Key Takeaways:

1. PedsHEAR uses readily available clinical data to predict hearing loss risk with 95% accuracy, offering a new level of individualized care.
2. The model was trained and validated using data from over 1,400 patients across North America, ensuring broad applicability and reliability.
3. This predictive tool marks a major shift in pediatric oncology, allowing doctors to tailor interventions like hearing monitoring or STS use based on each child’s specific risk.

The powerful chemotherapy drug cisplatin has been used since the late 1970s to treat a variety of cancers. It’s highly effective against solid tumors and is often a core element of treatment for children with brain and spinal cord tumors, neuroblastoma, and rhabdomyosarcoma.

Yet, cisplatin is well known to cause devastating side effects. In children, the most common side effect following therapy is debilitating hearing loss. Depending on the treatment plan, up to 80% of children treated with cisplatin end up with permanent hearing loss that affects their social lives, school performance, and future careers. 

Now, an international team led by Etan Orgel, MD, at Children’s Hospital Los Angeles has developed a novel machine learning model that can predict an individual child’s risk of developing hearing loss from cisplatin treatment. Called PedsHEAR, the tool uses routine, readily available information to quickly predict this risk—with 95% confidence.

The team, which includes researchers from the Keck School of Medicine of USC and other institutions across the U.S. and Canada, is the first to develop and validate a novel machine learning model for this purpose. 

Results were published in the Journal of Clinical Oncology, and the model is now available for public use.

A Decades-Long Journey to Personalize Care

The study grew out of two decades of efforts to try to prevent cisplatin-induced hearing loss in children. Investigators from CHLA led the pivotal phase 3 Children’s Oncology Group trial of sodium thiosulfate (STS), and in 2022, the Food and Drug Administration approved STS as the first treatment to reduce the risk of hearing loss in children given cisplatin.

But patients’ treatment regimens are already highly complex, and some may not need STS to prevent hearing loss. For those who are not eligible for STS, it’s critical for clinicians to understand each patient’s risk and what options they have to protect that child’s hearing.  

“We want to give families and providers the tools they need to understand their child’s risk and make an informed decision,” explains Dr Orgel, who directs Quality and Patient Safety at CHLA’s Cancer and Blood Disease Institute. “This is the paradigm shift we’re aiming for—speaking in certainties for each child versus speaking in generalities by regimen.”

This new predictive model is informed by a landmark study designed and led by Dr Orgel in 2021. Researchers analyzed data from more than 1,400 cisplatin-treated patients across the United States and Canada to establish the first benchmarks for the prevalence of cisplatin-induced hearing loss in children and adolescents.

Researchers used the 1,400-person dataset as the foundation for their model, training it to analyze risk factors and probabilities and accurately predict a child’s risk level for hearing loss. The researchers also brought in two new, real-world data sets from the Children’s Oncology Group and a children’s hospital in Texas to validate the model in other populations. The now publicly available web model provides each patient with a percentage indicating the child’s individual probability of hearing loss.

Machine Learning Approaches

Joshua Millstein, PhD, from the Keck School of Medicine of USC, led the creation and optimization of the highly complex machine learning model.

“We assessed a wide variety of modeling strategies to arrive at our final approach, which combines several machine learning methods, then applies a higher-level model—called an ensemble predictor—to integrate each model’s predictions into a single interpretable result,” he explains. “The main challenges of building the final model involved tuning it, which requires finding the model parameters that would optimize the tool’s performance.”

Recent advancements in ensemble predictor modeling helped the team overcome several challenges that have caused other models to fail in the past. “Ensuring that these models have enough patient data for pattern recognition can be exceedingly tricky when developing solutions for rare childhood cancers,” adds Dr Millstein. “These new statistical techniques empowered us to deliver a more refined output, even with many differences between patients within our cohorts.”

Creating a New Treatment Planning Standard

“My goal is for this to become a routine clinical tool,” says Dr Orgel. “What’s unique about this model is that it only uses routinely available data, so any doctor can use it from day one of diagnosis to plan treatment. 

“Forewarned is forearmed going into chemotherapy,” he adds. “It’s so important to understand the options in front of you—and how to approach potential interventions with planned monitoring, such as frequency and compliance, with hearing testing.”

The research team’s next goal is to expand the model to young adults and adults up to 65 and to integrate genomics to make the model even more powerful.  

“Ultimately, we aim to expand our approach to understand and predict risk for other common side effects of common chemotherapies,” says Dr Orgel. “We want to equip all patients beginning their cancer journey with knowledge that supports meaningful discussions with their doctors on what to expect during and after treatment.”

Key Takeaways:

1. Audiogene Trial Focus: The Phase 1/2 Audiogene trial evaluates the safety and efficacy of SENS-501 gene therapy for young children with hearing loss caused by OTOF gene mutations.
2. Clinical Presentation: Dr. Natalie Loundon, principal investigator, will present the trial’s progress and rationale at ASPO on May 1, 2025.
3. Early Intervention Goal: The trial targets infants and toddlers to leverage early brain plasticity, aiming to restore hearing and support normal speech development.

Sensorion, a clinical-stage biotechnology company specializing in the development of novel therapies to restore, treat, and prevent hearing loss disorders, announced that Natalie Loundon, pediatric ENT surgeon, director of the Center for Research in Pediatric Audiology at Necker Enfants Malades Hospital, AP-HP, in Paris, France, will deliver an oral presentation on the company’s Audiogene clinical trial at the annual meeting of the American Society of Pediatric Otolaryngology (ASPO).

The Conference is being held in Montreal, Canada, April 30 – May 3, 2025, and Dr Loundon will give her presentation, “Principle and Practice of a Gene Therapy for Hearing loss: A Phase 1/2 Clinical Trial with SENS-501 in Children Suffering from Severe to Profound Hearing Loss,” on May 1st at 4.20 pm CET (10.20 am ET).

