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1.
Sci Rep ; 14(1): 15655, 2024 Jul 08.
Article in English | MEDLINE | ID: mdl-38977724

ABSTRACT

Fear and anxiety among patients are sometimes evoked in dental clinics due to the sound of dental drills. This study aimed to explore the impact of age-related hearing loss in the extended high frequency (EHF) range above 8 kHz on individuals' subjective discomfort towards dental drill noise. After measuring pure-tone audiometric thresholds at both conventional and extended high frequencies, we used a psychoacoustic approach to evaluate subjective impressions of four dental drill sound stimuli, which featured varying frequency components, in 62 participants (aged 12-67 years). We found a significant decrease in hearing sensitivity within the EHF range as age increased, with notable differences in hearing thresholds at 14 kHz between teenage and older adults exceeding 65 dB. Furthermore, significant differences were observed between younger and older (above 40 years) participants in the subjective impressions of dental drill noise, emphasizing age as a critical factor in the perception of high frequency components. Consequently, age may influence the unpleasantness of dental drilling noise. Compared to older individuals, young participants may exhibit increased fear of dental procedures owing to physiological factors. These results underscore the need for age-appropriate noise control strategies in dental clinics to mitigate anxiety and improve patient comfort.


Subject(s)
Noise , Humans , Adult , Middle Aged , Aged , Male , Female , Adolescent , Young Adult , Child , Noise/adverse effects , Auditory Threshold/physiology , Age Factors , Hearing Loss, Noise-Induced/physiopathology , Hearing Loss, Noise-Induced/etiology , Audiometry, Pure-Tone
2.
World J Clin Cases ; 12(19): 3760-3766, 2024 Jul 06.
Article in English | MEDLINE | ID: mdl-38994283

ABSTRACT

BACKGROUND: Numerous studies have found that patients experiencing sudden sensorineural hearing loss (SSHL), with or without accompanying vertigo, often show impaired vestibular function. However, there is a dearth of studies analyzing vestibular-evoked myogenic potentials (VEMPs) in SSHL patients across various age groups. AIM: To investigate vestibular condition in SSHL patients across various age demographics. METHODS: Clinical data of 84 SSHL patients were investigated retrospectively. Audiometry, cervical vestibular evoked myogenic potentials (c-VEMPs), and ocular vestibular evoked myogenic potentials (o-VEMPs) were conducted on these patients. Parameters assessed included the latencies of P1 and N1 waves, as well as the amplitudes of P1-N1 waves. Moreover, the study evaluated the influence of factors such as sex, affected side, configuration of hearing loss, and presence of accompanying vertigo. RESULTS: Among the 84 SSHL patients, no significant differences were observed among the three groups in terms of gender, affected side, and the presence or absence of vertigo. Group II (aged 41-60 years) had the highest number of SSHL cases. The rates of absent o-VEMPs in the affected ears were 20.83%, 31.58%, and 22.72% for the three age groups, respectively, with no statistically significant difference among them. The rates of absent c-VEMPs in the affected ears were 8.3%, 34.21%, and 18.18% for the three age groups, respectively, with significant differences. In the unaffected ears, there were differences observed in the extraction rates of o-VEMPs in the unaffected ears among the age groups. In the three age groups, no significant differences were noted in the three age groups in the latencies of P1 and N1 waves or in the amplitude of N1-P1 waves for c-VEMPs and o-VEMPs, either on the affected side or on the unaffected side, across the three age groups. CONCLUSION: The extraction rate of VEMPs is more valuable than parameters. Regardless of the presence of vertigo, vestibular organs are involved in SSHL. Notably, SSHL patients aged 41-60 appear more susceptible to damage to the inferior vestibular nerve and saccule.

