Cochlear Implantation in A Case With Down Syndrome, Neonatal Meningitis With Cochlear Malformation And Anomalous Facial Nerve – A Case Study
INTRODUCTION: Down syndrome is the most prevalent neurodevelopmental disorder which causes hearing loss in children, predominantly conductive in nature, with a small portion of this population also affected with sensorineural hearing loss (SNHL) (1)(2). Neonatal meningitis, another common cause for hearing loss is a serious infection of the central nervous system (3). Meningitis also affects hearing due to fibrotic changes in the cochlear fluid, perilymph, which is originated from cerebrospinal fluid. In this retrospective case study, we aim to present about cochlear implantation (CI) as a treatment option in a case with Down syndrome with congenital SNHL with cochlear hypoplasia and a history of meningitis and other anatomical challenges.
METHOD: A 3year old male presented with Down syndrome with a history of congenital SNHL with cochlear hypoplasia and delayed developmental milestones. Patient’s history revealed neonatal meningitis for which he was treated in the NICU till day 57. Objective hearing evaluation at 7 months of age revealed bilateral severe to profound SNHL. No benefit reported from amplification. Radiological evaluation revealed cochlear malformation along with posteriorly rotated cochlea and enlarged diameter in vertical segment of facial nerve, anteriorly placed sigmoid, contracted mastoid and low-lying dura on both sides. 3D reconstruction using Slicer software showed cochlear hypoplasia bilaterally. CI was recommended in the left ear due to persistent serous otitis media in the right ear. Endaural transcanal approach was chosen to insert the electrode array through a tunnel drilled in the mastoid bone to avoid injury to the enlarged facial nerve and overcome the challenges of a contracted mastoid with a narrow facial recess.
RESULTS: A MEDIUM MED-EL electrode with 24 mm length was inserted. Intraoperative test results show presence of ECAP and normal impedance levels and with an uneventful post operative period.
CONCLUSION: Thorough pre-operative surgical investigation and family counselling have provided valuable information for surgical planning in this case. Choice of surgical technique and surgical route depended on the anatomy which provided a successful surgical outcome. Also, importance of pre-operative counselling in a case with multiple challenges to set realistic expectations and post-operative rehabilitation is reiterated in this case study.

Cochlear Implantation in a Child with Down Syndrome, Neonatal Meningitis, Cochlear Malformation and Anomalous Facial Nerve – A Case Study

It is with great pleasure and enthusiasm, that on behalf of the Cochlear Implant centers of the Radboud UMC in Nijmegen and Erasmus MC Sophia Children’s Hospital in Rotterdam to invite you to attend the 16th European Symposium on Pediatric Cochlear Implantation to Rotterdam, the Netherlands in 2023.

Yours sincerely, on behalf of the CI Centers at Radboud UMC and Erasmus MC,