Would Zika virus Infection in Pregnancy Be a Sentence of Poor Neurological Prognosis for Exposed Children? Neurodevelopmental Outcomes in a Cohort from Brazilian Amazon.

Infections with Flavivirus in pregnant women are not associated with vertical transmission. However, in 2015, severe cases of congenital infection were reported during the Zika virus outbreak in Brazil. More subtle infections in children born to mothers with ZIKV still remain uncertain and the spectrum of this new congenital syndrome is still under construction. This study describes outcomes regarding neurodevelopment and neurological examination in the first years of life, of a cohort of 77 children born to pregnant women with ZIKV infection in Manaus, Brazil, from 2017 to 2020. In the group of normocephalic children (92.2%), most showed satisfactory performance in neuropsychomotor development, with a delay in 29.6% and changes in neurological examination in 27.1%, with two children showing muscle-strength deficits. All microcephalic children (5.2%) evolved with severe neuropsychomotor-development delay, spastic tetraparesis, and alterations in the imaging exam. In this cohort, 10.5% of the children had macrocephaly at birth, but only 2.6% remained in this classification. Although microcephaly has been considered as the main marker of congenital-Zika-virus syndrome in previous studies, its absence does not exclude the possibility of the syndrome. This highlights the importance of clinical follow-up, regardless of the classification of head circumference at birth.

Neurological Findings in Children without Congenital Microcephaly Exposed to Zika Virus in Utero: A Case Series Study.

The Zika virus can induce a disruptive sequence in the fetal brain and is manifested mainly by microcephaly. Knowledge gaps still exist as to whether the virus can cause minor disorders that are perceived later on during the first years of life in children who are exposed but are asymptomatic at birth. In this case series, we describe the outcomes related to neurodevelopment through the neurological assessment of 26 non-microcephalic children who had intrauterine exposure to Zika virus. Children were submitted for neurological examinations and Bayley Scales-III (cognition, language, and motor performance). The majority (65.4%) obtained satisfactory performance in neurodevelopment. The most impaired domain was language, with 30.7% impairment. Severe neurological disorders occurred in five children (19.2%) and these were spastic hemiparesis, epilepsy associated with congenital macrocephaly (Zika and human immunodeficiency virus), two cases of autism (one exposed to Zika and Toxoplasma gondii) and progressive sensorineural hearing loss (GJB2 mutation). We concluded that non-microcephalic children with intrauterine exposure to Zika virus, in their majority, had achieved satisfactory performance in all neurodevelopmental domains. One third of the cases had some impairment, but the predominant group had mild alterations, with low occurrence of moderate to severe disorders, similar to other studies in Brazil.

Establishing auditory steady-state response thresholds to narrow band CE-chirps(®) in full-term neonates.

UNLABELLED: Narrow band CE-chirps(®) were developed to provide a better synchronization of neural response due to the compensation of the traveling wave delay in the basilar membrane. These stimuli combined with a detection method that includes higher response harmonics on the auditory steady-state response (ASSR) recording was studied in this research. OBJECTIVE: (1) To establish air conduction thresholds for ASSR to narrow band CE-chirp(®) in normal hearing full-term neonates; (2) describe the test time needed for the above in one ear and (3) to compare the results in infants and normally hearing adults. METHOD: ASSR to air-conducted stimuli were obtained in 30 full-term neonates (14 girls and 16 boys) with an average age of 34.3h of life. All neonates were presented presence of transient-evoked otoacoustic emissions (TEOAE) and result "pass" in automatic ABR at 35dB nHL before ASSR test. ASSR thresholds of both ears of 10 normal hearing adults (5 girls and 5 boys) varied in age between 23 and 30 years and with hearing thresholds better than or equal to 15dB HL at all frequencies between 250 and 8000Hz were recorded to compare with the neonate data. RESULTS: The neonate ASSR thresholds estimated from 50% using cumulative distributions were 24.5, 13.5, 7.5 and 10dB nHL at 500, 1000, 2000, and 4000Hz, respectively. For the same frequency order, ASSR thresholds estimated from 90% of the neonates were 34.5, 28, 12.5 and 15dB nHL. It required 21.2 (±5)min on average to obtain threshold in each ear in neonates, with a range of 12-29min. When ASSR thresholds recorded in full-term neonates and adults were directly compared, the differences between these groups were not significant for 1000Hz (p=0.500), 2000Hz (p=0.610) and 4000Hz (p=0.362). However, at 500Hz, ASSR thresholds in neonates tend to be greater than in adults (p=0.001). CONCLUSION: In this study ASSR thresholds estimated from 90% of the neonates were 34.5, 28, 12.5 and 15dB nHL. It required 21.2 (±5)min on average to obtain threshold in each ear and ASSR thresholds to narrow band CE-chirp(®) in neonates are not significant for adults ASSR thresholds, except at 500Hz, when the ASSR thresholds in neonates tend to be greater than in adults.

