Audiological Tests Research Articles

Assessment of auditory perception of children with single-sided deafness after cochlear implantation

Unilateral deafness will lead to the decline of children's speech recognition rate, language development retardation and spatial positioning ability, which will have many adverse effects on children's life and study. Cochlear implantation can help children rebuild binaural hearing, and systematic audiological evaluation after operation is particularly important for clinicians to evaluate the hearing recovery of children. In this study, a variety of commonly used audiological evaluation, testing processes and methods after cochlear implantation in children with unilateral deafness are described in detail, and the related research status and results are summarized.

Sensorineural hearing loss among type 2 diabetic patients and its association with peripheral neuropathy: a cross-sectional study from a lower middle-income country

IntroductionDespite potential links between diabetes and sensorineural hearing loss (SNHL), routine hearing assessments for diabetic patients are not standard practice. Our study aimed to investigate the prevalence of SNHL and...

Audiological Characteristics of Vestibular Schwannoma Patients With Normal Pure-Tone Audiometry.

To investigate the audiological characteristics of vestibular schwannoma (VS) patients with normal pure-tone audiometry (PTA) results. A retrospective study. Forty-two VS patients with normal PTA results from October 2016 to October 2022 were included. Normal PTA was defined when the hearing threshold is ≤25 dB hearing loss (HL) in each test frequency and the PTA is ≤25 dB HL. Results of multiple audiological tests such as the auditory brainstem response (ABR), distortion product otoacoustic emission (DPOAE), multiple auditory steady-state responses threshold (ASSR), and speech discrimination score were retrospectively reviewed. Demographic data of these patients were also been collected. According to our results, the ABR and average ASSR threshold of the affected side were statistically significantly higher in VS patients with normal PTA. ABR waveforms on the affected side also showed more abnormalities. The DPOAE pass rates of the affected side were lower than the unaffected side while the amplitude and signal-to-noise ratio rate was also lower. In addition, we used magnetic resonance imaging 3-dimensional reconstruction images to measure the volume of tumors in these patients. We also found that higher ABR threshold means lager tumor size in patients with normal PTA. VS patients with normal PTA result cannot be assumed to have no impairment of hearing function. ABR, DPOAE, and ASSR results showed the characteristic changes in the affect ear. ABR threshold has the highest sensitivity for hearing abnormalities and is strong relative with tumor size in patients with normal PTA.

Endoscopic Broad Palisade Cartilage Graft Without Raising a Tympanomeatal Flap in the Repair of Subtotal Perforation.

Objective: This study evaluated the outcome of a broad palisade cartilage graft in the repair of subtotal perforation. Study Design: Prospective case series. Materials and Methods: This was a prospective study of 43 patients with subtotal perforations who underwent an endoscopic broad palisade cartilage graft procedure that did not include raising a tympanomeatal flap. The patients were followed up for 6 months. Results: The 43 patients (43 ears) included in this study had a mean operation time of 38.6 ± 7.4 minutes. Five (11.6%) patients were lost to follow-up; 38 (88.4%) completed the 6 month follow-up. The graft success rate in the latter was 92.1% (35/38). Audiological testing showed no sensorineural threshold shift. The mean preoperative air-bone gap (ABG) was 28.4 ± 5.1 dB, while the mean ABG at 6 months postoperatively was 13.6 ± 3.1 dB; the difference between these values was significant (P < .05; paired samples t test). According to the audiometry assessment, the successful surgery rate (postoperative ABG ≤ 20 dB) was 89.5% (34/38). No graft-related complications (eg, graft lateralization, significant blunting, graft medialization) were encountered during the follow-up period. However, granular myringitis with minimal moistness but without infection was noted in 5.3% (2/38) of the patients. Conclusions: In the repair of subtotal perforation, an endoscopic broad palisade cartilage graft, performed without raising a tympanomeatal flap, is simple and feasible, resulting in a high graft success rate and good hearing restoration.

