OBJECTIVE: To evaluate and compare audiological outcomes of atresiaplasty and Bonebridge (BB) implantation in patients with unilateral congenital aural atresia (UCAA), to guide clinical decision-making.
METHODS: Twenty-seven subjects diagnosed with UCAA were included in the study. Thirteen were implanted with the BB, while 14 undergone atresiaplasty. All patients underwent pre-and post-surgery examinations, including pure-tone audiometry, sound field threshold (SFT), speech reception threshold (SRT), word recognition score (WRS), and horizontal sound source localization tests.
RESULTS: (1) Postoperatively, the average SFT decreased by 11.79 ± 5.93 dB HL in the atresiaplasty group and by 24.46 ± 9.36 dB HL in the BB group, with a significantly greater decrease in the BB group compared to the atresiaplasty group (P < 0.05). (2) Both groups demonstrated a significant improvement in average disyllabic WRS postoperatively under normal ear-masking conditions, with the BB group showing a significantly higher improvement than the atresiaplasty group. (3) When the speech signal was presented from the CAA side with noise from the normal hearing side, both surgical groups exhibited a significant decrease in postoperative signal-to-noise ratio compared to preoperative levels, with improvements of 2.14 ± 2.95 dB SNR in the atresiaplasty group and 4.92 ± 5.83 dB SNR in the BB group (P < 0.05). (4) The average minimum audible angle preoperative in the atresiaplasty group was 29.71 ± 18.42°, which decreased to 18.1 ± 10.07° at 6 months postoperatively, showing a statistically significant improvement (P < 0.05).
CONCLUSIONS: We concluded that both atresiaplasty and Bonebridge implantation can significantly improve speech perception under both quiet and noisy conditions in children with UCAA. BoneBridge implantation appears to provide better audiological outcomes than atresiaplasty. Atresiaplasty can significantly improve the accuracy of sound localization. No significant improvement in sound localization accuracy was observed in the short period after Bonebridge implantation. Further research should be conducted with a larger sample size and longer follow-up time.

OBJECTIVE: Little is known about the hearing characteristics in patients with congenital round window atresia (CRWA). This study aimed to investigate hearing characteristics in patients with CRWA by comparing them with two relatively common congenital middle ear anomalies: congenital stapedial fixation (CSF) and congenital ossicular discontinuity (COD).
METHODS: Literature searches yielded five patients with surgically confirmed CRWA (seven ears), who were included in the CRWA group, along with one of our patients. Air and bone conduction thresholds; air-bone gap (ABG); and presence and depth of the Carhart notch were analyzed. These audiometric variables in the CRWA group were compared with those in the CSF (n = 15) and COD (n = 22) groups, comprising patients identified from our institution\'s medical database.
RESULTS: Average bone and air conduction thresholds in the CRWA group were 16.4 (standard deviation [SD]: 2.9; 95 % confidence interval [CI]: 14.6-18.3) and 44.6 (SD: 3.5; 95 % CI: 42.6-47.3) dB hearing level (HL). Bone conduction thresholds at high frequencies (≥2 kHz) were higher than those at low frequencies (<2 kHz), while air conduction thresholds at high frequencies were lower than those at low frequencies: ABGs at high frequencies were significantly smaller than those at low frequencies (2 kHz vs. 0.5 kHz, p = 0.027; 2 kHz vs. 1 kHz, p = 0.041; 4 kHz vs. 0.5 kHz, p = 0.042; 4 kHz vs. 1 kHz, p = 0.027). There were no between-group differences in incidence and depth of the Carhart notch.
CONCLUSIONS: CRWA could manifest as a distinct audiometric pattern with poorer bone conduction and better air conduction at ≥2 kHz, resulting in significantly smaller ABGs at higher frequencies than that at lower frequencies. Our findings indicated that this pattern differed from that of CSF and COD. The unique beer bottle-shaped audiogram associated with CRWA might facilitate its early diagnosis in patients with congenital conductive hearing loss.

