Ross Hauser, MD
People with hypermobile Ehlers-Danlos syndrome (hEDS) and hypermobility spectrum disorders (HSD) frequently experience headaches, migraines, neck pain, dizziness, and symptoms of autonomic dysfunction. For many patients, these problems overlap so extensively that it becomes difficult to determine which diagnosis best explains the symptoms.
At Caring Medical, we see patients who have already been treated for migraine, temporomandibular joint dysfunction, postural orthostatic tachycardia syndrome (POTS), Chiari malformation, or suspected cerebrospinal fluid (CSF) leakage. Some also report neck clicking, a feeling that the head is too heavy, pain triggered by neck motion, visual symptoms, tinnitus, or worsening symptoms when upright.
It is very rare that a patient diagnosed with Ehlers-Danlos Syndrome will come in with one or two symptoms or health challenges. They will have many, sometimes dozens. These are the type of things we hear from people. Very likely these people will tell a story that sounds very familiar to your own.
I suffer from severe headaches. I also have severe TMJ. I had a lot of treatments for the TMJ. My doctors thought that if I could address the TMJ I could address my headache. After years of treatment and no success, my doctors finally realized that I have hEDS Ehlers-Danlos Syndrome. So now after chasing TMJ for years my doctors are telling me that my C1-C2 are hypermobile and out of place.
I have hEDS and the “only way out,” is an anterior cervical discectomy surgery which scares me. I have so many symptoms. Terrible hip pain, knee pain, shoulder pain, wrist pain for which I had a carpal tunnel surgery that failed. I have terrible neck pain, vision problems, and migraines. The headaches and migraines are now every days. I have also developed tinnitus along with dizziness.
Current research supports a careful, individualized approach. Migraine is common in hEDS, but it is not the only possible headache mechanism. In selected patients, cervical dysfunction or upper cervical instability may contribute alongside migraine, dysautonomia, altered CSF pressure, TMJ dysfunction, or other disorders. No single mechanism should be assumed without an appropriate medical evaluation.
Why are headaches common in hEDS and HSD?
hEDS is a connective tissue disorder characterized by generalized joint hypermobility, joint instability, pain, and other systemic features. HSD describes symptomatic hypermobility in people who do not meet the full diagnostic criteria for hEDS or another connective tissue disorder.
A 2026 review in Cephalalgia concluded that migraine is the predominant primary headache disorder in hypermobile populations. The authors also emphasized that spontaneous intracranial hypotension, craniocervical instability, Chiari I malformation, TMJ dysfunction, dysautonomia, POTS, and vascular conditions must sometimes be considered as secondary or contributing diagnoses. (1)
A separate 2024 review similarly described migraine as highly prevalent in EDS and HSD while warning that orthostatic headache, CSF leakage, medication-overuse headache, cervicogenic headache, Chiari malformation, and craniocervical pathology can produce overlapping presentations. (2)
The central message is that headache in hEDS is often multifactorial. A patient may have a true migraine disorder and a cervical pain generator at the same time. Likewise, dizziness may relate to migraine, POTS, an inner-ear disorder, medication effects, or cervical sensorimotor dysfunction. Identifying one diagnosis does not automatically exclude the others.
Migraine is the most common headache pattern
Migraine is a neurological disorder, not simply a severe headache. It can include throbbing or pulsating head pain, nausea, light and sound sensitivity, visual aura, and worsening with ordinary physical activity. Some patients also experience vestibular migraine, in which dizziness, motion sensitivity, or vertigo may be prominent.
Research predating the newer reviews found that migraine can begin earlier and be more burdensome in people with joint hypermobility. In a comparative study, patients with joint hypermobility syndrome or hEDS developed migraine at a younger average age, experienced more migraine days per month, and reported greater disability than migraine patients without hypermobility. (3)
Another retrospective study of 140 patients with hypermobility disorders found that 66% reported headache or neck pain. Among patients with a diagnosed headache disorder, migraine was the most common type. Migraine and cervical spondylosis frequently coexisted, raising the possibility that cervical pain contributed to the headache burden in some patients. (4)
These findings do not mean that every migraine in hEDS comes from the neck. They do suggest that clinicians should ask about neck pain, mechanical triggers, hypermobility, and other symptoms rather than treating the headache label in isolation.
