RESUMEN

Introduction Hearing impairment in neonates and infants is a critical concern due to its potential to impede language acquisition, cognitive development, and overall quality of life. Brainstem-evoked response audiometry (BERA) stands out as a valuable diagnostic tool. The early detection of hearing impairments is paramount in neonatal care. Hearing loss during infancy can impede speech and language development, social interaction, and academic achievement. High-risk neonates, including those born prematurely or with low birth weight, have a heightened susceptibility to hearing impairment due to various factors such as exposure to ototoxic medications, mechanical ventilation, and complications associated with prematurity. Methods A hospital-based prospective study was conducted in the department of otorhinolaryngology; the study focused on high-risk neonates and infants from the outpatient department and inpatient department. The study was conducted from October 2022 to March 2024. A sample size of 70 patients was taken, including high-risk neonates and infants. Healthy term neonates and healthy infants were excluded from the study. Results In the current study, there were 40 males and 30 females. Among the infants surveyed, prematurity was the most prevalent risk factor, followed by perinatal asphyxia. Low birth weight was observed in 43% of cases, while hyperbilirubinemia and neonatal sepsis were the next. Among the 70 infants assessed, 50% were found to have normal hearing. Mild hearing loss was observed in 23% of cases, while 14% had moderate hearing loss. Severe and profound hearing loss were less common. Conclusion Our study highlighted the importance of early and routine auditory screening using BERA in high-risk neonates and infants, revealing a significant prevalence of hearing loss linked to various risk factors such as premature babies, low birth weight, hyperbilirubinemia, neonatal intensive care unit stay, perinatal asphyxia, and ototoxic drugs during pregnancy. Prematurity is the most common risk factor. For language development, early diagnosis and intervention were crucial. If babies have profound sensorineural hearing loss, they can go for a cochlear implant.

RESUMEN

Noise-induced hearing loss (NIHL) studies have focused on the lemniscal auditory pathway, but little is known about how NIHL impacts different cortical regions. Here we compared response recovery trajectories in the auditory and frontal cortices (AC, FC) of mice following NIHL. We recorded EEG responses from awake mice (male n = 15, female n = 14) before and following NIHL (longitudinal design) to quantify event related potentials and gap-in-noise temporal processing. Hearing loss was verified by measuring the auditory brainstem response (ABR) before and at 1-, 10-, 23-, and 45-days after noise-exposure. Resting EEG, event related potentials (ERP) and auditory steady state responses (ASSR) were recorded at the same time-points after NIHL. The inter-trial phase coherence (ITPC) of the ASSR was measure to quantify the ability of AC and FC to synchronize responses to short gaps embedded in noise. Despite the absence of click-evoked ABRs up to 90 dB SPL and up to 45-days post-exposure, ERPs from the AC and FC showed full recovery in ∼ 50 % of the mice to pre-NIHL levels in both AC and FC. The ASSR ITPC was reduced following NIHL in AC and FC in all the mice on day 1 after NIHL. The AC showed full recovery of ITPC over 45-days. Despite ERP amplitude recovery, the FC does not show recovery of ASSR ITPC. These results indicate post-NIHL plasticity with similar response amplitude recovery across AC and FC, but cortical region-specific trajectories in temporal processing recovery.

RESUMEN

OBJECTIVES: Standard diagnostic measures focus on threshold elevation but hearing concerns may occur independently of threshold elevation - referred to as "hidden hearing loss" (HHL). A deeper understanding of HHL requires measurements that locate dysfunction along the auditory pathway. This study aimed to describe the relationship and interdependence between certain behavioural and physiological measures of auditory function that are thought to be indicative of HHL. DESIGN: Data were collected on a battery of behavioural and physiological measures of hearing. Threshold-dependent variance was removed from each measure prior to generating a multiple regression model of the behavioural measures using the physiological measures. STUDY SAMPLE: 224 adults in the United States with audiometric thresholds ≤65 dB HL. RESULTS: Thresholds accounted for between 21 and 60% of the variance in our behavioural measures and 5-51% in our physiological measures of hearing. There was no evidence that the behavioural measures of hearing could be predicted by the selected physiological measures. CONCLUSIONS: Several proposed behavioural measures for HHL: thresholds-in-noise, frequency-modulation detection, and speech recognition in difficult listening conditions, are influenced by hearing sensitivity and are not predicted by outer hair cell or auditory nerve physiology. Therefore, these measures may not be able to assess threshold-independent hearing disorders.

