Demographic analysis of hearing impairment based on various parameters in patients with cochlear implant.

Objectives: To analyse the demographic and clinical variables in children having undergone cochlear implant surgery because of deafness. METHODS: The cross-sectional study was conducted from January to November 2022 at the Centre for Research in Experimental and Applied Medicine laboratory of the Department of Biochemistry and Molecular Biology, Army Medical College, Rawalpindi, Pakistan, in collaboration with the Ear, Nose and Throat Department of Combined Military Hospital, Rawalpindi, and comprised children of eith gender aged up to 10 years who had received cochlear implant. Data was collected through questionnaire-based detailed interviews. Syndromic Hearing Loss, Non-Syndromic Hearing Loss, and Acquired Hearing Loss were identified among the subjects. Data was analysed using SPSS 22. RESULTS: Of the 250 cases, 147(58.8%) were boys, 146(58.4%) were aged 0-5 years, 219(87.6%) had prelingual onset of disease, and 202(80.8%) had a non-progressive disease course. In 203(81.2%) cases, normal developmental milestones were seen. Parental consanguinity was observed in 219(87.6%) cases. However, 63(25.2%) patients had a first-degree relative who had a history of deafness. In 170(68%) cases, hearing loss was hereditary, whereas in 80(32%) it was acquired. Meningitis was the most commonly identified risk factor 55(68.75%). Acquired risk factors and family history had significant association with hearing loss (p<0.05). Speech perception significantly improved in all 219(100%) patients with prelingual hearing loss who underwent cochlear implantation. CONCLUSIONS: Majority of the cases were found to be male, had a prelingual disease onset and a non-progressive disease course. Family history was a significant factor, while meningitis was the most common acquired cause of hearing loss.

Increased risk of psychiatric disorder in patients with hearing loss: a nationwide population-based cohort study.

BACKGROUND: Hearing loss has been shown to be a risk factor for psychiatric disorders. In addition, long-term hearing loss is associated with increased hospitalization and mortality rates; however, the increased risk and duration of effect of hearing loss in combination with other chronic diseases on each psychiatric disorder are still not clearly defined. The purpose of this article is to clarify the risk of hearing loss for each disorder over time. METHODS: This was a retrospective cohort study, and a national health insurance research database in Taiwan was utilized. All (n = 1,949,101) Taiwanese residents who had a medical visit between 2000 and 2015 were included. Patients with hearing loss and a comparative retrospective cohort were analyzed. Every subject was tracked individually from their index date to identify the subjects who later received a diagnosis of a psychiatric disorder. The Kaplan‒Meier method was used to analyze the cumulative incidence of psychiatric disorders. Cox regression analysis was performed to identify the risk of psychiatric disorders. RESULTS: A total of 13,341 (15.42%) and 31,250 (9.03%) patients with and without hearing loss, respectively, were diagnosed with psychiatric disorders (P < 0.001). Multivariate analysis indicated that hearing loss significantly elevated the risk of psychiatric disorders (adjusted HR = 2.587, 95% CI 1.723-3.346, p < 0.001). CONCLUSION: Our findings indicate that patients with hearing loss are more likely to develop psychiatric disorders. Furthermore, the various psychiatric disorders are more likely to occur at different times. Our findings have important clinical implications, including a need for clinicians to implement early intervention for hearing loss and to pay close attention to patients' psychological status. Trial registration TSGHIRB No. E202216036.

Chronological change of left ventricular global longitudinal strain in patients with maternally inherited diabetes and deafness: A case series.