3.
J Audiol Otol ; 2024 Jul 09.
Article in English | MEDLINE | ID: mdl-38973326

ABSTRACT

Background and Objectives: : Hearing loss is the largest potentially modifiable risk factor for dementia. Early evaluation and intervention are crucial for older adults with cognitive impairment. However, pure-tone audiometry (PTA), the gold standard, may have limitations. This study reviewed auditory tests for detecting hearing loss in this population, comparing to PTA. Materials and Methods: Following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, we conducted a systematic review in PubMed, Scopus, and Embase. Ten studies (1,071 participants) comparing auditory tests and PTA in patients with dementia and mild cognitive impairment were included. Results: : Electrophysiological and behavioral tests demonstrated strong correlation and accurate hearing impairment detection compared to PTA. Conversely, self-reported assessment showed weaker correlations when aligned with hearing thresholds. Conclusions: : This study highlights the potential of electrophysiological and behavioral auditory tests in detecting hearing impairment in older adults with cognitive impairment, emphasizing the need for further research to develop practical screening protocols for this vulnerable population.

4.
BMC Psychiatry ; 24(1): 459, 2024 Jun 19.
Article in English | MEDLINE | ID: mdl-38898451

ABSTRACT

BACKGROUND: Tinnitus affects approximately 740 million adults globally, involving hearing, emotion, and sleep systems. However, studies using polysomnography and pure-tone audiometry (PTA) are limited. We aimed to assess the correlation between tinnitus and hearing, sleep quality, characteristics, and depression using polysomnography and PTA. METHODS: In this cross-sectional study, we divided participants into tinnitus and non-tinnitus groups. We included 100 outpatients (65 with tinnitus, 35 without) from a medical center in Taiwan, who underwent polysomnography and completed rating scales including the Patient Health Questionnaire-9 (PHQ-9), Chinese version of the Pittsburgh Sleep Quality Index (PSQI), and Chinese-Mandarin version of the Tinnitus Handicap Inventory (THI-CM). We analyzed correlations, conducted group comparisons, assessed factors related to THI-CM scores, constructed ROC curves to predict depression in the tinnitus group, and performed multinomial and logistic regression to explore associations. RESULTS: Descriptive statistics identified a cohort with mean age 53.9 ± 12.80 years, 63% exhibited PHQ-9 scores ≥ 10, and 66% had Apnea-Hypopnea Index (AHI) > 5. The ratio of rapid eye movement and deep sleep to stage 1 + 2 sleep was relatively low and non-significant. Likewise, leg movements was higher in the tinnitus group but not statistically significant. In the tinnitus group, 63.08% had depression, and 81.54% had AHI > 5. Univariate logistic regression linked tinnitus to AHI > 5 (Odds ratio (OR) 2.67, p = 0.026) and male sex (OR 2.49, p = 0.034). A moderate positive correlation was found between the THI-CM score and PHQ-9 score (rs = 0.50, p < 0.001). Further adjustment for obstructive sleep apnea showed associations between PHQ-9 (total score) or depression and THI-CM Grade 3-5 (OR = 1.28; OR = 8.68). Single- and multifactor regression analyses highlighted significant associations of PSQI scores > 13 (OR 7.06, p = 0.018) and THI-CM scores > 47 (OR 7.43, p = 0.002) with depression. CONCLUSIONS: Our study recruited tinnitus participants with slight or mild hearing loss and mild tinnitus handicap. Depression was identified as a predominant factor in tinnitus-related handicap. The mild tinnitus handicap in tinnitus participants may explain the lack of significant differences in depression, sleep quality, and polysomnographic sleep characteristics between tinnitus and non-tinnitus groups. Further extensive and prospective studies are needed to elucidate the complex links among depression, sleep, and tinnitus.


Subject(s)
Audiometry, Pure-Tone , Polysomnography , Sleep Quality , Tinnitus , Humans , Male , Female , Tinnitus/complications , Tinnitus/psychology , Tinnitus/diagnosis , Middle Aged , Cross-Sectional Studies , Adult , Aged , Taiwan , Depression/complications , Depression/diagnosis
5.
Trends Hear ; 28: 23312165241259704, 2024.
Article in English | MEDLINE | ID: mdl-38835268

