Sensorineural Hearing Loss In Patients Research Articles

A Retrospective Comparative Chart Review of Hearing Recovery in Neural and Sensory Type Sudden Sensorineural Hearing Loss Patients.

A Retrospective Comparative Chart Review of Hearing Recovery in Neural and Sensory Type Sudden Sensorineural Hearing Loss Patients.

High Prevalence of Cochlear Nerve Deficiency in Pediatric Patients With Cochlear Aperture Stenosis.

Cochlear nerve deficiency (CND) is a common radiologic finding among unilateral sensorineural hearing loss (USNHL) patients. It is generally detected with magnetic resonance imaging (MRI), which is associated with higher cost, less availability, and possible need for sedation. Therefore, identifying computed tomography (CT) findings, such as cochlear aperture stenosis (CAS), that can reliably predict CND is valuable. Our study aimed to determine the prevalence of CND in pediatric patients with CT-diagnosed CAS. Retrospective study. Tertiary care center. We included pediatric patients diagnosed with CAS on temporal bone CT and with available temporal bone MRI. For each patient, an otolaryngologist and a pediatric neuroradiologist measured the cochlear aperture width on CT to confirm CAS (cochlear aperture < 1.4 mm) and assessed the status of the cochlear nerve on MRI. Fifty-five patients, representing 65 ears, had CAS on CT measurement. Median cochlear aperture width in CAS ears was 0.70 mm (interquartilerange [IQR]: 0.40-1.05 mm) versus 2.00 mm in non-CAS ears (IQR: 1.80-2.30 mm, P < .001). CND was found in 98.5% (n = 64/65) of CAS ears, while a normal cochlear nerve was found in 1.5% (n = 1/65) of CAS ears. CND is highly prevalent among pediatric patients with CAS. This suggests that MRI may not be needed to assess for CND in USNHL patients with CAS, as initial CT may provide sufficient information to determine cochlear implant candidacy. We recommend thoughtful shared decision-making with parents of USNHL patients when determining whether to pursue MRI in the setting of a CAS diagnosis.

Sensorineural hearing loss in patients with type 2 diabetes mellitus: a hospital based cross sectional study

Background: One of the most important of the five senses in human beings is hearing. It plays an important role in the development of speech, cognitive and communication skills. The decrease in length, quality of life and depletion of social networks can be caused by hearing impairment. Therefore, the sense of hearing, perception of sound and its biological purposes, can’t be lightly dismissed. Diabetes mellitus is a common metabolic disorder that causes impairment in various systems of the body. One of the complications of diabetes mellitus is hearing impairment and tinnitus which in turn leads to decreased quality of life in affected individuals. Methods: 75 type 2 diabetes mellitus patients age group of 35 -55 yrs with or without aural symptoms (deafness, tinnitus) were taken into the study. Results: prevalence of sensorineural hearing loss (SNHL) was found 60%. Prevalence of SNHL is lowest among 35-40 age group (12.5%) and highest among 51-55 age group (80.5%). Prevalence of SNHL among the diabetic patients was calculated and it was noted to be 36.8%, 60% and 74% with the duration of diabetes ≤5 yrs, 6-10 yrs, ≥11 yrs. respectively. Conclusions: SNHL is prevalent in 60% of type II diabetic patients.

Comparison of vestibular evoked myogenic potentials between low and high tone idiopathic sudden sensorineural hearing loss

Objectives: Vestibular evoked myogenic potentials (VEMP) have been reported to be useful in evaluating not only vestibular function but also the prognosis of idiopathic sudden sensorineural hearing loss (ISSNHL) patients. Even though low frequency, high frequency, and all frequency-involved ISSNHL groups tend to show varied clinical characteristics, there is a lack of data using VEMP results to analyze these subgroups. We investigated if the VEMP test is a valuable tool to predict recovery from hearing loss in association with different frequencies. Methods: A total of 26 ISSNHL patients were divided into three different groups impaired with low tone (ascending type), high tone (descending type), and all tones (flat type) based on the initial audiograms. Each group included five, 10, and 11 patents, respectively, and their VEMP results were compared between the three subgroups. Results: Abnormal VEMP results were found in five of the total 26 ISSNHL patients (19.2%). Two (40.0%), one (10.0%), and two (18.1%) patients of low tone, high tone, and all tone hearing loss groups, respectively, showed abnormal VEMP results. However, there was no statistically significant difference between the three groups. Conclusions: Even though VEMP is known as a valuable tool for predicting the prognosis of ISSNHL patients, it does not seem to reflect frequency-sensitive aspects of ISSNHL.