For more information about overlapping dizziness and migraine symptoms, see our discussion of vestibular migraine and spontaneous vertigo.
When could the cervical spine be part of the headache?
A cervicogenic headache is head pain referred from structures in the neck. It is often accompanied by restricted or painful neck motion, tenderness, and headache provoked by particular positions or movements. The pain may begin in the upper neck or back of the head and travel toward the forehead, temple, or eye.
In hEDS, collagen-related ligament laxity can reduce passive joint stability. Muscles may then work harder to control the head and neck. This does not prove craniocervical instability, but it provides a reasonable basis for assessing cervical mechanics when headache is consistently associated with neck pain or motion.
The upper cervical region is especially complex. The occiput, atlas (C1), and axis (C2) permit a large proportion of head rotation and flexion-extension. Ligaments help restrain excessive motion and protect the spinal cord, brainstem, nerve roots, and vertebral arteries.
In a 2021 investigation, researchers compared myodural-bridge tissue and dynamic spinal-cord motion in patients with and without EDS. They reported abnormal collagen ultrastructure and increased or irregular spinal-cord motion in the EDS group, even when conventional imaging did not demonstrate craniocervical instability. The authors proposed—not proved—that laxity of spinal-cord suspension structures could contribute to neck pain and headache. (5)
Patients who want a broader explanation can read our article about cervicogenic headaches, migraine, and cervical instability.
Video: Cervical spine instability in the hEDS patient
In this video, Ross Hauser, MD, explains how ligament laxity in patients with hypermobile Ehlers-Danlos syndrome may contribute to upper-cervical instability and discusses the headaches, dizziness, autonomic symptoms, and other neurological complaints that can accompany it. These proposed relationships do not mean that every headache in a patient with hEDS originates in the cervical spine.
How the muscles in the back of your neck and the nerve pathways used to move your head, may be causing a tugging on your spinal cord and triggering headaches
I discuss this subject further in my article The myodural bridge and dural tension. A missing diagnosis of neurologic-like symptoms? In this article I explain, with supportive research, how the muscles in the back of your neck and the nerve pathways used to move your head, may be causing a tugging on your spinal cord. This tugging can occur at the myodural bridge, a thick, dense collection of connective tissue, that connects the muscles to the dura of the spinal cord.
The myodural bridge helps anchor the spinal cord during head and neck motions. When upper cervical instability is present, the tension in the suboccipital muscles is transferred to the upper spinal cord and brainstem via the myodural bridge. This can cause symptoms of sharp neck pain, headaches, autonomic storms, and a host of other symptoms.
Craniocervical instability diagnosis
Craniocervical instability (CCI) refers to excessive or pathological motion where the skull meets the upper cervical spine. Atlantoaxial instability involves abnormal movement between C1 and C2. These are not diagnoses that should be made from symptoms alone.
A systematic review of CCI in EDS found inconsistent diagnostic criteria and limited evidence across the available literature. The authors recommended that surgical treatment be considered only when a patient has severe symptoms, clear radiographic instability, and corresponding clinical findings. (6)
This uncertainty matters because headache, dizziness, brain fog, nausea, tinnitus, fatigue, and palpitations are nonspecific. They occur in many neurological, vestibular, cardiovascular, inflammatory, and medication-related conditions.
A 2024 retrospective surgical series reported improvement in headache, neck pain, syncope, presyncope, vertigo, fatigue, and other symptoms after occipitocervical fusion in 53 carefully selected patients with EDS and radiographically documented CCI. (7) These outcomes support the clinical significance of severe CCI in a selected surgical population. They do not show that fusion is appropriate for most people with hEDS and headache, and the uncontrolled design cannot establish how much improvement was attributable to surgery rather than selection, concurrent care, or other factors.
At Caring Medical, our evaluation is focused on determining whether a patient has signs of clinically meaningful neck pain and cervical instability, rather than assuming that hypermobility automatically means upper cervical instability.
