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Audiology Research May 2024(1) Background: Patients affected by Ménière's disease can experience Tumarkin's syndrome, which is characterized by postural instability, gait abnormalities, and,...
(1) Background: Patients affected by Ménière's disease can experience Tumarkin's syndrome, which is characterized by postural instability, gait abnormalities, and, occasionally, an abrupt loss of balance known as vestibular drop attack or Tumarkin's crisis. In this study, semicircular canal plugging is proposed as the definitive treatment for this condition. The outcomes of this type of surgery are discussed. (2) Methods: A total of 9 patients with a confirmed diagnosis of Ménière disease suffering from Tumarkin crisis underwent posterior semicircular canal plugging. These patients were assessed with Video Head Impulse Tests, vestibular evoked myogenic potentials, and Pure Tone Audiometry preoperatively and postoperatively. (3) Results: VHIT showed a postoperative decrease in PSC gain median (Preop. 0.86 and postop. 0.52; < 0.009). No statistically significant differences were described for the anterior semicircular canal and the lateral semicircular canal. No patient experienced new Tumarkin crisis after the surgical treatment. (4) Conclusions: Our ten years of experience with posterior semicircular canal plugging in Ménière disease patients with Tumarkin's syndrome has shown that this type of surgical procedure is successful in controlling Tumarkin's crisis, with high patient satisfaction and little worsening in hearing level.
PubMed: 38804460
DOI: 10.3390/audiolres14030036 -
Journal of Surgical Case Reports May 2024This retrospective case series evaluates treatment outcomes post-cochlear implantation in pediatric patients diagnosed with Cockayne syndrome (CS) and bilateral...
This retrospective case series evaluates treatment outcomes post-cochlear implantation in pediatric patients diagnosed with Cockayne syndrome (CS) and bilateral sensorineural hearing loss. Two female pediatric patients with CS type I underwent either bilateral or unilateral cochlear implantation. Visual reinforcement audiometry (VRA) and postoperative cochlear implant tolerance were the main outcome measures. Patient 1 demonstrated notable improvements in VRA results and school performance following bilateral implantation. Patient 2 experienced enhanced quality of life and environmental awareness post-unilateral implantation, despite a lack of objective VRA results due to developmental delay. The study underscores the benefits of cochlear implantation in CS patients, especially in patients who are post-lingual or with better cognitive function.
PubMed: 38803843
DOI: 10.1093/jscr/rjae346 -
Revista Medica de Chile Jun 2023Ototoxicity is a side effect of drugs and medications that usually leads to bilateral and symmetric sensorineural hearing loss that commonly affects the high-frequency...
BACKGROUND
Ototoxicity is a side effect of drugs and medications that usually leads to bilateral and symmetric sensorineural hearing loss that commonly affects the high-frequency range initially, with or preceded by tinnitus. Possible ototoxic side effects of calcineurin inhibitor immunosuppressants have been suggested, but this remains unclear. Therefore, this study aims to evaluate audiological changes in patients undergoing transplantation receiving immunosuppressive treatment with calcineurin inhibitors.
METHODS
Prospective cohort study. Adult patients undergoing liver or kidney transplantation treated with calcineurin inhibitors were included. Pure-tone audiometry, distortion product otoacoustic emissions, and the Tinnitus Handicap Inventory questionnaire were completed at baseline, one, three, and six months after transplantation. Hearing thresholds were compared and correlated with plasma concentrations of calcineurin inhibitors.
RESULTS
Seventeen patients were included, 59% males, with a median age of 54.7 years (29-68 years). Twelve patients underwent liver transplantation, four underwent kidney transplantation, and one patient underwent both. The medianfollow-up was 5.8 months (4-8 months). Significant pure-tone average shifts were observed in two patients. Both cases presented fluctuations in their hearing levels, which were not bilateral or symmetrical and affected the higher frequencies. All patients received tacrolimus within the therapeutic range during the follow-up period. Three different patients exceeded the expected range once; however, they were rapidly corrected and did not correlate with any changes in hearing.
CONCLUSIONS
It appears that tacrolimus does not cause hearing loss when levels are within the therapeutic range for a follow-up period of six months post-transplantation.
