Introdução: A presença de perdas auditivas de origem neural no adulto que já desenvolveu linguagem pode acarretar alteração de compreensão da fala com dificuldade na discriminação auditiva dos sons e entendimento completo da mensagem. Entre as causas de perdas auditivas neurais está o distúrbio do espectro da neuropatia auditiva (DENA). A maioria das publicações sobre o DENA descrevem o padrão do diagnóstico auditivo, entretanto as consequências dessa alteração auditiva para a comunicação do indivíduo e as implicações dessas para o tratamento fonoaudiólogico são escassas. Faz-se necessária a identificação das especificidades linguísticas a serem avaliadas nos neuropatas, por meio de um protocolo de avaliação direcionado, para permitir a elaboração de diretrizes terapêuticas bem delineadas. Objetivo: Este estudo teve como objetivo descrever as manifestações linguísticas em adultos com o Distúrbio do espectro da neuropatia auditiva (DENA). Métodos: Foram incluídos neste estudo pacientes adultos identificados com o diagnóstico de DENA, alfabetizados, sem alterações neurológicas e cognitivas, no período entre 2007 e 2009 no setor de Fonoaudiologia do Ambulatório de Otorrinolaringologia do HCFMUSP. Doze pacientes foram selecionados, sendo 8 do sexo masculino (66,7%), com idades entre 18 e 50 anos. Foi elaborado um protocolo de anamnese incluindo dados sobre escolaridade, uso de amplificação sonora individual (AASI) e queixas auditivas específicas. O protocolo de avaliação constou de provas que abordaram a avaliação da recepção auditiva e da emissão de fala (identificação fonêmica; inteligibilidade; leitura e compreensão de texto e consciência fonológica) e da expressão (fala e elaboração). Os estímulos foram dados por via somente auditiva e no modo auditivo e visual (com leitura orofacial - LOF). Resultados: As principais características observadas nestes pacientes: sexo masculino, ensino fundamental incompleto, uso de AASI menor que três meses em ambas as orelhas, dificuldade de ouvir em ambientes ruidosos e diálogo foram as situações comunicativas que geraram maior dificuldade na expressão. Observou-se que em todas as provas com apoio da LOF, houve melhora significativa da percepção da fala do ponto de vista clínico. Conclusões: As especificidades linguísticas dos pacientes adultos com DENA encontradas foram: baixa escolaridade, velocidade de fala alterada, dificuldade de compreensão de texto tanto pela via auditiva como pela leitura, dificuldade de consciência fonológica, melhora da repetição de palavras e frases com o uso da LOF. / Introduction: Post linguistic neural hearing loss in adults can lead to speech alterations and difficulties in auditory discrimination of sounds and comprehension of the message. Auditory neuropathy spectrum disorder (ANSD) is among the causes of neural hearing loss. Most studies on ANSD describe the standard for auditory diagnosis. However, the consequences of such hearing impairment in communication and its implication on speech therapy are scarce. Thus, it is necessary to identify the specific language aspects to be assessed in neurologically impaired individuals through a directed assessment protocol to allow the development of outlined treatment guidelines. Objective: This study aimed to describe the linguistic manifestations in adults with ANSD. Methods: The study included adults diagnosed with ANSD, who were literate and had no neurological or cognitive alterations. Data collection was carried out between 2007 and 2009 at the Speech, Language and Hearing service of the Clinic of Otorhinolaryngology of HCFMUSP. Twelve patients, eight males (66,7%) with ages ranging from 18 and 50 years of age were selected. An anamnesis protocol was designed. This protocol included data on education, use of hearing aids (HA) and specific hearing complaints. The assessment protocol consisted on tests of auditory reception and production of speech (phonemic identification; intelligibility; reading and text comprehension; and phonological awareness) and expression (speech and elaboration). The stimuli input were given in auditory only and in auditory plus visual mode (with lip reading). Results: The main characteristics observed in all participants were: male gender; incomplete primary school; use of hearing aids for less than three months in both ears; difficulty hearing in noisy environments; and dialogue, were the communicative situations that led to greater difficulty in expression. A significant improvement in speech perception was observed in all tests with lip reading. Conclusions: The language specificities of individuals with ANSD were: low educational level; speech rate alterations; difficulty in reading comprehension both by hearing and by reading; difficulty in phonological awareness; improvement of words and phrases repetition using LR.

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.

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