先天性重度、极重度感音神经性聋患儿内耳及颅脑影像学评估

目的探讨先天性重度、极重度感音神经性聋患儿中内耳畸形及颅脑异常的发病情况,为人工耳蜗植入适应证的选择提供参考。方法对确诊为先天性重度、极重度感音神经性聋的245例患儿进行影像学检查,其中232例行颞骨高分辨率CT检查,204例行内听道MRI检查,196例行常规颅脑MRI检查,分析内耳结构异常及颅脑异常患儿的影像表现。结果行颞骨高分辨率CT扫描的232例中,195例内耳正常,37例内耳异常,占15.95%;行内听道MRI检查的204例中,167例正常,37例异常,占18.14%;行颅脑MRI平扫的196例中,110例正常,86例异常,占43.88%。耳蜗畸形包括:Michel畸形3例4耳,耳蜗未发育4例7耳,耳蜗发育不全2例3耳,不完全分隔Ⅰ型5例8耳,不完全分隔Ⅱ型5例10耳。前庭畸形16例26耳,半规管畸形14例24耳;内听道畸形7例12耳;前庭导水管畸形16例29耳。颅脑异常者包括:脑白质病61例,颅内发育变异9例,脑先天畸形7例,脑损伤、破坏性疾病6例,颅骨异常2例及松果体囊肿1例。结论本组先天性重度或极重度感音神经性聋患儿内耳畸形发生率为15.10%(37/245),颅脑异常发生率为43.88%(86/196),术前常规进行颞骨高分辨率CT、内听道MRI及颅脑MRI检查能够发现内耳及颅脑病变,可作为人工耳蜗植入适应人群的筛选及植入耳选择的参考。     

小儿专科医院开展人工耳蜗植人手术的优势

目的通过本组6家小儿专科医院开展人工耳蜗植入手术的临床工作,讨论小儿专科医院开展人工耳蜗植入手术的优势。方法从1998年5月-2009年3月11年间,本组6家小儿专科医院耳鼻咽喉科共开展了103例人工耳蜗植入手术。术前信息：全部为双耳重度和极重度感音神经性聋,年龄8．5个月-10．2岁,平均年龄为3．8岁,全部为语前聋患儿。术前进行听力学评估、发育和智力评估、影像学评估、人工耳蜗植入适应证的评估。术前进行全麻风险的评估。进行与综合性医院开展人工耳蜗植入手术的优势比较,包括：①术前评估水平,②术中麻醉风险,③人工耳蜗植入手术结果,④围手术期护理和并发症处理水平。结果①术前评估：术前视觉强化行为测听检查：103例查出有残留听力49例,平均听力98dB,余54例无残留听力。畸变产物耳声发射检查：103例均未引出耳声发射。听觉脑干诱发电位、40Hz、多频稳态诱发电位检查：引出波形41例,未引出波形62例。术前影像学检查：颞骨CT检查103例中正常颞骨形态84例,内耳畸形19例,其中双耳大前庭导水管畸形ll例、Mondini畸形7例、前庭与外半规管共同腔畸形1例。脑常规MRI检查,正常98例、脑白质轻度异常5例。行为和智力测试：格雷费斯智力测试〉86分99例、〈86分4例。术前检查结果与综合性医院检查结果比较,无显著差异。②术中麻醉风险：全麻插管、拔管中出现心电、氧、二氧化碳监测问题、气管支气管痉挛并发症0例,综合性医院发生率为0．37％,有明显的安全优势。③人工耳蜗植入手术结果：103例手术均成功,与综合性医院比较,无明显差异。④围手术期护理与并发症的处理：103例术前常规静脉采血、术中,术后常规建立静脉通道未出现失败,综合性医院出现静脉采血、建立静脉通道失败率约5%右,?     

