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Trials Mar 2021This systematic review aimed to identify, compare and contrast outcome domains and outcome instruments reported in studies investigating interventions that seek to... (Review)
Review
Systematic review of outcome domains and instruments used in designs of clinical trials for interventions that seek to restore bilateral and binaural hearing in adults with unilateral severe to profound sensorineural hearing loss ('single-sided deafness').
BACKGROUND
This systematic review aimed to identify, compare and contrast outcome domains and outcome instruments reported in studies investigating interventions that seek to restore bilateral (two-sided) and/or binaural (both ears) hearing in adults with single-sided deafness (SSD). Findings can inform the development of evidence-based guidance to facilitate design decisions for confirmatory trials.
METHODS
Records were identified by searching MEDLINE, EMBASE, PubMed, CINAHL, ClinicalTrials.gov, ISRCTN, CENTRAL, WHO ICTRP and the NIHR UK clinical trials gateway. The search included records published from 1946 to March 2020. Included studies were those as follows: (a) recruiting adults aged 18 years or older diagnosed with SSD of average threshold severity worse than 70 dB HL in the worse-hearing ear and normal (or near-normal) hearing in the better-hearing ear, (b) evaluating interventions to restore bilateral and/or binaural hearing and (c) enrolling those adults in a controlled trial, before-and-after study or cross-over study. Studies that fell just short of the participant eligibility criteria were included in a separate sensitivity analysis.
RESULTS
Ninety-six studies were included (72 full inclusion, 24 sensitivity analysis). For fully included studies, 37 exclusively evaluated interventions to re-establish bilateral hearing and 29 exclusively evaluated interventions to restore binaural hearing. Overall, 520 outcome domains were identified (350 primary and 170 secondary). Speech-related outcome domains were the most common (74% of studies), followed by spatial-related domains (60% of studies). A total of 344 unique outcome instruments were reported. Speech-related outcome domains were measured by 73 different instruments and spatial-related domains by 43 different instruments. There was considerable variability in duration of follow-up, ranging from acute (baseline) testing to 10 years after the intervention. The sensitivity analysis identified no additional outcome domains.
CONCLUSIONS
This review identified large variability in the reporting of outcome domains and instruments in studies evaluating the therapeutic benefits and harms of SSD interventions. Reports frequently omitted information on what domains the study intended to assess, and on what instruments were used to measure which domains.
TRIAL REGISTRATION
The systematic review protocol is registered on PROSPERO (International Prospective Register of Systematic Reviews): Registration Number CRD42018084274 . Registered on 13 March 2018, last revised on 7th of May 2019.
Topics: Adult; Cochlear Implantation; Cross-Over Studies; Deafness; Hearing; Hearing Loss, Sensorineural; Humans; Speech Perception
PubMed: 33743802
DOI: 10.1186/s13063-021-05160-5 -
Journal of the American Academy of... 2017The pure-tone audiogram, though fundamental to audiology, presents limitations, especially in the case of central auditory involvement. Advances in auditory neuroscience... (Review)
Review
BACKGROUND
The pure-tone audiogram, though fundamental to audiology, presents limitations, especially in the case of central auditory involvement. Advances in auditory neuroscience underscore the considerably larger role of the central auditory nervous system (CANS) in hearing and related disorders. Given the availability of behavioral audiological tests and electrophysiological procedures that can provide better insights as to the function of the various components of the auditory system, this perspective piece reviews the limitations of the pure-tone audiogram and notes some of the advantages of other tests and procedures used in tandem with the pure-tone threshold measurement.
PURPOSE
To review and synthesize the literature regarding the utility and limitations of the pure-tone audiogram in determining dysfunction of peripheral sensory and neural systems, as well as the CANS, and to identify other tests and procedures that can supplement pure-tone thresholds and provide enhanced diagnostic insight, especially regarding problems of the central auditory system.
RESEARCH DESIGN
A systematic review and synthesis of the literature.
DATA COLLECTION AND ANALYSIS
The authors independently searched and reviewed literature (journal articles, book chapters) pertaining to the limitations of the pure-tone audiogram.
RESULTS
The pure-tone audiogram provides information as to hearing sensitivity across a selected frequency range. Normal or near-normal pure-tone thresholds sometimes are observed despite cochlear damage. There are a surprising number of patients with acoustic neuromas who have essentially normal pure-tone thresholds. In cases of central deafness, depressed pure-tone thresholds may not accurately reflect the status of the peripheral auditory system. Listening difficulties are seen in the presence of normal pure-tone thresholds. Suprathreshold procedures and a variety of other tests can provide information regarding other and often more central functions of the auditory system.
