Moderate Sensorineural Hearing Loss Research Articles

Clinical and genetic characterisation of childhood-onset sensorineural hearing loss reveal associated phenotypes and enrichment of pathogenic founder mutations in the Finnish population

Objective To examine the clinical and genetic characteristics of childhood-onset bilateral sensorineural hearing loss (SNHL) in Finland. Design Retrospective analysis. Study sample A total of 249 children younger than 18 years were diagnosed with bilateral SNHL in Oulu University Hospital, Finland, from 2017 to 2022. Results Pathogenic or likely pathogenic gene variants or chromosome abnormalities explaining SNHL were identified in 41% (N = 101/249) of children. Likely causative variants were more commonly identified in patients with severe SNHL than in those with moderate or mild SNHL. Our study identified likely causative gene variants in 24 different genes and six different likely causative chromosome abnormalities, demonstrating the genetic heterogeneity of SNHL. Population-enriched founder mutations were identified in the CABP2, CLRN1, MYO7A, SUCLA2, TMC1, and TWNK genes. A significant number of patients had associated phenotypes, including global developmental delay or intellectual disability (16%), language disorder (20%), ophthalmological abnormalities (16%), or malformations other than those involving the ear (10%). Conclusions SNHL is genetically and clinically heterogeneous. Pathogenic variants in GJB2 were the most common. Several population-enriched variants were identified as causing SNHL in the northern Finnish population. Associated medical phenotypes are common and should be taken into account in patients’ follow-up and treatment.

Longitudinal follow-up of hearing, speech, and language skills in 6-year-old children with congenital moderate hearing loss

ObjectivesChildren born with moderate hearing loss present with speech and language outcomes at both ends of the spectrum. To explore reasons for this, the objective of this study was to follow up a group of children born with moderate sensorineural hearing loss at 6 years of age (n=7) by investigating their outcomes in hearing, speech, and language development from time point of hearing aid fitting at 6 months. Another objective was to investigate the relationship between earlier outcomes on precursing variables to the current status in auditory, speech and language development. MethodEarlier data from a project with the same participants of auditory variables, speech, and language development were compared to the current study outcomes at 6 years of age. Children in this study performed standardized tests of phonology (SVANTE), expressive vocabulary (BNT), and speech-in-noise test (Hagerman´s sentences). Parents reported on their child´s functional auditory performance in everyday life (PEACH), and demographics and general development (questionnaire). Etiology and frequency of speech and language-directed intervention from time point of diagnosis to 6 years of age were collected through medical journals. ResultsHearing levels were stable over time in all children but one, who had received bilateral cochlear implants. Performance on speech-in-noise testing varied in aided condition (-0.8 to 8, mean 2.65, SD 3.09) and unaided condition (7.2 dB to 21.2 dB, mean 12.06, SD 4.82). Scores on the PEACH indicated further review in four of the seven children. Mean group score on consonant proficiency had increased from 3 to 6 years of age and were within age norms. Vocabulary scores were below the norms of children with typical hearing. Outcomes on vocabulary measures at 2.5 years years showed strong correlations that were significant to scores on the BNT at 6 years of age (r = 0.87, p = 0.05). Correlations between hours of hearing aid use and vocabulary was not significant at 6 years of age. The frequency of intervention sessions in the first 6 years varied between participants (4 to 55, mean 19.1, SD 17.1). ConclusionDespite homogeneous hearing and other background variables in the participants from birth, large individual variations in speech and language outcomes at 6 years of age were found. Considering the many factors involved that impact the development of children with moderate hearing loss, the results suggest that monitoring early precursors in auditory, speech and language development may be helpful in setting commensurate goals for each child. Detecting additional conditions that may pose challenges in future speech and language as early as possible is important. There is ample room for improvement in terms of increasing the frequency of intervention for children with moderate hearing loss and their families.

