Functional Polymorphism of MMP9 and BDNF as Potential Biomarker of Auditory Neuroplasticity in Prelingual Deafness Treatment With Cochlear Implantation-A Retrospective Cohort Analysis.
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ABSTRACT:
SUBMITTER: Matusiak M
PROVIDER: S-EPMC8020743 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:Individuals with single-sided deafness (SSD) have no access to binaural hearing, which limits their ability to localize sounds and understand speech in noisy environments. In addition, children with prelingual SSD are at risk for neurocognitive and academic difficulties. Early cochlear implantation may lead to improved hearing outcomes by restoring bilateral hearing. However, its longitudinal impact on the development of children with SSD remains unclear. In the current study, a group of young children with prelingual SSD received a cochlear implant at an early age. From the age of four, the children's spatial hearing skills could be assessed using a spatial speech perception in noise test and a sound localization test. The results are compared to those of two control groups: children with SSD without a cochlear implant and children with bilateral normal hearing. Overall, the implanted group exhibited improved speech perception in noise abilities and better sound localization skills, compared to their non-implanted peers. On average, the children wore their device approximately nine hours a day. Given the large contribution of maturation to the development of spatial hearing skills, further follow-up is important to understand the long-term benefit of a cochlear implant for children with prelingual SSD.
| S-EPMC9174487 | biostudies-literature
Project description:Because of vast variability of cochlear implantation outcomes in prelingual deafness treatment, identification of good and poor performers remains a challenging task. To address this issue, we investigated genetic variants of matrix metalloproteinase 9 (MMP9) and brain-derived neurotrophic factor (BDNF) and plasma levels of MMP-9, BDNF, and pro-BDNF that have all been implicated in neuroplasticity after sensory deprivation in the auditory pathway. We recruited a cohort of prelingually deaf children, all implanted before the age of 2, and carried out a prospective observation (N = 61). Next, we analyzed the association between (i) functional MMP9 (rs20544, rs3918242, rs2234681) and BDNF (rs6265) gene variants (and their respective protein levels) and (ii) the child's auditory development as measured with the LittlEARS Questionnaire (LEAQ) before cochlear implant (CI) activation and at 8 and 18 months post-CI activation. Statistical analyses revealed that the plasma level of MMP-9 measured at implantation in prelingually deaf children was significantly correlated with the LEAQ score 18 months after CI activation. In the subgroup of DFNB1-related deafness (N = 40), rs3918242 of MMP9 was significantly associated with LEAQ score at 18 months after CI activation; also, according to a multiple regression model, the ratio of plasma levels of pro-BDNF/BDNF measured at implantation was a significant predictor of overall LEAQ score at follow-up. In the subgroup with DFNB1-related deafness, who had CI activation after 1 year old (N = 22), a multiple regression model showed that rs3918242 of MMP9 was a significant predictor of overall LEAQ score at follow-up.
| S-EPMC9262127 | biostudies-literature
Project description:ObjectiveHearing loss is a highly disabling condition. Cochlear implantation is an established remedy if conventional hearing aids have failed to alleviate the level of disability. Unfortunately, cochlear implant (CI) performance varies dramatically. This study aims to examine the effects of duration of deafness (DoD) prior to cochlear implantation and the postoperative duration of implant experience with resulting hearing performance in postlingually deaf patients.MethodsA systematic literature review and two meta-analyses were conducted using the search terms cochlear implant AND duration deafness. Included studies evaluate the correlation between the DoD and auditory performance after cochlear implantation using monosyllabic and sentence tests. Correlation coefficients were determined using Pearson's correlation and Spearman rho.ResultsA total of 36 studies were identified and included data on cochlear implantations following postlingual deafness and postoperative speech testing of hearing outcomes for 1802 patients. The mean age ranged from 44 to 68 years with a DoD of 0.1 to 77 years. Cochlear implant use varied from 3 months to 14 years of age. Speech perception, which was assessed by sentence and monosyllabic word perception, was negatively correlated with DoD. Subgroup analyses revealed worse outcomes for longer DoD and shorter postoperative follow-up.ConclusionDoD is one of the most important factors to predict speech perception after cochlear implantation in postlingually deaf patients. The meta-analyses revealed a negative correlation between length of auditory deprivation and postoperative sentence and monosyllabic speech perception. Longer DoD seems to lead to worse CI performance, whereas more experience with CI mitigates the effect.