The Role of Vascular Endothelial Dysfunction in Hypertension With Hearing Loss

Hypertension can cause hearing loss, but there is no clear definition of the mechanism(s) involved. The study aimed to explore the role of vascular endothelial dysfunction in hypertension with hearing loss. Patients with hypertension were divided into two groups based on hearing loss. Pure tone audiometry (PTA) and endothelial function testing were performed. A total of 216 (432 ears) hypertensive patients were divided into hypertension with hearing loss group (n = 104) and hypertension without hearing loss group (n = 112). The vascular endothelial biomarkers, ET-1 (endothelin-1) and vWF (von Willebrand factor) were significantly higher ( P &lt; .05) in the hypertension with hearing loss group. RHI (reactive hyperemia index), ET-1, and vWF were the factors related to hearing loss. The area under the receiver operating characteristic (ROC) curve (AUC) of RHI in the diagnosis of hypertension with hearing loss was .652 (95% CI .552-.751, P = .005), and the Youden index was 26.2%. The AUC of ET-1 was .706 (95% CI .612-.799, P = .001), and the Youden index was 38.9%. The AUC of vWF was .617 (95% CI .512-.721, P = .003), and the Youden index was 28.1%. Vascular endothelial dysfunction may play a role in the pathogenesis of hypertension with hearing loss.

Hearing dysfunction heralds an increase in non-motor burden and a worse quality of life in Parkinson's disease: new insights from non-motor spectrum.

Sensorial non-motor symptoms (NMSs) in Parkinson's disease (PD) still lack appropriate investigation in clinical practice. This study aimed to assess if and to what extent auditory dysfunction is associated with other NMSs in PD and its impact on patient's quality of life (QoL). We selected patients with idiopathic PD, without other concomitant neurological diseases, dementia, or diagnosis of any audiological/vestibular disease. Demographic and clinical data were collected. Patients underwent otoscopic examination, audiological testing with pure tone audiometry (PTA) and distortion product otoacoustic emissions (DPOAEs) and completed Non-Motor Symptoms Scale (NMSS) and Parkinson's Disease Questionnaires-39 (PDQ-39). ANCOVA and partial correlation analysis have been used for statistical analysis. 60 patients were enrolled and completed PTA and DPOAEs. 32 patients with hearing impairment (HI), assessed by PTA, (hearing threshold ≥ 25dB) showed similar disease duration, motor impairment, and staging, compared to patients without HI, but higher scores both in NMSS and in PDQ-39, except for cardiovascular (CV), gastrointestinal (GI), urogenital (U) and sexual function (SF) of NMSS. In addition, DPOAEs showed a significant correlation with higher scores both in NMSS and PDQ-39, except for CV, SF, GI, U and perceptual problem subdomains of NMSS. This study demonstrated that PD patients with HI have a greater burden of NMS and lower related QoL and functioning. Our results highlight the importance to reconsider HI as a NMS, in parallel with the others. HI evaluation, even in asymptomatic patients, may reveal a wider pathology with a worse QoL.

Evaluation of oVEMP and cVEMP in Superior Semicircular Canal Dehiscence Cases

Superior semisircular canal dehiscence (SSCD) is a disease of the audiovestibular system, which occurs as a result of the lack of bone layer covering the superior semisircular canal. The disease forms a third window in the inner ear due to the lack of bone layer. The third window that occurs forms a pseudo conductive type of hearing loss, which has decreased bone thresholds in the audiometry test. Vestibular evoked myogenic potentials are an electrophysiological measurement method in which the responses of the neck or eye muscles to an acoustic stimulus are recorded by means of electrodes. The purpose of use is to evaluate the functions of the urticle and saccule, which are the vestibular end organs. Therefore its primary purpose is to diagnose semicircular canal dehiscence. This review is based on research from the last decade; diagnostic results of ocular vestibular evoked myogenic potentials (oVEMP) and cervical vestibular evoked myogenic potentials (cVEMP) tests were evaluated in cases of superior semicircular canal dehiscence (SSCD). Studies indicate abnormal decreases in cVEMP test thresholds on the affected side in SSCD cases. Additionally, a significant increase in oVEMP amplitude on the affected side has been reported. Results; It shows that evaluating these two tests together will contribute to the diagnosis of semicircular canal dehiscence cases.