RATIONALE: Maternally inherited diabetes and deafness (MIDD) is a rare genetic disorder arising from mitochondrial DNA mutations, characterized by a combination of diabetes mellitus and sensorineural deafness. It is known that MIDD patients with cardiomyopathy have a poor prognosis, but there are no established guidelines for the diagnosis and follow-up of cardiomyopathy in MIDD patients. PATIENT CONCERNS: Patient 1 was a 48-year-old woman who visited the hospital with cardiomegaly and had been taking oral hypoglycemic agents for 8 years. Patient 2 was a 21-year-old man, the son of patient 1, who visited the hospital for genetic screening. Patient 2 was also diagnosed diabetes mellitus 2 years ago. DIAGNOSIS: Patient 1 was found to have restrictive cardiomyopathy on echocardiography and underwent endomyocardial biopsy and genetic testing to determine the etiology. The m.3243A>G mutation was confirmed and she was diagnosed with MIDD accompanied with diabetes and hearing loss. Additionally, patient 2 had m.3243 A>G mutation and was diagnosed with MIDD due to diabetes and hearing loss. INTERVENTIONS: Because MIDD does not have a specific treatment, patient 1 took ubidecarenone (coenzyme Q10), acetylcarnitine, and multivitamin along with the treatment for diabetes control and heart failure. Patient 2 was taking ubidecarenone (coenzyme Q10), acetylcarnitine, and multivitamin along with treatment for diabetes. OUTCOMES: She subsequently underwent routine transthoracic echocardiography, and a progressive decline in global longitudinal strain (GLS) was first observed, followed by a worsening of the patient's clinical situation. Patient 2 had concentric remodeling and decreased GLS. On periodic echocardiography, GLS decreased at a very slow rate, and the patient's clinical course was stable. LESSONS: The findings of this report contribute to the understanding of the clinical course of MIDD-associated cardiomyopathy and highlight the potential of GLS as a sensitive marker for disease progression.

Impact of hearing impairment on the mental status of the adults and older adults in Jordanian society.

BACKGROUND: Hearing loss is a common disorder, affecting both children and adults worldwide. Individuals with hearing loss suffer from mental health problems that affect their quality of life. OBJECTIVE: This study aimed to investigate the social and emotional consequences of hearing loss in a Jordanian population using Arabic versions of the Hearing Handicap Inventory for Adults (HHIA) and the Hearing Handicap Inventory for the Elderly (HHIE). METHODS: This study included 300 Jordanian participants aged 18-90 years with hearing loss. Each participant underwent a complete audiological evaluation before answering the questionnaires. RESULTS: The median overall scores of the HHIA and HHIE groups were 39 and 65, respectively. Both HHIA (Cronbach's alpha = 0.79, p < 0.001) and HHIE (Cronbach's alpha = 0.78, p < 0.001) were significantly associated with the social, emotional, and overall scores. Compared to the adult group, the median emotional and social scores of the older adults group were significantly higher than the adults group (Z = -4.721, p = 0.001), using the Mann-Whitney test. CONCLUSION: The present research revealed that psychological disabilities associated with hearing loss in the adult Jordanian population are more frequent and severe than in other nations. This may be attributed to the lack of awareness of the mental consequences of hearing loss among Jordanian healthcare providers and the public.

Association between sarcopenia and hearing impairment in middle-aged and elderly people in China: a prospective cohort study.

This study used longitudinal data from CHARLS 2011-2018 for cross-sectional and longitudinal analyses to investigate the relationship between sarcopenia and hearing impairment in middle-aged and elderly adults in China. The study selected 9723 participants aged 45 years and older from CHARLS 2011 and followed up in 2015 and 2018. Binary logistic regression and cox proportional risk regression models were used for testing. The results of the study showed that in the cross-sectional analysis, probable sarcopenia was significantly associated with hearing impairment compared with the group without sarcopenia [OR (95% CI) 0.342 (1.187, 1.669), p < 0.001], but sarcopenia was not significantly associated with hearing impairment. In the longitudinal analysis, middle-aged and elderly adults with sarcopenia [HR (95% CI) 0.354 (1.043, 1.945), p < 0.01] were more likely to have hearing impairment than those with probable sarcopenia and without sarcopenia. Probable sarcopenia was strongly associated with hearing impairment in middle-aged and elderly adults, whereas sarcopenia was a strong predictor of hearing impairment over the next 7 years. The results of this study emphasize the urgent need for measures to address sarcopenia in order to prevent and delay the decline in hearing function.

