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Auditory neuropathy spectrum disorder (ANSD) represents a variety of sensorineural deafness conditions characterized by abnormal inner hair cells and/or auditory nerve function, but with the preservation of outer hair cell function. ANSD represents up to 15% of individuals with hearing impairments. Through mutation screening, bioinformatic analysis and expression studies, we have previously identified several apoptosis-inducing factor (AIF) mitochondria-associated 1 (AIFM1) variants in ANSD families and in some other sporadic cases. Here, to elucidate the pathogenic mechanisms underlying each AIFM1 variant, we generated AIF-null cells using the clustered regularly interspersed short palindromic repeats (CRISPR)/CRISPR-associated protein 9 (Cas9) system and constructed AIF-wild type (WT) and AIF-mutant (mut) (p.‍T260A, p.‍R422W, and p.‍R451Q) stable transfection cell lines. We then analyzed AIF structure, coenzyme-binding affinity, apoptosis, and other aspects. Results revealed that these variants resulted in impaired dimerization, compromising AIF function. The reduction reaction of AIF variants had proceeded slower than that of AIF-WT. The average levels of AIF dimerization in AIF variant cells were only 34.5%‍‒‍49.7% of that of AIF-WT cells, resulting in caspase-independent apoptosis. The average percentage of apoptotic cells in the variants was 12.3%‍‒‍17.9%, which was significantly higher than that (6.9%‍‒‍7.4%) in controls. However, nicotinamide adenine dinucleotide (NADH) treatment promoted the reduction of apoptosis by rescuing AIF dimerization in AIF variant cells. Our findings show that the impairment of AIF dimerization by AIFM1 variants causes apoptosis contributing to ANSD, and introduce NADH as a potential drug for ANSD treatment. Our results help elucidate the mechanisms of ANSD and may lead to the provision of novel therapies.

A non-noble-metal modulation strategy has been developed to adjust the electronic structure of Pd, resulting in enhancement of the catalytic performance for the dehydrogenation of formic acid.

Genetic screening can benefit early detection and intervention for hearing loss. The frequency of common deafness-associated variants in general populations is highly important for genetic screening and genetic counseling tailored to different ethnic backgrounds. We aimed to analyze the frequency of common deafness-associated variants in a large population-based Chinese newborn cohort and to explore the population-specific features in diverse populations worldwide.This population-based cohort study analyzed the frequency of common deafness-associated variants in 3,555,336 newborns in the Chinese Newborn Concurrent Hearing and Genetic Screening cohort. Participants were newborn infants born between January 2007 and September 2020. Limited genetic screening for 20 variants in 4 common deafness-associated genes and newborn hearing screening were offered concurrently to all newborns in the Chinese Newborn Concurrent Hearing and Genetic Screening cohort. Sequence information of 141,456 individuals was also analyzed from seven ethnic populations from the Genome Aggregation Database for 20 common deafness-related variants. Statistical analysis was performed using R.A total of 3,555,326 Chinese neonates completed the Newborn Concurrent Hearing and Genetic Screening were included for analysis. We reported the distinct landscape of common deafness-associated variants in this large population-based cohort. We found that the carrier frequencies of GJB2 , SLC26A4 , GJB3 , and MT-RNR were 2.53%, 2.05%, 0.37%, and 0.25%, respectively. Furthermore, GJB2 c.235delC was the most common variant with an allele frequency of 0.99% in the Chinese newborn population. We also demonstrated nine East-Asia-enriched variants, one Ashkenazi Jewish-enriched variant, and one European/American-enriched variant for hearing loss.We showed the distinct landscape of common deafness-associated variants in the Chinese newborn population and provided insights into population-specific features in diverse populations. These data can serve as a powerful resource for otolaryngologists and clinical geneticists to inform population-adjusted genetic screening programs for hearing loss.

