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1.
J Audiol Otol ; 2024 Jul 02.
Article in English | MEDLINE | ID: mdl-38946331

ABSTRACT

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.

2.
Int J Audiol ; : 1-11, 2024 Jul 01.
Article in English | MEDLINE | ID: mdl-38949044

ABSTRACT

OBJECTIVE: Describe how the Revised Hearing Handicap Inventory (RHHI) changes over time and determine associated factors. DESIGN: Data were from a community-based cohort study. Linear regression models were used to estimate mean baseline and final RHHI scores and change (final minus baseline score). Logistic regression models were used to determine factors associated with substantial RHHI change, defined as ±6 points. Factors included baseline age, sex, race, hearing aid use, and baseline pure-tone average (PTA; 0.5, 1.0, 2.0, 4.0 kHz, worse ear). STUDY SAMPLE: This study included 583 participants (mean age: 66.4 [SD 9.1] years; 59.9% female; 14.2% Minority race) with a mean follow-up time of 7.6 (SD 4.9) years. RESULTS: Baseline and final RHHI scores were 7.9 and 9.2 points, corresponding to an average 1.3-point increase in hearing difficulty over time. Most participants (65.4%) did not show substantial RHHI change, whereas 21.4% and 13.2% experienced substantial increase and decrease, respectively. In separate multivariable models, PTA and hearing aid use were associated with substantial increase in hearing difficulty, and PTA was associated with substantial decrease. CONCLUSIONS: The average RHHI change was relatively small. Hearing aid use and PTA were associated with RHHI change.

3.
Int J Audiol ; : 1-4, 2024 Jul 01.
Article in English | MEDLINE | ID: mdl-38949065

ABSTRACT

OBJECTIVES: A case of sudden sensorineural hearing loss following use of sildenafil was examined in detail over a period of three days from first report to recovery. DESIGN: Case study. The subject presented with sudden sensorineural hearing loss and diplacusis a day after onset. Testing involved detailed interview, standard audiometry, detailed inter-octave audiometry, and measurement of detailed psychophysical frequency tuning curves during a two day recovery period. STUDY SAMPLE: One male aged in his thirties with otherwise normal hearing. RESULTS: Although standard audiometry was within normal limits, detailed inter-octave audiometry and psychophysical frequency tuning curves were consistent with a punctate unilateral intra-cochlear lesion that resolved over a period of three days. CONCLUSIONS: This is the first report of such a frequency-specific audiometric shift and diplacusis after sildenafil, and is not consistent with previous reports of direct ototoxic pharmacological effects. We propose that the lesion was most likely caused by a cochlear bleed, and may have been due to physical exertion rather than a direct pharmaceutical effect. The study highlights the important role of additional diagnostic testing that can be easily achieved in a clinical setting with minimal equipment.

4.
J Gene Med ; 26(7): e3714, 2024 Jul.
Article in English | MEDLINE | ID: mdl-38949079

ABSTRACT

Mouse models are used extensively to understand human pathobiology and mechanistic functions of disease-associated loci. However, in this review, we investigate the potential of using genetic mouse models to identify genetic markers that can disrupt hearing thresholds in mice and then target the hearing-enriched orthologues and loci in humans. Currently, little is known about the real prevalence of genes that cause hearing impairment (HI) in Africa. Pre-screening mouse cell lines to identify orthologues of interest has the potential to improve the genetic diagnosis for HI in Africa to a significant percentage, for example, 10-20%. Furthermore, the functionality of a candidate gene derived from mouse screening with heterogeneous genetic backgrounds and multi-omic approaches can shed light on the molecular, genetic heterogeneity and plausible mode of inheritance of a gene in hearing-impaired individuals especially in the absence of large families to investigate.


Subject(s)
Disease Models, Animal , Hearing Loss , Animals , Humans , Mice , Hearing Loss/genetics , Africa/epidemiology , Genetic Predisposition to Disease
5.
S Afr J Commun Disord ; 71(1): e1-e10, 2024 Jun 28.
Article in English | MEDLINE | ID: mdl-38949431

ABSTRACT

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.


