Auditory And Speech Outcomes Post Cochlear-Implantation: A Comparative Analysis Of Cap And Sir Scores In Jervell–Lange-Nielsen Syndrome And Non-Syndromic Children

Abstract

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.