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Neonates and infants are obligate nasal breathers and nasal obstruction in this age group can be a life threatening emergency. Even though the commonest cause for nasal obstruction is nasal oedema, bony stenosis of the posterior choanae or pyriform aperture is quite common and may be seen on CT scans. We describe a case of mid-nasal stenosis in a neonate and discuss the aetiology and management of nasal obstruction in this age group.

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OBJECTIVE: To determine durability of cochlear implant devices in a large paediatric cohort. DESIGN: Retrospective review of database records of children consecutively implanted between 1989 and March 2002. METHODS: The records of 363 children were studied. The review examined cases requiring explantation of the implant device for device failure with or without reimplantation. RESULTS: 15 failures were identified. The failure rates based on failures per number of implanted devices as well as cumulative user experience were 4.0% and 0.8% respectively. CONCLUSIONS: As implant programmes grow, so will the number of children requiring device explantation and reimplantation. This will have implications on implant programme development and resource allocation.

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OBJECTIVE: Tracheostomies in children are increasingly performed for chronic medical conditions. There are no published studies reporting the experience of children with a tracheostomy in school. Such information would be valuable in planning the care and education of these children. The aims of this study were to identify those children with a tracheostomy in Nottinghamshire schools and determine the support they were receiving. DESIGN, SETTING AND PARTICIPANTS: Questionnaire survey to families and school carers of 11 children with a tracheostomy. RESULTS: All children of school age were in full-time education (five mainstream, five special schools). One preschool child attended a Family Centre. Four had problems finding suitable carers, delaying return to school in three. Four parents were dissatisfied with aspects of the child's experience at school: two felt the teaching staff were unsupportive, one was unhappy with the care of the tracheostomy, and one had problems funding a carer. Ten out of 11 school carers were satisfied with their training; 10 would have liked regular update sessions. The amount of care required varied. Those with complex medical problems in a special school setting needed frequent care, and one had required admission to hospital from school. One child had time off school because of lack of carer availability. Those who were severely disabled had less time off school for ill health after the tracheostomy than before the tracheostomy. CONCLUSION: Children with tracheostomies can successfully and safely achieve full-time education in both mainstream and special schools. A dedicated multidisciplinary team, including input from the parents, is essential to achieve this goal. Regular revision of skills and information sessions for the teaching staff would be beneficial.

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The combined use of integrity testing (IT) and impedance telemetry (ImTe) intra-operatively is evaluated. One hundred and fifty children implanted with the Nucleus device were studied. In 81% of patients, normal results were obtained on all electrodes from both ImTe and IT. In seven cases where the back-up device was used, the intra-operative analysis of the device and subsequent postoperative quality assurance testing did not always correlate. In conclusion, intra-operatively, only ImTe is needed to verify the function of the implant if all impedance values are normal. However, in the case of abnormal ImTe results, additional IT data provide valuable assistance with the decision of whether to leave the implant in place or to use the backup device.

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Following the reports of a cluster of meningitis cases in recently implanted patients the FDA issued cautionary advice relating to the risk of meningitis after cochlear implantation (US Food and Drug Administration, 2002). Similar advice and a national reporting call has been issued by the Department of Health in the UK (Medical Devices Agency, 2002) and universal prophylactic pneumococcal vaccination started. We present a case of bilateral Mondini-type dysplasia associated with a defective stapes footplate and highlight the need for surgical vigilance to reduce the risks of meningitis from undiagnosed anatomical defects.

