Auditory Neuropathy Sound Support Parent Webinar

Auditory Neuropathy Sound Support Parent Webinar


(upbeat music) – [Moderator] Hello everyone and welcome to the University of
Michigan Sound Support Parent Webinar Summer Series. – [Brooks] My name is Emma Brooks and I am the Sound Support coordinator for the University of Michigan Hearing Rehabilitation Center. I hope you all are off to a
great start to your summer. As many of you are aware, Sound
Support is an outreach grant funded by the University of Michigan Department of Otolaryngology
and Michigan Medicaid. The grant is designed to
provide outreach and support to children with hearing
loss throughout Michigan. We’ve put together this
series of webinars, webinars for parents due
to the great responses from the past few years’ series. We hope you’ll find them to be helpful and we encourage you to share topic ideas for future discussions
with us on our evaluation, which will be sent at the conclusion of our three-part series. We strongly encourage your
feedback and suggestions. You will find presentation slides located on the handouts
tab, located on the toolbar. You are able to download these or print them directly from
your computer or device. There is also a questions tab. Please feel free to ask questions throughout the presentation and we will address as
many of them as we can at the end of the presentation. If you are watching a recorded
version of this webinar, feel free to send your questions to me at [email protected] Today our webinar is titled
“Auditory Neuropathy.” I would like to take the opportunity today to introduce our presenters Kelly Starr and Doctor Caroline Arnedt. Kelly Nichols Starr is a
speech-language pathologist and a certified auditory verbal therapist and listening and spoken
language specialist at the University of Michigan’s
Cochlear Implant Program. Kelly received her bachelor’s degree from Michigan State University and completed her master’s
in speech-language pathology at Wayne State University. She has presented
nationally on various topics related to pediatric hearing loss and development of listening
and spoken language. Kelly is part of U of M’s
Sound Support outreach grant, supported by the state of Michigan and the University of Michigan
Department of Otolaryngology providing outreach and lectures to educational professionals and students in the state of Michigan. Caroline Arnedt is a
cochlear implant audiologist at the University of
Michigan in Ann Arbor. She has worked with pediatric and adult cochlear implant patients
for over 10 years. She coordinates a multi-site, longitudinal clinical research study looking at factors affecting performance of pediatric cochlear implant patients. She has previously served as a
research audiology consultant for Kresge Hearing Research Institute at the University of
Michigan Medical School. She has presented
nationally on various topics related to cochlear
implants, pediatric audiology and auditory neuropathy. I’ll go ahead and hand it over and we will get things started. – [Arnedt] Thank you, Emma,
and I wanna thank all of you for joining us today either live or through the recording of this webinar, for this parent webinar
on auditory neuropathy, sponsored by the University of Michigan Sound Support outreach grant. So as Emma indicated today, both myself and my colleague Kelly Starr are gonna be providing
a basic introduction to auditory neuropathy. Our goal today is to help you build a solid foundation in the
basics of this condition, so that you have a better understanding of auditory neuropathy and as
well you can better explain this complicated and
often confusing condition to friends and relatives. So let’s start by orienting
ourselves to how we hear and how the auditory system works. So sound is picked up by our outer ear, travels along our ear canal, causes our eardrum to move back and forth, which then causes these little bones in our middle ear to
also move back and forth, which then pushes on our cochlea, moving the fluid in our cochlea and causing those little hair
cells in the cochlea to bend, which then sends a signal
to the auditory nerve, which then sends a signal up to the brain. Now in different types of hearing loss, let’s say there’s an issue
kind of in this area, so either in the ear canal
or in the middle ear. This type of hearing loss is called the conductive hearing loss. We can’t conduct to the sound, we can’t get it where it needs to go. Oftentimes this type of
hearing loss is temporary. It can be treated medically. The next most common type of hearing loss happens when there’s something
wrong in the cochlea. So for some reason, we can
get the sound to there, the ear canal’s clear,
the middle ear is clear, but once the sound gets to the cochlea, there’s a problem. And this type of hearing loss is called sensorineural hearing loss. The type of hearing loss that
we’re gonna talk about today, auditory neuropathy, there’s
something that’s not working in the connection between
the cochlea and the brain. So somehow in that area
something’s going wrong, which is causing a type of hearing loss, which we refer to as auditory neuropathy. So let’s look a little bit more in depth into what really is going
wrong with auditory neuropathy and why it’s so complicated. Auditory neuropathy really challenges the way the field of audiology has traditionally thought
about hearing loss. And to be honest, I was
originally drawn to audiology because I loved math and science and the black-and-white nature of audiology really appealed to me. Individuals could either
hear or they couldn’t, and an audiologist would determine the degree of hearing loss,
prescribe the treatment and re-test to ensure
that the treatment worked. Now with auditory neuropathy,
it’s a condition where we see individuals who have
a significant mismatch between what they can hear and their ability to make sense of and understand and use what they’ve heard. And we’ve had to re-think a
