Cochlear implantation in children with Mondini dysplasia: our experience.

OBJECTIVE This study details the intra-operative complications, and compares auditory scales post-implantation of either profoundly deaf young children with radiologically normal inner ears (group A) or children with Mondini dysplasia (group B). METHODS A retrospective survey was carried out of 338 patients with severe to profound sensorineural hearing loss who underwent cochlear implant surgery from February 2015 to May 2017. Patients were divided into 2 groups of 27 patients each. Both groups were followed up to three years post-implantation. RESULTS Cerebrospinal fluid ooze developed in 12 patients, and 2 patients had a cerebrospinal fluid 'gusher', one of which had to be explored within 24 hours. After implant use for one year, both groups had similar speech perception scores. CONCLUSION The cerebrospinal fluid gusher in Mondini dysplasia should be anticipated and adequately managed intra-operatively. This study highlights the tailoring of a post-implantation rehabilitation programme according to individual needs.