A Strategic Approach to Safe and Effective Cochlear Implantation in Patients With CHARGE Syndrome

NCT07320482 | Completed

• 1 other identifier: B-2512-1012-104
• Study Type: observational
• Target: 12
• Locations: 1 country
• Sites: 1

Brief Summary

This retrospective observational study aims to evaluate cochlear anatomy, surgical challenges, and strategic approaches for safe cochlear implantation in pediatric patients with CHARGE syndrome. CHARGE syndrome is frequently associated with complex temporal bone malformations, which may increase the risk of intraoperative complications and limit the applicability of standard surgical techniques. The investigators reviewed preoperative imaging findings, operative records, and perioperative clinical data from pediatric patients with CHARGE syndrome who underwent cochlear implantation at Seoul National University Bundang Hospital. The objective of this study is to identify anatomical variations, characterize surgical difficulties, and describe practical surgical strategies that may enhance safety during cochlear implantation in this high-risk population. The findings of this study are expected to contribute to improved understanding of cochlear and temporal bone anatomy in CHARGE syndrome and to support safer and more effective surgical planning for affected pediatric patients.

Conditions

CHARGE Syndrome

Outcome Measures

Primary Outcomes (2)

• "Characterization of Cochlear and Temporal Bone Anatomical Abnormalities in Pediatric Patients With CHARGE Syndrome"

  This outcome evaluates the type and severity of cochlear, vestibular, and temporal bone malformations based on retrospective review of preoperative radiologic imaging, including computed tomography (CT) and magnetic resonance imaging (MRI), as documented in existing medical records.

  Preoperative (Baseline)

• "Characterization of Cochlear and Temporal Bone Anatomical Abnormalities in Pediatric Patients With CHARGE Syndrome"

  From existing medical records and preoperative imaging (CT/MRI)

  Preoperative (Baseline)

Interventions

No intervention (observational study) OTHER

No intervention (Observation Study)

Eligibility Criteria

• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

The study population consists of pediatric patients diagnosed with CHARGE syndrome who underwent cochlear implantation at Seoul National University Bundang Hospital. These patients typically present with complex inner ear and temporal bone malformations, including cochlear hypoplasia, semicircular canal anomalies, and aberrant facial nerve courses. All participants included in this retrospective review had available preoperative imaging (CT and/or MRI) and complete operative records.

You may qualify if:

• Pediatric patients diagnosed with CHARGE syndrome
• Underwent cochlear implantation at Seoul National University Bundang hospital
• Availability of preoperative imaging (CT and/or MRI)
• Complete operative records available

Sponsors & Collaborators

• Seoul National University Bundang Hospital: lead

Study Sites (1)

Seoul National University Bundang Hospital

Seongnam, Kyeonggido, 135-796, South Korea

Location

MeSH Terms

Conditions

CHARGE Syndrome

Interventions

Observation

Condition Hierarchy (Ancestors)

Deaf-Blind Disorders; Deafness; Hearing Loss; Hearing Disorders; Ear Diseases; Otorhinolaryngologic Diseases; Sensation Disorders; Neurologic Manifestations; Nervous System Diseases; Blindness; Vision Disorders; Coloboma; Eye Diseases, Hereditary; Eye Diseases; Abnormalities, Multiple; Congenital Abnormalities; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Genetic Diseases, Inborn; Signs and Symptoms; Pathological Conditions, Signs and Symptoms

Intervention Hierarchy (Ancestors)

Methods; Investigative Techniques

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: RETROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Professor of Otolaryngology-Head and Neck Surgery Investigator Affiliation: Seoul National University Bundang Hospital

Study Record Dates

• First Submitted: November 22, 2025
• First Posted: January 6, 2026
• Study Start: November 21, 2025
• Primary Completion: November 22, 2025
• Study Completion: November 22, 2025
• Last Updated: January 6, 2026

Record last verified: 2025-12

Data Sharing

• IPD Sharing: Will not share

Locations

KR (1)