ROUGH EDITED COPY

EHDI

BECKHAM

AUDIOLOGY FOR PARENTS AND CLINICIANS: DIAGNOSTICS

PRESENTER: DONALD GOLDBERG

3/9/15

2:30-3:00 P.M. ET

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(This text is being provided in a rough draft format. Communication Access Realtime Translation (CART) is provided in order to facilitate communication accessibility and may not be a totally verbatim record of the proceedings.)

> All right, our next presentation is titled Audiology For Parents and Clinicians: Diagnostics presented by Donald Goldberg.

If you have those pinkish-orange forms, I'll be collecting them or you can leave them in your chair. I'll just pick them up afterwards. That's totally fine too, okay.

> DONALD GOLDBERG: Okay, in the interest of time, actually for advancing is it -- sir?

[ speaker is off microphone ]

> DONALD GOLDBERG: Sounds good. Okay, in the interest of time I'm just going to get going, and my first slide asks -- although it doesn't move. Enter does not work.

He ran out so fast. Have him come run back in. Or...

All right, now we're going. You can stay around. This is not -- raise your hand, but are there any audiologists in the room? I ask that you leave. No, just kidding.

But you really don't need to be here. But you've got an idea how I would talk about the audiogram. Parents in the audience? Great.

Speech pathologists? Teachers of the hearing impaired?

We have a slew of audiologists. It's very slow to respond.

CertABTsand classroom teachers? Et cetera, et cetera.

My premise of talking about audiology and the audiogram and there is another session in another room at 2:30, is we need excellence in audiology for all of the work that we're doing if you're an auditory based program. In fact, it's the foundation of our auditory teaching. And needless to say after hearing Carol Flexer probably in other sessions it's important people walk away with the idea that regardless of the degree of hearing loss, parents and intervention, they can and are making use of auditory information for the development of spoken language. One of the reasons why today, being an audiologist, being an interventionist, has never been a better time because we are seeing kids so young. At this point I probably have started at least six kids who are two months old whereas years ago the average age of identification of a severe to profound hearing loss was two and a half to three years, much less a child coming fitted with hearing aids at two months. I think it's also important to keep in mind that because of great advances in medical technology, son-in-law to have children we're working -- some of the children we're working with have other additional disabilities but those I find to be the greatest fighters and maybe the most interesting patients to teach us how to be a better clinician and counselor and there's no question, I think all of us are more inclined to be very attentive to evidence-based research and clinical effectiveness, measurements of our kids, and there's no getting around cochlear implants have changed things completely for what we're doing. So the sky is the limit for children with hearing loss and it's because of these things, including excellence in audiology and hopefully good intervention included by individuals who have certification in listening and spoken language, the sky is the limit for these children. But I'll still come back to the mantra that audiology and excellence in audiology is the key to our auditory teaching. Without it, one would really raise the question of doing an auditory-based approach.

So how many of you are -- thinking back, most of you are professionals. How many of you can raise your hand and say hearing science was your favorite class in graduate school. Speech science? We know one person in this room is like my wife, a scientist, very smart, and that's why you have speech and hearing science love. But I couldn't stand it. And now I have to teach it. So now that I have to teach it, what better way to do it than try to think about its clinical relevance into audiology, and I'm sure you all are aware that sound is based on the vibration of air molecules and it has to come about by some force, and so this is physics 101, and when, in fact, you're talking about sound, you want to stipulate what the matter is. For speech, typically it's in air but it is possible to have sound and other acoustic stimulation and other mediums. But the real key is sound has to do with vibration. This happens to be a website on the bottom that you can take a look at, and some of these -- hopefully with the lights still bright are going to be sort of vibrating and uniquely moving slides if everything works well. You all know about a tuning fork. A tuning fork, oftentimes you get a tuning fork and look at the size and number. It's set up to be 125 or 250 or 500-hertz. And when you bang it on your heel, the tuning fork tines begin to vibrate. And you learn words like condensation and rarefaction, what does it mean? It comes down to when you analyze sound, that tuning fork is like a sine wave, pure tone, and as it vibrates air molecules come close and they come apart. This is another example of the movement of sound and, in fact, the condensation where it's all the molecules close together on the top right and rarefaction where they're moving apart. And another way to represent it with these waveforms is what you see on the bottom. So, you know, this would have been much more fun if somebody was banging tuning forks and talking about what it did in the air and then relate it to an audiogram. And this is your classic sine wave. It's a pure tone like 125-hertz on an audiometer or 500-hertz and you have these beautiful up-and-down pattern of condensation and rarefaction, and the way you measure, as I'm sure you recall, is in the number of cycles in a second has to do with pitch or the frequency. So literally years ago, because I'm old, we talked about 1,000-hertz. We used to call it 1000 cycles per second. So literally in one second that tuning fork would have vibrated 1,000 times. A high pitch, even more vibrations and more and more cycles up and down, a low frequency stimuli would be a much more rounded waveform because in one cycle or in one second you can calculate how many cycles per second and figure out also with your math formula, because of the inverse relationship of frequency and pitch. So getting to the audiogram in just a moment, we talk about hertz. I never like to end a semester when I say, how does a person hear from 20 to 20,000 dB if they haven't at the end of the semester realized they mean 20 to 20,000-hertz, because if it's 20 to 20,000 dB you would blow up a person's ear. It's essentially important that you don't walk away not realizing that frequency and pitch are in hertz and it has to do with the psychological attribute of the pitch. High pitch and low pitch. And there's the math formula that I'm sure you enjoyed in physics.

