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1

Postnatal growth retardation, facial dysmorphism, spondylocarpal synostosis, cardiac defect, and inner ear malformation (cardiospondylocarpofacial syndrome?)--a distinct syndrome?  

PubMed

We report on two unrelated cases born to nonconsanguineous parents with a similar clinical presentation: hypotonia since the neonatal period, severe failure to thrive, postnatal growth retardation, facial dysmorphism, congenital cardiac defects (septal defect and non progressive multiple valve dysplasia), shortened extremities, carpal/tarsal and extensive vertebral synostosis, delayed carpal bone age, deafness, and inner ear malformations. Presently, both patients present with normal psychomotor development. Additional abnormal findings include extra oral frenulum, nasal speech, and vesico-ureteral reflux. Molecular analysis in one patient excluded the Noggin gene and Filamin B (FLNB) was excluded in the other patient. Although some features are similar to spondylocarpotarsal synostosis syndrome, the exclusion of FLNB and this constellation of findings suggest a new entity, closely similar to an autosomal dominant condition reported by Forney et al. 1966 in a unique family. Identification of similarly affected patients should aid in the further elucidation of this syndrome. PMID:20186786

Sousa, Sérgio B; Baujat, Geneviéve; Abadie, Véronique; Bonnet, Damien; Sidi, Daniel; Munnich, Arnold; Krakow, Deborah; Cormier-Daire, Valérie

2010-03-01

2

Reconstruction of middle ear malformations  

PubMed Central

Malformations of the middle ear are classified as minor and major malformations. Minor malformations appear with regular external auditory canal, tympanic membrane and aerated middle ear space. The conducting hearing loss is due to fixation or interruption of the ossicular chain. The treatment is surgical, following the rules of ossiculoplasty and stapes surgery. In major malformations (congenital aural atresia) there is no external auditory canal and a deformed or missing pinna. The mastoid and the middle ear space may be underdevelopped, the ossicular chain is dysplastic. Surgical therapy is possible in patients with good aeration of the temporal bone, existing windows, a near normal positioned facial nerve and a mobile ossicular chain. Plastic and reconstructive surgery of the pinna should proceed the reconstruction of the external auditory canal and middle ear. In cases of good prognosis unilateral aural atresia can be approached already in childhood. In patients with high risk of surgical failure, bone anchored hearing aids are the treatment of choice. Recent reports of implantable hearing devices may be discussed as an alternative treatment for selected patients. PMID:22073077

Schwager, Konrad

2008-01-01

3

The ear and its malformations: strange beliefs and misconceptions  

Microsoft Academic Search

Objective. To explore the strange beliefs and misconceptions related to the ear and its malformations, and how these have changed from ancient times until today.Methods. Ancient documents, journal articles, and history books were studied to research ancient and current beliefs and misconceptions with regard to the ear and its malformations.Results. The ear has been the centre of various beliefs and

Irene E Gamatsi; Thomas P Nikolopoulos; Dimitra E Lioumi

2003-01-01

4

Endoscopic assisted cochlear implants in ear malformations.  

PubMed

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

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

2014-08-01

5

Otosclerosis associated with type B-1 inner ear malformation  

PubMed Central

Summary Malformations of bony inner ear are rare anomalies occurring in approximately 20% of patients with congenital sensorineural hearing loss. Conductive hearing loss is usually associated with abnormalities of the external and middle ear. Recent reports of patients with lateral semicircular canal malformations indicate inner ear malformations to be associated with sensorineural or conductive hearing loss. Differential diagnosis of conductive hearing loss should include otosclerosis, isolated ossicular deformities, inner ear anomalies or a combination of these. In this report, a case is described with right vestibule-lateral semicircular canal dysplasia presenting at our centre with bilateral otosclerosis. PMID:20948592

De Stefano, A; Dispenza, F; Aggarwal, N; Russo, A

2010-01-01

6

FGF23 Deficiency Leads to Mixed Hearing Loss and Middle Ear Malformation in Mice  

PubMed Central

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 mice are profoundly deaf. Fgf 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 mice demonstrate dysplastic bulla and ossicles; Fgf 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 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. PMID:25243481

Lysaght, Andrew C.; Yuan, Quan; Fan, Yi; Kalwani, Neil; Caruso, Paul; Cunnane, MaryBeth; Lanske, Beate; Stankovi?, Konstantina M.

2014-01-01

7

Optical and tomographic imaging of a middle ear malformation in the bullfrog (Rana catesbeiana).  

PubMed

Using a combination of in vivo computerized tomography and histological staining, a middle ear anomaly in two wild-caught American bullfrogs (Rana catesbeiana) is characterized. In these animals, the tympanic membrane, extrastapes, and pars media (shaft) of the stapes are absent on one side of the head, with the other side exhibiting normal morphology. The pars interna (footplate) of the stapes and the operculum are present in their normal positions at the entrance of the otic capsule on both the affected and unaffected sides. The pattern of deformity suggests a partial failure of development of tympanic pathway tissues, but with a preservation of the opercularis pathway. While a definitive proximate cause of the condition could not be determined, the anomalies show similarities to developmental defects in mammalian middle ear formation. PMID:16158670

Horowitz, Seth S; Simmons, Andrea Megela; Ketten, Darlene R

2005-08-01

8

Optical and tomographic imaging of a middle ear malformation in the bullfrog (Rana catesbeiana)  

NASA Astrophysics Data System (ADS)

Using a combination of in vivo computerized tomography and histological staining, a middle ear anomaly in two wild-caught American bullfrogs (Rana catesbeiana) is characterized. In these animals, the tympanic membrane, extrastapes, and pars media (shaft) of the stapes are absent on one side of the head, with the other side exhibiting normal morphology. The pars interna (footplate) of the stapes and the operculum are present in their normal positions at the entrance of the otic capsule on both the affected and unaffected sides. The pattern of deformity suggests a partial failure of development of tympanic pathway tissues, but with a preservation of the opercularis pathway. While a definitive proximate cause of the condition could not be determined, the anomalies show similarities to developmental defects in mammalian middle ear formation.

Horowitz, Seth S.; Simmons, Andrea Megela; Ketten, Darlene R.

2005-08-01

9

Deletion of a conserved regulatory element required for Hmx1 expression in craniofacial mesenchyme in the dumbo rat: a newly identified cause of congenital ear malformation  

PubMed Central

SUMMARY Hmx1 is a homeodomain transcription factor expressed in the developing eye, peripheral ganglia, and branchial arches of avian and mammalian embryos. Recent studies have identified a loss-of-function allele at the HMX1 locus as the causative mutation in the oculo-auricular syndrome (OAS) in humans, characterized by ear and eye malformations. The mouse dumbo (dmbo) mutation, with similar effects on ear and eye development, also results from a loss-of-function mutation in the Hmx1 gene. A recessive dmbo mutation causing ear malformation in rats has been mapped to the chromosomal region containing the Hmx1 gene, but the nature of the causative allele is unknown. Here we show that dumbo rats and mice exhibit similar neonatal ear and eye phenotypes. In midgestation embryos, dumbo rats show a specific loss of Hmx1 expression in neural-crest-derived craniofacial mesenchyme (CM), whereas Hmx1 is expressed normally in retinal progenitors, sensory ganglia and in CM, which is derived from mesoderm. High-throughput resequencing of 1 Mb of rat chromosome 14 from dmbo/dmbo rats, encompassing the Hmx1 locus, reveals numerous divergences from the rat genomic reference sequence, but no coding changes in Hmx1. Fine genetic mapping narrows the dmbo critical region to an interval of ?410 kb immediately downstream of the Hmx1 transcription unit. Further sequence analysis of this region reveals a 5777-bp deletion located ?80 kb downstream in dmbo/dmbo rats that is not apparent in 137 other rat strains. The dmbo deletion region contains a highly conserved domain of ?500 bp, which is a candidate distal enhancer and which exhibits a similar relationship to Hmx genes in all vertebrate species for which data are available. We conclude that the rat dumbo phenotype is likely to result from loss of function of an ultraconserved enhancer specifically regulating Hmx1 expression in neural-crest-derived CM. Dysregulation of Hmx1 expression is thus a candidate mechanism for congenital ear malformation, most cases of which remain unexplained. PMID:22736458

Quina, Lely A.; Kuramoto, Takashi; Luquetti, Daniela V.; Cox, Timothy C.; Serikawa, Tadao; Turner, Eric E.

2012-01-01

10

Amphibian malformations  

USGS Publications Warehouse

Frog malformations have been reported from 42 states. The broad geographic distribution of these malformations warrants national attention. Scientists at the USGS National Wildlife Health Center in Madison, Wisconsin are studying this problem in an effort to document its scope and to determine the causes of the observed malformations.

National Wildlife Health Center

1998-01-01

11

Ear Infections  

MedlinePLUS

... your doctor. Complications Will earaches hurt my child's hearing? Middle ear infections and fluid in the ear are the ... Leave ear wax alone. If you think your ear wax affects your hearing, see your doctor to be sure there's no ...

12

Ear Problems  

MedlinePLUS

... ear dry while it's healing. Putting a warm heating pad over your ear may help relieve the pain. You can prevent swimmer's ear by placing 3 to 5 ... medicine for a few days. Putting a warm heating pad on your ear may help relieve the pain. ... bite down? Yes A tooth problem can radiate pain to the ear on the same ...

13

Ear Disorders  

MedlinePLUS

... most common illness in infants and young children. Tinnitus, a roaring in your ears, can be the ... problems in your inner ear; its symptoms include tinnitus and dizziness. Ear barotrauma is an injury to ...

14

Ear Pieces  

ERIC Educational Resources Information Center

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…

DiJulio, Betsy

2011-01-01

15

Ear wax  

MedlinePLUS

... water to drain. You may need to repeat irrigation several times. To avoid damaging your ear or ... who may remove the wax by: Repeating the irrigation attempts Suctioning the ear canal Using a small ...

16

Elephant ear  

MedlinePLUS

Elephant ear plants are indoor or outdoor plants with very large, arrow-shaped leaves. Poisoning may occur ... Elephant ear grows naturally in tropical and subtropical areas, but is easily found in northern climates as ...

17

Ear Tubes  

MedlinePLUS

... In most cases, this causes no problem with hearing. ?Infection? Ear infections can still occur in the middle ear ... infections are usually less frequent, result in less hearing loss, and are easier to treat?often only with ear drops. Sometimes an oral antibiotic is still needed. ? ...

18

Cauliflower Ear  

MedlinePLUS

... Works Main Page The Pink Locker Society What's Cauliflower Ear? KidsHealth > Kids > Q&A > Q & A > What's Cauliflower Ear? Print A A A Text Size Have ... looks bumpy and lumpy? The person might have cauliflower ear. That sure is a funny name. Let's ...

19

Anorectal malformations  

Microsoft Academic Search

Anorectal malformations comprise a wide spectrum of diseases, which can affect boys and girls, and involve the distal anus and rectum as well as the urinary and genital tracts. They occur in approximately 1 in 5000 live births. Defects range from the very minor and easily treated with an excellent functional prognosis, to those that are complex, difficult to manage,

Marc A Levitt; Alberto Peña

2007-01-01

20

Ear Injuries (For Parents)  

MedlinePLUS

... minor any signs of problems with balance or hearing severe ear pain blood or fluid draining from the ear ( ... My Ears? Taking Care of Your Ears Quiz: Ears Hearing Aids Perforated Eardrum Earbuds Swimmer's Ear (External Otitis) ...

21

Anorectal malformations.  

PubMed

Anorectal malformations (ARMs) are among the more frequent congenital anomalies encountered in paediatric surgery, with an estimated incidence ranging between 1 in 2000 and 1 in 5000 live births. Antenatal diagnosis of an isolated ARM is rare. Most cases are diagnosed in the early neonatal period. There is a wide spectrum of presentation ranging from low anomalies with perineal fistula having simple management to high anomalies with complex management. Advances in the imaging techniques with improvement in knowledge of the embryology, anatomy and physiology of ARM cases have refined diagnosis and initial management. There has been marked improvement in survival of such patient over the last century. The management of ARM has moved forward from classical procedures to PSARP to minimal invasive procedures. But still the fecal and urinary incontinence can occur even with an excellent anatomic repair, mainly due to associated problems. There has been a paradigm shift in approach to these patients which involves holistic approach to the syndrome of Anorectal malformations with a long term goal of achievement of complete fecal and urinary continence with excellent quality of life. PMID:25552824

Gangopadhyay, Ajay Narayan; Pandey, Vaibhav

2015-01-01

22

Anorectal malformations  

PubMed Central

Anorectal malformations (ARMs) are among the more frequent congenital anomalies encountered in paediatric surgery, with an estimated incidence ranging between 1 in 2000 and 1 in 5000 live births. Antenatal diagnosis of an isolated ARM is rare. Most cases are diagnosed in the early neonatal period. There is a wide spectrum of presentation ranging from low anomalies with perineal fistula having simple management to high anomalies with complex management. Advances in the imaging techniques with improvement in knowledge of the embryology, anatomy and physiology of ARM cases have refined diagnosis and initial management. There has been marked improvement in survival of such patient over the last century. The management of ARM has moved forward from classical procedures to PSARP to minimal invasive procedures. But still the fecal and urinary incontinence can occur even with an excellent anatomic repair, mainly due to associated problems. There has been a paradigm shift in approach to these patients which involves holistic approach to the syndrome of Anorectal malformations with a long term goal of achievement of complete fecal and urinary continence with excellent quality of life. PMID:25552824

Gangopadhyay, Ajay Narayan; Pandey, Vaibhav

2015-01-01

23

Animal Ears  

NSDL National Science Digital Library

This activity (page 2 of the PDF) is a full inquiry investigation into animal behavior and communication. Groups of learners will fashion a headband with fake ears, similar in shape to those of the animal they are going to observe. Then, they record observations of the animal’s reactions when a learner, wearing the ears in different positions, brings it a snack. Learners develop categories of behavior to organize and evaluate the results. Safety Note: an adult handler must be present if working with a horse or even a large dog. Relates to linked video, DragonflyTV: Horse Ears.

Twin Cities Public Television, Inc.

2006-01-01

24

Arteriovenous Malformations and Other Vascular Malformation Syndromes  

PubMed Central

Vascular malformations are a disruption of the normal vascular pattern in which it is expected that a capillary network of microscopic vessels lies interposed between high-pressure arteries that deliver blood and thin-walled veins that collect low-pressure blood for return to the heart. In the case of arteriovenous malformations, arteries or arterioles connect directly to the venous collection system, bypassing any capillary bed. Clinical consequences result from rupture and hemorrhage, from dramatically increased blood flow, or from the loss of capillary functions such as nutrient exchange and filtering function. These malformations can occur sporadically or as a component of inherited vascular malformation syndromes. In these and other hereditary vascular malformation syndromes, genetic studies have identified proteins and pathways involved in vascular morphogenesis and development. A common theme observed is that vascular malformations result from disruption in pathways involved in vascular stability. Here we review the vascular malformations and pathways involved in hereditary hemorrhagic telangiectasia, capillary malformation–arteriovenous malformation, cerebral cavernous malformations, and mucocutaneous venous malformations. PMID:23125071

Whitehead, Kevin J.; Smith, Matthew C. P.; Li, Dean Y.

2013-01-01

25

Pierced Ears  

MedlinePLUS

... run the risk of getting infected ears. Continue Metal Matters Your first earrings should have gold posts ( ... infection and swelling. Later, you may find some metals cause an allergic reaction. You're probably wondering ...

26

Right Ear/Left Ear  

NSDL National Science Digital Library

In this activity (4th on the page), learners conduct a series of tests to find out which of their ears is more dominant. In other words, do they prefer to use their right or left ear to complete tasks? Learners work in pairs and observe each other doing simple tasks like listening to a whisper, listening to a mystery object in a box, and listening through a wall. Learners collect data to draw conclusions about their partner's "earedness."

2012-06-26

27

How the Ear Works  

MedlinePLUS

... or policy questions to our media and public relations staff at newsroom@entnet.org . The ear has three main parts: the outer ear (including the external auditory canal), middle ear, and inner ear. The ...

28

Ear Cells  

NSDL National Science Digital Library

Spindly cells in the inner ear, called "hair" cells, are critical for both hearing and balance. Now, in a boon for research, neuro-scientists Jeffrey Corwin and Zhenqing Hu at the University of Virginia School of Medicine have finally grown and multiplied these cells in the lab.

Science Update (AAAS; )

2008-05-06

29

Ear Infections in Children  

MedlinePLUS

Home Health Info Hearing, Ear Infections, and Deafness Ear Infections in Children Ear Infections in Children On this page: What is an ear infection? ... fight new infections and also can affect their hearing. Top How can I tell if my child has an ear infection? Most ear infections happen to children before ...

30

Neck auricles with microtia and low position of the right ear: a case report.  

PubMed

Neck auricles are relatively rare malformation. We report the case of a 22-year-old male with two auricles on the anterior borders of sternocleidomastoid muscles, associated with microtia and low position of the right ear. PMID:12927293

Fuad, Brkic; Elmir, Cickusic; Samir, Delibegovic-Dedic

2003-08-01

31

Ear Training  

NSDL National Science Digital Library

Using these websites, you can practice your ear training skills. There are links for intervals, chords, and short melodic segments. First, you should go to this website to practice intervals. Intervals Begin with the first option (simple intervals up). After mastering this, move to simple intervals down. As you become more advanced, continue down the line of interval options. They will become more difficult as you ...

2009-09-15

32

Scalp-Ear-Nipple Syndrome: A Case Report  

PubMed Central

The scalp-ear-nipple (SEN) syndrome is an infrequent congenital disease. Its main features are scalp defects, malformed ears, and absence of nipples. Most of the reported cases are autosomal dominant. We report on a patient suffering SEN syndrome with possible autosomal recessive inheritance. It is concluded that SEN syndrome should be recognized as an entity with genetic heterogeneity once there is evidence of different genetic manner of inheritance described in this disease. PMID:24660003

Morales-Peralta, Estela; Andrés, Vivian; Campillo Betancourt, Dainé

2014-01-01

33

Ear Plastic Surgery  

MedlinePLUS

Ear Plastic Surgery Ear Plastic Surgery Patient Health Information News media interested in covering the latest from AAO-HNS/F can ... weight earrings. Does Insurance Pay for Cosmetic Ear Surgery? Insurance usually does not cover surgery solely for ...

34

Prenatal Ultrasound Screening for External Ear Abnormality in the Fetuses  

PubMed Central

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

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

2014-01-01

35

MALFORMATIONS, MALFORMATIONS EVERYWHERE ... Prepared by Juan Swanepoel & Marieka Gryzenhout  

E-print Network

afflicted by malformation symptoms are commercial pistachio in Iran and the South African native tree karee. Malformations of pistachio are found to be caused by a phytoplasma, but the causal agent for karee is still to the same foe as its relatives mango and pistachio ­ malformations (Fig. 1a, b). #12;20 Fig. 1 Malformations

36

Understanding the Ear  

MedlinePLUS

... this page, go to Types and Causes of Hearing Loss . Outer Ear: The outside of the ear, plus the ear ... which is the pathway of sound from the ear to the brain. Back to Hearing Loss Information Back to Info to Go home ...

37

Autoimmune Inner Ear Disease  

MedlinePLUS

... percent of the 28 million Americans with a hearing loss. How Does the Healthy Ear Work? The ear has three main parts: the ... of AIED The symptoms of AIED are sudden hearing loss in one ear progressing rapidly to the second ear. The hearing ...

38

Posterior fossa malformations.  

PubMed

Understanding embryologic development of the cerebellum and the 4th ventricle is essential for understanding posterior fossa malformations. Posterior fossa malformations can be conveniently classified into those that have a large posterior fossa and those with normal or small posterior fossa. Disorders associated with a large posterior fossa include classic Dandy-Walker malformation, Blake's pouch cyst, mega cisterna magna, and posterior fossa arachnoid cyst. Disorders associated with normal or small posterior fossa include Dandy-Walker variant, Joubert syndrome, tecto-cerebellar dysraphia, rhombencephalosynapsis, the neocerebellar hypoplasias, and cerebellar atrophy. Neuro-imaging features should enable the imager to provide the referring physician a logical approach to these complex posterior fossa malformations. PMID:21596278

Shekdar, Karuna

2011-06-01

39

Congenital Hepatic Vascular Malformations  

Microsoft Academic Search

\\u000a Congenital hepatic vascular malformations are rare entities that result in abnormal shunting of blood through the liver. Three\\u000a different types of shunting can occur: arteriovenous (hepatic artery to hepatic vein), arterioportal (hepatic artery to portal\\u000a vein) and portovenous (portal vein to hepatic vein). Malformations result from alterations in the formation of blood vessels\\u000a during fetal development and can occur as

Guadalupe Garcia-Tsao

40

Fetal bronchopulmonary malformations.  

PubMed

Abstract Introduction: Fetal body tumors are rare, but the ability to diagnose them has improved over recent years. Most masses discovered in the chest results from fetal bronchopulmonary malformations, such as congenital cystic adenomatoid malformation and bronchopulmonary sequestration. Congenital cystic adenomatoid malformation and bronchopulmonary sequestration have a reported incidence of 50% and 33% of all prenatally diagnosed lung lesions, respectively. Material and methods: Retrospective analysis of the congenital cystic adenomatoid malformation and bronchopulmonary sequestration cases diagnosed or surveilled at our department, between January 2003 and March 2013. Prenatal examination, evolution, management and patient outcome were analyzed. Results: A total of 918 fetal malformations were diagnosed at our hospital, 17 of them representing fetal bronchopulmonary malformations. The majority were diagnosed during the second trimester and stabilized or regressed during the third trimester of pregnancy. The pregnancies and deliveries had no other relevant findings or complications, except in three cases. Nine children required surgery. All of the children are healthy and have a normal development, with regular surveillance by the pediatricians. Discussion: The majority of these fetal lung masses are isolated findings that partially regress during intrauterine life. With adequate postnatal surveillance and eventual surgery the prognosis is good. PMID:25394612

Nunes, Carla; Pereira, Isabel; Araújo, Cláudia; Santo, Susana Ferreira; Carvalho, Rui Marques; Melo, Antonieta; Graça, Luís Mendes

2014-12-01

41

Normal Ear, Ear with Hearing Loss, and Cochlear Implant Procedure  

MedlinePLUS

... Implant Surgery Recalls and Safety Animation: Normal Ear, Ear with Hearing Loss, and Cochlear Implant Procedure This is a ... outer ear to the auditory nerve because of hearing loss. In this ear there are fewer nerve impulses in the auditory ...

42

Ear infection - acute  

MedlinePLUS

... there is no improvement or symptoms get worse, schedule an appointment with your health care provider to determine whether antibiotics are needed. ANTIBIOTICS A virus or bacteria can cause ear ... Removing tonsils does not seem to help with ear infections.

43

Cavernous malformations in pregnancy.  

PubMed

Cavernous malformation is a relatively rare disease, but is important in the etiology of cerebral hemorrhage in pregnant and puerperal women. The risk of bleeding is particularly high in patients with a previous history of bleeding, patients with a family history of cavernous malformations, and patients with the causative cerebral cavernous malformation gene. Cavernous malformations are more likely to bleed or to increase in size during pregnancy, under the influence of female hormones and vascular growth factors such as vascular endothelial growth factor. We report our strategy for the treatment of cavernous malformations in pregnant women, with reference to the relevant literature. Asymptomatic patients and those with mild symptoms are usually followed up conservatively by magnetic resonance imaging, without active treatment, but surgical treatment is indicated in patients with severe or progressive symptoms; surgery should also be considered in patients with mild symptoms having risk factors for bleeding. If surgical treatment is selected, the operation plan needs to be devised in collaboration with the specialties of anesthesiology and obstetrics and gynecology, rather than by the brain surgeon alone, in view of the possibility of occurrence of complications specific to pregnant women, such as complications related to weight gain and difficulty in securing the airway, which develop during the perioperative period. PMID:23979052

Yamada, Shuichi; Nakase, Hiroyuki; Nakagawa, Ichiro; Nishimura, Fumihiko; Motoyama, Yasushi; Park, Young-Su

2013-01-01

44

Large plaque-like glomuvenous malformation (glomangioma) simulating venous malformation.  

PubMed

Glomuvenous malformations and venous malformations are vascular lesions that can be distinguished on the basis of clinical and pathological features. A vascular lesion of the skin and superficial and deep soft tissues of a lower limb in a 5-year-old child is described. The clinical and radiological features, including skeletal muscle involvement, were typical of venous malformation, whereas the histopathological features were those of a glomuvenous malformation. The clinical and histopathological features are briefly discussed. PMID:16716158

Vercellino, N; Nozza, P; Oddone, M; Bava, G L

2006-07-01

45

Vascular malformations revisited.  

PubMed

Vascular malformations are congenital anomalies that can affect each part of the vasculature. Combined forms are common and they are often part of complex syndromes. Most malformations are diagnosed during infancy, but some get obvious only later in life. The field of vascular malformations is emerging with recently described new entities and treatments. Still, misdiagnosis is common in this field, leading to nosologic confusion and wrong treatment. Clinical evaluation and imaging are the gold standard for diagnostic confirmation. Sclerotherapy and embolization are the main treatment techniques but are also used preoperatively to reduce blood loss and shrink the lesion if surgery is planned. Despite new treatment options, especially if extensive in size or involving vulnerable structures, vascular malformations are still considered chronic diseases and cause significant morbidity. Common understanding and agreement on terminology and a multidisciplinary approach are the basis of successful treatment and long-term support for these patients. Continuing research in the field of vascular anomalies will improve knowledge and create further treatment options. PMID:25537054

Clemens, Robert K; Pfammatter, Thomas; Meier, Thomas O; Alomari, Ahmad I; Amann-Vesti, Beatrice R

2015-01-01

46

Spinal cord malformations.  

PubMed

Malformations of the spinal cord are one of the most frequent malformations. They should be clearly divided into two completely different families of malformations: open dysraphisms and occult dysraphisms. Open dysraphism mostly consists in myelomeningocele (MMC). Its incidence is 1/1000 live births with a wide variation. Folic acid supplementation has been shown to reduce its risk. In most cases, the diagnosis is done prenatally by serum screening and ultrasound and may lead to termination of pregnancy. In case of decision to continue pregnancy, surgical treatment must be achieved during the first days of life, and in 50 to 90% of cases, a ventricular shunt must be installed. The follow-up of these children must be continued throughout life looking for late complications (Chiari II and syringomyelia, vertebral problems, neuropathic bladder, tethered cord). Occult dysraphisms are a heterogeneous group of malformations. Lipomas (filum and conus) are the most frequent and their treatment remains controversial. Diastematomyelia, neurenteric cysts, dermal sinus, and more complex forms (Currarino syndrome) belong to this group. Most of them can and must be diagnosed prenatally or at birth by careful examination of the lower back for the cutaneous stigmata of the disease to decrease the risk of neurological, urological, or orthopedic permanent handicap. PMID:23622306

Zerah, Michel; Kulkarni, Abhaya V

2013-01-01

47

Middle Ear Infections and Ear Tube Surgery (For Parents)  

MedlinePLUS

... KidsHealth > Parents > Diseases & Conditions > Ears, Nose, Throat/Speech & Hearing > Middle Ear Infections and Ear Tube Surgery Print A A ... What Happens in the Operating Room? Swimmer's Ear Hearing Impairment Swimmer's Ear (External Otitis) Contact Us Print Additional resources Send ...

48

Pathology of the Ear  

PubMed Central

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

Orengo, Ida; Robbins, Kerri; Marsch, Amanda

2011-01-01

49

Hearing, Ear Infections, and Deafness  

MedlinePLUS

... Health Info Hearing, Ear Infections, and Deafness DefaultPage Hearing, Ear Infections, and Deafness Diseases and Conditions Age-Related ... Neuropathy Auditory Processing Disorder Do You Need a Hearing Test? Ear Infections in Children Enlarged Vestibular Aqueducts (EVA) Hearing ...

50

Chiari II Malformation and Syringomyelia  

Microsoft Academic Search

The Chiari II malformation was described at the end of the nineteenth century (1891–1896) by Hans Chiari, a German pathologist,\\u000a as a congenital malformation in a post-mortem examination of a child who died from a constellation of malformations including\\u000a prolapse of the cerebellum, part of the brain stem and part of the hindbrain, involving the upper part of the cervical

Spyros Sgouros

51

Redundant functions of Rac GTPases in inner ear morphogenesis  

PubMed Central

Development of the mammalian inner ear requires coordination of cell proliferation, cell fate determination and morphogenetic movements. While significant progress has been made in identifying developmental signals required for inner ear formation, less is known about how distinct signals are coordinated by their downstream mediators. Members of the Rac family of small GTPases are known regulators of cytoskeletal remodeling and numerous other cellular processes. However, the function of Rac GTPases in otic development is largely unexplored. Here, we show that Rac1 and Rac3 redundantly regulate many aspects of inner ear morphogenesis. While no morphological defects were observed in Rac3-/- mice, Rac1CKO; Rac3-/- double mutants displayed enhanced vestibular and cochlear malformations compared to Rac1CKO single mutants. Moreover, in Rac1CKO; Rac3-/- mutants, we observed compromised E-cadherin-mediated cell adhesion, reduced cell proliferation and increased cell death in the early developing otocyst, leading to a decreased size and malformation of the membranous labyrinth. Finally, cochlear extension was severely disrupted in Rac1CKO; Rac3-/-mutants, accompanied by a loss of epithelial cohesion and formation of ectopic sensory patches underneath the cochlear duct. The compartmentalized expression of otic patterning genes within the Rac1CKO; Rac3-/- mutant otocyst was largely normal, however, indicating that Rac proteins regulate inner ear morphogenesis without affecting cell fate specification. Taken together, our results reveal an essential role for Rac GTPases in coordinating cell adhesion, cell proliferation, cell death and cell movements during otic development. PMID:22182523

Grimsley-Myers, Cynthia M.; Sipe, Conor W.; Wu, Doris K.; Lu, Xiaowei

2012-01-01

52

Familial Cavernous Malformations (CCM) - Common Hispanic Mutation  

MedlinePLUS

... Learn More Familial Cavernous Malformations (CCM) - Common Hispanic Mutation What is Familial Cavernous Malformations (CCM)? Cerebral Cavernous ... Malformations (CCM)? CCM may be inherited due to mutations in one of three genes, CCM1, CCM2, or ...

53

Arteriovenous Malformation Management  

SciTech Connect

Arteriovenous malformations (AVM) are rare vascular lesions that can present with a myriad of clinical presentations. In our institutions, initial workup consists of a clinical exam, color Doppler imaging, and magnetic resonance imaging. After the initial noninvasive workup, arteriography, at times closed system venography, and ethanol endovascular repair of the AVM is performed under general anesthesia. Depending on the size of the lesion, additional Swan-Ganz line and arterial line monitoring are performed. Patients are usually observed overnight and uneventfully discharged the following day if no complication occurs. Patients are followed at periodic intervals despite cure of their lesion. Long-term follow-up is essential in AVM management.

Yakes, Wayne F. [Interventional Radiology and Interventional Neuroradiology, Radiology Imaging Associates, P.C., Colorado NeurologicalInstitute, Swedish Medical Center, 501 E. Hampden Avenue, Englewood, CO 80110 (United States); Rossi, Plinio [Department of Radiology, Universita Degli Studi Di Roma, 'La Sapienza', 00161 Rome (Italy); Odink, Henk [Department of Radiology, De Wever Hospital, Henri Dunanstrat 5, 6419 PC Heerlen (Netherlands)

1996-11-15

54

Ear problems in swimmers.  

PubMed

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. PMID:16138712

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

2005-08-01

55

Wildervanck's syndrome with severe inner ear dysplasia and agenesis of the right internal carotid artery.  

PubMed

We describe a case with Wildervanck syndrome (cervico-oculo-acoustic syndrome) comprising Klippel-Feil anomaly, retractio bulbi (Duane syndrome), and congenital sensorineural deafness. An 18-month male baby had a severe inner ear dysplasia, and MRI also showed a complex vascular carotid malformation associated. PMID:24576452

Hernando, Mónica; Urbasos, María; Amarillo, Viviana Elizabeth; Herrera, María Teresa; García-Peces, Victoria; Plaza, Guillermo

2014-04-01

56

Amphibian Population Declines and Malformations  

E-print Network

Journal of Science 49:70-71 Alberta Naturalist 11:1-4 Conservation Biology 7:355-362, 8:72-85, 10 growth ·Organophosphate Insecticides: Chemicals & Effects: Above plus malformations and altered behavior

Gray, Matthew

57

CHARGE association in Sweden: malformations and functional deficits.  

PubMed

CHARGE association (CA) consists of a non-random association of ocular coloboma (C), heart anomaly (H), atresia of choanae (A), retarded growth and/or development (R), genital hypoplasia (G), and ear anomalies and/or hearing impairment (E). A prospective multidisciplinary study of 31 Swedish patients with CA was undertaken in order to describe the associated malformations and functional deficits, find possible etiological factors and identify critical time periods for the maldevelopment. The clinical files were analyzed, the mothers answered a questionnaire on history of prenatal events, and a clinical evaluation of systemic findings, vision, hearing, balance, speech, oral and swallowing function, and neuro-psychiatric function, especially autism, was performed. The most frequent physical abnormalities affected ears (90%), eyes (90%), brain (61%), heart (52%), retarded growth (48%), genitals (38%), choanae (35%), and facial nerve (32%). Sixty-one percent of the patients were visually impaired or blind, and 74% had hearing loss or deafness. Problems in balance, speech, and eating were common. Forty percent of the patients had autism/atypical autism, and 82% had developmental delay. Three children were born following assisted fertilization and two mothers had diabetes. The mothers reported infections, bleedings, and drug use during pregnancy. Analysis of possible critical time periods suggested that most malformations were produced early in pregnancy, mainly during post conceptual weeks 4, 5, and 6. A multidisciplinary approach is essential in the assessment and management of CA. PMID:15633180

Strömland, Kerstin; Sjögreen, Lotta; Johansson, Maria; Ekman Joelsson, Britt-Marie; Miller, Marilyn; Danielsson, Susanna; Billstedt, Eva; Gillberg, Christopher; Jacobsson, Catharina; Norinder, Jan Andersson; Granström, Gösta

2005-03-15

58

Signaling regulating inner ear development: Cell fate determination, patterning, morphogenesis, and defects.  

PubMed

The membranous labyrinth of the inner ear is a highly complex organ that detects sound and balance. Developmental defects in the inner ear cause congenital hearing loss and balance disorders. The membranous labyrinth consists of three semicircular ducts, the utricle, saccule, and endolymphatic ducts, and the cochlear duct. These complex structures develop from the simple otic placode, which is established in the cranial ectoderm adjacent to the neural crest at the level of the hindbrain at the early neurula stage. During development, the otic placode invaginates to form the otic vesicle, which subsequently gives rise to neurons for the vestibulocochlear ganglion, the non-sensory and sensory epithelia of the membranous labyrinth that includes three ampullary crests, two maculae, and the organ of Corti. Combined paracrine and autocrine signals including fibroblast growth factor, Wnt, retinoic acid, hedgehog, and bone morphogenetic protein regulate fate determination, axis formation, and morphogenesis in the developing inner ear. Juxtacrine signals mediated by Notch pathways play a role in establishing the sensory epithelium, which consists of mechanosensory hair cells and supporting cells. The highly differentiated organ of Corti, which consists of uniformly oriented inner/outer hair cells and specific supporting cells, develops during fetal development. Developmental alterations/arrest causes congenital malformations in the inner ear in a spatiotemporal-restricted manner. A clearer understanding of the mechanisms underlying inner ear development is important not only for the management of patients with congenital inner ear malformations, but also for the development of regenerative therapy for impaired function. PMID:25040109

Nakajima, Yuji

2015-02-01

59

Occurrence of Different External Ear Deformities in Monozygotic Twins: Report of 2 Cases  

PubMed Central

Summary: Microtia is a spectrum of congenital deformities, which varies from barely discernable to anotia. Twinning is a well-known risk factor for congenital defects including external ear deformities. Monozygotic twins usually show identical appearances as well as congenital malformations. In special conditions as ear deformities, “mirror-image” may also occur. We report 2 cases of monozygotic twins with different ear deformities. The 8-year-old propositus with lobule type microtia and her identical female twin presented with facial symmetry. Patient A had sausage-type right microtia with absence of external auditory canal. The left external ear showed normal appearance. Patient B presented with left-sided preauricular skin tag and right-sided malformation of tragus with skin tag combined with hyperplasia of underlying cartilage. A granule-size skin tag was also noticed at crus of right helix. A 7-year-old male patient with right-sided conchal type microtia presented to an ear reconstruction center. The patient’s mother showed normal auricular appearance. Her monozygotic twin sister, whose son and daughter had normal ear appearance, was diagnosed with a leftsided lobule type microtia. PMID:25426389

Hu, Jin-Tian; Liu, Tun; Qian, Jin; Zhang, Yong-Biao; Zhou, Xu

2014-01-01

60

Macroglossia due to venous malformation  

Microsoft Academic Search

Massive vascular malformation involving tongue can cause significant functional impairment. In this report we describe a rare\\u000a case of extensive venous malformation involving tongue leading to obstructive sleep apnoea, inability to speak, eat, severe\\u000a discomfort due to exposure induced dryness and ulceration. Multimodality treatment approach comprising of sclerotherapy, electrocautery\\u000a and radiation therapy was used in this case.

T. S Anand; Ritu Mittal; T. B. Shashidhar; R. K. Sharma

2006-01-01

61

Malformations of cortical development  

PubMed Central

Background Malformations of cortical development (MCD) are increasingly recognized as an important cause of epilepsy and developmental delay. MCD encompass a wide spectrum of disorders with various underlying genetic etiologies and clinical manifestations. High resolution imaging has dramatically improved our recognition of MCD. Review Summary This review will provide a brief overview of the stages of normal cortical development, including neuronal proliferation, neuroblast migration, and neuronal organization. Disruptions at various stages lead to characteristic MCD. Disorders of neurogenesis give rise to microcephaly (small brain) or macrocephaly (large brain). Disorders of early neuroblast migration give rise to periventricular heterotopia (neurons located along the ventricles), whereas abnormalities later in migration lead to lissencephaly (smooth brain) or subcortical band heterotopia (smooth brain with a band of heterotopic neurons under the cortex). Abnormal neuronal migration arrest give rise to over-migration of neurons in cobblestone lissencephaly. Lastly, disorders of neuronal organization cause polymicrogyria (abnormally small gyri and sulci). This review will also discuss the known genetic mutations and potential mechanisms that contribute to these syndromes. Conclusion Identification of various gene mutations has not only given us greater insight into some of the pathophysiologic basis of MCD, but also an understanding of the processes involved in normal cortical development. PMID:18469675

Pang, Trudy; Atefy, Ramin; Sheen, Volney

2012-01-01

62

Pancreatic Arteriovenous Malformation  

PubMed Central

An unusual case of pancreatic arteriovenous malformation (P-AVM) combined with esophageal cancer is reported. A 59-year-old man was admitted with upper abdominal pain. Contrast-enhanced computed tomography showed numerous strongly enhanced abnormal vessels and a hypovascular lesion in the area of the pancreatic tail. Angiographic study of the celiac artery confirmed racemose vascular networks in the tail of the pancreas. Endoscopic retrograde pancreatography revealed narrowing and displacement of the main pancreatic duct in the tail of the pancreas. Screening esophagoscopy showed a 0-IIa+IIc type tumor in the lower thoracic esophagus. Histological examination of esophagoscopic biopsies showed squamous cell carcinoma. Based on these findings, P-AVM or pancreatic cancer and esophageal cancer were diagnosed. Video-assisted thoracoscopic esophagectomy and distal pancreatectomy were performed. Histological examination of the resected pancreas revealed abundant abnormal vessels with intravascular thrombi. In addition, rupture of a dilated pancreatic duct with pancreatic stones and both severe atrophy and fibrosis of the pancreatic parenchyma were observed. The final diagnoses were P-AVM consequent to severe chronic pancreatitis and esophageal carcinoma. The patient's postoperative course was relatively good. PMID:24574946

Yamabuki, Takumi; Ohara, Masanori; Kimura, Noriko; Okamura, Kunishige; Kuroda, Aki; Takahashi, Ryo; Komuro, Kazuteru; Iwashiro, Nozomu

2014-01-01

63

Ototoxicity (Ear Poisoning) (For Parents)  

MedlinePLUS

... part of the ear responsible for receiving/sending sounds and controlling balance — it's called ototoxicity or "ear ... have trouble hearing certain things, from high-pitched sounds to talking if there's background noise. Or they ...

64

The red ear syndrome  

PubMed Central

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

2013-01-01

65

Ear Infections and Language Development.  

ERIC Educational Resources Information Center

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…

Roberts, Joanne E.; Zeisel, Susan A.

66

Pulmonary arteriovenous malformations.  

PubMed

Within the past decade, pulmonary arteriovenous malformations (PAVMs) have evolved from rare curiosities to not uncommon clinical states, with the latest estimates suggesting a prevalence of ?1 in 2,600. PAVMs provide anatomic right-to-left shunts, allowing systemic venous blood to bypass gas exchange and pulmonary capillary bed processing. Hypoxemia and enhanced ventilatory demands result, although both are usually asymptomatic. Paradoxical emboli lead to strokes and cerebral abscesses, and these commonly occur in individuals with previously undiagnosed PAVMs. PAVM hemorrhage is rare but is the main cause of maternal death in pregnancy. PAVM occlusion by embolization is the standard of care to reduce these risks. However, recent data demonstrate that currently recommended management protocols can result in levels of radiation exposure that would be classified as harmful. Recent publications also provide a better appreciation of the hematologic and cardiovascular demands required to maintain arterial oxygen content and oxygen consumption in hypoxemic patients, identify patient subgroups at higher risk of complications, and emphasize the proportion of radiologically visible PAVMs too small to treat by embolization. This review, therefore, outlines medical states that exacerbate the consequences of PAVMs. Chief among these is iron deficiency, which is commonly present due to concurrent hereditary hemorrhagic telangiectasia: iron deficiency impairs hypoxemia compensations by restricting erythropoiesis and increases the risk of ischemic strokes. Management of periodontal disease, dental interventions, pulmonary hypertension, and pregnancy also requires specific consideration in the setting of PAVMs. The review concludes by discussing to what extent previously recommended protocols may benefit from modification or revision. PMID:25420112

Shovlin, Claire L

2014-12-01

67

Pax3 function is required specifically for inner ear structures with melanogenic fates.  

PubMed

Pax3 mutations result in malformed inner ears in Splotch mutant mice and hearing loss in humans with Waardenburg's syndrome type I. In the inner ear, Pax3 is thought to be involved mainly in the development of neural crest. However, recent studies have shown that Pax3-expressing cells contribute extensively to multiple inner ear structures, some of which were considered to be derived from the otic epithelium. To examine the specific functions of Pax3 during inner ear development, fate mapping of Pax3 lineage was performed in the presence or absence of functional Pax3 proteins using Pax3(Cre) knock-in mice bred to Rosa26 reporter (R26R) line. ?-gal-positive cells were widely distributed in Pax3(Cre/+); R26R inner ears at embryonic day (E) 15.5, including the endolymphatic duct, common crus, cristae, maculae, cochleovestibular ganglion, and stria vascularis. In the absence of Pax3 in Pax3(Cre/Cre); R26R inner ears, ?-gal-positive cells disappeared from regions with melanocytes such as the stria vascularis of the cochlea and dark cells in the vestibule. Consistently, the expression of Dct, a melanoblast marker, was also absent in the mutant inner ears. However, when examined at E11.5, ?-gal positive cells were present in Pax3(Cre/Cre) mutant otocysts, whereas Dct expression was absent, suggesting that Pax3 lineage with a melanogenic fate migrated to the inner ear, yet failed to differentiate and survive without Pax3 function. Gross inner ear morphology was generally normal in Pax3(Cre/Cre) mutants, unless neural tube defects extended to the cranial region. Taken together, these results suggest that despite the extensive contribution of Pax3-expressing cells to multiple inner ear tissues, Pax3 function is required specifically for inner ear components with melanogenic fates. PMID:24565836

Kim, Hongkyung; Ankamreddy, Harinarayana; Lee, Dong Jin; Kong, Kyoung-Ah; Ko, Hyuk Wan; Kim, Myoung Hee; Bok, Jinwoong

2014-03-14

68

Drug delivery to the ear.  

PubMed

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. PMID:23323784

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

2013-01-01

69

[Bilateral multilobular cystic adenomatoid malformation].  

PubMed

We reported a case of bilateral and multilobar Congenital Cystic Adenomatoid Malformation (C.C.A.M.) in a four months old child with good clinical results after resections of the lesions. This is a relatively rare form of pulmonary illness. The final prognosis, in those patients, depends on the type of malformation, the presence or absence of fetal hydrops and on the degree of affected lung. There have been reported a few cases of multiple affectation. We will consider the physiopathological aspects of the case, late clinical presentation and treatment and the positive surgical response based in the findings of the functional and anatomic imagen studys. PMID:8679395

Tristán, J U; Gracía Urgellés, X; Wiehoff Neumann, A; Hernández Briz Estévez Rosas, S; Ruiz, M P; Pavcovich Calvo, F M

1995-10-01

70

Ear recognition: a complete system  

NASA Astrophysics Data System (ADS)

Ear Recognition has recently received significant attention in the literature. Even though current ear recognition systems have reached a certain level of maturity, their success is still limited. This paper presents an efficient complete ear-based biometric system that can process five frames/sec; Hence it can be used for surveillance applications. The ear detection is achieved using Haar features arranged in a cascaded Adaboost classifier. The feature extraction is based on dividing the ear image into several blocks from which Local Binary Pattern feature distributions are extracted. These feature distributions are then fused at the feature level to represent the original ear texture in the classification stage. The contribution of this paper is three fold: (i) Applying a new technique for ear feature extraction, and studying various optimization parameters for that technique; (ii) Presenting a practical ear recognition system and a detailed analysis about error propagation in that system; (iii) Studying the occlusion effect of several ear parts. Detailed experiments show that the proposed ear recognition system achieved better performance (94:34%) compared to other shape-based systems as Scale-invariant feature transform (67:92%). The proposed approach can also handle efficiently hair occlusion. Experimental results show that the proposed system can achieve about (78%) rank-1 identification, even in presence of 60% occlusion.

Abaza, Ayman; Harrison, Mary Ann F.

2013-05-01

71

Abernethy malformation: a case report  

PubMed Central

Background Abernethy malformation is a very rare congenital vascular malformation defined by diversion of portal blood away from liver. It is commonly associated with multiple congenital anomalies. We present a case of Abernethy malformation, without associated congenital anomalies from India. Case presentation A 5-year-old female child presented with short history of jaundice. A provisional diagnosis of acute viral hepatitis was made in view of clinical presentation and local endemicity of viral hepatitis A. Persistence of jaundice on follow up after 4?weeks led to detailed investigations. Ultrasound and doppler study of abdomen revealed drainage of portal vein into inferior vena cava. CT angiography was performed which confirmed the diagnosis of Type 1 b Abernethy malformation without associated major anomalies. We discuss the common clinical presentations, associated anomalies, diagnostic workup and treatment options of this disorder. Conclusion The treatment of the patients with congenital porto-systemic shunts depends on the site of the shunt, associated congenital anomalies and the extent of liver damage but the prognosis depends on the complications irrespective of anatomical type. However, the extent of associated abnormalities should not deter paediatricians to refer patients for treatment. Whenever possible closure of the shunt should be advised for cure or to prevent complications. Only symptomatic type I patients with absence of possibility to close the shunt may require liver transplant. Long-term follow-up is indicated for all patients. PMID:22642663

2012-01-01

72

Congenital Pulmonary Malformation in Children  

PubMed Central

Congenital Pulmonary Malformations (CPMs) are a group of rare lung abnormalities affecting the airways, parenchyma, and vasculature. They represent a spectrum of abnormal development rather than discrete pathological entities. They are caused by aberrant embryological lung development which occurs at different stages of intrauterine life. PMID:24278678

Nadeem, Montasser; Elnazir, Basil; Greally, Peter

2012-01-01

73

Management of perinatal lung malformations.  

