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Sample records for abnormal ear development

  1. Low-set ears and pinna abnormalities

    MedlinePlus

    Low-set ears; Microtia; "Lop" ear; Pinna abnormalities; Genetic defect-pinna; Congenital defect-pinna ... The outer ear or "pinna" forms when the baby is growing in the mother's womb. The growth of this ear part ...

  2. Prenatal Ultrasound Screening for External Ear Abnormality in the Fetuses

    PubMed Central

    Wei, Jun; Ran, Suzhen; Yang, Zhengchun; Lin, Yun; Tang, Jing

    2014-01-01

    Objectives. To investigate the best time of examination and section chosen of routine prenatal ultrasound screening for external ear abnormalities and evaluate the feasibility of examining the fetal external ear with ultrasonography. Methods. From July 2010 until August 2011, 42118 pregnant women with single fetus during 16–40 weeks of pregnancy were enrolled in the study. Fetal auricles and external auditory canal in the second trimester of pregnancy were evaluated by routine color Doppler ultrasound screening and systematic screening. Ultrasound images of fetal external ears were obtained on transverse-incline view at cervical vertebra level and mandible level and on parasagittal view and coronal view at external ear level. Results. Five fetuses had anomalous ears including bilateral malformed auricles with malformed external auditory canal, unilateral deformed external ear, and unilateral microtia. The detection rate of both auricles was negatively correlated with gestational age. Of the 5843 fetuses undergoing a routine ultrasound screening, 5797 (99.21%) had bilateral auricles. Of the 4955 fetuses following systematic screening, all fetuses (100%) had bilateral auricles. The best time for fetal auricles observation with ultrasonography is 20–24 weeks of pregnancy. Conclusions. Detection of external ear abnormalities may assist in the diagnosis of chromosomal abnormalities. PMID:25050343

  3. Ear Infections and Language Development.

    ERIC Educational Resources Information Center

    Roberts, Joanne E.; Zeisel, Susan A.

    Ear infections in infants and preschoolers can cause mild or moderate temporary hearing loss, which may in turn affect a child's ability to understand and learn language. Noting that providing children with proper medical treatment for ear infections or middle ear fluid is important in preventing possible problems with language development, this…

  4. Nonsurgical correction of congenital ear abnormalities in the newborn: Case series

    PubMed Central

    Smith, WG; Toye, JW; Reid, A; Smith, RW

    2005-01-01

    OBJECTIVE To determine whether a simple, nonsurgical treatment for congenital ear abnormalities (lop-ear, Stahl’s ear, protruding ear, cryptotia) improved the appearance of ear abnormalities in newborns at six weeks of age. METHODS This is a descriptive case series. All newborns with identified abnormalities were referred by their family physician to one paediatrician (WGS) in a small level 2 perinatal centre. The ears were waxed and taped in a standard manner within 10 days of birth. Pictures were taken before taping and at the end of taping (one month). All patients and pictures were assessed by one plastic surgeon (JWT) at six weeks of age and scored using a standard scoring system. A telephone survey of the nontreatment group was conducted. RESULTS The total number of ears assessed was 90. Of this total, 69 ears were taped and fully evaluated in the study (77%). The refusal rate was 23%. In the treatment group, 59% had lop-ear, 19% had Stahl’s ear, 17% had protruding ear and 3% had cryptotia. Overall correction (excellent/improved) for the treatment group was 90% (100% for lop-ear, 100% for Stahl’s ear, 67% for protruding ear and 0% for cryptotia). In the nontreatment (refusal) group, 67% of the ears failed to correct spontaneously. No complications were recognized by the authors or parents by six weeks. The percentage of newborns in one year in the perinatal centre with recognized ear abnormalities was 6% (90 of 1600). CONCLUSIONS A simple, nonsurgical treatment in a Caucasian population appeared to be very effective in correcting congenital ear abnormalities with no complications and high patient/parent satisfaction. PMID:19675840

  5. Sp8 regulates inner ear development.

    PubMed

    Chung, Hyeyoung A; Medina-Ruiz, Sofia; Harland, Richard M

    2014-04-29

    A forward genetic screen of N-ethyl-N-nitrosourea mutagenized Xenopus tropicalis has identified an inner ear mutant named eclipse (ecl). Mutants developed enlarged otic vesicles and various defects of otoconia development; they also showed abnormal circular and inverted swimming patterns. Positional cloning identified specificity protein 8 (sp8), which was previously found to regulate limb and brain development. Two different loss-of-function approaches using transcription activator-like effector nucleases and morpholino oligonucleotides confirmed that the ecl mutant phenotype is caused by down-regulation of sp8. Depletion of sp8 resulted in otic dysmorphogenesis, such as uncompartmentalized and enlarged otic vesicles, epithelial dilation with abnormal sensory end organs. When overexpressed, sp8 was sufficient to induce ectopic otic vesicles possessing sensory hair cells, neurofilament innervation in a thickened sensory epithelium, and otoconia, all of which are found in the endogenous otic vesicle. We propose that sp8 is an important factor for initiation and elaboration of inner ear development.

  6. Sp8 regulates inner ear development.

    PubMed

    Chung, Hyeyoung A; Medina-Ruiz, Sofia; Harland, Richard M

    2014-04-29

    A forward genetic screen of N-ethyl-N-nitrosourea mutagenized Xenopus tropicalis has identified an inner ear mutant named eclipse (ecl). Mutants developed enlarged otic vesicles and various defects of otoconia development; they also showed abnormal circular and inverted swimming patterns. Positional cloning identified specificity protein 8 (sp8), which was previously found to regulate limb and brain development. Two different loss-of-function approaches using transcription activator-like effector nucleases and morpholino oligonucleotides confirmed that the ecl mutant phenotype is caused by down-regulation of sp8. Depletion of sp8 resulted in otic dysmorphogenesis, such as uncompartmentalized and enlarged otic vesicles, epithelial dilation with abnormal sensory end organs. When overexpressed, sp8 was sufficient to induce ectopic otic vesicles possessing sensory hair cells, neurofilament innervation in a thickened sensory epithelium, and otoconia, all of which are found in the endogenous otic vesicle. We propose that sp8 is an important factor for initiation and elaboration of inner ear development. PMID:24722637

  7. Can you hear me now? Understanding vertebrate middle ear development.

    PubMed

    Chapman, Susan Caroline

    2011-01-01

    The middle ear is a composite organ formed from all three germ layers and the neural crest. It provides the link between the outside world and the inner ear, where sound is transduced and routed to the brain for processing. Extensive classical and modern studies have described the complex morphology and origin of the middle ear. Non-mammalian vertebrates have a single ossicle, the columella. Mammals have three functionally equivalent ossicles, designated the malleus, incus and stapes. In this review, I focus on the role of genes known to function in the middle ear. Genetic studies are beginning to unravel the induction and patterning of the multiple middle ear elements including the tympanum, skeletal elements, the air-filled cavity, and the insertion point into the inner ear oval window. Future studies that elucidate the integrated spatio-temporal signaling mechanisms required to pattern the middle ear organ system are needed. The longer-term translational benefits of understanding normal and abnormal ear development will have a direct impact on human health outcomes.

  8. Can you hear me now? Understanding vertebrate middle ear development.

    PubMed

    Chapman, Susan Caroline

    2011-01-01

    The middle ear is a composite organ formed from all three germ layers and the neural crest. It provides the link between the outside world and the inner ear, where sound is transduced and routed to the brain for processing. Extensive classical and modern studies have described the complex morphology and origin of the middle ear. Non-mammalian vertebrates have a single ossicle, the columella. Mammals have three functionally equivalent ossicles, designated the malleus, incus and stapes. In this review, I focus on the role of genes known to function in the middle ear. Genetic studies are beginning to unravel the induction and patterning of the multiple middle ear elements including the tympanum, skeletal elements, the air-filled cavity, and the insertion point into the inner ear oval window. Future studies that elucidate the integrated spatio-temporal signaling mechanisms required to pattern the middle ear organ system are needed. The longer-term translational benefits of understanding normal and abnormal ear development will have a direct impact on human health outcomes. PMID:21196256

  9. Molecular Mechanisms of Inner Ear Development

    PubMed Central

    Wu, Doris K.; Kelley, Matthew W.

    2012-01-01

    The inner ear is a structurally complex vertebrate organ built to encode sound, motion, and orientation in space. Given its complexity, it is not surprising that inner ear dysfunction is a relatively common consequence of human genetic mutation. Studies in model organisms suggest that many genes currently known to be associated with human hearing impairment are active during embryogenesis. Hence, the study of inner ear development provides a rich context for understanding the functions of genes implicated in hearing loss. This chapter focuses on molecular mechanisms of inner ear development derived from studies of model organisms. PMID:22855724

  10. Development and Integration of the Ear.

    PubMed

    Fuchs, Jennifer C; Tucker, Abigail S

    2015-01-01

    The perception of our environment via sensory organs plays a crucial role in survival and evolution. Hearing, one of our most developed senses, depends on the proper function of the auditory system and plays a key role in social communication, integration, and learning ability. The ear is a composite structure, comprised of the external, middle, and inner ear. During development, the ear is formed from the integration of a number of tissues of different embryonic origin, which initiate in distinct areas of the embryo at different time points. Functional connections between the components of the hearing apparatus have to be established and maintained during development and adulthood to allow proper sound submission from the outer to the middle and inner ear. This highly organized and intimate connectivity depends on intricate spatiotemporal signaling between the various tissues that give rise to the structures of the ear. Any alterations in this chain of events can lead to the loss of integration, which can subsequently lead to conductive hearing loss, in case of outer and middle ear defects or sensorineural hearing loss, if inner ear structures are defective. This chapter aims to review the current knowledge concerning the development of the three ear compartments as well as mechanisms and signaling pathways that have been implicated in the coordination and integration process of the ear.

  11. Development and Integration of the Ear.

    PubMed

    Fuchs, Jennifer C; Tucker, Abigail S

    2015-01-01

    The perception of our environment via sensory organs plays a crucial role in survival and evolution. Hearing, one of our most developed senses, depends on the proper function of the auditory system and plays a key role in social communication, integration, and learning ability. The ear is a composite structure, comprised of the external, middle, and inner ear. During development, the ear is formed from the integration of a number of tissues of different embryonic origin, which initiate in distinct areas of the embryo at different time points. Functional connections between the components of the hearing apparatus have to be established and maintained during development and adulthood to allow proper sound submission from the outer to the middle and inner ear. This highly organized and intimate connectivity depends on intricate spatiotemporal signaling between the various tissues that give rise to the structures of the ear. Any alterations in this chain of events can lead to the loss of integration, which can subsequently lead to conductive hearing loss, in case of outer and middle ear defects or sensorineural hearing loss, if inner ear structures are defective. This chapter aims to review the current knowledge concerning the development of the three ear compartments as well as mechanisms and signaling pathways that have been implicated in the coordination and integration process of the ear. PMID:26589927

  12. Vitamin D receptor deficiency impairs inner ear development in zebrafish.

    PubMed

    Kwon, Hye-Joo

    2016-09-16

    The biological actions of vitamin D are largely mediated through binding to the vitamin D receptor (VDR), a member of the nuclear hormone receptor family, which regulates gene expression in a wide variety of tissues and cells. Mutations in VDR gene have been implicated in ear disorders (hearing loss and balance disorder) but the mechanisms are not well established. In this study, to investigate the role of VDR in inner ear development, morpholino-mediated gene knockdown approaches were used in zebrafish model system. Two paralogs for VDR, vdra and vdrb, have been identified in zebrafish. Knockdown of vdra had no effect on ear development, whereas knockdown of vdrb displayed morphological ear defects including smaller otic vesicles with malformed semicircular canals and abnormal otoliths. Loss-of-vdrb resulted in down-regulation of pre-otic markers, pax8 and pax2a, indicating impairment of otic induction. Furthermore, zebrafish embryos lacking vdrb produced fewer sensory hair cells in the ears and showed disruption of balance and motor coordination. These data reveal that VDR signaling plays an important role in ear development. PMID:27526995

  13. Evolution and development of the vertebrate ear

    NASA Technical Reports Server (NTRS)

    Fritzsch, B.; Beisel, K. W.

    2001-01-01

    This review outlines major aspects of development and evolution of the ear, specifically addressing issues of cell fate commitment and the emerging molecular governance of these decisions. Available data support the notion of homology of subsets of mechanosensors across phyla (proprioreceptive mechanosensory neurons in insects, hair cells in vertebrates). It is argued that this conservation is primarily related to the specific transducing environment needed to achieve mechanosensation. Achieving this requires highly conserved transcription factors that regulate the expression of the relevant structural genes for mechanosensory transduction. While conserved at the level of some cell fate assignment genes (atonal and its mammalian homologue), the ear has also radically reorganized its development by implementing genes used for cell fate assignment in other parts of the developing nervous systems (e.g., neurogenin 1) and by evolving novel sets of genes specifically associated with the novel formation of sensory neurons that contact hair cells (neurotrophins and their receptors). Numerous genes have been identified that regulate morphogenesis, but there is only one common feature that emerges at the moment: the ear appears to have co-opted genes from a large variety of other parts of the developing body (forebrain, limbs, kidneys) and establishes, in combination with existing transcription factors, an environment in which those genes govern novel, ear-related morphogenetic aspects. The ear thus represents a unique mix of highly conserved developmental elements combined with co-opted and newly evolved developmental elements.

  14. Patterning and cell fate in ear development.

    PubMed

    Alsina, Berta; Giraldez, Fernando; Pujades, Cristina

    2009-01-01

    The inner ear is a complex structure responsible for the senses of audition and balance in vertebrates. The ear is organised into different sense organs that are specialised to detect specific stimuli such as sound and linear or angular accelerations. The elementary sensory unit of the ear consists of hair cells, supporting cells, neurons and Schwann cells. Hair cells are the mechano-electrical transducing elements, and otic neurons convey information coded in electrical impulses to the brain. With the exception of the Schwann cells, all cellular elements of the inner ear derive from the otic placode. This is an ectodermal thickening that is specified in the head ectoderm adjacent to the caudal hindbrain. The complex organisation of the ear requires precise coupling of regional specification and cell fate decisions during development, i.e. specificity in defining particular spatial domains containing particular cell types. Those decisions are taken early in development and are the subject of this article. We review here recent work on: i) early patterning of the otic placode, ii) the role of neural tube signals in the patterning of the otic vesicle, and iii) the genes underlying cell fate determination of neurons and sensory hair cells.

  15. The development of the mammalian outer and middle ear.

    PubMed

    Anthwal, Neal; Thompson, Hannah

    2016-02-01

    The mammalian ear is a complex structure divided into three main parts: the outer; middle; and inner ear. These parts are formed from all three germ layers and neural crest cells, which have to integrate successfully in order to form a fully functioning organ of hearing. Any defect in development of the outer and middle ear leads to conductive hearing loss, while defects in the inner ear can lead to sensorineural hearing loss. This review focuses on the development of the parts of the ear involved with sound transduction into the inner ear, and the parts largely ignored in the world of hearing research: the outer and middle ear. The published data on the embryonic origin, signalling, genetic control, development and timing of the mammalian middle and outer ear are reviewed here along with new data showing the Eustachian tube cartilage is of dual embryonic origin. The embryonic origin of some of these structures has only recently been uncovered (Science, 339, 2013, 1453; Development, 140, 2013, 4386), while the molecular mechanisms controlling the growth, structure and integration of many outer and middle ear components are hardly known. The genetic analysis of outer and middle ear development is rather limited, with a small number of genes often affecting either more than one part of the ear or having only very small effects on development. This review therefore highlights the necessity for further research into the development of outer and middle ear structures, which will be important for the understanding and treatment of conductive hearing loss.

  16. Historical development of active middle ear implants.

    PubMed

    Carlson, Matthew L; Pelosi, Stanley; Haynes, David S

    2014-12-01

    Active middle ear implants (AMEIs) are sophisticated technologies designed to overcome many of the shortcomings of conventional hearing aids, including feedback, distortion, and occlusion effect. Three AMEIs are currently approved by the US Food and Drug Administration for implantation in patients with sensorineural hearing loss. In this article, the history of AMEI technologies is reviewed, individual component development is outlined, past and current implant systems are described, and design and implementation successes and dead ends are highlighted. Past and ongoing challenges facing AMEI development are reviewed.

  17. Mice Haploinsufficient for Ets1 and Fli1 Display Middle Ear Abnormalities and Model Aspects of Jacobsen Syndrome.

    PubMed

    Carpinelli, Marina R; Kruse, Elizabeth A; Arhatari, Benedicta D; Debrincat, Marlyse A; Ogier, Jacqueline M; Bories, Jean-Christophe; Kile, Benjamin T; Burt, Rachel A

    2015-07-01

    E26 transformation-specific 1 (ETS1) and friend leukemia integration 1 (FLI1) are members of the ETS family of transcription factors, of which there are 28 in humans. Both genes are hemizygous in Jacobsen syndrome, an 11q contiguous gene deletion disorder involving thrombocytopenia, facial dysmorphism, growth and mental retardation, malformation of the heart and other organs, and hearing impairment associated with recurrent ear infections. To determine whether any of these defects are because of hemizygosity for ETS1 and FLI1, we characterized the phenotype of mice heterozygous for mutant alleles of Ets1 and Fli1. Fli1(+/-) mice displayed mild thrombocytopenia, as did Ets1(+/-)Fli1(+/-) animals. Fli1(+/-) and Ets1(+/-)Fli1(+/-) mice also displayed craniofacial abnormalities, including a small middle ear cavity, short nasal bone, and malformed interface between the nasal bone process and cartilaginous nasal septum. They exhibited hearing impairment, otitis media, fusions of ossicles to the middle ear wall, and deformed stapes. Hearing impairment was more penetrant and stapes malformations were more severe in Ets1(+/-)Fli1(+/-) mice than in Fli1(+/-) mice, indicating partial functional redundancy of these transcription factors during auditory development. Our findings indicate that the short nose, otitis media, and hearing impairment in Jacobsen syndrome are likely because of hemizygosity for ETS1 and FLI1.

  18. Maternal diseases and risk of isolated ear abnormalities in their children.

    PubMed

    Paput, László; Czeizel, Andrew E; Bánhidy, Ferenc

    2011-09-01

    The objective of the study was to estimate the possible association of maternal diseases with the risk of isolated ear congenital abnormalities (IECA) including mainly microtia and anotia in their children. Incidence of acute and prevalence of chronic maternal diseases in the mothers with IECA and in the mothers of their matched controls and all controls without any defects, in addition in the mothers of malformed controls with other isolated defects was compared in the population-based large dataset of the Hungarian Case-Control Surveillance of Congenital Abnormalities. Of 354 cases with IECA, 32 had mothers with high fever related influenza-common cold during the critical period of IECA and it resulted in a higher risk of IECA in their live-born infants (adjusted OR with 95% CI: 4.3, 1.9-7.4) compared with their matched controls. However, the early and effective antifever treatment in these pregnant women prevented them from the IECA. In addition there was an association of maternal otitis media with higher risk of complex defects of middle and external ears (OR with 95% CI: 5.2, 1.6-28.3), however, this association was based on 4 cases only. In conclusion high fever related influenza-common cold with secondary complications may play a role in the origin of IECA, but is preventable with the early and effective antifever treatment of these pregnant women.

  19. The development of the middle ear in neonatal chinchillas II. Two weeks to adulthood.

    PubMed

    Hsu, R W; Margolis, R H; Schachern, P A; Javel, E

    2001-09-01

    Studies of auditory function in the human neonate indicate adult-like hearing sensitivity, mature cochlear function and well-developed responses in the auditory pathway. Paradoxically, measurements of middle ear function are characterized by responses that would be interpreted as abnormal in older subjects. Consequently, there is not an accepted clinical test for middle ear disease in the newborn population. Like human neonates, chinchillas have normal hearing sensitivity at birth, but middle ear function tested by multifrequency tympanometry is abnormal compared to the adult. A previous study from our laboratory indicated that the newborn chinchilla middle ear is free of mesenchyme and other debris. Over the first 2 weeks of life there were no significant changes in tympanic membrane thickness and diameter, tympanic membrane to promontory distance and stapes footplate length. There were small changes in mastoid bulla area and perimeter and in mastoid bulla bone thickness. The most striking difference between the newborn and adult temporal bone was in bone composition, the newborn bone having a less dense, spongy appearance. Impedance characteristics of the newborn chinchilla ear, measured by multifrequency tympanometry, were abnormal relative to adult animals and did not change over the first 2 weeks of life. This investigation is an extension of the previous study, designed to better understand the relationship between middle ear function, hearing sensitivity and the structural changes of the newborn chinchilla middle ear. Twenty animals, aged 2-8 weeks, were studied. Additional adult animals were used as controls. Middle ear function was assessed by a wideband reflectance impedance system. Hearing sensitivity was measured by auditory brainstem response in 2- and 8-week-old animals. Structural characteristics of the temporal bone were analyzed using histopathologic preparations. There was an orderly progression in middle ear impedance and reflectance

  20. The maize rachis affects Aspergillus flavus movement during ear development

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Aspergillus flavus expressing green fluorescent protein (GFP) was used to follow infection in ears of maize hybrids resistant and susceptible to the fungus. Developing ears were needle-inoculated with GFP-transformed A. flavus 20 days after silk emergence, and GFP fluorescence in the pith was evalu...

  1. A de novo microdeletion in a patient with inner ear abnormalities suggests that the 10q26.13 region contains the responsible gene

    PubMed Central

    Sangu, Noriko; Okamoto, Nobuhiko; Shimojima, Keiko; Ondo, Yumiko; Nishikawa, Masanori; Yamamoto, Toshiyuki

    2016-01-01

    Microdeletions in the 10q26.1 region are related to intellectual disability, growth delay, microcephaly, distinctive craniofacial features, cardiac defects, genital abnormalities and inner ear abnormalities. The genes responsible for inner ear abnormalities have been narrowed to fibroblast growth factor receptor 2 gene (FGFR2), H6 family homeobox 2 gene (HMX2) and H6 family homeobox 3 gene (HMX3). An additional patient with distinctive craniofacial features, congenital deafness and balance dysfunctions showed a de novo microdeletion of 10q26.11q26.13, indicating the existence of a gene responsible for inner ear abnormalities in this region. PMID:27274859

  2. Spatiotemporal patterns of Musashi1 expression during inner ear development.

    PubMed

    Sakaguchi, Hirofumi; Yaoi, Takeshi; Suzuki, Toshihiro; Okano, Hideyuki; Hisa, Yasuo; Fushiki, Shinji

    2004-04-29

    Musashi1 (Msi 1) is an RNA binding protein associated with asymmetric cell divisions in neural progenitor cells. To investigate the involvement of Msi1 in the inner ear development, we studied the expression of Msi1 in mouse inner ears with RT-PCR and immunohistochemistry. Immunohistochemistry revealed that Msi1 was expressed in all otocyst cells at embryonic day (E) 10 and 12. Msi1 immunoreactivity became lost in hair cells after E14 in vestibule and after E16 in cochlea, whereas it persisted in supporting cells until adulthood. The subcellular localization of Msi1 changed from "cytoplasmic predominance" to "nuclear predominance" during the first 2 weeks after birth. The present data suggested that Msi may play a role in inner ear development. PMID:15076722

  3. Development of optoelectronic monitoring system for ear arterial pressure waveforms

    NASA Astrophysics Data System (ADS)

    Sasayama, Satoshi; Imachi, Yu; Yagi, Tamotsu; Imachi, Kou; Ono, Toshirou; Man-i, Masando

    1994-02-01

    Invasive intra-arterial blood pressure measurement is the most accurate method but not practical if the subject is in motion. The apparatus developed by Wesseling et al., based on a volume-clamp method of Penaz (Finapres), is able to monitor continuous finger arterial pressure waveforms noninvasively. The limitation of Finapres is the difficulty in measuring the pressure of a subject during work that involves finger or arm action. Because the Finapres detector is attached to subject's finger, the measurements are affected by inertia of blood and hydrostatic effect cause by arm or finger motion. To overcome this problem, the authors made a detector that is attached to subject's ear and developed and optoelectronic monitoring systems for ear arterial pressure waveform (Earpres). An IR LEDs, photodiode, and air cuff comprised the detector. The detector was attached to a subject's ear, and the space adjusted between the air cuff and the rubber plate on which the LED and photodiode were positioned. To evaluate the accuracy of Earpres, the following tests were conducted with participation of 10 healthy male volunteers. The subjects rested for about five minutes, then performed standing and squatting exercises to provide wide ranges of systolic and diastolic arterial pressure. Intra- and inter-individual standard errors were calculated according to the method of van Egmond et al. As a result, average, the averages of intra-individual standard errors for earpres appeared small (3.7 and 2.7 mmHg for systolic and diastolic pressure respectively). The inter-individual standard errors for Earpres were about the same was Finapres for both systolic and diastolic pressure. The results showed the ear monitor was reliable in measuring arterial blood pressure waveforms and might be applicable to various fields such as sports medicine and ergonomics.

  4. Development of calretinin immunoreactivity in the mouse inner ear.

    PubMed

    Dechesne, C J; Rabejac, D; Desmadryl, G

    1994-08-22

    Calretinin is a calcium-binding protein of the EF-hand family. It has been previously identified in particular cell types of adult guinea pig, rat, and chinchilla inner ear. Development of calretinin immunoreactivity in the mouse inner ear was investigated from embryonic day 13 (E13) to the adult stage. In the adult mouse vestibule, calretinin immunoreactivity was present in the same structures as described for the rat and guinea pig: the population of afferent fibers forming calyx units and a small number of ganglion neurons. The earliest immunoreactivity was found at E17 in vestibular hair cells (VHCs), then, at E19, in afferent fibers entering the sensory epithelia and in rare ganglion neurons. At postnatal day 4 (P4), a few vestibular nerve fibers and ganglion neurons were reactive. From this stage until P14, immunoreactivity developed in the calyx units and disappeared from VHCs. At P14, immunostaining was adult-like. In the adult mouse cochlea, immunoreactivity was present in the same cell populations as described in the rat: the inner hair cells (IHCs) and most of Corti's ganglion neurons. Calretinin immunoreactivity appeared at E19-P0 in IHCs and ganglion neurons of the basal turn. At P1, outer hair cells (OHCs) of the basal turn were positive. Calretinin immunoreactivity then appeared in IHCs, OHCs, and ganglion neurons of the medial turn, then of the apical turn. At P4, all IHCs and OHCs and most of the ganglion neurons were immunostained. Immunoreactivity gradually disappeared from the OHCs starting at P10 and, at P22, only IHCs and ganglion neurons were positive. The sequences of appearance of calretinin were specific to each cell type of the inner ear and paralleled their respective maturation. Calretinin was transiently expressed in VHCs and OHCs. PMID:7983242

  5. Histone deacetylase 1 is required for the development of the zebrafish inner ear

    PubMed Central

    He, Yingzi; Tang, Dongmei; Li, Wenyan; Chai, Renjie; Li, Huawei

    2016-01-01

    Histone deacetylase 1 (HDAC1) has been reported to be important for multiple aspects of normal embryonic development, but little is known about its function in the development of mechanosensory organs. Here, we first confirmed that HDAC1 is expressed in the developing otic vesicles of zebrafish by whole-mount in situ hybridization. Knockdown of HDAC1 using antisense morpholino oligonucleotides in zebrafish embryos induced smaller otic vesicles, abnormal otoliths, malformed or absent semicircular canals, and fewer sensory hair cells. HDAC1 loss of function also caused attenuated expression of a subset of key genes required for otic vesicle formation during development. Morpholino-mediated knockdown of HDAC1 resulted in decreased expression of members of the Fgf family in the otic vesicles, suggesting that HDAC1 is involved in the development of the inner ear through regulation of Fgf signaling pathways. Taken together, our results indicate that HDAC1 plays an important role in otic vesicle formation. PMID:26832938

  6. Gene Expression by Mouse Inner Ear Hair Cells during Development

    PubMed Central

    Scheffer, Déborah I.; Shen, Jun

    2015-01-01

    Hair cells of the inner ear are essential for hearing and balance. As a consequence, pathogenic variants in genes specifically expressed in hair cells often cause hereditary deafness. Hair cells are few in number and not easily isolated from the adjacent supporting cells, so the biochemistry and molecular biology of hair cells can be difficult to study. To study gene expression in hair cells, we developed a protocol for hair cell isolation by FACS. With nearly pure hair cells and surrounding cells, from cochlea and utricle and from E16 to P7, we performed a comprehensive cell type-specific RNA-Seq study of gene expression during mouse inner ear development. Expression profiling revealed new hair cell genes with distinct expression patterns: some are specific for vestibular hair cells, others for cochlear hair cells, and some are expressed just before or after maturation of mechanosensitivity. We found that many of the known hereditary deafness genes are much more highly expressed in hair cells than surrounding cells, suggesting that genes preferentially expressed in hair cells are good candidates for unknown deafness genes. PMID:25904789

  7. Mechanistic basis of otolith formation during teleost inner ear development.

    PubMed

    Wu, David; Freund, Jonathan B; Fraser, Scott E; Vermot, Julien

    2011-02-15

    Otoliths, which are connected to stereociliary bundles in the inner ear, serve as inertial sensors for balance. In teleostei, otolith development is critically dependent on flow forces generated by beating cilia; however, the mechanism by which flow controls otolith formation remains unclear. Here, we have developed a noninvasive flow probe using optical tweezers and a viscous flow model in order to demonstrate how the observed hydrodynamics influence otolith assembly. We show that rotational flow stirs and suppresses precursor agglomeration in the core of the cilia-driven vortex. The velocity field correlates with the shape of the otolith and we provide evidence that hydrodynamics is actively involved in controlling otolith morphogenesis. An implication of this hydrodynamic effect is that otolith self-assembly is mediated by the balance between Brownian motion and cilia-driven flow. More generally, this flow feature highlights an alternative biological strategy for controlling particle localization in solution.

  8. Mechanistic basis of otolith formation during teleost inner ear development

    PubMed Central

    Wu, David; Freund, Jonathan B.; Fraser, Scott E.; Vermot, Julien

    2011-01-01

    Otoliths, which are connected to stereociliary bundles in the inner ear, serve as inertial sensors for balance. In teleostei, otolith development is critically dependant on flow forces generated by beating cilia; however, the mechanism by which flow controls otolith formation remains unclear. Here, we have developed a non-invasive flow probe using optical tweezers and a viscous flow model in order to demonstrate how the observed hydrodynamics influence otolith assembly. We show that rotational flow stirs and suppresses precursor agglomeration in the core of the cilia-driven vortex. The velocity field correlates with the shape of the otolith and we provide evidence that hydrodynamics is actively involved in controlling otolith morphogenesis. An implication of this hydrodynamic effect is that otolith self-assembly is mediated by the balance between Brownian motion and cilia-driven flow. More generally, this flow feature highlights an alternative biological strategy for controlling particle localization in solution. PMID:21316594

  9. Development of Structure and Sensitivity of the Fish Inner Ear.

    PubMed

    Vasconcelos, Raquel O; Alderks, Peter W; Sisneros, Joseph A

    2016-01-01

    Fish represent the largest group of vertebrates and display the greatest diversity of auditory structures. However, studies addressing how the form and function of the auditory system change during development to enhance perception of the acoustic environment are rather sparse in this taxon compared to other vertebrate groups. An ontogenetic perspective of the auditory system in fishes provides a readily testable framework for understanding structure-function relationships. Additionally, studying ancestral models such as fish can convey valuable comparable information across vertebrates, as early developmental events are often evolutionary conserved. This chapter reviews the literature on the morphological development of the fish auditory system, with particular focus on the inner ear structures that evolve from an otic placode during early embryonic development and then continue to undergo differentiation and maturation in the postembryonic phase. Moreover, the chapter provides a systematic overview of how auditory sensitivity develops during ontogeny. Although most studies indicate a developmental improvement in auditory sensitivity, there is considerably species-specific variation. Lastly, the paucity of information and literature concerning the development of auditory capabilities for social communication in fishes is also discussed. Further investigation on the development of structure and function of the fish auditory system is recommended in order to obtain a deeper understanding of how ontogenetic morphological changes in the auditory pathway relate to modifications in acoustic reception, auditory processing, and the capacity to communicate acoustically.

  10. Clonal analysis of corn plant development. I. The development of the tassel and the ear shoot

    SciTech Connect

    Johri, M.M.; Coe, E.H. Jr.

    1983-05-01

    The development of the tassel and the ear shoot has been investigated in corn (Zea mays L.). X irradiation of dry kernels and seedlings heterozygous for anthocyanin markers or for factors altering tassel and ear morphology results in the formation of clones (sectors) from cells of the apical meristem. Most tassels develop from 4 +/- 1 cells of the mature embryo. The expression of ramosa-1, tunicate, tassel seed-6, and vestigial is cell autonomous in the tassel. These genes act late in development and modify the developmental fate or decision of an individual clone and not of the whole group of cells producing a tassel. The ear shoot develops from lineages of one to three cells derived each from the L-I (outmost cell layer) and L-II (second cell layer) of the apical meristem. Typically the clones start in the ear shoot (in the husks and possibly in the cob), extend upward in an internode, continue along the margin of the leaf sheath or leaf blade at the node above, and terminate in this or the next higher leaf. The separation of lineages for ear shoot and internode occurs in the period around 13 days after sowing. The analysis of clonal boundaries shows that a small number of embryonic cells become isolated in their developmental capacity. This commitment process appears to be analogous to the process of compartmentation in the imaginal disks of fruit flies. The extent of proliferation of individual cells within a group of highly flexible and any particular clone does not generate a specific part of a tassel or an ear shoot. There must be cellular communication between various clones so that the overall size and morphology of an organ remain normal and more or less fixed. Thus the process of development in plants is also highly regulative in nature and shares many features in common with development in fruit flies.

  11. Modulation of Wnt Signaling Enhances Inner Ear Organoid Development in 3D Culture.

    PubMed

    DeJonge, Rachel E; Liu, Xiao-Ping; Deig, Christopher R; Heller, Stefan; Koehler, Karl R; Hashino, Eri

    2016-01-01

    Stem cell-derived inner ear sensory epithelia are a promising source of tissues for treating patients with hearing loss and dizziness. We recently demonstrated how to generate inner ear sensory epithelia, designated as inner ear organoids, from mouse embryonic stem cells (ESCs) in a self-organizing 3D culture. Here we improve the efficiency of this culture system by elucidating how Wnt signaling activity can drive the induction of otic tissue. We found that a carefully timed treatment with the potent Wnt agonist CHIR99021 promotes induction of otic vesicles-a process that was previously self-organized by unknown mechanisms. The resulting otic-like vesicles have a larger lumen size and contain a greater number of Pax8/Pax2-positive otic progenitor cells than organoids derived without the Wnt agonist. Additionally, these otic-like vesicles give rise to large inner ear organoids with hair cells whose morphological, biochemical and functional properties are indistinguishable from those of vestibular hair cells in the postnatal mouse inner ear. We conclude that Wnt signaling plays a similar role during inner ear organoid formation as it does during inner ear development in the embryo. PMID:27607106

  12. Modulation of Wnt Signaling Enhances Inner Ear Organoid Development in 3D Culture

    PubMed Central

    DeJonge, Rachel E.; Liu, Xiao-Ping; Deig, Christopher R.; Heller, Stefan; Koehler, Karl R.; Hashino, Eri

    2016-01-01

    Stem cell-derived inner ear sensory epithelia are a promising source of tissues for treating patients with hearing loss and dizziness. We recently demonstrated how to generate inner ear sensory epithelia, designated as inner ear organoids, from mouse embryonic stem cells (ESCs) in a self-organizing 3D culture. Here we improve the efficiency of this culture system by elucidating how Wnt signaling activity can drive the induction of otic tissue. We found that a carefully timed treatment with the potent Wnt agonist CHIR99021 promotes induction of otic vesicles—a process that was previously self-organized by unknown mechanisms. The resulting otic-like vesicles have a larger lumen size and contain a greater number of Pax8/Pax2-positive otic progenitor cells than organoids derived without the Wnt agonist. Additionally, these otic-like vesicles give rise to large inner ear organoids with hair cells whose morphological, biochemical and functional properties are indistinguishable from those of vestibular hair cells in the postnatal mouse inner ear. We conclude that Wnt signaling plays a similar role during inner ear organoid formation as it does during inner ear development in the embryo. PMID:27607106

  13. Inner Ear Conductive Hearing Loss and Unilateral Pulsatile Tinnitus Associated with a Dural Arteriovenous Fistula: Case Based Review and Analysis of Relationship between Intracranial Vascular Abnormalities and Inner Ear Fluids

    PubMed Central

    Cassandro, Ettore; Cassandro, Claudia; Sequino, Giuliano; Scarpa, Alfonso; Petrolo, Claudio; Chiarella, Giuseppe

    2015-01-01

    While pulsatile tinnitus (PT) and dural arteriovenous fistula (DAVF) are not rarely associated, the finding of a conductive hearing loss (CHL) in this clinical picture is unusual. Starting from a case of CHL and PT, diagnosed to be due to a DAVF, we analyzed relationship between intracranial vascular abnormalities and inner ear fluids. DAVF was treated with endovascular embolization. Following this, there was a dramatic recovery of PT and of CHL, confirming their cause-effect link with DAVF. We critically evaluated the papers reporting this association. This is the first case of CHL associated with PT and DAVF. We describe the most significant experiences and theories reported in literature, with a personal analysis about the possible relationship between vascular intracranial system and labyrinthine fluids. In conclusion, we believe that this association may be a challenge for otolaryngologists. So we suggest to consider the possibility of a DAVF or other AVMs when PT is associated with CHL, without alterations of tympanic membrane and middle ear tests. PMID:26693371

  14. Electroporation-Mediated Gene Transfer to the Developing Mouse Inner Ear

    PubMed Central

    Brigande, John V.; Gubbels, Samuel P.; Woessner, David W.; Jungwirth, Jonathan J.; Bresee, Catherine S.

    2010-01-01

    The mammalian inner ear forms from a thickened patch of head ectoderm called the otic placode. The placodal ectoderm invaginates to form a cup whose edges cinch together to establish a fluid-filled sac called the otic vesicle or otocyst. The progenitor cells lining the otocyst lumen will give rise to sensory and non-sensory cells of the inner ear. These formative stages of inner ear development are initiated during the first week of postimplantation embryonic development in the mouse. The inaccessibility of the inner ear in utero has hampered efforts to gain insight into the molecular mechanisms regulating essential developmental processes. An experimental embryological method to misexpress genes in the developing mammalian inner ear is presented. Expression plasmid encoding a gene of interest is microinjected through the uterine wall into the lumen of the otocyst and electroporated into otic epithelial progenitor cells. Downstream analysis of the transfected embryonic or postnatal inner ear is then conducted to gain insight into gene function. PMID:18839345

  15. Development of a Temporal Bone Model for Transcanal Endoscopic Ear Surgery.

    PubMed

    Dedmon, Matthew M; Kozin, Elliott D; Lee, Daniel J

    2015-10-01

    Transcanal endoscopic ear surgery (TEES) is being increasingly used in chronic ear disease for cholesteatoma removal and middle ear reconstruction, reducing the need for a postauricular incision and mastoidectomy. However, TEES is a challenging technique even for the most experienced otologist, requiring one-handed dissection using angled instrumentation. We have therefore developed a high-fidelity dissection model incorporating key aspects of TEES and cholesteatoma removal to facilitate the acquisition of these skills. Artificial cholesteatoma was implanted into middle ear spaces of a human temporal bone via a facial recess approach. A pilot study was conducted whereby surgeons endoscopically elevated a tympanomeatal flap with artificial bleeding and removed artificial cholesteatoma with angled instrumentation. Surgeons were uniformly satisfied with the experience and felt it would translate into improved performance in the operating room. This study suggests that the TEES dissection model could become an integral tool in the training of emerging TEES techniques.

  16. Pierced Ears

    MedlinePlus

    ... Homework? Here's Help White House Lunch Recipes Pierced Ears KidsHealth > For Kids > Pierced Ears Print A A ... cool, but infected ears do not! Getting Your Ears Pierced It's important to get your ears pierced ...

  17. The molecular basis of neurosensory cell formation in ear development: a blueprint for hair cell and sensory neuron regeneration?

    PubMed Central

    Fritzsch, Bernd; Beisel, Kirk W.; Hansen, Laura

    2014-01-01

    Summary The inner ear of mammals uses neurosensory cells derived from the embryonic ear for mechanoelectric transduction of vestibular and auditory stimuli (the hair cells) and conducts this information to the brain via sensory neurons. As with most other neurons of mammals, lost hair cells and sensory neurons are not spontaneously replaced and result instead in age-dependent progressive hearing loss. We review the molecular basis of neurosensory development in the mouse ear to provide a blueprint for possible enhancement of therapeutically useful transformation of stem cells into lost neurosensory cells. We identify several readily available adult sources of stem cells that express, like the ectoderm-derived ear, genes known to be essential for ear development. Use of these stem cells combined with molecular insights into neurosensory cell specification and proliferation regulation of the ear, might allow for neurosensory regeneration of mammalian ears in the near future. PMID:17120192

  18. Segregating neural and mechanosensory fates in the developing ear: patterning, signaling, and transcriptional control.

    PubMed

    Raft, Steven; Groves, Andrew K

    2015-01-01

    The vertebrate inner ear is composed of multiple sensory receptor epithelia, each of which is specialized for detection of sound, gravity, or angular acceleration. Each receptor epithelium contains mechanosensitive hair cells, which are connected to the brainstem by bipolar sensory neurons. Hair cells and their associated neurons are derived from the embryonic rudiment of the inner ear epithelium, but the precise spatial and temporal patterns of their generation, as well as the signals that coordinate these events, have only recently begun to be understood. Gene expression, lineage tracing, and mutant analyses suggest that both neurons and hair cells are generated from a common domain of neural and sensory competence in the embryonic inner ear rudiment. Members of the Shh, Wnt, and FGF families, together with retinoic acid signals, regulate transcription factor genes within the inner ear rudiment to establish the axial identity of the ear and regionalize neurogenic activity. Close-range signaling, such as that of the Notch pathway, specifies the fate of sensory regions and individual cell types. We also describe positive and negative interactions between basic helix-loop-helix and SoxB family transcription factors that specify either neuronal or sensory fates in a context-dependent manner. Finally, we review recent work on inner ear development in zebrafish, which demonstrates that the relative timing of neurogenesis and sensory epithelial formation is not phylogenetically constrained.

  19. Early steps in inner ear development: induction and morphogenesis of the otic placode

    PubMed Central

    Sai, Xiaorei; Ladher, Raj K.

    2015-01-01

    Various cellular replacement therapies using in vitro generated cells to replace damaged tissue have been proposed as strategies to alleviate hearing loss. All such therapies must involve a complete understanding of the earliest steps in inner ear development; its induction as a thickened plate of cells in the non-neural, surface ectoderm of the embryo, to its internalization as an otocyst embedded in the head mesenchyme of the embryo. Such knowledge informs researchers addressing the feasibility of the proposed strategy and present alternatives if needed. In this review we describe the mechanisms of inner ear induction, concentrating on the factors that steer the fate of ectoderm into precursors of the inner ear. Induction then leads to inner ear morphogenesis and we describe the cellular changes that occur as the inner ear is converted from a superficial placode to an internalized otocyst, and how they are coordinated with a particular emphasis on how the signaling environment surrounding the inner ear influences these processes. PMID:25713536

  20. Sonographic Measurement of Fetal Ear Length in Turkish Women with a Normal Pregnancy

    PubMed Central

    Özdemir, Mucize Eriç; Uzun, Işıl; Karahasanoğlu, Ayşe; Aygün, Mehmet; Akın, Hale; Yazıcıoğlu, Fehmi

    2014-01-01

    Background: Abnormal fetal ear length is a feature of chromosomal disorders. Fetal ear length measurement is a simple measurement that can be obtained during ultrasonographic examinations. Aims: To develop a nomogram for fetal ear length measurements in our population and investigate the correlation between fetal ear length, gestational age, and other standard fetal biometric measurements. Study Design: Cohort study. Methods: Ear lengths of the fetuses were measured in normal singleton pregnancies. The relationship between gestational age and fetal ear length in millimetres was analysed by simple linear regression. In addition, the correlation of fetal ear length measurements with biparietal diameter, head circumference, abdominal circumference, and femur length were evaluated.Ear length measurements were obtained from fetuses in 389 normal singleton pregnancies ranging between 16 and 28 weeks of gestation. Results: A nomogram was developed by linear regression analysis of the parameters ear length and gestational age. Fetal ear length (mm) = y = (1.348 X gestational age)−12.265), where gestational ages is in weeks. A high correlation was found between fetal ear length and gestational age, and a significant correlation was also found between fetal ear length and the biparietal diameter (r=0.962; p<0.001). Similar correlations were found between fetal ear length and head circumference, and fetal ear length and femur length. Conclusion: The results of this study provide a nomogram for fetal ear length. The study also demonstrates the relationship between ear length and other biometric measurements. PMID:25667783

  1. Epigenetic regulation in the inner ear and its potential roles in development, protection, and regeneration

    PubMed Central

    Layman, Wanda S.; Zuo, Jian

    2014-01-01

    The burgeoning field of epigenetics is beginning to make a significant impact on our understanding of tissue development, maintenance, and function. Epigenetic mechanisms regulate the structure and activity of the genome in response to intracellular and environmental cues that direct cell-type specific gene networks. The inner ear is comprised of highly specialized cell types with identical genomes that originate from a single totipotent zygote. During inner ear development specific combinations of transcription factors and epigenetic modifiers must function in a coordinated manner to establish and maintain cellular identity. These epigenetic regulatory mechanisms contribute to the maintenance of distinct chromatin states and cell-type specific gene expression patterns. In this review, we highlight emerging paradigms for epigenetic modifications related to inner ear development, and how epigenetics may have a significant role in hearing loss, protection, and regeneration. PMID:25750614

  2. Swimmer's Ear

    MedlinePlus

    ... Homework? Here's Help White House Lunch Recipes Swimmer's Ear KidsHealth > For Kids > Swimmer's Ear Print A A ... How Do I Know if I Have Swimmer's Ear? Swimmer's ear may start with some itching, but ...

  3. Ear Tubes

    MedlinePlus

    ... Meeting Calendar Find an ENT Doctor Near You Ear Tubes Ear Tubes Patient Health Information News media ... and throat specialist) may be considered. What are ear tubes? Ear tubes are tiny cylinders placed through ...

  4. Ear Playing and Aural Development in the Instrumental Lesson: Results from a "Case-Control" Experiment

    ERIC Educational Resources Information Center

    Baker, David; Green, Lucy

    2013-01-01

    This article reports on a case-control experiment that was conducted in 2012 as part of the Ear Playing Project (EPP) at the Institute of Education, University of London. The EPP developed from the "informal learning" strand of Musical Futures and engaged instrumental students in the UK in learning from specially-created audio recordings…

  5. Fetal development of the elastic-fiber-mediated enthesis in the human middle ear.

    PubMed

    Takanashi, Yoshitaka; Shibata, Shunichi; Katori, Yukio; Murakami, Gen; Abe, Shinichi; Rodríguez-Vázquez, Jose Francisco; Kawase, Tetsuaki

    2013-10-01

    In the human middle ear, the annular ligament of the incudostapedial joint and the insertions of the tensor tympani and stapedius muscles contain abundant elastic fibers; i.e., the elastic-fiber-mediated entheses. Hyaluronan also coexists with the elastic fibers. In the present study using immunohistochemistry, we demonstrated the distribution of elastin not only in the incudostapedial joint but also in the other two joints of the middle ear in adults and fetuses. In adults, the expression of elastin did not extend out of the annular ligament composed of mature elastic fibers but clearly overlapped with it. Electron microscopic observations of the annular ligament demonstrated a few microfibrils along the elastic fibers. Thus, in contrast to the vocal cord, the middle ear entheses seemed not to contain elaunin and oxytalan fibers. In mid-term fetuses (at approximately 15-16 weeks of gestation) before opening of the external acoustic meatus, the incudostapedial joint showed abundant elastic fibers, but the incudomalleolar and stapediovestibular joints did not. At this stage, hyaluronan was not colocalized, but distributed diffusely in loose mesenchymal tissues surrounding the ear ossicles. Therefore, fetal development of elastin and elastic fibers in the middle ear entheses is unlikely to require acoustic oscillation. In late-stage fetuses (25-30 weeks), whose ear ossicles were almost the same size as those in adults, we observed bundling and branching of elastic fibers. However, hyaluronan expression was not as strong as in adults. Colocalization between elastic fibers and hyaluronan appeared to be a result of postnatal maturation of the entheses.

  6. The anatomy and development of normal and abnormal coronary arteries.

    PubMed

    Spicer, Diane E; Henderson, Deborah J; Chaudhry, Bill; Mohun, Timothy J; Anderson, Robert H

    2015-12-01

    At present, there is significant interest in the morphology of the coronary arteries, not least due to the increasingly well-recognised association between anomalous origin of the arteries and sudden cardiac death. Much has also been learnt over the last decade regarding the embryology of the arteries. In this review, therefore, we provide a brief introduction into the recent findings regarding their development. In particular, we emphasise that new evidence, derived using the developing murine heart, points to the arterial stems growing out from the adjacent sinuses of the aortic root, rather than the arteries growing in, as is currently assumed. As we show, the concept of outgrowth provides an excellent explanation for several of the abnormal arrangements encountered in the clinical setting. Before summarising these abnormal features, we draw attention to the need to describe the heart in an attitudinally appropriate manner, following the basic rule of human anatomy, rather than describing the cardiac components with the heart in the "Valentine" orientation. We then show how the major abnormalities involving the coronary arteries in humans can be summarised in terms of abnormal origin from the pulmonary circulation, abnormal aortic origin, or fistulous communications between the coronary arteries and the cardiac cavities. In the case of abnormal aortic origin, we highlight those malformations known to be associated with sudden cardiac death.

  7. Raldh3 gene expression pattern in the developing chicken inner ear.

    PubMed

    Sánchez-Guardado, Luis Oscar; Ferran, José Luis; Mijares, José; Puelles, Luis; Rodríguez-Gallardo, Lucía; Hidalgo-Sánchez, Matías

    2009-05-01

    Retinoic acid (RA), an active metabolite of vitamin A, is a diffusible molecule that regulates the expression of several families of genes, playing a key role in specification processes during chordate development. With the aim of defining its possible role in the developing chick inner ear, we obtained in this work a detailed spatiotemporal distribution of the enzymes involved in its synthesis, the retinaldehyde dehydrogenases (RALH1-4). Our results showed that, in contrast to the mouse inner ear, Raldh3 expression was the only Raldh gene detected in the developing chick inner ear, where it appears as early as stage 18. During inner ear morphogenesis, Raldh3 expression was predominantly observed in the endolymphatic system. The Raldh3 expression pattern delimited totally or partially the Bmp4-positive presumptive territories of vestibular sensory epithelia by stage 24 and the basilar papilla at stage 34, suggesting a possible involvement of RA in their specification. In addition, several vestibular sensory areas showed some Raldh3-expressing cells close to the Raldh3-positive domain. These results suggest that the RA signaling pathway may play a role in the initial patterning of the otic epithelium and cell differentiation therein, providing local positional information. Having in mind this Raldh3 expression pattern, we discuss the regulatory interactions among the RA, bone morphogenetic protein, and fibroblast growth factor signaling pathways in the specification of otic sensory elements. Our investigation may underpin further experimental studies aimed at understanding the possible role of signaling pathways in patterning of the developing chick inner ear.

  8. [Development of one corn ear and verification the three laws of inheritance].

    PubMed

    Zhao, Wen-Yao; Liu, Jun-Bo; Zhao, Liang

    2008-09-01

    A new corn was developed as a lab material to verify the three Laws of Inheritance on only one F2 ear with correct segregation ratios of kernel traits. In order to investigate the three Inheritance Laws simultaneously with one ear, the sweet endosperm (Sn1), kernel color (C1) and the glutinous endosperm (Wx) were chosen as the original phenotypes. Gene Sn1sn1 is located on chromosome four, genes C(1c1) and Wxwx are located on chromosome nine. After several generations of continuous self-pollination and test crossing, direct crossing and back crossing, with strict selections, the homozygous parents were obtained. With different combinations of homozygous parents, different hybrids were developed. The segregation ratios of each phenotype on the same F2 ear are compared to the theoretical segregation ratios according to the corn gene exchange and gamete types and quantities produced through meiosis. The results showed three ratios of 3:1, two ratios of 9:3:3:1, and one ratio of linkage and exchange, with all the chi-square values smaller than the permitted ones. This means that only one ear of this new hybrid corn can be used as a new teaching aid to examine and verify the three Laws of Inheritance.

  9. Development of computer assisted orthosis design and manufacturing system for malformed ears.

    PubMed

    Hanafusa, A; Takahashi, H; Akagi, K; Isomura, T

    1997-01-01

    Most cases of malformed ears in neonates can be treated by mounting a suitably shaped orthosis. Our objective was to develop a computer-assisted orthosis design and manufacturing system for the treatment of malformed ears. Also, we aimed to manufacture a first experimental orthosis made of nitinol shape-memory alloy wire. First, the target posttherapeutic auricular shape is input from computed tomography scans, and characteristic points and contours are extracted. The shape of the orthosis is then created by tracing the necessary contours automatically. Finally, the orthosis shape data are projected onto one or more approximate planes and autoconverted into numerical control (NC) data for plate groove processing. The nitinol shape-memory alloy wires are inserted in the groove and the shape is memorized by heat treatment. The shape of the manufactured orthosis can be memorized from the generated NC data within 1.0 mm of error. Stahl's ear in a 9-month-old baby was treated 7 months ago by mounting the orthosis manufactured by the system. The developed system allows the design and manufacture of orthoses made of nitinol shape-memory alloy wire for the treatment of malformed ears.

  10. [Development of one corn ear and verification the three laws of inheritance].

    PubMed

    Zhao, Wen-Yao; Liu, Jun-Bo; Zhao, Liang

    2008-09-01

    A new corn was developed as a lab material to verify the three Laws of Inheritance on only one F2 ear with correct segregation ratios of kernel traits. In order to investigate the three Inheritance Laws simultaneously with one ear, the sweet endosperm (Sn1), kernel color (C1) and the glutinous endosperm (Wx) were chosen as the original phenotypes. Gene Sn1sn1 is located on chromosome four, genes C(1c1) and Wxwx are located on chromosome nine. After several generations of continuous self-pollination and test crossing, direct crossing and back crossing, with strict selections, the homozygous parents were obtained. With different combinations of homozygous parents, different hybrids were developed. The segregation ratios of each phenotype on the same F2 ear are compared to the theoretical segregation ratios according to the corn gene exchange and gamete types and quantities produced through meiosis. The results showed three ratios of 3:1, two ratios of 9:3:3:1, and one ratio of linkage and exchange, with all the chi-square values smaller than the permitted ones. This means that only one ear of this new hybrid corn can be used as a new teaching aid to examine and verify the three Laws of Inheritance. PMID:18779182

  11. Gross Motor Development, Movement Abnormalities, and Early Identification of Autism

    ERIC Educational Resources Information Center

    Ozonoff, Sally; Young, Gregory S.; Goldring, Stacy; Greiss-Hess, Laura; Herrera, Adriana M.; Steele, Joel; Macari, Suzanne; Hepburn, Susan; Rogers, Sally J.

    2008-01-01

    Gross motor development (supine, prone, rolling, sitting, crawling, walking) and movement abnormalities were examined in the home videos of infants later diagnosed with autism (regression and no regression subgroups), developmental delays (DD), or typical development. Group differences in maturity were found for walking, prone, and supine, with…

  12. The quest for restoring hearing: Understanding ear development more completely.

    PubMed

    Jahan, Israt; Pan, Ning; Elliott, Karen L; Fritzsch, Bernd

    2015-09-01

    Neurosensory hearing loss is a growing problem of super-aged societies. Cochlear implants can restore some hearing, but rebuilding a lost hearing organ would be superior. Research has discovered many cellular and molecular steps to develop a hearing organ but translating those insights into hearing organ restoration remains unclear. For example, we cannot make various hair cell types and arrange them into their specific patterns surrounded by the right type of supporting cells in the right numbers. Our overview of the topologically highly organized and functionally diversified cellular mosaic of the mammalian hearing organ highlights what is known and unknown about its development. Following this analysis, we suggest critical steps to guide future attempts toward restoration of a functional organ of Corti. We argue that generating mutant mouse lines that mimic human pathology to fine-tune attempts toward long-term functional restoration are needed to go beyond the hope generated by restoring single hair cells in postnatal sensory epithelia.

  13. Gene transfer to the developing mouse inner ear by in vivo electroporation.

    PubMed

    Wang, Lingyan; Jiang, Han; Brigande, John V

    2012-06-30

    The mammalian inner ear has 6 distinct sensory epithelia: 3 cristae in the ampullae of the semicircular canals; maculae in the utricle and saccule; and the organ of Corti in the coiled cochlea. The cristae and maculae contain vestibular hair cells that transduce mechanical stimuli to subserve the special sense of balance, while auditory hair cells in the organ of Corti are the primary transducers for hearing. Cell fate specification in these sensory epithelia and morphogenesis of the semicircular canals and cochlea take place during the second week of gestation in the mouse and are largely completed before birth. Developmental studies of the mouse inner ear are routinely conducted by harvesting transgenic embryos at different embryonic or postnatal stages to gain insight into the molecular basis of cellular and/or morphological phenotypes. We hypothesize that gene transfer to the developing mouse inner ear in utero in the context of gain- and loss-of-function studies represents a complimentary approach to traditional mouse transgenesis for the interrogation of the genetic mechanisms underlying mammalian inner ear development(6). The experimental paradigm to conduct gene misexpression studies in the developing mouse inner ear demonstrated here resolves into three general steps: 1) ventral laparotomy; 2) transuterine microinjection; and 3) in vivo electroporation. Ventral laparotomy is a mouse survival surgical technique that permits externalization of the uterus to gain experimental access to the implanted embryos. Transuterine microinjection is the use of beveled, glass capillary micropipettes to introduce expression plasmid into the lumen of the otic vesicle or otocyst. In vivo electroporation is the application of square wave, direct current pulses to drive expression plasmid into progenitor cells. We previously described this electroporation-based gene transfer technique and included detailed notes on each step of the protocol(11). Mouse experimental

  14. Swimmer's ear

    MedlinePlus

    ... such as acetaminophen (Tylenol) or ibuprofen (Advil, Motrin) Vinegar (acetic acid) ear drops People with chronic swimmer's ear may ... drop of alcohol with 1 drop of white vinegar and placing the mixture into the ears after ...

  15. Ear Disorders

    MedlinePlus

    ... ear, where they make your eardrum vibrate. The vibrations are transmitted through three tiny bones, called ossicles, in your middle ear. The vibrations travel to your inner ear, a snail-shaped ...

  16. Ear Infections

    MedlinePlus

    MENU Return to Web version Ear Infections Overview How does the ear work? A tube called the eustachian (say: "you-stay-shee-an") tube connects the middle ear with the back of the nose. Normally this ...

  17. Cauliflower Ear

    MedlinePlus

    ... Here's Help White House Lunch Recipes What's Cauliflower Ear? KidsHealth > For Kids > What's Cauliflower Ear? Print A A A Text Size Have you ever seen someone whose ear looks bumpy and lumpy? The person might have ...

  18. Ear barotrauma

    MedlinePlus

    ... Ear popping - barotrauma; Pressure-related ear pain; Eustachian tube dysfunction - barotrauma ... air pressure outside of the body. The Eustachian tube is a connection between the middle ear and ...

  19. SoxC transcription factors are essential for the development of the inner ear

    PubMed Central

    Gnedeva, Ksenia; Hudspeth, A. J.

    2015-01-01

    Hair cells, the mechanosensory receptors of the inner ear, underlie the senses of hearing and balance. Adult mammals cannot adequately replenish lost hair cells, whose loss often results in deafness or balance disorders. To determine the molecular basis of this deficiency, we investigated the development of a murine vestibular organ, the utricle. Here we show that two members of the SoxC family of transcription factors, Sox4 and Sox11, are down-regulated after the epoch of hair cell development. Conditional ablation of SoxC genes in vivo results in stunted sensory organs of the inner ear and loss of hair cells. Enhanced expression of SoxC genes in vitro conversely restores supporting cell proliferation and the production of new hair cells in adult sensory epithelia. These results imply that SoxC genes govern hair cell production and thus advance these genes as targets for the restoration of hearing and balance. PMID:26504244

  20. Macrophage migration inhibitory factor acts as a neurotrophin in the developing inner ear.

    PubMed

    Bank, Lisa M; Bianchi, Lynne M; Ebisu, Fumi; Lerman-Sinkoff, Dov; Smiley, Elizabeth C; Shen, Yu-chi; Ramamurthy, Poornapriya; Thompson, Deborah L; Roth, Therese M; Beck, Christine R; Flynn, Matthew; Teller, Ryan S; Feng, Luming; Llewellyn, G Nicholas; Holmes, Brandon; Sharples, Cyrrene; Coutinho-Budd, Jaeda; Linn, Stephanie A; Chervenak, Andrew P; Dolan, David F; Benson, Jennifer; Kanicki, Ariane; Martin, Catherine A; Altschuler, Richard; Koch, Alisa E; Koch, Alicia E; Jewett, Ethan M; Germiller, John A; Barald, Kate F

    2012-12-01

    This study is the first to demonstrate that macrophage migration inhibitory factor (MIF), an immune system 'inflammatory' cytokine that is released by the developing otocyst, plays a role in regulating early innervation of the mouse and chick inner ear. We demonstrate that MIF is a major bioactive component of the previously uncharacterized otocyst-derived factor, which directs initial neurite outgrowth from the statoacoustic ganglion (SAG) to the developing inner ear. Recombinant MIF acts as a neurotrophin in promoting both SAG directional neurite outgrowth and neuronal survival and is expressed in both the developing and mature inner ear of chick and mouse. A MIF receptor, CD74, is found on both embryonic SAG neurons and adult mouse spiral ganglion neurons. Mif knockout mice are hearing impaired and demonstrate altered innervation to the organ of Corti, as well as fewer sensory hair cells. Furthermore, mouse embryonic stem cells become neuron-like when exposed to picomolar levels of MIF, suggesting the general importance of this cytokine in neural development.

  1. Abortion and moral development theory: listening with different ears.

    PubMed

    Smith, J E

    1989-01-01

    Listening to the reasons used by women to justify their action of having an abortion is a good way of determining its moral status. However, it must be remembered that the opinions one has about abortion cannot be separated from the manner in which questions are asked of these women. In fact the moral opinion of the researcher is interwoven with the methodology and questions used to interview women. This examination of the above mentioned issue takes place in the context of analyzing Carol Gilligan's book, In a Different Voice. This work will be used to illustrate how a researchers own opinions about abortion can be seen in the process of setting up a study to interview women to determine the moral value of abortion based on the moral justification of abortion seekers. There seems to be a difference of opinion about the use of the terms caring, responsible and non-violent by the women being interviewed. Gilligan states that the moral development of women occurs in 3 stages. Part of the transformation process involves women realizing the good does not necessarily involve self-sacrifice. Gilligan's treatment of the interviews is not always consistent with her own philosophical views; however, she routinely fails to ask rather obvious questions of women that may suggest that they did not reason well about their abortion decision. This ultimately is the weak link in Gilligan's analysis and theories. If you assume that women are the pivotal decision makers in determining the moral status of abortion, you must assume that they always reason correctly, which is of course not the case.

  2. Embryonic development of the concave-eared torrent frog with its significance on taxonomy.

    PubMed

    Xiong, Rong-Chuan; Jiang, Jian-Ping; Fei, Liang; Wang, Bin; Ye, Chang-Yuan

    2010-10-01

    We investigated the early embryonic and larval development of the concave-eared torrent frogs, Odorrana tormota (Amphibia, Anura, Ranidae). Embryos were derived from artificial fertilization of frogs' eggs, and the staging of development was based on morphological and physiological characteristics. Two major periods of development were designated: i) early embryonic period, from fertilization to operculum completion stage, lasted for 324 h at water temperature (WT) 18 - 23degree; ii) larval period, from operculum completion stage to tail absorbed stage, took 1207 h at WT 20 - 24degree. Tadpoles of the concave-eared torrent frogs showed no evidence of abdominal sucker. Absence of this key characteristic supports the view from molecular systematics that concave-eared torrent frog does not belong to the genus Amolops. Two cleavage patterns were observed in embryos at 8-cell and 16-cell stages, with Pattern I - 2 (latitudinal cleavage at the 8-cell stage, and meridional cleavage at the 16-cell stage with two perpendicular meridional furrows) being the predominant pattern and only 1.5% belonging to Pattern II (meridional cleavage at the 8-cell stage and latitudinal cleavage at the 16-cell stage). The factors affecting cleavage and hatching ratios, developmental speed, and ecological adaptation were discussed.

  3. MEKK4 Signaling Regulates Sensory Cell Development and Function in the Mouse Inner Ear

    PubMed Central

    Haque, Khujista; Pandey, Atul K.; Zheng, Hong-Wei; Riazuddin, Saima; Sha, Su-Hua

    2016-01-01

    Mechanosensory hair cells (HCs) residing in the inner ear are critical for hearing and balance. Precise coordination of proliferation, sensory specification, and differentiation during development is essential to ensure the correct patterning of HCs in the cochlear and vestibular epithelium. Recent studies have revealed that FGF20 signaling is vital for proper HC differentiation. However, the mechanisms by which FGF20 signaling promotes HC differentiation remain unknown. Here, we show that mitogen-activated protein 3 kinase 4 (MEKK4) expression is highly regulated during inner ear development and is critical to normal cytoarchitecture and function. Mice homozygous for a kinase-inactive MEKK4 mutation exhibit significant hearing loss. Lack of MEKK4 activity in vivo also leads to a significant reduction in the number of cochlear and vestibular HCs, suggesting that MEKK4 activity is essential for overall development of HCs within the inner ear. Furthermore, we show that loss of FGF20 signaling in vivo inhibits MEKK4 activity, whereas gain of Fgf20 function stimulates MEKK4 expression, suggesting that Fgf20 modulates MEKK4 activity to regulate cellular differentiation. Finally, we demonstrate, for the first time, that MEKK4 acts as a critical node to integrate FGF20-FGFR1 signaling responses to specifically influence HC development and that FGFR1 signaling through activation of MEKK4 is necessary for outer hair cell differentiation. Collectively, this study provides compelling evidence of an essential role for MEKK4 in inner ear morphogenesis and identifies the requirement of MEKK4 expression in regulating the specific response of FGFR1 during HC development and FGF20/FGFR1 signaling activated MEKK4 for normal sensory cell differentiation. SIGNIFICANCE STATEMENT Sensory hair cells (HCs) are the mechanoreceptors within the inner ear responsible for our sense of hearing. HCs are formed before birth, and mammals lack the ability to restore the sensory deficits associated

  4. Applications for single cell trajectory analysis in inner ear development and regeneration

    PubMed Central

    Durruthy-Durruthy, Robert; Heller, Stefan

    2015-01-01

    Single cell trajectory analysis is a computational approach that orders cells along a pseudotime axis. This temporal modeling approach allows the characterization of transitional processes such as lineage development, response to insult, and tissue regeneration. The concept can also be applied to resolve spatial organization of cells within the originating tissue. Known as temporal and spatial transcriptomics, respectively, these methods belong to the most powerful analytical analysis techniques for quantitative gene expression data currently available. Here, we discuss three different approaches: principal component analysis, the ‘Monocle’ algorithm, and self-organizing maps. We use a previously published qRT-PCR dataset of single neuroblast cells isolated from the developing mouse inner ear to highlight the basic features of the three methods, their individual limitations, as well as the distinct advantages that make them useful for research in the inner ear. The complex developmental morphogenesis of the inner ear and its specific challenges such as the paucity of cells as well as important open questions such as sensory hair cell regeneration render this organ as a prime target for single cell trajectory analysis strategies. PMID:25532874

  5. Expression and function of FGF10 in mammalian inner ear development

    NASA Technical Reports Server (NTRS)

    Pauley, Sarah; Wright, Tracy J.; Pirvola, Ulla; Ornitz, David; Beisel, Kirk; Fritzsch, Bernd

    2003-01-01

    We have investigated the expression of FGF10 during ear development and the effect of an FGF10 null mutation on ear development. Our in situ hybridization data reveal expression of FGF10 in all three canal crista sensory epithelia and the cochlea anlage as well as all sensory neurons at embryonic day 11.5 (E11.5). Older embryos (E18.5) displayed strong graded expression in all sensory epithelia. FGF10 null mutants show complete agenesis of the posterior canal crista and the posterior canal. The posterior canal sensory neurons form initially and project rather normally by E11.5, but they disappear within 2 days. FGF10 null mutants have no posterior canal system at E18.5. In addition, these mutants have deformations of the anterior and horizontal cristae, reduced formation of the anterior and horizontal canals, as well as altered position of the remaining sensory epithelia with respect to the utricle. Hair cells form but some have defects in their cilia formation. No defects were detected in the organ of Corti at the cellular level. Together these data suggest that FGF10 plays a major role in ear morphogenesis. Most of these data are consistent with earlier findings on a null mutation in FGFR2b, one of FGF10's main receptors. Copyright 2003 Wiley-Liss, Inc.

  6. Pulmonary vascular development goes awry in congenital lung abnormalities.

    PubMed

    Kool, Heleen; Mous, Daphne; Tibboel, Dick; de Klein, Annelies; Rottier, Robbert J

    2014-12-01

    Pulmonary vascular diseases of the newborn comprise a wide range of pathological conditions with developmental abnormalities in the pulmonary vasculature. Clinically, pulmonary arterial hypertension (PH) is characterized by persistent increased resistance of the vasculature and abnormal vascular response. The classification of PH is primarily based on clinical parameters instead of morphology and distinguishes five groups of PH. Congenital lung anomalies, such as alveolar capillary dysplasia (ACD) and PH associated with congenital diaphragmatic hernia (CDH), but also bronchopulmonary dysplasia (BPD), are classified in group three. Clearly, tight and correct regulation of pulmonary vascular development is crucial for normal lung development. Human and animal model systems have increased our knowledge and make it possible to identify and characterize affected pathways and study pivotal genes. Understanding of the normal development of the pulmonary vasculature will give new insights in the origin of the spectrum of rare diseases, such as CDH, ACD, and BPD, which render a significant clinical problem in neonatal intensive care units around the world. In this review, we describe normal pulmonary vascular development, and focus on four diseases of the newborn in which abnormal pulmonary vascular development play a critical role in morbidity and mortality. In the future perspective, we indicate the lines of research that seem to be very promising for elucidating the molecular pathways involved in the origin of congenital pulmonary vascular disease. PMID:25424472

  7. Development of a surgical instrument for measuring forces applied to the ossicles of the middle ear.

    PubMed

    Sheedy, Michael; Bergin, Mike; Wylie, Grant; Ross, Peter; Dove, Richard; Bird, Phil

    2012-12-01

    Surgery of the middle ear is a delicate process that requires the surgeon to manipulate the ossicles, the smallest bones in the body. Excessive force applied to the ossicles can easily be transmitted through to the inner ear which may cause a permanent sensorineural hearing loss. An instrument was required to measure the forces applied to cadaveric temporal bone ossicles with the vision of measuring forces in vivo at a later stage. A feasibility study was conducted to investigate a method of measuring force and torque applied to the ossicles of the middle ear. Information from research papers was gathered to determine the expected amplitudes. The study looked at commercially available transducers as well as constructing an instrument using individual axis transducers coupled together. A prototype surgical instrument was constructed using the ATI industrial automation Nano17 six axis transducer. The Nano17 allows for the measurement of force and torque in the X, Y and Z axis to a resolution of 1/320 N. The use of the Nano17 enabled rapid development of the surgical instrument. It meets the requirements for its use on cadaveric models and has the potential to be a useful data collection tool in vivo.

