Hearing Rehabilitation through Bone-Conducted Sound Stimulation: Preliminary Results

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. (AU)

Hearing Rehabilitation through Bone-Conducted Sound Stimulation: Preliminary Results.

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 to mild 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.

Analysis of electrically evoked compound action potential of the auditory nerve in children with bilateral cochlear implants / Análise do potencial de ação composto do nervo auditivo evocado eletricamente em crianças usuárias de implante coclear bilateral

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.

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.

Analysis of electrically evoked compound action potential of the auditory nerve in children with bilateral cochlear implants.

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.

Familial Alström syndrome: a rare cause of bilateral progressive hearing loss.

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.

Familial Alström syndrome: a rare cause of bilateral progressive hearing loss / Familial Alström syndrome: a rare cause of bilateral progressive hearing loss

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. .

Comparative analysis of vocabulary in children with cochlear implants.

INTRODUCTION: The use of tests assessing the speech of patients who underwent cochlear implant (CI) surgery is warranted to increase knowledge on the development of these patients' language skills. The ABFW Child Language Test evaluates vocabulary, phonology, fluency, and pragmatics and can be administered to these children. OBJECTIVE: To evaluate the vocabulary of children using CI. METHOD: This study included 16 children who underwent CI surgery between 1-4 years and 11 months old. Patients were divided into five groups according to age upon CI activation. RESULTS: In comparison with the age of brain's auditory development vs. chronological age of hearing children, all children using CI performed better. The comparison between children using unilateral CI and bilateral CI showed that those using bilateral CI had better results. When we compared children's performance considering the chronological age of deaf and hearing children, hearing children performed better. However, there were similar results when patients effectively used their CI and attended auditory rehabilitation sessions. CONCLUSION: In our study, the vocabulary of children using CI is similar to the vocabulary of hearing children.

Auditory neuropathy spectrum: the importance of adequate diagnosis.

OBJECTIVE: To evaluate the prevalence of misdiagnosis of the hearing impaired children, in order to investigate the existence of Auditory Neuropathy Spectrum Disorder. METHODS: Analytical, prospective study conducted with 15 participants (30 ears) aged 10-12 years, with bilateral sensorineural hearing loss, attending a Hearing Rehabilitation Center, accompanied by a multidisciplinary team with otolaryngologist, audiologist, psychologist, teacher and social worker. The participants underwent ENT examination and audiological assessment: tympanometry, acoustic reflexes, otoacoustic emissions test and Auditory Evoked response. RESULTS: From the total sample (30 ears), 8 ears (26.7%) presented absent responses in the Auditory Evoked response with the presence of cochlear microphonism. Within the selected eight ears, six (75%) showed presence of otoacoustic emissions test in isolated frequencies and two (25%) ears had otoacoustic emissions test even in the presence of the isolated frequencies. It was found that 26.7% of the ears tested presented results that are compatible with Auditory Neuropathy Spectrum Disorder. CONCLUSION: The results of this study have identified characteristics related to ANSD in 26.7% of ears tested, 6 participants with age over 10 years old, showing that misdiagnosis in hearing loss patients is still very frequent.