Abstract Objectives: To review and provide evidence-based recommendations for the diagnosis and treatment of otosclerosis. Methods: Task force members were educated on knowledge synthesis methods, including electronic database search, review and selection of relevant citations, and critical appraisal of selected studies. Articles written in English or Portuguese on otosclerosis were eligible for inclusion. The American College of Physicians’ guideline grading system and the American Thyroid Association’s guideline criteria were used for critical appraisal of evidence and recommendations for therapeutic interventions. Results: The topics were divided into 2 parts: 1) Diagnosis – audiologic and radiologic; 2) Treatment – hearing AIDS, pharmacological therapy, stapes surgery, and implantable devices - bone-anchored devices, active middle ear implants, and Cochlear Implants (CI). Conclusions: The pathophysiology of otosclerosis has not yet been fully elucidated, but environmental factors and unidentified genes are likely to play a significant role in it. Women with otosclerosis are not at increased risk of worsening clinical condition due to the use of contraceptives or during pregnancy. Drug treatment has shown little benefit. If the patient does not want to undergo stapedotomy, the use of hearing aids is well indicated. Implantable systems should be indicated only in rare cases, and the CI should be indicated in cases of profound deafness.

Abstract Objectives: To provide an overview of the main evidence-based recommendations for the diagnosis of hearing loss in children and adolescents aged 0–18 years. Methods: Task force members were educated on knowledge synthesis methods, including electronic database search, review and selection of relevant citations, and critical appraisal of selected studies. Articles written in English or Portuguese on childhood hearing loss were eligible for inclusion. The American College of Physicians’ guideline grading system and the American Thyroid Association’s guideline criteria were used for critical appraisal of evidence and recommendations for therapeutic interventions. Results: The topics were divided into 2 parts: (1) treatment of sensorineural hearing loss: individual hearing aids, bilateral cochlear implants, cochlear implants in young children, unilateral hearing loss, and auditory neuropathy spectrum disorder; and (2) treatment of conductive/mixed hearing loss: external/middle ear malformations, ventilation tube insertion, and tympanoplasty in children. Conclusions: In children with hearing loss, in addition to speech therapy, Hearing AIDS (HAs) or implantable systems may be indicated. Even in children with profound hearing loss, both the use of HAs and behavioral assessments while using the device are important.

Abstract Objectives: To provide an overview of the main evidence-based recommendations for the diagnosis of hearing loss in children and adolescents aged 0 to 18 years. Methods: Task force members were educated on knowledge synthesis methods, including electronic database search, review and selection of relevant citations, and critical appraisal of selected studies. Articles written in English or Portuguese on childhood hearing loss were eligible for inclusion. The American College of Physicians’ guideline grading system and the American Thyroid Association’s guideline criteria were used for critical appraisal of evidence and recommendations for therapeutic interventions. Results: The evaluation and diagnosis of hearing loss: universal newborn hearing screening, laboratory testing, congenital infections (especially cytomegalovirus), genetic testing and main syndromes, radiologic imaging studies, vestibular assessment of children with hearing loss, auditory neuropathy spectrum disorder, autism spectrum disorder, and noise-induced hearing loss. Conclusions: Every child with suspected hearing loss has the right to diagnosis and appropriate treatment if necessary. This task force considers 5 essential rights: (1) Otolaryngologist consultation; (2) Speech assessment and therapy; (3) Diagnostic tests; (4) Treatment; (5) Ophthalmologist consultation.

Abstract Objective: To review the literature on the diagnosis and treatment of vestibular schwannoma. Methods: Task force members were educated on knowledge synthesis methods, including electronic database search, review and selection of relevant citations, and critical appraisal of selected studies. Articles written in English or Portuguese on vestibular schwannoma were eligible for inclusion. The American College of Physicians’ guideline grading system and the American Thyroid Association’s guideline criteria were used for critical appraisal of evidence and recommendations for therapeutic interventions. Results: The topics were divided into 2 parts: (1) Diagnosis – audiologic, electrophysiologic tests, and imaging; (2) Treatment – wait and scan protocols, surgery, radiosurgery/radiotherapy, and systemic therapy. Conclusions: Decision making in VS treatment has become more challenging. MRI can diagnose increasingly smaller tumors, which has disastrous consequences for the patients and their families. It is important to develop an individualized approach for each case, which highly depends on the experience of each surgical team.

