English consensus protocol evaluating candidacy for auditory brainstem and cochlear implantation in neurofibromatosis type 2

James R. Tysome, Patrick R. Axon, Neil P. Donnelly, Dafydd Gareth Evans, Rosalie E. Ferner, Alec F Fitzgerald O'Connor, Simon R. Freeman, Michael Gleeson, Dorothy Halliday, Frances Harris, Dan Jiang, Richard Kerr, Andrew King, Richard D. Knight, Simon K. Lloyd, Robert Macfarlane, Richard Mannion, Deborah Mawman, Martin O'Driscoll, Allyson ParryJames Ramsden, Richard Ramsden, Scott A. Rutherford, Shakeel R. Saeed, Nick Thomas, Zebunnisa H. Vanat

    Research output: Contribution to journalArticlepeer-review


    Objective: Hearing loss resulting from bilateral vestibular schwannomas (VSs) has a significant effect on the quality of life of patients with neurofibromatosis Type 2 (NF2). A national consensus protocol was produced in England as a guide for cochlear implantation (CI) and auditory brainstem implantation (ABI) in these patients. Study Design: Consensus statement. Setting: English NF2 Service. Participants: Clinicians from all 4 lead NF2 units in England. Main Outcome Measures: A protocol for the assessment, insertion and rehabilitation of CI and ABI in NF2 patients. Results: Patients should undergo more detailed hearing assessment once their maximum aided speech discrimination score falls below 50% in the better hearing ear. Bamford-Kowal-Bench sentence testing scores below 50% should trigger assessment for auditory implantation, as recommended by the National Institute for Clinical Excellence guidelines on CI. Where this occurs in patients with bilateral stable VS or a unilateral stable VS where the contralateral cochlear nerve was lost at previous surgery, CI should be considered. Where VS surgery is planned, CI should be considered where cochlear nerve preservation is thought possible, otherwise an ABI should be considered. Intraoperative testing using electrically evoked auditory brainstem responses or cochlear nerve action potentials may be used to determine whether a CI or ABI is inserted. Conclusion: The NF2 centers in England agreed on this protocol. Multisite, prospective assessments of standardized protocols for auditory implantation in NF2 provide an essential model for evaluating candidacy and outcomes in this challenging patient population. © 2013, Otology & Neurotology, Inc.
    Original languageEnglish
    Pages (from-to)1743-1747
    Number of pages4
    JournalOtology and Neurotology
    Issue number9
    Publication statusPublished - 2013


    • Auditory brain stem implantation
    • Cochlear implantation
    • Neurofibromatosis type 2


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