Equivalence and test–retest reliability of Tulu sentence lists for measuring speech recognition threshold in noise among individuals with sensorineural hearing loss

BackgroundThe difficulty in understanding speech becomes worse in the presence of background noise for individuals with sensorineural hearing loss. Speech-in-noise tests help to assess this difficulty. Previously, the Tulu sentence lists have been assessed for their equivalency to measure speech recognition threshold in noise among individuals with normal hearing. The present study aimed to determine the equivalence and test–retest reliability of Tulu sentence lists for measuring speech recognition threshold in noise among individuals with sensorineural hearing loss.ResultsThe SNR-50 was measured for 13 sentence lists in 20 Tulu-speaking individuals with mild to moderate sensorineural hearing loss. Retesting was done by administering all lists to eight participants after an average of 25.25 days (SD = 19.44). Friedman test was administered to check for the list equivalency. Intraclass correlation coefficient was measured to assess test–retest reliability. A regression analysis was performed to understand the influence of pure-tone average on SNR-50. A Kruskal–Wallis test was administered to check the statistical significance of the SNR-50 obtained across different configurations and degrees of hearing loss. Nine of the 13 Tulu sentence lists (lists 2, 4, 5, 6, 9, 10, 11, 12, and 13) were equivalent in individuals with sensorineural hearing loss. The mean SNR-50 for these nine lists was 1.13 dB (SD = 2.04 dB). The test–retest reliability was moderate (ICC = 0.727). The regression analysis showed that a pure-tone average accounted for 24.7% of the variance in SNR-50 data (p = 0.026). Individuals with mild to moderate hearing loss obtained the worst SNR-50, followed by mild and high-frequency hearing loss.ConclusionNine Tulu sentence lists are equivalent and reliable and can be used to measure speech recognition threshold in noise among individuals with sensorineural hearing loss who are Tulu speakers.

Evaluation of the effect of hearing loss on cognitive-vestibular functions in the geriatric population

BackgroundPresbycusis is known to accelerate mental decline processes and to cause secondary consequences such as social isolation and depression when not intervened. The association of presbycusis and presbyvestibulopathy disorders is likely. These changes in vestibular functions are closely related to cognitive functions. This study investigated the effect of hearing loss on cognitive and vestibular functions in a geriatric population.MethodsThis study was conducted with 200 participants over the age of 65. The study was conducted with three groups: individuals with normal hearing, moderate sensorineural hearing loss, and severe sensorineural hearing loss. The relationship between the participants’ responses to the Cognitive Vestibular Function Scale and their hearing levels was examined.ResultsOf the individuals with hearing loss in the study group, 55 had moderate SNHL, and 45 had severe SNHL. The age range of the participants was 65 to 91 years (71.06 ± 4.92). When the control (Group N) and study groups (Group M and Group S) were compared according to CVFS and subscale scores, they were found to be statistically different (p < 0.05). In pairwise comparisons, a significant difference (p < 0.05) was found in all pairwise combinations except Group N and Group M (p = 0.109) for the VSM subscale.ConclusionsIt was concluded that having a healthy hearing is the most influential factor for maintaining cognitive vestibular functions in the geriatric population. It was found that the degree of hearing loss primarily affects cognitive vestibular functions and that cognitive vestibular functions deteriorate more as hearing loss increases.

Infant Cervical Vestibular Evoked Myogenic Potentials: A Scoping Review.