A Prospective Comparative Study of Sensorineural Hearing Loss in Patients of Head and Neck Cancers Treated with 2-D RT vs 3-D CRT

Background: SNHL is disabling for the patient, since it has a chronic and often Progressive natural history. sensorineural hearing loss is irreversible, whereas conductive hearing loss (as often found after radiotherapy) is mainly reversible. Material and Methods: This prospective randomized study was conducted in Department of Radiation Oncology, S P medical college Bikaner, Rajasthan. The study was done from November 2020 to October 2021. After taking informed consent total of 50 patients of biopsy proven squamous cell carcinoma of head and neck region randomly distributed among Group A who received 2D RT (2Gy/Fr in 30 to 35 fractions for 6 to 7 weeks) and Group B who received 3D CRT (2Gy/fr) in 30 to 35 fractions for 6 to 7 weeks).Result: After completion of treatment SNHL assessment was done in patients treated with conventional and conformal radiotherapy which was for speech frequency 15% and 8 % respectively and for high frequency was 45% and 28% respectively. After 3 month of completion SNHL for speech frequency 27.5% and 12% respectively and for high frequency was 62.5% and 32% respectively. Conclusion: High-frequency hearing loss can have a significant impact on quality of life because it affects speech discrimination, conventional radiotherapy experienced greater hearing loss as compared of conformal.

Gram‐negative Bacteria are Associated With Sensorineural Hearing Loss in Chronic Otitis Media

ObjectChronic otitis media (COM) is an inflammatory disease that commonly presents with otorrhea and hearing loss. Bacteria‐induced inflammation can cause inner ear damage, leading to sensorineural hearing loss (SNHL). This study aimed to compare the prevalence and severity of SNHL in patients with gram‐negative versus gram‐positive cultures and examine associations between the concentrations of circulating monocytes and neutrophils with bacteria species and SNHL.MethodsThis was a retrospective study. Cholesteatoma or chronic suppurative otitis media patients with otorrhea were enrolled. Middle ear secretions were collected using sterile swabs under an otoscope, and sent for bacterial detection within 30 min. Pure tone audiometry and circulating leukocyte counts were recorded and analyzed in patients infected with different pathogens. Logistic regression analysis was used to identify the risk factors associated with SNHL.ResultsA total of 137 patients were enrolled, including 45 patients infected with gram‐negative bacteria, 41 with gram‐positive bacteria, 20 with polymicrobial infection, and 31 with no bacterial growth. Logistic regression analysis showed that bacterial culture positive infections (OR = 7.265, 95% CI 2.219–23.786, p = 0.001) were an independent risk factor for SNHL. Patients with gram‐negative bacteria had higher risks of SNHL (p &lt; 0.0001) and more severe hearing loss (p = 0.005) than those with gram‐positive bacteria. COM patients infected with gram‐negative bacteria showed an increase in circulating monocytes, which correlated with the occurrence of SNHL (p = 0.0343).ConclusionGram‐negative bacteria are associated with elevated circulating monocyte counts and have a higher risk of severe SNHL.Level of Evidence4 Laryngoscope, 2024

Increased risk of sudden sensorineural hearing loss in patients with cervical spondylosis

