Neuropathy Spectrum(神经病谱)研究综述
Neuropathy Spectrum 神经病谱 - OTOF is one of the most frequent causes of hereditary hearing loss and a main cause of auditory neuropathy spectrum disorder (ANSD). [1] Klhl18 mutant mice exhibit an uncommon low frequency hearing impairment with physiological features consistent with Auditory Neuropathy Spectrum Disorder (ANSD). [2] Hearing loss is a characteristic feature of Brown-Vialetto-Van Laere syndrome and has been shown in recent studies to be characterised by auditory neuropathy spectrum disorder. [3] OBJECTIVE To determine the incidence of auditory neuropathy spectrum disorder (ANSD) and its risk factors among the neonatal intensive care unit (NICU) population from 2009 to 2018 in the Pediatric Health Information System database. [4] The aim of the study was to assess auditory stream segregation in individuals with cochlear pathology (CP) and auditory neuropathy spectrum disorder. [5] Objective The aim of this mini-systematic review was to evaluate the evidence reporting speech, language, and auditory behavioral outcome measures for children with a diagnosis of auditory neuropathy spectrum disorder (ANSD) who received cochlear implants (CIs) prior to 3 years of age. [6] CND includes a small number of cases that respond to DPOAE, indicating that some CND cases are clinically diagnosed with auditory neuropathy spectrum disorder (ANSD). [7] To refine the natural history and genotype-phenotype correlations of OTOF-related auditory neuropathy spectrum disorders (ANSD), audiograms and distortion product otoacoustic emissions (DPOAEs) were collected from a diverse cohort of individuals diagnosed with OTOF-related ANSD by comprehensive genetic testing and also reported in the literature. [8] OBJECTIVE To investigate how speech perception in noise (signal to noise ratio-50), difference limen frequency, temporal modulation transfer function, hearing handicap inventory for adults, and quality of life in auditory neuropathy spectrum disorder differs from sensorineural hearing loss. [9] BACKGROUND The current study attempted to assess efferent auditory system functioning in individuals with auditory neuropathy spectrum disorder (ANSD) using a new approach, contralateral suppression of SOAE, which has not yet been extensively researched. [10] The cortical auditory evoked potential (CAEP)-based P1 component acts as a biomarker for cochlear implantation (CI) outcomes in children with auditory neuropathy spectrum disorder (ANSD). [11] Objective The benefit of hearing aids as a clinical intervention strategy for children with Auditory Neuropathy Spectrum Disorder (ANSD) remains controversial. [12] OBJECTIVE The purpose of this paper is to describe a child with auditory neuropathy spectrum disorder (ANSD) associated with Brown-Vialetto-Van Laere (BVVL) syndrome, which is a rare, inherited, neurodegenerative disorder that is caused by defects in riboflavin transporter genes. [13] Background: Auditory neuropathy spectrum disorder (ANSD) in the auditory neural pathway can affect the auditory and speech development of children. [14] This study aimed to explore the central auditory system development in children with auditory neuropathy spectrum disorder (ANSD) using cochlear implants (CIs) and to find the correlation of CAEP biomarkers with speech perception. [15] The study aimed to investigate the working memory and speech recognition in noise from individuals