Comparing auditory brainstem responses (ABRs) to toneburst and narrow band CE-chirp in young infants.

OBJECTIVE: The difference of characteristics (latency and amplitude) between toneburst and narrow CE-chirp stimuli on ABR recording was analyzed in normal hearing infants. METHODS: 500, 1000, 2000 and 4000 Hz toneburst and narrow band CE-chirp auditory brainstem responses (ABRs) were recorded in 40 normal-hearing infants. The amplitude and latency parameters of the ABR were collected for each of the four stimulus levels: 80, 60, 40, and 20 dB nHL. Both stimuli started from 80 dB nHL using alternating polarity and the rates were both 27.1/s. RESULTS: The toneburst latencies are greater than narrow band CE-chirp latencies for all intensities at 500, 1000 and 2000 Hz (p < 0.001). However, at 4000 Hz this difference was not significant. At 500 Hz, wave V amplitude is larger for toneburst than narrow CE-chirp (p < 0.001) in 80 dB nHL. The difference between the two stimuli in 60 dB nHL was not significant (p = 0.495) and at 40 and 20 dB nHL the wave V narrow band CE-chirp amplitude is greater than toneburst amplitude (p < 0.001). At 1000, 2000 and 4000 Hz there is no difference between the wave V toneburst and narrow band CE-chirp amplitudes at 80 dB nHL (p = 0.940; p = 0.776 and p = 0.217 respectively). On the other hand, in the levels to 60, 40 and 20 dB nHL, narrow band CE-chirp amplitudes are larger than toneburst amplitude (p < 0.001). CONCLUSION: Narrow band CE-chirp ABRs generates shorter latencies than the toneburst ABRs, especially to low frequencies. Higher amplitudes were found with narrow band CE-chirp stimuli for all frequencies tested, except to high levels.

Testing time comparison between two procedures with new technologies of Automated Auditory Brainstem Response (AABR).

PURPOSE: To compare the testing time of two Automated Auditory Brainstem Response (AABR) procedures using different repetition rates and detection modes. METHODS: A hearing screening using AABR was performed in 30 newborns with mean age of 21 days. Each newborn was submitted to two hearing screening procedures using different AABR equipments (Procedure 1 and Procedure 2). Procedure 1 used a repetition rate of 53 Hz and the one-sample test for response detection; Procedure 2 used a repetition rate of 90 Hz, and the q-sample test for response detection. The ABR with click stimulus was then registered as gold standard test, and the responses were analyzed by a trained audiologist. RESULTS: The mean time observed for Procedure 1 considering both ears was 84.8 (±53.5) seconds; for Procedure 2 the mean time was 27.9 (±20.0) seconds. The testing time of the first procedure was three times longer than the second one. Statistical analysis showed significant difference between the testing times of the procedures. CONCLUSION: The q-sample test and the repetition rate of 90 Hz used in the AABR equipment showed earlier response detection.

Threshold prediction in children with sensorioneural hearing loss using the auditory steady-state responses and tone-evoked auditory brain stem response.

OBJECTIVE: The purpose of this study was to compare ASSRs to tone-evoked ABR and to behavioral thresholds obtained on follow-up audiometry at 500, 1000, 2000, and 4000 Hz in infants and young children. METHODS: The study included 17 infants and young children ages between 2 months and 3 years old, with sensorineural hearing loss. The ASSRs thresholds were compared with the tone-evoked ABR thresholds, and with the behavioral thresholds obtained on follow-up audiometry. RESULTS: The correlation of tone-evoked ABR and ASSRs thresholds at 500, 1000, 2000 and 4000 Hz was 0.91, 0.76, 0.81, 0.89, respectively. ASSRs and behavioral hearing thresholds obtained on follow-up were highly correlated, with Pearson r values exceeding 0.94 at each of the test frequencies. CONCLUSIONS: Multiple ASSRs have strong correlations to tone-evoked ABR and to behavioral thresholds obtained during follow-up in hearing impaired infants and young children. These results might be useful in order to provide further evidence for the use of multiple ASSRs, as an alternative tool to tone-evoked ABR, although further data are still required.

Auditory steady-state response in children with cochlear hearing loss.

