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1

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

2

Single gene influences on radiologically-detectable malformations of the inner ear.  

PubMed

Inner ear malformations associated with hearing loss or vestibular dysfunction are discussed from the viewpoint of the etiologies of the malformation. Symptoms of classification of inner ear malformations are discussed. The significance of malformations of the cochlea and vestibular aqueduct to auditory function are discussed. Genetics features and characteristics of Branchio-oto-renal, Waardenburg's, Pendred's, DiGeorge's, Wildervanck, Fountain, and Treacher Collins syndromes are discussed in relation to ear abnormalities and hearing. Similar attention is given to genetic studies of nonsyndromic hearing loss. PMID:9777486

Smith, S D; Harker, L A

1998-01-01

3

Patterns of anomalies in children with malformed ears.  

PubMed

Sixteen children with anomalies of the auricle and/or middle ear who presented malformations of the face, mouth, upper airway, spine, limbs, heart, gastrointestinal (GI), and/or genitourinary (GU) systems, were described. While clusters of anomalies suggested syndromes such as the oculo-auriculo-vertebral syndrome of Goldenhar, hamifacial microsomia, mandibulo-facial dysostosis (Treacher Collins syndrome), Pierre Robin, Klippel-Feil, Moebius, Duane, and/or VATER syndromes, many children did not fit what are usually considered even minimal criteria for these syndromes. Several children had malformations which fit the description of more than one syndrome. The importance of investigating the children for unsuspected anomalies, especially of the GU system, was emphasized. Life threatening problems in this group consisted of airway problems, congenital heart disease, and major anomalies of the GI and GU systems. Better management of sucking, swallowing and airway problems might have decreased the early morbidity and mortality (3/16) in this group. Children with multiple defacing anomalies may not be mentally retarded so that aggressive management of their visceral anomalies and hearing problems, and early educational intervention are mandatory. Delay in development may be due to hearing loss, vestibular impairment, ataxia, the consequences of early malnutrition, and multiple hospitalizations rather than to mental retardation. A pessimistic attitude in infancy is unwarranted since it is impossible to predict which children will end up competitive individuals. PMID:966914

Rapin, I; Ruben, R J

1976-10-01

4

Middle and inner ear malformations in two siblings exposed to valproic acid during pregnancy: A case report.  

PubMed

Valproic acid (VPA) is a known teratogenic drug. Exposure to VPA during the pregnancy can lead to a distinct facial appearance, a cluster of major and minor anomalies and developmental delay. In this case report, two siblings with fetal valproate syndrome and a mild conductive hearing loss were investigated. Radiologic evaluation showed middle and inner ear malformations in both children. Audiologic, vestibular and motor examination was performed. This is the first case report to describe middle and inner ear malformations in children exposed to VPA. PMID:25216807

Van Houtte, Evelyne; Casselman, Jan; Janssens, Sandra; De Kegel, Alexandra; Maes, Leen; Dhooge, Ingeborg

2014-11-01

5

Cleft lip and palate, pili torti, malformed ears, partial syndactyly of fingers and toes, and mental retardation: a new syndrome?  

Microsoft Academic Search

Two sibs with a syndrome including cleft lip and palate, sparse scalp hair, malformed protruding ears, and partial syndactyly of the fingers and toes are reported. The older child also has mental retardation and pili torti. This syndrome is most probably inherited as an autosomal recessive disorder.

J Zlotogora; Y Zilberman; A Tenenbaum; M R Wexler

1987-01-01

6

[Novel possibilities for the rehabilitation of patients presenting with congenital external and middle ear malformations].  

PubMed

The objective of the present study was to estimate the advantages of cochlear Baha BIA 400 abutments in the intraoperative and early postoperative periods. A total of 10 implantations of the systems with the use of hydroxyapatite bone cement were performed in 9 patients of different age. Stability of the implants and intensity of skin reactions were evaluated. The data obtained indicate that the use of cochlear Baha BIA 400 abutments significantly simplifies and shortens the surgical stage of rehabilitation producing a good cosmetic result. The use of the Osstell instrument made it possible to estimate stability of the implants intraoperatively and evaluate the effectiveness of osteointegration during the follow-up period. Analysis of the results of the study provided a basis on which to improve the quality and shorten duration of the rehabilitative treatment of the patients presenting with congenital external and middle ear malformations. Moreover, the data obtained can be used to develop practical recommendations for the further work in this area. PMID:24781169

Mileshina, N A; Osipenkov, S S; Bakhshinian, V V; Tavartkiladze, G A

2014-01-01

7

Ear Problems  

MedlinePLUS

... outer ear and the surrounding skin? Yes Your ear canal, outer ear and the skin around your ear ... thick pus-filled or bloody drainage from the ear canal that started after a sharp, sudden pain? Yes ...

8

Ear examination  

MedlinePLUS

... or forward on the ear to straighten the ear canal. Then, the tip of the otoscope will be ... light beam shines through the otoscope into the ear canal. The health care provider will carefully move the ...

9

Swimmer's Ear  

MedlinePLUS

... skin that's protected by a thin coating of earwax . Most of the time, water can run in ... an Ear Infection? Perforated Eardrum Your Ears What's Earwax? How Do Pain Relievers Work? Taking Care of ...

10

Ear wax  

MedlinePLUS

... in the ear: Baby oil Commercial drops Glycerin Mineral oil Water Another method is to wash out the ... cloth or paper tissue wrapped around your finger. Mineral oil can be used to moisturize the ear and ...

11

Ear Infections  

MedlinePLUS

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

12

Super Ears.  

ERIC Educational Resources Information Center

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

Thompson, Stan

1995-01-01

13

Ear emergencies  

MedlinePLUS

... a clean cloth and keep it on ice. DRAINAGE FROM INSIDE THE EAR Cover the outside of ... DO NOT block any drainage coming from the ear. DO NOT try to clean or wash the inside of the ear canal. DO NOT put ...

14

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 Peńa

2007-01-01

15

Arteriovenous Malformation  

Microsoft Academic Search

\\u000a Intracranial arteriovenous malformations (AVMs) are congenital lesions arising from abnormal blood vessel formation [1]–[3]. Whereas normal embryogenesis results in the differentiation of primordial vascular channels into mature arteries, veins,\\u000a and capillaries, patients with AVMs develop direct arteriovenous shunts without the appropriate intervening vascular beds.\\u000a Recent large, prospective, population-based studies have determined the incidence of newly diagnosed AVM patients to range

Bruce E. Pollock

16

Ear Tumors  

MedlinePLUS

... infections may have an increased risk of developing squamous cell carcinoma. When these cancers first appear, they can be successfully treated by removing them surgically or by applying radiation therapy. More advanced cancers may require surgical removal of a larger area of the external ear. ... Back to Top Previous: Dermatitis of the Ear ...

17

Your Ears  

MedlinePLUS

... fluid in the semicircular canals stops moving, your brain gets the right message and you regain your balance. Three Cheers for the Ears! Your ears take care of you, so take care of them. Protect your hearing by wearing earplugs at loud music concerts and around noisy machinery, like in wood ...

18

Cerebral Cavernous Malformation  

MedlinePLUS

NINDS Cerebral Cavernous Malformation Information Page Synonym(s): Cavernomas, Cavernous Angioma, Congenital Vascular Cavernous Malformations, Familial Hemangioma, Nevus Cavernosus, Vascular Erectile Tumor, Cavernous Malformation Table of Contents (click to jump to sections) ...

19

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 animalâs 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

20

Ear Tubes  

MedlinePLUS

... more below. Please direct any interview requests or policy questions to our media and public ... nearly every child has experienced at least one episode. Most ear infections either resolve on their ...

21

How the Ear Works  

MedlinePLUS

... the part you can see) opens into the ear canal. The eardrum (tympanic membrane) separates the ear canal from the middle ear. The middle ear contains ... funnel through the ear opening, down the external ear canal, and strike your eardrum, causing it to vibrate. ...

22

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

23

Congenital malformations in Shimla  

Microsoft Academic Search

Congenital malformations were studied over a period of five years in 10, 100 consecutive births including still births at\\u000a Indira Gandhi Medical College, Shimla. Out of these, 180 babies had one or the other congenital malformations and the overall\\u000a incidence was 1.78%. Amongst the 311 still born babies 47 had congenital malformations indicating that the incidence of congenital\\u000a malformations was

Neelam Grover

2000-01-01

24

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; Andres, Vivian; Campillo Betancourt, Daine

2014-01-01

25

Travel Inside the Ear  

MedlinePLUS Videos and Cool Tools

... for Educators Videos Travel Inside the Ear Video Travel Inside the Ear Video When sound waves reach ... are smaller than an orange seed. It then travels into the inner ear, which is filled with ...

26

Genetic features of hearing loss associated with ear anomalies: PDS and EYA1 mutation analysis  

Microsoft Academic Search

Mutation analysis of the PDS gene and the EYA1 gene, which are reported to be responsible for hearing loss associated with ear anomalies, was performed in 24 deaf patients\\u000a with various middle and inner ear anomalies. The present study was done to clarify the spectrum of middle and inner ear malformations\\u000a covered by these two genes. PDS mutations were found

Atsushi Namba; Satoko Abe; Hideichi Shinkawa; William J. Kimberling; Shin-ichi Usami

2001-01-01

27

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

28

Ear Plastic Surgery  

MedlinePLUS

... receive light-weight earrings. Does Insurance Pay for Cosmetic Ear Surgery? Insurance usually does not cover surgery solely for ... you can expect. Updated 6/11/12 Ears Cosmetic Surgery, Facelift, Rhinoplasty, Blepharoplasty

29

Ears and Altitude  

MedlinePLUS

... variety of reasons. When that occurs, the middle ear pressure cannot be equalized. The air already there is ... as pilots, are taught how to equalize their ear pressure. Anybody can learn the trick too. How to ...

30

Ear infection - chronic  

MedlinePLUS

Middle ear infection - chronic; Otitis media - chronic; Chronic otitis media; Chronic ear infection ... Kerschner JE. Otitis media. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, PA: Saunders ...

31

Pelvic vascular malformations.  

PubMed

Vascular malformations (VMs) comprise a wide spectrum of lesions that are classified by content and flow characteristics. These lesions, occurring in both focal and diffuse forms, can involve any organ and tissue plane and can cause significant morbidity in both children and adults. Since treatment strategy depends on the type of malformation, correct diagnosis and classification of a vascular lesion are crucial. Slow-flow VMs (venous and lymphatic malformations) are often treated by sclerotherapy, whereas fast-flow lesions (arteriovenous malformations) are generally managed with embolization. In addition, some cases of VMs are best treated surgically. This review will present an overview of VMs in the female pelvis as well as a discussion of endovascular therapeutic techniques. PMID:24436563

Christenson, Brian M; Gipson, Matthew G; Smith, Mitchell T

2013-12-01

32

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

33

Ear tube insertion  

MedlinePLUS

... trapped fluid can flow out of the middle ear. This prevents hearing loss and reduces the risk of ear infections. ... do not have long-term damage to their hearing or speech, even when ... many months. Ear tube insertion may be done when fluid builds ...

34

Management of Vascular Malformations  

PubMed Central

Background: Even though vascular malformations are well categorized, further details are relatively unknown. Of treated patients regarding the frequency, demographic distributions, and other related factors by multivariate regression analyses in proportion to total vascular malformations, methods of treatment and how to manage them have not been elucidated thoroughly. Methods: From January 2006 to March 2012, consecutively treated patients with vascular anomalies were included in this investigation at least 1-year follow-up. Results: Of the total of 123 cases, 86 females and 37 males, the mean follow-up was 3.5?±?1.68 years, and the frequency of treatment was 1–8 times (1.8?±?1.30). Surgery was performed for 22 cases (17.9%) of venous malformations and arteriovenous malformations. In multivariate regression, the frequency of treatment was significantly correlated with the length of follow-up (P < 0.001), age (P < 0.05), and type of malformations (P < 0.05) (R2 = 0.18). Need for surgery was significantly increased with age at odds ratio (OR) of 1.06 [95% confidence interval (CI), 1.03–1.80] (P < 0.001), and head/face/neck, and upper limb are more performed at OR of 0.24 (95% CI, 0.07–0.85) (P < 0.05). The satisfaction score varied from 1 to 5 (3.9?±?0.68). Complications occurred in 3 cases (2.4%). In logistic regression of complications, the OR of the satisfaction score was 0.13 (95% CI, 0.02–0.80) (P < 0.05). Conclusions: Treatment of vascular malformations is an integral part of multidisciplinary approaches. Venous malformations are more frequent in combination surgery, and if there are fewer complications, the patients’ satisfaction increases.

Houbara, Seiji; Hirano, Akiyoshi

2014-01-01

35

Ear candling: a case report.  

PubMed

We describe a case of ear candling presenting as hearing loss, and review the literature. Ear candling is considered as an alternate medical therapy for dewaxing the ears, discomfort in ears and sinuses, rhinitis, sinusitis, glue ear, colds, flu, migraines, poor or "muffled" hearing, high stress, and ringing in the ear. There is no evidence of its effectiveness, and it can actually cause damage to the ears. PMID:19958263

Zackaria, Mohamed; Aymat, Antony

2009-01-01

36

Rare malformation of glans penis: arteriovenous malformation.  

PubMed

Pediatric glans penis malformations, especially arteriovenous malformations (AVM), are very rare. Herein, we report two rare cases. A 14-year-old boy attended our outpatient clinic with chief complaints of purple swelling and rapidly growing lesion on the glans penis. The lesion was excised surgically after physical and radiological evaluations. Pathology reported AVM and the patient is being followed up. The second case is a 2-year-old boy who was admitted with a big lesion involving glans penis and genital area that has been present since birth. In physical and radiological evaluations, lesion on the glans penis was pulsatile. Parents of the patient did not want any surgery and patient has been in follow-up. Diagnosis of the vascular lesions on glans penis is very easy by physical and radiological examinations today. Long-term follow-up is very important for AVM. Clinicians must make a careful effort to document new glans lesions in the pediatric population and decrease anxiety in the parents of affected children. PMID:23771468

Akin, Y; Sarac, M; Yucel, S

2013-01-01

37

Reoperation for Chiari Malformations  

Microsoft Academic Search

Background: We undertook this study to characterize those patients who required reoperations for Chiari malformation and to determine whether modifications in surgical technique at the initial procedure might have obviated the need for repeat surgery. Methods: We reviewed the hospital records, imaging studies, operative reports, and follow-up data of those patients who were undergoing a second operation as part of

David Sacco; R. Michael Scott

2003-01-01

38

Autoimmune Inner Ear Disease (AIED)  

MedlinePLUS

... debris in the ear can cause problems. Some autoimmune disorders that can affect the ear include Cogan’s syndrome, ... even indistinguishable, from other vestibular disorders. Diagnosing an autoimmune disorder as the cause of inner ear symptoms can ...

39

Congenital Chiari malformations.  

PubMed

Chiari malformation is the commonest anomaly of the craniovertebral junction involving both the skeletal as well as the neural structures. This entity has rapidly evolved over the past decade with newer visualization techniques, thus posing new challenges to diagnosis and management. This review includes the developmental theories, the latest nomenclature and existing treatment modalities of this interesting anomaly. Five theories tried to explain the malformation of the hindbrain and the neuraxis but no single theory completes the development of embryonic defects. Several atypical presentations have been reported with either incidental/asymptomatic features resulting in further classifications. The new magnetic resonance imaging flow techniques attempt to substantiate the clinical presentations and correlate with the abnormality which can be subtle in correlation. Surgical correction to improve the cranial volume, decrease the hydrocephalus and improve flow across the foramen magnum is the mainstay but needs to be tailored to a given type of malformation. Further clinical and imageological studies, especially longitudinal natural history, might improve our understanding of the atypical/asymptomatic presentations and the management that is currently available. PMID:20228456

Vannemreddy, Prasad; Nourbakhsh, Ali; Willis, Brian; Guthikonda, Bharat

2010-01-01

40

Multiple cerebral arteriovenous malformations (AVMs)  

Microsoft Academic Search

From our series of 203 patients with cerebral vascular lesions, 18 (9%) could be included in the multiple arteriovenous malformation category. There were five patients with Rendu-Osler-Weber, one with Wyburn-Mason syndromes and two with concurrent arteriovenous malformations. The remaining ten patients (4%) had multiple brain arteriovenous malformations. Careful angiography with magnification is necessary to try to diagnose multiple brain AVMs,

R. A. Willinsky; P. Lasjaunias; K. Terbrugge; P. Burrows

1990-01-01

41

Ear Injuries (For Parents)  

MedlinePLUS

... They also might need listening therapy with an audiologist (hearing specialist). Make sure to call your doctor ... ear, nose, and throat specialist and possibly an audiologist to figure out the next step to take. ...

42

Ear Infections in Children  

MedlinePLUS

... to the middle ear, drain fluid, and keep air pressure at a steady level between the nose and ... behind it. Tympanometry, which uses sound tones and air pressure, is a diagnostic test a doctor might use ...

43

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

44

Anencephaly and its Associated Malformations  

PubMed Central

Introduction: Anencephaly is a serious neural tube defect in which parts of the brain and skull are not developed. But apart from this it is also associated with other malformations which are not related to neural tube in development. Aim: The present study was undertaken to find the associated congenital malformations in western region of India and establish a aetiological correlation. Materials and Methods: The study was conducted using 20 anencephalic fetuses. Results: Nearly 80% of fetuses had associated malformations. Spina bifida was seen in 9 fetuses and cleft palate in 8. Female fetus with cleft palate had other severe associated gastrointestinal and skeletal malformation. Conclusion: In cases of anencephaly other associated malfor–mations like spina bifida and cleft palate are commonly seen. PMID:25386414

Meshram, Pritee Madan; Hattangdi, Shanta Sunil

2014-01-01

45

Middle Ear Infections and Ear Tube Surgery (For Parents)  

MedlinePLUS

... the nose — called the eustachian tube — equalizes the air pressure between the middle ear and the outside world. ( ... or swallowing, the eustachian tubes are adjusting the air pressure in the middle ears.) Infection Bacteria or viruses ...

46

Digital ear scanner : measuring the compliance of the ear  

E-print Network

This paper seeks to resolve the biggest problem with hearing aids, their physical fit. By digitally scanning the ear canal and taking the dynamics of the ear into account the performance and comfort of a hearing aid can ...

Hernandez-Stewart, Daniel

2010-01-01

47

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

48

Brain arteriovenous malformations.  

PubMed

An arteriovenous malformation (AVM) is a particular abnormality of blood vessels. Brain AVMs are congenital, but symptoms usually do not appear until the second decade of life - if at all. The most common presenting symptom is a brain hemorrhage, but other possible symptoms include neurological deficits, seizures and headaches. Until recently, the gold standard for diagnosing AVM was conventional angiography. However, computed tomography and magnetic resonance angiography are now the first-line diagnostic tools for AVMs. This article reviews the presenting symptoms, diagnostic procedures and treatment options for brain AVMs, including embolization, micro-surgery and radiosurgery. This article is a Directed Reading. Your access to Directed Reading quizzes for continuing education credit is determined by your CE preference. For access to other quizzes, go to www.asrt.org/store. PMID:21771938

Ferrara, Adi R

2011-01-01

49

Radiosurgery for arteriovenous malformations.  

PubMed

Stereotactic radiosurgery is the term coined by Lars Leksell to describe the application of a single, high dose of radiation to a stereotactically defined target volume. In the 1970s, reports began to appear documenting the successful obliteration of arteriovenous malformations (AVMs) with radiosurgery. When an AVM is treated with radiosurgery, a pathologic process appears to be induced that is similar to the response-to-injury model of atherosclerosis. Radiation injury to the vascular endothelium is believed to induce the proliferation of smooth-muscle cells and the elaboration of extracellular collagen, which leads to progressive stenosis and obliteration of the AVM nidus thereby eliminating the risk of hemorrhage. The advantages of radiosurgery - compared to microsurgical and endovascular treatments - are that it is noninvasive, has minimal risk of acute complications, and is performed as an outpatient procedure requiring no recovery time for the patient. The primary disadvantage of radiosurgery is that cure is not immediate. While thrombosis of the lesion is achieved in the majority of cases, it commonly does not occur until two or three years after treatment. During the interval between radiosurgical treatment and AVM thrombosis, the risk of hemorrhage remains. Another potential disadvantage of radiosurgery is possible long term adverse effects of radiation. Finally, radiosurgery has been shown to be less effective for lesions over 10 cc in volume. For these reasons, selection of the optimal treatment for an AVM is a complex decision requiring the input of experts in endovascular, open surgical, and radiosurgical treatment. In the pages below, we will review the world's literature on radiosurgery for AVMs. Topics reviewed will include the following: radiosurgical technique, radiosurgery results (gamma knife radiosurgery, particle beam radiosurgery, linear accelerator radiosurgery), hemorrhage after radiosurgery, radiation induced complications, repeat radiosurgery, and radiosurgery for other types of vascular malformation. PMID:22004703

Friedman, William A; Bova, Frank J

2011-10-01

50

Management strategy for facial venous malformations  

PubMed Central

Venous malformations (VMs) are slow-flow vascular malformations, caused by abnormalities in the development of the veins. Venous malformations vary in size and location within the body. When the skin or tissues just under the skin are affected, they appear as slightly blue-colored skin stains or swellings. These can vary in size from time to time because of swelling within the malformation. As these are vascular malformations, they are present at birth and grow proportionately with the child. Venous malformations can be very small to large in size, and sometimes, can involve a significant area within the body, When the venous malformation is well localized, this may cause localized swelling, however, when the venous malformation is more extensive, there may be more widespread swelling of the affected body part. Some patients with venous malformations have abnormal blood clotting within the malformation. Most venous malformations cause no life-threatening problems for patients. Some venous malformations cause repeated pain due to intermittent swelling and congestion of the malformation or due to the formation of blood clots within the malformation. Rarely, venous malformations may be part of a syndrome (an association of several clinically recognizable features) or be linked to an underlying genetic abnormality. We present 12 cases of venous malformations of the head and neck area, which have been managed at our hospital.

