Analysis of narrative ability in children with cochlear implants.

A cochlear implant (CI) is a hearing device introduced in the 1980s for profoundly deaf subjects who gained little or no benefit from powerful hearing aids. This device comprises an electrode array inserted in the cochlea, connected to an internal receiver, and an externally worn speech processor. The CI transforms acoustic signals into electrical currents which directly stimulate the auditory nerve. Since the early 1990s, cochlear implantation in children has been developing rapidly. Although it is still difficult to predict how a child will perform with a cochlear implant, the success of cochlear implantation can no longer be denied. In this paper, some recent papers and reports, and the results of the various Nijmegen cochlear implant studies, are reviewed. Issues about selection, examinations, surgery and the outcome are discussed. Overall, our results were comparable with those of other authors. It can be concluded that cochlear implantation is an effective treatment for postlingually deaf as well as prelingually (congenital or acquired) deaf children with profound bilateral sensorineural deafness.

Cochlear implants (CIs) give children with severe to profound hearing loss access to sound. There appears to be a dose effect of sound exposure on speech perception abilities as shown by the positive influence of early implantation and CI experience. The consistency in device use per day could also affect sound dose, potentially affecting perceptual abilities in children with CIs. The objectives of the present study were to identify the impact of consistency in device use on: (1) speech perception abilities and (2) asymmetry in speech perception abilities between bilateral CIs. Retrospective analysis. To achieve the first objective, data from 65 children (age range at speech test: 1.91-18.05 yrs) with one (unilaterally implanted or bimodal) or two CIs (sequentially or simultaneously implanted) were included. A subset of data from 40 children with bilateral CIs was included to achieve the second objective. Of the 40 children with two CIs, 15 received their CIs sequentially. Device use information was extracted from datalogs stored in personal speech processors using custom software. Speech perception scores per CI collected in quiet were also evaluated. Multiple regression was used to assess the impact of daily CI use, while controlling for factors previously identified to affect speech perception: age at speech test, length of pre-CI (acoustic) hearing experience, length of CI hearing experience, and order of CI for the first objective, and CI category (simultaneous/sequential implantation), interimplant delay, and length of CI experience for the second objective. On average, children wore their CIs for 11.59 ± 2.86 hours/day and, with one CI, exhibited 65.07 ± 22.64% accuracy on speech perception tests. Higher monaural speech perception scores were associated with longer everyday CI use and CI experience (p < 0.05). Among children with bilateral CIs, those with simultaneously implanted CIs and similar bilateral hearing experience demonstrated a small but significant right ear advantage with higher speech perception scores when using the right rather than left CI (mean difference = 4.55 ± 9.83%). The asymmetry in speech perception between CIs was larger and more variable in children who received their CIs sequentially (mean difference CI1-CI2 = 27.48 ± 24.87%). These asymmetries decreased with longer/consistent everyday use of the newer CI (p < 0.05). Yet, despite consistent everyday device use of the second CI (>12 hours/day), only a small proportion of children implanted sequentially (one out of seven children) achieved symmetrical function similar to children with simultaneously received bilateral CIs. Consistent everyday CI use contributes to higher speech perception scores. Although consistent CI use can help reduce the asymmetry in speech perception abilities of children with sequentially implanted CIs subsequent to interimplant delay, residual asymmetry often persists.

Speech perception plays a significant role in the development of speech and language ability in children with hearing loss. There are various speech perception tests with the specific protocol to administer and score. Auditory evoked potential is an objective measure used for assessing hearing acuity in young children. Higher cortical evoked potential test provides an effective indication of the physiological status of auditory cortex. CEAP can be obtained in young children with assistive devices. The present research study aims to investigate whether latency and amplitude of aided cortical evoked potential can predict Speech perception score in cochlear implant users. 52 cochlear implant users and 102 typical children were tested. The mean age for children using cochlear implant was 8.05 years, standard deviation 1.4. The normal hearing group had a mean age of 8.12 years and standard deviation 1.46. Cochlear implant users were having at least 15 active electrodes. Speech stimulus was used with 100 ms duration, 20 ms rise/fall, 20 ms plateau tones. The inter-stimulus interval was kept at 1125 ms. During statistical analysis of data, CEAP P1 latencies and speech perception scores were found to be related. A further regression equation was obtained. The present study reveals speech stimuli can evoke distinct neural response patterns from auditory cortex. The present research study also helps in understanding the neural processing of speech in individuals with hearing impairment using Cochlear implant. Statistical analysis of data CEAP P1 latency and speech perception score regression equation were obtained. The result reveals that speech stimuli can evoke distinct neural response patterns from auditory cortex. CEAP finding support speech perception ability and auditory evoked potential help to the study of neural processing of speech in individuals with the cochlear implant. The evoked potential and speech perception ability has a significant relation between them.