BACKGROUND: auditory steady-state response (ASSR) is indicated as a promising technique in the assessment of the hearing status of children. AIM: to investigate the level of agreement between the results of the ASSR and the visual reinforcement audiometry (VRA) in a group of children, thus examining the clinical applicability of this technique in hearing assessment of children. METHOD: participants were 14 children with ages between 4 and 36 months (mean 16 months) with the diagnosis of cochlear hearing loss. The ASSR was recorded in the frequencies of 0.5, 1, 2 and 4kHz for multiple simultaneous stimulation and the results were compared with the visual reinforcement audiometry (VRA). RESULTS: the intraclass correlation coefficients between ASSR and VRA were 0.90, 0.93, 0.93 and 0.89 respectively for the frequencies of 0.5, 1, 2 and 4kHz, indicating a strong correlation between the techniques. CONCLUSION: the ASSR can provide accurate information to support the selection of hearing aids for children when it is not possible to perform the VRA.

Steady-state auditory evoked responses in audiological diagnosis in children: a comparison with brainstem evoked auditory responses.

UNLABELLED: Auditory Steady-State Responses (ASSR) are being recognized as a promising technique in the assessment of hearing in children. AIM: To investigate the agreement level between results obtained from ASSR and click-ABR in a group of children with sensorineural hearing loss, in order to study the clinical applicability of this technique to evaluate the hearing status in young children. STUDY DESIGN: clinical prospective with a cross-sectional cohort. MATERIALS AND METHODS: 15 children aged between two and 36 months and with diagnosis of sensorineural hearing loss. The correlation between the responses of the two tests was evaluated by intraclass correlation coefficient and McNemar test comparing the probability of responses in both tests. RESULTS: The correlation coefficients were: 0.70; 0.64; 0.49; 0.69; 0.63 and 0.68 respectively for frequencies of 1, 2, 4, 1-2, 2-4 and 1-2-4kHz. In McNemar test p = 0.000, indicating that the probability of obtaining responses in both tests was not equal, but greater for the ASSR. CONCLUSION: we found good agreement between the techniques among the four frequencies evaluated, suggesting that both tests may be complementary. However, the ASSR was able to obtain additional information in cases of severe and profound hearing losses, adding important data to the management of these children, providing greater accuracy to the audiological diagnosis.

A triagem auditiva neonatal antecipa o diagnóstico e a intervenção em crianças com perda auditiva? / Does newborn hearing screening anticipate the diagnosis and the intervention in children with hearing loss?

Objetivo Identificar a idade de diagnóstico, intervenção e amplificação pré e pós-implantação da Triagem Auditiva Neonatal (TAN) em um serviço de saúde auditiva e comparar aos indicadores propostos pelo Comitê Conjunto para Audição Infantil.Métodos Trezentos e treze prontuários de crianças atendidas no setor de reabilitação auditiva foram analisados, verificando se foi realizada a triagem auditiva e seu resultado, suspeita e idade de diagnóstico, intervenção e amplificação e se estas últimas atendiam aos indicadores preconizados: três meses para diagnóstico e seis meses para intervenção.Resultados Crianças identificadas pela TAN foram diagnosticadas e iniciaram a intervenção mais cedo do que as que não realizaram. Considerando-se a demanda institucional pré e pós a implantação da TAN, observou-se redução da idade de intervenção e amplificação após a implantação. Independentemente do resultado obtido na TAN (passa/falha), as crianças que passaram pela triagem apresentaram vantagem, quando comparadas às não triadas, uma vez que, dentre as triadas, antecipou-se o diagnóstico, a intervenção e a amplificação. Menos da metade das crianças que falharam na TAN concluíram o diagnóstico e iniciaram a intervenção no tempo preconizado.Conclusão A TAN antecipou o diagnóstico e a intervenção em crianças com perda auditiva. Contudo, fatores como a não adesão da família e as peculiaridades do diagnóstico retardaram os processos, impedindo que os indicadores preconizados fossem alcançados, na maior parte das crianças.

Purpose To identify the age at diagnosis, intervention and amplification, pre and post Newborn Hearing Screening (NHS) implantation into a hearing health service and to compare with the indicators proposed by Joint Committee on Infant Hearing.Methods Three hundred and thirteen files of children enrolled in the auditory rehabilitation sector were analyzed, verifying if the newborn hearing screening and its results were performed, suspicion and age at the diagnosis, intervention and amplification and if they reach the recommended indicators: 3 months for diagnosis and 6 months for intervention.Results Children identified by the NHS were diagnosed and started the intervention sooner than those who did not perform newborn hearing screening. Considering the institutional demand pre and post NHS implementation, there was a reduction of intervention and amplification ages, post newborn hearing screening implementation. Regardless the NHS outcome (pass/fail), screened children had advantage when compared to the non-screened ones, once, among those screened, the diagnosis, intervention and amplification were anticipated. Less than a half of the children who failed NHS completed the diagnosis and began the intervention in the recommended period.Conclusion NHS anticipates the diagnosis and the intervention in children with hearing loss; however, some factors such as family non-adherence and the diagnosis slowed the process, making it impossible for the recommended indicators to be reached in most of the children.