Topics: Humans; Male; Middle Aged; Female; Adult; Calcineurin Inhibitors; Immunosuppressive Agents; Prospective Studies; Kidney Transplantation; Aged; Liver Transplantation; Follow-Up Studies; Ototoxicity; Audiometry, Pure-Tone; Tacrolimus; Hearing Loss, Sensorineural; Time Factors
PubMed: 38801378
DOI: 10.4067/s0034-98872023000600702 -
Medicina (Kaunas, Lithuania) May 2024Besides classical stapedotomy, reverse stapedotomy has been used for many years in the management of otosclerosis. Our study aims to investigate whether reversing the...
Besides classical stapedotomy, reverse stapedotomy has been used for many years in the management of otosclerosis. Our study aims to investigate whether reversing the surgical steps in stapedotomy impacts vestibular function and hearing improvement. A cohort of 123 patients underwent either classic or reverse stapedotomy procedures utilizing a fiber-optic argon laser. Audiological assessments, following the guidelines of the Committee on Hearing and Equilibrium, were conducted, including pure tone average, air-bone (AB) gap, overclosure, and AB gap closure. Vestibular evaluation involved pre- and postoperative comparison of rotatory test parameters, including frequency, amplitude, and slow phase velocity of nystagmus. The study demonstrated an overall median overclosure of 3.3 (3.3, 5.0) dB and a mean AB gap closure of 20.3 ± 8.8 dB. Postoperative median AB gap was 7.5 (7.5, 11.3) dB in the reverse stapedotomy group and 10.0 (10.0, 12.5) dB in the classic stapedotomy group. While overclosure and AB gap closure were marginally superior in the reverse stapedotomy group, these differences did not reach statistical significance. No significant disparities were observed in the frequency, slow phase velocity, or amplitude of nystagmus in the rotational test. Although not always possible, reverse stapedotomy proved to be a safe surgical technique regarding postoperative outcomes. Its adoption may mitigate risks associated with floating footplate, sensorineural hearing loss, and incus luxation/subluxation, while facilitating the learning curve for less experienced ear surgeons.
Topics: Humans; Stapes Surgery; Male; Female; Middle Aged; Adult; Otosclerosis; Audiometry; Cohort Studies; Treatment Outcome; Audiometry, Pure-Tone; Aged; Vestibular Function Tests
PubMed: 38792986
DOI: 10.3390/medicina60050803 -
Medicina (Kaunas, Lithuania) Apr 2024Hearing loss has been recognized as a risk factor for dementia and non-motor features of Parkinson's disease (PD). The apolipoprotein E (APOE) protein contributes to...
Hearing loss has been recognized as a risk factor for dementia and non-motor features of Parkinson's disease (PD). The apolipoprotein E (APOE) protein contributes to maintenance and repair of neuronal cell membranes, causing age-related disorders. This study aimed to analyze the impact of hearing loss on cognitive impairment, PD severity, and APOE gene expression in these patients. A total of 72 out-patients diagnosed with either PD or hearing loss were enrolled in this study. The hearing assessment included pure-tone audiometry, speech reception thresholds, and speech discrimination ability. Dementia was assessed by filling out the Clinical Dementia Rating and Mini-Mental State Examination questionnaires. The severity of PD was assessed using the Modified Hoehn and Yahr scale. Blood samples were tested for the gene expression of APOE. Out of the 72 cases, there were 44 males and 28 females, with an average age of 64.4 ± 9.1 years. A total of 41 out of 72 cases had dementia and had a worse hearing threshold than those without dementia (47.1 ± 24.4 vs. 31.7 ± 22.1 dB, = 0.006). A total of 58 patients were diagnosed with PD, with 14 of them classified as having severe symptoms (Modified Hoehn and Yahr scale > 2). Patients with severe PD were found to have a worse hearing threshold (49.6 ± 28.3 vs. 30.3 ± 17.8 dB, = 0.028) and higher prevalence of dementia (12/14 vs. 18/44, = 0.006). Among 10 individuals with the APOE ε4 gene, the prevalence of dementia was higher than those without the ε4 allele (9/10 vs. 32/62, = 0.036). Hearing loss is common in severe PD and in dementia patients. Severe PD has a negative impact on the hearing threshold and cognitive dysfunction. Patients with APOE ε4 have a higher prevalence of dementia.
