BACKGROUND: Wernicke\'s encephalopathy (WE) is an acute neurological syndrome resulting from thiamine (vitamin B1) deficiency. It has been recognized increasingly in non-alcoholic patients, such as in the condition of malnutrition. Recent literature has shed light on uncommon symptoms and neuroimaging findings.
METHODS: We reported a case of a 44-year-old male who initially presented with bilateral hearing loss, and exhibited abnormality in the splenium of the corpus callosum on magnetic resonance imaging (MRI) diffusion-weighted imaging sequence. On the following day the patient developed new symptoms, including unstable walking, double vision and hallucination. The subsequent brain MRI demonstrated lesions involving periaqueductal grey matter and bilateral medial thalamus, indicating the diagnosis of WE. Empirical treatment with intravenous thiamine resulted in complete clinical and radiological resolution.
CONCLUSIONS: To the best of our knowledge, the current case is the first report of WE in literature with uncommon but reversible manifestations. This case warns us to maintain a heightened level of suspicion for WE in malnourished patients with neurological deficits, despite the possibility of atypical presentations encompassing bilateral hearing disturbances and unusual neuroradiological results. Early diagnosis and timely administration of thiamine in WE are likely to lead to a favorable outcome and full recovery.

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OBJECTIVE: We aimed to evaluate the association of occupational noise with metabolic syndrome (MetS) and its components, and to assess the potential role of miRNAs in occupational noise-associated MetS.
METHODS: A total of 854 participants were enrolled in our study. Cumulative noise exposure (CNE) was estimated in conjunction with workplace noise test records and research participants\' employment histories. Logistic regression models adjusted for potential confounders were used to assess the association of CNE and miRNAs with MetS and its components.
RESULTS: We observed linear positive dose-response associations between occupational noise exposure and the prevalence of MetS (OR: 1.031; 95 % CI: 1.008, 1.055). And linear and nonlinear relationship were also found for the association of occupational noise exposure with high blood pressure (OR: 1.024; 95 % CI: 1.007, 1.041) and reduced high-density lipoprotein (OR: 1.051; 95 % CI: 1.031, 1.072), respectively. MiR-200a-3p, miR-92a-3p and miR-21-5p were inversely associated with CNE, or the prevalence of MetS and its components (all P < 0.05). However, we did not find any statistically significant mediation effect of miRNAs in the associations of CNE with MetS. Furthermore, the prevalence of bilateral hearing loss in high-frequency increased (OR: 1.036; 95 % CI: 1.008, 1.067) with CNE level rising, and participants with bilateral hearing loss in high-frequency had a significantly higher risk of MetS (OR: 1.727; 95 % CI: 1.048, 2.819).
CONCLUSIONS: Our study suggests that occupational noise exposure is associated with MetS and its components, and the role of miRNAs in noise-induced increasing MetS risk needs to be confirmed in future studies.

BACKGROUND: Sudden bilateral deafness is often associated with serious systematic conditions such as neoplasms, vascular events, autoimmune diseases, infections, and iatrogenic injury, but very rarely to cerebrovascular disease. This is a rare case of sudden bilateral deafness in a patient with the vertebrobasilar artery occlusion.
METHODS: A 46-year-old man was admitted to a local hospital for sudden bilateral deafness, the patient suffered inarticulate speech and walking unsteadily 6 days later.
METHODS: Difusion-weighted magnetic resonance imagin demonstrated acute cerebral infarction in the pons and bilateral cerebellum; Magnetic resonance angiography showed vertebrobasilar artery occlusion.
METHODS: Aspirin and clopidogrel were given for antiplatelet therapy, revascularization was obtained by endovascular treatment.
RESULTS: The symptoms of dysarthria, ataxia and weakness gradually improved and were discharged 14 days after admission revascularization. After 3 months telephone followed-up the patient was self-cared.
CONCLUSIONS: Deafness sometimes can be an early warning sign of impending vertebrobasilar ischemic stroke. Early recognition of deafness with acute ischemic stroke should allow special management, and misdiagnosis may result in significant morbidity, or even mortality.

BACKGROUND: Wernicke encephalopathy (WE) is a devastating acute or subacute neurological disorder caused by thiamine deficiency. Wernicke encephalopathy is characterized by the triad of ocular signs, cerebellar dysfunction, and confusion. Visual loss and hearing loss are less common findings in WE. Here, we report a case of Wernicke encephalopathy in a nonalcoholic liver cirrhosis patient who presented with acute bilateral deafness and bilateral blindness. CASE PRESENTATION: A 60-year-old Chinese man presented with a history of bilateral blindness and bilateral hypoacousia for 3 days. He had a history of liver cirrhosis and chronic hepatitis C virus infection and did not have a habit of alcohol consumption. Ophthalmologic and otologic examinations showed no obvious abnormalities. MRI findings revealed symmetric fluid-attenuated inversion recovery (FLAIR) hyperintensities in the bilateral medial dorsal thalamus, periventricular region around the third ventricle and tectum, and dorsal medulla oblongata. One day after hospitalization, the patient developed a mild coma. Based on the laboratory and neuroimaging findings, we diagnosed the patient with Wernicke encephalopathy. He soon regained consciousness after administration of thiamine. Both his visual acuity and his hearing function improved gradually.
CONCLUSIONS: We suggest that Wernicke encephalopathy can present with bilateral blindness and bilateral deafness.

