Desenvolvimento de habilidades auditivas de crianças no primeiro ano após o implante coclear unilateral e bilateral / Development of children's auditory skills in the first year after unilateral and bilateral cochlear implant

RESUMO Objetivo identificar se as diferenças no desenvolvimento da aquisição das habilidades auditivas iniciais em crianças após o implante coclear, unilateral ou bilateral, podem ser evidenciadas durante o primeiro ano de uso. Métodos estudo longitudinal retrospectivo de levantamento de prontuários. Foram incluídas crianças que receberam o implante coclear antes dos 4 anos de idade, separadas em dois grupos. O primeiro, composto por crianças implantadas unilateralmente e o segundo, por crianças implantadas bilateral e simultaneamente. Foram coletados os dados referentes à escala IT-MAIS (Infant-Toddler Meaningful Auditory Integration Scale) antes da cirurgia e três meses, seis meses e um ano após a cirurgia. Esses resultados foram comparados com os marcadores clínicos já existentes. Além disso, as famílias foram classificadas segundo a Escala de Avaliação de Envolvimento Familiar. Resultados foram avaliados os resultados de 29 crianças com implante coclear bilateral e 30 crianças com implante coclear unilateral. A pontuação obtida no IT-MAIS dos dois grupos foi semelhante aos marcadores clínicos. Quanto à Escala de Avaliação de Envolvimento Familiar, houve diferença, mostrando que as famílias do grupo dos bilaterais estavam mais envolvidas. Conclusão no primeiro ano de uso do implante coclear, o desenvolvimento da aquisição das habilidades auditivas iniciais das crianças implantadas bilateralmente não diferiu do desenvolvimento de crianças implantadas unilateralmente, sugerindo que as habilidades auditivas iniciais são passíveis de desenvolvimento com entrada auditiva unilateral.

ABSTRACT Purpose To identify the development of initial auditory skills in children with unilateral and bilateral cochlear implant during the first year of use. Methods Retrospective longitudinal study of medical records of children who received cochlear implant under the age of four, separated into two groups. The first, composed of children implanted unilaterally and the second, by children implanted and simultaneously. Data on the IT-MAIS scale (Infant-Toddler Meaningful Auditory Integration Scale) were collected before surgery and three months, six months and one year after surgery. These results were compared with the existing Clinical Markers. In addition, the family's classification was noted, according to the Family Involvement Assessment Scale. Results The data of 29 children with bilateral cochlear implant and 30 children with unilateral cochlear implant were evaluated. The IT-MAIS score of the two groups was similar to the Clinical Markers. Regarding the Family Involvement Assessment Scale, there was a difference, showing that families in the bilateral group were more involved. Conclusion In the first year of use of the cochlear implant, the development of children implanted bilaterally did not differ from the development of children implanted unilaterally, suggesting that initial auditory skills are likely to develop with unilateral auditory input.

A tecnologia a favor da educação continuada no implante coclear / Technology in favor of continuous professional development in cochlear implant

RESUMO Objetivo avaliar a teleconsulta de forma síncrona no mapeamento do implante coclear (IC) como ferramenta de educação continuada para fonoaudiólogos em capacitação e treinamento. Métodos estudo prospectivo longitudinal, na modalidade de preceptorado clínico, com teleconsulta de forma síncrona direcionada ao atendimento de mapeamento dos usuários de IC. A educação continuada foi oferecida aos fonoaudiólogos aprimorandos em um hospital e o fonoaudiólogo tutor, em unidade remota. A plataforma utilizada permitiu ao tutor visualizar e realizar intervenções na tela de mapeamento do IC. Foram aplicados três questionários abrangendo os marcadores de ensino; qualidade e importância da teleconsulta; interação entre os profissionais e autoavaliação sobre a confiança na realização das etapas do mapeamento antes e após as teleconsultas. Resultados participaram sete fonoaudiólogos e foram analisados 268 atendimentos, ao longo de três anos. Os marcadores clínicos mostraram, na média e individualmente, que houve correlação estatística com diminuição do número de intervenções totais, nas intervenções do mapeamento do IC e raciocínio clínico, no decorrer das sessões. A média das notas nas avaliações da importância da teleconsulta foi 9,7 e a interação com o tutor, 9,3. Na autoavaliação, todos os aprimorandos responderam que estavam "nada/pouco confiantes" para as etapas do mapeamento antes da intervenção e, ao final do curso, responderam "moderadamente/muito confiantes" para as mesmas etapas. Conclusão o uso da teleconsulta síncrona foi viável e eficiente como ferramenta de ensino para fonoaudiólogos. Foi possível observar a diminuição do número de intervenções ao longo do tempo, evidenciando a curva de aprendizado.