with Sensori Neural Hearing Loss (SNHL) with and without auditory neuropathy spectrum disorder (ANSD). [16] Mutations in the OTOF gene are a common cause of hereditary hearing loss and the main cause of auditory neuropathy spectrum disorder (ANSD). [17] METHODS 15 participants with Auditory Neuropathy Spectrum Disorder were involved. [18] Auditory Neuropathy Spectrum Disorder is a rare condition wherein neural transmission through the VIIIth nerve and auditory brainstem is disrupted with intact peripheral hearing. [19] Significance Auditory neuropathy spectrum disorder (ANSD) is a confounding auditory disease in which the subjects respond to sound but have difficulties in speech discrimination. [20] Objective To evaluate the utility of pre-operative transtympanic electrically evoked auditory brainstem responses and post-operative neural response telemetry in auditory neuropathy spectrum disorder patients. [21] OBJECTIVE The objective of this study is to evaluate the audiologic outcomes with hearing aids in pediatric patients with auditory neuropathy spectrum disorder (ANSD) using the Infant Toddler-Meaningful Auditory Integration Scale (IT-MAIS), and the Ling 6 Sound Test (Ling 6). [22] Auditory neuropathy spectrum disorders, late-onset hearing loss, low-frequency and minimal hearing impairment are sometimes missed in newborn hearing screening or they manifest later. [23] This is a case report of a patient with Auditory Neuropathy Spectrum Disorder (ANSD) that resolved after shunt placement in a patient with hydrocephalus. [24] Objective Our study attempted to determine if tinnitus is a major concern in individuals with auditory neuropathy spectrum disorder (ANSD). [25] PurposeThe present study aimed to investigate the discrimination ability for acoustic cues in individuals with auditory neuropathy spectrum disorder (ANSD) using both behavioral and neural measures and to compare the result with normal hearing individuals. [26] Background: Auditory neuropathy spectrum disorder (ANSD) is a distinct type of SNHL that is characterized by the presence of otoacoustic emissions and/or cochlear microphonics. [27] Results: In two of the participants with auditory neuropathy spectrum disorder (ANSD), hypofunctional caloric responses were observed along with reduced vestibuloocular reflex (VOR) gain and refixation saccades. [28] Auditory neuropathy spectrum disorder (ANSD) is a complex and heterogeneous disorder associated with altered neural synchrony with respect to auditory stimuli. [29] PURPOSE To verify the effect of the multiple exemplar instruction at the acquisition and integration of listening and speaking behaviors, with substantives and substantive-adjective combinations, in children with Auditory Neuropathy Spectrum Disorder (ANSD) and cochlear implant (CI). [30] The sample included 40 children, aged 4 years to 11 years and 11 months old, divided into 4 groups: (1) 10 normal hearing children; (2) 13 children with hearing aids and frequency-modulated system; (3) 12 children using cochlear implant and fitted with the frequency-modulated system; and (4) 5 children diagnosed with auditory neuropathy spectrum disorder, fitted with hearing aids and/or cochlear implant and with the frequency-modulated system. [31] OBJECTIVE To explore the effects of exchange transfusion on auditory neuropathy spectrum disorder (ANSD) in neonates with severe hyperbilirubinemia (SH). [32] Studies