J Korean Radiol Soc.  2005 Mar;52(3):165-171. 10.3348/jkrs.2005.52.3.165.

Inner Ear Anomalies Causing Congenital Sensorineural Hearing Loss: CT and MR Imaging Findings

• Hong HS
• Paik SH
• Cha JG
• Park SJ
• Joh JH
• Park JS
• Kim DH
• Lee HK
• Kim SC

• KMID: 1834226
• DOI: http://doi.org/10.3348/jkrs.2005.52.3.165

Abstract

Many congenital dysplasias of the osseous labyrinth have been identified, and the differential diagnosis of these dysplasias is essential for delivering proper patient management. We retrospectively reviewed the computed tomography (CT) and magnetic resonance (MR) imaging findings of 20 children who had congenital sensorineural hearing loss. The children included cases of enlarged vestibular aqueduct and endolymphatic sac (n=8), aplasia of the semicircular canal (n=4), lateral semicircular canal-vestibule dysplasia (n=3), common cavity malformations with a large vestibule (n=1), cochlear hypoplasia (n=1), Mondini's dysplasia with large vestibular aqueduct (n=1), Mondini's dysplasia with a large vestibule (n=1), and small internal auditory canal (n=1). Six cases were unilateral. Nine cases had combined deformities, and nine cases had cochlear implants. CT was performed with a 1.0-mm thickness in the direct coronal and axial sections with using bone algorithms. MR was performed with a temporal 3D T2 FSE 10-mm scan and with routine brain images. We describe here the imaging features for the anomalies of the inner ear in patients suffering from congenital sensorineural hearing loss.

Keyword

Ear abnormalities; Computed tomography (CT); Magnetic resonance imaging (MR)

MeSH Terms

Brain
Child
Cochlear Implants
Congenital Abnormalities
Diagnosis, Differential
Ear, Inner*
Endolymphatic Sac
Hearing Loss, Sensorineural*
Humans
Magnetic Resonance Imaging*
Retrospective Studies
Semicircular Canals
Vestibular Aqueduct