Cochlear implants in the management of hearing loss in Neurofibromatosis Type 2.

OBJECTIVE: Review of cochlear implant (CI) outcomes in patients with Neurofibromatosis Type 2 (NF2), implanted in the presence of an ipsilateral vestibular schwannoma (VS). Hearing restoration was combined in some cases with a Bevacizumab regime. METHOD: Retrospective review of 12 patients, managed over the period 2009-2016, at a tertiary referral multidisciplinary NF2 clinic. The patients are grouped by hearing outcomes to explore likely protective factors, and to generate a proposed decision-making tool for the selection of either CI or Auditory Brainstem Implant (ABI). RESULTS: Four of the 12 patients achieved speech discrimination without lip-reading. In these individuals there is reason to think that the mechanism of their hearing loss was cochlear dysfunction. A further four patients received benefit to lip-reading and awareness of environmental sound. For such patients their hearing loss may have been due to both cochlear and neural dysfunction. Two patients gained access to environmental sound only from their CI. Two patients derived no benefit from their CIs, which were subsequently explanted. Both these latter patients had had prior ipsilateral tumour surgery, one just before the CI insertion. CONCLUSION: Cochlear implantation can lead to open set speech discrimination in patients with NF2 in the presence of a stable VS. Use of promontory stimulation and intraoperative electrically evoked auditory brainstem response testing, along with case history, can inform the decision whether to implant an ABI or CI.

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

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.

Surgical management of vestibular schwannomas and hearing rehabilitation in neurofibromatosis type 2.

OBJECTIVES: To report our approach to the surgical management of vestibular schwannomas (VSs) and hearing rehabilitation in neurofibromatosis Type 2 (NF2). DESIGN: Retrospective cohort study. SETTING: Tertiary referral NF2 unit. PATIENTS: Between 1981 and 2011, seventy-five patients were managed in our NF2 unit, of which, 58 patients are under current review. MAIN OUTCOME MEASURES: Patients who underwent VS excision were evaluated for tumor size, surgical approach, and outcomes of hearing and facial nerve function. All current patients were evaluated for NF2 mutation, hearing, and auditory implantation outcomes. RESULTS: Forty-four patients underwent resection of 50 VS in our unit, of which, 14% had facial neuroma excision and reinnervation during the same operation. At 12 months after surgery, facial nerve outcomes were House-Brackmann (HB) 1 in 33%, HB2 in 21%, and HB3 in 30%. Total VS resection was achieved in 78% of patients using a translabyrinthine approach. Seventy-two percent of the current patients have American Association of Otolaryngology-Head and Neck Surgery class A to C hearing (maximum speech discrimination score over 50%) in the better hearing ear, and a further 14% are full-time users of cochlear implants or auditory brainstem implants. The remaining patients have been assessed for auditory implantation. CONCLUSION: By following a policy of treating VS in NF2 patients where tumor growth is observed, complete tumor resection can be achieved through a translabyrinthine approach while achieving comparable facial nerve outcomes to published series. We advocate proactive hearing rehabilitation in all patients with timely assessment for auditory implantation to maintain quality of life.

The use of Lean Thinking techniques in implementing the Department of Health, UK, 18-week waiting time directive for cochlear implantation.

In October 2007, Primary Care Trusts throughout the East of England agreed to fund assessment and implantation for all patients referred to Cambridge University Hospitals' Cochlear Implant Unit ('the Unit') providing they met locally agreed criteria. Subsequently, the Unit has employed Lean Thinking techniques to maximize departmental efficiency in order to comply with the Department of Health's 18-week guideline. Analysis of the patient's pathway, from referral to implantation surgery, highlighted areas of 'waste' that were causing delays in downstream processes. Through a number of streamlining measures a large waiting list of long-waiting patients (n = 141) has been cleared. Of those patients not receiving a preliminary hearing aid trial, 89% were implanted within 18 weeks (31/35 patients). Of those receiving the hearing aid trial 100% were assessed and commenced on the trial within 18 weeks, and 47% received implantation within the allotted time frame (7/15 patients). The Unit is continuing to monitor these processes to ensure these change s are continuing to maintain shorter waiting times. Equally, patients must feel that they have received optimal care and received sufficient information throughout the entire patient pathway.