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Updated: Sep 23, 2025
Robotic Cochlear Implantation for Direct Cochlear Access
Published on: June 16, 2022
Cochlear implantation in a 16-month-old with Chudley-McCullough Syndrome
Grant Kalil1, Shelby Barrera2, Alex Elkins2
1The University of Mississippi Medical Center, School of Medicine, 2500 North State St., Jackson, MS 39216, United States of America.
Insights
Bilateral cochlear implantation (CI) is safe and effective for pediatric patients with Chudley-McCullough Syndrome (CMS). Early detection and treatment of hearing loss in CMS are crucial for preventing speech and language delays.
Area of Science:
- Otolaryngology
- Genetics
- Pediatrics
Background:
- Chudley-McCullough Syndrome (CMS) is a rare genetic disorder.
- Sensorineural hearing loss is a common manifestation of CMS.
- Early diagnosis and intervention are critical for managing CMS-related hearing loss.
Observation:
- A 16-month-old female with CMS presented with bilateral sensorineural hearing loss after failing a newborn hearing screen.
- Hearing amplification was unsuccessful.
- The patient underwent bilateral cochlear implantation (CI).
Findings:
- The bilateral CI surgery was successful with no complications.
- Post-implantation, the patient demonstrated satisfactory speech and language development.
- This case highlights the efficacy of CI in pediatric CMS patients.
Implications:
- Cochlear implantation is a safe and effective treatment for hearing loss in pediatric CMS.
- Early identification and management of hearing impairment in CMS can prevent developmental delays.
- This case contributes to understanding the clinical presentation and management of CMS.
Objective:
The purpose of this report is to describe a case of bilateral cochlear implantation (CI) in a pediatric patient with Chudley-McCullough Syndrome (CMS). By reviewing the literature, we hope to describe common clinical presentations to aid in early diagnosis and management of pediatric patients with CMS.
Methods:
Case report with literature review.
Results:
We present a case of a 16-month-old female with CMS who presented to clinic after a failed newborn hearing screen and was found to have bilateral sensorineural hearing loss. After a failed trial of hearing amplification, the patient underwent successful bilateral CI. The patient had no surgical complications, and her follow up visit showed satisfactory speech and language development.
Conclusion:
This case validates that cochlear implantation in pediatric patients who present with CMS is both safe and efficacious. It also demonstrates the importance of early detection and treatment of sensorineural hearing loss in CMS to prevent speech and language delay.
