REALTIME FILE

18th Annual Early Hearing 
Detection & Intervention Meeting
Medical Considerations in the Management 
of Pediatric Hearing Loss
Heathrow AB 
March 3, 2019, 1:00 P.M.  

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>> Good afternoon, everyone.  We're going to go ahead and get started.  This is medical considerations in the management of pediatric hearing loss from 1:00 to 3:30.  On the monitor if you guys have any questions let me know.  There's in the back and paper and pens are in the back.  
>> Okay.  Good afternoon, everyone.  I hope lunch wasn't too tiring here.  Well, thanks for having us.  Thank you for signing up for this.  I think we have 47 people who signed up for this, so obviously some folks will continue to trickle in here.  I don't think ‑‑ I haven't counted but it doesn't look like we're complete yet.  Also we have sign language interpreters.  Thank you very much for providing this service.  Obviously you have to ‑‑ the captions as well.  So thank you guys.  We really appreciate it.  
    Today we would like to provide an overview over the medical considerations in the management, also the evaluation, but evaluation in management of pediatric hearing loss.  My name is Oliver Adunka I'm a neurologist in Columbus Ohio.  Dr. Craig Buchman here is the chairman at Wash U in St. Louis, Missouri and we both arguably have some experience with pediatric hearing loss.  And cochlear implants, that kind of stuff.  So again, thank you for allowing us to speak here.  So again, here on introductions, that's myself here are our disclosures for both myself and Dr. Buchman.  The outline for today and suggested schedule is we have sort of the first hour as a presentation by both Dr. Buchman and I and we're going to switch around certain topics and we talk about the potential burden of hearing loss, educational aspects and newborn hearing screening data and it's not really a medical topic, per se.  But we felt it was a very important topic as a basis for really what we do.  Because this is how we identify most of the children hopefully.  We would like to then talk about the medical and surgical work‑up really in management and we then move to case presentations in the second hour.  Hopefully we have some time for questions.  And specifically we recommend we have some breaks for Dr. Buchman and I will switch and if you guys have questions at that point that may be a good time to ask them otherwise it gets hard to keep track of your thoughts when a different topic has just been discussed.  So this is at outline.  
    So the first switch is really Dr. Buchman will start here on the overview 
>> All right, thanks, Oliver.  This isn't a great break to ask questions so we'll hold off for the next one.  JCIH as well so it's sort of a good opportunity for us to give you some update in terms of what's going on there as well.  So from my perspective is I always look back on this, otolaryngologist I don't think I ever saw a newborn that failed a screen.  During my residency that wasn't even part of it.  So much of this has come about from learning on the fly, if you want to know the truth.  It has president been where someone handed us a document or textbook and said here's how it's done.  You'll see a lot of this came together really by working with a large multidisciplinary deem that we assembled over time and it originally started for me at the university of Miami actually I did some at my fellowship house group and some in Miami and the lion's share came from the University of North Carolina at Chapel Hill.  Many of you already know these data, the instance estimate for hearing loss is very common, roughly it's probably on the order of about two per thousand, honestly.  Three to four depends on the state that you pick off, but at least two per thousand.  An roughly a quarter have a severe to profound hearing loss.  And so the estimates, at least from some of the older data, is roughly 4 to 5,000 children in the U.S. in that range and then it doubles.  This is one of the key take‑homes is that it doubles by school age.  So that remember that the newborn infant hearing process roughly picks up only half of those kids and it doubles as time goes on.  Just to keep this in perspective, hearing loss, when you compare it to some of the common ones that we think about in terms of disabilities that are out there, we all recognize cleft lip and pallet, spina bifida, we see those and we recognize them, hearing loss doubles those more than double in terms of its frequency.  And in fact even in the JCIH yesterday people were saying hearing loss is such a rare disability.  In fact it's the most common of the disabilities.  It's just that you can't see it.  Because you can't see it it's very difficult for people to recognize its impact.  When people take you down that pathway, don't let them take you there.  We all know, for those of you in the room, the impact of hearing loss is really drastic.  It is drastic, and I think it's important to convey that to families as you're talking to them.  We all know that hearing loss sound awareness, most importantly for children it impacts speech and language educational performance.  And so ultimately we know those things have major consequences for going on in life, whether it be employment, whether it be income, whether it be other things and especially socialization and all the other things that are so important in our lives.  It's roughly $1 million per child over a lifetime to have a profound hearing loss.  And a lot of that of course is because of lost wages.  So and that's really old data so I bet it's a lot more now because that data hasn't been repeated in some time over a decade but it's still an important piece of work.  I did leave out one thing.  And this is a really important one.  Is in the United States today, still well less than 50% of the children who are cochlear implant candidates were actually receiving cochlear implants.  Less than 50%.  And it's not primarily just because of insurance.  Everyone wants to think this is an insurance problem, it's not.  There are other things out there that are holding that back.  Judy Lu from Wash U looked at impact of hearing loss on quality of life.  She has this so‑called hear QL subscale or scale that looks like at hearing loss related quality of life in children.  It looks across a variety of different sub areas, social interaction, of course hearing, school, feelings and total.  And then the three colors blue is normal hearing, children with unilateral and children with bilateral hearing loss.  So you can see that pretty much across each of those sub areas there's really substantial impact in terms of having hearing loss on quality of life in children.  Important to know.  That's not infants.  You can't look at quality of life in infants, so these are a little older kids.  
    So what's the answer?  I think I'm preaching to the choir which I say the answer of course is early identification, making a correct diagnosis.  Diagnosis is audiologically important, but it's also medically important and we'll get to that at some point.  So understanding the type, severity and of course the medical conditions that are wrapped up with this.  And then we'll talk a lot about family‑centered intervention which I think we all agree is critically important.  And part of that intervention of course is providing a good signal from our perspective.  It doesn't necessarily ‑‑ you know, there are many ways to provide a good signal, right?  Sign language implants amplification, there are all options out there.  We believe that all families deserve to hear about all options and making informed decisions about that.  There's an appropriate intervention window so we know in terms of auditory plasticities or brain plasticity there's sort of a key window there.  And of course education, education, education.  
    So newborn screening again as you all know, at least talked about in 1993 by Everett Koop and he jumped in early on as a proponent for that and it hadn't been caught  until the late '90s and early 2000s across the United States and really all states are going at it now but it was something that took a while to come to fruition.  The JCIH has proposed, and I think many states are advocating for this now, the so‑called 1, 3, 6 approach to early intervention.  The sense is that the final screening should be carried out by one month of age and then a diagnosis of permanent hearing loss, which requires of course diagnostic testing by audiologists by three months of age and intervention started by six months of age.  The data that's out there regarding this is all over the place.  So it's really interesting.  But I think that's certainly an aspirational goal.  I'm going to show you what the newborn infant hearing screening process actually looks like schematically because it seems so simple when you think about it, but it's really complicated.  So babies are born.  We have a pretty good idea of that.  And it's roughly 4 million births per year and 98% are screened.  Of those that pass their initial screen, they're passed.  They might ultimately go on to develop hearing loss but they're passed.  If they don't pass, which roughly represents somewhere between 4 and 40 per thousand, or roughly 18 per thousand rate of births don't pass, they can either go into 3 buckets.  One of the buckets is ‑‑ sorry.  It looks like we're missing an arrow here.  Sorry.  Here we go.  One of the buckets is that they've truly failed and they need to go on to audiologic testing so that's confirmed.  The second one is that they're lost to follow‑up, which is something we've all done a lot of focusing on in the last five to seven years.  The other is sort of cleared.  A cleared is a sort of a new term that you might not have heard of.  Cleared means they might have been screened and then they say I'm out.  And so the parents just refused to move on in the process.  And then basically they're not part of the loss to follow‑up that we know that they decided to opt out of the system and it's not an insignificant can't number, it's roughly 7 per thousand or 1.8 per thousand are sort of cleared out of the system.  Loss to follow‑up rates roughly 5 per thousand.  And then true failure rates, you see what that is.  1.7 per thousand.  Ultimately, these ‑‑ excuse me 1.7 per thousand go on to be identified with hearing loss, 9 per thousand are identified with normal hearing.  Okay?  So this is a pretty complicated algorithm when you really think about what's going on here.  The other thing to recall, these are professionals in the room, right?  9 per thousand end up of the failures, 9 per thousand go on to be identified with normal hearing.  1.7 per thousand that failed ultimately are identified with hearing loss.  That's a little secret that people don't like to talk about.  So we screen lots and lots of children.  The vast majority of the failures, the vast majority go on to be identified as normal hearing, but 1.7 per thousand are true positives.  
    So that's interesting information, I think.  But I think looking at each of the states is very instructional to see how the states are actually performing.  And I'm just going to spend a few minutes here because I think it's interesting.  Jim Cesir who is a colleague of mine, Ph.D., who is just an interested individual who works in the EHDI program in Missouri has been so kind to plot out these data in a different way.  And I think they're very instructional.  Over here is the U.S. as a whole.  So if you look at the U.S. as a whole roughly, if you take this whole bucket all the colors in the bottom, that's the rate of no pass.  And then in the bottom you see those that have normal hearing.  You see the blue which is those that end up with hearing loss.  These are the number of kids that are cleared.  And then these are the loss to follow‑up rate.  And this is plotted across all the years.  And so you get a sense of roughly whatever it is.  18 per thousand fail and then roughly what is it, 1.7 per thousand end up with a hearing loss.  So that's the numbers that I just showed you.  But it's easier to look at it in a graphic format.  So if you look at the state of Missouri, which I'm from, overall probably looks really similar, right?  The normal hearing rate roughly is about the same.  The diagnostic hearing loss rate, so you say well, that's pretty good.  The efficiency is literally the number of children that have hearing loss over those that don't pass.  So one in 10 kids that don't pass ultimately end up with hearing loss.  75,000 births.  That's what Missouri does.  And Missouri is happy they're just like the rest of the United States.  So that's interesting.  Here's Kansas, which is the state next to us.  Some weird stuff has happened in Kansas.  So it looks like it started off with a high loss to follow‑up rate, so they were failing a lot of kids and many of them were lost to follow‑up.  What they probably did in Kansas is spend a much of money on the diagnostic side so it looks to me if I had to guess, if they took a lot of those kids and after they failed their newborn screen, they got a diagnostic study that proved that they didn't have hearing loss and ultimately they changed like this.  This could be a reporting issue too, so don't get me wrong.  But it's interesting Kansas roughly 7% efficiency.  It's a little bit different than what you see in the U.S. trend.  Overall the blue number is about the same.  So number of hearing losses.  So you see, Kansas is doing a lot of work and getting a lot of kids that end up being diagnosed with no hearing loss.  So they're probably spending a ton of money in the ABR space to identify this many kids.  