Loundon is the principal investigator of Audiogene, Sensorion’s Phase 1/2 clinical trial evaluating SENS-501, a gene therapy treatment for DFNB9, a genetic disorder causing severe to profound hearing loss due to mutations in the OTOF gene. Her presentation will include an overview of the rationale behind gene therapy approaches for inner ear hearing loss disorders and the company’s Audiogene clinical trial.

About the Audiogene Trial

Audiogene aims to evaluate the safety, tolerability, and efficacy of intra-cochlear injection of SENS-501 for the treatment of OTOF gene-mediated hearing loss in infants and toddlers aged 6 months to 31 months at the time of gene therapy treatment. By targeting the first years of life, when brain plasticity is optimal, the chances of these young children with pre-linguistic hearing loss acquiring normal speech and language are maximized. The study comprises two cohorts of two doses followed by an expansion cohort at the selected dose. While safety will be the primary endpoint of the first part of the dose escalation study, auditory brainstem response (ABR) will be the primary efficacy endpoint of the second part of the expansion. Audiogene will also evaluate the clinical safety, performance, and ease of use of the delivery system developed by Sensorion.

About Sensorion

Sensorion is a clinical-stage biotech company, which specializes in the development of novel therapies to restore, treat, and prevent hearing loss disorders, a significant global unmet medical need. Sensorion has built a unique R&D technology platform to expand its understanding of the pathophysiology and etiology of inner ear related diseases, enabling it to select the best targets and mechanisms of action for drug candidates. It has two gene therapy programs aimed at correcting hereditary monogenic forms of deafness, developed in the framework of its broad strategic collaboration focused on the genetics of hearing with the Institut Pasteur. 

SENS-501

SENS-501 (OTOF-GT) currently being developed in a Phase 1/2 clinical trial, targets deafness caused by mutations of the gene encoding for otoferlin and GJB2-GT targets hearing loss related to mutations in GJB2 gene to potentially address important hearing loss segments in adults and children. The company is also working on the identification of biomarkers to improve diagnosis of these underserved illnesses. 

SENS-401

Sensorion’s portfolio also comprises programs of a clinical-stage small molecule, SENS-401 (Arazasetron), for the treatment and prevention of hearing loss disorders. Sensorion’s small molecule progresses in a Phase 2 proof of concept clinical study of SENS-401 in Cisplatin-Induced Ototoxicity (CIO) for the preservation of residual hearing. Sensorion, with partner Cochlear Limited, completed in 2024 a Phase 2a study of SENS-401 for residual hearing preservation in patients scheduled for cochlear implantation. A Phase 2 study of SENS-401 was also completed in Sudden Sensorineural Hearing Loss (SSNHL) in January 2022.

Featured image: Dreamstime

Key Takeaways:

1. Extended Development Timeline: Binaural pitch fusion, vital for distinguishing speech in noisy settings, matures between ages 6 and 14, suggesting an extended period of auditory development.
2. Clinical and Educational Implications: Identifying interventions, such as quieter classrooms and music training, may improve speech perception and academic performance in children with typical hearing and hearing loss.
3. Potential for Targeted Treatments: Understanding how binaural fusion develops could lead to more effective hearing device applications and strategies to prevent broad fusion-related difficulties.

A new study from Oregon Health & Science University is the first to suggest that binaural pitch fusion—a process that involves merging different pitches from each ear into a single sound —is one type of central hearing processing that may still be developing in pre-adolescent children and could present an opportunity for treatment. 

The study was published in the Journal of the Association for Research in Otolaryngology.

Binaural pitch fusion is relevant to the “cocktail party effect,” or the brain’s ability to focus one’s attention and listen to a single person talking while filtering out other voices in the room. Individuals who experience what’s known clinically as “broad binaural fusion” are not able to effectively separate out these multiple voices, which can limit their ability to understand speech in noisy environments.

With better understanding of how and when this ability develops in children, clinicians may be able to develop more effective, practical interventions for broad binaural fusion in children with hearing loss and auditory processing disorders.

“What was very surprising is that we found that even the children with typical hearing had abnormal broad fusion, just like adults with hearing loss, and then over time their fusion became sharper and more mature,” says the study’s corresponding author Lina Reiss, PhD, professor of otolaryngology/head and neck surgery in the OHSU School of Medicine. 

“This indicates an extended timeline of auditory development in children, which has exciting scientific and clinical implications. We hope this will contribute to more effective interventions to support development of speech in noise perception in children.”

Understanding Hearing, Improving Outcomes

Researchers measured and compared binaural pitch fusion changes among children of varying ages and developmental stages. After long-term follow ups, they then compared results of children with normal hearing to those with hearing loss, as well as to those with different hearing device combinations, such as a cochlear implant.

The study found that even children with typical hearing will have greater difficulties with speech perception in noise due to immature binaural fusion development. Findings demonstrated that binaural fusion sharpens significantly from 6 to 14 years old, indicating that binaural connections are still maturing and likely guided by hearing experience during childhood development.

Jennifer Fowler, AuD, assistant professor of otolaryngology/head and neck surgery in the OHSU School of Medicine and co-author of the study, emphasized that identifying interventions to improve speech and noise recognition, even in children with typical hearing, has been shown to be important for developmental outcomes, including academic performance.

“We should be considering interventions to create quieter classroom environments for learning, such as providing microphone systems in a larger classroom, to ensure children can hear over background noise and from a distance,” Fowler says. “Music training has been shown to be an effective way to sharpen binaural fusion, so exploring how to create a music intervention for children in a clinical model could be beneficial for patients.”

Looking forward, researchers hope to identify ways to prevent the development of abnormally broad fusion and reduce its impacts with new or more targeted uses of hearing devices.

