FROM SILENCE TO SOUND: A 16-MONTH-OLD’S REMARKABLE HEARING STORY
Opening Overview
Some weeks in hearing news are dominated by another new hearing aid.
This week offers something much bigger.
A 16-month-old Alabama boy named Everett traveled with his family to Boston for a treatment that would have sounded like science fiction only a few years ago: doctors placed a working copy of a defective gene directly into his inner ear in hopes of allowing him to hear naturally.
Meanwhile, another revolution is occurring at the opposite end of the hearing spectrum. Smartphones and everyday earbuds are increasingly becoming part of hearing care, while a new Houston program is putting OTC hearing aids directly into the hands of seniors and asking them what works — and what doesn’t.
And as hearing care receives more mainstream attention, consumers are also encountering increasingly bold claims linking hearing aids with dementia prevention.
This week’s stories offer a useful reminder:
Better hearing can be life-changing. But consumers still need to distinguish remarkable medical advances from promising technology — and promising research from proven fact.
❤️ A 16-Month-Old Gets a Chance to Hear Naturally
Everett Tynes was born deaf.
The Alabama toddler has a rare genetic condition involving mutations in a gene called OTOF. That gene normally produces otoferlin, a protein necessary for the inner ear to transmit sound information to the brain.
Everett’s ears could detect sound, but the signal wasn’t successfully making the rest of the journey.
Traditional hearing aids couldn’t solve that problem.
After genetic testing identified the cause, Everett’s parents discovered an extraordinary new possibility: Otarmeni, the first gene therapy approved by the FDA specifically for genetic hearing loss.
On August 6, the 16-month-old became the first patient at Boston Children’s Hospital to receive the treatment following its approval.
Doctors delivered a functioning copy of the OTOF gene directly into Everett’s cochlea. The goal is for cells in his inner ear to begin producing the missing protein — potentially allowing sound information to reach his brain naturally.
This isn’t an experimental treatment being casually described as a “breakthrough.”
The FDA granted Otarmeni accelerated approval on April 23 for children and adults with a very specific form of severe-to-profound or profound hearing loss caused by confirmed mutations in both copies of the OTOF gene. In the clinical evidence reviewed by the FDA, 80% of evaluable patients experienced improved hearing. Continued approval is contingent on additional evidence confirming the clinical benefit.
Everett’s family also illustrates an important side of medical breakthroughs that headlines can overlook.
The manufacturer is providing the therapy without charge to U.S. patients, but Everett’s family still had to travel roughly 1,200 miles and remain near Boston Children’s for several weeks. Their temporary housing alone reportedly cost about $10,000.
HArC Insight: Gene therapy isn’t about replacing hearing aids for millions of adults with age-related hearing loss. Otarmeni treats one rare genetic cause of profound hearing loss. But successfully restoring a damaged hearing pathway rather than simply compensating for it represents an extraordinary change in what may someday be possible.
Read the FDA’s information about Otarmeni
📱 Innovation: Your Smartphone Is Becoming Part of Your Hearing System
Last week we covered Samsung receiving FDA clearance for a hearing-aid feature that allows compatible Galaxy Buds to serve as OTC hearing aids.
This week, the bigger story is becoming clearer.
Samsung isn’t an isolated development.
Apple’s AirPods Pro entered this territory earlier. Samsung has now followed. Dedicated OTC hearing aids increasingly depend upon smartphone apps for hearing tests, personalization and controls. Prescription hearing aids have used smartphone connectivity for years.
The boundary between consumer electronics and hearing technology is getting blurrier.
An August 18 technology analysis examined why the smartphone may be particularly important to the next generation of affordable hearing devices.
Instead of requiring every function to fit inside a tiny device behind or inside the ear, smartphones can provide processing power, user interfaces, hearing-screening tools and personalization.
That could make hearing assistance easier to try — especially for someone who already owns compatible earbuds.
But there is an important distinction.
An OTC hearing aid is not simply an amplifier.
FDA-regulated OTC hearing aids must meet specific requirements and are intended for adults 18 and older with perceived mild-to-moderate hearing loss. They are not intended to address every degree or cause of hearing difficulty.
And a smartphone hearing test is not equivalent to a comprehensive evaluation performed by an audiologist.
HArC Insight: The smartphone may become as important to some hearing solutions as the device in the ear. That could lower barriers to trying hearing assistance — but convenience shouldn’t be confused with comprehensive hearing care.
👂 OTC Hearing Aids Meet the People Who Actually Have to Use Them
There’s another interesting OTC story this week — and it isn’t really about a new product.
It’s about usability.
On August 18 and 19, OTC hearing-aid company Ceretone partnered with Easter Seals Greater Houston, Catholic Charities and AmeriCorps Seniors to put its Core One Pro hearing aids into the hands of older adults.
