When Flexibility Isn’t a Gift: How Ehlers-Danlos Can Disrupt the Auditory System

When people think of Ehlers-Danlos Syndrome (EDS), they usually picture joint hypermobility, stretchy skin, and chronic pain. But one of the lesser-known impacts of EDS is how it can affect the auditory system—and not just in ways that show up on a standard hearing test.

We tend to treat hearing like it’s binary. You either have hearing loss, or you don’t. But for many people with EDS, especially children, their audiogram looks fine—yet they’re clearly struggling. They might complain of sensitivity to sound, fatigue in noisy environments, difficulty keeping up with verbal instruction, or frequent misunderstandings. And when they do, those symptoms are often brushed off as behavioral, attention-related, or emotional. In reality, their auditory system just isn’t working the way it’s supposed to.

Hearing depends on a delicate system of pressure regulation, mechanical movement, and reflexive protection. That means the eardrum has to vibrate with just the right tension. The ossicles—the tiny bones in the middle ear—need to move efficiently, with stable joints and well-tuned ligaments. And the acoustic reflex, which helps dampen loud noises, needs to fire quickly and reliably to protect the cochlea and brain from overstimulation. But in EDS, connective tissue doesn’t behave normally. The ligaments can be too lax, the membranes too stretchy, and the reflexes too inconsistent.

It’s like trying to control a marionette puppet with strings made of soft, unpredictable elastic. One day, everything looks normal. The next, the whole puppet collapses. The movement is inconsistent, the signal is unstable, and when that happens in the ear, what reaches the brain is already degraded.

That instability doesn’t always show up on traditional hearing tests. Tympanometry might look borderline. Reflexes may be present one moment and absent the next. But what the child experiences is fluctuating clarity, hypersensitivity to sound, and difficulty filtering speech in background noise. In other words, they look exactly like a child with auditory processing disorder (APD)—and they often get tested and diagnosed as such.

And that’s the problem. Because while APD is a real and serious condition, we still treat it as if it’s purely central—something in the brain that can’t be traced to the ear. But if a mechanical issue like EDS can result in the same symptoms—and even produce similar scores on behavioral tests—then we need to rethink the foundation of the diagnosis.

It doesn’t make auditory processing issues less real. It makes them more physiological, not less. More deserving of intervention. More deserving of accommodations. And more deserving of a spot within the broader category of hearing impairment—because they are.

In some cases, what looks like APD might even be a form of auditory neuropathy spectrum disorder (ANSD)—a condition where the ear detects sound normally, but the signal doesn’t travel properly to the brain. ANSD can coexist with connective tissue disorders, especially when the cochlear membranes or synapses between hair cells and the auditory nerve are compromised. The child may pass a hearing screening but still struggle with clarity, timing, and fatigue. And if we mistake this for a learning or behavioral issue, we risk missing the real problem—and the right interventions.

Children with EDS who present like they have APD are not imagining their symptoms. Their ears are simply not holding together. Their reflexes aren’t kicking in when they should. Their middle ear system may be functioning well enough on a quiet day but falling apart in the chaos of a cafeteria or classroom. And if we only test them under ideal conditions, we’re going to miss it.

In fact, many children with EDS may go years without ever being correctly identified, misdiagnosed instead with attention issues, anxiety, or even laziness. But if the auditory system itself is unstable—if the “marionette” is tripping over its own strings—then the brain is left trying to make sense of a signal that was compromised before it even arrived.

This is why we must stop seeing auditory processing as a singular diagnosis. There’s no one central disorder. There are multiple auditory processing disorders—and they don’t all start in the brain. Some are born in the eardrum. Some in the ossicles. Some in the reflexes or the auditory nerve. Some in how the brainstem or cortex handles fatigue, overload, or distortion.

And still, despite all this, we don’t have a gold standard for diagnosing APD. As Dr. Andrew Vermiglio has pointed out, most tests used to diagnose APD are validated only against other APD tests—not against an independent, verifiable reference. It’s circular. And that means a child can “fail” a test and get a diagnosis, while another child with equally severe symptoms passes and is told nothing’s wrong.

