Normal Hearing Level Research Articles

Treacher Collins Syndrome 2 caused by a novel pathogenic variant in PLOR1D: clinical report and literature review

Objective: To investigate the clinical features, molecular etiology, and treatment of a family with Treacher Collins Syndrome 2 (TCS2). Methods: Information of the proband (female, 8 years old) including medical history and family history was collected. Physical examination and examinations concerning laboratory, audiology, and radiology were performed on the proband. Physical examination was also performed on the family members. Genomic DNA of proband was extracted for whole exome sequencing, and then the genomic DNA of family members was extracted for Sanger sequencing. POLR1D and TCS2 related literatures published before August 31,2023 were searched and sifted in PubMed and CKNI databases. The clinical characteristics of TCS2 were summarized. Results: The proband had poor hearing since childhood, with pure tone audiometry indicating conductive hearing loss. She had a smaller jaw, bilateral preauricular fistulas and cup-shaped ear deformities. Temporal bone CT scan revealed deformities in the left external ear canal, bilateral middle ear and inner ear. A bone-conduction hearing aid device was surgically implanted, resulting in restoration of almost normal hearing levels. The proband's mother also had a slightly smaller jaw. Genetic analysis revealed a novel heterozygous variant NM_015972.4:c.38_47del in the POLR1D gene in the proband, which was inherited from her mother. A review of the literature revealed no clear evidence of genotype-phenotype correlation in TCS2. Conclusions: Molecular diagnosis plays a vital role in the diagnosis of TCS2. Patients with normal facial phenotype may be carriers of pathogenic variants in the POLR1D gene and have the risk of passing it to the offsprings with complete penetrance. Proper bone conductive hearing devices can improve the quality of life of TCS2 patients.

Effectiveness of Noise Cancelling Earbuds in Reducing Hearing and Auditory Attention Deficits in Children with Autism.

Background/Objectives: Autism spectrum disorder (ASD) is a lifelong neurodevelopmental condition characterised by impairments in social communication, sensory abnormalities, and attentional deficits. Children with ASD often face significant challenges with speech perception and auditory attention, particularly in noisy environments. This study aimed to assess the effectiveness of noise cancelling Bluetooth earbuds (Nuheara IQbuds Boost) in improving speech perception and auditory attention in children with ASD. Methods: Thirteen children aged 6-13 years diagnosed with ASD participated. Pure tone audiometry confirmed normal hearing levels. Speech perception in noise was measured using the Consonant-Nucleus-Consonant-Word test, and auditory/visual attention was evaluated via the Integrated Visual and Auditory Continuous Performance Task. Participants completed these assessments both with and without the IQbuds in situ. A two-week device trial evaluated classroom listening and communication improvements using the Listening Inventory for Education-Revised (teacher version) questionnaire. Results: Speech perception in noise was significantly poorer for the ASD group compared to typically developing peers and did not change with the IQbuds. Auditory attention, however, significantly improved when the children were using the earbuds. Additionally, classroom listening and communication improved significantly after the two-week device trial. Conclusions: While the noise cancelling earbuds did not enhance speech perception in noise for children with ASD, they significantly improved auditory attention and classroom listening behaviours. These findings suggest that Bluetooth earbuds could be a viable alternative to remote microphone systems for enhancing auditory attention in children with ASD, offering benefits in classroom settings and potentially minimising the stigma associated with traditional assistive listening devices.

Phase I/II Study of a Vascular Endothelial Growth Factor Receptor Vaccine in Patients With NF2-Related Schwannomatosis.

