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Introduction: Recent studies have reported poor cognition, such as attention and working memory, in adults with listening difficulties (LiD). However, they do not adequately describe the actual state of poor attention ability in adults with LiD. We examined the state of auditory attention in adults with and without LiD in tasks requiring multiple attention controls. Methods: Twenty-one adults who had normal hearing but complained about LiD encountered during everyday life and 22 healthy controls were included. We presented a target detection task using an odd-ball format for one ear and a sentence repetition task for the other ear. In the target detection task, participants listened to the 1,000-Hz tone served as the standard stimulus, while they had to accept a 2,000-Hz tone presented as the deviant stimulus. In the sentence repetition task, short sentences were presented. The stimuli presented to them were played on a personal computer at the most comfortable level. The participants heard these stimuli through headphones. They were required to press a key for standard stimuli in the target detection task and repeat what they heard immediately in the repetition task. We compared the response accuracy for each ear task between adults with and without LiD. Results: Our results showed that there were significant differences between the participant groups in the auditory dual-task under the dichotic listening situation. When examined individually, four adults with LiD had decreased scores in both the sentence repetition and target detection task, while the other nine participants showed a bias toward either task. Furthermore, the analysis of reaction time for pressing button revealed that the standard deviation of reaction time was extended in participants who scored poorly in either of the ear tasks. On the other hand, all adults without LiD were able to conduct the auditory dual-task exactly and promptly. Conclusion: The results suggest that adults with LiD have difficulties in appropriately allocating various cognitive abilities required for each task. We concluded that auditory attention is an important ability to conduct the auditory dual-task, and this is applicable for adults with LiD. Therefore, we believe that it is necessary to use auditory tests that require complex attentional abilities in listening, such as those required in daily life, to assess adults with LiD.

There is no report on acute sensorineural hearing loss with congenital cytomegalovirus (cCMV) infection in basic experiments.The aim of this study was to evaluate the effect of dexamethasone, an anti-inflammatory steroid, on acute sensorineural hearing loss in the mouse cytomegalovirus (MCMV) infection model mice.Sensorineural hearing loss model mice were divided into two groups, one with and one without intratympanic dexamethasone. Dexamethasone was injected into the tympanic cavity of only the right ear, and hearing ability was assessed at the ages of three, six, and eight weeks by auditory brainstem response measurement.Among the 23 mice intratympanically injected with dexamethasone (15 μg/mouse) at the age of three weeks, five (21.7%) had a hearing improvement of at least 10 dB and 18 (78.3%) had no improvement at the age of six weeks. Among the 19 mice that did not receive a dexamethasone injection, one (5.3%) showed improvement and 18 (94.7%) showed no improvement (In this study, transtympanic infusion of dexamethasone into the tympanic cavity was effective in some mice with sensorineural hearing loss, suggesting that, in addition to angiogenesis, anti-inflammatory activity might be a mechanism of treatment for hearing loss.

Objective To study the pathophysiology of Charcot-Marie-Tooth disease (CMT) with auditory neuropathy (AN) and to follow up cochlear implant or hearing aid use over the long term. Study design Clinical capsule report. Patients Two adult CMT patients with AN. Intervention Cochlear implantation for case 1 and hearing aid use for case 2. Result Case 1 was a 50-year-old man who was diagnosed as having CMT at 15 years of age. He noted his hearing impairment at the age of 22. We considered that he had AN as a complication on the basis of the findings of normal distortion product otoacoustic emission and the absence of auditory brainstem response on both ears. We performed cochlear implantation for his progressive hearing loss when he was 41 years old. His postoperative discrimination scores for words and sentences in the CI-2004 test 4 years after cochlear implantation were 80% and 93%, respectively. His sound discrimination scores were 23/24 in the test with picture matching and 9/24 in that without picture matching 8 years after cochlear implantation.Case 2 was a 38-year-old woman who was neurologically diagnosed as having CMT with AN as a complication on the basis of auditory brainstem response and distortion product otoacoustic emission test findings. During 15 years of follow-up, her discrimination score decreased gradually. She is currently wearing a hearing aid. Conclusions We found a significant improvement in case 1 and a slight increase in hearing level in case 2. The pathoneurological findings of CMT might be either demyelination or axonal degeneration depending on the type of genetic abnormality (e.g., PMP22 and MPZ mutations). The symptoms and courses vary among patients with CMT. Therefore, the selection of an appropriate intervention for hearing loss depending on the severity and course is very important.