RESUMEN

The binaural interaction component (BIC) of the auditory evoked potential is the difference between the waveforms of the binaural response and the sum of left and right monaural responses. This investigation examined BICs of the auditory brainstem (ABR) and middle-latency (MLR) responses concerning three objectives: 1) the level of the auditory system at which low-frequency dominance in BIC amplitudes begins when the binaural temporal fine structure is more influential with lower- than higher-frequency content; 2) how BICs vary as a function of frequency and lateralization predictability, as could relate to the improved lateralization of high-frequency sounds; 3) how attention affects BICs. Sixteen right-handed participants were presented with either low-passed (< 1000 Hz) or high-passed (> 2000 Hz) clicks at 30 dB SL with a 38 dB (A) masking noise, at a stimulus onset asynchrony of 180 ms. Further, this repeated-measures design manipulated stimulus presentation (binaural, left monaural, right monaural), lateralization predictability (unpredictable, predictable), and attended modality (either auditory or visual). For the objectives, respectively, the results were: 1) whereas low-frequency dominance in BIC amplitudes began during, and continued after, the Na-BIC, binaural (center) as well as summed monaural (left and right) amplitudes revealed low-frequency dominance only after the Na wave; 2) with a predictable position that was fixed, no BIC exhibited equivalent amplitudes between low- and high-passed clicks; 3) whether clicks were low- or high-passed, selective attention affected the ABR-BIC yet not MLR-BICs. These findings indicate that low-frequency dominance in lateralization begins at the Na latency, being independent of the efferent cortico-collicular pathway's influence.

Asunto(s)

RESUMEN

Introduction: This study investigates the comparative effectiveness of Click Auditory Brainstem Response (Click ABR) and Multiple Auditory Steady-State Response (Multi-ASSR) in identifying hearing impairments in infants. Recognizing auditory issues early is crucial for a child's cognitive and language development, as emphasized by the Joint Committee on Infant Hearing (JCIH) and the American Academy of Audiology (AAA). While Click ABR is widely utilized, Multi-ASSR offers a modern technique for detailed hearing assessment. Methods: A comparative analysis was conducted on 111 infants aged 1-6 months, previously screened for hearing at a tertiary care centre. The study employed both Click ABR and Multi-ASSR to evaluate their respective efficacy in assessing infant hearing. Results: Click ABR detected normal hearing in 87.4% of the infants, slightly higher than Multi-ASSR's 84.7%. A noteworthy finding was the higher incidence of bilateral versus unilateral hearing loss, with Click ABR identifying bilateral loss in 10 infants and unilateral loss in 4, compared to Multi-ASSR, which found bilateral loss in 12 infants and unilateral loss in 5. There was a minor but significant difference in auditory thresholds between the methods, with a mean discrepancy of 1.2 dB and a significant statistical variance (t-value of 15; p < 0.001), indicating variations in sensitivity. Conclusion: Both Click ABR and Multi-ASSR are indispensable tools in paediatric audiology, each with unique advantages. Click ABR excels in efficiency, suitable for rapid assessments and early detection. In contrast, Multi-ASSR offers comprehensive frequency-specific data, facilitating thorough evaluations. Healthcare professionals must grasp these methods' strengths to optimize infant hearing screenings and enhance early intervention strategies, aligning with JCIH and AAA guidelines. Supplementary Information: The online version contains supplementary material available at 10.1007/s12070-024-04639-2.

RESUMEN

Introduction: The Newborn Hearing Screening (NHS) program was officially launched in Jordan in 2021. Since its inception, no studies have examined the effectiveness of the program. This study seeks to assess the effectiveness and outcomes of the NHS program in Jordan. Methods: A retrospective cross-sectional study was conducted to investigate the program coverage rate, referral rate, loss to follow-up rate and the hearing status of newborns who successfully completed the necessary diagnostic assessment. Live births in all hospitals administered by the Ministry of Health (MoH) in Jordan from July 2021 to November 2023 were included. Results: Out of 25,825 newborns delivered, 99.4% (25,682) were screened. A referral rate of 0.7% (189) was recorded. Approximately 61.9% of those referred (n = 117) had normal hearing, while 31.7% (60 infants) were diagnosed with hearing loss. The prevalence of congenital hearing loss was 0.14%, and the mean age for identifying hearing loss was 11 months. Discussion: The current status of the NHS program in Jordan is promising. The program has achieved most benchmarks recommended by the Joint Committee on Infant Hearing (JCIH), demonstrating encouraging outcomes. There is a need to investigate and address the factors causing delays in the identification of hearing loss in Jordan.

