Frequent Sensory Deficit Research Articles

Mutation spectrum of hearing loss patients in Northwest China: Identification of 20 novel variants.

Hearing loss (HL) is the most frequent sensory deficit in humans, with strong genetic heterogeneity. The genetic diagnosis of HL is very important to aid treatment decisions and to provide prognostic information and genetic counselling for the patient's family. We detected and analysed 362 Chinese non-syndromic HL patients by screening of variants in 15 hot spot mutations. Subsequently, 40 patients underwent further whole-exome sequencing (WES) to determine genetic aetiology. The candidate variants were verified using Sanger sequencing. Twenty-three carrier couples with pathogenic variants or likely pathogenic variants chose to proceed with prenatal diagnosis using Sanger sequencing. Among the 362 HL patients, 102 were assigned a molecular diagnosis with 52 different variants in 22 deafness genes. A total of 41 (11.33%) cases with the biallelic GJB2 (OMIM # 220290) gene mutations were detected, and 21 (5.80%) had biallelic SLC26A4 (OMIM # 605646) mutations. Mitochondrial gene (OMIM # 561000) mutations were detected in seven (1.93%) patients. Twenty of the variants in 15 deafness genes were novel. SOX10 (OMIM # 602229), MYO15A (OMIM # 602666) and WFS1 (OMIM # 606201) were each detected in two patients. Meanwhile, OSBPL2 (OMIM # 606731), RRM2B (OMIM # 604712), OTOG (OMIM # 604487), STRC (OMIM # 606440), PCDH15 (OMIM # 605514), LOXHD1 (OMIM # 613072), CDH23 (OMIM # 605516), TMC1 (OMIM # 606706), CHD7 (OMIM # 608892), DIAPH3 (OMIM # 614567), TBC1D24 (OMIM # 613577), TIMM8A (OMIM # 300356), PTPRQ (OMIM # 603317), SALL1 (OMIM # 602218), and GSDME (OMIM # 608798) were each detected in one patient. In addition, as regards one couple with a heterozygous variant of CDH23 and PCDH15, respectively, prenatal diagnosis results suggest that the foetus had double heterozygous (DH) variants of CDH23 and PCDH15, which has a high risk to cause ID/F type Usher syndrome. Our study expanded the spectrum of deafness gene variation, which will contribute to the genetic diagnosis, prenatal diagnosis and the procreation guidance of deaf couple. In addition, the deafness caused by two genes should be paid attention to in the prenatal diagnosis of families with both deaf patients.

Assessment of Auditory Brainstem Response in Infants Suffering from Congenital Heart Disease

Background: Congenital heart defects are the singular most common congenital anomalies and account for a significant fraction of childhood mortality and morbidity. Hearing impairment was the most frequent sensory deficit. Auditory brainstem response (ABR) was determined to assess the maturation and function of brainstem, and hence, hearing in infants. Therefore, we used ABR for hearing assessment in infants diagnosed with congenital heart disease.
 Methods: This observational cross-sectional study was carried out on 60 infants, below or above the age of 12 months, both sexes, with CHD. Infants were furtherly divided into two equal groups: Group A with a cyanotic infant, and group B with cyanotic infants. All patients were subjected to Echocardiography.
 Results: 20% of our cases to be affected with varying degrees of hearing loss. No significant difference was found between the cyanotic and a cyanotic infant. However, cyanotic infants below 12 months had higher incidence of hearing loss when compared to those above the age of 12 months; whether the cyanotic or the cyanotic peers.
 Conclusions: High prevalence of hearing loss amongst patients with CHD warrants the addition of those patients to the high-risk registry for hearing loss, in order that they may benefit from early detection and intervention, and ergo protected against the affection of hearing impairment on their neurodevelopment. Routine follow-ups for patients with cyanotic heart disease from the age of 6 months to assess the effect of chronic hypoxemia on the inner ear and monitor the rate of improvement in ABR results, as to establish an accepted rate of improvement, and an appropriate time of intervention, if needed.

