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Published in: Indian Journal of Otolaryngology and Head & Neck Surgery 3/2021

01-09-2021 | Vestibular Schwannoma | Clinical Report

Neurofibromatosis 2: Primary Modality of Hearing Rehabilitation with Cochlear Implant

Authors: Dathathri Anantaramaiah Halyur, Praveen H. Rayanagoudar, Apurva Kumar, Sunil Narayan Dutt

Published in: Indian Journal of Otolaryngology and Head & Neck Surgery | Issue 3/2021

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Abstract

Auditory Brainstem Implants have been recommended as the gold standard in hearing rehabilitation of Neurofibromatosis Type 2 patients who lose hearing completely in both the ears and the cochlear nerves are not stimulable. Some patients have undergone cochlear implantation, in whom the cochlear nerve was spared during surgery or have undergone stereotactic radiotherapy preserving the function of the cochlear nerve. Here we report a case in whom we chose cochlear implantation prior to any definitive treatment for the tumour itself during the ‘wait and watch’ period. The reasons in favour of this approach have been discussed in this article. Post switch-on the implant is benefitting the patient satisfactorily and she is on regular follow up for monitoring the bilateral tumours.
Literature
1.
go back to reference Evans DG (2009) Neurofibromatosis 2 Bilateral acoustic neurofibromatosis, central neurofibromatosis, NF2, neurofibromatosis type II. Genet Med 11:599CrossRef Evans DG (2009) Neurofibromatosis 2 Bilateral acoustic neurofibromatosis, central neurofibromatosis, NF2, neurofibromatosis type II. Genet Med 11:599CrossRef
2.
go back to reference Dutt SN, Kumar A (2019) The methodology and electro-physiological classification of pre-operative trans-tympanic electrically-evoked auditory brainstem response (TT-EABR). Indian J Otolaryngol Head Neck Surg 71:1–4CrossRef Dutt SN, Kumar A (2019) The methodology and electro-physiological classification of pre-operative trans-tympanic electrically-evoked auditory brainstem response (TT-EABR). Indian J Otolaryngol Head Neck Surg 71:1–4CrossRef
3.
go back to reference Archbold S, Lutman M, Marshall DH (1995) Categories of auditory performance. Ann Otol Rhinol Laryngol 166:312–314 Archbold S, Lutman M, Marshall DH (1995) Categories of auditory performance. Ann Otol Rhinol Laryngol 166:312–314
4.
go back to reference Allen MC, Nikolopoulos TP, O’Donoghue GM (1998) Speech intelligibility in children after cochlear implantation. Am J Otol 19:742–746PubMed Allen MC, Nikolopoulos TP, O’Donoghue GM (1998) Speech intelligibility in children after cochlear implantation. Am J Otol 19:742–746PubMed
5.
go back to reference Linthicum H, Brackmann DE (1980) Bilateral acoustic tumours. A diagnostic and surgical challenge. Arch Otolaryngol 106(12):729–733CrossRef Linthicum H, Brackmann DE (1980) Bilateral acoustic tumours. A diagnostic and surgical challenge. Arch Otolaryngol 106(12):729–733CrossRef
6.
go back to reference Abaza MM, Makariou E, Armstrong M, Lalwani AK (1996) Growth rate characteristics of acoustic neuromas associated with neurofibromatosis type 2. Laryngoscope 106:694CrossRef Abaza MM, Makariou E, Armstrong M, Lalwani AK (1996) Growth rate characteristics of acoustic neuromas associated with neurofibromatosis type 2. Laryngoscope 106:694CrossRef
7.
go back to reference Asthagiri AR et al (2012) Mechanisms of hearing loss in neurofirbomatosis type 2. PLoS ONE 7(9):e46132 Epub 2012 Sep 26CrossRef Asthagiri AR et al (2012) Mechanisms of hearing loss in neurofirbomatosis type 2. PLoS ONE 7(9):e46132 Epub 2012 Sep 26CrossRef
8.
