Cochlear implantation in children with congenital inner ear malformations

OBJECTIVE/HYPOTHESIS: To assess the audiologic and surgical outcomes for pediatric cochlear implant patients with inner ear malformations. STUDY DESIGN: Retrospective review of 315 pediatric cochlear implant cases from 1994 to 2002. METHODS: Twenty-eight pediatric cochlear implant patients with known inner ear malformations determined on high-resolution computed tomography (HRCT) of the temporal bone were the subjects of review. Results of HRCT findings, intraoperative findings, postoperative complications, and objective measures of both closed- and open-set testing of speech perception were analyzed. RESULTS: Patients with the constellation of an incompletely partitioned (IP) cochlea, enlarged vestibular aqueduct (EVA), and a dilated vestibule (i.e., Mondini's malformation) as well as those with an isolated EVA or partial semicircular canal aplasia have relatively good levels of speech perception. Patients with total semicircular canal aplasia, isolated IP, cochlear hypoplasia, or common cavity demonstrated lower levels of performance. Poor performance may be related to associated developmental delays rather than labyrinthine anatomy alone. Complications of surgery were relatively limited. CONCLUSIONS: Cochlear implantation can be successfully performed in children with inner ear malformations. These children and their parents can expect significant auditory benefits from this intervention. The various types of inner ear malformations may have quite different prognoses for good auditory performance.     

Cochlear implantation in children with auditory neuropathy spectrum disorders

Abstract

Aim

To present the outcomes of cochlear implantation (CI) in children with auditory neuropathy spectrum disorders (ANSD).

Materials and methods

The pre- and post-CI hearing outcomes in children with ANSD were retrospectively evaluated. Performance was assessed with categories of auditory performance (CAP) and the Manchester spoken language development scale (MSLDS).

Results

Full data were available in 27 implanted children with ANSD with average age at implantation 35.4 months (range 19–68 months). Nine children were implanted bilaterally, while 13 were bimodal. The pre-CI CAP and MSLDS scores were 2.5 (range 0–5) and 2.5 (range 0–6), while the post-CI scores 5.8 (range 2–9) and 7.7 (range 3–10), respectively.

Conclusions

Although the outcome of CI in children with ANSD might vary, it is favourable in most of the cases. CI seems a justified hearing rehabilitation option for children with ANSD and limited benefits from conventional hearing aids.     

Cochlear implants in children deafened by congenital cytomegalovirus and matched Connexin 26 peers

Objective

To compare the long-term speech perception and production outcomes after cochlear implantation (CI) in children deafened by congenital cytomegalovirus (cCMV) with a matched group of Cx26-CI children by controlling for chronological age and magnetic resonance imaging (MRI) findings.

Methods

Retrospective review of 12 cCMV-CI children and matched Cx26-CI children for speech perception and speech production outcomes.

Results

Two trends were seen in our data. First, cCMV-CI children with normal MRI scans perform equally or even slightly better on speech perception tests compared to their Cx26-CI peers during the first three years. The majority of cCMV-CI children with normal MRI scans (5 out of 7), suffered from a delayed-onset SNHL. Their mean age at first implantation (2y9m, range 15–82m) was higher compared to their matched Cx26 peers (9m, range 7–12m). Before being implanted, the majority of these delayed-onset hearing impaired children had benefited from a certain period of normal hearing (with or without amplification of a hearing aid). Possibly, this input might have led to an advantage the first three years after CI. Second, results between cCMV-CI children with and cCMV-CI children without MRI abnormalities and their matched Cx26-CI counterparts tentatively suggest that, over a 5-yr follow-up period, cCMV-CI children with abnormalities on MRI scans catch up for speech perception, but lag behind for speech production.

Conclusion

cCMV-CI children with normal MRI scans perform equally or even slightly better on speech perception tests compared to their Cx26-CI peers during the first three years, whereas results between cCMV-CI children with and cCMV-CI children without MRI abnormalities and their matched Cx26-CI counterparts tentatively suggest that, over a 5-yr follow-up period, cCMV-CI children with abnormal MRI scans catch up for speech perception, but lag behind for speech production. In future, the inclusion of MRI results may assist in improved counseling of parents with cCMV deafened children seeking CI.     