Molecular genetic diagnosis in children with cochlear implants in the Western french speaking part of Switzerland

Hearing loss is the most frequent sensory deficit at birth. Newborn hearing screening helps with early identification and clinical management of hearing deficits. A cochlear implantation is advised for profound hearing loss. Previously, an etiologic diagnosis was difficult to obtain, and many laboratory tests were required. Today, genetics has up to 60% success rate in etiologic diagnosis and is now part of the international pediatric ENT recommendations. The Centre Universitaire Romand des Implants Cochléaires (CURIC) follows children with cochlear implants. From 2015 to 2021, 26 implanted children received testing, with a 73% success rate. The genetic diagnosis helped guide their clinical management and helped to avoid unnecessary and costly clinical testing.

Variant analysis of 92 Chinese Han families with hearing loss

BackgroundHearing loss (HL) is the most frequent sensory deficit in humans, HL has strong genetic heterogeneity. The genetic diagnosis of HL is very important to aid treatment decisions and to provide prognostic information and genetic counseling for the patient’s family.MethodsWe undertook pedigree analysis in 92 Chinese non-syndromic HL patients by targeted next-generation sequencing and Sanger sequencing.ResultsAmong the 92 HL patients, 18 were assigned a molecular diagnosis with 33 different variants in 14 deafness genes. Eighteen of the variants in 12 deafness genes were novel. Variants in TMC1, CDH23, LOXHD1 and USH2A were each detected in two probands, and variants in POU3F4, OTOA, GPR98, GJB6, TRIOBP, SLC26A4, MYO15A, TNC, STRC and TMPRSS3 were each detected in one proband.ConclusionOur findings expand the spectrum of deafness gene variation, which will inform genetic diagnosis of deafness and add to the theoretical basis for the prevention of deafness.

Implementation of National Infant Screening for Hearing Program in India: Challenges, Opportunities and Suggested Framework.

Hearing impairment is most frequent sensory deficit present in infants at birth and unaddressed hearing impairment causes severe damages to both infants and their families. In India 77,756 live children are born per day which accounts for about 25 million children annually among them approximately 1.5 lacs (@ 6 per1000) children born with hearing impairment. These children have no prospects of early identification & intervention. As a result, majority of these children suffers from oral communication difficulties, poor academic performance, reduced psychosocial & cognitive development, socioeconomic insufficiency, deprived vocational opportunities & employability. However, consequences of hearing impairment are ameliorable if timely hearing screening and effective intervention strategies are instituted. Unfortunately, India has not yet envisaged any dedicated early identification and intervention program for children with hearing impairment. However, there are ample of opportunities for implementing such program. Hence, this paper endeavours to address the necessity to plan and to implement the early identification of hearing impairment by discussing burden of disorder, suggesting means to overcome the challenges and to explore and emphasize the opportunities of purposeful utilization of available infrastructure and resources. This paper also intends to suggest a conceptual framework for planning and implementing of National Infant Screening for Hearing Program to deal with serious consequences of hearing impairment in India.

A biallelic variant in CLRN2 causes non-syndromic hearing loss in humans

Deafness, the most frequent sensory deficit in humans, is extremely heterogeneous with hundreds of genes involved. Clinical and genetic analyses of an extended consanguineous family with pre-lingual, moderate-to-profound autosomal recessive sensorineural hearing loss, allowed us to identify CLRN2, encoding a tetraspan protein, as a new deafness gene. Homozygosity mapping followed by exome sequencing identified a 14.96 Mb locus on chromosome 4p15.32p15.1 containing a likely pathogenic missense variant in CLRN2 (c.494C > A, NM_001079827.2) segregating with the disease. Using in vitro RNA splicing analysis, we show that the CLRN2 c.494C > A variant leads to two events: (1) the substitution of a highly conserved threonine (uncharged amino acid) to lysine (charged amino acid) at position 165, p.(Thr165Lys), and (2) aberrant splicing, with the retention of intron 2 resulting in a stop codon after 26 additional amino acids, p.(Gly146Lysfs*26). Expression studies and phenotyping of newly produced zebrafish and mouse models deficient for clarin 2 further confirm that clarin 2, expressed in the inner ear hair cells, is essential for normal organization and maintenance of the auditory hair bundles, and for hearing function. Together, our findings identify CLRN2 as a new deafness gene, which will impact future diagnosis and treatment for deaf patients.

