Post-surgical Hearing Improvement in a Case of Jugular Foramen Schwannoma Despite the Lack of Response in a Preoperative Auditory Brainstem Response Test.

We report a rare case involving improved hearing after surgery for a jugular foramen schwannoma despite the lack of response during the preoperative auditory brainstem response (ABR) test. A left jugular foramen tumor was diagnosed in a 79-year-old man with hearing loss. No response was observed during the preoperative ABR test. However, his hearing improved after surgery using the lateral suboccipital approach. Following Gamma Knife radiation to the residual tumor post-surgery, the ABR test detected V waves. The hearing of patients with cerebellopontine angle tumors can improve even when there is no response during the preoperative ABR test.

Clinical practice guidelines for the diagnosis and management of acute sensorineural hearing loss.

OBJECTIVE: Acute sensorineural hearing loss represents a spectrum of conditions characterized by sudden onset hearing loss. The "Clinical Practice Guidelines for the Diagnosis and Management of Acute Sensorineural Hearing Loss" were issued as the first clinical practice guidelines in Japan outlining the standard diagnosis and treatment. The purpose of this article is to strengthen the guidelines by adding the scientific evidence including a systematic review of the latest publications, and to widely introduce the current treatment options based on the scientific evidence. METHODS: The clinical practice guidelines were completed by 1) retrospective data analysis (using nationwide survey data), 2) systematic literature review, and 3) selected clinical questions (CQs). Additional systematic review of each disease was performed to strengthen the scientific evidence of the diagnosis and treatment in the guidelines. RESULTS: Based on the nationwide survey results and the systematic literature review summary, the standard diagnosis flowchart and treatment options, including the CQs and recommendations, were determined. CONCLUSION: The guidelines present a summary of the standard approaches for the diagnosis and treatment of acute sensorineural hearing loss. We hope that these guidelines will be used in medical practice and that they will initiate further research.

A novel variant in GAS2 is associated with autosomal dominant nonsyndromic hearing impairment in a Chinese family.

Knockout of GAS2 (growth arrest-specific protein 2), causes disorganization and destabilization of microtubule bundles in supporting cells of the cochlear duct, leading to hearing loss in vivo. However, the molecular mechanism through which GAS2 variant results in hearing loss remains unknown. By Whole-exome sequencing, we identified a novel heterozygous splicing variant in GAS2 (c.616-2 A > G) as the only candidate mutation segregating with late-onset and progressive nonsyndromic hearing loss (NSHL) in a large dominant family. This splicing mutation causes an intron retention and produces a C-terminal truncated protein (named GAS2mu). Mechanistically, the degradation of GAS2mu via the ubiquitin-proteasome pathway is enhanced, and cells expressing GAS2mu exhibit disorganized microtubule bundles. Additionally, GAS2mu further promotes apoptosis by increasing the Bcl-xS/Bcl-xL ratio instead of through the p53-dependent pathway as wild-type GAS2 does, indicating that GAS2mu acts as a toxic molecule to exacerbate apoptosis. Our findings demonstrate that this novel variant of GAS2 promotes its own protein degradation, microtubule disorganization and cellular apoptosis, leading to hearing loss in carriers. This study expands the spectrum of GAS2 variants and elucidates the underlying pathogenic mechanisms, providing a foundation for future investigations of new therapeutic strategies to prevent GAS2-associated progressive hearing loss.

Hearing loss is not associated with risk of Parkinson's disease: A Mendelian randomization study.

