OBJECTIVE: To report the audiological outcomes of cochlear implantation in two patients with severe to profound sensorineural hearing loss secondary to superficial siderosis of the CNS and discuss some programming peculiarities that were found in these cases. METHOD: Retrospective review. Data concerning clinical presentation, diagnosis and audiological assessment pre and post implantation were collected of two patients with superficial siderosis of the CNS. RESULTS: Both patients showed good hearing thresholds but variable speech perception outcomes. One patient did not achieve open-set speech recognition, but the other achieved 70% speech recognition in quiet. Electrical compound action potentials could not be elicited in either patient. Map parameters showed the need for increased charge. Electrode impedances showed high longitudinal variability. CONCLUSION: The review of the literature shows that the outcomes of cochlear implantation for the management of SNHL in superficial siderosis are irregular and may be unsatisfactory sometimes. Performance data described are scarce and cannot provide a final CONCLUSION regarding the use of cochlear implantation in this population. Further long-term experience is required. It is important to clarify patients with superficial siderosis and their families that results are unforeseeable and not as trustworthy as expected in patients with acquired deafness due to pure cochlear involvement.