Whether cervical spondylosis (CS) is a risk factor for sudden sensorineural hearing loss (SSNHL) remains unclear. This study used national population-based data to investigate the risk of SSNHL in patients with CS in Taiwan of different ages and sexes. This study used data covering 2 million people in Taiwan, which were obtained from the National Health Insurance Research Database. The data that support the findings of this study are available from National Health Insurance Research Database but restrictions apply to the availability of these data, which were used under license for the current study, and so are not publicly available. Data are however available from the corresponding authors upon reasonable request and with permission of National Health Insurance Research Database. This retrospective cohort study enrolled 91,587 patients with a newly diagnosed CS between January 2000 and December 2018. Case and control cohorts were matched 1:1 according to age, sex, and comorbidities. SSNHL incidence rate and risk were compared between the groups. Cox regression was used to estimate hazard ratios (HRs) and 95% confidence intervals (CIs). The mean follow-up period was 8.80 (SD = 4.12) and 8.24 (SD = 4.09) years in the CS and control cohorts, respectively. The incidence rate of SSNHL in the CS cohort (85.28 per 100 000 person-years) was 1.49-fold significantly higher than that in the non-CS cohort (57.13 per 100,000 person-years) (95% CI 1.32–1.68, P < .001). After age, sex, and selected comorbidities were adjusted for, CS exhibited an independent risk factor for SSNHL (adjusted HR = 1.52; 95% CI 1.34–1.71, P < .001). An age-stratified analysis in this study demonstrated a strong and highly significant association between CS and SSNHL in patients aged < 35 years (IRR = 2.28, 95% CI 1.18–4.39, P = .013). This large-scale Taiwanese-population-based retrospective study found that CS was associated with an increased risk of SSNHL. Acute hearing loss in patients with CS, particularly at a young age, should be carefully evaluated, and prompt treatment for SSNHL should be initiated.

Salvage therapy for refractory sudden sensorineural hearing loss (RSSNHL): a systematic review and network meta-analysis.

Approximately 30-50% of sudden sensorineural hearing loss (SSNHL) patients show poor response to systemic steroid therapy. Additionally, the most appropriate treatment for patients with refractory sudden sensorineural hearing loss (RSSNHL) is unknown. This study aimed to explore the best treatment for RSSNHL. Using a frequentist contrast-based model and PRISMA guidelines, this study compared five salvage regimes: intratympanic injection of steroids (ITS), hyperbaric oxygen (HBO) therapy, post auricle steroid injection (PSI), ITS combined with HBO therapy, and continued systemic steroids. We searched the PubMed, EMBASE, Web of Science, and Cochrane Library databases for randomised controlled trials and cohort studies comparing treatment regimens for RSSNHL. Compared with the control group (no additional treatment), PSI and ITS demonstrated significant improvements. The mean hearing gain was greater after PSI (11.1 dB [95% CI, 4.4-17.9]) than after ITS (7.7 dB [95% CI, 4.8-10.7]). When a restricted definition of RSSNHL was used, the ITS + HBO therapy showed the largest difference in improvement for pure tone average compared with the control group (14.5 dB [95% CI, 4.2-25.0]). The administration of either PSI or ITS leads to the greatest therapeutic effect in patients with RSSNHL. However, a consensus on the definition of RSSNHL is needed.

Bidirectional associations between sensorineural hearing loss and depression and anxiety: a meta-analysis.

Recently, the prevalence of sensorineural hearing loss (SNL) has been increasing, and several studies have suggested that depression, anxiety, and SNL may be associated with each other, however, individual findings still have discrepancies. To the best of our knowledge, no scholars have systematically elucidated the bidirectional associations between SNL, depression, and anxiety disorders from the perspective of meta-analysis. In this study, we aimed to systematically evaluate the bidirectional associations between SHL and depressive and anxiety symptoms, and to provide evidence-based medical evidence for reducing SNL, depression, and anxiety disorders. We performed systematic review based on priori protocol that was registered with PROSPERO (No. CRD42022365963). Systematic search of PubMed, Embase, and Web of Science databases identified articles published as of June 1, 2023, on the relationship between SNL and depression and anxiety. Meta-analysis was performed to calculate the odds ratios (OR) and 95% confidence intervals (CIs) for the outcome metrics, and the results were combined to assess bivariate associations between the disorders with fixed or random effects. Sensitivity and subgroup analyzes were conducted to analyze sources of heterogeneity, and Egger's and Begg's tests combined with funnel plots were applied to assess publication bias. Summary analysis of the results of 20 studies covering 675,291 individuals showed that the bidirectional association between SNL and depression and anxiety disorders. The incidence (OR = 0.17, 95% CI: 0.09-0.28) and risk (OR = 1.43, 95% CI: 1.32-1.55) of depression and morbidity were higher in SNL patients than the general population. Elevated prevalence (OR = 0.46, 95% CI: 0.28-0.65) and risk (OR = 1.30, 95% CI: 1.11-1.48) of SNL were also observed in depressed patients. The prevalence of anxiety disorders among SNL patients was about 40% (OR = 0.40, 95% CI: 0.24%-0.57), which was associated with higher risk (OR = 1.83, 95% CI: 1.42-2.24) of development than the general population. Incidence of SNL in patients with anxiety disorders was approximately 31% (OR = 0.31, 95% CI: 0.29-0.33). Additionally, subgroup analyzes showed that the bidirectional associations between SNL, depression, and anxiety disorders was influenced by age, region, and mode of diagnosis of the disorders (SNL, depression, anxiety). There are bidirectional associations between SNL and depression and anxiety disorders, which was influenced by age and region and the method the disorders (SNL, depression, anxiety) were diagnosed.

