Traditionally, cochlear implants are only provided to children with severe-to-profound hearing loss in both ears. At St. Louis Children’s Hospital, the cochlear implant program is one of the only in the area—and one of few in the nation—providing cochlear implants to children with single-sided deafness.
Bernadette Rakszawski, pediatric audiologist at St. Louis Children’s Hospital, joins the show to discuss how her team is leading the way in assessing the impact of single-sided deafness, as well as understanding how various treatment options affect expected outcomes.
Selected Podcast
New Advancements in Pediatric Cochlear Implantation
Featured Speaker:
Bernadette Rakszawski, AuD
Dr. Rakszawski completed her Bachelor of Science degree at Penn State University before moving to St. Louis. She attended Washington University School of Medicine where she earned her AuD. Bernadette completed her clinical externship as the Judith Gravel Fellow in Pediatric Audiology at the University of North Carolina where she gained extensive experience working in diagnostic audiology and with amplification for children of all ages. Areas of interest include bilateral/bimodal programming and cochlear implant optimization within a family centered approach. Transcription:
New Advancements in Pediatric Cochlear Implantation
Melanie Cole, MS (Host): The cochlear implant program at St. Louis Children’s Hospital is a joint program offered by St. Louis Children’s Hospital and the Washington University department of otolaryngology. My guest to tell us today about pediatric cochlear implantation is Dr. Bernadette Rakszawski. She’s a pediatric cochlear implant audiologist at St. Louis Children’s Hospital. Dr. Rakszawski, tell us a little bit about the cochlear implant program at Children’s. What conditions are treated here?
Bernadette Rakszawski AuD, CCC-A (Guest): The cochlear implant program and St. Louis Children’s Hospital began in the late 1980s. We will soon be celebrating our 1,000th cochlear implant, which his really exciting. Our team consists of audiology, speech language pathology, and otolaryngology. We do pull in other specialties as needed like neuropsychology, social work. We might work with an NF2 clinic, things like that. We are treating permanent severe to profound hearing loss most typically. Overtime, the criteria for cochlear implants has expanded so we do open up the candidacy evaluation for kids with greater amounts of residual hearing. Maybe kids with ANSD, auditory neuropathy, or even single sided deafness.
Host: So tell us a little bit about the implants. What type of implants are available today that maybe weren’t available 10 or 20 years ago.
Dr. Rakszawski: We work with all three of the FDA approved cochlear implant manufacturers. The technology today involves using multiple electro channels, which is much different than how they started back in the late 70s and the early 80s. They mostly all have an internal component and an external component that needs to be worn for them to be used. In our audiology department here, we work with other implantable auditory devices like the bone conduction systems or an auditory brain stem implant—or an ABI—as well.
Host: So then tell us about implants for single sided deafness first. Why wasn’t this done before? What's been the evolution of it?
Dr. Rakszawski: Sure. That’s a great question. Historically we’ve reserved cochlear implants for patients who have a bilateral severe to profound hearing loss. But we have seen that over the past 20/30/40 years, we’ve seen these CI recipients—or cochlear implant recipients—have a lot of success. So the hearing rehab field began to expand that candidacy criteria. To step away from cochlear implants and just look at single sided deafness, but specifically talking about somebody who has a severe to profound hearing loss in one ear and normal or near normal hearing in their opposite ear. Due to that degree of hearing loss, the patient isn’t considered a hearing aid candidate in their severe to profound hearing loss ear, but we know that they still have a lot of issues and concerns that arrive. One big one can be tinnitus, but really it’s difficulty for binaural listening situations. For instance, difficulty hearing in background noise, localizing to a sound, or hearing sounds that are presented on the side of the deaf ear—which we call the [inaudible] effect.
These three issues can lead to a host of subsequent issues, like speech and language delays in children, academic concerns, social and emotional issues. All which can effect their quality of life. So as cochlear implants and that technology improved, we started to think—as a field—that this might be something that could help single sided deafness patients. Most importantly, it starts with single sided deafness patients who had intractable tinnitus. So the first CI recipients who had really normal hearing on one ear but a severe to profound hearing loss on the other received the cochlear implant really as a treatment for tinnitus and not for hearing issues. Through those initial studies, those same recipients began to experience improvements in their binaural listening situations. So then we began to open that up more for people who had their chief complaint being hearing concerns because of the single sided deafness—or the SSD—as opposed to just the tinnitus.
