Cochlear Implantation in deformed cochlea

Studies have shown that 20 to 28 % of children with congenital sensory neural hearing loss have malformation in cochlea and mondini dysplasia is one among them. Mondini dysplasia have a cochlea with one and half turn instead of two and a half and an incomplete interscalar septum. Cochlear implant is a treatment of choice and studies have shown that successful outcomes can be seen with cochlear implantation and they have good speech skills compared to implanted kids of same age , except for their performance in speech in noise tests.