Auditory rehabilitation of patients with neurofibromatosis Type 2 by using cochlear implants

Clinical article

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  • 1 Departments of Otolaryngology and
  • 2 Neurosurgery, New York University School of Medicine, New York, New York
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Object

The aim of this study was to determine whether patients with neurofibromatosis Type 2 (NF2) who have intact ipsilateral cochlear nerves can have open-set speech discrimination following cochlear implantation.

Methods

Records of 7 patients with documented NF2 were reviewed to determine speech discrimination outcomes following cochlear implantation. Outcomes were measured using consonant-nucleus-consonant words and phonemes; Hearing in Noise Test sentences in quiet; and City University of New York sentences in quiet and in noise.

Results

Preoperatively, none of the patients had open-set speech discrimination. Five of the 7 patients had previously undergone excision of ipsilateral vestibular schwannoma (VS). One of the patients who received a cochlear implant had received radiation therapy for ipsilateral VS, and another was undergoing observation for a small ipsilateral VS. Following cochlear implantation, 4 of 7 patients with NF2 had open-set speech discrimination following cochlear implantation during extended follow-up (15–120 months). Two of the 3 patients without open-set speech understanding had a prolonged period between ipsilateral VS resection and cochlear implantation (120 and 132 months), and had cochlear ossification at the time of implantation. The other patient without open-set speech understanding had good contralateral hearing at the time of cochlear implantation. Despite these findings, 6 of the 7 patients were daily users of their cochlear implants, and the seventh is an occasional user, indicating that all of the patients subjectively gained some benefit from their implants.

Conclusions

Cochlear implantation can provide long-term auditory rehabilitation, with open-set speech discrimination for patients with NF2 who have intact ipsilateral cochlear nerves. Factors that can affect implant performance include the following: 1) a prolonged time between VS resection and implantation; and 2) cochlear ossification.

Abbreviations used in this paper: ABI = auditory brainstem implant; CNC = consonant-nucleus-consonant; CNCp, CNCw = CNC phonemes and words; CROS = contralateral routing of sound devices; CUNY = City University of New York; CUNYn, CUNYq = CUNY sentences in noise and in quiet; HINT = Hearing in Noise Test; HINTn, HINTq = HINT performed in noise and in quiet; MCF = middle cranial fossa; NF2 = neurofibromatosis Type 2; RS = retrosigmoid; SDT = speech detection threshold; SNHL = sensorineural hearing loss; SRT = speech reception threshold; VS = vestibular schwannoma.

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Contributor Notes

Address correspondence to: Pamela Roehm, M.D., Ph.D., Department of Otolaryngology, New York University School of Medicine, 530 First Avenue, Suite 7Q, New York, New York 10016. email: pamela.roehm@nyumc.org.

Please include this information when citing this paper: published online July 15, 2011; DOI: 10.3171/2011.5.JNS101929.

  • 1

    Ahsan S, , Telischi F, , Hodges A, & Balkany T: Cochlear implantation concurrent with translabyrinthine acoustic neuroma resection. Laryngoscope 113:472474, 2003

    • Search Google Scholar
    • Export Citation
  • 2

    Arístegui M, & Denia A: Simultaneous cochlear implantation and translabyrinthine removal of vestibular schwannoma in an only hearing ear: report of two cases (neurofibromatosis type 2 and unilateral vestibular schwannoma). Otol Neurotol 26:205210, 2005

    • Search Google Scholar
    • Export Citation
  • 3

    Arriaga MA, & Marks S: Simultaneous cochlear implantation and acoustic neuroma resection: imaging considerations, technique, and functional outcome. Otolaryngol Head Neck Surg 112:325328, 1995

    • Search Google Scholar
    • Export Citation
  • 4

    Asthagiri AR, , Parry DM, , Butman JA, , Kim HJ, , Tsilou ET, & Zhuang Z, : Neurofibromatosis type 2. Lancet 373:19741986, 2009

  • 5

    Colletti V, & Shannon RV: Open set speech perception with auditory brainstem implant?. Laryngoscope 115:19741978, 2005

  • 6

    Colletti V, , Shannon RV, , Carner M, , Veronese S, & Colletti L: Complications in auditory brainstem implant surgery in adults and children. Otol Neurotol 31:558564, 2010

