RESUMO Objetivo Determinar os indicadores de risco para deficiência auditiva, que afetam as diferentes etapas de avaliação de um programa de triagem auditiva neonatal universal. Métodos Estudo retrospectivo longitudinal de triagem auditiva neonatal, realizado em 832 neonatos nascidos em hospital público terciário, no período de janeiro a dezembro de 2012. O exame de Emissões Otoacústicas Evocadas por Estímulo Transiente foi realizado na primeira avaliação auditiva de todos os neonatos. Nos casos de “falha” na primeira avaliação, foi aplicado um reteste e, quando houve a permanência da “falha”, foi realizado o Potencial Evocado Auditivo de Tronco Encefálico (PEATE). Os neonatos com indicador de risco para deficiência auditiva realizaram o PEATE independente do resultado das emissões otoacústicas. Resultados A presença de, ao menos, um indicador de risco para deficiência auditiva, associado ou não a malformações craniofaciais, síndromes genéticas e peso menor que 1500 g ao nascimento, aumentaram significativamente as chances de “falha” na avaliação por emissões otoacústicas. Meningite e malformações craniofaciais aumentaram de maneira expressiva as chances de PEATE alterado. Dois neonatos com emissões otoacústicas normais apresentaram diagnóstico de neuropatia auditiva. Conclusão A malformação craniofacial é um indicador fortemente associado ao diagnóstico da surdez, independente de a triagem auditiva ter sido realizada por emissões otoacústicas ou por PEATE em suas diferentes etapas, o que justifica o monitoramento contínuo e sistemático do serviço de triagem, na busca da melhoria da qualidade do programa de saúde auditiva do neonato.

ABSTRACT Purpose To determine risk indicators for hearing loss affecting different evaluation steps of a universal newborn hearing screening program. Methods Longitudinal, retrospective study of newborn hearing screening in a tertiary public hospital, including 832 newborns born between January and December 2012. Transient evoked otoacoustic emissions were measured at the first hearing evaluation in all newborns and retested in cases of “failure”. When a “failure” result persisted, auditory brainstem responses (ABR) was performed. All newborns with risk indicator for hearing loss were evaluated with ABR screening regardless of the outcome of the otoacoustic emissions test. Results The presence of at least one risk indicator for hearing loss, associated or not with craniofacial malformations, genetic syndromes and birth weight below 1500 g significantly increased the chances of “failure” in the otoacoustic emissions test. Meningitis and craniofacial malformations significantly increased the odds of an abnormal ABR. Two newborns with normal otoacoustic emissions were diagnosed with auditory neuropathy. Conclusion Craniofacial malformation was an indicator strongly associated with a diagnosis of deafness, regardless of the hearing screening being performed by otoacoustic emissions or ABR at different steps of a universal newborn hearing screening program. This finding justifies continuous and systematic monitoring of the screening service seeking quality improvement of the newborn health hearing program.

INTRODUÇÃO: O diagnóstico precoce da surdez minimiza impactos no desenvolvimento infantil. Fatores que interferem na efetividade dos programas de triagem são estudados.OBJETIVO: Verificar a relação entre sexo, peso ao nascimento, idade gestacional, presença de risco para deficiência auditiva, local de realização da triagem auditiva neonatal e resultados "passa" e "falha" no reteste.MÉTODO: Estudo de coorte prospectiva, em hospital de referência terciário. A triagem foi realizada em 565 neonatos, por meio das emissões otoacústicas evocadas transientes, em três unidades de internação antes da alta hospitalar e o reteste, no ambulatório. Sexo, peso ao nascimento, idade gestacional, presença de indicadores de risco para deficiência auditiva e local de realização do exame foram considerados.RESULTADOS: Nasceram a termo 86%, prematuros 14% e risco para deficiência auditiva, 11%. Dentre os 165 neonatos retestados, apenas o local de realização do exame, Unidade de Cuidados Intermediários, se relacionou com manutenção da "falha" no reteste.CONCLUSÕES: Sexo, peso ao nascimento, idade gestacional e presença de indicadores de risco para deficiência auditiva não se relacionaram com "passar" e/ou "falhar" no reteste. A realização do exame em unidades de cuidados intermediários aumenta a chance de permanência de "falha" no exame de Emissões Otoacústicas Evocadas Transientes.