The humanized antivascular endothelial growth factor (VEGF) antibody bevacizumab (Bev) is efficacious for the treatment of NF2-related schwannomatosis (NF2), previously known as neurofibromatosis type 2. This study evaluated the safety and efficacy of a VEGF receptor (VEGFR) vaccine containing VEGFR1 and VEGFR2 peptides in patients with NF2 with progressive schwannomas (jRCTs031180184). VEGFR1 and VEGFR2 peptides were injected subcutaneously into infra-axillary and inguinal regions, once a week for 4 weeks and then once a month for 4 months. The primary end point was safety. Secondary end points included tolerability, hearing response, imaging response, and immunologic response. Sixteen patients with NF2 with progressive schwannomas completed treatment and were assessed. No severe vaccine-related adverse events occurred. Among the 13 patients with assessable hearing, word recognition score improved in five patients at 6 months and two at 12 months. Progression of average hearing level of pure tone was 0.168 dB/mo during the year of treatment period, whereas long-term progression was 0.364 dB/mo. Among all 16 patients, a partial response was observed in more than one schwannoma in four (including one in which Bev had not been effective), minor response in 5, and stable disease in 4. Both VEGFR1-specific and VEGFR2-specific cytotoxic T lymphocytes (CTLs) were induced in 11 patients. Two years after vaccination, a radiologic response was achieved in nine of 20 assessable schwannomas. This study demonstrated the safety and preliminary efficacy of VEGFR peptide vaccination in patients with NF2. Memory-induced CTLs after VEGFR vaccination may persistently suppress tumor progression.

Clinical course of five patients definitively diagnosed with idiopathic perilymphatic fistula treated with transcanal endoscopic ear surgery.

An idiopathic perilymphatic fistula (PLF) can be difficult to diagnose because patients present with sudden sensorineural hearing loss (SSHL) and/or vestibular symptoms without any preceding events. In such cases, we currently test for cochlin-tomoprotein (CTP) to confirm the diagnosis of idiopathic PLF because CTP is only detected in the perilymph. In this study, we report the clinical course of five patients definitively diagnosed with idiopathic PLF who underwent PLF repair surgery using transcanal endoscopic ear surgery (TEES). Five patients were initially treated with intratympanic dexamethasone for SSHL, at which time a CTP test was also performed (preoperative CTP test). Due to refractory hearing loss and/or fluctuating disequilibrium, PLF repair surgery using TEES was performed to seal the oval and round windows using connective tissue and fibrin glue. These patients were diagnosed with definite idiopathic PLF based on pre- or intra-operative CTP test results (negative, < 0.4 ng/mL; intermediate, 0.4-< 0.8 ng/mL; and positive, > 0.8 ng/mL). We evaluated pre- and intra-operative CTP values, intraoperative surgical findings via a magnified endoscopic view, and pre- and post-operative changes in averaged hearing level and vestibular symptoms. Pre- and intra-operative CTP values were positive and intermediate in three patients, positive and negative in one patient, and negative and positive in one patient. None of the patients had intraoperative findings consistent with a fistula between the inner and middle ears or leakage of perilymph. Only two patients showed a slight postoperative recovery in hearing. Four patients complained of disequilibrium preoperatively, of whom two had resolution of disequilibrium postoperatively. A positive CTP test confirms PLF in patients without obvious intraoperative findings. The CTP test is considered more sensitive than endoscopic fistula confirmation. We consider that CTP test results are important indicators to decide the surgical indication for idiopathic PLF repair surgery. In our experience with the five cases, two of them showed improvements in both hearing and vestibular symptoms.

Investigation of Pathogenesis and Otolithic Organ Responses in Sailors with Seasickness Using cVEMP.

There are different theories cited as the cause of Motion Sickness. Asymmetrical otolithic organ function is one of the etiology theories of motion sickness. In our study, the aim was to reveal whether there is a cause originating from the sacculocolic reflex pathway in the physiopathology of seasickness. The patient group included 15 man sailors with diagnosis of seasickness and the control group included 15 man sailors without seasickness in our study. Cervical evoked myogenic vestibular potential (cVEMP) findings were compared between the groups. In the right and left ears of sailors with seasickness, the mean latencies of the p13 and n23 waves at 100, 95, and 90dB normal hearing level (nHL) levels were found significantly shorter than in the control group (p < 0.05, for all). In the seasickness group, cVEMP interaural wave amplitude asymmetry ratio at 100 nHL level were found significantly higher than the control group (p = 0.001). The findings of our study indicated that there may be reasons arising rom the sacculocolic reflex pathway in the pathophysiology of seasickness.

Assessment of Interaural Attenuation in Infants and Young Children Using Bone-Conducted Auditory Brainstem Response.