Impact Of Sensorineural Hearing Loss On Subjective Tinnitus Quality In Patients With Bilateral Tinnitus.

INTRODUCTION: Tinnitus is a frequent condition that indicates the sensation of sound in the absence of a corresponding external stimulus and can significantly impair the quality of life. The main risk factor for developing tinnitus is hearing loss. The diagnosis of tinnitus is based on history, assessment of tinnitus severity, clinical examination, and audiological tests. The main purpose of this research was to examine the relationship between the presence and level of hearing loss and the characteristics of tinnitus in patients with bilateral subjective tinnitus. METHODS: Total number of 50 participants, 20 men, and 30 women were included in the research. Demographic data, data on hearing impairment obtained by tone audiometry, and data on difficulties caused by tinnitus obtained in two questionnaires - Tinnitus Handicap Inventory (THI) and Tinnitus Functional Index (TFI) were used. RESULTS: Age above 30 years is significantly associated with tinnitus with hearing loss. Hearing impairment is also significantly more often associated with an auditory TFI index >6.7, a total THI index >20, and an emotional THI index >3. Hearing loss was noted in 76% of patients. CONCLUSION: Tinnitus represents a significant burden for patients, therefore it is important to assess the impact of tinnitus on daily activities and quality of life.

Hearing Loss Among Families with 2 and More Affected Members in Golestan Province, Iran: A Cross-Sectional Study of 320 Families.

BACKGROUND: Our study centers on various aspects of families who have 2 or more members with hearing loss (HL) and are living in Golestan province in Iran. We aimed to identify those families with the highest probability of hereditary HL and also to examine the impact of consanguinity among them. METHODS: The families included in the study underwent a comprehensive screening process that involved their prenatal and postnatal histories as well as family medical histories. Additionally, each patient received a thorough clinical ear examination. The evaluation also took into account factors such as patterns of inheritance, consanguinity, a 3-generation pedigree, and physical examination. Following this initial assessment, patients were referred for a complete hearing evaluation, which included pure-tone audiometry, speech recognition threshold, otoacoustic emission, and auditory brainstem response tests. RESULTS: We identified a total of 8553 individuals living in Golestan province who are hearing impaired. Among those, our records indicate that 320 families had at least 2 affected members. The rate of consanguinity marriage in non-syndromic families was 64.43%. Also, a significant number (88.12%, or n=282) of the families exhibited hereditary HL, among which a substantial proportion (89.72%, or n=253) presented with nonsyndromic forms of HL. Furthermore, bilateral, stable, and prelingual HL were the most frequently observed types, and a majority of the patients were diagnosed with sensorineural and profound HL. CONCLUSION: This study revealed a correlation between consanguinity and the incidence of familial HL, with more probability of bilateral, prelingual, sensorineural, and profound forms.

Impact of Concurrent Visual and Hearing Impairment on Incident Alzheimer's Disease: The LIFE Study.

Background: The prevalence of Alzheimer's disease (AD) is increasing in Japan due to population aging. The association between sensory impairment and incident AD remains unclear. Objective: This study aimed to investigate the impact of sensory impairment on incident AD. Methods: We analyzed residents of five municipalities participating in the Longevity Improvement & Fair Evidence (LIFE) Study. The participants comprised individuals who had newly applied for long-term care needs certification between 2017 and 2022 and had no cognitive impairment upon application or AD diagnosis within the preceding six months. Participants were classified according to sensory impairment status: visual impairment (VI), hearing impairment (HI), neither sensory impairment (NSI), and dual sensory impairment (DSI). The month succeeding the certification application was set as the index month, and the interval from that month until AD onset was assessed. Multivariable Cox proportional hazards analysis was performed to calculate the risk of AD onset according to sensory impairment status while adjusting for sex, age, dependence level, self-reliance level, and comorbidities. Results: Among 14,186 participants, we identified 1,194 (8.4%) who developed AD over a median follow-up period of 22.6 months. VI and HI only were not associated with incident AD. However, DSI conferred a significantly higher risk (HR: 1.6, CI: 1.1-2.2, p = 0.008) of AD onset than NSI. Conclusions: Individuals with concurrent DSI have a higher risk of developing AD than those with single or NSI. Preventing and treating sensory impairment may not only improve functional outcomes, but could also help to reduce the future risk of AD.

