Ehlers-Danlos Syndrome and Hearing Disorders

Ehlers-Danlos syndrome (EDS) is primarily recognized for its impact on joints, skin, and blood vessels. Marcello Cherchi, in a recent review, argues that its effects on hearing and balance are a significant yet understudied component of the disorder. The connective tissue abnormalities that define EDS can directly compromise the intricate structures of the inner ear and auditory pathways, contributing to tinnitus, hyperacusis, dizziness, and hearing loss.

The Overlooked Connection: How EDS Damages Hearing and Balance

Cherchi’s review outlines several direct mechanisms. The collagen defects in EDS can weaken the tiny ossicles (bones) in the middle ear, leading to conductive hearing loss. More significantly, the disorder can affect the inner ear. The cochlea and vestibular system rely on a precise extracellular matrix and connective tissue scaffolding. Abnormalities here can disrupt hair cell function, membrane integrity, and fluid dynamics, resulting in sensorineural hearing loss, tinnitus, and vertigo. This provides a clear structural basis for symptoms that might otherwise be misattributed.

Craniocervical Instability as a Major Contributor

A particularly critical pathway highlighted is craniocervical instability (CCI). The lax ligaments in the neck of individuals with EDS can allow excessive movement at the junction of the skull and spine. This instability can compress the brainstem, cranial nerves, and vertebral arteries. Such compression can disrupt the central auditory and vestibular processing pathways, manifesting as sound sensitivity (hyperacusis), tinnitus, and persistent imbalance. This link explains why some patients’ auditory symptoms fluctuate with neck position or are intertwined with chronic headaches and dysautonomia.

Methodology: A Comprehensive Evidence Review

Cherchi’s work is a synthesis of existing clinical and research literature. The review methodically examines case reports, clinical series, and the known pathophysiology of EDS to construct a coherent model of audiovestibular involvement. It connects dots between the systemic collagen vulnerability and specific otologic structures, from the tympanic membrane to the cochlear apex. The paper does not present new experimental data but consolidates scattered evidence to build a compelling argument for greater clinical awareness.

Practical Implications for Diagnosis and Management

The review concludes with actionable recommendations for clinicians. For patients with EDS, baseline audiologic and vestibular evaluations are advised, even without overt symptoms. For patients presenting with idiopathic hearing loss, tinnitus, or dizziness, a screening for joint hypermobility and other EDS signs (skin elasticity, easy bruising) should be considered. This is especially relevant when symptoms co-occur with migraine or neck pain, conditions with known overlaps.

Management must be multidisciplinary. Audiologists can address hearing loss and hyperacusis with sound therapy and hearing aids. Vestibular rehabilitation is essential for balance retraining. Crucially, addressing the cervical spine component is vital. Physical therapy for neck stabilization and, in some cases, interventions targeting cervical instability may provide relief for both dizziness and auditory symptoms, an approach aligned with emerging research on cervical stimulation for hearing disorders.

A Call for Integrated Care and Further Research

Cherchi’s analysis makes it clear that hearing and balance issues in EDS are not incidental but integral to the disorder’s pathophysiology. Recognizing this connection can prevent diagnostic delays and guide more effective, holistic treatment. It also underscores the need for more targeted research. Understanding the specific collagen subtypes involved in inner ear integrity could open future avenues for targeted therapies, similar to the precision explored in nanocarrier research for hearing health.

For patients, this review validates often-dismissed symptoms and provides a biological framework for their experiences. It encourages them to seek comprehensive evaluations that look beyond isolated symptoms to their systemic cause.

Source: Cherchi, M. (2026). Audiologic and vestibular manifestations of Ehlers-Danlos syndrome: a review. Journal of Hearing and Balance Disorders. DOI: 10.1080/23995270.2026.2701706