Appropriate Imaging Modality for the Etiologic Diagnosis of Congenital Single-Sided Deafness in Children.
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ABSTRACT:
SUBMITTER: Lee SY
PROVIDER: S-EPMC6306910 | biostudies-literature | 2018 Dec
REPOSITORIES: biostudies-literature
Publications
Appropriate Imaging Modality for the Etiologic Diagnosis of Congenital Single-Sided Deafness in Children.
Lee Sang-Yeon SY Kim Shin Hye SH Bae Yun Jung YJ Kim Eun Hee EH Koo Ja-Won JW Choi Byung Yoon BY
Journal of clinical medicine 20181204 12
We aimed to compare the diagnostic yield between temporal bone computed tomography (TBCT) and internal auditory canal MRI (IAC MRI) for the etiologic diagnosis of children with congenital single-sided deafness (SSD) and the evaluation of cochlear implant (CI) candidacy. In the original cohort, 24 subjects with congenital SSD were enrolled and underwent both TBCT and IAC MRI. We recruited an additional 22 consecutive infants with congenital SSD (the supplementary cohort) and evaluated in particul ...[more]
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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:Prelingual single-sided deafness (SSD) not only affects children's hearing skills, but can also lead to speech-language delays and academic underachievement. Early cochlear implantation leads to improved spatial hearing, but the impact on language development is less studied. In our longitudinal study, we assessed the language skills of young children with SSD and a cochlear implant (CI). In particular, we investigated their narrative skills in comparison to two control groups: children with SSD without a CI, and children with bilateral normal hearing. We found that children with SSD and a CI performed in line with their normal-hearing peers with regard to narrative and verbal short-term memory skills. Children with SSD without a CI had worse narrative (group difference = - 0.67, p = 0.02) and verbal short-term memory (group difference = - 0.68, p = 0.03) scores than the implanted group. Verbal short-term memory scores and grammar scores each correlated positively with narrative scores across all groups. Early grammar scores (at 2-3 years of age) could partially predict later narrative scores (at 4-6 years of age). These results show that young children with prelingual SSD can benefit from early cochlear implantation to achieve age-appropriate language skills. They support the provision of a CI to children with prelingual SSD.
| S-EPMC10587124 | 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:Objectives/hypothesisThe aim of this study was to obtain a reliable estimate of single-sided deafness (SSD) prevalence in the adult U.S.PopulationMethodsA cross-sectional national epidemiologic study was performed. Participants were included from the National Health and Nutrition Examination Survey (NHANES). Each cohort includes a nationally representative sample of approximately 5,000 noninstitutionalized civilians. Subjects 20 years old and over with audiometric testing were included. SSD was defined as normal hearing (pure-tone average [PTA] of ≤25 dB) in one ear and severe or worse hearing (PTA > 70 dB) in the other, using both three- and four-frequency PTA definition. Prevalence was measured as a raw number (n) and percentage (%) of the sample. Weighted estimates of prevalence were calculated based on the 2019 U.S. population census.ResultsAn estimated 345,064 Americans (estimated prevalence of 0.14%, 95% confidence interval = 0.08-0.24) had SSD. SSD was more prevalent in individuals 60 to 79 years of age (estimated 155,917 U.S. adults, prevalence of 0.25%). A higher prevalence of SSD was noted among women compared to men (215,430 U.S. adult women, prevalence of 0.17% vs. 131,726 U.S. adult men, prevalence of 0.11%). Using a three-frequency PTA definition resulted in an estimated prevalence of 0.11%. Finally, 27% of adults with SSD reported having "good" or "excellent" hearing despite their hearing loss.ConclusionsThe prevalence of SSD in the United States is estimated at 0.11%-0.14% (271,122 to 345,064 adults), depending on PTA definition used. These individuals could potentially benefit from auditory rehabilitation, including cochlear implantation.Level of evidence2 Laryngoscope, 132:1652-1656, 2022.
| S-EPMC9085960 | biostudies-literature
Project description:Potentially neuroprotective effects of CI use were studied in 22 children with single sided deafness (SSD). Auditory-evoked EEG confirmed strengthened representation of the intact ear in the ipsilateral auditory cortex at initial CI activation in children with early-onset SSD (n = 15) and late-onset SSD occurring suddenly in later childhood/adolescence (n = 7). In early-onset SSD, representation of the hearing ear decreased with chronic CI experience and expected lateralization to the contralateral auditory cortex from the CI increased with longer daily CI use. In late-onset SSD, abnormally high activity from the intact ear in the ipsilateral cortex reduced, but responses from the deaf ear weakened despite CI use. Results suggest that: (1) cortical reorganization driven by unilateral hearing can occur throughout childhood; (2) chronic and consistent CI use can partially reverse these effects; and (3) CI use may not protect children with late-onset SSD from ongoing deterioration of pathways from the deaf ear.
| S-EPMC7726152 | biostudies-literature
Project description:BackgroundSingle-sided deafness (SSD) has functional, psychological, and social consequences. Interventions for adults with SSD include hearing aids and auditory implants. Benefits and harms (outcome domains) of these interventions are until now reported inconsistently in clinical trials. Inconsistency in reporting outcome measures prevents meaningful comparisons or syntheses of trial results. The Core Rehabilitation Outcome Set for Single-Sided Deafness (CROSSSD) international initiative used structured communication techniques to achieve consensus among healthcare users and professionals working in the field of SSD. The novel contribution is a set of core outcome domains that experts agree are critically important to assess in all clinical trials of SSD interventions.MethodsA long list of candidate outcome domains compiled from a systematic review and published qualitative data, informed the content of a two-round online Delphi survey. Overall, 308 participants from 29 countries were enrolled. Of those, 233 participants completed both rounds of the survey and scored each outcome domain on a 9-point scale. The set of core outcome domains was finalised via a web-based consensus meeting with 12 participants. Votes involved all stakeholder groups, with an approximate 2:1 ratio of professionals to healthcare users participating in the Delphi survey, and a 1:1 ratio participating in the consensus meeting.ResultsThe first round of the survey listed 44 potential outcome domains, organised thematically. A further five outcome domains were included in Round 2 based on participant feedback. The structured voting at round 2 identified 17 candidate outcome domains which were voted on at the consensus meeting. Consensus was reached for a core outcome domain set including three outcome domains: spatial orientation, group conversations in noisy social situations, and impact on social situations. Seventy-seven percent of the remaining Delphi participants agreed with this core outcome domain set.ConclusionsAdoption of the internationally agreed core outcome domain set would promote consistent assessment and reporting of outcomes that are meaningful and important to all relevant stakeholders. This consistency will in turn enable comparison of outcomes reported across clinical trials comparing SSD interventions in adults and reduce research waste. Further research will determine how those outcome domains should best be measured.
| S-EPMC9454406 | 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