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Otolaryngology--head and Neck Surgery :... Jan 2021Outcomes following cochlear implantation in children with X-linked deafness-2 are variable, resulting in challenges in appropriate preoperative counseling. To address...
OBJECTIVE
Outcomes following cochlear implantation in children with X-linked deafness-2 are variable, resulting in challenges in appropriate preoperative counseling. To address this uncertainty, we performed a systematic review and synthesis of the literature on audiologic and speech outcomes after cochlear implantation in these patients to inform prognostic counseling.
DATA SOURCES
PubMed, Embase, and Cochrane Library were queried for articles published between January 2000 and July 2019.
REVIEW METHODS
We performed a systematic review of all studies published between 2000 and 2019 that reported on (1) children with confirmed X-linked deafness-2 undergoing cochlear implantation and (2) formal assessment of hearing and/or speech capabilities postimplantation.
RESULTS
Our initial database search yielded 313 articles. Fourteen articles met inclusion criteria. These studies reported on 61 children with X-linked deafness-2 who underwent implantation at a wide age range (1-29 years) for severe-profound sensorineural hearing loss of prelingual onset. The mean follow-up duration after implant activation was 32 months (range, 12-61). Outcome domains assessed at follow-up were heterogeneous, though each study employed at least 1 assessment of hearing (eg, pure tone audiometry), speech perception (eg, Early Speech Perception Test), or auditory perception (eg, Categories of Auditory Perception scores). In 10 of 14 studies, cochlear implantation afforded significant improvement in hearing and speech capabilities relative to preoperative performance or as compared with age-matched, normal-hearing controls.
CONCLUSION
The majority of studies demonstrate that cochlear implantation provides improvements in hearing and speech performance in patients with X-linked deafness-2. This information is valuable for decision making regarding cochlear implantation in these patients.
Topics: Auditory Perception; Child; Cochlear Implantation; Cochlear Implants; Deafness; Genetic Diseases, X-Linked; Humans
PubMed: 32600118
DOI: 10.1177/0194599820932138 -
Journal of the European Academy of... Mar 2022The association between vitiligo and hearing loss has been noted but the specific frequencies and degrees of hearing impairment remain unclear. The objective of this... (Meta-Analysis)
Meta-Analysis Review
The association between vitiligo and hearing loss has been noted but the specific frequencies and degrees of hearing impairment remain unclear. The objective of this systematic review was to investigate the relationship between vitiligo and hearing thresholds at various specific frequencies. We followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. We searched MEDLINE and Embase for relevant studies from inception to 10th April 2021. Case-control studies, cross-sectional, or cohort studies that compared the frequency-specific hearing thresholds between vitiligo patients and age-matched non-vitiligo controls were included. There were neither language nor geographic limitations. We used the Newcastle-Ottawa Scale to assess the risk of bias of included studies. The DerSimonian and Laird random-effects model was utilized in meta-analyses due to expected clinical heterogeneity. We included 9 case-control studies with 371 vitiligo patients and 349 controls, which were rated with low or unclear risk. We found neither relevant cross-sectional nor cohort studies. The meta-analysis showed that when compared with controls, vitiligo patients had significantly higher pure-tone hearing thresholds at 2000, 4000, and 8000 Hz. In conclusions, vitiligo patients are prone to high-frequency sensorineural hearing loss.
Topics: Case-Control Studies; Cross-Sectional Studies; Deafness; Hearing Loss, Sensorineural; Humans; Vitiligo
PubMed: 34779053
DOI: 10.1111/jdv.17815 -
Frontiers in Neuroscience 2022Neuroplasticity following deafness has been widely demonstrated in both humans and animals, but the anatomical substrate of these changes is not yet clear in human...