    So let's look at North Carolina.  Congratulations, Jack.  Here's North Carolina.  120,000 births, so one and a half times what you see in Missouri.  You look at the no‑pass rate, drastically lower than the rest of the United States, drastically lower than Missouri, drastically lower than Kansas.  The lost to follow‑up rate is a lot lower than the national average.  You actually look at the hearing loss rate even higher.  So the interesting thing is that more no‑passes does not find more hearing loss.  In fact, more no passes probably clogs the system.  So saying to yourself wow, what we should do is just be conservative and just do diagnostic ABRs on everybody that don't pass a single, that's probably clogging up your system and causing problems, remember look at Kansas as a good example of that.  Okay?  So North Carolina does a great job.  In essence, one in four kids that fail the newborn infant screen roughly in North Carolina end up being diagnosed with hearing loss.  One in four.  And roughly a little bit less get cleared out, a little bit less are lost to follow‑up and a small amount end up with normal hearing.  So a highly efficient group.  Why is this different?  My guess is that they screen, they rescreen and they rescreen.  Roughly three strikes and you're out.  Roughly speaking.  Although it's not the same everywhere.  And if you failed three screens, it's time to get a diagnostic ABR.  But one screen doesn't get you a diagnostic ABR.  And the birthing hospitals have taken some pride in working on that as well.  Let's compare it to Ohio.  Oliver hang your head.  Here's Ohio on the left.  Had to change the scale to get Ohio.  Ohio is the same size as North Carolina.  Ohio is interesting in that they're failing roughly 30 per thousand kids.  A large number of those are ending up in the normal hearing group and in lost to follow‑up.  But look at their hearing loss rate is still pretty darn low.  They probably have a clogged up system.  They have a high number of births, a fair number of lost to follow‑ups and my sense from talking to Oliver is what they're doing they do one screen if you fail the screen they're given information and the family is told to go deal with it.  So you could argue that if they take a 2 or a 3, sort of a 3 strikes and you're out type of approach, they probably would improve this system drastically.  Ohio is really having some trouble.  They really could use some help.  I think what's interesting is we get a chance to compare.  It's really important that we compare states because the legislation nationally looks the same.  But the way these are implemented at the state level is on an individual by individual program basis and that's a huge opportunity for all of us in the room to get together and start to think about how it is that we should work on this.  So higher no‑pass rates generally, this is the take‑home.  Higher no pass rates don't identify more hearing loss.  It increases utilization and costs and there are a number of potential issues for it.  If I had to guess it's not technology or OAE or automated ABR.  My guess is probably implementation protocols.  We've never talked a lot about that in the JCIH.  We haven't talked a lot about implementation and protocols.  We've talked more about technology.  Is OAEs or automated ABR and those things are more appropriate.  My guess is how the technology is being used is really critically important.  And then we should learn from the good states.  These are who they are.  California, North Carolina, Wisconsin, Utah, Nebraska.  They're really doing a great job.  Just to give you some idea, here's California on the left.  Look at their no‑pass rate.  7 per thousand.  Okay?  Look at the number of hearing losses they identify.  So their efficiency is 36%.  That means one of every three kids that ultimately ends up with a no‑pass ends up being identified with a hearing loss.  485,000 births.  What are they doing?  They're spending a lot of money on this.  They're spending a lot of money on the front end of the process.  So screen, rescreen, rescreen.  The thing with California is they're not making the 1‑3‑6.  They don't do a good job on 1‑3‑6 but the kids do fail ultimately end up with hearing loss.  So it's good food for thought.  So beyond the newborn screen there are a lot of options working on lost to follow‑up but I think our biggest issue is what I pointed out than lost to follow‑up.  I think getting the screen right is critical.  Take one question and then we're going to switch.  Unless there's not.  
    [Off microphone] 
>> I don't know that we have the information to know.  All of our states have been working on lost to follow‑up, but everyone thinks about it in a different way.  Everyone thinks there might be something special to their particular honestly I don't think there I as one thing that triggers the issue.  More importantly I think if we were identify the ones that were at the highest risk for hearing loss if you fail the screen, the rescreen and maybe another rescreen, especially if we could do it locally in the community where the baby is born, then I think the chances of having this lost to follow‑up issue will reduce a lot.  The other thing is that the lost to follow‑up group has very little hearing loss in it.  We can tell that.  So many of the states have spent lots and lots of effort working on lost to follow up.  I'm not telling you not to, I'm just saying that it's very deconcentrated in terms of the number of kids that have hearing loss in that group.  
    Okay 
>> So let's move on to the next topic.  And really, talking about the medical and surgical work‑up of hearing loss, permanent hearing loss.  As you all know and the reason we're all here today is because we all know it takes a village to care for children with hearing loss.  We have the obviously a patient‑centered approach with a child and their family in the center here.  We have the medical‑surgical arm here which of course Craig and I represent.  But it's just one of the bullets and these are obviously not scaled to size here.  I would argue we're sort of a smaller portion of all of this in terms of the amount of time spent with the family.  But we all think it's really important and of course we do consult with other medical specialties, depending on the needs.  So let's talk about this here.  And this is some of the classic timeline that we all talk about, which happens, depending on the state and the center and where you are, that happens probably less frequent than we all like to.  So this is the child that comes in, fails, refers to screening, has a diagnostic ABR at one to two months and gets enrolled in early intervention at three months, gets hearing aid fitted at the same time.  This is sort of the classic one.  But what we can see here, in our model that discussing here, we in the medical model, at 2‑4 months of age, once that hearing loss is confirmed with a diagnostic ABR for the most part, we have a medical evaluation including a history, a physical with certain tests.  We're going to talk about those soon.  And enroll the child in early intervention.  And initiate a hearing aid trial, depending on the level of hearing loss.  If it's unilateral or bilateral, et cetera.  And then at 6‑9 months we can confirm the hearing loss via behavioral tests.  We would like to have two separate tests telling us the same story.  If the ABR tells us one story and the VRA testing is not consistent there's more work to be done.  Six to nine months like to confirm everything.  Consider a implant evaluation obviously depending on the levels of hearing loss and the child's progress and early speech and language signs obviously.  And then I put it here as 10 to 14 months as you all know cochlear implantation is FDA approved in 12 months and over but obviously we are trying to push the envelope on this.  We're sort of a little hesitant about the reports of cochlear implantation at six to nine months because we don't have good behavioral data available at the time.  A conditioned response often cannot be obtained at that time.  So this is again the ideal timeline.  So the role of the pediatrician in all this, not just per se is to know the incidence and consequences of hearing loss because they are going to be the medical home of that family.  So we're sort of as otolaryngologists, we get consulted in the whole process, but we feel the pediatrician needs to take the pivotal role in this.  They need to confirm the screening status, they need to know about the technology, the number of screens.  We feel like the pediatrician community needs to know at least something about this, about that.  And also needs to know about the risk profile.  So babies who spend time in the NICU obviously have a much greater risk for developing hearing loss then a well child baby.  And counsel the needs for follow‑up.  This is where the system breaks down many times.  Like Craig just said, it's an invisible disability.  So we feel like the counseling by the pediatrician instead of to falsely reassure the family are a huge problem across the country, really.  And also know the presentation of delayed loss, that goes on to the additional incidence of children presenting with hearing loss at school age and really also refer early if there's presence of hearing loss in otitis media on top and even just otitis media alone, send to an ENT, basically have this evaluated properly.  Those are all things that can delay a child from obtaining speech and language.  And there are vaccinations which we feel like the pediatrics community should be knowledgeable about.  
    And if we're talking about, Craig talked about this three strikes and you're out.  The initial screening at the hospital and some states allow the pediatricians to rescreen, obviously the pediatricians need to be familiar with that technology, not just have the nurse do it and some little closet room and then nothing comes out of that.  So not just buy the equipment, but also know about the equipment.  It should be auto mated because that makes the process much more easy and reliable for people who are not electrophysiology experts.  So know the equipment well.  It should be maintained, annual calibrations and so forth, right.  Screen every field must be maintained, at least in Ohio that we have been facing not all screeners are actually competent and of course NICU children deserve special attention, really.  So we really like pediatrician audiologists specifically to be involved here.  And there's a state database that needs to be just reported to but also the state needs to maintain that database and as we just saw from the data that Craig showed, a lot of these ‑‑ I mean, 49 or 50 states reported this data and it's publicly available.  
    So pediatrician audiology issues, at times it's hard to test kids.  We all know that, I'm sure you echo that here in this room.  It's just much harder than adults.  Babies obviously can tell you hey, I'm hearing the tone, right?  So how sure are we really about the degree of hearing loss.  And I think we as physicians play a role in that in having a discussion with our audiologists, speech pathologists, the whole team really about is this something you feel comfortable fitting a hearing aid on based on the data you obtained.  So are your results sufficient, are they really accurate, are they reliable.  Is the amplification adequate?  Do they perk up if the family comes in fit with hearing aids.  Obviously infants don't like a foreign body in their ear when they're a little older they understand the benefit of properly fit amplification.  If it's not the case it may be just fine, but we would like to see ultimately that the child tolerates hearing aids.  So is it amplification adequate really for us because if it's not it's either an earplug or it's a noise‑induced hearing loss producer.  So we obviously want to avoid that.  And you have one big topic ‑‑ co‑morbidities I should say, really filtering out the non‑syndromic populations, there are no other behaviors versus the one kid that deserves more attention than the non‑syndromic one.  A comprehensive evaluation rather than just relying on one result is critical.  We like the big team, team meetings that seems absolutely paramount in this evaluation.  We all know right, we use those five waves as a reminder.  Wave 5 is really a potential from the inferior ‑‑ from the mid brain, it's very consistent and really using that wave 5 to really determine what's going on in the auditory system with this child.  That's what we're doing.  And the threshold estimation, it's always fascinating when we tell our residents about and we teach them about an ABR, they're fascinated how cumbersome it is.  Both ears, frequencies, bone conduction and you have to run those threshold series until wave 5 goes away.  We ask for what I call the spaghetti, the actual waves that are in the report to see when wave 5 goes away but it's at often times a very dynamic things that you have to be on that machine, whether it's biologics or sonic at the time of the ABR.  It's really an art form in understanding ‑‑ if it's done right and pediatric audiology is doing a great job, it's a reliable tool.  But it can be misinterpreted as we all know.  So that's one of the biggest hurdles I think.  In what I call an actionable ABR, when I get a child and they had a diagnostic study done, there are certain things I look at as a physician that I like to see before signing off on that hearing aid.  I like threshold estimates, four for each ear, there are arguments that at least you have to define for them into 2000 on each side you have the slow up right so I usually recommend for audiology not just start with one ear and then the baby wakes up and you've diagnosed one ear.  But 5 on the left right, 2,000 left right and fill in the blanks basically.  So having this kind of efficient protocol makes a lot of sense.  Bone conduction.  A click can be added in my opinion, in electrophysiology is sort of a hobby of mine.  If you're probably asking why is this guy talking about electrophysiology.  It's sort of something I learned from Craig and Jack's wife here.  I just really think that amplification should not be done before you actually have a good data set to operate on.  Because again, otherwise it's an earplug and those are potentially some of the kids that pull out their hearing aids all the time.  Again, there are some other options, but this is one of the reasons that this could actually happen.  So again, amplification really unless not done unless you have a good timeline.  And the timeline really, so a lot of us get, from outside diagnostic places, that you know referred here in screening and had like three natural sleep ABRs, still not a complete data set, there's some diagnostic uncertainty, maybe the kid was a preemie and the family gets frustrated.  A lot of times they come we went to this audiologist and we did everything they told us to do and we still don't know how my child hears.  For a family it's extraordinarily frustrating and at some point you need to know how the child hears and preferably not when VRA is possible.  Diagnosing a problem is always better just to not.  So when really, if there are two attempts, the child is greater than three months of age, just do a sedative study.  Especially if you have other evidence of there is a hearing loss, let's just get it diagnosed because we need to enroll the children in early intervention and we need to put a hearing aid on this child so you need to have the data.  We all know early is better.  I guess that's why we're all here.  And the JCIH guidelines is 1‑3‑6.  Of course we actually just moved to an even earlier recommendation at some point probably, but as it stands it's 1‑3‑6.  For us it's really the hearing aid is on the patient, early intervention enrollment, et cetera.  From a medical audio logical perspective we need the hearing on.  Hearing aids are an exact science.  We need to avoid the earplug dearly.  Ear models changing every two months, just the size and having the family come back, it's a lot for family to come back to have hearing aids on an infant frankly.  Having good data to fit on, adequate amplification, and regular checks.  But really from when you diagnose at one to two months, to the VRA testing at eight months, there's little you can do because observation audiometry hasn't been shown to be reliable enough.  And this is a study Craig and I did with one of our residents on the paper.  So we looked at 1,143 ABRs.  Those were children diagnosed between 2006 and 2011 and 105 had no response ABRs.  So about 9%.  We looked at several things like demographics, etiology, the imaging data, et cetera, behavioral testing and their ultimate therapy.  If you look at these 105 kids, you can see that each bubble basically represents the final behavioral pure tone testing and you can see these kids have 71 had no response to 50 the herz and they ended up between 90 and 100‑decibel thresholds.  Really you can do that, the ABRs are a little less reliable for low frequencies, very reliable for high frequencies.  But if you look at what these children ended up is really 91 out of 94 he who had a no response ABR at 2 months of age, 91 out of 94 ended up with cochlear implants.  And the three that didn't get cochlear implants just didn't get them at the time when we concluded the study because of other issues.  Medical as well as multiply involved children.  So the meantime from diagnosis to CTI was 7, 8 months and there were delays in receiving the CI was obviously otitis media with effusion, fluids, PE tubes, which is also a common reason our interventions are delayed and it affects amplification as well.  Lost to follow‑up, scheduling issue, parental choice, et cetera.  So really we feel like no child should be denied a cochlear implant as long as there's family behind it, based on the hearing success if you have a no response ABR.  It just basically means you don't have enough in your cochlea.  And we need to provide more ‑‑ of course, however, there are some subpopulations with the no response ABR is a little more tricky.  This is a child, for example, a five‑year‑old boy, a very successful BAHA user in fact.  The ABR has no response as we can see here on the left side of the slide here.  And the CT scan showed what we call an X link ‑‑ it calls a pseudo‑conductive hearing loss because of what you see on the CT scan this child, and we're going to talk about imaging here in a minute.  This is the inner ear, this is the internal auditory canal you can see the cochlea, they're really not separated.  So what those children is is their cerebral spinal fluid pressure is transmitted into the cochlea and causes this inability for the inner ear to basically process the mechanical energy which is again here reflected in this mixed pseudo‑‑conductive component.  But again, no response ABR.  We need to be cognizant.  Of course this bone line would have been different.  But we need to be cognizant of other causes of a no response ABR.  Not an uncommon reason for having no response ABR is to have no wave 5 I should say is auditory neuropathy.  Which further complicates.  So no wave 5, no threshold estimation, no hearing aid.  Which is really complicated to have up to almost 10%, especially NICU population that really can't get hearing aids until we have good behavioral data available.  Which even the behavioral data in that population can be delayed in terms of being obtained.  So really you can see here, nicely on the left side, you can see condensation in the ABR so single polarity stimuli and you can see how that mirrors each other for the right and left ear respectively and no wave 5.  It what would be called a cochlea micro phonic application, but there is no wave 5.  So this child would be ASD.  So as you can see this child can have this audio gram.  This child could also have this audio gram.  So we just don't know until we get them in the booth and get a conditioned response.  So really an important, not really outlier, but important aspect to exclude these because of children.  This concludes this section.  Do you guys have any questions at this point?  