go back to reference Hoffman RA, Kohan D, Cohen NL (1992) Cochlear implants in the management of bilateral acoustic neuromas. Am J Otol 13:525–528PubMed Hoffman RA, Kohan D, Cohen NL (1992) Cochlear implants in the management of bilateral acoustic neuromas. Am J Otol 13:525–528PubMed
9.
go back to reference Dewan R, Pemov A, Kim HJ et al (2015) Evidence of polyclonality in neurofibromatosis type 2-associated multilobulated vestibular schwannomas. Neuro Oncol 17:566CrossRef Dewan R, Pemov A, Kim HJ et al (2015) Evidence of polyclonality in neurofibromatosis type 2-associated multilobulated vestibular schwannomas. Neuro Oncol 17:566CrossRef
10.
go back to reference Masuda A, Fisher LM, Oppenheimer ML et al (2004) Hearing changes after diagnosis in neurofibromatosis type 2. Otol Neurotol 25:150CrossRef Masuda A, Fisher LM, Oppenheimer ML et al (2004) Hearing changes after diagnosis in neurofibromatosis type 2. Otol Neurotol 25:150CrossRef
11.
go back to reference Carlson ML, Breen JT, Driscoll CL, Link MJ, Neff BA, Gifford RH et al (2012) Cochlear implantation in patients with Neurofibromatosis type 2: variables affecting auditory performance. Otol Neurotol 33:853–862CrossRef Carlson ML, Breen JT, Driscoll CL, Link MJ, Neff BA, Gifford RH et al (2012) Cochlear implantation in patients with Neurofibromatosis type 2: variables affecting auditory performance. Otol Neurotol 33:853–862CrossRef
12.
go back to reference Lambert PR, Ruth RA, Thomas JF (1992) Promontory electrical stimulation in postoperative acoustic tumour patients. Laryngoscope 102(7):814–819CrossRef Lambert PR, Ruth RA, Thomas JF (1992) Promontory electrical stimulation in postoperative acoustic tumour patients. Laryngoscope 102(7):814–819CrossRef
13.
go back to reference Cueva RA, Thedinger BA, Harris JP, Glasscock ME (1992) Electrical promontory stimulation in patients with intact cochlear nerve and anacusis following acoustic neuroma surgery. Laryngoscope 102(11):1220–1224CrossRef Cueva RA, Thedinger BA, Harris JP, Glasscock ME (1992) Electrical promontory stimulation in patients with intact cochlear nerve and anacusis following acoustic neuroma surgery. Laryngoscope 102(11):1220–1224CrossRef
14.
go back to reference Neff BA, Wiet RM, Lasak JM et al (2007) Cochlear implantation in the neurofibromatosis type 2 patient: long-term follow-up. Laryngoscope 117(6):1069–1072CrossRef Neff BA, Wiet RM, Lasak JM et al (2007) Cochlear implantation in the neurofibromatosis type 2 patient: long-term follow-up. Laryngoscope 117(6):1069–1072CrossRef
15.
go back to reference Vincenti V, Pasanisi E, Guida M, Di Trapani G, Sanna M (2008) Hearing rehabilitation in neurofibromatosis type 2 patients: cochlear versus auditory brainstem implantation. Audiol Neurotol 13(4):273–280CrossRef Vincenti V, Pasanisi E, Guida M, Di Trapani G, Sanna M (2008) Hearing rehabilitation in neurofibromatosis type 2 patients: cochlear versus auditory brainstem implantation. Audiol Neurotol 13(4):273–280CrossRef
16.
go back to reference Kida Y, Kobayashi T, Tanaka T, Mori Y (2000) Radiosurgery for bilateral neurinomas associated with neurofibromatosis type 2. Surg Neurol 53:383CrossRef Kida Y, Kobayashi T, Tanaka T, Mori Y (2000) Radiosurgery for bilateral neurinomas associated with neurofibromatosis type 2. Surg Neurol 53:383CrossRef
17.
go back to reference Mathieu D, Kondziolka D, Flickinger JC et al (2007) Stereotactic radiosurgery for vestibular schwannomas in patients with neurofibromatosis type 2: an analysis of tumor control, complications, and hearing preservation rates. Neurosurgery 60:460CrossRef Mathieu D, Kondziolka D, Flickinger JC et al (2007) Stereotactic radiosurgery for vestibular schwannomas in patients with neurofibromatosis type 2: an analysis of tumor control, complications, and hearing preservation rates. Neurosurgery 60:460CrossRef
