Search

Background: The reported data on European universal neonatal hearing screening (UNHS) practices tend to be scarce, despite the fact that the European Union project, EUScreen, collected unofficial data from 38 collaborating European institutions. The objectives of this systematic review were as follows: (a) to identify the most recent (in a 20-year span) literature information about UNHS programs in Europe and (b) to provide data on the procedures used to assess the population, the intervention policies, and on the estimated prevalence of congenital hearing loss with emphasis on the bilateral hearing loss cases. Methods: Queries were conducted via the Pubmed, Scopus and Google Scholar databases for the time period of 2004–2024. The Mesh terms used were "OAE", "Universal Neonatal Hearing Screening", "congenital hearing loss" and "well babies". Only research articles and review papers of European origin were considered good candidates. The standard English language filter was not used, in order to identify information from non-English-speaking scientific communities and groups. Results: Very few data and reports were identified in the literature search. Eleven manuscripts were identified corresponding to eight UNHS programs. Except in Poland, most of the data refer to regional and not national programs. The screening coverage estimates of all programs exceed 90%; infants were mostly assessed by a three-stage protocol (TEOAE + TEOAE + AABR), followed by a clinical ABR test. The average prevalence (i.e., from well babies AND NICU infants) of bilateral hearing loss ranged from 0.5 to 20.94 per 1000 (Zurich sample). Infants presenting unilateral or bilateral hearing losses were first rehabilitated by hearing aids and consequently (>15 mo) by cochlear implants. Conclusions: Even though UNHS programs are well-established clinical practices in the European States, the amount of information in the literature about these programs is surprising low. The existing data in the timespan 2004–2024 corroborate the international UNHS data in terms of coverage and bilateral hearing loss prevalence, but there is a strong need to supplement the existing information with the latest developments, especially in the area of hearing loss rehabilitation. [ABSTRACT FROM AUTHOR]

Copyright of Istanbul Gelisim University Journal of Health Sciences / İstanbul Gelişim Üniversitesi Sağlık Bilimleri Dergisi is the property of Istanbul Gelisim Universitesi Saglik Bilimleri Yuksekokulu and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

The onset of Non Syndromic Hearing Loss (NSHL) typically occurs without any other symptoms and can vary from person to person, even within the same family. Hearing loss can be unilateral or bilateral and can range from mild to profound degrees of hearing loss. Epidermodysplasia Verruciformis (EDV) is a dermatologic condition in which patients show reduced immunologic ability to defend and eradicate certain types of Human Papillomavirus (HPV), leading to persistent infection and an increased lifetime risk of developing cutaneous dysplasia and malignancy. Both conditions have a genetic background. However, their concurrent occurrence is very rare. Therefore, a case study is presented of a four-year-old female child who visited the dermatology outpatient department with a chief complaint of hearing loss and white-coloured lesions covering her arms, neck, back, and chest for the last two years. The hearing loss was gradual, not apparent at birth, and progressed slowly to complete hearing loss. A final diagnosis of EDV with Non Syndromic Sensorineural Hearing Loss (NSSNHL) was made based on clinical and histological examination.

Introduction Cochlear implant (CI) activation usually takes place at ∼ 30 days postoperative (PO). In our service, CI surgery is performed with local anesthesia and sedation, so activation is possible with the patient's cooperation, immediately after the CI surgery, still in the operating room (OR). Objective The objective of the present study was to provide the patient with hearing experience with the CI and to assess auditory perception immediately after surgery while still in the OR, as well as to compare impedance telemetry (IT), neural response telemetry (NRT), and comfort (C) level at two moments: in the OR and at the definitive activation, ∼ 30 days PO. Methods Nine adult patients (12 ears) with acquired (postlingual) deafness were included. Auditory perception was evaluated through the Ling Six Sound Check, musical instruments, and clapping, presented in two different programming maps, elaborated using t-NRT, and comparing IT, NRT, and C level between the two moments. Results We observed that while still in the OR, the patient can already present auditory detection and recognition responses. The values of IT, NRT threshold (t-NRT), and C on both dates differed, with statistical significance. Conclusion We concluded that it is possible to provide the patient with an auditory experience with the CI immediately after surgery, and that the auditory experience and the values of electrode IT, NRT, and C vary significantly between the two moments. [ABSTRACT FROM AUTHOR]

Background: The reported data on European universal neonatal hearing screening (UNHS) practices tend to be scarce, despite the fact that the European Union project, EUScreen, collected unofficial data from 38 collaborating European institutions. The objectives of this systematic review were as follows: (a) to identify the most recent (in a 20-year span) literature information about UNHS programs in Europe and (b) to provide data on the procedures used to assess the population, the intervention policies, and on the estimated prevalence of congenital hearing loss with emphasis on the bilateral hearing loss cases. Methods: Queries were conducted via the Pubmed, Scopus and Google Scholar databases for the time period of 2004–2024. The Mesh terms used were “OAE”, “Universal Neonatal Hearing Screening”, “congenital hearing loss” and “well babies”. Only research articles and review papers of European origin were considered good candidates. The standard English language filter was not used, in order to identify information from non-English-speaking scientific communities and groups. Results: Very few data and reports were identified in the literature search. Eleven manuscripts were identified corresponding to eight UNHS programs. Except in Poland, most of the data refer to regional and not national programs. The screening coverage estimates of all programs exceed 90%; infants were mostly assessed by a three-stage protocol (TEOAE + TEOAE + AABR), followed by a clinical ABR test. The average prevalence (i.e., from well babies AND NICU infants) of bilateral hearing loss ranged from 0.5 to 20.94 per 1000 (Zurich sample). Infants presenting unilateral or bilateral hearing losses were first rehabilitated by hearing aids and consequently (>15 mo) by cochlear implants. Conclusions: Even though UNHS programs are well-established clinical practices in the European States, the amount of information in the literature about these programs is surprising low. The existing data in the timespan 2004–2024 corroborate the international UNHS data in terms of coverage and bilateral hearing loss prevalence, but there is a strong need to supplement the existing information with the latest developments, especially in the area of hearing loss rehabilitation.