lot of our treatments as well. I have a lot of parents of
children with auditory neuropathy who are confused and frustrated as to why, if hearing loss in general has been around since the beginning of
time, why does it seem like we haven’t really figured
out auditory neuropathy yet? And we can’t give them a straight answer about their child’s hearing loss. So I wanna give you a little
bit of background on that. One of the reasons for this is although auditory neuropathy
is not a new condition, people didn’t just start
having this condition, it’s a newly identifiable
or a new diagnosis. And so back in the 1990s, a test called an otoacoustic
emissions test or an OAE test, became commercially available. And this test was able to isolate and test the function of the cochlea independent from all the other parts
of the auditory system. So as you can see here a mini probe with an ultrasensitive microphone is simply placed in the ear and can measure the health of the cochlea. So we were then able
to uncover information about the cochlea in isolation. So as this test was used more and more after its development, the fields of audiology started to note that some patients who appeared via an auditory brainstem test or an ABR like many of you are
probably very familiar with, that the ABR indicated that an individual could not hear well, but actually then they had OAE test which told us that they
had good cochlear health. And this discrepancy was
found again and again. And further investigation
led us to understand that the auditory signal, which is highly dependent on this synchronous transmission from the nerve to the brain was somehow being disrupted or it was no longer synchronous. And these individuals
appeared from this OAE test to have normal functioning cochleas, but when they had the ABR test, they had abnormal neural function. Thus we have this condition
where an individual appears to have a healthy cochlea,
but a hearing nerve or an auditory nerve that
wasn’t firing reliably or not with enough synchrony to get a clear message to the brain and this is what has become
known as auditory neuropathy. Just as a note, there were
also several other names that were trialed kind of along the way to attempt to better
describe this condition, including auditory dyssynchrony and auditory synaptic disorder but auditory neuropathy has really seemed to stick throughout the years. There’s been some work done to simulate what an individual with auditory
neuropathy actually hears. So I’m gonna play one of
these simulations now. And what you’ll hear is Arnold
Starr who is an individual, a researcher credited with
first uncovering this condition. He’s gonna repeat the same
sentence several times with varying degrees of severity, starting with kind of the most
severe mirrored simulation and then he’s going to end with the sentence completely unaltered. And you can hear this
timing just isn’t right. There’s this significant
temporal processing or a timing problem with the sounds rendering you unable to kinda make out the sentence in the beginning. So I’ll go ahead and start that now. That was the wrong button. I went to the next slide, sorry. (laughs) Let’s try that again. – [Starr] (playback of distorted sounds) – [Arnedt] So it’s
believed that the severity of that distortion you just heard is dependent on exactly
where the damage is. So we come back again to this graphic. And in talking about auditory neuropathy, we’ve localized the damage to this area between the cochlea
and the auditory nerve. But within this area,
there are several locations for possible damage. And unfortunately, we do
not have the needed tests that are extremely specified to determine the exact site of the damage that’s causing
this auditory neuropathy. So damage can be where the
signal leaves the cochlea or it can be where the
signal gets transmitted from the cochlea to the nerve or it can be where the
signal reaches the nerve or it can actually, there can be damage with the nerve itself. So individuals with the same diagnosis of auditory neuropathy can have varying areas
of damage in their ear. It also makes sense then that the effects of auditory neuropathy on
one’s hearing can also vary. So here are some hallmarks of
kind of auditory neuropathies again, that can vary per individual. But they can have hearing
detection at any level. So if we’re behaviorally
just testing their hearing in the sound booth and we’re looking to see when
they first respond to sound, those behavioral measures
can reveal normal hearing all the way up to a profound hearing loss. That hearing can also fluctuate. So parents will say that their child seems to have good hearing
days and bad hearing days. Their understanding of
speech is going to be poorer than we would expect. So often we’ll see kind of a discrepancy between someone having
kind of a mild hearing loss but responding similar to what a child with a profound hearing
loss would respond as or at more, at the connected speech level. So they’re able to respond
to and understand words in isolation or if you just
say a single word like shoes. But if you have words kind of
connected together as we speak and say, “Go get me your shoes
and bring them over here, and make sure that you
also have socks on,” that individual can’t make out any of that because it all smears together
like in that simulation and it makes sense also because
of that timing discrepancy that there’s also these individuals have an increased difficulty
in background noise where they have a lot more
difficulty understanding. So the damage in auditory
neuropathy can vary. The hearing symptoms can vary. And as well, the reason or the cause of the auditory neuropathy can also vary. So auditory neuropathy can also arise as a result of genetic factors. So there are certain genetic mutations that have been identified. Very likely not all of
