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1.

Objectives

Cochlear implantation (CI) is a revolutionary method for hearing rehabilitation in patients with severe or profound sensorineural hearing loss. One of the surgical complications may be the necrosis of the skin flap above the receiver-stimulator coil, resulting in device extrusion. Our aim was to find the plausible causes of the silicone covered implant rejection.

Patients and methods

Authors present four cases of cochlear implant rejection, briefly describe their dermato-surgical solutions and analyse their innovative method – the epicutanoeus patch testing with silicone samples.

Results

They observed positive skin reaction in three of the four cases.

Conclusion

Authors analyse the applicability and results of their surgical solutions and the epicutaneous testing in connection with the prevention of skin flap necrosis and rejection of silicone-covered cochlear implants.  相似文献   

2.

Purpose

The advent of cochlear implantation has been followed by an explosion in surgical and technological advances with subsequent alterations in the treatment of sensorineural hearing loss management. Many individuals with rare genetic abnormalities who once may have not been considered candidates for cochlear implantation are now benefiting from cochlear implantation. One of these unusual syndromes is the 13q deletion syndrome. The clinical features of 13q deletion syndrome, a rare chromosomal abnormality, were originally described in the early 1960s, though the literature currently lacks defined phenotypic abnormalities. Patients with 13q deletion syndrome present with varying degrees of cognitive and growth delay, craniofacial dysmorphisms, and congenital malformations. The etiology of the sensorineural hearing loss has not been elucidated, and genes associated with other syndromes which include sensorineural hearing loss have been proposed as the causative agents. We describe the unique clinical and surgical considerations for 13q deletion syndrome and review the considerations when deliberating on cochlear implantation in syndromic hearing loss.

Materials and Methods

Case report.

Results

Successful cochlear implantation in a patient with 13q deletion syndrome.

Conclusion

Many patients with 13q deletion syndrome suffer from sensorineural hearing loss, and when appropriately selected may likely benefit from cochlear implantation. Many other syndromic patients following careful selection may also benefit from cochlear implantation and should not be excluded from consideration until appropriately evaluated.  相似文献   

3.

Objective

To explore audiologic outcome of auditory brain stem implantation (ABI) and cochlear implantation (CI) in NF2 patients and patients with vestibular schwannoma (VS) in the only hearing ear.

Patients and methods

Study includes retrospective analysis of 2 cases. One is totally deaf patient due to NF2, and the other one is totally deaf due to VS development in only hearing ear. Tumor was removed by retrosigmoid approach in NF2 patient and ABI was performed simultaneously. For the VS in only hearing ear case, tumor was removed by translabyrinthine approach and CI was performed simultaneously.

Results

ABI patient showed quite well outcome during the 15 months of follow-up. She has 25 dB hearing threshold at speaking frequencies. She developed open set speech discrimination with 87.5% word discrimination score, and 70% sentence discrimination score. She uses device daily manner, she can use telephone. For CI patient, outcome is not perfect but satisfactory. She couldn’t develop open set speech discrimination during the 18 months of follow-up. She has 67% the disyllabic words recognition score (close set). She is daily user of device. CI improves quite well lip reading.

Conclusion

ABI and CI are the two options to restore hearing in VS caused deafness. We advocate giving every effort to preserve cochlear nerve during the VS resection and place CI simultaneously. However if it is not possible to preserve cochlear nerve during surgery, ABI is also a good alternative for hearing restoration.  相似文献   

4.

Objectives

Universal newborn hearing screening has significantly improved the ability to identify patients with congenital sensorineural hearing loss (SNHL), which results in earlier treatment and better hearing and development outcomes. It is recommended that patients born with SNHL who meet criteria receive cochlear implants (CIs) by a target age of 12 months, however many children are being implanted at an older age. This study aims to describe populations of pre-lingual patients with SNHL that are at risk for delayed implantation and to identify and analyze barriers that cause this delay.