Children diagnosed with hearing loss typically demonstrate increased rates of vestibular loss as compared with their peers, with hearing within normal limits. Decreased vestibular function is linked with delays in gross motor development, acquisition of gross motor skills, and academic challenges. Timely development of sitting and walking gross motor skills aids in the progress of environmental exploratory activities, which have been tied to cognitive, language, and vocabulary development. Considering the time-sensitive development of gross motor skills and cognitive, language, and vocabulary development, identifying vestibular loss in infancy can support early intervention. This scoping review analyzes stimulus, recording, and participant factors relevant to assessing cervical vestibular evoked myogenic potentials (cVEMPs) in the infant population. The scoping literature review was conducted on literature published between 2000 and 2023, focusing on articles assessing cVEMPs in infants. Two authors independently followed Preferred Reporting Items for Systematic and Meta-Analysis guidelines for title and abstract screening, full-text review, data extraction, and quality assessments. Sixteen articles meeting the inclusion criteria were included in the analysis. The existing literature lacks consensus regarding stimulus and recording parameters for measuring infant cVEMPs. In addition, the review reveals a decrease in cVEMP response occurrence rates with the severity of hearing loss, especially in cases of severe to profound hearing loss, compared with mild to moderate sensorineural hearing loss in infants. This scoping review demonstrates the increasing use of cVEMP as a reliable tool for objectively assessing infant vestibular function. The lack of consensus in stimulus and recording parameters emphasizes the need for systematic research to establish an evidence-based protocol for cVEMP measurements in infants. Such a protocol will ensure the reliable measurement of cVEMPs in infants and enhance the effectiveness of cVEMP as part of the infant vestibular test battery. In addition, there is a necessity for a comprehensive large-scale study to evaluate the practicality and feasibility of implementing vestibular screening protocols for infants diagnosed with sensorineural hearing loss in the United States.

Importance of ocular examination as a prerequisite in renal dysfunction patients prior to renal transplantation - A case report

Alport’s syndrome is a heterogeneous genetic disease involving the basement membrane of the glomeruli, inner ear, retina, and lens capsule. A 30-years-old male patient, recently diagnosed with chronic kidney disease (CKD) stage 5 awaiting for renal transplantation presented to the ophthalmology outpatient department with a complaint of gradual decrease in vision in both eyes since 2 years. Patient has a history of anemia, hypertension for the past 3 years and was on medication for both. On ocular examination, unaided vision was counting fingers (CF)- 2 meters in both eyes with pinhole improvement to 6/60 in both eyes. On slit lamp examination, bilateral anterior lenticonus was seen after pupil dilation. On detailed history taking, the patient has a history of hearing loss since the past 6 months. ENT consultation was done, and the patient was found to have bilateral moderate sensorineural hearing loss. The presence of ocular abnormalities, bilateral sensorineural hearing loss and renal failure is highly suggestive of Alport’s syndrome. When a genetic disorder is identified, it aids in identifying the cause of renal dysfunction in that patient but also enables us to screen other family members for the same before deciding on the potential living donor. Keywords: lenticonus; hematuria; deafness; renal failure; Alport’s syndrome

Clinical characteristics of patients diagnosed with bilateral sudden sensorineural hearing loss.

This study investigated the etiology, clinical features, and prognosis of patients diagnosed with bilateral sudden sensorineural hearing loss (BSSNHL). The clinical data of 100 patients with bilateral sudden hearing loss as a chief complaint treated at Xiangya Second Hospital of Central South University between January 2010 and August 2022, including clinical characteristics, audiometric data, and prognosis, were retrospectively analyzed. These 100 cases accounted for 8.09% (100/1235) of all patients admitted for sudden sensorineural hearing loss (SSNHL) during the same period. Of these, 71 were simultaneous cases and 29 were sequential cases of BSSNHL. Among the 200 ears analyzed in this study, 13, 36, 57, and 94 had mild, moderate, severe, and profound sensorineural hearing loss, respectively. The overall effective rate after comprehensive treatment was 32%, with significant differences in efficacy and prognosis among different degrees of hearing loss (p < 0.05). Comorbidities of hypertension (24 cases), diabetes (14 cases), and coronary heart disease (9 cases) significantly impacted therapeutic efficacy and prognosis in patients with BSSNHL (p < 0.05). Compared to unilateral SSNHL, BSSNHL exhibits distinctive characteristics.

Silent Hearing Loss in Kidney Transplant Patients Receiving Tacrolimus: A Fact or a Myth?