Investigação etiológica da deficiência auditiva em neonatos identificados em um programa de triagem auditiva neonatal universal / Etiological investigation of deafness in neonates screened in a universal newborn hearing screening program

Objetivo descrever os resultados da investigação etiológica da deficiência auditiva realizada em neonatos rastreados em um programa de triagem auditiva neonatal universal. Métodos estudo descritivo, transversal e prospectivo. Foram incluídos no estudo todos os neonatos diagnosticados com deficiência auditiva identificados em um programa de triagem auditiva neonatal universal no período de agosto de 2003 a dezembro de 2006. A provável etiologia da deficiência auditiva foi determinada após anamnese detalhada realizada pelo médico otorrinolaringologista; pesquisa das sorologias para toxoplasmose, rubéola, citomegalovírus, herpes, sífilis e HIV; tomografia dos ossos temporais e exames genéticos. Resultados foram diagnosticados 17 sujeitos com deficiência auditiva no período estudado. 64.7% dos casos estudados apresentaram como provável etiologia causas pré-natais, 29.4% causas peri-natais e um sujeito (5,9%) apresentou etiologia desconhecida. Das causas pré-natais, 36.4% tiveram origem genética confirmada e 36.4% etiologia presumida de hereditariedade. Foi confirmada a presença de infecções congênitas em 18.2% dos casos e um sujeito (9%) apresentou anomalia craniofacial como provável etiologia. O grau de perda auditiva mais frequente observado nos sujeitos estudados foi o profundo (47,1%). Conclusão a maior ocorrência de etiologias observada neste estudo foram as de origem pré-natal, seguida das de origem peri-natal. .

Purpose to describe the results of etiology of deaf in neonates screened in a universal newborn hearing screening program. Methods a descriptive, cross-sectional and prospective study. The study included all newborns diagnosed with hearing loss identified in a universal newborn hearing screening program from August 2003 to December 2006. The etiology of deaf was determined after detailed anamnesis performed by the otorhinolaryngologist; survey of serological tests for toxoplasmosis, rubella, cytomegalovirus, herpes, syphilis and HIV; tomography of the temporal bone and genetic tests. Results 17 neonates were diagnosed with hearing loss in the period studied. 64.7% of cases presented as probable causes prenatal etiology, 29.4% perinatal causes and one child (5.9%) had unknown etiology. Of prenatal causes, 36.4% had confirmed genetic origin and 36.4% presumed etiology of heredity. We confirmed the presence of congenital infections in 18.2% of cases and one child (9%) had craniofacial anomalies as a possible etiology. The degree of hearing loss more frequently observed in the subjects studied was the profound (47.1%). Conclusion the increased occurrence of etiologies in this study was of prenatal origin, followed by perinatal origin. .

Comparação do tempo de dois procedimentos com novas tecnologias de Potencial Evocado Auditivo de Tronco Encefálico Automático (PEATE-A) / Testing time comparison between two procedures with new technologies of Automated Auditory Brainstem Response (AABR)

OBJETIVO: Comparar o tempo de dois procedimentos de Potencial Evocado Auditivo de Tronco Encefálico Automático (PEATE-A) utilizando taxa de repetição e modo de detecção diferentes. MÉTODOS: Foi realizada a triagem auditiva com Potencial Evocado Auditivo de Tronco Encefálico Automático (PEATE-A) em 30 neonatos, com média de idade de 21 dias. Cada neonato foi submetido a dois procedimentos de triagem auditiva com diferentes equipamentos de PEATE-A (Procedimento 1 e Procedimento 2). O Procedimento 1 utilizou taxa de repetição de 53 Hz e teste estatístico para detecção da resposta denominado one-sample test, e o Procedimento 2 utilizou taxa de repetição de 90 Hz e teste estatístico para detecção da resposta denominado q-sample test. Em seguida, foi registrado o PEATE com estímulo clique como teste padrão ouro, cuja análise das respostas foi realizada por um fonoaudiólogo com experiência neste procedimento. RESULTADOS: O tempo médio observado para o Procedimento 1 considerando ambas orelhas foi de 84,8 (±53,5) segundos; para o Procedimento 2, o tempo médio foi de 27,9 (±20,0) segundos. O primeiro procedimento apresentou tempo de exame aproximadamente três vezes maior quando comparado ao segundo. A análise estatística mostrou diferença significativa entre os tempos dos procedimentos. CONCLUSÃO: O q-sample test e taxa de repetição em torno de 90 Hz nos equipamentos de PEATE-A detectaram a resposta de maneira mais rápida.

