed she couldn't hear it.

I try and give her lots of face to face her favourite position seems to be sat on our knee facing us.


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## daisii

given those levels you are right she is prob not hearing a lot but anything she is hearing will be better without background noise. Think about yourself in a noisy pub! it is difficult to hear because of the background noise, reducing it effectively helps your voice to be louder. But this will be more important post hearing aids. 

Have you thought about baby sign to help with early communication? I use it with my son (he is hearing) and it is so useful, from about 5 months he could sign milk and food when he was hungry or thirsty.

Also when she gets hearing aids, persevere with them! It is not easy (sometimes almost impossible) to keep them on a baby, but it will make a HUGE difference long term. Also a parent facing pushchair is great as you can keep an eye on the aids (i.e. has she pulled them out and is trying to eat them!) and it improves communication. 

hope this helps
x


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## Marleysgirl

We ended up having to make Andrew wear a "bonnet" to keep the aids in, otherwise he would use his tiny fingers to catch the tube and pull them out! It's taken until now (he's two) for him to finally accept the aids.


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## Charliej101

Thanks for the advice. Im planning on introducing baby sign, Ive got a book on it and been trying to find classes in my area but not been able to find any yet. Ive started doing a few for milk and mummy and daddy. 

Ive heard the aids are difficult to stick with but we'll try everything we can. It'll be winter when she gets them so I can probs keep a hat on her most of the time and see if that helps


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## daisii

there is a pattern for a hearing aid bonnet somewhere on the web, I'll post the link when I find it. Also this might be of interest to you both. It is the NDCS (national deaf children's society) site, they have loads of local groups as well as national events. Almost everything is free and they are always fantastic. 

https://www.ndcs.org.uk/family_support/support_in_your_area/about_local_groups/index.html


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## Marleysgirl

I just wanted to update this thread with the latest news about Andrew ...

We had an Audiology Review meeting today (as opposed to his regular testing). One of our frustrations has been the Audiologist treating Andrew as if he is totally deaf, because he refuses to co-operate in behavioural testing. We had told her that we think he can hear, but she's never really believed and has come up with other thoughts e.g. vibrations

Until today. We compiled some home video that shows him choosing to play with noisy toys (no lights or vibration) and he controls the sounds, it's not random hitting but a most definite press of the button to repeat a tune. We also had footage of him responding to claps & high-pitched voice, and of course we had to show him responding to his favourite Underdog television advert. The Audiologist was completely taken aback by the video. 

She now believes us, and wants to work with us to determine what frequencies he can hear, and to help encourage his "listening" ability (which is linked to his attention issues). No more talk of increasing the volume level on his aids, no mention of cochlear implants. We have come out of the meeting with a referral to a specialist Speech & Language Therapist who works with deaf children, and a potential referral to an Auditory Verbal therapist.

Progress!!!


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## Charliej101

That's great news, hope he now gets the support he needs x


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## daisii

great news! So glad you finally got some positive dialogue with the audiologist. It can only go in the right direction from here! x


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## CityGirlEire

Just a note to say, I didn't want to read and dash, my sons hearing test was fine as a new born... And he reacted with glee at any music.... To the point of refusing to sleep if he heard the music stop and waking up once it had.... However due to his other challenges including epilepsy, ADHD, sensory intergration disorder, learning difficulties and now they think dyslexia... He REFUSED to interact at all in the tests.... Refused to pay attention to a single thing.... My recommendation is if you need to, film your child and noise in the home where you can control the factors....... And I automatically taught him baby sign as a baby anyway as he gets so easily frustrated.... His speech was a bit delayed but that could be due to the epilepsy.... I find the signs which mr tumble uses are fairly easy to do, and are fun for children with and without hearing loss to watch...


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## Foogirl

Abby hears and speaks very well and also loves Mr Tumble. shhhh don't tell anyone but I love it too!


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## Marleysgirl

CityGirlEire said:


> He REFUSED to interact at all in the tests.... Refused to pay attention to a single thing.... My recommendation is if you need to, film your child and noise in the home where you can control the factors .......

Exactly what we did, and it paid off. 



Foogirl said:


> Abby hears and speaks very well and also loves Mr Tumble. shhhh don't tell anyone but I love it too!

Why the need for "shhhh"? Sherryberry and I took out littl'uns to see Mr Tumble live at Salford Quays earlier this year, was a very damp day (outdoors show) but great fun :happydance:


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