were categorized according to hearing loss of any degree, with separate categories for documents providing specific criteria for mild bilateral, unilateral, and auditory neuropathy spectrum disorders. [33] Auditory neuropathy spectrum disorder (ANSD), also called auditory neuropathy (AN), is a unique type of prelingual hearing impairment. [34] INTRODUCTION Auditory Neuropathy Spectrum Disorder (ANSD) is characterized by hearing loss ranging from normal to profound. [35] Auditory neuropathy spectrum disorder (ANSD) is characterized by dysfunctional transmission of sound from the cochlea to the brain due to defective synaptic function or neural conduction. [36] The OTOF (Locus: DFNB9), encoding otoferlin, is reported to be one of the major causes of non-syndromic hearing loss, and is also reported to be the most common cause of non-syndromic recessive auditory neuropathy spectrum disorder. [37] OBJECTIVES The aims of the present study are to: describe diagnostic findings in patients with auditory neuropathy spectrum disorder (ANSD); and demonstrate the outcomes of different therapies like hearing aids (HAs) or cochlear implantation. [38] We report a Han Chinese male, born to consanguineous parents, presenting with nonsyndromic sensorineural hearing loss, whose clinical phenotype was also consistent with auditory neuropathy spectrum disorder (ANSD). [39] OBJECTIVES Auditory neuropathy spectrum disorder (ANSD) is an audiological diagnosis characterised by hearing dysfunction in the presence of intact outer hair cell function in the cochlea. [40] Introduction: Individuals with auditory neuropathy spectrum disorder (ANSD) are known to have temporal processing deficits and poor speech perception abilities. [41] The program includes the Otoacoustic Emissions (OAE) test for all newborns and Automated Auditory Brainstem Response (A-ABR) test for failed OAE and infants at risk for auditory neuropathy spectrum disorders. [42] Auditory neuropathy spectrum disorder (ANSD) is a hearing disorder which characterized with normal outer hair cell function but disrupted neural synchrony in the afferent auditory pathway. [43] The OTOF gene (Locus: DFNB9), encoding otoferlin, is reported to be one of the major causes of non-syndromic recessive sensorineural hearing loss, and is also reported to be the most common cause of non-syndromic recessive auditory neuropathy spectrum disorder (ANSD). [44] Audiological investigations revealed sudden onset auditory neuropathy spectrum disorder bilaterally. [45]OTOF 是遗传性听力损失的最常见原因之一，也是听神经病谱系障碍 (ANSD) 的主要原因。 [1] Klhl18 突变小鼠表现出罕见的低频听力障碍，其生理特征与听觉神经病谱系障碍 (ANSD) 一致。 [2] 听力损失是 Brown-Vialetto-Van Laere 综合征的一个特征，并且在最近的研究中表明其特征在于听神经病谱系障碍。 [3] 客观的 在儿科健康信息系统数据库中确定 2009 年至 2018 年新生儿重症监护病房 (NICU) 人群中听神经病谱系障碍 (ANSD) 的发病率及其危险因素。 [4] 该研究的目的是评估耳蜗病理学 (CP) 和听神经病谱系障碍患者的听觉流分离。 [5] 目的 本小型系统评价的目的是评估在 3 岁之前接受人工耳蜗植入 (CIs) 的诊断为听神经病谱系障碍 (ANSD) 的儿童的言语、语言和听觉行为结果测量的证据。 . [6] CND 包括少数对 DPOAE 有反应的病例，表明部分 CND 病例临床诊断为听神经病谱系障碍 (ANSD)。 [7] 为了完善与 OTOF 相关的听神经病谱系障碍 (ANSD) 的自然史和基因型-表型相关性，通过综合基因测试从被诊断为 OTOF 相关 ANSD 的不同人群中收集听力图和畸变产物耳声发射 (DPOAE)文献中也有报道。 [8] 客观的 研究噪声中的语音感知（信噪比-50）、差异阈值频率、时间调制传递函数、成人听力障碍清单以及听神经病谱系障碍的生活质量与感觉神经性听力损失有何不同。 [9] 背景 目前的研究试图使用一种新的方法，即对侧抑制 SOAE 来评估听神经病谱系障碍 (ANSD) 患者的传出听觉系统功能，该方法尚未得到广泛研究。 [10] 基于皮质听觉诱发电位 (CAEP) 的 P1 成分可作为听觉神经病谱系障碍 (ANSD) 儿童人工耳蜗植入 (CI) 结果的生物标志物。 [11] 目的 助听器作为听神经病谱系障碍（ANSD）儿童临床干预策略的益处仍然存在争议。 [12] 客观的 本文的目的是描述一名患有与 Brown-Vialetto-Van Laere (BVVL) 综合征相关的听神经病谱系障碍 (ANSD) 的儿童，这是一种由核黄素转运蛋白基因缺陷引起的罕见的遗传性神经退行性疾病。 [13] 背景：听觉神经通路中的听觉神经病谱系障碍（ANSD）会影响儿童的听觉和语言发育。 [14] 本研究旨在探索使用人工耳蜗 (CIs) 的听神经病谱系障碍 (ANSD) 儿童的中枢听觉系统发育，并找出 CAEP 生物标志物与言语感知的相关性。 [15] 该研究旨在调查患有和不患有听觉神经病谱系障碍 (ANSD) 的感觉神经性听力损失 (SNHL) 患者的工作记忆和语音识别。 [16] OTOF 基因突变是遗传性听力损失的常见原因，也是听神经病谱系障碍 (ANSD) 的主要原因。 [17] 方法 涉及听神经病谱系障碍的 15 名参与者。 [18] 听神经病谱系障碍是一种罕见的疾病，其中通过第 VIII 神经和听性脑干的神经传递被完整的外周听力破坏。 [19] 意义 听神经病谱系障碍 (ANSD) 是一种混杂的听觉疾病，受试者对声音有反应，但在言语辨别方面有困难。 [20] 目的评价术前经鼓室电诱发听觉脑干反应和术后神经反应遥测在听神经病谱系障碍患者中的应用。 [21] 客观的 本研究的目的是使用婴幼儿有意义听觉整合量表 (IT-MAIS) 和 Ling 6 声音测试 (Ling 6) 评估听神经病谱系障碍 (ANSD) 儿科患者的助听器听力结果. [22] 听神经病谱系障碍、迟发性听力损失、低频和轻微听力障碍有时会在新生儿听力筛查中被遗漏或稍后出现。 [23] 这是一例听神经病谱系障碍 (ANSD) 患者的病例报告，该患者在脑积水患者的分流器放置后得到解决。 [24] 目的 我们的研究试图确定耳鸣是否是听神经病谱系障碍 (ANSD) 患者的主要问题。 [25] 目的本研究旨在通过行为和神经测量来研究听觉神经病谱系障碍 (ANSD) 个体对听觉线索的辨别能力，并将结果与正常听力个体进行比较。 [26] 背景：听神经病谱系障碍 (ANSD) 是一种独特类型的 SNHL，其特征是存在耳声发射和/或耳蜗颤音。 [27] 结果：在两名患有听神经病谱系障碍 (ANSD) 的参与者中，观察到功能低下的热量反应以及前庭眼反射 (VOR) 增益和再固定扫视减少。 [28] 听神经病谱系障碍 (ANSD) 是一种复杂且异质的疾病，与听觉刺激的神经同步性改变有关。 [29] 目的 验证多重示范教学在听力和口语行为的获得和整合方面的效果，以及实质性和实质性 - 形容词组合，在患有听神经病谱系障碍 (ANSD) 和人工耳蜗 (CI) 的儿童中。 [30] 样本包括 40 名 4 岁至 11 岁 11 个月大的儿童，分为 4 组：（1）10 名听力正常的儿童； (2) 13名儿童助听器和调频系统； (3) 12名儿童使用人工耳蜗并安装调频系统； (4) 5 名被诊断患有听神经病谱系障碍的儿童，配备助听器和/或人工耳蜗以及调频系统。 [31] 客观的 探讨换血对重度高胆红素血症（SH）新生儿听神经病谱系障碍（ANSD）的影响。 [32] 研究根据任何程度的听力损失进行分类，文件的不同类别提供了轻度双侧、单侧和听神经病谱系障碍的具体标准。 [33] 听神经病谱系障碍 (ANSD)，也称为听神经病 (AN)，是一种独特的语前听力障碍。 [34] 介绍 听神经病谱系障碍 (ANSD) 的特点是听力损失范围从正常到严重。 [35] 听神经病谱系障碍 (ANSD) 的特征是由于突触功能或神经传导缺陷导致声音从耳蜗到大脑的功能失调。 [36] 据报道，编码 otoferlin 的 OTOF（基因座：DFNB9）是非综合征性听力损失的主要原因之一，也是非综合征性隐性听神经病谱系障碍的最常见原因。 [37] 目标 本研究的目的是：描述听神经病谱系障碍（ANSD）患者的诊断结果；并展示不同疗法的结果，如助听器 (HA) 或人工耳蜗植入。 [38] 我们报告了一名近亲出生的汉族男性，表现为非综合征性感觉神经性听力损失，其临床表型也与听神经病谱系障碍 (ANSD) 一致。 [39] 目标 听神经病谱系障碍 (ANSD) 是一种听力学诊断，其特征是在耳蜗中存在完整的外毛细胞功能的情况下出现听力障碍。 [40] 简介：已知患有听神经病谱系障碍 (ANSD) 的个体存在时间处理缺陷和较差的言语感知能力。 [41] 该计划包括针对所有新生儿的耳声发射 (OAE) 测试和针对失败的 OAE 和有听神经病谱系障碍风险的婴儿的自动听觉脑干反应 (A-ABR) 测试。 [42] 听神经病谱系障碍（ANSD）是一种听力障碍，其特点是外毛细胞功能正常，但传入听觉通路中的神经同步性被破坏。 [43] 据报道，编码 otoferlin 的 OTOF 基因（基因座：DFNB9）是非综合征隐性感觉神经性听力损失的主要原因之一，也是非综合征隐性听神经病谱系障碍的最常见原因。 ANSD）。 [44] 听力学调查显示双侧突发性听神经病谱系障碍。 [45]
non syndromic recessive
The OTOF (Locus: DFNB9), encoding otoferlin, is reported to be one of the major causes of non-syndromic hearing loss, and is also reported to be the most common cause of non-syndromic recessive auditory neuropathy spectrum disorder. [1] The OTOF gene (Locus: DFNB9), encoding otoferlin, is reported to be one of the major causes of non-syndromic recessive sensorineural hearing loss, and is also reported to be the most common cause of non-syndromic recessive auditory neuropathy spectrum disorder (ANSD). [2]据报道，编码 otoferlin 的 OTOF（基因座：DFNB9）是非综合征性听力损失的主要原因之一，也是非综合征性隐性听神经病谱系障碍的最常见原因。 [1] 据报道，编码 otoferlin 的 OTOF 基因（基因座：DFNB9）是非综合征隐性感觉神经性听力损失的主要原因之一，也是非综合征隐性听神经病谱系障碍的最常见原因。 ANSD）。 [2]
frequency modulated system
The sample included 40 children, aged 4 years to 11 years and 11 months old, divided into 4 groups: (1) 10 normal hearing children; (2) 13 children with hearing aids and frequency-modulated system; (3) 12 children using cochlear implant and fitted with the frequency-modulated system; and (4) 5 children diagnosed with auditory neuropathy spectrum disorder, fitted with hearing aids and/or cochlear implant and with the frequency-modulated system. [1]样本包括 40 名 4 岁至 11 岁 11 个月大的儿童，分为 4 组：（1）10 名听力正常的儿童； (2) 13名儿童助听器和调频系统； (3) 12名儿童使用人工耳蜗并安装调频系统； (4) 5 名被诊断患有听神经病谱系障碍的儿童，配备助听器和/或人工耳蜗以及调频系统。 [1]
Auditory Neuropathy Spectrum 听神经病谱
