Objective:To retrospectively analyze the results of auditory examination,vestibular function examination and laboratory examination of 63 patients diagnosed as vestibular neuritis.Methods:A total of 63 patients diagnosed with vestibular neuritis hospitalized in the Department of Otolaryngology, Head and Neck Surgery of the Third Affiliated Hospital of Sun Yat-sen University, from October 2012 to December 2022 were recruited. All patients met the diagnostic criteria for the 2022 Bárány association vestibular neuritis. Clinical data and the results of pure tone audiometry, electrocochleogram, video electronystagmogram, caloric test, cervical vestibuloevoked myogenic potential（cVEMP）, ocular vestibuloevoked myogenic potential（oVEMP）, video head impulse test（vHIT） was collected.A total of 63 age-and sex-matched healthy subjects in the physical examination center were randomly selected as the control group. The differences of blood indexs and lipid metabolism indexes between the two groups were compared. Results:In patients with vestibular neuritis, 50 out of 63 patients presented normal threshold in pure tone audiometry, 8 out of 63 patients had bilateral high-frequency sensorineural hearing loss and 5 out of 63 patients had unilateral mild high-frequency sensorineural hearing loss, 56 out of 63 cases completed the electrocochleogram, of which 3 cases had a binaural-SP/AP amplitude ratio≥0.4, 5 cases had monaural amplitude ratio ≥0.4. Fifty-five out of 63 patients completed the caloric test with CP values greater than 30% in all. The ratio of patients completed cVEMP, oVEMP and vHIT were 46 cases, 22 cases and 30 cases, respectively. 17 out of 63 cases completed all the four vestibular function tests. According to these tests, 49 patients could determine the extent of injury，including 27 cases with unilateral superior vestibular nerve injury, 21 cases with unilateral superior and inferior vestibular nerve injury and 1 case with unilateral inferior vestibular nerve injury. There were significant differences in neutrophil value（P<0.001）, lymphocyte value（P<0.005）, neutrophil/lymphocyte ratio（P<0.001） and apolipoprotein A1（P<0.001） between patient group and control group. Inflammatory markers were risk factors for patients with vestibular neuritis. The OR values of neutrophil value and blood neutrophil/lymphocyte ratio were 1.81（1.38-2.37, P<0.001） and 2.11（1.41-3.16, P<0.001）, respectively. Apolipoprotein A1 was a protective factor for patients with vestibular neuritis, and the OR value was 0.004（0.001-0.042, P<0.001）. Conclusion:Electrocochleogram could be used in vestibular neuritis patients with normal pure tone threshold to test whether there is hidden hearing loss in these patients. Neutrophil value, lymphocyte value, neutrophil/lymphocyte ratio and apolipoprotein A1 were correlated with vestibular neuritis. The Neutrophil value and neutrophil/lymphocyte ratio were risk factors for morbidity.
Humans ; Vestibular Neuronitis/physiopathology* ; Retrospective Studies ; Female ; Male ; Audiometry, Pure-Tone ; Hearing Loss, Sensorineural/physiopathology* ; Middle Aged ; Adult ; Vestibular Function Tests ; Vestibular Evoked Myogenic Potentials ; Aged

