Episode 2 - Wait and see or maximise hearing

Introduction

Hannah Cooper

Hello and welcome back to Little ears, big challenges, where we're talking about mild and unilateral deafness in children. My name is Dr Hannah Cooper.

Amanda Hall

And I'm Dr Amanda Hall. Today we'll be talking with parents about their decision making processes around technologies and other management options for their children. There isn't clear evidence about what works for mild and unilateral deafness, so this can make decisions difficult.

Hannah Cooper

We ask Claudia, Jo, Louise, Kim, Abby and Waqas to tell us whether they chose to wait and see or maximise hearing and to talk about what motivated their decisions. We also asked them about whether they challenged the professionals looking after their child, or sought a second opinion, and whether they could tell us about any technology their child uses. Some of the parents mentioned a device called a BAHA, which is a bone conduction hearing aid often worn on a soft band. Some parents also talk about the cause of their child's deafness, including conditions called CMV and EVA. CMV stands for cytomegalovirus and is a common virus that is usually harmless, but it can sometimes cause deafness in babies. EVA stands for enlarged vestibular aqueduct and is a difference in a person's inner ear, which leads to deafness of varying degrees. Let's hear from them.

Abby

Yeah. So at the beginning, obviously Betsy was very little. Six weeks, six weeks when we found out that she definitely had the profound hearing loss, and that we decided obviously not to do anything then. We were with audiology and I've got to say that they've been absolutely fantastic throughout. We've always felt so well supported by them and different charities as well that have helped with kind of her diagnosis and how we navigate it. We always felt like it was up to us, so myself and my husband, it was our decision. Never felt pressured, but also never felt like if we'd suggested something, it would get shut down. There was always kind of an open conversation and I think with because she's got a unilateral hearing loss, her right ear has always stayed as it should essentially, we would call it her good ear. So we were told that with her good ear, she would be able to access all speech and language sounds and as long as the hearing in that ear didn't change that, we wouldn't potentially need to think too much about cochlear implants and things like that. But with the condition she was born with, which caused her deafness hearing can change quite quickly and it can deteriorate. So she has regular checks, we go every three months to audiology to check on her right ear, to make sure that it's still good and touchwood, luckily, it's stayed the same, and hopefully it continues to do so.

Hannah Cooper

Abby, are you happy to talk about the condition that Betsy has?

Abby

Yeah. Of course. Yeah. So she was born with a condition called cytomegalovirus, or CMV for short, which lots of people haven't heard of. Most people haven't actually. But it's actually the leading cause of hearing loss in children so it's something that we probably do need to be more aware of. We were really lucky, when she was born, we found out on day two, day three, when she was in the special care baby unit, which meant she could go on a course of medication to try and lower the viral load, which hopefully has helped her right ear. Lots of people aren't lucky. They don't find out that their child had this condition until they're, you know, five, six, seven, sometimes a lot later even, and by then it's almost too late in the damage is done. So yeah, we feel really, you know, unlucky that she was born with it and it's caused her hearing loss but lucky actually that we found out early and we could we could treat it. So yeah just because of that we have the very regular hearing checks and so far so good with her right ear.

Hannah Cooper

And how did you make a decision about hearing aids or technology at the beginning?

Abby

We kind of put everything on the table. When we first found out we were quite, I think you kind of spiral a little bit. And I remember my husband online booking BSL courses, you know, to start the next day locally to us. And then we spoke to audiology and they were like, at the moment we don't think that you'll need that, we're quite certain that she'll be able to communicate with you as, as your other, as your other son and as other people, so we put the brakes on that. And then I discussed, we spoke about cochlear implants and again, I think if we if we’d pushed for it, it's something that we probably could have, we could have gone down that route. But just by speaking to different people and people in different support groups, other families that have gone through it, we kind of held off on it. And then it was only when Betsy was about two, two and a half and we went for a hearing check up, and she just gets very congested. Betsy, in the winter months, and, unfortunately, it's always her right ear. Her left ear seems to get congested and clears really quickly, which isn't really of help to us. But it's her right ear, and obviously when it's congested, it then impacts her ability to hear those speech sounds and everyday sounds that she would need to hear. So it was then that we decided to go down the route of a bone anchored hearing aid, so a BAHA. Yeah, to just under two and a half she was, and we've been using it ever since.