OTOF is one of the most frequent causes of hereditary hearing loss and a main cause of auditory neuropathy spectrum disorder (ANSD). [1] Klhl18 mutant mice exhibit an uncommon low frequency hearing impairment with physiological features consistent with Auditory Neuropathy Spectrum Disorder (ANSD). [2] Hearing loss is a characteristic feature of Brown-Vialetto-Van Laere syndrome and has been shown in recent studies to be characterised by auditory neuropathy spectrum disorder. [3] OBJECTIVE To determine the incidence of auditory neuropathy spectrum disorder (ANSD) and its risk factors among the neonatal intensive care unit (NICU) population from 2009 to 2018 in the Pediatric Health Information System database. [4] The aim of the study was to assess auditory stream segregation in individuals with cochlear pathology (CP) and auditory neuropathy spectrum disorder. [5] Objective The aim of this mini-systematic review was to evaluate the evidence reporting speech, language, and auditory behavioral outcome measures for children with a diagnosis of auditory neuropathy spectrum disorder (ANSD) who received cochlear implants (CIs) prior to 3 years of age. [6] CND includes a small number of cases that respond to DPOAE, indicating that some CND cases are clinically diagnosed with auditory neuropathy spectrum disorder (ANSD). [7] To refine the natural history and genotype-phenotype correlations of OTOF-related auditory neuropathy spectrum disorders (ANSD), audiograms and distortion product otoacoustic emissions (DPOAEs) were collected from a diverse cohort of individuals diagnosed with OTOF-related ANSD by comprehensive genetic testing and also reported in the literature. [8] OBJECTIVE To investigate how speech perception in noise (signal to noise ratio-50), difference limen frequency, temporal modulation transfer function, hearing handicap inventory for adults, and quality of life in auditory neuropathy spectrum disorder differs from sensorineural hearing loss. [9] BACKGROUND The current study attempted to assess efferent auditory system functioning in individuals with auditory neuropathy spectrum disorder (ANSD) using a new approach, contralateral suppression of SOAE, which has not yet been extensively researched. [10] The cortical auditory evoked potential (CAEP)-based P1 component acts as a biomarker for cochlear implantation (CI) outcomes in children with auditory neuropathy spectrum disorder (ANSD). [11] Objective The benefit of hearing aids as a clinical intervention strategy for children with Auditory Neuropathy Spectrum Disorder (ANSD) remains controversial. [12] OBJECTIVE The purpose of this paper is to describe a child with auditory neuropathy spectrum disorder (ANSD) associated with Brown-Vialetto-Van Laere (BVVL) syndrome, which is a rare, inherited, neurodegenerative disorder that is caused by defects in riboflavin transporter genes. [13] Background: Auditory neuropathy spectrum disorder (ANSD) in the auditory neural pathway can affect the auditory and speech development of children. [14] This study aimed to explore the central auditory system development in children with auditory neuropathy spectrum disorder (ANSD) using cochlear implants (CIs) and to find the correlation of CAEP biomarkers with speech perception. [15] The study aimed to investigate the working memory and speech recognition in noise from individuals with Sensori Neural Hearing Loss (SNHL) with