Purpose: A few observational studies have indicated that Parkinson's disease (PD) risk may be higher in those with hearing loss, but the two's causal relationship is yet unknown. Using Mendelian randomization (MR) methods, this study sought to explore the causal link between hearing loss and the risk of PD. Methods: We identified single nucleotide polymorphisms (SNPs) linked to hearing loss (P-value<5E-08) in a genome-wide association study (GWAS) included 323,978 people from the UK Biobank. The summary data for PD in the discovery group came from a GWAS meta-analysis of 33,647 cases and 449,056 healthy participants of European descent. Using summary data from the aforementioned GWAS of PD (N = 33,647) and hearing loss (N = 323,978), we carried out a two-sample MR study. As validation groups, two separate PD GWAS studies were used. Inverse variance weighting (IVW) was utilized in the principal MR analysis. For our findings to be reliable, further analyses were carried out with the Cochran's Q test, MR-Egger intercept, and leave-one-out analysis. In addition, we assessed the causal link between various forms of hearing loss and PD using the IVW approach. Results: Twenty-two SNPs with genome-wide significance linked to hearing loss were used as instrumental factors. In the discovery dataset, we failed to detect a causal relationship between hearing loss and PD (OR = 1.297; 95 % CI = 0.420-4.007; P-value = 0.651). The findings of other methods agreed with the IVW method. The results were robust under sensitivity analyses. Furthermore, the above findings were confirmed in two validation PD datasets. Additionally, no causal correlation was found between genetic prediction of four different types of hearing loss and PD (conductive hearing loss, IVW: OR = 1.058, 95%CI = 0.988-1.133, P-value = 0.108; sudden idiopathic hearing loss, IVW: OR = 0.936, 95%CI = 0.863-1.016, P-value = 0.113; mixed conductive and sensorineural hearing loss, IVW: OR = 0.963, 95%CI = 0.878-1.058, P-value = 0.436; sensorineural hearing loss, IVW: OR = 1.050, 95%CI = 0.948-1.161, P-value = 0.354). Conclusion: In those of European heritage, our investigation revealed no causal link between hearing loss and PD risk.

Newborn and infant hearing screening for early detection of hearing loss in Nairobi, Kenya.

Background: Early detection of hearing loss and subsequent intervention leads to better speech, language and educational outcomes giving way to improved social economic prospects in adult life. This can be achieved through establishing newborn and infant hearing screening programs. Objective: To determine the prevalence of hearing loss in newborns and infants in Nairobi, Kenya. Methods: A cross-sectional pilot study was conducted at the National hospital and at a sub county hospital immunization clinic. A total of 9,963 babies aged 0-3 years, were enrolled in the hearing screening program through convenient sampling over a period of nine months. A case history was administered followed by Distortion Product Oto-acoustic emissions (DPOAEs) and automated auditory brainstem response (AABR) hearing screening. Results: The screening coverage rate was 98.6% (9963/10,104). The referral rate for the initial screen was 3.6% (356/ 9,963), the return rate for follow-up rescreening was 72% (258 babies out of 356) with a lost to follow-up rate of 28% (98/356). The referral rate of the second screen was 10% (26/258). All the 26 babies referred from the second screen returned for diagnostic hearing evaluation and were confirmed with hearing loss, yielding a prevalence of 3/1000. Conclusions: Establishing universal newborn and infant hearing screening programs is essential for early detection and intervention for hearing loss. Data management and efficient follow-up systems are an integral part of achieving diagnostic confirmation of hearing loss and early intervention.

Association of oxidative balance score with hearing loss and tinnitus: NHANES 1999-2018.

Background: Oxidative stress is associated with the occurrence of hearing loss and tinnitus. The oxidative balance score (OBS), a composite indicator evaluating the balance between antioxidant and pro-oxidative components across various dietary and lifestyle factors, indicates the overall oxidative balance status. However, the association of OBS with hearing loss and tinnitus has not been reported previously. Methods: Cross-sectional data from the National Health and Nutrition Examination Survey (NHANES) 1999-2018 were analyzed. Weighted multivariable logistic regression, weighted multivariable linear regression, and restricted cubic spline curve (RCS) regression were employed to explore the relationship between OBS and hearing loss at speech, low, and high frequencies, along with tinnitus. Subgroup analysis and sensitivity analysis were used to ascertain the consistency across subgroups and stability of the results. Results: We included 13,715 and 21,644 individuals to investigate the association between OBS and hearing loss, as well as between OBS and tinnitus, respectively. The second, third, and fourth quartiles of OBS were significantly associated with a lower risk of hearing loss at speech, low, and high frequencies, as well as tinnitus, compared to the lowest quartile. The RCS regression analysis indicated a negative linear association of OBS with hearing loss and tinnitus. Most associations were maintained in subgroup analysis and sensitivity analysis. Additionally, the dietary and lifestyle OBS independently contribute to the protection against hearing loss and tinnitus. Conclusion: OBS is negatively correlated with the risk of hearing loss and tinnitus. The findings suggest that combined antioxidant diet and lifestyle hold promise as potential strategies for reducing the prevalence of hearing loss and tinnitus.

Compliance with spectacle wear among learners with hearing impairment in Ghana.

