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

A Retrospective Observational Study Evaluating Surgical Strategies and Safety Profiles of Cochlear Implantation in Pediatric Patients With CHARGE Syndrome

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.

Study Overview

• Status: Completed
• Conditions: CHARGE Syndrome
• Intervention / Treatment: Other: No intervention (observational study)

Detailed Description

This retrospective observational study evaluates surgical strategies and safety profiles of cochlear implantation in pediatric patients diagnosed with CHARGE syndrome. Medical records, preoperative imaging studies (CT and/or MRI), and operative reports were retrospectively reviewed to assess cochlear and temporal bone anatomy, surgical challenges, and intraoperative findings.

The study focuses on identifying anatomical variations commonly encountered in CHARGE syndrome and describing practical surgical approaches used to manage complex inner ear and temporal bone malformations. No interventions were assigned as part of this observational study.

• Study Type: Observational
• Enrollment (Actual): 12

Contacts and Locations

Study Locations

• South Korea
  • Kyeonggido
    • Seongnam, Kyeonggido, South Korea, 135-796
      • Seoul National University Bundang Hospital

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: Child; Adult; Older Adult
• Accepts Healthy Volunteers: No

• 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.

Description

• Inclusion Criteria

  • 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
• Exclusion Criteria -Patients with CHARGE syndrome who did not undergo cochlear implantation

Study Plan

How is the study designed?

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
"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)

Collaborators and Investigators

• Sponsor: Seoul National University Bundang Hospital

Study record dates

Study Major Dates

• Study Start (Actual): November 21, 2025
• Primary Completion (Actual): November 22, 2025
• Study Completion (Actual): November 22, 2025

Study Registration Dates

• First Submitted: November 22, 2025
• First Submitted That Met QC Criteria: December 21, 2025
• First Posted (Actual): January 6, 2026

Study Record Updates

• Last Update Posted (Actual): January 6, 2026
• Last Update Submitted That Met QC Criteria: December 21, 2025
• Last Verified: December 1, 2025

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Neurologic Manifestations; Nervous System Diseases; Genetic Diseases, Inborn; Eye Diseases; Eye Diseases, Hereditary; Congenital Abnormalities; Otorhinolaryngologic Diseases; Vision Disorders; Sensation Disorders; Abnormalities, Multiple; Ear Diseases; Deaf-Blind Disorders; Deafness; Hearing Loss; Hearing Disorders; Blindness; Coloboma; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Pathological Conditions, Signs and Symptoms; Signs and Symptoms; CHARGE Syndrome; Investigative Techniques; Methods; Observation
• Other Study ID Numbers: B-2512-1012-104

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO
• IPD Plan Description: Individual participant data (IPD) will not be shared because this study involves a small cohort of pediatric patients with CHARGE syndrome, a rare condition in which the risk of re-identification is high even after de-identification. The dataset includes sensitive clinical details, imaging findings, and operative records that cannot be safely disclosed under institutional privacy and ethical policies