 Last fall, at the face-to-face with the PLC, we had an opportunity to meet and talk about areas that we would like to find out some information. And one of the areas that was brought up, I believe, primarily from the Calgary contingency was auditory neuropathy. Who are these kids? What is auditory neuropathy? How can we best support them? And it took me a little while to find Beatrice, but I did find her through my connections with people in the Glen Rose, and Beatrice finally agreed to providing us with some information about auditory neuropathy spectrum disorder. And Beatrice, is that the Glen Rose Hospital as a temporary individual? And a lot of her experience has been in Calgary, I believe at the Children's Hospital, and you can help me out with that, Beatrice, in your introduction. So I'm going to hand over the mic to Beatrice, unfortunately not the camera, and we can all learn something today. So thank you, Beatrice. It's for you. Thank you for the opportunity. It's always good to discuss this with everybody. Make sure we're all on the same page. First of all, I'm going to apologize. I'm just getting over a cold. So if I mute suddenly, it's because I'm coughing. So an overview of auditory neuropathy spectrum disorder. So basically, I would like to kind of look at what exactly it is. Just hang on. There we go. So just briefly about me. As you were saying, I'm currently at the Glen Rose rehabilitation hospital as a temporary audiologist, but I spent a lot of my time at the Children's Hospital in Calgary, and we were seeing these children with this particular set of problems come through the clinic. And a lot of times we were struggling as to what to do with these kids and how to follow up with them and what services and what supports do they need. And so while I do not at all claim to be an expert, I have seen quite a number of cases just from my time there. And I'm finding also with my time here in Edmonton at the Glen Rose, because oftentimes we see quite a few adults with this particular problem as well. So just a brief overview. I'm just going to go briefly into each of these areas. So a quick definition of what it is, a little bit about the anatomy involved, how often it occurs, and what we do to assess auditory neuropathy and the sort of risk factors that you would expect to see and the types of breakdowns in transmission of sound that we would expect from it, and what you would see yourself on the other end after we've found this pattern of results. And finally, the sorts of things that we would look to to try and support some of these kids. So a really brief explanation of what this is, a rare form of hearing loss where sound isn't transmitted from the inner ear to the hearing nerve or from the nerve to the brain. So this is probably the simplest explanation. What it is is just a breakdown in where the sound is transmitted or how the sound is transmitted due to numerous numbers of problems. So a quick little, this is the briefest history I could come up with. So sort of in the 80s, where a lot of those electrophysiological measures were being developed in the 90s, they were noticing sort of a mismatch between their test results. So the behavioral audiograms that you would guess versus the electrophysiological measures that we were finding. And so the term auditory neuropathy sort of came about somewhere in the 90s. After that, there was a whole proliferation of a whole bunch of times. And a lot of these terms were trying to reflect where that transmission difficulty had occurred. In general, there are a whole number more than what I have here, more terms than what I have here. But in general, there are a lot of names up in the literature about this until around 2008. This guideline development conference in Italy, it was sort of internationally agreed that the name used for this particular pattern of results would be auditory neuropathy spectrum disorder. So I'm just going to go back and talk a little bit about the ear. So I'm sure many of you already know or have seen, well, should be familiar with the ear. So very briefly sound will travel through the eardrum and the middle ear system to the nerve cells of the inner ear, and that's converted to electrical impulses that travel along the auditory nerve up to the brain. So you're probably already familiar with conductive hearing loss, which would be problems with the eardrum and with or with the middle ear system and central neural loss, which would be difficulties with the inner ear or the cochlea. But for auditory neuropathy, what we're going to have to look more closely at is the inner ear and how it as it connects to the auditory nerve and also how that auditory nerve sends the information up to the brain. So for this next slide, I'm just going to take a cross section of the cochlea there. So this is just sort of a slice into the middle of the inner ear. So sound pressure waves will come into the ear and it moves the fluid in the ear and that in turn moves or stimulates the the hair cells. So you can see labeled sort of in that middle section there where that box is. We have outer hair cells and we have inner hair cells. So the sound will simulate those hair cells and transmit the sound to the nerve, which is that lovely yellow part of the picture spiral gangling on all the way up to up to the brain. So this is going to become important later. There is a reason I'm going through this. So the outer hair cells in general act to kind of amplify the sound. So there's a little or what they call a motor unit at the base of those cells. So basically what happens is when your ear hears a sound, it actually makes that sound wave a little bit bigger because it contracts and it moves that whole section a little bit more. The inner hair cells, as far as I understand it, their function is to encode sort of the clarity or the resolution of the sound. And that is important to kind of send the information up to