Imatinib mesylate (IM) is highly effective in the treatment of gastrointestinal stromal tumors (GISTs). However, the most of GISTs patients develop secondary drug resistance after 1-3 years of IM treatment. The aim of this study was to explore the IM-resistance mechanism via the multi-scope combined with plasma concentration of IM, genetic polymorphisms and plasma sensitive metabolites.This study included a total of 40 GISTs patients who had been regularly treated and not treated with IM. The plasma samples were divided into three experiments, containing therapeutic drug monitoring (TDM), OCT1 genetic polymorphisms and non-targeted metabolomics. According to the data of above three experiments, the IM-resistant cell line, GIST-T1/IMR cells, was constructed for verification the IM-resistance mechanism.The results of non-targeted metabolomics analysis suggested that the sphingophospholipid metabolic pathway including the SPK1/S1P axis was inferred in IM-insensitive patients with GISTs. A GIST cell line (GIST-T1) was immediately induced as an IM resistance cell model (GIST-T1/IMR) and we found that blocking the signal pathway of SPK1/S1P in the GIST-T1/IMR could sensitize treatment of IM and reverse the IM-resistance.Our findings suggest that IM secondary resistance is associated with the elevation of S1P, and blockage the signaling pathway of SPK1/S1P warrants evaluation as a potential therapeutic strategy in IM-resistant GISTs. The design of this study from blood management, group information collection, IM plasma concentration with different elements, identification of sphingolipid metabolism and lastly verification the function of SPK1/S1P in the IM-resistance GISTs cells.

The positive externalities of green producers usually reduce the company's earnings. Whether the markets give sufficient premium is important. Sampling the data of listed companies from May 2005 to April 2017 in Shanghai and Shenzhen A-share main-board markets, we construct 12 portfolios based on market factor RMRF, scale factor SMB, book-to-market factor HML and green factor GF. Results show: 1) SMB premium is significant positive, while HML is negative; 2) For green concept stocks, HML has a significant positive impact; 3) Portfolio with non-green concept stocks has a higher return; 4) GF has a significant negative risk premium on China’s green concept stocks, and the premium level will decrease as the book-to-market ratio increases. The interpretation of the above premium anomalies improves national environmental protection policies which is of great significance for the formation of a sound environmental protection industry. Keywords: Factor Model; Green Finance; Premium Anomalies; Excess Return; Environmental Protection.

The purpose of this study was to investigate the effect of Bacillus amyloliquefaciens LSG2-8 on the growth performance, immune function, antioxidant capacity, and disease resistance of Rhynchocypris lagowskii. Fish were fed with the feed containing five levels such as 0, 1.0 × 10

Tumor relapse and drug resistance are major factors that limit the curability of multiple myeloma (MM). New regimens have improved overall MM survival rates, but patients with high-risk features continue to have inferior outcomes. Chromosome 17p13 deletion (del17p) which includes the loss of the TP53 gene is a high-risk cytogenetic abnormality and is associated with poor clinical outcomes due to relatively short remissions and the development of pan-drug resistant disease. Increased relapse rates suggest that del17p enhances clonogenic growth, and we found that the loss of p53 increased both the frequency and drug resistance of tumor-initiating MM cells (TICs). Subsequent RNA sequencing (RNA-seq) studies demonstrated significant activation of the Notch signaling pathway and upregulation of inhibitor of DNA binding (ID1/ID2) genes in p53-knock out (p53-KO) cells. We found that the loss of ID1 or HES-1 expression or treatment with a gamma-secretase inhibitor (GSI) significantly decreased the clonogenic growth of p53-KO, but not p53 wild-type cells. GSI treatment in a small set of MM specimens also reduced the clonogenic growth in del17p samples but not in non-del17p samples. This effect was specific as overexpression of the Notch intracellular domain (NICD) rescued the effects of GSI treatment. Our study demonstrates that the Notch signaling and ID1 expression are required for TIC expansion in p53-KO MM cells. These findings also suggest that GSI may be specifically active in patients with p53 mutant MM.

The Hedgehog (Hh) signaling pathway is aberrantly activated in a wide variety of human cancers, and recent clinical studies have demonstrated that pathway inhibitors are effective in advanced basal cell carcinoma (BCC). The majority of these agents have been designed to target SMOOTHENED (SMO), a transmembrane regulator of Hh signaling, but subsequent mutations in SMO have been found to generate drug resistance. In other cancers, oncogenic events that bypass SMO may activate canonical Hh signaling, and SMO antagonists have not demonstrated significant activity in several diseases. Therefore, alternative strategies targeting the Hh pathway downstream of SMO may have clinical utility. Liver X receptors (LXR) regulate cholesterol and fatty acid homeostasis, and LXR activation can inhibit the Hh pathway in normal mouse embryonic fibroblasts. We examined the effects of LXR activation on Hh signaling in human multiple myeloma cells and found that LXR agonists inhibited Hh pathway activity and clonogenic tumor growth in vitro. LXR activation also inhibited putative multiple myeloma cancer stem cells in vivo leading to the loss of tumor initiating and self-renewal potential. Finally, Hh signaling was inhibited downstream of SMO, suggesting that LXR agonists may represent a novel strategy to target pathogenic Hh signaling as well as treat multiple myeloma. Mol Cancer Ther; 13(7); 1873–81. ©2014 AACR.