“If we can try to understand the biological structures that are involved in binaural fusion by looking closely at the brains of children during this development process,” Reiss says, “we will have a much better idea of how to improve it.” 

This study was supported by the National Institute on Deafness and Other Communication Disorders of the National Institutes of Health, under Award Number R01DC013307. The content is solely the responsibility of the authors and does not necessarily represent the official views of the NIH.

Featured image: Dreamstime

Key Takeaways:

1. A child born profoundly deaf demonstrated near-normal hearing and speech perception improvements after receiving DB-OTO, highlighting its potential impact.
2. Among 11 assessed participants, 10 showed hearing improvements, with some reaching normal or near-normal hearing levels.
3. DB-OTO was well tolerated, with only mild, transient post-surgical vestibular side effects, reinforcing its potential as a safe gene therapy option.

Regeneron Pharmaceuticals Inc. announced updated data for the investigational gene therapy DB-OTO from the Phase 1/2 CHORD trial in 12 children who have profound genetic hearing loss due to variants of the otoferlin (OTOF) gene. These include 72-week results showing speech and development progress in the first child dosed at 10 months of age, as well as initial results in 11 children (aged 10 months to 16 years old)—three of whom received DB-OTO bilaterally (in both ears). The latest results were presented in an oral presentation at the Association for Research in Otolaryngology’s (ARO) 48th Annual MidWinter Meeting.

“Sound is a significant part of the human experience that connects us to each other and our environment,” says Jay T. Rubinstein, MD, PhD, Virginia Merrill Bloedel Professor of Otolaryngology and Bioengineering and Director, Bloedel Hearing Research Center, University of Washington School of Medicine, and a CHORD clinical trial investigator. “A year after treatment in one ear with DB-OTO, a child born profoundly deaf was able to enjoy music, engage in imaginative play, and participate in bedtime reading when the cochlear implant on their other ear was removed. These seemingly small interactions are life-changing for these children as well as their families and these results continue to underscore the revolutionary promise of DB-OTO as a potential treatment for otoferlin-related hearing loss.”

In the trial, 12 participants have received DB-OTO to date—of whom nine were administered an intracochlear injection in one ear and three received it bilaterally. The surgical procedure to administer DB-OTO leverages an approach similar to cochlear implantation, which enables use in young infants.

As presented at ARO, 48-week results from the first participant dosed in the trial showed improvement of hearing to near-normal levels across key speech frequencies. This included hearing thresholds that were within normal limits (0.25-2.0 kHz) in most speech-relevant frequencies and corroborated with positive auditory brainstem responses (ABRs). Particularly encouraging were results from formal speech perception tests in which the child demonstrated improvement from week 48 to week 72 and correctly identified words—such as mommy, cookies, and airplane—that were presented at a conversational level without any visual cues.

Among the 11 participants with at least one post-treatment assessment, 10 demonstrated a notable response, with improved hearing at various decibel hearing levels (dBHL). Additionally, among five participants with 24-week assessments, three experienced improvements in average hearing thresholds to “nearly normal” (n=1; ≤40 dBHL) or normal (n=2; ≤25 dBHL) hearing levels. All ABR responses were corroborated by hearing improvements assessed by pure tone audiometry (PTA). One participant has not experienced a change from their baseline hearing at 24 weeks post-dosing.

Across all 12 participants, both the surgical procedure and DB-OTO were well tolerated, and there were no adverse events or serious adverse events considered related to DB-OTO. Five of 12 participants experienced transient post-surgical vestibular adverse events (e.g., nystagmus, nausea, dizziness, vomiting), which resolved within 6 days of dosing.

DB-OTO received Orphan Drug, Rare Pediatric Disease, Fast Track, and Regenerative Medicine Advanced Therapy designations from the U.S. Food and Drug Administration and Orphan Drug Designation was granted by the European Medicines Agency. The potential use of DB-OTO for otoferlin-related hearing loss is currently under clinical investigation, and its safety and efficacy have not been evaluated by any regulatory authority.

About Otoferlin-related Hearing Loss

Congenital deafness (hearing loss present at birth) is a significant unmet medical need that affects approximately 1.7 out of every 1,000 children born in the U.S. and approximately half of these cases have genetic causes. However, otoferlin-related hearing loss is ultra-rare. This specific condition is caused by variants in the OTOF gene, which leads to a lack of a functional otoferlin protein that is critical for the communication between the sensory cells of the inner ear and the auditory nerve.

About the CHORD Trial

The CHORD trial is an ongoing, Phase 1/2 first-in-human, multicenter, open-label trial to evaluate the safety, tolerability and preliminary efficacy of DB-OTO in infants, children and adolescents with otoferlin variants.

Currently enrolling children across sites in the U.S., United Kingdom, and Spain (<18 years of age), CHORD is being conducted in two parts. In the initial dose-escalation cohort (Part A), participants receive a single intracochlear injection of DB-OTO in one ear, and in the expansion cohort (Part B), participants receive simultaneous single intracochlear injections of DB-OTO in both ears at the selected dose from Part A.

Hearing improvements were assessed by PTA and ABR. PTA is considered by auditory experts to be the gold standard measurement of hearing and is measured through behavioral responses to sound (e.g., turning head towards sound) that is emitted at different intensity levels and measured in decibels (dB). ABR corroborates these behavioral responses, serving as an objective confirmation of hearing function, and is measured through electrical brainstem responses to sound emitted at different dBs. At baseline, all participants had no behavioral (PTA) or electrophysiological (ABR) responses at maximum sound levels (≥100 dB).

Additional information about the trial, including enrollment, can be obtained by contacting clinicaltrials@regeneron.com. 

Featured image: Photo: 22611957 © Tom Wang | Dreamstime.com

S3.2 Diagnostic Hearing Evaluation of the Pediatric Patient addresses the tasks performed by an audiologist when evaluating hearing in children.