them have been identified. But there are several
that have been identified that lead to auditory neuropathy. Auditory neuropathy can be due
to anatomical abnormalities. I think there’s some research out there that suggests that 40% of
cases with auditory neuropathy actually have a genetic basis whereas fewer than that are actually due to an anatomical abnormality. Neuropathic conditions can
result in auditory neuropathy. Conditions related to
prematurity or immaturity, such as extremely low birth weight, prolonged mechanical ventilation
or issues with jaundice. And there are still patients
who have auditory neuropathy and there is no identifiable cause with the means that we
have now to determine that. So as you can kind of see, this point that I keep hovering on and that is that this great variability. So there is great variability
as to where the damage is in the inner ear or the nerve. We can’t exactly pinpoint
where the damage is exactly and it varies per individual. The reason for the damage also varies. And this is going to lead
then to great variability in the auditory impairment or the symptoms that that
individual is going to have and as well then there’s
going to be variability in the treatment outcomes. So in recent years,
there has been a movement to classify these individuals that have auditory neuropathy where we have this kind of present OAE, so that OAE test told us that
they have a healthy cochlea and we have absent or abnormal ABR testing that these individuals actually are part of a spectrum disorder and very likely with future advances in available diagnostic tests, many of these conditions that are kind of put in
the same bucket right now will be recognized as
individual conditions with treatment recommendations
that can be more specified. However, until that time, all possible treatment options should be considered for
every individual diagnosed with auditory neuropathy. So hearing aids, hearing aids
are one possible treatment. And prior to 12 months of age, they absolutely should be trialed. However, it’s important to remember that one of the hallmarks
of auditory neuropathy is this temporal processing impairment. So this kind of inability to process the timing clues of sound. So hearing aids take sounds in the room and they make them louder. And hearing aids, they’re
improving your sound audibility or ability to detect sound. They cannot resolve this
temporal processing impairment. Cochlear implants are also an option. Following a hearing aid trial with a child who is in
regular speech therapy, if a child demonstrates poor progress in speech understanding and
auditory language development, they absolutely should be
evaluated for a cochlear implant. Cochlear implants do have the ability to improve temporal processing because the electrodes that are
implanted within the cochlea can stimulate a synchronous
firing of the auditory nerve. And FM systems are highly recommended, either coupled through a
hearing aid or an implant or used independently because
they’re able to lift up or get that target sound even louder. And it helps with this
temporal processing impairment. So patients with auditory neuropathy are better able to process speech in the presence of background noise because we’re kind of getting rid of that background noise factor. So to summarize, one of the reasons why auditory neuropathy seems
new and seems complicated is because it’s newly identifiable, right? So back in the 1990s is when we started to regularly use these audiologic tests that are going to be able
to more specifically measure specific auditory functions. And that’s where we found out
that in some of these cases, we have a cochlea that’s
going to be able to work for this neural
transmission that is faulty. It’s also important to remember that a lot of these cases
with auditory neuropathy a lot of these kids also
have comorbid diagnoses. So they have other things that they’re working through as well. And so, kind of medical complications are going to add to the
complexity of the diagnosis of auditory neuropathy, the means of treatment and the outcome. So kind of putting all of that together, given auditory neuropathy’s
kind of newness, combined with all this variability, there is no gold standard of treatment. And as well, one other
thing to think about is that the incidence
of auditory neuropathy is likely on the rise
due to medical advances that are allowing for
increasing survival rates of extremely premature infants. So kind of all of that that goes along with being extremely premature
puts you at greater risk for developing auditory neuropathy. So as those babies are having
increasing survival rates, the likelihood that auditory neuropathy is going to be diagnosed
more and more is pretty high. As parents, this is very complicated. And I encourage you to
give yourself a break. Many of you, the first
person you’ve likely ever met with auditory neuropathy is the person that you love the most in this world, and that’s your child. So this is a lot. And our recommendation for parents is always to make as many
appointments as you need, and the more appointments the better, with your audiologists for
kind of continuous monitoring of your child’s progress but also for continuous understanding as a parent as to how auditory neuropathy affects your child. When you get a diagnosis
of auditory neuropathy, it’s going to tell us
that that’s the condition that your child has. Auditory neuropathy does not tell us where your child is going or
how they’re going to get there. And because of all of this variability, every child with auditory
neuropathy spectrum disorder is going to have their own story. And they’re going to
need an individualized consistent treatment plan that will help them write their story. So now, I’m going to turn
it over to Kelly Starr, who’s one of our speech pathologists, who’s going to speak more to
this individualized management of auditory neuropathy. – [Starr] All right, thank you, Caroline. So how does speech and
