Objective: To investigate the possible pathogenesis and possible risk factors of tinnitus related to female menopause. Methods: From April 2016 to October 2016, 59 female patients with menopausal syndrome were diagnosed in the menopause comprehensive management clinic. Tinnitus and menopause questionnaires were conducted, based on whether having tinnitus, those patients were divided into two groups: tinnitus group and no tinnitus group. Age, body mass index (BMI), Kupperman menopausal index (KMI) score, follicle-stimulating hormone (FSH) level of patients in the two groups were analyzed. Menopausal symptoms, related medical history and possible related factors of tinnitus were statistically analyzed. Results: A total of 59 cases were collected, 22 of which were accompanied by tinnitus. The incidence of idiopathic tinnitus was 35.1% (20/57) because 2 cases of thyroid related tinnitus with clear etiology were removed. Complete data were obtained from 17 of 20 patients with idiopathic tinnitus and 26 of 37 patients without tinnitus. Age, BMI, menopause KMI score, hormone level, menopause symptoms and possible factors related to tinnitus were statistically analyzed between the two groups, and the incidence of headache was statistically different between the two groups (χ2=9.098, P=0.003), but no other factors were statistically significant(P>0.05). The severity of insomnia and tinnitus were further analyzed (χ2=2.841, P=0.417), and there was no significant difference between the two groups. Conclusion: Headache history may be one of the high risk factors for the occurrence of menopausal tinnitus.
Objective: To elucidate the patterns of neural activity alterations associated with auditory speech comprehension across the lifespan and the impact of varying listening environments on these dynamics. Methods: Functional near-infrared spectroscopy (fNIRS) was employed to measure the concentration of oxygenated hemoglobin in the brains of 93 adults aged from 20 to 70 with normal hearing. These participants were recruited from Beijing Tongren Hospital, affiliated with Capital Medical University, between March 2021 and February 2023. Brain activity was recorded as subjects passively listened to sentences in both silent and noise conditions with varying signal-to-noise ratios (SNR). The alterations in brain activity were analyzed to delineate the age-related trends under different auditory conditions. Statistical analysis was performed using SPSS 22.0 software. Results: The bilateral primary auditory cortex, superior temporal gyrus, and Wernicke's area, critical for sound signal discrimination and perception, exhibited enhanced activity post-stimulus presentation. Broca's area, pivotal for speech production, demonstrated an initial decrease in activity followed by an increment after stimulus onset. The ventral middle temporal gyrus and dorsal postcentral gyrus showed augmented activity in later time windows. Furthermore, it was observed that in quiet conditions and at low noise levels (SNR=10 dB), auditory cortical activity diminished with age. With increasing noise levels (SNR=5 dB), compensatory brain regions (right ventral middle temporal gyrus and dorsal postcentral gyrus) showed enhanced activity with advancing age. As noise intensity further escalated (SNR=0, SNR=-5 dB), not only did auditory cortical activity decline, but also the activity in regions associated with semantic processing and motor functions reduced with age. Conclusion: During auditory speech comprehension, dual-pathway brain regions exhibit distinct activity patterns. With heightened noise exposure, an increasing number of brain regions are influenced by aging, manifesting as a general decline in activity in most dual-pathway regions, alongside a selective augmentation in some compensatory regions on the right hemisphere.
Objective: To investigate the clinical features, molecular etiology, and treatment of a family with Treacher Collins Syndrome 2 (TCS2). Methods: Information of the proband (female, 8 years old) including medical history and family history was collected. Physical examination and examinations concerning laboratory, audiology, and radiology were performed on the proband. Physical examination was also performed on the family members. Genomic DNA of proband was extracted for whole exome sequencing, and then the genomic DNA of family members was extracted for Sanger sequencing. POLR1D and TCS2 related literatures published before August 31,2023 were searched and sifted in PubMed and CKNI databases. The clinical characteristics of TCS2 were summarized. Results: The proband had poor hearing since childhood, with pure tone audiometry indicating conductive hearing loss. She had a smaller jaw, bilateral preauricular fistulas and cup-shaped ear deformities. Temporal bone CT scan revealed deformities in the left external ear canal, bilateral middle ear and inner ear. A bone-conduction hearing aid device was surgically implanted, resulting in restoration of almost normal hearing levels. The proband's mother also had a slightly smaller jaw. Genetic analysis revealed a novel heterozygous variant NM_015972.4:c.38_47del in the POLR1D gene in the proband, which was inherited from her mother. A review of the literature revealed no clear evidence of genotype-phenotype correlation in TCS2. Conclusions: Molecular diagnosis plays a vital role in the diagnosis of TCS2. Patients with normal facial phenotype may be carriers of pathogenic variants in the POLR1D gene and have the risk of passing it to the offsprings with complete penetrance. Proper bone conductive hearing devices can improve the quality of life of TCS2 patients.