Spontaneous Cerebrospinal Fluid Leak Presenting as Middle Ear Effusion Caused by Arachnoid Granulations

While middle ear effusion (MEE) is a common ailment in children with Eustachian tube dysfunction, it occurs less frequently in adults. In such cases, a meticulous assessment is essential to discern the underlying cause of MEE. We present an unusual case of spontaneous cerebrospinal fluid (CSF) leak attributed to arachnoid granulation presenting as MEE. A 59-year-old obese female presented with right ear fullness, reduced hearing, and a persistent postnasal drip for 2 years. Audiological testing shows unilateral mild-to-moderate conductive hearing loss in the right ear. Tympanometry assessment revealed a persistent type B pattern in the right ear, indicating persistent unilateral conductive hearing loss. However, despite receiving treatment with nasal decongestants and antihistamines for 2 weeks, no signs of improvement were observed. Subsequently, a right myringotomy and grommet insertion were performed, resulting in the drainage of clear watery fluid that persisted for 2 weeks. A high-resolution computed tomographic scan of the temporal bone revealed the right MEE, bony defect in the right tegmen tympani, and bony irregularities of both tegmen tympani. Magnetic resonance imaging unveiled herniation of the right inferior temporal lobe through the identified tegmen defect. The patient subsequently underwent cortical mastoidectomy, lateral skull base repair, and mastoid obliteration. The histopathological examination of the biopsy taken from the right middle ear tissue revealed fragments of glial tissue with small fragments of trabeculae, which were confirmed by the positivity of the glial fibrillary acidic protein stain. The patient has remained asymptomatic for 10 months following the operation, and her hearing has fully returned to normal. It is advisable to consider CSF effusion as a potential differential diagnosis, particularly among adults aged over 50 years, even in the absence of a history of head trauma, mastoid surgery, or meningitis, when confronted with persistent unilateral MEE.

Speech Audiometry: The Development of Lithuanian Bisyllabic Phonemically Balanced Word Lists for Evaluation of Speech Recognition

Background and Objectives: Speech audiometry employs standardized materials, typically in the language spoken by the target population. Language-specific nuances, including phonological features, influence speech perception and recognition. The material of speech audiometry tests for the assessment of word recognition comprises lists of words that are phonemically or phonetically balanced. As auditory perception is influenced by a variety of linguistic features, it is necessary to develop test materials for the listener’s mother tongue. The objective of our study was to compose and evaluate new lists of Lithuanian words to assess speech recognition abilities. Materials and Methods: The main criteria for composing new lists of Lithuanian words included the syllable structure and frequency, the correlation between consonant and vowel phonemes, the frequency of specific vowel and consonant phonemes, word familiarity and rate. The words for the new lists were chosen from the Frequency Dictionary of Written Lithuanian according to the above criteria. Word recognition was assessed at different levels of presentations. The word list data were analyzed using a linear mixed-effect model for repeated measures. Results: Two hundred bisyllabic words were selected and organized into four lists. The results showed no statistically significant difference between the four sets of words. The interaction of the word list and presentation level was not statistically significant. Conclusions: Monaural performance functions indicated good inter-list reliability with no significant differences between the word recognition scores on the different bisyllabic word lists at each of the tested intensities. The word lists developed are equivalent, reliable and can be valuable for assessing speech recognition in a variety of conditions, including diagnosis, hearing rehabilitation and research.

