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profound hearing loss & balance/gross motor issues

Discussion
Posted by f
24/08/20 12:17 PM

Hi

My son was born with bilateral profound hearing loss, the cause of which is still unknown. We are awaiting our genetics appointment which is the last area that could provide a cause. He had cochlear implants at age 9.5 months and is doing amazingly thanks to the amazing efforts of the team! 

Our main concern is that he sat late around 10 months and although he has been standing and cruising since about 13months, he is still not walking independently at nearly 19 months.

I guess we are just worried about genetic syndromes related to balance and certain genetic conditions that would reveal a future vision loss  like Ushers syndrome that are known to be linked to balance issues and delayed gross motor development.

Does anyone have any reassuring stories whereby their children had delayed gross motor development in a similar case of profound deafness from birth,  but no subsequent genetic conditions/syndromes found as a cause? 

Thank you so much!

Discussion
Posted by dkm
08/09/20 04:13 PM

Hello,

My daughter has bilateral profound hearing loss and also has delayed motor skills. She sat independently only at the age of 18 months and started walking at 27 months. She was a premature baby and we adopted her when she was 5 months old, and her birth history is unknown. The cause for her delayed motor skills was reasoned to her premature birth status and lack of complete development. She also wears glasses.

Did your son's pediatrician refer him to a physiotherapist. If not, do get that sorted cause they check this further. Sometimes it could just be a delay and he can start walking all of a sudden one day. But anyways good to get it checked. Take care.

Regards,

Devi.

Discussion
Posted by f
08/09/20 05:45 PM

HI Devi

Thank you for your reply. Thanks for sharing the information about your daughter - hope she is getting all the support she needs. We saw the physio when he was not sitting, but they said everything was on track because he was sitting by the time we got the appointment. However due to Covid we have not seen them again but I will chase it up..thank you for reminding me. Thank you once again. 

Discussion
Posted by mmbn11
08/09/20 08:16 PM

Hi

Is he developing well in other areas? Speech? Communication etc? Is it just his physical development that seems behind? I know it may be a worry but it could just be a coincidence that he is slow to reach his physical milestones.

A friend I used to know had a little girl, she was a hearing child, no issues, healthy and same as any other kid as she is now (at 11yrs). But I was flabbergasted when she literally took her first unaided steps a couple of days before her 2nd birthday!! I have 15 nieces and nephews and had never seen anything like it! My son was the same age and running around and she was just about sitting up by herself and couldn't even crawl!! I couldn't believe it! But this can be normal, although probably less common. 
I have a bilaterally implanted 2 year old now and his balance isn't 100%, he can be clumsy and he has only had 1 appointment with the paediatrician regarding finding out the cause of his deafness. He passed his NHS and did respond to sound in the first few months but it just disappeared. I am less than happy about how he has just been left and no investigation into the reason has taken place. He had an ECG and a blood test that showed nothing.

he has been implanted for 13 months now and is still not really vocalising. I am told it's nothing to worry about because kids can develop at great different ranges. But it doesn't stop you worrying and becomin frustrated.

i hope all turns out ok and he will just crack on one day.

good luck x

Discussion
Posted by f
08/09/20 10:33 PM

Hi!

Thanks for your reply. Yes he seems to be absolutely fine communicating and he has started saying some words too. Fine motor and vision seems fine too. 

We have only had 1 paediatrician appt where she requested the connexin gene test and also some other tests for CMV etc - all negative. One year down the line we are still awaiting the kidney ultrasound scan. At the initial paediatrician appt she asked if we wanted a referral to the genetics dept and we agreed, but because of covid its all got delayed by like 6 months as our appt was meant to be around Easter. We then spoke to her briefly over the phone during the covid time,  but there was nothing further she said she needed to request. 

Can you see if your paediatrician will see you again/refer you onwards for further investigations if you want? I think it sounds like you've had all the tests the paediatrician can do as that's basically what we had done too! I really hope you get some answers but it seems like it all takes a while which is very stressful right? 

