A Desordem do Espectro da Neuropatia Auditiva (DENA) é uma alteração auditiva periférica com manifestações clínicas heterogêneas, o que pode tornar o processo de intervenção auditiva em crianças com essa patologia um desafio para os profissionais e para as famílias. Neste sentido, o objetivo deste estudo foi compreender a vivência de mães, no processo de intervenção auditiva de crianças com DENA. O estudo foi de natureza qualitativa e prospectiva. Como método investigativo foram realizadas entrevistas abertas, norteadas por uma pergunta disparadora, na busca por mais profundidade e reflexão sobre o fenômeno em questão. No total, treze mães foram entrevistas e, para compreensão dos relatos empreendeu-se uma análise de conteúdo de acordo com os referenciais teóricos de Bardin, que resultou nas seguintes categorias subcategorias: Manifestações emocionais durante o tratamento, com as subcategorias Sentimentos da mãe no processo de intervenção da deficiência auditiva, Expectativas durante o processo de tratamento, Atendimento interdisciplinar; Condutas terapêuticas e educacionais, com subcategorias Indicação e uso dos dispositivos eletrônicos, Condutas educacionais; Redes de apoio, com as subcategorias Grupos de Apoio, Aspectos da fé; Preconceito e Impactos da pesquisa para as participantes. O processo de intervenção auditiva de seus filhos com DENA inicialmente significou para as mães um mundo desconhecido, vivenciado por sentimentos de sofrimento e insegurança diante do diagnóstico. Conforme compreenderam melhor a DENA e observaram os resultados positivos das intervenções, puderam viver com mais tranquilidade. A esperança de um futuro melhor e o apoio recebido deram o sentido para continuarem sua trajetória. Esta pesquisa trouxe reflexões sobre o papel social dessas mães na relação DENA e família. Revelou também a importância da equipe interdisciplinar, das redes de apoio e do suporte psicológico. Aponta-se a necessidade de novos olhares paras as políticas públicas em saúde auditiva desde as redes básicas até os centros especializados de saúde. / The Auditory Neuropathy Spectrum Disorder (ANSD) consists in a specific type of hearing impairment with different etiologies and manifestation forms. Therefore, the process of auditory intervention in children with this pathology can be challenging for health professionals and families. Giving this circumstance, the objective of this study was to comprehend the experience of mothers focusing on the auditory intervention process of children with ANSD disorder. The research performed was qualitative and prospective. As an investigative method, open interviews were conducted, guided by a guiding question, in the search for more depth and reflection on the phenomenon in focus. In total thirteen mothers were interviewed and, in order to comprehend the narratives, a content analysis was performed based on Bardin theoretical references. The results were gathered in the following categories: Emotional manifestations during treatment, subcategories, Mother feelings during the auditory intervention process, Expectations during the treatment process, Interdisciplinary service; Therapeutic and educational conduct, subcategories Electronic device indication and usage, educational conduct, Support network, Support Groups, Faith aspects, Prejudice, and Research impact on participants. The process of auditory intervention of their children with ANSD initially meant for the mothers an unknown world, experienced by feelings of suffering and insecurity before the diagnosis. However, as they understood DENA better and the positive results of the intervention emerged, could live more peacefully. The hope for a better future and the support received has given the sense to continue their trajectories. This research brought reflections on the social role of these mothers in relation to ANSD and family. It also revealed the importance of the interdisciplinary team, the psychological and social support which mothers received, as well as the promotion of mothers\' health. It is pointed out the need for new public policies in hearing health from the basic to the specialized centers of health.

Introduction: Auditory neuropathy spectrum disorder (ANSD) is a disorder characterized by impaired temporal coding of acoustic signals due to a deficiency in neural synchrony or neural transmission. Despite variations in speech perception outcomes within both patients and studies, current evidence demonstrates that children with ANSD, who use hearing aids (HAs) or cochlear implants (CIs), generally achieve speech perception performance comparable to peers with sensorineural hearing loss (SNHL). However, limited studies have reported factors that have prognostic value for auditory intervention outcomes. The objectives of this doctoral thesis, consisting of three consecutive associated projects on children with ANSD, were (1) to determine factors with predictive value for post-intervention (CIs and/or HAs) outcomes through a retrospective study, (2) to systematically summarize and critically appraise existing evidence of the prognostic value of early auditory electrophysiologic tests and MRI findings for CI outcomes through a systematic review (SR), and (3) to systematically overview, summarize, and critically appraise evidence of CI outcomes through an umbrella review of current SRs (overview of SRs). -- Methods: For the first project, the records of 38 children with ANSD between 5 and 18 years old, 63.20% males, who used CIs (71%) and/or HAs, identified at the Children's Hospital of Eastern Ontario (CHEO) were reviewed. For the second and third projects, the SRs were guided by the PRISMA 2020 statement, and electronic databases were searched without restrictions on language, publication status, or year of publication. In the second project, studies on children with ANSD (including those with cochlear nerve deficiency [CND]), cochleovestibular nerve (CVN) abnormalities, or SNHL reporting the relevance of preoperative and/or postoperative electric compound action potential (eCAP), electric auditory brainstem response (eABR), and/or MRI results to CI outcomes were included. The methodological quality and strength of evidence were assessed using the Crowe Critical Appraisal Tool (CCAT) and Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) tool, respectively. In the third project, all SRs that reported CI outcomes in children with ANSD were included. The methodological quality of the selected SRs was evaluated using the Assessment of Multiple Systematic Reviews 2 (AMSTAR-2) checklist, and the risk of bias in evidence was assessed using the Risk of Bias in Systematic Reviews (ROBIS) tool. -- Results: In the retrospective chart review (first project), ages at HL diagnosis and CI activation and the length of follow-up with CI/HA showed a significant relationship with open-set speech perception outcomes (i.e., the scores of Phonetically Balanced Kindergarten [PBK] test with word and phoneme speech materials and Hearing in Noise Test [HINT] in quiet and noise conditions). Using a Forward Linear Multiple Regression Model, the length of follow-up with CI/HA and bilateral amplification showed prognostic value for speech perception performance. In the second project, 25 papers were included in the review. While it was difficult to draw a firm conclusion about the eCAP findings, current evidence strongly supports the prognostic value of eABR and MRI for post-CI speech perception outcomes. According to the eight SRs selected for the third project, children with ANSD achieve CI outcomes comparable to their peers with SNHL. However, in children with postsynaptic ANSD (i.e., those with CND), cochlear nerve hypoplasia is associated with better speech recognition outcomes compared to cochlear nerve aplasia, especially in the absence of additional disabilities and/or medical comorbidities (ADs/MCs). -- Conclusion: Children with ANSD, especially those without cochlear nerve aplasia and ADs/MCs, achieve speech perception outcomes comparable to their peers with SNHL. In addition, age at HL diagnosis, age at CI activation, the length of follow-up with CI/HA, bilateral amplification, and eABR and MRI findings are associated with or have predictive value for intervention outcomes. The findings of the SRs should be interpreted with caution given the low quality of evidence and risk of bias in the studies selected for SRs.