Dysautonomia, POTS, and orthostatic headache
Dysautonomia is an umbrella term for disorders affecting the autonomic nervous system, which regulates involuntary functions such as heart rate, blood pressure, digestion, temperature control, and pupil response. POTS is characterized by excessive heart-rate elevation with standing in the appropriate clinical setting.
In a 2025 study of women with hEDS or HSD, more than half had physician-diagnosed POTS and migraine. Participants reported substantial autonomic symptom burden, chronic pain, multiple comorbidities, and reduced quality of life. One quarter had the clinical combination of hEDS/HSD, POTS, and mast cell activation syndrome. (8)
Another 2025 study reported reduced orthostatic cerebral blood-flow velocity in many patients with hEDS, along with frequent but generally mild autonomic abnormalities and evidence of small-fiber neuropathy. (9)
An orthostatic headache worsens when upright and improves after lying down. This pattern can occur with a spinal CSF leak, but it is not specific to a leak. POTS, migraine, cervicogenic mechanisms, and disorders of intracranial pressure can also produce positional symptoms. Evaluation may therefore require neurology, cardiology, autonomic testing, or specialized CSF-leak assessment depending on the history and examination.
Our article about POTS, the vagus nerve, and cervical instability explains why we investigate the neck in a subset of patients with both mechanical cervical symptoms and autonomic complaints. This remains a proposed relationship, not a universal cause of POTS.
CSF-pressure disorders, venous outflow, and Chiari findings
Spontaneous intracranial hypotension is most often caused by a spinal CSF leak. A classic symptom is a headache that worsens upright, although presentations vary. Conversely, intracranial hypertension can cause pressure-like headache, pulsatile tinnitus, transient visual obscurations, double vision, or papilledema—swelling of the optic nerve visible during an eye examination.
Chiari I malformation describes downward displacement of the cerebellar tonsils through the foramen magnum. Some patients have associated cough headache, neck pain, balance problems, swallowing difficulty, or spinal-cord changes, while other tonsillar-descent findings are incidental. Low-lying tonsils can also occur secondary to altered CSF pressure, so imaging must be interpreted in clinical context.
Our article on Chiari malformation, cerebellar tonsillar ectopia, and cervical instability discusses these distinctions in greater detail.
At Caring Medical, we have proposed that cervical dysstructure may affect the internal jugular veins or adjacent vagus nerves in a subset of patients. In our 2026 retrospective study of 84 patients with hEDS evaluated at an outpatient neck center, cervical abnormalities frequently co-occurred with reduced internal-jugular-vein and vagus-nerve cross-sectional areas. (10)
These findings are hypothesis-generating. The study was conducted in a specialty clinic without a matched healthy control group, and it cannot determine whether the cervical findings caused headache, dysautonomia, or other symptoms. Referral bias also limits generalization to the broader hEDS population.
A second 2026 Caring Medical study of 227 patients with persistent brain-based symptoms likewise found frequent cervical structural abnormalities, reduced jugular and vagus-nerve measurements, and increased optic-nerve-sheath measurements. The correlations between instability measurements and neurovascular measurements were statistically significant but weak. (11) This again supports further prospective study rather than a conclusion of causation.
For our full clinical hypothesis concerning cerebral venous drainage, see cervical instability, venous outflow, and intracranial pressure.
TMJ dysfunction can add another headache generator
TMJ dysfunction can cause jaw pain, facial pain, ear symptoms, painful clicking, chewing difficulty, and headache. It may coexist with cervical pain because trigeminal and upper cervical sensory pathways converge within the nervous system.
In a hypermobile patient, both the jaw and cervical spine can require evaluation. Treating the jaw will not resolve every headache, and finding cervical tenderness does not prove that the neck is the primary cause. Our review of TMJ injections and conservative treatments discusses this overlapping pain pattern.
