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Laryngoscope Investigative... Aug 2023This systematic review aims to establish the expected hearing and speech outcomes following cochlear implantation (CI) in patients with profound congenital deafness... (Review)
Review
OBJECTIVE
This systematic review aims to establish the expected hearing and speech outcomes following cochlear implantation (CI) in patients with profound congenital deafness secondary to Waardenburg syndrome (WS).
METHODS
A systematic review of the literature and narrative synthesis was performed in accordance with the PRISMA statement. Databases searched: Medline, Pubmed, Embase, Web of Science, Cochrane Collection, and ClinicalTrials.gov. No limits were placed on language or year of publication.
RESULTS
Searches identified 186 abstracts and full texts. Of these, 16 studies met inclusion criteria reporting outcomes in 179 patients and at least 194 implants. Hearing outcomes of those receiving cochlear implantation were generally good. Five studies included genetic analysis of one or more of the participants. A total of 11 peri/post-operative complications were reported. The methodological quality of included studies was modest, mainly comprising noncontrolled case series with small cohort size. All studies were OCEBM grade III-IV.
CONCLUSION
Cochlear implantation in congenitally deafened children with Waardenburg Syndrome is a well-established intervention as a method of auditory rehabilitation. Due to the uncommon nature of the condition, there is a lack of large-scale high-quality studies examining the use of cochlear implantation in this patient group. However, overall outcomes following implantation are positive with the majority of patients demonstrating improved audiometry, speech perception and speech intelligibility supporting its use in appropriately selected cases.
PubMed: 37621295
DOI: 10.1002/lio2.1110 -
Frontiers in Psychology 2021An association between age-related hearing loss (ARHL) and Alzheimer's Disease (AD) has been widely reported. However, the nature of this relationship remains poorly...
An association between age-related hearing loss (ARHL) and Alzheimer's Disease (AD) has been widely reported. However, the nature of this relationship remains poorly understood. Quantification of hearing loss as it relates to AD is imperative for the creation of reliable, hearing-related biomarkers for earlier diagnosis and development of ARHL treatments that may slow the progression of AD. Previous studies that have measured the association between peripheral hearing function and AD have yielded mixed results. Most of these studies have been small and underpowered to reveal an association. Therefore, in the current report, we sought to estimate the degree to which AD patients have impaired hearing by performing a meta-analysis to increase statistical power. We reviewed 248 published studies that quantified peripheral hearing function using pure-tone audiometry for subjects with AD. Six studies, with a combined total of 171 subjects with AD compared to 222 age-matched controls, met inclusion criteria. We found a statistically significant increase in hearing threshold as measured by pure tone audiometry for subjects with AD compared to controls. For a three-frequency pure tone average calculated for air conduction thresholds at 500-1,000-2,000 Hz (0.5-2 kHz PTA), an increase of 2.3 decibel hearing level (dB HL) was found in subjects with AD compared to controls ( = 0.001). Likewise, for a four-frequency pure tone average calculated at 500-1,000-2,000-4,000 (0.5-4 kHz PTA), an increase of 4.5 dB HL was measured ( = 0.002), and this increase was significantly greater than that seen for 0.5-2 kHz PTA. There was no difference in the average age of the control and AD subjects. These data confirm the presence of poorer hearing ability in AD subjects, provided a quantitative estimate of the magnitude of hearing loss, and suggest that the magnitude of the effect is greater at higher sound frequencies. https://www.crd.york.ac.uk/prospero/, identifier: CRD42021288280.