Assessing the accuracy and reliability of application-based audiometry for hearing evaluation

Pure-tone audiometry (PTA) is the gold standard for assessing hearing loss. However, traditional PTA tests require specialized equipment, trained personnel, and a soundproof environment. Recently, smartphone-based PTA tests have been developed as an alternative method for hearing assessment. The aim of this study was to validate the accuracy and reliability of a smartphone application-based audiometry test. This study was conducted to assess the performance of application-based audiometry from November 2021 to January 2022. Pure-tone thresholds were measured using a smartphone application-based PTA test and compared with results obtained using a traditional audiometer in a sound-treated booth. The smartphone application used in this study was the "Care4Ear (Care4ear, version 1.0.6, MIJ Co., Ltd.)". Hearing thresholds less than 35 dB HL were classified as group A, 35-64 dB HL as group B, and 65 dB HL or greater as group C for the classification of hearing levels. We evaluated the accuracy of smartphone audiometry for each group and compared the results of frequency-specific hearing tests. Additionally, we examined the results of smartphone audiometry in individuals (n = 27) with asymmetric hearing loss. Seventy subjects completed both conventional audiometry and smartphone application-based hearing tests. Among the ears assessed, 55.7% were classified as group A, while 25.7% and 18.6% were classified as group B and group C, respectively. The average hearing threshold obtained from conventional pure-tone audiometry was 37.7 ± 25.2 dB HL, whereas the application-based hearing test yielded thresholds of 21.0 ± 23.0 dB HL. A significant correlation (r = 0.69, p < 0.01) was found between the average hearing thresholds obtained from the application-based and conventional pure-tone audiometry tests. The application-based test achieved a 97.4% hit rate for classifying hearing thresholds as class A, but lower rates of 22.2% for class B and 38.5% for class C. Notably, a discrepancy was observed between the hearing threshold measured by the application and the conventional audiometry for the worse ear with asymmetric hearing. The smartphone-based audiometry is a feasible method for hearing evaluation especially in persons with normal hearing. In cases of hearing loss or asymmetric hearing loss, the results of the application-based audiometry may be inaccurate, limiting its diagnostic utility.

Pediatric normative data for a novel and fast speech perception test in noise

ObjectivesCommunicating in noisy settings can be difficult due to interference and environmental noise, which can impact intelligibility for those with hearing impairments and those with normal hearing threshold. Speech intelligibility is commonly assessed in audiology through speech audiometry in quiet environments. Nevertheless, this test may not effectively assess hearing challenges in noisy environments, as total silence is rare in daily activities. A recently patented method, known as the SRT50 FAST, has been developed for conducting speech audiometry in noise. This new method enables the acceleration and simplification of free field speech audiometry tests involving competition noise.This study aims to establish normative scores and standardize the SRT50 FAST method as a test for evaluating speech perception in noise in pediatric patients. MethodsThe study included 30 participants with normal hearing, consisting of 11 females and 19 males, ranging in age from 6 to 11 years. A series of speech audiometry tests were conducted to determine the speech reception threshold 50% (SRT50) in competing conditions. This included testing both the fast mode (SRT50 FAST) currently being studied and the traditional method (SRT50 CLASSIC). The SRT50, or Signal to Noise Ratio (SNR) at which 50% of speech recognition occurred, was investigated for both methods. ResultsThe mean SRT50 FAST test score was −2.69 (SD = 3.15). The dataset exhibited a normal distribution with values ranging from 3.60 to −8.60. Since the scores are expressed in SRT, higher scores indicate poorer performance. We have established a threshold of 3.60 as the upper limit of the normal range, therefore, patients with scores above this threshold are considered to have abnormal results. ConclusionsThis study aimed to establish normative data for the evaluation of free field speech in noise recognition using the SRT50 FAST method in the pediatric population. This method accurately investigates the necessary signal-to-noise ratio for achieving 50% recognition scores with bisyllabic words in a quick manner. The ultimate objective is to employ this test to identify the optimal configuration of hearing rehabilitation devices, particularly for pediatric patients with hearing aids and/or cochlear implants. Additionally, it can be used to assess pediatric patients with unilateral hearing loss.