Neuroplasticity following deafness has been widely demonstrated in both humans and animals, but the anatomical substrate of these changes is not yet clear in human brain. However, it is of high importance since hearing loss is a growing problem due to aging population. Moreover, knowing these brain changes could help to understand some disappointing results with cochlear implant, and therefore could improve hearing rehabilitation. A systematic review and a coordinate-based meta-analysis were realized about the morphological brain changes highlighted by MRI in severe to profound hearing loss, congenital and acquired before or after language onset. 25 papers were included in our review, concerning more than 400 deaf subjects, most of them presenting prelingual deafness. The most consistent finding is a volumetric decrease in gray matter around bilateral auditory cortex. This change was confirmed by the coordinate-based meta-analysis which shows three converging clusters in this region. The visual areas of deaf children is also significantly impacted, with a decrease of the volume of both gray and white matters. Finally, deafness is responsible of a gray matter increase within the cerebellum, especially at the right side. These results are largely discussed and compared with those from deaf animal models and blind humans, which demonstrate for example a much more consistent gray matter decrease along their respective primary sensory pathway. In human deafness, a lot of other factors than deafness could interact on the brain plasticity. One of the most important is the use of sign language and its age of acquisition, which induce among others changes within the hand motor region and the visual cortex. But other confounding factors exist which have been too little considered in the current literature, such as the etiology of the hearing impairment, the speech-reading ability, the hearing aid use, the frequent associated vestibular dysfunction or neurocognitive impairment. Another important weakness highlighted by this review concern the lack of papers about postlingual deafness, whereas it represents most of the deaf population. Further studies are needed to better understand these issues, and finally try to improve deafness rehabilitation.
PubMed: 35418829
DOI: 10.3389/fnins.2022.850245 -
The Journal of International Advanced... Dec 2020Summarise outcomes following cochlear implantation (CI) in patients with post-meningitis deafness. Systematic review and narrative synthesis. Databases searched:...
Summarise outcomes following cochlear implantation (CI) in patients with post-meningitis deafness. Systematic review and narrative synthesis. Databases searched: Medline, Pubmed, Embase, Web of Science, Cochrane Collection and ClinicalTrials.gov. No limits placed on language or year of publication. Studies with a minimum of 20 individuals with post-meningitis deafness were included. Review conducted in accordance with the PRISMA statement. Searches identified 906 abstracts and 291 full texts. Of these, 19 studies met the inclusion criteria, reporting outcomes in 610 patients with 650 implants. Audiological outcomes improved across all studies following cochlear implantation. 7 studies demonstrated a statistically significant difference between pre and post-CI outcomes. Patients with no cochlear ossification, full electrode insertion, shorter duration of deafness and no neurological sequelae generally appeared to perform best. A total of 31 minor and 19 major complications were reported, with 15 cases of reimplantation. The methodological quality of the included studies was sufficient, predominantly consisting of cohort studies. 15 studies were OCEBM grade III and 4 studies were OCEBM grade IV. All studies had a minimum of 20 individuals with post-meningitic deafness and used multi-channel cochlear implant devices. Audiological outcomes following cochlear implantation in meningitis are satisfactory, providing functional levels of speech perception and intelligibility. Improvement in hearing is dependent on the amount of cochlear ossification, duration of deafness prior to implantation, electrode insertion depth and presence of neurological sequalae. Cochlear implantation in meningitis patients can be challenging due to the presence of ossification and inaccuracies of pre-operative imaging. Therefore, early and bilateral implantation is recommended in all patients with post-meningitis hearing loss to improve the likelihood of full electrode insertion.
Topics: Adult; Child, Preschool; Cochlear Implantation; Cochlear Implants; Cohort Studies; Deafness; Humans; Infant; Infant, Newborn; Meningitis; Prospective Studies; Retrospective Studies; Speech Perception; Treatment Outcome
PubMed: 33136024
DOI: 10.5152/iao.2020.9040 -
Health Technology Assessment... Sep 2009To investigate whether it is clinically effective and cost-effective to provide (i) a unilateral cochlear implant for severely to profoundly deaf people (using or not... (Review)
Review
OBJECTIVES
To investigate whether it is clinically effective and cost-effective to provide (i) a unilateral cochlear implant for severely to profoundly deaf people (using or not using hearing aids), and (ii) a bilateral cochlear implant for severely to profoundly deaf people with a single cochlear implant (unilateral or unilateral plus hearing aid).