Resumo Introdução: A legislação brasileira atual exige que todos os trabalhadores expostos a ruído recebam um audiograma na admissão, 6 meses após a admissão (primeiro teste periódico), e anualmente após o primeiro teste periódico, mas em outros países as regulamentações dos programas de conservação auditiva não incluem a exigência de teste audiométrico após 6 meses de admissão, mas apenas anualmente. Não há evidências de que a periodicidade adotada pela legislação brasileira seja a mais adequada. Objetivo: Avaliar os três primeiros exames audiométricos ocupacionais de trabalhadores expostos ao ruído. Método: Estudo de coorte histórica com análise transversal. Os participantes eram todos trabalhadores metalúrgicos do sexo masculino com até 40 anos. Os três primeiros audiogramas de cada trabalhador foram analisados: teste audiométrico pré-admissão, teste audiométrico periódico 1 e teste audiométrico periódico 2. Para cada trabalhador, os limiares de frequência médios foram calculados em 3, 4 e 6 kHz nas orelhas esquerda e direita para cada teste. A análise estatística foi feita através do teste não paramétrico de Wilcoxon. Resultados: Foram incluídos 988 trabalhadores. Houve uma diferença significante nos limiares auditivos entre o teste pré-admissão e os 2 testes periódicos subsequentes para as orelhas direita e esquerda. Não houve diferença significante entre o teste 1 e o teste 2 em nenhuma das orelhas. Conclusão: Dada a falta de diferença entre os 2 primeiros testes periódicos, acreditamos que eles poderiam ser combinados em um único teste, ou seja, o primeiro teste audiométrico periódico poderia ser feito após 12 meses de admissão sem comprometer a saúde dos trabalhadores.

Abstract Introduction: Current Brazilian legislation requires that all workers exposed to noise are to be given an audiogram upon hiring, after 6 months of employment (first periodic test), and annually after the first periodic test. In other countries, the regulations of hearing conservation programs do not include the requirement for audiometric testing at 6 months of employment, but only annually. There is no evidence that the periodicity adopted by Brazilian legislation is the most appropriate. Objective: The present study aimed to evaluate the first 3 occupational audiometric tests of workers exposed to noise. Methods: Historical cohort study with cross-sectional analysis. Participants were all male metallurgy workers aged up to 40 years. The first 3 audiograms of each worker were analyzed: pre-employment audiometric test, periodic audiometric test 1, and periodic audiometric test 2. For each worker, mean frequency thresholds were calculated at 3, 4, and 6 kHz in the left and right ears for each test. Statistical analysis was performed using the nonparametric Wilcoxon test. Results: A total of 988 workers were included. There was a significant difference in auditory thresholds between the pre-employment test and the 2 subsequent periodic tests for the right and left ears. There was no significant difference between Test1 and Test2 in either ear. Conclusion: Given the lack of difference between the first 2 periodic tests, we believe that they could be merged into a single test, i.e., first periodic audiometric testing could be performed at 12 months of employment without compromising workers’ health.