I've been told that after implants it varies regarding their speech development so don't worry,  it'll come.  Is your teacher of the deaf helpful? 

Do you mind me asking how old you son was when he started sitting and walking? Im starting to wonder whether perhaps the clumsiness and balance issues are in fact normal because of the hearing loss and perhaps it will improve over time (I hope)!

Discussion
Posted by mmbn11
09/09/20 10:32 AM

Well that's a good sign. 

try not to worry too much unless you have been given something to worry about. But it is hard, especially  at the moment because everything is just put on hold, it's very frustrating. 

To be honest, when it was all happening he was newly diagnosed and I was thrown into a massive world of information, coming at me from every angle you can imagine. I was on my own at each appointment as his dad is in the army and based down south and all my family and friends are in Cumbria, so I had to take all the info in by myself and when I came to recall it, it was just all mashed up and I wasn't actually sure what they said or what was going on!

They didn't offer any further testing, they did mention genetics or something but it wasn't an offer, it was for the future, like if he needed to know or had kids or something, but I may have gotten mixed up. I know for definite that he wasn't offered anything more in the immediate future though. And he hasn't seen him in almost 2 years.

his ENT consultant at our local hospital has cancelled 2 recent appointments and I am awaiting another. She said he would chase the pediatrician up so I got an appointment to see him again but that was almost a year ago and I haven't heard anything from either of them. 

When I see the ENT finally I will really push to see the paediatrician.

His ToD is very good, and kid CI key worker so I am thankful to have them. I hope it does come and there isn't something the matter. But I am

told it's ok at this stage so I just have to go with it.


No of course... I can't remember honestly but I know it was very slightly after the usual age but nothing concerning. he was very wobbly and clumsy for a long time and even after a friend of his (2.5 months younger) started walking she steadied up way before Noah. he is still a bit clumsy and although very subtle, he can still be a little off with his balance. I know hearing loss can naturally cause a little problems with balance and doesn't have to be underlying issues so just think positive and keep going. 
Thats all we can do for The moment :)

Discussion
Posted by curlymoptop
09/09/20 12:43 PM

Hi there

I don't know if this will be reassuring or unreassuring, but all I will say is please enjoy your wonderful baby boy and don't get too hooked up with "what if he has this" or "what if he has that?"  You will drive yourself crazy.  It may just be a gross motor delay, completely unconnected with his deafness, and then you will have worried for nothing.  If his deafness is under the umbrella of a genetic condition, there is nothing you can do to change it.  It will be hard, you will need support, but you will accept it and deal with it head-on, like you have dealt with everything else.  Us parents are stronger than we think!  But, of course, we are allowed to be blubbering wrecks in the process.  It's only natural.  But psychologists always tell us to only worry about the things we can change, not the things we can't.

My son was also born with a profound hearing loss and gross motor delay.  I immediately put two and two together, just like you.  He finally walked at 20 months.  Our physiotherapist said it was unlikely that there was anything more going on.  He was also diagnosed with severe verbal dyspraxia, so I learnt BSL and he had weekly SALT sessions.

We, too, underwent various genetic tests which all proved inconclusive or negative.

He started wearing glasses to correct his vision aged 5 years.  It was only the chance spot by a locum optician at age 8 that sent us down a completely new path we weren't prepared for.  First it was GOSH and then Moorfields Eye Hospital.  The genetic testing we had had was competely focused on the deafness, but now there was a new slant regarding his vision.  He had tests for his eyes - also inconclusive - but then two years ago, we were told they could screen for 150 different eye conditions.  That resulted in us being given the news that he had Ushers type 1.

This year, before Covid, he had his vestibular function and balance tested.  All completely normal!  He's 15 now, approaching his GCSEs in a mainstream school, and he's on target for getting 8s and 9s in all of his subjects.

Don't let any diagnosis define your son.  We tend to hear more about the worst case scenarios, which is unhelpful.  But there are spectrums to every condition.  Please try not to worry.  I've had so many sleepless nights, but it will be ok.