PubMed: 35153910
DOI: 10.3389/fpsyg.2021.788045 -
Journal of Clinical Medicine Dec 2020Congenital cytomegalovirus (CMV) infection induces a clinical syndrome usually associated with hearing loss. However, the effect of acquired CVM infection in adults and... (Review)
Review
Congenital cytomegalovirus (CMV) infection induces a clinical syndrome usually associated with hearing loss. However, the effect of acquired CVM infection in adults and children has not been clearly defined. The objective of this review is to critically appraise scientific evidence regarding the association of acquired CMV infection with postnatal hearing loss or tinnitus. A systematic review of records reporting sensorineural hearing loss (SNHL) or tinnitus and acquired CMV infection including articles published in English was performed. Search strategy was limited to human studies with acquired CMV infection. After screening and quality assessment, nine studies involving 1528 individuals fulfilled the inclusion criteria. A total of 14% of patients with SNHL showed evidence of previous exposure to CMV, while in individuals without SNHL (controls) the percentage rose up to 19.3%. SNHL was reported as unilateral or bilateral in 15.3%, and not specified in 84.7% of cases. The degree of SNHL ranged from mild to profound for both children and adults. None of the records reported tinnitus. The prevalence of children or adults with acquired SNHL with a confirmed acquired CMV infection by Polymerase Chain Reaction (PCR) or IgM anti-CMV antibodies is low. Phenotyping of patients with acquired CMV infection was limited to hearing loss by pure tone audiometry and no additional audiological testing was performed in most of the studies. Additional symptoms deserve more attention, including episodic vertigo or tinnitus, since some patients with the clinical spectrum of Meniere Disease could result from a CMV latent infection.
PubMed: 33322509
DOI: 10.3390/jcm9124011 -
Journal of Personalized Medicine Nov 2023Profound sensorineural hearing loss (SNHL) can be successfully treated with a cochlear implant (CI), and treatment is usually accompanied by increased quality of life... (Review)
Review
Profound sensorineural hearing loss (SNHL) can be successfully treated with a cochlear implant (CI), and treatment is usually accompanied by increased quality of life (QoL). Therefore, the aim of this study was to investigate generic and health-related QoL, as well as the level of audiological outcomes, of CI users, in addition to whether Qol can be restored to the extent of those with normal hearing. Furthermore, different implantation timepoints were compared (early vs. late), and a possible correlation between health and generic QoL questionnaires was investigated. The outcomes from 93 pediatric CI users from Romania were analyzed in the study. Two QoL questionnaires (SSQ12, AQoL-6D), as well as the HSM sentence test and Soundfield measurements, were assessed. The outcomes revealed that the CI users were able to achieve the same QoL as their age- and-gender matched peers with normal hearing, and hearing was restored with good speech comprehension. No significant difference between early- and late-implanted children was detected, although a tendency of a better Word Recognition Score (+10%) in the early-implanted group was discovered. A moderate and significant correlation between the generic and health-related Qol questionnaire was observed. Audiological examinations are still the standard practice by which to measure the benefit of any hearing intervention; nonetheless, generic and health-related QoL should be assessed in order to provide a full picture of a successful and patient-satisfactory cochlear implant procedure.
PubMed: 38003925
DOI: 10.3390/jpm13111610 -
Otology & Neurotology : Official... Aug 2021To calculate the incidence of sensorineural hearing loss (SNHL; pure or as part of a mixed hearing loss, MHL) among bullous myringitis (BM) patients with a coexisting HL...
OBJECTIVE
To calculate the incidence of sensorineural hearing loss (SNHL; pure or as part of a mixed hearing loss, MHL) among bullous myringitis (BM) patients with a coexisting HL to assess whether steroidal treatment should be initiated even before the findings of a formal audiogram are available.
METHODS
Retrospective medical record review in a tertiary referral center and systematic review of the English literature.
RESULTS
The medical records of 81 patients with BM were retrieved among whom 50 patients (62%) had actually sustained a HL, although only 39/81 patients reported a HL when asked. Twenty-four patients had a MHL, 18 had a SNHL, and eight had a conductive HL (CHL). The systematic review included 106 ears: 17 had no HL, 53 had a MHL, 20 had a SNHL, and 16 had a CHL. The combined published and current results yielded a SNHL and MHL rates of 38/139 (27%) and 77/139 (55%), respectively of all BM patients with confirmed HL.