  8. Ear Pieces

    ERIC Educational Resources Information Center

    DiJulio, Betsy

    2011-01-01

    In this article, the author describes an art project wherein students make fanciful connections between art and medicine. This project challenges students to interpret "ear idioms" (e.g. "blow it out your ear," "in one ear and out the other") by relying almost entirely on realistic ear drawings, the placement of them, marks, and values. In that…

  9. Ear Mite Removal in the Santa Catalina Island Fox (Urocyon littoralis catalinae): Controlling Risk Factors for Cancer Development

    PubMed Central

    Moriarty, Megan E.; Vickers, T. Winston; Clifford, Deana L.; Garcelon, David K.; Gaffney, Patricia M.; Lee, Kenneth W.; King, Julie L.; Duncan, Calvin L.; Boyce, Walter M.

    2015-01-01

    Ear mites (Otodectes cynotis) and ear canal tumors are highly prevalent among federally endangered Island foxes (Urocyon littoralis catalinae) living on Santa Catalina Island off the coast of Southern California. Since studies began in the 1990s, nearly all foxes examined were found to be infected with ear mites, and ceruminous gland tumors (carcinomas and adenomas) were detected in approximately half of all foxes ≥ 4 years of age. We hypothesized that reduction of ear mite infection would reduce otitis externa and ceruminous gland hyperplasia, a risk factor for tumor development. In this study, we conducted a randomized field trial to assess the impact of acaricide treatment on ear mite prevalence and intensity of infection, otitis externa, ceruminous gland hyperplasia, and mite-specific IgG and IgE antibody levels. Treatment was highly effective at eliminating mites and reducing otitis externa and ceruminous gland hyperplasia, and mite-specific IgG antibody levels were significantly lower among uninfected foxes. Ceruminous gland hyperplasia increased in the chronically infected, untreated foxes during the six month study. Our results provide compelling evidence that acaricide treatment is an effective means of reducing ear mites, and that mite removal in turn reduces ear lesions and mite-specific IgG antibody levels in Santa Catalina Island foxes. This study has advanced our understanding of the underlying pathogenesis which results in ceruminous gland tumors, and has helped inform management decisions that impact species conservation. PMID:26641820

  10. Ear Mite Removal in the Santa Catalina Island Fox (Urocyon littoralis catalinae): Controlling Risk Factors for Cancer Development.

    PubMed

    Moriarty, Megan E; Vickers, T Winston; Clifford, Deana L; Garcelon, David K; Gaffney, Patricia M; Lee, Kenneth W; King, Julie L; Duncan, Calvin L; Boyce, Walter M

    2015-01-01

    Ear mites (Otodectes cynotis) and ear canal tumors are highly prevalent among federally endangered Island foxes (Urocyon littoralis catalinae) living on Santa Catalina Island off the coast of Southern California. Since studies began in the 1990s, nearly all foxes examined were found to be infected with ear mites, and ceruminous gland tumors (carcinomas and adenomas) were detected in approximately half of all foxes ≥ 4 years of age. We hypothesized that reduction of ear mite infection would reduce otitis externa and ceruminous gland hyperplasia, a risk factor for tumor development. In this study, we conducted a randomized field trial to assess the impact of acaricide treatment on ear mite prevalence and intensity of infection, otitis externa, ceruminous gland hyperplasia, and mite-specific IgG and IgE antibody levels. Treatment was highly effective at eliminating mites and reducing otitis externa and ceruminous gland hyperplasia, and mite-specific IgG antibody levels were significantly lower among uninfected foxes. Ceruminous gland hyperplasia increased in the chronically infected, untreated foxes during the six month study. Our results provide compelling evidence that acaricide treatment is an effective means of reducing ear mites, and that mite removal in turn reduces ear lesions and mite-specific IgG antibody levels in Santa Catalina Island foxes. This study has advanced our understanding of the underlying pathogenesis which results in ceruminous gland tumors, and has helped inform management decisions that impact species conservation. PMID:26641820

  11. Ear trauma.

    PubMed

    Eagles, Kylee; Fralich, Laura; Stevenson, J Herbert

    2013-04-01

    Understanding basic ear anatomy and function allows an examiner to quickly and accurately identify at-risk structures in patients with head and ear trauma. External ear trauma (ie, hematoma or laceration) should be promptly treated with appropriate injury-specific techniques. Tympanic membrane injuries have multiple mechanisms and can often be conservatively treated. Temporal bone fractures are a common cause of ear trauma and can be life threatening. Facial nerve injuries and hearing loss can occur in ear trauma.

  12. Ear manipulations reveal a critical period for survival and dendritic development at the single-cell level in Mauthner neurons.

    PubMed

    Elliott, Karen L; Houston, Douglas W; DeCook, Rhonda; Fritzsch, Bernd

    2015-12-01

    Second-order sensory neurons are dependent on afferents from the sense organs during a critical period in development for their survival and differentiation. Past research has mostly focused on whole populations of neurons, hampering progress in understanding the mechanisms underlying these critical phases. To move toward a better understanding of the molecular and cellular basis of afferent-dependent neuronal development, we developed a new model to study the effects of ear removal on a single identifiable cell in the hindbrain of a frog, the Mauthner cell. Ear extirpation at various stages of Xenopus laevis development defines a critical period of progressively-reduced dependency of Mauthner cell survival/differentiation on the ear afferents. Furthermore, ear removal results in a progressively decreased reduction in the number of dendritic branches. Conversely, addition of an ear results in an increase in the number of dendritic branches. These results suggest that the duration of innervation and the number of inner ear afferents play a quantitative role in Mauthner cell survival/differentiation, including dendritic development.

  13. Morphogenesis of the inner ear at different stages of normal human development.

    PubMed

    Toyoda, Saki; Shiraki, Naoto; Yamada, Shigehito; Uwabe, Chigako; Imai, Hirohiko; Matsuda, Tetsuya; Yoneyama, Akio; Takeda, Tohoru; Takakuwa, Tetsuya

    2015-12-01

    This study examined the external morphology and morphometry of the human embryonic inner ear membranous labyrinth and documented its three-dimensional position in the developing embryo using phase-contrast X-ray computed tomography and magnetic resonance imaging. A total of 27 samples between Carnegie stage (CS) 17 and the postembryonic phase during trimester 1 (approximately 6-10 weeks after fertilization) were included. The otic vesicle elongated along the dorso-ventral axis and differentiated into the end lymphatic appendage and cochlear duct (CD) at CS 17. The spiral course of the CD began at CS18, with anterior and posterior semicircular ducts (SDs) forming prominent circles with a common crus. The spiral course of the CD comprised more than two turns at the postembryonic phase, at which time the height of the CD was evident. A linear increase was observed in the length of anterior, posterior, and lateral SDs, in that order, and the length of the CD increased exponentially over the course of development. Bending in the medial direction was observed between the cochlear and vestibular parts from the latero-caudal view, with the angle decreasing during development. The position of the inner ear was stable throughout the period of observation on the lateral to ventral side of the rhombencephalon, caudal to the pontine flexure, and adjacent to the auditory ganglia. The plane of the lateral semicircular canal was approximately 8.0°-14.6° with respect to the cranial caudal (z-)axis, indicating that the orientation of the inner ear changes during growth to adulthood.

  14. The boron efflux transporter ROTTEN EAR is required for maize inflorescence development and fertility.

    PubMed

    Chatterjee, Mithu; Tabi, Zara; Galli, Mary; Malcomber, Simon; Buck, Amy; Muszynski, Michael; Gallavotti, Andrea

    2014-07-01

    Although boron has a relatively low natural abundance, it is an essential plant micronutrient. Boron deficiencies cause major crop losses in several areas of the world, affecting reproduction and yield in diverse plant species. Despite the importance of boron in crop productivity, surprisingly little is known about its effects on developing reproductive organs. We isolated a maize (Zea mays) mutant, called rotten ear (rte), that shows distinct defects in vegetative and reproductive development, eventually causing widespread sterility in its inflorescences, the tassel and the ear. Positional cloning revealed that rte encodes a membrane-localized boron efflux transporter, co-orthologous to the Arabidopsis thaliana BOR1 protein. Depending on the availability of boron in the soil, rte plants show a wide range of phenotypic defects that can be fully rescued by supplementing the soil with exogenous boric acid, indicating that rte is crucial for boron transport into aerial tissues. rte is expressed in cells surrounding the xylem in both vegetative and reproductive tissues and is required for meristem activity and organ development. We show that low boron supply to the inflorescences results in widespread defects in cell and cell wall integrity, highlighting the structural importance of boron in the formation of fully fertile reproductive organs. PMID:25035400

  15. The boron efflux transporter ROTTEN EAR is required for maize inflorescence development and fertility.

    PubMed

    Chatterjee, Mithu; Tabi, Zara; Galli, Mary; Malcomber, Simon; Buck, Amy; Muszynski, Michael; Gallavotti, Andrea

    2014-07-01

    Although boron has a relatively low natural abundance, it is an essential plant micronutrient. Boron deficiencies cause major crop losses in several areas of the world, affecting reproduction and yield in diverse plant species. Despite the importance of boron in crop productivity, surprisingly little is known about its effects on developing reproductive organs. We isolated a maize (Zea mays) mutant, called rotten ear (rte), that shows distinct defects in vegetative and reproductive development, eventually causing widespread sterility in its inflorescences, the tassel and the ear. Positional cloning revealed that rte encodes a membrane-localized boron efflux transporter, co-orthologous to the Arabidopsis thaliana BOR1 protein. Depending on the availability of boron in the soil, rte plants show a wide range of phenotypic defects that can be fully rescued by supplementing the soil with exogenous boric acid, indicating that rte is crucial for boron transport into aerial tissues. rte is expressed in cells surrounding the xylem in both vegetative and reproductive tissues and is required for meristem activity and organ development. We show that low boron supply to the inflorescences results in widespread defects in cell and cell wall integrity, highlighting the structural importance of boron in the formation of fully fertile reproductive organs.

  16. Your Ears

    MedlinePlus

    ... Protect your hearing by wearing earplugs at loud music concerts and around noisy machinery, like in wood ... For Parents MORE ON THIS TOPIC Can Loud Music Hurt My Ears? What Is an Ear Infection? ...

  17. Ear wax

    MedlinePlus

    The ear canal is lined with hair follicles and glands that produce a waxy oil called cerumen. The wax will most ... Wax can build up and block the ear canal. Wax blockage is one of the most common ...

  18. Inborn errors of metabolism: a cause of abnormal brain development.

    PubMed

    Nissenkorn, A; Michelson, M; Ben-Zeev, B; Lerman-Sagie, T

    2001-05-22

    Brain malformations are caused by a disruption in the sequence of normal development by various environmental or genetic factors. By modifying the intrauterine milieu, inborn errors of metabolism may cause brain dysgenesis. However, this association is typically described in single case reports. The authors review the relationship between brain dysgenesis and specific inborn errors of metabolism. Peroxisomal disorders and fatty acid oxidation defects can produce migration defects. Pyruvate dehydrogenase deficiency, nonketotic hyperglycinemia, and maternal phenylketonuria preferentially cause a dysgenetic corpus callosum. Abnormal metabolism of folic acid causes neural tube defects, whereas defects in cholesterol metabolism may produce holoprosencephaly. Various mechanisms have been proposed to explain abnormal brain development in inborn errors of metabolism: production of a toxic or energy-deficient intrauterine milieu, modification of the content and function of membranes, or disturbance of the normal expression of intrauterine genes responsible for morphogenesis. The recognition of a metabolic disorder as the cause of the brain malformation has implications for both the care of the patient and for genetic counseling to prevent recurrence in subsequent pregnancies. PMID:11383558

  19. Evolution and development of hair cell polarity and efferent function in the inner ear.

    PubMed

    Sienknecht, Ulrike J; Köppl, Christine; Fritzsch, Bernd

    2014-01-01

    The function of the inner ear critically depends on mechanoelectrically transducing hair cells and their afferent and efferent innervation. The first part of this review presents data on the evolution and development of polarized vertebrate hair cells that generate a sensitive axis for mechanical stimulation, an essential part of the function of hair cells. Beyond the cellular level, a coordinated alignment of polarized hair cells across a sensory epithelium, a phenomenon called planar cell polarity (PCP), is essential for the organ's function. The coordinated alignment of hair cells leads to hair cell orientation patterns that are characteristic of the different sensory epithelia of the vertebrate inner ear. Here, we review the developmental mechanisms that potentially generate molecular and morphological asymmetries necessary for the control of PCP. In the second part, this review concentrates on the evolution, development and function of the enigmatic efferent neurons terminating on hair cells. We present evidence suggestive of efferents being derived from motoneurons and synapsing predominantly onto a unique but ancient cholinergic receptor. A review of functional data shows that the plesiomorphic role of the efferent system likely was to globally shut down and protect the peripheral sensors, be they vestibular, lateral line or auditory hair cells, from desensitization and damage during situations of self-induced sensory overload. The addition of a dedicated auditory papilla in land vertebrates appears to have favored the separation of vestibular and auditory efferents and specializations for more sophisticated and more diverse functions.

  20. Histochemical Localisation of Carbonic Anhydrase in the Inner Ear of developing Cichlid Fish, Oreochromis mossambicus

    NASA Astrophysics Data System (ADS)

    Beier, M.; Hilbig, R.; Anken, R.

    Inner ear otolith growth in terms of mineralisation mainly depends on the enzyme carbonic anhydrase CA CA is located in specialised mitochondria-rich macular cells ionocytes which are involved in the endolymphatic ion exchange and the enzyme is responsible for the provision of the pH-value necessary for otolithic calcium carbonate deposition In the present study for the first time the localisation of histochemically demonstrated CA was analysed during the early larval development of a teleost the cichlid fish Oreochromis mossambicus CA-reactivity was observed already in stage 7 animals onset of otocyst development staging follows Anken et al Zool Anz 231 1-10 1993 Neuroblasts from which sensory and supporting cells as well as ionocytes are derived proved to be CA positive Already at stage 12 hatch CA-positive regions could be attributed to ionocyte containg regions both in the so-called meshwork and patches area of the macula i e clearly before ionocytes can be identified on ultrastructural level or by employing immunocytochemistry In contrast to the circumstances observed in mammalian species sensory hair cells stained negative for CA in the cichlid With the onset of stage 16 finray primordia in dorsal fin yolk-sac being increasingly absorbed CA-reactivity was observed in the vestibular nerve This indicates the onset of myelinisation and thus commencement of operation The localisation of CA in the inner ear of fish especially the differences in comparison to mammals is discussed on the basis of its role in otolith

  1. Ear Infections

    MedlinePlus

    ... affects the middle ear and is called otitis media. The tubes inside the ears become clogged with fluid and mucus. This can affect hearing, because sound cannot get through all that fluid. If your child isn't old enough to say "My ear ...

  2. Super Ears.

    ERIC Educational Resources Information Center

    Thompson, Stan

    1995-01-01

    Presents an activity in which students design, construct, and test "super ears" to investigate sound and hearing. Students work in groups of three and explore how the outer ear funnels sound waves to the inner ear and how human hearing compares to that of other animals. (NB)

  3. Methods to study the development, anatomy, and function of the zebrafish inner ear across the life course.

    PubMed

    Baxendale, S; Whitfield, T T

    2016-01-01

    The inner ear is a remarkably intricate structure able to detect sound, motion, and gravity. During development of the zebrafish embryo, the ear undergoes dynamic morphogenesis from a simple epithelial vesicle into a complex labyrinth, consisting of three semicircular canals and three otolithic sensory organs, each with an array of differentiated cell types. This microcosm of biology has led to advances in understanding molecular and cellular changes in epithelial patterning and morphogenesis, through to mechanisms of mechanosensory transduction and the origins of reflexive behavior. In this chapter, we describe different methods to study the zebrafish ear, including high-speed imaging of otic cilia, confocal microscopy, and light-sheet fluorescent microscopy. Many dyes, antibodies, and transgenic lines for labeling the ear are available, and we provide a comprehensive review of these resources. The developing ear is amenable to genetic, chemical, and physical manipulations, including injection and transplantation. Chemical modulation of developmental signaling pathways has paved the way for zebrafish to be widely used in drug discovery. We describe two chemical screens with relevance to the ear: a fluorescent-based screen for compounds that protect against ototoxicity, and an in situ-based screen for modulators of a signaling pathway involved in semicircular canal development. We also describe methods for dissection and imaging of the adult otic epithelia. We review both manual and automated methods to test the function of the inner ear and lateral line, defects in which can lead to altered locomotor behavior. Finally, we review a collection of zebrafish models that are generating new insights into human deafness and vestibular disorders. PMID:27312494

  4. CHRONIC PERCHLORATE EXPOSURE CAUSES MORPHOLOGICAL ABNORMALITIES IN DEVELOPING STICKLEBACK

    PubMed Central

    Bernhardt, Richard R.; Von Hippel, Frank A.; O’Hara, Todd M.

    2011-01-01

    Few studies have examined the effects of chronic perchlorate exposure during growth and development, and fewer still have analyzed the effects of perchlorate over multiple generations. We describe morphological and developmental characteristics for threespine stickleback (Gasterosteus aculeatus) that were spawned and raised to sexual maturity in perchlorate-treated water (G1,2003) and for their offspring (G2,2004) that were not directly treated with perchlorate. The G1,2003 displayed a variety of abnormalities, including impaired formation of calcified traits, slower growth rates, aberrant sexual development, poor survivorship, and reduced pigmentation that allowed internal organs to be visible. Yet these conditions were absent when the offspring of contaminated fish (G2,2004) were raised in untreated water, suggesting a lack of transgenerational effects and that surviving populations may be able to recover following remediation of perchlorate-contaminated sites PMID:21465539

  5. The role of the Utah Artificial Ear project in the development of the modern cochlear implant.

    PubMed

    Dorman, Michael F; Parkin, James L

    2015-05-01

    The cochlear implant has provided the first substantial restoration of a human sense by a medical intervention. This accomplishment was brought about by the efforts, over a 50+ year period, of many individuals in laboratories around the world. In this paper, we recount the history of one of the early projects - the Utah Artificial Ear project. In 1970 researchers at the University of Utah began work on an auditory prosthesis. A critical early decision was to create a 'transparent' link between external signal processing and the electrodes implanted in the cochlea, i.e., a percutaneous pedestal. The pedestal allowed D. Eddington, then a graduate student, to conduct, in 1975-1978, the first thorough, parametric, psychophysical studies of electrical stimulation of the cochlea in multiple human volunteers. The early work by Eddington and colleagues evolved in 1983 into the 4-channel, Ineraid cochlear implant. Many years later, highly effective, modern signal processing algorithms, e.g., continuous interleaved sampling (CIS), fine structure processing (FSP), and virtual channel processing, were first tested and developed with the aid of Ineraid patients fit with pedestals of the Utah design. Because for many years the Ineraid provided as high a level of speech understanding as that provided by other devices and because the percutaneous pedestal allowed the first testing of many modern signal processing algorithms, the Utah Artificial Ear project may be viewed as one of the most valuable research projects in the history of cochlear implants. PMID:25941941

  6. Normal and Abnormal Development in the Arabidopsis Vegetative Shoot Apex.

    PubMed Central

    Medford, JI; Behringer, FJ; Callos, JD; Feldmann, KA

    1992-01-01

    Vegetative development in the Arabidopsis shoot apex follows both sequential and repetitive steps. Early in development, the young vegetative meristem is flat and has a rectangular shape with bilateral symmetry. The first pair of leaf primordia is radially symmetrical and is initiated on opposite sides of the meristem. As development proceeds, the meristem changes first to a bilaterally symmetrical trapezoid and then to a radially symmetrical dome. Vegetative development from the domed meristem continues as leaves are initiated in a repetitive manner. Abnormal development of the vegetative shoot apex is described for a number of mutants. The mutants we describe fall into at least three classes: (1) lesions in the shoot apex that do not show an apparent alteration in the shoot apical meristem, (2) lesions in the apical meristem that also (directly or indirectly) alter leaf primordia, and (3) lesions in the apical meristem that alter meristem size and leaf number but not leaf morphology. These mutations provide tools both to genetically analyze vegetative development of the shoot apex and to learn how vegetative development influences floral development. PMID:12297656

  7. [Evolution of the ear].

    PubMed

    Qvist, Morten Rosenkilde

    2009-12-14

    The evolution of the ear may be traced through transitional fossils, comparative anatomy and embryology. The organ of hearing evolved from receptors of the vestibulary organ of fish. The tympanic ear developed in amphibians at the transition to terrestrial life, and the hyomandibula was isolated as the first middle ear bone, the columella stapes. Reptile jaw bones, quadratum and articulare, transformed to malleus and incus in mammals. With selective advantages during the evolution, an increasing structural complexity of the ear accompanied improved sound transmission and reception.

  8. Testin interacts with Vangl2 genetically to regulate inner ear sensory cell orientation and the normal development of the female reproductive tract in mice

    PubMed Central

    Ren, Dong-Dong; Kelly, Michael; Kim, Sun Myoung; Grimsley-Myers, Cynthia Mary; Chi, Fang-Lu; Chen, Ping

    2014-01-01

    Background Planar cell polarity (PCP) signaling regulates the coordinated polarization of cells and is required for the normal development and function of many tissues. Previous studies have identified conserved PCP genes, such as Van gogh-like 2 (Vangl2) and Prickle (Pk), in the regulation of coordinated orientation of inner ear hair cells and female reproductive tract development. Testin shares a PET-LIM homology with Pk. It is not clear whether Testin acts in PCP processes in mammals. Results We identified Testin as a Vangl2-interacting protein through a 2-hybrid screen with a cochlea cDNA library. Testin is enriched to cell-cell boundaries in the presence of Vangl2 in cultured cells. Genetic inactivation of Testin leads to abnormal hair cell orientation in the vestibule and cellular patterning defects in the cochlea. In addition, Testin genetically interacts with Vangl2 to regulate hair cell orientation in the cochlea and the opening of the vaginal tract. Conclusions Our findings suggested Testin as a gene involved in coordinated hair cell orientation in the inner ear and in female reproductive tract development. Furthermore, its genetic interaction with Vangl2 implicated it as a potential molecular link, responsible for mediating the role of Vangl2-containing membranous PCP complexes in directing morphologic polarization. PMID:23996638

  9. Inner ear development: Building a spiral ganglion and an organ of Corti out of unspecified ectoderm

    PubMed Central

    Fritzsch, Bernd; Pan, Ning; Jahan, Israt; Elliott, Karen L.

    2014-01-01

    The mammalian inner ear develops from a placodal thickening into a complex labyrinth of ducts with five sensory organs specialized to detect position and movement in space. In addition, the mammalian ear develops a spiraled cochlear duct containing the auditory organ, the organ of Corti (OC), specialized to translate sound into hearing. Developing the OC out of a uniform sheet of ectoderm requires an unparalleled precision in topological developmental engineering of four different general cell types, sensory neurons, hair cells, supporting cells, and general otic epithelium, into a mosaic of ten distinctly recognizable cell types in and around the OC, each with a unique distribution. In addition, the OC receives a unique innervation by ear-derived spiral ganglion afferents and brainstem-derived motor neurons as efferents, and requires neural crest-derived Schwann cells to form myelin and neural crest-derived cells to induce the stria vascularis. To achieve this transformation of a sheet of cells into a complicated interdigitating set of cells necessitates the orchestrated expression of multiple transcription factors that enable the cellular transformation from ectoderm into neurosensory cells forming the spiral ganglion neurons (SGN) while simultaneously transforming the flat epithelium into a tube, the cochlear duct housing the OC. In addition to the cellular and conformational changes to make the cochlear duct with the OC, additional changes in the surrounding periotic mesenchyme form passageways for sound to stimulate the OC. This article reviews molecular developmental data generated predominantly in mice. The available data are ordered into a plausible scenario that integrates the well described expression changes of transcription factors and their actions revealed in mouse mutants for formation of SGNs and OC in the right position and orientation with the right kind of innervation. Understanding the molecular basis of these developmental changes leading to

  10. Understanding the evolution and development of neurosensory transcription factors of the ear to enhance therapeutic translation

    PubMed Central

    Pan, Ning; Kopecky, Benjamin; Jahan, Israt; Fritzsch, Bernd

    2012-01-01

    Reconstructing a functional organ of Corti is the ultimate target towards curing hearing loss. Despite the impressive technical gains made over the last few years, many complications remain ahead for the two main restoration avenues: in vitro transformation of pluripotent cells into hair cell-like cells and adenovirus-mediated gene therapy. Most notably, both approaches require a more complete understanding of the molecular networks that ensure specific cell types form in the correct places to allow proper function of the restored organ of Corti. Important to this understanding are the basic helix-loop-helix (bHLH) transcription factors (TFs) that are highly diverse and serve to increase functional complexity but their evolutionary implementation in the inner ear neurosensory development is less conspicuous. To this end, we review the evolutionary and developmentally dynamic interactions of the three bHLH TFs that have been identified as the main players in neurosensory evolution and development, Neurog1, Neurod1 and Atoh1. These three TFs belong to the neurogenin/atonal family and evolved from a molecular precursor that likely regulated single sensory cell development in the ectoderm of metazoan ancestors but are now also expressed in other parts of the body, including the brain. They interact extensively via intracellular and intercellular cross-regulation to establish the two main neurosensory cell types of the ear, the hair cells and sensory neurons. Furthermore, the level and duration of their expression affect the specification of hair cell subtypes (inner hair cells vs. outer hair cells). We propose that appropriate manipulation of these TFs through their characterized binding sites may offer a solution by itself, or in conjunction with the two other approaches currently pursued by others, to restore the organ of Corti. PMID:22688958

  11. Histochemical localisation of carbonic anhydrase in the inner ear of developing cichlid fish, Oreochromis mossambicus

    NASA Astrophysics Data System (ADS)

    Beier, M.; Hilbig, R.; Anken, R.

    2008-12-01

    Inner ear otolith growth in terms of mineralisation mainly depends on the enzyme carbonic anhydrase (CAH). CAH is located in specialised, mitochondria-rich macular cells (ionocytes), which are involved in the endolymphatic ion exchange, and the enzyme is responsible for the provision of the pH-value necessary for otolithic calcium carbonate deposition. In the present study, for the first time the localisation of histochemically demonstrated CAH was analysed during the early larval development of a teleost, the cichlid fish Oreochromis mossambicus. CAH-reactivity was observed already in stage 7 animals (onset of otocyst development; staging follows Anken et al. [Anken, R., Kappel, T., Slenzka, K., Rahmann, H. The early morphogenetic development of the cichlid fish, Oreochromis mossambicus (Perciformes, Teleostei). Zool. Anz. 231, 1-10, 1993]). Neuroblasts (from which sensory and supporting cells are derived) proved to be CAH-positive. Already at stage 12 (hatch), CAH-positive regions could be attributed to ionocyte containing regions both in the so-called meshwork and patches area of the macula (i.e., clearly before ionocytes can be identified on ultrastructural level or by employing immunocytochemistry). In contrast to the circumstances observed in mammalian species, sensory hair cells stained negative for CAH in the cichlid. With the onset of stage 16 (finray primordia in dorsal fin, yolk-sac being increasingly absorbed), CAH-reactivity was observed in the vestibular nerve. This indicates the onset of myelinisation and thus commencement of operation. The localisation of CAH in the inner ear of fish (especially the differences in comparison to mammals) is discussed on the basis of its role in otolith calcification. Since the vestibular system is a detector of acceleration and thus gravity, also aspects regarding effects of altered gravity on CAH and hence on the mineralisation of otoliths in an adaptive process are addressed.

  12. Effect of Hypergravity on Carbonanhydrase Reactivity in inner Ear Ioncytes of developing Cichlid Fish

    NASA Astrophysics Data System (ADS)

    Beier, M.; Anken, R.; Rahmann, H.

    It has been shown earlier that hypergravity slows down inner ear otolith growth in developing fish. Otolith growth in terms of mineralisation mainly depends on the enzyme carboanhydrase (CAH), which is responsible for the provision of the pH- value necessary for calcium carbonate deposition and thus also is presumed to play a prominent role in Ménière's disease (a sensory - motor disorder inducing vertigo and kinetosis). Larval siblings of cichlid fish (Oreochromis mossambicus) were subjected to hypergravity (3g; 6 hours) during development and separated into normally and kinetotically swimming individuals following the transfer to 1g (i.e., stopping the centrifuge; kinetotically behaving fish performed spinning movements). Subsequently, CAH was histochemically demonstrated in inner ear ionocytes (cells involved in the endolymphatic ion exchange) and enzyme reactivity was determined densitometrically. The results showed that CAH-reactivity was significantly increased in normally behaving hyper-g specimens as compared to controls kept at 1g, whereas no difference in enzyme reactivity was evident between the controls and kinetotically behaving fish. On the background of earlier studies, according to which (1) hypergravity induces a decrease of otolith growth and (2) the otolithic calcium incorporation (visualized using the calcium -tracer alizarin complexone) of kinetotically swimming hyper - g fish was lower as compared to normally behaving hyper - g animals, the present study strongly supports the concept that an increase in CAH-reactivity may result in a decrease of otolithic calcium deposition. The mechanism regulating CAH-activity hitherto remains to be determined. Acknowledgement: This work was financially supported by the German Aerospace Center (DLR) (FKZ: 50 WB 9997).

  13. Normal and abnormal spine and thoracic cage development

    PubMed Central

    Canavese, Federico; Dimeglio, Alain

    2013-01-01

    Development of the spine and thoracic cage consists of a complex series of events involving multiple metabolic processes, genes and signaling pathways. During growth, complex phenomena occur in rapid succession. This succession of events, this establishment of elements, is programmed according to a hierarchy. These events are well synchronized to maintain harmonious limb, spine and thoracic cage relationships, as growth in the various body segments does not occur simultaneously at the same magnitude or rate. In most severe cases of untreated progressive early-onset spinal deformities, respiratory insufficiency and pulmonary and cardiac hypertension (cor pulmonale), which characterize thoracic insufficiency syndrome (TIS), can develop, sometimes leading to death. TIS is the inability of the thorax to ensure normal breathing. This clinical condition can be linked to costo-vertebral malformations (e.g., fused ribs, hemivertebrae, congenital bars), neuromuscular diseases (e.g., expiratory congenital hypotonia), Jeune or Jarcho-Levin syndromes or to 50% to 75% fusion of the thoracic spine before seven years of age. Complex spinal deformities alter normal growth plate development, and vertebral bodies become progressively distorted, perpetuating the disorder. Therefore, many scoliotic deformities can become growth plate disorders over time. This review aims to provide a comprehensive review of how spinal deformities can affect normal spine and thoracic cage growth. Previous conceptualizations are integrated with more recent scientific data to provide a better understanding of both normal and abnormal spine and thoracic cage growth. PMID:24147251

  14. Ear problems and injuries in athletes.

    PubMed

    Cassaday, Kacie; Vazquez, Gerardo; Wright, Justin M

    2014-01-01

    The ear is an unique organ--the principal structure involved in both hearing and balance. Although not common, problems with the ear may be encountered in specific sporting populations. Common conditions affecting the ear in the athlete include otitis externa, an infection of the external ear; external auditory canal exostoses, or abnormal bony growths in the canal; and otitis media, an infection of the middle ear. Given its position on the head, the ear is subject to trauma, often resulting in an auricular hematoma. Divers, due to pressure changes on descent and ascent, are subject to both ear barotrauma and ear decompression sickness. This article will discuss recognition, treatment, and prevention of these conditions affecting the ear in the athlete.

  15. Impact of Bug-in-Ear Professional Development on Early Childhood Co-Teachers' Use of Communication Strategies

    ERIC Educational Resources Information Center

    Ottley, Jennifer R.; Coogle, Christan G.; Rahn, Naomi L.; Spear, Caitlin F.

    2016-01-01

    The goal of this study was to build the capacity of early childhood teachers to implement evidence-based strategies. We investigated the efficacy of professional development with bug-in-ear peer coaching in improving teachers' use of communication strategies, the teachers' maintenance of strategies post intervention, and the social validity of the…

  16. Exceeding Parents' Expectations in Ear-Nose-Throat Outpatient Facilities: The Development and Analysis of a Questionnaire

    ERIC Educational Resources Information Center

    Margaritis, Eleftherios; Katharaki, Maria; Katharakis, George

    2012-01-01

    The study attempts to develop an outpatient service quality scale by investigating the key dimensions which assess parental satisfaction and provides a recommendation on an improved health service delivery system. The survey was conducted in an Ear-Nose-Throat outpatient clinic of a Greek public pediatric hospital. A total of 127 parents in…

  17. Homozygous HOXA1 mutations disrupt human brainstem, inner ear, cardiovascular and cognitive development.

    PubMed

    Tischfield, Max A; Bosley, Thomas M; Salih, Mustafa A M; Alorainy, Ibrahim A; Sener, Emin C; Nester, Michael J; Oystreck, Darren T; Chan, Wai-Man; Andrews, Caroline; Erickson, Robert P; Engle, Elizabeth C

    2005-10-01

    We identified homozygous truncating mutations in HOXA1 in three genetically isolated human populations. The resulting phenotype includes horizontal gaze abnormalities, deafness, facial weakness, hypoventilation, vascular malformations of the internal carotid arteries and cardiac outflow tract, mental retardation and autism spectrum disorder. This is the first report to our knowledge of viable homozygous truncating mutations in any human HOX gene and of a mendelian disorder resulting from mutations in a human HOX gene critical for development of the central nervous system. PMID:16155570

  18. Maternal immune activation and abnormal brain development across CNS disorders.

    PubMed

    Knuesel, Irene; Chicha, Laurie; Britschgi, Markus; Schobel, Scott A; Bodmer, Michael; Hellings, Jessica A; Toovey, Stephen; Prinssen, Eric P

    2014-11-01

    Epidemiological studies have shown a clear association between maternal infection and schizophrenia or autism in the progeny. Animal models have revealed maternal immune activation (mIA) to be a profound risk factor for neurochemical and behavioural abnormalities in the offspring. Microglial priming has been proposed as a major consequence of mIA, and represents a critical link in a causal chain that leads to the wide spectrum of neuronal dysfunctions and behavioural phenotypes observed in the juvenile, adult or aged offspring. Such diversity of phenotypic outcomes in the mIA model are mirrored by recent clinical evidence suggesting that infectious exposure during pregnancy is also associated with epilepsy and, to a lesser extent, cerebral palsy in children. Preclinical research also suggests that mIA might precipitate the development of Alzheimer and Parkinson diseases. Here, we summarize and critically review the emerging evidence that mIA is a shared environmental risk factor across CNS disorders that varies as a function of interactions between genetic and additional environmental factors. We also review ongoing clinical trials targeting immune pathways affected by mIA that may play a part in disease manifestation. In addition, future directions and outstanding questions are discussed, including potential symptomatic, disease-modifying and preventive treatment strategies.

  19. Numb is not a critical regulator of Notch-mediated cell fate decisions in the developing chick inner ear

    PubMed Central

    Eddison, Mark; Weber, Sara J.; Ariza-McNaughton, Linda; Lewis, Julian; Daudet, Nicolas

    2015-01-01

    The Notch signaling pathway controls differentiation of hair cells and supporting cells in the vertebrate inner ear. Here, we have investigated whether Numb, a known regulator of Notch activity in Drosophila, is involved in this process in the embryonic chick. The chicken homolog of Numb is expressed throughout the otocyst at early stages of development and is concentrated at the basal pole of the cells. It is asymmetrically allocated at some cell divisions, as in Drosophila, suggesting that it could act as a determinant inherited by one of the two daughter cells and favoring adoption of a hair-cell fate. To test the implication of Numb in hair cell fate decisions and the regulation of Notch signaling, we used different methods to overexpress Numb at different stages of inner ear development. We found that sustained or late Numb overexpression does not promote hair cell differentiation, and Numb does not prevent the reception of Notch signaling. Surprisingly, none of the Numb-overexpressing cells differentiated into hair cells, suggesting that high levels of Numb protein could interfere with intracellular processes essential for hair cell survival. However, when Numb was overexpressed early and more transiently during ear development, no effect on hair cell formation was seen. These results suggest that in the inner ear at least, Numb does not significantly repress Notch activity and that its asymmetric distribution in dividing precursor cells does not govern the choice between hair cell and supporting cell fates. PMID:25814931

  20. Development and evolution of the vestibular sensory apparatus of the mammalian ear

    PubMed Central

    Beisel, Kirk W.; Wang-Lundberg, Yesha; Maklad, Adel; Fritzsch, Bernd

    2014-01-01

    Herein, we will review molecular aspects of vestibular ear development and present them in the context of evolutionary changes and hair cell regeneration. Several genes guide the development of anterior and posterior canals. Although some of these genes are also important for horizontal canal development, this canal strongly depends on a single gene, Otx1. Otx1 also governs the segregation of saccule and utricle. Several genes are essential for otoconia and cupula formation, but protein interactions necessary to form and maintain otoconia or a cupula are not yet understood. Nerve fiber guidance to specific vestibular endorgans is predominantly mediated by diffusible neurotrophic factors that work even in the absence of differentiated hair cells. Neurotrophins, in particular Bdnf, are the most crucial attractive factor released by hair cells. If Bdnf is misexpressed, fibers can be redirected away from hair cells. Hair cell differentiation is mediated by Atoh1. However, Atoh1 may not initiate hair cell precursor formation. Resolving the role of Atoh1 in postmitotic hair cell precursors is crucial for future attempts in hair cell regeneration. Additional analyses are needed before gene therapy can help regenerate hair cells, restore otoconia, and reconnect sensory epithelia to the brain. PMID:16614470

  1. X Chromosome Abnormalities and Cognitive Development: Implications for Understanding Normal Human Development.

    ERIC Educational Resources Information Center

    Walzer, Stanley

    1985-01-01

    Argues that knowledge from studies of individuals with sex chromosome abnormalities can further understanding of aspects of normal human development. Studies of XO girls, XXY boys, XXX girls, and males with a fragile X chromosome are summarized to demonstrate how results contribute to knowledge about normal cognitive development and about…

  2. Abnormal Canine Bone Development Associated with Hypergravity Exposure

    NASA Technical Reports Server (NTRS)

    Morgan, J. P.; Fisher, G. L.; McNeill, K. L.; Oyama, J.

    1979-01-01

    Chronic centrifugation of 85- to 92-day-old Beagles at 2.0 x g and 2.6 x g for 26 weeks during the time of active skeletal growth caused skeletal abnormalities in the radius and the ulna of ten of 11 dogs. The pattern of change mimicked that found in naturally occurring and experimentally induced premature distal ulnar physeal closure or delayed growth at this physis. Minimal changes in bone density were detected by sensitive photon absorptiometric techniques. Skeletal abnormalities also were found in five of the six cage-control dogs, although the run-control dogs were radiographically normal.

  3. Development of inner ear afferent connections: forming primary neurons and connecting them to the developing sensory epithelia

    NASA Technical Reports Server (NTRS)

    Fritzsch, Bernd

    2003-01-01

    The molecular and cellular origin of the primary neurons of the inner ear, the vestibular and spiral neurons, is reviewed including how they connect to the specific sensory epithelia and what the molecular nature of their survival is. Primary neurons of the ear depend on a single basic Helix-Loop-Helix (bHLH) protein for their formation, neurogenin 1 (ngn1). An immediate downstream gene is the bHLH gene neuronal differentiation (NeuroD). Targeted null mutations of ngn1 results in absence of primary neuron formation; targeted null mutation of NeuroD results in loss of almost all spiral and many vestibular neurons. NeuroD and a later expressed gene, Brn3a, play a role in pathfinding to and within sensory epithelia. The molecular nature of this pathfinding property is unknown. Reduction of hair cells in ngn1 null mutations suggests a clonal relationship with primary neurons. This relationship may play some role in specifying the identity of hair cells and the primary neurons that connect with them. Primary neuron neurites growth to sensory epithelia is initially independent of trophic factors released from developing sensory epithelia, but becomes rapidly dependent on those factors. Null mutations of specific neurotrophic factors lose distinct primary neuron populations which undergo rapid embryonic cell death.

  4. Development of Abnormality Detection System for Bathers using Ultrasonic Sensors

    NASA Astrophysics Data System (ADS)

    Ohnishi, Yosuke; Abe, Takehiko; Nambo, Hidetaka; Kimura, Haruhiko; Ogoshi, Yasuhiro

    This paper proposes an abnormality detection system for bather sitting in bathtub. Increasing number of in-bathtub drowning accidents in Japan draws attention. Behind this large number of bathing accidents, Japan's unique social and cultural background come surface. For majority of people in Japan, bathing serves purpose in deep warming up of body, relax and enjoyable time. Therefore it is the custom for the Japanese to soak in bathtub. However overexposure to hot water may cause dizziness or fainting, which is possible to cause in-bathtub drowning. For drowning prevention, the system detects bather's abnormal state using an ultrasonic sensor array. The array, which has many ultrasonic sensors, is installed on the ceiling of bathroom above bathtub. The abnormality detection system uses the following two methods: posture detection and behavior detection. The function of posture detection is to estimate the risk of drowning by monitoring bather's posture. Meanwhile, the function of behavior detection is to estimate the risk of drowning by monitoring bather's behavior. By using these methods, the system detects bathers' different state from normal. As a result of experiment with a subject in the bathtub, the system was possible to detect abnormal state using subject's posture and behavior. Therefore the system is useful for monitoring bather to prevent drowning in bathtub.

  5. Exploring regulatory networks of miR-96 in the developing inner ear

    PubMed Central

    Lewis, Morag A.; Buniello, Annalisa; Hilton, Jennifer M.; Zhu, Fei; Zhang, William I.; Evans, Stephanie; van Dongen, Stijn; Enright, Anton J.; Steel, Karen P.

    2016-01-01

    Mutations in the microRNA Mir96 cause deafness in mice and humans. In the diminuendo mouse, which carries a single base pair change in the seed region of miR-96, the sensory hair cells crucial for hearing fail to develop fully and retain immature characteristics, suggesting that miR-96 is important for coordinating hair cell maturation. Our previous transcriptional analyses show that many genes are misregulated in the diminuendo inner ear and we report here further misregulated genes. We have chosen three complementary approaches to explore potential networks controlled by miR-96 using these transcriptional data. Firstly, we used regulatory interactions manually curated from the literature to construct a regulatory network incorporating our transcriptional data. Secondly, we built a protein-protein interaction network using the InnateDB database. Thirdly, gene set enrichment analysis was used to identify gene sets in which the misregulated genes are enriched. We have identified several candidates for mediating some of the expression changes caused by the diminuendo mutation, including Fos, Myc, Trp53 and Nr3c1, and confirmed our prediction that Fos is downregulated in diminuendo homozygotes. Understanding the pathways regulated by miR-96 could lead to potential therapeutic targets for treating hearing loss due to perturbation of any component of the network. PMID:26988146

  6. Exploring regulatory networks of miR-96 in the developing inner ear.

    PubMed

    Lewis, Morag A; Buniello, Annalisa; Hilton, Jennifer M; Zhu, Fei; Zhang, William I; Evans, Stephanie; van Dongen, Stijn; Enright, Anton J; Steel, Karen P

    2016-03-18

    Mutations in the microRNA Mir96 cause deafness in mice and humans. In the diminuendo mouse, which carries a single base pair change in the seed region of miR-96, the sensory hair cells crucial for hearing fail to develop fully and retain immature characteristics, suggesting that miR-96 is important for coordinating hair cell maturation. Our previous transcriptional analyses show that many genes are misregulated in the diminuendo inner ear and we report here further misregulated genes. We have chosen three complementary approaches to explore potential networks controlled by miR-96 using these transcriptional data. Firstly, we used regulatory interactions manually curated from the literature to construct a regulatory network incorporating our transcriptional data. Secondly, we built a protein-protein interaction network using the InnateDB database. Thirdly, gene set enrichment analysis was used to identify gene sets in which the misregulated genes are enriched. We have identified several candidates for mediating some of the expression changes caused by the diminuendo mutation, including Fos, Myc, Trp53 and Nr3c1, and confirmed our prediction that Fos is downregulated in diminuendo homozygotes. Understanding the pathways regulated by miR-96 could lead to potential therapeutic targets for treating hearing loss due to perturbation of any component of the network.

  7. Effect of dietary inclusion of whole ear corn silage on stomach development and gastric mucosa integrity of heavy pigs at slaughter.

    PubMed

    Mason, Federico; Pascotto, Ernesto; Zanfi, Cristina; Spanghero, Mauro

    2013-12-01

    The effect of dietary inclusion of whole ear corn silage on stomach development and on the incidence of gastric lesions was studied in heavy pigs. Three groups of 14 castrated male pigs were fed a control cereal-based diet and two diets containing whole ear corn silage (15% or 30% DM) from 90 kg bodyweight to slaughter at 170 kg. The diets with whole ear corn silage increased the amount of neutral detergent fibre in the stomach contents, the weight of the organs and the area of the pyloric region. Follicular gastritis was significantly lower and gastritis less severe in pigs fed the whole ear corn silage diets than pigs fed the control diet. The inclusion of whole ear corn silage in the diet influenced the development of the stomach and reduced the incidence of gastritis in heavy pigs.

  8. Ear emergencies

    MedlinePlus

    ... and ruptured eardrums can be caused by: Inserting cotton swabs, toothpicks, pins, pens, or other objects into ... The person will have severe pain. Place sterile cotton gently in the outer ear canal to keep ...

  9. Elephant ear

    MedlinePlus

    The harmful substances in elephant ear plants are: Oxalic acid Asparagine, a protein found in this plant Note: ... days to a week if treated correctly. Rarely, oxalic acid may cause swelling severe enough to block the ...

  10. Molecular Characterization and Expression Analysis of Triticum aestivum Squamosa-Promoter Binding Protein-Box Genes Involved in Ear Development

    PubMed Central

    Zhang, Bin; Liu, Xia; Zhao, Guangyao; Mao, Xinguo; Li, Ang; Jing, Ruilian

    2014-01-01

    Wheat (Triticum aestivum L.) is one of the most important crops in the world. Squamosa-promoter binding protein (SBP)-box genes play a critical role in regulating flower and fruit development. In this study, 10 novel SBP-box genes (TaSPL genes) were isolated from wheat ((Triticum aestivum L.) cultivar Yanzhan 4110). Phylogenetic analysis classified the TaSPL genes into five groups (G1–G5). The motif combinations and expression patterns of the TaSPL genes varied among the five groups with each having own distinctive characteristics: TaSPL20/21 in G1 and TaSPL17 in G2 mainly expressed in the shoot apical meristem and the young ear, and their expression levels responded to development of the ear; TaSPL6/15 belonging to G3 were upregulated and TaSPL1/23 in G4 were downregulated during grain development; the gene in G5 (TaSPL3) expressed constitutively. Thus, the consistency of the phylogenetic analysis, motif compositions, and expression patterns of the TaSPL genes revealed specific gene structures and functions. On the other hand, the diverse gene structures and different expression patterns suggested that wheat SBP-box genes have a wide range of functions. The results also suggest a potential role for wheat SBP-box genes in ear development. This study provides a significant beginning of functional analysis of SBP-box genes in wheat. PMID:24386921

  11. Molecular characterization and expression analysis of Triticum aestivum squamosa-promoter binding protein-box genes involved in ear development.

    PubMed

    Zhang, Bin; Liu, Xia; Zhao, Guangyao; Mao, Xinguo; Li, Ang; Jing, Ruilian

    2014-06-01

    Wheat (Triticum aestivum L.) is one of the most important crops in the world. Squamosa-promoter binding protein (SBP)-box genes play a critical role in regulating flower and fruit development. In this study, 10 novel SBP-box genes (TaSPL genes) were isolated from wheat ((Triticum aestivum L.) cultivar Yanzhan 4110). Phylogenetic analysis classified the TaSPL genes into five groups (G1-G5). The motif combinations and expression patterns of the TaSPL genes varied among the five groups with each having own distinctive characteristics: TaSPL20/21 in G1 and TaSPL17 in G2 mainly expressed in the shoot apical meristem and the young ear, and their expression levels responded to development of the ear; TaSPL6/15 belonging to G3 were upregulated and TaSPL1/23 in G4 were downregulated during grain development; the gene in G5 (TaSPL3) expressed constitutively. Thus, the consistency of the phylogenetic analysis, motif compositions, and expression patterns of the TaSPL genes revealed specific gene structures and functions. On the other hand, the diverse gene structures and different expression patterns suggested that wheat SBP-box genes have a wide range of functions. The results also suggest a potential role for wheat SBP-box genes in ear development. This study provides a significant beginning of functional analysis of SBP-box genes in wheat.

  12. Mammalian development does not recapitulate suspected key transformations in the evolutionary detachment of the mammalian middle ear.

    PubMed

    Ramírez-Chaves, Héctor E; Wroe, Stephen W; Selwood, Lynne; Hinds, Lyn A; Leigh, Chris; Koyabu, Daisuke; Kardjilov, Nikolay; Weisbecker, Vera

    2016-01-13

    The ectotympanic, malleus and incus of the developing mammalian middle ear (ME) are initially attached to the dentary via Meckel's cartilage, betraying their origins from the primary jaw joint of land vertebrates. This recapitulation has prompted mostly unquantified suggestions that several suspected--but similarly unquantified--key evolutionary transformations leading to the mammalian ME are recapitulated in development, through negative allometry and posterior/medial displacement of ME bones relative to the jaw joint. Here we show, using µCT reconstructions, that neither allometric nor topological change is quantifiable in the pre-detachment ME development of six marsupials and two monotremes. Also, differential ME positioning in the two monotreme species is not recapitulated. This challenges the developmental prerequisites of widely cited evolutionary scenarios of definitive mammalian middle ear (DMME) evolution, highlighting the requirement for further fossil evidence to test these hypotheses. Possible association between rear molar eruption, full ME ossification and ME detachment in marsupials suggests functional divergence between dentary and ME as a trigger for developmental, and possibly also evolutionary, ME detachment. The stable positioning of the dentary and ME supports suggestions that a 'partial mammalian middle ear' as found in many mammaliaforms--probably with a cartilaginous Meckel's cartilage--represents the only developmentally plausible evolutionary DMME precursor.

  13. Mammalian development does not recapitulate suspected key transformations in the evolutionary detachment of the mammalian middle ear.

    PubMed

    Ramírez-Chaves, Héctor E; Wroe, Stephen W; Selwood, Lynne; Hinds, Lyn A; Leigh, Chris; Koyabu, Daisuke; Kardjilov, Nikolay; Weisbecker, Vera

    2016-01-13

    The ectotympanic, malleus and incus of the developing mammalian middle ear (ME) are initially attached to the dentary via Meckel's cartilage, betraying their origins from the primary jaw joint of land vertebrates. This recapitulation has prompted mostly unquantified suggestions that several suspected--but similarly unquantified--key evolutionary transformations leading to the mammalian ME are recapitulated in development, through negative allometry and posterior/medial displacement of ME bones relative to the jaw joint. Here we show, using µCT reconstructions, that neither allometric nor topological change is quantifiable in the pre-detachment ME development of six marsupials and two monotremes. Also, differential ME positioning in the two monotreme species is not recapitulated. This challenges the developmental prerequisites of widely cited evolutionary scenarios of definitive mammalian middle ear (DMME) evolution, highlighting the requirement for further fossil evidence to test these hypotheses. Possible association between rear molar eruption, full ME ossification and ME detachment in marsupials suggests functional divergence between dentary and ME as a trigger for developmental, and possibly also evolutionary, ME detachment. The stable positioning of the dentary and ME supports suggestions that a 'partial mammalian middle ear' as found in many mammaliaforms--probably with a cartilaginous Meckel's cartilage--represents the only developmentally plausible evolutionary DMME precursor. PMID:26763693

  14. Bearded-Ear Encodes a MADS-box Transcription Factor Critical for Maize Floral Development

    Technology Transfer Automated Retrieval System (TEKTRAN)

    We cloned bde by positional cloning and found that it encodes zag3, a MADS-box transcription factor in the conserved AGL6 clade. Mutants in the maize homolog of AGAMOUS, zag1, have a subset of bde floral defects. bde; zag1 double mutants have a severe ear phenotype, not observed in either single m...

  15. Normal and abnormal neuronal migration in the developing cerebral cortex.

    PubMed

    Sun, Xue-Zhi; Takahashi, Sentaro; Cui, Chun; Zhang, Rui; Sakata-Haga, Hiromi; Sawada, Kazuhiko; Fukui, Yoshihiro

    2002-08-01

    Neuronal migration is the critical cellular process which initiates histogenesis of cerebral cortex. Migration involves a series of complex cell interactions and transformation. After completing their final mitosis, neurons migrate from the ventricular zone into the cortical plate, and then establish neuronal lamina and settle onto the outermost layer, forming an "inside-out" gradient of maturation. This process is guided by radial glial fibers, requires proper receptors, ligands, other unknown extracellular factors, and local signaling to stop neuronal migration. This process is also highly sensitive to various physical, chemical and biological agents as well as to genetic mutations. Any disturbance of the normal process may result in neuronal migration disorder. Such neuronal migration disorder is believed as major cause of both gross brain malformation and more special cerebral structural and functional abnormalities in experimental animals and in humans. An increasing number of instructive studies on experimental models and several genetic model systems of neuronal migration disorder have established the foundation of cortex formation and provided deeper insights into the genetic and molecular mechanisms underlying normal and abnormal neuronal migration.

  16. Identification of miRNAs and their target genes in developing maize ears by combined small RNA and degradome sequencing

    PubMed Central

    2014-01-01

    Background In plants, microRNAs (miRNAs) are endogenous ~22 nt RNAs that play important regulatory roles in many aspects of plant biology, including metabolism, hormone response, epigenetic control of transposable elements, and stress response. Extensive studies of miRNAs have been performed in model plants such as rice and Arabidopsis thaliana. In maize, most miRNAs and their target genes were analyzed and identified by clearly different treatments, such as response to low nitrate, salt and drought stress. However, little is known about miRNAs involved in maize ear development. The objective of this study is to identify conserved and novel miRNAs and their target genes by combined small RNA and degradome sequencing at four inflorescence developmental stages. Results We used deep-sequencing, miRNA microarray assays and computational methods to identify, profile, and describe conserved and non-conserved miRNAs at four ear developmental stages, which resulted in identification of 22 conserved and 21-maize-specific miRNA families together with their corresponding miRNA*. Comparison of miRNA expression in these developmental stages revealed 18 differentially expressed miRNA families. Finally, a total of 141 genes (251 transcripts) targeted by 102 small RNAs including 98 miRNAs and 4 ta-siRNAs were identified by genomic-scale high-throughput sequencing of miRNA cleaved mRNAs. Moreover, the differentially expressed miRNAs-mediated pathways that regulate the development of ears were discussed. Conclusions This study confirmed 22 conserved miRNA families and discovered 26 novel miRNAs in maize. Moreover, we identified 141 target genes of known and new miRNAs and ta-siRNAs. Of these, 72 genes (117 transcripts) targeted by 62 differentially expressed miRNAs may attribute to the development of maize ears. Identification and characterization of these important classes of regulatory genes in maize may improve our understanding of molecular mechanisms controlling ear development

  17. Immortalized Mouse Inner Ear Cell Lines Demonstrate a Role for Chemokines in Promoting the Growth of Developing Statoacoustic Ganglion Neurons

    PubMed Central

    Daruwalla, Zeeba; Roth, Therese M.; Attia, Naweah P.; Lukacs, Nicholas W.; Richards, Ayo-Lynn; White, Ian O.; Allen, Susan J.; Barald, Kate F.

    2005-01-01

    The target-derived factors necessary for promoting initial outgrowth from the statoacoustic ganglion (SAG) to the inner ear have not been fully characterized. In the present study, conditioned medium from embryonic Immortomouse inner ear cell lines that maintain many characteristics of developing inner ear sensory epithelia were screened for neurite-promoting activity. Conditioned medium found to be positive for promoting SAG neurite outgrowth and neuronal survival was then tested for the presence of chemokines, molecules that have not previously been investigated for promoting SAG outgrowth. One candidate molecule, monocyte chemotactic protein 1 (MCP-1), was detected in the conditioned medium and subsequently localized to mouse hair cells by immunocytochemistry. In vitro studies demonstrated that function-blocking MCP-1 antibodies decreased the amount of SAG neurite outgrowth induced by the conditioned medium and that subsequent addition of MCP-1 protein was able to promote outgrowth when added to the antibody-treated conditioned medium. The use of the Immortomouse cell lines proved valuable in identifying this candidate cofactor that promotes outgrowth of early-stage SAG nerve fibers and is expressed in embryonic hair cells. PMID:16240240

  18. Surgical correction of constricted ear combined with Stahl's ear.

    PubMed

    Bi, Ye; Lin, Lin; Yang, Qinhua; Pan, Bo; Zhao, Yanyong; He, Leren; Jiang, Haiyue

    2015-07-01

    Constricted ear combined with Stahl's ear is a rare ear deformity, which is a kind of complex congenital auricular deformity. From 1 January 2007 to 1 January 2014, 19 patients with constricted ear combined with Stahl's ear (Spock ear) were enrolled in this study, most of which were unilaterally deformed. To correct the deformity, a double Z-shaped skin incision was made on the posterior side of the auricle, with the entire layer of cartilage cut parallel to the helix traversing the third crus to form a fan-shaped cartilage flap. The superior crura of the antihelix were shaped by the folding cartilage rim. The cartilage of the abnormal third crus was made part of the new superior crura of antihelix, and the third crus was eliminated. The postoperative aesthetic assessment of the reshaped auricle was graded by both doctors and patients (or their parents). Out of the 19 patients, the number of satisfying cases of the symmetry, helix stretch, elimination of the third crus, the cranioauricular angle, and the substructure of the reshaped ears was 14 (nine excellent and five good), 16 (six excellent and 10 good), 17 (eight excellent and nine good), 15 (five excellent and 10 good), and 13 (two excellent and 11 good), respectively. With a maximum of a 90-month follow-up, no complication was observed. The results of the study suggested that this rare deformity could be corrected by appropriate surgical treatment, with a satisfied postoperative appearance.

  19. Abnormal ventricular development in preterm neonates with visually normal MRIs

    NASA Astrophysics Data System (ADS)

    Shi, Jie; Wang, Yalin; Lao, Yi; Ceschin, Rafael; Mi, Liang; Nelson, Marvin D.; Panigrahy, Ashok; Leporé, Natasha

    2015-12-01

    Children born preterm are at risk for a wide range of neurocognitive and neurobehavioral disorders. Some of these may stem from early brain abnormalities at the neonatal age. Hence, a precise characterization of neonatal neuroanatomy may help inform treatment strategies. In particular, the ventricles are often enlarged in neurocognitive disorders, due to atrophy of surrounding tissues. Here we present a new pipeline for the detection of morphological and relative pose differences in the ventricles of premature neonates compared to controls. To this end, we use a new hyperbolic Ricci flow based mapping of the ventricular surfaces of each subjects to the Poincaré disk. Resulting surfaces are then registered to a template, and a between group comparison is performed using multivariate tensor-based morphometry. We also statistically compare the relative pose of the ventricles within the brain between the two groups, by performing a Procrustes alignment between each subject's ventricles and an average shape. For both types of analyses, differences were found in the left ventricles between the two groups.

  20. Ear examination

    MedlinePlus

    The ear canal differs in size, shape, and color from person to person. Normally, the canal is skin-colored and has small hairs. Yellowish-brown earwax may be present. The eardrum is a light-gray color or a shiny pearly-white. Light should reflect off ...

  1. Cosmetic ear surgery

    MedlinePlus

    Otoplasty; Ear pinning; Ear surgery - cosmetic; Ear reshaping; Pinnaplasty ... Cosmetic ear surgery may be done in the surgeon's office, an outpatient clinic, or a hospital. It can be performed under ...

  2. Ear Plastic Surgery

    MedlinePlus

    ... Meeting Calendar Find an ENT Doctor Near You Ear Plastic Surgery Ear Plastic Surgery Patient Health Information ... they may improve appearance and self-confidence. Can Ear Deformities Be Corrected? Formation of the ear during ...

  3. Autoimmune Inner Ear Disease

    MedlinePlus

    ... Find an ENT Doctor Near You Autoimmune Inner Ear Disease Autoimmune Inner Ear Disease Patient Health Information ... with a hearing loss. How Does the Healthy Ear Work? The ear has three main parts: the ...

  4. Better Ear Health

    MedlinePlus

    ... Calendar Find an ENT Doctor Near You Better Ear Health Better Ear Health Patient Health Information News ... often helpful to those with this condition. Swimmer’s Ear An infection of the outer ear structures caused ...

  5. How the Ear Works

    MedlinePlus

    ... Find an ENT Doctor Near You How the Ear Works How the Ear Works Patient Health Information News media interested in ... public relations staff at newsroom@entnet.org . The ear has three main parts: the outer ear (including ...

  6. The influence of brain abnormalities on psychosocial development, criminal history and paraphilias in sexual murderers.

    PubMed

    Briken, Peer; Habermann, Niels; Berner, Wolfgang; Hill, Andreas

    2005-09-01

    The aim of this study was to investigate the number and type of brain abnormalities and their influence on psychosocial development, criminal history and paraphilias in sexual murderers. We analyzed psychiatric court reports of 166 sexual murderers and compared a group with notable signs of brain abnormalities (N = 50) with those without any signs (N = 116). Sexual murderers with brain abnormalities suffered more from early behavior problems. They were less likely to cohabitate with the victim at the time of the homicide and had more victims at the age of six years or younger. Psychiatric diagnoses revealed a higher total number of paraphilias: Transvestic fetishism and paraphilias not otherwise specified were more frequent in offenders with brain abnormalities. A binary logistic regression identified five predictors that accounted for 46.8% of the variance explaining the presence of brain abnormalities. Our results suggest the importance of a comprehensive neurological and psychological examination of this special offender group. PMID:16225232

  7. Constitutive Notch Signaling Causes Abnormal Development of the Oviducts, Abnormal Angiogenesis, and Cyst Formation in Mouse Female Reproductive Tract.

    PubMed

    Ferguson, Lydia; Kaftanovskaya, Elena M; Manresa, Carmen; Barbara, Agustin M; Poppiti, Robert J; Tan, Yingchun; Agoulnik, Alexander I

    2016-03-01

    The Notch signaling pathway is critical for the differentiation of many tissues and organs in the embryo. To study the consequences of Notch1 gain-of-function signaling on female reproductive tract development, we used a cre-loxP strategy and Amhr2-cre transgene to generate mice with conditionally activated Notch1 (Rosa(Notch1)). The Amhr2-cre transgene is expressed in the mesenchyme of developing female reproductive tract and in granulosa cells in the ovary. Double transgenic Amhr2-cre, Rosa(Notch1) females were infertile, whereas control Rosa(Notch1) mice had normal fertility. All female reproductive organs in mutants showed hemorrhaging of blood vessels progressing with age. The mutant oviducts did not develop coiling, and were instead looped around the ovary. There were multiple blockages in the lumen along the oviduct length, creating a barrier for sperm or oocyte passage. Mutant females demonstrated inflamed uteri with increased vascularization and an influx of inflammatory cells. Additionally, older females developed ovarian, oviductal, and uterine cysts. The significant change in gene expression was detected in the mutant oviduct expression of Wnt4, essential for female reproductive tract development. Similar oviductal phenotypes have been detected previously in mice with activated Smo and in beta-catenin, Wnt4, Wnt7a, and Dicer conditional knockouts, indicating a common regulatory pathway disrupted by these genetic abnormalities. PMID:26843448

  8. [Inner Ear Hearing Loss].

    PubMed

    Hesse, G

    2016-06-01

    Hearing loss is one of the most dominant handicaps in modern societies, which additionally very often is not realized or not admitted. About one quarter of the general population suffers from inner ear hearing loss and is therefore restricted in communicational skills. Demographic factors like increasing age play an important role as well as environmental influences and an increasing sound and noise exposure especially in leisure activities. Thus borders between a "classical" presbyacusis - if it ever existed - and envirionmentally induced hearing loss disappear. Today restrictions in hearing ability develop earlier in age but at the same time they are detected and diagnosed earlier. This paper can eventually enlighten the wide field of inner ear hearing loss only fragmentarily; therefore mainly new research, findings and developments are reviewed. The first part discusses new aspects of diagnostics of inner ear hearing loss and different etiologies. PMID:27259171

  9. Contemporary issues in the management of abnormal placentation during pregnancy in developing nations: An Indian perspective.

    PubMed

    Bajwa, Sukhwinder Kaur; Singh, Anita; Bajwa, Sukhminder Jit Singh

    2013-07-01

    The gap between the developed and developing nations with regards to maternal mortality and morbidity may have narrowed but still a lot of dedicated work is required to bridge these differences. Obstetrical haemorrhage is the leading cause of maternal deaths in these developing nations especially in India. The most common causes of this fatal haemorrhage are the placental abnormalities which rarely get detected before delivery. Numerous factors have been incremental in the causation of this abnormal placental implantation with resultant complications. The present article is an attempt to review possible predictors of abnormal placental implantation. Also, a genuine attempt has been made to enumerate possible measures to identify the predictors of abnormal placentation during early pregnancy and their suitable prevention and management.

  10. Mixing model systems: Using zebrafish and mouse inner ear mutants and other organ systems to unravel the mystery of otoconial development

    PubMed Central

    Hughes, Inna; Thalmann, Isolde; Thalmann, Ruediger; Ornitz, David M.

    2007-01-01

    Human vestibular dysfunction is an increasing clinical problem. Degeneration or displacement of otoconia is a significant etiology of age-related balance disorders and Benign Positional Vertigo (BPV). In addition, commonly used antibiotics, such as aminoglycoside antibiotics, can lead to disruption of otoconial structure and function. Despite such clinical significance, relatively little information has been compiled about the development and maintenance of otoconia in humans. Recent studies in model organisms and other mammalian organ systems have revealed some of the proteins and processes required for the normal biomineralization of otoconia and otoliths in the inner ear of vertebrates. Orchestration of extracellular biomineralization requires bringing together ionic and proteinaceous components in time and space. Coordination of these events requires the normal formation of the otocyst and sensory maculae, specific secretion and localization of extracellular matrix proteins, as well as tight regulation of the endolymph ionic environment. Disruption of any of these processes can lead to the formation of abnormally shaped, or ectopic, otoconia, or otoconial agenesis. We propose that normal generation of otoconia requires a complex temporal and spatial control of developmental and biochemical events. In this review, we suggest a new hypothetical model for normal otoconial and otolith formation based on matrix vesicle mineralization in bone which we believe to be supported by information from existing mutants, morphants, and biochemical studies. PMID:16529728

  11. The Boron Efflux Transporter ROTTEN EAR Is Required for Maize Inflorescence Development and Fertility[C][W][OPEN

    PubMed Central

    Chatterjee, Mithu; Tabi, Zara; Galli, Mary; Malcomber, Simon; Buck, Amy; Muszynski, Michael; Gallavotti, Andrea

    2014-01-01

    Although boron has a relatively low natural abundance, it is an essential plant micronutrient. Boron deficiencies cause major crop losses in several areas of the world, affecting reproduction and yield in diverse plant species. Despite the importance of boron in crop productivity, surprisingly little is known about its effects on developing reproductive organs. We isolated a maize (Zea mays) mutant, called rotten ear (rte), that shows distinct defects in vegetative and reproductive development, eventually causing widespread sterility in its inflorescences, the tassel and the ear. Positional cloning revealed that rte encodes a membrane-localized boron efflux transporter, co-orthologous to the Arabidopsis thaliana BOR1 protein. Depending on the availability of boron in the soil, rte plants show a wide range of phenotypic defects that can be fully rescued by supplementing the soil with exogenous boric acid, indicating that rte is crucial for boron transport into aerial tissues. rte is expressed in cells surrounding the xylem in both vegetative and reproductive tissues and is required for meristem activity and organ development. We show that low boron supply to the inflorescences results in widespread defects in cell and cell wall integrity, highlighting the structural importance of boron in the formation of fully fertile reproductive organs. PMID:25035400

  12. A multicolor carbocyanine dye analysis of the development of the vestibular projections from canal and otolithic endorgans of the ear.

    NASA Astrophysics Data System (ADS)

    Fritzsch, B.; Maklad, A.

    The vestibular apparatus of the ear consists of two distinct sensory organs, the angular acceleration perceiving canal organs and the gravity and linear acceleration sensing utricle and saccule. Previous work has shown that microgravity or hypergravity affects the pattern of movement of pregnant rats and thus the way the ear is stimulated. Understanding how this activity affects the pattern of projections requires a more detailed understanding of the central projections, in particular during development. We report data generated with a technique using old and new carbocyanine dyes. The properties of these dyes to diffuse in the lipid bilayer of aldehyde fixed neurons make them ideal for such studies. Our data show that the initial projection develops prior to the onset of functional input from the endorgans and is likely governed exclusively by genetic developmental programs. However, our data also provide two aspects of the neonatal segregation that might be influenced by activity. One of these areas o continued segregation is in thef brainstem, specifically the rostral part of the medial vestibular nucleus. The other part is in the cerebellum, specifically the nodulus and flocculus. These areas show more overlap in the embryos that progressively is reduced during further development suggesting the possible influence of activity in this process. Supported by NASA (NAG 2-1353).

