Search

Objective/hypothesis: The aim of this study was to explore the cost-utility of pediatric cochlear implantation, incorporating savings associated with education into the analysis. Methods: The costs of pediatric cochlear implantation were based on the full costs levied to purchasers, inclusive of complications and maintenance, by a large pediatric cochlear implant program in the United Kingdom. After implantation, profoundly hearing-impaired children have been found to develop hearing threshold levels equivalent to severely hearing-impaired children who wear hearing aids. An independent study calculated the educational costs for severely hearing-impaired and profoundly hearing-impaired children. From this study, savings in educational costs that would result from enabling the profoundly hearing-impaired to function as severely hearing-impaired were determined. Cost-utility was established conservatively by applying to children the known gains in utility reported by adults with cochlear implants. Results: The discounted costs of creating a pediatric cochlear implant user and of maintaining the child over the first 12 years were 48,757 pound silver($78,011). The discounted difference in education costs associated with a profoundly hearing-impaired child (HL >95 dB) as compared with a severely hearing-impaired child (HL 70-95 dB) over the same period was 26,781 pound silver($42,850). These represent the potential savings in educational costs associated with pediatric cochlear implantation. Assuming implantation at age 4 years, the discounted net average cost of pediatric cochlear implantation over compulsory school years (ages 4-16) was 21,976 pound silver ($35,162). Cochlear implants have been shown to improve the quality of life in adults by 0.23 points per annum (where quality of life is rated on a scale from 0 to 1). Applying this weight to children receiving implantation at age 4 years, and assuming a life expectancy of 74 years, the quality-adjusted life-year (QALY) gain is calculated to be 16.33. The cost per undiscounted QALY gain was estimated to be 1,345.70 pound silver ($2153.12) and per discounted QALY gain to be 10,341 pound silver ($16,545.60). Conclusion: This study provides evidence, based on conservative assumptions, to support the view that pediatric cochlear implantation is a cost-effective health care intervention in profoundly hearing-impaired young children. [ABSTRACT FROM AUTHOR]

Background: Following the approval of bilateral paediatric cochlear implantation in 2009, the prospective multi-centre UK National Paediatric Cochlear Implant Audit was established to collect a large dataset of paediatric implantations. The aim of the surgical part of the audit, reported here, was to collect data on surgical practice, outcomes and complications. Methods: Data from 14 surgical centres was collected prospectively, including simultaneous and sequential bilateral as well as unilateral implantations. Data collected included age at implantation, aetiology of deafness, implant type, duration of surgery, the use of electrophysiological testing, and the use of pre- and post-operative imaging. Details of major and immediate minor complications were also recorded. Results: 1397 CI procedures in 961 CI recipients were included; 436 bilateral simultaneous, 394 bilateral sequential, 131 unilateral. The overall major complication rate was 1.6% (0.9% excluding device failure) and was similar following bilateral CI compared to sequential and unilateral CI. Conclusion: This prospective multi-centre audit provides evidence that bilateral paediatric CI is a safe procedure in the UK, thus endorsing its role as a major therapeutic intervention in childhood deafness. [ABSTRACT FROM AUTHOR]

Objective: To assess how surgery affected the quality of life of patients with acoustic neuromas and to investigate possible predictors of the functional outcome following surgery.Study Design: A questionnaire based on the Glasgow Benefit Inventory was completed by patients randomly selected following acoustic neuroma surgery.Setting: Skull base surgery unit of a university teaching hospital (tertiary referral center).Patients: Fifty-three patients with acoustic tumors (follow-up, 1 to 3 y).Results: With regard to overall quality of life, nine patients (17.4%) reported that it became better, 28 patients (53.8%) worse, and 15 patients (28.8%) that it remained the same. Four patients (7.8%) became better off financially, 15 patients (29.4%) worse, and 32 (62.8%) remained unchanged. Forty-one patients (78.8%) did not change their occupation, and 11 (21.2%) had to change their occupation, mainly because of the adverse effects of the operation. With regard to the age at operation, older patients were found to have better overall quality of life. Moreover, younger patients had worse postoperative financial status and they were more likely to change their occupation after the operation. The tumor size did not significantly affect the overall postoperative quality, but it did affect the postoperative financial status (patients with larger tumors were more likely to have worse postoperative financial status).Conclusion: Acoustic neuroma surgery has a significant impact on patients' overall quality of life. Surgeons proposing to operate on small tumors should not assume that the impact on patients' life will be necessarily less than that following the removal of larger tumors. All patients, especially in the younger age group, should be prepared and thoroughly informed about the consequences of the operation on their quality of life. [ABSTRACT FROM AUTHOR]

The human inner ear contains minute three-dimensional neurosensory structures that are deeply embedded within the skull base, rendering them relatively inaccessible to regenerative therapies for hearing loss. Here we provide a detailed characterisation of the functional architecture of the space that hosts the cell bodies of the auditory nerve to make them safely accessible for the first time for therapeutic intervention. We used synchrotron phase-contrast imaging which offers the required microscopic soft-tissue contrast definition while simultaneously displaying precise bony anatomic detail. Using volume-rendering software we constructed highly accurate 3-dimensional representations of the inner ear. The cell bodies are arranged in a bony helical canal that spirals from the base of the cochlea to its apex; the canal volume is 1.6 μL but with a diffusion potential of 15 μL. Modelling data from 10 temporal bones enabled definition of a safe trajectory for therapeutic access while preserving the cochlea's internal architecture. We validated the approach through surgical simulation, anatomical dissection and micro-radiographic analysis. These findings will facilitate future clinical trials of novel therapeutic interventions to restore hearing., (© 2022. The Author(s).)

