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Purpose: To review possible risk factors for permanent delayed-onset, progressive sensorineural hearing loss (SNHL) in the paediatric population to recommend follow-up protocols for early detection. Methods: PRISMA-compliant systematic review was performed, including observational studies on the paediatric population up to 16 years old who have passed the newborn hearing screening programme (NHSP), investigating the development of late-onset, progressive SNHL. Electronic searches were performed through Medline, Embase, Cochrane, and Emcare. Results: 37 studies were included. 21 showed an association between late-onset SNHL and congenital cytomegalovirus (cCMV) infection (age at hearing loss diagnosis 0.75 to 204 months, mean 45.6 ± 43.9), while 16 between late-onset SNHL and other congenital or perinatal factors, namely Neonatal Intensive Care Unit (NICU) stay, prematurity, neonatal respiratory failure, mechanical ventilation, extracorporeal membrane oxygenation (ECMO) support, hypocapnia, hypoxia, alkalosis, seizure activity, congenital diaphragmatic hernia (CDH), inner ear malformation, and gene mutations (age at hearing loss diagnosis 2.5 to 156 months, mean 38.7 ± 40.7). Conclusions: cCMV infection may cause late-onset SNHL, which can be missed on standard NHSP. There is, therefore, evidence to support universal screening programmes to enable detection in even asymptomatic neonates. Ongoing audiological follow-up for all children with cCMV is advisable, to enable timely treatment. In the paediatric population presenting conditions such as NICU stay > 5 days, prematurity ≤ 34 weeks gestation, severe neonatal respiratory failure, mechanical ventilation, ECMO support, and CDH surgery, an audiological follow-up from 3 months of age up to at least 3–4 years of age, and at least annually, should be recommended. [ABSTRACT FROM AUTHOR]

NF2-related schwannomatosis (NF2) is a rare autosomal-dominant genetic disorder characterized by bilateral vestibular schwannomas and multiple meningiomas. This case report presents the extremely rare occurrence of an anaplastic meningioma in a 12-year-old male with previously undiagnosed NF2. The patient presented with a history of abdominal pain and episodic emesis, gait unsteadiness, right upper and lower extremity weakness, and facial weakness. He had sensorineural hearing loss and wore bilateral hearing aids. MR imaging revealed a sizable left frontoparietal, dural-based meningioma with heterogeneous enhancement with mass effect on the brain and midline shift. Multiple additional CNS lesions were noted including a homogenous lesion at the level of T5 indicative of compression of the spinal cord. The patient underwent a frontotemporoparietal craniotomy for the removal of his large dural-based meningioma, utilizing neuronavigation and transdural ultrasonography for precise en bloc resection of the mass. Histopathology revealed an anaplastic meningioma, WHO grade 3, characterized by brisk mitotic activity, small-cell changes, high Ki-67 proliferation rate, and significant loss of P16. We report an anaplastic meningioma associated with an underlying diagnosis of NF2 for which we describe clinical and histopathological features. [ABSTRACT FROM AUTHOR]

Background: Mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) is a maternally inherited mitochondrial disease that affects various systems in the body, particularly the brain, nervous system, and muscles. Among these systems, sensorineural hearing loss is a common additional symptom. Methods: A 42-year-old female patient with MELAS who experienced bilateral profound deafness and underwent bilateral sequential cochlear implantation (CIs). Speech recognition and subjective outcomes were evaluated. Results: Following the first CI follow-up, the patient exhibited improved speech recognition ability and decided to undergo the implantation of the second ear just two months after the initial CI surgery. The second CI also demonstrated enhanced speech recognition ability. Subjective outcomes were satisfactory for bilateral CIs. Conclusions: MELAS patients receiving bilateral CIs can attain satisfactory post-CI speech recognition, spatial hearing, and sound qualities. [ABSTRACT FROM AUTHOR]

Purpose: Patient-to-image registration is a preliminary step required in surgical navigation based on preoperative images. Human intervention and fiducial markers hamper this task as they are time-consuming and introduce potential errors. We aimed to develop a fully automatic 2D registration system for augmented reality in ear surgery. Methods: CT-scans and corresponding oto-endoscopic videos were collected from 41 patients (58 ears) undergoing ear examination (vestibular schwannoma before surgery, profound hearing loss requiring cochlear implant, suspicion of perilymphatic fistula, contralateral ears in cases of unilateral chronic otitis media). Two to four images were selected from each case. For the training phase, data from patients (75% of the dataset) and 11 cadaveric specimens were used. Tympanic membranes and malleus handles were contoured on both video images and CT-scans by expert surgeons. The algorithm used a U-Net network for detecting the contours of the tympanic membrane and the malleus on both preoperative CT-scans and endoscopic video frames. Then, contours were processed and registered through an iterative closest point algorithm. Validation was performed on 4 cases and testing on 6 cases. Registration error was measured by overlaying both images and measuring the average and Hausdorff distances. Results: The proposed registration method yielded a precision compatible with ear surgery with a 2D mean overlay error of 0.65 ± 0.60 mm for the incus and 0.48 ± 0.32 mm for the round window. The average Hausdorff distance for these 2 targets was 0.98 ± 0.60 mm and 0.78 ± 0.34 mm respectively. An outlier case with higher errors (2.3 mm and 1.5 mm average Hausdorff distance for incus and round window respectively) was observed in relation to a high discrepancy between the projection angle of the reconstructed CT-scan and the video image. The maximum duration for the overall process was 18 s. Conclusions: A fully automatic 2D registration method based on a convolutional neural network and applied to ear surgery was developed. The method did not rely on any external fiducial markers nor human intervention for landmark recognition. The method was fast and its precision was compatible with ear surgery. [ABSTRACT FROM AUTHOR]

Hearing loss affects nearly 1.6 billion people and is the third-leading cause of disability worldwide. Chronic kidney disease (CKD) is also a common condition that is associated with adverse clinical outcomes and high health-care costs. From a developmental perspective, the structures responsible for hearing have a common morphogenetic origin with the kidney, and genetic abnormalities that cause familial forms of hearing loss can also lead to kidney disease. On a cellular level, normal kidney and cochlea function both depend on cilial activities at the apical surface, and kidney tubular cells and sensory epithelial cells of the inner ear use similar transport mechanisms to modify luminal fluid. The two organs also share the same collagen IV basement membrane network. Thus, strong developmental and physiological links exist between hearing and kidney function. These theoretical considerations are supported by epidemiological data demonstrating that CKD is associated with a graded and independent excess risk of sensorineural hearing loss. In addition to developmental and physiological links between kidney and cochlear function, hearing loss in patients with CKD may be driven by specific medications or treatments, including haemodialysis. The associations between these two common conditions are not commonly appreciated, yet have important implications for research and clinical practice. Chronic kidney disease is associated with a graded and independent excess risk of sensorineural hearing loss. This Review describes how disruption of shared signalling pathways that are important for the development of both the ear and the kidney and/or the presence of clinical drivers, such as specific medications or treatments, may underlie these associations. Key points: Chronic kidney disease (CKD) and hearing loss are common conditions that individually cause tremendous morbidity but which often coexist. The kidneys and the hearing organs share a common morphogenetic origin and rely on similar biological structures (for example, cilia) and processes (for example, specialized cellular transport mechanisms) to function. Genetic abnormalities that cause CKD can also cause hearing loss, and vice versa. A strong, graded and independent relationship exists between kidney function and the risk of hearing loss; the highest risk is observed in patients on haemodialysis, but kidney transplant recipients and people with mild CKD are also at increased risk. Infants, children and possibly adults with malformation or dysfunction of their hearing organs should be evaluated for the presence of malformation or dysfunction of their kidneys, and vice versa. Additional funding is needed to support basic, clinical and health services research that explores the intersection between these two relatively neglected conditions. [ABSTRACT FROM AUTHOR]

Purpose: Determine the prevalence of otological symptoms and tympanic membrane perforation, healing rates of tympanic membrane perforation with surgical and conservative management, and hearing function in civilian victims of terrorist explosions. Methods: A systematic review was conducted with searches on Medline, Embase, EMCare and CINAHL for publications between the 1st January 1945 and 26th May 2023. Studies with quantitative data addressing our aims were included. This review is registered with PROSPERO: CRD42020166768. Among 2611 studies screened, 18 studies comprising prospective and retrospective cohort studies were included. Results: The percentage of eardrums perforated in patients admitted to hospital, under ENT follow up and attending the emergency department is 69.0% (CI 55.5–80.5%), 38.7% (CI 19.0–63.0%, I2 0.715%) and 21.0% (CI 11.9–34.3%, I2 0.718%) respectively. Perforated eardrums heal spontaneously in 62.9% (CI 50.4–73.8%, I2 0.687%) of cases and in 88.8% (CI 75.9–96.3%, I2 0.500%) of cases after surgery. Common symptoms present within one month of bombings are tinnitus 84.7% (CI 70.0–92.9%, I2 0.506%), hearing loss 83.0% (CI 64.5–92.9%, I2 0.505%) and ear fullness 59.7% (CI 13.4–93.4%, I2 0.719). Symptomatic status between one and six months commonly include no symptoms 57.5% (CI 46.0–68.3%), hearing loss 35.4% (CI 21.8–51.8%, I2 0.673%) and tinnitus 15.6% (CI 4.9–40.0%, I2 0.500%). Within one month of bombings, the most common hearing abnormality is sensorineural hearing loss affecting 26.9% (CI 16.9–40.1%, I2 0.689%) of ears 43.5% (CI 33.4–54.2%, I2 0.500) of people. Conclusion: Tympanic membrane perforation, subjective hearing loss, tinnitus, ear fullness and sensorineural hearing loss are common sequelae of civilian terrorist explosions. [ABSTRACT FROM AUTHOR]

