Severe Hearing Loss Research Articles

Audiometric Outcomes in Chronic Otitis Media with Mastoid Involvement: A Five-Year Clinical Overview

Background/Objectives. Otomastoiditis, an inflammatory condition affecting the middle ear and mastoid cells, poses significant risks for hearing impairment. This study aimed to analyze the clinical presentations, anatomical variations, and audiometric outcomes associated with acute and chronic otomastoiditis over a five-year period at the ENT Clinic of the Clinical County Emergency Hospital of Craiova. Methods. A retrospective clinical–statistical analysis was conducted on 145 patients aged 2 to 78 years, who were treated for otomastoiditis. The study involved a comprehensive review of clinical and audiometric data, with a focus on the type of hearing loss (conductive or mixed), audiometric thresholds, and the relationship between the anatomical form of the disease and the severity of hearing loss. Results. The majority of cases (93.83%) were chronic otomastoiditis, with 66.89% of patients presenting with mixed hearing loss and 33.10% with conductive hearing loss. Audiometric assessments revealed significant air conduction deficits, particularly at low and mid-range frequencies, with losses averaging 50–55 dB in cases of conductive hearing loss. Chronic cases demonstrated notable bone conduction impairments, indicating progressive cochlear damage. Statistical analysis identified a moderate correlation between the anatomical form of the disease and the severity of hearing loss, particularly in patients with cholesteatomatous-suppurative forms. Conclusions. This study underlines the critical need for the early and precise diagnosis of otomastoiditis, supported by audiometric evaluations. Our findings emphasize the substantial risk of progressive cochlear damage in chronic cases, underscoring the necessity for timely intervention to mitigate long-term hearing loss. These results offer valuable insights for clinicians, potentially guiding improved therapeutic approaches and contributing to enhanced patient outcomes in managing chronic otomastoiditis.

Polygenic Risk Scores and Hearing Loss Phenotypes in Children

Monogenic causes of childhood hearing loss are well established, as are polygenic risk contributions to age-related hearing loss. However, an untested possibility is that polygenic risk scores (PRS) also contribute to childhood hearing loss of all severities, alongside environmental and/or monogenic causes. To examine the association between a PRS for adult hearing loss and childhood hearing loss phenotypes. This cross-sectional study used a unique population-based dataset spanning normal hearing to profound loss, combining 2 contemporaneous population cohorts in Australia. This included the Child Health CheckPoint, a national population-based cross-sectional study nested within the Longitudinal Study of Australian Children, and the Victorian Childhood Hearing Longitudinal Databank (VicCHILD), a statewide population-based longitudinal data bank open to every child with congenital hearing loss in Victoria, Australia. The analysis took place from March to August 2023. Genotype data were generated from saliva- or blood-derived DNA using global single-nucleotide variations arrays. Based on genotype data, PRS was computed using published UK Biobank genome-wide association study results for self-reported hearing difficulty in individuals aged 40 to 69 years. Hearing outcomes were classified by laterality (bilateral, unilateral), severity (mild, moderate, severe or worse) and types (sensorineural, conductive, mixed, auditory neuropathy, atresia). Analyses included multinominal logistic regressions of PRS with hearing outcomes. Overall, 1488 CheckPoint study children (49.8% boys, aged 11-12 years) and 527 VicCHILD study children (55.2% boys, aged 0-13 years) with hearing and genotype data were included. A 1-SD increment in PRS was associated with higher odds of mild (odds ratio [OR], 1.3; 95% CI, 1.0-1.6), moderate (OR, 5.1; 95% CI, 3.2-8.1), and severe or worse (OR, 5.3; 95% CI, 3.9-7.3) unilateral hearing loss compared with normal hearing. Similarly, the PRS was associated with increased odds of mild, moderate, and severe or worse bilateral hearing loss (per-SD ORs, 3.9-6.6) and all hearing loss types (per-SD ORs, 8.5-10.6). In this cross-sectional study, a PRS initially developed for adult hearing difficulty was associated with wide-ranging childhood hearing loss phenotypes, partly explaining hearing phenotype variations despite shared genetic and environmental factors (eg, preterm birth). Large-scale studies with objectively defined hearing phenotypes are crucial for refining PRS and predicting high-risk children.