RESUMEN

BACKGROUND: No studies to date have compared audiologic characteristics in patients with continuous and intermittent tinnitus. The present study classified tinnitus patients into continuous and intermittent groups based on tinnitus duration and compared their audiologic characteristics. METHODS: This study enrolled 604 patients with tinnitus from January 2019 to December 2022. Clinical manifestations, PTA results, the frequency and loudness of tinnitus, ABR, DPOAE, and TEOAE tests were compared in patients with continuous and intermittent tinnitus. RESULTS: Of the 604 patients, 231 (38.2%) had continuous and 373 (61.8%) had intermittent tinnitus. There were no significant between-group differences in otologic symptoms, tinnitus onomatopoeia. PTA showed that hearing thresholds, except at 125 Hz, were significantly higher in patients with continuous rather than intermittent tinnitus. The loudness of tinnitus was significantly greater in patients with continuous rather than intermittent tinnitus. ABR tests showed that the absolute latency of wave V was significantly longer in continuous than in intermittent tinnitus. Signal-to-noise ratios on TEOAE tests were significantly lower in patients with continuous rather than intermittent tinnitus at all frequencies tested (1, 1.5, 2, 3, and 4 kHz). Response rates to sound stimuli at all frequencies, except for 1 kHz, were significantly lower on DPOAE tests in patients with continuous rather than intermittent tinnitus. CONCLUSIONS: Continuous tinnitus is more common in males, more persistent over time, and is associated with a higher rate of hearing loss. In contrast, intermittent tinnitus is more common in women, appears acutely, and is associated with a relatively lower rate of hearing loss. Based on the findings of the current paper, it seems that audiologic characteristics may differ between patients with continuous and intermittent tinnitus.

RESUMEN

Background noise disrupts the neural processing of sound, resulting in delayed and diminished far-field auditory-evoked responses. In young adults, we previously provided evidence that cognitively based short-term auditory training can ameliorate the impact of background noise on the frequency-following response (FFR), leading to greater neural synchrony to the speech fundamental frequency(F0) in noisy listening conditions. In this same dataset (55 healthy young adults), we now examine whether training-related changes extend to the latency of the FFR, with the prediction of faster neural timing after training. FFRs were measured on two days separated by ~8 weeks. FFRs were elicited by the syllable "da" presented at a signal-to-noise ratio (SNR) of +10 dB SPL relative to a background of multi-talker noise. Half of the participants participated in 20 sessions of computerized training (Listening and Communication Enhancement Program, LACE) between test sessions, while the other half served as Controls. In both groups, half of the participants were non-native speakers of English. In the Control Group, response latencies were unchanged at retest, but for the training group, response latencies were earlier. Findings suggest that auditory training can improve how the adult nervous system responds in noisy listening conditions, as demonstrated by decreased response latencies.

RESUMEN

Ultrasound, or sound at frequencies exceeding the conventional range of human hearing, is not only audible to mice, microbats, and dolphins, but also creates an auditory sensation when delivered through bone conduction in humans. Although ultrasound is utilized for brain activation and in hearing aids, the physiological mechanism of ultrasonic hearing remains unknown. In guinea pigs, we found that ultrasound above the hearing range delivered through ossicles of the middle ear evokes an auditory brainstem response and a mechano-electrical transduction current through hair cells, as shown by the local field potential called the cochlear microphonic potential (CM). The CM synchronizes with ultrasound, and like the response to audible sounds is actively and nonlinearly amplified. In vivo optical nano-vibration analysis revealed that the sensory epithelium in the hook region, the basal extreme of the cochlear turns, resonates in response both to ultrasound within the hearing range and to harmonics beyond the hearing range. The results indicate that hair cells can respond to stimulation at the optimal frequency and its harmonics, and the hook region detects ultrasound stimuli with frequencies more than two octaves higher than the upper limit of the ordinary hearing range.

