Functional Outcomes of Cochlear Implantation in Children with Bilateral Cochlear Nerve Aplasia

Many otologists face a dilemma in the decision-making process of surgical management of patients with cochlear nerve (CN) aplasia. Currently, evidence on cochlear implantation (CI) outcomes in patients with CN aplasia is limited. We scrutinized functional outcomes in 37 ears of 21 children with bilateral CN aplasia who underwent unilateral or bilateral CI based on cross-sectional and longitudinal assessments. The Categories of Auditory Performance (CAP) scores gradually improved throughout the 3-year follow-up; however, variable outcomes existed between individuals. Specifically, the majority of recipients with a 1-year postoperative CAP score ≤1 remained steady or achieved awareness of environmental sounds, while recipients with early stage hearing benefit had markedly improved auditory performance and could possibly discriminate some speech without lipreading. Meanwhile, intraoperative electrically evoked compound action potential was not correlated with postoperative CAP score. The dynamic range between T and C levels remained unchanged. Our results further refine those of previous studies on the clinical feasibility of CI as the first treatment modality to elicit favorable auditory performance in children with CN aplasia. However, special attention should be paid to pediatric patients with an early postoperative CAP score ≤1 for identification of unsuccessful cochlear implants and switching to auditory brainstem implants.

Translabyrinthine resection of NF2 associated vestibular schwannoma with cochlear implant insertion

The authors present the case of a 24-year-old female with neurofibromatosis type 2. Growth of the left vestibular schwannoma and progressive hearing loss prompted the decision to proceed to translabyrinthine resection with cochlear nerve preservation and cochlear implant insertion. Complete resection with preservation of the facial and cochlear nerves was achieved. The patient had grade 1 facial function and was discharged on postoperative day 4 following suturing of a minor CSF leak. This case highlights the feasibility of cochlear nerve preservation and cochlear implant insertion in appropriately selected patients, offering a combination of effective tumor control and hearing rehabilitation. The video can be found here: https://stream.cadmore.media/r10.3171/2021.7.FOCVID21122

Auditory and speech performance after unilateral cochlear implantation for cochlear nerve canal stenosis

To explore the correlation between the width of the bony cochlear nerve canal (CNC) and long-term auditory rehabilitation after unilateral cochlear implantation (CI) in pediatric patients with congenital deafness and bilateral cochlear nerve canal stenosis (CNCS). A retrospective review was performed on 10 patients with bilateral CNCS and bilateral congenital profound hearing loss who each underwent unilateral cochlear implantation. The width of the CNC was determined on computed tomography (CT) imaging and following CI, auditory and speech performance following CI were graded using categories of auditory performance (CAP), speech intelligibility rating (SIR), and the meaningful auditory integration scale (MAIS) at 24 months following implantation. No correlation was noted between CAP score and CNCS at 24 months post CI ( P > .05). A positive correlation was noted between SIR score and CNC width (ρ = .81, P < .05). Similarly, a positive correlation was noted between MAIS and CNC width (ρ = .71, P < .05). The width of the CNC in patients with CNCS is positively correlated with some long-term auditory and speech outcomes after CI.

Intraoperative Cochlear Nerve Monitoring for Vestibular Schwannoma Resection and Simultaneous Cochlear Implantation in Neurofibromatosis Type 2: A Case Series

BACKGROUND Neurofibromatosis type 2 (NF2) often results in profound hearing loss and cochlear implantation is an emerging hearing rehabilitation option. However, cochlear implant (CI) outcomes in this population vary, and intraoperative monitoring to predict cochlear nerve viability and subsequent outcomes is not well-established. OBJECTIVE To review the use of intraoperative electrically evoked cochlear nerve monitoring in patients with NF2 simultaneous translabyrinthine (TL) vestibular schwannoma (VS) resection and cochlear implantation. METHODS A retrospective review was performed of 3 patients with NF2 that underwent simultaneous TL VS resection and cochlear implantation with electrical auditory brainstem response (eABR) measured throughout tumor resection. Patient demographics, preoperative assessments, surgical procedures, and outcomes were reviewed. RESULTS Patients 1 and 3 had a reliable eABR throughout tumor removal. Patient 2 had eABR pretumor removal, but post-tumor removal eABR presence could not be reliably determined because of electrical artifact interference. All patients achieved auditory percepts upon CI activation. Patients 1 and 2 experienced a decline in CI performance after 1 yr and after 3 mo, respectively. Patient 3 continues to perform well at 9 mo. Patients 2 and 3 are daily users of their CI. CONCLUSION Cochlear implantation is attainable in cases of NF2-associated VS resection. Intraoperative eABR may facilitate cochlear nerve preservation during tumor removal, though more data and long-term outcomes are needed to refine eABR methodology and predictive value for this population.

