scholarly journals Translabyrinthine resection of NF2 associated vestibular schwannoma with cochlear implant insertion

2021 ◽  
Vol 5 (2) ◽  
pp. V14
Author(s):  
Cathal John Hannan ◽  
Priya Sharma ◽  
Matthew Edward Smith ◽  
Laurence Johann Glancz ◽  
Martin O’Driscoll ◽  
...  
Keyword(s):  
Cochlear Implant ◽  
Vestibular Schwannoma ◽  
Neurofibromatosis Type ◽  
Cochlear Nerve ◽  
Csf Leak ◽  
Tumor Control ◽  
Nerve Preservation ◽  
A Minor ◽  
Facial Function

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

scholarly journals Intraoperative Monitoring of the Cochlear Nerve during Neurofibromatosis Type-2 Vestibular Schwannoma Surgery and Description of a “Test Intracochlear Electrode”

2019 ◽  
Vol 80 (01) ◽  
pp. e1-e9 ◽  
Author(s):  
Anand Kasbekar ◽  
Yu Chuen Tam ◽  
Robert Carlyon ◽  
John Deeks ◽  
Neil Donnelly ◽  
...  
Keyword(s):  
Cochlear Implant ◽  
Vestibular Schwannoma ◽  
Intraoperative Monitoring ◽  
Neurofibromatosis Type ◽  
Auditory Brainstem ◽  
Cochlear Nerve ◽  
Auditory Brainstem Responses ◽  
Tertiary Referral Center ◽  
Objective Evidence ◽  
Brainstem Response

Objectives A decision on whether to insert a cochlear implant can be made in neurofibromatosis 2 (NF2) if there is objective evidence of cochlear nerve (CN) function post vestibular schwannoma (VS) excision. We aimed to develop intraoperative CN monitoring to help in this decision. Design We describe the intraoperative monitoring of a patient with NF2 and our stimulating and recording set up. A novel test electrode is used to stimulate the CN electrically. Setting This study was set at a tertiary referral center for skull base pathology. Main outcome measure Preserved auditory brainstem responses leading to cochlear implantation. Results Electrical auditory brainstem response (EABR) waveforms will be displayed from different stages of the operation. A cochlear implant was inserted at the same sitting based on the EABR. Conclusion Electrically evoked CN monitoring can provide objective evidence of CN function after VS excision and aid in the decision-making process of hearing rehabilitation in patients who will be rendered deaf.

scholarly journals Long-Term Outcomes of Stereotactic Radiosurgery for Vestibular Schwannoma Associated with Neurofibromatosis Type 2 in Comparison to Sporadic Schwannoma

Cancers ◽  
2019 ◽  
Vol 11 (10) ◽  
pp. 1498 ◽  
Author(s):  
Shinya ◽  
Hasegawa ◽  
Shin ◽  
Sugiyama ◽  
Kawashima ◽  
...  
Keyword(s):  
Overall Survival ◽  
Vestibular Schwannoma ◽  
Therapeutic Option ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Tumor Control ◽  
Minimal Invasiveness ◽  
Significant Difference

The efficacy of radiosurgery for neurofibromatosis type 2 (NF2)-associated vestibular schwannoma (VS) remains debatable. We retrospectively analyzed radiosurgical outcomes for NF2-associated VS compared to sporadic VS using our database of 422 consecutive VS patients. Twenty-five patients with 30 NF2-associated VSs with a mean follow-up of 121 months were identified. NF2-associated VSs exhibited excellent tumor control (10-year cumulative rate, 92% vs. 92% in sporadic VSs; p = 0.945) and worse overall survival (73% vs. 97%; p = 0.005), mainly due to tumor progression other than the treated VSs. The presence of NF2 was not associated with failed tumor control via multivariate Cox proportional hazard analyses. No difference in radiation-induced adverse events (RAEs) was confirmed between cohorts, and prescription dose (hazard ratio 8.30, 95% confidence interval 3.19–21.62, p < 0.001) was confirmed as a risk for cranial nerve injuries via multivariate analysis. Further analysis after propensity score matching using age, volume, and sex as covariates showed that NF2-associated VSs exhibited excellent local control (100% vs. 93%; p = 0.240) and worse overall survival (67% vs. 100%; p = 0.002) with no significant difference in RAEs. Excellent long-term tumor control and minimal invasiveness may make radiosurgery a favorable therapeutic option for NF2 patients with small to medium VS, preferably with non-functional hearing or deafness in combination with postoperative tumor growth or progressive non-operated tumors, or with functional hearing by patients’ wish.

