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Updated: May 6, 2026
Systematic Hearing Performance Evaluation Process for Adolescents with Cochlear Implantation at Early Ages
Published on: March 24, 2023
English consensus protocol evaluating candidacy for auditory brainstem and cochlear implantation in neurofibromatosis
James R Tysome1, Patrick R Axon, Neil P Donnelly
1*Department of Otolaryngology, Cambridge University Hospitals NHS Foundation Trust, Cambridge; †Department of Genetics, Manchester Royal Infirmary, Manchester; ‡Department of Neurology, and §Department of Otolaryngology, Guy's and St. Thomas' NHS Foundation Trust, London; ∥Department of Otolaryngology, Manchester Royal Infirmary, Manchester; ¶Department of Otolaryngology, National Hospital for Neurology and Neurosurgery, London; #Department of Neurology, John Radcliffe Hospital, Oxford; ** Emmeline Centre for Hearing Implants, Cambridge University Hospitals NHS Foundation Trust, Cambridge; ††Department of Neurosurgery, John Radcliffe Hospital, Oxford; ‡‡Department of Neurosurgery, Manchester Royal Infirmary, Manchester; §§Department of Audiology, and ∥∥Department of Neurosurgery, Cambridge University Hospitals NHS Foundation Trust, Cambridge; ¶¶Department of Audiology, Manchester Royal Infirmary, Manchester; ##Department of Otolaryngology, John Radcliffe Hospital, Oxford; ***Department of Otolaryngology, Royal National Throat Nose and Ear Hospital; and †††Department of Neurosurgery, King's College Hospital NHS Trust, London, U.K.
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.