Objective:This study aimed to compare the effects of cochlear implantation（CI） on vestibular function in patients with large vestibular aqueduct syndrome（LVAS） and in patients with extremely severe deafness with normal inner ear structure. Methods:A total of 28 LVAS patients and 28 patients with normal inner ear structure who suffered from extremely severe deafness were selected. The parameters of caloric tests, bone conduction evoked cervical vestibular-evoked myogenic potentials（cVEMP）, bone conduction evoked ocular vestibular-evoked myogenic potentials（oVEMP） and video head impulse tests（v-HIT） were compared between the two groups before and after CI. The data were analyzed using SPSS 26.0 software. Results:There was no significant difference in the results of the preoperative caloric test, v-HIT, and oVEMP（P1, N1） between the LVAS group and the control group（P>0.05）. Compared to the control group, the LVAS group exhibited a shorter cVEMP P1[（13.41±0.71）ms vs （16.28±0.89）ms, P<0.000 1], shorter N1[（19.83±0.54）ms vs （28.18±1.56）ms, P<0.000 1], higher amplitude[（123.60±83.80）μV vs （73.92±79.85）μV, P=0.049 4] and higher oVEMP amplitude[（16.60±13.87）μV vs （9.96±10.47）μV, P=0.028 5] before CI. The abnormal rate of caloric test increased in both groups after CI（25.00% vs 57.14%, P=0.028 8, 32.14% vs 82.14%, P=0.000 3, respectively）. There was no significant difference in the v-HIT parameters in both groups before and after the operation. As for the LVAS group, there was no statistically significant difference in cVEMP and oVEMP induction rates before and after operation. In the control group, there was a decrease in cVEMP induction rate（96.42% vs 64.28%, P=0.005 2） and oVEMP induction rate（96.42% vs 57.14%, P=0.000 9） after CI. LVAS group showed a shorter cVEMP P1[（13.41±0.71）ms vs （10.30±0.60）ms, P<0.000 1]; shorter cVEMP N1[（19.86±0.53）ms vs （18.97±1.33）ms, P=0.004 7]; decreased amplitude[（124.50±84.86）μV vs （64.35±61.57）μV, P=0.001 0] and shorter oVEMP amplitude[（15.92±13.03）μV vs （9.16±9.20）μV, P=0.009 9] after CI. The oVEMP N1 in the control group was longer than that before operation[（11.73 ± 0.91）ms vs （13.35 ± 2.60）ms, P=0.019 6], whereas there was no significant difference in other VEMP parameters after CI. Conclusion:Before CI, there was no significant difference in the results of the caloric test and v-HIT between the LVAS group and the control group, but the LVAS group exhibited increased sensitivity to acoustic stimulation-induced myogenic potentials. After CI, the function of the semicircular canal was impaired in both groups in the low-frequency area, and remained largely unaffected in the high-frequency area. Additionally, the function of the otolith in the LVAS group was less affected than that in the control group after CI, which may be related to the fact that the enlarged vestibular aqueduct of the LVAS patients acted as the third window of the inner ear.
Humans ; Vestibular Aqueduct/physiopathology* ; Cochlear Implantation ; Male ; Female ; Vestibular Evoked Myogenic Potentials ; Deafness/physiopathology* ; Child ; Adolescent ; Adult ; Young Adult ; Hearing Loss, Sensorineural/physiopathology* ; Vestibular Function Tests

Objective:This study aims to investigate the mechanism and potential effects of two exposures to 100 dB sound pressure level（SPL） broadband white noise, with a 14-days interval, on the myelin sheath of the cochlear auditory nerve in mice. The research provides experimental evidence for understanding the pathophysiological processes of noise-induced hearing loss and hidden hearing loss. Methods:Fifteen 6-week-old male C57BL/6J mice with normal hearing thresholds were randomly divided into three groups: a control group（no noise exposure）, a single noise exposure group, and a double noise exposure group. The single noise exposure group was exposed to 100 dB SPL white noise for 2 hours, and ABR thresholds were measured 1 day（P1） and 14 days（P14） after the exposure. The double noise exposure group was exposed to the same conditions of 100 dB SPL white noise for 2 hours, followed by a second identical exposure 14 days later. ABR thresholds were measured 1 day（P15） and 14 days（P28） after the second exposure. The cochleae of all three groups were then collected for immunofluorescence observation of the basilar membrane and transmission electron microscopy to observe changes in the structure of the auditory nerve myelin sheath. Results:In the single noise exposure group, ABR thresholds at all frequencies were significantly elevated compared to the control group at P1. There were no significant changes in ABR thresholds at any frequency at P14. In the double noise exposure group, ABR thresholds at all frequencies were significantly elevated compared to the control group at P15 and P28（P<0.001）. After the first noise exposure, immunofluorescence observation revealed no significant weakening of the auditory nerve myelin sheath signal; transmission electron microscopy showed no significant changes in myelin sheath morphology. However, after the second noise exposure, immunofluorescence observation revealed a weakening of the myelin sheath signal, and transmission electron microscopy showed thinning of the myelin sheath, disruption of the lamellar structure, and separation from the axon, indicating demyelination. Conclusion:Two exposures to 100 dB SPL broadband white noise can lead to damage to the auditory nerve myelin sheath in mice, whereas a single exposure does not cause significant changes.
Animals ; Male ; Myelin Sheath/pathology* ; Mice ; Cochlear Nerve/pathology* ; Mice, Inbred C57BL ; Noise/adverse effects* ; Hearing Loss, Noise-Induced/physiopathology* ; Cochlea ; Evoked Potentials, Auditory, Brain Stem