Correlation of homocysteine and Vitamin B12 level as a surrogative marker for early detection of sensorineural hearing loss in children

Background: Hearing impairment is the frequent sensory deficit which affects newborns, children, adults, and the elderly. Objective: This study was conducted to find out the correlation of homocysteine (Hcy) and Vitamin B12 level in children aged 2–12 years having sensorineural hearing loss (SNHL). Materials and Methods: Seventy subjects were taken in this cross-sectional study after taking approval from the Institutional Ethics Committee of KGMU, UP. (No.2096/ Ethics/R. Cell-17Dated-20/11/2017). Half (n = 35) had moderate-to-severe hearing loss, while remaining 35 had severe to profound hearing loss. The biochemical assessment of serum Hcy and Vitamin B12 level was done by chemiluminescent microparticle immunoassay method. The statistical analysis was done using Statistical Package for the Social Sciences (SPSS) Version 21.0 statistical Analysis Software. The values were represented in Number(%) and Mean±SD. Results: The mean Vitamin B12 level was significantly higher in children with moderate-to-severe hearing loss (241.06±75.25 pg/ml) as compared to those having severe to profound hearing loss (203.03±80.53 pg/ml) (P = 0.045). However, mean Hcy level was significantly higher in severe to profound hearing loss group (13.49±4.60 μmol/ml) in comparison to moderate-to-severe hearing loss group (11.14±4.57 μmol/ml) (P = 0.035). On evaluating the correlation between Vitamin B12 level and Hcy level, a strong inverse significant correlation was observed for overall cases (r=−0.691; P < 0.001) and strongly significant for moderate-to-severe hearing loss group (r=−0.779; P < 0.001). The correlation was moderate significant for severe to profound hearing loss group (r=−0.572; P < 0.001). Conclusion: This study concluded that increase Hcy level is inversely associated with decrease Vitamin B12 level in SNHL. This increase Hcy level may be used as a predictor for early detection of SNHL in children so that early therapeutic interventions can be started to improve the hearing in children.

Screening for Hearing Impairment in Older Adults by Smartphone-Based Audiometry, Self-Perception, HHIE Screening Questionnaire, and Free-Field Voice Test: Comparative Evaluation of the Screening Accuracy With Standard Pure-Tone Audiometry.

BackgroundHearing impairment is the most frequent sensory deficit in humans, affecting more than 360 million people worldwide. In fact, hearing impairment is not merely a health problem, but it also has a great impact on the educational performance, economic income, and quality of life. Hearing impairment is therefore an important social concern.ObjectiveWe aimed to evaluate and compare the accuracy of self-perception, Hearing Handicap Inventory for the Elderly-Screening (HHIE-S) questionnaire, free-field voice test, and smartphone-based audiometry as tests for screening moderate hearing impairment in older adults in China.MethodsIn this study, 41 patients were recruited through a single otology practice. All patients were older than 65 years. Patients with otorrhea and cognitive impairment were excluded. Moderate hearing impairment was defined as mean hearing thresholds at 500, 1000, 2000, and 4000 Hz >40 dB hearing loss (pure-tone average > 40 dB hearing loss). All patients completed 5 hearing tests, namely, the self-perception test, HHIE-S questionnaire test, free-field voice test, smartphone-based audiometry test, and standard pure-tone audiometry by the same audiologist. We compared the results of these tests to the standard audiogram in the better-hearing ear.ResultsThe sensitivity and the specificity of the self-perception test were 0.58 (95% CI 0.29-0.84) and 0.34 (95% CI 0.19-0.54), respectively. The sensitivity and the specificity of the HHIE-S questionnaire test were 0.67 (95% CI 0.35-0.89) and 0.31 (95% CI 0.316-0.51), respectively. The sensitivity and the specificity of the free-field voice test were 0.83 (95% CI 0.51-0.97) and 0.41 (95% CI 0.24-0.61), respectively. The sensitivity and the specificity of the smartphone-based audiometry test were 0.92 (95% CI 0.60-0.99) and 0.76 (95% CI 0.56-0.89), respectively. Smartphone-based audiometry correctly diagnosed the presence of hearing loss with high sensitivity and high specificity.ConclusionsSmartphone-based audiometry may be a dependable screening test to rule out moderate hearing impairment in the older population.