  13. Developmental vitamin D deficiency causes abnormal brain development.

    PubMed

    Eyles, D W; Feron, F; Cui, X; Kesby, J P; Harms, L H; Ko, P; McGrath, J J; Burne, T H J

    2009-12-01

    There is now clear evidence that vitamin D is involved in brain development. Our group is interested in environmental factors that shape brain development and how this may be relevant to neuropsychiatric diseases including schizophrenia. The origins of schizophrenia are considered developmental. We hypothesised that developmental vitamin D (DVD) deficiency may be the plausible neurobiological explanation for several important epidemiological correlates of schizophrenia namely: (1) the excess winter/spring birth rate, (2) increased incidence of the disease in 2nd generation Afro-Caribbean migrants and (3) increased urban birth rate. Moreover we have published two pieces of direct epidemiological support for this hypothesis in patients. In order to establish the "Biological Plausibility" of this hypothesis we have developed an animal model to study the effect of DVD deficiency on brain development. We do this by removing vitamin D from the diet of female rats prior to breeding. At birth we return all dams to a vitamin D containing diet. Using this procedure we impose a transient, gestational vitamin D deficiency, while maintaining normal calcium levels throughout. The brains of offspring from DVD-deficient dams are characterised by (1) a mild distortion in brain shape, (2) increased lateral ventricle volumes, (3) reduced differentiation and (4) diminished expression of neurotrophic factors. As adults, the alterations in ventricular volume persist and alterations in brain gene and protein expression emerge. Adult DVD-deficient rats also display behavioural sensitivity to agents that induce psychosis (the NMDA antagonist MK-801) and have impairments in attentional processing. In this review we summarise the literature addressing the function of vitamin D on neuronal and non-neuronal cells as well as in vivo results from DVD-deficient animals. Our conclusions from these data are that vitamin D is a plausible biological risk factor for neuropsychiatric disorders and that

  14. Environmental Enteropathy: Elusive but Significant Subclinical Abnormalities in Developing Countries.

    PubMed

    Watanabe, Koji; Petri, William A

    2016-08-01

    Environmental enteropathy/Environmental enteric dysfunction (EE/EED) is a chronic disease of small intestine characterized by gut inflammation and barrier disruption, malabsorption and systemic inflammation in the absence of diarrhea. It is predominantly diseases of children in low income countries and is hypothesized to be caused by continuous exposure to fecally contaminated food, water and fomites. It had not been recognized as a priority health issue because it does not cause overt symptoms and was seen in apparently healthy individuals. However, there is a growing concern of EE/EED because of its impact on longitudinal public health issues, such as growth faltering, oral vaccine low efficacy and poor neurocognitive development. Recent works have provided important clues to unravel its complex pathogenesis, and suggest possible strategies for controlling EE/EED. However, effective diagnostic methods and interventions remain unsettled. Here, we review the existing literature, especially about its pathogenesis, and discuss a solution for children living in the developing world.

  15. Benign ear cyst or tumor

    MedlinePlus

    Osteomas; Exostoses; Tumor - ear; Cysts - ear; Ear cysts; Ear tumors; Bony tumor of the ear canal ... bony tumors of the ear canal (exostoses and osteomas) are caused by excess growth of bone. Repeated ...

  16. Closure of the middle ear with special reference to the development of the tegmen tympani of the temporal bone

    PubMed Central

    Rodríguez-Vázquez, José Francisco; Murakami, Gen; Verdugo-López, Samuel; Abe, Shin-ichi; Fujimiya, Mineko

    2011-01-01

    Closure of the middle ear is believed to be closely related to the evolutionary development of the mammalian jaw. However, few comprehensive descriptions are available on fetal development. We examined paraffin-embedded specimens of 20 mid-term human fetuses at 8–25 weeks of ovulation age (crown-rump length or CRL, 38–220 mm). After 9 weeks, the tympanic bone and the squamous part of the temporal bone, each of which was cranial or caudal to Meckel's cartilage, grew to close the lateral part of the tympanosquamosal fissure. At the same time, the cartilaginous tegmen tympani appeared independently of the petrous part of the temporal bone and resulted in the petrosquamosal fissure. Subsequently, the medial part of the tympanosquamosal fissure was closed by the descent of a cartilaginous inferior process of the tegmen tympani. When Meckel's cartilage changed into the sphenomandibular ligament and the anterior ligament of the malleus, the inferior process of the tegmen tympani interposed between the tympanic bone and the squamous part of the temporal bone, forming the petrotympanic fissure for the chorda tympani nerve and the discomalleolar ligament. Therefore, we hypothesize that, in accordance with the regression of Meckel's cartilage, the rapidly growing temporomandibular joint provided mechanical stress that accelerated the growth and descent of the inferior process of the tegmen tympani via the discomalleolar ligament. The usual diagram showing bony fissures around the tegmen tympani may overestimate the role of the tympanic bone in the fetal middle-ear closure. PMID:21477146

  17. Environmental Enteropathy: Elusive but Significant Subclinical Abnormalities in Developing Countries.

    PubMed

    Watanabe, Koji; Petri, William A

    2016-08-01

    Environmental enteropathy/Environmental enteric dysfunction (EE/EED) is a chronic disease of small intestine characterized by gut inflammation and barrier disruption, malabsorption and systemic inflammation in the absence of diarrhea. It is predominantly diseases of children in low income countries and is hypothesized to be caused by continuous exposure to fecally contaminated food, water and fomites. It had not been recognized as a priority health issue because it does not cause overt symptoms and was seen in apparently healthy individuals. However, there is a growing concern of EE/EED because of its impact on longitudinal public health issues, such as growth faltering, oral vaccine low efficacy and poor neurocognitive development. Recent works have provided important clues to unravel its complex pathogenesis, and suggest possible strategies for controlling EE/EED. However, effective diagnostic methods and interventions remain unsettled. Here, we review the existing literature, especially about its pathogenesis, and discuss a solution for children living in the developing world. PMID:27495791

  18. Prominent ears: Anthropometric study of the external ear of primary school children of Harare, Zimbabwe

    PubMed Central

    Muteweye, Wilfred; Muguti, Godfrey I.

    2015-01-01

    Background Prominent ear is the most common congenital ear deformity affecting 5% of children in the Western world and has profound psychosocial effects on the bearer. It is important to know the prevalence in the local population to have a better appreciation of the local burden of the abnormality as well as to know the parameters of ear morphology locally. These parameters can be useful in the diagnosis and evaluation of ear anomalies and may help reconstructive surgeons in reproducing an anatomically correct ear of an African/Zimbabwean child. Objectives To evaluate the frequency of prominent ears in black school going children in Zimbabwe and to establish morphometric properties of the ear. Design Prospective observational, cross sectional study. Setting Three Primary schools in Harare. Two in a high density area and one in a low density area. Materials and methods Three Primary schools in Harare were selected at random. The following measurements were taken: ear lengths, ear projection and face height using a sliding caliper. Three hundred and five healthy pupils of the age range 9–13 years of both sexes were included in the study, whilst children with congenital anomalies, ear tumours and history of ear trauma were excluded. Results The mean ear height across the cohort was 56.95 ± 5.00 (right ear) and 56.86 ± 4.92 (left ear). Ear projection was 19.52 ± 2.14 (right ear) and 19.59 ± 2.09 (left ear). Gender related differences were noted. Mean ear height was significantly higher in males (p-value = 0.000). Ear projection was higher in males compared to females. A total of 6.89% had prominent ears. Among males, 7.69% had prominent ears whilst 6.17% of females had prominent ears. Conclusion The prevalence of prominent ear among black African children in the studied population is comparable to that of Caucasians. The study provides a set of biometric data of auricular dimensions for normal black African children aged 9–13 years. PMID:26468372

  19. Transgenic mice overexpressing reticulon 3 develop neuritic abnormalities

    PubMed Central

    Hu, Xiangyou; Shi, Qi; Zhou, Xiangdong; He, Wanxia; Yi, Hong; Yin, Xinghua; Gearing, Marla; Levey, Allan; Yan, Riqiang

    2007-01-01

    Dystrophic neurites are swollen dendrites or axons recognizable near amyloid plaques as a part of important pathological feature of Alzheimer's disease (AD). We report herein that reticulon 3 (RTN3) is accumulated in a distinct population of dystrophic neurites named as RTN3 immunoreactive dystrophic neurites (RIDNs). The occurrence of RIDNs is concomitant with the formation of high-molecular-weight RTN3 aggregates in brains of AD cases and mice expressing mutant APP. Ultrastructural analysis confirms accumulation of RTN3-containing aggregates in RIDNs. It appears that the protein level of RTN3 governs the formation of RIDNs because transgenic mice expressing RTN3 will develop RIDNs, initially in the hippocampal CA1 region, and later in other hippocampal and cortical regions. Importantly, we show that the presence of dystrophic neurites in Tg-RTN3 mice causes impairments in spatial learning and memory, as well as synaptic plasticity, implying that RIDNs potentially contribute to AD cognitive dysfunction. Together, we demonstrate that aggregation of RTN3 contributes to AD pathogenesis by inducing neuritic dystrophy. Inhibition of RTN3 aggregation is likely a therapeutic approach for reducing neuritic dystrophy. PMID:17476306

  20. Listening to the Ear

    NASA Astrophysics Data System (ADS)

    Shera, Christopher Alan

    Otoacoustic emissions demonstrate that the ear creates sound while listening to sound, offering a promising acoustic window on the mechanics of hearing in awake, listening human beings. That window is clouded, however, by an incomplete knowledge of wave reflection and transmission, both forth and back within the cochlea and through the middle ear. This thesis "does windows," addressing wave propagation and scattering on both sides of the middle ear. A summary of highlights follows. Measurements of the cochlear input impedance in cat are used to identify a new symmetry in cochlear mechanics--termed "tapering symmetry" after its geometric interpretation in simple models--that guarantees that the wavelength of the traveling wave changes slowly with position near the stapes. Waves therefore propagate without reflection through the basal turns of the cochlea. Analytic methods for solving the cochlear wave equations using a perturbative scattering series are given and used to demonstrate that, contrary to common belief, conventional cochlear models exhibit negligible internal reflection whether or not they accurately represent the tapering symmetries of the inner ear. Frameworks for the systematic "deconstruction" of eardrum and middle-ear transduction characteristics are developed and applied to the analysis of noninvasive measurements of middle-ear and cochlear mechanics. A simple phenomenological model of inner-ear compressibility that correctly predicts hearing thresholds in patients with missing or disarticulated middle-ear ossicles is developed and used to establish an upper bound on cochlear compressibility several orders of magnitude smaller than that provided by direct measurements. Accurate measurements of stimulus -frequency evoked otoacoustic emissions are performed and used to determine the form and frequency variation of the cochlear traveling-wave ratio noninvasively. Those measurements are inverted to obtain the spatial distribution of mechanical

  1. Listening to the ear

    NASA Astrophysics Data System (ADS)

    Shera, Christopher A.

    Otoacoustic emissions demonstrate that the ear creates sound while listening to sound, offering a promising acoustic window on the mechanics of hearing in awake, listening human beings. That window is clouded, however, by an incomplete knowledge of wave reflection and transmission, both forth and back within the cochlea and through the middle ear. This thesis "does windows," addressing wave propagation and scattering on both sides of the middle ear. A summary of highlights follows. Measurements of the cochlear input impedance in cat are used to identify a new symmetry in cochlear mechanics-termed "tapering symmetry" after its geometric interpretation in simple models-that guarantees that the wavelength of the traveling wave changes slowly with position near the stapes. Waves therefore propagate without reflection through the basal turns of the cochlea. Analytic methods for solving the cochlear wave equations using a perturbative scattering series are given and used to demonstrate that, contrary to common belief, conventional cochlear models exhibit negligible internal reflection whether or not they accurately represent the tapering symmetries of the inner ear. Frameworks for the systematic "deconstruction" of eardrum and middle-ear transduction characteristics are developed and applied to the analysis of noninvasive measurements of middle-ear and cochlear mechanics. A simple phenomenological model of inner-ear compressibility that correctly predicts hearing thresholds in patients with missing or disarticulated middle-ear ossicles is developed and used to establish an upper bound on cochlear compressibility several orders of magnitude smaller than that provided by direct measurements. Accurate measurements of stimulus frequency evoked otoacoustic emissions are performed and used to determine the form and frequency variation of the cochlear traveling-wave ratio noninvasively. Those measurements are inverted to obtain the spatial distribution of mechanical

  2. Salivary gland choristoma of the middle ear.

    PubMed

    Bottrill, I D; Chawla, O P; Ramsay, A D

    1992-07-01

    A salivary gland choristoma is an extremely uncommon tumour in the middle ear space. It appears to be a developmental abnormality and may be associated with abnormalities of adjacent structures. It usually presents with unilateral conductive deafness which may be long-standing and the tumour often pursues a benign, slow growing course. It is usually possible to excise it, but problems may arise as there may be variable associated anatomical abnormalities of the middle ear. We present the nineteenth recorded case, review the literature and discuss the management of this condition.

  3. RAF Kinase Activity Regulates Neuroepithelial Cell Proliferation and Neuronal Progenitor Cell Differentiation during Early Inner Ear Development

    PubMed Central

    Magariños, Marta; Aburto, María R.; Sánchez-Calderón, Hortensia; Muñoz-Agudo, Carmen; Rapp, Ulf R.; Varela-Nieto, Isabel

    2010-01-01

    Background Early inner ear development requires the strict regulation of cell proliferation, survival, migration and differentiation, coordinated by the concerted action of extrinsic and intrinsic factors. Deregulation of these processes is associated with embryonic malformations and deafness. We have shown that insulin-like growth factor I (IGF-I) plays a key role in embryonic and postnatal otic development by triggering the activation of intracellular lipid and protein kinases. RAF kinases are serine/threonine kinases that regulate the highly conserved RAS-RAF-MEK-ERK signaling cascade involved in transducing the signals from extracellular growth factors to the nucleus. However, the regulation of RAF kinase activity by growth factors during development is complex and still not fully understood. Methodology/Principal Findings By using a combination of qRT-PCR, Western blotting, immunohistochemistry and in situ hybridization, we show that C-RAF and B-RAF are expressed during the early development of the chicken inner ear in specific spatiotemporal patterns. Moreover, later in development B-RAF expression is associated to hair cells in the sensory patches. Experiments in ex vivo cultures of otic vesicle explants demonstrate that the influence of IGF-I on proliferation but not survival depends on RAF kinase activating the MEK-ERK phosphorylation cascade. With the specific RAF inhibitor Sorafenib, we show that blocking RAF activity in organotypic cultures increases apoptosis and diminishes the rate of cell proliferation in the otic epithelia, as well as severely impairing neurogenesis of the acoustic-vestibular ganglion (AVG) and neuron maturation. Conclusions/Significance We conclude that RAF kinase activity is essential to establish the balance between cell proliferation and death in neuroepithelial otic precursors, and for otic neuron differentiation and axonal growth at the AVG. PMID:21203386

  4. A Brief History of the Development of Abnormal Psychology: A Training Guide. Final Report.

    ERIC Educational Resources Information Center

    Phelps, William R.

    Presented for practitioners is a history of the development of abnormal psychology. Areas covered include the following: Early medical concepts, ideas carried over from literature, early treatment of the mentally ill, development of the psychological viewpoint, Freud's psychoanalytic theory, Jung's analytic theory, the individual psychology of…

  5. Ear tube insertion

    MedlinePlus

    Myringotomy; Tympanostomy; Ear tube surgery; Pressure equalization tubes; Ventilating tubes; Ear infection - tubes; Otitis - tubes ... trapped fluid can flow out of the middle ear. This prevents hearing loss and reduces the risk ...

  6. Ears and Altitude

    MedlinePlus

    ... Meeting Calendar Find an ENT Doctor Near You Ears and Altitude Ears and Altitude Patient Health Information ... uncomfortable feeling of fullness or pressure. Why do ears pop? Normally, swallowing causes a little click or ...

  7. Ear tube insertion - slideshow

    MedlinePlus

    ... this page: //medlineplus.gov/ency/presentations/100045.htm Ear tube insertion - series—Normal anatomy To use the ... 4 Overview The eardrum (tympanic membrane) separates the ear canal from the middle ear. Update Date 8/ ...

  8. Travel Inside the Ear

    MedlinePlus

    ... Menu Home Health Info Hearing, Ear Infections, and Deafness Balance Taste and Smell Voice, Speech, and Language ... here Home » Health Info » Hearing, Ear Infections, and Deafness Travel Inside the Ear Video When sound waves ...

  9. Disorders of sexual development and abnormal early development in domestic food-producing mammals: the role of chromosome abnormalities, environment and stress factors.

    PubMed

    Favetta, L A; Villagómez, D A F; Iannuzzi, L; Di Meo, G; Webb, A; Crain, S; King, W A

    2012-01-01

    The management of disorders of sexual development (DSD) in humans and domestic animals has been the subject of intense interest for decades. The association between abnormal chromosome constitutions and DSDs in domestic animals has been recorded since the beginnings of conventional cytogenetic analysis. Deviated karyotypes consisting of abnormal sex chromosome sets and/or the coexistence of cells with different sex chromosome constitutions in an individual seem to be the main causes of anomalies of sex determination and sex differentiation. In recent years, a growing interest has developed around the environmental insults, such as endocrine-disrupting compounds (EDC) and heat stressors, which affect fertility, early embryonic development and, in some instances, directly the sex ratio and/or the development of 1 specific sex versus the other. A variety of chemical compounds present in the environment at low doses has been shown to have major effects on the reproductive functions in human and domestic animals following prolonged exposure. In this review, we present an overview of congenital/chromosomal factors that are responsible for the DSDs and link them and the lack of proper embryonic development to environmental factors that are becoming a major global concern.

  10. Abnormal Development of Thalamic Microstructure in Premature Neonates with Congenital Heart Disease

    PubMed Central

    Paquette, Lisa B.; Votava-Smith, Jodie K.; Ceschin, Rafael; Nagasunder, Arabhi C.; Jackson, Hollie A.; Blüml, Stefan; Wisnowski, Jessica L.; Panigrahy, Ashok

    2015-01-01

    Background and Purpose Preterm birth is associated with alteration in cortico-thalamic development, which underlies poor neurodevelopmental outcomes. Our hypothesis was that preterm neonates with CHD would demonstrate abnormal thalamic microstructure when compared to critically ill neonates without CHD. A secondary aim was to identify any association between thalamic microstructural abnormalities and peri-operative clinical variables. Material and Methods We compared thalamic DTI measurements in 21 preterm neonates with CHD to two cohorts of neonates without CHD: 28 term and 27 preterm neonates, identified from the same neonatal intensive care unit. Comparison was made with three other selected white matter regions using ROI manual based measurements. Correlation was made with post-conceptional age and peri-operative clinical variables. Results In preterm neonates with CHD, there were age-related differences in thalamic diffusivity (axial and radial) compared to the preterm and term non-CHD group, in contrast to no differences in anisotropy. Contrary to our hypothesis, abnormal thalamic and optic radiation microstructure was most strongly associated with an elevated first arterial blood gas pO2 and elevated pre-operative arterial blood gas pH (p<0.05). Conclusion Age-related thalamic microstructural abnormalities were observed in preterm neonates with CHD. Perinatal hyperoxemia and increased peri-operative serum pH was associated with abnormal thalamic microstructure in preterm neonates with CHD. This study emphasizes the vulnerability of thalamo-cortical development in the preterm neonate with CHD. PMID:25608695

  11. Trks and p75 genes are differentially expressed in the inner ear of human embryos. What may Trks and p75 null mutant mice suggest on human development?

    PubMed

    Vega, J A; San José, I; Cabo, R; Rodriguez, S; Represa, J

    1999-09-10

    Recent work has shown the expression of Neurotrophins low (p75) and high affinity (Trk's A, B, and C) receptors in the developing inner ear sensory neurons of chick and mouse. Likewise the biological significance of such receptor expression was demonstrated by using both Trks and Neurotrophins null mutant mice. The present study was conducted to determine the expression of Trks and p75 proteins in the human inner ear throughout development. Hence to assess the potential role of Neurotrophins in the development of auditory and vestibular specific innervation in man. In other words, we intend to address the issue whether or not what null mutant mice for Trks and p75 have revealed on inner ear development may be relevant for human embryos. Fifty-two inner ears and their cochleovestibular ganglions (CVG) from human embryos and fetuses, ranging from 5 to 24 weeks of pregnancy were analyzed. Both Western blot and immunocytochemistry on frozen sections were used as complementary procedures. Quantitative Western blot studies revealed that Trk-B and C immunoreactivity (IR) appeared by embryonic week 5 in CVG neurons, increased at high levels between embryonic weeks 7 and 12, and later on, in 15 week-old specimens and older began to decrease to minimal levels. Trk-A IR was detected at just moderate levels during 5 and 7 weeks reflecting the presence of NGF high affinity receptors only at these earlier developmental ages. The p75 IR was detected at high degrees in the early stage of the 5th week and at abundant levels in all studied inner ears from the 7th to the 24th pregnancy week. These Western blot observations were corroborated by immunocytochemistry on frozen sections, which also revealed a major distribution of both p75 and Trks on neuronal bodies while p75 appears localized on supporting cells. Our findings reveal a tight correlation between p75 and Trks expression throughout human development and specific inner ear developmental events, such as target

  12. Association of Traditional Cardiovascular Risk Factors With Development of Major and Minor Electrocardiographic Abnormalities: A Systematic Review.

    PubMed

    Healy, Caroline F; Lloyd-Jones, Donald M

    2016-01-01

    Electrocardiographic (ECG) abnormalities are prevalent in middle aged and are associated with risk of adverse cardiovascular events. It is unclear whether and to what extent traditional risk factors are associated with the development of ECG abnormalities. To determine whether traditional cardiovascular risk factors are associated with the presence or development of ECG abnormalities, we performed a systematic review of the English-language literature for cross-sectional and prospective studies examining associations between traditional cardiovascular risk factors and ECG abnormalities, including major and minor ECG abnormalities, isolated nonspecific ST-segment and T-wave abnormalities, other ST-segment and T-wave abnormalities, QT interval, Q waves, and QRS duration. Of the 202 papers initially identified, 19 were eligible for inclusion. We examined data analyzing risk factor associations with ECG abnormalities in individuals free of cardiovascular disease. For composite major or minor ECG abnormalities, black race, older age, higher blood pressure, use of antihypertensive medications, higher body mass index, diabetes, smoking, and evidence of left ventricular hypertrophy or higher left ventricular mass are the factors most commonly associated with prevalence and incidence. Risk factor associations differ somewhat according to types of specific ECG abnormalities. Because major and minor ECG abnormalities have important and independent prognostic significance, understanding the groups at risk for their development may inform prevention strategies focused on modifiable risk factors to reduce the burden of ECG abnormalities, which may in turn promote CVD prevention. PMID:27054606

  13. A Rapid, Cost-Effective Method to Prepare Recombinant Adeno-Associated Virus for Efficient Gene Transfer to the Developing Mouse Inner Ear.

    PubMed

    Gomes, Michelle M; Wang, Lingyan; Jiang, Han; Kahl, Christoph A; Brigande, John V

    2016-01-01

    There is keen interest to define gene therapies aimed at restoration of auditory and vestibular function in the diseased or damaged mammalian inner ear. A persistent limitation of regenerative medical strategies that seek to correct or modify gene expression in the sensory epithelia of the inner ear involves efficacious delivery of a therapeutic genetic construct. Our approach is to define methodologies that enable fetal gene transfer to the developing mammalian inner ear in an effort to correct defective gene expression during formation of the sensory epithelia or during early postnatal life. Conceptually, the goal is to atraumatically introduce the genetic construct into the otocyst-staged mouse inner ear and transfect otic progenitors that give rise to sensory hair cells and supporting cells. Our long-term goal is to define therapeutic interventions for congenital deafness and balance disorders with the expectation that the approach may also be exploited for therapeutic intervention postnatally.In the inaugural volume of this series, we introduced electroporation-mediated gene transfer to the developing mouse inner ear that encompassed our mouse survival surgery and transuterine microinjection protocols (Brigande et al., Methods Mol Biol 493:125-139, 2009). In this chapter, we first briefly update our use of sodium pentobarbital anesthesia, our preferred anesthetic for mouse ventral laparotomy, in light of its rapidly escalating cost. Next, we define a rapid, cost-effective method to produce recombinant adeno-associated virus (rAAV) for efficient gene transfer to the developing mouse inner ear. Our immediate goal is to provide a genetic toolkit that will permit the definition and validation of gene therapies in mouse models of human deafness and balance disorders. PMID:27259920

  14. The abnormal phosphorylation of tau protein at Ser-202 in Alzheimer disease recapitulates phosphorylation during development.

    PubMed Central

    Goedert, M; Jakes, R; Crowther, R A; Six, J; Lübke, U; Vandermeeren, M; Cras, P; Trojanowski, J Q; Lee, V M

    1993-01-01

    Tau is a neuronal phosphoprotein whose expression is developmentally regulated. A single tau isoform is expressed in fetal human brain but six isoforms are expressed in adult brain, with the fetal isoform corresponding to the shortest of the adult isoforms. Phosphorylation of tau is also developmentally regulated, as fetal tau is phosphorylated at more sites than adult tau. In Alzheimer disease, the six adult tau isoforms become abnormally phosphorylated and form the paired helical filament, the major fibrous component of the characteristic neurofibrillary lesions. We show here that Ser-202 (in the numbering of the longest human brain tau isoform) is a phosphorylation site that distinguishes fetal from adult tau and we identify it as one of the abnormal phosphorylation sites in Alzheimer disease. The abnormal phosphorylation of tau at Ser-202 in Alzheimer disease thus recapitulates normal phosphorylation during development. Images Fig. 1 Fig. 2 Fig. 3 Fig. 4 Fig. 5 PMID:8506352

  15. Understanding normal and abnormal development of the Wolffian/epididymal duct by using transgenic mice

    PubMed Central

    Murashima, Aki; Xu, Bingfang; Hinton, Barry T

    2015-01-01

    The development of the Wolffian/epididymal duct is crucial for proper function and, therefore, male fertility. The development of the epididymis is complex; the initial stages form as a transient embryonic kidney; then the mesonephros is formed, which in turn undergoes extensive morphogenesis under the influence of androgens and growth factors. Thus, understanding of its full development requires a wide and multidisciplinary view. This review focuses on mouse models that display abnormalities of the Wolffian duct and mesonephric development, the importance of these mouse models toward understanding male reproductive tract development, and how these models contribute to our understanding of clinical abnormalities in humans such as congenital anomalies of the kidney and urinary tract (CAKUT). PMID:26112482

  16. Understanding normal and abnormal development of the Wolffian/epididymal duct by using transgenic mice.

    PubMed

    Murashima, Aki; Xu, Bingfang; Hinton, Barry T

    2015-01-01

    The development of the Wolffian/epididymal duct is crucial for proper function and, therefore, male fertility. The development of the epididymis is complex; the initial stages form as a transient embryonic kidney; then the mesonephros is formed, which in turn undergoes extensive morphogenesis under the influence of androgens and growth factors. Thus, understanding of its full development requires a wide and multidisciplinary view. This review focuses on mouse models that display abnormalities of the Wolffian duct and mesonephric development, the importance of these mouse models toward understanding male reproductive tract development, and how these models contribute to our understanding of clinical abnormalities in humans such as congenital anomalies of the kidney and urinary tract (CAKUT). PMID:26112482

  17. Chromosome Abnormalities

    MedlinePlus

    ... decade, newer techniques have been developed that allow scientists and doctors to screen for chromosomal abnormalities without using a microscope. These newer methods compare the patient's DNA to a normal DNA ...

  18. Developing an online steady-state visual evoked potential-based brain-computer interface system using EarEEG.

    PubMed

    Wang, Yu-Te; Nakanishi, Masaki; Kappel, Simon Lind; Kidmose, Preben; Mandic, Danilo P; Wang, Yijun; Cheng, Chung-Kuan; Jung, Tzyy-Ping

    2015-01-01

    The purpose of this study is to demonstrate an online steady-state visual evoked potential (SSVEP)-based BCI system using EarEEG. EarEEG is a novel recording concept where electrodes are embedded on the surface of earpieces customized to the individual anatomical shape of users' ear. It has been shown that the EarEEG can be used to record SSVEPs in previous studies. However, a long distance between the visual cortex and the ear makes the signal-to-noise ratio (SNR) of SSVEPs acquired by the EarEEG relatively low. Recently, filter bank- and training data-based canonical correlation analysis algorithms have shown significant performance improvement in terms of accuracy of target detection and information transfer rate (ITR). This study implemented an online four-class SSVEP-based BCI system using EarEEG. Four subjects participated in offline and online BCI experiments. For the offline classification, an average accuracy of 82.71±11.83 % was obtained using 4 sec-long SSVEPs acquired from earpieces. In the online experiment, all subjects successfully completed the tasks with an average accuracy of 87.92±12.10 %, leading to an average ITR of 16.60±6.55 bits/min. The results suggest that EarEEG can be used to perform practical BCI applications. The EarEEG has the potential to be used as a portable EEG recordings platform, that could enable real-world BCI applications. PMID:26736745

  19. Numerical simulation of the human ear and the dynamic analysis of the middle ear sound transmission

    NASA Astrophysics Data System (ADS)

    Yao, W.; Ma, J.; Huang, X.

    2013-06-01

    Based on the clinical CT of normal right ear, a 3-D ?nite element (FE) model of the human ear consisting of the external ear canal, middle ear(tympanic membrane, ossicular chain, ligaments, tendons), and inner ear (including semicircular canals, vestibular, spiral cochlear)was constructed in this paper. The complicated structures and inner boundary conditions of middle ear were described in this model. Model analysis and acoustic-structure-?uid coupled dynamic frequency response analysis were conducted on the model. The validity of this model was confirmed by comparing the results with published experimental data. The amplitudes and velocities of tympanic membrane and stapes footplate, sound pressure gain across the middle ear, and the cochlear input impedance were derived. Besides, it was concluded that the ear canal can amplify the sound signal in low frequencies.The modes of vibration of middle ear auditory ossicles, oval window and round window have been analysed. This model can well simulate the acoustic behavior with the interaction of external ear, middle ear and inner ear, which can supply more valuable theoretical support for development and improvement of hearing-aid and artificial inner ear.

  20. Cranial index of children with normal and abnormal brain development in Sokoto, Nigeria: A comparative study

    PubMed Central

    Musa, Muhammad Awwal; Zagga, Abdullahi Daudu; Danfulani, Mohammed; Tadros, Aziz Abdo; Ahmed, Hamid

    2014-01-01

    Background: Abnormal brain development due to neurodevelopmental disorders in children has always been an important concern, but yet has to be considered as a significant public health problem, especially in the low- and middle-income countries including Nigeria. Aims: The aim of this study is to determine whether abnormal brain development in the form of neurodevelopmental disorders causes any deviation in the cranial index of affected children. Materials and Methods: This is a comparative study on the head length, head width, and cranial index of 112 children (72 males and 40 females) diagnosed with at least one abnormal problem in brain development, in the form of a neurodevelopmental disorder (NDD), in comparison with that of 218 normal growing children without any form of NDD (121 males and 97 females), aged 0-18 years old seen at the Usmanu Danfodiyo University Teaching Hospital, Sokoto, over a period of six months, June to December, 2012. The head length and head width of the children was measured using standard anatomical landmarks and cranial index calculated. The data obtained was entered into the Microsoft excel worksheet and analyzed using SPSS version 17. Results: The mean Cephalic Index for normal growing children with normal brain development was 79.82 ± 3.35 and that of the children with abnormal brain development was 77.78 ± 2.95 and the difference between the two groups was not statistically significant (P > 0.05). Conclusion: It can be deduced from this present study that the cranial index does not change in children with neurodevelopmental disorders. PMID:24966551

  1. mTOR signaling and its roles in normal and abnormal brain development

    PubMed Central

    Takei, Nobuyuki; Nawa, Hiroyuki

    2014-01-01

    Target of rapamycin (TOR) was first identified in yeast as a target molecule of rapamycin, an anti-fugal and immunosuppressant macrolide compound. In mammals, its orthologue is called mammalian TOR (mTOR). mTOR is a serine/threonine kinase that converges different extracellular stimuli, such as nutrients and growth factors, and diverges into several biochemical reactions, including translation, autophagy, transcription, and lipid synthesis among others. These biochemical reactions govern cell growth and cause cells to attain an anabolic state. Thus, the disruption of mTOR signaling is implicated in a wide array of diseases such as cancer, diabetes, and obesity. In the central nervous system, the mTOR signaling cascade is activated by nutrients, neurotrophic factors, and neurotransmitters that enhances protein (and possibly lipid) synthesis and suppresses autophagy. These processes contribute to normal neuronal growth by promoting their differentiation, neurite elongation and branching, and synaptic formation during development. Therefore, disruption of mTOR signaling may cause neuronal degeneration and abnormal neural development. While reduced mTOR signaling is associated with neurodegeneration, excess activation of mTOR signaling causes abnormal development of neurons and glia, leading to brain malformation. In this review, we first introduce the current state of molecular knowledge of mTOR complexes and signaling in general. We then describe mTOR activation in neurons, which leads to translational enhancement, and finally discuss the link between mTOR and normal/abnormal neuronal growth during development. PMID:24795562

  2. Exceeding parents' expectations in Ear-Nose-Throat outpatient facilities: the development and analysis of a questionnaire.

    PubMed

    Margaritis, Eleftherios; Katharaki, Maria; Katharakis, George

    2012-05-01

    The study attempts to develop an outpatient service quality scale by investigating the key dimensions which assess parental satisfaction and provides a recommendation on an improved health service delivery system. The survey was conducted in an Ear-Nose-Throat outpatient clinic of a Greek public pediatric hospital. A total of 127 parents in outpatient waiting areas were chosen; 74.8% of the sampled parents were under 40, and 78% were mothers. A factor analysis was performed; while a Fischer's exact test and multinomial logistic regression analysis was conducted. All Cronbach's α exceeded 0.70 and all factor loadings exceeded 0.50. Twenty-three items were retained through the scale development process and seven factors were formed that appear to be statistically valid and clinically meaningful: access and convenience, doctor's attention, customization, reliability, assurance, satisfaction and loyalty. Findings were discussed in relation to parents' overall satisfaction and intention of reusing and recommending outpatient clinic. Satisfaction was found to be positively affected by access and convenience and doctors' attention. Staff attitude and the telephone procedure of scheduling the child's examination found positively correlated to the likelihood of recommending services to friends and relatives. Time and communication in the waiting room influenced parents' satisfaction. Overall, results reveal the measures that need to be taken in order to improve outpatient service quality. PMID:22221890

  3. Abnormal visual experience during development alters the early stages of visual-tactile integration.

    PubMed

    Niechwiej-Szwedo, Ewa; Chin, Jessica; Wolfe, Paul J; Popovich, Christina; Staines, W Richard

    2016-05-01

    Visual experience during the critical periods in early postnatal life is necessary for the normal development of the visual system. Disruption of visual input during this period results in amblyopia, which is associated with reduced activation of the striate and extrastriate cortices. It is well known that visual input converges with other sensory signals and exerts a significant influence on cortical processing in multiple association areas. Recent work in healthy adults has also shown that task-relevant visual input can modulate neural excitability at very early stages of information processing in the primary somatosensory cortex. Here we used electroencephalography to investigate visual-tactile interactions in adults with abnormal binocular vision due to amblyopia and strabismus. Results showed three main findings. First, in comparison to a visually normal control group, participants with abnormal vision had a significantly lower amplitude of the P50 somatosensory event related potential (ERP) when visual and tactile stimuli were presented concurrently. Second, the amplitude of the P100 somatosensory ERP was significantly greater in participants with abnormal vision. These results indicate that task relevant visual input does not significantly influence the excitability of the primary somatosensory cortex, instead, the excitability of the secondary somatosensory cortex is increased. Third, participants with abnormal vision had a higher amplitude of the P1 visual ERP when a tactile stimulus was presented concurrently. Importantly, these results were not modulated by viewing condition, which indicates that the impact of amblyopia on crossmodal interactions is not simply related to the reduced visual acuity as it was evident when viewing with the unaffected eye and binocularly. These results indicate that the consequences of abnormal visual experience on neurophysiological processing extend beyond the primary and secondary visual areas to other modality

  4. Cytogenetic studies of 1232 patients with different sexual development abnormalities from the Sultanate of Oman.

    PubMed

    Al-Alawi, Intisar; Goud, Tadakal Mallana; Al-Harasi, Salma; Rajab, Anna

    2016-02-01

    The aim of this study was to evaluate cytogenetic findings in Omani patients who had been referred for suspicion of sex chromosome abnormalities that resulted in different clinical disorders. Furthermore, it sought to examine the frequency of chromosomal anomalies in these patients and to compare the obtained results with those reported elsewhere. Cytogenetic analysis was performed on 1232 cases with variant characteristics of sexual development disorders who had been referred to the cytogenetic department, National Genetic Centre, Ministry of Health, from different hospitals in the Sultanate of Oman between 1999 and 2014. The karyotype results demonstrated chromosomal anomalies in 24.2% of the cases, where 67.5% of abnormalities were identified in referral females, whereas only 32.6% were in referral males. Of all sex chromosome anomalies detected, Turner syndrome was the most frequent (38.2%) followed by Klinefelter syndrome (24.9%) and XY phenotypic females (16%). XXX syndrome and XX phenotypic males represented 6.8% and 3.8% of all sex chromosome anomalies, respectively. Cytogenetic analysis of patients referred with various clinical suspicions of chromosomal abnormalities revealed a high rate of chromosomal anomalies. This is the first broad cytogenetic study reporting combined frequencies of sex chromosome anomalies in sex development disorders in Oman. PMID:26706459

  5. [A comparative morphological analysis of the development of the ear in mammals].

    PubMed

    Solntseva, G N

    1993-01-01

    This paper is the first comparative study into prenatal ontogenesis of the peripheral auditory system in mammals. A wide range of ecologically different species was studied, including terrestrial, semi-aquatic and aquatic forms with auditory systems functioning at low, medium, and high frequencies. Structural peculiarities of the development of auditory organ were determined for each studied species in relation to its operation frequency and acoustic properties of the outer medium. The origin and individual stages of morphological adaptations found in semi-aquatic, aquatic, and echolocating species are described. The data obtained allowed to reveal general principles of development of the peripheral auditory system in mammals with different ecological specialization.

  6. The Listening Ear: The Development of Speech as a Creative Influence in Education (Learning Resource Series).

    ERIC Educational Resources Information Center

    McAllen, Audrey E.

    This book gives teachers an understanding of speech training through specially selected exercises. The book's exercises aim to help develop clear speaking in the classroom. Methodically and perceptively used, the book will assist those concerned with the creative powers of speech as a teaching art. In Part 1, there are sections on the links…

  7. A physical study of vibrating membranes in developing a middle ear prosthesis.

    PubMed

    Ledoux, L; Defebvre, A; Pouliquen, J; Vaneecloo, F M

    1991-01-01

    We present a prosthesis meant to replace the tympano-ossicular set as well as the measuring apparatus for developing and characterizing this prosthesis. Such biocompatible materials as silicone elastomer and Teflon are used. Effects due to aging and sterilization have been negligible. Comparisons with anatomic specimens have yielded good results. Furthermore, the first clinical trials with this prosthesis have been promising.

  8. Gaps in comprehension of ear development impede successful human hearing organ restoration

    PubMed Central

    Jahan, Israt; Pan, Ning; Elliott, Karen L.; Fritzsch, Bernd

    2015-01-01

    Neurosensory hearing loss is a growing problem of super-aged societies. Cochlear implants can restore some hearing, but rebuilding a lost hearing organ would be superior. Research has discovered many cellular and molecular steps to develop a hearing organ but translating those insights into hearing organ restoration remains unclear. We cannot make various hair cell types and arrange them into their specific patterns surrounded by the right type of supporting cells in the right numbers. Our overview of the topologically highly organized and functionally diversified cellular mosaic of the mammalian hearing organ highlights what is known and unknown about its development. Following this analysis, we suggest critical steps to guide future attempts toward restoration of a functional OC. We argue that generating mutant mouse lines that mimic human pathology to fine-tune attempts toward long-term functional restoration are needed to go beyond the hope generated by restoring single hair cells. PMID:26208302

  9. [Abnormal psychosocial development and legal responsibility--results of psychopathometric studies].

    PubMed

    Littmann, E; Friemert, K; Szewczyk, H

    1989-05-01

    The introduction (1968) of the legal concept of Grave Abnormal Development of the Personality Amounting to a Disorder into the penal code, made possible criminal deculpation on the basis of psychosocial maldevelopment. On the basis of an intelligence- and personality-diagnostic test-battery (Psychopathometry), the findings obtained in the examination of a sample of culprits on probation under this legal provision, has been compared with a control group homogeneous in respect of the significant parameters. Psychopathometric methods can and should reasonably supplement expertises of this culprits with defective psychosocial development.

  10. Middle ear infection (image)

    MedlinePlus

    A middle ear infection is also known as otitis media. It is one of the most common of childhood infections. With this illness, the middle ear becomes red, swollen, and inflamed because of bacteria ...

  11. Ear drainage culture

    MedlinePlus

    ... needed. Your health care provider will use a cotton swab to collect the sample from inside the ... Using a cotton swab to take a sample of drainage from the outer ear is not painful. However, ear pain may ...

  12. Ear Infection and Vaccines

    MedlinePlus

    ... an ENT Doctor Near You Ear Infection and Vaccines Ear Infection and Vaccines Patient Health Information News ... or may need reinsertion over time. What about vaccines? A vaccine is a preparation administered to stimulate ...

  13. Ear Infections in Children

    MedlinePlus

    ... shaped organ that converts sound vibrations from the middle ear into electrical signals. The auditory nerve carries these signals from the cochlea to the brain. Other nearby parts of the ear also can be involved in ...

  14. Ear surgery - slideshow

    MedlinePlus

    ... this page: //medlineplus.gov/ency/presentations/100016.htm Ear surgery - series—Normal anatomy To use the sharing ... Overview This image demonstrates normal appearance of the ears in relation to the face. Update Date 10/ ...

  15. Ear infection - chronic

    MedlinePlus

    Middle ear infection - chronic; Otitis media - chronic; Chronic otitis media; Chronic ear infection ... Chole RA. Chronic otitis media, mastoiditis, and petrositis. In: Flint PW, Haughey BH, Lund V, et al, eds. Cummings Otolaryngology: Head & Neck Surgery . 6th ed. ...

  16. The development of hepatic stellate cells in normal and abnormal human fetuses – an immunohistochemical study

    PubMed Central

    Loo, Christine K C; Pereira, Tamara N; Pozniak, Katarzyna N; Ramsing, Mette; Vogel, Ida; Ramm, Grant A

    2015-01-01

    The precise embryological origin and development of hepatic stellate cells is not established. Animal studies and observations on human fetuses suggest that they derive from posterior mesodermal cells that migrate via the septum transversum and developing diaphragm to form submesothelial cells beneath the liver capsule, which give rise to mesenchymal cells including hepatic stellate cells. However, it is unclear if these are similar to hepatic stellate cells in adults or if this is the only source of stellate cells. We have studied hepatic stellate cells by immunohistochemistry, in developing human liver from autopsies of fetuses with and without malformations and growth restriction, using cellular Retinol Binding Protein-1 (cRBP-1), Glial Fibrillary Acidic Protein (GFAP), and α-Smooth Muscle Actin (αSMA) antibodies, to identify factors that influence their development. We found that hepatic stellate cells expressing cRBP-1 are present from the end of the first trimester of gestation and reduce in density throughout gestation. They appear abnormally formed and variably reduced in number in fetuses with abnormal mesothelial Wilms Tumor 1 (WT1) function, diaphragmatic hernia and in ectopic liver nodules without mesothelium. Stellate cells showed similarities to intravascular cells and their presence in a fetus with diaphragm agenesis suggests they may be derived from circulating stem cells. Our observations suggest circulating stem cells as well as mesothelium can give rise to hepatic stellate cells, and that they require normal mesothelial function for their development. PMID:26265759

  17. Ear infections in autistic and normal children.

    PubMed

    Konstantareas, M M; Homatidis, S

    1987-12-01

    The frequency of ear infections, ear tube drainage, and deafness was examined through parental reports in autistic and yoke-matched, normal children. For the autistic group these difficulties were additionally examined as a function of the children's cognitive and communication abilities, verbal versus nonverbal status, sex, and degree of autistic symptomatology. Autistic children had a greater incidence of ear infections than matched normal peers. Lower-functioning children had an earlier onset of ear infections than their higher-functioning autistic peers. Ear infections coexisted with low-set ears, and with a higher autistic symptomatology score. The findings are discussed in terms of greater CNS vulnerability in the autistic children, which is likely present since embryogenesis. The possible adverse consequences of intermittent hearing loss on language, cognitive, and socioaffective development are considered.

  18. A mechanical model predicts morphological abnormalities in the developing human brain

    NASA Astrophysics Data System (ADS)

    Budday, Silvia; Raybaud, Charles; Kuhl, Ellen

    2014-07-01

    The developing human brain remains one of the few unsolved mysteries of science. Advancements in developmental biology, neuroscience, and medical imaging have brought us closer than ever to understand brain development in health and disease. However, the precise role of mechanics throughout this process remains underestimated and poorly understood. Here we show that mechanical stretch plays a crucial role in brain development. Using the nonlinear field theories of mechanics supplemented by the theory of finite growth, we model the human brain as a living system with a morphogenetically growing outer surface and a stretch-driven growing inner core. This approach seamlessly integrates the two popular but competing hypotheses for cortical folding: axonal tension and differential growth. We calibrate our model using magnetic resonance images from very preterm neonates. Our model predicts that deviations in cortical growth and thickness induce morphological abnormalities. Using the gyrification index, the ratio between the total and exposed surface area, we demonstrate that these abnormalities agree with the classical pathologies of lissencephaly and polymicrogyria. Understanding the mechanisms of cortical folding in the developing human brain has direct implications in the diagnostics and treatment of neurological disorders, including epilepsy, schizophrenia, and autism.

  19. A mechanical model predicts morphological abnormalities in the developing human brain

    PubMed Central

    Budday, Silvia; Raybaud, Charles; Kuhl, Ellen

    2014-01-01

    The developing human brain remains one of the few unsolved mysteries of science. Advancements in developmental biology, neuroscience, and medical imaging have brought us closer than ever to understand brain development in health and disease. However, the precise role of mechanics throughout this process remains underestimated and poorly understood. Here we show that mechanical stretch plays a crucial role in brain development. Using the nonlinear field theories of mechanics supplemented by the theory of finite growth, we model the human brain as a living system with a morphogenetically growing outer surface and a stretch-driven growing inner core. This approach seamlessly integrates the two popular but competing hypotheses for cortical folding: axonal tension and differential growth. We calibrate our model using magnetic resonance images from very preterm neonates. Our model predicts that deviations in cortical growth and thickness induce morphological abnormalities. Using the gyrification index, the ratio between the total and exposed surface area, we demonstrate that these abnormalities agree with the classical pathologies of lissencephaly and polymicrogyria. Understanding the mechanisms of cortical folding in the developing human brain has direct implications in the diagnostics and treatment of neurological disorders, including epilepsy, schizophrenia, and autism. PMID:25008163

  20. Prevalence of middle ear disorders in coal miners

    SciTech Connect

    Lempert, B.L.; Hopkinson, N.T.; Keith, R.W.; Motl, M.L.; Horine, J.

    1981-06-01

    Results are presented from a study of the prevalence of middle and external ear disorders in coal miners who work underground. The study followed from an earlier NIOSH report (1976) that indicated a possibly large number of otoscopic abnormalities in this population of workers. Otoscopic examinations, pure tone air- and bone-conduction audiometry tests, and impedance tests were administered to 350 underground miners and 150 industrial workers not associated with mining. The study was conducted completely within a hospital otolaryngology/audiology clinic setting. Results of the investigation showed a highly similar prevalence of middle ear and ear canal abnormalities in the miner group and the control group (19 percent). Middle ear abnormalities observed in the miners were judged by the examining otolaryngologists to have preceded their experience in the mines and were not related solely to underground noise exposure or coal dust. Nearly half of the subjects who had an air-bone gap had no middle ear abnormality observable by otoscopic examination. There was substantial agreement between the finding of abnormal otoscopy and abnormal tympanometry. By itself, acoustic reflex was not useful in identifying middle ear disorders, since this reflex may be absent for other reasons, including presence of severe sensorineural hearing loss.

  1. Vocal development during postnatal growth and ear morphology in a shrew that generates seismic vibrations, Diplomesodon pulchellum.

    PubMed

    Zaytseva, Alexandra S; Volodin, Ilya A; Mason, Matthew J; Frey, Roland; Fritsch, Guido; Ilchenko, Olga G; Volodina, Elena V

    2015-09-01

    The ability of adult and subadult piebald shrews (Diplomesodon pulchellum) to produce 160Hz seismic waves is potentially reflected in their vocal ontogeny and ear morphology. In this study, the ontogeny of call variables and body traits was examined in 11 litters of piebald shrews, in two-day intervals from birth to 22 days (subadult), and ear structure was investigated in two specimens using micro-computed tomography (micro-CT). Across ages, the call fundamental frequency (f0) was stable in squeaks and clicks and increased steadily in screeches, representing an unusual, non-descending ontogenetic pathway of f0. The rate of the deep sinusoidal modulation (pulse rate) of screeches increased from 75Hz at 3-4 days to 138Hz at 21-22 days, probably relating to ontogenetic changes in contraction rates of the same muscles which are responsible for generating seismic vibrations. The ear reconstructions revealed that the morphologies of the middle and inner ears of the piebald shrew are very similar to those of the common shrew (Sorex araneus) and the lesser white-toothed shrew (Crocidura suaveolens), which are not known to produce seismic signals. These results suggest that piebald shrews use a mechanism other than hearing for perceiving seismic vibrations.

  2. Vocal development during postnatal growth and ear morphology in a shrew that generates seismic vibrations, Diplomesodon pulchellum.

    PubMed

    Zaytseva, Alexandra S; Volodin, Ilya A; Mason, Matthew J; Frey, Roland; Fritsch, Guido; Ilchenko, Olga G; Volodina, Elena V

    2015-09-01

    The ability of adult and subadult piebald shrews (Diplomesodon pulchellum) to produce 160Hz seismic waves is potentially reflected in their vocal ontogeny and ear morphology. In this study, the ontogeny of call variables and body traits was examined in 11 litters of piebald shrews, in two-day intervals from birth to 22 days (subadult), and ear structure was investigated in two specimens using micro-computed tomography (micro-CT). Across ages, the call fundamental frequency (f0) was stable in squeaks and clicks and increased steadily in screeches, representing an unusual, non-descending ontogenetic pathway of f0. The rate of the deep sinusoidal modulation (pulse rate) of screeches increased from 75Hz at 3-4 days to 138Hz at 21-22 days, probably relating to ontogenetic changes in contraction rates of the same muscles which are responsible for generating seismic vibrations. The ear reconstructions revealed that the morphologies of the middle and inner ears of the piebald shrew are very similar to those of the common shrew (Sorex araneus) and the lesser white-toothed shrew (Crocidura suaveolens), which are not known to produce seismic signals. These results suggest that piebald shrews use a mechanism other than hearing for perceiving seismic vibrations. PMID:26112702

  3. Grain Yield and Fusarium Ear Rot of Maize Hybrids Developed From Lines With Varying Levels of Resistance

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Fusarium ear rot, caused by Fusarium verticillioides and other Fusarium spp. is found in all U.S. maize growing regions. Affected grain often contains carcinogenic mycotoxins called fumonisins. We tested the hypothesis that inbred lines with greater resistance to fumonisin contamination would pro...

  4. Frequency of ear symptoms and hearing loss in ichthyosis: a pilot survey study.

    PubMed

    Huang, Jennifer T; Mallon, Kaitlin; Hamill, Shannon; Ohlms, Laurie A; Liang, Marilyn G

    2014-01-01

    Ichthyoses comprise a heterogeneous array of skin conditions resulting from impairment of cornification. Although ear structures can be affected, ear-related symptoms have never been investigated in patients with ichthyosis. In this pilot survey study, our aim was to determine the frequency of ear symptoms, hearing loss, and related medical interventions in patients with ichthyosis. Our secondary aim was to compare the frequency of these items according to age group. An online survey using Redcap was developed and posted online on the Foundation for Ichthyosis and Related Skin Types website for 6 months. Patients or parents of patients with ichthyosis were asked to complete the survey. Data analysis excluded patients with keratitis-ichthyosis-deafness syndrome and surveys that had fewer than two completed items. One hundred thirty-five unique surveys were used for data analysis. Of all participants, 80% reported ear pruritus, 66% reported trouble hearing, 29% reported frequent ear pain, 28% had abnormal hearing test results, and 16% had used hearing aids. Of the 88 participants who reported trouble hearing, 24 (27.3%) had never been to a hearing specialist. Significantly more participants older than 18 years of age (74%, 57/77) reported trouble hearing than participants age 18 years and younger (53%, 31/58; p = 0.02). The frequencies of other ear symptoms and hearing loss were not statistically significantly different between the age groups. Ear pruritus, ear pain, and hearing loss are important concerns in patients with all forms of ichthyosis in all age groups. Early diagnosis and intervention may improve the quality of life of patients with ichthyosis.

  5. Genetic variants associated with motion sickness point to roles for inner ear development, neurological processes and glucose homeostasis

    PubMed Central

    Hromatka, Bethann S.; Tung, Joyce Y.; Kiefer, Amy K.; Do, Chuong B.; Hinds, David A.; Eriksson, Nicholas

    2015-01-01

    Roughly one in three individuals is highly susceptible to motion sickness and yet the underlying causes of this condition are not well understood. Despite high heritability, no associated genetic factors have been discovered. Here, we conducted the first genome-wide association study on motion sickness in 80 494 individuals from the 23andMe database who were surveyed about car sickness. Thirty-five single-nucleotide polymorphisms (SNPs) were associated with motion sickness at a genome-wide-significant level (P < 5 × 10−8). Many of these SNPs are near genes involved in balance, and eye, ear and cranial development (e.g. PVRL3, TSHZ1, MUTED, HOXB3, HOXD3). Other SNPs may affect motion sickness through nearby genes with roles in the nervous system, glucose homeostasis or hypoxia. We show that several of these SNPs display sex-specific effects, with up to three times stronger effects in women. We searched for comorbid phenotypes with motion sickness, confirming associations with known comorbidities including migraines, postoperative nausea and vomiting (PONV), vertigo and morning sickness and observing new associations with altitude sickness and many gastrointestinal conditions. We also show that two of these related phenotypes (PONV and migraines) share underlying genetic factors with motion sickness. These results point to the importance of the nervous system in motion sickness and suggest a role for glucose levels in motion-induced nausea and vomiting, a finding that may provide insight into other nausea-related phenotypes like PONV. They also highlight personal characteristics (e.g. being a poor sleeper) that correlate with motion sickness, findings that could help identify risk factors or treatments. PMID:25628336

  6. Genetic variants associated with motion sickness point to roles for inner ear development, neurological processes and glucose homeostasis.

    PubMed

    Hromatka, Bethann S; Tung, Joyce Y; Kiefer, Amy K; Do, Chuong B; Hinds, David A; Eriksson, Nicholas

    2015-05-01

    Roughly one in three individuals is highly susceptible to motion sickness and yet the underlying causes of this condition are not well understood. Despite high heritability, no associated genetic factors have been discovered. Here, we conducted the first genome-wide association study on motion sickness in 80 494 individuals from the 23andMe database who were surveyed about car sickness. Thirty-five single-nucleotide polymorphisms (SNPs) were associated with motion sickness at a genome-wide-significant level (P < 5 × 10(-8)). Many of these SNPs are near genes involved in balance, and eye, ear and cranial development (e.g. PVRL3, TSHZ1, MUTED, HOXB3, HOXD3). Other SNPs may affect motion sickness through nearby genes with roles in the nervous system, glucose homeostasis or hypoxia. We show that several of these SNPs display sex-specific effects, with up to three times stronger effects in women. We searched for comorbid phenotypes with motion sickness, confirming associations with known comorbidities including migraines, postoperative nausea and vomiting (PONV), vertigo and morning sickness and observing new associations with altitude sickness and many gastrointestinal conditions. We also show that two of these related phenotypes (PONV and migraines) share underlying genetic factors with motion sickness. These results point to the importance of the nervous system in motion sickness and suggest a role for glucose levels in motion-induced nausea and vomiting, a finding that may provide insight into other nausea-related phenotypes like PONV. They also highlight personal characteristics (e.g. being a poor sleeper) that correlate with motion sickness, findings that could help identify risk factors or treatments. PMID:25628336

  7. Transcriptome Analysis for Abnormal Spike Development of the Wheat Mutant dms

    PubMed Central

    Zhu, Xin-Xin; Li, Qiao-Yun; Shen, Chun-Cai; Duan, Zong-Biao; Yu, Dong-Yan; Niu, Ji-Shan; Ni, Yong-Jing; Jiang, Yu-Mei

    2016-01-01

    Background Wheat (Triticum aestivum L.) spike development is the foundation for grain yield. We obtained a novel wheat mutant, dms, characterized as dwarf, multi-pistil and sterility. Although the genetic changes are not clear, the heredity of traits suggests that a recessive gene locus controls the two traits of multi-pistil and sterility in self-pollinating populations of the medium plants (M), such that the dwarf genotype (D) and tall genotype (T) in the progeny of the mutant are ideal lines for studies regarding wheat spike development. The objective of this study was to explore the molecular basis for spike abnormalities of dwarf genotype. Results Four unigene libraries were assembled by sequencing the mRNAs of the super-bulked differentiating spikes and stem tips of the D and T plants. Using integrative analysis, we identified 419 genes highly expressed in spikes, including nine typical homeotic genes of the MADS-box family and the genes TaAP2, TaFL and TaDL. We also identified 143 genes that were significantly different between young spikes of T and D, and 26 genes that were putatively involved in spike differentiation. The result showed that the expression levels of TaAP1-2, TaAP2, and other genes involved in the majority of biological processes such as transcription, translation, cell division, photosynthesis, carbohydrate transport and metabolism, and energy production and conversion were significantly lower in D than in T. Conclusions We identified a set of genes related to wheat floral organ differentiation, including typical homeotic genes. Our results showed that the major causal factors resulting in the spike abnormalities of dms were the lower expression homeotic genes, hormonal imbalance, repressed biological processes, and deficiency of construction materials and energy. We performed a series of studies on the homeotic genes, however the other three causal factors for spike abnormal phenotype of dms need further study. PMID:26982202

  8. [An ear thermometer based on infrared thermopiles sensor].

    PubMed

    Xie, Haiyuan; Qian, Mingli

    2013-09-01

    According to the development of body temperature measurement mode, an ear thermometer with infrared thermopiles sensor is designed for body thermometry Compared with oral thermometer, the accuracy of ear thermometer is acceptable.

  9. Heterosis in early maize ear inflorescence development: a genome-wide transcription analysis for two maize inbred lines and their hybrid.

    PubMed

    Ding, Haiping; Qin, Cheng; Luo, Xirong; Li, Lujiang; Chen, Zhe; Liu, Hongjun; Gao, Jian; Lin, Haijian; Shen, Yaou; Zhao, Maojun; Lübberstedt, Thomas; Zhang, Zhiming; Pan, Guangtang

    2014-08-11

    Heterosis, or hybrid vigor, contributes to superior agronomic performance of hybrids compared to their inbred parents. Despite its importance, little is known about the genetic and molecular basis of heterosis. Early maize ear inflorescences formation affects grain yield, and are thus an excellent model for molecular mechanisms involved in heterosis. To determine the parental contributions and their regulation during maize ear-development-genesis, we analyzed genome-wide digital gene expression profiles in two maize elite inbred lines (B73 and Mo17) and their F1 hybrid using deep sequencing technology. Our analysis revealed 17,128 genes expressed in these three genotypes and 22,789 genes expressed collectively in the present study. Approximately 38% of the genes were differentially expressed in early maize ear inflorescences from heterotic cross, including many transcription factor genes and some presence/absence variations (PAVs) genes, and exhibited multiple modes of gene action. These different genes showing differential expression patterns were mainly enriched in five cellular component categories (organelle, cell, cell part, organelle part and macromolecular complex), five molecular function categories (structural molecule activity, binding, transporter activity, nucleic acid binding transcription factor activity and catalytic activity), and eight biological process categories (cellular process, metabolic process, biological regulation, regulation of biological process, establishment of localization, cellular component organization or biogenesis, response to stimulus and localization). Additionally, a significant number of genes were expressed in only one inbred line or absent in both inbred lines. Comparison of the differences of modes of gene action between previous studies and the present study revealed only a small number of different genes had the same modes of gene action in both maize seedlings and ear inflorescences. This might be an indication that in

  10. Heterosis in Early Maize Ear Inflorescence Development: A Genome-Wide Transcription Analysis for Two Maize Inbred Lines and Their Hybrid

    PubMed Central

    Ding, Haiping; Qin, Cheng; Luo, Xirong; Li, Lujiang; Chen, Zhe; Liu, Hongjun; Gao, Jian; Lin, Haijian; Shen, Yaou; Zhao, Maojun; Lübberstedt, Thomas; Zhang, Zhiming; Pan, Guangtang

    2014-01-01

    Heterosis, or hybrid vigor, contributes to superior agronomic performance of hybrids compared to their inbred parents. Despite its importance, little is known about the genetic and molecular basis of heterosis. Early maize ear inflorescences formation affects grain yield, and are thus an excellent model for molecular mechanisms involved in heterosis. To determine the parental contributions and their regulation during maize ear-development-genesis, we analyzed genome-wide digital gene expression profiles in two maize elite inbred lines (B73 and Mo17) and their F1 hybrid using deep sequencing technology. Our analysis revealed 17,128 genes expressed in these three genotypes and 22,789 genes expressed collectively in the present study. Approximately 38% of the genes were differentially expressed in early maize ear inflorescences from heterotic cross, including many transcription factor genes and some presence/absence variations (PAVs) genes, and exhibited multiple modes of gene action. These different genes showing differential expression patterns were mainly enriched in five cellular component categories (organelle, cell, cell part, organelle part and macromolecular complex), five molecular function categories (structural molecule activity, binding, transporter activity, nucleic acid binding transcription factor activity and catalytic activity), and eight biological process categories (cellular process, metabolic process, biological regulation, regulation of biological process, establishment of localization, cellular component organization or biogenesis, response to stimulus and localization). Additionally, a significant number of genes were expressed in only one inbred line or absent in both inbred lines. Comparison of the differences of modes of gene action between previous studies and the present study revealed only a small number of different genes had the same modes of gene action in both maize seedlings and ear inflorescences. This might be an indication that in

  11. Development of an indirect immunofluorescence assay for diagnosis of bovine viral diarrhoea virus on ear notch tissue samples in cattle infected persistently.

    PubMed

    Bedeković, Tomislav; Lemo, Nina; Lojkić, Ivana; Cvetnić, Zeljko; Cač, Zeljko; Madić, Josip

    2011-12-01

    Bovine viral diarrhoea virus (BVDV) causes a disease that has a wide range of clinical symptoms in domestic and wild ruminants. It is a major problem in cattle and causes significant economic losses in the cattle industry. The virus infects bovines of all ages and causes both immunosuppression and reproductive, respiratory and digestive disorders. Cattle infected persistently, as a continuing source of the virus and the main factor in transmission of the disease between and among herds, are the main source of BVDV and a primary factor in the epidemiology of the disease. To determine whether a BVDV infection is persistent, two samples should be taken at 3-4 week intervals and tested for the virus antigen. Animal sera, whole blood, organ and ear notch tissue samples can be used for BVDV diagnosis. In ear notch tissue, viral antigen can be detected by an antigen enzyme-linked immunosorbent assay (antigen ELISA), immunohistochemistry (IHC) and reverse-transcription polymerase chain reaction (RT-PCR). This paper describes the development and implementation of an indirect immunofluorescence (IF) method using ear notch tissue samples for diagnosis of cattle infected persistently. Results obtained by this method show that IF is a good alternative to RT-PCR and antigen ELISA and can be a quick and accurate method in diagnosis of BVDV in cattle infected persistently with this virus.

  12. [The influence of dead lymphocytes from middle ear secretion on the development of sensorineural hearing impairment associated with acute otitis media].

    PubMed

    Yashan, A I; Khoruzhiĭ, I V

    2015-01-01

    The objective of the present work was to estimate the number of lymphocytes in the middle ear secretion that had died as a result of apoptosis or necrosis in the patients presenting with acute otitis media and to elucidate the relationship between this phenomenon and the development of sensorineural impairment of hearing. The study included a total of 106 patients suffering from acute middle otitis allocated two groups. Group 1 was comprised of 75 (70.8%) patients with hearing loss of the conductive type alone while group 2 contained 31 (29.2%) patients with the combined type of hearing loss. The contents of the tympanic cavity was obtained by means of tympanic puncture, the lymphocytes isolated from middle ear secretion were studied with the use of a flow cytometer in order to determine the number of dead cells. In the patients of group 1, 15.2±0.6% and 10.6±0.5% of all lymphocytes underwent apoptosis and necrosis respectively. In the patients of group, lymphocyte apoptosis was observed in 24.7±1.% of the cases (p<0.05) and necrosis in 14.0±0.5% ones (p<0.05). It was shown that liberation of intracellular proteolytic enzymes and cytokines taking place largely in the course of necrosis activates phagocytosis, stimulates the inflammatory reaction, and thereby promotes resolution of the pathological processes in the middle ear due to the prevention of their extension into the labyrinth.

  13. INNER EAR EMBRYOGENESIS: GENETIC AND ENVIRONMENTAL DETERMINANTS

    EPA Science Inventory

    The anatomy and developmental molecular genetics of the inner ear from establishment of the otic placode to formation of the definitive cochlea and vestibular apparatus will be reviewed and the complex 3-D structural changes that shape the developing inner ear will be illustrated...

  14. Preadolescent and adolescent endocrinology: physiology and physiopathology. II. Hormonal changes during abnormal pubertal development.

    PubMed

    Sizonenko, P C

    1978-08-01

    Based on the knowledge of the physiology of regulation of gonadotropins and gonadal steroids, basal levels of these hormones might be indicative of the etiologic factors of abnormal pubertal development. In addition, stimulatory tests may help in the diagnosis of such conditions. It is interesting that the pubertal maturation of the adrenal cortex is independent of the hypothalamic-pituitary-gonadal axis. The role of the adrenal cortex for the pubertal development remains questionable: adrenal androgens are low in isosexual precocious puberty, low in delayed adolescence, and normal in hyper- or hypogonadotropic hypogonadism. The importance of this role is doubled in congenital virilizing adrenal hyperplasia. When the disease is untreated, although adrenal androgens in excess advance bone age and hypothalamic maturation, girls remain prepubertal. When the therapeutic control is good, normal puberty occurs. The action of the adrenal androgens on growth and puberty remains to be determined.

  15. The character of abnormalities found in eye development of quail embruos exposed under space flight conditions

    NASA Astrophysics Data System (ADS)

    Grigoryan, E.; Dadheva, O.; Polinskaya, V.; Guryeva, T.

    The avian embryonic eye is used as a model system for studies on the environmental effects on central nervous system development. Here we present results of qualitative investigation of the eye development in quail embryos incubated in micro-"g" environment. In this study we used eyes of Japanese quail (Coturnix coturnix Japonica) embryos "flown" onboard biosatellite Kosmos-1129 and on Mir station within the framework of Mir-NASA Program. Eyes obtained from embryos ranging in age from 3-12 days (E3-E12) were prepared histologically and compared with those of the synchronous and laboratory gound controls. Ther most careful consideration was given to finding and analysis of eye developmental abnormalities. Then they were compared with those already described by experimental teratology for birds and mammals. At the stage of the "eye cup" (E3) we found the case of invalid formation of the inner retina. The latter was represented by disorganized neuroblasts occupying whole posterior chamber of the eye. On the 7th day of quail eye development, at the period of cellular growth activation some cases of small eyes with many folds of overgrowing neural and pigmented retinal layers were detected. In retinal folds of these eyes the normal layering was disturbed as well as the formation of aqueous body and pecten oculi. At this time point the changes were also found in the anterior part of the eye. The peculiarities came out of the bigger width of the cornea and separation of its layers, but were found in synchronous control as well. Few embryos of E10 had also eyes with the abnormities described for E7 but this time they were more vivid because of the completion of eye tissue differentiation. At the stage E12 we found the case evaluated as microphthalmia attending by overgrowth of anterior pigmented tissues - iris and ciliary body attached with the cornea. Most, but not all, of abnormalities we found in eye morphogeneses belonged to the birds "flown" aboard Kosmos- 1129 and

  16. The abnormal distribution of development: policies for southern women and children.

    PubMed

    Burman, E

    1995-03-01

    This paper offers a feminist critique of the relationships between gender and development by exploring the intersections between three sets of debates: firstly, the relations between interventions for women and for children through the anomalous position accorded to 'the girl child' in aid and development policies; secondly, the relations between psychological and economic models of development; and thirdly, the gendered and geographical allocation of attributes and opportunities. Drawing on analyses of the 'psychological complex' the author suggests that the cultural resources that inform developmental psychological models are highly cultural and class-specific (white, middle class, of the northern hemisphere), giving rise to a globalization of development that is reinscribed within international aid and development policies. In homogenizing difference to its norms, this globalization paradoxically reproduces the north-south opposition as an expression of cultural and political imperialism. While northern children 'develop', dominant discourses of children of the South are preoccupied with 'survival'. By such means the cultural hegemony of a unitary psychology remains intact. This paper discusses the 'abnormal distribution' of development to draw attention to the ways cultural and gender inequalities flow from the norms and generalized descriptions central to the current practice of developmental psychology and to urge that this is an important site of intervention for feminists addressing gender and development issues.

  17. The abnormal distribution of development: policies for southern women and children.

    PubMed

    Burman, E

    1995-03-01

    This paper offers a feminist critique of the relationships between gender and development by exploring the intersections between three sets of debates: firstly, the relations between interventions for women and for children through the anomalous position accorded to 'the girl child' in aid and development policies; secondly, the relations between psychological and economic models of development; and thirdly, the gendered and geographical allocation of attributes and opportunities. Drawing on analyses of the 'psychological complex' the author suggests that the cultural resources that inform developmental psychological models are highly cultural and class-specific (white, middle class, of the northern hemisphere), giving rise to a globalization of development that is reinscribed within international aid and development policies. In homogenizing difference to its norms, this globalization paradoxically reproduces the north-south opposition as an expression of cultural and political imperialism. While northern children 'develop', dominant discourses of children of the South are preoccupied with 'survival'. By such means the cultural hegemony of a unitary psychology remains intact. This paper discusses the 'abnormal distribution' of development to draw attention to the ways cultural and gender inequalities flow from the norms and generalized descriptions central to the current practice of developmental psychology and to urge that this is an important site of intervention for feminists addressing gender and development issues. PMID:12319980

  18. Effects of Weightlessness on Vestibular Development of Quail

    NASA Technical Reports Server (NTRS)

    Fritzsch, Bernd; Bruce, Laura L.