Early Results With the New Active Bone-Conduction Hearing Implant: A Systematic Review and Meta-Analysis.

The bone conduction implant (BCI) 602 is a new transcutaneous BCI with smaller dimensions. However, limited patient numbers restrict the statistical power and generalizability of the current studies. The present systematic review and meta-analysis summarize early audiological and medical outcomes of adult and pediatric patients implanted with the BCI 602 due to mixed or conductive hearing loss. Following the Preferred Reporting items for Systematic Reviews and Meta-analyses guidelines, 108 studies were reviewed, and 6 (5.6%) were included in the meta-analysis. The data on study and patient characteristics, surgical outcomes, and audiological test results were extracted from each article. Meta-analysis employed the fixed-effect and random-effects models to analyze the mean differences (MDs) between pre- and postoperative performances. In total, 116 patients were evaluated, including 64 (55%) adult and 52 (45%) pediatric patients. No intraoperative adverse events were reported, while postoperative complications were reported in 2(3.1%) adult and 2 (3.8%) pediatric patients. Studies consistently showed significant improvements in audiological outcomes, quality of life, and sound localization in the aided condition. In the meta-analysis, we observed a significant difference in the unaided compared to the aided condition in sound field thresholds (n = 112; MD, -27.05 dB; P < 0.01), signal-to-noise ratio (n = 96; MD, -6.35 dB; P < 0.01), and word recognition scores (n = 96; MD, 68.89%; P < 0.01). The implantation of the BCI 602 was associated with minimal surgical complications and excellent audiological outcomes for both the pediatric and the adult cohort. Therefore, our analysis indicates a high level of safety and reliability. Further research should focus on direct comparisons with other BCIs and long-term functional outcomes.

Tinnitus treatment: an experimental study

ObjectiveThe objective of the present study was to compare the effectiveness of the selected tinnitus management protocols for the experimental groups, based on the scores obtained for psychosocial variables, quality of life and severity of tinnitus between the four experimental groups following the various treatment protocols.MethodTwo-hundred adults ages ranged from 20 to 55 years (mean age 44.14, SD = 6.16) with mild-to-moderate sensorineural hearing loss without tinnitus were randomly selected for the control group, whereas the experimental group comprised 200 adults with mild-to-moderate sensorineural hearing loss with tinnitus without any other associated medical problems. Participants of both control and experimental groups underwent audiological tests and were administered the QoL questionnaire. The experimental group, in addition, was also administered the PSQ, TSI and THI questionnaire and psychoacoustic tests, i.e. measuring pitch, loudness and MML of tinnitus. Participants of experimental group were randomly assigned to 4 groups, each of 50 participants, and treatment was given as below: Group 1: only tinnitus masking sound was administered, Group 2: only counselling was given, Group 3: masking with counselling was given and Group 4: tinnitus masking combined with counselling and attention diversion task therapy was given.ResultThe patients without tinnitus had significantly better quality of life in comparison to the patients with tinnitus. Pre-post comparisons of the treatment groups revealed that “masking + counselling + attention diversion task” group showed highly significant differences for psychosocial aspects, QoL and severity of tinnitus. Further, pairwise comparison based on differences in mean scores indicated significant impact of “masking + counselling + attention diversion task” as compared to both in combination like “masking + counselling” or independently, i.e. masking or counselling alone.ConclusionIt appears that an integrated package of intervention (masking + counselling + attention diversion task) might be preferable for providing immediate tinnitus relief by masking through reducing tinnitus loudness and pitch by altering neuroplasticity (tonotopicity); in the long term, it reduces tinnitus impact through positive thinking by counselling treatment and also diverts attention to daily activity through attention training.