Inner Ear Gene Therapies Take Off: Current Promises and Future Challenges.

Hearing impairment is the most frequent sensory deficit in humans of all age groups, from children (1/500) to the elderly (more than 50% of the over-75 s). Over 50% of congenital deafness are hereditary in nature. The other major causes of deafness, which also may have genetic predisposition, are aging, acoustic trauma, ototoxic drugs such as aminoglycosides, and noise exposure. Over the last two decades, the study of inherited deafness forms and related animal models has been instrumental in deciphering the molecular, cellular, and physiological mechanisms of disease. However, there is still no curative treatment for sensorineural deafness. Hearing loss is currently palliated by rehabilitation methods: conventional hearing aids, and for more severe forms, cochlear implants. Efforts are continuing to improve these devices to help users to understand speech in noisy environments and to appreciate music. However, neither approach can mediate a full recovery of hearing sensitivity and/or restoration of the native inner ear sensory epithelia. New therapeutic approaches based on gene transfer and gene editing tools are being developed in animal models. In this review, we focus on the successful restoration of auditory and vestibular functions in certain inner ear conditions, paving the way for future clinical applications.

Towards optogenetic approaches for hearing restoration

Hearing impairment (HI) is the most frequent sensory deficit in humans. As yet there is no causal therapy for sensorineural HI - the most common form – that results from cochlear dysfunction. Hearing aids and electrical cochlear implants (eCIs) remain the key options for hearing rehabilitation. The eCI, used by more than 0.7 Mio people with profound HI or deafness, is considered the most successful neuroprosthesis as it typically enables open speech comprehension in quiet. By electrically stimulating the auditory nerve, eCIs constitute a brain-machine interface re-connecting the patient with the auditory scene. Nonetheless, there are short-comings resulting from the wide spread of electric current inside the cochlea which limit the quality of artificial hearing. Since light can be better confined in space than electric current, optogenetic stimulation of the auditory nerve has been suggested as an alternative approach for hearing restoration, enabling higher resolution of artificial sound encoding. Future optical CIs (oCIS) promise increased spectral selectivity of artificial sound encoding, and hence might improve speech recognition in background noise as well as processing of music.

Molecular study of hearing loss in Minas Gerais, Brazil

IntroductionDeafness is the most frequent sensory deficit in humans. Incidence is estimated at 4:1000 births in Brazil. Specific programs for clinical care of patients with hearing loss are still scarce in Brazil and the issue is an important public health problem. ObjectiveTo determine the frequency of 35delG and D13S1830 mutations in GJB2 and GJB6 genes respectively in patients with non-syndromic sensorineural hearing loss from Minas Gerais, Brazil. MethodsThis research involved 53 individuals, who were assessed by a questionnaire for predicting the possibility of non-syndromic deafness and for data collecting. Samples were tested for the presence of the 35delG mutation in GJB2 gene and D13S1830 in GJB6 gene by polymerase chain reaction and restriction enzyme digestion. ResultsEpidemiological research has shown that the majority of the subjects are unaware of the etiology and the pathogenesis of hearing loss. In 9 patients (16.98%), 35delG mutation was found in heterozygosis and the allele frequency was estimate to be around 8.5%. Although 9.61% of the patients reported having some degree of consanguinity between the parents and 12.08% reported other cases of deafness in their families, this mutation was not found in homozygosis. The D13S1830 mutation was not found in this study. ConclusionThis research describes for the first time the frequency of the 35delG and D13S1830 mutation in hearing-impaired individuals from Minas Gerais, Brazil, and the collected data reinforce the need for further studies in this population due to heterogeneity of hearing loss.