Purpose: To determine the prevalence of perilymphatic fistula (PLF) in sudden-onset sensorineural hearing loss (SSNHL) patients by employing the Cochlin-tomoprotein (CTP) detection test, a specific diagnostic marker for perilymph. We also analyzed the clinical characteristics associated with hearing outcomes in this cohort. Methods: A total of 74 eligible patients were prospectively enrolled. Following myringotomy, middle ear lavage (MEL) samples underwent the CTP test to identify perilymph leakage. Intratympanic dexamethasone (IT-DEX) therapy was administered, and hearing outcomes were assessed. Control groups comprised patients with chronic otitis media (n = 40) and non-inflammatory middle ears (n = 51) with concurrent MEL sample collection. Results: CTP was positive in 16 (22%) patients. No control samples showed positive results. Multiple regression analysis indicated that age and pre-treatment hearing levels significantly contributed to the CTP value. We found a positive correlation between CTP values, age, and pre-treatment pure-tone averages. Notably, CTP values in SSNHL cases aged 60 and above were significantly higher than in those below 60 years. Patients with positive CTP had significantly worse recovery rates after IT-DEX treatment. Conclusion: This study is the first prospective investigation demonstrating a positive relationship between CTP values, age, and hearing severity in SSNHL, indicating that PLF might be the essential cause of SSNHL, particularly in the elderly. Our findings suggest that IT-DEX may be less effective for PLF-associated SSNHL. Future research could reveal that PLF repair surgery is a viable treatment strategy for SSNHL. This study was registered under the UMIN Clinical Trials Registry (UMIN000010837) on 30/May/2013. [ABSTRACT FROM AUTHOR]

Purpose: Idiopathic sudden sensorineural hearing loss (ISSHL) can cause acute damage not only to the auditory function, but also to the vestibular function in addition to damage to the hearing function. The aim of this study was to perform vestibular assessment using caloric test and video head impulse test in patients with idiopathic sudden sensorineural hearing loss. In addition, to evaluate the relationship of dizziness with vestibular tests and post-treatment responses of vestibular tests. Methods: This is an observational, longitudinal and prospective study, including patients diagnosed with idiopathic sudden sensorineural hearing. Patients were divided into two groups according to the presence of vestibular complaints at presentation: Group 1: Patients with vestibular complaints, Group 2: Patients without vestibular complaints. All subjects underwent pure tone audiometry (PTA) testing, cold caloric test and video head impulse test (vHIT) during their admission and on the 10th day, 3rd month, and 1st year of their follow-up outpatient clinic controls. A unilateral weakness (UW) in the caloric test response was quantified according to the Jongkees formula. Results: A positive and significant relationship was found between the degree of hearing loss according to the ASHA criteria pre-treatment and the level of improvement created according to Siegel criteria at the 10th day, 3rd month, 1st year after treatment (respectively p = 0.001, p = 0.001, p < 0.001). When both short-term and long-term results were evaluated after treatment, a positive improvement in the degree of hearing loss was observed. A statistically significant difference was observed between Groups 1 and 2 when the pre-treatment, at 10 days, 3 months and 1 year after treatment the caloric test UW value was compared (respectively p = 0.020, p = 0.004, p = 0.004, p = 0.004). A statistically significant difference was observed between Groups 1 and 2 when the pre-treatment, at 10 days, 3 months and 1 year after treatment vHIT lateral canal VOR value was compared (respectively p = 0.000, p = 0.001, p = 0.000, p = 0.004). When both short-term and long-term results were evaluated after treatment, a positive improvement was observed in both caloric test results and lateral vHIT VOR values. Pre-treatment, post-treatment 10th day, 3rd month, 1st year vHIT anterior and posterior canal VOR values were found to be VOR˃0.8 in all patients. No difference was observed in anterior and posterior canal VOR values. Conclusion: Vertigo in patients with ISSHL "as objectively confirmed through caloric testing and vHIT" can be considered a sign of severe cochlear damage. Our study demonstrated a significantly increased risk of vestibular affect in patients with ISSHL, especially in the presence of vertigo. Thus, we conclude that the focus in ISSHL should not only be on the cochlea but also on the vestibular system. [ABSTRACT FROM AUTHOR]

Objective: To investigate the hypothesis that day-case cochlear implantation is associated with equal quality of life, hearing benefits and complications rates, compared to inpatient cochlear implantation. Study design: A single-center, non-blinded, randomized controlled, equivalence trial in a tertiary referral center. Methods: Thirty adult patients with post-lingual bilateral sensorineural hearing loss eligible for unilateral cochlear implantation surgery were randomly assigned to either the day-case or inpatient treatment group. The effect on general quality of life, patient satisfaction, (subjective) hearing improvement, postoperative complications and causes of crossover and/or readmission were assessed using questionnaires, auditory evaluations and patients' charts over a follow-up period of 1 year. Results: Overall quality of life measured by the HUI3 was equal between the day-case (n = 14) and inpatient group (n = 14). The overall patients' satisfaction showed a slight favor towards an inpatient approach. There was no significant difference in the subjective and objective hearing improvement between both treatment groups. During the 1-year follow-up period no major complications occurred. Minor complications occurred intraoperatively in three day-case patients resulting in three out of nine admissions of day-case patients. Other causes of admission of day-case patients were nausea and vomiting (n = 1), drowsiness (n = 1), late scheduled surgery (n = 2), social reasons (n = 1), or due to an unclear reason (n = 1). No patients required readmission. Conclusion: We found equal outcomes of QoL, patient satisfaction, objective, and subjective hearing outcomes between day-case and inpatient unilateral cochlear implantation. Nine out of 14 day-case patients were admitted for at least one night postoperatively (crossover). No major complications occurred in both groups. A day-case approach seems feasible when using specific patient selection, surgical planning and the preoperative provision of patient information into account. Besides this, the familiarity with a day-case approach of both patient and the surgical team can increase the feasibility of day-case surgery. Level of evidence: 1. [ABSTRACT FROM AUTHOR]

The cause of sudden sensorineural hearing loss (SSNHL) remains unknown in a significant number of cases, but vascular involvement in its pathophysiology has been proposed. Our study aimed to assess the incidence of stroke following idiopathic SSNHL (iSSNHL) and to evaluate associated cardiovascular risk factors and comorbidities. We extracted electronic medical record data from iSSNHL patients aged ≥ 50 years retrospectively from 84 general practices. Patients were matched for age, sex and general practice in a 1:4 ratio to controls. Primary outcome was the 5-years stroke risk following iSSNHL diagnosis. 480 iSSNHL cases could be matched to 1911 controls. The hazard ratio for iSSNHL compared with controls was 1.25 (95%CI 0.50–3.27; P = 0.646) for CVA (cerebrovascular accident) alone and 0.92 (95% CI 0.50–1.71; P = 0.804) for CVA and TIA (transient ischemic attack) combined. The hazard ratio for the interaction term between iSSNHL and age ≥ 60 years was 4.84 (95% CI 1.02–23.05; P = 0.048) for CVA and TIA combined. Patients with iSSNHL used antihypertensives and beta-blocking agents more frequently than controls (P = 0.006 and P = 0.022, respectively). In conclusion, no overall significant difference in the risk of stroke was observed, but the hazard ratio for stroke increased in iSSNHL patients aged 60 and older, suggesting potential vascular involvement in older subjects presenting with sudden sensorineural hearing loss. [ABSTRACT FROM AUTHOR]

