scholarly journals Hearing Phenotypes of Patients with Hearing Loss Homozygous for the GJB2 c.235delc Mutation

2020 ◽  
Vol 2020 ◽  
pp. 1-11
Author(s):  
Chang Guo ◽  
Sha-Sha Huang ◽  
Yong-Yi Yuan ◽  
Ying Zhou ◽  
Ning Wang ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Type A ◽  
Pathogenic Mutation ◽  
Considerable Proportion ◽  
Homozygous Mutation ◽  
Type B ◽  
Profound Hearing Loss ◽  
Severe Hearing Loss ◽  
Type C ◽  
Loss Severity

Hereditary hearing loss is characterized by remarkable phenotypic heterogeneity. Patients with the same pathogenic mutations may exhibit various hearing loss phenotypes. In the Chinese population, the c.235delC mutation is the most common pathogenic mutation of GJB2 and is closely related to hereditary recessive hearing loss. Here, we investigated the hearing phenotypes of patients with hearing loss associated with the homozygous c.235delC mutation, paying special attention to asymmetric interaural hearing loss. A total of 244 patients with the GJB2 c.235delC homozygous mutation encountered from 2007 to 2015 were enrolled. The severity of hearing loss was scaled with the American Speech-Language-Hearing Association (ASHA). Auditory phenotypes were analyzed, and three types of interaural asymmetry were defined based on audiograms: Type A (asymmetry of hearing loss severity), Type B (asymmetry of audiogram shape), and Type C (Type A plus Type B). Of the 488 ears (244 cases) examined, 71.93% (351) presented with profound hearing loss, 14.34% (70) with severe hearing loss, and 9.43% (46) with moderate to severe hearing loss. The most common audiogram shapes were descending (31.15%) and flat (24.18%). A total of 156 (63.93%) of the 244 patients exhibited asymmetric interaural hearing loss in terms of severity and/or audiogram shape. Type A was evident in 14 of these cases, Type B in 106, and Type C in 36. In addition, 211 of 312 ears (67.63%) in the interaural hearing asymmetry group showed profound hearing loss, and 59 (18.91%) exhibited severe hearing loss, with the most common audiogram shapes being flat (27.88%) and descending (22.12%). By contrast, in the interaural hearing symmetry group, profound hearing loss was observed in 140 ears (79.55%), and the most common audiograms were descending (46.59%) and residual (21.59%). Hearing loss associated with the GJB2 c.235delC homozygous mutation shows diverse phenotypes, and a considerable proportion of patients show bilateral hearing loss asymmetry.

Tympanometric findings in cleft palate patients: influence of age and cleft type

2001 ◽  
Vol 115 (2) ◽  
pp. 91-96 ◽  
Author(s):  
Jadranka Handžić-Ćuk ◽  
Višeslav Ćuk ◽  
Miroslav Gluhinić ◽  
Ranko Rišavi ◽  
Smiljana štajner-Katušić
Keyword(s):  
Hearing Loss ◽  
Cleft Palate ◽  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Type A ◽  
Total Sample ◽  
Hearing Level ◽  
Type B ◽  
Age Related ◽  
Type C

Tympanometry was analysed according to cleft type with respect to age in 239 cleft palate patients (57 with bilateral cleft lip and palate (BCLP)), 122 with unilateral cleft lip and palate (UCLP) and 60 with isolated cleft palate (ICP)). The frequency of type B tympanograms was 46.5 per cent in the BCLP group, 50.6 per cent in the UCLP group and 58.3 per cent in the ICP group. Type A tympanograms were more frequently observed in older patients (median age 11 years) in comparison to type B (median age five years; p<0.001) and type C (median age six years; p<0.001). The total sample showed an age-related decrease in the frequency of type B tympanograms (rs = −0.3942; p<0.001). The frequency of type A tympanograms increased significantly with age (rs = 0.4263; p<0.001), whereas type C was not correlated with age. In the UCLP group, the frequency of type B tympanograms decreased with age (rs = −0.4430; p<0.001), the decrease being faster than in the BCLP group (rs = −0.3186; p = 0.001) and the ICP group (rs = −0.3378; p<0.0001)). Type B tympanograms had the highest correlation with a hearing loss of 21–40 dB at mean hearing level at speech frequencies (MHLSF) (rs = 0.4574; p<0.001), a lower correlation with a hearing loss of 11–20 dB (rs = 0.2184; p = 0.02) and the lowest correlation with hearing loss above 40 dB. At the ages of one to three, the frequency of type B is higher in UCLP patients than in the BCLP and ICP groups, decreasing at seven to 12, increasing again at 13 to 15, and thereafter showing a continuous decrease. In the BCLP group, the frequency of type B increased significantly at the ages of four to six and then decreased continuously from seven to 18. In ICP patients, the changes in the frequency of type B with age are not significant until the age of 15; at the age of seven, it is higher than in the BCLP and ICP groups. The type C tympanogram is not typically found in cleft palate patients and its frequency is not correlated with age.It can be presumed that each type of cleft lip and palate, due to its characteristic conditions in the epipharynx, will favour a different mode of pathophysiological development of middle-ear disease.

