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Auditory steady-state responses in babies with normal hearing and with temporary conductive hearing loss 听力正常和暂时性传导性听力损失婴儿的听觉稳态反应
Pub Date : 2011-02-22 DOI: 10.3109/1651386X.2010.538515
P. Watkin, Dani Tomlin, M. Baldwin
Abstract Objectives: To investigate whether test age and middle ear dysfunction had a significant effect on the variability of Auditory Steady-State Response (ASSR) thresholds when this test was undertaken on referrals from the newborn hearing screen. Methods: ASSR thresholds were measured at octave intervals from 500 Hz to 4000 Hz. They were recorded in 389 ears with normal hearing and middle ear function, and in a further 189 ears with temporary middle ear dysfunction. The modal corrected age at which the tests were undertaken was four weeks, with a minimum of one week and maximum corrected age of 26 weeks. Results: The mean ASSR thresholds of those with normal hearing were 47dB HL at 500 Hz, 36dB HL at 1000 Hz, and 34dB HL at both 2000 Hz and 4000 Hz. Standard deviations (SDs) from the mean ranged from 7.7 to 9.9dB. For those with middle ear dysfunction the mean ASSR thresholds were significantly greater at each frequency, with thresholds of 65dB HL at 500 Hz, 55dB HL at 1000 Hz, 53dB HL at 2000 Hz and 52dB HL at 4000 Hz. SDs from the mean ranged from 12.9 to 17.9dB. Mean thresholds decreased at all frequencies during the first half of infancy. In those with normal middle ear function the decrease was only 2dB at 500 Hz, but was over 5dB at both 1000 and 2000 Hz and was 4dB at 4000 Hz. A similar decrease was seen over the same period in those with middle ear dysfunction. Conclusions: ASSR thresholds measured in early infancy are affected by both test age and the presence of middle ear dysfunction and are more variable than ABR thresholds measured in babies with normal hearing or a mild conductive loss. A flexible test battery for gaining frequency specific information is therefore required at this age.
摘要:目的:研究新生儿听力筛查转诊患者进行听力稳态反应(ASSR)阈值测试时,测试年龄和中耳功能障碍是否对该测试的变异性有显著影响。方法:在500 ~ 4000 Hz的倍频区间测量ASSR阈值。他们记录了389只听力和中耳功能正常的耳朵,以及另外189只中耳功能暂时障碍的耳朵。进行试验时的模态校正年龄为四周,最小为一周,最大校正年龄为26周。结果:听力正常者的平均ASSR阈值为500 Hz时47dB HL, 1000 Hz时36dB HL, 2000 Hz和4000 Hz时34dB HL。平均值的标准差(sd)范围为7.7 ~ 9.9dB。对于中耳功能障碍患者,每个频率下的ASSR平均阈值明显更高,阈值为500hz时65dB HL, 1000hz时55dB HL, 2000hz时53dB HL, 4000hz时52dB HL。标准差从12.9到17.9dB不等。在婴儿期的前半段,所有频率的平均阈值都有所下降。在中耳功能正常的人群中,500hz时下降幅度仅为2dB,但在1000hz和2000hz时下降幅度均超过5dB, 4000hz时下降幅度为4dB。在同一时期,中耳功能障碍患者的听力也出现了类似的下降。结论:婴儿期早期测量的ASSR阈值受测试年龄和中耳功能障碍的影响,与听力正常或轻度传导性损失的婴儿测量的ABR阈值相比,其变化更大。因此,在这个年龄段需要一种灵活的测试电池来获得频率特定信息。
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引用次数: 2
Heart problems and deafness: Are they more common than supposed? 心脏问题和耳聋:它们比想象的更普遍吗?