Supplemental Figure 1. IQGAP1 levels correlate with disease progression and a poor prognosis;Supplemental Figure 2. IQGAP1 levels correlate with RAS pathway activation gene sets;Supplemental Figure 3. The impact of IQGAP1 WW peptide on cell cycle is reversible.

S1. Ezrin expression in PDAC CSCs. S2. Effects of Ezrin on self-renewal and migration in PDAC cells. S3. Actin dynamics following Ezrin knock-down and in low-passage PDAC xenografts. S4. Actin dynamics in L3.6pl cells. S5. Treatment of PDAC cells with NSC305787. S6. Effects of Ezrin loss on in vivo tumor initiation.

PDF - 177K, Figure S1. LXR expression in myeloma cell lines. Figure S2. Induction of LXR target genes by non-steroidal LXR agonists. Figure S3. Effects of LXR agonists on clonogenic myeloma growth. Figure S4. Impact of T0901317 on myeloma cell growth and survival. Figure S5. Dose-response relationships of T0901317 in U266 cells. Figure S6. Gene knock-down by siRNA. Figure S7. Effects of LXR agonists on myeloma tumor initiating cells. Figure S8. In vivo activation of LXR target genes.

KCNQ4 gene mutation can lead to deafness non-syndromic autosomal dominant 2A, which is a type of autosomal dominant non-syndromic hearing loss. Deafness non-syndromic autosomal dominant 2A patients with KCNQ4 gene mutation usually present with symmetrical, delayed, progressive high-frequency-affected hearing loss, which eventually can involve all frequencies. In this article, we comprehensively reviewed the research on the role and function of KCNQ4 gene in genetic hearing loss. We discussed the pathological and physiological mechanisms of KCNQ4 gene and the related clinical phenotypes of KCNQ4 gene mutations. We also reviewed the latest developments in the treatment of KCNQ4 gene mutation-related genetic hearing loss, including selective potassium channel activation drugs and gene therapy.

Preimplantation genetic diagnosis (PGD) uses molecular biological techniques to genetically diagnose embryos before in vitro fertilization. The information obtained through PGD can help clinicians select healthy embryos for implantation, prevent the transmission of inherited diseases and help affected families have healthy children. This paper reviews the development of PGD technology, the history of its application to hereditary hearing loss, and the general process of how PGD is applied to screen for hereditary hearing loss. The aim of this review is to demonstrate the reliability of PGD in the primary prevention of hereditary hearing loss, assist clinicians in counseling patients at risk of transmitting an inherited disease, and explore the journey from PGD to in vitro fertilization. Given that the application of PGD technology to hereditary hearing loss varies in different countries and regions, there is still a long way to go before PGD is routinely applied for the primary prevention of hereditary hearing loss.

Objective To investigate the genetic causes of sudden sensorineural hearing loss (SSNHL) patients in China. This study focused on analyzing variations of coding sequence of common genes related to deafness, revealing the molecular pathogenesis of sudden deafness from a genomics perspective, discovering molecular markers associated with the onset of deafness, and then supplying prevention to high-risk populations, classifying disease according to accurate etiology, and choosing a much more precision therapy. Methods We retrospectively analyzed the clinical characteristics of 51 patients diagnosed as SSNHL with vertigo treated in the Chinese PLA General Hospital. In this study, mutation screening of 307 nuclear genes and mitochondrial genome responsible for human or mouse deafness was performed on the 51 cases of unilateral sudden deafness patients with vertigo. Results We identified 51 cases of unilateral sudden deafness, including 2 cases of low-mid frequency hearing impairment, 18 cases of mid-high frequency hearing loss, 11 cases of flat-type hearing loss, and 20 cases of all frequency hearing loss. Among the 51 cases, 8 (15.69%) cases of GJB2 heterozygous variations, 1 (1.96%) case of GJB3 heterozygous variations, 5 (9.8%) cases of SLC26A4 heterozygous variations, 2 (3.92%) cases of COCH heterozygous variations, 14 (27.45%) cases of CDH23 heterozygous variations, 14 (27.45%) cases of OTOF heterozygous variations, 1 (1.96%) case of SLC17A8 heterozygous variations and 2 (3.92%) cases of KCNE1 heterozygous variations. No mtDNA gene variations were identified. Conclusion SSNHL has some relationship with hereditary in Chinese population, but its complex genetic pathogenic mechanisms need further study.