scholarly journals The Physiologic and Psychophysical Consequences of Severe-to-Profound Hearing Loss

2018 ◽  
Vol 39 (04) ◽  
pp. 349-363 ◽  
Author(s):  
Eric Hoover ◽  
Pamela Souza
Keyword(s):  
Hearing Loss ◽  
Speech Recognition ◽  
Treatment Options ◽  
Sufficient Information ◽  
Noisy Environments ◽  
Cochlear Function ◽  
Profound Hearing Loss ◽  
Sound Perception ◽  
Severe Hearing Loss ◽  
Tuning Curves

AbstractSubstantial loss of cochlear function is required to elevate pure-tone thresholds to the severe hearing loss range; yet, individuals with severe or profound hearing loss continue to rely on hearing for communication. Despite the impairment, sufficient information is encoded at the periphery to make acoustic hearing a viable option. However, the probability of significant cochlear and/or neural damage associated with the loss has consequences for sound perception and speech recognition. These consequences include degraded frequency selectivity, which can be assessed with tests including psychoacoustic tuning curves and broadband rippled stimuli. Because speech recognition depends on the ability to resolve frequency detail, a listener with severe hearing loss is likely to have impaired communication in both quiet and noisy environments. However, the extent of the impairment varies widely among individuals. A better understanding of the fundamental abilities of listeners with severe and profound hearing loss and the consequences of those abilities for communication can support directed treatment options in this population.

scholarly journals The effectiveness of TiO2 additions to mortar to maintain initial conditions in terms of its reflectance to solar radiation

2017 ◽  
Vol 17 (3) ◽  
pp. 39-56 ◽  
Author(s):  
Sérgio Roberto Andrade Dantas ◽  
Fulvio Vittorino ◽  
Kai Loh
Keyword(s):  
Solar Radiation ◽  
Ultraviolet Radiation ◽  
Initial Conditions ◽  
Type A ◽  
Just Noticeable Difference ◽  
Type B ◽  
Climate Conditions ◽  
Type D ◽  
Type C ◽  
Over Time

Abstract Contact of facades with degradation agents and direct incidence of ultraviolet radiation on external coatings make them more opaque over time, affecting their colour and reflectance characteristics. This study evaluated the effect of adding different TiO2 contents to mortars applied in concrete substrates in order to verify the reflectance maintenance on surfaces after exposure over time. Mortar with different concentrations of TiO2 (1%, 5%, 10%) were produced in relation to the total dry premix, added as a powder and compared to unpainted mortar without TiO2 (type "A") and painted mortar without TiO2 (type "B"), both used as a reference for colour and reflectance. Exposed over 16 months to climate conditions in São Paulo, regarding the maintenance of reflectance and solar radiation, the results showed that type "B" (0%TiO2) painted mortar presented the best performance. Type "C" (1%TiO2) and type "D" (5%TiO2) unpainted mortar remained more stable. Type "A" (0%TiO2) and type "E" (10%TiO2) unpainted mortar showed greater differences according to the Just Noticeable Difference (JND) range caused by dirt pick up.

Judgments of Grammatically by Normal and Language-Disordered Children

1977 ◽  
Vol 42 (2) ◽  
pp. 199-209 ◽  
Author(s):  
Betty Z. Liles ◽  
Martin D. Shulman ◽  
Susan Bartlett
Keyword(s):  
Word Order ◽  
Sentence Type ◽  
Type A ◽  
Order Type ◽  
Specific Reference ◽  
Type B ◽  
Normal Children ◽  
Grammatical Errors ◽  
Type C