Moderate-to-profound sensorineural hearing loss in humans is treatable by electrically stimulating the auditory nerve (AN) with a cochlear implant (CI). In the cochlea, the modiolus presents a porous bony interface between the CI electrode and the AN. New bone growth caused by the presence of the CI electrode or neural degeneration inflicted by ageing or otological diseases might change the effective porosity of the modiolus and, thereby, alter its electrical material properties. Using a volume conductor description of the cochlea, with the aid of a 'mapped conductivity' method and an ad-hoc 'regionally kinetic' equation system, we show that even a slight variation in modiolus porosity or pore distribution can disproportionately affect AN stimulation. Hence, because of porosity changes, an inconsistent CI performance might occur if neural degeneration or new bone growth progress after implantation. Appropriate electrical material properties in accordance with modiolar morphology and pathology should be considered in patient-specific studies. The present first-of-its-kind in-silico study advocates for contextual experimental studies to further explore the utility of modiolus porous morphology in optimising the CI outcome. [ABSTRACT FROM AUTHOR]

Introduction: As Hearing loss and dementia affect people with the same profile, several epidemiological studies have evaluated their relationship. However, the link between age-related hearing loss and Alzheimer's disease is still unclear. Methods: We selected subjects with no history of exposure to loud noises, blasts, head trauma with hearing loss, or sudden sensorineural hearing loss from a cohort intended to study preclinical phases of Alzheimer's disease. Participants are volunteers over 55 years without cognitive impairment. We correlated the results of an objective auditory evaluation with brain amyloid and p-tau181 levels and with the outcomes of a comprehensive neuropsychological assessment. Results: Fifty-five subjects at different stages of the Alzheimer's disease continuum were evaluated. There were no statistically significant correlations between amyloid-β and p-tau levels and any of the objective auditory measures. A weak but significant correlation was found between amyloid-β values and the Hearing Handicap Inventory for the Elderly. The neuropsychological domains more correlated to hearing loss were executive function and processing speed. Discussion: Age-related hearing loss is not linked to any pathological markers of Alzheimer's disease nor to neuropsychological domains typically affected in this disease. The Hearing Handicap Inventory for the Elderly has an important component of subjectivity and further studies are needed to explore its relationship with amyloid-β levels. [ABSTRACT FROM AUTHOR]

Purpose: Despite the recent trend of cochlear implantation (CI) at the age of six or even four months is prevalent in many centers around the world, clinicians should be cautious because perinatal risk factors of auditory neuropathy and/or delayed maturation carry the possibility of reversible hearing loss, yielding better auditory performance at the age of one year. The purpose of this study is to raise awareness that early CI may not be universal for all patients. In addition, we specify the factors to be considered in the pre-operative evaluation of CI in infants younger than one year. Methods and results: This study describes four cases provisionally diagnosed with severe to profound sensorineural hearing loss that were presented to the CI clinic to determine candidacy for implantation. Two cases had histories of prematurity, one had Down syndrome, and one had a family history of hearing loss. None of the study cases were candidates for CI, as they had varying degrees of hearing improvement. Conclusion: Although early CI may yield better auditory performance, the final diagnosis should be made only after repeated subjective and objective measurements as well as family feedback on the child's auditory performance, especially in preterm children. Early auditory brainstem response (ABR) prior to the age of one year in children with cognitive, neurologic, or developmental comorbidities should be interpreted with caution, as ABR "alone" could not accurately represent the child's true hearing ability in this patient population. [ABSTRACT FROM AUTHOR]

Purpose: To investigate the effect of the interval between bilateral cochlear implantation on the development of bilateral peripheral auditory pathways as revealed by the electrically evoked auditory brainstem response (EABR). Methods: Fifty-eight children with profound bilateral sensorineural hearing loss were recruited. Among them, 33 children received sequential bilateral cochlear implants (CIs), and 25 children received simultaneous bilateral CIs. The bilateral EABRs evoked by electrical stimulation from the CI electrode were recorded on the day of second-side CI activation. Results: The latencies of wave III (eIII) and wave V (eV) were significantly shorter on the first CI side than on the second CI side in children with sequential bilateral CIs but were similar between the two sides in children with simultaneous bilateral CIs. Furthermore, the latencies were prolonged from apical to basal channels along the cochlea in the two groups. In children with sequential CIs, the inter-implant interval was negatively correlated with the eV latency on the first CI side and was positively correlated with bilateral differences in the eIII and eV latencies. Conclusions: Unilateral CI use promotes the maturation of ipsilateral auditory conduction function. However, a longer inter-implant interval results in more unbalanced development of bilateral auditory brainstem pathways. Bilateral cochlear implantation with no or a short interval is recommended. [ABSTRACT FROM AUTHOR]

Purpose: Arterial stiffness, represented by estimated pulse wave velocity (ePWV), is the independent surrogate marker for cardiovascular event. The aim of the study was to investigate the significance of ePWV in the treatment outcome of idiopathic sudden sensorineural hearing loss (SSNHL). Methods: One hundred and ten patients with idiopathic SSNHL who hospitalized between April 2019 and March 2022 were evaluated. Arterial stiffness was calculated with formula for ePWV and other cardiovascular parameters of body mass index (BMI), and serum lipid level was determined. All patients received systemic high-dose steroid therapy and intratympanic steroid injections as a salvage management. Treatment outcome was assessed at 6 months after treatment, and classified as recovery and nonrecovery groups according to hearing recovery. Results: The initial pure-tone hearing threshold was 72.6 ± 23.8 dB and final hearing threshold was 52.63 ± 31.10 dB. After treatment, 60 (54.5%) patients included in recovery group and other 50 (45.5%) were classified as nonrecovery group. Age, days of onset to treatment, BMI, waist circumference, and ePWV were higher in the nonrecovery group compared to recovery group in univariate analysis (p = 0.039, p = 0.049, p = 0.003, p = 0.004, p = 0.007, respectively). In multivariate analysis, days of onset to treatment, BMI, and ePWV were associated with recovery (p = 0.030, p = 0.007, p = 0.022). Conclusion: Higher ePWV, a measure of arterial stiffness, was associated with a poor hearing recovery of SSNHL. [ABSTRACT FROM AUTHOR]

Purpose: In patients with unilateral sensorineural hearing loss (USNHL), we explored both objective functional audiological gains and subjective satisfaction, indicating when a unilateral hearing aid is valuable. Methods: Thirty-seven patients with mild-to-moderate USNHL (mean pure-tone thresholds between 25 and 70 dB) were prescribed unilateral hearing aids. Functional gain, the aided speech discrimination score (SDS), the Hearing in Noise Test (HINT) score, and the sound localization test score were collected, and a questionnaire (the Hearing Handicap Inventory for the Elderly, HHIE) completed after 1, 2, and 3 months of hearing aid use. We classified the participants as having 'no handicap' (HHIE < 17), 'mild-to-moderate handicap' (17–42), and 'significant handicap' (> 42). Results: The decrease in handicap afforded by unilateral hearing aids was largest in the 'significant handicap' group (the HHIE total score fell from 59.1 to 37.2; P = 0.007). There were no between-group differences in either functional gain or the aided SDS. Only the 'significant handicap' group evidenced an improved HINT score; the composite signal-to-noise ratio (SNR) fell from – 1.5 to – 2.2 dB [S/N] (P = 0.023). The HHIE usefully indicated when a hearing aid alleviated the discomfort of USNHL; patients with unaided HHIE scores ≥ 20 evidenced significant decreases in the composite SNR (– 1.7 to – 2.0 dB [S/N]; P = 0.045). Conclusions: When considering whether to prescribe a unilateral hearing aid for patients with mild-to-moderate USNHL, it is helpful to use the HHIE to evaluate discomfort. If the total score is ≥ 20, a hearing aid is appropriate. [ABSTRACT FROM AUTHOR]

Chronic myeloid leukemia is a type of blood cancer that affects the bone marrow and results in an overproduction of immature WBCs. The genetic mutation that causes CML is the BCR-ABL fusion gene. Adolescents are rarely affected. The case study aims to discuss a rare case of chronic myeloid leukemia causing bilateral hearing impairment, tinnitus, and vertigo. A 30-year-old female presented to the hospital in November, 2021, with sudden hearing impairment and other symptoms, leading to a CML diagnosis. Blood tests revealed hyperleukocytosis with marked neutrophilia, mild basophilia, and eosinophilia, and a BCR-ABL quantitation of 85%. Bone marrow aspiration showed granulocytic hyperplasia, mild left-shifted maturation, and less than 1% blasts. The patient was started with options including tyrosine kinase inhibitors (TKIs) such as Imatinib, which target the BCR-ABL fusion gene, reducing the number of leukemia cells and improving her white blood cell count. However, her deafness persisted, and she became dependent on hearing aids. CML presenting with hearing loss is rare, believed to be related to the infiltration of leukemic cells in the inner ear or microvascular complications. Treatment with tyrosine kinase inhibitors such as Imatinib can improve hematologic parameters, but the effect on hearing loss is uncertaint. [ABSTRACT FROM AUTHOR]