Kumar, Shailendra; Kumar, Vijay; Kumar, Sanjeev; Kumar, Surender

2014-01-01

51

Hearing, Ear Infections, and Deafness  

MedlinePLUS

... Health Info Hearing, Ear Infections, and Deafness DefaultPage Hearing, Ear Infections, and Deafness Diseases and Conditions Age- ... for parents Communication Methods & Devices for People With Hearing Loss American Sign Language Assistive Devices for People ...

52

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

53

From Ear to Brain  

ERIC Educational Resources Information Center

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

Kimura, Doreen

2011-01-01

54

Middle Ear Infections (For Parents)  

MedlinePLUS

... the eardrum. There's no single best approach for treating all middle ear infections. In deciding how to manage your child's ear infection, ... to large groups of other kids, such as in child-care centers. Because multiple upper respiratory infections may also lead to frequent ear infections, ...

55

Embolization of uterine arteriovenous malformation  

PubMed Central

Background: Uterine arteriovenous malformation is a rare but potential life-threatening source of bleeding. A high index of suspicion and accurate diagnosis of the condition in a timely manor are essential because instrumentation that is often used for other sources of uterine bleeding can be lead to massive hemorrhage. Case: We describe here a case of uterine arteriovenous malformation. A 32-year-old woman presented abnormal vaginal bleeding following the induced abortion. A diagnosis of uterine arteriovenous malformation made on the basis of Doppler ultrasonraphy was confirmed through pelvic angiography. The embolization of bilateral uterine arteries was performed successfully. Conclusion: Uterine arteriovenous malformation should be suspected in patient with abnormal vaginal bleeding, especially who had the past medical history incluing cesarean section, induced abortion, or Dillation and Curethage and so on. Although angiography remains the gold standard, Doppler ultrasonography is also a good noninvasive technique. The transcatheter uterine artery embolization offers a safe and effective treatment PMID:24639742

Chen, Yan; Wang, Guoyun; Xie, Fubo; Wang, Bo; Tao, Guowei; Kong, Beihua

2013-01-01

56

Cerebral malformations without antenatal diagnosis  

Microsoft Academic Search

Cerebral malformations are usually described following the different steps in development. Disorders of neurulation (dysraphisms),\\u000a or diverticulation (holoprosencephalies and posterior fossa cysts), and total commissural agenesis are usually diagnosed in\\u000a utero. In contrast, disorders of histogenesis (proliferation-differentiation, migration, organization) are usually discovered\\u000a in infants and children. The principal clinical symptoms that may be a clue to cerebral malformation include congenital

Nadine J. Girard

2010-01-01

57

Cerebral Cavernous Malformations: Surgical Perspective  

Microsoft Academic Search

\\u000a Cerebral cavernous malformations (CCMs) are rare vascular lesions [1], [2]; however, because hemorrhage of CCMs can result in significant morbidity and mortality [3], [4], a great deal of effort has been devoted to their detection and treatment. Although previously thought to be solely congenital,\\u000a it is now well recognized that many cavernous malformations are acquired [5], arising de novo or

Robert L. Dodd; Gary K. Steinberg

58

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

59

Pulmonary arteriovenous malformations.  

PubMed

Pulmonary arteriovenous malformations (PAVMs) are abnormal vascular structures that most often connect a pulmonary artery to a pulmonary vein, bypassing the normal pulmonary capillary bed and resulting in an intrapulmonary right-to-left shunt. As a consequence, patients with PAVM can have hypoxemia and paradoxical embolization complications, including stroke and brain abscess. PAVMs may be single or multiple, unilateral or bilateral, and simple or complex. Most PAVMs are hereditary and occur in hereditary hemorrhagic telangiectasia, an autosomal dominant vascular disorder, and screening for PAVM is indicated in this subgroup. PAVMs may also be idiopathic, occur as a result of trauma and infection, or be secondary to hepatopulmonary syndrome and bidirectional cavopulmonary shunting. Diagnostic testing involves identifying an intrapulmonary shunt, with the most sensitive test being transthoracic contrast echocardiography. Chest CT scan is useful in characterizing PAVM in patients with positive intrapulmonary shunting. Transcatheter embolotherapy is the treatment of choice for PAVM. Lifelong follow-up is important because recanalization and collateralization may occur after embolization therapy. Surgical resection is rarely necessary and reserved for patients who are not candidates for embolization. Antibiotic prophylaxis for procedures with a risk of bacteremia (eg, dental procedures) is recommended in all patients with PAVM because of the risk of cerebral abscess. PMID:24008954

Cartin-Ceba, Rodrigo; Swanson, Karen L; Krowka, Michael J

2013-09-01

60

Pediatric dural arteriovenous malformations.  

PubMed

Pediatric dural arteriovenous malformations (dAVMs) are rare lesions that have a high mortality rate and require complex management. The authors report 3 cases of pediatric dAVMs that presented with macrocrania and extracranial venous distension. Dural sinus thrombosis developed in 2 of the cases prior to any intervention, which is an unusual occurrence for this particular disease. All 3 cases were treated using staged endovascular embolization with a favorable outcome in 1 case and a poor outcome in the other 2 cases. Complications developed in all cases and included dural sinus thrombosis, parenchymal hemorrhage, intracranial venous hypertension, and seizures. The strategies and challenges used in managing these patients will be presented and discussed, along with a review of the literature. While outcomes remain poor, the authors conclude that prompt treatment with endovascular embolization provides the best results for children with these lesions. A well-established venous collateral circulation draining directly to the internal jugular veins may further improve the rate of favorable outcome after embolization. PMID:24867128

Appaduray, Shaun P; King, James A J; Wray, Alison; Lo, Patrick; Maixner, Wirginia

2014-07-01

61

Embolization of Arteriovenous Malformation  

PubMed Central

Summary Treatment options for cerebral arteriovenous malformation (AVM) are still controversial due to the recent result of stereotactic radiosurgery and the improved result of microsurgical resection. We investigated previously treated AVM cases and discussed the efficacy and safety of preoperative embolization especially for microsurgical resection of high-grade AVM in the Spetzler-Martin grading. Efficacy of preoperative embolization was evaluated based on 126 previously treated AVM cases at Shinshu University Hospital during the last 25 years. The safety of embolization was evaluated based on our previously-embolized 58 AVM cases (91 procedures) in the last 11 years after introduction of preoperative embolization for AVM. In all 126 cases, 82 were treated before introduction of embolization and 44 were treated after introduction of embolization. In 82 cases of the pre-embolization era, 63 lesions were removed totally in 63 AVMs (77%), partially resected in 11 (13%) and untreated in eight (10%). In 74 surgically removed cases, 11 (15%) cases showed severe intra/postoperative bleeding. In 44 cases of the embolization era, lesions were removed totally in 29 AVMs (66%), disappeared only with embolization in one (2%), disappeared with radiosurgery in seven (16%) and were untreated in five (11%). In 32 surgically removed cases, only one (2%) case showed severe intra/postoperative bleeding. In all 58 embolized cases, 44 were surgically removed, six were treated with radiosurgery, one was eliminated with embolization alone and six were partially obliterated and followed up for their location. In 91 procedures for 58 cases, two haemorrhagic and three ischemic complications occurred, three were transient and two remained having neurological deficits. The introduction of preoperative embolization improved the total removal rate and reduced the intra/postoperative bleeding rate in surgical removal of AVM. The total risk of embolization is low and well-designed preoperative embolization makes surgical resection safer even in high-grade AVM in the Spetzler-Martin grading. PMID:20587250

Nagashima, H.; Hongo, K.; Kobayashi, S.; Takamae, T.; Okudera, H.; Koyama, J.I.; Oya, F.; Matsumoto, Y.

2004-01-01

62

[Arteriovenous malformation in the mandibula].  

PubMed

A 15-year-old autistic boy of subnormal intelligence appeared at the office of his dental practitioner complaining about pain in the third quadrant. Investigation revealed that the second molar could be intruded easily. No other irregularities, such as caries or periodontal disease were apparent. Due to the severe pain it was decided that the tooth should be extracted. Extraction of the tooth induced massive bleeding. Hemostasis was achieved by repositioning the molar in its alveolus, according to the principle of putting a cork in a bottle. Radiographic investigation showed the presence of an intraosseous arteriovenous malformation. These malformations are potentially life-threatening lesions. Treatment of choice is a combination of transarterial embolization and surgical removal of the malformation. PMID:17715775

Kruizinga, E H W; Meijer, G J; Koole, R; van Es, R J J

2007-07-01

63

Glioblastoma Mimicking an Arteriovenous Malformation  

PubMed Central

Abnormal cerebral vasculature can be a manifestation of a vascular malformation or a neoplastic process. We report the case of a patient with angiography-negative subarachnoid hemorrhage (SAH) who re-presented 3?years later with a large intraparenchymal hemorrhage. Although imaging following the intraparenchymal hemorrhage was suggestive of arteriovenous malformation, the patient was ultimately found to have an extensive glioblastoma associated with abnormal tumor vasculature. The case emphasizes the need for magnetic resonance imaging to investigate angiography-negative SAH in suspicious cases to rule out occult etiologies, such as neoplasm. We also discuss diagnostic pitfalls when brain tumors are associated with hemorrhage and abnormal vasculature. PMID:24137154

Khanna, Arjun; Venteicher, Andrew S.; Walcott, Brian P.; Kahle, Kristopher T.; Mordes, Daniel A.; William, Christopher M.; Ghogawala, Zoher; Ogilvy, Christopher S.

2013-01-01

64

Glioblastoma mimicking an arteriovenous malformation.  

PubMed

Abnormal cerebral vasculature can be a manifestation of a vascular malformation or a neoplastic process. We report the case of a patient with angiography-negative subarachnoid hemorrhage (SAH) who re-presented 3?years later with a large intraparenchymal hemorrhage. Although imaging following the intraparenchymal hemorrhage was suggestive of arteriovenous malformation, the patient was ultimately found to have an extensive glioblastoma associated with abnormal tumor vasculature. The case emphasizes the need for magnetic resonance imaging to investigate angiography-negative SAH in suspicious cases to rule out occult etiologies, such as neoplasm. We also discuss diagnostic pitfalls when brain tumors are associated with hemorrhage and abnormal vasculature. PMID:24137154

Khanna, Arjun; Venteicher, Andrew S; Walcott, Brian P; Kahle, Kristopher T; Mordes, Daniel A; William, Christopher M; Ghogawala, Zoher; Ogilvy, Christopher S

2013-01-01

65

Arteriovenous malformation of the pancreas.  

PubMed

Pancreatic arteriovenous malformation (PAVM) is a very rare and mostly congenital lesion, with less than 80 cases described in the English-published literature. It is defined as a tumorous vascular abnormality that is constructed between an anomalous bypass anastomosis of the arterial and venous networks within the pancreas. It represents about 5% of all arteriovenous malformations found in the gastrointestinal tract. Herein, we present a 64-year-old patient with symptomatic PAVM involving the body and tail of the organ, which was successfully treated by transcatheter arterial embolization. The disease spectrum and review of the literature are also presented. PMID:21765845

Charalabopoulos, Alexandros; Macheras, Nikolas; Krivan, Sylvia; Petropoulos, Konstantinos; Misiakos, Evangelos; Macheras, Anastasios

2011-01-01

66

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

67

Radiosurgery for Cerebral Arteriovenous Malformations  

Microsoft Academic Search

From September 1991 to May 1997, a total of 59 patients diagnosed with cerebral arteriovenous malformations were treated using radiosurgery. There were 29 men and 30 women, with an age range of 5 to 75 years (mean 36). Of these patients, 39 patients were treated using a LINAC-based system (group 1) and 20 using the Leksell ?-knife unit (group 2).

Lucia Zamorano; Amgad Matter; Laurie Caspar; Arturo Saenz; Razvan Buciuc; James Fontanesi; Azucena Garzon; Fernando Diaz

1998-01-01

68

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

69

Otoscopic exam of the ear (image)  

MedlinePLUS

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

70

Omphalocele and gastroschisis and associated malformations.  

PubMed

The etiology of gastroschisis and omphalocele is unclear and their pathogenesis is controversial. Because previous reports have inconsistently noted the type and frequency of malformations associated with omphalocele and gastroschisis, we assessed these associated malformations ascertained between 1979 and 2003 in 334,262 consecutive births. Of the 86 patients with omphalocele, 64 (74.4%) had associated malformations. These included patients with chromosomal abnormalities (25, 29.0%); non-chromosomal syndromes including Beckwith-Wiedemann syndrome, Goltz syndrome, Marshall-Smith syndrome, Meckel-Gruber syndrome, Oto-palato-digital type II syndrome, CHARGE syndrome, and fetal valproate syndrome; malformation sequences, including ectopia cordis, body stalk anomaly, exstrophy of bladder, exstrophy of cloaca, and OEIS (Omphalocele, Exstrophy of bladder, Imperforate anus, Spinal defect); malformation complexes including Pentalogy of Cantrell, and non-syndromic multiple congenital anomalies (MCA) (26, 30.2%). Malformations of the musculoskeletal system (31, 23.5%), urogenital system (27, 20.4%), cardiovascular system (20, 15.1%), and central nervous system (12, 9.1%) were the most common other congenital malformations in patients with omphalocele and non-syndromic MCA. Of the 60 patients with gastroschisis, 10 (16.6%) had associated malformations. In contrast to omphalocele, gastroschisis was rarely associated with a complex pattern of malformation, that is, one each (1.7%) with a chromosomal abnormality (trisomy 21), sequence (amyoplasia congenita), unspecified dwarfism, and 7 (11.7%) with MCA. We observed a striking difference in the prevalence of total malformations (74.4% vs. 16.6%, P < 0.001) and specific patterns of malformations associated with omphalocele and gastroschisis which emphasizes the need to evaluate all patients with omphalocele and gastroschisis for possible associated malformations. Malformation surveillance programs should be aware that the malformations associated with omphalocele can be often classified into a recognizable malformation syndrome or pattern (44.2%). PMID:18386803

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

2008-05-15

71

Arteriovenous Malformation of the Oral Cavity  

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 tumors. Lymphatic, capillary, venous, and arteriovenous malformations make up the majority of vascular malformations. Arteriovenous malformation of the head and neck is a rare vascular anomaly but when present is persistent and progressive in nature and can represent a lethal benign disease. Here we present a case report of a 25-year-old male patient with arteriovenous malformation involving the base of tongue. PMID:24660070

Manjunath, S. M.; Shetty, Sujan; Moon, Ninad J.; Metta, Kiran Kumar; Gupta, Nitin; Goyal, Sandeep

2014-01-01

72

Successful Bilateral Composite Ear Reattachment  

PubMed Central

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

2014-01-01

73

AUTISM WITH OPHTHALMOLOGIC MALFORMATIONS: THE PLOT THICKENS  

PubMed Central

ABSTRACT Purpose To review the association of autism spectrum disorder (ASD) in individuals manifesting thalidomide embryopathy and Möbius sequence and compare them with three new studies in which ASD was also associated with ocular and systemic malformations: (1) a Swedish study of individuals with CHARGE association (Coloboma, Heart, choanal Atresia, developmental or growth Retardation, Genital anomaly, and Ear involvement); (2) a Swedish study of Goldenhar syndrome; and (3) Brazilian Möbius syndrome (sequence) study. Methods In the Swedish CHARGE study, 31 patients met the inclusion criteria (3+ or 4 of the common characteristics of the CHARGE syndrome). The same team of investigators also evaluated 20 Swedish patients with Goldenhar syndrome. In the Brazilian Möbius study, 28 children with a diagnosis of Möbius sequence were studied; some children had a history of exposure during their mother’s pregnancy to the abortifacient drug misoprostol in an unsuccessful abortion attempt Results In the CHARGE study, five patients had the more severe autism disorder and five had autistic-like condition. In the Goldenhar study, two had autism disorder and one had autistic-like condition. In the Brazilian Möbius study, the systemic findings of the misoprostol-exposed and misoprostol-unexposed patients were almost undistinguishable, and ASD was present in both groups (autism disorder in five and autistic-like condition in three). Conclusion Autism spectrum disorder has been reported in two conditions with known early pregnancy exposure to the teratogenic agents thalidomide and misoprostol. In the Brazilian Möbius study, autism also occurred in both the misoprostol-exposed and misoprostol-unexposed groups. Autism also was present in patients with both CHARGE association and Goldenhar syndrome. PMID:15747750

Miller, Marilyn T; Stromland, Kerstin; Ventura, Liana; Johansson, Maria; Bandim, Jose M; Gillberg, Christopher

2004-01-01

74

Cerebellar arteriovenous malformations in children  

Microsoft Academic Search

We review the presentation, imaging findings and outcome in 18 children with cerebellar arteriovenous malformations (AVM).\\u000a This group is of particular interest because of the reported poor outcome despite modern imaging and neurosurgical techniques.\\u000a All children had CT and 15 underwent catheter angiography at presentation. Several of the children in the latter part of the\\u000a study had MRI. Of the

P. D. Griffiths; S. Blaser; D. Armstrong; S. Chuang; R. P. Humphreys; D. Harwood-Nash

1998-01-01

75

Arteriovenous malformations in the brain  

Microsoft Academic Search

Opinion statement  Arteriovenous malformations (AVM) are a leading cause of intracerebral hemorrhage, especially among the young. Because they\\u000a pose a lifelong risk of serious bleeding, definitive treatment to obliterate the AVM should be pursued in the majority of\\u000a patients. Microsurgical resection of a small AVM located in the superficial or noneloquent brain achieves high cure rates\\u000a with low morbidity, and is

Glenn D. Graham

2002-01-01

76

Cerebral arteriovenous malformations in children  

Microsoft Academic Search

The treatment of cerebral arteriovenous malformations (AVM) or vascular anomalies are challenging neurosurgical procedures\\u000a for an anaesthetist. Large AVMs are uncommon in children. Only 18% of AVMs become symptomatic before the age of 15 yr. This\\u000a series reviews the experience at this institution during the period of 1982 to 1992. The symptoms at the time of presentation\\u000a are varied and

Carol Millar; Bruno Bissonnette; R. P. Humphreys

1994-01-01

77

Endovascular Treatment of Arteriovenous Malformation  

Microsoft Academic Search

Vascular anomalies are common congenital or neonatal abnormalities. According to the approved classification of vascular lesions\\u000a by Glowacki and Mulliken, hemangiomas and vascular malformations are distinguishable. Hemangiomas usually appear during the\\u000a first days or weeks after birth and grow faster than the whole body of the infant. They are proliferating benign tumors that\\u000a often involute. The opposite of hemangiomas, vascular

Robert Juszkat; Bartosz ?abicki; Pawe? Ch?ci?ski; Marcin Gabriel; Nawal Matar

2009-01-01

78

Otoacoustic Emissions from a Nonvertebrate Ear  

Microsoft Academic Search

Otoacoustic emissions are produced by the inner ear of vertebrates and result from the active and nonlinear processing of input sound by sensory hair cells. We recorded pronounced distortion-product otoacoustic emissions from the ear of the grasshopper, and these emissions proved remarkably similar to those described for the mammalian ear. This is despite the fact that the grasshopper ear is

M. Kössl; G. S. Boyan

1998-01-01

79

What Is an Ear Infection?  

MedlinePLUS

... For Kids For Parents MORE ON THIS TOPIC Movie: Ears Your ... Nemours Web site. Note: All information on KidsHealth® is for educational purposes only. For specific medical advice, diagnoses, and ...

80

Ototoxicity (Ear Poisoning) (For Parents)  

MedlinePLUS

... may recommend that your child regularly see an audiologist (hearing specialist) or vestibular therapist (someone trained in ... or balance abilities, or refer you to an audiologist or otolaryngologist (ear, nose, and throat specialist) for ...

81

[Pulmonary arteriovenous malformation: two sibling cases].  

PubMed

Pulmonary arteriovenous malformations, are abnormal connections between pulmonary arteries and veins. However it can be presented as an isolated single anomaly, also may be multiple when accompanying with autosomal dominant hereditary hemorrhagic telengiectasia (Rendu-Osler-Weber Syndrome; ROWS). In this case report, two patients operated with the diagnosis of multiple pulmonary arteriovenous malformation familial screening done with the possibility of ROWS and pulmonary arteriovenous malformation found in her sister, are presented. PMID:21554236

Yenigün, Bülent Mustafa; Yüksel, Cabir; Enön, Serkan; Kay? Cang?r, Ayten; Atasoy, Kayhan Cetin

2011-01-01

82

Genetics Home Reference: Cerebral cavernous malformation  

MedlinePLUS

... Information Center Information about genetic conditions and rare diseases Additional NIH Resources ... cavernous malformations are collections of small blood vessels (capillaries) in the brain that are enlarged and ...

83

Craniosynostosis, hydrocephalus, Chiari I malformation and radioulnar synostosis: probably a new syndrome.  