CT与磁共振成像在儿童人工耳蜗植入术前研究

目的 评价CT与磁共振成像（magnetic resonance imaging,MRI)在儿童人工耳蜗植入术前的诊断作用，以及对手术选择的影响。方法 71例（142耳）双耳重度耳聋、拟行人工耳蜗植入的患儿进行术前CT与MRI检查。结果 CT与MRI均发现，12例（22耳）患者有前庭水管扩大（15.5%)；14例（25耳）患儿有Mondini畸形（17.6%)；3例（5耳）患儿有内耳道扩大（3.5%)；2例（4耳）患者有可疑内耳道底骨质缺损（2.8%)。MRI发现有5例（5耳）患者单侧耳蜗纤维化（3.5%)，而CT未见异常。1例（2耳）患儿的CT显示面神经裸露（1.4%)，MRI正常。结论 人工耳蜗植入术前应该进行CT与MRI检查。对于发现前庭水管综合征、Mondini畸形、内耳道扩张及内耳道底骨质缺损有重要意义。这二种影像学检查结果可以相互补充诊断耳蜗纤维化与面神经裸露。对手术适应证的选择以及保证手术正常进行有重要意义。     

CT与磁共振成像在儿童人工耳蜗植入术前研究

目的　评价CT与磁共振成像 (magneticresonanceimaging ,MRI)在儿童人工耳蜗植入术前的诊断作用 ,以及对手术选择的影响。方法　 71例 ( 142耳 )双耳重度耳聋、拟行人工耳蜗植入的患儿进行术前CT与MRI检查。结果　CT与MRI均发现 ,12例 ( 2 2耳 )患者有前庭水管扩大 ( 15 5 % ) ;14例 ( 2 5耳 )患儿有Mondini畸形 ( 17 6% ) ;3例 ( 5耳 )患儿有内耳道扩大 ( 3 5 % ) ;2例 ( 4耳 )患者有可疑内耳道底骨质缺损 ( 2 8% )。MRI发现有 5例 ( 5耳 )患者单侧耳蜗纤维化 ( 3 5 % ) ,而CT未见异常。1例 ( 2耳 )患儿的CT显示面神经裸露 ( 1 4% ) ,MRI正常。结论　人工耳蜗植入术前应该进行CT与MRI检查。对于发现前庭水管综合征、Mondini畸形、内耳道扩张及内耳道底骨质缺损有重要意义。这二种影像学检查结果可以相互补充诊断耳蜗纤维化与面神经裸露。对手术适应证的选择以及保证手术正常进行有重要意义     

乙状窦前移小儿人工耳蜗手术方法的探讨

目的:探讨乙状窦前移小儿人工耳蜗植入术式的选择和术中处理的方法,为特殊情况下的人工耳蜗植入手术的顺利完成提供经验。方法:538例行人工耳蜗植入手术的听力障碍患儿,术前进行的常规听力学与影像学检查,术前通过高分辨率CT判断乙状窦是否前移,行人工耳蜗植入手术中证实分为显著前移和轻度前移。结果:538例通过常规术式进行人工耳蜗植入患儿中,乙状窦轻度前移64例,占11.9%,对常规人工耳蜗植入手术无影响。4例患儿乙状窦显著前移,对常规人工耳蜗植入手术带来困难,占0.74%,通过处理骨性外耳道后壁和切除砧骨顺利完成手术。结论:小儿人工耳蜗植入手术患儿中,乙状窦轻度前移者对手术无影响,显著前移者经过适当手术处理,亦能成功进行手术。     

内耳畸形患儿的人工耳蜗植入

目的：探讨几种内耳畸形患儿的人工耳蜗植入效果。方法：对10例内耳结构异常的感音性耳聋患儿进行了人工耳蜗植入。结果：术后随访0．5～4．0年，10例基本达到了较满意的听觉言语恢复效果。结论：内耳畸形息儿行人工耳蜗植入，应严格进行术前听力学与影像学的评估，严格掌握手术的适应证，可以达到较满意效果。     

