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BMJ Open Aug 2022Systematically investigate the effects of multiple sclerosis (MS) on the audio-vestibular system.
OBJECTIVE
Systematically investigate the effects of multiple sclerosis (MS) on the audio-vestibular system.
METHODS
Systematic review of literature investigating audio-vestibular conditions in persons with MS (PwMS) aged ≥18 years. PubMed, Scopus, NICE and Web of Science were searched. Randomised controlled trials, and cohort, case-control, observational and retrospective studies in English, published from 2000 to 21 November 2021, evaluated PwMS with at least one outcome (pure tone audiometry, auditory brainstem response, otoacoustic emissions, cortical auditory evoked potentials, functional MRI assessing auditory function, vestibular evoked myogenic potentials, videonystagmography, electronystagmography, posturography, rotary chair, gaps in noise, word discrimination scores, duration pattern sequence test), were included. Study selection and assessments of bias were independently conducted by two reviewers using the Risk of Bias Assessment Tool for Non-randomized Studies, Newcastle-Ottawa Scale (NOS) and the NOS adapted for cross-sectional studies.
RESULTS
35 studies were included. Auditory function was evaluated in 714 PwMS and 501 controls, vestibular function was evaluated in 682 PwMS and 446 controls. Peripheral auditory function results were contradictory between studies; some found abnormalities in PwMS, and others found no differences. Tests of brainstem and central auditory functions were more consistently found to be abnormal in PwMS. Most vestibular tests were reported as abnormal in PwMS, abnormalities were either peripheral or central or both. However, quantitative analyses could not be performed due to discrepancies between studies in results reporting, test stimulus and recording parameters.
CONCLUSIONS
Although abnormal results on auditory and vestibular tests were noted in PwMS, specific effects of MS on the audio-vestibular system could not be determined due to the heterogeneity between studies that restricted the ability to conduct any quantitative analyses. Further research with consistent reporting, consistent stimulus and consistent recording parameters is needed in order to quantify the effects of MS on the auditory and vestibular systems.
PROSPERO REGISTRATION NUMBER
CRD42020180094.
Topics: Adolescent; Adult; Audiometry, Pure-Tone; Cross-Sectional Studies; Humans; Multiple Sclerosis; Otoacoustic Emissions, Spontaneous; Retrospective Studies
PubMed: 35977771
DOI: 10.1136/bmjopen-2021-060540 -
Ear, Nose, & Throat Journal Jun 2024To analyze the etiology, diagnosis, and treatment of unexplained conductive hearing loss (UCHL) with intact tympanic membrane. A systematic review was conducted based... (Review)
Review
To analyze the etiology, diagnosis, and treatment of unexplained conductive hearing loss (UCHL) with intact tympanic membrane. A systematic review was conducted based on the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. A total of 642 articles were retrieved from databases such as PubMed, Embase, Web of Science, and Cochrane. Fifty-four research articles and 21 case reports were screened out according to the inclusion and exclusion criteria for analysis of the etiology of UCHL. Seven research articles with UCHL who underwent exploratory tympanotomy were selected for data extraction and analysis of clinical characteristics. UCHL is a common manifestation of various diseases, including congenital ossicular anomalies (COA), otosclerosis (OTS), congenital middle ear cholesteatoma (CMEC), oval window atresia, superior semicircular-canal dehiscence, congenital stapedial footplate fixation, middle ear osteoma or adenoma, congenital ossification of stapedial tendon, and so on. A total of 522 patients were included in the 7 articles; among whom OTS showed a tendency to increase with age. The main symptoms were hearing loss, followed by tinnitus, dizziness, ear fullness, ear pain, facial paralysis. A total of 87.5% to 93.0% patients with COA manifested as nonprogressive deafness that occurred since childhood, with tinnitus incidence of 15.6% to 30.2%, and 86.4% to 96.4% patients with OTS presented with progressive hearing loss, with tinnitus incidence of 60.1% to 90.9%. The diagnosis positive rate of high-resolution computed tomography (HRCT) was 33.8% to 87.1%, and CMEC was higher than that of COA (83.3%-100% vs 28.6%-64%). All the articles reported good hearing recovery. The most common surgical complications included taste abnormalities, tinnitus, and dizziness. UCHL presents with similar clinical manifestations and poses challenges in preoperative diagnosis. Exploratory tympanotomy is the primary method for diagnosis and treatment, with good prognosis after removing the lesion and reconstructing hearing during the operation. Children can also safely undergo the surgery.
