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Enlarged Vestibular Aqueduct Syndrome: Sudden Hearing Loss in a Child with a Cerebral Shunt. Pediatr Emerg Care 2019 Jul;35(7):e135-e137

Date

07/06/2017

Pubmed ID

28678056

DOI

10.1097/PEC.0000000000001043

Scopus ID

2-s2.0-85021823721

Abstract

Enlarged vestibular aqueduct syndrome (EVAS) is the most common congenital ear anomaly that causes sensorineural hearing loss in children and may predispose a child to sudden hearing loss from sudden pressure changes or minor head trauma. We report a case of a 4-year-old boy with a history of a ventriculoperitoneal shunt and migraines who presented to the emergency department with parental and child care provider reports of acute hearing loss, without a history of trauma, infection, or hardware malfunction, who was diagnosed with bilateral EVAS. Diagnosis of EVAS occurs with specific temporal bone imaging with either high-resolution, thin-cut computed tomography or magnetic resonance imaging scans. Enlarged vestibular aqueduct syndrome is typically refractory to medical treatment and often results in hearing loss that is too severe to benefit from amplification, requiring cochlear implantation.

Author List

Wendt WJ, Hashikawa A

Author

Wendi-Jo Wendt MD Assistant Professor in the Pediatrics department at Medical College of Wisconsin




MESH terms used to index this publication - Major topics in bold

Child, Preschool
Hearing Loss, Sensorineural
Hearing Loss, Sudden
Humans
Magnetic Resonance Imaging
Male
Syndrome
Tomography, X-Ray Computed
Ventriculoperitoneal Shunt
Vestibular Aqueduct