    1999-01-01

    The data confirm previous findings that quail embryos can, under proper circumstances, develop until hatching in microgravity. There were no gross abnormalities in the few ears of the late embryos (we received 3 ears at E14.5 and 4 ears at E16.5). Due to inadequate numbers of samples returned and their fully insufficient fixation, no conclusions could be reached that warrant any publications.

  19. Middle ear cholesteatoma in 11 dogs

    PubMed Central

    Greci, Valentina; Travetti, Olga; Di Giancamillo, Mauro; Lombardo, Rocco; Giudice, Chiara; Banco, Barbara; Mortellaro, Carlo M.

    2011-01-01

    Middle ear cholesteatoma is a rare condition in dogs with chronic otitis. Otorrhea, otodinia, and pain on temporomandibular joint palpation are the most common clinical signs. Neurological abnormalities are often detectable. Computed tomography reveals the presence of an expansive and invasive unvascularized lesion involving the tympanic cavity and the bulla, with little or no contrast enhancement after administration of contrast mediu. Video-otoscopy may detect pearly growth or white/yellowish scales in the middle ear cavity. Surgery is the only therapy but is associated with a high risk of recurrence. PMID:22131579

  20. Middle Ear Infections and Ear Tube Surgery (For Parents)

    MedlinePlus

    ... Zika & Pregnancy Middle Ear Infections and Ear Tube Surgery KidsHealth > For Parents > Middle Ear Infections and Ear ... medio y colocación de tubos de ventilación Why Surgery? Many kids get middle ear infections (known as ...

  1. A new mouse model with cochleo-saccular type inner ear defects.

    PubMed

    Oda, S; Hanai, A; Masaki, S; Yonezawa, S

    2001-10-01

    We found a new inner ear mutant exhibiting abnormal behavior, such as circling and head shaking, in a breeding stock of SJL/J mice. The traits are inherited in a simple autosomal-recessive fashion. Animals homozygous for the responsible gene, designated cosa, show no startle response to sounds and an inability to swim. In the inner ears of cosa/cosa homozygous, but not +/cosa heterozygous adults, histopathological features of severe damage that are typical for 'cochleo-saccular' or 'spotting' mutants have been demonstrated. We suggest here that the abnormal mice carry a mutation of a gene that is developmentally switched on in the early stages of development and is involved in endolymph homeostasis.

  2. Abnormal Mammary Development in 129:STAT1-Null Mice is Stroma-Dependent.

    PubMed

    Chen, Jane Q; Mori, Hidetoshi; Cardiff, Robert D; Trott, Josephine F; Hovey, Russell C; Hubbard, Neil E; Engelberg, Jesse A; Tepper, Clifford G; Willis, Brandon J; Khan, Imran H; Ravindran, Resmi K; Chan, Szeman R; Schreiber, Robert D; Borowsky, Alexander D

    2015-01-01

    Female 129:Stat1-null mice (129S6/SvEvTac-Stat1(tm1Rds) homozygous) uniquely develop estrogen-receptor (ER)-positive mammary tumors. Herein we report that the mammary glands (MG) of these mice have altered growth and development with abnormal terminal end buds alongside defective branching morphogenesis and ductal elongation. We also find that the 129:Stat1-null mammary fat pad (MFP) fails to sustain the growth of 129S6/SvEv wild-type and Stat1-null epithelium. These abnormalities are partially reversed by elevated serum progesterone and prolactin whereas transplantation of wild-type bone marrow into 129:Stat1-null mice does not reverse the MG developmental defects. Medium conditioned by 129:Stat1-null epithelium-cleared MFP does not stimulate epithelial proliferation, whereas it is stimulated by medium conditioned by epithelium-cleared MFP from either wild-type or 129:Stat1-null females having elevated progesterone and prolactin. Microarrays and multiplexed cytokine assays reveal that the MG of 129:Stat1-null mice has lower levels of growth factors that have been implicated in normal MG growth and development. Transplanted 129:Stat1-null tumors and their isolated cells also grow slower in 129:Stat1-null MG compared to wild-type recipient MG. These studies demonstrate that growth of normal and neoplastic 129:Stat1-null epithelium is dependent on the hormonal milieu and on factors from the mammary stroma such as cytokines. While the individual or combined effects of these factors remains to be resolved, our data supports the role of STAT1 in maintaining a tumor-suppressive MG microenvironment.

  3. Pathology of the Ear

    PubMed Central

    Orengo, Ida; Robbins, Kerri; Marsch, Amanda

    2011-01-01

    The external ear is exposed to weathering and trauma; it also has sparse vascularity, making it prone to infection and disease. The external location of the cutaneous ear makes it easily visible for diagnosis and accessible for treatment. In this article, the authors focus on diseases of the ear that are most commonly encountered and may be subject to surgical and medical evaluation and/or treatment. Epidemiology, pathogenesis, clinical course, and treatment for each disease entity are discussed. PMID:23115534

  4. Zucker diabetic fatty rats, a model for type 2 diabetes, develop an inner ear dysfunction that can be attenuated by losartan treatment.

    PubMed

    Meyer zum Gottesberge, Angela-Maria; Massing, Thomas; Sasse, Anja; Palma, Silvia; Hansen, Stefan

    2015-11-01

    Hearing loss secondary to diabetes remains under debate. In our study, we used Zucker Diabetic Fatty (ZDF) rats as an animal model of type 2 diabetes to investigate whether (1) hearing ability impairment and structural alterations of the inner ear occur in diabetes and (2) an angiotensin II receptor blocker (losartan) can protect rats from diabetic damage. Homozygous mutants were treated with a placebo or losartan and heterozygous animals served as non-diabetic controls. All animals underwent immunohistochemical and electronmicroscopical analysis. Functional testing of hearing ability was performed by click-evoked auditory brainstem responses. The present study showed significant sensorineural hearing impairment in placebo-treated diabetic rats (hearing threshold, 45.0 ± 2.1 dB SPL) compared to both non-diabetic controls (34.7 ± 4 dB SPL) and losartan-treated diabetic rats (36.1 ± 7.4 dB SPL). Concurrently, the functional decline in the placebo-treated rats was associated with significant morphological abnormalities, particularly in the intermediate cells of the stria vascularis and with strial dysfunction. These degenerative changes were indicated by the down-regulation of several pumps, ionic and cellular channels, which are involved in the cycling of K(+) and the maintenance of the endocochlear potential essential for the hearing process. Thus, the inner ear can be regarded as a target organ during hyperglycemic disorders and a metabolically induced "diabetic otopathy" may be added to angiopathy, nephropathy and neuropathy as a specific complication of diabetes mellitus. Blockade of the angiotensin II receptor can prevent this "diabetic otopathy" despite hyperglycemic serum levels.

  5. Apert and Crouzon syndromes-Cognitive development, brain abnormalities, and molecular aspects.

    PubMed

    Fernandes, Marilyse B L; Maximino, Luciana P; Perosa, Gimol B; Abramides, Dagma V M; Passos-Bueno, Maria Rita; Yacubian-Fernandes, Adriano

    2016-06-01

    Apert and Crouzon are the most common craniosynostosis syndromes associated with mutations in the fibroblast growth factor receptor 2 (FGFR2) gene. We conducted a study to examine the molecular biology, brain abnormalities, and cognitive development of individuals with these syndromes. A retrospective longitudinal review of 14 patients with Apert and Crouzon syndromes seen at the outpatient Craniofacial Surgery Hospital for Rehabilitation of Craniofacial Anomalies in Brazil from January 1999 through August 2010 was performed. Patients between 11 and 36 years of age (mean 18.29 ± 5.80), received cognitive evaluations, cerebral magnetic resonance imaging, and molecular DNA analyses. Eight patients with Apert syndrome (AS) had full scale intelligence quotients (FSIQs) that ranged from 47 to 108 (mean 76.9 ± 20.2), and structural brain abnormalities were identified in five of eight patients. Six patients presented with a gain-of-function mutation (p.Ser252Trp) in FGFR2 and FSIQs in those patients ranged from 47 to78 (mean 67.2 ± 10.7). One patient with a gain-of-function mutation (p.Pro253Arg) had a FSIQ of 108 and another patient with an atypical splice mutation (940-2A →G) had a FSIQ of 104. Six patients with Crouzon syndrome had with mutations in exons IIIa and IIIc of FGFR2 and their FSIQs ranged from 82 to 102 (mean 93.5 ± 6.7). These reveal that molecular aspects are another factor that can be considered in studies of global and cognitive development of patients with Apert and Crouzon syndrome (CS). © 2016 Wiley Periodicals, Inc. PMID:27028366

  6. Overcoming the right-ear advantage: a study of focused attention in children.

    PubMed

    Hiscock, M; Beckie, J L

    1993-09-01

    Several studies indicate that normal right-handed children of various ages show a right-ear advantage (REA) for dichotic verbal stimuli even when instructed to attend to the left ear. Other evidence, however, suggests that selective listening ability begins to develop in early childhood and that children reliably can overcome the REA by the age of 8 or 9 years. We used a signal detection procedure to address this apparent contradiction. In the first of two experiments, 58 children in two age groups (M = 7 and 10 years) were able to overcome the REA for dichotic consonant-vowel (CV) stimuli when instructed to focus attention on the left ear. Success in detecting and localizing signals from the left ear, as reflected in hit rates, was independent of age and reading level. A second experiment, in which strings of dichotic monosyllabic words were presented to 56 children at the same two age levels, yielded similar results. These findings challenge the claim that ability to overcome the REA when attending to the left ear reflects an abnormality of cerebral functioning. PMID:8276934

  7. Perspective methods of human identification: Ear biometrics

    NASA Astrophysics Data System (ADS)

    Choraś, M.

    2008-03-01

    Geometrical methods of feature extraction from ear images in order to perform human identification are presented. Geometrical approach is motivated by the actual procedures used by police and forensic experts (so-called ear otoscopy). In their work, geometrical features of ears such as size, height, width, and shapes of earlobe are useful and valid proofs of identity. The contribution of the article is development of the new and original methods of geometrical feature extraction from 2D ear images. Four novel algorithms of ear feature extraction from contour images are described in detail. Moreover, identification results obtained for each of the methods, based on the distance of feature vectors in the feature space, are presented.

  8. In vivo and in vitro biophysical properties of hair cells from the lateral line and inner ear of developing and adult zebrafish

    PubMed Central

    Olt, Jennifer; Johnson, Stuart L; Marcotti, Walter

    2014-01-01

    Hair cells detect and process sound and movement information, and transmit this with remarkable precision and efficiency to afferent neurons via specialized ribbon synapses. The zebrafish is emerging as a powerful model for genetic analysis of hair cell development and function both in vitro and in vivo. However, the full exploitation of the zebrafish is currently limited by the difficulty in obtaining systematic electrophysiological recordings from hair cells under physiological recording conditions. Thus, the biophysical properties of developing and adult zebrafish hair cells are largely unknown. We investigated potassium and calcium currents, voltage responses and synaptic activity in hair cells from the lateral line and inner ear in vivo and using near-physiological in vitro recordings. We found that the basolateral current profile of hair cells from the lateral line becomes more segregated with age, and that cells positioned in the centre of the neuromast show more mature characteristics and those towards the edge retain a more immature phenotype. The proportion of mature-like hair cells within a given neuromast increased with zebrafish development. Hair cells from the inner ear showed a developmental change in current profile between the juvenile and adult stages. In lateral line hair cells from juvenile zebrafish, exocytosis also became more efficient and required less calcium for vesicle fusion. In hair cells from mature zebrafish, the biophysical characteristics of ion channels and exocytosis resembled those of hair cells from other lower vertebrates and, to some extent, those in the immature mammalian vestibular and auditory systems. We show that although the zebrafish provides a suitable animal model for studies on hair cell physiology, it is advisable to consider that the age at which the majority of hair cells acquire a mature-type configuration is reached only in the juvenile lateral line and in the inner ear from >2 months after hatching. PMID

  9. Congenital Hydrocephalus and Abnormal Subcommissural Organ Development in Sox3 Transgenic Mice

    PubMed Central

    Lee, Kristie; Tan, Jacqueline; Morris, Michael B.; Rizzoti, Karine; Hughes, James; Cheah, Pike See; Felquer, Fernando; Liu, Xuan; Piltz, Sandra; Lovell-Badge, Robin; Thomas, Paul Q.

    2012-01-01

    Congenital hydrocephalus (CH) is a life-threatening medical condition in which excessive accumulation of CSF leads to ventricular expansion and increased intracranial pressure. Stenosis (blockage) of the Sylvian aqueduct (Aq; the narrow passageway that connects the third and fourth ventricles) is a common form of CH in humans, although the genetic basis of this condition is unknown. Mouse models of CH indicate that Aq stenosis is associated with abnormal development of the subcommmissural organ (SCO) a small secretory organ located at the dorsal midline of the caudal diencephalon. Glycoproteins secreted by the SCO generate Reissner's fibre (RF), a thread-like structure that descends into the Aq and is thought to maintain its patency. However, despite the importance of SCO function in CSF homeostasis, the genetic program that controls SCO development is poorly understood. Here, we show that the X-linked transcription factor SOX3 is expressed in the murine SCO throughout its development and in the mature organ. Importantly, overexpression of Sox3 in the dorsal diencephalic midline of transgenic mice induces CH via a dose-dependent mechanism. Histological, gene expression and cellular proliferation studies indicate that Sox3 overexpression disrupts the development of the SCO primordium through inhibition of diencephalic roof plate identity without inducing programmed cell death. This study provides further evidence that SCO function is essential for the prevention of hydrocephalus and indicates that overexpression of Sox3 in the dorsal midline alters progenitor cell differentiation in a dose-dependent manner. PMID:22291885

  10. Ear - blocked at high altitudes

    MedlinePlus

    ... ears; Flying and blocked ears; Eustachian tube dysfunction - high altitude ... the middle ear and the back of the nose and upper throat. ... down from high altitudes. Chewing gum the entire time you are ...

  11. Abnormal Development of Tapetum and Microspores Induced by Chemical Hybridization Agent SQ-1 in Wheat

    PubMed Central

    Wang, Shuping; Zhang, Gaisheng; Song, Qilu; Zhang, Yingxin; Li, Zheng; Guo, Jialin; Niu, Na; Ma, Shoucai; Wang, Junwei

    2015-01-01

    Chemical hybridization agent (CHA)-induced male sterility is an important tool in crop heterosis. To demonstrate that CHA-SQ-1-induced male sterility is associated with abnormal tapetal and microspore development, the cytology of CHA-SQ-1-treated plant anthers at various developmental stages was studied by light microscopy, scanning and transmission electron microscopy, in situ terminal deoxynucleotidyl transferasemediated dUTP nick end-labelling (TUNEL) assay and DAPI staining. The results indicated that the SQ-1-treated plants underwent premature tapetal programmed cell death (PCD), which was initiated at the early-uninucleate stage of microspore development and continued until the tapetal cells were completely degraded; the process of microspore development was then blocked. Microspores with low-viability (fluorescein diacetate staining) were aborted. The study suggests that premature tapetal PCD is the main cause of pollen abortion. Furthermore, it determines the starting period and a key factor in CHA-SQ-1-induced male sterility at the cell level, and provides cytological evidence to further study the mechanism between PCD and male sterility. PMID:25803723

  12. Abnormal development of tapetum and microspores induced by chemical hybridization agent SQ-1 in wheat.

    PubMed

    Wang, Shuping; Zhang, Gaisheng; Song, Qilu; Zhang, Yingxin; Li, Zheng; Guo, Jialin; Niu, Na; Ma, Shoucai; Wang, Junwei

    2015-01-01

    Chemical hybridization agent (CHA)-induced male sterility is an important tool in crop heterosis. To demonstrate that CHA-SQ-1-induced male sterility is associated with abnormal tapetal and microspore development, the cytology of CHA-SQ-1-treated plant anthers at various developmental stages was studied by light microscopy, scanning and transmission electron microscopy, in situ terminal deoxynucleotidyl transferasemediated dUTP nick end-labelling (TUNEL) assay and DAPI staining. The results indicated that the SQ-1-treated plants underwent premature tapetal programmed cell death (PCD), which was initiated at the early-uninucleate stage of microspore development and continued until the tapetal cells were completely degraded; the process of microspore development was then blocked. Microspores with low-viability (fluorescein diacetate staining) were aborted. The study suggests that premature tapetal PCD is the main cause of pollen abortion. Furthermore, it determines the starting period and a key factor in CHA-SQ-1-induced male sterility at the cell level, and provides cytological evidence to further study the mechanism between PCD and male sterility.

  13. Serotonin transporter variant drives preventable gastrointestinal abnormalities in development and function

    PubMed Central

    Margolis, Kara Gross; Li, Zhishan; Stevanovic, Korey; Saurman, Virginia; Anderson, George M.; Snyder, Isaac; Blakely, Randy D.; Gershon, Michael D.

    2016-01-01

    Autism spectrum disorder (ASD) is an increasingly common behavioral condition that frequently presents with gastrointestinal (GI) disturbances. It is not clear, however, how gut dysfunction relates to core ASD features. Multiple, rare hyperfunctional coding variants of the serotonin (5-HT) transporter (SERT, encoded by SLC6A4) have been identified in ASD. Expression of the most common SERT variant (Ala56) in mice increases 5-HT clearance and causes ASD-like behaviors. Here, we demonstrated that Ala56-expressing mice display GI defects that resemble those seen in mice lacking neuronal 5-HT. These defects included enteric nervous system hypoplasia, slow GI transit, diminished peristaltic reflex activity, and proliferation of crypt epithelial cells. An opposite phenotype was seen in SERT-deficient mice and in progeny of WT dams given the SERT antagonist fluoxetine. The reciprocal phenotypes that resulted from increased or decreased SERT activity support the idea that 5-HT signaling regulates enteric neuronal development and can, when disturbed, cause long-lasting abnormalities of GI function. Administration of a 5-HT4 agonist to Ala56 mice during development prevented Ala56-associated GI perturbations, suggesting that excessive SERT activity leads to inadequate 5-HT4–mediated neurogenesis. We propose that deficient 5-HT signaling during development may contribute to GI and behavioral features of ASD. The consequences of therapies targeting SERT during pregnancy warrant further evaluation. PMID:27111230

  14. Serotonin transporter variant drives preventable gastrointestinal abnormalities in development and function.

    PubMed

    Margolis, Kara Gross; Li, Zhishan; Stevanovic, Korey; Saurman, Virginia; Israelyan, Narek; Anderson, George M; Snyder, Isaac; Veenstra-VanderWeele, Jeremy; Blakely, Randy D; Gershon, Michael D

    2016-06-01

    Autism spectrum disorder (ASD) is an increasingly common behavioral condition that frequently presents with gastrointestinal (GI) disturbances. It is not clear, however, how gut dysfunction relates to core ASD features. Multiple, rare hyperfunctional coding variants of the serotonin (5-HT) transporter (SERT, encoded by SLC6A4) have been identified in ASD. Expression of the most common SERT variant (Ala56) in mice increases 5-HT clearance and causes ASD-like behaviors. Here, we demonstrated that Ala56-expressing mice display GI defects that resemble those seen in mice lacking neuronal 5-HT. These defects included enteric nervous system hypoplasia, slow GI transit, diminished peristaltic reflex activity, and proliferation of crypt epithelial cells. An opposite phenotype was seen in SERT-deficient mice and in progeny of WT dams given the SERT antagonist fluoxetine. The reciprocal phenotypes that resulted from increased or decreased SERT activity support the idea that 5-HT signaling regulates enteric neuronal development and can, when disturbed, cause long-lasting abnormalities of GI function. Administration of a 5-HT4 agonist to Ala56 mice during development prevented Ala56-associated GI perturbations, suggesting that excessive SERT activity leads to inadequate 5-HT4-mediated neurogenesis. We propose that deficient 5-HT signaling during development may contribute to GI and behavioral features of ASD. The consequences of therapies targeting SERT during pregnancy warrant further evaluation. PMID:27111230

  15. Ear canal cholesteatoma.

    PubMed

    Holt, J J

    1992-06-01

    Although cholesteatomas are more commonly found in the middle ear and the mastoid, the disease can occur in the external ear canal. All cases of ear canal cholesteatoma treated by the author were reviewed. There were nine ears in seven patients, who had an average age of 62 years. The lesions ranged in size from a few millimeters to extensive mastoid destruction. Smaller lesions can be managed by frequent cleaning as an office procedure. Larger lesions require surgery, either canaloplasty or mastoidectomy. The otolaryngologist should suspect this disease in the elderly. Microscopic examination of the ear with meticulous cleaning of all wax, especially in elderly patients, is most useful in detecting early disease. Frequent applications of mineral oil to the canal should be used in the management of the disease and to prevent recurrence.

  16. Identification and characterization of a repertoire of genes differentially expressed in developing top ear shoots between a superior hybrid and its parental inbreds in Zea mays L.

    PubMed

    Qin, Jun; Scheuring, Chantel F; Wei, Gang; Zhi, Hui; Zhang, Meiping; Huang, James J; Zhou, Xin; Galbraith, David W; Zhang, Hong-Bin

    2013-12-01

    Heterosis has been widely used in crop breeding and production; however, little is known about the genes controlling trait heterosis. The shortage of genes known to function in heterosis significantly limits our understanding of the molecular basis underlying heterosis. Here, we report 748 genes differentially expressed (DG) in the developing top ear shoots between a maize heterotic F1 hybrid (Mo17 × B73) and its parental inbreds identified using maize microarrays containing 28,608 unigene features. Of the 748 DG, over 600 were new for the inbred and hybrid combination. The DG were enriched for 35 of the total 213 maize gene ontology (GO) terms, including those describing photosynthesis, respiration, DNA replication, metabolism, and hormone biosynthesis. From the DG, we identified six genes involved in glycolysis, three genes in the citrate cycle, and four genes in the C4-dicarboxylic acid cycle. We mapped 533 of the 748 DG to the maize B73 genome, 298 (55.9 %) of which mapped to the QTL intervals of 11 maize ear traits. Moreover, we compared the repertoire of the DG with that of 14-day seedlings of the same inbred and hybrid combination. Only approximately 5 % of the DG was shared between the two organs and developmental stages. Furthermore, we mapped 417 (55.7 %) of the 748 maize DG to the QTL intervals of 26 rice yield-related traits. Therefore, this study provides a repertoire of genes useful for identification of genes involved in maize ear trait heterosis and information for a better understanding of the molecular basis underlying heterosis in maize.

  17. Ultrastructural and cellular basis for the development of abnormal myocardial mechanics during the transition from hypertension to heart failure.

    PubMed

    Shah, Sanjiv J; Aistrup, Gary L; Gupta, Deepak K; O'Toole, Matthew J; Nahhas, Amanda F; Schuster, Daniel; Chirayil, Nimi; Bassi, Nikhil; Ramakrishna, Satvik; Beussink, Lauren; Misener, Sol; Kane, Bonnie; Wang, David; Randolph, Blake; Ito, Aiko; Wu, Megan; Akintilo, Lisa; Mongkolrattanothai, Thitipong; Reddy, Mahendra; Kumar, Manvinder; Arora, Rishi; Ng, Jason; Wasserstrom, J Andrew

    2014-01-01

    Although the development of abnormal myocardial mechanics represents a key step during the transition from hypertension to overt heart failure (HF), the underlying ultrastructural and cellular basis of abnormal myocardial mechanics remains unclear. We therefore investigated how changes in transverse (T)-tubule organization and the resulting altered intracellular Ca(2+) cycling in large cell populations underlie the development of abnormal myocardial mechanics in a model of chronic hypertension. Hearts from spontaneously hypertensive rats (SHRs; n = 72) were studied at different ages and stages of hypertensive heart disease and early HF and were compared with age-matched control (Wistar-Kyoto) rats (n = 34). Echocardiography, including tissue Doppler and speckle-tracking analysis, was performed just before euthanization, after which T-tubule organization and Ca(2+) transients were studied using confocal microscopy. In SHRs, abnormalities in myocardial mechanics occurred early in response to hypertension, before the development of overt systolic dysfunction and HF. Reduced longitudinal, circumferential, and radial strain as well as reduced tissue Doppler early diastolic tissue velocities occurred in concert with T-tubule disorganization and impaired Ca(2+) cycling, all of which preceded the development of cardiac fibrosis. The time to peak of intracellular Ca(2+) transients was slowed due to T-tubule disruption, providing a link between declining cell ultrastructure and abnormal myocardial mechanics. In conclusion, subclinical abnormalities in myocardial mechanics occur early in response to hypertension and coincide with the development of T-tubule disorganization and impaired intracellular Ca(2+) cycling. These changes occur before the development of significant cardiac fibrosis and precede the development of overt cardiac dysfunction and HF.

  18. Prenatal ketamine exposure causes abnormal development of prefrontal cortex in rat

    PubMed Central

    Zhao, Tianyun; Li, Chuanxiang; Wei, Wei; Zhang, Haixing; Ma, Daqing; Song, Xingrong; Zhou, Libing

    2016-01-01

    Ketamine is commonly used for anesthesia and as a recreational drug. In pregnant users, a potential neurotoxicity in offspring has been noted. Our previous work demonstrated that ketamine exposure of pregnant rats induces affective disorders and cognitive impairments in offspring. As the prefrontal cortex (PFC) is critically involved in emotional and cognitive processes, here we studied whether maternal ketamine exposure influences the development of the PFC in offspring. Pregnant rats on gestational day 14 were treated with ketamine at a sedative dose for 2 hrs, and pups were studied at postnatal day 0 (P0) or P30. We found that maternal ketamine exposure resulted in cell apoptosis and neuronal loss in fetal brain. Upon ketamine exposure in utero, PFC neurons at P30 showed more dendritic branching, while cultured neurons from P0 PFC extended shorter neurites than controls. In addition, maternal ketamine exposure postponed the switch of NR2B/2A expression, and perturbed pre- and postsynaptic protein expression in the PFC. These data suggest that prenatal ketamine exposure impairs neuronal development of the PFC, which may be associated with abnormal behavior in offsprings. PMID:27226073

  19. Over-expression of thymosin beta 4 promotes abnormal tooth development and stimulation of hair growth.

    PubMed

    Cha, Hee-Jae; Philp, Deborah; Lee, Soo-Hyun; Moon, Hye-Sung; Kleinman, Hynda K; Nakamura, Takashi

    2010-01-01

    Thymosin beta 4 has multi-functional roles in cell physiology. It accelerates wound healing, hair growth and angiogenesis, and increases laminin-5 expression in corneal epithelium. Furthermore, thymosin beta 4 stimulates tumor growth and metastasis by induction of cell migration and vascular endothelial growth factor-mediated angiogenesis. Using a construct on the skin-specific keratin-5 promoter, we have developed thymosin beta 4 over-expressing transgenic mice to further study its functional roles. Thymosin beta 4 in adult skin and in embryonic stages of the transgenic mouse was analyzed by both Western blot and immunohistochemistry. The over-expression of thymosin beta 4 was observed especially around hair follicles and in the teeth in the transgenic mice. We examined the phenotype of the thymosin beta 4 over-expressing mice. Hair growth was accelerated. In addition, the transgenic mice had abnormally-shaped white teeth and dull incisors. We found that the expression of laminin-5 was up-regulated in the skin of the transgenic mice. We conclude that thymosin beta 4 has an important physiological role in hair growth and in tooth development.

  20. Neural tube defects and abnormal brain development in F52-deficient mice.

    PubMed Central

    Wu, M; Chen, D F; Sasaoka, T; Tonegawa, S

    1996-01-01

    F52 is a myristoylated, alanine-rich substrate for protein kinase C. We have generated F52-deficient mice by the gene targeting technique. These mutant mice manifest severe neural tube defects that are not associated with other complex malformations, a phenotype reminiscent of common human neural tube defects. The neural tube defects observed include both exencephaly and spina bifida, and the phenotype exhibits partial penetrance with about 60% of homozygous embryos developing neural tube defects. Exencephaly is the prominent type of defect and leads to high prenatal lethality. Neural tube defects are observed in a smaller percentage of heterozygous embryos (about 10%). Abnormal brain development and tail formation occur in homozygous mutants and are likely to be secondary to the neural tube defects. Disruption of F52 in mice therefore identifies a gene whose mutation results in isolated neural tube defects and may provide an animal model for common human neural tube defects. Images Fig. 1 Fig. 2 Fig. 3 Fig. 4 Fig. 5 PMID:8700893

  1. Facial Metrics in Children with Corticotrophin-Producing Pituitary Adenomas Suggest Abnormalities in Midface Development

    PubMed Central

    Keil, Margaret F.; Stratakis, Constantine A.

    2011-01-01

    Background Tumors of the hypothalamic-pituitary unit have been linked to genetic syndromes that are associated with midfacial abnormalities. Aim We hypothesized that mutations of genes that affect the development of the face (and consequently of the anterior pituitary) may be present in children with ACTH-producing pituitary adenomas, and if this is true then facial measurements would be different from those predicted by parental features. Methods We studied 20 children with cortico-tropinomas and a control group and their parents. All facial measurements were expressed according to standard deviation scores. Results Significant differences were seen between the children with pituitary adenomas and their parents for vertical facial height measures: nasal length (p <0.001), lower facial height (p <0.03) and overall facial height (p <0.01). Conclusion We conclude that some of the indices of midline craniofacial development, in particular those affecting the vertical axis, are different in children with corticotroph adenomas producing ACTH. PMID:19344074

  2. Ear problems in swimmers.

    PubMed

    Wang, Mao-Che; Liu, Chia-Yu; Shiao, An-Suey; Wang, Tyrone

    2005-08-01

    Acute diffuse otitis externa (swimmer's ear), otomycosis, exostoses, traumatic eardrum perforation, middle ear infection, and barotraumas of the inner ear are common problems in swimmers and people engaged in aqua activities. The most common ear problem in swimmers is acute diffuse otitis externa, with Pseudomonas aeruginosa being the most common pathogen. The symptoms are itching, otalgia, otorrhea, and conductive hearing loss. The treatment includes frequent cleansing of the ear canal, pain control, oral or topical medications, acidification of the ear canal, and control of predisposing factors. Swimming in polluted waters and ear-canal cleaning with cotton-tip applicators should be avoided. Exostoses are usually seen in people who swim in cold water and present with symptoms of accumulated debris, otorrhea and conductive hearing loss. The treatment for exostoses is transmeatal surgical removal of the tumors. Traumatic eardrum perforations may occur during water skiing or scuba diving and present with symptoms of hearing loss, otalgia, otorrhea, tinnitus and vertigo. Tympanoplasty might be needed if the perforations do not heal spontaneously. Patients with chronic otitis media with active drainage should avoid swimming, while patients who have undergone mastoidectomy and who have no cavity problems may swim. For children with ventilation tubes, surface swimming is safe in a clean, chlorinated swimming pool. Sudden sensorineural hearing loss and some degree of vertigo may occur after diving because of rupture of the round or oval window membrane.

  3. Backdoor pathway for dihydrotestosterone biosynthesis: implications for normal and abnormal human sex development.

    PubMed

    Fukami, Maki; Homma, Keiko; Hasegawa, Tomonobu; Ogata, Tsutomu

    2013-04-01

    We review the current knowledge about the "backdoor" pathway for the biosynthesis of dihydrotestosterone (DHT). While DHT is produced from cholesterol through the conventional "frontdoor" pathway via testosterone, recent studies have provided compelling evidence for the presence of an alternative "backdoor" pathway to DHT without testosterone intermediacy. This backdoor pathway is known to exist in the tammar wallaby pouch young testis and the immature mouse testis, and has been suggested to be present in the human as well. Indeed, molecular analysis has identified pathologic mutations of genes involved in the backdoor pathway in genetic male patients with undermasculinized external genitalia, and urine steroid profile analysis has argued for the relevance of the activated backdoor pathway to abnormal virilization in genetic females with cytochrome P450 oxidoreductase deficiency and 21-hydroxylase deficiency. It is likely that the backdoor pathway is primarily operating in the fetal testis in a physiological condition to produce a sufficient amount of DHT for male sex development, and that the backdoor pathway is driven with a possible interaction between fetal and permanent adrenals in pathologic conditions with increased 17-hydroxyprogesterone levels. These findings provide novel insights into androgen biosynthesis in both physiological and pathological conditions. PMID:23073980

  4. Abnormalities in synaptic dynamics during development in a mouse model of spinocerebellar ataxia type 1

    PubMed Central

    Hatanaka, Yusuke; Watase, Kei; Wada, Keiji; Nagai, Yoshitaka

    2015-01-01

    Late-onset neurodegenerative diseases are characterized by neurological symptoms and progressive neuronal death. Accumulating evidence suggests that neuronal dysfunction, rather than neuronal death, causes the symptoms of neurodegenerative diseases. However, the mechanisms underlying the dysfunction that occurs prior to cell death remain unclear. To investigate the synaptic basis of this dysfunction, we employed in vivo two-photon imaging to analyse excitatory postsynaptic dendritic protrusions. We used Sca1154Q/2Q mice, an established knock-in mouse model of the polyglutamine disease spinocerebellar ataxia type 1 (SCA1), which replicates human SCA1 features including ataxia, cognitive impairment, and neuronal death. We found that Sca1154Q/2Q mice exhibited greater synaptic instability than controls, without synaptic loss, in the cerebral cortex, where obvious neuronal death is not observed, even before the onset of distinct symptoms. Interestingly, this abnormal synaptic instability was evident in Sca1154Q/2Q mice from the synaptic developmental stage, and persisted into adulthood. Expression of synaptic scaffolding proteins was also lower in Sca1154Q/2Q mice than controls before synaptic maturation. As symptoms progressed, synaptic loss became evident. These results indicate that aberrant synaptic instability, accompanied by decreased expression of scaffolding proteins during synaptic development, is a very early pathology that precedes distinct neurological symptoms and neuronal cell death in SCA1. PMID:26531852

  5. Abnormal germling development by brown rust and powdery mildew on cer barley mutants.

    PubMed

    Rubiales, D; Ramirez, M C; Carver, T L; Niks, R E

    2001-01-01

    The barley leaf rust fungus forms appressoria over host leaf stomata and penetrates via the stomatal pore. High levels of avoidance to leaf rust fungi have been described in some wild accessions of Hordeum species where a prominent wax layer on the stomata inhibits triggering of fungal appressorium differentiation. Leaf rust avoidance has not yet been found in H. vulgare. Since cuticular leaf waxes are implicated in the avoidance trait, we screened 27 eceriferum (cer) mutant lines of H. vulgare for avoidance to barley leaf rust. These mutations affect leaf waxes. Reduction in numbers of germ tubes forming appressoria over stomata was found in some lines, but the greatest reduction (ca 30%) was less than previously found in wild barley spp. or in an accession of H. chilense used here as a check. In one line (cer-zh654), avoidance was due to a combination of factors. Firstly, fewer germ tubes oriented towards stomata and so failed to contact them. Secondly, some germ tubes that encountered stomata did not form appressoria but over-grew them. In this line, therefore, the fungus tended to fail both to locate and to respond to stomata. The appressoria of barley powdery mildew form on leaf epidermal cells that they penetrate directly. On certain cer lines, a proportion of germlings of the barley powdery mildew fungus developed abnormally, suggesting that germlings failed to recognise and/or respond to the leaf surface waxes on these mutants.

  6. Abnormalities in synaptic dynamics during development in a mouse model of spinocerebellar ataxia type 1.

    PubMed

    Hatanaka, Yusuke; Watase, Kei; Wada, Keiji; Nagai, Yoshitaka

    2015-11-04

    Late-onset neurodegenerative diseases are characterized by neurological symptoms and progressive neuronal death. Accumulating evidence suggests that neuronal dysfunction, rather than neuronal death, causes the symptoms of neurodegenerative diseases. However, the mechanisms underlying the dysfunction that occurs prior to cell death remain unclear. To investigate the synaptic basis of this dysfunction, we employed in vivo two-photon imaging to analyse excitatory postsynaptic dendritic protrusions. We used Sca1(154Q/2Q) mice, an established knock-in mouse model of the polyglutamine disease spinocerebellar ataxia type 1 (SCA1), which replicates human SCA1 features including ataxia, cognitive impairment, and neuronal death. We found that Sca1(154Q/2Q) mice exhibited greater synaptic instability than controls, without synaptic loss, in the cerebral cortex, where obvious neuronal death is not observed, even before the onset of distinct symptoms. Interestingly, this abnormal synaptic instability was evident in Sca1(154Q/2Q) mice from the synaptic developmental stage, and persisted into adulthood. Expression of synaptic scaffolding proteins was also lower in Sca1(154Q/2Q) mice than controls before synaptic maturation. As symptoms progressed, synaptic loss became evident. These results indicate that aberrant synaptic instability, accompanied by decreased expression of scaffolding proteins during synaptic development, is a very early pathology that precedes distinct neurological symptoms and neuronal cell death in SCA1.

  7. Tympanometric evaluation of middle ear barotrauma during recreational scuba diving.

    PubMed

    Green, S M; Rothrock, S G; Green, E A

    1993-10-01

    We report the first prospective evaluation of middle ear barotrauma in experienced recreational scuba divers. In this pilot study, tympanometric and otoscopic evaluations were performed daily on two experienced scuba divers engaged in multi-day repetitive diving. Middle ear pressures decreased in proportion to diving frequency, demonstrating eustachian tube dysfunction which promptly reversed upon cessation of diving Otoscopic evidence of traumatic injury to the middle ear occurred in proportion to diving frequency, and also readily reversed upon cessation of diving. Tympanic membrane compliance remained normal, often despite pronounced otoscopic abnormalities. Otologic symptoms and impairment of acuity were not observed. Tympanometry appears to be a valuable modality for the verification of middle ear hemorrhage or tympanic membrane rupture. This preliminary data should assist investigators in planning more comprehensive studies of middle ear barotrauma, including clinical trials of treatment and prophylactic interventions for this common condition.

  8. Genome-Wide Identification of miRNAs and Their Targets Involved in the Developing Internodes under Maize Ears by Responding to Hormone Signaling

    PubMed Central

    Yang, Huili; Li, Huimin; Sun, Gaoyang; Zhao, Xiaofeng; Ding, Dong; Tang, Jihua

    2016-01-01

    Internode length is one of the decisive factors affecting plant height (PH) and ear height (EH), which are closely associated with the lodging resistance, biomass and grain yield of maize. miRNAs, currently recognized as important transcriptional/ post-transcriptional regulators, play an essential role in plant growth and development. However, their roles in developing internodes under maize ears remain unclear. To identify the roles of miRNAs and their targets in the development of internodes under maize ears, six miRNA and two degradome libraries were constructed using the 7th, 8th and 9th internodes of two inbred lines, ‘Xun928’ and ‘Xun9058’, which had significantly different internode lengths. A total of 45 and 54 miRNAs showed significant changes for each pairwise comparison among the 7th, 8th and 9th internodes of ‘Xun9058’ and ‘Xun928’, respectively. The expression of 31 miRNAs showed significant changes were common to the corresponding comparison groups of the 7th, 8th and 9th internodes of ‘Xun9058’ and ‘Xun928’. For the corresponding internodes of ‘Xun9058’ and ‘Xun928’, compared with the expression of miRNAs in the 7th, 8th and 9th internodes of ‘Xun928’, the numbers of up-regulated and down-regulated miRNAs were 11 and 36 in the 7th internode, 9 and 45 in the 8th internode, and 9 and 25 in the 9th internode of ‘Xun9058’, respectively. Moreover, 10 miRNA families containing 45 members showed significant changes at least in two internodes of ‘Xun928’ by comparing with the corresponding internodes of ‘Xun9058’. Based on the sequencing data, 20 miRNAs related to hormone signaling among the candidates, belonging to five conserved miRNA families, were selected for expression profiling using quantitative reverse-transcription polymerase chain reaction (qRT-PCR). The five miRNA families, zma-miR160, zma-miR167, zma-miR164, zma-miR169 and zma-miR393, targeted the genes encoding auxin response factor, N

  9. Ear infection - acute

    MedlinePlus

    ... Risk factors for acute ear infections include: Attending day care (especially centers with more than 6 children) Changes ... hands and toys often. If possible, choose a day care that has 6 or fewer children. This can ...

  10. Ear Injuries (For Parents)

    MedlinePlus

    ... the eardrum, ear canal, ossicles, cochlea, or the vestibular nerve. Here's a look at the most common ... may cause permanent hearing loss or balance problems. Vestibular therapy may help kids with balance problems. And ...

  11. Middle Ear Infections

    MedlinePlus

    ... Health Issues Conditions Abdominal ADHD Allergies & Asthma Autism Cancer Chest & Lungs Chronic Conditions Cleft & Craniofacial Developmental Disabilities Ear Nose & Throat Emotional Problems Eyes Fever From Insects or Animals Genitals and Urinary Tract Glands & Growth ...

  12. Sports injuries of the ear.

    PubMed

    Wagner, G A

    1972-07-01

    The author describes common sports injuries involving the ear. Such injuries include hematoma, lacerations, foreign bodies (tattoo), and thermal injuries. Ear canal injuries include swimmer's ear and penetrating injuries. Tympanum injuries include tympanic membrane perforations, ossicular discontinuity, eustachian tube dysfunction, temporal bone fractures and traumatic facial nerve palsy. Inner ear injuries include traumatic sensorineural deafness. The author emphasizes the management of these injuries.

  13. Development, support, evaluation, and productivity of community-based Earth science research facilities: The NSF-EAR/IF model and why it has been so successful

    NASA Astrophysics Data System (ADS)

    Hochella, M. F.

    2002-12-01

    The Instrumentation and Facilities (IF) program within the Division of Earth Sciences (EAR), Directorate for Geosciences, at the National Science Foundation in Washington, D.C., has been highly successful in encouraging the development of, and thereafter nurturing, a number of outstanding community-based research facilities. Currently about 70% of the EAR/IF budget goes to 13 such facilities, accounting for about \\$20M annually. These facilities range widely in their scope and cost of operations, from laboratories with single instruments whose in-house directors give outside academic users scheduling priorities and lower cost per time rates, to very large consortia of universities with an extensive instrumentation base and funding from a number of sources beyond NSF. The attribute that all of these facilities share is the ability to bring to the at-large user community facilities that would be otherwise impractical to provide to such a number of individual investigators. Especially when this NSF funding is supplemented by support from the participating universities and other agencies, these facilities have been exceptionally successful and long-lived, in part due to the rigorous scrutiny of extensive and objective review procedures. Perhaps most interesting of all is the fact the NSF scientific staff, and the staffs of cooperating agencies, as program administrators, are skilled in knowing how to keep in mind the pulse of the community that they serve and the direction of future scientific development. As they collaborate in the development of new initiatives, NSF staff are really mirrors, reflecting and focusing the carefully formulated visions determined by the outside community itself. Within this system, EAR/IF has multiple checks and balances to keep the research that they are presently supporting fresh, progressive, and often unique. These include constantly searching for new initiatives, as well as developing innovative cost-sharing strategies with other

  14. Estimation of outer-middle ear transmission using DPOAEs and fractional-order modeling of human middle ear

    NASA Astrophysics Data System (ADS)

    Naghibolhosseini, Maryam

    Our ability to hear depends primarily on sound waves traveling through the outer and middle ear toward the inner ear. Hence, the characteristics of the outer and middle ear affect sound transmission to/from the inner ear. The role of the middle and outer ear in sound transmission is particularly important for otoacoustic emissions (OAEs), which are sound signals generated in a healthy cochlea, and recorded by a sensitive microphone placed in the ear canal. OAEs are used to evaluate the health and function of the cochlea; however, they are also affected by outer and middle ear characteristics. To better assess cochlear health using OAEs, it is critical to quantify the impact of the outer and middle ear on sound transmission. The reported research introduces a noninvasive approach to estimate outer-middle ear transmission using distortion product otoacoustic emissions (DPOAEs). In addition, the role of the outer and middle ear on sound transmission was investigated by developing a physical/mathematical model, which employed fractional-order lumped elements to include the viscoelastic characteristics of biological tissues. Impedance estimations from wideband refectance measurements were used for parameter fitting of the model. The model was validated comparing its estimates of the outer-middle ear sound transmission with those given by DPOAEs. The outer-middle ear transmission by the model was defined as the sum of forward and reverse outer-middle ear transmissions. To estimate the reverse transmission by the model, the probe-microphone impedance was calculated through estimating the Thevenin-equivalent circuit of the probe-microphone. The Thevenin-equivalent circuit was calculated using measurements in a number of test cavities. Such modeling enhances our understanding of the roles of different parts of the outer and middle ear and how they work together to determine their function. In addition, the model would be potentially helpful in diagnosing pathologies of

  15. Effects of altered gravity on the expression of Calcium -binding and matrix proteins in the inner ear of developing fish following ∆g-expositions.

    NASA Astrophysics Data System (ADS)

    Hilbig, Reinhard; Hendrik Anken, Ralf; Weigele, Jochen

    The results of the Foton-M3 mission (OmegaHab) give evidence that the otoliths of the fish form OmegaHab were larger as compared to the ground control. Additionally the shape (raphe) and morphology especially the mode of crystallization of the otoliths were affected during growth in weightlessness. The reason for these changes is assumed to originate from changes in the composition of the otolith matrix and Ca-binding proteins (OMP). The OMPs play an important role in controlling the crystallization process and additionally the morphology of crystals, determining the crystallpolymorph and the strength of the crystals. The matrix of otoliths is a complex functional structure containing several calcium-binding proteins, structural proteins and protease inhibitors. Furthermore it is composed of otolith matrix protein-1, Otolin, Otoconin, SPARC and Neuroserpin, which is a specific expression in the otolth matrix for chichlid fish. During embryonic development of the fish inner ear, these proteins show a spacial and temporal expression pattern. The formation of the inner ear -including otoliths and sensory cells -starting from the otocyst-anlage -can be subdivided in several major developmental stages e.g. the forming of the otic cavity (stage 7/8), the tetha cell or seeding stage (stage 8, 9), the development of the semicircular channels (stage 12), the transition to further daily growth (post stage15) and the development of the third otolith, asteriscus (stage 23). These developmental phases contain different constitutions or involvements of matrix proteins. We investigated the matrixprotein composition of the chichlid fish Oreochromis mossambicus and found that the otolith matrix differentiate between other fishes. In this case some matrix proteins seem to be uniform in fishes, other known matrix proteins are lacking and we have also references to new candidates for matrix proteins chichlids. In this case we investigated the expression of the matrix proteins otolith

  16. Structure and function of the mammalian middle ear. I: Large middle ears in small desert mammals.

    PubMed

    Mason, Matthew J

    2016-02-01

    Many species of small desert mammals are known to have expanded auditory bullae. The ears of gerbils and heteromyids have been well described, but much less is known about the middle ear anatomy of other desert mammals. In this study, the middle ears of three gerbils (Meriones, Desmodillus and Gerbillurus), two jerboas (Jaculus) and two sengis (elephant-shrews: Macroscelides and Elephantulus) were examined and compared, using micro-computed tomography and light microscopy. Middle ear cavity expansion has occurred in members of all three groups, apparently in association with an essentially 'freely mobile' ossicular morphology and the development of bony tubes for the middle ear arteries. Cavity expansion can occur in different ways, resulting in different subcavity patterns even between different species of gerbils. Having enlarged middle ear cavities aids low-frequency audition, and several adaptive advantages of low-frequency hearing to small desert mammals have been proposed. However, while Macroscelides was found here to have middle ear cavities so large that together they exceed brain volume, the bullae of Elephantulus are considerably smaller. Why middle ear cavities are enlarged in some desert species but not others remains unclear, but it may relate to microhabitat.

  17. Making connections in the inner ear: recent insights into the development of spiral ganglion neurons and their connectivity with sensory hair cells

    PubMed Central

    Coate, Thomas M.; Kelley, Matthew W.

    2013-01-01

    In mammals, auditory information is processed by the hair cells (HCs) located in the cochlea and then rapidly transmitted to the CNS via a specialized cluster of bipolar afferent connections known as the spiral ganglion neurons (SGNs). Although many anatomical aspects of SGNs are well described, the molecular and cellular mechanisms underlying their genesis, how they are precisely arranged along the cochlear duct, and the guidance mechanisms that promote the innervation of their hair cell targets are only now being understood. Building upon foundational studies of neurogenesis and neurotrophins, we review here new concepts and technologies that are helping to enrich our understanding of the development of the nervous system within the inner ear. PMID:23660234

  18. Human fetal inner ear involvement in congenital cytomegalovirus infection

    PubMed Central

    2013-01-01

    Background Congenital cytomegalovirus (CMV) infection is a leading cause of sensorineural hearing loss (SNHL). The mechanisms of pathogenesis of CMV-related SNHL are still unclear. The aim is to study congenital CMV-related damage in the fetal inner ear, in order to better understand the underlying pathophysiology behind CMV-SNHL. Results We studied inner ears and brains of 20 human fetuses, all at 21 week gestational age, with a high viral load in the amniotic fluid, with and without ultrasound (US) brain abnormalities. We evaluated histological brain damage, inner ear infection, local inflammatory response and tissue viral load. Immunohistochemistry revealed that CMV was positive in 14/20 brains (70%) and in the inner ears of 9/20 fetuses (45%). In the cases with inner ear infection, the marginal cell layer of the stria vascularis was always infected, followed by infection in the Reissner’s membrane. The highest tissue viral load was observed in the inner ear with infected Organ of Corti. Vestibular labyrinth showed CMV infection of sensory cells in the utricle and in the crista ampullaris. US cerebral anomalies were detected in 6 cases, and in all those cases, the inner ear was always involved. In the other 14 cases with normal brain scan, histological brain damage was present in 8 fetuses and 3 of them presented inner ear infection. Conclusions CMV-infection of the marginal cell layer of the stria vascularis may alter potassium and ion circulation, dissipating the endocochlear potential with consequent SNHL. Although abnormal cerebral US is highly predictive of brain and inner ear damage, normal US findings cannot exclude them either. PMID:24252374

  19. Abnormal cerebellar development and axonal decussation due to mutations in AHI1 in Joubert syndrome.

    PubMed

    Ferland, Russell J; Eyaid, Wafaa; Collura, Randall V; Tully, Laura D; Hill, R Sean; Al-Nouri, Doha; Al-Rumayyan, Ahmed; Topcu, Meral; Gascon, Generoso; Bodell, Adria; Shugart, Yin Yao; Ruvolo, Maryellen; Walsh, Christopher A

    2004-09-01

    Joubert syndrome is a congenital brain malformation of the cerebellar vermis and brainstem with abnormalities of axonal decussation (crossing in the brain) affecting the corticospinal tract and superior cerebellar peduncles. Individuals with Joubert syndrome have motor and behavioral abnormalities, including an inability to walk due to severe clumsiness and 'mirror' movements, and cognitive and behavioral disturbances. Here we identified a locus associated with Joubert syndrome, JBTS3, on chromosome 6q23.2-q23.3 and found three deleterious mutations in AHI1, the first gene to be associated with Joubert syndrome. AHI1 is most highly expressed in brain, particularly in neurons that give rise to the crossing axons of the corticospinal tract and superior cerebellar peduncles. Comparative genetic analysis of AHI1 indicates that it has undergone positive evolutionary selection along the human lineage. Therefore, changes in AHI1 may have been important in the evolution of human-specific motor behaviors.

  20. Sex differences in abnormal white matter development associated with conduct disorder in children.

    PubMed

    Decety, Jean; Yoder, Keith J; Lahey, Benjamin B

    2015-08-30

    Associations between white matter pathway abnormalities and antisocial personality disorder in adults are well replicated, and there is some evidence for an association of white matter abnormalities with conduct disorder (CD) in adolescents. In this study, white matter maturation using diffusion tensor imaging (DTI) was examined in 110 children aged 10.0 ± 0.8 years selected to vary widely in their numbers of CD symptoms. The results replicated age-related increases in fractional anisotropy (FA) found in previous studies. There was not a significant association between the number of CD symptoms and FA, but CD symptoms were found to be significantly associated with greater axial and radial diffusivity in a broad range of white matter tracts, particularly in girls. In complementary analyses, there were similar significant differences in axial and radial diffusivity between children who met diagnostic criteria for CD and healthy children with no symptoms of CD, particularly in girls. Brain structural abnormalities may contribute to the emergence of CD in childhood, perhaps playing a greater role in girls.

  1. Disruption of Ah Receptor Signaling during Mouse Development Leads to Abnormal Cardiac Structure and Function in the Adult

    PubMed Central

    Carreira, Vinicius S.; Fan, Yunxia; Kurita, Hisaka; Wang, Qin; Ko, Chia-I; Naticchioni, Mindi; Jiang, Min; Koch, Sheryl; Zhang, Xiang; Biesiada, Jacek; Medvedovic, Mario; Xia, Ying; Rubinstein, Jack; Puga, Alvaro

    2015-01-01

    The Developmental Origins of Health and Disease (DOHaD) Theory proposes that the environment encountered during fetal life and infancy permanently shapes tissue physiology and homeostasis such that damage resulting from maternal stress, poor nutrition or exposure to environmental agents may be at the heart of adult onset disease. Interference with endogenous developmental functions of the aryl hydrocarbon receptor (AHR), either by gene ablation or by exposure in utero to 2,3,7,8-tetrachlorodibenzo-p-dioxin (TCDD), a potent AHR ligand, causes structural, molecular and functional cardiac abnormalities and altered heart physiology in mouse embryos. To test if embryonic effects progress into an adult phenotype, we investigated whether Ahr ablation or TCDD exposure in utero resulted in cardiac abnormalities in adult mice long after removal of the agent. Ten-months old adult Ahr-/- and in utero TCDD-exposed Ahr+/+ mice showed sexually dimorphic abnormal cardiovascular phenotypes characterized by echocardiographic findings of hypertrophy, ventricular dilation and increased heart weight, resting heart rate and systolic and mean blood pressure, and decreased exercise tolerance. Underlying these effects, genes in signaling networks related to cardiac hypertrophy and mitochondrial function were differentially expressed. Cardiac dysfunction in mouse embryos resulting from AHR signaling disruption seems to progress into abnormal cardiac structure and function that predispose adults to cardiac disease, but while embryonic dysfunction is equally robust in males and females, the adult abnormalities are more prevalent in females, with the highest severity in Ahr-/- females. The findings reported here underscore the conclusion that AHR signaling in the developing heart is one potential target of environmental factors associated with cardiovascular disease. PMID:26555816

  2. The HyPRP gene EARLI1 has an auxiliary role for germinability and early seedling development under low temperature and salt stress conditions in Arabidopsis thaliana.

    PubMed

    Xu, Dan; Huang, Xuan; Xu, Zi-Qin; Schläppi, Michael

    2011-09-01

    The effect of the hybrid proline-rich protein (HyPRP) gene EARLI1 on the rate of germination (germinability) of Arabidopsis seeds and seedling growth under low temperature and salt stress conditions was investigated. EARLI1 was induced during germination in embryonic tissues, and was strongly expressed in certain parts of young seedlings. Comparisons of control, overexpressing (OX), and knockout (KO) lines indicated that higher than wild type levels of EARLI1 improved germinability, root elongation, and reduction of sodium accumulation in leaves under salt stress, as well as germinability under low-temperature stress. Abscisic acid (ABA) contents were relatively low after prolonged salt stress, suggesting that EARLI1 has an ABA-independent effect on germinability under these conditions. Overexpression of EARLI1 during germination enhanced the sensitivity of seeds to exogenously applied ABA, suggesting that EARLI1 has an ABA-dependent negative effect on seed germinability under high ABA stress conditions. Well-known stress response marker genes such as COR15a, KIN1, P5SC1, and RD29 were unaffected whereas P5SC2, RD22, or RAB18 were only slightly affected in OX and KO plants. The pleiotropic effects of EARLI1 during stress and an absence of strong regulatory effects on stress marker genes suggest that this HyPRP gene has an auxiliary role for various stress protection responses in Arabidopsis.

  3. Morphogenesis of the middle ear ossicles and spatial relationships with the external and inner ears during the embryonic period.

    PubMed

    Ozeki-Satoh, Maimi; Ishikawa, Aoi; Yamada, Shigehito; Uwabe, Chigako; Takakuwa, Tetsuya

    2016-10-01

    We describe the three-dimensional morphogenesis of the middle ear ossicles (MEOs) according to Carnegie stage (CS) in human embryos. Seventeen samples including 33 MEOs from CS18 to 23 were selected from the Kyoto Collection. The primordia of the MEOs and related structures were histologically observed and three-dimensionally reconstructed from digital images. The timing of chondrogenesis was variable among structures. The stapes was recognizable as a vague condensation of the mesenchymal cells in all samples from CS18, whereas the malleus and incus were recognizable at CS19. Chondrogenesis of all MEOs was evident in all samples after CS21. The chondrocranium was recognizable in all samples by CS18, and the perichondrium border of the auricular cartilage and otic capsule was distinct in all samples at CS23. At CS19, the MEOs were positioned in the anterior to posterior direction, following the order malleus, incus, stapes, which adjusted gradually during development. The MEOs connected in all samples after CS22. The stapes was located close to the vestibular part of the inner ear, although the basal part was not differentiated into the "footplate" form, even at CS23. The handles of the malleus were close to the tubotympanic recess at CS23, but were distant from the external auditory meatus. Determining the timeline of the formation of MEOs and connection of the external and inner ears can be informative for understanding hearing loss caused by failure of this connection. These data may provide a useful standard for morphogenesis, and will contribute to distinguishing between normal and abnormal MEO development. Anat Rec, 299:1325-1337, 2016. © 2016 Wiley Periodicals, Inc.

  4. A rabbit ear model for cold stress testing.

    PubMed

    Smith, T L; Gordon, S; Holden, M B; Smith, B P; Russell, G B; Koman, L A

    1994-01-01

    A rabbit ear model resembling the human digit was studied to determine the vascular response of the rabbit ear to a cold stress. Following moderate cooling (10 minutes at 5 degrees - 8 degrees C), auricular blood flow and cutaneous perfusion were reduced. This decrease was reversed by 30 minutes of warming. The response in the rabbit ear to cold stress is similar to that of normal human digits. The similarities between the control of the circulation in human digits and rabbit ears may result from the similarities in digital and auricular vascular receptors and receptor subtypes. Verification of the rabbit model provides an experimental method for obtaining important data regarding digital pathophysiology and the treatment of cold intolerance. Further study with this model will provide clinically relevant information regarding the pathophysiology of digital thermoregulatory abnormalities. PMID:7830538

  5. Meiotic abnormalities

    SciTech Connect

    1993-12-31

    Chapter 19, describes meiotic abnormalities. These include nondisjunction of autosomes and sex chromosomes, genetic and environmental causes of nondisjunction, misdivision of the centromere, chromosomally abnormal human sperm, male infertility, parental age, and origin of diploid gametes. 57 refs., 2 figs., 1 tab.

  6. The red ear syndrome

    PubMed Central

    2013-01-01

    Red Ear Syndrome (RES) is a very rare disorder, with approximately 100 published cases in the medical literature. Red ear (RE) episodes are characterised by unilateral or bilateral attacks of paroxysmal burning sensations and reddening of the external ear. The duration of these episodes ranges from a few seconds to several hours. The attacks occur with a frequency ranging from several a day to a few per year. Episodes can occur spontaneously or be triggered, most frequently by rubbing or touching the ear, heat or cold, chewing, brushing of the hair, neck movements or exertion. Early-onset idiopathic RES seems to be associated with migraine, whereas late-onset idiopathic forms have been reported in association with trigeminal autonomic cephalalgias (TACs). Secondary forms of RES occur with upper cervical spine disorders or temporo-mandibular joint dysfunction. RES is regarded refractory to medical treatments, although some migraine preventative treatments have shown moderate benefit mainly in patients with migraine-related attacks. The pathophysiology of RES is still unclear but several hypotheses involving peripheral or central nervous system mechanisms have been proposed. PMID:24093332

  7. From Ear to Brain

    ERIC Educational Resources Information Center

    Kimura, Doreen

    2011-01-01

    In this paper Doreen Kimura gives a personal history of the "right-ear effect" in dichotic listening. The focus is on the early ground-breaking papers, describing how she did the first dichotic listening studies relating the effects to brain asymmetry. The paper also gives a description of the visual half-field technique for lateralized stimulus…

  8. External Otitis (Swimmer's Ear)

    MedlinePlus

    ... drops, keeping water out of the ear, and pain relievers are the most common forms of treatment. External otitis may involve the entire canal, as ... does not allow fungus to grow as well. Treatment of boils depends on ... relievers, such as oxycodone with acetaminophen , can be given ...

  9. Altered structure of cortical sulci in gilles de la Tourette syndrome: Further support for abnormal brain development.

    PubMed

    Muellner, Julia; Delmaire, Christine; Valabrégue, Romain; Schüpbach, Michael; Mangin, Jean-François; Vidailhet, Marie; Lehéricy, Stéphane; Hartmann, Andreas; Worbe, Yulia

    2015-04-15

    Gilles de la Tourette syndrome is a neurodevelopmental disorder characterized by the presence of motor and vocal tics. We hypothesized that patients with this syndrome would present an aberrant pattern of cortical formation, which could potentially reflect global alterations of brain development. Using 3 Tesla structural neuroimaging, we compared sulcal depth, opening, and length and thickness of sulcal gray matter in 52 adult patients and 52 matched controls. Cortical sulci were automatically reconstructed and identified over the whole brain, using BrainVisa software. We focused on frontal, parietal, and temporal cortical regions, in which abnormal structure and functional activity were identified in previous neuroimaging studies. Partial correlation analysis with age, sex, and treatment as covariables of noninterest was performed amongst relevant clinical and neuroimaging variables in patients. Patients with Gilles de la Tourette syndrome showed lower depth and reduced thickness of gray matter in the pre- and post-central as well as superior, inferior, and internal frontal sulci. In patients with associated obsessive-compulsive disorder, additional structural changes were found in temporal, insular, and olfactory sulci. Crucially, severity of tics and of obsessive-compulsive disorder measured by Yale Global Tic severity scale and Yale-Brown Obsessive-Compulsive scale, respectively, correlated with structural sulcal changes in sensorimotor, temporal, dorsolateral prefrontal, and middle cingulate cortical areas. Patients with Gilles de la Tourette syndrome displayed an abnormal structural pattern of cortical sulci, which correlated with severity of clinical symptoms. Our results provide further evidence of abnormal brain development in GTS.

  10. Ear, Nose & Throat Issues & Down Syndrome

    MedlinePlus

    ... Throat Issues & Down Syndrome Ear, Nose & Throat Issues & Down Syndrome Ear, nose, and throat (ENT) problems are common ... What ENT Problems Are Common in Children With Down Syndrome? External Ear Canal Stenosis Stenotic ear canals (narrow ...

  11. The pleiotropic ABNORMAL FLOWER AND DWARF1 affects plant height, floral development and grain yield in rice

    PubMed Central

    Ren, Deyong; Rao, Yuchun; Wu, Liwen; Xu, Qiankun; Li, Zizhuang; Yu, Haiping; Zhang, Yu; Leng, Yujia; Hu, Jiang; Zhu, Li; Gao, Zhenyu; Dong, Guojun; Zhang, Guangheng; Guo, Longbiao

    2016-01-01

    Abstract Moderate plant height and successful establishment of reproductive organs play pivotal roles in rice grain production. The molecular mechanism that controls the two aspects remains unclear in rice. In the present study, we characterized a rice gene, ABNORMAL FLOWER AND DWARF1 (AFD1) that determined plant height, floral development and grain yield. The afd1 mutant showed variable defects including the dwarfism, long panicle, low seed setting and reduced grain yield. In addition, abnormal floral organs were also observed in the afd1 mutant including slender and thick hulls, and hull‐like lodicules. AFD1 encoded a DUF640 domain protein and was expressed in all tested tissues and organs. Subcellular localization showed AFD1‐green fluorescent fusion protein (GFP) was localized in the nucleus. Meantime, our results suggested that AFD1 regulated the expression of cell division and expansion related genes. PMID:26486996

  12. Development of a sintering methodology through abnormal glow discharge for manufacturing metal matrix composites

    NASA Astrophysics Data System (ADS)

    Pérez, S.; Pineda, Y.; Sarmiento, A.; López, A.

    2016-02-01

    In this study, a sintering methodology is presented by using abnormal glow discharge to metal matrix composites (MMC), consisting of 316 steel, reinforced with titanium carbide (TiC). The wear behaviour of these compounds was evaluated according to the standard ASTM G 99 in a tribometer pin-on-disk. The effect of the percentage of reinforcement (3, 6, and 9%), with 40 minutes of mixing in the planetary mill is analysed, using compaction pressure of 700MPa and sintering temperature of 1,100°C±5°C, gaseous atmosphere of H2 - N2, and sintering time of 30 minutes. As a result of the research, it shows that the best behaviour against wear is obtained when the MMC contains 6% TiC. Under this parameter the lowest percentage of pores and the lowest coefficient of friction are achieved, ensuring that the incorporation of ceramic particles (TiC) in 316 austenitic steel matrix significantly improves the wear resistance. Also, it is shown that it is possible to sinter such materials using the abnormal glow discharge, being a novel and effective method in which the working temperature is reached in a short time.

  13. [Blast injuries of the ear].

    PubMed

    Haralampiev, K; Ristić, B

    1991-01-01

    Blast injury of the ear is the actual military medical problem. The ear, due to its anatomo-physiologic characteristics, is more sensitive to effects of blast waves than other organs and systems. The anatomic and functional ear damages, their symptoms, etiology and clinical course are described. The diagnosis and treatment have been pointed out. PMID:1807053

  14. Influence of middle ear mucosal condition on post-tympanoplasty audiologic outcome.

    PubMed

    Song, Chan Il; Hong, Hye Ran; Yoon, Tae Hyun

    2016-03-01

    In this study, the association between the middle ear mucosal condition and post-tympanoplasty audiologic outcome was investigated in patients with chronic otitis media without cholesteatoma. One hundred and forty-three patients with chronic otitis media were collected in the Department of Otorhinolaryngology-Head and Neck Surgery at Asan Medical Center between January, 2009 and December, 2011. In the course of tympanoplasty, the status of the middle ear mucosa was divided into normal or abnormal by one surgeon. Pure tone audiometry was performed preoperatively and postoperatively, and post-tympanoplasty tympanogram was also conducted to estimate the condition of middle ear cavity. Of the 143 patients, there were 73 patients with normal middle ear mucosa and 70 patients with abnormal middle ear mucosa around Eustachian tube opening. The mean ABG of subjects with normal middle ear mucosa was 20.1 dB preoperatively, and 9.7 dB postoperatively (p < 0.001). Preoperative mean ABG was 22.4 dB and postoperative mean ABG was 16.4 dB in abnormal middle ear mucosa group (p = 0.137). Postoperative ABGs for 500 and 1000 Hz (7.1, 7.7 dB) in normal middle ear mucosa patients were significantly lower than those (17.2, 19.4 dB) in abnormal middle ear mucosa patients (p < 0.001). There was statistically significant correlation between middle ear mucosa status and post-tympanoplasty audiologic outcomes. The better condition of middle ear ventilation, the better postoperative hearing thresholds revealed after tympanoplasty.

  15. Two Ears Are Not Always Better than One: Mandatory Vowel Fusion Across Spectrally Mismatched Ears in Hearing-Impaired Listeners.

    PubMed

    Reiss, Lina A J; Eggleston, Jessica L; Walker, Emily P; Oh, Yonghee

    2016-08-01

    Hearing loss and auditory prostheses can alter auditory processing by inducing large pitch mismatches and broad pitch fusion between the two ears. Similar to integration of incongruent inputs in other sensory modalities, the mismatched, fused pitches are often averaged across ears for simple stimuli. Here, we measured parallel effects on complex stimulus integration using a new technique based on vowel classification in five bilateral hearing aid users and eight bimodal cochlear implant users. Continua between five pairs of synthetic vowels were created by varying the first formant spectral peak while keeping the second formant constant. Comparison of binaural and monaural vowel classification functions for each vowel pair continuum enabled visualization of the following frequency-dependent integration trends: (1) similar monaural and binaural functions, (2) ear dominance, (3) binaural averaging, and (4) binaural interference. Hearing aid users showed all trends, while bimodal cochlear implant users showed mostly ear dominance or interference. Interaural pitch mismatches, frequency ranges of binaural pitch fusion, and the relative weightings of pitch averaging across ears were also measured using tone and/or electrode stimulation. The presence of both large interaural pitch mismatches and broad pitch fusion was not sufficient to predict vowel integration trends such as binaural averaging or interference. The way that pitch averaging was weighted between ears also appears to be important for determining binaural vowel integration trends. Abnormally broad spectral fusion and the associated phoneme fusion across mismatched ears may underlie binaural speech perception interference observed in hearing aid and cochlear implant users. PMID:27220769

  16. Modeling abnormal early development with induced pluripotent stem cells from aneuploid syndromes.

    PubMed

    Li, Wen; Wang, Xianming; Fan, Wenxia; Zhao, Ping; Chan, Yau-Chi; Chen, Shen; Zhang, Shiqiang; Guo, Xiangpeng; Zhang, Ya; Li, Yanhua; Cai, Jinglei; Qin, Dajiang; Li, Xingyan; Yang, Jiayin; Peng, Tianran; Zychlinski, Daniela; Hoffmann, Dirk; Zhang, Ruosi; Deng, Kang; Ng, Kwong-Man; Menten, Bjorn; Zhong, Mei; Wu, Jiayan; Li, Zhiyuan; Chen, Yonglong; Schambach, Axel; Tse, Hung-Fat; Pei, Duanqing; Esteban, Miguel A

    2012-01-01

    Many human diseases share a developmental origin that manifests during childhood or maturity. Aneuploid syndromes are caused by supernumerary or reduced number of chromosomes and represent an extreme example of developmental disease, as they have devastating consequences before and after birth. Investigating how alterations in gene dosage drive these conditions is relevant because it might help treat some clinical aspects. It may also provide explanations as to how quantitative differences in gene expression determine phenotypic diversity and disease susceptibility among natural populations. Here, we aimed to produce induced pluripotent stem cell (iPSC) lines that can be used to improve our understanding of aneuploid syndromes. We have generated iPSCs from monosomy X [Turner syndrome (TS)], trisomy 8 (Warkany syndrome 2), trisomy 13 (Patau syndrome) and partial trisomy 11;22 (Emanuel syndrome), using either skin fibroblasts from affected individuals or amniocytes from antenatal diagnostic tests. These cell lines stably maintain the karyotype of the donors and behave like embryonic stem cells in all tested assays. TS iPSCs were used for further studies including global gene expression analysis and tissue-specific directed differentiation. Multiple clones displayed lower levels of the pseudoautosomal genes ASMTL and PPP2R3B than the controls. Moreover, they could be transformed into neural-like, hepatocyte-like and heart-like cells, but displayed insufficient up-regulation of the pseudoautosomal placental gene CSF2RA during embryoid body formation. These data support that abnormal organogenesis and early lethality in TS are not caused by a tissue-specific differentiation blockade, but rather involves other abnormalities including impaired placentation.