| S-EPMC8035957 | biostudies-literature
Project description:PurposeThis study investigated neuroplastic changes induced by postlingual single-sided deafness (SSD) and the effects of a cochlear implantation for the deaf ear. Neural processing of acoustic signals from the normal hearing ear to the brain was studied before and after implantation using a positron emission tomography (PET)/CT scanner.MethodsEight patients with postlingual SSD received a cochlear implant (CI) in a prospective clinical trial. Dynamic imaging was performed in a PET/CT scanner using radioactively labeled water ([15O]H2O) to localize changes in the regional cerebral blood flow (rCBF) with and without an auditory task of logatomes containing speech-like elements without meaningful context. The normal hearing ear was stimulated before implantation and after the use of the cochlear implant for at least 8 months (mean 13.5, range 8.1-26.6). Eight age- and gender-matched subjects with normal hearing on both sides served as healthy control subjects (HCS).ResultsWhen the normal hearing ear of SSD patients was stimulated before CI implantation, the [15O]H2O-PET showed a more symmetrical rCBF in the auditory regions of both hemispheres in comparison to the HCS. The use of CI increased the asymmetry index (AI) in six of eight patients indicating an increase of activity of the contralateral hemisphere. Non-parametric statistics revealed a significant difference in the AI between patients before CI implantation and HCS (p < .01), which disappeared after CI implantation (p = .195).ConclusionThe functional neuroimaging data showed a tendency towards normalization of neuronal activity after CI implantation, which supports the effectiveness of CI in SSD patients.Trial registrationClinicalTrials.gov Identifier: NCT01749592, December 13, 2012.
| S-EPMC10907329 | biostudies-literature
Project description:Cochlear implantation is usually not recommended for prelingual profoundly deaf adults, although some of these patients might benefit from it. This study aims to define the candidates for cochlear implantation in this population. This retrospective study reviewed 34 prelingual profoundly deaf patients who had received a cochlear implant at 32 ± 1.7 years old (16-55), with at least 1 year of follow-up. Speech perception and quality of life were assessed before and 3, 6, and 12 months after cochlear implantation, then every year thereafter. According to the word speech intelligibility in quiet (WSI) 1 year after implantation, two groups were identified: good performer (GP) with WSI ≥ 50% (n = 15), and poor performer (PP) with WSI ≤ 40% (n = 19). At the 1 year mark, mean WSI improved by 28 ± 4.6% (-20-100) (p < 0.0001). In GP, the intelligibility for words and sentences, communication and quality of life scales improved. In PP, the communication scale improved, but not auditory performance or quality of life. GP and PP differed pre-operatively in speech production, communication abilities, and WSI in best-aided conditions. In prelingual profoundly deaf adults, a dramatic auditory performance benefit could be expected after cochlear implantation if the patients have some degree of speech intelligibility in aided conditions and have developed oral communication and speech production.
| S-EPMC8999851 | biostudies-literature
Project description:Cochlear implantation has become the standard-of-care for adults and children with severe to profound hearing loss. There is growing evidence that qualitative as well as quantitative deficits in the auditory nerve may affect cochlear implant (CI) outcomes. Auditory neuropathy spectrum disorder (ANSD) is characterized by dysfunctional transmission of sound from the cochlea to the brain due to defective synaptic function or neural conduction. In this review, we examine the precise mechanisms of genetic lesions causing ANSD and the effect of these lesions on CI outcomes. Reviewed data show that individuals with lesions that primarily affect the cochlear sensory system and the synapse, which are bypassed by the CI, have optimal CI outcomes. Individuals with lesions that affect the auditory nerve show poor performance with CIs, likely because neural transmission of the electrical signal from the CI is affected. We put forth a nuanced molecular classification of ANSD that has implications for preoperative counseling for patients with this disorder prior to cochlear implantation. We propose that description of ANSD patients should be based on the molecular site of lesion typically derived from genetic evaluation (synaptopathy vs. neuropathy) as this has implications for expected CI outcomes. Improvements in our understanding of genetic site of lesions and their effects on CI function should lead to better CI outcomes, not just for individuals with auditory neuropathy, but all individuals with hearing loss.