Ipsilateral Cochlear Implantation After Cochlear Nerve Preserving Vestibular Schwannoma Surgery in Patients With Neurofibromatosis Type 2

2014 ◽  
Vol 35 (1) ◽  
pp. 43-51 ◽  
Author(s):  
Simon Kingsley Wickham Lloyd ◽  
Fergal John Glynn ◽  
Scott Alexander Rutherford ◽  
Andrew Thomas King ◽  
Deborah Jane Mawman ◽  
...  
Keyword(s):  
Vestibular Schwannoma ◽  
Cochlear Implantation ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Cochlear Nerve

Clinical course of vestibular schwannoma in pediatric neurofibromatosis Type 2

2014 ◽  
Vol 13 (6) ◽  
pp. 650-657 ◽  
Author(s):  
Jung Won Choi ◽  
Ji Yeoun Lee ◽  
Ji Hoon Phi ◽  
Kyu-Chang Wang ◽  
Hyun-Tai Chung ◽  
...  
Keyword(s):  
Growth Rate ◽  
Vestibular Schwannoma ◽  
Clinical Course ◽  
Clinical Manifestations ◽  
Neurofibromatosis Type ◽  
Tumor Control ◽  
Natural Growth ◽  
The Mean ◽  
Hearing Outcomes

Object Neurofibromatosis Type 2 (NF2) is an autosomal-dominant inherited disease, characterized by multiple neoplasia syndromes, including meningioma, schwannoma, glioma, and ependymoma. In this report, the authors present their clinical experience with pediatric NF2 patients. In particular, they focused on the clinical course of vestibular schwannoma (VS), including the natural growth rate, tumor control, and functional hearing outcomes. Methods From May 1988 to June 2012, the authors recruited patients who were younger than 18 years and fulfilled the Manchester criteria. In total, 25 patients were enrolled in this study. The authors analyzed the clinical course of these patients. In addition, they measured the natural growth rate of VS before any treatment in these children with NF2. Then, they evaluated the tumor control rate and functional hearing outcomes after the treatment of VS. Results The mean age at the onset of NF2-related symptoms was 9.9 ± 4.5 years (mean ± SD, range 1–17 years). The mean age at the diagnosis of NF2 was 12.9 ± 2.9 years (range 5–17 years). The mean follow-up period was 89.3 months (range 12–311 months). As initial manifestations, nonvestibular symptoms were frequently observed in pediatric patients with NF2. The mean natural growth rate of VS was 0.33 ± 0.41 cm3/year (range 0–1.35 cm3/year). The tumor control rate of VS was 35.3% at 3 years after Gamma Knife surgery (GKS). The actuarial rate of useful hearing preservation was 67% in the 1st year and 53% in the 5th year after GKS. Conclusions Clinical manifestations in children with NF2 were highly variable, compared with their adult counterparts. The natural growth rate of VS in children is slow, and this oncological feature may explain the diverse clinical manifestations besides vestibular symptoms in children with NF2. The treatment outcome of GKS for VS in children with NF2 was not favorable compared with previous reports of affected adults.

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

2021 ◽  
Author(s):  
Margaret J Butler ◽  
Cameron C Wick ◽  
Matthew A Shew ◽  
Michael R Chicoine ◽  
Amanda J Ortmann ◽  
...  
Keyword(s):  
Vestibular Schwannoma ◽  
Cochlear Implantation ◽  
Case Series ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Cochlear Nerve ◽  
Tumor Removal ◽  
Nerve Monitoring ◽  
Brainstem Response

Abstract 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.

scholarly journals Contextualizing the Modern Epidemiology of Neurofibromatosis Type 2 in an Era of Heightened Detection of Sporadic Vestibular Schwannoma

2020 ◽  
Vol 41 (4) ◽  
pp. e501-e506 ◽  
Author(s):  
Eric M. Dowling ◽  
John P. Marinelli ◽  
Christine M. Lohse ◽  
Matthew L. Carlson
Keyword(s):  
Vestibular Schwannoma ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2

Cochlear Implantation in Patients with Neurofibromatosis Type 2 and Bilateral Vestibular Schwannoma

2006 ◽  
Vol 27 (4) ◽  
pp. 512-518 ◽  
Author(s):  
Lawrence R. Lustig ◽  
Jennifer Yeagle ◽  
Colin L. W. Driscoll ◽  
Nikolas Blevins ◽  
Howard Francis ◽  
...  
Keyword(s):  
Vestibular Schwannoma ◽  
Cochlear Implantation ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2
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