The effect of pre-operative developmental delays on the speech perception of children with cochlear implants

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.     

Auditory performance and speech intelligibility of Mandarin-speaking children implanted before age 5

Objectives

(1) To report the auditory performance and speech intelligibility of 84 Mandarin-speaking prelingually deaf children after using cochlear implants (CIs) for one, two, three, four, and five years to understand how many years of implant use were needed for them to reach a plateau-level performance; (2) to investigate the relation between subjective rating scales and objective measurements (i.e., speech perception tests); (3) to understand the effect of age at implantation on auditory and speech development.

Methods

Eighty-four children with CIs participated in this study. Their auditory performance and speech intelligibility were rated using the Categorical Auditory Performance (CAP) and the Speech Intelligibility Rating (SIR) scales, respectively. The evaluations were made before implantation and six months, one, two, three, four, and five years after implantation. At the fifth year after implantation, monosyllabic-word, easy-sentence, and difficult-sentence perception tests were administered.

Results

The median CAP score reached a plateau at category 6 after three years of implant use. The median SIR arrived at the highest level after five years of use. With five years of CI experiences, 86% of the subjects understood conversation without lip-reading, and 58% were fully intelligible to all listeners. The three speech perception tests had a moderate-to-strong correlation with the CAP and SIR scores. The children implanted before the age of three years had significantly better CAP and monosyllabic word perception test scores.

Conclusions

Five years of follow-up are needed for assessing the post-implantation development of communication ability of prelingually deafened children. It is recommended that hearing-impaired children receive cochlear implantation at a younger age to acquire better auditory ability for developing language skills. Constant postoperative aural–verbal rehabilitation and speech and language therapy are most likely required for the patients to reach the highest level on the CAP and SIR scales.     

Oral communicating children using a cochlear implant: Good reading outcomes are linked to better language and phonological processing abilities

Introduction/objective

Cochlear implantation provides children with a significant hearing loss the potential to engage in phonological processing via audition; however these children can still have poor or inadequately detailed mental (phonological) representations of speech and as such phonological awareness and reading difficulties. Heterogeneous participant profiles, particularly varying modes of communication have clouded the research regarding reading outcomes of children using a cochlear implant. The objective of this study was to explore the relationships between word reading and reading comprehension outcomes, and a range of variables of a relatively homogenous group of children using cochlear implants.

Method

Forty-seven oral communicating children using a cochlear implant and who had attended auditory-verbal therapy served as participants. They were administered a comprehensive battery of 10 different assessments covering 22 different tasks across the domains of speech perception, speech production, language, phonological processing and reading. Correlation and principal component analyses were used to examine the relationships between outcome areas.

Results

Audiologic and demographic variables were not significantly related to reading outcomes, with the exception of family size. Language and word reading were most strongly related to reading comprehension, while phonological awareness and language were most strongly related to word reading. It is proposed that the development of well-specified phonological representations might underlie these relationships.

Conclusion

For oral communicating children using a cochlear implant, good reading outcomes are linked to better language and phonological processing abilities.     

Comparing the effect of auditory-only and auditory–visual modes in two groups of Persian children using cochlear implants: A randomized clinical trial

Objective

Since the introduction of cochlear implantation, researchers have considered children's communication and educational success before and after implantation. Therefore, the present study aimed to compare auditory, speech, and language development scores following one-sided cochlear implantation between two groups of prelingual deaf children educated through either auditory-only (unisensory) or auditory–visual (bisensory) modes.

Design

A randomized controlled trial with a single-factor experimental design was used.

Methods

The study was conducted in the Instruction and Rehabilitation Private Centre of Hearing Impaired Children and their Family, called Soroosh in Shiraz, Iran. We assessed 30 Persian deaf children for eligibility and 22 children qualified to enter the study. They were aged between 27 and 66 months old and had been implanted between the ages of 15 and 63 months. The sample of 22 children was randomly assigned to two groups: auditory-only mode and auditory–visual mode; 11 participants in each group were analyzed. In both groups, the development of auditory perception, receptive language, expressive language, speech, and speech intelligibility was assessed pre- and post-intervention by means of instruments which were validated and standardized in the Persian population.