and without auditory neuropathy spectrum disorder (ANSD). [16] Mutations in the OTOF gene are a common cause of hereditary hearing loss and the main cause of auditory neuropathy spectrum disorder (ANSD). [17] METHODS 15 participants with Auditory Neuropathy Spectrum Disorder were involved. [18] Auditory Neuropathy Spectrum Disorder is a rare condition wherein neural transmission through the VIIIth nerve and auditory brainstem is disrupted with intact peripheral hearing. [19] Significance Auditory neuropathy spectrum disorder (ANSD) is a confounding auditory disease in which the subjects respond to sound but have difficulties in speech discrimination. [20] Objective To evaluate the utility of pre-operative transtympanic electrically evoked auditory brainstem responses and post-operative neural response telemetry in auditory neuropathy spectrum disorder patients. [21] OBJECTIVE The objective of this study is to evaluate the audiologic outcomes with hearing aids in pediatric patients with auditory neuropathy spectrum disorder (ANSD) using the Infant Toddler-Meaningful Auditory Integration Scale (IT-MAIS), and the Ling 6 Sound Test (Ling 6). [22] Auditory neuropathy spectrum disorders, late-onset hearing loss, low-frequency and minimal hearing impairment are sometimes missed in newborn hearing screening or they manifest later. [23] This is a case report of a patient with Auditory Neuropathy Spectrum Disorder (ANSD) that resolved after shunt placement in a patient with hydrocephalus. [24] Objective Our study attempted to determine if tinnitus is a major concern in individuals with auditory neuropathy spectrum disorder (ANSD). [25] PurposeThe present study aimed to investigate the discrimination ability for acoustic cues in individuals with auditory neuropathy spectrum disorder (ANSD) using both behavioral and neural measures and to compare the result with normal hearing individuals. [26] Background: Auditory neuropathy spectrum disorder (ANSD) is a distinct type of SNHL that is characterized by the presence of otoacoustic emissions and/or cochlear microphonics. [27] Results: In two of the participants with auditory neuropathy spectrum disorder (ANSD), hypofunctional caloric responses were observed along with reduced vestibuloocular reflex (VOR) gain and refixation saccades. [28] Auditory neuropathy spectrum disorder (ANSD) is a complex and heterogeneous disorder associated with altered neural synchrony with respect to auditory stimuli. [29] PURPOSE To verify the effect of the multiple exemplar instruction at the acquisition and integration of listening and speaking behaviors, with substantives and substantive-adjective combinations, in children with Auditory Neuropathy Spectrum Disorder (ANSD) and cochlear implant (CI). [30] The sample included 40 children, aged 4 years to 11 years and 11 months old, divided into 4 groups: (1) 10 normal hearing children; (2) 13 children with hearing aids and frequency-modulated system; (3) 12 children using cochlear implant and fitted with the frequency-modulated system; and (4) 5 children diagnosed with auditory neuropathy spectrum disorder, fitted with hearing aids and/or cochlear implant and with the frequency-modulated system. [31] OBJECTIVE To explore the effects of exchange transfusion on auditory neuropathy spectrum disorder (ANSD) in neonates with severe hyperbilirubinemia (SH). [32] Studies were categorized according to hearing loss