Hannah Cooper

Is the BAHA on a soft band?

Abby

Yes, on a soft headband on her left ear. But during potentially autumn and winter, when her congestion is quite bad, we actually put it on her right ear so that, again, she's able to access everything there, yeah.

Hannah Cooper

Did you challenge professionals or look for a second opinion?

Abby

No, I've got to say we never did. We've always, we really, really do feel lucky because I know lots of people, everybody has different experiences and we've never felt like that. We saw a lot of different, we had the same audiologists, but we would see still a lot of different people within audiology and we always felt so well supported, never felt undermined or that they were pushing anything on us. We yeah, feel really lucky, actually, that we had that support to start with because I think it's made all the difference to us for our experience and ultimately to Betsy.

Hannah Cooper

Anything you want to share about the hearing aid?

Abby

Yeah, we've I was I was a bit apprehensive about starting it purely because the class that we went to, children had all sorts, so there were some that had double cochlear implants, I'd seen the BAHA a couple of times. Lots of people just had the inner ear hearing aids as well, lots of the little children, and I'd seen a lot of them struggling with it and not wanting to wear them and kind of pulling them off all the time. So we were a bit panicked about that. And in the BAHA as well, the battery is absolutely tiny which is the only thing, you just have to keep your eye on it, because obviously if one of them goes missing, I mean, you don't know where it's gone. So that that was something that we were a bit concerned about, but with the BAHA it just sits on a little soft band, which again, I think for Betsy as well, probably being a girl if it's worn, lots of people just think she's wearing a hairband and they wouldn't actually notice the hearing aid when it sits behind her ear. We've always, she's always worn it, we've always put clips in, like to hold it in place and with the BAHA as well, I would say if anybody does have one, and I think they do give them out with the BAHAs, but you can get a very, very small, it's just a really a clear, thin thread that attaches to the BAHA that you then hook on to their clothing so that if, you know, you're in a soft play or, you know, they're in the garden and they're running about or they're on a trampoline, if it was to come off the band, it should then stay clipped on to their clothes, because otherwise, obviously it's about the size of, it wouldn't even be like a £0.50 piece, it's a very, very small little hearing aid, and if you were to lose that somewhere, you'd probably hear it because you can get quite a lot of feedback off of the BAHAs, I've got to say, and that noise is, oh, gosh, I think I'll always hear that noise wherever we go! But yeah, I would just say that's a top tip. Make sure you use that and use it all the time with the BAHA and then clipping it if you can. It's obviously a bit trickier if you've got a son with shorter hair, but just trying to almost keep it attached to their, keep the band attached to their head.

Clodagh

Yeah. So we went down the maximise hearing route. We opted to aid from, you know, the moment we found out that Bethany did have that hearing loss. Our audiologist gave us the option to not aid and wait and see but Bethany's our first child, so I, we didn't know what was normal and what wasn't. We didn't know if, you know, if she started talking at a year or three or, you know, we didn't know what was going to be normal. Obviously, you can spend your life reading things on the internet, can't you? But for us, we just wanted to give her as much as we could from the outset. So obviously it's hard, isn't it, because it's hard enough making decisions for yourself. And what's the right choice for you but to be making choices for your child, and you don't know whether they're going to like the hearing aid, which in our case, she didn't and whether it is going to help or hinder, but from our thought process, we didn't think it could hinder, we thought it could only do good and we chose to get our hearing aid as soon as we could really.

Hannah Cooper

Did you challenge professionals or look for a second opinion?

Clodagh

No. For us, like I said, our audiologist was fantastic from day one and continues to be.

Hannah Cooper

Anything that you'd like to share about the hearing aid?