Topics: Humans; Female; Male; Parkinson Disease; Dementia; Middle Aged; Aged; Hearing Loss; Apolipoproteins E; Genotype; Audiometry, Pure-Tone
PubMed: 38792885
DOI: 10.3390/medicina60050703 -
Genes May 2024CHARGE syndrome, characterized by a distinct set of clinical features, has been linked primarily to mutations in the gene. Initially defined by specific clinical...
CHARGE syndrome, characterized by a distinct set of clinical features, has been linked primarily to mutations in the gene. Initially defined by specific clinical criteria, including coloboma, heart defects, choanal atresia, delayed growth, and ear anomalies, CHARGE syndrome's diagnostic spectrum has broadened since the identification of . Variants in this gene exhibit considerable phenotypic variability, leading to the adoption of the term " disorder" to encompass a wider range of associated symptoms. Recent research has identified variants in individuals with isolated features such as autism spectrum disorder or gonadotropin-releasing hormone deficiency. In this study, we present three cases from two different families exhibiting audiovestibular impairment as the primary manifestation of a variant. We discuss the expanding phenotypic variability observed in -related disorders, highlighting the importance of considering in nonsyndromic hearing loss cases, especially when accompanied by inner ear malformations on MRI. Additionally, we underscore the necessity of genetic counseling and comprehensive clinical evaluation for individuals with variants to ensure appropriate management of associated health concerns.
Topics: Humans; CHARGE Syndrome; DNA Helicases; Male; DNA-Binding Proteins; Female; Mutation; Child; Adult; Phenotype; Pedigree; Child, Preschool; Adolescent
PubMed: 38790272
DOI: 10.3390/genes15050643 -
Genes May 2024Hearing impairment, a rare inherited condition, is notably prevalent in populations with high rates of consanguinity. The most common form observed globally is autosomal...
Hearing impairment, a rare inherited condition, is notably prevalent in populations with high rates of consanguinity. The most common form observed globally is autosomal recessive non-syndromic hearing loss. Despite its prevalence, this genetic disorder is characterized by a substantial genetic diversity, making diagnosis and screening challenging. The emergence of advanced next-generation sequencing (NGS) technologies has significantly advanced the discovery of genes and variants linked to various conditions, such as hearing loss. In this study, our objective was to identify the specific variant causing hearing loss in a family from Syria using clinical exome sequencing. The proband in the family exhibited profound deafness as shown by pure-tone audiometry results. The analysis of the different variants obtained by NGS revealed the presence of a nonsense mutation within the gene. Through Sanger sequencing, we verified that this variant segregates with the disease and was not present in the control population. Moreover, we conducted a comprehensive review of all reported deafness-related mutations and their associated phenotypes. Furthermore, we endeavored to carry out a comparative analysis between the and genes, with the objective of identifying potential factors that could explain the notable discrepancy in mutation frequency between these two genes.
Topics: Humans; Male; Female; Connexin 26; Syria; Deafness; Phenotype; Claudins; Pedigree; Mutation; Exome Sequencing; Adult; Codon, Nonsense; Connexins
PubMed: 38790217
DOI: 10.3390/genes15050588 -
EBioMedicine May 2024Hearing impairment is a common condition in the elderly. However, a comprehensive understanding of its neural correlates is still lacking.
BACKGROUND
Hearing impairment is a common condition in the elderly. However, a comprehensive understanding of its neural correlates is still lacking.
METHODS
We recruited 284 elderly adults who underwent structural MRI, magnetic resonance spectroscopy, audiometry, and cognitive assessments. Individual hearing abilities indexed by pure tone average (PTA) were correlated with multiple structural MRI-derived cortical morphological indices. For regions showing significant correlations, mediation analyses were performed to examine their role in the relationship between hearing ability and cognitive function. Finally, the correlation maps between hearing ability and cortical morphology were linked with publicly available connectomic gradient, transcriptomic, and neurotransmitter maps.
FINDINGS
Poorer hearing was related to cortical thickness (CT) reductions in widespread regions and gyrification index (GI) reductions in the right Area 52 and Insular Granular Complex. The GI in the right Area 52 mediated the relationship between hearing ability and executive function. This mediating effect was further modulated by glutamate and N-acetylaspartate levels in the right auditory region. The PTA-CT correlation map followed microstructural connectomic hierarchy, were related to genes involved in certain biological processes (e.g., glutamate metabolic process), cell types (e.g., excitatory neurons and astrocytes), and developmental stages (i.e., childhood to young adulthood), and covaried with dopamine receptor 1, dopamine transporter, and fluorodopa. The PTA-GI correlation map was related to 5-hydroxytryptamine receptor 2a.
