European Archives of Oto-Rhino-Laryngology

, Volume 272, Issue 11, pp 3143–3150 | Cite as

Progress of hearing loss in neurofibromatosis type 2: implications for future management

  • Georgios KontorinisEmail author
  • Jaya Nichani
  • Simon R. Freeman
  • Scott A. Rutherford
  • Samantha Mills
  • Andrew T. King
  • Deborah Mawman
  • Sue Huson
  • Martin O’Driscoll
  • D. Gareth Evans
  • Simon K. W. Lloyd


The objective of this study was to describe changes in hearing over time in patients with neurofibromatosis type 2 (NF2) treated conservatively. A retrospective case review was conducted in a tertiary referral centre. Pure tone audiometry, speech discrimination scores, serviceable hearing (American Academy of Otolaryngology class A or B) and measurement of vestibular schwannoma (VS) size on magnetic resonance imaging were evaluated in 56 patients (89 ears) with NF2 with at least one conservatively managed VS. Over a mean follow-up period of 7 years (range 0.8–21 years) pure tone average thresholds increased gradually with a mean annual rate of 1.3 dB for the right ear (p = 0.0003) and 2 dB for the left ear (p =  0.0009). Speech discrimination scores dropped with an average annual rate of 1.3 and 0.34 % in the right and left ear, respectively. Patients maintained serviceable hearing for an average of 7.6 years (range 2.7–19.3 years). The average annual VS growth was 0.4 mm without any correlation with hearing loss. There was a correlation between patients’ age and pure tone threshold increase (p < 0.05 for both ears). In this selected population of patients with NF2, hearing threshold increases were very slow. In NF2 patients with indolently behaving tumours, serviceable hearing can be maintained for a significant length of time, making conservative management an attractive option.


Conservative treatment Deafness Neurofibromatosis type 2 Tumour size Vestibular schwannoma Watch wait rescan protocol 


Conflict of interest

None to declare.