Longitudinal investigation of hearing disorders in children with congenital cytomegalovirus

This investigation consisted of a longitudinal study of the effects of congenital cytomegalovirus (CMV) infection on hearing sensitivity in 860 children with documented asymptomatic or symptomatic congenital CMV infection. Of the 651 children with asymptomatic CMV infection, 48 (7.4%) developed sensorineural hearing loss (SNHL), compared to 85 (40.7%) of the children with symptomatic CMV infection. Children in both groups experienced latent effects consisting of delayed onset of loss, threshold fluctuations, and/or progressive loss of hearing. It can be concluded that congenital CMV infection is a leading cause of SNHL in children. The late onset and progression of loss necessitates continued monitoring of hearing sensitivity in this population.     

Cochlear implantation in children

Cochlear implantation has revolutionized the treatment and prognosis of children with severe to profound sensorineural hearing loss who receive limited benefits from hearing aids. Children who receive cochlear implantation at young age, in particular before 2 years of age, can be expected to reach their normal age-equivalent developmental milestones and have higher chance to integrate into the mainstream educational settings. With the positive outcomes after cochlear implantation and the improvements in technology and surgical techniques, candidacy for cochlear implantation in children has been expanding to include hearing-impaired children with significant residual hearing, severe inner ear malformations, multiple handicaps such as mental retardation or visual impairment, and auditory neuropathy. Furthermore, there is growing interest in offering bilateral cochlear implantation to give children the benefits of binaural hearing. As the candidacy criteria expand, cochlear implant programs including preoperative evaluation, surgery, and habilitation have become more complex. Therefore, candidates should be selected prudently by multidisciplinary approach and cochlear implantation in children is much better to be provided by experienced cochlear implant team consisting of experts in relevant fields for the best results.     

Cochlear implantation in children deafened by cytomegalovirus: speech perception and speech intelligibility outcomes.

BACKGROUND: Concerns have been expressed with regard to suitability for cochlear implantation of children deafened by cytomegalovirus because of possible coexisting central disorders/learning difficulties. The aim of the current study was to assess speech perception and intelligibility of speech produced by children deafened by cytomegalovirus and compare their progress with that of congenitally deaf children after cochlear implantation. METHODS: The study assessed 16 implanted children who were deafened by cytomegalovirus, using the Iowa Closed Sentence Test and Speech Intelligibility Rating. The results were compared with those of a group of 131 children who had undergone implantation who were congenitally deaf but did not have cytomegalovirus as the cause of deafness. The mean age at implantation was 3.9 years for the cytomegalovirus group (median, 3.5 years) and 4.1 years (median, 4 years) for the congenitally deaf children. They all received the Nucleus multichannel cochlear implant system. The follow-up period ranged from 1 to 5 years after implantation for both groups. RESULTS: After cochlear implantation, the intelligibility of speech produced by children deafened by cytomegalovirus had a wide range, varying from unintelligible speech to connected speech intelligible to all listeners. Relative to the median score for the control group at the last evaluation interval, 3 of the 16 children with cytomegalovirus (19%) performed better, 8 children (50%) performed more poorly, and 5 (31%) performed the same. The difference between the two groups was not statistically significant (p > 0.05). With regard to speech perception Iowa Sentence Test (Level B), relative to the median score for the control group at the last evaluation interval, 5 of the 16 children with cytomegalovirus (31%) performed better, 3 children (19%) performed more poorly, and 8 (50%) performed the same. The difference between the two groups was not statistically significant (p > 0.05). With regard to Level A and relative to the median score for the control group at the last evaluation interval, 1 of the 16 children with cytomegalovirus (6%) performed better, 6 children (38%) performed more poorly, and 9 (56%) performed the same. The difference between the two groups was statistically significant (p = 0.04). CONCLUSION: The results of the current study showed that cytomegalovirus alone, as a cause of deafness, is not a contraindication for cochlear implantation. Parents should be informed about the wide range of linguistic outcomes after implantation and that these children may need more specific or intensive rehabilitation. Although additional problems are common and outcomes may, on average, be poorer, cochlear implantation can provide useful auditory input to these children. Further research is needed to identify factors associated with cytomegalovirus that may influence the outcomes.     

