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Pediatric Cochlear Implants: Candidacy, Diagnosis, & Mapping

Author Wasiq Nadeem covers Pediatric Cochlear Implants: Candidacy, Diagnosis, & Mapping on BackTable ENT

Wasiq Nadeem • Sep 2, 2021 • 39 hits

Pediatric Cochlear Implants are an important tool in the treatment of hearing loss refractory to other therapies. Screening and diagnostic timelines for children with cochlear implants have been evolving over time, with imaging and testing for infectious or genetic etiologies being essential in the workup. Dr. Prashant Malhotra explains his diagnostic steps on the BackTable ENT Podcast with emphasis on proper cochlear implant mapping, which should occur early and regularly by a multidisciplinary team to form a consistent and cohesive message for the patient and their families.

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

The BackTable ENT Brief

• Pediatric cochlear implant candidacy should be evaluated as early as possible, with appropriate newborn screening followed by coordinated visits to appropriate specialists with a thorough workup to establish a diagnosis.

• Diagnostic tests that Dr. Malhotra considers important in pediatric cochlear implant candidates include imaging with CT or MRI and additional testing for CMV, connexin, and genetic sequencing.

• Regular follow up appointments are important for cochlear implant mapping to keep up with device changes and patient needs. Dr. Malhotra begins initially every 6 months, followed by once a year for follow-up appointments.

Patient with Pediatric Cochlear Implant

Table of Contents

(1) Pediatric Cochlear Implant Candidacy

(2) Pediatric Cochlear Implants: Diagnostic Workup

(3) Cochlear Implant Mapping in Children

Pediatric Cochlear Implant Candidacy

Dr. Malhotra discusses the importance of early hearing loss detection and intervention, which has been improving overtime due to newborn screenings and systems in place to identify pediatric cochlear implant candidates. He underlines the importance of setting up appointments with all members of the multidisciplinary team that will be involved and to make those roles and expectations clear for children with cochlear implants and families with a consistently cohesive message.

[Dr. Prashant Malhotra]
Our goal is to see them right upon identification of hearing loss. We're not just a pediatric implant center. We actually manage kids with hearing losses of all types.

[Dr. Gopi Shah]
For the pediatric cochlear implant population, what percentage is your failed newborn hearing screen? And then what percentage is your delayed onset kids who are over the age of two? I guess your two categories would be prelingual and postlingual?

[Dr. Prashant Malhotra]
I think that earlier on, about eight years ago, we were capturing a lot of the older kids because there was a backlog of their cases. It was probably 80% postlingual, late ID patients. Now, I would say that we are seeing more young babies and kids, so the split is about 50%.

We still see with etiologies like congenital CMV and other progressive losses as we've implemented targeted and then universal screening for CMV in our NICUs and trying to extend them to the surrounding areas. We're capturing those kids and monitoring them very carefully, and then following them when they become implantable later on. But I would still say, we're probably 50% of the babies at this point.

[Dr. Ashley Agan]
What does the appointment look like? First, we can talk about the patients who were diagnosed at birth. So they've failed their newborn hearing screen and they're referred to you to have additional testing. What can parents expect as they go through that process, up to the point when they're actually being recommended for a cochlear implant?

[Dr. Prashant Malhotra]
After diagnosis, which may be done at an outside facility, they'll come to us. Actually, we have a pretty wide catchment area, so we might capture them at first. A very consistent message from the very beginning is incredibly valuable. We've got good relationships with audiologists, and they are pretty well aware of when patients are coming to cochlear implant category level. After that first diagnostic ABR is complete, audiologists may say, "Hey, look, we need to set up an ENT appointment and a follow-up audiology appointment." They'll kind of lay that groundwork.

At that first ENT appointment with me, I view these counseling appointments as super essential. I take the appropriate amount of time to do it, and I tell this to the residents and the fellows as well. I honestly believe that if we don't take the time to do it, nobody else does. I don't think that a lot of general ENTs feel very comfortable with the pediatric cochlear implant counseling pathway. They might not want to put words in the mouth of the program that they refer to.