  1. 1.
    Evans GR, Lloyd S, Ramsden RT (2011) Neurofibromatosis type 2. Adv Otorhinolaryngol 70:91–98PubMedGoogle Scholar
  2. 2.
    Evans DG (2009) Neurofibromatosis type 2 (NF2): a clinical and molecular review. Orphanet J Rare Dis 4:16–27PubMedCentralCrossRefPubMedGoogle Scholar
  3. 3.
    National Institute of Health Consensus Development Conference (1991) Statement on acoustic neuroma. 9:1–24Google Scholar
  4. 4.
    Evans DGR, Huson SM, Donnai D et al (1992) A genetic study of type 2 neurofibromatosis: II. Guidelines for genetic counseling. J Med Genet 29:847–852PubMedCentralCrossRefPubMedGoogle Scholar
  5. 5.
    Baser ME, Friedman JM, Joe H et al (2011) Empirical development of improved diagnostic criteria for neurofibromatosis 2. Genet Med 13:576–581CrossRefPubMedGoogle Scholar
  6. 6.
    Lalwani AK, Abaza MM, Makariou EV, Armstrong M (1998) Audiologic presentation of vestibular schwannomas in neurofibromatosis type 2. Am J Otol 19:352–357PubMedGoogle Scholar
  7. 7.
    Blakeley JO, Evans DG, Adler J et al (2012) Consensus recommendations for current treatments and accelerating clinical trials for patients with neurofibromatosis type 2. Am J Med Genet 158:24–41CrossRefGoogle Scholar
  8. 8.
    Lloyd SK, Kasbekar A, Baguley DM, Moffat DA (2010) Audio-vestibular factors influencing quality of life in patients undergoing conservative management of sporadic vestibular schwannoma. Otol Neurotol 31:968–976CrossRefPubMedGoogle Scholar
  9. 9.
    Masuda A, Fisher LM, Oppenheimer ML, Iqbal Z, Slattery WH (2004) Natural history consortium. Hearing changes after diagnosis in neurofibromatosis type 2. Otol Neurotol 25:150–154CrossRefPubMedGoogle Scholar
  10. 10.
    Massick DD, Welling DB, Dodson EE et al (2000) Tumor growth and audiometric change in vestibular schwannomas managed conservatively. Laryngoscope 110:1843–1849CrossRefPubMedGoogle Scholar
  11. 11.
    Rosenberg SI (2000) Natural history of acoustic neuromas. Laryngoscope 110:497–508CrossRefPubMedGoogle Scholar
  12. 12.
    Abaza MM, Makariou EM, Armstrong M et al (1996) Growth rate characteristics of acoustic neuromas associated with neurofibromatosis type 2. Laryngoscope 106:694–699CrossRefPubMedGoogle Scholar
  13. 13.
    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 40:696–705CrossRefPubMedGoogle Scholar
  14. 14.
    Pikus AT (1995) Pediatric audiologic profile in type 1 and type 2 neurofibromatosis. J Am Acad Audiol 6:54–62PubMedGoogle Scholar
  15. 15.
    Tierney PA, Chitnavis BP, Sherriff M, Strong AJ, Gleeson MJ (1998) The relationship between pure tone thresholds and the radiological dimensions of acoustic neuromas. Skull Base Surg 8:149–151PubMedCentralCrossRefPubMedGoogle Scholar
  16. 16.
    Warrick P, Bance M, Rutka J (1999) The risk of hearing loss in nongrowing, conservatively managed acoustic neuromas. Am J Otol 20:758–762PubMedGoogle Scholar
  17. 17.
    Yoshimoto Y (2005) Systematic review of the natural history of vestibular schwannoma. J Neurosurg 103:59–63CrossRefPubMedGoogle Scholar
  18. 18.
    Gutman DH, Aylsworth A, Carey JC et al (1997) The diagnostic evaluation and multidisciplinary management of neurofibromatosis 1 and neurofibromatosis 2. JAMA 278:51–57CrossRefGoogle Scholar
  19. 19.
    Committee on hearing and equilibrium guidelines for the evaluation of hearing preservation in acoustic neuroma (vestibular schwannoma) (1995) American academy of otolaryngology-head and neck surgery foundation, INC. Otolaryngol Head Neck Surg 113:179–180CrossRefGoogle Scholar
  20. 20.
    Sughrue ME, Kane AJ, Kaur R et al (2011) A prospective study of hearing preservation in untreated vestibular schwannomas. J Neurosurg 114:381–385CrossRefPubMedGoogle Scholar
  21. 21.
    Walsh RM, Bath AP, Bance ML, Keller A, Rutka JA (2000) Consequences to hearing during the conservative management of vestibular schwannomas. Laryngoscope 110:250–255CrossRefPubMedGoogle Scholar
  22. 22.
    Asthagiri AR, Vasquez RA, Butman JA et al (2012) Mechanisms of hearing loss in neurofibromatosis type 2. PLoS One 7:e46132PubMedCentralCrossRefPubMedGoogle Scholar
  23. 23.
    Peyre M, Goutagny S, Bah A et al (2013) Conservative management of bilateral vestibular schwannomas in neurofibromatosis type 2 patients: hearing and tumor growth results. Neurosurgery 72:907–913CrossRefPubMedGoogle Scholar
