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Item does not contain fulltext, The cause of childhood hearing impairment (excluding infectious pathology of the middle ear) can be extrinsic (embryofoetopathy, meningitis, trauma, drug ototoxicity, noise trauma, etc [...].

Recent data collected on adult patients with vestibular loss (VL) tend to demonstrate possible cognitive impairments in visuospatial working memory, mental rotation, selective attention, and space orientation. However, the neuropsychological profile of children with VL remains largely under-investigated in the scientific literature. Although previous research has shown that children with VL may experience some degree of delayed motor development, it is not yet clear if VL could also lead to specific delayed cognitive development. In this study, we will present the development and validation of a new tablet-based computerized test battery (VSAD) that evaluates visuospatial working memory, mental rotation, selective attention, and space orientation abilities. Thirteen children with VL and 54 average-age matched healthy children performed the VSAD and classical paper-and-pencil neuropsychological tasks twice within a 1-month interval. Our results demonstrated a good concurrent validity with strong correlations between the visuospatial working memory, mental rotation, and space orientation tests of the VSAD and classical tasks. Test–retest reliability was also supported through good intra-class coefficients. However, the test of selective attention showed no concurrent validity with the matched classical task. The discriminant validity of the VSAD was partially supported for visuospatial working memory and mental rotation performance accuracy. The VSAD shows good concurrent validity and reliability for measuring visuospatial working memory, mental rotation, and space orientation in children with VL. Future studies are needed to extend discriminant validity with other populations., SCOPUS: ar.j, DecretOANoAutActif, info:eu-repo/semantics/published

Introduction: The Esteem is a totally implantable hearing system that uses piezoelectric technology. It is indicated in case of moderate to severe stable sensorineural hearing loss with a minimum discrimination score of 60% and a middle ear which is anatomically and functionally intact. Its two components (sensor and driver) are positioned and fixed in the mastoid cavity and coupled respectively to the incus body and capitulum with cement. Study Design: Retrospective study. Settings: Tertiary referral center. Materials and Methods: Pure-tone average (PTA) gain in the implanted ear was calculated at 1, 2, and 4 KHz, word recognition score (WRS) gain at 50 dB SPL, and average WRS (AWRS) gain at 40, 55, and 70 dB SPL. WRS was also evaluated in silence and noise. All patients were asked to fill in the abbreviated profile of hearing aid benefit (APHAB) questionnaire pre- and postoperatively as well as an Esteem questionnaire. Results: Thirteen patients received implants between May 2008 and April 2010. Five minor complications occurred (1 temporary partial facial palsy, 1 secondary healing difficulty, and 3 revision surgeries for poor and deteriorating functional results and progressive gain loss after use of a heart defibrillator). Two patients (15%) suffered major complications and their implants had to be removed 4 months postoperatively because of a Staphylococcus aureus wound infection. One patient underwent reimplantation 6 months later. Mean PTA gain was 25 ± 11 dB, mean WRS gain at 50 dB SPL was 64 ± 33%, and mean AWRS gain was 40 ± 20%. WRS in silence and with a signal-noise ratio of 10, 0, and -5 dB was 91 ± 11, 85 ± 14, 71 ± 19, and 64 ± 30%, respectively. The APHAB questionnaire revealed 84% of satisfaction improvement compared to the previous classic hearing aid. Conclusion: The totally implantable hearing device Esteem 2 can offer good functional and satisfaction results. Careful selection of patients is required, however, based on hearing tests, exclusion, SCOPUS: ar.j, info:eu-repo/semantics/published

Ménière's disease is an idiopathic inner ear disorder characterized by endolymphatic hydrops (ELH), vertigo attacks, sensorineural hearing loss, tinnitus and pressure sensation in the affected ear. The disorder has to be differentiated from known causes of ELH and from other disorders provoking similar symptoms. Ménière's disease can seriously affect quality of life, and medical support is often necessary on a life long basis. The treatment modalities are counselling, preventive measures and life style adaptation, drug therapy, tinnitus coping, provision of hearing devices, and sometimes surgery. We review the indications for these modalities, and the evidence level of their beneficial effect., SCOPUS: re.j, info:eu-repo/semantics/published