Cochlear implantation in children with cochlear aplasia

Abstract Conclusion: Children with cochlear aplasia can achieve favorable speech perception abilities from cochlear implantation (CI) by inserting an electrode array into the vestibule if an audiological response is reliably demonstrated before surgery by behavioral audiometry and objective measures. Objectives: The purpose of this study was to evaluate the surgical outcome of CI in children with cochlear aplasia. Methods: The medical records of 454 children who underwent CI at the Dong-A University Hospital between January 1994 and September 2011 were reviewed. The prevalence of cochlear aplasia and the preoperative evaluations, operative findings, and postoperative speech perception abilities of implanted children with cochlear aplasia were evaluated. Results: The implanted children with cochlear aplasia accounted for 2% of those with inner ear malformations and 0.4% of all pediatric cochlear implantees. They showed audiological responses in preoperative electrophysiologic tests, and the eighth nerve was present in magnetic resonance imaging. The electrode array was inserted into the vestibule through an oval window after removing the stapes or through a transmastoid labyrinthotomy site. Electrical compound action potential and electrical auditory brainstem response tests showed clear neural responses after implantation. The hearing thresholds of the implanted ear improved to 25 dB HL and the children achieved significant open set speech perception abilities.     

Cochlear implantation in children with otitis media

OBJECTIVE: To describe the outcome of cochlear implantation in a cohort of pediatric patients with a history of otitis media. STUDY DESIGN: Retrospective chart review and follow-up phone survey. METHODS: The medical records of patients younger than 48 months of age who underwent cochlear implantation were reviewed for demographics, complications, and history of otitis media and bilateral myringotomy and ventilation tubes. A follow-up phone survey was performed to determine the long-term natural history of otitis media. RESULTS: The records of 126 profoundly deaf pediatric patients were reviewed. Seventy-two percent had a history of at least one episode of otitis media, 31% had a history of greater than three episodes, and 2% had a history of chronic serous otitis media. Twenty-three percent underwent bilateral myringotomy and tubes in the distant past, 6 to 8 weeks before implantation, at the time of implantation, or after implantation. A phone survey was successfully completed for 76 patients with a mean follow-up period of 46 months. There was a significant decrease in the incidence of otitis media after the cochlear implantation in the majority of patients with a history of otitis media with and without a history of bilateral myringotomy and tubes. CONCLUSIONS: Placement of ventilation tubes either before or at the time of cochlear implantation seems to adequately prevent infectious disease-related complications in otitis-prone children. Therefore, implantation should not be delayed, and subsequent episodes of otitis media can be managed by conventional therapy.     

Cochlear implantation in children with cerebral palsy

Objective

Few studies have looked at the outcomes of children with complex needs following cochlear implantation. Increasing evidence supports the case for implantation in these children. To date there is very little evidence available evaluating the role of cochlear implantation in children with cerebral palsy. In this paper we look at the Manchester Cochlear Implant Programme's experience of implantation in 36 children with cerebral palsy.

Methods

A retrospective review of prospectively collected data for all children with cerebral palsy was undertaken. Cognitive and physical disability was scored by members of the cochlear implant team. A modified version of Geers and Moogs 1987 Speech Reception Score was used to assess outcome. Data was analysed looking at the relationship between cognitive and physical impairment, age at implantation and the SRS outcomes.

Results

This study demonstrated that children with cerebral palsy and a mild cognitive impairment do significantly better following implantation than those with a severe impairment (p = 0.008). Children with mild physical impairment did not appear to do significantly better than those with moderate or severe impairments (mild versus severe p = 0.13). Age at implantation was not a significant prognostic factor in this study group.

Conclusions

Children with complex needs are increasingly being referred for consideration of cochlear implantation. Further research is required to help guide candidacy, but each case must be considered individually. Higher functioning does appear to be the most important prognostic indicator regarding outcome but the effect of modest improvement in sound perception should not be underestimated.     

