小儿电子耳蜗植入后的听功能变化

目的:探讨小儿电子耳蜗植入后不同时期的听功能变化。方法:将接受电子耳蜗植入的47例患儿分为NucleusN24和ClarionS两组,使用Nottingham大学的听觉活用进步教育听力进程评价体系(Nottingham大学LIP)观察患儿术后不同时期的听功能变化。结果:术前及开机后3、6、12个月的Nottingham大学LIP平均得分,NucleusN24组分别为(8.5±9.9)、(35.0±8.6)、(39.0±2.4)、(40.0±2.9)分,ClarionS组分别为(5.6±6.5)、(28.1±13.4)、(34.4±9.2)、(39.8±3.5)分。开机后的最初3个月听功能提高显著,3～12个月提高缓慢。开机后1年两组的听功能测试均接近满分。结论:两种电子耳蜗植入后听功能均可获得不同程度的提高,且两者无明显差异。     

Knowledge about cochlear implantation: A parental perspective

Objectives: Cochlear implantation (CI) is used for children with severe to profound hearing loss who show little or no improvement using hearing aids. This study explored parental knowledge of their children’s CI.

Methods: A cross-sectional study involving the parents of 115 pediatric CI patients was conducted at King Abdullah Specialized Children’s Hospital in Riyadh, Saudi Arabia. Parents were interviewed by telephone using a 50-question validated questionnaire.

Results: Most parents of children with CI reported being comfortable in using the internet (68.7%) and social media (40.9%) to obtain information regarding CI. Although most parents of children with CI relied on health professionals and websites as their main sources of information, they were also able to obtain necessary information at meetings for CI patients and health professionals. Parents of children with CI felt they had sufficient information regarding the impact of hearing loss (78%) and CI (71%) on speech understanding and language development; however, they had insufficient information regarding criteria for CI candidacy, available brands of CI devices, and the advantages and disadvantages of each.

Conclusion: Parents reported that health professionals were the ideal source of information regarding hearing loss and CI. Moreover, our study showed that parents should learn more about cochlear implant devices, the post-implantation process, and candidacy criteria.     

Compliance with cochlear implantation in children subsequently diagnosed with autism spectrum disorder

Objective: To assess the compliance with cochlear implantation (CI) in children subsequently diagnosed with autism spectrum disorder (ASD).

Methods: This was a retrospective case review and survey performed at a tertiary referral centre. Children meeting the criteria for CI who were implanted between 1989 and 2015 and who subsequently received a diagnosis of ASD were included. The primary outcome measure was to assess compliance with CI in children subsequently diagnosed with ASD. Secondary outcome measures included assessment of pre-CI risk factors that may have identified children at higher risk of a subsequent diagnosis of ASD, as well as the benefit obtained by these children following CI.

Results: 1050 children were implanted between 1989 and 2015. Of these, 22 children were diagnosed with ASD after receiving their CI. The average age at implantation was 2.6 years (median 3, range 1–8 years). The average age for diagnosis of ASD was 5 years, approximately 2 years (median 22 months, range 2–85 months) following CI. Of these, 16/22 (712.7%) regularly use their CI. 6/22 (27.2%) children became non-users of their implant. Some degree of verbal communication was used by 13/22 (59%) of our studied group.

Conclusion: There is a range of level of disabilities in ASD, with some relatively minor social communication difficulties through to severe language, cognitive, and behavioural difficulties. Compliance with CI is variable and appears to correlate with the severity of the ASD. Preoperative counselling should include information about the possible impact of later diagnosed disabilities such as ASD on performance.     

Balance in children following cochlear implantation

Objectives: To assess the vestibular function of children who had unilateral and bilateral cochlear implants compared with a control group of otherwise healthy children who had not been implanted.

Study design: Observational case–control study.

Methods: Posturography was carried out in the form of a Modified Clinical Test of Sensory Interaction on Balance using a Wii Balance Board and the Vestio App on an iPod Touch. Thirty children in total were tested, 10 children in each cohort. Results in the form of root mean square calculations were available for each child.

Results: Results showed a significant difference in the vestibular function of implanted children and the non-implanted control group (P?P?Conclusions: Our study demonstrates posturography as an inexpensive, easily operated tool that can be used to assess paediatric vestibular function. It showed a significant difference between the control group and the implanted groups. Further work prompted by this study will include interval post-operative testing to more accurately assess the effect that implantation has on vestibular function.     

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.     

