Impact of cochlear implantation on the management strategy of patients with neurofibromatosis type 2

Tan, Haisong; Jia, Hong-Ti; Li, Yun; Zhang, Zhihua; Zhu, Weidong; Cai, Yun; Wang, Zhaoyan; Wu, Hao

doi:10.1007/s00405-018-5127-9

Cited by 22 publications

(25 citation statements)

References 26 publications

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“…4 Since that time, additional data have emerged supporting safe and effective cochlear implantation in patients with unresected vestibular schwannomas following treatment with radiation or a period of observation of small, stable schwannomas. 2,3,[5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21] Our study aims to compile the current data regarding hearing outcomes for cochlear implantation in ears with non-operated tumors and also add to the recent growing body of evidence with this multi-center series.…”

Section: Introductionmentioning

confidence: 99%

Ipsilateral Cochlear Implantation in the Presence of Observed and Irradiated Vestibular Schwannomas

Urban

Moore

Kwarta

et al. 2020

Ann Otol Rhinol Laryngol

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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.

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Section: Introductionmentioning

confidence: 99%

Ipsilateral Cochlear Implantation in the Presence of Observed and Irradiated Vestibular Schwannomas

Urban

Moore

Kwarta

et al. 2020

Ann Otol Rhinol Laryngol

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“…Again, the results are less good than in non-tumor patients needing an acoustic implant 10 . In few selected cases and series, good results could be reported with CI in NF2 23 with speech discrimination up to 63% in 10 of 12 patients. Lloyd et al found better benefit from CI, if the related tumor was neither treated by surgery nor by radiosurgery 8 .…”

Section: Discussionmentioning

confidence: 92%

“…In most cases, ABI was implanted on the only hearing side, although in some cases it was implanted on the first side with the device switched off until contralateral total hearing loss was present (Sleeper ABI) 16 . The ABI showed the expected auditory benefit with lip-reading aid in most cases.…”

Section: The View Of the Expert: A Free Choice Topicmentioning

confidence: 99%

Surgery of the lateral skull base: a 50-year endeavour

Zanoletti¹,

Mazzoni²,

Martini³

et al. 2019

Acta Otorhinolaryngol Ital

100

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SUMMARY Disregarding the widely used division of skull base into anterior and lateral, since the skull base should be conceived as a single anatomic structure, it was to our convenience to group all those approaches that run from the antero-lateral, pure lateral and postero-lateral side of the skull base as “Surgery of the lateral skull base”. “50 years of endeavour” points to the great effort which has been made over the last decades, when more and more difficult surgeries were performed by reducing morbidity. The principle of lateral skull base surgery, “remove skull base bone to approach the base itself and the adjacent sites of the endo-esocranium”, was then combined with function preservation and with tailoring surgery to the pathology. The concept that histology dictates the extent of resection, balancing the intrinsic morbidity of each approach was the object of the first section of the present report. The main surgical approaches were described in the second section and were conceived not as a step-by-step description of technique, but as the highlighthening of the surgical principles. The third section was centered on open issues related to the tumor and its treatment. The topic of vestibular schwannoma was investigated with the current debate on observation, hearing preservation surgery, hearing rehabilitation, radiotherapy and the recent efforts to detect biological markers able to predict tumor growth. Jugular foramen paragangliomas were treated in the frame of radical or partial surgery, radiotherapy, partial “tailored” surgery and observation. Surgery on meningioma was debated from the point of view of the neurosurgeon and of the otologist. Endolymphatic sac tumors and malignant tumors of the external auditory canal were also treated, as well as chordomas, chondrosarcomas and petrous bone cholesteatomas. Finally, the fourth section focused on free-choice topics which were assigned to aknowledged experts. The aim of this work was attempting to report the state of the art of the lateral skull base surgery after 50 years of hard work and, above all, to raise questions on those issues which still need an answer, as to allow progress in knowledge through sharing of various experiences. At the end of the reading, if more doubts remain rather than certainties, the aim of this work will probably be achieved.

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“…In conclusion, these three IVS cases were initially treated in the early 2000s by surgical removal which might not be at the present time the first-line treatment for NF2 apart from cases with life-threatening large VS (26,27). However, this study demonstrates that removal of IVS with concomitant CI implantation provides a long-term benefit from CI, although it could be altered by the occurrence of other intracanalicular and/or CPA NF2-related tumors, the treatment of which might restore the initial CI benefit.…”

Section: Discussionmentioning

confidence: 96%

NF2-Related Intravestibular Schwannomas: Long-Term Outcomes of Cochlear Implantation

Jia

Nguyen

Hochet

et al. 2020

Otology &Amp; Neurotology

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Objective: Intravestibular schwannomas (IVSs) are uncommon tumors in Neurofibromatosis type 2 (NF2) and are mainly associated with multiple internal auditory meatus (IAM) and cerebellopontine angle (CPA) tumors. They usually induce profound hearing loss which can be rehabilitated by cochlear implantation (CI). The aim of this study was to analyze the longterm outcomes of CI during the unpredictable evolution of NF2 disease. Study Design: Retrospective case review. Setting: Tertiary referral center. Patients: Three adults with neurofibromatosis type 2 and intravestibular schwannomas, and who were cochlear implant recipients. Interventions: Periodic radiologic follow-up, tumor resection and hearing rehabilitation. Main outcome measures: Audiological evolution, tumor evolution, surgical outcome, cochlear implant outcome. Results: Three NF2 patients (mean age at diagnosis, 21 ± 2.5 years) were identified with IVS in the period between 2000 and 2014. IVS were first observed by serial MRI and profound hearing loss occurred in this ear after 4±1.5 years of follow-up. IVS were removed via a translabyrinthine (TL) approach, and ipsilateral cochlear implantations were simultaneously performed. In two patients, large contralateral CPA tumors had previously been removed without hearing preservation, whereas in the third patient, a small, growing contralateral VS was excised via a retrosigmoid approach 6 months after IVS removal/cochlear implantation with serviceable hearing preservation. In all cases, CI provided good hearing outcomes. In two cases, hearing outcomes were even better for more than 5 years when ipsilateral intracanalicular vestibular schwannomas were removed in either the same or subsequent procedures. Conclusions: Rehabilitation of hearing with CI provides a favorable long-term outcome in patients with NF2-related IVS which could be altered by the occurrence of other intracanalicular and/or CPA NF2-related tumors.

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Impact of cochlear implantation on the management strategy of patients with neurofibromatosis type 2

Abstract: CI is an effective option in auditory rehabilitation and should be considered primarily for NF2 patients with intact cochlear nerve. EPS might be a predictor for cochlear implant performance. Good contralateral hearing may present a barrier to daily use.

Cited by 22 publications

References 26 publications

Ipsilateral Cochlear Implantation in the Presence of Observed and Irradiated Vestibular Schwannomas

Ipsilateral Cochlear Implantation in the Presence of Observed and Irradiated Vestibular Schwannomas

Surgery of the lateral skull base: a 50-year endeavour

NF2-Related Intravestibular Schwannomas: Long-Term Outcomes of Cochlear Implantation

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