OBJECTIVE: To provide accurate genetic counseling, the genotype-phenotype correlation of the patients with KCNQ4 mutations was analyzed. METHODS: Two hearing loss families, 1807956(a five-generation family with 34 members) and 1707806(a three-generation family with 12 members) were recruited. The candidate variants were detected by next generation sequencing technology. Sanger sequencing was performed to verify the co-segregation of the phenotype in the recruited family members. According to American College of Medical Genetics and Genomics(ACMG) guideline, combined with clinical data, genetic testing, bioinformatic analysis and electrophysiological experiments, the pathogenicity of mutations was analyzed and genetic counseling was provided for family members. RESULTS: The proband of family 1807956 was a pregnant woman, who carried KCNQ4 c.808T>G p.Y270D and developed hearing loss at the age of 15 years old, she had profound hearing loss in both ears, with middle-frequency highly affected. The proband of family 1707806 was an adolescent whose onset age was 11 years old, carrying KCNQ4 c.733G>A p.G245R, he presented with bilateral moderately severe hearing loss. The inheritance pattern of these two families were autosomal dominant inheritance. The two variants were missense mutations that were co-segregation in the two families and were not found in normal population. The mutations predicted by bioinformatic analysis tools were damaging and highly conserved in different species. Electrophysiological experiments showed that the function of the mutant ion channels was impaired. According to ACMG guideline, KCNQ4 c.808T>G was pathogenic, and KCNQ4 c.733G>A was likely pathogenic. CONCLUSION: The two mutations in this research were reported for the first time. The hearing loss of the patients showed heterogeneity, enriching the variation spectrum and clinical phenotype of KCNQ4.

The widely available crop oil is an effective alternative to the increasingly scarce marine fish oil. However, simple alternative strategies have led to declining growth and the edible value of farmed fish. It is worthwhile to explore the effects of micro supplements in diets to improve the tolerance of fish to different dietary lipid sources, which finally optimizes the feeding strategies. This study aimed to investigate the regulation of L-carnitine and dietary oil conditions on nutrient composition, lipid metabolism, and glucose regulation of Rhynchocypris lagowskii. Four diets were prepared according to fish oil, fish oil supplemented with L-carnitine, corn oil, and corn oil supplemented with L-carnitine, and FO, LCFO, CO, and LCCO were labeled, respectively. R. lagowskii was fed experimental diets for 8 weeks, and the glucose tolerance test was performed. The CO diet significantly resulted in higher crude lipid content in muscle but a lower level of serum lipid parameters of R. lagowskii than the FO diet. However, dietary L-carnitine supplementation significantly reduced the crude lipid content in the hepatopancreas and muscle of the fish fed with the CO diet yet increased the serum lipid parameters. Additionally, the crude lipid content of muscle was reduced in the fish fed with an FO diet supplemented with L-carnitine. Compared with the FO diet, the CO diet significantly reduced the ratio of n3/n6 polyunsaturated fatty acid in the hepatopancreas and muscle of R.lagowskii. Dietary L-carnitine supplementation significantly reduced the contents of total saturated fatty acids and total monounsaturated fatty acids in hepatopancreas under both dietary lipid sources. The CO diet significantly up-regulated the expression of genes related to lipid uptake and adipogenesis in hepatopancreas, including lipoprotein lipase (lpl), acetyl-coenzyme A carboxylase alpha (accα), and sterol regulatory element binding protein-1 (srebp1), compared with the FO diet. While dietary L-carnitine supplementation significantly down-regulated the expressions of lpl, accα, srebp1, and fatty acid synthase in hepatopancreas and muscle of fish under both dietary lipid sources, along with up-regulated expression of carnitine palmitoyltransferase 1 in hepatopancreas. Moreover, the fish fed with a CO diet significantly increased the expression of glucose uptake and clearance and significantly down-regulated the expressions of glucose regulation-related genes, including glucose transporter 1, glycogen synthase 1, and phosphofructokinase in hepatopancreas and muscle, resulting in slower glucose uptake and clearance than fish fed with FO diet. Nevertheless, dietary L-carnitine supplementation up-regulated the expression of gluconeogenesis-related genes, including glucose-6-phosphatase and phosphoenolpyruvate carboxykinase in the hepatopancreas of R. lagowskii under both dietary lipid sources. In conclusion, a higher dietary n6 PUFA resulted in lipid deposition, decreased serum lipid parameters, and limited serum glucose utilization of R. lagowskii. While the regulatory effect of L-carnitine on lipid metabolism and glucose utilization of R. lagowskii varies with dietary lipid sources and tissues.

