Introduction: Cochlear implantation has become the standard of care for the treatment of moderate-to-profound bilateral sensorineural hearing loss. However, current technologies, all of which rely on an external sound processor, have intrinsic limitations that prevent certain activities and diagnostics, thus hampering full integration into a patient’s lifestyle. The Envoy Medical (White Bear Lake, MN, USA) Acclaim® fully implanted cochlear implant is a new device currently undergoing testing that has been designed to alleviate many of the current constraints by housing all components within the patient, thus allowing for near-constant use in many environments that are not conducive to a traditional cochlear implant. Methods: As part of an Early Feasibility Study, three adult implant candidates were implanted with the Acclaim® cochlear implant. Surgical video and photography were taken, and initial observations were recorded. Implantation with the Acclaim® device is largely similar to a traditional cochlear implant, with modifications to allow room for the implanted sensor as well as the implantation of a battery in the subcutaneous tissues of the chest. Results: This study demonstrates a step-by-step overview of implanting the Acclaim® and discusses initial insight and experiences with the first three implantations with this new device. Conclusions: All three surgeries proceeded without complication, and at activation, all three patients were hearing through their devices. Surgery is more technically challenging compared to a standard cochlear implant, but the skills needed can all be mastered by a dedicated otologic surgeon.
Preliminary Evidence to Support a De-Escalated Cochlear Implant Programming Paradigm for New Adult Recipients: A Systematic Review
Background: No standard schedule for cochlear implant (CI) programming has been developed, and common practices may have CI recipients seen in excess of what is necessary. The objective of this study was to review evidence for a de-escalated, evidence-based schedule for adult CI programming. Methods: Systematic review was undertaken in March 2023 of PubMed, Scopus, and CINAHL databases using the Preferred Reporting Items for Systemic Reviews and Meta-analyses (PRISMA) guidelines. Studies were included if (1) they evaluated an evidence-based programming/follow-up schedule in new adult CI patients or (2) they evaluated programming or outcomes in a longitudinal fashion such that they could inform CI follow-up strategies. Level of evidence was evaluated using the LEGEND evidence assessment tool. Results: Our review identified 940 studies. After screening with a priori inclusion criteria, 18 studies were ultimately included in this review. Of these, 2 demonstrated feasibility of de-escalated approaches to new adult CI programming. The remainder presented longitudinal speech and programming parameter data that demonstrated relative stability of both categories by 3 to 6 months post-activation. Conclusions: Overall, there is a paucity of literature evaluating any form of evidence-based CI programming or follow-up. Most applicable data derive from longitudinal outcomes featured in studies of other CI features, with only a handful of studies directly evaluating CI programming strategies over time. However, stability in outcomes and programming detailed in the available data supports consideration of a de-escalated programming paradigm that could primarily limit programming to the very early post-activation period (before 3 to 6 months) to enhance patient care and reduce operational strains on cochlear implant programs.
Cochlear Implant Outcomes between Patients with Sporadic and Neurofibromatosis Type 2–Associated Vestibular Schwannoma
Objective Compare 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 Design Historical cohort. Setting Tertiary academic center. Patients Forty-nine patients (52 ears) undergoing cochlear implantation in the setting of ipsilateral sporadic (n = 21) or NF2-associated VS (n = 28). Interventions CI ipsilateral to VS. Main Outcome Measures Auditory thresholds, consonant–nucleus–consonant (CNC) word scores, and AzBio sentences in quiet scores. Results Among 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. Conclusion Select 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.
Multidisciplinary Neurofibromatosis Type 2 Clinic: The Mayo Clinic Experience