To evaluate the results of late cochlear implantation in prelingually deaf patients with significant residual hearing loss and to evaluate patient factors relevant to postoperative auditory outcomes in this patient group. Analysis of results of cochlear implantation using postoperative speech perception test scores per each condition. Tertiary referral center. Thirty-two subjects with severe to profound hearing loss that developed before the age of 4. Subjects were implanted at a mean age of 24.8 years (range, 16-44) with Nucleus CI24 (n = 18, 56%), Clarion HiRes 90K (n = 11, 34%), and Medel PULSA (n = 3, 10%) device. Details of etiology, duration of deafness, hearing aid history, hearing thresholds before operation, communication mode, and educational environment were investigated. Speech perception tests were performed preoperatively and 12 months after the operation. Postoperative speech perception test scores between different options within patient group. : The results showed significant improvement in open set speech perception (sentence) scores after the implantation (mean scores from 7.0 to 46.7, p < 0.05). Preoperative hearing of better ear and preoperative speech perception scores correlated with postoperative performances (r = -0.70 and r = 0.46, respectively, p < 0.05). Education and communication mode were also closely related to postoperative performances. In the group with poorer performances, preoperative hearing thresholds were significantly worse than those with better performances, and a larger portion of those patients attended special schools and used sign language. We found that residual auditory capacity in the better ear is an important factor in predicting outcomes after cochlear implantation in patients with prelingual hearing loss.

Cochlear Implantation in Adults With Single-Sided Deafness: A Systematic Review and Meta-analysis.

In February 2020, Advanced Bionics initiated a field action notice to remove the MS Ultra (V1) implant from circulation. In this study, we quantify a single site's incidence with device failure and examine the relationship between impedance change and declining speech perception. Retrospective/cohort study. Tertiary healthcare center. Forty-nine adult patients (52 devices) were implanted between October 2017 and December 2019, with the following exclusion criteria: <18 years of age, medical/surgical failures, and lack of English-language proficiency. Diagnostic. Postoperative speech perception (AzBio sentence test) scores at 12 months postactivation were compared with repeat testing at 6- to 12-month intervals. Degree of change in impedances from 1 month postactivation was analyzed at similar intervals. Device failures were suspected when impedance levels on three or more electrodes dropped to <3.5 kΩ and AzBio scores declined >15%. Device failures were confirmed through analyses completed by the manufacturer. To date, 21% (11/52) of devices have met all three criteria for a confirmed failure with an average decline in speech perception of 27.7 ± 22%. The average length of time for detection of device failure was 21.9 ± 5 months postimplantation. An additional 7 devices (13%) are currently being monitored for failure but have not yet met full criteria. There was no predictive relationship between degree of impedance change and speech perception scores. Electrodes along the entire array (channels 1-16) can be affected. Impedance values can be used as a reliable indicator of device malfunction, thus facilitating patient counseling and early intervention. No correlation was identified between impedance changes and speech perception scores.

Background and Objectives：In this study, we evaluated the effect of bimodal hearing in the speech perception test and the increasing level of bimodal hearing over cochlear implantation in speech perception score regarding residual hearing. Subjects and Method：Nineteen prelingually deaf patients, who had used bimodal hearing over a period of 8 months, were divided in two groups in accordance to their low frequency residual hearing. The children were tested in open-set speech perception under +10 SNR and +20 SNR. The scores of bimodal hearing and unilateral cochlear implantation were compared by the Wilcoxon signed-rank test. Also, the increased level of speech perception scores of bimodal hearing over cochlear implantation alone were calculated using the formula, [bimodal score (%) - unilateral cochlear implantation (%)], and was compared between groups. Results：In pure tone audiometry under silent condition, the result of bimodal hearing was similar to unilateral cochlear implantation in magnitude in both groups. Under the noisy condition, both groups had better result in bimodal hearing in terms of speech perception. Also, the increased level was higher in bimodal hearing over unilateral cochlear implantation in the better residual hearing group. However, since the sample size was small enough, it is considered that there was no statistical significance. Conclusion：Cochlear implanted patients with residual hearing are expected to get better speech perception in noisy environment with bimodal hearing regardless of the level of residual hearing. (Korean J Otorhinolaryngol-Head Neck Surg 2009;52:29-35)