PubMed: 38895947
DOI: 10.1177/01455613241262129 -
PloS One 2023Growing evidence suggests that alcohol consumption is a risk factor for hearing loss; however, the evidence has been inconsistent. This systematic review and... (Meta-Analysis)
Meta-Analysis
OBJECTIVE
Growing evidence suggests that alcohol consumption is a risk factor for hearing loss; however, the evidence has been inconsistent. This systematic review and meta-analysis aimed to evaluate the effect of alcohol consumption on hearing loss.
METHODS
We searched several databases up to November 2021, for published articles using the keywords "alcohol drinking" and "hearing loss". Two investigators independently conducted the study selection and data extraction. Based on the results of the heterogeneity analysis (Q statistic and I2 statistic), a fixed- or random-effects model was used to calculate the pooled odds ratios (ORs). Subgroup and sensitivity analyses were performed to assess the potential sources of heterogeneity and robustness of the pooled estimation. Publication bias in the literature was evaluated using Egger's test.
RESULTS
In total, 18 (9 cross-sectional, 5 case-control, and 4 cohort) observational studies were identified in this search; 27,849 participants were included. Compared with non-drinkers, the pooled OR of drinkers was 1.22 (95% confidence interval: 1.09-1.35).
CONCLUSION
Evidence suggests a positive association between alcohol consumption and hearing loss. Drinkers were at a higher risk than non-drinkers. Drinking limitations may be useful for preventing hearing loss.
Topics: Humans; Cross-Sectional Studies; Risk Factors; Alcohol Drinking; Hearing Loss; Deafness; Hearing
PubMed: 36662896
DOI: 10.1371/journal.pone.0280641 -
American Journal of Speech-language... Aug 2020Purpose Functional brain imaging is playing an increasingly important role in the diagnosis and treatment of communication disorders, yet many populations and settings...
Purpose Functional brain imaging is playing an increasingly important role in the diagnosis and treatment of communication disorders, yet many populations and settings are incompatible with functional magnetic resonance imaging and other commonly used techniques. We conducted a systematic review of neuroimaging studies using functional near-infrared spectroscopy (fNIRS) with individuals with speech or language impairment across the life span. We aimed to answer the following question: To what extent has fNIRS been used to investigate the neural correlates of speech-language impairment? Method This systematic review was preregistered with PROSPERO, the international prospective register of systematic reviews (CRD42019136464). We followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) protocol for preferred reporting items for systematic reviews. The database searches were conducted between February and March of 2019 with the following search terms: (a) fNIRS or functional near-infrared spectroscopy or NIRS or near-infrared spectroscopy, (b) speech or language, and (c) disorder or impairment or delay. Results We found 34 fNIRS studies that involved individuals with speech or language impairment across nine categories: (a) autism spectrum disorders; (b) developmental speech and language disorders; (c) cochlear implantation and deafness; (d) dementia, dementia of the Alzheimer's type, and mild cognitive impairment; (e) locked-in syndrome; (f) neurologic speech disorders/dysarthria; (g) stroke/aphasia; (h) stuttering; and (i) traumatic brain injury. Conclusions Though it is not without inherent challenges, fNIRS may have advantages over other neuroimaging techniques in the areas of speech and language impairment. fNIRS has clinical applications that may lead to improved early and differential diagnosis, increase our understanding of response to treatment, improve neuroprosthetic functioning, and advance neurofeedback.
Topics: Brain; Humans; Language Development Disorders; Longevity; Spectroscopy, Near-Infrared; Speech
PubMed: 32640168
DOI: 10.1044/2020_AJSLP-19-00050 -
European Archives of... Oct 2023To review hearing and surgical outcomes after reconstructive middle ear surgery in class 4 congenital middle ear anomalies (CMEA), e.g., patients with oval- or round... (Review)
Review
OBJECTIVE
To review hearing and surgical outcomes after reconstructive middle ear surgery in class 4 congenital middle ear anomalies (CMEA), e.g., patients with oval- or round window atresia of dysplasia.
DATA SOURCES
Pubmed/Medline, Embase and Cochrane library.
REVIEW METHODS
Articles containing data on hearing outcomes and complications after reconstructive ear surgery in class 4 anomalies were analyzed and critically appraised. The following data were included and reviewed: patient demographics, audiometric testing, surgical techniques, complications, revision surgeries and their outcomes. Risk of bias was determined, and GRADE certainty of evidence was assessed. Primary outcomes were postoperative air conduction thresholds (AC), change in AC, and success rates (closure of the ABG to within 20 dB), the occurrence of complications (most importantly sensorineural hearing loss) and the long-term stability of hearing results (> 6-month follow-up) and occurrence of recurrence of preoperative hearing loss.
RESULTS
Success rates varied from 12.5 to 75% at long-term follow-up with larger cohorts reporting success rates around 50%, mean postoperative gain in AC varied from 4.7 to 30 dB and - 8.6 to 23.6 dB at, respectively, short- and long-term follow-up. No postoperative change in hearing occurred in 0-33.3% of ears, and recurrence of hearing loss occurred in 0-66.7% of ears. SNHL occurred in a total of seven ears across all studies of which three experienced complete hearing loss.