Fifteen linguistically normal children and 15 linguistically deviant children were presented with three types of agrammatical sentences. The subjects were asked to judge the sentences as right or wrong and to change the sentences judged as wrong, rendering them correct. The three types of agrammatical sentences represented rule violations of syntactic agreement (Type A), lexical restrictions (Type B), and word order (Type C). The two groups of children were compared in terms of the number of sentences of each type that were recognized as agrammatical. Those productions which represented the child’s correction of agrammatical sentences were subjected to descriptive analyses (percentages) with specific reference to the number of attempted changes and the number of those changes which demonstrated corrections of the specific deviation from well formedness. Results indicated that the two groups of subjects were significantly different in their ability to recognize grammatical errors in sentence Types A and C, but did not differ in their ability to recognize errors in sentence Type B. The descriptive comparison of the groups' verbal corrections reflected this trend, in that the language-disordered subjects made corrections specific to the error on more of the Type B sentences (for example, “The dog writes the food.”) than on Types A (for example, “'She will pick some flowers last week.”) or C (for example, “Get and come your dinner.”). Linguistically normal children accurately corrected 90.7% of the sentences judged as agrammatical; this percentage did not vary more than 1% across sentence types.

scholarly journals Structural and fractal characterization of adsorption pores of middle–high rank coal reservoirs in western Yunnan and eastern Guizhou: An experimental study of coals from the Panguan syncline and Laochang anticline

2018 ◽  
Vol 37 (1) ◽  
pp. 251-272 ◽  
Author(s):  
Junjian Zhang ◽  
Chongtao Wei ◽  
Gaoyuan Yan ◽  
Guanwen Lu
Keyword(s):  
Specific Surface ◽  
Surface Area ◽  
Specific Surface Area ◽  
Pore Volume ◽  
Pore Diameter ◽  
Structural Parameters ◽  
Total Pore Volume ◽  
Type A ◽  
Type B ◽  
Type C

To better understand the structural characteristic of adsorption pores (pore diameter < 100 nm) of coal reservoirs around the coalbed methane production areas of western Yunnan and eastern Guizhou, we analyzed the structural and fractal characteristics of pore size range of 0.40–2.0 nm and 2–100 nm in middle–high rank coals ( Ro,max = 0.93–3.20%) by combining low-temperature N2/CO2 adsorption tests and surface/volume fractal theory. The results show that the coal reservoirs can be divided into three categories: type A ( Ro,max < 2.15%), type B (2.15% <  Ro,max <2.50%), and type C ( Ro,max > 2.15%). The structural parameters of pores in the range from 2 to 100 nm are influenced by the degree of coal metamorphism and the compositional parameters (e.g., ash and volatile matter). The dominant diameters of the specific surface areas are 10–50 nm, 2–50 nm, and 2–10 nm, respectively. The pores in the range from <2 nm provide the largest proportion of total specific surface area (97.22%–99.96%) of the coal reservoir, and the CO2-specific surface area and CO2-total pore volume relationships show a positive linear correlation. The metamorphic degree has a much greater control on the pores (pore diameter less than 2 nm) structural parameters than those of the pore diameter ranges from 2 to 100 nm. Dv1 and Dv2 can characterize the structure of 2–100 nm adsorption pores, and Dv1 (volume heterogeneity) has a positive correlation with the pore structural parameters such as N2-specific surface area and N2-total pore volume. This parameter can be used to characterize volume heterogeneity of 2–10 nm pores. Dv2 (surface heterogeneity) showed type A > type B > type C and was mainly affected by the metamorphism degree. Ds2 can be used to characterize the pore surface heterogeneity of micropores in the range of 0.62–1.50 nm. This parameter has a good correlation with the pore parameters (CO2-total pore volume, CO2-specific surface area, and average pore size) and is expressed as type C < type B < type A. In conclusion, the heterogeneity of the micropores is less than that of the meso- and macropores (2–100 nm). Dv1, Dv2, and Ds2 can be used as effective parameters to characterize the pore structure of adsorption pores. This result can provide a theoretical basis for studying the pore structure compatibility of coal reservoirs in the region.

scholarly journals Postsurgical residual lung complications following left upper trisegmentectomy

2019 ◽  
Author(s):  
Yutaro Koike ◽  
Aritoshi Hattori ◽  
Takeshi Matsunaga ◽  
Kazuya Takamochi ◽  
Shiaki Oh ◽  
...  
Keyword(s):  
Surgical Intervention ◽  
Type A ◽  
X Rays ◽  
Limited Information ◽  
Type B ◽  
Type D ◽  
Thoracic Computed Tomography ◽  
Isolated Segment ◽  
Type C ◽  
Lung Torsion