ABSTRACT Purpose To evaluate the teleconsultation synchronously in the mapping of the cochlear implant (CI) as an ongoing professional development tool in training programmes for audiologists. Methods It was a prospective longitudinal study, in the form of clinical preceptor with teleconsultation in a synchronous way directed at assisting the mapping of CI users. Distance learning was offered to fellow audiologists in a hospital and the audiology tutor in the remote unit. The chosen platform allowed the tutor to view and perform interventions on the CI mapping screen. Three questionnaires covering teaching markers were applied; the quality and importance of teleconsultation; interaction between professionals; and self-assessment of confidence in carrying out the mapping steps before and after teleconsultations. Results Seven audiologists participated; 268 consultations were analyzed over three years. The clinical markers showed, on average and individually, that there is a statistical correlation with a decrease in the number of total interventions, in the interventions of CI mapping and clinical reasoning throughout the sessions. The average of the scores of the importance of teleconsultation was 9.7 and the interaction with the tutor was 9.3. In the self-assessment, all audiologists responded that they were "not at all/not very confident" for the mapping steps before the intervention, and at the end of the course they responded "moderately/very confident" for the same steps. Conclusion The use of synchronous teleconsultation was feasible and efficient as a teaching tool for audiologists. It was possible to observe the decrease in the number of interventions over time, evidencing the learning curve.

Speech Perception Changes in the Acoustically Aided, Nonimplanted Ear after Cochlear Implantation: A Multicenter Study.

In recent years there has been an increasing percentage of cochlear implant (CI) users who have usable residual hearing in the contralateral, nonimplanted ear, typically aided by acoustic amplification. This raises the issue of the extent to which the signal presented through the cochlear implant may influence how listeners process information in the acoustically stimulated ear. This multicenter retrospective study examined pre- to postoperative changes in speech perception in the nonimplanted ear, the implanted ear, and both together. Results in the latter two conditions showed the expected increases, but speech perception in the nonimplanted ear showed a modest yet meaningful decrease that could not be completely explained by changes in unaided thresholds, hearing aid malfunction, or several other demographic variables. Decreases in speech perception in the nonimplanted ear were more likely in individuals who had better levels of speech perception in the implanted ear, and in those who had better speech perception in the implanted than in the nonimplanted ear. This raises the possibility that, in some cases, bimodal listeners may rely on the higher quality signal provided by the implant and may disregard or even neglect the input provided by the nonimplanted ear.

Benefit of Cochlear Implantation in Children with Multiple-handicaps: Parent's Perspective

Abstract Introduction The cochlear implants centers in the world are studying the cochlear implantation in children with multiple handicaps. Objective To develop a questionnaire to analyze the subjective benefits of the cochlear implantation in multiple handicapped children according to their parent's perspective. Methods A questionnaire was applied to 14 families of multiple handicapped children, aging from 2-12 years old and having from 11 months to 11 years of implant use. Results The social-emotional abilities were improved because of many factors, such as: auditory exposure, which happened in 84% of the children; recognition of their own names, which increased in 56%; and development of eye contact, in 28% of the subjects. Other benefits appeared to be: music appreciation and more attention and adherence to other therapies and school activities. Besides, some children became interested in objects, playing with other children, and more adapted to daily routines. Thirty-five percent of the children acquired oral language, mainly the bilaterally implanted, while 14% of them were engaged in sign language. Although all of the children showed a significant improvement in communication, the emotional issues of some families and the severity of the handicaps negatively impacted the outcomes. In spite of the families' acknowledgement of some benefits, the diagnosis of autism spectrum disorder caused frustration, requiring a readjustment of the expectations. Conclusion The questionnaire turned out to be an adequate tool to reveal the social-emotional benefits of cochlear implantation. Although oral language was not the major outcome in these cases, the cochlear implant benefits involved the whole family. All of the families recommended the implant to other children in a similar situation.

Benefit of Cochlear Implantation in Children with Multiple-handicaps: Parent's Perspective.

Introduction The cochlear implants centers in the world are studying the cochlear implantation in children with multiple handicaps. Objective To develop a questionnaire to analyze the subjective benefits of the cochlear implantation in multiple handicapped children according to their parent's perspective. Methods A questionnaire was applied to 14 families of multiple handicapped children, aging from 2-12 years old and having from 11 months to 11 years of implant use. Results The social-emotional abilities were improved because of many factors, such as: auditory exposure, which happened in 84% of the children; recognition of their own names, which increased in 56%; and development of eye contact, in 28% of the subjects. Other benefits appeared to be: music appreciation and more attention and adherence to other therapies and school activities. Besides, some children became interested in objects, playing with other children, and more adapted to daily routines. Thirty-five percent of the children acquired oral language, mainly the bilaterally implanted, while 14% of them were engaged in sign language. Although all of the children showed a significant improvement in communication, the emotional issues of some families and the severity of the handicaps negatively impacted the outcomes. In spite of the families' acknowledgement of some benefits, the diagnosis of autism spectrum disorder caused frustration, requiring a readjustment of the expectations. Conclusion The questionnaire turned out to be an adequate tool to reveal the social-emotional benefits of cochlear implantation. Although oral language was not the major outcome in these cases, the cochlear implant benefits involved the whole family. All of the families recommended the implant to other children in a similar situation.