INTRODUCTION: Early diagnosis of hearing loss minimizes its impact on child development. We studied factors that influence the effectiveness of screening programs.OBJECTIVE: To investigate the relationship between gender, weight at birth, gestational age, risk factors for hearing loss, venue for newborn hearing screening and "pass" and "fail" results in the retest.METHODS: Prospective cohort study was carried out in a tertiary referral hospital. The screening was performed in 565 newborns through transient evoked otoacoustic emissions in three admission units before hospital discharge and retest in the outpatient clinic. Gender, weight at birth, gestational age, presence of risk indicators for hearing loss and venue for newborn hearing screening were considered.RESULTS: Full-term infants comprised 86% of the cases, preterm 14%, and risk factors for hearing loss were identified in 11%. Considering the 165 newborns retested, only the venue for screening, Intermediate Care Unit, was related to "fail" result in the retest.CONCLUSIONS: Gender, weight at birth, gestational age and presence of risk factors for hearing loss were not related to "pass" and/or "fail" results in the retest. The screening performed in intermediate care units increases the chance of continued "fail" result in the Transient Otoacoustic Evoked Emissions test.

<sec><title>Introduction</title><p> Literature data are not conclusive as to the influence of neonatal complications in the maturational process of the auditory system observed by auditory brainstem response (ABR) in infants at term and preterm.</p></sec><sec><title>Objectives</title><p> Check the real influence of the neonatal complications in infants by the sequential auditory evaluation.</p></sec><sec><title>Methods</title><p> Historical cohort study in a tertiary referral center. A total of 114 neonates met inclusion criteria: treatment at the Universal Neonatal Hearing Screening Program of the local hospital; at least one risk indicator for hearing loss; presence in both evaluations (the first one after hospital discharge from the neonatal unit and the second one at 6 months old); all latencies in ABR and transient otoacoustic emissions present in both ears.</p></sec><sec><title>Results</title><p> The complications that most influenced the ABR findings were Apgar scores less than 6 at 5 minutes, gestational age, intensive care unit stay, peri-intraventricular hemorrhage, and mechanical ventilation.</p></sec><sec><title>Conclusion</title><p> Sequential auditory evaluation is necessary in premature and term newborns with risk indicators for hearing loss to correctly identify injuries in the auditory pathway.</p></sec>

INTRODUÇÃO: Pacientes submetidos a transplante de células hematopoiéticas recebem altas doses de quimioterapia e radioterapia que podem causar imunossupressão e doenças orais graves.OBJETIVO: Apresentar um protocolo de avaliação de doenças orais antes e após transplante de células hematopoiéticas.MÉTODO: Estudo clínico prospectivo de 65 pacientes com idade acima de 18 anos, com doenças hematológicas submetidas a transplante de células hematopoiéticas, divididos em dois grupos: A (transplante alogênico, 34 pacientes) e B (transplante autólogo). Foram realizadas três avaliações odontológicas: período antes do transplante (momento 1), uma semana (momento 2) e 100 dias após o transplante (momento 3). Em cada momento as alterações orais foram pontuadas e classificadas como leve, moderada e grave.RESULTADOS: As alterações orais mais frequentes foram: gengivite, pericoronite do terceiro molar e úlceras. Entretanto nos momentos dois e três a principal doença foi a mucosite associada a toxicidades das drogas usadas na imunossupressão.CONCLUSÃO: Mucosite foi principal alteração, com a pontuação mais alta e com maior risco de complicações. Gengivites, úlceras e pericoronites foram outras alterações com risco menor de complicações.

INTRODUCTION: Patients undergoing hematopoietic stem cell transplantation receive high doses of chemotherapy and radiotherapy, which cause severe immunosuppression.OBJECTIVE: To report an oral disease management protocol before and after hematopoietic stem cell transplantation.METHODS: A prospective study was carried out with 65 patients aged > 18 years, with hematological diseases, who were allocated into two groups: A (allogeneic transplant, 34 patients); B (autologous transplant, 31 patients). A total of three dental status assessments were performed: in the pre-transplantation period (moment 1), one week after stem cell infusion (moment 2), and 100 days after transplantation (moment 3). In each moment, oral changes were assigned scores and classified as mild, moderate, and severe risks.RESULTS: The most frequent pathological conditions were gingivitis, pericoronitis in the third molar region, and ulcers at the third moment assessments. However, at moments 2 and 3, the most common disease was mucositis associated with toxicity from the drugs used in the immunosuppression.CONCLUSION: Mucositis accounted for the increased score and potential risk of clinical complications. Gingivitis, ulcers, and pericoronitis were other changes identified as potential risk factors for clinical complications.