PURPOSE: To compare the testing time of two Automated Auditory Brainstem Response (AABR) procedures using different repetition rates and detection modes. METHODS: A hearing screening using AABR was performed in 30 newborns with mean age of 21 days. Each newborn was submitted to two hearing screening procedures using different AABR equipments (Procedure 1 and Procedure 2). Procedure 1 used a repetition rate of 53 Hz and the one-sample test for response detection; Procedure 2 used a repetition rate of 90 Hz, and the q-sample test for response detection. The ABR with click stimulus was then registered as gold standard test, and the responses were analyzed by a trained audiologist. RESULTS: The mean time observed for Procedure 1 considering both ears was 84.8 (±53.5) seconds; for Procedure 2 the mean time was 27.9 (±20.0) seconds. The testing time of the first procedure was three times longer than the second one. Statistical analysis showed significant difference between the testing times of the procedures. CONCLUSION: The q-sample test and the repetition rate of 90 Hz used in the AABR equipment showed earlier response detection.

Comparação dos estímulos clique e CE-chirp® no registro do Potencial Evocado Auditivo de Tronco Encefálico / Comparison of click and CE-chirp® stimuli on Brainstem Auditory Evoked Potential recording

OBJETIVO: Comparar as latências e as amplitudes da onda V no registro do Potencial Evocado Auditivo de Tronco Encefálico (PEATE) com os estímulos clique e CE-chirp® e a presença ou ausência das ondas I, III e V em fortes intensidades. MÉTODOS: Estudo transversal com 12 adultos com limiares audiométricos <15 dBNA (24 orelhas) e idade média de 27 anos. Os parâmetros utilizados para o registro com os dois estímulos nas intensidades de 80, 60, 40, 20 dBnNA foram polaridade alternada e taxa de repetição de 27,1 Hz. RESULTADOS: As latências da onda V observadas com CE-chirp® foram maiores que as observadas com o clique nas intensidades fracas (20 e 40 dBnNA). Já nas intensidades fortes (60 e 80 dBnNA), o oposto ocorreu. Maiores amplitudes foram observadas com o CE-chirp® em todas as intensidades, exceto em 80 dBnNA. CONCLUSÃO: O CE-chirp® apresentou latências mais curtas que as observadas com o clique em fortes intensidades e maiores amplitudes em todas as intensidades, exceto em 80 dBnNA. As ondas I e III tenderam a desaparecer quando o estímulo CE-chirp® foi utilizado.

PURPOSE: To compare the latencies and amplitudes of wave V on the Brainstem Auditory Evoked Potential (BAEP) recording obtained with click and CE-chirp® stimuli and the presence or absence of waves I, III and V in high intensities. METHODS: Cross-sectional study with 12 adults with audiometric thresholds <15 dBHL (24 ears) and mean age of 27 years. The parameters used for the recording with both stimuli in intensities of 80, 60, 40, 20 dBnHL were alternate polarity and repetition rate of 27.1 Hz. RESULTS: The CE-chirp® latencies for wave V were longer than click latencies at low intensity levels (20 and 40 dBnHL). At high intensity levels (60 and 80 dBnHL), the opposite occurred. Larger wave V amplitudes were observed with CE-chirp® in all intensity levels, except at 80 dBnHL. CONCLUSION: The CE-chirp® showed shorter latencies than those observed with clicks at high intensity levels and larger amplitudes at all intensity levels, except at 80 dBnHL. The waves I and III tended to disappear with CE-chirp® stimulation.

Potenciais evocados auditivos por frequência específica em lactentes com audição normal / Frequency-specific auditory brainstem response in infants with normal hearing auditory evoked potentials

OBJETIVO: determinar os níveis mínimos de resposta (NMR) e a latência da onda V em lactentes ouvintes nas frequências de 0.5, 1, 2 e 4 kHz. MÉTODOS: foram avaliados 23 lactentes sem indicadores de risco para deficiência auditiva que apresentavam emissões otoacústicas evocadas por estímulo transiente (EOAET) e potenciais evocados auditivos de tronco encefálico automático (PEATE-A) presentes bilateralmente. RESULTADOS: obtiveram-se NMR médios de 34.2 dBnNA, 25.4 dBnNA, 19 dBnNA e 17.5 dBnNA para as frequências de 0.5, 1, 2 e 4 kHz, respectivamente. Os tempos médios de latência da onda V em 70 e 20 dBnNA, respectivamente, na frequência de 0.5 kHz foram de 10.75 ms e 15.2 ms, em 1 kHz de 8.9 ms e 13.4 ms; de 7.7 ms e 10.2 ms em 2 kHz, e para 4 kHz foram de 7.3 ms e 9.4 ms. CONCLUSÃO: os valores encontrados podem ser utilizados na prática clínica para orientar o diagnóstico diferencial da perda auditiva, complementando a avaliação auditiva de lactentes.