Vocabulário expressivo em crianças usuárias de implante coclear / Expressive vocabulary in children with cochlear implants

RESUMO Objetivo: verificar a aquisição de vocabulário em crianças surdas, usuárias de implante coclear, bem como os fatores que influenciam esse desenvolvimento. Métodos: foi aplicada a parte de vocabulário do teste de linguagem infantil ABFW em 20 crianças usuárias de implante coclear por no mínimo três anos. Além disso, foi avaliada a participação familiar no desenvolvimento dessas crianças. Resultados: foi observado que as crianças implantadas apresentam possibilidade de alcançar o desenvolvimento normal de vocabulário, quando comparadas às crianças ouvintes, a depender de diversos fatores. O fator que apresentou influência estatisticamente significante no vocabulário foi a participação familiar, sendo que quanto maior o envolvimento da família no processo terapêutico, melhores os resultados no teste de vocabulário. Conclusão: as crianças implantadas podem apresentar desempenho similar às crianças ouvintes no teste de vocabulário, a depender das variáveis que transcendem a idade à implantação ou mesmo o tempo de uso do implante coclear. A estimulação/ participação familiar no desenvolvimento das crianças se mostrou de extrema importância no desenvolvimento da linguagem oral.

ABSTRACT Objective: to verify whether children with cochlear implants (CI) acquire vocabulary at the same pace as normally hearing children, and which factors influence their acquisition. Methods: the vocabulary test of the ABFW was performed on 20 children who had been using the cochlear implant for at least three years. Historical information, such as age at time of implant, hearing age (i.e., at time of implant use), and family participation in the rehabilitation process, was gathered from patients' files. Correlation statistical analysis was then performed. Results: it was observed that children with CI may acquire vocabulary similar to that of hearing children, depending on many aspects. The age at time of implantation and hearing age did not significantly correlate to the vocabulary results. The factor that demonstrated statistical significance was family participation, which showed a positive correlation: the more the family was involved in the rehabilitation process, the better the children's results on the vocabulary test. Conclusion: it was, thus, possible to conclude that children with CI develop their vocabulary in a similar manner as hearing children, depending on factors that transcend the child's age at time of implantation and hearing age. Family participation, in the rehabilitation process, was shown to be of critical importance in the child's vocabulary development.

Satisfaction and quality of life in users of auditory brainstem implant. / Satisfação e qualidade de vida em usuários de implante auditivo de tronco cerebral.

Purpose To evaluate satisfaction and quality of life of users of Auditory Implant Brainstem. Methods This is a cross-sectional and descriptive study conducted at Divisão de Clínica Otorrinolaringológica of Hospital das Clínicas of Faculdade de Medicina da Universidade de São Paulo, Brazil. For the research, 19 users of an Auditory Brainstem Implant answered the following questionnaires: KINDLR (Questionnaire for Measuring Health-Related Quality of Life in Children and Adolescents), for children and adolescents, their parents and/or caregivers; WHOQOL-BREF questionnaire, for adult participants; and the Satisfaction with Amplification in Daily Life (SADL) questionnaire culturally adapted to Brazilian Portuguese. Results The quality of life of children using Auditory Brainstem Implant from the perspective of their parents showed global results above average, as for most domains, except for the emotional well-being domain. Adults showed results above average for all domains. Regarding satisfaction with the device, the adult users of auditory brainstem implant were satisfied in general, except with regard to personal image. The parents of the children showed dissatisfaction in all subscales, except for the subscale of services and cost. Conclusion The results indicated that although patients are dissatisfied with the device in some aspects, overall the quality of life was rated as good for most of the aspects assessed.