AbstractOBJECTIVESSegmentectomy has become an increasingly popular surgical procedure for small-sized lung lesions. Left upper trisegmentectomy (LUTS) is one of the most common segmentectomies performed because of its relative ease and simplicity; however, limited information is currently available on the specific postoperative complications associated with this procedure.METHODSAmong 2060 surgically resected cases in our institute between 2009 and 2016, 129 (6.2%) underwent LUTS. Postoperative chest X-rays and/or thoracic computed tomography (CT) scans were retrospectively assessed for all cases to assess postsurgical residual lung complications following LUTS. We categorized cases into 4 groups: type A (atelectasis of the lingular segment), type B (lung torsion of the lingular segment), type C (necrosis of the ‘isolated segment’) and type D (haematoma along stapling lines).RESULTSPostsurgical lung complications following LUTS were observed in 17 (13.1%) patients (type A: n = 7, type B: n = 1, type C: n = 4 and type D: n = 5). Three patients (2.3%) required surgical intervention because of type B (n = 1) and type C (n = 2), namely, decreased permeability and remaining ground glass opacities in the residual lung, showing an exacerbated systemic inflammatory response. In contrast, type A and D cases were successfully observed by chest CT without any surgical intervention, and patients recovered within a few months of surgery.CONCLUSIONSWe identified several postoperative residual lung complications following LUTS. Lung torsion or necrosis of the residual segment may require intensive care, including reoperation. Potentially serious complications always need to be ruled out after LUTS when radiological consolidation is detected postoperatively.

Research on Flow Units of Extra-Low Permeability Reservoirs in Fault Block Sheng554 of Shengping Oilfield

2013 ◽  
Vol 295-298 ◽  
pp. 3162-3165
Author(s):  
Lu Lu Zhou ◽  
Zi Nan Li ◽  
Jun Gang Liu ◽  
Yan Yun Zhang ◽  
Guang Qiang Shu
Keyword(s):  
Fluid Flow ◽  
Water Injection ◽  
Type A ◽  
Low Permeability ◽  
Type B ◽  
Flow Units ◽  
Fault Block ◽  
Remaining Oil ◽  
Type C ◽  
Low Permeability Reservoirs

Taking the example of the fourth member of the Lower Cretaceous Quantou formation reservoirs in fault block Sheng554 of Sanzhao sag, this article discusses the methodology of flow units in extra-low permeability reservoirs. The research on flow units in such reservoirs can be divided into two ranks, one is to determine the distribution of seepage barriers and inner connected sands， the other is to analyze the differentia of fluid flow in the inner connected sands so as to subdivide the flow units. The result shows that the pelitic barriers are rather developed in fault block Sheng554. Through the analysis of differentia of fluid flow, according to the value of flow zone index (FZI), the inner connected sands can be classified into three types of flow units, among which type A with FZI value greater than 1.0 has better permeable property and higher intensity of water injection, and the ability of permeability and water injection of type B with FZI value between 0.5 and 1.0 takes the second place, and type C is the worst flow unit with the worst permeable property and intensity of water injection with FZI value less than 0.5. Among the three types of flow units, type A poorly develops, while type B and type C develops well. The research on flow units can provide reliable geologic bases for forecasting the distribution of remaining oil in extra-low permeability reservoirs and for developing remaining oil in the study area.

A New Classification of Cochleovestibular Malformations and Implications for Predicting Speech Perception Ability after Cochlear Implantation

2015 ◽  
Vol 20 (2) ◽  
pp. 90-101 ◽  
Author(s):  
Sung-Wook Jeong ◽  
Lee-Suk Kim
Keyword(s):  
Speech Perception ◽  
Cochlear Implantation ◽  
Test Scores ◽  
Type A ◽  
Cochlear Nerve ◽  
Type B ◽  
New Classification ◽  
Type D ◽  
Type C