CONCLUSIONS
The audiogram is a primary tool for determining type, degree, and configuration of hearing loss; however, it provides the clinician with information regarding only hearing sensitivity, and no information about central auditory processing or the auditory processing of real-world signals (i.e., speech, music). The pure-tone audiogram offers limited insight into functional hearing and should be viewed only as a test of hearing sensitivity. Given the limitations of the pure-tone audiogram, a brief overview is provided of available behavioral tests and electrophysiological procedures that are sensitive to the function and integrity of the central auditory system, which provide better diagnostic and rehabilitative information to the clinician and patient.
Topics: Adult; Audiometry, Pure-Tone; Auditory Perception; Auditory Threshold; Evoked Potentials, Auditory, Brain Stem; Hearing Loss; Humans
PubMed: 28722648
DOI: 10.3766/jaaa.16061 -
The Laryngoscope Jun 2023To evaluate speech outcomes and facial nerve stimulation (FNS) rates in patients with far advanced otosclerosis (FAO) after cochlear implantation. (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
To evaluate speech outcomes and facial nerve stimulation (FNS) rates in patients with far advanced otosclerosis (FAO) after cochlear implantation.
METHODS
A systematic review was performed using standardized methodology of Medline, EMBASE, PubMed, Cochrane, and Web of Science databases. Studies were included if adults with FAO underwent cochlear implantation. Exclusion criteria included concurrent otologic history (e.g., Meniere's disease, superior canal dehiscence), non-English-speaking implant users, case reports, abstracts, and letters/commentaries. Bias was assessed using the Newcastle-Ottawa Scale for cohort studies and the National Institute of Health Scale for case series. The primary outcome measure was speech discrimination and the secondary outcomes were rates of partial insertion and FNS.
RESULTS
Twenty-seven studies evaluated cochlear implantation in FAO. Due to the heterogeneity of testing methods, statistical pooling of speech discrimination was not feasible, but qualitative synthesis indicated a positive effect of implantation. Pooled rates of FNS were 18% (95% confidence interval, CI 12%-27%) and the rate of partial insertion was 10% (95% CI 7%-15%).
CONCLUSION
Cochlear implantation in FAO demonstrates significant gains in speech discrimination scores with higher rates of FNS and partial insertion. Laryngoscope, 133:1288-1296, 2023.
Topics: Adult; Humans; Cochlear Implantation; Otosclerosis; Retrospective Studies; Meniere Disease; Facial Nerve; Cochlear Implants; Speech Perception; Treatment Outcome
PubMed: 36082830
DOI: 10.1002/lary.30386 -
BMJ Open Apr 2024The objective of this study is to determine the relationship between serum vitamin D level and the risk of developing benign paroxysmal positional vertigo (BPPV)... (Meta-Analysis)
Meta-Analysis
OBJECTIVES
The objective of this study is to determine the relationship between serum vitamin D level and the risk of developing benign paroxysmal positional vertigo (BPPV) incidence and recurrence in countries in the Northern Hemisphere.
DESIGN
Systematic review and meta-analysis.
DATA SOURCES
PubMed, Scopus and Web of Science databases were searched for studies published between January 2000 and February 2023.
ELIGIBILITY CRITERIA FOR SELECTING STUDIES
Participants located in the Northern Hemisphere aged 18 or over with at least one episode of BPPV, serum 25-hydroxyvitamin D levels measured and reported, no comorbidities or history of vitamin D supplementation.
DATA EXTRACTION AND SYNTHESIS
Data extraction and synthesis were performed by a single reviewer and checked by a second reviewer. Inclusion and exclusion criteria and risk of bias were assessed by two independent reviewers using the Newcastle Ottawa Tool for Cohort studies and Risk of Bias Assessment Tool for Nonrandomised Studies checklist for case-control studies. Meta-analysis was conducted using random effects models. Standard mean difference with a 95% CI was used to measure the relationship between vitamin D level and BPPV.
RESULTS
The 35 articles identified by the literature search reported data of 9843 individuals. 19 studies (7387 individuals) were included in the BPPV incidence meta-analysis while 7 studies (622 individuals) were included in the BPPV recurrence meta-analysis. Lower serum vitamin D levels were found in BPPV incidence compared with controls, but the relationship between vitamin D levels in recurrent BPPV compared with non-recurrent disease remained uncertain.