Satisfação e qualidade de vida em usuários de implante auditivo de tronco cerebral / Satisfaction and quality of life in users of auditory brainstem implant

RESUMO Objetivo Avaliar a satisfação e qualidade de vida em usuários de Implante Auditivo de Tronco Cerebral. Método Trata-se de um estudo transversal e descritivo realizado na Divisão de Clínica Otorrinolaringológica do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, Brasil. Para a realização da pesquisa, 19 usuários de implante auditivo de tronco cerebral responderam aos seguintes questionários: KINDLR (Questionnaire for Measuring Health-Related Quality of Life in Children and Adolescents) para crianças e adolescentes, pais e/ou cuidadores; o questionário WHOQOL-BREF para os participantes adultos; e o questionário SADL (Satisfaction with Amplification in Daily Life), adaptado culturalmente para o português brasileiro. Resultados A qualidade de vida das crianças usuárias de implante auditivo de tronco cerebral do ponto de vista dos pais apresentou resultados acima da média para o resultado global e para a maioria dos domínios, exceto o domínio de bem-estar emocional. Os adultos apresentaram resultados acima da média para todos os domínios. Com relação à satisfação com o dispositivo, os adultos usuários de implante auditivo de tronco cerebral estavam satisfeitos de modo geral, exceto no que diz respeito à imagem pessoal, e os pais das crianças mostraram insatisfação para todas as subescalas, exceto para a subescala de serviços e custos. Conclusão Os resultados indicaram que, apesar de os pacientes estarem insatisfeitos em alguns aspectos com o dispositivo, de modo geral, a qualidade de vida foi classificada como boa, para a maioria dos aspectos avaliados.

ABSTRACT Purpose To evaluate satisfaction and quality of life of users of Auditory Implant Brainstem. Methods This is a cross-sectional and descriptive study conducted at Divisão de Clínica Otorrinolaringológica of Hospital das Clínicas of Faculdade de Medicina da Universidade de São Paulo, Brazil. For the research, 19 users of an Auditory Brainstem Implant answered the following questionnaires: KINDLR (Questionnaire for Measuring Health-Related Quality of Life in Children and Adolescents), for children and adolescents, their parents and/or caregivers; WHOQOL-BREF questionnaire, for adult participants; and the Satisfaction with Amplification in Daily Life (SADL) questionnaire culturally adapted to Brazilian Portuguese. Results The quality of life of children using Auditory Brainstem Implant from the perspective of their parents showed global results above average, as for most domains, except for the emotional well-being domain. Adults showed results above average for all domains. Regarding satisfaction with the device, the adult users of auditory brainstem implant were satisfied in general, except with regard to personal image. The parents of the children showed dissatisfaction in all subscales, except for the subscale of services and cost. Conclusion The results indicated that although patients are dissatisfied with the device in some aspects, overall the quality of life was rated as good for most of the aspects assessed.

Residual Hearing Preservation with the Evo® Cochlear Implant Electrode Array: Preliminary Results

Abstract Introduction The preservation of residual hearing is currently an important challenge for cochlear implant surgeries. Indeed, if patients exhibit functional hearing after cochlear implantation, they can benefit from the combination of acoustical stimulation, usually in the low-frequencies and electrical stimulation in the high-frequencies. This combined mode of stimulation has proven to be beneficial both in terms of speech perception and of sound quality. Finding the right procedures for conducting softsurgeries and designing electrode arrays dedicated to hearing preservation is an open issue. Objective The objective of this study is to evaluate the combination of a soft-surgery procedure implicating round-window insertion and the use of dexamethasone and hyaluronic acid during surgery, with the use of a specifically designed straight soft electrode array, on hearing preservation in patients with functional hearing in the low frequencies. Methods This pre-clinical trial was conducted on seven patients with residual hearing in the low frequencies. The surgical method used employed a round window insertion and the use of topical dexamethasone. Results The soft-surgery protocol could be successfully followed in five patients. In this group, the average hearing threshold shift compared with pre-operative values was of 18.7 þ/ 16.1 dB HL up to 500 Hz and 15.7 þ/ 15.1 up to 1 kHz, demonstrating satisfying levels of hearing preservation. Conclusion We were able to demonstrate the possibility of preserving residual hearing in most of the patients using the EVO electrode. Significant residual hearing preservation levels were was obtained when a soft surgical approach involving round window insertion, dexamethasone and hyaluronic use during the surgery.

Residual Hearing Preservation with the Evo® Cochlear Implant Electrode Array: Preliminary Results.

Introduction The preservation of residual hearing is currently an important challenge for cochlear implant surgeries. Indeed, if patients exhibit functional hearing after cochlear implantation, they can benefit from the combination of acoustical stimulation, usually in the low-frequencies and electrical stimulation in the high-frequencies. This combined mode of stimulation has proven to be beneficial both in terms of speech perception and of sound quality. Finding the right procedures for conducting soft-surgeries and designing electrode arrays dedicated to hearing preservation is an open issue. Objective The objective of this study is to evaluate the combination of a soft-surgery procedure implicating round-window insertion and the use of dexamethasone and hyaluronic acid during surgery, with the use of a specifically designed straight soft electrode array, on hearing preservation in patients with functional hearing in the low frequencies. Methods This pre-clinical trial was conducted on seven patients with residual hearing in the low frequencies. The surgical method used employed a round window insertion and the use of topical dexamethasone. Results The soft-surgery protocol could be successfully followed in five patients. In this group, the average hearing threshold shift compared with pre-operative values was of 18.7 +/- 16.1 dB HL up to 500 Hz and 15.7 +/- 15.1 up to 1 kHz, demonstrating satisfying levels of hearing preservation. Conclusion We were able to demonstrate the possibility of preserving residual hearing in most of the patients using the EVO electrode. Significant residual hearing preservation levels were was obtained when a soft surgical approach involving round window insertion, dexamethasone and hyaluronic use during the surgery.