Background: Hearing loss affects over 50% of people in the US across their lifespan and there is a lack of decision modeling frameworks to inform optimal hearing healthcare delivery. Our objective was to develop and validate a microsimulation model of hearing loss across the lifespan in the US., Methods: We collaborated with the Lancet Commission on Hearing Loss to outline model structure, identify input data sources, and calibrate/validate DeciBHAL-US (Decision model of the Burden of Hearing loss Across the Lifespan). We populated the model with literature-based estimates and validated the conceptual model with key informants. We validated key model endpoints to the published literature, including: 1) natural history of sensorineural hearing loss (SNHL), 2) natural history of conductive hearing loss (CHL), and 3) the hearing loss cascade of care. We reported the coefficient of variance root mean square error (CV-RMSE), considering values ≤15% to indicate adequate fit., Findings: For SNHL prevalence, the CV-RMSE for model projected male and female age-specific prevalence compared to sex-adjusted National Health and Nutrition Examination Survey (NHANES) data was 4.9 and 5.7%, respectively. Incorporating literature-based age-related decline in SNHL, we validated mean four-frequency average hearing loss in the better ear (dB) among all persons to longitudinal data (CV-RMSE=11.3%). We validated the age-stratified prevalence of CHL to adjusted NHANES data (CV-RMSE=10.9%). We incorporated age- and severity-stratified time to first hearing aid (HA) use data and HA discontinuation data (adjusted for time-period of use) and validated to NHANES estimates on the prevalence of adult HA use (CV-RMSE=10.3%)., Interpretation: Our results indicate adequate model fit to internal and external validation data. Future incorporation of cost and severity-stratified utility data will allow for cost-effectiveness analysis of US hearing healthcare interventions across the lifespan. Further research might expand the modeling framework to international settings., Funding: This study was funded by the National Institute on Deafness and Other Communication Disorders and the National Institute on Aging (3UL1-TR002553-03S3 and F30 DC019846)., Competing Interests: JRD reports grants from the National Institutes of Health, participation on the National Institute on Deafness and Other Communication Disorders Data Safety Monitoring Board (DSMB) and the National Institute on Aging ACHIEVE (Aging and Cognitive Health Evaluation in Elders) DSMB, and participation on the Board of Directors of the Hearing Health Foundation and on the Executive Council of the Acoustical Society of America. All other authors declare no competing interests., (© 2021 Published by Elsevier Ltd.)

Background: Mastoid surgery is an aerosol-generating procedure that involves the use of a high-speed drill, which produces a mixture of water, bone, blood and tissue that may contain the viable coronavirus disease 2019 pathogen. This potentially puts the surgeon and other operating theatre personnel at risk of acquiring the severe acute respiratory syndrome coronavirus-2 from contact with droplets or aerosols. The use of an additional drape designed to limit the spread of droplets and aerosols has been described; such drapes include the 'Southampton Tent' and 'OtoTent'., Objectives: To evaluate the use of a novel drape 'tent' that has advantages over established 'tent' designs in terms of having: (1) a CE marking; (2) no requirement for modification during assembly; and (3) no obstruction to the surgical visual field., Results and Conclusion: During mastoid surgery, the dispersion of macroscopic droplets and other particulate matter was confined within the novel drape 'tent'. Use of this drape 'tent' had no adverse effects upon the surgeon's manual dexterity or efficiency, the view of the surgical field, or the sterility. Hence, our findings support its use during mastoid surgery in the coronavirus disease 2019 era.

In 2015, approximately half a billion people had disabling hearing loss, about 6·8% of the world's population. These numbers are substantially higher than estimates published before 2013, and point to the growing importance of hearing loss and global hearing health care. In this Review, we describe the burden of hearing loss and offer our and others' recommendations for halting and then reversing the continuing increases in this burden. Low-cost possibilities exist for prevention of hearing loss, as do unprecedented opportunities to reduce the generally high treatment costs. These possibilities and opportunities could and should be exploited. Additionally, a comprehensive worldwide initiative like VISION 2020 but for hearing could provide a focus for support and also enable and facilitate the increased efforts that are needed to reduce the burden. Success would produce major personal and societal gains, including gains that would help to fulfil the "healthy lives" and "disability inclusive" goals in the UN's new 2030 Agenda for Sustainable Development., (Copyright © 2017 Elsevier Ltd. All rights reserved.)