Objective:To investigate the impact of Waardenburg syndrome（WS） -associated Sox10 p.S100Rfs*9 mutation on inner ear function and its mechanism in noise-induced hearing impairment. Methods:A mice model carrying the Sox10 p.S100Rfs*9 mutation was established using CRISPR-Cas9 gene editing technology. Auditory phenotypes were assessed under baseline conditions and after noise exposure（96 dB SPL, 2 hours）. Auditory brainstem response（ABR） tests were performed to evaluate hearing function, combined with immunofluorescence staining of cochlear basilar membrane whole-mounts to observe hair cells and ribbon synapses. Transcriptome sequencing was conducted to analyze molecular mechanisms. Results:Sox10 p.S100Rfs*9 heterozygous mice exhibited normal hearing thresholds with characteristic ventral pigmentation abnormalities under baseline conditions. Following noise exposure, mutant mice showed significantly higher ABR thresholds at 24 000 Hz compared to wild-type controls（[60.00±6.12]vs[48.13±4.28]dB SPL, P<0.000 1）, and a significant reduction in ribbon synapses（CtBP2-positive puncta） in the basal turn（[55.0±2.3]vs[64.8±3.3]per inner hair cell, P=0.006 6）, while hair cell morphology and number remained intact. Transcriptome analysis revealed altered expression of genes involved in immune regulation, membrane structures, ion channels, and neuroactive ligand-receptor interactions. Conclusion:The Sox10 p.S100Rfs*9 mutation does not alter baseline hearing function but significantly increases inner ear susceptibility to noise damage, primarily manifested as enhanced ribbon synapse vulnerability, especially in high-frequency regions. This gene-environment interaction reveals that Sox10 haploinsufficiency may compromise noise tolerance by affecting synaptic stability and inner ear protective mechanisms. These findings provide new perspectives on the phenotypic heterogeneity in WS patients and theoretical basis for individualized noise protection strategies for patients carrying SOX10 mutations.
Animals ; SOXE Transcription Factors/genetics* ; Waardenburg Syndrome/physiopathology* ; Mice ; Hearing Loss, Noise-Induced/genetics* ; Evoked Potentials, Auditory, Brain Stem ; Mutation ; Noise ; Disease Models, Animal ; Ear, Inner/physiopathology*

Objective:To explore various factors influencing speech recognition ability in patients with age-related hearing loss（ARHL） and to investigate the correlation between speech recognition ability and cognitive function. Methods:This case-control study enrolled 150 ARHL patients（experimental group） and 132 normal-hearing controls. Participants underwent relevant assessments of auditory function, cognitive function, and tinnitus severity. Various statistical analyses were performed to evaluate the results. Results:①The PBmax and MoCA scores were significantly lower in the ARHL group compared to the control group（P<0.05）. ②PBmax in the ARHL group was significantly influenced by multiple factors（P<0.05）. ③Negative correlations were observed between PBmax in the ARHL group and age, degree of hearing loss, duration of the disease, duration of the worst hearing loss, smoking status, and tinnitus severity（P<0.05）, while positive correlations were found between PBmax and education level, occupation type, frequency of verbal communication, and cognitive function level（P<0.05）. ④Higher education level, frequent verbal communication, and high cognitive function level were protective factors for PBmax in ARHL patients（P<0.05）, whereas the other factors were independent risk factors（P<0.05）. ⑤A significant correlation was found between PBmax and MoCA scores in the ARHL group, and this correlation between cognitive function and speech recognition ability remained significant across different degrees of hearing loss（<0.05）. Conclusion:Speech recognition ability in ARHL patients is influenced by multiple factors. Cognitive function demonstrates a robust, bidirectional association with speech recognition ability, even after adjusting for hearing loss severity.
Humans ; Case-Control Studies ; Middle Aged ; Male ; Female ; Aged ; Speech Perception ; Cognition ; Presbycusis/physiopathology* ; Adult ; Hearing Loss

Hearing loss is one of the most prevalent sensory disorders affecting the human nervous system. Liquid-liquid phase separation (LLPS) is a physiological process that facilitates the reversible and dynamic assembly of biomolecular condensates. Increasing evidence suggests that LLPS plays a significant role in the pathogenesis of hereditary hearing loss. Nevertheless, there is a conspicuous lack of systematic investigations exploring the impact of LLPS abnormalities on the etiology of hereditary hearing loss. In this review, we examine the mechanisms by which dysfunctions in LLPS contribute to hereditary hearing loss, specifically focusing on its effects on mechanoelectrical transduction in hair bundles, transcriptional regulation, post-transcriptional modifications, the actin cytoskeleton, ion homeostasis within the inner ear, and energy and redox homeostasis. Furthermore, we evaluate the considerable potential of targeting LLPS as a therapeutic approach for hearing loss and propose innovative perspectives on LLPS that may guide future research initiatives in the field of auditory disorders.
Humans ; Animals ; Hearing Loss/physiopathology* ; Phase Separation