Discussion
Posted by f
11/09/20 10:05 PM

Hi

@mmbn11 thank you for your reply. I’m sorry it was so difficult for you at the start but am glad your ToD Is helpful and you have managed to get support..I think once you see ENT again you can ask more questions/get referred back to the paediatrician and hopefully get a few more answers. Yes you’re right, keep positive and keep going, agreed!

@curlymoptop thanks for your reply too. Yes you are quite right..even if it something genetic I cannot change it..I am guilty of over worrying and I know I need to stop! Wow..definitely a lucky spot by the optician but am so glad that his tests are all okay currently and he’s doing really well! 

Do you both have other kids or just the one? I guess in my head the other question is if tests etc take years to come through/a diagnosis to be made how that impacts on thinking about future children who may be affected..a minefield of worrying and what ifs I guess! 

when I started this thread I wasn’t sure how many people would reply but I do feel reassured that others are there for advice and support so I’m grateful for everyones replies! 

Discussion
Posted by curlymoptop
13/09/20 01:18 PM

Hi again f

We have just the one child.  After the Ushers diagnosis, we were referred to a genetitist to see what was what.  It was found that both my husband and I carry the recessive gene that is associated with Ushers.  We couldn't have known.  Neither of us have any deafness or sight conditions in the family.

Our son had a one in four chance of inheriting both of our dodgy genes.  That would have been the same for any other children we may have had.

Of course it's going to play on your mind when thinking about future children, but none of us know what dodgy genes we are carrying! And it's only if we have a child with someone who is carrying the same dodgy genes that problems might occur.  But even then it's not a foregone conclusion.

And, as parents, "overworrying" is not a thing.  You can't worry too much!  It is completely normal.

Discussion
Posted by mmbn11
13/09/20 01:39 PM

Haha @curlymoptop you have posted that comment about 27 times 😂

Hi f

yeah I have another son, he is 12 and he is hearing with no issues to my knowledge. He does however have a different dad to my 2 year old. 

i wouldnt know if we both have the dodgy gene as the person who donated what I needed to have a baby (I do not use the 'f' or 'd' word) has no interest and hasn't been part of his life. 
so I am not sure what answers we will be able to get. But I also dont have to worry about it happening again. 

I will certainly be more forward this time round. You know what it's like at first, you're like a rabbit caught in the headlight. You're just floating about and being nudged in the right direction by the professionals and don't know enough to have your own say. This far into the process I am not as shell shocked so will be more alert and pushing for what is necessary.

i hope you find all the answers you need. Thing is, if it isn't the best news, it's better to just get it out of the way so you can re-adjust and get on with things. And if it is, you can put it out of your mind. It's the not knowing that makes you worry. 

just try to think positive but also prepare for receiv the news you don't want. Either way, just enjoy him for now. I have spent so long obsessing over the things noah ISN'T doing, instead of enjoying the things he IS. its Easier said than done I know. But try your best x

Discussion
Posted by curlymoptop
14/09/20 01:41 PM

Thank you mmbn11.  Blimey, don't know what happened there! I honestly only posted once, and then I got about ten email confirmations that my comment was live!  Followed by several more saying that my comment did not meet community guidelines and that it was being removed.  So I spent the whole of last night worrying that I had offended someone.

Maybe "dodgy" wasn't quite the term I was looking for.  Maybe "altered" would have been better.  But it seems that only my extra comments were removed, not the actual comment itself, but I do aplogise if anything I have said has hit a nerve.  Having a child with special needs and talking about it can be upsetting for someone else reading it, if they are at an earlier stage of that journey.  But everyone's journey is different.  And how we cope with news is different.

For those few years when my son was being tested for Ushers, I was happy to get the "we don't really know" news from the doctors, because it still meant there was a chance of a happier ending.  But I worried constantly.  Every single day it was on my mind.  The day we got the diagnosis (from a doc with no bedside manner whatsoever) I felt like someone had kicked the life out of me.

However, fast forward a little while, I actually now feel better knowing.  As for my son, he doesn't think too much in to the future.  He concentrates more on the here and now, like getting teachers to be more deaf aware.  So I'm trying to adopt that approach more!