Substance abuse is a rare but known cause of sensorineural hearing loss (SNHL). We report a case of acute SNHL in a 28-year-old man following an overdose of methamphetamine and incidental ingestion of fentanyl. On initial encounter, he had moderate-to-severe hearing loss in the right ear and severe-to-profound hearing loss in the left ear in addition to acute kidney injury, liver failure, and lactic acidosis. The patient was treated with a two-week course of high-dose steroids and expressed a subjective improvement in hearing. This case highlights the importance of auditory testing following a drug overdose and is one of the only documented cases of hearing loss following methamphetamine use in recent years. There is a paucity of literature regarding the mechanism causing acute SNHL secondary to methamphetamines. Proposed etiologies include neurotransmitter depletion or reduced cochlear blood flow as possible causes of ototoxicity. [ABSTRACT FROM AUTHOR]

The onset of Non Syndromic Hearing Loss (NSHL) typically occurs without any other symptoms and can vary from person to person, even within the same family. Hearing loss can be unilateral or bilateral and can range from mild to profound degrees of hearing loss. Epidermodysplasia Verruciformis (EDV) is a dermatologic condition in which patients show reduced immunologic ability to defend and eradicate certain types of Human Papillomavirus (HPV), leading to persistent infection and an increased lifetime risk of developing cutaneous dysplasia and malignancy. Both conditions have a genetic background. However, their concurrent occurrence is very rare. Therefore, a case study is presented of a four-year-old female child who visited the dermatology outpatient department with a chief complaint of hearing loss and white-coloured lesions covering her arms, neck, back, and chest for the last two years. The hearing loss was gradual, not apparent at birth, and progressed slowly to complete hearing loss. A final diagnosis of EDV with Non Syndromic Sensorineural Hearing Loss (NSSNHL) was made based on clinical and histological examination. [ABSTRACT FROM AUTHOR]

Lyme neuroborreliosis (LNB) is a rare but potentially serious manifestation of Lyme disease, caused by the spirochete Borrelia burgdorferi. Although LNB can affect various neurological systems, neurosensory hearing loss as the sole presentation is uncommon. We report a case of a 23-year-old woman who presented with a 2-month history of temporal headache, tinnitus, and instability, which was followed by sudden bilateral hearing loss without any other associated symptoms. Pure-tone audiometry revealed profound bilateral hearing loss. Serological testing for various pathogens was negative, except for B. burgdorferi IgM, which was confirmed using Western blot analysis. The patient received doxycycline treatment; unfortunately, no recovery of hearing was observed. This case report highlights the importance of considering LNB as a potential cause of neurosensory hearing loss, particularly in areas where Lyme disease is endemic, as well as the need for timely diagnosis and treatment to prevent potential complications. [ABSTRACT FROM AUTHOR]

In multiple sclerosis (MS), the occurrence of sudden sensorineural hearing loss (SSNHL) is considered rare, with reported cases predominantly being unilateral. Bilateral cases are even rarer. Here, we report a case of bilateral SSNHL in a 20-year-old male diagnosed with MS. The patient, undergoing corticosteroid therapy for the management of MS, additionally received an intratympanic dexamethasone injection; however, it could not achieve significant improvement. Subsequently, the systemic dosage was increased for one week, resulting in substantial hearing improvement in both ears after three months. A review of MS-related SSNHL cases from 1987 to 2022 revealed 39 ears in the literature, with only five ears showing no hearing recovery. A remarkable 87.2% exhibited restored hearing, presenting a more favorable prognosis compared with idiopathic SSNHL. Although there were slight variations in administration methods and duration, all documented treatment approaches involve systemic corticosteroids. In some instances, SSNHL manifested as the initial symptom of MS. When SSNHL occurs in MS, auditory brainstem response (ABR) tests may reveal prolonged abnormalities, making ABR testing effective in cases where MS is suspected following SSNHL. In conclusion, the treatment of MS-related SSNHL appears appropriate with systemic corticosteroids, showing a significantly superior prognosis compared with idiopathic SSNHL. [ABSTRACT FROM AUTHOR]

Introduction: In challenging listening situations, speech perception with a cochlear implant (CI) remains demanding and requires high levels of listening effort, which can lead to increased levels of listening-related fatigue. The body of literature on these topics increases as the number of CI users rises. This scoping review aims to provide an overview of the existing literature on listening effort, fatigue, and listening-related fatigue among CI users and the measurement techniques to evaluate them. Methods: The Preferred Reporting Items for Systematic Reviews and Meta- Analyses (PRISMA) Statements were used to conduct the scoping review. The search was performed on PubMed, Scopus, and Web of Science to identify all relevant studies. Results: In total, 24 studies were included and suggests that CI users experience higher levels of listening effort when compared to normal hearing controls using scales, questionnaires and electroencephalogram measurements. However, executing dual-task paradigms did not reveal any difference in listening effort between both groups. Uncertainty exists regarding the difference in listening effort between unilateral, bilateral, and bimodal CI users with bilateral hearing loss due to ambiguous results. Only five studies were eligible for the research on fatigue and listening-related fatigue. Additionally, studies using objective measurement methods were lacking. Discussion: This scoping review highlights the necessity for additional research on these topics. Moreover, there is a need for guidelines on how listening effort, fatigue, and listening-related fatigue should be measured to allow for study results that are comparable and support optimal rehabilitation strategies. [ABSTRACT FROM AUTHOR]