CONCLUSION
BM is intuitively associated with a CHL resulting from the impeded function of the affected middle ear. Medical history and physical examination are not sufficient to exclude a SNHL in BM patients, and formal audiometry is mandatory for exclusion/confirmation in this setting. Nevertheless, steroidal treatment should not be delayed until the performance of formal audiometry because the actual rate of pure CHL in this setting is very low.Level of Evidence: 2.
Topics: Audiometry; Hearing; Hearing Loss, Sensorineural; Humans; Incidence; Retrospective Studies
PubMed: 33883520
DOI: 10.1097/MAO.0000000000003160 -
Efficacy of behavioral audiological tests in identifying cochlear synaptopathy: a systematic review.European Archives of... Feb 2022Cochlear synaptopathy or hidden hearing loss is difficult to assess due to the lack of sensitivity with standard audiological tests. Poor speech perception, especially... (Review)
Review
PURPOSE
Cochlear synaptopathy or hidden hearing loss is difficult to assess due to the lack of sensitivity with standard audiological tests. Poor speech perception, especially in the presence of noise or tinnitus, is the most common complaint of these patients. The purpose of this systematic review is to identify articles in peer-reviewed journals that used behavioral measures in the effective assessment of cochlear synaptopathy or hidden hearing loss.
METHODS
The manuscripts were searched in various international databases, and the manuscripts were screened based on titles, abstracts, and full-length content. A total of 14 human studies were selected after the appropriate exclusion of other articles.
RESULTS
Results showed that high-frequency audiometry could be used for the early identification of cochlear synaptopathy. The tone in noise detection test can also be added in the test battery along with speech perception in noise. The amplitude modulation detection test, interaural phase difference, and differential sensitivity tests require more research before using them for the assessment of cochlear synaptopathy or hidden hearing loss.
CONCLUSIONS
Self-reports and questionnaires also help in determining the extent of noise exposure.
Topics: Auditory Threshold; Cochlea; Evoked Potentials, Auditory, Brain Stem; Hearing Loss, Noise-Induced; Humans; Noise
PubMed: 34106328
DOI: 10.1007/s00405-021-06927-x -
CoDAS 2022To verify the accuracy of smartphone apps to identify hearing loss.
PURPOSE
To verify the accuracy of smartphone apps to identify hearing loss.
RESEARCH STRATEGIES
A systematic review followed the PRISMA-DATA checklist. The search strategies were applied across four databases (Lilacs, PubMed, Scopus and Web of Science) and grey literature (Google Scholar, OpenGrey, and ProQuest Dissertations and Thesis).
SELECTION CRITERIA
The acronym PIRD was used in review. This included populations of any gender and all age groups. The Index test is the smartphone-based hearing screening test; the Reference test is the pure-tone audiometry, which is considered the gold reference for hearing diagnostics; the diagnosis was performed via validity data (sensitivity and specificity) to identify hearing loss and diagnostic studies.
DATA ANALYSIS
Two reviewers selected the studies in a two-step process. The risk of bias was assessed according to the criteria of the QUADAS-2.
RESULTS
Of 1395 articles, 104 articles were eligible for full-text reading and 17 were included. Only four met all criteria for methodological quality. All of the included studies were published in English between 2015 and 2020. The applications Digits-in noise Test (5 articles), uHear (4 articles), HearScreen (2 articles), hearTest (2 articles) and Hearing Test (2 articles) were the most studied. All this application showed sensitivity and specificity values between 75 and 100%. The other applications were EarScale, uHearing Test, Free field hearing (FFH) and Free Hearing Test.
CONCLUSION
uHear, Digit-in-Noise Test, HearTest and HearScreen have shown significant values of sensitivity and specificity and can be considered as the most accurate methods for screening of hearing impairment.
Topics: Audiometry, Pure-Tone; Hearing; Hearing Loss; Humans; Noise; Sensitivity and Specificity; Smartphone
PubMed: 35239828
DOI: 10.1590/2317-1782/20212020380 -
Otology & Neurotology : Official... Sep 2023To compare audiometric outcomes, complications, and revisions required for implantable bone-conduction devices (BCDs) versus atresia surgery for the treatment of hearing... (Meta-Analysis)
Meta-Analysis
Comparison of Outcomes of Surgery Versus Implantable Device for the Treatment of Hearing Loss Associated With Congenital Aural Atresia: A Systematic Review and Meta-Analysis.