Clodagh

A couple! Well, as I alluded to earlier, to start with, Bethany wouldn't wear the hearing aid, it was an ongoing battle. I would try every day after she'd get upset, I would continue to try every day, but I wouldn't force it. And then one day, the teacher of the deaf went into nursery and just, you know, got it out of the bag and was playing with it and Bethany just put it in, and that was it, and she's worn it every day since. Another time we were walking through town and we'd crossed a road and we got to the other side of the road, and Bethany was going ear, ear, ear and like, why are you saying ear? So I looked and it had fallen out on the other side of the road, and I wouldn't have had an idea if she hadn't have told me that her ear had fallen out! So yes, I was very grateful she did that day. And the other day, Saturday just gone actually, we got in, we'd had a busy day out and about, and we had got in and we'd been playing in the living room and she'd taken it out, again fine. But she's going through a phase where I can't leave her side at the moment, so I had just popped it onto the edge of the sofa, and the next morning I had gone to put it in, and it wasn't in the pot where I normally put it, and I thought, oh my gosh, where did I leave it? Remembered I'd left it on the side of the sofa, went to the side of the sofa, and it wasn't there. And I thought, oh my gosh, I've lost it, what am I going to do? Anyway, I found it down the side of the sofa, it had just fallen down, so yes. Initially I sort of thought when we'd got it, oh, I'll never lose it, you know, I'll never misplace it will be fine, but it's very easily done!

Hannah Cooper

I suppose parents really have to be careful of where they put it and make sure the child isn't playing with it.

Clodagh

Exactly. Yeah. And to start with, when Bethany was wearing it, you put it in and she'd be playing with it in the car seat, so, you know, and you're driving along and you can't do anything, you know, you're trying to say, pass it to me, pass it to me but if they're ignoring you there's nothing you can do whilst you're driving. So we went through a period of not putting it in until we'd reached our destination. But now we can put it in before we get into the car and she's absolutely fine now.

Joe and Louise

Louise

So obviously, we knew she was single sided, deaf. He had an MRI scan in the March 2023, and that revealed he had bilateral EVA. And then that triggered a genetic test basically because they said it could be associated with some syndromes. And then we found out he had Pendred. And when we googled it, because we didn't get any information about what it was, it was just literally your son's got EVA.

Joe

Yeah. They're going to lose the hearing.

Louise

When we went on Google it just said it's progressive loss, it can happen gradually, suddenly, or some people are lucky, it doesn't happen at all. But, you know, I'm a very anxious person anyway, and I was like, I couldn't believe it was happening. I was like, this is not, I don't know how I'm going to are going to control this.

Joe

I don't think it helped as well, you know, we rang George's paediatric consultant and just for advice to say, you know, what is this is what we're reading correct, because we have no information on it. And when we spoke with him, he was open and honest, you know, he apologised, he said there should be information for you there rather than you have to turn the Google. Yeah. But you know, he openly admitted he wasn't familiar with the condition.

Louise

And again, like obviously we’ve mentioned implants since George was about six months old but we sort of settled down a bit because we were seen as a bit of a problem for asking for this. So we just sort of put it in the back of our minds for a bit but when we found out about the progressive nature, we thought, surely you know we can be referred to a centre and we can speak to a surgeon just to express our concerns.

Joe

Our approach, we've always wanted to maximise hearing, you know, we see that as doing the best by George. And we felt that, you know, from our early engagement with the NHS and, you know, guidelines, it's a very much a wait and see strategy. And it's, it's horrible because you think to yourself wait and see and you know, you think wait and see, wait and see our son's hearing decline. See how bad it gets.

Hannah Cooper

Did you challenge professionals or look for a second opinion?

Joe

With challenging professionals, you've got to remember professionals are individuals as well. And so you can have a good experience, you can have a bad experience. And you know, if you're not entirely happy with the way any of your care team or, you know, any of the professionals that were related to looking after your child and managing the treatment and care plan, you know, seek second opinions. Don't let them be a blocker. You've got to push. And I would say the biggest benefit to us, you know, although we were kind of forced into it was do your research whether that's reading, you know, published papers, journals, not just in the UK but worldwide. It can be exhausting and time consuming. But having that information, you know, we challenged audiology we challenged CI centres, we challenged NICE. We wrote to them and they were in support of us, NICE. But the, you know, some of the local team were actually blocking that because you needed them, to submit new evidence or support any changes, your general person from the public can't do that. But we can collate evidence and give that to a professional to submit. But they weren't willing to support that.