Results

No significant differences were found between the two groups. The children with cochlear implants who had been instructed using either the auditory-only or auditory–visual modes acquired auditory, receptive language, expressive language, and speech skills at the same rate.

Conclusion

Overall, spoken language significantly developed in both the unisensory group and the bisensory group. Thus, both the auditory-only mode and the auditory–visual mode were effective. Therefore, it is not essential to limit access to the visual modality and to rely solely on the auditory modality when instructing hearing, language, and speech in children with cochlear implants who are exposed to spoken language both at home and at school when communicating with their parents and educators prior to and after implantation.The trial has been registered at IRCT.ir, number IRCT201109267637N1.     

Effect of age at cochlear implantation on auditory and speech development of children with auditory neuropathy spectrum disorder

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.     

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.     

Development of Mandarin spoken language after pediatric cochlear implantation

Objective

The purpose of this study was to evaluate early spoken language development in young Mandarin-speaking children during the first 24 months after cochlear implantation, as measured by receptive and expressive vocabulary growth rates. Growth rates were compared with those of normally hearing children and with growth rates for English-speaking children with cochlear implants.

Method

Receptive and expressive vocabularies were measured with the simplified short form (SSF) version of the Mandarin Communicative Development Inventory (MCDI) in a sample of 112 pediatric implant recipients at baseline, 3, 6, 12, and 24 months after implantation. Implant ages ranged from 1 to 5 years. Scores were expressed in terms of normal equivalent ages, allowing normalized vocabulary growth rates to be determined. Scores for English-speaking children were re-expressed in these terms, allowing direct comparisons of Mandarin and English early spoken language development.

Results

Vocabulary growth rates during the first 12 months after implantation were similar to those for normally hearing children less than 16 months of age. Comparisons with growth rates for normally hearing children 16–30 months of age showed that the youngest implant age group (1–2 years) had an average growth rate of 0.68 that of normally hearing children; while the middle implant age group (2–3 years) had an average growth rate of 0.65; and the oldest implant age group (>3 years) had an average growth rate of 0.56, significantly less than the other two rates. Growth rates for English-speaking children with cochlear implants were 0.68 in the youngest group, 0.54 in the middle group, and 0.57 in the oldest group. Growth rates in the middle implant age groups for the two languages differed significantly.

Conclusions

The SSF version of the MCDI is suitable for assessment of Mandarin language development during the first 24 months after cochlear implantation. Effects of implant age and duration of implantation can be compared directly across languages using normalized vocabulary growth rates. These comparisons for Mandarin and English reveal comparable results, despite the diversity of these languages, underscoring the universal role of plasticity in the developing auditory system.     

Paediatric cochlear implantation in north-western Nigeria case report and review of our challenges

Objective

To report the commencement of cochlear implantation (CI) in children in Northern Nigeria and review the challenges encountered with a view to establishing CI programme.

Cases

Two children with post-meningitic sensorineural hearing loss had standard pre-implantation assessment and cochlear implantation surgery in Nigeria in conjunction with surgeons from another developing country. Post-implantation meningitis was encountered and managed in one of the patients. Post-CI rehabilitation started in a foreign country and continued in our centre after training of our speech therapist. Both patients had improvement in their hearing.

Conclusion

These showcase successful CI in patients from Sub-Saharan Africa, the challenges included need for development of surgical and post-CI rehabilitation personnel and affordability of the cost of the implant. The support of all the stakeholders needed for commencement of a CI programme was noted.     

Analysis of electrical thresholds and maximum comfortable levels in cochlear implant patients

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.     

Acquisition of early auditory milestones with a cochlear implant

Objective

Speech acquisition after cochlear implant is a long process. Various studies have followed the auditory milestones in the early period after implantation. The aim of the present study was to track the development of hearing skills in the early period after cochlear implantation and evaluate which factors influence the process.

