Objective:To analyze genetic factors and phenotype characteristics in pediatric population with slight-to-moderate sensorineural hearing loss. Methods:Children with slight-to-moderate sensorineural hearing loss of and their parents, enrolled from the Chinese Deafness Genome Project, were studied. Hearing levels were assessed using pure tone audiometry, behavioral audiometry, auditory steady state response（ASSR）, auditory brainstem response（ABR） thresholds, and deformed partial otoacoustic emission（DPOAE）. Classification of hearing loss is according to the 2022 American College of Medical Genetics and Genomics（ACMG） Clinical Practice Guidelines for Hearing Loss. Whole exome sequencing（WES） and deafness gene Panel testing were performed on peripheral venous blood from probands and validations were performed on their parents by Sanger sequencing. Results:All 134 patients had childhood onset, exhibiting bilateral symmetrical slight-to-moderate sensorineural hearing loss, as indicated by audiological examinations. Of the 134 patients, 29（21.6%） had a family history of hearing loss, and the rest were sporadic patients. Genetic causative genes were identified in 66（49.3%） patients. A total of 11 causative genes were detected, of which GJB2 was causative in 34 cases（51.5%）, STRC in 10 cases（15.1%）, MPZL2 gene in six cases（9.1%）, and USH2A in five cases（7.6%）.The most common gene detected in slight-to-moderate hearing loss was GJB2, with c. 109G>A homozygous mutation found in 16 cases（47.1%） and c. 109G>A compound heterozygous mutation in 9 cases（26.5%）. Conclusion:This study provides a crucial genetic theory reference for early screening and detection of mild to moderate hearing loss in children, highlighting the predominance of recessive inheritance and the significance of gene like GJB2, STRC, MPZL2, USH2A.
Humans ; Child ; Connexins/genetics* ; Connexin 26/genetics* ; Hearing Loss, Sensorineural/diagnosis* ; Mutation ; Usher Syndromes ; Hearing Loss, Bilateral ; Audiometry, Pure-Tone ; Intercellular Signaling Peptides and Proteins

Hyperlipidemia is characterized by elevated levels of blood lipids. The clinical manifestations are mainly atherosclerosis caused by the deposition of lipids in the vascular endothelium. The link between abnormal lipid metabolism and sudden hearing loss remains unclear. This article presents a case study of sudden hearing loss accompanied by familial hyperlipidemia. Pure tone audiometry indicated intermediate frequency hearing loss in one ear. Laboratory tests showed abnormal lipid metabolism, and genetic examination identified a heterozygous mutation in theAPOA5 gene. Diagnosis: Sudden hearing loss; hypercholesterolemia. The patient responded well to pharmacological treatment. This paper aims to analyze and discuss thepotential connection between abnormal lipid metabolism and sudden hearing loss.
Humans ; Audiometry, Pure-Tone ; Deafness/complications* ; Hearing Loss, Sensorineural/diagnosis* ; Hearing Loss, Sudden/diagnosis* ; Hyperlipidemias/complications* ; Lipids

Objective: To analyze the surgical efficacy of patients with mixed hearing loss and otosclerosis with different air bone gap (ABG) before surgery, and to provide reference for the prognosis evaluation of otosclerosis surgery. Methods: The clinical data of 108 cases(116 ears) of otosclerosis who had undergone stapes fenestration technique artificial stapes implantation in Xiangya Hospital of Central South University from November 2013 to May 2020 and had mixed hearing loss before surgery were collected, including 71 women(76 ears)and 37 men (40 ears), with an average age of 38.5 years. According to preoperative pure tone audiometry ABG, they were divided into three groups: group S, 15 dB≤ABG<31 dB, a total of 39 ears; group M, 31 dB≤ABG<46 dB, a total of 58 ears; and group L, ABG≥46 dB, 19 ears in total. The hearing outcomes of three groups of patients at 6-12 months after surgery were compared and analyzed using SPSS 24.0 statistical software. Results: A total of 3 patients (group S: 2 cases; group L: 1 case) experienced severe sensorineural hearing loss after surgery and were not included in the statistical analysis. After surgery, the pure tone hearing threshold of patients with otosclerosis in each group was significantly improved compared to before surgery, with an average air conduction threshold improvement of(21.6±13.4) dB. The difference between before and after surgery was statistically significant(t=17.13, P<0.01). The average bone conduction threshold improved by(3.7±7.6) dB, and the difference was statistically significant before and after surgery(t=5.20, P<0.01). The postoperative ABG was(18.3±9.3) dB, which was significantly reduced compared to preoperative(36.2±8.6)dB. Among the three groups of patients, the L group had the highest improvement in air conduction threshold[(29.9±10.8)dB], while the S group had the lowest improvement[(15.7±11.4)dB]. There was no statistically significant difference in post operative pure tone hearing thresholds between the three groups(P>0.05). The postoperative ABG in group S was the smallest[(16.5±9.0)dB], while in group L, the postoperative ABG was the largest[(20.5±10.0)dB]. Compared with group S, group M and group L still had a large residual ABG at 2 000 Hz after surgery. The bone conduction threshold of both S and M groups improved to some extent after surgery compared to before (P<0.01). Conclusions: Surgery can benefit patients with mixed hearing loss and otosclerosis with different preoperative ABG. Patients with small preoperative ABG have better surgical results and ideal ABG closure at all frequencies after surgery. Patients with large preoperative ABG can significantly increase the gas conduction threshold during surgery, but certain frequencies of ABG may still be left behind after surgery. The improvement effect of surgery on bone conduction threshold is not significant. Patients should be informed of treatment methods such as hearing aids based on their actual situation for selection.
Male ; Humans ; Female ; Adult ; Bone Conduction ; Otosclerosis/surgery* ; Hearing Loss, Mixed Conductive-Sensorineural/surgery* ; Stapes Surgery/methods* ; Treatment Outcome ; Auditory Threshold ; Hearing ; Audiometry, Pure-Tone ; Deafness ; Retrospective Studies

