The cause of idiopathic sudden sensorineural hearing loss (ISSNHL) remains unknown. It has been found that the functional status of the vestibular otolith is relevant to its prognosis; however, the evaluation of the vestibular otolith (intra-labyrinth) and superior and inferior vestibular nerve pathways (retro-labyrinth) in ISSNHL patients is not well-documented.This study aimed to investigate the functional status of the vestibular otolith and conductive pathway in patients with unilateral ISSNHL and analyze the correlations between vestibular evoked myogenic potentials (VEMPs) and hearing improvement after treatment.A total of 50 patients with unilateral ISSNHL underwent a battery of audio-vestibular evaluations, including pure tone audiometry, middle ear function, air-conducted sound-cervical VEMP (ACS-cVEMP), ACS-ocular VEMP (ACS-oVEMP), galvanic vestibular stimulation-cervical VEMP (GVS-cVEMP), and GVS-ocular VEMP (GVS-oVEMP). The results of auditory and VEMPs were retrospectively analyzed.p = 0.025), while it was similar between GVS-cVEMP and GVS-oVEMP (p = 0.218). Compared with GVS-cVEMP, affected ears presented with a significantly higher abnormal rate of ACS-cVEMP (p = 0.005), and the abnormal rate of ACS-oVEMP was significantly higher than that of GVS-oVEMP (p < 0.001). No significant difference existed in latency and amplitude between affected and unaffected ears in ACS-VEMPs or GVS-VEMPs (p > 0.05). The abnormal rate of VEMPs in the poor recovery group was significantly higher than that of the good recovery group (p = 0.040). The abnormality percentages of ACS-oVEMP and GVS-oVEMP in the poor recovery group were significantly higher than that of the good recovery group . The good hearing recovery rates were 76.47% in the normal VEMPs group, 58.33% in the intra-labyrinth lesion group, and 22.22% in the retro-labyrinth lesion group. Hearing recovery worsened as a greater number of abnormal VEMPs was presented.The abnormal rates of ACS-cVEMP, ACS-oVEMP, GVS-cVEMP, and GVS-oVEMP in affected ears were 30, 52, 8, and 16%, respectively. In affected ears, the abnormal rate of ACS-oVEMP was significantly higher than that of ACS-cVEMP (Besides Corti's organ, the impairment of otolithic organs was prominent in patients with ISSNHL. The normal VEMPs group had the highest rate of good recovery, followed by the intra-labyrinth lesion group and the retro-labyrinth lesion group presented with the lowest recovery rate. Abnormalities in ACS-oVEMP and/or GVS-oVEMP were indicators of a poor prognosis. Idiopathic sudden sensorineural hearing loss (ISSNHL) was defined as a sudden hearing loss that occurs within 72 h, and pure tone audiometry results show a decline at least in the three adjacent frequencies (>30 dBHL) without any identifiable cause , which mAlthough several etiologies of ISSNHL, such as viral infection, inflammation, inner ear circulation disorders, cochlear membrane breaks, and vascular occlusion, have been suggested, the exact pathogenesis is still unclear , 5. Due Considering that a high proportion of patients with ISSNHL have vestibular dysfunction, it is of great importance to evaluate vestibular function in these populations. The most commonly used tests include the caloric test, cervical vestibular evoked myogenic potential (cVEMP), and ocular VEMP (oVEMP), as well as video head impulse test (vHIT) –16. The A retrospective study was performed on 50 patients with ISSNHL who were hospitalized at the Department of Otolaryngology-Head and Neck Surgery, Xinhua Hospital, affiliated with Shanghai Jiao Tong University School of Medicine from October 2019 to March 2023, including 26 male participants and 24 female participants aged between 8 and 75 years (an average of 45.87 ± 20.91 years). There were 28 ears with severe hearing loss and 22 ears with profound hearing loss. For the entire cohort, 28 patients had hearing loss in the left ear and 22 in the right ear, and 25 patients had vertigo. Patients were included if the results of VEMPs were normal on the contralateral unaffected ear, which could eliminate the influence of advanced age.The inclusion criteria were as follows: (1) unilateral sudden sensorineural hearing loss without apparent cause at least in the adjacent three-frequency hearing loss of ≥30 dB HL in 72 h; (2) initiation of treatment within 20 days after onset; (3) underwent all the required tests; (4) Type A tympanogram in both ears; and (5) the same comprehensive treatment plan was used during hospitalization. The exclusion criteria were as follows: (1) abnormal results of VEMPs on the contralateral healthy ear; (2) external and middle ear diseases; (3) space-occupying lesions of the internal auditory canal and central organic pathology; and (4) sensorineural hearing loss due to noise exposure or ototoxic drugs.A tympanogram was obtained by the Interacoustics AT235H Middle Ear Analyzer . Type A at 226 Hz probe tone was considered a normal middle ear function.Pure-tone audiometry was conducted in a soundproof room using an audiometer . The pure-tone average (PTA) is the average of the 0.5, 1, 2, and 4 kHz pure-tone thresholds. According to the latest standards of the World Health Organization, PTA <20 dB HL is defined as normal hearing. Repeated pure-tone audiometry was carried out before and after the 10-day treatment. The hearing outcome was classified as good recovery (referring to PTA gain ≥15 dB HL) and poor recovery (hearing improvement <15 dB HL). If no response was obtained for a certain frequency, which exceeded the maximum output of the audiometer (120 dB HL), 120 dB HL was used as the estimated hearing threshold.ACS-VEMPs were recorded by the electrophysiological device . A sound stimulus of Tone-Burst 500 Hz  at 132 dB peSPL was presented monaurally through a calibrated headphone TDH-39 at a rate of 5 Hz. A minimum of 100 sweeps were averaged and at least repeated twice to verify the waveform repeatability. The electromyogram (EMG) signals were amplified and bandpass filtered between 10 and 3,000 Hz.For ACS-cVEMP, the two recording electrodes were placed on the upper third of the bilateral sternocleidomastoid muscles (SCMs). The two reference electrodes were placed on the sternal end of the SCM. Then the ground electrode was placed in the middle of the forehead. Patients were asked to rotate their heads toward the shoulder in a sitting position, keeping the SCMs activated and tense until the stimulus sound stopped.ACS-oVEMP was also performed in a sitting position. The two recording electrodes were placed 1 cm below the middle of the contralateral lower eyelid, the reference electrodes were placed below the same side of the recording electrodes, and the ground electrode was placed in the middle of the forehead. Patients were required to maintain eye gaze upward for 25–30° when hearing a single acoustic stimulus and minimize blinking to maintain tension in the inferior oblique muscle until the stimulation stopped.GVS-VEMPs were performed by the same device. The electrode placement of GVS-VEMPs was similar to that of ACS-VEMPs, but there was a set of cathode and anode electrodes for direct current stimulation. The cathode of direct current stimulation was placed at the mastoid, and the anode was placed over the forehead .GVS-cVEMP: The initial stimulation intensity was 3.0 mA/1 ms . The waveform of muscle relaxation was subtracted from the waveform of muscle contraction to eliminate the artifact of the mechanical wave and obtain the final waveform. The method of muscle contraction was the same as in ACS-cVEMP.GVS-oVEMP: The initial stimulation intensity was 3.0 mA/1 ms . The waveforms of the extraocular muscles were recorded during upward gaze (extraocular muscles contraction) and downward gaze (extraocular muscles relaxation), and the final GVS-oVEMPs waveform was obtained by subtracting the waveform of muscle relaxation from muscle contraction.To verify the repeatability of the waveform, the process was repeated at least twice. If 3.0 mA cannot elicit repeatable waveforms, the stimulation intensity can be appropriately increased according to the patient's tolerance level but usually does not exceed 5.0 mA. Characteristics of latencies, amplitudes, and the interaural asymmetry ratio (IAR) were recorded. IAR = (AL-AS)/(AL+AS) × 100%, where AL is the larger corrected amplitude and AS is the smaller corrected amplitude , 24, 26.t-test. Latencies and amplitudes of various modes of VEMPs were determined by independent t-test for parametric variables and Mann-Whitney U-test for non-parametric variables. A chi-square test with Bonferroni correction was applied to evaluate the good hearing recovery rate among different groups. Significance was determined at p < 0.05.All statistical analyses were performed using SPSS 26 . A chi-square test was used to evaluate the demographics of the two groups of ISSNHL patients and the abnormal rates of various VEMPs. The pre-treatment and post-treatment PTAs were compared using the paired U-tests in the two groups are shown in p > 0.05). However, the number of patients accompanied by vestibular symptoms and the presence of abnormal VEMPs were significantly higher in the PR group .According to the inclusion criteria, 50 patients with severe to profound ISSNHL were enrolled in this study. Based on the hearing recovery, they were categorized into two groups, namely, the good recovery (GR) group and the poor recovery (PR) group, with 29 cases (58%) in the GR group and 21 cases (42%) in the PR group. p = 0.005). Similar to the results of cVEMP, the abnormal rate of ACS-oVEMP significantly exceeded that of GVS-oVEMP in affected ears (p < 0001). No significant difference existed between the abnormal rates of GVS-cVEMP and GVS-oVEMP (p = 0.218). However, the abnormal rate of ACS-oVEMP was significantly higher than that of ACS-cVEMP (p = 0.025).As shown in p > 0.05).The descriptive data, including the mean and standard deviation (SD) of the latency and amplitude of VEMPs in affected and unaffected ears, were displayed in p = 0.004 and 0.039, respectively). However, no significant difference was observed in terms of abnormality percentage in ACS-cVEMP, GVS-cVEMP, or normal VEMPs between the GR and PR groups . Additionally, 24 affected ears suffered from intra-labyrinth lesion, including three with saccule dysfunction, 13 with utricle dysfunction, and eight with abnormal function both in the saccule and utricle; nine affected ears showed lesions in retro-labyrinth; and 17 affected ears had normal function in the vestibular otolith and conductive pathway. The good hearing recovery rates are shown in According to the VEMP results, vestibular dysfunction locations were categorized into intra-labyrinth and retro-labyrinth lesions. The normal VEMPs group refers to normal results in ACS-VEMPs and GVS-VEMPs. Intra-labyrinth lesion refers to abnormal ACS-cVEMP and/or ACS-oVEMP but normal GVS-cVEMP and GVS-oVEMP. Retro-labyrinth lesion refers to abnormal GVS-cVEMP and/or GVS-oVEMP. As shown in According to the results, the rate of abnormalities in ACS-VEMPs was greater than in GVS-cVEMPs, suggesting that the otolith organs may be involved more frequently than vestibular afferents, which was consistent with previous studies , 22–25. A recent meta-analysis reported that the utricle was the most easily affected organ in ISSNHL , which wHowever, some researchers have reported inconsistent results. It has been found that vestibular dysfunction in patients with ISSNHL affects the vestibular organs close to the cochlea first . AtrophiIt has been documented that VEMP test results have predictive value for hearing outcomes in SSNHL patients. Several recent reports have included the VEMP test in the evaluation of patients with ISSNHL, for whom abnormal results of vestibular examinations were associated with poor hearing recovery , 32, 33.The improvement of the pure tone threshold is currently a common and international outcome index , 35. We The cause of this discrepancy may possibly relate to the following factors. First, the degree of hearing loss, accompanying symptoms (vertigo and tinnitus), delays after the