This is a pilot study that aims (1) to help design a protocol for fitting and optimizing cochlear implants and hearing aids, (2) to help design a test battery that can help monitor children's progress and (3) to assess the benefit of using a cochlear implant with a contralateral hearing aid. Seven children between the ages of seven and 15 years completed the study. None of them had worn a contralateral hearing aid (HA) since cochlear implantation (five to seven years after implantation). The Listening Inventory for Education (LIFE), Life Situation Questionnaire (LSQ), and Client Orientated Scale of Improvement for Children (COSI-C) questionnaires together with subject's feedback were used as subjective measures, and speech perception tests – the City of New York (sentences list) (CUNY) and Bamford-Kowal-Bench (sentences list) (BKB) depending on child's speech perception skills – in quiet and in noise were used as objective measures. The results showed mixed subjective feedback, even though objectively all children improved their speech perception scores when wearing cochlear implants and hearing aids. The COSI-C proved to be the most successful tool to collect feedback from parents. Copyright © 2007 John Wiley & Sons, Ltd.

ABSTRACTThis study aims to compare word spelling outcomes for French-speaking deaf children with a cochlear implant (CI) with hearing children who matched for age, level of education and gender. A picture written naming task controlling for word frequency, word length, and phoneme-to-grapheme predictability was designed to analyze spelling productions. A generalized linear mixed model on the percentage of correct spelling revealed an effect of participant’s reading abilities, but no effect of hearing status. Word frequency and word length, but not phoneme-to-grapheme predictability, contributed to explaining the spelling variance. Deaf children with a CI made significantly less phonologically plausible errors and more phonologically unacceptable errors when compared to their hearing peers. Age at implantation and speech perception scores were related to deaf children’s errors. A good word spelling level can be achieved by deaf children with a CI, who nonetheless use less efficiently the phoneme-to-grapheme strategy than do hearing children.

Cochlear implant (CI) candidacy and postoperative outcomes are assessed using sets of speech perception tests that vary from center to center, limiting comparisons across institutions and time periods. The objective of this study was to determine if scores on one speech perception test could be reliably predicted from scores on another test. Arizona Biomedical (AzBio) Sentence Test, Consonant-Nucleus-Consonant word (CNCw), and Hearing in Noise Test (HINT) scores in quiet for the implanted ear were collected for individuals who received a CI between 1985 and 2019. Scores collected during the same testing session were analyzed using Bland-Altman plots to assess agreement between testing methods. Simple linear regression with logit transformation was used to generate predictive functions and 95% confidence intervals for expected mean and individual scores. Single academic medical center. A total of 1,437 individuals with a median age of 59.9 years (range, 18-95 yr) and 46% (654 of 1,437) male. N.A. Agreement as a function of test score, mean, variance, and correlation coefficients. A total of 2,052 AzBio/CNCw, 525 AzBio/HINT, and 7,187 CNCw/HINT same-session score pairings were identified. Pairwise test comparisons demonstrated limited agreement between different tests performed in the same session, and a score correlation between different speech tests revealed large variances. Transformation functions between test batteries were predictive of mean scores but performed poorly for prediction of individual scores. Point-wise comparisons of scores across CI test batteries should be used with caution in clinical and research settings.