CONCLUSION
Reconstructive surgery can be an effective treatment option which should be considered in patients with very favorable baseline parameters, while also considering the substantial risk of recurrence of hearing loss, the possibility of unchanged hearing despite surgery and the rare occurrence of SNHL.
LEVEL OF EVIDENCE
2c.
Topics: Humans; Ear Ossicles; Ear, Middle; Ear; Treatment Outcome; Hearing Loss, Conductive; Deafness; Retrospective Studies; Ossicular Prosthesis
PubMed: 37410147
DOI: 10.1007/s00405-023-08091-w -
Ear and HearingThe indications for cochlear implantation have expanded to include individuals with profound sensorineural hearing loss in the impaired ear and normal hearing (NH) in...
The indications for cochlear implantation have expanded to include individuals with profound sensorineural hearing loss in the impaired ear and normal hearing (NH) in the contralateral ear, known as single-sided deafness (SSD). There are additional considerations for the clinical assessment and management of adult cochlear implant candidates and recipients with SSD as compared to conventional cochlear implant candidates with bilateral moderate to profound sensorineural hearing loss. The present report reviews the current evidence relevant to the assessment and management of adults with SSD. A systematic review was also conducted on published studies that investigated outcomes of cochlear implant use on measures of speech recognition in quiet and noise, sound source localization, tinnitus perception, and quality of life for this patient population. Expert consensus and systematic review of the current literature were combined to provide guidance for the clinical assessment and management of adults with SSD.
Topics: Adult; Humans; Cochlear Implantation; Cochlear Implants; Quality of Life; Speech Perception; Sound Localization; Hearing Loss, Sensorineural; Deafness; Hearing Loss, Unilateral; Treatment Outcome
PubMed: 35994570
DOI: 10.1097/AUD.0000000000001260 -
Omics : a Journal of Integrative Biology Jan 2022Hearing impairment (HI) is a silent planetary health crisis that requires attention worldwide. The prevalence of HI in South Africa is estimated as 5.5 in 100 live...
Hearing impairment (HI) is a silent planetary health crisis that requires attention worldwide. The prevalence of HI in South Africa is estimated as 5.5 in 100 live births, which is about 5 times higher than the prevalence in high-income countries. This also offers opportunity to drive progressive science, technology and innovation policy, and health systems. We present here a systematic analysis and review on the prevalence, etiologies, clinical patterns, and genetics/genomics of HI in South Africa. We searched PubMed, Scopus, African Journals Online, AFROLIB, and African Index Medicus to identify the pertinent studies on HI in South Africa, published from inception to April 30, 2021, and the data were summarized narratively. We screened 944 records, of which 27 studies were included in the review. The age at diagnosis is ∼3 years of age and the most common factor associated with acquired HI was middle ear infections. There were numerous reports on medication toxicity, with kanamycin-induced ototoxicity requiring specific attention when considering the high burden of tuberculosis in South Africa. The Waardenburg Syndrome is the most common reported syndromic HI. The Usher Syndrome is the only syndrome with genetic investigations, whereby a founder mutation was identified among black South Africans (-c.6377delC). and genes are not major contributors to nonsyndromic HI among Black South Africans. Furthermore, emerging data using targeted panel sequencing have shown a low resolution rate in Black South Africans in known HI genes. Importantly, mutations in known nonsyndromic HI genes are infrequent in South Africa. Therefore, whole-exome sequencing appears as the most effective way forward to identify variants associated with HI in South Africa. Taken together, this article contributes to the emerging field of planetary health genomics with a focus on HI and offers new insights and lessons learned for future roadmaps on genomics/multiomics and clinical studies of HI around the world.
Topics: Deafness; Genomics; Hearing Loss; Humans; Mutation; South Africa
PubMed: 35041532
DOI: 10.1089/omi.2021.0181 -
BMC Medical Genomics Apr 2023Wolfram syndrome type 1 gene (WFS1), which encodes a transmembrane structural protein (wolframin), is essential for several biological processes, including proper inner...
BACKGROUND
Wolfram syndrome type 1 gene (WFS1), which encodes a transmembrane structural protein (wolframin), is essential for several biological processes, including proper inner ear function. Unlike the recessively inherited Wolfram syndrome, WFS1 heterozygous variants cause DFNA6/14/38 and wolfram-like syndrome, characterized by autosomal dominant nonsyndromic hearing loss, optic atrophy, and diabetes mellitus. Here, we identified two WFS1 heterozygous variants in three DFNA6/14/38 families using exome sequencing. We reveal the pathogenicity of the WFS1 variants based on three-dimensional (3D) modeling and structural analysis. Furthermore, we present cochlear implantation (CI) outcomes in WFS1-associated DFNA6/14/38 and suggest a genotype-phenotype correlation based on our results and a systematic review.