CONCLUSION
Results of this systematic review and meta-analysis demonstrated a negative correlation between serum vitamin D and BPPV incidence, while any relationship between serum vitamin D and BPPV recurrence remained uncertain. Risk of bias analysis revealed evidence of variable quality. There were insufficient data available to evaluate seasonal relationships between serum vitamin D and BPPV. Given the potential for this as a confounding factor, future research should aim to investigate this further.
PROSPERO REGISTRATION NUMBER
CRD42021271840.
Topics: Humans; Benign Paroxysmal Positional Vertigo; Vitamin D Deficiency; Recurrence; Incidence; Vitamin D
PubMed: 38653514
DOI: 10.1136/bmjopen-2023-077986 -
Frontiers in Neuroscience 2020Auditory deprivation alters cortical and subcortical brain regions, primarily linked to auditory and language processing, resulting in behavioral consequences....
Auditory deprivation alters cortical and subcortical brain regions, primarily linked to auditory and language processing, resulting in behavioral consequences. Neuroimaging studies have reported various degrees of structural changes, yet multiple variables in deafness profiles need to be considered for proper interpretation of results. To date, many inconsistencies are reported in the gray and white matter alterations following early profound deafness. The purpose of this study was to provide the first systematic review synthesizing gray and white matter changes in deaf individuals. We conducted a systematic review according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement in 27 studies comprising 626 deaf individuals. Evidence shows that auditory deprivation significantly alters the white matter across the primary and secondary auditory cortices. The most consistent alteration across studies was in the bilateral superior temporal gyri. Furthermore, reductions in the fractional anisotropy of white matter fibers comprising in inferior fronto-occipital fasciculus, the superior longitudinal fasciculus, and the subcortical auditory pathway are reported. The reviewed studies also suggest that gray and white matter integrity is sensitive to early sign language acquisition, attenuating the effect of auditory deprivation on neurocognitive development. These findings suggest that understanding cortical reorganization through gray and white matter changes in auditory and non-auditory areas is an important factor in the development of auditory rehabilitation strategies in the deaf population.
PubMed: 32292323
DOI: 10.3389/fnins.2020.00206 -
Frontiers in Genetics 2021Mutations in the STRC (MIM 606440) gene, inducing DFNB16, are considered a major cause of mild-moderate autosomal recessive non-syndromic hearing loss (ARNSHL). We...
Mutations in the STRC (MIM 606440) gene, inducing DFNB16, are considered a major cause of mild-moderate autosomal recessive non-syndromic hearing loss (ARNSHL). We conducted a systematic review and meta-analysis to determine the global prevalence and characteristics of STRC variations, important information required for genetic counseling. PubMed, Google Scholar, Medline, Embase, and Web of Science were searched for relevant articles published before January 2021. The pooled prevalence of DFNB16 in GJB2-negative patients with hearing loss was 4.08% (95% CI: 0.0289-0.0573), and the proportion of STRC variants in the mild-moderate hearing loss group was 14.36%. Monoallelic mutations of STRC were 4.84% (95% CI: 0.0343-0.0680) in patients with deafness (non-GJB2) and 1.36% (95% CI: 0.0025-0.0696) in people with normal hearing. The DFNB16 prevalence in genetically confirmed patients (non-GJB2) was 11.10% (95% CI: 0.0716-0.1682). Overall pooled prevalence of deafness-infertility syndrome (DIS) was 36.75% (95% CI: 0.2122-0.5563) in DFNB16. The prevalence of biallelic deletions in STRC gene mutations was 70.85% (95% CI: 0.5824-0.8213). Variants in the STRC gene significantly contribute to mild-moderate hearing impairment. Moreover, biallelic deletions are a main feature of STRC mutations. Copy number variations associated with infertility should be seriously considered when investigating DFNB16.
PubMed: 34621290
DOI: 10.3389/fgene.2021.707845 -
European Archives of... Nov 2023This paper was aimed at estimating the prevalence and risk factors of hearing loss (HL) among the middle-aged and elderly in China. (Meta-Analysis)
Meta-Analysis Review
PURPOSE
This paper was aimed at estimating the prevalence and risk factors of hearing loss (HL) among the middle-aged and elderly in China.