And then the other component when you think the basics of an audiogram is the amplitude. How high up and how high down or peak-to-peak amplitude is reflecting the intensity or the strength of the sound, the loudness of the sound. So if it's just up and down on that horizontal red line it's not very intense and if it's much more elaborate peak-to-peak, you would have, quote, a louder sound. And then we also talk about phase as we talk about physics.

So with amplitude it's the amount of displacement from baseline. It's perceived as loudness. And there are lots of different ways to measure it and we refer to it -- the reference is in decibels. But you probably also remember that decibels are logarithmic and the only way we would ever fit it all on an audiogram is by talking about the decibel and we convert it and name it dB with capital B after Alexander Graham Bell and there are three different types of decibels we usually talk about. The decibel in an audiogram is in hearing level, but it really -- the pure measurement of decibel is in sound pressure level or the amount of power in the particular space. So with decibels, some people do talk about intensity level, but the real issue is on a sound level meter, measure how loud the sound is in this room, that would be a reading on dB SPL and as soon as we look at an audiogram, we're converting it to a dB HL and then you also probably remember there's also an SL that has to do with the relationship of a sound to the threshold. So you do SRT measurements and get this particular number and then anything above that SRT would be how many dB SL.

This is one of my favorite graphs, because it's measured off hundreds of college age males and they did this measurement and it was measured in sound pressure level and they were then able to convert the relationship at each different frequency of the relationship of SPL to HL. So you really see very distinct differences across the frequency range how much power is in the low frequencies versus how much power in the mid frequencies and high frequencies.

So let's talk briefly about hearing and a little bit about audiogram and audiogram testing. The beautiful ear, most of us think about the outer here, the external auditory medias moving in, obviously an eardrum, three ossicles and the organ of hearing called the cochlea. But the cool thing in the cochlea -- brace yourselves -- is those hair cells. So you probably remember that hair cells are the sheering action. There's this chemical-electrical change that occurs at the hair cell level. Well, if you look carefully and if the lights were out, those are moving outer hair cells, which I sure think is pretty cool.

Anyway, then, of course, you also know from this morning's talk, it starts at the ear. That's just the entry way, but it's going to make it to the brain. So if you look over to the right, the classic three outer hair cell rows on the right. You have one inner hair cell in that triangle. And watch the electrical firing starting at the cochlea and then going up the brainstem to eventually -- whoops -- to eventually the gyrus in the temporal lobe of the auditory cortex. You have to look carefully. It's about to fire. And it crosses over and goes to the auditory complex. We do listen with our brain.