of any degree, with separate categories for documents providing specific criteria for mild bilateral, unilateral, and auditory neuropathy spectrum disorders. [33] Auditory neuropathy spectrum disorder (ANSD), also called auditory neuropathy (AN), is a unique type of prelingual hearing impairment. [34] INTRODUCTION Auditory Neuropathy Spectrum Disorder (ANSD) is characterized by hearing loss ranging from normal to profound. [35] Auditory neuropathy spectrum disorder (ANSD) is characterized by dysfunctional transmission of sound from the cochlea to the brain due to defective synaptic function or neural conduction. [36] The OTOF (Locus: DFNB9), encoding otoferlin, is reported to be one of the major causes of non-syndromic hearing loss, and is also reported to be the most common cause of non-syndromic recessive auditory neuropathy spectrum disorder. [37] OBJECTIVES The aims of the present study are to: describe diagnostic findings in patients with auditory neuropathy spectrum disorder (ANSD); and demonstrate the outcomes of different therapies like hearing aids (HAs) or cochlear implantation. [38] We report a Han Chinese male, born to consanguineous parents, presenting with nonsyndromic sensorineural hearing loss, whose clinical phenotype was also consistent with auditory neuropathy spectrum disorder (ANSD). [39] OBJECTIVES Auditory neuropathy spectrum disorder (ANSD) is an audiological diagnosis characterised by hearing dysfunction in the presence of intact outer hair cell function in the cochlea. [40] Introduction: Individuals with auditory neuropathy spectrum disorder (ANSD) are known to have temporal processing deficits and poor speech perception abilities. [41] The program includes the Otoacoustic Emissions (OAE) test for all newborns and Automated Auditory Brainstem Response (A-ABR) test for failed OAE and infants at risk for auditory neuropathy spectrum disorders. [42] Auditory neuropathy spectrum disorder (ANSD) is a hearing disorder which characterized with normal outer hair cell function but disrupted neural synchrony in the afferent auditory pathway. [43] The OTOF gene (Locus: DFNB9), encoding otoferlin, is reported to be one of the major causes of non-syndromic recessive sensorineural hearing loss, and is also reported to be the most common cause of non-syndromic recessive auditory neuropathy spectrum disorder (ANSD). [44] Audiological investigations revealed sudden onset auditory neuropathy spectrum disorder bilaterally. [45]OTOF 是遗传性听力损失的最常见原因之一，也是听神经病谱系障碍 (ANSD) 的主要原因。 [1] Klhl18 突变小鼠表现出罕见的低频听力障碍，其生理特征与听觉神经病谱系障碍 (ANSD) 一致。 [2] 听力损失是 Brown-Vialetto-Van Laere 综合征的一个特征，并且在最近的研究中表明其特征在于听神经病谱系障碍。 [3] 客观的 在儿科健康信息系统数据库中确定 2009 年至 2018 年新生儿重症监护病房 (NICU) 人群中听神经病谱系障碍 (ANSD) 的发病率及其危险因素。 [4] 该研究的目的是评估耳蜗病理学 (CP) 和听神经病谱系障碍患者的听觉流分离。 [5] 目的 本小型系统评价的目的是评估在 3 岁之前接受人工耳蜗植入 (CIs) 的诊断为听神经病谱系障碍 (ANSD) 的儿童的言语、语言和听觉行为结果测量的证据。 . [6] CND 包括少数对 DPOAE 有反应的病例，表明部分 CND 病例临床诊断为听神经病谱系障碍 (ANSD)。 [7] 为了完善与 OTOF 相关的听神经病谱系障碍 (ANSD) 的自然史和基因型-表型相关性，通过综合基因测试从被诊断为 OTOF 相关 ANSD 的不同人群中收集听力图和畸变产物耳声发射 (DPOAE)文献中也有报道。 [8] 客观的 研究噪声中的语音感知（信噪比-50）、差异阈值频率、时间调制传递函数、成人听力障碍清单以及听神经病谱系障碍的生活质量与感觉神经性听力损失有何不同。 [9] 背景 目前的研究试图使用一种新的方法，即对侧抑制 SOAE 来评估听神经病谱系障碍 (ANSD) 患者的传出听觉系统功能，该方法尚未得到广泛研究。 [10] 基于皮质听觉诱发电位 (CAEP) 的 P1 成分可作为听觉神经病谱系障碍 (ANSD) 儿童人工耳蜗植入 (CI) 结果的生物标志物。 [11] 目的 助听器作为听神经病谱系障碍（ANSD）儿童临床干预策略的益处仍然存在争议。 [12] 客观的 本文的目的是描述一名患有与 Brown-Vialetto-Van Laere (BVVL) 综合征相关的听神经病谱系障碍 (ANSD) 的儿童，这是一种由核黄素转运蛋白基因缺陷引起的罕见的遗传性神经退行性疾病。