Methods

Charts of patients receiving a CI between January 2008 and June 2012 at a tertiary care cochlear implant center were reviewed retrospectively. We looked at patient demographics, age at hearing loss diagnosis, age at implantation, and etiology of hearing loss. Barriers to implantation were identified through surveys completed by team members.

Results

Fifty-seven CI recipients were identified of which 42 were in patients with pre-lingual SNHL. SNHL etiology included: cochlear dysplasia (18%), GJB2/GJB6 (17%), acquired (10%) extreme prematurity (9%), and idiopathic (46%). The median age of SNHL diagnosis for pre-lingual patients was 15 months. Compared to private insurance, public insurance status was associated with SNHL diagnosis at a significantly later median age (20.0 vs. 4.0 months, p = 0.024), and with a significantly longer median interval from diagnosis to implantation (25.5 vs. 11.0 months, p = 0.029). While cochlear implant team members identified delayed insurance approval and medical comorbidities as reasons for delayed implantation, the most significant factor identified was parental, with delayed/missed appointments or reluctance for evaluations or surgery.

Conclusion

52% of patients with pre-lingual SNHL that met criteria for CI were implanted more than 12 months after diagnosis. Having public or no insurance was significantly associated with delayed implantation. Parental barriers were most common factors cited for delays in implantation. Overcoming these delays necessitates appropriate identification of at risk patients and creating a system to educate families and chaperone them through the process.  相似文献   

5.

Objectives

To report of a 65-year-old woman with bilateral Meniere's disease was referred for cochlear implantation (CI) due to severe/profound sensorineural hearing loss.

Methods

During the assessment workup, a vestibular schwannoma in the right ear was found by MR imaging. She underwent a translabyrinthine removal of the acoustic neuroma (AN) with sparing of the cochlear nerve and concurrent ipsilateral CI with a Nucleus Freedom device (Cochlear Ltd., Lane Cove, New South Wales, Australia).

Results

Complete removal of the AN was achieved without complications. Neural Response Telemetry (NRT) measurements, which showed poor morphology at the intraoperative tests, rapidly improved after activation, similarly to electrically evoked auditory brainstem responses (E-ABR). The patient reached 100% speech perception performances within 2 months from implantation, in the monaural condition. She was relieved from vertigo spell up to 14 months after the operation.

Conclusion

Cochlear implantation at the time of acoustic neuroma removal with VIII nerve sparing can be a safe and effective hearing restoration procedure.  相似文献   

6.

Introduction

Research on early cochlear implantation and first language milestones is limited. To compare language performance in cochlear implant (CI) users and hearing children, the establishment of normative data for both groups would be of benefit. To aid the data collection for Turkish hearing children and children with a CI diaries can be used.

Aims

This study aimed to document the first 100-word lexicon acquired by Turkish hearing children and children with a CI during the first 2 years of hearing experience, to determine the distribution of the first 100-word lexicon per word category, the rate of acquisition of words and the effects of age at implantation on language performance.

Methods

First word data was collected from 63 Turkish hearing children and 71 CI users implanted under 36 months of chronological age using a diary. The mean number of words recorded at each time interval was calculated. The time taken to achieve the first 100-word lexicon and the categories of the first words were documented. Performance under 18 months and over 24 months of age at first fitting was compared.

Results

By 19–21 months of hearing age both hearing and CI user's vocabularies were of similar size. CI users developed a lexicon earlier than hearing children, but once hearing children started to acquire words their acquisition rate was faster. The distribution of words acquired per category were similar. 83% of first words were shared by both groups. No significant difference in performance was found between: hearing versus: implanted children; or earlier (<18 months) versus later (≥24 months) implanted children.

Conclusions

The vocabulary of hearing children compared to CI users are similar in size and the category. Early access to auditory stimuli facilitates children with a hearing loss to develop vocabularies similar to hearing children in the short term.  相似文献   

7.

Objective

To demonstrate the added value of magnetic resonance imaging (MRI) over computed tomography (CT) during adult cochlear implant evaluation.

