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Child's Nervous System
Published in: Child's Nervous System 10/2020

01-10-2020 | Vestibular Schwannoma | Annual issue paper

Options and strategies for hearing restoration in pediatric neurofibromatosis type 2

Authors: Hossein Mahboubi, William H. Slattery III, Gautam U. Mehta, Gregory P. Lekovic

Published in: Child's Nervous System | Issue 10/2020

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Abstract

Purpose

In this article, we will review the mechanisms and natural history of hearing loss in neurofibromatosis type 2 (NF2) and discuss the hearing outcomes with different rehabilitation options.

Methods

Review of the published literature.

Results

NF2 is a rare autosomal dominant syndrome characterized by vestibular schwannomas and other intracranial and spinal tumors. Bilateral vestibular schwannomas are the hallmark of the disease which occur in 90 to 95% of the patients. As a result, hearing loss will eventually occur in almost all NF2 patients. Deafness can occur from tumor progression or from treatment of vestibular schwannomas and is among the most debilitating aspects of NF2. A number of surgical and non-surgical rehabilitation options are available for these patients including cochlear and auditory brainstem implants. The audiologic outcomes with surgical rehabilitation options have been variable but most patients are able to achieve sound awareness and benefit from auditory cues in lip reading.

Conclusion

Early identification and treatment of NF2 patients can help in achieving better hearing outcomes in the pediatric population. An increasing number of NF2 patients are receiving open set word understanding with refinement in surgical techniques.
Literature
1.
Plotkin SR, Merker VL, Muzikansky A, Barker FG 2nd, Slattery W 3rd. (2014) Natural history of vestibular schwannoma growth and hearing decline in newly diagnosed neurofibromatosis type 2 patients. Otol Neurotol 35(1):e50–e56PubMed
2.
Evans DG, Moran A, King A, Saeed S, Gurusinghe N, Ramsden R (2005) Incidence of vestibular schwannoma and neurofibromatosis 2 in the North West of England over a 10-year period: higher incidence than previously thought. Otol Neurotol 26(1):93–97PubMed
3.
Rouleau GA, Wertelecki W, Haines JL, Hobbs WJ, Trofatter JA, Seizinger BR, Martuza RL, Superneau DW, Conneally PM, Gusella JF (1987) Genetic linkage of bilateral acoustic neurofibromatosis to a DNA marker on chromosome 22. Nature. 329(6136):246–248PubMed
4.
Slattery WH (2015) Neurofibromatosis type 2. Otolaryngol Clin N Am 48(3):443–460
5.
6.
Gutmann DH, Aylsworth A, Carey JC, Korf B, Marks J, Pyeritz RE, Rubenstein A, Viskochil D (1997) The diagnostic evaluation and multidisciplinary management of neurofibromatosis 1 and neurofibromatosis 2. Jama. 278(1):51–57PubMed
7.
Baser ME, Friedman JM, Joe H, Shenton A, Wallace AJ, Ramsden RT, Evans DGR (2011) Empirical development of improved diagnostic criteria for neurofibromatosis 2. Genet Med 13(6):576–581PubMed
8.
Asthagiri AR, Parry DM, Butman JA, Kim HJ, Tsilou ET, Zhuang Z, Lonser RR (2009) Neurofibromatosis type 2. Lancet. 373(9679):1974–1986PubMedPubMedCentral
9.
Ruggieri M (1999) The different forms of neurofibromatosis. Childs Nerv Syst 15(6–7):295–308PubMed
10.
Mautner VF, Tatagiba M, Guthoff R, Samii M, Pulst SM (1993) Neurofibromatosis 2 in the pediatric age group. Neurosurgery. 33(1):92–96PubMed
11.
Ruggieri M, Pratico AD, Serra A et al (2016) Childhood neurofibromatosis type 2 (NF2) and related disorders: from bench to bedside and biologically targeted therapies. Acta Otorhinolaryngol Ital 36(5):345–367PubMedPubMedCentral