Background: Hearing-impaired learners with refractive problems require correction because poor vision hinders their development and educational pursuits. Objectives: To determine the level of compliance with spectacle wear in learners with hearing impairment in Ghana. Method: A descriptive cross-sectional study design was used to investigate the level of compliance with spectacle wear in hearing-impaired learners with uncorrected refractive errors (URE). The participants were from six schools for the hearing impaired, comprising three schools from each sector (Northern and Southern) of Ghana. Results: Of the 1914 learners screened, 69 (3.61% CI: 2.82-4.54%) had URE. Sixty-two (89.9%) learners with URE had myopia (-0.50 Dioptre Sphere (DS) to -2.00DS), and 7 (10.1%) had hyperopia (+2.00DS to +10.00DS). There were more females (53.6%) with URE than males, and their ages ranged from 8 to 35 years, with a mean of 17.35 ± 5.19 years. Many (56.5%) learners complied with spectacle wear after 3 months of reassessment, with females being more compliant than males, but the difference was not significant (p = 0.544). Learners who complied well with the spectacle wear were those with moderate visual impairment (VI), followed by mild VI, while those with no VI were the least compliant. A significant difference was observed between spectacle compliance and presenting VI (p = 0.023). Conclusion: The spectacle wear compliance level was high compared to a previous study (33.7%) in Ghana. Contribution: This study highlights the importance of addressing URE among learners with hearing impairment in Ghana and Africa.

Occupational health disorders among physical education teachers compared to classroom and subject specialist teachers.

During the course of their work, teachers may be subjected to conditions that cause different health problems. This study examines occupational health disorders in a representative sample of 858 teachers (528 female; age 44.0 ± 9.67 years) divided into three groups of teachers with specific occupational requirements: specialist physical education teachers (specialist PETs), classroom teachers, and specialist teachers. The number of health disorders in the last 12 months was recorded using the Chronic Health Disorders Questionnaire. The differences between the different types of teachers, controlled for sex and age, were analyzed using binary logistic regression. The results showed that 89% of teachers experienced colds as the most frequently reported health problem, followed by 58% for lower back problems, 57% for headaches, 51% for hoarseness, and 43% for neck problems. A binary logistic regression showed that specialist PETs were the group with the highest health risk. They were about twice as likely to have musculoskeletal or hearing disorders than the other two groups of teachers. They were also significantly more likely to suffer from hoarseness. Understanding these different health challenges is critical to developing targeted interventions and robust support systems. These interventions should include initiatives aimed at raising awareness of health risk factors, implementing injury interventions and vocal cord hygiene programs, making ergonomic adjustments, and promoting awareness of self-care (both mental and physical). Given that the teaching profession is currently struggling with an aging workforce and a shortage of teachers, addressing these challenges is critical to the continued well-being of the teaching professionals.

Hearing impairment increases the risk of hip fracture-related mortality and recurrent hip fractures: A propensity score matching analysis.

PURPOSE: Despite the ongoing rise in hip fractures and the adverse effects of hearing impairment (HI) on increased mortality and morbidity, research addressing the influence of HI on mortality risk or complications in patients with hip fractures remains absent. This study aimed to analyze the effects of HI on mortality and treatment outcomes among patients with hip fracture. METHODS: We retrospectively collected data from consecutive patients diagnosed with hip fractures between January 2007 and March 2022 who had auditory examination records. From the initially enrolled 265 patients, data for 58 with HI and 58 without HI (control group) were extracted using a 1:1 propensity score matching. The primary outcome included comparison of mortality rates, and the secondary outcome encompassed the comparison of postoperative medical and surgical complications. RESULTS: The 1-year cumulative mortality rate was not significantly different between the HI and control groups, but the overall cumulative mortality rate was significantly higher in the HI than in the control group (63.0 % and 48.6, respectively; P = 0.046) in a follow-up period of up to 16 years. The HI group had a significantly higher incidence of "second hip fractures due to falls" than the control group (P = 0.016), although no differences in other medical and surgical complications were revealed. CONCLUSIONS: Awareness of the long-term risk of higher mortality when managing patients with hip fracture and HI is important. To reduce the risk of second hip fractures, paying more attention to fall prevention education and taking a more proactive approach, especially for those with HI.

The use of hearing tests to assess otitis media with effusion in children with Down syndrome.