Hearing impairment in rural area of Himalaya: prevalence and etiology

&lt;p class="abstract"&gt;&lt;strong&gt;Background:&lt;/strong&gt; &lt;span lang="EN-IN"&gt;Hearing impairment is most frequent sensory deficit in human population. The disease burden estimations based on sound epidemiological research provide the foundation for appropriate public policy focus and measures for effective management of disease conditions. Data regarding the magnitude of hearing impairment in our country is limited and the literature search revealed that no such studies have been conducted in this region. &lt;/span&gt;&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Methods:&lt;/strong&gt; &lt;span lang="EN-IN"&gt;A community based cross sectional observational study was carried out among the 306 individuals in rural area of Shimla district, Himachal Pradesh. Information was obtained by a structured questionnaire, clinical ENT examination and audiological tests after obtaining informed consent. &lt;/span&gt;&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Results:&lt;/strong&gt; &lt;span lang="EN-IN"&gt;The study group had 52.6% males and 47.4% females. Maximum 26.5% of individuals were in the age group of 31-45 years. Mean age was 36.56±18.83. The prevalence of hearing impairment was 16.7%. Among individuals with hearing loss, maximum 52.9% were in the age group of ≥60 years. Sensorineural hearing loss was found in 68.6%, maximum 56.9% had mild hearing loss. Among majority of individuals with hearing loss cause was presbyacusis 52.9% followed by infectious ear disease 33.3%.&lt;/span&gt;&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Conclusions:&lt;/strong&gt; &lt;span lang="EN-IN"&gt;In the present study, prevalence of hearing loss was found to be 16.7% and predominantly mild sensorineural or conductive type of hearing loss. An early intervention and quality patient education is necessary for prevention of hearing impairment in majority of cases.&lt;/span&gt;&lt;/p&gt;

Distinct Expression Patterns Of Causative Genes Responsible For Hereditary Progressive Hearing Loss In Non-Human Primate Cochlea.

Hearing impairment is the most frequent sensory deficit in humans. Deafness genes, which harbor pathogenic mutations that have been identified in families with hereditary hearing loss, are commonly expressed in the auditory end organ or the cochlea and may contribute to normal hearing function, yet some of the mouse models carrying these mutations fail to recapitulate the hearing loss phenotype. In this study, we find that distinct expression patterns of those deafness genes in the cochlea of a non-human primate, the common marmoset (Callithrix jacchus). We examined 20 genes whose expression in the cochlea has already been reported. The deafness genes GJB3, CRYM, GRHL2, DFNA5, and ATP6B1 were expressed in marmoset cochleae in patterns different from those in mouse cochleae. Of note, all those genes are causative for progressive hearing loss in humans, but not in mice. The other tested genes, including the deafness gene COCH, in which mutation recapitulates deafness in mice, were expressed in a similar manner in both species. The result suggests that the discrepancy in the expression between rodents and primates may account for the phenotypic difference. This limitation of the rodent models can be bypassed by using non-human primate models such as the marmoset.

