Hearing and hEDS
Hearing and balance issues are increasingly recognized as part of the multisystem profile of hypermobile Ehlers-Danlos syndrome (hEDS). Though traditionally viewed as a connective tissue disorder affecting joints and skin, hEDS can also impact auditory and vestibular structures, leading to symptoms such as hearing loss, tinnitus, and dizziness. These complications may result from the same underlying collagen abnormalities that affect the rest of the body, disrupting the structure and function of the middle and inner ear. While often overlooked, early identification and management of hearing-related symptoms can significantly improve quality of life for individuals with hEDS.
The co-occurrence of hEDS and mast cell activation syndrome (MCAS) adds further complexity, as both are multisystem disorders that may influence auditory and vestibular health through overlapping or additive mechanisms. While direct evidence is limited, theoretical models suggest that MCAS-related inflammation may exacerbate ear-related symptoms in hEDS, particularly through vascular permeability, immune activation, and connective tissue degradation.
Impacts of hEDS on Hearing
Hearing loss is more prevalent in individuals with hEDS than in the general population. One large pediatric cohort study found that children with EDS were twice as likely to be diagnosed with hearing loss compared to controls (4.4% vs. 2.1%).
A retrospective analysis of pediatric EDS patients reported hearing loss in approximately 23%, with a nearly equal distribution of conductive and sensorineural types, often bilateral and with a flat audiometric profile. Severity increased with age but did not correlate with the type of loss.
Symptoms commonly reported include difficulty hearing, tinnitus (ear ringing), and in rare cases, vestibular symptoms such as dizziness or imbalance.
Some patients may experience superior semicircular canal dehiscence (SSCD), a bony abnormality causing auditory and vestibular disturbances such as autophony, vertigo, and imbalance.
Pathophysiology (How the Disease Works)
The underlying cause is thought to be connective tissue fragility affecting key ear structures, including the ossicles (middle ear bones), tympanic membrane, and cochlear components in the inner ear.
Abnormal collagen and extracellular matrix may impair the mechanical and supportive function of these structures.
MCAS may further contribute through inflammatory mediators (e.g., histamine, tryptase) that increase vascular permeability, induce local edema, and disrupt the function of the hair cells, endolymphatic system, and Eustachian tubes.
When combined with the inherent tissue fragility of hEDS, MCAS-mediated inflammation may heighten susceptibility to conductive or mixed hearing loss, recurrent middle ear problems, and vestibular dysfunction.
Symptoms
Hearing difficulties range from mild to severe and include trouble understanding speech, especially in noisy environments.
Tinnitus is commonly reported, possibly due to nerve involvement or inner ear dysfunction.
Vestibular symptoms such as dizziness, imbalance, and vertigo occur in a smaller subset, sometimes related to SSCD or mast cell-related inflammation.
Eustachian tube dysfunction may be more prevalent due to structural instability and mast cell-related swelling, potentially leading to recurrent middle ear pressure issues.
Empirical Evidence
Clinical observations, small cohort studies, and case reports consistently note an increased prevalence of hearing loss and vestibular issues in hEDS.
Pediatric data are more robust, though adult-specific findings remain sparse.
There is currently no large-scale empirical study directly examining the additive effects of hEDS and MCAS on hearing, though their frequent co-occurrence and overlapping symptom profiles suggest a potential synergistic impact.
Treatments and Management
Treatment is individualized and follows standard audiologic protocols:
Hearing aids are used for sensorineural hearing loss.
Surgical or non-surgical interventions may be indicated for conductive losses or anatomical abnormalities like SSCD.
Vestibular rehabilitation addresses balance and dizziness symptoms.
Caution is advised in surgical procedures due to tissue fragility and the potential for poor wound healing, especially when MCAS is also present.
Pharmacologic management of MCAS—such as antihistamines, leukotriene inhibitors, or mast cell stabilizers—may help reduce inflammation-related otologic symptoms in select patients. (See separate article on MCAS)
Early audiologic screening, especially in children with hEDS or those with suspected MCAS, is recommended to identify hearing loss promptly and prevent developmental or social impact.
Broader Context and Challenges
Hearing loss adds to the multisystem burden of hEDS, complicating communication, education, and social participation.
Patients with both hEDS and MCAS may experience amplified symptoms due to overlapping inflammatory and structural vulnerabilities.
Diagnostic delays and lack of provider awareness contribute to under-recognition and undertreatment of hearing issues in this population.
Summary
Individuals with hypermobile Ehlers-Danlos syndrome have a higher risk of hearing loss—conductive, sensorineural, or mixed—often bilateral and worsening with age. Symptoms include difficulty hearing, tinnitus, and sometimes vestibular disturbances like dizziness, potentially linked to connective tissue fragility, bony abnormalities such as superior semicircular canal dehiscence, and mast cell-mediated inflammation. Treatment follows standard audiological care with individualized management and a cautious approach to surgery. When MCAS is also present, inflammatory contributions may exacerbate symptoms and should be addressed through appropriate pharmacologic and environmental management. Early hearing screening is vital, particularly in pediatric patients, to address this under-recognized aspect of hEDS and mitigate its impact on communication and quality of life.