Minimally Invasive Surgery for Intracochlear Schwannoma Removal and Simultaneous Cochlear Implantation

Abstract Introduction Hearing preservation has not yet been reported in patients undergoing resection of intracochlear schwannomas. This study describes a minimally invasive procedure for intracochlear schwannoma resection with simultaneous cochlear implantation that resulted in good hearing. Objective This study aims to describe a minimally invasive procedure for intracochlear schwannoma resection with simultaneous cochlear implantation. Data Synthesis The technique described in this study was developed for a 55-year-old male with a 20-year history of bilateral progressive hearing loss and tinnitus that had a mass in the left apical turn of the cochleameasuring 0.3 cm. Surgery accessed the apical turn of the cochlea. We performed mastoidectomy and posterior tympanotomy and removed incus and tensor tympani muscle to expose the cochlear apex. The tumor was identified and completely resected. After the cochleawas anatomically preserved, it was implanted with a straight electrode via round window insertion. The histopathological examination confirmed intracochlear schwannoma. Speech perception test revealed 100% speech recognition with closed sentences and the average audiometric threshold (500 to 2000 Hz) was 23 dB. Conclusion Our technique led to rehabilitation of the patient and improved hearing without damaging the intracochlear structure, making it possible to perform CI in the same procedure with good results.

Minimally Invasive Surgery for Intracochlear Schwannoma Removal and Simultaneous Cochlear Implantation.

INTRODUCTION: Hearing preservation has not yet been reported in patients undergoing resection of intracochlear schwannomas. This study describes a minimally invasive procedure for intracochlear schwannoma resection with simultaneous cochlear implantation that resulted in good hearing. OBJECTIVE: This study aims to describe a minimally invasive procedure for intracochlear schwannoma resection with simultaneous cochlear implantation. DATA SYNTHESIS: The technique described in this study was developed for a 55-year-old male with a 20-year history of bilateral progressive hearing loss and tinnitus that had a mass in the left apical turn of the cochlea measuring 0.3 cm. Surgery accessed the apical turn of the cochlea. We performed mastoidectomy and posterior tympanotomy and removed incus and tensor tympani muscle to expose the cochlear apex. The tumor was identified and completely resected. After the cochlea was anatomically preserved, it was implanted with a straight electrode via round window insertion. The histopathological examination confirmed intracochlear schwannoma. Speech perception test revealed 100% speech recognition with closed sentences and the average audiometric threshold (500 to 2000 Hz) was 23 dB. CONCLUSION: Our technique led to rehabilitation of the patient and improved hearing without damaging the intracochlear structure, making it possible to perform CI in the same procedure with good results.

Audiological outcomes of cochlear implantation in Waardenburg Syndrome

The most relevant clinical symptom in Waardenburg syndrome is profound bilateral sensorioneural hearing loss. AIM: To characterize and describe hearing outcomes after cochlear implantation in patients with Waardenburg syndrome to improve preoperative expectations. METHOD: This was an observational and retrospective study of a series of cases. Children who were diagnosed with Waardenburg syndrome and who received a multichannel cochlear implant between March 1999 and July 2012 were included in the study. Intraoperative neural response telemetry, hearing evaluation, speech perception, and speech production data before and after surgery were assessed. RESULTS: During this period, 806 patients received a cochlear implant and 10 of these (1.2%) were diagnosed with Waardenburg syndrome. Eight of the children received a Nucleus 24® implant and 1 child and 1 adult received a DigiSonic SP implant. The mean age at implantation was 44 months among the children. The average duration of use of a cochlear implant at the time of the study was 43 months. Intraoperative neural responses were present in all cases. Patients who could use the speech processor effectively had a pure tone average of 31 dB in free-field conditions. In addition, the MUSS and MAIS questionnaires revealed improvements in speech perception and production. Four patients did not have a good outcome, which might have been associated with ineffective use of the speech processor. CONCLUSION: Despite the heterogeneity of the group, patients with Waardenburg syndrome who received cochlear implants were found to have hearing thresholds that allowed access to speech sounds. However, patients who received early intervention and rehabilitation showed better evolution of auditory perception...

