Audiometric Outcomes of Auditory Brainstem Implantation during Vestibular Schwannoma Resection in NF2 Patients

Abstract

Background Many patients with neurofibromatosis type 2 (NF2) suffer from sensorineural hearing loss, and associated cochlear nerve compromise in NF2 patients makes auditory brainstem implant (ABI) an attractive treatment option. The long-term outcomes and benefits of the device are still being explored.

Methods A retrospective review was conducted for 11 ABI recipients at a single-institution tertiary center between November 2017 and August 2022. Patients diagnosed with NF2 undergoing resection for concurrent vestibular schwannoma (VS) were included. Pre- and postaudiometric assessments were reviewed. Evaluation included pure-tone audiometry and speech testing.

Results Our cohort included 11 patients with a median age of 34 years. All patients underwent a translabyrinthine approach for implant placement with concurrent VS resection. Average tumor size of VS was 2.87 cm. Preoperatively, 8 patients had pure-tone averages with no response at 110 dB, 2 were within mild–moderate hearing loss (25–45 dB), and one patient had a PTA of profound loss (92 dB). Postoperatively, 9 (81%) patients had improvement in PTA. In total, seven patients reported mild side effects upon ABI activation which included dizziness (n = 2), tinnitus (n = 1), and abdominal and lower extremity tingling sensation (n = 3). Of the seven patients with early speech perception (ESP) scores, five had a score >75%, indicating the auditory ability to detect pattern perception upon auditory stimulation through the ABI alone.

Conclusion Nine of 11 patients derived benefits from ABI placement. These findings demonstrate that ABI placement during concurrent VS resection can provide a significant hearing benefit for NF2 patients.

Keywords

auditory brainstem implant - vestibular schwannoma - NF2 - neurofibramatosis 2 - audiometric outcomes

Publication History

Received: 06 October 2023

Accepted: 21 December 2023

Accepted Manuscript online:
28 December 2023

Article published online:
29 January 2024

• References

• 1 Scoles DR, Huynh DP, Morcos PA. et al. Neurofibromatosis 2 tumour suppressor schwannomin interacts with betaII-spectrin. Nat Genet 1998; 18 (04) 354-359
• 2 Bachir S, Shah S, Shapiro S. et al. Neurofibromatosis type 2 (NF2) and the implications for vestibular schwannoma and meningioma pathogenesis. Int J Mol Sci 2021; 22 (02) 690
• 3 Marinelli JP, Beeler CJ, Carlson ML, Caye-Thomasen P, Spear SA, Erbele ID. Global incidence of sporadic vestibular schwannoma: a systematic review. Otolaryngol Head Neck Surg 2022; 167 (02) 209-214
• 4 Barber SR, Kozin ED, Remenschneider AK. et al. Auditory brainstem implant array position varies widely among adult and pediatric patients and is associated with perception. Ear Hear 2017; 38 (06) e343-e351
• 5 Peyre M, Bernardeschi D, Sterkers O, Kalamarides M. Natural history of vestibular schwannomas and hearing loss in NF2 patients. Neurochirurgie 2018; 64 (05) 342-347
• 6 Arriaga MA, Marks S. Simultaneous cochlear implantation and acoustic neuroma resection: imaging considerations, technique, and functional outcome. Otolaryngol Head Neck Surg 1995; 112 (02) 325-328
• 7 Behr R, Colletti V, Matthies C. et al. New outcomes with auditory brainstem implants in NF2 patients. Otol Neurotol 2014; 35 (10) 1844-1851
• 8 Shetty KR, Ridge SE, Kanumuri V, Zhu A, Brown MC, Lee DJ. Clinical and scientific innovations in auditory brainstem implants. World J Otorhinolaryngol Head Neck Surg 2021; 7 (02) 109-115
• 9 Otto SR, Brackmann DE, Hitselberger WE, Shannon RV, Kuchta J. Multichannel auditory brainstem implant: update on performance in 61 patients. J Neurosurg 2002; 96 (06) 1063-1071
• 10 Sanna M, Di Lella F, Guida M, Merkus P. Auditory brainstem implants in NF2 patients: results and review of the literature. Otol Neurotol 2012; 33 (02) 154-164
• 11 Wong K, Kozin ED, Kanumuri VV. et al. Auditory brainstem implants: recent progress and future perspectives. Front Neurosci 2019; 13: 10
• 12 Anwar A, Singleton A, Fang Y. et al. The value of intraoperative EABRs in auditory brainstem implantation. Int J Pediatr Otorhinolaryngol 2017; 101: 158-163
• 13 Peng KA, Lorenz MB, Otto SR, Brackmann DE, Wilkinson EP. Cochlear implantation and auditory brainstem implantation in neurofibromatosis type 2. Laryngoscope 2018; 128 (09) 2163-2169
• 14 Sorrentino F, Tealdo G, Cazzador D. et al. Cochlear implant in vestibular schwannomas: long-term outcomes and critical analysis of indications. Eur Arch Otorhinolaryngol 2022; 279 (10) 4709-4718
• 15 Schwartz MS, Otto SR, Brackmann DE, Hitselberger WE, Shannon RV. Use of a multichannel auditory brainstem implant for neurofibromatosis type 2. Stereotact Funct Neurosurg 2003; 81 (1-4): 110-114
• 16 Schierholz I, Finke M, Kral A. et al. Auditory and audio-visual processing in patients with cochlear, auditory brainstem, and auditory midbrain implants: An EEG study. Hum Brain Mapp 2017; 38 (04) 2206-2225
• 17 Song JJ, Lee HJ, Kang H, Lee DS, Chang SO, Oh SH. Effects of congruent and incongruent visual cues on speech perception and brain activity in cochlear implant users. Brain Struct Funct 2015; 220 (02) 1109-1125
• 18 Colletti L, Shannon R, Colletti V. Auditory brainstem implants for neurofibromatosis type 2. Curr Opin Otolaryngol Head Neck Surg 2012; 20 (05) 353-357
• 19 Sennaroglu L, Ziyal I. Auditory brainstem implantation. Auris Nasus Larynx 2012; 39 (05) 439-450
• 20 Schwartz MS, Otto SR, Shannon RV, Hitselberger WE, Brackmann DE. Auditory brainstem implants. Neurotherapeutics 2008; 5 (01) 128-136
• 21 Grayeli AB, Bouccara D, Kalamarides M. et al. Auditory brainstem implant in bilateral and completely ossified cochleae. Otol Neurotol 2003; 24 (01) 79-82
• 22 Grayeli AB, Kalamarides M, Bouccara D, Ben Gamra L, Ambert-Dahan E, Sterkers O. Auditory brainstem implantation to rehabilitate profound hearing loss with totally ossified cochleae induced by pneumococcal meningitis. Audiol Neurotol 2007; 12 (01) 27-30
• 23 Colletti V, Carner M, Fiorino F. et al. Hearing restoration with auditory brainstem implant in three children with cochlear nerve aplasia. Otol Neurotol 2002; 23 (05) 682-693
• 24 Colletti V, Fiorino FG, Carner M, Miorelli V, Guida M, Colletti L. Auditory brainstem implant as a salvage treatment after unsuccessful cochlear implantation. Otol Neurotol 2004; 25 (04) 485-496 , discussion 496