In hearing assessment, the term interaural attenuation (IAA) is used to quantify the reduction in test signal intensity as it crosses from the side of the test ear to the nontest ear. In the auditory brainstem response (ABR) testing of infants and young children, the size of the IAA of bone-conducted (BC) stimuli is essential for the appropriate use of masking, which is needed for the accurate measurement of BC ABR thresholds. This study aimed to assess the IAA for BC ABR testing using 0.5 to 4 kHz narrowband (NB) CE-chirp LS stimuli in infants and toddlers with normal hearing from birth to three years of age and to examine the effects of age and frequency on IAA. A total of 55 infants and toddlers with normal hearing participated in the study. They were categorized into three age groups: the young group (n = 31, infants from birth to 3 mo), middle-aged group (n = 13, infants aged 3-12 mo), and older group (n = 11, toddlers aged 12-36 mo). The participants underwent BC ABR threshold measurements for NB CE-chirp LS stimuli at 0.5 to 4 kHz. For each participant, one ear was randomly defined as the "test ear" and the other as the "nontest ear." BC ABR thresholds were measured under two conditions. In both conditions, traces were recorded from the channel ipsilateral to the test ear, whereas masking was delivered to the nontest ear. In condition A, the bone oscillator was placed on the mastoid of the test ear, whereas in condition B, the bone oscillator was placed on the mastoid contralateral to the test ear. The difference between the thresholds obtained under conditions A and B was calculated to assess IAA. The means of IAA (and range) in the young age group for the frequencies 0.5, 1, 2, and 4 kHz were 5.38 (0-15) dB, 11.67 (0-30) dB, 21.15 (10-40) dB, and 23.53 (15-35) dB, respectively. Significant effects were observed for both age and frequency on BC IAA. BC IAA levels decreased with age from birth to 36 mo. In all age groups, smaller values were observed at lower frequencies and increased values were observed at higher frequencies. BC IAA levels were both age and frequency dependent. The study found that the BC IAA values for lower stimulus frequencies were smaller than previously assumed, even in infants younger than 3 mo. These results suggest that masking should be applied in BC ABR threshold assessments for NB CE-chirp LS stimuli at 0.5, 1, and 2 kHz, even in young infants. Masking may not be necessary for testing at 4 kHz if a clear response is obtained at 15 dB normal-hearing level (nHL) in infants younger than 3 mo.

Центральные слуховые расстройства: причины, симптомы и способы преодоления дефицита в условиях учебного процесса

&lt;p style="text-align: justify;"&gt;Central Auditory Processing Disorders (CAPD) are impairments in the ability to recognize sounds, localize their sources, and/or determine their identity and meaning due to functional impairments of the central auditory system. CAPD is observed in individuals with normal hearing levels and is not the result of high-level speech or cognitive impairment. This disorder is not well known to Russian specialists in the field of correctional psychology, while according to foreign sources it is observed in 2&amp;mdash;7% of schoolchildren and is often combined with other developmental disorders (dyslexia, speech developmental disorders, ASD, ADHD). Here we provide a brief review of CAPD: its causes, symptoms, and approaches to diagnosis. In particular, we draw the readers' attention to the insufficient development of tools and practices for diagnosing and treating CAPD in Russia. Next, we review current approaches to the correction of CAPD, with special emphasis on assistive technologies that improve the signal-to-noise ratio (FM systems). Evidence in the literature indicates that in addition to the immediate effect (improved speech understanding), prolonged use of FM systems has a positive long-term effect based on mechanisms of neural plasticity.&lt;/p&gt;

Measurement of Ocular Vestibular Evoked Myogenic Potentials: Nasion Reference Montage as an Alternative to the Clinical Standard Montage.

To compare two novel electrode montages for ocular, vestibular evoked myogenic potential using single-nasion reference electrodes with the clinical standard montage. Randomized crossover experiment. Tertiary referral center. Sixty healthy participants. Normal hearing and vestibular function were confirmed with an extensive test-battery. All ocular, vestibular evoked myogenic potential settings were measured with air-conducted tone bursts at 100-dB normal hearing level and a frequency of 500 Hz. Three electrode montages were measured in randomized order: the clinical standard montage ("S"), the nasion reference montage ("N"), and the nasion reference montage with a more lateral active electrode ("L"). Upgaze was standardized to 35 degrees. Detection rate, latency of N1 and P1, peak-to-peak amplitude of N1 and P1, signal-to-noise ratio (SNR), asymmetry ratio (AR), concordance of expert assessment, and reliability. All electrode montages showed detection rates greater than 90%. Latencies for "L" were shorter than for "S" and "N." Amplitudes and SNR for "S" and "N" were higher than for "L," whereas the values for "S" and "N" did not differ significantly. For AR, no significant differences between the montages were assessed. Concordance of experts ranged from 78% for "L" and 89.8% for "N." All montages provided excellent day-to-day reliability (intraclass correlation coefficient ≥0.9) for amplitudes and SNR. Montage N could be a useful alternative to the clinical standard montage: although results are roughly equivalent, montage N requires one less electrode to do so.

