UNASSIGNED: NF2-schwannomatosis (NF2) is an autosomal dominant disorder prone to hearing loss. Auditory brainstem implants (ABIs) offer a promising solution for hearing rehabilitation in NF2.
UNASSIGNED: To synthesize existing literature on ABI implantation in NF2, focusing on audiological outcomes and ABI-related complications.
UNASSIGNED: The systematic review followed PRISMA guidelines and was registered in the PROSPERO database (CRD42022362155). Relevant studies were identified by searching PubMed, EMBASE, CENTRAL, CMB, and CNKI from inception to August 2023. Data on environmental sound discrimination, open-set discrimination, closed-set discrimination, and ABI-related complications were extracted and subjected to meta-analysis. Publication bias was evaluated using funnel plots and Egger\'s test.
UNASSIGNED: Thirty-three studies were included. The pooled estimate was 58% (95% CI 49-66%) for environmental sound discrimination and 55% (95% CI 40-69%) for closed-set discrimination. Regarding open-set discrimination, the pooled estimates were 30% (95% CI 19-42%) for sound only, 46% (95% CI 37-54%) for lip-reading only, and 63% (95% CI 55-70%) for sound plus lip-reading. The pooled occurrence of ABI-related complications was 33% (95% CI 15-52%).
UNASSIGNED: This meta-analysis underscores the effectiveness and safety of ABIs in NF2, providing valuable insights for evidence-based decision-making and hearing rehabilitation strategies.

BACKGROUND: The auditory brainstem implant (ABI) is a significant treatment to restore hearing sensations for neurofibromatosis type 2 (NF2) patients. However, there is no ideal method in assisting the placement of ABIs. In this case series, intraoperative cochlear nucleus mapping was performed in awake craniotomy to help guide the placement of the electrode array.
METHODS: We applied the asleep-awake-asleep technique for awake craniotomy and hearing test via the retrosigmoid approach for acoustic neuroma resections and ABIs, using mechanical ventilation with a laryngeal mask during the asleep phases, utilizing a ropivacaine-based regional anesthesia, and sevoflurane combined with propofol/remifentanil as the sedative/analgesic agents in four NF2 patients. ABI electrode arrays were placed in the awake phase with successful intraoperative hearing tests in three patients. There was one uncooperative patient whose awake hearing test needed to be aborted. In all cases, tumor resection and ABI were performed safely. Satisfactory electrode effectiveness was achieved in awake ABI placement.
CONCLUSIONS: This case series suggests that awake craniotomy with an intraoperative hearing test for ABI placement is safe and well tolerated. Awake craniotomy is beneficial for improving the accuracy of ABI electrode placement and meanwhile reduces non-auditory side effects.

To outline a possible decision-making process for sporadic vestibular schwannoma (VS) with contralateral nonserviceable hearing at diagnosis.
Retrospective case series.
Diagnosed VS was studied in a tertiary referral center from 1995 to 2013.
Twenty-eight patients were included, with a mean follow-up of 6.9 years (range = 0.5-20 years). Ten were stage 1, 10 were stage 2, five were stage 3, and three were stage 4. Ipsilateral hearing levels were A (n = 3), B (n = 10), C (n = 7) and D (n = 8) American Academy of Otolaryngology-Head and Neck Surgery classification. Contralateral hearing levels were C (n = 11) and D (n = 17). Initial VS management included surveillance (n = 12) or surgery (n = 16), and four patients were later treated with surgery (n = 3) or stereotactic irradiation (n = 1). Hearing was improved by hearing aids and/or etiological treatment (n = 8), restored by contralateral (n = 15) or ipsilateral (n = 4) cochlear implants (CIs), or ipsilateral auditory brainstem implants (ABIs) (n = 3). Finally, 18 CIs were active daily; 14 of them presented high or moderate benefit with mean open-set dissyllabic word scores (WRS) of 58.1% and sentence recognition scores (SRS) of 69.7%, but only one ABI was still active (WRS of 70% and SRS of 87% with lip reading).
When early removal of VS was not necessary, contralateral CI or etiological treatment for hearing loss might be recommended initially. Ipsilateral CI is proposed, whereas VS should be operated on if previous hearing restoration was not successful. ABI should be reserved for the rare cases where a contralateral CI could not be implanted or the cochlear nerve was sectioned during VS removal.
3 Laryngoscope, 130:E407-E415, 2020.

The main manifestation of neurofibromatosis type 2 (NF2) is the development of bilateral vestibular schwannomas (VS). Consequently, one of the most severe functional sequelae is bilateral sensorineural hearing loss, caused by spontaneous tumor progression and/or treatment-related damage (surgery or radiosurgery). Preserving or restoring hearing is still challenging in NF2 no matter the strategy applied to each individual based on the natural history of VS. In this review, the different strategies for hearing preservation or rehabilitation are discussed and illustrated by several cases. A decisional algorithm for NF2 patients with VS is proposed that takes into consideration the tumor size and hearing level.

OBJECTIVE: Auditory brainstem implants (ABIs) may be the only opportunity for patients with NF2 to regain some sense of hearing sensation. However, only a very small number of individuals achieved open-set speech understanding and high sentence scores. Suboptimal placement of the ABI electrode array over the cochlear nucleus may be one of main factors for poor auditory performance. In the current study, we present a method of awake craniotomy to assist with ABI placement.
METHODS: Awake surgery and hearing test via the retrosigmoid approach were performed for vestibular schwannoma resections and auditory brainstem implantations in four patients with NF2. Auditory outcomes and complications were assessed postoperatively.
RESULTS: Three of 4 patients who underwent awake craniotomy during ABI surgery received reproducible auditory sensations intraoperatively. Satisfactory numbers of effective electrodes, threshold levels and distinct pitches were achieved in the wake-up hearing test. In addition, relatively few electrodes produced non-auditory percepts. There was no serious complication attributable to the ABI or awake craniotomy.
CONCLUSIONS: It is safe and well tolerated for neurofibromatosis type 2 (NF2) patients using awake craniotomy during auditory brainstem implantation. This method can potentially improve the localization accuracy of the cochlear nucleus during surgery.