Background: It has been claimed that tacrolimus may have harmful effects on the auditory system, where it has been linked to ototoxicity and sensorineural hearing loss (SNHL). We evaluated silent SNHL in kidney transplant recipients (KTRs) receiving tacrolimus and the different factors affecting it compared to healthy controls. Materials and Methods: In this case control study, hearing functions were studied in 42 KTRs receiving tacrolimus as maintenance immunosuppressive therapy for more than 3 months in comparison to 27 age- and gender-matched healthy subjects using tympanometry, pure-tone audiometry (PTA), extended high frequency audiometry (EHFA), and transient evoked oto-acoustic emissions (TEOAEs). Also, different factors were studied in relation to SNHL. Results: PTA showed that 23.8%, 21.4%, and 4.8% had mild, moderate, and severe SNHL, respectively. One-fifth of KTRs had severe SNHL, according to EHFA. According to TEOAEs, 28.6% of KTRs had abnormal hearing. There was a significant positive correlation between the tacrolimus trough levels and the results of both the PTA (P = 0.002) and EHFA (P = 0.035) tests. Conclusion: SNHL was detected in about half of the studied KTRs. Silent SNHL in KTRs might be associated with higher tacrolimus trough levels.

Psychoacoustic and electrophysiologic auditory neural encoding in school-aged children with mild to moderate sensorineural hearing loss

Psychoacoustic and electrophysiologic auditory neural encoding in school-aged children with mild to moderate sensorineural hearing loss

Epidemiological Profile of Hearing Deficiency in a Tertiary Care Hospital (2019-2022)

Abstract Introduction This study is an attempt to view the demographic profile of individuals presenting with hearing loss in an urban referral hospital with the help of available database of pure tone audiometry results over a three years period. Materials and methods It is adescriptive epidemiologicalstudy over a period of 3 years conducted in the Department of Otorhinolaryngology (ORL) of an urban referral hospital.The subjects presented with a complain of hearing loss were investigated with pure tone audiometry. Subjects below 10 years of age or with gross mental or physical disabilities were excluded. The results of pure tone audiometry done were analyzed followed by systematic analysis. Results A total number of 3536individuals inclusive of1980 males and1556 females were studied. Nearly, 570 individuals suffered B/L hearing loss (16%) which was again mostly Sensori-neural. Sensori-neural hearing loss was the most common hearing loss near about 52%.Mixed Hearing loss accounted for 13%.Conductive hearing loss accounted for 35 %. Mild hearing loss (25-40dB) accounted for maximum bulk of the patients.The bulk of the population comprised of people in their middle age (30-60) and elderly (&gt;60 years). Conclusion The demographic profile of hearing loss in an urban population is the ground work for any comprehensive planning to resolve the issue. In our study the increased burden of mild to moderate sensorineural hearing loss as seen in the elderly population and increased prevalence of conductive hearing loss as seen in the young population shows us how respective management protocols can be instituted for the target populations.

Comprehensive analysis of two hotspot codons in the TUBB4B gene and associated phenotypes