For some families I'm going to do a quicker play. But sometimes, I’ll do a slow play and say, "Hey, look, you know what? Let's just make sure we go to the hearing aid evaluation, or we'll get our second ABR, and then we'll meet again to take the next steps." Typically at that first appointment, we'll try to do two things: Set up the diagnostic pathway, and then set up the treatment pathway.

My first appointment (and actually all every follow-up appointment) is: Where are we at on those pathways? My diagnostic part, do we know the reason why? And then, what are we doing about it? And are we where we need to be on our timeline for that?

Listen to the Full Podcast

Pediatric Cochlear Implants with Dr. Prashant Malhotra on the BackTable ENT Podcast)
Ep 22 Pediatric Cochlear Implants with Dr. Prashant Malhotra
00:00 / 01:04

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Pediatric Cochlear Implants: Diagnostic Workup

Dr. Malhotra continues to discuss his diagnostic algorithm; he mentions if patients don’t come to him with an already established cause of hearing loss (i.e CMV), a thorough history is key. This includes family history, understanding exposure to inciting agents, and a complete physical exam to detect any congenital malformations. Further testing includes CMV, Connexin, and imaging by way of MRI or CT. The last factor Dr. Malhotra includes in his diagnostic workup is genetic sequencing to evaluate pediatric cochlear implant candidacy.

[Dr. Gopi Shah]
I know we've talked about workup and congenital hearing loss, but I would love to hear your personal algorithm or diagnostic part. Do you have a checklist in your head?

[Dr. Prashant Malhotra]
I have a pretty thorough questionnaire. It asks for a lot of historical context like perinatal stuff, such as the prematurity, hyperbilirubinemia, and ECMO exposure. There's also a family history part of it, which delves into genetic patterns of hearing loss. Then, I do a thorough physical exam, which is fairly limited in infants. I look at the eyes and the palate. I look for branchial anomalies and obvious facial dysmorphisms or asymmetry. Now, if something comes up pretty clearly, like they're premature with all this exposure, or they come to me already knowing that it is CMV, then it's a readily apparent diagnosis.

If it's an unknown diagnosis, and I can see them early, then I'll do a CMV IDG. Oftentimes, I'm not going to see them within three weeks. But I want to at least know as early as I can, if they've been exposed to CMV. I'll do that and the Connexin test with one blood draw. Connexin is done at our institution, so we get it back within a few weeks.

At the first visit, I start with those targeted tests if it's a bilateral loss. By the next visit, I'll know the Connexin status and whether they have CMV exposure or not. After that, I will usually get imaging, especially if we're talking about implantation. I'm going to want an MRI, unless I know the Connexin, in which case I’d be comfortable with a CT. But I want to know the nerve status and the MRI will let me look at that much better. If they're syndromic, I want an MRI and a CT, typically if they have a CHARGE syndrome or something along those lines.

After imaging and initial testing, I'll typically do the next generation genetic sequencing. I encourage families to do it. It’s not a must, but it's typically well-covered, soI'll do a genetics referral. Our genetics people will pre-authorize it, write a letter to get it covered, and we have good experience and good success with getting the next generation sequence genetic panel of the 170 different genes. It depends on whether it's unilateral or bilateral hearing loss, but I will usually make sure I know my CMV status, my Connexin status, my imaging, and the overall genetic status.

Cochlear Implant Mapping in Children

Dr. Malhotra continues to highlight the importance of regular follow up in pediatric cochlear implant recipients. He states it is important to follow up not only in the postoperative period to identify possible cochlear implant risks, but also long term to ensure the patients are progressing as they should with proper cochlear implant mapping. The importance of teaching families about different resources is highlighted with consistent evaluations as the patient grows. Speech therapy, audiology, and ENT should be among the follow up care team for children with cochlear implants. Ideally Dr. Malhotra has them follow every six months initially, followed by once every year as the years progress.

