Hearing optimisation in neurofibromatosis type 2: A systematic review.

BACKGROUND: It is common for patients with neurofibromatosis type 2 to develop bilateral profound hearing loss hearing loss, and this is one of the main determinants of quality of life in this patient group. OBJECTIVES: The aim of this systematic review was to review the current literature regarding hearing outcomes of treatments for vestibular schwannomas in neurofibromatosis type 2 including conservative and medical management, radiotherapy, hearing preservation surgery and auditory implantation in order to determine the most effective way of preserving or rehabilitating hearing. SEARCH STRATEGY: A MESH search in PubMed using search terms (('Neurofibromatosis 2' [Mesh]) AND 'Neuroma, Acoustic'[Mesh]) AND 'Hearing Loss' [Mesh] was performed. A search using keywords was also performed. Studies with adequate hearing outcome data were included. With the exception of the cochlear implant studies (cohort size was very small), case studies were excluded. EVALUATION METHOD: The GRADE system was used to assess quality of publication. Formal statistical analysis of data was not performed because of very heterogenous data reporting. RESULTS: Conservative management offers the best chance of hearing preservation in stable tumours. The use of bevacizumab probably improves the likelihood of hearing preservation in growing tumours in the short term and is probably more effective than hearing preservation surgery and radiotherapy in preserving hearing. Of the hearing preservation interventions, hearing preservation surgery probably offers better hearing preservation rates than radiotherapy for small tumours but recurrence rates for hearing preservation surgery were high. For patients with profound hearing loss, cochlear implantation provides significantly better auditory outcomes than auditory brainstem implantation. Patients with untreated stable tumours are likely to achieve the best outcomes from cochlear implantation. Those who have had their tumours treated with surgery or radiotherapy do not gain as much benefit from cochlear implantation than those with untreated tumours. CONCLUSIONS: This review summarises the current literature related to hearing preservation/rehabilitation in patients with NF2. Whilst it provides indicative data, the quality of the data was low and should be interpreted with care. It is also important to consider that the management of vestibular schwannomas in NF2 is complex and decision-making is determined by many factors, not just the need to preserve hearing.

Predictors of audiological outcome following cochlear implantation in adults.

The objective of this study was to examine variables that may predict open set speech discrimination following cochlear implantation. It consisted of a retrospective case review conducted in a tertiary referral centre with a cochlear implant programme. The patients were 117 postlingually deafened adult cochlear implant recipients. The main outcome measures were Bench, Kowal, Bamford (BKB) sentence scores recorded nine months following implant activation. The variables studied were age at the time of surgery, sex, duration of hearing loss, aetiology of hearing loss, residual hearing, implant type, speech processor strategy, number of active electrodes inserted. Variables found to have a significant effect on BKB following univariate analysis were entered into a multivariate analysis to determine independent predictors. Multivariate ordinal regression analysis gave an odds ration of 1.09 for each additional year of deafness prior to implantation (confidence interval 1.06-1.13; p < 0.001). Duration of deafness prior to implantation is an independent predictor of implant outcome. It accounted for 9% of the variability. Other factors must influence implant performance.

Device nonuse among adult cochlear implant recipients.

OBJECTIVE: To examine the causes and prevalence of previous and current device nonuse among adults who have received cochlear implants. STUDY DESIGN: Retrospective case review. SETTING: Adult tertiary referral center for cochlear implantation. METHODS: Two hundred fourteen consecutively implanted adult patients. The length of implant use ranged from 1 month to 14 years. MAIN OUTCOME MEASURE: A period of 4 consecutive weeks of nonuse of cochlear implant, including both obligatory and elective nonuse. RESULTS: Twenty-nine adults (13.6% of implantees) were identified as having at some stage not used their device for a period of more than 4 consecutive weeks. The main reason was device failure (n = 11). Ten adults are current nonusers (4.7% of implantees). Reasons include surgical complication necessitating explantation (n = 3), comorbid illness (n = 3), elective nonuse (n = 2), audiologic complication (n = 1), and device failure (n = 1). CONCLUSION: The overall prevalence of device nonuse was noted to increase slowly with time. The role of psychologic factors in contributing to the decision of an individual to elect to opt out of device use remains unproven.

Trends and outcomes in the Manchester adult cochlear implant series.

The adult cochlear implant programme in Manchester was established in 1988, initially using funding obtained from the HEAR (Help Ear and Allied Research: charity number: 519784) charity before government resources became available in the mid-1990s. Manchester was the first centre in the UK to implant multichannel devices on a regular basis. To date, over 250 adults have been implanted, including nine bilateral and eight deaf-blind patients. All the patients have a postlingual onset of severe-profound hearing loss; 73% (n = 175) of the implants performed used a Nucleus multichannel implant and 24% (n = 58) used a Medel multichannel implant. In addition, the team has implanted three Medel single channel devices, two Ineraid devices and one Clarion High Focus II device. This study is a retrospective analysis of the trends and outcomes in implant fitting during the first 14 years (1988-2002) of the programme. The paper describes the patient demographics and audiological complications for 240 implantations performed on 214 patients. Speech perception outcomes are reported for a subset of the patients. The average score for the Bench, Kowal, Bamford sentence test at the post-18-month stage of implant use is 66% and for Arthur Boothroyd words 53%. Trends in the series are analysed with respect to the change in criteria for adult implantation, the move towards bilateral implantation and the rate of uptake of cochlear implants by different ethnic groups.

Cochlear implantation in the presence of chronic suppurative otitis media.

Nine patients are presented who underwent cochlear implantation in the presence of chronic suppurative otitis media. Four had a simple tympanic membrane perforation, four had a pre-existing mastoid cavity and one had cholesteatoma in the ear chosen for implantation. Patients with a simple perforation had a staged procedure with myringoplasty followed by cochlear implantation after an interval of three months. Patients with cholesteatoma or with an unstable mastoid cavity were also staged. A mastoidectomy or revision mastoidectomy was performed with obliteration of the middle ear and mastoid using a superiorly pedicled temporalis muscle flap and blind sac closure of the external meatal skin. After a further six months a second stage procedure was performed to confirm that the middle-ear cleft was healthy and to insert the implant. Patients presenting with a stable mastoid cavity underwent obliteration of the cavity and implantation of the electrode as a one-staged procedure. To date there have been no serious problems such as graft breakdown, recurrence of disease or implant extrusion, and all patients are performing well.

An audit of the cochlear implant service in Manchester.

The adult cochlear implant programme in Manchester was established in 1988 and the evaluation of the cochlear implant service involved the first 58 implants users (mean age = 51.65 years, range 19-75 years). Questionnaires were sent to implant users and their partners to evaluate the service with regard to provision of information, clinical care during in-patient assessments, waiting times, operation for cochlear implant and post-operative rehabilitation. The results show that the majority of patients (78 per cent) felt that the implant gave them as much or more benefit than expected. Areas identified for improvements include provision of more written information about cochlear implants; reduction in waiting times for first appointments; more information about the surgical risks and more instruction about home auditory training exercises for family and friends. As a consequence of the audit results the clinical practice and service provision for cochlear implantation in Manchester has been modified.