The Role of Hearing Aids in Improving Dual-Task Gait Performance in Older Adults With Presbycusis: A Cognitive and Motor Analysis.

Cognitive decline is a common challenge faced by older individuals with presbycusis; their performance on dual-task (DT) activities is generally lower compared to those without hearing loss. However, the influence of hearing aids on nonauditory cognitive decline in this population remains unclear. This study aims to investigate the effect of hearing aids on nonauditory DT performance in older adults with presbycusis. This study included older people with presbycusis who used hearing aids (P&HA group), those with presbycusis who did not use hearing aids (PoHA group), and a control group of healthy adults. Forward and backward digit span tests and timed up and go test (TUG) were administered to all individuals. TUG and motor and cognitive (forward and backward digit span) tasks were applied simultaneously to evaluate the participants' DT performance. The study comprised 60 individuals with presbycusis (19 females, 41 males; mean age: 73.23±6.49years) and 30 healthy adults (15 females, 15 males; mean age: 35.93±8.57years). Healthy adults performed better than the P&HA and PoHA groups in all the administered tests (p<0.05). There was a moderate negative relationship between the severity of hearing loss and the forward and backward digit span test performances (p<0.05). The P&HA group performed better than the PoHA group on the DT cognitive forward and backward digit span tests. The use of hearing aids and their proper fitting are important not only for improving communication skills and reducing listening effort but also for supporting nonauditory cognitive functions, minimizing the risk of falls during DT activities, and enhancing the overall quality of life.

Sensorineural hearing loss among hypertensive patients attending cardiac institute in Tanzania: A cross-sectional study

INTRODUCTION Hypertension is among non-communicable diseases worldwide and it has been associated with sensorineural hearing loss. Almost 1.39 billion people (1 out of 4 adults) have hypertension and this figure is expected to increase. In Sub-Saharan Africa, hypertension is the leading cause of death and disability. Hypertension is associated with sensorineural hearing loss by compromising the blood supply in the cochlea. MATERIALS AND METHODS An assessment of the proportion and patterns of sensorineural hearing loss among hypertensive patients was conducted. 191 patients aged 18 years and above were recruited. History and physical examination including blood pressure, and otoscopy were done. Pure tone audiometry (PTA) was conducted. RESULTS Patients aged from 36 to 88 years with a mean age of 60 years and a standard deviation of 10. The patients aged between 61–76 years accounted for 85 (44.5%), whereas 29–44 and &gt; 76 years accounted for 12 (6.3%) each. The female-to-male ratio was 1:1. Sensorineural hearing loss was detected in 144 (75.4%), commonly above 76 years of age which was 11 (91.7%. Mild sensorineural hearing loss was the most common degree revealed. There was no relationship between the degree of sensorineural hearing loss and the grades of hypertension (p-value of 0.113). No relationship between the duration of hypertension with the severity of hearing loss, p-value =0.369. CONCLUSION The prevalence of sensorineural hearing loss among hypertensive patients was 75.4%. The bilateral and mild degree was the commonest presentation. Increasing age was found to be associated with increased hearing loss. The duration and grade of hypertension were not related to the severity of hearing loss.