脑白质异常聋儿人工耳蜗植入后效果初步分析

目的探讨对脑白质异常的重度感音神经性聋患儿,人工耳蜗植入手术的可行性及听力言语康复效果分析。方法自2012年1月至2014年12月行人工耳蜗植入术的120例(120耳)重度感音神经性聋患儿中,有脑白质异常的患儿15例。随机抽取15例脑白质无异常的聋儿作为对照组,比较两组患儿植入人工耳蜗后手术效果、开机调试结果及听力言语康复效果。结果 130例(30耳)电极全部植入耳蜗内;2所有患者术后无脑膜炎、面瘫、脑脊液漏等并发症;3所有脑白质异常患儿在人工耳蜗植入术后都有听觉;4脑白质异常儿童术后平均听阈与对照组无明显区别;5随访6至24月后,脑白质异常患儿的听觉行为分级标准(categories of auditory performance,CAP)和言语可懂度分级标准(speech intelligibility rating,SIR)与对照组比较,差异均无显著性意义(P>0.05)。结论经过严格术前评估后,人工耳蜗植入可应用于脑白质异常伴重度感音神经性聋的患儿,其术后效果与脑白质无异常的聋儿植入效果基本一致,术后的听力言语康复效果无明显差异。     

伴有分泌性中耳炎的人工耳蜗植入适应证与手术时机

目的 探讨伴有分泌性中耳炎的人工耳蜗植入手术适应证与手术时机.方法 对26例伴有分泌性中耳炎的重度或极莆度耳聋患儿行人工耳蜗植入手术,术前对分泌性中耳炎进行必要的非手术治疗,选择手术适应证和手术时机.人工耳蜗植入术中清理分泌性中耳炎病变、通畅引流,术后定期随访,同步进行人工耳蜗植入效果的评估.结果 26例伴有分泌性中耳炎的人工耳蜗植入者均进行单侧耳手术,手术一次成功,其中左耳5例,右耳21例.术后1个月开机调试,26例均有主观电听觉.术后随访13个月～4.3年,分泌性中耳炎痊愈19例,鼓膜形态正常、声导抗检查为A型鼓室压曲线;分泌性中耳炎好转7例,鼓膜轻度浑浊内陷,声导抗吃不开C型鼓室压曲线;无鼓膜穿孔、中耳粘连或分泌性中耳炎急性发作病例.结论 伴有分泌性中耳炎的人工耳蜗植入手术在严格掌握手术适应证的前提下,应及早手术,以争取术后听觉言语康复的最佳时期;通过术前必要的药物治疗、术中的病变处理与术后的治疗以及密切随访,都可以避免并发症的发生,达到人工耳蜗植入的预期效果.     

共同腔畸形人工耳蜗手术适应证及手术方法的探讨

目的：探讨共同腔畸形人工耳蜗手术适应证以及人工耳蜗电极植入人路的选择。方法：在对重度或全聋患者进行人工耳蜗植入术前影像掌检查中,发现了6例耳蜗、前庭、外半规管呈共同腔畸形,其中5例有残留听力,1例未查到残留听力。结果：6例影像学检查呈共同腔畸形患者中,对5例有残留听力患者进行了人工耳蜗植入,其中3例选择了常规入路植入电极,2例选择了经乳突侧入路植入电极,术后均建立了人工耳蜗的听觉反应。1例因未查到残留听力,放弃了人工耳蜗手术治疗。结论：有残留听力的共同腔畸形患者,如果能够接受术后听觉言语识别效果差的事实,可以进行人工耳蜗手术。无残留听力或无法了解到有听觉反应的共同腔畸形患者,在现有技术条件下应放弃人工耳蜗植入手术。     

高分辨CT和MRI对人工耳蜗植入患者的术前评价

人工耳蜗植入术前影像学检查最佳方案迄今尚未确定，术前常用高分辨CT和MRI了解耳蜗的解剖结构、帮助选择手术入路、确定耳蜗植入侧。然而，究竟术前选择哪种检查方法可提供更多帮助仍有分歧，或两种方法联合效果更好?回顾分析爱尔兰国立人工耳蜗植入中心早期手术的88位患，     

Advantages of magnetic resonance imaging over computed tomography in preoperative evaluation of pediatric cochlear implant candidates.

