Abstract Introduction The bone-anchored hearing aid (BAHA) is a bone conduction system that transmits the sound directly to the inner ear by surpassing the skin impedance and the subcutaneous tissue. It is indicated for patients with mixed, conductive and unilateral sensorineural hearing loss who did not benefit from conventional hearing aids (HAs). Although the benefits from BAHA are well demonstrated internationally, this field still lacks studies in Brazil. Objective To assess the auditory rehabilitation process in BAHA users through audiological, speech perception and tinnitus aspects. Methods Individuals with hearing loss were assessed before and after the implantation. The participants were subjected to pure tone audiometry in free field, functional gain audiometry, speech perception tests, tinnitus handicap inventory (THI) in open format, and to the visual analog scale (VAS). Results It was found that the participants benefited from the use of BAHA. The difference in the performance of the participants before and after the BAHA surgery was significant in terms of hearing acuity. There was no statistically significant difference in the speech perception tests. The tinnitus assessment showed that 80% of the participants scored slight tinnitus severity in THI after using a BAHA. Eighty percent of the participants classified their tinnitus as absent tomild in the VAS after the surgery. Conclusion Based on the results of the current study, we can conclude that the participants improved both the auditory perception and the tinnitus handicap.

RESUMO INTRODUÇÃO: O implante coclear tem a capacidade de medir o potencial de ação composto eletricamente evocado do nervo auditivo (ECAP). Para esta verificação utiliza-se uma medida chamada telemetria de respostas neurais. OBJETIVO: Analisar o potencial de ação composto evocado eletricamente, por meio da neurotelemetria de respostas neurais, em crianças usuárias de implante coclear bilateral. MÉTODO: Trata-se de um estudo analítico, prospectivo, de coorte histórica longitudinal. Foram recrutadas seis crianças, com idades entre de 1-4 anos, usuárias de implante coclear bilateral. Estas crianças foram avaliadas em cinco momentos durante o primeiro ano de uso do implante coclear. RESULTADOS: Houve diferença significativa no tempo de acompanhamento (p = 0,0082) e posição do eletrodo (p = 0,0019) na medida de T-NRT. Houve diferença significativa na interação entre tempo de acompanhamento e posição do eletrodo (p = 0,0143) na medida da amplitude das ondas N1-P1 entre os três eletrodos a cada tempo de acompanhamento. CONCLUSÃO: A mensuração do ECAP por meio da NRT nas crianças com implante coclear bilateral durante o primeiro ano de acompanhamento foi uma medida importante para apresentar o desenvolvimento bilateral da via auditiva periférica de forma sincronizada nesta população estudada.

ABSTRACT INTRODUCTION: The cochlear implant device has the capacity to measure the electrically evoked compound action potential of the auditory nerve. The neural response telemetry is used in order to measure the electrically evoked compound action potential of the auditory nerve. OBJECTIVE: To analyze the electrically evoked compound action potential, through the neural response telemetry, in children with bilateral cochlear implants. METHODS: This is an analytical, prospective, longitudinal, historical cohort study. Six children, aged 1-4 years, with bilateral cochlear implant were assessed at five different intervals during their first year of cochlear implant use. RESULTS: There were significant differences in follow-up time (p = 0.0082) and electrode position (p = 0.0019) in the T-NRT measure. There was a significant difference in the interaction between time of follow-up and electrode position (p = 0.0143) when measuring the N1-P1 wave amplitude between the three electrodes at each time of follow-up. CONCLUSION: The electrically evoked compound action potential measurement using neural response telemetry in children with bilateral cochlear implants during the first year of follow-up was effective in demonstrating the synchronized bilateral development of the peripheral auditory pathways in the studied population.

Introdução: A Síndrome de Alstrom é uma doença muito rara, causada pela mutação no gene Obesidade infantil; ALMS1, que apresenta uma degeneração progressiva das funções sensoriais, resultando em de-Diabetes mellitus tipo 2; ficiências visuais e auditivas, além de distúrbios metabólicos como obesidade na infância, hipe-Retinite pigmentosa rinsulinemia e diabetes tipo II. Objetivo: Apresentar o perfil audiométrico de dois irmãos da mesma família afetados pela Síndrome de Alström. Método: Estudo prospectivo, analítico descritivo, os pacientes afetados foram submetidos a um questionário previamente testado, audiometria tonal e vocal seriadas, análise de emissões otoacústicas, e de respostas de potencial evocado auditivo de tronco encefálico, além de análise genético-molecular para comprovação diagnóstica. Resultados: Ambos os pacientes apresentaram perda auditiva bilateral com o início na infância e progressão lenta para perda auditiva neurosensorial severa no primeiro caso e, profunda, no segundo. As emissões otoacústicas estavam ausentes, e o potencial evocado auditivo de tronco encefálico estava normal em ambos os pacientes, bilateralmente. Conclusão: A Síndrome de Alström apresenta início precoce de perda auditiva neurossensorial, antes da adolescência, 10 a 20 anos para desenvolver perda auditiva severa a profunda. A lesão auditiva é essencialmente coclear, de acordo com os resultados dos testes de emissões otoacústicas e de potenciais evocados auditivos de tronco encefálico.

Introduction: Alström Syndrome is a rare disease caused by mutations in ALMS1 gene. It is characterized by a progressive degeneration of sensory functions, resulting in visual and audiological impairment, as well as metabolic disturbances such as childhood obesity, hyperinsulinemia, and diabetes mellitus type 2. Objective: To report and discuss the genetic and audiological findings in two siblings with Alström syndrome. Methods: This was a prospective, analytical and descriptive study, using questionnaires, serial audiograms, otoacoustic emissions, and auditory brainstem response analysis, as well as molecular genetic analysis. Results: Both patients presented childhood-onset bilateral sensorineural hearing loss, which progressed to moderate impairment in the first case and severe hearing loss in the second. Otoacoustic emissions were absent, and auditory brainstem responses were bilaterally normal in both cases. Conclusion: In the present patients, Alström Syndrome began with a neurosensory hearing loss in early childhood that progressed to a profound loss in ten to twenty years. The auditory lesions were cochlear in origen according to the otoacoustic emissions and auditory brainstem responses.