    [Off microphone] 
>> The reason for the age‑old criteria from the NIH, from the FDA, sorry, that limit us to 12 months of age for cochlear implantation, more recently though, technology has obviously improved.  We don't make big incisions anymore, there's almost no blood loss, the surgery is sort of stream lined.  Now we sort of feel like you need those two tests showing you the same thing.  Also we still do a confirmatory VRA testing even if we have no response ABRs.  We know what the track for the child will look like.  The question is do we feel confident as a community to implant based on electro physiology only, and then it depends on well, do you have one good ABR, do you need two good ABRs.  A lot of centers still want two ABRs for example.  I would say how good is your first ABR.  And it's really to me centered around that.  There are some other medical considerations such as the size of the child obviously.  And well and you know, we do implant children that are younger than that if they have meningitis, simply because we have to.  Because that cochlea can ossify and then it will be lost for the rest of their life.  But those are really the cases unless you have done any other cases.  But that where we would consider implantation before that, I want to see that VRA test it could happen the day before surgery.  If you work this child up and there's a hearing aid trial and at nine months they had their VRA tested the day before, that would be fine.  I think that would be probably very good.  But I don't know that ‑‑ yeah.  Do you have a lot of thoughts on that?  
>> We'll take one more question.  I agree, behavioral testing is still sort of the hold‑up.  It's not really surgical risk.  It's really behavioral testing.  There was one other question. 
>> Where is JCIH on protocol what we consider a good pediatric test?  ABR, what's the criteria?  The criteria in the old JCIH is not really in keeping with the current equipment.  Is there any criteria coming out?  It's really difficult in the state, we've been trying to look and see what are we going to recommend that is considered a full evaluation. 
>> You're not talking about screening, you're talking about the diagnostic testing, right?  
>> Right.  
>> I'm going to defer on that question.  The document ‑‑ well, it's not really a part of our space.  I mean, that's a real solid audiology question, frankly.  And I think I would be outside my realm giving you details on that. 
>> You both talk about being part of JCIH, in the near knew tour ‑‑ 
>> We have a document on the way on that topic.  On a lot, 300 pages.  So you'll have plenty of chance to chew on that.  
    Just to jump back, we're not audiologists and we don't profess to be but we work very carefully and closely with audiologists as a team so we've had a lot of opportunity to have those discussions.  But from an otolaryngology perspective, our job is to classify hearing loss really as it relates to the anatomy and structure and function of the ear not just to classify it that you send us an audiogram or an ABR and we say that what says.  You guys are well equipped to do that kind of work.  But it is our job to try and put things in perspective for families relative to what's going on here, relative to what we're seeing in the testing and also put together some etiology information to try and give people some perspective on what might be causing the hearing loss and how that might be may or may not be remediable.  We talk about treatment and we talk about treatment together with our audiology and intervention colleagues.  We talk as a team.  And so treatment is not just medical/surgical.  That's our space of course but we talk about the whole prospect.  The thing that Oliver talked about, and I just want to sort of emphasize, is that all the guidelines for otitis media, all of the guidelines that you read for otitis media have nothing to do with children who have sensorineural hearing loss.  In fact it says this is not for children with underlying sensorineural hearing loss.  Children that have failed their screen and ultimately go on to a diagnostic ABR and they have fluid in their ear should be drained.  Does it need to be drained permanently or semi‑permanently with a tube or can it just be drained?  It can be drained.  But most importantly if a child is suspect of having permanent underlying hearing loss, we need to know if that child has permanent underlying here loss.  So we need to deal with that.  Low threshold for tympanostomy tubes.  The see dated ABR when we believe there's fluid in the way of that, we would commonly schedule a child.  Because we need to resolve these.  These parents come in, they have two or three ABRs, they have a bunch of unreliable information, they need reliable information so that we can move forward in the process.  So if I didn't say it enough, low threshold for draining fluid out of ears.  And then of course lots of communication across the group.  Really, really important.  I can't emphasize how important communication is.  Most of you know, roughly speaking, if you take permanent sensorineural hearing loss half of them are thought to be genetic these days.  Certainly about half of them are thought to be non‑genetic.  And those far and away genetics, CMV is most common.  Probably similar to auditory neuropathy, put these in your same category in your mind.  10% of new hearing losses are CMV related.  About 10% are auditory neuropathy, about a third have a malformation.  50% of the kids are falling into those 3 buckets.  Then about 10% have connects in 26 too.  So we're starting to reel this in a little bit.  We're starting to feel like we're getting closer to having a good idea.  These other things are pretty darn uncommon.  When you think about aminoglycosides, it's very rare that it causes a problem.  Hypothyroidism is screened for so rare.  So just across all of pediatric otology or pediatric hearing loss, complicated topic.  I know lots of people want to talk about lots of things.  It's really hard to cover all of these.  Etiology we sort of just talked about.  Obviously mild unilateral and progressive are all interesting topics and we'll mention a little on on that.  Neuropathy we don't talk much about but it's about 10% we'll maybe show you a case of that later.  Other things that are popping up which are very relevant to where we are today is objective measures.  If we had really, really, really solid objective measures that could potentially replace the behavioral audiogram then maybe we could move implantation up even earlier.  There is some issue those, once you start thinking about below like 7, 6 months, like families haven't figured it out yet, we're asking a awful lot, they're still proving to themselves the child even has a hearing loss, so I don't know.  I mean, my personal sense is that some time in the latter half of that first year is sort of a sweet spot.  If you think 10 to 14 months, that's a really good spot.  We're never going to show that 6 months is better than 9 months is better ‑‑ we may be, but that's not my sense, but if you look at the data that I have.  A year is a really good number.  If a child has meningitis, if a child has something that's really pressing I can see moving it up.  I've done a 5 month old before, really little.  I think sort of the sweet spot for me, behavioral testing around whenever we can do that, sometime in the 6‑8 month range.  Good solid VRA testing really conference the ABR it's in line with how the hearing aids have benefit, the child is under a good intervention program or is learning good skills from a therapist in some way or another, I think that's a really good spot.  And I feel bad when families are ready to go at 9 months and insurance won't go for it so we write a lot of sob letters and we get some approval.  But we shouldn't get too side tracked by the fact that a year is a pretty good spot.  I could see moving it up a little bit, but not six more months.  I'm not going to talk about ABI.  There's a lot of interest there, but it's ‑‑ yeah.  So implantation under 12 months.  SSD is emerging.  SSD is emerging.  For children.  In adults I would say it's emerged.  It's not totally common practice, but it's emerging.  Really interesting information coming out.  Cochlear nerve deficiency we'll show you some examples of that.  Most kids that are bilateral that are CI candidates there's no reason not to do two unless the family is really hesitant.  Two is definitely better than one unless you have a lot of low frequency residual hearing and the family wants to keep the hearing aid on the other ear.  I have very low threshold to do that assuming the family is in line.  Why does etiology matter and this has been asked to me many times over the years.  This is iron clad hard information that shows you were etiology matters.  It's not just touchy feelly.  It's the real stuff.  There are other medical conditions that can come along with hearing loss.  So some examples that are actually treatable, I put these here just because these are the treatable ones.  If you have a long QT on your EKG you can die and a child would only present would hearing loss.  Most of these are severe and profound.  Almost every year I hear about a child somewhere across the country in one of the Deaf schools or somewhere else that drops dead for no apparent reason, that should be identify with an EKG and they should get an AICD implanted immediately.  Another example is usher syndrome.  You think okay, well it affects their eyes but the child has a hearing loss, whatever.  This is really important with regard tos to communication strategy.  If a child is ultimately going to go on to lose their vision, it's probably not appropriate to be sent down a purely manual communication track.  I'm not saying that I'm against a manual communication track, I'm saying for these particular children identifying Usher is very, very important.  And the family needs to recognize that their ability to go down a manual communication track will time out at some point in the future.  Very important with regards to communication choice.  BVVL syndrome presents as garden variety auditory neuropathy.  So you think well, okay.  These kids have progressive blindness and progressive motor and sensory problems and they look like they are neurologically deteriorating before your eyes.  It's related to a riboflavin transport.  It's treatable.  It matters.  So if you have a child who is not a garden variety kid out of the NICU, they end up being identified with auditory neuropathy and there's no other apparent reason, they should probably be screened for BVVL.  There's a lot of buzz amongst BVVL families because many of them were missed including for myself, because we didn't know about it back in the day.  And then on the other side, here's medical conditions.  The other things identifying for prognosis.  So imaging is the highest yield of any of the tests.  Imaging has a 30% yield.  One in three kids that gets imaging we identify an inner ear malformation.  In some cases it's horrible, meaning that the cochlea is terrible, the nerves are absent or it has a really, really poor prognosis.  I'm going to say the exact opposite thing I just said about Usher.  I think it's wrong to send those families down a pure auditory pathway.  To push them to auditory oral purely, we should start talking to them about manual strategies to supplement their communication.  Exactly for the same reason that we feel bad about Usher syndrome and why they should be sent down an auditory oral pathway, you should say the same thing about severe malformations.  Most of our severe malformations do not achieve high level open set speech perception.  There are exceptions but these families need a communication strategy for their child so that should happen early.  Okay?  