Aim: Anaemia is a prevalent medical condition that impacts a significant proportion of the worldwide populace. While the cardiovascular and respiratory systems' influence on anaemia has been extensively researched, its effect on the auditory system remains unclear. The objective of this investigation was to assess the pure tone audiometry of individuals with anaemia and establish a connection between the type of hearing impairment and the level of anaemia, if any. Materials and Methods: This cross-sectional study comprised 100 patients who were diagnosed with anaemia. All study participants underwent a thorough general examination and hearing assessment, which encompassed tuning fork tests, and pure-tone audiometry. Statistical analysis was utilized to determine the type and severity of hearing loss and its correlation with the degree of anaemia. Results: Our research findings indicate that 46.8% of moderately anaemic patients and 62.9% of patients with severe anaemia exhibited sensorineural hearing loss. A significant correlation was observed between the degree of anaemia (p < 0.05) and hearing loss. Our research findings indicate that individuals with moderate and severe anaemia exhibit a notably greater incidence of hearing impairment in comparison to those with mild anaemia. Conclusion: The research findings thus suggest a potential correlation between anaemia and auditory impairment. The timely identification and management of anaemia could potentially play a crucial role in preventing or reducing hearing impairment among individuals with anaemia. Additional research is required to clarify the mechanisms that underlie this association and to investigate possible interventions for mitigating the risk of hearing impairment in individuals with anaemia. [ABSTRACT FROM AUTHOR]

SNHL in children is an important issue. Cochlear implant is a highly technological device that is surgically inserted in the cochlea to solve this issue. To evaluate types of anomalies of the inner ear in children with sensorineural hearing loss in a tertiary care hospital and confirm that the routine MRI, MR cisternography and HRCT provides the surgeon with the imaging finding and criteria of patients candidates for CI. Patients and method: 600 patients with SNHL underwent HRCT and MRI. CT examinations were normal in 457 patients (76.2%) and 143 patients (23.8%) with inner ear anomalies. MRI examinations were normal in 440 patients (73.3%) and had inner ear anomalies in 160 patients (26.7%). 3D bFFE and 3D DRIVE was summarized. The 3D bFFE sequence was statistically significantly better than the 3D DRIVE for the demonstration of the cochlear vestibule. Superior, inferior vestibular nerves and facial nerves while 3D DRIVE is superior to 3D bFFE in the visualization of the semicircular canals. HRCT and MRI provide accurate anatomical delineation of complex inner ear structures and 3D improves pre-implant evaluation. [ABSTRACT FROM AUTHOR]

There are discrepancies in the literature about the specific influence of leprosy on auditory functions. In routine clinical practice regular hearing screening of leprosy patients is not done. Due to conflicting reports of auditory system involvement in the literature, we conducted this case control study to evaluate the need of regular hearing screening in leprosy patients. A complete otological examination of thirty leprosy patients was conducted. Thirty age and sex matched healthy individual attending ear, nose and throat outpatient department were enrolled as control. Ten cases (33.3%) out of the thirty leprosy patients were found to have high frequency (4 and 8 kHZ) sensorineural hearing loss. The results of this study suggest that leprosy patients require routine monitoring for auditory functions for early identifications of sensorineural hearing loss. As our study is a case-control study with small sample size, in future large prospective studies are required to evaluate the correlation of hearing loss with leprosy and to see the progression hearing loss. Key points: In routine clinical practice regular hearing screening of leprosy patients is not done. We found high frequency sensorineural hearing loss in 33.3% of leprosy patients. From the results it can be concluded that hearing loss in leprosy is cochlear. Future large prospective studies are required to evaluate the correlation of hearing loss with leprosy and its progression with disease. [ABSTRACT FROM AUTHOR]

Cochlear implant surgery is the standard of care for severe sensorineural hearing loss. Infection followed by implant extrusion is an infrequent complication of this surgery. The ideal treatment is explantation of the implant. However, implant removal and reimplantation is a challenging surgery and may have poor speech reception outcomes. The cost of a new implant especially in developing countries is also a deterrent. Our study dwells on the feasibility of salvaging exposed cochlear implants by a combination of pericranial flaps followed by a scalp flap cover. The study was done in a tertiary care hospital over a period of six years. Out of 303 cochlear implant surgeries, 12 patients had implant exposure and extrusion. Patients having meningitis and sepsis were excluded from the study. All patients underwent debridement and cover with double flap (Pericranial flaps and scalp rotation flap). The average operating time was 2.17 h. The surgery is technically simple with a short learning curve. It brings in rich blood supply and there is fair amount of tissue mobility. In 11 patients we were able to salvage the implant. Patients were followed for a period of 01 year post operatively. Our study suggests that salvage of infected implant should be attempted as it is feasible, durable and effective in appropriate patients. [ABSTRACT FROM AUTHOR]

The Objective of the study was to assess the ossicular status in chronic otitis media (COM)—mucosal and squamosal type and statistically evaluate the extent of ossicular destruction intraoperatively in COM patients. The findings of this study could help us to predict preoperatively the probability of having ossicular chain destruction in COM ears and thus patients could therefore be properly consented about these potential issues before surgery. The study was carried out in ENT department of tertiary health care hospital, between January 2019 to January 2020. All patients of all age groups and both genders, diagnosed with COM Mucosal and Squamosal Type with complaints of ear discharge and hearing loss with good cochlear reserve and requiring surgery were included in the study, after taking informed written consent in vernacular language. All the patients included in the study were evaluated with detailed history, clinical examination including otomicroscopy, tuning fork tests and pure tone audiometry. The patients were then posted for ear surgery and the middle ear status and ossicular chain status were assessed using a microscope intraoperatively. Out of 98 patients, 45(45.9%) had mucosal and 53 (54.08%) had squamosal disease. Ossicular chain was eroded in 69 cases (70.5%). 23 out of 45 (51.1%) mucosal cases and 46 out of 53 squamosal cases (86.7%) reported ossicular erosion. Most frequently involved was long process of incus > stapes > malleus. From our study, we concluded that there is a significant relationship between type of disease pathology in middle ear and ossicular erosion being higher in Squamosal type of COM, with malleus being the most resistant and incus being the most susceptible ossicle. [ABSTRACT FROM AUTHOR]

Sensorineural hearing loss and chronic rhinosinusitis with nasal polyps affect a large proportion of the UK population. However, treatment with high efficacy to manage these pathologies remains to be found. With the development of biologic drug treatments, there is hope that this will improve quality of life for patients affected by these conditions. A literature review was carried out using PubMed. Studies that investigated the use of biologic drug treatment in chronic rhinosinusitis with nasal polyps, head and neck squamous cell carcinoma and sensorineural hearing loss were included in this paper. Studies have demonstrated that cost is quite possibly the greatest limiting factor for the licencing of these novel drugs by the National Institute for Health and Care Excellence. Biologics are currently approved in the management of squamous cell carcinoma of the head and neck for curative and palliative management. Although sensorineural hearing loss and chronic rhinosinusitis with nasal polyps are not life-threatening conditions, they do have a great effect upon a patients' quality of life. Current literature has identified several drug targets for biologics to be developed. Biologics, used off-licence, have been demonstrated to be effective in the management of the ear, nose and throat pathologies described. Although the off-licence use of biologics have been found to be beneficial, larger randomised control trials will be required to demonstrate the true efficacy of these drugs and allow for approval by the National Institute for Health and Care Excellence. Cost will become less of a limiting factor as time progresses as biosimilars will be able to be produced once drug patents expire, thus making the market more competitive, driving prices down. [ABSTRACT FROM AUTHOR]

