Objective: The aim was to evaluate cochlear implantation (CI) outcomes in children with Down syndrome (DS) with severe-to-profound sensorineural hearing loss (SNHL), addressing a literature gap and discussing challenges including anatomical abnormalities, cognitive deficits, and Eustachian tube dysfunction. Data Sources: Systematic searches were conducted in PubMed, Web of Science, Scopus, and Embase from inception through to June 2025. Review Methods: A systematic review adhering to PRISMA guidelines was performed. Included studies reported CI outcomes in DS patients receiving otolaryngologic care for SNHL. Extracted data included findings on ear anatomy, auditory performance, speech/language development, intelligibility, and duration of CI use. Results: A total of 149 abstracts were screened, yielding six studies with 26 patients that met the inclusion criteria. The review included pediatric DS patients with documented ages at implantation spanning from 11 months to 17.9 years. CI provided significant benefits for DS patients, including improved audiometric results, enhanced environmental awareness, and psychosocial gains. Optimal outcomes were associated with early implantation, thorough preoperative imaging (CT/MRI), and management of middle ear disease. Variability in outcomes often reflected cognitive limitations and anatomical challenges such as cochlear nerve hypoplasia and Eustachian tube dysfunction. Conclusions: CI can significantly improve quality of life and communication in children with DS when tailored to their unique needs. Preoperative imaging is essential to assess candidacy, and middle ear disease should be addressed prior to surgery. Clinicians should counsel families with individualized goals that emphasize functional hearing gains over normative speech benchmarks. Broader adoption of CI in this population may be supported by standardized, population-sensitive outcome measures and future prospective studies.

Objective: To compare hearing preservation (HP) outcomes between lateral wall (LW) and perimodiolar (PM) cochlear implant electrode arrays. Databases reviewed: PubMed, Embase, Scopus, Web of Science, and ScienceDirect for studies published between January 2018 and April 2025. Methods: This review adhered to PRISMA guidelines. Studies were included if they reported HP outcomes stratified by electrode type (LW or PM) undergoing primary cochlear implantation (CI). The Joanna Briggs Institute (JBI) tools were used to assess risk of bias. Meta-analyses were conducted using random-effects models, and subgroup analyses were performed based on follow-up duration and HP criteria. Heterogeneity was assessed through the I ² statistic. Results: Of 976 screened records, 80 studies met inclusion criteria for synthesis, and 72 studies (n=3783 ears) were included in the meta-analysis. The overall pooled HP rate was 45.9% for LW electrodes and 34.2% for PM electrodes ( P <0.000005). In 18 head-to-head comparison studies (n=1710), the pooled risk ratio favored LW arrays (RR=1.24 [0.99, 1.56], P =0.057). A sensitivity analysis excluding short-term studies (≤1 mo follow-up) revealed statistically significant superiority for LW arrays (RR=1.37 [1.06, 1.77], P =0.016). Conclusions: LW electrode arrays were associated with higher overall HP rates compared with PM designs, although differences varied across follow-up durations and study types. Electrode selection should consider preoperative hearing status, cochlear anatomy, and surgeon experience to optimize outcomes.