人工耳蜗植入术残余听力保留现状和研究进展

随着手术方法和电极设计等技术的创新,人工耳蜗手术取得了一定的进步,使患者残余听力得到一定的保留。所有手术方法的共同目标即是：保护耳蜗内结构,保留残余听力,以提高听觉言语能力。人工耳蜗术后残余听力的丧失被认为是多种因素的结果,近年来,为获得更好的残余听力保留,人们在电极设计与选择、新型电极涂层、围手术期药物治疗、机器人辅助、图像和电生理引导等方面的研究有了新的突破,对于残余听力的保护有了很大程度的提高。本文将对人工耳蜗术后残余听力保护相关因素的进展作一综述。     

极重度感音神经性聋佩戴助听器与人工耳蜗植入患儿残余听力的比较分析

目的:通过回顾性比较分析极重度感音神经性聋患儿接受佩戴助听器和人工耳蜗植入手术2种不同干预方式人群的听力学评估参数,探讨极重度感音神经性聋患儿有效残余听力的临床判断方法.方法:选取听力学评估和言语康复训练资料完整的22例双耳极重度感音神经性聋患儿,其中10例佩戴助听器并接受超过3个月的言语康复训练,另外12例接受人工耳蜗植入手术,其中有10例年龄区间与佩戴助听器组一致.佩戴助听器组患儿,根据言语康复训练效果分为良好(7例)和较差(3例),分别统计其佩戴助听器前ASSR和听力言语康复训练期间裸耳纯音测听在500、1000、2000、4000 Hz 4个频点的听阈阈值情况.年龄与佩戴助听器组一致的10例接受人工耳蜗植入术患儿,根据术前有否佩戴助听器情况,分为曾佩戴助听器但听力言语康复效果差者(5例)和未接受佩戴助听器者(5例),分别统计其佩戴助听器前和(或)手术前的ASSR在500、1000、2000、4000 Hz 4个频点的听阈阈值情况.结果:①ASSR的阈值情况.佩戴助听器且言语康复训练效果良好的7例(14耳)患儿,平均每耳有2.71个频点可引出ASSR反应,所引出的ASSR平均阈值为(110.92±7.43 )dB HL;佩戴助听器言语康复训练效果较差的3例患儿,再加上曾佩戴助听器但听力言语康复效果差而接受人工耳蜗植入术的5例,共8例(16耳),平均每耳只有1.06个频点可引出ASSR反应,所引出的ASSR平均阈值为(110.88±8.52 )dB HL.②裸耳纯音测听情况.佩戴助听器且言语康复训练效果良好的7例(14耳)患儿,所测频点的平均听阈为(96.11±7.81) dB HL;其中,每耳平均有3个频率点的裸耳纯音测听阈值≤100 dB HL.而在效果差的3例(6耳)患儿中,所测频点的平均听阈为(112.19±5.15) dB HL,裸耳纯音测听阈值≤100 dB HL耳的数量为0.结论:500、1000、2000、4000 Hz 各频率引出ASSR的频点数量和裸耳纯音测听阈值≤100 dB HL的数量,是临床上判断极重度感音神经性聋患儿有否存在可利用助听残余听力的有效指标.     

微创人工耳蜗植入术后残余听力保留的效果观察

目的报道解放军总医院微创人工耳蜗术后残余听力保留情况的初步观察结果,探讨术后听力稳定性以及低频残余听力与言语功能的关系。方法解放军总医院2011年对29例有残余听力的患者行微创人工耳蜗植入术,并对其术后低频残余听力及听觉言语能力进行随访1-2年,探讨分析不同低频残余听力水平及其他因素对言语识别能力的影响。结果29例患者术后1周低频残余听力保留率100%,完全保留率72.4%（21/29）,部分保留率27.5%（8/29）。25例随访成功,平均随访时间为18.8个月,低频残余听力保留率100%,完全保留率72%（18/25）,部分保留率28%（7/25）。术后18个月pre-GLFH组言语识别率高于pre-PLFH组,post-GLFH组言语识别率高于post-PFLH组,但均无统计学意义（P＞0.05）。研究中术后言语识别率与植入前耳聋时间、植入年龄、性别等未见明显相关性,语前聋患者术后言语识别率高于语后聋患者（P＜0.05）。结论微创人工耳蜗植入术能很好的保留患者低频残余听力,保留率可达到100%;较好的术前低频残余听力可能预示较好的术后听觉言语功能;CI患者术后残余听力可较长时间保留,为术后获得较好的听觉言语功能提供基础;语后聋患者术后言语感知能力要优于语前聋患者,尤其是在困难聆听环境下。     