Objective: The aim of this multicentric cross-sectional study was to collect phenotypes and clinical variability on a large sample of 244 patients enrolled in different university centers in Italy, trying to differentiate subtypes of VM. Background: VM is one of the most frequent episodic vertigo characterized by a great clinical variability for duration of attacks and accompanying symptoms. Diagnosis is based only on clinical history of episodic vertigo in 50% of cases associated with migrainous headache or photo/phonophobia. Methods: We enrolled in different university centers 244 patients affected by definite VM according to the criteria of the Barany Society between January 2022 and December 2022. An audiometric examination and a CNS MRI were performed before inclusion. Patients with low-frequency sensorineural hearing loss were not included, as well as patients with an MRI positive otherwise that for microischemic lesions. Patients were asked to characterize vestibular symptoms choosing among (multiple answers were allowed): internal vertigo, dizziness, visuo-vestibular symptoms/external vertigo; onset of vertigo and duration, neurovegetative, and cochlear accompanying symptoms (hearing loss, tinnitus, and fullness during attacks) were collected as well as migrainous headache and/or photo/phonophobia during vertigo; autoimmune disorders were also analyzed. A bedside examination was performed including study of spontaneous-positional nystagmus with infrared video goggles, post head shaking ny, skull vibration test, and video head impulse test. Results: We included 244 subjects, 181 were females (74.2%). The age of onset of the first vertigo was 36.6 ± 14.5 while of the first headache was 23.2 ± 10.1. A positive correlation has been found between the first headache and the first vertigo. The mean duration of vertigo attacks was 11 ± 16 h. We carried on a cluster analysis to identify subgroups of patients with common clinical features. Four variables allowed to aggregate clusters: age of onset of vertigo, duration of vertigo attacks, presence of migrainous headache during vertigo, and presence of cochlear symptoms during vertigo. We identified 5 clusters: cluster 1/group 1 (23 subjects, 9.4%) characterized by longer duration of vertigo attacks; cluster 2/group 2 (52 subjects, 21.3%) characterized by absence of migrainous headache and cochlear symptoms during vertigo; cluster 3/group 3 (44 subjects, 18%) characterized by presence of cochlear symptoms during vertigo but not headache; cluster 4/group 4 (57 subjects, 23.4%) by the presence of both cochlear symptoms and migrainous headache during vertigo; cluster 5/group 5 (68 subjects, 27.9%) characterized by migrainous headache but no cochlear symptoms during vertigo. Conclusion: VM is with any evidence a heterogeneous disorder and clinical presentations exhibit a great variability. In VM, both symptoms orienting toward a peripheral mechanism (cochlear symptoms) and central ones (long lasting positional non-paroxysmal vertigo) may coexist. Our study is the first published trying to characterize subgroups of VM subjects, thus orienting toward different pathophysiological mechanisms. [ABSTRACT FROM AUTHOR]

Biallelic pathogenic variants in MAP3K20, which encodes a mitogen-activated protein kinase, are a rare cause of split-hand foot malformation (SHFM), hearing loss, and nail abnormalities or congenital myopathy. However, heterozygous variants in this gene have not been definitively associated with a phenotype. Here, we describe the phenotypic spectrum associated with heterozygous de novo variants in the linker region between the kinase domain and leucine zipper domain of MAP3K20. We report five individuals with diverse clinical features, including craniosynostosis, limb anomalies, sensorineural hearing loss, and ectodermal dysplasia-like phenotypes who have heterozygous de novo variants in this specific region of the gene. These individuals exhibit both shared and unique clinical manifestations, highlighting the complexity and variability of the disorder. We propose that the involvement of MAP3K20 in endothelial–mesenchymal transition provides a plausible etiology of these features. Together, these findings characterize a disorder that both expands the phenotypic spectrum associated with MAP3K20 and highlights the need for further studies on its role in early human development. [ABSTRACT FROM AUTHOR]

Auditory neuropathy (AN) is a unique type of language developmental disorder, with no precise rate of genetic contribution that has been deciphered in a large cohort. In a retrospective cohort of 311 patients with AN, pathogenic and likely pathogenic variants of 23 genes were identified in 98 patients (31.5% in 311 patients), and 14 genes were mutated in two or more patients. Among subgroups of patients with AN, the prevalence of pathogenic and likely pathogenic variants was 54.4% and 56.2% in trios and families, while 22.9% in the cases with proband-only; 45.7% and 25.6% in the infant and non-infant group; and 33.7% and 0% in the bilateral and unilateral AN cases. Most of the OTOF gene (96.6%, 28/29) could only be identified in the infant group, while the AIFM1 gene could only be identified in the non-infant group; other genes such as ATP1A3 and OPA1 were identified in both infant and non-infant groups. In conclusion, genes distribution of AN, with the most common genes being OTOF and AIFM1, is totally different from other sensorineural hearing loss. The subgroups with different onset ages showed different genetic spectrums, so did bilateral and unilateral groups and sporadic and familial or trio groups. [ABSTRACT FROM AUTHOR]

Identification of genes associated with nonsyndromic hearing loss is a crucial endeavor given the substantial number of individuals who remain without a diagnosis after even the most advanced genetic testing. PKHD1L1 was established as necessary for the formation of the cochlear hair-cell stereociliary coat and causes hearing loss in mice and zebrafish when mutated. We sought to determine if biallelic variants in PKHD1L1 also cause hearing loss in humans. Exome sequencing was performed on DNA of four families segregating autosomal recessive nonsyndromic sensorineural hearing loss. Compound heterozygous p.[(Gly129Ser)];p.[(Gly1314Val)] and p.[(Gly605Arg)];p[(Leu2818TyrfsTer5)], homozygous missense p.(His2479Gln) and nonsense p.(Arg3381Ter) variants were identified in PKHD1L1 that were predicted to be damaging using in silico pathogenicity prediction methods. In vitro functional analysis of two missense variants was performed using purified recombinant PKHD1L1 protein fragments. We then evaluated protein thermodynamic stability with and without the missense variants found in one of the families and performed a minigene splicing assay for another variant. In silico molecular modeling using AlphaFold2 and protein sequence alignment analysis were carried out to further explore potential variant effects on structure. In vitro functional assessment indicated that both engineered PKHD1L1 p.(Gly129Ser) and p.(Gly1314Val) mutant constructs significantly reduced the folding and structural stabilities of the expressed protein fragments, providing further evidence to support pathogenicity of these variants. Minigene assay of the c.1813G>A p.(Gly605Arg) variant, located at the boundary of exon 17, revealed exon skipping leading to an in-frame deletion of 48 amino acids. In silico molecular modeling exposed key structural features that might suggest PKHD1L1 protein destabilization. Multiple lines of evidence collectively associate PKHD1L1 with nonsyndromic mild–moderate to severe sensorineural hearing loss. PKHD1L1 testing in individuals with mild–moderate hearing loss may identify further affected families. [ABSTRACT FROM AUTHOR]

Objective: Report three cases of simultaneous triple semicircular canal occlusion (TSCO) and cochlear implantation (CI) as the treatment of intractable Meniere's disease (MD). Case reports: Patients with MD can present occasionally with intractable vertigo and profound sensorineural hearing loss (SNHL). TSCO and CI have been proposed to control vertigo and restore profound deafness in patients with MD separately. However, a few studies have reported simultaneous TSCO and CI in the same surgical procedure for the treatment of MD. In the present study, we described three patients with MD showing incapacitating vertigo and severe SNHL who underwent simultaneous TSCO and CI after examinations of auditory system, vestibular system, and imaging. Their symptoms were significantly alleviated during the follow-up period. Conclusion: The combined TSCO and CI remains a viable treatment option which is effective for the control of vertigo as well as the restoring of hearing in patients with MD. [ABSTRACT FROM AUTHOR]

Hearing loss is considered one of the most common sensory neurological defects, with approximately 60% of cases attributed to genetic factors. Human pathogenic variants in the TBC1D24 gene are associated with various clinical phenotypes, including dominant nonsyndromic hearing loss DFNA65, characterized by progressive hearing loss after the development of language. This study provides an in-depth analysis of the causative gene and mutations in a family with hereditary deafness. We recruited a three-generation family with autosomal dominant nonsyndromic hearing loss (ADNSHL) and conducted detailed medical histories and relevant examinations. Next-generation sequencing (NGS) was used to identify genetic variants in the proband, which were then validated using Sanger sequencing. Multiple computational software tools were employed to predict the impact of the variant on the function and structure of the TBC1D24 protein. A series of bioinformatics tools were applied to determine the conservation characteristics of the sequence, establish a three-dimensional structural model, and investigate changes in molecular dynamics. A detailed genotype and phenotype analysis were carried out. The family exhibited autosomal dominant, progressive, postlingual, and nonsyndromic sensorineural hearing loss. A novel heterozygous variant, c.1459C>T (p.His487Tyr), in the TBC1D24 gene was identified and confirmed to be associated with the hearing loss phenotype in this family. Conservation analysis revealed high conservation of the amino acid affected by this variant across different species. The mutant protein showed alterations in thermodynamic stability, elasticity, and conformational dynamics. Molecular dynamics simulations indicated changes in RMSD, RMSF, Rg, and SASA of the mutant structure. We computed the onset age of non-syndromic hearing loss associated with mutations in the TBC1D24 gene and identified variations in the hearing progression time and annual threshold deterioration across different frequencies. The identification of a new variant associated with rare autosomal dominant nonsyndromic hereditary hearing loss in this family broadens the range of mutations in the TBC1D24 gene. This variant has the potential to influence the interaction between the TLDc domain and TBC domain, thereby affecting the protein's biological function. [ABSTRACT FROM AUTHOR]