Mucopolysaccharidosis IVA (OMIM 253000; also known as Morquio A syndrome) is associated with skeletal, airway, and hearing abnormalities. Cochlear implantation is an effective intervention for patients with severe-to-profound hearing loss. Patients can gain substantial improvement in auditory performance, speech perception, and their quality of life from cochlear implantation. Although severe progressive sensorineural hearing loss is a common feature of mucopolysaccharidosis IVA, no detailed description of cochlear implantation for mucopolysaccharidosis IVA has been reported. To review the effectiveness and special considerations associated with cochlear implantation in patients with mucopolysaccharidosis IVA, we here report the case of cochlear implantation in mucopolysaccharidosis IVA by a multidisciplinary team. A retrospective chart review was conducted on a 34-year-old female with mucopolysaccharidosis IVA, who received a cochlear implant. Audiometric thresholds, speech perception scores, and cochlear implant processor mapping information were reviewed during the first 12 months following cochlear implantation. The results of audiological tests indicate improved hearing thresholds as well as remarkable enhancement of speech perception skills over 12 months of cochlear implant use. Cochlear implantation improved auditory performance in a mucopolysaccharidosis IVA patient with postlingually severe-to-profound sensorineural hearing loss. The benefits of cochlear implantation could be meaningful for other Morquio patients with progressive hearing loss, although the risks of surgery and anesthesia should be carefully considered by a multidisciplinary team of experts during the cochlear implant candidacy process.

To determine whether significant differences in cochlear implant (CI) performance exist between older and younger CI recipients. Retrospective comparison of audiometric data. University hospital center. Sixty participants who received CIs at age 70 and older and 48 younger adults implanted between age 18 and 69. Speech outcome scores (Consonant-Nucleus-Consonant words and phonemes and City University of New York Sentence test in quiet and noise). Older participants show significant improvement in speech perception scores after implantation. Although on average they do not perform as well as younger adults, this difference correlates with duration of deafness rather than age. In contrast to younger adults, significant differences in CI performance are seen in older recipients depending on the side of implantation, with those implanted on the right performing better than those implanted on the left. Postlingually deafened older adults obtained significant speech perception benefits from CIs, although differences in speech outcomes were seen between younger recipients and those implanted after age 70. In older adults considering CIs, hearing benefits appear greater if they are implanted in the right ear.

The aim of this study was to investigate 1) whether a hearing aid needs to be adjusted differently depending on whether a child wears a cochlear implant or another hearing aid in the contralateral ear; 2) whether the use of a hearing aid and a cochlear implant in opposite ears leads to binaural interference; and 3) whether the use of a hearing aid and a cochlear implant in opposite ears leads to binaural benefits in speech perception, localization, and communicative functioning in real life. Sixteen children participated in this study. All children used a Nucleus 22 or Nucleus 24 cochlear implant system programmed with the SPEAK strategy in one ear. The hearing aid amplification requirements in the nonimplanted ear of these children were determined using two procedures. A paired comparison technique was used to identify the frequency response that was best for speech intelligibility in quiet, and a loudness balancing technique was used to match the loudness of speech in the ear with a hearing aid to that with a cochlear implant. Eleven of the 16 children participated in the investigation of binaural effects. Performance in speech perception, localization, and communicative functioning was assessed under four aided conditions: cochlear implant with hearing aid as worn, cochlear implant alone, hearing aid alone, and cochlear implant with hearing aid adjusted according to individual requirements. Fifteen of the 16 children whose amplification requirements were determined preferred a hearing aid frequency response that was within +/-6 dB/octave of the NAL-RP prescription. On average, the children required 6 dB more gain than prescribed to balance the loudness of the implanted ear for a speech signal presented at 65 dB SPL. For all 11 children whose performance was evaluated for investigating binaural effects, there was no indication of significantly poorer performance under bilaterally aided conditions compared with unilaterally aided conditions. On average, there were significant benefits in speech perception, localization, and aural/oral function when the children used cochlear implants with adjusted hearing aids than when they used cochlear implants alone. All individuals showed benefits in at least one of the measures. Hearing aids for children who also use cochlear implants can be selected using the NAL-RP prescription. Adjustment of hearing aid gain to match loudness in the implanted ear can facilitate integration of signals from both ears, leading to better speech perception. Given that there are binaural advantages from using cochlear implants with hearing aids in opposite ears, clinicians should advise parents and other professionals about these potential advantages, and facilitate bilateral amplification by adjusting hearing aids after stable cochlear implant MAPs are established.

