Cornelia de Lange syndrome (CdLS) is a rare congenital multisystem syndrome characterized by a distinctive craniofacial appearance, developmental delay, intellectual disability, limb abnormalities, and hypertrichosis. Hearing loss is one of the common manifestations in CdLS....
No actionable change based on a single case; clinicians treating children with Cornelia de Lange syndrome should anticipate complex anatomy and behavioral challenges, but specific protocol modifications cannot be derived from one report.
As cochlear implantation expands to children with complex syndromes, documenting rare cases like Cornelia de Lange syndrome builds the evidence base needed to develop tailored candidacy criteria and peri-operative protocols.
- 01Cornelia de Lange syndrome is a rare multisystem genetic condition associated with intellectual disability and hearing loss.
- 02Cochlear implantation in this population involves heightened surgical, anesthetic, and rehabilitation challenges.
- 03The case demonstrates that CI can be attempted in syndromic children, though outcomes may differ from typical candidates.
- 04Multidisciplinary team involvement is described as essential in this complex patient group.
- 05Evidence is limited to a single pediatric case report.
Cochlear implantation is feasible in children with Cornelia de Lange syndrome despite significant surgical and rehabilitation challenges.
studypartially supportedCornelia de Lange syndrome presents unique challenges to cochlear implant candidacy assessment and post-operative rehabilitation.
studypartially supported- PMID
- 42345422
- DOI
- 10.65717/iao.2026.252138.
- Journal
- International Archives of Otorhinolaryngology
- Publication type
- case_report
- Evidence level
- 4
- Sample size
- 1
- Population
- Single pediatric patient with Cornelia de Lange syndrome and hearing loss
- Intervention
- Cochlear implantation
Primary outcomes
Feasibility of cochlear implantation; Identification of procedural challenges and prospects