PubMed

This review uses the most up-to-date literature to help guide obstetrical providers through the diagnosis and management of perinatal lung malformations. These lesions, which include congenital pulmonary airway malformation [CPAM, formerly congenital cystic adenomatoid malformation (CCAM)] and bronchopulmonary sequestration (BPS), are relatively rare but are becoming increasingly common because of the improved resolution and enhanced sensitivity of fetal ultrasound. Serial assessment throughout pregnancy remains the norm rather than the exception. Perinatal management strategies can differ based on the sonographic characteristics and dynamic growth patterns of lung masses. Fetal magnetic resonance imaging and other diagnostic testing can sometimes be helpful in providing additional prognostic information. Over the last decade, the importance of echocardiography and utility of maternal steroids have been recognized in cases of non-immune hydrops. Fetal surgery is now rarely performed. Decisions regarding whether delivery of these fetuses should occur in a tertiary care center with pediatric surgery coverage versus delivery at a local community hospital are now highly relevant in most prenatal counseling discussions with families. Large lung malformations may require urgent surgical removal in the early postnatal period because of respiratory distress. Other complications, such as recurrent pneumonia, pneumothorax, and cancer, are indications for lung resection on an elective basis. In the vast majority of cases, the overall prognosis remains excellent. PMID:25310108

Macardle, C A; Kunisaki, S M

2015-02-01

74

Elastotic nodules of the ears.  

PubMed

A case of bilateral elastotic nodules of the ears is reported. This distinctive entity occurs on the ears in response to chronic actinic damage and is seen infrequently. Elastotic nodules of the ears may therefore be misdiagnosed clinically as basal cell carcinoma or some infiltrative process such as gout or amyloid. Although the lesions are usually found on the anthelix, they may also arise on the helix, and can then simulate chondrodermatitis nodularis helicis. PMID:2612205

Requena, L; Aguilar, A; Sánchez Yus, E

1989-12-01

75

Save Your Ears  

NSDL National Science Digital Library

This game depicts a woman going through her day, faced with various loud sounds. In each situation, she has the choice of how to deal with the loud sound: Ear Plugs, Turn It Down, Move Away, or Do Nothing. You only have 3 of each choice available, symbolized by cards on screen. Your goal is to reach the end of the game with undamaged hearing and one set of earplugs, so you can enjoy an awesome rock concert. After each decision, you find out whether your hearing was damaged and if you made the best choice.

OMSI

2010-01-01

76

Ear, Nose & Throat Issues & Down Syndrome  

MedlinePLUS

... have frequent ear wax impactions that may impair hearing. Routine ear examinations can assess wax impactions, and periodic screening ... properly identified, it can be greatly improved with hearing aids, ear cleanings and environmental adaptations. Airway obstruction and Sleep ...

77

Elastotic nodules of the ear.  

PubMed

Eight cases of an elastotic degenerative lesion, developing on the ears in response to actinic damage are reported. Elastotic nodules may be diagnosed clinically as basal cell carcinoma, amyloid or even gout. Although usually found on the antihelix of the ear, they may arise on the helix and simulate chondrodermatitis nodularis helicis. PMID:7334162

Weedon, D

1981-12-01

78

Cryptic vascular malformations involving the brainstem  

SciTech Connect

Six patients with angiographically cryptic vascular malformations involving the brainstem were examined with computed tomography (CT). The clinical and CT findings of cryptic vascular malformations of the brainstem are described and distinguished from those of brainstem glioma and multiple sclerosis. Calcification within a brainstem lesion that displays relatively little mass effect and shows little contrast enhancement, particularly when associated with a long history of waxing and waning brainstem symptoms, should suggest a vascular malformation.

Yeates, A.; Enzmann, D.

1983-01-01

79

[Malformations of the esophagus: diagnosis and therapy].  

PubMed

Esophageal malformations are rare and can occur sporadically or as a component of various syndromes. The variations and classifications are manifold. With the available modern operation techniques most malformations can be resolved with good results. However, esophageal malformations are often combined with further malformations which limit the prognosis. The separation of the trachea and esophagus after gastrulation is not yet completely researched. The results so far indicate that the localized expression of various homeodomain transcription factors is essential for normal development of the trachea and esophagus. PMID:23423505

Falkeis, C; Hager, T; Freund-Unsinn, K; Wohlschläger, J; Veits, L; Hager, J

2013-03-01

80

Inner and outer ear anatomy  

NSDL National Science Digital Library

Sounds are actually waves from vibrations. The outer ear catches these waves and funnels them down into the inner ear. The waves reach the eardrum and in turn make the eardrum vibrate. Three small bones receive these vibrations next, then a snail shell-shaped structure called the cochlea. The cochlea is filled with liquid, and this liquid stimulates hairs inside the inner ear. The hairs transmit the signal to the auditory nerve where the signal is taken to the brain and processed as sound.

Zina Deretsky (National Science Foundation; )

2006-10-23

81

Four cases of trisomy 18 syndrome with limb reduction malformations.  

PubMed Central

Limb reduction malformations of the arms are well documented in the trisomy 18 syndrome. Four cases of trisomy 18 syndrome with limb reduction malformations of the legs are described and compared with the upper limb malformations. Images PMID:6492096

Christianson, A L; Nelson, M M

1984-01-01

82

Diffuse malformations of cortical development.  

PubMed

Malformations of cortical development (MCD) represent a major cause of developmental disabilities and severe epilepsy. Advances in imaging and genetics have improved the diagnosis and classification of these conditions. Up to now, eight genes have been involved in different types of MCD. Lissencephaly-pachygyria and subcortical band heterotopia (SBH) represent a malformative spectrum resulting from mutations of either LIS1 or DCX genes. LIS1 mutations cause a more severe malformation in the posterior brain regions. DCX mutations usually cause anteriorly predominant lissencephaly in males and SBH in female patients. Additional forms are X-linked lissencephaly with corpus callosum agenesis and ambiguous genitalia associated with mutations of the ARX gene. Lissencephaly with cerebellar hypoplasia (LCH) encompass heterogeneous disorders named LCH types a to d. LCHa is related to mutation in LIS1 or DCX, LCHb with mutation of the RELN gene, and LCHd could be related to the TUBA1A gene. Polymicrogyria encompasses a wide range of clinical, etiological, and histological findings. Among several syndromes, recessive bilateral fronto-parietal polymicrogyria has been associated with mutations of the GPR56 gene. Bilateral perisylvian polymicrogyria has been associated with mutations in the SRPX2 gene in a few individuals and with linkage to chromosome Xq28 in a some other families. X-linked bilateral periventricular nodular heterotopia (PNH) consists of PNH with focal epilepsy in females and prenatal lethality in males. Filamin A (FLNA) mutations have been reported in some families and in sporadic patients. It is possible to infer the most likely causative gene by brain imaging studies and other clinical findings. PMID:23622213

Bahi-Buisson, Nadia; Guerrini, Renzo

2013-01-01

83

Orbital arteriovenous malformation mimicking cavernous sinus dural arteriovenous malformation  

PubMed Central

AIMS—Orbital arteriovenous malformations (OAVM) are rare, mostly described with high flow characteristics. Two cases are reported with an OAVM of distinct haemodynamic abnormality. The clinical, angiographic features, and the management considerations are discussed.?METHODS—Case review of two patients with dural AVM (DAVM) who presented to referral neuro-ophthalmology and endovascular services because of clinical symptoms and signs consistent with a cavernous sinus dural AVM.?RESULTS—In each patient, superselective angiography revealed a small slow flow intraorbital shunt supplied by the ophthalmic artery. The transarterial and transvenous endovascular approaches to treat the malformation were partially successful. Although, the abnormal flow was reduced, complete closure of the DAVM could not be accomplished without significant risk of iatrogenic injury. Neither patient's vision improved after intervention.?CONCLUSION—A DAVM in the orbit can cause similar clinical symptoms and signs to those associated with a cavernous sinus DAVM. Even with high resolution magnetic resonance imaging, only superselective angiography can identify this small intraorbital slow flow shunt. The location in the orbital apex and the small size precludes a surgical option for treatment. The transarterial and transvenous embolisation options are limited.?? PMID:10873992

Huna-Baron, R.; Setton, A.; Kupersmith, M.; Berenstein, A.

2000-01-01

84

Management of Pulmonary Arteriovenous Malformations  

PubMed Central

Pulmonary arteriovenous malformations are rare lesions with significant clinical complications. These lesions are commonly seen in patients with hereditary hemorrhagic telangiectasia (formerly Osler-Weber-Rendu syndrome). Interventional radiologists are a key part of the treatment team in this complex disease, and a thorough understanding of the disease process is critical to providing good patient care. In this article, the authors review the disease course and its association with hereditary hemorrhagic telangiectasia, discusses the clinical evaluation and treatment of these complex patients, and outlines complications and follow-up. PMID:22379273

Meek, Mary E.; Meek, James C.; Beheshti, Michael V.

2011-01-01

85

Scalp arteriovenous malformations in young  

PubMed Central

Scalp arteriovenous malformations are an exceptional group of vascular lesions with curious presentations and an elusive natural history. Their detection in the pediatric population is a rarer occurrence. We discuss our experience with five children suffering from this pathology and their surgical management carried at our institution from 2007 to 2013. The genesis in pediatric patients is, usually, spontaneous in contrast with the history of trauma seen in adults. Clinical symptoms, usually, range from an asymptomatic lesion, local discomfort, headaches to necrosis and massive hemorrhage. Selective angiography remains the cornerstone for investigation. Complete surgical excision, embolization or an approach combining the modalities is curative.

Gupta, Rakesh; Kayal, Akshat

2014-01-01

86

Responses of the Inner Ear toResponses of the Inner Ear to InfrasoundInfrasound  

E-print Network

Responses of the Inner Ear toResponses of the Inner Ear to InfrasoundInfrasound Alec N. Salt, Ph this TalkTake-Home Messages from this Talk · The ear is sensitive and responds to low frequency sounds Noise Floor Electrical recording from the guinea pig earElectrical recording from the guinea pig ear

Salt, Alec N.

87

Wonder Ears: Identification of Identical Twins from Ear Images Hossein Nejati  

E-print Network

Wonder Ears: Identification of Identical Twins from Ear Images Hossein Nejati , Li Zhang ,Terence explored automatic ear recognition for identical twin identification. Ear image recognition has been stud (performed manually). We here explore the possibility of automatic twin identification from their ear images

Sim, Terence

88

[Middle ear salivary gland choristoma related to branchio-oto-renal syndrome diagnosed by array-CGH].  

PubMed

Branchio-oto-renal (BOR) syndrome is characterized by ear malformations associated with sensorineural or mixed hearing loss. In addition, preauricular tags, preauricular pits, branchial cleft fistulas and cysts, as well as renal dysplasia are seen. A genetic mutation on chromosome 8, either autosomal dominantly inherited or occuring as a spontaneous mutation, is the cause in the majority of cases. Using array-based comparative genomic hybridization (CGH), it is possible to detect even the smallest genetic changes. Salivary gland choristoma in the middle ear is very rare. Surgical removal and histological clarification are required. PMID:23868653

Amrhein, P; Sittel, C; Spaich, C; Kohlhase, J; Boppert, R; Kohlhof, P; Koitschev, A

2014-05-01

89

Complicated pneumothorax and congenital lung cystic malformation.  

PubMed

Congenital cystic adenomatoid malformation, also named congenital pulmonary airway malformation (CPAM), is a congenital lung abnormality which is uncommon in adults. The usual radiological appearance of CPAM is a cystic space-occupying lesion. We present one case of CPAM with unusual clinical and radiological findings, a complicated spontaneous pneumothorax with intracystic haemorrhage with successful conservative initial treatment, despite acute haemodynamic instability. PMID:24694267

Attou, Rachid; Reper, Pascal

2014-04-01

90

North American Reporting Center for Amphibian Malformations  

NSDL National Science Digital Library

Created in June, 1997 and funded by the US Geological Survey and Environmental Protection Agency, this site is a response to the discovery of numerous deformed amphibians (mostly frogs) in areas as widespread as Minnesota, California, and Florida. Malformations include extra or missing limbs, missing eyes, and split limbs. The site is intended to serve as a central repository for data on the type and relative frequency of such malformations throughout the US. Researchers hope to use the site to help discover the cause(s) of these deformities. The site contains a map of where malformations have been reported, background on the occurrence and possible causes of defects, numerous images of malformed amphibians, a searchable bibliography, instructions on how to report the discovery of a malformed amphibian, and links to eight related web sites. NARCAM's site also contains a toll-free number for citizen reports.

Center., Northern P.

1997-01-01

91

Cervical spine arterio venous malformation  

PubMed Central

We present an unusual case of a young patient who presented to our emergency department with a history of sudden onset of pain on the left side of the neck and numbness to the left arm after lifting a small weight. Patient continued to work as normal for approximately 30?min after the event and then attended the emergency department as numbness in the left arm was not resolving. On examination there was no sensorymotor deficit in the lower limbs but neurological deficits were found in the upper limbs which made us suspect a diagnosis of cervical spine injury/brachial plexus. The patient then rapidly developed numbness in both upper and lower limbs and eventually became aphasic and developed a rapid, shallow respiration and was unable to maintain the airway. The patient was then intubated and ventilated. The patient was then transferred to a neurosurgical centre after the relevant investigations was found to have an arteriovenous malformation of spinal cord. PMID:23349172

Parla, Giridhar; Ameh, Victor

2013-01-01

92

Chiari malformations: diagnosis, treatments and failures.  

PubMed

Chiari malformations refer to abnormalities of the hindbrain originally described by the Austrian pathologist Hans Chiari in the early 1890s. These malformations range from herniation of the cerebellar tonsils through the foramen magnum to complete agenesis of the cerebellum. In this review, we review the different classification schemes of Chiari malformations. We discuss the different signs and symptoms that the two most common malformations present with and diagnostic criteria. We next discuss current treatment paradigms, including the new measure of possible in utero surgery to help decrease the incidence of Chiari type II malformations. There is also a small discussion of treatment failures and salvage procedures in these difficult cases. Chiari malformations are a difficult clinical entity to treat. As more is learned about the genetic and environmental factors relating to their characteristics, it will be interesting if we are able to predict which treatments are better suited for different patients. Similarly, with the evolution of in utero techniques especially for Chiari II malformations, it will be interesting to see if the incidence and practice of treating these difficult patients will change. PMID:25418275

Abd-El-Barr, M M; Strong, C I; Groff, M W

2014-12-01

93

Inflammatory diseases of the ear.  

PubMed

The inflammatory diseases of external and middle ear are one of the commonest conditions encountered by the pediatric physician. Inner ear inflammations are less common and need special and urgent attention. Special management in each case requires detailed history, examination, necessary investigations and appropriate referral to otolaryngologist when necessary. The article is aimed to help formulate a plan in managing the inflammatory conditions of ear. Otalgia constitutes the most prominent of the symptoms in external and middle ear inflammations whereas vertigo, tinnitus and sensory hearing loss form the symptom complex for inner ear infections. It is necessary to understand the basic pathophysiology of the inflammatory condition to be able to institute a targetted treatment. The audiometry impedance studies, microbiology of discharge and occasionally ABR and CT scan from the mainstay of investigative workup. The treatment is specific and based on the precise diagnosis. It often requires the help of an otolaryngologist. Decisions may have to be made with regards to the need for any surgical intervention particularly in acute otitis media, an external canal abscess or an acute mastoiditis. A case of chronic otitis media with facial palsy or vertigo (labyrinthitis being a possibility) needs urgent intervention. PMID:10771916

Cheng, A T; Young, N M

1997-01-01

94

3D printed bionic ears.  

PubMed

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

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

95

The upper airway: congenital malformations.  

PubMed

The upper airway extends from the nasal aperture to the subglottis and can be the site of multiple types of congenital malformations leading to anatomical or functional obstruction. This can cause severe respiratory distress. Newborns are obligate nasal breathers; therefore nasal obstruction can lead to airway compromise and respiratory distress. The etiologies are varied and include, choanal atresia, pyriform aperture stenosis, and rarely tumors such as glioma, encephalocele, teratoma, or dermoid. More common upper airway congenital anomalies include laryngomalacia, vocal cord paralysis, and subglottic stenosis. Laryngolmalacia is the most common congenital laryngeal anomaly. Inspiratory stridor often does not present until two weeks after birth and resolves by 18 months of age. Most cases are managed with watchful waiting. Severe cases require a surgical intervention. Bilateral vocal cord paralysis is usually idiopathic. In certain cases, paralysis may occur secondary to central nervous system abnormality including Arnold-Chiari malformation, cerebral palsy, hydrocephalus, myelomeningocele, spina bifida, or hypoxia. Severe cases may necessitate endotracheal intubation and tracheostomy. Congenital subglottic stenosis is the third most common laryngeal anomaly. It is defined as a diameter of less than 4mm of the cricoid region in a full-term infant, and less than 3mm in a premature infant. This condition is the most common laryngeal anomaly that requires tracheotomy in newborns. Laryngotracheoplasty may be required to achieve decanulation. Knowledge of the upper airway embryological development and congenital anomalies is off prime importance in assessing the newborn with respiratory distress. In most cases flexible endoscopy establishes the diagnosis. Management is tailored to each condition and its degree of severity. PMID:16798587

Daniel, Samuel J

2006-01-01

96

Ear recognition based on edge potential function  

NASA Astrophysics Data System (ADS)

The use of ear information for people identification has been under testing at least for 100 years. However, it is still an open issue if the ears can be considered unique or unique enough to be used as biometric feature. In this paper a biometric system for human identification based on ear recognition is presented. The ear is modeled as a set of contours extracted from the ear image with an edge potential function. The matching algorithm has been tested in presence of several image modifications. Two human ear databases have been used for the tests. The experimental results show the effectiveness of the proposed scheme.

Battisti, F.; Carli, M.; De Natale, F. G. B.; Neri, A.

2012-03-01

97

A gene map of congenital malformations.  

PubMed Central

Congenital malformations frequently arise sporadically, making it difficult to determine whether or not they are genetic in aetiology, let alone which gene(s) may be involved. Nevertheless, rapid progress has been made over recent years in the localisation and identification of gene mutations in specific malformations. This review draws from Mendelian inheritance in man (Johns Hopkins University Press, 11th ed, 1994) and the online version (OMIM) to catalogue 139 loci (including 65 specifically identified genes) implicated in congenital malformations. Some of the most interesting recent developments are discussed. PMID:7966186

Wilkie, A O; Amberger, J S; McKusick, V A

1994-01-01

98

Mutations in KCTD1 cause scalp-ear-nipple syndrome.  

PubMed

Scalp-ear-nipple (SEN) syndrome is a rare, autosomal-dominant disorder characterized by cutis aplasia of the scalp; minor anomalies of the external ears, digits, and nails; and malformations of the breast. We used linkage analysis and exome sequencing of a multiplex family affected by SEN syndrome to identify potassium-channel tetramerization-domain-containing 1 (KCTD1) mutations that cause SEN syndrome. Evaluation of a total of ten families affected by SEN syndrome revealed KCTD1 missense mutations in each family tested. All of the mutations occurred in a KCTD1 region encoding a highly conserved bric-a-brac, tram track, and broad complex (BTB) domain that is required for transcriptional repressor activity. KCTD1 inhibits the transactivation of the transcription factor AP-2? (TFAP2A) via its BTB domain, and mutations in TFAP2A cause cutis aplasia in individuals with branchiooculofacial syndrome (BOFS), suggesting a potential overlap in the pathogenesis of SEN syndrome and BOFS. The identification of KCTD1 mutations in SEN syndrome reveals a role for this BTB-domain-containing transcriptional repressor during ectodermal development. PMID:23541344

Marneros, Alexander G; Beck, Anita E; Turner, Emily H; McMillin, Margaret J; Edwards, Matthew J; Field, Michael; de Macena Sobreira, Nara Lygia; Perez, Ana Beatriz A; Fortes, Jose A R; Lampe, Anne K; Giovannucci Uzielli, Maria Luisa; Gordon, Christopher T; Plessis, Ghislaine; Le Merrer, Martine; Amiel, Jeanne; Reichenberger, Ernst; Shively, Kathryn M; Cerrato, Felecia; Labow, Brian I; Tabor, Holly K; Smith, Joshua D; Shendure, Jay; Nickerson, Deborah A; Bamshad, Michael J

2013-04-01

99

A biomechanical ear model to evaluate middle-ear reconstruction.  

PubMed

In order to evaluate the efficiency of middle-ear prostheses in near-real conditions, an artificial model was developed that approximately simulates the actual geometrical and biomechanical properties of the ear system (excluding the ossicular chain). The sound transmission characteristics of selected commercial middle-ear prostheses and of a synthetic test material were measured using laser Doppler vibrometry (LDV) in this model. The model's realistic properties enabled clinical tympanometry to be used to control the stiffness. In addition the influences of the implant mass on transmission characteristics were investigated. With an averaged displacement between 10 and 100 nm/Pa up to 2000 Hz, the transmission characteristic of the model was comparable with data obtained from the intact middle ear in temporal bone experiments. From the acoustical point of view, no significant material-specific differences could be found. Increasing the mass of the implants to more than 50 mg results in poorer acoustic transmission. In general, changes to the stiffness involving compliance values greater than 3.5 ml and smaller than 0.2 ml led to poorer acoustic transmission. PMID:20017684

Mojallal, Hamidreza; Stieve, Martin; Krueger, Ilka; Behrens, Peter; Mueller, Peter P; Lenarz, Thomas

2009-12-01

100

Multifocal and Metameric Spinal Cord Arteriovenous Malformations  

PubMed Central

Summary We describe 19 (16.0%) multiple vascular malformations (AVMs) in 119 spinal cord arteriovenous malformations (SCAVMs). The associated lesions were eight vertebral vascular malformations, two cutaneous, four limbs, four radicular AVMs, three bifocal SCAVMs; one patient had a bifocal cord lesion associated with vertebral and limb localisations. Various syndromic associations were seen: nine Cobb, two Klippel-Trenaunay-Weber, one Parkes Weber. An additional subgroup of unclassified associations is constituted by seven cases with bifocal intradural uni or multimetameric lesions. In our SCAVMs series, the incidence of multiple vascular lesions is high, in particular multifocal intradural malformations. Metameric distribution is the most frequent type of multiplicity. Identification of the myelomeric level involved in SCAVM allows segmental link between various lesions of mesodermal or neural crest origin to be discussed. PMID:20670488

Matsumaru, Y.; Pongpech, S.; Laothamas, J.; Alvarez, H.; Rodesch, G.; Lasjaunias, P.

1999-01-01

101

[Malformations and abnormalities of the petrous portion of the temporal bone].  

PubMed

High-resolution computed tomography (HRCT) is the procedure of choice in the diagnostics of abnormalities of the middle and inner ear. It allows a detailed presentation of anatomical features and achieves the prerequisites for selection of the various therapeutic options. The highly diverse abnormalities can be described using detailed imaging analyses. Malformations with an abnormally developed modiolus are assumed to be early embryological defects, such as the classical Mondini dysplasia. The essential therapeutic option for middle ear deformities is still a cochlear implant. The domain of magnetic resonance imaging (MRI) is not only in the analysis of the cochlear nerve and for exclusion of fibrosis or ossification of the labyrinth but is also able to visualize details of isolated malformations, such as an extended vestibular aqueduct or subtle alterations to the vestibule or can visualize them better in comparison to CT. Radiological diagnostics are used not only for classification but also to recognize typical clinical problem situations and play a key role in the diagnostics of hearing disorders and selection of the optimal therapeutic procedure. PMID:24706250

Reith, W; Yilmaz, U; Heumüller, I

2014-04-01

102

Physiological functioning of the ear and masking  

NASA Technical Reports Server (NTRS)

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.

1984-01-01

103

21 CFR 878.3590 - Ear prosthesis.  

... FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES GENERAL AND PLASTIC SURGERY DEVICES Prosthetic Devices § 878.3590 Ear prosthesis. (a) Identification. An ear prosthesis is a...

2014-04-01

104

21 CFR 878.3590 - Ear prosthesis.  

Code of Federal Regulations, 2013 CFR

... FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES GENERAL AND PLASTIC SURGERY DEVICES Prosthetic Devices § 878.3590 Ear prosthesis. (a) Identification. An ear prosthesis is a...

2013-04-01

105

21 CFR 878.3590 - Ear prosthesis.  

Code of Federal Regulations, 2011 CFR

... FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES GENERAL AND PLASTIC SURGERY DEVICES Prosthetic Devices § 878.3590 Ear prosthesis. (a) Identification. An ear prosthesis is a...

2011-04-01

106

21 CFR 878.3590 - Ear prosthesis.  

Code of Federal Regulations, 2012 CFR

... FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES GENERAL AND PLASTIC SURGERY DEVICES Prosthetic Devices § 878.3590 Ear prosthesis. (a) Identification. An ear prosthesis is a...

2012-04-01

107

21 CFR 878.3590 - Ear prosthesis.  

Code of Federal Regulations, 2010 CFR

... FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES GENERAL AND PLASTIC SURGERY DEVICES Prosthetic Devices § 878.3590 Ear prosthesis. (a) Identification. An ear prosthesis is a...

2010-04-01

108

The Microcephaly-Capillary Malformation Syndrome  

PubMed Central

We report on three children from two families with a new pattern recognition malformation syndrome consisting of severe congenital microcephaly (MIC), intractable epilepsy including infantile spasms, and generalized capillary malformations that was first reported recently in this journal [Carter et al. (2011); Am J Med Genet A 155: 301–306]. Two of our reported patients are an affected brother and sister, suggesting this is an autosomal recessive severe congenital MIC syndrome. PMID:21815250

Mirzaa, Ghayda M.; Paciorkowski, Alex R.; Smyser, Christopher D.; Willing, Marcia C.; Lind, Anne C.; Dobyns, William B.

2012-01-01

109

Malformation and plastic surgery in childhood  

PubMed Central

Malformations of the head and neck show a huge variety of clinical symptoms with functional and esthetic consequences. Often times its rehabilitation requires multi-staged and multi-disciplinary procedures and concepts. These must consider eating, speech, mimic expression, hearing and “esthetics” or at least “normality”. A survey of the most common head and neck malformations and their treatment options are presented here. PMID:25587361

Siegert, Ralf; Magritz, Ralph

2014-01-01

110

Ear Recognition Based on Gabor Features and KFDA  

PubMed Central

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. PMID:24778595

Mu, Zhichun

2014-01-01

111

Effects of Middle-Ear Disorders on Power Reflectance Measured in Cadaveric Ear Canals  

E-print Network

Effects of Middle-Ear Disorders on Power Reflectance Measured in Cadaveric Ear Canals Susan E. Voss,1 Gabrielle R. Merchant,2 and Nicholas J. Horton3 Objective: Reflectance measured in the ear canal offers a noninvasive method to monitor the acoustic properties of the middle ear, and few systematic

Allen, Jont

112

The caecilian ear: further observations.  

PubMed

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

Wever, E G; Gans, C

1976-10-01

113

[Tympanoplasty on only hearing ears].  

PubMed

Tympanoplasty on only hearing ears was performed on 11 patients in Osaka University Hospital and Osaka Rosai Hospital during 1986 1994; this was 0.68% of all cases of middle ear surgery. The patients consisted of 5 with cholesteatoma, 5 with chronic otitis media and 1 with cholesterin granuloma. The mean age was 47.6 years (13-68 years) and the mean follow-up period was 2 years and 7 months (6 months-4 years). Postoperative hearing results was obtained in 9 patients (81.8%) according to the criteria of Otology Japan (1987), and all ears were dry at the final examination. Compared with the preoperative hearing level, postoperative hearing level showed improvement in 3 cases, not in 7 cases (within difference of 5 dB) and deterioration by 10-15 dB in 1 case. Although indications for surgery on only hearing ears are still controversial, we suggest the following: 1) patients with cholesteatoma should be operated on in the ordinary way; 2) patients with chronic otitis media should be operated on only by myringoplasty; 3) all patients must be operated on very carefully by skillful surgeons. PMID:8776969

Sakagami, M; Ogasawara, H; Node, M; Seo, T; Mishiro, Y; Okumura, S

1996-07-01

114

Middle Ear Infections (For Parents)  

MedlinePLUS

After the common cold , ear infections are the most frequently diagnosed childhood illness in the United States. Most kids will have had ... winter season, when lots of people get upper respiratory tract infections or colds. Signs and Symptoms The signs and ...

115

Frog eye, ear, and nostril  

NSDL National Science Digital Library

You can clearly see the frog's eye. There is an air opening near a black spot on the frog's skin. The opening to the frog's ear is covered by the round, tan membrane to the left in the picture. This membrane is called the tympanum.

Ren West (None;)

2006-08-07

116

Mechanics of the frog ear  

PubMed Central

The frog inner ear contains three regions that are sensitive to airborne sound and which are functionally distinct. (1) The responses of nerve fibres innervating the low-frequency, rostral part of the amphibian papilla (AP) are complex. Electrical tuning of hair cells presumably contributes to the frequency selectivity of these responses. (2) The caudal part of the AP covers the mid-frequency portion of the frog's auditory range. It shares the ability to generate both evoked and spontaneous otoacoustic emissions with the mammalian cochlea and other vertebrate ears. (3) The basilar papilla functions mainly as a single auditory filter. Its simple anatomy and function provide a model system for testing hypotheses concerning emission generation. Group delays of stimulus frequency otoacoustic emissions (SFOAEs) from the basilar papilla are accounted for by assuming that they result from forward and reverse transmission through the middle ear, a mechanical delay due to tectorial membrane filtering and a rapid forward and reverse propagation through the inner ear fluids, with negligible delay. PMID:20149854

van Dijk, Pim; Mason, Matthew J.; Schoffelen, Richard L. M.; Narins, Peter M.; Meenderink, Sebastiaan W. F.

2010-01-01

117

EAR TO THE GROUND IN THIS ISSUE  

E-print Network

EAR TO THE GROUND IN THIS ISSUE Update from the Division Director 1 New Program Officer Dr. Jonathan Wynn 2 Meet the EAR Staff 3 Broader Impacts ­ Examples from the Ground 3 The Division of Earth Sciences (EAR) is part of the Geosciences Directorate at the National Science Foundation. News from

118

Ear Biometrics in Human Identification A Dissertation  

E-print Network

Ear Biometrics in Human Identification A Dissertation Submitted to the Graduate School and Engineering Notre Dame, Indiana June 2006 #12;Ear Biometrics in Human Identification Abstract by Ping Yan the ear as a biometric and investigate its potential with both 2D and 3D data. Our work is the largest

Bowyer, Kevin W.

119

Designing Medical Devices for the Ear  

NSDL National Science Digital Library

Students are introduced to engineering, specifically to biomedical engineering and the engineering design process, through a short lecture and an associated hands-on activity in which they design their own medical devices for retrieving foreign bodies from the ear canal. Through the lesson, they learn the basics of ear anatomy and how ear infections occur and are treated. Besides antibiotic treatment, the most common treatment for chronic ear infections is the insertion of ear tubes to drain fluid from the middle ear space to relieve pressure on the ear drum. Medical devices for this procedure, a very common children's surgery, are limited, sometimes resulting in unnecessary complications from a simple procedure. Thus, biomedical engineers must think creatively to develop new solutions (that is, new and improved medical devices/instruments) for inserting ear tubes into the ear drum. The class learns the engineering design process from this ear tube example of a medical device design problem. In the associated activity, students explore biomedical engineering on their own by designing prototype medical devices to solve another ear problem commonly experienced by children: the lodging of a foreign body (such as a pebble, bead or popcorn kernel) in the ear canal. The activity concludes by teams sharing and verbally analyzing their devices.

2014-09-18

120

Immunologic Disorders of the Inner Ear.  

ERIC Educational Resources Information Center

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)

Kinney, William C.; Hughes, Gordon B.

1997-01-01

121

Can Loud Music Hurt My Ears?  

MedlinePLUS

... Main Page The Pink Locker Society Can Loud Music Hurt My Ears? KidsHealth > Kids > Q&A > Q & A > 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 ...

122

Pulmonary arteriovenous malformations: therapeutic options.  

PubMed

We have treated 21 patients (13 female, 8 male) with pulmonary arteriovenous malformations (PAVMs). Mean age at diagnosis was 37.5 years (range, 15 to 72 years). Presenting symptoms included dyspnea on exertion (67%), hereditary hemorrhagic telangiectasia (57%), and major neurologic events (33%). In our early experience, 8 patients had no specific treatment; their case histories illustrate the major neurologic complications of untreated PAVMs. Nine patients (8 primarily, 1 after recurrence) underwent conservative surgical excision; 4 had lobectomy, and 5 had segmentectomy or subsegmental excision. One patient underwent staged bilateral thoracotomies for multiple bilateral lesions. The arterial oxygen tension was found to increase after excision of large or solitary PAVMs. All surgically treated patients were relieved of dyspnea, and none had postoperative recurrence of PAVMs or neurologic complications related to PAVMs. Five patients underwent balloon occlusion of PAVMs. Two patients chose to have solitary PAVMs occluded rather than undergo thoracotomy. One underwent surgical excision 5 years later, and the other required repeat balloon embolization 4 years later when recanalization of the PAVMs was documented. Three patients with numerous PAVMs received palliation with multiple balloon embolizations. The high incidence of associated major neurologic complications mandates aggressive treatment of PAVMs whenever feasible. Conservative surgical resection remains the treatment of choice. Balloon embolization offers an alternative therapy for patients who are poor surgical risks or those whose lesions are too numerous to resect. PMID:8347006

Puskas, J D; Allen, M S; Moncure, A C; Wain, J C; Hilgenberg, A D; Wright, C; Grillo, H C; Mathisen, D J

1993-08-01

123

Chiari Malformation in otology practice.  

PubMed

The purpose of the study was to evaluate prevalence, characteristic symptoms, and management of Chiari Malformation 1 (CM1). A retrospective chat review was made in Otology Tertiary Department including 439 otologic patients referred to the Helsinki University Hospital Radiology Department for head magnetic resonance imaging (MRI) during 2005 and also among 42 patients seen at the Department of Neurosurgery in years 2001-2005 with a diagnosis of CM1. We made a structured analysis of medical records focusing on patient history, neurologic symptoms, and radiologic findings. For surgical patients, information was collected on symptoms, treatment, and operative outcome. The prevalence of CM1 in the 439 otologic patients was 0.9%. Most CM1 patients sent to the Neurosurgery Department were operated on. Two months postoperatively, 26 patients (68%) had benefited from the surgery, but 12 patients (32%) experienced no change to symptoms. The possibility of CM1 should be borne in mind in patients presenting with atypical benign positional vertigo or recurrent facial paresis. PMID:19409739

Levo, Hilla; Tapani, Erna; Karppinen, Atte; Kentala, Erna

2010-02-01

124

The Effect of Ear Canal Pressure on Spontaneous Otoacoustic Emissions:. Comparison Between Human and Lizard Ears  

NASA Astrophysics Data System (ADS)

The center frequency, height and width of peaks in SOAE spectra depend on ear canal pressure. The width is interpreted as a measure of the inner ear source-signal-to-(e.g. thermal)-noise ratio. In humans, width increases with decreasing height. Apparently, ear canal pressure modifies the amplitude of the inner ear emission source signal. In lizards, the relation between peak width and height is not consistent. Here, middle ear transmission changes may account for many the observed amplitude effects.

van Dijk, P.; Manley, G. A.

2009-02-01

125

Delayed presentation of anorectal malformations  

PubMed Central

Aims and Objectives: Delayed management of anorectal malformation (ARM) increases the surgical and functional complications for the patient. We defined “delayed presentation of ARM” and reviewed our patients with ARM to find out the incidence and causes of delayed presentation. Materials and Methods: Patients satisfying the criteria of “delayed presentation of ARM” were involved. Detailed information of each patient including the mode of presentation, associated anomalies, plan of management and follow-up was obtained from the hospital records. Results: Between 2003 and 2006, 43 patients satisfied our criteria of “delayed presentation of ARM”. There were 21 males and 22 females. Seventeen of these males presented with low-type ARM. Eleven of them were managed by a single-stage procedure. These “delayed presenters” had to live with constipation, inadequate weight gain and parental anxiety for a greater time. Analysis of the outcomes showed more functional complications in patients who had undergone failed perineal surgery previously. In females with low ARM, the procedure of choice was anterior sagittal anorectoplasty (ASARP). Single stage surgery provides good outcomes for most of low type of ARMs. High-type ARMs in males and females were managed by a staged procedure. Conclusion: “Delayed presentation of ARM” is a major group of ARM in our setup. The management and results of their treatment are not different from those of the early presenters. The most common cause of delayed ARM is wrong advice given by the health care providers followed by inadequate treatment elsewhere. Corrective surgeries taking second attempt in perineum always produces poor outcomes. PMID:20011470

Sinha, Shandip Kumar; Kanojia, Ravi P.; Wakhlu, Ashish; Rawat, J. D.; Kureel, S. N.; Tandon, R. K.

2008-01-01

126

Surgical treatment for venous malformation.  

PubMed

Sclerotherapy is generally the preferred treatment for venous malformation (VM) with surgery usually playing an adjunctive role. This study presents our experience with surgical treatment of VMs. Consecutive patients were identified from our vascular anomalies database 1996-2011 and patient demographics, location of the lesion, type of tissue(s) affected and symptoms were analysed. The patients completed a questionnaire to assess the impact of surgery on the severity of symptoms, appearance, function and overall quality of life (QoL), using a visual analogue scale of 0 (no symptom) to 10 (maximal symptom). They also rated their overall satisfaction of treatment using a scale of 0 (complete dissatisfaction) to 10 (complete satisfaction). Fifty patients with VM underwent a total of 58 procedures. Complication occurred in six patients (9.7% of operations), including transient sensory loss (n=3) and permanent frontal branch palsy (n=1), haematoma formation (n=1) and minor wound dehiscence (n=1). At least 50% improvement in symptoms of background pain, acute episodic pain, contour deformity and skin discolouration occurred in 88.9%, 92.3%, 83.3% and 75.0% of patients, respectively. At least 50% improvement in the appearance, function and overall QoL occurred in 54.3%, 71.4% and 70.4% of patients, respectively. The mean overall patient satisfaction with the treatment was 8.9 (range, 1-10). Surgery remains an important treatment modality for selected patients with VM having low complication rates and high patient satisfaction. It improves the appearance, function and overall QoL for the majority of the patients by reducing the severity of pain, contour deformity and skin discolouration. PMID:24012651

Steiner, Frederica; FitzJohn, Trevor; Tan, Swee T

2013-12-01

127

Inner Time and Inner Ear  

E-print Network

Sounds are information sequences that cannot exist outside of a time base and therefore cannot be analyzed inside an animal without an accurate internal clock. It is suggested that the clock may be hidden in the inner ear. It is shown that if a mechanism of counting of the electrical charge passing through the inner ear hair cells exists then the mechanism can be used both for the conversion of acceleration into velocity and as the inner clock, in the presence of a constant current. The causes of vertigo during rotation are discussed. It is shown that if a continuous inner time exists then sleeping is a "mathematical necessity". It is indicated that both for visual and hearing inputs the recognition of an input signal is recognition of function(s) of two variables.

Michael Rvachov

2012-10-30

128

Ear canal dynamic motion as a source of power for in-ear devices  

NASA Astrophysics Data System (ADS)

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.

Delnavaz, Aidin; Voix, Jérémie

2013-02-01

129

Percutaneous Cryotherapy of Vascular Malformation: Initial Experience  

SciTech Connect

The present report describes a case of percutaneous cryotherapy in a 36-year-old woman with a large and painful pectoral venous malformation. Cryoablation was performed in a single session for this 9-cm mass with 24 h hospitalisation. At 2- and 6-month follow-up, the pain had completely disappeared, and magnetic resonance imaging demonstrated a significant decrease in size. Percutaneous cryoablation shows promise as a feasible and apparently safe method for local control in patients with symptomatic venous vascular malformations.

Cornelis, F., E-mail: francoiscornelis@hotmail.com [Institut Bergonie, Department of Radiology (France); Neuville, A. [Institut Bergonie, Department of Pathology (France); Labreze, C. [Pellegrin Hospital, Department of Pediatric Dermatology (France); Kind, M. [Institut Bergonie, Department of Radiology (France); Bui, B. [Institut Bergonie, Department of Oncology (France); Midy, D. [Pellegrin Hospital, Department of Vascular Surgery (France); Palussiere, J. [Institut Bergonie, Department of Radiology (France); Grenier, N. [Pellegrin Hospital, Department of Radiology (France)

2013-06-15

130

[Hyperkeratotic vascular malformations. Presentation of three cases].  

PubMed

Hyperkeratotic vascular malformations (verrucous hemangiomas) are infrequent vascular lesions present from birth. Initially, they are reminiscent of port wine stains or childhood hemangioma, but over time they gradually take on their typical warty and hyperkeratotic appearance. These changes are probably due to trauma, scratching and bleeding episodes. Because the lesion is deep and goes beyond the clinical lesion, treatment is difficult and involves many recurrences. We present three cases of this vascular malformation, in two males aged 45 and 62 and a 30-year-old female. All three had the lesion from birth, and presented with frequent bleeding episodes. PMID:16476321

Piqué, Enric; Pérez-Cejudo, Juan A; Palacios, Santiago; Martínez, María Sol

2005-12-01

131

Sequelae of dental trauma: the malformed tooth  

PubMed Central

Here, we report a case of 10-year-old boy who came with a complaint of missing upper front teeth and was also concerned about his aesthetics. Significant history of trauma was present in his toddler period. Diagnosis of malformed upper right central incisor (11) by radiological investigations was carried out. Surgery was performed and there was removal of the impacted malformed upper right central incisor (11). Healing was uneventful. The patient is under follow-up for aesthetic rehabilitation to be carried out. PMID:23362061

Chaudhary, Seema; Chaitra, T R; Vijayran, Manisha; Kulkarni, Adwait Uday

2013-01-01

132

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

NASA Astrophysics Data System (ADS)

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.

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

2013-06-01

133

Congenital pulmonary airway malformation (CPAM) [congenital cystic adenomatoid malformation] associated with tracheoesophageal fistula and agensesis of the corpus callosum.  

PubMed

Congenital pulmonary airway malformations (CPAM) are a family of hamartomatous disorders due to the uncontrolled overgrowth of the terminal bronchioles. Congenital pulmonary airway malformations can co-exist with cardiovascular and/or urogenital malformations, but their association with thoracopulmonary malformations is extremely rare. We report the first case of CPAM type I, co-existing with tracheo-esophageal fistula and corpus callosum agenesis. PMID:22414029

Pizzi, Marco; Fassan, Matteo; Ludwig, Kathrin; Cassina, Matteo; Gervasi, Maria Teresa; Salmaso, Roberto

2012-06-01

134

Molecular Mechanisms of Inner Ear Development  

PubMed Central

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

Wu, Doris K.; Kelley, Matthew W.

2012-01-01

135

Angiographically occult arteriovenous malformations of the brain  

PubMed Central

Six patients with cerebral arteriovenous malformations which did not show any pathological circulation at angiography are described. Computed tomogram appearances of such lesions may be difficult to distinguish from tumours. The need for surgical exploration in localised high attenuation lesions of uncertain nature is stressed, and the literature is reviewed. Images PMID:731249

Bell, B. A.; Kendall, B. E.; Symon, L.

1978-01-01

136

Segmental costovertebral malformation associated with lipomyelomeningocoele.  

PubMed

We describe 2 patients with segmental costovertebral malformation, a form of spondylocostal dysostosis, associated with tethering of the conus to a lipomyelomeningocoele. Such an association is rare. In both these patients the defects occurred sporadically. The relevant literature is reviewed. PMID:15936198

Nadkarni, Trimurti D; Menon, Ram Kumar; Desai, Ketan I; Goel, Atul

2005-06-01

137

Additional congenital defects in anorectal malformations  

Microsoft Academic Search

From 1974 until 1995 a total of 264 (141 ?, 123 ?) patients born with an anorectal malformation (ARM) were referred to the University Hospital Nijmegen in the Netherlands. All additional congenital defects (ACDs) were registered. Special attention was paid to whether the ACDs take part in associations, syndromes, or sequences. One or more ACDs were observed in 67% of

E. A. M. Hassink; P. N. M. A. Rieu; B. C. J. Hamel; R. S. V. M. Severijnen; F. H. J. M. van der Staak; C. Festen

1996-01-01

138

Fear the EAR: Discovering and Mitigating Execution After Redirect Vulnerabilities  

E-print Network

Fear the EAR: Discovering and Mitigating Execution After Redirect Vulnerabilities Adam Doupé, Bryce Execution After Redirect, or EAR. A web application de- veloper can introduce an EAR by calling a redirect web frameworks are to EAR vulnerabilities. We then discuss the results from the EAR challenge

California at Santa Barbara, University of

139

Congenital vascular malformations in scintigraphic evaluation  

PubMed Central

Summary Background Congenital vascular malformations are tumour-like, non-neoplastic lesions caused by disorders of vascular tissue morphogenesis. They are characterised by a normal cell replacement cycle throughout all growth phases and do not undergo spontaneous involution. Here we present a scintigraphic image of familial congenital vascular malformations in two sisters. Material/Methods A 17-years-old young woman with a history of multiple hospitalisations for foci of vascular anomalies appearing progressively in the upper and lower right limbs, chest wall and spleen. A Parkes Weber syndrome was diagnosed based on the clinical picture. Due to the occurrence of new foci of malformations, a whole-body scintigraphic examination was performed. A 12-years-old girl reported a lump in the right lower limb present for approximately 2 years, which was clinically identified as a vascular lesion in the area of calcaneus and talus. Phleboscintigraphy visualized normal radiomarker outflow from the feet via the deep venous system, also observed in the superficial venous system once the tourniquets were released. In static and whole-body examinations vascular malformations were visualised in the area of the medial cuneiform, navicular and talus bones of the left foot, as well as in the projection of right calcaneus and above the right talocrural joint. Conclusions People with undiagnosed disorders related to the presence of vascular malformations should undergo periodic follow-up to identify lesions that may be the cause of potentially serious complications and to assess the results of treatment. Presented scintigraphic methods may be used for both diagnosing and monitoring of disease progression. PMID:24567769

Pilecki, Stanis?aw; Gierach, Marcin; Gierach, Joanna; ?wi?taszczyk, Cyprian; Junik, Roman; Lasek, W?adys?aw

2014-01-01

140

Cleft Lip and Palate Associated with Other Malformations in a Neotropical Primate (Saimiri ustus)  

PubMed Central

Cleft lip (with or without cleft palate) has been documented in several species of nonhuman primates, which in general are susceptible at similar doses and stages of gestation to the same teratogens as humans. Cleft lip can be unilateral or bilateral, isolated, syndromic, familial, or genetic. Here we report the first case of syndromic cleft lip and palate in a male bare-eared squirrel monkey (Saimiri ustus). Associated with the orofacial clefts, the monkey manifested absence of bones, malformation of vertebrae L3, only 4 fingers in each hand, and shortening of tendons leading to inflection of the hands and fingers. Previous reports describing cleft lip and palate in other squirrel monkeys (Saimiri sciureus) in other breeding units have suggested consanguineous mating as a possible cause. Although the etiology in the case we present is unknown, we discuss factors associated with orofacial clefts in humans and various nonhuman primates. PMID:20587169

Goldschmidt, Beatriz; Lopes, Claudia AA; Moura, Marina; Nogueira, Denise M; Gonçalves, Miguel AB; Fasano, Daniele M; Andrade, Marcia CR; Nascimento, Laine WF; Marinho, Antonio M

2010-01-01

141

Temporal lobe arteriovenous malformations: anatomical subtypes, surgical strategy, and outcomes  

PubMed Central

Object Descriptions of temporal lobe arteriovenous malformations (AVMs) are inconsistent. To standardize reporting, the authors blended existing descriptions in the literature into an intuitive classification with 5 anatomical subtypes: lateral, medial, basal, sylvian, and ventricular. The authors’ surgical experience with temporal lobe AVMs was reviewed according to these subtypes. Methods Eighty-eight patients with temporal lobe AVMs were treated surgically. Results Lateral temporal lobe AVMs were the most common (58 AVMs, 66%). Thirteen AVMs (15%) were medial, 9 (10%) were basal, and 5 (6%) were sylvian. Ventricular AVMs were least common (3 AVMs, 3%). A temporal craniotomy based over the ear was used in 64%. Complete AVM resection was achieved in 82 patients (93%). Four patients (5%) died in the perioperative period (6 in all were lost to follow-up); 71 (87%) of the remaining 82 patients had good outcomes (modified Rankin Scale scores 0–2); and 68 (83%) were unchanged or improved after surgery. Conclusions Categorization of temporal AVMs into subtypes can assist with surgical planning and also standardize reporting. Lateral AVMs are the easiest to expose surgically, with circumferential access to feeding arteries and draining veins at the AVM margins. Basal AVMs require a subtemporal approach, often with some transcortical dissection through the inferior temporal gyrus. Medial AVMs are exposed tangentially with an orbitozygomatic craniotomy and transsylvian dissection of anterior choroidal artery and posterior cerebral artery feeders in the medial cisterns. Medial AVMs posterior to the cerebral peduncle require transcortical approaches through the temporooccipi tal gyrus. Sylvian AVMs require a wide sylvian fissure split and differentiation of normal arteries, terminal feeding arteries, and transit arteries. Ventricular AVMs require a transcortical approach through the inferior temporal gyrus that avoids the Meyer loop. Surgical results with temporal lobe AVMs are generally good, and classifying them does not offer any prediction of surgical risk. PMID:23848823

Canals, Andreu Gabarrós; Rodríguez-Hernández, Ana; Young, William L.; Lawton, Michael T.