S1.1 General Intake Standards was reviewed in 2024 and recommended for editing. The standard describes the intake process of general audiologists and an updated version will be available for review. 

Both standards will be open for public review and comment Monday, March 24, 2025, through Friday, April 11, 2025. Any U.S. audiologist may review and comment on the standards and all comments are welcome.

APSO strives to create standards that describe the basic practices of all audiologists, are based in evidence, and are produced with the support of the entire profession. 

APSO standards may be accessed and comments may be submitted at https://www.audiologystandards.org/standards/working.php. 

APSO encourages reviewers to provide evidence-based comments that include a rationale for edits. All comments are read by subject matter experts and considered in creating a final draft of the standard.

About APSO

Audiology Practice Standards Organization is a non-profit professional organization dedicated to the purpose of developing and maintaining practice standards in the profession of audiology. Standards are developed by recognized subject matter experts in the subject area of each standard and are subject to review and comment by all practicing audiologists. APSO standards documents represent accepted standards of practice by audiologists, as described in peer-reviewed literature. All APSO standards are freely accessible to audiologists and the public. For more information about APSO or to view any standard, visit https://www.audiologystandards.org.

The Hearing Review: The study published in the Journal of Autism and Developmental Disorders, which you conducted with Deborah Mood and Mary Dietrich, focused on four developmental disabilities that co-occur with reduced hearing: cerebral palsy, Down syndrome, autism, and intellectual disability. How many children are we talking about who have a lack of access to ideal hearing assessments?

Angela Bonino: In this particular study, we have access to about 131,000 children who are being seen at three clinical sites in the United States: Children’s Hospital of Philadelphia, Vanderbilt University Medical Center, and Boston Children’s Hospital. And what we see within our sample of children who are receiving clinical services at these sites is about 10% of children in our sample have one of those developmental disabilities that we’re considering. So that gives us a sample of almost 13,000 children who have developmental disabilities that we’re looking at.

Autism is the most common of the four conditions for what we’re seeing. And then in terms of thinking about how often does reduced hearing happen within children who have developmental disabilities, we know from previous literature that children with cerebral palsy, Down syndrome, and intellectual disabilities have a higher rate of having reduced hearing than what we see in the general population. And in our study specifically, children with cerebral palsy and Down syndrome were twice as likely as children in the comparison group to have reduced hearing.

HR: Why is it so important for hearing care professionals to focus on the gold standard of hearing assessment for young patients with developmental disabilities? 

Bonino: Great question. Based on our clinical guidelines, the audiogram or behavioral testing is considered the most reliable source of hearing data. Now, auditory brainstem response or ABR is also considered a gold standard for infants or for other individuals who can’t perform behavioral testing. Those are the only two assessments for which we can actually use the data to fit a hearing aid.

So when it comes to actually managing reduced hearing in individuals, we need to have either audiogram or ABR thresholds to fit a hearing aid, as well as to determine eligibility for special education services or early intervention services. And most states in the United States are going to require you to have either ABR or audiogram results. So we’re really focusing in on those two particular assessments because we know they’re critical to access intervention services and support.

HR: What differences in access to gold standard assessments did you see for children with developmental disabilities in your study?

Bonino: For children in the comparison group—children who don’t have any of those four developmental disabilities in their medical record—what we see is that about 9% of children in the comparison group don’t get access to either audiogram or ABR in the first three months of hearing healthcare. In contrast, for the children with a developmental disability diagnosis in their medical record, we see 24% of children are not getting access to one of those tests in the first three months of hearing healthcare.

We then build statistical models that compute relative risk and we adjust for differences based on site, the age at the time of the first encounter, race, ethnicity, and sex because we know that those factors can also contribute to the likelihood of getting access to a gold standard assessment.

To summarize those findings, what we’re seeing is that broadly when we define children with developmental disabilities, we see that they’re four times more likely not to get access to a gold standard assessment relative to the comparison group. If children have two or more developmental diagnoses of the four diagnoses that we considered, their relative risk is actually the highest of the subgroups that we looked at. So they’re 13 times more likely not to get access in the first three months of hearing healthcare for that particular group.

We then also see differences for looking at the four specific developmental disabilities. Children with autism have a three-fold increase, whereas children with cerebral palsy have a ten-fold increase, children with Down syndrome have a six-fold increase, and then children with intellectual disability have an eleven-fold increase, again, all relative to children in the comparison group.

Listen to The Hearing Review’s Podcast Episode for the entire conversation with Angela Bonino, PhD:

HR: What are some of the main barriers to better hearing assessment access for children with developmental disabilities?

Bonino: I think there’s a lot of things to think about. Our particular study didn’t directly measure factors or barriers or facilitators. So we’re drawing on what’s been published in the literature and certainly what other clinicians and parents have also discussed with us. But I think it’s likely a multifaceted reason for some of the challenges that are happening. One of the things I think is really important to address is the misalignments of the developmental and medical profile of these children with what our assumptions are for our testing.

Behavioral testing, for example, really does assume typical development across a variety of domains. Now, our guidelines always talk about picking the test method that’s developmentally aligned with a child, but the challenge is that all of our methods still assume relatively uniform development across and within developmental domains. And that really can be at odds with what we commonly see in many of these developmental disability profiles.

It’s a common profile within autism that a child might have areas of their cognition that are actually very much so a strength for them. But then they have other areas like executive function and shifting of attention that can be very challenging for them. But our methods assume a pretty uniform cognitive profile as well as considering other domains too. And so I think that misalignment can happen with what our assumptions are for the testing. I think that is really important to consider.

There certainly has also been a lot of discussion within the broader healthcare field thinking about disability-based discrimination that children and their families can experience. And I think that that’s important to be thinking about, too, and what might be some perceptions and experiences that these individuals are routinely encountering within the healthcare field more broadly. I think there’s also challenges around the training that some audiologists may have, and their comfort level sometimes within the context of pediatrics and then also developmental disabilities.