    • Search Google Scholar
    • Export Citation
  • 7

    Denia A, & Arístegui M: Cochlear implantation after translabyrinthine acoustic tumour removal: preliminary results. Cochlear Implants Int 6:Suppl 1 2024, 2005

    • Search Google Scholar
    • Export Citation
  • 8

    Di Nardo W, , Fetoni A, , Buldrini S, & Di Girolamo S: Auditory brainstem and cochlear implants: functional results obtained after one year of rehabilitation. Eur Arch Otorhinolaryngol 258:58, 2001

    • Search Google Scholar
    • Export Citation
  • 9

    El-Kashlan HK, , Ashbaugh C, , Zwolan T, & Telian SA: Cochlear implantation in prelingually deaf children with ossified cochleae. Otol Neurotol 24:596600, 2003

    • Search Google Scholar
    • Export Citation
  • 10

    Evans DG, Neurofibromatosis 2. Pagon RA, , Bird TC, & Dolan CR, : GeneReviews Seattle, University of Washington, Seattle, 1998. (http://www.ncbi.nlm.nih.gov/books/NBK1201/) [Accessed June 3, 2011]

    • Search Google Scholar
    • Export Citation
  • 11

    Evans DG: Neurofibromatosis type 2 (NF2): a clinical and molecular review. Orphanet J Rare Dis 4:16, 2009

  • 12

    Graham J, , Lynch C, , Weber B, , Stollwerck L, , Wei J, & Brookes G: The magnetless Clarion cochlear implant in a patient with neurofibromatosis 2. J Laryngol Otol 113:458463, 1999

    • Search Google Scholar
    • Export Citation
  • 13

    Green JD Jr, , Marion MS, & Hinojosa R: Labyrinthitis ossificans: histopathologic consideration for cochlear implantation. Otolaryngol Head Neck Surg 104:320326, 1991

    • Search Google Scholar
    • Export Citation
  • 14

    Hinojosa R, , Green JD Jr, & Marion MS: Ganglion cell populations in labyrinthitis ossificans. Am J Otol 12:Suppl 37, 1991

  • 15

    Hoffman RA, , Kohan D, & Cohen NL: Cochlear implants in the management of bilateral acoustic neuromas. Am J Otol 13:525528, 1992

  • 16

    Hulka GF, , Bernard EJ, & Pillsbury HC: Cochlear implantation in a patient after removal of an acoustic neuroma. The implications of magnetic resonance imaging with gadolinium on patient management. Arch Otolaryngol Head Neck Surg 121:465468, 1995

    • Search Google Scholar
    • Export Citation
  • 17

    Kanowitz SJ, , Shapiro WH, , Golfinos JG, , Cohen NL, & Roland JT Jr: Auditory brainstem implantation in patients with neurofibromatosis type 2. Laryngoscope 114:21352146, 2004

    • Search Google Scholar
    • Export Citation
  • 18

    Kaylie DM, , Horgan MJ, , Delashaw JB, & McMenomey SO: A meta-analysis comparing outcomes of microsurgery and gamma knife radiosurgery. Laryngoscope 110:18501856, 2000

    • Search Google Scholar
    • Export Citation
  • 19

    Kondziolka D, , Lunsford LD, & Flickinger JC: Acoustic neuromas. Curr Treat Options Neurol 4:157165, 2002

  • 20

    Lustig LR, , Yeagle J, , Driscoll CL, , Blevins N, , Francis H, & Niparko JK: Cochlear implantation in patients with neurofibromatosis type 2 and bilateral vestibular schwannoma. Otol Neurotol 27:512518, 2006

    • Search Google Scholar
    • Export Citation
  • 21

    Neff BA, , Wiet RM, , Lasak JM, , Cohen NL, , Pillsbury HC, & Ramsden RT, : Cochlear implantation in the neurofibromatosis type 2 patient: long-term follow-up. Laryngoscope 117:10691072, 2007

    • Search Google Scholar
    • Export Citation
  • 22

    Nölle C, , Todt I, , Basta D, , Unterberg A, , Mautner VF, & Ernst A: Cochlear implantation after acoustic tumour resection in neurofibromatosis type 2: impact of intra- and postoperative neural response telemetry monitoring. ORL J Otorhinolaryngol Relat Spec 65:230234, 2003