Patients

Two adult patients are discussed in whom MRI studies diagnosed bilateral vestibular schwannomas during cochlear implant candidacy evaluation.

Interventions

Temporal bone CT and MRI.

Main outcome measure

Diagnosis of NF2.

Results

Two adult patients, ages 67 and 68 years, were evaluated for cochlear implant candidacy. Both patients experienced slowly progressive, bilateral hearing loss without complaints of vertigo, and neither patient had a family history of hearing loss or neurogenic tumors. Both patients had near-symmetric pure tone thresholds on audiometric testing. An MRI and a CT scan were performed on both patients, and bilateral vestibular schwannomas were identified on MRI.

Conclusions

Though MRI is not routinely utilized in adult cochlear implant evaluation, it may be of greater clinical value than CT in the setting of adult-onset hearing loss. MRI allows for sensitive evaluation of cochlear patency and architecture, and cochlear nerve status. As demonstrated in the two index cases, MRI also provides the added benefit of evaluating for causes of retrocochlear hearing loss. These two patients would have likely experienced a significant delay in diagnosis of NF2 without preoperative MRI, particularly given the limitations of scanning following CI magnet placement.  相似文献   

8.

Objective

To predict cochlear implant efficacy and investigate the cortical processing of the visual component of language in profoundly deafened patients with asymptomatic congenital cytomegalovirus (CMV) infection.

Methods and cases

The cortical activity of two children with CMV-related hearing loss was evaluated with fluorodeoxyglucose-positron emission tomography (FDG-PET) with a visual language task before cochlear implantation. Total development and auditory perception ability were assessed one year after implantation.

Results

The two children with CMV-related hearing loss showed activation in the auditory association area where no activation was found in the controls, and exhibited nearly identical cortical activation patterns to those seen in patients with profound congenital hearing loss. In contrast, differences in total development in verbal ability and discrimination of sentences between the two cases were revealed one year after implantation.

Conclusion

These results might indicate that the differences of cortical activities according to hearing abilities could have been influenced by CMV infection that involves higher function of the brain directly and/or affects the cochlea peripherally. Additionally, if CMV infection might have affected only the cochlea, these cortical activation patterns were influenced secondary by the time course of hearing loss characterized by CMV infection, which had varied manifestations.Accurate diagnosis and cochlear implantation at the appropriate time are important for successful speech development, and each patient needs a personalized habilitation program based on their etiology and brain function.  相似文献   

9.

Objectives

The aim of the present study was to evaluate whether the arithmetic achievement of children with cochlear implants (CI) was lower or comparable to that of their normal hearing peers and to identify predictors of arithmetic achievement in children with CI. In particular we related the arithmetic achievement of children with CI to nonverbal IQ, reading skills and hearing variables.

Methods

23 children with CI (onset of hearing loss in the first 24 months, cochlear implantation in the first 60 months of life, atleast 3 years of hearing experience with the first CI) and 23 normal hearing peers matched by age, gender, and social background participated in this case control study. All attended grades two to four in primary schools. To assess their arithmetic achievement, all children completed the “Arithmetic Operations” part of the “Heidelberger Rechentest” (HRT), a German arithmetic test. To assess reading skills and nonverbal intelligence as potential predictors of arithmetic achievement, all children completed the “Salzburger Lesetest” (SLS), a German reading screening, and the Culture Fair Intelligence Test (CFIT), a nonverbal intelligence test.

Results

Children with CI did not differ significantly from hearing children in their arithmetic achievement. Correlation and regression analyses revealed that in children with CI, arithmetic achievement was significantly (positively) related to reading skills, but not to nonverbal IQ. Reading skills and nonverbal IQ were not related to each other. In normal hearing children, arithmetic achievement was significantly (positively) related to nonverbal IQ, but not to reading skills. Reading skills and nonverbal IQ were positively correlated. Hearing variables were not related to arithmetic achievement.

Conclusions

Children with CI do not show lower performance in non-verbal arithmetic tasks, compared to normal hearing peers.  相似文献   

10.

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.  相似文献   

11.

