Objective: The focus of this study was to evaluate the prevalence, type, and severity of hearing impairment in patients with Ehlers-Danlos Syndrome (EDS) and to compare these characteristics with patient demographics and other otologic factors.

Study design: Retrospective analysis of the AudGen Database.

Setting: Tertiary academic referral center.

Patients: Pediatric patients in AudGenDB with a diagnosis EDS.

Interventions: Appropriate audiologic, otologic, and demographic data were recorded.

Main outcome measure: One hundred forty one patients met inclusion criteria. Data for each patient were selected based on their first encounter by age with available audiometric data or their first encounter with a type of hearing loss documented. The patients were then stratified by type and severity of hearing loss, and available otologic issues were documented.

Results: One hundred nine patients had normal hearing. Thirty two patients had hearing loss, with 19 bilateral, and 13 unilateral. Of the 51 individual ears, 25 had conductive, 23 had sensorineural, and 3 had mixed hearing loss. Audiometric configuration was primarily flat in nature. The hearing loss severity was significantly correlated with age, but not influenced by the type of loss.

Conclusion: Hearing loss is prevalent in patients with EDS. The hearing loss is equally of conductive and sensorineural origin, with a predisposition to be bilateral. Further evaluation of the underlying pathology of hearing loss in these patients is warranted to aid otolaryngologists and audiologists in diagnosis and management.