Follow-up of children diagnosed with deafness in a neonatal hearing screening program in Manaus / Seguimento de crianças com diagnóstico de surdez em programa de triagem auditiva neonatal em Manaus

ABSTRACT OBJECTIVE To evaluate the follow-up of children diagnosed with deafness in neonatal hearing screening and risk factors for hearing loss. METHODS Quantitative, cross-sectional, and retrospective study to evaluate factors associated with hearing loss and the follow-up of cases of children diagnosed with audiological dysfunction, by analyzing electronic medical records of 5,305 children referred to a Specialized Center in Type I Rehabilitation, from January/2016 to February/2020, in the city of Manaus, Amazonas. The statistical study used Pearson's chi-square test and binary logistic regression in which odds ratio scans were obtained with reliability intervals of 95%. RESULTS Of the 5,305 children referred for the otoacoustic emission retest, 366 (6.9%) failed the retest. Children diagnosed with sensorineural hearing loss continued in the study, totaling 265 (72.4%). Only 58 (21.9%) children continued in the study to its end, of these 39 had received hearing aids at that point; and 16 (41%) had surgical indication for cochlear implants, of which only 3 (18.7%) had undergone surgery. Among the risk factors for hearing loss, we found 2.6 times more chance of failure in the otoacoustic emissions retest in those children who had a family history of hearing loss and ICU stay. CONCLUSION Although the screening flow reaches a large part of live births, the dropout rates during the process are high, therefore, the socioeconomic and geographic characteristics of regions such as the Amazon should be considered as relevant factors to the evasion of rehabilitation programs of these children. Hospitalization in the neonatal ICU and family history of hearing loss in the investigations could be identified as the main and most important factors for alteration of the otoacoustic emissions retests.

RESUMO OBJETIVO Avaliar o seguimento das crianças com diagnóstico de surdez na triagem auditiva neonatal e fatores de risco para deficiência auditiva. MÉTODOS Estudo quantitativo, transversal e retrospectivo para avaliação de fatores associados à perda auditiva e o seguimento dos casos de crianças diagnosticadas com disfunção audiológica, por meio da análise de prontuários eletrônicos de 5.305 crianças encaminhadas a um Centro Especializado em Reabilitação Tipo I, no período de janeiro/2016 a fevereiro/2020, na cidade de Manaus, Amazonas. O estudo estatístico utilizou o teste qui-quadrado de Pearson e por regressão logística binária nos quais foram obtidos odds ratio com intervalos de confiabilidade de 95%. RESULTADOS Das 5.305 crianças encaminhadas para realização do reteste da orelhinha, 366 (6,9%) falharam no reteste. Prosseguiram no estudo as crianças com diagnóstico de perda auditiva neurossensorial, totalizando 265 (72,4%). Permanecendo, no final da pesquisa, apenas 58 (21,9%) crianças, destas 39 receberam aparelho auditivo até o presente estudo; e 16 (41%) já tinham indicação cirúrgica para implante coclear, sendo que apenas três (18,7%) haviam realizado a cirurgia. Dentre os fatores de risco para deficiência auditiva encontramos 2,6 vezes mais chance de falha no reteste da orelhinha naquelas crianças que tinham história familiar de perda auditiva e internação em UTI. CONCLUSÕES Embora o fluxo de triagem alcance boa parte dos nascidos vivos, as taxas de evasão durante o processo são altas, portanto, as características socioeconômicas e geográficas de regiões como a Amazônia devem ser consideradas como fatores relevantes à evasão dos programas de reabilitação dessas crianças. Foi possível identificar que a internação em UTI neonatal e o histórico familiar de perda auditiva presentes nas investigações compõem os principais e mais importantes fatores para alteração dos retestes da orelhinha.

Prevalence of cochlear implants in Europe: trend between 2010 and 2016.

Objective: To collect figures on the numbers of children and adults receiving cochlear implants across Europe, compare the figures for 2016 with those for 2010, and identify any trends. Materials and methods: In 2018 EURO-CIU invited their 23 member countries to conduct a survey collecting data on the number of CI recipients in 2016 and 2017. Data were received from 15 countries, representing more than 100 000 CI recipients in Europe. Results: For paediatric CI, there was an increase in nearly all European countries (except Denmark, the UK and Luxembourg) between 2010 and 2016. We found an annual figure of one CI per 1000 newborns common in most countries where reimbursement of paediatric CI's is available. Conversely the adult data reveals no increase between 2010 and 2016 and the data is less homogeneous than the paediatric data with huge differences across countries. Conclusion: There is little agreement on data on numbers of CI across Europe, which makes it difficult to plan public health policy, funding or services. In all European countries included in this study (except Germany) there needs to be work on raising more awareness of adult hearing loss and adult cochlear implantation to improve access.

Pediatric Minimum Speech Test Battery.