the brain and kind of give you that clarity of speech that we're looking for. All right. So overall, the technical definition of auto-tringeropathy would be normal measures of that hair cell activity or that inner ear activity. And we measure that with two different things, autoacoustic emissions and cochlear microphonic. I'll go over those in just a moment. And the other half of this is that the auditory brain stem response. So the electrophysiological measure of the nerve is either extremely degraded or absent. So the general idea is part of the inner ear is intact, but that transmission onto the nerve up to the brain is interrupted at some point. And just keep in mind this describes a clinical profile or a pattern of test results. This isn't really a label for the child. It's just a particular issue that was noticed with a subset of children. We do have different tests to look at different parts of that inner ear versus the nerve and we'll go over that in just a moment. But why we should be looking at this. So about one in seven thousand has been noted previously to have abnormal auditory nerve function. I think it's a relatively new diagnosis, but with the move towards universal newborn hearing screening programs, I think this is becoming, I think the awareness of this problem is becoming quite a bit wider because we know it exists and we're identifying it much earlier. So I think it's more kind of an awareness of a particular problem. So of the children who have permanent hearing loss, it's estimated somewhere between seven to 10 percent of those kids have this particular pattern of results in addition to whatever other issues with hearing they might have. So I think that works out on average of children who have hearing loss, about one in 10 of those children will also have this pattern of auditory neuropathy spectrum disorder. So I'm just going to move on a little bit as to how we measure or establish this pattern of results. So the first thing I'm going to go over autoacoustic emissions. So if you recall that photo of that inner ear, that cross section, we had those outer hair cells. So basically, autoacoustic emissions is a measure of the function of those hair cells. So with central hearing loss, autoacoustic emissions are absent because often this doesn't function with straightforward central hearing loss. But with auditory neuropathy spectrum disorder in a fair number of these cases and finding different rates of present autoacoustic emissions, I think it's sitting somewhere between 60 to 75 percent. Oftentimes, these responses are present at the beginning. There is evidence that this appears later on whether or not they use hearing aids, but at the beginning they do have that cochlear response that you can see. So just moving on, I'm just going to move to the measures of the auditory nerve. So auditory brain stem response, basically it's an electrophysiological measure of that nerve system. So anytime you have any sort of nerve response, so be it muscle or any sort of sensory response, it creates an electrical potential and that is something that we can read with our auditory brain stem response. So this is just a general definition of that. So that ABR response, it's generated in response to a sound. So the way we test infants, very small infants, is to play very quiet sounds and force that nerve to fire and measure what level it takes for that nerve to fire. Now for, as I mentioned previously, for auditory neuropathy, what happens is that nerve response is either absent or it's only present at extremely high levels and it doesn't look the way you would typically expect it to look. I'm going to keep in mind with this auditory nerve response, it really just moves in one direction. So regardless of what the sound is that you present to the ear, it should look the same. So for instance, if you have a clicking sound, okay that's getting too complicated. So if you have, basically it moves in one direction, that was my point. So besides measuring auditory nerve function, we can also measure a little bit of inner ear function with the ABR response and that is what we call a cochlear microphonic. So basically with neuropathy, auditory neuropathy, what happens is the normal waves that we would see in the auditory brain stem response are absent. However, we do see something at the very beginning and what that response is, is actually the inner ear. So the nerve cells are, sorry, those hair cells in the inner ear also create an electrical response as well. Now the difference between the hair cells in the ear creating the response versus the nerve, the hair cells will actually reflect changes in the sound. So it basically can change direction. So this is how we show the difference between what we think a neural response would be versus an inner ear response. So just to go through and have a look at what a normal ABR would look like versus what that inner ear cochlear microphonic response would be. So just keep in mind, this is not to scale at all. So the measures for the left side are very different than the right side. The right side is actually blown up in size quite a bit. So on the left side, we have what a normal auditory nerve response would be. So if you notice there's a lot of, so they're, and it's marked, so there's one, three, and five. So there are these peaks in the normal ABR response and those peaks correspond to different structures in the auditory system. So for instance, that would be sort of the auditory nerve and the back of the brainstem up to the top of the brainstem. So essentially that's what those peaks would represent or where those electrical responses are generated from. For the abnormal ABR waves, you notice that there's something that's really, really high in PV at the beginning, but the response sort of peters out and you're not getting this nice wavy response that you would expect. So what that response is at the very beginning, this