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.


METHODS
Retrospective AudGen Database analysis


METHODS
Tertiary academic referral center


METHODS
Pediatric patients in AudGenDB with a diagnosis of Duane's Retraction Syndrome (DRS).


METHODS
Appropriate audiologic, otologic, and demographic data were recorded.


METHODS
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.


CONCLUSIONS
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.
Department of Otolaryngology-Head and Neck Surgery, Medical University of South Carolina, Charleston, SC, USA. Electronic address: weir.forest@gmail.com.
Forest W
FW
Weir
Department of Otolaryngology-Head and Neck Surgery, Medical University of South Carolina, Charleston, SC, USA.
Kathryn L
KL
Kreicher
Department of Otolaryngology-Head and Neck Surgery, Medical University of South Carolina, Charleston, SC, USA.
Jonathan L
JL
Hatch
Department of Otolaryngology-Head and Neck Surgery, Medical University of South Carolina, Charleston, SC, USA.
Shaun A
SA
Nguyen
Department of Otolaryngology-Head and Neck Surgery, Medical University of South Carolina, Charleston, SC, USA.
Ted A
TA
Meyer
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D016208:Databases, Factual; D003937:Diagnosis, Differential; D004370:Duane Retraction Syndrome / Q000150:complications*; D005260:Female; D034381:Hearing Loss / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology*; D006320:Hearing Tests; D006801:Humans; D007223:Infant; D008297:Male; D010641:Phenotype; D015995:Prevalence; D012189:Retrospective Studies
Audiogram; Audiology; Congenital retraction syndrome; Duane syndrome; Duane's Retraction Syndrome; Eye retraction syndrome; Hearing loss; Pure-tone audiometry; Retraction syndrome; Stilling-Türk-Duane Syndrome