Our purpose was to elucidate the genotype and ophthalmological and audiological phenotype in TUBB4B-associated inherited retinal dystrophy (IRD) and sensorineural hearing loss (SNHL), and to model the effects of all possible amino acid substitutions at the hotspot codons Arg390 and Arg391. Six patients from five families with heterozygous missense variants in TUBB4B were included in this observational study. Ophthalmological testing included best-corrected visual acuity, fundus examination, optical coherence tomography, fundus autofluorescence imaging, and full-field electroretinography (ERG). Audiological examination included pure-tone and speech audiometry in adult patients and auditory brainstem response testing in a child. Genetic testing was performed by disease gene panel analysis based on genome sequencing. The molecular consequences of the substitutions of residues 390 and 391 on TUBB4B and its interaction with α-tubulin were predicted in silico on its three-dimensional structure obtained by homology modelling. Two independent patients had amino acid exchanges at position 391 (p.(Arg391His) or p.(Arg391Cys)) of the TUBB4B protein. Both had a distinct IRD phenotype with peripheral round yellowish lesions with pigmented spots and mild or moderate SNHL, respectively. Yet the phenotype was milder with a sectorial pattern of bone spicules in one patient, likely due to a genetically confirmed mosaicism for p.(Arg391His). Three patients were heterozygous for an amino acid exchange at position 390 (p.(Arg390Gln) or p.(Arg390Trp)) and presented with another distinct retinal phenotype with well demarcated pericentral retinitis pigmentosa. All showed SNHL ranging from mild to severe. One additional patient showed a variant distinct from codon 390 or 391 (p.(Tyr310His)), and presented with congenital profound hearing loss and reduced responses in ERG. Variants at codon positions 390 and 391 were predicted to decrease the structural stability of TUBB4B and its complex with α-tubulin, as well as the complex affinity. In conclusion, the twofold larger reduction in heterodimer affinity exhibited by Arg391 substitutions suggested an association with the more severe retinal phenotype, compared to the substitution at Arg390.

Remote or in-clinic? The effect of service delivery mode on hearing aid output: study protocol for a double-blinded, randomised trial in adults with mild to moderate sensorineural hearing loss

BackgroundTeleaudiology can potentially improve access to hearing healthcare services. Remote hearing aid fittings offer a new mode of service delivery that removes barriers of geography and access to an audiologist. Real-ear measurements (REMs) are the gold standard for hearing aid output verification but require in-clinic appointments. This study will investigate whether remote hearing aid fittings can provide clinically equivalent outcomes when compared to current, in-clinic, best practice guidelines.Research designA repeated measure, double-blinded crossover design will be used. Participants will be randomly allocated to one of two groups to determine order of intervention, balanced for degree of hearing loss.Study sampleSixty adults with mild to moderate hearing loss and at least 1 year of experience with hearing aids will be recruited.Data collection and analysisParticipants will complete two hearing aid fitting protocols, one using an in-clinic fitting process and the other using a remote (at-home) fitting process. In-clinic fittings will include REMs with adjustments to standard (NAL-NL2) prescription targets. The two fitting protocols will then be randomly assigned to participants in a crossover design, so participants and researchers will be blinded to the order of the two fitting protocols. Participants will then have a 4-week period with follow-up appointments for participant-directed gain adjustment. For each fitting protocol, participants will complete objective measurements of final hearing aid output with REMs, speech-in-noise testing, subjective measurements of hearing aid performance, and quality of life measurements. They will then begin an identical period of living with, adjusting, and objective assessment with the other fitting protocol. Data will be analysed as repeated measures with statistical control for potential confounding variables.ResultsData will compare the four-frequency average real-ear aided response (4FREAR) for hearing aids programmed in-clinic and hearing aids programmed remotely, after participant-directed gain adjustments. Secondary measures will assess clinically significant differences in estimated speech intelligibility, hearing-related quality of life, hearing aid benefit, sound quality and preference, and speech-in-noise ability.ConclusionsThis study will inform the development of best practice guidelines for remote hearing aid fittings. If no clinically significant differences are found between in-clinic and remote fit hearing aids, it has the potential to expand teleaudiology initiatives.Trial registrationAustralian New Zealand Clinical Trial Registry, ACTRN12623000028606p. Date of registration: 12 January 2023.