is not a neural response, this is actually a response from the inner ear, from either the inner or the outer hair cells from that inner ear. And you can see the top two lines, the sound presented there is actually exactly opposite as compared to the sound that's presented on the sort of the middle two lines there. So at the very bottom, they've actually stacked those two together and you can see it creates this big butterfly kind of plot. What that butterfly plot is telling you is that the response is actually from the inner ear. So in response to changing the sound that we're presenting to the ear, those hair cells in the ear actually change in direction as well. So it's giving us sort of an opposite electrical potential to each other. So it's sort of a nice double check for us to see if that part of the ear is functioning versus the neural part of the ear. Now in a normal hearing ear, you still have cochlear microphonics. The thing is the nerve response is so much larger that it actually hides the cochlear microphonic response. The only reason you see cochlear microphonic response when the rest of the ABR is absent is because there are no other neural responses. So when we blow it up, we see this nice kind of opposite butterfly plot effect for when we're measuring that inner ear. So for this next slide, I found this photo of one type of auditory neuropathy. Excuse me. And this is just one type of auditory neuropathy. So it's actually a cross-section of a normal auditory nerve on the left side versus an auditory nerve that is affected on the other side. And you can see the fibers on the right side. There's much fewer as compared to the left. So this is one type I just thought it was a little bit interesting to look at. So looking at a few of the risk factors, I know there looks to be a lot of them. Mostly these fall into three main categories. The first would be some sort of neonatal history. So hyperbili rubenemia or jaundice, high jaundice requiring transfusion, extreme prematurity, so below 28 weeks, low birth weight, oxygen deprivation, mumps, sepsis, meningitis. Any sort of lack of oxygen to the brain is also known to be a risk factor to develop auditory neuropathy. And as you can probably guess, we see quite a number of cases, especially with that extremely premature crowd, just because of that lack of oxygen. So oxygen is always a huge problem when you have extremely premature children, when those lungs aren't developed yet when they come out. And so it just puts them at risk to develop this particular issue. The second main set of causes or risk factors for auditory neuropathy would be particular genetic conditions. So as you can see, they have a few in there. So the OTOF mutation sort of in the middle there. And that's actually specific to a type of transmitter in the inner ear. And then there are other ones that basically look at vestibular and auditory nerves issues. So it is known to be part of those particular genetic syndromes. The other main category that you would see would be if there is something anatomically a little bit different. So for instance, microcephaly is like a really small head or any sort of brain stem or abnormality, that kind of thing. Just keep in mind, this isn't a comprehensive list of risk factors. There are a whole host of other things that do serve as a risk factor for auditory neuropathy. But this is just sort of a nice summary of some of the main ones that we see in general. So I just wanted to use the slide a little bit to show that based on the test that we have, we can occasionally narrow down specifically where the sound isn't being transmitted, but the majority of the time we can't really rely on it because our test results have the tendency to look the same for different areas of where the system isn't transmitting. So we can't really depend on our pattern of results to say exactly where the issue or that breakdown in transmission might be because it would be similar clinical responses for multiple causes. So it just does a summary to this. The main parts that it could break down, so this is just a list. So essentially where the those inner hair cells are, where the auditory nerve, how the auditory nerve connects, and well just essentially just based on the test we have, because we can't tell the difference between the causes of auditory neuropathy spectrum disorder, we can't really predict how a child will perform in a classroom setting or in day-to-day interactions. So this is what we call it a spectrum disorder and why outcomes for hearing might be extremely unpredictable. So in general what we would tend to do for kids, so if we're looking at below six to eight months, we would be doing those otocoustic emissions and we would have those estimated levels from the auditory brainstem results. Now the problem with using ABR in that population, we don't actually know what level that child is actually hearing at. We know it's at least that level but potentially it could be much better than what we're getting from that test. Now for children six to eight months or older, we would also do those otocoustic emissions in ABR but more what we would be relying on would be that behavioral testing in the sound booth because where the child is actually responding would really give us an indication as to whether or not they're able to detect sound in the first place. So just to mention there is some work that's being done right now and it's very preliminary still and you get sort of later brain responses, so higher level brain responses in determining sound differences to try and figure out what levels infants are hearing at. So in that before six to eight months crowd that's the sort of thing we would be looking at. It's not been an Alberta, those tests in particular and I think from what I understand they're still at the beginning stages of using those results to kind of determine hearing levels still so it's still to