Audiological and Speech Performance After Cochlear Implantation in Cochlear Aplasia Deformity.

Inner ear malformation is a congenital anomaly associated with prelingual sensory neural hearing loss in the pediatric population. This is a case report of bilateral radiologically diagnosed cochlear aplasia in a child who underwent unilateral left cochlear implantation with audiological results at one-year follow-up after surgery. Sixteen months after the cochlear implantation surgery, the child could produce 200 words and say a sentence with two words. In certain cases of inner ear malformation, the subgrouping of cystic cavity can be difficult and should not delay the trial of cochlear implant provided an acceptable anatomical appearance of the inner ear with cochleovestibular nerve existence and a proper electrode used for implantation.