PubMed

We report on clinical and molecular findings of two brothers that both presented with sagittal craniosynostosis, hydrocephalus, Chiari I malformation, blepharophimosis, small low-set ears, hypoplastic philtrum, radioulnar synostosis, kidney malformation, and hypogenitalism. Their father presented mild brachydactyly. Conventional cytogenetic and array CGH screening did not show any chromosomal gains or losses. Furthermore, molecular genetic screening of genes involved in different craniosynostosis syndromes, namely FGFR1, FGFR2, FGFR3, TWIST, RECQL4, and POR genes failed to detect any mutations in genomic DNA. The unique range of clinical manifestations in these two patients and the negative findings of the molecular genetic screening suggest the hypothesis of a previously unrecognized syndrome. PMID:19022412

Capra, Valeria; De Marco, Patrizia; Merello, Elisa; Baffico, Ave Maria; Baldi, Maurizia; Divizia, Maria Teresa; Gimelli, Stefania; Mallet, Delphine; Raso, Alessandro; Mascelli, Samantha; Tomŕ, Paolo; Rossi, Andrea; Pavanello, Marco; Cama, Armando; Magnani, Cinzia

2009-01-01

84

Management of pulmonary arteriovenous malformations.  

PubMed

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

2011-03-01

85

Daily fluctuations of middle ear pressure in atelectatic ears.  

PubMed

The position of the drum of 84 atelectatic ears, of patients 5 to 79 years old, was examined with the help of the operating microscope at different times of the day. All ears were found to be atelectatic during the daytime, yet on the patients' awakening in the morning, 37.73% of the drums of the adolescents' and adults' ears were found to be inflated, usually even hyperinflated. None of the children showed an inflated eardrum in the morning. All eardrums that were inflated in the morning returned to their original atelectatic position within an average of 54.56 minutes after awakening. This observation illustrates another fluctuating aspect of the atelectatic condition. PMID:2310136

Luntz, M; Sadé, J

1990-03-01

86

Aspects cliniques des malformations capillaires  

Microsoft Academic Search

Capillary malformations (CM) are defects of the dermal capillary bed. These slow-flow malformations can affect any part of the body and are always lateralized, despite Unna's naevus. Present at birth, they grow proportionally with the child. In rare instance, they can be part of a more complex syndrome such as Sturge-Weber syndrome. Ectatic CMs of telangiectatic types can be cutaneous,

A.-C. Bataille; L.-M. Boon

2006-01-01

87

Chiari II Malformation and Occult Spinal Dysraphism  

Microsoft Academic Search

We report two cases of children with occult spinal dysraphism who were also found to have many associated brain anomalies seen in the Chiari II malformation. No previous report has commented on the possible association between the Chiari II malformation and the ‘closed’ form of neural tube defect. One child had symptoms referable to pathology at the craniocervical junction. Neither

R. Shane Tubbs; John C. Wellons III; Paul A. Grabb; W. Jerry Oakes

2003-01-01

88

[Ureteral triplication whitout other associates malformations].  

PubMed

We report a case of ureteral type III triplication according to Smith's classification, without associated anomalies of the urinary tract. The revision of the bibliography indicates the rarity of this maldformacion and its frequent association with other urinary and extraurinary malformations. The absence of associate malformations in this case increases his exceptionality. PMID:19537075

Diz Rodríguez, Ramón; Arance Gil, Ignacio; Vírseda Chamorro, Miguel; Quijano Barroso, Pablo; Alpuente Román, Carlos; Sáenz Benito, Domingo

2009-03-01

89

The Chiari II Malformation: A Surgical Series  

Microsoft Academic Search

Thirty patients between the ages of 7 months and 24 years were treated surgically for symptomatic Chiari II malformation at the Arkansas Children’s Hospital. All patients underwent at least bony decompression of the malformation. Assessments of the patients’ conditions were made at 6 weeks and 1 year after surgery, and complications of surgery were noted. For a majority of the

Charles Teo; Erik C. Parker; Sharon Aureli; Frederick A. Boop

1997-01-01

90

Congenital bronchopulmonary foregut malformations: concepts and controversies  

Microsoft Academic Search

This article addresses the scope, etiology, important associations and imaging features of congenital bronchopulmonary foregut malformations. Etiologic concepts, including airway obstruction and vascular anomalies, are highlighted. Technical imaging advances, especially CT and MR, have greatly enhanced our diagnostic abilities in evaluating these lesions; however, thorough and careful assessment of all aspects of the malformation is still necessary. Several specific lesions

Beverley Newman

2006-01-01

91

Ear disorders in scuba divers.  

PubMed

History of underwater diving dates back to antiquity. Breath-hold technique in diving was known to the ancient nations. However, deep diving progressed only in the early decades of the 19th century as the result of advancements in efficient underwater technologies which subsequently led to invention of sophisticated sets of scuba diving in the 20th century. Currently, diving is performed for various purposes including commercial, recreational, military, underwater construction, oil industry, underwater archeology and scientific assessment of marine life. By increasing popularity of underwater diving, dive-related medical conditions gradually became more evident and created a new challenge for the health care professionals, so that eventually, a specialty the so-called "diving medicine" was established. Most of the diving-associated disorders appear in the head and neck. The most common of all occupational disorders associated with diving are otologic diseases. External otitis has been reported as the most common otolaryngologic problem in underwater divers. Exostosis of the external ear canal may be formed in divers as the result of prolonged diving in cold waters. Other disorders of the ear and paranasal sinuses in underwater divers are caused by barometric pressure change (i.e., barotraumas), and to a lesser extent by decompression sickness. Barotrauma of the middle ear is the most prevalent barotrauma in divers. The inner ear barotraumas, though important, is less common. The present paper is a brief overview of diving-related ear disorders particularly in scuba divers. PMID:23022815

Azizi, M H

2011-01-01

92

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

93

3D Printed Bionic Ears  

PubMed Central

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 precise 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.

2013-01-01

94

[Ear diseases and diabetes mellitus].  

PubMed

In the literature there is very little information about the importance of diabetes mellitus for ear diseases. Therefore, this article gives an overview about contact points between endocrinological and otological problems in patients with diseases of the ear and pre-existing diabetes mellitus. In particular the focus is on the impact of diabetes mellitus on patients with external otitis, malignant external otitis, otitis media, sudden sensorineural hearing loss and slowly progressive hearing loss. Diseases, such as osteomyelitis of the lateral skull base, sudden and slowly progressive hearing loss are also discussed in detail. PMID:25370360

Avetisyan, N; Lautermann, J

2014-11-01

95

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

96

Mutations in KCTD1 Cause Scalp-Ear-Nipple Syndrome  

PubMed Central

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.

2013-01-01

97

Coffee May Keep Your Ears from Ringing  

MedlinePLUS

... sharing features on this page, please enable JavaScript. Coffee May Keep Your Ears From Ringing Study found ... FRIDAY, Aug. 8, 2014 (HealthDay News) -- Being a coffee lover may be good for your ears, a ...

98

Neurosensory Development in the Zebrafish Inner Ear  

E-print Network

The vertebrate inner ear is a complex structure responsible for hearing and balance. The inner ear houses sensory epithelia composed of mechanosensory hair cells and non-sensory support cells. Hair cells synapse with neurons of the VIIIth cranial...

Vemaraju, Shruti

2012-02-14

99

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...

2011-04-01

100

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...

2014-04-01

101

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...

2013-04-01

102

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...

2012-04-01

103

Inner Ear Drug Delivery for Auditory Applications  

PubMed Central

Many inner ear disorders cannot be adequately treated by systemic drug delivery. A blood-cochlear barrier exists, similar physiologically to the blood-brain barrier, which limits the concentration and size of molecules able to leave the circulation and gain access to the cells of the inner ear. However, research in novel therapeutics and delivery systems has led to significant progress in the development of local methods of drug delivery to the inner ear. Intratympanic approaches, which deliver therapeutics to the middle ear, rely on permeation through tissue for access to the structures of the inner ear, whereas intracochlear methods are able to directly insert drugs into the inner ear. Innovative drug delivery systems to treat various inner ear ailments such as ototoxicity, sudden sensorineural hearing loss, autoimmune inner ear disease, and for preserving neurons and regenerating sensory cells are being explored. PMID:18848590

Swan, Erin E. Leary; Mescher, Mark J.; Sewell, William F.; Tao, Sarah L.; Borenstein, Jeffrey T.

2008-01-01

104

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

NSF Publications Database

Title : NSF 93-61 EAR Dear Colleague Letter Type : Letter NSF Org: GEO / EAR Date : May 12, 1993 ... of Earth Sciences (EAR) is 1 June 1993. This will be the second EAR proposal deadline since the ...

105

A Noncoding Point Mutation of Zeb1 Causes Multiple Developmental Malformations and Obesity in Twirler Mice  

PubMed Central

Heterozygous Twirler (Tw) mice develop obesity and circling behavior associated with malformations of the inner ear, whereas homozygous Tw mice have cleft palate and die shortly after birth. Zeb1 is a zinc finger protein that contributes to mesenchymal cell fate by repression of genes whose expression defines epithelial cell identity. This developmental pathway is disrupted in inner ears of Tw/Tw mice. The purpose of our study was to comprehensively characterize the Twirler phenotype and to identify the causative mutation. The Tw/+ inner ear phenotype includes irregularities of the semicircular canals, abnormal utricular otoconia, a shortened cochlear duct, and hearing loss, whereas Tw/Tw ears are severely malformed with barely recognizable anatomy. Tw/+ mice have obesity associated with insulin-resistance and have lymphoid organ hypoplasia. We identified a noncoding nucleotide substitution, c.58+181G>A, in the first intron of the Tw allele of Zeb1 (Zeb1Tw). A knockin mouse model of c.58+181G>A recapitulated the Tw phenotype, whereas a wild-type knockin control did not, confirming the mutation as pathogenic. c.58+181G>A does not affect splicing but disrupts a predicted site for Myb protein binding, which we confirmed in vitro. In comparison, homozygosity for a targeted deletion of exon 1 of mouse Zeb1, Zeb1?Ex1, is associated with a subtle abnormality of the lateral semicircular canal that is different than those in Tw mice. Expression analyses of E13.5 Twirler and Zeb1?Ex1 ears confirm that Zeb1?Ex1 is a null allele, whereas Zeb1Tw RNA is expressed at increased levels in comparison to wild-type Zeb1. We conclude that a noncoding point mutation of Zeb1 acts via a gain-of-function to disrupt regulation of Zeb1Tw expression, epithelial-mesenchymal cell fate or interactions, and structural development of the inner ear in Twirler mice. This is a novel mechanism underlying disorders of hearing or balance. PMID:21980308

Kurima, Kiyoto; Hertzano, Ronna; Gavrilova, Oksana; Monahan, Kelly; Shpargel, Karl B.; Nadaraja, Garani; Kawashima, Yoshiyuki; Lee, Kyu Yup; Ito, Taku; Higashi, Yujiro; Eisenman, David J.; Strome, Scott E.; Griffith, Andrew J.

2011-01-01

106

Ultrastructure of the Insect Ear  

Microsoft Academic Search

LIGHT microscope studies1-4 have long established that the sensory unit (chordotonal sensillum or stiftführendes Organ) of the insect ear consists of three cells, namely, the bipolar neuron, the axon of which runs directly to the central nervous system, the sheath cell, which surrounds the dendrite of the neuron, and the attachment cell, which appears to anchor the whole assembly to

E. G. Gray

1958-01-01

107

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

108

21 CFR 870.2710 - Ear oximeter.  

Code of Federal Regulations, 2010 CFR

...2710 Ear oximeter. (a) Identification. An ear oximeter is an extravascular device used to transmit light at a known wavelength(s) through blood in the ear. The amount of reflected or scattered light as indicated by this device is used to...

2010-04-01

109

21 CFR 870.2710 - Ear oximeter.  

Code of Federal Regulations, 2011 CFR

...2710 Ear oximeter. (a) Identification. An ear oximeter is an extravascular device used to transmit light at a known wavelength(s) through blood in the ear. The amount of reflected or scattered light as indicated by this device is used to...

2011-04-01

110

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 ...

111

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

112

21 CFR 870.2710 - Ear oximeter.  

...2710 Ear oximeter. (a) Identification. An ear oximeter is an extravascular device used to transmit light at a known wavelength(s) through blood in the ear. The amount of reflected or scattered light as indicated by this device is used to...

2014-04-01

113

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.

Biomedical Engineering

114

[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

115

[Hemangiomas and vascular malformations. Review and update].  

PubMed

In 1982, Mulliken and Glowacki classificated congenital vascular lesions in two biological groups with different treatment, evolution and prognosis: Haemangiomas and Vascular malformations. We have seen 230 patients, 145 with haemangiomas and 85 with vascular malformations (74 with low flow and 11 with high flow). We have reviewed clinical, evolutive and ecographic findings. Depending on the type and localization of the lesion, we completed the study with CT, MRI and angiography. Among all the patients with haemangiomas; 45 were treated with local or systemic steroids because of bleeding, ulceration or affection in functional areas. In two more, we used interferon alfa 2a because of Kassabach-Merritt Syndrome. 23 were operated on with good aesthetic results. Patients with vascular malformations were managed with embolization, sclerotherapy, surgical approach or conservatively. Capillary malformations were treated with lasertherapy. We emphasize in correct classification of vascular lesions prior to start diagnosis, treatment or parents information. PMID:9376236

Lobato, R; Martínez, L; Leal, N; Díaz, M; Díez-Pascual, R; Velasco, B; Ros, Z; López-Gutiérrez, J C

1997-07-01

116

Inner ear deficits after chronic otitis media.  

PubMed

Investigation of the causes of vestibular symptoms in patients with chronic otitis media (COM) faces frustration, mainly because the bithermal caloric test using tap water is generally contraindicated in perforated ears. This study utilized audiometry, ocular vestibular-evoked myogenic potential (oVEMP) test, and cervical VEMP (cVEMP) test to evaluate inner ear deficits after COM. A total of 85 COM patients (117 ears) underwent otoscopy, image study, audiometry, oVEMP test, and cVEMP test. Mean bone-conducted (BC) hearing threshold ?25 dB was observed in 74 ears, 26-40 dB in 30 ears, and >40 dB in 13 ears. Restated, abnormal BC hearing threshold was identified in 43 ears (37 %). Percentages of abnormal cVEMP test, oVEMP test, and BC hearing threshold in 117 COM ears were 65, 62, and 37 %, respectively, exhibiting a significantly declining sequence in inner ear function. Furthermore, cVEMP/oVEMP test results were significantly correlated with BC hearing threshold, whereas no correlation existed between the cVEMP and oVEMP test results. In conclusion, the sequence of inner ear deficits after COM runs from the saccule/utricle to the cochlea and semicircular canals. Restated, in addition to BC hearing test, the cVEMP/oVEMP test may serve as a supplementary tool for early detection of inner ear involvement in COM patients. PMID:24061573

Chang, Chun-Wei; Cheng, Po-Wen; Young, Yi-Ho

2014-08-01

117

MR imaging of fetal brain malformations  

Microsoft Academic Search

MethodsFrom the generally accepted data on the morphogenesis of the brain, the principles for the classification of brain malformations are given, and the salient features of each malformation which may be considered as independent from the developmental stage and therefore practical for MR imaging in the fetus after mid-gestation, are discussed.Results and discussionHowever, the correlation with the clinical results in

Charles Raybaud; Olivier Levrier; Hervé Brunel; Nadine Girard; Philippe Farnarier

2003-01-01

118

Interdisciplinary management of craniofacial vascular malformations  

Microsoft Academic Search

Beside their sometimes unsightly appearance, vascular malformations of the head and neck area can lead to very severe functional\\u000a problems. Reduced field of vision and impairments in breathing and swallowing can occur and are serious problems to deal with.\\u000a During the last 4 years, 17 patients with vascular malformations of the head and neck region were treated in our department.\\u000a Initially,

H. Kubiena; M. Cejna; S. Kreuzer; E. Frey; M. Schoder; M. Frey

2007-01-01

119

Animal models for human craniofacial malformations.  

PubMed

Holoprosencephaly malformations, of which the fetal alcohol syndrome appears to be a mild form, can result from medial anterior neural plate deficiencies as demonstrated in an ethanol treated animal model. These malformations are associated with more medial positioning of the nasal placodes and resulting underdevelopment or absence of the medial nasal prominences (MNPs) and their derivatives. Malformations seen in the human retinoic acid syndrome (RAS) can be produced by administration of the drug 13-cis-retinoic acid in animals. Primary effects on neural crest cells account for most of these RAS malformations. Many of the malformations seen in the RAS are similar to those of hemifacial microsomia, suggesting similar neural crest involvement. Excessive cell death, apparently limited to trigeminal ganglion neuroblasts of placodal origin, follows 13-cis retinoic acid administration at the time of ganglion formation and leads to malformations virtually identical to those of the Treacher Collins syndrome (TCS). Secondary effects on neural crest cells in the area of the ganglion appear to be responsible for the TCS malformations. Malformations of the DiGeorge Syndrome are similar to those of the RAS and can be produced in mice by ethanol administration or by "knocking out" a homeobox gene (box 1.5). Human and animal studies indicate that cleft lips of multifactorial etiology may be generically susceptible because of small MNP)s or other MNP developmental alterations, such as those found in A/J mice, that make prominence contact more difficult. Experimental maternal hypoxia in mice indicates that cigarette smoking may increase the incidence of cleft lip by interfering with morphogenetic movements. Other human cleft lips may result from the action of a single major gene coding for TGF-alpha variants. A study with mouse palatal shelves in culture and other information suggest that a fusion problem may be involved. PMID:1812129

Johnston, M C; Bronsky, P T

1991-01-01

120

The War of Jenkins' Ear  

PubMed Central

Objective In 1731, Spanish sailors boarded the British brig Rebecca off the coast of Cuba and sliced off the left ear of its captain, Robert Jenkins. This traumatic auriculectomy was used as a pretext by the British to declare war on Spain in 1739, a conflict that is now known as the War of Jenkins’ Ear. Here, we examine the techniques available for auricular repair at the time of Jenkins’ injury and relate them to the historical events surrounding the incident. Methods Review of relevant original published manuscripts and monographs. Results Surgeons in the mid-18th century did not have experience with repair of traumatic total auriculectomies. Some contemporary surgeons favored auricular prostheses over surgical treatment. Methods for the reconstruction of partial defects were available, and most authors advocated a local post-auricular flap instead of a free tissue transfer. Techniques for repair of defects of the auricle lagged behind those for repair of the nose. Conclusion Limitations in care of traumatic auricular defects may have intensified the significance of Jenkins’ injury and helped lead to the War of Jenkins’ Ear, but conflict between Britain and Spain was probably unavoidable due to their conflicting commercial interests in the Caribbean. PMID:23444484

Graboyes, Evan M.; Hullar, Timothy E.

2012-01-01

121

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

122

Local Inner Ear Drug Delivery and Pharmacokinetics  

PubMed Central

Summary A number of drugs are in widespread clinical use for the treatment of inner ear disorders by applying them directly to the inner ear. Many new substances and drug delivery systems specific to the inner ear are under development, and in some cases are undergoing evaluations in animal experiments and in clinical studies. The pharmacokinetics of drugs in the inner ear, however, is not well defined and the field is plagued by technical problems in obtaining pure samples of the inner ear fluids for analysis. Nevertheless, a basic understanding of the mechanisms of drug dispersal in the inner ear has emerged that facilitates the design and interpretation of future pharmacokinetic studies. PMID:16214674

Salt, Alec N.; Plontke, Stefan K.R.

2008-01-01

123

[High flow vascular malformations in children].  

PubMed

Unlike hemangiomas and low-flow vascular malformations which are very common in children, arterial anomalies have small incidence. Differential diagnosis is difficult, and needs a physician familiarized with vascular anomalies. Appropriate treatment must be planned by multidisciplinary team considering the patient's age, and anatomical location. Twenty-eight children with high flow vascular malformations have been treated since 1990 at La Paz Children's Hospital Vascular Anomalies Program. We excluded of the study group patients with central nervous system lesions. 85% of the patients had malformation in stage I or II (according the ISSVA accepted Schöbinger stating) and most of them were erroneously diagnosed as hemangioms with a variety of inappropriate treatments previously performed. Doppler Ultrasound and Magnetic Resonance confirmed malformation flow and extension. Angiography and selective embolization was only considered as therapeutic approach in candidates to surgical resection. 16 patients underwent complete resection of the malformation including one foot and two fingers amputation and five more incomplete resection of the ulcerate area. In conclusion, we did not find age at onset, sex and symptoms relationship. Laser, radiotherapy, surgical ligation or partial resection must be considered inappropriate therapies which may stimulate AVM exacerbation. Only radical surgical procedure after selective endovascular embolization will be successful but then reconstructive surgery should be performed to achieve good aesthetic and functional results. PMID:12601971

López Gutiérrez, J C; Ros, Z; Martínez, L; Díaz, M; Leal, N; Rivas, S; Hernández, F

2002-10-01

124

Sensory innervation of the ear drum and middle-ear mucosa: Retrograde tracing and immunocytochemistry  

Microsoft Academic Search

The distribution and origin of nerve fibers of presumed sensory nature in the ear drum and middle-ear mucosa of the rat were studied by a retrograde tracing technique in combination with immunocytochemistry.

R. Uddman; T. Grunditz; A. Larsson; F. Sundler

1988-01-01

125

[Hypopharyngeal carcinoma and red ear drum].  