Victim justice sensitivity might be positively associated with envy; however, there is little understanding of the mechanism of this relationship. For the first time, we explored the effect of victim justice sensitivity on envy, and whether this effect is mediated by upward social comparison and moderated by perceived control. A sample of 1570 Chinese undergraduates was collected. The results revealed that victim justice sensitivity was positively associated with envy. Additionally, upward social comparison partially mediated the relationship between victim justice sensitivity and envy, and this mediating effect was further moderated by perceived control. A more positive correlation was found between upward social comparison and envy in individuals with lower levels of perceived control. This finding not only clarities the underlying mechanism but also suggests a potential way of alleviating envy.

Objective: To explore the audiological characteristics of infant auditory neuropathy (AN) patients with cochlear microphonic (CM) recorded but no otoacoustic emission (OAE) response and clinically reduce the rate of missed diagnosis of AN. Design: Retrospective clinical study of medical data from 2003 to 2020. Setting: Otolaryngology head and neck surgery clinical hearing center. Participants: Eighteen infant AN patients with CM present and distortion product otoacoustic emission (DPOAE) absent in both ears were OAE absent group. Forty-four infant AN patients with CM and DPOAE present in both ears were OAE present group. Main outcome measures: Audiological characteristics. Results: 1. The age of onset in OAE absent group was 0.9 (0.02) years old, which was less than 1.11 (1.63) years old in OAE present group (P=0.041). 2. The CM threshold of OAE absent group was 80 (10) dB nHL, which was significantly higher (P

T-cadherin functions as a suppressor gene, which is frequently inactivated by aberrant promoter methylation in several human cancers, but its methylation status in oral squamous cell carcinoma (OSCC) has been scarcely studied. Thus this study aimed at exploring the clinical significance and prognostic value of T-cadherin methylation in sera of patients with OSCC. Methylation-specific PCR (MSP) and bisulfate sequencing PCR (BSP) was performed to examine the methylation status of T-cadherin. Then, the associations between methylation status of T-cadherin and various clinicopathological variables or patient survival were investigated in 202 patients with OSCC and 68 controls. T-cadherin methylation was detected in 62 out of 202 (30.7%) patients with OSCC, and the methylation status of T-cadherin in corresponding tissues was confirmed by BSP. Methylation of T-cadherin was significantly associated with advanced tumor T-stage (P < 0.001) and N-stage (P = 0.003), positive lymphatic metastasis (P = 0.004) and tumor recurrence (P = 0.001). In addition, patients with methylation of T-cadherin had worse overall survival (P = 0.018) and progression-free survival (P < 0.001) than patients without, and methylation of T-cadherin in sera was an independent prognostic factor for worse overall survival (HR: 3.626, 95% CI: 1.112-9.624, P = 0.007) and progression-free survival (HR: 4.201, 95% CI: 1.562-10.038, P < 0.001) of patients with OSCC. These results demonstrated that methylation of T-cadherin was frequently detected in sera of patients with OSCC, which was associated with risk factors of poor outcomes, and may act as a potential independent prognostic marker for patients with OSCC.