Smart noise pollution monitoring system

Increasing pollution poses serious health risks, necessitating urgent control measures for a healthier future for all. Among various pollution types, sound pollution significantly impacts quality of life and well-being, especially in densely populated urban areas. This proposal describes the creation and deployment of an Internet of Things (IoT)-based noise pollution monitoring system intended to track sound levels in certain regions in real time. The core of this system comprises advanced sensors strategically placed to measure sound intensity accurately. These sensors can detect even minor variations in noise levels, providing precise and reliable data. After being gathered, the data is sent to a microcontroller, which effectively processes it. This microcontroller ensures the data is accurately captured and sent over the internet to an online server. Once the data reaches the online server, it becomes accessible for analysis and monitoring by authorities. The system's real-time data capability is crucial for timely intervention. Authorities can continuously monitor noise levels and identify trends or spikes in sound pollution, allowing them to address issues promptly. This feature is particularly beneficial for sensitive areas such as schools, hospitals, and residential zones, areas with heavy traffic, library, Where excessive noise can have severe health effects, including stress, sleep disturbances, and hearing loss. The system includes an alert mechanism that notifies relevant authorities when noise levels exceed predefined thresholds. These alerts enable quick responses to mitigate noise pollution. For instance, in a school, reducing noise levels can enhance the learning atmosphere and improve students' concentration. In hospitals, maintaining a quiet environment is crucial for patient recovery. The integration of IoT technology in this noise pollution monitoring system not only enhances efficiency but also provides a scalable solution adaptable to various urban and rural settings. This scalability ensures the system can be expanded to cover larger areas or multiple locations as needed.

Angular Insertion Depth in Inner Ear Malformations, Relationship to Cochlear Size, and Implications for Electrode Selection.

The objectives were to determine the interrater agreement of the Skull AP X-ray in measuring angular insertion depth (AID), to provide descriptive information about the insertion depths of different electrodes used in inner ear malformations (IEMs), to investigate the effect of cochlear size and electrode length on AID, and to guide clinicians in electrode selection in IEMs. Retrospective case review. Tertiary referral center. A total of 198 IEMs (n = 169 patients) and 60 cochleae with normal anatomy (n = 60 patients) were selected from patients with severe mixed or sensorineural hearing loss who presented to our clinic and underwent cochlear implantation (CI) between January 2010 and December 2022. Three neurotologists independently measured AID on Skull AP X-rays. Basal turn length of the cochlea was measured in axial and coronal oblique reformatted sections on HRCT images. Interrater reliability (ICC) of the AID measurements on Skull AP X-ray, determining the impact of cochlea size and electrode length on AID measurements. The interrater reliability (ICC) test showed a high level of consistency in measuring AID in the Skull AP X-ray (R = 0.906, p < 0.001). In the control group, a negative correlation was observed between the AID and the basal turn length of the cochlea, while a positive correlation was found between electrode length and AID (R = 0.947, p < 0.001). The Skull AP X-ray appears to be a dependable tool for measuring AID. In cases of IEMs, it is important to select an electrode of appropriate length, considering the dimensions of the cochlea.

Impact of age-related hearing loss on decompensation of left DLPFC during speech perception in noise: a combined EEG-fNIRS study.

Understanding speech-in-noise is a significant challenge for individuals with age-related hearing loss (ARHL). Evidence suggests that increased activity in the frontal cortex compensates for impaired speech perception in healthy aging older adults. However, whether older adults with ARHL still show preserved compensatory function and the specific neural regulatory mechanisms underlying such compensation remains largely unclear. Here, by utilizing a synchronized EEG-fNIRS test, we investigated the neural oscillatory characteristics of the theta band and synchronous hemodynamic changes in the frontal cortex during a speech recognition task in noise. The study included healthy older adults (n = 26, aged 65.4 ± 2.8), those with mild hearing loss (n = 26, aged 66.3 ± 3.8), and those with moderate to severe hearing loss (n = 26, aged 67.5 ± 3.7). Results showed that, relative to healthy older adults, older adults with ARHL exhibited lower activation and weakened theta band neural oscillations in the left dorsolateral prefrontal cortex (DLPFC) under noisy conditions, and this decreased activity correlated with high-frequency hearing loss. Meanwhile, we found that the connectivity of the frontoparietal network was significantly reduced, which might depress the top-down articulatory prediction function affecting speech recognition performance in ARHL older adults. The results suggested that healthy aging older adults might exhibit compensatory attentional resource recruitment through a top-down auditory-motor integration mechanism. In comparison, older adults with ARHL reflected decompensation of the left DLPFC involving the frontoparietal integration network during speech recognition tasks in noise.

Comparative analysis of classic and delayed Ménière's disease: clinical characteristics and endolymphatic hydrops manifestations in an Asian cohort.

