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Critical Factors for Binaural Hearing in Children with Bilateral Sequential Cochlear Implantation: First Implant Performance and Inter-Implant Interval 双侧序贯人工耳蜗植入术儿童双耳听力的关键因素:首次人工耳蜗的表现和间隔时间
Pub Date : 2019-08-08 DOI: 10.1159/000500700
J. Jang, Ji-Min Roh, O. Choo, You-Jeong Kim, Hantai Kim, H. Park, Y. Choung
Objectives: This study evaluated the influence of the performance of the first cochlear implant (CI-1) on the second implant (CI-2) and the significance of inter-implant intervals in children with sequential cochlear implantation. Method: Thirty-four patients were included for speech perception outcome and 38 patients were included for subjective hearing satisfaction in daily life in this study. The patients were classified into 3 groups according to inter-implant interval: group I, <4 years; group II, 4–7 years; group III, >7 years. Open-set speech perception scores before and at 3, 6, 12, and 24 months after the second implantation was compared among the CI-1, CI-2, and bilateral CI conditions. Subjective hearing benefits in daily life were measured using a questionnaire of Speech, Spatial, and Qualities of Hearing Scale (SSQ) for parents of children with impaired hearing, and device use and preference were evaluated (by phone interview.) Results: The speech perception of children using the CI-2 only improved quickly within 3 months postoperatively and were similar to those using the CI-1 only. The results of monosyllabic and disyllabic word tests using CI-2 at 3 months after second implantation were not significantly different from those in patients using the CI-1 only. In the sentence test, the scores using the CI-2 only were not significantly different from those using the first implant only at 6 months after second implantation. SSQ scores were similar among groups and the worst score in each section was shown from the questions about performance under noisy conditions in each section. Device usage habits showed that 17 (44.7%) and 16 (42.1%) patients preferred bilateral and the CI-1, respectively. Only 5 (13.2%) patients wanted the CI-2 rather than the CI-1. While group I and II showed a prominent positive attitude to bilateral use, group III, for which the inter-implant interval was 7 years or more, showed a definite preference for the CI-1 (p < 0.01). Conclusions: Functional binaural benefits were achieved in patients who were good performers with the CI-1 after the second implantation irrespective of the inter-implant interval. Sequential CI should be strongly recommended for patients with unilateral CI showing a good performance.
目的:本研究评价序贯人工耳蜗植入术儿童首次人工耳蜗(CI-1)性能对第二次人工耳蜗(CI-2)性能的影响及间隔时间的意义。方法:对34例患者进行语音感知评分,对38例患者进行日常生活主观听力满意度评分。根据种植间间隔将患者分为3组:I组,7年。在CI-1、CI-2和双侧CI条件下,比较第二次植入前和第3、6、12和24个月的开放集语音感知评分。对听力障碍儿童的父母使用语音、空间和听力质量量表(SSQ)来测量日常生活中的主观听力益处,并通过电话访谈评估设备的使用和偏好。结果:单纯使用CI-2组患儿术后3个月内言语感知改善较快,与单纯使用CI-1组患儿相近。第二次植入后3个月使用CI-2的单音节和双音节单词测试结果与仅使用CI-1的患者无显著差异。在句子测试中,仅使用CI-2的患者在第二次种植后6个月的得分与仅使用第一次种植的患者无显著差异。SSQ得分在各组之间相似,每个部分的最差得分来自每个部分在嘈杂条件下的表现问题。器械使用习惯分别显示17例(44.7%)和16例(42.1%)患者偏好双侧和CI-1。只有5例(13.2%)患者需要CI-2而不是CI-1。I组和II组对双侧使用CI-1表现出明显的积极态度,而III组对CI-1表现出明确的偏好,种植体间间隔为7年或更长(p < 0.01)。结论:在第二次植入后,无论植入间隔如何,使用CI-1性能良好的患者均可获得双耳功能益处。对于单侧CI表现良好的患者,应强烈推荐序贯CI。
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引用次数: 16
Audiovestibular Phenotypes and Advanced Magnetic Resonance Imaging Features of Cochlin Gene Mutation Carriers 耳蜗基因突变携带者的听前庭表型和高级磁共振成像特征
Pub Date : 2019-08-07 DOI: 10.1159/000501292
G. Conte, F. Lo Russo, L. Caschera, D. Zanetti, P. Castorina, C. Sina, F. Triulzi, F. Di Berardino
Objective: To describe clinical and imaging findings in a group of patients affected by nonsyndromic deafness A9 (DFNA9), using advanced magnetic resonance imaging (MRI) with 3-dimensional (3D) fluid-attenuated inversion recovery (FLAIR) sequence. Method: A retrospective case review was conducted in a tertiary referral center in Italy. Four sequential adult DFNA9-affected patients, who had undergone MRI at our Department between January 2017 and June 2018, were enrolled (male = 2, female = 2; median age: 65.6 years; 8 diseased ears analyzed). Three patients were relatives; the fourth was unrelated. The main outcome measures – age, sex, records of audiological and vestibular testing, genetic assessment, MRI findings – were analyzed. Results: All subjects suffered from bilateral progressive sensorineural hearing loss, more severely at the high frequencies and with a typical clinical pattern of bilateral chronic degenerative cochleovestibular deficit. Aural fullness was reported at the onset of the disease. All patients revealed a pathogenic heterozygous mutation in the Limulus factor C, Coch-5b2 and Lgl1 domain of cochlin. None of the patients showed a significant vestibular and cochlear endolymphatic hydrops at MRI, while high bilateral contrast enhancement on 4-h delayed postcontrast 3D FLAIR sequence was observed in all ears. Conclusions: Increased perilymph enhancement on 4-h delayed postcontrast 3D FLAIR sequence is the common imaging feature of DFNA9 ears, suggesting that blood-labyrinthine barrier breakdown may play the main role in the pathophysiology of this disease. Significant hydrops has been excluded by MRI. This finding might be clinically useful in differentiating DFNA9 disease from other pathologies with similar clinical findings like Ménière’s disease.