Objective:To investigate the application value of NB CE-Chirp ASSR in the assessment of hearing threshold in children with hearing impairment. Methods:The hearing test results of 44 children with sensorineural hearing loss（88 ears） diagnosed in Shanghai Children's Hospital from January 2023 to December 2023 were retrospectively studied, including 19 ears in mild hearing loss group, 26 ears in moderate group, 20 ears in severe group and 23 ears in extremely severe group. Correlations between auditory brainstem response（ABR） thresholds, pure tone audiometry（PTA） thresholds and NB CE-Chirp ASSR response thresholds in different hearing loss groups were analyzed. Results:The threshold difference between ABR and NB CE-Chirp ASSR（2 000-4 000 Hz） was between 0.50-4.08 dB in different degrees of hearing loss group. There was a significant correlation between NB CE-Chirp ASSR and ABR response threshold in each group（P<0.001）, and the correlation coefficients of mild, severe and extremely severe groups were all greater than 0.8, showing a strong correlation. The average threshold difference between PTA and NB CE-Chirp ASSR in each group was between 1.88-3.91 dB. The correlation coefficients were all greater than 0.8, showing a strong correlation. The difference between PTA and NB CE-Chirp ASSR response threshold at 500, 1 000, 2 000 and 4 000 Hz in each groupwas between 0.25-3.84 dB. With the increase of frequency, the correlation coefficient between PTA and NB CE-Chirp ASSR response threshold showed an upward trend. The frequency with the highest coefficient was 4 000 Hz, and the r values were all greater than 0.9, followed by 2 000 Hz, and the correlation coefficient was relatively low at 500 Hz. In the extremely severe group, each frequency coefficient was greater than 0.7, showing a strong correlation. Conclusion:NB CE-Chirp ASSR has good frequency specificity and can reliably assess hearing thresholds in hearing-impaired children.
Humans ; Auditory Threshold ; Child ; Retrospective Studies ; Female ; Male ; Audiometry, Pure-Tone/methods* ; Evoked Potentials, Auditory, Brain Stem ; Child, Preschool ; Hearing Loss, Sensorineural/physiopathology* ; Adolescent

Objective:This study aimed to analyze the results of auditory and vestibular function tests in patients with unilateral vestibular schwannoma and explore their association with tumor size. Methods:Clinical data from 81 patients diagnosed with unilateral vestibular schwannoma who underwent pure-tone audiometry（PTA）, cervical and ocular vestibular evoked myogenic potentials（c/oVEMP）, as well as video head impulse test（vHIT）, and subsequently underwent surgical treatment, were retrospectively analyzed. Patients were categorized into groups based on tumor size: small （≤ 15 mm）, medium（16-30 mm）, and large （>30 mm）, determined by the maximum tumor diameter on contrast-enhanced MRI scans. Results:PTA results indicated hearing loss in 73 cases（90.1%）; vestibular function tests revealed abnormal rates for the anterior semicircular canal, horizontal semicircular canal, posterior semicircular canal, utricle, and saccule at 29.6%, 77.8%, 54.3%, 90.1%, and 92.6%, respectively. Statistically, no significant differences were found in preoperative hearing test results among patients in different groups（F=0.393, P=0.676）. However, significant differences were observed in horizontal semicircular canal gain（r=-0.248, P=0.025）, abnormal rates of horizontal semicircular canal catch-up saccades（r=0.507, P<0.001）, as well as cVEMP（χ²=15.111, P=0.004） and oVEMP thresholds（χ²=18.948, P<0.001） across varying tumor size groups. Conclusion:The extent of hearing loss in patients with vestibular schwannoma is not correlated with tumor size, whereas the degree of vestibular dysfunction demonstrates a correlation with tumor size.
Humans ; Neuroma, Acoustic/physiopathology* ; Retrospective Studies ; Audiometry, Pure-Tone ; Vestibular Function Tests ; Vestibular Evoked Myogenic Potentials ; Female ; Male ; Head Impulse Test ; Middle Aged ; Adult ; Semicircular Canals/physiopathology* ; Aged ; Hearing Loss/physiopathology*

BACKGROUNDMitochondrial diseases are a group of energy metabolic disorders with multisystem involvements. Variable clinical features present a major challenge in pediatric diagnoses. We summarized the clinical spectrum of m.3243A>G mutation in Chinese pediatric patients, to define the common clinical manifestations and study the correlation between heteroplasmic degree of the mutation and clinical severity of the disease.

METHODSClinical data of one-hundred pediatric patients with symptomatic mitochondrial disease harboring m.3243A>G mutation from 2007 to 2013 were retrospectively reviewed. Detection of m.3243A>G mutation ratio was performed by polymerase chain reaction (PCR)-restriction fragment length polymorphism. Correlation between m.3243A>G mutation ratio and age was evaluated. The differences in clinical symptom frequency of patients with low, middle, and high levels of mutation ratio were analyzed by Chi-square test.