Prevalence of perilymphatic fistula in patients with sudden-onset sensorineural hearing loss as diagnosed by Cochlin-tomoprotein (CTP) biomarker detection: its association with age, hearing severity, and treatment outcomes

PurposeTo 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.MethodsA 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.ResultsCTP 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.ConclusionThis 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.

DNA methylation in hearing-related genes in non-syndromic sensorineural hearing loss patients

BackgroundOur understanding of epigenetic modifications in the inner ear is very limited. Although epigenetic regulation of genes related to individual organ- and system-limited pathologies are generally expected to be tissue-specific, DNA methylation patterns in peripheral blood (PB) are found to be associated with the presence of several diseases with no typical hematological involvement. Here, we aimed to investigate whether there is a correlation between hearing-related genes’ promoter region methylation in the PB samples with the presence of non-syndromic sensorineural hearing loss (NSSHL) with an aim of future utilization of DNA methylation as biomarkers in hearing loss. The study included 26 patients with NSSHL and a control group of 20 healthy individuals. CpG islands in the promoter regions of the GJB-2, GJB-6, and SLC24A genes were analyzed using bisulfite sequencing, and methylation percentages were analyzed.ResultsMethylation levels at the 1st region of GJB-6 and the 1st and the 4th regions of SLC26A4 were found to differ significantly (p = 0.039, p = 0.042, and p = 0.029, respectively) between the patients and the control group. There was no statistically significant difference in methylation percentages of GJB-2 promoters. We also found that parents’ consanguinity determines the methylation levels in patients’ families.ConclusionsAccording to our knowledge, this is the first study that investigates epigenetic changes in the PB of patients with NSSHL. Despite the small sample size, our findings indicate that DNA methylation patterns in the PB could be of use for understanding epigenetic changes in the inner ear and the clinical management of NSSHL.

Expanding Genotype-Phenotype Correlation of CLCNKA and CLCNKB Variants Linked to Hearing Loss.

The ClC-K channels CLCNKA and CLCNKB are crucial for the transepithelial transport processes required for sufficient urinary concentrations and sensory mechanoelectrical transduction in the cochlea. Loss-of-function alleles in these channels are associated with various clinical phenotypes, ranging from hypokalemic alkalosis to sensorineural hearing loss (SNHL) accompanied by severe renal conditions, i.e., Bartter's syndrome. Using a stepwise genetic approach encompassing whole-genome sequencing (WGS), we identified one family with compound heterozygous variants in the ClC-K channels, specifically a truncating variant in CLCNKA in trans with a contiguous deletion of CLCNKA and CLCNKB. Breakpoint PCR and Sanger sequencing elucidated the breakpoint junctions derived from WGS, and allele-specific droplet digital PCR confirmed one copy loss of the CLCNKA_CLCNKB contiguous deletion. The proband that harbors the CLCNKA_CLCNKB variants is characterized by SNHL without hypokalemic alkalosis and renal anomalies, suggesting a distinct phenotype in the ClC-K channels in whom SNHL predominantly occurs. These results expanded genotypes and phenotypes associated with ClC-K channels, including the disease entities associated with non-syndromic hearing loss. Repeated identification of deletions across various extents of CLCNKA_CLCNKB suggests a mutational hotspot allele, highlighting the need for an in-depth analysis of the CLCNKA_CLCNKB intergenic region, especially in undiagnosed SNHL patients with a single hit in CLCNKA.

Pregnant Patients with Sudden Sensorineural Hearing Loss: Treatments and Efficacy.

