Cochlear Implantation in Children with Congenital Inner Ear Malformations

AbstractBackground: Cochlear Implantation has proven beneficial in patients with profound congenital hearing impairments. Performing cochlear implantation in patients with inner ear malformation has always been a matter of dispute as congenital deaf ears may have anatomical malformations and difficulties during operation, it is important to focus on problems and complications that may face the surgeon who is performing this operation.Objective: Evaluate the problems and surgical difficulties encountered in patients with congenital inner ear malformation, who underwent cochlear implantation.Patients and methods: A series of 21 pediatric cochlear implant (CI) patients with known inner ear malformations determined on computed tomography (CT) of the temporal bone who underwent cochlear implantation in the department of otolaryngology in Hospital Martyr Ghazi Al-Hariri and AL-Yarmouk Teaching Hospital, Baghdad, Iraq, during the period from January 2018 to January 2021 were the subjects of the study to determine intraoperative adverse effects of these anomalies regarding electrode array insertion depths, predisposition to cerebrospinal fluid (CSF) leak through the cochleostomy, and associated facial nerve anomalies that can create access problems for scala tympani.Results: The most common inner ear anomalies were wide vestibular aqueduct (15) patients with percentage of (71.4%), incomplete partitions (5) patients with percentage of (23.8%), and common cavity (1) patient with percentage of (4.8%). CSF leak, the most common was CSF ooze (11) patients with percentage of (52.4%), CSF gusher seen in (6) patients (28.6%), and no CSF leak in (4) patients (19%). Facial nerve anomalies, only one case has abnormal position (more superficial and not injured) with a percentage of (4.8%), while in 20 patients (95.2%) no anomaly was detected. Electrode insertion depth, full insertion was achieved in (17) patients with percentage of (81%), while incomplete insertion was occurred in (4) patients (19%).Conclusion: Cochlear implant can be successfully and safely done in patients with selected congenital inner ear anomalies. Surgery can be challenging in certain cases of gusher and facial nerve abnormalities, but proper radiology and good surgical technique will help avoid complications. Keywords: Cochlear Implantation, Congenital inner ear malformation, facial nerve anomalies.