Severity and Types of Hearing Loss in Patients with Hypertrophic Adenoids

Background: Hearing loss in children can significantly impact language development and social interaction. Hypertrophic adenoids are a common pediatric condition that can contribute to hearing impairments. Understanding the prevalence and severity of hearing loss in this demographic is crucial for effective management and intervention strategies. Objective: The study aimed to determine the prevalence and severity of hearing loss among children with hypertrophic adenoids, assessing the demographic characteristics and the types of hearing loss observed in this population. Methods: This observational cross-sectional survey was conducted over nine months at the Children's Hospital and Institute of Child Health, University of Lahore Teaching Hospital. The study included a sample size of 339 children aged 5-13 years, calculated based on a prevalence rate of 67.3% for mild hearing loss. Children with comorbidities like Down syndrome, Autism, ADHD, and other disabilities were excluded. Hearing assessments were conducted using an otoscope, a tympanometer, and a diagnostic audiometer (MAICO MA41 model). Data were analyzed using SPSS Version 25.0, focusing on the frequency and percentage of various types of hearing loss. Results: The study included 183 males (54%) and 156 females (46%), with a higher representation from rural areas (60%, n=203). Tympanometric analysis revealed 51.9% (n=176) with Type A tympanograms, 43.1% (n=146) with Type B, and 5% (n=17) with Type C. Audiometric results showed that 53.1% (n=180) of participants had normal hearing levels, while 27.1% (n=92) experienced mild hearing loss, 16.2% (n=55) had moderate hearing loss, 3.5% (n=12) had moderately severe hearing loss, 1.5% (n=5) had sensorineural hearing loss, and 2.1% (n=7) had mixed hearing loss. Conclusion: The study demonstrates a significant occurrence of mild to moderate conductive hearing loss in children with hypertrophic adenoids, particularly in rural settings. These findings highlight the need for regular auditory screening and early intervention strategies in this demographic to prevent long-term speech and language complications.

Higher Prevalence and Severity of Eosinophilic Otitis Media in Patients with Asthma-COPD Overlap Compared with Asthma Alone

Introduction: Eosinophilic otitis media (EOM) is well-known to frequently co-exist with adult-onset asthma. Both diseases are similar type 2 inflammation and are considered to have a “one airway, one disease” relationship. Asthma-chronic obstructive pulmonary disease (COPD) overlap (ACO), characterized by airway obstruction caused by airway wall thickening (AWT), is a severe condition with a higher incidence of mortality compared to asthma alone or COPD alone. Based on the “one airway, one disease” concept, we hypothesized that the inflammatory pathophysiology of EOM differs depending on its comorbidity with ACO or with asthma alone. Methods: A total of 77 chronic rhinosinusitis (CRS) patients with asthma were enrolled in this study. The subjects were divided into 2 groups: a group with comorbid asthma alone (asthma group; 46 patients), and a group with comorbid ACO (ACO group; 31 patients). The 2 groups were compared and assessed with regard to various factors, including the patients’ clinical characteristics, prevalence rate of EOM, EOM severity, EOMs relationships with smoking and AWT, and the eosinophil and neutrophil cell counts in the middle ear effusion (MEE). Results: The ACO group included significantly more males (p < 0.05), was significantly older (p < 0.05), and showed significantly lower lung function values (FEV1 [L], FEV1 [%pred]) (p < 0.01) compared with the asthma group. The ACO group also had a significant history of smoking as shown by the Brinkman index (p < 0.01) and greater AWT as assessed by high-resolution computed tomography (p < 0.05). The EOM prevalence rate was significantly higher in the ACO group (p < 0.05), especially with increased ACO severity (p < 0.05). The EOM severity was also significantly higher in the ACO group (p < 0.05) and also correlated with the ACO severity (p < 0.05). The pretreatment ear clinical characteristics score and the average air conduction hearing level were significantly higher in the ACO group (p < 0.05). The eosinophil percentage in the MEE/otorrhea was significantly lower in the ACO group (25.3%) than in the asthma group (54.7%) (p < 0.05). Conversely, the neutrophil percentage was significantly higher in the ACO group (75.7% vs. 41.9%) (p < 0.05). Conclusions: Our findings suggest that, in CRS patients with asthma, comorbidity with ACO may be a clinical factor leading to increased EOM prevalence and severity, as well as a higher neutrophil infiltration percentage in the middle ear. Cessation of smoking and early therapeutic intervention for ACO may mitigate progression of bronchial remodeling (i.e., reduce AWT) and help reduce the prevalence and severity of EOM.