PubMed

A 46-year-old male patient with an unresectable hypopharyngeal carcinoma was treated with primary radio-chemotherapy. At follow-up, the patient presented with a red ear drum and combined hearing loss. Because of radiotherapy-induced tubal dysfunction, paracentesis was performed. Biopsy of the polypoid middle ear mucosa revealed petrous bone infiltration of hypopharyngeal carcinoma. MRI studies revealed paracarotideal tumor infiltration to the petrous bone and the middle ear arising from a cervical retropharyngeal lymph node metastasis. PMID:20963385

Bender, B; Widmann, G; Riechelmann, H; Schmutzhard, J

2011-04-01

126

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

127

Real-Ear to Coupler Difference in Patients with Ear Drum Perforation  

Microsoft Academic Search

The aim of this study is to investigate the effects of ear drum perforation on real-ear to coupler difference (RECD) in adults. RECD was measured using a probe tube microphone system in 22 patients with ear drum perforations. Twenty-two normal subjects served as controls. For normal subjects, RECD was in good agreement with the values reported in the literature. For

Tien-Chen Liu; Kai-Nan Lin

1999-01-01

128

A middle ear microphone design based on the physiology of the ear  

Microsoft Academic Search

Contrary to present external microphones, the proposed middle ear microphone does not measure the sound pressure but the acoustic deflection of a middle ear bone caused by an incoming pressure wave at the ear drum. By means of a seismic inertial transducer, minute vibrations in the range from 0.1 to 10 kHz should be measured. The innovation of our new

M. Sachse; W. Hortschitz; F. Kohl; J. Schalko; F. Keplinger

2010-01-01

129

Ear Modeling and Sound Signal Processing Ear modeling can significantly improve sound signal processing and  

E-print Network

Ear Modeling and Sound Signal Processing Jack Xin Abstract Ear modeling can significantly improve sound signal processing and the design of hearing devices. Ear models based on mechanics and neu- ral. The PDEs also facilitates an alternative sound amplification method for hearing aid design. Department

Xin, Jack

130

Congenital malformations and testicular germ cell tumors  

PubMed Central

Cryptorchidism is one of the few known risk factors for testicular germ cell tumors (TGCT). It has been postulated that other congenital malformations, in particular hypospadias, are also associated with increased risk; however, associations with birth defects have not been extensively studied. Using Swedish population-based registries we evaluated the relationship between birth defects and risk of TGCT. TGCT cases (n=6,593) diagnosed between 15 and 65 years of age were identified from the Swedish Cancer Registry between 1964 and 2008. Five controls per case were randomly selected from the population register and matched on birth year and birth county. Congenital malformations were identified via linkage with the Hospital Discharge Register. Odds ratios (OR) and 95% confidence intervals (CI) for the association between each group of malformations and TGCT were estimated using conditional logistic regression. In addition to the expected association between cryptorchidism and TGCT risk [OR (95% CI): 3.18 (2.50 to 4.04)], hypospadias [2.41 (1.27 to 4.57)], inguinal hernia [1.37 (1.11–1.68)] and other genital malformations [2.19 (1.17 to 4.10)] were associated with an increased risk of TGCT. Mutual adjustment for cryptorchidism, hypospadias, inguinal hernia, and other genital malformations did not appreciably change the associations (ORs: 3.16; 2.25; 1.30; 1.90, respectively). The other (non-genital) malformations evaluated were not associated with TGCT. These data suggest that developmental urogenital abnormalities, specifically cryptorchidism, hypospadias, and inguinal hernia, are associated with an increased risk of TGCT; further supporting the hypothesis that prenatal exposure(s) related to proper genital development are related to this tumor. PMID:23580254

Trabert, Britton; Zugna, Daniela; Richiardi, Lorenzo; McGlynn, Katherine A; Akre, Olof

2014-01-01

131

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

132

Extrapyramidal dysfunction with cerebral arteriovenous malformations 1  

PubMed Central

Arteriovenous malformations have only rarely been implicated as a cause of basal ganglia dysfunction. In four instances where such a lesion was uncovered, abnormal involuntary movements were present. In two, tremor involving the contralateral limbs occurred, while in others the head and neck were involved in dystonic movements and posture. The clinical and angiographic characteristics of these four patients have been assessed and are presented in detail in this report. The possible mechanism by which arteriovenous malformations may disturb the internal circuitry of the basal ganglia and induce symptoms are discussed. Images PMID:4829531

Lobo-Antunes, Joao; Yahr, Melvin D.; Hilal, Sadek K.

1974-01-01

133

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 ...

134

[Craniofacial malformations in prenatal ultrasound evaluation. Literature review].  

PubMed

Fetal face is the key anatomical location, both psychologically and clinically for the mother and the clinician. Ultrasound prenatal examination of the maxillofacial region allows to evaluate the fetal face in the first weeks of gestation. In ambulatory intravaginal ultrasound, sensitivity of the facial defect detection is 20-30% in cases without the risk of TORCH and fetal abnormalities, which may arouse suspicion of the presence of facial malformation. Facial defects form a wide group of pathologies. Unfortunately challenges connected with 2D and 3D ultrasound imaging cause frequent misdiagnoses in early gestation. Maxillofacial abnormalities can be solitary or they can coexist with other abnormalities or syndromes. In case of detecting a facial defect, a precise and thorough ultrasound of whole fetal body is necessary whereas in case of detecting any fetal body abnormality a precise and thorough ultrasound examination of the fetal face is obligatory Unfortunately most contemporary prenatal ultrasound standards propose only the overall "face and orbits" evaluation of the fetal face. The evaluation is difficult at 23 and 24 weeks of gestation and seems to be rather challenging in the third trimester of gestation. Not only facial malformations but also facial dimorphic features may lead to the suspicion of genetic syndrome and they may be extremely important in making correct diagnosis. Attempts at standardization in fetal face ultrasound evaluation have proved to be extremely difficult. Advantages of 2D ultrasound over 3D ultrasound and 3D ultrasound over 2D ultrasound in fetal face evaluation have been a topic of much debate. Most typically fetal face is examined with 2D ultrasound in a few basic planes: coronary sagittal, frontal and oblique. The planes preferred in the evaluation of facial structures are discussed in details in the paper Fetal facial defects evaluated in the ultrasound examination may be divided into a few main groups: examination of the orbit and eyeball defects, examination of the external nose and nasal cavity defects, examination of the cleft defects involving the lip, hard and soft palate which may be unilateral or bilateral, examination of external ear defects, examination of mandibular defects and detection of fetal tumors. 3D ultrasound evaluation of the fetal face is extremely useful in visualization of the face, thus presenting a problem to parents and clinicians. Prenatal ultrasound examination provides necessary and extremely useful data concerning fetal facial abnormalities, which allows to plan care and further treatment including interventions in pediatric ENT, pediatric surgery and plastic surgery areas. Cooperation of ultrasound diagnostician and clinicians taking care of a child in the future is therefore necessary when designing treatment scheme in cases of fetal facial defects. PMID:24191520

Zieli?ski, Rafa?; Respondek-Liberska, Maria

2013-09-01

135

Segmental colonic motility in patients with anorectal malformations  

Microsoft Academic Search

Background: Constipation is one of the most important functional sequelae in patients with anorectal malformations. The cause of this motility disorder is unknown. Aim: The purpose of this study was to assess total colonic transit time (TCT) and segmental colonic transit time (SCT) in patients with anorectal malformations. Method: Ninety patients with anorectal malformations (40 low and 50 high; median

R. J Rintala; E Marttinen; K Virkola; M Rasanen; C Baillie; H Lindahl

1997-01-01

136

Value of Ear Endoscopy in Cholesteatoma Surgery  

Microsoft Academic Search

Objective: The purpose of this study was to assess the value of ear endoscopy in cholesteatoma surgery and to demonstrate its consequence in improving surgical outcome. Materials and Methods: A total of 92 ears with acquired cholesteatoma (primary or secondary) were operated on. In this prospective study, 82 cases were operated on by using canal wall up (CWU) technique, and

M. Badr-el-Dine

2002-01-01

137

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...

138

Ear Biometrics in Human Identification A Dissertation  

E-print Network

Biometrics are physical or behavioral characteristics that can be used for hu- man identification. SecurityEar 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

Bowyer, Kevin W.

139

Genetic Requirement for Pneumococcal Ear Infection  

Microsoft Academic Search

BackgroundEar 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

Huaiqing Chen; Yueyun Ma; Jun Yang; Christopher J. O'Brien; Scott L. Lee; Joseph E. Mazurkiewicz; Sauli Haataja; Jing-Hua Yan; George F. Gao; Jing-Ren Zhang; Debbie Fox

2008-01-01

140

Ultrasound Characterization of Middle Ear Effusion  

PubMed Central

Purpose To further enhance and assess the ability to characterize middle ear effusion (MEE) using non-invasive ultrasound technology. Materials and Methods This is a prospective unblinded comparison study. Fifty-six children between the ages of 6 months and 17 years scheduled to undergo bilateral myringotomy with pressure equalization tube placement were enrolled. With the child anesthetized, the probe was placed into the external ear canal after sterile water was inserted. Ultrasound recordings of middle ear contents were analyzed by computer algorithm. Middle ear fluid was collected during myringotomy and analyzed for bacterial culture and viscosity. Results Ultrasound waveforms yielded a computer algorithm interpretation of middle ear contents in 66% of ears tested. When a result was obtained, the sensitivity and specificity for successfully characterizing middle ear fluid content as either void of fluid, thick fluid (mucoid), or thin fluid (serous or purulent) was at least 94%. Mucoid effusions had higher measured viscosity values (P=0.002). Viscosity measures were compared to culture result, and those with low viscosity (thin consistency) had a higher likelihood of having a positive culture (P=0.048). Conclusion The device sensitivity and specificity for fluid detection was 94% or greater among interpretable waveforms (66% of those tested). Although this technology provides important information of the middle ear effusion presence and characteristic, further technological improvements are needed. PMID:23084430

Seth, Rahul; Discolo, Christopher M; Palczewska, Grazyna M; Lewandowski, Jan J; Krakovitz, Paul R

2012-01-01

141

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

142

Stem Cell Therapy for the Inner Ear  

PubMed Central

In vertebrates, perception of sound, motion, and balance is mediated through mechanosensory hair cells located within the inner ear. In mammals, hair cells are only generated during a short period of embryonic development. As a result, loss of hair cells as a consequence of injury, disease, or genetic mutation, leads to permanent sensory deficits. At present, cochlear implantation is the only option for profound hearing loss. However, outcomes are still variable and even the best implant cannot provide the acuity of a biological ear. The recent emergence of stem cell technology has the potential to open new approaches for hair cell regeneration. The goal of this review is to summarize the current state of inner ear stem cell research from a viewpoint of its clinical application for inner ear disorders to illustrate how complementary studies have the potential to promote and refine stem cell therapies for inner ear diseases. The review initially discusses our current understanding of the genetic pathways that regulate hair cell formation from inner ear progenitors during normal development. Subsequent sections discuss the possible use of endogenous inner ear stem cells to induce repair as well as the initial studies aimed at transplanting stem cells into the ear. PMID:22514095

Okano, Takayuki

2012-01-01

143

Reading disability and middle ear disease.  

PubMed Central

The association between specific reading disability and middle ear disease was investigated in a longitudinal study of 962 children followed from age 5 to 11. No significant differences were found in the prevalence of middle ear abnormalities between the reading disabled group (n = 49) and the remainder. PMID:3707193

Share, D L; Chalmers, D; Silva, P A; Stewart, I A

1986-01-01

144

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

145

Ear asymmetries in middle-ear, cochlear, and brainstem responses in human infants  

PubMed Central

In 2004, Sininger and Cone-Wesson examined asymmetries in the signal-to-noise ratio (SNR) of otoacoustic emissions (OAE) in infants, reporting that distortion-product (DP)OAE SNR was larger in the left ear, whereas transient-evoked (TE)OAE SNR was larger in the right. They proposed that cochlear and brainstem asymmetries facilitate development of brain-hemispheric specialization for sound processing. Similarly, in 2006 Sininger and Cone-Wesson described ear asymmetries mainly favoring the right ear in infant auditory brainstem responses (ABRs). The present study analyzed 2640 infant responses to further explore these effects. Ear differences in OAE SNR, signal, and noise were evaluated separately and across frequencies (1.5, 2, 3, and 4 kHz), and ABR asymmetries were compared with cochlear asymmetries. Analyses of ear-canal reflectance and admittance showed that asymmetries in middle-ear functioning did not explain cochlear and brainstem asymmetries. Current results are consistent with earlier studies showing right-ear dominance for TEOAE and ABR. Noise levels were higher in the right ear for OAEs and ABRs, causing ear asymmetries in SNR to differ from those in signal level. No left-ear dominance for DPOAE signal was observed. These results do not support a theory that ear asymmetries in cochlear processing mimic hemispheric brain specialization for auditory processing. PMID:18345839

Keefe, Douglas H.; Gorga, Michael P.; Jesteadt, Walt; Smith, Lynette M.

2008-01-01

146

Perspective methods of human identification: Ear biometrics  

NASA Astrophysics Data System (ADS)

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

Chora?, M.

2008-03-01

147

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

148

Obstetric complications and congenital malformation in schizophrenia  

Microsoft Academic Search

Recent years have witnessed increasingly intense research activity concerning early life somatic trauma and dysmorphogenesis which are associated with the later development of schizophrenia. The two somatic factors that have received the most extensive scientific attention as antecedents of schizophrenia are obstetric complications (OCs) and the congenital malformations termed `minor physical anomalies' (MPAs). Head circumference (HC) at birth has also

Thomas F McNeil; Elizabeth Cantor-Graae; Baher Ismail

2000-01-01

149

Differential Gene Expression in Human Cerebrovascular Malformations  

PubMed Central

OBJECTIVE We sought to identify genes with differential expression in cerebral cavernous malformations (CCMs), arteriovenous malformations (AVMs), and control superficial temporal arteries (STAs) and to confirm differential expression of genes previously implicated in the pathobiology of these lesions. METHODS Total ribonucleic acid was isolated from four CCM, four AVM, and three STA surgical specimens and used to quantify lesion-specific messenger ribonucleic acid expression levels on human gene arrays. Data were analyzed with the use of two separate methodologies: gene discovery and confirmation analysis. RESULTS The gene discovery method identified 42 genes that were significantly up-regulated and 36 genes that were significantly down-regulated in CCMs as compared with AVMs and STAs (P = 0.006). Similarly, 48 genes were significantly up-regulated and 59 genes were significantly down-regulated in AVMs as compared with CCMs and STAs (P = 0.006). The confirmation analysis showed significant differential expression (P < 0.05) in 11 of 15 genes (angiogenesis factors, receptors, and structural proteins) that previously had been reported to be expressed differentially in CCMs and AVMs in immunohistochemical analysis. CONCLUSION We identify numerous genes that are differentially expressed in CCMs and AVMs and correlate expression with the immunohistochemistry of genes implicated in cerebrovascular malformations. In future efforts, we will aim to confirm candidate genes specifically related to the pathobiology of cerebrovascular malformations and determine their biological systems and mechanistic relevance. PMID:12535382

Shenkar, Robert; Elliott, J. Paul; Diener, Katrina; Gault, Judith; Hu, Ling-Jia; Cohrs, Randall J.; Phang, Tzulip; Hunter, Lawrence; Breeze, Robert E.; Awad, Issam A.

2009-01-01

150

Atypical Presentations in Chiari II Malformation  

Microsoft Academic Search

Myelomeningocele with Chiari II malformation and hydrocephalus is a common association seen in infants with a congenital failure of neurulation. Here we report two cases of such an association presenting with different sets of problems. The first patient presented with severe inspiratory stridor due to bilateral abductor vocal cord paralysis, which was relieved completely within 24 h of definitive surgery.

G. P. Rath; P. K. Bithal; A. Chaturvedi

2006-01-01

151

Split Cord Malformations of the Lumbar Region  

Microsoft Academic Search

From a group of 84 patients with split cord malformations presenting to our Department between 1976 and 1990, we have selected 47 cases in whom the split cord was confined to the lower dorsal-lumbar region and in whom there were no other dysraphic features such as meningocele, lipoma or dermoid cyst. We have studied these cases of ‘pure split cord

U. B Andar; W. F. J. Harkness; R. D. Hayward

1997-01-01

152

Pancreatic arteriovenous malformation with duodenal ulcer  

Microsoft Academic Search

Summary We report the color Doppler ultrasonography features of arteriovenous malformation (AVM) of the pancreas, a very rare disease. The patient was a 52-year-old man with congenital AVM of the pancreas and a duodenal ulcer that had been resistant to medication. Endoscopic color Doppler ultrasonography (color Doppler EUS) revealed many abnormal color signals showing pulsatile wave form at the portion

Shigeo Tano; Norio Ueno; Tomio Ueno; Shin-Ichi Wada; Toshiyuki Aizawa; Ken Kimura

1996-01-01

153

Body growth in urinary tract malformations  

Microsoft Academic Search

Body height and height velocity were analysed in 54 children with obstructive urinary tract malformations over a mean period of 8.7 years, using new auxological methods. At the time of diagnosis, 9% of patients had a height of more than 2 standard deviations below the normal mean. Mean relative height changed significantly from the first to the last observation, the

C. Seidel; F. Schaefer; K. Schärer

1993-01-01

154

Otic ablation of smoothened reveals direct and indirect requirements for Hedgehog signaling in inner ear development  

PubMed Central

In mouse embryos lacking sonic hedgehog (Shh), dorsoventral polarity within the otic vesicle is disrupted. Consequently, ventral otic derivatives, including the cochlear duct and saccule, fail to form, and dorsal otic derivatives, including the semicircular canals, endolymphatic duct and utricle, are malformed or absent. Since inner ear patterning and morphogenesis are heavily dependent on extracellular signals derived from tissues that are also compromised by the loss of Shh, the extent to which Shh signaling acts directly on the inner ear for its development is unclear. To address this question, we generated embryos in which smoothened (Smo), an essential transducer of Hedgehog (Hh) signaling, was conditionally inactivated in the otic epithelium (Smoecko). Ventral otic derivatives failed to form in Smoecko embryos, whereas vestibular structures developed properly. Consistent with these findings, we demonstrate that ventral, but not dorsal, otic identity is directly dependent on Hh. The role of Hh in cochlear-vestibular ganglion (cvg) formation is more complex, as both direct and indirect signaling mechanisms are implicated. Our data suggest that the loss of cvg neurons in Shh–/– animals is due, in part, to an increase in Wnt responsiveness in the otic vesicle, resulting in the ectopic expression of Tbx1 in the neurogenic domain and subsequent repression of Ngn1 transcription. A mitogenic role for Shh in cvg progenitor proliferation was also revealed in our analysis of Smoecko embryos. Taken together, these data contribute to a better understanding of the intrinsic and extrinsic signaling properties of Shh during inner ear development. PMID:21831920

Brown, Alexander S.; Epstein, Douglas J.

2011-01-01

155

Effects of middle-ear disorders on power reflectance measured in cadaveric ear canals  

PubMed Central

Objective Reflectance measured in the ear canal offers a noninvasive method to monitor the acoustic properties of the middle ear, and few systematic measurements exist on the effects of various middle-ear disorders on the reflectance. This work utilizes a human cadaver-ear preparation and a mathematical middle-ear model to both measure and predict how power reflectance ? is affected by the middle-ear disorders of static middle-ear pressures, middle-ear fluid, fixed stapes, disarticulated incudo-stapedial joint, and tympanic-membrane perforations. Design ? was calculated from ear-canal pressure measurements made on human-cadaver ears in the normal condition and five states: (1) positive and negative pressure in the middle-ear cavity, (2) fluid-filled middle ear, (3) stapes fixed with dental cement, (4) incudo-stapedial joint disarticulated, and (5) tympanic-membrane perforations. The middle-ear model of Kringlebotn (1988) was modified to represent the middle-ear disorders. Model predictions are compared to measurements. Results For a given disorder, the general trends of the measurements and model were similar. The changes from normal in ?, induced by the simulated disorder, generally depend on frequency and the extent of the disorder (except for the disarticulation). Systematic changes in middle-ear static pressure (up to ± 300 daPa) resulted in systematic increases in ?. These affects were most pronounced for frequencies up to 1000 to 2000 Hz. Above about 2000 Hz there were some asymmetries in behavior between negative and positive pressures. Results with fluid in the middle-ear air space were highly dependent on the percentage of the air space that was filled. Changes in ? were minimal when a smaller fraction of the air space was filled with fluid, and as the air space was filled with more saline, ? increased at most frequencies. Fixation of the stapes generally resulted in a relatively small low-frequency increase in ?. Disarticulation of the incus with the stapes led to a consistent low-frequency decreases in ? with a distinctive minimum below 1000 Hz. Perforations of the tympanic membrane resulted in a decrease in ? for frequencies up to about 2000 Hz; at these lower frequencies, smaller perforations led to larger changes from normal as compared to larger perforations. Conclusions These preliminary measurements help assess the utility of power reflectance as a diagnostic tool for middle-ear disorders. In particular, the measurements document (1) the frequency ranges for which the changes are largest and (2) the extent of the changes from normal for a spectrum of middle-ear disorders. PMID:22037477

Merchant, Gabrielle R.; Horton, Nicholas J.

2011-01-01

156

A 6-year experience treating vascular malformations with foam sclerotherapy.  

PubMed

In this study, the authors present an analysis of the outcomes of 105 low-flow vascular malformation patients treated over a 6-year period and report specific lesion characteristics that correlate with those vascular malformations that will benefit from sodium tetradecyl sulfate foam sclerotherapy (STS FS) versus surgical resection as well as morphological characteristics of vascular malformations that are associated with a poor response to FS treatment. Improvement in symptoms was documented in 92.9% of patients treated with STS FS. There were no complications. Low-flow vascular malformations that were morphologically characterized by microcystic, septated vessels did not respond to FS, and these vascular malformations are best treated with surgical resection. Primary surgical resection is also the treatment of choice for localized, microcystic, and superficial low-flow vascular malformations. Symptomatic, diffuse, extensive, macrocystic malformations that involve multiple tissue planes and vital structures are best treated with FS. PMID:22918935

Markovic, Jovan N; Kim, Charles Y; Lidsky, Michael E; Shortell, Cynthia K

2012-06-01

157

[Cognitive and affective characteristics of children with malformation syndrome].  