language present itself for children with auditory neuropathy? And for the way my mind is wired, auditory neuropathy spectrum disorder helps me remember that auditory neuropathy can present itself on a spectrum, and therefore will have
different performance outcomes. So for communication and talking, we need to have clear access
to all the sounds of speech. Here we have the audiogram. And across the top, we have frequencies that go from low to high,
like a piano keyboard. And then, going from top to bottom, we have sounds that go from quiet to loud. So the audiogram shows us that
they have detection of sound and usually this is my
go-to for talking about what a child hears in comparison
to the sounds of speech or our speech banana, which
is highlighted here in blue. When discussing auditory neuropathy, that does not give us a complete picture because the audiogram does not show the information about the timing. And the timing is what’s
needed for comprehension. The audiogram does show
us about the audibility. So what we are talking about is the difference between
audibility and intelligibility. Audibility means that,
“Oh, I can hear it,” and the intelligibility,
“Like I can hear it, I can detect sound is there. But it’s like having the TV on, but it’s not loud enough to understand.” There we go. And then, intelligibility
allows for understanding. And intelligibility is what we need for learning spoken language. Another way to talk about this is you may recognize this voice. And I’ll play this. This is where the signal is audible. – [Playback] (Charlie
Brown teacher mumbles) – [Starr] So it’s the
Charlie Brown teacher. And so, you can tell
there that it’s speech, that somebody’s talking. But we aren’t able to understand what is being said. And then, here is an
intelligible auditory signal. – [Moderator] Welcome to
today’s parent webinar about auditory neuropathy. – [Starr] Now, here’s
where auditory neuropathy makes it even more confusing because someone with auditory neuropathy may have normal detection, but only have audibility because the timing of the
signal may be impaired, making speech unclear. With auditory neuropathy, we need to think about
normal detection of sounds across all the frequencies, but also determine if the sounds are clear or is the timing normal. And I’m a visual learner. So this is the one way I’ve thought about how the timing may affect understanding. This is a phrase where
I’ve changed the spacing of the letters and the words to demonstrate what it would be like if the timing was off. And we can’t really understand
what is being said there. And then, for intelligibility,
if the spacing is normal or the timing is normal, it would say, “It’s time to go. Get your shoes on after you
take your bowl to the sink. And remember grab your lunchbox.” So for intelligibility there, we need the consistent
detection and a clear signal. So if we have that incoming signal, even if it’s in the normal range, if we don’t have clarity, it’s not going to be understood, regardless of where it’s coming in. All right, so how do we determine if your child has audibility
or intelligibility? In combination with audiology, we want to have a speech
and language evaluation. And this provides a baseline
for future comparisons to know how much progress is being made within a certain time period. So we expect the child to
make six months of progress in six months of time with their language. Our speech and language eval also shows how to talk, allows you to talk with the speech pathologist
about your expectations and communication for your child. We recommend the evaluations be done for all children with auditory
neuropathy after a diagnosis. Even at a few months of
age, the speech and language can be assessed to determine if the child’s following a
normal developmental pattern with their listening
and their vocalization. There’s a lot happening within that first year
kind of behind the scenes with listening that sets the
stage for language development. I like to think of it
as the duck in the water with its feet underneath
that are constantly moving just like newborns, that first year, they’re constantly listening and there’s a lot happening in that brain. Think if they have good
detection of sound, your child might hit some of those expected language targets in the first year such
as startling to a sound or turning to voice. But by nine to 12 months,
they need to have more than just that detection of sound. And we don’t want to miss out on a year of learning language
to find out there’s a delay. So the sooner, the better. Even for children with auditory neuropathy that have normal speech and language, we recommend interval evaluations to monitor progress and these evals look at
a variety of the areas, including articulation, or
how a child’s speech sounds, their vocabulary, so single words, understanding of language
at the sentence level, how your child uses language words or how they put their words together. Also looks at place skills and literacy depending on their age. So this variety lets us see how a child functions in different areas. So for example can they do really well at the single world level? Like if you say, “Ball,” like
can they go get the ball. But if you put that into a direction, “Go find the ball under the chair after you get your shoes on,” is that where that breakdown happens when it’s more connected speech? So frequently for children
with auditory neuropathy, the longer the phrase, the more difficult it
can be to comprehend. There’s more areas for that
timing breakdown to occur. Monitoring the language and listening is our way of following
the child’s language and knowing if they’ve
progressed in a timely manner or if it’s slowed or stopped. So we want to make sure
they just stay on track. Given that no two children are the same, our evaluations allow