Objective: To determine the accuracy, sensitivity, specificity, positive predictive values, and use of the Reflexive Behavioral “Baah” Test and NHSRC Level 1 and Level 2 Questionnaires in detecting hearing impairment in rural health communities. Methods: This was a prospective cross-sectional study conducted at the rural health unit of five municipalities. Infants less than six months old were screened for hearing impairments using the OAE device (standard), the Reflexive Behavioral “Baah” test, and the NHSRC Level 1 and Level 2 Questionnaires. The “Baah” test and the filling out of the NHSRC Level 1 and 2 Questionnaires were done by trained health workers while OAE was done by an audiologist. Results: A total of 103 babies, with a mean age of 41.9 days at the time of testing and a male to female ratio of 1.02:1 (52 males and 51 females) were tested. A hearing impairment prevalence of 4.9% (5 out of 103) was noted. The “Baah” test showed to have a sensitivity of 60%, specificity of 97.96% and an accuracy rate of 96.12%. The NHSRC Level 1 and Level 2 Questionnaires showed sensitivity, specificity, and accuracy rate of 40%, 67.35% and 66.02%, respectively for the former and 40%, 85.71% and 83.50%, respectively for the latter. Analysis of the complimentary use of the NHSRC Level 1 and Level 2 Questionnaires with the “Baah” test also showed no significant improvement to using the “Baah” test as a stand-alone screening tool with sensitivity, specificity, and accuracy of 60%, 67.35% and 66.99%, respectively for the “Baah” test and Level 1 Questionnaire, and 60%, 83.67% and 82.52%, respectively for the “Baah” test and Level 2 Questionnaire. Conclusion The Reflexive Behavioral “Baah” test is a potentially accurate, sensitive, specific, and acceptable standalone hearing screening test to identify infants with higher risk of hearing impairment in the rural health community setting. On the other hand, the use of the NHSRC Questionnaires as a stand-alone or complementary tool for “Baah” is unnecessary as it results to more false positive and false negative results.
Surveys and Questionnaires ; Infant, Newborn ; Audiometry ; Behavior Rating Scale

Objective: To assess the usage of the “Baah” Test compared to the AABR (Automated Auditory Brainstem Response) in detecting hearing loss of neonates in the community setting. Methods: This is a retrospective cross-sectional study. The targeted sample population are infants less than a month old who underwent screening at a testing facility in Malolos, Bulacan spanning the years 2011 and 2012. Results: A total of 201 infants were included in the study, with a mean age of 10.77 days with a standard deviation of 7.79. The ratio of males to females was almost equal at 1:1.01. For infants who passed hearing screening on at least one ear, 96% (193 infants) correlated with the results of “Baah” testing. For those with bilateral refer results on AABR, 4 out of the 6 correlated with the “Baah” Test. Conclusion There is potential in using the “Baah” Test as a tool for hearing loss assessment of infants in situations wherein the usual hearing screening tests are inaccessible. It makes use of little resources, and though it does have its limitations in assessing for unilateral hearing loss (as the test cannot test ears in isolation), it would be able to identify infants likely to have bilateral hearing loss.
Audiometry, Evoked Response ; Infant, Newborn