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The electrically evoked action potential (EAP) was recorded intra-operatively by use of neural response telemetry (NRT) on the Nucleus C124M cochlear implant. The aim of the present study was to investigate the EAP in young children immediately following implant surgery and whilst the children were still anaesthetized. The effect of data collection parameters on the reliability of the EAP was assessed and the relationships of the EAP findings to the intra-operative electrical auditory brainstem response (EABR) and early behavioural threshold levels (T-levels) were also investigated. The study data comprised intra-operative recordings in 60 children. Age at implantation was less than five years in 42 (70%) of the children. Aetiology of deafness was congenital in the majority of children (55, 92%), meningitic in four children and of unknown origin in one child. Optimum test parameters for the intra-operative EAP were an amplifier gain of 40 dB and a delay of 50 micros in order to minimize the effects of amplifier saturation due to stimulus artefact and to maximize the identification of the N1 component. An intra-operative protocol was established which involved recording four stimulus levels on each of the 22 electrodes of the electrode array, the range of stimulus levels being tailored towards the expected EAP thresholds and T-levels so as to identify response threshold. There was significant correlation between the intraoperative EAP thresholds and the early T-levels (Pearson's r = 0.93 ;p<0.01) when a correction factor was introduced based on a reliable behavioural measure of the threshold of electrical stimulation on electrode 10. The intra-operative EAP threshold, when combined with a limited amount of behavioural data, may therefore be used to predict the T-level with a useful degree of accuracy. This result is also supported by the significant correlation observed between the intra-operative thresholds of the EAP and EABR.

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Three groups of children undertook an interactive computer-based closed-set test of the ability to identify pre-recorded spoken words presented acoustically. The test was completed by 31/39 children with profound hearing loss who had used the Nucleus Spectra-22 cochlear-implant system for at least one year (Group A); by 30 children with normal hearing (Group B); and by 22 children with severe-profound hearing loss who used acoustic hearing aids (Group C). Among the implanted children, those who were younger when implanted and who had used their devices for longer produced higher scores (multiple-r = 0.68). Logistic regression functions were fitted to the data from Group B to describe the relationship between performance and age, and to the data from Group C to describe the relationships between performance and average hearing level (AHL) and aided threshold. By use of the regression equations, the performance of each implanted child was converted into a functionally equivalent (FE) age, an FE AHL and an FE aided threshold. Despite high variability leading to wide confidence intervals, these transformations showed that: (1) mean FE age (3.4 years) lagged mean chronological age (7.4 years), but some implanted children performed within the range expected for children with normal hearing of the same age; (2) mean FE AHL was 94 dB compared with a mean pre-implant AHL of 117 dB; (3) mean FE aided threshold was 45 dB(A) compared with a mean pre-implant aided threshold of 99 dB(A). These results confirm that implantation of appropriate candidates leads to functionally better hearing than would be expected with acoustic hearing aids. The results also demonstrate that many implanted children can participate in interactive tests with pre-recorded speech, thus providing robust data for comparison with future performance.

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OBJECTIVE: Children with Large Vestibular Aqueduct Syndrome (LVAS) frequently develop speech and language skills prior to deterioration of their hearing. Operations designed to halt the progression of hearing loss have largely failed so the question of Cochlear Implantation in these children has arisen. It had been suggested that there would be technical difficulties in implanting these patients and, therefore, there had been an initial reluctance to proceed to implantation. The aim of the present paper is to assess surgical and functional outcomes in implanted children with LVAS and review the related literature. MATERIAL AND METHODS: From the 170 children assessed by MRI in the Nottingham Paediatric Cochlear Implant Programme, seven (4%) were identified as having LVAS. Four of these children were implanted and had at least 12 months follow up. Two of the children are on the waiting list for implantation and one child was not implanted because of absence of the cochlear nerve. Operative findings, complications and outcome measures were recorded. The auditory skills of the children were assessed before implantation and 1 year following implantation. A literature search was done to identify other series with experience in implanting children with LVAS. RESULTS: Full insertion of the electrode array was achieved in all our cases. After cochleostomy two patients experienced a mild CSF leak that was easily controlled by the muscle graft. On the first day post-operation two patients were nauseous and one had an episode of vomiting, however, all were discharged within 24 h of surgery. Initial outcome measures at 12 months post-implantation were encouraging showing significant progress in children's auditory skills. CONCLUSIONS: The results of the present study and the review of the literature suggest that LVAS is not a contraindication to implantation as initial concerns about severe perilymph leaks and surgical complications have proved to be unfounded. The post-operative progress of these children in listening skills also suggest that these children are suitable for cochlear implantation