Introduction The first locus for nonsyndromic autosomal recessive hearing loss is on chromosome 13q11-22. The 35delGmutation is present in 80% of cases in which GJB2 is involved, which makes the study of this mutation very important. The viability and benefits of screening for mutations in the connexin 26 gene are now beginning to change the diagnostic evaluation and identification of the etiology of hearing loss. Objective To investigate the occurrence of the 35delG mutation in patients with nonsyndromic sensorineural hearing loss and their first degree relatives. Methods This transversal study included 72 patients from the local hospital. The patients were divided into three groups: group A, sensorineural hearing loss (n = 58); group B, first-degree relatives of group A with sensorineural hearing loss (n = 09); and group C, first-degree relatives of patients from group A without hearing loss (n = 05). All patients had audiological evaluation and genetic testing of the 35delG mutation. Results The 35delG mutation was found in four heterozygous mutations (three of them found in the same family). The other heterozygous mutation was found in a female patient with bilateral, moderate, prelingual, sensorineural hearing loss. A single homozygous mutation was found in a male patient, with severe sensorineural hearing loss in his right ear and profound hearing loss in the left ear. Conclusions The 35delG mutation was found in 7% of the cases. The test is easy to perform and inexpensive, but it is necessary to investigate other genes related to hearing loss.

Introdução: Doença renal crônica (DRC) é definida pela presença de lesão renal levando à perda lenta e progressiva da função renal. Objetivo: Comparar testes auditivos entre pacientes com DRC submetidos a diferentes método de tratamento. Material e método: Estudo clínico transversal. Os grupos foram divididos de acordo com o método de tratamento: hemodiálise (n = 35), diálise peritoneal (n =15), conservador (n = 51) e 27 pacientes saudáveis (controle). Pacientes com idade superior a 60 anos, perda auditiva congênita, síndromes genéticas, infecções de orelha média e transplante renal foram excluídos da pesquisa. A avaliação audiológica incluiu audiometria tonal, emissões otoacústicas evocadas transientes e Potencial Evocado Auditivo de Tronco Encefálico (PEATE); e as variáveis avaliadas foram: sexo, idade, diagnóstico de hipertensão arterial e diabetes, estádio da DRC, tempo de diagnóstico do diabetes e da hipertensão arterial, duração da DRC e do tratamento. Resultados: A idade, presença de hipertensão arterial e tempo de DRC foram estatisticamente significantes e controlados. O grupo conservador apresentou piores limiares auditivos na audiometria tonal e o intervalo III-V do PEATE significativamente maior que o da hemodiálise. Conclusão: O tratamento conservador mostrou piores resultados na avaliação auditiva, independente de diabetes e de hipertensão, reforçando que os pacientes submetidos a tratamento para DRC devem realizar avaliação auditiva completa para melhor compreensão da doença e de seus efeitos sobre o sistema auditivo.

Introduction: Chronic kidney disease (CKD) is defined as the presence of renal injury that leads to the slow and progressive loss of kidney function. Aim: To compare audiological tests between patients with CKD receiving different types of treatment. Material and method: This was a clinical and experimental study. Groups were divided according to treatment: hemodialysis (n = 35), peritoneal dialysis (n = 15), and conservative (n = 51), and were compared to 27 healthy controls. Patients older than 60 years; those with congenital hearing loss, genetic syndromes, and middle-ear infections; and those who had been submitted to a kidney transplant were excluded. Audiologic evaluation included pure-tone audiometry, transient evoked otoacoustic emissions, and auditory brainstem response (ABR). The variables considered were gender, age, diagnosis of arterial hypertension, time since the diagnosis of diabetes and hypertension, CKD stage, duration of CKD, and duration of treatment. Results: The variables age, presence of arterial hypertension, and time of CKD were statistically significant and controlled. The auditory thresholds measured by pure-tone threshold audiometry were worse for the conservative treatment group, and the III-V interval of the ABR of the conservative treatment group was significantly greater than that of the hemodialysis groups. Conclusion: The conservative treatment group presented worse audiological tests, regardless of hypertension and diabetes, reinforcing that patients need to undergo a complete hearing assessment for better understanding of the disease and its effects on the auditory system.