We’ve seen a wide range of outcomes in our national research that’s been done, but here at SLCH we created a really thorough evaluation process because we wanted to make sure that we were offering cochlear implants to participants that could really benefit from them. So we’ve learned through the research that if you have a shorter length of deafness before receiving the cochlear implant, if you're a motivated user, and if you participate in auditory training after activating the cochlear implant then you're likely to have better outcomes. Sometimes we see poorer outcomes and that might be linked to experiences where users have had a really long length of deafness in between—you know having hearing loss before they receive the cochlear implant or if they're not very motivated to work at the rehab side of things on the backend.
Host: What a great point, and I'm so glad that you pointed that out and got to my questions about outcomes. Thank you for that. So what’s the criteria for cochlear implantation?
Dr. Rakszawski: First and foremost is we’re looking for a severe to profound hearing loss in the ear with hearing loss. If you have a lesser degree of hearing loss then we might opt to fit a hearing aid or other less invasive approaches. Beyond that, we’re only offering this to patients who are expressing hearing concerns and that obtain limited benefit either from a traditional hearing aid or from a different contralateral routing device. We really try to talk to families and assess their level of motivation for continued device us, appointment follow up, and auditory training after implantation. However, we do consider each patient on an individual level and we tailor our team recommendations accordingly.
Host: So once the process has gotten started and patient selection criteria is determined, what are some of the long and short term effects of implantation?
Dr. Rakszawski: Great question because we see a big range from right after cochlear implantation to longer follow up. Right after we activate the cochlear implant, typically recipients will say that the sound quality is pretty poor. It might be robotic, high-pitched. There's not very good clarity that comes from it. The good news is the more that you wear it, the better it sounds. Overtime, we hope that that improves. We know that with continued so putting the cochlear implant on every day and wearing it during what we call all waking hours—anytime you're awake—through optimizing the programming through your audiology appointments, and then through auditory training, we begin to see binaural hearing benefits. Which could include improved audibility to hear noise that’s presented on that side, improved ability to hear in background noise or to localize to where sounds are coming from. Then once those hearing benefits come through we start to see them benefit your social listening situations and your quality of life.
So the long-term effects though of implantation, you know we hope to have these good outcomes where it’s improving your listening ability, but there are things to consider because you do have an implant in your head. So kids or adults with cochlear implants, they're able to play sports and live their life, but we do have certain precautions we want to keep in mind because of that implanted device. So we’re cautious of hits to the head, static electric discharge, and we consider MRI compatibility because there is a magnet component to the internal piece that’s implanted.
Host: That’s so interesting. What about bilateral implantation? Is this becoming the standard of care for patients who cannot receive adequate benefit from hearing aids? As you discussed single sided deafness, what about bilateral implantation?
Dr. Rakszawski: We are seeing a lot more kids receive bilateral cochlear implants, and it really does go in line with what we’re thinking about for the patients with single sided deafness in that you hear best whenever your brain receives information from both sides of your body. It helps with all of those tricky situations like listening in background noise, localizing and such. So the trend really is to provide adequate and optimized hearing in both ears. So if you have a severe to profound hearing loss or some degree that puts you in the candidacy range then we are likely to recommend a bilateral cochlear implantation. That being said, we also know that in terms of language learning, one cochlear implant—especially for our young patients, our 12 month old/16 month old patients—one cochlear implant will give them the access to sound that they need to learn spoken language. So we sometimes will offer a sequential cochlear implantation where we plan to do both ears eventually, but we might start with one and then do surgery a few months later for the second. Or we could opt to do them simultaneously where surgery happens at each ear at the same time and we activate them pretty close to one another.
Host: Isn’t that amazing what you can do now doctor? It really is fascinating. So tell us about some of the research in this area including projects that are currently taking place at St. Louis Children’s Hospital.
Dr. Rakszawski: We have—Thankfully this is a very interesting area of the field and so there’s tons of research happening internationally and nationally. Here at Children’s we are working closely with our Wash-U partners at Washington University so complete some research studies. We’re often times looking at specifically for cochlear implants. What programming works best? There are different noise algorithms or different processing strategies that we can use to create a clearer, more beneficial auditory signal so we can do different research and figure out which kids respond well to those.
Our current research project is specifically looking at this population of kids with single-sided deafness. While we offer a cochlear implant to some SSD patients, we don’t do that for all. So we do have a very thorough evaluation process that we do both clinically and through our research protocol which allows us to provide a lot of education to the family and to the child as well as information about all of the treatment options available for single-sided deafness, which might include maybe a monitoring approach or different less invasive devices to be worn. So our current research project is really looking at all of those options and what outcomes we see whenever families choose different devices. Then through that we can really specifically focus in on the cochlear implant users and how they do with the cochlear implant after receiving it three months out, six months out, twelve months out. Looking at cochlear implants in the single sided deafness population is still pretty new. And especially in children there’s not a lot of published research yet. So we’re really excited to be kind of on the front line of that.