OBJECTIVES: To compare magnetic resonance imaging (MRI) to high-resolution computed tomography (HRCT) in the preoperative evaluation of pediatric cochlear implant candidates. METHODS: The charts of pediatric cochlear implant candidates evaluated between July 1, 2000 and November 30, 2003 with an MRI scan of the inner ear were included in the study. Fifty-six patients were included. Associated HRCT scans were examined. Abnormalities of the cochlea, cochlear nerve, endolymphatic sac, endolymphatic duct, vestibule, and modiolus were noted. A pediatric neuroradiologist gave an opinion as to whether patients with anomalies seen with MRI but without associated HRCT would have been identified by HRCT. RESULTS: Of the 112 temporal bones imaged with MRI, the following abnormalities were encountered: 32% (36/112) had abnormalities of the cochlear turns, 30% (34/112) had abnormal signal in the modiolus, 23% (26/112) had abnormal vestibulae, 16% (18/112) had abnormal endolymphatic ducts, 15% (17/112) had abnormal endolymphatic sacs, 12% (13/112) had abnormalities of the cochlear nerves, 29% (17/56) had abnormalities of the brain. HRCT cannot directly evaluate the cochlear nerve. Available HRCT findings were combined with radiologic opinion and compared with MRI findings. The percentages of abnormalities identifiable by HRCT when compared with those seen with MRI are cochlea 42% (15/36), modiolus 35% (12/34), vestibulae 88% (23/26), endolymphatic duct 100% (18/18), and endolymphatic sac 6% (1/17). CONCLUSION: MRI is more sensitive and specific in diagnosing soft tissue abnormalities in the inner ear than HRCT in cochlear implant candidates (Fig. 4). Moreover, the abnormalities detected with MRI are more likely to influence the implantation process (e.g., asymmetric nerve aplasia, cochlear obstruction). (Figure is included in full-text article).     

合并脑白质异常聋儿人工耳蜗植入后听力言语及认知能力评估分析

目的 探讨合并脑白质异常聋儿行人工耳蜗植入手术的可行性及术后听觉、言语康复和认知发展情况。 方法 以2012年11月至2015年4月于南京鼓楼医院耳鼻咽喉头颈外科行人工耳蜗植入术的聋儿425例(425耳)中合并脑白质异常的6例患儿为实验组,选取同时期手术的6例脑白质正常聋儿为对照组。比较实验组手术前后言语能力,以及两组患儿术后听力、言语康复效果和认知发展水平。 结果 (1) 实验组和对照组手术前后的有意义听觉整合量表(MAIS)、听觉行为分级标准(CAP)和言语可懂度分级标准(SIR)差异有统计学意义(实验组:术后6个月,Z_CAP=-3.127, P_CAP=0.002; Z_SIR =-3.146, P_SIR=0.002; Z_MAIS=-2.898, P_MAIS=0.004;术后12个月,Z_CAP=-3.317, P_CAP=0.001, Z_SIR=-3.108, P_SIR=0.002, Z_MAIS=-2.656, P_MAIS=0.008。对照组:术后6个月,Z_CAP=-3.108, P_CAP=0.002; Z_SIR=-3.127, P_SIR=0.002; Z_MAIS=-2.918, P_MAIS=0.004;术后12个月,Z_CAP=-3.146, P_CAP=0.002, Z_SIR=-3.207, P_SIR=0.001, Z_MAIS=-2.913, P_MAIS=0.004); (2) 手术前后,实验组平均听阈与对照组相比,差异无统计学意义(F=0.797,P=0.393); (3) 实验组MAIS、CAP和SIR与对照组相比,术前差异均无统计学意义(Z_CAP=0.000, P_CAP=1.000; Z_SIR=0.000, P_SIR=1.000; Z_MAIS=-0.756, P_MAIS=0.450);开机后6个月差异均无统计学意义(Z_CAP=-1.673, P_CAP=0.094; Z_SIR=-1.369, P_SIR=0.171; Z_MAIS=-0.978, P_MAIS=0.32);开机后12个月,差异均有统计学意义(Z_CAP=-2.667, P_CAP=0.008; Z_SIR=-2.272, P_SIR=0.023; Z_MAIS=-2.656, P_MAIS=0.008); (4) 开机后12个月,实验组的韦氏幼儿智力量表(C-WYCSI)评分与对照组比较,总分差异有统计学意义(P<0.05)。 结论 合并脑白质异常聋儿接受人工耳蜗手术后言语能力较术前显著提高,且其术后平均听阈与脑白质正常聋儿相比差异无统计学意义。因此在进行严格术前筛选后,合并脑白质异常的聋儿可以通过人工耳蜗植入手术获益,手术是可行的。但由于合并脑白质异常聋儿术后12个月的言语康复效果及认知发展水平与脑白质正常聋儿差异有统计学意义,因此远期随访结果仍待进一步研究,且术前需与家长充分沟通。     