OBJETIVO: Relatar o caso de um lactente com citomegalovírus congênito e disacusia neurossensorial progressiva, analisado por três métodos de avaliação auditiva. DESCRIÇÃO DO CASO: Na primeira avaliação auditiva, aos quatro meses de idade, o lactente apresentou ausência de Emissões Otoacústicas (EOA) e Potencial Evocado Auditivo de Tronco Encefálico (PEATE) dentro dos padrões de normalidade para a faixa etária, com limiar eletrofisiológico em 30dBnHL, bilateralmente. Com seis meses, apresentou ausência de PEATE bilateral em 100dBnHL. A avaliação comportamental da audição mostrou-se prejudicada devido ao atraso no desenvolvimento neuropsicomotor. Aos oito meses, foi submetido ao exame de Resposta Auditiva de Estado Estável (RAEE) e os limiares encontrados foram 50, 70, ausente em 110 e em 100dB, respectivamente para 500, 1.000, 2.000 e 4.000Hz, à direita, e 70, 90, 90 e ausente em 100dB, respectivamente para 500, 1.000, 2.000 e 4.000Hz, à esquerda. COMENTÁRIOS: Na primeira avaliação, o lactente apresentou alteração auditiva no exame de EOA e PEATE normal, que passou a ser alterado aos seis meses de idade. A intensidade da perda auditiva só pôde ser identificada pelo exame de RAEE, permitindo estabelecer a melhor conduta na adaptação de aparelho de amplificação sonora individual. Ressalta-se a importância do acompanhamento audiológico para crianças com CMV congênito.

OBJECTIVE: To report an infant with congenital cytomegalovirus and progressive sensorineural hearing loss, who was assessed by three methods of hearing evaluation. CASE DESCRIPTION: In the first audiometry, at four months of age, the infant showed abnormal response in Otoacoustic Emissions and normal Auditory Brainstem Response (ABR), with electrophysiological threshold in 30dBnHL, in both ears. With six months of age, he showed bilateral absence of the ABR at 100dBnHL. The behavioral observational audiometry was impaired due to the delay in neuropsychomotor development. At eight months of age, he was submitted to Auditory Steady State Response (ASSR) and the thresholds were 50, 70, absent in 110 and in 100dB, respectively for 500, 1,000, 2,000 and 4,000Hz in the right ear, and 70, 90, 90 and absent in 100dB, respectively for 500, 1,000, 2,000 and 4,000Hz in the left ear. COMMENTS: In the first evaluation, the infant had abnormal Otoacoustic Emission and normal ABR, which became altered at six months of age. The hearing loss severity could be identified only by the ASSR, which allowed the best procedure for hearing aids adaptation. The case description highlights the importance of the hearing status follow-up for children with congenital cytomegalovirus.

OBJETIVO: Relatar el caso de un lactante con citomegalovirus congénito y disacusianeurosensorial progresiva, analizado por tres métodos de evaluación auditiva. DESCRIPCIÓN DEL CASO: En la primera evaluación auditiva, a los cuatro meses de edad, el lactante presentó ausencia de Emisiones Otoacústicas (EOA) y Potencial Evocado Auditivo de Tronco Encefálico (PEATE) dentro de los estándares de normalidad para la franja de edad, con umbral electrofisiológico en 30dBnHL, bilateralmente. Con seis meses, presentó ausencia de PEATE bilateral en 100dBnHL. La evaluación comportamental de la audición se mostró perjudicada debido al retardo en el desarrollo neuropsicomotor. A los ocho meses, fue sometido al examen de Respuesta Auditiva de Estado Estable (RAEE) y los umbrales encontrados fueron 50, 70, ausente en 110 y en 100dB, respectivamente para 500, 1.000, 2.000 y 4.000Hz, a la derecha, y 70, 90, 90 y ausente en 100dB, respectivamente para 500, 1.000, 2.000 y 4.000, a la izquierda. COMENTARIOS: En la primera evaluación, el lactante presentó alteración auditiva en el examen de EOA y PEATE normal, que pasó a ser alterado a los seis meses de edad. La intensidad de la pérdida auditiva solo puede identificarse por el examen de RAEE, permitiendo establecer la mejor conducta en la adaptación de aparato de amplificación sonora individual. Se subraya la importancia del seguimiento audiológico para niños con CMV congénito.