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Strategies to Support Children Living with Hearing Loss

Author Quynh-Anh Dang covers Strategies to Support Children Living with Hearing Loss on BackTable ENT

Quynh-Anh Dang • Aug 23, 2021 • 100 hits

Pediatrician and hearing loss specialist Dr. St. John joins us to discuss strategies to support children living with hearing loss. There are important counseling considerations throughout a child’s life, starting from early language development, through schooling years, and into adolescence. Overall, she emphasizes the importance of providing full information to the family and respecting the child’s individual decisions.

We’ve provided the highlight reel in this article, but you can listen to the full podcast below.

The BackTable ENT Brief

• Dr. St. John advocates for early language exposure through sign language, which can help children cement neural pathways and prepare them for future oral language development.

• Mild hearing loss in children is often misdiagnosed as attention or behavioral problems at school. Children might struggle with hearing and absorbing new content, which could discourage them from speaking up in class and performing well in school.

• Dr. St. John offers advice for counseling pediatric patients who are starting to wear hearing aids. Since there may be some initial social discomfort, she focuses on presenting their long term academic benefits.

• A patient with an enlarged vestibular apparatus faces a higher risk of hearing loss after significant head trauma or barotrauma. Dr. St. John warns patients to be cautious when playing contact sports, but she also believes that they should individually weigh the risks and benefits of participating.

Sign language can be an important step towards oral language development in children living with hearing loss

Table of Contents

(1) Sign Language and Oral Language Development

(2) Mild Hearing Loss in Children and School Performance

(3) Managing Enlarged Vestibular Aqueduct

Sign Language and Oral Language Development

Children living with hearing loss can benefit from learning nonverbal modes of communication. Since the critical period for language development occurs in between ages zero to four, sign language can serve as an early introduction to thought concepts, which a child can use later during oral language development. According to Dr. St. John, the early formation of thought concepts is more important than the actual mode of communication. Additionally, she does not believe that exposure to different modes is detrimental to oral language development.

[Dr. Ashley Agan]
How does sign language come into your treatment plan? Do you help families connect with resources for using sign language?

[Dr. Rachel St. John]
The answer is a resounding yes. The most important thing to me, as a pediatrician and as a developmentalist, is early language access. A small but growing body of data that looks at deaf kids and language emergence in both spoken and sign language. Recent data points to early language acquisition being the cornerstone for language development, not the mode.

There are some people who really cling to, "If you want to develop spoken language, you can only do spoken language. If you do sign language, it's going to somehow detract from that." Well, it's not what the data shows. It's certainly not what my last couple of decades of experience has shown me. The much more concerning thing is that, unfortunately, I see kids who don't get early language access. They missed a lot of that critical language window. They end up with early language deprivation, and they can’t catch up with the other kids.

[Dr. Gopi Shah]
What exactly does language access mean?

[Dr. Rachel St. John]
So recently, I heard something from a speech pathologist. She said, "Remember that speech articulation is an oral motor, but language originates in the brain." Speech and language are not the same thing. Language is the thought construct that we develop as humans to generate a concept. And then we share that concept through a mode, whether it's spoken language, visual sign language, or ProTactile, so that would be sign language by touch for people who are deaf-blind.

The data says that the thought concept is the building block, and mode is irrelevant. We have a lot of good data that shows if you expose kids to sign language, it doesn't impede the development of their spoken language as they move on. In the past 20 years, I've only seen one study come out that overtly says, "Sign language is harmful to the spoken language development of kids," and it's been hotly contested in the community.

With the idea of too many languages, I've only seen that be a problem when we have a kid who's not neurotypical, like autism, where there is cognitive stress of several different languages in early childhood. But most of the time, if you have a child where the parents speak English, the nanny speaks French, they do Spanish at school, and then they do sign language with their friends, they're going to develop all of them. There's a lot of code switching in the first few years, but as a whole, these kids are not delayed. Usually by about age four or five, they've ironed out when to use which language.

[Dr. Ashley Agan]
What is the window?