That’s why we have to look broader. We need better screening. We need comprehensive case histories. We need to ask about fatigue, hypersensitivity, and variability over time. And we need to bring in other professionals who see the child from a different angle.

Because audiologists, on their own, often don’t follow the child long enough to see the full picture. Speech therapists may pick up on the expressive and receptive language delays. Occupational therapists may notice the sensory processing sensitivities. Teachers might observe the difficulty focusing, or the odd behavior in noisy environments. Psychologists might see anxiety, school avoidance, or shutdowns. And if those professionals aren’t sharing what they’re seeing—if we aren’t collaborating—we miss the diagnosis entirely.

This is why interprofessional communication matters so much. No one has the full picture. But if each of us is only measuring one part of the elephant, we end up defining the whole child based on our narrow slice of expertise—and that’s how they fall through the cracks.

We also have to stop waiting. Early intervention is everything. The longer we delay access to accommodations and treatment, the more likely a child is to experience auditory deprivation, speech and language delays, poor phonemic awareness, and academic struggle. That damage is preventable—but only if we recognize the signs early and act on them without gatekeeping.

And most of all, we need to stop pretending that auditory processing issues are vague or behavioral. They are real. They are physiological. They are often invisible on a basic hearing test—but they are no less impactful. And they deserve our attention, our respect, and our support.

If we treat auditory processing disorder like a behavioral quirk, we miss the opportunity to protect a child’s access to sound, language, and learning. But if we treat it like a hearing condition—as something real and measurable and worthy of intervention—we have a chance to change their entire trajectory.

We owe them that chance. And we owe each other the kind of collaboration that makes that possible.

Visual Description:

This educational cartoon-style image is split into two halves.

On the left side, there’s a labeled drawing of the human ear, shown in profile. Strings are drawn connecting various parts of the ear—including the eardrum, ossicles, and cochlea—visually resembling the strings of a marionette. The strings appear slack and unstructured. Bold black text above reads “Ehlers-Danlos Syndrome.” Below the ear, a caption says: “Loose and overly stretchy connective tissue causes the ‘strings’ of the auditory system to be slack.”

On the right side, there is a cartoon marionette puppet sitting on the ground with a sad expression. The puppet’s strings are drooping and disconnected, suggesting poor control or support. A hand-drawn marionette control bar floats above its head. The puppet’s limbs are bent and its posture is slouched, emphasizing fragility. Next to the puppet, the caption reads:

“or Auditory Processing Disorders?

Breakdown can appear inconsistent, leading to disbelief.”

The image uses warm tan and brown tones, giving it a slightly vintage, medical poster feel. The overall message visually connects Ehlers-Danlos Syndrome and auditory processing breakdowns, highlighting how inconsistent auditory system function—caused by connective tissue issues—can be misunderstood or dismissed.

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References

Chudley, A. E., & Rosebush, P. I. (1994). Neurological findings in Ehlers–Danlos syndrome. American Journal of Medical Genetics, 53(1), 30–36.

Weir, F. W., Wallace, S. A., White, D. R., Hatch, J. L., Nguyen, S. A., & Meyer, T. A. (2016). Audiologic outcomes in Ehlers-Danlos syndrome. Otology & Neurotology, 37(6), 727–732.

Sininger, Y. S., & Oba, S. I. (2001). Auditory neuropathy/dys-synchrony and its perceptual consequences. Trends in Amplification, 5(1), 1–22.

Sangani, N., & Cederberg, C. (2014). Ehlers-Danlos Syndrome presenting with bilateral auditory neuropathy: A case report. Case Reports in Otolaryngology, Article ID 652583.

Vermiglio, A. J. (2018). Is there a gold standard for diagnosing auditory processing disorder? Audiology Online. https://www.audiologyonline.com/.../20q-auditory...