First, Fix the Signal
As an audiologist, I want to share something that is often overlooked but deeply relevant for families navigating Ehlers-Danlos Syndrome. We all know that EDS affects connective tissue throughout the body, but what many do not realize is that this includes the tiny, delicate structures of the ear. When these structures become unstable or inconsistent, the result can be what looks like a language delay, auditory processing disorder, or even sensory overwhelm. But these issues may not stem from how the brain processes sound. Instead, they may begin with unreliable input from the ears themselves.
I recently worked with a young girl I will call Lily, whose family has a strong history of EDS. She herself shows clear signs of the condition, including joint hyperflexibility and bowel issues. Despite having “normal” hearing on a basic audiogram, she showed marked language delays, particularly in following directions, articulation, and expressive language.
We decided to trial low-gain hearing aids to see if consistent access to sound could stabilize her input and reduce listening fatigue. The devices were programmed to provide about 10 decibels of gentle amplification for soft input, with significantly less gain for moderate or loud sounds. When her environment became loud, the hearing aids would essentially turn off, helping protect her from overload. We also applied mild frequency compression to make high-pitched consonants more accessible without increasing harshness.
The earlier we intervene, the better the chance we have to prevent the brain from adapting to a distorted signal. Without consistent access to sound, children may develop compensatory patterns that are difficult to unlearn later. What begins as a mechanical issue in the ear can turn into a functional reorganization in the brain, especially during early development.
Some might ask why we did not begin with auditory training. The answer is simple. In Lily’s case, the issue was not yet a true auditory processing disorder. It was a physiological disruption in sound transmission, and we needed to address that first. She had already been in speech therapy for quite some time, but her progress had been slow and limited. Auditory training can be extremely valuable when the brain has adapted to distorted input and needs help reorganizing. But Lily had a history of difficulty following directions, both during formal hearing tests and in school settings.
Starting with an approach that required sustained attention, listening compliance, and verbal repetition would have been unrealistic and potentially frustrating for her. It would have been like prescribing physical therapy before setting a broken bone. We chose to begin with a physiological solution for a physiological problem, knowing that if we could intervene early enough, we might prevent the need for weeks or months or even years of remediation later.
People often express concern about the safety of using low levels of amplification in children with normal hearing. I would like to offer some context. The devices used in Lily’s case were low-gain programmed hearing aids, and the programming itself was specifically designed to be safe for children with normal hearing thresholds. These settings are not meant to make everything louder, but rather to provide subtle, consistent access to soft sounds while reducing listening effort and preventing distortion.
In fact, devices like the Phonak Roger Focus II, which are commonly fitted to children with normal hearing in school settings, provide nearly double the gain we were using. Yet these systems are widely accepted and used across classrooms to support auditory access.
The difference in Lily’s case was that our approach was more individualized. She had full control over volume and listening mode, which allowed her to safely regulate her own soundscape in real time. What we offered was not only gentler but also more adaptable, helping her feel both supported and empowered.
The most important element of her treatment was giving her more control over her own soundscape. She could adjust the volume herself and switch between listening modes, which gave her the ability to create a more predictable and unthreatening environment. In addition to her main program, she had access to a pink noise setting, which she could activate when overstimulated. Her mother also used a remote microphone during reading time and in situations like car rides, where Lily might otherwise struggle to hear or feel left out. Lily was able to turn the microphone volume up or down depending on how connected she wanted to feel.
The change was immediate. Within weeks, her speech-language pathologist reported major improvements in articulation. Lily became more talkative, playful, and socially engaged. Her family noticed she was clearer in conversation, even over the phone.
Completing standard hearing tests had been difficult for her in the past, but by the middle of the trial, she was following directions much more easily and was able to complete a full audiometric evaluation. She repeated words clearly and responded to tones just as a cooperative adult would, despite her young age.
Her mother, an educator, understood the importance of carefully observing whether a treatment trial was truly making a difference. We had discussed ahead of time that one of the best ways to evaluate this is to remove the intervention briefly and see whether any of the original challenges return. When she removed the hearing aids for a day, Lily’s expressive language skills held, but her energy dropped significantly. She spent most of the day self-isolating, asking for her hearing aids back. When she finally got them, she was ecstatic and visibly relieved. As soon as the aids were back in, she became more talkative, more energetic, more engaged. Her brain had adapted to clarity, and she did not want to return to a world that felt harder to decode.
Many children with EDS show overlapping traits commonly associated with autism, misophonia, hyperacusis, and even oppositional defiance. These children may appear irritable, inattentive, or reactive, but they are often struggling to process inconsistent auditory input. When the signal changes from moment to moment, it becomes hard to focus, hard to filter out distractions, and even harder to feel safe. Hyperacusis may develop when the brain is not protected from sudden loud sounds.
Misophonia can emerge from chronic discomfort and emotional hypersensitivity to particular sound patterns. In other children, especially those on the autism spectrum, this ongoing discomfort can contribute to fatigue, shutdown, or behaviors that may look like defiance but are really expressions of nervous system distress. What is often labeled as oppositional or dysregulated may, in part, be the result of living in a world that is too loud, too unclear, and too inconsistent.
For Lily, some of those challenges may have been on the horizon. She was already struggling with bowel symptoms and early signs of anxiety. It is impossible to know how much of that was influenced by delayed language and inconsistent auditory input, but it is certainly possible that her sensory world felt unpredictable and overwhelming. If she continues to gain better access to language and more control over her environment, starting with her auditory system, she may also experience more emotional stability. When children feel safe, understood, and in control of even one part of their sensory experience, it often has ripple effects far beyond speech and hearing.
Lily’s case reminds us that children are not difficult by nature. They are often doing their best with the input they are given. Once that input becomes consistent and reliable, so much of the struggle begins to lift. What she needed most was not more instruction, more pressure, or more compliance training. She needed a clear, stable signal that her brain could trust. Once she had that, everything else started to change.
This article does not include formal citations. However, if you are interested in learning more about EDS, misophonia, hyperacusis, or the role of stable auditory input in child development, I have written extensively about these topics in other posts on my blog. This story is based on 25 years of clinical experience as an audiologist, working at the intersection of physiology, language, and sensory regulation.
Visual Description:
A young girl in a bright red polka-dot dress and dark pants stands on a sandy or concrete boat launch at a lakeside. In front of her, a boy in a gray shirt and shorts is walking toward the water’s edge. The lake is calm with gentle ripples, surrounded by trees and greenery under a clear sky. The scene has a peaceful, playful summer-day atmosphere.
