Outcomes of cochlear implantation in children with inner ear malformations

European Archives of Oto-Rhino-Laryngology - To evaluate the surgical experience and auditory functions and progress of speech development of cochlear implantation in malformed ears. Between...     

内耳发育畸形患者人工耳蜗植入术后的疗效分析

目的分析内耳发育畸形患者行人工耳蜗植入术后的疗效。方法回顾性分析2011年1月~2017年7月在西南医科大学附属医院行人工耳蜗植入术的30例内耳发育畸形患者的临床资料,其中大前庭水管综合征(large vestibular aqueduct syndrome,LVAS) 8例,Mondini畸形（不完全分隔Ⅱ型）7例,同时伴有LVAS和Mondini畸形10例,内听道狭窄5例。随机选取同时期临床资料相匹配的30例耳蜗结构正常的人工耳蜗植入患者作为对照组。采用听觉行为分级标准和言语可懂度分级标准进行评估。比较两组患者的术前听力、术中情况以及术后听觉言语康复情况。结果3例LVAS患者在术中耳蜗钻孔后出现外淋巴液不同程度的外涌,1例Mondini畸形患者术中出现脑脊液井喷现象。两组患者术后均获得有意义的听觉反应,术后的听觉言语能力相比较,差异无统计学意义（P>0.05）。同组患者,术后3,6,9,12个月的听觉言语能力均较各自术前均有明显提高（P<0.05）。结论内耳发育畸形患者不是人工耳蜗植入术的绝对禁忌证,其术后言语康复效果与内耳结构正常者无明显差异,但手术难度比内耳结构正常者要大,术前应根据不同的畸形类型制定个性化的手术方案,以减少并发症的发生。     

Unilateral cochlear implantation in children with a potentially useable contralateral ear

Increasingly, children are considered for a unilateral CI, even if the contralateral ear falls outside current audiological guidelines, especially if they are not considered to be reaching their educational potential. The primary aim was to investigate the benefit of unilateral CI in children currently outside UK [National Institute for Health and Care Excellence Technology Appraisal Guidance. 2009. Cochlear implants for children and adults with severe to profound deafness. NICE technology appraisal guidance [TAG166]. Available January 29, 2016 from http://www.nice.org.uk/ta166] audiological guidelines in the contralateral ear. The secondary aim was to measure compliance. A retrospective case review with standard demographic data was performed. Forty-seven children were identified as having received a unilateral CI with the contralateral ear falling outside of current UK audiological criteria. These children were allocated to two groups; with hearing between 50 and 70?dB, and 70 and 90?dB at 2 and 4?kHz in the contralateral ear, respectively. Categories of auditory performance (CAP) were assessed. Pre- and post-operative CAP scores demonstrated a statistically significant improvement in auditory perception. We would suggest that assessing candidacy in individual ears and subsequent unilateral CI, has given these children a benefit they may not otherwise have acquired if they only had bilateral hearing aid.     

Preservation of basal inner ear structures in cochlear implantation

The aim of this report was to examine basal trauma in implanted human temporal bones and discuss modified approaches to the basal cochlear turn to avoid destruction of basal cochlear structures. Thirty-three human temporal bones were implanted with four different cochlear implant electrode arrays manufactured by MED-EL using either a caudal approach cochleostomy or round window membrane insertions. All specimens were processed with a special histological technique that allows sectioning of undecalcified bone with the electrode in situ. All bones were evaluated histologically in terms of basal cochlear trauma. Two pathomechanisms of basal trauma could be distinguished and were evaluated separately, buckling of the basal end of the array and trauma by drilling. Using the caudal approach cochleostomy, the total percentage of destructive basal trauma was 48% compared to less than 15% when performing round window membrane insertions. Although it is still unclear whether basal cochlear trauma influences apical cochlear function or not, adapted surgical procedures and no forceful insertion maneuvers should be used when performing cochlear implantations with hearing preservation.     

内耳畸形人工耳蜗植入后听力言语康复效果分析

目的探讨内耳畸形患者人工耳蜗植入术,并对术后听力言语康复效果进行评估.方法 1997年至今,北京同仁医院共开展人工耳蜗植入术700余例,其中双侧内耳畸形患者108例,随访并回顾性分析97例,就不同原因内耳畸形的人工耳蜗植入术后开机调试及术后听力言语康复效果进行评估.结果①所有内耳畸形患者人工耳蜗植入术后都有听觉;②术后经正规康复训练,患者听力言语康复效果均有不同程度提高;③目前大部分患者于正常学校就读或已经工作,重返主流社会.结论对内耳畸形患者行人工耳蜗植入术是安全可靠的,人工耳蜗植入术后远期康复效果肯定.     