PubMed

The aim of this paper is to study the psychological and relational aspects in children suffering from specific malformative syndrome and precisely Down s., Sotos s., X-Fragile s. and Williams s. Indeed literature provides much data related to the phenotype, to the organic-biological characteristics, but little or nothing is known about the affective structure, the episodes and to the particular dynamics that emerge in he relation between the parents and the malformed child. A protocol was applied to our sample group (16 subjects). This protocol includes laboratory and instrumental tests (chromosome test, neurometabolic screening, EEG, CT or cranial MRI, cardiac and abdominal ultrasonography, ear and eye test) aspects. This evaluation is carried out through the proposal of standardized situations (psychometric tests) and a use of a freer observational setting. This permits us to understand how the child perceives himself the awareness and the image he has of himself and how able he is to integrate his illness experiences and his way of relating with the environment. The data of our observations are thus used to compile a grill for the structural diagnosis of the personality. Besides, this evaluation is flanked by the observation of the family in order to explore the psychological image that parents have of their child, his character, his good points, his bad points, his similarities, how he relates to them, any educational problems and the emotional reaction that the communication of the diagnosis has raised in them. The videotaped observations are subsequently evaluated through the application of a grill for the study of the mother-child relationship. The results obtained from the psychological research underline a reasonable heterogeneity both of the intellectual level and of the metapsychological profile. Twelve subjects were mentally retarded (5 with mild mental retardation, 7 with moderate mental retardation); the remaining 4 had a normal cognitive development (3 with Sotos s., 1 with Williams s.). Psychological disturbances are present and thus divided: light disturbances (affective immaturity, neurotic-depressive organisation) in 11 subjects. Average disturbances (dysharmonious structure, and borderline personality) in 4 subjects; severe disturbances (psychosis) in 1 subject. Besides, above all in the group of subjects with X-Fragile s. and Down s., the tendency to assume behaviour of a regressive type, also postural, emerges. Among the 4 groups it is frequent to resort to defence mechanisms of hypomaniac type, accompanied by the denial of the patient's "sick parts". Another common characteristic concerns the quality of imaginary life which is shown to be repetitive and stereotype in content. Indeed these children's play activity characterized by a limited capacity of symbolization. Instead, when the symbolic process is more developed, contents concerning a deteriorated and destructive image of the Self emerges. Through the evaluation of family dynamics what is more noticeable is that the parent-malformed child interaction appears to be quite nonstimulating and noninvolving or incoherent, lacking in harmony and empathy towards the child's inner world. Indeed we can notice a lack of both verbal and extraverbal exchange of communication and brief interactive sequences which do not usually take into account the child's proposals and an affective tonality of depressive and nonaffective type. Therefore it may be concluded a certain smoothness in the clinical expression of the syndromes considered, both as far as the cognitive deficit entity and the psychic problems are concerned. Referring to the interactive dynamics between parents and children with dismorphic syndrom it seems that the child's pathology becomes the organizational summit of the above-mentioned relational dynamics among most of the patients examined... PMID:8569638

Tosi, B; Maestro, S; Marcheschi, M

1995-10-01

158

Human antimicrobial proteins in ear wax.  

PubMed

The external auditory canal is vulnerable to bacterial infections, but little is known about thechemical compositions of ear wax regarding antimicrobial peptides. We, therefore, studied the proteinconcentrations of ten well-known human antimicrobial peptides from ear wax.Twenty ear wax samples from healthy individuals were analysed using enzyme-linked immunosorbent assay (ELISA) to determine theprotein concentrations of the antimicrobial peptides hBD1-3, lactoferrin, LL-37, BPI, hSLPI and HNP1-3. All ten antimicrobial peptides are present in ear wax. Some of these proteins seem to be merelycell-bound in ear wax. Antimicrobial peptides in ear wax prevent bacteria and fungi from causing infections inthe external auditory canal. The role and importance of these proteins for the blind-ending ear externalcanal is discussed. If this local defence system fails, infections of the external auditory canal may result.The knowledge about the presence of antimicrobial peptides in cerumen may lead to new concepts ofthe local treatment of external auditory canal diseases in the future. PMID:21298458

Schwaab, M; Gurr, A; Neumann, A; Dazert, S; Minovi, A

2011-08-01

159

Congenital malformations in experimental diabetic pregnancy: aetiology and antioxidative treatment. Minireview based on a doctoral thesis.  

PubMed

Diabetes mellitus in pregnancy causes congenital malformations in the offspring. The aim of this work was to characterize biochemical and morphologic anomalies in the conceptus of an animal model of diabetic pregnancy. In addition, a preventive treatment against diabetes-induced dysmorphogenesis was developed. Congenital cataract was often found in the offspring of diabetic rats. The fetal lenses had increased water accumulation, sorbitol concentration and aldose reductase activity compared to control lenses. The results suggest that the cataracts form via osmotic attraction of water due to sorbitol accumulation in the fetal lens. Another set of malformations, with possible neural crest cell origin, occurred frequently in offspring of diabetic rats. These included low set ears, micrognathia, hypoplasia of the thymus, thyroid and parathyroid glands, as well as anomalies of the heart and great vessels. Furthermore, diabetes caused intrauterine death and resorptions more frequently in the late part of gestation. When the pregnant diabetic rats were treated with the antioxidants butylated hydroxytoluene, vitamin E or vitamin C, the occurrence of gross malformations was reduced from approximately 25% to less than 8%, and late resorptions from 17% to 7%. This suggests that an abnormal handling of reactive oxygen species (ROS) is involved in diabetes-induced dysmorphogenesis in vivo. Indeed, an increased concentration of lipid peroxides, indicating damage caused by ROS, was found in fetuses of diabetes rats. In addition, embryos of diabetic rats had low concentrations of the antioxidant vitamin E compared to control embryos. These biochemical alterations were normalized by vitamin E treatment of the pregnant diabetic rats. The antioxidants are likely to have prevented ROS injury in the embryos of the diabetic rats, in particular in the neural crest cells, thereby normalizing embryonic development. These results provide a rationale for developing new anti-teratogenic treatments for pregnant women with diabetes mellitus. PMID:9394431

Simán, M

1997-01-01

160

Middle-ear dynamics before and after ossicular replacement  

Microsoft Academic Search

The mechanism of hearing involves conduction of mechanical vibrations along the ossicular chain to the inner ear. An acoustic wave is collected and transformed as it passes down the ear canal and impacts on the tympanic membrane (ear drum). The drum is connected to the inner-ear by three ossicle bones (malleus, incus, and stapes) in a complex arrangement, which serves

P. Ferris; P. J. Prendergast

2000-01-01

161

Middle-ear dynamics before and after ossicular replacementq  

Microsoft Academic Search

The mechanism of hearing involves conduction of mechanical vibrations along the ossicular chain to the inner ear. An acoustic wave is collected and transformed as it passes down the ear canal and impacts on the tympanic membrane (ear drum). The drum is connected to the inner-ear by three ossicle bones (malleus, incus, and stapes) in a complex arrangement, which serves

P. Ferris; P. J. Prendergast

2000-01-01

162

Ear Infections in Autistic and Normal Children. Brief Report.  

ERIC Educational Resources Information Center

Evaluation of the frequency of ear infections, ear tube drainage, and deafness for 51 autistic children (ages 2-18) indicated that autistic children had a greater incidence of ear infections than matched normal peers and lower functioning children had an earlier onset of ear infections than higher functioning autistic peers. (Author)

Konstantareas, M. Mary; Homatidis, Soula

1987-01-01

163

A new analytical theory for earing generated from anisotropic plasticity  

Microsoft Academic Search

Commercial canmaking processes include drawing, redrawing and several ironing operations. It is experimentally observed that during the drawing and redrawing processes earing develops, but during the ironing processes earing is reduced. It is essential to understand the earing mechanism during drawing and ironing for an advanced material modeling. A new analytical approach that relates the earing profile to r-value and

J. W. Yoon; R. E. Dick; F. Barlat

2011-01-01

164

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

165

Bronchopulmonary foregut malformations: embryology, radiology and quandary  

Microsoft Academic Search

.  Bronchopulmonary foregut malformations (BPFM) are a heterogeneous group of pulmonary developmental anomalies that present\\u000a at varying ages and with overlapping symptoms, signs and radiology. This article discusses the embryology of these lesions\\u000a with reference to possible common origins and the link between aetiology and radiological appearance. The radiology of each\\u000a lesion, both antenatally and postnatally, is described and illustrated. A

N. A. Barnes; D. W. Pilling

2003-01-01

166

Spontaneous resolution of isolated Chiari I malformation  

Microsoft Academic Search

Introduction  Spontaneous resolution of Chiari I abnormality is very rare. In most patients, the radiological abnormality either stays unchanged with time or deriorates.Case report  We present a male patient who was diagnosed at the age of 18 months as having radiological evidence of Chiari I malformation without syringomyelia, which had resolved 5 years later on a subsequent MR scan. At the time

N. S. Jatavallabhula; J. Armstrong; S. Sgouros; W. Whitehouse

2006-01-01

167

Epilepsy in type 1 Chiari malformation  

Microsoft Academic Search

In patients with Chiari malformation type 1 (CMI), epileptic seizures are occasionally reported both in symptomatic patients\\u000a candidate to surgery and in patients without symptoms of tonsillar displacement in whom CM1 is often an incidental finding\\u000a in the diagnostic work up for idiopathic epilepsies. In both groups of patients, the course of epilepsy is almost invariably\\u000a favorable, with a few

Tiziana GranataLaura; Laura Grazia Valentini

168

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

169

Tympanosclerosis of the ear drum in children.  

PubMed

Tympanosclerosis of the ear drum in children is associated with secretory otitis media. The tympanosclerotic changes affect the pars tensa and seem to be a dynamic process of new formation together with regress and restoration to normal tissue. Our study includes 90 cleft palate children followed for 7.8 years with a total of 2068 examinations. During the observation period 59% of the ear drums were affected by tympanosclerosis, while only 42% had tympanosclerosis at the last examination. The tympanosclerotic lesion of the ear drum disappeared completely in 30%, seemed stable in 40% and tended to grow steadily in 30% of the ear drums affected. The pathogenesis of tympanosclerosis is not clear, although the inflammation known to exist in secretory otitis media probably plays an important part. PMID:6541211

Mřller, P

1984-07-01

170

Mozart ear: diagnosis, treatment, and literature review.  

PubMed

Mozart ear is a congenital auricular deformity, which is mainly characterized by a bulging appearance of the anterosuperior portion of the auricle, a convexly protruded cavum conchae, and a slit-like narrowing of the orifice of the external auditory meatus. It is said to be uncommon, and because no one has yet fully described neither the disease nor the treatment, the concept of Mozart ear has not been unified. This report describes a case of a 13-year-old girl presented with an unusual congenital deformity which showed the features of Mozart ear. It is an extremely rare deformity that only about 4 clinical cases have been reported in medical literature thereby a treatment method has not been fully discussed. For surgical correction of our cases, we excised deformed conchal cartilage, turned it over, regrafted, and maintained a cosmetically positive result. We also reviewed and described the origin, current concept, and treatment method of Mozart ear. PMID:21587051

Yamashita, Ken; Yotsuyanagi, Takatoshi; Saito, Tamotsu; Isogai, Noritaka; Mori, Hiromasa; Itani, Yoshihito

2011-11-01

171

Animal communication: flies' ears are tuned in.  

PubMed

Male fruit flies sing to females with quiet, close-range wing vibrations. A new study has found that the flies' antennal ears show active tuning to the species-specific frequencies of songs. PMID:21514508

Immonen, Elina; Ritchie, Michael G

2011-04-26

172

Middle Ear Surgery in Only Hearing Ears and Postoperative Hearing Rehabilitation  

PubMed Central

Background and Objectives The aim of this study was to evaluate surgical interventions and hearing rehabilitation in patients with chronic middle ear disease of only hearing ears. Subjects and Methods Thirty-one patients with chronic middle ear disease of only hearing ears were enrolled in this retrospective study. Patients were classified into three groups according to the hearing level: groups A [pure tone audiometry (PTA)<40], B (40?PTA<70), and C (PTA?70). We evaluated hearing results and patterns of auditory rehabilitation. Results The main consideration for a surgical procedure was the presence of recurrent otorrhea and structural destruction. The reasons for surgical intervention in only hearing ears were otorrhea caused by chronic otitis media (68%), cholesteatoma (29%), and cholesterol granuloma (3%). The causes of contralateral deaf ears were chronic otitis media (81%) and sensorineural hearing loss (19%). Although there was hearing deterioration in some patients with severe hearing loss (PTA?70), all patients achieved dry ears after surgery and functional hearing using auditory rehabilitation. Hearing aids were used in most patients with moderate to moderately severe hearing loss and cochlear implants were used for auditory rehabilitation in patients with severe to profound hearing loss. Conclusions Proper evaluation and indications for surgery in only hearing ears are important for successful eradication of inflammation and hearing preservation. Surgical interventions can achieve dry ear and enable further auditory rehabilitations using hearing aids and cochlear implantation. PMID:25279226

Yoo, Myung Hoon; Kang, Byung Chul; Park, Hong Ju

2014-01-01

173

Neuroembryology and brain malformations: an overview.  

PubMed

Modern neuroembryology integrates descriptive morphogenesis with more recent insight into molecular genetic programing and data enabled by cell-specific tissue markers that further define histogenesis. Maturation of individual neurons involves the development of energy pumps to maintain membrane excitability, ion channels, and membrane receptors. Most malformations of the nervous system are best understood in the context of aberrations of normal developmental processes that result in abnormal structure and function. Early malformations usually are disorders of genetic expression along gradients of the three axes of the neural tube, defective segmentation, or mixed lineages of individual cells. Later disorders mainly involve cellular migrations, axonal pathfinding, synaptogenesis, and myelination. Advances in neuroimaging now enable the diagnosis of many malformations in utero, at birth, or in early infancy in the living patient by abnormal macroscopic form of the brain. These images are complimented by modern neuropathological methods that disclose microscopic, immunocytochemical, and subcellular details beyond the resolution of MRI. Correlations may be made of both normal and abnormal ontogenesis with clinical neurological and EEG maturation in the preterm or term neonate for a better understanding of perinatal neurological disease. Precision in terminology is a key to scientific communication. PMID:23622157

Sarnat, Harvey B; Flores-Sarnat, Laura

2013-01-01

174

Gynecologic concerns in patients with anorectal malformations.  

PubMed

Children with anorectal malformations (ARMs) constitute a significant group within a pediatric surgery practice. In females, the most common ARM is an imperforate anus with a rectovestibular fistula, followed by an imperforate anus with a rectoperineal fistula and then cloacal anomalies. Some malformations, such as an imperforate anus with a rectovestibular fistula, may seem straightforward, treated with a posterior sagittal anorectoplasty; however, it is vital to recognize the association with gynecologic anomalies. Girls with the most complex anorectal defect, cloacal malformation, have significant associated urological and gynecologic anomalies, which should be recognized and treated. An opportunity to diagnose and treat such anomalies may be missed in the newborn period or at the definitive surgery, unless the pediatric surgeon is aware of this association. With the knowledge of the associated anomalies and long-term sequelae, surgeons can provide better care for girls and important counseling for parents. Important gynecologic issues to consider may be divided into several categories, such as infancy, puberty, sexual intimacy, and obstetrical concerns. Knowledge of reproductive-related issues in females with ARMs allows the pediatric surgeon and their gynecology colleagues to provide optimal surgical management throughout infancy, childhood, and into young adulthood. Appropriate counseling for patients and families about potential reproductive concerns that may develop many years after the definitive surgical repair allows preparation and planning to preserve future fertility. PMID:20307850

Breech, Lesley

2010-05-01

175

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.

176

Correcting for ear canal collapse during audiometry.  

PubMed

The hearing thresholds of 20 subjects with normal hearing and normal ear canals and two subjects with collapsible ear canals were measured at octave frequencies from 250 to 8000 Hz under standard transducers (TDH-39) fitted with standard cushions (MX-41/AR) and experimental cushions (Telephonics 266CIII). When correction factors based on normal threshold differences were applied, thresholds obtained under the experimental cushion reasonably approximated the actual hearing of individuals with collapsible canals. PMID:6628851

Rizzo, S R

1983-01-01

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

Vascular malformations of the gastrointestinal tract.  

PubMed

The advent of fiberoptic endoscopy, which became widespread in the evaluation of gastrointestinal bleeding throughout the late 1970s and 1980s, has dramatically changed both our understanding of the extent to which vascular malformations account for gastrointestinal blood loss and our ability to treat these lesions at the time of diagnosis. Colonic vascular malformations appear to be the single most common cause of acute or recurrent gastrointestinal bleeding episodes in patients over 60 years of age, being responsible for the bleeding in as many as 35% of such patients. Although less common as a cause of upper gastrointestinal bleeding, these lesions still account for 2% to 5% of bleeding lesions in older patients. Diagnosis is accomplished by endoscopy, and the vascular malformations can then be coagulated via the endoscope using one of a number of thermal systems. The argon laser, the heater probe, and the BICAP system are all effective and safe throughout the gastrointestinal tract, especially in the cecum and right colon, where the majority of sporadic vascular malformations occur. Monopolar cautery and the Nd:YAG laser are equally efficacious, but their greater and less predictable depth of coagulation make them much less safe in the cecum and right colon. There are no apparent advantages in terms of efficacy and safety between laser treatment and the other thermal modalities. The laser has the advantage of being quicker, which is especially important when treating large or multiple lesions. The other modalities have the advantages of portability and low relative cost. Endoscopic therapy with lasers or other thermal devices is nonspecific. The effects are achieved by thermally coagulating the mucosal vascular lesions, allowing the coagulated tissue to slough, and leaving a mucosal ulceration that subsequently heals with re-epithelialization. Endoscopic coagulation has thus been reported effective in the treatment of gastrointestinal mucosal vascular lesions regardless of their etiology or characteristics. It has been effective for sporadic vascular malformations, hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease), radiation proctocolitis, the blue rubber-bleb nevus syndrome, and diffuse gastric antral vascular ectasia (the watermelon stomach). As we move through the 1990s and beyond, these endoscopic modalities offer an effective, relatively safe, and clearly less invasive treatment option for the many patients who experience acute, recurrent, or chronic gastrointestinal bleeding from any of these lesions. PMID:1589831

Buchi, K N

1992-06-01

179

Malformations in neotropical viperids: qualitative and quantitative analysis.  

PubMed

Malformations can occur in all living species, but there is little information about anomalies that occur in snakes and their frequency. This study assessed malformations in newborn South American pit vipers (Bothrops jararaca) and South American rattlesnakes (Crotalus durissus) from wild captured pregnant females (240 and 35 litters, respectively). Newborn snakes were measured, weighed, sexed and studied grossly and by radiography for the presence of malformations. Ninety-five malformed pit vipers were identified from 4,087 births (2.3%), while 36 malformed rattlesnakes were found from 324 births (11.1%). Spinal abnormalities were the most common in both species, followed by fusion of ventral scales. Pit vipers showed a greater range of malformations including schistosomia (22.1%), kinked tail (13.7%), bicephaly (3.1%) and hydrocephaly (2.1%). PMID:23885804

Sant'Anna, S S; Grego, K F; Lorigados, C A B; Fonseca-Pinto, A C B C; Fernandes, W; Sá-Rocha, L C; Catăo-Dias, J L

2013-11-01

180

An evaluation of tympanometric estimates of ear canal volume.  

PubMed

The accuracy of tympanometric estimates of ear canal volume was evaluated by testing the following two assumptions on which the procedure is based: (a) ear canal volume does not change when ear canal pressure is varied, and (b) an ear canal pressure of 200 daPa drives the impedance of the middle ear transmission system to infinity so the immittance measured at 200 daPa can be attributed to the ear canal volume alone. The first assumption was tested by measuring the changes in ear canal volume in eight normal subjects for ear canal pressures between +/- 400 daPa using a manometric procedure based on Boyle's gas law. The data did not support the first assumption. Ear canal volume changed by a mean of .113 ml over the +/- 400 daPa pressure range with slightly larger volume changes occurring for negative ear canal pressures than for positive ear canal pressures. Most of the volume change was attributed to movement of the probe and to movement of the cartilaginous walls of the ear canal. The second assumption was tested by comparing estimates of ear canal volume from susceptance tympanograms with a direct measurement of ear canal volume adjusted for changes in volume due to changes in ear canal pressure between +/- 400 daPa. These data failed to support the second assumption. All tympanometric estimates of ear canal volume were larger than the measured volumes. The largest error (39%) occurred for an ear canal pressure of 200 daPa at 220 Hz, whereas the smallest error (10%) occurred for an ear canal pressure of -400 daPa at 660 Hz. This latter susceptance value (-400 daPa at 660 Hz) divided by three is suggested to correct the 220-Hz tympanogram to the plane of the tympanic membrane. Finally, the effects of errors in estimating ear canal volume on static immittance and on tympanometry are discussed. PMID:7329051

Shanks, J E; Lilly, D J

1981-12-01

181

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

182

An Eye for an Ear and an Ear for an Eye: Bidirectional Control in Virtual Multimedia  

E-print Network

An Eye for an Ear and an Ear for an Eye: Bidirectional Control in Virtual Multimedia Instrument Design Christian Jacquemin LIMSI-CNRS and University Paris 11, BP 133, 91403 ORSAY, France Abstract is illustrated on two examples of systems that use bidirectional control, autonomous mapping and a high degree

Jacquemin, Christian

183

Epileptogenic brain malformations: clinical presentation, malformative patterns and indications for genetic testing  

Microsoft Academic Search

We review here those malformations of the cerebral cortex which are most often observed in epilepsy patients, for which a genetic basis has been elucidated or is suspected and give indications for genetic testing.There are three forms of lissencephaly (agyria-pachygyria) resulting from mutations of known genes, which can be distinguished because of their distinctive imaging features. They account for about

Romeo Carrozzo

2001-01-01

184

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

185

Ear acupuncture in European traditional medicine.  