Hearing Loss and Dementia: Where to From Here?

Victorian era psychologists were the first to comment on associations between sensory and cognitive function. More recently, hearing loss has been shown as a marker of risk for dementia. However, it is not known whether this association represents a causal impact of hearing loss, nor whether treating hearing loss may help prevent dementia. Most studies on relationships between hearing loss and cognitive outcomes are observational, are at risk of confounding, and cannot reach conclusions about causation. A recent high quality randomized controlled trial, relatively uncommon in audiology, reported no impact of a comprehensive hearing intervention in mitigating cognitive decline in older adults. Although secondary analysis revealed potential benefits in a sub-sample of adults, this finding may be spurious. Encouraging policymakers, patients, and other health care practitioners to address hearing loss in terms of dementia prevention may be inappropriate on the grounds of both relevance at individual level and lack of clear evidence of benefit. In addition, advocating need to address hearing loss in terms of mitigating dementia risk may reduce the importance of addressing hearing loss in its own right. Linking hearing loss to dementia risk may also exacerbate the stigma of hearing loss, inadvertently discouraging people from seeking help for hearing. We suggest that treating hearing loss may have important benefits in preventing or delaying diagnosis of dementia via improving orientation and functioning in daily life, without changing the underlying pathology. Rather than linking hearing loss to dementia risk, we suggest a positive message focusing on the known benefits of addressing hearing loss in terms of improved communication, quality of life, and healthy aging.

Analysis of characteristics of and risk factors for otological symptoms after COVID-19 infection.

The purpose of this study was to explore the characteristics of and risk factors for otological symptoms after contracting COVID-19. We invited 468 participants who had been infected with COVID-19 to participate in a survey. 310 (66.2%) were women and 158 (33.8%) were men. The mean age is 38.73 (12.21) years. The questionnaire included their basic information, symptoms and symptom duration after SARS-CoV-2 infection, number of vaccine doses received, and details regarding otological symptoms. In total, 106/468 (22.6%) participants experienced tinnitus, 66/468 (14.1%) hearing loss, 103/468 (22.0%) aural fullness, and 71/468 (15.2%) dizziness. Women were more prone to experience tinnitus (P = 0.022) and dizziness (P = 0.001) than men. The group with hearing loss were older (P = 0.025), and their initial COVID-19 symptoms lasted longer (P = 0.028) than those of patients without. Patients with aural fullness were more likely to experience fatigue than patients without (P = 0.002). Patients experiencing dizziness were more likely to experience pharyngalgia (P = 0.040) and fatigue (P = 0.005) than those without. The number of vaccine doses was positively associated with the resolution of otological symptoms (P = 0.035). Multiple logistic regression analysis revealed that sex was an independent risk factor for tinnitus (odds ratio [OR], 1.802; 95% confidence interval [CI], 1.099-2.953; P = 0.020), the duration of initial COVID-19 symptoms for hearing loss (OR, 1.055; 95% CI, 1.008-1.105; P = 0.023), and sex for dizziness (OR, 2.870; 95% CI, 1.489-5.535; P = 0.002). Sex, age, COVID-19-related fatigue, and the duration of initial COVID-19 symptoms may affect the occurrence of otological symptoms, and vaccines may aid their resolution.