DATA SOURCES
Main electronic databases [MEDLINE; EMBASE; Cochrane Database of Systematic Reviews; CENTRAL; NHS EED; DARE; HTA (NHS-CRD); EconLit; National Research Register; and ClinicalTrials.gov] searched in October 2006, updated July 2007.
REVIEW METHODS
A systematic review of the literature was undertaken according to standard methods. A state-transition (Markov) model of the main care pathways deaf people might follow and the main complications and device failures was developed.
RESULTS
The clinical effectiveness review included 33 papers, of which only two were RCTs. They used 62 different outcome measures and overall were of moderate to poor quality. All studies in children comparing one cochlear implant with non-technological support or an acoustic hearing aid reported gains on all outcome measures, some demonstrating greater gain from earlier implantation. The strongest evidence for an advantage from bilateral over unilateral implantation was for understanding speech in noisy conditions (mean improvement 13.2%, p < 0.0001); those receiving their second implant earlier made greater gains. Comparison of bilateral with unilateral cochlear implants plus an acoustic hearing aid was compromised by small sample sizes and poor reporting, but benefits were seen with bilateral implants. Cochlear implants improved children's quality of life, and those who were implanted before attending school were more likely to do well academically and attend mainstream education than those implanted later. In adults, there was a greater benefit from cochlear implants than from non-technological support in terms of speech perception. Increased age at implantation may reduce effectiveness and there is a negative correlation between duration of deafness and effectiveness. Speech perception measures all showed benefits for cochlear implants over acoustic hearing aids [e.g. mean increase in score of 37 points in noisy conditions (p < 0.001) with BKB sentences]; however, prelingually deafened adults benefited less than those postlingually deafened (mean change scores 20% versus 62%). For unilateral versus bilateral implantation, benefits in speech perception were significant in noisy conditions on all measures [e.g. 76% for HINT sentences (p < 0.0001)]. Quality of life measured with generic and disease-specific instruments or by interview mostly showed significant gains or positive trends from using cochlear implants. The Markov model base-case analysis estimated that, for prelingually profoundly deaf children, the incremental cost-effectiveness ratio (ICER) for unilateral implantation compared with no implantation was 13,413 pounds per quality-adjusted life-year (QALY). Assuming the utility gain for bilateral implantation is the same for adults and children, the ICERs for simultaneous and sequential bilateral implantation versus unilateral implantation were 40,410 pounds and 54,098 pounds per QALY respectively. For postlingually sensorineurally profoundly deaf adults, the corresponding ICERs were 14,163 pounds, 49,559 pounds and 60,301 pounds per QALY respectively. Probabilistic threshold analyses suggest that unilateral implants are highly likely to be cost-effective for adults and children at willingness to pay thresholds of 20,000 pounds or 30,000 pounds per QALY. There are likely to be overall additional benefits from bilateral implantation, enabling children and adults to hold conversations more easily in social situations.
CONCLUSIONS
Unilateral cochlear implantation is safe and effective for adults and children and likely to be cost-effective in profoundly deaf adults and profoundly and prelingually deaf children. However, decisions on the cost-effectiveness of bilateral cochlear implants should take into account the high degree of uncertainty within the model regarding the probable utility gain.