Subject(s)
Health Knowledge, Attitudes, Practice , Hearing Loss, Noise-Induced , Leisure Activities , Noise , Students , Humans , South Africa , Male , Female , Cross-Sectional Studies , Young Adult , Noise/adverse effects , Adult , Hearing Loss, Noise-Induced/prevention & control , Hearing Loss, Noise-Induced/etiology , Hearing Loss, Noise-Induced/psychology , Adolescent , Students/psychology , Awareness , Surveys and Questionnaires , Universities
6.
Eur Arch Otorhinolaryngol ; 281(7): 3839-3843, 2024 Jul.
Article in English | MEDLINE | ID: mdl-38825603

ABSTRACT

PURPOSE: The aim of the study is to capture the difference between the groups in direct relation to the type of electrode array insertion during cochlear implantation (CI). The robotic insertion is expected to be a more gently option. As recent studies have shown, there is a difference in perception of visual vertical (SVV) and postural control related to the CI. We assume that there can be differences in postural control and space perception outcomes depending on the type of the surgical method. METHODS: In total, 37 (24 females, mean age ± SD was 42.9 ± 13.0) candidates for CI underwent an assessment. In 14 cases, the insertion of the electrode array was performed by a robotic system (RobOtol, Colin, France) and 23 were performed conventionally. In all of these patients, we performed the same examination before the surgery, the first day, and 3 weeks after the surgery. The protocol consists of static posturography and perception of visual vertical. RESULTS: The both groups, RobOtol and conventional, responded to the procedure similarly despite the dissimilar electrode insertion. There was no difference between two groups in the dynamic of perception SVV and postural parameters. Patients in both groups were statistically significantly affected by the surgical procedure, SVV deviation appeared in the opposite direction from the implanted ear: 0.90° ± 1.25; - 1.67° ± 3.05 and - 0.19° ± 1.78 PRE and POST surgery (p < 0.001). And this deviation was spontaneously adjusted in FOLLOW-UP after 3 weeks (p < 0.01) in the both groups. We did not find a significant difference in postural parameters between the RobOtol and conventional group, even over time. CONCLUSION: Although the robotic system RobOtol allows a substantial reduction in the speed of insertion of the electrode array into the inner ear, our data did not demonstrate a postoperative effect on vestibular functions (SVV and posturography), which have the same character and dynamics as in the group with standard manual insertion. REGISTRATION NUMBER: The project is registered on clinicaltrials.gov (registration number: NCT05547113).


Subject(s)
Cochlear Implantation , Postural Balance , Robotic Surgical Procedures , Space Perception , Humans , Female , Cochlear Implantation/methods , Male , Adult , Robotic Surgical Procedures/methods , Middle Aged , Space Perception/physiology , Postural Balance/physiology , Treatment Outcome , Cochlear Implants
7.
bioRxiv ; 2024 May 21.
Article in English | MEDLINE | ID: mdl-38826449

ABSTRACT

Hearing loss is one of the most common types of disability; however, there is only one FDA-approved drug to prevent any type of hearing loss. Treatment with the highly effective chemotherapy agent, cisplatin, and exposure to high decibel noises are two of the most common causes of hearing loss. The mitogen activated protein kinase (MAPK) pathway, a phosphorylation cascade consisting of RAF, MEK1/2, and ERK1/2, has been implicated in both types of hearing loss. Pharmacologically inhibiting BRAF or ERK1/2 is protective from noise and cisplatin-induced hearing loss in multiple mouse models. Trametinib, a MEK1/2 inhibitor, protects from cisplatin induced outer hair cell death in mouse cochlear explants; however, to the best of our knowledge, inhibiting MEK1/2 has not yet been shown to be protective from hearing loss in vivo. In this study, we demonstrate that trametinib protects from cisplatin-induced hearing loss in a translationally relevant mouse model and does not interfere with cisplatin's tumor killing efficacy in cancer cell lines. Higher doses of trametinib were toxic to mice when combined with cisplatin but lower doses of the drug were protective from hearing loss without any known toxicity. Trametinib also protected mice from noise-induced hearing loss and synaptic damage. This study shows that MEK1/2 inhibition protects from both insults of hearing loss and that targeting all three kinases in the MAPK pathway protect from cisplatin and noise-induced hearing loss in mice.