  17. A genome-wide linkage and association study of musical aptitude identifies loci containing genes related to inner ear development and neurocognitive functions.

    PubMed

    Oikkonen, J; Huang, Y; Onkamo, P; Ukkola-Vuoti, L; Raijas, P; Karma, K; Vieland, V J; Järvelä, I

    2015-02-01

    Humans have developed the perception, production and processing of sounds into the art of music. A genetic contribution to these skills of musical aptitude has long been suggested. We performed a genome-wide scan in 76 pedigrees (767 individuals) characterized for the ability to discriminate pitch (SP), duration (ST) and sound patterns (KMT), which are primary capacities for music perception. Using the Bayesian linkage and association approach implemented in program package KELVIN, especially designed for complex pedigrees, several single nucleotide polymorphisms (SNPs) near genes affecting the functions of the auditory pathway and neurocognitive processes were identified. The strongest association was found at 3q21.3 (rs9854612) with combined SP, ST and KMT test scores (COMB). This region is located a few dozen kilobases upstream of the GATA binding protein 2 (GATA2) gene. GATA2 regulates the development of cochlear hair cells and the inferior colliculus (IC), which are important in tonotopic mapping. The highest probability of linkage was obtained for phenotype SP at 4p14, located next to the region harboring the protocadherin 7 gene, PCDH7. Two SNPs rs13146789 and rs13109270 of PCDH7 showed strong association. PCDH7 has been suggested to play a role in cochlear and amygdaloid complexes. Functional class analysis showed that inner ear and schizophrenia-related genes were enriched inside the linked regions. This study is the first to show the importance of auditory pathway genes in musical aptitude.

  18. A genome-wide linkage and association study of musical aptitude identifies loci containing genes related to inner ear development and neurocognitive functions.

    PubMed

    Oikkonen, J; Huang, Y; Onkamo, P; Ukkola-Vuoti, L; Raijas, P; Karma, K; Vieland, V J; Järvelä, I

    2015-02-01

    Humans have developed the perception, production and processing of sounds into the art of music. A genetic contribution to these skills of musical aptitude has long been suggested. We performed a genome-wide scan in 76 pedigrees (767 individuals) characterized for the ability to discriminate pitch (SP), duration (ST) and sound patterns (KMT), which are primary capacities for music perception. Using the Bayesian linkage and association approach implemented in program package KELVIN, especially designed for complex pedigrees, several single nucleotide polymorphisms (SNPs) near genes affecting the functions of the auditory pathway and neurocognitive processes were identified. The strongest association was found at 3q21.3 (rs9854612) with combined SP, ST and KMT test scores (COMB). This region is located a few dozen kilobases upstream of the GATA binding protein 2 (GATA2) gene. GATA2 regulates the development of cochlear hair cells and the inferior colliculus (IC), which are important in tonotopic mapping. The highest probability of linkage was obtained for phenotype SP at 4p14, located next to the region harboring the protocadherin 7 gene, PCDH7. Two SNPs rs13146789 and rs13109270 of PCDH7 showed strong association. PCDH7 has been suggested to play a role in cochlear and amygdaloid complexes. Functional class analysis showed that inner ear and schizophrenia-related genes were enriched inside the linked regions. This study is the first to show the importance of auditory pathway genes in musical aptitude. PMID:24614497

  19. A Rare Stapes Abnormality

    PubMed Central

    Kanona, Hala; Virk, Jagdeep Singh; Kumar, Gaurav; Chawda, Sanjiv; Khalil, Sherif

    2015-01-01

    The aim of this study is to increase awareness of rare presentations, diagnostic difficulties alongside management of conductive hearing loss and ossicular abnormalities. We report the case of a 13-year-old female reporting progressive left-sided hearing loss and high resolution computed tomography was initially reported as normal. Exploratory tympanotomy revealed an absent stapedius tendon and lack of connection between the stapes superstructure and footplate. The footplate was fixed. Stapedotomy and stapes prosthesis insertion resulted in closure of the air-bone gap by 50 dB. A review of world literature was performed using MedLine. Middle ear ossicular discontinuity can result in significant conductive hearing loss. This can be managed effectively with surgery to help restore hearing. However, some patients may not be suitable or decline surgical intervention and can be managed safely conservatively. PMID:25628909

  20. Skeletal development and abnormalities of the vertebral column and of the fins in hatchery-reared turbot Scophthalmus maximus.

    PubMed

    Tong, X H; Liu, Q H; Xu, S H; Ma, D Y; Xiao, Z Z; Xiao, Y S; Li, J

    2012-03-01

    To describe the skeletal development and abnormalities in turbot Scophthalmus maximus, samples were collected every day from hatching to 60 days after hatching (DAH). A whole-mount cartilage and bone-staining technique was used. Vertebral ontogeny started with the formation of anterior haemal arches at 5·1 mm standard length (L(S) ) c. 11 DAH, and was completed by the full attainment of parapophyses at 16·9 mm L(S) c. 31 DAH. Vertebral centra started to develop at 6·3 mm L(S) c. 16 DAH and ossification in all centra was visible at 11·0 mm L(S) c. 25 DAH. The caudal fin appeared at 5·1 mm L(S) c. 11 DAH and ossification was visible at 20·6 mm L(S) c. 37 DAH. The onset of dorsal and anal fin elements appeared at 5·8 mm L(S) c. 15 DAH and 6·3 mm L(S) c. 16 DAH, respectively. Ossifications of both dorsal fin and anal fin were visible at 20·6 mm L(S) c. 37 DAH. The pectorals were the only fins present before first feeding, their ossifications were completed at 23·5 mm L(S) c. 48 DAH. Pelvic fins began forming at 7·2 mm L(S) c. 19 DAH and calcification of the whole structure was visible at 19·8 mm L(S) c. 36 DAH. In the present study, 24 types of skeletal abnormalities were observed. About 51% of individuals presented skeletal abnormalities, and the highest occurrence was found in the haemal region of the vertebral column. As for each developmental stage, the most common abnormalities were in the dorsal fin during early metamorphic period (stage 2), vertebral fusion during climax metamorphosis (stage 3) and caudal fin abnormality during both late-metamorphic period (stage 4) and post-metamorphic period (stage 5). Such research will be useful for early detection of skeletal malformations during different growth periods of reared S. maximus.

  1. Craniofacial Abnormalities

    MedlinePlus

    ... of the skull and face. Craniofacial abnormalities are birth defects of the face or head. Some, like cleft ... palate, are among the most common of all birth defects. Others are very rare. Most of them affect ...

  2. Walking abnormalities

    MedlinePlus

    ... include: Arthritis of the leg or foot joints Conversion disorder (a psychological disorder) Foot problems (such as a ... injuries. For an abnormal gait that occurs with conversion disorder, counseling and support from family members are strongly ...

  3. Nail abnormalities

    MedlinePlus

    Beau's lines; Fingernail abnormalities; Spoon nails; Onycholysis; Leukonychia; Koilonychia; Brittle nails ... Just like the skin, the fingernails tell a lot about your health: ... the fingernail. These lines can occur after illness, injury to ...

  4. Drug delivery to the ear.

    PubMed

    Hoskison, E; Daniel, M; Al-Zahid, S; Shakesheff, K M; Bayston, R; Birchall, J P

    2013-01-01

    Drug delivery to the ear is used to treat conditions of the middle and inner ear such as acute and chronic otitis media, Ménière's disease, sensorineural hearing loss and tinnitus. Drugs used include antibiotics, antifungals, steroids, local anesthetics and neuroprotective agents. A literature review was conducted searching Medline (1966-2012), Embase (1988-2012), the Cochrane Library and Ovid (1966-2012), using search terms 'drug delivery', 'middle ear', 'inner ear' and 'transtympanic'. There are numerous methods of drug delivery to the middle ear, which can be categorized as topical, systemic (intravenous), transtympanic and via the Eustachian tube. Localized treatments to the ear have the advantages of targeted drug delivery allowing higher therapeutic doses and minimizing systemic side effects. The ideal scenario would be a carrier system that could cross the intact tympanic membrane loaded with drugs or biochemical agents for the treatment of middle and inner ear conditions.

  5. Real-Time Coaching with Bug-in-Ear Technology: A Practical Approach to Support Families in Their Child's Development

    ERIC Educational Resources Information Center

    Ottley, Jennifer Riggie

    2015-01-01

    Variability in the quality of adult-child interactions among caregivers of young children can be problematic for children's development. However, professional development has been successful in improving caregivers' positive interactions with children. Blending principles of adult learning theory with technology-based coaching can provide a…

  6. Incorporating anthropometry into design of ear-related products.

    PubMed

    Liu, Bor-Shong

    2008-01-01

    To achieve mass customization and collaborative product design, human factors and ergonomics should play a key development role. The purpose of this study was to provide product designers with the anthropometic dimensions of outer ears for different demographic data, including gender and age. The second purpose was to compare the dimensions of various ear-related products (i.e., earphone, bluetooth earphone and ear-cup earphone) with the anthropometic database and recommend appropriate solutions for design. Two hundred subjects aged 20-59 was selected for this study and divided into four age stratifications. Further, three different dimensions of the outer ear (i.e., the earhole length, the ear connection length and the length of the pinna) were measured by superimposed grid photographic technique. The analysis of variance (ANOVA) was used to investigate the effects of gender, and age on ear dimensions. The results showed that all ear dimensions had significant gender effects. A comparison between the anthropometric dimensions and those of current products revealed that most current ear-related products need to be redesigned using anthropometric data. The shapes of earhole and pinna are not circular. Consequently, ear products need to be elongated so that users may feel more comfortably and not have the product slip off easily.

  7. The Maize PI/GLO Ortholog Zmm16/sterile tassel silky ear1 Interacts with the Zygomorphy and Sex Determination Pathways in Flower Development[OPEN

    PubMed Central

    Bartlett, Madelaine E.; Williams, Steven K.; Taylor, Zac; DeBlasio, Stacy; Hall, Darren H.; Schmidt, Robert J.; Jackson, David P.

    2015-01-01

    In monocots and eudicots, B class function specifies second and third whorl floral organ identity as described in the classic ABCE model. Grass B class APETALA3/DEFICIENS orthologs have been functionally characterized; here, we describe the positional cloning and characterization of a maize (Zea mays) PISTILLATA/GLOBOSA ortholog Zea mays mads16 (Zmm16)/sterile tassel silky ear1 (sts1). We show that, similar to many eudicots, all the maize B class proteins bind DNA as obligate heterodimers and positively regulate their own expression. However, sts1 mutants have novel phenotypes that provide insight into two derived aspects of maize flower development: carpel abortion and floral asymmetry. Specifically, we show that carpel abortion acts downstream of organ identity and requires the growth-promoting factor grassy tillers1 and that the maize B class genes are expressed asymmetrically, likely in response to zygomorphy of grass floral primordia. Further investigation reveals that floral phyllotactic patterning is also zygomorphic, suggesting significant mechanistic differences with the well-characterized models of floral polarity. These unexpected results show that despite extensive study of B class gene functions in diverse flowering plants, novel insights can be gained from careful investigation of homeotic mutants outside the core eudicot model species. PMID:26518212

  8. Passive and active middle ear implants

    PubMed Central

    Beutner, Dirk; Hüttenbrink, Karl-Bernd

    2011-01-01

    Besides eradication of chronic middle ear disease, the reconstruction of the sound conduction apparatus is a major goal of modern ear microsurgery. The material of choice in cases of partial ossicular replacement prosthesis is the autogenous ossicle. In the event of more extensive destruction of the ossicular chain diverse alloplastic materials, e.g. metals, ceramics, plastics or composits are used for total reconstruction. Their specialised role in conducting sound energy within a half-open implant bed sets high demands on the biocompatibility as well as the acoustic-mechanic properties of the prosthesis. Recently, sophisticated titanium middle ear implants allowing individual adaptation to anatomical variations are widely used for this procedure. However, despite modern developments, hearing restoration with passive implants often faces its limitations due to tubal-middle-ear dysfunction. Here, implantable hearing aids, successfully used in cases of sensorineural hearing loss, offer a promising alternative. This article reviews the actual state of affairs of passive and active middle ear implants. PMID:22073102

  9. Abnormal gene expression in cerebellum of Npc1-/- mice during postnatal development

    PubMed Central

    Liao, Guanghong; Wen, Zhining; Irizarry, Kristopher; Huang, Ying; Mitsouras, Katherine; Darmani, Mariam; Leon, Terry; Shi, Leming; Bi, Xiaoning

    2010-01-01

    Niemann-Pick Type C disease is an autosomal recessive neurodegenerative disorder with abnormal lipid storage as the major cellular pathologic hallmark. Genetic analyses have identified mutations in NPC1 gene in the great majority of cases, while mutations in NPC2 account for the remainders. Yet, little is known regarding the cellular mechanisms responsible for NPC pathogenesis, especially for neurodegeneration, which is the usual cause of death. To identify critical steps that could account for the pathological manifestations of the disease in one of the most affected brain structures, we performed global gene expression analysis in the cerebellum from three-week old Npc1+/+ and Npc1-/- mice with two different microarray platforms (Agilent and Illumina). Differentially-expressed genes identified by both microarray platforms were then subjected to KEGG pathway analysis. Expression of genes in six pathways was significantly altered in Npc1-/- mice; functionally, these signaling pathways belong to the following three categories: 1) steroid and terpenoid biosynthesis, 2) immune response, and 3) cell adhesion/motility. In addition, the expression of several proteins involved in lipid transport was significantly altered in Npc1-/- mice. Our results provide novel molecular insight regarding the mechanisms of pathogenesis in NPC disease and reveal potential new therapeutic targets. PMID:20153740

  10. Microbial community structure and function during abnormal curve development of substrate-induced respiration measurements.

    PubMed

    Bartling, Johanna; Kotzerke, Anja; Mai, Maike; Esperschütz, Jürgen; Buegger, Franz; Schloter, Michael; Wilke, Berndt-Michael

    2009-12-01

    Soil respiration measurements are an established method to test the abundance, activity and vitality of the soil microorganisms. However, abnormal progressions of soil respiration curves impede a clear interpretation of the data. The aim of this study was to investigate the changes in the microbial structure during the formation of phenomena like double peaks and terraces by analysis of the PLFA composition (phospholipid fatty acid composition). Moreover, 13C labeled glucose was used as substrate; therefore it was possible to measure delta13C values both within the PLFA fraction as well as within the carbon dioxide evolved during respiration. As contaminants trinitrotoluene, cycloheximide, and hexadecane were used. The results showed that the appearance of double peaks was mainly related to the growth of fungi with the marker 18:2delta9,12 due to a toxic effect of trinitrotoluene and cycloheximide. In contrast, the phenomenon of terrace formation was related to the utilization of hexadecane as a carbon source mainly by bacteria.

  11. The Philadelphia Neurodevelopmental Cohort: A publicly available resource for the study of normal and abnormal brain development in youth.

    PubMed

    Satterthwaite, Theodore D; Connolly, John J; Ruparel, Kosha; Calkins, Monica E; Jackson, Chad; Elliott, Mark A; Roalf, David R; Ryan Hopsona, Karthik Prabhakaran; Behr, Meckenzie; Qiu, Haijun; Mentch, Frank D; Chiavacci, Rosetta; Sleiman, Patrick M A; Gur, Ruben C; Hakonarson, Hakon; Gur, Raquel E

    2016-01-01

    The Philadelphia Neurodevelopmental Cohort (PNC) is a large-scale study of child development that combines neuroimaging, diverse clinical and cognitive phenotypes, and genomics. Data from this rich resource is now publicly available through the Database of Genotypes and Phenotypes (dbGaP). Here we focus on the data from the PNC that is available through dbGaP and describe how users can access this data, which is evolving to be a significant resource for the broader neuroscience community for studies of normal and abnormal neurodevelopment.

  12. Abnormal P-selectin localization during megakaryocyte development determines thrombosis in the gata1low model of myelofibrosis.

    PubMed

    Zetterberg, Eva; Verrucci, Maria; Martelli, Fabrizio; Zingariello, Maria; Sancillo, Laura; D'Amore, Emanuela; Rana, Rosa Alba; Migliaccio, Anna Rita

    2014-01-01

    Patients with primary myelofibrosis have increased risk for bleeding and thrombosis. It is debated whether propensity to thrombosis is due to increased numbers of platelet microparticles and/or to pathological platelet-neutrophil interactions. Platelet neutrophil interactions are mediated by P-selectin and even though the megakaryocytes of myelofibrosis patients express normal levels of P-selectin, it remains abnormally localized to the demarcation membrane system rather than being assembled into the α-granules in platelets. Mice carrying the hypomorphic Gata1(low) mutation express the same megakaryocyte abnormalities presented by primary myelofibrosis patients, including abnormal P-selectin localization to the DMS and develop with age myelofibrosis, a disease that closely resembles human primary myelofibrosis. Whether these mice would also develop thrombosis has not been investigated as yet. The aim of this study was to determine whether Gata1(low) mice would develop thrombosis with age and, in this case, the role played by P-selectin in the development of the trait. To this aim, Gata1(low) mice were crossed with P-sel(null) mice according to standard genetic protocols and Gata1(low)P-sel(wt), Gata1(low)P-sel(null) and Gata1(WT)P-sel(null) or Gata1(wt)P-sel(wt) (as controls) littermates obtained. It was shown that platelet counts, but not hematocrit, are reduced in Gata1(low) mice. Moreover, platelet microparticles are reduced in Gata1(low) mice and P-selectin positive platelet microparticles were not found. To determine the phenotypic implications of the different mutations, bleeding time was estimated by a tail cut procedure. Mutant mice were sacrificed and presence of thrombosis was determined by immunohistological staining of organs. Gata1(low) mice with or without the P-selectin null trait had a prolonged bleeding time compared to wild type mice. However, in Gata1(low) mice significantly higher frequency of thrombotic events was seen in adult and old Gata1

  13. Semicircular canal morphogenesis in the zebrafish inner ear requires the function of gpr126 (lauscher), an adhesion class G protein-coupled receptor gene

    PubMed Central

    Geng, Fan-Suo; Abbas, Leila; Baxendale, Sarah; Holdsworth, Celia J.; Swanson, A. George; Slanchev, Krasimir; Hammerschmidt, Matthias; Topczewski, Jacek; Whitfield, Tanya T.

    2013-01-01

    Morphogenesis of the semicircular canal ducts in the vertebrate inner ear is a dramatic example of epithelial remodelling in the embryo, and failure of normal canal development results in vestibular dysfunction. In zebrafish and Xenopus, semicircular canal ducts develop when projections of epithelium, driven by extracellular matrix production, push into the otic vesicle and fuse to form pillars. We show that in the zebrafish, extracellular matrix gene expression is high during projection outgrowth and then rapidly downregulated after fusion. Enzymatic disruption of hyaluronan in the projections leads to their collapse and a failure to form pillars: as a result, the ears swell. We have cloned a zebrafish mutant, lauscher (lau), identified by its swollen ear phenotype. The primary defect in the ear is abnormal projection outgrowth and a failure of fusion to form the semicircular canal pillars. Otic expression of extracellular matrix components is highly disrupted: several genes fail to become downregulated and remain expressed at abnormally high levels into late larval stages. The lau mutations disrupt gpr126, an adhesion class G protein-coupled receptor gene. Expression of gpr126 is similar to that of sox10, an ear and neural crest marker, and is partially dependent on sox10 activity. Fusion of canal projections and downregulation of otic versican expression in a hypomorphic lau allele can be restored by cAMP agonists. We propose that Gpr126 acts through a cAMP-mediated pathway to control the outgrowth and adhesion of canal projections in the zebrafish ear via the regulation of extracellular matrix gene expression. PMID:24067352

  14. Endoscopic assisted cochlear implants in ear malformations.

    PubMed

    Marchioni, Daniele; Soloperto, Davide; Guarnaccia, Maria C; Genovese, Elisabetta; Alicandri-Ciufelli, Matteo; Presutti, Livio

    2015-10-01

    The aim of present study is to describe the use of the endoscopic assisted cochlear implant approach in cases with severely malformed temporal bones and with anomalous anatomy of the inner ear and tympanic cavity. Eight patients with malformed middle and inner ear and bilateral profound hearing loss were operated using an endoscopic assisted cochlear implant procedure at our tertiary university referral center between January and September 2013. Five patients received a cochlear implant using a suprameatal endoscopic assisted approach. A chart review of clinical data and videos from the operations was performed. All procedures were re-analyzed and codified. In all patients, discharge from hospital was on the third day post-surgery. No immediate or late postoperative complications were noted. The current mean follow-up is 6 months, with range between 4 and 12 months. This approach proved to be successful in cochlear implant placement. It guaranteed a very good control on the facial nerve, even in cases with difficult anatomical conditions, mainly thanks to the endoscopic procedure. It also permitted an appropriate anatomical orientation of the abnormal middle ear with a direct safe cochleostomy, when the round window position would have been difficult to treat using a traditional approach. PMID:25085636

  15. Endoscopic anatomy of the pediatric middle ear.

    PubMed

    Isaacson, Glenn

    2014-01-01

    Traditionally, otologists have aimed to produce a clean, dry, safe ear with the best possible hearing result. More recently, "less invasively" has been added to this list of goals. The development of small-diameter, high-quality rigid endoscopes and high-definition video systems has made totally endoscopic, transcanal surgery a reality in adult otology and a possibility in pediatric otology. This article reviews the anatomy of the pediatric middle ear and its surrounding airspaces and structures based on the work of dozens of researchers over the past 50 years. It will focus on the developmental changes in ear anatomy from birth through the first decade, when structure and function change most rapidly. Understanding the limits and possibilities afforded by new endoscopic technologies, the pediatric otologist can strive for results matching or exceeding those achieved by more invasive surgical approaches.

  16. DLX4 is associated with orofacial clefting and abnormal jaw development

    PubMed Central

    Wu, Di; Mandal, Shyamali; Choi, Alex; Anderson, August; Prochazkova, Michaela; Perry, Hazel; Gil-Da-Silva-Lopes, Vera L.; Lao, Richard; Wan, Eunice; Tang, Paul Ling-Fung; Kwok, Pui-yan; Klein, Ophir; Zhuan, Bian; Slavotinek, Anne M.

    2015-01-01

    Cleft lip and/or palate (CL/P) are common structural birth defects in humans. We used exome sequencing to study a patient with bilateral CL/P and identified a single nucleotide deletion in the patient and her similarly affected son—c.546_546delG, predicting p.Gln183Argfs*57 in the Distal-less 4 (DLX4) gene. The sequence variant was absent from databases, predicted to be deleterious and was verified by Sanger sequencing. In mammals, there are three Dlx homeobox clusters with closely located gene pairs (Dlx1/Dlx2, Dlx3/Dlx4, Dlx5/Dlx6). In situ hybridization showed that Dlx4 was expressed in the mesenchyme of the murine palatal shelves at E12.5, prior to palate closure. Wild-type human DLX4, but not mutant DLX4_c.546delG, could activate two murine Dlx conserved regulatory elements, implying that the mutation caused haploinsufficiency. We showed that reduced DLX4 expression after short interfering RNA treatment in a human cell line resulted in significant up-regulation of DLX3, DLX5 and DLX6, with reduced expression of DLX2 and significant up-regulation of BMP4, although the increased BMP4 expression was demonstrated only in HeLa cells. We used antisense morpholino oligonucleotides to target the orthologous Danio rerio gene, dlx4b, and found reduced cranial size and abnormal cartilaginous elements. We sequenced DLX4 in 155 patients with non-syndromic CL/P and CP, but observed no sequence variants. From the published literature, Dlx1/Dlx2 double homozygous null mice and Dlx5 homozygous null mice both have clefts of the secondary palate. This first finding of a DLX4 mutation in a family with CL/P establishes DLX4 as a potential cause of human clefts. PMID:25954033

  17. Value of middle ear inflation as a diagnostic indicator of eustachian tube patency.

    PubMed

    Luntz, M; Sadé, J

    1990-02-01

    The value of tubal inflation as a diagnostic procedure for Eustachian tube patency and function is controversial. In an attempt to assess the diagnostic value of air douche in atelectatic ears, 49 such ears of 40 patients were politzerized. The procedure was successful in 45 ears. However, of the four unsuccessful cases, two of the patients were able to autoinflate their ears. These results show that air douches pass regularly through the Eustachian tube into the tympanic cavity even in atelectatic ears, which by definition suffer from aeration deficiency, which is often considered to be secondary to 'Eustachian tube obstruction', or alternatively 'Eustachian tube dysfunction'. Thus, the ability to force air through the Eustachian tube by politzerization is of no diagnostic value as an indicator of normal or abnormal tubal patency or functioning in atelectatic ears and most probably in allied conditions. PMID:2324620

  18. [The tempestuous history of middle ear operation].

    PubMed

    Betlejewski, Stanisław; Betlejewski, Andrzej

    2008-01-01

    The paper is a review of primary and secondary historical and scientific literature concerning the surgical treatment of the middle ear diseases. The development of mastoid surgery can be traced through the past 4 centuries. Once used as a means of evacuating a postauricular abscess, it has evolved to become a method for gaining entry into the middle ear to control acute and chronic ear diseases, or for treatment of otogenic complications. Earlier works led the way to the postauricular "Wilde incision", which gave rise to Schwartze mastoidectomy. Oscar Wilde's ultimate demise from an otogenic meningitis appears all the more ironic when one considers the role his father, Sir William Wilde, played as one of the founding fathers of modern otology. The death of baron von Berger after mastoidectomy performed for treatment of tinnitus and hypacusis, stopped the further development of surgical procedures for about hundred years. The Joseph Toynbee's "Diseases of the ear" was the first work about ear diseases on a pathologic anatomical base, and fundamental for otology of the German speaking countries in the nineteenth and early twentieth centuries. Otology was emerging as a specific specialty. Von Tröltsch was the first surgeon, who proposed the antral opening through the external ear canal. When Schwartze and his assistant, Eysell, published their paper: "On the Artificial Opening of the Mastoid Air Cells," a century or so had passed since the few previous attempts to remove the tegmen of the mastoid had been reported. One of the greatest otologists of the 19th century was Adam Politzer, His influence on the 50 years of otology has never been equaled. It is in his honor that the International Society of Otology bears his name.

  19. A panel of free fatty acid ratios to predict the development of metabolic abnormalities in healthy obese individuals

    PubMed Central

    Zhao, Linjing; Ni, Yan; Ma, Xiaojing; Zhao, Aihua; Bao, Yuqian; Liu, Jiajian; Chen, Tianlu; Xie, Guoxiang; Panee, Jun; Su, Mingming; Yu, Herbert; Wang, Congrong; Hu, Cheng; Jia, Weiping; Jia, Wei

    2016-01-01

    Increasing evidences support that metabolically healthy obese (MHO) is a transient state. However, little is known about the early markers associated with the development of metabolic abnormalities in MHO individuals. Serum free fatty acids (FFAs) profile is highlighted in its association with obesity-related insulin resistance, type 2 diabetes mellitus (T2DM) and cardiovascular diseases (CVD). To examine the association of endogenous fatty acid metabolism with future development of metabolic abnormalities in MHO individuals, we retrospectively analyzed 24 [product FFA]/[precursor FFA] ratios in fasting sera and clinical data from 481 individuals who participated in three independent studies, including 131 metabolic healthy subjects who completed the 10-year longitudinal Shanghai Diabetes Study (SHDS), 312 subjects cross-sectionally sampled from the Shanghai Obesity Study (SHOS), and 38 subjects who completed an 8-week very low carbohydrate diet (VLCD) intervention study. Results showed that higher baseline level of oleic acid/stearic acid (OA/SA), and lower levels of stearic acid/palmitic acid (SA/PA) and arachidonic acid/dihomo-γ-linolenic acid (AA/DGLA) ratios were associated with higher rate of MHO to MUO conversion in the longitudinal SHDS. Further, the finding was validated in the cross-sectional and interventional studies. This panel of FFA ratios could be used for identification and early intervention of at-risk obese individuals. PMID:27344992

  20. A panel of free fatty acid ratios to predict the development of metabolic abnormalities in healthy obese individuals.

    PubMed

    Zhao, Linjing; Ni, Yan; Ma, Xiaojing; Zhao, Aihua; Bao, Yuqian; Liu, Jiajian; Chen, Tianlu; Xie, Guoxiang; Panee, Jun; Su, Mingming; Yu, Herbert; Wang, Congrong; Hu, Cheng; Jia, Weiping; Jia, Wei

    2016-01-01

    Increasing evidences support that metabolically healthy obese (MHO) is a transient state. However, little is known about the early markers associated with the development of metabolic abnormalities in MHO individuals. Serum free fatty acids (FFAs) profile is highlighted in its association with obesity-related insulin resistance, type 2 diabetes mellitus (T2DM) and cardiovascular diseases (CVD). To examine the association of endogenous fatty acid metabolism with future development of metabolic abnormalities in MHO individuals, we retrospectively analyzed 24 [product FFA]/[precursor FFA] ratios in fasting sera and clinical data from 481 individuals who participated in three independent studies, including 131 metabolic healthy subjects who completed the 10-year longitudinal Shanghai Diabetes Study (SHDS), 312 subjects cross-sectionally sampled from the Shanghai Obesity Study (SHOS), and 38 subjects who completed an 8-week very low carbohydrate diet (VLCD) intervention study. Results showed that higher baseline level of oleic acid/stearic acid (OA/SA), and lower levels of stearic acid/palmitic acid (SA/PA) and arachidonic acid/dihomo-γ-linolenic acid (AA/DGLA) ratios were associated with higher rate of MHO to MUO conversion in the longitudinal SHDS. Further, the finding was validated in the cross-sectional and interventional studies. This panel of FFA ratios could be used for identification and early intervention of at-risk obese individuals. PMID:27344992

  1. Cerebellar cortex development in the weaver condition presents regional and age-dependent abnormalities without differences in Purkinje cells neurogenesis.

    PubMed

    Martí, Joaquín; Santa-Cruz, María C; Hervás, José P; Bayer, Shirley A; Villegas, Sandra

    2016-01-01

    Ataxias are neurological disorders associated with the degeneration of Purkinje cells (PCs). Homozygous weaver mice (wv/wv) have been proposed as a model for hereditary cerebellar ataxia because they present motor abnormalities and PC loss. To ascertain the physiopathology of the weaver condition, the development of the cerebellar cortex lobes was examined at postnatal day (P): P8, P20 and P90. Three approaches were used: 1) quantitative determination of several cerebellar features; 2) qualitative evaluation of the developmental changes occurring in the cortical lobes; and 3) autoradiographic analyses of PC generation and placement. Our results revealed a reduction in the size of the wv/wv cerebellum as a whole, confirming previous results. However, as distinguished from these reports, we observed that quantified parameters contribute differently to the abnormal growth of the wv/wv cerebellar lobes. Qualitative analysis showed anomalies in wv/wv cerebellar cytoarchitecture, depending on the age and lobe analyzed. Such abnormalities included the presence of the external granular layer after P20 and, at P90, ectopic cells located in the molecular layer following several placement patterns. Finally, we obtained autoradiographic evidence that wild-type and wv/wv PCs presented similar neurogenetic timetables, as reported. However, the innovative character of this current work lies in the fact that the neurogenetic gradients of wv/wv PCs were not modified from P8 to P90. A tendency for the accumulation of late-formed PCs in the anterior and posterior lobes was found, whereas early-generated PCs were concentrated in the central and inferior lobes. These data suggested that wv/wv PCs may migrate properly to their final destinations. The extrapolation of our results to patients affected with cerebellar ataxias suggests that all cerebellar cortex lobes are affected with several age-dependent alterations in cytoarchitectonics. We also propose that PC loss may be regionally

  2. Cerebellar cortex development in the weaver condition presents regional and age-dependent abnormalities without differences in Purkinje cells neurogenesis.

    PubMed

    Martí, Joaquín; Santa-Cruz, María C; Hervás, José P; Bayer, Shirley A; Villegas, Sandra

    2016-01-01

    Ataxias are neurological disorders associated with the degeneration of Purkinje cells (PCs). Homozygous weaver mice (wv/wv) have been proposed as a model for hereditary cerebellar ataxia because they present motor abnormalities and PC loss. To ascertain the physiopathology of the weaver condition, the development of the cerebellar cortex lobes was examined at postnatal day (P): P8, P20 and P90. Three approaches were used: 1) quantitative determination of several cerebellar features; 2) qualitative evaluation of the developmental changes occurring in the cortical lobes; and 3) autoradiographic analyses of PC generation and placement. Our results revealed a reduction in the size of the wv/wv cerebellum as a whole, confirming previous results. However, as distinguished from these reports, we observed that quantified parameters contribute differently to the abnormal growth of the wv/wv cerebellar lobes. Qualitative analysis showed anomalies in wv/wv cerebellar cytoarchitecture, depending on the age and lobe analyzed. Such abnormalities included the presence of the external granular layer after P20 and, at P90, ectopic cells located in the molecular layer following several placement patterns. Finally, we obtained autoradiographic evidence that wild-type and wv/wv PCs presented similar neurogenetic timetables, as reported. However, the innovative character of this current work lies in the fact that the neurogenetic gradients of wv/wv PCs were not modified from P8 to P90. A tendency for the accumulation of late-formed PCs in the anterior and posterior lobes was found, whereas early-generated PCs were concentrated in the central and inferior lobes. These data suggested that wv/wv PCs may migrate properly to their final destinations. The extrapolation of our results to patients affected with cerebellar ataxias suggests that all cerebellar cortex lobes are affected with several age-dependent alterations in cytoarchitectonics. We also propose that PC loss may be regionally

  3. Zebrafish Trap230/Med12 is required as a coactivator for Sox9-dependent neural crest, cartilage and ear development.

    PubMed

    Rau, Marlene J; Fischer, Sabine; Neumann, Carl J

    2006-08-01

    The vertebrate Sox9 transcription factor directs the development of neural crest, otic placodes, cartilage and bone. In zebrafish, there are two Sox9 orthologs, Sox9a and Sox9b, which together perform the functions of the single-copy tetrapod Sox9. In a large-scale genetic screen, we have identified a novel zebrafish mutant that strongly resembles the Sox9a/Sox9b double mutant phenotype. We show that this mutation disrupts the zebrafish Trap230/Med12 ortholog, a member of the Mediator complex. Mediator is a coactivator complex transducing the interaction of DNA-binding transcription factors with RNA polymerase II, and our results reveal a critical function of the Trap230 subunit as a coactivator for Sox9.

  4. Role of AQP1 in inner ear in motion sickness.

    PubMed

    Huang, Yi-Deng; Xia, Si-Wen; Dai, Pu; Han, Dong-Yi

    2011-10-24

    Inner ear is critical for the development of motion sickness (MS). The present work was designed to test the role of aquaporins (AQPs) in inner ear in MS. After repetitive stimulus of rotation, the MS symptom was steadily alleviated in mice. After repetitive stimulus of rotation, several AQPs mRNA levels including AQP1, AQP2, AQP3, AQP4, AQP6, AQP7, and AQP9 in the inner ears of mice were analyzed. It was found that AQP1 mRNA level was increased remarkably, which was reconfirmed by Western blotting analysis. In addition, the relationship between AQP1 expression and MS sensitivity was studied and it was shown that AQP1 mRNA level was negatively related to MS index in mice. We sought to examine the function of AQP1 in inner ear using an RNAi approach to reduce the AQP1 protein expression in vivo. It was first observed that AQP1 knockdown in inner ear resulted in a significant increase of MS sensitivity in mice. In conclusion, after repetitive stimulus of rotation, the alleviation of MS symptom in mice was, at least in part, due to the upregulation of AQP1 expression in inner ear. In addition, the sensitivity to MS in mice was, at least in part, dependent on the expression of AQP1 in inner ear. AQP1 in inner ear plays an important role in the development of MS, and might be a potential target for the prevention or management of MS.

  5. [Complications Resulting from Taking Ear Impressions].

    PubMed

    Sugiuchi, Tomoko; Kodera, Kazuoki; Zusho, Hiroyuki; Asano, Yoshikazu; Kanesada, Keiko; Hayashida, Mitsuhiro; Kanaya, Koichiro; Tokumaru, Takeshi

    2015-08-01

    In 2012, we carried out a study in a large sample to understand the secondary injuries caused during the taking ear impressions for hearing aids. This study is a follow-up of previous research conducted in 1986 (285 medical institutions) and 1999 (98 medical institutions). We posted a questionnaire survey to the otolaryngology departments of 3,257 medical institutions. The response rate to the questionnaire was 62.9% (2,050 of the 3,257 institutions), and the results indicated that 301 of the 2050 institutions (14.7%) had experience with secondary injuries, with a total of 460 cases reported. In 342 of the 460 cases (74.3%), the secondary injuries occurred at hearing-aid dealerships, followed by 67 cases (14.6%) at affiliated medical institutions, and 51 cases (11.1%) in other locations, including other medical institutions, rehabilitation counseling centers, and educational institutions. The most common type of secondary injury (298 cases, 64.8%) was caused by the presence of foreign bodies in the ear, which in turn was a result of complications occurring during the removal of residual ear impression material. Of these 298 cases, 32 required excision of the foreign bodies and surgical intervention under general anesthesia. The remaining 10 cases exhibited isolated tympanic membrane perforation without foreign body-related complications. Furthermore, 146 cases (31.7%) developed bleeding and otitis externa following removal of the ear impression, and there were reports of cases with bleeding that required long-term outpatient care and treatment. Therefore, since retention of a foreign body in the ear and tympanic membrane perforation can occur even in patients without a history of surgery or prior otologic history, adjustment of hearing aids requires prior otorhinolaryngological examination. Furthermore, because of the risk of secondary injury when taking ear impressions, this procedure must be performed with caution under the guidance of an otolaryngologist.

  6. Abnormal pressures as hydrodynamic phenomena

    USGS Publications Warehouse

    Neuzil, C.E.

    1995-01-01

    So-called abnormal pressures, subsurface fluid pressures significantly higher or lower than hydrostatic, have excited speculation about their origin since subsurface exploration first encountered them. Two distinct conceptual models for abnormal pressures have gained currency among earth scientists. The static model sees abnormal pressures generally as relict features preserved by a virtual absence of fluid flow over geologic time. The hydrodynamic model instead envisions abnormal pressures as phenomena in which flow usually plays an important role. This paper develops the theoretical framework for abnormal pressures as hydrodynamic phenomena, shows that it explains the manifold occurrences of abnormal pressures, and examines the implications of this approach. -from Author

  7. The ear: Diagnostic imaging

    SciTech Connect

    Vignaud, J.; Jardin, C.; Rosen, L.

    1986-01-01

    This is an English translation of volume 17-1 of Traite de radiodiagnostic and represents a reasonably complete documentation of the diseases of the temporal bone that have imaging manifestations. The book begins with chapters on embryology, anatomy and radiography anatomy; it continues with blood supply and an overview of temporal bone pathology. Subsequent chapters cover malformations, trauma, infections, tumors, postoperative changes, glomus tumors, vertebasilar insufficiency, and facial nerve canal lesions. A final chapter demonstrates and discusses magnetic resonance images of the ear and cerebellopontine angle.

  8. Abnormal Development of the Femoral Head Epiphysis in an Infant with no Developmental Dysplasia of the Hip Apparent on Ultrasonography

    PubMed Central

    Atalar, Hakan; Gunay, Cuneyd; Aytekin, Mahmut Nedim

    2014-01-01

    Introduction: In the investigation of hip development in newborns and infants, ultrasonography and radiography are widely used, but their optimal roles in this setting remain controversial. Case Report: Here we describe an 8.5-month-old infant who had undergone hip radiography at a primary care facility and was referred to our hospital to be evaluated for developmental dysplasia of the hip. Ultrasonography showed no developmental dysplasia of the hip according to standard criteria, but developmental retardation of the femoral head was apparent on the radiograph. Conclusion: This patient's findings demonstrate that abnormalities in femoral head epiphysis development can go undetected during routine ultrasonographic evaluations for developmental dysplasia of the hip. PMID:27298982

  9. Steroid abnormalities and the developing brain: Declarative memory for emotionally arousing and neutral material in children with congenital adrenal hyperplasia

    PubMed Central

    Maheu, Françoise S.; Merke, Deborah P.; Schroth, Elizabeth A.; Keil, Margaret F.; Hardin, Julie; Poeth, Kaitlin; Pine, Daniel S.; Ernst, Monique

    2008-01-01

    Summary Steroid hormones modulate memory in animals and human adults. Little is known on the developmental effect of these hormones on the neural networks underlying memory. Using Congenital Adrenal Hyperplasia (CAH) as a naturalistic model of early steroid abnormalities, this study examines the consequences of CAH on memory and its neural correlates for emotionally arousing and neutral material in children. Seventeen patients with CAH and 17 age- and sex-matched healthy children (ages 12 to 14 years) completed the study. Subjects were presented positive, negative and neutral pictures. Memory recall occurred about 30 minutes after viewing the pictures. Children with CAH showed memory deficits for negative pictures compared to healthy children (p < 0.01). There were no group differences on memory performance for either positive or neutral pictures (p’s >0.1). In patients, 24h urinary-free cortisol levels (reflecting glucocorticoid replacement therapy) and testosterone levels were not associated with memory performance. These findings suggest that early steroid imbalances affect memory for negative material in children with CAH. Such memory impairments may result from abnormal brain organization and function following hormonal dysfunction during critical periods of development. PMID:18162329

  10. Chronic discharging ear in a child: are we missing something?

    PubMed

    Dutta, Mainak; Ghatak, Soumya; Biswas, Gautam

    2013-08-01

    Chronic discharging ear, mostly due to middle or external ear infection, is one of the leading causes for seeking healthcare among the paediatric population in a developing country. However, a long-standing forgotten middle ear foreign body forms a rare cause for such presentation demanding a high index of suspicion from the clinicians. Most of them are iatrogenic or accidental, and are removed by conventional permeatal approach; need for tympanotomy is rarely documented in the recent literature. We report the first case where a large stone was introduced into the middle ear through a pre-existing tympanic membrane perforation by the child himself, and only the second documentation of removal of a middle ear foreign body by tympanotomy in a child.

  11. [Local drug therapy for inner ear hearing loss].

    PubMed

    Liebau, A; Plontke, S K

    2015-06-01

    The indications for local drug therapy of inner ear hearing loss include sudden sensorineural hearing loss, Menière's disease, autoimmune-associated hearing loss, ototoxicity as a side effect of other therapies, acute acoustic trauma and improvement of the safety and performance of cochlear implants. Various drugs are currently being used and tested for local treatment of inner ear hearing loss, including glucocorticoids, growth factors, apoptosis inhibitors, antioxidants, TNF-α inhibitors and antibodies. To further a better understanding of pharmacokinetics and the development of rational pharmacotherapy of the inner ear, the"liberation, absorption, distribution, metabolism, elimination" (LADME) principle can be applied to local therapy of the inner ear. Local application strategies can be differentiated into intratympanic applications to the middle ear cavity and direct intralabyrinthine or intracochlear applications.

  12. Abnormal development of sensory-motor, visual temporal and parahippocampal cortex in children with learning disabilities and borderline intellectual functioning

    PubMed Central

    Baglio, Francesca; Cabinio, Monia; Ricci, Cristian; Baglio, Gisella; Lipari, Susanna; Griffanti, Ludovica; Preti, Maria G.; Nemni, Raffaello; Clerici, Mario; Zanette, Michela; Blasi, Valeria

    2014-01-01

    Borderline intellectual functioning (BIF) is a condition characterized by an intelligence quotient (IQ) between 70 and 85. BIF children present with cognitive, motor, social, and adaptive limitations that result in learning disabilities and are more likely to develop psychiatric disorders later in life. The aim of this study was to investigate brain morphometry and its relation to IQ level in BIF children. Thirteen children with BIF and 14 age- and sex-matched typically developing (TD) children were enrolled. All children underwent a full IQ assessment (WISC-III scale) and a magnetic resonance (MR) examination including conventional sequences to assess brain structural abnormalities and high resolution 3D images for voxel-based morphometry analysis. To investigate to what extent the group influenced gray matter (GM) volumes, both univariate and multivariate generalized linear model analysis of variance were used, and the varimax factor analysis was used to explore variable correlations and clusters among subjects. Results showed that BIF children, compared to controls have increased regional GM volume in bilateral sensorimotor and right posterior temporal cortices and decreased GM volume in the right parahippocampal gyrus. GM volumes were highly correlated with IQ indices. The present work is a case study of a group of BIF children showing that BIF is associated with abnormal cortical development in brain areas that have a pivotal role in motor, learning, and behavioral processes. Our findings, although allowing for little generalization to the general population, contribute to the very limited knowledge in this field. Future longitudinal MR studies will be useful in verifying whether cortical features can be modified over time even in association with rehabilitative intervention. PMID:25360097

  13. Inner ear decompression sickness.

    PubMed

    Farmer, J C; Thomas, W G; Youngblood, D G; Bennett, P B

    1976-09-01

    With recent increases in commercial, military, and sport diving to deeper depths, inner ear injuries during such exposures have been encountered more frequently and noted during several phases of diving: during compression, at stable deep depths, with excessive noise exposure in diving, and during decompression. The pathophysiology of these injuries differs, depending upon the phase of diving in which the injuries occur. In this report, 23 cases of hearing loss, tinnitus, and/or vertigo occurring during or shortly after decompression are presented. Thirteen of these cases occurred in helium-oxygen dives involving a change to air during the latter stages of decompression. A significant correlation is present between prompt recompression treatment, relief of symptoms, and lack of residual deficits. Current knowledge indicates that the management of otologic decompression sickness should include: 1. prompt recompression to at least 99 feet deeper than the symptom onset depth; 2. recompression using the previous helium-oxygen mixture when the injuries occur during or shortly after a switch from helium-oxygen to air during the latter stages of decompression; 3. the use of parenteral diazepam for symptom relief and cyclic inhalations of oxygen enriched treatment gases; and 4. the avoidance of further diving by divers who exhibit permanent inner ear injuries after the acute symptoms have subsided.

  14. Radiographic Features of Superior Semicircular Canal Dehiscence in the Setting of Chronic Ear Disease

    PubMed Central

    Gentry, Lindell R.; Kennedy, Tabassum A.; Gubbels, Samuel P.

    2013-01-01

    Objective To determine if radiologic chronic otitis media (COM), both with and without cholesteatoma, is associated with superior semicircular canal dehiscence (SSCD). Study Design Retrospective review of consecutive high-resolution computed tomography (HRCT) scans of the temporal bone. Setting Tertiary care medical center Patients Two-hundred consecutive patients undergoing HRCT of the temporal bone beginning January 1st, 2012. Intervention Imaging was evaluated by three reviewers (two neuroradiologists and one neurotologist). All scans were assessed for the presence of SSCD, cholesteatoma, chronic otomastoiditis, tegmen dehiscence, and for abnormalities of the cochlea, vestibule, facial nerve, and temporal bone vasculature. Main Outcome Measure Ears with COM associated with chronic otomastoiditis or cholesteatoma were compared to those without COM with respect to the presence of SSCD or other temporal bone abnormalities. Statistical analysis was performed to assess for differences between the groups studied. Results 194 patients (388 ears) were included. Cholesteatoma was identified in 48 ears (12.4%) and chronic otomastoiditis in 62 ears (16%). Ten ears with cholesteatoma had ipsilateral SSCD and 8 ears with chronic otomastoiditis had ipsilateral SSCD. In 340 ears without either cholesteatoma or chronic otomastoiditis, SSCD was found in 18 (5.3%). SSCD was found to occur significantly more often in patients with ipsilateral radiologic cholesteatoma. No cases of SSCD were associated with cochlear, facial nerve, or vascular abnormalities. Conclusion Our findings suggest that COM with cholesteatoma is associated with the presence of SSCD, although the nature of this association is unclear. PMID:24136312

  15. Comparative effects of in ovo exposure to sodium perchlorate on development, growth, metabolism, and thyroid function in the common snapping turtle (Chelydra serpentina) and red-eared slider (Trachemys scripta elegans).

    PubMed

    Eisenreich, Karen M; Dean, Karen M; Ottinger, Mary Ann; Rowe, Christopher L

    2012-11-01

    Perchlorate is a surface and groundwater contaminant found in areas associated with munitions and rocket manufacturing and use. It is a thyroid-inhibiting compound, preventing uptake of iodide by the thyroid gland, ultimately reducing thyroid hormone production. As thyroid hormones influence metabolism, growth, and development, perchlorate exposure during the embryonic period may impact embryonic traits that ultimately influence hatchling performance. We topically exposed eggs of red-eared sliders (Trachemys scripta) and snapping turtles (Chelydra serpentina) to 200 and 177 μg/g of perchlorate (as NaClO(4)), respectively, to determine impacts on glandular thyroxine concentrations, embryonic growth and development, and metabolic rates of hatchlings for a period of 2 months post-hatching. In red-eared sliders, in ovo perchlorate exposure delayed hatching, increased external yolk size at hatching, increased hatchling mortality, and reduced total glandular thyroxine concentrations in hatchlings. In snapping turtles, hatching success and standard metabolic rates were reduced, liver and thyroid sizes were increased, and total glandular thyroxine concentrations in hatchlings were reduced after exposure to perchlorate. While both species were negatively affected by exposure, impacts on red-eared sliders were most severe, suggesting that the slider may be a more sensitive sentinel species for studying effects of perchlorate exposure to turtles. PMID:22871607

  16. Auditory Processing in Infancy: Do Early Abnormalities Predict Disorders of Language and Cognitive Development?

    ERIC Educational Resources Information Center

    Guzzetta, Francesco; Conti, Guido; Mercuri, Eugenio

    2011-01-01

    Increasing attention has been devoted to the maturation of sensory processing in the first year of life. While the development of cortical visual function has been thoroughly studied, much less information is available on auditory processing and its early disorders. The aim of this paper is to provide an overview of the assessment techniques for…

  17. Abnormal development of glomerular endothelial and mesangial cells in mice with targeted disruption of the lama3 gene.

    PubMed

    Abrass, C K; Berfield, A K; Ryan, M C; Carter, W G; Hansen, K M

    2006-09-01

    Mice with targeted disruption of the lama3 gene, which encodes the alpha3 chain of laminin-5 (alpha3beta3gamma2, 332), develop a blistering skin disease similar to junctional epidermolysis bullosa in humans. These animals also develop abnormalities in glomerulogenesis. In both wild-type and mutant animals (lama3(-/-)), podocytes secrete glomerular basement membrane and develop foot processes. Endothelial cells migrate into this scaffolding and secrete a layer of basement membrane that fuses with the one formed by the podocyte. In lama3(-/-) animals, glomerular maturation arrests at this stage. Endothelial cells do not attenuate, develop fenestrae, or form typical lumens, and mesangial cells (MCs) were not identified. LN alpha3 subunit (LAMA3) protein was identified in the basement membrane adjacent to glomerular endothelial cells (GEnCs) in normal rats and mice. In developing rat glomeruli, the LAMA3 subunit was first detectable in the early capillary loop stage, which corresponds to the stage at which maturation arrest was observed in the mutant mice. Lama3 mRNA and protein were identified in isolated rat and mouse glomeruli and cultured rat GEnCs, but not MC. These data document expression of LAMA3 in glomeruli and support a critical role for it in GEnC differentiation. Furthermore, LAMA3 chain expression and/or another product of endothelial cells are required for MC migration into the developing glomerulus. PMID:16850021

  18. Overexpression of the CmACS-3 gene in melon causes abnormal pollen development.

    PubMed

    Zhang, H; Luan, F

    2015-09-08

    Sexual diversity expressed by the Curcurbitaceae family is a primary example of developmental plasticity in plants. Most melon genotypes are andromonoecious, where an initial phase of male flowers is followed by a mixture of bisexual and male flowers. Over-expression of the CmACS-3 gene in melon plants showed an increased number of flower buds, and increased femaleness as demonstrated by a larger number bisexual buds. Transformation of CmACS-3 in melons showed earlier development of and an increased number of bisexual buds that matured to anthesis but also increased the rate of development of the bisexual buds to maturity. Field studies showed that CmACS-3-overexpressing melons had earlier mature bisexual flowers, earlier fruit set, and an increased number of fruits set on closely spaced nodes on the main stem.

  19. Overexpression of the CmACS-3 gene in melon causes abnormal pollen development.

    PubMed

    Zhang, H; Luan, F

    2015-01-01

    Sexual diversity expressed by the Curcurbitaceae family is a primary example of developmental plasticity in plants. Most melon genotypes are andromonoecious, where an initial phase of male flowers is followed by a mixture of bisexual and male flowers. Over-expression of the CmACS-3 gene in melon plants showed an increased number of flower buds, and increased femaleness as demonstrated by a larger number bisexual buds. Transformation of CmACS-3 in melons showed earlier development of and an increased number of bisexual buds that matured to anthesis but also increased the rate of development of the bisexual buds to maturity. Field studies showed that CmACS-3-overexpressing melons had earlier mature bisexual flowers, earlier fruit set, and an increased number of fruits set on closely spaced nodes on the main stem. PMID:26400274

  20. A mouse model for eukaryotic translation initiation factor 2B-leucodystrophy reveals abnormal development of brain white matter.

    PubMed

    Geva, Michal; Cabilly, Yuval; Assaf, Yaniv; Mindroul, Nina; Marom, Liraz; Raini, Gali; Pinchasi, Dalia; Elroy-Stein, Orna

    2010-08-01

    Eukaryotic translation initiation factor 2B is a major housekeeping complex that governs the rate of global protein synthesis under normal and stress conditions. Mutations in any of its five subunits lead to leucoencephalopathy with vanishing white matter, an inherited chronic-progressive fatal brain disease with unknown aetiology, which is among the most prevalent childhood white matter disorders. We generated the first animal model for the disease by introducing a point mutation into the mouse Eif2b5 gene locus, leading to R132H replacement corresponding to the clinically significant human R136H mutation in the catalytic subunit. In contrast to human patients, mice homozygous for the mutant Eif2b5 allele (Eif2b5(R132H/R132H) mice) enable multiple analyses under a defined genetic background during the pre-symptomatic stages and during recovery from a defined brain insult. Time-course magnetic resonance imaging revealed for the first time the delayed development of the brain white matter due to the mutation. Electron microscopy demonstrated a higher proportion of small-calibre nerve fibres. Immunohistochemistry detected an abnormal abundance of oligodendrocytes and astrocytes in the brain of younger animals, as well as an abnormal level of major myelin proteins. Most importantly, mutant mice failed to recover from cuprizone-induced demyelination, reflecting an increased sensitivity to brain insults. The anomalous development of white matter in Eif2b5(R132H/R132H) mice underscores the importance of tight translational control to normal myelin formation and maintenance.

  1. Wax blockage in the ear (image)

    MedlinePlus

    The ear canal is lined with hair follicles and glands that produce a waxy oil called cerumen. Sometimes the ... wax than can be easily excreted out the ear. This extra wax may harden within the ear ...

  2. Otoscopic exam of the ear (image)

    MedlinePlus

    ... intrument which is used to look into the ear canal. The ear speculum (a cone-shaped viewing piece of the otoscope) is slowly inserted into the ear canal while looking into the otoscope. The speculum ...

  3. Middle ear infection (otitis media) (image)

    MedlinePlus

    ... is an inflammation and/or infection of the middle ear. Acute otitis media (acute ear infection) occurs ... or viral infection of the fluid of the middle ear, which causes production of fluid or pus. ...

  4. Annual Research Review: Growth connectomics – the organization and reorganization of brain networks during normal and abnormal development

    PubMed Central

    Vértes, Petra E; Bullmore, Edward T

    2015-01-01

    Background We first give a brief introduction to graph theoretical analysis and its application to the study of brain network topology or connectomics. Within this framework, we review the existing empirical data on developmental changes in brain network organization across a range of experimental modalities (including structural and functional MRI, diffusion tensor imaging, magnetoencephalography and electroencephalography in humans). Synthesis We discuss preliminary evidence and current hypotheses for how the emergence of network properties correlates with concomitant cognitive and behavioural changes associated with development. We highlight some of the technical and conceptual challenges to be addressed by future developments in this rapidly moving field. Given the parallels previously discovered between neural systems across species and over a range of spatial scales, we also review some recent advances in developmental network studies at the cellular scale. We highlight the opportunities presented by such studies and how they may complement neuroimaging in advancing our understanding of brain development. Finally, we note that many brain and mind disorders are thought to be neurodevelopmental in origin and that charting the trajectory of brain network changes associated with healthy development also sets the stage for understanding abnormal network development. Conclusions We therefore briefly review the clinical relevance of network metrics as potential diagnostic markers and some recent efforts in computational modelling of brain networks which might contribute to a more mechanistic understanding of neurodevelopmental disorders in future. PMID:25441756

  5. A mitochondrial DNA sequence is associated with abnormal pollen development in cytoplasmic male sterile bean plants.

    PubMed Central

    Johns, C; Lu, M; Lyznik, A; Mackenzie, S

    1992-01-01

    Cytoplasmic male sterility (CMS) in common bean is associated with the presence of a 3-kb unique mitochondrial sequence designated pvs. The pvs sequence encodes at least two open reading frames (297 and 720 bp in length) with portions derived from the chloroplast genome. Fertility restoration by the nuclear restorer gene Fr results in the loss of this transcriptionally active unique region. We examined the effect of CMS (pvs present) and fertility restoration by Fr (pvs absent) on the pattern of pollen development in bean. In the CMS line, pollen aborted in the tetrad stage late in microgametogenesis. Microspores maintained cytoplasmic connections throughout pollen development, indicating aberrant or incomplete cytokinesis. Pollen-specific events associated with pollen abortion and fertility restoration imply that a gametophytic factor or event may be involved in CMS. In situ hybridization experiments suggested that significant reduction or complete loss of the mitochondrial sterility-associated sequence occurred in fertile pollen of F2 populations segregating for fertility. These observations support a model of fertility restoration by the loss of a mitochondrial DNA sequence prior to or during microsporogenesis/gametogenesis. PMID:1498602

  6. P53 functional abnormality in mesenchymal stem cells promotes osteosarcoma development

    PubMed Central

    Velletri, T; Xie, N; Wang, Y; Huang, Y; Yang, Q; Chen, X; Chen, Q; Shou, P; Gan, Y; Cao, G; Melino, G; Shi, Y

    2016-01-01

    It has been shown that p53 has a critical role in the differentiation and functionality of various multipotent progenitor cells. P53 mutations can lead to genome instability and subsequent functional alterations and aberrant transformation of mesenchymal stem cells (MSCs). The significance of p53 in safeguarding our body from developing osteosarcoma (OS) is well recognized. During bone remodeling, p53 has a key role in negatively regulating key factors orchestrating the early stages of osteogenic differentiation of MSCs. Interestingly, changes in the p53 status can compromise bone homeostasis and affect the tumor microenvironment. This review aims to provide a unique opportunity to study the p53 function in MSCs and OS. In the context of loss of function of p53, we provide a model for two sources of OS: MSCs as progenitor cells of osteoblasts and bone tumor microenvironment components. Standing at the bone remodeling point of view, in this review we will first explain the determinant function of p53 in OS development. We will then summarize the role of p53 in monitoring MSC fidelity and in regulating MSC differentiation programs during osteogenesis. Finally, we will discuss the importance of loss of p53 function in tissue microenvironment. We expect that the information provided herein could lead to better understanding and treatment of OS. PMID:26775693

  7. Successful Bilateral Composite Ear Reattachment

    PubMed Central

    2014-01-01

    Summary: A successful bilateral ear composite graft nonmicrosurgical reattachment is presented. In cases where suitable vessels are unavailable for microsurgical revascularization, the reconstructive challenge can be formidable for salvaging the unique anatomic and aesthetic structure of the ear. The case is presented of an 18-year-old woman who was a victim of an assault wherein both of her ears were intentionally amputated by her attacker. She underwent successful surgical reattachment followed by a postoperative regimen of hyperbaric oxygen, cooling, and meticulous wound care. The patient achieved 100% survival of her left ear graft and 95% survival of her right ear graft. Clinical photographs at 18 months are presented, along with a discussion of the possible implications for other reconstructive applications. PMID:25289367

  8. Ear Reconstruction in Young Children.

    PubMed

    Reinisch, John

    2015-12-01

    The use of a porous high-density polyethylene ear implant, rather than a costal cartilage framework, allows ear reconstruction in young children before they enter school. The fact that the growth of the normal ear matures early allows for good symmetry. If the implant is covered completely with a large, well-vascularized superficial parietal fascia flap and appropriately color-matched skin, an ear with excellent projection and definition can be obtained with minimal complications and long-term viability. Ear reconstruction in young children is preferred by the author because the necessary fascial flap coverage is thinner, easier to harvest than in older patients, and can be done in a single outpatient procedure with minimal discomfort or psychological trauma. PMID:26667634

  9. Abnormal development of monoaminergic neurons is implicated in mood fluctuations and bipolar disorder.

    PubMed

    Jukic, Marin M; Carrillo-Roa, Tania; Bar, Michal; Becker, Gal; Jovanovic, Vukasin M; Zega, Ksenija; Binder, Elisabeth B; Brodski, Claude

    2015-03-01

    Subtle mood fluctuations are normal emotional experiences, whereas drastic mood swings can be a manifestation of bipolar disorder (BPD). Despite their importance for normal and pathological behavior, the mechanisms underlying endogenous mood instability are largely unknown. During embryogenesis, the transcription factor Otx2 orchestrates the genetic networks directing the specification of dopaminergic (DA) and serotonergic (5-HT) neurons. Here we behaviorally phenotyped mouse mutants overexpressing Otx2 in the hindbrain, resulting in an increased number of DA neurons and a decreased number of 5-HT neurons in both developing and mature animals. Over the course of 1 month, control animals exhibited stable locomotor activity in their home cages, whereas mutants showed extended periods of elevated or decreased activity relative to their individual average. Additional behavioral paradigms, testing for manic- and depressive-like behavior, demonstrated that mutants showed an increase in intra-individual fluctuations in locomotor activity, habituation, risk-taking behavioral parameters, social interaction, and hedonic-like behavior. Olanzapine, lithium, and carbamazepine ameliorated the behavioral alterations of the mutants, as did the mixed serotonin receptor agonist quipazine and the specific 5-HT2C receptor agonist CP-809101. Testing the relevance of the genetic networks specifying monoaminergic neurons for BPD in humans, we applied an interval-based enrichment analysis tool for genome-wide association studies. We observed that the genes specifying DA and 5-HT neurons exhibit a significant level of aggregated association with BPD but not with schizophrenia or major depressive disorder. The results of our translational study suggest that aberrant development of monoaminergic neurons leads to mood fluctuations and may be associated with BPD.

  10. Craniofacial abnormalities among patients with Edwards Syndrome

    PubMed Central

    Rosa, Rafael Fabiano M.; Rosa, Rosana Cardoso M.; Lorenzen, Marina Boff; Zen, Paulo Ricardo G.; Graziadio, Carla; Paskulin, Giorgio Adriano

    2013-01-01

    OBJECTIVE To determine the frequency and types of craniofacial abnormalities observed in patients with trisomy 18 or Edwards syndrome (ES). METHODS This descriptive and retrospective study of a case series included all patients diagnosed with ES in a Clinical Genetics Service of a reference hospital in Southern Brazil from 1975 to 2008. The results of the karyotypic analysis, along with clinical data, were collected from medical records. RESULTS: The sample consisted of 50 patients, of which 66% were female. The median age at first evaluation was 14 days. Regarding the karyotypes, full trisomy of chromosome 18 was the main alteration (90%). Mosaicism was observed in 10%. The main craniofacial abnormalities were: microretrognathia (76%), abnormalities of the ear helix/dysplastic ears (70%), prominent occiput (52%), posteriorly rotated (46%) and low set ears (44%), and short palpebral fissures/blepharophimosis (46%). Other uncommon - but relevant - abnormalities included: microtia (18%), orofacial clefts (12%), preauricular tags (10%), facial palsy (4%), encephalocele (4%), absence of external auditory canal (2%) and asymmetric face (2%). One patient had an initial suspicion of oculo-auriculo-vertebral spectrum (OAVS) or Goldenhar syndrome. CONCLUSIONS: Despite the literature description of a characteristic clinical presentation for ES, craniofacial alterations may be variable among these patients. The OAVS findings in this sample are noteworthy. The association of ES with OAVS has been reported once in the literature. PMID:24142310

  11. Developing and testing a multi-probe resonance electrical impedance spectroscopy system for detecting breast abnormalities

    NASA Astrophysics Data System (ADS)

    Gur, David; Zheng, Bin; Dhurjaty, Sreeram; Wolfe, Gene; Fradin, Mary; Weil, Richard; Sumkin, Jules; Zuley, Margarita

    2009-02-01

    In our previous study, we reported on the development and preliminary testing of a prototype resonance electrical impedance spectroscopy (REIS) system with a pair of probes. Although our pilot study on 150 young women ranging from 30 to 50 years old indicated the feasibility of using REIS output sweep signals to classify between the women who had negative examinations and those who would ultimately be recommended for biopsy, the detection sensitivity was relatively low. To improve performance when using REIS technology, we recently developed a new multi-probe based REIS system. The system consists of a sensor module box that can be easily lifted along a vertical support device to fit women of different height. Two user selectable breast placement "cups" with different curvatures are included in the system. Seven probes are mounted on each of the cups on opposing sides of the sensor box. By rotating the sensor box, the technologist can select the detection sensor cup that better fits the breast size of the woman being examined. One probe is mounted in the cup center for direct contact with the nipple and the other six probes are uniformly distributed along an outside circle to enable contact with six points on the outer and inner breast skin surfaces. The outer probes are located at a distance of 60mm away from the center (nipple) probe. The system automatically monitors the quality of the contact between the breast surface and each of the seven probes and data acquisition can only be initiated when adequate contact is confirmed. The measurement time for each breast is approximately 15 seconds during which time the system records 121 REIS signal sweep outputs generated from 200 KHz to 800 KHz at 5 KHz increments for all preselected probe pairs. Currently we are measuring 6 pairs between the center probe and each of six probes located on the outer circle as well as two pairs between probe pairs on the outer circle. This new REIS system has been installed in our

  12. Neural tube opening and abnormal extraembryonic membrane development in SEC23A deficient mice

    PubMed Central

    Zhu, Min; Tao, Jiayi; Vasievich, Matthew P.; Wei, Wei; Zhu, Guojing; Khoriaty, Rami N.; Zhang, Bin

    2015-01-01

    COPII (coat protein complex-II) vesicles transport proteins from the endoplasmic reticulum (ER) to the Golgi. Higher eukaryotes have two or more paralogs of most COPII components. Here we characterize mice deficient for SEC23A and studied interactions of Sec23a null allele with the previously reported Sec23b null allele. SEC23A deficiency leads to mid-embryonic lethality associated with defective development of extraembryonic membranes and neural tube opening in midbrain. Secretion defects of multiple collagen types are observed in different connective tissues, suggesting that collagens are primarily transported in SEC23A-containing vesicles in these cells. Other extracellular matrix proteins, such as fibronectin, are not affected by SEC23A deficiency. Intracellular accumulation of unsecreted proteins leads to strong induction of the unfolded protein response in collagen-producing cells. No collagen secretion defects are observed in SEC23B deficient embryos. We report that E-cadherin is a cargo that accumulates in acini of SEC23B deficient pancreas and salivary glands. Compensatory increase of one paralog is observed in the absence of the second paralog. Haploinsufficiency of the remaining Sec23 paralog on top of homozygous inactivation of the first paralog leads to earlier lethality of embryos. Our results suggest that mammalian SEC23A and SEC23B transport overlapping yet distinct spectra of cargo in vivo. PMID:26494538

  13. Zebrafish embryos exposed to alcohol undergo abnormal development of motor neurons and muscle fibers.

    PubMed

    Sylvain, Nicole J; Brewster, Daniel L; Ali, Declan W

    2010-01-01

    Children exposed to alcohol in utero have significantly delayed gross and fine motor skills, as well as deficiencies in reflex development. The reasons that underlie the motor deficits caused by ethanol (EtOH) exposure remain to be fully elucidated. The present study was undertaken to investigate the effects of embryonic alcohol exposure (1.5%, 2% and 2.5% EtOH) on motor neuron and muscle fiber morphology in 3 days post fertilization (dpf) larval zebrafish. EtOH treated fish exhibited morphological deformities and fewer bouts of swimming in response to touch, compared with untreated fish. Immunolabelling with anti-acetylated tubulin indicated that fish exposed to 2.5% EtOH had significantly higher rates of motor neuron axon defects. Immunolabelling of primary and secondary motor neurons, using znp-1 and zn-8, revealed that fish exposed to 2% and 2.5% EtOH exhibited significantly higher rates of primary and secondary motor neuron axon defects compared to controls. Examination of red and white muscle fibers revealed that fish exposed to EtOH had significantly smaller fibers compared with controls. These findings indicate that motor neuron and muscle fiber morphology is affected by early alcohol exposure in zebrafish embryos, and that this may be related to deficits in locomotion. PMID:20211721

  14. Roles of retinoic acid signaling in normal and abnormal development of the palate and tongue.

    PubMed

    Okano, Junko; Udagawa, Jun; Shiota, Kohei

    2014-05-01

    Palatogenesis involves various developmental events such as growth, elevation, elongation and fusion of opposing palatal shelves. Extrinsic factors such as mouth opening and subsequent tongue withdrawal are also needed for the horizontal elevation of palate shelves. Failure of any of these steps can lead to cleft palate, one of the most common birth defects in humans. It has been shown that retinoic acid (RA) plays important roles during palate development, but excess RA causes cleft palate in fetuses of both rodents and humans. Thus, the coordinated regulation of retinoid metabolism is essential for normal palatogenesis. The endogenous RA level is determined by the balance of RA-synthesizing (retinaldehyde dehydrogenases: RALDHs) and RA-degrading enzymes (CYP26s). Cyp26b1 is a key player in normal palatogenesis. In this review, we discuss recent progress in the study of the pathogenesis of RA-induced cleft palate, with special reference to the regulation of endogenous RA levels by RA-degrading enzymes.