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The intra-operative electrical auditory brainstem response (EABR), electrical stapedius reflex threshold (ESRT) and the early post-operative behavioural threshold level (T-level) were recorded in five children undergoing cochlear re-implantation. The aim of the study was to assess objectively the effect of re-implantation on intra-operative objective measures and to investigate neuronal function. The children were aged between 2.06 years and 4.5 years at first implantation. Following failure of the first device, re-implantation was carried out 1.42-5.52 years later. Characteristics of the EABR and ESRT across the electrode array were typical of the expected pattern of responses on both occasions. In particular, the slopes of the amplitude input/output (I/O) functions for wave eV of the intraoperative EABR were similar for both the first and second implants even though absolute thresholds were generally elevated after re-implantation. This elevation in intra-operative threshold was more pronounced than the change in early post-operative behavioural threshold level for electrical stimulation (T-level). Our findings confirm a high level of neuronal survival after re-implantation. Threshold of the intra-operative EABR at the time of re-implantation greatly underestimates the sensitivity of the subsequent early post-operative T-levels.

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OBJECTIVE: To test the hypothesis that children with clear promontory electrically evoked auditory brain stem responses (prom-EABRs) would outperform, after cochlear implantation, children who had no prom-EABR preoperatively. DESIGN: A prospective study was undertaken on 47 implanted children assigning them to two groups (group A: 35 children with a clear wave e-V in the preoperative prom-EABR and group B: 12 children with no prom-EABR). Speech perception and speech intelligibility were assessed annually up to 3 yr after implantation with the IOWA sentence test (level A and level B), Connected Discourse Tracking, Categories of Auditory Performance, and Speech Intelligibility Rating. t-test and Mann-Whitney U test were used to compare the above outcome measures in the two groups. RESULTS: There was no statistically significant difference between the two groups on any of the outcome measures at any interval. Moreover, the small differences observed showed no consistent trend toward either group of children. Further analysis revealed that the outcomes have not been affected by possible confounding factors (age at implantation, duration of deafness, preoperative unaided pure-tone thresholds, and number of inserted electrodes). CONCLUSIONS: The results suggest that children with no prom-EABR performed at levels comparable with children who had clear promontory responses preoperatively. The prognostic value of prom-EABR is limited and absence of a prom-EABR is not, by itself, a contraindication for cochlear implantation. However, in selected cases (congenital malformations, cochlear nerve dysplasia or suspected aplasia, narrow internal auditory canal, etc.) the presence of a prom-EABR is a positive finding in the assessment of candidates for cochlear implantation as it confirms the existence of intact auditory neurones.

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Hand-written operation notes are often produced as evidence in medico-legal cases. Incomplete and illegible notes, along with the use of confusing abbreviations, are a common source of weakness in a surgeon's defence. An audit of 100 sets of operation notes was carried out in a single otolaryngology department. Notes were scrutinised for the accuracy of data, ward, department and name of surgeon, as well as for the inclusion of unacceptable abbreviations. Using an aide-memoire attached to the front of the operation sheet, the audit was repeated with identical criteria. The aide-memoire improved the standard of operation note with respect to all measured criteria. Clear identification of operating surgeon improved from 74% to 93%, and the avoidance of unacceptable abbreviations rose from 53% to 84%. We conclude that a simple aide-memoire attached to operation note sheets can significantly improve the quality of note-keeping and potentially avoid medico-legal problems.

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This study compares the functional integrity of the auditory pathways of congenitally deaf and postmeningitically deaf children. We used the electrical auditory brain stem response evoked by promontory stimulation to assess 49 profoundly deaf children before cochlear implantation. The age at implantation ranged from 21 months to 15 years (mean 4.5 years). The onset of deafness was either congenital or up to the age of 2 years (mean 5 months). The cause of deafness was meningitis in 19 children (39%) and congenital in 30 (61%). The number of children with identifiable waveform components (eV, eIII, and eII) was significantly greater in the congenitally deaf group. We also analyzed the amplitudes, the latencies, and 4 parameters of the amplitude input-output functions. All the statistically significant differences were in favor of better responses in the congenitally deaf children. These results suggest that the functional status of the peripheral neurons of the auditory pathways may be more intact in congenitally deaf children than in postmeningitic children.

