A Comparative Study of Hearing Handicap Inventory and Pure-Tone Audiometry Scores in Unilateral Hearing Impaired Patients.

Objective: To investigate the consistency between the hearing handicap inventory (HHI) and pure-tone audiometry (PTA) scores in assessing hearing status to provide valuable insights for clinical application. Methods: Retrospective analysis of clinical data and the HHI reporting status of 6540 patients admitted between April 2020 and July 2022 for self-reported unilateral hearing loss who met the study inclusion and exclusion criteria. The kappa coefficient was used to evaluate the consistency of HHI and PTA in assessing the hearing status of the participants. Results: The PTA results showed that among the 6540 participants, 3895 exhibited normal hearing, 1434 showed mild hearing loss, 809 presented with moderate hearing loss, and 402 showed severe hearing loss. The mean hearing thresholds from 0.5 to 4 kHz in healthy ears ranged from 3.65 to 18.45 dB HL, with a mean of 10.83 ± 5.29 dB HL; in ears affected by hearing loss, this ranged from 35 to 125 dB HL, with a mean of 69.63 ± 28.45 dB HL. The HHI scores showed that 4820 people had normal hearing, 1245 had mild-to-moderate hearing loss, and 475 had severe hearing loss. The kappa coefficients of normal, mild-to-moderate, and severe hearing loss were 0.312, 0.223, and 0.716, respectively (P = .001). The consistency between the 2 groups was particularly significant in the assessment of severe hearing loss. Using the PTA results as a benchmark, the sensitivity, specificity, positive predictive value, and negative predictive value of the HHI were found to be 73.08%, 87.83%, 95.60%, and 70.98%, respectively. Conclusion: The HHI and PTA results were consistent in the assessment of hearing status, particularly in the assessment of severe hearing loss, and the level of consistency between the 2 methods was high. The combined use of these tools can facilitate a comprehensive assessment of the auditory status of patients with hearing loss.

Deafness-associated tRNAPhe mutation impaired mitochondrial and cellular integrity.

Defects in mitochondrial RNA metabolism have been linked to sensorineural deafness that often occurs as a consequence of damaged or deficient inner ear hair cells. In this report, we investigated the molecular mechanism underlying a deafness-associated tRNAPhe 593T > C mutation that changed a highly conserved uracil to cytosine at position 17 of the DHU-loop. The m.593T > C mutation altered tRNAPhe structure and function, including increased melting temperature, resistance to S1 nuclease-mediated digestion, and conformational changes. The aberrant tRNA metabolism impaired mitochondrial translation, which was especially pronounced by decreases in levels of ND1, ND5, CYTB, CO1, and CO3 harboring higher numbers of phenylalanine. These alterations resulted in aberrant assembly, instability, and reduced activities of respiratory chain enzyme complexes I, III, IV, and intact supercomplexes overall. Furthermore, we found that the m.593T > C mutation caused markedly diminished membrane potential, and increased the production of reactive oxygen species in the mutant cell lines carrying the m.593T > C mutation. These mitochondrial dysfunctions led to the mitochondrial dynamic imbalance via increasing fission with abnormal mitochondrial morphology. Excessive fission impaired the process of autophagy including the initiation phase, formation, and maturation of the autophagosome. In particular, the m.593T > C mutation upregulated the PARKIN-dependent mitophagy pathway. These alterations promoted an intrinsic apoptotic process for the removal of damaged cells. Our findings provide critical insights into the pathophysiology of maternally inherited deafness arising from tRNA mutation-induced defects in mitochondrial and cellular integrity.

A novel intronic variant causing aberrant splicing identified in two deaf Chinese siblings with enlarged vestibular aqueducts.

OBJECTIVE: We aimed to evaluate the genotype-phenotype relationship in two Chinese family members with enlarged vestibular aqueduct (EVA). METHODS: We collected blood samples and clinical data from each pedigree family member. Genomic DNA was isolated from peripheral leukocytes using standard methods. Targeted next-generation sequencing and Sanger sequencing were performed to find the pathogenic mutation in this family. Minigene assays were used to verify whether the novel intronic mutation SLC26A4c.765+4A>G influenced mRNA splicing. RESULTS: Hearing loss in the patients with EVA was diagnosed using auditory tests and imaging examinations. Two pathogenic mutations, c.765+4A>G and c.919-2A>G were detected in SLC26A4. In vitro minigene analysis confirmed that c.765+4A>G variant could cause aberrant splicing, resulting in skipping over exon 6. CONCLUSIONS: The SLC26A4c.765+4A>G mutation is the causative variant in the Chinese family with EVA. Particular attention should be paid to intronic variants.