Distinct changes in brain metabolism in patients with dementia and hearing loss.

INTRODUCTION: Previous studies have reported that hearing loss (HL) is associated with dementia, although the mechanistic underpinnings remain elusive. This study aimed to evaluate the changes in brain metabolism in patients with HL and different types of dementia. METHODS: Patients with cognitive impairment (CI) and HL treated at the university-based memory clinic from May 2016 to October 2021 were included. In total, 108 patients with CI and HL prospectively underwent audiometry, neuropsychological test, magnetic resonance imaging, and 18 F-fluorodeoxyglucose positron emission tomography. Twenty-seven individuals without cognitive impairment and hearing loss were enrolled as a control group. Multivariable regression was performed to evaluate brain regions correlated with each pathology type after adjusting for confounding factors. RESULTS: Multivariable regression analyses revealed that Alzheimer's disease-related CI (ADCI) was associated with hypometabolic changes in the right superior temporal gyrus (STG), right middle temporal gyrus (MTG), and bilateral medial temporal lobe. Lewy body disease-related CI (LBDCI) and vascular CI were associated with hypermetabolic and hypometabolic changes in the ascending auditory pathway, respectively. In the pure ADCI group, the degree of HL was positively associated with abnormal increase of brain metabolism in the right MTG, whereas it was negatively associated with decreased brain metabolism in the right STG in the pure LBDCI group. CONCLUSION: Each dementia type is associated with distinct changes in brain metabolism in patients with HL.

Cochlear Otosclerosis and Secondary Hydrops (Ménière's Syndrome).

ABSTRACT: This article discusses a case of cochlear otosclerosis leading to secondary hydrops and near-complete hearing loss. Histopathological examination revealed advanced multifocal otosclerosis in both temporal bones, with specific focus on cochlear invasion and significant bone resorption. The severity of the case ruled out surgical intervention due to the risk of further hearing loss. The article emphasizes the challenges in managing otosclerosis-related hydrops and highlights the potential use of advanced imaging techniques for diagnosis. The study underscores the complexity of otosclerosis-induced hearing loss, contributing to the understanding of this pathology and its impact on auditory function.

Granulomatosis with polyangiitis presenting headache: A case report and review of literature.

RATIONALE: Central nervous system involvement is a rare manifestation of active-phaselocalized Granulomatosis with polyangiitis (GPA). In hypertrophic dura meningitis, GPA with headache is typical. In this case, cerebral magnetic resonance (MR) enhancement revealed no meningeal thickening, to our knowledge, this manifestation had not been found previously. PATIENT CONCERNS: The patient presented to the Rheumatology and Immunology Clinic with severe headache and hearing loss, and central nervous system granulomatosis with polyangiitis was confirmed after a series of examinations. The patient had no significant effect after treatment with cyclophosphamide (CTX), but after the use of rituximab, the headache and hearing loss were significantly improved, and laboratory indicators returned to normal levels. DIAGNOSIS: We comprehensively screened for craniocerebral infection and malignant tumors, diagnosed central nervous system granulomatosis with polyangiitis. INTERVENTIONS: We gave sequential treatment of rituximab. OUTCOMES: All indicators are mostly back to normal when the patient was monitored at the outpatient clinic. LESSONS: GPA and severe headache are more prevalent in hypertrophic dura meningitis, but the patient early headache could not be explained by hypertrophic dura meningitis or localized granulomatous lesions that invaded the central nervous system. Patients with severe headaches likely have vascular inflammation and local bone destruction at the base of the skull.

Hearing Loss, Hearing Aid Use, and Risk of Dementia in Older Adults.