[Dr. Rachel St. John]
It's debatable. From what I have seen in terms of brain myelination, neuroplasticity, and temporal pruning, the human brain is fully myelinated around age four. That's when there is a lot of flexibility, in terms of "If I have a pathway and I use it, I'm going to cement it." If you don't have good language access and you don’t have a first language in place by age four, you're going to have some sort of neurological stiffness in terms of acquiring a first language beyond that. But also, in that first year, where you've got billions of excess brain cells, it’s also a critical moment. It's a big use-it-or-lose-it moment.

For example, if you're born completely deaf, and you're waiting for a cochlear implant, and the earliest that that's going to be covered by third-party insurance is six months. And the earliest that that's going to be covered by Medicaid is twelve months. So if you're going to sit around for those first six to twelve months and do nothing linguistically, that child will fall behind. Whereas if you start with sign language, which is usually available, you're starting to build the thought concepts, so when that sound input eventually starts coming, you have something to link it with.

Listen to the Full Podcast

Comprehensive Care For Deaf and Hard of Hearing Children with Dr. Rachel St. John on the BackTable ENT Podcast)
Ep 10 Comprehensive Care For Deaf and Hard of Hearing Children with Dr. Rachel St. John
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Mild Hearing Loss in Children and School Performance

For children living with hearing loss, the academic environment can present challenges to learning and retaining new concepts. While children with mild hearing loss might be able to socialize normally, they can struggle in the classroom, causing teachers to mistakenly identify behavior or attention problems. Dr. St. John describes her strategies for counseling pediatric patients about the long term benefits of hearing aids.

[Dr. Gopi Shah]
Do you change the way you manage your patients if they have unilateral or mild hearing loss?

[Dr. Rachel St. John]
I see the literature often will describe this as MMHL, which is minimal or mild hearing loss. These kids, who are deaf in one ear or who have sort of mild hearing changes, all function socially quite well. They all develop language, but their access to language varies wildly depending on what environment they're in. They'll often do well when they're at home, it's quiet, there's one-on-one interaction, and they're able to see people's faces. They could do worse when they're in school, where there's lots of competing background noise, and the teacher could be talking about new concepts, so they don't even have anything to draw on if they have to fill gaps in cognitively.

These kids are definitely at higher risk of having to repeat a grade. They have a higher usage of special education plans. Unfortunately for these kids, because they are socially functioning well, hearing issues often present as behavioral issues. It looks like inability to focus or being just defiant. But a lot of times, it's kids trying to compensate. You'll have kids who are the class clown because they're deflecting, since they don't want to get called on. You'll have a kid who will try to close to the best of their ability with the 70% of information they got, and then put out the wrong answer. You'll have kids who are deathly afraid to participate, because they're not working with the full amount of information that everybody else is being given.

Looking at a visual audiogram with those families is incredibly important, because it's really important for those families to acknowledge that, "Yes, your kid hears tons. But here's where things are difficult for them." The counseling that we do, even for mild or single-sided, is pretty significant. And a lot of it revolves around making sure that school is accessible.

[Dr. Ashley Agan]
Do you find that you have resistance or any kids who just don't want to wear hearing aids?

[Dr. Rachel St. John]
Yeah, it happens all the time. To the teenager who doesn't want to wear their hearing aids because they look different from their friends, the first thing I say is, "That's normal. It's okay to feel that way.” But my next discussion is, " Here's what's going to happen if you don't wear them. And because you're as old as you are, all these consequences are yours.” I talk to them about how hearing aids can help them in high school and help them eventually reach their goal of going to college.

Sometimes these kids’ concerns just need to be acknowledged. I think normalizing this discomfort is really helpful for these kids. It's also really helpful when I show them mine. I'll take them off and show them that I'm wearing them. Sometimes they didn't even notice.

Managing Enlarged Vestibular Aqueduct

The causes of enlarged vestibular aqueduct (EVA) remains unknown, but EVA has been associated with childhood hearing loss. Drops in hearing have been documented after situations involving head trauma and barotrauma. Dr. St. John shares her advice to parents about balancing activity limitations with the child’s individual decision making.

[Dr. Rachel St. John]
This is a Pandora's box, and it is pretty rare in the general population, but in our clinic, there are a large number of kids with enlarged vestibular aqueducts. It’s interesting because I've seen that mixed presentation referred to as a pseudo-conductive component. It's most characterized by a third window situation with the bony involvement with an enlarged vestibular aqueduct. When I see mixed and particularly late-onset hearing changes, one of the first things I think about is EVA. And I usually move to imaging.