In central auditory disorders caused by damage of the cerebral hemispheres, there are cortical deafness and auditory agnosia. Although clinical cases of cortical deafness have been reported, little is known about the hearing problems and localized lesions associated with cortical deafness.The aims of our research are to elucidate lesion sites associated with cortical deafness and to clarify why patients with cerebral lesions are not aware of any sound at all.Three patients diagnosed as having total loss of hearing participated in this study. We conducted pure-tone audiometry, speech audiometry, distortion product otoacoustic emission (DPOAE), auditory brainstem response (ABR), and brain magnetic resonance imaging (MRI) to diagnose cortical deafness with aphasia tests of these patients.Our studies showed that waves VI and VII as well as waves I to V have normal peak latencies in ABRs in all three patients. In brain MRI, we found complete damage of proximal parts of bilateral auditory radiations in the three patients.We propose 'subcortical deafness' as a subtype of auditory agnosia.

Purpose In areas of Africa where ivermectin is widely used as prophylaxis against neglected tropical diseases, COVID-19 morbidity and mortality are markedly lower. Thus, prophylactic administration of ivermectin is expected to be as effective as vaccine administration. This study will evaluate the effect of ivermectin on Delta variant by retrospective comparative epidemiology with vaccines and estimate its mechanism of action. Methods We used the 4-month observation period during which Delta variant was prevalent. Vaccine-using countries were divided into two groups according to the presence or absence of an inactivated vaccine approval. The number of confirmed cases, deaths, and recoveries in the ivermectin-using countries and countries with full vaccination coverage of 50% or higher were corrected using adjustment factors calculated from population factors, medical care, and test systems. The median test performed statistical comparisons by adjusted values between the ivermectin group and each vaccine group. Vaccine efficacy rates against ivermectin were calculated based on odds ratios. Spearman's rank correlation coefficient examined the interaction between ivermectin and each vaccine. Results Ivermectin was statistically less effective than the non-inactivated vaccines but was not statistically significantly different from the inactivated vaccine-approved group for all items. Vaccine efficacy against ivermectin was 35% for the non-inactivated vaccines and 13% for the inactivated vaccine-approved group. The interaction between ivermectin and the non-inactivated vaccines may reduce morbidity, while the inactivated vaccines may increase morbidity and mortality. Discussion Ivermectin may have both chemical actions and immune response mechanisms against SARS-CoV-2. Ivermectin is less effective than the non-inactivated vaccine but is more effective than the inactivated one. When used in combination with a non-activated vaccine, it may further reduce morbidity. The vaccine effect of ivermectin may last approximately 5 months. The vaccine-like effects of ivermectin may activate the retinoic acid-induced gene-I pathway, leading to antibody production and autophagy.

Clinical application of electrically-evoked intracochlear auditory brainstem responses (eABRs) for evaluation of brainstem maturity or aging changes has not been well investigated.We compare the eV latencies of intraoperative eABR measurements in one-year-olds, infants, children, adults, and the elderly, with the goal of investigating the changes in the brainstem auditory pathway due to development and aging.We studied 58 ears of 51 patients who underwent cochlear implantation between 2013 and 2019 using MED-EL's Concerto or Synchrony implants with Flex28 or Flex soft electrodes. EABRs were recorded during cochlear implantation. The stimuli were delivered by the MED-EL Maestro to the apical, middle, and basal turn electrodes at stimulus levels 1000, 800, and 600 cu, with a pulse width of 30 µs.In eABRs recorded from electrodes installed at both the mastoid and nape, there was no difference in latency between age groups within each stimulus level.ABR latency was not affected by development after age one and aging of the brainstem auditory pathway. Our study will be useful as a control in identifying abnormal eABR wave configurations in patients with cochlear malformations, cochlear nerve deficiencies, or auditory neuropathy, regardless of age.

To examine the effect of vestibular ocular reflex on gross motor development in children with semicircular canal aplasia and hypoplasia.Case series.Children with congenital hearing loss underwent temporal bone computed tomography to assess their inner ear morphology. In this study, we examined eight children with bilateral complete aplasia of semicircular canals and seven children with bilateral hypoplasia of semicircular canals. Their vestibular ocular reflex was assessed using a damped rotational chair test. Their gross motor development was assessed by examining their ages of acquisition of head control and independent walking.All eight children with semicircular canal aplasia were diagnosed as having CHARGE syndrome and five children were found to have CHD7 mutations. Cochlear morphological examination showed that cochlear hypoplasia type III was the most common in eight of 16 ears in children with semicircular canal aplasia. In semicircular canal hypoplasia, incomplete partition type II was found in seven of 14 ears. All eight children with semicircular canal aplasia showed no response in the damped rotational chair test. Even the three children followed up were unresponsive in all tests. In the group with semicircular canal hypoplasia, three children showed normal responses, one child showed no response, and three children showed poor responses. The mean age of acquisition of head control was 9.1 ± 6.3 months and that of independent walking was 31 ± 8.1 months in children with semicircular canal aplasia. The mean age of acquisition of head control was 6.0 ± 2.8 months and that of independent walking was 19.9 ± 4.9 months in children with semicircular canal hypoplasia.We found that children with semicircular canal aplasia have severe dysfunction of the vestibular ocular reflex, and vestibular sensory cells derived from the semicircular canal may not be present. Semicircular canal hypoplasia is associated with dysfunction of the vestibular ocular reflex, but vestibular sensory cells may be present, and rotary nystagmus develops with growth similar to that observed in normal children previously reported. Semicircular canal aplasia is associated with CHARGE syndrome, and affected children tended to have a slower gross motor development. It was suggested that the combination of visual impairment and mental retardation may delay the development of central-level integrated functions and may further delay motor development.