  15. Role of abnormal lipid metabolism in development, progression, diagnosis and therapy of pancreatic cancer

    PubMed Central

    Swierczynski, Julian; Hebanowska, Areta; Sledzinski, Tomasz

    2014-01-01

    There is growing evidence that metabolic alterations play an important role in cancer development and progression. The metabolism of cancer cells is reprogrammed in order to support their rapid proliferation. Elevated fatty acid synthesis is one of the most important aberrations of cancer cell metabolism. An enhancement of fatty acids synthesis is required both for carcinogenesis and cancer cell survival, as inhibition of key lipogenic enzymes slows down the growth of tumor cells and impairs their survival. Based on the data that serum fatty acid synthase (FASN), also known as oncoantigen 519, is elevated in patients with certain types of cancer, its serum level was proposed as a marker of neoplasia. This review aims to demonstrate the changes in lipid metabolism and other metabolic processes associated with lipid metabolism in pancreatic ductal adenocarcinoma (PDAC), the most common pancreatic neoplasm, characterized by high mortality. We also addressed the influence of some oncogenic factors and tumor suppressors on pancreatic cancer cell metabolism. Additionally the review discusses the potential role of elevated lipid synthesis in diagnosis and treatment of pancreatic cancer. In particular, FASN is a viable candidate for indicator of pathologic state, marker of neoplasia, as well as, pharmacological treatment target in pancreatic cancer. Recent research showed that, in addition to lipogenesis, certain cancer cells can use fatty acids from circulation, derived from diet (chylomicrons), synthesized in liver, or released from adipose tissue for their growth. Thus, the interactions between de novo lipogenesis and uptake of fatty acids from circulation by PDAC cells require further investigation. PMID:24605027

  16. Abnormal etioplast development in barley seedlings infected with BSMV by seed transmission.

    PubMed

    Harsányi, Anett; Böddi, Béla; Bóka, Károly; Almási, Asztéria; Gáborjányi, Richard

    2002-01-01

    The effect of barley stripe mosaic hordeivirus (BSMV) was studied on the ultrastructure of etioplasts, protochlorophyllide forms and the greening process of barley (Hordeum vulgare cv. Pannónia) plants infected by seed transmission. The leaves of 7- to 11-day-old etiolated seedlings were examined by transmission electron microscopy, fluorescence and absorption spectroscopy. The etioplasts of infected seedlings contained smaller prolamellar bodies with less regular membrane structure, while prothylakoid content was higher than in the control. The protochlorophyllide content of virus-infected seedlings was reduced to 74% of the control. In the 77 K fluorescence spectra the relative amount of 655 nm emitting photoactive protochlorophyllide form decreased, and the amount of the 645 and 633 nm emitting forms increased in the infected leaves. A characteristic effect was observed in the process of the Shibata-shift: 40 min delay was observed in the infected leaves. The results of this work proved that BSMV infection delays or inhibits plastid development and the formation of photosynthetic apparatus. PMID:11982946

  17. Deficiency in DGCR8-dependent canonical microRNAs causes infertility due to multiple abnormalities during uterine development in mice.

    PubMed

    Kim, Yeon Sun; Kim, Hye-Ryun; Kim, Hyongbum; Yang, Seung Chel; Park, Mira; Yoon, Jung Ah; Lim, Hyunjung J; Hong, Seok-Ho; DeMayo, Francesco J; Lydon, John P; Choi, Youngsok; Lee, Dong Ryul; Song, Haengseok

    2016-01-01

    DGCR8 is an RNA-binding protein that interacts with DROSHA to produce pre-microRNA in the nucleus, while DICER generates not only mature microRNA, but also endogenous small interfering RNAs in the cytoplasm. Here, we produced Dgcr8 conditional knock-out mice using progesterone receptor (PR)-Cre (Dgcr8(d/d)) and demonstrated that canonical microRNAs dependent on the DROSHA-DGCR8 complex are required for uterine development as well as female fertility in mice. Adult Dgcr8(d/d) females neither underwent regular reproductive cycles nor produced pups, whereas administration of exogenous gonadotropins induced normal ovulation in these mice. Interestingly, immune cells associated with acute inflammation aberrantly infiltrated into reproductive organs of pregnant Dgcr8(d/d) mice. Regarding uterine development, multiple uterine abnormalities were noticeable at 4 weeks of age when PR is significantly increased, and the severity of these deformities increased over time. Gland formation and myometrial layers were significantly reduced, and the stromal cell compartment did not expand and became atrophic during uterine development in these mice. These results were consistent with aberrantly reduced stromal cell proliferation and completely failed decidualization. Collectively, we suggest that DGCR8-dependent canonical microRNAs are essential for uterine development and physiological processes such as proper immune modulation, reproductive cycle, and steroid hormone responsiveness in mice. PMID:26833131

  18. Deficiency in DGCR8-dependent canonical microRNAs causes infertility due to multiple abnormalities during uterine development in mice

    PubMed Central

    Kim, Yeon Sun; Kim, Hye-Ryun; Kim, Hyongbum; Yang, Seung Chel; Park, Mira; Yoon, Jung Ah; Lim, Hyunjung J.; Hong, Seok-Ho; DeMayo, Francesco J.; Lydon, John P.; Choi, Youngsok; Lee, Dong Ryul; Song, Haengseok

    2016-01-01

    DGCR8 is an RNA-binding protein that interacts with DROSHA to produce pre-microRNA in the nucleus, while DICER generates not only mature microRNA, but also endogenous small interfering RNAs in the cytoplasm. Here, we produced Dgcr8 conditional knock-out mice using progesterone receptor (PR)-Cre (Dgcr8d/d) and demonstrated that canonical microRNAs dependent on the DROSHA-DGCR8 complex are required for uterine development as well as female fertility in mice. Adult Dgcr8d/d females neither underwent regular reproductive cycles nor produced pups, whereas administration of exogenous gonadotropins induced normal ovulation in these mice. Interestingly, immune cells associated with acute inflammation aberrantly infiltrated into reproductive organs of pregnant Dgcr8d/d mice. Regarding uterine development, multiple uterine abnormalities were noticeable at 4 weeks of age when PR is significantly increased, and the severity of these deformities increased over time. Gland formation and myometrial layers were significantly reduced, and the stromal cell compartment did not expand and became atrophic during uterine development in these mice. These results were consistent with aberrantly reduced stromal cell proliferation and completely failed decidualization. Collectively, we suggest that DGCR8-dependent canonical microRNAs are essential for uterine development and physiological processes such as proper immune modulation, reproductive cycle, and steroid hormone responsiveness in mice. PMID:26833131

  19. Diving injuries to the inner ear.

    PubMed

    Farmer, J C

    1977-01-01

    Most of the previous literature concerning otologic problems in compressed gas environments has emphasized middle ear barotrauma. With recent increases in commercial, military, and sport diving to deeper depths, inner ear disturbances during these exposures have been noted more frequently. Studies of inner ear physiology and pathology during diving indicate that the causes and treatment of these problems differ depending upon the phase and type of diving. Humans exposed to simulated depths of up to 305 meters without barotrauma or decompression sickness develop transient, conductive hearing losses with no audiometric evidence of cochlear dysfunction. Transient vertigo and nystagmus during diving have been noted with caloric stimulation, resulting from the unequal entry of cold water into the external auditory canals, and with asymmetric middle ear pressure equilibration during ascent and descent (alternobaric vertigo). Equilibrium disturbances noted with nitrogen narcosis, oxygen toxicity, hypercarbia, or hypoxia appear primarily related to the effects of these conditions upon the central nervous system and not to specific vestibular end-organ dysfunction. Compression of humans in helium-oxygen at depths greater than 152.4 meters results in transient symptoms of tremor, dizziness, and nausea plus decrements in postural equilibrium and psychomotor performance, the high pressure nervous syndrome. Vestibular function studies during these conditions indicate that these problems are due to central dysfunction and not to vestibular end-organ dysfunction. Persistent inner ear injuries have been noted during several phases of diving: 1) Such injuries during compression (inner ear barotrauma) have been related to round window ruptures occurring with straining, or a Valsalva's maneuver during inadequate middle ear pressure equilibration. Divers who develop cochlear and/or vestibular symptoms during shallow diving in which decompression sickness is unlikely or during

  20. Diving injuries to the inner ear.

    PubMed

    Farmer, J C

    1977-01-01

    Most of the previous literature concerning otologic problems in compressed gas environments has emphasized middle ear barotrauma. With recent increases in commercial, military, and sport diving to deeper depths, inner ear disturbances during these exposures have been noted more frequently. Studies of inner ear physiology and pathology during diving indicate that the causes and treatment of these problems differ depending upon the phase and type of diving. Humans exposed to simulated depths of up to 305 meters without barotrauma or decompression sickness develop transient, conductive hearing losses with no audiometric evidence of cochlear dysfunction. Transient vertigo and nystagmus during diving have been noted with caloric stimulation, resulting from the unequal entry of cold water into the external auditory canals, and with asymmetric middle ear pressure equilibration during ascent and descent (alternobaric vertigo). Equilibrium disturbances noted with nitrogen narcosis, oxygen toxicity, hypercarbia, or hypoxia appear primarily related to the effects of these conditions upon the central nervous system and not to specific vestibular end-organ dysfunction. Compression of humans in helium-oxygen at depths greater than 152.4 meters results in transient symptoms of tremor, dizziness, and nausea plus decrements in postural equilibrium and psychomotor performance, the high pressure nervous syndrome. Vestibular function studies during these conditions indicate that these problems are due to central dysfunction and not to vestibular end-organ dysfunction. Persistent inner ear injuries have been noted during several phases of diving: 1) Such injuries during compression (inner ear barotrauma) have been related to round window ruptures occurring with straining, or a Valsalva's maneuver during inadequate middle ear pressure equilibration. Divers who develop cochlear and/or vestibular symptoms during shallow diving in which decompression sickness is unlikely or during

  1. R6/2 Huntington’s disease Mice Develop Early and Progressive Abnormal Brain Metabolism and Seizures

    PubMed Central

    Cepeda-Prado, E; Popp, S; Khan, U; Stefanov, D; Rodriguez, J; Menalled, L; Dow-Edwards, D; Small, SA; Moreno, H

    2012-01-01

    A hallmark feature of Huntington's disease pathology is the atrophy of brain regions including, but not limited to, the striatum. Though MRI studies have identified structural CNS changes in several HD mouse models, the functional consequences of HD pathology during the progression of the disease have yet to be investigated using in vivo functional magnetic resonance imaging (fMRI). To address this issue, we first established the structural and functional MRI phenotype of juvenile HD mouse model R6/2 at early and advanced stages of disease. Significantly higher fMRI-signals (relative cerebral blood volumes-rCBV) and atrophy were observed in both age groups in specific brain regions. Next, fMRI results were correlated with electrophysiological analysis, which showed abnormal increases in neuronal activity in affected brain regions- thus identifying a mechanism accounting for the abnormal fMRI findings. [14C] deoxyglucose (2DG) maps to investigate patterns of glucose utilization were also generated. An interesting mismatch between increases in rCBV and decreases in glucose uptake was observed. Finally, we evaluated the sensitivity of this mouse line to audiogenic seizures early in the disease course. We found that R6/2 mice had an increased susceptibility to develop seizures. Together, these findings identified seizure activity in R6/2 mice, and show that neuroimaging measures sensitive to oxygen metabolism can be used as in vivo biomarkers, preceding the onset of an overt behavioral phenotype. Since fMRI-rCBV can also be obtained in patients, we propose that it may serve as a translational tool to evaluate therapeutic responses in humans and HD mouse models. PMID:22573668

  2. Knockdown of zebrafish Lgi1a results in abnormal development, brain defects and a seizure-like behavioral phenotype

    PubMed Central

    Teng, Yong; Xie, Xiayang; Walker, Steven; Rempala, Grzegorz; Kozlowski, David J.; Mumm, Jeff S.; Cowell, John K.

    2010-01-01

    Epilepsy is a common disorder, typified by recurrent seizures with underlying neurological disorders or disease. Approximately one-third of patients are unresponsive to currently available therapies. Thus, a deeper understanding of the genetics and etiology of epilepsy is needed to advance the development of new therapies. Previously, treatment of zebrafish with epilepsy-inducing pharmacological agents was shown to result in a seizure-like phenotype, suggesting that fish provide a tractable model to understand the function of epilepsy-predisposing genes. Here, we report the first model of genetically linked epilepsy in zebrafish and provide an initial characterization of the behavioral and neurological phenotypes associated with morpholino (MO) knockdown of leucine-rich, glioma-inactivated 1a (lgi1a) expression. Mutations in the LGI1 gene in humans have been shown to predispose to a subtype of autosomal dominant epilepsy. Low-dose Lgi1a MO knockdown fish (morphants) appear morphologically normal but are sensitized to epilepsy-inducing drugs. High-dose Lgi1a morphants have morphological defects which persist into adult stages that are typified by smaller brains and eyes and abnormalities in tail shape, and display hyperactive swimming behaviors. Increased apoptosis was observed throughout the central nervous system of high-dose morphant fish, accounting for the size reduction of neural tissues. These observations demonstrate that zebrafish can be exploited to dissect the embryonic function(s) of genes known to predispose to seizure-like behavior in humans, and offer potential insight into the relationship between developmental neurobiological abnormalities and seizure. PMID:20819949

  3. Radiologic evaluation of the ear anatomy in pediatric cholesteatoma.

    PubMed

    Manolis, Evangelos N; Filippou, Dimitrios K; Tsoumakas, Constantinos; Diomidous, Marianna; Cunningham, Michael J; Katostaras, Theophanis; Weber, Alfred L; Eavey, Roland D

    2009-05-01

    The aim of the study was to describe computed tomography (CT) findings in middle ear cholesteatoma in pediatric patients. A cohort of 32 children with cholesteatoma (3-14 years old) entered the study. From them, 30 presented acquired cholesteatoma (AC), and 2 presented congenital cholesteatoma. All of the children were investigated using CT before surgery of the middle ear and mastoid. Computed tomography was performed with 1- or 2-mm axial and coronal sections of both temporal bones. Nineteen children with AC (63.3%) revealed a diffuse soft-tissue density isodense with muscle, whereas in 6 of them, the mass mimicked inflammation. The remaining revealed localized soft-tissue mass with partially lobulated contour. In AC, ossicular erosion was detected in 23 cases (76.7%), abnormal pneumatization in 19 cases (63.3%), and erosion-blunting of spur and enlargement of middle ear or mastoid in 8 cases (26.7%). The 2 congenital cholesteatomas revealed soft-tissue mass with polypoid densities, while a semicircular canal fistula was detected in one of them. High-resolution CT facilitates early diagnosis and appropriate treatment of pediatric cholesteatoma by assessing the anatomic abnormalities and the extent of disease, which are crucial in middle ear and mastoid surgery. PMID:19390457

  4. Do Swiftlets have an ear for echolocation? The functional morphology of Swiftlets' middle ears.

    PubMed

    Thomassen, Henri A; Gea, Stefan; Maas, Steve; Bout, Ron G; Dirckx, Joris J J; Decraemer, Willem F; Povel, G David E

    2007-03-01

    The Oilbird and many Swiftlet species are unique among birds for their ability to echolocate. Echolocaters may benefit from improved hearing sensitivity. Therefore, morphological adaptations to echolocation might be present in echolocating birds' middle ears. We studied the functional morphology of the tympano-ossicular chain of seven specimens of four echolocating Swiftlet species and one specimen each of five non-echolocating species. Three dimensional (3D) reconstructions were made from micro-Computer-Tomographic (muCT) scans. The reconstructions were used in functional morphological analyses and model calculations. A two dimensional (2D) rigid rod model with fixed rotational axes was developed to study footplate output-amplitudes and to describe how changes in the arrangement of the tympano-ossicular chain affect its function. A 3D finite element model was used to predict ossicular-chain movement and to investigate the justification of the 2D approach. No morphological adaptations towards echolocation were found in the middle-ear lever system or in the mass impedance of the middle ear. A wide range of middle-ear configurations result in maximum output-amplitudes and all investigated species are congruent with these predicted best configurations. Echolocation is unlikely to depend on adaptations in the middle ear tympano-ossicular chain. PMID:17229537

  5. Developing an Ear Prosthesis Fabricated in Polyvinylidene Fluoride by a 3D Printer with Sensory Intrinsic Properties of Pressure and Temperature.

    PubMed

    Suaste-Gómez, Ernesto; Rodríguez-Roldán, Grissel; Reyes-Cruz, Héctor; Terán-Jiménez, Omar

    2016-03-04

    An ear prosthesis was designed in 3D computer graphics software and fabricated using a 3D printing process of polyvinylidene fluoride (PVDF) for use as a hearing aid. In addition, the prosthesis response to pressure and temperature was observed. Pyroelectric and piezoelectric properties of this ear prosthesis were investigated using an astable multivibrator circuit, as changes in PVDF permittivity were observed according to variations of pressure and temperature. The results show that this prosthesis is reliable for use under different conditions of pressure (0 Pa to 16,350 Pa) and temperature (2 °C to 90 °C). The experimental results show an almost linear and inversely proportional behavior between the stimuli of pressure and temperature with the frequency response. This 3D-printed ear prosthesis is a promising tool and has a great potentiality in the biomedical engineering field because of its ability to generate an electrical potential proportional to pressure and temperature, and it is the first time that such a device has been processed by the additive manufacturing process (3D printing). More work needs to be carried out to improve the performance, such as electrical stimulation of the nervous system, thereby extending the purpose of a prosthesis to the area of sensory perception.

  6. Developing an Ear Prosthesis Fabricated in Polyvinylidene Fluoride by a 3D Printer with Sensory Intrinsic Properties of Pressure and Temperature.

    PubMed

    Suaste-Gómez, Ernesto; Rodríguez-Roldán, Grissel; Reyes-Cruz, Héctor; Terán-Jiménez, Omar

    2016-01-01

    An ear prosthesis was designed in 3D computer graphics software and fabricated using a 3D printing process of polyvinylidene fluoride (PVDF) for use as a hearing aid. In addition, the prosthesis response to pressure and temperature was observed. Pyroelectric and piezoelectric properties of this ear prosthesis were investigated using an astable multivibrator circuit, as changes in PVDF permittivity were observed according to variations of pressure and temperature. The results show that this prosthesis is reliable for use under different conditions of pressure (0 Pa to 16,350 Pa) and temperature (2 °C to 90 °C). The experimental results show an almost linear and inversely proportional behavior between the stimuli of pressure and temperature with the frequency response. This 3D-printed ear prosthesis is a promising tool and has a great potentiality in the biomedical engineering field because of its ability to generate an electrical potential proportional to pressure and temperature, and it is the first time that such a device has been processed by the additive manufacturing process (3D printing). More work needs to be carried out to improve the performance, such as electrical stimulation of the nervous system, thereby extending the purpose of a prosthesis to the area of sensory perception. PMID:26959026

  7. Developing an Ear Prosthesis Fabricated in Polyvinylidene Fluoride by a 3D Printer with Sensory Intrinsic Properties of Pressure and Temperature

    PubMed Central

    Suaste-Gómez, Ernesto; Rodríguez-Roldán, Grissel; Reyes-Cruz, Héctor; Terán-Jiménez, Omar

    2016-01-01

    An ear prosthesis was designed in 3D computer graphics software and fabricated using a 3D printing process of polyvinylidene fluoride (PVDF) for use as a hearing aid. In addition, the prosthesis response to pressure and temperature was observed. Pyroelectric and piezoelectric properties of this ear prosthesis were investigated using an astable multivibrator circuit, as changes in PVDF permittivity were observed according to variations of pressure and temperature. The results show that this prosthesis is reliable for use under different conditions of pressure (0 Pa to 16,350 Pa) and temperature (2 °C to 90 °C). The experimental results show an almost linear and inversely proportional behavior between the stimuli of pressure and temperature with the frequency response. This 3D-printed ear prosthesis is a promising tool and has a great potentiality in the biomedical engineering field because of its ability to generate an electrical potential proportional to pressure and temperature, and it is the first time that such a device has been processed by the additive manufacturing process (3D printing). More work needs to be carried out to improve the performance, such as electrical stimulation of the nervous system, thereby extending the purpose of a prosthesis to the area of sensory perception. PMID:26959026

  8. Silencing abnormal wing disc gene of the Asian citrus psyllid, Diaphorina citri disrupts adult wing development and increases nymph mortality.

    PubMed

    El-Shesheny, Ibrahim; Hajeri, Subhas; El-Hawary, Ibrahim; Gowda, Siddarame; Killiny, Nabil

    2013-01-01

    Huanglongbing (HLB) causes considerable economic losses to citrus industries worldwide. Its management depends on controlling of the Asian citrus Psyllid (ACP), the vector of the bacterium, Candidatus Liberibacter asiaticus (CLas), the causal agent of HLB. Silencing genes by RNA interference (RNAi) is a promising tool to explore gene functions as well as control pests. In the current study, abnormal wing disc (awd) gene associated with wing development in insects is used to interfere with the flight of psyllids. Our study showed that transcription of awd is development-dependent and the highest level was found in the last instar (5(th)) of the nymphal stage. Micro-application (topical application) of dsRNA to 5(th) instar of nymphs caused significant nymphal mortality and adult wing-malformation. These adverse effects in ACP were positively correlated with the amounts of dsRNA used. A qRT-PCR analysis confirmed the dsRNA-mediated transcriptional down-regulation of the awd gene. Significant down-regulation was required to induce a wing-malformed phenotype. No effect was found when dsRNA-gfp was used, indicating the specific effect of dsRNA-awd. Our findings suggest a role for awd in ACP wing development and metamorphosis. awd could serve as a potential target for insect management either via direct application of dsRNA or by producing transgenic plants expressing dsRNA-awd. These strategies will help to mitigate HLB by controlling ACP.

  9. Electron beam irradiation induces abnormal development and the stabilization of p53 protein of American serpentine leafminer, Liriomyza trifolii (Burgess)

    NASA Astrophysics Data System (ADS)

    Koo, Hyun-Na; Yun, Seung-Hwan; Yoon, Changmann; Kim, Gil-Hah

    2012-01-01

    The American serpentine leafminer fly, Liriomyza trifolii (Burgess), is one of the most destructive polyphagous pests worldwide. In this study, we determined electron beam doses for inhibition of normal development of the leaf miner and investigated the effect of electron beam irradiation on DNA damage and p53 stability. Eggs (0-24 h old), larvae (2nd instar), puparia (0-24 h old after pupariation) and adults (24 h after emergence) were irradiated with increasing doses of electron beam irradiation (six levels between 30 and 200 Gy). At 150 Gy, the number of adults that developed from irradiated eggs, larvae and puparia was lower than in the untreated control. Fecundity and egg hatchability decreased depending on the doses applied. Reciprocal crosses between irradiated and unirradiated flies demonstrated that males were more radiotolerant than females. Adult longevity was not affected in all stages. The levels of DNA damage in L. trifolii adults were evaluated using the alkaline comet assay. Our results indicate that electron beam irradiation increased levels of DNA damage in a dose-dependent manner. Moreover, low doses of electron beam irradiation led to the rapid appearance of p53 protein within 6 h; however, it decreased after exposure to high doses (150 Gy and 200 Gy). These results suggest that electron beam irradiation induced not only abnormal development and reproduction but also p53 stability caused by DNA damage in L. trifolii. We conclude that a minimum dose of 150 Gy should be sufficient for female sterilization of L. trifolii.

  10. Can Signal Abnormalities Detected with MR Imaging in Knee Articular Cartilage Be Used to Predict Development of Morphologic Cartilage Defects? 48-Month Data from the Osteoarthritis Initiative.

    PubMed

    Schwaiger, Benedikt J; Gersing, Alexandra S; Mbapte Wamba, John; Nevitt, Michael C; McCulloch, Charles E; Link, Thomas M

    2016-10-01

    Purpose To determine the incidence with which morphologic articular cartilage defects develop over 48 months in cartilage with signal abnormalities at baseline magnetic resonance (MR) imaging in comparison with the incidence in articular cartilage without signal abnormalities at baseline. Materials and Methods The institutional review boards of all participating centers approved this HIPAA-compliant study. Right knees of 90 subjects from the Osteoarthritis Initiative (mean age, 55 years ± 8 [standard deviation]; 51% women) with cartilage signal abnormalities but without morphologic cartilage defects at 3.0-T MR imaging and without radiographic osteoarthritis (Kellgren-Lawrence score, 0-1) were frequency matched for age, sex, Kellgren-Lawrence score, and body mass index with right knees in 90 subjects without any signal abnormalities or morphologic defects in the articular cartilage (mean age, 54 years ± 5; 51% women). Individual signal abnormalities (n = 126) on intermediate-weighted fast spin-echo MR images were categorized into four subgrades: subgrade A, hypointense; subgrade B, inhomogeneous; subgrade C, hyperintense; and subgrade D, hyperintense with swelling. The development of morphologic articular cartilage defects (Whole-Organ MR Imaging Score ≥2) at 48 months was analyzed on a compartment level and was compared between groups by using generalized estimating equation logistic regression models. Results Cartilage signal abnormalities were more frequent in the patellofemoral joint than in the tibiofemoral joint (59.5% vs 39.5%). Subgrade A was seen more frequently than were subgrades C and D (36% vs 22%). Incidence of morphologic cartilage defects at 48 months was 57% in cartilage with baseline signal abnormalities, while only 4% of compartments without baseline signal abnormalities developed morphologic defects at 48 months (all compartments combined and each compartment separately, P < .01). The development of morphologic defects was not significantly

  11. Morphological abnormalities during early-life development of the estuarine mummichog, Fundulus heteroclitus, as an indicator of androgenic and anti-androgenic endocrine disruption.

    PubMed

    Boudreau, Monica; Courtenay, Simon C; Maclatchy, Deborah L; Bérubé, Céline H; Hewitt, L Mark; Van Der Kraak, Glen J

    2005-03-01

    We tested the hypothesis that gross morphological abnormalities are a sensitive indicator of exposure to waterborne androgenic and anti-androgenic compounds during embryonic, larval and juvenile stages of development in the common estuarine killifish, the mummichog (Fundulus heteroclitus; Pisces: Cyprinodontidae). Static exposures with daily renewal were carried out with 10-100,000 ng/L of the androgen agonist, 17alpha-methyltestosterone (MT), or the androgen antagonist, cyproterone acetate (CA), for 60 days post-fertilization (PF) in duplicate exposures. Measured concentrations were 78.4-155.8% of nominal concentrations for MT and 13.5-168.1% for CA. No dose-related or consistent effects of MT or CA were observed before hatch. In 60 days PF juveniles, incidence of skeletal abnormalities (scoliosis, lordosis, head, facial and fin), soft tissue abnormality (anal swelling) and hemorrhaging were significantly increased by MT but only at high concentrations (> or =1000 ng/L). The 10,000 and 100,000 ng/L concentrations of MT produced a wider range of abnormalities than 1000ng/L. Over 90% of fish exposed to 10,000 or 100,000 ng/L were abnormal with an average of over 3.5 abnormalities per fish. CA did not increase the incidence of any type of abnormality. Survival of juveniles to the end of the exposure was reduced by MT at concentrations of 1000 ng/L and greater in the first experiment and at concentrations of 10,000 ng/L and greater in the second experiment. Juvenile length was reduced by high concentrations of MT (> or =10,000 ng/L) in the first experiment and by most concentrations in the second experiment. We conclude that morphological abnormalities in early-life stages of mummichogs are not a sensitive indicator of exposure to androgenic or anti-androgenic waterborne EDSs at environmentally relevant concentrations.

  12. [Genetic nature of abnormal larval development in the progeny of l(1)ts403(sbr10) females of Drosophila melanogaster].

    PubMed

    Pugacheva, O M; Mamon, L A

    2005-01-01

    In Drosophila melanogaster the small bristles (sbr) gene is vital and evolutionary conservative and controls nuclear export of mRNA. Sbr mutant alleles had a broad pleiotropic effect. High frequency of abnormal larva dying (up to 18 %) at the first instar stage in progeny of heat shock (37 degrees C, 1 h) treated mutant females is one of the most interesting l(l)ts403(sbr10) allele effects. Abnormal larvae display characteristic phenotype that involves the Malpighian tubules defect. Using interphase FISH method (fluorescence in situ hybridization), we showed that abnormal larvae had monosomy on chromosomes 2 and 3. DNA content in neuroblast interphase nuclei of abnormal larvae is 2.1 times less than in normal larvae. We suggest that abnormal larvae could be full or mosaic haploids that appeared as a result of maternal genome loss during fertilization or the mitotic division. Larvae with the same abnormalities appear in a progeny of females with different genotypes mating with males carrying compound chromosomes 2 or 3. FISH analysis showed that such larvae had monosomy only on a chromosome that is compound in paternal strain. Thus, monosomy on large autosomes may cause aspecial phenotype of abnormal larvae in D. melanogaster.

  13. Blocking Endogenous Leukemia Inhibitory Factor During Placental Development in Mice Leads to Abnormal Placentation and Pregnancy Loss

    PubMed Central

    Winship, Amy; Correia, Jeanne; Krishnan, Tara; Menkhorst, Ellen; Cuman, Carly; Zhang, Jian-Guo; Nicola, Nicos A.; Dimitriadis, Evdokia

    2015-01-01

    The placenta forms the interface between the maternal and fetal circulation and is critical for the establishment of a healthy pregnancy. Specialized trophoblast cells derived from the embryonic trophectoderm play a pivotal role in the establishment of the placenta. Leukemia inhibitory factor (LIF) is one of the predominant cytokines present in the placenta during early pregnancy. LIF has been shown to regulate trophoblast adhesion and invasion in vitro, however its precise role in vivo is unknown. We hypothesized that LIF would be required for normal placental development in mice. LIF and LIFRα were immunolocalized to placental trophoblasts and fetal vessels in mouse implantation sites during mid-gestation. Temporally blocking LIF action during specific periods of placental development via intraperitoneal administration of our specific LIFRα antagonist, PEGLA, resulted in abnormal placental trophoblast and vascular morphology and reduced activated STAT3 but not ERK. Numerous genes regulating angiogenesis and oxidative stress were altered in the placenta in response to LIF inhibition. Pregnancy viability was also significantly compromised in PEGLA treated mice. Our data suggest that LIF plays an important role in placentation in vivo and the maintenance of healthy pregnancy. PMID:26272398

  14. Cells, molecules and morphogenesis: The making of the vertebrate ear

    PubMed Central

    Fritzsch, Bernd; Pauley, Sarah; Beisel, Kirk W.

    2014-01-01

    The development and evolution of mechanosensory cells and the vertebrate ear is reviewed with an emphasis on delineating the cellular, molecular and developmental basis of these changes. Outgroup comparisons suggests that mechanosensory cells are ancient features of multicellular organisms. Molecular evidence suggests that key genes involved in mechanosensory cell function and development are also conserved among metazoans. The divergent morphology of mechanosensory cells across phyla is interpreted here as ‘deep molecular homology’ that was in parallel shaped into different forms in each lineage. The vertebrate mechanosensory hair cell and its associated neuron are interpreted as uniquely derived features of vertebrates. It is proposed that the vertebrate otic placode presents a unique embryonic adaptation in which the diffusely distributed ancestral mechanosensory cells became concentrated to generate a large neurosensory precursor population. Morphogenesis of the inner ear is reviewed and shown to depend on genes expressed in and around the hindbrain that interact with the otic placode to define boundaries and polarities. These patterning genes affect downstream genes needed to maintain proliferation and to execute ear morphogenesis. We propose that fibroblast growth factors (FGFs) and their receptors (FGFRs) are a crucial central node to translate patterning into the complex morphology of the vertebrate ear. Unfortunately, the FGF and FGFR genes have not been fully analyzed in the many mutants with morphogenetic ear defects described thus far. Likewise, little information exists on the ear histogenesis and neurogenesis in many mutants. Nevertheless, a molecular mechanism is now emerging for the formation of the horizontal canal, an evolutionary novelty of the gnathostome ear. The existing general module mediating vertical canal growth and morphogenesis was modified by two sets of new genes: one set responsible for horizontal canal morphogenesis and another

  15. Abnormal immune system development and function in schizophrenia helps reconcile diverse findings and suggests new treatment and prevention strategies.

    PubMed

    Anders, Sherry; Kinney, Dennis K

    2015-08-18

    Extensive research implicates disturbed immune function and development in the etiology and pathology of schizophrenia. In addition to reviewing evidence for immunological factors in schizophrenia, this paper discusses how an emerging model of atypical immune function and development helps explain a wide variety of well-established - but puzzling - findings about schizophrenia. A number of theorists have presented hypotheses that early immune system programming, disrupted by pre- and perinatal adversity, often combines with abnormal brain development to produce schizophrenia. The present paper focuses on the hypothesis that disruption of early immune system development produces a latent immune vulnerability that manifests more fully after puberty, when changes in immune function and the thymus leave individuals more susceptible to infections and immune dysfunctions that contribute to schizophrenia. Complementing neurodevelopmental models, this hypothesis integrates findings on many contributing factors to schizophrenia, including prenatal adversity, genes, climate, migration, infections, and stress, among others. It helps explain, for example, why (a) schizophrenia onset is typically delayed until years after prenatal adversity, (b) individual risk factors alone often do not lead to schizophrenia, and (c) schizophrenia prevalence rates actually tend to be higher in economically advantaged countries. Here we discuss how the hypothesis explains 10 key findings, and suggests new, potentially highly cost-effective, strategies for treatment and prevention of schizophrenia. Moreover, while most human research linking immune factors to schizophrenia has been correlational, these strategies provide ethical ways to experimentally test in humans theories about immune function and schizophrenia. This article is part of a Special Issue entitled SI: Neuroimmunology in Health And Disease.

  16. Characterization of the skeletal fusion with sterility (sks) mouse showing axial skeleton abnormalities caused by defects of embryonic skeletal development.

    PubMed

    Akiyama, Kouyou; Katayama, Kentaro; Tsuji, Takehito; Kunieda, Tetsuo

    2014-01-01

    The development of the axial skeleton is a complex process, consisting of segmentation and differentiation of somites and ossification of the vertebrae. The autosomal recessive skeletal fusion with sterility (sks) mutation of the mouse causes skeletal malformations due to fusion of the vertebrae and ribs, but the underlying defects of vertebral formation during embryonic development have not yet been elucidated. For the present study, we examined the skeletal phenotypes of sks/sks mice during embryonic development and the chromosomal localization of the sks locus. Multiple defects of the axial skeleton, including fusion of vertebrae and fusion and bifurcation of ribs, were observed in adult and neonatal sks/sks mice. In addition, we also found polydactyly and delayed skull ossification in the sks/sks mice. Morphological defects, including disorganized vertebral arches and fusions and bifurcations of the axial skeletal elements, were observed during embryonic development at embryonic day 12.5 (E12.5) and E14.5. However, no morphological abnormality was observed at E11.5, indicating that defects of the axial skeleton are caused by malformation of the cartilaginous vertebra and ribs at an early developmental stage after formation and segmentation of the somites. By linkage analysis, the sks locus was mapped to an 8-Mb region of chromosome 4 between D4Mit331 and D4Mit199. Since no gene has already been identified as a cause of malformation of the vertebra and ribs in this region, the gene responsible for sks is suggested to be a novel gene essential for the cartilaginous vertebra and ribs.

  17. Multiple Osteomas in Middle Ear

    PubMed Central

    Li, Yongxin; Li, Qiuhuan; Gong, Shusheng; Liu, Honggang; Yu, Zilong; Zhang, Luo

    2012-01-01

    Since the first description of middle ear osteomas by Thomas in 1964, only few reports were published within the English literatures (Greinwalid et al., 1998; Shimizu et al., 2003; Cho et al., 2005; and Jang et al., 2009), and only one case of the multiple osteomas in middle ear was described by Kim et al., 2006, which arose from the promontory, lateral semicircular canal, and epitympanum. Here we describe a patient with multiple middle ear osteomas arising from the promontory, incus, Eustachian tube, and bony semicanal of tensor tympani muscle. This patient also contracted the chronic otitis media in the ipsilateral ear. The osteomas were successfully removed by performing type III tympanoplasty in one stage. PMID:22928138

  18. Keloid above the ear (image)

    MedlinePlus

    Keloids are overgrowths of scar tissue that follow skin injuries. Keloids may appear after such minor trauma as ear piercing. Dark skinned individuals tend to form keloids more readily than lighter skinned individuals.

  19. What Is an Ear Infection?

    MedlinePlus

    ... Quizzes Kids' Dictionary of Medical Words En Español What Other Kids Are Reading Back-to-School Butterflies? ... Got Homework? Here's Help White House Lunch Recipes What Is an Ear Infection? KidsHealth > For Kids > What ...

  20. Avoiding Infection After Ear Piercing

    MedlinePlus

    ... Health Issues Conditions Abdominal ADHD Allergies & Asthma Autism Cancer Chest & Lungs Chronic Conditions Cleft & Craniofacial Developmental Disabilities Ear Nose & Throat Emotional Problems Eyes Fever From Insects or Animals Genitals and Urinary Tract Glands & Growth ...

  1. Middle Ear Infections (For Parents)

    MedlinePlus

    ... in the first 2 to 4 years of life for several reasons: Their eustachian tubes are shorter and more horizontal than those of adults, which lets bacteria and viruses find their way into the middle ear more ...

  2. Ear identification: a pilot study.

    PubMed

    Cameriere, Roberto; DeAngelis, Danilo; Ferrante, Luigi

    2011-07-01

    Although several papers have recently been devoted to establishing the validity of identification using the ear, this part of the human body still remains underexploited in forensic science. The perfect overlap of two images of the same ear is not really possible, but photographs of the ears as a reliable means of inferring the identity of an individual are poorly treated in the literature. In this study, we illustrate a simple, reproducible method, which divides the photograph of an ear into four parts-helix, antihelix, concha, and lobe-by means of a suitable grid of four straight lines. Although the division does not follow exact anatomical features, their edges do join anatomical points which are more easily identifiable. Measurement of certain areas of these parts can be combined to produce a code allowing personal identification. This method produces false-positive identifications of <0.2%. Last, the repeatability and reproducibility aspects of the method are tested.

  3. "Swimmer's Ear" (Otitis Externa) Prevention

    MedlinePlus

    ... Work: Healthy Swimming Policy & Recommendations Fast Facts Healthy Water Sites Healthy Water Drinking Water Healthy Swimming Global ... you requested has moved to Ear Infections. Healthy Water Sites Healthy Water Drinking Water Healthy Swimming Global ...

  4. 21 CFR 870.2710 - Ear oximeter.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ... 21 Food and Drugs 8 2014-04-01 2014-04-01 false Ear oximeter. 870.2710 Section 870.2710 Food and... CARDIOVASCULAR DEVICES Cardiovascular Monitoring Devices § 870.2710 Ear oximeter. (a) Identification. An ear... ear. The amount of reflected or scattered light as indicated by this device is used to measure...

  5. 21 CFR 870.2710 - Ear oximeter.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 21 Food and Drugs 8 2011-04-01 2011-04-01 false Ear oximeter. 870.2710 Section 870.2710 Food and... CARDIOVASCULAR DEVICES Cardiovascular Monitoring Devices § 870.2710 Ear oximeter. (a) Identification. An ear... ear. The amount of reflected or scattered light as indicated by this device is used to measure...

  6. 21 CFR 870.2710 - Ear oximeter.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ... 21 Food and Drugs 8 2013-04-01 2013-04-01 false Ear oximeter. 870.2710 Section 870.2710 Food and... CARDIOVASCULAR DEVICES Cardiovascular Monitoring Devices § 870.2710 Ear oximeter. (a) Identification. An ear... ear. The amount of reflected or scattered light as indicated by this device is used to measure...

  7. 21 CFR 870.2710 - Ear oximeter.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ... 21 Food and Drugs 8 2012-04-01 2012-04-01 false Ear oximeter. 870.2710 Section 870.2710 Food and... CARDIOVASCULAR DEVICES Cardiovascular Monitoring Devices § 870.2710 Ear oximeter. (a) Identification. An ear... ear. The amount of reflected or scattered light as indicated by this device is used to measure...

  8. Inhibition of tyrosine kinase receptors by SU6668 promotes abnormal stromal development at the periphery of carcinomas

    PubMed Central

    Farace, P; Galiè, M; Merigo, F; Daducci, A; Calderan, L; Nicolato, E; Degrassi, A; Pesenti, E; Sbarbati, A; Marzola, P

    2009-01-01

    Dynamic contrast-enhanced (albumin-Gd-DTPA) magnetic resonance imaging, performed during 2 weeks of daily administration of an inhibitor of tyrosine kinase receptors (SU6668) in an HT-29 colon carcinoma model, revealed the onset of a hyper-enhancing rim, not observed in untreated tumours. To account for tissue heterogeneity in the quantitative analysis, we segmented tumours into three subunits automatically identified by cluster analysis of the enhancement curves using a k-means algorithm. Transendothelial permeability (Kps) and fractional plasma volume (fPV) were calculated in each subunit. An avascular and necrotic region, an intermediate zone and a well-vascularised periphery were reliably identified. During untreated tumour growth, the identified sub-regions did not substantially change their enhancement pattern. Treatment with SU6668 induced major changes at tumour periphery where a significant increase of Kps and fPV was observed with respect to control tumours. Histology revealed a sub-capsular layer composed of hyper-dense viable tumour cells in the periphery of untreated tumours. The rim of viable neoplastic cells was reduced in treated tumours, and replaced by loose connective tissue characterised by numerous vessels, which explains the observed hyper-enhancement. The present data show a peripheral abnormal development of cancer-associated stroma, indicative of an adaptive response to anti-angiogenic treatment. PMID:19384298

  9. Potential Adverse Effects of Prolonged Sevoflurane Exposure on Developing Monkey Brain: From Abnormal Lipid Metabolism to Neuronal Damage.

    PubMed

    Liu, Fang; Rainosek, Shuo W; Frisch-Daiello, Jessica L; Patterson, Tucker A; Paule, Merle G; Slikker, William; Wang, Cheng; Han, Xianlin

    2015-10-01

    Sevoflurane is a volatile anesthetic that has been widely used in general anesthesia, yet its safety in pediatric use is a public concern. This study sought to evaluate whether prolonged exposure of infant monkeys to a clinically relevant concentration of sevoflurane is associated with any adverse effects on the developing brain. Infant monkeys were exposed to 2.5% sevoflurane for 9 h, and frontal cortical tissues were harvested for DNA microarray, lipidomics, Luminex protein, and histological assays. DNA microarray analysis showed that sevoflurane exposure resulted in a broad identification of differentially expressed genes (DEGs) in the monkey brain. In general, these genes were associated with nervous system development, function, and neural cell viability. Notably, a number of DEGs were closely related to lipid metabolism. Lipidomic analysis demonstrated that critical lipid components, (eg, phosphatidylethanolamine, phosphatidylserine, and phosphatidylglycerol) were significantly downregulated by prolonged exposure of sevoflurane. Luminex protein analysis indicated abnormal levels of cytokines in sevoflurane-exposed brains. Consistently, Fluoro-Jade C staining revealed more degenerating neurons after sevoflurane exposure. These data demonstrate that a clinically relevant concentration of sevoflurane (2.5%) is capable of inducing and maintaining an effective surgical plane of anesthesia in the developing nonhuman primate and that a prolonged exposure of 9 h resulted in profound changes in gene expression, cytokine levels, lipid metabolism, and subsequently, neuronal damage. Generally, sevoflurane-induced neuronal damage was also associated with changes in lipid content, composition, or both; and specific lipid changes could provide insights into the molecular mechanism(s) underlying anesthetic-induced neurotoxicity and may be sensitive biomarkers for the early detection of anesthetic-induced neuronal damage.

  10. Multiple Renal Cyst Development but Not Situs Abnormalities in Transgenic RNAi Mice against Inv::GFP Rescue Gene

    PubMed Central

    Kamijho, Yuki; Shiozaki, Yayoi; Sakurai, Eiki; Hanaoka, Kazunori; Watanabe, Daisuke

    2014-01-01

    In this study we generated RNA interference (RNAi)-mediated gene knockdown transgenic mice (transgenic RNAi mice) against the functional Inv gene. Inv mutant mice show consistently reversed internal organs (situs inversus), multiple renal cysts and neonatal lethality. The Inv::GFP-rescue mice, which introduced the Inv::GFP fusion gene, can rescue inv mutant mice phenotypes. This indicates that the Inv::GFP gene is functional in vivo. To analyze the physiological functions of the Inv gene, and to demonstrate the availability of transgenic RNAi mice, we introduced a short hairpin RNA expression vector against GFP mRNA into Inv::GFP-rescue mice and analyzed the gene silencing effects and Inv functions by examining phenotypes. Transgenic RNAi mice with the Inv::GFP-rescue gene (Inv-KD mice) down-regulated Inv::GFP fusion protein and showed hypomorphic phenotypes of inv mutant mice, such as renal cyst development, but not situs abnormalities or postnatal lethality. This indicates that shRNAi-mediated gene silencing systems that target the tag sequence of the fusion gene work properly in vivo, and suggests that a relatively high level of Inv protein is required for kidney development in contrast to left/right axis determination. Inv::GFP protein was significantly down-regulated in the germ cells of Inv-KD mice testis compared with somatic cells, suggesting the existence of a testicular germ cell-specific enhanced RNAi system that regulates germ cell development. The Inv-KD mouse is useful for studying Inv gene functions in adult tissue that are unable to be analyzed in inv mutant mice showing postnatal lethality. In addition, the shRNA-based gene silencing system against the tag sequence of the fusion gene can be utilized as a new technique to regulate gene expression in either in vitro or in vivo experiments. PMID:24586938

  11. Genetically induced abnormal cranial development in human trisomy 18 with holoprosencephaly: comparisons with the normal tempo of osteogenic-neural development.

    PubMed

    Reid, Shaina N; Ziermann, Janine M; Gondré-Lewis, Marjorie C

    2015-07-01

    Craniofacial malformations are common congenital defects caused by failed midline inductive signals. These midline defects are associated with exposure of the fetus to exogenous teratogens and with inborn genetic errors such as those found in Down, Patau, Edwards' and Smith-Lemli-Opitz syndromes. Yet, there are no studies that analyze contributions of synchronous neurocranial and neural development in these disorders. Here we present the first in-depth analysis of malformations of the basicranium of a holoprosencephalic (HPE) trisomy 18 (T18; Edwards' syndrome) fetus with synophthalmic cyclopia and alobar HPE. With a combination of traditional gross dissection and state-of-the-art computed tomography, we demonstrate the deleterious effects of T18 caused by a translocation at 18p11.31. Bony features included a single developmentally unseparated frontal bone, and complete dual absence of the anterior cranial fossa and ethmoid bone. From a superior view with the calvarium plates removed, there was direct visual access to the orbital foramen and hard palate. Both the eyes and the pituitary gland, normally protected by bony structures, were exposed in the cranial cavity and in direct contact with the brain. The middle cranial fossa was shifted anteriorly, and foramina were either missing or displaced to an abnormal location due to the absence or misplacement of its respective cranial nerve (CN). When CN development was conserved in its induction and placement, the respective foramen developed in its normal location albeit with abnormal gross anatomical features, as seen in the facial nerve (CNVII) and the internal acoustic meatus. More anteriorly localized CNs and their foramina were absent or heavily disrupted compared with posterior ones. The severe malformations exhibited in the cranial fossae, orbital region, pituitary gland and sella turcica highlight the crucial involvement of transcription factors such as TGIF, which is located on chromosome 18 and contributes

  12. Epiphyseal abnormalities, trabecular bone loss and articular chondrocyte hypertrophy develop in the long bones of postnatal Ext1-deficient mice.

    PubMed

    Sgariglia, Federica; Candela, Maria Elena; Huegel, Julianne; Jacenko, Olena; Koyama, Eiki; Yamaguchi, Yu; Pacifici, Maurizio; Enomoto-Iwamoto, Motomi

    2013-11-01

    Long bones are integral components of the limb skeleton. Recent studies have indicated that embryonic long bone development is altered by mutations in Ext genes and consequent heparan sulfate (HS) deficiency, possibly due to changes in activity and distribution of HS-binding/growth plate-associated signaling proteins. Here we asked whether Ext function is continuously required after birth to sustain growth plate function and long bone growth and organization. Compound transgenic Ext1(f/f);Col2CreERT mice were injected with tamoxifen at postnatal day 5 (P5) to ablate Ext1 in cartilage and monitored over time. The Ext1-deficient mice exhibited growth retardation already by 2weeks post-injection, as did their long bones. Mutant growth plates displayed a severe disorganization of chondrocyte columnar organization, a shortened hypertrophic zone with low expression of collagen X and MMP-13, and reduced primary spongiosa accompanied, however, by increased numbers of TRAP-positive osteoclasts at the chondro-osseous border. The mutant epiphyses were abnormal as well. Formation of a secondary ossification center was significantly delayed but interestingly, hypertrophic-like chondrocytes emerged within articular cartilage, similar to those often seen in osteoarthritic joints. Indeed, the cells displayed a large size and round shape, expressed collagen X and MMP-13 and were surrounded by an abundant Perlecan-rich pericellular matrix not seen in control articular chondrocytes. In addition, ectopic cartilaginous outgrowths developed on the lateral side of mutant growth plates over time that resembled exostotic characteristic of children with Hereditary Multiple Exostoses, a syndrome caused by Ext mutations and HS deficiency. In sum, the data do show that Ext1 is continuously required for postnatal growth and organization of long bones as well as their adjacent joints. Ext1 deficiency elicits defects that can occur in human skeletal conditions including trabecular bone loss

  13. Absence of the lysophosphatidic acid receptor LPA1 results in abnormal bone development and decreased bone mass.

    PubMed

    Gennero, Isabelle; Laurencin-Dalicieux, Sara; Conte-Auriol, Françoise; Briand-Mésange, Fabienne; Laurencin, Danielle; Rue, Jackie; Beton, Nicolas; Malet, Nicole; Mus, Marianne; Tokumura, Akira; Bourin, Philippe; Vico, Laurence; Brunel, Gérard; Oreffo, Richard O C; Chun, Jerold; Salles, Jean Pierre

    2011-09-01

    Lysophosphatidic acid (LPA) is a lipid mediator that acts in paracrine systems via interaction with a subset of G protein-coupled receptors (GPCRs). LPA promotes cell growth and differentiation, and has been shown to be implicated in a variety of developmental and pathophysiological processes. At least 6 LPA GPCRs have been identified to date: LPA1-LPA6. Several studies have suggested that local production of LPA by tissues and cells contributes to paracrine regulation, and a complex interplay between LPA and its receptors, LPA1 and LPA4, is believed to be involved in the regulation of bone cell activity. In particular, LPA1 may activate both osteoblasts and osteoclasts. However, its role has not as yet been examined with regard to the overall status of bone in vivo. We attempted to clarify this role by defining the bone phenotype of LPA1((-/-)) mice. These mice demonstrated significant bone defects and low bone mass, indicating that LPA1 plays an important role in osteogenesis. The LPA1((-/-)) mice also presented growth and sternal and costal abnormalities, which highlights the specific roles of LPA1 during bone development. Microcomputed tomography and histological analysis demonstrated osteoporosis in the trabecular and cortical bone of LPA1((-/-)) mice. Finally, bone marrow mesenchymal progenitors from these mice displayed decreased osteoblastic differentiation. These results suggest that LPA1 strongly influences bone development both qualitatively and quantitatively and that, in vivo, its absence results in decreased osteogenesis with no clear modification of osteoclasis. They open perspectives for a better understanding of the role of the LPA/LPA1 paracrine pathway in bone pathophysiology.

  14. Absence of the lysophosphatidic acid receptor LPA1 results in abnormal bone development and decreased bone mass☆,☆☆

    PubMed Central

    Gennero, Isabelle; Laurencin-Dalicieux, Sara; Conte-Auriol, Françoise; Briand-Mésange, Fabienne; Laurencin, Danielle; Rue, Jackie; Beton, Nicolas; Malet, Nicole; Mus, Marianne; Tokumura, Akira; Bourin, Philippe; Vico, Laurence; Brunel, Gérard; Oreffo, Richard O. C.; Chun, Jerold; Salles, Jean Pierre

    2013-01-01

    Lysophosphatidic acid (LPA) is a lipid mediator that acts in paracrine systems via interaction with a subset of G protein-coupled receptors (GPCRs). LPA promotes cell growth and differentiation, and has been shown to be implicated in a variety of developmental and pathophysiological processes. At least 6 LPA GPCRs have been identified to date: LPA1–LPA6. Several studies have suggested that local production of LPA by tissues and cells contributes to paracrine regulation, and a complex interplay between LPA and its receptors, LPA1 and LPA4, is believed to be involved in the regulation of bone cell activity. In particular, LPA1may activate both osteoblasts and osteoclasts. However, its role has not as yet been examined with regard to the overall status of bone in vivo. We attempted to clarify this role by defining the bone phenotype of LPA1(−/−) mice. These mice demonstrated significant bone defects and low bone mass, indicating that LPA1 plays an important role in osteogenesis. The LPA1(−/−) mice also presented growth and sternal and costal abnormalities, which highlights the specific roles of LPA1 during bone development. Microcomputed tomography and histological analysis demonstrated osteoporosis in the trabecular and cortical bone of LPA1(−/−) mice. Finally, bone marrow mesenchymal progenitors from these mice displayed decreased osteoblastic differentiation. These results suggest that LPA1 strongly influences bone development both qualitatively and quantitatively and that, in vivo, its absence results in decreased osteogenesis with no clear modification of osteoclasis. They open perspectives for a better understanding of the role of the LPA/LPA1 paracrine pathway in bone pathophysiology. PMID:21569876

  15. The trajectory of gray matter development in Broca’s area is abnormal in people who stutter

    PubMed Central

    Beal, Deryk S.; Lerch, Jason P.; Cameron, Brodie; Henderson, Rhaeling; Gracco, Vincent L.; De Nil, Luc F.

    2015-01-01

    The acquisition and mastery of speech-motor control requires years of practice spanning the course of development. People who stutter often perform poorly on speech-motor tasks thereby calling into question their ability to establish the stable neural motor programs required for masterful speech-motor control. There is evidence to support the assertion that these neural motor programs are represented in the posterior part of Broca’s area, specifically the left pars opercularis. Consequently, various theories of stuttering causation posit that the disorder is related to a breakdown in the formation of the neural motor programs for speech early in development and that this breakdown is maintained throughout life. To date, no study has examined the potential neurodevelopmental signatures of the disorder across pediatric and adult populations. The current study aimed to fill this gap in our knowledge. We hypothesized that the developmental trajectory of cortical thickness in people who stutter would differ across the lifespan in the left pars opercularis relative to a group of control participants. We collected structural magnetic resonance images from 116 males (55 people who stutter) ranging in age from 6 to 48 years old. Differences in cortical thickness across ages and between patients and controls were investigated in 30 brain regions previously implicated in speech-motor control. An interaction between age and group was found for the left pars opercularis only. In people who stutter, the pars opercularis did not demonstrate the typical maturational pattern of gradual gray matter thinning with age across the lifespan that we observed in control participants. In contrast, the developmental trajectory of gray matter thickness in other regions of interest within the neural network for speech-motor control was similar for both groups. Our findings indicate that the developmental trajectory of gray matter in left pars opercularis is abnormal in people who stutter

  16. SMAX1-LIKE7 Signals from the Nucleus to Regulate Shoot Development in Arabidopsis via Partially EAR Motif-Independent Mechanisms.

    PubMed

    Liang, Yueyang; Ward, Sally; Li, Ping; Bennett, Tom; Leyser, Ottoline

    2016-07-01

    Strigolactones (SLs) are hormonal signals that regulate multiple aspects of shoot architecture, including shoot branching. Like many plant hormonal signaling systems, SLs act by promoting ubiquitination of target proteins and their subsequent proteasome-mediated degradation. Recently, SMXL6, SMXL7, and SMXL8, members of the SMAX1-LIKE (SMXL) family of chaperonin-like proteins, have been identified as proteolytic targets of SL signaling in Arabidopsis thaliana However, the mechanisms by which these proteins regulate downstream events remain largely unclear. Here, we show that SMXL7 functions in the nucleus, as does the SL receptor, DWARF14 (D14). We show that nucleus-localized D14 can physically interact with both SMXL7 and the MAX2 F-box protein in a SL-dependent manner and that disruption of specific conserved domains in SMXL7 affects its localization, SL-induced degradation, and activity. By expressing and overexpressing these SMXL7 protein variants, we show that shoot tissues are broadly sensitive to SMXL7 activity, but degradation normally buffers the effect of increasing SMXL7 expression. SMXL7 contains a well-conserved EAR (ETHYLENE-RESPONSE FACTOR Amphiphilic Repression) motif, which contributes to, but is not essential for, SMXL7 functionality. Intriguingly, different developmental processes show differential sensitivity to the loss of the EAR motif, raising the possibility that there may be several distinct mechanisms at play downstream of SMXL7.

  17. SMAX1-LIKE7 Signals from the Nucleus to Regulate Shoot Development in Arabidopsis via Partially EAR Motif-Independent Mechanisms.

    PubMed

    Liang, Yueyang; Ward, Sally; Li, Ping; Bennett, Tom; Leyser, Ottoline

    2016-07-01

    Strigolactones (SLs) are hormonal signals that regulate multiple aspects of shoot architecture, including shoot branching. Like many plant hormonal signaling systems, SLs act by promoting ubiquitination of target proteins and their subsequent proteasome-mediated degradation. Recently, SMXL6, SMXL7, and SMXL8, members of the SMAX1-LIKE (SMXL) family of chaperonin-like proteins, have been identified as proteolytic targets of SL signaling in Arabidopsis thaliana However, the mechanisms by which these proteins regulate downstream events remain largely unclear. Here, we show that SMXL7 functions in the nucleus, as does the SL receptor, DWARF14 (D14). We show that nucleus-localized D14 can physically interact with both SMXL7 and the MAX2 F-box protein in a SL-dependent manner and that disruption of specific conserved domains in SMXL7 affects its localization, SL-induced degradation, and activity. By expressing and overexpressing these SMXL7 protein variants, we show that shoot tissues are broadly sensitive to SMXL7 activity, but degradation normally buffers the effect of increasing SMXL7 expression. SMXL7 contains a well-conserved EAR (ETHYLENE-RESPONSE FACTOR Amphiphilic Repression) motif, which contributes to, but is not essential for, SMXL7 functionality. Intriguingly, different developmental processes show differential sensitivity to the loss of the EAR motif, raising the possibility that there may be several distinct mechanisms at play downstream of SMXL7. PMID:27317673

  18. SMAX1-LIKE7 Signals from the Nucleus to Regulate Shoot Development in Arabidopsis via Partially EAR Motif-Independent Mechanisms[OPEN

    PubMed Central

    Li, Ping

    2016-01-01

    Strigolactones (SLs) are hormonal signals that regulate multiple aspects of shoot architecture, including shoot branching. Like many plant hormonal signaling systems, SLs act by promoting ubiquitination of target proteins and their subsequent proteasome-mediated degradation. Recently, SMXL6, SMXL7, and SMXL8, members of the SMAX1-LIKE (SMXL) family of chaperonin-like proteins, have been identified as proteolytic targets of SL signaling in Arabidopsis thaliana. However, the mechanisms by which these proteins regulate downstream events remain largely unclear. Here, we show that SMXL7 functions in the nucleus, as does the SL receptor, DWARF14 (D14). We show that nucleus-localized D14 can physically interact with both SMXL7 and the MAX2 F-box protein in a SL-dependent manner and that disruption of specific conserved domains in SMXL7 affects its localization, SL-induced degradation, and activity. By expressing and overexpressing these SMXL7 protein variants, we show that shoot tissues are broadly sensitive to SMXL7 activity, but degradation normally buffers the effect of increasing SMXL7 expression. SMXL7 contains a well-conserved EAR (ETHYLENE-RESPONSE FACTOR Amphiphilic Repression) motif, which contributes to, but is not essential for, SMXL7 functionality. Intriguingly, different developmental processes show differential sensitivity to the loss of the EAR motif, raising the possibility that there may be several distinct mechanisms at play downstream of SMXL7. PMID:27317673

  19. The Relationship between Personality Dimensions and Resiliency to Environmental Stress in Orange-Winged Amazon Parrots (Amazona amazonica), as Indicated by the Development of Abnormal Behaviors

    PubMed Central

    Cussen, Victoria A.; Mench, Joy A.

    2015-01-01

    Parrots are popular companion animals, but are frequently relinquished because of behavioral problems, including abnormal repetitive behaviors like feather damaging behavior and stereotypy. In addition to contributing to pet relinquishment, these behaviors are important as potential indicators of diminished psychological well-being. While abnormal behaviors are common in captive animals, their presence and/or severity varies between animals of the same species that are experiencing the same environmental conditions. Personality differences could contribute to this observed individual variation, as they are known risk factors for stress sensitivity and affective disorders in humans. The goal of this study was to assess the relationship between personality and the development and severity of abnormal behaviors in captive-bred orange-winged Amazon parrots (Amazona amazonica). We monitored between-individual behavioral differences in enrichment-reared parrots of known personality types before, during, and after enrichment deprivation. We predicted that parrots with higher scores for neurotic-like personality traits would be more susceptible to enrichment deprivation and develop more abnormal behaviors. Our results partially supported this hypothesis, but also showed that distinct personality dimensions were related to different forms of abnormal behavior. While neuroticism-like traits were linked to feather damaging behavior, extraversion-like traits were negatively related to stereotypic behavior. More extraverted birds showed resiliency to environmental stress, developing fewer stereotypies during enrichment deprivation and showing lower levels of these behaviors following re-enrichment. Our data, together with the results of the few studies conducted on other species, suggest that, as in humans, certain personality types render individual animals more susceptible or resilient to environmental stress. Further, this susceptibility/resiliency can have a long

  20. The Relationship between Personality Dimensions and Resiliency to Environmental Stress in Orange-Winged Amazon Parrots (Amazona amazonica), as Indicated by the Development of Abnormal Behaviors.

    PubMed

    Cussen, Victoria A; Mench, Joy A

    2015-01-01

    Parrots are popular companion animals, but are frequently relinquished because of behavioral problems, including abnormal repetitive behaviors like feather damaging behavior and stereotypy. In addition to contributing to pet relinquishment, these behaviors are important as potential indicators of diminished psychological well-being. While abnormal behaviors are common in captive animals, their presence and/or severity varies between animals of the same species that are experiencing the same environmental conditions. Personality differences could contribute to this observed individual variation, as they are known risk factors for stress sensitivity and affective disorders in humans. The goal of this study was to assess the relationship between personality and the development and severity of abnormal behaviors in captive-bred orange-winged Amazon parrots (Amazona amazonica). We monitored between-individual behavioral differences in enrichment-reared parrots of known personality types before, during, and after enrichment deprivation. We predicted that parrots with higher scores for neurotic-like personality traits would be more susceptible to enrichment deprivation and develop more abnormal behaviors. Our results partially supported this hypothesis, but also showed that distinct personality dimensions were related to different forms of abnormal behavior. While neuroticism-like traits were linked to feather damaging behavior, extraversion-like traits were negatively related to stereotypic behavior. More extraverted birds showed resiliency to environmental stress, developing fewer stereotypies during enrichment deprivation and showing lower levels of these behaviors following re-enrichment. Our data, together with the results of the few studies conducted on other species, suggest that, as in humans, certain personality types render individual animals more susceptible or resilient to environmental stress. Further, this susceptibility/resiliency can have a long

  1. Decision making in patients with natural myringostapediopexy: A study of the contralateral ear.

    PubMed

    Schmidt, Viviane Bom; da Costa, Sady Selaimen; Rosito, Letícia Petersen Schmidt; Sperling, Neil; Dias, Rodrigo Gonçalves

    2016-09-01

    Naturally occurring myringostapediopexy frequently results in minimal hearing loss and is asymptomatic. Management decisions in such ears, however, often hinge on an appraisal of evolution toward cholesteatoma. The study of the contralateral ear has been used by our research team to infer the progression of chronic otitis media. This cross-sectional, comparative study describes the clinical findings of the contralateral ear in a series of patients with myringostapediopexy. This study included a historical and current sample of 46 patients divided into a pediatric (≤18 years) and an adult group. Patient distribution according to sex was similar (52.2% male), and 56.5% were adults. Mean conductive hearing loss ranged from 14.1 to 21.2 dB in ears with myringostapediopexy and from 16.0 to 26.6 dB in the contralateral ears according to the frequency assessed. The contralateral ear was normal in only 19.6% of the cases of myringostapediopexy. Central tympanic membrane perforation was found in 6.5% of the cases; perforation-retraction, in 17.4%; moderate or severe retraction, in 28.3%; and cholesteatoma, in 28.3%. The prevalence of cholesteatoma in the contralateral ear in the pediatric and adult groups was not significantly different (p = 0.5; χ(2) test). The presence of significant abnormalities, particularly cholesteatoma, in the contralateral ears suggests a probable unfavorable progression in cases of myringostapediopexy and may influence management decisions. PMID:27657316

  2. A single-blind, randomized study to compare the efficacy of two ear drop preparations ('Audax' and 'Cerumol') in the softening of ear wax.

    PubMed

    Dummer, D S; Sutherland, I A; Murray, J A

    1992-01-01

    A parallel group, single-blind, randomized study was carried out in a general practice to compare the effectiveness and tolerability of two ear drop preparations ('Audax' and 'Cerumol') in the softening of ear wax in 50 adult patients with impacted or hardened ear wax. Assessments were made on entry of the amount, colour and consistency of the ear wax, symptoms, and objective hearing. Patients were then allocated at random to receive one or other preparation and instructed to use the drops, morning and evening, for 4 days after which they were reassessed. Details were recorded of any side-effects or discomfort caused by the study medication and both physician and patients were asked to give their overall opinion of treatment efficacy. Both treatments were shown to be effective in the softening of ear wax and were well tolerated, there being no significant difference between the two groups in these parameters. However, patients who had abnormal hearing before treatment had a significantly greater improvement in objective hearing after treatment with 'Audax' ear drops compared to those patients treated with 'Cerumol' ear drops. There were no between-treatment differences in either either the physician's or patient's overall assessments of effectiveness.

  3. Gene transfer in inner ear cells: a challenging race.

    PubMed

    Sacheli, R; Delacroix, L; Vandenackerveken, P; Nguyen, L; Malgrange, B

    2013-03-01

    Recent advances in human genomics led to the identification of numerous defective genes causing deafness, which represent novel putative therapeutic targets. Future gene-based treatment of deafness resulting from genetic or acquired sensorineural hearing loss may include strategies ranging from gene therapy to antisense delivery. For successful development of gene therapies, a minimal requirement involves the engineering of appropriate gene carrier systems. Transfer of exogenous genetic material into the mammalian inner ear using viral or non-viral vectors has been characterized over the last decade. The nature of inner ear cells targeted, as well as the transgene expression level and duration, are highly dependent on the vector type, the route of administration and the strength of the promoter driving expression. This review summarizes and discusses recent advances in inner ear gene-transfer technologies aimed at examining gene function or identifying new treatment for inner ear disorders.

  4. Over-expression of a grape stilbene synthase gene in tomato induces parthenocarpy and causes abnormal pollen development.

    PubMed

    Ingrosso, Ilaria; Bonsegna, Stefania; De Domenico, Stefania; Laddomada, Barbara; Blando, Federica; Santino, Angelo; Giovinazzo, Giovanna

    2011-10-01

    A novel strategy to induce parthenocarpy in tomato fruits by the induction of resveratrol biosynthesis in flower tissues was exploited. Two transgenic tomato lines were considered: a higher resveratrol-producing (35SS) line, constitutively expressing a grape stilbene synthase cDNA, and a lower resveratrol-producing (LoxS) line, expressing stilbene synthase under a fruit-specific promoter. The expression of the stilbene synthase gene affected flavonoid metabolism in a different manner in the transgenic lines, and in one of these, the 35SS line, resulted in complete male sterility. Resveratrol was synthesised either in 35SS or LoxS tomato flowers, at an even higher extent (about 8-10 times) in the former line. We further investigated whether stilbene synthase expression may have resulted in impaired naringenin accumulation during flower development. In the 35SS flowers, naringenin was significantly impaired by about 50%, probably due to metabolic competition. Conversely, the amount of glycosylated flavonols increased in transgenic flowers, thereby excluding the diminished production of flavonols as a reason for parthenocarpy in tomato. We further investigated whether resveratrol synthesis may have resulted changes to pollen structure. Microscopic observations revealed the presence of few and abnormal flake-like pollen grains in 35SS flowers with no germination capability. Finally, the analysis of coumaric and ferulic acids, the precursors of lignin and sporopollenin biosynthesis, revealed significant depletion of these compounds, therefore suggesting an impairment in structural compounds as a reason for pollen ablation. These overall outcomes, to the best of our knowledge, reveal for the first time the major role displayed by resveratrol synthesis on parthenocarpy in tomato fruits. PMID:21843947

  5. A genome-wide association study identifies multiple loci for variation in human ear morphology.

    PubMed

    Adhikari, Kaustubh; Reales, Guillermo; Smith, Andrew J P; Konka, Esra; Palmen, Jutta; Quinto-Sanchez, Mirsha; Acuña-Alonzo, Victor; Jaramillo, Claudia; Arias, William; Fuentes, Macarena; Pizarro, María; Barquera Lozano, Rodrigo; Macín Pérez, Gastón; Gómez-Valdés, Jorge; Villamil-Ramírez, Hugo; Hunemeier, Tábita; Ramallo, Virginia; Silva de Cerqueira, Caio C; Hurtado, Malena; Villegas, Valeria; Granja, Vanessa; Gallo, Carla; Poletti, Giovanni; Schuler-Faccini, Lavinia; Salzano, Francisco M; Bortolini, Maria-Cátira; Canizales-Quinteros, Samuel; Rothhammer, Francisco; Bedoya, Gabriel; Calderón, Rosario; Rosique, Javier; Cheeseman, Michael; Bhutta, Mahmood F; Humphries, Steve E; Gonzalez-José, Rolando; Headon, Denis; Balding, David; Ruiz-Linares, Andrés

    2015-01-01

    Here we report a genome-wide association study for non-pathological pinna morphology in over 5,000 Latin Americans. We find genome-wide significant association at seven genomic regions affecting: lobe size and attachment, folding of antihelix, helix rolling, ear protrusion and antitragus size (linear regression P values 2 × 10(-8) to 3 × 10(-14)). Four traits are associated with a functional variant in the Ectodysplasin A receptor (EDAR) gene, a key regulator of embryonic skin appendage development. We confirm expression of Edar in the developing mouse ear and that Edar-deficient mice have an abnormally shaped pinna. Two traits are associated with SNPs in a region overlapping the T-Box Protein 15 (TBX15) gene, a major determinant of mouse skeletal development. Strongest association in this region is observed for SNP rs17023457 located in an evolutionarily conserved binding site for the transcription factor Cartilage paired-class homeoprotein 1 (CART1), and we confirm that rs17023457 alters in vitro binding of CART1. PMID:26105758

  6. A genome-wide association study identifies multiple loci for variation in human ear morphology.

    PubMed

    Adhikari, Kaustubh; Reales, Guillermo; Smith, Andrew J P; Konka, Esra; Palmen, Jutta; Quinto-Sanchez, Mirsha; Acuña-Alonzo, Victor; Jaramillo, Claudia; Arias, William; Fuentes, Macarena; Pizarro, María; Barquera Lozano, Rodrigo; Macín Pérez, Gastón; Gómez-Valdés, Jorge; Villamil-Ramírez, Hugo; Hunemeier, Tábita; Ramallo, Virginia; Silva de Cerqueira, Caio C; Hurtado, Malena; Villegas, Valeria; Granja, Vanessa; Gallo, Carla; Poletti, Giovanni; Schuler-Faccini, Lavinia; Salzano, Francisco M; Bortolini, Maria-Cátira; Canizales-Quinteros, Samuel; Rothhammer, Francisco; Bedoya, Gabriel; Calderón, Rosario; Rosique, Javier; Cheeseman, Michael; Bhutta, Mahmood F; Humphries, Steve E; Gonzalez-José, Rolando; Headon, Denis; Balding, David; Ruiz-Linares, Andrés

    2015-06-24

    Here we report a genome-wide association study for non-pathological pinna morphology in over 5,000 Latin Americans. We find genome-wide significant association at seven genomic regions affecting: lobe size and attachment, folding of antihelix, helix rolling, ear protrusion and antitragus size (linear regression P values 2 × 10(-8) to 3 × 10(-14)). Four traits are associated with a functional variant in the Ectodysplasin A receptor (EDAR) gene, a key regulator of embryonic skin appendage development. We confirm expression of Edar in the developing mouse ear and that Edar-deficient mice have an abnormally shaped pinna. Two traits are associated with SNPs in a region overlapping the T-Box Protein 15 (TBX15) gene, a major determinant of mouse skeletal development. Strongest association in this region is observed for SNP rs17023457 located in an evolutionarily conserved binding site for the transcription factor Cartilage paired-class homeoprotein 1 (CART1), and we confirm that rs17023457 alters in vitro binding of CART1.