Objective: To systematically review the available evidence on the association of HBA1c levels and development of sensorineural hearing loss and to quantitatively analyze the available data on HBA1c levels in patients with type 2 diabetes mellitus and sensorineural hearing loss to determine an HbA1c level that may be associated with the risk of having sensorineural hearing loss. Methods: Design: Systematic Review and Meta-analysis Eligibility Criteria: Cross-sectional studies, or cohort studies which were limited to English language that investigated the correlation of glycemic index using HBA1c and sensorineural hearing loss among adult type 2 diabetic patients which were done from January 2010 to December 2021. Studies with no published outcome, incomplete data or that were ongoing as of August 1, 2022 were also excluded. Information Sources: MEDLINE (through PubMed), Cochrane Library, Scopus, Embase (through OVID@journal), Directory of Open Access Journals (DOAJ), Google Scholar and HERDIN Plus Risk of Bias: Risk of Bias was assessed using the Guidelines for Cochrane Collaboration Synthesis of Results: Results were presented using forest plots for representation. Results: A total of 8 studies were reviewed with 2,103 participants in all. Six articles compared hearing loss incidence between diabetic and non-diabetic patients. Overall, there were a total of 881 diabetic patients and 1222 non-diabetic patients. There was a significantly lower incidence of sensorineural hearing loss in non-diabetic patients with a risk ratio of 1.89, 95% CI [1.65, 2.16]. Three articles compared the HbA1c levels of diabetic patients with or without sensorineural hearing loss. Diabetic patients without sensorineural hearing loss had significantly lower HbA1c levels compared to those with sensorineural hearing loss with mean difference of 1.04, 95%CI [0.82, 1.25]. Conclusion In conclusion, this meta-analysis showed a higher prevalence rate of sensorineural hearing loss among patients with diabetes mellitus compared to non-diabetic patients. Moreover, poor glycemic control among diabetic patients with a glycemic index based on HbA1c of more than 8.3 (6.97-9.6) is associated with sensorineural hearing loss.
diabetes mellitus ; glycemic index ; sensorineural hearing loss ; pure tone audiometry ; deafness

Objective: To explore the change of hearing threshold of workers exposed to noise, establish an individual-based hearing loss early warning model, accurately and differentiated the health of workers exposed to noise. Methods: In September 2019, all physical examination data of 561 workers exposed to noise from an enterprise were collected since their employment. Three indicators of average hearing threshold of the better ear, namely, at high frequency, 4000 Hz and speech frequency, were constructed. The generalized estimating equation (GEE) was used to adjust gender and age and establish the warning model of each indicator. Finally, sensitive indicators and warning models were screened according to AUC and Yoden index. Results: Among the 561 workers exposed to noise, 26 (4.6%) workers had hearing loss. The sensitivity indicators were the average hearing threshold at speech frequency ≥20 dB, high frequency ≥30 dB and 4000 Hz ≥25 dB. The AUC of each index was 0.602, 0.794 and 0.804, and the Youden indexes were 0.204, 0.588 and 0.608, respectively. In GEE of hearing loss warning models, high-frequency hearing threshold ≥20 dB and 4000 Hz hearing threshold ≥25 dB were the optimal models, with AUC of 0.862. Conclusion: Combined with the changes of individual hearing threshold over the years, can accurately assess the risk of individual hearing loss of workers exposed to noise.
Humans ; Hearing Loss, Noise-Induced/diagnosis* ; Noise, Occupational/adverse effects* ; Audiometry ; Deafness ; Employment ; Occupational Exposure/adverse effects* ; Occupational Diseases/diagnosis*