It remains unclear to what extent newborn hearing screening (NHS) detects congenital cytomegalovirus (cCMV)-associated sensorineural hearing loss (SNHL) in Japan. This study aimed to clarify the NHS results and audiological characteristics of patients with cCMV-associated SNHL. A total of 541 individuals with unilateral or bilateral hearing loss of unknown etiology were examined for cCMV infection. cCMV infection was defined by the presence of CMV DNA in the dried umbilical cord detected using real-time quantitative PCR. NHS results and audiological data were retrospectively obtained from medical records. Forty-four cases (8.1%) were positive for cCMV infection. Of them, 33 cases underwent NHS and 13 cases (39.4%) passed NHS bilaterally. The pure-tone audiograms of 21 patients were obtained. There were seven cases of unilateral SNHL, five cases of asymmetric bilateral SNHL, and nine cases of symmetric bilateral SNHL. cCMV-related hearing loss is highly heterogeneous, and there is a high risk of missing this condition through NHS.

Background: The electrically evoked auditory brainstem response (eABR) during cochlear implantation in common cavity (CC) deformity has not been clinically well studied.Aim/Objective: To investigate the eABR wave configuration during cochlear implantation, the postoperative development of hearing, and educational settings in infants with CC deformity.Materials and Methods: Nine infants who were congenitally deaf and found to have CC deformity and suspected cochlear nerve deficiency by temporal bone CT and MRI were studied. All of them underwent sequential and unilateral cochlear implantation and their eABRs were recorded during the surgery. The postoperative development was followed up.Results: eABRs of all the nine infants with CC deformity showed that the mean peak of the evoked wave V (eV) was the same as that in controls at latencies but was twofold higher than that in controls at thresholds and their postoperative hearing thresholds improved markedly.Conclusion and Significance: The brainstem auditory pathways of the infants were considered intact on the basis of the eV peak latencies in the eABRs. The postoperative development of their speech and hearing abilities and their educational settings varied, probably because of the twofold higher thresholds of their eABRs and limited number of auditory neural units.

Purpose: There are many requirements to assess and support individuals with auditory processing disorder (APD); however, there are a few questionnaires that can be employed to detect the listening ...

The purpose of this study is to profile the clinical and genetic features of Japanese Waardenburg syndrome (WS) patients and validate the W index. Sixteen Japanese WS families with congenital sensorineural hearing loss were included in the study. The inner canthal, interpupillary, and outer canthal distances (ICD, IPD, and OCD) were measured for all patients, and patients were screened for presence of PAX3, MITF, SOX10, and EDNRB mutations. The WS patients were clinically classified under the current W index as follows: 13 families with WS1, 2 families with WS2, and 1 family with WS4. In the 13 WS1 families, genetic tests found PAX3 mutations in 5 families, MITF mutations in 4 families, SOX10 mutations in 3 families, and EDNRB mutations in 1 family. 61% of clinically classified WS1 patients under the current W index conflicted with the genetic classification, which implies W index is not appropriate for Japanese population. Resetting the threshold of W index or novel index formulated with ethnicity matched samples is necessary for clinical classification which is consistent with genetic classification for WS patients with distinct ethnicity.