onset of hearing loss, and etiology can lead to a significant risk of selection bias and unmatched groups. The extent of vestibular abnormalities correlated well with the degree of hearing loss , 32. NeaAlthough some valuable results were achieved, there were still some limitations in our study. First, we focused on a subgroup of ISSNHL patients with severe to profound hearing loss, so the effect of the degree of hearing loss was not mentioned in this study, which should be further studied on a large sample scale. Second, VEMPs do not reflect canal function; a comprehensive evaluation is required in combination with other vestibular tests. Due to the insufficient sample size of this study, it was not grouped according to etiology, medical diseases, etc. The effect of these potential factors on hearing prediction should be incorporated into statistical analysis in future studies with larger sample sizes. In addition, we could also follow up on hearing and vestibular recovery at 1 and 3 months after treatment, making the study more comprehensive.There were significant differences in the recovery of hearing loss in patients with ISSNHL. Patients with abnormal VEMPs have unfavorable hearing outcomes compared with those with normal VEMPs. Otolith organs are involved more frequently than afferents in patients with ISSNHL. Furthermore, the utricle was more susceptible compared to the saccule. The combination of ACS and GVS-VEMPs can better evaluate the lesion site and contribute to the clinical diagnosis, treatment, and prognosis evaluation of ISSNHL.The original contributions presented in the study are included in the article/supplementary material, further inquiries can be directed to the corresponding authors.This study was approved by the Ethics Committee of Xinhua Hospital Affiliated to Shanghai Jiaotong University School of Medicine . Written informed consent to participate in this study was provided by the participants' legal guardian/next of kin.JSh was responsible for data interpretation and manuscript preparation. XM contributed to the data analysis. QingZ helped optimize the VEMP test procedure. JC contributed to the clinical consultation. LW, WW, KH, JSu, and QinZ were responsible for auditory tests and VEMP tests. XC contributed to the statistical consultation. MD and YJ reviewed and revised the manuscript and study design. JY was responsible for the research design and manuscript revision. All authors contributed to the article and approved the submitted version.


Viral infection serves as the crucial etiology for the development of sudden sensorineural hearing loss (SSNHL). We aimed to investigate whether there is an association between concurrent Epstein–Barr virus (EBV) infection and SSNHL in an East Asian population. Patients who were older than 18 years of age and met the criteria of sudden hearing loss without an identifiable etiology were enrolled from July 2021 until June 2022, followed by the serological testing of IgA antibody responses against EBV-specific early antigen (EA) and viral capsid antigen (VCA) with an indirect hemagglutination assay (IHA) and real-time quantitative polymerase chain reaction (qPCR) of EBV DNA in serum before the treatment was initiated. After the treatment for SSNHL, post-treatment audiometry was performed to record the treatment response and degree of recovery. Among the 29 patients included during enrollment, 3 (10.3%) had a positive qPCR result for EBV. In addition, a trend of poor recovery of hearing thresholds was noted for those patients with a higher viral PCR titer. This is the first study to use real-time PCR to detect possible concurrent EBV infection in SSNHL. Our study demonstrated that approximately one-tenth of the enrolled SSNHL patients had evidence of concurrent EBV infection, as reflected by the positive qPCR test results, and a negative trend between hearing gain and the viral DNA PCR level was found within the affected cohort after steroid therapy. These findings indicate a possible role for EBV infection in East Asian patients with SSNHL. Further larger-scale research is needed to better understand the potential role and underlying mechanism of viral infection in the etiology of SSNHL. Sudden sensorineural hearing loss (SSNHL) is a common otologic emergency that refers to sudden-onset and unexplained hearing loss. SSNHL is defined as hearing loss at a decibel hearing level (dB HL) of 30 or greater in three consecutive frequencies that develops within 3 days or less . PreviouCurrently, the pathogenesis of SSNHL has not been well elucidated. It is often considered to be related to infectious, autoimmune, and blood circulation disorders ,9,10. GiThe Epstein–Barr virus (EBV), also recognized as human herpesvirus 4, is a member of the herpesvirus family. It is one of the most common human viruses, disseminates most commonly via body fluids , is a frequent cause of infectious mononucleosis, and can even induce various malignancies  ,19,20. IThis case–control study was conducted from July 2021 to June 2022, and patients were included when the following criteria were met: Patients were older than 18 years of age and had sudden hearing loss without an identifiable etiology. Patients who were younger than 18 years of age or patients with hearing loss from an identified etiology of sensorineural hearing loss  or genetic deafness  were regarded as ineligible. The study was performed with informed consent from all the participants and under the approval of the Institutional Review Board of Taipei Veterans General Hospital (IRB number: 2020-06-001AC).All the patients underwent pure-tone audiometry (PTA) for the diagnosis of sudden hearing loss at presentation and the post-treatment follow-up. The eligible patients also underwent tympanometry, the auditory brainstem evoked response test, and serum examination to rule out identifiable otologic causes . The average PTA was calculated for the thresholds of 0.5, 1, 2, and 4 kHz. Audiograms were obtained at the initial visit (pretreatment) and at 1 week, 2 weeks, 1 month, and 2 months after treatment. The grade of the initial audiogram was recorded using the modified Siegel criteria proposed by our previous study . AccordiDNA and antibodies from the circulating Epstein–Barr virus (EBV) represent two of the most sensitive peripheral blood markers of EBV infection . All theAll the patients received the same standard treatment of intravenous steroids  and oral steroids , followed by post-treatment audiometry to record the treatment response and degree of recovery. The degree of hearing recovery was evaluated based on the modified Siegel criteria and classified into one of five groups as follows: complete recovery (CR), partial recovery (PR), slight improvement (SI), no improvement (NI), and nonserviceable ear (NS) . CR was p-value less than 0.05 was considered significant. All statistical calculations were performed with SPSS version 20.Comparisons of continuous variables between the two groups were performed with Mann—Whitney U tests as indicated, while comparisons of dichotomous data within groups were conducted using Fisher’s exact tests as indicated. All statistical tests were two-sided, and the level of significance was set at 5%. A p = 1.00). VCA-IgA and EA-IgA titer distributions are listed  were females, and 14 patients (53.8%) were males, while there were 2 females (66.7%) and 1 male (33.3%) with positive PCR results. Furthermore, 15 patients (57.7%) with undetectable IgA titers and 11 patients (42.3%) with detectable IgA titers were found in the PCR-negative group, while 2 patients (66.7%) with undetectable titers and 1 patient (33.3%) with detectable titers were found in the PCR-positive group. There were no differences in the detectable IgA ratio between the PCR-positive and PCR-negative groups (e listed . Twelve e listed .p = 0.635) , PR was observed in 2 patients (8.3%), SI was observed in 3 patients (12.5%), NI was observed in 8 patients (33.3%), and NS was found in 3 patients (33.3%). In addition, the results of the three patients with positive PCR showed one patient with CR, one patient with PR, and one patient with NI. The average hearing gain, compared with the initial presentation, was 25.7 dB HL and 33.3 dB HL in the PCR-negative group and PCR-positive group, respectively, without a significant difference (= 0.635) .Among the three patients with sudden hearing loss and positive PCR results, two (66.7%) had the profound type, and one (33.3%) had the flat type. The first patient was a 39-year-old female presenting with a hearing level of 80 dB HL. The serum level of EBV DNA in this patient was 35 IU/mL (21.7 copies/mL), and the patient showed complete recovery with a hearing gain of 61 dB .The second patient was a 26-year-old female presenting with a hearing level of 85 dB HL. While the serum level of EBV DNA in this patient was 59 IU/mL (36.58 copies/mL), the patient demonstrated good recovery with a hearing gain of 42 dB .The last patient was a 46-year-old male with a left hearing level of 75 dB HL B. This pThis prospective cohort study attempted to unveil the association between EBV infection and sudden hearing loss. Our study demonstrated that approximately one-tenth of the enrolled SSNHL patients had evidence of detectable circulating EBV DNA, as reflected by the qPCR test results. The present study also demonstrated that there was a negative correlation between EBV DNA level and hearing recovery when investigating the EBV viral load and the hearing outcome of each PCR-positive individual. This is the first study in which PCR results were used to evaluate the correlation between idiopathic sudden hearing loss and EBV infection.SSNHL is defined as sensorineural hearing impairment of 30 dB HL or more for at least three adjacent audiometric frequencies that develop within 72 h . The incDifferent lines of evidence, including clinical studies, animal studies, and histopathological studies, have found viral infection or viral reactivation within the inner ear as the cause of cochlear inflammation or damage ,38. ElevA strong association between elevated IgA antibodies against EBV-specific VCA and EA and the risk of nasopharyngeal cancer (NPC) has been well demonstrated ,49,50. HOur study showed that EB PCR-positive SSNHL cases might present an audiometric curve shape similar to that of EB PCR-negative cases. The initial severity and audiometric curve shape have been associated with prognosis in SSNHL. Ascending audiograms might have a better prognosis, and flat and profound audiograms have a poor prognosis, which was hypothesized to be due to the different susceptibilities of hair cells between the basal turn and apex . InconsiOur study revealed a negative trend between the EBV DNA level and hearing gain with systemic corticosteroid therapy, including one positive case with the highest serum level of EBV DNA that showed no improvement after steroid therapy. Our finding is consistent with a previous study indicating that EBV infection with a positive antibody test result should be considered a cause of SSNHL without any hearing recovery . From ouGiven the effort to investigate the link between EBV and SSNHL, this study had several weaknesses. First, the small number of 29 patients, the lack of a healthy control group, and the absence of previous studies are weaknesses of the present study. Statistically significant differences may not have been detected due to the small number of participants. Having a healthy control group would inform us about the percentage of the healthy population with IgA antibodies and EB DNA. Further study with larger populations with serial serological test evaluations is needed to draw more definitive conclusions.This is the first study to use real-time PCR to detect possible concurrent EBV infection in SSNHL populations in an East Asian population. Our study demonstrated that 10.3% of SSNHL patients showed detectable EBV DNA using PCR. Further prospective studies with larger populations and adequate control groups and hearing analyses among SSNHL patients with positive evidence of EBV infection are needed.


