Music perception and its correlation with auditory speech perception in pediatric Mandarin-speaking cochlear implant users.

Background: Cochlear implants (CI) help regain perception of sound for patients with sensorineural hearing loss. The ability to recognize music pitch may be crucial for recognizing and producing speech for Mandarin.

Aims/objectives: This study aims to search for possible influencing factors of music perception and correlations between music perception and auditory speech abilities among prelingually deaf pediatric Mandarin-speaking CI users.

Endoscopic Management of Recurrent Epistaxis Caused by Retiform Hemangioendothelioma in a Child: A Case Report.

Retiform hemangioendothelioma (RH) is a rare intermediate (locally aggressive) vascular tumor that mostly affects the dermis of the trunk and limbs, but has never been reported in the inferior turbinate. A 10-year-old Chinese boy presented with recurrent epistaxis in his left nasal cavity and anemia for more than 2 years. Radiographic and electronic video laryngoscopic images showed an expansile mass in the left inferior turbinate.

Deafness-associated tRNA 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 tRNA 593T > C mutation that changed a highly conserved uracil to cytosine at position 17 of the DHU-loop. The m.

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

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. 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.

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.

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.

Research Advances on Deafness Genes Associated with Mitochondrial tRNA-37 Modifications.

As the most common cause of speech disorders, the etiological study of deafness is important for the diagnosis and treatment of deafness. The mitochondrial genome has gradually become a hotspot for deafness genetic research. Mitochondria are the core organelles of energy and material metabolism in eukaryotic cells.

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.

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

Background: 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 mutation groups.

Material And Methods: Clinical data of 71 patients with gene mutations who received CI in the Second Hospital of Lanzhou University from 2012 to 2015 were retrospectively reviewed.

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).

[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.

[Superficial angiomyxoma in nasal vestibule: a case report].

This paper reported a case of superficial angiomyxoma in the region of the nasal vestibule. The clinical manifestation was swelling of the left nasal vestibular skin, while paranasal sinus CT showed swell soft tissue in the anterior and superior region to the left maxilla. Under general anesthesia, the left nasal vestibular mass was resected under nasal endoscopy.

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.

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.

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

: The 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 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. : Ethics approval was obtained to collect 1330 cases of moderate to very severe nonsyndromic sensorineural deafness in northwestern China.

Surgical management of mucosal contact headache.

Background: Most research on mucosal contact headache has focused on mucosal contact between the nasal septum and middle or inferior turbinate. However, rarely have any studies explored how headache is related to the only one contact point between superior turbinate and nasal septum.

Objective: To explore how headache is related to the only one contact point between superior turbinate and nasal septum.

[Computer tomography demonstrations of single-sided deafness].

To investigate the distribution of common inner ear and internal auditory canal malformations in children with single-sided deafness（SSD） ,and to explore the imaging etiology of SSD by comparing the quantitative parameters of key bone structures between deaf and normal ears in children with congenital SSD. Forty children with SSD diagnosed in the Second Hospital of Lanzhou University from September 2016 to March 2019 were collected. All of them underwent HRCT examinations of temporal bone .

Targeted next-generation sequencing identified a novel variant of in a Chinese family with Waardenburg syndrome type 2.

Objective: Waardenburg syndrome type 2 (WS2) is an autosomal dominant syndrome, characterized by bright blue eyes, hearing loss, and depigmented patches of hair and skin. It exhibits high phenotypic and genetic heterogeneity. We explored the molecular etiology in a Chinese family with WS2.

Study on the correlation between ambient environment-meteorological factors and the number of visits of acute otitis media, Lanzhou, China.

Unlabelled: To investigate the correlation between environmental-meteorological factors and daily visits for acute otitis media (AOM) in Lanzhou, China.

Methods: Data were collected in 2014-2016 by the Departments of Otolaryngology-Head and Neck Surgery at two hospitals in Lanzhou. Relevant information, including age, sex and visiting time, was collected.

Targeted next-generation sequencing identifies a novel frameshift EYA1 variant causing branchio-otic syndrome in a Chinese family.

Objective: To evaluate the genotype-phenotype correlation of branchio-otic syndrome (BOS) in a Chinese family.

Methods: The proband in this study was an 18-month-old boy with hearing loss, preauricular pit, and branchial fistula without a renal anomaly. We collected blood samples from 6 family members, including 4 who were affected by the syndrome.

The mutation frequencies of GJB2, GJB3, SLC26A4 and MT-RNR1 of patients with severe to profound sensorineural hearing loss in northwest China.

Objective: To expose the spectrum and frequency of GJB2, GJB3, SLC26A4 and MT-RNR1 in northwest China and to investigate the underlying causative genes in patients without common mutations.

Methods: We analyzed the mutation screening results of GJB2, GJB3, SLC26A4 and MT-RNR1 in 398 unrelated severe-to-profound probands with bilateral, symmetrical sensorineural hearing loss. Subsequently, we selected 10 probands with a significant family history of inherited hearing loss (HL) that did not have the above four common gene mutations to perform next-generation sequencing (NGS) of 139 known deafness genes, followed by co-segregation analysis of all available family members.