Introduction: Cleft lip with or without cleft palate (CL/P) is one of the most common birth defects in the world. Prevalence varies between populations, with an average of 1/700. CL/P has a major clinical impact, requiring surgical, dental, orthodontic, speech, hearing and psychological management throughout childhood. The aetiology of CL/P is mostly unknown, and it is thought that both genetic and environmental factors play a role. Several causative genes for inherited syndromic forms of CL/P have been identified, and some recent studies have shown that these genes also contribute to the occurrence of isolated forms. Van der Woude syndrome (VWS) is one of the best models for non-syndromic CLP. It is an autosomal dominant disorder characterised by the presence of pits on the lower lip in addition to CL/P. Pits are the only feature distinguishing VWS from isolated clefts. Interestingly, in numerous VWS patients, the lip pits are very small and not readily diagnosed, thus mimicking isolated CL/P. Mutations in the IRF6 gene were shown to be the major genetic cause of VWS.1 Results: We performed direct sequence analysis of IRF6 on samples from a large European cohort and identified mutations in 27 (80%) families. This shows that IRF6 is the major causative gene of VWS in Europe also. Moreover, it is the gene to study when a seemingly isolated CL/P patient has minor signs, such as lip pits, since the identification of a mutation in IRF6 is associated with an increase in the risk of having a child with CL/P from 4-6%, the risk of transmission of an isolated cleft, to 50%, the risk of transmission of a dominant Mendelian disorder like VWS. Moreover, we studied the association of isolated CL/P with the IRF6 locus using two variants in a set of 195 patients from Belgium. As in an American study, a clear association was observed. This suggests that IRF6 also contributes to the occurrence of sporadic, isolated CL/P, even if no mutation in the gene can be identified in such pat, SCOPUS: cp.j, info:eu-repo/semantics/published

Item does not contain fulltext, One hundred and forty-seven adult recipients of the Nucleus 24 cochlear implant system, from 13 different European countries, were tested using neural response telemetry to measure the electrically evoked compound action potential (ECAP), according to a standardised postoperative measurement procedure. Recordings were obtained in 96% of these subjects with this standardised procedure. The group results are presented in terms of peak amplitude and latency, slope of the amplitude growth function and ECAP threshold. The effects of aetiological factors and the duration of deafness on the ECAP were also studied. While large intersubject variability and intrasubject variability (across electrodes) were found, results fell within a consistent pattern and a normative range of peak amplitudes and latencies was established. The aetiological factors had little effect on the ECAP characteristics. However, age affected ECAP amplitude and slope of the amplitude growth function significantly; i.e., the amplitude is higher in the lowest age category (15-30 years). Principal component analysis of the ECAP thresholds shows that the thresholds across 5 electrodes can be described by two factors accounting for 92% of the total variance. The two factors represent the overall level of the threshold profiles ('shift') and their slopes across the electrode array ('tilt'). Correlation between these two factors and the same factors describing the T- and C-levels appeared to be moderate, in the range of 0.5-0.6.

One hundred and forty-seven adult recipients of the Nucleus 24 cochlear implant system, from 13 different European countries, were tested using neural response telemetry to measure the electrically evoked compound action potential (ECAP), according to a standardised postoperative measurement procedure. Recordings were obtained in 96% of these subjects with this standardised procedure. The group results are presented in terms of peak amplitude and latency, slope of the amplitude growth function and ECAP threshold. The effects of aetiological factors and the duration of deafness on the ECAP were also studied. While large intersubject variability and intrasubject variability (across electrodes) were found, results fell within a consistent pattern and a normative range of peak amplitudes and latencies was established. The aetiological factors had little effect on the ECAP characteristics. However, age affected ECAP amplitude and slope of the amplitude growth function significantly; i.e., the amplitude is higher in the lowest age category (15-30 years). Principal component analysis of the ECAP thresholds shows that the thresholds across 5 electrodes can be described by two factors accounting for 92% of the total variance. The two factors represent the overall level of the threshold profiles ('shift') and their slopes across the electrode array ('tilt'). Correlation between these two factors and the same factors describing the T- and C-levels appeared to be moderate, in the range of 0.5-0.6.

Guillain-Barré's Syndrome and hearing loss. A child presenting Guillain-Barré's syndrome, together with bilateral hearing loss is presented. The incidence of hypoacusis is unknown. The necessity of behavioural audiometry and electrophysiologic testing is stressed., SCOPUS: ar.j, info:eu-repo/semantics/published