PURPOSE: to determine the ABR thresholds and the latency of V wave in hearing infants at the frequencies: 0.5, 1, 2 and 4 kHz. METHODS: 23 infants with no risk factors concerning hearing loss that had transient otoacoustic emissions (TOAE) and automatic auditory brainstem response (A-ABR), bilaterally present, were evaluated. RESULTS: ABR thresholds were obtained in average, namely: 34.2 dBHL, 25.4 dBHL, 19 dBHL and 17.5 dBHL for frequencies of 0.5, 1, 2 and 4 kHz, respectively. The mean latency of V wave at 70 and 20 dBHL, respectively, at a 0.5 kHz frequency were 10.75 ms and 15.2 ms, 1 kHz 8.9 ms and 13.4 ms; 7.7 ms and 10.2 ms at 2 kHz, and as for4 kHz they were 7.3 ms and 9.4 ms. CONCLUSIONS: the found values can be used in clinical practice in order to guide the differential diagnosis of hearing loss, complementing the evaluation as for hearing infants.

Potenciais evocados auditivos de estado estável em crianças com perdas auditivas cocleares / Auditory steady-state response in children with cochler hearing loss

Background: auditory steady-state response (ASSR) is indicated as a promising technique in the assessment of the hearing status of children. AIM: to investigate the level of agreement between the results of the ASSR and the visual reinforcement audiometry (VRA) in a group of children, thus examining the clinical applicability of this technique in hearing assessment of children. Method: participants were 14 children with ages between 4 and 36 months (mean 16 months) with the diagnosis of cochlear hearing loss. The ASSR was recorded in the frequencies of 0.5, 1, 2 and 4kHz for multiple simultaneous stimulation and the results were compared with the visual reinforcement audiometry (VRA). Results: the intraclass correlation coefficients between ASSR and VRA were 0.90, 0.93, 0.93 and 0.89 respectively for the frequencies of 0.5, 1, 2 and 4kHz, indicating a strong correlation between the techniques. Conclusion: the ASSR can provide accurate information to support the selection of hearing aids for children when it is not possible to perform the VRA.

Tema: os potenciais evocados auditivos de estado estável (PEAEE) têm sido apontados como uma técnica promissora na avaliação audiológica infantil. Objetivo: investigar o nível de concordância entre os resultados do PEAEE e a audiometria de reforço visual (VRA) em um grupo de crianças, averiguando assim a aplicabilidade clínica desta técnica na avaliação audiológica infantil. Método: foram avaliadas 14 crianças com idade entre 4 e 36 meses (média 16 meses) com diagnóstico de perda auditiva coclear. Os PEAEE foram registrados nas frequências de 0,5; 1; 2 e 4kHz pela estimulação múltipla simultânea, e os resultados obtidos foram comparados com os resultados da VRA. Resultados: os coeficientes de correlação intraclasse entre as respostas dos PEAEE e da VRA foram de 0,90; 0,93; 0,93 e 0,89 para as frequências de 0,5; 1; 2 e 4kHz, respectivamente, indicando forte concordância entre as técnicas. Conclusão: os PEAEE podem fornecer informações precisas para que se possa dar início à seleção e adaptação dos AASI em crianças nas quais ainda não é possível a realização da VRA.

Presença de microfonismo coclear no peate-clique: diagnóstico diferencial entre espectro da neuropatia auditiva e perdas auditivas cocleares descendentes em crianças / Presence of cochlear microphonics in click-ABR: differential diagnosis between auditory neuropathy spectrum disorder and steeply sloping cochlear hearing loss in children

TEMA: diagnóstico diferencial entre espectro da neuropatia auditiva e perdas auditivas cocleares descendentes em crianças com presença de microfonismo coclear no PEATE-clique. PROCEDIMENTOS: este relato de caso descreve os resultados da avaliação audiológica de duas crianças atendidas no Centro "Audição na Criança" da Divisão de Educação e Reabilitação dos Distúrbios da Comunicação da Pontifícia Universidade Católica de São Paulo (CeAC/DERDIC/PUCSP) que apresentaram microfonismo coclear no registro do PEATE-clique. As crianças foram submetidas às avaliações utilizando-se o PEATE-clique, o registro das emissões otoacústicas e a avaliação audiológica tonal, com a técnica da Audiometria de Reforço Visual. RESULTADOS: as avaliações comportamental, eletroacústica e eletrofisiológica revelaram que as crianças apresentam perda auditiva sensorioneural (coclear) com configuração descendente, de modo que a presença do microfonismo coclear no registro do PEATE-clique era provavelmente gerada pela preservação da cóclea nas frequências baixas. CONCLUSÃO: os casos apresentados mostram que na ausência das emissões otoacústicas e presença do microfonismo coclear, não se deve interpretar isoladamente cada exame, para que não ocorram equívocos no diagnóstico, que pode ser confundido com o Espectro da Neuropatia Auditiva. O microfonismo coclear pode aparecer em outras condições, tais como em perdas auditivas cocleares descendentes.