    Genetic testing is becoming more common.  There is well over 100 types of or 100 different genes that are responsible for hearing loss.  I always put this in here.  This is from Walter Nance at medical college of Virginia.  It's a beautiful cross‑section scale with outer hair cells, inner hair cells, I love this stuff.  And then of course the spiral ligament.  It's just cool.  But what's interesting is this shows you the various mutations that impact the various areas of inner ear.  Some outer hair cells, some inner hair cells, some cochlear nerve or spiral ganglion cells.  If you look carefully at the mutations it's a variety of mutations and all of these look like clinically sensorineural hearing loss.  See all of these different genetic mutations look like sensorineural hearing loss.  There's no way to know that other than genetic testing.  The important thing is somebody has a Reissner membrane problem, are you going to give them hair cells to fix their problem?  It's not a hair cell replacement problem.  If this have a tectorial membrane problem, are we going to give them stem cells or hair cells to fix their problem?  No.  If they have a spiral ligament problem, are we going to give them hair cells to fix their problem?  This is a complicated problem.  What's the answer most importantly?  When the family asks you should we wait for a biologic therapy the answer can be a really solid nice definitive no.  It's not coming tomorrow.  The one that might ultimately have an impact in the next decade I believe is hearing loss from aminoglycoside toxicity in adults.  It's a clear, discrete leagues.  We know what they've lost, we know what they need.  But for children with all these different mutations, we've got a long way to go.  If their child is severe to profound, don't wait.  In fact I wouldn't even save an ear because we know that if it takes a long time for the second here that you've loved the binaural opportunity.  That's where we are today.  I might change my story in five years if something pops up for food I could tell families don't wait.  CMV really common, over a half of percent of all newborns are affected by the it.  Almost 15% experience some degree of hearing loss.  Many are asymptomatic which is scary.  It can be unilateral, bilateral, progressive, also see fluctuating vestibular orders in children as well.  It's a very interesting problem.  We did a study at UNC and looked at 100 kids that failed their newborn screen that were identified as hearing loss and about 10% were PCR positive on their Guthrie card blood spot, 60% had no clinical CMV.  These were kids that failed their screen, got their card from the state, did PCR for CMV.  CMV popped up asymptomatic.  One kid developed delayed seizures, a child that wasn't expected to have anything and all of a sudden pops up with seizures.  Many of these go on to be profound and you al of them have MRI changes.  So this is an example of what the MRIs look like.  So CMV kids, when kids have no etiology for the hearing loss they get their MRI and they say in the report that there's nonspecific white matter changes most common diagnosis is probably going to be undetected CMV.  I can't say that definitively but I can say most likely.  An 8 or 9 years old has specific white matter specific changes which most doctors blow off.  Many of those CMV and you can see examples here.  And here.  
    So CMV treatment works.  Unfortunately it's lethal.  It can be toxic, toxic lethal stuff.  So you have to be super careful.  About 30% of children who received valganciclovir, end up with severe neutropenia which means your white blood cell counts drop all the way down and you have to go to the intensive care unit for it.  For kids who are symptomatic with CMV, it's a reasonable thing to do because it's a life‑saving therapy.  For kids who walk in completely unsymptomatic other than hearing loss we're not there yet.  The clinical trials are ongoing to see if we have something that's less toxic but we're not there yet.  But my guess is this is something you'll see within the next five years.  My guess is you're going to have an oral agent which is going to be acceptable in terms of toxicity that will at least block the progression of hearing loss.  And in the trials of the children that are affected currently that have been treated today with valganciclovir there has been effect.  We've seen less progression of their hearing loss.  
    [Off microphone] 
>> It's a therapeutic course.  You get treated ‑‑ I will tell you, probably someone in this room is seeing doctors treating children with CMV‑related hearing loss with valganciclovir.  There are people actually doing it.  The problem is the risk profile for my way of thinking doesn't align.  So we have a pretty reasonable therapy for CMV‑related hearing loss.  If they lose all their hearing it's called a cochlear implant.  If not, there are hearing aids and those are pretty low risk.  And to have the risk of see veer neutropenia, end up in the unit with life‑threatening infection, we're just not there yet.  That's my personal opinion.  But there are people out there using it.  So.  
    Inner ear malformations like we said is about a third of kids that have identified sensorineural hearing loss end up having an inner ear malformation on a scan.  There's a lot of discussion about head trauma.  Head trauma is constantly talked about, the data is weak but it's something that we still owe to the families to talk to them about.  Because often times kids have minor degrees of head trauma and the family connects those things.  The data though is weak.  If someone is having progressive loss, especially if they have an inner ear malformation and spinal fluid behind their eardrum, a middle ear exploration is reasonable to try and create, correct any of the communications that are in there and I can show you an example of that.  I wouldn't routinely explore children who have inner ear malformations looking for these abnormalities because they haven't been shown to block the progression of hearing loss.  And implantation in the setting of malformations will show you some examples of that.  So this is normal ear anatomy.  For those of you who don't live in this space.  This would be a right ear.  So soft tissue is gray, white is bone, black is air.  The nose is up here.  The other ear is over here.  And this is the temporal bone.  The ear canal is that's the oracle here.  There's the malleus and the incus, the middle ear is this space here, mastoid is this space.  This is the vestibule.  This is the cochlea with the various turns, internal auditory canal which is where the nerves run down.  So this is beautiful anatomy, you can see an awful lot but you still can't see the nerves.  And that's a problem.  So the CT scan is good for identifying stuff in the air spaces like fluid, infection, erosive processes.  It's pretty good at identifying the malformations of the inner ear, it's really good at identifying the facial nerve in the middle ear and certainly the ossicle.  CT scan pretty good for those things.  MRI, different bird.  All of this stuff from here out on MRI, just looks block.  So not very good for bone, not very good for air.  But really good for soft tissue, which this is brain, liquid, which is white.  So this is cerebrospinal flood, cochlea, vestibule.  All of this is filled with fluid so we see that really quite nicely.  And the big advantage of MRI is here's the cochlear nerve.  You can see this is a little vessel and probably vestibular nerves here.  You can see all four nerves in the vast majority of cases.  So MRI and CT are complementary.  The question that we struggle with is knowing which of those is most appropriate for evaluating a child that has sensorineural hearing loss.  The MRI really gives you the critical structures.  The critical ones are do you have a cochlea, the answer is either yes or know and do you have a nerve.  The answer is yes or no.  It also gives a good look at the rest of the brain.  So for that CMV or if God forbid NF2 or some of these other things, it identifies all these things.  The CT scan for diagnostic test is pretty good, but it does miss cochlear nerve deficiency and that's a big one.  These are just of examples of abnormal in contrast of normal is here in the middle.  Actually normal is not here in the middle.  The normal cochlea has a basal turn and some of these other turns, boney partition here.  And this is a dysplastic cochlea.  You see it almost looks like a lollipop no partialing in the middle.  So you can envision in your mind that the nerves don't separate into all their various areas and here's an aqueduct as part of that.  This constellation, big group and large vestibular aqueduct is called Mondini's malformation.  It's a very specific malformation.  It's not just that you have a malformation.  A lot of people refer to Mondini's malformation as any abnormality, that's not the case.  This is specifically speaking Mondini's malformation.  Here you see sort of less severe effect.  So you look here, maybe a little bit less partial cochlea, although there is a little bony partition here, the vestibule still a big EVA, this is sort of a less severe of the Mondini's version.  And this is what Oliver showed you before.  Here's an example of a middle ear exploration here.  Right ear looking down the ear canal speculum with the eardrum turned forward this way.  The facial nerve is that little white thing there.  The stapes is that right there.  Do you see that blister, this is a child with EVA, and a little sort of cerebrospinal fluid blister there, hole in the base of the food plate.  This child had a CSF in the middle ear and needed it to be packed off.  We did a study about EVA and it's roughly about 10% of the kids that we saw.  It seems like everything is 10%, doesn't it?  10%, 10%.  And it's easily seen by both MRI and CT.  So don't let anyone ever tell you that it's not seen by both of them.  And many of them go on to implants and about half of the kids with EVA have a spinal fluid gusher at the time of surgery and it has no impact on performance at all.  It's just a thing for your doctor to clean up.  
    One of the interesting things about EVA is about two‑thirds of them have an air bone gap.  Probably because of a third window.  Their large vestibular aqueduct or lymphatic sac.  That he is important many of them are progressive.  And MRI is just as good as CT in identifying it.  Compare here on the right normal, normal IC, normal he cochlea, and you see this little cyst.  That's sort of a common cavity.  Compare this one.  This is the normal one.  Here's no cochlea, one vestibular system.  This is Michele's aplasia.  Not a good cochlear implant candidate since there's no inner ear.  This is one to remember.  If you look at this one, sort of a flat looking cochlea, almost like someone stepped on it.  It's not very big in this orientation.  It seems like it's okay that way to me when I look add it, but sort of flat here.  The internal canal, you would have to see it on a different cut, but relatively small and this funny thing here but no canals.  This sort of an ant mini for charge.  So if you see an ear like this, sort of a flattened hypoplastic cochlea and a poorly form vestibular system like this especially with a narrow auditory canal, the vast majority of those kids we see have charge.  And this is often the first finding and then they go on to be identified with all other things.  Because of newborn screening they're screened and we might see them and image them early and lo and behold, these are the surgical Olympics for cases.  Really tough.  So this shows the distribution of hearing types by malformation that Oliver put together.  And so is this is sort of that cystic group of malformations like the common cavity and then hypoplastic group which is sort of the small cochleas and then that incomplete partition group.  And what's interesting about it is over here is normal hearing and over this way is more profound.  So all of them have a fair amount of hearing loss, but the IP EVA group, the group that lives in that Mondini's spectrum can have very minor degrees of hearing loss.  So your protocol suggests that minor degrees of hearing loss you don't image you're missing some of these and that's important information for families so for me we image every child with new sensorineural hearing loss.  100%.  There's just the way I do it.  And if it pops up with a union lateral finding, what am I looking for?  The other ear to see if they have bilateral malformations in the setting of a unilateral.  If they have unilateral hearing loss that's profound and they have no nerve, that's really powerful information for the family, and for you as clinicians.  It tells you you don't need to spend time trying to get a hearing aid on that child and turning it up loud if the nerve is gone, the nerve is gone and let's move on with other thoughts.  I think it's powerful information and that's my two cents on that.  So in conclusion, malformations are common, they certainly impact what we do for families.  Surgery can be a little more complicated although generally speaking it's not.  So it's important to have good conversations with families and importantly if you have severe malformations, again we talked about a little bit you should think of other types of communication modes in addition.  I I'm not saying that you shouldn't consider intervention in the form of hearing aids and implants, but you should add to the mix because oftentimes even on their best day, they're not ideal.  And Oliver will show you some data there.  
    So this is my medical evaluation.  It might vary from others'.  When I see a child.  This is all the stuff I do if I see a new baby, history and physical exam, if they're complicated and they come out of the NICU, that's a little more complicated history and physical exam and the basics are MRI, EKG, CMV and connexon, in.  If I see a baby, MRI, EKG, CMV and I also talk to the family about getting an eye exam because I want to identify the usher cases and ERG doesn't do a fantastic job with that.  Electroretinography and it's asking a lot to put a family through especially when there's intervention and often times they're dealing with hearing loss at the same time.  The visual loss pops up in a more delayed fashion.  These are my things.  I consider CT scan for other unusual things.  If the internal canal is really narrow and I can't see the nerves, if there's other pathology.  And then in auditory neuropathy, MRI as well but you're not looking so much for inner ear malformations.  What you're looking for is brain changes.  If they're coming out of the unit, then they don't need an extensive valuation, something happened in the unit, nothing bad it's just what happens in the intensive care unit, most of our kids about 60% with ANSD come out of the intensive care unit.  If they look like a normal kid and they were identified with auditory neuropathy, if you had to guess, they'll have an otoferlin mutation.  That's the most common.  That was a whirlwind.  More questions?  
>> How old does the newborn have to be before you will do imaging?  Do you put an age limit on it?  
>> See I told you they would ask that question.  