Age at implantation and auditory memory in cochlear implanted children

Abstract

Early cochlear implantation, before the age of 3 years, provides the best outcome regarding listening, speech, cognition an memory due to maximal central nervous system plasticity. Intensive postoperative training improves not only auditory performance and language, but affects auditory memory as well. The aim of this study was to discover if the age at implantation affects auditory memory function in cochlear implanted children. A total of 50 cochlear implanted children aged 4 to 8 years were enrolled in this study: early implanted (1-3y) n = 27 and late implanted (4-6y) n = 23. Two types of memory tests were used: Immediate Verbal Memory Test and Forward and Backward Digit Span Test. Early implanted children performed better on both verbal and numeric tasks of auditory memory. The difference was statistically significant, especially on the complex tasks. Early cochlear implantation, before the age of 3 years, significantly improve auditory memory and contribute to better cognitive and education outcomes.     

Cochlear implantation in infants less than 12 months of age

OBJECTIVE: Infants are diagnosed with severe to profound hearing loss at an earlier age due to the advent of universal newborn hearing screening. This offers the opportunity to provide intervention in the form of cochlear implantation at an earlier age than was previously possible. The purpose of this investigation is to evaluate the risk of cochlear implant surgery in children less than 12 months of age. DESIGN: Retrospective review of children who underwent cochlear implantation before 12 months of age. SETTING: Patients were identified from a database of pediatric cochlear implant patients at a tertiary care center. All patients were diagnosed with severe to profound hearing loss by otoacoustic emission and auditory brainstem response. Follow-up ranged from 2 months to 5 years. RESULTS: Fourteen of 15 patients had full insertions of the electrode hardware. Less than full insertion and post-operative CSF otorrhea occurred in one patient with severe cochlear abnormalities. There were no other perioperative surgical complications. The average speech detection threshold was 27.6 dB (20-45 dB) at approximately 1-3 months post-stimulation and 25 dB (15-30 dB) at approximately 5-7 months. CONCLUSION: In our experience, we feel cochlear implantation is safe for infants as young as 6 months of age. The current standard at our institution is to implant by 7 months of age for prelingual deafness as opposed to waiting additional time until 12 months of age before the brain is presented with speech.     

Pneumolabyrinth,intracochlear and vestibular fluid loss after cochlear implantation

The present case was a 38-year-old male who presented with progressive hearing loss, resulting in profound bilateral hearing loss. He had a past history of childhood medulloblastoma, which was treated with posterior fossa craniotomy and radiotherapy. A ventriculoperitoneal (VP) shunt was put in place to manage the hydrocephalus. Cochlear implantation (CI) was carried out on his right ear by a standard procedure. At CI activation, the electric impedance of the electrode was very high, and computed tomography revealed that there was no area of liquid density, suggesting depletion of the perilymph in the cochlea and vestibule. Eight months later, the impedance improved gradually, and the cochlea was filled with perilymph. Consequently, one of the causes of the pneumolabyrinth in the present case was that a scarred stenotic cochlear canaliculus secondary to surgery or radiation therapy might have prevented the CSF from filling the scala. In addition, it is also possible that the VP shunt might have altered the CSF pressure, leading to depletion of the perilymph.     

Evidence-based guidelines for recommending cochlear implantation for young children: Audiological criteria and optimizing age at implantation

Abstract

Objective: Establish up-to-date evidence-based guidelines for recommending cochlear implantation for young children. Design: Speech perception results for early-implanted children were compared to children using traditional amplification. Equivalent pure-tone average (PTA) hearing loss for cochlear implant (CI) users was established. Language of early-implanted children was assessed over six years and compared to hearing peers. Study sample: Seventy-eight children using CIs and 62 children using traditional amplification with hearing losses ranging 25–120?dB HL PTA (speech perception study). Thirty-two children who received a CI before 2.5 years of age (language study). Results: Speech perception outcomes suggested that children with a PTA greater than 60?dB HL have a 75% chance of benefit over traditional amplification. More conservative criteria applied to the data suggested that children with PTA greater than 82?dB HL have a 95% chance of benefit. Children implanted under 2.5 years with no significant cognitive deficits made normal language progress but retained a delay approximately equal to their age at implantation. Conclusions: Hearing-impaired children under three years of age may benefit from cochlear implantation if their PTA exceeds 60?dB HL bilaterally. Implantation as young as possible should minimize any language delay resulting from an initial period of auditory deprivation.     