8.
Article in English | MEDLINE | ID: mdl-38871535

ABSTRACT

Clinical applications of artificial intelligence (AI) have grown exponentially with increasing computational power and Big Data. Data rich fields such as Otology and Neurotology are still in the infancy of harnessing the power of AI but are increasingly involved in training and developing ways to incorporate AI into patient care. Current studies involving AI are focused on accessible datasets; health care wearables, tabular data from electronic medical records, electrophysiologic measurements, imaging, and "omics" provide huge amounts of data to utilize. Health care wearables, such as hearing aids and cochlear implants, are a ripe environment for AI implementation.

9.
Ear Nose Throat J ; : 1455613241261461, 2024 Jun 13.
Article in English | MEDLINE | ID: mdl-38872311

ABSTRACT

Objectives: This study aims to showcase the complementary nature of utilizing both histopathology and magnetic resonance imaging (MRI) in understanding the otologic pathophysiology of Meniere disease. In addition, it seeks to raise awareness of the value of preserving and curating historical temporal bone collections which continue to inform our understanding of otologic diseases. Methods: The essential anatomical feature of Meniere disease-the distended membranous labyrinth-is explored through a comparison of early temporal bone studies with contemporary MRI techniques. The histopathologic photomicrographs are of inner ear specimens from deceased patients with symptoms consistent with Meniere disease. The MRI sequences from living patients exhibiting classic Meniere disease symptoms during life are captured 4 hours post-administration of gadolinium. Results: Both histopathologic examination and MRI imaging reveal consistent distention of the saccule, utricle, and scala media in patients with Meniere disease. The study shows the histologic photomicrographs of actual Meniere patients compared to the MRIs and successfully demonstrates the correlation between postmortem histological findings and MRI evidence of distension in living patients. Conclusions: A corresponding distension of the membranous labyrinth is seen in both the histologic specimens and the Meniere MRIs. This correlation suggests the potential utility of utilizing MRI to aid in diagnosing atypical Meniere disease and distinguishing it from other disease processes, such as migraine equivalent vertigo. The integration of historical temporal bone studies with modern MRI techniques offers valuable insights into the pathophysiology of otologic diseases. In addition, it emphasizes the importance of preserving and curating historical temporal bone collections for continued research and medical education purposes. Previous studies of delayed MRIs did not use Meniere disease temporal bone histopathology images.

10.
Sci Rep ; 14(1): 13376, 2024 06 10.
Article in English | MEDLINE | ID: mdl-38862572

ABSTRACT

In individuals with hearing loss, protection of residual hearing is essential following cochlear implantation to facilitate acoustic and electric hearing. Hearing preservation requires slow insertion, atraumatic electrode and delivery of the optimal quantity of a pharmacological agent. Several studies have reported variable hearing outcomes with osmotic pump-mediated steroid delivery. New drugs, such as sialyllactose (SL) which have anti-inflammatory effect in many body parts, can prevent tissue overgrowth. In the present study, the positive effects of the pharmacological agent SL against insults were evaluated in vitro using HEI-OC1 cells. An animal model to simulate the damage due to electrode insertion during cochlear implantation was used. SL was delivered using osmotic pumps to prevent loss of the residual hearing in this animal model. Hearing deterioration, tissue fibrosis and ossification were confirmed in this animal model. Increased gene expressions of inflammatory cytokines were identified in the cochleae following dummy electrode insertion. Following the administration of SL, insertion led to a decrease in hearing threshold shifts, tissue reactions, and inflammatory markers. These results emphasize the possible role of SL in hearing preservation and improve our understanding of the mechanism underlying hearing loss after cochlear implantation.