To investigate the clinical characteristics and endolymphatic hydrops (ELH) Manifestations in patients with delayed Ménière's disease (DMD) compared to classic Ménière's disease (CMD). A prospective cohort study was conducted involving 523 patients diagnosed with MD. Clinical data, inner ear function, and ELH characteristics were analyzed and compared between CMD and DMD groups. Assessments included audiometry, VEMP, and MRI. DMD prevalence was 6.1%. Bilateral MD was significantly more common in DMD (34.4% vs. 14.5%, P = 0.003). DMD patients had more severe hearing loss in the first involved ear and more deteriorated VEMP in the contralateral ear (P < 0.05). ELH was present in 100% of affected ears, with a higher contralateral ELH proportion in DMD (34.4% vs. 15.0%, P = 0.005). DMD patients exhibit a higher prevalence of bilateral MD, more severe hearing loss, and greater contralateral ELH compared to CMD patients. Both subtypes share similar ELH characteristics, suggesting common underlying mechanisms.

Sensorineural hearing loss in post-COVID-19 patients

&lt;b&gt;Background: &lt;/b&gt;Hearing loss (HL) is one of the most common chronic conditions after hypertension and arthritis. Mounting indices suggest that sensorineural hearing loss (SNHL) may be one of the adverse effects of severe acute respiratory syndrome coronavirus 2.&lt;br /&gt; &lt;b&gt;Objective: &lt;/b&gt;This work aimed at studying SNHL in post-COVID-19 patients, alongside with exploring the relationship between severity of the disease and degree of hearing disability.&lt;br /&gt; &lt;b&gt;Materials and methods: &lt;/b&gt;This prospective cross-sectional study was conducted at Al-Azhar University Hospitals, Cairo, Egypt. It included 100 post-COVID-19 patients selected according to the study inclusion and exclusion criteria. Pure tone audiometry was done to confirm the presence of HL and to determine its degree.&lt;br /&gt; &lt;b&gt;Results: &lt;/b&gt;Hundred patients with recent oncent hearing symptoms after documented COVID-19 infection were included. Males (71%) were more affected than females (29%). The most of our cases had bilateral moderate HL (45%). Tinnitus was the most frequently associated otological symptom (96.96%). The time of HL onset varied from days to weeks (55% had time onset between 11-30 days post-confirmed infection). Higher thresholds were detected in all frequencies of affected ears with more deterioration of hearing as frequencies increase. All degrees of HL were detected; mild, moderate, severe and profound. There was no significant correlation between severity of COVID-19 infection and severity of HL.&lt;br /&gt; &lt;b&gt;Conclusions: &lt;/b&gt;SNHL is one of the extrapulmonary complications of COVID-19. COVID-19 patients may develop hearing affection regardless the severity of the infection. It is recommended to assess hearing functions in patients after COVID-19 for early detection and proper management.

A Non-Surgical Wearable Option for Bone Conduction Hearing Implants: A Comparative Study with Conventional Bone Conduction Hearing Aids Mounted on Eyeglasses.

