Appropriate Imaging Modality for the Etiologic Diagnosis of Congenital Single-Sided Deafness in Children.
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SUBMITTER: Lee SY
PROVIDER: S-EPMC6306910 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:ObjectiveTo describe and study the population statistics, hearing phenotype, and pathological changes of a porcine congenital single-sided deafness (CSSD) pedigree.MethodsClick auditory brainstem response (ABR), full-frequency ABR, and distortion product otoacoustic emission (DPOAE) were used to assess the hearing phenotype of the strain. Tympanogram was used to assess the middle ear function since birth. Celloidin embedding-hematoxylin-eosin (CE-HE) stain and scanning electron microscopy (SEM) were used to study the pathological changes of cochlear microstructures. Chi-square analysis was used to analyze the relation between hearing loss and other phenotypes.ResultsThe mating mood of CSSD with CSSD was most efficient in breeding-targeted CSSD phenotype (47.62%), and the prevalence of CSSD reached 46.67% till the fifth generation, where 42.22% were bilateral hearing loss (BHL) and 9.00% were normal hearing (NH) individuals. Hearing loss was proved to have no relation with coat color (P = 0.0841 > 0.05) and gender (P = 0.4621 > 0.05) by chi-square analysis. The deaf side of CSSD offspring in the fifth generation had no relation with that of their maternal parent (P = 0.2387 > 0.05). All individuals in this strain exhibited congenital severe to profound sensorineural hearing loss with no malformation and dysfunction of the middle ear. The good hearing ear of CSSD stayed stable over age. The deaf side of CSSD and BHL presented cochlear and saccular degeneration, and the hair cell exhibited malformation since birth and degenerated from the apex to base turn through time. The pathology in BHL cochlea progressed more rapidly than CSSD and till P30, the hair cell had been totally gone. The stria vascularis (SV) was normal since birth and degenerated through time and finally exhibited disorganization of three layers of cells.ConclusionThis inbred porcine strain exhibited high and stable prevalence of CSSD, which highly resembled human non-syndromic CSSD disease. This porcine model could be used to further explore the etiology of CSSD and serve as an ideal tool for the studies of the effects of single-sided hearing deprivation on neural, cognitive, and behavioral developments and the benefits brought by CI in CSSD individuals.
| S-EPMC8226144 | biostudies-literature
Project description:ImportanceIn 2019, the US Food and Drug Administration approved cochlear implantation for children with single-sided deafness (SSD). The absence of robust clinical data specific to pediatric patients to guide shared decision-making and to identify potential advantages is a challenge in family counseling.ObjectiveTo evaluate the audiological and patient-reported outcomes in children who underwent cochlear implantation for SSD and to assess the association between time of implantation, subjective outcomes, and cochlear implant device use rates.Data sourceMEDLINE, Embase, Scopus, Cochrane, and PubMed were searched for English-language articles that were published in a peer-reviewed journal from database inception to February 18, 2020.Study selectionInclusion criteria were designed to capture studies that evaluated pediatric patients (1) younger than 18 years, (2) with a diagnosis of SSD for which they underwent a cochlear implantation, and (3) with at least 1 outcome of interest measured numerically: speech perception, sound localization, device use, and patient-reported outcomes. Of the 526 articles reviewed, 12 (2.3%) met the selection criteria.Data extraction and synthesisThe Meta-analyses Of Observational Studies in Epidemiology (MOOSE) reporting guidelines were followed. Data were pooled using fixed-effect and random-effect models. The following information was obtained from each article: study characteristics, patient characteristics, hearing loss and intervention characteristics, and outcomes.Main outcomes and measuresOutcomes were (1) postoperative changes in speech perception (in quiet was measured as a proportion of correct responses, and in noise was measured as decibel signal to noise ratio for speech reception threshold) and sound localization (measured in degree of localization error), (2) patient-reported audiological outcomes (measured by the speech, spatial, and qualities of hearing scale), and (3) device use rates among children who received cochlear implantation for SSD.ResultsTwelve observational studies that evaluated 119 children (mean [SD] age, 6.6 [4.0] years) with SSD who received a cochlear implant were included. Most children showed clinically meaningful improvement in speech perception in noise (39 of 49 children [79.6%]) and in quiet (34 of 42 children [81.0%]). Long duration of deafness (>4 years in congenital SSD and >7 years in perilingual SSD) was the most commonly proposed reason for lack of improvement. Sound localization as measured by degrees of error from true location (mean difference [MD], -24.78°; 95% CI, -34.16° to -15.40°; I2 = 10%) improved statistically significantly after cochlear implantation. Patients with acquired SSD and shorter duration of deafness compared with those with congenital SSD reported greater improvements in speech (MD, 2.27; 95% CI, 1.89-2.65 vs 1.58; 95% CI, 1.00-2.16) and spatial (MD, 2.95; 95% CI, 2.66-3.24 vs 1.68; 95% CI, 0.96-2.39) hearing qualities. The duration of deafness among device nonusers was statistically significantly longer than the duration of deafness among regular device users (median difference, 6.84; 95% CI, 4.02-9.58).Conclusions and relevanceThis systematic review and meta-analysis found that cochlear implantation for children with SSD was associated with clinically meaningful improvements in audiological and patient-reported outcomes; shorter duration of deafness may lead to better outcomes. These findings can guide future research efforts, refine cochlear implantation candidacy criteria, and aid in family counseling and shared decision-making.