So those are just some of the examples to be thinking about that may be contributing to some of these disparities that we’re seeing.

HR: What can HCPs do to improve access to hearing assessments for children with developmental disabilities?

Bonino: I think one thing to acknowledge is that there is a tremendous amount of research that needs to be done to help give practitioners better tools and better guidance around what can be done. So I do want to recognize that practicing audiologists are in a tricky position because there’s not really great guidance that’s specifically tailored to children who have developmental disabilities, and especially when we start talking about very specific developmental disability conditions. And that makes it really challenging to do their job. And even a lot of our literature that we have around newborn hearing screening and early intervention and language outcomes, a lot of times children with developmental disabilities are excluded from that research base.

Now, one thing I can tell our audiologists that I think is always important to remind them is that we all know that early intervention is really important. And we often talk about the 1-3-6 model—and many states have started moving even to the 1-2-3 model—of “screen by one month, get a diagnosis by three months, and then start intervention by six months” for when we talk about the newborn hearing screening process. We know based on that data that achieving the 1-3-6 benchmarks results in better language and developmental outcomes. And that is true for children who have co-occurring developmental disabilities as well. And so hitting those benchmarks is really important.

And I think that we all should be motivated to make sure we’re continuing to hit those benchmarks and having early diagnosis, even when we’re talking about kids who might show up in the clinic at age three, age four, or even 10 years of age, and well outside of that newborn period, because that is a really common experience. That’s one thing our study also emphasizes is that the median age of the first encounter is three years within these clinical samples. And so even though we oftentimes really think about our newborns, we also need to think about our toddlers and preschoolers, early school-aged kids, and how we’re going to make sure we promote timely diagnosis and access to the appropriate supports to facilitate developmental outcomes for these kids.

HR: Do you think it’s time for audiologists and other HCPs to look at changing the standard of pediatric care with a goal of more broadly applicable hearing assessments?

Bonino: I think it is well time for us to have solid clinical trials, pragmatic trials that are pushing into clinic to really be thinking about how do we make a more expansive toolkit for pediatric audiologists to facilitate testing and starting to collect evidence that supports usage of developmentally informed strategies to start building that evidence base around what care could look like when we step back and really focus on how do we make this care inclusive.

I think a challenge is a lot of the practices and methods that we use in pediatric audiology really make a lot of assumptions around development and the assumption around the child having typical development or having typical health profiles.

And the reality is because of the high survival rates that we have within our NICU graduates, as we have shifting etiologies for what reduced hearing and who we’re monitoring, we need to make sure that our practices also evolve to reflect our common patients that we’re seeing and that we have really good tools for clinical audiologists to use.

That means thinking about how can we either redesign our current test methods or evolve into more inclusive test methods, but also thinking about even simple things like scheduling. Like how do children who have developmental disabilities benefit from a longer test session? And how do we work with our schedulers to make that happen at our sites? What does reimbursement look like? There’s just a lot of factors to think about.

We also have a lot of concern that even when those children do get access to gold standard testing, the amount of thresholds that are collected in a test session may be less than for a child who’s typically developing of the same age. And so how do we have robust support structures to make sure that that child gets in quickly to finish that testing, even when trying to hit those benchmarks with older children, so it doesn’t become a nine-month process to get a full audiogram and to move on with hearing aids if that’s what needs to happen for that child? How do we go ahead and build those systems and have the right types of training for our providers so that families and children can move through the system and get to a timely diagnosis and access to supports?

Listen to The Hearing Review’s entire Hearing Horizons podcast episode with Angela Bonino at https://hearingreview.com/resource-center/hearing-podcasts/improving-hearing-care-for-children-with-developmental-disabilities.

Takeaways

1. Expanded Eligibility: Tricare’s new hearing aid coverage includes children of retired service members enrolled in Tricare Prime, offering benefits for biological, adopted, stepchildren, or court-placed dependents under 21 (or 23 for full-time students).
2. Retroactive Coverage: The benefit applies retroactively to services received on or after Dec. 22, 2023, allowing families to file claims for reimbursement through their regional contractors.
3. Eligibility Criteria: Children must demonstrate a hearing loss of at least 26 decibels in one or both ears through a hearing test, emphasizing the program’s focus on addressing qualifying medical needs.

Tricare, a government-managed health insurance program for military personnel, their families, and other eligible individuals recently expanded its hearing aid coverage to include eligible children of retired service members. 

Providing Pediatric Hearing Care

This new coverage results from a new statute. It aims to improve access to life-changing medical technology and help eligible children of retired service members get the hearing aids they need.

“Before the passage of the National Defense Authorization Act, TRICARE coverage of hearing aids was limited to dependents of active duty service members with qualifying hearing loss,” says Erica Ferron, management and program analyst, TRICARE Health Plan, at the Defense Health Agency. “Now, more TRICARE beneficiaries will be able to receive hearing aids and services.”

Keep reading to learn about eligibility, coverage, and more.

Who is eligible?

For retirees, their children may qualify for hearing aid coverage if:

• They’re entitled to retired or retainer pay, or equivalent pay; and
• Their child is enrolled in TRICARE Prime. This includes the US Family Health Plan, as described in the TRICARE Plans Overview Fact Sheet.

The benefit covers unmarried children of living sponsors who are:

• Under 21 years old, or
• A full-time college student aged 21-23, or
• Unable to support themselves due to a disability that started before age 21 (or before 23 if they were a student)

This includes:

• Biological children
• Adopted children
• Stepchildren
• Children placed in your legal care by a court

Determining Qualification

To get coverage for hearing aids, your child needs to take a hearing test. They must demonstrate at least 26 decibels of hearing loss in one or both ears.