Posterior fossa tumours (PFT), among the most common childhood brain tumours, place children at risk of speech, language, and communication difficulties, often described in relation to cerebellar mutism syndrome (CMS) but also seen in children without mutism. Narrative ability refers to the capacity to understand, create, and share stories. Narratives can be analysed at the macrostructural level, capturing structure and content, and the microstructural level, reflecting, for example, morphosyntax and lexicon. Narrative ability has not previously been investigated in children with PFT at both levels, including pre- and postoperative data, or in relation to CMS or dysarthria. The aims were to investigate narrative ability at macrostructural and microstructural levels in children with PFT compared with typically developing (TD) peers, to investigate pre- to postoperative changes at both levels in children with PFT, and to examine relations between macro- and microstructure and the presence of CMS and dysarthria. A story-generation task from the Expression, Reception and Recall of Narrative Instrument (ERRNI) was administered pre- and postoperatively, in the early postoperative phase (1-4 weeks after surgery). Macrostructure was analysed using ideascore, and microstructure using mean length of utterance in words (MLUw), grammaticality (GY), subordination index (SI), lexical diversity (MATTR), and words per minute (WPM). Group comparisons and associations with CMS and dysarthria were analysed using linear regressions, and changes over time with mixed-effects models. Children with PFT scored significantly lower than TD peers on the macrostructural measure ideascore and on several microstructural measures, including MLUw, GY, and WPM, both pre- and postoperatively (all p< 0.05). SI and MATTR did not differ between groups. Difficulties were most pronounced among older children. No significant pre- to postoperative changes were found, although individual variability was evident. The small group of children with CMS did not differ significantly from other children with PFT, while those with postoperative dysarthria showed a decline in both macro- and microstructure (p< 0.05). Children with PFT showed pre- and postoperative difficulties at macro- and microstructural levels, producing narratives with fewer story elements, shorter utterances, and more grammatical errors compared with TD peers. The age-related differences suggest that narrative difficulties become more prominent as language and cognitive demands increase. The findings underline the clinical importance of including narrative tasks encompassing macro- and microstructural aspects, together with motor-speech evaluation, in assessment and follow-up of children treated for PFT to guide appropriate interventions. What is already known on this subject Previous studies have described speech, language and/or communication difficulties following posterior fossa tumour surgery, particularly in association with cerebellar mutism syndrome (CMS). One aspect of language that may be affected is narrative ability, which can be analysed at two levels: the macrostructural level (content) and the microstructural level (form). Still, little is known about narrative ability in children with posterior fossa tumours (PFT), although a small study based on postoperative data has shown microstructural difficulties in children undergoing PFT surgery. What this paper adds to existing knowledge Our study adds new knowledge about language profiles in children with PFT, showing that they have narrative difficulties at macro- as well as at microstructural levels, present both pre- and postoperatively. These difficulties are most evident among older children and show individual variability. We did not find more pronounced difficulties in the small group of children with CMS compared with the other children undergoing PFT surgery. What are the potential or actual clinical implications of this work? The findings underline the importance of including narrative assessment in the evaluation and follow-up of children treated for PFT. Assessing macro- and microstructural aspects together with motor-speech evaluation can help identify children in need of interventions. Regular follow-up assessments are essential, as narrative difficulties may become more apparent as language and cognitive demands increase.

There are individual differences in rehabilitation after cochlear implantation that can be explained by brain plasticity. However, from the perspective of brain networks, the effect of implantation age on brain plasticity is unclear. The present study investigated electroencephalography functional networks in the resting state, including eyes-closed and eyes-open conditions, in 31 children with early cochlear implantation, 24 children with late cochlear implantation, and 29 children with normal hearing. Resting-state functional connectivity was measured with phase lag index, and we investigated the connectivity between the sensory regions for each frequency band. Network topology was examined using minimum spanning tree to obtain the network backbone characteristics. The results showed stronger connectivity between auditory and visual regions but reduced global network efficiency in children with late cochlear implantation in the theta and alpha bands. Significant correlations were observed between functional backbone characteristics and speech perception scores in children with cochlear implantation. Collectively, these results reveal an important effect of implantation age on the extent of brain plasticity from a network perspective and indicate that characteristics of the brain network can reflect the extent of rehabilitation of children with cochlear implantation.