人工耳蜗植入后术耳残余听力跟踪分析

目的观察人工耳蜗植入后植入耳残余听力的变化。方法对人工耳蜗植入术前能较好配合行为测听的58例听障儿童,人工耳蜗植入后利用游戏测听或纯音测听评估其术耳残余听力,58例术后随访3个月,43例术后随访1年,17例术后随访2年。结果与术前0．25～4 kHz残余听力相比较,58例术耳术后3个月残余听力较术前明显下降（P＜0．05）,43例术后1年残余听力明显下降（P＜0．05）,但0．25～0．5 kHz残余听力较术后3个月提高,差异有统计学意义（P＜0．05）;17例术后2年0．25～0．5 kHz残余听力较术后3个月明显提高。结论人工耳蜗植入后可导致0．25～4 kHz各频率残余听力下降,但术后1、2年时0．25、0．5 kHz处的残余听力较术后3个月有所提高,1、2、4 kHz残余听力无明显改变。     

人工耳蜗植入术中保存残余听力的手术技巧

近些年来,随着声电联合刺激(Electric Acoustic Stimulatlion,EAS)技术的发展,学者们越来越重视保存人工耳蜗植入患者的残余听力.EAS是一种结合了助听器功能的新型人工耳蜗装置,它通过联合使用人工耳蜗与助听器来改善患者的听觉.中、高频重度听力损失,低频区有残余听力,配戴助听器效果不佳的患者,EAS植入后能够获得良好的听功能.EAS的作用原理是中、高频区借助人工耳蜗的电刺激产生听觉,而低频区的残余听力由助听器来放大,从而有效提高植入后耳聋患者的听力表现,尤其是声音的定位、音调的感知以及噪声环境下的言语识别率[1,2].     

人工耳蜗植入术后听力言语康复训练的重要性

目的：阐述小儿人工耳蜗植入术后听觉言语康复训练的重要性和原则。方法：术前、术后采用问卷及用“聋儿康复听力语言评估”进行评估。结果：术后及时进行科学的康复训练，其康复效果明显优于未及时进行训练者。结论：人工耳蜗植入手术使患儿重新获得听的感觉，但要取得好的康复效果，达到回归主流社会的目的，必须进行有效的听力言语康复训练。     

人工耳蜗植入及其电刺激对不同频率残余听力的影响

目的 从听力学的角度评价人工耳蜗植入的安全性，并进一步探讨人工耳蜗植入及其电刺激对患者不同频率残余听力的影响。方法 筛选出手术前后能够良好配合纯音听力检查、中耳内耳无明显异常、测听条件基本相同的单侧Nucleus人工耳蜗植入者31例，了解并分析其耳蜗植入前后不同频率残余听力的变化情况。结果 经人工耳蜗植入手术及一段时间的耳蜗电刺激后，部分患者植入耳的残余听力会有所保留（66.67%）；植入侧1kHz以下频率段的残余听力较手术前有显著差别，损失最重的是500Hz或1kHz，其次是250Hz；植入侧2、4kHz较手术前无显著差别；非植入侧听力较手术前无显著差别。结论 人工耳蜗植入及耳蜗电刺激对植入耳的残余听力并非完全破坏，对不同的频率会造成不同程度的损害，且1kHz以下频率受损较重。     

两种不同长度的人工耳蜗电极对不同频率残余听力的影响

目的探讨植入两种不同长度的人工耳蜗电极对患者不同频率残余听力的影响。方法以中耳、内耳无明显异常的15例单侧植入Nucleus 24型人工耳蜗(A组,电极长度17mm)患儿与15例单侧植入Medel C40+15型(B组,电极长度29.2mm)人工耳蜗患儿为对象,两组对象于手术前后分别进行听性稳态反应(ASSR)测试,比较不同长度的人工耳蜗电极植入对不同频率听力的影响。结果①人工耳蜗植入后一月开机经耳蜗电刺激后,有51.67%患者植入耳保留残余听力;②手术前0.5、1、2、4kHz ASSR反应阈A组分别为98.93±6.28、107.80±8.16、104.07±8.83及104.73±5.93dB HL,B组分别为99.67±4.20、110.87±8.17、109.93±7.51及106.93±4.35dB HL;手术后一月上述各频率ASSR反应阈A组分别为101.60±4.64、112.60±6.11、106.13±5.19及106.60±6.25dB HL;B组分别为101.40±3.96、116.33±2.58、113.13±3.84及108.46±3.76dB HL;③术后B组0.5、1kHz的残余听力损伤明显,2、4kHz处残余听力损伤程度与A组差异无统计学意义;④A组术后残余听力损失以1kHz最显著,B组术后残余听力损失以500Hz最显著。结论人工耳蜗植入及耳蜗电极电刺激对植入耳的残余听力并非完全破坏,电极长短对不同频率残余听力会造成不同程度的损害,植入电极越长,植入耳蜗的部位越深,对低频残余听力损伤越明显。     