High resolution computed tomography and magnetic resonance imaging in the pre-operative assessment of cochlear implant patients

Optimal imaging protocols for cochlear implantation have yet to be determined. Pre-operative computed tomography (CT) and magnetic resonance image (MRI) scans are used to assess cochlear anatomy and patency, to delineate surgical access, and to aid in choice of side for implantation. However, opinion still differs as to which modality provides more information in pre-operative assessment, or if, indeed, a combination of the two is superior. The first 88 patients on the Irish National Cochlear Implant Programme (NCIP) were retrospectively studied to determine the accuracy of pre-operative CT and MRI in predicting abnormalities at the time of surgery. Correlation with surgical findings was determined in three separate groups of patients (those who had CT only, those who had MRI only, and those who had both CT and MRI performed). Of the 24 patients that had both CT and MRI performed, both modalities had a 79 per cent correlation with surgical findings. CT and MRI reports concurred in 75 per cent of cases. Specificity and negative predictive value were high (86 per cent and 90 per cent, respectively). CT alone (47 cases) correlated with surgery in 39 cases (83 per cent); MRI alone (17 cases) correlated in 15 cases (88 per cent). The findings of this study suggest that CT and MRI are effective at predicting normal inner ear anatomy, and thus at predicting the patient and the cochlea most suitable for implantation. Both modalities are useful in determining the side of implantation, thus avoiding potential surgical difficulties in cases of unilateral abnormalities. There was no significant difference between the ability of MRI and CT to detect abnormalities at the time of surgery. In this series the combination of CT and MRI has not been shown to be superior to either modality used alone, although anecdotal evidence to the contrary was noted.     

Cochlear implantations in children with Waardenburg syndrome: an electrophysiological and psychophysical review

Abstract

Waardenburg syndrome presents with dystopia canthorum, pigmentary abnormalities of hair, iris and skin (often a white forelock and heterochromia iridis) and sensorineural deafness. The authors review the electrophysiological and psychophysical findings of implanted children with Waardenburg syndrome at the Sydney Cochlear Implant Centre. Twenty children with Waardenburg syndrome received cochlear implants between 1985 and 2001. Electrical auditory brainstem response (EABR) was performed in all of these patients intra-operatively as part of the routine investigations. Only 13 of these patients were assessed one year or more post-operatively by means of the Melbourne Categories (0–7). Four patients (20%) were found to have abnormal EABR recordings. The mode of Melbourne Categories in this group (n = 3) was 1 at one year post-operation. The other 16 patients were found to have normal EABR and the mode of Melbourne Categories in this group (n = 10) was 7. A poor outcome after cochlear implantation was associated with abnormal EABR recordings (a ‘true’ auditory neuropathy) and was found in a significant proportion of patients with Waardenburg syndrome. Copyright © 2006 John Wiley & Sons, Ltd.     

The Clinical Significance of Preoperative Brain Magnetic Resonance Imaging in Pediatric Cochlear Implant Recipients

Although central nervous system abnormalities are incidentally detected in preoperative brain magnetic resonance imaging (MRI) studies in pediatric cochlear implant (CI) candidates, the clinical significance of the abnormalities remains unclear. We aimed to assess post-implantation auditory and speech performance in patients with brain lesions seen on MRI. Pediatric CI recipients (n = 177) who underwent preoperative MRI scans of the brain between January 2002 and June 2009 were included in this study. Patients with brain lesions on MRI were reviewed and categorized into the following groups: brain parenchymal lesions (focal vs. diffuse), ventriculomegaly, and extra-axial lesion. The main communication mode as well as progress in auditory perception and speech production were evaluated preoperatively and at 3, 6, 12, and 24 months postoperatively. Performance in patients with brain lesions was compared with the age- and sex-matched control group. Various brain lesions were found in 27 out of 177 patients. Children with brain lesions who received CIs showed gradual progress in auditory and speech outcomes for 2 years, though performance was reduced compared with the control group. In addition, there was a significant difference in the main communication mode between the two groups at 2 years following cochlear implantation. This difference was especially significant in patients with diffuse brain parenchymal lesions after further stratification of the brain lesion group. Preoperative brain MRI may have a role in improving the prediction of adverse outcomes in pediatric CI recipients. In particular, children with diffuse brain parenchymal lesions should be counseled regarding the poor prognosis preoperatively, and followed up with special attention.     