Development and validation of words in babble test in the modern Greek language

Abstract Purpose: The aim of this paper is to present and describe the development and validation of new Words in the Babble test in modern Greek. Materials and Methods: Four 50-word phonemically balanced lists in babble noise were administered to 44 normal hearing subjects and 53 patients with hearing loss (HL) ears with mild and moderate sensorineural HL (SNHL). The word lists were monoaurally presented (i.e., separately for the right and left ears) at 11 signal-to-noise (babble) intensity levels, from −12 to +12 dB. The signal remained constant at 65 dB for all participants with normal hearing and at 20 dB SL for participants with SNHL, whereas the multitalker babble stimulus varied according to the desired signal-to-noise ratio (SNR). Results: The results showed that word recognition scores were negatively affected by HL and were negatively related to the degree of HL. There was a statistically significant difference for all scores in all SNRs, both between participants with normal hearing and those with mild HL as well as between participants with mild and moderate HL, except for −3 dB SNR and −6 dB SNR between mild and moderate HL. In addition, there were no statistically significant differences between the right and left ears in any of the SNRs. Conclusions: The existing literature suggests that the use of monosyllabic words produces recognition performance in multitalker babble tests, as observed in languages such as Spanish, Estonian, and Arabic, whereas the present study provides evidence that a similar effect is produced when disyllabic words are used.

Effect of Digital Noise Reduction in Hearing Aids on Speech Intelligibility in Both Quiet and Noisy Environments.

Digital noise reduction (DNR) minimizes the effect of noise on speech signals by continuously monitoring frequency bands in the presence of noise. In the present study, we explored the effect of DNR technology on speech intelligibility in individuals using hearing aids (HAs) and investigated implications for daily use. Eighteen participants with bilateral moderate sensorineural hearing loss (aged 16-45 years) were included. Bilateral receiver-in-the-ear HAs were fitted in the participants. The adaptive and nonadaptive (with a signal-to-noise ratio (SNR) of +5 and -5 dB, respectively) Turkish matrix sentence test (TURMatrix) in noise and free-field hearing assessments, including hearing thresholds with hearing aids, speech recognition thresholds (SRT), and speech discrimination scores, were conducted in two different conditions: HA in the DNR-on and DNR-off conditions. No significant difference was observed between free-field hearing assessments with the HA in the DNR-off and DNR-on conditions (P > 0.05). Furthermore, the adaptive and nonadaptive TURMatrix revealed significant differences between the scores under the DNR-on and DNR-off conditions (P < 0.05). Nevertheless, under the DNR-on condition, there was no correlation between free-field hearing assessments with HA and TURMatrix results (P > 0.05). However, a significant correlation was observed between SRT scores with HA and TURMatrix scores (adaptive and nonadaptive, +5 and -5 dB SNR, respectively) under the DNR-off condition (P < 0.05). Our study findings suggest that DNR can improve speech intelligibility in noisy environments. Therefore, DNR can enhance an individual's auditory comfort by improving their capacity to grasp speech in background noise.

The congenital hearing phenotype in GJB2 in Queensland, Australia: V37I and mild hearing loss predominates.

GJB2 was originally identified in severe, non-syndromic sensorineural hearing loss (SNHL), but was subsequently associated with mild and moderate SNHL. Given the increasing utilisation of genetic testing pre-conceptually, prenatally, and neonatally, it is crucial to understand genotype-phenotype correlations. This study evaluated the nature and frequency of GJB2 variants in an Australian paediatric population with varying degrees of SNHL ascertained through newborn hearing screening. Audiograms from individuals with GJB2 variants and/or a GJB6 deletion (GJB6-D13S11830) were retrospectively reviewed (n = 127). Two-thirds were biallelic (homozygous/compound heterozygous) for pathogenic/likely pathogenic variants of GJB2 and/or GJB6 (n = 80). The most frequent variant was c.109 G > A, followed by c.35delG and c.101 T > C. Compared to biallelic carriage of other GJB2 variants, c.109 G > A positive individuals (homozygous/compound heterozygous) were more likely to have mild HL at their initial and latest audiograms (p = 0.0004). Biallelic carriage of c.35delG was associated with moderately-severe or greater SNHL at both initial and latest audiograms (p = 0.007). The c.101 T > C variant presented with milder SNHL and U-shaped audiograms (p = 0.02). In this agnostically identified cohort, mild SNHL predominated in GJB2/GJB6 carriers in contrast to previous studies targeting individuals with significant loss. Consequently, c.109 G > A, associated with milder phenotypes, was the most frequent. This study provides valuable data to support prognostic confidence in genetic counselling.