BACKGROUND: Assessment of patient outcomes and documentation of treatment efficacy serves as an essential component of (re)habilitative audiology; however, no standardized protocol exists for the assessment of speech perception abilities for children with hearing loss. This presents a significant challenge in tracking performance of children who utilize various hearing technologies for within-subjects assessment, between-subjects assessment, and even across different facilities. PURPOSE: The adoption and adherence to a standardized assessment protocol could help facilitate continuity of care, assist in clinical decision making, allow clinicians and researchers to define benchmarks for an aggregate clinical population, and in time, aid with patient counseling regarding expectations and predictions regarding longitudinal outcomes. DESIGN: The Pediatric Minimum Speech Test Battery (PMSTB) working group-comprised of clinicians, scientists, and industry representatives-commenced in 2012 and has worked collaboratively to construct the first PMSTB, which is described here. CONCLUSIONS: Implementation of the PMSTB in clinical practice and dissemination of associated data are both critical for achieving the next level of success for children with hearing loss and for elevating pediatric hearing health care ensuring evidence-based practice for (re)habilitative audiology.

Paediatric cochlear implantation: adverse prognostic factors and trends from a review of 174 cases.

OBJECTIVES: Identification and evaluation of prognostic factors that are associated with paediatric cochlear implantation (PCI) outcomes was the aim of this study. METHODS: A retrospective review of 174 charts was performed at the Royal Children's Hospital and the Hear and Say Centre, Brisbane. This examined the possible influence of a number of variables (including age at implant, family, additional disabilities, surgical complications, gender, GJB2 mutations, meningitis, inner ear malformations, and prematurity) on outcome measures: receptive, expressive, and total language, receptive and expressive vocabulary, speech articulation and categories of auditory performance at 18-24 months post-implant. Multiple regression analysis was used to identify variables related to language and vocabulary outcomes. RESULTS: The findings suggest that inner ear malformations and family concern are negatively associated with receptive and expressive language and receptive vocabulary scores. There was marginal evidence to suggest that increasing age at implantation was associated with lower receptive and expressive language scores. DISCUSSION: Prognostic factors that have been adequately validated statistically include inner ear malformations, the influence of family and late age at implantation. However, this study identified a need to define better the impact of the various degrees of inner ear malformations, to particularly emphasize the role of family as a strong predictor of PCI outcomes, and to confine the study of 'age at implantation' to pre-lingually deafened children. CONCLUSION: Evaluation of prognostic factors is a key element in PCI. This study confirmed several factors that are strongly associated with outcomes. For better research, there is a need for universal standardized outcome measures and development of a standardized framework for recording patient data.

Education of deaf and hard of hearing students and accountability reform: issues for the future.

The No Child Left Behind Act of 2001 (NCLB) has had a monumental impact over the past decade on how educational reform is viewed in the United States. As a result of how the law is structured, schools for students who are deaf or hard of hearing have been the focus of attention for educational reform under NCLB. While there have been some shifts in policy, reauthorization of NCLB will need to respond to some of the larger calls for changes to the law. The author discusses some key issues related to assessment and accountability that are central to how the reauthorization of NCLB affects the education of deaf and hard of hearing students.

[Present state of cochlear implant treatment in adults and children]. / Gegenwärtiger Stand der Kochleaimplantatversorgung bei Erwachsenen und Kindern.

The technique of cochlear implantation was the first method which allowed replacement of a sense organ by a (partially) implantable electronic prosthesis. By this method the cells of the spiral ganglion of the cochlea are directly stimulated by the electrodes introduced into the cochlea, bypassing the functions of the outer and middle ear as well as the cochlea. Treatment with a cochlear implant (CI) has been established over the last 20-30 years as a reliable method for restoring hearing in adults and children with severe hearing disorders and is now a routine method in many centers. For optimal results in understanding speech and for children in the speech development phase, rehabilitation must be an integral part of the total treatment and follow soon after implantation. To achieve this early diagnosis of hearing difficulties or deafness is necessary, which is simplified by a consistent hearing screening of newborns, now a statutory procedure in Germany.

[Audiologic rehabilitation of patients with cochlear implants]. / Audiologische Rehabilitation von Kochleaimplantat-Trägern.

The outcome and success of rehabilitation of patients with cochlear implants (CIs) is determined to a large extent by the preoperative diagnostics and individual fitting of the speech processor and is assessed by the auditory performance of the CI recipient. Due to the wide spectrum of auditory abilities of recipients and to the large variability with respect to their age and experience, the postoperative audiologic assessment is based on a large battery of subjective tests and objective measures. The results of these functional tests are used for documenting the level of rehabilitation, and they determine the actions taken for fine-tuning the fitting of the speech processor.

[(Re)habilitation after cochlear implantation]. / (Re)habilitation nach Versorgung mit einem Kochleaimplantat.

Over the last years, indications for cochlear implants (CIs) have changed dramatically. The benefits depend on the preconditions of the individual patient as well as on the subsequent (re)habilitation. Therefore, many variables influencing the hearing and speech perception of a CI user must be kept in mind. As an example, the special situation of children having Turkish as their mother tongue is described. The most convincing argument concerning (re)habilitation is its benefit. Indeed, this benefit represents the final standard of quality and serves as the yardstick for standard assessments of (re)habilitation quality. CI (re)habilitation includes medical, pedagogical, audiological, hearing and speech, and psychological therapeutic aspects.