Scarce evidence exists on pediatric colorectal polyp risk factors. This study explored the clinical manifestations, morphological and pathological characteristics of, and risk factors for pediatric colorectal polyps.This retrospective case-control study included children who received colonoscopy, divided into a colorectal polyp group and a normal control group based on colonoscopy results. The risk factors for colorectal polyps in children were analyzed through logistic regression analysis.P = 0.037), age (P < 0.001), family aggregation (P < 0.001), specific immunoglobulin E (sIgE) (P < 0.001), platelet count (P = 0.001), aspartate aminotransferase (AST) (P = 0.016), meat intake (P = 0.010), and vegetable intake (P < 0.001) were significantly associated with colorectal polyps. Age ≤ 6 years , positive family aggregation , positive sIgE , and higher meat intake  were risk factors for pediatric colorectal polyps in logistic regression analysis. Higher vegetable intake  was a protective factor against pediatric colorectal polyps. The area under the curve (AUC) of meat intake in the receiver operating characteristic (ROC) curve analysis for predicting colorectal polyps was 0.607; the best cut-off value was 92.14 g/d . The meat and vegetable intake combination AUC in predicting pediatric colorectal polyps was 0.781 .The mean age of children with polyps was 6.77 ± 3.44 years. Polyps were detected predominantly in males (72.9%); hematochezia was the primary clinical manifestation (80.25%). Most polyps were juvenile (88.9%) and solitary (87.7%); 50.6% were located in the rectosigmoid area. Univariate analysis showed that gender (Juvenile, solitary, and located in the rectosigmoid region polyps are most common in children. Hematochezia is the main clinical manifestation. Most polyps were, but multiple and proximally located polyps were also detected. Age ≤ 6 years, especially 3–6 years, positive family aggregation, positive sIgE, and higher meat intake are risk factors for pediatric colorectal polyps. A higher vegetable intake is a protective factor. Colorectal polyps consist of colonic mucosa that rises from the mucous membrane to the bowel lumen . Most chA total number of 81 children who were hospitalized from October 2020 to October 2022 and diagnosed with colorectal polyps by colonoscopy were enrolled as a case group. In the control group, we randomly selected and included 123 children who underwent colonoscopy during the same period and whose results showed no obvious abnormalities. If a patient had undergone multiple colonoscopies within the time frame selected, only the first colonoscopy was included in our research work to ensure per-patient analysis results have been obtained.The following inclusion criteria were implemented: (1) Children aged 0–18 years who underwent colonoscopy; (2) Complete clinical data, including clear image data of colonoscopy and polyp histopathology; (3) Informed consent was acquired, and the respective forms were signed by the patient’s parents, including informed consent for colonoscopy, informed consent for biopsy tissue, and informed consent for endoscopic polypectomy.The exclusion criteria applied were as follows: (1) Severe cardiopulmonary disease or other serious underlying diseases; (2) Acute active stage of intestinal inflammatory diseases; (3) Coagulation dysfunction, bleeding tendency; (4) Severe spinal deformity; (5) Poor compliance. Colonoscopy is not recommended in children with these conditions.This clinical study was approved by the Ethics Committee of The Second Affiliated Hospital and Yuying Children’s Hospital of Wenzhou Medical University . All methods and procedures were performed in accordance with the ethical standards as laid down in the Declaration of Helsinki. Written informed consent was obtained from all participants’ legal guardian.Clinical data were collected from electronic and paper medical records, including demographic characteristics and clinical manifestations. The residential addresses were divided into urban and rural. Based on clinical significance, we converted continuous data into categorical data. Age was classified into four categories:< 3 years, 3–6 years, 6–11 years, and ≥ 12 years; the course of disease was divided into a short course (< 6 months) and a long course (≥ 6 months).The following blood test data were collected: hemoglobin (venous blood), sIgE, the white blood cell (WBC) count, platelet count, blood eosinophil count, C-reactive protein (CRP), albumin, alanine aminotransferase (ALT), AST, alkaline phosphatase (ALP), and gamma-glutamyl transferase (γ-GT). Based on the criteria of the World Health Organization (2011) , anemia Information was obtained by asking parents about their children’s defecation habits, dietary habits, and family aggregation. Diarrhea was considered to be present if their defecation habits met the Rome IV diagnostic criteria for diarrhea . SimilarFor the colonoscopy report of each patient, we extracted the following results: quality of intestinal preparation, depth and location of colonoscopy, presence or absence of polyps, location and characterizations of the polyps , and the pathologic type of the polyps (juvenile polyps/adenomatous polyps/hyperplastic polyps/inflammatory polyps/juvenile polyposis). To facilitate statistical data collection and analysis, the locations of polyps were grouped into rectal polyps, sigmoid polyps, descending colon polyps, pan-colorectal polyps, and polyps in other locations , the number of polyps was grouped into solitary polyp (only one polyp) and multiple polyps (containing at least two polyps), the size of polyps was categorized into large polyps (maximum diameter of the polyps > 15 mm) and small polyps (maximum diameter of the polyps ≤ 15 mm). The shapes of polyps were grouped into sessile, pedunculated- sessile, and pedunculated polyps.P < 0.10) were included in multiple analyses conducted using logistic regression. A P < 0.05 (two tailed) was considered statistically significant. ROC curve analysis was used to evaluate the predictive ability and optimal cut-off values of statistically significant continuous variables.All statistical analyses were conducted using SPSS 22.0. Continuous variables were presented as mean with standard vision or medians with interquartile ranges. The difference between groups was compared by t-test or nonparametric test. Categorical variables were expressed as frequencies with percentages, and the differences between groups were tested by χ2 tests or Fisher’s exact tests and considerable covariates (P > 0.05).A total number of 204 patients were enrolled in the study, of whom 81 had colorectal polyps. The mean age of the children with polyps was 6.77 ± 3.44 years, and the mean age of children without polyps was 9.27 ± 3.02 years. Compared with the normal control group, the patients who had colorectal polyps tended to have the following characteristics: male, younger, with anemia, with positive family aggregation and sIgE, with a higher platelet count and AST level, and with a higher intake of meat but a lower intake of vegetable (Table P = 0.007), whereas the differences in the remaining clinical manifestations were not statistically significant (P > 0.05) (Table P = 0.024), whereas the differences in the incidence of the remaining clinical manifestations were not statistically significant (P > 0.05) , abdominal pain (23.5%), and anal mass prolapse (8.6%). There were also a few patients without obvious clinical manifestations (6.2%) . Long-course disease was observed in 67.9% of the cases, whereas short-course disease accounted for 32.1%. Comparing the incidence of each clinical manifestation in different age groups, the differences in abdominal pain was statistically significant . In contrast, type of residence, defecation habits, the WBC count, blood eosinophil count, CRP, albumin, ALT, ALP, γ-GT, and seafood intake were not associated with colorectal polyps (P > 0.05).The potential influencing factors of colorectal polyps in children are listed in Table P < 0.10 or with important clinical relevance) were included in the logistic regression analysis. Multivariate analysis revealed that age ≤ 6 years, positive family aggregation, positive sIgE, and a higher intake of meat were risk factors for the presence of colorectal polyps in children. Meanwhile, a higher intake of vegetable was a protective factor for colorectal polyps in children. Compared to children who aged > 6 years, children with onset age at 6 or younger had a higher risk for colorectal polyps   and the risk of colorectal polyps is highest in children aged 3–6 years. Children with positive family aggregation had a higher risk for colorectal polyps than those with negative family aggregation . Children with positive sIgE had a higher risk for colorectal polyps than those with negative sIgE . Children with higher intake of meat had a higher risk for colorectal polyps  and those with higher intake of vegetable had a lower risk for colorectal polyps   and the sensitivity and specificity were 66.67% and 69.92%, respectively. The AUC of vegetable intake for predicting colorectal polyps in children was 0.319, and the predictive value alone was low. The AUC of the combination of meat intake and vegetable intake for predicting colorectal polyps in children was 0.781. The predictive value was better than that of meat intake alone .Considerable covariates (Colorectal polyps are the most frequently reported cause of lower gastrointestinal bleeding in children . In our Our study showed that polyps were mainly solitary and located in the rectosigmoid region, which is generally consistent with the available clinical analysis data , 18, 19.Juvenile polyps were the most common pathological type of pediatric colorectal polyps in this study. Juvenile polyps were present in 88.9% of the cases, followed by adenomatous polyps, inflammatory polyps, hyperplastic polyps, and juvenile polyposis, which accounted for 6.2%, 2.5%, 1.2%, and 1.2%, respectively. Previously published data for Hongkong  showed sThe incidence of colorectal polyps has been on the increase recently, which may be related to the improvements in equipment or colonoscopy techniques that increase the detection of colorectal polyps, as well as changes in diet and lifestyle.As modifiable risk factors, studies on adults suggest that the presence of colorectal polyps may be closely related to weight , 28, dieStudies have found that children with colorectal polyps usually have changes in defecation , 35, 36.Alexand et al.  found a It is widely mentioned the association between polyposis syndrome and family aggregation , 19, 40.Age may be an immutable factor for colorectal polyps. Our study found that age ≤ 6 years, especially 3–6 years, was a risk factor for colorectal polyps, which is in agreement with the findings of domestic and foreign data , 42. TheIn the univariate analysis of colorectal polyps, we found that gender was associated with the presence of colorectal polyps, but in multivariate analysis, gender was not a risk factor for polyps. There may be some bias related to the fact that our overall sample was more males dominated. It was reported that the incidence of colorectal polyps is higher in male than in female , 44, whiThere are some limitations in this study. First, this was a retrospective single-center observational study. Second, eating and defecation habits were self-reported by parents and there may have some recall bias. Finally, the number of cases included in this study is not large, and other risk factors that may be associated with the presence of colorectal polyps in children remain to be explored. The association and predictive value of these factors with colorectal polyps in children cannot be fully determined based on the results of this study alone, and the findings need to be validated in a multicenter study with a large sample.In conclusion, solitary juvenile polyps in the rectosigmoid area are the most common type of polyps in children. Nevertheless, multiple and proximally located polyps are also present in some pediatric patients. Hematochezia is the main clinical manifestation. The presence of colorectal polyps in children is associated with age ≤ 6 years, positive family aggregation, positive sIgE, higher intake of meat, and lower intake of vegetable.