Model construction and theoretical evaluation of the performance improvement of acetone-butanol-ethanol extractive fermentation by adding surfactant.

Severe butanol toxicity to the metabolism of solventogenic clostridia significantly impede the application of fermentative butanol as a biofuel. Liquid-liquid extraction is an efficient method to reduce the butanol toxicity by in-situ removing it in the extractant phase. Butanol mass transfer into extractant phase in static acetone-butanol-ethanol (ABE) extractive fermentation with biodiesel as the extractant could be enhanced by adding a tiny amount of surfactant such as tween-80. In the case of corn-based ABE extractive fermentation by Clostridium acetobutylicum ATCC 824 using biodiesel originated from waste cooking oil as extractant, addition of 0.14% (w/v) tween-80 could increase butanol production in biodiesel and total solvents production by 21% and 17%, respectively, compared to those of control under non-surfactant existence. Furthermore, a mathematical model was developed to elucidate the mechanism of enhanced ABE extractive fermentation performance. The results indicated that the mass transfer improvement was obtained by effectively altering the physical properties of the self-generated bubbles during ABE extractive fermentation, such as reducing bubble size and extending its retention time in extractant phase, etc. Overall, this study provided an efficient approach for enhancing biobutanol production by integration of bioprocess optimization and model interpretation.

The mental health of paediatric cochlear implant recipients.

OBJECTIVES: To evaluate the mental health of paediatric cochlear implant users and analyse the relationship between six dimensions (movements, cognitive ability, emotion and will, sociality, living habits and language) and hearing and speech rehabilitation. METHODS: Eighty-two cochlear implant users were assessed using the Mental Health Survey Questionnaire. Age at implantation, time of implant use and listening modes were investigated. Categories of Auditory Performance and the Speech Intelligibility Rating Scale were used to score hearing and speech abilities. RESULTS: More recipients scored lower in cognitive ability and language. Age at implantation was statistically significant (p < 0.05) for movements, cognitive ability, emotion and will, and language. The time of implant usage and listening mode indicated statistical significance (p < 0.05) in cognitive ability, sociality and language. CONCLUSION: Timely attention should be paid to the mental health of paediatric cochlear implant users, and corresponding psychological interventions should be implemented to make personalised rehabilitation plans.

[Extranodal NK/T cell lymphoma, nasal type involving the larynx and digestive tract: a case report and literature review].

Extranodal NK/T cell lymphoma, nasal type（ENKTL） is a highly aggressive malignant tumor derived from NK cells. This article reports a case of ENKTL invading the larynx and digestive tract. The clinical clinical manifestations include hoarseness and intranasal masses.

The effect of SLC26A4 gene mutations on long-term rehabilitative outcomes in cochlear implant patients.

BACKGROUND: SLC26A4 gene mutations related to hearing loss patients can obtain good hearing and speech rehabilitation effects after cochlear implantation (CI). OBJECTIVE: To explore the long-term rehabilitative outcomes of CI in patients with different SLC26A4 mutation groups. MATERIAL AND METHODS: Clinical data of 71 patients with SLC26A4 gene mutations who received CI in the Second Hospital of Lanzhou University from 2012 to 2015 were retrospectively reviewed. According to the genetic test results, use One-way ANOVA analysis to compare the differences in auditory results, categories of auditory performance (CAP) and speech intelligibility rating (SIR) index questionnaire scores and speech recognition rates among different groups in 4-5 years after CI. RESULT: Compared with other genotypes of SLC26A4, the patients with homozygous mutation of c.919-2A > G in SLC26A4 had better hearing aid threshold at 500 Hz and better recognition rates of Yangyang words than other monoallelic mutation groups after CI (p < .05). CONCLUSIONS AND SIGNIFICANCE: The most common hot spot mutation of SLC26A4 gene is c.919-2A > G. The patients with homozygous mutation of c.919-2A > G in SLC26A4 gene had partly better hearing and speech rehabilitation than other monoallelic mutation groups after CI.

Research on congenital severe-to-profound sensorineural hearing loss associated with central lucency of the bony island of the lateral semicircular canal.