BACKGROUND: Down syndrome is associated with an increased risk for otitis media with effusion (OME), a childhood condition in which fluid accumulates in the middle ear, potentially leading to hearing loss. The American Academy of Pediatrics Down syndrome guidelines and the American Academy of Otolaryngology - Head and Neck Surgery OME guidelines recommend hearing testing to assess the hearing status of children with Down syndrome diagnosed with OME. METHODS: Through an Institutional Review Board approved retrospective chart review at Children's Mercy, this project assessed how clinical factors affect the frequency in which children with Down syndrome receive hearing testing after diagnosis of OME. The study included data from all children with Down syndrome between 1 and 8 years old diagnosed with OME in the Down syndrome, general pediatrics, and otolaryngology clinics between 2018 and 2020. Demographics and clinical factors, including clinic setting, were collected. RESULTS: Of the 124 patients identified, 91.1 % were diagnosed with OME in the otolaryngology clinic and 33.1 % received hearing testing. While most diagnoses occurred in the otolaryngology clinic, a higher proportion of hearing testing at the time of diagnosis occurred in the Down syndrome clinic. This could be explained by the fact that the Down syndrome clinic is a multidisciplinary clinic, where yearly visits include hearing screening. Bivariate analysis using chi-square or Fisher's tests showed that clinic setting had a significant association (p-value <0.001) with hearing testing. However, logistic regression depicted all clinical factors had an insignificant effect on hearing testing at 5 % significance. CONCLUSION: While results indicate hearing testing is largely not performed to assess OME early in otolaryngology clinics, they may be used to assess intervention efficacy post-diagnosis. Results point to the importance of Down syndrome clinics in early diagnosis of hearing loss leading to timely referrals to otolaryngology clinics which diagnose and manage OME in children with Down syndrome.

Lexical tone recognition in multi-talker babbles and steady-state noise by Mandarin-speaking children with unilateral cochlear implants or bimodal hearing.

BACKGROUND AND OBJECTIVES: Lexical tone presents challenges to cochlear implant (CI) users especially in noise conditions. Bimodal hearing utilizes residual acoustic hearing in the contralateral side and may offer benefits for tone recognition in noise. The purpose of the present study was to evaluate tone recognition in both steady-state noise and multi-talker babbles by the prelingually-deafened, Mandarin-speaking children with unilateral CIs or bimodal hearing. METHODS: Fifty-three prelingually-deafened, Mandarin-speaking children who received CIs participated in this study. Twenty-two of them were unilateral CI users and 31 wore a hearing aid (HA) in the contralateral ear (i.e., bimodal hearing). All subjects were tested for Mandarin tone recognition in quiet and in two types of maskers: speech-spectrum-shaped noise (SSN) and two-talker babbles (TTB) at four signal-to-noise ratios (-6, 0, +6, and +12 dB). RESULTS: While no differences existed in tone recognition in quiet between the two groups, the Bimodal group outperformed the Unilateral CI group under noise conditions. The differences between the two groups were significant at SNRs of 0, +6, and +12 dB in the SSN conditions (all p < 0.05), and at SNRs of +6 and +12 dB of TTB conditions (both p < 0.01), but not significant at other conditions (p > 0.05). The TTB exerted a greater masking effect than the SSN for tone recognition in the Unilateral CI group as well as in the Bimodal group at all SNRs tested (all p < 0.05). Among demographic or audiometric variables, only age at implantation showed a weak but significant correlation with the mean tone recognition performance under the SSN conditions (r = -0.276, p = 0.045). However, when Bonferroni correction was applied to the correlation analysis results, the weak correlation became not significant. CONCLUSION: Prelingually-deafened children with CIs face challenges in tone perception in noisy environments, especially when the noise is fluctuating in amplitude such as the multi-talker babbles. Wearing a HA on the contralateral side when residual hearing permits is beneficial for tone recognition in noise.

Auditory brainstem response to paired clicks as a candidate marker of cochlear synaptopathy in humans.