Subject(s)
Cochlear Implantation , Hearing Loss , Lactose , Animals , Lactose/analogs & derivatives , Lactose/pharmacology , Hearing Loss/prevention & control , Hearing Loss/drug therapy , Hearing/drug effects , Cochlea/drug effects , Cochlea/metabolism , Mice , Disease Models, Animal , Cell Line , Cytokines/metabolism , Male , Sialic Acids
11.
Cureus ; 16(5): e60214, 2024 May.
Article in English | MEDLINE | ID: mdl-38868294

ABSTRACT

INTRODUCTION: Aerosol mitigation equipment implemented due to COVID-19 has increased noise levels in the operating room (OR) during otolaryngological procedures. Intraoperative sound levels may potentially place personnel at risk for occupational hearing loss. This study hypothesized that cumulative intraoperative noise exposures with aerosol mitigation equipment exceed recommended occupational noise exposure levels. METHODS: Sound levels generated by the surgical smoke evacuator (SSE) during adenotonsillectomy were measured using a sound level meter and compared to surgery without SSE. RESULTS: Thirteen adenotonsillectomy surgeries were recorded. Mean sound levels with the SSE were greater than the control (72 ± 3 A-weighted decibels (dBA) vs. 68 ± 2 dBA; p=0.015). Maximum noise levels during surgery with SSE reached 82 ± 3 dBA. CONCLUSION: Surgeons performing adenotonsillectomy with aerosol mitigation equipment are exposed to significant noise levels. Intraoperative sound levels exceeded international standards for work requiring concentration. Innovation is needed to reduce cumulative OR noise exposures.

12.
J Environ Manage ; 363: 121413, 2024 Jul.
Article in English | MEDLINE | ID: mdl-38850921

ABSTRACT

As urbanization and population growth escalate, the challenge of noise pollution intensifies, particularly within the aviation industry. This review examines current insights into noise-induced hearing loss (NIHL) in aviation, highlighting the risks to pilots, cabin crew, aircraft maintenance engineers, and ground staff from continuous exposure to high-level noise. It evaluates existing noise management and hearing conservation strategies, identifying key obstacles and exploring new technological solutions. While progress in developing protective devices and noise control technologies is evident, gaps in their widespread implementation persist. The study underscores the need for an integrated strategy combining regulatory compliance, technological advances, and targeted educational efforts. It advocates for global collaboration and policy development to safeguard the auditory health of aviation workers and proposes a strategic framework to enhance hearing conservation practices within the unique challenges of the aviation sector.


Subject(s)
Aviation , Hearing Loss, Noise-Induced , Noise, Occupational , Hearing Loss, Noise-Induced/prevention & control , Humans , Noise, Occupational/prevention & control , Noise, Occupational/adverse effects , Occupational Exposure/prevention & control , Aircraft
13.
Eur J Med Genet ; 69: 104952, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38852772

ABSTRACT

21-hydroxylase deficiency stands as the most prevalent form of congenital adrenal hyperplasia, primarily resulting from mutations in the CYP21A2 gene. On the other hand, mutations within the CYP17A1 gene lead to 17α-hydroxylase/17,20-lyase enzyme deficiencies. The scarcity of 17-OH deficiency is noteworthy, accounting for less than 1% of all congenital adrenal hyperplasia cases. The male patient, born from a first-degree cousin marriage, exhibited several symptoms, including left undescended testis, micropenis, penile chord, left sensorineural hearing loss, and gynecomastia. He reported micropenis as a concern at the age of 13.5 years. His hormone profile revealed high levels of serum 17-hydroxyprogesterone, progesterone, and pregnenolone. In this case with a 46 XY karyotype, suspicions arose regarding Cytochrome P450 oxidoreductase deficiency due to ambiguous genitalia and an atypical hormone profile. Analysis unveiled two distinct homozygous and pathogenic variants in the CYP21A2 and CYP17A1 genes. Notably, mineralocorticoid precursors escalated, while cortisol and sex steroid precursors decreased during the high (250 mcg) dose ACTH stimulation test. The mutation c.1169C > G (p.Thr390Arg) in CYP17A1, which is the second documented case in literature, stands out due to its unique set of accompanying features. Mutations occurring in CYP21A2 and CYP17A1 result in complete or partial enzyme deficiencies, and the detection of homozygous mutations in two different enzyme systems within the steroidogenic pathway is noteworthy.


