Cochlear implantation in a 16-month-old with Chudley-McCullough Syndrome

Am J Otolaryngol. 2022 Jul-Aug;43(4):103487. doi: 10.1016/j.amjoto.2022.103487. Epub 2022 May 6.


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.

Keywords: Child; Chudley McCullough Syndrome; Cochlear implantation; Hearing loss; Pediatric.

Publication types

  • Case Reports

MeSH terms

  • Agenesis of Corpus Callosum / complications
  • Agenesis of Corpus Callosum / surgery
  • Arachnoid Cysts* / complications
  • Arachnoid Cysts* / surgery
  • Child
  • Cochlear Implantation* / adverse effects
  • Cochlear Implants* / adverse effects
  • Female
  • Hearing Loss, Sensorineural* / diagnosis
  • Hearing Loss, Sensorineural* / etiology
  • Hearing Loss, Sensorineural* / surgery
  • Humans
  • Infant
  • Infant, Newborn
  • Speech Perception*
  • Treatment Outcome

Supplementary concepts

  • Chudley-Mccullough syndrome