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

作者：Weir Forest W^*; Kreicher Kathryn L; Hatch Jonathan L; Nguyen Shaun A; Meyer Ted A

来源：International Journal of Pediatric Otorhinolaryngology, 2016, 89: 154-158.

DOI：10.1016/j.ijporl.2016.08.014

• Duane's Retraction Syndrome
• Duane syndrome
• Eye retraction syndrome
• Retraction syndrome
• Congenital retraction syndrome
• Stilling-Turk-Duane Syndrome
• Hearing loss
• Audiology
• Audiogram
• Pure-tone audiometry

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.

• 出版日期2016-10