PubMed

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

Gori, Luigi; Firenzuoli, Fabio

2007-09-01

186

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

187

Evolution and development of the vertebrate ear  

NASA Technical Reports Server (NTRS)

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

Fritzsch, B.; Beisel, K. W.

2001-01-01

188

Stereotactic radiosurgery of intracranial arteriovenous malformations.  

PubMed

Stereotactic radiosurgery for intracranial arteriovenous malformations (AVMs) has been performed since the 1970s. When an AVM is treated with radiosurgery, radiation injury to the vascular endothelium induces the proliferation of smooth muscle cells and the elaboration of extracellular collagen, which leads to progressive stenosis and obliteration of the AVM nidus. Obliteration after AVM radiosurgery ranges from 60% to 80%, and relates to the size of the AVM and the prescribed radiation dose. The major drawback of radiosurgical AVM treatment is the risk of bleeding during the latent period (typically 2 years) between treatment and AVM thrombosis. PMID:24093574

Friedman, William A

2013-10-01

189

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

190

Surgical management of pediatric cerebral arteriovenous malformations  

Microsoft Academic Search

Background  Arteriovenous malformations (AVMs) are the most common cause of intracerebral hemorrhage in children. Different options exist\\u000a for their successful management consisting of surgery, endovascular embolization, stereotactic radiosurgery, or a combination\\u000a of these treatments.\\u000a \\u000a \\u000a \\u000a \\u000a Discussion  In this paper, we discuss the different treatment modalities in the treatment of pediatric cerebral AVMs emphasizing the role\\u000a of surgery and endovascular embolization as a preoperative

David Rubin; Alejandro Santillan; Jeffrey P. Greenfield; Mark Souweidane; Howard A. Riina

2010-01-01

191

Toward postnatal reversal of ocular congenital malformations.  

PubMed

Aniridia is a panocular disorder that severely affects vision in early life. Most cases are caused by dominantly inherited mutations or deletions of the PAX6 gene, which encodes a transcription factor that is essential for the development of the eye and the central nervous system. In this issue of the JCI, Gregory-Evans and colleagues demonstrate that early postnatal topical administration of an ataluren-based formulation reverses congenital malformations in the postnatal mouse eye, providing evidence that manipulation of PAX6 after birth may lead to corrective tissue remodeling. These findings offer hope that ataluren administration could be a therapeutic paradigm applicable to some major congenital eye defects. PMID:24355915

Sahel, José-Alain; Marazova, Katia

2014-01-01

192

[Pelvic arteriovenous malformation : a case report].  

PubMed

A 70-year-old male presented with intermittent macroscopic hematuria. There was no history of previous trauma or pelvic operation. At first, we were unable to clarify the origin of the hematuria, but 3D computed tomography revealed an arteriovenous malformation (AVM) consisting of multiple feeding vessels arising from the bilateral, especially right, internal iliac artery. Treatment with transcatheter arterial embolization (TAE) with a combination of lipiodol and N-butyl-2-cyanoacrylate twice was effective. He needs to be followed up carefully for recurrence of AVM. PMID:23945325

Suzuki, Kotaro; Kusuda, Yuji; Yamada, Yuji; Nose, Ryuichiro; Matsui, Takashi; Sanda, Yuki; Mori, Takeki; Sugimoto, Koji

2013-07-01

193

Opium addiction and cauliflower ears: a case report.  

PubMed

The case of an elderly Chinese male opium addict with cauliflower ears is discussed. He had no history of contact sports that could have led to auricular trauma resulting in deformed ears. Besides cauliflower ears, he had features of chronic bronchitis. The association between opium addiction and cauliflower ears was first described way back in 1932. It was attributed to the prolonged opium induced sleep on hard surface subjecting the ears to repeated pressure and trauma. With the changing pattern of drug abuse, opium abuse related cauliflower ears will become a vanishing sign. PMID:2017717

Muthusamy, E

1991-02-01

194

Correlation between morbid adenoid and atelectatic ear.  

PubMed

We observed the nasopharynx of 30 patients with atelectatic ears (AE), and 10 with healthy ear drums and mouth breathing by CT scan, to examine the relation between adenoid and otitis media. The adenoids of those with healthy ear drums were of the large posterior type with free space in the pharyngeal fossa, while those with AE were of the large anterior type in 18 patients and of the pendulous projection type over the choana in the other 12. The anatomical location of the adenoids should be considered when one discusses the relation between the adenoid and otitis media. The "Toynbee phenomenon", in the pendulous projection type and in the large anterior type of adenoid, was considered to be one of the etiological factors in AE. To understand the etiology of otitis media, it seems important to investigate the reason why the anterior projection of the adenoids occurs. PMID:3478943

Kowata, I; Awataguchi, T

1987-01-01

195

Precise individualized armature for ear reconstruction  

NASA Astrophysics Data System (ADS)

The cosmetic result of an ear restored surgically or via prosthetics is dependent on the surgeon''s ability to carve a precise cartilage armature at the time of surgery or the prosthetist''s ability to sculpt in wax an exact duplicate of the patient''s " missing" ear. Introducing CAD/CAM technology into the process benefits the esthetic outcome of these procedures. By utilizing serial section information derived from CAT MRI or moulage techniques a mirrorimage of the patient''s " donor" ear is generated. The resulting earform data is then used for the design of a cartilage armature produced by multi-axis milling or to produce by stereolithography a model which serves as the basis for a prosthesis.

Evenhouse, Raymond J.; Chen, Xiaoming

1991-04-01

196

Scanning electron microscopic studies on floral malformation in mango  

Microsoft Academic Search

Studies with scanning electron microscope in mango variety Amrapali revealed the presence of many hair line cracks, pin-sized to large holes, disorganised cells and fungal mycelial infection at the base of the malformed bud during bud-inception stages. Among other possible reasons, tissue damage caused by the fungi and sucking pests may lead to the abnormal development of the malformed tissues.

K. Usha; A. M. Goswami; H. C. Sharma; Bhupinder Singh; P. C. Pande

1997-01-01

197

Pulmonary arteriovenous malformation: a rare anterior mediastinal mass.  

PubMed

Pulmonary arteriovenous malformations are rare pulmonary vascular lesions which are associated with Osler Weber Rendu syndrome (hereditary haemorrhagic telangectasia). They act as right-to-left shunts and have cardiovascular and embolic complications. We present a patient with an apparent anterior mediastinal mass secondary to a pulmonary arteriovenous malformations which was successfully treated percutaneously. PMID:23043574

Walklin, Ryan Patrick; Entwisle, James; Cheung, Ying Kei; Dayal, Viswas

2012-10-01

198

History, anatomic forms, and pathogenesis of Chiari I malformations  

Microsoft Academic Search

IntroductionChiari I malformations constitute a group of entities of congenital or acquired etiology that have in common descent of the cerebellar tonsils into the cervical spinal canal. In recent years, since the advent of magnetic resonance imaging, an increasing number of asymptomatic, doubtfully symptomatic, and minimally symptomatic patients with Chiari I malformations have been diagnosed. This has resulted in controversy

Edgardo Schijman; C. Durand

2004-01-01

199

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

200

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 recently metamorphosed frogs that were collected in Minnesota, Vermont, Wisconsin and Maine and examined on the northern leopard frog (Rana pipiens). The malformations and the method of their classification are reported

Torgersen, Christian

201

Abernethy malformation with portal vein aneurysm in a child  

PubMed Central

Abernethy malformation is an extremely rare anomaly of the splanchnic venous system. We describe multidetector computed tomography findings of an incidentally detected Abernethy malformation with portal vein aneurysm in a two-and-half-year old child. The computed tomography scan was performed for the evaluation of respiratory distress, poor growth, and loss of appetite. PMID:21430844

Chandrashekhara, Sheragaru H.; Bhalla, Ashu Seith; Gupta, Arun Kumar; Vikash, C. S.; Kabra, Susheel Kumar

2011-01-01

202

Counseling pregnant women treated with paroxetine Concern about cardiac malformations  

Microsoft Academic Search

QUESTION I have always reassured my patients that taking selective serotonin reuptake inhibitors (SSRIs) during pregnancy would not increase their risk of having children with major malformations. A recent warning from Health Canada, based on results of a study from GlaxoSmithKline, stated that infants exposed to paroxetine might be at higher risk of congenital malformations, specifi cally cardiovascular defects. Some

Adrienne Einarson; Gideon Koren

203

Nursery care of the newborn with malformation syndrome  

Microsoft Academic Search

Congenital malformations are not so rare, and represent a relevant challenge to health care providers. We delineate a methodology for the clinical approach to the malformed newborn, that takes into account the needs of the patient as single person and as part of a familiar and social context. We consider first the medical problem that the neonatologists face in the

Francesca Romana Pluchinotta; Luigi Memo

2009-01-01

204

Surgical Treatment of Chiari I Malformation, with Preservation of Arachnoid  

Microsoft Academic Search

Objective: The author describe the results of performing a standard posterior craniovertebral decompression and duroplasty, with preservation of arachnoid layer, for the treatment of patients with symptomatic chiari I malformation with and without syringomyelia Patients and methods: This is a retrospectively analyzed series of ten patients with symptomatic chiari I malformation underwent surgical treatment from June 2003 to January 2007.

Alaa A. Farag

2007-01-01

205

Cerebral cavernous malformations (cavernomas) in the pediatric age-group  

Microsoft Academic Search

Cavernomas are vascular malformations composed of a compact mass of sinusoidal-type vessels that are immediately contiguous with one another and have no intervening parenchyma. Cavernous malformations were previously held to be a rare pathology occurring predominantly in adults. New neuroradiological techniques such as computed tomography (CT) and magnetic resonance imaging (MRI) demonstrate, on the contrary, that these lesions are also

C. Mazza; R. Scienza; A. Beltramello; R. Da Pian

1991-01-01

206

Intralesional laser therapy for vascular malformations.  

PubMed

Intralesional laser therapy for the treatment of vascular malformations (VMs) has been previously reported for select patient populations. Larger studies, over a wider variety of indications, are needed to better define the potential role of this technology. In the current study, a 12-year, retrospective review of 44 patients who underwent 73 intralesional Nd:YAG or diode laser treatments of VMs was performed. The most commonly encountered lesions were venous malformations (66%) and the most commonly involved anatomic locations were the head and neck regions (41%) and lower extremity (39%). Primary indications for treatment were enlargement (73%) and pain (52%). Lesion size was reduced in 94% of cases after treatment and pain was improved in 91% of cases. Minor postoperative complications occurred in 16 (36%) patients. There was no difference in treatment response among various VM subtypes or anatomic locations (P = 0.497, P = 0.866) or in the incidence of complications (P = 0.531, P = 0.348). Age was the only factor associated with an increased risk of complications (odds ratio, 1.034; P = 0.038). When used in accordance with the suggested guidelines, intralesional laser therapy is a safe and effective treatment modality for VMs of varying compositions and locations. PMID:24625513

Ma, Linda W; Levi, Benjamin; Oppenheimer, Adam J; Kasten, Steven J

2014-11-01

207

Middle ear cholesteatoma in 11 dogs  

PubMed Central

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.

2011-01-01

208

The study of malformations "by the company they keep".  

PubMed Central

Our study of individuals with thalidomide embryopathy reaffirms many of the principles of clinical teratology concerning sensitivity of developing structures and specificity of action. It also highlights important features of ocular teratology that differ from those of other organ systems. Because malformations of the eye and associated structures do not significantly threaten the survival of the embryo, fetus, or neonate, any variation in prevalence in older individuals is not due to the nature of the ocular malformation but may be significantly modified by "the company they keep," the systemic malformations occurring at the same time as the original insult, whether it be an environmental, a genetic, or a local disturbance. Additionally, the presence of clusters of malformations caused by a teratogen at a specific time supports the concept of a common pathogenic mechanism and provides suggested avenues for further research into the cause of these malformations. PMID:1494822

Miller, M T; Stromland, K

1992-01-01

209

38 CFR 4.87 - Schedule of ratings-ear.  

Code of Federal Regulations, 2013 CFR

...6208Malignant neoplasm of the ear (other than skin only) 100 Note: A rating of 100... 6209Benign neoplasms of the ear (other than skin only): Rate on impairment of function. 6210Chronic otitis externa:...

2013-07-01

210

38 CFR 4.87 - Schedule of ratings-ear.  

Code of Federal Regulations, 2012 CFR

...6208Malignant neoplasm of the ear (other than skin only) 100 Note: A rating of 100... 6209Benign neoplasms of the ear (other than skin only): Rate on impairment of function. 6210Chronic otitis externa:...

2012-07-01

211

38 CFR 4.87 - Schedule of ratings-ear.  

Code of Federal Regulations, 2011 CFR

...6208Malignant neoplasm of the ear (other than skin only) 100 Note: A rating of 100... 6209Benign neoplasms of the ear (other than skin only): Rate on impairment of function. 6210Chronic otitis externa:...

2011-07-01

212

38 CFR 4.87 - Schedule of ratings-ear.  

...87 Schedule of ratings—ear. Diseases of the Ear Rating 6200Chronic suppurative otitis media, mastoiditis, or cholesteatoma (or any combination): During suppuration, or with aural polyps 10 Note: Evaluate hearing impairment,...

2014-07-01

213

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/ ...

214

14 CFR 67.105 - Ear, nose, throat, and equilibrium.  

...CONTINUED) AIRMEN MEDICAL STANDARDS AND CERTIFICATION First-Class Airman Medical Certificate § 67.105 Ear, nose...equilibrium. Ear, nose, throat, and equilibrium standards for a first-class airman medical certificate are: (a) The...

2014-01-01

215

Approche psychologique de la malformation congénitale de la main.La malformation congénitale, le désir de savoir  

Microsoft Academic Search

The psychological approach to congenital malformation is determined by the stares of the others and the individual?s needs. The relationship of the body of the person afflicted with a congenital malformation is a question of aesthetic suffering and not of functional use. Caught up in a network of a priori and anxiety- based projections, it is difficult for patients, children,

A. Didierjean-Pillet

2002-01-01

216

Identification of direct downstream targets of Dlx5 during early inner ear development  

PubMed Central

Dlx5, a homeobox transcription factor, plays a key role in the development of many organ systems. It is a candidate gene for human split-hand/split-foot type 1 malformation associated with sensorineural hearing loss. A deletion of one of its enhancers has been implicated in human craniofacial defects/hearing loss and it has also been associated with autism. However, little is known of how Dlx5 exerts its regulatory effects. We identified direct targets of Dlx5 in the mouse inner ear by gene expression profiling wild-type and Dlx5 null otic vesicles from embryonic stages E10 and E10.5. Four hundred genes were differentially expressed. We examined the genomic DNA sequences in the promoter regions of these genes for (i) previously described Dlx5 binding sites, (ii) novel 12 bp long motifs with a canonical homeodomain element shared by two or more genes and (iii) 100% conservation of these motifs in promoters of human orthologs. Forty genes passed these filters, 12 of which are expressed in the otic vesicle in domains that overlap with Dlx5. Chromatin immunoprecipitation using a Dlx5 antibody confirmed direct binding of Dlx5 to promoters of seven of these (Atbf1, Bmper, Large, Lrrtm1, Msx1, Ebf1 and Lhx1) in a cell line over-expressing Dlx5. Bmper and Lrrtm1 were up-regulated in this cell line, further supporting their identification as targets of Dlx5 in the inner ear and potentially in other organs. These direct targets support a model in which Bmp signaling is downstream of Dlx5 in the early inner ear and provide new insights into how the Dlx5 regulatory cascade is initiated. PMID:21227998

Sajan, Samin A.; Rubenstein, John L.R.; Warchol, Mark E.; Lovett, Michael

2011-01-01

217

Tympanosclerosis of the ear drum. A scanning electronmicroscopic study.  

PubMed

15 specimens from tympanosclerotic ear drums were studied by scanning electron microscope. Comparative studies were made in 5 normal ear drums and 21 specimens from ear drums in secretory otitis media. Calcification of the ear drum affected mainly the fibrous layer. The submucosa seemed stiff with congested vessels. The degeneration and calcification in the fibrous layer started medially near the submucosa, involving the inner circular fibres first. Signs of regeneration were seen, with fibrocyte-like cells producing fibrils. PMID:7257755

Mřller, P

1981-01-01

218

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

219

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

220

Diving injuries to the inner ear  

Microsoft Academic Search

Two skin divers and 7 SCUBA divers, all men, aged 21–33 years, are presented. The injury occurred at shallow depths and difficulties with pressure equilibration to the ears were a common complaint. Vertigo and hearing losses. When a perilymph fistula is suspected and decompression sickness can be in the round, the other in the oval window. The latter patient also

O. I. Molvćr; E. Natrud; S. Eidsvik

1978-01-01

221

The Fine Structure of the Insect Ear  

Microsoft Academic Search

A brief account is given of observations of the fine structure of the insect ear by light microscopy. The observations generally confirm those of previous workers. The electron microscope has revealed many new structures in the ganglion. Problems of the structure of the neuron and its sensory hair, the scolopale, and the relationships of the membranes of the neuron and

E. G. Gray

1960-01-01

222

Ectopic muscle in the middle ear  

Microsoft Academic Search

An ectopic muscle was found in the hypotympanum of a 31-year-old Japanese male. The muscle produced symptoms quite similar to those seen in cases of glomus jugulare tumor: a red mass visible through the ear drum, conductive deafness, a compressed jugular bulb demonstrable by retrograde jugulography, and VIIth and IXth nerve paresis. However, the audible pulsating tinnitus of glomus jugulare

Kiyotaka Murata; Haruo Saito; Manabi Hinoki

1977-01-01

223

Ear Tracking: Visualizing Auditory Localization Strategies  

E-print Network

Ear Tracking: Visualizing Auditory Localization Strategies W. Joseph King Suzanne J. Weghorst Human further cluttering the visual channel. Auditory displays may be used to reinforce the information which,weghorst}@hitl.washington.edu ABSTRACT Auditory displays are an ongoing topic of human computer interaction researchandhave been shown

Washington at Seattle, University of

224

Do Your Ears Pop in Space?  

Microsoft Academic Search

R Mike Mullane is a US astronaut who has flown into space three times on the Space Shuttle. He resigned from NASA in 1990 and has since pursued a career as a professional speaker and author. Do Your Ears Pop in Space? is his third book, and is based on the simple idea of writing down the 500 questions he

Robert Lambourne

1997-01-01

225

Ear Acupuncture in European Traditional Medicine  

Microsoft Academic Search

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.

Luigi Gori; Fabio Firenzuoli

2007-01-01

226

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

227

Middle Ear Implantable Hearing Devices: An Overview  

PubMed Central

Hearing loss affects approximately 30 million people in the United States. It has been estimated that only approximately 20% of people with hearing loss significant enough to warrant amplification actually seek assistance for amplification. A significant interest in middle ear implants has emerged over the years to facilitate patients who are noncompliant with conventional hearing aides, do not receive significant benefit from conventional aides, or are not candidates for cochlear implants. From the initial studies in the 1930s, the technology has greatly evolved over the years with a wide array of devices and mechanisms employed in the development of implantable middle ear hearing devices. Currently, these devices are generally available in two broad categories: partially or totally implantable using either piezoelectric or electromagnetic systems. The authors present an up-to-date overview of the major implantable middle ear devices. Although the current devices are largely in their infancy, indications for middle ear implants are ever evolving as promising studies show good results. The totally implantable devices provide the user freedom from the social and practical difficulties of using conventional amplification. PMID:19762429

Haynes, David S.; Young, Jadrien A.; Wanna, George B.; Glasscock, Michael E.

2009-01-01

228

Carbon Dioxide Exchange via the Mucosa in Healthy Middle Ear  

Microsoft Academic Search

Background: Recent studies have shown that gas ex- change via the middle ear mucosa, which is performed be- tween the middle ear cleft and capillaries in the submu- cosal connective tissue, has an essential role in ventilation and pressure regulation in the middle ear cleft. We specu- lated that gas exchange via the mucosa is induced by the gas diffusion

Fumio Ikarashi; Sugata Takahashi; Yutaka Yamamoto

1999-01-01

229

Early Ear Problems and Developmental Problems at School Age  

Microsoft Academic Search

Retrospective history of middle ear disease was compared with developmental diagnosis in 507 consecutively referred school-age children. History of major ear problems was positively associated with discrepancies between the performance and verbal IQ on the WISC-R. History of major ear problems was positively associated with the presence of articulation disorders for children in the low social class, hyperactivity in the

Ronald L. Lindsay; Terry Tomazic; Barbara Y. Whitman; Pasquale J. Accairdo

1999-01-01

230

Sources of Variability in Reflectance Measurements on Normal Cadaver Ears  

E-print Network

-ear lesions for all ages, (2) determine the duration of fluid in the ears of children prone to otitis media closed off at the aditus ad antrum (small air space). Results: Measurement-location effects are generally systematically as the measurement location moves away from the tympanic membrane but in other ears the effects

Allen, Jont

231

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

232

Multimodal device for assessment of skin malformations  

NASA Astrophysics Data System (ADS)

A variety of multi-spectral imaging devices is commercially available and used for skin diagnostics and monitoring; however, an alternative cost-efficient device can provide an advanced spectral analysis of skin. A compact multimodal device for diagnosis of pigmented skin lesions was developed and tested. A polarized LED light source illuminates the skin surface at four different wavelengths - blue (450 nm), green (545 nm), red (660 nm) and infrared (940 nm). Spectra of reflected light from the 25 mm wide skin spot are imaged by a CMOS sensor. Four spectral images are obtained for mapping of the main skin chromophores. The specific chromophore distribution differences between different skin malformations were analyzed and information of subcutaneous structures was consecutively extracted.

