Resumo Introdução: A avaliação convencional da telemetria neural e de impedâncias implica o uso do computador acoplado a uma interface, o software fornece o estímulo e a visualização das respostas. Recentemente, foi lançado um controle remoto (CR220®), que possibilita testes intraoperatórios com instrumental mínimo. Objetivo: Avaliar a concordância dos valores das impedâncias e dos limiares da telemetria neural e o tempo de execução no procedimento convencional e pelo controle remoto. Método: Estudo prospectivo transversal multicêntrico. Foram incluídas as avaliações intraoperatórias de implante coclear compatível com o uso do CR220®. Os testes foram realizados nos 22 eletrodos para comparar os tempos de execução nas duas situações. Foi analisada a concordância dos valores do limiar da telemetria neural obtidos em cinco eletrodos e a concordância das impedâncias foi avaliada pelo número de eletrodos com valores alterados em cada procedimento. Resultados: Não houve diferença significante entre as impedâncias. Obteve-se moderada a forte correlação entre os limiares do potencial de ação composto eletricamente evocado. O tempo médio para os procedimentos com o CR220 foi significativamente menor do que com o procedimento convencional. Conclusão: O uso do CR220 proporcionou registros bem-sucedidos para a telemetria de impedância e a telemetria automática de respostas neurais.

Abstract Introduction: The conventional evaluation of neural telemetry and impedance requires the use of the computer coupled to an interface, with software that provides visualization of the stimulus and response. Recently, a remote control (CR220®) was launched in the market, that allows the performance of intraoperative tests with minimal instrumentation. Objective: To evaluate the agreement of the impedance values and neural telemetry thresholds, and the time of performance in the conventional procedure and by the remote control. Methods: Multicentric prospective cross-sectional study. Intraoperative evaluations of cochlear implants compatible with the use of CR220® were included. The tests were carried out in the 22 electrodes to compare the time of performance in the two situations. The agreement of the neural telemetry threshold values obtained from five electrodes was analyzed, and the agreement of impedance was evaluated by the number of electrodes with altered values in each procedure. Results: There were no significant difference between the impedance values. There was a moderate to strong correlation between the electrically-evoked compound action potential thresholds. The mean time to perform the procedures using the CR220 was significantly lower than that with the conventional procedure. Conclusion: The use of the CR220 provided successful records for impedance telemetry and automatic neural response telemetry.

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.

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.