BACKGROUND: Central lucency of the bony island of the lateral semicircular canal (LSCC) is commonly found in patients with congenital severe-to-profound sensorineural hearing loss (SNHL). OBJECTIVE: Exploring the significance of bony island lucency of LSCC in congenital severe-to-profound SNHL patients. MATERIAL AND METHODS: Retrospective measurements of the inner ear structures were made on axial temporal bone CT scans from 182 (364 ears) congenital severe-to-profound SNHL patients and 50 (100 ears) tympanic membrane perforation (TMP) patients. RESULTS: The incidence of bony island lucency of LSCC was 46.7% in the congenital severe-to-profound SNHL group and 0% in the TMP group. There was a statistically significant difference in inner ear structures among congenital severe-to-profound SNHL patients with normal inner ear structure and bony island lucency of LSCC, congenital severe-to-profound SNHL patients with normal inner ear structure and no bony island lucency of LSCC, and TMP patients. The importance of the bony island lucency of LSCC was further confirmed through multiple linear regression analysis. CONCLUSIONS AND SIGNIFICANCE: Bony island lucency may have significance in congenital severe-to-profound SNHL and may be a manifestation of largely overlooked SCC malformation or hypoplasia of the inner ear.

The value of nonverbal intelligence in cochlear implant.

BACKGROUND: Congenital sensorineural hearing loss is a common congenital condition. OBJECTIVES: The purpose of this study was to assess the correlation between nonverbal mental development and the effect of post-cochlear implant in children. MATERIAL AND METHODS: The study is a retrospective analysis of the CI program implemented at the ENT in the Lanzhou University Second Hospital (China). We reviewed data of 225 children who received CI between 2015 and 2018. Finally, 115 children met the inclusion criteria. Our hospital used The Griffith mental development scales to evaluate the preoperative non-verbal intelligence. The outcome of CI was evaluated using the categories of IT-MAIS, MUSS, CAP and SIR at 2 years after surgery. The associations between the preoperative non-verbal development quotient (DQ) and the postoperative outcomes were analyzed. RESULTS: Preoperative non-verbal DQ correlates with the long-term postoperative result, especially the Eye-hand co-ordination and Performance DQ. CONCLUSIONS AND SIGNIFICANCE: Preoperative non-verbal intelligence would predict postoperative effect. The single postoperative scale does not fully reflect the postoperative result.

Effective fucoxanthin production in the flagellate alga Poterioochromonas malhamensis by coupling heterotrophic high-cell-density fermentation with illumination.

The unicellular flagellate algae Poterioochromonas malhamensis is a potential fucoxanthin-rich resource for sustainable and cost-effective fucoxanthin production. Light and nutrients are critical regulators for the accumulation of fucoxanthin in P. malhamensis. In this study, the maximum fucoxanthin yield of 50.5 mg L-1 and productivity of 6.31 mg L-1 d-1 were achieved by coupling high-cell-density fermentation with illumination. It was found that the combined use of organic and inorganic nitrogen (N) nutrition could improve the fucoxanthin yield as single inorganic or organic N had limitation to enhance cell growth and fucoxanthin accumulation. White light was the optimal light quality for fucoxanthin accumulation. Under white light and a moderate light intensity of 150 µmol m-2 s-1, the highest biomass concentration and fucoxanthin content reached 32.9 g L-1 and 1.56 mg g-1 of dry cell weight (DCW), respectively. This is the first study on effective fucoxanthin production in P. malhamensis by integrating illumination with high-cell-density fermentation, which paved the way for further development of P. malhamensis as a potential source for commercial fucoxanthin production.

A new l-cysteine-assisted glycerol organosolv pretreatment for improved enzymatic hydrolysis of corn stover.

Deconstruction of lignocellulose via efficient pretreatment is crucial for producing fermentable sugars. In this study, effects of glycerol organosolv pretreatment (GOP) on main chemical composition of corn stover were investigated. Results indicate that the residual corn stover after 80 wt% glycerol pretreatment (at 220 °C for 0.5 h) yielded 75.97 % glucose and 78.21 % xylose after enzymatic hydrolysis, which were enhanced by 3.39- and 6.08-fold compared to the untreated corn stover. Subsequently, an l-cysteine-assisted GOP was proposed with higher yields of glucose (86.20 %) and xylose (91.13 %). When pretreating corn stover with 80 wt% glycerol containing 0.07 wt% l-cysteine at 220 °C for 0.5 h, higher fermentable sugars of 26.08 g were produced from 100 g feedstock after enzymolysis. Intrinsic mechanisms of the proposed pretreatment for enhancing enzymatic digestibility were elucidated by physiochemical characterization technologies and techno-economic analysis was also studied. This study provides guidance for fermentable sugars production from renewable lignocellulose.