[ Laughter ]
We went through this.  I said one of the people are going to ask what's the age.  I usually don't recommend to put babies to sleep for an MRI like at one or two months of age.  If they're showing up like 3, 4, 5 months of age, especially if they need a diagnostic ABR I like to try to do them together.  It takes something short of an act of Congress to make happen but that's a really good thing to do because you reduce the number of anesthetics.  I do like to get it in the first six months somewhere.  You could argue that for a unilateral delaying it some, but I wouldn't delay it until they're like five or something like that and they have a unilateral and lo and behold they start losing their hearing in their other ear an you see they have bilateral EVA.  For me I like to see it in their first year, it identifies bilateral malformations, it identifies white matter changes in the brain when I want to talk to them about CMV and those sorts of things.  So usually in the first six months for me 
>> So it's okay for a family if their physician is telling them to wait until a year, two years, you feel like that is okay for me to not push with the family to say you might want to ‑‑ 
>> I just told you what I thought.  
>> I know.  So 12 months is okay?  
>> I personally believe that it should be done in the first six months.  Is that rock solid?  No.  You heard my reasons for it.  And I'm not trying to be belligerent about it, but I think that waiting, there are times that things pop up and then you're feeling ‑‑ so you do it at three years and all of a sudden you pop up and you have bilateral malformations and they're like why didn't we do this earlier.  I would have liked to have known about it.  From my perspective I tend to be more proactive.  But this is the conversation I have with the families too.  Was there one other question?  And then we'll switch.  [Off microphone]. 
>> Certainly the most important part if there's no nerves, bilateral.  The most important thing is to give the child a communication strategy.  It's really, really, really important.  We've done ABIs and you know, the best that I can tell you is that we ‑‑ there's three groups across the United States, four groups across the United States that are working on this and none has produced an open set child.  And there's a fair number of kids that have been implanted.  So I don't think it's there to say we're going down the auditory oral route, don't waste your time with manual communication.  I think that it's a, it gives you sound awareness.  It's an adjunct to communication.  It's not ready for prime time when it comes to open set.  
>> Is your site saying ASD recovery and therefore is that impacting your recommendation for when to do an cochlear implant on an ASE kid. 
>> The question is about ANSD recovery.  I was talking with somebody about this the other day.  I think we tend on like to focus on the exceptions rather than the rule.  The vast majority of kids well over 95% of kids with ANSD do not have any recovery.  I've never seen a child recover from profound to normal.  And so everybody has their exceptions.  I'll tell you that most of the exceptions that we've seen have been related to poor testing.  It's been exceedingly unusual to have well‑done testing that clearly shows it.  There is a 10% temperature‑sensitive mutant that does show substantial fluctuations in auditory neuropathy, but again it's the exception rather than the rule.  I wouldn't design your entire practice around unusual exceptions.  The vast majority of kids don't have recovery.  We should keep moving on.  
>> I'm curious, you talked about testing for connexon.  When would you do a genetics referral?  
>> The sooner the better, right?  It's the same for imaging.  And I think you can do the connexon screener which can usually be done in halves so it doesn't take very long.  If you do the whole genetic testing, the 150, 180 genes, that will take a few months.  So why not ‑‑ well, it depends on where you are and where you practice.  And whether or not the child's insurance will actually pay for that.  We're looking at a substantial bill where I practice in the children' hospital I have to send to a jen et cyst to get the blood work done.  Some other people will draw it themselves and send it off.  I think it depends on a lot of factors, in my mind I agree with Craig, the more you know early on.  It's like the ABR scenario where you still don't know about hearing and families want answers and we like to provide those answers.  
>> Audience:  Thank you.  
>> Would you agree with that?  Okay.  
    Moving on.  So we talked about the evaluation of hearing loss.  Now these are the tools that we as physicians have and obviously not talking about hearing aids because we don't dispense hearing aids.  But we have implantable auditory devices that are either there to provide neural stimulation, so poor speech discrimination, we need to stimulate behind hair cells.  Good speech discrimination, or preserved speech discrimination, we can actually amplify.  For us it's either making the signal louder, and hearing aids do that well.  And if the speech discrimination is poor, hearing aids do not do well.  If you lose inner hair cells, if you look at the histology of sensorineural hearing loss, the most common forms of sensory hearing loss.  At a certain point you will lose inner hair cells and therefore, the child, the adult, the patient will lose speech discrimination.  So this is really where we try to figure out.  Obviously speech production can act as a surrogate marker for how much speech discrimination actually gets there.  But this is in our mind, this is what we, how we classify those two groups.  So obviously for poor speech discrimination we have a cochlear implant.  They have the conventional cochlear implant for bilateral moderate to profound sensorineural hearing loss and the hybrid cochlear implant which is down here as electrostimulation.  There's a lot of data on the adults cochlear implant world but there's limited data in children and frankly we don't know if it's really relevant for children to have that bimodal stimulation but I would argue that big issue with early implantation is better but the child has residual hearing.  So we're going to hold on to that residual hearing as long as we can despite the fact that the child has no speech discrimination sand therefore will fall behind on their educational measures.  So it's often times this back an forth and we all see it in our clinics.  We all have these progressive losses that it's really hard for families to make that next step.  And for us it is too, frankly.  Single‑sided deafness, bilateral cochlear implantation, I don't think it deserves any further belaboring in this setting and hair cell stimulation obviously with preserved speech discrimination.  We talked about hearing aids which is audio logical, bone‑anchored, a bone bridge which works similar to a BAHA.  Those are all devices we have in the management.  And you know, the problem as I just outlined, it's often a complex problem.  We would like to see that perfect timeline that I showed before, and Craig showed before that we like that perfect timeline to happen but oftentimes it's not that perfect.  The majority I would argue.  So severe to profound hearing loss pediatric hearing loss, no middle pathology, we as the otologists how we divide up the burden of the work.  But what it requires is we all talk to each other.  One of my first slides, it does take a village.  Those are some of the players involved.  We would like to think that we are one of the groups that should be involved.  We believe in this varies reasons we showed but it does required a complex, multidisciplinary network and we need to talk to each other.  A lot can be accomplished in a team meeting in my mind.  And it's really better for the family.  Again, the essence of the problem, do we destroy hearing and early is better.  Are we willing to destroy the hearing because that's what cochlear implants do or have done to this point.  Obviously there are hybrid implants.  Hearing preservation rates are not like stapedectomy.  I hope at some point we will get there and I hope I don't have to put this slide up because it would make our lives I whole lot easier if you don't get rid of something but instead of provide a cochlear implant, you just add.  That would be our wish.  And again, this is from the big study, the largest study, the NIDCI or the NIH funded.  The first author here and you can clearly see that earlier, and again these are children that were implanted about 15 years ago, arguably the age of implantation wasn't ‑‑ the poster child that we implant now.  So you can see that comprehension of expression scores for both domains early implanted children clearly have better scores.  So again, the basis of our data and of course this is one data set.  A very controlled data set because it was a perspective clinical child but there are other data sets supporting this.  And this is data from when Craig and I were at UNC and these are about a thousand children and each dot represents a speech discrimination outcome.  If you look on the Y‑axis we have a personal questionnaire.  We have ESPs, phoneme, ss a child has to be a certain age to complete this test and on the X‑axis we can see duration of use.  So the ability to basically comprehend.  Speech comprehension versus duration of use.  There are dots everywhere.  They follow this trend line, meaning that a lot of our children will ultimately be able to obtain open set speech discrimination as a relatively different task but again, there is a big variance, unfortunately.  And if you look at malformation what Craig showed before, how those children heard and we have to take into account biology, that's why imaging so important.  We node to know what substrate we're dealing with from an anatomic perspective because it does matter.  Just to remind you to Craig's slide, a lot of children with cystic malformation, basically all of them had a profound hearing loss.  And if you look at what intervention they received, they were either CI candidates, there was some sign language, but if you look at hypoplastic spectrum, a group of malformations, the highly associated with charge syndrome since the Jean has been identified recently.  A high incidence of no nerves.  Those are the populations where your cochlea will not allow a good enough signal to be delivered to the brain even with a cochlear implant for this child to develop spoken language.  I think it's important to set the expectations right and to look at the anatomy.  That's why we would say image early.  You see 60% of folks who have incomplete partition had either a cochlear implant or a cochlear implant and hearing aid on the other side.  So bimodal.  So you see even for not just for the hearing outcomes, but for the management outcomes and how those kids do, it really does matter what the imaging shows, right?  So similar to the other graph which I showed, a the dots representing a child's ability to comprehend spoken language.  We have several groups, the green one maybe direct you to the green children on the upper left‑hand side corner.  This is age and implantation and this is the speech perception where as between 4 and 500 denotes CNC open speech perception.  You can see the other dots are all children who have malformations.  But look at the left‑hand side.  Folks who have connection‑based hearing.  We have a very specific issue in the cochlea.  They cannot recirculate their potassium.  They have hair cells but they can't use them and eventually the hair cells die.  We know a specific genetic defect and I mean a cochlear implant is really good about replacing hair cells because that's what these children need.  And so what Craig mentioned the Mondini's kids, whether they have surgical issue of spinal leak during surgery, they do pretty good.  Between 400 and 500 they do have open speech perception.  We all struggle with some IPVA kids that they're implanted too late because of their residual hearing, et cetera.  We all know the speech production issues because of the fluctuating hearing loss, but from a perception stand point they do really well.  And on the bottom here, that orange line you can see folks who had, kids who had hypoplastic malformations and a lot of those are associated with not just having a nerve.  And it's not sort of shocking when we went to some of the conferences especially ten years ago where this whole topic about hey we should switch to MRIs instead of CT scans a lot of folks told us do you really need a nerve for a cochlear implant and we're like yeah.  You do need a nerve for a cochlear implant to work.  I mean, I guess we need a clinical trial for that, but it's similar do you need a parachute when you jump out of a plane.  Probably, right?  So, yeah.  
>> Any questions?  We're going to keep on the same sort of the same topics here.  We're going to talk a bit about cochlear deficiency.  I think everyone has one or two of these circulating in their practice one way or the other.  Very frustrating cases.  It's a smaller absent nerve on imaging.  We can't biopsy their internal canal to figure it out.  So we have to use imaging to know that better.  And what the actual cause, I guess it's sort of semantics, was it there and degenerated or was it never there to begin with, we don't know.  A lot of people ask that question.  There is direct evidence that this actually exists.  This is a temporal bone from the University of Chicago and what it shows is a normal cochlea, a normal rest of the ear is over here and there's a beautiful vestibular nerve and you see the meatus and you see there's no nerve.  This is sort of an a‑ha moment for us some areas is that the scala media or forms normally, cochlea duct forms normally and you can have a completely organ with no cochlear nerve.  Sort of an interesting nuance there that we've seen.  So this is the classic stuff that was picked out in the 1980s by rob Jackler, bill lucks Ford and a whole group.  They recognized in children that had CT scans before cochlear implants that were really narrow like this and especially if there was no communication between this narrow channel and that cochlea, you see how there's no communication, you see how it's going to think about it but it doesn't really happen, the internal auditory canal does not communicate with the cochlea, that's a really good communication that there is no nerve.  You could argue is there some different plane or whatever, we could look at these a million different ways and they said it's an absolute contraindication to cochlear implantation.  If the internal auditory canal does not communicate, just move on.  I think that's a really good point.  I'll show you some other issues which are more gray.  