伴有听力障碍的多残儿童人工耳蜗疗效分析

目的 探讨多残儿童人工耳蜗植入手术的经验及问题。方法 我院近年来开展多导人工耳蜗植入术600余例，对其中8名多残儿童开展了多导人工耳蜗植入手术。8名患者中，深度感音性聋同时合并智障与脑瘫者1例，余合并智障者2例，其中一例智障患者合并Mondini畸形，合并脑瘫者3例，Usher’s综合症2例。对这8例患者进行回顾性分析，随访3～5年。就术后开机调试特点，术后听力言语康复效果以及存在的问题等进行讨论。结果 （1）术后无面瘫、脑脊液漏、脑膜炎等手术并发症；（2）手术后护理比常规人工耳蜗术后病人复杂：病人哭闹、卧床困难、输液困难、不配合医护人员工作等；（3）开机调试后所有术后多残儿童都有听觉反应，除1例听到声音后极为恐惧外，其余患者都非常兴奋，但调试比较困难，患者配合程度差，有时一个病人需要多次调试才能获得结果，增加了调机人员的工作难度；（4）经过艰苦的康复训练，言语辨别率比术前明显提高，家长对此很满意。结论 伴有多种残疾的深度感音性聋患者可以行人工耳蜗植入，术后增加了一个全新的康复手段，从而为整个康复计划起到了推动作用，减轻了患者家庭和社会的负担，但增加了医护人员术后护理、调机以及康复上的工作难度。     

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。     

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.     

耳道上径路耳蜗植入术

目的 探讨中国聋儿采用耳道上径路耳蜗植入的手术方法 .方法 2005年5月至2007年1月为50例(53侧)极重度感音性聋患儿采用耳道上径路行耳蜗植入.电极通过上鼓室的隧道于砧骨体与鼓索神经之间植入耳蜗鼓阶.结果 53侧手术电极全部植入者51侧;1例患儿(1侧)因耳蜗骨化只植入9对电极(Med-E1 C40+);另1例患儿(1侧)因耳蜗重度发育不良仅植入8对电极(Med-E1 C40+),该患儿术中发生井喷,植入电极时取出了砧骨并切断鼓索神经.1例患儿因鼓索神经与砧骨不易分离,电极经上鼓室隧道沿鼓索神经外侧植入鼓阶.所有病例术后无严重并发症发生.所有患儿耳蜗植入术后声场测听(听力级)均在30～40 dB;50例患儿随访均达6个月以上,其中26例具开放环境下的言语辨别力,己可进行交流;18例患儿可以讲短句,交流不太清楚;6例患儿只会讲单词.结论 耳道上径路耳蜗植入术是一种简单、安全的技术,中耳腔暴露好,不需要行乳突、面隐窝切开,无误伤面神经、鼓索神经的危险,尤其适用于面隐窝狭窄或面神经前移的患儿.     

Cochlear implantation in children with large vestibular aqueduct syndrome

OBJECTIVE: This study describes the effectiveness of a multielectrode cochlear implant prosthesis (Cochlear; Cochlear Pty., Lane Cove, Australia) for providing hearing to children with deafness caused by large vestibular aqueduct syndrome (LVAS). STUDY DESIGN: The study design was a retrospective study. SETTING: All the children attended The Children's Cochlear Implant Center (NSW), which is a specialist center that provides audiologic testing, speech therapy, habilitation, and medical assistance for children with cochlear implants. PATIENTS: Ten children were studied who had profound hearing loss and radiologic evidence of a vestibular aqueduct larger than 2 mm in width in its intraosseous portion. INTERVENTION: The children received a multielectrode (Cochlear) cochlear implant prosthesis, and the associated programming of the device and habitation were performed postoperatively. No significant problems were encountered at any of the surgeries, although there was an initial gush of perilymph when the otic capsule was opened in 7 ears. MAIN OUTCOME MEASURES: Postoperative audiologic performance at six monthly intervals and school performance were assessed. RESULTS: The postoperative auditory performance was improved in all children. At 6 months, their average BKB score had increased from 31% to 79%; average word score, from 8% to 43%; and average phoneme score, from 38% to 70%. The older children were able to continue their education in their usual setting with less reliance on hearing support staff. CONCLUSION: Children with a deteriorating hearing loss caused by LVAS can derive considerable benefit from a cochlear implant.