Objective: To present and analyse the radiological findings, surgical findings, pre-implantation and post-implantation speech and hearing outcomes of nine children with SMS Type III cochleovestibular malformation.
Study design: Retrospective case series of nine children with pre-lingual profound sensorineural hearing loss who underwent cochlear implantation (Jan 2012 to July 2019). These children had been classified as Type III malformation according to the SMS Classification of cochleovestibular anomalies. Facial nerve anomalies, CSF leaks and any other significant surgical finding were noted. Meaningful Auditory Integration Scale (MAIS) was used to report the child's pre-operative and two-year post-implantation auditory and speech abilities. Any significant improvement was assessed using the Wilcoxon signed rank test. P value < 0.05 was considered significant.
Results: Out of nine patients, five patients sustained CSF gushers, while three patients had mild CSF leak, which were plugged adequately. No facial nerve anomalies were encountered. Post-op course was uneventful for all nine patients. MAIS scores at a two-year follow-up showed significant statistical improvement (P < 0.05) when compared to pre-operative scores.
Conclusion: Cochlear implant is the treatment of choice for children with type III cochleovestibular malformation. There are significant auditory and speech improvements expected. However, the surgeon should bear in mind the risk of CSF leak and subsequent meningitis.
Keywords: CSF leak; Cochleovestibular anomalies; SMS type III cochleovestibular malformations.