for testing of language to determine if they’re in
their age range for language or if their chronological age is above what their language age is. So is there a gap or a delay or are they meeting their milestones. So if there is a delay, we
recommend weekly therapy. And here, we do auditory verbal sessions, which are diagnostic in nature. So it allows ongoing
assessment of our goals, also for you to know
what to watch for at home and how to implement those goals at home, as we have you as parents in our sessions. This also provides an
opportunity to collaborate with the child’s audiologist
on a consistent basis for us to check in with them and provide information about
how their progress is going. Here at U of M, we have four therapists, speech therapists
specializing in listening and spoken language. And for more information, check
out our next parent webinar but also there’s great
information on the AG Bell website and the Hearing First website. Overall, listening and
spoken language specialists work with children who are
deaf and hard of hearing and their parents that are seeking listening and spoken language outcomes. Therapy allows us to assess
speech production errors and determine if they’re
able to produce the sound through listening and
monitor that development with in combination to their age, is it age appropriate or not. In order for natural
sounding speech to develop, your child has to have
access to all of those sounds and it has to be a clear signal. So these sessions allow us to learn about your child’s auditory behaviors, what they can do through listening alone, are they using gestures and visuals to decipher the messages or are they able to do
it through listening. Your child may be acting out to avoid difficult
situations of listening. And think about it, especially
in noisy environments, where the listening environment
is even more difficult. So just to give another visual here, so what if one day they
have poor detection. It might be giving us part of the picture. Or the next day, the
sound quality is poor, like the timing is off and then maybe it’s changing. There’s fluctuations in hearing. Or it’s becoming clearer, but in general, we need that clear signal to have a good picture and understanding. If you want more information
about language milestones, AG Bell has some handouts for parents which highlight the different
ages and stages of language. So under I think it’s the
teach header on the website, there’s a section family resources. And in there, there’s a
section called ages and stages, which is just nice to kind of see what a child should be
doing within a certain age. So what we know is there’s
an impaired auditory system. We know the brain needs a clear signal for learning spoken language. With cochlear implantation, we expect a year’s growth
within a year’s time. And as your child grows, we want his or hear
language to grow as well. Cochlear implants may improve that timing or that incoming auditory signal, resulting in improved speech recognition. So children with cochlear
implants and auditory neuropathy here at U of M perform
equivalent to children with sensorineural hearing loss when there are no additional disabilities. Things to watch for,
that might be indicators that your child does not
have a clear auditory signal, speech may not be understood
by others or strangers. You may be able to understand your child, but somebody, a new listener may say that it sounds like they’re
speaking a foreign language. Does your child always
want to watch your face during communication and
use lip reading for support, kind of telling us that they need more than just the auditory for understanding? They may comprehend single words but need support when
it’s a longer phrase. And their progress may be slower than expected with language. So all children with hearing loss have difficulty listening in noise. But something to think
about is your environment. And life is noisy and it’s
always going to be that way. But think about if they’re struggling or their behavior is acting out more when it is in a noisy
restaurant or the gymnasium and think keeping that in mind. Your placement may be being
close to your child’s ear, if it is noisy, that they can also see you to have that lip reading support. If you’re at home, think about
keeping the laundry room, the door shut or running
the dishwasher at nighttime if possible so that you can
kind of reduce the noise within your own environment. Additional supports, making
sure that they are able to see your face when
you’re talking with them, that the lighting is good. We have a lot of parents that use closed captioning on the television for older children so that they can read what is being said, as well as listen. So there are solutions for children with auditory neuropathy
to develop listening and spoken language. And therapy can help determine if that progress can be made
with their current hearing or if cochlear implantation
is recommended. And parenting isn’t easy. I think of these appointments
as information gathering to help you create what is
the best plan for your child. So hopefully we’ve answered
some more questions. But if you have any specific
ones, we can take those now. – [Brooks] Thank you, Kelly. Again, for those of you
watching a recorded version of this webinar, please send
your questions via email to me at [email protected] Our next webinar is going to
be “Auditory Verbal Therapy: The Who’s, What’s and Why’s,” presented by two of our
speech-language pathologists, Abaries Farhad and Ellen Thomas. I would like to thank Kelly and Caroline for taking the time to present today and for those of you logging on. We look forward to continuing
our series this summer and with that, we will
conclude today’s webinar. Thank you again for logging on, and I hope everyone has a great day. And we hope to see you
at our next webinar. (upbeat music)

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