目的:利用三维(3D)液体衰减反转恢复(FLAIR)序列的先进磁共振成像(MRI)描述一组非综合征性耳聋A9 (DFNA9)患者的临床和影像学表现。方法:对意大利某三级转诊中心的病例进行回顾性分析。本研究纳入了2017年1月至2018年6月期间在我科接受MRI检查的4例成年dfna9影响患者(男2例,女2例;中位年龄:65.6岁;分析8只患病耳)。3例患者为亲属;第四个与此无关。分析了主要结局指标——年龄、性别、听力学和前庭测试记录、遗传评估、MRI结果。结果:所有受试者均出现双侧进行性感音神经性听力损失,高频加重,临床表现为双侧慢性退行性耳蜗前庭功能障碍。在发病时报告有听觉充盈。所有患者均在耳蜗的limus因子C、Coch-5b2和Lgl1结构域出现致病性杂合突变。所有患者在MRI上均未出现明显的前庭和耳蜗内淋巴积液,而在4小时延迟后3D FLAIR序列上观察到双侧高对比度增强。结论:延迟4 h后3D FLAIR序列淋巴周围增强是DFNA9耳的常见影像学特征,提示血迷路屏障破坏可能在本病的病理生理中起主要作用。MRI已排除明显水肿。这一发现可能在临床上有助于将DFNA9疾病与其他具有类似临床表现的病理(如msamimni病)区分开来。
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引用次数: 1
Front & Back Matter 正面和背面
Pub Date : 2019-08-01 DOI: 10.1159/000502554
J. Harris
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引用次数: 0
Impairment of Vestibular Function and Balance Control in Patients with Type 2 Diabetes 2型糖尿病患者前庭功能损害及平衡控制
Pub Date : 2019-07-19 DOI: 10.1159/000501291
Jin Li, Jana Jiang, Yi Zhang, Bo Liu, Luo Zhang
Background: Recent evidence suggests that falls are a major complication of diabetes in elderly patients, leading to disability and preventable death. However, the potential risk factors leading to falls in patients with type 2 diabetes are not fully understood. This study was designed to explore the characteristics of vestibular dysfunction and balance control in patients with type 2 diabetes and to analyse the risk factors associated with falls. Methods: The study recruited 51 patients with type 2 diabetes and 43 controls who underwent vestibular function tests and balance control capability tests between January 2013 and December 2015. Vestibular function and balance control capability assessment was based on slow-phase velocity, canal paresis, Sensory Organisation Test (SOT) score, Limits of Stability Test (LOS) score, and Motor Control Test (MCT) score. Results: In all, 56.7% of the diabetic patients had vestibular dysfunction, compared with 27.9% of the controls (p = 0.005). Vestibular dysfunction was dependent on the duration of the disease and serum HbA1c levels. There were no significant differences between the two groups with respect to the balance test results for SOT score, somatosensory subtest score, vestibular subtest score, or LOS score. However, the visual system and MCT scores were significantly lower in the diabetic patients than in the controls (p = 0.032 and p = 0.018, respectively). Conclusions: Patients with type 2 diabetes have a higher incidence of vestibular dysfunction. Vestibular dysfunction, visual system impairment, and a decline in motion control may be the risk factors that can lead to falls, and thus need to be managed accordingly in diabetic patients.