RESUMEN

Background: Hearing loss is an unknown complication of diabetes mellitus (DM). The aim of this study was to evaluate hearing function using auditory brainstem response (ABR) in diabetic patients. Methods: The present case-control study was performed on thirty diabetic patients as a case group and thirty healthy individuals as a control group. Baseline demographic information, HbA1c level, and duration of diabetes were obtained from all diabetic patients. In all subjects, the ABR and pure-tone audiometry (PTA) tests were performed and the results were analyzed using the t-test and logistic regression. Results: The absolute latency of I was significantly lower in diabetes patients. The absolute latency of III and the interpeak latencies (IPL) I-III were significantly higher in diabetic patients. No significant relationship was noticed in the absolute latency of V and the IPL I-V among diabetic patients in the right and left ears (P>0.05). Conclusion: The results of this study suggested that diabetes may cause central auditory dysfunction manifested on the absolute latency of III, the IPL I-III and III-V.

RESUMEN

OBJECTIVES: Evaluate pediatric auditory brainstem response (ABR) findings in children with Autism Spectrum Disorder (ASD) after the 2013 DSM-5 update. STUDY DESIGN: This was an IRB-approved, six-year retrospective chart review evaluating ABR results from pediatric patients with speech delay. Diagnosis of ASD and other neurodevelopmental abnormalities were collected for patient stratification. METHODS: From 2017 to 2023, 148 pediatric patients with speech delay were identified through diagnosis of speech delay and underwent ABR testing. Patients were then separated into two groups: Neurotypical (N = 79) and ASD (N = 69). ABR results were obtained through chart review and waveform and interpeak latency (IPL) results were recorded. Differences in waveform and IPL results were determined via Pearson's chi-square test, with multivariate analysis accounting for race, sex, and age. RESULTS: 28 patients with ASD (40.6 %) had at least one waveform/IPL prolongation. Analysis showed an increased incidence of waveform III (p = 0.028) and IPL III-V (p = 0.03) prolongation in the ASD group compared to their neurotypical counterparts. Waveform III prolongation was noted more in females with ASD (p = 0.001) than in males. No statistically significant difference when comparing race and age was found, except in the 2-3 age range (p = 0.003). CONCLUSIONS: There were higher percentages of prolongation for all waveforms and IPLs in the ASD group versus neurotypical, though not as high as previously reported. Race and age did not appear to be factors in ABR findings though more data is needed to make clinical associations.

Asunto(s)

RESUMEN

The frequency-following response (FFR) is an evoked potential that provides a neural index of complex sound encoding in the brain. FFRs have been widely used to characterize speech and music processing, experience-dependent neuroplasticity (e.g., learning and musicianship), and biomarkers for hearing and language-based disorders that distort receptive communication abilities. It is widely assumed that FFRs stem from a mixture of phase-locked neurogenic activity from the brainstem and cortical structures along the hearing neuraxis. In this study, we challenge this prevailing view by demonstrating that upwards of ~50% of the FFR can originate from an unexpected myogenic source: contamination from the postauricular muscle (PAM) vestigial startle reflex. We measured PAM, transient auditory brainstem responses (ABRs), and sustained frequency-following response (FFR) potentials reflecting myogenic (PAM) and neurogenic (ABR/FFR) responses in young, normal-hearing listeners with varying degrees of musical training. We first establish that PAM artifact is present in all ears, varies with electrode proximity to the muscle, and can be experimentally manipulated by directing listeners' eye gaze toward the ear of sound stimulation. We then show this muscular noise easily confounds auditory FFRs, spuriously amplifying responses 3-4-fold with tandem PAM contraction and even explaining putative FFR enhancements observed in highly skilled musicians. Our findings expose a new and unrecognized myogenic source to the FFR that drives its large inter-subject variability and cast doubt on whether changes in the response typically attributed to neuroplasticity/pathology are solely of brain origin.