INTRODUÇÃO: A estimulação eletroacústica é uma excelente opção para pessoas com audição residual nas baixas frequências, que obtêm benefício insuficiente com aparelhos auditivos. Para ser eficaz, a audição residual deve ser preservada durante a cirurgia de implante coclear.OBJETIVOS: Avaliar a preservação auditiva de pacientes implantados e comparar os resultados de acordo com a abordagem da orelha interna.MÉTODO: 19 indivíduos foram implantados com uma técnica cirúrgica para preservação auditiva, tendo sido utilizado o eletrodo MED-EL FLEXTM EAS, concebido para ser atraumático. Foram avaliados os exames audiométricos tonais no pré e pós-operatório, com uma média de 18,4 meses após o implante para medir a taxa de preservação da audição residual.RESULTADOS: 17 pacientes tiveram preservação total ou parcial da audição residual; cinco obtiveram preservação da audição total e dois indivíduos não tiveram preservação da audição. A inserção do eletrodo ocorreu por cocleostomia em 3 pacientes; em 2 destes pacientes não houve preservação da audição. Os outros 16 pacientes foram submetidos à abordagem pela janela redonda. Todos os pacientes foram beneficiados com o implante coclear, mesmo aqueles pacientes que utilizando apenas estimulação elétrica.CONCLUSÃO: A preservação auditiva ocorreu em 89,4% dos casos. Não houve diferença significativa entre as formas de abordagem da orelha interna.

INTRODUCTION: Electroacoustic stimulation is an excellent option for people with residual hearing in the low frequencies, who obtain insufficient benefit with hearing aids. To be effective, the subject's residual hearing should be preserved during cochlear implant surgery.OBJECTIVES: To evaluate the hearing preservation in patients that underwent implant placement and to compare the results in accordance with the approach to the inner ear.METHODS: 19 subjects underwent a soft surgical technique, and the electrode MED-EL FLEX(tm) EAS, designed to be atraumatic, was used. We evaluated pre- and postoperative tonal audiometric tests with an average of 18.4 months after implantation, to measure the rate of hearing preservation.RESULTS: 17 patients had total or partial preservation of residual hearing; 5 had total hearing preservation and two individuals had no preservation of hearing. The insertion of the electrode occurred through a cochleostomy in 3 patients, and in 2 of these there was no hearing preservation; the other 16 patients experienced electrode insertion through a round window approach. All patients benefited from the cochlear implant, even those who are only using electrical stimulation.CONCLUSION: The hearing preservation occurred in 89.4% of cases. There was no significant difference between the forms of inner ear approach.

Atriagem auditiva, a identificação e intervenção precoces da perda auditiva possibilitam um bom prognóstico para o desenvolvimento infantil. OBJETIVO: Analisar os resultados obtidos pelo Programa de Saúde Auditiva em neonatos que permaneceram em Unidade de Terapia Intensiva-UTI. MÉTODO: Estudo de corte transversal prospectivo. A amostra foi constituída por recém-nascidos, internados em unidade neonatal do CAISM/Unicamp, por pelo menos 48 horas, no período de 13 meses. O procedimento utilizado para triagem auditiva foi o Potencial Evocado Auditivo de Tronco Encefálico Automático-Madsen Accuscreen, próximo à alta hospitalar. As crianças que falharam na triagem foram encaminhadas para diagnóstico audiológico, otorrinolaringológico e genético. RESULTADOS: Fizeram a triagem auditiva 84,7% dos neonatos vivos, sendo realizada em 39,7% dos casos após 30 dias de vida. O diagnóstico mostrou que 63,8% das crianças apresentaram audição normal. A incidência da perda auditiva foi de 4%, sendo de 1,4% para perda do tipo neurossensorial, 0,24% com Espectro da Neuropatia Auditiva e 2,2% do tipo condutiva. CONCLUSÃO: Concluiu-se que a triagem auditiva neonatal não foi universal e nem aplicada, em muitos casos, no primeiro mês de vida. Deve ser realizada antes da alta hospitalar e em de mais de uma etapa. A incidência da perda auditiva foi elevada.