背景:最近的证据表明,跌倒是老年糖尿病患者的主要并发症,可导致残疾和可预防的死亡。然而,导致2型糖尿病患者跌倒的潜在危险因素尚不完全清楚。本研究旨在探讨2型糖尿病患者前庭功能障碍和平衡控制的特点,并分析与跌倒相关的危险因素。方法:本研究招募了51例2型糖尿病患者和43例对照组,于2013年1月至2015年12月期间进行了前庭功能测试和平衡控制能力测试。前庭功能和平衡控制能力的评估基于慢相速度、管性麻痹、感觉组织测试(SOT)评分、稳定极限测试(LOS)评分和运动控制测试(MCT)评分。结果:56.7%的糖尿病患者有前庭功能障碍,对照组为27.9% (p = 0.005)。前庭功能障碍取决于病程和血清HbA1c水平。两组在平衡测试SOT评分、体感亚测试评分、前庭亚测试评分和LOS评分方面无显著差异。然而,糖尿病患者的视觉系统和MCT评分明显低于对照组(p = 0.032和p = 0.018)。结论:2型糖尿病患者前庭功能障碍发生率较高。前庭功能障碍、视觉系统损伤和运动控制能力下降可能是导致跌倒的危险因素,因此需要对糖尿病患者进行相应的管理。
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引用次数: 8
Fluctuating Sensorineural Hearing Loss 波动感音神经性听力损失
Pub Date : 2019-07-17 DOI: 10.1159/000500658
Hui Liu, Kunpeng Zhou, Xuemei Zhang, K. Peng
Background: Several otologic conditions can present with fluctuating sensorineural hearing loss, including Ménière’s disease, autoimmune inner ear disease, and enlarged vestibular aqueduct. Although these 3 etiologies vary greatly, distinguishing between these conditions at initial presentation can be challenging. Furthermore, initial treatment of these conditions is often similar. In this review, we discuss historical and current perspectives on diagnosis and treatment of these conditions. Summary: A literature search was performed regarding fluctuating hearing loss, and current treatment of these etiologies of fluctuating hearing loss was summarized. Immediate measures at the onset of acute hearing loss include corticosteroid therapy, while preventative and chronic therapies, which can limit disease severity and frequency, vary based on the specific condition treated. Key Messages: Fluctuating hearing loss can represent a range of pathologies, but the precise etiology may not be clear at initial presentation. Timely treatment and long-term follow-up, along with appropriate diagnostics, are necessary to optimize long-term hearing.
背景:一些耳科疾病可表现为波动感音神经性听力损失,包括msamuni病、自身免疫性内耳疾病和前庭导水管扩大。虽然这三种病因差异很大,但在最初表现时区分这些疾病可能具有挑战性。此外,这些疾病的初始治疗通常是相似的。在这篇综述中,我们讨论了历史和当前的诊断和治疗这些疾病的观点。摘要:我们检索了有关波动性听力损失的文献,并总结了波动性听力损失病因的目前治疗方法。急性听力损失发病时立即采取的措施包括皮质类固醇治疗,而可以限制疾病严重程度和频率的预防性和慢性治疗则根据所治疗的具体情况而有所不同。关键信息:波动性听力损失可以代表一系列的病理,但确切的病因在最初的表现可能不清楚。及时治疗和长期随访,以及适当的诊断,是优化长期听力的必要条件。
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引用次数: 9
Cochlear Nerve Hypoplasia: Audiological Characteristics in Children and Adults 耳蜗神经发育不全:儿童和成人的听力学特征
Pub Date : 2019-07-15 DOI: 10.1159/000500938
B. Cinar, E. Tahir, Merve Ozbal Batuk, M. Yaralı, G. Sennaroğlu, L. Sennaroğlu
Background: Cochlear nerve deficiency is a general term used to describe both cochlear nerve hypoplasia (CNH) and cochlear nerve aplasia. Although these two conditions can have similar results on audiological evaluation, CNH yields more variation in audiological tests. Objectives: To describe the audiological characteristics of the CNH cases in our series in relation to radiological findings. Methods: We reviewed the medical charts, audiological findings, and radiological findings on cases with CNH. We included cases with CNH in one ear or both ears. Out of 90 subjects with CNH, we included a total of 40 individuals (21 women and 19 men; 49 ears) in the current study. We reviewed and analyzed the participants’ audiological test results according to the radiological findings. Results: Cases with CNH showed variations according to the cochlear structure. There were 13 normal cochleae, 4 with incomplete partition type I, and 32 with cochlear hypoplasia. The accompanying cochlear apertures also showed variation: 17 were normal, 28 stenotic, and 4 aplastic cochlear apertures. The subjects displayed hearing loss ranging from moderate to profound; furthermore, 4 subjects had no response to sound whatsoever. The degree of hearing loss was not statistically significantly different with regard to the presence or absence of cochlear malformation with CNH (p > 0.005). We observed both sensorineural hearing loss and mixed-type hearing loss among the CNH cases. Conclusions: CNH is the presence of a cochlear nerve that is smaller in diameter than the facial nerve. It can be accompanied with other associated inner ear malformations of different degrees of severity. We observed degrees of hearing loss ranging from moderate to profound.