Hearing loss is common among Veterans, and extensive hearing care resources are prioritized within the Veterans Administration (VA). Severe hearing loss poses unique communication challenges with speech understanding that may not be overcome with amplification. We analyzed data from the VA Audiometric Repository between 2005 and 2017 and the relationship between hearing loss severity with speech recognition scores. We hypothesized that a significant subset of Veterans with severe or worse hearing loss would have poor unaided speech perception outcomes even with adequate audibility. Sociodemographic characteristics and comorbidities were compiled using electronic medical records as was self-report measures of hearing disability. We identified a cohort of 137,500 unique Veterans with 232,789 audiograms demonstrating bilateral severe or worse hearing loss (four-frequency PTA > 70 dB HL). The median (IQR; range) age of Veterans at their first audiogram with severe or worse hearing loss was 81 years (74 to 87; 21-90+), and a majority were male (136,087 [99%]) and non-Hispanic white (107,798 [78.4%]). Among those with bilateral severe or worse hearing loss, 41,901 (30.5%) also had poor speech recognition scores (<50% words), with greater hearing loss severity correlating with worse speech perception. We observed variability in speech perception abilities in those with moderate-severe and greater levels of hearing loss who may derive limited benefit from amplification. Veterans with communication challenges may warrant alternative approaches and treatment strategies such as cochlear implants to support communication needs.

Musicians are at risk of hearing loss and tinnitus due to regular exposure to high levels of noise. This level of risk may have been underestimated previously since damage to the auditory system, such as cochlear synaptopathy, may not be easily detectable using standard clinical measures. Most previous research investigating hearing loss in musicians has involved cross-sectional study designs that may capture only a snapshot of hearing health in relation to noise exposure. The aim of this study was to investigate the effects of cumulative noise exposure on behavioural, electrophysiological, and self-report indices of hearing damage in early-career musicians and non-musicians with normal hearing over a 2-year period. Participants completed an annual test battery consisting of pure tone audiometry, extended high-frequency hearing thresholds, distortion product otoacoustic emissions (DPOAEs), speech perception in noise, auditory brainstem responses, and self-report measures of tinnitus, hyperacusis, and hearing in background noise. Participants also completed the Noise Exposure Structured Interview to estimate cumulative noise exposure across the study period. Linear mixed models assessed changes over time. The longitudinal analysis comprised 64 early-career musicians (female n = 34; age range at T0 = 18-26 years) and 30 non-musicians (female n = 20; age range at T0 = 18-27 years). There were few longitudinal changes as a result of musicianship. Small improvements over time in some measures may be attributable to a practice/test-retest effect. Some measures (e.g., DPOAE indices of outer hair cell function) were associated with noise exposure at each time point, but did not show a significant change over time. A small proportion of participants reported a worsening of their tinnitus symptoms, which participants attributed to noise exposure, or not using hearing protection. Future longitudinal studies should attempt to capture the effects of noise exposure over a longer period, taken at several time points, for a precise measure of how hearing changes over time. Hearing conservation programmes for \"at risk\" individuals should closely monitor DPOAEs to detect early signs of noise-induced hearing loss when audiometric thresholds are clinically normal.

Hearing impairment in patients with chronic kidney disease (CKD), can affect the quality of life. At present, hearing dysfunction does not have an approved pharmacologic therapy. This study aimed to investigate the protective effects and possible mechanisms of curcumin as a therapeutic agent on hearing impairment in patients with chronic kidney disease. We conducted a randomized controlled trial of 40 chronic kidney disease patients not on dialysis with hearing impairment. Participants were randomly divided into two groups. One group received curcumin daily and the other received a placebo for 12 weeks. The interval between III and V waves, latency of wave V, auditory brain stem response (ABR) threshold, speech reception threshold (SRT), and speech discrimination score (SDS) were evaluated and analyzed before and after the intervention. After treatment, in the curcumin group, III-V waves interval and the latency of wave V were significantly reduced (P value < 0.0001), also ABR threshold was demonstrated a significant improvement (P value < 0.0001). In the trial group, the SDS was increased (P = 0.001) and the SRT was attenuated (P < 0.0001). We had either significant deterioration due to the course of the disease or insignificant changes in the placebo group. Daily administration of curcumin, can significantly improve hearing impairment in CKD patients. Accordingly, curcumin should be considered as a therapeutic option for treating hearing impairment in patients with chronic kidney disease.

OBJECTIVE: The objective of this study is to assess the accuracy of the Mimi Hearing Test (MHT) mobile application in the detection of air conduction (AC) thresholds and in screening for moderate hearing loss.
METHODS: Prospective clinical study.
METHODS: Tertiary care center.
METHODS: Participants with or without a varying degree of hearing loss, aged 18 years and over, without cognitive impairment and without active otorrhea or earwax impaction at the time of the hearing assessment were included.
METHODS: Subjects with a ranging severity of hearing loss underwent a conventional in-booth audiogram as well as mobile-based screening with MHT in a quiet room (45.5 dB background noise) on the same day. Both regular over-the-ear and noise-canceling headphones were tested with MHT.
METHODS: Comparisons of AC thresholds between conventional audiometry and mobile-based audiometry at discrete frequencies and with pure-tone averages (PTA) were performed.
RESULTS: A total of 75 adults (mean age: 56.2 yr, 54.7% male) were recruited and 63 used for analysis. Of the thresholds measured with MHT using regular headphones, 44.0% were within 10 dB of the conventional audiogram, compared to 39.3% using noise-canceling headphones. MHT demonstrated best accuracy at high frequencies (4-8 kHz). When screening for moderate hearing loss (PTA >40 dB HL), MHT demonstrated a sensitivity and specificity of 100.0% and 80.2%, respectively.
CONCLUSIONS: MHT is reliable for identifying moderate hearing loss but lacks precision in detecting thresholds at low frequencies. Noise canceling headphones seem to improve its precision at 4,000 Hz only.