ABSTRACT

The use of in-situ audiometry for hearing aid fitting is appealing due to its reduced resource and equipment requirements compared to standard approaches employing conventional audiometry alongside real-ear measures. However, its validity has been a subject of debate, as previous studies noted differences between hearing thresholds measured using conventional and in-situ audiometry. The differences were particularly notable for open-fit hearing aids, attributed to low-frequency leakage caused by the vent. Here, in-situ audiometry was investigated for six receiver-in-canal hearing aids from different manufacturers through three experiments. In Experiment I, the hearing aid gain was measured to investigate whether corrections were implemented to the prescribed target gain. In Experiment II, the in-situ stimuli were recorded to investigate if corrections were directly incorporated to the delivered in-situ stimulus. Finally, in Experiment III, hearing thresholds using in-situ and conventional audiometry were measured with real patients wearing open-fit hearing aids. Results indicated that (1) the hearing aid gain remained unaffected when measured with in-situ or conventional audiometry for all open-fit measurements, (2) the in-situ stimuli were adjusted for up to 30 dB at frequencies below 1000 Hz for all open-fit hearing aids except one, which also recommends the use of closed domes for all in-situ measurements, and (3) the mean interparticipant threshold difference fell within 5 dB for frequencies between 250 and 6000 Hz. The results clearly indicated that modern measured in-situ thresholds align (within 5 dB) with conventional thresholds measured, indicating the potential of in-situ audiometry for remote hearing care.


Subject(s)
Auditory Threshold , Hearing Aids , Humans , Acoustic Stimulation , Prosthesis Fitting/methods , Reproducibility of Results , Audiometry/methods , Audiometry, Pure-Tone , Hearing Loss/diagnosis , Hearing Loss/rehabilitation , Hearing Loss/physiopathology , Hearing , Predictive Value of Tests , Persons With Hearing Impairments/rehabilitation , Persons With Hearing Impairments/psychology , Equipment Design , Male , Female
6.
Work ; 2024 Jun 04.
Article in English | MEDLINE | ID: mdl-38848149

ABSTRACT

BACKGROUND: Varied noise environments, such as impulse noise and steady-state noise, may induce distinct patterns of hearing impairment among personnel exposed to prolonged noise. However, comparative studies on these effects remain limited. OBJECTIVE: This study aims to delineate the different characteristics of hearing loss in workers exposed to steady-state noise and impulse noise. METHODS: As of December 2020, 96 workers exposed to steady-state noise and 177 workers exposed to impulse noise were assessed. Hearing loss across various frequencies was measured using pure tone audiometry and distortion product otoacoustic emission (DPOAE) audiometry. RESULTS: Both groups of workers exposed to steady-state noise and impulse noise exhibited high frequencies hearing loss. The steady-state noise group displayed significantly greater hearing loss at lower frequencies in the early stages, spanning 1- 5 years of work (P < 0.05). Among individuals exposed to impulse noise for extended periods (over 10 years), the observed hearing loss surpassed that of the steady-state noise group, displaying a statistically significant difference (P < 0.05). CONCLUSION: Hearing loss resulting from both steady-state noise and impulse noise predominantly occurs at high frequencies. Early exposure to steady-state noise induces more pronounced hearing loss at speech frequencies compared to impulse noise.

7.
J Appl Stat ; 51(8): 1609-1617, 2024.
Article in English | MEDLINE | ID: mdl-38863801

ABSTRACT

In this paper, we consider the estimation of intracluster correlation for ordinal data. We focus on pure-tone audiometry hearing threshold data, where thresholds are measured in 5 decibel increments. We estimate the intracluster correlation for tests from iPhone-based hearing assessment applications as a measure of test/retest reliability. We present a method to estimate the intracluster correlation using mixed effects cumulative logistic and probit models, which assume the outcome data are ordinal. This contrasts with using a mixed effects linear model which assumes that the outcome data are continuous. In simulation studies, we show that using a mixed effects linear model to estimate the intracluster correlation for ordinal data results in a negative finite sample bias, while using mixed effects cumulative logistic or probit models reduces this bias. The estimated intracluster correlation for the iPhone-based hearing assessment application is higher when using the mixed effects cumulative logistic and probit models compared to using a mixed effects linear model. When data are ordinal, using mixed effects cumulative logistic or probit models reduces the bias of intracluster correlation estimates relative to using a mixed effects linear model.