You will be fine, f.  I honestly don't know sometimes how we get through these challenges, but we do!

Discussion
Posted by mmbn11
14/09/20 02:09 PM

Haha, yeah, I got about 10 emails alerting me to a new comment 😆

well you certainly didn't offend me.

none of this is easy for anyone but it is harder for others. All you can do it make the best of it and pull the positives out. your Son sounds like a really positive boy and that's testiment to you 😊 

Discussion
Posted by mmbn11
14/09/20 02:37 PM

That sounded like I meant it's harder for Thera than it is for you!! I meant it's hard for most but some have it worse. Like deafness and then ushers.... one is worse than the other and you go through so much more.... just so I don't offend 😬

Discussion
Posted by mmbn11
14/09/20 02:38 PM

That sounded like I meant it's harder for others than it is for you!! I meant it's hard for most but some have it worse. Like deafness and then ushers.... one is worse than the other and you go through so much more.... just so I don't offend 😬

Discussion
Posted by f
21/09/20 02:32 PM

Thanks for both Your replies! We had our genetics appt and the doctor is going To request a whole host of genetic tests so will keep you posted! 

Discussion
Posted by mmbn11
21/09/20 03:12 PM

Ah that's good news for you. Yes, keep us posted on the progres. will be interested to know what's going on. good luck x

Discussion
Posted by curlymoptop
21/09/20 04:11 PM

That will certainly be a way forward for you.  Some results seem to take an age to come back, so try not to worry too much while you're waiting.  I hope it all goes well for you and, yes, let us know how it goes.

Discussion
Posted by jaybird
28/02/21 09:21 PM

Hello! Slightly late to the party (!) but just seen your post.


My daughter is 12 now, and was diagnosed at 2y 8m as having a moderate hearing loss in one ear and profound in the other.

It was her delayed gross motor skills that was the thing that caused me to get her 'seen' when she was about 18 months old, as she was unable to walk at that age (I had two older children who had been walking at 12 months ish) and fell a LOT even when sitting. I was concerned about her balance and general motor development and initially the Drs referred us to orthopaedics to see if there was any issue with hip/bone/skeletal development that could be causing the issues - there wasn't. 

She did start walking by herself around the age of 2, but was constantly falling. She would fall even when standing still if she turned her head. 

We also noticed that she wasn't hearing directional sound very well, and so knowing that ears and balance and hearing are all connected, I pushed for a hearing appointment. For a number of reasons it was hard to get that appt, but eventually at 2y8m she was seen by audiology who were amazing - and we discovered that she was deaf - the extent of her loss was quite a shock to us as she was developing language 'normally'.

Anyway - long story short was that it was apparent that her deafness had been caused by a CMV infection, and that this had also impacted her vestibular system.

A number of consultant appointments later, both at our local hospital and at GOSH, and she was 'diagnosed' as having no vestibular function at all. The vestibular system is the part of our ear system which orients us in space and tells us which way is up, which way we are moving and whether we are going fast or slow.

My reassurance/positive for you is that now, at 12, you would not notice that there is any issue at all with her balance. She did learn to walk, and to run and jump. She can swim and dive, and learned to ride her bike without stabilisers before she was 5. Don't get me wrong - it wasn't easy - she had several years of physio and has worked incredibly hard at developing core strength and flexibility with ballet, gymnastics and other sports. There are some things that she will always struggle with - she cannot balance in the dark for instance - but other things which she has taken as a huge positive, like that fact that she can read on car journeys as she is physically unable to get travel sick!


Anyhow, I'm more than happy to message further if you have any more questions or if our journey could be helpful for you. I hope that in the 5 months since your post, you've had more answers and help from your professionals too, and that you're all well :)


jaybird

Discussion
Posted by f
26/05/21 09:31 PM

Hi all

It has been a long time since I posted on this thread and thank you for all your replies. 

In the last few weeks we received a call from genetics to say that they found a genetic cause of my son's deafness - it is autosomal recessive - due to us as parents being carriers for the gene. It is the TMIE gene or Transmembrane Inner Ear gene. It is quite rare and came as a big surprise because we have no history of deafness in our families or each other. 

