The Latest on Pediatric Unilateral Hearing Loss and Cochlear Implants

Podcast Transcript

Paul Bryson: Welcome to Head and Neck Innovations, a Cleveland Clinic podcast for medical professionals exploring the latest innovations, discoveries, and surgical advances in otolaryngology-head and neck surgery.

Thanks for joining us for another episode of Head and Neck Innovations. I'm your host, Paul Bryson, Director of the Cleveland Clinic Voice Center. You can follow me on X, formerly Twitter, @PaulCBryson, and you can get the latest updates from Cleveland Clinic Otolaryngology-Head and Neck Surgery by following @CleClinicHNI on X. That's @CleClinicHNI. You can also find us on LinkedIn at Cleveland Clinic Otolaryngology - Head and Neck Surgery, and Instagram at Cleveland Clinic Otolaryngology.

Today I'm excited to speak with my colleague and friend, Dr. Samantha Anne, Medical Director of Pediatric Ear and Hearing Disorders at the Cleveland Clinic. Dr. Anne is a professor of Otolaryngology Head and Neck Surgery in the Cleveland Clinic's Lerner College of Medicine. Welcome to Head and Neck Innovations.

Samantha Anne: Thank you, Dr. Bryson. How are you?

Paul Bryson: Doing well, thanks. I appreciate you coming on the podcast today and sharing some stuff about your program. Before we dive into that, let's start by having you share some background on yourself for our listeners, where you're from, where you trained, how you came to Cleveland Clinic, but then also what excites you about hearing rehabilitation and your program in particular.

Samantha Anne: Yeah, so I trained at Case Western Reserve University and then did my fellowship in pediatric audiology at Children's Hospital of Pittsburgh, and then came here for my first job at the Cleveland Clinic 15 years ago, and I'm still here. So, I've been taking care of these children with hearing loss for the past 15 years. When I first started, we started this clinic called the Pediatric Hearing Management Clinic because we felt that there should be a multidisciplinary care for children with hearing loss where they get to see the geneticists, the communication specialists, audiologists, all the team members that would be involved in taking care of these children, and that clinic has flourished and continues to grow. And in the past few years, I also taken over as the Medical Director for Pediatric Ear and Hearing Disorders and all of these things. We essentially are at the forefront in terms of managing children with hearing loss.

We have some of the best experts and the best management team that's out there, and we're able to handle almost, if not all, children with hearing loss and coming up with the best solutions in terms of getting hearing rehabilitation and getting them into the place where they succeed, along with speech and language milestones and just developmentally achieving everything they can. That's my background and that's what we do. And what gets me very excited about children with hearing loss is it's something that we can easily address and really make their quality of life, and also developmentally meeting their milestones and their achievements so they're not hindered and limited with the hearing loss. Really, we can do everything we can to get these children to where they need to be and not let hearing loss hold them back. And that's what gets me excited because I feel that we have so much to offer for these children so that they can live their lives as if hearing loss is not part of the equation.

Paul Bryson: Yeah, it's pretty gratifying I have to imagine. You've seen kids now, you've been, your practice’s grown. It's very mature. You've seen kids that you probably met at a very young age and now they're in middle school or maybe doing things that maybe their parents were concerned that they wouldn't get to do.

Samantha Anne: Absolutely. I mean, I've seen newborn deaf children get implants and then act like they've never had a handicap in their life. And I've seen the really mild hearing loss. Children get hearing aids and now they're adolescents and they're doing great, and it really is gratifying to watch that transition over time. But yeah, it's great.

Paul Bryson: For our listeners, you've really made consistent contributions to our research literature on a range of topics, and we're going to talk a little bit about that, but what are your primary areas of focus or interest within your center?

Samantha Anne: I think what's great about research you is start going after one thing and it leads you to another thing, and as you start digging into that, it leads you into another path. And I feel a lot of my research and career trajectory has been that way. We initially started with looking at evaluation of hearing loss, and figuring out the optimal techniques including imaging and audiological testing. From there, I started to get more and more interested in unilateral hearing loss in children and looking at the impact of unilateral hearing loss on children. And then most recently, that has evolved into looking at management of these children with hearing loss when they have especially one-sided deficits. And within that, we're looking at rehabilitation and what that means to these children.

In the past in history, if you had a hearing loss on one side, they would put you in front of the classroom, put the good ear facing the teacher, and you're good to go. However, the evidence out there is that these children with unilateral hearing loss still struggle with speech and language, and behaviorally they start to fall behind in school and sometimes need to repeat the grades. So now we're a lot more aggressive about treating these children. So, we've looked at both the evaluation and the impact of unilateral hearing loss.

The most recent research has focused on cochlear implantation for unilateral hearing loss. So, CI for UHL has been FDA approved within the past five years. So, there's very limited data and evidence on how these children do, and it really is a big mystery. There are so many variables that come into play in terms of if children with UHL benefit from cochlear implantation or not, we find some children who have had unilateral hearing loss for very short period of time. They're very young and we do implantation, and the children are not wearing their implants - for some reason they don't like them - they take them off.