Auditory screening and early identification and management of patients with hearing loss improve the development prospects of infants. OBJECTIVE: To analyze the outcomes produced by an Auditory Health Program in neonates managed in an intensive care unit. METHOD: This prospective cross-sectional study enrolled neonates referred to the neonatal care unit at hospital CAISM/Unicamp with stays lasting for 48 hours and more within a period of 13 months. Automated monitoring of brainstem auditory evoked potentials was used in the auditory screening of neonates at the time of discharge. Children with poor BAEPs were sent to undergo audiological, otorhinolaryngological, and genetic tests. RESULTS: Auditory screening was performed for 84.7% of the live births; 39.7% were screened at 30 days or more of age. Diagnostic tests revealed that 63.8% of the children had normal hearing. Incidence of hearing loss was 4%; sensorineural hearing loss was observed in 1,4% of the subjects; 0.24% had auditory neuropathy spectrum disorder; and 2.2% had conductive hearing loss. CONCLUSION: Neonatal auditory screening was not offered universally, and nor was it carried out, in many cases, within the child's first month of life. Screening must be performed before neonates are discharged and in more than one stage. A high incidence of hearing loss was observed.

O implante coclear é um grande avanço no tratamento da surdez. Há grandes evidências que a audição bilateral traz diversas vantagens ao ser humano e muito mais quando se trata de audição binaural. OBJETIVO: Este artigo tem por objetivo descrever o primeiro caso operado no Brasil, sendo o terceiro país das Américas (México e Colômbia) a realizar, com implante binaural Neurelec Digisonic SP® demonstrando a técnica cirúrgica. MÉTODO: Descrição da técnica cirúrgica. RESULTADOS: Ver técnica cirúrgica. Procedimento ocorreu sem intercorrências. DISCUSSÃO: O efeito "squelch", a somação binaural, a localização da fonte sonora e o efeito sombra da cabeça são umas das principais razões que justificam a superioridade da reabilitação binaural. O custo do tratamento deve ser levado em conta em políticas de saúde pública. CONCLUSÃO: Custo do implante coclear é um dos grandes limitadores da bilateralidade na reabilitação desse grupo de pacientes, porém, com essa tecnologia, é possível solucionar esse problema, expondo o paciente a poucos riscos, com o uso de uma técnica cirúrgica pouco invasiva.

Cochlear implants represent a significant breakthrough in the treatment of hearing loss. Evidence indicates bilateral hearing brings significant benefits to patients, particularly when binaural hearing is offered. OBJECTIVE: To describe the first case of implantation of a Digisonic SP® Binaural Neurelec device in Brazil (the third implant placed in the Americas, after Mexico and Colombia) and the chosen surgical approach. METHOD: Description of a surgical approach. RESULTS: The procedure was successfully completed. DISCUSSION: The squelch effect, binaural summation, location of the sound source, and the shadow effect of the head are listed among the reasons to explain the superiority of binaural rehabilitation. Cost of treatment must be considered in the development of public health policies. CONCLUSION: The cost of cochlear implants has been one of the main impediments to bilateral rehabilitation. The Digisonic SP® Binaural Neurelec device addresses this issue and exposes patients to less risk through a minimally invasive implantation procedure.

O implante coclear para pacientes com disacusia grave/profunda revolucionou o modo com que estes interagem com o meio ambiente. A Neurelec desenvolveu um sistema que permite fixação com uso de dois simples parafusos de titânio, sem necessidade de broqueamento do osso do crânio do paciente. Este artigo teve o objetivo de descrever uma casuística de pacientes submetidos a implante coclear com o Digisonic® SP, para mostrar os resultados cirúrgicos e detalhes do procedimento. MÉTODO: Estudo retrospectivo que avaliou os pacientes, no período de 18 meses, submetidos a implante coclear com o Digisonic® SP. Todos os pacientes eram pós-linguais. Coleta dos dados realizada por meio da análise dos prontuários dos pacientes, além de questionário padronizado aplicado nos cirurgiões que realizaram o procedimento. RESULTADOS: Os seis casos implantados com o Digisonic® SP foram realizados por cirurgiões experientes e o tempo cirúrgico variou de 95 a 203 minutos (média de 135 minutos), que é menor do que o descrito com outras formas de fixação. Não houve nenhum tipo de complicação e o ganho auditivo foi satisfatório. CONCLUSÃO: O implante Digisonic® SP desenvolvido pela Neurelec apresentou bons resultados audiológicos em adultos, tempo de cirurgia menor e não ocorreram complicações cirúrgicas ou pós-operatórias.