Copyright of HNO is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Primary mediastinal seminomas are exceedingly rare tumors, often localized to the anterior mediastinum. They may present with numerous complications, including superior vena cava syndrome, chylothorax, and pericardial effusions. Less commonly, they may present with paraneoplastic encephalitis. In this report we describe a case of a 19-year-old male with no significant past medical history who presented with bilateral hearing loss, progressive neuropathy, and ataxia. Subsequently the patient was found to have mediastinal mass with a high-titer anti-Hu antibody. To our knowledge, only one other case of mediastinal seminoma presenting with anti-Hu antibodies has been described in the literature. In this report, we describe a rare case of mediastinal seminoma, describe treatment options, and discuss additional known cases presenting with paraneoplastic encephalitis. [ABSTRACT FROM AUTHOR]

IntroductionIn challenging listening situations, speech perception with a cochlear implant (CI) remains demanding and requires high levels of listening effort, which can lead to increased levels of listening-related fatigue. The body of literature on these topics increases as the number of CI users rises. This scoping review aims to provide an overview of the existing literature on listening effort, fatigue, and listening-related fatigue among CI users and the measurement techniques to evaluate them.MethodsThe Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) Statements were used to conduct the scoping review. The search was performed on PubMed, Scopus, and Web of Science to identify all relevant studies.ResultsIn total, 24 studies were included and suggests that CI users experience higher levels of listening effort when compared to normal hearing controls using scales, questionnaires and electroencephalogram measurements. However, executing dual-task paradigms did not reveal any difference in listening effort between both groups. Uncertainty exists regarding the difference in listening effort between unilateral, bilateral, and bimodal CI users with bilateral hearing loss due to ambiguous results. Only five studies were eligible for the research on fatigue and listening-related fatigue. Additionally, studies using objective measurement methods were lacking.DiscussionThis scoping review highlights the necessity for additional research on these topics. Moreover, there is a need for guidelines on how listening effort, fatigue, and listening-related fatigue should be measured to allow for study results that are comparable and support optimal rehabilitation strategies.

Background and Objectives: The speech tests used to evaluate language performance in patients with bilateral deafness (BiD) and cochlear implant (CI) are problematic if applied to patients with single-sided deafness (SSD) because normal ear hearing should be excluded. Thus, we investigated the feasibility of using wireless connection to evaluate speech intelligibility of the CI ear in patients with SSD. Subjects and Methods: Patients with BiD and SSD were administered the word recognition scores (WRS) and speech intelligibility tests using an iPad-based wireless connection and conventional methods. To exclude normal side hearing in patients with SSD, masking noise and "plugged and muffed" method were used in the WRS and speech intelligibility tests, respectively. Results: In patients with BiD, the WRS and speech intelligibility tests results using wireless connection and conventional methods were similar. In patients with SSD, the WRS using masking noise in the normal hearing ear was similar to that of using wireless connection. However, 3 of 11 patients with SSD showed under-masked results if using the "plugged and muffed" method. Conclusions: Speech intelligibility testing using wireless connection is a convenient and reliable method for evaluating CI performance in patients with SSD. The "plugged and muffed" method is not recommended for evaluating CI performance in patients with SSD. [ABSTRACT FROM AUTHOR]

Through a cross-sectional community study of 2044 children aged 2 years, we (1) examine the impact of hearing loss on early spoken expressive vocabulary outcomes and (2) investigate how early intervention-related factors impact expressive vocabulary outcomes in children with hearing loss predominantly identified through universal newborn hearing screening. We used validated parent/caregiver-reported checklists from two longitudinal cohorts (302 children with unilateral or bilateral hearing loss, 1742 children without hearing loss) representing the same population in Victoria, Australia. The impact of hearing loss and amplification-related factors on vocabulary was estimated using g-computation and multivariable linear regression. Children with versus without hearing loss had poorer expressive vocabulary scores, with mean scores for bilateral loss 0.5 (mild loss) to 0.9 (profound loss) standard deviations lower and for unilateral loss marginally (0.1 to 0.3 standard deviations) lower. For children with hearing loss, early intervention and amplification by 3 months, rather than by 6 months or older, resulted in higher expressive vocabulary scores. Children with hearing loss demonstrated delayed spoken expressive vocabulary despite whole-state systems of early detection and intervention. Our findings align with calls to achieve a 1-2-3 month timeline for early hearing detection and intervention benchmarks for screening, identification, and intervention. [ABSTRACT FROM AUTHOR]

Primary mediastinal seminomas are exceedingly rare tumors, often localized to the anterior mediastinum. They may present with numerous complications, including superior vena cava syndrome, chylothorax, and pericardial effusions. Less commonly, they may present with paraneoplastic encephalitis. In this report we describe a case of a 19-year-old male with no significant past medical history who presented with bilateral hearing loss, progressive neuropathy, and ataxia. Subsequently the patient was found to have mediastinal mass with a high-titer anti-Hu antibody. To our knowledge, only one other case of mediastinal seminoma presenting with anti-Hu antibodies has been described in the literature. In this report, we describe a rare case of mediastinal seminoma, describe treatment options, and discuss additional known cases presenting with paraneoplastic encephalitis.