OBJECTIVE: This study aims to characterize the hearing benefits and sound localization accuracy of bilateral adhesive bone conduction devices (aBCDs) compared to unilateral devices in patients with congenital bilateral conductive hearing loss (BCHL).
METHODS: Sixteen children and adolescents with congenital BCHL were enrolled and tested under four listening conditions: (1) unaided, (2) R aided: aided with a right-side aBCD, (3) L aided: aided with a left-side aBCD, and (4) B aided: aided with aBCDs on both sides. The sound field hearing threshold (SFHT, in dB hearing level [HL]) and the word recognition score (WRS) were measured. The mean absolute error (MAE) of sound source localization was calculated to assess the sound localization accuracy.
RESULTS: The performance in SFHT and WRS was significantly higher in the B aided condition than that in the unaided, R and L aided conditions; moreover, no significant difference was observed between the R and L aided conditions. Concerning sound source localization, the accuracy of localization exhibited a sharp decline when using a single aBCD, while the application of bilateral aBCDs (B aided condition) resulted in a significantly improved localization accuracy as compared to the unilaterally aided conditions (both R and L); however, no significant difference was found between the unaided and B aided condition.
CONCLUSIONS: Patients with congenital BCHL experienced suboptimal hearing benefits and manifested significant challenges in sound source localization when utilizing a single aBCD, as compared to the utilization of bilateral aBCDs.

Rudimentary otocyst (RO) is characterized by an otic capsule without an internal auditory canal, which is considered a contraindication to cochlear implantation (CI). In this study, we were the first to report two patients with ROs who underwent CI.
Two patients (18 months old and 2 years old) presenting with bilateral congenital hearing loss were diagnosed with ROs.
CI was performed. The transmastoid slotted labyrinthotomy approach was used with customized MED-EL electrode arrays.
Categorical auditory performance, infant-toddler meaningful auditory integration of sound, the speech intelligibility rating, and meaningful use of speech scale.
Both children could understand common phrases and had intelligible, connected speech 2 years after CI.
With proper indication, surgical approach and postoperative training, a child with an RO may benefit from CI.

Long-term noise exposure is reported to damage cardiovascular system, but the relationship between occupational noise exposure and arterial stiffness (AS) and the underlying mechanism is still unclear. We aimed to investigate the association of occupational noise exposure with arterial stiffness (AS), and further explore the mediation roles of microRNAs (miRNAs). A total of 838 workers were recruited from two companies in Wuhan, Hubei, China. Cumulative occupational noise exposure (CNE) was assessed through noise level of job title and work years in occupational noise. The AS for the participants were evaluated using brachial-ankle pulse wave velocity (baPWV) measured by an oscillometric device. Each 1-unit increase in CNE levels was significantly associated with a 0.002 (95% confidence interval (CI) = 0.001-0.003) unit increase in ln-transformed values of baPWV. In the sex-specific analysis, the association was significant in males (β = 0.002, 95%CI = 0.001-0.003). Meanwhile, the risk of bilateral hearing loss at high frequency was significantly higher in the high-exposed group than non-exposed group (OR = 1.895, 95%CI = 1.024-3.508), and participants with bilateral hearing loss at high frequency had a significantly higher level of ln-transformed baPWV (β = 0.032, 95%CI = 0.003-0.061). Occupational noise exposure and AS were both negatively associated with plasma miR-92a-3p and miR-21-5p, and the two miRNAs mediated 15.0% and 16.8% of the association of occupational noise with AS (P < 0.05). Our findings suggest that occupational noise exposure is positively associated with AS, and plasma miR-92a-3p and miR-21-5p may partly mediate such association.

Functional magnetic resonance imaging (fMRI) studies have revealed a functional reorganization in patients with sensorineural hearing loss (SNHL). The structural basement of functional changes has also been investigated recently. Graph theory analysis brings a new understanding of the structural connectome and topological features in central neural system diseases. However, little is known about the structural network connectome changes in SNHL patients, especially in children. We explored the differences in topologic organization, rich-club organization, and structural connection between children with congenital bilateral profound SNHL and normal hearing under the age of three using graph theory analysis and probabilistic tractography. Compared with the normal-hearing (NH) group, the SNHL group showed no difference in global and nodal topological parameters. Increased structural connection strength were found in the right cortico-striatal-thalamus-cortical circuity. Decreased cross-hemisphere connections were found between the right precuneus and the left auditory cortex as well as the left subcortical regions. Rich-club organization analysis found increased local connection in the SNHL group. These results revealed structural organizations after hearing deprivation in congenital bilateral profound SNHL children.

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