Host: As you should be. That is very exciting research. So at what point should a physician refer to a specialist and what can that referring physician expect from the cochlear implant team?
Dr. Rakszawski: I would say anytime you're working with a child that’s diagnosed with a hearing loss it is worthwhile reaching out to a pediatric audiology team, especially if there is unilateral hearing loss or single sided deafness. At that point, we can just provide the family and the child with a lot of information and education. But beyond that, especially if you have parents who are having concerns in the academic setting or in the social setting. They feel like their child isn’t responding as well as they should be, or if they're just seeking more information. One of the largest components of our practice with the single sided deafness or hearing loss community in general is educating the families so that they can be advocates for their children. So it’s never too soon to refer to an audiologist or anyone on our team here, even if it’s just as a reconnaissance mission for the families.
Whenever a referring physician contacts our team, they should expect a really thorough evaluation that provides that counselling and that education about hearing loss. We can discuss all of the possible treatment options. For the single sided deafness community, there are several pathways, and it’s hard to know for each individual child which one is the right one until we really get to know them more and know what their concerns and their priorities are. But that can be said for multiple types of hearing loss and different devices. We work with all devices here at our center. We also offer a trial of these devices, so families have the opportunity to gain experience with them before they're financially responsible either through insurance or out of pocket expenses for their own personal equipment.
Most importantly, we’re offering this team approach where we bring in the speech pathology, the ENT environment. So we’re really looking at the whole child. We strive to bring in and communicate with the local providers because we want to get to know that we’re providing the best service for these families. Working with the team that provides their routine follow up, that has the rapport with the family, we find that to be a crucial component to that.
Host: What else would a referring physician want to know about the cochlear implant program at Children’s? What would you like to tell us?
Dr. Rakszawski: I’d like them to know that we utilize a whole child approach in a family centered model. We’re learning a lot more about this population and we’re eager to share that, and we’re eager to learn from our referring physicians to see what they're experiencing with their patients.
Host: Doctor, thank you so much for joining us today. As I said before, what an amazing time for cochlear implantation and the work that you're doing. Thank you, again, for joining us. That wraps up this episode of Radio Rounds with St. Louis Children’s Hospital. To consult with a specialist or to learn more about services or resources available at St. Louis Children’s Hospital, please call the Children’s direct physician access line at 1-800-678-HELP. You can also head on over to our website at stlouischildrens.org for more information and to get connected with one of our providers. If you found this podcast informative as a provider, please share it with other providers that you know. Share it with your patients so that we can all learn from the experts together. Don’t miss all the other fascinating podcasts in our library. Until next time, I'm Melanie Cole.
New Advancements in Pediatric Cochlear Implantation
Melanie Cole, MS (Host): The cochlear implant program at St. Louis Children’s Hospital is a joint program offered by St. Louis Children’s Hospital and the Washington University department of otolaryngology. My guest to tell us today about pediatric cochlear implantation is Dr. Bernadette Rakszawski. She’s a pediatric cochlear implant audiologist at St. Louis Children’s Hospital. Dr. Rakszawski, tell us a little bit about the cochlear implant program at Children’s. What conditions are treated here?
Bernadette Rakszawski AuD, CCC-A (Guest): The cochlear implant program and St. Louis Children’s Hospital began in the late 1980s. We will soon be celebrating our 1,000th cochlear implant, which his really exciting. Our team consists of audiology, speech language pathology, and otolaryngology. We do pull in other specialties as needed like neuropsychology, social work. We might work with an NF2 clinic, things like that. We are treating permanent severe to profound hearing loss most typically. Overtime, the criteria for cochlear implants has expanded so we do open up the candidacy evaluation for kids with greater amounts of residual hearing. Maybe kids with ANSD, auditory neuropathy, or even single sided deafness.
Host: So tell us a little bit about the implants. What type of implants are available today that maybe weren’t available 10 or 20 years ago.
Dr. Rakszawski: We work with all three of the FDA approved cochlear implant manufacturers. The technology today involves using multiple electro channels, which is much different than how they started back in the late 70s and the early 80s. They mostly all have an internal component and an external component that needs to be worn for them to be used. In our audiology department here, we work with other implantable auditory devices like the bone conduction systems or an auditory brain stem implant—or an ABI—as well.
Host: So then tell us about implants for single sided deafness first. Why wasn’t this done before? What's been the evolution of it?