18.
go back to reference Samii M, Matthies C, Tatagiba M (1997) Management of vestibular schwannomas (acoustic neuromas): auditory and facial nerve function after resection of 120 vestibular schwannomas in patients with neurofibromatosis 2. Neurosurgery 40:696–706CrossRef Samii M, Matthies C, Tatagiba M (1997) Management of vestibular schwannomas (acoustic neuromas): auditory and facial nerve function after resection of 120 vestibular schwannomas in patients with neurofibromatosis 2. Neurosurgery 40:696–706CrossRef
19.
go back to reference Balasubramaniam A, Shannon P, Hodaie M et al (2007) Glioblastoma multiforme after stereotactic radiotherapy for acoustic neuroma: case report and review of the literature. Neuro Oncol 9:447CrossRef Balasubramaniam A, Shannon P, Hodaie M et al (2007) Glioblastoma multiforme after stereotactic radiotherapy for acoustic neuroma: case report and review of the literature. Neuro Oncol 9:447CrossRef
20.
go back to reference Wackym PA, Runge-Samuelson CL, Nash JJ et al (2010) Gamma knife surgery of vestibular schwannomas: volumetric dosimetry correlations to hearing loss suggest stria vascularis devascularization as the mechanism of early hearing loss. Otol Neurotol 31(9):1480–1487CrossRef Wackym PA, Runge-Samuelson CL, Nash JJ et al (2010) Gamma knife surgery of vestibular schwannomas: volumetric dosimetry correlations to hearing loss suggest stria vascularis devascularization as the mechanism of early hearing loss. Otol Neurotol 31(9):1480–1487CrossRef
21.
go back to reference Crane BT, Gottschalk B, Kraut M, Aygun N, Niparko JK (2010) Magnetic resonance imaging at 1.5 T after cochlear implantation. Otol Neurotol 31(8):1215–1220CrossRef Crane BT, Gottschalk B, Kraut M, Aygun N, Niparko JK (2010) Magnetic resonance imaging at 1.5 T after cochlear implantation. Otol Neurotol 31(8):1215–1220CrossRef
22.
go back to reference Gubbels SP, McMenomey SO (2006) Safety study of the cochlear nucleus #24 device with internal magnet in the 1.5 Tesla magnetic resonance imaging scanner. Laryngoscope 116(6):865–871CrossRef Gubbels SP, McMenomey SO (2006) Safety study of the cochlear nucleus #24 device with internal magnet in the 1.5 Tesla magnetic resonance imaging scanner. Laryngoscope 116(6):865–871CrossRef
23.
go back to reference Baumann R, Lesinski-Schiedat A, Goldring JE et al (1999) The influence of ionizing radiation on the CLARION 1.2 cochlear implant during radiation therapy. Am J Otol 20(1):50–52PubMed Baumann R, Lesinski-Schiedat A, Goldring JE et al (1999) The influence of ionizing radiation on the CLARION 1.2 cochlear implant during radiation therapy. Am J Otol 20(1):50–52PubMed
24.
go back to reference Ralston A, Stevens G, Mahomudally E, Ibrahim I, Leckie E (1999) Cochlear implants: response to therapeutic irradiation. Int J Radiat Oncol Biol Phys 44(1):227–231CrossRef Ralston A, Stevens G, Mahomudally E, Ibrahim I, Leckie E (1999) Cochlear implants: response to therapeutic irradiation. Int J Radiat Oncol Biol Phys 44(1):227–231CrossRef
25.
go back to reference Klenzner T, Knapp F, Rohner F et al (2005) Influence of ionizing radiation on nucleus 24 cochlear implants. Otol Neurotol 26(4):661–667CrossRef Klenzner T, Knapp F, Rohner F et al (2005) Influence of ionizing radiation on nucleus 24 cochlear implants. Otol Neurotol 26(4):661–667CrossRef
Metadata
Title
Neurofibromatosis 2: Primary Modality of Hearing Rehabilitation with Cochlear Implant
Authors
Dathathri Anantaramaiah Halyur
Praveen H. Rayanagoudar
Apurva Kumar
Sunil Narayan Dutt
Publication date
01-09-2021

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