RESUMEN

INTRODUCTION: Congenital Zika virus syndrome is a distinct pattern of birth defects in fetuses infected by the Zika virus. It presents a broad clinical spectrum that includes occurrences of microcephaly, hypertonia, dysphagia, hyperexcitability, seizures, and arthrogryposis. Imaging findings show neuronal migration disorders. METHODOLOGY: Case reports have suggested that arthrogryposis has a neurogenic cause. We analyzed needle electromyography and nerve conduction examinations on 77 patients aged 2-24 months presenting highly probable congenital Zika virus syndrome, with or without arthrogryposis. RESULTS: All those with arthrogryposis presented with chronic muscle denervation in the electromyography examination. Similarly, children with single or reversible joint abnormalities at birth showed the same findings. Denervation in the paravertebral musculature was found in all of the children with diaphragmatic paralysis or thoracic deformities. CONCLUSIONS: We propose that congenital contractures associated with congenital Zika virus syndrome are caused by the malformation of upper and lower motor neurons during embryogenesis.

Asunto(s)

RESUMEN

BACKGROUND: the auditory steady state response (ASSR) is an electrophysiological procedure that evaluates, at the same time, the hearing thresholds of both ears at various frequencies. This procedure reduces the time of testing and enables the stimulation of levels close to 125dB HL, characterizing residual hearing. AIM: to verify the applicability of the ASSR to determine the hearing thresholds in different levels of sensorineural hearing loss. METHOD: 48 individuals aged 7-30 years, with different levels of hearing loss were assessed. The Pure tone Audiometry (PTA) and the ASSR were carried out in following carrier frequencies 0.5; 1; 2 and 4k Hz. The carrier frequencies in the ASSR were modulated in amplitude and frequency, with multiple dicotic stimulation for mild and moderate hearing loss. Simple stimulation was used to test the other hearing deficit levels. RESULTS: there was a significant association (p<0.01) between the thresholds obtained in the PTA and in the ASSR for all of the tested frequencies, especially for the severe levels. However, for some patients, the hearing loss level was overestimated in the ASSR. CONCLUSION: the ASSR can be used to estimate the PTA threshold; nevertheless, it should not be analyzed in an isolated way, but seen as a complementary procedure to the behavioral auditory assessment.

Asunto(s)

RESUMEN

TEMA: a resposta auditiva de estado estável (RAEE) é um procedimento eletrofisiológico que permite avaliar ao mesmo tempo os limiares auditivos de várias freqüências em ambas às orelhas, reduzindo assim o tempo de teste, e permite estimular até níveis próximos a 125dB HL, caracterizando assim a audição residual. OBJETIVO: verificar a aplicabilidade da RAEE para determinar os limiares auditivos nos diferentes graus de perda auditiva neurossensorial coclear. MÉTODO: foram avaliados 48 indivíduos com idade entre sete e trinta anos e diferentes graus de perdas auditivas. A Audiometria Tonal Liminar (ATL) e a RAEE foram avaliadas nas seguintes freqüências portadoras, 0,5; 1; 2 e 4k Hz. As freqüências portadoras na RAEE foram moduladas em amplitude e freqüência, com estimulação múltipla e dicótica nas perdas auditivas de grau leve e moderado. Estimulação simples foi utilizada nos outros graus de perdas auditivas. RESULTADOS: houve associação significante (p < 0,01) entre os limiares obtidos na ATL e RAEE para todas as freqüências testadas, principalmente para perdas auditivas de grau profundo. Contudo em alguns pacientes o grau da perda auditiva pode ser super-estimado. CONCLUSÃO: a RAEE pode ser utilizada para predizer os limiares auditivos da ATL, porém não deve ser analisada de forma isolada, mas de forma complementar a avaliação audiológica comportamental.

BACKGROUND: the auditory steady state response (ASSR) is an electrophysiological procedure that evaluates, at the same time, the hearing thresholds of both ears at various frequencies. This procedure reduces the time of testing and enables the stimulation of levels close to 125dB HL, characterizing residual hearing. AIM: to verify the applicability of the ASSR to determine the hearing thresholds in different levels of sensorineural hearing loss. METHOD: 48 individuals aged 7-30 years, with different levels of hearing loss were assessed. The Pure tone Audiometry (PTA) and the ASSR were carried out in following carrier frequencies 0.5; 1; 2 and 4k Hz. The carrier frequencies in the ASSR were modulated in amplitude and frequency, with multiple dicotic stimulation for mild and moderate hearing loss. Simple stimulation was used to test the other hearing deficit levels. RESULTS: there was a significant association (p<0.01) between the thresholds obtained in the PTA and in the ASSR for all of the tested frequencies, especially for the severe levels. However, for some patients, the hearing loss level was overestimated in the ASSR. CONCLUSION: the ASSR can be used to estimate the PTA threshold; nevertheless, it should not be analyzed in an isolated way, but seen as a complementary procedure to the behavioral auditory assessment.

Asunto(s)