This study aimed to compare the audiological benefits of a non-implantable wearable option for a bone conduction (BC) implant mounted on an arch (SoundArc) to those of traditional BC hearing aids (HAs) mounted on eyeglasses (BCHAs) in patients with moderate to severe conductive or mixed hearing loss. A preliminary cross-sectional observational prospective cohort study was conducted in the Tertiary Audiological Department, University Hospital. Fourteen adults with conductive or mixed hearing loss (PTA at 0.5-1-2-4 KHz = 67 ± 15 dB HL) who had been wearing conventional BCHAs mounted on eyeglasses for at least 3 years and had declined surgical implantation of a bone conduction hearing implant (BCHI) were included in the study. Unaided and aided pure-tone air conduction (AC) and bone conduction (BC) thresholds, as well as speech tests in quiet and noise, were recorded at baseline and in two different settings: with a BCHI mounted on SoundArc® and with their own BCHAs mounted on eyeglasses using two couplers. Participants completed questionnaires in both conditions, including the International Inventory for Hearing Aids (IOI-HA), the Hearing Handicap Inventory for Adults/Elderly (HHIA/E), the Speech, Spatial, and Qualities of Hearing Scale (SSQ), a 10-point visual analog scale (VAS), and the Fatigue Impact Scale (FIS). A significant functional gain was observed in both settings (p = 0.0001). Better speech perception in quiet and noise was observed with SoundArc compared to conventional BCHAs on eyeglasses (improvements in word repetition scores in noise: +19.3 at SNR +10 dB, p = 0.002; +12.1 at SNR 0 dB, p = 0.006; and +11.4 at SNR -10 dB, p = 0.002). No significant differences were found in IOI-HA, FIS, and HHIA/E scores. However, significantly better SSQ scores were reported for SoundArc in all domains (p = 0.0038). Although patients were accustomed to using BCHAs mounted on eyeglasses, the bone conduction wearable option of the BCHI (SoundArc) proved to be a viable alternative for adult patients with conductive or mixed hearing loss who are unable or unwilling to undergo BCHI surgery.

Slight and hidden hearing loss in young rats is associated with impaired recognition memory and reduced myelination in the corpus callosum.

Slight and hidden hearing loss in children have been linked to cognitive and social difficulties, and yet the neurobiological mechanisms behind these issues remain poorly understood. Most animal models focus on severe hearing loss, leaving the effects of hidden or slight hearing loss largely unexplored. To uncover the neural mechanisms connecting slight/hidden hearing loss to cognitive and social challenges, we induced hearing loss in young (4-week-old) Wistar rats through noise exposure. We then examined cognitive function (object recognition test) and social behavior (juvenile play behavior and social interaction). Changes in brain anatomy were assessed using cortical thickness and hippocampal size measurements, while (immuno)histochemical staining investigated neuronal circuitry maturation (myelin basic protein, parvalbumin, and perineuronal nets) and neurogenesis (doublecortin). Noise-exposed rats displayed slight high-frequency hearing loss (around 20 dB) and hidden hearing loss at other tested frequencies. This slight/hidden hearing loss was associated with impaired object recognition but did not alter social behavior. Slight/hidden hearing loss was associated with reduced myelin basic protein expression in the corpus callosum but no other alterations in cortical thickness, hippocampal size, or other markers of maturation and neurogenesis were found. These findings show that even slight/hidden hearing loss can lead to subtle brain alterations tied to cognitive deficits. This study emphasizes the need for further research to fully understand the brain changes associated with slight/hidden hearing loss and to pinpoint the mechanisms connecting these changes to behavioral deficits. This information is crucial to develop interventions to prevent the cognitive and social consequences of hearing loss. (PsycInfo Database Record (c) 2024 APA, all rights reserved).

Objective audiometric evaluation of individuals with type 2 diabetes mellitus: A cross-sectional survey in South West Nigeria

Aims: Diabetes mellitus affects more than 450 million people around the world. The relationship between diabetes and sensorineural hearing loss has been an area of interest. The aim of this study is to evaluate hearing loss, among type 2 diabetes diagnosed in the endocrinology unit of a private tertiary health facility in South West Nigeria. Methods: This was a hospital-based cross-sectional study carried out among type 2 diabetic patients and normal healthy non-diabetic individuals as control. A total of 120 participants were recruited for the test and control. Otoacoustic emission and auditory steady-state response audiometry were carried out on all the participants. Result: The total number of study participants was 120 (60 for test and control) with an age range of 30–79 years for the test and 20–80 years for the control. Otoacoustic emission showed passes for both ears, 61.7% pass in both ears for the test group and 76.7% pass in both ears for the control group. 21.7% of the test and control were referred for both ears. 16.6% of the test group was referred in one ear compared to 1.7% in the control group. Auditory steady-state response (ASSR) audiometry showed mild to profound hearing loss in all test groups while in the control group 20% had normal hearing. Moderate to moderately severe hearing loss in at least one ear was 38.4% in the test ear compared to 16.7% in the control group. Conclusion: Hearing loss is an established complication of type 2 diabetes. Therefore, it is necessary to incorporate regular hearing screening for patients with diabetes for early detection, counseling, and rehabilitation for those with disabling hearing loss.