The Impact of Age-Related Hearing Loss on Working Memory among Older Individuals: An Event-Related Potential Study

Introduction: Age-related hearing loss (ARHL) may affect working memory (WM), which impacts problem-solving, decision-making, language comprehension, and learning. Limited research exists on how ARHL affects WM using N-back tasks, but studying this is crucial for understanding neural markers and associated cognitive processes. Our study explores the impact of ARHL on WM using behavioral and electrophysiological measures and how it correlates with speech-in-noise scores in older individuals with ARHL. Method: The study involved two groups, each with 20 participants aged 60–80. Group 1 had individuals with mild to moderate sensorineural hearing loss, while Group 2 had age- and education-matched controls with normal or near-normal hearing. Participants underwent audiological assessments and completed cognitive tests, including simple reaction time and N-back tests. During the performance of cognitive tasks, a simultaneous electroencephalography was recorded. Data analysis included behavioral and event-related potentials, source estimation, and functional connectivity analysis. Results: The study revealed significantly poor accuracy, longer reaction time, and smaller P300 amplitude among individuals with ARHL, even after controlling for general slowing. Individuals with ARHL experience compromised neural activity, particularly in the temporal and parietal regions, which are vital for cognition and WM. Furthermore, individuals with ARHL exhibited poor communication between the superior temporal gyrus and insulae regions among the brain regions mediating WM during the 1-back task. Also, the study found a strong correlation between hearing measures and WM outcomes. Conclusion: The study findings suggest that individuals with ARHL have impaired WM compared to those with normal hearing. This indicates a potential link between ARHL and cognitive decline, which could significantly affect daily life and quality of life. The widely used WM test with simultaneous EEG recording and source estimation analysis would further validate the usefulness of the study in assessing WM in this population.

The impact of hearing-aid amplification and its integrated tinnitus feature on tinnitus management

Tinnitus is the potentially debilitating perception of phantom sound with no current cure. Although some management approaches are available, the benefits of hearing aid amplification and its added noiser are uncertain. This study assessed impacts of hearing-aid amplification, and amplification with an added noiser feature, on adults with hearing loss and chronic bothersome tinnitus. Thirty adults [42–75 (Mean = 61.1) years old; 18 males], with mild to moderate sensorineural hearing loss but no previous amplification exposure; and bothersome tinnitus [Tinnitus Functional Index (TFI) baseline scores of at least 20], were randomly assigned to one of two groups in a cross-over study to experience amplification-only, and amplification + noiser for one month each, following one month of no intervention. Study hearing aids were worn minimally for 5-h/day. TFI questionnaires evaluated tinnitus severity before and after each condition. A one-way repeated measures ANOVA with 30 participants revealed a significant effect on TFI across baseline, amplification-only, and amplification + noiser conditions (p = .023), suggesting tinnitus improvement across conditions. Further post-hoc tests indicated lower TFI scores in the amplification + noiser condition (p = 0.048), identifying its distinct benefit. Preliminary analysis suggests benefit of hearing aid amplification, with a prominent advantage of the noiser feature on tinnitus management.

Acoustic change complex findings in mild and moderate sensorineural hearing loss

BackgroundAuditory electrophysiological tests of the cortex, which are processed in or close to the auditory cortex, are brain reactions to sound. A variation in a continuous stimulus causes the acoustic change complex potential (ACC), which is a wave following the P1-N1-P2 response.ObjectiveTo measure the amplitude and latency of different components of ACC in normal subjects and across individuals with mild and moderate degrees of sensorineural hearing loss (SNHL).Patients and methodsThe study includes 100 individuals with the age ranged from 10 to 50 years with different degrees of SNHL. The ACC was evoked by a change of second formant in the middle of ongoing steady-state synthetic, 3 formant vowels (ooee). The total duration was 500 ms. Changing occurred at 250 ms.ResultsThe SNHL subgroups showed statistically significantly longer P1 and N1 latencies. N1 and P2 amplitudes of ACC onset response were larger with a statistical significance as compared to controls. Post hoc analysis revealed no statistically significant difference between mild and moderate SNHL on ACC parameters. Age showed a significant negative correlation with ACC N1 and P2 latency, ACC P1 and N1 amplitude, and onset P2 latency. Onset response P1 latency was significantly higher in children than adults. Median ACC P1 amplitude significantly increased in children than adults.ConclusionACC is a reliable tool for testing the auditory cortex function of detecting difference in sounds presented that can be recorded readily in patients with mild and moderate SNHL.