2014-01-01

142

Pediatric Obesity and Ear, Nose, and Throat Disorders  

MedlinePLUS

Pediatric Obesity and Ear, Nose, and Throat Disorders Pediatric Obesity and Ear, Nose, and Throat Disorders Patient Health Information ... self-esteem, and isolation from their peers. Pediatric obesity and otolaryngic problems Otolaryngologists, or ear, nose, and ...

143

Cutaneous lesions of the external ear  

PubMed Central

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

Sand, Michael; Sand, Daniel; Brors, Dominik; Altmeyer, Peter; Mann, Benno; Bechara, Falk G

2008-01-01

144

Cutaneous lesions of the external ear  

Microsoft Academic Search

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

Michael Sand; Daniel Sand; Dominik Brors; Peter Altmeyer; Benno Mann; Falk G Bechara

2008-01-01

145

Playing by Ear: Foundation or Frill?  

ERIC Educational Resources Information Center

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…

Woody, Robert H.

2012-01-01

146

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...

147

Review Article Mechanics of the frog ear  

E-print Network

Review Article Mechanics of the frog ear Pim Van Dijk a,b,*, Matthew J. Mason c , Richard L s t r a c t The frog inner ear contains three regions that are sensitive to airborne sound and which of the frog's auditory range. It shares the ability to generate both evoked and spontaneous otoacoustic

Allen, Jont

148

"Hot Tub Rash" and "Swimmer's Ear" (Pseudomonas)  

MedlinePLUS

Facts About “Hot Tub Rash” and “Swimmer’s Ear” (Pseudomonas) What is Pseudomonas and how can it affect me? Pseudomonas (sue-doh- ... a major cause of infections commonly known as “hot tub rash” and “swimmer’s ear.” This germ is ...

149

Gene therapy for the inner ear  

PubMed Central

Animal studies on inner ear development, repair and regeneration provide understanding of molecular pathways that can be harnessed for treating inner ear disease. Use of transgenic mouse technology, in particular, has contributed knowledge of genes that regulate development of hair cells and innervation, and of molecular players that can induce regeneration, but this technology is not applicable for human treatment, for practical and ethical reasons. Therefore other means for influencing gene expression in the inner ear are needed. We describe several gene vectors useful for inner ear gene therapy and the practical aspects of introducing these vectors into the ear. We then review the progress toward using gene transfer for therapies in both auditory and balance systems, and discuss the technological milestones needed to advance to clinical application of these methods. PMID:23265411

Fukui, Hideto; Raphael, Yehoash

2012-01-01

150

[Cochlear damage caused by middle ear surgeries].  

PubMed

A prospective study was set up to study the reaction of the cochlea after the trauma of middle-ear surgery. For this purpose, the bone conduction of 50 patients was tested every day, beginning on the first post-operative day. To collect information on possible damaging mechanisms, three surgical techniques were studied: Stapes surgery with the opening of the inner ear; mastoidectomy with drill-generated noise; tympanoplasty with manipulations at the stapes. The comparison of the bone conduction thresholds with audiometry results after completed healing of the ear, disclosed that even under the ear-packing, bone conduction can give reliable information on cochlea function, if a 10-15 dB variance due to methodological causes is taken into account. Excessive drilling may result in a temporary threshold shift, which has already resolved at the time of unpacking the ear. No signs of hydraulic damage after manipulation at the stapes could be discovered. PMID:2029305

Hüttenbrink, K B

1991-02-01

151

Biometric recognition using 3D ear shape.  

PubMed

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. PMID:17568136

Yan, Ping; Bowyer, Kevin W

2007-08-01

152

Arteriovenous malformation detected by small bowel endoscopy.  

PubMed

Gastrointestinal bleeding that originates in the small intestine is often difficult to diagnose. When successful diagnosis reveals a lesion that can be localized preoperatively, the laparoscopic approach is an appropriate and beneficial treatment modality for small bowel resection. A 69-year-old man presented with a 6-month history of gastrointestinal bleeding and symptomatic transfusion-dependent anemia. Upper and lower endoscopy were normal. Double-balloon endoscopy established the source of the bleeding as a 0.5-cm polypoid mass appearing as a submucosal tumor with redness and pulsation in the lower ileum, suggesting a vascular lesion. Laparoscopic small bowel resection was successful in removing the mass in the ileum. Histological evaluation of the mass revealed an arteriovenous malformation. Preoperative small bowel endoscopy can be useful for diagnosing the cause and localization of arteriovenous malformation in the small intestine. PMID:25473391

Fujii, Takaaki; Morita, Hiroki; Sutoh, Toshinaga; Takada, Takahiro; Tsutsumi, Soichi; Kuwano, Hiroyuki

2014-09-01

153

Laser treatment of congenital vascular malformations.  

PubMed

Treatment of vascular malformations is one of the main indications for laser application in dermatology. The argon laser is suitable for coagulation of superficial lesions, the Nd:YAG laser for thicker ones. Vaporization or excision of vascular lesions can be performed by means of the CO2 laser. International standard of argon laser therapy of port wine stains are 60 to 70% good results in adult patients afflicted with red and purple lesions. In contrast argon laser therapy of pink lesions and of children is less successful. But more recently the flashlamp pumped dye laser has improved treatment results in children, since this laser acts much more vessel specific than the argon laser. Other congenital vascular lesions that respond to laser therapy are capillary hemangiomas, cavernous hemangiomas, and lymphangiomas. Future development may expand application of laser in the treatment of vascular malformations. PMID:2090705

Landthaler, M; Hohenleutner, U

1990-01-01

154

Dandy–Walker malformation: An incidental finding  

PubMed Central

Dandy–Walker malformation (DWM) is a rare intracranial congenital abnormality that affects the cerebellum and some of its components; particularly cerebellar vermis, fourth ventricle and is characterized by an enlarged posterior fossa. Although there is an extensive list of signs attributed to DWM, final diagnosis is solely dependent on imaging techniques as there are no signs that are characteristic of DWM. This article reports a case with DWM who was diagnosed by magnetic resonance imaging. PMID:20838490

Tadakamadla, Jyothi; Kumar, Santhosh; Mamatha, G. P.

2010-01-01

155

Embolization and radiosurgery for arteriovenous malformations  

PubMed Central

The treatment of arteriovenous malformations (AVMs) requires a multidisciplinary management including microsurgery, endovascular embolization, and stereotactic radiosurgery (SRS). This article reviews the recent advancements in the multimodality treatment of patients with AVMs using endovascular neurosurgery and SRS. We describe the natural history of AVMs and the role of endovascular and radiosurgical treatment as well as their interplay in the management of these complex vascular lesions. Also, we present some representative cases treated at our institution. PMID:22826821

Plasencia, Andres R.; Santillan, Alejandro

2012-01-01

156

Malformations of cortical development and epilepsy  

PubMed Central

Malformations of cortical development (MCDs) are macroscopic or microscopic abnormalities of the cerebral cortex that arise as a consequence of an interruption to the normal steps of formation of the cortical plate. The human cortex develops its basic structure during the first two trimesters of pregnancy as a series of overlapping steps, beginning with proliferation and differentiation of neurons, which then migrate before finally organizing themselves in the developing cortex. Abnormalities at any of these stages, be they environmental or genetic in origin, may cause disruption of neuronal circuitry and predispose to a variety of clinical consequences, the most common of which is epileptic seizures, A large number of MCDs have now been described, each with characteristic pathological, clinical, and imaging features. The causes of many of these MCDs have been determined through the study of affected individuals, with many MCDs now established as being secondary to mutations in cortical development genes. This review will highlight the best-known of the human cortical malformations associated with epilepsy. The pathological, clinical, imaging, and etioiogic features of each MCD will be summarized, with representative magnetic resonance imaging (MRI) images shown for each MCD, The malformations tuberous sclerosis, focal cortical dysplasia, hemimegalencephaiy, classical iissencephaly, subcortical band heterotopia, periventricular nodular heterotopia, polymicrogyria, and schizencephaly will be presented. PMID:18472484

Leventer, Richard J.; Guerrini, Renzo; Dobyns, William B.

157

Congenital lung malformations: an ongoing controversy  

PubMed Central

Introduction Congenital lung malformations are rare lesions that are most commonly diagnosed antenatally. Management of such lesions, particularly those that are asymptomatic, remains controversial. We undertook a survey to ascertain current practice of surgeons in the UK and Ireland. Methods All consultant members of the British Association of Paediatric Surgeons were asked to complete a survey on congenital lung malformations with respect to antenatal management, symptomatic and asymptomatic lesions, and operative techniques. Results Responses were received from 20 paediatric surgical centres and highlighted the ongoing variability in management of such lesions, particularly those that are asymptomatic. Twenty per cent of surgeons never resect an asymptomatic lesion and twenty-four per cent always do. The remainder intervene selectively, with size being the most commonly stated indication. Most resections are undertaken via thoracotomy although 35% of surgeons use thoracoscopy for some procedures. Conclusions National data based on congenital anomaly registers are needed to determine the natural history of these malformations and to guide future management. PMID:23484999

Burge, DM; Marven, SS

2013-01-01

158

Congenital Intracranial Vascular Malformations in Children*  

PubMed Central

The clinical, laboratory, and other features of 16 children with congenital intracranial vascular malformations seen over a period of 15 years are presented. 13 patients had an arteriovenous (AV) malformation, 2 patients an aneurysm of the vein of Galen, and 1 a congenital internal carotid/internal jugular fistula. Based on onset of symptoms the patients ranged in age from 3 months to 16 years with an average age of 7½ years; however, 5 patients had their first clinical manifestation before 6 years of age. Unfortunately, definitive diagnosis could not be made until an average of 15½ years. The most important clinical manifestations were focal or generalized seizures and spontaneous intracranial bleeding, each occurring in 6 patients. Of 2 patients who presented with hydrocephalus, 1 had had an unexplained episode of cardiac failure during infancy. Inequality in the size of the legs, and periodic headaches were the initial manifestations in 2 other patients. Analysis of this series of patients with intracranial AV malformations suggests the following diagnostic recommendations: careful auscultation of the skull for bruits should be performed in all infants and children with cardiac failure of unknown aetiology; patients with focal seizures refractory to anticonvulsant therapy should be re-examined at frequent intervals to detect focal neurological deficit which otherwise may go unnoticed; and, finally, patients with a seizure disorder who develop focal signs of neurological deficit deserve a complete diagnostic investigation, including contrast studies. ImagesFIG. 1FIG. 2FIG. 3FIG. 4 PMID:5314563

Lagos, Jorge C.; Riley, Harris D.

1971-01-01

159

Cytoarchitecture and Transcriptional Profiles of Neocortical Malformations in Inbred Mice  

PubMed Central

Malformations of neocortical development are associated with cognitive dysfunction and increased susceptibility to epileptogenesis. Rodent models are widely used to study neocortical malformations and have revealed important genetic and environmental mechanisms that contribute to neocortical development. Interestingly, several inbred mice strains commonly used in behavioral, anatomical, and/or physiological studies display neocortical malformations. In the present report we examine the cytoarchitecture and myeloarchitecture of the neocortex of 11 inbred mouse strains and identified malformations of cortical development, including molecular layer heterotopia, in all but one strain. We used in silico methods to confirm our observations and determined the transcriptional profiles of cells found within heterotopia. These data indicate cellular and transcriptional diversity present in cells in malformations. Furthermore, the presence of dysplasia in nearly every inbred strain examined suggests that malformations of neocortical development are a common feature in the neocortex of inbred mice. PMID:18308707

Ramos, Raddy L.; Smith, Phoebe T.; DeCola, Christopher; Tam, Danny; Corzo, Oscar

2008-01-01

160

The Ear Pages - Nobel Prize Educational Game  

NSDL National Science Digital Library

The 1961 Nobel Prize in Physiology or Medicine was awarded for the discovery of how sound is analyzed and communicated in the cochlea in the inner ear. Explore "The Ear Pages" and collect the snail shaped symbols of the cochlea to gain chances to answer a question correct in the quiz! (The cochlea works as a frequency/pitch analyzer in the inner ear.) You can choose between three levels of quizzes Â? beginner, advanced and expert. If you manage to get all the answers correct you will appear on the "High score of the week" list!

2009-01-01

161

Hearing: How Do Our Ears Work?  

NSDL National Science Digital Library

Students learn about the anatomy of the ear and how the ears work as a sound sensor. Ear anatomy parts and structures are explained in detail, as well as how sound is transmitted mechanically and then electrically through them to the brain. Students use LEGO® robots with sound sensors to measure sound intensities, learning how the NXT brick (computer) converts the intensity of sound measured by the sensor input into a number that transmits to a screen. They build on their experiences from the previous activities and establish a rich understanding of the sound sensor and its relationship to the TaskBot's computer.

2014-09-18

162

Gouty tophi on the ear: a review.  

PubMed

Although the classic location of gouty tophi is the great toe (podagra), gouty tophi of the ear also is common and is worth including in the differential diagnosis in patients presenting with ear lesions. Other entities presenting as papules or nodules on the ear include chondrodermatitis nodularis helicis (CNH), actinic keratosis, basal cell carcinoma, squamous cell carcinoma, verruca vulgaris, amyloids, rheumatoid nodules, and elastotic nodules. If tophaceous gout is suspected, alcohol fixation of the biopsy specimen is preferable, as it enables visualization of characteristic needle-shaped urate crystals. PMID:24195091

Chabra, Indy; Singh, Rajendra

2013-10-01

163

EARS: Electronic Access to Reference Service.  

PubMed

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

Weise, F O; Borgendale, M

1986-10-01

164

Extracranial vascular malformations (hemangiomas and vascular malformations) in children and adolescents – diagnosis, clinic, and therapy  

PubMed Central

The field of extracranial vascular anomalies is considered as special focus of pediatric otolaryngology and it has shown a rapid development during the last years. The reason for this interest is finally also due to the global acceptance of the classification introduced by the ISSVA (International Society for the Study of Vascular Anomalies). Hemangiomas are the most frequently observed vascular tumors. Today the systemic propranolol therapy is mostly used for therapy of hemangiomas requiring treatment. Increasingly, the topical application of beta blocker is discussed while the benefit in the head and neck seems to be limited. Vascular malformations are classified according to the morphology of the affected part of the vascular system in arterial, venous, arterio-venous, lymphatic, capillary, and combined vascular malformations. Conventional surgery, sclerosing therapy, and laser treatment are invasive options for the treatment of lymphatic malformations. The options for the treatment of venous malformations could be significantly improved during the last years. In this context, the use of Nd:YAG laser, the conservative treatment of the localized disseminated intravascular coagulation with low-molecular weight heparin, the re-discovery of bleomycin as effective sclerosing agent, and the improvement of alcohol-based embolization agents must be mentioned. Today the treatment with dye laser is the preferred therapy for capillary malformations and it is superior to other therapeutic options as for example photodynamic therapy. Arterio-venous malformations as representatives for high-flow lesions are the high-risk lesions. Frequently they are compared to malignant head and neck tumors, in particular when a curative treatment can no longer be assured because of diffuse or multifocal extent and when the disease shows a progressive course. The combined treatment of embolization and surgical resection and if necessary consecutive defect reconstruction have turned out to be appropriate for arterio-venous malformations. Incurable findings are still a major challenge. Despite the introduction of antiangiogenetic drugs in oncology, the medicamentous therapeutic approach could not be established for arterio-venous malformations up to now. PMID:25587362

Eivazi, Behfar; Werner, Jochen A.

2014-01-01

165

Macrocephaly–capillary malformation syndrome: Three new cases  

Microsoft Academic Search

Overgrowth syndromes can be associated with asymmetry, obesity and various vascular malformations. Macrocephaly–Capillary Malformation (M–CM) is a more recently defined overgrowth syndrome characterized by cutaneous capillary malformation occurring in association with macrocephaly with tendency to progressive enlargement, abnormalities of somatic growth with body asymmetry including brain asymmetry, developmental delay, typical face with full cheeks, nevus flammeus of the nose and\\/or

Inusha Panigrahi; Mani Bhushan; Mukesh Yadav; Niranjan Khandelwal; Pratibha Singhi

166

Hemangiomas and Vascular Malformations: Current Theory and Management  

PubMed Central

Vascular anomalies are a heterogeneous group of congenital blood vessel disorders more typically referred to as birthmarks. Subcategorized into vascular tumors and malformations, each anomaly is characterized by specific morphology, pathophysiology, clinical behavior, and management approach. Hemangiomas are the most common vascular tumor. Lymphatic, capillary, venous, and arteriovenous malformations make up the majority of vascular malformations. This paper reviews current theory and practice in the etiology, diagnosis, and treatment of these more common vascular anomalies. PMID:22611412

Richter, Gresham T.; Friedman, Adva B.

2012-01-01

167

Temporal variability in birth prevalence of cardiovascular malformations  

Microsoft Academic Search

OBJECTIVETo investigate changes over time in the prevalence at live birth of cardiovascular malformations and to compare “anatomical” and “physiological” diagnostic hierarchies within a population.DESIGNRetrospective and prospective ascertainment of all congenital cardiovascular malformations diagnosed in infancy.SETTINGThe resident population of one health region.PATIENTSAll infants live born from 1985 to 1997 with cardiovascular malformations confirmed by echocardiography, cardiac catheterisation, surgery or autopsy.MAIN

C Wren; S Richmond; L Donaldson

2000-01-01

168

A Cluster of Hypoplastic Left Heart Malformation in Baltimore, Maryland  

Microsoft Academic Search

Congenital cardiovascular malformations (CCVMs) of the left side of the heart show familial recurrence of various forms of\\u000a obstructive malformations, including hypoplastic left heart (HLH), interrupted aortic arch, coarctation of the aorta, and\\u000a aortic stenosis. In a previous population-based study in the Baltimore–Washington region, these malformations were associated\\u000a with parental reports of occupational or leisure solvent exposure, overt diabetes, and

K. S. Kuehl; C. A. Loffredo

2006-01-01

169

Malformations Among the X-Linked Intellectual Disability Syndromes  

PubMed Central

Malformations are significant contributions to childhood mortality and disability. Their co-occurrence with intellectual disability may compound the health burden, requiring additional evaluation and management measures. Overall, malformations of greater or lesser severity occur in at least some cases of almost half of the 153 XLID syndromes. Genitourinary abnormalities are most common, but tend to contribute little or no health burden and occur in only a minority of cases of a given XLID syndrome. Some malformations (e.g., lissencephaly, hydranencephaly, long bone deficiency, renal agenesis/dysplasia) are not amenable to medical or surgical intervention; others (e.g., hydrocephaly, facial clefting, cardiac malformations, hypospadias) may be substantially corrected. PMID:24166814

Stevenson, Roger E.; Schwartz, Charles E.; Rogers, R. Curtis

2013-01-01

170

EGFR mutation of adenocarcinoma in congenital cystic adenomatoid malformation/congenital pulmonary airway malformation: a case report.  

PubMed

An 80-year-old man underwent right upper lobectomy for the resection of multiple cysts accompanied by a nodule. The pathological diagnosis was adenocarcinoma with surrounding atypical epithelial cell proliferation in a Type 1 congenital cystic adenomatoid malformation/congenital pulmonary airway malformation. There was epidermal growth factor receptor mutation in the adenocarcinoma and surrounding atypical epithelial cells that had proliferated. Malignant transformation of congenital cystic adenomatoid malformation/congenital pulmonary airway malformation may be related to the epidermal growth factor receptor pathway in this case, with atypical epithelial cell proliferation as a precursor. We emphasize the importance of complete resection of congenital cystic adenomatoid malformation/congenital pulmonary airway malformation and the possibility of treatment with epidermal growth factor receptor tyrosine kinase inhibitors in epidermal growth factor receptor-mutated cases. PMID:24482415

Hasegawa, Mizue; Sakai, Fumikazu; Arimura, Ken; Katsura, Hideki; Koh, Eitetsu; Sekine, Yasuo; Hiroshima, Kenzo

2014-03-01

171

An analysis of the acoustic input impedance of the ear.  

PubMed

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. PMID:23917695

Withnell, Robert H; Gowdy, Lauren E

2013-10-01

172

PURDUE EXTENSIONArrested Ear Development in Hybrid Corn PURDUE EXTENSION  

E-print Network

1 PURDUE EXTENSIONArrested Ear Development in Hybrid Corn BP-85-W PURDUE EXTENSION D I S E A S E S O F C O R N Arrested Ear Development in Hybrid Corn www.btny.purdue.edu Arrested ear develop- ment to the abnormal corn ear development caused by a number of stress factors, including the application of nonionic

Holland, Jeffrey

173

Purdue extensionDiplodia Ear Rot Purdue extension  

E-print Network

1 Purdue extensionDiplodia Ear Rot BP-75-W Purdue extension d i s e a s e s o f c o r n Diplodia Ear Rot Authors: Charles Woloshuk Kiersten Wise www.btny.purdue.edu Diplodia ear rot, caused Diplodia ear rot. Hybrid susceptibility and weather also contribute to disease development. This bulletin

Holland, Jeffrey

174

Technique Forms Working Inner Ear Cells  

MedlinePLUS

... cells in the inner ear detect head movements, gravity and sound. Researchers know the general scheme of ... Humans Gene Disruptions Associated with Autism Risk Computer Models Can Help Guide Ebola Response Diet Affects Autoinflammatory ...

175

Characterizing regeneration in the mammalian external ear  

PubMed Central

We have previously shown that MRL/MpJ mice have a capacity for regeneration instead of scar formation following an ear punch wound. Understanding the differences that occur between scar-free regeneration or repair with scarring will have great impact upon advances in skin tissue engineering. A key question that remains unanswered in the MRL/MpJ mouse model is whether regeneration was restricted to the ear or whether it extended to the skin. A histological analysis was conducted up to 4 months post-wounding, not only with 2-mm punch wounds to the ear but also to the skin on the backs of the same animals. MRL/MpJ mouse ear wounds regenerate faster than control strains, with enhanced blastema formation, a markedly thickened tip epithelium and reduced scarring. Interestingly, in the excisional back wounds, none of these regenerative features was observed and both the C57BL/6 control and MRL/MpJ mice healed with scarring. This review gives an insight into how this regenerative capacity may be due to evolutionary processes as well as ear anatomy. The ear is thin and surrounded on both sides by epithelia, and the dorsal skin is devoid of cartilage and under greater tensile strain. Analysis of apoptosis during ear regeneration is also discussed, assessing the role and expression of various members of the Bcl-2 family of proteins. Ongoing studies are focusing on de novo cartilage development in the regenerating ear, as well as understanding the role of downstream signalling cascades in the process. Identification of such signals could lead to their manipulation and use in a novel tissue-engineered skin substitute with scar-free integration. PMID:17005017

Metcalfe, Anthony D; Willis, Hayley; Beare, Alice; Ferguson, Mark W J

2006-01-01

176

Genetic Requirement for Pneumococcal Ear Infection  

PubMed Central

Background Ear infection or otitis media (OM) accounts for most bacterial respiratory infections in children in both developed and developing nations. Streptococcus pneumoniae, nontypeable Haemophilus influenzae, and Moraxella catarrhalis are the major OM pathogens. However, little is known about the genetic basis of bacterial OM largely due to practical difficulties in conducting research in ear infection models and genetically manipulating clinical isolates. Here, we report the first genome-scale in vivo screen for bacterial genes required for ear infection in a chinchilla model by signature tagged mutagenesis (STM), a high throughput mutant screen technique. Methodology/Principal Findings STM strains were constructed with a multi-drug resistant OM isolate ST556 (serotype 19F) and screened in a chinchilla OM model. Out of 5,280 mutants tested, 248 mutants were substantially underrepresented in the mutant pools recovered from the middle ear fluids of the infected chinchillas, indicating the impaired ability to survive and replicate in the middle ears due to genetic disruptions in the chromosome of strain ST556. Further DNA sequencing analysis mapped the mutations to 169 pneumococcal genes. Surprisingly, only 52 of these genes were required for pneumococcal nasopharyngeal colonization in a murine model. This infection site-specific gene requirement was verified by targeted mutagenesis in the selected genes. Conclusions/Significance These findings suggest that there are a subset of pneumococcal genes required for ear infection and that these may be distinct from those required for nasal colonization. Our data thus provide comprehensive gene targets for mechanistic understanding of pneumococcal ear infection. Finally, this study has also developed a model for future genome-scale search for virulence determinants in other pathogens associated with ear infections. PMID:18670623

Yang, Jun; O'Brien, Christopher J.; Lee, Scott L.; Mazurkiewicz, Joseph E.; Haataja, Sauli; Yan, Jing-Hua; Gao, George F.; Zhang, Jing-Ren

2008-01-01

177

Dermatologic diseases of the external ear.  

PubMed

The external ear is composed of the auricle (pinna) and the external auditory canal. Both of these structures contain elastic cartilage (except the earlobe) and a small amount of subcutaneous fat, which are covered by skin. The skin of the cartilaginous canal contains hair cells, sebaceous (lipid-producing) glands, and apocrine (ceruminous) glands; this is in contrast with the osseous canal, which contains neither glands nor hair follicles. The auricle is susceptible to environmental influences and trauma. Due to its exposed locale, the ear is particularly vulnerable to the effects of ultraviolet light and, consequently, to preneoplastic and neoplastic skin lesions. The ear also has a sound-receiving function and a location that is both visible and aesthetically obvious, thereby drawing considerable attention from the patient. Dermatologic diseases on the external ear are seen in a variety of medical disciplines. Dermatologists, otorhinolaryngologists, family practitioners, and general and plastic surgeons are regularly consulted about cutaneous lesions on the ear. These lesions can be grouped into three main categories: (1) infectious; (2) tumoral; and (3) noninfectious inflammatory. The purposes of this contribution are to review various dermatologic diseases of the external ear and to update current diagnosis and treatment information related to these conditions. PMID:24314388

Oztürkcan, Sedat; Oztürkcan, Serap

2014-01-01

178

Laser treatment of oral vascular malformations  

NASA Astrophysics Data System (ADS)

Oral Vascular Malformations (OVM) are congenital anomalies characterized by morph-structural and/or functional changes of nature in severity and extension. OVM can affect any type of vessels arterial, venous or lymphatic and any capillary or anatomical. They are divided into two categories: low and high flow. In this study were treated 40 patients with OVM with a range size from 2 mm to 44 mm; they were subjected to clinical examination supported by Colour-Doppler Ultrasound instrumental examination and only for doubt cases the Magnetic Resonance Imaging (MRI) was prescribed. Only low flow venous and capillary malformations were treated by GaAlAs laser (Wiser®, Lambda, Brindole,Italy, 980nm) and KTP laser (SmartLite®, DEKA, Florence, Italy, 532nm) with two different techniques: the Transmucosal Thermophotocoagulation (TMT) and the Intralesional Photocoagulation (ILP). These techniques permitted a good control of haemostasis, avoiding bleeding both during surgery and in the postoperative. It is obtained an excellent and good healing respectively in 10% and 60% of cases, a moderate and poor resolution respectively in 22.5% and 7.5% of cases. A clear diagnosis allowed the management of Venous malformations (VM) by laser devices with wavelengths highly absorbed in haemoglobin in safety and efficacy and according to the principles of minimal invasive surgery. The aim of this study was to verify if the laser is effective in the treatment of OVM for the purpose of the clinical findings and the postoperative course. The Authors concluded that the laser can be considered the "gold standard" for treating OVM.

Romeo, U.; Gaimari, G.; Mohsen, M.; Tenore, G.; Palaia, G.

2014-01-01

179

Intraventricular cavernous malformation radiologically mimicking meningioma.  

PubMed

We report a case of trigonal cavernous malformation (CM) radiologically mimicking meningioma. The computed tomographic (CT) head angiography and magnetic resonance imaging (MRI) showed a partially calcified lesion with slight contrast enhancement located in the area of the left atrium of lateral ventricle. The lesion was completely removed using microsurgery with a parieto-occipital transcortical approach. The resected mass was histologically confirmed as CM. CM should be considered as differential diagnosis in case of the atrial mass lesion due to lack of hemosiderin ring characteristically seen other seated CM. PMID:19119474

Jin, Sung-Chul; Ahn, Jae-Sung; Kwun, Byung-Duk; Kwon, Do Hoon

2008-11-01

180

Stereotactic radiosurgery of intracranial cavernous malformations.  

PubMed

Despite increasing worldwide experience, the role of stereotactic radiosurgery (SRS) in the management of cerebral cavernous malformations (CMs) remains controversial. Microsurgical excision of easily accessible CMs is typically safe; therefore, removal remains the gold standard for most of the symptomatic hemispheric lesions. However, there is now sufficient evidence supporting the use of SRS for the difficult cases. Waiting for the cumulative morbidity of the natural history to justify intervention does not serve the patient's interest, therefore, we argue for early radiosurgical intervention. Carefully designed randomized controlled trials might resolve controversies concerning the role of SRS in treating cerebral CMs. PMID:24093575

Nagy, Gábor; Kemeny, Andras A

2013-10-01

181

Congenital lung malformations: informing best practice.  

PubMed

The management of congenital lung malformations is controversial both in the prenatal and postnatal periods. This article attempts to inform best practice by reviewing the level of evidence with regard to prenatal diagnosis, prognosis, and management and postnatal management, including imaging, surgical indication, surgical approach, and risk of malignancy. We present a series of clinically relevant statements along those topics and analyze the evidence for each. In the end, we make a plea for an adequate description of the lesions, both before and after birth, which will allow future comparisons between management options and the initiation of prospective registries. PMID:25459011

Baird, Robert; Puligandla, Pramod S; Laberge, Jean-Martin

2014-10-01

182

Brain arteriovenous malformations: from diagnosis to treatment.  

PubMed

Brain arteriovenous malformations (bAVM) are a major cause of morbidity in young people. The main mode of presentation is with a cerebral bleeding or seizures, although nowadays, due to the increased use of MRI, more asymptomatic bAVMs are encountered. The clinical course of asymptomatic bAVMs seems to be mild in comparison to bAVMs that presented with a cerebral hemorrhage. This finding may lead to a paradigm shift regarding treatment of asymptomatic bAVMs. This review discusses the latest findings in bAVM epidemiology and natural history, and compares the optimal imaging modalities and best treatment options. PMID:21464809

Buis, D R; Van Den Berg, R; Lagerwaard, F J; Vandertop, W P

2011-03-01

183

Endovascular approaches to pial arteriovenous malformations.  

PubMed

Endovascular approaches to arteriovenous malformations (AVMs) are often necessary to define and help treat these often complex lesions. Angiography provides important information to help plan surgical or radiosurgical approaches. Modern embolization techniques allow AVMs to be treated with the goals of making surgery safer and easier, eliminating high-risk features in patients with AVMs who are otherwise not candidates for treatment, and even potentially curing the patient of the lesion. Liquid embolic agents have significantly advanced what is possible with endovascular treatment of AVMs. PMID:24994088

Sanborn, Matthew R; Park, Min S; McDougall, Cameron G; Albuquerque, Felipe C

2014-07-01

184

The pediatric Chiari I malformation: a review  

Microsoft Academic Search

Background  Both the diagnosis and treatment regimens for the Chiari I malformation (CIM) are varied and controversial. The present paper\\u000a analyzes the literature regarding this form of hindbrain herniation in regard to definition, anatomy, pathobiology, symptoms,\\u000a findings, treatment, and outcomes.\\u000a \\u000a \\u000a \\u000a Discussions  Appropriate literature germane to the CIM is reviewed and discussed. There is variation in the reported anatomy, outcome,\\u000a and treatment for

R. Shane Tubbs; Michael J. Lyerly; Marios Loukas; Mohammadali M. Shoja; W. Jerry Oakes

2007-01-01

185

Role of Embolization for Cerebral Arteriovenous Malformations  

PubMed Central

Cerebral arteriovenous malformations (AVMs) are complex high-flow lesions that can result in devastating neurological injury when they hemorrhage. Embolization is a critical component in the management of many patients with cerebral AVMs. Embolization may be used as an independent curative therapy or more commonly in an adjuvant fashion prior to either micro- or radiosurgery. Although the treatment-related morbidity and mortality for AVMs—including that due to microsurgery, embolization, and radiosurgery—can be substantial, its natural history offers little solace. Fortunately, care by a multidisciplinary team experienced in the comprehensive management of AVMs can offer excellent results in most cases.

Ellis, Jason A.; Lavine, Sean D.

2014-01-01

186

Oculocerebrocutaneous syndrome: the brain malformation defines a core phenotype  

PubMed Central

Background: Oculocerebrocutaneous syndrome (OCCS) is characterised by orbital cysts and anophthalmia or microphthalmia, focal aplastic or hypoplastic skin defects, skin appendages, and brain malformations. The eye and skin abnormalities are well described but the neuropathological features less so. To date, 28 patients with an unequivocal diagnosis of OCCS have been reported, with a preponderance of males. Objective: To evaluate the brain imaging studies, clinical records, photographs, and pathological material of two new and nine previously reported cases of OCCS. Results: There was a consistent pattern of malformations in eight of the 11 cases, consisting of frontal predominant polymicrogyria and periventricular nodular heterotopia, enlarged lateral ventricles or hydrocephalus, agenesis of the corpus callosum sometimes associated with interhemispheric cysts, and a novel mid-hindbrain malformation. The latter consisted of a giant and dysplastic tectum, absent cerebellar vermis, small cerebellar hemispheres in most cases, and a large posterior fossa fluid collection. Conclusions: The mid-hindbrain malformation appears pathognomonic for OCCS. The eye and skin features of OCCS show considerable overlap with several other syndromes, such as encephalocraniocutaneous lipomatosis, oculo-auriculo-vertebral spectrum, and focal dermal hypoplasia, none of which has a comparable pattern of brain malformations. In particular the unique mid-hindbrain malformation also distinguishes OCCS from related syndromes with comparable forebrain anomalies. The pattern of malformation described thus helps in differentiating OCCS from other entities. The mid-hindbrain malformation points to a defect of the mid-hindbrain organiser as the underlying pathogenic mechanism. PMID:15879499

Moog, U; Jones, M; Bird, L; Dobyns, W

2005-01-01

187

The cerebral cortex malformation in thanatophoric dysplasia: neuropathology and pathogenesis  

Microsoft Academic Search

Thanatophoric dysplasia (TD) is a relatively common, fatal form of chondrodysplastic dwarfism in which the cerebral cortex displays a unique and complex malformation. The malformation is characterized by a combination of abnormalities, which affect the temporal lobe most severely. Salient features include temporal lobe enlargement, deep transverse sulci across the inferomedial temporal surface, and hippocampal dysplasia. TD is caused by

Robert F. Hevner

2005-01-01

188

Long term respiratory outcomes of congenital thoracic malformations.  

PubMed

The advent of universal antenatal ultrasonography in many countries has revealed the full spectrum of congenital thoracic malformations (CTMs) and presented clinicians with a number of practical dilemmas to do with diagnosis and management. We present a review of the most common forms of CTMs, including congenital cystic adenomatoid malformation, bronchopulmonary sequestration, and lobar and segmental emphysema. PMID:22305631

Davenport, Mark; Eber, Ernst

2012-04-01

189

Field Guide to Malformations of Frogs and Toads  

E-print Network

Field Guide to Malformations of Frogs and Toads With Radiographic Interpretations U.S. Department and Toads With Radiographic Interpretations Carol U. Meteyer USGS National Wildlife Health Center Madison. Suggested citation: Meteyer, C.U. 2000. Field guide to malformations of frogs and toads with radiographic

Torgersen, Christian

190

Congenital Malformations in Perinatal Autopsies – A Study of 100 Cases  

PubMed Central

Background Congenital malformations remain a common cause of perinatal deaths and even though ultrasonogram can give fairly accurate diagnosis, perinatal autopsy is essential to confirm the diagnosis and look for associated malformations. Objectives To emphasize the importance of perinatal autopsy in diagnosing congenital malformations and to compare the same with the prenatal ultrasound findings. Methods The present study comprises 100 consecutive perinatal autopsies conducted after obtaining the approval from the Institutional Ethics Committee. In cases where prenatal ultrasound findings were available they were compared with the autopsy findings. Results Out of 100 perinatal autopsies, 44 cases were congenital anomalies with M:F = 1:1.5. Majority of the fetuses with congenital malformations (36.36%) were therapeutically terminated, Cental nervous system malformations being the commonest indication. The most common timing of therapeutic termination being 20 -24weeks. Congenital malformations were common between 35-39 weeks gestational age and birth weight range 350- 1000g. The malformations involving the central nervous system were commonest, seen in 15 cases (34.09%) followed by renal anomalies in 9 cases (20.45%) and multiple malformations in 7cases ( 15.91%). Autopsy confirmed the prenatal ultrasound findings in 50% of the cases, added to diagnosis in 29.54%, while it completely changed the primary diagnosis in 9.09% of the cases. Conclusion This study highlights the importance of perinatal autopsy in confirming the diagnosis of congenital anomalies by prenatal ultrasound findings. PMID:23373038

Andola, Uma S; AM, Anita; Ahuja, Mukta; Andola, Sainath K

2012-01-01

191

Stereotactic proton beam therapy for intracranial arteriovenous malformations  

Microsoft Academic Search

Purpose: To investigate hypofractionated stereotactic proton therapy of predominantly large intracranial arteriovenous malformations (AVMs) by analyzing retrospectively the results from a cohort of patients. Methods and Materials: Since 1993, a total of 85 patients with vascular lesions have been treated. Of those, 64 patients fulfilled the criteria of having an arteriovenous malformation and sufficient follow-up. The AVMs were grouped by

Frederik J. A. I.. Vernimmen; Jacobus P. Slabbert; Jennifer A. Wilson; Shaheeda Fredericks; Roger Melvill

2005-01-01

192

Ethanol Sclerotherapy of Superficial Venous Malformation: A New Procedure  

Microsoft Academic Search

Background: Superficial venous malformations (SVM) are the most frequent vascular malformations. Outpatient percutaneous treatment with ethanol injection has rarely been described. Objective: To analyze the results from treating SVM patients with ethanol sclerotherapy. Methods: 81 patients were followed up prospectively over a median period of 18 months. 47 were female and 34 were male with a median age of 21

José Luiz Orlando; Jose Guilherme Mendes Pereira Caldas; Heloisa Galvão do Amaral Campos; Kenji Nishinari; Nelson Wolosker

2010-01-01

193

Parallel transverse-sigmoid sinus harboring dural arteriovenous malformation. How to differentiate the pathological and normal sinus in order to treat and preserve patency and function.  

PubMed

An unusual case of dural arteriovenous malformation (DAVM) harboring a parallel transverse-sigmoid sinus (TSS) is presented. The patient had a 2-year history of left-sided pulsatile tinnitus in the left ear refractory to medical management. Angiography demonstrated a DAVM involving the left TSS. Super-selective transvenous dural sinus occlusion of the DAVM situated at the pathological compartment of the TSS provided cure. We were able to spare the normal compartment providing anatomical venous drainage from this system. PMID:19517058

de Paula Lucas, César; Prandini, Mirto Nelso; Spelle, Laurent; Piotin, Michel; Mounayer, Charbel; Moret, Jacques

2010-03-01

194

Mid-hindbrain malformations due to drugs taken during pregnancy.  

PubMed

Although genetic defects are the leading cause of central nervous system malformations including in the posterior fossa, specific malformative patterns should alert the clinician to consider rather a teratogenic etiology. We discuss the imaging features of 2 mid-hindbrain malformations consecutive to the intake of isotretinoin (Roaccuatane®; case 1) and misoprostol (Cytotec®; case 2) during pregnancy and review the pertinent literature. We correlate the morphological appearance of the mid-hindbrain malformation, as seen on high-resolution magnetic resonance imaging to possible drug-induced pathogenetical mechanisms. The recognition of characteristic imaging patterns enables diagnosis of and/or confirmation of suspected drug-induced hindbrain malformations. This has important medicolegal implications and also clinical significance to avoid unsuccessful and misleading genetic testing. PMID:23390117

Merlini, Laura; Fluss, Joël; Dhouib, Amira; Vargas, Maria I; Becker, Minerva

2014-04-01

195

[Passive and active middle ear implants].  

PubMed

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:19353457

Beutner, D; Hüttenbrink, K B

2009-05-01

196

Ear Acupuncture in European Traditional Medicine  

PubMed Central

Auricular acupuncture is a diagnostic and treatment system based on normalizing the body's dysfunction through stimulation of definite points on the ear. Rudimentary forms of acupuncture which probably arose during the Stone Age have survived in many parts of the world right down to present day. It was used in the ancient Egypt, Rome, Greece and all the Mediterranean area. It is a microacupuncture technique similar to reflexology, and was first described in France in 1950 by Paul Nogier who is considered the Father of modern ear acupuncture. It was speculated that the technique works because groups of pluripotent cells contain information from the whole organism and create regional organization centers representing different parts of the body. Nevertheless stimulation of a reflex point in the ear seems relieve symptoms of distant pathologies. Modern research is confirming the efficacy of ear acupuncture for analgesia and anxiety related disease, while tobacco dependence and other substance abuse still need confirmation. Actually main methodological problems with auricular acupuncture are that exist too many maps with little agreement regarding point location in the ear, and that the correspondence or reflex systems does not correlated with modern knowledge of anatomy and physiology. PMID:18227925

Firenzuoli, Fabio

2007-01-01

197

Assessment of Ear Disorders Using Power Reflectance  

PubMed Central

This paper describes the effect of various pathologies on power reflectance (PR) and absorbance measured in human adults. The pathologies studied include those affecting the tympanic membrane, the middle-ear ossicles, the middle-ear cavity, the inner ear, and intracranial pressure. Interesting pathology-induced changes in PR that are statistically significant have been reported. Nevertheless, because measurements of PR obtained from normal-hearing subjects have large variations and some pathology-induced changes are small, it can be difficult to use PR alone for differential diagnosis. There are, however, common clinical situations without reliable diagnostic methods that can benefit from PR measurements. These conditions include ears with a normal-appearing tympanic membrane, aerated middle-ear cavity and unknown etiology of conductive hearing loss. PR measurements in conjunction with audiometric measurements of air-bone gap have promise in differentiating among stapes fixation, ossicular discontinuity and superior semicircular canal dehiscence. Another possible application is to monitor an individual for possible changes in intracranial pressure. Descriptions of mechanisms affecting PR change and utilization of PR measurements in clinical scenarios are presented. PMID:23900180

Nakajima, Hideko Heidi; Rosowski, John J.; Shahnaz, Navid; Voss, Susan E.

2013-01-01

198

Passive and active middle ear implants  

PubMed Central

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

Beutner, Dirk; Hüttenbrink, Karl-Bernd

2011-01-01

199

Associated malformations in patients with limb reduction deficiencies.  

PubMed

Infants with limb reduction deficiencies (LRD) often have other associated congenital malformations. The purpose of this investigation was to assess the prevalence and the types of associated malformations in a defined population. This study included special strengths: each affected child was examined by a geneticist, all elective terminations were ascertained, and the surveillance for malformations was continued until 1 year of age. The associated malformations in infants with LRD were collected in all livebirths, stillbirths and terminations of pregnancy during 25 years in 347,810 consecutive births in the area covered by our population based registry of congenital malformations. Of the 271 LRD infants born during this period, representing a prevalence of 7.8 per 10,000, 57.9% had associated malformations. There were 17(6.3%) patients with chromosomal abnormalities including 10 trisomies 18, and 62 (22.9%) nonchromosomal recognized dysmorphic conditions. There were no predominant recognized dysmorphic conditions, but VA(C)TER(L) association. However numerous recognized dysmorphic conditions were registered including Poland, ectrodactyly-ectodermal dysplasia-clefting, oral-facial-digital, Klippel-Trenaunay-Weber, oculo-auriculo-vertebral defect spectrum, CHARGE, Townes-Brocks, Moebius, Du Pan, Smith-Lemli-Opitz, hypoglossia-hypodactyly, amniotic band, De Lange, Rubinstein-Taybi, Fanconi, radius aplasia- thrombocytopenia, Roberts, Holt-Oram, and fetal diethylstilbestrol. Seventy eight (28.8%) of the patients were multiply, non-syndromic, non chromosomal malformed infants (MCA). Malformations in the cardiac system, in the genital system, and in the central nervous system were the most common other malformations, 11.4%, 9.4%, and 7.7% of the associated malformations, respectively, followed by malformations in the renal system (4.8%), and in the digestive system (4.6%). Prenatal diagnosis was performed in 48.4% of dysmorphic syndromes with LRD. The overall prevalence of associated malformations, which was more than one in two infants, emphasizes the need for a thorough investigation of infants with LRD.A routine screening for other malformations especially cardiovascular system, urogenital system, central nervous system, and digestive system may be considered in infants and in fetuses with LRD. PMID:20670696

Stoll, Claude; Alembik, Yves; Dott, Beatrice; Roth, Marie-Paule

2010-01-01

200

Endoscopic anatomy of the pediatric middle ear.  

