Type:
Educational Exhibit
Keywords:
Head and Neck, Anatomy, Ear / Nose / Throat, CT-High Resolution, MR, Diagnostic procedure, Congenital, Retrospective, Not applicable, Performed at one institution
Authors:
S. Siala, M. Jrad, S. Sbaihi, S. Boukriba, W. Frikha, H. Mizouni; Tunis/TN
DOI:
10.26044/ecr2020/C-13269
Background
Inner ear malformations is a general term describing various types of cochlear anomalies. The latest classification of IEM established by Sennaroglu et al. using computerized tomography (CT) and magnetic resonance imaging (MRI), included eight groups (1). Cochlear hypoplasia (CH) represents a group of malformations where external dimensions are less than those of a normal cochlea with various internal architecture anomalies. They are divided into four different subgroups, according to the cochlear development degree on CT (1).
These different subgroups present large different clinical presentations and post-operative outcomes if cochlear implant is indicated.
Cochlear hypoplasia represents 15 to 25 % of inner ear malformations (2,3).
Patients with cochlear hypoplasia may demonstrate different audiological characteristics. They may present with normal, mild or profound sensorineural hearing loss according to CH subtype. They may also present conductive hearing loss (1).