Cochlear Implantation Outcomes in Children with Agenesis of the Corpus Callosum: A Retrospective Study and A Review of the Literature

J Int Adv Otol. 2019 Dec;15(3):364-367. doi: 10.5152/iao.2019.6577.


Objectives: The aim of the present study was to analyze the outcomes of cochlear implantation (CI) in patients with agenesis of the corpus callosum (CCA). A literature review and a retrospective analysis of our cochlear implant database were performed.

Materials and methods: To the best of our knowledge, in the English literature, there was only one case reported with CCA who had undergone CI surgery. This case had Donnai-Barrow syndrome. In the Cukurova University School of Medicine Department of Otorhinolaryngology database, 5 of the 1317 patients who underwent CI surgery who had CCA were selected. The patients' demographic characteristics, operative findings, surgical outcomes, and additional disabilities were investigated. The patients' preoperative and postoperative Listening Progress Profile (LiP) and Meaningful Auditory Integration Scale (MAIS) tests were done to analyze the auditory performances.

Results: The participants of the study were 5 (0.38%) individuals (2 male and 3 female patients; ages 5.5, 7.5, 8, 9, and 12 years). Two of the patients had total agenesis, and the other three had partial agenesis of the CCA. In the histories of the patients, one patient had parental consanguinity, and one had febrile convulsion. No patient had an additional disability. None had experienced device failure. No patients were non-users or limited users of cochlear implants. Postoperative LiP and MAIS test scores were improved for all patients nearly as the patients without any deformity. They showed normal auditory performance in the analysis in their postoperative 48 months of follow-up.

Conclusion: Patients who had CCA are good candidates for CI surgery.

Publication types

  • Review

MeSH terms

  • Agenesis of Corpus Callosum / complications
  • Agenesis of Corpus Callosum / surgery*
  • Child
  • Child, Preschool
  • Cochlear Implantation*
  • Deafness / congenital
  • Deafness / surgery*
  • Female
  • Hearing Loss, Sensorineural / congenital
  • Hearing Loss, Sensorineural / surgery
  • Hearing Tests
  • Hernias, Diaphragmatic, Congenital / surgery
  • Humans
  • Language Development
  • Male
  • Myopia / congenital
  • Myopia / surgery
  • Proteinuria / congenital
  • Proteinuria / surgery
  • Renal Tubular Transport, Inborn Errors / surgery
  • Retrospective Studies
  • Treatment Outcome

Supplementary concepts

  • Donnai-Barrow syndrome