Louise

Because NICE recommended an individual funding request, which was something we weren't aware of because they said the guidelines aren't the law, they're just a guidance.

Joe

I think I think the take away from this, just as a short kind of headline is, you know, speak to as many people as possible, get as much information and the differences, you know, that we've seen is one place has took over eight months to reply to us and another place gave us an hour of their time on an unplanned call out the blue. That gave us a lot more information.

Louise

I think the main thing for us was that at least the tried, that's all. We wanted to push it as far as we could with the NHS because obviously me and Joe, we're not rich, we can't, it's a very expensive surgery.

Joe

I think the whole thing just it felt difficult to get information and the information is so scattered about, you know, the NDCS do a great job, their website’s got a lot of information. It doesn't have everything. But then, you know, it took months and months for us to find all these other bits of information and there's no real central source where you can find kind of absolutely everything. And to pull that together and understand, you know, what the regulations are and what can and can't be done within the UK.

Louise

Because I think like someone with a 70 decibel hearing loss, it's going to be a completely different experience with somebody else with a 70 decibel hearing loss. You know, some people with 70 decibels do very well, whereas some people over here probably aren't doing as well and would benefit from a CI. But yeah, we just we believe that, you know, just maximise here and now and why wait for something to go wrong. Like surely it's best to stop it in its tracks.

Hannah Cooper

Anything that you want to share about the BAHA on the soft band?

Joe

It was unique. I mean, you know, it's probably one of the first times George had something on his head. So he was wearing it on a soft band. And I think, you know, as with a toddler, the first thing they do is rip it off. The important thing is show them what you're putting on the head, let them play with it, you know, under supervision, let them see what it is and then put it on. Yes, they're going to pull it off, they're going to pull it off a hundred times and it's just keeping your patience, keep them calm.

Louise

We found when he, if he was distracted, he would keep it on, no bother. But if he caught his reflection in the mirror, or if he was just wanting our attention, he would just, he would look at you when you've done it and he would just slowly take it off. And he knew what he was doing, didn’t he!

Joe

I think try different retention methods as well. So, like, some are just nice little thin elasticated bands, but others are quite big sweatbands, you know, there's a lot of different methods out there, and I think it's just what suits your child. And it's I think you've just got to keep calm, understand they're a child, they're going to pull it off and, you know, test your patience but just don't make a big deal out of it and they'll come around to it.

Louise

We ordered different coloured bands as well didn't we.

Joe

Different colours with different pictures on so George liked animals at the time, so he had like a light green one with the jungle.

Louise

And then we've got stickers as well for the BAHA just to make it look a bit more fun because it was quite a plain colour, wasn't it?

Joe

Yeah and just let him pick out the stickers and, you know, there's, George later had hearing aids as well. And there's a great company that does all the custom sticker sheets so you can get like Batman or Toy Story ones or just colourful. Yeah. It's, just make it make it stand out. You know, it's not about hiding them.

Hannah Cooper

And did you feel that you noticed a difference with the hearing aid?

Joe

I did, I remember straight away in the corridor just as we were leaving audiology, I said his name, and he turned, where prior to that he was at a point where unless I really raised my voice to a loud level, he wouldn't have.

Louise

I think the only time I just didn't really benefit him was like, say like a noisy playgroup. That was the only time I think it used to, just because obviously it's all the sounds going to one ear. Like we can filter background noise. Just too much noise. But yeah, other than that, you know, we did notice a difference, especially in a more like quieter environment, didn't need to raise, you said you didn't need to raise your voice.