内耳结构异常患者人工耳蜗植入术的疗效观察

目的分析研究内耳结构异常患者人工耳蜗植入术的安全性及手术后的疗效情况。方法选取2011.07-2015.06在我院住院接受人工耳蜗植入的17例内耳结构异常患者的临床资料,其中语前聋14例,语后聋3例,针对内耳结构异常情况采用不同术式。用听觉分级行为标准(Categorise of Auditory Performance,CAP)、言语可懂度分级标准(Speech Intelligibility Rating,SIR)进行术后效果评估。选取24例同期接受人工耳蜗植入的内耳正常的语前聋患者作为对照组,分析并比较两组患者手术并发症及术后疗效。结果:17例患者手术顺利,术后出现1例眩晕,所有患者术后均无脑脊液耳漏、面瘫等并发症。内耳结构异常语前聋患者术后CAP和SIR值分别为5.8±1.4、3.5±1.1;对照组CAP和SIR值分别为6.9±1.3、3.8±0.9,两者差异无统计学意义(P>0.05)。结论内耳结构异常非人工耳蜗植入术的绝对禁忌证,根据异常程度制定个性化的手术方案,可以减少并发症的发生。内耳结构异常语前聋患者术后言语康复情况、对声音的自发性觉察能力与耳蜗形态正常患者术后无明显差异。     

Audiological performance after cochlear implantation in children with inner ear malformations

OBJECTIVES: To prove that cochlear implantation is a beneficial method of rehabilitation in deaf children with malformations of the inner ear. DESIGN: The evaluation of auditory responses to speech (EARS) test battery was performed on the children in this study after an average implant use of 3 years. RESULTS: Individual results of six children with inner ear anomalies receiving cochlear implants are presented in this study. Three of the patients showed an incomplete partition (Mondini dysplasia), one had a cochlear hypoplasia and two suffered from an intraoperative cerebrospinal fluid leak. The majority of the children in this study are successful implant users. Wherever possible, test scores are included and subjective case reports given. CONCLUSIONS: Results are similar to those in children with normal cochleas, therefore inner ear malformations found in as many as 20% of patients with congenital sensorineural hearing loss are no contraindication for cochlear implantation. Nevertheless, factors influencing the success of implantation are multiple, including a thorough preoperative radiological examination, a well-performed surgery and an individually tailored postoperative rehabilitation programme.     

先天性内耳畸形并中耳畸形患者多通道人工耳蜗植入术3例报告

目的：探讨先天性内耳畸形并中耳畸形患者行多通道人工耳蜗植入术的方法及效果。方法：1995年5月-2002年5月我院为3例罕见的先天性内耳畸形并中耳畸形患者经乳突进路行人工耳蜗植入术,分别植入27、28、32个电极,植入后3个月行声场测听。结果：3例患者均成功地行人工耳蜗植入,术中、术后无严重并发症发生,术后声场测听听阈达35-40dBHL。结论：罕见的先天性内耳畸形并中耳畸形患者也可行人工耳蜗植入术,术后效果满意。     

"井喷"在内耳畸形人工耳蜗植入术中的发生率

目的：探讨“井喷”在内耳畸形人工耳蜗植入术中的发生率，为有“井喷”现象的人工耳蜗植入术积累临床经验。方法：对680例行人工耳蜗植入术患者行术前高分辨CT检查，显示80例双耳有内耳畸形，其中20例并发内耳道底骨性缺损与内耳相通，行人工耳蜗植入术。结果：20例（25％）并发内耳道底骨性缺损与内耳相通者，术中发生了“井喷”，其中大前庭导水管畸形者5例，Mondini畸形者11例，耳蜗前庭与内耳道共同腔畸形者4例；耳蜗、前庭、外半规管共同腔和前庭、外半规管共同腔畸形者无“井喷”发生。结论：①80例内耳畸形人工耳蜗植入术患者“井喷”的发生率为25％；②内耳畸形人工耳蜗植入术中“井喷”的发生率依次为：耳蜗、前庭与内耳道共同腔畸形，Mondini畸形，大前庭导水管畸形；③单纯前庭、耳蜗、外半规管共同腔畸形及半规管畸形者，人工耳蜗植入术中未发生“井喷”；④80例内耳畸形人工耳蜗植入术中，发生“井喷”者均存在内耳道底骨缺损，与内耳形成共同开放的通道。     

Congenital malformation of the inner ear and pediatric cochlear implantation.

