ABSTRACT
Cochlear implantation is the treatment of choice for children with profound sensorineural hearing impairment (SNHI), yet the outcomes of cochlear implants (CI) vary significantly across individuals. To investigate the CI outcomes in pediatric patients with SNHI due to various etiologies, we prospectively recruited children who underwent CI surgery at two tertiary referral CI centers from 2010 to 2021. All patients underwent comprehensive history taking, next generation sequencing (NGS)-based genetic examinations, and imaging studies. The CI outcomes were evaluated using Categories of Auditory Performance (CAP) and Speech Intelligibility Rating (SIR) scores. Of the 160 pediatric cochlear implantees (76 females and 84 males) included in this study, comprehensive etiological work-up helped achieve clinical diagnoses in 83.1% (133/160) of the patients, with genetic factors being the leading cause (61.3%). Imaging studies identified certain findings in 31 additional patients (19.3%). Four patients (2.5%) were identified with congenital cytomegalovirus infection (cCMV), and 27 patients (16.9%) remained with unknown etiologies. Pathogenic variants in the four predominant non-syndromic SNHI genes (i.e., SLC26A4, GJB2, MYO15A, and OTOF) were associated with favorable CI outcomes (Chi-square test, p = 0.023), whereas cochlear nerve deficiency (CND) on imaging studies was associated with unfavorable CI outcomes (Chi-square test, p < 0.001). Our results demonstrated a clear correlation between the etiologies and CI outcomes, underscoring the importance of thorough etiological work-up preoperatively in pediatric CI candidates.
ABSTRACT
Cochlear implantation (CI) has developed for more than four decades. Initially, CI was used for profound bilateral hearing impairment. However, the indications for CI have expanded in recent years to include children with symptomatic partial deafness. Therefore, CI strategies to preserve residual hearing are important for both patients and otologists. The loss of residual low-frequency hearing is thought to be the result of many factors. All surgical methods have the same goal: protect the delicate intracochlear structures and preserve residual low-frequency hearing to improve speech perception abilities. Fully opening the round window membrane, a straight electrode array, slower insertion speed, and the use of corticosteroids result in a higher rate of hearing preservation. Several factors, like the way of surgical approaches, length of arrays and timing of activation, may not affect the residual hearing preservation. Therefore, the classic atraumatic technique, including the very slow and delicate insertion and administration of intraoperative corticosteroids, can improve hearing outcomes.