Assessment of Cochlear Function Among Hypertensive Patients in Bauchi: A Cross-Sectional Cohort Study

Background: Hypertension is an important public-health problem, and it has been identified as the leading cause of morbidity and mortality due to the high incidence of end-organ damages. Unlike other complications of hypertension, cochlear dysfunction in hypertensive patients is a hidden and progressive damage that remains undetected (subclinical) for a long time. The aim of this study was to assess the prevalence of subclinical hearing impairment, as well as the type and degree of hearing loss among hypertensive patients in our environment. Methods: This was a cross-sectional cohort study conducted among adults diagnosed with hypertension previously. Ethical clearance and informed consent were obtained. Previous history of related exposure event (hypertension) and outcome (hearing loss) was assessed. Each participant was then subjected to c-PTA and EHA tests, and findings were analyzed. Results: One hundred and ninety (190) hypertensive patients were enrolled, among which 107 (56.3%) were females and 83 (43.7%) males. Hypertension was more common in the age group of 51-60 years (26.8%) and 61-70 years (30.5%). The overall prevalence of hearing loss was 41.6%, of which 18.4% had subclinical hearing impairment. The most common type of hearing loss was sensorineural 68 (86.1%), found to be in varying degrees of 33.8%, 26.5%, 23.5%, and 16.2% for mild, moderate, severe, and profound hearing loss, respectively. Conclusion: The overall prevalence of hearing loss among hypertensive patients was 41.6%, with a significant proportion (18.4%) having subclinical hearing loss, which is not noticed by the patients. Therefore, routine pure tone audiometric hearing screening for all hypertensives is recommended to enable early detection and treatment of this hearing loss.

Correlation between the etiology of severe hearing loss and endolymphatic hydrops.

This study aimed to investigate correlation between the presence of endolymphatic hydrops(EH) and factors such as causes of hearing loss, patient age, duration of deafness, and results of vestibular function tests. We retrospectively reviewed medical charts of 128 ears of cochlear implantees who were not considered relevant to Meniere's disease. When comparing group with genetic variants of GJB2, SLC26A4, LMX1A and other genetic mutation group, the proportion of vestibular EH and cochlear EH found in group with genetic variants of GJB2, SLC26A4, LMX1A was significantly higher than group with other genetic etiology (p < 0.01) or the group with all the other causes of hearing loss (p < 0.01). The rate of vestibular and cochlear EH detection was higher in younger patients (41.5% and 35.4%) than in older patients (25.4% and 20.6%). A higher ratio of vestibular and cochlear EH was observed in patients with a longer duration of deafness (37.5% and 31.3%) than those with a shorter duration of deafness (29.7% and 25.0%). The group with vestibular EH showed a higher incidence of abnormal findings in the caloric test (42.9%) than the group without vestibular EH (28.2%). Patients with genetic variants of GJB2, SLC26A4, LMX1A, younger patients, those with longer deaf durations showed a higher prevalence of vestibular and cochlear EH, implying EH appears to be formed as a developmental disorder in association with a certain set of genetic variants, rather than a phenotypic marker as a result of severe to profound hearing loss.

Impact of Usher syndrome on athletic performance - navigating silence and darkness

Usher syndrome (USH) is an autosomal recessive genetic disorder and a leading cause of simultaneous hearing and vision loss. The aim of this paper is to provide a detailed review of Usher syndrome, including its pathogenesis, clinical symptoms, diagnostic methods, the role of sport in the lives of athletes and sportspeople with Usher syndrome and available therapeutic options. Understanding this disease is crucial for early detection and the implementation of appropriate therapeutic interventions. Currently, Usher syndrome is classified into three main types (I, II, and III), differentiated by the degree and progression of hearing and vision loss. Type I is characterized by profound congenital deafness and early-onset retinitis pigmentosa. Type II presents with moderate to severe hearing loss and later onset of visual problems. Type III is the rarest, with progressive hearing and vision loss that occurs later in life. The genetic basis of Usher syndrome is linked to mutations in various genes that affect proteins essential for the proper functioning of the inner ear and retina. Early diagnosis and an interdisciplinary approach to treatment are key to improving the quality of life for those affected by this disease. Ongoing research into the genetic mechanisms and potential therapies offers hope for future successful treatments.