The sulfhydryl content of cathepsin D decreased to 9.20% after oxidation, while the carbonyl content increased to 100.06%. The β-sheet in the secondary structure altered due to oxidation as well. The changes in the intrinsic fluorescence and UV absorption spectra indicated that oxidation could cause swelling and aggregation of cathepsin D molecules. The structure of cathepsin D could change its activity, and the activity was highest under 1 mM H2O2. Cathepsin D could degrade myofibrillar proteins in different treatment groups, and the degree of degradation is various. Therefore, this study could provide a scientific basis for the mechanism of interaction among hydroxyl radical oxidation, cathepsin D, and MP degradation.In this study, cathepsin D was oxidized in vitro with different concentrations of H Coregonus peled, a cold-water fish in Xinjiang, is rich in high-protein, high-fat, various amino acids, minerals, vitamins, and unsaturated fatty acids, making it a popular choice among consumers both domestically and abroad in recent years [Coregonus peled are limited due to its immediate death out of water, which can trigger autolysis and affect processing and marketing. Even if frozen Coregonus peled is brought back to room temperature, tissue autolysis still occurs. The degradation of protein skeleton by mitochondrial apoptotic enzymes, calpain, and cathepsin after fish death leads to meat softening.Meat and aquatic products are important consumables in people’s daily lives. However, after animals are slaughtered, a series of changes occur due to the interaction of physiology, chemistry, biochemistry, and microorganisms . Coregonnt years . UnfortuThe internal environment of the fish body collapses after death, and during the late stage of rigor, lysosomes rupture, intracellular pH decreases, and cathepsins are released into the cellular matrix . CathepsStudying the mechanism of cathepsin D in the tissue softening phenomenon after the death of fish and the effect of cathepsin D on the degradation of myofibrillar protein under the condition of hydroxyl radical oxidation system is of great significance for controlling oxidation in the production process, slowing down the deterioration of muscle texture, and producing high-quality products.Cathepsin D is present in almost all mammalian cells and tissues, and is the earliest discovered and most reported cathepsin . Catheps2O2 oxidation at different concentrations was measured by using acidified bovine serum protein as a substrate, and the results were shown in 2O2 was 1 mmol, the activity of cathepsin D was the highest, which increased by 36.74% compared with 0 probably owing to oxidation. The active site of cathepsin D was exposed, and the ability of cathepsin D to bind substrates was enhanced. The reduced activity of cathepsin at high oxidative concentrations may be due to the fact that at high concentrations of H2O2, the spatial construct of cathepsin D was further altered so that it began to fold, and the active site was hidden inside, thus resulting in reduced activity of cathepsin D.The activity of cathepsin D after H2O2 increased, the relative carbonyl content of cathepsin D also gradually increased. When the H2O2 concentration reached 10 mmol/L, the carbonyl content of cathepsin D increased by 100.06% compared with 0 mmol/L (p < 0.05). Carbonyl groups can form through direct oxidation, covalent binding to non-protein carbonyl compounds, cleavage of peptide backbone, and reducing sugar [3+. Amino acids such as arginine, lysine, and threonine in the amino acid side chain are easily modified and converted to carbonyl groups through dehydrogenation [Coregonus peled myofibrillar protein by hydroxyl radicals.ng sugar . Proteinng sugar . Numerougenation . Furthergenation  In this genation , who stu2O2 increased, the sulfhydryl content of the other treatment groups decreased to 85.49%, 75.221%, 54.87%, 21.95%, and 9.20% of the 0 mM treatment group, respectively (p < 0.05), indicating a significant effect of H2O2 on cathepsin D. Sulfhydryl groups in amino acids are susceptible to oxidation, which can lead to complex biochemical reactions resulting in various oxidation products such as sulfonic acid and disulfide bonds. These products make the spatial structure of proteins more compact, leading to a reduction in sulfhydryl group content [2O2 concentrations decreased monotonously with the increase in oxidation concentration. This phenomenon is consistent with the findings of Zhang et al. [2O2 on myofibrillar proteins in pigs. Fu et al. [2O2 on beef myofibrils.2O2 oxidation concentrations. The enzyme protein exhibited a pronounced absorption peak around 266 nm, which was the characteristic absorption peak of aromatic amino acid residues such as phenylalanine, tyrosine, and tryptophan [2O2 alter the primary structure of cathepsin D. This finding is consistent with the results of Lv et al. [UV absorption spectroscopy is a commonly used technique to assess the oxidative modification of protein aromatic amino acid side chains caused by oxidation, which allows for the evaluation of changes in protein structure . Figure yptophan . As depiv et al. , who inv2O2 oxidation concentration, the fluorescence absorption value of cathepsin D decreased because the tryptophan residue, previously located inside the protein, was exposed to oxidation-induced conformational changes that resulted in fluorescence quenching and reduced fluorescence intensity. This finding is consistent with Zhang et al.’s study [Endogenous fluorescence absorption of proteins is a useful tool to assess changes in protein structure and conformation. Proteins contain fluorescent luminescent groups such as tryptophan, tyrosine, and phenylalanine. Oxidation can trigger structural changes in proteins . Tryptop’s study , which i’s study  also rep2O2 oxidation, the CD spectrum of cathepsin D exhibited a clear positive peak at 195 nm and a distinct negative peak at 215 nm, indicating that the β-sheet structure was dominant in the cathepsin D group. Like all aspartic proteases, the cathepsin D structure is a double-leaf molecule primarily comprising β-sheets, which is consistent with previous studies on cathepsin D [2O2, indicating that the protein conformation of cathepsin D had changed to varying degrees. Oxidation of H2O2 could alter the secondary structure of cathepsin D and affect its enzyme activity.Far-UV CD is a technique used to study the secondary structure of proteins, where peptide bonds serve as chromophores, whereas near-UV CD allows for the evaluation of the tertiary structure of proteins . CD in thepsin D ,30. ThesCoregonus peled under the influence of hydroxyl radical oxidation system, oxidization of cathepsin D(OD) was performed, or untreated cathepsin D were incubated with oxidization myofibrillar protein (OMP) or non-oxidization myofibrillar protein for 2 h at 4 °C. SDS-PAGE and WB were then used to examine the extent and pattern of cathepsin D degradation of myofibrillar proteins in this treatment group. As shown in In order to evaluate the ability of cathepsin D to degrade myofibrillar proteins of According to the solubility of proteins, muscle proteins can be divided into three groups: sarcoplasmic proteins (soluble proteins), myofibrillar proteins , and meat matrix proteins (insoluble proteins) . Among tAmong them, in the OD + MP group, the degradation degree of the MHC band showed a trend of first increasing and then decreasing, which indicated that the MHC caused the greatest degradation of cathepsin D substrate at the oxidation concentration of 10 mM, degraded to 88.82% of the original protein. However, no obvious degradation bands of MHC were observed in this group.2O2 caused protein aggregation and reduced the binding site with cathepsin D. At the oxidation concentration of 0.1 mM, the degradation rate of MHC was the highest. It is possible that cathepsin D is a very active protease against native proteins, but it has higher activity against denatured proteins. In the OD + OMP group, cathepsin D and cathepsin D were oxidized at different concentrations, and MHC bands were significantly degraded, and the existence of degraded bands 1 and 2 was also obviously observed, indicating that oxidative modification could cause changes in the protein structure of myofibrillar protein and cathepsin D. Different concentrations of H2O2 had various effects on myofibrillar protein and enzyme protein. Hence, the degree of oxidized cathepsin D degradation of oxidized myofibrillar protein was also different, among which the degradation degree was greater in the 10 mM H2O2 treatment group, which was the same as the OD + MP treatment group. In the three different incubation groups, the degradation rate of both myofibrillar protein and enzymatic protein oxidized was significantly higher than that of the group only oxidized by cathepsin D, indicating that oxidation had a significant effect on both myofibrillar protein and enzymatic protein, and oxidation of myofibrillar protein could also accelerate the degradation rate of MHC by cathepsin D. This situation was also true in vivo after fish death, and the grouping of OD + OMP was closer to the real situation of cathepsin D degradation of myofibrillar proteins after fish death. These results demonstrated that the softening of fish tissue could be well inhibited by controlling the appropriate oxidation concentration.In the D + OMP group, the degree of MHC degradation first increased and then decreased, and the degradation band 1 and band 2 could be clearly observed. It may be due to the fact that the high concentration of H2O2 was quantified by WB (2O2 affected actin degradation and the degree of troponin degradation was inhibited at high concentrations of H2O2 (concentration greater than 0.5 mM). The high concentration of oxidation may cause actin aggregation and lead to reduced degradation. In the incubation group of oxidized cathepsin D and oxidized myofibrillar protein, that is, the OD + OMP group, actin was degraded compared with the control group. When the concentration of H2O2 was 5 mM, the degradation degree of actin was the lowest, which was reduced to 81.56% of the control group. This indicated that actin degradation was greatest when both cathepsin D and MP were oxidized by high concentrations of H2O2.Actin is an important component of the cytoskeleton and plays an extremely critical role in eukaryotic cells. The actin cytoskeleton plays a role in the generation and maintenance of cell morphology and polarity, intracellular transport and contractility, motility, and cell division . Actin ied by WB A, and thCoregonus peled, indicating that the skeletal muscle of Coregonus peled is degraded under the action of cathepsin D. As shown in 2O2 oxidation could accelerate the degradation of myofibrillar proteins by cathepsin D. In the treatment group incubated with unoxidized cathepsin D and oxidized myofibrillar protein, that is, D + OMP, the degradation degree of desmin was first decreased and then increased, indicating that desmin itself was sensitive to hydroxyl radicals, and the change of H2O2 concentration would affect the degradation of desmin. Thereinto, the degradation degree was greater at a high concentration. In the OD + OMP group, with the different oxidation concentrations, the degree of degradation of desmin was also different, indicating that when hydroxyl radicals oxidized cathepsin D and myofibrillar protein at the same time, they altered the action site, resulting in different degrees of degradation of desmin.Desmin is an important cytoskeletal protein in cells, which can connect single myofibrils around the Z disc or other organelles . The hyd2O2 oxidation. In the grouping of unoxidized cathepsin D and oxidized myofibrillar protein (D + OMP group), cathepsin D could degrade different concentrations of oxidized myofibrillar protein. The difference in oxidation concentration led to the difference in the degree of degradation of troponin-T by cathepsin D, and the degradation degree of this treatment group was higher than that of the OD + MP group. These results indicated that oxidized myofibrillar protein could be better degraded by cathepsin D. In the incubation group of oxidized cathepsin D and oxidized myofibrillar protein, that is, the OD + OMP group, there were different degrees of degradation of troponin-T. With the increase in oxidation degree, the degradation degree of troponin-T gradually decreased, and the structure of cathepsin D and myofibrillar protein was changed by oxidation. The degree of degradation of the treated group showed different results with different oxidation concentrations, indicating that H2O2 could change the activity of protease and the structure of protein, so that the degree of proteolytic degradation of myofibrillar protein also changed.Troponin is a trimeric complex composed of troponin-T, troponin -I and troponin -C. In proteomics, the degradation of troponin-T is commonly used as a marker of protein degradation . As showCoregonus peled used in this study were raised under the same feeding and management conditions  at a commercial fishery processing company . Freshly caught Coregonus peled were transported to the laboratory within 5 h by cold-chain transportation. The dorsal muscles were cut into blocks by removing the hemoglobin, frozen immediately in liquid nitrogen, and rapidly stored at −80 °C.All w/w) and homogenized by a homogenizer  at 10,000 rpm for 1 min until the muscle was broken (to avoid air bubbles). The homogenate was then centrifuged at 8000× g for 10 min , and the supernatant was discarded to collect the precipitate. The precipitate was washed by adding 10 times the volume of the precipitate in 0.3% NaCl solution and centrifuged again at 8000× g for 10 min, then the supernatant was discarded. Next, the precipitate was dissolved in a 10-fold volume of pre-cooled 0.6 M NaCl-Tris-HCl buffer (pH 7.0). After the supernatant was completely dissolved, it was homogenized with pre-cooled deionized water  and centrifuged at 8000× g for 10 min at 4 °C to obtain the precipitate. The final precipitate was resuspended in 0.6 M phosphate buffer (pH 7.0), the insoluble material was removed by filtration through three layers of gauze, and the filtrate was used as MP. The concentration of MP was measured using the biuret method [Myofibrillar protein (MP) was extracted following the method described previously  with slit method  with bov3+→Fe2+, Fe2+ + H2O2→OH. This oxidation system contained 0.1 mM Vc, 0.01 mM FeCl3 and different concentrations of H2O2  in 50 mM phosphate buffer (pH 6.0). The samples were then incubated for 1 h at 4 °C. After incubation, oxidation was terminated by the addition of Ethylene diamine tetraacetic acid (EDTA)/Ethylene diamine tetraacetic acid disodium salt (EDTA-2Na) at a final concentration of 1 mM. The oxidation system of myofibrillar protein was the same as that of cathepsin D, and the final concentration of myofibrillar protein was 10 mg per milliliter.The active and pure cathepsin D  was subjected to a hydroxyl radical oxidation according to the method of Park et al. . This syw/v) was mixed with 50 μL citrate buffer  and incubated at 37 °C for 30 min for denaturation, and 25 μL of the enzyme system was added and incubated at 37 °C for 60 min. The reaction was finished by adding 50 μL of 15% (w/v) trichloroacetic acid. The TCA soluble polypeptide in the supernatant was released by the enzymatic reaction and centrifuged at 2000× g for 5 min. The content of TCA soluble polypeptide was determined by the method of Lowry et al. [2O2 concentration as control.Cathepsin D activity was measured using acidified bovine serum protein as a substrate according to the method of Minarowska et al. . First, y et al. . The relg for 5 min at 4 °C, the supernatant was discarded, and the precipitate was dissolved in 1 mL absolute ethanol-ethyl acetate  and washed 3 times to remove free DNPH. The obtained precipitate was finally dissolved in 3 mL of a solution containing 6 M guanidine hydrochloride and kept in a 37 °C water bath for 10 min to dissolve the precipitate. The solution was then centrifuged under the conditions described above and the supernatant was retained. The carbonyl content was determined by the molar extinction coefficient of 22,000 M−1 CM−1 at 370 nm, and the carbonyl content of the 0 mM H2O2 treatment group was set to 1, and then the relative content was calculated for the remaining groups.According to the method of Oliver et al. , the car−1 CM−1. Moreover, the carbonyl content of the 0 mM H2O2 treatment group was set to 1, and the relative content was calculated for the remaining treatment groups.Following the method of Qin et al.  with sliThe intrinsic fluorescence spectroscopy of oxidized cathepsin D was performed by the method described previously  with minThe secondary structure of oxidized cathepsin D was studied using a MOS-450 circular dichroism spectrometer  and a quartz absorption cell with an optical path length of 1 mm . The scaThe UV spectroscopy were performed according to the previously reported methods . The UV To determine whether the degradation of MP by oxidized cathepsin D is different, we incubated both oxidized and unoxidized MP samples with known quantities of oxidized and unoxidized cathepsin D . In brieThe degradation of MP by oxidized cathepsin D was evaluated by SDS-PAGE and WB according to the method of Laemmli  with slip < 0.05.All experiments were completed in triplicate, and statistical analysis was performed by SPSS 25.0 . Analysis of variance (ANOVA) was used to measure the significance of the major influences, and significant differences between the means were determined by Duncan’s multiple range test. The significance level was set as As the oxidative concentration increased, the enzyme structure of the tryptophan residue gene of cathepsin D changed, resulting in fluorescence quenching and a decrease in the fluorescence absorption intensity of cathepsin D. The UV absorption intensity of cathepsin D also decreased with increasing oxidation intensity, indicating that cathepsin D was sensitive to hydroxyl radicals and that the protein’s primary structure had changed. Hydroxyl radical oxidation altered the tertiary structure of cathepsin D, as measured by CD.Furthermore, when oxidized/unoxidized cathepsin D was incubated with oxidized/unoxidized myofibrillar protein, it was found that oxidized cathepsin D was more effective in degrading the MHC of oxidized myofibrillar protein than unoxidized myofibrillar protein. However, the degradation of actin, myactin, and troponin varied with different treatments. In practice, protease, oxidation, and protein degradation are highly complex processes that involve many intricate physiological changes and require further exploration and study.