be to be seen if this is going to be effective to tell where exactly the child is hearing at but still I think we're still relying on that behavioral testing to try and tease apart where exactly they are responding at. So that's the very young kids. Now for older kids I forgot to talk about acoustic reflexes but there are there is a middle ear reflex that relies on function of the inner ear and the auditory nerve and oftentimes those are either elevated or just completely missing in children with this profile. Behavioral testing could be anywhere from normal to profound in any configuration and again that ABR is going to be relatively degraded or absent and the thing to note though the word recognition or the speech discrimination would be much poorer than your audiogram would suggest and the other thing you would see in the school age population would be greater difficulties hearing when there's a lot of background noise or a lot of competing noise and that's that's what we would typically expect. So listening and noise requires very fine tuned kind of timing to to understand speech and to figure out where sounds are coming from and if the nerve system isn't functioning optimally then that kind of creates a bit of a disadvantage for that child and that's where that difficulty kind of comprehending in noise comes from. So that's what school age children there is some preliminary research that shows that for teenage years so sort of mid to late teenage years it looks like some kids with this profile their behavioral thresholds actually get worse and their discrimination or rather their ability to comprehend speech also seems to decrease as they get a little bit older too. The causes of that aren't well known yet but it does seem to be a bit of a trend. So just a bit of a conundrum so we have two audiograms here and everybody here is probably versed with the audiogram so down the side you've got your your decibel to your levels of hearing and across from left to right on that horizontal axis you've got the different frequencies. So just based on the audiogram I guess the question would be would you predict better or worse speech and language for one versus the other? I'm not sure if anybody has a particular opinion about that. From what I've seen previously and from from what I've seen in the literature as well the the audiogram doesn't really tell you a whole lot it tells you where they can detect environmental sounds but it doesn't really tell you about how clear that sound would be or if the signal is reaching the brain in a fairly intact form. So really the question would be what does auditory neuropathy sound like and the answer is we don't really know. I mean the behavioral audiograms may be similar or you might have sensorineural loss that looks very similar but the sound quality might be different very very different for a child with this profile versus sensorineural hearing loss. So really ABR and behavioral thresholds so ABR results and the audiogram are pretty poor predictors of whether or not they're going to understand or they're going to going to be able to obtain enough intact sound to be able to distinguish speech and language. In some cases just as a side note there was some pretty suggestion that those ABR there was some suggestion that those ABR responses would improve but the jury is sort of out on that. It does seem to happen in some cases and personally I had seen it happen myself where the ABR does seem to improve but generally it doesn't improve completely to normal from what I've seen. Whether or not that actually happens it's still to be seen. Behavioral thresholds there is also some question whether or not those behavioral results will also improve and really the jury is out. There's a lot of literature and I mean that that is something that is that is thought to be the case in the UK but I know in North America I think they're moving away a little bit from that because I think the idea was that some of those initial tests ABR tests for neuropathy were perhaps not as clean or maybe a little bit noisier so you can really evaluate some of those responses as you would expect. So just as I guess a little bit of a simulation I did find an interesting sound simulation of auditory neuropathy. So because we don't know what quality of sound the child is getting there might be complete distortion or it might be just like a little bit blurred out. So what this simulation is looking at it actually presents extremely disturbed sound and it just gets comparatively clear. So with any child with that profile of auditory neuropathy we don't know where they are in terms of that clarity spectrum. So this is just sort of nice to illustrate the level of distortion that might occur. So hopefully everybody has their sound on and that doesn't seem to want to work. Okay never mind moving on. If anybody is interested I can send the link to that sound simulation another time. So this is just an analogy to vision because I had a feeling that the sound clip wasn't going to work. So I guess for some children without auditory neuropathy I mean the sound might be a little bit blurred or there might be big huge holes in what they're hearing and really we don't know which end of it they're on or what precisely they're hearing. It might even be even more degraded than what you're seeing on the screen right there. So because we don't know precisely where they are on that clarity scale the idea is that no single approach works for every child with this profile. So some will benefit from technology such as hearing aids or cochlear implants. Some might need a combination of like visual communication and spoken language. If they are using hearing aids or cochlear implants or cochlear implants just that listening technology is going to be very important and I'm going to come back to that in a little bit. So really from what I've seen in the past and from what a lot of the