Parent versus child assessment of quality of life in children using cochlear implants

Objective

Children with hearing loss who use cochlear implants have lower quality of life (QoL) in social situations and lower self-esteem than hearing peers. The child's QoL has been assessed primarily by asking the parent rather than asking the child. This poses a problem because parents have difficulty judging less observable aspects like self-esteem and socio-emotional functioning, the domains most affected by hearing loss.

Methods

This case-control study evaluated QoL in 50 preschoolers using a cochlear implant and their parents with the Kiddy KINDL^®, an established QoL measure. Children's responses were compared to a hearing control group and correlated with demographic variables. We used a questionnaire for parents and a face-to-face interview with children. T-tests were used to compare (a) paired parent-child ratings and (b) children with cochlear implants versus normal hearing. Pearson rank correlations were used to compare QoL with demographic variables.

Results

Children using cochlear implants rated overall QoL significantly more positively than their parents (M_Difference = 4.22, p = .03). Child rating of QoL did not differ significantly by auditory status (cochlear implant (82.8) vs. hearing (80.8), p = .42). Overall QoL correlated inversely with cochlear implant experience and chronologic age, but did not correlate with implantation age.

Conclusions

Preschool children using cochlear implants can assess adequately their own QoL, but parents afford valuable complementary perspective on the child's socio-emotional and physical well-being. Preschool children using cochlear implants rate overall QoL measures similar to hearing peers. A constellation of QoL measures should be collected to yield a better understanding of general QoL as well as specific domains centered on hearing loss.     

Bilateral cochlear implantation in children with Noonan syndrome

Noonan syndrome is a mostly autosomal dominant inherited disorder, which can be accompanied by hearing disorders or deafness, coagulation disorders, combined heart defects and developmental disorders. We are reporting on two children with an established Noonan syndrome with a severe bilateral hearing loss of respectively 95 and 100 dB and proper findings in the CT/MRI of the petrous bone. After complete otologic and radiologic diagnostics, both children underwent bilateral cochlear implantation successfully. According to the authors’ knowledge, this is the first time that cochlear implant therapy is discussed in patients with Noonan syndrome.     

Cochlear implantation in children with cerebral palsy. A preliminary report

Objectives

The aim of this study is to assess the post-implantation speech perception and intelligibility of speech produced by five profoundly deaf children with cerebral palsy.

Methods

This study is derived by a review of a prospectively maintained data collection on all patients entering the cochlear implant program. Five children with cerebral palsy who underwent cochlear implantation participated in this study. Functional outcome was assessed using the Speech Perception Categories and the Speech Intelligibility Rating scale. The follow-up of the series ranged from 12 to 45 months.

Results

At the last follow-up, two children who were placed into speech perception category 1 (detection of a speech signal) preoperatively progressed to category 6 (open-set word recognition with familiar words) postoperatively. Two children moved from preoperative category 2 (pattern perception) to postoperative category 6. One child placed into category 0 (no detection of speech) preoperatively progressed to category 4 (word identification) postoperatively. Before implantation, three children had connected speech unintelligible, and two subjects had connected speech intelligible to a listener who concentrates and lip-reads. At the last follow-up, one child had connected speech unintelligible, two children had connected speech intelligible to a listener who concentrate and lip-reads, one child had connected speech intelligible to a listener who has little experience of a deaf person's speech, and one child had connected speech intelligible to all listeners.

Conclusions

Cochlear implantation allowed these patients to dramatically improve their quality of life, increasing their self-confidence, independence and social integration.     

Atypical failure after cochlear implantation in children

We report a case of intermittent dysfunction in a 10-years-old boy, implanted with MedEL^® cochlear implant. Few weeks after the surgery the boy described short and intermittent episodes of implant dysfunction with rapid return to a normal function. No evidence for any electric or neural dysfunction was found. After few weeks, a clinical link was discovered to episodes of sneeze or nose blowing. Clinical and surgical implications are discussed.     

人工耳蜗及相关技术的进展

人工耳蜗植入(cochlear implantation, CI)是重度-极重度感音神经性聋患者的听觉和言语康复的主要方法, 随着人们对听觉系统和中枢可塑性的深入研究,手术技术及辅助工具的开发,以及人工耳蜗植入相关电子科技和材料科学的发展,人工耳蜗及相关技术有了很大的进步。本文分析人工耳蜗植入体,尤其是电极设计和言语处理器的升级、人工耳蜗植入适应证的拓宽、术前评估手段的完善及精准微创人工耳蜗植入和残余听力保留的开展,并对人工耳蜗植入相关技术进行展望。     