Bekina, A.; Garancis, V.; Rubins, U.; Spigulis, J.; Valeine, L.; Berzina, A.

2013-11-01

233

Malformations and the Manx Syndrome in Cats  

PubMed Central

Breeding experiments were conducted on cats with congenital taillessness, to test the dissemination pattern of taillessness in their offspring. Clinical evaluation, radiographic analysis of the vertebral column and histological studies of the digestive tract and central nervous tissue were conducted to determine the association of malformations of these systems in cats born with different degrees of taillessness noted in the rumpy and stumpy cats. The mode of transmission of the tailless (Manx) condition assumed to be through an autosomal dominant factor (M) was confirmed by this investigation. It is hypothesized that the problems associated with the tailless condition such as spina bifida, urinary and faecal incontinence and locomotor disturbances of the pelvic limbs may all be related to a disturbance affecting the development of the central nervous system in the early embryonic life. ImagesFigure 3.Figure 4.Figure 5.Figure 6.Figure 7.Figure 8.Figure 9.Figure 10. PMID:393376

DeForest, M. E.; Basrur, P. K.

1979-01-01

234

[The hand: embryology and main malformative mechanisms].  

PubMed

Upper limb bud appears in the cervical region of the embryo during the fifth week of development. It is made of epithelia and underlying mesenchyme. Diffusible growth factors, expressed by the apical ectodermal ridge, direct the proximal-distal growth. Other factors are expressed by zone of polarizing activity and ectoderm. They induce together anterior-posterior growth and dorsal-ventral polarity of the limb bud. The development of axial skeleton pattern is controlled by transcription factors from the HOX family, which are expressed in a stripe along the proximal and distal edges of the limb bud. Embryologic mechanisms of the main hand malformations are described, as well as their known genetic or mechanical aetiologies. PMID:18838286

Perrin, J; Geoffroy-Siraudin, C; Metzler-Guillemain, C

2008-12-01

235

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

236

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

237

Laparoscopic excision of a retroperitoneal lymphatic malformation in a newborn.  

PubMed

Abdominal lymphatic malformations may be challenging to eradicate. Retroperitoneal lesions may more difficult to resect than mesenteric ones; however, the latter may predispose to intestinal volvulus, leading to calls for their prompt excision. Such lesions identified perinatally may pose particular challenges: in one case, respiratory failure caused by abdominal distension required emergency drainage followed by later laparoscopic excision; laparoscopy has also been used promptly to diagnose and resect neonatal mesenteric lymphatic malformations with their inherent volvulus risk. We illustrate that even if neonatal laparoscopy identifies a retroperitoneal rather than mesenteric lymphatic malformation, curative endosurgical excision remains feasible. PMID:21292064

Solari, Valeria; Mullassery, Dhanya; Lansdale, Nick; Jesudason, Edwin C

2011-02-01

238

Neurosurgical management of congenital malformations of the brain.  

PubMed

Congenital malformations encompass a diverse group of disorders that often present at birth, either as the result of genetic abnormalities, infection, errors of morphogenesis, or abnormalities in the intrauterine environment. Congenital disorders affecting the brain are now often diagnosed before delivery with the use of prenatal ultrasonography. Over the past several decades, there have been major advances in the understanding and management of these conditions. This review focuses on the most common cranial congenital malformations, limiting the discussion to the neurosurgically relevant aspects of arachnoid cysts, pineal cysts, Chiari malformations, and encephaloceles. PMID:21807319

Hervey-Jumper, Shawn L; Cohen-Gadol, Aaron A; Maher, Cormac O

2011-08-01

239

Imaging of Chiari type I malformation and syringohydromyelia.  

PubMed

Chiari malformations are anatomic anomalies that comprise a broad spectrum of neurologic conditions. The most common malformation, a Chiari type I malformation, can present with a variety of signs and symptoms, most frequently an occipital Valsalva-induced headache. Cranial and spinal magnetic resonance (MR) imaging is used to identify the degree of tonsillar descent and document the presence of syringohydromyelia. The advent of cine-MR flow imaging (cine as in "cinema") has provided new insight as to the dynamic process involved in the evolution of this pathophysiology. This article reviews the neuroimaging of this fascinating disorder. PMID:24287386

McVige, Jennifer W; Leonardo, Jody

2014-02-01

240

Complex malformations of the female genital tract. New types and revision of classification  

Microsoft Academic Search

BACKGROUND: Complex malformations of the female genital tract are often incorrectly identified, treated and reported, probably due to not considering the malformation as a cause of the clinical symptoms and neither the embryological origin of the different elements of the genitourinary tract. METHODS: Complex malformations are studied and classified, and new types are presented. The new types of complex malformations

Pedro Acien; Maribel Acien; Marisa Sanchez-Ferrer

241

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

242

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

243

Genetics Home Reference: Megalencephaly-capillary malformation syndrome  

MedlinePLUS

... Registry: Megalencephaly cutis marmorata telangiectatica congenita M-CM Network: How is M-CM Diagnosed? You might also find information on the diagnosis or management of megalencephaly-capillary malformation syndrome in Educational resources ...

244

[Cystic adenomatoid malformation of the lung. Importance of prenatal diagnosis].  

PubMed

Cystic adenomatoid malformation of the lung is a rare malformation of the lung airway which often performed diagnosed in the prenatal period by ultrasound. Ultrasound monitoring should be performed during pregnancy to assess lung development. We report the case of a 4-year-old patient with prenatal diagnosis of cystic adenomatoid malformation of the lung, not confirmed by chest radiograph at birth. The patient underwent surgery at 4 years of age after diagnosis was made for presenting recurrent pneumonia. A normal chest radiograph at birth does not exclude this malformation and a computerized tomography at 4 weeks of birth must be done to confirm or rule out this anomaly. Once the diagnosis is made, surgical treatment should be prompted to avoid complications. PMID:21465061

Cabeza, Beatriz; Ońoro, Gonzalo; Cantarín Extremera, Verónica; Sanz Santiago, Verónica; Sequeiros, Adolfo

2011-04-01

245

Theory of forward and reverse middle-ear transmission applied to otoacoustic emissions in infant and adult ears  

PubMed Central

The purpose of this study is to understand why otoacoustic emission (OAE) levels are higher in normal-hearing human infants relative to adults. In a previous study, distortion product (DP) OAE input/output (I/O) functions were shown to differ at f2=6 kHz in adults compared to infants through 6 months of age. These DPOAE I/O functions were used to noninvasively assess immaturities in forward/reverse transmission through the ear canal and middle ear [Abdala, C., and Keefe, D. H., (2006). J. Acoust Soc. Am. 120, 3832–3842]. In the present study, ear-canal reflectance and DPOAEs measured in the same ears were analyzed using a scattering-matrix model of forward and reverse transmission in the ear canal, middle ear, and cochlea. Reflectance measurements were sensitive to frequency-dependent effects of ear-canal and middle-ear transmission that differed across OAE type and subject age. Results indicated that DPOAE levels were larger in infants mainly because the reverse middle-ear transmittance level varied with ear-canal area, which differed by more than a factor of 7 between term infants and adults. The forward middle-ear transmittance level was ?16 dB less in infants, so that the conductive efficiency was poorer in infants than adults. PMID:17348521

Keefe, Douglas H.; Abdala, Carolina

2008-01-01

246

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

247

Cloacal malformations: lessons learned from 490 cases.  

PubMed

In this review we describe lessons learned from the authors' series of patients born with the most complex of congenital anorectal problems, cloacal malformations, with the hope to convey the improved understanding and surgical treatment of the condition's wide spectrum of complexity learned from patients cared for over the last 25 years. This includes a series of 490 patients, 397 of whom underwent primary operations, and 93 who underwent reoperations after attempted repairs at other institutions. With regard to the newborn, we have learned that the clinician must make an accurate neonatal diagnosis, drain a hydrocolpos when present, and create an adequate, totally diverting colostomy, leaving enough distal colon available for the pull-through, and a vaginal replacement if needed. A correct diagnosis will avoid repairing only the rectal component. For the definitive reconstruction, all patients in the series were managed with a posterior sagittal approach; 184 of whom also required a laparotomy. The average length of the common channel was 4.6 cm for patients who required a laparotomy and 2.5 cm for those who did not. Hydrocolpos was present in 139 patients (30%). Vaginal reconstruction involved a vaginal pull-through in 308 patients, a vaginal flap in 44, vaginal switch in 48, and vaginal replacement in 90 (33 with rectum, 15 with colon, and 42 with small bowel). A total of 220 underwent total urogenital mobilization, which was first introduced in 1996. Complications included rectal prolapse in 26, vaginal stricture or atresia in 18, urethrovaginal fistula in 13, and urethral atresia in 6. A total of 53% of all cases have voluntary bowel movements. The others are kept clean with a mechanical daily emptying (an enema) as part of a bowel management program. Indications for reoperations included persistent urogenital sinus after initial repair in 39 patients. Fifty-one had problems such as rectal prolapse, stricture, retraction, dehiscence or atresia, 29 had a mislocated rectum, 34 had vaginal stricture, retraction, dehiscence, atresia, or stenosis, 16 had urethrovaginal or rectovaginal fistulae, and 5 had urethral stricture or atresia. The series was divided into 2 distinct groups of patients where common channel measurement was known (n = 400): group A were those with a common channel < or = 3.0 cm (n = 225, 56%) and group B had a common channel >3 cm (n = 175, 44%). The separation into these 2 groups has important therapeutic and prognostic implications. Patients in Group A can be repaired posterior sagittally with a reproducible operation. Because they represent most patients, most well-trained pediatric surgeons should be able to repair these types of malformations, and the prognosis is good. Patients in Group B (those with a common channel >3 cm), usually require a laparotomy, have a much higher incidence of associated urological problems, and often require special maneuvers for vaginal reconstruction. Surgeons who repair Group B malformations require special training in urology; the operations are prolonged and technically demanding, and the functional results are not as good as in group A. PMID:20307849

Levitt, Marc A; Peńa, Alberto

2010-05-01

248

Analysis of OH Bolted Ear Connection  

SciTech Connect

The D0 endcap calorimeter outer hadronic (OH) modules play a major structural role in the calorimeter assembly. The disrete modules, once connected together, form a ring within which other massive calorimetry will reside. It has been proposed that the connection of the OH at the downstream end be accomplished by extending the downstream endplates in the radial direction to form 'ears', and then through-bolting between adjacent ears as shown in Fig. 1. A single 2 1/4 in. dia. bolt is used, and previous calculations have determined that the design load on this joint should be 130,000 lbs tension. The high load and serious consequences of failure make this a critical component in the calorimeter assembly. The purpose of this analysis is to investigate the stresses in the connection and other mechanical characteristics which determine joint performance.

Wands, Bob; /Fermilab

1987-12-30

249

Coil Embolization of a Neonatal Pulmonary Arteriovenous Malformation  

Microsoft Academic Search

Pulmonary arteriovenous malformation (PAVM), as a part of Osler-Weber-Rendu Syndrome, in the neonate, is a rare hereditary vascular malformation. Large intrapulmonary right-to-left shunting, causing hypoxaemia and cyanosis, can be a life-threatening condition. Repeated transcatheter coil embolization procedures proved to be a favorable strategy to improve systemic arterial oxygen saturation, with a good outcome in a newborn child. While the radiation

R. G. Bennhagen; G. Holje; S. Laurin; E. Pesonen

2002-01-01

250

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

251

Lymphatic malformations: A dilemma in diagnosis and management  

PubMed Central

Lymphatic malformations of the head and neck, also known as lymphangiomas or cystic hygromas, are a diverse group of lesions. Lymphangiomas represent benign hamartomatous tumors of lymphatic vessels with a marked predilection for the head, neck and oral cavity. These lesions, like most of the swellings occurring in the neck, frequently pose a dilemma in diagnosis and treatment. This is a case report of a lymphatic malformation which presented as a painless mass in the lower border of mandible. PMID:24808711

Ramashankar; Prabhakar, Chandan; Shah, Nishit Kumar; Giraddi, Girish

2014-01-01

252

The Arnold-Chiari Type II Malformation at Midgestation  

Microsoft Academic Search

The Arnold-Chiari malformation type II (ACMII) is reported to be reversible after closure of a myelomeningocele at midgestation. To elucidate the developmental state of the ACMII malformation at the approximate time fetal surgery is performed, the ACMII of a 20-week human fetus was investigated in vitro using high-field magnetic resonance microscopy at 9.4 T and compared with the hindbrain of

E. Beuls; L. Vanormelingen; J. van Aalst; M. Vandersteen; P. Adriaensen; E. Cornips; H. Vles; Y. Temel; J. Gelan

2003-01-01

253

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

254

[Lung perfusion scintigraphy in pediatric patients with congenital malformations].  

PubMed

Congenital malformations are an heterogeneous group of disorders that can lead changes in pulmonary perfusion and then can be evaluated with lung perfusion scintigraphy. We selected five patients in which the role of lung perfusion scintigraphy emerges as a reliable and non-invasive imaging technique. Lung perfusion scintigraphy is a useful tool in pediatric patients with congenital malformations allowing an accurate evaluation of the best therapeutic strategy and its results. PMID:24042425

Niccoli Asabella, Artor; Stabile Ianora, Amato Antonio; Di Palo, Alessandra; Rubini, Domenico; Pisani, Antonio Rosario; Ferrari, Cristina; Notaristefano, Antonio; Rubini, Giuseppe

2013-01-01

255

Post-mortem examination of prenatally diagnosed fatal renal malformation  

Microsoft Academic Search

Objective:Renal malformations can be associated with genetic syndromes and chromosomal disorders. Fetal autopsy including histopathological examination of kidney is important to arrive at definite diagnosis. The objective was to assess importance of fetal autopsy and histopathology.Study Design:Retrospective analysis of cases with fetal renal malformations was done. All fetuses terminated were examined with whole body radiograph, external and internal examination and

N Kumari; M Pradhan; V H Shankar; N Krishnani; S R Phadke

2008-01-01

256

Three-dimensional spiral CT of craniofacial malformations in children  

Microsoft Academic Search

Objective. To assess the value of three-dimensional CT (3D CT) in the diagnosis and management of suspected paediatric craniofacial\\u000a malformations.¶Materials and methods. Twenty-eight children (12 girls, 16 boys) with a mean age of 4 years, suffering from craniofacial or cervical malformations,\\u000a underwent craniofacial spiral CT. 3D reformatting was performed using an independent workstation.¶Results. 3D CT allowed the preoperative evaluation of

S. Binaghi; F. Gudinchet; B. Rilliet

2000-01-01

257

The glue ear 'epidemic': a historical perspective.  

PubMed

This paper explores the historical context of the dramatic rise in surgery for glue ear in the mid-20th century, and questions the published assertion that this represented a manufactured 'epidemic'. In examining historical sources, the reader's theoretical viewpoint greatly influences their conclusions: the sustained rise in treatment for glue ear may be seen as the advance of science in a golden age or the resistance of insular professionals to reason in the light of new scientific study methods. Current views on the practice of medicine, consumerism, science and standardisation, rationing and the nature of 'truth' all affect the way that we see this period. Technological advances clearly allowed better diagnosis and more effective treatment, but these did not appear to drive an 'epidemic', rather they were developed to meet the pre-existing challenges of otological practice. The proposition that an 'epidemic' was created does not appear to have any solid grounding. Society's perception of what constitutes disease and what needs treatment may have evolved, but the prevalence of other important diseases changed dramatically over this time period, and a real change in the epidemiology of glue ear cannot be dismissed. In defining the case for and against surgical treatment, a solely positivist, quantitative worldview cannot give us a complete picture of benefit and risk to individuals, families and society at large. PMID:21653931

Alderson, David

2011-12-01

258

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

259

Ethical Aspects of Prenatal Diagnosis of Fetal Malformations  

PubMed Central

Fetal malformations complicate approximately 3% of all pregnancies. Many of these are minor or can be corrected after birth, but there are certain malformations that are lethal and others that are severe and others, that, even if corrected lead to permanent disability. Advances in prenatal diagnosis made possible the diagnosis of many fetal malformations. This led to the concept of the fetus a patient, independent of the pregnant woman, even though the moral status of the fetus is in dispute. Many of the lethal malformations are untreatable. However, for some, innovative in utero treatments, both medical and surgical, became possible. These interventions should be evaluated for the relative benefit and risk for both the fetus and the mother, because any such treatment has to involve the integrity of her body. This raises the ethical question of beneficence (to the fetus) versus the autonomy of the pregnant woman. The process of resolving this issue will be discussed, especially how to obtain a truly informed consent. For the lethal malformations or for those severe or multiple malformations whose treatment is theoretically possible but the results of such treatment are unpredictable or may lead to life long disabilities and serious burdens for the infant or child and the family, prenatal counseling should include “prenatal advance directive” and a plan for palliative care, the components of which will be described. PMID:23610506

Fadel, Hossam E.

2012-01-01

260

Syndromes associated with vascular tumors and malformations: a pictorial review.  

PubMed

Use of the International Society for the Study of Vascular Anomalies (ISSVA) classification system has been strongly recommended in recent years because of the need for separate therapeutic measures for patients with vascular tumors and malformations. In the ISSVA classification system, vascular tumors, which are neoplastic, are distinguished from vascular malformations, which are caused by vascular structural anomalies and are not neoplastic, on the basis of the presence or absence of neoplastic proliferation of vascular endothelial cells. It is important that radiologists be familiar with the development, diagnosis, and treatment of vascular tumors and malformations, especially the imaging features of low- and high-flow vascular malformations. Some vascular tumors and malformations develop in isolation, whereas others develop within the phenotype of a syndrome. Syndromes that are associated with vascular tumors include PHACE syndrome. Syndromes that are associated with vascular malformations include Sturge-Weber, Klippel-Trénaunay, Proteus, blue rubber bleb nevus, Maffucci, and Gorham-Stout syndromes, all of which demonstrate low flow, and Rendu-Osler-Weber, Cobb, Wyburn-Mason, and Parkes Weber syndromes, all of which demonstrate high flow. Because imaging findings may help identify such syndromes as systemic, it is important that radiologists familiarize themselves with these conditions. PMID:23322836

Nozaki, Taiki; Nosaka, Shunsuke; Miyazaki, Osamu; Makidono, Akari; Yamamoto, Asako; Niwa, Tetsu; Tsutsumi, Yoshiyuki; Aida, Noriko; Masaki, Hidekazu; Saida, Yukihisa

2013-01-01

261

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

262

A review of cavernous malformations with trigeminal neuralgia.  

PubMed

Cavernous malformation with trigeminal neuralgia is relatively rare; only 10 cases have been reported. In deciding treatment strategies, it is helpful to classify cavernous malformation according to its origin, as follows: in the Gasserian ganglion (Type G); between the cisternal and intra-axial portions of the trigeminal nerve root (Type C); in the intra-axial trigeminal nerve root in the pons (Type P); or in the spinal tract of the trigeminal nerve root (Type S). A 62-year-old male presented with left trigeminal neuralgia (V2 area) and left facial hypoesthesia. Imaging studies revealed a cerebellopontine angle mass lesion with characteristics of a cavernous malformation and evidence of hemorrhage. The lesion was completely removed via a left anterior transpetrosal approach. The mass was attached to the trigeminal nerve root; it was located between the cisternal and intra-axial portions of the nerve root, and feeding off microvessels from the trigeminal nerve vascular plexus. Histological examination confirmed a cavernous malformation. In this case, the cavernous malformation was Type C. We review cases of cavernous malformation with trigeminal neuralgia and discuss therapeutic strategies according to the area of origin. PMID:25129386

Adachi, Kazuhide; Hasegawa, Mitsuhiro; Hayashi, Takuro; Nagahisa, Shinya; Hirose, Yuichi

2014-10-01

263

Hypofractionated stereotactic radiotherapy for large arteriovenous malformations  

PubMed Central

Cerebral arteriovenous malformations (AVMs) are abnormal connections between the arteries and veins, with possible serious consequences of intracranial hemorrhage. The curative treatment for AVMs includes microsurgery and radiosurgery, sometimes with embolization as an adjunct. However, controversies exist with the treatment options available for large to giant AVMs. Hypofractionated stereotactic radiotherapy (HSRT) is one treatment option for such difficult lesions. We aim to review recent literature, looking at the treatment outcome of HSRT in terms of AVM obliteration rate and complications. The rate of AVM obliteration utilizing HSRT as a primary treatment was comparable with that of stereotactic radiosurgery (SRS). For those not totally obliterated, HSRT makes them smaller and turns some lesions manageable by single-dose SRS or microsurgery. Higher doses per fraction seemed to exhibit better response. However, patients receiving higher total dose may be at risk for higher rates of complications. Fractionated regimens of 7 Gy × 4 and 6–6.5 Gy × 5 may be accepted compromises between obliteration and complication. Embolization may not be beneficial prior to HSRT in terms of obliteration rate or the volume reduction. Future work should aim on a prospectively designed study for larger patient groups and long-term follow-up results. PMID:22826813

Wang, Huan-Chih; Chang, Rachel J.; Xiao, Furen

2012-01-01

264

Stereotactic linac radiosurgery for arteriovenous malformations.  