How we evaluate a patient with hEDS and headache
The first priority is to identify the headache type and exclude dangerous or more common causes. Depending on the presentation, evaluation may include:
- A detailed headache and medication history
- Neurological, cervical, vascular, and eye examinations
- Assessment for migraine, medication overuse, sleep disorders, TMJ dysfunction, and occipital neuralgia
- Orthostatic heart-rate and blood-pressure measurements or formal autonomic testing
- Brain or cervical imaging when clinically indicated
- Ophthalmologic assessment when intracranial hypertension is suspected
- Specialized imaging or testing for a suspected spinal CSF leak
- Vestibular evaluation for vertigo or persistent imbalance
When symptoms are strongly linked to neck motion and the examination suggests instability, we may consider dynamic assessment. Digital Motion X-ray records cervical motion during controlled movement. It can provide information not visible on a static image, but its findings must still be interpreted alongside symptoms, examination, and conventional imaging. An abnormal measurement alone does not prove the cause of a headache.
Headache warning signs requiring urgent evaluation
Treatment and the dominant mechanism
Migraine treatment may include trigger management, sleep and hydration strategies, acute migraine medication, preventive medication, and newer migraine-specific therapies. POTS, CSF leaks, TMJ disorders, vestibular disease, and severe CCI each require different evaluation and treatment pathways.
For hypermobility-related cervical symptoms, conservative care commonly begins with education, activity modification, carefully progressed stabilization exercises, and physical therapy from a clinician familiar with hEDS. Aggressive manipulation or end-range loading may be inappropriate for a patient with suspected upper cervical instability.
Severe CCI with neurological compromise may require neurosurgical consultation. Surgery should be reserved for carefully selected cases with concordant symptoms, examination findings, and imaging.
What is the evidence for Prolotherapy?
Prolotherapy involves injecting a solution—commonly containing dextrose—at selected ligament or tendon attachments with the goal of stimulating a reparative response. At Caring Medical, we consider cervical Prolotherapy only when the history, examination, and imaging support ligament-related instability and when the patient appears appropriate for a nonsurgical treatment program.
Our 2014 narrative review described capsular-ligament laxity as a possible contributor to chronic neck pain and discussed Prolotherapy as a proposed treatment. (12) Because this was a narrative review rather than a controlled trial, it does not demonstrate that Prolotherapy treats migraine or neurological symptoms.
A 2026 case report described symptomatic and structural improvement in one patient with CCI who received regenerative injections together with postural and chiropractic care. (13) This report is relevant but cannot establish treatment effectiveness because it involved one patient, lacked a control group, and combined several interventions.
The original Caring Medical headache study included only 15 patients and relied on retrospective patient-reported outcomes. Although participants reported improvements after cervical Prolotherapy, this uncontrolled pilot evidence is insufficient to prove efficacy for migraine, hEDS headache, or CCI. Larger prospective trials with standardized diagnosis, imaging, interventions, and outcome measures are needed.
Therefore, we do not present Prolotherapy as a treatment for all headaches or as a cure for hEDS. It is an investigational treatment option for selected patients in whom cervical ligament injury or instability is suspected to be an important pain generator. Potential benefits, alternatives, costs, and risks should be reviewed carefully before treatment.
Summary: hEDS headache is rarely a one-diagnosis problem
Headache and migraine are common and often disabling in hEDS and HSD. Migraine is the most frequent primary headache disorder, but it may coexist with neck pain, cervical dysfunction, POTS, CSF-pressure disorders, Chiari findings, TMJ dysfunction, and other conditions.
Current research supports broadening the differential diagnosis—not replacing migraine with cervical instability as a universal explanation. At Caring Medical, we investigate the cervical spine when the history contains meaningful mechanical clues, such as persistent neck pain, symptoms triggered by neck movement, instability sensations, or a consistent relationship between cervical position and neurological or autonomic complaints.
The goal is to identify which mechanisms are most relevant to the individual patient and to match treatment to those findings. Cervical instability may be one contributor in a subset of patients, but diagnosis and treatment must remain evidence-informed, cautious, and individualized.
Medical disclaimer
This article is for educational purposes and is not a substitute for diagnosis or treatment by a qualified healthcare professional. Headache can have many causes, including medical emergencies. Seek urgent care for a sudden severe headache, new neurological symptoms, visual loss, fever with neck stiffness, loss of consciousness, or other concerning changes. The cervical-instability and Prolotherapy concepts discussed here do not apply to every patient with hEDS, HSD, migraine, POTS, or headache.
References
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