Results of a Novel, Nonsurgical Bone-Conduction Hearing Aid for the Treatment of Conductive Hearing Loss in Australian Children.

To determine and compare the benefits a novel adhesive bone-conduction system and a conventional bone-conduction hearing aid (BCHA) on a softband for children with conductive hearing loss. Prospective, single-subject randomized, crossover trial. Tertiary referral center in Australia. Eight children aged from 4 to 17 years with conductive hearing loss. Rehabilitative with participants using the novel adhesive bone-conduction aid and a BCHA. Aided thresholds, as well as speech perception in quiet, unaided and aided with the novel adhesive bone-conduction aid and BCHA on a softband. For the six older children, speech in noise testing was also conducted. The mean unaided four frequency average hearing levels was 48 dB HL for air conduction, 10.5 dB HL for bone conduction, with a mean air-bone gap in the aided ear of 37.5 dB HL.Four-frequency average hearing level aided thresholds were 20.2 dB for the novel device and 19.8 dB for the BCHA, with no significant difference between the devices. Aided monosyllabic word scores improved from an average of 45% in the unaided condition to 81.6 and 85% for the novel adhesive and BCHA devices, respectively. Aided speech in noise performance improved, on average, by 1.6 dB SNR when wearing the BCHA and the novel adhesive device, with no significant difference in performance between the two devices. The novel device provided equivalent performance to the BCHA on all measures and can be considered as an alternative device for pediatric patients with conductive hearing loss.

Assessment of auditory processing skills in adolescents with cleft lip and palate

Objective Auditory deprivation due to middle ear defects caused by congenital malformations in the cleft lip and palate population may be a long-term risk factor for auditory processing disorder. The aim of the study is to assess the auditory processing skills in adolescents with cleft lip and palate by considering also maturation effect. Methods Thirty (10–17 years old) adolescents with operated, non-syndromic cleft lip and palate and thirty (10–17 years old) adolescents with normal development were included in the study. While both groups had bilateral normal peripheral hearing levels, study group had a history of chronic otitis media due to congenital malformations. In addition, participants were also evaluated in subgroups, under and over the age of 12, based on 12 years of age at which adult-like responses were obtained for auditory processing. Frequency pattern, duration pattern and Turkish matrix tests were used to assess auditory processing skills. Result The mean scores of the study group in frequency pattern, duration pattern and Turkish matrix tests were lower than the control group (p = 0.000). When the participants were evaluated as under and over the age of 12, while under the age of 12 showed lower performance than over the age of 12 for the study group (p < 0.05), this difference was not observed in the control group (p > 0.05). Conclusion Adolescents with cleft lip and palate may be disadvantaged in their auditory processing skills compared to their peers and they still may not catch up with their peers, even if improvement is observed with the maturation effect.

Influence of Wearing a Face Mask on Subjective Communication Difficulties According to Hearing Level

Background and Objectives The aim of this study was to assess the effect of wearing face masks on communication according to hearing levels using a questionnaire.Subjects and Method Individuals who underwent pure-tone audiometry at the otolaryngology department were invited to participate from September to December 2021. Each participant was asked to answer a questionnaire consisting of visual analog scales and open-ended questions about the experience of interactions involving face masks and the impact wearing face masks had on communication regarding listening, speaking and emotional exchange. The items of questionnaires were analyzed according to their hearing levels.Results A total of 119 subjects completed the questionnaire. The mean age of the subjects was 57.13±17.43 and the mean hearing threshold was 39.31±22.52 dB HL. Among them, 15.1% wore hearing aids. Across subjects, from those with the normal hearing level to those with moderate hearing loss, the average scores for subjective listening and speaking difficulty during conversation were significantly higher when individuals were wearing face masks than when they were not (p&lt;0.001). There was no significant difference in scores when asked about difficulty in exchanging emotion while wearing a mask.Conclusion Wearing a face mask might have a negative impact particularly on listening and speaking aspects of communication. There was no statistically significant difference in subjective difficulty scores according to hearing levels.