Case Presentation: A 53-year-old male presented to the emergency department with acute onset of bilateral hearing loss as well as vertigo and severe vomiting. The Head Impulse– Nystagmus–Test of Skew exam was indicative of a central neurologic process. Computed tomography angiogram of the head and neck revealed near-total bilateral vertebral artery occlusions in the second and third segments. The patient was admitted for further evaluation; subsequent magnetic resonance imaging revealed multiple areas of infarction in the cerebellar hemispheres, medulla, and occipital lobes.Discussion: This case describes a unique presentation of a posterior stroke. Common symptoms include vertigo, loss of balance, and vomiting. However, bilateral hearing loss as a prominent symptom is uncommon. Imaging revealed a rare finding of bilateral vertebral artery occlusion.

Objective:To reveal the correlation between implantation age, the internal acoustic canal (IAC) width, bony cochlear nerve canal (BCNC) width, and auditory performance in primary school children with bilateral cochlear implantation (CI).Methods:Preoperative IAC and BCNC widths of 57 pre-lingually deaf children aged 7–11 years who had previously undergone bilateral CI in our institution were reviewed and cut-off values were calculated. Twenty-four patients who had additional problems and could not attend school and those who refused to participate in the study were excluded. The remaining 33 were invited to the hospital, and their speech perception tests, and language development scores were analyzed (16 of 33 patients had been operated on before the age of 24 months).Results:The cut-off values calculated from the 114 ears of 57 patients were 3.86 mm for IAC width and 1.56 mm for BCNC width. The auditory performances of the 33 patients after CI were not significantly different in the narrow and normal width groups. However, speech perception test results, and language development scores of patients implanted before the age of 24 months were significantly higher.Conclusion:There are some studies showing that children with bilateral sensorineural hearing loss have narrower IAC and BCNC widths. However, we concluded that the widths of the IAC and the bone cochlear nerve canal did not affect auditory performance. We found that implantation age is the single most important determinant of speech-language development after CI.

Sensorineural hearing loss affects a significant portion of the global population, with its prevalence projected to rise sharply in the coming years. Most cases involve the degeneration of hair cells and spiral ganglion neurons within the inner ear, and current therapeutic options for hearing rehabilitation offer limited efficacy with variable outcomes among patients. This systematic review evaluates the existing evidence on stem cell therapy as an intervention for hearing loss, focusing on its impact on hearing restoration, quality of life, and safety. A thorough search of electronic databases and clinical trial registries identified randomized and quasi-randomized studies on this topic. Eight studies met the inclusion criteria, investigating various types of stem cells such as embryonic, umbilical cord, and inner ear cells administered intravenously or directly into the inner ear. Most studies used animal models to simulate hearing loss, while one was conducted in humans. Findings on hearing improvement were mixed, with some studies reporting significant improvements in hearing thresholds and others showing no effect. The safety of stem cell therapy was assessed in a single human study, which noted no significant adverse effects. While the results indicate potential therapeutic value, further human studies with standardized protocols and larger sample sizes are necessary to clarify the safety and effectiveness of stem cell therapy for sensorineural hearing loss., Competing Interests: Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2025, Henao Rincón et al.)

Introduction: The incidence of bilateral sensorineural hearing loss is quite notable in live births worldwide. Among the etiologies, the primary aetiology is thought to be genetic, followed by non heritable, and unknown. Aim: To study the effect of consanguinity on audiological profile in children presented with non syndromic sensorineural hearing loss. Materials And Methods: This cross-sectional study was conducted in the Department of Ear Nose Throat, McGANN Teaching District Hospital (Shimoga Institute of Medical Sciences), Shivamogga, Karnataka, India, from January 2019 to December 2020, among 95 children with non syndromic sensorineural hearing loss. A Pure Tone Audiometry (PTA) test was done for each patient both air conduction and bone conduction thresholds were tested using different transducers. An audiogram was collected and the degree of hearing loss of each patient was analysed. Statistical tests were performed using Statistical Package for Social Sciences (SPSS) version 34.0, Kruskal-Wallis test, one-way Analysis of Variance (ANOVA) was used for comparing the difference in PTA followed by the Mann-Whitney U test. Results: The mean age of the study population was 9.7 years; 39 subjects were female, and 56 were male. The PTA among the study population showed asymmetric audiograms in 17 (17.9%) children. A majority (60, 63%) of the participants were born out of consanguineous marriage. Total 80 (84.2%) children had bilateral hearing loss, among which 58 (72.5%) were cases of bilateral profound. One-way ANOVA showed that there was an overall significant difference between the consanguinity and non consanguinity groups and post-hoc analysis with the Mann-Whitney U test revealed a significant difference between the different degrees of consanguineous marriage. Conclusion: Consanguinity affects the audio profile (PTA) among the non syndromic hearing-impaired children. [ABSTRACT FROM AUTHOR]

