Evaluation of newborn hearing screening program / Avaliação de um programa de triagem auditiva neonatal

ABSTRACT OBJECTIVE To evaluate Newborn Hearing Screening Program of Hospital Regional de Sobradinho, from January 2016 to December 2017, according to Multiprofessional Committee on Auditory Health parameters and Joint Committee on Infant Hearing (JCIH) recommendations, as well as to describe the prevalence of risk factors for hearing loss within the study population and their impact on the respective program. METHOD This is a quantitative, cross-sectional and retrospective study that carefully analyzed registration books of screened newborns. It was established the prevalence of "pass" and "fail" in test and retest, retest percentage of attendance and referral for audiological diagnosis. Risk factors for hearing loss were described, as well as their influence on "pass" and "fail" rates. Inferential statistical analysis was performed using chi-square test and Anderson-Darling test, with 5% reliability index. RESULTS A total of 3,981 newborns were screened; 2,963 (74.4%) presented no risk factors whereas 1,018 (25.6%) did, prematurity being the most frequent (51.6%). In the test, 166 (4.2%) failed and 118 (71.1%) attended the retest. The referral rate for diagnosis was 0.3%. CONCLUSION Regarding the percentage of referral for diagnosis, the program reached indexes recommended by the Joint Committee on Infant Hearing and Multiprofessional Committee on Auditory Health. The most prevalent risk factor within the population was prematurity.

RESUMO OBJETIVO Avaliar o Programa de Triagem Auditiva Neonatal do Hospital Regional de Sobradinho, no período de janeiro de 2016 a dezembro de 2017, segundo os parâmetros do Comitê Multiprofissional em Saúde Auditiva e as recomendações do Joint Committee on Infant Hearing (JCIH), bem como descrever a prevalência dos indicadores de risco para deficiência auditiva na população estudada e seu impacto no respectivo programa. MÉTODO Trata-se de um estudo quantitativo, transversal e retrospectivo no qual foram analisados criteriosamente livros de registros dos neonatos triados. Foi estabelecida a prevalência de "passa" e "falha" no teste e reteste, o percentual de comparecimento para reteste e de encaminhamento para diagnóstico audiológico. Foram descritos os indicadores de risco para deficiência auditiva, bem como sua influência nos índices de "passa" e "falha". Foi realizada análise estatística inferencial utilizando o teste do qui-quadrado e o teste de Anderson-Darling, com índice de confiabilidade de 5%. RESULTADOS Foram triados 3.981 neonatos, 2.963 (74,4%) dos quais sem indicadores de risco e 1.018 (25,6%) com, sendo a prematuridade o mais frequente (51,6%). No teste, 166 (4,2%) falharam e 118 (71,1%) compareceram para o reteste. O índice de encaminhamento para diagnóstico foi de 0,3%. CONCLUSÃO O programa atingiu os índices recomendados pelo Joint Committee on Infant Hearing e pelo Comitê Multiprofissional em Saúde Auditiva quanto à porcentagem de encaminhamento para diagnóstico. O indicador de risco mais prevalente na população foi a prematuridade.

Caracterización clínica del síndrome de Usher. Provincia Holguín / Usher syndrome clinical characterization. Holguin province

Fundamento: El síndrome de Usher es una enfermedad determinada genéticamente, con una gran heterogeneidad clínica y genética; está caracterizada por hipoacusia neurosensorial de moderada a severa, retinosis pigmentaria progresiva y puede acompañarse de alteración vestibular. Por la alta prevalencia de esta enfermedad en la provincia de Holguín, se considera necesario este estudio. Objetivo: Caracterizar clínicamente todos los enfermos con diagnóstico clínico de síndrome de Usher en la provincia Holguín, en el período de enero del 2009 a enero del 2016. Metodología: Se realizó un estudio descriptivo, retrospectivo, tipo serie de casos, a los 53 pacientes con diagnóstico clínico de síndrome de Usher en la provincia Holguín. La muestra estuvo formada por los 53 enfermos residentes en la provincia. Se revisaron los registros del Centro Provincial de Retinosis Pigmentaria y las historias clínicas de estos pacientes; se recogieron los datos de interés en un instrumento que se confeccionó para ello. Las variables estudiadas fueron el sexo, la edad, edad del diagnóstico de la hipoacusia y severidad, edad del diagnóstico de la retinosis pigmentaria y los resultados de las pruebas audiológicas, lo que permitió conocer la función vestibular. Resultados: Se caracterizó clínicamente el 100 % de los enfermos estudiados. Predominó el sexo masculino (60,37 %). El 80 % presentó la retinosis pigmentaria en la primera infancia y la hipoacusia congénita profunda en 67,92 %. Las pruebas vestibulares demostraron que el 71,70 % presenta síndrome de Usher tipo II y el 28,30 % tiene el tipo I. Conclusiones: Predominó el sexo masculino, la hipoacusia precedió a la alteración visual. Se logró caracterizar clínicamente a estos afectados. Prevaleció el síndrome de Usher tipo II.

Background: Usher syndrome is a genetically determined disease with great clinical and genetic heterogeneity. This disease is characterized by sensorineural hearing loss of moderate to severe, progressive pigmentosa retinitis and may be accompanied by vestibular alteration. At the high prevalence of this disease in the province of Holguin, this study is considered necessary. Objective: To characterize all patients clinically with clinical diagnosis of Usher syndrome in Holguin province, in the period from January 2009 to January 2016. Methodology: A series types of retrospective cases, descriptive study with 53 patients with clinical diagnosis of Usher syndrome in Holguin province was conducted. The sample consisted of 53 patients residing in the province. Provincial records Pigmentosa Retinitis Pigmentosa Center and the medical records of these patients were reviewed, the data of interest are collected in an instrument that was drawn up for these. The variables studied were sex, age, age at diagnosis of hearing loss and severity, age of diagnosis of pigmentosa retinitis and the results of the audiological tests, allowing knowing the vestibular function. Results: It was possible to clinically characterize 100 % of the patients studied, predominantly male in a 60.37 %. 80 % had pigmentosa retinitis in early childhood and profound congenital hearing loss in 67.92 %. Vestibular tests showed that 71. 70 % have Usher syndrome type II and 28.30 % have the type I. Conclusions: mainly males, hearing loss preceded visual impairment. It was possible to clinically characterize those affected. It prevailed Usher syndrome type II.

Analysis of imaging of inner ear malformations in children with congenital sensorineural hearing loss / 上海第二医科大学学报

Objective To study the imaging of inner ear malformations in children with congenital sensorineural hearing loss(SNHL).Methods CT and MRI examinations were performed on children with SNHL diagnosed by audiological test.One hundred and eighty-eight patients with complete imaging information were obtained.The imaging of inner ear malformations was analyzed according to Sennaroglu's classification. Results Thirty-five patients(54 ears) were found with inner ear malformations by CT and MRI,3 of whom(5 ears) were accompanied by outer and middle ear malformations.Among the 35 patients,2(4 ears) were found to be common cavity deformity,1(1 ear) cochlear hypoplasia,13(26 ears) incomplete partition II(Mondini deformity),4(7 ears) vestibule dilation,13(19 ears) semicircular canal deformity and 19(34 ears) large vestibular aqueduct.Internal auditory canal was found narrow in 7 patients(10 ears) and wide in 1(2 ears) with classic Mondini deformity. Conclusion CT and MRI examinations are of great importance to the diagnosis and treatment of inner ear malformations in children with congenital SNHL,especially for the candidates of cochlear implantation.