8.
Trends Hear ; 28: 23312165241260041, 2024.
Article in English | MEDLINE | ID: mdl-38870447

ABSTRACT

Almost since the inception of the modern-day electroacoustic audiometer a century ago the results of pure-tone audiometry have been characterized by an audiogram. For almost as many years, clinicians and researchers have sought ways to distill the volume and complexity of information on the audiogram. Commonly used approaches have made use of pure-tone averages (PTAs) for various frequency ranges with the PTA for 500, 1000, 2000 and 4000 Hz (PTA4) being the most widely used for the categorization of hearing loss severity. Here, a three-digit triad is proposed as a single-number summary of not only the severity, but also the configuration and bilateral symmetry of the hearing loss. Each digit in the triad ranges from 0 to 9, increasing as the level of the pure-tone hearing threshold level (HTL) increases from a range of optimal hearing (< 10 dB Hearing Level; HL) to complete hearing loss (≥ 90 dB HL). Each digit also represents a different frequency region of the audiogram proceeding from left to right as: (Low, L) PTA for 500, 1000, and 2000 Hz; (Center, C) PTA for 3000, 4000 and 6000 Hz; and (High, H) HTL at 8000 Hz. This LCH Triad audiogram-classification system is evaluated using a large United States (U.S.) national dataset (N = 8,795) from adults 20 to 80 + years of age and two large clinical datasets totaling 8,254 adults covering a similar age range. Its ability to capture variations in hearing function was found to be superior to that of the widely used PTA4.


Subject(s)
Audiometry, Pure-Tone , Auditory Threshold , Hearing Loss , Adult , Aged , Aged, 80 and over , Female , Humans , Male , Middle Aged , Young Adult , Acoustic Stimulation , Auditory Threshold/physiology , Hearing/physiology , Hearing Loss/diagnosis , Hearing Loss/classification , Hearing Loss/physiopathology , Predictive Value of Tests , Reproducibility of Results , Severity of Illness Index
9.
Diagnostics (Basel) ; 14(12)2024 Jun 12.
Article in English | MEDLINE | ID: mdl-38928645

ABSTRACT

Eustachian tube dysfunction (ETD) affects a significant portion of the population, manifesting symptoms that impact the quality of life. Despite the prevalence of ETD, there remains a notable gap in comprehensive studies exploring the condition's dynamics within specific demographic contexts, particularly within Saudi Arabia. This study aimed to assess the prevalence and severity of ETD across different demographic groups, to evaluate the efficacy of various treatment modalities, and to identify key predictors of treatment response in a Saudi Arabian cohort. A cross-sectional study was conducted from June 2022 to May 2023 in tertiary care hospitals in the Aseer region, Saudi Arabia. Participants included adults diagnosed with ETD, assessed through clinical symptoms, otoscopic examinations, audiometric evaluations, tympanometry, and the ETDQ-7 questionnaire. The study incorporated advanced diagnostics such as nasopharyngoscopy and pressure equalization tube function tests and involved 154 participants, revealing significant variations in ETD severity, with the 46-60 age group exhibiting the highest mean ETDQ-7 score of 4.85, and urban residents displaying lower severity scores compared to rural counterparts. Pharmacological interventions were most effective, achieving the highest symptom relief and audiological improvement rates of 87.78%. Multivariate regression highlighted age, geographic location, and treatment modality as key predictors of treatment efficacy, with notable interaction effects between climate conditions and treatment types influencing outcomes. The findings underscore the heterogeneity in ETD presentation and the differential efficacy of treatment modalities.

10.
Sci Rep ; 14(1): 14203, 2024 06 20.
Article in English | MEDLINE | ID: mdl-38902305

ABSTRACT

Hearing problems are commonly diagnosed with the use of tonal audiometry, which measures a patient's hearing threshold in both air and bone conduction at various frequencies. Results of audiometry tests, usually represented graphically in the form of an audiogram, need to be interpreted by a professional audiologist in order to determine the exact type of hearing loss and administer proper treatment. However, the small number of professionals in the field can severely delay proper diagnosis. The presented work proposes a neural network solution for classification of tonal audiometry data. The solution, based on the Bidirectional Long Short-Term Memory architecture, has been devised and evaluated for classifying audiometry results into four classes, representing normal hearing, conductive hearing loss, mixed hearing loss, and sensorineural hearing loss. The network was trained using 15,046 test results analysed and categorised by professional audiologists. The proposed model achieves 99.33% classification accuracy on datasets outside of training. In clinical application, the model allows general practitioners to independently classify tonal audiometry results for patient referral. In addition, the proposed solution provides audiologists and otolaryngologists with access to an AI decision support system that has the potential to reduce their burden, improve diagnostic accuracy, and minimise human error.