背景:耳蜗神经缺损是耳蜗神经发育不全(CNH)和耳蜗神经发育不全的总称。虽然这两种情况在听力学评估中有相似的结果,但CNH在听力学测试中产生更多的差异。目的:描述本系列CNH病例的听力学特征与影像学表现的关系。方法:我们回顾了CNH病例的医学图表、听力学表现和影像学表现。我们纳入了单耳或双耳CNH病例。在90名CNH患者中,我们共纳入了40名个体(21名女性和19名男性;在当前的研究中。我们根据放射学结果回顾和分析了参与者的听力学测试结果。结果:CNH病例表现出不同耳蜗结构的差异。正常耳蜗13例,ⅰ型不完全分割耳蜗4例,耳蜗发育不全32例。伴随的耳蜗孔也有差异:17个正常,28个狭窄,4个再生耳蜗孔。受试者表现出中度到重度的听力损失;此外,4名受试者对声音没有任何反应。CNH患者的听力损失程度与是否存在耳蜗畸形差异无统计学意义(p < 0.05)。我们在CNH病例中观察到感觉神经性听力损失和混合型听力损失。结论:CNH是指耳蜗神经直径小于面神经。它可以伴随其他相关的内耳畸形不同程度的严重程度。我们观察到听力损失的程度从中度到重度不等。
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引用次数: 2
Front & Back Matter 正面和背面
Pub Date : 2019-07-01 DOI: 10.1159/000502217
A. Arora, L. Cascarini, J. Jeannon
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引用次数: 0
Front & Back Matter 正面和背面
Pub Date : 2019-05-01 DOI: 10.1159/000501054
J. Harris
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引用次数: 0
Front & Back Matter 正面和背面
Pub Date : 2019-03-01 DOI: 10.1159/000499615
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引用次数: 0
Contents Vol. 23, 2018 目录2018年第23期
Pub Date : 2019-03-01 DOI: 10.1159/000497736
D. Cotanche, R. Häusler, G. Housley, P. Jastreboff, M. Kenna, P. Lefebvre, L. Luxon, G. Manley, J. Melcher
Maurizio Barbara – University La Sapienza, Rome, Italy Olivier Bertrand – Centre Hospitalier Le Vinatier, Bron, France Thomas Brandt – Ludwig Maximilians University, Munich, Germany Barbara Canlon – Karolinska Institute, Stockholm, Sweden Douglas A. Cotanche – Harvard School of Public Health, Boston, USA Cor W.R.J. Cremers – University Medical Center St. Radboud, Nijmegen, The Netherlands Norbert Dillier – University Hospital, Zurich, Switzerland Robert Dobie – University of Texas Health Science Center, San Antonio, USA Manuel Don – House Ear Institute, Los Angeles, USA Jill B. Firszt – Washington University, St. Louis, USA Andrew Forge – University College London, London, United Kingdom Bernard Fraysse – Hôpital Purpan, Toulouse, France Rick Friedman – House Ear Institute, Los Angeles, USA Bruce J. Gantz – University of Iowa Hospitals and Clinics, Iowa City, USA Anthony W. Gummer – University of Tübingen, Tübingen, Germany James W. Hall, III. – University of Florida, Gainesville, USA Joseph W. Hall – University of North Carolina, Chapel Hill, USA Michael Halmagyi – Royal Prince Alfred Hospital, Camperdown, Australia The Science of Hearing and Balance
Maurizio Barbara -意大利罗马拉萨皮恩扎大学Olivier Bertrand -法国布朗勒维纳蒂埃医院中心Thomas Brandt -德国慕尼黑路德维希马克西米利安大学Barbara Canlon -瑞典斯德哥尔摩卡罗林斯卡研究所Douglas A. Cotanche -美国波士顿哈佛大学公共卫生学院Cor W.R.J. Cremers -荷兰奈梅亨圣拉德梅亨大学医学中心Norbert Dillier -苏黎世大学医院瑞士Robert Dobie -美国圣安东尼奥德克萨斯大学健康科学中心Manuel Don - House耳研究所,美国洛杉矶Jill B. Firszt -美国圣路易斯华盛顿大学Andrew Forge -伦敦大学学院,英国伦敦Bernard Fraysse - Hôpital Purpan,法国图卢兹Rick Friedman - House耳研究所,美国洛杉矶Bruce J. Gantz -爱荷华大学医院和诊所,美国爱荷华市Anthony W. Gummer - t宾根大学,詹姆斯·w·霍尔,III。-美国盖恩斯维尔佛罗里达大学Joseph W. Hall -美国教堂山北卡罗来纳大学Michael Halmagyi -澳大利亚坎珀当皇家阿尔弗雷德王子医院听力与平衡科学
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引用次数: 0
期刊
Audiology and Neurotology
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