| S-EPMC7645748 | biostudies-literature
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: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
Project description:The goals of the current study were to evaluate audibility and cortical speech processing, and to provide insight into binaural processing in children with single-sided deafness (CHwSSD) using a cochlear implant (CI). The P1 potential to acoustically-presented speech stimuli (/m/, /g/, /t/) was recorded during monaural [Normal hearing (NH), CI], and bilateral (BIL, NH + CI) listening conditions within a clinical setting in 22 CHwSSD (mean age at CI/testing 4.7, 5.7 years). Robust P1 potentials were elicited in all children in the NH and BIL conditions. In the CI condition: (1) P1 prevalence was reduced yet was elicited in all but one child to at least one stimulus; (2) P1 latency was prolonged and amplitude was reduced, consequently leading to absence of binaural processing manifestations; (3) Correlation between P1 latency and age at CI/testing was weak and not significant; (4) P1 prevalence for /m/ was reduced and associated with CI manufacturer and duration of CI use. Results indicate that recording CAEPs to speech stimuli in clinical settings is feasible and valuable for the management of CHwSSD. While CAEPs provided evidence for effective audibility, a substantial mismatch in timing and synchrony of early-stage cortical processing between the CI and NH ear remains a barrier for the development of binaural interaction components.
| S-EPMC9981742 | biostudies-literature
Project description:BackgroundSingle-sided deafness (SSD) describes the presence of a unilateral severe to profound sensorineural hearing loss. SSD disrupts spatial hearing and understanding speech in background noise. It has functional, psychological and social consequences. Potential options for rehabilitation include hearing aids and auditory implants. Benefits and harms of these interventions are documented inconsistently in the literature, using a variety of outcomes ranging from tests of speech perception to quality of life questionnaires. It is therefore difficult to compare interventions when rehabilitating SSD. The Core Rehabilitation Outcome Set for Single Sided Deafness (CROSSSD) study is an international initiative that aims to develop a minimum set of core outcomes for use in future trials of SSD interventions.Methods/designThe CROSSSD study adopts an international two-round online modified Delphi survey followed by a stakeholder consensus meeting to identify a patient-centred core outcome domain set for SSD based on what is considered critical and important for assessing whether an intervention for SSD has worked.DiscussionThe resulting core outcome domain set will act as a minimum standard for reporting in future clinical trials and could have further applications in guiding the use of outcome measures in clinical practice. Standardisation will facilitate comparison of research findings.
| S-EPMC7057560 | 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:While cochlear implantation has benefitted many patients with single-sided deafness (SSD), there is great variability in cochlear implant (CI) outcomes and binaural performance remains poorer than that of normal-hearing (NH) listeners. Differences in sound quality across ears-temporal fine structure (TFS) information with acoustic hearing vs. coarse spectro-temporal envelope information with electric hearing-may limit integration of acoustic and electric patterns. Binaural performance may also be limited by inter-aural mismatch between the acoustic input frequency and the place of stimulation in the cochlea. SSD CI patients must learn to accommodate these differences between acoustic and electric stimulation to maximize binaural performance. It is possible that training may increase and/or accelerate accommodation and further improve binaural performance. In this study, we evaluated lateralization training in NH subjects listening to broad simulations of SSD CI signal processing. A 16-channel vocoder was used to simulate the coarse spectro-temporal cues available with electric hearing; the degree of inter-aural mismatch was varied by adjusting the simulated insertion depth (SID) to be 25 mm (SID25), 22 mm (SID22) and 19 mm (SID19) from the base of the cochlea. Lateralization was measured using headphones and head-related transfer functions (HRTFs). Baseline lateralization was measured for unprocessed speech (UN) delivered to the left ear to simulate SSD and for binaural performance with the acoustic ear combined with the 16-channel vocoders (UN+SID25, UN+SID22 and UN+SID19). After completing baseline measurements, subjects completed six lateralization training exercises with the UN+SID22 condition, after which performance was re-measured for all baseline conditions. Post-training performance was significantly better than baseline for all conditions (p < 0.05 in all cases), with no significant difference in training benefits among conditions. Given that there was no significant difference between the SSD and the SSD CI conditions before or after training, the results suggest that NH listeners were unable to integrate TFS and coarse spectro-temporal cues across ears for lateralization, and that inter-aural mismatch played a secondary role at best. While lateralization training may benefit SSD CI patients, the training may largely improve spectral analysis with the acoustic ear alone, rather than improve integration of acoustic and electric hearing.
| S-EPMC6056606 | biostudies-literature
Project description:ObjectiveTo evaluate the audiological and subjective benefit from hearing rehabilitation with an active bone conduction implant in subjects with single-sided sensorineural deafness (SSD).Study designProspective, multicenter, single-subject repeated measures.SettingTertiary referral center, five clinics in Germany and Switzerland.PatientsSeventeen subjects aged 18 years and older with severe to profound unilateral sensorineural hearing loss and contralateral normal hearing were followed up for 24 months.InterventionActive bone conduction implant.Main outcome measuresSpeech understanding in noise was assessed in three situations: with signal from front, deaf, or normal hearing side (with noise from front in all set-ups). Subjective benefit was evaluated using the Speech, Spatial, and Qualities of Hearing (SSQ-B) and Bern Benefit in Single-Sided Deafness (BBSS) questionnaire.ResultsWhen the signal was coming from the deaf side the mean improvement of the speech reception threshold in noise ranged from 1.5 up to 2.2 dB with the device and was statistically and clinically significant at all tested timepoints. No significant difference between the aided and unaided situation was found when signal and noise were coming from the front. With the signal from the normal hearing side no clinically significant difference, that is, greater than 1 dB between the aided and unaided situation was found. The SSQ-B and BBSS questionnaire showed an overall improvement with no significant difference between time points.ConclusionsThe study demonstrates long-term efficacy and benefit of the device in adults with SSD. Patients reported substantial and persistent subjective benefit from the active bone conduction implant.
| S-EPMC8746904 | biostudies-literature