Hypothesis: Assess the clinical acceptability of direct acoustic cochlear implantation for patients with advanced otosclerosis and the support for conducting a controlled trial of its effectiveness in the United Kingdom., Background: Emerging evidence supports the efficacy of direct acoustic cochlear implantation in patients with advanced otosclerosis whose needs cannot be managed using the combination of stapes surgery and hearing aids. A controlled trial would provide evidence for its effectiveness and cost-effectiveness to healthcare commissioners., Methods: An online survey of clinical professionals was constructed to characterize current standard of care for patients with advanced otosclerosis and to assess whether clinicians would be willing to refer patients into a trial to evaluate direct acoustic cochlear implantation. A consensus process was conducted to define inclusion criteria for the future trial., Results: No survey respondent considered direct acoustic cochlear implantation to be inappropriate with a majority indicating that they would refer patients into a future trial. The consensus was that there is a lack of available treatment options for those patients with bone conduction thresholds worse than 55 dB HL and who did not meet current criteria for cochlear implantation., Conclusion: The present study confirms that a controlled trial to evaluate the effectiveness of direct acoustic cochlear implantation would have the support of clinicians in the United Kingdom. A feasibility study would be required to determine whether patients who meet the inclusion criteria could be recruited in a timely manner and in sufficient numbers to conduct a formal evaluation of effectiveness.

Objective: To assess patient-reported outcomes and experience of vestibular schwannoma (VS) management., Study Design: Survey of members of the British Acoustic Neuroma Association (BANA)., Methods: Members of the BANA were invited to complete an online survey. Questions were divided into five areas: Demographic details; symptoms at diagnosis; level of information received; treatment after-effects; and overall experience of diagnosis and/or treatment., Demographics: 598 (58%) BANA members completed the survey. 77.1% of respondents were aged between 41 and 70 years. Symptoms at diagnosis: hearing loss (84%), unilateral tinnitus (40%) and imbalance (51%) were commonest. Isolated tinnitus and imbalance occurred in 2% and 6%, respectively. Information received: 39% stated they were given 'just the right amount of information about all management options', and 32% 'not enough information'. Treatment after-effects: Overall quality of life was classified as 'a lot better' (11%), 'a little better' (7%), 'unchanged' (25%), 'a little worse' (38%) and 'a lot worse' (19%). 61% respondents continued in the same job. Return to social life, hobbies and sports was impaired in 65%. Overall experience: Experience of treatment was graded as 'much better than expected' (20%), 'a little better' (15%), 'about the same' (27%), 'a little worse' (22%) and 'much worse' (16%)., Conclusion: Quality of life measures are important in assessing VS management outcomes, and will increasingly inform clinical decision-making. Further examination of how patients with VS perceive their disease, cope with illness and use social support networks may also help to inform future practice and the creation of decision analytical models.

Progress in biomedical technology (cochlear, vestibular, and retinal implants) has led to remarkable success in neurosensory restoration, particularly in the auditory system. However, outcomes vary considerably, even after accounting for comorbidity-for example, after cochlear implantation, some deaf children develop spoken language skills approaching those of their hearing peers, whereas other children fail to do so. Here, we review evidence that auditory deprivation has widespread effects on brain development, affecting the capacity to process information beyond the auditory system. After sensory loss and deafness, the brain's effective connectivity is altered within the auditory system, between sensory systems, and between the auditory system and centres serving higher order neurocognitive functions. As a result, congenital sensory loss could be thought of as a connectome disease, with interindividual variability in the brain's adaptation to sensory loss underpinning much of the observed variation in outcome of cochlear implantation. Different executive functions, sequential processing, and concept formation are at particular risk in deaf children. A battery of clinical tests can allow early identification of neurocognitive risk factors. Intervention strategies that address these impairments with a personalised approach, taking interindividual variations into account, will further improve outcomes., (Copyright © 2016 Elsevier Ltd. All rights reserved.)

Background: With the global escalation of type 2 diabetes and evidence consistently showing that its onset can be prevented or delayed by changing lifestyle behaviours, there is an urgent need to translate practical, affordable and acceptable interventions from the research setting into the real world. One such approach to lifestyle interventions might be the introduction of a programme in which the individual is provided with choice and facilitated to 'self-select' an exercise programme. Previous research has shown that this is likely to be less resource intensive, an essential requirement for success outside the controlled research environment, while at the same time promoting positive responses relating to adherence, competence and self-efficacy, essential attributes for long-term success. Through a two-group parallel-randomised controlled trial, this study aims to assess the clinical and psychological impact of the DEXLIFE 'self-selected' lifestyle modification programme in adults at risk of developing type 2 diabetes., Methods/design: A total of 360 subjects at risk of developing type 2 diabetes are randomly assigned in a 1:3 ratio to a control (n = 90) or intervention arm (n = 270). Randomization is stratified by age, sex and body mass index. The control arm receives general information on lifestyle and diabetes risk. The intervention group participate in a 12 week 'self-selected' supervised exercise training programme accompanied with dietary advice to improve food choices. Participants are given access to Dublin City University Sport (an on-campus gym) and asked to perform four exercise classes per week. Dublin City University Sport offers over 50 classes per week, many of which are medically supervised. If weight loss is indicated, reduction in total calorie intake by 600 kcal/day is advised. Common to all food plans is <10% saturated fat intake, as well as a dietary fibre intake of >15 g/1000 kcal. Insulin sensitivity is the primary outcome measure. Secondary outcome measures include glucose function, fitness, body composition, anthropometrics, heart rate variability, lipid profiles, blood pressure, physical activity levels, dietary intake and quality of life., Discussion: "Self-selected" lifestyle intervention has not previously been evaluated in type 2 diabetes prevention and if shown to be successful could be implemented in practice immediately., Trial Registration: Current Controlled Trials: ISRCTN66987085.