Unilateral Sensorineural Hearing Loss in Congenital Cytomegalovirus Retrospective Observational Study.

The leading nonhereditary cause of childhood sensorineural hearing loss has been attributed to congenital cytomegalovirus (cCMV). Sensorineural hearing loss can be unilateral (UHL) or bilateral (BHL), and may be progressive. Our objective was to describe the characteristics, clinical nature and follow-up of ears in cCMV-associated UHL. This 16-year retrospective study was performed at Schneider's Medical Center, Israel. Data were collected from all cCMV infants with UHL at birth who were treated with antiviral treatment initiated within the first 4 weeks of life and had a follow-up period of at least 1 year. We enrolled 67 infants diagnosed with UHL at birth: 17 (25%) with mild hearing loss, 22 (33%) with moderate hearing loss and 28 (42%) with severe hearing loss. At the last follow-up visit, 7 (41%) ears in the mild hearing loss group improved to normal hearing, 8 (47%) ears exhibited no change and 2 (12%) ears deteriorated. In the moderate hearing loss group, 9 (41%) ears improved, 7 (32%) remained static and 6 (27%) deteriorated to the severe hearing loss group. One (4%) ear in the severe hearing loss group showed improvement. Of the 67 ears with normal hearing at birth, 4 (6%) ears deteriorated. This study assessed and elucidated the characteristics, clinical nature and long-term follow-up of both the affected and unaffected ears diagnosed with UHL due to cCMV. These data are crucial when medical and/or surgical interventions are considered.

Letter to the editor on "The cochlear dose and the age at radiotherapy predict severe hearing loss after passive scattering proton therapy and cisplatin in children with medulloblastoma".

Letter to the editor on "The cochlear dose and the age at radiotherapy predict severe hearing loss after passive scattering proton therapy and cisplatin in children with medulloblastoma".

Prevalence of and Risk Factors for Hearing Impairment in Craniofacial Microsomia

BackgroundWhile ear anomalies and hearing impairment are common in patients with craniofacial microsomia (CFM), their prevalence, characteristics, and relationship to speech-language development remain unclear. PurposeThis study analyzed the prevalence and risk factors for hearing impairment in patients with CFM. Study Design, Setting, SampleThis retrospective cohort study included patients with unilateral or bilateral CFM from a single center between January 1980 and July 2023 who had evidence of a hearing assessment at <18 years. Exclusion criteria were inconclusive CFM diagnosis by clinical examination and/or radiographs or incomplete medical records. Predictor VariablesThe predictor variables included presence or absence of cleft lip and/or palate, laterality of CFM, and external ear and mandibular anomaly scores measured using the Orbit, Mandible, Ear, Nerve, and Soft Tissue (OMENS) and Pruzansky-Kaban classifications. Main Outcome VariablesPrimary outcome variable was hearing impairment, measured through air and bone conduction audiometry and categorized by type, severity, and side. Secondary outcome variable was speech-language delay, evaluated through assessments of expressive and receptive language skills, vocabulary, speech intelligibility, general articulation, and phonological speech. CovariatesThe covariates included sex and age at first hearing assessment. AnalysesLogistic regression models were used to analyze the effect of predictors on outcomes. P-value <0.05 was considered significant. ResultsThe sample included 213 patients (61.5% male). Hearing assessments were performed at a mean age of 4.6 ± 4.8 years. Hearing impairment was found in 183 (85.9%) and was predominantly conductive (n=130, 91.5%). Of subjects with hearing impairment, 158 (86.3%) had external ear anomalies (P<0.001). In patients with unilateral CFM, 38 (21.8%) had bilateral hearing loss and 9 (5.2%) had contralateral-only hearing loss. Both M3 and E3 scores were associated with the presence and severity of hearing loss (P=0.01 and P<0.001, respectively). There was no significant association between hearing impairment and speech-language development (P>0.05). Conclusion and RelevanceAlthough the severity of mandibular and external ear anomalies was significantly associated with the prevalence and severity of hearing loss, clinicians should remain alert for concomitant or isolated contralateral hearing loss, given the high prevalence of hearing loss in ears without ipsilateral facial involvement or external abnormalities.

