Computed tomography findings in large vestibular aqueduct syndrome

• Published in: Acta oto-laryngologica Vol. 129; no. 7; pp. 700 - 708
• Main Authors: Ma, Xiufang, Yang, Ying, Xia, Ming, Li, Dumin, Xu, Anting
• Format: Journal Article
• Language: English
• Published: Stockholm Informa UK Ltd 01.01.2009; Taylor & Francis; Informa
• Subjects: Abnormalities, Multiple - diagnostic imaging; Biological and medical sciences; Child; Child, Preschool; computed tomography; Diagnosis, Differential; Ear, auditive nerve, cochleovestibular tract, facial nerve: diseases, semeiology; Endolymphatic Duct - abnormalities; Endolymphatic Duct - diagnostic imaging; Female; Hearing loss; Hearing Loss, Bilateral - congenital; Hearing Loss, Bilateral - diagnostic imaging; Hearing Loss, Sudden - congenital; Hearing Loss, Sudden - diagnostic imaging; Humans; Image Processing, Computer-Assisted; Infant; Magnetic Resonance Imaging; Male; Medical sciences; Nervous system (semeiology, syndromes); Nervous system as a whole; Neurology; Non tumoral diseases; Otorhinolaryngology. Stomatology; Radiology Information Systems; Retrospective Studies; semicircular canal dehiscence; Semicircular Canals - abnormalities; Semicircular Canals - diagnostic imaging; Syndrome; Tomography, Spiral Computed; Vestibular Aqueduct - abnormalities; Vestibular Aqueduct - diagnostic imaging; Vestibule, Labyrinth - abnormalities; Vestibule, Labyrinth - diagnostic imaging; computed tomography; Nervous system diseases; Radiodiagnosis; semicircular canal dehiscence; Internal ear disease; Auditory disorder; ENT; Hearing loss; Semicircular canal; Dehiscence; Medical imagery; ENT disease; Vestibular syndrome; Computerized axial tomography; Vestibular aqueduct
• ISSN: 0001-6489; 1651-2251
• DOI: 10.1080/00016480802412813

Conclusions. Patients with large vestibular aqueduct syndrome (LVAS) have disturbed morphogenesis of bony labyrinth. Semicircular canal anomalies are common in LVAS. Objective. To describe the additional inner ear anomalies on CT imaging in pediatric patients with LVAS, and to investigate the lateral semicircular canal (LSCC) anomalies associated with LVAS by measurement of the LSCC bony island width. Patients and methods. We retrospectively reviewed the digitally stored temporal bone CT imaging obtained for 23 patients with LVAS, additional inner ear anomalies were noted, and measurements of the LSCC bony island width were made on axial CT scans on the workstation. Measurements were compared to the normative data obtained from 20 patients without sensorineural hearing loss. Results. Of the 23 patients (bilateral in 22 and unilateral in 1), additional inner ear malformations were identified in 21 cases presenting either singly or in combination. A small LSCC bony island (<3 mm in diameter) appeared highly typical; vestibule and LSCC anomalies were identified in 13 cases (26 ears) based on visual inspection combined with abnormal measurements. Dehiscence of the superior and/or posterior semicircular canal was identified in 19 ears, and Mondini deformity was identified in 6 ears.