The aim of this study was to study the safety and effectiveness of oral and tympanic hormone injection in the treatment of sudden sensorineural hearing loss during pregnancy. Data were collected via prospective method. A total of 102 pregnant women with sensorineural hearing loss as experimental group and another 102 patients of sensorineural hearing loss without pregnancy as control group were simultaneously included in the study. Pure tone audiometry test was examined at pre- and posttreatment in 1 week, 2 weeks, and 12 weeks. The experimental group received oral and tympanic hormones, while the control group was treated with the Clinical Practice Guideline: Sudden Hearing Loss (2019) of USA. Recovery rate and hearing gain were assessed by the Clinical Practice Guidelines. After treatment, the effects of the experimental group and the control group were compared at the 1st, 2nd, and 12th week after treatment. It was found that at the 12th week after treatment, the curative effect of the experimental group was significantly different from that of the control group, and the difference was statistically significant. The pregnant women with sensorineural hearing loss were more serious than nonpregnant women, and the treatment efficacies were worse than control group. For pregnancy patients with sudden deafness, oral steroids and tympanic cavity injection is an effective, safe first-line treatment options.

Impairment of auditory function in persons with type 2 diabetes mellitus depending on the level of peripheral myelin protein 22

Background. One of the priority tasks of modern medicine is early diagnosis and treatment of micro- and macrovascular complications of diabetes mellitus (DM) which cause a decrease in the quality of life of working age people and an increase in mortali­ty. Variability of carbohydrate metabolism indicators, metabolic dysfunction are leading factors in the development and progression of disabling complications of type 2 DM with hearing loss due to degenerative changes in nerve fibers. An important aspect of diagnostic and treatment algorithms of sensorineural hearing disorders in patients with type 2 DM is early detection of demyelinating processes to prevent damage to neuronal structures. The purpose of the study is to determine the relationship between the level of peripheral myelin protein 22 (PMP 22) and the condition of the auditory analyzer according to the data of instrumental examination in patients with type 2 DM combined with hearing impairment. Materials and methods. There were examined 30 patients with type 2 DM (15 men and 15 women aged 56.28 ± 4.54 years) associated with degree I–II sensorineural disorders of the auditory function; 15 people with signs of sensorineural hearing loss (SNHL) (8 men, 7 women aged 59.33 ± 2.65 years) without diabetes and 15 controls. During the examination, the patients with type 2 DM and SNHL, depending on the severity of the underlying disease, were divided into groups: the first group (n = 15) with or without isolated microvascular complications of type 2 DM, the second group (n = 15) with neuro-, nephro- and retinopathy. All patients underwent a set of laboratory tests to determine the content of PMP 22 in the blood serum, clinical and instrumental diagnosis of the auditory function. The obtained data were processed statistically, and correlations were identified. Results. The analysis of the results revealed that in patients with type 2 DM and SNHL (groups 1 and 2), the level of peripheral myelin protein 22 significantly increased compared to both controls and the comparison group, and more significantly in those with microangiopathies. The correlations were found bet­ween the most informative indicators reflecting the state of various structures of the hearing analyzer and the content of PMP 22 in the blood serum. Conclusions. A significant increase was revealed in the content of PMP 22 and manifestations of sensorineural hearing loss in patients with type 2 diabetes mellitus associated with microangiopathies that indicate demyelinating processes in the neural structures of the auditory analyzer.

Postmeningitic pediatric hearing loss from non-type b Haemophilus influenzae

BackgroundPostmeningitic hearing loss from Haemophilus influenzae (H. influenzae) is increasingly due to encapsulated serotypes other than type b (Hib) and nontypeable strains (collectively, nHiB H. influenzae). Pediatric hearing loss after nHib H. influenzae meningitis remains poorly described. MethodsRetrospecive case series of nHiB H. influenzae meningitis cases identified from a microbiologic database at Children's Hospital Colorado from 2000 to 2020. Literature regarding nHiB H. influenzae and H. influenzae postmeningitic hearing loss was also reviewed. ResultsEleven cases of nHib H. influenzae meningitis (median age 15.9 months) were identified due to serotype f (36 %), serotype a (27 %), and nontypable strains (36 %). Seven (64 %) patients were male, 55 % were white and 18 % were Hispanic or Latino. Hearing loss was initially identified in 4 children (40 %), with two patients with moderate conductive hearing loss (CHL) and one child with unilateral moderate sensorineural (SNHL) hearing loss patients recovering normal hearing. One patient with bilateral profound sensorineural hearing loss and associated labyrinthitis ossificans required cochlear implantation. All children (4) with identified hearing loss were noted to have additional intracranial sequelae, which included empyema (2), sinus thrombosis (2), and seizures (2). Of patients receiving steroids, 25 % had hearing loss on initial testing, compared to 66 % of those who did not receive steroids. ConclusionsnHib H. influenzae can cause both transient and permanent postmeningitic hearing loss. Steroids may offer otoprotection in nHib H. influenzae meningitis similar to Hib meningitis. Given the limited literature, further study is needed to better characterize hearing outcomes after nHib H. influenzae meningitis.