Cochlear implants have revolutionized the way patients affected by severe hearing loss experience the world. Neurelec developed a fixation system with two titanium screws that requires no skull bone drilling. OBJECTIVE: To describe the outcomes and procedure-related details of a series of patients implanted with the Digisonic® SP cochlear implant. METHOD: This retrospective study analyzed patients submitted to cochlear implant placement within a period of 18 months. All patients had postlingual hearing impairment. Data was collected from patient charts and standard questionnaires answered by the surgeons in charge of carrying out the procedures. RESULTS: The six patients offered the Digisonic® SP cochlear implants were operated by experienced surgeons. The procedures took 95 to 203 minutes (mean = 135') to be completed, which is less time than what has been described for other fixation approaches. No complications were recorded and hearing improvement was satisfactory. CONCLUSION: The Digisonic® SP cochlear implant developed by Neurelec offered good audiological results for adult patients, shorter surgery time, and no surgical or postoperative complications.

A reabilitação auditiva entrou numa nova era com o desenvolvimento e aperfeiçoamento de próteses implantáveis, principalmente com a associação do conceito de a estimulação eletroacústica (EAS) e implante coclear. A EAS é indicada para pacientes com audição residual nas baixas frequências e disacusia grave/profunda nas médias e altas frequências. Esses pacientes não teriam indicação para o implante coclear convencional e teriam dificuldades na adaptação com o aparelho de amplificação sonoro individual. A técnica cirúrgica utilizada deve-se basear nos conceitos de preservação auditiva e é fundamental nesse processo. OBJETIVOS: Descrever a técnica cirúrgica para procedimento de implante coclear MED-EL Mand FlexEAS, com intuito de preservar o resíduo auditivo e reabilitar satisfatoriamente a audição deteriorada, sendo o primeiro centro a realizar tal tratamento no Brasil. MATERIAL E MÉTODOS: Descrição de técnica cirúrgica utilizada pelo serviço de otologia de hospital terciário por meio de estudo de caso. RESULTADOS: Procedimentos realizados sem intercorrências. A técnica utilizada segue passos que objetivam a preservação auditiva. CONCLUSÃO: Descrevemos a técnica utilizada para implantes com EAS realizados no nosso serviço. A técnica cirúrgica é complexa e inclui passos que visam à preservação auditiva.

A new era has arrived in auditory rehabilitation with the introduction of new technologies such as electroacoustic stimulation (EAS). EAS is indicated for patients with residual hearing at low frequencies and severe or profound hearing loss at high frequencies. These patients have no indication for conventional cochlear implant and have difficulties in adapting to individual sound amplification devices. Preservation of hearing is vital in this process; the surgical technique must be based on this concept. OBJECTIVES: To present the cochlear implant surgical technique with MED-EL Mand FlexEAS to preserve hearing in patients with hearing loss at high frequencies and to maintain low frequency hearing. We are the first institution to carry out this treatment in Brazil. METHODS: A case report of the surgical technique carried out in four patients; the procedure was carried out by the cochlear implant group of a specialized clinical hospital. RESULTS: The procedures were successful and uneventful. CONCLUSION: We described the technique used at our institution for implants using EAS; the surgical technique is complex and includes steps for preservation of hearing.