INTERPRETATION
Poorer hearing is associated with cortical thinning and folding reductions, which may be engaged in the relationship between hearing impairment and cognitive decline in the elderly and have different neurobiological substrates.
FUNDING
See the Acknowledgements section.
PubMed: 38788630
DOI: 10.1016/j.ebiom.2024.105160 -
Tobacco Induced Diseases 2024Although conventional cigarette smoking has been linked to an increased risk of hearing loss, the association between heated tobacco products (HTPs) and hearing loss is...
INTRODUCTION
Although conventional cigarette smoking has been linked to an increased risk of hearing loss, the association between heated tobacco products (HTPs) and hearing loss is unknown. The objective of this study was to investigate the association between cigarette and HTP use and hearing loss.
METHODS
This cross-sectional study examined the data of 7769 employees from five companies (Study I) and 34404 employees from a large company (Study II), all participants in the Japan Epidemiology Collaboration on Occupational Health Study. The participants were categorized into five groups based on their self-reported tobacco use: never smokers, former smokers, exclusive cigarette smokers, exclusive users of HTPs, and those who used both cigarettes and HTPs. Hearing levels were measured using pure-tone audiometry at 1 and 4 kHz frequencies. Separate analyses were carried out for each study, and the results were then combined using fixed-effect models to pool the estimates.
RESULTS
The analysis included 42173 participants, with a prevalence of 12.9% for exclusive cigarette smoking, 9.8% for exclusive HTP use, and 5.5% for dual use. The pooled adjusted odds ratios with 95% confidence intervals for unilateral hearing loss at 4 kHz were 1.21 (95% CI: 1.10-1.33) for former smokers, 1.83 (95% CI: 1.64-2.05) for exclusive cigarette smokers,1.46 (95% CI: 1.28-1.67) for exclusive HTP users, and 1.66 (95% CI: 1.41-1.96) for dual users, compared to never smokers. Additionally, the adjusted odds ratios for hearing loss at 4 kHz among exclusive cigarette smokers, exclusive HTP users, and dual users increased with the intensity of cigarette/HTP consumption (all p for trend <0.001). No significant associations were found between exclusive HTP use, dual use, and hearing loss at 1 kHz, apart from exclusive cigarette smoking.
CONCLUSIONS
In this cross-sectional study, associations were found between exclusive cigarette smoking, exclusive HTP use, dual use, and hearing loss, particularly at 4 kHz. Further research is needed to confirm these findings.
PubMed: 38783967
DOI: 10.18332/tid/187302 -
Frontiers in Artificial Intelligence 2024This research investigates the application of machine learning to improve the diagnosis of tinnitus using high-frequency audiometry data. A Logistic Regression (LR)...
This research investigates the application of machine learning to improve the diagnosis of tinnitus using high-frequency audiometry data. A Logistic Regression (LR) model was developed alongside an Artificial Neural Network (ANN) and various baseline classifiers to identify the most effective approach for classifying tinnitus presence. The methodology encompassed data preprocessing, feature extraction focused on point detection, and rigorous model evaluation through performance metrics including accuracy, Area Under the ROC Curve (AUC), precision, recall, and F1 scores. The main findings reveal that the LR model, supported by the ANN, significantly outperformed other machine learning models, achieving an accuracy of 94.06%, an AUC of 97.06%, and high precision and recall scores. These results demonstrate the efficacy of the LR model and ANN in accurately diagnosing tinnitus, surpassing traditional diagnostic methods that rely on subjective assessments. The implications of this research are substantial for clinical audiology, suggesting that machine learning, particularly advanced models like ANNs, can provide a more objective and quantifiable tool for tinnitus diagnosis, especially when utilizing high-frequency audiometry data not typically assessed in standard hearing tests. The study underscores the potential for machine learning to facilitate earlier and more accurate tinnitus detection, which could lead to improved patient outcomes. Future work should aim to expand the dataset diversity, explore a broader range of algorithms, and conduct clinical trials to validate the models' practical utility. The research highlights the transformative potential of machine learning, including the LR model and ANN, in audiology, paving the way for advancements in the diagnosis and treatment of tinnitus.
PubMed: 38774833
DOI: 10.3389/frai.2024.1381455