小儿微创圆窗入路人工耳蜗植入术

目的 探讨小儿微创圆窗入路人工耳蜗植入的可行性及关键手术技巧.方法 回顾性分析2011年10月到2013年10月接受微创人工耳蜗植入术的56例患儿(年龄6个月～5岁8个月,平均14.9±3.5个月)的临床资料,其中54例行微创圆窗植入,描述术中的圆窗暴露方式并统计并发症;对于14例术前有一定残余听力的患儿(250、500、或1 000 Hz三个频率中至少一个频率的纯音听阈≥75 dB HL),记录术前及术后的250、500及1 000Hz的纯音测听或行为测听听阈图,术后听力保护程度以完全保护(听力损失≤10 dB)、中度损失(听力损失约11～20 dB)、重度损失(听力损失约21～40 dB)或无听力(听力损失＞40 dB或最大声刺激无反应)为标准,统计分析残余听力保存率.结果 96.4％(54/56)的小儿使用微创圆窗入路顺利全植入人工耳蜗电极,随访5～28个月,所有小儿植入装置工作良好,3例(3/54,5.56％)发生小的并发症:2例为术区皮瓣肿胀,一周后治愈,1例并发周围性面瘫,3月后痊愈;术后残余听力完全保护8例(57.14％,8/14),3例为中度听力损失(21.43％,3/14),术后较术前低频听力平均损失10.5 dB,中频听力平均损失12 dB.结论 微创圆窗入路人工耳蜗植入术安全有效,面隐窝开放技术有利于圆窗的暴露,有一定残余听力的儿童人工耳蜗植入后其残余听力可得到完全或部分保护.     

人工耳蜗植入者的听力康复及其评估

人工耳蜗植入是当今双侧重度和极重度感音神经性聋患者重获听力的最佳途径.通过耳蜗电极刺激螺旋神经节所产生的“声音”与自然声音有较大区别,其声音类似于电子琴或金属撞击声.耳蜗植入术后的康复工作在整个过程中占有不可忽视的地位.本文结合一例人工耳蜗植入病例谈谈言语康复的情况.1 资料和方法患者,女性,36岁.自幼双耳听力较差,其父亲和两个姐妹有不同程度的感音神经性聋.植入手术前2年因接受庆大霉素治疗而致双耳重度聋.助听器对她来说只有声感而无语感.患者讲话口齿虽较清楚,但语速较慢.平时借唇读及笔谈与他人交流.     

Cochlear implant in patients with residual hearing

Objective: The postoperative speech perception abilities of severely hearing-impaired patients with multi-channel cochlear implant were compared with preoperative speech perception performance with conventional hearing aids. Methods: Cochlear implantation was performed in six severely to profoundly hearing-impaired patients. They had unaided pure-tone thresholds of 70–100-dB HL and aided thresholds of 35–90-dB HL in the better ear, but were not able to perceive speech sounds well with hearing aids. Results: Postoperatively, all the patients had significantly improved speech perception performance, exceeded the average skills of profoundly deaf cochlear implant users, and were able to communicate without writing. Conclusion: These results imply that cochlear implant may be indicated for severely to profoundly deaf subjects, if they receive little or no benefit from conventional hearing aids.     

Cochlear implantation in patients with substantial residual hearing

OBJECTIVES: Cochlear implantation is an effective means for providing auditory rehabilitation in adult patients with severe to profound sensorineural hearing loss. It has been hypothesized that patients with substantial, preoperative residual hearing would be excellent cochlear implant candidates because of surviving neural populations and a lack of auditory deprivation. The purpose of this study is to describe the outcomes of patients with substantial residual hearing who have undergone cochlear implantation. STUDY DESIGN: Retrospective chart review of patients with substantial preoperative residual hearing who underwent cochlear implantation. METHODS: Chart reviews were completed for patients with substantial residual hearing who underwent cochlear implantation (City University of New York Sentence Test [CUNY] > 60%, Hearing in Noise Test sentences presented in quiet [HINTQ] > 50%, or Consonant-Nucleus-Consonant [CNC] > 20% in the ear to be implanted). Preoperative and postoperative measures of audiologic performance as well as complications were assessed. RESULTS: All 12 patients who met inclusion criteria ultimately surpassed their preoperative aided performance level after implantation and gained significant benefit from their cochlear implant. At 6 months postimplantation, mean CUNY, HINTQ, and CNC scores were 93%, 78%, and 48% in the implant ear alone, respectively. However, progress was slower than expected for many patients, and at least one patient took 1 year to surpass his preoperative performance level. There were no complications from surgery in this selected group of patients. CONCLUSIONS: Patients with some degree of residual hearing do benefit from cochlear implantation. However, there may be an initial decline in performance as compared with preoperative levels. This decline is overcome in time in this patient population. These patients need to be counseled accordingly.     