Importance: Hearing loss has been suggested as a risk factor for dementia, but there is still a need for high-quality research to better understand the association between these 2 conditions and the underlying causal mechanisms and treatment benefits using larger cohorts and detailed data. Objective: To investigate the association between hearing loss and incident dementia, as well as how hearing aid use contributes to this association. Design, Setting, and Participants: This population-based cohort study was conducted in Southern Denmark between January 2003 and December 2017 and included all residents 50 years and older. We excluded all persons with dementia before baseline as well as those who did not live in the region 5 years before baseline, with incomplete address history, or who had missing covariate information. Exposures: Individual hearing status based on the Hearing Examinations in Southern Denmark database, which contains data on all pure-tone audiometry examinations performed at public hearing rehabilitation clinics in Southern Denmark. Main Outcomes and Measures: Incident cases of dementia and Alzheimer disease as identified from national registries. Results: The study population comprised 573 088 persons (298 006 women [52%]; mean [SD] age, 60.8 [11.3] years) with 23 023 cases of dementia and mean (SD) follow-up of 8.6 (4.3) years. Having a hearing loss was associated with an increased risk of dementia, with an adjusted hazard ratio (HR) of 1.07 (95% CI, 1.04-1.11) compared with having no hearing loss. Severe hearing loss in the better and worse ear was associated with a higher dementia risk, with an HR of 1.20 (95% CI, 1.09-1.32) and 1.13 (95% CI, 1.06-1.20), respectively, compared with having no hearing loss in the corresponding ear. Compared with people without hearing loss, the risk of dementia was higher among people with hearing loss who were not using hearing aids than those who had hearing loss and were using hearing aids, with HRs of 1.20 (95% CI, 1.13-1.27) and 1.06 (95% CI, 1.01-1.10), respectively. Conclusions and Relevance: The results of this cohort study suggest that hearing loss was associated with increased dementia risk, especially among people not using hearing aids, suggesting that hearing aids might prevent or delay the onset and progression of dementia. The risk estimates were lower than in previous studies, highlighting the need for more high-quality longitudinal studies.

Scuba Diving-Induced Inner-Ear Pathology: Imaging Findings of Superior Semicircular Canal and Tegmen Tympani Dehiscence.

BACKGROUND Superior semicircular canal dehiscence is an inner-ear pathology which presents with vertigo, disequilibrium, and hearing loss. Although the exact etiology of superior semicircular canal dehiscence is unknown, it is thought that an increase in middle-ear pressure disrupts a thin overlying temporal bone. Superior semicircular canal dehiscence is frequently seen in association with dehiscence of the tegmen tympani, which overlies the middle ear. Here, we present a case report of a 52-year-old Puerto Rican man with vertigo, dizziness, vomiting, and mild hearing loss associated with superior semicircular canal and tegmen tympani dehiscence after performing improper scuba diving techniques. CASE REPORT A 52-year-old Puerto Rican man presented to the emergency department with vertigo, dizziness, vomiting, and mild hearing loss in the right ear. The symptoms began shortly after scuba diving with inadequate decompression techniques on ascent. He was treated with recompression therapy with mild but incomplete improvement in symptoms. Bilateral temporal magnetic resonance imaging was suggestive of segmental dehiscence of the right superior semicircular canal and tegmen tympani. High-resolution computed tomography of the temporal bone confirmed right superior semicircular canal and tegmen tympani dehiscence with an intact left inner ear. CONCLUSIONS The increased inner-ear pressure that occurs during scuba diving can lead to dehiscence of the superior semicircular canal and tegmen tympani, causing vertigo and hearing loss. Performance of improper diving techniques can further increase the risk of dehiscence. Therefore, appropriate radiologic evaluation of the inner ear should be performed in such patients.

Protocol for a comparative cross-sectional study on characterisation of auditory impairment in sickle cell disease and sickle cell trait and its impact on health-related quality of life in Nigeria.