  24. 24.
    Fisher LM, Doherty JK, Lev MH, Slattery WH (2009) Concordance of bilateral vestibular schwannoma growth and hearing changes in neurofibromatosis 2: neurofibromatosis 2 natural history consortium. Otol Neurotol 30:835–841CrossRefPubMedGoogle Scholar
  25. 25.
    Mirz F, Pedersen CB, Fiirgaard B, Lundorf E (2000) Incidence and growth pattern of vestibular schwannomas in a Danish county, 1977–98. Acta Otolaryngol 543(Suppl):30–33Google Scholar
  26. 26.
    Stewart TJ, Liland J, Schuknecht HF (1975) Occult schwannomas of the vestibular nerve. Arch Otolaryngol 101:91–95CrossRefPubMedGoogle Scholar
  27. 27.
    Plotkin SR, Merker VL, Halpin C et al (2012) Bevacizumab for progressive vestibular schwannoma in neurofibromatosis type 2: a retrospective review of 31 patients. Otol Neurotol 33:1046–1052CrossRefPubMedGoogle Scholar
  28. 28.
    Phi JH, Kim DG, Chung HT, Lee J, Paek SH, Jung HW (2009) Radiosurgical treatment of vestibular schwannomas in patients with neurofibromatosis type 2: tumor control and hearing preservation. Cancer 115:390–398CrossRefPubMedGoogle Scholar
  29. 29.
    Rowe J, Radatz M, Kemeny A (2008) Radiosurgery for type II neurofibromatosis. Prog Neurol Surg 21:176–182CrossRefPubMedGoogle Scholar
  30. 30.
    Mallory GW, Pollock BE, Foote RL et al (2014) Stereotactic radiosurgery for neurofibromatosis 2-associated vestibular schwannomas: toward dose optimization for tumor control and functional outcomes. Neurosurgery 74:292–300CrossRefPubMedGoogle Scholar
  31. 31.
    Friedman RA, Goddard JC, Wilkinson EP et al (2011) Hearing preservation with the middle cranial fossa approach for neurofibromatosis type 2. Otol Neurotol 32:1530–1537CrossRefPubMedGoogle Scholar
  32. 32.
    Samii M, Gerganov V, Samii A (2008) Microsurgery management of vestibular schwannomas in neurofibromatosis type 2: indications and results. Prog Neurol Surg 21:169–175CrossRefPubMedGoogle Scholar
  33. 33.
    Lloyd SK, Glynn FJ, Rutherford SA et al (2014) Ipsilateral cochlear implantation after cochlear nerve preserving vestibular schwannoma surgery in patients with neurofibromatosis type 2. Otol Neurotol 35:43–51CrossRefPubMedGoogle Scholar
  34. 34.
    Mukherjee P, Ramsden JD, Donnelly N et al (2013) Cochlear implants to treat deafness caused by vestibular schwannomas. Otol Neurotol 34:1291–1298CrossRefPubMedGoogle Scholar
  35. 35.
    Pai I, Dhar V, Kelleher C et al (2013) Cochlear implantation in patients with vestibular schwannoma: a single United Kingdom center experience. Laryngoscope 123:2019–2023CrossRefPubMedGoogle Scholar
  36. 36.
    Colletti V, Fiorino F, Carner M, Sacchetto L, Miorelli V, Orsi A (2002) Auditory brainstem implantation: the University of Verona experience. Otolaryngol Head Neck Surg 127:84–96CrossRefPubMedGoogle Scholar
  37. 37.
    Sanna M, Di Lella F, Guida M, Merkus P (2012) Auditory brainstem implants in NF2 patients: results and review of the literature. Otol Neurotol 33:154–164CrossRefPubMedGoogle Scholar
  38. 38.
    Merkus P, Di Lella F, Di Trapani G et al (2014) Indications and contraindications of auditory brainstem implants: systematic review and illustrative cases. Eur Arch Otorhinolaryngol 271:3–13CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2014

Authors and Affiliations

  • Georgios Kontorinis
    • 1
    • 6
    Email author
  • Jaya Nichani
    • 1
  • Simon R. Freeman
    • 1
  • Scott A. Rutherford
    • 2
  • Samantha Mills
    • 3
  • Andrew T. King
    • 2
  • Deborah Mawman
    • 1
  • Sue Huson
    • 4
  • Martin O’Driscoll
    • 1
  • D. Gareth Evans
    • 4
  • Simon K. W. Lloyd
    • 1
    • 5
  1. 1.University Department of OtolaryngologyCentral Manchester University Hospitals NHS Foundation TrustManchesterUK
  2. 2.Department of NeurosurgerySalford Royal NHS Foundation TrustManchesterUK
  3. 3.Department of NeuroradiologySalford Royal NHS Foundation TrustManchesterUK
  4. 4.Department of Genetics MedicineCentral Manchester University Hospitals NHS Foundation TrustManchesterUK
  5. 5.School of Cancer and Enabling ServicesUniversity of ManchesterManchesterUK
  6. 6.Department of Otolaryngology, Institute of NeurosciencesSouthern Teaching HospitalGlasgowUK

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