This multi-center study examined the safety and effectiveness of cochlear implantation of children between 9 and 11 months of age. The intended impact was to support practice regarding candidacy assessment and prognostic counseling of pediatric cochlear implant candidates. Data in the clinical chart of children implanted at 9-11 months of age with Cochlear Ltd devices at five cochlear implant centers in the United States and Canada were included in analyses. The study included data from two cohorts implanted with one or two Nucleus devices during the periods of January 1, 2012-December 31, 2017 (Cohort 1, n = 83) or between January 1, 2018 and May 15, 2020 (Cohort 2, n = 50). Major adverse events (requiring another procedure/hospitalization) and minor adverse events (managed with medication alone or underwent an expected course of treatment that did not require surgery or hospitalization) out to 2 years post-implant were monitored and outcomes measured by audiometric thresholds and parent-reports on the IT-MAIS and LittlEARS questionnaires were collected. Results revealed 60 adverse events in 41 children and 227 ears implanted (26%) of which 14 major events occurred in 11 children; all were transitory and resolved. Improved hearing with cochlear implant use was shown in all outcome measures. Findings reveal that the procedure is safe for infants and that they show clear benefits of cochlear implantation including increased audibility and hearing development.

OBJECTIVE: To investigate which factors influence the adherence to hearing aid (HA) use in elderly patients with moderate-to-severe hearing loss.
METHODS: This observational, prospective, single-centre study enrolled patients with moderate-to-severe hearing loss. They were evaluated before and 1 year after having either one or two HAs fitted.
RESULTS: A total of 86 patients were enrolled in the study and of these 69.8% (60 of 86; USER group) continued to use their HA at 1 year after fitting; six patients had not continued their use (NON-USERS). The USER group was younger than the NON-USER group, but the difference was not significant. The USER group had a significantly better unaided auditory threshold at baseline than the NON-USER group. HA use resulted in improvements in speech audiometry and auditory threshold. There was also a maintenance of cognitive function in the USER group.
CONCLUSIONS: Use of HA for 1 year resulted in improved auditory performance and an absence of a deterioration of cognitive function.Trial registration: This research was retrospectively registered under no. NCT04333043 at ClinicalTrials.gov (http://www.clinicaltrials.gov/) on the 26 March 2020. This research has been registered with the Ethics Committee of the Area Vasta Emilia Nord under number 104, date of approval 17/07/2017.

UNASSIGNED: Clinically, we find that tinnitus patients often have hearing loss. According to the most accepted mechanism of tinnitus, that is, the spontaneous discharge and abnormal synchronization of neurons after afferent reduction, tinnitus frequency is closely related to the frequency of hearing loss.
UNASSIGNED: The purpose of this study was to investigate the correlation of tinnitus pitch with the frequency of hearing loss.
UNASSIGNED: A total of 500 patients with unilateral or bilateral chronic tinnitus were enrolled in this study. All patients underwent pure tone audiometry (PTA) and tinnitus acoustic examination. Hearing loss levels and frequencies were recorded. The relationship between tinnitus pitch and hearing loss level and frequency was statistically analyzed.
UNASSIGNED: Our results showed that 96.6% of the 500 tinnitus patients had hearing loss. Statistical analysis showed that low frequency (LF) tinnitus was correlated with LF hearing loss, but moderate frequency & high frequency (MF&HF) tinnitus was not significantly associated with MF&HF hearing loss. The coincidence of tinnitus pitch with the highest hearing threshold correlated with the degree of hearing loss.
UNASSIGNED: The vast majority of patients with chronic subjective tinnitus had hearing loss, and the frequency of tinnitus correlated with the degree and frequency of hearing loss but not exactly fall within the frequency range of hearing loss.

