Pathway to Cochlear Implants for Congenital Hearing Loss

[Dr. Gopi Shah]
What does the evaluation look like for these patients who are born with congenital hearing loss and who will ultimately end up getting a cochlear implant?

[Dr. Walter Kutz]
We've set up a really nice multidisciplinary team, which I think is essential, especially for children. They'll typically come in, they'll fill their newborn hearing screen, or they may come in later because of CMV or some other progressive hearing loss. I would say most of our kids come in, they're six months of age, been identified, or even three months of age. Then we typically have two meetings.

It's a multidisciplinary clinic. They'll come in, they'll meet the otologist, which would be myself or three other of the otologists in the practice. We always have the patient see Dr. St. John because of her input. I think it's a good checks and balances to make sure we've done all the testing. We want to do imaging. Typically, we get an MRI. The only time I get a CT scan is if there's some inner ear abnormalities. I'm worried about a facial nerve or something of that sort.

Then we'll have them see ophthalmology, do genetic testing, we'll get an EKG, and that's going to be our typical workup. I think by having seen Dr. St. John in our clinic a couple of times, we make sure to get all that covered.

We're also very careful these kids get vaccinated. They need to have their Prevnar 13, which is part of the normal vaccines until two years of age. When they're two years of age, they'll get a Pneumovax as well because with a cochlear implant, these kids have a higher risk of meningitis.

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Then at the meeting, it's going to be an otologist, a cochlear implant audiologist, we have a psychologist to meet with the family, a social worker. The team will generally meet with the patient twice.

One, we want to make sure the patient is a good cochlear implant candidate. We want to make sure they're going to-- The real work of the cochlear implant journey starts after the surgery. That's when the kids have to wear the implant, the parents have to be supportive. A lot of these young kids are just saying, "I don't want to wear it." That first week or two, the parent has to make sure the child's wearing the cochlear implant all the time.

We want to make sure they understand some of those issues. There could be behavioral issues, a child could have autism, they may have some other severe neurologic problems that may not be a great cochlear implant, they're just not going to be able to do anything with that input. It can be very complex decision-making. That's how we do it. Two clinic visits with our multidisciplinary team, and it seems to work pretty well.

[Dr. Gopi Shah]
What's the average age of implant for most patients?

[Dr. Walter Kutz]
We try to get them implanted by one year of age if they're Medicaid. If they have private insurance, we'll try to get them even sooner, 9 to 12 months of age. That doesn't always happen, unfortunately. There's some socioeconomic issues that come up, but we do the best we can. The earlier you can implant a child with severe-to-profound bilateral hearing loss, the better they're going to do.

Once they get over three years of age, I think their potential for being an excellent cochlear implant user really starts to decline. Average age, hopefully, we implant them by one year of age. I'm sure it's a little older than that, but that's what we shoot for.

[Dr. Rachel St. John]
One thing we've been doing with CI team, which I have found incredibly helpful, and I hope Dr. Kutz has as well, is that we do a monthly multidisciplinary team meeting by-- well, now by Zoom with COVID, which is I think made it a lot easier, honestly, in terms of scheduling. That's where we actually look at cases that are particularly more complicated, whether it's ethical, psychosocial, medical, all of the above.

We've had a case of a child who had unilateral sensorineural hearing loss. She actually had access to language, but she was deaf in one ear, and mom was really looking for a cochlear implant, and helping her understand that that wasn't going to change that child's access to language because she had the one ear that typically, that wasn't necessarily going to change the child's trajectory.

Having a multidisciplinary meeting where we all reviewed the case and reviewed what had been done and reviewed what mom had been coming to the team with, was really helpful, or families that have been declined elsewhere and are seeking a second opinion. I think I found that to be incredibly valuable, both in helping families with appropriate expectations. Then also, sometimes we will talk about things and say, "Okay, do we have a policy when we have a particular situation like this? How do we tend to handle it? What's our database to norm?" Sometimes it's easy and sometimes it's stuff we haven't seen before. It's a first time. I think that's been a really helpful part of the CI multidisciplinary process.

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I think it's very important for families to know that it can look just like YouTube where they turn it on and the baby lights up like a Christmas tree and everybody cries and laughs and claps. It's great. It can also look, the baby can be absolutely petrified and break down or just get really quiet and scared. Those are all typical responses. They are all expected. That first time that a baby hears sound, we don't know what they're going to do.

If parents don't know that and they have watched all the pretty YouTube-- because the babies crying when the cochlear implant is turned on, those don't make it to YouTube. You are not going to find those, but they're very important. That's a very normal expected response for a child who's experiencing something completely new for the first time. For families to know that it's okay if the baby freaks out a little bit, that little thing is so important.

I've had parents come to me with cochlear implants in a bag right after they've been activated and said the baby gets so upset. I'm like, "Okay, let's put one on and we're going to cry. It's going to happen. Then we're going to just keep talking, and then we're going to let the baby get used to it. Then we're going to be okay. Then we'll put the other one on. The baby's probably going to cry again." There's a process there.

I think a lot of what I do is helping families understand what is the range of outcomes. Cochlear implants have never been better. The technology has never been better. The surgical technique is continuing to evolve. It doesn't mean it's going to be hearing like the parents have. We know that. It's not a bionic ear. We might get there one day, but we're not there yet. Understanding that children are all unique, and so there's a range of potential outcomes.

We're shooting for access to listening and spoken language. Depending on the risk factors involved, we expect to get there, but we also have to be prepared for the fact that that may not happen. It's not to be doom and gloom with families, but just to make sure that they don't have this expectation that we're going to put this thing on and we're going to turn it on and everything's fine.

When we talked about this on the last episode, you have a deaf child and they will always be a deaf child no matter what we do. Helping families embrace that and helping their child embrace that, that's my job. Getting deaf role models involved, hooking parents up with family support programs like Texas Hands & Voices, which is just a bunch of other parents with deaf and hard of hearing kids.