Computed tomography findings in large vestibular aqueduct syndrome.
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.


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.
Ma
Xiufang
X
Department of Otorhinolaryngology, Qilu Hospital, Shandong University, Jinan, China.
Yang
Ying
Y
Xia
Ming
M
Li
Dumin
D
Xu
Anting
A
D000015:Abnormalities, Multiple / Q000000981:diagnostic imaging; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D004711:Endolymphatic Duct / Q000002:abnormalities* / Q000000981:diagnostic imaging; D005260:Female; D006312:Hearing Loss, Bilateral / Q000151:congenital* / Q000000981:diagnostic imaging*; D003639:Hearing Loss, Sudden / Q000151:congenital / Q000000981:diagnostic imaging; D006801:Humans; D007091:Image Processing, Computer-Assisted; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D011873:Radiology Information Systems; D012189:Retrospective Studies; D012665:Semicircular Canals / Q000002:abnormalities* / Q000000981:diagnostic imaging*; D013577:Syndrome; D036542:Tomography, Spiral Computed; D014723:Vestibular Aqueduct / Q000002:abnormalities* / Q000000981:diagnostic imaging*; D014722:Vestibule, Labyrinth / Q000002:abnormalities* / Q000000981:diagnostic imaging*