Converted and upgraded maps programmed in the newer speech processor for the first generation of multichannel cochlear implant.

OBJECTIVE: To identify the technological contributions of the newer version of speech processor to the first generation of multichannel cochlear implant and the satisfaction of users of the new technology. Among the new features available, we focused on the effect of the frequency allocation table, the T-SPL and C-SPL, and the preprocessing gain adjustments (adaptive dynamic range optimization). STUDY DESIGN: Prospective exploratory study. SETTING: Cochlear implant center at hospital. PATIENTS: Cochlear implant users of the Spectra processor with speech recognition in closed set. Seventeen patients were selected between the ages of 15 and 82 and deployed for more than 8 years. INTERVENTIONS: The technology update of the speech processor for the Nucleus 22. MAIN OUTCOME MEASURES: To determine Freedom's contribution, thresholds and speech perception tests were performed with the last map used with the Spectra and the maps created for Freedom. To identify the effect of the frequency allocation table, both upgraded and converted maps were programmed. One map was programmed with 25 dB T-SPL and 65 dB C-SPL and the other map with adaptive dynamic range optimization. To assess satisfaction, SADL and APHAB were used. RESULTS: All speech perception tests and all sound field thresholds were statistically better with the new speech processor; 64.7% of patients preferred maintaining the same frequency table that was suggested for the older processor. The sound field threshold was statistically significant at 500, 1,000, 1,500, and 2,000 Hz with 25 dB T-SPL/65 dB C-SPL. Regarding patient's satisfaction, there was a statistically significant improvement, only in the subscale of speech in noise abilities and phone use. CONCLUSION: The new technology improved the performance of patients with the first generation of multichannel cochlear implant.

Audiological outcomes of cochlear implantation in Waardenburg Syndrome.

INTRODUCTION: The most relevant clinical symptom in Waardenburg syndrome is profound bilateral sensorioneural hearing loss. AIM: To characterize and describe hearing outcomes after cochlear implantation in patients with Waardenburg syndrome to improve preoperative expectations. METHOD: This was an observational and retrospective study of a series of cases. Children who were diagnosed with Waardenburg syndrome and who received a multichannel cochlear implant between March 1999 and July 2012 were included in the study. Intraoperative neural response telemetry, hearing evaluation, speech perception, and speech production data before and after surgery were assessed. RESULTS: During this period, 806 patients received a cochlear implant and 10 of these (1.2%) were diagnosed with Waardenburg syndrome. Eight of the children received a Nucleus 24(®) implant and 1 child and 1 adult received a DigiSonic SP implant. The mean age at implantation was 44 months among the children. The average duration of use of a cochlear implant at the time of the study was 43 months. Intraoperative neural responses were present in all cases. Patients who could use the speech processor effectively had a pure tone average of 31 dB in free-field conditions. In addition, the MUSS and MAIS questionnaires revealed improvements in speech perception and production. Four patients did not have a good outcome, which might have been associated with ineffective use of the speech processor. CONCLUSION: Despite the heterogeneity of the group, patients with Waardenburg syndrome who received cochlear implants were found to have hearing thresholds that allowed access to speech sounds. However, patients who received early intervention and rehabilitation showed better evolution of auditory perception.

Result on speech perception after conversion from Spectra® to Freedom®.

UNLABELLED: New technology in the Freedom® speech processor for cochlear implants was developed to improve how incoming acoustic sound is processed; this applies not only for new users, but also for previous generations of cochlear implants. AIM: To identify the contribution of this technology-- the Nucleus 22®--on speech perception tests in silence and in noise, and on audiometric thresholds. METHODS: A cross-sectional cohort study was undertaken. Seventeen patients were selected. The last map based on the Spectra® was revised and optimized before starting the tests. Troubleshooting was used to identify malfunction. To identify the contribution of the Freedom® technology for the Nucleus22®, auditory thresholds and speech perception tests were performed in free field in sound-proof booths. Recorded monosyllables and sentences in silence and in noise (SNR = 0dB) were presented at 60 dBSPL. The nonparametric Wilcoxon test for paired data was used to compare groups. RESULTS: Freedom® applied for the Nucleus22® showed a statistically significant difference in all speech perception tests and audiometric thresholds. CONCLUSION: The Freedom® technology improved the performance of speech perception and audiometric thresholds of patients with Nucleus 22®.

Research ethics in animal models.

