Abstract
Objective The aim of this study was to determine the prevalence and types of inner ear and/or cochlear nerve anomalies in children who are candidates for cochlear implantation.
Methods This was a descriptive cross-sectional study with a retrospective review of medical and imaging records performed in a tertiary care children's hospital. All cochlear implants candidates under 15 years old with imaging assessment between January 2019 and December 2021 were concerned. The studied parameters were reason for consultation, risks factors, inner ear malformations (IEMs) classified and stratified by sensorineural hearing loss (SNHL) degree, gender, and age at diagnosis.
Results In total, 81 children (162 ears) were included in the study. Abnormalities of the inner ear were found in nine children representing a prevalence of 11.1%. These children were aged between 2 and 14 years. The average age at diagnosis of SNHL was 3.5 years. Seven children had bilateral anomalies and two unilateral anomalies, that is, 16 ears presenting 40 malformations. These included 1 complete labyrinthine aplasia (2.5%), 12 cochlear malformations (30%), 1 common cavity (2.5%), 17 vestibular/squamous cell carcinoma (SCC) malformations (42.5%), and 5 internal auditory canal malformations (12.5%). Three ear out of 16 with abnormalities demonstrated a deficient cochlear nerve. There was an association between IEMs and profound deafness, and age at diagnosis of SNHL less than 2 years.
Conclusion This study shows that 11.1% of children with profound deafness have IEMs. SCC malformations and cochlear hypoplasia were the most common. A precise description of these malformations during the imaging assessment is particularly useful for cochlear implantation to better plan this surgery.
Keywords
cochlear implantation - hearing loss - inner ear malformations - imaging - radiology