Objective

To evaluate the auditory and speech abilities in children with auditory neuropathy spectrum disorder (ANSD) after cochlear implantation (CI) and determine the role of age at implantation.

Methods

Ten children participated in this retrospective case series study. All children had evidence of ANSD. All subjects had no cochlear nerve deficiency on magnetic resonance imaging and had used the cochlear implants for a period of 12–84 months. We divided our children into two groups: children who underwent implantation before 24 months of age and children who underwent implantation after 24 months of age. Their auditory and speech abilities were evaluated using the following: behavioral audiometry, the Categories of Auditory Performance (CAP), the Meaningful Auditory Integration Scale (MAIS), the Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS), the Standard-Chinese version of the Monosyllabic Lexical Neighborhood Test (LNT), the Multisyllabic Lexical Neighborhood Test (MLNT), the Speech Intelligibility Rating (SIR) and the Meaningful Use of Speech Scale (MUSS).

Results

All children showed progress in their auditory and language abilities. The 4-frequency average hearing level (HL) (500 Hz, 1000 Hz, 2000 Hz and 4000 Hz) of aided hearing thresholds ranged from 17.5 to 57.5 dB HL. All children developed time-related auditory perception and speech skills. Scores of children with ANSD who received cochlear implants before 24 months tended to be better than those of children who received cochlear implants after 24 months. Seven children completed the Mandarin Lexical Neighborhood Test. Approximately half of the children showed improved open-set speech recognition.

Conclusion

Cochlear implantation is helpful for children with ANSD and may be a good optional treatment for many ANSD children. In addition, children with ANSD fitted with cochlear implants before 24 months tended to acquire auditory and speech skills better than children fitted with cochlear implants after 24 months.  相似文献   

12.

Objective

In this study causes, risk factors, prevention strategies, diffuculties encountered during revision cochlear implant surgery, reimplantation in pediatric age group were evaluated.

Methods

Charts of 957 CI patients younger than 18 years of age implanted at Izmir Bozyaka Teaching and Research Hospital between 1998 and July 2012 and 18 referred CI complications at same age group were retrospectively evaluated. Revision and reimplantation surgeries were systematically reviewed.

Results

We encountered 26 surgical complications in 24 patients and 36 device related problems in 36 patients. Eighteen referred cases including 7 surgical complications and 11 device related problems were also evaluated. A total number of 80 complications were evaluated. In 11 cases conservative management was successful, 19 revision surgery was performed but we failed in 4 cases. We reimplanted 48 cases and 4 additional cases were implanted after failed revision surgery. Implant was extracted in one case. In one case we could implant the other side. Forty-six of reimplantations were done in one stage surgery, 5 cases required second stage surgery.

Conclusions

Surgical complications and device related problems of cochlear implantation may be different in children and majority of them require revision surgery or reimplantation. Although surgical problems leading to revision surgery and reimplantation are expected to diminish by experience every center has to deal with device failures. Both revision surgery and reimplantation require extra care and it should be better carried out by experienced surgeons. Implant performances are expected to be comparable with primary implantations.  相似文献   

13.

Objective

The objective of this study was to evaluate the relationship between developmental delays and speech perception in pre-lingually deafened cochlear implant recipients.

Methods

This study was a retrospective review of patient charts conducted at a tertiary referral center. Thirty-five pre-lingually deafened children underwent multichannel cochlear implantation and habilitation at the Kyoto University Hospital Department of Otolaryngology-Head and Neck Surgery. A pre-operative cognitive–adaptive developmental quotient was evaluated using the Kyoto scale of psychological development. Post-operative speech performance was evaluated with speech perception tests two years after cochlear implantation. We computed partial correlation coefficients (controlled for age at the time of implantation and the average pre-operative aided hearing level) between the cognitive–adaptive developmental quotient and speech performance.

Results

A developmental delay in the cognitive–adaptive area was weakly correlated with speech perception (partial correlation coefficients for consonant–vowel syllables and phrases were 0.38 and 0.36, respectively).