[Dr. Prashant Malhotra]
I really appreciate you guys having me on. I guess a couple of other things that I was thinking about is, any child with an implant who's not doing well. If you see them, capture them in your office and send them out to the CI center to evaluate what's going on. They should make sure their mapping is up to date. If need be, get a CT scan to make sure there are no issues with the implants.

If I haven't seen them in a couple of years, because now they're in school, my goal is to make sure they're making the progress we anticipated them to make. It always pains me when I see somebody comes back and they haven't seen anybody in like six years, and their speech is not very well managed and they're struggling in school. I just wish that they had seen somebody.

The families don't always know that long-term mapping and programming needs to be done. I think that you come across someone and they're not really performing very well, send them back to the CI center. If they haven't had a mapping in a year, they need to be seeing somebody.

We do try to help families with those transitions on educational milestones. In kindergarten, as they go to middle school and high school, there is still a ceiling on some of the things that an implant can provide kids. They still have issues with reading, spelling, and literacy kinds of things.

If I think about all the things a family will ask me when they find out their kid is deaf. They always think about things like sports and academics and all these things, and the kids can usually do these very well. But, they still need to be ushered even after that critical period of when they started to get into school. And I think it's not in our realm to typically think about this as otolaryngologists. We’re not teachers, but I've definitely seen that as time has gone on, I've really taken on just making sure that we really plugged them in with the resources appropriately so they can succeed. It does take work even after they've left our halls. When I see the older kids, I'm really worried about the long-term outcomes a lot.

If you send them back to the CI center, then they should at least be visiting a speech therapist when they're older, doing literacy evaluations, and making sure their IPEs are written. All of these things can still help them from an educational standpoint, even if they don't specifically need speech therapy.

[Dr. Gopi Shah]
What's the followup? Should they be coming every year? Do they see you during that one month when you put the processor on? What does the post-op short-term and long-term look like?

[Dr. Prashant Malhotra]
After surgery, I'll see them at a post-op visit, which typically is a telemedicine visit, just for an incision check. Then, they will be seeing the audiologist in-person, who will be able to monitor. If anybody has any concerns, they'll notify me and I can always get them in for an in-person appointment.

I'll usually see them at six months afterwards, to make sure that they've been plugged in and making their appointments. I'd rather find out in several months, as opposed to like a year later, that they'd never made their activation appointment, or they're not seeing speech therapy. So I'll see them at the post-op, at six months, and every six months for the first year or so. For the younger kids, I'll see them every six months until they get into kindergarten. For the older kids, I'll see them every six months for at least a year and then annually. I do want to see them every year. There is a Medicaid expansion, in our state, in which hearing loss is an eligible qualifying diagnosis. In order to continue with these services, they have to see me once a year. So that's a nice thing to help some people keep coming back. The older kids don't often see me every year. That's just not going to necessarily happen, especially if they're doing really well. But, they should still be seeing audiology at least yearly from their mapping.

Podcast Contributors

Dr. Prashant Malhotra discusses Pediatric Cochlear Implants on the BackTable 22 Podcast

Dr. Prashant Malhotra

Dr. Prashant Malhotra is a member of the Department of Otolaryngology and the Hearing Program at Nationwide Children's Hospital and an Assistant Professor in the Department of Otolaryngology Head and Neck Surgery at The Ohio State University College of Medicine.

Dr. Gopi Shah discusses Pediatric Cochlear Implants on the BackTable 22 Podcast

Dr. Gopi Shah

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

Dr. Ashley Agan discusses Pediatric Cochlear Implants on the BackTable 22 Podcast

Dr. Ashley Agan

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

Cite This Podcast

BackTable, LLC (Producer). (2021, May 11). Ep. 22 – Pediatric Cochlear Implants [Audio podcast]. Retrieved from https://www.backtable.com

Disclaimer: The Materials available on BackTable.com 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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