PubMed

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. PMID:24154745

Isaacson, Glenn

2014-01-01

201

Ear lobule reconstruction using nasal septal cartilage.  

PubMed

Surgical reconstruction of an earlobe requires adequate support without sacrificing the delicacy necessary for an attractive result. A two-stage ear lobule reconstruction using a mastoid skin pocket and cartilage from the nasal septum was performed in six patients. The earlobe aesthetics were acceptable and allowed ear piercing. There were no major complications, including no loss of flap, graft extrusion, septal perforation, or infection. Range of follow-up was 1 to 6 years, with an average of 3 years. No revisions have been performed. A two-stage technique for ear lobule reconstruction is described using septal cartilage to preserve shape and definition that has the additional advantage of minimal morbidity. PMID:23542248

Bastidas, Nicholas; Jacobs, Jordan M S; Thorne, Charles H

2013-04-01

202

Multimodality imaging of pancreatic arteriovenous malformation.  

PubMed

Arteriovenous malformation of the pancreas (PAVM) is a very rare entity, although it may be increasingly diagnosed with the expanding use of cross-sectional imaging of the abdomen. PAVM is characterized by a network of tangled vasculature within and surrounding all or part of the pancreas, resulting in the shunting of the arteries of the pancreas directly into the portal venous system. Here, we present a patient with chronic abdominal pain and pancreatitis found to have PAVM, based on the findings of computed tomography, magnetic resonance imaging, endoscopic retrograde cholangiopancreatography, and angiography. Differential considerations are discussed. Although PAVM is uncommon, it should be considered in the differential of patients with recurrent abdominal pain or gastrointestinal bleeding. PMID:25262988

Hansen, Wendy; Maximin, Suresh; Shriki, Jabi E; Bhargava, Puneet

2015-01-01

203

Familial Dandy-Walker malformation and leukodystrophy.  

PubMed

We report the first familial cases with two different types of posterior fossa cystic malformation and a leukodystrophic-like aspect on cerebral magnetic resonance imaging (MRI). The girl and her brother had severe encephalopathy, marked hypotonia, absent deep tendon reflexes, macrocrania, gigantism, and dysmorphic face and extremities. The girl had generalized seizures. The boy had unilateral cataract and bilateral optic atrophy. The parents were first cousins, suggesting autosomal recessive transmission. MRI showed Dandy-Walker variant in the girl, with cerebellar vermis hypoplasia and expansion of the cisterna magna, which communicated with the fourth ventricle. Her brother had mega cisterna magna communicating with the fourth ventricle and a normal cerebellum. The 2 children had abnormally high signal in the supratentorial white matter. Visual and auditory evoked potentials revealed prolonged latencies. Motor and sensory conduction velocities were normal. Muscle and nerve biopsies were normal. Metabolic exploration demonstrated no abnormality. PMID:9258968

Humbertclaude, V T; Coubes, P A; Leboucq, N; Echenne, B B

1997-05-01

204

Intracranial arteriovenous malformation: contralateral steal phenomena.  

PubMed

Sixty-two patients with radiographically proven intracranial arteriovenous malformations underwent preoperative regional cerebral blood flow measurement with 133Xe single-photon emission computed tomography. Contralateral regions of hypoperfusion were detected in all cases. Steal severity was assessed according to the contralateral steal index [ISteal(c)]. ISteal(c) was greater than 0.7 (severe) in 22 (35%), 0.7-0.8 (intermediate) in 18 (29%), and greater than 0.8 (mild) in 22 (35%). ISteal(c) was more frequently severe or mild in females and more often intermediate in males (p less than 0.05). Hyperemic complications were encountered more frequently in patients with intermediate ISteal(c) (p = 0.086). An unfavorable outcome was associated with less severe contralateral steal (p = 0.12). A detailed clinical, radiographic, and hemodynamic profile may help to preoperatively identify patients at high risk for a poor surgical outcome. PMID:2477740

Batjer, H H; Devous, M D; Seibert, G B; Purdy, P D; Ajmani, A K; Delarosa, M; Bonte, F J

1989-05-01

205

Third ventricular cavernous malformation: an unusual lesion.  

PubMed

Cavernomas constitute 5-10% of all the vascular malformations of the CNS. They commonly present during the 2nd and 5th decades of life. Intraventricular cavernomas constitute rare pathological entity, constituting 2.5-10.8% of cerebral cavernomas. (1) The natural history of intraventricular cavernomas remains undefined to some extent. Those in third ventricle are different in biological nature and need more aggressive therapy. These cavernomas appear to have the ability to grow very rapidly, resulting in significant morbidity. It is not known whether waiting after acute hemorrhage from an intraventricular cavernoma improves our ability to remove the lesion safely or if waiting unnecessarily increases the risk of hydrocephalus, additional bleeding, or further lesion growth. PMID:23952135

Patibandla, Mohana Rao; Thotakura, Amit Kumar; Panigrahi, Manas Kumar

2014-01-01

206

Two major patterns of nongenetic malformations are found at autopsy.  

PubMed

Patterns of malformations seen in autopsies may contribute to the understanding of their pathogenetic mechanisms. Two entities, acardiac twins (ATs) and amniotic band disruption complex (ABDC), have distinct patterns, indicating different mechanisms, namely vascular perfusion deficit and external disruption. With ATs and ABDC as model groups, this study was undertaken to see if other dysmorphic infants with the characteristic defects of these models formed distinct and numerically important groups. A total of 192 autopsies with nongenetic malformations was divided into groups including (1) those with defects found in the ATs but not in the ABDC, (2) those with defects found only in the ABDC, and (3) those with a mixture of exclusive defects from each model group. The cases followed the characteristic defects of ATs or ABDC in 20% (group 1) and 28% (group 2), respectively, forming 2 large and distinct groups; only 4% had mixed malformations (group 3). Group 1 had different characteristics from group 2 as a result of the frequent multiple malformations, often with congenital heart defects (CHDs), internal and inferior malformations. These cases were probably related to a vascular perfusion deficit. Group 2 had a majority of females and single, external, and superior defects, but it lacked CHDs and inferior malformations. These cases were likely due to external disruption. Two large and distinct groups of autopsies with nongenetic malformations were thus identified, and their mechanisms are proposed to be similar to those of the model groups. PMID:21105785

Davies, Belinda R; Giménez-Scherer, Juan Antonio

2011-01-01

207

A developmental and genetic classification for midbrain-hindbrain malformations  

PubMed Central

Advances in neuroimaging, developmental biology and molecular genetics have increased the understanding of developmental disorders affecting the midbrain and hindbrain, both as isolated anomalies and as part of larger malformation syndromes. However, the understanding of these malformations and their relationships with other malformations, within the central nervous system and in the rest of the body, remains limited. A new classification system is proposed, based wherever possible, upon embryology and genetics. Proposed categories include: (i) malformations secondary to early anteroposterior and dorsoventral patterning defects, or to misspecification of mid-hindbrain germinal zones; (ii) malformations associated with later generalized developmental disorders that significantly affect the brainstem and cerebellum (and have a pathogenesis that is at least partly understood); (iii) localized brain malformations that significantly affect the brain stem and cerebellum (pathogenesis partly or largely understood, includes local proliferation, cell specification, migration and axonal guidance); and (iv) combined hypoplasia and atrophy of putative prenatal onset degenerative disorders. Pertinent embryology is discussed and the classification is justified. This classification will prove useful for both physicians who diagnose and treat patients with these disorders and for clinical scientists who wish to understand better the perturbations of developmental processes that produce them. Importantly, both the classification and its framework remain flexible enough to be easily modified when new embryologic processes are described or new malformations discovered. PMID:19933510

Millen, Kathleen J.; Dobyns, William B.

2009-01-01

208

Middle ear cholesteatoma in 11 dogs.  

PubMed

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

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

2011-06-01

209

Temporary umbilical loop colostomy for anorectal malformations.  

PubMed

Transumbilical surgical procedures have been reported to be a feasible, safe, and cosmetically excellent procedure for various pediatric surgical diseases. Umbilical loop colostomies have previously been created in patients with Hirschsprung's disease, but not in patients with anorectal malformations (ARMs). We assessed the feasibility and cosmetic results of temporal umbilical loop colostomy (TULC) in patients with ARMs. A circumferential skin incision was made at the base of the umbilical cord under general anesthesia. The skin, subcutaneous tissue, and fascia were cored out vertically, and the umbilical vessels and urachal remnant were individually ligated apart from the opening in the fascia. A loop colostomy was created in double-barreled fashion with a high chimney more than 2 cm above the level of the skin. The final size of the opening in the skin and fascia was modified according to the size of the bowel. The bowel wall was fixed separately to the peritoneum and fascia with interrupted 5-0 absorbable sutures. The bowel was opened longitudinally and everted without suturing to the skin. The loop was divided 7 days postoperatively, and diversion of the oral bowel was completed. The colostomy was closed 2-3 months after posterior saggital anorectoplasty through a peristomal skin incision followed by end-to-end anastomosis. Final wound closure was performed in a semi-opened fashion to create a deep umbilicus. TULCs were successfully created in seven infants with rectourethral bulbar fistula or rectovestibular fistula. Postoperative complications included mucosal prolapse in one case. No wound infection or spontaneous umbilical ring narrowing was observed. Skin problems were minimal, and stoma care could easily be performed by attaching stoma bag. Healing of umbilical wounds after TULC closure was excellent. The umbilicus may be an alternative stoma site for temporary loop colostomy in infants with intermediate-type anorectal malformations, who undergo radical anorectoplasty via a non-abdominal approach. PMID:23001137

Hamada, Yoshinori; Takada, Kohei; Nakamura, Yusuke; Sato, Masahito; Kwon, A-Hon

2012-11-01

210

Amplatzer vascular plugs in congenital cardiovascular malformations  

PubMed Central

Background: Amplatzer vascular plugs (AVPs) are devices ideally suited to close medium-to-large vascular communications. There is limited published literature regarding the utility of AVPs in congenital cardiovascular malformations (CCVMs). Aims: To describe the use of AVPs in different CCVMs and to evaluate their safety and efficacy. Materials and Methods: All patients who required an AVP for the closure of CCVM were included in this retrospective review of our catheterization laboratory data. The efficacy and safety of AVPs are reported. Results: A total of 39 AVPs were implanted in 31 patients. Thirteen (33%) were AVP type I and 23 (59%) were AVP type II. AVP type III were implanted in two patients and type IV in one patient. The major indications for their use included closure of pulmonary arteriovenous malformation (AVM) (n = 7), aortopulmonary collaterals (n = 7), closure of a patent Blalock-Taussig shunt (n = 5), systemic AVM (n = 5), coronary AVM (n = 4), patent ductus arteriosus (PDA) (n = 3), pulmonary artery aneurysms (n = 3), and venovenous collaterals (n = 2). Deployment of the AVP was done predominantly via the 5 – 7F Judkin's right coronary guide catheter. Overall 92% of the AVPs could be successfully deployed and resulted in occlusion of the target vessel in all cases, within 10 minutes. No procedure-related or access site complication occurred. Conclusions: AVPs are versatile, easy to use, and effective devices to occlude the vascular communications in a variety of settings. AVP II is especially useful in the closure of tubular structures with a high flow. PMID:24688229

Barwad, Parag; Ramakrishnan, Sivasubramanian; Kothari, Shyam S; Saxena, Anita; Gupta, Saurabh K; Juneja, Rajnish; Gulati, Gurpreet Singh; Jagia, Priya; Sharma, Sanjiv

2013-01-01

211

[Intestinal malrotation: genetics features and other congenital malformations in children].  

PubMed

The article is devoted to features of prenatal development of children with intestinal malrotation. Mass, height, head and chest circumferences of children after birth have been studied. In addition all associated malformations, a blood group (ABO system) and the rhesus-factor have been investigated. In comparison to the control group of newborn the disorders of prenatal development have included associated malformations, low anthropometric data after birth, decreasing of pA gene frequency. Notable, children with intestinal malrotation have had high frequency of malformations as in the abdominal and thoracic cavities as outside intestinal innervations: the small pelvis, the facial skeleton, the upper and lower extremity. PMID:22379897

Botvin'ev, O K; Eremeeva, A V; Razumovskaia, I N; Kondrikova, E V

2011-01-01

212

A case of lymphatic malformation/lymphangioma of the scrotum  

PubMed Central

Lymphatic malformation/lymphangioma of the scrotum is rare. It is caused by lymphatic abnormalities and the most common sites are the neck and axilla. The scrotum is one of the most uncommon sites. We report the case of a 12-year-old boy with pathologically confirmed cystic lymphangioma/lymphatic malformation in the scrotum. The diagnosis was suspected from ultrasonography and magnetic resonance imaging. The most common cause of a cystic mass in the scrotum is scrotal hydrocele, but cystic lymphangioma/lymphatic malformation should be considered as a differential diagnosis for multicystic scrotal mass. PMID:23986836

Akaike, Gensuke; Nozaki, Taiki; Makidono, Akari; Saida, Yukihisa; Hirabayashi, Takeshi; Suzuki, Koyu

2012-01-01

213

Prenatal diagnosis and management of fetal cardiovascular malformations.  

PubMed

Screening for fetal cardiovascular malformations is widely performed. Its accuracy is not yet satisfactory, but better training of ultrasonographers and extension from the four-chamber view to the study of the outflow tract are probably clues to an improvement. The main impact of prenatal diagnosis is still the termination of pregnancy for severe malformations and for those associated with chromosomal or extracardiac anomalies. There is now evidence that prenatal diagnosis improves perinatal morbidity or mortality for some malformations. New information about the molecular genetic basis of congenital heart disease will help in management and counselling. PMID:10813571

Todros, T

2000-04-01

214

Syndromes and malformations associated with congenital heart disease in a population-based study  

Microsoft Academic Search

Congenital cardiac malformations are frequently associated with non-cardiac malformations and chromosomal anomalies. Management is therefore influenced by interventional needs for all of the various anomalies. We have studied the association of congenital heart disease with extracardiac anomalies in the relatively closed population of Malta, where echocardiographic screening of all syndromic\\/multiply malformed infants is routinely carried out. Malformations were classified by

Victor Grech; Miriam Gatt

1999-01-01

215

Homozygous FGF3 mutations result in congenital deafness with inner ear agenesis, microtia, and microdontia.  

PubMed

Homozygous mutations in the fibroblast growth factor 3 (FGF3) gene have recently been discovered in an autosomal recessive form of syndromic deafness characterized by complete labyrinthine aplasia (Michel aplasia), microtia, and microdontia (OMIM 610706 - LAMM). In order to better characterize the phenotypic spectrum associated with FGF3 mutations, we sequenced the FGF3 gene in 10 unrelated families in which probands had congenital deafness associated with various inner ear anomalies, including Michel aplasia, with or without tooth or external ear anomalies. FGF3 sequence changes were not found in eight unrelated probands with isolated inner ear anomalies or with a cochlear malformation along with auricle and tooth anomalies. We identified two new homozygous FGF3 mutations, p.Leu6Pro (c.17T>C) and p. Ile85MetfsX15 (c.254delT), in four subjects from two unrelated families with LAMM. The p.Leu6Pro mutation occurred within the signal site of FGF3 and is predicted to impair its secretion. The c.254delT mutation results in truncation of FGF3. Both mutations completely co-segregated with the phenotype, and heterozygotes did not have any of the phenotypic findings of LAMM. Some affected children had large skin tags on the upper side of the auricles, which is a distinctive clinical component of the syndrome. Enlarged collateral emissary veins associated with stenosis of the jugular foramen were noted on computerized tomographies of most affected subjects with FGF3 mutations. However, similar venous anomalies were also detected in persons with non-syndromic Michel aplasia, suggesting that a direct causative role of impaired FGF3 signaling is unlikely. PMID:18435799

Tekin, M; Oztürkmen Akay, H; Fitoz, S; Birnbaum, S; Cengiz, F B; Sennaro?lu, L; Incesulu, A; Yüksel Konuk, E B; Hasanefendio?lu Bayrak, A; Sentürk, S; Cebeci, I; Utine, G E; Tunçbilek, E; Nance, W E; Duman, D

2008-06-01

216

CHD7 Deficiency in “Looper”, a New Mouse Model of CHARGE Syndrome, Results in Ossicle Malformation, Otosclerosis and Hearing Impairment  

PubMed Central

CHARGE syndrome is a rare human disorder caused by mutations in the gene encoding chromodomain helicase DNA binding protein 7 (CHD7). Characteristics of CHARGE are varied and include developmental ear and hearing anomalies. Here we report a novel mouse model of CHD7 dysfunction, termed Looper. The Looper strain harbours a nonsense mutation (c.5690C>A, p.S1897X) within the Chd7 gene. Looper mice exhibit many of the clinical features of the human syndrome, consistent with previously reported CHARGE models, including growth retardation, facial asymmetry, vestibular defects, eye anomalies, hyperactivity, ossicle malformation, hearing loss and vestibular dysfunction. Looper mice display an otosclerosis-like fusion of the stapes footplate to the cochlear oval window and blepharoconjunctivitis but not coloboma. Looper mice are hyperactive and have vestibular dysfunction but do not display motor impairment. PMID:24840056

Ogier, Jacqueline M.; Carpinelli, Marina R.; Arhatari, Benedicta D.; Symons, R. C. Andrew; Kile, Benjamin T.; Burt, Rachel A.

2014-01-01

217

Middle Ear Infection (Chronic Otitis Media) and Hearing Loss  

MedlinePLUS

Middle Ear Infection (Chronic Otitis Media) and Hearing Loss Middle Ear Infection (Chronic Otitis Media) and Hearing Loss Patient Health Information News media interested in covering the latest from AAO-HNS/F can find ...

218

Transmission matrix analysis of the chinchilla middle ear  

PubMed Central

Despite the common use of the chinchilla as an animal model in auditory research, a complete characterization of the chinchilla middle ear using transmission matrix analysis has not been performed. In this paper we describe measurements of middle-ear input admittance and stapes velocity in ears with the middle-ear cavity opened under three conditions: intact tympano-ossicular system and cochlea, after the cochlea has been drained, and after the stapes has been fixed. These measurements, made with stimulus frequencies of 100–8000 Hz, are used to define the transmission matrix parameters of the middle ear and to calculate the cochlear input impedance as well as the middle-ear output impedance. This transmission characterization of the chinchilla middle ear will be useful for modeling auditory sensitivity in the normal and pathological chinchilla ear. PMID:17672642

Songer, Jocelyn E.; Rosowski, John J.

2008-01-01

219

Shaping Magnetic Fields to Direct Therapy to Ears  

E-print Network

delivery, ear, eye, nanotherapy, stem cells Abstract Magnetic fields have the potential to noninvasively cells--in blood, through tissue, and across barriers--to disease locations. In this article, we consider . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 456 2. DRUG DELIVERY TO THE INNER EAR

Shapiro, Benjamin

220

Modeling Ear-Canal Acoustics, Incorporating Visco-Thermal Effects and the Influence of the Middle Ear  

NASA Astrophysics Data System (ADS)

The ear canal, below about 6 kHz, is well described by a uniform cylinder (sound propagates predominantly as plane waves) with the middle ear being a non-rigid termination. A non-rigid termination can be viewed as altering, as a function of frequency, the acoustic length and radius of the cylinder. It is generally assumed that sound transmission in the ear canal over this frequency range is lossless. This paper presents a method for calculating the influence of visco-thermal losses and the middle ear on ear canal acoustics. The acoustic input impedance was derived from sound pressure measurements in the ear canal and then a nonlinear least-square-fit to the data with a one-dimensional model incorporating visco-thermal losses generated length, radius, and middle ear impedance parameters. It was found that a rigid wall assumption for visco-thermal calculations was insufficient to account for damping in the ear canal. The properties of the ear canal wall (not being a rigid, low-friction surface), incorporated into visco-thermal losses as a scaling factor, provided a better fit to the data. Viscous and thermal losses were both found to affect sound propagation in the ear canal, viscous losses being more significant, altering the acoustic input impedance of the ear primarily in the region of the standing wave frequency. The model data suggests that the middle ear influences ear canal acoustics up to about 3 kHz.

Gowdy, Lauren E.; Withnell, Robert H.

2011-11-01

221

Ca2+ Signaling in the Inner Ear  

NSDL National Science Digital Library

The inner ear contains delicate sensory receptors that have adapted to detect the minutest mechanical disturbances. Ca2+ ions are implicated in all steps of the transduction process, as well as in its regulation by an impressive ensemble of finely tuned feedback control mechanisms. Recent studies have unveiled some of the key players, but things do not sound quite right yet.

2007-04-01

222

livious ear ie Michael J. Kearns  

E-print Network

livious ear ie Michael J. Kearns AT&T Bell Laboratories 600 Mountain Avenue, Room 2A-423 Murray different target concept f. Each run results in the ad- dition of a new hypothesis representation r to effectively support in- clusion tests between any pair of target concepts. More precisely, given any ri and r2

Kearns, Michael

223

Collagen types in the middle ear mucosa  

Microsoft Academic Search

The distribution of various collagen types — in particular that of type 11 as the major collagen in cartilage — in normal auricular structures is discussed with reference to a 1994 report by Ovesen describing the presence of collagen type II in normal middle ear mucosa. In contrast to this report, no collagen type 11 is normally found in the

A. G. Nerlich

1995-01-01

224

The fungal flora of zoo animals' ears.  

PubMed

The mycotic flora of the ears of zoo animals was investigated in a large zoological garden in Germany. Malassezia pachydermatis was isolated from the following animals: big ant-eater, brown bear, common wombat, Eurasian badger, Indian elephant, Mangaliza pig and wide-mouthed rhinoceros. Aspergillus and Penicillium species, yeasts and zygomycetes were also isolated from some animals. PMID:7935595

Kuttin, E S; Müller, J

1994-01-01

225

Getting Teens to Read with Their Ears  

ERIC Educational Resources Information Center

Audiobooks have been around for years in various formats, like cassette tapes and CDs. This article describes a new type of audiobook on the market which is generating an interest in "reading." The device, called Playaway, is the size of a MP3 player and comes with a lanyard and ear buds. Buttons on the back of the player control the speed and…

Fues, Marianne Cole

2009-01-01

226

Keep Your Ear-Lids Open.  

ERIC Educational Resources Information Center

This article suggests that the development of listening skills should extend to the "soundscape" of nonspeech acoustical information. It presents a model for effective aural processing, identifies categories of information obtained from nonverbal sound, and explores "ear-tuning" or listening exercises that use sound to glean information about…

Ferrington, Gary

1994-01-01

227

Ear biometric recognition using local texture descriptors  

NASA Astrophysics Data System (ADS)

Automated personal identification using the shape of the human ear is emerging as an appealing modality in biometric and forensic domains. This is mainly due to the fact that the ear pattern can provide rich and stable information to differentiate and recognize people. In the literature, there are many approaches and descriptors that achieve relatively good results in constrained environments. The recognition performance tends, however, to significantly decrease under illumination variation, pose variation, and partial occlusion. In this work, we investigate the use of local texture descriptors, namely local binary patterns, local phase quantization, and binarized statistical image features for robust human identification from two-dimensional ear imaging. In contrast to global image descriptors which compute features directly from the entire image, local descriptors representing the features in small local image patches have proven to be more effective in real-world conditions. Our extensive experimental results on the benchmarks IIT Delhi-1, IIT Delhi-2, and USTB ear databases show that local texture features in general and BSIF in particular provide a significant performance improvement compared to the state-of-the-art.

Benzaoui, Amir; Hadid, Abdenour; Boukrouche, Abdelhani

2014-09-01

228

Sources of Variability in Reflectance Measurements on Normal Cadaver Ears  

Microsoft Academic Search

Objectives: The development of acoustic reflectance measurements may lead to noninvasive tests that provide information currently unavailable from standard audiometric testing. One factor limiting the development of these tests is that normal-hear- ing human ears show substantial intersubject vari- ations. This work examines intersubject variability that results from measurement location within the ear canal, estimates of ear-canal area, and varia-

Susan E. Voss; Nicholas J. Horton; Rebecca R. Woodbury; Kathryn N. Sheffield

2008-01-01

229

Ear and kidney syndromes: Molecular versus clinical approach  

Microsoft Academic Search

Ear and kidney syndromes: Molecular versus clinical approach.The association between ear and kidney anomalies is not usually due to an insult to the embryo. In recent years, many essential development control genes that coordinate the assembly and function of kidney and ear have been discovered through the generation of animal mutants and have increased our understanding of the mechanisms of

HASSANE IZZEDINE; FREDERIC TANKERE; VINCENT LAUNAY-VACHER; GILBERT DERAY

2004-01-01

230

A Survey on Ear Biometrics AYMAN ABAZA, WVHTC Foundation  

E-print Network

22 A Survey on Ear Biometrics AYMAN ABAZA, WVHTC Foundation ARUN ROSS, West Virginia University Recognizing people by their ear has recently received significant attention in the literature. Several reasons account for this trend: first, ear recognition does not suffer from some problems associated with other

Ross, Arun Abraham

231

Understanding Inner Ear Development with Gene Expression Profiling  

E-print Network

Understanding Inner Ear Development with Gene Expression Profiling Zheng-Yi Chen,1,2 David P. Corey, Maryland 20815 ABSTRACT: Understanding the development of the inner ear requires knowing the spatial-tissue comparisons will identify genes unique to the inner ear, which will expe- dite the identification of new

Corey, David P.

232

History of Studies on Mammalian Middle Ear Evolution: A  

E-print Network

History of Studies on Mammalian Middle Ear Evolution: A Comparative Morphological and Developmental for Developmental Biology, RIKEN, Kobe, Japan The mammalian middle ear represents one of the most fundamental middle ear was derived from elements of the jaw joint of nonmammalian amniotes. Fossils of mammalian

Sullivan, Jack

233

Ear growth, developmental stages and yield in winter wheat  

E-print Network

Ear growth, developmental stages and yield in winter wheat Jean-François LEDENT Volkmar STOY Helena in length (R,) of ear primordia was measured in collections of winter wheats (Triticum aestivum (L.) em or morphological characters. However, genotypes with faster growing ear primordia tended to have smaller cars

Paris-Sud XI, Université de

234

Short Papers___________________________________________________________________________________________________ Comparison and Combination of Ear and Face  

E-print Network

that the ear may have advantages over the face for biometric recognition. Our previous experiments with ear and face recognition, using the standard principal component analysis approach, showed lower recognition rigorously controlled for relative quality of face and ear images. We find that recognition performance

Bowyer, Kevin W.

235

21 CFR 874.3430 - Middle ear mold.  

Code of Federal Regulations, 2012 CFR

...2012-04-01 2012-04-01 false Middle ear mold. 874.3430 Section 874.3430 Food and...Prosthetic Devices § 874.3430 Middle ear mold. (a) Identification. A middle ear mold is a preformed device that is...

2012-04-01

236

21 CFR 874.3430 - Middle ear mold.  

...2014-04-01 2014-04-01 false Middle ear mold. 874.3430 Section 874.3430 Food and...Prosthetic Devices § 874.3430 Middle ear mold. (a) Identification. A middle ear mold is a preformed device that is...

2014-04-01

237

21 CFR 874.3430 - Middle ear mold.  

Code of Federal Regulations, 2013 CFR

...2013-04-01 2013-04-01 false Middle ear mold. 874.3430 Section 874.3430 Food and...Prosthetic Devices § 874.3430 Middle ear mold. (a) Identification. A middle ear mold is a preformed device that is...

2013-04-01

238

21 CFR 874.3430 - Middle ear mold.  

Code of Federal Regulations, 2011 CFR

...2011-04-01 2011-04-01 false Middle ear mold. 874.3430 Section 874.3430 Food and...Prosthetic Devices § 874.3430 Middle ear mold. (a) Identification. A middle ear mold is a preformed device that is...

2011-04-01

239

21 CFR 874.3430 - Middle ear mold.  

Code of Federal Regulations, 2010 CFR

...2010-04-01 2010-04-01 false Middle ear mold. 874.3430 Section 874.3430 Food and...Prosthetic Devices § 874.3430 Middle ear mold. (a) Identification. A middle ear mold is a preformed device that is...

2010-04-01

240

A pilot randomized controlled trial comparing bismuth iodine paraffin paste external ear pack and no ear pack after middle ear surgery.  

PubMed

To pilot a substantive randomized control trial comparing post-operative external ear canal pack with no ear pack after middle ear surgery, 32 adults undergoing primary posterior bony canal wall preserving middle ear surgery were randomized to have either a bismuth iodoform paraffin paste pack or no ear pack post-operatively. Outcome measures included clinician- and patient-recorded visual analogue scale scores for ear signs and symptoms at 3 weeks and 3 months and audiometric findings at 3 months post-operatively. There was no statistically significant inter-group difference in 3-week clinician and patient cumulative scores for ear signs and symptoms. There was also no significant difference in graft take rate, appearance of ear canals and audiometric results in either group at 3 months. No difference in ear symptoms, clinician findings or hearing was demonstrated between patients with a post-operative pack compared to those without a pack following middle ear surgery in this pilot study. PMID:24337896

Javed, Faisal; Whitwell, Russell; Hajioff, Daniel; Robinson, Philip; Rea, David; Macleod, Iain; White, Paul; Nunez, Desmond A

2013-12-14

241

Malformation syndromes associated with disorders of sex development.  

PubMed

When embryological development of the internal and/or external genitalia is disrupted, the patient presents with a disorder of sex development (DSD) in the neonatal period or sometime later in life. Some of these patients have other, nongenital malformations, which makes their overall management more complex than if they just had a DSD. This Review summarises these malformation syndromes and discusses the recent research into their aetiology. The genetic causes of these malformation syndromes, when they are known, will also be described. Many specific genetic mutations are now known in malformation syndromes with a defect in hormonal function. By contrast, the genetic causes remain unknown in many nonhormonal morphological anomalies that affect the genitalia. PMID:24913517

Hutson, John M; Grover, Sonia R; O'Connell, Michele; Pennell, Samuel D

2014-08-01

242

The Chiari malformations: A review with emphasis on anatomical traits.  

PubMed

Hindbrain herniations come in many forms and have been further subdivided as their original descriptions. For cerebellar tonsillar ectopia, they can be divided into two categories, acquired and congenital. Acquired hindbrain herniations are due to increased intracranial pressure caused by certain conditions such as trauma or brain tumor. Although the mechanism for their formation is not clear, congenital hindbrain herniation makes up the majority of these congenital malformations. Furthermore, these malformations are often found to harbor additional anatomical derailments in addition to the hindbrain herniation whether it be the cerebellar tonsils (Chiari I malformation) or the cerebellar vermis (Chiari II malformation). This article reviews these forms of cerebellar ectopia and describes the details of their anomalous anatomy. Moreover, this article compares and contrasts the differing embryological theories found in this literature. Clin. Anat. 28:184-194, 2015. © 2014 Wiley Periodicals, Inc. PMID:25065525

Cesmebasi, Alper; Loukas, Marios; Hogan, Elizabeth; Kralovic, Sara; Tubbs, R Shane; Cohen-Gadol, Aaron A

2015-03-01

243

Variants in CUL4B are Associated with Cerebral Malformations.  

PubMed

Variants in cullin 4B (CUL4B) are a known cause of syndromic X-linked intellectual disability. Here, we describe an additional 25 patients from 11 families with variants in CUL4B. We identified nine different novel variants in these families and confirmed the pathogenicity of all nontruncating variants. Neuroimaging data, available for 15 patients, showed the presence of cerebral malformations in ten patients. The cerebral anomalies comprised malformations of cortical development (MCD), ventriculomegaly, and diminished white matter volume. The phenotypic heterogeneity of the cerebral malformations might result from the involvement of CUL-4B in various cellular pathways essential for normal brain development. Accordingly, we show that CUL-4B interacts with WDR62, a protein in which variants were previously identified in patients with microcephaly and a wide range of MCD. This interaction might contribute to the development of cerebral malformations in patients with variants in CUL4B. PMID:25385192

Vulto-van Silfhout, Anneke T; Nakagawa, Tadashi; Bahi-Buisson, Nadia; Haas, Stefan A; Hu, Hao; Bienek, Melanie; Vissers, Lisenka E L M; Gilissen, Christian; Tzschach, Andreas; Busche, Andreas; Müsebeck, Jörg; Rump, Patrick; Mathijssen, Inge B; Avela, Kristiina; Somer, Mirja; Doagu, Fatma; Philips, Anju K; Rauch, Anita; Baumer, Alessandra; Voesenek, Krysta; Poirier, Karine; Vigneron, Jacqueline; Amram, Daniel; Odent, Sylvie; Nawara, Magdalena; Obersztyn, Ewa; Lenart, Jacek; Charzewska, Agnieszka; Lebrun, Nicolas; Fischer, Ute; Nillesen, Willy M; Yntema, Helger G; Järvelä, Irma; Ropers, Hans-Hilger; de Vries, Bert B A; Brunner, Han G; van Bokhoven, Hans; Raymond, F Lucy; Willemsen, Michèl A A P; Chelly, Jamel; Xiong, Yue; Barkovich, A James; Kalscheuer, Vera M; Kleefstra, Tjitske; de Brouwer, Arjan P M

2015-01-01

244

Management strategy after diagnosis of Abernethy malformation: a case report  

PubMed Central

Introduction The Abernethy malformation is a rare anomaly with a widely variable clinical presentation. Many diagnostic dilemmas have been reported. Nowadays, with the evolution of medical imaging, diagnosis can be made more easily, but management of patients with an Abernethy malformation is still open for discussion. Case presentation In this case study, we describe a 34-year-old Caucasian man who presented with a large hepatocellular carcinoma in the presence of an Abernethy malformation, which was complicated by the development of pulmonary arterial hypertension. Conclusion This case underlines the importance of regular examination of patients with an Abernethy malformation, even in older patients, to prevent complications and to detect liver lesions at an early stage. PMID:22742057

2012-01-01

245

Genetics Home Reference: Microcephaly-capillary malformation syndrome  

MedlinePLUS

... proper balance of protein production and breakdown (protein homeostasis) that cells need to function and survive. Studies ... cell ; degrade ; developmental delay ; egg ; endocytosis ; epilepsy ; gene ; homeostasis ; inheritance ; inherited ; malformation ; microcephaly ; myoclonus ; pattern of inheritance ; ...

246

Prenatal Diagnosis of Unusual Fetal Pial Arteriovenous Malformation  

PubMed Central

Summary Cerebral arteriovenous malformations (C-AVMs) are rarely diagnosed in utero. Most prenatal imaging of intracranial vascular malformations relates to Vein of Galen aneurysmal malformations (VGAMs) or Dural Arteriovenous Malformations (D-AVMs). We report a case of a fetal pial AVF with multiple fistulae and venous pouches, which appeared as an anechoic lesion on the prenatal ultrasound scan. The patient was asymptomatic with normal postnatal growth. No haemodynmaic disturbance was evident. Postnatal Computed tomography (CT), Magnetic Resonance Imaging (MRI) and catheter Digital Subtraction Angiography (DSA) confirmed the presence of a pial AVF. The angiographic findings and family history of nose bleeds suggests the diagnosis of Hereditary Hemorrhagic Telangiectasia. The largest AVF was embolized with tissue adhesive; the residual AVF subsequently removed by surgical excision. PMID:20591266

Auyeung, K.M.; Laughlin, S.; TerBrugge, K.G.

2003-01-01

247

Multiple medullary venous malformations decreasing cerebral blood flow: Case report  

SciTech Connect

A rare case of multiple medullary venous malformations in the right cerebral hemisphere is reported. The literature review yielded only one case of multiple medullary venous malformations. Computed tomography scan showed multiple calcified lesions with linear contrast enhancement representing abnormal dilated vessels and mild atrophic change of the right cerebral hemisphere. Single-photon emission computed tomography using N-isopropyl-p-({sup 123}I) iodoamphetamine demonstrated decreased cerebral blood flow in the right cerebral hemisphere.

Tomura, N.; Inugami, A.; Uemura, K.; Hadeishi, H.; Yasui, N. (Research Institute for Brain and Blood Vessels, Akita (Japan))

1991-02-01

248

Novel transcatheter closure of internal iliac arteriovenous malformation.  

PubMed

Arteriovenous malformations arising from iliac arteries are rare anomalies. Percutaneous embolization of such malformations is an established therapeutic option. In this paper, we will describe a case, wherein the internal iliac artery feeding a giant arteriovenous fistula was closed using a patent ductus arteriosus (PDA) duct occluder percutaneously. The PDA duct occluder is a versatile device that can be used as an alternative to vascular plugs. PMID:24122535

Shankarappa, Ravindranath Khandenahally; Panneerselvam, Arunkumar; Dwarakaprasad, Ramesh; Chikkaswamy, Srinivas Budanur; Nayak, Mohan Honnayya; Nanjappa, Manjunath Cholenahally

2011-05-01

249

Contemporary Themes: Congenital Malformations and the Problem of their Control*  

PubMed Central

The possibility of preventing or reducing the prevalence of human congenital malformations is discussed in the light of current knowledge about their aetiology. The original data presented are derived from an epidemiological study of all the infants (92,982) born in the three years 1964-6 to women resident in a defined area of South Wales and all the congenital malformations (3,242) discovered in those infants by the second anniversary of their birth. PMID:4262651

Lowe, C. R.

1972-01-01

250

Intracranial hypertension associated with unruptured cerebral arteriovenous malformations.  

PubMed

Only 6 patients with intracranial hypertension associated with unruptured cerebral arteriovenous malformations have been reported. We report 6 additional patients seen at the Cleveland Clinic during the past 10 years. The average age was 28 years (range, 19-44 years); 4 were women. Symptoms and signs included papilledema (6 patients), headache (6), transient nonepileptic focal symptoms (4), visual obscurations (3), ipsilateral carotid or ocular bruits (3), abnormal visual fields (3), focal seizures (2), and progressive visual loss (1). Enhanced computed tomography (CT) or magnetic resonance imaging (MRI) demonstrated the malformations in all 6 patients. The malformations were large, supplied by the branches of the middle and anterior cerebral arteries, with the posterior cerebral artery contributing in 3 patients, and all drained into the superior sagittal sinus. Associated venous obstruction was seen in 2 patients. Four patients underwent excision of the arteriovenous malformation, with resolution of papilledema in all 4. Measurements of cortical arterial and venous pressures during surgery in 3 patients showed decreased feeding artery pressures and elevated draining vein pressures, which normalized after removal of the malformation. Treatment in the 2 remaining patients consisted of medical therapy (acetazolamide, furosemide, steroids) alone in 1 patient, and in conjunction with proton beam radiation in the other. Papilledema resolved in the former patient, but the patient receiving proton beam radiation still had papilledema 2 years later. Intracranial hypertension associated with unruptured cerebral arteriovenous malformations occurs in young patients with high flow malformations that drain into the superior sagittal sinus, and is likely the result of increased cortical venous and superior sagittal sinus pressure. Excision of the malformation effectively reduces the intracranial pressure. PMID:2193610

Chimowitz, M I; Little, J R; Awad, I A; Sila, C A; Kosmorsky, G; Furlan, A J

1990-05-01

251

Fraser syndrome and cryptophthalmos: review of the diagnostic criteria and evidence for phenotypic modules in complex malformation syndromes  

PubMed Central

Fraser syndrome is characterised by cryptophthalmos, cutaneous syndactyly, malformations of the larynx and genitourinary tract, craniofacial dysmorphism, orofacial clefting, mental retardation, and musculoskeletal anomalies. The inheritance is autosomal recessive. No diagnostic cytogenetic abnormalities have been documented in affected patients, and no molecular genetic studies have been reported. We have reviewed 117 cases diagnosed as Fraser syndrome or cryptophthalmos published since the comprehensive review of Thomas et al in 1986 in order to validate the published diagnostic criteria and to delineate the phenotype associated with this syndrome. Our series showed more females (57/117) than males and consanguinity was present in 29/119 (24.8%). Eighty-eight patients satisfied the diagnostic criteria for Fraser syndrome (75%). Cryptophthalmos was present in 103/117 (88%), syndactyly in 72/117 (61.5%), and ambiguous genitalia in 20/117 (17.1%). Ear malformations were recorded in 69/117 (59%), and renal agenesis in 53/117 (45.3%). Use of the published diagnostic criteria excluded several patients with cryptophthalmos and one or more physical feature(s) consistent with Fraser syndrome. The frequency of additional anomalies in our series was also higher than previously reported (for example, imperforate anus or anal stenosis were found in 34/117 (29%) compared with 2/124 (2%) in the series of Thomas et al (1986) and choanal stenosis or atresia was present in 7/117 (6%) compared to 0/124. These findings emphasise the clinical variability associated with Fraser syndrome and support genetic heterogeneity of the syndrome. We also noted patterns of anomalies (for example, bicornuate uterus with imperforate anus or anal stenosis and renal malformations) that are found in other syndromes and associations without cryptophthalmos, suggesting that common modifier genes may explain some of the phenotypic variation in Fraser syndrome. PMID:12205104

Slavotinek, A; Tifft, C

2002-01-01

252

Sleep Spindle Alterations in Patients with Malformations of Cortical Development  

PubMed Central

Malformations of cortical development are disorders of altered brain anatomy and architecture that arise from abnormalities in the usual processes of cerebral cortical development. Although they often lead to epilepsy, cognitive delay, and motor impairment, little is known about their effect on sleep. Since malformations may anatomically or functionally disrupt the cerebral circuits that mediate sleep spindles, we hypothesized that these disorders would be associated with abnormal spindle characteristics. We analyzed the density, maximum frequency, laterality and distribution of sleep spindles seen in routine and long-term electroencephalographic recordings performed in ten brain malformation subjects and ten matched controls. There were no significant differences in spindle density or maximum frequency between the two groups, but malformation subjects had a significantly lower proportion of bilateral spindles and a significantly higher proportion of anterior and diffuse spindles compared to controls. In addition, unilateral malformations appeared to be associated with a skewing of unilateral spindles toward the contralateral side. Our findings suggest that brain malformations disrupt the thalamocortical circuits responsible for sleep spindle generation, and support the need for further studies on the relationships between cortical maldevelopment and sleep. PMID:18667284

Selvitelli, Megan F.; Krishnamurthy, Kaarkuzhali B.; Herzog, Andrew G.; Schomer, Donald L.; Chang, Bernard S.

2009-01-01

253

Limb malformations and abnormal sex hormone concentrations in frogs.  

PubMed Central

Declines in amphibian populations, and amphibians with gross malformations, have prompted concern regarding the biological status of many anuran species. A survey of bullfrogs, Rana catesbeiana, and green frogs, Rana clamitans, conducted in central and southern New Hampshire showed malformed frogs at 81% of the sites sampled (13 of 16 sites). Brain gonadotropin-releasing hormone (GnRH) and the synthesis of androgens and estradiol, hormones essential to reproductive processes, were measured from limb-malformed and normal (no limb malformation) frogs. Normal frogs had significantly higher concentrations (nearly 3-fold) of in vitro produced androgens and of brain GnRH than malformed frogs. Because most malformations are thought to occur during development, we propose that environmental factors or endocrine-disrupting chemicals that may cause developmental abnormalities also act during early development to ultimately cause abnormally reduced GnRH and androgen production in adult frogs. The consequences of reduced GnRH and androgens on anuran reproductive behavior and population dynamics are unknown but certainly may be profound and warrant further research. PMID:11102301

Sower, S A; Reed, K L; Babbitt, K J

2000-01-01

254

Giant cystic cerebral cavernous malformation with multiple calcification - case report.  

PubMed

Cerebral cavernous malformation with giant cysts is rare and literature descriptions of its clinical features are few. In this case study, the authors describe the clinical symptoms, radiological findings, and pathological diagnosis of cerebral cavernous malformations with giant cysts, reviewing the relevant literature to clearly differentiate this from other disease entities. The authors present a case of a 19-year-old male with a giant cystic cavernous malformation, who was referred to the division of neurosurgery due to right sided motor weakness (grade II/II). Imaging revealed a large homogenous cystic mass, 7.2×4.6×6 cm in size, in the left fronto-parietal lobe and basal ganglia. The mass had an intra-cystic lesion, abutting the basal portion of the mass. The initial diagnosis considered this mass a glioma or infection. A left frontal craniotomy was performed, followed by a transcortical approach to resect the mass. Total removal was accomplished without post-operative complications. An open biopsy and a histopathological exam diagnosed the mass as a giant cystic cavernous malformation. Imaging appearances of giant cavernous malformations may vary. The clinical features, radiological features, and management of giant cavernous malformations are described based on pertinent literature review. PMID:24167810

Kim, Il-Chun; Kwon, Ki-Young; Rhee, Jong-Joo; Lee, Jong-Won; Hur, Jin-Woo; Lee, Hyun-Koo

2013-09-01

255

Cerebral arteriovenous malformation associated with moyamoya disease.  

PubMed

The coexistence of moyamoya disease (MMD) with an arteriovenous malformation (AVM) is exceedingly rare. We report two cases of AVM associated with MMD. The first case was an incidental AVM diagnosed simultaneously with MMD. This AVM was managed expectantly after encephalo-duro-arterio-synangiosis (EDAS) as the main feeders stemmed from the internal carotid artery, which we believed would be obliterated with the progression of MMD. However, the AVM persisted with replacement of the internal carotid artery feeders by new external carotid artery feeders from the EDAS site. The AVM was eventually treated with gamma knife radiosurgery considering an increasing steal effect. The second case was a de novo AVM case. The patient was initially diagnosed with MMD, and acquired an AVM eight years later that was slowly fed by the reconstituted anterior cerebral artery. Because the patient remained asymptomatic, the AVM is currently being closely followed for more than 2 years without further surgical intervention. Possible differences in the pathogenesis and the radiologic presentation of these AVMs are discussed with a literature review. No solid consensus exists on the optimal treatment of MMD-associated AVMs. Gamma knife radiosurgery appears to be an effective treatment option for an incidental AVM. However, a de novo AVM may be managed expectantly considering the possible risks of damaging established collaterals, low flow characteristics, and probably low risks of rupture. PMID:25371789

Noh, Jung-Hoon; Yeon, Je Young; Park, Jae-Han; Shin, Hyung Jin

2014-10-01

256

Intraoperative embolization of cerebral arteriovenous malformations.  

PubMed

Nineteen patients with large cortical or deep seated cerebral arteriovenous malformation (AVM) were treated with intraoperative embolization using bucrylate (isobutyl-2-cyanoacrylate, IBC). In three cases of medium-sized AVM, the procedure was followed by a total excision of the lesion during the same operation. The results were excellent. In one case a hematoma and brain swelling following embolization necessitated total removal immediately afterwards. In eight patients embolization was carried out in two or three stages, to achieve a gradual obliteration of the shunt and to diminish the risk of postoperative brain swelling. Nevertheless, in three of these patients, postoperative hemiplegia was seen, which, however, improved considerably over a 1-2 year period. Complete occlusion of the shunt was obtained in only two patients. Seven patients underwent single-stage embolization, which resulted in partial obliteration of the AVM in six of them. In two of these, this treatment had been proposed for the accompanying symptoms of seizures and ischemic attacks. In all but one case the epileptic seizures decreased or disappeared completely after embolization. One patient suffered intracerebral bleeding three months after partial obliteration. Mortality was zero. It is argued that an alteration of the cerebral circulation with subsequent hyperemia is the most important sequela of rapid obliteration of large AVMs. Some details concerning the structure of cerebral AVMs are discussed. PMID:3736906

van Alphen, H A

1986-01-01

257

[Laparoscopic surgical treatment of anorectal malformations].  