METHODS
We performed molecular genetic test and evaluated clinical phenotypes of three WFS1-associated DFNA6/14/38 families. A putative WFS1-NCS1 interaction model was generated, and the impacts of WFS1 variants on stability were predicted by comparing intramolecular interactions. A total of 62 WFS1 variants associated with DFNA6/14/38 were included in a systematic review.
RESULTS
One variant is a known mutational hotspot variant in the endoplasmic reticulum (ER)-luminal domain WFS1(NM_006005.3) (c.2051 C > T:p.Ala684Val), and the other is a novel frameshift variant in transmembrane domain 6 (c.1544_1545insA:p.Phe515LeufsTer28). The two variants were pathogenic, based on the ACMG/AMP guidelines. Three-dimensional modeling and structural analysis show that non-polar, hydrophobic substitution of Ala684 (p.Ala684Val) destabilizes the alpha helix and contributes to the loss of WFS1-NCS1 interaction. Also, the p.Phe515LeufsTer28 variant truncates transmembrane domain 7-9 and the ER-luminal domain, possibly impairing membrane localization and C-terminal signal transduction. The systematic review demonstrates favorable outcomes of CI. Remarkably, p.Ala684Val in WFS1 is associated with early-onset severe-to-profound deafness, revealing a strong candidate variant for CI.
CONCLUSIONS
We expanded the genotypic spectrum of WFS1 heterozygous variants underlying DFNA6/14/38 and revealed the pathogenicity of mutant WFS1, providing a theoretical basis for WFS1-NCS1 interactions. We presented a range of phenotypic traits for WFS1 heterozygous variants and demonstrated favorable functional CI outcomes, proposing p.Ala684Val a strong potential marker for CI candidates.
Topics: Humans; Wolfram Syndrome; Cochlear Implants; Cochlear Implantation; Pedigree; Hearing Loss; Deafness
PubMed: 37041640
DOI: 10.1186/s12920-023-01506-x -
International Journal of Molecular... Oct 2023With more than 466 million people affected, hearing loss represents the most common sensory pathology worldwide. Despite its widespread occurrence, much remains to be... (Review)
Review
With more than 466 million people affected, hearing loss represents the most common sensory pathology worldwide. Despite its widespread occurrence, much remains to be explored, particularly concerning the intricate pathogenic mechanisms underlying its diverse phenotypes. In this context, metabolomics emerges as a promising approach. Indeed, lying downstream from molecular biology's central dogma, the metabolome reflects both genetic traits and environmental influences. Furthermore, its dynamic nature facilitates well-defined changes during disease states, making metabolomic analysis a unique lens into the mechanisms underpinning various hearing impairment forms. Hence, these investigations may pave the way for improved diagnostic strategies, personalized interventions and targeted treatments, ultimately enhancing the clinical management of affected individuals. In this comprehensive review, we discuss findings from 20 original articles, including human and animal studies. Existing literature highlights specific metabolic changes associated with hearing loss and ototoxicity of certain compounds. Nevertheless, numerous critical issues have emerged from the study of the current state of the art, with the lack of standardization of methods, significant heterogeneity in the studies and often small sample sizes being the main limiting factors for the reliability of these findings. Therefore, these results should serve as a stepping stone for future research aimed at addressing the aforementioned challenges.
Topics: Animals; Humans; Reproducibility of Results; Hearing Loss; Deafness; Metabolomics; Metabolome
PubMed: 37894867
DOI: 10.3390/ijms242015188 -
International Journal of Pediatric... Jun 2023This review compiles the scientific evidence to date on the effectiveness of musical/rhythmic training for improving and/or enhancing the development of language skills... (Review)
Review
OBJECTIVE
This review compiles the scientific evidence to date on the effectiveness of musical/rhythmic training for improving and/or enhancing the development of language skills in deaf children aged 6-16 years with cochlear implants.
METHODS
PubMed, ScienceDirect, and Web of Science were used for the research following the PRISMA protocol.
RESULTS
The reviewed studies indicate that rhythmic training can improve language skills (perception, production, and comprehension) in this population, as well as in other cognitive skills.
CONCLUSION
Although further research is still needed, the current evidence can help identify new and more effective early intervention methods for deaf children.
Topics: Child; Humans; Adolescent; Cochlear Implants; Deafness; Cochlear Implantation; Linguistics; Cognition; Speech Perception
PubMed: 37088038
DOI: 10.1016/j.ijporl.2023.111561