literature states like you might start with one approach and then you have to change just depending on what's happening with the child and whether or not they're hearing enough clarity to be able to develop that speech and language. So as you can probably imagine I'll just for neuropathy this inquire if this requires a complete management team. So this is just an example so pediatric audiologists you would need like the diagnostic the assessments educational audiologists of course prior practice or the sitting or dispensing audiologists pediatric ENT and neurology usually they would need to do some monitoring and to see if there are other other issues involved with the auditory neuropathy. So oftentimes our auditory neuropathy doesn't occur necessarily in isolation it can occur with other types of neuropathies as well so a lot of that though is dependent on what the initial cause or etiology would be. Of course you definitely need a speech language pathologist on board mostly because we need to monitor that speech and language development or rather they need to monitor that speech and language development particularly that speech and language trajectory because we need to know that the child is obtaining enough information with whatever intervention they have to develop that speaking speech and language and if they aren't then that's where we're queued to switch gears and try to try to find another way to support the children. And of course the teacher in the classroom support is very important because oftentimes children with this profile need a little more support. So other professionals that you would probably have on this team would be social work and psychology the psychology in particular because you at some point you would want to look at that nonverbal learning ability of the child particularly if that child hasn't progressed in speech and languages as you would have expected so then you can separate it from language versus there are any sort of cognitive delay as well. In terms of interventions and mode of communication pardon me so a lot of the interventions are really determined by what we see from the child again that progress so the monitoring is really important there and and really what the needs of the family would be. So every every family every case is going to look very different and really we need to take this case by case there isn't a cookie cutter method that is going to work for every child with particular profiles. Now with with interventions I'm sure probably wondering how effective something like hearing aids would be. The current recommendations for and I'm blanking on it's in the States the American Academy of Audiology in the States the current recommendations would be to trial hearing aid technology if that behavioral audiogram is showing that their the levels that they're responding at are insufficient to support normal speech perception. So this is particularly important to give it a go because you know a lot of times like yes amplification outcomes may be mixed but a lot of kids do show quite a bit of progress with hearing aids so it's really that constant monitoring to really determine whether or not they're they're holding their own or whether or not we need to switch gears. You know I mentioned briefly before especially with how the nerve transmits up to the brain those timing cues and the I guess the clarity of sound is not the same as what you would expect. So I'm going to come back to come to this in the next couple of slides. We do know that children don't with auditory neuropathy do not do as well in noise and so I just wanted to briefly mention that there are different types of technology does like hearing aid technology out there to manage that background noise more effectively. So you do have just those different levels of technology so you have your entry level you're mid and you're high and I'm not saying you need to go high level to get those noise management features but oftentimes a lot of those sound management or noise management strategies aren't really available in the basic entry level technology so basically what I'm saying is for kids who are undergoing for neuropathy kids who are undergoing hearing aid trial they really need to have a good conversation with whoever's defensing the hearing aids just to see what would be appropriate in terms of trying to manage that background noise problem and and seeing what their options would be for that excuse me. So I did find a nice little kind of summary study of comparing children who have that profile of auditory neuropathy versus center neural hearing loss and so this particular study was looking at children who identified via universal newborn hearing screening programs in the states and this is across multiple centers and they matched the kids based on hearing levels so audibility, age of identification, gender, a degree of hearing as I was saying degree of hearing levels so the nice thing about this study is when they looked at these kids they followed these kids out for quite some time I think it was three to five years and as they were measuring different parts of language and teaching language they were finding that there really wasn't any significant difference between the children with that auditory neuropathy spectrum disorder versus the center neural hearing loss so all these kids had hearing aids at about the same age they were identified at about the same age and they have about the same levels of audibility so as you can just glance down that column of auditory neuropathy or the ANSD so that compared to the next column over those differences are not significant the thing to note though at the very bottom so what that third section is that last data point they didn't have enough numbers to really compare those two those were just based on I think about six children in total between those two groups but it is an interesting trend so what that last line