[Hearing aid efficacy according to the data from the Tula regional Deaf and Dumb Centre].

Auditory inefficiency creates a serious social problem due to the ever increasing number of people with this pathology. The use of hearing aids constitutes the basis of rehabilitative measures for these patients. The authors present results of the analysis of hearing aid efficacy among adult subjects residing in the Tula region carried out in the framework of the relevant Federal program during the last 3 years. Criteria for the assessment of the efficacy of hearing aids are proposed and factors on which it depends are considered.

Maximizing cochlear implant patients' performance with advanced speech training procedures.

Advances in implant technology and speech processing have provided great benefit to many cochlear implant patients. However, some patients receive little benefit from the latest technology, even after many years' experience with the device. Moreover, even the best cochlear implant performers have great difficulty understanding speech in background noise, and music perception and appreciation remain major challenges. Recent studies have shown that targeted auditory training can significantly improve cochlear implant patients' speech recognition performance. Such benefits are not only observed in poorly performing patients, but also in good performers under difficult listening conditions (e.g., speech noise, telephone speech, music, etc.). Targeted auditory training has also been shown to enhance performance gains provided by new implant devices and/or speech processing strategies. These studies suggest that cochlear implantation alone may not fully meet the needs of many patients, and that additional auditory rehabilitation may be needed to maximize the benefits of the implant device. Continuing research will aid in the development of efficient and effective training protocols and materials, thereby minimizing the costs (in terms of time, effort and resources) associated with auditory rehabilitation while maximizing the benefits of cochlear implantation for all recipients.

[Protection and regeneration of sensory epithelia of the inner ear]. / Protektion und Regeneration sensorischer Epithelien des Innenohres.

Dysfunctions of the inner ear such as hearing impairment due to noise exposure or presbycusis and vertigo are often caused by loss of hair cells in the sensory epithelium. There is still no specific therapy, just technical aids. Options for protecting and regenerating hair cells are explained here. The inhibition of apoptosis via caspases is presently the main target of research. They are involved in damage caused by aminoglycosides, cisplatin, or noise exposure. Bcl-2, growth factors, and oxidative stress are discussed. In regeneration the transdifferentiation of supporting cells to hair cells is explained. This can be achieved with local gene therapy using math1. Approach and media for the application are discussed, while viral vectors such as the adenovector seem the most promising in research.

[Age connected hearing disorders (presbyacusis) as a social problem]. / Niedosluch zwiazany z wiekiem (presbyacusis) jako problem spoleczny.

The data of World Health Organization anticipate very rapid growth of percentage participation of elderly people in the population on the whole world. In Poland, in the year 2005, the percentage of people in the "post productive" age (women over 60 years, men over 65 years) was 15,32% of population, the expected data in 2020 is about 16%. The problem of this group becomes very essential social question. One of very important vital difficulties of that particular age is the problem with verbal communication, caused by the hearing disorders, very often stated in the older population. The hearing disorders (presbyacusis) are characterized by difficulties in speech understanding, the so called "social deafness", which often is a cause of psychical changes and isolation of persons with such hearing impairment. Because of its character, presbyacusis is very difficult in hearing rehabilitation. A new method of selection and fitting of hearing aids, adapted to the specificity of hearing disorders of older people is discussed.

[Mechanisms of age-related hearing loss and strategies for protection and restoration of hearing].

Hearing impairment is the most frequent disability in elderly individuals; however, we have limited options for treatment of age-associated hearing loss. To establish novel strategies for the treatment of age-associated hearing loss, it is crucial to elucidate the mechanisms of age-associated hearing loss. Studies on animal models and human temporal bones have indicated a close relationship between degeneration of the spiral ganglion neurons and stria vascularis and age-associated hearing loss. We have developed a drug delivery system using biodegradable gel for the inner ear to protect inner ears against aging. In addition, recent studies on cell therapy for the inner ear have suggested the efficacy of cell transplantation for restoration of hearing.

[Developments in the area of hearing aids]. / Ontwikkelingen op het gebied van hoorhulpmiddelen.

During the past 10-15 years, several techniques have been developed and refined for the treatment of hearing impairment and deafness. The size of hearing aids has been reduced and the sound processing has become more advanced. In the next 20 years sound processing will improve substantially. Although the reduction of size will not continue, the technical possibilities of small hearing aids will improve considerably. These developments will by and large remedy the present shortcomings of hearing aids. The functionality of cochlear implants has improved considerably in recent years, mainly due to improvements in sound processing and it is in this area in particular that improvements are expected during the next 20 years. In the area of bone conducting implants considerable improvements in the apparatus and operating techniques have been made. Minor developments are expected during the next 20 years. As a result of population growth and the aging of the population, the number of hearing aid users will probably multiply in the next 20 years from 380,000 to 523,000. The number of implant users will probably increase in this period. The bulk of the total costs for hearing devices will continue to be the supply of hearing aids.