Investigate the auditory function of the elderly using the middle latency potentials.Group 1 (G1): 20 healthy individuals of both genders, older than 60 years, without hearing loss. Group 2 (G2): 20 healthy individuals of both sexes, older than 60 years, with hearing loss in frequencies from 4 to 8 kHz. Potential recording was performed with unilateral and bilateral stimulation and the Binaural Interaction Component was calculated.st negative peak was more negative in G1 and the 2nd positive peak showed greater amplitude in C4A2 in both groups.Na latency in C3A1 was greater in the stimulation of the right ear in G2 and the amplitude of Na-Pa was greater in the stimulation of the right ear and recording in C3A1 in G1. The latency of the Pa component was higher in the stimulation of the right ear recorded in C4A2. The Pb component in G2 by bilateral stimulation and recorded in C4A2 had higher latency. The first and second negative and positive peaks presented greater amplitude in G1. In C3A1, the 1The transmission of auditory information to the primary auditory cortex is impaired with aging, especially in unilateral stimulation, reinforced by losses in elderly people with peripheral hearing loss, such as in the binaural interaction at the cortical and subcortical levels. Thus, the AMLR has shown to be a sensitive examination to investigate neuroauditory disorders in the elderly, especially related to high-frequency hearing loss and primary auditory cortex dysfunctions caused by the aging process. Currently, the number of elderly people in Brazil is increasing due to the reduction in the birth rate and the increase in life expectancy.It is noted that with aging, there may be difficulty in locating the sound source, in perceiving and understanding speech in noise, which are some of the binaural abilities. They demonstrate the importance of binaural integration, essential for communication and which may be compromised even in the absence of peripheral hearing loss.The Auditory Middle Latency Responses (AMLR) have been used in Audiology scientific research as a complementary method for assessing auditory function and binaural processing. Since this measure sensitizes the hearing assessment and brings more accurate information regarding the processing of the auditory information and provides critical information for audiologic rehabilitation strategies.Because the binaural processing presents greater activity after the brainstem, performing AMLR is appropriate as it is generated by structures at higher levels from the brainstem to the primary auditory cortex aged.Cortical auditory potentials examinations showed age-related changes in auditory evoked responses, with increased P1 and P2 latencies when diagnosed in young adults, which may be related to the synchrony of the cortex in the sensory prefrontal region, and a longer processing of sensory information.Due to the maturation process of the structures responsible for generating the AMLR, the amplitude of the components is highly related to age.According to some studies, there is a worsening in the AMLR response in older individuals, and there is a strong correlation between the quality of the response and increasing age.In this sense, this study seeks the response to the following questions: “Is there a change in the AMLR in the elderly?”; “Is there a difference in the AMLR when comparing the records of the two cerebral hemispheres in the elderly without hearing loss at high frequencies?”; “Are monaural and/or binaural abilities compromised regardless of the auditory threshold at high frequencies?”; “Is the process of binaural interaction impaired in the two groups of elderly?”.Hearing and understanding speech require processing spoken words at the cortical level. Thus, the AMLR can be an important tool in the management of the specific demands of the elderly in relation to their communication difficulties, and in the establishment of more efficient auditory rehabilitation strategies.In this context, the aim of the study was to investigate the auditory function of the elderly using the middle latency potentials, to analyze and compare the unilateral and bilateral stimulation in the elderly.•Group 1 (G1): 20 individuals of both sexes , older than 60 years right-handedness and tone audibility threshold in the frequencies of 0.25, 0.50, 1, 2, 3, 4, 6 and 8 kHz ≤ 25 dBHL  and Speech Reception Threshold (SRT) ≤ 25 dBHL, according to Lloyd and Kaplan's classification (1978);•Group 2 (G2): 20 individuals of both sexes , older than 60 years of right-handedness and who had symmetric bilateral sensorineural hearing loss  with tonal audibility thresholds ranging from 30 at 70 dBNA, considering frequencies from 3 to 8 kHz, and frequencies from 0.25, 0.50, 1, 2 ≤ 25 dBNA, according to the classification by Lloyd and Kaplan (1978). The configuration of the audiometric curve was symmetrical in both ears, with a difference between the interaural audibility thresholds for the assessed frequency ≤ 15 dBHL and air-bone gap of up to 10 dB and Speech Reception Threshold (SRT) ≤ 25 dBHL.The study was approved by the Research Ethics Committee of the institution under number 1.905.689. A descriptive and analytical cross-sectional study that followed compliance according to the STROBE Declaration, included 40 subjects, sampled by convenience, divided into two groups:The study did not include individuals outside the age range of the studied group, individuals who had any cognitive impairment diagnosed by a neurologist, and who did not present preserved functionality for activities of daily living, used ototoxic drugs, had a history of exposure to noise, smoking and changes in middle ear and/or hearing loss in the frequencies of 500 Hz, 1 kHz and 2 kHz and/or thresholds ≥ 71 dB HL in frequencies from 4 to 8 kHz.The two-channel auditory evoked potential equipment Bio-logic Evoked Potential System (EP)  and ERA-39 intra-aural earphones were used. The ASC II software  was used to extract data from each record obtained for the calculation of the Binaural Interaction Component (BIC), which corresponds to the sum of the responses obtained with the unilateral stimulation subtracted from the response obtained with the bilateral stimulation through the grand-average of the recorded waves with unilateral and bilateral stimulation.As a pre-collection procedure, the authors performed: audiological anamnesis, an inspection of the external acoustic meatus, and Mini-Mental State Examination to characterize the sample, with 30 as the cut-off score, according to the median scores proposed by Brucki et al., 2003 tympanometry, audiometry tonal threshold for characterization of samples and groups and Speech Reception Threshold (SRL).For AMLR recording, the subjects were accommodated in an acoustically treated room and temperature of 24°C, positioned in a reclining chair and they all received the same guidelines for keeping relaxed, but alert, their eyes open trying to stare at a fixed spot somewhere in the room to avoid moving the head. This way, the records  did not present more than 10% of artifacts from the total of 1000 stimuli.The electrodes were fixed with microporous adhesive tape after the skin was cleaned with abrasive paste and positioned at C3 and C4  with reference to A1 and A2 (left and right ear lobe), and ground on the Forehead (F4). This arrangement assures the observation of the ipsi and contralateral measures and optimizes the recording of cortical activity. The impedance of each electrode did not exceed 5 Kohms and did not exceed 2 Kohms between the electrode impedances.For the AMLR recording, unilateral click-stimuli were used in the right ear and left, and then bilateral stimuli (simultaneously in both ears), rarefaction polarity at 30 dB NS, with a presentation rate of 11 stimuli/second, time of analysis (window) of 100 ms, acoustic filter of 10 to 100 Hz, amplification of 75.000 × .After recording the responses performed with unilateral and bilateral stimulation, the binaural interaction component could be calculated. In order to obtain the sum of the waves obtained by the right (D) and the left (L) ear stimulation followed by the subtraction of the obtained wave by bilateral stimulation (BI): BIC = ((D + E) - BIN), a second software was used (ASC II) for extracting the waves, which extracted the 256 amplitude points of each wave allowing to achieve grand-average of the records of each group and then the sum and subtraction to obtain the BIC.AMRL components are generated via the inferior colliculus, medial geniculate body, formation reticularis and primary auditory area along with associated areas and the corpus callosum. The waves were identified based on the consistency of the latency and amplitude values of their components. Thus, the Na component is among the first highest negative peak and lies between 12 and 27 milliseconds (ms); Pa o peak highest positive after the Na wave, between 25 and 40 ms; Nb is the positive peak immediately after Pa, between 30 and 50 ms and Pb, the subsequent higher positive peak, immediately after Nb, between 45 and 65 ms.st negative peak, 1st positive peak, and 2nd negative peak. To control possible biases, the analysis of records, identification, and marking of each AMLR component was carried out by two judges.Regarding Na-Pa latency and interamplitude, unilateral and bilateral stimulation was in both groups. The peaks in the BIC waveform were labeled as 1The distribution of normality was verified by the Shapiro-Wilk test with Liliefors correction. An analysis of Repeated Measures Mixed-Design Ancova was performed to observe the group effect, condition, and interaction (group vs. condition) controlling the effect of covariate age. Box's M Test was used to verify if the covariance matrices of the dependent variables were the same for the two groups and the Mauchly's Test was used to test the sphericity hypothesis. In the case of rejection of the sphericity hypothesis, the analyzes were based on the Greenhouse-Geisser multivariate test. The main effect within the group and/or condition was analyzed by the Bonferroni multiple comparison test. The adopted confidence level was 5%.The mean age in G1 was 65.75 years, and 67.4 years in G2. The participants' schooling level ranged from incomplete elementary school to complete higher education; the average of the Mini-Mental State Examination (MMSE) was 26.81 points.There was a difference between the elderly with hearing loss in the latency of the Na component when compared to the stimulation of the right and left ears of the record in C3A1 (left hemisphere) with a significant effect for the covariate age so that older elderly people with hearing loss had worse performance.A difference was also observed between the groups of elderly people with and without loss of Na-Pa amplitude in C3A1 (left hemisphere) when stimulated in the right ear, and the amplitude was lower in elderly people with hearing loss.There was a significant difference between the latency of Pa component obtained with right ear stimulation and recorded at C4A2.It is also observed that the Pb component obtained through bilateral stimulation and recorded at C4A2 presents a higher latency than that recorded at C3A1, with age as a significant factor influencing the increase in latency.st negative peak and 1st positive peak inter-amplitude in C3A1 is observed, whereas Na amplitude is more negative than when compared in the elderly with hearing loss.In the elderly without hearing loss, a more prominent 1st negative peak, 1st positive peak, 2nd negative peak and 2nd positive peak present a higher amplitude in G1. Moreover, the 2nd positive peak presents a higher amplitude in C4A2 in both groups.In addition, it is observed that binaural interaction at C4A2 in the elderly without hearing loss also presents a better wave morphology when compared to the elderly with hearing loss, and the 1The present study aimed to investigate the auditory function of elderly people through middle latency potentials, and to compare unilateral and bilateral stimulation in elderly people with and without hearing loss. The authors demonstrated that the average latency values of components Na, Pa, Nb and Pb were within the normal range according to the age group, as well as the Na-Pa interamplitude, which was lower in the elderly with and without high-frequency hearing loss.Few studies address the measurement of the AMLR in the elderly. The paper by MoosaviWhen checking the ear effect , it is oThese findings can be confirmed in the comparison of the Na-Pa amplitude values . At C3A1,As reported by Fonseca and Costa-FerreiraIn the hemisphere comparison, electrode effect, According to Jerger et al.Na component is generated from the thalamo-cortical connections, and Pa in the medial portion of Heschl's gyrus, responsible for acoustic recognition and discrimination abilities of the auditory cortex.Therefore, as the latency of the described components was higher at C4A2 than at C3A1 in the elderly with normal hearing, it is possible to observe auditory CNS degradation at the cortical level, even in the absence of hearing loss.nd positive peak higher at C4A2 in the elderly with and without hearing loss when compared to C3A1. As the 2nd positive peak is generated from the thalamic nuclei, entry of the reticular activation system.In relation to BIC when comparing C3A1 and C4A2, it occurs at the cortical level in both groups, and it is possible to visualize the 2nd positive peak in the elderly,,However, in this study, there was an increase in the amplitude of the 2It was possible to conclude that the AMLR in the ear effect observed in the elderly group with hearing loss shows a slower unilateral auditory information transmission, resulting in losses in the thalamic-cortical transmission and poor quality in the central auditory pathway response with aging in the elderly with hearing loss at high frequencies. When the electrode effect was observed in the elderly without hearing loss, the differences in the responses between the hemispheres can indicate damages to the cortical level even in the absence of hearing loss at high frequencies. Finally, binaural interaction in the cortical and subcortical regions is impaired with aging, with greater impairment in elderly individuals with hearing loss at high frequencies.Thus, the AMLR showed to be a sensitive examination to investigate neuroauditory disorders in the elderly, especially related to high-frequency hearing loss and primary auditory cortex dysfunctions caused by the aging process. In addition, the BIC from the AMLR reflected damages in the binaural auditory pathways at the cortical level in the elderly, ensuring the integral assessment of the central auditory system and allowing them to choose a more effective form of auditory rehabilitation, especially in this population.However, conducting behavioral tests that assess central auditory processing associated with electrophysiological measures would broaden the analysis of the central auditory system in the elderly, a methodological point that should be considered in future studies.The authors declare no conflicts of interest.