Conclusion

A pre-operative developmental delay was only weakly associated with poor post-operative speech perception in pre-lingually deafened cochlear implant recipients.  相似文献   

14.

Objective

The present study investigates how auditory stimulation from cochlear implants (CI) is associated with the development of Theory of Mind (ToM) in severely and profoundly hearing impaired children with hearing parents. Previous research has shown that deaf children of hearing parents have a delayed ToM development. This is, however, not always the case with deaf children of deaf parents, who presumably are immersed in a more vivid signing environment.

Methods

Sixteen children with CI (4.25 to 9.5 years of age) were tested on measures of cognitive and emotional ToM, language and cognition. Eight of the children received their first implant relatively early (before 27 months) and half of them late (after 27 months). The two groups did not differ in age, gender, language or cognition at entry of the study. ToM tests included the unexpected location task and a newly developed Swedish social–emotional ToM test. The tests aimed to test both cognitive and emotional ToM. A comparison group of typically developing hearing age matched children was also added (n = 18).

Results

Compared to the comparison group, the early CI-group did not differ in emotional ToM. The late CI-group differed significantly from the comparison group on both the cognitive and emotional ToM tests.

Conclusion

The results revealed that children with early cochlear implants solved ToM problems to a significantly higher degree than children with late implants, although the groups did not differ on language or cognitive measures at baseline. The outcome suggests that early cochlear implantation for deaf children in hearing families, in conjunction with early social and communicative stimulation in a language that is native to the parents, can provide a foundation for a more normalized ToM development.  相似文献   

15.

Objectives

To determine the long term effect of cochlear implant (CI) in children with GJB2-related deafness in Japan.

Methods

Genetic testing was performed on 29 children with CI. The speech perception in 9 children with GJB2 gene-related deafness fitted with CI was compared with those in matched 10 children who were diagnosed as having no genetic loci. The average follow-up period after CI was 55.9 months and 54.6 months, respectively.

Results

A definitive inherited hearing impairment could be confirmed in 12 (41.4%) of the 29 CI children, including 10 with GJB2-related hearing impairment and 2 with SLC26A4-related hearing impairment. The results of IT-MAIS, word or speech perception testing under the noise, and development of speech perception and production testing using the Enjoji scale were slightly better for the GJB2 group after CI than for the control group without statistical significant difference.

Conclusion

The long-term results of this study show that CI is also effective in the development of speech performance after CI in Japanese children with GJB2-related hearing impairments as HL due to other etiologies.  相似文献   

16.

Objective

It is well known that a proper fitting of the cochlear implant processor is relevant to provide good quality in speech perception. The aim of this study is to extract statistical information to be applied for fitting the processor.

Methods

This study is based on the programming maps of 121 patients, aged from 18 months to 68 years at the moment of implantation. All subjects were implanted with the COMBI 40+ cochlear implant at San Cecilio University Hospital, Granada (Spain). The patients were classified into groups based on their age at implantation: younger than 5 years, between 5 and 16 years, and older than 16 years. The patients in each age-based group were divided into two subgroups, considering whether they had recent hearing experience or not. A special group including patients affected by severe damages in the cochlea was also defined.

Results

Relationships between the programming parameters and factors like the age at implantation, the hearing experience and the presence of severe cochlear damage were found. The THR levels for patients younger than 5 years were significantly lower than those for patients implanted between 5 and 16 years, and this group presented significantly lower THR levels than adults. The MCL levels were not significantly influenced by the age at implantation. A significant increment was observed for both, MCL and THR levels, when patients were affected by severe cochlear damage. A significant increment in the THR levels were observed for patients with no recent hearing experience, while no significant differences were found for MCL levels. This study also analyzes the distribution along the cochlea of the stimulation levels. In the case of patients not affected by severe cochlear damage, the most basal electrodes presented a significant increment in the stimulation levels with respect to the rest of electrodes.