Pub Date : 2011-02-22 DOI: 10.3109/1651386X.2011.558184
A. Martini, T. Volo, S. Ghiselli
In 2005, the World Health Organization (WHO) estimated that 12.5 million people worldwide are affected by bilateral profound hearing loss. Congenital hearing loss is the hereditary sensorial defect most commonly observed in newborns; it affects 1.12 per 1000 children. If we also consider acquired forms and late-onset forms of hearing loss, prevalence grows to 1.33 per 1000 children. In Italy the overall prevalence is 0.78 per 1000 for males and 0.69 per 1000 for females (1). Fifty percent of sensorineural deafness is due to genetic causes, most of which are inherited in an autosomal recessive manner. Genetic deafness is very heterogeneous and can occur in non-syndromic form (70% of cases) or in syndromic form (30% of cases). Several syndromes are associated with deafness, heart anomalies and other diseases: Charge syndrome, Leopard syndrome, Fabry syndrome, Refsum syndrome, SALL4 syndrome and Mobius syndrome. Until recently only one syndrome in which deafness and heart problems are the sole features has been described – Jervell and Lange-Nielsen syndrome (J-LNS). This condition, if not timely discovered and treated, may culminate in sudden death. However, in December 2010, Nature Neuroscience published an article about a new channelopathy termed SANDD syndrome, characterized by sinoatrial node dysfunction and deafness (2). These recent data underline the importance of a cardiac investigation in cases of suspected genetic deafness of unknown cause. It is probable that in the future other syndromes involving both the heart and the hearing system will be found. We have to consider that many of the causative genes of deafness have not yet been identifi ed. Furthermore, genes involved in hearing impairment encode proteins of different classes, such as cell adhesion and cytoskeleton proteins, transcription factors, extracellular matrix components, membrane
2005年,世界卫生组织(世卫组织)估计全世界有1 250万人受到双侧重度听力损失的影响。先天性听力损失是最常见于新生儿的遗传性感觉缺陷;每1000名儿童中有1.12名患有此病。如果我们还考虑后天性听力损失和晚发性听力损失,患病率上升到每1000名儿童1.33例。在意大利,男性的总体患病率为0.78 / 1000,女性为0.69 / 1000(1)。50%的感音神经性耳聋是由遗传原因引起的,其中大多数以常染色体隐性方式遗传。遗传性耳聋是非常异质性的,可以以非综合征形式(70%的病例)或综合征形式(30%的病例)发生。几种综合征与耳聋、心脏异常和其他疾病有关:Charge综合征、Leopard综合征、Fabry综合征、Refsum综合征、SALL4综合征和Mobius综合征。直到最近,只有一种以耳聋和心脏问题为唯一特征的综合征被描述- Jervell和Lange-Nielsen综合征(J-LNS)。这种情况,如果不及时发现和治疗,可能会导致猝死。然而,在2010年12月,《自然神经科学》发表了一篇关于一种新的通道病变称为SANDD综合征的文章,其特征是窦房结功能障碍和耳聋(2)。这些最近的数据强调了在怀疑原因不明的遗传性耳聋病例中进行心脏检查的重要性。将来可能还会发现涉及心脏和听力系统的其他综合征。我们必须考虑到许多耳聋的致病基因尚未被确定,此外,与听力障碍有关的基因编码不同类别的蛋白质,如细胞粘附蛋白和细胞骨架蛋白、转录因子、细胞外基质成分、膜
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引用次数: 1
A novel mutation in the vWFA2 domain of the COCH gene in an Italian DFNA9 family 意大利DFNA9家族COCH基因vWFA2结构域的新突变
Pub Date : 2011-02-22 DOI: 10.3109/1651386X.2010.538523
F. Faletra, N. Pirastu, E. Athanasakis, Alessio Somaschini, G. Pianigiani, P. Gasparini
Abstract Objective: Hearing loss (HL) is the most common form of sensory impairment, affecting millions of individuals worldwide. Pre-lingual non-syndromic hearing loss is present in 1 in 700 children and in half of the cases the hearing impairment is caused by genetic factors. Methods: We analysed an Italian family with a bilateral sensorineural HL present in several family members and inherited in an autosomal dominant manner. To perform a linkage study, subjects were genotyped with high density SNP arrays. Parametric linkage analysis using Merlin detected a significant 40-Mb locus on chromosome 14. Since the COCH gene, already known to be involved in causing HL, was located within this interval, a mutational search was carried out. Result: A novel mutation (p.A487P) affecting a highly conserved residue located within the vWFA2 domain was detected and segregates with the disease. Conclusion: This is the first Italian case of HL due to mutations within the COCH gene and it is also the first one showing some individuals affected by vestibular dysfunction due to the direct involvement of the vWFA2 domain.
摘要目的:听力损失(HL)是最常见的感觉障碍形式,影响着全世界数百万人。每700名儿童中就有1名出现语前非综合征性听力损失,其中一半的听力损害是由遗传因素引起的。方法:我们分析了一个意大利家庭与双侧感音神经性HL存在于几个家庭成员和遗传常染色体显性方式。为了进行连锁研究,用高密度SNP阵列对受试者进行基因分型。Merlin参数连锁分析在14号染色体上检测到一个显著的40mb位点。由于已知与HL致病相关的COCH基因位于这一区间,因此进行了突变搜索。结果:检测到一个新的突变(p.A487P)影响位于vWFA2结构域内的高度保守残基,并与该疾病分离。结论:这是意大利首例因COCH基因突变而导致的HL病例,也是第一例显示一些个体因vWFA2结构域直接参与前庭功能障碍而受到影响的病例。
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引用次数: 3
How useful are ‘add-on’ questions in questionnaires? 问卷中的附加问题有多有用?