Methods

195 records of children implanted in the Hadassah Medical Center were examined retrospectively. Data on etiology, age at implantation and type of implant were collected. In addition, information on the rate of progress was measured: the first time that there was detection and identification of Ling sounds, the first time it was possible to obtain SDT (speech detection threshold), SRT (speech reception threshold) and an audiogram, and the first accurate repetition of VCV (vowel consonant vowel) sounds.

Results

Results show a consistent pattern of auditory milestone acquisition similar to that of normal development, from milestones that do not require decoding beginning with SDT, detection of Ling sounds followed by an audiogram which requires cooperation, to tasks that involve decoding starting with SRT and repetition of Ling sounds and finally VCV repetition.The children implanted before 24 months of age achieved the auditory milestones later than children implanted between 2 and 6 years, apparently since these tasks involve cognitive abilities which are not yet developed in the youngest children.Previous hearing experience improved the rate of acquisition of the auditory milestones and progress was faster in the second implanted ear compared to the first implanted ear.

Conclusion

More research is needed to address the relationship between acquisition of early auditory milestones and performance with the cochlear implant later on in life.     

An investigation of the first lexicon of Turkish hearing children and children with a cochlear implant

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.     

Auditory–cognitive training improves language performance in prelingually deafened cochlear implant recipients

Objectives

Phonological and working memory skills have been shown to be important for the development of spoken language. Children who use a cochlear implant (CI) show performance deficits relative to normal hearing (NH) children on all constructs: phonological skills, working memory, and spoken language. Given that phonological skills and working memory have been shown to be important for spoken language development in NH children, we hypothesized that training these foundational skills would result in improved spoken language performance in CI-using children.

Design

Nineteen prelingually deafened CI-using children aged 4- to 7-years-old participated. All children had been using their implants for at least one year and were matched on pre-implant hearing thresholds, hearing thresholds at study enrollment, and non-verbal IQ. Children were assessed on expressive vocabulary, listening language, spoken language, and composite language. Ten children received four weeks of training on phonological skills including rhyme, sound blending, and sound discrimination and auditory working memory. The remaining nine children continued with their normal classroom activities for four weeks. Language assessments were repeated following the training/control period.

Results

Children who received combined phonological-working memory training showed significant gains on expressive and composite language scores. Children who did not receive training showed no significant improvements at post-test. On average, trained children had gain scores of 6.35 points on expressive language and gain scores of 6.15 points whereas the untrained children had test–retest gain scores of 2.89 points for expressive language and 2.56 for composite language.

Conclusion

Our results suggest that training to improve the phonological and working memory skills in CI-using children may lead to improved language performance.     

Inferences and metaphoric comprehension in unilaterally implanted children with adequate formal oral language performance

Objectives

To assess skills in inferences during conversations and in metaphors comprehension of unilaterally cochlear implanted children with adequate abilities at the formal language tests, comparing them with well-matched hearing peers; to verify the influence of age of implantation on overall skills.

Methods

The study was designed as a matched case–control study. 31 deaf children, unilateral cochlear implant users, with normal linguistic competence at formal language tests were compared with 31 normal hearing matched peers. Inferences and metaphor comprehension skills were assessed through the Implicit Meaning Comprehension, Situations and Metaphors subtests of the Italian Standardized Battery of “Pragmatic Language Skills MEDEA”. Differences between patient and control groups were tested by the Mann–Whitney U test. Correlations between age at implantation and time of implant use with each subtest were investigated by the Spearman rank correlation coefficient.

Results

No significant differences between the two groups were found in inferencing skills (p = 0.24 and p = 0.011 respectively for Situations and Implicit Meaning Comprehension). Regarding figurative language, unilaterally cochlear implanted children performed significantly below their normal hearing peers in Verbal Metaphor comprehension (p = 0.001). Performances were related to age at implantation, but not with time of implant use.