Through a cross-sectional community study of 2044 children aged 2 years, we (1) examine the impact of hearing loss on early spoken expressive vocabulary outcomes and (2) investigate how early intervention-related factors impact expressive vocabulary outcomes in children with hearing loss predominantly identified through universal newborn hearing screening. We used validated parent/caregiver-reported checklists from two longitudinal cohorts (302 children with unilateral or bilateral hearing loss, 1742 children without hearing loss) representing the same population in Victoria, Australia. The impact of hearing loss and amplification-related factors on vocabulary was estimated using g-computation and multivariable linear regression. Children with versus without hearing loss had poorer expressive vocabulary scores, with mean scores for bilateral loss 0.5 (mild loss) to 0.9 (profound loss) standard deviations lower and for unilateral loss marginally (0.1 to 0.3 standard deviations) lower. For children with hearing loss, early intervention and amplification by 3 months, rather than by 6 months or older, resulted in higher expressive vocabulary scores. Children with hearing loss demonstrated delayed spoken expressive vocabulary despite whole-state systems of early detection and intervention. Our findings align with calls to achieve a 1-2-3 month timeline for early hearing detection and intervention benchmarks for screening, identification, and intervention.

This study aimed to evaluate the association between dry eye and inner ear diseases in a Korean population. Methods: Data from the Korean National Health and Nutrition Survey (KNHANES V, 2010–2012), a national cross-sectional health examination and survey, were collected by the Korea Centers for Disease Control and Prevention. The association between dry eye and inner ear disease was determined using the chi-square test and logistic regression analysis. The individuals were divided into two age groups (<60 and ≥60 years). Results: In total, 17,542 individuals (n = 11,932 in the <60 years group and n = 5610 in the ≥60 years group) were enrolled. After adjusting for confounding factors, the logistic regression model revealed that the associated factors were dizziness and loss of balance experience (OR, 1.315; 95% CI, 1.143–1.513), self-awareness of abnormal voice (OR, 1.372; 95% CI, 1.120–1.679), subjective hearing discomfort (OR, 1.278; CI, 1.084–1.506), and tinnitus (OR, 1.265; 95% CI, 1.101–1.453). The inversely associated factor for dry eye was bilateral hearing loss (OR, 0.497; 95% CI, 0.367–0.672). The hearing threshold was lower in the dry eye group than in the non-dry eye group (p < 0.05). Conclusions: Tinnitus was associated with dry eye and bilateral hearing loss was inversely associated with dry eye. These results suggest that hypersensitivity of the senses and nerves, which is neuropathic hyperesthesia, is one of the main mechanisms of dry eye. Treatment of neuropathy may help in treating dry eye associated with dizziness or tinnitus. [ABSTRACT FROM AUTHOR]

Objective: To reveal the correlation between implantation age, the internal acoustic canal (IAC) width, bony cochlear nerve canal (BCNC) width, and auditory performance in primary school children with bilateral cochlear implantation (CI). Methods: Preoperative IAC and BCNC widths of 57 pre-lingually deaf children aged 7-11 years who had previously undergone bilateral CI in our institution were reviewed and cut-off values were calculated. Twenty-four patients who had additional problems and could not attend school and those who refused to participate in the study were excluded. The remaining 33 were invited to the hospital, and their speech perception tests, and language development scores were analyzed (16 of 33 patients had been operated on before the age of 24 months). Results: The cut-off values calculated from the 114 ears of 57 patients were 3.86 mm for IAC width and 1.56 mm for BCNC width. The auditory performances of the 33 patients after CI were not significantly different in the narrow and normal width groups. However, speech perception test results, and language development scores of patients implanted before the age of 24 months were significantly higher. Conclusion: There are some studies showing that children with bilateral sensorineural hearing loss have narrower IAC and BCNC widths. However, we concluded that the widths of the IAC and the bone cochlear nerve canal did not affect auditory performance. We found that implantation age is the single most important determinant of speech-language development after CI. [ABSTRACT FROM AUTHOR]

Sudden-onset bilateral cortical deafness is a very rare symptom of stroke, but must be recognized as stroke, as it is a treatable condition, and the treatment is highly time dependent. We report a 53-year-old man with an acute onset of complete bilateral hearing loss that gradually improved spontaneously over 4 h. The hearing loss was explained by an infarction visualized on magnetic resonance imaging, which showed a subacute temporoparietal ischemic lesion in the left cerebral hemisphere involving the insular cortex and an older infarction in the right temporoparietal region. The location of these kinds of lesions may typically not cause motor deficits, but sensory and cognitive (e.g., aphasia) symptoms, which can be challenging to recognize in a suddenly deaf patient. Taking the possible differential diagnoses into account, immediate stroke workup should always be prioritized in patients with sudden bilateral deafness, as acute revascularizing treatment is possible.

The events following the emergence of SARS-CoV-2, in Wuhan, China, in December 2019, established COVID-19 as the pandemic airborne disease of the year 2019. The infection has been declared a global health emergency. In addition to the now already common symptoms, such as fever, fatigue and mild respiratory symptoms like dry cough, the patients with COVID-19 can also develop neurological manifestations, such as headache, anosmia, hyposmia, dysgeusia, meningitis, encephalitis and acute cerebrovascular incidents during the course of this disease. The first case of meningitis associated with COVID-19 was reported in Japan in February 2020. Since then, more cases have been described, each with their own particularities. Although very rare, the neurological manifestations are now part of a large scale of symptoms that SARS-CoV-2 infection may exhibit. Perhaps as rare as the neurological symptoms are the auditory manifestations of the same disease. In this article, we focused on presenting our experience with postCOVID-19 related meningitis and the subsequent vestibular and cochlear loss that followed. [ABSTRACT FROM AUTHOR]