Subject(s)
Adrenal Hyperplasia, Congenital , Steroid 17-alpha-Hydroxylase , Steroid 21-Hydroxylase , Humans , Adrenal Hyperplasia, Congenital/genetics , Male , Steroid 17-alpha-Hydroxylase/genetics , Steroid 21-Hydroxylase/genetics , Adolescent , Mutation
14.
Conscious Cogn ; 123: 103718, 2024 Jun 15.
Article in English | MEDLINE | ID: mdl-38880020

ABSTRACT

The phenomenon of "hearing voices" can be found not only in psychotic disorders, but also in the general population, with individuals across cultures reporting auditory perceptions of supernatural beings. In our preregistered study, we investigated a possible mechanism of such experiences, grounded in the predictive processing model of agency detection. We predicted that in a signal detection task, expecting less or more voices than actually present would drive the response bias toward a more conservative and liberal response strategy, respectively. Moreover, we hypothesized that including sensory noise would enhance these expectancy effects. In line with our predictions, the findings show that detection of voices relies on expectations and that this effect is especially pronounced in the case of unreliable sensory data. As such, the study contributes to our understanding of the predictive processes in hearing and the building blocks of voice hearing experiences.

15.
Article in English | MEDLINE | ID: mdl-38881377

ABSTRACT

OBJECTIVE: To determine whether immigrant status is associated with likelihood of audiogram and hearing aid use among US adults with hearing loss. STUDY DESIGN: Cross-sectional study. SETTING: Nationally representative data from 2009 to 2010, 2011 to 2012, 2015 to 2016, and 2017 to 2020 National Health and Nutrition Examination Survey (NHANES) cycles. METHODS: This cross-sectional study of 4 merged cycles of NHANES included 12,455 adults with subjective (self-reported) or objective (audiometric) hearing loss. Sequentially adjusted logistic regressions were used to assess the association of immigration status with likelihood of having undergone audiogram among those with objective and self-reported hearing loss, and with likelihood of hearing aid use among candidates with objective hearing loss. RESULTS: Immigrants were less likely to have received an audiogram among subjects with subjective (odds ratio [OR]: 0.81, 95% confidence interval [CI]: 0.75-0.87), and objective (OR: 0.76, 95% CI: 0.72-0.81) hearing loss, compared to nonimmigrants. The association persisted for those with subjective (OR: 0.88, 95% CI: 0.81-0.96), and objective (OR: 0.87, 95% CI: 0.80-0.96) hearing loss after adjusting for sociodemographic factors, comorbidities, insurance, and hearing quality, but disappeared in both groups after adjusting for English proficiency. Immigrants were less likely to use hearing aids (OR: 0.90, 95% CI: 0.87-0.93). However, this association disappeared (OR: 0.98, 95% CI: 0.93-1.04) in the adjusted model. CONCLUSION: Immigrant status is a significant barrier to hearing health care and is associated with lower rates of audiometric testing and hearing aid use among individuals with hearing loss.