Dr. Rakszawski: Sure. That’s a great question. Historically we’ve reserved cochlear implants for patients who have a bilateral severe to profound hearing loss. But we have seen that over the past 20/30/40 years, we’ve seen these CI recipients—or cochlear implant recipients—have a lot of success. So the hearing rehab field began to expand that candidacy criteria. To step away from cochlear implants and just look at single sided deafness, but specifically talking about somebody who has a severe to profound hearing loss in one ear and normal or near normal hearing in their opposite ear. Due to that degree of hearing loss, the patient isn’t considered a hearing aid candidate in their severe to profound hearing loss ear, but we know that they still have a lot of issues and concerns that arrive. One big one can be tinnitus, but really it’s difficulty for binaural listening situations. For instance, difficulty hearing in background noise, localizing to a sound, or hearing sounds that are presented on the side of the deaf ear—which we call the [inaudible] effect.
These three issues can lead to a host of subsequent issues, like speech and language delays in children, academic concerns, social and emotional issues. All which can effect their quality of life. So as cochlear implants and that technology improved, we started to think—as a field—that this might be something that could help single sided deafness patients. Most importantly, it starts with single sided deafness patients who had intractable tinnitus. So the first CI recipients who had really normal hearing on one ear but a severe to profound hearing loss on the other received the cochlear implant really as a treatment for tinnitus and not for hearing issues. Through those initial studies, those same recipients began to experience improvements in their binaural listening situations. So then we began to open that up more for people who had their chief complaint being hearing concerns because of the single sided deafness—or the SSD—as opposed to just the tinnitus.
We’ve seen a wide range of outcomes in our national research that’s been done, but here at SLCH we created a really thorough evaluation process because we wanted to make sure that we were offering cochlear implants to participants that could really benefit from them. So we’ve learned through the research that if you have a shorter length of deafness before receiving the cochlear implant, if you're a motivated user, and if you participate in auditory training after activating the cochlear implant then you're likely to have better outcomes. Sometimes we see poorer outcomes and that might be linked to experiences where users have had a really long length of deafness in between—you know having hearing loss before they receive the cochlear implant or if they're not very motivated to work at the rehab side of things on the backend.
Host: What a great point, and I'm so glad that you pointed that out and got to my questions about outcomes. Thank you for that. So what’s the criteria for cochlear implantation?
Dr. Rakszawski: First and foremost is we’re looking for a severe to profound hearing loss in the ear with hearing loss. If you have a lesser degree of hearing loss then we might opt to fit a hearing aid or other less invasive approaches. Beyond that, we’re only offering this to patients who are expressing hearing concerns and that obtain limited benefit either from a traditional hearing aid or from a different contralateral routing device. We really try to talk to families and assess their level of motivation for continued device us, appointment follow up, and auditory training after implantation. However, we do consider each patient on an individual level and we tailor our team recommendations accordingly.
Host: So once the process has gotten started and patient selection criteria is determined, what are some of the long and short term effects of implantation?
Dr. Rakszawski: Great question because we see a big range from right after cochlear implantation to longer follow up. Right after we activate the cochlear implant, typically recipients will say that the sound quality is pretty poor. It might be robotic, high-pitched. There's not very good clarity that comes from it. The good news is the more that you wear it, the better it sounds. Overtime, we hope that that improves. We know that with continued so putting the cochlear implant on every day and wearing it during what we call all waking hours—anytime you're awake—through optimizing the programming through your audiology appointments, and then through auditory training, we begin to see binaural hearing benefits. Which could include improved audibility to hear noise that’s presented on that side, improved ability to hear in background noise or to localize to where sounds are coming from. Then once those hearing benefits come through we start to see them benefit your social listening situations and your quality of life.
So the long-term effects though of implantation, you know we hope to have these good outcomes where it’s improving your listening ability, but there are things to consider because you do have an implant in your head. So kids or adults with cochlear implants, they're able to play sports and live their life, but we do have certain precautions we want to keep in mind because of that implanted device. So we’re cautious of hits to the head, static electric discharge, and we consider MRI compatibility because there is a magnet component to the internal piece that’s implanted.
Host: That’s so interesting. What about bilateral implantation? Is this becoming the standard of care for patients who cannot receive adequate benefit from hearing aids? As you discussed single sided deafness, what about bilateral implantation?