PubMed

Anorectal Malformations (ARM) include a wide spectrum of anomalies, ranging from anal and rectal agenesis (usually associated with a recto-urethral, rectovaginal or recto-vestibular fistula) to ano-cutaneous fistula. On the basis of studies on the anatomy of the muscular structures contained in the pelvic cavity and on the physiology of the mechanisms which regulate the continence, many different techniques have been performed to allow a surgical treatment for the ARMs and to obtain post-operative results as nearer as possible to normality, that is to an adequate colic motility and a defecation control. In 2000, a new surgical technique for high and intermediate ARMs has been introduced, the laparoscopically assisted ano-rectal pull-through (LAARP) according to Georgeson. At the Department of Pediatric Surgery, Bologna University, from 2000 to nowadays, we have treated with LAARP 7 cases of high ARMs, all male patients and with colostomy. The average age at operation was between 2 and 10 months. PMID:17533901

Lima, M; Antonellini, C; Ruggeri, G; Libri, M; Gargano, T; Mondardini, M C

2006-01-01

258

Urotensin-II Induces Ear Flushing in Rats  

PubMed Central

Background and purpose: While investigating the effects of systemic urotensin II (U-II), a potent vasoactive peptide acting at the UT receptor, we observed ear pinna flushing after systemic administration to conscious rats. In the present study, U-II-induced ear flushing was quantified in terms of ear pinna temperature change and potential mechanisms were explored. Experimental approach: U-II-induced ear flushing was quantified by measuring lateral ear pinna temperature changes and compared to that of calcitonin gene-related peptide (CGRP), a known cutaneous vasodilator. Further, the effects of a variety of pharmacological agents on U-II-induced ear flushing were explored. Key results: Subcutaneous injection of U-II (9 ?g kg?1)produced localized ear pinna flushing with an onset of ?15?min, a duration of ?30?min and a maximal temperature change of 9°C. In contrast, CGRP caused cutaneous flushing within multiple cutaneous beds including the ear pinna with a shorter onset and greater duration than U-II. A potent UT receptor antagonist, urantide, blocked U-II-induced ear flushing but did not affect CGRP-induced ear flushing. Pretreatment with indomethacin or L-N ? -nitroarginine methylester (L-NAME) abolished U-II-induced ear flushing. Mecamylamine or propranolol did not affect this response to U-II. Direct intracerebroventricular injection studies suggested that the ear flushing response to U-II was not mediated directly by the CNS. Conclusion and implications: Our results suggest that U-II-induced ear flushing and temperature increase is mediated by peripheral activation of the UT receptor and involves prostaglandin- and nitric oxide-mediated vasodilation of small capillary beds in the rat ear pinna. PMID:17211454

Qi, J-S; Schulingkamp, R; Parry, T J; Colburn, R; Stone, D; Haertlein, B; Minor, L K; Andrade-Gordon, P; Damiano, B P

2007-01-01

259

Mouse Middle Ear Ion Homeostasis Channels and Intercellular Junctions  

PubMed Central

Hypothesis The middle ear contains homeostatic mechanisms that control the movement of ions and fluids similar to those present in the inner ear, and are altered during inflammation. Background The normal middle ear cavity is fluid-free and air-filled to allow for effective sound transmission. Within the inner ear, the regulation of fluid and ion movement is essential for normal auditory and vestibular function. The same ion and fluid channels active in the inner ear may have similar roles with fluid regulation in the middle ear. Methods Middle and inner ears from BALB/c mice were processed for immunohistochemistry of 10 specific ion homeostasis factors to determine if similar transport and barrier mechanisms are present in the tympanic cavity. Examination also was made of BALB/c mice middle ears after transtympanic injection with heat-killed Haemophilus influenza to determine if these channels are impacted by inflammation. Results The most prominent ion channels in the middle ear included aquaporins 1, 4 and 5, claudin 3, ENaC and Na+,K+-ATPase. Moderate staining was found for GJB2, KCNJ10 and KCNQ1. The inflamed middle ear epithelium showed increased staining due to expected cellular hypertrophy. Localization of ion channels was preserved within the inflamed middle ear epithelium. Conclusions The middle ear epithelium is a dynamic environment with intrinsic mechanisms for the control of ion and water transport to keep the middle ear clear of fluids. Compromise of these processes during middle ear disease may underlie the accumulation of effusions and suggests they may be a therapeutic target for effusion control. PMID:22720014

Morris, Lisa M.; DeGagne, Jacqueline M.; Kempton, J. Beth; Hausman, Frances; Trune, Dennis R.

2012-01-01

260

Diseases of the middle ear in childhood  

PubMed Central

Middle ear diseases in childhood play an important role in daily ENT practice due to their high incidence. Some of these like acute otitis media or otitis media with effusion have been studied extensively within the last decades. In this article, we present a selection of important childhood middle ear diseases and discuss the actual literature concerning their treatment, management of complications and outcome. Another main topic of this paper deals with the possibilities of surgical hearing rehabilitation in childhood. The bone-anchored hearing aid BAHA® and the active partially implantable device Vibrant Soundbridge® could successfully be applied for children. In this manuscript, we discuss the actual literature concerning clinical outcomes of these implantable hearing aids. PMID:25587371

Minovi, Amir; Dazert, Stefan

2014-01-01

261

The inner ear and the neurologist  

PubMed Central

Inner ear disorders are common and patients with vestibular failure often present to a neurology clinic because of their dizziness, gait unsteadiness and oscillopsia. Vestibular disorders can be divided into peripheral and central vestibular disorders. Most of the peripheral vestibular disorders have a clinical diagnosis, and a thorough history and examination will often provide a clear direction as to the diagnosis. Correct diagnosis allows treatment for many of the peripheral and central vestibular disorders. As inner ear damage is generally irreversible, early diagnosis allowing prompt treatment is important. The aim of this review is to discuss some audiovestibular conditions that may well appear in a neurology clinic, and to discuss some recent advances within the audiovestibular field that may be of interest to neurologists. Some of the most common audiovestibular conditions will be discussed along side more uncommon conditions. PMID:17229743

Agrup, Charlotte; Gleeson, Michael; Rudge, Peter

2007-01-01

262

A Rare Case of Petrified Ear  

PubMed Central

Calcification or ossification of the auricle, also referred to as petrified ear, is a rare diagnosis in dermatology. In medical literature, it has most often been attributed to trauma, hypothermia and frostbite, or hypercalcemia secondary to a metabolic or endocrine disorder, such as Addison's disease. Here, we report the clinical and radiologic findings of a 79-year-old African American male whose unilateral petrified auricle was an incidental finding. He had a preceding history of hyperparathyroidism and subsequent hypercalcemia treated with a subtotal parathyroidectomy three years prior to presentation. In addition to laboratory analysis, a history and physical examination was performed which revealed no other signs of hypercalcemia. Radiologic studies demonstrated partial ossification of the external auricular cartilage on the left side. The patient was diagnosed with the rare occurrence of a petrified ear. In light of this case, we provide a discussion concerning the possible etiologies of this diagnosis including appropriate patient evaluation and possible treatment recommendations. PMID:23259082

Buikema, Kathryn E.; Adams, Erin G.

2012-01-01

263

Scaling of the mammalian middle ear.  

PubMed

This study considers the general question how animal size limits the size and information receiving capacity of sense organs. To clarify this in the case of the mammalian middle ear, I studied 63 mammalian species, ranging from a small bat to the Indian elephant. I determined the skull mass and the masses of the ossicles malleus, incus and stapes (M, I and S), and measured the tympanic membrane area, A1. The ossicular mass (in mg) is generally negatively allometric to skull mass (in g), the regression equation for the whole material (excluding true seals) being y = 1.373 x(0.513). However, for very small mammals the allometry approaches isometry. Within a group of large mammals no distinct allometry can be discerned. The true seals (Phocidae) are exceptional by having massive ossicles. The size relations within the middle ear are generally rather constant. However, the I/M relation is slightly positively allometric, y = 0.554 x(1.162). Two particularly isometric relations were found; the S/(M + I) relation for the ossicles characterized by the regression equation y = 0.054 x(0.993), and the relation between a two-dimensional measure of the ossicles and the tympanic membrane ares, (M + I)2/3 /A1. As in isometric ears the sound energy collected by the tympanic membrane is linearly related to its area, the latter isometry suggests that, regardless of animal size, a given ossicular cross-sectional area is exposed to a similar sound-induced stress. Possible morphological middle ear adaptations to particular acoustic environments are discussed. PMID:7559173

Nummela, S

1995-05-01

264

[Correction of prominent ears (author's transl)].  

PubMed

The the last 20 years plastic surgery was increasingly concerned with the correction of prominent ears. It is not possible to exactly define this anomaly, since it is not only subject to certain tangible criteria from a medical point of view, but also to the purely personal, esthetic perception of the patient. In the present publication an attempt is made to make a broad classification of those factors which together result in the impression of too prominent ears. There are basically 3 techniques for correcting this anomaly: 1. simple suture techniques, 2. simple section techniques, 3. combined techniques. The publication deals specifically with one operation-technique. It is basically a modification of the "Stenstroem-Anthelix-plastic;; procedure. This technique is indicated in the case of uncontoured, flatly prominent ears, whereby the correction is achieved by creating an anthelix bulge. An instrument which we developed as well as our dressing technique are described. Finally, the 123 operations which we carried out according to this technique between 1975 and 1979 are critically assessed. PMID:7007300

Staindl, O

1980-07-01

265

An investigation of ear necrosis in pigs  

PubMed Central

Porcine ear necrosis was investigated in 23 conveniently chosen farms, consisting of 14 case farms and 9 control farms. Biopsies of lesions and oral swabs from pigs on 11 case farms were examined by histology and bacterial culture. All farms were visited for observations and a survey on management, housing, and the presence of other clinical signs or behavioral vices. Histological examination revealed that the lesions began on the surface and progressed to deeper layers, and that vascular damage did not appear to be the initiating cause. Spirochetes were only rarely observed in histological examination and were not cultured from biopsies and oral swabs. Staphylococcus aureus and Staphylococcus hyicus were cultured from 91% and 66% of samples, respectively. Ear biting and a humid environment were associated with ear necrosis. On some farms large numbers of pigs were affected and lesions were sometimes extensive. The condition appears to be an infectious disease beginning on the surface of the skin; contributing environmental and management factors are likely. PMID:24155434

Park, Jeonghwa; Friendship, Robert M.; Poljak, Zvonimir; DeLay, Josepha; Slavic, Durda; Dewey, Catherine E.

2013-01-01

266

Why Do Elephants Flap Their Ears?  

NASA Astrophysics Data System (ADS)

It is estimated that a 4200 kg elephant generates as much as 5.12 kW of heat. How the elephant dissipates its metabolic heat and regulates its body temperature has been investigated during the past seven decades. Findings and conclusions differ sharply. The high rate of metabolic heat coupled with low surface area to volume ratio and the absence of sweat glands eliminate surface convection as the primary mechanism for heat removal. Noting that the elephant ears have high surface area to volume ratio and an extensive vascular network, ear flapping is thought to be the principal thermoregulatory mechanism. A computational and experimental program is carried out to examine flow and heat transfer characteristics. The ear is modeled as a uniformly heated oscillating rectangular plate. Our computational work involves a three-dimensional time dependent CFD code with heat transfer capabilities to obtain predictions of the flow field and surface temperature distributions. This information was used to design an experimental setup with a uniformly heated plate of size 0.2m x 0.3m oscillating at 1.6 cycles per second. Results show that surface temperature increases and reaches a steady periodic oscillation after a period of transient oscillation. The role of the vortices shed off the plate in heat transfer enhancement will be discussed.

Koffi, Moise; Jiji, Latif; Andreopoulos, Yiannis

2009-11-01

267

Middle-ear velocity transfer function, cochlear input immittance, and middle-ear efficiency in chinchilla  

PubMed Central

The transfer function HV between stapes velocity VS and sound pressure near the tympanic membrane PTM is a descriptor of sound transmission through the middle ear (ME). The ME power transmission efficiency (MEE), the ratio of sound power entering the cochlea to power entering the middle ear, was computed from HV measured in seven chinchilla ears and previously reported measurements of ME input admittance YTM and ME pressure gain GMEP [Ravicz and Rosowski, J. Acoust. Soc. Am. 132, 2437–2454 (2012); J. Acoust. Soc. Am. 133, 2208–2223 (2013)] in the same ears. The ME was open, and a pressure sensor was inserted into the cochlear vestibule for most measurements. The cochlear input admittance YC computed from HV and GMEP is controlled by a combination of mass and resistance and is consistent with a minimum-phase system up to 27?kHz. The real part Re{YC}, which relates cochlear sound power to inner-ear sound pressure, decreased gradually with frequency up to 25 kHz and more rapidly above that. MEE was about 0.5 between 0.1 and 8?kHz, higher than previous estimates in this species, and decreased sharply at higher frequencies. PMID:24116422

Ravicz, Michael E.; Rosowski, John J.

2013-01-01

268

Middle-ear velocity transfer function, cochlear input immittance, and middle-ear efficiency in chinchilla.  

PubMed

The transfer function H(V) between stapes velocity V(S) and sound pressure near the tympanic membrane P(TM) is a descriptor of sound transmission through the middle ear (ME). The ME power transmission efficiency (MEE), the ratio of sound power entering the cochlea to power entering the middle ear, was computed from H(V) measured in seven chinchilla ears and previously reported measurements of ME input admittance Y(TM) and ME pressure gain G(MEP) [Ravicz and Rosowski, J. Acoust. Soc. Am. 132, 2437-2454 (2012); J. Acoust. Soc. Am. 133, 2208-2223 (2013)] in the same ears. The ME was open, and a pressure sensor was inserted into the cochlear vestibule for most measurements. The cochlear input admittance Y(C) computed from H(V) and G(MEP) is controlled by a combination of mass and resistance and is consistent with a minimum-phase system up to 27 kHz. The real part Re{Y(C)}, which relates cochlear sound power to inner-ear sound pressure, decreased gradually with frequency up to 25 kHz and more rapidly above that. MEE was about 0.5 between 0.1 and 8 kHz, higher than previous estimates in this species, and decreased sharply at higher frequencies. PMID:24116422

Ravicz, Michael E; Rosowski, John J

2013-10-01

269

Epidemiology and natural history of arteriovenous malformations.  

PubMed

The epidemiology and natural history of cerebral arteriovenous malformations (AVMs) remains incompletely elucidated. Several factors are responsible. With regard to the incidence and prevalence of AVMs, the results of prior studies have suffered because of the retrospective design, the use of nonspecific ICD-9 codes, and a focus on small genetically isolated populations. Recent data from the New York Islands AVM Hemorrhage Study, an ongoing, prospective, population-based survey determining the incidence of AVM-related hemorrhage and the associated rates of morbidity and mortality in a zip code-defined population of 10 million people, suggests that the AVM detection rate is 1.21/100,000 person-years (95% confidence interval [CI] 1.02-1.42) and the incidence of AVM-hemorrhage is 0.42/100,000 person-years (95% CI 0.32-0.55). Contemporaneous data from the Northern Manhattan Stroke Study, a prospective, longitudinal population-based study of nearly 150,000 patients in which the focus is to define the incidence of stroke, suggest the crude incidence for first-ever AVM-related hemorrhage to be 0.55/100,000 person-years (95% CI 0.11-1.61). Efforts are ongoing to study the natural history of both ruptured and unruptured AVMs in these datasets to examine the relevance of prior studies of patients selected for conservative follow up in Finland. In addition, data are being gathered to determine whether risk factors for future hemorrhage, which have previously been established in small case series, are valid when applied to whole populations. Together, these data should help inform therapeutic decisionmaking. PMID:16466233

Stapf, C; Mohr, J P; Pile-Spellman, J; Solomon, R A; Sacco, R L; Connolly, E S

2001-01-01

270

[Materials for reconstruction of the middle ear].  

PubMed

To rehabilitate most cases of conductive hearing loss closure of ear drum perforations and rebuilding of the ossicular chain can be performed. Due to the great number of biocompatible bone substitute materials available it is occasionally difficult for the surgeon to choose the most favorable substitute. Autogenous structures (ossicles, cortical bone, cartilage) and allogenous tissues (ossicles, cortical bone, cartilage, dentin) are possible bone replacement materials. Xenogenic tissue is currently not used in middle ear surgery. Ionomer cement is a hybrid material for replacement of bone but does not fit direct classification of the various classes of alloplastic materials in current use: that is, metals (gold, steel wire, platinum, titanium), plastics (polyethylene, polytetrafluorethylene) and ceramics (ceramic oxide, carbon, calcium-phosphate ceramic, vitreous ceramic). For restoration of the sound conductive apparatus preference is given to autogenous ossicles because cortical bone is resorbed and cartilage weakens over time. Most surgeons do not use allogenous tissue, because of the possible transmission of such infectious disease as immunodeficiency syndrome or Creutzfeldt-Jakob disease. Only dentin deserves special attention as a possible bone substitute in the middle ear because its form can be preserved during sterilization. Based on the observations available to date, it becomes apparent that titanium implants hold greater promise than gold. Form-stable synthetic materials are not generally recommended due to foreign body reactions which have been confirmed by many investigators. Ceramic materials (e.g. ceramic oxide, carbon, calcium-phosphate ceramic, glass ceramic) are well tolerated in the middle ear and have also proved to be useful over time. Hybrid bone substitute ionomer cement is easily workable and well integrated, showing a good functional outcome. For many years good results in otosclerosis surgery have been achieved with a prosthesis made of platinum-wire and Teflon. Short-term follow-up periods hold great promise with pistons made of gold. Autogenous ossicles, ionomer cement and recently titanium protheses--as far as usable--are employed by the author for reconstructing the middle ear. For the time being platinum-Teflon prostheses and gold are used in otosclerosis surgery. PMID:10197273

Geyer, G

1999-02-01

271

Local Model of Arteriovenous Malformation of the Human Brain  

NASA Astrophysics Data System (ADS)

Vascular diseases of the human brain are one of the reasons of deaths and people's incapacitation not only in Russia, but also in the world. The danger of an arteriovenous malformation (AVM) is in premature rupture of pathological vessels of an AVM which may cause haemorrhage. Long-term prognosis without surgical treatment is unfavorable. The reduced impact method of AVM treatment is embolization of a malformation which often results in complete obliteration of an AVM. Pre-surgical mathematical modeling of an arteriovenous malformation can help surgeons with an optimal sequence of the operation. During investigations, the simple mathematical model of arteriovenous malformation is developed and calculated, and stationary and non-stationary processes of its embolization are considered. Various sequences of embolization of a malformation are also considered. Calculations were done with approximate steady flow on the basis of balanced equations derived from conservation laws. Depending on pressure difference, a fistula-type AVM should be embolized at first, and then small racemose AVMs are embolized. Obtained results are in good correspondence with neurosurgical AVM practice.

Nadezhda Telegina, Ms; Aleksandr Chupakhin, Mr; Aleksandr Cherevko, Mr

2013-02-01

272

Anorectal Malformations Associated with Esophageal Atresia in Neonates  

PubMed Central

Purpose Anorectal malformations are often associated with other anomalies, reporting frequency with 40-70%. Gastrointestinal anomalies have been known to be relatively less common than associated anomalies of other organ system. This study was performed to assess a distinctive feature of cases associated with esophageal atresia. Methods Clinical data (from January 2000 through December 2011) on the 196 subjects with anorectal malformations, managed in our Hospital, were reviewed. Total 14 neonates were identified with accompanying esophageal atresia and retrospective analysis was conducted. Results The incidence was 7.1% and there were 8 male and 6 female subjects. Only 2 cases were associated with esophageal atresia without tracheoesophageal fistula. Although variable cases of anorectal malformation in female subjects, almost cases were anorectal malformations with rectourethral fistula in male. Other associated anomalies were identified in all cases, with more than 3 anomalies in 10 cases. There were 4 VACTERL (Vertebral abnormalities, Anal atresia, Cardiac anomalies, Tracheoesophageal fistula, Esophageal atresia, Renal and Limb anomalies) associations accounting for 28.6%, but could not identify chromosomal anomaly. Most cases were managed with staged procedure, usually primary repair of esophageal atresia and diverting colostomy. Overall mortality rate was 21.4%, mainly caused by heart problems. Conclusion This study shows that early diagnosis and rational surgical approach with multidisciplinary plan are mandatory in managing anorectal malformations with esophageal atresia, when considering a high frequency of associated anomaly and a relative high mortality. PMID:24010103

Byun, Shin Yun; Lim, Ryoung Kyoung; Park, Kyung Hee; Kim, Hae Young

2013-01-01

273

Neuroimaging of Dandy-Walker malformation: new concepts.  

PubMed

Dandy-Walker malformation (DWM) is the most common human cerebellar malformation, characterized by hypoplasia of the cerebellar vermis, cystic dilation of the fourth ventricle, and an enlarged posterior fossa with upward displacement of the lateral sinuses, tentorium, and torcular. Although its pathogenesis is not completely understood, there are several genetic loci related to DWM as well as syndromic malformations and congenital infections. Dandy-Walker malformation is associated with other central nervous system abnormalities, including dysgenesis of corpus callosum, ectopic brain tissue, holoprosencephaly, and neural tube defects. Hydrocephalus plays an important role in the development of symptoms and neurological outcome in patients with DWM, and the aim of surgical treatment is usually the control of hydrocephalus and the posterior fossa cyst. Imaging modalities, especially magnetic resonance imaging, are crucial for the diagnosis of DWM and distinguishing this disorder from other cystic posterior fossa lesions. Persistent Blake's cyst is seen as a retrocerebellar fluid collection with cerebrospinal fluid signal intensity and a median line communication with the fourth ventricle, commonly associated with hydrocephalus. Mega cisterna magna presents as an extraaxial fluid collection posteroinferior to an intact cerebellum. Retrocerebellar arachnoid cysts frequently compress the cerebellar hemispheres and the fourth ventricle. Patients with DWM show an enlarged posterior fossa filled with a cystic structure that communicates freely with the fourth ventricle and hypoplastic vermis. Comprehension of hindbrain embryology is of utmost importance for understanding the cerebellar malformations, including DWM, and other related entities. PMID:24132069

Correa, Gustavo Gumz; Amaral, Lázaro Faria; Vedolin, Leonardo Modesti

2011-12-01

274

Sham control methods used in ear-acupuncture/ear-acupressure randomized controlled trials: a systematic review.  

PubMed

Ear-acupuncture/ear-acupressure (EAP) has been used for a range of health conditions with numerous randomized controlled trials (RCTs) investigating its efficacy and safety. However, the design of sham interventions in these RCTs varied significantly. This study systematically reviewed RCTs on EAP for all clinical conditions involving a number of sham EAPs as a control intervention. The review is guided by the Cochrane Handbook for Systematic Reviews of Interventions 5.1.0 and investigated the types and differences of sham EAP interventions. Four electronic English databases (The Cochrane Library, PubMed, Embase, CINAHL®) and two Chinese databases (CQVIP, CNKI) were searched in December 2012 and 55 published RCTs comparing real and sham EAP for any clinical condition were included. Characteristics of participants, real and sham interventions, and outcomes were extracted. Four types of sham methods were identified. Among the 55 RCTs, 25 studies involved treatment on nonspecific ear acupoints as the sham method; seven studies used nonacupoints on the ear; nine studies selected placebo needles or placebo ear-acupressure on the same ear acupoints for the real treatment; 10 studies employed pseudo-intervention; and five studies combined two of the above methods to be the sham control. Other factors of treatment such as number of points, treatment duration, and frequency also varied greatly. Risk of bias assessment suggests that 32 RCTs were "high risk" in terms of participants blinding, and 45 RCTs were "high risk" in terms of personnel blinding. Meta-analysis was not conducted due to the high clinical heterogeneity across included studies. No relationship was found between the sham designs and efficacy outcomes, or between the sham types and dropout rate. No solid conclusion of which design is the most appropriate sham control of EAP could be drawn in this review. PMID:24138333

Zhang, Claire Shuiqing; Yang, Angela Weihong; Zhang, Anthony Lin; May, Brian H; Xue, Charlie Changli

2014-03-01

275

Comparison of an animal model of arteriovenous malformation with human arteriovenous malformation.  

PubMed

This study assessed the blood flow and histological changes of an animal model of arteriovenous malformation (AVM) over 84 days in 71 rats, and compared the histological findings to 17 specimens of human AVM. Carotid-jugular fistula blood flow positively correlated with time. The maximum flow rate occurred at 42 days, at which time the nidus was considered mature and was histologically similar to human AVMs. Morphological similarities between the model and human AVM vessels included heterogeneously thickened walls, splitting of the elastic lamina, thickened endothelial layers, endothelial cushions, lack of tight junctions, loss of endothelial continuity, endothelial-subendothelial adherent junctions, and luminally directed filopodia. These findings support the theory that vascular changes in human AVMs are secondary to increased flow and provide a basis for using this model in studies of AVMs. PMID:19910197

Tu, Jian; Karunanayaka, Athula; Windsor, Apsara; Stoodley, Marcus A

2010-01-01

276

Manual therapy and ear pain: a report of four cases  

PubMed Central

Purpose: To report and discuss four cases of ear pain which were treated successfully with manual therapy. Methods: Report of four cases. Results: Four patients with ear pain were referred for chiropractic consult. They were all treated with a combination of manual therapy and exercise with resolution of their ear symptoms. Conclusions: The mechanism of idiopathic ear pain that may be amenable to manual therapy is not fully known. Further research is needed to investigate the etiology of this disorder and to determine whether manual therapy and exercise are viable options in some patients with idiopathic ear pain. In the meantime, it may be advantageous for otolaryngologists to seek input from physicians skilled in assessment and treatment of the musculoskeletal system in cases ear pain for which an otolarygologic etiology cannot be found. PMID:21403781

Murphy, Donald R.; Gay, Charles W.

2011-01-01

277

Cutaneous vascular malformation in a guinea pig (Cavia porcellus).  

PubMed

A skin lesion classified as a vascular malformation is reported in a young-adult, female guinea pig. The physical examination revealed a 3 x 2-cm irregularly shaped violaceous plaque located on the left caudal flank. The surface of the plaque was ulcerated and bled intermittently, resulting in fatal blood loss. On histology the mass consisted of variably sized vascular spaces filled with red blood cells and variable amounts of extramedullary haematopoietic cells, lined by well-differentiated endothelial cells often surrounded by one layer of spindle-shaped cells. Based on immunohistochemistry, the spindle cell population was confirmed to be smooth muscle cells and no proliferation of endothelial cells was found with the Ki67 proliferation marker. Histological and immunohistochemical findings were consistent with a vascular malformation. Classification of vascular malformations and potential treatments are discussed. To the authors' knowledge, this is the first reported case of a cutaneous vascular lesion in a guinea pig. PMID:14989706

Osofsky, Anna; De Cock, Hilde E V; Tell, Lisa A; Norris, Aimee J; White, Stephen D

2004-02-01

278

Cranial malformations in related white lions (Panthera leo krugeri).  

PubMed

White lions (Panthera leo krugeri) have never been common in the wild, and at present, the greatest population is kept in zoos where they are bred for biological and biodiversity conservation. During the years 2003 to 2008 in a zoological garden in northern Italy, 19 white lions were born to the same parents, who were in turn paternally consanguineous. Out of the 19 lions, 4 (21%) were stillborn, 13 (69%) died within 1 month, and 1 (5%) was euthanatized after 6 months because of difficulty with prehension of food. Six lions (32%) showed malformations involving the head (jaw, tongue, throat, teeth, and cranial bones). One lion (5%) still alive at 30 months revealed an Arnold-Chiari malformation upon submission for neurological evaluation of postural and gait abnormalities. Paternal consanguinity of the parents, along with inbreeding among white lions in general, could account for the high incidence of congenital malformations of the head in this pride of white lions. PMID:20826844

Scaglione, F E; Schröder, C; Degiorgi, G; Zeira, O; Bollo, E

2010-11-01

279

Spinal dysraphism: genetic relation to neural tube malformations.  

PubMed Central

The families of 207 index patients treated for spinal dysraphism at The Hospital for Sick Children were studied to discover whether the condition was aetiologically related to the classical neural tube malformation--spina bifida cystica and anencephaly. The index patients had all had a tethered conus medullaris and one or more of a variety of anomalies of the spinal cord, vertebrae, or skin overlying the vertebral column. Of 364 sibs of index patients, 9 had an encephaly and 6 spina bifida cystica, a pro-proportion of 4.12%. This approximates to the proportion of sibs affected by neural tube malformations in the London region when the index patients themselves have spina bifida or anencephaly. It is, therefore, appropriate that the mothers of children with spinal dysraphism should be offered prenatal screening for neural tube malformations. PMID:794474

Carter, C O; Evans, K A; Till, K

1976-01-01

280

[Congenital malformations of the lung, the radiologist's point of view].  

PubMed

Congenital lung malformations include a complex range of developmental abnormalities. Currently, most are diagnosed prenatally or during early childhood. They may, however, be discovered later, incidentally or in connection with non-specific symptoms, sometimes severe. Knowledge of their radiological appearances is necessary for their detection. Proper technique and analysis of cross-sectional imaging, computed tomography and magnetic resonance imaging, allow a definitive diagnosis in most patients and pre-treatment evaluation of surgical cases. This review will describe the radiological aspects of congenital pulmonary malformations, especially those which may occur in late childhood or adult life. When present, alternative diagnoses will be discussed. A distinction will be made between anomalies originating from bronchopulmonary structures, such as bronchial atresia, bronchogenic cyst, congenital lobar overinflation, cystic adenomatoid malformation, and forms related to vascular anomalies (vascular rings, anomalous left pulmonary artery, pulmonary underdevelopment, proximal interruption of the pulmonary artery, pulmonary sequestration, scimitar syndrome). PMID:22742469

Berteloot, L; Bobbio, A; Millischer-Bellaïche, A-E; Lambot, K; Breton, S; Brunelle, F

2012-06-01

281

Review of congenital inner ear abnormalities on CT temporal bone  

PubMed Central

The aetiology of profound hearing loss in children is complex and multifactorial. Congenital inner ear abnormality is a major cause of hearing loss in children. CT temporal bone imaging is the modality of choice in the investigation of hearing loss. Recognising the congenital abnormalities of the inner ear guides the clinician's management of the condition. This pictorial essay illustrates the congenital abnormalities of the inner ear on high resolution CT temporal bone images and correlation with developmental arrest during embryology. PMID:21849370

Yiin, R S Z; Tang, P H; Tan, T Y

2011-01-01

282

Management of scrotal arteriovenous malformation with transcatheter embolisation coils and percutaneous sclerotherapy under angiographic guidance.  

PubMed

Arteriovenous malformations of the scrotum are infrequent and are usually treated by a combination of endovascular embolisation and surgery. We present a case of scrotal arteriovenous malformation treated effectively by a combination of endovascular and direct percutaneous techniques. PMID:25492446

So, Wing Lung Alvin; Chaganti, Joga; Waugh, Richard; Ferguson, Richard J

2014-12-10

283

ASSESSMENT OF ENVIRONMENTAL STRESSORS POTENTIALLY RESPONSIBLE FOR MALFORMATIONS IN NORTH AMERICAN ANURAN AMPHIBIANS  

EPA Science Inventory

A number of species of anuran amphibians from different regions across North America have recently exhibited an increased occurrence of, predominantly, hind limb malformations. Research concerning factors potentially responsible for these malformations has focused extensively on ...

284

Pulmonary arteriovenous malformations presenting as refractory heart failure  

PubMed Central

A 22-year-old young man with a history of idiopathic dilated cardiomyopathy (IDC) was admitted to our hospital due to difficult-to-control heart failure. A thoracic X-ray showed multiple nodules at the both pulmonary hilus and upper lobe of the right lung. Computed tomography (CT) angiography of the thorax confirmed arteriovenous malformation (AVM). However, effective treatment was impossible due to the poor physical condition; he died a few days later. Here we reported on the case of pulmonary arteriovenous malformations (PAVMs) being misdiagnosed as refractory heart failure. PMID:25276390

Chen, Kai-Hong; Huang, Guo-Yong; Song, Wei

2014-01-01

285

Multiple Complex Congenital Malformations in a Rabbit Kit (Oryctolagus cuniculi)  

PubMed Central

Congenital malformations may occur during early embryogenesis in cases of genetic abnormalities or various environmental factors. Affected subjects most often have only one or 2 abnormalities; subjects rarely have several unrelated congenital defects. Here we describe a case of a stillborn New Zealand white rabbit with multiple complex congenital malformations, including synophthalmia, holoprosencephaly, gastroschisis, and a supernumerary hindlimb, among other anomalies. There was no historical exposure to teratogens or other known environmental causes. Although not confirmed, this case was most likely a rare spontaneous genetic event. PMID:24209970

Booth, Jennifer L; Peng, Xuwen; Baccon, Jennifer; Cooper, Timothy K

2013-01-01

286

Prenatal diagnosis of congenital renal and urinary tract malformations  

PubMed Central

Congenital abnormalities of the kidneys and the urinary tract are the most common sonographically identified ­malformations in the prenatal period. Obstructive uropathies account for the majority of cases. The aim of prenatal diagnosis and management is to detect those anomalies having impact on the prognosis of the affected child and ­requiring early postnatal evaluation or treatment to minimize adverse outcomes. In this paper, we summarize the embryology of kidneys and urinary tract, the normal sonographic appearance through­out pregnancy and the prenatal diagnosis of their congenital malformations. PMID:24753862

Hindryckx, A.; De Catte, L.

2011-01-01

287

Uncommon cavernous malformation of the optic chiasm: a case report  

PubMed Central

Cavernous malformation (CM) is a vascular malformation disorder characterized by a berry-like mass of expanded blood vessels. CM, originating from the optic chiasm. usually leads to chiasma syndrome presenting with bitemporal hemianopsia. We report a 28-year-old male presenting with left homonymous hemianopsia. Magnetic resonance imaging (MRI) revealed an occupied lesion located in the right side of the optic chiasm, and a clinical diagnosis of chiasmal CM was made. Microsurgical excision was performed via anterolateral pterional craniotomy. The patient showed good recovery with slight improvement of the visual field deficits after the operation. No CM recurrence was discovered during the follow-up MRI scans. PMID:22892383

2012-01-01

288

Maternal smoking habits and congenital malformations: a population study.  

PubMed Central

Maternal smoking habits in 67 609 singleton pregnancies were examined. The overall incidence of congenital malformations was 2.8% in both non-smokers and smokers. On analysing congenital defects according to individual systems there was no significant difference in the incidence of malformations according to the number of cigarettes smoked, except for neural tube defects. Further analysis, taking social class into account, showed that neural tube defects were less common in non-smokers in social classes I and II. This study suggests that maternal smoking does not have teratogenic effects in the offspring, except in the case of neural tube defects, where the effect is at most modest. PMID:466337

Evans, D R; Newcombe, R G; Campbell, H

1979-01-01

289

A case report of meningioma extending to the middle ear  

PubMed Central

Extracranial meningioma with extension into a middle ear is very uncommon. A 74-year-old female was admitted to our hospital with right ear bleeding when removing earwax. In this case, magnetic resonance imaging, computed tomography, her past history and operative findings would consider as infiltrative growth from the right sphenoid ridge meningioma to the right middle ear via the right petrous pyramid and bilateral optic nerve. She underwent only partial extirpation with decompression for optic nerve, rather than total extirpation including middle ear and temporal bone, due to wide invasion of the middle cranial fossa and caversinus sinus. PMID:24765466

Kusunoki, Takeshi; Ikeda, Katsuhisa; Miyashita, Mie

2012-01-01

290

EARS, MARS combined radio observations - 2014  

NASA Astrophysics Data System (ADS)

The Lyrid meteor shower was generated on 21-22 April 2014 by the passage of the Earth through the path of the debris of the comet C/1861 G1 (Thatcher). The Camelopardalids meteor shower was generated on 23-24 May 2014 by the passage of the Earth through the path of the debris of the comet 209P/Linear. The EurAstro Radio Station (EARS) and the Malta Astro Radio Station (MARS) were operated in parallel for two combined radio observation campaigns. The campaigns revealed that further combined radio observation campaigns are necessary to solve the problem of estimating the number of lost radio meteor echoes.

Tomezzoli, Giancarlo

2014-02-01

291

Empirical Evaluation of Advanced Ear Biometrics Ping Yan Kevin W. Bowyer  

E-print Network

Empirical Evaluation of Advanced Ear Biometrics Ping Yan Kevin W. Bowyer Department of Computer experimental inves- tigation of ear biometrics to date. Approaches consid- ered include a PCA ("eigen-ear the robustness and variability of ear biometrics, ear symmetry is also in- vestigated. In our experiments around

Bowyer, Kevin W.

292

A large pelvic arteriovenous malformation in an adult patient with cystic fibrosis  

PubMed Central

We present a prepubertal male cystic fibrosis patient with high circulating oestrogen levels (as a consequence of severe cystic-fibrosis-related hepatobiliary disease) who subsequently developed a large pelvic arteriovenous malformation. This has not previously been described in patients with cystic fibrosis, despite the association between high oestrogen levels and arteriovenous malformations. The aetiology and treatment options of arteriovenous malformations are discussed.???Keywords: arteriovenous malformation; cystic fibrosis PMID:10435171

Ledson, M; Wahbi, Z; Harris, P; Walshaw, M

1999-01-01

293

Congenital bilateral perisylvian syndrome (CBPS): do concomitant esophageal malformations indicate a poor prognosis?  

PubMed

Congenital bilateral perisylvian syndrome (CBPS) is a syndrome of cortical malformation characterized by faciopharyngoglossomasticatory diplegia. We report on two cases of CBPS with associated esophageal malformations and a poor mental and motor development. The association of CBPS and esophageal malformations may indicate a subgroup of patients with a very early prenatal injury, characterised by a bad prognosis due to severe cortical disorganization. However, it can not be excluded that the association of CBPS and esophageal malformation is purely coincidental. PMID:11508551

Küker, W; Friese, S; Riethmüller, J; Krägeloh-Mann, I

2000-12-01

294

Ear emergence in perennial ryegrass as affected by differences in light and temperature before ear initiation  

Microsoft Academic Search

The effects of differences in daylength and temperature, before ear initiation, on subsequent emergence in perennial ryegrass selections were studied in the glasshouse. When no artificial light or heat was used, prolific and uniform emergence was observed. Of seven other combinations compared, uniform but much earlier emergence was obtained from only that treatment where the natural sequence of events, cold

S. O. Fejer

1960-01-01

295

Maximum Real-Ear Gain of in-the-Ear Hearing Aids.  

ERIC Educational Resources Information Center

Three hearing aid manufacturers provided custom full-shell in-the-ear hearing aids designed for maximum acoustic gain for each of three hearing-impaired adults. Full-on coupler gain curves were similar across all nine hearing aids, with individual differences producing substantial variance in insertion gain across hearing aids. (Author/DB)

Erickson, Faye N.; Van Tasell, Dianne J.

1991-01-01

296

[Coexistence of pulmonary sequestration and congenital cystic adenomatoid malformation. Case report and review of literature].  

PubMed

This paper presents a case report of an infant, with a prenatally diagnosed congenital lung malformation, which proved to be pulmonary sequestration and congenital cystic adenomatoid malformation. The authors discuss current knowledge on diagnosis, clinical course and suggestions of ante- and postnatal management of patients with pulmonary sequestration or/and congenital cystic lung malformation. PMID:24964242

Sanocki, Marcin Tomasz; Dudek, Anna; Krenke, Katarzyna; Karolczak, Maciej; Motylewicz, Barbara; Kulus, Marek

2014-01-01

297

Vibrations in the human middle ear  

PubMed Central

Summary Background Middle ear surgery techniques can improve hearing destroyed by disease, but results of treatment are difficult to predict. Therefore, researchers use a Laser Doppler Vibrometer to measure vibrations of human middle ear ossicles. Material/Methods Measurements of ossicular chain vibrations are performed on fresh human temporal bone specimens using Laser Doppler Vibrometer. Vibrations of stapes are recorded in 3 cases: 1) for intact ossicular chain, 2) when incus long process is removed, and 3) after long process reconstruction with bone cement. A typical analysis of transfer function is completed by other methods applied in dynamics. Results Measurements and analysis of stapes vibrations in case of intact and damaged ossicular chain show regular and irregular behavior which can be recognize with the help of phase portraits, recurrence plots, correlation dimension, and Hurst and Lyapunov exponents. The long process reconstruction with bone cement gives good results in improving hearing. Conclusions Recurrence plots, and Lyapunov and Hurst exponents used in the study complete information obtained from transfer function and can be employed to enrich the classical approach to ossicular chain vibrations. PMID:22129895

Rusinek, Rafa?; Szyma?ski, Marcin; Warmi?ski, Jerzy; Zadrozniak, Marek; Morshed, Kamal

2011-01-01

298

Sp8 regulates inner ear development  

PubMed Central

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

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

2014-01-01

299

Enhanced visualization of inner ear structures  

NASA Astrophysics Data System (ADS)

Recently surgery requires extensive support from imaging technologies in order to increase effectiveness and safety of operations. One of important tasks is to enhance visualisation of quasi-phase (transparent) 3d structures. Those structures are characterized by very low contrast. It makes differentiation of tissues in field of view very difficult. For that reason the surgeon may be extremly uncertain during operation. This problem is connected with supporting operations of inner ear during which physician has to perform cuts at specific places of quasi-transparent velums. Conventionally during such operations medical doctor views the operating field through stereoscopic microscope. In the paper we propose a 3D visualisation system based on Helmet Mounted Display. Two CCD cameras placed at the output of microscope perform acquisition of stereo pairs of images. The images are processed in real-time with the goal of enhancement of quasi-phased structures. The main task is to create algorithm that is not sensitive to changes in intensity distribution. The disadvantages of existing algorithms is their lack of adaptation to occuring reflexes and shadows in field of view. The processed images from both left and right channels are overlaid on the actual images exported and displayed at LCD's of Helmet Mounted Display. A physician observes by HMD (Helmet Mounted Display) a stereoscopic operating scene with indication of the places of special interest. The authors present the hardware ,procedures applied and initial results of inner ear structure visualisation. Several problems connected with processing of stereo-pair images are discussed.

Niemczyk, Kazimierz; Kucharski, Tomasz; Kujawinska, Malgorzata; Bruzgielewicz, Antoni

2004-07-01

300

Evaluation of fungal flora in normal and diseased canine ears.  

PubMed

This study was undertaken to characterize otic fungal flora encountered in normal dogs, atopic dogs with no clinical or cytological evidence of otitis and dogs with otitis externa. Forty-two normal dogs, 23 atopic dogs and 32 dogs with otitis were included in the study. Samples for otic fungal culture and cytology were obtained from all animals, for a total of 194 ears. Sixty-seven ear samples (34%) were culture positive for saprophytic fungal organisms, as follows: 43 (64%) Penicillium species, 13 (19%) Aspergillus species and the remaining 17% comprised of various other saprophytic fungal organisms. Cytological evidence of saprophytic fungal colonization or infection was not found in any animal. There was no relationship between positive saprophytic fungal culture and any study group. Thirty-three ear samples (17%) were positive for Malassezia pachydermatis. Cytological findings of Malassezia were significantly associated with positive culture for Malassezia (P = 0.006 left ear; P = 0.019 right ear). Furthermore, increased numbers of Malassezia led to a higher chance of positive culture (P = 0.003 left ear; P = 0.008 right ear; McNemar's test). Malassezia pachydermatis was more likely to be cultured from ears with increased cerumen. Ear type (erect or pendulous) was not significantly associated with positive culture for Malassezia or saprophytic fungal organisms. There was no relationship between positive Malassezia culture and any study group; however, Malassezia was more likely to be cultured from individual dogs in the atopic or otitis groups that also had other dermatological signs consistent with allergic dermatitis and/or pyoderma (P = 0.031 left ear; P = 0.005 right ear). PMID:20868397

Campbell, Jacquelyn J; Coyner, Kimberly S; Rankin, Shelley C; Lewis, Thomas P; Schick, Anthea E; Shumaker, Amy K

2010-12-01

301

496. Phys. Lett A., (2006) A.G.Ramm, The shape of the ear canal The shape of the ear canal #+  

E-print Network

496. Phys. Lett A., (2006) A.G.Ramm, The shape of the ear canal 1 #12; The shape of the ear canal@math.ksu.edu Abstract It is proved that the measurement of the acoustic pressure on the ear membrane allows one to determine the shape of the ear canal uniquely. 1 Introduction Consider a bounded domain D # R n , n = 3

302

Within-ear and across-ear interference in a cocktail-party listening task Douglas S. Brungart1 and Brian D. Simpson2  

E-print Network

Within-ear and across-ear interference in a cocktail-party listening task Douglas S. Brungart1 talker is present in the same ear as the target speech or when a masking talker is present in a different ear than the target speech, little is known about selective auditory attention in tasks with a target

Allen, Jont

303

Hereditary hemorrhagic telangiectasia in children: endovascular treatment of neurovascular malformations  

Microsoft Academic Search

Hereditary hemorrhagic telangiectasia (HHT) is a heterogeneous disease that can present with a variety of clinical manifestations. The neurovascular complications of this disease, especially in children, may be potentially devastating. The purpose of this article was to review the therapeutic results of endovascular treatment of neurovascular malformations in children. A total of 31 patients under the age of 16 were

T. Krings; S. M. Chng; A. Ozanne; H. Alvarez; G. Rodesch; P. L. Lasjaunias

2005-01-01

304

Anorectal malformations associated with a presacral tumor and sacral defect  

Microsoft Academic Search

The triad of anorectal malformation, presacral mass, and sacral bony defect is quite rare. During the past 18 years, five patients with this triad were treated in our institutions. Four had covered anal or anorectal stenosis, while one infant had anorectal agenesis. There was a central defect of the sacral bone in three and sacral hemivertebrae in two patients. An

Yoshiaki Tsuchida; Watit Watanasupt; Toshio Nakajo

1989-01-01

305

The role of colostomy in the management of anorectal malformations  

Microsoft Academic Search

The role of the colostomy in the definitive correction of anorectal malformations is controversial with regard no only to indications for its use, but also to location and type. We reviewed the records of 272 patients with 307 colostomies and analyzed the frequency of complications related to these controversial points. There were 250 completely divided and 57 loop colostomies, constructed

Steven Wilkins; Alberto Pefia

1988-01-01

306

Macrocephaly-capillary malformation syndrome: three new cases.  