Presence of endolymphatic hydrops on listening difficulties in patients with normal hearing level

Background Listening difficulties (LiD) present difficulties in listening and paying attention to spoken information despite normal pure tone audiometry. Endolymphatic hydrops (EH) is a common inner ear condition associated with Ménière’s disease but may also be present in the asymptomatic ear. Objectives Using magnetic resonance imaging, we investigated EH in patients with LiD and assessed whether the severity of EH was related to the results of auditory processing tests (APTs). Materials and Methods 111 patients with no abnormalities on pure tone audiometry, but displaying difficulties in listening, underwent evaluation through APTs and questionnaires. Upon obtaining informed consent, the inner ears of 20 consenting patients were evaluated utilizing a 3-Tesla magnetic resonance imaging. Results A higher percentage of patients diagnosed with LiD by APTs had significant EH in the cochlea and vestibule than in previously reported control cases. The percentage of correct answers in the speech-in-noise test was significantly lower in patients with than in those without significant EH. Conclusion and significance In this study, significant EH of the cochlea was associated with poor listening to noise. The presence of EH affects the functioning of the auditory processing system, even in ears that test normally on standard audiometric tests.

Virtual audiometric testing using smartphone mobile applications to detect hearing loss.

The COVID-19 pandemic drove the need for remote audiometric testing in the form of mobile applications for hearing assessment. This study sought to determine the accuracy of two smartphone-based hearing assessment applications, Mimi and uHear, against the gold standard of in-clinic audiometric testing. One hundred patients that presented to clinic for hearing assessment were randomly assigned to take either the Mimi or uHear hearing test alongside standard audiometric testing. Hearing thresholds measured using mobile applications were compared to those from audiometric testing to assess validity. Patient satisfaction was measured using a questionnaire that queried if the app met the user's need, if they would recommend the app to others, and how likely they were to use the app again. Using Mimi, there were no differences in average hearing levels measured at any frequency when compared to standard audiometric testing. uHear overestimated hearing loss at 500 and 1000 Hz (p < .001 for both) by 5-10Hz, and underestimated hearing loss at 6000 Hz (p < .001) by 5-10Hz compared to standard audiometric testing. When stratified by level of hearing impairment, uHear overestimated impairment in those with normal hearing (p < .001). Mimi had higher sensitivity (0.971) and specificity (0.912) for hearing loss (defined as a pure tone average for 500, 1000, 2000, and 4000 Hz greater than 25 dB) than uHear (0.914 and 0.780, respectively). However, uHear outranked Mimi on all three questions in the satisfaction questionnaire (p=.01, p=.03, and p=.02, respectively). Mimi appears to be a reasonable substitute for standard audiometric testing when individuals cannot present to clinic for gold standard testing; however, the Mimi user experience can be improved. Level II.

Salivary Gland Choristoma of the Middle Ear: Case Report

Choristoma, a salivary gland tumor, may localize at unforeseen locations. Since 1961 when it was first described, roughly 50 cases of choristoma have been accounted for, including 30 cases among pediatric–adolescents. The patient in this current study is a girl of 14 years of age. She was admitted to a tertiary otolaryngology polyclinic with right-sided hearing problems and aural fullness complaint. Her average airway hearing level measured using the pure tone audiometry hearing test was 70 dB, whereas the bone conduction pure tone average was 6 dB. A high-resolution computerized tomography of the temporal bone demonstrated a mass behind the intact tympanic membrane. The tumor was excised entirely over the facial nerves tympanic part of the facial nerve with careful dissection. Histopathological examination revealed the tumor to be a salivary gland choristoma. In this article, we present the case of a 14-year-old girl with unilateral conductive hearing loss caused by salivary gland choristoma.