    MRI really helped us a lot.  And you can see this is a beautiful example of an MRI.  And you see here on the left side a beautiful cochlea vestibular nerve coming out with the branching going into the cochlea.  You can see differentiation.  If you take it and look at it in cross‑section, you would expect to see four nerves in the canal.  The four nerves would be two vestibular nerves on the back side, a facial nerve above and a cochlear nerve below.  An in a relatively otherwise normal anatomy case usually gives a beautiful example of the anatomy so you can see that here.  So you know, MRI really started to kick in around the early 2000s.  Really when I got to UNC I decided we were going to do solely MRI rather than CT and that was a big change for what had previously been done.  And this case actually completely flipped my world upside down and it was one of the ones that sort of put my foot on the gas for it.  This is a child who showed up with bilateral total hearing loss acutely.  He was three I think at the time.  Had a little bit of an articulation problem but other ways was talking normal.  Came in with bilateral profound hearing loss.  And otherwise was developing as speech and language and otherwise a pretty normal kid.  He was treated with steroids, didn't get any response.  And they one of my partners saw him, they did an ABR on him for whatever reason.  He was able to get behavioral testing.  And his ABR showed an inverting response to condensation rare fax stimuli and the nose sound run was blank.  He had a cochlear micro phonetic with no distal waves and in the other ear he had a flat.  So back in the time this was not like right in our wheelhouse today you would look at us and say this is neuropathy and that's not, right?  But that's not where we were back then.  Lo and behold he had an MRI that had been done previously which was read as normal.  He underwent a cochlear implant in his ear that had responses.  Not by me.  And he got nothing out of it.  A sudden on set of hearing loss and then lo and behold implanted in this ear and if you look at this ear in cross‑section, here's the cochlea, looks fine.  Some cross‑section you see here's his normal side, two vestibulars, a cochlea, facial and a cochlea, he was implanted in this ear and got nothing out of it and ultimately we implanted him in this ear.  So how do you explain that in he probably had a sudden loss in his only hearing ear and it was missed on screening because he was screened with OAEs.  Did I just go the wrong way?  So cochlea nerve deficiency.  We've looked back on it over a number of years and we've identified a fair number of kids that have had that.  Most of ‑‑ you see 21 unilaterals.  If you see a child who has unilateral profound hearing loss for no good reason, we used to be told in our training that that was mumps.  That's not what it is.  Often times it's cochlea nerve deficiency.  I don't know why it occurs unilaterally.  These kids are otherwise normal.  Occasionally you see it bilaterally and you see rarely they pass their newborn screen like the kid I just talked about.  Most of them have a normal sized auditory canal.  And many over a third of them have a normal labyrinth as well.  So they can have a normal inner ear, a normal internal auditory canal and what that tells, and no nerve the ohm way to pick that up is with an MRI.  Because you guys are now experts on looking at these scans.  And 70% of the ears have a cochlear microphonic now that you saw the histology that says the inner ear can still develop with regards to the organ of Corti but yet the nerve doesn't have to be there.  So kids with cochlear nerve deficiency can have the electrophysiology of auditory neuropathy.  Some with auditory spectrum disorder actually have cochlea nerve deficiency.  The worst type of neuropathy possible.  A neuropathy so bad that there is no nerve there.  That's roughly 20% of the kids with AN.  It says here, you better be wear.  Especially if kids that are profound.  When you have no response ABRs, I would say that's an absolute indication for an MRI.  If you want to be a CTer, that's okay.  But you're going to miss some of these.  Really if you're profound, you really need to look for no nerve.  It's really important.  If there is auditory neuropathy you should do an MRI not a CT as well.  As you start to think about it, CT gives you radiation.  CT misses some of the brain stuff.  I'm sort of hard‑pressed to think that's the primary way to go into this.  Although it's certainly faster and it might be done pretty easily with light sedation or nothing in some cases.  You have to pick your battles at home.  Don't feel like you have to do it one way or another if your primary imaging modality ask CT you will miss some.  We usually go without CT unless there is some really compelling factors that are in place.  So with that being said, there's always exceptions.  We talked about exceptions before.  So here's sort of a typical case.  Five‑year‑old with charge has this flat‑looking little cochlea, a very, very narrow internal auditory canal.  Maybe there's a nerve there.  It looks like there's some are the some of a block across this side.  The left ear has a no‑response ABR, the right ear has what looks like a moderate conductive hearing loss, left facial paralysis, right normal small canals on both sides.  That certainly looks like a nerve.  There's a lot of uncertainty here.  We see a lot of uncertainty in these cases and I think they take an awful lot of discussion.  And you have to put all of your stuff together.  You have to put your MRI, you have to put your CT, you have to put your ABR, you have to put the response to amplification, you have to use all of your tools and stick them all together.  And that's why it's so critically important.  If you look ‑‑ I know Oliver would say the same.  If you look at our implant meetings as a group.  The garden variety child with Connexon, probably a cochlear implant, next.  One‑minute sort of thing.  These cases, they take up time.  In the end the best number we come up with is about 30% of the time, we just frankly can't tell.  Sometimes narrow internal auditory canal can't find any nerves, maybe profound, usually is not making progress with amplification.  We just don't know in some of those cases.  People usually asks about prom stim ABR.  It has not arrived.  Prom stim ABR makes a lot of sense.  Paul Calini has done a fair amount of work on this.  It hasn't been normed in kids with normal nerves and more importantly, sticking a needle on the promontory and stimulating results in some funky current spread so it's not definitive and I worry about false negatives because that means you would deny the child the cochlear implant based on not having a stimulus, but if there's a nerve there and your needle wasn't quite right, then that's a little bit of an issue.  So put all your information together.  Again here's another example of difficulties.  Right ear you don't see an internal auditory canal.  Really narrow canal.  It looks like a charged type cochlea, a little more of a vestibular system.  Have no idea what's going on back there.  Implant or not.  I think in the end you end up with a really long discussion with the family trying to figure out what the right thing to do is.  In this kind of anatomy I'm also talking about manual communication in addition.  
    Just to sort of jump on to what Oliver was showing.  This looks at a group of kids with cochlear nerve deficiency.  You might have say why did you implant 19 kids with no nerves.  I'll show you why, first of all I didn't totally understand it at the time.  The kids the speech discrimination hierarchy.  Pretty good performers, duration of use an months.  The kids in green are the hypoplastics.  Most of the hypoplastics get something.  There's some degree of open set, there's a few of them down here.  But look at the blues.  The blues really hang out down low and these are kids with cochlear nerve deficiency they don't do well with cochlear implants.  Some would say I've seen one.  We have two.  Here's one, two, three, four.  There's four kids out of the group that we've done with cochlear nerve deficiency that have some degree of open set speech perception.  That's why the FDA made us put cochlear implants into kids that were ABI candidates before we took them to do an ABI.  Because four kids, 19% is what it ended up being, was the group that did get some benefit.  So is it possible?  Sure.  You don't see any up here.  You don't see any up here.  Their anatomy is not normal.  Again, talk to them about enhancing their communication with other things.  They might be one of those, but again, they're going to be the exception rather than the rule.  Maybe one in five, one in six type chance.  And if you're implanting kids with charge, then the surgical risk is still substantial too.  So it's important to think about that.  Most don't get open set speech perception.  
    And this looks at ECAP which I think is interesting.  If you look at NRT, NRI, ART at the time of surgery in kids that have suspect neural problems, tells a pretty good story.  You can see here.  Speech perception and then duration of use again and abnormal e‑cap really abnormal e‑cap it's not 100%, there are on some outliers.  It's a pretty good indicator in children.  If you take a kid that you're not expecting anything abnormal and you go to the operating room put an implant in, you have great impede answers and you have no e‑caps, that makes me a little nervous.  I guess there are exceptions but there are not that many.  Adults is a different thing and I don't know exactly why that is.  So to conclude MRI over CT, CT in these four things.  Single nerve in the canal on MRI, cochlear obstruction in the instance of like post‑meningitis ossification, semicircular canal is abnormal really for surgical planning more than anything else and if there's something in the temporal bone that makes us nervous.  You have to use all the information that you have though to sort of make progress there.  
    And then the last little thing to say is about vaccination.  Remember vaccination is as health care providers, it's critically important.  I've had a number of kids over the years, I think now five kids that have had post‑implant meningitis.  And interestingly three of them we could clearly document that it came from the contralateral ear.  The classic sort of case, the single case that brought this to everyone's attention was a case from Pittsburgh in the early 1990s, it was written up by Suzuki and in that case the child had what looked like the Mondini and lo and behold the kid had strip meningitis and died and it was absolutely clear that the pus was in the bone of the ear that was implanted.  Does that mean that implants don't cause meningitis?  No I'm not saying that.  But importantly bad malformations predispose children to meningitis.  And whether you have a cochlear implant or not they need to be evaluated.  It can come from the implant ear as well much the positioner which was an issue back about a decade ago has pretty much gone away for the most part.  So the positioner devices that we were saying that were related to meningitis are not so much an issue but we need to make sure that we're clearly talking to families.  Prevnar 1 is where we are and the pneumovax and you can look on the CDC website on how to do that.  Last questions and we'll go over to a couple of cases.  
>> You had talked about the minimum age for CI's, but do you have any inaction maximum age in terms of children who were prelingually deaf and then are interring school or 5 or 6 and the family would like to pursue a cochlear implant. 
>> So the question is, is there an age that we start to think about I guess not providing cochlear implants.  I think that's what you're getting at.  I would say that the way that we work today, it's more about changing expectations.  I think as children get older, especially if they're prelingual and they're deaf, I think that we have to modify expectations.  We know that the device, unless you have no nerve and no cochlea, the device will give you auditory awareness in some way, shape or form.  But you have to have conversations with families.  If they're purely manual, and they're lag for some magic, it's pretty unusual to make that magic.  So a lot of it is about having conversations with families about expectations.  5 an 6 is not way over the curve.  10, 12, 13 is pretty far over the curve, but it's still not an absolute no.  I see a lot of families coming in late asking for it.  And I'm hard‑pressed to think that I should say absolutely no, because they won't stand up to the standards that I think they should ‑‑ because they should have been implanted earlier, does that make any sense?  So I talk to the family about expectations honestly.  And we have a good conversation.  The risks are pretty much the same.  The risks are the same whether they're an infant or a teenager or elderly.  So except in rare instances.  Does that address your question?  
>> And maybe if I can add here that in teenagers that are congenital profound ASL users, there's a substantial non‑user rate with cochlear implants that you guys are aware of.  And adding that to the counseling as part of the expectation discussion I think is important.  
>> There was another question.  Two others.  
>> I know you weren't talking about ABI but I have a quick question.  So we have a patient that we decided not to implant because it showed on her MRI that she had absent auditory nerve bilaterally.  She received an implant at another facility and now she came back to our facility and she's being programmed and she's getting some sound awareness definitely and it is a facility where they do do ABI's as well but they decided to do the cochlear implant first just to see how it goes.  She's not making much gains in her spoken language but she definitely is getting something from the CI and the family continues to ask us if they should go back, because the implanting facility had recommended maybe getting an A ban in addition to the CI even if she is getting benefit.  We told them to go back.  I was under the assumption that you if you get something with the CI you would get the ABI on top of it. 
>> The question is what is something.  So if the threshold with the CIs are usually in the 50dB range usually it's nonauditory stimulation.  And Craig taught me if you put a cochlear implant on your pinky finger you get an audiogram every time your finger wiggles and you raise your hand.  The question is if it's really auditory. 
>> Her thresholds are at 25.  And it's consistent.  Like really consistent and we're like impressed.  
>> Maybe the ABI is not necessary.  How long has she had the implant?  
>> She's had it about eight months so far. 
>> And how old?  
>> She's 20 months old.  So I mean, she's got great language.  She uses ASL primarily.  But they've asked everybody that sees her if they should go back.  
>> You know ‑‑ 
>> It's hard ‑‑ 
>> I think the adage is if a little good is more better.  And is more meaning should you undergo brain surgery to get more?  We haven't seen any of the children that vastly outperform that kind of audiogram with an ABI.  We've seen enhanced performance, but we're not seeing that they get the ABI and lo and behold it's open set.  But you know, there are pioneering families out there.  You can feel that spirit in them.  They want to go after stuff.  It's not the obvious next step.  I think a good point is to say that listen, this is not the obvious next step for your child to get there.  But some people want to go for it.  I think these are hard conversations.  And it's not really clear what the outcome is.  None of the kids are walking around with open set from their ABI.  
>> Right.  Thanks.  
>> Yes?  
>> I'm just curious about your management or intervention for a child with normal imaging that has ANSD but cannot behaviorally test.  Yeah.  