OBJECTIVE: This study aimed to evaluate whether auditory brainstem response (ABR) using a paired-click stimulation paradigm could serve as a tool for detecting cochlear synaptopathy (CS). METHODS: The ABRs to single-clicks and paired-clicks with various inter-click intervals (ICIs) and scores for word intelligibility in degraded listening conditions were obtained from 57 adults with normal hearing. The wave I peak amplitude and root mean square values for the post-wave I response within a range delayed from the wave I peak (referred to as the RMSpost-w1) were calculated for the single- and second-click responses. RESULTS: The wave I peak amplitudes did not correlate with age except for the second-click responses at an ICI of 7 ms, and the word intelligibility scores. However, we found that the RMSpost-w1 values for the second-click responses significantly decreased with increasing age. Moreover, the RMSpost-w1 values for the second-click responses at an ICI of 5 ms correlated significantly with the scores for word intelligibility in degraded listening conditions. CONCLUSIONS: The magnitude of the post-wave I response for the second-click response could serve as a tool for detecting CS in humans. SIGNIFICANCE: Our findings shed new light on the analytical methods of ABR for quantifying CS.

A Clinical Neurological Approach to the Child With Adenosine Deaminase Deficiency.

BACKGROUND: Severe combined immunodeficiency secondary to adenosine deaminase deficiency is rare. The deficiency of this enzyme results in the accumulation of substrates in the tissues, including the brain. Clinical signs of neurological involvement may include seizures, neurodevelopmental disorders, hypotonia, and sensorineural hearing loss. Hematopoietic stem cell transplantation corrects the failure of the immune system but not the neurological involvement. OBJECTIVES: To describe the spectrum of neurological complications identified in a series of children with severe combined immunodeficiency due to adenosine deaminase deficiency. Additionally, we propose a neurological approach including electrophysiological, radiological, and neurocognitive studies to address this group of children in an efficient and timely manner. METHODS: A descriptive, observational, retro-, and prospective analysis of patients with a confirmed immunological diagnosis seen between 1996 and 2021 and referred to the Department of Neurology for neurological evaluation was conducted. RESULTS: Ten patients met the inclusion criteria. The median age at diagnosis was 4 months (range, 1-36 months). All patients had neurodevelopmental delay with hypotonia in six, language delay in three, sensorineural hearing loss in four, and spastic paraparesis in one patient. Two children developed an epileptic syndrome, consisting of generalized epilepsy in one and focal epilepsy in the other. Neuroimaging showed brain calcifications in the basal ganglia and/or centrum semiovale in four patients and enlarged subarachnoid spaces in two other patients. CONCLUSION: In this pediatric series, the rate of neurological involvement associated with abnormalities on neuroimaging was high. Although this involvement could be related to accumulation of adenosine metabolites in the central nervous system, the possibility of associated chronic infections should be ruled out. Given the neurological manifestations, it is important to involve the pediatric neurologist in the multidisciplinary follow-up team.

The Neural Representation of Binaural Sound Localization Cues Across Different Subregions of the Chicken's Inferior Colliculus.

The sound localization behavior of the nocturnally hunting barn owl and its underlying neural computations is a textbook example of neuroethology. Differences in sound timing and level at the two ears are integrated in a series of well-characterized steps, from brainstem to inferior colliculus (IC), resulting in a topographical neural representation of auditory space. It remains an important question of brain evolution: How is this specialized case derived from a more plesiomorphic pattern? The present study is the first to match physiology and anatomical subregions in the non-owl avian IC. Single-unit responses in the chicken IC were tested for selectivity to different frequencies and to the binaural difference cues. Their anatomical origin was reconstructed with the help of electrolytic lesions and immunohistochemical identification of different subregions of the IC, based on previous characterizations in owl and chicken. In contrast to barn owl, there was no distinct differentiation of responses in the different subregions. We found neural topographies for both binaural cues but no evidence for a coherent representation of auditory space. The results are consistent with previous work in pigeon IC and chicken higher-order midbrain and suggest a plesiomorphic condition of multisensory integration in the midbrain that is dominated by lateral panoramic vision.

Effect of combining batroxobin with acupuncture treatment on hearing recovery in patients with sudden sensorineural hearing loss.