12.
Ruggieri M, Gabriele AL, Polizzi A, Salpietro V, Nicita F, Pavone P, Platania N, Milone P, Distefano A, Privitera G, Belfiore G, Granata F, Caltabiano R, Albanese V, Pavone L, Quattrone A (2013) Natural history of neurofibromatosis type 2 with onset before the age of 1 year. Neurogenetics. 14(2):89–98PubMed
13.
Otsuka G, Saito K, Nagatani T, Yoshida J (2003) Age at symptom onset and long-term survival in patients with neurofibromatosis type 2. J Neurosurg 99(3):480–483PubMed
14.
Baser ME, Friedman JM, Aeschliman D, Joe H, Wallace AJ, Ramsden RT, Evans DGR (2002) Predictors of the risk of mortality in neurofibromatosis 2. Am J Hum Genet 71(4):715–723PubMedPubMedCentral
15.
Mahboubi H, Maducdoc MM, Yau AY, Ziai K, Ghavami Y, Badran KW, al-Thobaiti M, Brandon B, Djalilian HR (2015) Vestibular Schwannoma excision in sporadic versus neurofibromatosis type 2 populations. Otolaryngol Head Neck Surg 153(5):822–831PubMed
16.
Committee on Hearing and Equilibrium guidelines for the evaluation of hearing preservation in acoustic neuroma (vestibular schwannoma). American Academy of Otolaryngology-Head and Neck Surgery Foundation, INC. Otolaryngol Head Neck Surg. 1995;113(3):179–180
17.
Strasnick B, Glasscock ME 3rd, Haynes D, McMenomey SO, Minor LB (1994) The natural history of untreated acoustic neuromas. Laryngoscope. 104(9):1115–1119PubMed
18.
Evans DG, Huson SM, Donnai D, Neary W, Blair V, Newton V, Harris R (1992) A clinical study of type 2 neurofibromatosis. Q J Med 84(304):603–618PubMed
19.
Lee JM, Chang JW, Choi JY, Chang WS, Moon IS (2016) Hearing restoration in neurofibromatosis type II patients. Yonsei Med J 57(4):817–823PubMedPubMedCentral
20.
Roosli C, Linthicum FH Jr, Cureoglu S, Merchant SN (2012) Dysfunction of the cochlea contributing to hearing loss in acoustic neuromas: an underappreciated entity. Otol Neurotol 33(3):473–480PubMedPubMedCentral
21.
Asthagiri AR, Vasquez RA, Butman JA, Wu T, Morgan K, Brewer CC, King K, Zalewski C, Kim HJ, Lonser RR (2012) Mechanisms of hearing loss in neurofibromatosis type 2. PLoS One 7(9):e46132PubMedPubMedCentral
22.
Silverstein H (1973) Labyrinthine tap as a diagnostic test for acoustic neurinoma. Otolaryngol Clin N Am 6(1):229–244
23.
Hoa M, Slattery WH 3rd. (2012) Neurofibromatosis 2. Otolaryngol Clin N Am 45(2):315–332 viii
24.
Masuda A, Fisher LM, Oppenheimer ML, Iqbal Z, Slattery WH (2004) Hearing changes after diagnosis in neurofibromatosis type 2. Otol Neurotol 25(2):150–154PubMed
25.
Slattery WH 3rd, Fisher LM, Hitselberger W, Friedman RA, Brackmann DE (2007) Hearing preservation surgery for neurofibromatosis type 2-related vestibular schwannoma in pediatric patients. J Neurosurg 106(4 Suppl):255–260PubMed
26.
Slattery WH 3rd, Brackmann DE, Hitselberger W (1998) Hearing preservation in neurofibromatosis type 2. Am J Otolaryngol 19(5):638–643
27.
Doyle KJ, Shelton C (1993) Hearing preservation in bilateral acoustic neuroma surgery. Am J Otolaryngol 14(6):562–565
28.
Friedman RA, Goddard JC, Wilkinson EP, Schwartz MS, Slattery WH III, Fayad JN, Brackmann DE (2011) Hearing preservation with the middle cranial fossa approach for neurofibromatosis type 2. Otol Neurotol 32(9):1530–1537PubMed
29.
Gugel I, Grimm F, Liebsch M, Zipfel J, Teuber C, Kluwe L, Mautner VF, Tatagiba M, Schuhmann MU (2019) Impact of surgery on long-term results of hearing in neurofibromatosis type-2 associated vestibular schwannomas. Cancers (Basel) 11(9):1376
30.