Turkish dichotic sentence mobile scan test results in young and elderly people

BackgroundThis study investigated the impact of age on dichotic listening performance by comparing results from the Turkish Dichotic Sentence Mobile Screening Test in young and elderly individuals with normal hearing. A total of 60 adults (30 young, 30 old) underwent otoscopic examination, audiological tests, and, for the elderly group, a mini-mental test. The Turkish Dichotic Sentence Mobile Screening Test was administered, and directed and undirected scores for the right and left ears were compared between age groups.ResultsIn the young group, the directed left ear mean (96.67% ± 8.02%), directed right ear mean (98.33% ± 3.79%), undirected left ear mean (97.67% ± 6.26%), and undirected right ear mean (98.67% ± 5.71%) were obtained. In the elderly group, corresponding values were lower: directed left ear mean (49.00% ± 36.33%), directed right ear mean (54.67% ± 40.66%), undirected left ear mean (64.00% ± 32.86%), and undirected right ear mean (65.00% ± 30.82%). Statistically significant differences were found between the young and elderly groups (p < 0.001), with the young group consistently outperforming the elderly group.ConclusionsThese results align with existing literature on dichotic listening tests, highlighting age-related declines in performance. The study contributes valuable insights into age-related changes in auditory processing and cognition, emphasizing the importance of considering dichotic listening as a marker of cognitive aging. Future research should explore potential interventions to mitigate age-related declines in dichotic listening performance.

Unilateral Sensorineural Hearing Loss in Children: Etiology, Audiological Characteristics, and Treatment.

The aim of this study was to evaluate audiological characteristics and parents' opinions on hearing device use in children with unilateral sensorineural hearing loss (USNHL) who attended a tertiary-level audiologic center. The medical charts of 70 children aged 6 to 12 years with USNHL were reviewed. In 51.4% of cases, the children were diagnosed with USNHL after the age of 2 years. The main causes of USNHL were congenital cytomegalovirus infection (21.4%) and unilateral cochlear nerve hypoplasia (12.9%). The percentage of patients wearing a hearing device was 45.7% (32/70); of these, 28 (87.5%) wore a conventional hearing aid, 2 (6.3%) a CROS device, and 2 (6.3%) a cochlear implant. Regarding the choice to use a hearing device, no significant differences were found between the subcategories of hearing loss degree (p = 0.55) and audiometric configuration (p = 0.54). Most parents of children with mild-to-severe USNHL observed improved attention (90.9%), and reduced fatigue and restlessness (86.4%) using the hearing aid. These children performed significantly better on all audiological tests (speech perception in quiet and in noise conditions, and sound localization) while wearing the hearing aid (p < 0.001). More efforts should be made to raise awareness among professionals and parents about the negative consequences of uncorrected USNHL.

Does coronavirus disease 2019 affect peripheral and central auditory systems? Matched group cross-sectional study and six-month follow up.

This study aimed to compare the peripheral-to-central auditory systems of people with coronavirus disease 2019 to a well-matched control group and examine the long-term effects of coronavirus disease 2019 on the auditory system. Participants who were outpatients of coronavirus disease 2019 (n = 30) were compared with a well-matched control group (n = 30). Behavioural and electrophysiological tests were performed, and tests were repeated at six months in the coronavirus disease 2019 group. Statistically significant differences were observed in the right ear at 10 kHz (p = 0.007) and 12.5 kHz (p = 0.028), and in the left ear at 10 kHz (p = 0.040) and 12.5 kHz (p = 0.040) between groups. The groups had no difference regarding the other audiological test results (p > 0.05). Extended high-frequency thresholds were affected in the coronavirus disease 2019 patients. No other findings indicated that the peripheral-to-central auditory system was affected. The effect on extended high-frequency thresholds appeared permanent, but no clinically significant new, late-onset auditory system effects were observed.

Detection of Reduced Diameter of the Cochlear Nerve in Long-term Deaf Patients Quantified With Semiautomatic Measurement of Nerve Cross-sectional Area Using 3T MRI Data.