INTRODUCTION: Sickle cell disease (SCD) and sickle cell traits (SCT) are genetically inherited red blood cell disorders common among people of African descent. Nigeria has a high prevalence of SCD, with a prevalence of 2.28%-3% and SCT, 25%-30%. Poorly managed SCD and SCT can lead to sensorineural hearing loss and health-related quality of life (HRQoL) issues. This research aims to assess these possible complications of SCD and SCT in Nigeria. METHODS AND ANALYSIS: The study will use a comparative cross-sectional design at study power 80% to investigate the association between SCD/SCT, hearing impairment and HRQoL. Participants will be divided into two groups: a cohort and a control group. Hearing levels will be assessed through audiometric assessments and categorised by type and severity of hearing impairments using WHO classifications. HRQoL will also be assessed using WHO Disability Assessment Schedule 2.0. Statistical analyses will be performed using the SAS V.9.4, with parametric or non-parametric analysis depending on the distribution. Relationship between key variables will be determined via correlational tests, χ2, Fisher's exact test and multivariable logistic regression analyses. ETHICS AND DISSEMINATION: The proposal has been fully reviewed and registered by the University of Cape Town's Faculty of Health Sciences Human Research Ethics Committee (HREC REF 228/2022) and the University of Abuja Teaching Hospital Human Research Ethics Committee (HREC/PR/2020/08/007). Information dissemination will be through conferences, peer-review publication and personal communications. The Strengthening the Reporting of Observational Studies in Epidemiology statement will be followed in writing the manuscript.

Estimating the impact of different types hearing loss on cognitive decline and the joint effect of hearing loss and depression on cognitive decline among older adults in China.

BACKGROUND: Current research lacks examination of the relationship between different subtypes of hearing loss (HL) and cognitive decline (CD). Additionally, the co-effects of HL and depression on CD remain unexplored. This study aims to investigate the relationship between HL, various types of HL, and CD, as well as the combined impact of HL and depression on CD. METHODS: Data from a total of 5218 older adults who participated in the most recent three waves of Chinese Longitudinal Healthy Longevity Survey (CLHLS) (2011-2012, 2014, and 2018) were included. HL was assessed through self-report and objective measures. CD was defined as a decrease in MMSE score of≥3 between any two survey periods for older adults. Cox proportional hazards model was applied to analyzed. RESULTS: Among Chinese older adults, bilateral HL (HR = 1.202, 95%CI = 1.093-1.322, P < 0.001), onset of HL after the age of 40 (HR = 1.155, 95%CI = 1.056-1.264, P = 0.002), and chronic HL (HR = 1.143, 95%CI = 1.040-1.255, P = 0.005) posed a greater risk. HL (HR = 1.146, 95%CI = 1.048-1.254, P = 0.003) and depression (HR = 1.162, 95%CI = 1.038-1.301, P = 0.009) were independently or jointly associated with CD. Participants who were simultaneously exposed to both HL and depression experienced the highest risk of CD (HR = 1.314, 95%CI = 1.117-1.545, P = 0.001). LIMITATIONS: Given the observational design, unidentified confounding variables may still be present, such as whether to wear a hearing aid. CONCLUSION: This study emphasizes the high risk of specific types of HL for CD and the importance of implementing health interventions that address both physiological and psychological aspects to enhance cognitive function and prevent CD in older adults.

Disease Manifestations and Complications in Dutch X-Linked Hypophosphatemia Patients.