Important Things to Know

• The child must be enrolled in TRICARE Prime or the US Family Health Plan.
• Coverage is retroactive to Dec. 22, 2023. If a child got hearing aids or services on or after that date, you can file for reimbursement for TRICARE covered services. But it won’t happen automatically. You must file a claim with your regional contractor.

Further Reading

Considering Special cases

For whoever is interested in exploring TRICARE’s expanded coverage of hearing aids, please note that the coverage isn’t available overseas. If the child lives in the U.S. but travels overseas, they can keep their coverage, but they need special approval for overseas care.

TRICARE doesn’t cover hearing aids for retirees. Those who are interested may, however, qualify for other special programs. Check out TRICARE’s hearing aids page to learn more.

For a child to get this benefit, they must have TRICARE Prime and a qualifying hearing loss. The ability to hear properly is essential for your child’s social and physical development.

Photo: Dreamstime

Takeaways:

1. Children with cochlear implants who initially learn more shape-based nouns, like “chair” or “cup,” exhibit stronger language development over the following three years.
2. The study indicates that shape-based nouns significantly benefit vocabulary growth and grammar skills, especially for children with cochlear implants, helping them close language gaps with peers.
3. Researchers suggest that early vocabulary strategies emphasizing shape-based nouns could help children with hearing loss overcome initial language delays, with further studies needed to confirm causation.

A University of Miami study offers new insights into language development in children with hearing loss, suggesting language learning strategies that may help children with cochlear implants—surgically implanted hearing devices—overcome initial language development delays. 

The study, conducted by University of Miami College of Arts and Sciences researchers Lynn K. Perry and Daniel S. Messinger and University of Miami Leonard M. Miller School of Medicine researcher Ivette Cejas, sheds light on the relationship between early vocabulary knowledge and later language development in children with cochlear implants. 

The researchers focused on the proportion of shape-based nouns in children’s initial vocabularies. Shape-based nouns are words like “chair” or “cup” that describe a category of objects based on their shape, rather than other characteristics such as color or material.

Cochlear Implants and Language Development

Their findings, published in Developmental Science, show that a higher proportion of shape-based nouns in a child’s vocabulary shortly after cochlear implantation was associated with better language development for the next three years. 

The researchers also found that the association between the shape-based nouns and long-term language development was stronger in children who had received cochlear implants, compared to children with normal hearing. The results have implications for efforts to help children with hearing loss surmount initial language delays caused by a lack of auditory input and access to speech sounds before they receive cochlear implants. 

“Learning more shape-based nouns seemed to affect both how many words they knew and also their grammar skills and other aspects of language,” says Perry, the first author on the paper and an associate professor in the Department of Psychology. “Especially for the children with cochlear implants, it was such a strong predictor that even three years later, we were able to account for some of the differences in their language skills.” 

The data used in this study was collected as part of the Childhood Development after Cochlear Implantation Study, a national, multi-site longitudinal study. The researchers analyzed data on the language abilities of young children with cochlear implants prior to their implantation surgery and every six months after implantation. They also looked at data on children with normal hearing who were recruited from preschools. 

Understanding Shape-Based Nouns

The study, which was supported by the National Institute on Deafness and Other Communication Disorders of the National Institutes of Health, found that the children with a larger proportion of shape-based nouns in their vocabularies shortly after implantation had larger vocabularies at a year, two years, and three years after implantation. They also scored higher on standardized tests of other language abilities, and they were more likely to have caught up with their peers with normal hearing. 

Previous studies have indicated the importance of shape-based nouns in the language development of children with normal hearing, and that picking up on this pattern in early-learned English vocabulary can help children to acquire new words. But prior to this study, little was known about the role of shape-based nouns in the language development of children with cochlear implants. 

“This is a real experiment in nature showing that the types of words a child knows shape their language development,” says Messinger, a professor in the Department of Psychology. “It’s remarkable that these effects were strongest for cochlear implant users, perhaps because shape-based nouns guided their word learning after implants gave them access to hearing.” 

Further Reading

The findings suggest that knowing shape-based nouns facilitates children’s language development and may help to make up for initial language delays in children with cochlear implants. 

“While cochlear implants have become the standard of care for children with bilateral severe to profound hearing loss, there continues to be significant variability in their spoken language development,” says Cejas, a professor and the director of family support services in the Department of Otolaryngology at the Miller School of Medicine. “Our work highlights a potential avenue for intervention that may aid in closing the vocabulary and language gap that exists for some of these children.” 

Although the study found an association between the initial proportion of shape-based nouns in a child’s vocabulary and later language development, the researchers say further investigation is needed to establish a causal link. 

They also noted that they don’t yet know why some children had a larger proportion of shape-based nouns in their vocabulary than others. 

“Both within the group of children who have cochlear implants and children in general, we do see differences in terms of what words children learn first,” Perry says. “I think figuring out where those differences come from will be important to knowing how to best support all learners.”

Takeaways:

1. Young will deliver the annual Niparko Memorial Lecture at CI2025, highlighting her research on using pre-surgical brain anatomy and AI to improve language outcomes for children with cochlear implants.
2. The Niparko lecture honors the legacy of Dr. John K. Niparko for his contributions to cochlear implant research and clinical care.
3. CI2025 Boston will feature multi-disciplinary insights for professionals across fields like audiology, otolaryngology, speech pathology, and engineering, with registration opening Dec. 4, 2024.

American Cochlear Implant Alliance (ACI Alliance) announced that Nancy M. Young, MD, FACS, will deliver the annual Niparko Memorial Lecture at CI2025 Boston, on “Language Prediction to Improve Outcomes for Children with Cochlear Implants.” 

The Niparko lecture was established to recognize Dr John K. Niparko’s enduring commitment to cochlear implant research and clinical care and honor his significant contributions to the field. 