UNLABELLED: The use of animals in scientific experiments has been described since the fifth century BC. A number of scientific advances in health are attributed to animal models. The issue of the moral status of animals has always been debated. OBJECTIVES: This article aims to review and to present a historical summary of the current laws, to guide researchers who wish to use animal models in otolaryngology research. MATERIAL AND METHODS: Research on the medline database. RESULTS: For many years there were no laws ruling the use of animals in scientific experimentation in Brazil. Standards set by national and international organizations were followed. Recently, Law No. 11.794/08 established procedures for the scientific use of animals. Studies in otolaryngology have used the larynxes of rabbits, pigs, dogs, guinea pigs (Cavia porcellus), and mice. There were also studies comparing rabbits, rats, and dogs, rhinoplasty on rabbits, and inner ear studies on rats and guinea pigs (albino). CONCLUSIONS: The researchers involved in scientific work with animals should know the principles of Law 11.794/08 and investigate what animals are appropriate for each area of study in their models. Otolaryngologists, especially those dedicated to research, need to be mindful of the ethical rules regarding the use of animals in their studies.

Resultados na percepção de fala após conversão do Spectra® para Freedom® / Result on speech perception after conversion from Spectra® to Freedom®

As novas tecnologias do processador Freedom® foram criadas para proporcionar melhorias no processamento do som acústico de entrada, não apenas para novos usuários, como para gerações anteriores de implante coclear. OBJETIVO: Identificar a contribuição da tecnologia do processador de fala Freedom® para implante coclear multicanal, Nucleus22®, no desempenho de percepção de fala no silêncio e no ruído, e nos limiares audiométricos. MATERIAL E MÉTODO: A forma de estudo foi de coorte histórico com corte transversal. Dezessete pacientes preencheram os critérios de inclusão. Antes de iniciar os testes, o último mapa em uso com o Spectra® foi revisto e otimizado e o funcionamento do processador foi verificado. Os testes de fala foram apresentados a 60dBNPS em material gravado: monossílabos; frases em apresentação aberta no silêncio; e no ruído (SNR = 0dB). Foram realizadas audiometrias em campo livre com ambos os processadores de fala. A análise estatística utilizou testes não-paramétricos. RESULTADOS: Quando analisada a contribuição do Freedom® para pacientes com Nucleus22®, observa-se diferença estatisticamente significativa em todos os testes de percepção de fala e em todos os limiares audiométricos. CONCLUSÃO: A tecnologia contribuiu no desempenho de percepção de fala e nos limiares audiométricos dos pacientes usuários de Nucleus22®.

New technology in the Freedom® speech processor for cochlear implants was developed to improve how incoming acoustic sound is processed; this applies not only for new users, but also for previous generations of cochlear implants. AIM: To identify the contribution of this technology - the Nucleus 22® - on speech perception tests in silence and in noise, and on audiometric thresholds. METHODS: A cross-sectional cohort study was undertaken. Seventeen patients were selected. The last map based on the Spectra® was revised and optimized before starting the tests. Troubleshooting was used to identify malfunction. To identify the contribution of the Freedom® technology for the Nucleus22®, auditory thresholds and speech perception tests were performed in free field in sound-proof booths. Recorded monosyllables and sentences in silence and in noise (SNR = 0dB) were presented at 60 dBSPL. The nonparametric Wilcoxon test for paired data was used to compare groups. RESULTS: Freedom® applied for the Nucleus22® showed a statistically significant difference in all speech perception tests and audiometric thresholds. CONCLUSION: The reedom® technology improved the performance of speech perception and audiometric thresholds of patients with Nucleus 22®.

Ética da pesquisa em modelos animais / Research ethics in animal models

A utilização de animais em experimentos científicos é descrita desde o século V a.C. Avanços científicos na área da saúde são atribuídos a modelos animais. O status moral dos animais sempre foi debatido. OBJETIVOS: Este artigo visa à revisão histórica e resumo da legislação atual, para orientar pesquisadores ao utilizar modelos animais na pesquisa em otorrinolaringologia. MATERIAL E MÉTODOS: Pesquisa na base de dados Medline. RESULTADOS: no Brasil, por muitos anos não havia regulamentação para o uso de animais em experimentação. Eram seguidas normas de organizações nacionais e internacionais. Recentemente, foi sancionada a lei nº 11.794/08, que estabelece procedimentos para o uso científico de animais. Na otorrinolaringologia, os estudos com laringe utilizaram coelho, porco, cachorro, cobaias (Cavia porcellus) e camundongo; estudos para face coelho, rato e cachorro; rinoplastia com coelho; e orelha interna com ratos e cobaias (albinas). CONCLUSÕES: Os pesquisadores envolvidos em trabalhos científicos com animais devem conhecer os princípios da lei nº 11.794/08 e pesquisar quais animais são apropriados para cada subárea estudada seus modelos com maior aplicabilidade. Os otorrinolaringologistas, especialmente aqueles que se dedicam à pesquisa, necessitam estar sempre atentos para o respeito às regras éticas de utilização de animais em seus estudos.