Sensorineural Hearing Loss in Patients With Chronic Kidney Disease: A Comprehensive Review.

This article aims to ascertain the prevalence of loss of hearing in patients with chronic kidney disease (CKD) and also to examine potential causes of sensorineural hearing loss (SNHL) in patients suffering from CKD. It has been discovered in recent years that there is a relationship between the occurrence of SNHL and CKD. Nowadays many people are suffering from CKD. These patients deal with several otorhinolaryngological issues, such as SNHL, candidiasis, epistaxis, halitosis, dysgeusia, xerostomia, and lip and thyroid malignancies. One of the most frequent otorhinolaryngological complications is audiovestibular system impairment. There are various proposed mechanisms for the appearance of loss of hearing in people suffering from CKD. The kidney and the inner ear have multiple functional and structural similarities, which may be the cause of these problems in CKD patients. In addition, changes in the homeostasis of water and electrolytes can affect the endolymphatic fluid and result in endolymphatic hydrops. Finally, some medications, like aminoglycosides and loop diuretics, are well known for their ototoxicity and are utilized to treat patients with CKD. Only a small number of population-based research have so far been able to show a connection between CKD and audiovestibular system impairment. Some investigationhasshown that CKD patients are more likely than healthy people to experience vestibular impairment. The quality of life of a patient can be reduced by hearing loss. People with hearing loss experience communication issues in daily life, which negatively affects their cognitive and psychosocial functioning. Social isolation and a poor quality of life in terms of health can all result from hearing loss. In addition, decreased renal function has also been linked to poor quality of life, hospitalization, and cognitive dysfunction.

Functional to structural plasticity in unilateral sudden sensorineural hearing loss: neuroimaging evidence

A cortical plasticity after long-duration single side deafness (SSD) is advocated with neuroimaging evidence while little is known about the short-duration SSDs. In this case-cohort study, we recruited unilateral sudden sensorineural hearing loss (SSNHL) patients and age-, gender-matched health controls (HC), followed by comprehensive neuroimaging analyses. The primary outcome measures were temporal alterations of varied dynamic functional network connectivity (dFNC) states, neurovascular coupling (NVC) and brain region volume at different stages of SSNHL. The secondary outcome measures were pure-tone audiograms of SSNHL patients before and after treatment. A total of 38 SSNHL patients (21 [55%] male; mean [standard deviation] age, 45.05 [15.83] years) and 44 HC (28 [64%] male; mean [standard deviation] age, 43.55 [12.80] years) were enrolled. SSNHL patients were categorized into subgroups based on the time from disease onset to the initial magnetic resonance imaging scan: early- (n = 16; 1-6 days), intermediate- (n = 9; 7-13 days), and late- stage (n = 13; 14-30 days) groups. We first identified slow state transitions between varied dFNC states at early-stage SSNHL, then revealed the decreased NVC restricted to the auditory cortex at the intermediate- and late-stage SSNHL. Finally, a significantly decreased volume of the left medial superior frontal gyrus (SFGmed) was observed only in the late-stage SSNHL cohort. Furthermore, the volume of the left SFGmed is robustly correlated with both disease duration and patient prognosis. Our study offered neuroimaging evidence for the evolvement from functional to structural brain alterations of SSNHL patients with disease duration less than 1 month, which may explain, from a neuroimaging perspective, why early-stage SSNHL patients have better therapeutic responses and hearing recovery.

Abnormal outer hair cell efferent innervation in Hoxb1-dependent sensorineural hearing loss.