About the CI2025 Speaker

Nancy M. Young, MD, FACS, FAAP, is the Lillian S. Wells Professor of pediatric otolaryngology at the Northwestern University Feinberg School of Medicine and Fellow of the Knowles Hearing Center of Northwestern. She is head of the section of otology and neurotology in the Division of Otolaryngology, and medical director of Audiology & the Cochlear Implant Programs at the Ann and Robert H. Lurie Children’s Hospital of Chicago. She is president-elect of the American Otological Society, a member of the Board of Directors of the Hearing Health Foundation, and was a founding board member of the ACI Alliance. 

Further reading: ACI Alliance Launches Blog to Support Understanding of Cochlear Implants

Young leads a multi-center study supported by NIH/NIDCD to develop individual child level language prediction after cochlear implantation using pre-surgical brain anatomy derived from magnetic resonance imaging and AI-enabled analytical methods. 

The goal of this research is to develop a predict-to-prescribe method to improve language outcome by implementation of effective individualized behavioral therapy. She is also principal investigator of the lead site of a multi-center sponsored FDA clinical trial to expand pediatric indications for the MED-EL Synchrony implant system. 

CI2025 Boston: Conference on Cochlear Implants will be held April 30 – May 3, 2025, at the OMNI Seaport Hotel in Boston, Massachusetts. The conference will foster dissemination of multi-disciplinary scientific information applicable to audiologists, physicians, speech pathologists, psychologists, scientists, engineers, educators, students, and others involved in cochlear implantation. Conference registration opens on Dec. 4, 2024 with discounted rates available for ACI Alliance members and early bird registrants.

Featured image: Nancy M. Young, MD, FACS, FAAP. Photo: ACI Alliance

By Jodie Nelson, MAudA; Angela Pelosi, MBA, MAudA; Kaan Bulut, MSc; Laura Jagoda, PhD

Introduction: Audiology Insights from Large-Scale Data Analytics

The analysis of large data sets has become a fundamental part of medical device development throughout the healthcare sector. Insights derived from these analyses have proven instrumental in numerous applications, enabling the prediction and resolution of health-related issues before they escalate, or forecasting pathways toward the improvement of patient outcomes.^1-3 Data analytics of this nature have also been used to evaluate the efficacy of medical treatments, clinical protocols, and provide patients with insights that empower them to take a more active role in managing their health.^1-3

Data analytics have also proven valuable in audiology, providing insights about adherence to clinical guidelines, types of hearing loss managed with hearing aids, hearing aid use patterns, and the programming of hearing aid features. At Phonak, we use these analyses to provide valuable insights into patterns of hearing aid fittings and usage across various client demographics and levels of hearing loss. The objective of the project summarized here was to gain a deeper understanding of fitting and usage patterns among pediatric users up to 18 years. This was achieved by analyzing a substantial number of pediatric hearing aid fitting files.

Data Collection

Data are collected for every fitting undertaken with the Target software in alignment with global legal and privacy requirements; see the EU Medical Devices Regulation (Regulation (EU) 2017/745 of European Parliament and the Council of 5 April 2017 on medical devices) and 45 C.F.R. § 164.501 et seq.  Consent for the collection of this data is discussed in the applicable Privacy Notice of the Target fitting software, including those standards of data collection concerning an end user/patient.

During this study, we collected data pertaining to U.S. hearing care professionals’ utilization of the Target software and particularly its use with pediatric recipients of a Phonak hearing aid. All data used in this study has been de-identified in compliance with United States’ Health Insurance Portability and Accountability Act of 1996, as amended, and its implementing regulations (collectively, “HIPAA”) (see 45 CFR § 164.514(b)(2)).

Each pediatric client’s data comprises two categories—fitting logs and data logs. Fitting logs encompass all information related to the hearing aid fitting found in a Target session. Conversely, data logs are collected each time the hearing aid connects to Target. The fitting state of the hearing aids at the end of each session is sent to a central server, where the fittings can be subjected to filtering and analysis.

Methodology

Once consent is obtained through agreement with the Target privacy policy, all programming adjustments and navigation in the software are aggregated for analyses. During this project, a total of 19,201 fitting files were examined. These data were sourced from the United States and included fittings with Phonak Marvel, Paradise, and Lumity hearing aids. The fittings were conducted within a specific timeframe between February 26, 2024 and June 13, 2024. The target demographic for this study comprised children and teenagers under the age of 18 years, who had a hearing loss ranging from mild to profound, per the World Health Organization (WHO) definition.⁴

Results: Distribution of Hearing Loss Levels and Age Group

Data provided detailed insight into the audiological profiles of pediatric clients. Throughout this article, the data are frequently presented in relation to four distinct age groups: Infants and young children (0-3 years), young school-aged children (4-8 years), adolescents (9-12 years), and teenagers (13-18 years). These age groups correspond with the four Junior Modes available in the Target programming software. Figure 1 illustrates the distribution of data files gathered over this four-month period, demonstrating a balanced representation across the four age groups.

The gender of participating children was evenly distributed with male (47%), female (43%), and undefined or non-binary (10%). A majority of children in this data set have mild to moderate hearing loss (64%), with severe and profound representing 14% and 7%, respectively. Considering the WHO classification of ‘Normal’ hearing as being up to 25 dB HL, it is unclear from these data whether children in the ‘Normal’ hearing category (15%) have a mild hearing loss or if they have a unilateral hearing loss.

Breaking the data down further shows the distribution of hearing loss across the Junior Mode age groups. Results confirm that the percentage of children with mild or moderate hearing loss is consistent across the age groups. Of note is a decrease in the percentage of children with profound hearing loss in the three older age groups. The shifting proportions of hearing loss severity may reflect the availability and uptake of cochlear implantation in the U.S.

