Cohort Characteristics of Pediatric Candidates for Cochlear Implantation With Inner Ear Malformations: A Multicenter Retrospective Study.

OBJECTIVE

To investigate comorbidities and severity correlations among inner ear malformations (IEMs) classifications and analyze subtype characteristics to optimize preoperative evaluation and cochlear implantation (CI) strategies.

METHODS

Retrospective multicenter data from Hubei Province were analyzed for 336 children with IEMs who received unilateral CI (2012-2020). IEMs were classified and graded using Sennaroğlu and Adibelli criteria, and data visualization tools were used to assess subtypes, comorbidities, and severity correlations.

RESULTS

Among pediatric candidates for CI with IEMs, isolated enlarged vestibular aqueduct, isolated incomplete partition type II, and multiple malformations were the 3 most common subtypes, accounting for 80.7% of cases. Vestibular aqueduct malformation (VAM) primarily occurred as an isolated condition, whereas internal auditory canal malformation (IACM), cochlear nerve malformation (CNM), cochlear malformation (CM), and vestibular and semicircular canal malformation (VSCM) tended to coexist, exhibiting significant co-occurrence patterns. Severity analysis revealed a positive correlation between the severity of IACM and CNM (r = .13, P = .015), as well as between CM and VSCM (r = .17, P < .001). Additionally, VAM severity was significantly negatively correlated with the severity of IACM, CNM, CM, and VSCM (r = -.39, -.21, -.38, and -.30, respectively; P < .001).

CONCLUSION

This study elucidated a detailed analysis of the subtype characteristics and comorbid relationships of IEMs in pediatric candidates for CI. Furthermore, it identified significant correlations between the severity levels of different malformation types. These insights contribute to refining preoperative evaluation protocols and tailoring treatment strategies for CI candidates.