Methodology. This study was a descriptive cross-sectional study adapting the MDAS into the I-MDAS by adding the International scale. The study surveyed 465 participants from a dental clinic, SurveySwap, and distributed flyers. Data was collected through Qualtrics through the self-administered I-MDAS and analyzed through the SPSS computer software version 28. Participants were categorized into two subgroups where 41.3% received dental care only within the United States and are termed the domestic population, and 58.7% received dental care outside of the United States and are labeled the nondomestic population. Information about demographics, past negative dental experiences, and current dental anxiety was collected. The conducted analyses utilized an independent sample t-test to compare the subgroups' anxiety levels, a bivariate correlation to find the Pearson correlation, a Cronbach's coefficient α, and a one way ANOVA test to compare the genders' I-MDAS scores.  The aim of this study was to improve upon the Modified Dental Anxiety Scale (MDAS) by developing the empathy-based International-Modified Dental Anxiety Scale (I-MDAS). This new measure was then utilized to compare the dental anxiety of patients cross-culturally. M = 12.73, SD = 5.13) and the nondomestic population ; t (463) = −0.58, p = 0.95). The I-MDAS shows evidence of validity and reliability. There was a significant and positive relationship between the International scale items and the MDAS scale items (r (463) = 0.60, p < 0.001), indicating the criterion validity of the I-MDAS. Content validity was strengthened by expanding the inquired topics in the new measure. The Cronbach's α value of 0.85 shows that the I-MDAS is reliable for clinical applications.  There was no significant difference in dental anxiety levels between the domestic population ( The I-MDAS improves upon the MDAS by providing dentists with a tool for encouraging empathy. Dental clinicians across nations can use the I-MDAS to combat the vicious cycle of dental anxiety. Dental anxiety is described as a cycle between fear/anxiety, avoidance, deterioration of dental state, and feelings of shame and inferiority toward dentists resulting from genetic, psychological, and social factors . RoughlyFurthermore, there is limited data regarding dental anxiety varying cross-culturally and what can be done to alleviate dental anxiety in ethnically diverse nations, especially the United States. Thus, the United States' demographic diversity provides a prime opportunity to contrast different cultural backgrounds in correlation to dental anxiety. It is important to note that in seeking to compare diverse patients' dental anxiety levels, the training programs for dentists throughout the world should be considered. Different dental programs utilize a variety of approaches in training student dentists across nations, some enforcing greater scientific advances or new models of dental education , 12. ThiAs stated above, there is much research supporting the epidemiology of dental anxiety. However, it is now a priority and a requirement to break the vicious cycle of dental fear and avoidance patterns . Thus, tBeing empathetic toward patients in healthcare has shown more positive patient and doctor relationships, greater satisfaction, and improvement in patient adherence to treatments and perceptions of health outcomes . Yet, fiα of 0.78 from Nepal [The I-MDAS is an adapted version of the MDAS, which was utilized as the foundation for this study due to its strong reliability and validity. A variety of previous studies have researched the reliability and validity of the MDAS in accordance with different patient populations, with the lowest Cronbach's coefficient om Nepal , and theom Nepal .For this study, it was hypothesized that (1) the domestic group would have varying MDAS scores compared with the nondomestic group, possibly due to differences in dental training programs , and (2)This was a descriptive cross-sectional study. The survey was provided through Qualtrics and participants were gathered from SurveySwap where the survey was posted to US resident and foreign participants, from dental patients of a participating dental clinic in Los Angeles, and by providing a scannable QR code that was randomly distributed to various neighborhoods in Los Angeles by placing flyers in mailboxes. Data collection occurred from April 2021 to February 2022. More specifically, SurveySwap is a website that sends the survey to participants all over the world. In order to align with the consent form and maintain the privacy rights of the participants, the specific locations of the responders will be withheld; however, generally it may be stated that participants were located in England, China, Netherlands, India, Jordan, Saudi Arabia, North and South Africa, and from a variety of states in the United States including New York, Boston, Kentucky, Texas, and California. Any translations were provided through the website. Participants' ethnicity was also inquired.The inclusion criteria required that participants were 18 years or older and have the capacity to give consent and be willing to complete the requested surveys and share their thoughts and experiences. All participants who met the inclusion criteria also provided consent. The exclusion criteria included patients who were not mentally sound.n = 192) and an nondomestic population (n = 273). The domestic population is defined as participants who have solely experienced dental care within the United States, and the nondomestic population is defined as any participant who has experienced dental care outside of the United States.The sample size minimum was found with the population adjustment formula for single proportion estimation  and follThis study adapted the MDAS , which iWith the addition of five new empathy-based questions, the MDAS was adapted into the I-MDAS Table . The I-MThe I-MDAS also has an adapted scoring system according to its two scales Table . The Intt-test was used. This t-test demonstrated the level of significance between the MDAS mean scores of the domestic and nondomestic groups. Normality was measured by testing the skewness of the data. A bivariate correlation was used to find the Pearson correlation to show the relationship between the original MDAS and the International scale and was utilized to indicate the criterion validity of the measure. Reliability analysis for the MDAS and the overall I-MDAS was completed by using Cronbach's coefficient α. A one way ANOVA test was utilized to compare the I-MDAS scores of the gender categories.The data collected was analyzed by using IBM SPSS Statistics (version 28) . To compEthical clearance was sought from the Pepperdine University Institution Review Board, and permission to conduct the study was obtained from this IRB and the participating dental clinic. Only participants who provided consent were included in this study and all information was handled confidentially. Any objection to participating in this study did not result in negative consequences toward the participant.t-test showed that the mean MDAS score for the domestic population  was not significantly different from the nondomestic population the ; t (463) = −0.58, p = 0.95). The MDAS scores are normally distributed as indicated by the measured skewness which was found to be 0.53, falling between 1 and −1.In regard to the first hypothesis, which states that the nondomestic population would have varying MDAS scores compared with the domestic population, a r (463) = 0.60, p < 0.001. Thus, as To test the second hypothesis, which states that the International scale and the MDAS would be positively correlated, a correlational analysis was used and produced a Pearson coefficient of α for the I-MDAS in this study was 0.85 indicating that this new measure is reliable. The Cronbach's α coefficient for the MDAS scale alone in this study was 0.88. α and reliability of the I-MDAS. The deletion of any items has a negligible effect on the Cronbach's coefficient α.The Cronbach's coefficient F = 9.65, p < 0.001, η2 = 0.04) between the I-MDAS scores among men , women , and nonbinary , with women producing significantly higher I-MDAS scores on average than men (p < 0.001).Additionally, it was found that there was a significant difference (Lastly, The aim of this study was to improve upon the MDAS by adding items related to empathy. This resulted in the I-MDAS. Past research has focused on the epidemiological elements that contribute to dental anxiety –5. HowevThe first hypothesis, stating that the domestic population would have varying MDAS scores compared with the nondomestic population due to differences in dental training programs across the globe , was rejThe second hypothesis, stating that the International scale and the MDAS would be positively correlated, was supported as the MDAS and the International scale showed a significant, positive correlation. Thus, the scales' items function together successfully.α scores. In fact, the MDAS score from this study strongly aligns with the findings of other MDAS reliability studies [Beyond the two hypotheses, the following analyses provided evidence of reliability and validity for the new I-MDAS measure. The I-MDAS increases and strengthens the content validity in which it expands the topics inquired in the original MDAS: it adds items one through five which increase a dentist's awareness of past experiences which may contribute to their patient's anxiety. Additionally, the significant and positive correlation between the MDAS and the International scale supports the criterion validity of this new measure. The I-MDAS is also valuable in that it increased validity, in comparison to the original MDAS, with the addition of new items while there was a negligible impact on the reliability of the original MDAS as shown by the Cronbach's  studies , 20. OveFurthermore, the I-MDAS was developed to provide an answer to the anxiety-inducing factors which are the lack of empathy and distrust in dentists . The IntAnother finding was that the I-MDAS scores were significantly higher for the women participants of this study compared with the men and nonbinary groups. This finding aligns with previous results where women showed higher MDAS scores compared with men , 8, 28 aThis study experienced limitations. Due to the use of online platforms and a participating dental clinic, a wide variety and a large number of participants were reached; however, these participants did center around the 18–34-year-old population with direct access to the SurveySwap website and the internet. This study also included a larger number of White, female participants. Thus, for future research, it would be beneficial to branch out to other patient populations which could address older age groups, greater variety in sexual orientation, and other ethnicities beyond the limits of Southern California and SurveySwap.α values show that the I-MDAS is also reliable for clinical applications. The significant and positive correlation between the International scale and the MDAS scale displayed strong validity for their functionality and the overall I-MDAS measure. Importantly, this new measure provides evidence of criterion validity and also increases content validity with a negligible impact on the reliability of the original MDAS. This showcases the strength of this new measure.The findings of this study show that the I-MDAS is an improved measure that has been successfully validated and is reliable for this subject population. The Cronbach's coefficient These findings encourage dentists across nations to implement the I-MDAS in dental clinics around the world in order to give dentists a quick, accessible tool to further understand each of their patients. It is a guide for the dentist or dental hygienist to perform case-sensitive, empathetic, and individualized treatments to prevent further trauma and promote trust. Future research will include testing the I-MDAS's reliability and validity with other patient populations, as well as further testing for gender differences in I-MDAS scores. This will also allow for test–retest reliability analysis in reference to the future patient populations.