The optimal treatment for sudden sensorineural hearing loss (SSNHL) is unclear. Hyperbaric oxygen therapy (HBOT) has been suggested as a viable option for treatment of SSNHL as it improves vascular dysfunction. In this study, we evaluated the therapeutic effects of HBOT by retrospectively reviewing the records of 2206 patients with SSNHL. 54 who had received HBOT were selected for the HBOT groups, while 59 age-matched controls who had not were selected for the control groups. The HBOT and control groups were divided into subgroups according to intratympanic steroid (ITS) use. Groups A–D had received oral steroids + HBOT, oral steroids only, oral steroids + ITS + HBOT, and oral steroids + ITS, respectively. Of the 113 SSNHL patients, 21 had diabetes mellitus (DM) (2, 0, 9, and 10 patients in Groups A–D, respectively). There was no notable difference in hearing improvement between patients receiving HBOT and those in the control group. However, among diabetic patients, those who underwent HBOT demonstrated a significant improvement in hearing when compared to the control group. The combination of HBOT and steroids could potentially be beneficial for treating severe to profound SSNHL patients with DM. [ABSTRACT FROM AUTHOR]

Background: To report the presentation, diagnostic process, management and results of cochlear implantation of patients diagnosed with Bjornstad syndrome with profound sensorineural hearing loss (SNHL). Case presentation and management: A retrospective report of two siblings with Bjornstad syndrome suffering profound SNHL unresponsive to conventional hearing aids treated with bilateral simultaneous cochlear implantation. Setting: Tertiary-referral center. Results: Cochlear implant surgeries of two siblings (four ears) with profound SNHL and bilateral inner ear anomaly (incomplete partition type 1) were performed without complications. Postoperative audiometric measurements showed a significiant improvement in pure-tone threshold and a word recognition score. In the literature review, no previous case of Bjornstad syndrome treated with cochlear implantation has been reported. Conclusions: Cochlear implantation is an effective, safe, and ultimate treatment option for Bjornstad syndrome with profound SNHL not responding to hearing aids. [ABSTRACT FROM AUTHOR]

Purpose: To investigate 2-year post-operative hearing performance, safety, and patient-reported outcomes of hearing-impaired adults treated with the Osia® 2 System, an active osseointegrated bone-conduction hearing implant that uses piezoelectric technology. Methods: A prospective, multicenter, open-label, single-arm, within-subject clinical study conducted at three tertiary referral clinical centers located in Melbourne, Sydney and Hong Kong. Twenty adult recipients of the Osia 2 System were enrolled and followed up between 12 and 24 months post-implantation: 17 with mixed or conductive hearing loss and 3 with single-sided sensorineural deafness. Safety data, audiological thresholds, speech recognition thresholds in noise, and patient-reported outcomes were collected and evaluated. In addition, pre-and 6-month post-implantation data were collected retrospectively for this recipient cohort enrolled into the earlier study (ClinicalTrials.gov NCT04041700). Results: Between 6- and 24-month follow-up, there was no statistically significant change in free-field hearing thresholds or speech reception thresholds in noise (p = > 0.05), indicating that aided improvements were maintained up to 24 months of follow-up. Furthermore, improvements in health-related quality of life and daily hearing ability, as well as clinical and subjective measures of hearing benefit remained stable over the 24-month period. No serious adverse events were reported during extended follow-up. Conclusions: These study results provide further evidence to support the longer term clinical safety, hearing performance, and patient-related benefits of the Osia 2 System in patients with either a conductive hearing loss, mixed hearing loss, or single-sided sensorineural deafness. Trial Registration: ClinicalTrials.gov Identifier: NCT04754477. First posted: February 15, 2021. [ABSTRACT FROM AUTHOR]

Purpose: Age-related sensorineural hearing loss (SNHL), is a progressive, usually bilateral hearing loss that occurs in elderly. It is correctable using rehabilitative hearing devices, which can vastly improve the older person's quality of life. This study aimed to find out the prevalence and related factors of age-related sensorineural hearing loss among elderly patients in Tanzania. Methods: A total of 380 elderly patients who received Otorhinolaryngology (ORL) services at Muhimbili tertiary hospital were enrolled. Participants were screened for hearing loss and interviewed by the structured questionnaire followed by otoscopic examination and Pure tone Audiometry (PTA). Results: This study included 182(47.9%) males, and 198 (52.1%) females. The prevalence of SNHL was 27.6%, males were mostly affected 57(31.3%) p = 0.574. Bilateral SNHL was found 90 (85.7%) p = 0.026, and the severity of age-related SNHL was found to increase as age increases. Conclusion: Sensorineural hearing loss among the elderly was prevalent, mostly bilateral, and the severity increases with age. Confounding factors such as hypertension, diabetes mellitus (DM), cigarette smoking, and ototoxic medication may have contributed. Elderly should be screened for hearing loss especially on the individuals with other co-morbidities for early diagnosis and management in order to improve the quality of life of the elderly. [ABSTRACT FROM AUTHOR]

Purpose: The round window approach has become the most preferred route for electrode insertion in cochlear implant surgery; however, it is not possible at times due to difficult round window membrane (RWM) visibility. Our study aims to investigate the relationship between preoperative radiological parameters and the surgical visibility of the RWM in Cochlear implant patients. Methodology: A prospective cross-sectional study of 31 patients, age < 6 years, with bilateral severe to profound sensorineural hearing loss was conducted at a tertiary care hospital. The preoperative HRCT temporal bone scan was studied, and the parameters evaluated were facial nerve location, facial recess width, and RWM visibility prediction. All patients were operated on via the posterior tympanotomy. The surgical RWM visibility was done after optimal drilling of the posterior tympanotomy recess. The relationship between the radiological parameters and surgical visibility of RWM was evaluated. Results: The difference in the facial nerve location as per the type of RWM was found to be significant (p value < 0.05). However, the facial recess width was not significantly associated with RWM visibility. The radiological prediction of RWM visibility by tracing the prediction line over RWM was significantly associated with intraoperative RWM visibility. Conclusion: The goal to look for preoperative scans is to predict the ease or difficulty of RWM visibility during surgery. The difficult visualization of the RWM, can result in dire intraoperative consequences. A comprehensive understanding of preoperative radiological parameters, coupled with meticulous surgical planning, is crucial to address these challenges effectively by focusing on enhancing RWM visualization. [ABSTRACT FROM AUTHOR]

Cochlear Implantation (CI) is a well-accepted treatment for severe-to-profound sensorineural hearing loss, refractory to conventional hearing amplification. Pre-operative Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) play pivotal roles in patient selection to rule out findings that preclude surgery or identify conditions that may impact the surgical procedure. A prospective study was carried out in a tertiary care center over three years, from January 2020 to January 2023. One hundred and ninety (380 ears) patients' High-Resolution Computed Tomography (HRCT) studies of the temporal bone and MRI scans of the auditory pathways were analyzed. A reporting format was followed which was devised by a team of senior implant surgeons and senior neuro-radiologists. Our study aims to provide a comprehensive radiologic protocol for CI candidacy including normative data for the essential morphometrics in the Indian setting. [ABSTRACT FROM AUTHOR]

It seems that food factors and the type of nutrition have an effect on the function of the auditory system. Hearing is one of the most important senses for social communication and high cognitive behaviors. Sensorineural hearing loss leaves adverse and permanent consequences in all aspects of personal and social life of affected patients. Hence, this narrative review study was designed to determine the relationship between sensorineural hearing loss and type of diet. Based on the inclusion criteria, the full text of 62 articles published between 2005 and 2023 were extracted from Scopus, Medline [PubMed], Web of Science, and Google Scholar websites and constituted the sources of this research. The results of the studies showed that by limiting the consumption of foods rich in cholesterol, sugar, carbohydrates, and protein, hearing is protected against the factors that cause sensorineural hearing loss. Also, increasing the consumption of vegetables, fruits, omega-3, antioxidants in the form of vitamins A, C, E reduce hearing susceptibility due to noise exposure, presbycusis, ototoxic agents, and etc. Healthy diet includes eating all the nutrients the body needs in a balanced way. Healthy lifestyle factors including continuous physical activity, good sleep quality, quitting smoking, stay away from stressful factors or relaxation, and avoiding exposure to environmental noise. By following healthy eating and lifestyle patterns, the conditions for hearing, physical and mental health are provided. [ABSTRACT FROM AUTHOR]

Bilateral sensorineural hearing loss can be a very distressing symptom and can affect the efficiency of a person and one's quality of life. Conditions causing bilateral hearing loss are very few and autoimmune aetiology is one of them. Autoimmune ear disease is characterised by bilateral, mostly fluctuating audiovestibular symptoms and symptoms which respond to steroids. Diagnosis of AIED presents a unique challenge to clinicians due to the lack of standardized diagnostic criteria or reliable pathognomonic tests. The purpose of the study is to evaluate the patients who fit into criteria of autoimmune inner ear disease and understand the clinical features and response to medications for the same. A retrospective chart review of patients presenting with rapidly progressive bilateral hearing loss was done. The clinical presentation including detailed history and examination findings along with the blood investigation reports and audiograms were recorded in a tabular form. The study included 6 patients – 3 male and 3 female patients. Age of the patients at onset of hearing loss varied between 24–35 years. 3 of 6 patients presented with primary autoimmune ear disease and other 3 had hearing loss secondary to systemic autoimmune disease. All patients were treated with systemic steroids, but however showed a varied response. Patients with primary AIED were administered inner ear steroid therapy as well. AIED is thus a diagnosis of exclusion done with high index of suspicion. Patients with bilateral progressive sensorineural hearing loss should be evaluated for autoimmune etiology. Oral steroids with intratympanic steroids are currently the mainstay of treatment for AIED. Guarded prognosis of hearing improvement is noted in these patients. Hence, emphasis should be placed on early hearing rehabilitation for better quality of life. [ABSTRACT FROM AUTHOR]