Topics: Adolescent; Adult; Aged; Child; Child, Preschool; Cochlear Implantation; Cost-Benefit Analysis; Deafness; Female; Humans; Infant; Male; Middle Aged; Models, Economic; United Kingdom; Young Adult
PubMed: 19799825
DOI: 10.3310/hta13440 -
Audiology & Neuro-otology 2015Unilateral severe-to-profound hearing loss, or single-sided deafness (SSD), impairs listening abilities supported by the use of two ears, including speech perception in... (Meta-Analysis)
Meta-Analysis Review
Unilateral severe-to-profound hearing loss, or single-sided deafness (SSD), impairs listening abilities supported by the use of two ears, including speech perception in background noise and sound localisation. Hearing-assistive devices can aid listening by re-routing sounds from the impaired to the non-impaired ear or by restoring input to the impaired ear. A systematic review of the literature examined the impact of hearing-assistive devices on the health-related quality of life (HRQoL) of adults with SSD as measured using generic and disease-specific instruments. A majority of studies used observational designs, and the quality of the evidence was low to moderate. Only two studies used generic instruments. A mixed-effect meta-analysis of disease-specific measures suggested that hearing-assistive devices have a small-to-medium impact on HRQoL. The Speech, Spatial and Qualities of Hearing Scale and the Health Utilities Index Mark 3 (HUI3) were identified as instruments that are sensitive to device-related changes in disease-specific and generic HRQoL, respectively.
Topics: Cochlear Implantation; Deafness; Health Status; Hearing Aids; Hearing Loss, Unilateral; Humans; Quality of Life
PubMed: 25997991
DOI: 10.1159/000380753 -
Journal of Clinical Medicine Sep 2022Patients with single-sided deafness can experience an ipsilateral disabling tinnitus that has a major impact on individuals' social communication and quality of life.... (Review)
Review
Patients with single-sided deafness can experience an ipsilateral disabling tinnitus that has a major impact on individuals' social communication and quality of life. Cochlear implants appear to be superior to conventional treatments to alleviate tinnitus in single-sided deafness. We conducted a systematic review to evaluate the effectiveness of cochlear implants in single-sided deafness with disabling tinnitus when conventional treatments fail to alleviate tinnitus (PROSPERO ID: CRD42022353292). All published studies in PubMed/MEDLINE and SCOPUS databases until December 2021 were included. A total of 474 records were retrieved, 31 studies were included and were divided into two categories according to whether tinnitus was assessed as a primary complaint or not. In all studies, cochlear implantation, evaluated using subjective validated tools, succeeded in reducing tinnitus significantly. Objective evaluation tools were less likely to be used but showed similar results. A short-(3 months) and long-(up to 72 months) term tinnitus suppression was reported. When the cochlear implant is disactivated, complete residual tinnitus inhibition was reported to persist up to 24 h. The results followed a similar pattern in studies where tinnitus was assesed as a primary complaint or not. In conclusion, the present review confirmed the effectiveness of cochlear implantation in sustainably reducing disabling tinnitus in single-sided deafness patients.
PubMed: 36233532
DOI: 10.3390/jcm11195664 -
Psychonomic Bulletin & Review Oct 2023Previous research suggests Deaf signers may have different short-term and working memory processes compared with hearing nonsigners due to prolonged auditory... (Meta-Analysis)
Meta-Analysis Review
Previous research suggests Deaf signers may have different short-term and working memory processes compared with hearing nonsigners due to prolonged auditory deprivation. The direction and magnitude of these reported differences, however, are variable and dependent on memory modality (e.g., visual, verbal), stimulus type, and research design. These discrepancies have made consensus difficult to reach which, in turn, slows progress in areas such as education, medical decision-making, and cognitive sciences. The present systematic review and meta-analysis included 35 studies (N = 1,701 participants) that examined verbal (n = 15), visuospatial (n = 10), or both verbal and visuospatial (n = 10) serial-memory tasks comparing nonimplanted, Deaf signers to hearing nonsigners across the life span. Multivariate meta-analyses indicated a significant, negative effect of deafness on verbal short-term memory (forward recall), g = -1.33, SE = 0.17, p < .001, 95% CI [-1.68, -0.98], and working memory (backward recall), g = -0.66, SE = 0.11, p < .001, 95% CI [-0.89, -0.45], but no significant effect of deafness on visuospatial short-term memory, g = -0.055, SE = 0.17, p = 0.75, 95% CI [-0.39, 0.28]. Visuospatial working memory was not analyzed due to limited power. Population estimates for verbal and visuospatial short-term memory were moderated by age wherein studies with adults demonstrated a stronger hearing advantage than studies with children/adolescents. Quality estimates indicated most studies were of fair quality, with only 38% of studies involving Deaf authors. Findings are discussed in the context of both Deaf equity and models of serial memory.