And then there's children who you would not expect good results from because they've had prolonged duration of deafness, and they're older and we do implantation on them, and they love their implants and they're wearing it every day. And there's a considerable amount of research and efforts going into trying to figure out what makes these children do well with their implants, and what makes these children want to wear them, and what are the variables that cause these children to be high risk for non-use. So that's been the focus of our research in the last few years, and most recently trying to tease out all these variables that can impact CI outcomes in children with UHL - looking at age of hearing loss, duration of deafness, and quality of life impact. Most recently the impact of streaming, meaning direct input of sound into the deaf ear with an implant to try to force that ear to work. Looking at all these variables has been the focus of our research.

Paul Bryson: It's very exciting. Like you alluded to earlier, you ask one question, you then have three other questions. There's new technology or a new patient group that gets access to technology where there's all these questions. As you look out on the horizon, what other things do you foresee people exploring? I know on my own, I don't, don't work in hearing loss or in surgical rehabilitation of it, but you start to see things on a traumatic insertion, different arrays, like I didn't know about streaming directly into the ear. What are some other things that you're hearing that you think could come to the fore a little bit in some of your work?

Samantha Anne: Yeah, Paul, there's literally so much to learn from this population. We looked at, for example, quality of life benefits for children with CI. Because we were seeing children with poor outcomes who audiologically love their implants, so we were like what benefit are they getting that they want to wear them, versus the children with poor outcomes that wear them with good outcomes that don't want to wear them? So, looking at quality of life benefits, our study found that these children, there's definitely positive quality of life benefits, and even in the children with long duration of deafness where you would expect poor outcomes, they had good quality of life benefits, and that could be one of the reasons that they're using their implants. Even if audiologically, we'll look at them and say they don't seem to be getting benefit. We already submitted research for publication and it's in revision right now looking at the impact of duration of deafness and age of hearing loss in auditory outcomes.

And that's coming out on the horizon. We are now in the process of publishing our data, looking at correlation of data logging, which is the amount of time that the child is using the implant, and then outcomes. For example, do the children that wear these implants for a longer period of time tend to do better with their outcomes and are they making more use of them? Interestingly, it's not just data logging that we found makes an impact in terms of use. It's not just the number of hours to dangle it on their head. We found that both direct auditory input streaming, in other words, forcing that ear to work, is actually playing an impact on the outcomes. So, in bilateral hearing loss children, you put an implant in. Data logging has been consistently shown to improve outcomes. The longer you wear them, the better they do in unilateral hearing loss children.

Interestingly, it's not just about how long you wear it, but also the direct auditory input and forcing that ear to work using streaming. So there's a wealth of information and wealth of research that's coming out now. And as we learn more and more about these patients, on the horizon the next things that we're looking at the Cleveland Clinic is looking at the cognitive brain level. Because again, if you can't figure out why these children like them or not like them based on the duration of deafness and age of hearing loss and all these other variables, they're not giving us an exact recipe for what makes a good implant user, so well, maybe we need to look a little deeper. So, we are considering using functional MRI to evaluate brain function on a cortical level to see how these children with good outcomes, how their brains are working versus the children with bad outcomes or poor outcomes? I should say, how their brains are working, and to see if there's any differences that could explain these outcomes? So that's on the horizon and there's additional studies that are coming out, but there are lots to come yet and a lot to learn still.

Paul Bryson: Well, it's really exciting and it's really been a pleasure to kind of see your research program grow and to just see your contributions. Congratulations on all of this, and I really appreciate your time today. And as we wrap up, any final take home messages for our listeners or any advice that you would give a parent that might suspect that their child has hearing loss or know that they have a hearing loss?

Samantha Anne: Yeah, I think the main point I have for families is that if you have a concern about a hearing loss, get them evaluated. There should be no reason that hearing should be the cause of a child not reaching their potential. There are so many different options and so many different things we can do. And yes, there are those extreme situations where unfortunately, there's no quick answer for them, but most times, we're able to help the child work with the hearing loss that they have so that they reach their potential. So, if there's a question of hearing loss or if there's a documented hearing loss, absolutely they should reach out to a provider to look into it further and how we can help these children.

Paul Bryson: Well, I appreciate you joining today, and I'd tell our listener. For more information on our section of pediatric otolaryngology at the Cleveland Clinic, please visit ClevelandClinic.org/PediatricENT. That's ClevelandClinic.org/PediatricENT. And to connect directly with a specialist or to submit a referral, call 216.444.8500. That's 216.444.8500. Dr. Anne, thanks for joining Head and Neck Innovations.

Samantha Anne: Thanks Paul, my pleasure.

Paul Bryson: Thanks for listening to Head and Neck Innovations. You can find additional podcast episodes on our website at clevelandclinic.org\podcasts, or you can subscribe to the podcast on iTunes, Google Play, Spotify, SoundCloud, or wherever you listen to podcasts. Don't forget, you can access real-time updates from Cleveland Clinic experts in otolaryngology – head and neck surgery on our Consult QD website at consultqd.clevelandclinic.org/headandneck. Thank you for listening and join us again next time.