The cause of childhood hearing impairment (excluding infectious pathology of the middle ear) can be extrinsic , genetic or both. In industrialized countries, the proportion of genetic sensorineural hearing impairment is currently estimated at 66%. Hereditary hearing loss can be classified into two broad categories: non-syndromic estimated at 70–90% and syndromic at 10–30%. Several hundred of syndromic associations have been described in which the hearing deficit may be associated with abnormalities or malformations in other organs [The knowledge of non-syndromic hearing loss has tremendously increased since 1995 and more than 120 genes have been described [The development of routine molecular diagnosis has allowed the identification of a genetic cause in a large part of sporadic hearing losses previously classified as “unknown cause” and a genetic cause is now estimated to account for about 80% of causes of non-syndromic congenital sensorineural hearing impairment (NSCSHI). In practice, clinical genetics bring together two main activities: etiological diagnosis and genetic counseling performed in specialized hospital departments. Since 2004, centers aiming at the diagnosis, management, training and research in the field of rare diseases (reference centers for rare diseases) have been built in several European countries. Each genetic deafness network brings together clinical and molecular geneticists, ENTs and research teams. Using the same model and in parallel with the labeling of the ERN (European rare network), a European network for genetic deafness was created in 2017.The expectations of families regarding genetics are different and depend on the history of each family. However, “making sense” seems to be a common need for patients and their families. Indeed, the narcissistic injury caused by the passage from the dreamed child to the real child causes high expectations of understanding from parents. In the search for etiology, parents seek relief for feelings of guilt and anxiety. Therefore, during the genetic consultation, preconceived ideas, sometimes rooted for a long time in the family’s imagination, resurface. In addition to researching etiology, this consultation brings scientific and medical information on hearing loss. Obtaining a molecular result can also make the existence of a hearing defect concrete and “scientific”, thus offering relief by putting an end to a psychic blur.-to establish the etiology of the hearing impairment;-to identify associated features;-to establish a prognosis of the hearing impairment;-to improve the management of hearing loss;-to evaluate the recurrence risk in parents’ and patient’s offspring (genetic counseling);-to detect possibly other affected subjects within the family.To date, the goals of genetic evaluation of a CSHI are multiple:In the last decade, scientific and technological improvement as well as clinical experience has allowed us to change our practices. During genetic consultations, a thorough clinical examination, revision of the clinical history, a precise and complete interrogation on the personal  and familial  history of each patient are conducted. The phenotype of the hearing impairment is defined as: age of onset or at first diagnosis, evolution , laterality/symmetry, degree , type of deficit , and shape of the audiometric curve are collected. In addition, attention should be paid to the acquisition of motor milestones, speech–language development and the presence of associated features such as tinnitus or vestibular symptoms. The results of complementary testing such as vestibular examination, and temporal bones, olfactory bulbs and brain imaging should be reviewed.-CMV serology in the child and mother (in order to exclude CMV fetopathy if at least one of the two is negative) and if possible CMV research on dried blood spot, urine or saliva samples (depending on the age of the child), collected as early as possible to diagnose prenatal CMV infection ;-Ophthalmological examination in order to take care of refractive problems for optimal visual acuity and to research visual pathologies associated within a syndromic form;-Audiograms of the siblings to diagnose familial cases of variable severity or progressive hearing loss and other family members if indicated by pedigree analysis.The following examinations are systematically required:-An electroretinogram in the case of a bilateral profound congenital hearing impairment with motor delay without malformation of the inner ear (suspicion of Usher syndrome type 1) ;-A renal ultrasound in the case of sensorineural and/or conductive hearing loss associated with preauricular tags or pits ) ;-A dosage of free thyroid hormones, TSH and Tg, in the case of a hearing defect with bilateral dilatation of the vestibular aqueduct (suspicion of Pendred syndrome) ;-GJB2 pathogenic variations (cf infra) (suspicion of Jervel–Lange-Nielsen syndrome);An electrocardiogram in the case of profound congenital bilateral deafness without inner ear malformations and without -A specialized ophthalmological examination in case of an auditory neuropathy ;-Audiograms of the parents at the slightest doubt of a hearing defect or in a second intention (undiagnosed cases).Further examinations and investigations will be requested depending on the clinical context or on the results of comprehensive genetic testing:At the end of this consultation, molecular tests may be proposed if a genetic etiology is suspected.-The search for alterations at the DFNB1 locus (GJB2 pathogenic variations and/or GJB6 deletions: del(GJB6-D13S1830) and del(GJB6-D13S1854)): responsible for 20 to 30% of bilateral NSCSHIs. The imaging of the inner ear is normal in the vast majority of cases and there is no vestibular deficit ,8,9. The-The search for STRC pathogenic variations or alterations at the DFN16 locus: the phenotype is the same as the previous except that the hearing loss is mild or moderate without evolution over time ,11;-The search for SLC26A4 pathogenic variations: hearing impairment is often prelingual, immediately or rapidly bilateral, sometimes asymmetrical, fluctuating and often progressive ;-The search for OTOF pathogenic variations: this gene remains the most frequently involved in isolated early auditory neuropathy/synaptopathy ,14;-The search for POU3F4 pathogenic variations or alterations at the DFNX2 locus: moderate to profound bilateral congenital isolated, mixed or sensorineural hearing loss among males and moderate postlingual, mixed or sensorineural hearing loss among females . Among mFor non-syndromic hearing loss, targeted molecular analysis are still proposed in some departments:All these genes are responsible for bilateral NSCSHI. The genetic causes of unilateral non-syndromic hearing loss have not been elucidated to date.The great genetic heterogeneity of prelingual hearing impairments makes it very interesting to use a recent high-throughput sequencing (NGS: next generation sequencing) technology to simultaneously analyze a large number of genes ,18. The -The clinical presentation suggests a known syndromic diagnosis , and the clinician will ask for an analysis of the gene(s) known to be responsible for this phenotype  ;-The patient has a polymalformative syndrome or a set of clinical signs associated with deafness that does not evoke a known diagnosis, and the clinician will most often request a chromosome assessment by a CGH array (comparative genomic hybridization array). This technique makes it possible to identify an abnormal number of copies of a chromosomal segment (deletion or duplication = copy number variation). These chromosomal abnormalities may involve one to hundreds of different genes located contiguously on a chromosome segment. An unusual association of clinical signs may be due to a fortuitous association of several genetic defects.For syndromic hearing loss, we can distinguish two frequently encountered situations:Regardless of the technology used, the confirmation of a molecular genetic result also requires the analysis of all deaf siblings and both parents. Indeed, it is essential to verify that the family segregation of the genetic pathogenic variants identified in the patient matches with the inheritance of the hearing loss. In light of this, it could also be necessary to carry out genetic and phenotypic analyses of available healthy members of the family in order to better characterize the “Variants of Uncertain Significance” which still remain a huge difficulty for laboratories, clinicians, and patients alike .Finally, even if the sequence of all our genes  is known, many of these are not yet related to a human pathology. For about 30% of congenital hearing impairments the analysis of known genes does not identify the causative anomaly. The implication of new genes in hearing defects currently involves the analysis (sequencing) of all the genes (exome) of one or more patients. This technology remains mostly in the field of research. However, the identification of new genetic causes of hearing loss would make it possible to increase the diagnostic rate .Regarding hearing impairment, genetic evaluation is rarely motivated by a request for genetic counseling in terms of prenatal diagnosis or preimplantation diagnosis. Most prenatal diagnosis applications for hearing defects are for syndromic forms in which hearing loss is associated with other disabilities or malformations, for example: Treacher–Collins syndrome, mitochondrial diseases, Alport syndrome, Usher syndrome or BOR syndrome. Couples asking for prenatal diagnosis in case of non-syndromic hearing loss are rare. Regardless of the context, both the availability of molecular tests and the legislative framework of each country must be considered.To date, no curative treatment is available in cases of sensorineural hearing impairment, but the identification of a genetic origin helps to better understand the normal functioning of the ear and the mechanisms at the origin of hearing impairment. This could be the first step towards the development of therapies, based on the determination of the causal genetic anomaly. In fact, the first studies of gene and/or cell therapies conducted in animals show promising prospects ,23,24,25Mandatory examinations for all casesPhenotype of proband case’s hearing impairment: age of onset, type of deficit, degree, evolution, laterality/symmetry and shape of the audiometric curve;Complete clinical examination, personal and familial history;CMV research on dried blood spot or CMV serology in the patient and their mother;Ophthalmological examination;Temporal bone and brain imaging;Audiograms of the kinship.Principal genetic investigationsTargeted molecular analysis: e.g., DFNB1 locus ; DFN16 locus ; SLC26A4 ;Chromosomal analysis by CGH array for polymalformative syndrome or a set of clinical signs associated with deafness that does not evoke a known diagnosis;Next generation sequencing: High-throughput sequencing technology to simultaneously analyze a set of genes implicated in syndromic or non-syndromic HL;Exome or genome analysis.