[Dr. Gopi Shah]
Do you usually order CT, or give the family the option? With CT, we think of radiation. With MRI, we think of sedation, depending on the age and the child's ability.

[Dr. Rachel St. John]
I've talked to different providers about what they prefer for seeing different things. I've heard people say, "MRI is better for EVA." I've heard people say, "No, a CT is better for EVA." I think when kids have almost primarily or exclusively a conductive component, CT is more helpful because you are thinking about things like bony involvement or ossicular chain. However, I think a lot of it depends on the age of the child and what you're thinking about in terms of sedation versus radiation, and the length of the procedure.

Also, is there another hearing test that needs to be combined with those things? We often will combine sedated ABRs with MRI.

[Dr. Gopi Shah]
In terms of EVA, and again, how do you counsel families in terms of activities? We're always taught that with EVA you have to avoid head trauma and significant barotrauma because those can make the hearing worse. Do you tell them to have activity restrictions and head protection?

[Dr. Rachel St. John]
If you look at the literature, the exact underlying mechanism for the hearing change associated with an enlarged vestibular aqueduct is unknown. It’s not the EVA that is causing the hearing to drop. The EVA is a radiologic marker for an underlying process, potentially at the cellular level. In terms of counseling, we do know that there are some situations that tend to predispose EVA children to faster or more abrupt drops in hearing. Those situations tend to be significant trauma to the head or significant changes in barometric pressure.

In terms of sports, I always tell families, "Your kid's not in a bubble. You do not need to wrap them in plastic and put them in the attic. That is not the whole point of being a person. They can go do sports." I think the sports that have the highest risk, because you're really thinking about head contact, particularly head to head contact. The really big ones are football, rugby, and soccer. Other stuff tends to be more incidental. "Yeah, you could knock heads with somebody playing basketball. It's not supposed to happen, and if it happens, it's an accident." I really try not to get parents super anxious about this. I firmly believe these kids need to be active, because certainly being sedentary and overweight is a much bigger issue.

And then in terms of barometric changes, people ask me about planes. Usually, I encourage them to go ahead. The cabin is pressurized. The other thing I really will counsel high school students and young adults about is scuba diving, because some schools will offer it for credit.

I want to make sure that the patient is involved with their own decision making. EVA is there for a lifetime. For example, I had a patient who was being recruited for college baseball. We talked about the potential risks if he ever took a ball to the head at fast velocity. He was able to articulate his decision and he said, "I am okay with the fact that I might have a drop in hearing due to a freak incident. It's more important for me to keep this part of my life and pursue this." I supported him a hundred percent, because he made that choice as a young adult.

Decision-making should involve the emerging adult. It's not about avoiding everything that could possibly drop their hearing.

Additional resources:

Podcast Contributors

Dr. Rachel St. John discusses Comprehensive Care For Deaf and Hard of Hearing Children on the BackTable 10 Podcast

Dr. Rachel St. John

Dr. Rachel St. John is a practicing ENT and Associate Professor of Clinical Medicine at Children's Medical Center Dallas/UTSW Department of Otolaryngology.

Dr. Ashley Agan discusses Comprehensive Care For Deaf and Hard of Hearing Children on the BackTable 10 Podcast

Dr. Ashley Agan

Dr. Ashley Agan is a practicing ENT and assistant professor at UT Southwestern Medical Center in Dallas, TX.

Dr. Gopi Shah discusses Comprehensive Care For Deaf and Hard of Hearing Children on the BackTable 10 Podcast

Dr. Gopi Shah

Dr. Gopi Shah is a practicing ENT at UT Southwestern Medical Center in Dallas, TX.

Cite This Podcast

BackTable, LLC (Producer). (2020, November 24). Ep. 10 – Comprehensive Care For Deaf and Hard of Hearing Children [Audio podcast]. Retrieved from

Disclaimer: The Materials available on are for informational and educational purposes only and are not a substitute for the professional judgment of a healthcare professional in diagnosing and treating patients. The opinions expressed by participants of the BackTable Podcast belong solely to the participants, and do not necessarily reflect the views of BackTable.



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