Hearing with a cochlear implant (CI) is limited compared to natural hearing. Although CI users may develop compensatory strategies, it is currently unknown whether these extend from auditory to visual functions, and whether compensatory strategies vary between different CI user groups. To better understand the experience-dependent contributions to multisensory plasticity in audiovisual speech perception, the current event-related potential (ERP) study presented syllables in auditory, visual, and audiovisual conditions to CI users with unilateral or bilateral hearing loss, as well as to normal-hearing (NH) controls. Behavioural results revealed shorter audiovisual response times compared to unisensory conditions for all groups. Multisensory integration was confirmed by electrical neuroimaging, including topographic and ERP source analysis, showing a visual modulation of the auditory-cortex response at N1 and P2 latency. However, CI users with bilateral hearing loss showed a distinct pattern of N1 topography, indicating a stronger visual impact on auditory speech processing compared to CI users with unilateral hearing loss and NH listeners. Furthermore, both CI user groups showed a delayed auditory-cortex activation and an additional recruitment of the visual cortex, and a better lip-reading ability compared to NH listeners. In sum, these results extend previous findings by showing distinct multisensory processes not only between NH listeners and CI users in general, but even between CI users with unilateral and bilateral hearing loss. However, the comparably enhanced lip-reading ability and visual-cortex activation in both CI user groups suggest that these visual improvements are evident regardless of the hearing status of the contralateral ear.

Background: Few studies report prevalence rates of hearing loss in newborns for nationwide populations. The Dutch well-baby newborn hearing screening covers almost all eligible children and has high participation rates for follow-up screening rounds and diagnosis. This allows calculating reliable prevalence rates of permanent neonatal hearing loss specified by severity. Methods: Results from the well-baby newborn hearing screening program and diagnostic follow-up of referred children from 2015 to 2019 were included in calculating prevalence rates. Hearing loss was classified according to the degree of severity. Results: A total of 99.7% of 833,318 children eligible for screening were included. A total of 0.3% were referred for audiological diagnostics. Permanent bilateral hearing loss of ≥40 dB was diagnosed in 23.7% of them and unilateral hearing loss in 14.4%. A prevalence rate of 1.23 per 1000 children was found, 0.46 for unilateral hearing loss and 0.76 for bilateral hearing loss. Moderate hearing loss is most common in children with bilateral hearing loss (0.47), followed by profound (0.21) and severe (0.06) hearing loss. In children with unilateral hearing loss, prevalence rates are the highest for profound hearing loss (0.21), followed by moderate (0.16) and severe (0.09) hearing loss. A total of 87.5% of the children were diagnosed within the age of 3 months. Conclusions: Because of the high quality of the Dutch well-baby hearing screening program, reported results approximate true prevalence rates of permanent hearing loss by severity. [ABSTRACT FROM AUTHOR]

AIM: To evaluate and to analyze hearing impairment in auditory pathway in children with compromised hearing ability using brainstem evoked response audiometry. SUBJECTAND METHOD: Total 25 children between 1-5 years with compromised hearing ability were subjected to brainstem evoked response audiometry. Click stimuli were given having intensity of approximately 60db or above and the waveforms were recorded. They were labeled I to VII waves, of which I, III and V waves are the most visible and more significant waves. RESULT: Totally 25 children were taken for study. Out of them, 20 (80%) had bilateral hearing loss with no wave pattern and remaining five, out of them 3 children had bilateral hearing loss while 2 had unilateral hearing loss. Difficult labour was most commonly associated risk factor for hearing impairment. CONCLUSION: We believe that a larger sample and the follow up of these infants at risks by means of the study of BERA may offer valuable information on the state of maturation of the acoustic pathway achieved by the children

Hearing loss is a heterogeneous disorder thought to affect brain reorganization across the lifespan. Here, structural alterations of the brain due to hearing loss are assessed by using unique effect size metrics based on Cohen's d and Hedges’ g. These metrics are used to map coordinates of gray matter (GM) and white matter (WM) alterations from bilateral congenital and acquired hearing loss populations. A systematic review and meta-analysis revealed m = 72 studies with structural alterations measured with magnetic resonance imaging (MRI) (bilateral = 64, unilateral = 8). The bilateral studies categorized hearing loss into congenital and acquired cases (n = 7,445) and control cases (n = 2,924), containing 66,545 datapoint metrics. Hearing loss was found to affect GM and underlying WM in nearly every region of the brain. In congenital hearing loss, GM decreased most in the frontal lobe. Similarly, acquired hearing loss had a decrease in frontal lobe GM, albeit the insula was most decreased. In congenital, WM underlying the frontal lobe GM was most decreased. In congenital, the right hemisphere was more negatively impacted than the left hemisphere; however, in acquired, this was the opposite. The WM alterations most frequently underlined GM alterations in congenital hearing loss, while acquired hearing loss studies did not frequently assess the WM metric. Future studies should use the endophenotype of hearing loss as a prognostic template for discerning clinical outcomes.

