Audiologic and otologic phenotype in children with Duane's Retraction Syndrome: A rare ophthalmologic disorder

Objective The focus of this study was to evaluate the prevalence, type, and severity of hearing impairment in patients with Duane's Retraction Syndrome and to relate these measures to patient demographics and other otologic and audiologic factors. Study design Retrospective AudGen Database analysis Setting Tertiary academic referral center Patients Pediatric patients in AudGenDB with a diagnosis of Duane's Retraction Syndrome (DRS). Interventions Appropriate audiologic, otologic, and demographic data were recorded. Main outcome measure Seventy-nine patients (n = 79) met inclusion criteria. The first encounter with available audiometric data or the first encounter with hearing loss were documented. Audiograms were stratified by type and severity of hearing loss, and common associated medical issues were documented. Results 57 children had normal hearing; 22 had hearing loss. 9 ears had pure conductive hearing loss, 1 had pure sensorineural, 14 ears had components of both, and 79 had hearing loss that could not be specified. Multivariate regression revealed episodes of chronic otitis and craniofacial anomalies are associated with worse hearing loss. Conclusion This study presents a detailed characterization of hearing loss in patients with Duane's retraction syndrome. Conductive and sensorineural hearing loss are both prevalent among these children. Careful and early audiologic evaluation of all patients with DRS is important.