BACKGROUND: The effect of batroxobin on hearing recovery in patients with Sudden Sensorineural Hearing Loss (SSNHL) is still controversial, and acupuncture shows auxiliary benefits for SSNHL. OBJECTIVES: To investigate the effectiveness of combining acupuncture with batroxobin therapy for patients with SSNHL. MATERIAL AND METHODS: One hundred and fifty-six patients with SSNHL were retrospectively enrolled in this study, and categorized into the control group (only batroxobin treatment) and observation group (batroxobin and acupuncture treatment). Pure Tone Audiograms (PTA) threshold and clinical outcomes of hearing recovery were compared. Logistic regression analysis was used to evaluate the association between hearing recovery and potential risk factors. RESULTS: Compared to the control group, the observation group had a higher overall effective rate (p = 0.006) and improvement in PTA threshold (p = 0.007). Among SSNHL patients with high-frequency and flat-type hearing loss, observation group demonstrated superior hearing recovery post-treatment compared to the control group (p < 0.05). Additionally, hearing recovery in patient with SSNHL were associated with SSNHL types, disease duration, neutrophil count and acupuncture (p < 0.05). CONCLUSIONS AND SIGNIFICANCE: Combining batroxobin and acupuncture treatments enhences the improvement of hearing recovery in SSNHL patients compared to only batroxobin treatments, especially high-frequency and flat-type hearing loss.

Access to Sudden Hearing Loss Care at Urgent Care Centers.

OBJECTIVES: To compare patient access to urgent care centers (UCCs) with a diagnosis of sudden hearing loss based on insurance. METHODS: One hundred twenty-five random UCCs in states with Medicaid expansion and 125 random UCCs in states without Medicaid expansion were contacted by a research assistant posing as a family member seeking care on behalf of a patient with a one-week history of sudden, unilateral hearing loss. Each clinic was called once as a Medicaid patient and once as a private insurance (PI) patient for 500 total calls. Each phone encounter was evaluated for insurance acceptance and self-pay price. Secondary outcomes included other measures of timely/accessible care. Chi-square/McNemar's tests and independent/paired sample t-tests were performed to determine whether there were statistically significant differences between expansion status and insurance type. Calls ended before answering questions were not included in the analysis. RESULTS: Medicaid acceptance rate was significantly lower than PI (68.1% vs. 98.4%, p < 0.001). UCCs in Medicaid expansion states were significantly more likely to accept Medicaid (76.8% vs. 59.2%, p = 0.003). The mean wage-adjusted self-pay price was significantly greater in states with Medicaid expansion at $169.84 than in states without at $145.34 when called as a Medicaid patient (mean difference: $24.50, 95% Confidence Interval: $0.45-$48.54, p = 0.046). The rates of referral to an emergency department and self-pay price nondisclosure rates were greater for Medicaid calls than for private insurance calls (8.2% vs. 0.4% and 17.4% vs. 5.8%; p < 0.001 for both). CONCLUSION: Medicaid patients with otologic emergencies face reduced access to care at UCCs. LEVEL OF EVIDENCE: NA Laryngoscope, 2024.

Outcomes of Aural Rehabilitation Provided in Person or by Telehealth Among Deaf/Hard of Hearing Young Children with Cochlear Implants or Hearing Aids.

Background: Cochlear implants and hearing aids may facilitate the development of listening and spoken language (LSL) in deaf/hard of hearing young children, but they require aural rehabilitation therapy-often unavailable outside urban areas-for optimal outcomes. This trial assessed the relative effectiveness of LSL therapy delivered either in person or by interactive video. The hypothesis was that telehealth service delivery would be noninferior to in-person therapy. Methods: Most parents refused randomization of their children to telehealth or in-person conditions; therefore, randomization was impossible. In consultation with the funder (NIDCD), the study design was modified. Parents were allowed to select their preferred study condition, and the study team was blinded to group membership. Forty-two families were in the in-person group and 35 in telehealth (40 and 30, respectively, after attrition). Primary endpoints were total score, auditory comprehension, and expressive communication on the Preschool Language Scale, 5th edition. There were several secondary speech, hearing, and language outcome measures. Assessments occurred at baseline and at follow-up after 6 months of LSL therapy. Results: Propensity scores were used to create two matched groups. At baseline, groups did not differ on PLS-5 scores. Change from baseline to F/U on age-equivalents for all three scores was nearly identical for both groups, although the telehealth group was younger, on average, than the in-person group. Discussion: Telehealth was noninferior to in-person services for all primary endpoints. For secondary outcomes, neither group demonstrated a significant advantage. Magnitudes of estimated group differences were small, suggesting nonsignificant differences not predominantly because of sample size. The telehealth group showed greater improvement on 15/24 of secondary language outcome measures. The findings provide evidence that telehealth is equivalent to in-person care for providing LSL therapy to young children with cochlear implants and hearing aids.

Awareness, attitudes and perceptions of students towards leisure noise in Durban, South Africa.