Objectives Auditory neuropathy (AN) is a clinical disorder characterized by the absence of auditory brainstem response and presence of otoacoustic emissions. A gradual loss of otoacoustic emissions has been reported for some cases of AN. Such cases could be diagnosed as cochlear hearing loss and lead to misunderstanding of the pathology when patients first visit clinics after the loss of otoacoustic emissions. The purpose of this study was to investigate the time course of changes in distortion product otoacoustic emissions (DPOAEs) in association with patients' genetic and clinical backgrounds, including the use of hearing aids. Design DPOAE measurements from 31 patients with AN were assessed. Genetic analyses for GJB2, OTOF, and mitochondrial m.1555A> G and m.3243A> G mutations were conducted for all cases, and the analyses for CDH23 and OPA1 were conducted for the selected cases. Patients who were younger than 10 years of age at the time of AN diagnosis were designated as the pediatric AN group (22 cases), and those who were 18 years of age or older were designated as the adult AN group (9 cases). DPOAE was measured at least twice in all patients. The response rate for DPOAEs was defined and analyzed. Results The pediatric AN group comprised 10 patients with OTOF mutations, 1 with GJB2 mutations, 1 with OPA1 mutation, and 10 with indefinite causes. Twelve ears (27%) showed no change in DPOAE, 20 ears (46%) showed a decrease in DPOAE, and 12 ears (27%) lost DPOAE. Loss of DPOAE occurred in one ear (2%) at 0 years of age and four ears (9%) at 1 year of age. The time courses of DPOAEs in patients with OTOF mutations were divided into those with early loss and those with no change, indicating that the mechanism for deterioration of DPOAEs includes not only the OTOF mutations but also other common modifier factors. Most, but not all, AN patients who used hearing aids showed deterioration of DPOAEs after the start of using hearing aids. A few AN patients also showed deterioration of DPOAEs before using hearing aids. The adult AN group comprised 2 patients with OPA1 mutations, 2 with OTOF mutations, and 5 with indefinite causes. Four ears (22%) showed no change in DPOAE, 13 ears (72%) showed a decrease, and one ear (6%) showed a loss of DPOAE. Although the ratio of DPOAE decrease was higher in the adult AN group than in the pediatric AN group, the ratio of DPOAE loss was lower in the adult AN group. DPOAE was not lost in all four ears with OPA1 mutations and in all four ears with OTOF mutations in the adult group. Conclusions DPOAE was decreased or lost in approximately 70% of pediatric and about 80% of adult AN patients. Eleven percent of pediatric AN patients lost DPOAEs by 1 year of age. Genetic factors were thought to have influenced the time course of DPOAEs in the pediatric AN group. In most adult AN patients, DPOAE was rarely lost regardless of the genetic cause.

BackgroundScientists have so far been unable to determine the reason for the low number of COVID-19 cases in Africa.ObjectiveTo evaluate the impact of ivermectin interventions for onchocerciasis on the morbidity, mortality, recovery, and fatality rates caused by COVID-19.MethodA retrospective statistical analysis study of the impact of ivermectin against COVID-19 between the 31 onchocerciasis-endemic countries using the community-directed treatment with ivermectin (CDTI) and the non-endemic 22 countries in Africa. The morbidity, mortality, recovery rate, and fatality rate caused by COVID-19 were calculated from the WHO situation report in Africa. We investigated the onchocerciasis endemic 31 countries and the non-endemic 22 countries. Statistical comparisons used by the Welch test of them in the two groups were made.ResultsThe morbidity and mortality were statistically significantly less in the 31 countries using CDTI. The recovery and fatality rates were not statistically significant difference. The average life expectancy was statistically significantly higher in the non-endemic countries.ConclusionsThe morbidity and mortality in the onchocerciasis endemic countries are lesser than those in the non-endemic ones. The community-directed onchocerciasis treatment with ivermectin is the most reasonable explanation for the decrease in morbidity and fatality rate in Africa. In areas where ivermectin is distributed to and used by the entire population, it leads to a significant reduction in mortality.

Background Hearing loss needs to be diagnosed and treated early, especially in older individuals, since presbycusis potentially increases the risk of depression and dementia. However, standard data on hearing thresholds across the life-span in Japanese individuals are lacking. Methods In a retrospective consecutive sample of 10681 native-Japanese speakers (37.3% men; 10–99 years; left-right hearing threshold difference of 1000 Hz) were significantly worse in men than in women. For participants ≥70 years, hearing thresholds at low frequencies were higher in women. Hearing thresholds at 1000, 2000, and 4000 Hz tended to deteriorate, starting in the teenage years through the 50s, with some decades showing significantly worse decline. Sex differences were absent in the youngest and oldest groups. Interpretation Standard age- and sex-specific audiometric data reported here for Japanese individuals over nine age-decades are based on the largest dataset analyzed to date. While hearing thresholds of men and women in the very young and the very old age groups were indistinguishable in their cohorts, patterns of hearing changes for other age cohorts differed by direction and sex. Funding The authors had no outside funding for this study.

Pelizaeus-Merzbacher disease (PMD) is classified into six types. Patients with type II PMD show congenital nystagmus, hypotonia, rigidity of the trunk and extremities, and mental and speech retardation as the main clinical symptoms. Brain MRIs show a high signal intensity in the white matter and a low signal intensity in the gray matter of the cerebrum. ABR tracings commonly show only waves I and II.

We herein present a patient with auditory agnosia that may provide insight into this condition.

There are several types of brain anomalies. It is an important issue with respect to auditory function whether children with brain anomaly but with a preserved brainstem and thalamus are able to hear. If they can hear certain sounds how can this pathophysiology be understood?