Topics: Adult; Adolescent; Child; Humans; Deafness; Hearing; Memory, Short-Term; Mental Recall; Sign Language
PubMed: 37012579
DOI: 10.3758/s13423-023-02282-6 -
Ear and HearingEarly hearing detection and intervention (EHDI) is guided by the 1-3-6 approach: screening by one month, diagnosis by 3 mo, and early intervention (EI) enrollment by 6...
OBJECTIVES
Early hearing detection and intervention (EHDI) is guided by the 1-3-6 approach: screening by one month, diagnosis by 3 mo, and early intervention (EI) enrollment by 6 mo. Although screening rates remain high, successful diagnosis and EI-enrollment lag in comparison. The aim of this systematic review is to critically examine and synthesize the barriers to and facilitators of EHDI that exist for families, as they navigate the journey of congenital hearing loss diagnosis and management in the United States. Understanding barriers across each and all stages is necessary for EHDI stakeholders to develop and test novel approaches which will effectively reduce barriers to early hearing healthcare.
DESIGN
A systematic literature search was completed in May and August 2021 for empirical articles focusing on screening, diagnosis, and EI of children with hearing loss. Two independent reviewers completed title and abstract screening, full-text review, data extraction, and quality assessments with a third independent reviewer establishing consensus at each stage. Data synthesis was completed using the Framework Analysis approach to categorize articles into EHDI journey timepoints and individual/family-level factors versus system-level factors.
RESULTS
Sixty-two studies were included in the narrative synthesis. Results revealed that both individual/family-level (e.g., economic stability, medical status of the infant including middle ear involvement) and system-level barriers (e.g., system-service capacity, provider knowledge, and program quality) hinder timely diagnosis and EI for congenital hearing loss. Specific social determinants of health were noted as barriers to effective EHDI; however, system-level facilitators such as care coordination, colocation of services, and family support programs have been shown to mitigate the negative impact of those sociodemographic factors.
CONCLUSIONS
Many barriers exist for families to obtain appropriate and timely EHDI for their children, but system-level changes could facilitate the process and contribute to long-term outcomes improvement. Limitations of this study include limited generalizability due to the heterogeneity of EHDI programs and an inability to ascertain factor interactions.
Topics: Infant; Infant, Newborn; Child; United States; Humans; Neonatal Screening; Hearing Tests; Hearing Loss; Deafness; Hearing
PubMed: 36579673
DOI: 10.1097/AUD.0000000000001312 -
The Annals of Otology, Rhinology, and... Oct 2018Radiation-associated soft tissue sarcomas of the neck (RASN) constitute a rare and aggressive tumor type. (Meta-Analysis)
Meta-Analysis Review
INTRODUCTION
Radiation-associated soft tissue sarcomas of the neck (RASN) constitute a rare and aggressive tumor type.
METHODS
A retrospective chart review at the authors' institution revealed 3 patients with RASN. A systematic review of the literature was also conducted using MEDLINE, Ovid, the Cochrane Library, and Embase.
RESULTS
Patients within the authors' institutional chart review presented from 6 to 26 years after neck radiation with neck masses. All patients underwent surgical resection with clear margins, and adjuvant radiation was offered when feasible. Patients had no evidence of disease at most recent follow-up. A total of 867 articles were screened for systematic review, revealing 9 articles detailing outcomes of RASN. Studies were small and heterogeneous, precluding pooled data. The importance of complete surgical extirpation was noted.
CONCLUSIONS
Complete surgical resection appears to be the mainstay of therapy, but there are limited data on management and outcomes of patients with RASN.
Topics: Combined Modality Therapy; Head and Neck Neoplasms; Humans; Radiation Injuries; Sarcoma
PubMed: 30047787
DOI: 10.1177/0003489418789966