Background: Individuals with a unilateral severe-to-profound hearing loss, or single-sided deafness, report difficulty with listening in many everyday situations despite having access to well-preserved acoustic hearing in one ear. The standard of care for single-sided deafness available on the UK National Health Service is a contra-lateral routing of signals hearing aid which transfers sounds from the impaired ear to the non-impaired ear. This hearing aid has been found to improve speech understanding in noise when the signal-to-noise ratio is more favourable at the impaired ear than the non-impaired ear. However, the indiscriminate routing of signals to a single ear can have detrimental effects when interfering sounds are located on the side of the impaired ear. Recent published evidence has suggested that cochlear implantation in individuals with a single-sided deafness can restore access to the binaural cues which underpin the ability to localise sounds and segregate speech from other interfering sounds., Methods/design: The current trial was designed to assess the efficacy of cochlear implantation compared to a contra-lateral routing of signals hearing aid in restoring binaural hearing in adults with acquired single-sided deafness. Patients are assessed at baseline and after receiving a contra-lateral routing of signals hearing aid. A cochlear implant is then provided to those patients who do not receive sufficient benefit from the hearing aid. This within-subject longitudinal design reflects the expected care pathway should cochlear implantation be provided for single-sided deafness on the UK National Health Service. The primary endpoints are measures of binaural hearing at baseline, after provision of a contra-lateral routing of signals hearing aid, and after cochlear implantation. Binaural hearing is assessed in terms of the accuracy with which sounds are localised and speech is perceived in background noise. The trial is also designed to measure the impact of the interventions on hearing- and health-related quality of life., Discussion: This multi-centre trial was designed to provide evidence for the efficacy of cochlear implantation compared to the contra-lateral routing of signals. A purpose-built sound presentation system and established measurement techniques will provide reliable and precise measures of binaural hearing., Trial Registration: Current Controlled Trials http://www.controlled-trials.com/ISRCTN33301739 (05/JUL/2013).

Objective: Spatial hearing uses both monaural and binaural mechanisms that require sensitive hearing for normal function. Deaf children using either bilateral (BCI) or unilateral (UCI) cochlear implants would thus be expected to have poorer spatial hearing than normally hearing (NH) children. However, the relationship between spatial hearing in these various listener groups has not previously been extensively tested under ecologically valid conditions using a homogeneous group of children who are UCI users. We predicted that NH listeners would outperform BCI listeners who would, in turn, outperform UCI listeners., Methods: We tested two methods of spatial hearing to provide norms for NH and UCI using children and preliminary data for BCI users. NH children (n=40) were age matched (6-15 years) to UCI (n=12) and BCI (n=6) listeners. Testing used a horizontal ring of loudspeakers within a booth in a hospital outpatient clinic. In a 'lateral release' task, single nouns were presented frontally, and masking noises were presented frontally, or 90° left or right. In a 'localization' task, allowing head movements, nouns were presented from loudspeakers separated by 30°, 60° or 120° about the midline., Results: Normally hearing children improved with age in speech detection in noise, but not in quiet or in lateral release. Implant users performed more poorly on all tasks. For frontal signals and noise, UCI and BCI listeners did not differ. For lateral noise, BCI listeners performed better on both sides (within ~2 dB of NH), whereas UCI listeners benefited only when the noise was opposite the unimplanted ear. Both the BCI and, surprisingly, the UCI listeners performed better than chance at all loudspeaker separations on the ecologically valid, localization task. However, the BCI listeners performed about twice as well and, in two cases, approached the performance of NH children., Conclusion: Children using either UCI or BCI have useful spatial hearing. BCI listeners gain benefits on both sides, and localize better, but not as well as NH listeners., (Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.)