Pub Date : 2011-02-22 DOI: 10.3109/1651386X.2011.558333
D. Stephens, I. Pyykkö
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引用次数: 5
Test-retest reliability and validity of Audioscan and Békésy compared with pure tone audiometry 与纯音测听法比较,重新测试了Audioscan和bsamuys的信度和效度
Pub Date : 2011-02-22 DOI: 10.3109/1651386X.2010.537124
W. Ishak, Fei Zhao, D. Stephens, J. Culling, Z. Bai, C. Meyer-bisch
Abstract Objective: With the development of more sophisticated and sensitive measures of auditory dysfunction, both the Audioscan test and Békésy audiometry can provide detailed information on hearing status between the octaves, which has clinical importance. This study investigated the reliability and validity of Audioscan and Békésy audiometry. Method: Audioscan and Bekesy audiometry were undertaken at a comparable sweep rate in 21 subjects with normal hearing. Each subject underwent conventional pure tone audiometry, Audioscan and Békésy audiometry. Test sessions were conducted on four consecutive days. Results: The results indicate that more than 90% of participants had variability of hearing threshold of less than 10dB at all frequencies. Median variances for thresholds obtained from Audioscan were less than 10dB2 for all frequencies except 4.0 kHz. In contrast, thresholds obtained from Békésy showed greater variance, more than 15dB2 at all frequencies when it was undertaken at the same sweep rate. Audioscan results were in excellent agreement with the PTA at all frequencies except at 0.25, 6 and 8 kHz, whereas Békésy results differed significantly from PTA at all frequencies except at 6 kHz. Conclusion: Data from the present study indicate that the Audioscan can provide reliable and valid hearing thresholds. However, a slow sweep rate appears to be more desirable for Békésy audiometry to improve its reliability and validity in detecting hearing sensitivity.
摘要目的:随着听觉功能障碍检测手段的发展,Audioscan测试和bsamk听力测量法均能提供八度音阶间的详细听力信息,具有重要的临床意义。本研究考察了Audioscan和bsamksamsy听力学的信度和效度。方法:对21名听力正常的受试者进行听力扫描和Bekesy听力测量。每个受试者都进行了常规纯音测听、Audioscan测听和b忧郁测听。测试阶段连续进行4天。结果:90%以上的被试在所有频率下的听阈变异性均小于10dB。除4.0 kHz外,Audioscan获得的阈值的中位数方差小于10dB2。相比之下,从bsamksamsy获得的阈值显示出更大的差异,当以相同的扫描速率进行时,在所有频率上都超过15dB2。除0.25、6和8 kHz外,音频扫描结果与PTA在所有频率上都非常一致,而bsamksamsy的结果与PTA在除6 kHz外的所有频率上都有显著差异。结论:本研究数据表明,Audioscan可以提供可靠有效的听力阈值。然而,较慢的扫描速率似乎是更理想的,以提高其在检测听觉灵敏度的可靠性和有效性。
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引用次数: 9
Aspects on quality of life of subjects with developmental language disorders 发展性语言障碍患者的生活质量
Pub Date : 2010-11-24 DOI: 10.3109/1651386X.2010.524796
E. Arkkila
Abstract This article is a brief overview on quality of life aspects in subjects with developmental language disorders. After the introduction, some results of personal studies on health related quality of life are summarized, followed by a more general discussion on the topic. To summarize, taking the self-perceptions of individuals with language disorders as part of clinical practice could improve the recognition of subjects with the greatest risk of diminished well-being subsequently.