Objectives: The aims of this study were to introduce a new classification of cochleovestibular malformation (CVM) and to investigate how well this classification can predict speech perception ability after cochlear implantation in children with CVM. Methods: Fifty-nine children with CVM who had used a cochlear implant for more than 3 years were included. CVM was classified into 4 subtypes based on the morphology of the cochlea and the modiolus on temporal bone computed tomography (TBCT): normal cochlea and normal modiolus (type A, n = 16), malformed cochlea and partial modiolus (type B, n = 31), malformed cochlea and no modiolus (type C, n = 6), and no cochlea and no modiolus (type D, n = 6). Speech perception test scores were compared between the subtypes of CVM using analysis of covariance with post hoc Bonferroni test. Univariate and multivariate regression analyses were used to identify the significant predictors of the speech perception test scores. Results: The speech perception test scores after implantation were significantly better in children with CVM type A or type B than in children with CVM type C or type D. The test scores did not differ significantly between the implanted children with CVM type A or type B and those without CVM. In univariate regression analysis, the type of CVM was a significant predictor of the speech perception test scores in implanted children with CVM. Multivariate regression analysis revealed that the age at cochlear implantation, cochlear nerve size and preimplantation speech perception test scores were significant predictors of the postimplantation speech perception test scores. The chance of cochlear nerve deficiency increased progressively from CVM type A to type D. Conclusion: The new classification of CVM based on the morphology of the cochlea and the modiolus is simple and easy to use, and correlated well with postimplantation speech perception ability and cochlear nerve status. This simple classification of CVM using TBCT with cochlear nerve assessment by magnetic resonance imaging is helpful in the preoperative evaluation of children with CVM.

GROUPING OF ISOLATES OF A LOW-TEMPERATURE BASIDIOMYCETE ON THE BASIS OF CULTURAL BEHAVIOR AND PATHOGENICITY

1961 ◽  
Vol 39 (2) ◽  
pp. 297-306 ◽  
Author(s):  
E. W. B. Ward ◽  
J. B. Lebeau ◽  
M. W. Cormack
Keyword(s):  
Low Temperature ◽  
Host Plants ◽  
Type A ◽  
Effect Of Temperature ◽  
Western Canada ◽  
Type B ◽  
Highly Pathogenic ◽  
Cultural Behavior ◽  
Type C ◽  
Ph Range

Isolates of an unidentified low-temperature basidiomycete, associated with snow mold in Western Canada, were divided into three types, A, B, and C, on the basis of their general cultural appearance. Support for this classification was obtained when representative isolates of each type were examined to determine: the effect of temperature and pH on growth; tolerance of antibiotics and HCN; ability to liberate HCN in culture and in the host plant; pathogenicity.Type A isolates grew slowly under most conditions and were least tolerant of the extremes of temperature and pH employed, moderately inhibited by antibiotics, and strongly inhibited by HCN. They produced HCN in large quantities in host plants and none in culture. They were moderately pathogenic on grass and highly pathogenic on alfalfa. Type B isolates grew somewhat more rapidly than type A, especially at the upper and lower temperatures, and grew over a wider pH range. These isolates were more tolerant of antibiotics and HCN. They produced smaller quantities of HCN than type A in infected alfalfa plants but released large amounts in culture. They were less pathogenic than type A on alfalfa but similarly pathogenic on grass. Type C isolates were fast-growing forms which were strongly inhibited by antibiotics and HCN. They did not liberate HCN under any conditions and were not pathogenic.

A Molecular Dynamics Simulation Study on the Relationship between Hydrogen Bond and Damping Properties of AO-70/NBR Composites

2017 ◽  
Vol 748 ◽  
pp. 29-34 ◽  
Author(s):  
Jing Zhu ◽  
Xiu Ying Zhao ◽  
Meng Song ◽  
Yue Han ◽  
Li Liu ◽  
...  
Keyword(s):  
Md Simulation ◽  
Type A ◽  
Dynamics Simulation ◽  
Butadiene Rubber ◽  
Damping Properties ◽  
Type B ◽  
Fractional Free Volume ◽  
Type C ◽  
Hindered Phenol ◽  
The Relationship

This work was try to study the number and types of hydrogen bonds (H-bonds) formed in hindered phenol AO-70/nitrile butadiene rubber (NBR) composites and their contributions to the damping properties by molecular dynamic (MD) simulation and experimental methods. MD simulation results showed that there were four types of H-bonds, namely, type A (AO-70) –OH...NC– (NBR) H-bonds in AO-70/NBR composites, type B (AO-70) –OH...O=C– (AO-70) H-bonds, type C (AO-70) –OH...OH–(AO-70) and D (AO-70) –OH...O–C– (AO-70) H-bonds, what's more, type A and type B H-Bonds formed more easily than others. Fourier transform infrared spectroscopy (FTIR) confirmed the existence of H-bonds. Meanwhile, the AO-70/NBR composites with AO-70 content of 109 phr had the largest number of H-bonds, smallest fractional free volume (FFV) and resulting in the optimistic damping performance of the composites.

Sign in / Sign up

Export Citation Format

Share Document