Dr. Rakszawski: We are seeing a lot more kids receive bilateral cochlear implants, and it really does go in line with what we’re thinking about for the patients with single sided deafness in that you hear best whenever your brain receives information from both sides of your body. It helps with all of those tricky situations like listening in background noise, localizing and such. So the trend really is to provide adequate and optimized hearing in both ears. So if you have a severe to profound hearing loss or some degree that puts you in the candidacy range then we are likely to recommend a bilateral cochlear implantation. That being said, we also know that in terms of language learning, one cochlear implant—especially for our young patients, our 12 month old/16 month old patients—one cochlear implant will give them the access to sound that they need to learn spoken language. So we sometimes will offer a sequential cochlear implantation where we plan to do both ears eventually, but we might start with one and then do surgery a few months later for the second. Or we could opt to do them simultaneously where surgery happens at each ear at the same time and we activate them pretty close to one another.
Host: Isn’t that amazing what you can do now doctor? It really is fascinating. So tell us about some of the research in this area including projects that are currently taking place at St. Louis Children’s Hospital.
Dr. Rakszawski: We have—Thankfully this is a very interesting area of the field and so there’s tons of research happening internationally and nationally. Here at Children’s we are working closely with our Wash-U partners at Washington University so complete some research studies. We’re often times looking at specifically for cochlear implants. What programming works best? There are different noise algorithms or different processing strategies that we can use to create a clearer, more beneficial auditory signal so we can do different research and figure out which kids respond well to those.
Our current research project is specifically looking at this population of kids with single-sided deafness. While we offer a cochlear implant to some SSD patients, we don’t do that for all. So we do have a very thorough evaluation process that we do both clinically and through our research protocol which allows us to provide a lot of education to the family and to the child as well as information about all of the treatment options available for single-sided deafness, which might include maybe a monitoring approach or different less invasive devices to be worn. So our current research project is really looking at all of those options and what outcomes we see whenever families choose different devices. Then through that we can really specifically focus in on the cochlear implant users and how they do with the cochlear implant after receiving it three months out, six months out, twelve months out. Looking at cochlear implants in the single sided deafness population is still pretty new. And especially in children there’s not a lot of published research yet. So we’re really excited to be kind of on the front line of that.
Host: As you should be. That is very exciting research. So at what point should a physician refer to a specialist and what can that referring physician expect from the cochlear implant team?
Dr. Rakszawski: I would say anytime you're working with a child that’s diagnosed with a hearing loss it is worthwhile reaching out to a pediatric audiology team, especially if there is unilateral hearing loss or single sided deafness. At that point, we can just provide the family and the child with a lot of information and education. But beyond that, especially if you have parents who are having concerns in the academic setting or in the social setting. They feel like their child isn’t responding as well as they should be, or if they're just seeking more information. One of the largest components of our practice with the single sided deafness or hearing loss community in general is educating the families so that they can be advocates for their children. So it’s never too soon to refer to an audiologist or anyone on our team here, even if it’s just as a reconnaissance mission for the families.
Whenever a referring physician contacts our team, they should expect a really thorough evaluation that provides that counselling and that education about hearing loss. We can discuss all of the possible treatment options. For the single sided deafness community, there are several pathways, and it’s hard to know for each individual child which one is the right one until we really get to know them more and know what their concerns and their priorities are. But that can be said for multiple types of hearing loss and different devices. We work with all devices here at our center. We also offer a trial of these devices, so families have the opportunity to gain experience with them before they're financially responsible either through insurance or out of pocket expenses for their own personal equipment.
Most importantly, we’re offering this team approach where we bring in the speech pathology, the ENT environment. So we’re really looking at the whole child. We strive to bring in and communicate with the local providers because we want to get to know that we’re providing the best service for these families. Working with the team that provides their routine follow up, that has the rapport with the family, we find that to be a crucial component to that.
Host: What else would a referring physician want to know about the cochlear implant program at Children’s? What would you like to tell us?
Dr. Rakszawski: I’d like them to know that we utilize a whole child approach in a family centered model. We’re learning a lot more about this population and we’re eager to share that, and we’re eager to learn from our referring physicians to see what they're experiencing with their patients.
Host: Doctor, thank you so much for joining us today. As I said before, what an amazing time for cochlear implantation and the work that you're doing. Thank you, again, for joining us. That wraps up this episode of Radio Rounds with St. Louis Children’s Hospital. To consult with a specialist or to learn more about services or resources available at St. Louis Children’s Hospital, please call the Children’s direct physician access line at 1-800-678-HELP. You can also head on over to our website at stlouischildrens.org for more information and to get connected with one of our providers. If you found this podcast informative as a provider, please share it with other providers that you know. Share it with your patients so that we can all learn from the experts together. Don’t miss all the other fascinating podcasts in our library. Until next time, I'm Melanie Cole.