Autosomal recessive mutation of HOXB1 and Hoxb1 causes sensorineural hearing loss in patients and mice, respectively, characterized by the presence of higher auditory thresholds; however, the origin of the defects along the auditory pathway is still unknown. In this study, we assessed whether the abnormal auditory threshold and malformation of the sensory auditory cells, the outer hair cells, described in Hoxb1null mutants depend on the absence of efferent motor innervation, or alternatively, is due to altered sensory auditory components. By using a whole series of conditional mutant mice, which inactivate Hoxb1 in either rhombomere 4-derived sensory cochlear neurons or efferent motor neurons, we found that the hearing phenotype is mainly reproduced when efferent motor neurons are specifically affected. Our data strongly suggest that the interactions between olivocochlear motor neurons and outer hair cells during a critical postnatal period are crucial for both hair cell survival and the establishment of the cochlear amplification of sound.

Variation in cochlear size: A systematic review.

Advancements in imaging and implantation technology have invited reexamination of the classic teaching that the human cochlea maintains uniform size across demographics. Yet, studies yield conflicting results and relatively few broad systematic reviews have examined cochlear size variation. The purpose of this study is to quantify cochlear variability across eight different measurement categories and suggest normative values and ranges for each with consideration of disease state and gender where possible. A systematic search was conducted up to October 1, 2022, using the search terms "Cochlea/anatomy and histology"[Mesh]) AND 'size'" with filters "Humans" and "English" across three databases (PubMed, CINAHL, Medline). Further inclusion criteria involved reporting of numerical measurements in any of the eight included categories. Of the 625 articles manually reviewed for relevance by title and abstract, 91 were selected for full-text review and 33 met all eligibility criteria. 5,791 cochleae were included and weighted means and ranges were calculated: "A" value (defined as the distance from the round window, through the modiolus, to the oppsite lateral wall)=9.23mm (8.43-10.4mm, n=2559); cochlear duct length (CDL)=33.04mm (range 28.2-36.4mm, n=2252); cochlear height=5.14mm (2.8-6.9mm, n=2098); the basal turn lumen diameter=2.09mm (1.7-2.2mm, n=617); "B" value (defined as perpendicular to "A" value and in the same plane)=6.52mm (5.73-6.9mm, n=908); width of the basal turn=6.4mm (6.22-6.86mm, n=356); height of the basal turn=1.96mm (1.77-2.56mm, n=204); length of the basal turn 21.87mm (21.03-22.5mm, n=384). A notable size range exists across the eight different cochlear parameters considered and we provide normative values for each measurement. Females tend to have smaller CDL and "A" value than males and the sensorineural hearing loss patients had smaller CDL and "A" value but larger cochlear height than the general population.

Cardiocerebrovascular risk in sensorineural hearing loss: results from the National Health and Nutrition Examination Survey 2015 to 2018.

This study aims to determine whether the risks of cardiocerebrovascular disease are relevant to sensorineural hearing loss (SNHL) based on a national database. A total of 1,321 participants aged from 18 to 69 with complete data including medical history and audiometry from the NHANES database (2015-2018) were analyzed. All included participants had available hearing data and the average thresholds of the hearing data were measured and calculated as low-frequency pure-tone average (LFPTA; 500, 1,000, and 2,000 Hz) and high-frequency pure-tone average (HFPTA; 3,000, 4,000, 6,000, and 8,000 kHz). SNHL was defined as an average pure tone of more than or equal to 20 dB in at least one better ear. Multivariable models to assess the association between cardiocerebrovascular risks and SNHL were used in this study. The prevalence of stroke was 1.6% in individuals with SNHL and 0.4% in individuals without SNHL (p = 0.023). A higher cardiovascular risk score was observed in SNHL patients compared to participants without SNHL (1.58 vs. 0.90, p < 0.001). Stroke was associated with a 3.67-fold increase in the risk of SNHL (95% CI: 1.12-12.00, p = 0.032) in univariable logistic regression, and the association (OR = 4.22, 95%CI = 1.28-13.93, p = 0.020) remained significant after adjusting for several covariates. Multivariable logistic regression models indicated a positive correlation between cardiovascular risk and SNHL (OR = 1.66, 95% CI = 1.40-1.96, p < 0.001), but no significant relationship was shown with all covariates adjusted. However, significant associations were found between SNHL and both age and sex in both univariable and multivariable logistic regression models. Our findings suggested that a higher cardiocerebrovascular risk burden was associated with an increased risk of SNHL, and the relationship may be influenced by age and sex. Future longitudinal studies are needed to investigate the mechanistic and pathologic vascular hypothesis of SNHL.