Introduction: This Observational Study Aims To Evaluate Auditory And Speech Outcomes In Children With Jervell And Lange-Nielsen Syndrome (Jlns) Compared To Non-Syndromic Children, Using Cap (Categories Of Auditory Performance) And Sir (Speech Intelligibility Rating) Scores After Cochlear Implantation. Background: Jlns Is A Rare Autosomal Recessive Disorder Characterized By A Prolonged Qt Interval And Severe Bilateral Sensorineural Hearing Loss. Cochlear Implantation Is A Common Intervention In These Children To Enhance Auditory Perception And Speech Development. However, Associated Systemic Complications May Influence Their Post-Implantation Outcomes Relative To Non-Syndromic Peers. Objective: To Compare Cap And Sir Scores In Children With Jlns And Those With Non-Syndromic Hearing Loss Following Cochlear Implantation. Methods: A Six-Month Observational Study Was Carried Out In The Department Of Ent At Saveetha Medical College And Hospital, Chennai, Starting In October 2024. A Total Of 102 Children Were Recruited And Divided Equally Into Group A (51 Children With Jlns) And Group B (51 Non-Syndromic Children), All Of Whom Had Undergone Cochlear Implantation. Cap And Sir Scores Were Used To Assess And Compare Auditory And Speech Outcomes. Results: Children With Jlns Exhibited Significantly Higher Cap And Sir Scores Than Their Non-Syndromic Counterparts, Indicating Better Cognitive And Speech Outcomes. Conclusion: Findings Suggest Enhanced Auditory And Speech Performance And Better Follow-Up Adherence Among Children With Jlns, Possibly Due To Structured Medical Care And Early Intervention