Joe

I think you've just got to remember with, like, any of the hearing aids and, you know, deafness, it's all individual and everybody's different, you know so just because somebody has this type of experience or goes down this route, doesn't mean your child's going to do the same.

Louise

It's like, I know there's a lot of children, their parents decide not they give them any like hearing device and they still do well and you know, that's not a wrong decision. What works for one family doesn't work for the other. We know there's some families probably wouldn't go down an implant route for a single sided deaf child, but that's fine. You know, it's just everyone's different.

Joe

But I think it's just important to expose yourself to as much information as possible and make make the best decision that's right for you, your child and family really.

Louise

And I just think maybe as well, if audiologists could, just from the one experience we had with what like one audiologist, it'd be nice if they would just maybe try and listen to families a bit more and don't fight against them. Yeah, I think that's probably something that needs to be looked at as well, because you were saying like there’s audiology and then there's no one else we can really talk to.

Joe

No, there's a gap in accessing that information.

Waqas

So now Zoe is two and a half months old now, and she was fitted for her hearing aids, and that's a decision that we took. The four weeks of intervening period between her diagnosis and her fitting and her getting fitted with hearing aids, we did a lot of research, me and Sana both, and we came up to a conclusion that we're going to deploy all the interventions that are possible, whether provided by NHS or whether we need to fund it ourselves, because hearing aids were expensive, right. Thankfully they were funded from the NHS. And the moulds that go in the ear, the babies outgrow them really, really fast. So you need to go and get that done every three months or every two months initially, now is three months. What we didn't know and what we learned through our research and through the conversations, was that a lot of learning happens, what they call incidental learning, because we're not talking to the child, but the child is listening to the surrounding because she might be busy playing with her stuff or whatever her activity right now is, but she's listening to what, she's passively listening to, to her environment. And that's where she takes a lot of her inputs, a lot of her feedback. And that's essential for developing her speech and basically understanding and spoken language. We made a conscious effort to have the hearing aids on all of her waking hours, which was which is difficult for a baby. The first 3 or 4 months were fine, because they haven't really discovered that there's something called, there's something like ears that they have. But as soon as they discover, they are constantly doing like this and then they throw them off.

Hannah Cooper

And were there any challenges that you had in particular with the hearing aids?

Waqas

So we are from a South Asian culture and in South Asian culture, it's like a stigma, any disability, whether it's mental or physical, you try to hide, you don't talk about it. But from a cultural perspective, we made it a point that hearing aids need to be front and centre, right. They need to be as visible as possible. So we have a band in which, you know, the hearing aid actually goes and then it comes so the band has very bright colours and then the mould itself is sparkly. So that it becomes part of her personality and from this stage on she doesn't look at it as a disability. It's in my mind or in our mind is simply hearing aid is nothing but like glasses. Glasses help us to see, hearing aids help to basically hear that’s pretty much it.

Hannah Cooper

Did you challenge professionals or look for a second opinion?

Waqas

So the only challenge that I had to really do was was getting that radio aid. But that was not related to mild hearing loss. It was related to the funding situation at the council. They just simply their policy was that they can only provide it once the child reaches reception. And that would be then for all hearing losses not specific to mild to moderate. So it I wouldn't say it as a challenge, I would say, I would term it as a constructive conversation.