PubMed

Overgrowth syndromes can be associated with asymmetry, obesity and various vascular malformations. Macrocephaly-Capillary Malformation (M-CM) is a more recently defined overgrowth syndrome characterized by cutaneous capillary malformation occurring in association with macrocephaly with tendency to progressive enlargement, abnormalities of somatic growth with body asymmetry including brain asymmetry, developmental delay, typical face with full cheeks, nevus flammeus of the nose and/or philtrum and upper lip, joint laxity, thickened subcutaneous tissue and 2/3 syndactyly of the toes. We evaluated three patients who demonstrated characteristic features of the disorder. Patients seen in the Genetic clinic of a tertiary care center were subjects of the analysis. We present three cases of overgrowth syndrome with common features of macrocephaly, capillary malformation, dysmorphic face and abnormal neurocognitive profile. These features are consistent with the newly defined M-CM syndrome. This condition must be differentiated from other overgrowth syndromes for appropriate surveillance for known complications and genetic counseling. We discuss the diagnostic criteria for the disorder and also recommend to include typical face with full cheeks, nevus flammeus of the nose and/or philtrum and upper lip, considering it as minor criterion on the basis of findings in present cases. One of the cases had bluish white iris which has not been described earlier. PMID:22029941

Panigrahi, Inusha; Bhushan, Mani; Yadav, Mukesh; Khandelwal, Niranjan; Singhi, Pratibha

2012-02-15

307

Surgical treatment of venous malformations in Klippel-Trénaunay syndrome  

Microsoft Academic Search

Purpose: Klippel-Trénaunay syndrome (KTS) is a complex congenital anomaly, characterized by varicosities and venous malformations (VMs) of one or more limbs, port-wine stains, and soft tissue and bone hypertrophy. Venous drainage is frequently abnormal because of embryonic veins, agenesis, hypoplasia, valvular incompetence, or aneurysms of deep veins. We previously reported on the surgical management of KTS. In this article, we

Audra A Noel; Peter Gloviczki; Kenneth J Cherry; Thom W Rooke; Anthony W Stanson; David J Driscoll

2000-01-01

308

Congenital Malformations and Birthweight in Areas with Potential Environmental Contamination  

Microsoft Academic Search

Public concern exists about the potential for reproductive damage that may result from exposures to environmental contaminants. Therefore, the authors sought to determine if there was an association between a child's congenital malformation or a child's lowered weight at birth and his or her mother's residence in a census tract where a site of environmental contamination had been documented. Exposure

Gary M. Shaw; Jane Schulman; Jonathan D. Frisch; Susan K. Cummins; John A. Harris

1992-01-01

309

Congenital malformations and birthweight in areas with potential environmental contamination  

Microsoft Academic Search

Public concern exists about the potential for reproductive damage that may result from exposures to environmental contaminants. Therefore, the authors sought to determine if there was an association between a child's congenital malformation or a child's lowered weight at birth and his or her mother's residence in a census tract where a site of environmental contamination had been documented. Exposure

G. M. Shaw; J. Schulman; J. D. Frisch; J. A. Harris; S. K. Cummins

2009-01-01

310

Proximity to Pollution Sources and Risk of Amphibian Limb Malformation  

PubMed Central

The cause of limb deformities in wild amphibian populations remains unclear, even though the apparent increase in prevalence of this condition may have implications for human health. Few studies have simultaneously assessed the effect of multiple exposures on the risk of limb deformities. In a cross-sectional survey of 5,264 hylid and ranid metamorphs in 42 Vermont wetlands, we assessed independent risk factors for nontraumatic limb malformation. The rate of nontraumatic limb malformation varied by location from 0 to 10.2%. Analysis of a subsample did not demonstrate any evidence of infection with the parasite Ribeiroia. We used geographic information system (GIS) land-use/land-cover data to validate field observations of land use in the proximity of study wetlands. In a multiple logistic regression model that included land use as well as developmental stage, genus, and water-quality measures, proximity to agricultural land use was associated with an increased risk of limb malformation (odds ratio = 2.26; 95% confidence interval, 1.42–3.58; p < 0.001). The overall discriminant power of the statistical model was high (C = 0.79). These findings from one of the largest systematic surveys to date provide support for the role of chemical toxicants in the development of amphibian limb malformation and demonstrate the value of an epidemiologic approach to this problem. PMID:16263502

Taylor, Brynn; Skelly, David; Demarchis, Livia K.; Slade, Martin D.; Galusha, Deron; Rabinowitz, Peter M.

2005-01-01

311

LIMITED PCB ANTAGONISM OF TCDD-INDUCED MALFORMATIONS IN MICE  

EPA Science Inventory

C57BL/6N mice, used to model induction of cleft palate and kidney malformations in offspring following maternal treatment with TCDD, were dosed on gestation day (gd) 9 with ,2',4,4',5,5'-hexachloro-biphenyl (HCB) (62.5, 125, 250, 500. 1000 mg/kg) and/or gd 10 with 2,3,7,8-tetrach...

312

Magnetic resonance imaging in the malformative syndromes with mental retardation  

Microsoft Academic Search

We reported on thirteen children affected with malformative syndromes associated with mental retardation. MRI examination showed in all of them different types of anomalies; agenesis of corpus callosum, ventricular dilatation and cerebral matter alterations were the most frequent findings. The authors give a brief description of the clinical reports and of the anomalies observed with MRI; they conclude that MRI

O. Gabrielli; U. Salvolini; G. V. Coppa; C. Catassi; R. Rossi; A. Manca; R. Lanza; P. L. Giorgi

1990-01-01

313

Polymicrogyria: a common and heterogeneous malformation of cortical development.  

PubMed

Polymicrogyria (PMG) is one of the most common malformations of cortical development. It is characterized by overfolding of the cerebral cortex and abnormal cortical layering. It is a highly heterogeneous malformation with variable clinical and imaging features, pathological findings, and etiologies. It may occur as an isolated cortical malformation, or in association with other malformations within the brain or body as part of a multiple congenital anomaly syndrome. Polymicrogyria shows variable topographic patterns with the bilateral perisylvian pattern being most common. Schizencephaly is a subtype of PMG in which the overfolded cortex lines full-thickness clefts connecting the subarachnoid space with the cerebral ventricles. Both genetic and non-genetic causes of PMG have been identified. Non-genetic causes include congenital cytomegalovirus infection and in utero ischemia. Genetic causes include metabolic conditions such as peroxisomal disorders and the 22q11.2 and 1p36 continguous gene deletion syndromes. Mutations in over 30 genes have been found in association with PMG, especially mutations in the tubulin family of genes. Mutations in the (PI3K)-AKT pathway have been found in association PMG and megalencephaly. Despite recent genetic advances, the mechanisms by which polymicrogyric cortex forms and causes of the majority of cases remain unknown, making diagnostic and prenatal testing and genetic counseling challenging. This review summarizes the clinical, imaging, pathologic, and etiologic features of PMG, highlighting recent genetic advances. PMID:24888723

Stutterd, Chloe A; Leventer, Richard J

2014-06-01

314

SHORT COMMUNICATION Fusarium mangiferae associated with mango malformation  

E-print Network

SHORT COMMUNICATION Fusarium mangiferae associated with mango malformation in the Sultanate of Oman in the beginning of 2005 in plantations at Sohar in the Sultanate of Oman. The affected inflorescences were factor 1 . -tubulin Mango (Mangifera indica) is an important perennial crop in the Sultanate of Oman

315

Neurodevelopmental Outcomes in Children with Cerebellar Malformations: A Systematic Review  

ERIC Educational Resources Information Center

Cerebellar malformations are increasingly diagnosed in the fetal period. Consequently, their consideration requires stressful and often critical decisions from both clinicians and families. This has resulted in an emergent need to understand better the impact of these early life lesions on child development. We performed a comprehensive literature…

Bolduc, Marie-Eve; Limperopoulos, Catherine

2009-01-01

316

Neurodevelopmental outcomes in children with cerebellar malformations: a systematic review.  

PubMed

Cerebellar malformations are increasingly diagnosed in the fetal period. Consequently, their consideration requires stressful and often critical decisions from both clinicians and families. This has resulted in an emergent need to understand better the impact of these early life lesions on child development. We performed a comprehensive literature search of studies describing neurodevelopmental outcomes of cerebellar malformations between January 1997 and December 2007. Overall, the data suggested that children with isolated inferior vermis hypoplasia (IVH) and mega cisterna magna (MCM) have a good developmental outcome, whereas children with molar tooth sign/Joubert syndrome, vermis hypoplasia, pontocerebellar hypoplasia (PCH) type II, and cerebellar agenesis experience moderate to severe global developmental delays. Reports for Dandy-Walker malformation (DWM) were conflicting; however, the presence of a normally lobulated vermis and the absence of associated brain anomalies were associated with a more favourable outcome. Finally, children with isolated cerebellar hypoplasia experienced fewer impairments. Important methodological limitations highlighted include a lack of standardized outcome measure use in 79% of studies and the predominant use of retrospective study designs (85%), with 40% limited to case reports or case-series. In summary, rigorous outcome studies describing the spectrum of disabilities in survivors are urgently needed to accurately delineate the long-term neurodevelopmental consequences of cerebellar malformations. PMID:19191827

Bolduc, Marie-Eve; Limperopoulos, Catherine

2009-04-01

317

Radiological anatomy of the vascularization of cranial dural arteriovenous malformations  

Microsoft Academic Search

Summary Knowledge of the radiological anatomy of the cranial durai vascularization allows a flexible and appropriate approach to the pretherapeutic investigation of cranial durai arteriovenous malformations. The variability of the origin of these arteries requires that several possible sources of vascular supply be investigated — internal carotid, internal maxillary, ascending pharyngeal, occipital and vertebral — and that each of their

P. Lasjaunias; L. Lopez-Ibor; A. Abanou; Ph. Halimi

1984-01-01

318

[MRI of malformative syringomyelia. Descriptive and developmental aspect].  

PubMed

132 cases of malformative syringomyelia have been studied at the C.I.E.R.M. (Interdepartmental Magnetic Resonance Center) of Bicêtre Hospital. The authors describe their technique for the exploration on the cord in case of suspected intramedullary cavitation, and emphasize the morphological and evolutive aspects of these abnormalities, whether they have been operated or not. PMID:1602436

Bekkali, F; Doyon, D; Idir, A B; Belal, N; Hurth, M; Ayache, B; Ducot, B

1992-02-01

319

Recurrent Arteriovenous Malformation of the Scrotum Secondary to Pelvic Trauma  

Microsoft Academic Search

We describe a case of recurrent scrotal arteriovenous malformation (AVM) in a young man secondary to pelvic fracture sustained during a road traffic accident. This was diagnosed as a lipoma on the ultrasound and clinical examination. To our knowledge, this is the first reported case of recurrent scrotal AVM secondary to trauma.

Vineet Agrawal; Pankaj Dangle; Suks Minhas; David Ralph; Nim Christopher

2006-01-01

320

Rolandic arteriovenous malformations: improvement in limb function by IBC embolization.  

PubMed

Three patients with arteriovenous malformations in the rolandic region and significant limb deficit showed virtually complete functional recovery after awake operative embolization of most of the malformations using isobutyl-2 cyanoacrylate. Two of these patients, with functionally useless hands, had sustained the deficits months earlier as the result of a specific brain-damaging event: one as a result of surgery and the other as a result of a hemorrhage. Both of these showed significant return of function during the awake operative embolization procedure. The other patient had had progressive leg weakness over a 2 year period. The theory of steal phenomenon as an explanation for progressive neurologic deficits in association with large arteriovenous malformations must be extended to explain apparently stable deficits after some brain trauma (surgery or hemorrhage). These results suggest that some patients with arteriovenous malformations and without clinical deficits who are near a critical level of "near ischemia" may be thrown out of balance by an acute interceding event. PMID:3927674

Fox, A J; Girvin, J P; Viñuela, F; Drake, C G

1985-01-01

321

Diagnosis and Nonsurgical Management of Uterine Arteriovenous Malformation  

SciTech Connect

Uterine arteriovenous malformation (AVM) is an uncommon problem and traditional treatment by hysterectomy excludes the possibility of future pregnancy. Developments in interventional techniques make transcatheter embolization of the feeding vessel(s) a therapeutic alternative, potentially preserving the patient's fertility. We present a case of successful endovascular treatment of uterine AVM.

Rangarajan, R. D.; Moloney, J. C.; Anderson, H. J. [Eastbourne District General Hospital, Department of Radiology (United Kingdom)], E-mail: Hugh.Anderson@esht.nhs.uk

2007-11-15

322

[Significance of the ear in the determination of age].  

PubMed

The morphology of human ear conch is said to be rather individual, but a perfect person-identification by this mean is not possible. It is demonstrated by photographs-made in a 15 years' interval-that ear conch and auricular area can be typically marked by proceeding age and specific diseases. PMID:3741048

Röhm, E; Adam, E

1986-01-01

323

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

PubMed

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

Chapman, Susan Caroline

2011-01-01

324

Can you hear me now? Understanding vertebrate middle ear development  

PubMed Central

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

Chapman, Susan Caroline

2010-01-01

325

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...

326

Within-consonant perceptual differences in the hearing impaired ear  

E-print Network

) The consonant recognition of 17 ears with sensorineural hearing loss is evaluated for 14 consonants /p, t, k, f and Delhorne (1987). Overall, the effects of hearing impaWithin-consonant perceptual differences in the hearing impaired ear Andrea Trevinoa) and Jont B

Allen, Jont

327

2 Cases of Intracranial Meningioma Extending into the Middle Ear  

Microsoft Academic Search

The meningiomas are slowly growing tumors arising from meningoepithelial cells and they generally occur throughout the cra- niospinal axis. Meningiomas extending to the middle ear cavity are uncommom and these patients complain of otologic symptoms such as hearing loss, ear fullness, or otorrhea. Clinical findings frequently suggest otitis media resistant to conventional treatments, thereby facilitating misdiagnosis. CT and MRI of

Ki-Hong Chang; Jae-Hyun Seo; Dong Chang Lee; Myung Han Lee

2009-01-01

328

[Inverted caloric nystagmus of perforated ears upon air caloric stimulation].  

PubMed

It is well known that inverted caloric nystagmus is seen during air caloric testing in cases of chronic otitis media. The mechanism of inversion and its clinical significance are discussed here. Temperature changes in the tympanic cavity and external ear canal were measured with a microthermister and a digital tester in seventeen ears with tympanic membrane perforation, during bithermal air caloric testing. The tympanic cavity mucosa was cooled by hot stimulation because of the evaporation of heat. When the perforation was closed or humidified air was used, the tympanic cavity mucosa was not cooled by hot stimulation and the inverted caloric nystagmus changed to a normal response. Inverted caloric nystagmus occurred in 30.4% of 335 ears affected by chronic otitis media with perforation. Inverted caloric nystagmus occurred in 90 ears with hot stimulation and in 12 ears with cold stimulation. Inverted caloric nystagmus turned to normal response after myringoplasty in all of 10 ears. The cooling effect caused by evaporation of water from the moist middle ear mucosa during dry air blowing and direct thermal conduction to the vestibulum through a perforation of the ear drum and inversion of the endolymphatic convection seemed to cause the inversion. PMID:2040915

Koide, C

1991-03-01

329

Rhabdomyosarcoma in middle ear of an adult: a rare presentation  

PubMed Central

Rhabdomyosarcoma of the middle ear is a rare tumor, even rarer in adults and has a very poor prognosis. We report here an unusual case of rhabdomyosarcoma in middle ear of an adult, mimicking chronic suppurative otitis media and facial nerve palsy. PMID:24960749

Bhargava, S; Grover, M; Mehta, J; Maheshwari, V

2012-01-01

330

Preventing Cauliflower Ear with a Modified Tie-Through Technique.  

ERIC Educational Resources Information Center

Describes a quick, simple tie-through suture technique (in which a collodion packing is secured to the auricle with two buttons) for preventing cauliflower ear following external ear trauma in wrestlers and boxers. The technique ensures constant compression; multiple treatments for fluid reaccumulation are rarely necessary. (SM)

Dimeff, Robert J.; Hough, David O.

1989-01-01

331

cis-Regulatory Mutations Are a Genetic Cause of Human Limb Malformations  

PubMed Central

The underlying mutations that cause human limb malformations are often difficult to determine, particularly for limb malformations that occur as isolated traits. Evidence from a variety of studies shows that cis-regulatory mutations, specifically in enhancers, can lead to some of these isolated limb malformations. Here, we provide a review of human limb malformations that have been shown to be caused by enhancer mutations and propose that cis-regulatory mutations will continue to be identified as the cause of additional human malformations as our understanding of regulatory sequences improves. PMID:21509892

VanderMeer, Julia E.; Ahituv, Nadav

2011-01-01

332

Is a Swine Model of Arteriovenous Malformation Suitable for Human Extracranial Arteriovenous Malformation? A Preliminary Study  

SciTech Connect

Objective: A chronic arteriovenous malformation (AVM) model using the swine retia mirabilia (RMB) was developed and compared with the human extracranial AVM (EAVM) both in hemodynamics and pathology, to see if this brain AVM model can be used as an EAVM model. Methods: We created an arteriovenous fistula between the common carotid artery and the external jugular vein in eight animals by using end-to-end anastomosis. All animals were sacrificed 1 month after surgery, and the bilateral retia were obtained at autopsy and performed hematoxylin and eosin staining and immunohistochemistry. Pre- and postsurgical hemodynamic evaluations also were conducted. Then, the blood flow and histological changes of the animal model were compared with human EAVM. Results: The angiography after operation showed that the blood flow, like human EAVM, flowed from the feeding artery, via the nidus, drained to the draining vein. Microscopic examination showed dilated lumina and disrupted internal elastic lamina in both RMB of model and nidus of human EAVM, but the thickness of vessel wall had significant difference. Immunohistochemical reactivity for smooth muscle actin, angiopoietin 1, and angiopoietin 2 were similar in chronic model nidus microvessels and human EAVM, whereas vascular endothelial growth factor was significant difference between human EAVM and RMB of model. Conclusions: The AVM model described here is similar to human EAVM in hemodynamics and immunohistochemical features, but there are still some differences in anatomy and pathogenetic mechanism. Further study is needed to evaluate the applicability and efficacy of this model.

Lv, Ming-ming, E-mail: lvmingming001@163.com [Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Department of Oral and Maxillofacial Surgery, Shanghai Key Laboratory of Stomatology (China)] [Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Department of Oral and Maxillofacial Surgery, Shanghai Key Laboratory of Stomatology (China); Fan, Xin-dong, E-mail: fanxindong@yahoo.com.cn [Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Department of Radiology (China)] [Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Department of Radiology (China); Su, Li-xin, E-mail: sulixin1975@126.com [Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Department of Oral and Maxillofacial Surgery, Shanghai Key Laboratory of Stomatology (China)] [Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Department of Oral and Maxillofacial Surgery, Shanghai Key Laboratory of Stomatology (China)

2013-10-15

333

A MANUAL FOR THE REMOVAL, FIXATION AND PRESERVATION OF CETACEAN EARS  

E-print Network

Julie Arruda Biology Department Woods Hole Oceanographic Institution Woods Hole, Massachusetts..............................................................................................8 Sperm Whale (Physeter macrocephalus) Ear Removal.............................................11 Beaked Whale Ear Removal..............................................................................15

334

"Play It by Ear"--Teachers' Responses to Ear-Playing Tasks during One-to-One Instrumental Lessons  

ERIC Educational Resources Information Center

This paper reports findings from the Ear-Playing Project in relation to the teaching strategies that 15 instrumental teachers adopted during one-to-one instrumental lessons whilst helping their students to copy music by ear from a recording. Overall, the teachers used a variety of strategies including singing and humming along with or without the…

Varvarigou, Maria

2014-01-01

335

The prevalence of middle ear pathogens in the outer ear canal and the nasopharyngeal cavity of healthy young adults.  

PubMed

Culturing middle ear fluid samples from children with chronic otitis media with effusion (OME) using standard techniques results in the isolation of bacterial species in approximately 30-50% of the cases. Haemophilus influenzae, Streptococcus pneumoniae and Moraxella catarrhalis, the classic middle ear pathogens of acute otitis media, are involved but, recently, several studies suggested Alloiococcus otitidis as an additional pathogen. In the present study, we used species-specific PCRs to establish the prevalence, in both the nasopharyngeal cavity and the outer ear, of H. influenzae, M. catarrhalis, S. pneumoniae and A. otitidis. The study group consisted of 70 healthy volunteers (aged 19-22 years). The results indicate a high prevalence (>80%) of A. otitidis in the outer ear in contrast to its absence in the nasopharynx. H. influenzae was found in both the outer ear and the nasopharynx (6% and 14%, respectively), whereas S. pneumoniae and M. catarrhalis were found only in the nasopharynx (9% and 34%, respectively).A. otitidis, described as a fastidious organism, were able to be cultured using an optimized culture protocol, with prolonged incubation, which allowed the isolation of A. otitidis in five of the nine PCR-positive samples out of the total of ten samples tested. Given the absence of the outer ear inhabitant A. otitidis from the nasopharynx, its role in the aetiology of OME remains ambiguous because middle ear infecting organisms are considered to invade the middle ear from the nasopharynx through the Eustachian tube. PMID:19895585

De Baere, T; Vaneechoutte, M; Deschaght, P; Huyghe, J; Dhooge, I

2010-07-01

336

Mechanics of the exceptional anuran ear  

PubMed Central

The anuran ear is frequently used for studying fundamental properties of vertebrate auditory systems. This is due to its unique anatomical features, most prominently the lack of a basilar membrane and the presence of two dedicated acoustic end organs, the basilar papilla and the amphibian papilla. Our current anatomical and functional knowledge implies that three distinct regions can be identified within these two organs. The basilar papilla functions as a single auditory filter. The low-frequency portion of the amphibian papilla is an electrically tuned, tonotopically organized auditory end organ. The high-frequency portion of the amphibian papilla is mechanically tuned and tonotopically organized, and it emits spontaneous otoacoustic emissions. This high-frequency portion of the amphibian papilla shows a remarkable, functional resemblance to the mammalian cochlea. PMID:18386018

Segenhout, Johannes M.; van Dijk, Pim

2008-01-01

337

3D ear identification based on sparse representation.  

PubMed

Biometrics based personal authentication is an effective way for automatically recognizing, with a high confidence, a person's identity. Recently, 3D ear shape has attracted tremendous interests in research field due to its richness of feature and ease of acquisition. However, the existing ICP (Iterative Closet Point)-based 3D ear matching methods prevalent in the literature are not quite efficient to cope with the one-to-many identification case. In this paper, we aim to fill this gap by proposing a novel effective fully automatic 3D ear identification system. We at first propose an accurate and efficient template-based ear detection method. By utilizing such a method, the extracted ear regions are represented in a common canonical coordinate system determined by the ear contour template, which facilitates much the following stages of feature extraction and classification. For each extracted 3D ear, a feature vector is generated as its representation by making use of a PCA-based local feature descriptor. At the stage of classification, we resort to the sparse representation based classification approach, which actually solves an l1-minimization problem. To the best of our knowledge, this is the first work introducing the sparse representation framework into the field of 3D ear identification. Extensive experiments conducted on a benchmark dataset corroborate the effectiveness and efficiency of the proposed approach. The associated Matlab source code and the evaluation results have been made publicly online available at http://sse.tongji.edu.cn/linzhang/ear/srcear/srcear.htm. PMID:24740247

Zhang, Lin; Ding, Zhixuan; Li, Hongyu; Shen, Ying

2014-01-01

338

Human fetal inner ear involvement in congenital cytomegalovirus infection  

PubMed Central

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

2013-01-01

339

Diencephalic-mesencephalic junction dysplasia: a novel recessive brain malformation.  

PubMed

We describe six cases from three unrelated consanguineous Egyptian families with a novel characteristic brain malformation at the level of the diencephalic-mesencephalic junction. Brain magnetic resonance imaging demonstrated a dysplasia of the diencephalic-mesencephalic junction with a characteristic 'butterfly'-like contour of the midbrain on axial sections. Additional imaging features included variable degrees of supratentorial ventricular dilatation and hypoplasia to complete agenesis of the corpus callosum. Diffusion tensor imaging showed diffuse hypomyelination and lack of an identifiable corticospinal tract. All patients displayed severe cognitive impairment, post-natal progressive microcephaly, axial hypotonia, spastic quadriparesis and seizures. Autistic features were noted in older cases. Talipes equinovarus, non-obstructive cardiomyopathy and persistent hyperplastic primary vitreous were additional findings in two families. One of the patients required shunting for hydrocephalus; however, this yielded no change in ventricular size suggestive of dysplasia rather than obstruction. We propose the term 'diencephalic-mesencephalic junction dysplasia' to characterize this autosomal recessive malformation. PMID:22822038

Zaki, Maha S; Saleem, Sahar N; Dobyns, William B; Barkovich, A James; Bartsch, Hauke; Dale, Anders M; Ashtari, Manzar; Akizu, Naiara; Gleeson, Joseph G; Grijalvo-Perez, Ana Maria

2012-08-01

340

Management of Cerebral Cavernous Malformations: From Diagnosis to Treatment  

PubMed Central

Cerebral cavernous malformations are the most common vascular malformations and can be found in many locations in the brain. If left untreated, cavernomas may lead to intracerebral hemorrhage, seizures, focal neurological deficits, or headaches. As they are angiographically occult, their diagnosis relies on various MR imaging techniques, which detect different characteristics of the lesions as well as aiding in planning the surgical treatment. The clinical presentation and the location of the lesion are the most important factors involved in determining the optimal course of treatment of cavernomas. We concisely review the literature and discuss the advantages and limitations of each of the three available methods of treatment—microsurgical resection, stereotactic radiosurgery, and conservative management—depending on the lesion characteristics.

Mouchtouris, Nikolaos; Chitale, Ameet; Starke, Robert M.; Tjoumakaris, Stavropoula I.; Rosenwasser, Robert H.; Jabbour, Pascal M.

2015-01-01

341

Behavioral effects of congenital ventromedial prefrontal cortex malformation  

PubMed Central

Background A detailed behavioral profile associated with focal congenital malformation of the ventromedial prefrontal cortex (vmPFC) has not been reported previously. Here we describe a 14 year-old boy, B.W., with neurological and psychiatric sequelae stemming from focal cortical malformation of the left vmPFC. Case Presentation B.W.'s behavior has been characterized through extensive review Patience of clinical and personal records along with behavioral and neuropsychological testing. A central feature of the behavioral profile is severe antisocial behavior. He is aggressive, manipulative, and callous; features consistent with psychopathy. Other problems include: egocentricity, impulsivity, hyperactivity, lack of empathy, lack of respect for authority, impaired moral judgment, an inability to plan ahead, and poor frustration tolerance. Conclusions The vmPFC has a profound contribution to the development of human prosocial behavior. B.W. demonstrates how a congenital lesion to this cortical region severely disrupts this process. PMID:22136635

2011-01-01

342

Diencephalic–mesencephalic junction dysplasia: a novel recessive brain malformation  

PubMed Central

We describe six cases from three unrelated consanguineous Egyptian families with a novel characteristic brain malformation at the level of the diencephalic–mesencephalic junction. Brain magnetic resonance imaging demonstrated a dysplasia of the diencephalic–mesencephalic junction with a characteristic ‘butterfly’-like contour of the midbrain on axial sections. Additional imaging features included variable degrees of supratentorial ventricular dilatation and hypoplasia to complete agenesis of the corpus callosum. Diffusion tensor imaging showed diffuse hypomyelination and lack of an identifiable corticospinal tract. All patients displayed severe cognitive impairment, post-natal progressive microcephaly, axial hypotonia, spastic quadriparesis and seizures. Autistic features were noted in older cases. Talipes equinovarus, non-obstructive cardiomyopathy and persistent hyperplastic primary vitreous were additional findings in two families. One of the patients required shunting for hydrocephalus; however, this yielded no change in ventricular size suggestive of dysplasia rather than obstruction. We propose the term ‘diencephalic–mesencephalic junction dysplasia’ to characterize this autosomal recessive malformation. PMID:22822038

Saleem, Sahar N.; Dobyns, William B.; Barkovich, A. James; Bartsch, Hauke; Dale, Anders M.; Ashtari, Manzar; Akizu, Naiara; Gleeson, Joseph G.; Grijalvo-Perez, Ana Maria

2012-01-01

343

A Case of Atypical Granuloma Annulare Involving Both Ears  

PubMed Central

We report a rare case of granuloma annulare (GA), affecting both ear antihelixes, in a 28-year old male patient that presented with a 1-year history of non-tender, firm, skin-colored, 1~5 mm papules on both ear antihelixes. There was no history of trauma. An excisional biopsy specimen taken from one of the lesions of the right ear revealed infiltration of histiocytes and lymphocytes around a zone of collagen alteration in the dermis. Based on the clinical and pathological findings, the patient was diagnosed with a rare case of bilateral GA of both antihelixes; this is the first report in the Korean dermatology literature. PMID:20523836

Kim, Jin Gu; Lee, Seung Hun

2009-01-01

344

Red ear syndrome: case report and review of the literature.  

PubMed

Red ear syndrome (RES) is characterized by the attack-like occurrence of erythema, edema and dysesthesia of one or less frequently both ears. We report the case of a 31-year-old woman with stabbing pain and marked erythema and discrete edema of one ear, existing for 4 months, occasionally accompanied by burning and local hyperhidrosis. Differential diagnoses such as perichondritis, contact dermatitis, erysipelas and other infections were ruled out. Based on her history, the clinical pattern and the unremarkable further diagnostics, we diagnosed RES. A review of the literature is included. PMID:21985901

Eismann, Regina; Gaul, Charly; Wohlrab, Johannes; Marsch, Wolfgang Christian; Fiedler, Eckhard

2011-01-01

345

Tumors and tumorlike lesions of dog and cat ears.  

PubMed

Bacterial and fungal otitis constitutes most ear disease in companion animals. However, a wide spectrum of infectious and noninfectious disease processes involve the structures of the ear and are of primary diagnostic consideration in cases of recurrent otitis or those refractive to traditional treatments. This article discusses several common to reasonably rare neoplastic and nonneoplastic space-occupying lesions of the external, middle, and internal ear. Although some conditions present as unique entities, many present similar to or concurrent with otitis, and should be considered in cases of clinically unresponsive otitis. PMID:23122175

Sula, Mee Ja M

2012-11-01

346

Red ear syndrome and auricular erythromelalgia: the same condition?  

PubMed

Several cases of relapsing attacks during which the ear becomes red and patients experience a burning sensation have been reported in the literature. This combination of symptoms has been described as 'red ear syndrome' (RES). We report on a 7-year-old boy who had episodes of reddening, swelling and a burning sensation in one ear with local hyperthermia persisting for 3 years. The differential diagnosis included RES and erythromelalgia, as isolated auricular variants of erythromelalgia have been described and the symptoms are difficult to distinguish from RES. In this report, we discuss the similarities and differences between RES and erythromelalgia. PMID:19489849

Brill, T J; Funk, B; Thaçi, D; Kaufmann, R

2009-12-01

347

[Iliac vessels malformations as a cause of gluteal varices].  

PubMed

Gluteal varices are caused by different malformations (congenital or other disorders) within pelvic vessels. They are usually congenital, rarely acquired disorders. The rarity of incidence, diagnostic difficulties and sometimes ineffective methods of treatment made the authors of this article to present a case of 51-year-old patient with hemorrhoids with concomitant left gluteal varices caused by internal iliac artery-vein fistula. PMID:18175563

Rudnicki, Piotr; Mazur, Ireneusz; Sosada, Krystyn; Koz?owski, Andrzej; Jopek, Janusz; Sitkiewicz, Tomasz

2007-01-01

348

Characteristics influencing informed consent on a congenital malformations registry.  

PubMed Central

Maryland law requires that all babies born with "sentinel birth defects" be reported to the State Department of Health, but mothers may deny consent for further contact. Consent was not strongly related to maternal age, race, or self-reported data on exposures, smoking, and drugs but was much less likely if the infant was dead. Selection bias in congenital malformations research may lead to underrepresentation of lethal defects, but self-reported data appear to be unbiased. PMID:3354744

Law, C; Robertson, M O; Panny, S R; Wulff, L M

1988-01-01

349

Fetal Facial Defects: Associated Malformations and Chromosomal Abnormalities  

Microsoft Academic Search

During an 8-year period, facial defects were observed in 146 (7%) of the 2,086 fetuses that underwent karyotyping in our unit because of fetal malformations and\\/or growth retardation. Chromosomal abnormalities were detected in 37 of 56 (66%) fetuses with micrognathia, in 10 of 13 (77%) with macroglossia, in 31 of 64 (48 %) with cleft lip and palate, in 5

K. H. Nicolaides; D. R. Salvesen; R. J. M. Snijders; C. M. Gosden

1993-01-01

350

An interesting finding in a congenital cystic adenomatoid malformation.  

PubMed

Here we describe a case of antenatally diagnosed congenital cystic adenomatoid malformation. Due to increasing cyst size and fetal hydrops an antenatal drain was inserted. The lesion was excised surgically soon after birth due to significant respiratory distress. During histological assessment an area of squamous metaplasia was found within the cyst wall. This developed as a result of the drain placement. At 4-year follow-up the child remains well and has experienced no long-term respiratory sequalae. PMID:23093507

Jones, Katherine Elizabeth; Teo, Ying; Lakhoo, Kokila

2012-01-01

351

Congestive hepatopathy secondary to large renal arteriovenous malformation  

PubMed Central

A 75-year-old woman presented with acute onset dyspnoea, and was found to have signs of pulmonary congestion on clinical examination. Imaging revealed cardiomegaly and coincident congestive hepatopathy, secondary to a left renal arteriovenous malformation. The presence of a high flow vascular shunt in the left kidney was possibly the causative factor behind both the high-output cardiac failure and congestive hepatopathy. PMID:23349173

Khalife, Mohammad; Faraj, Walid; Salah, Fatima; Haydar, Ali A

2013-01-01

352

[Medication-based therapy of infantile hemangioma and lymphatic malformations].  

PubMed

The therapy of vascular tumors and malformations should be interdisciplinary and performed according to available guidelines. Infantile hemangiomas (IH) are the most frequent vascular tumors of childhood and do not require treatment in most cases. If the IH is complicated by its location (e.g. facial or genital) or if the lesion threatens to cause loss of function, small localized IH should be treated by laser- or cryotherapy. If the IH is diffuse or rapidly growing it can be successfully treated using the ? blocker propranolol. The mechanism underlying the efficacy of this medication-based therapy is not completely understood and this still represents an experimental therapy. The results of molecular studies on vascular malformations have indicated new strategies for medical therapies. However, lymphatic malformations (LM) are still treated by surgery where possible, or sclerotherapy. Further investigations are necessary to determine whether new drugs such as the mTOR inhibitor rapamycin may be effective for treatment of diffuse LM. First case reports seem to be promising. PMID:24434958

Rössler, J; Braunschweiger, F; Schill, T

2014-01-01

353

Guidelines for the treatment of head and neck venous malformations  

PubMed Central

Venous malformation is one of the most common benign vascular lesions, with approximately 40% of cases appearing in the head and neck. They can affect a patient’s appearance and functionality and even cause life-threatening bleeding or respiratory tract obstruction. The current methods of treatment include surgery, laser therapy, sclerotherapy, or a combined. The treatment of small and superficial venous malformations is relatively simple and effective; however, the treatment of deep and extensive lesions involving multiple anatomical sites remains a challenge for the physicians. For complex cases, the outcomes achieved with one single treatment approach are poor; therefore, individualized treatment modalities must be formulated based on the patient’s condition and the techniques available. Comprehensive multidisciplinary treatments have been adapted to achieve the most effective results. In this paper, based on the national and international literature, we formulated the treatment guidelines for head and neck venous malformations to standardize clinical practice. The guideline will be renewed and updated in a timely manner to reflect cutting-edge knowledge and to provide the best treatment modalities for patients. PMID:23724158

Zheng, Jia Wei; Mai, Hua Ming; Zhang, Ling; Wang, Yan An; Fan, Xin Dong; Su, Li Xin; Qin, Zhong Ping; Yang, Yao Wu; Jiang, Yin Hua; Zhao, Yi Fang; Suen, James Y

2013-01-01

354

Prenatal diagnosis of lethal fetal malformation in Irish obstetric practice.  

PubMed

The diagnosis of lethal fetal malformation prenatally has profound implications for the pregnancy, the expectant couple and the medical care provided. The aim of this study was to investigate these implications and the medical factors pertaining to prenatal diagnosis of lethal fetal abnormality in current obstetric practice in Ireland. Data was collected prospectively from all cases of lethal fetal malformation diagnosed at the Fetal Medicine Unit, University College Hospital Galway from December 1997 to June 1998 inclusive. Diagnosis was made on the basis of ultrasound findings and invasive procedures (amniocentesis and chorionic villus sampling). Thirteen cases of lethal fetal abnormality were diagnosed: Edward's syndrome, Patau's syndrome, bilateral multicystic renal dysplasia, Potters sequence, hypoplastic left heart, anencephaly with craniorrhachischisis, lethal osteogenesis imperfecta and non-immune hydrops. Intrauterine death occurred in four cases. Four women had preterm complications e.g. preterm premature rupture of membranes, preterm labour, placental abruption, coagulopathy and severe pre-eclampsia. Three pregnancies progressed to term, two of which had a vaginal delivery and one had an elective caesarean section for malpresentation, all of which were early neonatal deaths. Three women chose to travel abroad in order to obtain a termination of pregnancy. Obstetric and neonatal dilemmas in management of lethal fetal malformation are discussed. PMID:10360111

Byrne, B M; Morrison, J J

1999-03-01

355

The malformations of the urinary system in autosomal disorders.  

PubMed

Data from the world literature about the pathology of the urinary system in autosomal chromosomal disorders are analyzed and compared with our own morphological investigations of this system in 63 cases of chromosomal disorders (Patau's, Edwards', Down's, Orbeli's, Wolf-Hirschhorn's syndromes, partial trisomy B and inversion of chromosome 2). The urinary system is most frequently involved in "cat-eye", triploidy, Orbeli's, Patau's and Edwards' syndromes. All known malformations of the urinary system are observed in children with chromosomal diseases, except infantile polycystic kidney and medullary "sponge" kidney. The authors recognize specific and nonspecific abnormalities of the urinary system. Nonspecific abnormalities, as simple renal dysgenesis, may be observed in all chromosomal disorders. Specific abnormalities are found only in certain chromosomal diseases. These malformations are an excess of embryonal lobulation of the kidney with the increase of its weight and cystic changes (Patau's syndrome), crossed renal ectopy with fusion and horseshoe kidney (Edwards' syndrome), unilateral renal agenesis (Orbeli's syndrome), hypospadia (Wolf-Hirschhorn's syndrome). A possible pathogenesis of malformations of the urinary system in chromosomal disorders is discussed. PMID:127413

Kravtzova, G I; Lazjuk, G I; Lurie, I W

1975-10-20

356

Computational modelling for the embolization of brain arteriovenous malformations.  

PubMed

Treatment of arteriovenous malformations (AVMs) of the brain often requires the injection of a liquid embolic material to reduce blood flow through the malformation. The type of the liquid and the location of injection have to be carefully planned in a pre-operative manner. We introduce a new model of the interaction of liquid embolic materials with blood for the simulation of their propagation and solidification in the AVM. Solidification is mimicked by an increase of the material's viscosity. Propagation is modelled by using the concept of two-fluids modelling and that of scalar transport. The method is tested on digital phantoms and on one anatomically derived patient AVM case. Simulations showed that intuitive behaviour of the two-fluid system can be confirmed and that two types of glue propagation through the malformation can be reproduced. Distinction between the two types of propagation could be used to identify fistulous and plexiform compartments composing the AVM and to characterize the solidification of the embolic material in them. PMID:22056793

Orlowski, Piotr; Summers, Paul; Noble, J Alison; Byrne, James; Ventikos, Yiannis

2012-09-01

357

Maternal smoking and congenital malformations: an epidemiological study.  

PubMed Central

In a case-control study undertaken in several hospitals in Connecticut, it was found that women who reported smoking more than 20 cigarettes a day during pregnancy had a relative risk of about 1.6 for congenital malformations in the offspring of that pregnancy compared with women who said they had not smoked at all during pregnancy. However, there was no significant increase in risk among women who reported smoking 20 or fewer cigarettes a day during pregnancy compared with those who said they had not smoked at all during pregnancy. The higher risk among moderate and heavy smokers could not be attributed to any of the potentially confounding variables considered in this study; furthermore, it was specific to smoking during pregnancy rather than before pregnancy, and increased with the average amount smoked a day. Nevertheless, because the increase in risk was modest, because response bias could exist in a study of this type, and because no other studies have examined in detail the smoking-congenital malformation hypothesis, further research is needed to determine whether the relationship between maternal smoking and congenital malformations in offspring is causal. PMID:355285

Kelsey, J L; Dwyer, T; Holford, T R; Bracken, M B

1978-01-01

358

Evolution of Gravity Receptors in the Ear  

NASA Technical Reports Server (NTRS)

The general status of a grant to investigate the origins and evolution of two hair cell types in the ears of a teleost fish, Astronotus ocellatus (the oscar), is presented. First, it was demonstrated that the cells in the rostral end of the saccule of the , Carassius auratus, are type 1-like, while those at the caudal end are type 2 cells. It was demonstrated that the dichotomy of hair cell types found in the utricle of the oscar is also found in the goldfish. Second, the lateral line system of the oscar was examined using gentamicin sulphate, an ototocix drug that destroys type 1- like hair cells but does not appear to damage type 2 hair cells. It was demonstrated that the hair cells found in neuromasts of lateral line canal organs were totally destroyed within 1 day of treatment, while the hair cells in free neuromasts were undamaged after 12 days of treatment. Third, it was demonstrated that the calyx, the specialized nerve ending, is not unique to amniotes and that it is present at least in the cristae of semicirular canals in goldfish. These results have demonstrated that: (1) there are multiple hair cell types in the vestibular endorgans of the ear of fishes, (2) these hair cell types are very similar to those found in the mammalian vestibular endorgans, (3) the nerve calyx is also present in fishes, and (4) multiple hair cell types and the calyx have evolved far earlier in the course of vertebrate evolution than heretofore thought. Understanding the structure of the vestibular endorgans has important implications for being able to understand how these organs respond to gravistatic, acceleration and acoustic input. The vestibular endorgans of fishes may provide an ideal system in which to analyze functional differences in hair cells. Not only are the two hair cell types similar to those found in mammals, they are located in very discrete regions in each endorgan. Thus, it is relatively easy to gain access to cells of one or the other type. The presence of two cell types in the lateral line have equally significant implications for studies of the vestibular system.

Popper, Arthur N. (Principal Investigator)

1996-01-01

359

Wideband Power Reflectance and Power Transmittance as Tools for Assessing Middle-Ear Function  

E-print Network

44 Wideband Power Reflectance and Power Transmittance as Tools for Assessing Middle-Ear Function using otoacoustic emissions can have high false positive rates, due to temporary middle-ear and outer-ear is limited, uncomfortable, and unreliable in young ears. By incorporating wideband acoustic power flow

Allen, Jont

360

Q What is a unilateral hearing loss? What causes a hearing loss in only one ear?  

E-print Network

Q· What is a unilateral hearing loss? · What causes a hearing loss in only one ear? · Will the hearing in my child's better ear get worse? · Why would my child have trouble hearing if one ear has normal hearing? · Will my child benefit from wearing a hearing aid in the ear with the hearing loss

O'Toole, Alice J.

361

ON A MATROID DEFINED BY EAR-DECOMPOSITIONS OF GRAPHS ZOLTAN SZIGETI *  

E-print Network

ON A MATROID DEFINED BY EAR-DECOMPOSITIONS OF GRAPHS ZOLT´AN SZIGETI * December 1, 1995 A-edge­connected graphs this value equals the minimum number (G) of even ears in ear­decompositions of G that G is 2-edge­ connected. Let G = (V, E) be an undirected, 2-edge­connected graph. An ear

Szigeti, Zoltán

362

Reflectance in ME disorders 1 Running head: REFLECTANCE IN MIDDLE-EAR DISORDERS  

E-print Network

Reflectance in ME disorders 1 Running head: REFLECTANCE IN MIDDLE-EAR DISORDERS Wideband energy reflectance measurements in adults with middle-ear disorders M. Patrick Feeney University of Washington with a variety of middle-ear disorders. The ER results from nine participants with middle-ear disorders and one

Allen, Jont

363

Have WISE EARS! for Life: Protect Yourself and Your Family from Noise-Induced Hearing Loss  

MedlinePLUS

Home Health Info Hearing, Ear Infections, and Deafness Have WISE EARS! for life Have WISE EARS! for life Protect yourself and your family from ... Loud bookmark . Top How can I protect my hearing in noisy situations? Wear ear plugs or special earmuffs when you are exposed ...

364

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

PubMed Central

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.

Özdemir, Mucize Eriç; Uzun, I??l; Karahasano?lu, Ay?e; Aygün, Mehmet; Ak?n, Hale; Yaz?c?o?lu, Fehmi

2014-01-01

365

Feature-level fusion method based on KFDA for multimodal recognition fusing ear and profile face  

Microsoft Academic Search

A novel method of feature-level fusion based on kernel Fisher discriminant analysis (KFDA) is proposed and applied to fusion of ear and profile face biometrics in this paper. Ear recognition is proved to be a new and promising authentication technique. Because of ear's special physiological structure and location, it is reasonable to combine ear with profile face for recognition in

Xiao-Na Xu; Zhi-Chun Mu; Li Yuan

2007-01-01

366

Modeling Directional Brightness Temperature of the Winter Wheat Canopy at the Ear Stage  

Microsoft Academic Search

The ear is the top layer of mature wheat and has very different geometric and thermal characteristics from that of leaves. Compared to the directional brightness temperature (DBT) of wheat canopy without ears, the DBT at the ear stage has specific features, and the ear effects could not be explained by previous models. This paper proposes a hybrid geometric optical

Yongming Du; Qinhuo Liu; Liangfu Chen; Qiang Liu; Tao Yu

2007-01-01

367

Prediction of the sound pressure at the ear drum for open fittings  

E-print Network

Prediction of the sound pressure at the ear drum for open fittings T. Sankowsky-Rothea , M. Blaua of hearing aids requires knowledge of the sound pressure generated at the ear drum. Traditionally, the sound pressure at the ear drum is estimated by the use of a model of an average ear canal (e.g. a coupler

Paris-Sud XI, Université de

368

Nanomedicine strategies for drug delivery to the ear.  

PubMed

The highly compartmentalized anatomy of the ear aggravates drug delivery, which is used to combat hearing-related diseases. Novel nanosized drug vehicles are thought to overcome the limitations of classic approaches. In this article, we summarize the nanotechnology-based efforts involving nano-objects, such as liposomes, polymersomes, lipidic nanocapsules and poly(lactic-co-glycolic acid) nanoparticles, as well as nanocoatings of implants to provide an efficient means for drug transfer in the ear. Modern strategies do not only enhance drug delivery efficiency, in the inner ear these vector systems also aim for specific uptake into hair cells and spiral ganglion neurons. These novel peptide-mediated strategies for specific delivery are reviewed in this article. Finally, the biosafety of these vector systems is still an outstanding issue, since long-term application to the ear has not yet been assessed. PMID:23837855

Pritz, Christian Oliver; Dudás, József; Rask-Andersen, Helge; Schrott-Fischer, Anneliese; Glueckert, Rudolf

2013-07-01

369

Energy extraction from the biologic battery in the inner ear  

E-print Network

Endocochlear potential (EP) is a battery-like electrochemical gradient found in and actively maintained by the inner ear [superscript 1, 2]. Here we demonstrate that the mammalian EP can be used as a power source for ...

Bandyopadhyay, Saurav

370

21 CFR 344.12 - Ear drying aid active ingredient.  

Code of Federal Regulations, 2010 CFR

...Ingredients § 344.12 Ear drying aid active ingredient. The active ingredient of the product consists of isopropyl alcohol 95 percent in an anhydrous glycerin 5 percent base. [65 FR 48905, Aug. 10,...