Comparison of ABR and ASSR using narrow-band-chirp-stimuli in children with cochlear malformation and/or cochlear nerve hypoplasia suffering from severe/profound hearing loss

Objectives In pediatric audiology, objective techniques for hearing threshold estimation in infants and children with profound or severe hearing loss play a key role. Auditory brainstem responses (ABR) and auditory steady-state responses (ASSR) are available for frequency-dependent hearing threshold estimations and both techniques show strong correlations but sometimes with considerable differences. The aim of the study was to compare hearing threshold estimations in children with and without cochlear and cochlear nerve malformations. Methods Two groups with profound or severe hearing loss were retrospectively compared. In 20 ears (15 children) with malformation of the inner ear and/or cochlear nerve hypoplasia and a control group of 20 ears (11 children) without malformation, ABR were measured with the Interacoustics Eclipse EP25 ABR system® (Denmark) with narrow-band CE-chirps® at 500, 1000, 2000 and 4000 Hz and compared to ASSR at the same center frequencies under similar conditions. Results ABR and ASSR correlated significantly in both groups (r = 0.413 in malformation group, r = 0.82 in control group). The malformation group showed a significantly lower percentage of “equal” hearing threshold estimations than the control group. In detail, patients with isolated cochlear malformation did not differ significantly from the control group, whereas patients with cochlear nerve hypoplasia showed significantly greater differences. Conclusion ABR and ASSR should be used jointly in the diagnostic approach in children with suspected profound or severe hearing loss. A great difference in hearing threshold estimation between these techniques could hint at the involvement of cochlear nerve or cochlear nerve hypoplasia itself.

Prophylactic nimodipine treatment for hearing preservation after vestibular schwannoma surgery: study protocol of a randomized multi-center phase III trial—AkniPro 2

Background A previously performed phase III trial on 112 subjects investigating prophylactic nimodipine treatment in vestibular schwannoma (VS) surgery showed no clear beneficial effects on preservation of facial and cochlear nerve functions, though it should be considered that protection of facial nerve function was the primary outcome. However, the risk for postoperative hearing loss was halved in the nimodipine group compared to the control group (OR 0.49; 95% CI 0.18–1.30; p = 0.15). Accordingly, this phase III extension trial investigates the efficacy and safety of prophylactic nimodipine for hearing preservation in VS surgery. Methods This is a randomized, multi-center, two-armed, open-label phase III trial with blinded expert review and two-stage with interim analysis. Three hundred thirty-six adults with the indication for microsurgical removal of VS (Koos I–IV) and serviceable preoperative hearing (Gardner-Robertson scale (GR) 1–3) are assigned to either the therapy (intravenous nimodipine 1–2 mg/h from the day before surgery until the fifth postoperative day and standard of care) or the control group (surgery only and standard of care). The primary endpoint of the trial is postoperative cochlear nerve function measured before discharge according to GR 1–3 versus GR 4–5 (binary). Hearing function will be determined by pre- and postoperative audiometry with speech discrimination, which will be evaluated by a blinded expert reviewer. Furthermore, patient-reported outcomes using standardized questionnaires will be analyzed. Discussion Prophylactic parenteral nimodipine treatment may have a positive effect on hearing preservation in VS surgery and would improve patient’s quality of life. Further secondary analyses are planned. Except for dose-depending hypotension, nimodipine is known as a safe drug. In the future, prophylactic nimodipine treatment may be recommended as a routine medication in VS surgery. VS can be considered as an ideal model for clinical evaluation of neuroprotection, since hearing outcome can be classified by well-recognized criteria. The beneficial effect of nimodipine may be transferable to other surgical procedures with nerves at risk and may have impact on basic research. Trial registration EudraCT 2019-002317-19, DRKS00019107. 8th May 2020.

“Save the Nerve”: Technical Nuances for Hearing Preservation and Restoration in Vestibular Schwannoma Surgery: 2-Dimensional Operative Video

Hearing loss is a significant disability that inflects dysfunction and affects the patient quality of life. Consequently, hearing preservation and the potential of hearing restoration are prized quests in the management of vestibular schwannoma.1 Although small intracanalicular vestibular schwannomas are commonly observed, progressive hearing loss occurs despite the absence of tumor growth; hence, surgical resection can be performed with the sole aim of hearing preservation in well-informed and eager patients. Hearing preservation by surgical resection has proven to be durable.1-4 In this group of patients, we concur with Yamakami et al2 that vascularized meatal flap to reconstruct the canal helps prevent scarring of the cochlear nerve and provides cerebrospinal fluid (CSF) bathing to the cochlear nerve, yielding better long-term hearing preservation. With larger tumors and more severe hearing loss at presentation, microsurgical resection should aim at preserving the cochlear nerve, a goal frequently achievable, which offers the potential for hearing restoration with cochlear implants.3 The results of cochlear implants in restoration of severe hearing loss have been to say the least most impressive.5 We demonstrate these 2 frequently encountered clinical situations with 2 surgical videos showing specific surgical tenets, including intra-arachnoidal dissection, medial to lateral manipulation of the tumor, preservation of the labyrinthine artery, as well as reconstruction of the internal auditory canal.2,3,6,7 The patients consented to the surgery and to the publication of their picture in a surgical video. Illustration in video © 1997 O. Al-Mefty. Used with permission. All rights reserved.