Objective: To design an international standard to be used when reporting reliability of the implanted components of cochlear implant systems to appropriate governmental authorities, cochlear implant (CI) centers, and for journal editors in evaluating manuscripts involving cochlear implant reliability., Study Design: The International Consensus Group for Cochlear Implant Reliability Reporting was assembled to unify ongoing efforts in the United States, Europe, Asia, and Australia to create a consistent and comprehensive classification system for the implanted components of CI systems across manufacturers., Setting: All members of the consensus group are from tertiary referral cochlear implant centers., Interventions: None., Main Outcome Measure: A clinically relevant classification scheme adapted from principles of ISO standard 5841-2:2000 originally designed for reporting reliability of cardiac pacemakers, pulse generators, or leads., Results: Standard definitions for device failure, survival time, clinical benefit, reduced clinical benefit, and specification were generated. Time intervals for reporting back to implant centers for devices tested to be "out of specification," categorization of explanted devices, the method of cumulative survival reporting, and content of reliability reports to be issued by manufacturers was agreed upon by all members. The methodology for calculating Cumulative survival was adapted from ISO standard 5841-2:2000., Conclusion: The International Consensus Group on Cochlear Implant Device Reliability Reporting recommends compliance to this new standard in reporting reliability of implanted CI components by all manufacturers of CIs and the adoption of this standard as a minimal reporting guideline for editors of journals publishing cochlear implant research results.

Cochlear implantation is effective at restoring partial hearing to profoundly deaf adults, but not all patients receive equal benefit. The present study evaluated the effectiveness of a computer-based self-administered training package that was designed to improve speech perception among adults who had used cochlear implants for more than three years. Eleven adults were asked to complete an hour of auditory training each day, five days a week, for a period of three weeks. Two training tasks were included, one based around discriminating isolated words, and the other around discriminating words in sentences. Compliance with the protocol was good, with eight out of eleven participants completing approximately 15 hours of training, as instructed. A significant improvement of eight percentage points was found on a test of consonant discrimination, but there were no significant improvements on sentence tests or on a test of vowel discrimination. Self-reported benefits were variable and generally small. Further research is needed to establish whether auditory training is particularly effective for identifiable sub-groups of cochlear-implant users.

Background: While approximately 70% of chronic low back pain (CLBP) sufferers complain of sleep disturbance, current literature is based on self report measures which can be prone to bias and no objective data of sleep quality, based exclusively on CLBP are available. In accordance with the recommendations of The American Sleep Academy, when measuring sleep, both subjective and objective assessments should be considered as the two are only modestly correlated, suggesting that each modality assesses different aspects of an individual's sleep experience. Therefore, the purpose of this study was to expand previous research into sleep disturbance in CLBP by comparing objective and subjective sleep quality in participants with CLBP and healthy age and gender matched controls, to identify correlates of poor sleep and to test logistics and gather information prior to a larger study., Methods: 15 CLBP participants (mean age = 43.8 years (SD = 11.5), 53% female) and 15 healthy controls (mean age = 41.5 years (SD = 10.6), 53% female) consented. All participants completed the Pittsburgh Sleep Quality Index, Insomnia Severity Index, Pittsburgh Sleep Diary and the SF36v2. CLBP participants also completed the Oswestry Disability Index. Sleep patterns were assessed over three consecutive nights using actigraphy. Total sleep time (TST), sleep efficiency (SE), sleep latency onset (SL) and number of awakenings after sleep onset (WASO) were derived. Statistical analysis was conducted using unrelated t-tests and Pearson's product moment correlation co-efficients., Results: CLBP participants demonstrated significantly poorer overall sleep both objectively and subjectively. They demonstrated lower actigraphic SE (p = .002) and increased WASO (p = .027) but no significant differences were found in TST (p = .43) or SL (p = .97). Subjectively, they reported increased insomnia (p =< .001), lower SE (p =< .001) and increased SL (p =< .001) but no difference between TST (p = .827) and WASO (p = .055). Statistically significant associations were found between low back pain (p = .021, r = -.589), physical health (p = .003, r = -.713), disability levels (p = .025, r = .576), and subjective sleep quality in the CLBP participants but not with actigraphy., Conclusion: CLBP participants demonstrated poorer overall sleep, increased insomnia symptoms and less efficient sleep. Further investigation using a larger sample size and a longer period of sleep monitoring is ongoing.

Objectives/hypothesis: Previous studies on hearing loss (HL) after acoustic neuroma removal concentrate mainly on pure-tone hearing results rather than hearing disability. Our objectives were to use the Speech, Spatial and Qualities of Hearing scale (SSQ), a comprehensively validated questionnaire, to characterize and quantify the auditory disabilities that patients experience with a profound unilateral HL after acoustic neuroma removal., Study Design: Forty-four patients with profound unilateral HL after acoustic neuroma surgery completed the SSQ. Their findings were compared with those of a control population sample matched for age, sex, and hearing level in the better hearing ear., Results: In comparison with controls, with use of analysis of variance, acoustic neuroma patients scored poorly on all items except for the identification of sounds and objects (P = .123). The greatest difficulties involved speech in the presence of noise, situations of multiple speech-streams and switching (such as listening to someone speaking and the television at the same time), the location of unseen objects, and increased listening effort (P < .05)., Conclusion: This study demonstrates that, compared with a control population, these patients experience a significant range of auditory disabilities. It is important that clinicians be aware of the impact of such a profound unilateral HL and its potential to affect daily life. Patient counseling prior to surgery is essential, especially in patients whose loss of binaural hearing could constitute a major disability.