OBJECTIVES: To study the surgical aspects and performance outcome of cochlear implantation in children with malformed inner ears. STUDY DESIGN: Clinical and audiometric evaluation in 13 patients. METHODS: Patient data concerning surgery, postoperative follow-up, and pre- and postimplantation audiometry were obtained from the cochlear implant center's database and evaluated. A review of the literature has been included. SETTING: Tertiary referral center. PATIENTS: The patients had a variety of inner ear malformations and profound hearing loss. One patient with recurrent meningitis had a severe cochlear malformation (common cavity). RESULTS: Major complications did not occur. In one patient with an abnormal position of the cochlea and concurring middle ear disease, it was difficult to find the scala tympani during surgery. A cerebrospinal fluid gusher was encountered in two patients and an aberrant facial nerve in another, which did not lead to any complications. The patients with mild cochlear malformation such as an incomplete partition demonstrated a good performance in speech perception tests. Even the child with the common cavity deformity had some open-set speech perception 1 year after implantation. CONCLUSIONS: Viewing the patients from this study and patients from a review of the literature concerning cochlear implantation in children with malformed inner ears including severe cochlear malformations, the occurrence of an aberrant facial nerve was 17%, which increases to 27% if one reviews the surgical findings in children with severe malformed cochleae such as a common cavity or a severe cochlear hypoplasia. In the latter patients, results in speech perception vary. Although the result of cochlear implantation may be promising, as in our patient with a common cavity, during preoperative counseling the child's parents must be informed that the result is uncertain.     

Development of auditory skills after cochlear implantation in children with inner ear malformations

Conclusions: CI improves hearing thresholds and auditory skills in children with most types of inner ear malformations. However, the development of sound detection skills is not as good as it is in children without inner ear malformations. Objectives: To investigate the influence of inner ear malformations on development of auditory skills after cochlear implantation (CI). Methods: Records of 20 children with inner ear malformations who underwent cochlear implantation before 4 years of age and followed up for more than 2 years were retrospectively reviewed. Hearing thresholds, the Meaningful Auditory Integration Scale (MAIS), and Meaningful Use of Speech Scale (MUSS) scores before and after CI were analyzed and compared with 20 age-matched deaf children who underwent CI. Results: The children with inner ear malformations showed significant improvements in hearing thresholds and the MAIS and MUSS scores 1 year after CI (p?p?p?相似文献   

MRI内耳水成像在人工耳蜗植入电极的应用价值

目的 了解耳蜗的膜迷路结构。为人工耳蜗手术檀入电极做好术前准备。方法 分析11例双耳极重度感音神经性耳聋的MRI内耳水成像圈像。使用GE Signa Infinity TwinSpeed 1．5T扫描仪，采集内耳所在区域内水信号。送AW4．0工作站进行圈像的后处理。结果 双耳极重度感音神经性耳聋10例显示耳蜗的膜迷路正常，1例双侧耳蜗只显示一圈半，且耳蜗直径缩小。诊断为耳蜗骨化。结论 MRI内耳水成像能清晰显示耳蜗的膜迷路，为人工耳蜗植入电极提供保障。     

电诱发听性脑干反应和电诱发镫骨肌反射阈值在内耳畸形儿童人工耳蜗植入术后调机中的运用

目的　探讨内耳畸形小儿人工耳蜗植入手术后,植入体电诱发听性脑干反应（electrically evoked auditory brainstem responses,EABR）、电诱发镫骨肌反射阈值（electrically evoked stapedius reflex threshold,ESRT）的变化特点及规律,以指导术后设备调试。方法　将88例澳大利亚Cochlear Nucleus24型人工耳蜗植入手术患儿分为耳蜗形态正常组与内耳畸形组,测试手术后1年内不同时期EABR和ESRT值,术后1年运用行为测试法检测主观阈值（T值）和最大舒适阈（C值）,分析特点及变化规律。结果  内耳畸形组患儿术后不同时期EABR和ESRT阈值较正常组高（P＜0.05）,两组EABR和ESRT阈值变化趋势相同,总的趋势是低频值较低,高频值较高,术后1年EABR和ESRT阈值逐渐增高;两组EABR与T值显著相关,ESRT与C值显著相关。结论　内耳畸形组人工耳蜗植入手术后EABR和ESRT阈值变化规律及特点与正常组患儿相同,阈值可用于指导内耳畸形人工耳蜗植入者手术后设备的调试。     

Noise exposure of the inner ear during drilling a cochleostomy for cochlear implantation