Cochlear implantation in children with cochlear nerve deficiency

Objective

The aim of this study was to report on auditory performance after cochlear implantation in children with cochlear nerve deficiency.

Methods

A retrospective case review was performed. Five patients with pre-lingual profound sensorineural hearing loss implanted in an ear with cochlear nerve deficiency participated in the study. Postoperative auditory and speech performance was assessed using warble tone average threshold with cochlear implant, speech perception categories, and speech intelligibility ratings. All patients underwent high resolution computed tomography and magnetic resonance imaging.

Results

According to Govaerts classification, three children had a type IIb and two a type IIa cochlear nerve deficiency. Preoperatively, four patients were placed into speech perception category 1 and one into category 2. All patients had an improvement in hearing threshold with the cochlear implant. Despite this, at the last follow-up (range 18–81 months, average 45 months), only one girl benefited from cochlear implantation; she moved from speech perception category 2 to 6 and developed spoken language. Another child developed closed set speech perception and had connected speech that was unintelligible. The other 3 children showed little benefit from the cochlear implant and obtained only an improved access to environmental sounds and improved lipreading skills. None of these 4 children developed a spoken language, but they were all full-time users of their implants.

Conclusions

The outcomes of cochlear implantation in these five children with cochlear nerve deficiency are extremely variable, ranging from sporadic cases in which open set speech perception and acquisition of a spoken language are achieved, to most cases in which only an improved access to environmental sound develops. Regardless of these limited outcomes, all patients in our series use their device on a daily basis and derive benefits in everyday life. In our opinion, cochlear implantation can be a viable option in children with cochlear nerve deficiency, but careful counseling to the family on possible restricted benefit is needed.     

Residual hearing in cochlear implant patients

Preservation of residual hearing should be a desirable outcome of implant surgery. Prevention of neural degeneration due to loss of residual hair cells, together with the continuous progress in cochlear implant technology should be able to preserve cochlear integrity as well as possible. The degree of hearing preservation may vary depending on surgical approach, maximum insertion depth and other factors not uniformly considered to date. The aim of this retrospective case controlled study is to evaluate residual hearing after cochlear implant surgery. In particular, we analyzed data obtained with use of two different kinds of electrode arrays, with and without rigid introductor (stylet). We report the results on 37 patients with measurable preoperative hearing thresholds, mean age of 28 years (5–70 years), having the following implants: seven Advanced Bionics^®, four Med-El^®, 24 Cochlear^®, two MXM^®; 19 of them were performed using the stylet and the other 18 without it. A minimally invasive surgical approach was performed with a short retroauricular incision and a 1.2 mm cochleostomy. A complete electrode array insertion was obtained in all patients. Responses to pure-tone stimuli were measured for each ear in pre-implantation conditions and 3–12 months after surgery. After implantation 14 patients (38%) showed no hearing threshold variation, 29 (78%) maintained an appreciable hearing threshold level in the implanted ear, 8 (22%) had a total loss of residual hearing. Median increases of threshold levels were, in all 37 studied patients, 5, 10, 10 and 5 dB HL, respectively, for 125, 250, 500 and 1 kHz. For the 18 patients having implants without the stylet median increases of threshold levels were 0, 10, 5 and 7 dB HL; in the stylet group, they were 10, 5, 5 and 10 dB HL. On a comparison between the stylet and the non-stylet group, no significant differences in mean hearing threshold worsening were found. Data seem to suggest that cochlear function is less sensitive to mechanical trauma during implant surgery than was thought. Besides, electrode array stiffness seems not to influence preservation of cochlear residual functional integrity. Finally, the authors hypothesize a direct spiral ganglion activation under strong mechanical stimulation.     

Cochlear implantation and residual hearing preservation long-term follow-up of the first consecutively operated patients using the round window approach in Uppsala,Sweden

Objective: We conducted a long-term follow-up study to investigate the time course of residual hearing in our first 21 consecutively operated cochlear implant (CI) patients using the round window (RW) approach . The study may provide additional information about the influence of a flexible lateral wall electrode array on cochlear function.