Subject(s)
Audiometry, Pure-Tone , Neural Networks, Computer , Humans , Audiometry, Pure-Tone/methods , Female , Male , Hearing Loss/diagnosis , Hearing Loss/classification , Adult , Middle Aged , Hearing Loss, Sensorineural/diagnosis , Hearing Loss, Sensorineural/classification , Hearing Loss, Sensorineural/physiopathology , Hearing Loss, Conductive/diagnosis , Hearing Loss, Conductive/classification
11.
Indian J Otolaryngol Head Neck Surg ; 76(3): 2684-2689, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38883463

ABSTRACT

Aims: In this study, we attempt to compare the pure tone audiometry findings with ossicular chain status intraoperatively in patients of chronic otitis media. Materials and methods: 102 patients who presented with COM during a period of one year and met the inclusion criteria were included in the study. All patients underwent preoperative pure tone Audiometry and findings were tabulated. All patients were evaluated intraoperatively by the same surgeon and observations were made regarding ossicular chain integrity. Results: Small central perforation was noted in 10%, medium central perforation in 38.57%, large central perforation in 27.14% and subtotal perforation in 24.28%. 71.56% patients had an intact ossicular chain, of which 94.5% was mucosal disease and 5.5% were squamous disease. 29 cases showed eroded/absent ossicles, out of which, 28 had squamous type and 1 case had central perforation. Ossicular status was classified based on Austin Classification. Conductive hearing loss was found to be maximum where all 3 ossicles were eroded/absent, with a mean AB gap of 45.33 and mean air conduction threshold of 60.33. Conclusion: There is a good correlation between the hearing threshold of the patient and the status of ossicular chain. Preoperative knowledge of the degree of hearing loss and status of ossicular chain would allow the surgeon to plan proper ossicular reconstruction and give the patient a better advice regarding prognosis of hearing improvement after surgery.

12.
Indian J Otolaryngol Head Neck Surg ; 76(3): 2367-2372, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38883469

ABSTRACT

To comprehensively understand the manifestation, treatments, and resultant consequences of temporal bone fractures, given their substantial impact on auditory and balance systems and the overall quality of life. A prospective study, adhering to the STROBE guidelines, spanning five years (2011-2015) was conducted on 83 male patients aged between 20 and 54 years, diagnosed with temporal bone fractures primarily caused by road traffic accidents. Evaluations comprised symptom presentation, otologic manifestations, radiological classifications, and management strategies, including both conservative and surgical interventions. Our study found that patients commonly presented with symptoms such as Oto-haematorrhoea, hearing impairment, and vertigo. Specifically, longitudinal fractures were the most frequent radiological finding, occurring in 63 cases (p < 0.001 for road traffic accidents). In terms of treatment outcomes, there was a notable improvement in the average hearing threshold, decreasing from 50 dB to 25 dB post-treatment (p < 0.001), and the air-bone gap reduced from 30 dB to 10 dB (p < 0.001). Audiometric outcomes varied significantly with fracture type, showing severe hearing loss was more common in transverse fractures (50%, p < 0.001) compared to longitudinal and mixed fractures. Additionally, the study revealed a significant reduction in the incidence of post-trauma vertigo over eight weeks (p < 0.001), underscoring the importance of early and appropriate intervention in managing temporal bone fractures. Efficient early detection and tailored interventions for temporal bone fractures lead to optimistic results. This research underscores the imperative for healthcare practitioners to adopt a comprehensive approach, from initial diagnosis to ongoing monitoring, to achieve optimal patient care. Supplementary Information: The online version contains supplementary material available at 10.1007/s12070-024-04519-9.