Changes in the Electrically Evoked Compound Action Potential over time After Implantation and Subsequent Deafening in Guinea Pigs

The electrically evoked compound action potential (eCAP) is a direct measure of the responsiveness of the auditory nerve to electrical stimulation from a cochlear implant (CI). CIs offer a unique opportunity to study the auditory nerve’s electrophysiological behavior in individual human subjects over time. In order to understand exactly how the eCAP relates to the condition of the auditory nerve, it is crucial to compare changes in the eCAP over time in a controlled model of deafness-induced auditory nerve degeneration. In the present study, 10 normal-hearing young adult guinea pigs were implanted and deafened 4 weeks later, so that the effect of deafening could be monitored within-subject over time. Following implantation, but before deafening, most examined eCAP characteristics significantly changed, suggesting increasing excitation efficacy (e.g., higher maximum amplitude, lower threshold, shorter latency). Conversely, inter-phase gap (IPG) effects on these measures – within-subject difference measures that have been shown to correlate well with auditory nerve survival – did not vary for most eCAP characteristics. After deafening, we observed an initial increase in excitability (steeper slope of the eCAP amplitude growth function (AGF), lower threshold, shorter latency and peak width) which typically returned to normal-hearing levels within a week, after which a slower process, probably reflecting spiral ganglion cell loss, took place over the remaining 6 weeks (e.g., decrease in maximum amplitude, AGF slope, peak area, and IPG effect for AGF slope; increase in IPG effect for latency). Our results suggest that gradual changes in peak width and latency reflect the rate of neural degeneration, while peak area, maximum amplitude, and AGF slope reflect neural population size, which may be valuable for clinical diagnostics.

Hearing Health Perceptions and Literacy Among Primary Healthcare Providers in the United States: A National Cross-Sectional Survey.

ObjectiveTo characterize current awareness, perceptions, and literacy surrounding hearing loss among United States primary healthcare professionals.Study designNational cross-sectional survey study.SettingUnited States.ParticipantsFour hundred six healthcare professionals.ResultsSurvey respondents included 205 primary care physicians and 201 nurse practitioners or physician assistants. When compared with 10 other common health conditions, only 1% of respondents ranked hearing loss as a “most important” health condition to manage. Less than half of providers reported recommending hearing testing for their patients at least once per year, whereas evaluation of blood pressure, total cholesterol, body mass index, and blood glucose levels are recommended at least annually by more than 80% of providers. Although 95% of respondents indicated that it is somewhat important or very important for patients to know the standard definition for normal hearing, only 57% of surveyed providers know of a standard definition themselves, and only 28% reported familiarity with the concept of “20/20 hearing.” Conversely, more than 80% of respondents know the “normal” metric for blood pressure, total cholesterol, body mass index, blood glucose, and vision. Most respondents realize that hearing is important to overall health and hearing loss can impact personal safety, lead to social isolation, and negatively impact quality of life. Fifty-four percent also acknowledged a link between hearing loss and depression, but a majority were not very aware of the relationship of hearing loss to risk of falling and dementia, reduced income and job opportunities, and type 2 diabetes. Importantly, only 40% of providers believe hearing loss is treatable, and only 17% believe it is preventable.ConclusionDespite widespread literacy of what constitutes normal blood pressure, total cholesterol, body mass index, blood glucose, and vision metrics, healthcare providers exhibit a poor understanding of normal hearing levels. Few providers prioritize hearing health or regularly recommend for annual hearing evaluation. Most providers believe that options for people with hearing loss are limited, which may have important implications for prioritizing discussion of hearing loss with patients.