Incidence of risk factors for hearing impairment in premature babies

According to the World Health Organization, the incidence of hearing impairment in newborn population is 1-3 per 1000 (WHO, 2012). Apart from that, many authors have found that the incidence of hearing impairment is twenty times higher, 2-4%, in neonatal intensive care unit (NICU). Thus, a congenital hearing loss is the most frequent sensory or motor deficit that could be diagnosed immediately upon birth. The objective of this study was to determine the incidence of risk factors for hearing impairment in the population of preterm babies. We were especially interested in the impact of gestational age at birth on the incidence of risk factors for hearing loss. A cohort of 150 preterm babies was enrolled in the study. They were hospitalized in the Institute for Neonatology in Belgrade during 2014 and 2015 and the data were obtained from their medical files. The results of this study indicate high incidence of risk factors for hearing impairment in this population of babies. Gestational age at birth had a strong, statistically significant, correlation with risk factor incidence in lower gestational age and vice versa. High incidence of risk factors and their interaction could account for twenty times higher occurrence of congenital and early acquired hearing loss in population of preterm babies compared to term neonates. These results imply the need for systematic audiological surveillance of prematurely born babies at least until 12 months of corrected age.

Road users with hearing loss and evaluation of tactile support systems

Hearing loss (HL) is one of the most frequent sensory deficit in humans (Stevens et al., 2013), which might reduce traffic safety. The prevalence of age-related HL is increasing (Roth et al., 2001), and as a consequence the number of road users with HL will also increase. The effects of HL on traffic safety have been investigated and the use of tactile support systems has been evaluated both in a driving simulator and in real traffic. Tactile support was used to alert the driver during the simulator drive and to facilitate navigation with a GPS during the drive in real traffic. Differences related to HL in terms of driving behavior were bound to driving condition and occurred when complexity of driving task increased. There was also an effect of HL on visual behavior, indicated in the simulator and confirmed in the field study, suggesting that drivers with HL have a more active visual behavior with more frequent glances in the rear view mirror and a general scanning of the environment before looking away from road. Tactile signal in the driver seat was found useful, both for calling for driver attention and to facilitate navigation. Also, of high relevance for the traffic safety aspect and regardless of hearing status, the tactile support lead to higher satisfaction with the navigation system, less time spent to look at the navigation display, and thus more focus on road and better driving performance. The effects of HL on traffic safety consistently point towards a generally more cautious driving behavior. Compensatory strategies associated with HL include driving at lower speeds, more comprehensive visual search behavior and less engagement in distracting activities. Evaluation of a tactile signal suggests that by adding a tactile modality, some driver assistance systems can be made accessible also for drivers with HL. At the same time the systems might be more effective for all users, which could generally increase both traffic safety and mobility. Roth, T.N., Hanebuth, D., &amp; Probst, R. (2001). Prevalence of age-related hearing loss in Europe: a review. European Archives of Oto-Rhino-Laryngology, 268(8), 1101–1107. Stevens, G., Flaxman, S., Brunskill, E., Mascarenhas, M., Mathers, CD., &amp; Finucane, M. (2013). Global and regional hearing impairment prevalence: an analysis of 42 studies in 29 countries. European Journal of Public Health, 23(1), 146–152.

Self-reported hearing impairment among rural adult population of coastal Tamil Nadu