is showing is that when you try to do word recognition with a little bit of background noise and look the trend was that the auditory neuropathy spectrum disorder that the kids with that profile do much worse when you put a little bit of noise in there compared to the children who have a center neural hearing loss so the next slide I'm going to show actually goes into that a little bit further I don't know if that's coming across very clearly so basically these are it's sort of a speech sound perception test and so they compared so down the left hand side you can see they compared children who have normal hearing versus children who have center neural hearing loss and children who have auditory neuropathy and each column so you have the SN ratio and the SN that's called the signal to noise ratio so the plus 20 dB most pretty much everybody was normal hearing you can see with the normal hearing children in the study they were getting 96 or high 90s in the particular test so the noise level at plus 20 basically that means you have 20 decibels over and above how much noise there is so the performance of children with normal hearing at that level is really really good if you children with center neural hearing loss do quite a bit poorer than children with normal hearing but then when you look at the auditory neuropathy grouping it drops down to by half and so you can see the effect of noise where for normal hearing subjects it really doesn't impact them at all for children with auditory neuropathy it really drops their ability to comprehend speech and language and it is significantly different especially in that first column the difference is as you as you go down so you have a plus 20 decibels and then you have the plus 10 which means that there's a little bit more noise as compared to the signal and see there's still like the normal hearing children are still doing quite well but the center neural hearing loss drops even further but the neuropathy group is starting to really bottom out there so how noise affects this particular population is pretty dramatic so what that means in terms of what we do in the classroom would be to try and give them as good a speech signal as we possibly can and that's where FM systems or direct microphone systems would come into play so to kind of overcome that huge drop in noise that children with that profile have FM systems are really really important and it really gives a child with this profile who is probably dealing with an already really degraded signal at the best of time it sort of gives them the best chance of catching what you're saying to kind of give them the best support and the best chance of succeeding and I'm not sure if my sound clips are going to work but if they do it would be nice and no they're not going to work if anybody would like to hear these clips I can send them to you but basically it was comparing what it sounds like with a hearing aid only in a noisy classroom as compared to what it sounds like with a hearing aid combined with a directional FM system or a direct microphone system and it really is very dramatic so and that's just with your typical hearing aid use this isn't even adding the distortion that you would have with a child with that auditory neuropathy spectrum disorder so those are the kids with hearing aids so those are mostly mild moderate kids the children who behaviorally respond more in the severe to profound range we would be monitoring them much more closely for something such as cochlear implantation and and this occurs in a handful of cases not the majority of auditory neuropathy cases so in cases where the hearing aids and FM systems aren't enough to provide the child with enough information to develop that speech and language and I mean if the parents really want that audibility in the hearing then we would move to possibly considering cochlear implant and so again that close monitoring of that speech perception and the audibility and the development is also very very important so some of the just as a side note some of the studies of children with auditory neuropathy who have had cochlear implantation just as compared to sensor neural hearing loss in the severe to profound range who also have cochlear implantation when they compare across those two populations auditory neuropathy and sensor neural loss they do end up looking very similar in terms of their language outcomes as well which is sort of nice why that is it's it's sort of just to say that some of the theories which suggests that a cochlear implant provides more more stimulation of the nerve and so it creates a stronger response to travel up to the brain which gives the child more information at that level pardon me in general you're wondering how best to help students so of course the educational ideology to make sure all of the equipment is working at optimal levels checking the equipment regularly and encouraging that regular follow-up and equipment maintenance and again just wearing that personal microphone system or FM system that is the best thing you can do with this particular population in terms of that that follow-up I mean with younger children we would want to follow them quite closely so probably at three to six month intervals school age children I mean at minimum they should probably be seen about once a year if not more even more if there are changes in hearing or changes in how the children are performing or hearing of course I mean those are just minimum sort of follow-ups and I mean private practice you would expect to see them at least twice a year regardless of your age just for regular equipment maintenance so just to summarize really quickly auditory neuropathy this is a label or for a pattern of test results for the child or sorry this is a label for a pattern of test results and not a label necessarily for the child and so that absent ABR at the very beginning it doesn't really imply profound