Relationship of Serum Prestin Levels to the Severity of Sensorineural Hearing Loss.

Prestin is an outer hair cell (OHC) protein responsible for increasing cochlear sensitivity and has been proposed as a biomarker.We aimed to evaluate whether the serum prestin level is related to the severity of chronic sensorineural hearing loss (SNHL). Ninetysubjects were recruited from the patient base at Samarra public hospitals and clinics in Iraq from January to October of 2022. They were divided into three groups equally: agroup of healthy people without hearing loss (G0), a group with moderate SNHL (G1), and a group with severe SNHL (G2). The subjects ranged from 20 to 80 years of age and included 51 males and 39 females. Blood samples were collected, then serum was separated, and enzyme-linked immunosorbent assays were performed to quantify the levels of prestin. Hearing thresholds were sequentially statistically higher across the three groups. While prestin levels were significantly higher in G1 and G2 than that in G0, there were no differences between the G1 and G2 levels. Serum prestin levels were positively correlated with hearing thresholds in G1, but not G2. Our results suggest that in the clinical setting, prestin is sensitive to chronic mild to moderate SNHL (i.e., up to 40-60 dB), not more severe loss.This range is consistent with the added sensitivity provided by OHCs in the cochlea and provides support for prestin as a biomarker of OHC-mediated SNHL.

MPZL2—a common autosomal recessive deafness gene related to moderate sensorineural hearing loss in the Chinese population

BackgroundMutations in MPZL2, the characteristic genetic etiology of autosomal recessive deafness loci 111 (DFNB111), cause non-syndromic and moderate sensorineural hearing loss.MethodsIn this study, we analyzed the phenotype and genotype of eight pedigrees consisting of 10 hearing loss patients with bi-allelic pathogenic or likely pathogenic variants in MPZL2. These patients were identified from a 3272 Chinese patient cohort who underwent genetic testing.ResultsApart from symmetrical and moderate sensorineural hearing loss, the MPZL2-related phenotype was characterized by progressive hearing loss with variation in the onset age (congenital defect to onset at the young adult stage). We determined that in the Chinese population, the genetic load of MPZL2 defects was 0.24% (8/3272) in patients diagnosed with hearing loss and 7.02% (8/114) in patients diagnosed with hereditary moderate sensorineural hearing loss caused by STRC, OTOA, OTOG, OTOGL, TECTA, MPZL2 and others. Three known MPZL2 variants (c.220C > T (p.Gln74*), c.68delC (p.Pro23Leufs*2), c.463delG (p.Ala155Leufs*10)) and a novel start loss variant (c.3G > T (p.Met1?)) were identified. MPZL2 c.220C > T was identified as the hotspot variant in the Chinese population and even in East Asia compared with c.72delA (p.Ile24Metfs*22) in European and West Asia through allele frequency.ConclusionsWe concluded that apart from moderate HL, progressive HL is another character of MPZL2-related HL. No specified variant was verified for the progression of HL, the penetrance and expressivity cannot be determined yet. A novel MPZL2 variant at the start codon was identified, enriching the variant spectrum of MPZL2. The hotspot variants of MPZL2 vary in different ethnicities. This study provides valuable data for the diagnosis, prognosis evaluation and genetic counseling of patients with moderate sensorineural hearing loss related to MPZL2.