OBJECTIVE
To compare audiometric outcomes, complications, and revisions required for implantable bone-conduction devices (BCDs) versus atresia surgery for the treatment of hearing loss associated with congenital aural atresia.
DATABASES REVIEWED
PubMed, Scopus, CINAHL.
METHODS
Databases were searched for English articles from inception to July 1, 2022, for studies reporting audiometric outcomes or complications for either BCDs or atresia surgery for the treatment of congenital aural atresia. Main outcome measures included pure-tone audiometry, air-bone gap, speech reception threshold, associated complications, and rates of revision for each treatment option.
RESULTS
We identified 973 abstracts, of which 89 were selected for data extraction and analysis. A total of 2,611 patients were included, 1,901 in the atresia surgery group and 710 in the BCDs group. A meta-analysis of single means was conducted for age and audiometric outcomes, and a meta-analysis of proportions was conducted for complications and revisions. The average short-term improvement in pure-tone audiometry for the BCDs group was 34.4 ± 1.6 dB compared with 22.4 ± 1.5 dB for the atresia surgery group, representing a significant difference (12.0 dB; 95% confidence interval, 11.9-12.2; p < 0.0001). A smaller proportion of complications were reported in the devices group (16.9%) compared with the atresia surgery group (45.7%). In addition, a smaller proportion of cases in the devices group required some degree of revision (17.8%) compared with the atresia surgery group (23.0%).
CONCLUSIONS
This study demonstrates that implantable BCDs have significantly better audiometric outcomes as well as a lower rate of complications and revisions required compared with atresia surgery.
Topics: Humans; Treatment Outcome; Hearing Loss; Deafness; Audiometry, Pure-Tone; Ossicular Prosthesis; Hearing Loss, Conductive; Retrospective Studies; Bone Conduction
PubMed: 37464461
DOI: 10.1097/MAO.0000000000003950 -
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 -
Ear and HearingCurrent evidence supports the growing application of extended high-frequency (EHF: 9 to 20 kHz) audiometry in hearing research, which likely results from the high... (Meta-Analysis)
Meta-Analysis
OBJECTIVES
Current evidence supports the growing application of extended high-frequency (EHF: 9 to 20 kHz) audiometry in hearing research, which likely results from the high vulnerability of this frequency region to damage induced by known auditory risk factors. The present systematic review and meta-analysis were performed to investigate whether adults with a normal audiogram and tinnitus show increased EHF hearing thresholds relative to control peers.
DESIGN
A comprehensive search was undertaken on electronic databases consisting of PubMed, ScienceDirect, Wiley, and Google Scholar using combined keywords: "tinnitus," "extended high frequency," "normal audiogram," and "hidden hearing loss."
RESULTS
From 261 articles found by searching databases, nine studies met the inclusion criteria for the meta-analysis. A significant difference was observed between tinnitus and control groups in the effect size analysis of hearing thresholds at 10, 12.5, 14, 16, and 18 kHz ( p ≤ 0.001), and the I-square heterogeneity analysis was below 50% in all studies ( p ≥ 0.131). Visual inspection by the Funnel plot and Egger's regression test ( p ≥ 0.211) also exhibited no publication bias in the meta-analyses.
CONCLUSIONS
Our findings are in support of the idea that in most cases, tinnitus is associated with some degree of cochlear mechanical dysfunction, which may not be detected by conventional audiometry alone. This finding underscores the significance of EHF audiometry in clinical practice, which may help both early identification of individuals susceptible to developing tinnitus and reduce the number of new cases through preventive counseling programs.
Topics: Adult; Humans; Tinnitus; Auditory Threshold; Hearing; Audiometry; Hearing Loss; Audiometry, Pure-Tone
PubMed: 35612517
DOI: 10.1097/AUD.0000000000001229