13.
Indian J Otolaryngol Head Neck Surg ; 76(3): 2919-2921, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38883507

ABSTRACT

Hearing loss is a highly prevalent condition worldwide, affecting over 5% of the global population. Determining disability and eligibility for rehabilitation services due to hearing loss is complex, as countries employ differing audiometric criteria and methods. This report reviews current literature on audiometric thresholds used globally to determine hearing disability, highlighting challenging cases worldwide. Databases PubMed, Embase, and Web of Science were searched for articles on hearing disability criteria published from 2010-present in English. Overall, developed countries employ a pure-tone average (PTA) of ≥ 40 dB as disability criteria, while developing countries use ≥ 41 dB PTA. The World Health Organization uses ≥ 41 dB disabling hearing loss in better ear. Studies show widespread use of a single frequency threshold in some countries can exclude milder losses. Individualized approaches accounting for communication function, rather than fixed PTA cut-offs alone, enable more accurate disability determination. Further research on optimal, equitable criteria accounting for resource availability is needed. Standardized guidelines balancing sensitivity and specificity in disability determination worldwide would enable improved rehabilitation access and outcomes.

14.
Clin Exp Otorhinolaryngol ; 17(2): 109-115, 2024 May.
Article in English | MEDLINE | ID: mdl-38711305

ABSTRACT

OBJECTIVES: A primary reason for the low adoption of hearing aids (HAs) among the large population with sensorineural hearing loss is the perception that these devices may negatively impact remaining hearing ability. Research addressing this issue has yielded conflicting results. This study examined the long-term effects of HAs on standard audiometric changes in individuals with sensorineural hearing loss. METHODS: We retrospectively analyzed patients who acquired unilateral HAs between 2015 and 2017 and demonstrated consistent use over a 5-year period. We examined demographics, medical comorbidities, audiometric data, and questionnaire results from the Hearing Handicap Inventory for the Elderly and the International Outcome Inventory for Hearing Aids. Additionally, we reviewed each patient's history of noise exposure and prior HA use. RESULTS: The study included 55 patients who used unilateral HAs, with a mean follow-up period of 5.32 years. Among them, 31 patients (56.4%) used the HA on the right side. Audiometric data from the aided side showed no significant difference from the unaided side in either the pre-fit pure-tone average of air conduction (AC) or word recognition score (WRS) (P =0.73 and P =0.11, respectively). Similarly, no significant differences were noted in the 5-year follow-up audiometry of AC and WRS (P=0.98 and P=0.07, respectively) or in the change from pre-fit to final audiometry for either parameter (AC, P=0.58; WRS, P=0.70). Eleven patients (20%) exhibited a deterioration in hearing (as measured by AC) of 5 dB or greater on the aided side compared with the unaided side, while 23 (53.5%) showed greater WRS deterioration on the aided side. No significant factors were found to contribute to the difference in hearing deterioration between groups for either AC or WRS. CONCLUSION: No significant factors were identified as contributing to hearing deterioration after prolonged HA use. Overall, the use of HAs did not adversely impact residual hearing.

15.
Int J Pediatr Otorhinolaryngol ; 181: 111982, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38776720

ABSTRACT

OBJECTIVE: To describe the prevalence of routine white noise exposure in children who undergo sound field audiometry. METHODS: A retrospective cohort study was conducted at a pediatric otolaryngology clinic affiliated with an academic tertiary care hospital. The medical records of children who underwent sound field audiometry were reviewed and data was collected regarding demographics and audiogram results. The group was divided into two cohorts based on routine exposure to white noise. Children exposed to white noise were tested with warble tones, while those not exposed were tested with narrow-band noise. RESULTS: 127 patients underwent sound field audiometry testing, of which 96 (75.6%) were reported by their parents to use white noise for sleep. The mean age at time of testing was 1.6 years (95% Confidence Interval [CI] 1.5-1.7). 104 (81.9%) children were able to respond to at least four of the sound field thresholds, and there was no significant difference between the children exposed to white noise and those who were not (P = 0.459). Mean pure tone average (PTA) was 26.2 dB (95% CI 25.2-27.2) and mean speech reception threshold (SRT) was 19.2 dB (95% CI 18.2-20.2). The sound field response rate, PTA, and SRT were similar between these two groups. CONCLUSIONS: The routine use of white noise therapy was extremely common in this pediatric population. The use of warble tones as the audiometric stimuli for children exposed to white noise resulted in similar testing success compared to the use of narrow-band noise in children not exposed to white noise.