16.
Article in English | MEDLINE | ID: mdl-38881387

ABSTRACT

OBJECTIVE: To identify the prevalence of and relevant information for video head impulse test (vHIT) abnormality in a large population. STUDY DESIGN: A cross-sectional design. SETTING: Korean National Health and Nutrition Examination Survey, 2021. METHODS: The sample was representative of the Korean population, with 2237 participants aged ≥40 years. A vHIT was performed to evaluate vestibular function. The vestibulo-ocular reflex (VOR) gain and the presence of reproducible catch-up saccades was assessed in a vHIT. Participants also completed questionnaires for demographics, socioeconomic status, and basic information regarding systemic diseases and dizziness and underwent hearing tests with automated pure-tone audiometry. RESULTS: The prevalence of vHIT abnormality was 22.5%, with unilateral (14.3%) being more common than bilateral (8.2%). The prevalence of vHIT abnormality increased significantly with age, with the highest rate observed in individuals aged >70 years (42.5%). Both hearing and VOR gain deteriorated with age, but the patterns of age-related progression were different. While hearing loss (HL) deteriorated gradually and progressively throughout adulthood, VOR gain deterioration was markedly evident after 70 years of age. CONCLUSION: Considering the high prevalence of vHIT abnormality, appropriate social and medical policies are needed to prevent associated injuries and improve patients' quality of life. The distinct age-related changes in HL and objective findings of vestibular dysfunction indicate the need for different approaches to address these social problems in aging countries.

17.
Article in English | MEDLINE | ID: mdl-38881393

ABSTRACT

OBJECTIVE: Operating room (OR) sounds may surpass noise exposure thresholds and induce hearing loss. Noise intensity emitted by various surgical instruments during common pediatric otolaryngologic procedures were compared at the ear-level of the surgeon and patient to evaluate the need for quality improvement measures. STUDY DESIGN: Cross-sectional study. SETTING: Single tertiary care center. METHODS: Noise levels were measured using the RISEPRO Sound Level Meter and SoundMeter X 10.0.4 at the ear level of surgeon and patient every 5 minutes. Operative procedure and instrument type were recorded. Measured noise levels were compared against ambient noise levels and the Apple Watch Noise application. RESULTS: Two hundred forty-two total occasions of noise were recorded across 62 surgical cases. Cochlear implantation surgery produces the loudest case at the ear-level of the patient (91.8 Lq Peak dB; P < .001). The otologic drill was the loudest instrument for the patient (92.1 Lq Peak dB; P < .001), while the powered microdebrider was the loudest instrument for the surgeon (90.7 Lq Peak dB; P = .036). Noise measurements between surgeon and patient were similar (P < .05). Overall agreement between the Noise application and Sound Level Meter was excellent (intraclass correlation coefficient of 0.8, with a 95% confidence interval ranging from 0.32 to 0.92). CONCLUSION: Otolaryngology OR noises can surpass normal safe thresholds. Failure to be aware of this may unwittingly expose providers to noise-related hearing loss. Mitigation strategies should be employed. Quality improvement measures, including attention to surgical instrument volume settings and periodic decibel measurements with sound applications, can promote long-term hearing conservation. DISCUSSION: Otolaryngology OR noises can surpass normal safe thresholds. Failure to be aware of this may unwittingly expose providers to noise-related hearing loss. The duration, frequency of exposure, and volume levels of noise should be studied further. IMPLICATIONS FOR PRACTICE: Mitigation strategies should be employed. Quality improvement measures, including attention to surgical instrument volume settings and periodic decibel measurements with sound applications, can promote long-term hearing conservation.