Abstract Objective Hearing loss may be associated with certain sleep abnormalities. We recently reported that subclinical hearing loss (SHL) was more prevalent in individuals in a broad Japanese population who slept longer than 8 h; however, the underlying mechanism was unknown. Therefore, we investigated the association between SHL and self-reported restorative sleep (RS), assessed by questionnaire, in a database of 33,888 Japanese aged 40–69 years without overt or diagnosed hearing loss (20,225 men, 13,663 women). Results The proportion of individuals with RS (more than half of the subjects) was significantly higher in the group with bilateral than with unilateral SHL at 4000 Hz and intact hearing; however, that was not the case at 1000 Hz, independent of age (P

Objective: To conduct a medical audit of bilateral simultaneous cochlear implantation (CI) in patients with severe prelingual sensorineural hearing loss (SNHL).Methods: A medical audit of a tertiary care ear, nose, and throat center in Southern India was conducted on data collected from January 2007 to December 2014. All cochlear implantees 6 years, sequential bilateral CI, revision cases, abnormal or malformed cochlea, and children with global developmental delay in milestones. Subjective outcome scores used were Category of Auditory Performance (CAP) and Speech Intelligibility Rating (SIR).Results: The CAP and SIR results showed that 20% of implantees achieved peak scores of 7 and 5, respectively. Mean CAP and SIR scores at 12 months were 5.4 and 3.1, respectively.Conclusion: The present study supports the claim that bilateral CI in severe prelingual bilateral SNHL is better than unilateral and recommends that bilateral CI should be the standard of care in children.

Sudden-onset bilateral cortical deafness is a very rare symptom of stroke, but must be recognized as stroke, as it is a treatable condition, and the treatment is highly time dependent. We report a 53-year-old man with an acute onset of complete bilateral hearing loss that gradually improved spontaneously over 4 h. The hearing loss was explained by an infarction visualized on magnetic resonance imaging, which showed a subacute temporoparietal ischemic lesion in the left cerebral hemisphere involving the insular cortex and an older infarction in the right temporoparietal region. The location of these kinds of lesions may typically not cause motor deficits, but sensory and cognitive (e.g., aphasia) symptoms, which can be challenging to recognize in a suddenly deaf patient. Taking the possible differential diagnoses into account, immediate stroke workup should always be prioritized in patients with sudden bilateral deafness, as acute revascularizing treatment is possible. [ABSTRACT FROM AUTHOR]

Background: Few studies report prevalence rates of hearing loss in newborns for nationwide populations. The Dutch well-baby newborn hearing screening covers almost all eligible children and has high participation rates for follow-up screening rounds and diagnosis. This allows calculating reliable prevalence rates of permanent neonatal hearing loss specified by severity. Methods: Results from the well-baby newborn hearing screening program and diagnostic follow-up of referred children from 2015 to 2019 were included in calculating prevalence rates. Hearing loss was classified according to the degree of severity. Results: A total of 99.7% of 833,318 children eligible for screening were included. A total of 0.3% were referred for audiological diagnostics. Permanent bilateral hearing loss of ≥40 dB was diagnosed in 23.7% of them and unilateral hearing loss in 14.4%. A prevalence rate of 1.23 per 1000 children was found, 0.46 for unilateral hearing loss and 0.76 for bilateral hearing loss. Moderate hearing loss is most common in children with bilateral hearing loss (0.47), followed by profound (0.21) and severe (0.06) hearing loss. In children with unilateral hearing loss, prevalence rates are the highest for profound hearing loss (0.21), followed by moderate (0.16) and severe (0.09) hearing loss. A total of 87.5% of the children were diagnosed within the age of 3 months. Conclusions: Because of the high quality of the Dutch well-baby hearing screening program, reported results approximate true prevalence rates of permanent hearing loss by severity.

Background: Bilateral sudden sensorineural hearing loss (BSSHL) is rare and assumed to be a different clinical entity compared to unilateral SSHL (USSHL). This study examined the differences between the idiopathic BSSHL and USSHL. Methods: Forty-six sequential BSSHL patients (Se-BSSHL) and 68 simultaneous BSSHL (Si-BSSHL) were consecutively admitted between June 2008 and December 2015. Two sets of patients served as control groups: (1) USSHL patients with healthy contralateral ear and (2) USSHL patients with contralateral preexisting hearing loss (USSHLwCHL). We retrospectively analyzed differences among four cohorts using analysis of variance, Kruskal-Wallis test, Welch's t-test, and Chi-square test as appropriate before and after propensity score matching (PSM) based on age, gender, and body mass index (BMI). Results: The prevalence of idiopathic BSSHL was 8.6% (114/1329) among the total SSHL patients. In the total cohort, USSHL patients tended to be younger, female, and tended to have lower BMI, renal parameters, and total cholesterol in addition to higher high-density lipoprotein compared to the other three groups. Most routine blood indicators, some coagulation markers, and immunoglobulin M (H = 13.4, P = 0.004) were significantly different among the study groups. After PSM, the major significant differences were found in audiometric characteristics. Si-BSSHL and Se-BSSHL patients demonstrated similar hearing thresholds as USSHL but were significantly better than the USSHLwCHL patients across most frequencies before and after treatment (H = 30.0, P < 0.001 for initial hearing and H = 12.0, P = 0.007 for final hearing). Moreover, the BSSHL patients showed different hearing loss distribution patterns (more descending type, χ2 = 33.8, P = 0.001) with less hearing gain (H = 17.5, P < 0.001) compared to the USSHL patients. Conclusions: Idiopathic BSSHL is a relatively rare subtype of SSHL with a higher rate of descending audiogram type and inferior hearing outcome rather than being classified as a completely different disease entity compared to USSHL.