Subject(s)
Audiometry, Pure-Tone , Noise , Humans , Male , Female , Retrospective Studies , Noise/adverse effects , Infant , Child, Preschool , Hearing Loss, Noise-Induced/diagnosis , Hearing Loss, Noise-Induced/epidemiology , Hearing Loss, Noise-Induced/etiology , Auditory Threshold , Cohort Studies , Child , Audiometry/methods , Prevalence
16.
Genes (Basel) ; 15(5)2024 05 19.
Article in English | MEDLINE | ID: mdl-38790272

ABSTRACT

CHARGE syndrome, characterized by a distinct set of clinical features, has been linked primarily to mutations in the CHD7 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 CHD7. Variants in this gene exhibit considerable phenotypic variability, leading to the adoption of the term "CHD7 disorder" to encompass a wider range of associated symptoms. Recent research has identified CHD7 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 CHD7 variant. We discuss the expanding phenotypic variability observed in CHD7-related disorders, highlighting the importance of considering CHD7 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 CHD7 variants to ensure appropriate management of associated health concerns.


Subject(s)
CHARGE Syndrome , DNA Helicases , DNA-Binding Proteins , Humans , CHARGE Syndrome/genetics , CHARGE Syndrome/diagnosis , DNA Helicases/genetics , Male , DNA-Binding Proteins/genetics , Female , Mutation , Child , Adult , Phenotype , Pedigree , Child, Preschool , Adolescent
17.
Article in English | MEDLINE | ID: mdl-38782831

ABSTRACT

PURPOSE: Age-related hearing loss is the most common form of permanent hearing loss that is associated with various health traits, including Alzheimer's disease, cognitive decline, and depression. The present study aims to identify genetic comorbidities of age-related hearing loss. Past genome-wide association studies identified multiple genomic loci involved in common adult-onset health traits. Polygenic risk scores (PRS) could summarize the polygenic inheritance and quantify the genetic susceptibility of complex traits independent of trait expression. The present study conducted a PRS-based association analysis of age-related hearing difficulty in the UK Biobank sample (N = 425,240), followed by a replication analysis using hearing thresholds (HTs) and distortion-product otoacoustic emissions (DPOAEs) in 242 young adults with self-reported normal hearing. We hypothesized that young adults with genetic comorbidities associated with age-related hearing difficulty would exhibit subclinical decline in HTs and DPOAEs in both ears. METHODS: A total of 111,243 participants reported age-related hearing difficulty in the UK Biobank sample (> 40 years). The PRS models were derived from the polygenic risk score catalog to obtain 2627 PRS predictors across the health spectrum. HTs (0.25-16 kHz) and DPOAEs (1-16 kHz, L1/L2 = 65/55 dB SPL, F2/F1 = 1.22) were measured on 242 young adults. Saliva-derived DNA samples were subjected to low-pass whole genome sequencing, followed by genome-wide imputation and PRS calculation. The logistic regression analyses were performed to identify PRS predictors of age-related hearing difficulty in the UK Biobank cohort. The linear mixed model analyses were performed to identify PRS predictors of HTs and DPOAEs. RESULTS: The PRS-based association analysis identified 977 PRS predictors across the health spectrum associated with age-related hearing difficulty. Hearing difficulty and hearing aid use PRS predictors revealed the strongest association with the age-related hearing difficulty phenotype. Youth with a higher genetic predisposition to hearing difficulty revealed a subclinical elevation in HTs and a decline in DPOAEs in both ears. PRS predictors associated with age-related hearing difficulty were enriched for mental health, lifestyle, metabolic, sleep, reproductive, digestive, respiratory, hematopoietic, and immune traits. Fifty PRS predictors belonging to various trait categories were replicated for HTs and DPOAEs in both ears. CONCLUSION: The study identified genetic comorbidities associated with age-related hearing loss across the health spectrum. Youth with a high genetic predisposition to age-related hearing difficulty and other related complex traits could exhibit sub-clinical decline in HTs and DPOAEs decades before clinically meaningful age-related hearing loss is observed. We posit that effective communication of genetic risk, promoting a healthy lifestyle, and reducing exposure to environmental risk factors at younger ages could help prevent or delay the onset of age-related hearing difficulty at older ages.

