Cochlear Implant Outcomes between Patients with Sporadic and Neurofibromatosis Type 2-Associated Vestibular Schwannoma

ObjectiveCompare cochlear implant (CI) performance between patients with ipsilateral sporadic vestibular schwannoma (VS) and NF2-related schwannomatosis (NF2). Compare CI performance according to VS management modality.Study DesignHistorical cohort.SettingTertiary academic center.PatientsForty-nine patients (52 ears) undergoing cochlear implantation in the setting of ipsilateral sporadic (n = 21) or NF2-associated VS (n = 28).InterventionsCI ipsilateral to VS.Main Outcome MeasuresAuditory thresholds, consonant-nucleus-consonant (CNC) word scores, and AzBio sentences in quiet scores.ResultsAmong all patients, median post-CI pure tone average was 28 dB HL (interquartile range [IQR], 21-38), CNC word score was 39% (IQR, 6-62), and AzBio sentences in quiet score was 60% (IQR, 11-83) at a median of 12.5 months postimplantation. Despite the NF2 cohort having larger tumors, when comparing patients with sporadic versus NF2-associated VS, there were no statistically significant differences in CNC word (49% [30-70] vs. 31% [0-52]) or AzBio sentences in quiet (66% [28-80] vs. 57% [5-83]) scores. Regardless of NF2 status, all patients managed with observation, and radiosurgery achieved open-set speech. In patients who underwent microsurgery, 6 (46%) of 13 with NF2 achieved open-set speech recognition compared with 4 (67%) of 6 with sporadic disease.ConclusionSelect patients with VS achieve successful hearing rehabilitation with a CI. In this cohort, tumor management strategy significantly influenced CI performance, whereas differences in NF2 status exhibited less effect. Specifically, all patients managed with observation or radiosurgery achieved open-set speech perception, whereas approximately half of people with NF2-related VS and two-thirds of people with sporadic VS achieved this outcome after tumor microsurgery. When disease permits, observation and radiosurgery should be considered in patients who may later pursue a CI.