Objective: The purpose of this study was to analyze the clinical characteristics of auditory neuropathy (AN) patients with normal hearing or mild hearing loss. Methods: Data from Multicenter Study on Clinical Diagnosis and Intervention of Acoustic Neuropathy (registration number: ChiCTR2100050125). According to the Chinese clinical practice guideline of auditory neuropathy (version 2022), these patients divided into two groups: the normal hearing group (PTA Normal, PTAN group, the average hearing threshold<20 dB HL) and the mild hearing loss group (PTA Mild hearing loss, PTAM group, the average hearing threshold between 20-35 dBHL). The audiology characteristics, clinical features, and follow-up were analyzed. Data analysis was conducted using GraphPad Prism 8 and SPSS 20.0 software. Results: A total of 75 AN with normal hearing or mild hearing loss were included in this study. The PTAN group consisted of 19 patients (38 ears), including 12 males and 7 females. The average onset age was (16.9±4.5) years old, while the test age was (22.1±5.8) years old for PTAN group. The PTAM group consisted of 56 patients (112 ears), including 29 males and 27 females. The average onset age was (16.2±7.9) years old, while the test age was (23.9±9.0) yeas old for PTAM group. The average hearing threshold of low frequency (0.125-0.5 kHz) was significantly decreased. ABR disappeared in 86.00% (126/150) of the patients. The speech recognition rate was 71.80±22.44% in the PTAN group and 58.08±29.28% in the PTAM group.-SP/AP was 0.98±0.47 in the PTAN and 1.07±0.63 in PTAM group; 40 (53.33%) patients had tinnitus. 29 patients (58 ears) were followed up, including 10 patients (20 ears) in the PTAN group and 19 patients (38 ears) in the PTAM group. There was no significant change in hearing threshold in short-term follow-up (<3 years). With the extension of the disease duration (>3 years), the PTAN group tended to decrease at low frequency, and the PTAM group decreased at high frequency first. The hearing threshold at 0.25 kHz in the PTAN group and 4 kHz in the PTAM group decreased significantly. Conclusions: AN patients with normal hearing or mild hearing loss exhibit abnormal results in audiological examination results, including ABR, electrocochleography and speech discrimination score. A combination of audiological tests should be used to make the diagnosis of AN. With the progression of the disease, AN with normal hearing or mild hearing loss tends to decrease.
目的： 探究纯音测听气导平均听阈（pure-tone average，PTA）正常或轻度听力损失的听神经病（auditory neuropathy，AN）患者的临床特征及其听力变化规律。 方法： 研究对象来自听神经病临床诊断和干预多中心研究（注册号：ChiCTR2100050125），AN诊断标准依照2022年中国听神经病临床实践指南。筛选首诊纯音测听平均听阈（500、1 000、2 000、4 000 Hz）正常或存在轻度听力损失的AN患者，分为纯音平均听阈正常组（PTA-Normal组，即PTAN组，平均听阈<20 dBHL）和轻度听力损失组（PTA-Mild hearing loss组，即PTAM组，平均听阈20~35 dBHL），观察记录患者的听力学特征、临床表现和伴随症状以及随访结果，采用GraphPad Prism 8及SPSS 20.0软件进行数据分析。 结果： 共纳入纯音平均听阈正常或轻度听力损失的AN患者75例（150耳），其中PTAN组19例（38耳），男12例、女7例，发病年龄（16.9±4.5）岁（均数±标准差，下同）、检测年龄为（22.1±5.8）岁；PTAM组共56例（112耳），男29例、女27例，发病年龄（16.2±7.9）岁、检测年龄（23.9±9.0）岁。两组患者首诊低频（125~500 Hz）平均听阈显著升高；86.0%（126/150）的患耳听性脑干反应（ABR）波形缺失；PTAN组言语识别率为71.8%±22.4%，PTAM组言语识别率为58.1%±29.3%；PTAN组耳蜗电图-SP/AP为0.98±0.47，PTAM组为1.07±0.63。75例AN患者中40例（53.3%）存在耳鸣，部分患者还伴有其他系统功能异常。29例患者完成随访，其中PTAN组10例，PTAM组19例。短期随访（<3年），29例患者听阈均未发生明显变化；随着病程延长（>3年），PTAN组趋向于低频听力先下降，而PTAM组则表现为高频听力先下降，PTAN组250 Hz与PTAM组4 000 Hz听阈均显著升高。 结论： 纯音平均听阈正常或轻度听力损失的AN患者存在ABR、耳蜗电图、言语识别率等听力学检查结果异常，临床实践中应组合运用各项听力学检查对其进行综合评估；随着病程延长，这部分患者的听力逐渐下降，应动态评估其听力改变。.