RESULTSSixty-six patients (66%) had suffered a delayed diagnosis for an average of 2 years. The most frequent symptoms were seizures (76%), short stature (73%), elevated plasma lactate (70%), abnormal magnetic resonance imaging/computed tomography (MRI/CT) changes (68%), vomiting (55%), decreased vision (52%), headache (50%), and muscle weakness (48%). The mutation ratio was correlated negatively with onset age (r = -0.470, P < 0.001). Myopathy was more frequent in patients with a high level of mutation ratio. However, patients with a low or middle level of m.3243A>G mutation ratio were more likely to suffer hearing loss, decreased vision, and gastrointestinal disturbance than patients with a high level of mutation ratio.

CONCLUSIONSOur study showed that half of Chinese pediatric patients with m.3243A>G mutation presented seizures, short stature, abnormal MRI/CT changes, elevated plasma lactate, vomiting, and headache. Pediatric patients with these recurrent symptoms should be considered for screening m.3243A>G mutation. Clinical manifestations and laboratory abnormalities should be carefully monitored in patients with this point mutation.

Adolescent ; Age of Onset ; Asian Continental Ancestry Group ; Chi-Square Distribution ; Child ; Child, Preschool ; DNA, Mitochondrial ; genetics ; Female ; Hearing Loss ; pathology ; physiopathology ; Humans ; Infant ; Lactic Acid ; blood ; Magnetic Resonance Imaging ; Male ; Mitochondrial Diseases ; blood ; genetics ; pathology ; physiopathology ; Mutation ; Point Mutation ; genetics ; Polymerase Chain Reaction ; Retrospective Studies ; Seizures ; pathology ; physiopathology

BACKGROUNDMeniere's disease is a unique, progressive disease of the inner ear. The complex manifestation presents diagnostic challenges. The cochlear symptoms often present before vertigo and tend to be ignored. This study aimed to analyze the characteristics of cochlear symptoms and functions associated with Meniere's disease to investigate the regularity of the development of this disorder.

METHODSOne-hundred fifteen patients who were diagnosed with definite unilateral Meniere's disease at the Hearing and Vestibular Clinic of the Department of Otorhinolaryngology of Beijing Tongren Hospital from August 2013 to November 2015 were recruited in this retrospective study. Initial symptoms, duration from initial symptoms to the diagnosis, hearing thresholds, audiogram patterns, and caloric test results were collected and analyzed for each patient. Data were analyzed using SPSS 13.0 statistical software by Spearman's correlation, Kruskal-Wallis H test, Chi-square test, and Fisher's exact test.

RESULTSThe average hearing threshold of these patients was 45.24 ± 18.40 dB HL. A majority of the patients (55.65%) were in Stage 3. The initial presentation of the disorder in 58 cases (50.43%) comprised only cochlear symptoms without vertigo. A weak, positive correlation was found between the degree of hearing loss and duration of the disease from initial symptoms to the diagnosis (rs = 0.288, P = 0.002). Upward-sloping, inverted "V," downward-sloping, and flat pattern were the main audiometric patterns observed with a distinctive distribution between stages (P < 0.001). Based on the configurations of audiograms, the audiometric patterns had a weak correlation to the duration (rs = 0.269, P = 0.004), and there was a tendency of duration to rising from upward-sloping, inverted "V", downward-sloping to flat pattern. (H = 10.024, P = 0.018). Frequencies of tinnitus in 56 patients (64.4%) were at the lowest points of the audiograms, i.e., the frequencies of the poorest hearing threshold. The patients at an advanced stage (Stage 3 [56] and Stage 4 [73]) exhibited a significantly higher abnormality of canal paresis than those at the earlier stages (Stage 1 [23] and Stage 2 [42]) (χ2 = 5.973, P = 0.015).

CONCLUSIONSPatients with definite Meniere's disease always have a moderate to severe sensorineural hearing loss before diagnosis. Cochlear symptoms are the most common initial presentation. With the progression of the duration, the hearing impairment becomes more severe and the distribution of the audiometric pattern is distinctive between stages.

Adolescent ; Adult ; Aged ; Caloric Tests ; Cochlea ; physiopathology ; Female ; Hearing Loss ; diagnosis ; physiopathology ; Humans ; Male ; Meniere Disease ; diagnosis ; physiopathology ; Middle Aged ; Retrospective Studies ; Tinnitus ; diagnosis ; physiopathology ; Vertigo ; diagnosis ; physiopathology ; Young Adult