Kim

So initially we closely monitored Ela. She was very young, and she was at the age where her communication seemed to be meeting all the kind of milestones that you expect at that age, especially for communication. But what we recognised was that in her audiology appointments, her hearing was getting worse, only a little, but enough that we had to consider what would be best for her in the future. So we decided in January that we would fit her for a hearing aid. It was a choice that we made quite easily because we felt that her hearing was getting worse and that the pros and cons of having a hearing aid weighed up more positively, particularly as we wanted to make sure that she was able to communicate. The nursery that she goes to is in quite an old building so the acoustics are not very supportive for children with deafness and they have quite a few different age groups in quite a large room. So for her to be able to focus on her primary care workers in the nursery was becoming challenging for her. Only a little but we felt that, why not support her in being able to learn more and we're so fortunate that Ela enjoys learning and she really, she's a great watcher, but we wanted to give her the best chance that we possibly could. I am a primary school teacher and my understanding of how children learn was something that really, really helped me to kind of figure out if we should go down the route of hearing aids or not, because ultimately in a classroom environment as well, it's very busy, there's only one teacher, maybe a support assistant if you're lucky. So for a child with hearing loss, to be in an environment where there's lots of noise it is challenging for them to focus on the individual who they need to listen to, and for a child with deafness, of course, it's going to be more challenging. So we decided that it would be really important for her to have that and especially because she was not yet two, we wanted her to get used to the idea of having the ear mould in her ear and having this, you know, artificial kind of sound going in her ear so that she could get used to it. We're really lucky as well because she seems to have taken to it really well, and she lets us know if she's not happy with it. She's been really good with it and is happy to wear it. The only time she doesn't like wearing it is if she's ill and she'll take it out immediately and we know straight away that that's her way of communicating with us that she's not happy with having it on and, or if we're going to bedtime, she knows she's tired and she'd like the peace and quiet she's very happy to just yank it out!

Hannah Cooper

And did you challenge professionals or look for a second opinion.

Kim

We have been really fortunate. We've had an absolutely amazing audiology team who have been very supportive with giving us lots of different options on what to do. So we haven't fortunately, we haven't had to challenge anyone in that sense. Our teacher of the deaf has been really supportive as well, particularly as she came to visit us at home and the nursery quite soon before Ela got fitted with her first hearing aid. And she gave us kind of, she helped us to weigh up the pros and cons, and we were given lots of different options on what to do in terms of having a hearing aid as well. So we yeah, we have been really fortunate to have such an amazing team helping us.

Hannah Cooper

Is there anything that you wanted to share about the hearing aid?

Kim

I think firstly, we are in a world now where children get given so many options, so it becomes almost a fashion accessory. Ela has chosen it herself. It's pink and sparkly with stars on it, it's just perfect for her. So it's been, the world of hearing aids has been turned into something that is so positive. And I think in some ways, the fact that you can choose lots of different colours and all sorts of things, firstly, the child likes it more, and secondly, I think it does raise a bit more awareness because you can't miss a pink sparkly hearing aid in a little child's ear. And I mean, obviously we're really open to everything. So if someone asks us questions, we're very happy to answer. And obviously, I understand that some people might find it more challenging depending on their understanding of deafness and their opinions of it and so on. But I just think that we've been really fortunate to have the support we have had as well, which has made it a really positive experience having Ela’s hearing aid fitted and with her deafness. I think actually we've had a lot of positive feedback from lots of people whenever they see us with her. I think people are a lot more aware of parents signing to their children as well. We had a great conversation with an old couple on the train, because Ela started singing the ABC song and we were signing it together, and we had a really lovely conversation about deafness and how they had learnt, they’d learnt Makaton when they were younger parents to help in a school with special needs, and so I was saying, oh, we were using BSL and it was, we've had a really lovely, positive experience. Obviously the older generation in our family, they have always been a bit wary from the beginning because they don't particularly understand why Ela's deaf. And they, I mean, to be honest, we don't really fully understand why Ela was born that way. She was diagnosed with CMV, which was the cause of her deafness. And I think for our parents, my husband, Tom, and my parents, they felt like, oh, she was born with a virus so it you must be able to fix it. So teaching them about the fact that it's permanent and really recognising that whilst it's not going to get any better, we can take positive steps to support her and be pro communication, and that's something that our teacher of the deaf and us as a family, we've really had a great relationship about because we are, we always say we're pro communication, it's nothing to do with deafness, it's all to do with communication, and having a hearing aid is something that supports her in her steps to communication.

Amanda

Thanks so much to Abby, Clodagh, Kim, Jo, Louise and Waqas for sharing their experiences and thoughts with us.

Hannah

Next time we'll be talking to the parents about how they make sure their child gets what they need. So do join us for episode three: prioritising your child as an individual from Little ears, big challenges: navigating mild and unilateral deafness, here on UCL Minds.