18.
HNO ; 2024 May 02.
Article in German | MEDLINE | ID: mdl-38695898

ABSTRACT

BACKGROUND: The diagnosis of third window syndromes often poses a challenge in clinical practice. OBJECTIVE: This paper provides an up-to-date overview of diagnostic procedures in third window syndromes, with special emphasis on superior canal dehiscence syndrome (SCDS), large vestibular aqueduct syndrome (LVAS), and X-chromosomal malformation of the cochlea. MATERIALS AND METHODS: A literature search was performed in PubMed up to December 2023. Furthermore, a selection of the authors' own cases is presented. RESULTS: Audiovestibular tests for the diagnosis of third window syndromes are most often reported for patients with SCDS in the literature. In this context, cut-off values with different sensitivities and specificities have been defined for different outcome parameters of vestibular evoked myogenic potentials. Current developments include the application of electrocochleography, broadband tympanometry, video head impulse testing, and vibration-induced nystagmus. Genetic analyses are increasingly applied in LVAS. CONCLUSION: The diagnosis of third window syndromes is always based on the synthesis of patients' symptoms, clinical signs, audiovestibular test results, and imaging.

19.
Front Artif Intell ; 7: 1381455, 2024.
Article in English | MEDLINE | ID: mdl-38774833

ABSTRACT

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.

20.
Laryngoscope ; 2024 May 29.
Article in English | MEDLINE | ID: mdl-38808803

ABSTRACT

OBJECTIVE: Although corticosteroids and other treatments for idiopathic sudden sensorineural hearing loss (ISSNHL) have been described, understanding its prognosis without intervention provides valuable information for patient management. The objective of this study is to provide a comprehensive, quantitative statistical analysis of the natural history of untreated idiopathic sudden sensorineural hearing loss (ISSNHL). STUDY TYPE AND DESIGN: A systematic review and meta-analyses. METHODS: Two independent searches of PubMed, Scopus, Web of Science, and Cochrane Library databases up to June 30, 2022, were performed. Adults with idiopathic sudden sensorineural hearing loss who received placebo or were untreated and had audiometric outcome measures in all study types were reviewed. These data indicative of the natural history of ISSNHL were analyzed, as were study characteristics related to risk of bias. Heterogeneity as assessed via I2 and random effects analyses were performed. RESULTS: Six studies meeting the inclusion criteria yielded 319 untreated patients whose natural history could be assessed. Heterogeneity among studies was moderate, with a variety of reported outcomes. A hearing improvement of at least 30 dB HL was observed in 36% (95% CI 0.28-0.44) of untreated patients, and of at least 10 dB HL was observed in 70% (95% CI 0.57-0.82) of untreated patients at 3 months. The mean hearing gain among untreated patients was 24.0 dB HL (95% CI 2.65-45.37) at 2-3 months. CONCLUSIONS: The observed natural history of ISSNHL suggests that patients can regain some hearing without active treatment. In the absence of future studies collecting prospective natural history data from untreated or placebo-treated ISSNHL patients, the data presented here provide the best available historical control data for reconsideration of results in past ISSNHL studies, as well as a roadmap for design and interpretation of future ISSNHL treatment clinical trials. Furthermore, knowing there is a statistically significant mean hearing gain of 24.0 dB HL in the untreated/placebo group provides an ethical basis for future placebo study of ISSNHL. The current status on ISSNHL management calls for a multi-institutional, randomized, double-blind placebo-controlled trial with validated outcome measures to provide science-based treatment guidance. Laryngoscope, 2024.

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