  7. A genome-wide association study identifies multiple loci for variation in human ear morphology

    PubMed Central

    Adhikari, Kaustubh; Reales, Guillermo; Smith, Andrew J. P.; Konka, Esra; Palmen, Jutta; Quinto-Sanchez, Mirsha; Acuña-Alonzo, Victor; Jaramillo, Claudia; Arias, William; Fuentes, Macarena; Pizarro, María; Barquera Lozano, Rodrigo; Macín Pérez, Gastón; Gómez-Valdés, Jorge; Villamil-Ramírez, Hugo; Hunemeier, Tábita; Ramallo, Virginia; Silva de Cerqueira, Caio C.; Hurtado, Malena; Villegas, Valeria; Granja, Vanessa; Gallo, Carla; Poletti, Giovanni; Schuler-Faccini, Lavinia; Salzano, Francisco M.; Bortolini, Maria- Cátira; Canizales-Quinteros, Samuel; Rothhammer, Francisco; Bedoya, Gabriel; Calderón, Rosario; Rosique, Javier; Cheeseman, Michael; Bhutta, Mahmood F.; Humphries, Steve E.; Gonzalez-José, Rolando; Headon, Denis; Balding, David; Ruiz-Linares, Andrés

    2015-01-01

    Here we report a genome-wide association study for non-pathological pinna morphology in over 5,000 Latin Americans. We find genome-wide significant association at seven genomic regions affecting: lobe size and attachment, folding of antihelix, helix rolling, ear protrusion and antitragus size (linear regression P values 2 × 10−8 to 3 × 10−14). Four traits are associated with a functional variant in the Ectodysplasin A receptor (EDAR) gene, a key regulator of embryonic skin appendage development. We confirm expression of Edar in the developing mouse ear and that Edar-deficient mice have an abnormally shaped pinna. Two traits are associated with SNPs in a region overlapping the T-Box Protein 15 (TBX15) gene, a major determinant of mouse skeletal development. Strongest association in this region is observed for SNP rs17023457 located in an evolutionarily conserved binding site for the transcription factor Cartilage paired-class homeoprotein 1 (CART1), and we confirm that rs17023457 alters in vitro binding of CART1. PMID:26105758

  8. SHIELD: an integrative gene expression database for inner ear research

    PubMed Central

    Shen, Jun; Scheffer, Déborah I.; Kwan, Kelvin Y.; Corey, David P.

    2015-01-01

    The inner ear is a highly specialized mechanosensitive organ responsible for hearing and balance. Its small size and difficulty in harvesting sufficient tissue has hindered the progress of molecular studies. The protein components of mechanotransduction, the molecular biology of inner ear development and the genetic causes of many hereditary hearing and balance disorders remain largely unknown. Inner-ear gene expression data will help illuminate each of these areas. For over a decade, our laboratories and others have generated extensive sets of gene expression data for different cell types in the inner ear using various sample preparation methods and high-throughput genome-wide approaches. To facilitate the study of genes in the inner ear by efficient presentation of the accumulated data and to foster collaboration among investigators, we have developed the Shared Harvard Inner Ear Laboratory Database (SHIELD), an integrated resource that seeks to compile, organize and analyse the genomic, transcriptomic and proteomic knowledge of the inner ear. Five datasets are currently available. These datasets are combined in a relational database that integrates experimental data and annotations relevant to the inner ear. The SHIELD has a searchable web interface with two data retrieval options: viewing the gene pages online or downloading individual datasets as data tables. Each retrieved gene page shows the gene expression data and detailed gene information with hyperlinks to other online databases with up-to-date annotations. Downloadable data tables, for more convenient offline data analysis, are derived from publications and are current as of the time of publication. The SHIELD has made published and some unpublished data freely available to the public with the hope and expectation of accelerating discovery in the molecular biology of balance, hearing and deafness. Database URL: https://shield.hms.harvard.edu PMID:26209310

  9. FGF23 deficiency leads to mixed hearing loss and middle ear malformation in mice.

    PubMed

    Lysaght, Andrew C; Yuan, Quan; Fan, Yi; Kalwani, Neil; Caruso, Paul; Cunnane, MaryBeth; Lanske, Beate; Stanković, Konstantina M

    2014-01-01

    Fibroblast growth factor 23 (FGF23) is a circulating hormone important in phosphate homeostasis. Abnormal serum levels of FGF23 result in systemic pathologies in humans and mice, including renal phosphate wasting diseases and hyperphosphatemia. We sought to uncover the role FGF23 plays in the auditory system due to shared molecular mechanisms and genetic pathways between ear and kidney development, the critical roles multiple FGFs play in auditory development and the known hearing phenotype in mice deficient in klotho (KL), a critical co-factor for FGF23 signaling. Using functional assessments of hearing, we demonstrate that Fgf[Formula: see text] mice are profoundly deaf. Fgf[Formula: see text] mice have moderate hearing loss above 20 kHz, consistent with mixed conductive and sensorineural pathology of both middle and inner ear origin. Histology and high-voltage X-ray computed tomography of Fgf[Formula: see text] mice demonstrate dysplastic bulla and ossicles; Fgf[Formula: see text] mice have near-normal morphology. The cochleae of mutant mice appear nearly normal on gross and microscopic inspection. In wild type mice, FGF23 is ubiquitously expressed throughout the cochlea. Measurements from Fgf[Formula: see text] mice do not match the auditory phenotype of Kl-/- mice, suggesting that loss of FGF23 activity impacts the auditory system via mechanisms at least partially independent of KL. Given the extensive middle ear malformations and the overlap of initiation of FGF23 activity and Eustachian tube development, this work suggests a possible role for FGF23 in otitis media.

  10. Do sleep abnormalities and misaligned sleep/circadian rhythm patterns represent early clinical characteristics for developing psychosis in high risk populations?

    PubMed

    Zanini, Marcio; Castro, Juliana; Coelho, Fernando Morgadinho; Bittencourt, Lia; Bressan, Rodrigo A; Tufik, Sergio; Brietzke, Elisa

    2013-12-01

    Sleep architecture changes, such as slow-wave sleep (SWS) percentage variations and reductions in latency and density of rapid eye movement (REM), are found in most patients with schizophrenia and are considered to be an important part of the pathophysiology of the disorder. In addition to these sleep parameters changes, disruptions in sleep homeostasis and the sleep/circadian rhythm also occur in these patients. Sleep/circadian rhythm abnormalities negatively affect neocortical plasticity and cognition and often precede the diagnosis of the illness. Thus, it has been suggested that the sleep/circadian rhythm might be involved in the pathophysiology of psychosis. Recent advances in the identification of individuals at a high risk for developing schizophrenia allow us to investigate several neurobiological processes involved in the development of psychosis. In this article, we review the current evidence of the effects of sleep parameter abnormalities, disruptions in sleep homeostasis and misalignments of sleep circadian rhythm on the early stages of schizophrenia. In addition, we discuss the preliminary evidence of sleep and circadian rhythm abnormalities during the prodromal stages of psychosis and propose that these abnormalities can be explored as potential predictors, as an adjunct to clinical diagnosis, of developing a psychotic disorder in at risk populations.

  11. 3D printed bionic ears.

    PubMed

    Mannoor, Manu S; Jiang, Ziwen; James, Teena; Kong, Yong Lin; Malatesta, Karen A; Soboyejo, Winston O; Verma, Naveen; Gracias, David H; McAlpine, Michael C

    2013-06-12

    The ability to three-dimensionally interweave biological tissue with functional electronics could enable the creation of bionic organs possessing enhanced functionalities over their human counterparts. Conventional electronic devices are inherently two-dimensional, preventing seamless multidimensional integration with synthetic biology, as the processes and materials are very different. Here, we present a novel strategy for overcoming these difficulties via additive manufacturing of biological cells with structural and nanoparticle derived electronic elements. As a proof of concept, we generated a bionic ear via 3D printing of a cell-seeded hydrogel matrix in the anatomic geometry of a human ear, along with an intertwined conducting polymer consisting of infused silver nanoparticles. This allowed for in vitro culturing of cartilage tissue around an inductive coil antenna in the ear, which subsequently enables readout of inductively-coupled signals from cochlea-shaped electrodes. The printed ear exhibits enhanced auditory sensing for radio frequency reception, and complementary left and right ears can listen to stereo audio music. Overall, our approach suggests a means to intricately merge biologic and nanoelectronic functionalities via 3D printing.

  12. 3D printed bionic ears.

    PubMed

    Mannoor, Manu S; Jiang, Ziwen; James, Teena; Kong, Yong Lin; Malatesta, Karen A; Soboyejo, Winston O; Verma, Naveen; Gracias, David H; McAlpine, Michael C

    2013-06-12

    The ability to three-dimensionally interweave biological tissue with functional electronics could enable the creation of bionic organs possessing enhanced functionalities over their human counterparts. Conventional electronic devices are inherently two-dimensional, preventing seamless multidimensional integration with synthetic biology, as the processes and materials are very different. Here, we present a novel strategy for overcoming these difficulties via additive manufacturing of biological cells with structural and nanoparticle derived electronic elements. As a proof of concept, we generated a bionic ear via 3D printing of a cell-seeded hydrogel matrix in the anatomic geometry of a human ear, along with an intertwined conducting polymer consisting of infused silver nanoparticles. This allowed for in vitro culturing of cartilage tissue around an inductive coil antenna in the ear, which subsequently enables readout of inductively-coupled signals from cochlea-shaped electrodes. The printed ear exhibits enhanced auditory sensing for radio frequency reception, and complementary left and right ears can listen to stereo audio music. Overall, our approach suggests a means to intricately merge biologic and nanoelectronic functionalities via 3D printing. PMID:23635097

  13. Ear disorders in scuba divers.

    PubMed

    Azizi, M H

    2011-01-01

    History of underwater diving dates back to antiquity. Breath-hold technique in diving was known to the ancient nations. However, deep diving progressed only in the early decades of the 19th century as the result of advancements in efficient underwater technologies which subsequently led to invention of sophisticated sets of scuba diving in the 20th century. Currently, diving is performed for various purposes including commercial, recreational, military, underwater construction, oil industry, underwater archeology and scientific assessment of marine life. By increasing popularity of underwater diving, dive-related medical conditions gradually became more evident and created a new challenge for the health care professionals, so that eventually, a specialty the so-called "diving medicine" was established. Most of the diving-associated disorders appear in the head and neck. The most common of all occupational disorders associated with diving are otologic diseases. External otitis has been reported as the most common otolaryngologic problem in underwater divers. Exostosis of the external ear canal may be formed in divers as the result of prolonged diving in cold waters. Other disorders of the ear and paranasal sinuses in underwater divers are caused by barometric pressure change (i.e., barotraumas), and to a lesser extent by decompression sickness. Barotrauma of the middle ear is the most prevalent barotrauma in divers. The inner ear barotraumas, though important, is less common. The present paper is a brief overview of diving-related ear disorders particularly in scuba divers.

  14. 3D finite element model of the chinchilla ear for characterizing middle ear functions.

    PubMed

    Wang, Xuelin; Gan, Rong Z

    2016-10-01

    Chinchilla is a commonly used animal model for research of sound transmission through the ear. Experimental measurements of the middle ear transfer function in chinchillas have shown that the middle ear cavity greatly affects the tympanic membrane (TM) and stapes footplate (FP) displacements. However, there is no finite element (FE) model of the chinchilla ear available in the literature to characterize the middle ear functions with the anatomical features of the chinchilla ear. This paper reports a recently completed 3D FE model of the chinchilla ear based on X-ray micro-computed tomography images of a chinchilla bulla. The model consisted of the ear canal, TM, middle ear ossicles and suspensory ligaments, and the middle ear cavity. Two boundary conditions of the middle ear cavity wall were simulated in the model as the rigid structure and the partially flexible surface, and the acoustic-mechanical coupled analysis was conducted with these two conditions to characterize the middle ear function. The model results were compared with experimental measurements reported in the literature including the TM and FP displacements and the middle ear input admittance in chinchilla ear. An application of this model was presented to identify the acoustic role of the middle ear septa-a unique feature of chinchilla middle ear cavity. This study provides the first 3D FE model of the chinchilla ear for characterizing the middle ear functions through the acoustic-mechanical coupled FE analysis.

  15. 3D finite element model of the chinchilla ear for characterizing middle ear functions.

    PubMed

    Wang, Xuelin; Gan, Rong Z

    2016-10-01

    Chinchilla is a commonly used animal model for research of sound transmission through the ear. Experimental measurements of the middle ear transfer function in chinchillas have shown that the middle ear cavity greatly affects the tympanic membrane (TM) and stapes footplate (FP) displacements. However, there is no finite element (FE) model of the chinchilla ear available in the literature to characterize the middle ear functions with the anatomical features of the chinchilla ear. This paper reports a recently completed 3D FE model of the chinchilla ear based on X-ray micro-computed tomography images of a chinchilla bulla. The model consisted of the ear canal, TM, middle ear ossicles and suspensory ligaments, and the middle ear cavity. Two boundary conditions of the middle ear cavity wall were simulated in the model as the rigid structure and the partially flexible surface, and the acoustic-mechanical coupled analysis was conducted with these two conditions to characterize the middle ear function. The model results were compared with experimental measurements reported in the literature including the TM and FP displacements and the middle ear input admittance in chinchilla ear. An application of this model was presented to identify the acoustic role of the middle ear septa-a unique feature of chinchilla middle ear cavity. This study provides the first 3D FE model of the chinchilla ear for characterizing the middle ear functions through the acoustic-mechanical coupled FE analysis. PMID:26785845

  16. Alternative splicing of inner-ear-expressed genes.

    PubMed

    Wang, Yanfei; Liu, Yueyue; Nie, Hongyun; Ma, Xin; Xu, Zhigang

    2016-09-01

    Alternative splicing plays a fundamental role in the development and physiological function of the inner ear. Inner-ear-specific gene splicing is necessary to establish the identity and maintain the function of the inner ear. For example, exon 68 of Cadherin 23 (Cdh23) gene is subject to inner-ear-specific alternative splicing, and as a result, Cdh23(+ 68) is only expressed in inner ear hair cells. Alternative splicing along the tonotopic axis of the cochlea contributes to frequency tuning, particularly in lower vertebrates, such as chickens and turtles. Differential splicing of Kcnma1, which encodes for the α subunit of the Ca(2+)-activated K(+) channel (BK channel), has been suggested to affect the channel gating properties and is important for frequency tuning. Consequently, deficits in alternative splicing have been shown to cause hearing loss, as we can observe in Bronx Waltzer (bv) mice and Sfswap mutant mice. Despite the advances in this field, the regulation of alternative splicing in the inner ear remains elusive. Further investigation is also needed to clarify the mechanism of hearing loss caused by alternative splicing deficits. PMID:27376950

  17. Household number associated with middle ear disease at an urban Indigenous health service: a cross-sectional study.

    PubMed

    Spurling, Geoffrey K P; Askew, Deborah A; Schluter, Philip J; Simpson, Fiona; Hayman, Noel E

    2014-01-01

    Few epidemiological studies of middle ear disease have been conducted in Aboriginal and Torres Strait Islander populations, yet the disease is common and causes hearing impairment and poorer educational outcomes. The objective of this study is to identify factors associated with abnormal middle ear appearance, a proxy for middle ear disease. Aboriginal and Torres Strait Islander children aged 0-14 years receiving a Child Health Check (CHC) at an urban Indigenous Health Service, Brisbane, Australia were recruited from 2007 to 2010. Mixed-effects models were used to explore associations of 10 recognised risk factors with abnormal middle ear appearance at the time of the CHC. Ethical approval and community support for the project were obtained. Four hundred and fifty-three children were included and 54% were male. Participants were Aboriginal (92%), Torres Strait Islander (2%) or both (6%). Abnormal middle ear appearance was observed in 26 (6%) children and was significantly associated with previous ear infection (odds ratio (OR), 8.8; 95% confidence interval (CI), 3.2-24.0) and households with eight or more people (OR, 3.8; 95% CI, 1.1-14.1) in the imputed multivariable mixed-effects model. No significant associations were found for the other recognised risk factors investigated. Overcrowding should continue to be a core focus for communities and policy makers in reducing middle ear disease and its consequences in Aboriginal and Torres Strait Islander peoples.

  18. Human-induced contaminant impacts on migratory birds: lessons from the North American eared grebe (Podiceps nigricollis)

    USGS Publications Warehouse

    Sladky, Kurt K.; Quist, Charlotte; Ramirez, Pedro; Hill, David; Dein, F. Joshua

    2003-01-01

    The effects of aquatic contaminants generated by soda ash mining processes on the North American eared grebe (Podiceps nigricollis) population illustrates important issues associated with human-induced habitat degradation on the health of migratory species. Eared grebes have been extensively studied in their staging and breeding habitats, but little is known about their 2- to 3-day migratory periods. During migration, few bodies of water are available to the birds for refuge between freshwater breeding areas in Canada and hypersaline lakes (e.g., Great Salt Lake in Utah or Mono Lake in California) to which they migrate. One geographic refuge area includes a series of "tailings" ponds associated with soda ash mining operations in southwestern Wyoming. The ponds range from 100-1200 acres, with water containing high concentrations of sodium decahydrate (Na2CO3•10H2O). At cool temperatures (generally < 40°F) sodium decahydrate precipitates out of the water and crystallizes on solid objects in the ponds or on the water surface. Bird mortality on these ponds has been recognized since the early 1970's, and the mining companies have developed hazing strategies and rehabilitation programs in order to minimize mortality. In order to determine causes of grebe mortality and devise strategies to reduce mortality, a field epidemiologic investigation was developed with the following objectives: 1) to determine whether eared grebes have quantifiable physiologic abnormalities associated with exposure to soda ash mine pond water; 2) to evaluate physical effects of sodium decahydrate crystallization on grebe survival; 3) to establish cause of death based on necropsy of deceased grebes; 4) to determine long-term survivability of eared grebes after exposure to the pond water; and 5) to evaluate water quality and determine whether aquatic invertebrates are present in the ponds as a possible food source.

  19. The impact of steroid withdrawal on the development of lipid abnormalities and obesity in heart transplant recipients.

    PubMed

    Lake, K D; Reutzel, T J; Pritzker, M R; Jorgensen, C R; Emery, R W

    1993-01-01

    Hyperlipidemia and obesity are common problems after heart transplantation, which may increase the risk of chronic graft atherosclerosis. The intent of this study was to (1) determine the impact of a history of hyperlipidemia on the occurrence of lipid abnormalities after transplantation, (2) compare lipid profiles of those patients being treated with triple-drug immunosuppression versus those patients weaned from prednisone therapy, and (3) identify any factors that would predict which patients are at highest risk for the development of hyperlipidemia after transplantation. Of 89 patients who lived for more than 12 months, 35 patients had a history of hyperlipidemia before heart transplantation (cholesterol level of more than 240 mg/dl; low-density lipoprotein cholesterol level of more than 160 mg/dl). The most dramatic rise in cholesterol level was observed in patients with no history of hyperlipidemia who were treated with triple-drug immunosuppression, in whom a 64% increase occurred versus a 24% increase in patients receiving steroid-free immunosuppression (p < 0.001). In patients with a history of hyperlipidemia, cholesterol level increased by 20% with triple-drug immunosuppression versus 14% with steroid-free immunosuppression (p = 0.613); however, 83% of the patients in the triple-drug group and 92% in the steroid-free group had elevated cholesterol levels. Multiple regression analysis revealed that significant independent and additive (p < 0.00001) contributions with respect to percent change in cholesterol level were evident for (1) a negative history of hyperlipidemia (p = 0.005), (2) triple-drug immunosuppression (p = 0.0021), and (3) female sex (p = 0.0113). A negative history of hyperlipidemia was predictive of the percent change in low-density lipoprotein cholesterol level (p = 0.0049), and triple-drug immunosuppression administration predicted the percent change in high-density lipoprotein cholesterol (p = 0.0119). Patients with a positive history

  20. Deletion at 14q22-23 indicates a contiguous gene syndrome comprising anophthalmia, pituitary hypoplasia, and ear anomalies.

    PubMed

    Nolen, Leisha D; Amor, David; Haywood, Ashley; St Heaps, Luke; Willcock, Chris; Mihelec, Marija; Tam, Patrick; Billson, Frank; Grigg, John; Peters, Greg; Jamieson, Robyn V

    2006-08-15

    Anophthalmia and pituitary gland hypoplasia are both debilitating conditions where the underlying genetic defect is unknown in the majority of cases. We identified a patient with bilateral anophthalmia and absence of the optic nerves, chiasm and tracts, as well as pituitary gland hypoplasia and ear anomalies with a de novo apparently balanced chromosomal translocation, 46,XY,t(3;14)(q28;q23.2). Translocation breakpoint analysis using FISH and high-resolution microarray comparative genomic hybridization (CGH) has identified a 9.66 Mb deleted region on the long arm of chromosome 14 which includes the genes BMP4, OTX2, RTN1, SIX6, SIX1, and SIX4. Three other patients with interstitial deletions involving 14q22-23 have been described, all with bilateral anophthalmia, pituitary abnormalities, ear anomalies, and a facial phenotype similar to our patient. OTX2 is involved in ocular developmental defects, and the severity of the ocular phenotype in our patient and the other 14q22-23 deletion patients, suggests this genomic region harbors other gene/s involved in ocular development. BMP4 haploinsufficiency is predicted to contribute to the ocular phenotype on the basis of its expression pattern and observed murine mutant phenotypes. In addition, deletion of BMP4 and SIX6 is likely to contribute to the abnormal pituitary development, and SIX1 deletion may contribute to the ear and other craniofacial features. This indicates that contiguous gene deletion may contribute to the phenotypic features in the 14q22-23 deletion patients.

  1. Parental smoking and middle ear effusions in children.

    PubMed

    Hinton, A E; Buckley, G

    1988-11-01

    A study was conducted on seventy children to ascertain any relationship between parental smoking and the presence of middle ear effusions in the children. Information was collected using a questionnaire, clinical examination and audiological tests. Both groups of children (with and without effusions) had similar age, sex and social class distributions. It was found that the presence of middle ear effusions in the children was associated with an increased incidence of parental smoking. Those children with an abnormal tympanometry result were more likely to have at least one parent who smoked than those with normal tympanometry results. There was no relationship between resolution of the effusion and parental smoking. It is therefore suggested that as part of the management of this condition parents should be advised of the effects of smoking on the condition and encouraged to avoid smoking in the same environment as their children.

  2. Pathophysiology of inner ear decompression sickness: potential role of the persistent foramen ovale.

    PubMed

    Mitchell, Simon J; Doolette, David J

    2015-06-01

    Inner-ear decompression sickness (inner ear DCS) may occur in isolation ('pure' inner-ear DCS), or as part of a multisystem DCS presentation. Symptoms may develop during decompression from deep, mixed-gas dives or after surfacing from recreational air dives. Modelling of inner-ear inert gas kinetics suggests that onset during decompression results from supersaturation of the inner-ear tissue and in-situ bubble formation. This supersaturation may be augmented by inert gas counterdiffusion following helium to nitrogen gas switches, but such switches are unlikely, of themselves, to precipitate inner-ear DCS. Presentations after surfacing from air dives are frequently the 'pure' form of inner ear DCS with short symptom latency following dives to moderate depth, and the vestibular end organ appears more vulnerable than is the cochlea. A large right-to-left shunt (usually a persistent foramen ovale) is found in a disproportionate number of cases, suggesting that shunted venous gas emboli (VGE) cause injury to the inner-ear. However, this seems an incomplete explanation for the relationship between inner-ear DCS and right-to-left shunt. The brain must concomitantly be exposed to larger numbers of VGE, yet inner-ear DCS frequently occurs in the absence of cerebral symptoms. This may be explained by slower inert gas washout in the inner ear than in the brain. Thus, there is a window after surfacing within which VGE arriving in the inner-ear (but not the brain) would grow due to inward diffusion of supersaturated inert gas. A similar difference in gas kinetics may explain the different susceptibilities of cochlear and vestibular tissue within the inner-ear itself. The cochlea has greater perfusion and a smaller tissue volume, implying faster inert gas washout. It may be susceptible to injury by incoming arterial bubbles for a shorter time after surfacing than the vestibular organ.

  3. Physiological functioning of the ear and masking

    NASA Technical Reports Server (NTRS)

    1984-01-01

    The physiological functions of the ear and the role masking plays in speech communication are examined. Topics under investigation include sound analysis of the ear, the aural reflex, and various types of noise masking.

  4. How to Prevent Painful Swimmer's Ear

    MedlinePlus

    ... fullstory_159452.html How to Prevent Painful Swimmer's Ear Simple steps after a day in the water ... 2016 SATURDAY, June 18, 2016 (HealthDay News) -- Swimmer's ear -- a common summertime problem among children -- is easy ...

  5. Some Studies of Spontaneous and Evoked Acoustic Emissions from the Human Ear.

    NASA Astrophysics Data System (ADS)

    Sutton, Graham John

    Available from UMI in association with The British Library. Several studies of spontaneous and evoked oto -acoustic emissions (SOAEs and EOAEs) and their relationship to normal and pathological hearing mechanisms are described. (1) A computer model was developed to study the theory that the EOAE is produced by summation of responses from activity distributed along the cochlea. It was found that, whilst with a regular cochlea and mapping the summed response is small owing to phase cancellation, any irregularity of mapping or structure or sensitivity gives rise to a sharply-tuned response showing many of the characteristics of EOAEs. (2) A search for SOAEs in 17 normal subjects revealed a prevalence of 53% (42% of ears), which was significantly higher among the females. (3) A group of 49 clinical tinnitus sufferers was investigated to test the hypothesis that SOAEs might be responsible in some cases. 3 such cases were found, all with tonal tinnitus and normal hearing at the tinnitus pitch (although 1 had a bilateral loss at other frequencies). The SOAEs in both studies were found between 680 and 9610 Hz at levels up to 24 dB SPL. They were not found at frequencies where hearing threshold was greater than 20 dB, and no consistent association with audiometric abnormalities was noted, implying that they should be seen as part of normal hearing variation, rather than the result of minor pathology. (4) Suppression effects were studied for (a) SOAEs in 2 ears and (b) both click -evoked and continuous-tone evoked OAEs in another ear. A number of features were observed, most notably multiple lobes in many of the suppression tuning curves. The implications of these results for theories of OAE generation are discussed.

  6. A Ubiquitous Blood Pressure Sensor Worn at the Ear

    NASA Astrophysics Data System (ADS)

    Koizumi, Hiroshi; Shimada, Junichi; Uenishi, Yuji; Tochikubo, Osamu

    2009-12-01

    Blood pressure (BP) measurement and BP control are important for the prevention of lifestyle diseases, especially hypertension, which can lead to more serious conditions, such as cardiac infarction and cerebral apoplexy. The purpose of our study is to develop a ubiquitous blood pressure sensor that is more comfortable and less disruptive of users' daily activities than conventional blood pressure sensors. Our developed sensor is worn at an ear orifice and measures blood pressure at the tragus. This paper describes the concept, configuration, and the optical and electronic details of the developed ear-worn blood pressure sensor and presents preliminary evaluation results. The developed sensor causes almost no discomfort and produces signals whose quality is high enough for detecting BP at an ear, making it suitable for ubiquitous usage.

  7. The caecilian ear: further observations.

    PubMed

    Wever, E G; Gans, C

    1976-10-01

    The structure of the ear is examined in two species of caecilians, Ichthyophis glutinosus and I. orthoplicatus, and the sensitivity to aerial sounds is assessed in terms of the electrical potentials of the cochlea. The results are in general agreement with previous reports on other caecilian species. PMID:1068485

  8. Adenomatous tumors of the middle ear.

    PubMed

    Pelosi, Stanley; Koss, Shira

    2015-04-01

    Adenomatous tumors are an uncommon cause of a middle ear mass. Clinical findings may be nonspecific, leading to difficulties in differentiation from other middle ear tumors. Controversy also exists whether to classify middle ear adenoma and carcinoid as separate neoplasms, or alternatively within a spectrum of the same pathologic entity. Most adenomatous middle ear tumors are indolent in behavior, with a benign histologic appearance and slowly progressive growth. The mainstay of treatment is complete surgical resection, which affords the greatest likelihood of cure.

  9. Salivary gland choristoma of the middle ear in a child with situs inversus totalis.

    PubMed

    Toros, Sema Zer; Egeli, Erol; Kiliçarslan, Yasin; Gümrükçü, Gülistan; Gökçeer, Tanju; Noşeri, Hülya

    2010-06-01

    Salivary gland choristoma of the middle ear cavity is an extremely rare entity. It is thought to be a developmental abnormality and may be associated with abnormalities of adjacent structures. We report a case of salivary gland choristoma of the middle ear with prominent Körner's septum in a 7-year-old girl with situs inversus totalis. Situs inversus totalis is the mirror image reversal of the normal position of the internal organs and frequently associated with other congenital anomalies. This patient is the first reported case with situs inversus totalis, having middle ear salivary gland choristoma and prominent bilateral Körner's septae. The management and the differential diagnosis of this condition are discussed with the review of the literature.

  10. Ear recognition based on Gabor features and KFDA.

    PubMed

    Yuan, Li; Mu, Zhichun

    2014-01-01

    We propose an ear recognition system based on 2D ear images which includes three stages: ear enrollment, feature extraction, and ear recognition. Ear enrollment includes ear detection and ear normalization. The ear detection approach based on improved Adaboost algorithm detects the ear part under complex background using two steps: offline cascaded classifier training and online ear detection. Then Active Shape Model is applied to segment the ear part and normalize all the ear images to the same size. For its eminent characteristics in spatial local feature extraction and orientation selection, Gabor filter based ear feature extraction is presented in this paper. Kernel Fisher Discriminant Analysis (KFDA) is then applied for dimension reduction of the high-dimensional Gabor features. Finally distance based classifier is applied for ear recognition. Experimental results of ear recognition on two datasets (USTB and UND datasets) and the performance of the ear authentication system show the feasibility and effectiveness of the proposed approach.

  11. Pediatric Obesity and Ear, Nose, and Throat Disorders

    MedlinePlus

    ... an ENT Doctor Near You Pediatric Obesity and Ear, Nose, and Throat Disorders Pediatric Obesity and Ear, ... all children be regularly screened for snoring. Middle ear infections Acute otitis media (AOM) and chronic ear ...

  12. Functional characterization of middle ear mucosa residues in cholesteatoma samples.

    PubMed

    Sudhoff, H; Bujía, J; Holly, A; Kim, C; Fisseler-Eckhoff, A

    1994-03-01

    Cholesteatoma epithelium is characterized by a keratinocyte dysregulation with an aggressive growth that leads to the destruction of normal middle ear mucosa. The abnormal behavior of cholesteatoma epithelium seems to be induced by the presence of a heavy immune cell infiltrate releasing different cytokines and growth factors in high amounts. Middle ear mucosa rests are often observed within the cholesteatoma stroma or adjacent to the advancing front of cholesteatoma epithelium. This study investigated the presence of interleukin-1 (IL-1), transforming growth factor-alpha (TGF-alpha), epidermal growth factor (EGF), and epidermal growth factor-receptor (EGF-R) in the mucosa rests as well as the expression of an activation marker, 4F2. The findings were correlated with the features of a surrounding stroma with an enhanced immune cell infiltrate. Cholesteatoma epithelium showed a high staining intensity of IL-1, TGF-alpha, and EGF-R. In contrast to this, middle ear mucosa did not show any positive reactions for the mentioned factors. Epidermal growth factor immunoreactivity was found in neither cholesteatoma epithelium nor in middle ear mucosa residues. The authors found a high concentration of lymphocytes and macrophages in the surrounding stroma. Most of these cells expressed TGF-alpha, IL-1, and 4F2, suggesting an activated form. Results indicate that keratinocytes present in the middle ear mucosa do not appear to react to the stimuli released by the inflamed stroma, reflecting important differences in the cell biological features of the keratinocytes that form parts of both types of epithelium.

  13. Immunologic Disorders of the Inner Ear.

    ERIC Educational Resources Information Center

    Kinney, William C.; Hughes, Gordon B.

    1997-01-01

    Immune inner ear disease represents a series of immune system mediated problems that can present with hearing loss, dizziness, or both. The etiology, presentation, testing, and treatment of primary immune inner ear disease is discussed. A review of secondary immune inner ear disease is presented for comparison. (Contains references.) (Author/CR)

  14. Can Loud Music Hurt My Ears?

    MedlinePlus

    ... Here's Help White House Lunch Recipes Can Loud Music Hurt My Ears? KidsHealth > For Kids > Can Loud Music Hurt My Ears? Print A A A Text ... up? Oh! You want to know if loud music can hurt your ears . Are you asking because ...

  15. 21 CFR 878.3590 - Ear prosthesis.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 21 Food and Drugs 8 2011-04-01 2011-04-01 false Ear prosthesis. 878.3590 Section 878.3590 Food and... GENERAL AND PLASTIC SURGERY DEVICES Prosthetic Devices § 878.3590 Ear prosthesis. (a) Identification. An ear prosthesis is a silicone rubber solid device intended to be implanted to reconstruct the...

  16. 21 CFR 878.3590 - Ear prosthesis.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ... 21 Food and Drugs 8 2014-04-01 2014-04-01 false Ear prosthesis. 878.3590 Section 878.3590 Food and... GENERAL AND PLASTIC SURGERY DEVICES Prosthetic Devices § 878.3590 Ear prosthesis. (a) Identification. An ear prosthesis is a silicone rubber solid device intended to be implanted to reconstruct the...

  17. 21 CFR 878.3590 - Ear prosthesis.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 21 Food and Drugs 8 2010-04-01 2010-04-01 false Ear prosthesis. 878.3590 Section 878.3590 Food and... GENERAL AND PLASTIC SURGERY DEVICES Prosthetic Devices § 878.3590 Ear prosthesis. (a) Identification. An ear prosthesis is a silicone rubber solid device intended to be implanted to reconstruct the...

  18. 21 CFR 878.3590 - Ear prosthesis.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ... 21 Food and Drugs 8 2013-04-01 2013-04-01 false Ear prosthesis. 878.3590 Section 878.3590 Food and... GENERAL AND PLASTIC SURGERY DEVICES Prosthetic Devices § 878.3590 Ear prosthesis. (a) Identification. An ear prosthesis is a silicone rubber solid device intended to be implanted to reconstruct the...

  19. 21 CFR 878.3590 - Ear prosthesis.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ... 21 Food and Drugs 8 2012-04-01 2012-04-01 false Ear prosthesis. 878.3590 Section 878.3590 Food and... GENERAL AND PLASTIC SURGERY DEVICES Prosthetic Devices § 878.3590 Ear prosthesis. (a) Identification. An ear prosthesis is a silicone rubber solid device intended to be implanted to reconstruct the...

  20. MicroRNA-122 Influences the Development of Sperm Abnormalities from Human Induced Pluripotent Stem Cells by Regulating TNP2 Expression

    PubMed Central

    Huang, Yongyi; Liu, Jianjun; Zhao, Yanhui; Jiang, Lizhen; Huang, Qin

    2013-01-01

    Sperm abnormalities are one of the main factors responsible for male infertility; however, their pathogenesis remains unclear. The role of microRNAs in the development of sperm abnormalities in infertile men has not yet been investigated. Here, we used human induced pluripotent stem cells to investigate the influence of miR-122 expression on the differentiation of these cells into spermatozoa-like cells in vitro. After induction, mutant miR-122-transfected cells formed spermatozoa-like cells. Flow cytometry of DNA content revealed a significant increase in the haploid cell population in spermatozoa-like cells derived from mutant miR-122-transfected cells as compared to those derived from miR-122-transfected cells. During induction, TNP2 and protamine mRNA and protein levels were significantly higher in mutant miR-122-transfected cells than in miR-122-transfected cells. High-throughput isobaric tags for relative and absolute quantification were used to identify and quantify the different protein expression levels in miR-122- and mutant miR-122-transfected cells. Among all the proteins analyzed, the expression of lipoproteins, for example, APOB and APOA1, showed the most significant difference between the two groups. This study illustrates that miR-122 expression is associated with abnormal sperm development. MiR-122 may influence spermatozoa-like cells by suppressing TNP2 expression and inhibiting the expression of proteins associated with sperm development. PMID:23327642

  1. Down-Turner Syndrome: A Case with Double Monoclonal Chromosomal Abnormality

    PubMed Central

    Alvarez-Manassero, Denisse; Merino-Luna, Alfredo

    2016-01-01

    Introduction. The coexistence of Down and Turner syndromes due to double chromosome aneuploidy is very rare; it is even more rare to find the presence of a double monoclonal chromosomal abnormality. Objective. To report a unique case of double monoclonal chromosomal abnormality with trisomy of chromosome 21 and an X ring chromosome in all cells studied; no previous report has been found. Case Report. Female, 28 months old, with pathological short stature from birth, with the following dysmorphic features: tilted upward palpebral fissures, short neck, brachycephaly, and low-set ears. During the neonatal period, the infant presented generalized hypotonia and lymphedema of hands and feet. Karyotype showed 47,X,r(X),+21 [30]. Conclusion. Clinical features of both Down and Turner syndromes were found, highlighting short stature that has remained below 3 z score from birth to the present, associated with delayed psychomotor development. G-banded karyotype analysis in peripheral blood is essential for a definitive diagnosis. PMID:27672470

  2. Down-Turner Syndrome: A Case with Double Monoclonal Chromosomal Abnormality.

    PubMed

    Manassero-Morales, Gioconda; Alvarez-Manassero, Denisse; Merino-Luna, Alfredo

    2016-01-01

    Introduction. The coexistence of Down and Turner syndromes due to double chromosome aneuploidy is very rare; it is even more rare to find the presence of a double monoclonal chromosomal abnormality. Objective. To report a unique case of double monoclonal chromosomal abnormality with trisomy of chromosome 21 and an X ring chromosome in all cells studied; no previous report has been found. Case Report. Female, 28 months old, with pathological short stature from birth, with the following dysmorphic features: tilted upward palpebral fissures, short neck, brachycephaly, and low-set ears. During the neonatal period, the infant presented generalized hypotonia and lymphedema of hands and feet. Karyotype showed 47,X,r(X),+21 [30]. Conclusion. Clinical features of both Down and Turner syndromes were found, highlighting short stature that has remained below 3 z score from birth to the present, associated with delayed psychomotor development. G-banded karyotype analysis in peripheral blood is essential for a definitive diagnosis. PMID:27672470

  3. Down-Turner Syndrome: A Case with Double Monoclonal Chromosomal Abnormality

    PubMed Central

    Alvarez-Manassero, Denisse; Merino-Luna, Alfredo

    2016-01-01

    Introduction. The coexistence of Down and Turner syndromes due to double chromosome aneuploidy is very rare; it is even more rare to find the presence of a double monoclonal chromosomal abnormality. Objective. To report a unique case of double monoclonal chromosomal abnormality with trisomy of chromosome 21 and an X ring chromosome in all cells studied; no previous report has been found. Case Report. Female, 28 months old, with pathological short stature from birth, with the following dysmorphic features: tilted upward palpebral fissures, short neck, brachycephaly, and low-set ears. During the neonatal period, the infant presented generalized hypotonia and lymphedema of hands and feet. Karyotype showed 47,X,r(X),+21 [30]. Conclusion. Clinical features of both Down and Turner syndromes were found, highlighting short stature that has remained below 3 z score from birth to the present, associated with delayed psychomotor development. G-banded karyotype analysis in peripheral blood is essential for a definitive diagnosis.

  4. Down-Turner Syndrome: A Case with Double Monoclonal Chromosomal Abnormality.

    PubMed

    Manassero-Morales, Gioconda; Alvarez-Manassero, Denisse; Merino-Luna, Alfredo

    2016-01-01

    Introduction. The coexistence of Down and Turner syndromes due to double chromosome aneuploidy is very rare; it is even more rare to find the presence of a double monoclonal chromosomal abnormality. Objective. To report a unique case of double monoclonal chromosomal abnormality with trisomy of chromosome 21 and an X ring chromosome in all cells studied; no previous report has been found. Case Report. Female, 28 months old, with pathological short stature from birth, with the following dysmorphic features: tilted upward palpebral fissures, short neck, brachycephaly, and low-set ears. During the neonatal period, the infant presented generalized hypotonia and lymphedema of hands and feet. Karyotype showed 47,X,r(X),+21 [30]. Conclusion. Clinical features of both Down and Turner syndromes were found, highlighting short stature that has remained below 3 z score from birth to the present, associated with delayed psychomotor development. G-banded karyotype analysis in peripheral blood is essential for a definitive diagnosis.

  5. Robust algorithmic detection of the developed cardiac pathologies and emerging or transient abnormalities from short periods of RR data

    NASA Astrophysics Data System (ADS)

    Gavrishchaka, Valeriy V.; Senyukova, Olga

    2011-06-01

    Numerous research efforts and clinical testing have confirmed validity of heart rate variability (HRV) analysis as one of the cardiac diagnostics modalities. The majority of HRV analysis tools currently used in practice are based on linear indicators. Methods from nonlinear dynamics (NLD) provide more natural modeling framework for adaptive biological systems with multiple feedback loops. Compared to linear indicators, many NLD-based measures are much less sensitive to data artifacts and non-stationarity. However, majority of NLD measures require long time series for stable calculation. Similar restrictions also apply for linear indicators. Such requirements could drastically limit practical usability of HRV analysis in many applications, including express diagnostics, early indication of subtle directional changes during personalization of medical treatment, and robust detection of emerging or transient abnormalities. Recently we have illustrated that these challenges could be overcome by using classification framework based on boosting-like ensemble learning techniques that are capable of discovering robust meta-indicators from existing HRV measures and other incomplete empirical knowledge. In this paper we demonstrate universality of such meta-indicators and discuss operational details of their practical usage. Using such pathology examples as congestive heart failure (CHF) and arrhythmias, we show that classifiers trained on short RR segments (down to several minutes) could achieve reasonable classification accuracy (˜80-85% and higher). These indicators calculated from longer RR segments could be applicable for accurate diagnostics with classification accuracy approaching 100%. In addition, it is feasible to discover single "normal-abnormal" meta-classifier capable of detecting multiple abnormalities.

  6. Investigation of middle ear anatomy and function with combined video otoscopy-phase sensitive OCT

    PubMed Central

    Park, Jesung; Cheng, Jeffrey T.; Ferguson, Daniel; Maguluri, Gopi; Chang, Ernest W.; Clancy, Caitlin; Lee, Daniel J.; Iftimia, Nicusor

    2016-01-01

    We report the development of a novel otoscopy probe for assessing middle ear anatomy and function. Video imaging and phase-sensitive optical coherence tomography are combined within the same optical path. A sound stimuli channel is incorporated as well to study middle ear function. Thus, besides visualizing the morphology of the middle ear, the vibration amplitude and frequency of the eardrum and ossicles are retrieved as well. Preliminary testing on cadaveric human temporal bone models has demonstrated the capability of this instrument for retrieving middle ear anatomy with micron scale resolution, as well as the vibration of the tympanic membrane and ossicles with sub-nm resolution. PMID:26977336

  7. LDV measurement of bird ear vibrations to determine inner ear impedance and middle ear power flow

    NASA Astrophysics Data System (ADS)

    Muyshondt, Pieter G. G.; Pires, Felipe; Dirckx, Joris J. J.

    2016-06-01

    The mechanical behavior of the middle ear structures in birds and mammals is affected by the fluids in the inner ear (IE) that are present behind the oval window. In this study, the aim was to gather knowledge of the acoustic impedance of the IE in the ostrich, to be able to determine the effect on vibrations and power flow in the single-ossicle bird middle ear for future studies. To determine the IE impedance, vibrations of the ossicle were measured for both the quasi-static and acoustic stimulus frequencies. In the acoustic regime, vibrations were measured with a laser Doppler vibrometer and electromagnetic stimulation of the ossicle. The impedance of the inner ear could be determined by means of a simple RLC model in series, which resulted in a stiffness reactance of KIE = 0.20.1012 Pa/m3, an inertial impedance of MIE = 0.652.106 Pa s2/m3, and a resistance of RIE = 1.57.109 Pa s/m. The measured impedance is found to be considerably smaller than what is found for the human IE.

  8. Developing Software to “Track and Catch” Missed Follow-up of Abnormal Test Results in a Complex Sociotechnical Environment

    PubMed Central

    Smith, M.; Murphy, D.; Laxmisan, A.; Sittig, D.; Reis, B.; Esquivel, A.; Singh, H.

    2013-01-01

    Summary Background Abnormal test results do not always receive timely follow-up, even when providers are notified through electronic health record (EHR)-based alerts. High workload, alert fatigue, and other demands on attention disrupt a provider’s prospective memory for tasks required to initiate follow-up. Thus, EHR-based tracking and reminding functionalities are needed to improve follow-up. Objectives The purpose of this study was to develop a decision-support software prototype enabling individual and system-wide tracking of abnormal test result alerts lacking follow-up, and to conduct formative evaluations, including usability testing. Methods We developed a working prototype software system, the Alert Watch And Response Engine (AWARE), to detect abnormal test result alerts lacking documented follow-up, and to present context-specific reminders to providers. Development and testing took place within the VA’s EHR and focused on four cancer-related abnormal test results. Design concepts emphasized mitigating the effects of high workload and alert fatigue while being minimally intrusive. We conducted a multifaceted formative evaluation of the software, addressing fit within the larger socio-technical system. Evaluations included usability testing with the prototype and interview questions about organizational and workflow factors. Participants included 23 physicians, 9 clinical information technology specialists, and 8 quality/safety managers. Results Evaluation results indicated that our software prototype fit within the technical environment and clinical workflow, and physicians were able to use it successfully. Quality/safety managers reported that the tool would be useful in future quality assurance activities to detect patients who lack documented follow-up. Additionally, we successfully installed the software on the local facility’s “test” EHR system, thus demonstrating technical compatibility. Conclusion To address the factors involved in missed

  9. Modeling and estimating acoustic transfer functions of external ears with or without headphones.

    PubMed

    Deng, Huiqun; Yang, Jun

    2015-08-01

    The acoustic transfer functions of external ears with or without headphones affect the features of perceived sounds and vary considerably with listeners and headphones. A method for estimating the frequency responses of external-ear transfer functions from the sound at the entrance of a blocked ear canal (or from the input of a headphone) to the sound at the eardrum for different listeners and headphones is developed based on an acoustic signal model of external ears. The model allows for applying realistic data about individual external ears and headphones and is advantageous over current standard ear simulators with fixed structures limited to simulating average ear canals and eardrum impedances below 10 kHz. Given different eardrum impedances, ear canal shapes, lengths, and headphones, the frequency responses of external-ear transfer functions are estimated and presented. In addition, a method of determining the Norton equivalent volume velocity or Thevenien equivalent sound pressure sources of a headphone from sound pressure signals in an acoustic tube is presented. These methods are validated via direct measurements and expected to have applications in headphone sound reproduction, headphone and hearing aid design, and audiometric and psychoacoustic measurements to produce desired sounds at the eardrums of different listeners.

  10. A short-wave infrared otoscope for middle ear disease diagnostics (Conference Presentation)

    NASA Astrophysics Data System (ADS)

    Carr, Jessica A.; Valdez, Tulio; Bruns, Oliver; Bawendi, Moungi

    2016-02-01

    Otitis media, a range of inflammatory conditions of the middle ear, is the second most common illness diagnosed in children. However, the diagnosis can be challenging, particularly in pediatric patients. Otitis media is commonly over-diagnosed and over-treated and has been identified as one of the primary factors in increased antibiotic resistance. We describe the development of a short-wave infrared (SWIR) otoscope for objective middle ear effusion diagnosis. The SWIR otoscope can unambiguously detect the presence of middle ear fluid based on its strong light absorption in the SWIR. This absorption causes a stark, visual contrast between the presence and absence of fluid behind the tympanic membrane. Additionally, when there is no middle ear fluid, the deeper tissue penetration of SWIR light allows the SWIR otoscope to better visualize middle ear anatomy through the tympanic membrane than is possible with visible light. We demonstrate that in healthy, adult human ears, SWIR otoscopy can image a range of middle ear anatomy, including landmarks of the entire ossicular chain, the promontory, the round window niche, and the chorda tympani. We suggest that SWIR otoscopy can provide valuable diagnostic information complementary to that provided by visible pneumotoscopy in the diagnosis of middle ear effusions, otitis media, and other maladies of the middle ear.

  11. Altered mRNA Splicing, Chondrocyte Gene Expression and Abnormal Skeletal Development due to SF3B4 Mutations in Rodriguez Acrofacial Dysostosis

    PubMed Central

    Nevarez, Lisette; Pogue, Robert; Krakow, Deborah; Cohn, Daniel H.

    2016-01-01

    The acrofacial dysostoses (AFD) are a genetically heterogeneous group of inherited disorders with craniofacial and limb abnormalities. Rodriguez syndrome is a severe, usually perinatal lethal AFD, characterized by severe retrognathia, oligodactyly and lower limb abnormalities. Rodriguez syndrome has been proposed to be a severe form of Nager syndrome, a non-lethal AFD that results from mutations in SF3B4, a component of the U2 small nuclear ribonucleoprotein particle (U2 snRNP). Furthermore, a case with a phenotype intermediate between Rodriguez and Nager syndromes has been shown to have an SF3B4 mutation. We identified heterozygosity for SF3B4 mutations in Rodriguez syndrome, confirming that the phenotype is a dominant disorder that is allelic with Nager syndrome. The mutations led to reduced SF3B4 synthesis and defects in mRNA splicing, primarily exon skipping. The mutations also led to reduced expression in growth plate chondrocytes of target genes, including the DLX5, DLX6, SOX9, and SOX6 transcription factor genes, which are known to be important for skeletal development. These data provide mechanistic insight toward understanding how SF3B4 mutations lead to the skeletal abnormalities observed in the acrofacial dysostoses. PMID:27622494

  12. Identification of genes expressed in the Xenopus inner ear.

    PubMed

    Serrano, E E; Trujillo-Provencio, C; Sultemeier, D R; Bullock, W M; Quick, Q A

    2001-11-01

    Recent studies indicate that hearing loss in humans has strong hereditary components associated with expression of specific genes in the auditory apparatus of the inner ear. However, the inner ear poses challenges for molecular research because the amount of tissue that can be isolated is limited, and extraction procedures yield small quantities of RNA and protein. To begin to identity genes essential for auditory function, we synthesized a cDNA library using an RT-PCR protocol and total RNA isolated from eight Xenopus laevis inner ears. Sequence analysis of randomly selected clones demonstrated expression of both identified (calmodulin, SNARE protein, syndecan-2) and unidentified genes, and confirmed synthesis of full length transcripts. Confocal and scanning electron microscopy (SEM) were used to examine the structure of inner ear organs that serve as auditory receptors in amphibians: the sacculus, the amphibian papilla and the basilar papilla. SEM images illustrate the heterogeneity of bundle morphology and demonstrate the continuous appearance of stereociliary bundles in the X. laevis amphibian papilla during larval development and adult life. Investigations of gene expression in Xenopus auditory organs using clones recovered from inner ear cDNA libraries should provide insight regarding the molecular basis of hearing.

  13. [Immunohistochemical studies with middle ear mucosal remnants in cholesteatoma].

    PubMed

    Sudhoff, H; Borkowski, G; Bujia, J; Hildmann, H; Fisseler-Eckhoff, A

    1997-08-01

    The development of a middle ear cholesteatoma is usually associated with chronic inflammation and displacement of the mucosa present by the invading squamous epithelium. To analyze the clinically different behaviors of both epithelia, we used immunohistochemical methods to study the distribution and expression of interleukin-1 (Il-1), transforming growth factor-alpha (TGF-alpha), epidermal growth factor (EGF), epidermal growth factor-receptor (EGF-R), the proliferation marker MIB 1, c-myc proto-oncogene product and activation marker 4F2. Results stromal that keratinocytes in a cholesteatoma exhibited a much higher activation and proliferation rate when compared to middle ear mucosa cells. Middle ear epithelial cells showed no immunoreactivity for TGF-alpha, EGF-R, Il-1 and c-myc in contrast to the markedly positive immunoreactivity found in cholesteatoma matrix. The local release of cytokines and growth factors, such as TGF-alpha, EGF and Il-1 by inflammatory cells seems to be an important factor for the hyper-proliferative behavior of cholesteatoma epithelium. Our findings could contribute to the pathogenesis of middle ear cholesteatoma and give a possible explanation for the sustained progression of its growth leading to displacement of the middle ear mucosa.

  14. The War of Jenkins’ Ear

    PubMed Central

    Graboyes, Evan M.; Hullar, Timothy E.

    2012-01-01

    Objective In 1731, Spanish sailors boarded the British brig Rebecca off the coast of Cuba and sliced off the left ear of its captain, Robert Jenkins. This traumatic auriculectomy was used as a pretext by the British to declare war on Spain in 1739, a conflict that is now known as the War of Jenkins’ Ear. Here, we examine the techniques available for auricular repair at the time of Jenkins’ injury and relate them to the historical events surrounding the incident. Methods Review of relevant original published manuscripts and monographs. Results Surgeons in the mid-18th century did not have experience with repair of traumatic total auriculectomies. Some contemporary surgeons favored auricular prostheses over surgical treatment. Methods for the reconstruction of partial defects were available, and most authors advocated a local post-auricular flap instead of a free tissue transfer. Techniques for repair of defects of the auricle lagged behind those for repair of the nose. Conclusion Limitations in care of traumatic auricular defects may have intensified the significance of Jenkins’ injury and helped lead to the War of Jenkins’ Ear, but conflict between Britain and Spain was probably unavoidable due to their conflicting commercial interests in the Caribbean. PMID:23444484

  15. Injury Patterns and Reconstruction in Acquired Ear Deformities.

    PubMed

    Stewart, Kenneth; Majdak-Paredes, Ewa

    2015-12-01

    Reconstruction following ear trauma presents a heterogeneous group of abnormalities with significantly more variation in presentation. The aim of the study was to analyze our experience and expound some broad principles of auricular reconstruction in acquired ear deformities. A total of 117 patients with human, animal bites and revision otoplasty presented to our clinic. Demographic data were extracted from medical records and photographs. Management options included no reconstruction, external silicone prosthesis, or autologous reconstruction. Fifty percent of patients with human bite injuries and 62% with animal bite injuries opted for autologous ear reconstruction. A flap with either a costal cartilage framework (37/39; 95%) or conchal cartilage (2/39; 5%) was used. In revision otoplasty group, 12% required autologous reconstruction either with conchal or costal cartilage. We discuss our indications, techniques, complications, and predictable pattern of injuries in human bites. Autologous auricular reconstruction of traumatic injuries is a safe procedure associated with aesthetically pleasing outcome and improved quality of life despite physical and psychosocial comorbidities. Elderly patients are more likely to opt for prosthetic camouflage. PMID:26667640

  16. Human Action Recognition Using Wireless Wearable In-Ear Microphone

    NASA Astrophysics Data System (ADS)

    Nishimura, Jun; Kuroda, Tadahiro

    To realize the ubiquitous eating habits monitoring, we proposed the use of sounds sensed by an in-ear placed wireless wearable microphone. A prototype of wireless wearable in-ear microphone was developed by utilizing a common Bluetooth headset. We proposed a robust chewing action recognition algorithm which consists of two recognition stages: “chew-like” signal detection and chewing sound verification stages. We also provide empirical results on other action recognition using in-ear sound including swallowing, cough, belch, and etc. The average chewing number counting error rate of 1.93% is achieved. Lastly, chewing sound mapping is proposed as a new prototypical approach to provide an additional intuitive feedback on food groups to be able to infer the eating habits in their daily life context.

  17. Ear canal dynamic motion as a source of power for in-ear devices

    NASA Astrophysics Data System (ADS)

    Delnavaz, Aidin; Voix, Jérémie

    2013-02-01

    Ear canal deformation caused by temporomandibular joint (jaw joint) activity, also known as "ear canal dynamic motion," is introduced in this paper as a candidate source of power to possibly recharge hearing aid batteries. The geometrical deformation of the ear canal is quantified in 3D by laser scanning of different custom ear moulds. An experimental setup is proposed to measure the amount of power potentially available from this source. The results show that 9 mW of power is available from a 15 mm3 dynamic change in the ear canal volume. Finally, the dynamic motion and power capability of the ear canal are investigated in a group of 12 subjects.

  18. [Computer-aided surgery planning for implantation of artificial ear].

    PubMed

    Huang, Yufeng; Huang, Yuanliang; Niu, Mao; Wang, Lisheng; Chang, Shixin

    2009-08-01

    In conventional ear implantation surgery, clinical physicians usually make a surgery planning based on their observation on series of 2D X-ray images or CT images. Such a planning method requires the physicians to have a high level of clinical experience. Besides, the whole operation is unintuitive, and might have certain risk. Considering these facts, we have developed a computer-aided system for the surgery planning of the implantation of artificial ear based on CT imaging and 3D reconstruction techniques. The system effectively overcomes the main drawbacks in conventional surgery planning techniques, and it makes the surgery planning procedure more precise, safe, and intuitive. PMID:19813593

  19. To Have an Ear: Music and the Otological Experience.

    PubMed

    Sattar, Atia

    2016-09-01

    This essay analyzes the historical development of otology in relation to music. It illustrates the integral role of music perception and appreciation in the study of hearing, where hearing operates not simply as a scientific phenomenon but signifies particular meaningful experiences in society. The four historical moments considered-Helmholtz's piano-keyed cochlea, the ear phonautograph, the hearing aid, and the cochlear implant-show how the sounds, perceptions, and instruments of music have mediated and continue to mediate our relationships with hearing. To have an ear, one does not just bear a physiological hearing mechanism; one experiences the aesthetics of musical sound. PMID:24994077

  20. Ear malformation and hearing loss in patients with Treacher Collins syndrome.

    PubMed

    Pron, G; Galloway, C; Armstrong, D; Posnick, J

    1993-01-01

    Although the hearing loss of patients with Treacher Collins syndrome is well documented, few studies have reported jointly on their hearing loss and ear pathology. This paper reports on the hearing loss and computerized tomography (CT) assessments of ear malformations in a large pediatric series of patients with Treacher Collins. Of the 29 subjects assessed by the Craniofacial Program between 1986 and 1990, paired audiologic and complete CT assessments were available for 23 subjects. The external ear canal abnormalities were largely symmetric, either bilaterally stenotic or atretic. In most cases, the middle ear cavity was bilaterally hypoplastic and dysmorphic, and ossicles were symmetrically dysmorphic or missing. Inner ear structures were normal in all patients. The majority of patients had a unilateral or bilateral moderate or greater degree of hearing loss and almost half had an asymmetric hearing loss. The hearing loss of all subjects was conductive, except for three whose loss was bilateral mixed. Two types of bilaterally symmetric hearing loss configurations, flat and reverse sloping, were noted. Conductive hearing loss in patients with Treacher Collins is mainly attributable to their middle ear malformations, which are similar for those of patients with malformed or missing ossicles. PMID:8418881

  1. Investigation of bacterial biofilm in the human middle ear using optical coherence tomography and acoustic measurements.

    PubMed

    Nguyen, Cac T; Robinson, Sarah R; Jung, Woonggyu; Novak, Michael A; Boppart, Stephen A; Allen, Jont B

    2013-07-01

    Children with chronic otitis media (OM) often have conductive hearing loss which results in communication difficulties and requires surgical treatment. Recent studies have provided clinical evidence that there is a one-to-one correspondence between chronic OM and the presence of a bacterial biofilm behind the tympanic membrane (TM). Here we investigate the acoustic effects of bacterial biofilms, confirmed using optical coherence tomography (OCT), in adult ears. Non-invasive OCT images are collected to visualize the cross-sectional structure of the middle ear, verifying the presence of a biofilm behind the TM. Wideband measurements of acoustic reflectance and impedance (0.2-6 [kHz]) are used to study the acoustic properties of ears with confirmed bacterial biofilms. Compared to known acoustic properties of normal middle ears, each of the ears with a bacterial biofilm has an elevated power reflectance in the 1 to 3 [kHz] range, corresponding to an abnormally small resistance (real part of the impedance). These results provide assistance for the clinical diagnosis of a bacterial biofilm, which could lead to improved treatment of chronic middle ear infection and further understanding of the impact of chronic OM on conductive hearing loss. This article is part of a special issue entitled "MEMRO 2012".

  2. Sex Chromosome Meiotic Drive Systems in DROSOPHILA MELANOGASTER I. Abnormal Spermatid Development in Males with a Heterochromatin-Deficient X Chromosome (sc4sc8)

    PubMed Central

    Peacock, W. J.; Miklos, George L. Gabor; Goodchild, D. J.

    1975-01-01

    The meiotic drive characteristics of the In(1)sc4Lsc8R/Y system have been examined by genetic analysis and by light and electron microscopy. sc4sc8/Y males show a direct correlation between nondisjunction frequency and meiotic drive. Temperature-shift experiments reveal that the temperature-sensitive period for nondisjunction is at meiosis, whereas that for meiotic drive has both meiotic and post-meiotic components. Cytological analyses in the light and electron microscopes reveal failures in spermiogenesis in the testes of sc4sc8 males. The extent of abnormal spermatid development increases as nondisjunction becomes more extreme. PMID:805751

  3. Histopathology of the Human Inner Ear in Alström Syndrome

    PubMed Central

    Nadol, Joseph B.; Marshall, Jan D.; Bronson, Roderick T.

    2015-01-01

    Alström syndrome is an autosomal recessive syndromic genetic disorder caused by mutations in the ALMS1 gene. Sensorineural hearing loss occurs in greater than 85% of patients. Histopathology of the inner ear abnormalities in the human has not previously been fully described. Histopathology of the inner ear in Alström syndrome is presented in two genetically confirmed cases. The predominant histopathologic correlates of the sensorineural loss were degeneration of the organ of Corti, both inner and outer hair cells, degeneration of spiral ganglion cells, and atrophy of the stria vascularis and spiral ligament. PMID:26111748

  4. Explicit News Writing Rules and the Intuitive "Good Ear."

    ERIC Educational Resources Information Center

    Abbott, Clifford; Slattery, Karen

    1990-01-01

    Argues that news-writing instruction that relies on explicit rules is inadequate because: (1) rule violations are common; (2) some rules work well only in particular situations; and (3) some rules overgeneralize. Identifies the difficulty of teaching students to develop a "good ear" for writing. Concludes that instruction must balance rule-based…

  5. Future Approaches for Inner Ear Protection and Repair

    ERIC Educational Resources Information Center

    Shibata, Seiji B.; Raphael, Yehoash

    2010-01-01

    Health care professionals tending to patients with inner ear disease face inquiries about therapy options, including treatments that are being developed for future use but not yet available. The devastating outcome of sensorineural hearing loss, combined with the permanent nature of the symptoms, make these inquiries demanding and frequent. The…

  6. [Hypopharyngeal carcinoma and red ear drum].

    PubMed

    Bender, B; Widmann, G; Riechelmann, H; Schmutzhard, J

    2011-04-01

    A 46-year-old male patient with an unresectable hypopharyngeal carcinoma was treated with primary radio-chemotherapy. At follow-up, the patient presented with a red ear drum and combined hearing loss. Because of radiotherapy-induced tubal dysfunction, paracentesis was performed. Biopsy of the polypoid middle ear mucosa revealed petrous bone infiltration of hypopharyngeal carcinoma. MRI studies revealed paracarotideal tumor infiltration to the petrous bone and the middle ear arising from a cervical retropharyngeal lymph node metastasis. PMID:20963385

  7. Red ear syndrome precipitated by a dietary trigger: a case report

    PubMed Central

    2014-01-01

    Introduction Red ear syndrome is a rare condition characterized by episodic attacks of erythema of the ear accompanied by burning ear pain. Symptoms are brought on by touch, exertion, heat or cold, stress, neck movements and washing or brushing of hair. Diagnosis and treatment of this condition are challenging. The case we report here involves a woman whose symptoms were brought on by a dietary trigger: orange juice as well as stress, causing significant physical and psychological morbidity. Avoidance of triggers resulted in symptomatic improvement. Case presentation A 22-year-old Caucasian woman who was a student presented twice to our department with evolving symptoms, the first time with hyperacusis (abnormal sound sensitivity arising from within the auditory system to sounds of moderate volume), intermittent right tinnitus and subjective hearing difficulties. She presented five years later with highly distressing episodes of erythematous ears, which were associated with burning pain around the ear and temporal areas, and intolerance to noise. After keeping a symptom diary, she identified orange juice and stress as triggers of her symptoms. No local head and neck pathology was present. Investigations and imaging were negative. Avoidance of triggers led to great symptomatic improvement. To the best of our knowledge, dietary triggers have not previously been reported as a trigger for this syndrome. This case shows a direct temporal link to a dietary trigger and supports a primary pathogenesis. Recognition and management of primary headache disorder and simple dietary and lifestyle changes brought about symptomatic relief. Conclusion Red ear syndrome is a little-known clinical syndrome of unknown etiology and management. To the best of our knowledge, our present case report is the first to describe primary red ear syndrome triggered by orange juice. Clinical benefit derived from avoidance of this trigger, which is already known to precipitate migraines, gives some

  8. An Effective 3D Ear Acquisition System

    PubMed Central

    Liu, Yahui; Lu, Guangming; Zhang, David

    2015-01-01

    The human ear is a new feature in biometrics that has several merits over the more common face, fingerprint and iris biometrics. It can be easily captured from a distance without a fully cooperative subject. Also, the ear has a relatively stable structure that does not change much with the age and facial expressions. In this paper, we present a novel method of 3D ear acquisition system by using triangulation imaging principle, and the experiment results show that this design is efficient and can be used for ear recognition. PMID:26061553

  9. An Effective 3D Ear Acquisition System.

    PubMed

    Liu, Yahui; Lu, Guangming; Zhang, David

    2015-01-01

    The human ear is a new feature in biometrics that has several merits over the more common face, fingerprint and iris biometrics. It can be easily captured from a distance without a fully cooperative subject. Also, the ear has a relatively stable structure that does not change much with the age and facial expressions. In this paper, we present a novel method of 3D ear acquisition system by using triangulation imaging principle, and the experiment results show that this design is efficient and can be used for ear recognition.

  10. Listening to Nature's orchestra with peculiar ears

    NASA Astrophysics Data System (ADS)

    Yager, David D.

    2003-04-01

    Insects use hearing for the crucial tasks of communicating with conspecifics and avoiding predators. Although all are based on the same acoustic principles, the diversity of insect ears is staggering and instructive. For instance, a South African grasshopper demonstrates that hearing conspecific calls is possible over distances 1 km with ears that do not have tympana. Actually, these creatures have six pairs of ears that play different roles in behavior. In numerical contrast, praying mantises have just a single ear in the ventral midline. The ear is very effective at detecting ultrasonic bat cries. However, the bioacoustics of sound transduction by two tympana facing each other in a deep, narrow slit is a puzzle. Tachinid flies demonstrate that directional hearing at 5 kHz is possible with a pair of ears fused together to give a total size of 1 mm. The ears are under the fly's chin. Hawk moths have their ears built into their mouthparts and the tympanum is more like a hollow ball than the usual membrane. As an apt last example, cicada ears are actually part of the orchestra: their tympana function both in sound reception and sound production.

  11. An Effective 3D Ear Acquisition System.

    PubMed

    Liu, Yahui; Lu, Guangming; Zhang, David

    2015-01-01

    The human ear is a new feature in biometrics that has several merits over the more common face, fingerprint and iris biometrics. It can be easily captured from a distance without a fully cooperative subject. Also, the ear has a relatively stable structure that does not change much with the age and facial expressions. In this paper, we present a novel method of 3D ear acquisition system by using triangulation imaging principle, and the experiment results show that this design is efficient and can be used for ear recognition. PMID:26061553

  12. In vivo imaging of middle-ear and inner-ear microstructures of a mouse guided by SD-OCT combined with a surgical microscope.

    PubMed

    Cho, Nam Hyun; Jang, Jeong Hun; Jung, Woonggyu; Kim, Jeehyun

    2014-04-21

    We developed an augmented-reality system that combines optical coherence tomography (OCT) with a surgical microscope. By sharing the common optical path in the microscope and OCT, we could simultaneously acquire OCT and microscope views. The system was tested to identify the middle-ear and inner-ear microstructures of a mouse. Considering the probability of clinical application including otorhinolaryngology, diseases such as middle-ear effusion were visualized using in vivo mouse and OCT images simultaneously acquired through the eyepiece of the surgical microscope during surgical manipulation using the proposed system. This system is expected to realize a new practical area of OCT application.