The use of animals in scientific experiments has beendescribed since the fifth century BC. A number of scientific advances in health are attributed to animal models. The issue of the moral status of animals has always been debated. OBJECTIVES: This article aims to review and to present a historical summary of the current laws, to guide researchers who wish to use animal models in otolaryngology research. MATERIAL AND METHODS: Research on the medline database. RESULTS: For many years there were no laws ruling the use of animals in scientific experimentation in Brazil. Standards set by national and international organizations were followed. Recently, Law No. 11.794/08 established procedures for the scientific use of animals. Studies in otolaryngology have used the larynxes of rabbits, pigs, dogs, guinea pigs (Cavia porcellus), and mice. There were also studies comparing rabbits, rats, and dogs, rhinoplasty on rabbits, and inner ear studies on rats and guinea pigs (albino). CONCLUSIONS: The researchers involved in scientific work with animals should know the principles of Law 11.794/08 and investigate what animals are appropriate for each area of study in their models. Otolaryngologists, especially those dedicated to research, need to be mindful of the ethical rules regarding the use of animals in their studies.

Adaptação de próteses auditivas no candidato ao implante coclear / Fitting hearing aid in patients candidate for cochlear implants

OBJETIVO: caracterizar o perfil audiométrico e demográfico de pacientes candidatos ao implante coclear encaminhados ao setor de prótese auditiva e entre esses, a frequência de indivíduos que se beneficiaram da amplificação sonora. MÉTODOS: foram estudados os pacientes atendidos no período de maio de 2007 a dezembro 2008. Foi realizado o levantamento do perfil da população segundo sexo, idade, escolaridade e etiologia. Foi calculada a média da melhor e pior orelha e classificada segundo Frota (2003). A média dos limiares auditivos com as próteses auditivas indicadas foi calculada e considerada como benefício quando permitia acesso aos sons da fala do português brasileiro (Russo e Behlau, 1993). RESULTADOS: foram avaliados nesse período 194 prontuários. 108 pacientes já eram usuários de próteses auditivas (55,6 por cento), 100 do sexo masculino (52 por cento), 94 do sexo feminino (48 por cento), 109 eram crianças (56 por cento) e 85 eram adultos (44 por cento). A média de idade foi 4,8 anos (crianças) e 41,9 anos (adultos). Entre os adultos, 24 possuíam ensino fundamental incompleto (33 por cento). A etiologia mais frequente foi a desconhecida (33 por cento). Dezenove pacientes se beneficiaram do uso da prótese auditiva (10 por cento). No grupo de pacientes que se beneficiaram da prótese auditiva, a média dos limiares em campo livre com amplificação foi de 47 dBNA e 48 dBNA, na melhor e na pior orelha, respectivamente, nas crianças e 50 dBNA e 45 dBNA, respectivamente, nos adultos. CONCLUSÃO: o perfil dos pacientes foi: na maioria criança, sexo masculino, etiologia desconhecida, já usuários de próteses auditivas com média de idade 4,8 anos (crianças) e 41,9 anos (adultos), audiometria com perda auditiva neurossensorial profunda bilateral, sendo que a prótese beneficiou 10 por cento dos pacientes.

PURPOSE: to characterize the audiometric and demographic profile of cochlear implant candidates who were referred to hearing aid sector, and if they benefit from the amplification of the hearing aid. METHODS: patients who had been attended from May 2007 to December 2008 were studied. We collected the patients' profile involving: gender, age, education and etiology. The hearing threshold's average for the good and worse ears was calculated and classified according to Frota (2003). The averaged sound field thresholds with hearing aid was calculated for both ears and considered as a benefit when allowed for an access to the speech sounds of Brazilian Portuguese. RESULTS: a hundred and ninety-four medical records were evaluated in this period. Among them, 108 patients had already been wearing hearing aids (55.6 percent), 100 were male (52 percent), 94 female (48 percent), 109 were children (56 percent) and 85 adults (44 percent). The average age was 4.8year old for children and 41.9-year old for adults. Among the adults, 24 patients had incomplete high school education (33 percent). Unknown etiology was the most frequent cause (33 percent). Nineteen patients have benefited from the use of hearing aids (10 percent). The averaged sound field thresholds with hearing aid was 47 dBHL and 48 dBHL, in best and worse ears, respectively, for children, and 50 dBHL and 45 dBHL, respectively, for adults. CONCLUSIONS: the profile of the patients was: majority of children, males, unknown etiology, previously hearing aid users, average of age 4.8-year old for children and 41.9 for adults. Pure tone thresholds revealed profound bilateral hearing loss. About 10 percent of patients were benefited with the use of hearing aids.