Cochlear implantation for cochlear dysplasia

Profound sensorineural hearing loss secondary to cochlear dysplasia presents a number of surgical challenges during cochlear implantation. The standard transmastoid-facial recess approach can be performed in the majority of cases. In cases of common cavity deformity, the transmastoid labyrinthotomy approach has a number of advantages. A high incidence of CSF gushers occurs in this population but can be managed by creating a small cochleostomy and tightly sealing the cochleostomy with connective tissue. Acceptable postoperative speech perception results can be expected.