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

Clinical article

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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.


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.


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.


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.

Article Information

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:

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

© AANS, except where prohibited by US copyright law.



  • View in gallery

    Illustration of an ABI. The device consists of an external microphone and processor, which connects to an internalized receiver and electrode array through the scalp. The electrode paddle, shown in greater detail in the inset, is placed within the lateral recess by the cochlear nucleus. Illustration provided courtesy of CochlearTM Americas, ©2009 Cochlear Americas.

  • View in gallery

    Illustration of the cochlear implant. The device consists of an external microphone and processor, which connects to an internalized receiver and electrode array through the scalp. The linear electrode is placed within the cochlea. Illustration provided courtesy of CochlearTM Americas, ©2009 Cochlear Americas.



Ahsan STelischi FHodges ABalkany T: Cochlear implantation concurrent with translabyrinthine acoustic neuroma resection. Laryngoscope 113:4724742003


Arístegui MDenia 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 26:2052102005


Arriaga MAMarks S: Simultaneous cochlear implantation and acoustic neuroma resection: imaging considerations, technique, and functional outcome. Otolaryngol Head Neck Surg 112:3253281995


Asthagiri ARParry DMButman JAKim HJTsilou ETZhuang Z: Neurofibromatosis type 2. Lancet 373:197419862009


Colletti VShannon RV: Open set speech perception with auditory brainstem implant?. Laryngoscope 115:197419782005


Colletti VShannon RVCarner MVeronese SColletti L: Complications in auditory brainstem implant surgery in adults and children. Otol Neurotol 31:5585642010


Denia AArístegui M: Cochlear implantation after translabyrinthine acoustic tumour removal: preliminary results. Cochlear Implants Int 6:Suppl 120242005


Di Nardo WFetoni ABuldrini SDi Girolamo S: Auditory brainstem and cochlear implants: functional results obtained after one year of rehabilitation. Eur Arch Otorhinolaryngol 258:582001


El-Kashlan HKAshbaugh CZwolan TTelian SA: Cochlear implantation in prelingually deaf children with ossified cochleae. Otol Neurotol 24:5966002003


Evans DGNeurofibromatosis 2. Pagon RABird TCDolan CR: GeneReviews SeattleUniversity of Washington, Seattle1998. ( [Accessed June 3 2011]


Evans DG: Neurofibromatosis type 2 (NF2): a clinical and molecular review. Orphanet J Rare Dis 4:162009


Graham JLynch CWeber BStollwerck LWei JBrookes G: The magnetless Clarion cochlear implant in a patient with neurofibromatosis 2. J Laryngol Otol 113:4584631999


Green JD JrMarion MSHinojosa R: Labyrinthitis ossificans: histopathologic consideration for cochlear implantation. Otolaryngol Head Neck Surg 104:3203261991


Hinojosa RGreen JD JrMarion MS: Ganglion cell populations in labyrinthitis ossificans. Am J Otol 12:Suppl371991


Hoffman RAKohan DCohen NL: Cochlear implants in the management of bilateral acoustic neuromas. Am J Otol 13:5255281992


Hulka GFBernard EJPillsbury 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 121:4654681995


Kanowitz SJShapiro WHGolfinos JGCohen NLRoland JT Jr: Auditory brainstem implantation in patients with neurofibromatosis type 2. Laryngoscope 114:213521462004


Kaylie DMHorgan MJDelashaw JBMcMenomey SO: A meta-analysis comparing outcomes of microsurgery and gamma knife radiosurgery. Laryngoscope 110:185018562000


Kondziolka DLunsford LDFlickinger JC: Acoustic neuromas. Curr Treat Options Neurol 4:1571652002


Lustig LRYeagle JDriscoll CLBlevins NFrancis HNiparko JK: Cochlear implantation in patients with neurofibromatosis type 2 and bilateral vestibular schwannoma. Otol Neurotol 27:5125182006


Neff BAWiet RMLasak JMCohen NLPillsbury HCRamsden RT: Cochlear implantation in the neurofibromatosis type 2 patient: long-term follow-up. Laryngoscope 117:106910722007


Nölle CTodt IBasta DUnterberg AMautner VFErnst 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 65:2302342003


Piccirillo EGuida MFlanagan SLauda LFois PSanna M: CNAP to predict functional cochlear nerve preservation in NF-2: cochlear implant or auditory brainstem implant. Skull Base 18:2812872008


Plotkin SRHalpin CMcKenna MJLoeffler JSBatchelor TTBarker FG II: Erlotinib for progressive vestibular schwannoma in neurofibromatosis 2 patients. Otol Neurotol 31:113511432010


Plotkin SRSingh MAO'Donnell CCHarris GJMcClatchey AIHalpin C: Audiologic and radiographic response of NF2-related vestibular schwannoma to erlotinib therapy. Nat Clin Pract Oncol 5:4874912008


Rauch SDHerrmann BSDavis LANadol JB Jr: Nucleus 22 cochlear implantation results in postmeningitic deafness. Laryngoscope 107:160616091997


Rouleau GAMerel PLutchman MSanson MZucman JMarineau C: Alteration in a new gene encoding a putative membrane-organizing protein causes neuro-fibromatosis type 2. Nature 363:5155211993


Schwartz MSOtto SRShannon RVHitselberger WEBrackmann DE: Auditory brainstem implants. Neurotherapeutics 5:1281362008


Temple RHAxon PRRamsden RTKeles NDeger KYücel E: Auditory rehabilitation in neurofibromatosis type 2: a case for cochlear implantation. J Laryngol Otol 113:1611631999


Tono TUshisako YMorimitsu T: Cochlear implantation in an intralabyrinthine acoustic neuroma patient after resection of an intracanalicular tumour. J Laryngol Otol 110:5705731996


Tran Ba Huy PKania RFrachet BPoncet CLegac MS: Auditory rehabilitation with cochlear implantation in patients with neurofibromatosis type 2. Acta Otolaryngol 129:9719752009


Trotter MIBriggs RJ: Cochlear implantation in neurofibromatosis type 2 after radiation therapy. Otol Neurotol 31:2162192010


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 25:3613742004


Vincenti VPasanisi EGuida MDi Trapani GSanna M: Hearing rehabilitation in neurofibromatosis type 2 patients: cochlear versus auditory brainstem implantation. Audiol Neurootol 13:2732802008


Wong HKLahdenranta JKamoun WSChan AWMcClatchey AIPlotkin SR: Anti-vascular endothelial growth factor therapies as a novel therapeutic approach to treating neurofibromatosis-related tumors. Cancer Res 70:348334932010


Yang ISughrue MEHan SJAranda DPitts LHCheung SW: A comprehensive analysis of hearing preservation after radiosurgery for vestibular schwannoma. Clinical article. J Neurosurg 112:8518592010


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