Distribution of Form Factors

The needs of a child with hearing loss may be addressed by hearing aids of different form factors. Best practice guidelines recommend that younger children are fit with behind-the-ear (BTE) hearing aids for safety, maximum flexibility, usability, and maintenance options.^5,6 For children aged 8 years and older a receiver-in-canal (RIC) device can be considered.⁷ With advances in technology that include direct connectivity to remote microphones and the availability of shorter receiver lengths, selection of the RIC form factor is becoming more common for older children.

Analysis of the data shows most children are fit with BTE devices (see Figure 3). BTEs are fit across all age groups with the number of RICs increasing from 9 years of age. Teens are fit equally with BTEs and RICs, which may relate to increased interest in cosmetically appealing devices and an adolescent’s ability to care for their own hearing devices.

Distribution of Performance Levels

In the U.S., Phonak hearing aids are available in three technology levels: premium (90), advanced (70), and standard (50). The selection of a technology level is influenced by various factors that may include: options for financial reimbursement (e.g., Medicaid or insurance options), parental preference, or hearing care professional (HCP) recommendation. The majority of pediatric hearing aids in the U.S. are the standard technology level (51.3%), followed by advanced (32.3%) and premium (16.4%), respectively.

This pattern of technology level distribution remains relatively consistent across all four age groups as shown in Figure 5; although, there is a trend toward increasing the percentage of premium technology level fittings with older children.

Fitting Formulas

Reports from the Outcomes of Children with Hearing Loss (OCHL) study provided three guiding principles for protection against delay in speech and language. These principles indicate the need for (1) hearing aids fit to prescriptive targets, (2) wearing hearing aids a minimum of 10 hours per day, and (3) a language-rich environment provides children with the means to maximize their speech and language development.⁸

The prescription of any hearing aid begins with the selection of a validated formula that calculates gain and output targets based on a range of audiology diagnostic variables. In the case of Pediatric prescriptions, the targets are most commonly derived from the DSL v5 or the NAL- NL2 algorithms, each of which has unique settings for children. These settings account for pediatric ear canal acoustics and provide output levels that are estimated to match children’s listening needs.

The data in Figure 6 show that the majority of children are fitted with a DSL v5 pediatric prescription. This suggests that many HCPs are beginning their prescriptive routine with the selection of an appropriately validated pediatric prescription. However, as children get older, there is a small increase in the number of children who are fitted with Phonak’s default prescriptive formula for adults.

Following a series of studies comparing speech intelligibility and children’s preferences,^9,10 the default program Phonak recommends in consultation with the Phonak Pediatric Advisory Board is AutoSense Sky OS (ASOS Sky). This is an automatic program that uses environmental classification to detect different listening scenarios and automatically adapt signal processing for the situational listening needs (e.g., speech in quiet, speech in noise, streamed audio inputs, etc). Figure 7 shows that the majority of fittings use the recommended ASOS Sky as the start-up program, with some instances of an alternate start-up program.

Daily Wearing Time

For children with hearing loss, the routine use of hearing aids is essential to successful development. Each additional hour of wear time returns a significant benefit in developmental progress. However, the data indicate that wearing time is influenced by degree of hearing loss, the age of the child, and device performance level. Each of these factors, combined with the realities of daily life, should be considered when discussing expectations for daily use.¹¹

Figure 8 shows that median use times increase systematically with increasing age during childhood. Within these data, Premium level devices were worn 1hr 19 mins longer per day than Standard level devices and 55 mins longer than Advanced level devices. Note that this latter trend was observed in the data set but is not shown in Figure 8.

Research has shown that the daily use of hearing aids can be increased by ensuring that parents and caregivers have a clear understanding of a child’s hearing loss, hearing aid maintenance, and expectations for daily use.¹² The myPhonak Junior mobile application provides parents with a report of wearing time on a daily and monthly basis, while also including a summary of sound environments experienced throughout the day. This information can be used to support counseling conversations including opportunities for use of Roger.

Conclusion

The use of large-scale data analytics is a relatively new occurrence in the field of audiology. Only in recent years has the technology existed to centralize data collected from hearing aid programming software and hearing aids worn during daily life. With insights collected from these large data sets, HCPs can reinforce their clinical decisions and develop more personalized treatment plans, a benefit for both pediatric and adult patients. In the data reviewed here, we see how trends in the prescription of pediatric hearing aids varied across 19,201 different fittings.

Of particular interest was a decrease in the number of patients with profound hearing loss after the age of 3 years, possibly relating to the availability of cochlear implants in the United States. Next was a systematic increase in the number of RIC fittings after the age of 8 years. It was also apparent that most pediatric fittings begin with selection of the DSL v5 or NAL-NL2 prescriptions and that a significant proportion of fittings start up in AutoSense Sky OS or AutoSense OS. Finally, median daily use times increased with age of the hearing aid wearer and with use of a higher-tier technology level.

Each of these insights is drawn from a very large data set, which will continue to be mined and support the development of future Phonak technology. In this summary, it is clear that HCPs providing pediatric services are following key elements of best practice in the treatment of their patients. They are also adapting the treatment plan to meet the needs of children as they age. It’s also clear that providing a flexible portfolio of prescription hearing aids is essential, as access to different technology levels and form factors play a role in long-term success. 

About the authors: Jodie Nelson, MAudA, is senior product audiologist for Pediatrics at Phonak Headquarters, Staefa, Switzerland. Angela Pelosi, MBA, MAudA, is senior director, global audiology and customer success at Phonak Headquarters, Staefa, Switzerland. Kaan Bulut, MSc, is senior solution experience manager at Phonak Headquarters, Staefa, Switzerland. Laura Jagoda, PhD, is an audiological researcher in Sonova R&D, Staefa, Switzerland.

Original citation for this article: Nelson J, Pelosi A, Bulut K, Jagoda L, Using Large-Scale Data Analytics to Understand Pediatric Hearing Aid Prescription and Use. Hearing Review. 2024;31(10):16-19.

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