Thornwaldt cyst is a rare cystic formation, located along the midline of the nasopharynx. We present the case of a 60-year-old man with impaired nasal breathing and a several months-long history of serous otitis media. His only concomitant disease was arterial hypertension. The diagnostic imaging tests revealed a well-rounded cystic formation involving the upper part of the nasopharynx, characteristic of Thornwaldt cyst. Following, endoscopic transnasal marsupialization was performed and the benign cystic nature was confirmed on histopathology. The patient responded to the administered treatment and reported no persistence or emergence of new symptoms. The current case presents a symptomatic Thornwaldt cyst successfully treated by endoscopic transnasal marsupialization., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2024, Konsulov et al.)

Background and Aim: It is well known that hearing aid fitting is an effective approach to improve the communication ability of hearingimpaired people. In the past, most of the hearing aids were fitted unilaterally rather than bilaterally. Whereas the unilateral hearing aid fitting improves verbal communication partially, it causes late-onset auditory deprivation. The main aim of this study is to investigate the ANL for each ear among the users with unilateral hearing aid experience. Methods: A total of 23 participants were recruited (14 females, 9 males). The mean age was 74.65 years (ranged from 41 to 83). All subjects had bilateral symmetric sensorineural hearing loss. The most comfortable level (MCL), Background Noise Level (BNL), and acceptable noise level (ANL) were measured for ear with amplification experience and ear without experience. Results: MCL, BNL and ANL in the aided ear was 82.22, 73.48 and 8.74 respectively, in addition in the unaided ear the results for MCL, BNL and ANL was 81.78, 72.13 and 9.65 respectively. Comparing the mean values of MCL, BNL and ANL between two ears showed no significant difference. Conclusion: There was not any difference for BNL and ANL measures [ABSTRACT FROM AUTHOR]

The aim of the study was to clarify differences in the prevalence and features of bony malformations in inner ear between congenital unilateral sensorineural hearing loss (USNHL) and congenital bilateral sensorineural hearing loss (BSNHL). We conducted a retrospective study of 378 consecutive infants referred from routine newborn hearing screening in the past 18 years. Clinical background, audiological data, and temporal bone computed tomography (CT) findings were analyzed. The prevalence of malformations between USNHL and BSNHL groups were compared using the Chi-square test. The proportion of family history of hearing loss was significantly higher in infants with BSNHL than in those with USNHL (26/107 [24.3%] vs. 4/105 [3.7%]; p = 0.0001). Temporal bone CT scanning revealed significantly a higher prevalence of inner ear malformations in infants with USNHL than in those with BSNHL (93/109 [85.3%] vs. 4/107 [3.7%]; p < 0.0001). The most frequent anomaly in USNHL was cochlear nerve canal stenosis (69.7%), followed by cochlear malformations (20.2%), and narrow internal auditory canal (17.4%). Four infants with BSNHL accompanied by inner ear anomaly had complications such as Down's syndrome, developmental delay, or epilepsy. The prevalence of bony malformations in inner ear and/or IAC was markedly higher in infants with congenital USNHL than in infants with BSNHL. Temporal bone CT scanning may help to clarify the etiology of congenital hearing loss, especially in USNHL. [ABSTRACT FROM AUTHOR]

This study aims to evaluate the etiology of pediatric sensorineural hearing loss (SNHL). A total of 423 children with SNHL were evaluated, with the focus on the determination of causative genetic and acquired etiologies of uni- and bilateral SNHL in relation to age at diagnosis and severity of the hearing loss. We found that a stepwise diagnostic approach comprising of imaging, genetic, and/or pediatric evaluation identified a cause for SNHL in 67% of the children. The most common causative finding in children with bilateral SNHL was causative gene variants (26%), and in children with unilateral SNHL, a structural anomaly of the temporal bone (27%). The probability of finding an etiologic diagnosis is significantly higher in children under the age of 1 year and children with profound SNHL.Conclusions: With our stepwise diagnostic approach, we found a diagnostic yield of 67%. Bilateral SNHL often has a genetic cause, whereas in unilateral SNHL structural abnormalities of the labyrinth are the dominant etiologic factor. The diagnostic yield is associated with the age at detection and severity of hearing loss: the highest proportion of causative abnormalities is found in children with a young age at detection or a profound hearing loss. What is Known: • Congenital sensorineural hearing loss is one of the most common congenital disorders • Determination of the cause is important for adequate management and prognosis and may include radiology, serology, and DNA analysis What is New: • Using a stepwise diagnostic approach, causative abnormalities are found in 67% both in uni- and bilateral SNHL, with the highest diagnostic yield in very young children and those suffering from profound hearing loss • Bilateral SNHL often has a genetic cause, whereas in unilateral SNHL structural abnormalities of the labyrinth are the dominant etiologic factor. [ABSTRACT FROM AUTHOR]

Objectives: The objective was to describe a unique case of sudden bilateral hearing loss, tinnitus, and vertigo presenting as the first symptoms of chronic myeloid leukemia (CML). Methods: A patient case is discussed along with a review of associated literature. Results: A patient presented with sudden onset hearing loss, tinnitus, and vertigo, and was diagnosed with CML with an initial white blood cell (WBC) count of 555 000 per microliter. The proposed etiology of otologic symptoms is hyperleukocytosis. Serial audiograms showed minimal improvement in hearing despite reduction of WBC. Conclusion: In cases of bilateral, sudden onset sensorineural hearing loss, evaluation for systemic causes of otologic symptoms, including CML, should be considered. [ABSTRACT FROM AUTHOR]

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