Ménière's disease (MD) that results in bilateral severe to profound sensorineural hearing loss is a rare indication for cochlear implantation; only a few studies exist documenting performance in these patients. The primary objective was to compare the difference in preoperative to 12-month postoperative speech perception scores among subjects with MD and controls. Groupwise comparisons of secondary postoperative outcomes (Tinnitus Handicap Inventory [THI] scores, 36-Item Short Form [SF-36] scores, and postoperative dizziness) were also performed. A retrospective cohort study was conducted. Subjects with MD and controls matched by age, device manufacturer and model, preoperative sentence score, and sentence test used for preimplantation and postimplantation performance assessments were identified from 1,130 patients in the prospectively maintained cochlear implant database at our center. Speech perception, THI, and SF-36 scores and demographic variables were obtained from the database. Vestibular outcomes were obtained by retrospective chart review. Statistical comparisons were performed to compare preoperative to postoperative change between groups. Twenty patients with MD were identified. At 1 year after CI, improvements in sentence and word understanding did not differ in magnitude from the controls. Tinnitus was reduced significantly in patients with MD, whereas there was a trend for improvement in the controls. Quality of life as measured by the SF-36 improved in both groups. Patients with MD had significant improvements in 1 domain compared with 5 domains for the controls. Subjects with MD had significantly more chronic dizziness in the postoperative period than did controls. Patients with MD who have bilateral severe to profound sensorineural hearing loss benefit significantly from CI. Ongoing dizziness in some patients with MD may result in quality of life improvements that are slightly less than seen for the average adult patient with CI. Larger studies are needed to corroborate the results.