Impact of cochlear implantation on peripheral vestibular function in adults

ObjectiveCochlear implantation may have a detrimental effect on vestibular function and residual hearing. Our goal was to investigate the impact of cochlear implantation on peripheral vestibular function and the symptomatology that ensues.Material and methodsA prospective observational study included all adults undergoing cochlear implantation by the same operator between July 2014 and December 2015, with pre- and postoperative (4 months) neurovestibular balance examination comprising a questionnaire and clinical tests [head impulse test (HIT), head-shaking test (HST), skull vibration test (SVT)] and instrumental tests [caloric test of the lateral semicircular canal and cervical vestibular-evoked myogenic potentials (cVEMP)].ResultsTwenty-two patients were included, with a mean age of 62 years and sex-ratio of 1.2. Before implantation, 50% of subjects (n = 11) reported at least one episode of vertigo associated with balance disorder during their life. After implantation, there were 11 cases of vertigo but only one patient described persistent discomfort related to vertigo 4 months after surgery. Patients with impaired vestibular function after 4 months, taking all symptoms together, were all aged more than 75 years. HIT was abnormal in 18% of cases before implantation and in 59% after (P = NS). HST showed nystagmus in one patient both before and after surgery. Only 18% of patients showed nystagmus induced by SVT before surgery, increasing to one-third after surgery (P = NS). Caloric test of the lateral canal showed hypofunction in 50% of cases before surgery, including 10% of cases with areflexia. This rate increased after surgery to 58%, with 18% areflexia (P = NS). cVEMPs were not detected in 68% of cases before implantation and this rate increased to 86% after surgery (P = NS). There were no significant associations (P > 0.05) between test results and symptoms.ConclusionsIn the medium term, although older subjects more frequently presented vestibular disorder, cochlear implantation induced little vertigo or balance disorder, sometimes even improving vestibular function. However, vestibular disorders were frequent preoperatively and increased postoperatively. We tested vestibular function on different stimulation frequencies and yet found no correlation between postoperative test results and postoperative vertigo.     

Tinnitus and cochlear implants

Objective

Several published studies seem to confirm the clinical observation that cochlear implants (CI) have a suppressive effect on tinnitus in most CI users. The aim of this paper is to present our personal experiences on tinnitus improvement after cochlear implantation.

Methods

Before surgery, 51 post-lingually profoundly deaf adults, who underwent cochlear implantation between 2005 and 2007, filled out a questionnaire reporting tinnitus characteristics and the Italian version of the Tinnitus Handicap Inventory (THI-I). Moreover, they were asked to scale tinnitus loudness and annoyance. Six months after implant activation, patients repeated the same evaluations. Scores were statistically analysed by means of a Wilcoxon signed ranks test on the hypothesis that a CI could change the tinnitus magnitude and/or its annoyance.

Results

In our series of 36 patients, 36.10% reported total loudness suppression and another 41.6% reported a reduction. Annoyance scores reduced in 75% of patients. THI reduced in 72.2% of patients.

Conclusion

Tinnitus reduction after CI may manifest as several mechanisms, such as habituation, acoustic masking, direct electrical nerve stimulation and cortical reorganization. Nevertheless, it is our opinion that data on CI benefits on tinnitus should be interpreted with caution and it seems reasonable to admit that further data is still necessary before considering CI as a treatment of tinnitus especially in unilateral deafness.     

17例人工耳蜗再植入原因分析

目的总结17例人工耳蜗患者耳蜗再植入的原因,探讨降低耳蜗再植入风险的方法,提高患者手术治疗满意度及术后听力言语康复水平。方法回顾性分析2012年11月—2018年7月解放军总医院海南医院17例采用面隐窝入路圆窗再植入人工耳蜗患者的临床资料,分析其再植入原因。结果17例患者均顺利完成耳蜗再植入手术,再植入术后随访观察患者满意度高,人工耳蜗助听听阈大幅改善,言语分辨能力明显提高,患者听力言语康复效果提升。17例人工耳蜗再植入原因分别为:植入体故障6例;植入体不工作2例;植入体受撞击后损坏2例;植入体接收刺激器移位1例; 植入体接收刺激器部位破裂1例;皮瓣感染3例,其中1例为右侧感染后原植入体同侧再植入,皮瓣感染切口无法愈合后右侧植入体取出后行左侧人工耳蜗植入;电极未完全植入1例;应患者要求取出旧植入体同侧再植入新型植入体1例。结论人工耳蜗再植入原因复杂多样,选择合适材料的植入体、避免剧烈撞击、执行严格的无菌操作以及进行准确的人工耳蜗植入术前评估是避免人工耳蜗再植入的关键。