We are going to have carrier testing done on ourselves to confirm that this is the case.

There is hardly any information about this gene, but as far as they know it only causes deafness. So still unsure about the balance issue and also about any future issues. So I guess we will just take it as it comes and see what happens!

Just thought I would update you all!

Discussion
Posted by mmbn11
26/05/21 10:10 PM

Hi f

wow, that's been a long time!! But it's great that you have an answer as to what caused it and seems good news that it generally only causes deafness. How is he doing now? Any improvements? 
perhaps its all just late developmen, it can happen and it's a worry when you know other things can be linked to it so your mind assumes the worse. But you're right, we cant change anything so I guess you just have to wait and see but it won't stop you worrying.

Can I ask what was this 'genetics appointmen'? So after you said I should ask the consultant I did when I finally got to see him a few weeks ago and he said he was going to get him in for a scan on his heart (his lips go blue when he exhurts himself) but said with all the other info I have given he is only doing it to put my mind at rest as he thinks he is fine, but also do something called a genetic panel test or something 🤷🏼‍♀️ can't remember the term but it's basically testing lots of genes, I am sure that's what your son had. However I was having a meltdown when they suggested taking blood for it so asked if there was any left from last time (I know it sounds ridiculous but he said that they would likely have samples kept for the future for genetic testing at his first appointment so I wanted to avoid any more needles, he went through hell last year with an infection in his implant site and daily canulars, sometimes TWICE daily because they were constantly failing and ended up going under general anaesthetic to put a long line in which is supposed to last and that lasted one use too!! Anyway, that's another story), so he said he would get on to the lab and find out if they had any stored and let me know if he needed to come back for a blood test. Haven't heard about either thing 🤨 I will have to ring up. But you said you had an appointmen, was that just for the blood test? And how long did you have to wait to get the appointment and for the results to come in?


Discussion
Posted by f
26/05/21 10:18 PM

Hiya

Thanks for your reply. He is still off balance and still tilts his head to one side when walking - this is intermittent - once again unsure if there is any other issue - his paediatrician says to watch and wait! 

He is otherwise well and doing well with his speech! 

So we finally saw genetics face to face in Sept 2020. They initiated the genetic panel testing on his newborn blood sample. We just got results in May 2021. So took a while...she said they tested the usual hearing panel which included things like Ushers genes etc which is what my main worry was. 

I think that I still am not 100% convinced re the diagnosis (maybe in denial!) - once myself and my husband have been tested and its confirmed we carry the gene I will probably be a bit more reassured that it is this gene and nothing else! 

hope your son is okay and the scans etc are all normal. I hope you get some answers soon..the waiting is the hardest part! 

Discussion
Posted by mmbn11
26/05/21 10:26 PM

Well that's great that he is improving but I understand why you are still concerned and not happy until more details are in, I am exactly the same!

it's a massive long and drawn out process isn't it, just to get some answers! It's a worrying time so it's really not great 😳

i was aware you actually saw genetic people, just thought the doc requested it, then you got results 🤔 Either way, seems like I am in for a long wait, and you will be too for your next lot I expect! 

i am sure the results are accurate so keep us posted x

Discussion
Posted by curlymoptop
27/05/21 11:25 AM

How lovely to hear from you both again!

Yes, genetic testing takes an age.  The blood takes an age to be screened and then you get put on a long waiting list to see the genetetist, as appointments are highly sought after.

It's great you have some answers, f , and it could be a very long time before all becomes clear - if it ever does.  They are only screening for genetic changes that they have suspicions about. But we could all have something lurking inside us, ready to emerge at a later date. We just don't know.

My son has had two check-up appointments cancelled at Moorfields Eye Hospital in London, because of the pandemic.  Instead, I have been given a telephone appointment for next week! Too funny.

Keep in touch xx

Discussion
Posted by dp
11/06/21 09:10 PM

Hey I was looking for some advice on how to get a 4 year old to keep his hearing aid in ?