Slattery WH, Hoa M, Bonne N, Friedman RA, Schwartz MS, Fisher LM, Brackmann DE (2011) Middle fossa decompression for hearing preservation: a review of institutional results and indications. Otol Neurotol 32(6):1017–1024PubMed
31.
Mathieu D, Kondziolka D, Flickinger JC, Niranjan A, Williamson R, Martin JJ, Lunsford LD (2007) Stereotactic radiosurgery for vestibular schwannomas in patients with neurofibromatosis type 2: an analysis of tumor control, complications, and hearing preservation rates. Neurosurgery. 60(3):460–468 discussion 468-470PubMed
32.
Jansson KJ, Hakansson B, Reinfeldt S, Rigato C, Eeg-Olofsson M (2015) Magnetic resonance imaging investigation of the bone conduction implant - a pilot study at 1.5 Tesla. Med Devices (Auckl) 8:413–423
33.
Drusin MA, Lubor B, Losenegger T, Selesnick S (2019) Trends in hearing rehabilitation use among vestibular schwannoma patients. Laryngoscope
34.
Hitselberger WE, House WF, Edgerton BJ, Whitaker S (1984) Cochlear nucleus implants. Otolaryngol Head Neck Surg 92(1):52–54PubMed
35.
Otto SR, Shannon RV, Wilkinson EP, Hitselberger WE, McCreery DB, Moore JK, Brackmann DE (2008) Audiologic outcomes with the penetrating electrode auditory brainstem implant. Otol Neurotol 29(8):1147–1154PubMed
36.
Choi JY, Song MH, Jeon JH, Lee WS, Chang JW (2011) Early surgical results of auditory brainstem implantation in nontumor patients. Laryngoscope. 121(12):2610–2618PubMed
37.
Colletti V, Shannon RV (2005) Open set speech perception with auditory brainstem implant? Laryngoscope. 115(11):1974–1978PubMed
38.
Ebinger K, Otto S, Arcaroli J, Staller S, Arndt P (2000) Multichannel auditory brainstem implant: US clinical trial results. J Laryngol Otol Suppl 27:50–53
39.
Otto SR, Brackmann DE, Hitselberger WE, Shannon RV, Kuchta J (2002) Multichannel auditory brainstem implant: update on performance in 61 patients. J Neurosurg 96(6):1063–1071PubMed
40.
Schwartz MS, Wilkinson EP (2017) Auditory brainstem implant program development. Laryngoscope. 127(8):1909–1915PubMed
41.
Otto SR, Brackmann DE, Hitselberger W (2004) Auditory brainstem implantation in 12- to 18-year-olds. Arch Otolaryngol Head Neck Surg 130(5):656–659PubMed
42.
Colletti L, Shannon R, Colletti V (2012) Auditory brainstem implants for neurofibromatosis type 2. Curr Opin Otolaryngol Head Neck Surg 20(5):353–357PubMed
43.
Harris F, Tysome JR, Donnelly N, Durie-Gair J, Crundwell G, Tam YC, Knight RD, Vanat ZH, Folland N, Axon P (2017) Cochlear implants in the management of hearing loss in neurofibromatosis type 2. Cochlear Implants Int 18(3):171–179PubMed
44.
Peng KA, Lorenz MB, Otto SR, Brackmann DE, Wilkinson EP (2018) Cochlear implantation and auditory brainstem implantation in neurofibromatosis type 2. Laryngoscope. 128(9):2163–2169PubMed
45.
Charlett SD, Biggs N (2015) The prevalence of cochlear obliteration after labyrinthectomy using magnetic resonance imaging and the implications for cochlear implantation. Otol Neurotol 36(8):1328–1330PubMed
46.
Lim HH, Lenarz T (2015) Auditory midbrain implant: research and development towards a second clinical trial. Hear Res 322:212–223PubMedPubMedCentral
Metadata
Title
Options and strategies for hearing restoration in pediatric neurofibromatosis type 2
Authors
Hossein Mahboubi
William H. Slattery III
Gautam U. Mehta
Gregory P. Lekovic
Publication date
01-10-2020
Publisher
Springer Berlin Heidelberg
Published in
Child's Nervous System / Issue 10/2020
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI
https://doi.org/10.1007/s00381-020-04721-4

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