BACKGROUND: differential diagnosis between auditory neuropathy spectrum disorder andsteeply sloping cochlear hearing loss in children with presence of cochlear microphonics for click-ABR. PROCEDURES: this case report describes the results of the audiological evaluation for two children assisted at Centro "Audição na Criança" of Divisão de Educação e Reabilitação dos Distúrbios da Comunicação of Pontifícia Universidade Católica of São Paulo (CeAC/DERDIC/PUCSP) with presence of cochlear microphonics for click-ABR. The children are assessed by click-ABR, otoacoustic emissions and pure tone audiological evaluation, using the Visual Reinforcement Audiometry technique. RESULTS: the test battery showed that children have sensorineural hearing loss (cochlear) with steeply sloping configuration, so that the presence of cochlear microphonics in the click-ABR was probably generated by the preservation of the cochlea in the low frequencies. CONCLUSION: the cases demonstrated that the absence of the otoacustic emissions, and presence of cochlear microphonics should not be interpreted as Auditory Neuropathy Spectrum Disorder, since that these conditions are similar to steeply sloping cochlear hearing loss.

Potenciais Evocados Auditivos de Estado Estável no diagnóstico audiológico infantil: uma comparação com os Potenciais Evocados Auditivos de Tronco Encefálico / Steady-state auditory evoked responses in audiological diagnosis in children: a comparison with brainstem evoked auditory responses

Os Potenciais Evocados Auditivos de Estado Estável (PEAEE) têm sido apontados como uma técnica promissora na avaliação audiológica infantil. Objetivo: Investigar o nível de concordância entre os resultados dos PEAEE e dos Potenciais Evocados Auditivos de Tronco Encefálico (PEATE-clique) em um grupo de crianças com perda auditiva sensorioneural, averiguando assim a aplicabilidade clínica desta técnica na avaliação audiológica infantil. Forma de estudo: Clínico prospectivo de coorte transversal. Material e método: 15 crianças com idade entre dois e 36 meses e diagnóstico de perda auditiva sensorioneural. A concordância entre as respostas dos dois testes foi avaliada por meio do coeficiente de correlação intraclasse e o teste de McNemar comparou os dois testes quanto à probabilidade de ocorrência de resposta. Resultados: Os coeficientes de correlação encontrados foram 0,70; 0,64; 0,49; 0,69; 0,63 e 0,68 respectivamente para as frequências de 1, 2, 4, 1-2, 2-4 e 1-2-4kHz. No teste de McNemar foi obtido p=0.000, indicando que a probabilidade de se obter resposta presente nos dois testes não é igual, sendo maior nos PEAEE. Conclusão: A boa concordância observada entre as técnicas sugere que um exame pode ser complementar ao outro. Os PEAEE, entretanto, promoveram informações adicionais nos casos de perdas severas e profundas, acrescentando dados importantes para a reabilitação destas crianças e proporcionando maior precisão no diagnóstico audiológico.

Auditory Steady-State Responses (ASSR) are being recognized as a promising technique in the assessment of hearing in children. AIM: To investigate the agreement level between results obtained from ASSR and click-ABR in a group of children with sensorineural hearing loss, in order to study the clinical applicability of this technique to evaluate the hearing status in young children. Study Design: clinical prospective with a cross-sectional cohort. Materials and methods: 15 children aged between two and 36 months and with diagnosis of sensorineural hearing loss. The correlation between the responses of the two tests was evaluated by intraclass correlation coefficient and McNemar test comparing the probability of responses in both tests. Results: The correlation coefficients were: 0.70; 0.64; 0.49; 0.69; 0.63 and 0.68 respectively for frequencies of 1, 2, 4, 1-2, 2-4 and 1-2-4kHz. In McNemar test p = 0.000, indicating that the probability of obtaining responses in both tests was not equal, but greater for the ASSR. Conclusion: we found good agreement between the techniques among the four frequencies evaluated, suggesting that both tests may be complementary. However, the ASSR was able to obtain additional information in cases of severe and profound hearing losses, adding important data to the management of these children, providing greater accuracy to the audiological diagnosis.