2010-04-01

371

Making an Effort to Listen: Mechanical Amplification in the Ear  

PubMed Central

The inner ear’s performance is greatly enhanced by an active process defined by four features: amplification, frequency selectivity, compressive nonlinearity, and spontaneous otoacoustic emission. These characteristics emerge naturally if the mechanoelectrical transduction process operates near a dynamical instability, the Hopf bifurcation, whose mathematical properties account for specific aspects of our hearing. The active process of non-mammalian tetrapods depends upon active hair-bundle motility, which emerges from the interaction of negative hair-bundle stiffness and myosin-based adaptation motors. Taken together, these phenomena explain the four characteristics of the ear’s active process. In the high-frequency region of the mammalian cochlea, the active process is dominated instead by the phenomenon of electromotility, in which the cell bodies of outer hair cells extend and contract as the protein prestin alters its membrane surface area in response to changes in membrane potential. PMID:18760690

Hudspeth, A. J.

2009-01-01

372

Get Smart: Know When Antibiotics Work - Ear Infections  

MedlinePLUS

... Antibiotic Use Respiratory Illnesses Sinus Infection Sore Throat Common Cold and Runny Nose Ear Infections Bronchitis (Chest Cold) ... Tips Appropriate Treatment Summary Cough Illness/Bronchitis The Common Cold Otitis Media Pharyngitis: Treat Only Proven GAS Online ...

373

Penetrating middle ear trauma: a report of 2 cases.  

PubMed

Penetrating middle ear injury can result in hearing loss, vertigo, and facial nerve injury. We describe the cases of 2 children with penetrating trauma to the right ear that resulted in ossicular chain disruption; one injury was caused by cotton-tipped swabs and the other by a wooden matchstick. Symptoms in both children included hearing loss and otalgia; in addition, one child experienced ataxia and the other vertigo. Physical examination in both cases revealed a perforation in the posterosuperior quadrant of the tympanic membrane and visible ossicles. Audiometry identified a moderate conductive hearing loss in one child and a mild sensorineural hearing loss in the other. Both children underwent middle ear exploration and reduction of a subluxed stapes. We discuss the diagnosis, causes, and management of penetrating middle ear trauma. To reduce the morbidity associated with these traumas, otologic surgeons should act promptly and be versatile in choosing methods of repairing ossicular chain injuries. PMID:15742770

Neuenschwander, Michael C; Deutsch, Ellen S; Cornetta, Anthony; Willcox, Thomas O

2005-01-01

374

Division of Earth Sciences (EAR) -- Dear Colleague Letter  

NSF Publications Database

... Date : May 12, 1993 File : nsf9361 DIVISION OF EARTH SCIENCES NSF 93-61 (New) Dear Colleague: The ... National Science Foundation's Division of Earth Sciences (EAR) is 1 June 1993. This will be the ...

375

Music to our ears During a visit to Santa  

E-print Network

#12;Music to our ears During a visit to Santa Cruz coordinated by UCSC's Arts & Lectures, Wynton Marsalis helped the young--and young at heart--celebrate the timeless music of Duke Ellington. Features

California at Santa Cruz, University of

376

How minute sooglossid frogs hear without a middle ear  

PubMed Central

Acoustic communication is widespread in animals. According to the sensory drive hypothesis [Endler JA (1993) Philos Trans R Soc Lond B Biol Sci 340(1292):215–225], communication signals and perceptual systems have coevolved. A clear illustration of this is the evolution of the tetrapod middle ear, adapted to life on land. Here we report the discovery of a bone conduction–mediated stimulation of the ear by wave propagation in Sechellophryne gardineri, one of the world’s smallest terrestrial tetrapods, which lacks a middle ear yet produces acoustic signals. Based on X-ray synchrotron holotomography, we measured the biomechanical properties of the otic tissues and modeled the acoustic propagation. Our models show how bone conduction enhanced by the resonating role of the mouth allows these seemingly deaf frogs to communicate effectively without a middle ear. PMID:24003145

Boistel, Renaud; Aubin, Thierry; Cloetens, Peter; Peyrin, Françoise; Scotti, Thierry; Herzog, Philippe; Gerlach, Justin; Pollet, Nicolas; Aubry, Jean-François

2013-01-01

377

Green laser light activates the inner ear  

NASA Astrophysics Data System (ADS)

The hearing performance with conventional hearing aids and cochlear implants is dramatically reduced in noisy environments and for sounds more complex than speech (e. g. music), partially due to the lack of localized sensorineural activation across different frequency regions with these devices. Laser light can be focused in a controlled manner and may provide more localized activation of the inner ear, the cochlea. We sought to assess whether visible light with parameters that could induce an optoacoustic effect (532 nm, 10-ns pulses) would activate the cochlea. Auditory brainstem responses (ABRs) were recorded preoperatively in anesthetized guinea pigs to confirm normal hearing. After opening the bulla, a 50-?m core-diameter optical fiber was positioned in the round window niche and directed toward the basilar membrane. Optically induced ABRs (OABRs), similar in shape to those of acoustic stimulation, were elicited with single pulses. The OABR peaks increased with energy level (0.6 to 23 ?J/pulse) and remained consistent even after 30 minutes of continuous stimulation at 13 ?J, indicating minimal or no stimulation-induced damage within the cochlea. Our findings demonstrate that visible light can effectively and reliably activate the cochlea without any apparent damage. Further studies are in progress to investigate the frequency-specific nature and mechanism of green light cochlear activation.

Wenzel, Gentiana I.; Balster, Sven; Zhang, Kaiyin; Lim, Hubert H.; Reich, Uta; Massow, Ole; Lubatschowski, Holger; Ertmer, Wolfgang; Lenarz, Thomas; Reuter, Guenter

2009-07-01

378

Bill malformations in double-crested cormorants with low exposure to organochlorines  

SciTech Connect

Eight of 20 newly hatched double-crested cormorants (Phalacrocorax auritus), captured at Dore Lake (Saskatchewan, Canada) and raised in captivity, developed malformed bills when they were 2 to 3 weeks old. Malformation was characterized by abnormal flexure and rotation of the maxilla and mandible, resulting in a crossed bill. By radiography, the premaxillary and dental bones were misshapen. Morphologically similar malformed bills in free-living comorants have been attributed to exposure to polyhalogenated aromatic hydrocarbons. However, the concentrations of total PCBs in the livers of these captive cormorants with malformed bills and in their diet were lower than have been previously associated with such malformations and were considered too low to have been the cause. The bill malformations may have been caused by deficiency of vitamin D{sub 3}, because the cormorants were kept indoors without exposure to ultraviolet light and were fed frozen fish that may have been deficient in this vitamin.

Kuiken, T.; Fox, G.A.; Danesik, K.L.

1999-12-01

379

Blackbird and Starling Feeding Behavior on Ripening Corn Ears1  

Microsoft Academic Search

The behavior of red-winged blackbirds {Agelaius phoemcei\\/s), common grackles (Quiscalus quiscula), brown-headed cowbirds (Molothrus ater), and juvenile European starlings (Sturnus vulgaris) feeding on ears of corn was studied in an aviary. The species differed significantly in their propensity to attack (penetrate the husk and feed on kernels) ears of corn. Redwings and starlings were more active attackers than grackles and

GLEN E. BERNHARDT; LYNDA VAN ALLSBURG; RICHARD A. DOLBEER

380

Comparison between cartilage and soft tissue ear piercing complications  

Microsoft Academic Search

Purpose: Despite growing interest in cosmetic piercing, a detailed evaluation of associated medical complications is lacking.Materials and Methods: A questionnaire addressing ear, nose, and other body parts piercing was anonymously presented to 1,000 nurses at a major Midwestern teaching hospital, and responses were obtained from 552.Results: One hundred sixty (35%) of the 452 nurses who had an ear pierced reported

Timothy C. Simplot; Henry T. Hoffman

1998-01-01

381

Differential Senescence of Maize Hybrids following Ear Removal 1  

PubMed Central

In conjunction with a study of the effects of ear removal on the senescence of whole maize (Zea mays L.) plants, visual symptoms and associated changes in constituent contents and activities of a selected leaf (first leaf above the ear) were determined. Leaves were sampled from field-grown eared and earless Pioneer brand 3382, B73 × Mo17, and Farm Services brand 854 maize hybrids at nine times during the grainfilling period. Visual symptoms indicated the following sequence and rate of senescence: earless B73 × Mo17 > earless P3382 » eared B73 × Mo17 » eared P3382 ? earless FS854 > eared FS854. All earless hybrids showed increases in leaf dry weight and sugar content; however, the increases were transitory for P3382 and B73 × Mo17, but continuous throughout the grain-filling period for FS854, indicative of continued photosynthetic activity of the latter. All earless hybrids exhibited similar and transitory starch accumulation patterns. Thus, FS854 was an exception to the concept that carbohydrate accumulation accelerates leaf senescence. Ear removal resulted in accelerated losses of reduced N, phosphoenolpyruvate and ribulose bisphosphate carboxylases, phosphorus, chlorophyll, nitrate reductase activity, and moisture for P3382 and B73 × Mo17 plants. In contrast, the loss of all components (except phosphorus) was similar for the selected leaf of earless and eared FS854. Although the loss of nitrate reductase activity, reduced N, and carboxylating enzymes accurately reflected the development of senescence of the selected leaf, the rate of net loss of reduced N and carboxylating enzymes appeared to be regulated. We deduced that the rate of flux of N into the leaf was a factor in regulating the differing rates of senescence observed for the six treatments; however, we cannot rule out the possibility of concurrent influence of growth regulators or other metabolites. Images Fig. 2 PMID:16663424

Crafts-Brandner, Steven J.; Below, Frederick E.; Wittenbach, Vernon A.; Harper, James E.; Hageman, Richard H.

1984-01-01

382

[Common infections of the ear, nose, and throat].  

PubMed

Infectious diseases of the ear, nose and throat are common reasons why patients visit their family physician. Therefore, the aim of this mini-review is to give an overview of the most important and frequent infectious diseases of the ear, nose and throat, the appropriate diagnostic measurements and therapy with relevance for the family physician. Most of these infectious diseases can be treated by the family physician and do not necessarily need to be seen by an ENT-specialist. PMID:25146944

Weber, Claudia Sandra; Kleinjung, Tobias

2014-08-20

383

Computed tomography features of middle ear cholesteatoma in dogs.  

PubMed

We describe the computed tomography (CT) findings in 11 dogs with middle ear cholesteatoma. The cholesteatoma appeared as an expansile tympanic cavity mass with a mean attenuation value of 55.8 +/- 4.2 Hounsfield units. There was no appreciable contrast enhancement of the tympanic bulla contense but ring enhancement was seen in four dogs. Due to the slow progressive growth, the lesion causes severe bone changes at the contour of the tympanic bulla, including osteolysis, osteoproliferation and osteosclerosis, expansion of the tympanic cavity, and sclerosis or osteoproliferation of the ipsilateral temporomandibular joint and paracondylar process. Cholesteatoma can cause lysis of the petrosal part of the temporal bone, leading to intracranial complications. Although not definitive, CT provides useful information for distinguishing a middle ear cholesteatoma from otitis media and neoplasia. In otitis media, enlargement of the tympanic cavity is not routinely observed. In tumors that primarily affect the middle or inner ear, the predominant signs are lysis of the contour of the tympanic bulla or the petrosal part of the temporal bone, soft tissue swelling around the middle ear and marked contrast enhancement. In tumors that arise from the external ear, a soft tissue mass is visible within the external acusticus meatus, and the middle ear is only involved secondarily. PMID:20806867

Travetti, Olga; Giudice, Chiara; Greci, Valentina; Lombardo, Rocco; Mortellaro, Carlo Maria; Di Giancamillo, Mauro

2010-01-01

384

Auditory Brainstem Circuits That Mediate the Middle Ear Muscle Reflex  

PubMed Central

The middle ear muscle (MEM) reflex is one of two major descending systems to the auditory periphery. There are two middle ear muscles (MEMs): the stapedius and the tensor tympani. In man, the stapedius contracts in response to intense low frequency acoustic stimuli, exerting forces perpendicular to the stapes superstructure, increasing middle ear impedance and attenuating the intensity of sound energy reaching the inner ear (cochlea). The tensor tympani is believed to contract in response to self-generated noise (chewing, swallowing) and nonauditory stimuli. The MEM reflex pathways begin with sound presented to the ear. Transduction of sound occurs in the cochlea, resulting in an action potential that is transmitted along the auditory nerve to the cochlear nucleus in the brainstem (the first relay station for all ascending sound information originating in the ear). Unknown interneurons in the ventral cochlear nucleus project either directly or indirectly to MEM motoneurons located elsewhere in the brainstem. Motoneurons provide efferent innervation to the MEMs. Although the ascending and descending limbs of these reflex pathways have been well characterized, the identity of the reflex interneurons is not known, as are the source of modulatory inputs to these pathways. The aim of this article is to (a) provide an overview of MEM reflex anatomy and physiology, (b) present new data on MEM reflex anatomy and physiology from our laboratory and others, and (c) describe the clinical implications of our research. PMID:20870664

Mukerji, Sudeep; Windsor, Alanna Marie; Lee, Daniel J.

2010-01-01

385

Aortic Runoff as a Sign of Intracranial Arteriovenous Malformation: Report of Two Cases  

PubMed Central

Background Intracranial arteriovenous malformation rarely causes pulmonary hypertension and congestive heart failure in the newborn. Its diagnosis is challenging because cardiomegaly may suggest an intra-cardiac structural lesion. Case Presentation We present two newborns, one 2-day-old male and the other 11-day-old female, with intracranial arteriovenous malformation and misdiagnosis of congenital heart disease. Conclusion Precise echocardiography revealed the secondary signs of cranial arteriovenous malformation and had the major role in early diagnosis. PMID:23724190

Moradian, Maryam; Nokhostin-Davari, Paridokht; Merajie, Mahmood; Pouraliakbar, Hamid-Reza

2013-01-01

386

A large pelvic arteriovenous malformation in an adult patient with cystic fibrosis.  

PubMed

We present a prepubertal male cystic fibrosis patient with high circulating oestrogen levels (as a consequence of sever cystic-fibrosis-related hepatobiliary disease) who subsequently developed a large pelvic arteriovenous malformation. This has not previously been described in patients with cystic fibrosis, despite the association between high oestrogen levels and arteriovenous malformations. The aetiology and treatment options of arteriovenous malformations are discussed. PMID:10435171

Ledson, M J; Wahbi, Z; Harris, P; Walshaw, M J

1999-06-01

387

Primary intra osseous venous malformation of nasal bone: A rare case report  

PubMed Central

Primary intra osseous venous malformation with involvement of nasal bone is a rare phenomenon. Nasal bone intraosseous venous malformation on a back ground of port wine stain of face has not been reported in the available literature. We report the very rare case of intraosseous venous malformation of left nasal bone developing on a background of port wine stain of face, its diagnosis, pathology, management and review of literature.

Pati, Ajit Kumar; Nayak, Bibhuti Bhusan; Choudhury, Arun Kumar; Rout, Debesh Kumar

2014-01-01

388

Erythema associated with pain and warmth on face and ears: a variant of erythermalgia or red ear syndrome?  

PubMed Central

Erythermalgia is a rare cutaneous disorder characterized by attacking of erythema, pain and increased temperature, which primarily involves the extremities and may infrequently extend to the neck, face, ears and even the scrotum. We reported an 18-year-old woman who presented with 3 years history of sole involvement of attacking erythema, pain and warmth over her face and ears without any other associations. The frequency and severity of the flares progressed gradually during the course. Cutaneous examination revealed erythema, increased temperature and tenderness on the face and ears during the flare. The symptoms could be relieved rapidly by cooling. Dermatoscope showed that vessels inside the erythema were more dilated during the episode than after application of ice. The lesion is considered a rare variant of erythermalgia with sole involvement of face and ears. The symptoms had mild response to oral antihistamines, topical steroids and tacrolimus, but had excellent response to the combinative therapy of aspirin and paroxetins. PMID:24670221

2014-01-01

389

Major Congenital Malformations in Barbados: The Prevalence, the Pattern, and the Resulting Morbidity and Mortality  

PubMed Central

Objectives. To study the prevalence and the pattern of major congenital malformations and its contribution to the overall perinatal morbidity and mortality. Methods. It is a retrospective population based study. It includes all major congenital malformations in newborns during 1993-2012. The data was collected from the birth register, the neonatal admission register and the individual patient records at the Queen Elizabeth Hospital where over 90% of deliveries take place and it is the only facility for the care of sick newborns in this country. Results. The overall prevalence of major congenital malformations among the live births was 59/10,000 live births and that among the stillbirths was 399/10,000 stillbirths. Circulatory system was the most commonly affected and accounted for 20% of all the major congenital malformations. Individually, Down syndrome (4.1/10, 000 live births) was the commonest major congenital malformation. There was a significant increase in the overall prevalence during the study period. Major congenital malformations were responsible for 14% of all neonatal death. Conclusions. Less than 1% of all live newborns have major congenital malformations with a preponderance of the malformations of the circulatory system. Major congenital malformations contribute significantly to the overall neonatal morbidity and mortality in this country. PMID:25006483

Singh, Keerti; Krishnamurthy, Kandamaran; Greaves, Camille; Kandamaran, Latha; Nielsen, Anders L.; Kumar, Alok

2014-01-01

390

496. Phys. Lett A., 355, N4-5,(2006),247-249. A.G.Ramm, The shape of the ear The shape of the ear canal  

E-print Network

496. Phys. Lett A., 355, N4-5,(2006),247-249. A.G.Ramm, The shape of the ear canal 1 #12;The shape of the ear canal A.G. Ramm Mathematics Department, Kansas State University, Manhattan, KS 66506-2602, USA ramm@math.ksu.edu Abstract It is proved that the measurement of the acoustic pressure on the ear

391

Symptoms of Sleep Disordered Breathing in Children with Craniofacial Malformations  

PubMed Central

Study Objective: The purpose of this study was to investigate the frequency of sleep disordered breathing (SDB) symptoms in a clinical sample of children with congenital craniofacial malformations (CFM) followed at a tertiary medical center and non-selected for sleep problems. Methods: Cross-sectional study of 575 children aged 2-18 years followed at the Craniofacial Anomalies Program between March 2007 and May 2011. The Sleep-Related Breathing Disturbance scale of the Pediatric Sleep Questionnaire was used to screen for SDB, snoring, and sleepiness. A cutoff value ? 0.33 of the total answered questions identified children with positive screening for SDB symptoms. Results: Overall, 25% of children screened positive for SDB, 28% for snoring, and 20% for sleepiness. In children with non-syndromic CFM, those with Robin sequence had the highest frequency of SDB, snoring, and sleepiness (43%, 44%, and 38%, respectively). In children with syndromic CFM, velocardiofacial/ DiGeorge syndrome had the highest frequency of SDB and sleepiness (48% and 43%, respectively). Children with Treacher Collins had the highest frequency of snoring (83%). The presence of cleft palate was not associated with an increased frequency of SDB symptoms. Nevertheless, children with syndromic CFM, compared to those with non-syndromic CFM, had a higher SDB score (0.27 ± 0.21 vs.0.21 ± 0.19, p = 0.003) and were more likely to have sleepiness (26% vs. 18%, p = 0.05). Conclusions: Congenital craniofacial malformations in children are associated with high risk for SDB symptoms. Our findings should encourage a high index of suspicion for SDB in children with CFM, with a low threshold for further testing and close follow-up. Citation: Moraleda-Cibrián M; Edwards SP; Kasten SJ; Berger M; Buchman SR; O'Brien LM. Symptoms of sleep disordered breathing in children with craniofacial malformations. J Clin Sleep Med 2014;10(3):307-312. PMID:24634629

Moraleda-Cibrián, Marta; Edwards, Sean P.; Kasten, Steven J.; Berger, Mary; Buchman, Steven R.; O'Brien, Louise M.

2014-01-01

392

Efficacy of ear-point stimulation on experimentally induced seizure.  

PubMed

This study was to observe the effects of ear-point stimulation on electrocorticogram of sensorimotor cortex and behaviors of rats with penicillin-induced seizure. The model of epilepsy was by injecting penicillin into the hippocampus. One hour later, the lower 1/2 auricular lobules containing ear-points Pizhixia, Shenmen_Zeng and Nao, etc. as humans, or great auricular nerve of seizure rats, were treated twice with electrical stimulation (parameters of stimulation were as follows: electrical current intensity 0.14 approximately 0.2 mA, frequency about 80Hz, 30 min on and 30 min off). The outcome showed that rats appeared epileptic-like electrocorticogram and convulsion behaviors 5 min after injected penicillin. When they were subsequently given the ear-point or great auricular nerve electrical stimulation separately, these epileptic-like electrocorticogram and seizure behaviors were definitely improved. These anti-seizure effects could be enhanced with hour extension of electrical stimulation. If the great auricular nerve of seizure rat was severed before electrical stimulating ear-points, the effects of anti-seizure disappeared. Otherwise, the seizure rats given sham ear-point electrical stimulation (the experimental conditions were same as that of ear-point stimulation other than electric current being no applied) did not show any improvement for epileptic-like electrocorticogram and seizure behaviors. Based on the results above, it was suggested that ear-point electrical stimulation could cause certainly efficacy of anti-seizure, which may be relative with the great auricular nerve. PMID:16231631

Shu, Jia; Liu, Rong-Yu; Huang, Xian-Fen

2005-01-01

393

Bacterial contamination of commercial ear cleaners following routine home use.  

PubMed

Ear cleaning solutions are designed for repeated use, which raises the possibility for bacterial contamination leading to recurrent or persistent infectious otitis. The purpose of this study was to investigate the prevalence of bacterial contamination of commercial ear cleaners following routine home use in dogs and to describe the characteristics that are associated with contamination. Used ear cleaner bottles and information regarding their use were obtained from canine owners visiting veterinary dermatologists. Both the bottle applicator tips and the solution contents were cultured for aerobic bacteria. Bacterial contamination was present on 10% of the bottle tips and in 2% of the solutions. Isolated bacteria included Staphylococcus pseudintermedius, Bacillus spp., coagulase-negative Staphylococcus spp., Micrococcus spp. and Burkholderia cepacia. The contamination rate was significantly higher on the applicator tips than in the solutions (P = 0.0076). The applicator tip contamination rate was significantly higher in expired samples (17%) than in-date samples (4%; P = 0.0277). The bottle sizes were significantly larger for the samples with contaminated applicator tips compared with noncontaminated tips (P = 0.0455). The contamination rate was significantly higher when Tris-EDTA was an ingredient. Cleanliness of the bottle, contact with the ear canal and infection status of the ear at time of culture had no bearing on the contamination rate. In summary, with routine home use of commercial ear cleaners, pathogenic bacterial contamination is of minor concern. This concern may increase when expired products or larger bottles of ear cleaner are used and when Tris-EDTA is an ingredient. PMID:21645141

Bartlett, Sarah J; Rosenkrantz, Wayne S; Sanchez, Susan

2011-12-01

394

Biamnial alpha fetoprotein concentration in twins, one with multiple malformations.  

PubMed Central

In the 18th week of pregnancy in a 22 year old patient, twins were diagnosed by ultrasound. It was found that one twin had an abnormal skull outline and an echo-free area covered by a thin membrane in the region of the abdomen. The second embryo showed no sign of malformation. Amniocentesis was performed and the AFP in both samples of amniotic fluid were in the pathological range. Our own observations with indirect immunofluorescence confirmed that one twin with defects leading to abnormally high AFP levels can cause pathological AFP levels in the amniotic sac of a healthy twin. Images PMID:6209397

Huber, J; Wagenbichler, P; Bartsch, F

1984-01-01

395

Sonohysterography is a useful diagnostic approach for uterine arteriovenous malformation.  

PubMed

Uterine arteriovenous malformation (AVM) is a rare cause of abnormal uterine bleeding; nevertheless, it is a potentially life-threatening condition when the diagnosis is not made. We report a case of uterine AVM with a secondary uterine hematoma diagnosed 2 weeks after curettage due to spontaneous abortion. Ultrasound examination revealed a mixed echogenic mass of approximately 4 cm × 1.5 cm with no blood flow and an additional contiguous heterogeneous mass with turbulent blood flow depicted by color Doppler. Transvaginal sonohysterography enabled us to exclude residual chorionic tissues and to make precise diagnosis of uterine AVM with a secondary hematoma. PMID:24888955

Mishina, Miyuki; Hasegawa, Junichi; Nakamura, Masamitsu; Ichizuka, Kiyotake; Sekizawa, Akihiko; Okai, Takashi

2014-06-01

396

An Unusual Left Upper Quadrant Mass: A Bronchopulmonary Foregut Malformation  

PubMed Central

We report a case of a lady who presented with epigastric discomfort. Physical examination revealed a large left upper quadrant mass filling the left upper quadrant. Following extensive preoperative evaluation, she underwent resection of this 9 × 10 × 11 centimeter mass with en bloc excision of a portion of the left hemidiaphragm. She made an uneventful postoperative recovery. Histopathology revealed a bronchopulmonary foregut malformation with pulmonary sequestration. This developmental anomaly of the foregut typically occurs in the thoracic cavity; however, it can occur below the diaphragm. Herein we report a case and a detailed review of the embryology, clinical features, and management of these extremely rare clinical entities. PMID:23533917

McDermott, R. L.; Kavanagh, D. O.; Bartosik, W.; Quinn, C.; O'Connell, P. R.

2013-01-01

397

Pediatric postcricoid vascular malformation: a diagnostic and treatment challenge.  

PubMed

Hemangioma of the upper aerodigestive tract is a rarely reported occurrence in the pediatric literature. To date, there have been three published case reports of postcricoid hemangiomas contributing to unexplained dysphagia and respiratory distress. We present three children with a history of swallowing difficulty and stridor who were found to have an occult postcricoid mass. Valsalva maneuvers confirmed the suspicion of a vascular malformation in both patients. Transoral laser therapy (KTP and CO2) was used to ablate the lesions. The patients are symptom-free at 5 months, 5 years, and 2 weeks, respectively. The diagnostic challenge in evaluating these children and the therapeutic choices are described. PMID:15961166

Zur, Karen B; Wood, Robert E; Elluru, Ravindhra G

2005-12-01

398

[Anesthetic management of a neonate with congenital cystic adenomatoid malformation].  

PubMed

We report the anesthetic management of a female neonate with congenital cyst adenoid malformation (CCAM) type III of the lung who underwent the lower right lobe resection 22 days after birth. General anesthesia was induced with propofol and rocuronium. The trachea was intubated with a 3.0 standard tube. Anesthesia was maintained with sevoflurane in an air/oxygen mixture and fentanyl. Intraoperative anesthetic course was uneventful except transient desaturation during lung compression. Immediately, the saturation was restored by interruption of lung compression. One lung ventilation was not necessary in this operation. Postoperative course was uneventful. Patient was discharged home on the 28th postoperative day. PMID:24558943

Tominaga, Hiroki; Hatori, Eiki; Takeda, Junzo; Morisaki, Hiroshi

2014-01-01

399

[Some features of the nose in craniofacial malformations].  

PubMed

In craniofacial malformations, the nose is variably affected: in its location, its shape or by lack of development. In this short chapter, some of the common problems encountered by the specialized teams are summarized. Craniofacial astronomies can modify the skeleton of the nose during growth, sometime at an early age. However, most rhinoplasties are performed at adulthood. The nasal pyramid may present deformations that produce functional and aesthetics impairment that should be treated when necessary. Respiratory problems should be recognized as early as possible and treated in priority. PMID:25303936

Arnaud, E

2014-12-01

400

Management of antenatally diagnosed pulmonary sequestration associated with congenital cystic adenomatoid malformation  

PubMed Central

BACKGROUND—Sequestration with associated cystic adenomatoid malformation is rare. A study was undertaken to determine whether pulmonary sequestration associated with congenital cystic adenomatoid malformation has a more favourable natural history than that of sequestration without associated cystic adenomatoid malformation.?METHODS—An outline of the postnatal work up leading to the management of extralobar or intralobar pulmonary sequestration with congenital cystic adenomatoid malformation diagnosed antenatally as pulmonary malformation is presented and the indications for surgical intervention are discussed.?RESULTS—In five infants in whom an antenatal ultrasound scan had detected a congenital lung malformation at 18-19 weeks gestation a final diagnosis of extralobar or intralobar pulmonary sequestration with congenital cystic adenomatoid malformation was made postnatally. Postnatal ultrasound and computerised axial tomographic scans confirmed the diagnosis of sequestration by delineating anomalous vascular supply. Cystic changes were also observed in the basal area of the sequestration in all patients. Four children remained asymptomatic and one infant presented at 10 months of age with pneumonia. The mean age at surgical resection was 6.8 months (range 2-10). Histopathological examination confirmed intralobar pulmonary sequestration with associated Stocker type 2 congenital cystic adenomatoid malformation in two patients and extralobar pulmonary sequestration with associated Stocker type 2 congenital cystic adenomatoid malformation in three patients. The mean period of follow up was four years (range 1-8). The children remain well and are developing normally.?CONCLUSIONS—The importance of seeking an anomalous blood supply in children with congenital lung lesions is emphasised. Pulmonary sequestration and congenital cystic adenomatoid malformation probably share a common embryogenesis despite diverse morphology. The natural history of antenatally diagnosed lung masses is variable. Early postnatal surgical resection of pulmonary sequestration with cystic adenomatoid malformation is recommended. Surgical excision should be conservative, sparing the normal lung parenchyma.?? PMID:10413723

Samuel, M.; Burge, D.

1999-01-01

401

Bovine Cartilage: A Near Perfect Training Tool for Carving Ear Cartilage Framework.  

PubMed

Objective :? To develop a training module for carving ear cartilage. Setting :? Designing the ear framework is one of the most challenging surgical steps during ear reconstruction in microtia and acquired deformities of the ear. Trainees do not get an opportunity to carve ear cartilage during their training period. Material :? Ox scapular cartilage was retrieved from a manual slaughterhouse. It was transported in a 4°C ice chamber. This was used as a training material for carving the ear framework. Each scapular cartilage was adequate for two to three ear frameworks. Results :? Twenty-two trainees used the bovine cartilage in a wet lab. All had positive feedback on their ear framework carving experience. In their opinion, the consistency, flexibility, and cutting experience almost matched that of human costal cartilage. Conclusion :? The ox scapular cartilage has been found to be a near perfect material for training and practicing carving of the ear cartilage. PMID:25372551

Agrawal, Karoon

2014-11-01

402

Molecular Conservation and Novelties in Vertebrate Ear Development  

PubMed Central

Evolution shaped the vertebrate ear into a complicated three-dimensional structure and positioned the sensory epithelia so that they can extract specific aspects of mechanical stimuli to govern vestibular and hearing-related responses of the whole organism. This information is conducted from the ear via specific neuronal connections to distinct areas of the hindbrain for proper processing. During development, the otic placode, a simple sheet of epidermal cells, transforms into a complicated system of ducts and recesses. This placode also generates the mechanoelectrical transducers, the hair cells, and sensory neurons of the vestibular and cochlear (spiral) ganglia of the ear. We argue that ear development can be broken down into dynamic processes that use a number of known and unknown genes to govern the formation of the three-dimensional labyrinth in an interactive fashion. Embedded in this process, but in large part independent of it, is an evolutionary conserved process that induces early the development of the neurosensory component of the ear. We present molecular data suggesting that this later process is, in its basic aspects, related to the mechanosensory cell formation across phyla and is extremely conserved at the molecular level. We suggest that sensory neuron development and maintenance are vertebrate or possibly chordate novelties and present the molecular data to support this notion. PMID:14674476

Fritzsch, B.; Beisel, K. W.

2014-01-01

403

Manganese Accumulation in the Mouse Ear Following Systemic Exposure  

PubMed Central

There is evidence in human populations that exposure to manganese (Mn), or Mn in combination with excessive noise exposure, results in hearing loss. Quantitative reverse-transcriptase polymerase chain reaction revealed expression of the metal transporters DMT1, ZIP8, and ZIP14 in control mouse ears. ZIP8 is known to have a high affinity (Km = 2.2 ?M) for Mn transport, and ZIP8 protein was localized to the blood vessels of the ear by immunohistochemistry. We treated mice (strains C57BL/6J and DBA/2J) with Mn (100 mg/kg MnCl2, by subcutaneous injection, on three alternating days), and Mn was significantly elevated in the ears of the treated mice. Mn concentrations remained elevated over controls for at least 2 weeks after treatment. These studies demonstrate that metal transporters are present in the mouse ear and that Mn can accumulate in the ear following systemic exposure. Future studies should focus on whether Mn exposure is associated with hearing deficits. PMID:18972394

Ma, Ci; Schneider, Scott N.; Miller, Marian; Nebert, Daniel W.; Lind, Caroline; Roda, Sandy M.; Afton, Scott E.; Caruso, Joseph A.; Genter, Mary Beth

2009-01-01

404

Anomalous Brownian motion discloses viscoelasticity in the ear’s mechanoelectrical-transduction apparatus  

PubMed Central

The ear detects sounds so faint that they produce only atomic-scale displacements in the mechanoelectrical transducer, yet thermal noise causes fluctuations larger by an order of magnitude. Explaining how hearing can operate when the magnitude of the noise greatly exceeds that of the signal requires an understanding both of the transducer’s micromechanics and of the associated noise. Using microrheology, we characterize the statistics of this noise; exploiting the fluctuation-dissipation theorem, we determine the associated micromechanics. The statistics reveal unusual Brownian motion in which the mean square displacement increases as a fractional power of time, indicating that the mechanisms governing energy dissipation are related to those of energy storage. This anomalous scaling contradicts the canonical model of mechanoelectrical transduction, but the results can be explained if the micromechanics incorporates viscoelasticity, a salient characteristic of biopolymers. We amend the canonical model and demonstrate several consequences of viscoelasticity for sensory coding. PMID:22328158

Kozlov, Andrei S.; Andor-Ardó, Daniel; Hudspeth, A. J.

2012-01-01

405

Model organisms inform the search for the genes and developmental pathology underlying malformations of the human hindbrain  

PubMed Central

Congenital malformations the human hindbrain, including the cerebellum, are poorly understood largely because their recognition is a relatively recent advance for imaging diagnostics. Cerebellar malformations are the most obvious and best characterized hindbrain malformations due to their relative ease to view by MRI and the recent identification of several causative genes1. Malformations of the pons and medulla have also been described both in isolation and in association with cerebellar malformations2. Although little is understood regarding the specific developmental pathologies underlying hindbrain malformations in humans, much is known regarding the mechanisms and genes driving hindbrain development in vertebrate model organisms. Thus, studies in vertebrate models provide a developmental framework in which to categorize human hindbrain malformations and serve to inform our thinking regarding disrupted developmental processes and candidate genes. Here we survey the basic principles of vertebrate hindbrain development and integrate our current knowledge of human hindbrain malformations into this framework. PMID:19778712

Aldinger, Kimberly A.; Elsen, Gina E.; Prince, Victoria E.; Millen, Kathleen J.

2009-01-01

406

COMPARING THE EFFECTS OF RETINOIC ACID ON AMPHIBIAN LIMB DEVELOPMENT AND LETHALITY: CHRONIC EXPOSURE RESULTS IN LETHALITY NOT LIMB MALFORMATIONS  

EPA Science Inventory

Recently, high frequencies of malformations have been reported in amphibians across the United States. It has been suggested that the malformations may be the result of xenobiotic disruption of retinoid signaling pathways during embryogenesis and tadpole development. Therefore, a...

407

ADDING INFECTION TO INJURY: SYNERGISTIC EFFECTS OF PREDATION AND PARASITISM ON AMPHIBIAN MALFORMATIONS  

Microsoft Academic Search

We explored the importance of interactions between parasite infection and predation in driving an emerging phenomenon of conservation importance: amphibian limb malformations. We suggest that injury resulting from intraspecific predation in combination with trematode infection contributes to the frequency and severity of malformations in salamanders. By integrating field surveys and experiments, we evaluated the individual and combined effects of conspecific

Pieter T. J. Johnson; Eric R. Preu; Daniel R. Sutherland; John M. Romansic; Barbara Han; Andrew R. Blaustein

2006-01-01

408

Presentation and Management of Hydromyelia in Children with Chiari Type-II Malformation  

Microsoft Academic Search

Hydromyelia in patients with myelomeningocele and Chiari-II malformation is a relatively frequent finding on MRI studies. However, not all children develop symptoms from the hydromyelia that requires treatment. Furthermore, treatment of hydromyelia in spina bifida patients is rather complex due to the associated malformations. The authors retrospectively analyzed 231 MRI studies carried out on spina bifida patients who presented neurological

Frank La Marca; Martin Herman; John A. Grant; David G. McLone

1997-01-01

409

Venous malformations in blue rubber bleb nevus syndrome: variable onset of presentation  

Microsoft Academic Search

Blue rubber bleb nevus syndrome (BRBNS) is a rare disorder characterized by discrete venous malformations of varying size and appearance that are present on the skin and within the gastrointestinal tract. The characteristic cutaneous lesions consist of deep-blue, soft, rubbery blebs, which are easily compressible. A serious complication is gastrointestinal bleeding. Because venous malformations were described historically as cavernous hemangiomas,

Walter K Nahm; Sharon Moise; Lawrence F Eichenfield; Amy S Paller; Laura Nathanson; Denise M Malicki; Sheila Fallon Friedlander

2004-01-01

410

MALFORMATION, MORTALITY AND PARASITES IN NORTHERN LEOPARD FROGS IN MN AND ND.  

Technology Transfer Automated Retrieval System (TEKTRAN)

In 2000 a field study compared the relative incidence of mortality and malformation of developing northern leopard frogs (Rana pipiens) at two Minnesota (MN)lake sites, one site with history of high incidence of malformations (CWB)and one with low incidence (BUT). Tadpoles were reared in enclosures ...

411

MALFORMATION VERSUS MORTALITY, A STUDY OF NORTHERN LEOPARD FROG DEVELOPMENT IN SITU.  

Technology Transfer Automated Retrieval System (TEKTRAN)

Field studies were designed to compare the relative incidence of mortality and malformation of developing northern leopard frogs (Rana pipiens) at two Minnesota (MN)Lake sites. Site selection was based on survey data that indicated one site had a high incidence of malformations (CWB) versus low inci...

412

Previously apparently undescribed syndrome: Shallow orbits, ptosis, coloboma, trigonocephaly, gyral malformations, and mental and growth retardation  

SciTech Connect

We describe 2 children with severe ptosis, trigonocephaly, broad nasal bridge, and major brain malformation. A total of 8 children have been reported who share most of these findings. Two of the individuals have had identical pericentric inversions involving chromosome 2p12-q14. These cases appear to represent a unique malformation syndrome. 14 refs., 6 figs., 1 tab.

Ramer, J.C.; Ladda, R.L. [Pennsylvania State Univ. Hospital, Hershey, PA (United States); Lin, A.E. [Francisco Children`s Hospital, Boston, MA (United States)] [and others

1995-07-03

413

The cyclops and the mermaid: an epidemiological study of two types of rare malformation  

Microsoft Academic Search

Infants with cyclopia or sirenomelia are born at an approximate rate of 1 in 100,000 births. Eight malformation monitoring systems around the world jointly studied the epidemiology of these rare malformations: 102 infants with cyclopia, 96 with sirenomelia, and one with both conditions were identified among nearly 10.1 million births. Maternal age is somewhat increased for cyclopia, indicating the likely

B Källén; E E Castilla; P A Lancaster; O Mutchinick; L B Knudsen; M L Martínez-Frías; P Mastroiacovo; E Robert

1992-01-01

414

Uterine arteriovenous malformation successfully embolized with a liquid polymer, isobutyl 2-cyanoacrylate.  

PubMed

A uterine arteriovenous malformation was diagnosed angiographically in a 27-year-old woman presenting with recurrent menometrorrhagia. Bilateral hypogastric artery embolization with a liquid polymer, isobutyl 2-cyanoacrylate (Bucrylate), resulted in subsequent normal menses. Bucrylate offers a number of advantages over other agents previously used to embolize uterine arteriovenous malformations. PMID:3752185

Markoff, G; Quagliarello, J; Rosen, R J; Beckman, E M

1986-09-01

415

Embolization of intracranial arteriovenous malformations and fistulas with polyvinyl alcohol particles and platinum fibre coils  

Microsoft Academic Search

In order to reduce the recanalization rate of arteriovenous malformations and multiple dural arteriovenous fistulas, embolization was carried out with polyvinyl alcohol (PVA) particles combined with platinum fibre coils in 20 patients. The malformation was occluded more effectively than by PVA alone. Distal deposition of the emboli was obtained by improved steerable catheters (Tracker-18-unibody) and guidewires. The complication rate was

P. H. Nakstad; S. J. Bakke; J. K. Hald

1992-01-01

416

Mediastinal venous vascular malformations: report of two cases, with discussion of imaging findings and classification systems.  

PubMed

Mediastinal vascular malformations are interesting group of disorders, which are rarely seen in clinical practice. In this case report, we discuss two cases of venous vascular malformations in the anterior and posterior mediastinum. Also, we discuss the findings on cross-sectional imaging and review the classification schemes of these lesions. PMID:24393540

Robert, Andres; Raymond, Daniel; Bolen, Michael; Renapurkar, Rahul

2014-01-01

417

Congenital malformations after the use of inhaled budesonide in early pregnancy  

Microsoft Academic Search

Objective: To study possible teratogenic risks with the use of an inhaled glucocorticoid, budesonide, in early pregnancy.Methods: Using the Swedish Medical Birth Registry, congenital malformations were studied in 2014 infants whose mothers had used inhaled budesonide for asthma in early pregnancy. The presence of congenital malformations was checked further with auxilliary registries.Results: No increase in the general rate of congenital

Bengt Källén; Hakan Rydhstroem; Anders Åberg

1999-01-01

418

The diagnostic yield of rescreening for arteriovenous malformations in children with hereditary hemorrhagic telangiectasia.  

PubMed

The diagnostic yield of rescreening children with hereditary hemorrhagic telangiectasia at regular intervals for arteriovenous malformations is unclear. Here, we show that when children with initially negative screening were reassessed after 5 years, no new arteriovenous malformations were detected suggesting that longer intervals between screenings may be adequate. PMID:24797954

Latino, Giuseppe A; Al-Saleh, Suhail; Carpenter, Susan; Ratjen, Felix

2014-07-01

419

Chiari malformation type I: a neuropsychological case study.  

PubMed

Chiari Malformation type I (CM-I) is a congenital anomaly that occurs when the cerebellar tonsils protrude through the foramen magnum. Individuals with this condition are typically asymptomatic and the identification of the malformation is usually an incidental finding during the course of treating another disorder. This case study explores the neuropsychological sequelae of two unique cases of CM-I in combat veterans. Neuropsychological evaluation revealed that both patients demonstrated preserved general cognitive functioning but had varying patterns of performance on measures of visuospatial, executive functioning, and processing speed. In terms of affective functioning, both endorsed significant depressive symptomatology, but had varying patterns of severity on other estimates of psychiatric symptomatology. Overall, results suggest that there is not one pattern of cognitive and affective functioning associated with CM-I and that environmental and psychological rather than neurological factors should be considered when evaluating cognitive and affective functioning. These cases also highlight the need for neuropsychological evaluation in CM-I in terms of providing guidance for psychoeducation and psychotherapy. PMID:24902143

Klein, Robert; Hopewell, C Alan; Oien, Michael

2014-06-01

420

Somatic Uniparental Isodisomy Explains Multifocality of Glomuvenous Malformations  

PubMed Central

Inherited vascular malformations are commonly autosomal dominantly inherited with high, but incomplete, penetrance; they often present as multiple lesions. We hypothesized that Knudson’s two-hit model could explain this multifocality and partial penetrance. We performed a systematic analysis of inherited glomuvenous malformations (GVMs) by using multiple approaches, including a sensitive allele-specific pairwise SNP-chip method. Overall, we identified 16 somatic mutations, most of which were not intragenic but were cases of acquired uniparental isodisomy (aUPID) involving chromosome 1p. The breakpoint of each aUPID is located in an A- and T-rich, high-DNA-flexibility region (1p13.1–1p12). This region corresponds to a possible new fragile site. Occurrences of these mutations render the inherited glomulin variant in 1p22.1 homozygous in the affected tissues without loss of genetic material. This finding demonstrates that a double hit is needed to trigger formation of a GVM. It also suggests that somatic UPID, only detectable by sensitive pairwise analysis in heterogeneous tissues, might be a common phenomenon in human cells. Thus, aUPID might play a role in the pathogenesis of various nonmalignant disorders and might explain local impaired function and/or clinical variability. Furthermore, these data suggest that pairwise analysis of blood and tissue, even on heterogeneous tissue, can be used for localizing double-hit mutations in disease-causing genes. PMID:23375657

Amyere, Mustapha; Aerts, Virginie; Brouillard, Pascal; McIntyre, Brendan A.S.; Duhoux, François P.; Wassef, Michel; Enjolras, Odile; Mulliken, John B.; Devuyst, Olivier; Antoine-Poirel, Hélène; Boon, Laurence M.; Vikkula, Miikka

2013-01-01

421

Cardiovascular malformations and organic solvent exposure during pregnancy in Finland  

SciTech Connect

In order to investigate the possible association between cardiovascular malformations and maternal exposure to organic solvents during the first trimester of pregnancy, 569 cases and 1,052 controls were retrospectively studied. The cases represented all infants with diagnosed cardiovascular malformations born in Finland in 1982-1984, and the controls were randomly selected from all normal births in the country during the same period. All mothers were interviewed approximately 3 months after delivery by a midwife using a structured questionnaire. Exposures to organic solvents at work during the first trimester of pregnancy were slightly more prevalent among the mothers of affected infants (10.4%) than among those of controls (7.8%). Logistic regression analysis of exposure to organic solvents showed an adjusted relative odds ratio of 1.3 (95% confidence interval, 0.8-2.2). In the analysis of ventricular septal defect, exposure to organic solvents showed an adjusted relative odds ratio of 1.5 (95% confidence interval, 1.0-3.7).

Tikkanen, J.; Heinonen, O.P.

1988-01-01

422

Antenatal and postnatal management of congenital cystic adenomatoid malformation.  