[Clinical research progress of bonebridge implantation].

Implantable bonebridge（BB） is suitable for patients over 5 years with conductive/mixed hearing loss and unilateral deafness, and the surgical approach depends on the anatomical structure of the patient's ear. Recent studies have shown that compared with other implantable hearing devices, implantable BB have more efficient hearing gain and lower incidence of complications. However, the postoperative effect of implantable BB on unilateral deafness patients and the poor compensation of low-frequency hearing threshold need to be further studied. This article reviews the clinical research on implantable BB, so as to provide some reference for clinicians to select hearing devices for patients individually.

A giant pyogenic granuloma gravidarum in the nasal cavity: A case report.

Pyogenic granuloma (PG) is a benign fibrovascular proliferative lesion on the skin and mucous membranes, but its pathogenesis remains unclear. PG usually occurs on the head and neck region, fingers and toes. The oral gingiva is the most common location for pregnant patients, while it is rarely found in the nasal cavity. This case is notable not only for its uncommon site and size but also for its gradual growth after delivery. Endoscopic surgery can achieve the desired cosmetic effect and a satisfactory airway. A rapidly growing hemorrhagic lesion in the nasal cavity should be considered as a differential diagnosis.

Endoscopic Management of Recurrent Maxillary Sinus Ameloblastoma in a Child With Autism Spectrum Disorder: A Case Report.

Ameloblastomas are slow-growing, aggressive odontogenic epithelial tumors that originate from the jawbone. One of the most easily relapsing maxillofacial tumors, ameloblastomas mainly occur in the mandibular molar area and ascending branch, although they can occasionally occur in the nasal cavity and paranasal sinuses. A 14-year-old child with autism spectrum disorder underwent sinus computed tomography (CT) under anesthesia. A swollen tumor had grown in the left maxillary sinus, and the bone of the maxillary sinus was damaged. Nine months after the first operation, recurrence was observed in the left maxillary sinus. The pathological diagnosis was ameloblastoma. Due to the child's inability to communicate and cooperate with the treatment normally, he underwent endoscopic surgery again combined with low-temperature plasma treatment. No tumor recurrence was found on reexamination 6 months after surgery.

[Scanning electron microscope of the human nasal septum].

Objective:Explore the significance of ultrastructural differences in tissue engineering, 3D printing, and rhinoplasty. Methods: 32 specimens （8 vomers, 8 perpendicular plates of ethmoid bone, 8 maxillary nasal crests, and 8 septal cartilage） of the nasal septum from patients with a nasal deviated septum and chronic sinusitis undergoing septoplasty were selected and examined using scanning electron microscopy. Results: The nasal septum of patients of different ages behaves similarly under the scanning electron microscope, and the bones of different parts of the nasal septum have similarities and differences. Conclusion:By observing the scanning electron micrograph of the nasal septum and analyzing the surface ultrastructure, it provides important information for the development of tissue engineering, assists in the refined modeling of 3D printing technology, and provides more ideal restoration materials for clinical operations.

Screening of SLC26A4 Gene Hotspots in 2673 Patients Associated with Sensorineural Hearing Loss in Northwestern China.

BACKGROUND: This study aimed to investigate the incidence of the hotspot mutations c.919-2A>G and c.2168A>G in SLC26A4 in the northwestern Chinese population. METHODS: A total of 2673 unrelated patients were recruited from northwestern China, and clinical information was obtained from all patients. Peripheral blood samples were acquired to detect the genotype of each patient by direct sequencing. Statistical analysis was conducted with Statistical Package for the Social Sciences 19.0 software. RESULTS: Overall, 118 patients (4.4%) were identified with biallelic mutations, including 84 (3.14%) homozygotes and 34 (1.27%) compound heterozygotes. Moreover, significant differences between Han and Uighur were identified regarding the frequencies of c.919-2A>G homozygous and biallelic mutations. CONCLUSION: This model for the rapid screening of hotspot mutations can identify the molecular cause for 4.4% of patients with severe to profound sensorineural hearing loss in northwestern China, and there may be distinctive hotspot mutations in different ethnic populations.