Differential diagnosis and management of hemangioma at geniculate ganglion

Objective: To investigate the clinical characteristics, differential diagnosis, treatments and prognosis of facial nerve hemangioma and schwannoma at genicular ganglion, so as to provide reference for clinical diagnosis and treatments of facial nerve tumor at genicular ganglion. Methods: Clinical data of 13 patients with facial nerve tumors at genicular ganglion confirmed by postoperative pathology in the Ninth People's Hospital affiliated to Shanghai Jiaotong University School of Medicine from March 2018 to April 2020 were retrospectively analyzed, including seven cases of hemangioma and six cases of schwannoma. There were eight males and five females. Their ages ranged from 20 to 65, with an average age of 40. The course of disease ranged from 3 to 118 months, with an average of 52 months. All the patients underwent preoperative HRCT of the temporal bone and facial nerve dynamic contrast-enhanced(DCE) MRI examinations. All the patients had detailed surgical procedures and at least one-year postoperative follow-up. Results: On HRCT of the temporal bone, (4/7) hemangioma at geniculate ganglion showed characteristic honeycomb appearance, while 6/6 schwannoma and 3/7 hemangiomas showed expansive bone changes. On DCE-MRI, geniculate ganglion hemangioma (7/7) showed characteristic "point-to-surface" enhancement, and schwannoma (6/6) showed characteristic "face-to-surface" enhancement. For five hemangioma-patients with HB-Ⅱ-Ⅳ before surgery, the facial nerve anatomy was completely preserved through transcanal endoscopic approach(TEA), and the facial nerve function improved one year after surgery (two cases of HB-I, two cases of HB-Ⅱ, and one case of HB-Ⅲ). For two patients, with preoperative facial nerve function HB-Ⅴ-Ⅵ, since their tumors was inseparable from the nerves, they were performed with facial nerve anastomosis during the surgery, and the facial nerve function was improved to HB-Ⅳ level one year after surgery. For six patients with meningioma whose facial nerve function was greater than or equal to HB-Ⅲ, based on the preoperative hearing level, the involved segments, and duration of facial paralysis, three of them were conducted surgeries through middle cranial fossa approach, one by translabyrinthine approach, and one via mastoid approach. Two patients among them with complete facial paralysis over three years preoperatively were not performed facial nerve anastomosis after total resections of the tumors, and there was no improvement in facial nerve function one year after surgery. Three patients underwent facial nerve anastomosis after total tumor resections, and their facial nerve function was HB-Ⅲ in one patient, HB-Ⅳ in two patients one year after surgery. One patient (preoperative HB-Ⅲ) had a normal hearing level preoperatively, and the tumor involved the labyrinth segment. To protect the hearing, partial tumor was resected through the middle cranial fossa approach, and facial nerve function improved to HB-Ⅱ one year after surgery. Conclusions: Temporal bone HRCT combined with DCE-MRI are useful for the differential diagnosis of hemangioma and schwannoma at geniculate ganglion and provide references for preoperative clinical decision makings. It is extremely necessary to select the appropriate surgical approach based on the patient's hearing and involved segments. For geniculate ganglion hemangioma, early surgery can improve the possibilities of anatomical integrity of facial nerve, thereby improving facial nerve function postoperatively.TEA is a kind of surgical method worth consideration, with the characteristics of minimally invasive, favorable postoperative features, and so on. For schwannoma, one-stage functional reconstruction of the facial nerve is recommended during the resection of the tumors because of the inevitable damage to the anatomical integrity of the facial nerve.

Safety and Efficacy of Mastoidoplasty With Demineralized Bone Matrix in Canal Wall Down Mastoidectomy: A Pilot Study

Background and Objectives The canal wall down mastoidectomy brings changes in the anatomy of the external auditory canal (EAC), causing potential problems, such as accumulated crust, vertigo attacks, and difficulties in wearing hearing aids (HAs). The objective of this study is to evaluate the safety and efficacy of mastoidoplasty using the demineralized bone matrix (DBM) to obliterate the mastoidectomized cavity and reconstruct EAC.Subjects and Method Medical records of patients with chronic otitis media with or without cholesteatoma who received mastoidoplasty using DBM by a single surgeon at Seoul St. Mary’s hospital between 2014 and 2021 were reviewed retrospectively.Results A total of 27 patients were included in this study. None of the patients showed any recurrence of cavity problem, wound infection, or any other complications during their followup period of 13.07±37 months. The average air and bone conduction hearing level of pure tone audiometry showed no significant change after surgery (&lt;i&gt;p&lt;/i&gt;=0.50, &lt;i&gt;p&lt;/i&gt;=0.54, respectively). Five patients indicated for hearing rehabilitation could adopt canal type HAs after surgery; six patients used completely-in-the canal type HAs, and one patient used in-the-canal type HAs. None of the patients using HAs complained of acoustic feedback or any other problem in wearing HAs.Conclusion Mastoidoplasty using DBM seems to be a very safe and effective surgical procedure that shows functionally acceptable EAC for hearing rehabilitation with canal type HAs and demonstrates no specific complication.