Participants received the devices at no cost and were allowed to keep them.
But the companies wanted something in return:
Feedback.
The seniors were asked about unboxing, setup, fit, comfort, sound quality and everyday usability. Many were trying hearing aids for the first time. Their feedback is expected to go back to Ceretone’s product-development team.
The information comes from a company-issued announcement, so this shouldn’t be interpreted as independent research demonstrating the performance of the hearing aids.
But the concept is worth watching.
The success of OTC hearing aids depends upon far more than sound quality.
Can someone open the package and understand what to do?
Can they insert the devices?
Can they pair them with a phone?
Can they make adjustments without becoming frustrated?
Can they tell whether the devices are working correctly?
Those questions become particularly important when the traditional hearing-care professional is removed from the process.
HArC Insight: Making a hearing aid available without a prescription solves only one barrier to hearing care. If consumers can’t comfortably set up, adjust and maintain the device themselves, affordability alone won’t make OTC hearing aids successful.
🧠 Consumer Watch: Do Hearing Aids Prevent Dementia?
If you follow hearing news, you’ve probably encountered some version of this claim:
“Untreated hearing loss causes dementia.”
Or:
“Hearing aids can prevent dementia.”
A detailed review published this week provides a useful reason to be careful with both statements.
Researchers have repeatedly found an association between hearing loss and cognitive decline. There are also several plausible explanations.
People who struggle to hear may become less socially engaged.
The brain may have to devote additional resources to understanding speech.
Hearing loss and cognitive decline could also share underlying biological causes.
But an association does not prove that hearing loss causes dementia — or that wearing hearing aids will prevent it.
The large ACHIEVE randomized trial is particularly instructive.
Among the overall study population, hearing intervention did not significantly reduce three-year cognitive decline compared with the control group. A higher-risk subgroup did appear to benefit, which is scientifically important and deserves further study.
That is much more nuanced than saying, “Hearing aids prevent dementia.”
None of this diminishes the value of treating hearing loss.
Hearing aids can improve communication, help people remain socially engaged and make everyday life easier. Those are substantial benefits without promising something science hasn’t yet proven.
HArC Consumer Tip: Be skeptical of hearing-aid advertising that implies a device has been proven to prevent Alzheimer’s disease or dementia. Ask what specific research supports the claim — and whether the research demonstrates causation or merely an association.
Read the August 18 evidence review from BBC Science Focus
💡 Practical Guidance: Hearing Aid, OTC Hearing Aid or Earbud?
As the categories begin to overlap, here’s a simple way to think about your options.
Hearing-assistance earbuds may be worth considering when you have perceived mild-to-moderate difficulty and want a relatively inexpensive way to explore whether amplification helps.
Dedicated OTC hearing aids may make sense when you have perceived mild-to-moderate hearing loss, want devices designed primarily for all-day hearing assistance and are comfortable handling setup and adjustments yourself.
Prescription hearing aids may make more sense when your hearing loss is more significant, your listening needs are complicated, or you want professional testing, fitting, verification and ongoing support.
And none of those should substitute for medical evaluation when you experience warning signs such as sudden hearing loss, a substantial difference between ears, persistent ear pain or drainage, or significant dizziness.
The arrival of more choices is good news.
Knowing which problem you are trying to solve remains the important first step.
💙 What This Means for You
This week shows just how dramatically the definition of “hearing technology” is changing.
At one end, doctors can now deliver a functioning gene directly into the inner ear of a child born with a particular form of genetic deafness.
At the other, a pair of earbuds someone may already own can potentially provide FDA-cleared hearing assistance for mild-to-moderate hearing loss.
Between those extremes are prescription hearing aids, OTC devices, smartphone apps, audiologists, remote care and emerging technologies we haven’t yet imagined.
More choices should ultimately be good for consumers.
But more choices also make good information increasingly important.
The right question isn’t:
“What’s the newest hearing technology?”
It’s:
“What technology is appropriate for my hearing loss, my lifestyle and the way I want to receive care?”
Closing Thought
Everett’s story is a remarkable reminder of how quickly assumptions can change.
For most of human history, a child born with his particular genetic condition would remain deaf.
Today, doctors can identify the responsible gene — and in carefully selected patients, deliver a working copy directly to the inner ear.
At the same time, millions of adults with much more common hearing loss can choose among technologies ranging from sophisticated prescription hearing aids to OTC devices and everyday earbuds.
There has probably never been a more exciting time in hearing science.
But technology is only useful when it helps a real person connect with the world around them.
And ultimately, that remains the measure that matters.
HArC I Hear™ shares hearing news and consumer information for educational purposes. It is not a substitute for evaluation, diagnosis or advice from a qualified hearing-care or medical professional.