>> Oliver Adunka. .  The problem is you need a basis.  You need some thresholds.  At this point, despite what we talked about medical evaluation, hearing aid fitting is still based on not the histology, it's based on thresholds.  So it delays, right?  Auditory neuropathy delays.  So just turning a hearing aid up on medium there's just a substantial risk that it's either under or over fit.  I hope you get some critical potentials or some other physiologic measure that will allow us to obtain an audiogram in these children but pushing for that in my mind is still ‑‑ 
>> Craig Buchman:  I agree.  I think objective measures ‑‑ I really didn't hit it that hard.  Objective measures I think have promise.  They haven't arrived.  But I think they have some promise.  I'll tell you that in the early days when we weren't doing any of that, we sort of came to a point in time where we were like okay, we're not making any, we're really sort of dragging our feet here, working with Pat Rausch and others in the team and we said at some point we're going to have to make a decision and so we talked to the family, had a good hard conversation with the family and said listen, are you willing to give up an ear for this.  It's probably a more conservative thing to do is to say hey, you know, we don't exactly know ‑‑ it's usually a multi‑involved kid.  So we say listen, if you get an implant, we probably are going to cover the ANSD bases for the most part assuming that there's a nerve there.  This isn't many.  These are exception cases.  But you know, and so there are times after two and a half years we said okay, we've already spent a lot of time with this, we're really not having any success, we didn't have corticals at the time.  So we just picked an ear and went for it.  
>> [Off microphone].
>>  The question was whether or not we had to do an explant.  Yes.  Patient preference sometimes.  So late implanted teenagers who end up being non‑users.  I had to do one recently.  Reimplantation, implants are quite reliable these days but we went through each manufacturer over the years had a batch of implants that just weren't reliable.  So a lot of we used to do more than those in the ‑‑ than now.  There are some reasons for medical explanations.  Sometimes we have to take the magnet out to get MRIs.  Hopefully more will be compatible down the road.  It's rare, thankfully.  Do you have any more thoughts?  
>> Most of the explantations are related to device failure.  Rarely we see other things occasionally a patient wants it out of their head for some reason.  Often times it's a prelingual that has other stuff going on.  But it's pretty unusual.  On any given year about 10% of our surgeries are revisions.  
>> Will insurance pay for that?  
>> Yes.  And sometimes the manufacturers will pay for it too, depending on if there's an identified.  
>> Yeah.  
>> Any other questions?  
>> Audience:  I work in Maine as a consulting teacher of the Deaf and I wonder if you see many meth babies and what you do for them.  
>> Oliver Adunka:  As you know I work in Ohio we have the largest NICU in the country.  We see a lot from maternal drug use.  We don't see a lot of hearing loss associated with that.  But some, certainly.  And it's usually preemie.  They're in the NICU maybe some other reasons not just prematurity.  They're really medically fragile children that we manage according to the protocol but yeah, we definitely see it. 
>> Craig Buchman:  Just to jump on to that, I think that maybe upwards as much as 30% to 40% of kids are sort of Deaf plus or hearing loss plus kids.  I think that as a field we have a real opportunity to grow into that.  I think that we've been good in audiology, good in med surge, good in intervention.  I don't know we've been fantastic in the other disabilities or other issues that these children have.  Meth, cocaine, perinatal drug use, these all fall into the NICU category of kids that are there that Oliver referred to.  And really complicated ‑‑ I assume the case that you guys just talked about, ANSD can't do behavioral testing, these are all in that same realm and really frustrating for us to deal with.  But we certainly need other professionals.  Other professionals have to jump in in this space to help us forward in the goals of implantation or the goals of communication are oftentimes very different. 
>> Audience:  That's a follow‑up a connection to my next question is I didn't see you mention teachers of the Deaf at all when do you bring them in, the early interventions. 
>> Oliver Adunka:  They were actually on the team slide that I showed very early on.  And as physicians we really don't interface that much with teachers, but the team does.  And I think that it's very important ‑‑ I mean, it depends on which setting you work in.  In North Carolina we had a preschool associated with the center so obviously the teacher of the Deaf was part of our team meeting.  In Ohio unfortunately where I practice, despite the fact that it's a large pediatric hospital, I'm fighting for it.  But we don't have that option.  So it's really other facilities in the state or in the city that provide those services.  So what we do, we ‑‑ you know, different centers have different mechanisms for that.  But we do like to invite people who take care of either sign language or any kind of intervention children across our state to our team meetings and what was a beautiful thing in North Carolina was that our speech pathologists for example actually rented a car and drove out to each of the 100 counties and attended IP meetings for example and really helped the community take care of this child.  And irrespective of communication mode.  We really feel that approach has led the way for us.  I learned a lot from it and yeah.  So but again, as many models for care delivery in this aspect, you know, as people in this room, I guess.  Yes?  
>> Audience:  You had previously mentioned that the guidelines for otitis media don't apply to children with sensorineural hearing loss.  When you are seeing children who have been diagnosed with a sensorineural hearing loss and are fit with hearing aids and have chronic middle ear dysfunction or have continued middle ear issues, do you manage those kids at your facilities in a more aggressive manner, and if you do, could you speak about how you guys do manage that?  
>> Oliver Adunka:  Let me clarify.  So a child with sensorineural hearing loss with concurrent middle ear problem.  It's a common problem especially in the first two years of life, maybe even three years of life.  Really managing ‑‑ most commonly it's otitis media with effusion, most commonly it's chronic and we just put tubes in.  Usually the trip to the operating room allows a unique opportunity to confirm thresholds with an ABR especially since a lot of those kids will have comorbidities and sometimes there's a CT scan MRI at the same setting.  So often times we like to package it up and reduce the burden of general anesthetic.  So but as Craig said, if you look at the practice guidelines of the American academy of otolaryngologist, these pertain to children who have a normal cochlea.  As Craig said we want to get rid of the middle ear as a problem and just let the family and the child and us focus on the inner ear problem.  
>> Craig Buchman:  But you're supposed to follow up with saying when we put hearing aids into kids with tubes, they drain chronically.  
    [Off microphone] 
>> Absolutely.  
>> Craig Buchman:  Lower the threshold substantially when it comes to middle ear fluid because it messes up the whole timeline.  I think that's really, really important.  
>> Oliver Adunka:  Absolutely. 
>> Craig Buchman:  There are many special situations around this.  Down syndrome really, really special.  There are kids that I have with down syndrome that have mixed loss, a fair amount of conductive tubes and they drain and drain and drain times I pull the tubes outlet the drums here and they end up with 40 db hearing and they wear hearing aids.  We all have some of those going on and in a lot of ways I think that's actually better management than the tubes and the drainage and not being implication and that sort of thing. 
>> Audience:  Is there any plan as far as having tubes ‑‑ [off microphone].
>>  Craig Buchman:  In the JCIH document we actually talked about tubes fairly aggressively 
>> Oliver Adunka:  Often times if you're in the community and you work with just a general ENT, that becomes less of a perceived issue.  
>> Craig Buchman:  They call me and ask, I tell them to put tubes in.  Other questions?  
    Is this information helpful?  Okay.  Because I think our next ‑‑ we have some different cases, some that might be ‑‑ may be good examples.  
>> Audience:  I have a question for you.  It was from ‑‑ as a deaf person going to Gallaudet University, working on ‑‑ [off microphone] and child psychology ‑‑ are you talking about the possibilities or the impact on the children's language?  How can we improve these children's access to full language in those early months and years.  You showed that they, that you know, deaf children exposed to early sign language have the same development as hearing children.  So do we bring in ASL consultants or do we proceed just with the cochlear implant is part of regular business?  What do we do?  
>> Craig Buchman:  First of all, it's a fantastic topic.  It's a great, very, very important topic.  It's a little outside of medical, but I'm going to touch on it a little bit.  I would say that historically speaking, the cochlear implant world, sort of the auditory oral world has taken on a very sort of restricted view of this.  And I think there's an opportunity for us to broaden our view of it.  I think the information that's out there is exciting about adding early language exposure.  I think that we've been maybe too divided in our way of viewing this over the last 25 years.  I think it actually came from the fact that most of the kids were showing up late to the scene and so kids that were showing up 4 and 5 years delayed, we took a very pure auditory approach trying to get them to a good place for their language with cochlear implants.  But I think now that we're identifying babies early, I think there is a real ripe opportunity to study this and I know some of the literature is starting to come out that supports early language exposure in the form of either spoken language if they have some thresholds or using manual communications, sign language early on as well.  I'm not completely against it.  I just think that the information is just starting to emerge.  Anybody want to jump in on that?  
>> Audience:  I'll jump in I'm a pediatrician and one of the things I wanted to say.  This is addressed in an excellent chapter in a new book that came out last year called language deprivation and mental health and there's a chapter on cochlear implant use and about supporting exactly the not being rigid, really.  In an approach.  And I recommend it to everyone who's interested in this.  The other thing is that I noticed you took a really strong stance on the importance of not prolonging lack of access to language for children who are not likely to achieve open set speech recognition.  And I would say ‑‑ I would like to add something to that, which is that a number of the children in those dots that you have out there, those children were thought by their parents and make the providers that they were going to do well and the hope was that they were going to do well with pure auditory exposure.  But they didn't.  There's always a subset of children who don't do well.  And what's always perplexed me is why we don't talk more about protecting those children.  And so I think that this lack of rigidity and maybe coming together and not stigmatizing ASL but really looking at it as a way to nourish the brain while implants are being programmed and all of that, is really important.  
>> Craig Buchman:  I completely agree.  I think that the days of being in sort of divided camps' view on this are over.  But I don't think all the information has landed with us yet.  I think a flexible view makes a lot of sense.  There's also kids that don't develop speech and language well that have normal hearing.  So you have to sort of keep that in the background as well as you look at these kids.  It's like it might not be just a pure sort of communication issue.  There's more there that meets the eye.  But I agree with you. 
>> Oliver Adunka:  And we have by the way adult cochlear implants don't work for 100% of adults either.  But as Craig said, we're dealing with a spectrum.  Any other questions?  So let's move on to the cases.  They're fairly quick and I think fairly straightforward.  So again, pediatric hearing loss, complicated multiple specialties, early hearing decision and problem CI compromises hearing.  Case one.  First‑time mom, two‑week‑old infant, referred, past medical history not contributory, healthy child assuming nonsyndromic, term delivery, no NICU stay, no family history pertinent for hearing loss.  Natural sleep ABR again at that time point shows bilateral to moderate severe hearing loss.  Straightforward, first time mom, no family history, ABR, bilateral symmetric, moderate to veer hearing loss.  Craig, what would be your next steps?  
>> Craig Buchman:  Is it on?  Medical evaluation from my standpoint, MRI, EKG and connexon.  Get fit early on.  Genetic testing is a decision I have with the family.  I mean genetic testing beyond Connexon.  Moderate to profound doesn't necessarily make you a cochlear implant candidate.  EKG.  This is a gray one for EKG moderate to profound but I probably would suggest it since they could get it on their way out the door, it's really simple to go and talk about ophthalmology at a year. 
>> Any thoughts on involving an SOP at this point?  
>> Craig Buchman:  How old?  
>> Two week. 
>> Craig Buchman:  Yeah they get on to the team radar and I think being on the radar ask good.  Often times therapy ‑‑ it's not so much therapy as it's diagnose particular stick assessment of ‑‑ ‑‑ diagnostic assessment of progress and I think that's very valuable. 
>> Oliver Adunka:  Often times families can benefit too and it may tie into the communication approach that was mentioned.  Audiology sounds like getting ear molds and ordering hearing aids and in this child we may about the 1‑3‑6 may not be a problem.  So getting an early start.  We talked about the timeline we wish would happen more often and the clinical algorithm, make the diagnosis, image early, like observe until VRA is possible again with a hearing aid in hopefully.  Then do VRA.  If there is some discrepancies maybe think about a follow‑up ABR, retest, et cetera.  Amplify, auditory therapy, failure speech perception and language consider cochlear implant.  That's sort of in a nutshell that we talked about for the last two hours.  CNS findings.  Craig mentioned all those.  
    Next case.  Any questions about the first case?  Any thoughts?  Pretty straightforward, right?  
    Case two.  Eight week old infant.  Referred hearing screening bilaterally.  Past medical history are.  Family history is pertinent for hearing loss on the father's side.  Three previous ABRs have been noncontributory.  Again the child is eight weeks old.  OAE's were absent.  Indicating yeah this probably is some issue.  Tympanograms are type A no middle ear problems.  Craig, next steps?  