Conclusions

Unilaterally cochlear implanted children with normal language level showed responses similar to NH children in discourse inferences, but not in figurative language comprehension. Metaphors still remains a challenge for unilateral implant users and above all when they have not any reference, as demonstrated by the significant difference in verbal rather than figurative metaphors comprehension. Older age at implantation was related to worse performance for all items. These aspects, until now less investigated, had to receive more attention to deeply understand specific mechanisms involved and possible effects of different levels of figurative language complexity (presence or absence of contextual input, degree of transparency and syntactic frozenness). New insight is needed to orient programs in early intervention settings in considering and adequately responding to all these complex communicative need of children with hearing loss.     

Simultaneous translabyrinthine removal of acoustic neuroma and cochlear implantation

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.     

Evaluation of cortical processing of language by use of positron emission tomography in hearing loss children with congenital cytomegalovirus infection

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.     

Vestibular evoked myogenic potentials in children after cochlear implantation

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.     

Working memory in Farsi-speaking children with normal development and cochlear implant

Aim and background

Working memory has an important role in language acquisition and development of cognition skills. The ability of encoding, storage and retrieval of phonological codes, as activities of working memory, acquired by audition sense. Children with cochlear implant experience a period that they are not able to perceive sounds. In order to assess the effect of hearing on working memory, we investigated working memory as a cognition skill in children with normal development and cochlear implant.

Methods

Fifty students with normal hearing and 50 students with cochlear implant aged 5–7 years participated in this study. Children educated in the preschool, the first and second grades. Children with normal development were matched based on age, gender, and grade of education with cochlear implant.Two components of working memory including phonological loop and central executive were compared between two groups. Phonological loop assessed by nonword repetition task and forward digit span. To assess central executive component backward digit span was used. The developmental trend was studied in children with normal development and cochlear implant as well. The effect of age at implantation in children with cochlear implants on components of working memory was investigated.

Results

There are significant differences between children with normal development and cochlear implant in all tasks that assess working memory (p < 0.001). The children's age at implantation was negatively correlated with all tasks (p < 0.001). In contrast, duration of usage of cochlear implant set was positively correlated with all tasks (p < 0.001). The comparison of working memory between different grades showed significant differences both in children with normal development and in children with cochlear implant (p < 0.05).

Conclusion

These results implied that children with cochlear implant may experience difficulties in working memory. Therefore, these children have problems in encoding, practicing, and repeating phonological units. The results also suggested working memory develops when the child grows up. In cochlear implant children, with decreasing age at implantation and increasing their experience in perceiving sound, working memory skills improved.     

Long term speech perception after cochlear implant in pediatric patients with GJB2 mutations

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.     

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.     

The effect of age at cochlear implantation outcomes in Saudi children

Objectives

To determine for the effect of age (late versus early age) on the cochlear implant outcomes; in terms of language development, auditory skills, speech perception, and production outcomes).

Methods

67 children were included in the study out of 93 implanted cases in the study period. Children were classified into 2 groups according to age at time of implantation. Group 1 contained 43 children who were implanted before the age of 5 years. Group 2 contained 24 children who were implanted after the age of 5 years. All children were evaluated pre-operatively and at 3, 6, 12, 24 months device experience using the language screening test, Standardized Arabic Language test, Listening Progress Profile (LiP Test), the Monosyllabic-Trochee-polysyllabic Test (MTP), and the meaningful Auditory Integration Scale (MAIS) Test. Charts with incomplete data were excluded.

Results

Only 67 children had complete data out of 93 patients. The mean age (in months) for Group 1 was (43.37 ± 8.63) and for Group 2 was (70.38 ± 9.97) at time of implantation. Significantly higher mean values were detected for Group 2 in comparison to Group 1 in the pre-operative period. No significant difference was detected after 2 years evaluation using the test battery for language development and auditory skills.

Conclusions

Children who were implanted under the 5 years of age had a better outcome in the form of better auditory skills, speech perception, and language production. Limited resources and the absence of a national hearing screening program in Saudi Arabia result in the late presentation of children for evaluation and intervention of hearing problem; this late intervention reduces the benefits the late – implanted children derive from cochlear implantation.