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A patient is presented who had bilateral abductor vocal fold paralysis pathologically proven to be due to multiple system atrophy (MSA) in the absence of other neurological features. MSA is a degenerative neurological condition that includes olivopontocerebellar atrophy, Shy-Drager syndrome and striatonigral degeneration. The usual predominant features of MSA are cerebellar ataxia, autonomic dysfunction and Parkinsonism. Stridor is present in over one third of patients and has been reported previously as a presenting symptom in MSA: however previously reported patients have always gone on to develop other neurological symptoms. The usual investigations of bilateral abductor vocal fold paralysis caused by MSA will not reveal the pathological process and we believe that magnetic resonance imaging (MRI) of the medulla and brain stem and autonomic function tests are probably the investigations of choice. It is a worthwhile exercise attempting to identify MSA as the cause of stridor as the prognosis is good in the medium term if appropriate support is offered.

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A review of the surgical complications of the first 115 patients who received a cochlear implant in this program demonstrate a negligible complication rate in the perioperative period and a low complication rate in the long term. The surgical technique is described in detail and the reasons for this low complication rate are analysed.

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AIM OF STUDY: Assess the value of computed tomography (CT) in the evaluation of abnormalities in the cochlea and auditory pathways. MATERIAL AND METHODS: We used CT to evaluate 108 children before cochlear implantation surgery. Children's ages at implantation ranged from 21 months to 16 years (mean age, 5.4 years). The etiology of deafness was meningitis in 44 children (40.8%), congenital in 51 (47.2%), and other in 13 children (12%). RESULTS: Eighteen of the 108 (16.6%) children and 34% of the postmeningitic children were found to have at least partial obliteration of the cochlea. Two (2%) children had congenital malformations of the cochlea and 12 children (11.1%) had abnormalities in the brain CT-scan. CT diagnostic values in postmeningitic children regarding cochlear obliteration were accuracy, 75%; sensitivity, 62%; specificity, 82%; positive predictive value, 66.6%; and negative predictive value, 79.3%. In six (20.6%) of postmeningitic children with normal CT-scans, some scala tympani drillout was required. CONCLUSION: CT-scan is capable neither of predicting with certainty the presence of minor degrees of cochlear obliteration nor of specifically imaging either the auditory nerve or its central connections.

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OBJECTIVE: To introduce four comprehensive electrical auditory brain stem response (EABR) parameters that objectively measure the input-output function and may be the base of comparison in related studies. MATERIALS AND METHODS: In 53 children (106 ears), recordings of the EABR evoked by electrical stimulation at the promontory were made at the time of surgery after the child was anesthetized and before cochlear implantation. RESULTS: Of the 106 ears studied, 81 (76.4%) produced clearly defined responses. These responses were used to develop a package of four comprehensive EABR parameters (slope, maximal slope, relative growth rate, and maximal relative growth rate) that measure objectively the input-output function. The methods of calculation are described in detail. CONCLUSION: These parameters may help us to refine and make more consistent the subjective EABR evaluation. They will also enable a comparison of the results from different cochlear implant centers and promote the progress of related research.

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OBJECTIVE: To test the view that prelinguistic postmeningitic deaf (PMD) children outperform congenitally deaf children (CD) in the first year following cochlear implantation. STUDY DESIGN AND PATIENTS: We evaluated 85 children with ages (at implantation) ranging from 1.9 years to 13.5 years (mean age 5.4 years). The Listening Progress scale was used to assess the developing use of audition 3, 6, and 12 months after implantation. RESULTS: In contrast to previous reports, the PMD children achieved statistically significantly lower scores than CD children.

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