RESUMEN

We aim to determine the accessibility of gold-standard hearing assessments - audiogram or auditory brainstem response (ABR) - during the first 3 months of hearing health care for children with and without developmental disabilities. Electronic health records were examined from children (0-18 years) who received hearing health care at three hospitals. Children with developmental disabilities had a diagnosis of autism, cerebral palsy, Down syndrome, or intellectual disability. Assessments from the first 3 months were reviewed to determine if ≥ 1 audiogram or ABR threshold was recorded. To evaluate differences in assessment based on disability status, logistic regression models were built while accounting for age, race, ethnicity, sex, and site. Of the 131,783 children, 9.8% had developmental disabilities. Whereas 9.3% of children in the comparison group did not access a gold-standard assessment, this rate was 24.4% for children with developmental disabilities (relative risk (RR) = 3.79; p < 0.001). All subgroups were at higher risk relative to the comparison group (all p < 0.001): multiple diagnoses (RR = 13.24), intellectual disabilities (RR = 11.52), cerebral palsy (RR = 9.87), Down syndrome (RR = 6.14), and autism (RR = 2.88). Children with developmental disabilities are at high risk for suboptimal hearing evaluations that lack a gold-standard assessment. Failure to access a gold-standard assessment results in children being at risk for late or missed diagnosis for reduced hearing. Results highlight the need for (1) close monitoring of hearing by healthcare providers, and (2) advancements in testing methods and guidelines.

RESUMEN

Hearing loss is the most common congenital sensory deficit worldwide and exhibits high genetic heterogeneity, making molecular diagnoses elusive for most individuals. Detecting novel mutations that contribute to hearing loss is crucial to providing accurate personalized diagnoses, tailored interventions, and improving prognosis. Copy number variants (CNVs) are structural mutations that are understudied, potential contributors to hearing loss. Here, we present the Abnormal Wobbly Gait (AWG) mouse, the first documented mutant exhibiting waltzer-like locomotor dysfunction, hyperactivity, circling behaviour, and profound deafness caused by a spontaneous CNV deletion in cadherin 23 (Cdh23). We were unable to identify the causative mutation through a conventional whole-genome sequencing (WGS) and variant detection pipeline, but instead found a linked variant in hexokinase 1 (Hk1) that was insufficient to recapitulate the AWG phenotype when introduced into C57BL/6J mice using CRISPR-Cas9. Investigating nearby deafness-associated genes revealed a pronounced downregulation of Cdh23 mRNA and a complete absence of full-length CDH23 protein, which is critical for the development and maintenance of inner ear hair cells, in whole head extracts from AWG neonates. Manual inspection of WGS read depth plots of the Cdh23 locus revealed a putative 10.4 kb genomic deletion of exons 11 and 12 that was validated by PCR and Sanger sequencing. This study underscores the imperative to refine variant detection strategies to permit identification of pathogenic CNVs easily missed by conventional variant calling to enhance diagnostic precision and ultimately improve clinical outcomes for individuals with genetically heterogenous disorders such as hearing loss.

Asunto(s)

RESUMEN

Listeners exhibit varying levels of tolerance for background noise during speech communication. It has been proposed that low tolerance of background noise may be the consequence of abnormally amplified gain in the central auditory system (CAS). Here, using a dataset of young adults with normal hearing thresholds, we asked whether central gain mechanisms might also explain cases of hypertolerance of background noise, as well as cases of reduced, but not abnormal, tolerance. We used the auditory brainstem response to derive a measure of CAS gain (wave V/wave I ratio) to compare listeners' background noise tolerance while listening to speech, grouping them into three categories: hyper, high, and medium tolerance. We found that hypertolerant listeners had reduced CAS gain compared to those with high tolerance. This effect was driven by wave V not wave I. In addition, the medium tolerant listeners trended toward having reduced wave I and reduced wave V amplitudes and generally higher levels of exposure to loud sound, suggestive of the early stages of noise-compromised peripheral function without an apparent compensatory increase in central gain. Our results provide physiological evidence that 1) reduced CAS gain may account for hypertolerance of background noise but that 2) increased CAS gain is not a prerequisite for medium tolerance of background noise.NEW & NOTEWORTHY Our findings strengthen the proposed mechanistic connection between background noise tolerance and auditory physiology by suggesting a link between hypertolerance and reduced central auditory gain, measured by the auditory brainstem response.