Objectives: The objective of this study was to evaluate long-term speech perception abilities of comparable groups of postmeningitic and congenitally deaf children after cochlear implantation., Methods: This prospective longitudinal study comprised 46 postmeningitic deaf children and 83 congenitally deaf children with age at implantation of < or = 5.6 years. Both groups were comparable with respect to educational setting and mode of communication and included children with additional disabilities., Results: Both postmeningitic and congenitally deaf children showed significant progress after implantation. Most (73% and 77%, respectively) could understand conversation without lip-reading or use the telephone with a known speaker 5 years after implantation, whereas none could do so before implantation. At the same interval, the postmeningitic and congenitally deaf children scored a mean open-set speech perception score of 47 (range: 0-91) and 46 (range: 0-107) words per minute, respectively, on connected discourse tracking. The respective mean scores at the 3-year interval were 22 and 29 correct words per minute, respectively. None of these children could score a single correct word per minute before implantation. The progress in both groups was statistically significant. When the 2 groups were compared, there was no statistically significant difference., Conclusion: Postmeningitic and congenitally deaf children showed significant improvement in their auditory receptive abilities at the 3- and 5-year intervals after cochlear implantation. There was no statistically significant difference between the outcomes of the 2 groups, suggesting that, provided that children receive an implant early, cause of deafness has little influence on outcome. Although the prevalence of other disabilities was similar in both groups, for individual children, their presence may have profound impact. The study supports the concept of implantation early in life, irrespective of the cause of deafness.

Background: Deaf children are heavily reliant on the sense of vision in order to develop efficient communication skills and explore the world around them. Any ophthalmic disorder may thus negatively impact on this process, especially if it is unrecognised in the early years of life. These disorders may be correctable (such as myopia) or treatable (such as cataract), and their early identification is of the utmost importance to optimise language development (spoken or sign, or both) and develop social cognition. Those children with non-correctable and non-treatable visual disorders, like retinitis pigmentosa in Usher syndrome, require multiple environmental adaptations and appropriate support services and information., Aim: : To review the accumulated scientific knowledge on ophthalmic disorders in deaf children and assess the quality of evidence published in the literature in order to contribute to better diagnosis and management of these conditions., Material and Methods: The project reviewed more than 1000 published papers and other sources. 191 papers complied with the aims of the study and were used in the project. From these studies, 95% were based on type III or IV evidence (mainly descriptive studies or case reports). Only 3% were based on type II evidence and 2% on type I evidence., Results-Conclusions: The main conclusions of this project are: a) the overall quality of evidence in the literature concerning deaf children and their ophthalmic problems is very low, b) the prevalence of ophthalmic problems in deaf children is very high (approximately 40% to 60%) and these problems may remain undetected for years although they may have a serious impact on children's acquisition of communication skills, c) screening for ophthalmic problems in deaf children should be encouraged and specialist ophthalmic examination should be carried out as soon as the diagnosis of deafness is confirmed irrespective of age, and may need to be repeated at intervals following diagnosis, d) families should be informed about the nature of the screening process in discussion with the relevant professionals and appropriate information should be available in a range of formats and in different community languages, e) professionals administering the tests should be familiar with the needs of deaf children with ophthalmic problems and should be sensitive to the communication needs of the child, especially undertaking behavioural testing where their collaboration is needed, f) while orthoptists can perform the majority of psychophysical tests (visual and stereo acuity tests, ocular motility tests, etc.) a comprehensive opthalmologic assessment by slit lamp biomicroscopy, streak retinoscopy, direct and indirect ophthalmoscopy, intraocular pressure measurement etc is required. Electrophysiologic testing to help identification of Usher syndrome may also be required, and finally g) serial hearing assessments of children with dual sensory deficits are needed to monitor hearing thresholds, to optimise hearing aid use and to ensure timely referral for cochlear implantation for those who need it.