A audição é um dos principais meios de contato do indivíduo com o mundo externo, desempenhando papel fundamental na integração com a sociedade. OBJETIVO: Analisar os resultados obtidos na avaliação audiológica, otorrinolaringológica e genética de lactentes de alto risco que falharam na triagem auditiva neonatal. MATERIAL E MÉTODO: Estudo clínico e experimental. Foram avaliados 38 lactentes, entre 1 e 6 meses de idade cronológica. Os procedimentos utilizados foram: anamnese, imitanciometria, Potencial Evocado Auditivo de Tronco Encefálico, Emissões Otoacústicas por Transiente e avaliação otorrinolaringológica. O estudo genético foi realizado a partir da extração de DNA da mucosa bucal utilizando o método de protocolo adaptado no Laboratório de Genética Humana do CBMEG/UNICAMP. RESULTADO: Não houve diferença estatisticamente significante entre neonatos com audição normal e perda auditiva e as variáveis gênero e número de indicadores de risco. Quanto à idade gestacional, neonatos a termo foram mais afetados. A perda auditiva esteve presente em 58% da amostra, sendo do tipo condutiva em 31,5%, e neurossensorial em 28,9% dos casos. Não foram encontradas as mutações genéticas mais comumente observadas em casos com etiologia genética. CONCLUSÃO: A perda auditiva foi diagnosticada na maioria dos lactentes de risco com provável etiologia ambiental.

Hearing is one of the main ways with which one person can contact the external world; it plays a key role in their integration with society. AIM: The objective of this study was to analyze the results of the hearing, medical and genetic evaluation of high-risk infants who failed the newborn hearing screening. MATERIALS AND METHODS: Clinical and experimental study. We assessed thirty-eight neonates, with ages between one and six months. The infants underwent the following procedures: medical interview; immittance testing; Brainstem Auditory Evoked Potential; Transient Evoked Otoacoustic Emission and otorhinolaryngological evaluation. DNA extraction from the oral mucosa was performed for genetic studies using the protocol method adapted from the Human Genetics Lab of the CBMEG/UNICAMP. RESULTS: Regarding gender and presence of risk factors, significant statistically differences were not found in normal hearing infants and in those with hearing loss. Concerning gestational age, term infants were more affected by hearing loss. Hearing loss was identified in 58% of the sample, conduction hearing loss represented 31.5% (12/38) and neurossensory 28.9% of cases. There were none of the genetic mutations most commonly seen in cases with a genetic etiology. CONCLUSION: Hearing loss was identified in the majority of High-risk infants.

A possibilidade de realizar o implante coclear em crianças pequenas torna necessário o uso de medidas objetivas para auxiliar a programação do processador de fala. Telemetria é a propriedade que permite, no Nucleus 24®, a obtenção do potencial de ação composto evocado do VIII par (EAP) utilizando o implante como instrumento de estimulação e gravação para o estudo das propriedades neurais remanescentes. OBJETIVO: Descrever a utilização do sistema de telemetria para a gravação do EAP, caracterizando as respostas obtidas e a sua prevalência na condição intraoperatória. FORMA DE ESTUDO: clínico com coorte transversal. MATERIAL E MÉTODO: Medidas das impedâncias dos eletrodos e do EAP em um grupo de 17 indivíduos usuários do implante Nucleus 24® durante a cirurgia. Análise das respostas de acordo com a etiologia, o tempo de duração da surdez e a posição dos eletrodos dentro da cóclea. RESULTADOS: Maior prevalência nos eletrodos apicais e limiares mais elevados nos casos de meningite e otosclerose. CONCLUSÃO: A telemetria é eficiente para a verificação da integridade dos eletrodos na condição intraoperatória e para a gravação do EAP, apresentando alta prevalência na população estudada.

Cochlear implantation has been recommended for children under 24 months of age. The use of objective measures is needed to help speech processor programming. The electrically evoked compound potential (EAP), which can be assessed by neural response telemetry (NRT), is one of those objective measures. AIM: to determine how often the EAP can be recorded by NRT system during surgery and to describe the responses. STUDY DESIGN: clinical with transversal cohort. MATERIAL AND METHOD: the impedances and NRT were measured in a group of 17 Nucleus 24 ® implant users. The responses were analyzed and compared to the etiology, hearing loss duration and electrode array position. RESULTS: The EAP was easily recorded in the apical electrodes and, in otosclerosis and meningitis cases the EAP threshold was higher than in the other etiology cases. CONCLUSIONS: The NRT can be found in 82% of the cases during surgery. The responses obtained may vary according to etiology and the position of electrodes along the cochlea.