High-resolution parallel transmit T2 sampling perfection with application optimized contrast using different flip angle evolution sequence with improved edge discrimination and semiautomatic determination of nerve cross-sectional area (CSA) can be used to evaluate nerve degeneration in the inner auditory canal (IAC) in long-term deaf patients. In patients with hearing loss, temporal bone MRI is routinely acquired to evaluate the morphology of the nerves within the IAC. Earlier studies have shown that the diameter of the cochlear nerve can be used as prognostic marker for the auditory performance after cochlear implantation in postlingually deaf patients. Eighty-two consecutive MRI scans were analyzed using a semiautomatic tool to measure CSA of cranial nerves in the IAC. Results were correlated with patient history and audiology testing as well as with age and gender. There was a significant reduced CSA of the cochlear nerve in ears with moderate-to-profound hearing loss and deafness compared with ears with normal hearing, but no significant difference in ears with mild-to-moderate hearing loss compared with normal hearing. In detail, normal hearing ears had a CSA of 1.23 ± 0.11 mm2, whereas ears with pantonal hearing loss of more than 40 dB had 1.02 ± 0.05 mm2 (P = 0.026). Maximal CSA of the facial nerve was not different among all groups (average, 1.04 mm2 ± 0.03; linear regression, P = 0.001) and stable with age. However, vestibular nerve CSA decreased significantly with age (average, 1.78 ± 0.05 mm2; linear regression, P = 0.128). In long-term deaf patients, smaller the diameter of cochlear nerve is the more severe the hearing loss is. The new semiautomatic tool can primarily be used to assess nerve diameter and possibly determine ears with nerve degeneration.

Examining Barriers to Early Hearing Diagnosis.

Many factors create barriers for early hearing detection and intervention (EHDI), especially those related to unfavorable social determinants of health (SDOH). The primary aim of this study was to evaluate diagnostic timing of infants at risk for congenital hearing loss in consideration of known barriers. Understanding the specific barriers to early diagnosis can inform interventions to improve timeliness of diagnosis and subsequent habilitation. A retrospective chart review was completed for infants referred for diagnostic audiologic testing at a tertiary urban-setting Children's Hospital from 2018 to 2021. After exclusion criteria were applied, 1,488 infants were included in the analysis. Various factors were recorded from electronic medical records including those specific to SDOH. Time to diagnosis was derived and compared across five factors of interest that have previously been shown to impact diagnostic timeline, including (a) insurance type, (b) race/ethnicity, (c) presence of middle ear dysfunction at first auditory brainstem response (ABR), (d) proximity to diagnostic center, and (e) diagnostic timing before and during/after the COVID-19 pandemic. Across the study time period, 77% of infants referred for diagnostic testing had confirmed diagnosis by the EHDI benchmark of 3 months. Analysis of time to diagnosis across factors of interest revealed no clinically significant differences for insurance type, race/ethnicity, proximity to diagnostic center, or timing in reference to the COVID-19 pandemic. Presence of middle ear dysfunction on first ABR was found to significantly protract final diagnostic timing. Although some known barriers for EHDI can be universal, other factors may have a differential impact on an infant's timeline to diagnosis based on their specific location, which can interact differently with additional known barriers. Understanding local challenges will serve to better guide programs in implementing facilitators that will address their specific needs for improved outcomes.

Speech-in-Noise Assessment in the Routine Audiologic Test Battery: Relationship to Perceived Auditory Disability.