A novel cellulase-producing actinomycete strain Gxj-6T, isolated from soil in the cold region (Heihe city, Heilongjiang province, the northernmost part of China), subjected to a taxonomic study using a polyphasic approach. In the neighbour-joining phylogenetic tree based on 16S rRNA gene sequences, strain Gxj-6T fell within the clade comprising the type strains of species of the genus Microbispora. 16S rRNA gene sequence similarity studies exhibited that species Gxj-6T was most closely related to Microbispora bryophytorum NEAU-TX2-2T (99.45%), Microbispora fusca NEAU-HEGS1-5T (99.41%), Microbispora camponoti 2C-HV3T (99.31%) and Microbispora rosea subsp. rosea JCM 3006T (98.68%). Organism Gxj-6T contained MK-9(H2) as the predominant ubiquinone and C18:0 10-methyl as the major fatty acid. The major polar lipids of culture Gxj-6T were diphosphatidylglycerol, phosphatidylethanolamine, phosphatidylinositol mannoside, three unidentified phospholipids, two unidentified glycolipids and two unidentified lipids. The DNA G+C content of strain Gxj-6T was 71.25 mol%. The morphological and chemotaxonomic properties of the strain are also consistent with those members of the genus Microbispora. Combinated with the lower DNA–DNA relatedness values, phenotypic properties, physiology and biochemistry distinctiveness with other recognized species strains, revealed that strain Gxj-6T is separated from other phylogenetically closely species of the genus Microbispora. Therefore, strain Gxj-6T is considered to represent a novel species of the genus Microbispora, for which the name Microbispora cellulosiformans sp. nov. is proposed. The type strain is Gxj-6T (= CGMCC 4.7605T = DSM 109712T).

The Nrf2 (nuclear factor erythroid 2-related factor 2) plays a central role in cell protection against a wide variety of environmental stressors through the Nrf2-Keap1 (Kelch-like ECH-associated protein 1) pathway, but its involvement in modulation of antioxidant system of fish cell is still largely unexplored. The present study focused on the molecular cloning and silencing of the Nrf2 in the fathead minnow muscle cell line (FHM) in response to the oxidative stress induced by H2O2. A full-length cDNA of coding Nrf2 was cloned from FHM cells by RT-PCR and RACE approaches. The obtained cDNA covered 2578 bp with an open reading frame (1770 bp) of encoding 589 amino acids. Sequence alignment and phylogenetic analysis revealed a high degree of conservation (51–86%) among 16 fishes. Based on the cloned Nrf2 sequence, the siRNA-242 of targeting Nrf2 with the best knocking down efficiency was designed and detected. Then, the mRNA levels of Keap1, Nrf2, Maf (musculoaponeurotic fibrosarcoma oncogene), and HO-1 (haemoxygenase-1); the activities of T-SOD (total superoxide dismutase), CAT (catalase), and GSH-PX (glutathione peroxidase); the levels of GSH (glutathione) and MDA (malonaldehyde); and the cell cycle and apoptosis were analyzed to investigate the molecular responses after H2O2 exposure. These results showed a coordinated transcriptional regulation of Keap1, Maf, and HO-1 and antioxidants (T-SOD, GSH, CAT, and GSH-PX) and MDA levels after H2O2 exposure, leading to oxidative damage and apoptosis. These findings provided an insight to understand the mechanisms of Nrf2 against oxidative stress in fish.

Previous studies have shown that patients with acoustic neuroma (AN) sometimes present with sudden sensorineural hearing loss (SSNHL) as an initial symptom. The purpose of this research was to investigate the clinical characteristics, diagnosis and treatment of AN in patients diagnosed as SSNHL initially. A total of 10 patients presented with SSNHL as the initial symptom and diagnosed as AN by magnetic resonance imaging (MRI) finally were enrolled in this study. Patient demographics, associated complaints (mostly tinnitus and vertigo), severity of hearing loss, audiogram configurations, auditory brainstem response (ABR) and MRI examination were reviewed and analyzed. Treatment outcome and management protocols were also included in this study. These patients exhibited varying severity of hearing loss and a variety of audiogram configurations. All patients showed an abnormal ABR. According to the Koos grading standard, there were 5 grade I (intracanalicular) tumors, 3 grade II tumors and 2 grade III tumors. The treatment outcome revealed that 2 patients exhibited recovery of the average hearing of impaired frequency by more than 15 dB, and 6 patients showed no recovery. Four patients were referred to undergo surgical treatment after being diagnosed with AN, 1 patient accepted stereotactic radiation therapy, and the remaining 5 patients were taken “waiting and scan” strategy. The findings of this study show that hearing recovery after corticosteroid treatment for SSNHL does not exclude the presence of AN and all patients diagnosed as SSNHL initially should undergo MRI and ABR to prevent misdiagnosis and delays in potential treatment.