Hearing Loss, Hearing Aids, and Cognition.

Most observational studies examining the association between hearing loss (HL) and cognitive impairment used subjective measures of hearing and addressed only one dimension of cognition, and very few investigated the potential benefit of hearing aids (HAs). To evaluate objectively measured HL and several dimensions of cognition and estimate the association with HA use. A cross-sectional analysis of the CONSTANCES cohort study, which recruited participants from January 1, 2012, to December 31, 2020, was conducted. Participants were a representative sample of adults (age, 45-69 years) with audiometric data and cognitive evaluation from 21 preventive health centers in France. Data analysis was conducted from April 1 to September 15, 2023. The main exposure was HL, which was defined by a pure-tone average in the best ear higher than 20 dB hearing level for mild loss and 35 dB hearing level for disabling loss. Secondary exposure was self-reported HA use. Cognition was evaluated at study inclusion by a standardized battery of 5 cognitive tests conducted by trained neuropsychologists. A global cognitive score was computed from principal component analysis and global cognitive impairment was defined as having a score less than or equal to the 25th percentile of the distribution. The study population included 62 072 participants with audiometric data (mean [SD] age, 57.4 [7] years; 52% women). Overall, 38% (n = 23 768) had mild HL, 10% (n = 6012) had disabling HL, and 3% (n = 1668) were HA users. In multivariable analyses, mild HL (odds ratio [OR], 1.10; 95% CI, 1.05-1.15) and disabling HL (OR, 1.24; 95% CI, 1.16-1.33) were associated with greater global cognitive impairment. The odds of cognitive impairment did not differ significantly between all participants with HA use and participants with disabling HL without HAs (OR, 0.94; 95% CI, 0.83-1.07), except among participants with depression (OR, 0.62; 95% CI, 0.44-0.88). In this cohort study, an association between the severity of HL and global cognitive impairment was found. The use of HAs was not associated with significantly lower odds of cognitive impairment. The findings suggest that it may be useful to monitor cognitive function in middle-aged individuals with HL.

Evaluation of angiotensin converting enzyme insertion/deletion, alpha adducin (ADD1) G460W, and IL-10 gene polymorphisms, and determination of prognostic effects in idiopathic sudden sensorineural hearing loss

ObjectiveThe aim of this study was to examine angiotensin converting enzyme (ACE) insertion/deletion, alpha adducin, and interleukin-10 (IL-10) gene polymorphisms (GPs) in terms of both idiopathic sudden sensorineural hearing loss (ISSNHL) risk and their potential prognostic effects. MethodsThe study group consisted of 70 patients and the control group consisted of 50 patients. Venous blood samples were analyzed for relevant GPs via kompetitive allele-specific polymerase chain reaction. Age, sex, affected side, tinnitus, and vertiginous symptom status, number of days between symptom onset and hospital admission, pure tone audiometry results at admission and after treatment were included in the study. Data were compared statistically. ResultsThe D allele of ACE insertion/deletion GP was significantly more frequent in patients with ISSNHL than in the control group (p = 0.032). II genotype was associated with a reduced risk of ISSNHL (p = 0.036). The amount of hearing loss was significantly higher in patients with the TT genotype (p = 0.027) and T allele of the IL-10 GP (p = 0.035) than in the patients without this allele. Severe hearing loss was a poor prognostic factor (p = 0.008). ConclusionsThe D allele of ACE insertion/deletion GP may be involved in the ISSNHL etiology. Due to the association of this allele with occlusive vascular pathologies, ischemia is believed to be a common pathway in the etiopathogenesis of ISSNHL.