Objectives: To assess a group of consecutively implanted children over 10 years after implantation with regard to implant device use and function, speech perception, and speech intelligibility outcomes; and to document current academic or occupational status., Study Design: A prospective longitudinal study assessing device function, device use, speech perception, speech intelligibility, and academic/occupational status of implanted deaf children., Setting: Pediatric tertiary referral center for cochlear implantation., Methods: The auditory performance and speech intelligibility development of 30 profoundly deaf children were rated before cochlear implantation and at 5 and 10 years after implantation using the Categories of Auditory Performance and the Speech Intelligibility Rating. The academic and/or occupational status of the participants after 10 years of implant experience was documented. All children received a Nucleus multichannel cochlear implant between the ages of 2.5 and 11 years (mean age at implantation, 5.2 yr). Implant experience ranged from 10 to 14 years of use., Results: After 10 years of implant experience, 26 subjects (87%) reported that they always wore their device; 2 subjects (7%), frequently; and 1 subject (3%), occasionally. Only one child had discontinued use of his device. After 10 years of implant use, 26 (87%) of the children understood a conversation without lip reading and 18 (60%) used the telephone with a familiar speaker. Ten years after implantation, 23 (77%) of the subjects used speech intelligible to an average listener or a listener with little experience of a deaf person's speech. One-third to one-half of the implanted children continued to demonstrate improvements at 5 to 10 years of implant use. Of the 30 implanted children, 8 (26.7%) experienced nine device failures. The length of time from identification of the first faulty electrode to reimplant surgery ranged from 2 weeks to 5.5 years, as several failures were gradual or intermittent. However, all children were successfully reimplanted. At the end of the study (10-14 yr after implantation), 19 subjects were in secondary school for children aged 11 to 16 years: 6 were in mainstream schools, 7 were in specialist hearing-impaired units attached to a mainstream secondary school, and 6 were in schools for the deaf. Of the remaining 11 subjects, 4 were in college studying vocational subjects, 2 were in a university studying for a bachelor's degree, 3 were working full-time, 1 was working and going to a university part-time, and 1 was a full-time mother of two young children., Conclusion: All but 1 of the 30 implanted children continue using their devices 10 to 14 years after implantation, showing significant progress in speech perception and production. Device failure was frequent, but successful reimplantation occurred in all cases. One-third to one-half of the implanted children in this study continued to demonstrate improvements at 5 to 10 years of implant use. All children are studying or working and are actively involved in their local communities. The results suggest that cochlear implantation provides long-term communication benefit to profoundly deaf children that does not plateau for some subjects even after reimplantation. This study further indicates that cochlear implant centers need the structure and funding to provide long-term support, counseling, audiologic follow-up, rehabilitation, and device monitoring to implanted children.

Objective: To assess performance of Nucleus 22 mini system pediatric users converted from the Spectra 22 body-worn to the ESPrit 22 ear-level speech processor using aided thresholds and speech discrimination measures before and after the conversion., Study Design: Spectra 22 body-worn speech processor users were chosen using preselection criteria (stable map, ability to report on the quality of the signal, no device problems). The subjects underwent tuning, map conversion, fitting of the ESPrit 22, and aided soundfield threshold and speech discrimination testing., Subjects: The first 100 consecutive conversions are analyzed in this study. Fifty children (50%) were female, and 50 (50%) were male. The average age at implantation was 4.6 years (median 4.3 years, range 1.7 to 11 years). The average age of fitting the ear level speech processor was 11.1 years (median 11 years, range 6.2 to 18.2 years)., Setting: Tertiary referral pediatric cochlear implant center in the United Kingdom., Results: Of the 100 fittings attempted, all Spectra 22 maps could to be converted for use in the ESPrit 22. Of these 100 fittings, 44 were straightforward with no adjustment to map parameters being required, and 56 needed rate reductions and other map adjustments to achieve the conversion. The difference of the mean thresholds before and after the conversion did not exceed 2 dB across the frequencies studied (0.5-4 kHz). In 95% of the cases, the differences were less than 9 dB(A). With regard to speech discrimination testing, the mean threshold before the conversion was 53.4 dB and after the conversion 52.7 dB. Of the 100 conversions, only five children stopped using the ESPrit 22 despite fitting being achieved., Conclusion: Conversion from the Spectra 22 body worn to the ESPrit 22 ear level speech processor was found to be feasible in all the 100 cases studied. Only a minority (5%) of children chose not to use the ear level speech processor suggesting that children and parents were satisfied from the conversion.

Objectives: The objectives of this study are to estimate time and out-of-pocket costs incurred by families attending a pediatric cochlear implant programme. In addition, qualitative data examine the intangible costs faced by families., Methods: Data was collected during semi-structured face-to-face interviews with parents of children with a cochlear implant attending a clinic visit at Nottingham Cochlear Implantation Programme (NPCIP), UK. Information supplied included socio-demographic characteristics, mode of travel, out-of-pocket expenses, time foregone, and details of companions. Quantitative data was stored and analysed in SPSS version 11.5., Results: Two hundred and sixteen face-to-face interviews were conducted with parents of children implanted for between 1 month and 13 years. Time and out-of-pocket costs were significantly higher for those in the first 2 years of the programme, mean UK pound 3090 per annum compared to UK pound 2159 per annum for those in years >2-5 and UK pound 1815 per annum for those in years >5 (P<0.001). The biggest component of this was time costs, although the sensitivity analysis revealed that these were also most variable depending on the methods used to estimate them. The largest out-of-pocket cost incurred by families was travel costs which accounted for 44%, although 16% of families received some financial help with travel costs. The qualitative findings are consistent with these findings, also showing that over time the financial and intangible costs incurred as a result of cochlear implantation decline., Conclusions: This study is the first to obtain primary data on the time and out-of-pocket costs incurred by families attending a pediatric cochlear implant programme in the UK. It finds that these costs are greatest for those families in the first 2 years of the programme and/or who live furthest from the programme.