METHODS
Databases including the CQVIP (VIP) Database, Chinese National Knowledge Infrastructure (CNKI), China Biology Medicine disc (CBMdisc), Wanfang, PubMed, Web of Science, Excerpta Medica Database (Embase) and the Cochrane Library were comprehensively searched. In this review, random-effect models were used for pooling the prevalence of HL and the odds ratios (ORs) of potential risk factors.
RESULTS
34 studies were included in the meta-analysis. HL among the middle-aged and elderly in China had a pooled prevalence of 45% (95% confidence interval (CI) 40-51%). There were significant differences in the prevalence of HL between males and females (47% vs. 42%), between different screening methods by self-report and pure-tone audiometry (44% vs. 46%), between the middle-aged and the elderly (18% vs. 52%), and between the uneducated and the educated (49% vs. 36%). In urban areas, the prevalence was slightly higher than that in rural areas (50% vs. 48%). The findings suggested that the middle-aged and elderly in the South Central China region (61%, 95% CI 45-78%) and Northwest China (57%, 95% CI 55-58%) were more likely to develop HL. In addition, it was confirmed that advanced age, being male, noise exposure history, hypertension and hyperglycemia were related to a higher prevalence of HL among middle-aged and older adults.
CONCLUSION
The prevalence of HL among the middle-aged and older population in China is 45%, nearly half of the total population. It is urgent to take great efforts to raise people's awareness of HL prevention and early hearing screening.
Topics: Aged; Female; Middle Aged; Humans; Male; Prevalence; Hearing Loss; Deafness; China; Risk Factors
PubMed: 37439927
DOI: 10.1007/s00405-023-08109-3 -
JAMA Otolaryngology-- Head & Neck... Jan 2022Although bone conduction devices (BCDs) have been shown to improve audiological outcomes of patients with single-sided sensorineural deafness (SSD), their effects on the... (Meta-Analysis)
Meta-Analysis
IMPORTANCE
Although bone conduction devices (BCDs) have been shown to improve audiological outcomes of patients with single-sided sensorineural deafness (SSD), their effects on the patients' quality of life (QOL) are unclear.
OBJECTIVE
To investigate the association of BCDs on QOL in patients with SSD.
DATA SOURCES
Literature search of databases (Medline, Embase, Cochrane Library, and ClinicalTrials.gov) from January 1, 1978, to June 24, 2021, was performed.
STUDY SELECTION
Prospective interventional studies with 10 or more participants with SSD (defined as pure tone average >70 dB hearing loss in the worse hearing ear and ≤30 dB in the better hearing ear) who underwent unilateral BCD implantation and assessment of QOL before and after the intervention using a validated tool were eligible for inclusion. Studies on adults and children were eligible for inclusion. Patients with only conductive, mixed, or bilateral hearing loss were excluded.
DATA EXTRACTION AND SYNTHESIS
Data were extracted by 2 independent reviewers. Study clinical and demographic characteristics were obtained. Meta-analysis of mean differences in QOL scores before and after the intervention was performed. Study bias was assessed using Joanna Briggs Institute risk of bias tool.
MAIN OUTCOMES AND MEASURES
The main study outcome was mean change in QOL scores at 6 months after insertion of BCDs. The 3 QOL instruments used in the studies included the Abbreviated Profile of Hearing Aid Benefit (APHAB), the Health Utilities Index-3 (HUI-3), and the Speech, Spatial and Qualities of Hearing Scale (SSQ). The APHAB and the SSQ are the hearing-related QOL measures, whereas the HUI-3 is a generic QOL measure.
RESULTS
A total of 486 articles were identified, and 11 studies with 203 patients met the inclusion criteria. Only adult studies met inclusion criteria. Ten of 11 studies were nonrandomized cohort studies. The BCDs assessed were heterogeneous. There was a significant statistical and clinically meaningful improvement in the global APHAB scores (mean change, 15.50; 95% CI, 12.63-18.36; I2 = 0) and the SSQ hearing qualities (mean change, 1.19; 95% CI, 0.46-1.92; I2 = 78.4%), speech (mean change, 2.03; 95% CI, 1.68-2.37; I2 = 0), and spatial hearing (mean change, 1.51; 95% CI, 0.57-2.44; I2 = 81.1%) subscales. There was no significant change detected in the mean HUI-3 scores (mean change, 0.03; 95% CI, -0.04 to 0.10; I2 = 0). The risk of bias was assessed to be low to moderate.
CONCLUSIONS AND RELEVANCE
These findings suggest that adult patients who receive BCDs may experience improvements in hearing-specific QOL measures but not in generic QOL measures. Prospective QOL studies should be considered in this cohort, particularly for children with SSD.