18.
J Biol Chem ; : 107474, 2024 Jun 13.
Article in English | MEDLINE | ID: mdl-38879011

ABSTRACT

Hearing, the ability to sense sounds, and the processing of auditory information are important for perception of the world. Mice lacking expression of neuroplastin (Np), a type-1 transmembrane glycoprotein, display deafness, multiple cognitive deficiencies, and reduced expression of plasma membrane calcium (Ca2+) ATPases (PMCAs) in cochlear hair cells and brain neurons. In this study, we transferred the deafness causing missense mutations pitch (C315S) and audio-1 (I122N) into human Np (hNp) constructs and investigated their effects at the molecular and cellular level. Computational molecular dynamics show that loss of the disulfide bridge in hNppitch causes structural destabilization of immunoglobulin-like domain (Ig) III and that the novel asparagine in hNpaudio-1 results in steric constraints and an additional N-glycosylation site in IgII. Additional N-glycosylation of hNpaudio-1 was confirmed by PNGaseF treatment. In comparison to hNpWT, transfection of hNppitch and hNpaudio-1 into HEK293T cells resulted in normal mRNA levels but reduced the Np protein levels and their cell surface expression due to proteasomal/lysosomal degradation. Furthermore, hNppitch and hNpaudio-1 failed to promote exogenous PMCA levels in HEK293T cells. In hippocampal neurons, expression of additional hNppitch or hNpaudio-1 was less efficient than hNpWT to elevate endogenous PMCA levels and to accelerate the restoration of basal Ca2+ levels after electrically-evoked Ca2+ transients. We propose that mutations leading to pathological Np variants, as exemplified here by the deafness causing Np mutants, can affect Np-dependent Ca2+ regulatory mechanisms and may potentially cause intellectual and cognitive deficits in humans.

19.
Growth Horm IGF Res ; 76: 101594, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38833815

ABSTRACT

OBJECTIVES: This study aims to investigate whether the middle ear resonance frequency (RF) is affected in acromegaly, which causes growth in the skull bone. METHODS: Thirty acromegaly patients and 38 volunteers were included in the study. Pure tone average scores and middle ear RF values of the groups that underwent pure tone audiometry, tympanometry, and multifrequency tympanometry tests were compared. RESULTS: The pure tone mean was 14.95 ± 12.13 in acromegaly patients and 5.70 ± 8.52 in the control group (p:0.18). Sensorineural hearing loss(SNHL) was observed in 16.6% of the patients. The average middle ear RF was calculated as 815 ± 179.05 Hz in patients with acromegaly and 773 ± 127.15 in the control group. (p = 0.0001). CONCLUSION: This study is the first to evaluate middle-ear RF in acromegaly patients. Acromegaly-induced changes in soft tissues and bone structures impact middle ear functions. In this patient group, we found an increase in middle ear RF without conductive-type hearing loss and a 16.6% rate of SNHL.


Subject(s)
Acromegaly , Ear, Middle , Skull , Humans , Acromegaly/physiopathology , Acromegaly/pathology , Female , Ear, Middle/pathology , Male , Adult , Skull/diagnostic imaging , Middle Aged , Case-Control Studies , Hearing Loss, Sensorineural , Acoustic Impedance Tests , Audiometry, Pure-Tone , Prognosis
20.
Sci Rep ; 14(1): 13685, 2024 06 13.
Article in English | MEDLINE | ID: mdl-38871776

ABSTRACT

Low-grade chronic inflammation is associated with many age-related conditions. Non-invasive methods to monitor low-grade chronic inflammation may improve the management of older people at risk of poorer outcomes. This longitudinal cohort study has determined baseline inflammation using neopterin volatility in monthly urine samples of 45 independent older adults (aged 65-75 years). Measurement of neopterin, an inflammatory metabolite, enabled stratification of individuals into risk categories based on how often in a 12-month period their neopterin level was raised. Hearing was measured (pure-tone audiometry) at baseline, 1 year and 3 years of the study. Results show that those in the highest risk category (neopterin raised greater than 50% of the time) saw greater deterioration, particularly in high-frequency, hearing. A one-way Welch's ANOVA showed a significant difference between the risk categories for change in high-frequency hearing (W (3, 19.6) = 9.164, p = 0.0005). Despite the study size and duration individuals in the highest risk category were more than twice as likely to have an additional age-related morbidity than those in the lowest risk category. We conclude that volatility of neopterin in urine may enable stratification of those at greatest risk of progression of hearing loss.


Subject(s)
Neopterin , Humans , Neopterin/urine , Aged , Male , Female , Longitudinal Studies , Hearing Loss/urine , Audiometry, Pure-Tone , Biomarkers/urine , Auditory Threshold , Inflammation/urine
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