OBJECTIVES: Inserting an electrode array into the cochlea may cause inner ear trauma, which has to be minimized, particularly in cochlear implant patients with substantial residual hearing. Another potential inner ear trauma has, to a large extent, been neglected so far: the acoustic trauma that can occur during cochleostomy using different techniques. In this study, the noise exposure of the inner ear during the drilling procedure was re-evaluated. In experiments on temporal bones, quantitative measurements of sound pressure level (SPL) were carried out while a cochleostomy for cochlear implantation was drilled. STUDY DESIGN: Experimental study. MATERIALS AND METHODS: Acoustic measurements during different drilling procedures were carried out on four human temporal bone preparations equipped with microphones attached to the round window. Special calibrations were carried out, which allowed determination of SPLs affecting the cochlea during the drilling procedure. RESULTS: The highest SPLs measured on the cochlea were recorded when a still-intact endosteal membrane was touched by the burr. The SPL exceeded 130 dB and reached a level almost comparable with the situation when the ossicular chain is touched by a running burr. CONCLUSIONS: In the drilling procedure for a cochleostomy, the inner ear may be affected by very high SPLs, particularly if the endosteal membrane is left intact and comes into contact with the running burr. Of course, the resulting SPLs depend on the drilling speed and the size and characteristics of the burr (larger burrs cause higher SPLs); however, we are of the opinion that the cochlear function is at risk, anyway, if special precaution is not exercised. Even when working with reduced drilling speed, the surgeon should be aware of the high risk in the form of an acoustic trauma, which may endanger residual hearing. Recommendations in terms of "soft surgery" are given in the paper (e.g., the use of microhooks instead of a drill to remove the very last shell of bone covering the cochlea).     

Degeneration of cochlear neurons as seen in the spiral ganglion of man

A light and electron microscopic study of the spiral ganglion cells of a normal individual, a patient with Meniere's syndrome, and a patient with long-standing unilateral profound deafness was done to evaluate patterns of neural degeneration in the human inner ear. Parametric data for the normal spiral ganglion are presented and compared with the pathologic ganglia. In the ear with Meniere's syndrome, the nuclear area and axonic diameter of spiral ganglion cells were significantly smaller than in the contralateral and the normal ear. This was interpreted as evidence of neuronal degeneration in Meniere's syndrome. In the spiral ganglion of the long-standing deaf ear, there appeared to be selective preservation of large cells with no dendritic processes, contrary to the pattern of degeneration seen in the spiral ganglion of the animal.     

Cochlear neuronal populations in developmental defects of the inner ear. Implications for cochlear implantation

A histological study was made to determine the cochlear neuronal populations of 20 human ears having hearing loss caused by developmental defects. The neuronal populations ranged from 7677 in an ear with Mondini dysplasia to 30 753 in an ear with DiGeorge's syndrome, the norm for young human subjects being 35 000 neurons. The length of the cochlear (spiral) ganglion varied from 7.3 mm to 14.8 mm, the norm for human subjects being 12 mm. The sensorineural hearing losses in all cases were attributable to malformation or degeneration of the sensorineural structures. The hearing loss was moderate in one case of Alport's syndrome, severe in one case of Usher's syndrome and in one case of severe Mondini dysplasia; it was profound in one case of maternal rubella, one of congenital deafness of unknown cause, another case of severe Mondini dysplasia and one of Down's syndrome (Trisomy 21). One case of mild Mondini dysplasia and one of DiGeorge's syndrome were known to have normal hearing. In 4 other cases hearing status was absent.     

Improved speech and language development after unilateral cochlear implantation in children with a potentially useable contralateral ear

Objectives: Increasingly, children are considered for a unilateral cochlear implant (CI), even if the contralateral ear falls outside current audiological guidelines, especially if they are not considered to be reaching their educational potential. Here we present the outcomes of CI in children with potentially useable hearing in the contralateral ear.

Methods: A retrospective case note review was performed for a total of 57 patients. Primary outcome was speech and language (SaL) development, as measured by the Manchester Speech and Language Development Scale (MSLDS) and SaL age equivalent. Secondary outcomes were auditory perception, perceived parental benefit and compliance; respectively measured by Categories of Auditory Performance (CAP), Brief Assessment of Parental Perception (BAPP) and reported use.

Results: SaL development improved after CI with a mean pre-operative MSLDS score of 5.8 to a postoperative score of 8.0 (n?=?57) and a mean SaL age equivalent of 14 months in a one-year period (n?=?14). Furthermore, CAP scores improved from 4.9 to 7.0 (n?=?57). Analysis of BAPP scores showed improved quality of life in 18/19 patients (94.7%). With regards to compliance, 50/57 (87.7%) are fulltime users of both their CI and their HA.

Conclusion: The present study indicates that despite one ear having potentially useable hearing outside national audiological criteria, the majority of participants received benefit from a CI in the poorer hearing ear. We suggest that assessment of each ear separately and treatment with the most appropriate amplification device, has given these children a benefit they may not otherwise have acquired if they only had bilateral HA.