Self-assessment of perceived communication difficulty has been used in clinical and research practices for decades. Such questionnaires routinely assess the perceived ability of an individual to understand speech, particularly in background noise. Despite the emphasis on perceived performance in noise, speech recognition in routine audiologic practice is measured by word recognition in quiet (WRQ). Moreover, surprisingly little data exist that compare speech understanding in noise (SIN) abilities to perceived communication difficulty. Here, we address these issues by examining audiometric thresholds, WRQ scores, QuickSIN signal to noise ratio (SNR) loss, and perceived auditory disability as measured by the five questions on the Speech Spatial Questionnaire-12 (SSQ12) devoted to speech understanding (SSQ12-Speech5). We examined data from 1633 patients who underwent audiometric assessment at the Stanford Ear Institute. All individuals completed the SSQ12 questionnaire, pure-tone audiometry, and speech assessment consisting of ear-specific WRQ, and ear-specific QuickSIN. Only individuals with hearing threshold asymmetries ≤10 dB HL in their high-frequency pure-tone average (HFPTA) were included. Our primary objectives were to (1) examine the relationship between audiometric variables and the SSQ12-Speech5 scores, (2) determine the amount of variance in the SSQ12-Speech5 scores which could be predicted from audiometric variables, and (3) predict which patients were likely to report greater perceived auditory disability according to the SSQ12-Speech5. Performance on the SSQ12-Speech5 indicated greater perceived auditory disability with more severe degrees of hearing loss and greater QuickSIN SNR loss. Degree of hearing loss and QuickSIN SNR loss were found to account for modest but significant variance in SSQ12-Speech5 scores after accounting for age. In contrast, WRQ scores did not significantly contribute to the predictive power of the model. Degree of hearing loss and QuickSIN SNR loss were also found to have moderate diagnostic accuracy for determining which patients were likely to report SSQ12-Speech5 scores indicating greater perceived auditory disability. Taken together, these data indicate that audiometric factors including degree of hearing loss (i.e., HFPTA) and QuickSIN SNR loss are predictive of SSQ12-Speech5 scores, though notable variance remains unaccounted for after considering these factors. HFPTA and QuickSIN SNR loss-but not WRQ scores-accounted for a significant amount of variance in SSQ12-Speech5 scores and were largely effective at predicting which patients are likely to report greater perceived auditory disability on the SSQ12-Speech5. This provides further evidence for the notion that speech-in-noise measures have greater clinical utility than WRQ in most instances as they relate more closely to measures of perceived auditory disability.

Analysis of congenital Zika syndrome clinicopathologic findings reported in the 8 years since the Brazilian outbreak

Aim: A Zika virus outbreak that began in Brazil, developed into an international public health emergency that extended from February 2015 until November 2016. Zika-infected pregnant women gave birth to a cohort of infants with congenital Zika syndrome (CZS) originally defined by severe microcephaly, retinal scarring, joint deformities, and hypertonia. This study examines the nature, extent, and severity of all CZS clinicopathologic findings described to date, compiled and analyzed by system. It reviews studies monitoring disease progression and proposing classification schemes for CZS stages. The teratogenic cellular and molecular mechanisms implicated in CZS pathogenesis are also discussed. Methods: A systematic review was conducted by literature search through WorldCat.org and ProQuest Central databases to identify studies on case series from the 2015–2016 CZS outbreak. Results: Twenty-six reports were included describing radiologic, ophthalmologic, audiologic, orthopedic, and laboratory test results in CZS cases including stillborns between 2016 and 2023. CZS neuropathology included prenatal and postnatal microcephaly, cerebral calcifications, quadriparesis, epilepsy, ventriculomegaly, reduced cerebral parenchyma, malformation of cortical development, and sleep electroencephalogram disturbances. Visual deficits were due to retinal and optic nerve lesions. Conductive and sensorineural hearing deficits were stable. Hypertonia, hypotonia, and spasticity with foot, hip, knee, and shoulder deformities resulted in arthrogryposis and restricted joint mobility. There was enlargement of immune organs, increased leukocyte counts, and cytokine dysregulation. Oro-craniofacial deformities affected the midface and caused dental eruption delay. Additional studies proposed that these systemic teratogenic effects could be attributable to transplacental Zika virus infection of multiple fetal progenitor cell lineages. Conclusions: The CZS-associated impairments in brain, eye, musculoskeletal, and immunologic functions caused disabilities that varied from moderate to severe, and significantly increased age-specific mortality rates. Further research is warranted to assess progression, classify stages, elucidate the precise molecular mechanisms mediating Zika teratogenicity, develop suitable therapeutic strategies, and design supportive social policies.