Methods: Data were available for long-term follow-up (>5 years) in 15 patients. Pure tone audiometry (PTA) was assessed at 0.125–8?kHz preoperatively, and at one, three and >5 years postoperatively. Insertion angle, number of electrodes inside the cochlea, user-time of the processor and stimulation strategy were documented.

Results: Twelve out of 15 patients had residual hearing after a follow-up period of five years (mean 86 months, range: 61–103 months). Four out of 15 patients had >75% complete hearing preservation (HP), 8 out of 15 had 25–75% partial HP and 3 out of 15 patients had complete loss of hearing. There was a high correlation between insertion angle and HP.

Conclusion: Long-term HP was possible in 12 out of 15 cases. Even patients with complete hearing loss at long-term follow-up showed high performance in speech understanding and were full-time users.     

Effects of loss of residual hearing on speech performance with the CI422 and the Hybrid-L electrode

Abstract

Objective

Preservation of residual low-frequency hearing has become a priority in cochlear implantation. The purpose of this study was to compare rates of hearing preservation and effects on performance of loss of low-frequency acoustic hearing with two different length electrodes.

Study design

Retrospective chart review.

Setting

Tertiary Care Hospital.

Patients

Twelve patients were implanted with the CI422 a slim-straight electrode; the second group consisted of 10 patients implanted with the Hybrid-L, a shorter hearing preservation electrode.

Main outcome measure

Audiometric thresholds and speech perception measures.

Results

At 1 year, 3/10 (30%) patients with the Hybrid-L and 7/12 (58%) patients with the CI422 lost residual acoustic hearing resulting in a profound hearing loss in the implanted ear. In comparing these patients in particular, mean CNC words in the implanted ear were 72% in the CI422 electrode group and 15% in the Hybrid-L electrode group at 1 year (P = 0.03). While hearing preservation rates with the Hybrid-L tended to be better, among recipients who lost residual hearing, speech perception was better in those with the longer CI422 electrode.

Conclusions

With emphasis on preservation of residual hearing, patients need to be counseled regarding possible outcomes and options should loss of residual hearing occur following implantation. While shorter electrodes may have better rates of hearing preservation, the patients with the longer straight electrode in our study had significantly better speech understanding following the loss of residual hearing.     

脑膜炎后耳蜗骨化患者人工耳蜗植入的临床分析

目的总结因脑膜炎而致严重感音神经性听力损失的人工耳蜗植入经验,探讨其诊疗策略。方法回顾性分析2010年9月—2020年9月于首都医科大学附属北京同仁医院耳鼻咽喉头颈外科因脑膜炎致重度及极重度感音神经性听力损失进行人工耳蜗植入的22例患者资料,其中成人13例,儿童9例。对其术前影像学、手术所见进行分析。结果颞骨HRCT检查中16例(72.7%)诊断耳蜗骨化;6例(27.3%)未诊断,但经手术探查证实存在耳蜗骨化。18例内耳MRI检查中,3例(16.7%)未发现耳蜗信号改变,而经手术探查存在耳蜗骨化。两者结合后检出率为90.9%(20/22),20例(90.9%)可见合并半规管等其他迷路病变。术中未见耳蜗骨化者1例(4.5%),圆窗骨化7例(31.8%),耳蜗底转骨化14例(63.6%)。电极完全植入者18例(81.8%),部分植入者4例（18.2%）。结论脑膜炎后耳蜗骨化可致严重感音神经性听力损失,人工耳蜗是理想的治疗策略,建议尽早植入。术前颞骨HRCT和MRI对确定是否适合耳蜗植入和术前计划至关重要,诊断存在假阴性,但联合检查可大大提高耳蜗骨化的术前诊断阳性率。     

Cochlear implantation in superficial siderosis

Superficial siderosis (SS) of the central nervous system has been thought to be a rare condition that generates progressive hearing loss, ataxia, pyramidal signs, and dementia. The main cause of hearing loss by SS is thought to be neuronal. Because there is no histopathologic report of the human temporal bone in SS, there is a debate about the possibility of cochlear involvement. We present a 25-year-old man who was investigated for bilateral progressive sensorineural hearing loss and vestibular failure after head trauma. On brain MRI, SS of the central nervous system was detected. Distortion product otoacoustic emission (DPOAE) and auditory brainstem response (ABR) showed no response on both sides. However, integrity of the eighth nerve was proved by the electrical ABR test on the right side and the patient benefited significantly from cochlear implantation. The sensorineural hearing loss in SS seems to be related to cochlear damage as well as neuronal damage. So, cochlear implantation would be a hearing rehabilitation modality for the sensorineural hearing loss caused by SS.