    • Search Google Scholar
    • Export Citation
  • 23

    Piccirillo E, , Guida M, , Flanagan S, , Lauda L, , Fois P, & Sanna M: CNAP to predict functional cochlear nerve preservation in NF-2: cochlear implant or auditory brainstem implant. Skull Base 18:281287, 2008

    • Search Google Scholar
    • Export Citation
  • 24

    Plotkin SR, , Halpin C, , McKenna MJ, , Loeffler JS, , Batchelor TT, & Barker FG II: Erlotinib for progressive vestibular schwannoma in neurofibromatosis 2 patients. Otol Neurotol 31:11351143, 2010

    • Search Google Scholar
    • Export Citation
  • 25

    Plotkin SR, , Singh MA, , O'Donnell CC, , Harris GJ, , McClatchey AI, & Halpin C: Audiologic and radiographic response of NF2-related vestibular schwannoma to erlotinib therapy. Nat Clin Pract Oncol 5:487491, 2008

    • Search Google Scholar
    • Export Citation
  • 26

    Rauch SD, , Herrmann BS, , Davis LA, & Nadol JB Jr: Nucleus 22 cochlear implantation results in postmeningitic deafness. Laryngoscope 107:16061609, 1997

    • Search Google Scholar
    • Export Citation
  • 27

    Rouleau GA, , Merel P, , Lutchman M, , Sanson M, , Zucman J, & Marineau C, : Alteration in a new gene encoding a putative membrane-organizing protein causes neuro-fibromatosis type 2. Nature 363:515521, 1993

    • Search Google Scholar
    • Export Citation
  • 28

    Schwartz MS, , Otto SR, , Shannon RV, , Hitselberger WE, & Brackmann DE: Auditory brainstem implants. Neurotherapeutics 5:128136, 2008

  • 29

    Temple RH, , Axon PR, , Ramsden RT, , Keles N, , Deger K, & Yücel E: Auditory rehabilitation in neurofibromatosis type 2: a case for cochlear implantation. J Laryngol Otol 113:161163, 1999

    • Search Google Scholar
    • Export Citation
  • 30

    Tono T, , Ushisako Y, & Morimitsu T: Cochlear implantation in an intralabyrinthine acoustic neuroma patient after resection of an intracanalicular tumour. J Laryngol Otol 110:570573, 1996

    • Search Google Scholar
    • Export Citation
  • 31

    Tran Ba Huy P, , Kania R, , Frachet B, , Poncet C, & Legac MS: Auditory rehabilitation with cochlear implantation in patients with neurofibromatosis type 2. Acta Otolaryngol 129:971975, 2009

    • Search Google Scholar
    • Export Citation
  • 32

    Trotter MI, & Briggs RJ: Cochlear implantation in neurofibromatosis type 2 after radiation therapy. Otol Neurotol 31:216219, 2010

  • 33

    UK Cochlear Implant Study Group: Criteria of candidacy for unilateral cochlear implantation in postlingually deafened adults III: prospective evaluation of an actuarial approach to defining a criterion. Ear Hear 25:361374, 2004

    • Search Google Scholar
    • Export Citation
  • 34

    Vincenti V, , Pasanisi E, , Guida M, , Di Trapani G, & Sanna M: Hearing rehabilitation in neurofibromatosis type 2 patients: cochlear versus auditory brainstem implantation. Audiol Neurootol 13:273280, 2008

    • Search Google Scholar
    • Export Citation
  • 35

    Wong HK, , Lahdenranta J, , Kamoun WS, , Chan AW, , McClatchey AI, & Plotkin SR, : Anti-vascular endothelial growth factor therapies as a novel therapeutic approach to treating neurofibromatosis-related tumors. Cancer Res 70:34833493, 2010

    • Search Google Scholar
    • Export Citation
  • 36

    Yang I, , Sughrue ME, , Han SJ, , Aranda D, , Pitts LH, & Cheung SW, : A comprehensive analysis of hearing preservation after radiosurgery for vestibular schwannoma. Clinical article. J Neurosurg 112:851859, 2010

    • Search Google Scholar
    • Export Citation

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