摘要本文简要综述了发展性语言障碍患者的生活质量。在介绍之后,总结了一些关于健康相关生活质量的个人研究结果,然后对该主题进行了更广泛的讨论。综上所述,将语言障碍患者的自我认知作为临床实践的一部分,可以提高对随后幸福感下降风险最大的受试者的认识。
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引用次数: 2
Perception of speech disorders: Difference between the degree of intelligibility and the degree of severity 言语障碍的感知:可理解程度和严重程度之间的差异
Pub Date : 2010-11-24 DOI: 10.3109/1651386X.2010.525375
V. Woisard, B. Lepage
Abstract Objective: In the clinical practice of speech disorders, trained listeners are often used as judges for scaling procedures in the perceptual assessment of a speech signal. The aim of this paper is to study how a group of experts performs a task of severity judgment compared with a task of intelligibility judgment. Study design: During an off-line experiment, 33 stimuli were presented as icons on a Powerpoint slide. Each icon was associated with a line allowing scoring of each stimulus heard by a simple click on it; each stimulus was judged in terms of voice, resonance, prosody and phonemic qualities. With an analogical scale placed at the bottom of the screen, the judges were asked to put a copy of the icon along the line; the space above the line was used for severity and the one under the line for the deterioration of intelligibility, the arrow being oriented on the right side for the ‘most severe’ or ‘the least intelligible’ and representing a range from 1 to 30. Results: The mean scores of severity and deterioration of intelligibility were 9.8 (+/− 1.72) and 8.2 (+/− 1.59), respectively. Three judges differed in the distribution of the scores, whereas two others gave a similar score distribution. Where a difference exists, the trend is, in the mid-range of severity, to give a lower score for the deterioration of intelligibility. This is associated with a difference as to how the judges use the characteristics of speech analysed just before performing the judgment. Conclusion: There is an argument for measuring intelligibility at the surface code level with a word recognition test or ordinal scales and for allowing the use of interval scales for severity judgment.
摘要目的:在言语障碍的临床实践中,训练有素的听者经常被用作判断语音信号感知评估的尺度程序。本文的目的是研究一组专家如何执行严重性判断任务和可理解性判断任务。研究设计:在一个离线实验中,33个刺激物以图标的形式呈现在幻灯片上。每个图标都与一条线相关联,只需点击一下,就可以对听到的每个刺激进行评分;每一个刺激都是根据声音、共鸣、韵律和音位来判断的。在屏幕底部放置了一个类似的刻度,评委们被要求沿着线放一个图标的副本;线上的空格表示严重程度,线下的空格表示可理解性的恶化,箭头指向右侧表示“最严重”或“最不可理解”,代表从1到30的范围。结果:严重程度和可理解性恶化的平均得分分别为9.8分(+/ - 1.72)和8.2分(+/ - 1.59)。三名裁判的分数分布不同,而另外两名裁判的分数分布相似。在存在差异的地方,趋势是,在严重程度的中等范围内,对可理解性恶化给予较低的分数。这与法官在执行判决之前如何使用所分析的言语特征的差异有关。结论:用单词识别测试或顺序量表来测量表面代码水平的可理解性和允许使用间隔量表来判断严重程度是有争议的。
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引用次数: 4
Type-D personality, psychosomatic symptoms and voice handicap in female voice patients: A perspective on vocal communication 女声患者的d型人格、心身症状与发声障碍:一个声音交流的视角
Pub Date : 2010-11-24 DOI: 10.3109/1651386X.2010.525302
M. V. Van Opstal, L. Claes, R. Smits, F. de Jong
Abstract Objectives: The association between vocal communication and personality was studied in 82 female voice patients with a mean age of the 39.3 years (range 18–65 years). Study design: The relation between type-D personality (Type-D Scale-16), psychosomatic symptoms (Symptom Check List–90) and the biopsychosocial impact of the voice problem (Voice Handicap Index) was assessed. Results: The findings indicate that type-D voice patients are relatively more handicapped by their voice problem, predominantly in the emotional domain, and have a relatively higher level of psychosomatic symptoms compared to non-type-D patients. Conclusions: Apparently, the well-being of females with a voice problem is more at risk in type-D than in non-type-D personalities. These results lead to the suspicion that voice patients with a type-D personality are especially at risk because they are prone to be involved in a circular negatively reinforcing process. This process influences the vocal problem in such a way that a detrimental course of vocal communication possibly occurs.