&lt;p class="abstract"&gt;&lt;strong&gt;&lt;span lang="EN-US"&gt;Background: &lt;/span&gt;&lt;/strong&gt;Hearing impairment is the most frequent sensory deficit in human population and irrespective of the age of development, has devastating consequences. This study was done to assess the burden of hearing impairment among the rural adult population of a coastal area in Tamil Nadu. &lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;&lt;span lang="EN-US"&gt;Methods: &lt;/span&gt;&lt;/strong&gt;This community-based descriptive study was conducted in a rural area of Tamil Nadu state in India. The study protocol was approved by the institute ethics committee of PIMS. Eligible participants from all the household in the study area were selected. A semi-structured questionnaire was used to collect the information and hearing impairment was assessed by self-reporting by the participants. Proportions for categorical variables were calculated and Chi-square test was applied to find the associations.&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;&lt;span lang="EN-US"&gt;Results: &lt;/span&gt;&lt;/strong&gt;Among a total of 5621 participants interviewed and examined, 232 (4.1%) participants reported to have hearing impairment. Among them, majority reported that they have hearing impairment due to advanced age or non-specific causes resulted in hard to hearing (77.6%) and 12.5% reported to have deafness. Ear discharge (4.3%), wax (3.0%) were other commonly reported cause of hearing impairment. The hearing impairment increased with age and was common among elderly. The reported hearing impairment ranges from 1.5% to 11.3% in different age groups. Age, sex, education and occupation were significantly associated with hearing impairment (p value &amp;lt;0.05).&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;&lt;span lang="EN-US"&gt;Conclusions: &lt;/span&gt;&lt;/strong&gt;A large number of people in rural communities were having hearing impairment. Improving access to health education and raising awareness about prevention and the needs of people with hearing loss in community is required.&lt;/p&gt;

A82 Road users with hearing loss and evaluation of tactile support systems

Abstract Background Research regarding the effect of Hearing loss (HL) on traffic safety and mobility is limited and the empirical findings are somewhat inconsistent. HL is one of the most frequent sensory deficit in humans, which may affect behavior in traffic situations and might reduce traffic safety. The prevalence of age-related HL in Europe is roughly 30% for men and 20% for women at the age of 70 years, and 55% for men and 45% for women at the age of 80 years. The prevalence of age-related HL is increasing, and as a consequence the number of road users with HL will also increase. Methods To investigate traffic safety and mobility for individuals with HL three studies have been conducted; 1) a questionnaire survey, 2) a driving simulator study, 3) a field study. Results The effects of HL on traffic safety and mobility are small, often bound to workload condition, however quite consistent. Differences in transportation habits related to HL include less likelihood of having a driving license and a higher valuing of written information, with the latter sometimes prioritized before time and safety issues. Respondents with higher degree of HL were less concerned about the effect of HL, indicating that they might be using compensatory strategies. Differences related to HL in driving behavior were bound to driving condition and occurred when complexity of driving task increased. There was also an effect of HL on visual behavior, suggesting that drivers with HL have a more active visual behavior with more frequent glances in the rear view mirror and a general scanning of the environment before looking away from road. Tactile signal in the driver seat was found useful in both experimental studies, both for calling for driver attention and to facilitate navigation with a GPS. Of high relevance for traffic safety and regardless of hearing status, the tactile support lead to higher satisfaction with the navigation system, less time spent to look at the navigation display, and more focus on road and better driving performance. Conclusions There are effects of HL on both traffic safety and mobility, consistently pointing towards a generally more cautious driving behavior, suggesting a difference in experienced safety. The appearance of compensatory strategies associated with HL is bound to driving condition and include driving at lower speeds, more comprehensive visual search behavior and less engagement in distracting activities. By adding a tactile modality, some driver assistance systems can be made accessible also for drivers with HL.

A Comparative Study on Efficacy of Fludrocortisones versus Glucocorticoids and Vasodilators in the Treatment of Idiopathic Sensorineural Cochlear Hearing Loss

Introduction and Objectives: The idiopathic sensorineural cochlear hearing loss is one of the most frequent human sensory deficits and there is no specific drug therapy for it. The possible hearing recovery is related with the reestablishment of normal ionic homeostasis of the endolymph controlled by the mineralocorticoid as could be demonstrate experimentally. The purpose of this clinical trial was to confirm the efficacy of mineralocorticoids to the recovery of hearing level in patients suffering idiopathic sensorineural hearing loss (SNHL) against the glucocorticoids and vasodilator drugs. Material and Methods: The research lasted three months and involved 90 patients allocated into four different groups: Placebo group, consisted of 20 patients (10 men and 10 women); the group consisting of 22 patients treated with glucocorticoid therapy (12 men and 10 female); the group treated with mineralocorticoid therapy encompassed 26 patients (13 males and 13 females) and the group of vasodilators formed by 22 patients (12 men and 10 women). The level of hearing loss was estimated by the tests Liminal Tone Audiometry (LTA) and Auditory Brainstem Response (ABR). Results: The main features in this research were overall better response in improving the hearing level with the mineralocorticoid therapy. This improvement in hearing levels was greater in women than in men, and a higher response was found in the left ear regardless of patient’s gender. Conclusions: The hearing gain was significantly superior in the mineralocorticoids group followed by the glucocorticoids group whereas the response to vasodilators was lesser and with no statistical significance.