Verification of an iPSC line (LZUi002-A) from a patient with a novel mutation in the TBL1X gene.

More than 120 genes have been reported to be associated with deafness, and deletion of the TBL1X gene may cause deafness in humans. In this study, we generated an induced pluripotent stem cell (iPSC) line from dermal fibroblasts of a 34-year-old deaf person with a novel variant c.342_343insGCGGCG in the TBL1X gene. The induced patient-specific iPSC line with a normal karyotype and expressed pluripotent markers, it also shows differentiation totipotency and tridermogenesis in vivo. It may be a good model for studying hearing loss in vitro and it will benefit to the development of new therapies for deafness.

Analysis of GJB2 Gene Mutations in 1330 Deafness Cases of Major Ethnic Groups in Northwest China.

Background: The GJB2 gene is the most common deafness gene, and epidemic characteristics have obvious racial specificity. Our study aimed to investigate the prevalence and ethnic specificity of the GJB2 gene in deafness in major ethnic groups in Northwest China, evaluate the value of molecular screening for deafness in minority populations, and explore the strategies and methods for genetic diagnosis. Methods: Ethics approval was obtained to collect 1330 cases of moderate to very severe nonsyndromic sensorineural deafness in northwestern China. The mutation characteristics of ethnic minorities were analyzed and compared with those of 464 patients with nonsyndromic sensorineural deafness among ethnic Han in the northwestern from research group by Sequence Scanner V25.0. Then, we analyzed the ethnic specificity of the mutations. Results: A total of 15 GJB2 sequence changes were detected in 1330 minority patients. The study showed that the allele frequency in Tibetan patients was significantly lower than that in Hui and Dongxiang patients, that in Uygur patients was significantly lower than that in Han and Hui patients, and that in Kazak and Tibetan patients was significantly lower than that in Han patients, and the differences between other ethnic groups were not statistically significant. Each ethnic group has a unique GJB2 gene mutation spectrum, and its hotspot mutation distribution has its own characteristics, with c.235delC, c.109 G > A, c.299-300delAT, and c.35delG being common. Conclusions: It has been confirmed that GJB2 gene mutation has a high prevalence in patients with nonsyndromic sensorineural hearing loss in Northwest China. Each ethnic group has a unique mutation spectrum for the GJB2 gene, which is related to its genetic background. It is necessary to develop a corresponding gene diagnosis strategy according to the hotspot mutations and mutation spectrum of each ethnic group.

[Effects of Butylphthalide on the expressions of HMGB1 and RAGE in frontal lobe of rats after chronic sleep deprivation].

OBJECTIVE: To investigate the effects of Butylphthalide on the expressions of HMGB1 and RAGE in frontal lobe of rats after chronic sleep deprivation. METHODS: Chronic sleep deprivation and butylphthalide treatment was performed in Sprague Dawley(SD)rats and the rats were divided into three groups (n＝6): platform control group, chronic sleep deprivation group and chronic sleep deprivation + butylphthalide intervention group. Rats suffering chronic sleep deprivation were put in multiple platforms box for 18 h per day and sleep deprivation lasted for 28 days. Rats in butylphthalide intervention group were intraperitoneally injected with butylphthalide 100 mg/(kg·d) for 14 days after sleep deprivation. After collecting brains, high-mobility group box (HMGB1) and nuclear transcription factor kappB (NF-κB)p65 were detected by immunohistochemistry. The expression of HMGB1, silent information regulator of transcription 1 (SIRT1), receptor for advanced glycation end-products (RAGE) and NF-κB in frontal lobe were determinated by Western blot. RESULTS: Compared with platform control group, the expression levels of HMGB1, RAGE and nuclear NF-κB p65 were increased significantly, while the expression of SIRT1 was decreased siginificantly in frontal lobe of chronic sleep deprivation group (all P＜0.05). Compared with chronic sleep deprivation group, the expression levels of of HMGB1, RAGE and nuclear NF-κB p65 were decreased significantly, while the expression of SIRT1 was increased significantly in chronic sleep deprivation + butylphthalide intervention group (all P＜0.05). CONCLUSION: Butylphthalide can inhibit HMGB1/RAGE/NF-κB pathway in frontal lobe of rats after chronic sleep deprivation by changing the expression of HMGB1 and RAGE, and reducing the nuclear translocation of NF-κBp65.