Then another thing that you all learned and you probably spell correctly now, tonotopic representation of the ear. I'm sure I learned that was a definition of something about tuning, but no one ever told me that, look at that beautiful cochlea! And it's tuned at the base end on the right. I wish I had a pointer, but the base end where it says 20,000-hertz, that section in the base is fine-tuned for high frequencies and as you go through the two and a half or two and three quarters of the cochlea, you'll eventually get to the apex for low frequency hearing. How do you think the left cochlea is doing? Not too well because there are no hair cells on the cochlea on the left. Probably needs a cochlear implant. But the beauty of the cochlea and the whole idea that with the insertion of electrical arrays, why wouldn't kids hear a sss and shhh with great finesse because you're getting wonderful stimulation in the basal end of the cochlea and certainly kids with implants, after an implant, rarely have difficulty. So, of course, the little boy that you just saw wasn't responding to sss. Kind of be curious if he responded to S-H, shh, but with an implant I'm imagine he'll hear those with great ease in distance and norms. All of the enthusiasm of testing early, I do want to make a caution, certainly a supporter and you can't come to EHDI and not support universal newborn hearing screenings, but I think it's very important that we realize not every kid is tested. Some kids are not born in hospitals. Isolated kids escape and don't have the testing done. In addition, some kids in various states are born at home and with midwives. One of the presentations I think earlier this afternoon was teaching midwives how to do universal newborn hearing screenings.

You should be aware that we have lots of undocumented individuals in the United States. What happens to the follow-up of those children who might be born in a hospital but don't make it back and are a continued problem of about half of our kids not coming back for the follow-up. In addition, in the United States, the majority of states use OAE testing. OAE measures our response of the cochlea. It is not a response beyond the cochlea. So because of time and money, many programs are doing OAEsin the absence of a two-stage program with OAEsand ABR, we will be missing many kids with something called auditory neuropathy spectrum disorder where you can have the presence of OAEsand absent or abnormal ABR responses.

So universal newborn hearing screening is a wonderful thing, but when families are told pass or everything is fine at 24 hours, it does not remove the importance that all of us have to remember about speech and language milestones and the ever-vigilant and the next time a physician says to a parent, you're a worrisome mother or the classic, he's a boy, so they're slow, or he has an older sister and she talks for the kid, all we have to do is teach physicians to eventually say, if you're concerned about hearing, go see a pediatric audiologist. Today we are still seeing kids who have been missed and parents not heard. And needless to say, there are some children that develop disease processes where they're fine at birth and then in fact lose their hearing. So we have to be very careful to not just keep resonating pass, pass, pass, when there are other indications that the child is not hearing.

Moving on to hearing testing. Acronym soup. We have AVRsand we have AAVRsand OAEs, but we also have behavioral testing and I skipped over ASSR auditory steady-state response. And, of course, the classic behavioral measures of BOA, VRA and CPA. This is just obviously one of the sponsors of the conference. And ABR, this is a measure of OAEs. And then we have a child who hopefully has tympanometry before other physiological measures. Hopefully we get ABR with clicks, and we also can do ABRswith clicks, bone conduction, but we also need to do tone specific ABR, because the problem with click stimuli is it's primarily 2,000 to 4,000-hertz, most of our kids can't hear and it might be possible that if we did click of 250, we might see many of those kids have some hearing in different frequency ranges. So it's not uncommon for a comprehensive program to do click as well as frequency specific ABR measure to catch a happy baby.

We have children watching silly people with suction cups on their head and sound comes out of the speaker and it scares the heck out of the child. Sometimes they cry. And I don't know about you, but I'm pretty sure I don't see any headphone on that kid. The challenge with behavioral measures is we are not getting ear specific data. Even if it comes out of the left speaker, you are not testing the left ear. It does get to the left and to the right ear. And should an ear difference exist, we will not know. So then we also have boxes that light up and monkeys that bang things and children who cry when that happens and is worse than trying to continue testing with a screaming child. And because the lights are so exciting, you have wobbly heads. Whack, whack, whack!

And then you have to give a guess, was that because they wanted it to move or they just happened to move when the sound came out? So unless you see Jane Midell, VA testing is not that easy. You need a team of testing but you can only get so much information. In the absence of specific information, we're not getting enough, send them for electrophysiology if are required. Then we have the cooperative child who will hold the ring for 474 more presentations and give us a right ear/left ear audiogram and just want to do even more. But some children are not cooperative.

In the world of audiological testing, before we see the audiograms we obviously need to make sure ideally that you have audiology in your intervention program. No child at the Cleveland clinic can be tested unless we have a second audiologist. In fact, the person in the booth may be more important than the person pushing the buttons and the coordination between the tester on the machine and the person inside, and collaboration about your observation. Parents should be participants, protect their ears if it's getting loud, because why create a hearing loss in the parent? And make sure the parents knees don't move every time the sound comes on. They do need to be counseled and we need comprehensive testing.