Brown Vialetto Van Laere syndrome is a rare disorder characterized by progressive pontobulbar palsy with bilateral sensorineural hearing loss and lower cranial nerve palsies. Fifty-eight cases have been reported in the last hundred years. As the most common presenting complaint of this disorder is hearing loss, audiological evaluation plays a vital role in pointing towards and narrowing its diagnosis. We present a case report of a 12-year male child affected by this disorder. [ABSTRACT FROM AUTHOR]

We present a patient who suffered a temporal bone fracture (TBF) encompassing the bony labyrinth. Sensorineural hearing loss was confirmed with an unfavorable prognosis for recovery. Thirteen years later, there is regression of the hearing loss. [ABSTRACT FROM AUTHOR]

The metabolic syndrome (MS) is a cluster of conditions that occur. togehther, increase risk of heart disease, storke, type 2 diabetes mellitus and hypertension as a possible outcome. The previous research has shown a link between hearing loss and being overweight, diabetic, or suffering from heart disease. However, research on the possible link between hearing loss and metabolic syndrome is limited. Hearing loss due to metabolic syndrome was evaluated in the present investigation. Two hundred individuals with metabolic syndrome were included. All the patients were evaluated on three types of audiometry (pure tone, impedence, and DPOAE).Anthropometric data, blood pressure, blood sugar, and lipid profiles, were all collected from each patient. We also asked about their smoking and drinking habits in the past. SPSS v. 22.0 was used to conduct the statistical analysis. Overall, SNHL affected 58.5% of patients. Patients having moderate hearing loss were the largest demographic group (40%), followed by those with mild hearing loss (15%). Severe hearing loss only occurred in 3.5% of patients. Hearing loss was shown to be more prevalent in patients with more than three components of metabolic syndrome. Significant associations were found between hearing impairment and metabolic risk factors as waist circumference, fasting blood sugar, serum high-density lipoprotein, serum triglycerides, and systolic and diastolic blood pressure. Hearing loss was only marginally connected to smoking and excessive drinking. [ABSTRACT FROM AUTHOR]

Senile deafness and hearing loss in adults over 50 are major public health issues as a result of the population's ageing demographic. Menopausal women tend to develop hearing loss, while no clear link has been found between the two. The purpose of this study was to determine how menopause and diabetes mellitus affects hearing loss. We assessed 158 menopausal women in total. Pure Tone Audiometry and HbA1c levels was measured, along with appropriate clinical history and examination. The association between those levels and hearing was researched by chi-square test. There were 158 study participants. Mean age of the study population was 50.5 (± 2.49) years. Onset and duration of hard of hearing was assessed in 41 patients (25.9%). 33% (N = 53) of the patients had history of Diabetes mellitus, of which 52.8% offered history of the disease for more than or equal to five years. On audiological assessment, 74.1% had no hearing loss (N = 117), 4.4% had unilateral sensorineural hearing loss (N = 7) and 21.5% had bilateral sensorineural hearing loss (N = 34). Statistics show that hearing loss is statistically connected with ageing and poor glycemic management. With chi square values of 9.629 and P value 0.002 found a significant correlation between ageing and hearing loss. Poor glycemic control is significantly associated with hearing loss with a chi-square value of 4.304 and P value 0.038. Poor glycemic control and menopause is found to be strongly associated with sensorineural hearing loss. Further prospective, hormonal studies including larger population is recommended. [ABSTRACT FROM AUTHOR]

Idiopathic sudden sensorineural hearing loss (ISSNHL) is challenging for both nephrologists and otolaryngologists treating patients undergoing dialysis. This single-center, retrospective, observational study investigated the treatment outcomes of patients with ISSNHL undergoing dialysis, enrolling 700 patients (47 undergoing and 653 not undergoing dialysis) diagnosed with ISSNHL between January 2005 and December 2021 at Asan Medical Center, Republic of Korea. To balance pre-existing clinical characteristics, 1:5 propensity score matching (PSM) was performed with the patients who were not undergoing dialysis. Treatment included high-dose systemic steroid therapy or intra-tympanic steroid injections. The pure tone average of the groups was compared before and 2 weeks and 2 months after treatment. The hearing-improvement degree was evaluated using Siegel's criteria. Before PSM, age, prevalence of diabetes or hypertension, initial hearing threshold at each frequency level (0.5, 1, 2, and 4 kHz), and treatment strategies exhibited significant between-group differences. However, in the PS-matched cohort, none of the confounders showed significant between-group differences. Two months after steroid treatment, the non-dialysis patient group demonstrated significantly higher average improvement in pure tone audiometry (P = 0.029) and greater percentage of complete response according to Siegel's criteria. This study suggests that treatment outcomes for ISSNHL are significantly poorer for patients undergoing than for those not undergoing dialysis. [ABSTRACT FROM AUTHOR]

Purpose: Benign paroxysmal positional vertigo (BPPV) may be found in patients complaining of hearing disorders. The aim of our investigation was to describe audiological findings in BPPV patients, focusing on subjects with asymmetric hearing loss (AHL), to better understand whether otoconial displacement may occur preferentially in the worst hearing ear. Methods: A prospective study was performed on 112 BPPV patients. We divided the sample into subjects who suffered from AHL (G1) and patients with did not (G2). Data regarding vestibular symptoms, tinnitus, migraine, antivertigo drug therapy, and vascular risk factors were collected. Results: Out of 30 AHL subjects, 83.33% of them were affected by sensorineural hearing loss (SNHL) in at least one ear, with a significant difference in the distribution of hearing loss type between groups (p = 0.0006). In 70% of cases, the ear affected by BPPV was the one with the worst hearing threshold (p = 0.02); threshold asymmetry predicted BPPV in the worst hearing ear (p = 0.03). The predictability depended neither on the hearing threshold gap between ears nor the severity of the hearing threshold in the worst ear (p > 0.05). No differences in vascular risk factors between groups were observed (p > 0.05). We evidenced a moderate correlation between age and hearing threshold (ρ = 0.43). Age did not result a predictive factor for residual dizziness or BPPV in the worst ear (p > 0.05). Conclusions: Our study supports the likelihood of an otoconial displacement in the worse hearing ear in BPPV patients. Clinicians should start testing the worst hearing ear when managing AHL patients with suspected BPPV. [ABSTRACT FROM AUTHOR]

Objectives: Internationally, corticosteroids are still the mainstay treatment for patients with idiopathic sudden sensorineural hearing loss (ISSHL). This is a retrospective monocentric study investing the impact of adding N-acetylcysteine (NAC) to prednisolone treatment on patients with ISSHL at a tertiary university otorhinolaryngology department. Methods: 793 patients (median age 60 years; 50.9% women) with a new diagnosis of ISSHL from 2009 to 2015 were included in the study. 663 patients received NAC administration in addition to standard tapered prednisolone treatment. Univariate and multivariable analysis were performed to identify independent factors regarding negative prognosis of hearing recovery. Results: Mean initial ISSHL and hearing gain after treatment in 10-tone pure tone audiometry (PTA) were 54.8 ± 34.5 dB and 15.2 ± 21.2 dB, respectively. In univariate analysis, treatment with prednisolone and NAC was associated with a positive prognosis of hearing recovery in the Japan classification in 10-tone PTA. In multivariable analysis on Japan classification in 10-tone PTA including all significant factors from univariate analysis, negative prognosis of hearing recovery were age > median (odds ratio [OR] 1.648; 95% confidence interval [CI] 1.139–2.385; p = 0.008), diseased opposite ear (OR 3.049; CI 2.157–4.310; p < 0.001), pantonal ISSHL (OR 1.891; CI 1.309–2.732; p = 0.001) and prednisolone alone without NAC treatment (OR 1.862; CI 1.200–2.887; p = 0.005). Conclusions: Prednisolone treatment combined with NAC resulted in better hearing outcomes in patients with ISSHL than treatment without NAC. [ABSTRACT FROM AUTHOR]