Conclusion

This work provides information of great value for programming the speech processors, particularly when the subjective responses of the patients are not sufficient. The application in our ENT Service has reduced substantially the average time needed to obtain an acceptable fitting of the processor, especially in children. Our study also shows that electrical thresholds are a good indicator of the functionality of the auditory nerve. The analysis of this parameter highlights the importance of an early intervention as well as a deep insertion of the electrode carrier in order to obtain the maximum functionality from the cochlear implant.  相似文献   

17.

Objective

To report electrode array misplacement into the superior semicircular canal occurring as an rare complication of cochlear implantation through round window insertion, and to explore the causative association between electrode array misplacement and cochlear implantation surgical techniques.

Methods

A chart review of the electrode array misplacement into the superior semicircular canal and their management in 695 patients undergoing cochlear implantation was undertaken from January 2003 and January 2014 in Anhui Provincial Hospital.

Results

There were two children of electrode array misplacement into the superior semicircular canal complication, and the rate was 0.28%.

Conclusions

Electrode array misplacement into the superior semicircular canal associated with cochlear implantation is rare. Surgeons should be aware of that the smaller round window maybe the reason of electrode array misplacement through round window insertion. Intra-operative neural response telemetry and X-ray can alert the surgeon the problem with the array's misplacement, which can be identified by postoperative CT.  相似文献   

18.

Objectives

Down Syndrome (DS) is associated with a high incidence of hearing loss. The majority of hearing loss is conductive, but between 4 and 20% is sensorineural, which in the main is mild or moderate and is managed with conventional behind-the-ear hearing aids. Cochlear implantation is an elective invasive procedure, performed to provide some form of hearing rehabilitation in individuals with severe to profound sensorineural hearing loss, and initially candidacy criteria were strict—excluding patients with additional disabilities. With good results and expanding experience, more candidates with additional disabilities are now being implanted. A survey of UK and Ireland Cochlear Implant Programmes sought to identify the number of individuals with DS who have been implanted with a cochlear implant (CI) and to provide relevant information on outcomes of implantation in these individuals.

Methods

E-mail survey of all programmes within the British Cochlear Implant Group (BCIG). Postal questionnaire to programmes identified to have implanted a child with Down Syndrome, with data collection on pre-operative assessment, surgical and post-operative outcomes. Case series review.

Results

Three of 23 BCIG programmes have implanted a child with Down Syndrome. Four children have received implants. No intraoperative or post-operative surgical complications were encountered. All children had middle ear disease, but no problems with implantation were associated with their middle ear condition. All children remain implant users, 12 months to 4 years post-implantation.

Conclusion

Cochlear implantation is an option for a child with Down Syndrome and associated severe to profound sensorineural hearing loss. Clinicians caring for these children and their families should consider referral for assessment by a Cochlear Implant Programme.  相似文献   

19.

Objective

The aim of this study was to report the effect of unilateral cochlear implantation to vestibular system using vestibular evoked myogenic potentials (VEMPs) by air-conduction in a sample of children aged less than 5 years.

Materials

This study consisted of 10 children (6 boys and 4 girls), who underwent cochlear implantation surgery at our clinic, and 8 normal hearing children (5 boys and 3 girls) matched for age. The VEMPs were performed before, 10 days, and 6 months after surgery. Both the implanted and unimplanted ears of each child were evaluated, with the cochlear implant both off and on.

Results

Preoperatively, six (60%) children had abnormal VEMPs responses on both ears. In the postoperative sessions, no child showed any VEMPs response on the implanted side. The VEMPs were not recorded on the unimplanted side either, except for one case. At 6 months, the VEMPs response on the unimplanted side of three children became normal when the cochlear implant was on, and in two children with the device off.

Conclusion

In the postoperative 6-month-period, the disappearance of VEMPs suggests that the saccule of children can be extensively damaged following cochlear implantation. A recovery of VEMPs can take place on the unimplanted side, with the cochlear implant both on and off. Despite this saccular injury, the absence of clinical signs in children could be explained by their ability to effectively compensate for such vestibular deficits.  相似文献   

20.
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