Asunto(s)

RESUMEN

OBJECTIVE: Recognition of auditory brainstem response (ABR) waveforms may be challenging, particularly for older individuals or those with hearing loss. This study aimed to investigate deep learning frameworks to improve the automatic recognition of ABR waveforms in participants with varying ages and hearing levels. STUDY DESIGN: The research used a descriptive study design to collect and analyze pure tone audiometry and ABR data from 100 participants. SETTING: The research was conducted at a tertiary academic medical center, specifically at the Clinical Audiology Center of Tsinghua Chang Gung Hospital (Beijing, China). METHODS: Data from 100 participants were collected and categorized into four groups based on age and hearing level. Features from both time-domain and frequency-domain ABR signals were extracted and combined with demographic factors, such as age, sex, pure-tone thresholds, stimulus intensity, and original signal sequences to generate feature vectors. An enhanced Wide&Deep model was utilized, incorporating the Light-multi-layer perceptron (MLP) model to train the recognition of ABR waveforms. The recognition accuracy (ACC) of each model was calculated for the overall data set and each group. RESULTS: The ACC rates of the Light-MLP model were 97.8%, 97.2%, 93.8%, and 92.0% for Groups 1 to 4, respectively, with a weighted average ACC rate of 95.4%. For the Wide&Deep model, the ACC rates were 93.4%, 90.8%, 92.0%, and 88.3% for Groups 1 to 4, respectively, with a weighted average ACC rate of 91.0%. CONCLUSION: Both the Light-MLP model and the Wide&Deep model demonstrated excellent ACC in automatic recognition of ABR waveforms across participants with diverse ages and hearing levels. While the Wide&Deep model's performance was slightly poorer than that of the Light-MLP model, particularly due to the limited sample size, it is anticipated that with an expanded data set, the performance of Wide&Deep model may be further improved.

RESUMEN

The present study attempted to understand the association between Auditory neuropathy spectrum disorder (ANSD) and Sickle cell anemia (SCA) and to recognize possible causative factors for the presence of ANSD in SCA individuals. Two cases, 24 years male and 17years female with a laboratory-confirmed diagnosis of Sickle cell anemia underwent detailed audiological evaluation i.e., pure tone audiometry, speech audiometry, immittance audiometry, otoacoustic emission, and auditory brainstem responses. Audiological evaluation revealed a bilateral moderate low-frequency sensorineural hearing loss in male and bilateral moderately severe sensorineural Hearing loss in female case with elevated Speech Recognition Threshold and poor Speech Identification Scores. 'A' type tympanogram with the absence of Acoustic reflexes and the presence of Otoacoustic emission with no distinct and reproducible peak V in Auditory Brainstem Response (ABR) at 90 dBnHL with the presence of ringing cochlear microphonics on polarity reversal collectively indicating bilateral ANSD in both cases. ANSD and SCA are reported to have a genetic basis of etiology. There might be possibilities that one genetic condition may be common in manifesting both conditions or one genetic condition can cause the presence of another genetic condition or can exaggerate the evolution of another genetic condition. Also, abnormal ABR findings indicate the possibility of neuropathological involvement in isolation or in combination with genetic abnormalities that need detailed investigation to understand non-genetic causative factors. Thus, paved the path for further research in this line and might provide better rehabilitative options.

RESUMEN

OBJECTIVE: Advancements in microsurgical technique and technology continue to improve outcomes in patients with skull base tumor. The primary cranial nerve eight monitoring systems used in hearing preservation surgery for vestibular schwannomas (VSs) are direct cranial nerve eight monitoring (DCNEM) and auditory brainstem response (ABR), although current guidelines are unable to definitively recommend one over the other due to limited literature on the topic. Thus, further research is needed to determine the utility of DCNEM and ABR. The authors performed a retrospective cohort study and created an interactive model that compares hearing preservation outcomes based on tumor size in patients receiving ABR+DCNEM and ABR-only monitoring. METHODS: Twenty-eight patients received ABR+DCNEM and 72 patients received ABR-only monitoring during VS hearing preservation surgery at a single tertiary academic medical center between January 2008 and November 2022. Inclusion criteria consisted of adult patients with a preoperative American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) hearing classification of A or B. Tumor size was measured as the maximal medial to lateral length, including the internal auditory canal component. RESULTS: Overall hearing preservation (word recognition score [WRS] > 0%) was achieved in 31 patients with ABR-only monitoring (43.1%) and in 18 patients with ABR+DCNEM (64.3%). Serviceable hearing preservation (AAO-HNS class A or B) was attained in 19 patients with ABR-only monitoring (26.4%) and in 11 patients with ABR+DCNEM (39.3%). There was no difference in overall hearing preservation between the two groups (p = 0.13). Change in tumor size was not associated with the odds of serviceable hearing preservation for the ABR-only group (p = 0.89); however, for ABR+DCNEM, there was some indication of an interaction between tumor size and the association of ABR+DCNEM versus ABR-only monitoring, with the odds of serviceable hearing preservation at p = 0.089. Furthermore, with ABR+DCNEM, every 0.5-cm increase in tumor size was associated with a decreased odds of serviceable hearing preservation on multivariable analysis (p = 0.05). For both overall and serviceable hearing preservation, a worse preoperative AAO-HNS classification was associated with a decreased odds of preservation (OR 0.43, 95% CI 0.19-0.97, p = 0.042; OR 0.17, 95% CI 0.053-0.55, p = 0.0031, respectively). CONCLUSIONS: The result of this interactive model study proposes that there may be a higher chance of hearing preservation when using ABR+DCNEM rather than ABR alone for smaller tumors, with that relationship reversing as tumor size increases.