Despite advances in obstetric care, hypoxic ischemic encephalopathy (HIE) remains a significant disease burden. We determined the national trends of HIE prevalence, therapeutic hypothermia (TH) use, mortality, and outcomes from 2012 to 2019. This study included term infants diagnosed with HIE between 2012 and 2019 from the National Health Insurance Service database. The prevalence of HIE was 2.4 per 1000 births without significant change during the period. TH was performed in approximately 6.7% of infants with HIE, and the annual variation ranged from 2.4 to 12.5%. The mortality among all term infants with HIE was 4.6%. The mortality rate among infants with HIE and TH significantly declined from 40 to 16.9% during the eight years. Infants with TH had higher mortality, increased use of inhaled nitric oxide, and more invasive ventilator use, indicating greater disease severity in the TH group. Infants with TH also showed significantly poorer outcomes, including delayed development, cerebral palsy, sensorineural hearing loss, and seizure, compared to infants without TH (p < 0.0001). With the increasing application of TH, mortality and developmental outcomes among infants with HIE have been improving in the past eight years in Korea. Further efforts to improve outcomes should be needed. [ABSTRACT FROM AUTHOR]

There has been a growing interest in studying the usefulness of chirp stimuli in recording cervical vestibular evoked myogenic potential (cVEMP) waveforms. Nevertheless, the study outcomes are debatable and require verification. In view of this, the aim of the present study was to compare cVEMP results when elicited by 500 Hz tone burst and narrowband (NB) CE-Chirp stimuli in adults with sensorineural hearing loss (SNHL). Fifty adults with bilateral SNHL (aged 20–65 years) underwent the cVEMP testing based on the established protocol. The 500 Hz tone burst and NB CE-Chirp (centred at 500 Hz) stimuli were presented to each ear at an intensity level of 120.5 dB peSPL. P1 latency, N1 latency, and P1–N1 amplitude values were analysed accordingly. The NB CE-Chirp stimulus produced significantly shorter P1 and N1 latencies (p < 0.001) with large effect sizes (d > 0.80). In contrast, both stimuli elicited cVEMP responses with P1–N1 amplitude values that were not statistically different from one another (p = 0.157, d = 0.15). Additionally, age and hearing level were found to be significantly correlated (r = 0.56, p < 0.001), as were age and cVEMP amplitude for each stimulus (p < 0.001). To conclude, since both stimuli were presented at an equivalent intensity level (in dB peSPL), the shorter P1 and N1 latencies of cVEMP produced by the NB CE-Chirp stimulus (centred at 500 Hz) were unlikely due to enhanced saccular stimulation. Another more sensible reason is the temporal adjustment of the chirp stimulus. [ABSTRACT FROM AUTHOR]

Chronic myelomonocytic leukemia is a myeloid stem cell disease characterized by an abnormal production and accumulation of monocytic cells in association with other signs of myeloproliferation. Extramedullary manifestations of CMML are common and can affect the spleen, liver skin, and lymph nodes. However, otologic manifestations are extremely rare and could have occurred from either direct leukemic infiltration, hemorrhage of the cochlea, labyrinth, leukostasis, or infection. There is no standard treatment protocol for sensorineural hearing loss in CMML patients. More research is needed to improve the understanding of the pathogenesis of this condition, in order to provide better treatment options. [ABSTRACT FROM AUTHOR]

Introduction: COVID-19 vaccines are essential to prevent complications and reduce the burden of SARS-CoV-2. However, these vaccines showed side effects such as fatigue, pain, fever, and rarely hearing loss. In this review, we aim to summarize studies investigating hearing loss following COVID-19 vaccination and try to find the possible association and risk factors for this hazardous complication. Methods: We performed a comprehensive search of five electronic databases (PubMed, Scopus, Web of Science, google scholar, Cochrane) from inception until 9 October 2022. We finally included 16 studies after the first and second scans. We used SPSS to analyze the extracted data. Results: A total of 630 patients were identified, with a mean age of 57.3. Of the patients, 328 out of 609 vaccinated patients took the Pfizer-BioNTech BNT162b2 vaccine, while 242 (40%) took the Moderna COVID-19 vaccine. The mean time from vaccination to hearing impairment was 6.2, ranging from a few hours to one month after the last dose. The results found a significant difference between vaccine types in terms of incidence and prognosis of the condition, while they showed that the number of doses prior to the onset had no significance. Conclusion: SNHL has been reported in a small number of people who have received the COVID-19 vaccine, but it is unclear at this time whether the vaccine is directly causing this condition. However, the COVID-19 vaccine has been demonstrated to be safe and effective in preventing illness, and the benefits of vaccination are significant compared to any potential risks. Protocol registration: The protocol of this study was registered on Prospero CRD42022367180. [ABSTRACT FROM AUTHOR]

Purpose: To observe vestibular impairment patterns in patients with Ramsay Hunt syndrome with dizziness (RHS_D) and sudden sensorineural hearing loss with dizziness (SSNHL_D) using hierarchical cluster analysis (HCA) to interpret results with possible mechanisms. Methods: The data of 30 RHS_D and 81 SSNHL_D patients from January 2017 to August 2022 in a single tertiary referral center were retrospectively analyzed. Video head impulse test (vHIT) and vestibular evoked myogenic potential (VEMP) were used for vestibular analysis of peripheral vestibular organs, and the results of vHIT and VEMP were analyzed. HCA was used to analyze vestibular impairment patterns. Results: In RHS_D patients, the lateral semicircular canal (LSCC) was the most impaired semicircular canal (SCC), followed by the anterior semicircular canal (ASCC) and the posterior semicircular canal (PSCC), and the utricle was more impaired than the saccule. In SSNHL_D patients, the PSCC was the most impaired SCC, followed by the LSCC and the ASCC, and the utricle was more impaired than the saccule. In HCA of RHS_D patients, the ASCC and utricle were initially clustered, followed by the LSCC, PSCC and saccule in order. In the HCA of SSNHL_D patients, the PSCC was solely merged and independently clustered. Conclusion: There were different patterns of vestibular impairments between RHS_D and SSNHL_D patients. The vestibular analysis and HCA results of SSNHL_D showed tendency of skip lesion, which could be explained by vascular pathophysiology. [ABSTRACT FROM AUTHOR]

The nature of association between chronic otitis media (COM)-mucosal disease and sensorineural hearing loss is controversial. Identifying the risk factors which influence the bone conduction threshold in these patients can help the clinician in counselling the patients for surgery at the earliest to achieve optimum hearing outcomes. The present study was undertaken to determine the association between COM-mucosal disease and cochlear dysfunction. The study also aimed at identifying the determinants and their influence on the bone conduction thresholds of the diseased ear. In this study, 72 patients with unilateral chronic otitis media—mucosal disease were enrolled consecutively. All patients were enquired in detail about their presenting ear symptoms. All the patients underwent a pure tone audiogram in a sound treated room. Patients were categorised into two groups according to the presence of conductive hearing loss only or with a sensorineural component. The bone conduction thresholds were calculated and compared for frequencies at 0.5, 1, 2 and 4 kHz. The average hearing threshold for air conduction and bone conduction were calculated across 0.5, 1 and 2 kHz. Bone conduction threshold more than 20 decibels (dB) in any of the frequencies were considered significant and indicative of having sensorineural hearing loss component. The contralateral healthy ear served as control to cancel out the confounding factors such as presbyacusis, noise induced hearing loss, congenital hearing loss, etc. Multivariate linear regression models were used to evaluate the relationships between bone conduction thresholds and chronic otitis media-mucosal disease. In the present study, 18.05% of participants had a sensorineural component. The difference between the bone conduction threshold in the diseased ear and normal ear ranged from 5.41 dB at 0.5 kHz to 3.77 dB at 4 kHz (p < 0.001). Bone conduction thresholds at 4 kHz were greater than that for speech frequencies (p < 0.5). 84.6% of participants with sensorineural component had a disease duration of less than 5 years and the remaining 15.4% had a duration of greater than 15 years. There was no statistically significant difference in the incidence of sensorineural component based on the site of the perforation (p = 0.341). 21.9% of participants who used topical antibiotic drops developed a sensorineural component, while 15% of participants who did not use antibiotic ear drop preparation developed a sensorineural component. Multivariate linear regression analysis revealed that increasing age was the only factor associated with increase in bone conduction thresholds of the diseased ear (p = 0.002). Chronic otitis media- mucosal disease appears to be associated with higher bone conduction thresholds, signifying cochlear dysfunction. A statistically significant higher bone conduction thresholds are seen across 0.5 to 4 kHz in the diseased ears compared to the normal ears, signifying the vulnerability of the inner ear against chronic otitis media. In our analysis increasing age is the most significant predisposing factor associated with higher bone conduction thresholds. Higher frequencies are more affected than lower speech frequencies. Sensorineural hearing loss can occur early in the disease process and early surgical intervention in COM—mucosal disease is recommended to prevent increase in bone conduction thresholds and achieve optimum hearing outcomes. [ABSTRACT FROM AUTHOR]