| S-EPMC6703118 | biostudies-literature
Project description:The cortical auditory evoked potential (CAEP)-based P1 component acts as a biomarker for cochlear implantation (CI) outcomes in children with auditory neuropathy spectrum disorder (ANSD). To date, early intervention primarily before the age of two years and six months of CI usage is necessary and sufficient to achieve age-appropriate cortical maturation and good prognosis. However, varying degrees of neural dyssynchrony, resulting from the etiological heterogeneity of ANSD, may preclude uniform application of this hypothesis to ensure auditory cortical maturation. Thus, a focused evaluation of those carrying OTOF variants, which may be the salient molecular etiology of prelingual ANSD, would circumvent the issue of heterogeneity. Here, we sought to provide a much better understanding of the brain perspectives (i.e., P1 maturation) in OTOF-associated ANSD subjects and set the stage for an optimal strategy to enhance language development. We conducted a preliminary study comprising 10 subjects diagnosed with OTOF-related ANSD who underwent CI by a single surgeon and subsequently underwent measurements of the P1 component. We observed that DFNB9 subjects who received CI after 2 years of age exhibited "absent" or "anomalous" P1 components that correspond to delayed language development. However, timely implantation, as early as 12 months of age per se, might be insufficient to achieve age-appropriate cortical maturation of DFNB9 in cases with six to seven months of device use. This suggests the importance of sustained rehabilitation in DFNB9 than in other etiologies. Indeed, an additional follow-up study showed that a reduction in P1 latency was linked to an improvement in auditory performance. Collectively, our results suggest that central auditory maturation and successful outcome of CI in DFNB9 may have more demanding requirements, that is, earlier implantation and more sustained rehabilitation. We believe that the current study opens a new path toward genome-based neuroimaging in the field of hearing research.
| S-EPMC8183996 | biostudies-literature
Project description:PurposeThis current study aimed to explore early prelingual auditory development (EPLAD) and early language development in Mandarin-speaking children who received simultaneous bilateral cochlear implants (BICI) during the first year of cochlear implantation and compare the performance of the children who received BICI with those received unilateral cochlear implant (UCI).Methods39 Mandarin-speaking children who received BICIs simultaneously and 36 children with UCIs were enrolled in this study. To access the EPLAD, the Infant-Toddler Meaningful Auditory Integration Scale (IT/MAIS) was conducted, and a subtest of the simplified short-form version of the Mandarin Communicative Development Inventory (SSF-MCDI) was used to evaluate the development of expressive and receptive vocabulary for the children at indicated time points after surgery.ResultsIn both the simultaneous BICI and UCI groups, we observed significantly increased scores of the SSF-MCDI and IT/MAIS 1 year after the surgery. There are indications of early advantages in children with BICI in IT/MAIS scores (at 1, 3, and 6 months after activation). For early development of language, a great difference between the expressive vocabulary scores and the receptive vocabulary scores was observed in both groups. We found there were not significant differences between the two groups on expressive or receptive vocabulary scores, the use of more differentiated measures might be required in future research. We further found that the development of the receptive or expressive vocabulary is dramatically correlated with the age at implantation and the total scores of IT/MAIS for children with simultaneous BICIs.ConclusionThese results may supplement the skills development of early language and auditory in Mandarin-native children with simultaneous CIs. It is obvious that children with normal hearing have mastery of receptive vocabulary before that of expressive vocabulary, which is the same as children with unilateral and bilateral CIs in this research. IT/MAIS total scores and age at CI were important factors for early language performance in children with simultaneous BICIs.