Objective: To measure the intra- and interobserver error in size estimation of acoustic schwannomas from magnetic resonance imaging (MRI) scans by experienced radiologists to determine whether small amounts of tumor growth that may affect management (2 mm) could be reliably measured in clinical practice., Design: Duplicated, blinded size estimation of acoustic neuromas (according to American Academy of Otolaryngology-Head and Neck Surgery guidelines, 1995) from MRI scans of patients with acoustic neuromas., Setting: Tertiary referral teaching hospital and DGH., Participants: Four radiologists (including 2 dedicated neuroradiologists) measuring positive MRI scans of 26 patients with an acoustic neuroma., Main Outcome Measure: Intraradiologist and inter-radiologist repeatability coefficients in millimeters for the maximal tumor diameter in the anteroposterior (AP) axis, medial-longitudinal (ML) axis, and the square-root of the product of these two measurements. Repeatability coefficients give the 95% range within which the differences in repeated measurements lie., Results: The intraradiologist repeatability for AP and ML measurements ranged from 1.51 to 6.03 mm and 2.01 to 3.83 mm, respectively. The repeatability of the square-root of the product ranged from 1.43 to 4.94 mm. The inter-radiologist repeatability was 6.48 mm and 7.46 mm for the AP and ML measurements, respectively, giving a repeatability of 3.65 mm for the square-root of the product., Conclusion: The study indicates that, in routine clinical practice, differences in tumor size of the order of 2 mm cannot be reliably measured, even by the same radiologist. Thus, reported growth of acoustic tumors should be interpreted with caution, especially if this is the criterion for recommending treatment.

Background: During 2002, there was an increase in reports of bacterial meningitis among people with cochlear implants in Europe and North America. One model of implant, which included a space-occupying 'positioner', was recalled. Implantation of a positioner was shown to be a risk factor for meningitis among children in the United States. The situation in the United Kingdom was not known., Methods: We ascertained the cohort of people who had received cochlear implants with intra-cochlear electrodes in UK hospitals prior to 1 October 2002 and were permanently resident in the United Kingdom. We compared the incidence of meningitis, and the causes and incidence of death from all causes, between the cohort and reference populations., Results: Of 1851 children (66 with positioners), none had contracted meningitis. Neither the incidence rate of meningitis, nor the cumulative mortality from all causes, differed significantly between implanted children and values expected for the general population. Of 1779 adults (139 with positioners), five had contracted meningitis with three fatalities. No case of meningitis involved a positioner and four of the cases, including the fatalities, possessed risk factors unrelated to implantation. Although the incidence rate of meningitis was significantly higher in implanted adults than the general population, cumulative mortality from all causes was never higher, and was significantly lower at some time points after implantation., Conclusion: Specific evidence of the association between bacterial meningitis and implantation with a positioner that arose in the United States and mainland Europe during 2002 has not been found in the United Kingdom.

Objective: To report the short- and long-term complications encountered in a large number of consecutive children undergoing implantation in a single center. The current study also describes the management and sequelae of each complication., Study Design: Prospective study assessing the surgical findings and complications of deaf children undergoing implantation., Setting: Pediatric tertiary referral center for cochlear implantation., Patients: The present study includes 300 consecutive children undergoing implantation, with a mean age at implantation of 5.1 years, ranging from 1.3 to 16.9 years. Of these children, 196 (65%) had congenital deafness of unknown cause. The commonest known cause was meningitis (73 of 300 [24%]) followed by congenital cytomegalovirus infection (17 of 300 [6%]). Children have been followed up regularly after implantation, typically at yearly intervals after the first year. The mean duration of follow-up at the time of the study was 4 years (range, 0.1-14 yr)., Results: There were no major perioperative (within 1 d after surgery) or major early postoperative (within 1 wk after surgery) complications. In the same periods, there were 19 and 15 minor complications, respectively. These complications (e.g., eardrum perforation, hematoma, flap swelling, wound infection, temporary facial weakness) settled with conservative treatment or minor intervention. With regard to the late surgical complications (>1 wk after surgery), there were 7 major (e.g., severe flap infection requiring explantation, cholesteatoma, persistent eardrum perforation) and 14 minor complications (e.g., mild flap infection, flap swelling, hematoma). A number of complications were encountered even 14 years after the original operation, and some of them needed repeated interventions, highlighting the importance of long-term follow-up. However, most of the complications occurred very close to the surgical procedure (<1 yr)., Conclusion: An overall rate of 2.3% for major surgical complications and an overall rate of 16% for minor surgical complications suggest that cochlear implantation is a relatively safe surgical operation in experienced centers. Most surgical complications are minor and can be managed with conservative treatment or minor surgical intervention. However, meticulous attention to surgical detail, especially handling soft tissues and leaving the posterior canal wall intact, and long-term follow-up are of paramount importance in minimizing the incidence of surgical complications.