Down's syndrome with abnormal inner ear: Is it suitable for cochlear implantation?

Hearing loss is a common problem in Down's syndrome (DS). The majority of this population, up to 80%, are suffering from a conductive type hearing loss, whereas estimating 4-20% are due to sensorineural hearing loss. Over the years, the treatment of profound sensorineural hearing loss has been changed since the introduction of cochlear implants. We report a case of a 4 years and 5 months old child with DS and low Intelligence Quotient that had been referred to our centre for cochlear implants. In view of late referral and multiple additional handicaps, with addition of having Larged Vestibular Aqueduct Syndrome (LVAS), bilateral incomplete partition of cochlear Type II and abnormal periventricular white matter, she had been rejected for cochlear implantation.