hearing loss necessarily we really just have to closely monitor the child because they might not respond in a predictable kind of way many children with neuropathy or with this profile are able to make good use of their hearing and generally can't predict how so early on when we're still trying to figure out those behavioral thresholds we can't really predict at a later stage how they're going to be how they're going to how their outcomes will be so we really need to do as much as possible to monitor and support the kids at early stages and the other component of this is just establishing that early communication and language and if they're deviating off of that trajectory that's that's that's our cue to really step in and try to figure out what are the supports they might need in place so that is about all I had planned does anybody have any questions thank you it's Sarah I'm gonna I'm gonna actually ask the question because it's something I'm interested in and I'm wondering in your preparation of this um interesting topic if you came across whether any auditory training for these kids with this diagnosis um helped support them in utilizing whatever technology they were um you know in test results benefiting from that's a really good question and no I didn't see anything in particular with this population but I do know that there is an effect of training in children with sensory neural hering loss and a lot of that is top-down processing so I mean you would sort of expect that any sort of auditory training would kind of help them in the long run but that isn't something that I've seen at all in literature if anybody else has feel free to jump in so does anybody else have any other questions I'm I'm trying to watch the participants that I'm not seeing any hands up or any chat you can do either one hi Beatrice hi yeah oh hi my name is Amy um I have a student uh he's in grade three he has a mild to moderate sensory neural hearing loss and plus auditory neuropathy um and I know the school is working really really hard to support him um he's having he's having a lot of difficulties even with number recognition you know if if you say like can't one to ten and then count backwards it's hard for him to get the numbers in order from one to ten even like and he's at grade three um through school or struggling um he has occupational therapy and speech language therapy services as well and we're just trying to work together as a team to figure out you know is there's something else happening here would would the auditory neuropathy be contributing to some of these issues do you feel or I know it's hard to say if you don't have the bigger picture but um because there any research kind of that well report that I think this is where that multi-disciplinary team really comes into play um you kind of um you would in general from what I've seen in the past you would need to look at their non-verbal kind of cognitive ability to do that kind of thing and then you would have to look at the speech perception part of it as well and the like yeah and I know the speech perception thresholds uh it's excellent in the right ear and good in the left I think 98% and 80% based on his last hearing test so would that be words in a quiet or um let me just see I actually have a support here I believe yes in quiet right right yeah um and okay so if speech perception in quiet is that good um yeah and I'm assuming he has the language to comprehend yeah you know like speech-wise but when I mean his reading levels are quite low as well right but right um can I ask Beatrice sorry Sarah would you request a speech recognition and noise from the audiologist to get a better handle on is that would that provide some additional information for Amy um that might give you a little bit of an idea of how they're performing in this classroom I would think um I guess my question is if his non-verbal cognitive abilities um are at that level or if it's the hearing specifically it's sort of hard to piece this apart without all the pieces of the information yeah yeah and I know uh the occupational therapist did some uh visual kind of spatial testing with him and he did quite low on that okay I remember the name of the test I might have that down but yeah I know he's struggling in those areas right right um in all honesty it is really hard to say I mean uh having that autothermopathy profile probably isn't helping matters but whether that's the sole reason for the difficulties he's experiencing it it it's really difficult to piece that apart at this stage I mean he probably needs a little bit more more evaluation to try and figure out each part of this a little bit more for him okay that's kind of what we thought but I I figured oh I'd ask just to see maybe there was a magical answer but we we thought that maybe further testing or maybe even a ed psych evaluation or something along those lines to see if there's another piece that we're maybe missing yeah okay thank you so much Beatrice I appreciate it not at all I'm I keep thinking back to somebody who said actually I think it was Karen Anderson and said um if you've met one child with hearing loss um you've met one child with hearing loss I think we can also add if you've met one child with hearing loss or auditory neuropathy spectrum disorder you've met one child with auditory ASND or ANSD um and so I uh I really appreciate you giving us this foundational information it allows it contributes to our practice out in the field and you know we'll continue to have conversations about kids and individual children in order to best meet their needs and thank you again for agreeing even and I think it's the second person that's presented with a really bad cold in the last few months so um I'm not going to talk to anybody anymore because apparently I have something I'm spreading so have a have a great rest of the day everybody stay dry and see you next month everybody thank you