PubMed

Congenital thoracic malformations (CTMs) are a heterogeneous group of rare disorders that may involve the airways or lung parenchyma. The authors have focused on the condition that causes the most controversy, namely, congenital cystic adenomatoid malformation (CCAM). The reported incidence is 3.5 and 0.94 per 10,000 live births for CTMs and CCAMs respectively. Ultrasound is the antenatal imaging modality of choice for screening for CCAMs whilst magnetic resonance imaging is complimentary for morphological and volumetric evaluation of the foetal lung. Most CCAMs are detected antenatally with only a small proportion presenting postnatally. Only a few CCAMs cause foetal problems, with foetal hydrops being the best predictor of death. Although many CCAMs regress during pregnancy, most remain detectable postnatally by CT scans. Surgical excision of symptomatic lesions is relatively straightforward, but management of asymptomatic lesions is controversial. Some surgeons adopt a "wait and see" approach operating only on those patients who develop symptoms, but others operate on asymptomatic patients usually within the first year of life. Due to the potential of malignant transformation, children should have long term follow up. There is an urgent need to delineate the natural history of antenatally detected CCAMs to guide future management. PMID:22726873

Kotecha, S; Barbato, A; Bush, A; Claus, F; Davenport, M; Delacourt, C; Deprest, J; Eber, E; Frenckner, B; Greenough, A; Nicholson, A G; Antón-Pacheco, J L; Midulla, F

2012-09-01

423

Chronic Encapsulated Intracerebral Hematoma Associated with Cavernous Malformation  

PubMed Central

Chronic encapsulated intracerebral hematoma (CEIH) is a rare cerebrovascular disease that behaves as a slowly expanding lesion with a gradual onset. It is well established that CEIH is associated with arteriovenous malformations; however, CEIH associated with cavernous malformation (CM) is extremely rare. We herein report a case of CEIH associated with CM, and discuss its pathogenesis. A 12-year-old female was admitted to our hospital because of a one week history of progressive headache and nausea. Brain computed tomography scan and magnetic resonance imaging showed an intracerebral hematoma surrounded by edema in the right frontal lobe. One week later, her headache and nausea worsened, and a brain computed tomography scan revealed the enlargement of hematoma. A right frontal craniotomy was performed. The capsule, mass, and hematoma were totally removed. Histological examination confirmed the diagnosis of CEIH associated with CM. Immunohistochemical analysis revealed increased expression of vascular endothelial growth factor (VEGF) and the VEGF receptor-1 in the endothelium and fibroblasts. Our findings suggest that the activated VEGF pathway might have positively contributed to development of CEIH in the present patient. PMID:24653802

Wada, Kojiro; Sakakibara, Fumihiro; Mori, Kentaro

2014-01-01

424

A tortuous proximal urethra in urorectal septum malformation sequence?  

PubMed

We observed a newborn boy with urorectal septum malformation sequence. Anomalies of the genitalia and rectum were present. He expired on the first day of life, due to severe lung hypoplasia. Autopsy showed a colon that ended in a blind sac, an enlarged bladder with no grossly visible urethra, and dysplastic kidneys. A cone-shaped tissue at the usual site of the bladder outlet contained tortuous and slit-like lumina, suggesting an undeveloped proximal urethra. The urethral structure was lined by transitional epithelium with squamous metaplasia. Many small buds-lined with columnar epithelium-branched from the urethral structure. These ductal buds lined with columnar epithelium stained for prostatic acid phosphatase. Basal cells surrounding the ductal buds stained for p63 and high molecular weight cytokeratin-supporting an interpretation that the buds were early prostatic ducts with normal histology. To our knowledge, these are the first histological images of an undeveloped, obstructed urethra associated with the urorectal septum malformation sequence. PMID:24665006

Lin, Henry J; Lugo, Hector; Tran, Thu; Tovar, Jason P; Corral, Julia; Zork, Noelia M; Smith, Lynne M; French, Samuel W; Barajas, Luciano

2014-05-01

425

Pituitary gland and axial skeletal malformations in human fetuses with spina bifida.  

PubMed

The purpose of the present study was to describe the pituitary gland and axial skeleton, including the sella turcica, in human fetuses with spina bifida. Ten fetuses with gestational ages (GA) 15 1/2-28 weeks were investigated radiographically (Faxitron X-ray apparatus) and immunohistochemically. Four of the fetuses have been described previously. The study showed that nine fetuses had minor or no skeletal abnormalities in the vertebral bodies of the spine, and one fetus had severely malformed vertebral bodies. In all cases the sella turcica and the pituitary gland were malformed. Adenopituitary tissue was in all cases located in both the sella turcica and the pharyngeal submucosa. The most severe sella turcica/pituitary gland malformation was seen in the fetus with the malformed spine. The connection between the prenatally registered sella turcica/pituitary gland malformation and the endocrinological status of children with spina bifida should be emphasized in future studies. PMID:10661842

Kjaer, I; Fischer Hansen, B; Reintoft, I; Keeling, J W

1999-12-01

426

Human Action Recognition Using Wireless Wearable In-Ear Microphone  

NASA Astrophysics Data System (ADS)

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.

Nishimura, Jun; Kuroda, Tadahiro

427

Sensory Cells of the Fish Ear: A Hairy Enigma  

NASA Technical Reports Server (NTRS)

Analysis of the structure of the ears in teleost fishes has led to the tentative suggestion that otolithic endorgans may function differently, in different species. Recently, evidence has demonstrated different 'types' of sensory hair cells can be found in the ears of teleost fishes, and individual hair cell types are found in discrete regions of individual sensory, epithelia. The presence of multiple hair cell types in fishes provides strong support to the hypothesis of regional differences in the responses of individual otolithic sensory epithelia. The finding of hair cell types in fishes that closely resemble those found in amniote vestibular endorgans also suggests that hair cell heterogeneity arose earlier in the evolution of the vertebrate ear than previously thought.

Popper, A. N.; Saidel, W. M.

1995-01-01

428

Magnetically driven middle ear ossicles for optical measurement of vibrations in an ear with opened tympanic membrane.  

PubMed

Vibrations of the middle ear ossicles are easily measured by means of laser vibrometry. However, laser vibrometry requires free visual access to the object under investigation, and acquiring free visual access to the ossicles through the ear canal requires the removal of the tympanic membrane (TM), with the result that the ossicles can no longer be stimulated acoustically. To overcome this, we devised a new setup in which the ossicles can be driven magnetically. After measuring the response of the TM to an acoustic signal, we then remove it and attach a small magnet to the exposed manubrium (a part of the most lateral auditory ossicle, the malleus, which is normally attached to the TM). An electromagnetic excitation coil is then used to drive the magnet, and the output to the coil adjusted until the vibration of the manubrium, as measured by the vibrometer, matches that measured in response to the acoustic signal. Such a setup may have uses in research on middle ear mechanics, such as the measurement of nonlinearities in their response, as well as applications in the diagnosis of middle ear conditions such as the fixation of the ossicles by otosclerosis or in chronic otitis media. We describe our setup and discuss the viability of our method and its future clinical potential by presenting some measurements on an artificially fixated ear. PMID:24387412

Peacock, J; von Unge, M; Dirckx, J

2013-12-01

429

Magnetically driven middle ear ossicles for optical measurement of vibrations in an ear with opened tympanic membrane  

NASA Astrophysics Data System (ADS)

Vibrations of the middle ear ossicles are easily measured by means of laser vibrometry. However, laser vibrometry requires free visual access to the object under investigation, and acquiring free visual access to the ossicles through the ear canal requires the removal of the tympanic membrane (TM), with the result that the ossicles can no longer be stimulated acoustically. To overcome this, we devised a new setup in which the ossicles can be driven magnetically. After measuring the response of the TM to an acoustic signal, we then remove it and attach a small magnet to the exposed manubrium (a part of the most lateral auditory ossicle, the malleus, which is normally attached to the TM). An electromagnetic excitation coil is then used to drive the magnet, and the output to the coil adjusted until the vibration of the manubrium, as measured by the vibrometer, matches that measured in response to the acoustic signal. Such a setup may have uses in research on middle ear mechanics, such as the measurement of nonlinearities in their response, as well as applications in the diagnosis of middle ear conditions such as the fixation of the ossicles by otosclerosis or in chronic otitis media. We describe our setup and discuss the viability of our method and its future clinical potential by presenting some measurements on an artificially fixated ear.

Peacock, J.; von Unge, M.; Dirckx, J.

2013-12-01

430

Ion Channel Gene Expression in the Inner Ear  

PubMed Central

The ion channel genome is still being defined despite numerous publications on the subject. The ion channel transcriptome is even more difficult to assess. Using high-throughput computational tools, we surveyed all available inner ear cDNA libraries to identify genes coding for ion channels. We mapped over 100,000 expressed sequence tags (ESTs) derived from human cochlea, mouse organ of Corti, mouse and zebrafish inner ear, and rat vestibular end organs to Homo sapiens, Mus musculus, Danio rerio, and Rattus norvegicus genomes. A survey of EST data alone reveals that at least a third of the ion channel genome is expressed in the inner ear, with highest expression occurring in hair cell-enriched mouse organ of Corti and rat vestibule. Our data and comparisons with other experimental techniques that measure gene expression show that every method has its limitations and does not per se provide a complete coverage of the inner ear ion channelome. In addition, the data show that most genes produce alternative transcripts with the same spectrum across multiple organisms, no ion channel gene variants are unique to the inner ear, and many splice variants have yet to be annotated. Our high-throughput approach offers a qualitative computational and experimental analysis of ion channel genes in inner ear cDNA collections. A lack of data and incomplete gene annotations prevent both rigorous statistical analyses and comparisons of entire ion channelomes derived from different tissues and organisms. Electronic supplementary material The online version of this article (doi:10.1007/s10162-007-0082-y) contains supplementary material, which is available to authorized users. PMID:17541769

Sokolowski, Bernd H.A.; Morton, Cynthia C.; Giersch, Anne B.S.

2007-01-01

431

“Turkey Ear” as a Cutaneous Maniestation of Tuberculosis  

PubMed Central

Lupus vulgaris is the most common morphological variant of cutaneous tuberculosis. Classical lupus lesions are often seen in the head and neck region. Turkey ear is a clinically descriptive term, previously being used for the earlobe with reddish indurated plaque lesions, which recently can be a sign for lupus vulgaris. A 65-year-old man presented with lupus vulgaris of the earlobe. The diagnosis was confirmed by conventional laboratory investigations and the patient showed well response to antituberculous therapy. This is the second reported case of “turkey ear” as a manifestation of cutaneous tuberculosis. PMID:23248385

Küçükünal, Asl?; Ekmekçi, Tu?ba R; Sak?z, Damlanur

2012-01-01

432

Inner ear supporting cells: Rethinking the silent majority  

PubMed Central

Sensory epithelia of the inner ear contain two major cell types: hair cells and supporting cells. It has been clear for a long time that hair cells play critical roles in mechanoreception and synaptic transmission. In contrast, until recently the more abundant supporting cells were viewed primarily as serving primarily structural and homeostatic functions. In this review we discuss the growing information about the roles that supporting cells play in the development, function and maintenance of the inner ear, their activities in pathological states, their potential for hair cell regeneration, and the mechanisms underlying these processes. PMID:23545368

Wan, Guoqiang; Corfas, Gabriel; Stone, Jennifer S

2014-01-01

433

Merkel cell carcinoma arising in the ear canal.  

PubMed

A case of rare tumor, Merkel cell carcinoma, located in the ear canal of a 25-year-old woman is presented. A polypoid tumor mass was extirpated, and tympanoplasty was done at the first operation, whereas at the second operation, all the bones of the ear canal were removed. Epitympanum and cavum were filled with tumor, and the tumor mass was removed in toto. The histopathology and immunohistochemical staining characteristics of tumor confirmed the presence of Merkel cell tumor. Postoperatively, radiation therapy to the tumor bed was completed. There was no clinical or radiographic evidence of recurrence or metastasis of Merkel cell tumor for 3 years. PMID:18387993

Petkovi?, Marija; Krstulja, Mira; Radic, Jelena; Zamolo, Gordana; Muhvi?, Damir; Lovasic, Ingrid; Kujundzic, Milodar; Franko, Artur

2008-07-01

434

Energy localization and frequency analysis in the locust ear  

PubMed Central

Animal ears are exquisitely adapted to capture sound energy and perform signal analysis. Studying the ear of the locust, we show how frequency signal analysis can be performed solely by using the structural features of the tympanum. Incident sound waves generate mechanical vibrational waves that travel across the tympanum. These waves shoal in a tsunami-like fashion, resulting in energy localization that focuses vibrations onto the mechanosensory neurons in a frequency-dependent manner. Using finite element analysis, we demonstrate that two mechanical properties of the locust tympanum, distributed thickness and tension, are necessary and sufficient to generate frequency-dependent energy localization. PMID:24196693

Malkin, Robert; McDonagh, Thomas R.; Mhatre, Natasha; Scott, Thomas S.; Robert, Daniel

2014-01-01

435

To Have an Ear: Music and the Otological Experience.  

PubMed

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

Sattar, Atia

2014-07-01

436

Short Communication Functional ear (a)symmetry in brainstem neural activity relevant to encoding  

E-print Network

Short Communication Functional ear (a)symmetry in brainstem neural activity relevant to encoding Keywords: Auditory Human Brainstem Pitch Language Mandarin Chinese Fundamental frequency-following response areas. This experiment investigates whether ear asymmetries vary in brainstem representation of pitch

Dasgupta, Dipankar

437

Coupler-related real ear gain. Interaction between subject and hearing aid type.  

PubMed

In order to select hearing aids with a highly predictable real ear gain, in situ gain and compliance through the individual ear mould were measured on 8 normal-hearing subjects. The latter were chosen in order to reduce gain variation caused by various pathological conditions of the ear. All behind-the-ear aids commonly used in Sweden and one in-the-ear aid were studied. One sample of each hearing aid type was tested and the subjects had identical ear moulds. Hence, the variables were hearing aid type and subject. The study showed wide intra-subject coupler-related real ear gain variation (30 dB). Most of the aids showed a distinct correlation between coupler-related overall gain, and mould compliance. Some of the aids gave more uniform real ear gain, i.e. low interaction between subject and frequency-dependent gain. These results are elucidated by measuring the aid's acoustic output impedance. PMID:1585125

Berninger, E; Ovegård, A; Svärd, I

1992-01-01

438

Reliability of real ear insertion gain in behind-the-ear hearing aids with different coupling systems to the ear canal  

PubMed Central

Objective The last decade has offered a multitude of instant fit coupling systems to be fitted with behind-the-ear (BTE) hearing aids. The impact of these designs on the reliability of real ear measurements (REMs) has not been reported. The purpose of this study was to obtain REM reliability data for instant fit coupling systems. Design REM reliability data was obtained for four different instant-fit coupling systems and for standard size 13 tubing and custom earmolds. REMs were performed for all five coupling systems two times and by two examiners. Study sample Ten normal-hearing individuals (20 ears) served as participants. Results The REM test-retest reliability is high for the four instant fit coupling systems as well as for the custom earmolds. The REM inter-examiner reliability is high for three of the four instant fit coupling systems. Conclusions Carrying out REMs with instant fit coupling systems appears to be fundamentally no different than performing REMs with conventional hearing aids. For either, care should be taken in probe tube placement in terms of insertion depth and maintaining the probe tube placement, and other best practices regarding test environment and test setup should be observed. PMID:23301659

2013-01-01

439

Transcatheter brachial fistula creation for treatment of pulmonary arteriovenous malformations.  

PubMed

Pulmonary arteriovenous malformations (PAVMs) are thought to form as a result of exclusion of hepatic venous blood from part of the pulmonary circulation. Surgical arteriovenous (AV) fistula creation has demonstrated therapeutic potential to reverse PAVM formation. We sought to demonstrate the feasibility and safety of transcatheter AV fistula creation for this indication. Fluoroscopically guided puncture from the basilic vein into the brachial artery using a Brockenbrough needle and sharpened 0.014" wire created a tract between these vessels. After balloon dilation of the tract, a covered stent was deployed, resulting in a functioning brachial AV fistula. The procedure was technically successful, with no clinical complications at 4 months follow-up. Repeat diagnostic catheterization revealed marked improvement in systemic saturation and near-resolution of PAVMs in the pilot patient. This report suggests that transcatheter brachial arteriovenous fistula formation is technically feasible, and may be effective in managing PAVMs in select single-ventricle patients. PMID:23765690

Esch, Jesse J; Marshall, Audrey C; Porras, Diego

2014-04-01

440

Amphibian ocular malformation associated with frog virus 3.  

PubMed

During an on-going amphibian ecology study, a free-ranging American bullfrog (Rana catesbeiana) metamorph was captured in a pitfall trap adjacent to a constructed farm pond at the Plateau Research and Education Center (PREC) on the Cumberland Plateau near Crossville, Tennessee, USA. Grossly, the right eye was approximately 50% the size of the left. Stereo and light microscopic examination revealed two granulomas within the orbit. Electron microscopic examination revealed virus particles scattered throughout one structure but mostly aggregated toward the center. Subsequent PCR and sequencing (GenBank accession Number EF175670) confirmed frog virus 3 (FV3). This represents the first report of a malformation in an anuran associated with FV3. PMID:17604194

Burton, Elizabeth C; Miller, Debra L; Styer, Eloise L; Gray, Matthew J

2008-09-01

441

Type II congenital pulmonary airway malformation in an esophageal lung  

PubMed Central

A seven-month-old girl, born prematurely (birth weight 1000 g) from a twin pregnancy, was admitted to hospital due to recurrent pneumonia and atelectasis. She experienced cough and respiratory distress during feeding. The right hemithorax was smaller than the left, with diminished breath sounds and dullness. Chest x-rays revealed decreased lung volume and multiple radiolucent images in the right lung, as well as overdistention of the left lung. An esophagogram revealed three bronchial branches arising from the lower one-third of the esophagus, corresponding to the right lung and ending in a cul-de-sac. A diagnosis of esophageal lung was established. On bronchography, the right lung was absent and the trachea only continued into the left main bronchus. Echocardiography and angiotomography revealed agenesis of the pulmonary artery right branch. The surgical finding was an esophageal right lung, which was removed; the histopathological diagnosis was type II congenital pulmonary airway malformation in an esophageal lung. PMID:23762890

Martínez-Martínez, Blanca Estela; Furuya, María Elena Yuriko; Martínez-Muñiz, Irma; Vargas, Mario H; Flores-Salgado, Rosalinda

2013-01-01

442

Congenital Cystic Adenomatoid Malformation with Bronchial Atresia in Elderly Patients  

PubMed Central

Congenital cystic adenomatoid malformation (CCAM) is an uncommon, nonhereditary anomaly caused by arrest of lung. Patients with CCAM may present with respiratory distress as newborns, or may remain asymptomatic until later in life. CCAM type I is rarely found in association with bronchial atresia (BA) in adults; we present such a case. Case: A 54-year-old female presented with chronic cough and blood-tinged sputum. Physical examination and laboratory tests were unremarkable. Chest radiographs and a CT scan of the chest showed multiple large air-filled cysts consistent with a CCAM in the right lower lobe, and an oval-shaped opacity in the distal right middle lobal bronchus. Based on the radiologic findings, right middle lobectomy and a medial basal segmentectomy of the right lower lobe were performed via a thoracotomy. These lesions were consistent with Stocker's Type I CCAM and BA in the different lobes. PMID:23101017

Kwak, Hyun Jung; Moon, Ji-Yong; Kim, Sa-Il; Kim, Tae Hyung; Sohn, Jang Won; Kim, Sang-Heon; Shin, Dong Ho; Park, Sung Soo; Chung, Won Sang

2012-01-01

443

[Multiple bilateral pulmonary nodules revealing a congenital cystic adenomatoid malformation].  

PubMed

Congenital cystic adenomatoid malformation (CCAM) of the lung is a rare congenital developmental abnormality, representing about 25% of all congenital lung lesions. It is very rare that presentation is delayed until adulthood. We report a case of 63-year-old woman without notable pathological antecedents in whom a systematic chest X-ray revealed multiple bilateral pulmonary nodules. The patient was asymptomatic and her physical examination was normal. CT scan showed bilateral liquid rounded thin-walled densities of various size, with a homogeneous non calcified content. The diagnosis was based on radiological findings and surgery. The result of histopathological examination obtained by thoracic surgery confirmed CCAM without malignancy. The postoperative follow up showed an excellent recovery. PMID:22522042

Aichaouia, C; Farah, S; Dabboussi, S; Moatamri, Z; M'hamdi, S; Bougrine, F; Bouzaiene, A; Khadhraoui, M; Cheikh, R

2012-08-01

444

Ulnar Nerve Compression at Guyon's Canal by an Arteriovenous Malformation  

PubMed Central

Guyon's canal at the wrist is not the common site of ulnar nerve compression. Ganglion, lipoma, anomalous tendon and muscles, trauma related to an occupation, arthritis, and carpal bone fracture can cause ulnar nerve compression at the wrist. However, ulnar nerve compression at Guyon's canal by vascular lesion is rare. Ulnar artery aneurysm, tortous ulnar artery, hemangioma, and thrombosis have been reported in the literature as vascular lesions. The authors experienced a case of ulnar nerve compression at Guyon's canal by an arteriovenous malformation (AVM) and the patient's symptom was improved after surgical resection. We can not easily predict vascular lesion as a cause of ulnar nerve compression at Guyon's canal. However, if there is not obvious etiology, we should consider vascular lesion as another possible etiology. PMID:19242575

Kim, Sung Soo; Kang, Hee In; Lee, Seung Jin

2009-01-01

445

Embolization of Arteriovenous Malformation with Diluted Mixture of NBCA  

PubMed Central

Summary The technique of nidus embolization with N-butyl-2-cyanoacrylate (NBCA) glue is presented. The microcatheter is navigated into the nidus and the tip is set in a wedged position. A diluted mixture of NBCA in ethiodized oil (a 25 % mixture is mainly used) is injected very slowly, using complete column technique. Using this technique, the progression of glues in the nidus can be seen easily, which leads to improve accuracy and effectiveness of embolization. The mean blood pressure is maintained 15% to 20% below baseline for several days after the embolization to prevent hemorrhage caused by normal pressure breakthrough. We believe this technique is very useful and effective method for the treatment of arteriovenous malformations. However; considerable experience in this technique should be required. PMID:20667245

Tamatani, S.; Koike, T.; Ito, Y.; Tanaka, R.

2000-01-01

446

Dual technique for obliteration of small arteriovenous malformations.  

PubMed

A 17-year-old man underwent an incomplete excision of a left fronto-parietal arterio-venous malformation (AVM), under image guidance. An initial attempt to treat the residual AVM by embolization was unsuccessful. The craniotomy was then re-opened in the angiography suite and the residual AVM was excised by, first, following the microcatheter within the arterial feeding vessel to the AVM and, secondly, by using metallic clips as radiographic markers to locate the position of the AVM during superselective angiography. The dual technique described is likely to be useful in selected cases with small residual AVMs and AVMs near eloquent areas of the brain, where the extent of surgical dissection and postoperative morbidity can be minimized by the precise localization of the AVM. PMID:12389892

Gnanalingham, K; Taylor, W; Watkins, L

2002-08-01

447

Histopathological changes in cerebral arteriovenous malformations following Gamma Knife radiosurgery.  

PubMed

Histological, immunohistochemical and electron microscopic investigations were carried out in a series of surgical pathology material that was removed from 7 patients. They were harboring cerebral arteriovenous malformations (AVMs) that had been previously treated with Leksell Gamma Knife radiosurgery, and presented subsequent bleeding 10-52 months after treatment. Light microscopic studies revealed a spindle cell proliferation in the connective tissue stroma and in the subendothelial region of the irradiated AVM vessels. The histological, immunohistochemical and ultrastructural characteristics of the spindle cell population in the Leksell Gamma Knife-treated AVMs are similar to those designated as myofibroblasts in wound healing processes and pathological fibromatoses. Considering that similar cell modifications have not been demonstrated in control, nonirradiated AVM specimens, these myofibroblasts might contribute to the shrinking process and final occlusion of AVMs after radiosurgery. PMID:17317989

Szeifert, György T; Timperley, Walter R; Forster, David M C; Kemeny, Andras A

2007-01-01

448

Relating middle-ear acoustic performance to body size in the cat family: measurements and models  

Microsoft Academic Search

Is the acoustic performance of the mammalian middle ear dependent on body size? We focus on the cat family, because of its\\u000a qualitatively uniform (and distinctive) middle-ear structure, large size range, and the extensive data available from domestic\\u000a cats which provide a framework for relating middle-ear acoustics to structure. We report measurements of acoustic admittance\\u000a in 17 live adult ears

G. T. Huang; J. J. Rosowski; W. T. Peake

2000-01-01

449

Pneumococcal meningitis threshold model: a potential tool to assess infectious risk of new or existing inner ear surgical interventions .  

E-print Network

??Hypothesis: A minimal threshold of Streptococcus pneumoniae is required to induce meningitis in healthy animals for intraperitoneal (hematogenous), middle ear, and inner ear inoculations, and… (more)

Wei, Benjamin P. C.

2006-01-01

450

Location of Periventricular Nodular Heterotopia Is Related to the Malformation Phenotype on MRI  

PubMed Central

BACKGROUND AND PURPOSE Periventricular nodular heterotopia are common malformations of cortical development that are associated with many clinical syndromes and with many different neuroimaging phenotypes. The purpose of this study was to determine whether specific malformation phenotypes may be related to location, side, or number of PNH as assessed by MR imaging. MATERIALS AND METHODS MR images of 200 patients previously diagnosed with PNH were retrospectively analyzed. PNH were classified according to their location along the ventricles (anterior, posterior, or diffuse), side (unilateral or bilateral), and number of nodules (<5, 6–10, or >10). The cerebrum, brain stem and cerebellum were analyzed to assess associated anomalies. Associations between PNH location and the presence of other anomalies were tested by using Fisher exact test and ?2 test. RESULTS Posterior PNH were significantly associated with malformations of the cerebral cortex, diminished white matter volume, and mid-/hindbrain anomalies. Diffuse PNH were associated with diminished white matter volume, callosal “anomalies,” and the presence of megacisterna magna. Unilateral PNH were strongly associated with cortical malformations. CONCLUSIONS Certain malformation complexes are associated with PNH in specific locations: posterior PNH with cerebral cortical and mid-/hindbrain malformations and diffuse PNH with callosal anomalies and megacisterna magna. Knowledge of these associations should allow more directed analyses of brain MR imaging in patients with PNH. In addition, knowledge of these associations may help to direct studies to elucidate the causes of these malformation complexes. PMID:23064591

González, G.; Vedolin, L.; Barry, B.; Poduri, A.; Walsh, C.; Barkovich, A.J.

2013-01-01

451

Risk of malformations associated with residential proximity to hazardous waste sites in Washington State.  

PubMed

Hazardous waste sites often contain substances harmful to fetal development. Using linked birth-hospital discharge and hazardous sites data for Washington State, we evaluated the association between malformation occurrence and maternal residential proximity to hazardous waste sites. Cases (N=63,006) were infants born 1987-2001 with malformations. Controls (N=315,030) were randomly selected infants without malformations born during these years. Distance between maternal residence and nearest hazardous waste site was measured using geographic information systems (GIS) software. Odds ratio (OR) estimates of the relative risk of malformation at varying distances were calculated. Relative to living >5 miles from a site, living < or = 5 miles was associated with increased risk of any malformations in offspring (for >2- < or = 5 miles: OR 1.15: 95% Confidence Interval (CI): 1.10, 1.21; for >1- < or = 2 miles: OR 1.26, 95% CI: 1.20, 1.32; for >0.5- < or = 1 miles: OR 1.28, 95% CI: 1.22, 1.35; for < or = 0.5 miles: OR 1.33, 95% CI: 1.27, 1.40.) Risk estimates varied by urban vs. rural maternal residence and by specific malformation type. Hazardous waste sites are often located within populated areas. Thus, the possibility of increased malformation occurrence among those in close proximity deserves closer scrutiny. PMID:17046743

Kuehn, Carrie M; Mueller, Beth A; Checkoway, Harvey; Williams, Marcia

2007-03-01

452

Malformations of Cortical Development: Diagnostic Accuracy of Fetal MR Imaging  

PubMed Central

Purpose: To determine the diagnostic accuracy of fetal magnetic resonance (MR) imaging for malformations of cortical development by using postnatal MR imaging as reference standard. Materials and Methods: Eighty-one patients who had undergone fetal and postnatal MR imaging of the brain were identified in this institutional review board–approved, HIPAA-compliant study. Images were retrospectively reviewed in consensus by two pediatric neuroradiologists who were blinded to clinical information. Sensitivity and specificity were calculated according to retrospective review of the images and clinical reports for fetal MR images. The Fisher exact test was used to compare results for fetuses imaged before and after 24 gestational weeks and for image review versus clinical reports for fetal MR images. Results: Median gestational age at fetal MR imaging was 25.0 weeks (range, 19.71–38.14 weeks). Postnatal MR imaging depicted 13 cases of polymicrogyria, three cases of schizencephaly, and 15 cases of periventricular nodular heterotopia. Sensitivity and specificity of fetal MR imaging were 85% and 100%, respectively, for polymicrogyria; 100% each for schizencephaly; and 73% and 92%, respectively, for heterotopia. When heterotopia was seen in two planes, specificity was 100% and sensitivity was 67%. Sensitivity for heterotopia decreased to 44% for fetuses younger than 24 weeks. According to reports for fetal MR images, prospective sensitivity and specificity, respectively, were 85% and 99% for polymicrogyria, 100% and 99% for schizencephaly, and 40% and 91% for heterotopia. Conclusion: Fetal MR imaging had the highest sensitivity for polymicrogyria and schizencephaly. Specificity was 100% for all cortical malformations when the abnormality was seen in two planes. Sensitivity for heterotopia was lower for fetuses younger than 24 weeks. Knowledge of the gestational age is important, especially for counseling patients about heterotopia. © RSNA, 2012 PMID:22495681

Cuneo, Addison A.; Barkovich, A. James; Hashemi, Zary; Bartha, Agnes I.; Xu, Duan

2012-01-01

453

NPHP4 Variants are Associated with Pleiotropic Heart Malformations  

PubMed Central

Rationale Congenital heart malformations are a major cause of morbidity and mortality especially in young children. Failure to establish normal left-right (L-R) asymmetry often results in cardiovascular malformations and other laterality defects of visceral organs. Objective To identify genetic mutations causing cardiac laterality defects. Methods and Results We performed a genome-wide linkage analysis in patients with cardiac laterality defects from a consanguineous family. The patients had combinations of defects that included dextrocardia, transposition of great arteries, double outlet right ventricle, atrio-ventricular septal defects and caval vein abnormalities. Sequencing of positional candidate genes identified mutations in NPHP4. We performed mutation analysis of NPHP4 in 146 unrelated patients with similar cardiac laterality defects. Forty-one percent of these patients also had laterality defects of the abdominal organs. We identified eight additional missense variants that were absent or very rare in controls. To study the role of nphp4 in establishing L-R asymmetry, we used antisense morpholinos to knockdown nphp4 expression in zebrafish. Depletion of nphp4 disrupted L-R patterning as well as cardiac and gut laterality. Cardiac laterality defects were partially rescued by human NPHP4 mRNA, whereas mutant NPHP4 containing genetic variants found in patients failed to rescue. We show that nphp4 is involved in the formation of motile cilia in Kupffer’s vesicle (KV), which generate asymmetric fluid flow necessary for normal L-R asymmetry. Conclusions NPHP4 mutations are associated with cardiac laterality defects and heterotaxy. In zebrafish, nphp4 is essential for the development and function of KV cilia and is required for global L-R patterning. PMID:22550138

French, Vanessa M.; van de Laar, Ingrid M.B.H.; Wessels, Marja W.; Rohe, Christan; Roos-Hesselink, Jolien W.; Wang, Guangliang; Frohn-Mulder, Ingrid M.E.; Severijnen, Lies-Anne; de Graaf, Bianca M.; Schot, Rachel; Breedveld, Guido; Mientjes, Edwin; van Tienhoven, Marianne; Jadot, Elodie; Jiang, Zhengxin; Verkerk, Annemieke; Swagemakers, Sigrid; Venselaar, Hanka; Rahimi, Zohreh; Najmabadi, Hossein; Meijers-Heijboer, Hanne; de Graaff, Esther; Helbing, Wim A.; Willemsen, Rob; Devriendt, Koen; Belmont, John W.; Oostra, Ben A.; Amack, Jeffrey D.; Bertoli-Avella, Aida M.

2014-01-01

454

Non-operative outcomes in Chiari I malformation patients.  

PubMed

While postoperative outcomes of Chiari I malformation patients have been well-reported, there is a paucity of literature concerning non-operative management in these patients. We retrospectively identified patients with Chiari I malformation who were not recommended for surgery based on lack of clinical objective findings or inconsistent cough headaches and conducted patient follow-up with a prospective telephone survey. Of the 68 patients (mean age at diagnosis 30.1±17.4years), 72% were female and 31% were pediatric patients (age at diagnosis ?18years). Average follow up was 4.9±2.9years. Typical presenting symptoms included cough headache, non-specific headache, nausea, ataxia, dysphagia and paresthesias. Overall, 40% of patients who had cough headaches and 61.5% of patients with non-specific headaches reported improvement. The presence of subjective sensory symptoms was significantly associated with less likelihood of cough headache improvement while the presence of a cough headache was also associated with a lower likelihood of improvement in all non-cough symptoms. The pediatric subgroup had a greater rate of improvement with all cases of nausea/emesis and paresthesias improved or resolved at follow-up. Overall 67% of pediatric patients had improved cough headache and 71% had improvement of migraines/diffuse headaches. We found that many symptoms of Chiari I patients from our conservatively managed cohort either improved or remained unchanged over time. However, the presence of cough headaches was a significant negative predictor of concomitant symptom improvement. This further validates the view that patients with cough headaches should be considered for surgical intervention and provides useful information to counsel patients. PMID:25080302

Killeen, Amy; Roguski, Marie; Chavez, Alexis; Heilman, Carl; Hwang, Steven

2015-01-01

455

Advanced Echocardiographic Imaging of the Congenitally Malformed Heart  

PubMed Central

There have been significant advancements in the ability of echocardiography to provide both morphological and functional information in children with congenitally malformed hearts. This progress has come through the development of improved technology such as matrix array probes and software which allows for the off line analysis of images to a high standard. This article focuses on these developments and discusses some newer concepts in advanced echocardiography such is multi-planar reformatting [MPR] and tissue motion annular displacement [TMAD]. Our aim is to discuss important aspects related to the quality and reproducibility of data, to review the most recent published data regarding advanced echocardiography in the malformed heart and to guide the reader to appropriate text for overcoming the technical challenges of using these methods. Many of the technical aspects of image acquisition and post processing have been discussed in recent reviews by the authors and we would urge readers to study these texts to gain a greater understanding [1]. The quality of the two dimensional image is paramount in both strain analysis and three dimensional echocardiography. An awareness of how to improve image quality is vital to acquiring accurate and usable data. Three dimensional echocardiography (3DE) is an attempt to visualise the dynamic morphology of the heart. Although published media is the basis for theoretical knowledge of how to practically acquire images, electronic media [eg.www.3dechocardiography.com] is the only way of visualising the advantages of this technology in real time. It is important to be aware of the limitations of this technology and that much of the data gleaned from using these methods is at a research stage and not yet in regular clinical practice. PMID:23228075

Black, D; Vettukattil, J

2013-01-01

456

FOOD HABITS AND HUNTING RANGES OF SHORT-EARED OWLS  

Microsoft Academic Search

ABSTRCT.--The diet of the Short-eared Owl (Asio flammeus) was quantified by analyzing 400 pellets collected in two agricultural landscapes of southern Chile (Osorno and Chahuilco). Diet composition fluctuated seasonally and included several species of small mammals, birds, and insects. Almost 80% of the annual biomass consumed was from two rodent species (Akodon olivaceus and Rattus norvegicus) and of a bird

DAVID R. MARTINEZ; RICARDO A. FIGUEROA; CARMEN L. OCAMPO; FABIAN M. JAKSIC

457

An outbreak of erysipelas in eared grebes (Podiceps nigricollis).  

PubMed

An outbreak of erysipelas killed an estimated 5,000 aquatic birds on Great Salt Lake (Utah) in late November, 1975. Although several thousand ducks and gulls were using the lake, at least 99 percent of the victims were eared grebes. A hypothetical explanation for the selective mortality is offered. PMID:16502704

Jensen, W I; Cotter, S E

1976-10-01

458

Quality Management in Middle Ear Surgery: Controversies Regarding Preoperative Imaging  

Microsoft Academic Search

Today a large variety of different imaging techniques are available for middle ear investigation. However, imaging is not suitable to give essential information in every case on the surgical strategy to be chosen. This article discusses the most frequent indications for preoperative imaging and the relevant techniques. CT scanning, MRI and rotational tomography are taken into consideration as well as

T. Zahnert; C. Offergeld

2010-01-01

459

Prevalence of red ear syndrome in juvenile primary headaches  

Microsoft Academic Search

Background: Previous studies have suggested a relationship between ‘red ear syndrome’ (RES) and pediatric migraine. Aims of this study were (i) to assess the frequency, specificity and sensitivity of RES in a population of pediatric migraineurs and (ii) to establish the pathophysiological mechanisms of RES associated with migraine.Methods and results: A total of 226 children suffering from headache (aged 4—17

V. Raieli; A. Compagno; F. Brighina; G. La Franca; D. Puma; D. Ragusa; G. Savettieri; M. DAmelio

2011-01-01

460

Ion Homeostasis in the Ear: Mechanisms, Maladies, and Management  

PubMed Central

Purpose of Review Describe ion and water homeostatic mechanisms in the inner ear, how they are compromised in hearing disorders, and what treatments are employed to restore auditory function. Recent Findings The ion and water transport functions in the inner ear help maintain the proper endolymph K+ concentration required for hair cell function. Gene defects and idiopathic alterations in these transport functions cause hearing loss, but often the underlying cause is unknown. Current therapies largely involve glucocorticoid treatment, although the mechanisms of restoration are often undeterminable. Recent studies of these ion homeostatic functions in the ear are characterizing their cellular and molecular control. It is anticipated that future management of these hearing disorders will be more targeted to the cellular processes involved and improve the likelihood of hearing recovery. Summary A better understanding of the ion homeostatic processes in the ear will permit more effective management of their associated hearing disorders. Sufficient insight into many homeostatic hearing disorders has now been attained to usher in a new era of better therapies and improved clinical outcomes. PMID:20693900

Trune, Dennis R.

2010-01-01

461

Maize starch fine structures affected by ear developmental temperature  

Microsoft Academic Search

Growing temperature is known to affect the grain yield and quality of maize. Two genetically unrelated normal dent maize inbreds, ICI63 and ICI92, with different heterotic backgrounds were grown in a greenhouse with the ears wrapped in temperature control devices set at 25 and 35 °C during the grain-filling period. Grain yield, kernel weight, and kernel density were less for

Ting-jang Lu; Jay-lin Jane; Peter L. Keeling; George W. Singletary

1996-01-01

462

Future Approaches for Inner Ear Protection and Repair  

ERIC Educational Resources Information Center

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…

Shibata, Seiji B.; Raphael, Yehoash

2010-01-01

463

Purdue extensionAspergillus Ear Rot Purdue extension  

E-print Network

causes Aspergillus ear rot, one of the most important diseases in corn. The fungus pro- duces a mycotoxin to livestock 3. Mycotoxin testing 4. How to minimize losses and handle diseased grain after harvest 5. How these levels. detecting Mycotoxins An ultraviolet lamp, or black light, is often used as an initial screen

Holland, Jeffrey

464

EARS: Toward Fast Analysis of 3D Human  

E-print Network

Team, US Army Natick Soldier RDEC 1 Kansas St, Natick, MA 01760, USA ABSTRACT We present the Enhanced-time feedback on the quality of a human body scan mesh. We have tested EARS on a set of 100 female and 100 male. The Ergonomics team at the Natick Soldier RDEC is conducting a survey of anthropometrics for the soldier

465

Biology and migration of Eared Grebes at the Salton Sea  

Microsoft Academic Search

The Eared Grebe (Podiceps nigricollis Brehm) is the North American bird species most closely associated with highly saline habitats, and in winter and early spring it is the most abundant waterbird at the Salton Sea. During the fall, the great majority of the North American population stages at hypersaline lakes in the Great Basin, departing in early winter for wintering

Joseph R. Jehl; Robert L. McKernan

2002-01-01

466

ORIGINAL COMMUNICATION Imaging Microscopy of the Middle and Inner Ear  

E-print Network

present high-resolution MicroCT images of the middle ear and bony labyrinth to highlight the utility:607­612 (2004) © 2004 Wiley-Liss, Inc. #12;properly in the microCT scanner. The specimen was scanned without decalcification or additional fixation. MicroCT scanning differs from clinical scanning in that the object

Allen, Jont

467

Acetone Photophysics at ear Critical to Supercritical Conditions  

E-print Network

Acetone Photophysics at ear Critical to Supercritical Conditions T. Tran* , Y. Kochar* , J-0150 The photophysics of vapor and liquid acetone are experimentally examined from subcritical to supercritical conditions with 266 nm excitation, motivated by an interest in using acetone to study transcritical fuel

Seitzman, Jerry M.

468

The fluid mechanics of the inner-ear disorder BPPV  

Microsoft Academic Search

The inner ear of mammals contains fluid-filled semi-circular canals with a flexible sensory membrane (called a cupula) which detects rotational acceleration. Benign Paroxysmal Positional Vertigo (BPPV) is one of the most common disorders of this system diagnosed today, and is characterized by symptoms of dizziness and nausea brought on by sudden changes in head orientation. BPPV is believed to have

Michael Weidman; Todd Squires; Howard Stone

2001-01-01

469

21 CFR 874.4140 - Ear, nose, and throat bur.  

Code of Federal Regulations, 2011 CFR

...Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES EAR...nose, or throat area. The bur consists of a carbide cutting tip on a metal shank or a coating of diamond on a metal...

2011-04-01

470

21 CFR 874.4140 - Ear, nose, and throat bur.  

Code of Federal Regulations, 2010 CFR

...Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES EAR...nose, or throat area. The bur consists of a carbide cutting tip on a metal shank or a coating of diamond on a metal...

2010-04-01

471

21 CFR 874.4140 - Ear, nose, and throat bur.  

...Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES EAR...nose, or throat area. The bur consists of a carbide cutting tip on a metal shank or a coating of diamond on a metal...

2014-04-01

472

21 CFR 874.4140 - Ear, nose, and throat bur.  

Code of Federal Regulations, 2012 CFR

...Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES EAR...nose, or throat area. The bur consists of a carbide cutting tip on a metal shank or a coating of diamond on a metal...

2012-04-01

473

21 CFR 874.4140 - Ear, nose, and throat bur.  

Code of Federal Regulations, 2013 CFR

...Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES EAR...nose, or throat area. The bur consists of a carbide cutting tip on a metal shank or a coating of diamond on a metal...

2013-04-01

474

EARS buoy applications by LADC: I. marine animal acoustics  

Microsoft Academic Search

Littoral Acoustic Demonstration Center (LADC) scientists have investigated sperm and beaked whale clicks as recorded on Environmental Acoustic Recording System (EARS) buoys to analyze whale behavior and the possibility of identifying individual whales acoustically. The research began in 2001 and continues through the present. LADC has conducted three experiments in the northern Gulf of Mexico and participated with the Naval

George E. Ioup; Juliette W. Ioup; Lisa A. Pflug; Arslan M. Tashmukhambetov; Natalia A. Sidorovskaia; Philip Schexnayder; Christopher O. Tiemann; Alan Bernstein; Stan A. Kuczaj; Grayson H. Rayborn; Joal J. Newcomb; R. Carlson; A. Ekimov

2009-01-01

475

How the Ear Works: Nature's Solutions for Listening.  

ERIC Educational Resources Information Center

A history of hearing and a review of the physics of sound is followed by an overview of how the ear works. The outer hair cell is the focus of particular attention because of its central role in the conversion of sound energy into neural energy used by the brain. Contains a list of recommended resources. (CR)

Brownell, William E.

1997-01-01

476

Intratympanic gentamicin therapy for vertigo in nonserviceable ears  

Microsoft Academic Search

Purpose: Intratympanic ototoxic agents have become a widely accepted means of managing vertigo in patients with Meniere's disease while preserving residual hearing. We investigated expanding the indications for intratympanic gentamicin to include control of vertigo in patients without serviceable hearing in the involved ear caused by a variety of end-organ pathologies. Materials and Methods: We present a retrospective series of

Paul W Bauer; C. Bruce MacDonald; L. Clarke Cox

2001-01-01

477

Baby corn, green ear, and grain yield of corn cultivars  

Microsoft Academic Search

Most maize cultivars have been developed for grain production. Because superior cultivars may differ in their exploiting purposes, interest has been demonstrated for the evaluation of corn cultivars with regard to their baby corn, green ear, and grain yields production ability. In the present work ten corn cultivars (AG 405, AG 1051, AG 2060, AG 6690, AG 7575, AG 8080,

Itala Paula de C. Almeida; Paulo Sérgio L. e Silva; Maria Z. de Negreiros; Zenaide Barbosa

2005-01-01

478

21 CFR 344.52 - Labeling of ear drying aid drug products.  

Code of Federal Regulations, 2013 CFR

...in bold type] [bullet] ear drainage or discharge [bullet] pain, irritation, or rash in the ear [bullet] had ear surgery...a doctor if [in bold type] irritation (too much burning) or pain occurs.” (d) Directions. The labeling of the product...

2013-04-01

479

21 CFR 344.52 - Labeling of ear drying aid drug products.  

...in bold type] [bullet] ear drainage or discharge [bullet] pain, irritation, or rash in the ear [bullet] had ear surgery...a doctor if [in bold type] irritation (too much burning) or pain occurs.” (d) Directions. The labeling of the product...

2014-04-01

480

21 CFR 344.52 - Labeling of ear drying aid drug products.  

Code of Federal Regulations, 2010 CFR

...in bold type] [bullet] ear drainage or discharge [bullet] pain, irritation, or rash in the ear [bullet] had ear surgery...a doctor if [in bold type] irritation (too much burning) or pain occurs.” (d) Directions. The labeling of the product...

2010-04-01

481

21 CFR 344.52 - Labeling of ear drying aid drug products.  

Code of Federal Regulations, 2011 CFR

...in bold type] [bullet] ear drainage or discharge [bullet] pain, irritation, or rash in the ear [bullet] had ear surgery...a doctor if [in bold type] irritation (too much burning) or pain occurs.” (d) Directions. The labeling of the product...

2011-04-01

482

Ear-Canal Reflectance, Umbo Velocity, and Tympanometry in Normal-Hearing Adults  

E-print Network

Ear-Canal Reflectance, Umbo Velocity, and Tympanometry in Normal-Hearing Adults John J. Rosowski,1. Halpin,4 and Saumil N. Merchant1,2,3 Objective: This study compares measurements of ear-canal reflectance (ECR) to other objective measurements of middle ear function including audiometry, umbo velocity (VU

Allen, Jont

483

Audiometric Predictions Using Stimulus-Frequency Otoacoustic Emissions and Middle Ear  

E-print Network

Audiometric Predictions Using Stimulus-Frequency Otoacoustic Emissions and Middle Ear Measurements or moderate-severe, and correlate with pure-tone thresholds in a population of adults with normal middle ear function. Other goals are to determine if middle ear function as assessed by wideband acoustic transfer

Allen, Jont

484

ARTICLE doi:10.1038/nature09921 Transitional mammalian middle ear from  

E-print Network

ARTICLE doi:10.1038/nature09921 Transitional mammalian middle ear from a new Cretaceous Jehol the dentary and the detached ossicles during mammalian evolution. This transitional mammalian middle ear narrows the morphological gap between the mandibular middle ear in basal mammaliaforms and the definitive

Sullivan, Jack

485

Measurements of human middle ear forward and reverse acoustics: Implications for otoacoustic emissions  

E-print Network

Measurements of human middle ear forward and reverse acoustics: Implications for otoacoustic and California Ear Institute at Stanford, 801 Welch Road, Palo Alto, California 94304 Received 11 July 2002; revised 1 February 2003; accepted 4 February 2003 Middle and inner ears from human cadaver temporal bones

Allen, Jont

486

Steps for Using the EAR Part 732-page 1 Export Administration Regulations  

E-print Network

Steps for Using the EAR Part 732-page 1 Export Administration Regulations §732.1 STEPS OVERVIEW (a)(1) Introduction In this part, references to the EAR are references to 15 CFR chapter VII, subchapter C. This part is intended to help you determine your obligations under the EAR by listing logical

Bernstein, Daniel