Strategies to Support Children Living with Hearing Loss

[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.

[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.