Objective: To clarify the long-term characteristics of tinnitus following treatment of sudden deafness and its long-term physical and mental effects on patients. Methods: A retrospective analysis was performed on 88 patients (46 males and 42 females; Age from 11 to 89 years) with sudden deafness treated in Department of Otoscope Surgery of Peoples's Libration Army General Hospital in Beijing from April 2020 to January 2021, and the occurrence of tinnitus and treatment effect of all patients were analyzed. Follow-up was conducted for patients with residual tinnitus after treatment for more than 1 year by the investigation and filling in the survey information collection form, Tinnitus Evaluation Questionnaire (TEQ) and Tinnitus Handicap Inventory (THI). Descriptive statistics and SPSS 22.0 software were used for statistical data analysis. Results: In this study, 93.2% (82/88) of patients with sudden deafness were accompanied by tinnitus at the onset, and the proportion of long-term tinnitus after treatment was 90.2% (74/82). After 1 year of treatment for sudden deafness, the improvement of tinnitus was significant in low-frequency sudden deafness compared with those of high-frequency, flat and total deafness sudden deafness (χ2 value was 6.801, 4.568 and 4.038, all P<0.05). In patients with residual tinnitus, 9 (12.2%) patients felt minimal loudness or even no loudness, 34 (46.0%) patients felt slight loudness, 28 (37.8%) patients felt tinnitus was relatively loud, and 3 (4.1%) patients felt tinnitus was loud or noisy. Nine (12.2%) patients's sleep was often affected, 41 (55.4%) patients's sleep was sometimes affected, 9 (12.2%) patients's sleep was rarely affected, 15 (20.3%) patients's sleep was almost not affected. Twenty-eight (37.8%) patients basically completely adapted to tinnitus and 46 (62.2%) patients did not completely adapted to residual tinnitus. Eight (10.8%) patients had no impact on life, 39 (52.7%) patients had slight impact, 22 (29.7%) patients had moderate impact, and the other 5 (6.8%) patients had greater impact. According to tinnitus evaluation questionnaire(TEQ), there were 12 cases (16.2%) of grade Ⅰ, 26 cases (35.1%) of grade Ⅱ, 28 cases (37.8%) of grade Ⅲ, 7 cases (9.5%) of grade Ⅳ and 1 case (1.4%) of grade Ⅴ. According to tinnitus handicap inventory(THI), tinnitus disability was classified into grade Ⅰ, 22 cases (29.7%), grade Ⅱ, 14 cases (18.9%), Grade Ⅲ, 27 cases (36.5%) and grade Ⅳ, 11 cases (14.9%). Conclusion: The rate of residual tinnitus following treatment of sudden deafness is high. Some of the patients can completely adapt residual tinnitus after one year, but some of them will be affected when sleep, work and study. Residual tinnitus can lead to tinnitus disability in different degrees.
Male ; Female ; Humans ; Child ; Adolescent ; Young Adult ; Adult ; Middle Aged ; Aged ; Aged, 80 and over ; Hearing Loss, Sudden/therapy* ; Tinnitus/therapy* ; Retrospective Studies ; Deafness/complications* ; Audiometry

Objective: To get insight into the current practice of noise reduction effect of workers as they wore hearing protectors in different domestic enterprises and the possible affected factors. Methods: From October 2020 to April 2021, using a random sampling method, 1197 workers exposed to noise in petrochemical factories, textile factories, and parts manufacturing factories were selected as the study subjects. The noise reduction effect of hearing protectors worn by workers in daily use was tested using a hearing protector suitability testing system. The personal sound attenuation level (PAR) was compared among workers in three enterprises, Targeted intervention and repetitive testing were conducted for workers who did not meet the noise reduction effect required by the enterprise, and the changes in PAR of workers before and after the intervention were compared. The comparison of baseline PARs between two or more groups was performed using the Mann Whitney test, the comparison of baseline PARs with post intervention PARs was performed using the Wilcoxon signed rank sum test, and the comparison of qualitative data between two or more groups was performed using the Chi square test. Results: The median baseline PAR for all workers was 15 dB. Men, age<30 years old, education level at or above college level, working experience of 5 to 15 years, and those who used hearing protectors for 5 to 15 years had higher PARs, with statistically significant differences (P<0.05). The median difference in baseline PAR among workers from three enterprises was statistically significant (H=175.06, P<0.01). The median PAR of subjects who did not pass the baseline increased from 3 dB to 21 dB after intervention (Z=-27.92, P<0.01) . Conclusion: Some workers wearing hearing protectors do not meet the required PAR, and low PARs may be related to incorrect wearing methods and incorrect selection of hearing protectors. As a tool for testing, training, and assisting in selection, the hearing protector suitability testing system is of great significance for worker hearing protection.
Male ; Humans ; Adult ; Hearing Loss, Noise-Induced/prevention & control* ; Ear Protective Devices ; Noise, Occupational/prevention & control* ; Hearing ; Audiometry

The qualitative, quantitative, and localization analysis of hearing loss is one of the important contents of forensic clinical research and identification. Pure-tone audiometry is the "gold standard" for hearing loss assessment, but it is affected by the subjective cooperation of the assessed person. Due to the complexity of the auditory pathway and the diversity of hearing loss, the assessment of hearing loss requires the combination of various subjective and objective audiometric techniques, along with comprehensive evaluation based on the case situation, clinical symptoms, and other examinations to ensure the scientificity, accuracy and reliability of forensic hearing impairment assessment. Objective audiometry includes acoustic impedance, otoacoustic emission, and various auditory evoked potentials. The frequency-specific auditory brainstem response (ABR), 40 Hz auditory event related potential, and auditory steady-state response are commonly used for objective hearing threshold assessment. The combined application of acoustic impedance, otoacoustic emission and ABR can be used to locate hearing loss and determine whether it is located in the middle ear, cochlea, or posterior cochlea. This article reviews the application value of objective audiometry techniques in hearing threshold assessment and hearing loss localization, aiming to provide reference for forensic identification of hearing loss.
Humans ; Reproducibility of Results ; Auditory Threshold/physiology* ; Evoked Potentials, Auditory, Brain Stem/physiology* ; Hearing Loss/diagnosis* ; Audiometry, Pure-Tone/methods* ; Clinical Medicine