Sep 28, 2020


Interviewer: So, today, we're here with Dr. John C. Carey. He's an MD and professor of pediatrics at the University of Utah Health and Primary Children's Hospital. So when we're talking about hearing loss in children, you know, when we're talking with newborns, you were telling me that there was a bit of this screening process that we do, what's it called?

Dr. Carey: So it's called universal newborn hearing screening. It was initiated in Utah in 1999, which was one of the first states to actually pass the legislation to screen all of our newborns. But by the mid part of the next decade, the mid-2000s, all 50 states had mandated that their health departments had to provide newborn screening so that we could detect early on, literally first few days of life, that that particular newborn had a hearing loss.

Interviewer: So, I guess, how frequently are we testing? Are we doing like, you know, the first week, the first month? I've heard 1-3-6.

Dr. Carey: In testing for all the babies that are born in the hospital, which in Utah is about 99%, the hearing screen is done before they're discharged. So it's done by one, two, three, four days of age. If they fail either ear, they get a second screen. That's usually done a week or so later. That's the one of the 1-3-6. We want to make sure that second screen is done before one month of age. And then from that, it turns out that the majority of those children who fail the first screen, don't have a hearing loss. There's something plugging their ear, like, you know, the ear wax of the newborn and so on.

So when they get their second screen, that really narrows things down to now we have a few percent of babies. And then when they're seen by three months by the audiology specialist, by the actual hearing specialist, we can determine whether or not they have what is called a permanent hearing loss or not. If there's fluid in the middle ear, it's not considered permanent because our ENT colleagues can drain that fluid with tubes. And eventually that, with time, can resolve itself. So that's not considered permanent. What's permanent is when it's not due to fluid and they still fail one or both ears, and then that's confirmed by the audiologist before three months.

Interviewer: And then you bring them in one more time at the six-month mark?

Dr. Carey: And then, well, by six months, we're hoping that all of those children who are determined to have permanent hearing loss of some degree could be mild, it could be more, but by six months all will have their hearing aids and also be, if they need them, and also be lined up for early education of children with hearing loss. So, by six months, we want that to be in motion.

Interviewer: And how many kids do you find are affected by these types of conditions?

Dr. Carey: After you go through the screening first time, second time, get the test by the audiologist, you're left at about 1 in 500 newborns. Now, there are a few weeks or months old. One in 500, which would be about 100 children a year in Utah, have a permanent hearing loss. After heart defects, hearing loss is the most common birth defect in human babies.

Interviewer: Wow.

Dr. Carey: So everything else you can think about from oral facial clefts or some of the orthopedic abnormalities or other syndromes, like Down syndrome, they all have a frequency of less than 1 in 500.

Interviewer: And what does the screening for a baby look like? Do you put little headphones on them? What does that look like?

Dr. Carey: You actually do put something in their ears. In the first screening, you put something in their ears, and this almost sounds magical, but what they're doing is they're putting a sound wave in through the ear canal. It goes through the eardrum and the three famous middle ear bones, the smallest bones in the body, through the oval window to the hearing structure called the cochlea. And so, when it gets to the cochlea, the hair cells inside, people are familiar with all that with maybe a hair cell problem, get to the hair cell, the hair cells bounce the sound wave back, and the computer detects that change.

So what you're doing, in Utah, what we're doing . . . other states do something different. Most states do what we do, which is to test cochlear, the inner hair cell function by bouncing these sound waves through, the hair cells react, bounce it back, and the computer at different frequencies, at different loudness records whether or not the cochlea is working properly.

Interviewer: Wow.

Dr. Carey: So if the hearing loss is due purely to the nerve and not necessarily the hearing nerve connected to the cochlea, but purely to the nerve, a child will pass the hearing screen much of the time. So the limitation of our hearing screening, which most states do, this is called an otoacoustic emission. You can see the words, oto, ear, acoustic, the sound. Emission is the hair cells bouncing it back, you know. Most states do otoacoustic emission.

Interviewer: So for one of these parents whose children are identified with one of these hearing issues, what kind of services can the Utah Hearing Center provide?

Dr. Carey: The beneficial aspect of early detection is that you actually get the kids in for treatment and proper education. And the majority of those children will go to a regular class in a regular school. Those that have the most profound degrees of hearing loss, that don't necessarily respond to hearing aids, can go to our outstanding special schools we have throughout the state. They're the schools for children with hearing loss and visual disabilities. They still do say deaf and blind. So those schools have their branches of them throughout the state. They provide for the children that have some of the more moderate to severe, but especially those that have the profound degree that if untreated with something like a cochlear implant, those children would go on to be called deaf.

So deafness is the degree of hearing loss preventing regular communication as you and I would have it in this type of setting so that one would have to learn sign language and then be a part of a very rich community of people with deafness. But on the other hand, would have more challenges with communicating with the rest of society. That particular place we're talking about, that particular situation, just, you know, in the last two decades, in half of my career here at Primary Children's can now be altered dramatically with a cochlear implant so that children with a cochlear implant will usually, with the right rehabilitation and such, will actually go to regular kindergarten.

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