Topics: Bone Conduction; Hearing Aids; Hearing Loss, Sensorineural; Humans; Quality of Life
PubMed: 34647990
DOI: 10.1001/jamaoto.2021.2769 -
Archives of Disease in Childhood. Fetal... May 2022This systematic review evaluates vestibular and balance dysfunction in children with congenital cytomegalovirus (cCMV), makes recommendations for clinical practice and...
OBJECTIVE
This systematic review evaluates vestibular and balance dysfunction in children with congenital cytomegalovirus (cCMV), makes recommendations for clinical practice and informs future research priorities.
DESIGN
MEDLINE, Embase, EMCARE, BMJ Best Practice, Cochrane Library, DynaMed Plus and UpToDate were searched from inception to 20 March 2021 and graded according to Strengthening the Reporting of Observational Studies in Epidemiology (STROBE) criteria.
PATIENTS
Children with cCMV diagnosed within 3 weeks of life from either blood, saliva and/or urine (using either PCR or culture).
INTERVENTION
Studies of vestibular function and/or balance assessments.
MAIN OUTCOME MEASURES
Vestibular function and balance.
RESULTS
1371 studies were identified, and subsequently 16 observational studies were eligible for analysis, leading to an overall cohort of 600 children with cCMV. All studies were of low/moderate quality. In 12/16 studies, vestibular function tests were performed. 10/12 reported vestibular dysfunction in ≥40% of children with cCMV. Three studies compared outcomes for children with symptomatic or asymptomatic cCMV at birth; vestibular dysfunction was more frequently reported in children with symptomatic (22%-60%), than asymptomatic cCMV (0%-12.5%). Two studies found that vestibular function deteriorated over time: one in children (mean age 7.2 months) over 10 months and the other (mean age 34.7 months) over 26 months.
CONCLUSIONS
Vestibular dysfunction is found in children with symptomatic and asymptomatic cCMV and in those with and without hearing loss. Audiovestibular assessments should be performed as part of neurodevelopmental follow-up in children with cCMV. Case-controlled longitudinal studies are required to more precisely characterise vestibular dysfunction and help determine the efficacy of early supportive interventions.
PROSPERO REGISTRATION
CRD42019131656.
PubMed: 35545420
DOI: 10.1136/archdischild-2021-323380 -
Journal of Clinical Immunology Jan 2023Sideroblastic anaemia with B-cell immunodeficiency, periodic fever and developmental delay (SIFD) syndrome is a novel rare autoinflammatory multisystem disorder. We... (Review)
Review
BACKGROUND AND PURPOSE
Sideroblastic anaemia with B-cell immunodeficiency, periodic fever and developmental delay (SIFD) syndrome is a novel rare autoinflammatory multisystem disorder. We performed a systematic review of the available clinical and therapeutics aspects of the SIFD syndrome.
METHODS
A systematic review according to PRISMA approach, including all articles published before the 30 of July 2021 in Pubmed and EMBASE database, was performed.
RESULTS
The search identified 29 publications describing 58 unique patients. To date, 41 unique mutations have been reported. Onset of disease is very early with a median age of 4 months (range 0-252 months). The most frequent manifestations are haematologic such as microcytic anaemia or sideroblastic anaemia (55/58), recurrent fever (52/58), neurologic abnormalities (48/58), immunologic abnormalities in particular a humoral immunodeficiency (48/58), gastrointestinal signs and symptoms (38/58), eye diseases as cataract and retinitis pigmentosa (27/58), failure to thrive (26/58), mucocutaneous involvement (29/58), sensorineural deafness (19/58) and others. To date, 19 patients (35.85%) died because of disease course (16) and complications of hematopoietic cell stems transplantation (3). The use of anti-TNFα and hematopoietic cell stems transplantation (HCST) is dramatically changing the natural history of this disease.
CONCLUSIONS
SIFD syndrome is a novel entity to consider in a child presenting with recurrent fever, anaemia, B-cell immunodeficiency and neurodevelopmental delay. To date, therapeutic guidelines are lacking but anti-TNFα treatment and/or HCST are attractive and might modify the clinical course of this syndrome.
Topics: Child; Humans; Anemia, Sideroblastic; Immunologic Deficiency Syndromes; Fever; Mutation; Developmental Disabilities
PubMed: 35984545
DOI: 10.1007/s10875-022-01343-0