| S-EPMC9669896 | biostudies-literature
Project description:BackgroundAdults with single sided deafness (SSD) have lost binaural function, which limits sound source localization, speech understanding in noise, and quality of life. For SSD patients, restoration of bilateral auditory input is possible only with a cochlear implant (CI). In this study, cortical auditory evoked potentials (CAEPs) and behavioral performance were measured in left-implanted (SSD-CI-L) and right-implanted (SSD-CI-R) patients before and after cochlear implantation. We hypothesized that improvements in behavioral performance would be accompanied by changes in CAEPs after cochlear implantation.DesignProspective longitudinal study.SettingTertiary referral center.MethodNine right-handed adult SSD CI patients participated in the study. CAEPs were recorded before cochlear implantation and at 6 and 12 months post-implantation. CAEPs were elicited using speech stimuli (/ba/) delivered in sound field at 70 dBA. Global field power (GFP) latency and amplitude were calculated for P1, N1 and P2 peaks at each test session. CAEP were analyzed at frontocentral (Cz) and temporal (P7, P8, T7 and T8) and mastoid electrodes (M1 and M2) contralateral to the CI ear. Behavioral measures (sentence recognition in noise, with and without spatial cues) were collected at the same test sessions as for CAEPs. Speech performance and CAEPs were also measured in a control group of normal-hearing (NH) subjects.ResultsWhile increased N1 amplitude was observed in the scalp potential maps for GFP and Cz for SSD-CI-L patients after implantation, the changes were not statistically significant. Peak CAEP amplitude at electrodes to contralateral to the CI ear increased after cochlear implantation for all SSD-CI patients, but significant increases were observed only for mastoid sites. Peak latencies for some components at temporal and mastoid sites remained significantly longer than for the NH control group, even after cochlear implantation. For SSD-CI-R patients, P2 peak amplitude for baseline GFP and Cz was significantly lower than for the NH control group. A significant improvement for speech understanding in noise was observed at 12 months post-implantation when speech was presented to the CI ear and noise to the non-implanted ear.ConclusionAfter cochlear implantation, speech understanding significantly improved when speech and noise were spatially separated. The increased N1 amplitude for SSD-CI-L patients and the increased bilateral activation for all SSD-CI patients may reflect cortical reorganization and restoration of binaural function after one year of experience with the CI. However, because of the limited number of SSD patients, significant changes in cortical activity after cochlear implantation were often difficult to observe.
| S-EPMC6152998 | biostudies-literature
Project description:ObjectivesTo evaluate the long-term audiometric outcomes, sound localization abilities, binaural benefits, and tinnitus assessment of subjects with cochlear implant (CI) after a diagnosis of unilateral severe-to-profound hearing loss.MethodThe study group consisted of 60 (mean age 52 years, range 19-84) subjects with profound hearing loss in one ear and normal to near-normal hearing in the other ear who underwent CI. Data analysis included pre- and postoperative Consonant-Nucleus-Consonant (CNC) Word scores, AzBio Sentence scores, pure tone thresholds, sound localization, and Iowa Tinnitus Handicap Questionnaire scores.ResultsPreoperative average duration of deafness was 3.69 years (standard deviation 4.31), with an average follow-up time of 37.9 months (range 1-87). CNC and AzBio scores significantly improved (both P < 0.001) postoperatively among the entire cohort, and there was much heterogeneity in outcomes with respect to deafness etiology subgroup analysis. Sound localization abilities tended to improve longitudinally in the entire cohort. Binaural benefits using an adaptive Hearing in Noise Test test showed a significant (P < 0.001) improvement with head shadow effect. Utilizing the Iowa Tinnitus Handicap Questionnaire, there was significant improvement in social, physical, and emotional well-being (P = 0.011), along with hearing abilities (P = 0.001).ConclusionsThis case series is the largest cohort of CI SSD subjects to date and systematically analyzes their functional outcomes. Subjects have meaningful improvement in word understanding, and sound localization tends to gradually improve over time. Binaural benefit analysis showed significant improvement with head shadow effect, which likely provides ease of listening.Level of evidence4 Laryngoscope, 130:1805-1811, 2020.
| S-EPMC8588258 | biostudies-literature