>> Craig Buchman:  Eight‑week‑old, may or may not sleep.  I think that three ABR's that are not contributory, I want a really good ABR and a really quiet room with really highly reliable results.  You know, this again requires a careful conversation with our audiologists about what the best approach would be.  The fact that the child has a strong family history for hearing loss probably for me I would love to do imaging and an ABR in the same setting so I would probably suggest to set up to do sedated ABR and MRI at the same time.  And then much of the same things that we talked about for the first case.  
>> Oliver Adunka. .  Obviously no hearing aid fitting based on what we talked previously.  We don't have thresholds yet.  What I mentioned previously comes in here the family is probably already frustrated.  They're like oh, I've did this three times, bringing the baby, not having the baby sleep.  Following all the rules we still don't know at this point.  You really don't want them to leave and get lost in the jungle.  You want to diagnosis this especially given the family history.  Again we're not big fans of putting kids to sleep.  We just like to know what's going on. 
>> Craig Buchman:  I can't emphasize enough the importance of an electrically quiet environment.  If you go to a particular operating room or MRI university or sedation place or wherever you are, if you're seeing lots of RF, if you're seeing lots of 60 herz stuff going on, you need to either get your facilities people there, or you need to go to a different place.  Don't keep going back to the same place and expecting it to go away.  If you've done that four, five, six times and you get in there, the baby is sleeping, everything is right and all you're getting is this really noisy ABR, you need to go elsewhere.  Because the vast majority of the time the noisy ABR is related to environment.  And so it's really, really important to ensure that that's being done properly.  I can't tell you the number of times you saw these really, really noisy ABRs and some little spec somewhere on the thing picks out as wave 5.  Just a bunch of chicken scratch and a wave 5 in the middle of normal.  So I think it's really important to find your quiet place. 
>> Oliver Adunka:  It's a great point especially in an operating room with a bunch of power outlets all over. 
>> Audience:  [Off microphone ]. 
>> Oliver Adunka:  There's likely a hearing loss present so why not.  If the OA's were present or, you know, likely you'll do a see dated ABR and you'll know soon.  We go back to actionable thresholds, getting those four frequencies for each ear.  I'm obviously not actually sitting there and working with the ABR machine but I see being efficient with it is a big part of this and again, amplification probably not without full ABR being present having at least two thresholds on each ear, preferably 4.  Following two attempts the reason Craig was eight weeks if it was four months okay it's time for ABR but three previous ones there are some slack obviously here.  And keep in mind the JCIH goal and the recommendation for 1‑3‑6 and maybe 1‑2‑3 which is not soon going to be recommended.  But there is some discussions about that.  And keep that in mind.  These are factors that where we do not meet that ideal timeline that we all love to talk about.  That delayed diagnosis, which is the case we just talked about, right?  And eight‑week‑old probably wet get a diagnosis here in the next month or so.  Residual delays.  If you have an arm that works somehow but there comes a surgeon I don't know that tells you I can get you a bigger and better arm but we have to amputate your existing arm, you're like probably not.  It has to be really not working well.  So fluctuating hearing, unreliable unconflicting test results which is common.  Oftentimes we get from our facility but also other facilities people come in have all these tests we try to get the records and all that and I don't really agree but you really don't know what happened.  It's easy to judge I guess.  And inappropriate amplification, speech development, good progress.  We've seen a lot a teenagers they come in, they have progressive losses and they have 100 db thresholds and you're like how are you doing this.  So that happens as well.  Parental issues, every time they come in they have a flat tire.  Economic, socioeconomic.  Multiple challenges obviously medically involved children, a factor for delay.  And anatomic uncertainty, deficiencies all those things we previously mentioned. 
>> Craig Buchman. .  And otitis media.  
>> Oliver Adunka:  Any questions on this?  
>> Craig Buchman:  On these cases actually I have a pretty low threshold to move sort of ahead of schedule.  I mean, if you have especially if there are three strong family history and cochlear implants and a really clear view of what their hearing loss, whatever their syndrome is, whatever it is, what it does.  My threshold actually for intervening earlier, my threshold is lower.  
>> Oliver Adunka:  So next case.  Former 33‑week preemie spent some time in the NICU for hyperbilirubinemia.  Had age 2 weeks corrected and these are OAEs and ABR are here.  So OAEs says pass.  On both sides.  And ABR this is just one ear now but you can see nicely that we have a reversing polarities and condensation and ABR's and no wave 5 obviously consistent.  Those are risk factors.  Again the child is now two weeks age corrected.  Auditory neuropathy real young child doing well otherwise but what are you going to do, Craig?  
>> Craig Buchman:  Actually I don't know that it's at this stage it's much different except that I think that providing amplification is difficult for you all since we don't know what the hearing is.  But medically I think it's still the same.  MRI, EKG, CMV and Connexon.  I think Connexon is really low yield in this setting.  Otherwise if it's a healthy baby, otherwise it's a 33‑week preemie.  I think the hard part is the conversation, obviously the audiology as well as us in sort of this watch and wait moment.  Some have talked about fitting hearing aids.  I don't exactly know how you would do that.  About 7% of our kids with ANSD end up with normal thresholds at the time of behavioral testing.  So careful observation of the child, see if they're making progress without amplification.  If the parents are coming in and saying nothing is going on you might have to change the way you're thinking, but for the most part you're in an observation mode until some time where behavioral testing is possible. 
>> Would you involve a speech and language pathologist at this time. 
>> Craig Buchman:  I don't know that it's so much therapy but I think it's diagnostic work and that could be helpful.  If it's a complicated child because they're a preemie that adds more than the story. 
>> Oliver Adunka. .  Go on in time, same child now nine months old.  This is VRA testing.  Next steps in. 
>> Craig Buchman:  It looks like they were one of our 7% that had ‑‑ I wouldn't just tell the family to go on their own.  I think that ‑‑ this is ‑‑ there is some art in this.  Because the question is, and someone brought it up earlier, is this a kid that this has resolved?  And you have to ‑‑ if you really want to prove that to yourself, you have to take the child back and do a sedated ABR and you have to ask yourself why are you doing that.  If the child has normal thresholds and they seem to be on target or all their other stuff, I would talk to the family and say this might have resolve but I don't know.  On the other hand I still think it's about sort of ‑‑ I would still put this child in the careful observation arm.  If there's white matter changes and all other stuff, then this child is still at risk for me.  But I don't know about taking them back for a second ABR.  I would probably ask the family what they want to do.  
>> Oliver Adunka:  Cool.  Again, this is the timeline, it's modified obviously can see highlighted it in red that you can see our ideal timeline really doesn't happen.  Likely with ANSD.  Initiation of the hearing we can do the genetic testing and obviously other things up here, but the initiation of the hearing aid trial will have to wait unfortunately since I don't know how I did that.  Okay.  But that's really the main problem.  Alternate scenario.  So let's just say this child also nine months old, conditions well, profound.  OAE still present.  Hair cells are wiggling, they're happy, they're just not connected.  Imaging MRI was normal, by the way.  Craig, any thoughts?  
>> Craig Buchman:  Well this child falls into your profound category.  Just to really oversimplify ANSD, because I think it was almost thought of as magical at some point.  But the real issue is this time between ABR and behavioral testing.  And then if behavioral testing is a problem, it adds another wrinkle to it.  But I think like once you're here, the hard part is gone.  This is basically a child who has profound hearing loss.  You can fit with high gain hearing aids as you're getting insurance in the mix and obviously you're having conversations with the family about what they prefer, et cetera, but this is a much more straightforward and outcomes from implantation are exceedingly good assuming the other factors aren't major. 
>> Would you think that the hearing aid trial in a case like this with or without ANSD, maybe you can comment on this.  Would you think that the hearing aid trial would be mandatory?  I know it is from an insurance perspective in your mind?  Is this what we're going to do in 10 years?  
>> Craig Buchman:  I think the amount of time it's going to take to get this approved ‑‑ if adding the hearing aid trial adds weeks and months, then I think it's not necessary.  I think that this was done back in the day when we didn't know if CI's were going to work.  The whole three month hearing aid trial.  I don't know that it's here fully to stay but I'm not against doing it at this point.  I think it's a good thing to do while we're trying to get insurance coverage and all those other aspects but I don't know that I would extend it to three months to check some box. 
>> Oliver Adunka:  Any thoughts from the audience?  
>> Audience:  There were some early questions about thresholds don't meet criteria.  
>> Oliver Adunka:  Let's assume this child has a moderate hearing loss.  This child otherwise doing well, not meeting ‑‑ it's a surrogate marker for the signal and if the signal is Charlie Brown teacher we hope that cochlear implant is doing better, right?  Absolutely.  
>> Craig Buchman:  I'm going to backtrack on that a little bit.  There was a group of researchers who were strong advocates for bypassing amplification in children in lieu of cochlear implantation because they believed that all of these had the Charlie Brown teacher.  I do not think that's the case.  So amplification does have a role.  If you have a moderate hearing loss.  If you don't make progress with amplification then I think it's reasonable to consider a cochlear implant in that scenario and it's a very complex insurance run.  
>> Oliver Adunka:  Sorry if I missed that it was in the lack of speech and language progress.  
    I think we have just one more case.  I brought a bunch but in interest of time.  A four‑week‑old infant, term delivery past history noncontributory.  Referred for hearing screening on the left only has a natural sleep ABR shows this.  It's not the cleanest study but bear with me here.  On the right you can see basically 20 still has a wave 5.  At 4,000, 2,000, 500 normal hearing essentially in the left ear.  The referred side has reversing polarity.  Again I apologize it's the cleanest of all ABRs.  And this goes back to the case that maybe you want to run us through that MRI, Craig?  
>> Craig Buchman:  I think it's pretty clear it shows unilateral cochlear deficiency.  This is very common.  This is not oh, I don't know how many kids you see.  This is not like one you're going to see one every five years.  We see this.  And I think this ‑‑ I really believe this resets the family expectation for that child.  We're not talking about maybe SSD, maybe consider cochlear implant.  It sort of takes that off the table because you have a normal hearing ear probably on the side assuming that behavioral lines up.  I think this is interesting for the family.  It's a child who has a single‑sided hearing loss.  The risk to the contralateral ear is very low although I did show you a case earlier, but it's very low.  I think it's helpful information. 
>> Oliver Adunka:  It's at least once or twice a month that you identify this.  If we didn't do MRIs, you don't identify.  People who tell you oh, I hardly ever see that, probably because you're not looking.  So it's a problem.  Right?  
    All right guys.  I think we're out of time.  I brought a bunch of cases here.  Sorry we don't have time 
>> Craig Buchman:  Did you get all those?  
>> Oliver Adunka. .  These are the conclusions.  Thank you for showing up.  It's been a long two and a half hours.  Thanks for participating.  Obviously it's an important topic.  That's why we're all here it makes us get up every morning.  It does require a large team approach and the diversity of the group is the key in all this.  Multiprofessional groups and again, I don't want to exclude anymore.  These are the ones I interface with as a physician for the most part but recognize there's a lot more happening on the field with what kids with hearing loss will acquire.  Appropriate newborn screening is appropriate.  In the United States, some states are better than others and by several hundred percent sometimes.  Early amplification, proper evaluation are critical.  Early amplification is absolutely critical.  We feel like not just amplification but early EI.  And providing families those options early on I think.  And delivering data for us it was very common that we see that hearing loss is not properly diagnosed, that a family still doesn't know what's going on exactly.  I think providing that data to families is absolutely critical.  Not just for audiology, for us to getting the imaging data.  Knowledge is power and I think it helps us in managing hearing loss.  Craig, do you have any other?  
>> Craig Buchman:  No.  
>> Oliver Adunka:  Thank you very much.  Hope you guys have a great meeting.  
>> If you guys could please leave the gold evaluation forms on your chairs, I'll come around and pick them up and I think I got everyone.  If anyone didn't check in with me in the beginning, please come see me before you leave so I can make sure I have your name written down.  
    [Session concluded]