RESUMEN

This study evaluates the efficacy of several Convolutional Neural Network (CNN) models for the classification of hearing loss in patients using preprocessed auditory brainstem response (ABR) image data. Specifically, we employed six CNN architectures-VGG16, VGG19, DenseNet121, DenseNet-201, AlexNet, and InceptionV3-to differentiate between patients with hearing loss and those with normal hearing. A dataset comprising 7990 preprocessed ABR images was utilized to assess the performance and accuracy of these models. Each model was systematically tested to determine its capability to accurately classify hearing loss. A comparative analysis of the models focused on metrics of accuracy and computational efficiency. The results indicated that the AlexNet model exhibited superior performance, achieving an accuracy of 95.93%. The findings from this research suggest that deep learning models, particularly AlexNet in this instance, hold significant potential for automating the diagnosis of hearing loss using ABR graph data. Future work will aim to refine these models to enhance their diagnostic accuracy and efficiency, fostering their practical application in clinical settings.

RESUMEN

BACKGROUND: Previously, we discovered a strain of Kunming mice, referred to as the KMush/ush strain, that exhibited notably abnormal electroretinogram (ERG) readings and elevated thresholds for auditory brainstem responses (ABRs), which resembled the characteristics of Usher Syndrome (USH). We successfully identified the pathogenic genes, Pde6b and Adgrv1, after KMush/ush crossbred with CBA/CaJ mice, referred to as CBA-1ush/ush, CBA-2ush/ush or CBA-2ush/ush. In this investigation, we crossbred KMush/ush and CBA/J mice to establish novel recombinant inbred lines and analysed their phenotypic and genotypic characteristics. METHODS: ERG readings, ABR testing, fundus morphology, histological examination of the retina and inner ear, reverse transcription-quantitative polymerase chain reaction (RT-qPCR) analysis, western blotting, DNA sequence analysis and behavioural experiments were performed to assess the phenotypes and genotypes of the progeny lines. RESULTS: No obvious waveforms in the ERG were detected in F1 hybrid mice while normal ABR results were recorded. The F2 hybrids, which were called J1ush/ush or J2ush/ush, exhibited segregated hearing-loss phenotypes. J1ush/ush mice had a retinitis pigmentosa (RP) phenotype with elevated ABR thresholds, whereas J2ush/ush mice exhibited only the RP phenotype. Interestingly, J1ush/ush mice showed significantly higher ABR thresholds than wild-type mice at 28 days post born (P28), and RT-qPCR and DNA-sequencing analysis showed that Adgrv1 gene expression was significantly altered in J1ush/ush mice, but histological analysis showed no significant structural changes in the organ of Corti or spiral ganglia. Further elevation of ABR-related hearing thresholds by P56 manifested only as a reduced density of spiral ganglion cells, which differed significantly from the previous pattern of cochlear alterations in CBA-2ush/ush mice. CONCLUSIONS: We successfully introduced the hearing-loss phenotype of inbred mice with USH into CBA/J mice, which provides a good animal model for future studies on the important physiological roles of the Adgrv1 gene in inner-ear structure and for therapeutic studies targeting Adgrv1-mutated USH.

Asunto(s)