BACKGROUND:  Young adults are exposed to high noise levels in leisure venues, which increases their risk of hearing loss, and can affect their quality of life. OBJECTIVES:  The aim of this study was to describe the young adults' awareness, attitudes and perceptions towards leisure noise at a university in South Africa. METHOD:  A descriptive cross-sectional study design with quantitative methods of data was considered for this study. Students from first to fourth years in the Education Department of a local university in Durban, South Africa, who were aged 18 years old - 25 years old were invited to participate in an online survey. RESULTS:  Of the 462 participants, most had a general awareness on noise and hearing loss but lacked knowledge on the negative effect of loud noise, with 95.2% using personal listening devices, followed by visiting restaurants and gyms, and 48.3% being unsure if noise can damage hearing permanently. They were unaware of methods to reduce their exposure to noise. A significant relationship between awareness of noise and attitudes (p = 0.029) indicated that the higher the level of awareness regarding leisure noise, the better their attitude and behaviour, thus the lower the risk of hearing loss. CONCLUSION:  The results highlight the need for implementing the World Health Organization (WHO) noise regulations and providing education for this age group to prevent irreversible hearing loss through exposure to leisure noise.Contribution: A national study is recommended to increase research evidence.

High prevalence of m.1555A > G in patients with hearing loss in the Baikal Lake region of Russia as a result of founder effect.

Mitochondrial forms account approximately 1-2% of all nonsyndromic cases of hearing loss (HL). One of the most common causative variants of mtDNA is the m.1555A > G variant of the MT-RNR1 gene (OMIM 561000). Currently the detection of the m.1555A > G variant of the MT-RNR1 gene is not included in all research protocols. In this study this variant was screened among 165 patients with HL from the Republic of Buryatia, located in the Baikal Lake region of Russia. In our study, the total contribution of the m.1555A > G variant to the etiology of HL was 12.7% (21/165), while the update global prevalence of this variant is 1.8% (863/47,328). The m.1555A > G variant was notably more prevalent in Buryat (20.2%) than in Russian patients (1.3%). Mitogenome analysis in 14 unrelated Buryat families carrying the m.1555A > G variant revealed a predominant lineage: in 13 families, a cluster affiliated with sub-haplogroup A5b (92.9%) was identified, while one family had the D5a2a1 lineage (7.1%). In a Russian family with the m.1555A > G variant the lineage affiliated with sub-haplogroup F1a1d was found. Considering that more than 90% of Buryat families with the m.1555A > G variant belong to the single maternal lineage cluster we conclude that high prevalence of this variant in patients with HL in the Baikal Lake region can be attributed to a founder effect.

Horizontal Sound Localization and Spatial Short-Term Memory Span in Hearing-Impaired Listeners and Listeners With Simulated Hearing Loss.

Background and Objectives: : Localization of a sound source in the horizontal plane depends on the listener's interaural comparison of arrival time and level. Hearing loss (HL) can reduce access to these binaural cues, possibly disrupting the localization and memory of spatial information. Thus, this study aimed to investigate the horizontal sound localization performance and the spatial short-term memory in listeners with actual and simulated HL. Subjects and Methods: : Seventeen listeners with bilateral symmetric HL and 17 listeners with normal hearing (NH) participated in the study. The hearing thresholds of NH listeners were elevated by a spectrally shaped masking noise for the simulations of unilateral hearing loss (UHL) and bilateral hearing loss (BHL). The localization accuracy and errors as well as the spatial short-term memory span were measured in the free field using a set of 11 loudspeakers arrayed over a 150° arc. Results: : The localization abilities and spatial short-term memory span did not significantly differ between actual BHL listeners and BHL-simulated NH listeners. Overall, the localization performance with the UHL simulation was approximately twofold worse than that with the BHL simulation, and the hearing asymmetry led to a detrimental effect on spatial memory. The mean localization score as a function of stimulus location in the UHL simulation was less than 30% even for the front (0° azimuth) stimuli and much worse on the side closer to the simulated ear. In the UHL simulation, the localization responses were biased toward the side of the intact ear even when sounds were coming from the front. Conclusions: : Hearing asymmetry induced by the UHL simulation substantially disrupted the localization performance and recall abilities of spatial positions encoded and stored in the memory, due to fewer chances to learn strategies to improve localization. The marked effect of hearing asymmetry on sound localization highlights the need for clinical assessments of spatial hearing in addition to conventional hearing tests.