摘要目的:对82例平均年龄39.3岁(18 ~ 65岁)的女性嗓音患者进行嗓音交际与人格的相关性研究。研究设计:评估d型人格(d型量表-16)、心身症状(症状检查表- 90)与声音问题(声音障碍指数)的生物心理社会影响之间的关系。结果:与非d型患者相比,d型患者的声音障碍相对更严重,主要表现在情绪领域,心身症状水平相对更高。结论:显然,有声音问题的d型人格女性比非d型人格女性的健康状况更危险。这些结果让人怀疑,d型人格的声音患者尤其有风险,因为他们容易陷入一个循环的负面强化过程。这一过程对发声问题的影响可能会导致发声交流的不利过程。
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引用次数: 2
AAC in communication disorders 交流障碍中的AAC
Pub Date : 2010-11-24 DOI: 10.3109/1651386X.2010.530024
E. Genovese, M. Vallarino, D. Farneti
Abstract Any condition that alters the acquisition of a morpho-syntactic organization of thought will compromise communicative interaction. Verbal disability relates to diversified morbid conditions; these could be congenital or acquired, permanent or temporary and could arise over the entire lifespan. The degree of verbal disability presents a different range of severity and not only involves speech but cognitive and emotional aspects. In all these conditions a possibility of communication can be facilitated or substituted through ecological or mediate interventions, using technological devices of differing complexity. AAC (Augmentative Alternative Communication) refers to any communication method used to increase natural communication or to replace it if entirely absent. The presuppositions and the general principles of AAC are described.
任何改变思维形态句法组织习得的条件都会损害交际互动。语言障碍涉及多种病症;这些可能是先天性的或后天的,永久的或暂时的,并可能在整个生命周期中出现。语言障碍的严重程度不同,不仅涉及语言方面,还涉及认知和情感方面。在所有这些条件下,通过使用不同复杂程度的技术设备进行生态或调解干预,可以促进或取代交流的可能性。AAC (augative Alternative Communication)是指任何一种用来增加自然沟通的沟通方式,或者在完全缺乏自然沟通的情况下替代自然沟通方式。介绍了AAC的前提条件和一般原则。
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引用次数: 1
Usefulness of low redundancy speech tests with dyslexic children 低冗余度言语测试在阅读困难儿童中的应用
Pub Date : 2010-11-24 DOI: 10.3109/1651386X.2010.524473
W. Wojnowski, B. Wiskirska-Woźnica, A. Pruszewicz, Barbara Maciejewska, G. Demenko, P. Świdziński
Abstract Objectives: Developmental dyslexia in children can affect auditory and linguistic skills. Due to the impairment of hearing discrimination, attention, memory and perception, dyslexia causes the inability to process and interpret linguistic and verbal information effectively. Standard audiological examination is much less useful than low redundancy speech tests and electro-physiological examination, which are the only objective measures of central auditory processing. The aim of the study was to assess the usefulness of low redundancy speech tests and dichotic tests in the diagnosis of developmental dyslexia in children. Methods: Ten children aged 7–15 years were tested by a dichotic numeral test, a dichotic verbal minimal-pair test, and a Calearo test. Results: The experiments demonstrated that the dyslectic group achieved worse results in the above tests compared to the control group. This suggests information exchange disorders between the hemispheres, a lack of synchronization of acoustic perception in dichotic tests, and damage of the structures responsible for central auditory processing in the dominating hemisphere. Conclusions: The results of our study confirm the existence of information exchange disorders between the hemispheres and the lack of synchronization of acoustic perception in dichotic tests, where the signal is presented to both ears simultaneously. Much worse results in low redundancy tests reveal, moreover, damage of the structures responsible for central auditory processing in the dominating hemisphere. Altogether, this research has proved the usefulness of the conducted tests in the diagnostics of central auditory processing disorders in patients with dyslexia.
摘要目的:儿童发展性阅读障碍会影响其听觉和语言技能。由于听力、辨别、注意力、记忆和感知的障碍,阅读障碍导致无法有效地处理和解释语言和言语信息。标准的听力学检查远不如低冗余语音测试和电生理检查有用,这是唯一客观衡量中央听觉处理的方法。本研究的目的是评估低冗余言语测验和二分测验在儿童发展性阅读障碍诊断中的有用性。方法:对10名7 ~ 15岁儿童进行数字二分测验、言语二分最小对测验和Calearo测验。结果:实验表明,与对照组相比,阅读障碍组在上述测试中的成绩较差。这表明两个半球之间的信息交换障碍,在二分测试中缺乏声音感知的同步,以及在占主导地位的半球负责中央听觉处理的结构受损。结论:我们的研究结果证实了两个半球之间的信息交换障碍的存在,以及在双耳测试中缺乏声音感知的同步,其中信号同时呈现给两只耳朵。在低冗余测试中,更糟糕的结果显示,主要半球负责中央听觉处理的结构受损。总之,这项研究已经证明了进行的测试在诊断患有阅读障碍的患者的中枢听觉处理障碍方面的有用性。
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引用次数: 0
期刊
Audiological medicine
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