Surgical outcome after paediatric cochlear implantation: diminution of complications with the evolution of new surgical techniques

OBJECTIVE: Cochlear implantation is a commonly performed surgical procedure with specific aspects in the paediatric population. The surgical outcome associated with this procedure in a paediatric population is analyzed. METHOD: A retrospective study was performed of all children receiving a cochlear implant at The Hospital for Sick Children from 1990 to 1998. During this period, 104 patients received a cochlear implant. RESULTS: The complications encountered were classified as major (4%), minor (3%), and device failure (2%). The surgical techniques developed to decrease complications are discussed. CONCLUSION: Paediatric cochlear implantation is a safe procedure with a low incidence of complications. Nevertheless, the surgeon is now facing new challenges with cochlear implantation performed in patients with an abnormal cochlea, who carry a higher rate of complications, and cochlear reimplantation in case of device failure.     

Temporal bone imaging for cochlear implantation

Computerized tomography (CT) imaging of the inner ear structures is helpful when evaluating a patient for cochlear implantation. The accuracy of CT scan imaging in determining the presence of cochlear ossification was assessed. A retrospective study of 104 implant patient charts, operative reports, and CT scans was performed. The CT scan was in agreement with the operative findings in 78% of patients included in the study. Twenty-two percent were found to have ossification at surgery which was not detected radiographically. One patient with extensive otospongiosis and an obstructed cochlea on CT scan had a patent cochlea demonstrated by magnetic resonance imaging (MRI) which was corroborated at surgery. In 32 children with postmeningitic hearing loss, the CT scan had only a 53% accuracy in assessing cochlear ossification. Since 69% of these patients were found to have some degree of cochlear ossification, otologists should expect to encounter some degree of bony obstruction within the basal turn of the cochlea even when the CT scan is normal. Nevertheless, cochlear implantation can successfully be performed in these cases.     

一例脑白质听障儿童植入人工耳蜗后的个别化教育报告

目的通过个案研究探讨脑白质听障儿童植入人工耳蜗后的全面康复教育手段及其听觉言语发展情况。方法运用观察法和测试法,对不同阶段个别化教育方式及个案的发展情况进行分析。结果经过2年的个别化教育康复,个案的学习能力、听觉能力和语言水平均有所提高．但言语清晰度进步较慢。结论个别化教育可以为脑白质听障儿童植入人工耳蜗后的康复教育提供一系列特殊支持服务,有助于其全面康复发展。     

Cochlear implantation in children with CHARGE association

OBJECTIVE: To explore the anomalies of the temporal bone found on radiologic examination, technical challenges in cochlear implantation, and audiologic benefit derived from cochlear implantation in a series of children with CHARGE association. DESIGN: Case series report. SETTING: Tertiary referral children's hospital pediatric cochlear implant program. PATIENTS: Six children with CHARGE association and sensorineural hearing loss. INTERVENTION: All patients were evaluated and followed up by the cochlear implant team. Cochlear implantation was attempted in all 6 children. MAIN OUTCOME MEASURES: Computed tomographic scans and cochlear implantation operative records were reviewed, and their findings were correlated. Audiometric and speech perception data before and after cochlear implantation were compared. RESULTS: Five children with CHARGE association received implants. A sixth child did not because of an aberrant course to the facial nerve. The 5 children receiving implants obtained varying degrees of measurable benefit from their implants. All 6 children had temporal bone abnormalities seen on their computed tomographic scans and documented at the time of surgery. CONCLUSIONS: Variations in the temporal bone anatomy of patients with CHARGE association can lead to increased technical challenges and risk to the facial nerve during cochlear implantation. Individual outcomes after implantation may vary; our patients receiving implants obtained benefit. Parents should be counseled thoroughly and have appropriate expectations before proceeding with implantation.