Objectives: Historically, eventual loss of cochlear nerve function has limited patients with neurofibromatosis type 2 (NF2) to auditory brainstem implants (ABI), which in general are less effective than modern cochlear implants (CI). Our objective is to evaluate hearing outcomes following ipsilateral cochlear implantation in patients with NF2 and irradiated vestibular schwannomas (VS), and sporadic VS that have been irradiated or observed. Methods: Multi-center retrospective analysis of ipsilateral cochlear implantation in the presence of observed and irradiated VS. MESH search in NCBI PubMed database between 1992 and 2019 for reported cases of cochlear implantation with unresected vestibular schwannoma. Results: Seven patients underwent ipsilateral cochlear implantation in the presence of observed or irradiated vestibular schwannomas. Four patients had sporadic tumors with severe-profound contralateral hearing loss caused by presbycusis/hereditary sensorineural hearing loss, and three patients with NF2 lost contralateral hearing after prior surgical resection. Prior to implantation, one VS was observed without growth for a period of 7 years and the others were treated with radiotherapy. Mean post-operative sentence score was 63.9% (range 48-91) at an average of 28 (range 2-84) months follow up. All patients in this cohort obtained open set speech perception. While analysis of the literature is limited by heterogenous data reporting, 85% of implants with observed schwannomas achieved some open set perception, and 67% of patients previously radiated schwannomas. Furthermore, blending literature outcomes for post implantation sentence testing in quiet without lip-reading show 59.0 ± 35% for patients with CI and observed tumors and 55.7 ± 35% for patients with radiated tumors, with both groups ranging 0 to 100%. Conclusion: This retrospective series and literature review highlight that hearing outcomes with CI for VS patients are superior to those achieved with ABI. However, important considerations including imaging, delayed hearing loss, and observation time cannot be ignored in this population.
Electrical auditory brainstem responses during cochlear implantation