  13. Selective forces on origin, adaptation and reduction of tympanal ears in insects.

    PubMed

    Strauß, Johannes; Stumpner, Andreas

    2015-01-01

    Insect ears evolved many times independently. As a consequence, a striking diversity exists in the location, construction and behavioural implementation of ears. In this review, we first summarise what is known about the evolutionary origin of ears and the presumed precursor organs in the various insect groups. Thereafter, we focus on selective forces for making and keeping an ear: we discuss detecting and localising predators and conspecifics, including establishing new "private" channels for intraspecific communication. More advanced aspects involve judging the distance of conspecifics, or assessing individual quality from songs which makes auditory processing a means for exerting sexual selection on mating partners. We try to identify negative selective forces, mainly in the context of energy expenditure for developing and keeping an ear, but also in conjunction with acoustic communication, which incorporates risks like eavesdropping by predators and parasitoids. We then discuss balancing pressures, which might oppose optimising an ear for a specific task (when it serves different functions, for example). Subsequently, we describe various scenarios that might have led to a reduction or complete loss of ears in evolution. Finally, we describe cases of sex differences in ears and potential reasons for their appearance.

  14. Hyperspectral imaging system for whole corn ear surface inspection

    NASA Astrophysics Data System (ADS)

    Yao, Haibo; Kincaid, Russell; Hruska, Zuzana; Brown, Robert L.; Bhatnagar, Deepak; Cleveland, Thomas E.

    2013-05-01

    Aflatoxin is a mycotoxin produced mainly by Aspergillus flavus (A.flavus) and Aspergillus parasitiucus fungi that grow naturally in corn. Very serious health problems such as liver damage and lung cancer can result from exposure to high toxin levels in grain. Consequently, many countries have established strict guidelines for permissible levels in consumables. Conventional chemical-based analytical methods used to screen for aflatoxin such as thin-layer chromatography (TLC) and high performance liquid chromatography (HPLC) are time consuming, expensive, and require the destruction of samples as well as proper training for data interpretation. Thus, it has been a continuing effort within the research community to find a way to rapidly and non-destructively detect and possibly quantify aflatoxin contamination in corn. One of the more recent developments in this area is the use of spectral technology. Specifically, fluorescence hyperspectral imaging offers a potential rapid, and non-invasive method for contamination detection in corn infected with toxigenic A.flavus spores. The current hyperspectral image system is designed for scanning flat surfaces, which is suitable for imaging single or a group of corn kernels. In the case of a whole corn cob, it is preferred to be able to scan the circumference of the corn ear, appropriate for whole ear inspection. This paper discusses the development of a hyperspectral imaging system for whole corn ear imaging. The new instrument is based on a hyperspectral line scanner using a rotational stage to turn the corn ear.

  15. Ear canal hyperostosis--surfer's ear. An improved surgical technique.

    PubMed

    Seftel, D M

    1977-01-01

    The increased populatiry of surfing has produced a marked augmentation in the incidence of ear canal exostosis. However, when it becomes moderately severe, I prefer to call it "hyperostosis." Exposure to cold ocean water for many years can be an important etiologic factor in hyperostosis. There is a serious risk, and a high incidence of tympanic membrane perforations during the removal of large external canal hyperostosis. This injury can be prevented by placing a sheet of Silastic against the tympanic membrane beforehand. I describe the method. Serious degrees of hyperostosis, causing transient hearing loss and otitis externa, are increasingly common in coastal towns, where cold-water surfing is a popular year-around sport. PMID:831701

  16. Human COL2A1-directed SV40 T antigen expression in transgenic and chimeric mice results in abnormal skeletal development

    PubMed Central

    1995-01-01

    The ability of SV40 T antigen to cause abnormalities in cartilage development in transgenic mice and chimeras has been tested. The cis- regulatory elements of the COL2A1 gene were used to target expression of SV40 T antigen to differentiating chondrocytes in transgenic mice and chimeras derived from embryonal stem (ES) cells bearing the same transgene. The major phenotypic consequences of transgenic (pAL21) expression are malformed skeleton, disproportionate dwarfism, and perinatal/neonatal death. Expression of T antigen was tissue specific and in the main characteristic of the mouse alpha 1(II) collagen gene. Chondrocyte densities and levels of alpha 1(II) collagen mRNAs were reduced in the transgenic mice. Islands of cells which express cartilage characteristic genes such as type IIB procollagen, long form alpha 1(IX) collagen, alpha 2(XI) collagen, and aggrecan were found in the articular and growth cartilages of pAL21 chimeric fetuses and neonates. But these cells, which were expressing T antigen, were not properly organized into columns of proliferating chondrocytes. Levels of alpha 1(II) collagen mRNA were reduced in these chondrocytes. In addition, these cells did not express type X collagen, a marker for hypertrophic chondrocytes. The skeletal abnormality in pAL21 mice may therefore be due to a retardation of chondrocyte maturation or an impaired ability of chondrocytes to complete terminal differentiation and an associated paucity of some cartilage matrix components. PMID:7822417

  17. Electrical Stimulation of the Ear, Head, Cranial Nerve, or Cortex for the Treatment of Tinnitus: A Scoping Review

    PubMed Central

    Adjamian, Peyman

    2016-01-01

    Tinnitus is defined as the perception of sound in the absence of an external source. It is often associated with hearing loss and is thought to result from abnormal neural activity at some point or points in the auditory pathway, which is incorrectly interpreted by the brain as an actual sound. Neurostimulation therapies therefore, which interfere on some level with that abnormal activity, are a logical approach to treatment. For tinnitus, where the pathological neuronal activity might be associated with auditory and other areas of the brain, interventions using electromagnetic, electrical, or acoustic stimuli separately, or paired electrical and acoustic stimuli, have been proposed as treatments. Neurostimulation therapies should modulate neural activity to deliver a permanent reduction in tinnitus percept by driving the neuroplastic changes necessary to interrupt abnormal levels of oscillatory cortical activity and restore typical levels of activity. This change in activity should alter or interrupt the tinnitus percept (reduction or extinction) making it less bothersome. Here we review developments in therapies involving electrical stimulation of the ear, head, cranial nerve, or cortex in the treatment of tinnitus which demonstrably, or are hypothesised to, interrupt pathological neuronal activity in the cortex associated with tinnitus. PMID:27403346

  18. Ear Infection and Its Associated Risk Factors in First Nations and Rural School-Aged Canadian Children

    PubMed Central

    Karunanayake, Chandima P.; Albritton, William; Rennie, Donna C.; Lawson, Joshua A.; McCallum, Laura; Gardipy, P. Jenny; Seeseequasis, Jeremy; Naytowhow, Arnold; Hagel, Louise; McMullin, Kathleen; Ramsden, Vivian; Abonyi, Sylvia; Episkenew, Jo-Ann; Dosman, James A.; Pahwa, Punam; Project Research Team, The First Nations Lung Health; Study Team, The Saskatchewan Rural Health

    2016-01-01

    Background. Ear infections in children are a major health problem and may be associated with hearing impairment and delayed language development. Objective. To determine the prevalence and the associated risk factors of ear infections in children 6–17 years old residing on two reserves and rural areas in the province of Saskatchewan. Methodology. Data were provided from two rural cross-sectional children studies. Outcome variable of interest was presence/absence of an ear infection. Logistic regression analysis was conducted to examine the relationship between ear infection and the other covariates. Results. The prevalence of ear infection was 57.8% for rural Caucasian children and 43.6% for First Nations children living on-reserve. First Nations children had a lower risk of ear infection. Ear infection prevalence was positively associated with younger age; first born in the family; self-reported physician-diagnosed tonsillitis; self-reported physician-diagnosed asthma; and any respiratory related allergy. Protective effect of breastfeeding longer than three months was observed on the prevalence of ear infection. Conclusions. While ear infection is a prevalent condition of childhood, First Nations children were less likely to have a history of ear infections when compared to their rural Caucasian counterparts. PMID:26977160

  19. Analysis of Earing in Deep Drawn Cups

    NASA Astrophysics Data System (ADS)

    Aretz, Holger; Aegerter, Johannes; Engler, Olaf

    2010-06-01

    The cup-drawing of a strongly anisotropic sheet metal is simulated using a commercial finite element software along with a user material subroutine. In order to accurately describe the plastic anisotropy of the material the well-known recent yield function `Yld2004-18p' is extended. Regarding the experimental characterization of the considered material the occurrence of dynamic strain aging lead to an oscillating signal of the width change of the tensile samples, which prevented a reliable determination of plastic strain ratios (r-values). Thus, an improved measurement concept was developed that leads to a very robust and reproducible determination of r-values. Furthermore, a novel plane-strain tensile test sample is presented which is used for the characterization of the plastic anisotropy in biaxial loading states. A quantitative comparison with measured earing profiles of deep drawn cups illustrates the predictive capabilities of the numerical simulation.

  20. [Noise level evaluation in acute ear trauma].

    PubMed

    Bobodzhanov, U B

    2003-01-01

    Subjective noise in the ears can be defined as a pathological acoustic sensation arising in the ear in different pathology of the acoustic analyzer. To make the treatment of the injury more effective, topical diagnosis of the acoustic analyzer affection is desirable.

  1. Playing by Ear: Foundation or Frill?

    ERIC Educational Resources Information Center

    Woody, Robert H.

    2012-01-01

    Many people divide musicians into two types: those who can read music and those who play by ear. Formal music education tends to place great emphasis on producing musically literate performers but devotes much less attention to teaching students to make music without notation. Some would suggest that playing by ear is a specialized skill that is…

  2. 21 CFR 870.2710 - Ear oximeter.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 21 Food and Drugs 8 2010-04-01 2010-04-01 false Ear oximeter. 870.2710 Section 870.2710 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES CARDIOVASCULAR DEVICES Cardiovascular Monitoring Devices § 870.2710 Ear oximeter. (a) Identification. An...

  3. Cutaneous lesions of the external ear

    PubMed Central

    Sand, Michael; Sand, Daniel; Brors, Dominik; Altmeyer, Peter; Mann, Benno; Bechara, Falk G

    2008-01-01

    Skin diseases on the external aspect of the ear are seen in a variety of medical disciplines. Dermatologists, othorhinolaryngologists, general practitioners, general and plastic surgeons are regularly consulted regarding cutaneous lesions on the ear. This article will focus on those diseases wherefore surgery or laser therapy is considered as a possible treatment option or which are potentially subject to surgical evaluation. PMID:18261212

  4. Biometric recognition using 3D ear shape.

    PubMed

    Yan, Ping; Bowyer, Kevin W

    2007-08-01

    Previous works have shown that the ear is a promising candidate for biometric identification. However, in prior work, the preprocessing of ear images has had manual steps and algorithms have not necessarily handled problems caused by hair and earrings. We present a complete system for ear biometrics, including automated segmentation of the ear in a profile view image and 3D shape matching for recognition. We evaluated this system with the largest experimental study to date in ear biometrics, achieving a rank-one recognition rate of 97.8 percent for an identification scenario and an equal error rate of 1.2 percent for a verification scenario on a database of 415 subjects and 1,386 total probes.

  5. Inner ear barotrauma from scuba diving.

    PubMed

    Sheridan, M F; Hetherington, H H; Hull, J J

    1999-03-01

    Inner ear barotrauma among scuba divers is believed to be caused by any of three conditions: a hemorrhage in the inner ear, a tear of the labyrinthine membrane, or a perilymphatic fistula. These injuries may occur concurrently or separately. Hemorrhage and membrane rupture are managed conservatively, while fistula requires surgical repair. In this report, we describe three cases of inner ear barotrauma in scuba divers. We also discuss the proposed etiologies of this injury and the controversy over whether or not divers who have suffered an inner ear trauma can safely resume scuba diving. Although the older literature clearly suggests otherwise, we believe that scuba divers who completely recover from inner (or middle) ear barotrauma may return to diving as long as they exercise caution and care.

  6. Inactivation of ca10a and ca10b Genes Leads to Abnormal Embryonic Development and Alters Movement Pattern in Zebrafish

    PubMed Central

    Aspatwar, Ashok; Barker, Harlan R.; Saralahti, Anni K.; Bäuerlein, Carina A.; Ortutay, Csaba; Pan, Peiwen; Kuuslahti, Marianne; Parikka, Mataleena; Rämet, Mika; Parkkila, Seppo

    2015-01-01

    Carbonic anhydrase related proteins (CARPs) X and XI are highly conserved across species and are predominantly expressed in neural tissues. The biological role of these proteins is still an enigma. Ray-finned fish have lost the CA11 gene, but instead possess two co-orthologs of CA10. We analyzed the expression pattern of zebrafish ca10a and ca10b genes during embryonic development and in different adult tissues, and studied 61 CARP X/XI-like sequences to evaluate their phylogenetic relationship. Sequence analysis of zebrafish ca10a and ca10b reveals strongly predicted signal peptides, N-glycosylation sites, and a potential disulfide, all of which are conserved, suggesting that all of CARP X and XI are secretory proteins and potentially dimeric. RT-qPCR showed that zebrafish ca10a and ca10b genes are expressed in the brain and several other tissues throughout the development of zebrafish. Antisense morpholino mediated knockdown of ca10a and ca10b showed developmental delay with a high rate of mortality in larvae. Zebrafish morphants showed curved body, pericardial edema, and abnormalities in the head and eye, and there was increased apoptotic cell death in the brain region. Swim pattern showed abnormal movement in morphant zebrafish larvae compared to the wild type larvae. The developmental phenotypes of the ca10a and ca10b morphants were confirmed by inactivating these genes with the CRISPR/Cas9 system. In conclusion, we introduce a novel zebrafish model to investigate the mechanisms of CARP Xa and CARP Xb functions. Our data indicate that CARP Xa and CARP Xb have important roles in zebrafish development and suppression of ca10a and ca10b expression in zebrafish larvae leads to a movement disorder. PMID:26218428

  7. Protecting short-term intravascular ear catheters in healthy rabbits.

    PubMed

    Sampieri, Francesca; Orchard, Rekha N; Antonopoulos, Aphroditi J; Hamilton, Donald L

    2012-01-20

    Researchers may place a catheter in the ear vessel of a rabbit for a short period of time in order to collect repeated blood samples without extensive restraint of the animal. Maintaining such a catheter in a healthy rabbit can be challenging, as the animal may scratch at the ear, removing the catheter or forming a large hematoma that might impede blood sampling. The authors developed a technique for protecting the indwelling catheter by cutting a section of moleskin to the same shape as the ear and gluing it to the surface of the ear and the catheter. They applied this technique to collect multiple blood samples during 12-h periods from nine rabbits in a pharmacokinetics study. Catheters remained patent in five rabbits for 12 h, in two rabbits for 8 h, in one rabbit for 6 h and in one rabbit for 4 h. This technique allowed for collection of repeated blood samples and prevented the rabbits from interfering with the catheter while allowing them to move freely during the sampling period.

  8. Age- and sex-related changes in the normal human ear.

    PubMed

    Sforza, Chiarella; Grandi, Gaia; Binelli, Miriam; Tommasi, Davide G; Rosati, Riccardo; Ferrario, Virgilio F

    2009-05-30

    The objective of this study was to supply information about: (1) normal sex-related dimensions of ears (linear distances and ratios, area); (2) left-right symmetry; and (3) growth changes between childhood and old age. The three-dimensional coordinates of several soft-tissue landmarks on the ears and face were obtained by a non-invasive, computerized electromagnetic digitizer in 497 male and 346 female healthy subjects aged 4-73 years. From the landmarks, paired ear width and length, the relevant ratios, ear areas and angles relative to the facial midline, as well as indices of left-right symmetry, were calculated, and averaged for age and sex. Comparisons were performed by factorial analysis of variance. All ear dimensions were significantly larger in men than in women (p<0.001). A significant effect of age was found (p<0.001), with larger values in older individuals. The ear width-to-length ratio and the sagittal angle of the auricle significantly decreased as a function of age (p<0.001) but without sex-related differences. On average, the three-dimensional position of ears was symmetric, with symmetry coefficients ranging between 92% and 96%. Asymmetry was found in the sagittal angle of the auricle (both sexes), in the ear width-to-length ratio and ear width (men only). Data collected in the present investigation could serve as a data base for the quantitative description of human ear morphology and position during normal growth, development and aging. Forensic applications (evaluations of traumas, craniofacial alterations, teratogenic-induced conditions, facial reconstruction, aging of living and dead persons, personal identification) may also benefit from age- and sex-based data banks.

  9. The Role of Chronic Hypoxia in the Development of Neurocognitive Abnormalities in Preterm Infants with Bronchopulmonary Dysplasia

    ERIC Educational Resources Information Center

    Raman, Lakshmi; Georgieff, Michael K.; Rao, Raghavendra

    2006-01-01

    Bronchopulmonary dysplasia is the most common pulmonary morbidity in preterm infants and is associated with chronic hypoxia. Animal studies have demonstrated structural, neurochemical and functional alterations due to chronic hypoxia in the developing brain. Long-term impairments in visual-motor, gross and fine motor, articulation, reading,…

  10. Ischial hypoplasia, tibial hypoplasia and facial abnormalities: a new syndrome?

    PubMed

    Nishimura, G; Haga, Y; Aoki, K; Hasegawa, T

    1998-12-01

    A child with facial abnormalities, short stature and a variety of skeletal alterations is reported. The facial abnormalities comprised low-set ears, short nose with a long philtrum, micrognathia and cleft palate. The skeletal alterations included ischial hypoplasia, malformations of the cervical spine, hypoplasia of the lesser trochanters, tibial hypoplasia with bowing of the lower legs, tibio-fibular diastasis with malformed distal tibial epiphyses, clubfeet and brachymesophalangy. The constellation of clinical and radiological findings in the present patient do not fit any known malformation syndrome. PMID:9880644

  11. Development of a diet-induced murine model of diabetes featuring cardinal metabolic and pathophysiological abnormalities of type 2 diabetes

    PubMed Central

    Morris, Jodie L.; Bridson, Tahnee L.; Alim, Md Abdul; Rush, Catherine M.; Rudd, Donna M.; Govan, Brenda L.; Ketheesan, Natkunam

    2016-01-01

    ABSTRACT The persistent rise in global incidence of type 2 diabetes (T2D) continues to have significant public health and economic implications. The availability of relevant animal models of T2D is critical to elucidating the complexity of the pathogenic mechanisms underlying this disease and the implications this has on susceptibility to T2D complications. Whilst many high-fat diet-induced rodent models of obesity and diabetes exist, growing appreciation of the contribution of high glycaemic index diets on the development of hyperglycaemia and insulin resistance highlight the requirement for animal models that more closely represent global dietary patterns reflective of modern society. To that end, we sought to develop and validate a murine model of T2D based on consumption of an energy-dense diet containing moderate levels of fat and a high glycaemic index to better reflect the aetiopathogenesis of T2D. Male C57BL/6 mice were fed an energy-dense (ED) diet and the development of pathological features used in the clinical diagnosis of T2D was assessed over a 30-week period. Compared with control mice, 87% of mice fed an ED diet developed pathognomonic signs of T2D including glucose intolerance, hyperglycaemia, glycosylated haemoglobin (HbA1c) and glycosuria within 30 weeks. Furthermore, dyslipidaemia, chronic inflammation, alterations in circulating leucocytes and renal impairment were also evident in ED diet-fed mice compared with mice receiving standard rodent chow. Longitudinal profiling of metabolic and biochemical parameters provide support of an aetiologically and clinically relevant model of T2D that will serve as a valuable tool for mechanistic and therapeutic studies investigating the pathogenic complications of T2D. PMID:27402965

  12. Aronia melanocarpa Concentrate Ameliorates Pro-Inflammatory Responses in HaCaT Keratinocytes and 12-O-Tetradecanoylphorbol-13-Acetate-Induced Ear Edema in Mice.

    PubMed

    Goh, Ah Ra; Youn, Gi Soo; Yoo, Ki-Yeon; Won, Moo Ho; Han, Sang-Zin; Lim, Soon Sung; Lee, Keun Wook; Choi, Soo Young; Park, Jinseu

    2016-07-01

    Abnormal expression of pro-inflammatory mediators such as cell adhesion molecules and cytokines has been implicated in various inflammatory skin diseases, including atopic dermatitis. In this study, we investigated the anti-inflammatory activity of Aronia melanocarpa concentrate (AC) and its action mechanisms using in vivo and in vitro skin inflammation models. Topical application of AC on mouse ears significantly suppressed 12-O-tetradecanoylphorbol-13-acetate (TPA)-induced ear edema formation, as judged by measuring ear thickness and weight, and histological analysis. Topical administration of AC also reduced the expression of pro-inflammatory cytokines such as TNF-α, IL-1β, and IL-6 in TPA-stimulated mouse ears. Pretreatment with AC suppressed TNF-α-induced ICAM-I expression and subsequent monocyte adhesiveness in human keratinocyte cell line HaCaT. In addition, AC significantly decreased intracellular reactive oxygen species (ROS) generation as well as mitogen-activated protein kinase (MAPK) activation in TNF-α-stimulated HaCaT cells. AC and its constituent cyanidin 3-glucoside also attenuated TNF-α-induced IKK activation, IκB degradation, p65 phosphorylation/nuclear translocation, and p65 DNA binding activity in HaCaT cells. Overall, our results indicate that AC exerts anti-inflammatory activities by inhibiting expression of pro-inflammatory mediators in vitro and in vivo possibly through suppression of ROS-MAPK-NF-κB signaling pathways. Therefore, AC may be developed as a therapeutic agent to treat various inflammatory skin diseases.

  13. Responses of the ear to low frequency sounds, infrasound and wind turbines.

    PubMed

    Salt, Alec N; Hullar, Timothy E

    2010-09-01

    Infrasonic sounds are generated internally in the body (by respiration, heartbeat, coughing, etc) and by external sources, such as air conditioning systems, inside vehicles, some industrial processes and, now becoming increasingly prevalent, wind turbines. It is widely assumed that infrasound presented at an amplitude below what is audible has no influence on the ear. In this review, we consider possible ways that low frequency sounds, at levels that may or may not be heard, could influence the function of the ear. The inner ear has elaborate mechanisms to attenuate low frequency sound components before they are transmitted to the brain. The auditory portion of the ear, the cochlea, has two types of sensory cells, inner hair cells (IHC) and outer hair cells (OHC), of which the IHC are coupled to the afferent fibers that transmit "hearing" to the brain. The sensory stereocilia ("hairs") on the IHC are "fluid coupled" to mechanical stimuli, so their responses depend on stimulus velocity and their sensitivity decreases as sound frequency is lowered. In contrast, the OHC are directly coupled to mechanical stimuli, so their input remains greater than for IHC at low frequencies. At very low frequencies the OHC are stimulated by sounds at levels below those that are heard. Although the hair cells in other sensory structures such as the saccule may be tuned to infrasonic frequencies, auditory stimulus coupling to these structures is inefficient so that they are unlikely to be influenced by airborne infrasound. Structures that are involved in endolymph volume regulation are also known to be influenced by infrasound, but their sensitivity is also thought to be low. There are, however, abnormal states in which the ear becomes hypersensitive to infrasound. In most cases, the inner ear's responses to infrasound can be considered normal, but they could be associated with unfamiliar sensations or subtle changes in physiology. This raises the possibility that exposure to the

  14. Responses of the ear to low frequency sounds, infrasound and wind turbines.

    PubMed

    Salt, Alec N; Hullar, Timothy E

    2010-09-01

    Infrasonic sounds are generated internally in the body (by respiration, heartbeat, coughing, etc) and by external sources, such as air conditioning systems, inside vehicles, some industrial processes and, now becoming increasingly prevalent, wind turbines. It is widely assumed that infrasound presented at an amplitude below what is audible has no influence on the ear. In this review, we consider possible ways that low frequency sounds, at levels that may or may not be heard, could influence the function of the ear. The inner ear has elaborate mechanisms to attenuate low frequency sound components before they are transmitted to the brain. The auditory portion of the ear, the cochlea, has two types of sensory cells, inner hair cells (IHC) and outer hair cells (OHC), of which the IHC are coupled to the afferent fibers that transmit "hearing" to the brain. The sensory stereocilia ("hairs") on the IHC are "fluid coupled" to mechanical stimuli, so their responses depend on stimulus velocity and their sensitivity decreases as sound frequency is lowered. In contrast, the OHC are directly coupled to mechanical stimuli, so their input remains greater than for IHC at low frequencies. At very low frequencies the OHC are stimulated by sounds at levels below those that are heard. Although the hair cells in other sensory structures such as the saccule may be tuned to infrasonic frequencies, auditory stimulus coupling to these structures is inefficient so that they are unlikely to be influenced by airborne infrasound. Structures that are involved in endolymph volume regulation are also known to be influenced by infrasound, but their sensitivity is also thought to be low. There are, however, abnormal states in which the ear becomes hypersensitive to infrasound. In most cases, the inner ear's responses to infrasound can be considered normal, but they could be associated with unfamiliar sensations or subtle changes in physiology. This raises the possibility that exposure to the

  15. Development of Abnormal Hemispheric Vascular Networks Mimicking Cerebral Proliferative Angiopathy in a Child Originally Diagnosed with Deep-Seated Arteriovenous Fistula.

    PubMed

    Sakata, Hiroyuki; Fujimura, Miki; Sato, Kenichi; Niizuma, Kuniyasu; Endo, Hidenori; Tominaga, Teiji

    2016-10-01

    Cerebral proliferative angiopathy (CPA), which is characterized by diffuse vascular abnormalities with intermingled normal brain parenchyma, is a rare clinical entity distinct from classical cerebral arteriovenous malformations. Its pathology at initial state and subsequent course of progression has totally been undetermined. We herein presented a case of a child who was initially diagnosed with deep-seated arteriovenous fistula (AVF), and ultimately developed symptomatic CPA-like vascular lesion over a long period of clinical follow-up. A 7-month-old boy was incidentally found to have an AVF in the right basal ganglia and conservatively followed up. Serial magnetic resonance angiograms revealed the gradual proliferation and enlargement of pial and medullary vessels surrounding the AVF. Seven years later, he had a transient ischemic attack followed by intraventricular hemorrhage. A catheter angiogram showed a diffuse large vascular malformation composed of 2 distinct structures, including AVF in the right basal ganglia and the surrounding proliferated pial and medullary arteries in the right hemisphere. Single-photon emission computed tomography with N-isopropyl[123I]-p-iodoamphetamine revealed apparent hemodynamic compromise on the right hemisphere. Targeted embolization of the pseudoaneurysm originating from the right A1 perforator was performed to prevent rebleeding without complications. The patient had no further cerebrovascular events. Perinidal hypoperfusion induced by a deep-seated AVF could be one of the underlying pathologies of progressive angiogenic activity. This is the first case showing the development of abnormal hemispheric vascular networks mimicking CPA, which offers insight into the pathogenesis of this new entity.

  16. Germ cell specific overactivation of WNT/βcatenin signalling has no effect on folliculogenesis but causes fertility defects due to abnormal foetal development

    PubMed Central

    Kumar, Manish; Camlin, Nicole J.; Holt, Janet E.; Teixeira, Jose M.; McLaughlin, Eileen A.; Tanwar, Pradeep S.

    2016-01-01

    All the major components of the WNT signalling pathway are expressed in female germ cells and embryos. However, their functional relevance in oocyte biology is currently unclear. We examined ovaries collected from TCFGFP mice, a well-known Wnt reporter mouse model, and found dynamic changes in the Wnt/βcatenin signalling activity during different stages of oocyte development and maturation. To understand the functional importance of Wnt signalling in oocytes, we developed a mouse model with the germ cell-specific constitutive activation of βcatenin using cre recombinase driven by the DEAD (Asp-Glu-Ala-Asp) box protein 4 (Ddx4) gene promoter. Histopathological and functional analysis of ovaries from these mutant mice (Ctnnb1ex3cko) showed no defects in ovarian functions, oocytes, ovulation and early embryonic development. However, breeding of the Ctnnb1ex3cko female mice with males of known fertility never resulted in birth of mutant pups. Examination of uteri from time pregnant mutant females revealed defects in ectoderm differentiation leading to abnormal foetal development and premature death. Collectively, our work has established the role of active WNT/βcatenin signalling in oocyte biology and foetal development, and provides novel insights into the possible mechanisms of complications in human pregnancy such as repeated spontaneous abortion, sudden intrauterine unexpected foetal death syndrome and stillbirth. PMID:27265527

  17. Lack of Cul4b, an E3 Ubiquitin Ligase Component, Leads to Embryonic Lethality and Abnormal Placental Development

    PubMed Central

    Yuan, Jupeng; Qian, Yanyan; Sun, Wenjie; Zou, Yongxin; Guo, Chenhong; Chen, Bingxi; Shao, Changshun; Gong, Yaoqin

    2012-01-01

    Cullin-RING ligases (CRLs) complexes participate in the regulation of diverse cellular processes, including cell cycle progression, transcription, signal transduction and development. Serving as the scaffold protein, cullins are crucial for the assembly of ligase complexes, which recognize and target various substrates for proteosomal degradation. Mutations in human CUL4B, one of the eight members in cullin family, are one of the major causes of X-linked mental retardation. We here report the generation and characterization of Cul4b knockout mice, in which exons 3 to 5 were deleted. In contrast to the survival to adulthood of human hemizygous males with CUL4B null mutation, Cul4b null mouse embryos show severe developmental arrest and usually die before embryonic day 9.5 (E9.5). Accumulation of cyclin E, a CRL (CUL4B) substrate, was observed in Cul4b null embryos. Cul4b heterozygotes were recovered at a reduced ratio and exhibited a severe developmental delay. The placentas in Cul4b heterozygotes were disorganized and were impaired in vascularization, which may contribute to the developmental delay. As in human CUL4B heterozygotes, Cul4b null cells were selected against in Cul4b heterozygotes, leading to various degrees of skewed X-inactivation in different tissues. Together, our results showed that CUL4B is indispensable for embryonic development in the mouse. PMID:22606329

  18. Peripheral nerve regeneration in the MRL/MpJ ear wound model.

    PubMed

    Buckley, Gemma; Metcalfe, Anthony D; Ferguson, Mark W J

    2011-02-01

    The MRL/MpJ mouse displays an accelerated ability to heal ear punch wounds without scar formation (whereas wounds on the dorsal surface of the trunk heal with scar formation), offering a rare opportunity for studying tissue regeneration in adult mammals. A blastema-like structure develops and subsequently the structure of the wounded ear is restored, including cartilage, skin, hair follicles and adipose tissue. We sought to assess if the MRL/MpJ strain also possessed an enhanced capacity for peripheral nerve regeneration. Female MRL/MpJ and C57BL/6 mice were wounded with a 2-mm excisional biopsy punch to the centre of each ear and two 4-mm excisional biopsy punches to the dorsal skin. Immunohistochemical dual staining of pan-neurofilament and CD31 markers was used to investigate reinnervation and vascularisation of both the dorsal surface of the trunk and ear wounds. The MRL/MpJ mouse ear exhibited a significantly (P > 0.01) higher density of regenerated nerves than C57BL/6 between 10 and 21 days post-wounding when the blastema-like structure was forming. Unlike dorsal skin wounds, nerve regeneration in the ear wound preceded vascularisation, recapitulating early mammalian development. Immunohistochemical data suggest that factors within the blastemal mesenchyme, such as aggrecan, may direct nerve regrowth in the regenerating ear tissue.

  19. The Social Validity of Bug-in-Ear Coaching: Findings from Two Studies Implemented in Inclusive Early Childhood Environments

    ERIC Educational Resources Information Center

    Ottley, Jennifer Riggie; Coogle, Christan Grygas; Rahn, Naomi L.

    2015-01-01

    Coaching is a promising method for providing professional development, which takes many forms. One such form is real-time coaching through bug-in-ear technology. This study explored the social validity of bug-in-ear coaching when provided as a form of professional development with pre-service and in-service early childhood educators. Data from two…

  20. The Social Validity of Bug-in-Ear Coaching: Findings from Two Studies Implemented in Inclusive Early Childhood Environments

    ERIC Educational Resources Information Center

    Ottley, Jennifer R.; Coogle, Christan Grygas; Rahn, Naomi L.

    2015-01-01

    Coaching is a promising method for providing professional development, which takes many forms. One such form is real-time coaching through bug-in-ear technology. This study explored the social validity of bug-in-ear coaching when provided as a form of professional development with preservice and in-service early childhood educators. Data from two…

  1. Tooth - abnormal shape

    MedlinePlus

    Hutchinson incisors; Abnormal tooth shape; Peg teeth; Mulberry teeth; Conical teeth ... The appearance of normal teeth varies, especially the molars. ... conditions. Specific diseases can affect tooth shape, tooth ...

  2. Abnormal differentiation of dopaminergic neurons in zebrafish trpm7 mutant larvae impairs development of the motor pattern.

    PubMed

    Decker, Amanda R; McNeill, Matthew S; Lambert, Aaron M; Overton, Jeffrey D; Chen, Yu-Chia; Lorca, Ramón A; Johnson, Nicolas A; Brockerhoff, Susan E; Mohapatra, Durga P; MacArthur, Heather; Panula, Pertti; Masino, Mark A; Runnels, Loren W; Cornell, Robert A

    2014-02-15

    Transient receptor potential, melastatin-like 7 (Trpm7) is a combined ion channel and kinase implicated in the differentiation or function of many cell types. Early lethality in mice and frogs depleted of the corresponding gene impedes investigation of the functions of this protein particularly during later stages of development. By contrast, zebrafish trpm7 mutant larvae undergo early morphogenesis normally and thus do not have this limitation. The mutant larvae are characterized by multiple defects including melanocyte cell death, transient paralysis, and an ion imbalance that leads to the development of kidney stones. Here we report a requirement for Trpm7 in differentiation or function of dopaminergic neurons in vivo. First, trpm7 mutant larvae are hypomotile and fail to make a dopamine-dependent developmental transition in swim-bout length. Both of these deficits are partially rescued by the application of levodopa or dopamine. Second, histological analysis reveals that in trpm7 mutants a significant fraction of dopaminergic neurons lack expression of tyrosine hydroxylase, the rate-limiting enzyme in dopamine synthesis. Third, trpm7 mutants are unusually sensitive to the neurotoxin 1-methyl-4-phenylpyridinium, an oxidative stressor, and their motility is partially rescued by application of the iron chelator deferoxamine, an anti-oxidant. Finally, in SH-SY5Y cells, which model aspects of human dopaminergic neurons, forced expression of a channel-dead variant of TRPM7 causes cell death. In summary, a forward genetic screen in zebrafish has revealed that both melanocytes and dopaminergic neurons depend on the ion channel Trpm7. The mechanistic underpinning of this dependence requires further investigation.

  3. Thymidine Kinase 2 Deficiency-Induced Mitochondrial DNA Depletion Causes Abnormal Development of Adipose Tissues and Adipokine Levels in Mice

    PubMed Central

    Villarroya, Joan; Dorado, Beatriz; Vilà, Maya R.; Garcia-Arumí, Elena; Domingo, Pere; Giralt, Marta; Hirano, Michio; Villarroya, Francesc

    2011-01-01

    Mammal adipose tissues require mitochondrial activity for proper development and differentiation. The components of the mitochondrial respiratory chain/oxidative phosphorylation system (OXPHOS) are encoded by both mitochondrial and nuclear genomes. The maintenance of mitochondrial DNA (mtDNA) is a key element for a functional mitochondrial oxidative activity in mammalian cells. To ascertain the role of mtDNA levels in adipose tissue, we have analyzed the alterations in white (WAT) and brown (BAT) adipose tissues in thymidine kinase 2 (Tk2) H126N knockin mice, a model of TK2 deficiency-induced mtDNA depletion. We observed respectively severe and moderate mtDNA depletion in TK2-deficient BAT and WAT, showing both tissues moderate hypotrophy and reduced fat accumulation. Electron microscopy revealed altered mitochondrial morphology in brown but not in white adipocytes from TK2-deficient mice. Although significant reduction in mtDNA-encoded transcripts was observed both in WAT and BAT, protein levels from distinct OXPHOS complexes were significantly reduced only in TK2-deficient BAT. Accordingly, the activity of cytochrome c oxidase was significantly lowered only in BAT from TK2-deficient mice. The analysis of transcripts encoding up to fourteen components of specific adipose tissue functions revealed that, in both TK2-deficient WAT and BAT, there was a consistent reduction of thermogenesis related gene expression and a severe reduction in leptin mRNA. Reduced levels of resistin mRNA were found in BAT from TK2-deficient mice. Analysis of serum indicated a dramatic reduction in circulating levels of leptin and resistin. In summary, our present study establishes that mtDNA depletion leads to a moderate impairment in mitochondrial respiratory function, especially in BAT, causes substantial alterations in WAT and BAT development, and has a profound impact in the endocrine properties of adipose tissues. PMID:22216345

  4. Abnormal pituitary development and function in three siblings of a Jamaican family: A new syndrome involving the Pit-1 gene

    SciTech Connect

    Sanchez, J.C.; Schiavi, A.; Parks, J.

    1994-09-01

    In 1967 Mckusick et al. reported three siblings in Canada who had combine pituitary hormone deficiencies (CPHD). Since that report there have been several families with multiple affected members who share the common characteristics of autosomal recessive inheritance and clinical expression of pituitary deficiencies at an early age. We report here a CPHD family of Jamaican origin with three affected and two unaffected siblings. The affected siblings have evidence of severe growth failure, growth hormone deficiency, hypothyroidism and variable prolactin deficiency. Recently, in some families with CPHD a defect has been detected in the Pit-1 gene, which encodes a transcription factor involved in the differentiation of the pituitary and the production of growth hormone, TSH and prolactin. We are studying the Pit-1 gene in this family as a candidate gene that may explain the family phenotype. The Pit-1 gene has been analyzed in DNA extracted from blood. No gross deletion were detected in exons 2, 3, 4, 5 and 6 using exon-specific PCR assay developed in our laboratory. Exon 1 is also currently being analyzed. Single stand conformational polymorphism (SSCP) analysis, a screening technique for point mutations within genes, is being used to identify putative base pair changes in the Pit-1 gene. The exon findings will be confirmed using standard DNA sequencing procedures. If a Pit-1 gene is detected, this family would provide a novel presentation, since gonadotropin deficiency appears to be present. Alternatively, this family may represent a mutation on another yet unknown factor involved in normal pituitary development.

  5. Using the shortwave infrared to image middle ear pathologies.

    PubMed

    Carr, Jessica A; Valdez, Tulio A; Bruns, Oliver T; Bawendi, Moungi G

    2016-09-01

    Visualizing structures deep inside opaque biological tissues is one of the central challenges in biomedical imaging. Optical imaging with visible light provides high resolution and sensitivity; however, scattering and absorption of light by tissue limits the imaging depth to superficial features. Imaging with shortwave infrared light (SWIR, 1-2 μm) shares many advantages of visible imaging, but light scattering in tissue is reduced, providing sufficient optical penetration depth to noninvasively interrogate subsurface tissue features. However, the clinical potential of this approach has been largely unexplored because suitable detectors, until recently, have been either unavailable or cost prohibitive. Here, taking advantage of newly available detector technology, we demonstrate the potential of SWIR light to improve diagnostics through the development of a medical otoscope for determining middle ear pathologies. We show that SWIR otoscopy has the potential to provide valuable diagnostic information complementary to that provided by visible pneumotoscopy. We show that in healthy adult human ears, deeper tissue penetration of SWIR light allows better visualization of middle ear structures through the tympanic membrane, including the ossicular chain, promontory, round window niche, and chorda tympani. In addition, we investigate the potential for detection of middle ear fluid, which has significant implications for diagnosing otitis media, the overdiagnosis of which is a primary factor in increased antibiotic resistance. Middle ear fluid shows strong light absorption between 1,400 and 1,550 nm, enabling straightforward fluid detection in a model using the SWIR otoscope. Moreover, our device is easily translatable to the clinic, as the ergonomics, visual output, and operation are similar to a conventional otoscope. PMID:27551085

  6. Middle-ear microsurgery simulation to improve new robotic procedures.

    PubMed

    Kazmitcheff, Guillaume; Nguyen, Yann; Miroir, Mathieu; Péan, Fabien; Ferrary, Evelyne; Cotin, Stéphane; Sterkers, Olivier; Duriez, Christian

    2014-01-01

    Otological microsurgery is delicate and requires high dexterity in bad ergonomic conditions. To assist surgeons in these indications, a teleoperated system, called RobOtol, is developed. This robot enhances gesture accuracy and handiness and allows exploration of new procedures for middle ear surgery. To plan new procedures that exploit the capacities given by the robot, a surgical simulator is developed. The simulation reproduces with high fidelity the behavior of the anatomical structures and can also be used as a training tool for an easier control of the robot for surgeons. In the paper, we introduce the middle ear surgical simulation and then we perform virtually two challenging procedures with the robot. We show how interactive simulation can assist in analyzing the benefits of robotics in the case of complex manipulations or ergonomics studies and allow the development of innovative surgical procedures. New robot-based microsurgical procedures are investigated. The improvement offered by RobOtol is also evaluated and discussed.

  7. Assessment of electron beam-induced abnormal development and DNA damage in Spodoptera litura (F.) (Lepidoptera: Noctuidae)

    NASA Astrophysics Data System (ADS)

    Yun, Seung-Hwan; Lee, Seon-Woo; Koo, Hyun-Na; Kim, Gil-Hah

    2014-03-01

    The armyworm, Spodoptera litura (F.) is a polyphagous and important agricultural pest worldwide. In this study, we examined the effect of electron beam irradiation on developmental stages, reproduction, and DNA damage of S. litura. Eggs (0-24 h old), larvae (3rd instar), pupae (3 days old after pupation), and adults (24 h after emergence) were irradiated with electron beam irradiation of six levels between 30 and 250 Gy. When eggs were irradiated with 100 Gy, egg hatching was completely inhibited. When the larvae were irradiated, the larval period was significantly delayed, depending on the doses applied. At 150 Gy, the fecundity of adults that developed from irradiated pupae was entirely inhibited. However, electron beam irradiation did not induce the instantaneous death of S. litura adults. Reciprocal crosses between irradiated and unirradiated moths demonstrated that females were more radiosensitive than males. We also conducted the comet assay immediately after irradiation and over the following 5 days period. Severe DNA fragmentation in S. litura cells was observed just after irradiation and the damage was repaired during the post-irradiation period in a time-dependent manner. However, at more than 100 Gy, DNA damage was not fully recovered.

  8. Assessment of abnormal blood flow and efficacy of treatment in patients with systemic sclerosis using a newly developed microwireless laser Doppler flowmeter and arm-raising test.

    PubMed

    Kido, M; Takeuchi, S; Hayashida, S; Urabe, K; Sawada, R; Furue, M

    2007-10-01

    Background Patients with systemic sclerosis (SSc) frequently suffer from recalcitrant digital ulceration because of impaired cutaneous blood flow (CBF). A simple and accurate CBF measurement would be helpful to evaluate the disease status and efficacy of treatment in such patients. Objectives To examine the feasibility of a newly developed, micromachined integrated laser blood flowmeter (MILBF) for evaluation of abnormal CBF responses in patients with SSc. Methods CBF of finger pulp was measured in eight patients with SSc and in six healthy controls using MILBF. CBF in the steady state and the responses to the arm-raising test and cold provocation were assessed. The therapeutic efficacy of a single and an intensive prostaglandin E(1) (PGE(1)) infusion treatment was also evaluated in some of the SSc patients. Results The patients with SSc showed significantly lower steady-state CBF than controls. The rate of blood flow with cold provocation and the velocity of blood flow recovery after cold provocation (VR-CP) tended to be lower in patients with SSc. Augmentation of amplitude of the digital pulse wave by arm raising (AA-AR) was observed in controls, but not in patients with SSc. We also found that VR-CP and AA-AR may be good markers for evaluating the efficacy of vasodilatory treatment. It should be noted that the examined patients did not complain of any pain and/or distress during the arm-raising test, as opposed to during cold provocation. Conclusions CBF assessment using MILBF and an arm-raising test is accurate, noninvasive and well tolerated and thus the combination may be a better alternative method to evaluate abnormal CBF and efficacy of treatment in patients with SSc.

  9. Chemical Leucoderma Induced by Ear-ring Stoppers Made of Polyvinyl Chloride

    PubMed Central

    Sharma, Reena; Singal, Archana; Verma, Prashant; Grover, Chander

    2012-01-01

    We report a case of chemical leucoderma (CL) in a 15-year-old girl, who developed patterned depigmentation at the back of both ear lobules after contact with plastic ear-ring stoppers made of polyvinyl chloride (PVC) after continuous use for 6–7 months. Patch test with Indian standard series and cosmetic series was negative after 48 h, but she refused patch testing for extended duration as the possibility of induced depigmentation at the test site was unacceptable to her. To the best of our knowledge, this is the first report of plastic ear-ring stopper induced CL. PMID:23060712

  10. Birth and Evolution of Chiselling and Drilling Techniques for Removing Ear Canal Exostoses.

    PubMed

    Mudry, Albert; Hetzler, Douglas

    2016-01-01

    The main surgical techniques used to remove ear canal exostoses are drilling and/or, chiselling. The aim of this study was to identify the origins and subsequent evolution of, the surgical removal of ear canal exostoses in the 19th century. A critical review and, compilation of primary and secondary historical sources was conducted. Two techniques for removal of exostoses were developed in the latter part of the 19th century and have largely remained unchanged. This demonstrates the importance of that era in the history of ear surgery. PMID:26649611

  11. Glial choristoma of the middle ear.

    PubMed

    Shemanski, Karen A; Voth, Spencer E; Patitucci, Lana B; Ma, Yuxiang; Popnikolov, Nikolay; Katsetos, Christos D; Sataloff, Robert T

    2013-12-01

    Glial choristomas are isolated masses of mature brain tissue that are found outside the spinal cord or cranial cavity. These masses are rare, especially in the middle ear. We describe the case of an 81-year-old man who presented with left-sided chronic otitis media, mastoiditis, hearing loss, tinnitus, and aural fullness. He was found to have a glial choristoma of the middle ear on the left. Otologic surgeons should be aware of the possibility of finding such a mass in the middle ear and be familiar with the differences in treatment between glial choristomas and the more common encephaloceles.

  12. Surgical Management of Ear Diseases in Rabbits.

    PubMed

    Csomos, Rebecca; Bosscher, Georgia; Mans, Christoph; Hardie, Robert

    2016-01-01

    Otitis externa and media are frequently diagnosed disorders in rabbits and are particularly common in lop-eared breeds because of the specific anatomy of the ear canal. Medical management for otitis externa and media often provides only a temporary improvement in clinical signs. Surgery by means of partial or total ear canal ablation (PECA or TECA) combined with lateral bulla osteotomy (LBO) represents a feasible approach that is well tolerated and provides a good clinical outcome. Short-term complications associated with PECA/TECA-LBO include facial nerve paralysis and vestibular disease.

  13. [Ear keloid and clinical research progress].

    PubMed

    Du, Guangyuan; Zhu, Jiang

    2014-04-01

    Keloid refers to the damaged skin due to excessive fibroblast proliferation. Ear is one predilection site. The pathogenesis of ear keloid is not very clear, and the treatment is also varied. Surgery, postoperative radiotherapy and laser treatment, steroid hormones, pressure therapy are the basic treatment methods. Integrated application of a variety of treatments, classification research and new materials using revealed the prospect for the treatment of the disease. This thesis reviews literature about ear keloid in recent 10 years, and introduces this disease and clinical research progress.

  14. EARS: Electronic Access to Reference Service.

    PubMed Central

    Weise, F O; Borgendale, M

    1986-01-01

    Electronic Access to Reference Service (EARS) is a front end to the Health Sciences Library's electronic mail system, with links to the online public catalog. EARS, which became operational in September 1984, is accessed by users at remote sites with either a terminal or microcomputer. It is menu-driven, allowing users to request: a computerized literature search, reference information, a photocopy of a journal article, or a book. This paper traces the history of EARS and discusses its use, its impact on library staff and services, and factors that influence the diffusion of new technology. PMID:3779167

  15. Complex patterns of abnormal heartbeats

    NASA Technical Reports Server (NTRS)

    Schulte-Frohlinde, Verena; Ashkenazy, Yosef; Goldberger, Ary L.; Ivanov, Plamen Ch; Costa, Madalena; Morley-Davies, Adrian; Stanley, H. Eugene; Glass, Leon

    2002-01-01

    Individuals having frequent abnormal heartbeats interspersed with normal heartbeats may be at an increased risk of sudden cardiac death. However, mechanistic understanding of such cardiac arrhythmias is limited. We present a visual and qualitative method to display statistical properties of abnormal heartbeats. We introduce dynamical "heartprints" which reveal characteristic patterns in long clinical records encompassing approximately 10(5) heartbeats and may provide information about underlying mechanisms. We test if these dynamics can be reproduced by model simulations in which abnormal heartbeats are generated (i) randomly, (ii) at a fixed time interval following a preceding normal heartbeat, or (iii) by an independent oscillator that may or may not interact with the normal heartbeat. We compare the results of these three models and test their limitations to comprehensively simulate the statistical features of selected clinical records. This work introduces methods that can be used to test mathematical models of arrhythmogenesis and to develop a new understanding of underlying electrophysiologic mechanisms of cardiac arrhythmia.

  16. Generation of inner ear sensory epithelia from pluripotent stem cells in 3D culture.

    PubMed

    Koehler, Karl R; Mikosz, Andrew M; Molosh, Andrei I; Patel, Dharmeshkumar; Hashino, Eri

    2013-08-01

    The inner ear contains sensory epithelia that detect head movements, gravity and sound. It is unclear how to develop these sensory epithelia from pluripotent stem cells, a process that will be critical for modelling inner ear disorders or developing cell-based therapies for profound hearing loss and balance disorders. So far, attempts to derive inner ear mechanosensitive hair cells and sensory neurons have resulted in inefficient or incomplete phenotypic conversion of stem cells into inner-ear-like cells. A key insight lacking from these previous studies is the importance of the non-neural and preplacodal ectoderm, two critical precursors during inner ear development. Here we report the stepwise differentiation of inner ear sensory epithelia from mouse embryonic stem cells (ESCs) in three-dimensional culture. We show that by recapitulating in vivo development with precise temporal control of signalling pathways, ESC aggregates transform sequentially into non-neural, preplacodal and otic-placode-like epithelia. Notably, in a self-organized process that mimics normal development, vesicles containing prosensory cells emerge from the presumptive otic placodes and give rise to hair cells bearing stereocilia bundles and a kinocilium. Moreover, these stem-cell-derived hair cells exhibit functional properties of native mechanosensitive hair cells and form specialized synapses with sensory neurons that have also arisen from ESCs in the culture. Finally, we demonstrate how these vesicles are structurally and biochemically comparable to developing vestibular end organs. Our data thus establish a new in vitro model of inner ear differentiation that can be used to gain deeper insight into inner ear development and disorder. PMID:23842490

  17. Injuries of the external ear.

    PubMed

    Templer, J; Renner, G J

    1990-10-01

    Ear injuries occur in people of all ages but predominate in active people such as wrestlers, boxers, and bike riders. The types and extent of injury are a function of the force causing the injury. Shearing forces of moderate intensity cause hematoma formation, whereas greater force causes lacerations or even amputation. Sharp objects cause lacerations determined by the force, direction, and point of impact. The high ratio of surface area to mass makes the auricle vulnerable to extremes of temperature. People participating in high-risk activities should wear protective headgear. The goal of treatment is to restore the normal contours while preventing infection. Hematoma results in disfigurement by organization or chondritis. Evacuation and pressure dressings using sterile technique correct the condition. Second-degree burns are treated by regular cleansing and application of topical antimicrobials. Deeper burns require debridement, biologic dressings, or burying the cartilage subcutaneously for later reconstruction. Simple lacerations are closed under aseptic technique using either skin-to-skin sutures only or sutures of the skin combined with intercartilage sutures. Extensive and complex lacerations require meticulous care to match all fragments and prevent infection or loss of tissue. Bare cartilage must be covered with vascularized tissue. The treatment of total amputation is controversial. Some advocate reattachment as a composite graft using intravenous low molecular weight dextrans and heparin as adjuvants. Mladick dermabrades the amputated pinna, reattaches it with sutures, and then slips it into a pocket of elevated postauricular skin for 2 weeks. Others urge microvascular reanastomosis of the small nutrient vessels. Brent and Byrd separate the cartilage from its overlying skin and envelope it first with vascularized temporoparietal fascia and then a split-thickness skin graft. Chondritis is the most feared complication of injury or surgery of the pinna. It

  18. Finite-element analysis of earing using non-quadratic yield surfaces

    SciTech Connect

    Logan, R.W.

    1995-06-18

    During deep draw cupping, the phenomenon known as earing may occur as the cup wall is formed, resulting in a periodic variation of cup wall height around the perimeter of the finished cup. This is generally due to planar anisotropy of flow in rolled sheet product. It is generally observed that the anisotropy parameter R will vary in the plane of the sheet when ears are observed in cupping, with a parameter {Delta}R describing the variation of R in the plane of the sheet. For many common textures in face-centered and body-centered materials, the ears form relative to the sheet rolling direction at 0{degrees} and 90{degrees} around the perimeter if {Delta}R>0, and at -45{degrees} and +45{degrees} if {Delta}R<0. There is extensive experimental evidence that ear height shows a linear correlation with {Delta}R/R, but attempts to duplicate this using the finite-element method are highly dependent on both the methodology and yield surface used. It was shown previously that using a coarse mesh and the quadratic Hill yield surface tends to greatly under predict earing. In this study, we have used two different finite-element codes developed at LLNL to examine the predicted earing using both quadratic Hill and alternative non-quadratic yield surfaces. These results are compared to experimental data and conclusions drawn about the most desirable closed-form yield surfaces to duplicate the observed earing phenomena.

  19. Structurally abnormal human autosomes

    SciTech Connect

    1993-12-31

    Chapter 25, discusses structurally abnormal human autosomes. This discussion includes: structurally abnormal chromosomes, chromosomal polymorphisms, pericentric inversions, paracentric inversions, deletions or partial monosomies, cri du chat (cat cry) syndrome, ring chromosomes, insertions, duplication or pure partial trisomy and mosaicism. 71 refs., 8 figs.

  20. Curcumin prevents haloperidol-induced development of abnormal oro-facial movements: possible implications of Bcl-XL in its mechanism of action.

    PubMed

    Sookram, Christal; Tan, Mattea; Daya, Ritesh; Heffernan, Spencer; Mishra, Ram K

    2011-08-01

    Curcumin (Curcuma Longa Linn), the active component of turmeric, has been shown to be effective in ameliorating several stress and drug-induced disorders in rats and humans. However, it is unclear whether short term curcumin administration can prevent the abnormal oro-facial movements (AOFM) which develop following blockade of dopamine D2 receptors by antagonist such as Haloperidol. The objective of this study is to determine whether short term treatment with curcumin along with Haloperidol can prevent the development of AOFM in rats. Male Sprague Dawley rats were administered curcumin at 200 mg/kg, and Haloperidol at 2 mg/kg daily for 2 weeks, and AOFMs and locomotor activity were assessed at baseline, day 7 and day 14. By day 14, rats receiving concurrent curcumin administration had a significant reduction in the incidence of Haloperidol-induced AOFMs, but no change on the Haloperidol-induced hypolocomotion. There was no spiked increase in locomotor activity in absence of challenge with dopamine D2 receptor agonist. The exact mechanism by which curcumin attenuates AOFMs remains unknown, therefore, we performed a proteomic analysis of the striatal samples obtained from control and curcumin treated groups. A number of proteins were altered by curcumin, among them an antiapoptotic protein, Bcl-XL, was significantly upregulated. These results suggest that curcumin may be a promising treatment to prevent the development of AOFMs and further suggest some therapeutic value in the treatment of movement disorders. PMID:21218454

  1. Abnormal mineralization of the Ts65Dn Down syndrome mouse appendicular skeleton begins during embryonic development in a Dyrk1a-independent manner.

    PubMed

    Blazek, Joshua D; Malik, Ahmed M; Tischbein, Maeve; Arbones, Maria L; Moore, Clara S; Roper, Randall J

    2015-05-01

    The relationship between gene dosage imbalance and phenotypes associated with Trisomy 21, including the etiology of abnormal bone phenotypes linked to Down syndrome (DS), is not well understood. The Ts65Dn mouse model for DS exhibits appendicular skeletal defects during adolescence and adulthood but the developmental and genetic origin of these phenotypes remains unclear. It is hypothesized that the postnatal Ts65Dn skeletal phenotype originates during embryonic development and results from an increased Dyrk1a gene copy number, a gene hypothesized to play a critical role in many DS phenotypes. Ts65Dn embryos exhibit a lower percent bone volume in the E17.5 femur when compared to euploid embryos. Concomitant with gene copy number, qPCR analysis revealed a  ~1.5 fold increase in Dyrk1a transcript levels in the Ts65Dn E17.5 embryonic femur as compared to euploid. Returning Dyrk1a copy number to euploid levels in Ts65Dn, Dyrk1a(+/-) embryos did not correct the trisomic skeletal phenotype but did return Dyrk1a gene transcript levels to normal. The size and protein expression patterns of the cartilage template during embryonic bone development appear to be unaffected at E14.5 and E17.5 in trisomic embryos. Taken together, these data suggest that the dosage imbalance of genes other than Dyrk1a is involved in the development of the prenatal bone phenotype in Ts65Dn embryos. PMID:25556111

  2. Ectopic expression of an apple apomixis-related gene MhFIE induces co-suppression and results in abnormal vegetative and reproductive development in tomato.

    PubMed

    Liu, Dan-Dan; Dong, Qing-Long; Fang, Mou-Jing; Chen, Ke-Qin; Hao, Yu-Jin

    2012-12-15

    It has been well documented that FERTILIZATION-INDEPENDENT ENDOSPERM (FIE) plays important regulatory roles in diverse developmental processes in model plant Arabidopsis thaliana. However, it is largely unknown how FIE genes function in economically important crops. In this study, MhFIE gene, which was previously isolated from apomictic tea crabapple (Malus hupehensis Redh. var. pingyiensis), was introduced into tomato. The hemizygous transgenic tomato lines produced curly leaves and decreased in seed germination. In addition, the co-suppression of the transgenic MhFIE and endogenous (SlFIE) genes occurred in homozygous transgenic tomatoes. As a result, FIE silencing brought about abnormal phenotypes during reproductive development in tomato, such as increased sepal and petal numbers in flower, a fused ovule and pistil and parthenocarpic fruit formation. A yeast two-hybrid assay and bimolecular fluorescence complementation (BiFC) demonstrated that MhFIE interacted with a tomato protein, EZ2 (SlEZ2). Its ectopic expression and SlFIE co-suppression notably influenced the expression of genes associated with leaf, flower, and fruit development. Therefore, together with other PcG proteins, FIE was involved in the regulation of vegetative and reproductive development by modulating the expression of related genes in plants.

  3. Morphological abnormalities among lampreys

    USGS Publications Warehouse

    Manion, Patrick J.

    1967-01-01

    The experimental control of the sea lamprey (Petromyzon marinus) in the Great Lakes has required the collection of thousands of lampreys. Representatives of each life stage of the four species of the Lake Superior basin were examined for structural abnormalities. The most common aberration was the presence of additional tails. The accessory tails were always postanal and smaller than the normal tail. The point of origin varied; the extra tails occurred on dorsal, ventral, or lateral surfaces. Some of the extra tails were misshaped and curled, but others were normal in shape and pigment pattern. Other abnormalities in larval sea lampreys were malformed or twisted tails and bodies. The cause of the structural abnormalities is unknown. The presence of extra caudal fins could be genetically controlled, or be due to partial amputation or injury followed by abnormal regeneration. Few if any lampreys with structural abnormalities live to sexual maturity.

  4. Autograft ossicle selection in cholesteatomatous ear disease: histopathological considerations.

    PubMed

    Rupa, V; Krishnaswami, H; Job, A

    1997-09-01

    In order to determine whether selection of autograft ossicles in cholesteatomatous ear disease should be based upon their appearance under the surgical operating microscope, we studied the histopathological features of 113 such ossicles. We attempted to correlate the extent of erosion of the ossicle, as noted under the surgical operating microscope, with their histopathological appearance. There were 60 mallei and 53 includes. Seventy-nine ossicles were eroded and 34 were intact. The commonest abnormality noted was erosion of the long process of the incus (75 per cent). Both intact and eroded ossicles had similar histological features. There was no evidence of intra-ossicular cholesteatoma. The results suggest that the extent of erosion of these ossicles as seen under the surgical operating microscope should in no way prejudice their use as autografts when required. PMID:9373543

  5. Developmental evolutionary biology of the vertebrate ear: conserving mechanoelectric transduction and developmental pathways in diverging morphologies

    NASA Technical Reports Server (NTRS)

    Fritzsch, B.; Beisel, K. W.; Bermingham, N. A.

    2000-01-01

    This brief overview shows that a start has been made to molecularly dissect vertebrate ear development and its evolutionary conservation to the development of the insect hearing organ. However, neither the patterning process of the ear nor the patterning process of insect sensory organs is sufficiently known at the moment to provide more than a first glimpse. Moreover, hardly anything is known about otocyst development of the cephalopod molluscs, another triploblast lineage that evolved complex 'ears'. We hope that the apparent conserved functional and cellular components present in the ciliated sensory neurons/hair cells will also be found in the genes required for vertebrate ear and insect sensory organ morphogenesis (Fig. 3). Likewise, we expect that homologous pre-patterning genes will soon be identified for the non-sensory cell development, which is more than a blocking of neuronal development through the Delta/Notch signaling system. Generation of the apparently unique ear could thus represent a multiplication of non-sensory cells by asymmetric and symmetric divisions as well as modification of existing patterning process by implementing novel developmental modules. In the final analysis, the vertebrate ear may come about by increasing the level of gene interactions in an already existing and highly conserved interactive cascade of bHLH genes. Since this was apparently achieved in all three lineages of triploblasts independently (Fig. 3), we now need to understand how much of the morphogenetic cascades are equally conserved across phyla to generate complex ears. The existing mutations in humans and mice may be able to point the direction of future research to understand the development of specific cell types and morphologies in the formation of complex arthropod, cephalopod, and vertebrate 'ears'.

  6. Sensory afferent segregation in three-eared frogs resemble the dominance columns observed in three-eyed frogs

    PubMed Central

    Elliott, Karen L.; Houston, Douglas W.; Fritzsch, Bernd

    2015-01-01

    The formation of proper sensory afferent connections during development is essential for brain function. Activity-based competition is believed to drive ocular dominance columns (ODC) in mammals and in experimentally-generated three-eyed frogs. ODC formation is thus a compromise of activity differences between two eyes and similar molecular cues. To gauge the generality of graphical map formation in the brain, we investigated the inner ear projection, known for its well-defined and early segregation of afferents from vestibular and auditory endorgans. In analogy to three eyed-frogs, we generated three-eared frogs to assess to what extent vestibular afferents from two adjacent ears could segregate. Donor ears were transplanted either in the native orientation or rotated by 90 degrees. These manipulations should result in either similar or different induced activity between both ears, respectively. Three-eared frogs with normal orientation showed normal swimming whereas those with a rotated third ear showed aberrant behaviors. Projection studies revealed that only afferents from the rotated ears segregated from those from the native ear within the vestibular nucleus, resembling the ocular dominance columns formed in three-eyed frogs. Vestibular segregation suggests that mechanisms comparable to those operating in the ODC formation of the visual system may act on vestibular projection refinements. PMID:25661240

  7. Directional Sensitivity of the Human Ear.

    ERIC Educational Resources Information Center

    Pitt, John D.; Bazley, Martin

    1985-01-01

    Presents a classroom experiment that demonstrates the directional sensitivity of the human ear. Outlines the activity's procedures and provides a diagrammatical view of the experimental arrangement. Also included is an analysis of the expected results. (ML)

  8. Neonatal Ear Molding: Timing and Technique.

    PubMed

    Anstadt, Erin Elizabeth; Johns, Dana Nicole; Kwok, Alvin Chi-Ming; Siddiqi, Faizi; Gociman, Barbu

    2016-03-01

    The incidence of auricular deformities is believed to be ∼11.5 per 10,000 births, excluding children with microtia. Although not life-threatening, auricular deformities can cause undue distress for patients and their families. Although surgical procedures have traditionally been used to reconstruct congenital auricular deformities, ear molding has been gaining acceptance as an efficacious, noninvasive alternative for the treatment of newborns with ear deformations. We present the successful correction of bilateral Stahl's ear deformity in a newborn through a straightforward, nonsurgical method implemented on the first day of life. The aim of this report is to make pediatric practitioners aware of an effective and simple molding technique appropriate for correction of congenital auricular anomalies. In addition, it stresses the importance of very early initiation of ear cartilage molding for achieving the desired outcome.

  9. Mozart ear: diagnosis, treatment, and literature review.

    PubMed

    Yamashita, Ken; Yotsuyanagi, Takatoshi; Saito, Tamotsu; Isogai, Noritaka; Mori, Hiromasa; Itani, Yoshihito

    2011-11-01

    Mozart ear is a congenital auricular deformity, which is mainly characterized by a bulging appearance of the anterosuperior portion of the auricle, a convexly protruded cavum conchae, and a slit-like narrowing of the orifice of the external auditory meatus. It is said to be uncommon, and because no one has yet fully described neither the disease nor the treatment, the concept of Mozart ear has not been unified. This report describes a case of a 13-year-old girl presented with an unusual congenital deformity which showed the features of Mozart ear. It is an extremely rare deformity that only about 4 clinical cases have been reported in medical literature thereby a treatment method has not been fully discussed. For surgical correction of our cases, we excised deformed conchal cartilage, turned it over, regrafted, and maintained a cosmetically positive result. We also reviewed and described the origin, current concept, and treatment method of Mozart ear.

  10. Evolution: Fossil Ears and Underwater Sonar.

    PubMed

    Lambert, Olivier

    2016-08-22

    A key innovation in the history of whales was the evolution of a sonar system together with high-frequency hearing. Fossils of an archaic toothed whale's inner ear bones provide clues for a stepwise emergence of underwater echolocation ability.

  11. Middle Ear Adenoma: Case Report and Discussion

    PubMed Central

    Vrugt, B.; Huber, A. M.

    2014-01-01

    Introduction. Despite modern radiological workup, surgeons can still be surprised by intraoperative findings or by the pathologist's report. Materials & Methods. We describe the case of a 52-year-old male who was referred to our clinic with a single sided conductive hearing loss. He ultimately underwent middle ear exploration and excision of a middle ear tumour followed by second look and ossiculoplasty a year later. Results. Though preoperative CT and MRI scanning were suggestive of a congenital cholesteatoma, the pathologist's report diagnosed a middle ear adenoma. Discussion. Middle ear glandular tumors are extremely rare and, despite numerous histological techniques, continue to defy satisfactory classification. Most surgeons advocate surgical excision though evidence of the tumour's natural course and risk of recurrence is lacking. PMID:25045567

  12. Ear Infections in Autistic and Normal Children. Brief Report.

    ERIC Educational Resources Information Center

    Konstantareas, M. Mary; Homatidis, Soula

    1987-01-01

    Evaluation of the frequency of ear infections, ear tube drainage, and deafness for 51 autistic children (ages 2-18) indicated that autistic children had a greater incidence of ear infections than matched normal peers and lower functioning children had an earlier onset of ear infections than higher functioning autistic peers. (Author)

  13. An analysis of the acoustic input impedance of the ear.

    PubMed

    Withnell, Robert H; Gowdy, Lauren E

    2013-10-01

    Ear canal acoustics was examined using a one-dimensional lossy transmission line with a distributed load impedance to model the ear. The acoustic input impedance of the ear was derived from sound pressure measurements in the ear canal of healthy human ears. A nonlinear least squares fit of the model to data generated estimates for ear canal radius, ear canal length, and quantified the resistance that would produce transmission losses. Derivation of ear canal radius has application to quantifying the impedance mismatch at the eardrum between the ear canal and the middle ear. The length of the ear canal was found, in general, to be longer than the length derived from the one-quarter wavelength standing wave frequency, consistent with the middle ear being mass-controlled at the standing wave frequency. Viscothermal losses in the ear canal, in some cases, may exceed that attributable to a smooth rigid wall. Resistance in the middle ear was found to contribute significantly to the total resistance. In effect, this analysis "reverse engineers" physical parameters of the ear from sound pressure measurements in the ear canal.

  14. 38 CFR 4.87 - Schedule of ratings-ear.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 1 2014-07-01 2014-07-01 false Schedule of ratings-ear...—ear. Diseases of the Ear Rating 6200Chronic suppurative otitis media, mastoiditis, or cholesteatoma... of the substance 10 6208Malignant neoplasm of the ear (other than skin only) 100 Note: A rating...

  15. 21 CFR 874.4140 - Ear, nose, and throat bur.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ... 21 Food and Drugs 8 2014-04-01 2014-04-01 false Ear, nose, and throat bur. 874.4140 Section 874...) MEDICAL DEVICES EAR, NOSE, AND THROAT DEVICES Surgical Devices § 874.4140 Ear, nose, and throat bur. (a) Identification. An ear, nose, and throat bur is a device consisting of an interchangeable drill bit that...

  16. 21 CFR 874.3430 - Middle ear mold.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ... 21 Food and Drugs 8 2013-04-01 2013-04-01 false Middle ear mold. 874.3430 Section 874.3430 Food... DEVICES EAR, NOSE, AND THROAT DEVICES Prosthetic Devices § 874.3430 Middle ear mold. (a) Identification. A middle ear mold is a preformed device that is intended to be implanted to reconstruct the middle...

  17. 38 CFR 4.87 - Schedule of ratings-ear.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 1 2011-07-01 2011-07-01 false Schedule of ratings-ear...—ear. Diseases of the Ear Rating 6200Chronic suppurative otitis media, mastoiditis, or cholesteatoma... of the substance 10 6208Malignant neoplasm of the ear (other than skin only) 100 Note: A rating...

  18. 38 CFR 4.87 - Schedule of ratings-ear.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 1 2012-07-01 2012-07-01 false Schedule of ratings-ear...—ear. Diseases of the Ear Rating 6200Chronic suppurative otitis media, mastoiditis, or cholesteatoma... of the substance 10 6208Malignant neoplasm of the ear (other than skin only) 100 Note: A rating...

  19. 21 CFR 874.4140 - Ear, nose, and throat bur.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ... 21 Food and Drugs 8 2012-04-01 2012-04-01 false Ear, nose, and throat bur. 874.4140 Section 874...) MEDICAL DEVICES EAR, NOSE, AND THROAT DEVICES Surgical Devices § 874.4140 Ear, nose, and throat bur. (a) Identification. An ear, nose, and throat bur is a device consisting of an interchangeable drill bit that...

  20. 38 CFR 4.87 - Schedule of ratings-ear.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 1 2010-07-01 2010-07-01 false Schedule of ratings-ear...—ear. Diseases of the Ear Rating 6200Chronic suppurative otitis media, mastoiditis, or cholesteatoma... of the substance 10 6208Malignant neoplasm of the ear (other than skin only) 100 Note: A rating...