Medical College of Wisconsin
CTSICores SearchResearch InformaticsREDCap

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



Pubmed ID




Scopus ID

2-s2.0-85021823721 (requires institutional sign-in at Scopus site)   1 Citation


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


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
Magnetic Resonance Imaging
Tomography, X-Ray Computed
Ventriculoperitoneal Shunt
Vestibular Aqueduct