Protective effect of rasagiline in aminoglycoside ototoxicity

Sensorineural hearing losses (SNHLs; e.g., ototoxicant- and noise-induced hearing loss or presbycusis) are among the most frequent sensory deficits, but they lack effective drug therapies. The majority of recent therapeutic approaches focused on the trials of antioxidants and reactive oxygen species (ROS) scavengers in SNHLs. The rationale for these studies was the prominent role of disturbed redox homeostasis and the consequent ROS elevation. Although the antioxidant therapies in several animal studies seemed to be promising, clinical trials have failed to fulfill expectations.We investigated the potential of rasagiline, an FDA-approved monomanine oxidase type B inhibitor (MAO-B) inhibitor type anti-parkinsonian drug, as an otoprotectant. We showed a dose-dependent alleviation of the kanamycin-induced threshold shifts measured by auditory brainstem response (ABR) in an ototoxicant aminoglycoside antibiotic-based hearing loss model in mice. This effect proved to be statistically significant at a 6-mg/kg (s.c.) dose. The most prominent effect appeared at 16kHz, which is the hearing sensitivity optimum for mice. The neuroprotective, antiapoptotic and antioxidant effects of rasagiline in animal models, all targeting a specific mechanism of aminoglycoside injury, may explain this otoprotection.The dopaminergic neurotransmission enhancer effect of rasagiline might also contribute to the protection. Dopamine (DA), released from lateral olivocochlear (LOC) fibers, was shown to exert a protective action against excitotoxicity, a pathological factor in the aminoglycoside-induced SNHL. We have shown that rasagiline enhanced the electric stimulation-evoked release of DA from an acute mouse cochlea preparation in a dose-dependent manner. Using inhibitors of voltage-gated Na+-, Ca2+ channels and DA transporters, we revealed that rasagiline potentiated the action potential-evoked release of DA by inhibiting the reuptake.The complex, multifactorial pathomechanism of SNHLs most likely requires drugs acting on multiple targets for effective therapy. Rasagiline, with its multi-target action and favorable adverse effects profile, might be a good candidate for a clinical trial testing the otoprotective indication.

Clinical features in Guillain–Barré syndrome with anti-Gal-C antibody

IntroductionGuillain–Barré syndrome (GBS) has often been associated with antibodies to glycolipids, such as galactocerebroside (Gal-C), a component of myelin. Whether patients who have GBS with anti-Gal-C antibody (Gal-C-GBS) more often have demyelinating neuropathy or axonal neuropathy remains controversial. Their clinical features have also been unestablished. MethodsWe enrolled 47 patients with Gal-C-GBS. Their clinical and electrophysiological data were retrospectively reviewed and compared to 119 patients with GBS without anti-Gal-C antibody (non-Gal-C-GBS). ResultsDemyelinating polyneuropathy occurred 4 times more frequently than axonal polyneuropathy in patients with Gal-C-GBS, but without statistical significance compared to patients with non-Gal-C-GBS (2.2:1). Patients with Gal-C-GBS had more frequent sensory deficits, autonomic involvements, and antecedent Mycoplasma pneumoniae (MP) infection than patients with non-Gal-C-GBS. ConclusionsThis is the largest study clarifying the clinical and electrophysiological findings that more frequent sensory deficits, autonomic involvements, and antecedent MP infection are associated with Gal-C-GBS.