Efeito de supressão das emissões otoacústicas evocadas por estímulo transiente em lactentes de risco para perda auditiva nascidos pré-termo / Transient otoacoustic emissions suppression in pre-term infants with high risk for hearing loss

OBJETIVO: verificar a ocorrência e a magnitude do efeito de supressão das emissões otoacústicas evocadas por estímulo transiente, em lactentes nascidos pré-termo, com indicadores de risco para perda auditiva. MÉTODOS: a supressão das emissões otoacústicas evocadas por estímulo transiente foi realizada em 15 lactentes nascidos pré-termo. O equipamento utilizado foi o ILO USB II V6 Clinical OAE, de marca Ododynamics. O estímulo clique linear foi apresentado a 60 dB pe NPS (± 5) e o ruído contralateral (ruído branco) a 60 dB NPS (± 5). RESULTADOS: Dos 15 lactentes, apenas um não apresentou supressão bilateralmente. Dentre os 14, quatro não apresentaram supressão na orelha esquerda. A ocorrência do efeito de supressão foi de 93,3 por cento na população estudada, sendo 71,4 por cento de efeito bilateral e 28,6 por cento de efeito unilateral. Os resultados indicaram diferença estatisticamente significante entre as orelhas. Não foram observadas diferenças estatisticamente significantes com relação as variáveis idade atual e idade gestacional. Os lactentes do sexo masculino apresentaram respostas maiores quando comparados aos do sexo feminino. CONCLUSÕES: a maioria da população estudada apresentou efeito de supressão das emissões otoacústicas. O ruído branco apresentado contralateralmente reduziu o nível de resposta das EOAT, demonstrando a participação do sistema eferente medial no efeito de supressão. As respostas foram diferentes segundo as variáveis lado da orelha e sexo, sendo maiores na orelha direita e no sexo masculino.

PURPOSE: to study the occurrence and magnitude of the transient otoacoustic emissions suppression in pre-term infants with high risk for hearing loss. METHODS: the study was carried out in 15 pre-term infants. The used equipment was ILO USB II V6 Clinical OAE Software from Ododynamics. The used stimulation was linear clicks displayed at the intensity of 60 dB pe SPL (± 5). The contralateral noise (white noise) was set at 60 dB SPL (± 5). RESULTS: From 15 infants, only one did not show bilateral suppression. Suppression effect was shown on 93.3 percent of the subjects, being 71.4 percent for both ears and 28.6 percent for only one ear. The results showed that there was a significant statistical difference between the right and left ears. We could not observe significant differences when actual and gestational age were analyzed. The baby boys showed higher responses when compared to baby girls. CONCLUSIONS: most of the studied population showed OAE suppression effect. White noise was found in the contralateral ear, and reduced the response level of the transient otoacoustic emissions, demonstrating the participation of the efferent system. The responses were different considering the side of the ear, being higher on the right ear and for boys.

Potenciais evocados auditivos de tronco encefálico por frequência específica e de estado estável na audiologia pediátrica: estudo de caso / Frequency-specific and steady-state evoked auditory brainstem responses in pediatric audiology: case study

Preconiza-se o diagnóstico até os três meses de idade em crianças com deficiência auditiva congênita. Após a etapa inicial de confirmação do diagnóstico, é necessário que se obtenha limiares precisos nas diferentes frequências, para que seja possível uma adequada seleção, indicação e regulagem de aparelhos de amplificação sonora. Nesse contexto, inserem-se os Potenciais Evocados Auditivos de Tronco Encefálico por Frequência Específica (PEATE-FE) e, mais recentemente, os Potenciais Evocados Auditivos de Estado Estável (PEAEE). O objetivo deste estudo de caso foi apresentar os achados das duas técnicas para estimar os limiares auditivos em uma criança de três meses de idade, com perda auditiva neurossensorial bilateral, diagnosticada utilizando-se como primeiro método de avaliação os Potenciais Evocados Auditivos de Tronco Encefálico com estímulo clique, tanto por via aérea como por via óssea. As duas técnicas mostraram-se eficientes para estimar os limiares auditivos, com uma vantagem dos PEAEE com relação ao tempo de duração de exame.

It is recommended that congenital hearing loss is identified as early as three months old. After the initial step of confirming the diagnosis, it is necessary to obtain accurate hearing thresholds, allowing an adequate selection, indication and regulation of hearing aids for these children. It is inserted, in this context, the Frequency-Specific Auditory Brainstem Responses (FSABR) and, more recently, the Auditory Steady-State Responses (ASSR). The aim of the present study was to describe the findings of the use of both techniques to estimate the auditory thresholds of a three-month-old infant with bilateral sensorineural hearing loss diagnosed using, as primary evaluation method, the click-evoked Auditory Brainstem Responses, with both air and bone stimuli conduction. Both techniques provided reliable findings for estimating auditory thresholds. The ASSR had an advantage regarding the duration of the evaluation.