Cochlear Implantation in Children with Inner Ear Malformations – A Challenging Case Series

Background: Congenital inner ear malformations (IEM) pose a challenge during cochlear implantation (CI) in terms of increased risk of surgical complications and uncertainty of outcomes. This case series presents the surgical complications encountered and outcomes of CI in those children with inner ear anomalies. Aims: (1) To examine the intraoperative findings and surgical complications in children with inner ear abnormalities undergoing CI and (2) to examine the reported speech perception outcomes in these children, postoperatively using the categories of auditory performance (CAP) scores, and speech intelligibility rating. Settings and Design: A retrospective study was conducted over a period of 2 years at a tertiary care center, involving three patients with IEMs who underwent CI. Subjects and Methods: Intraoperative complications encountered and their management alongside evaluation of surgical outcomes were analyzed. Results: Two children had incomplete partition type II, and one child was found to have a common cavity according to Sennaroglu classification. Intraoperative problems, such as facial nerve palsy and cerebrospinal fluid gusher, were few of the main difficulties faced. Nonetheless, following postoperative rehabilitation, all IEM patients demonstrated a notable increase in their verbal and auditory abilities. Conclusions: Children with IEMs may benefit from auditory rehabilitation with cochlear implants. A multidisciplinary approach aiding in definitive diagnosis, surgical planning, and postoperative rehabilitation is recommended for positive outcomes.