X-linked hypophosphatemia (XLH) is the most common monogenetic cause of chronic hypophosphatemia, characterized by rickets and osteomalacia. Disease manifestations and treatment of XLH patients in the Netherlands are currently unknown. Characteristics of XLH patients participating in the Dutch observational registry for genetic hypophosphatemia and acquired renal phosphate wasting were analyzed. Eighty XLH patients, including 29 children, were included. Genetic testing, performed in 78.8% of patients, showed a PHEX mutation in 96.8%. Median (range) Z-score for height was - 2.5 (- 5.5; 1.0) in adults and - 1.4 (- 3.7; 1.0) in children. Many patients were overweight or obese: 64.3% of adults and 37.0% of children. All children received XLH-related medication e.g., active vitamin D, phosphate supplementation or burosumab, while 8 adults used no medication. Lower age at start of XLH-related treatment was associated with higher height at inclusion. Hearing loss was reported in 6.9% of children and 31.4% of adults. Knee deformities were observed in 75.0% of all patients and osteoarthritis in 51.0% of adult patients. Nephrocalcinosis was observed in 62.1% of children and 33.3% of adults. Earlier start of XLH-related treatment was associated with higher risk of nephrocalcinosis and detection at younger age. Hyperparathyroidism longer than six months was reported in 37.9% of children and 35.3% of adults. This nationwide study confirms the high prevalence of adiposity, hearing loss, bone deformities, osteoarthritis, nephrocalcinosis and hyperparathyroidism in Dutch XLH patients. Early start of XLH-related treatment appears to be beneficial for longitudinal growth but may increase development of nephrocalcinosis.

Hearing Impairment and Severe Attention Deficit/Hyperactivity Disorder: A Nationwide Study.

BACKGROUND: The association between hearing impairment and attention-deficit/hyperactivity disorder (ADHD) is unclear. Therefore, we aimed to assess this association in Israel's national sample of over 1.1 million adolescents. METHODS: We conducted a nationwide, population-based, cross-sectional study of all Israeli adolescents (n = 1,175,534, 58% males; mean age, 17 yrs) who were examined before mandatory military service during 2004 to 2020. Board-certified specialists confirmed diagnoses of hearing impairment and severe ADHD. MAIN OUTCOMES AND MEASURES: We compared the prevalence of severe ADHD in adolescents with and without hearing impairment. Associations were analyzed using logistic regression models and sensitivity analyses accounting for hearing impairment type (sensorineural vs. conductive) and severity. RESULTS: Of the 8,769 adolescents with hearing impairment, 57 were diagnosed with severe ADHD (prevalence = 0.65%). Of the 1,166,765 adolescents without hearing impairment, 3,936 were diagnosed with severe ADHD (prevalence = 0.29%). We found a significant association between hearing impairment and severe ADHD (odds ratio = 1.93 [95% confidence interval, 1.47-2.49]), which persisted in a multivariable model adjusted to age, sex, socioeconomic status, educational status, cognitive performance, and immigration status (odds ratio = 1.70 [95% confidence interval, 1.29-2.20]). The association also persisted when stratified by hearing impairment type (sensorineural vs. conductive) and severity. CONCLUSIONS: Adolescents with hearing impairment had 70% increased odds of severe ADHD. Study findings suggest that active screening of patients with hearing impairment for ADHD should be considered.

Cognitive change in prevalent and incident hearing loss: The Maastricht Aging Study.

INTRODUCTION: Hearing loss (HL) has been associated with cognitive decline and dementia. We examined the temporal association between prevalent and incident HL and cognitive change. METHODS: A total of 1823 participants (24-82 years) from the Maastricht Aging Study (MAAS) were assessed at baseline, 6 and 12 years, including pure-tone audiometry. Linear-mixed models were used to test the association between HL and cognition, adjusted for demographics and other dementia risk factors. RESULTS: Participants with prevalent and incident HL showed a faster decline in verbal memory, information processing speed, and executive function than participants without HL. Decline was steady from baseline to 6 and 12 years for prevalent HL, but time-delayed from 6 to 12 years for incident HL. Having a hearing aid did not change associations. DISCUSSION: Findings support the notion that HL is a risk factor for cognitive decline independent of other dementia risk factors. Onset of HL preceded onset of cognitive decline. HIGHLIGHTS: We examined cognitive change in prevalent and incident hearing loss. Prevalent and incident hearing loss were associated with faster cognitive decline. For prevalent hearing loss, decline was steady from baseline to 6 and 12 years. Onset of hearing loss preceded the onset of cognitive decline. Having a hearing aid did not change the observed associations.