PubMed Central

Stereotactic linear accelerator (linac) radiosurgery has been in operation in the West Midlands since 1987, the first of its kind in the United Kingdom. Forty two patients with high-flow cerebral arteriovenous malformations have been treated, 26 of whom have been followed up. Angiography one year after treatment showed that five lesions were obliterated, 11 were reduced in size and/or flow rate and 10 were unchanged. Overall results show that nine out of 10 patients reviewed at 24 months had total obliteration. Three patients had complications; one has fully recovered, one died of an unrelated cause at 36 months and the other died from recurrent haemorrhage at nine months. Two patients had recurrent non-fatal haemorrhage within 24 months of treatment; both recovered without further deficit. All patients are fit to work but eight are unemployed. Although the follow up period is short, the early results indicate a success rate similar to those published by others using linac radiosurgery. Images PMID:1640237

Kenny, B G; Hitchcock, E R; Kitchen, G; Dalton, A E; Yates, D A; Chavda, S V

1992-01-01

265

The management of arteriovenous malformations in children.  

PubMed

Between January 1941 and June 1989, 46 children below the age of 18 with an arteriovenous malformation (AVM) were managed. There were 7 patients with AVM diagnosed before the age of 2; 10 patients were diagnosed between the ages of 3 and 10; and 29 patients were diagnosed between 11 and 18. There were equal numbers of male and female patients. Twenty-five of the AVMs were large (greater than 5 cm longest diameter). All 7 AVMs diagnosed before the age of 2 were large. The usual clinical presentation was congestive heart failure, bruit and an enlarging head. Three patients underwent excision with 2 deaths and 1 excellent result. In 11 patients (aged 3-18) with AVM without history of hemorrhage, 3 had excision with 2 excellent and 1 fair result. Four remained stable. Four developed progressive deficits or hemorrhage. In 10 patients (aged 3-18) with AVM and hemorrhage who were treated medically, 7 (70%) had an episode of re-hemorrhage. Three patients had excision of AVM after re-hemorrhage, but before the age of 18 with an excellent result. Eighteen patients (aged 3-18) with AVM and a single episode of hemorrhage underwent excision with 17 excellent or good results and 1 fair result. The overall mortality was 7%. Eighty-five percent of the children with excision of AVM had an excellent or good result. The best treatment for AVM in children is surgical excision. PMID:2054808

Malik, G M; Sadasivan, B; Knighton, R S; Ausman, J I

1991-02-01

266

Gas exchange function through the middle ear mucosa in piglets: comparative study of normal and inflamed ears.  

PubMed

The gas exchange function through the middle ear (ME) mucosa was investigated by comparing normal and inflamed ears in an animal model. Piglets were examined (n = 15) because their tympanic bulla closely resembles the human mastoid air cell system. Four untreated ears served as controls. Eleven ears were injected with glycerin into the tympanic bulla to induce inflammation and were studied as inflamed ears. Two respiratory conditions, spontaneous respiration and hyperventilation by a ventilator, were alternated repeatedly. ME pressure was measured intermittently by a tympanometer and blood gas was measured simultaneously. In all four normal ears, both ME pressure and carbon dioxide (CO2) partial pressure in the blood decreased in parallel following alternation of the respiratory conditions from spontaneous respiration to hyperventilation, while both pressure levels increased in parallel when respiration was changed from hyperventilation to spontaneous respiration. This result indicates that there is a gas exchange between the ME and the blood through the mucosa. However, ME pressure change in inflamed ears was limited, though the change in CO2 partial pressure in the blood was the same as that in normal ears. There was a significant difference in the degree of ME pressure change occurring in normal ears compared to that in inflamed ears, suggesting that inflammation of the mucosa reduced gas exchange function in the ME. PMID:10219389

Yamamoto, Y

1999-01-01

267

Effects of ear-canal pressurization on middle-ear bone- and air-conduction responses  

PubMed Central

In extremely loud noise environments, it is important to not only protect one’s hearing against noise transmitted through the air-conduction (AC) pathway, but also through the bone-conduction (BC) pathways. Much of the energy transmitted through the BC pathways is concentrated in the mid-frequency range around 1.5–2 kHz, which is likely due to the structural resonance of the middle ear. One potential approach for mitigating this mid-frequency BC noise transmission is to introduce a positive or negative static pressure in the ear canal, which is known to reduce BC as well as AC hearing sensitivity. In the present study, middle-ear ossicular velocities at the umbo and stapes were measured using human cadaver temporal bones in response to both BC and AC excitations, while static air pressures of ±400 mm H2O were applied in the ear canal. For the maximum negative pressure of ?400 mm H2O, mean BC stapes-velocity reductions of about 5–8 dB were observed in the frequency range from 0.8 to 2.5 kHz, with a peak reduction of 8.6(± 4.7) dB at 1.6 kHz. Finite-element analysis indicates that the peak BC-response reduction tends to be in the mid-frequency range because the middle-ear BC resonance, which is typically around 1.5–2 kHz, is suppressed by the pressure-induced stiffening of the middle-ear structure. The measured data also show that the BC responses are reduced more for negative static pressures than for positive static pressures. This may be attributable to a difference in the distribution of the stiffening among the middle-ear components depending on the polarity of the static pressure. The characteristics of the BC-response reductions are found to be largely consistent with the available psychoacoustic data, and are therefore indicative of the relative importance of the middle-ear mechanism in BC hearing. PMID:19944139

Homma, Kenji; Shimizu, Yoshitaka; Kim, Namkeun; Du, Yu; Puria, Sunil

2014-01-01

268

RASA1 mutations and associated phenotypes in 68 families with capillary malformation-arteriovenous malformation.  

PubMed

Capillary malformation-arteriovenous malformation (CM-AVM) is an autosomal-dominant disorder, caused by heterozygous RASA1 mutations, and manifesting multifocal CMs and high risk for fast-flow lesions. A limited number of patients have been reported, raising the question of the phenotypic borders. We identified new patients with a clinical diagnosis of CM-AVM, and patients with overlapping phenotypes. RASA1 was screened in 261 index patients with: CM-AVM (n = 100), common CM(s) (port-wine stain; n = 100), Sturge-Weber syndrome (n = 37), or isolated AVM(s) (n = 24). Fifty-eight distinct RASA1 mutations (43 novel) were identified in 68 index patients with CM-AVM and none in patients with other phenotypes. A novel clinical feature was identified: cutaneous zones of numerous small white pale halos with a central red spot. An additional question addressed in this study was the "second-hit" hypothesis as a pathophysiological mechanism for CM-AVM. One tissue from a patient with a germline RASA1 mutation was available. The analysis of the tissue showed loss of the wild-type RASA1 allele. In conclusion, mutations in RASA1 underscore the specific CM-AVM phenotype and the clinical diagnosis is based on identifying the characteristic CMs. The high incidence of fast-flow lesions warrants careful clinical and radiologic examination, and regular follow-up. PMID:24038909

Revencu, Nicole; Boon, Laurence M; Mendola, Antonella; Cordisco, Maria Rosa; Dubois, Josée; Clapuyt, Philippe; Hammer, Frank; Amor, David J; Irvine, Alan D; Baselga, Eulalia; Dompmartin, Anne; Syed, Samira; Martin-Santiago, Ana; Ades, Lesley; Collins, Felicity; Smith, Janine; Sandaradura, Sarah; Barrio, Victoria R; Burrows, Patricia E; Blei, Francine; Cozzolino, Mariarosaria; Brunetti-Pierri, Nicola; Vicente, Asuncion; Abramowicz, Marc; Désir, Julie; Vilain, Catheline; Chung, Wendy K; Wilson, Ashley; Gardiner, Carol A; Dwight, Yim; Lord, David J E; Fishman, Leona; Cytrynbaum, Cheryl; Chamlin, Sarah; Ghali, Fred; Gilaberte, Yolanda; Joss, Shelagh; Boente, Maria Del C; Léauté-Labrčze, Christine; Delrue, Marie-Ange; Bayliss, Susan; Martorell, Loreto; González-Enseńat, Maria-Antonia; Mazereeuw-Hautier, Juliette; O'Donnell, Brid; Bessis, Didier; Pyeritz, Reed E; Salhi, Aicha; Tan, Oon T; Wargon, Orli; Mulliken, John B; Vikkula, Miikka

2013-12-01

269

Ear morphology in Treacher Collins', Apert's, and Crouzon's syndromes.  

PubMed

Size, proportions, level, inclination and shape of the ears were assessed by anthrompometric methods in eight patients with Treacher Collins' syndrome, eleven with Apert's, and 25 with Crouzon's syndrome. The ears were most severely damaged in Treacher Collins' syndrome, in which they were microtic or disproportionally long; many were low-set and with great inclination. All patients with Apert's syndrome had low-set ears and a tendency to disproportion, with widening and small inclination of the longitudinal axis. The ears were least affected in Crouzon's syndrome; in two-thirds of the patients there were mild growth variations leading to disproportion in width (wide ears), or low-set ears, or both. PMID:580574

Farkas, L G

1978-03-01

270

Epidemiologic features of male genital malformations and subtypes in Texas.  

PubMed

Although distinct categories of male genital malformations share some common risk factors, few studies have systematically compared epidemiologic features across phenotypes. We evaluated the relationship between several maternal and infant characteristics and five categories of male genital malformations: second- or third-degree hypospadias, hypospadias (regardless of degree), small penis, cryptorchidism, and any male genital malformation. Data for 16,813 cases with isolated male genital malformations and 1,945,841 male live births delivered from 1999 to 2008 were obtained from the Texas Birth Defects Registry. For each phenotype category, 13 maternal and infant variables were assessed, and adjusted prevalence ratios were estimated based on the same multivariable Poisson regression model. A significant negative association was observed between previous live births versus no previous live births and four phenotypes (e.g., adjusted prevalence ratio [aPR] for any male genital malformation: 0.78, 95% confidence interval [CI]: 0.75-0.81). The prevalence of 4 of the phenotypes was significantly higher among multiple versus singleton pregnancies (e.g., aPR for any male genital malformation: 1.35, 95% CI: 1.25-1.47). We also observed significant associations between multiple phenotypes and residential region at delivery, delivery year, month of conception, and maternal age, race/ethnicity, education, and birthplace, including significant associations for trends (maternal age, maternal education, and birth year modeled ordinally). Our results allow for comparison of characteristics across phenotypes and suggest that there may be some common risk factors for multiple male genital malformations (e.g., characteristics related to maternal estrogen levels), while other risk factors may be unique to specific defects. PMID:24458943

Agopian, A J; Langlois, Peter H; Ramakrishnan, Anushuya; Canfield, Mark A

2014-04-01

271

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

272

Stereotactic radiosurgery after embolization for arteriovenous malformations.  

PubMed

We sought to define the long-term benefits and risks of stereotactic radiosurgery (SRS) for arteriovenous malformation (AVM) patients who underwent prior embolization. Between 1987 and 2006, we performed Gamma Knife® SRS on 120 patients with AVM who underwent embolization followed by SRS. Sixty-four patients (53%) had at least 1 prior hemorrhage. The median number of embolizations varied from 1 to 5. The median target volume was 6.6 cm3 (range, 0.2-26.3 cm3). The median margin dose was 18 Gy (range, 13.5-25 Gy). After embolization, 25 patients (21%) developed symptomatic neurological deficits. The overall rates of total obliteration documented by either angiography or magnetic resonance imaging were 35, 53, 55 and 59% at 3, 4, 5 and 10 years, respectively. Nine patients (8%) had a hemorrhage during the latency interval and 7 patients died due to hemorrhage. The actuarial rates of AVM hemorrhage after SRS were 0.8, 3.5, 5.4, 7.7 and 7.7% at 1, 2, 3, 5 and 10 years, respectively. Permanent neurological deficits due to adverse radiation effects developed in 3 patients (2.5%) after SRS. Using a case-match control technique, we found that embolization prior to SRS was associated with a lower rate of total obliteration (p=0.028) in comparison to radiosurgery alone. In this 20-year experience, we found that prior embolization reduced the rate of total obliteration after SRS and latency interval hemorrhage risks were not affected by prior embolization. In the future, the role of embolization after SRS should be explored. PMID:23258513

Kano, Hideyuki; Kondziolka, Douglas; Flickinger, John C; Park, Kyung-Jae; Iyer, Aditya; Yang, Huai-che; Liu, Xiaomin; Monaco, Edward A; Niranjan, Ajay; Lunsford, L Dade

2013-01-01

273

A case report of meningioma extending to the middle ear.  

PubMed

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

2012-05-29

274

Earth Sciences Research at the National Science Foundation (EAR)  

NSF Publications Database

Earth Sciences Research at the National Science Foundation (EAR) Program Solicitation NSF 04-613 ... approaches. Support is available for research and research infrastructure through grants, contracts ...

275

Cochlear fistula in a noncholesteatomatous ear.  

PubMed

Bony destruction of the labyrinth is usually associated with long-standing cholesteatomatous otitis media. The promontory is not a common site for bone resorption because (1) it is not an area that is involved in accumulation of cholesteatoma perimatrix substances, (2) it is the densest bone of the human body, and (3) pressure necrosis from overlying tissue is uncommon. We report a case of cochlear erosion associated with noncholesteatomatous middle ear disease. As far as we know, this is only the second such case reported in the literature. We also review decision-making factors and techniques for the safe management of this condition. PMID:25255363

Hahn, Yoav; Bojrab, Dennis I

2014-09-01

276

Defining anural malformations in the context of a developmental problem  

USGS Publications Warehouse

This paper summarizes terminology and general concepts involved in animal development for the purpose of providing background for the study and understanding of frog malformations. The results of our radiographic investigation of rear limb malformations in Rana pipiens provide evidence that frog malformations are the product of early developmental errors. Although bacteria, parasites and viruses were identified in these metamorphosed frogs, the relevant window to look for the teratogenic effect of these agents is in the early tadpole stage during limb development. As a result, our microbiological findings must be regarded as inconclusive relative to determining their contribution to malformations because we conducted our examinations on metamorphosed frogs not tadpoles. Future studies need to look at teratogenic agents (chemical, microbial, physical or mechanical) that are present in the embryo, tadpole, and their environments at the stages of development that are relevant for the malformation type. The impact of these teratogenic agents then needs to be assessed in appropriate animal models using studies that are designed to mimic field conditions. The results of these laboratory tests should then be analyzed in such a way that will allow comparison with the findings in the wild-caught tadpoles and frogs.

Meteyer, C.U.; Cole, R.A.; Converse, K.A.; Docherty, D.E.; Wolcott, M.; Helgen, J.C.; Levey, R.; Eaton-Poole, L.; Burkhart, J.G.

2000-01-01

277

Transgenerational transmission of radiation damage: genomic instability and congenital malformation.  

PubMed

The congenital malformation gastroschisis has a genetic disposition in the inbred mouse strain HLG/Zte. It is increased after preconceptional irradiation of males or females. Radiation exposures during the meiotic stages are most efficient. This malformation can also be induced by ionising radiation when the exposure takes place during the preimplantation period especially during the zygote stage. This latter effect can be transmitted to the next mouse generation. Other macroscopically visible or skeletal malformations are not significantly induced under these experimental conditions. These latter malformations are increased by radiation exposures during major organogenesis. The mechanisms for the development of the effects are different. Radiation exposure of the mouse zygote (1 to 3 hours p.c.) also leads to the induction of genomic instability in skin fibroblasts of the fetus. This phenomenon also occurs in a mouse strain (C57BL/6J) which is not susceptible to radiation-induced gastroschisis during the preimplantation period. The genomic instability is transmitted to the next mouse generation. During genomic instability chromatide breaks are dominating as in non-exposed cells. With respect to "spontaneous" malformations gastroschisis is dominating in HLG/Zte mice. Late radiation effects seem to have similar patterns as observed in non-exposed subjects, however, the rates are increased after irradiation. PMID:17019048

Streffer, Christian

2006-01-01

278

Population dependent differences of distribution of congenital heart malformations.  

PubMed

The aim of the work was to study the pattern of congenital circulatory system malformations in the newborns in Upper Silesia, Poland (N = 355), and to compare it with the data known from the literature, predominantly with genetically very distant Chinese population from Hong Kong (N = 744). There were significantly more boys (about 60%) than girls (about 40%) in both groups. Much higher rate of the heart defects with pulmonary outflow obstruction was found in Hong Kong neonates (37.8%) than in the Polish ones (16.1%). Left ventricular outflow obstruction malformations were more common in Chinese group (22.7%) than in Polish one (14.1%), as well. On the other hand, Polish neonates were significantly more often admitted to cardiological hospital because of left-to-right shunts (37.7%) as compared to Chinese population (15.5%). The rate of complete transposition of great arteries was similar in both studied groups (12.1% in Poland versus 12.4% in Hong Kong). So called common mixing malformations were not significantly more often found in Polish newborns (12.1%) than in Hong Kong ones (8.3%). It is concluded that it significant race dependent distribution difference of congenital heart malformations exists between Polish (Silesian) and Chinese (Hong Kong) newborn populations. On the other hand the pattern of congenital heart malformations found in Polish newborns seems to be not very different from these ones, known from literature, described in the USA or in Europe. PMID:12901266

Rokicki, W?adys?aw; Leung, Maurice; Kohut, Joanna; Durma?a, Jacek; Borowicka, Ewa

2003-01-01

279

Vein of Galen arteriovenous malformation mimicking coarctation of the aorta.  

PubMed

Arteriovenous malformation of the vein of Galen is a rare congenital intracranial anomaly lacking a capillary bed and subsequent aneurysmal enlargement of the arterial and venous system, warranting careful management due to associated morbidity and mortality. Coarctations of aorta demonstrate similar neonatal echocardiographic signs to the vein of Galen arterial malformation (VGAM). We present a boy at 37 weeks of gestation whose initial ultrasound and echocardiographic investigations showed a dominant right ventricle and isthmal hypoplasia, suggestive of coarctation of aorta. Follow-up ultrasound and echocardiography revealed an arteriovenous malformation involving middle and posterior cerebral artery branches, eliminating coarctation of aorta. VGAM was confirmed by further ultrasound and angiographic investigation, which demonstrated a tangle of cerebral and choroidal arterial branches centrally feeding into an enlarged vein of Galen. The boy's hemodynamic and neurological statuses were confirmed to be stable despite increased venous pressure. Elective embolization at 7 months of age was complicated by a cerebrovascular accident, resulting in right hemiparesis despite no residual cardiac issues. This case demonstrates that rarely, arteriovenous malformations such as the vein of Galen malformations may be the primary cause of patients presenting with coarctation of aorta. The rarity of this condition and its guarded prognosis make our case of special interest to cardiologists and the perinatal care team. PMID:25368688

Firdouse, Mohammed; Agarwal, Arnav; Mondal, Tapas

2014-12-01

280

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

281

Brain Vascular Malformation Consortium: Overview, Progress and Future Directions  

PubMed Central

Brain vascular malformations are resource-intensive to manage effectively, are associated with serious neurological morbidity, lack specific medical therapies, and have no validated biomarkers for disease severity and progression. Investigators have tended to work in “research silos” with suboptimal cross-communication. We present here a paradigm for interdisciplinary collaboration to facilitate rare disease research. The Brain Vascular Malformation Consortium (BVMC) is a multidisciplinary, inter-institutional group of investigators, one of 17 consortia in the Office of Rare Disease Research Rare Disease Clinical Research Network (RDCRN). The diseases under study are: familial Cerebral Cavernous Malformations type 1, common Hispanic mutation (CCM1-CHM); Sturge-Weber Syndrome (SWS); and brain arteriovenous malformation in hereditary hemorrhagic telangiectasia (HHT). Each project is developing biomarkers for disease progression and severity, and has established scalable, relational databases for observational and longitudinal studies that are stored centrally by the RDCRN Data Management and Coordinating Center. Patient Support Organizations (PSOs) are a key RDCRN component in the recruitment and support of participants. The BVMC PSOs include Angioma Alliance, Sturge Weber Foundation, and HHT Foundation International. Our networks of clinical centers of excellence in SWS and HHT, as well as our PSOs, have enhanced BVMC patient recruitment. The BVMC provides unique and valuable resources to the clinical neurovascular community, and recently reported findings are reviewed. Future planned studies will apply successful approaches and insights across the three projects to leverage the combined resources of the BVMC and RDCRN in advancing new biomarkers and treatment strategies for patients with vascular malformations.

Akers, Amy L.; Ball, Karen L.; Clancy, Marianne; Comi, Anne M.; Faughnan, Marie E.; Gopal-Srivastava, Rashmi; Jacobs, Thomas P.; Kim, Helen; Krischer, Jeffrey; Marchuk, Douglas A.; McCulloch, Charles E.; Morrison, Leslie; Moses, Marsha; Moy, Claudia S.; Pawlikowska, Ludmilla; Young, William L.

2013-01-01

282

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

283

[Eustachian tube and middle ear mechanics].  

PubMed

Even the most sophisticated prostheses for reconstruction of the ossicular chain do not work in the presence of Eustachian tube dysfunction. This review gives an update on the mechanisms of middle ear pressure regulation and middle ear ventilation, as well as methods for measuring the opening and closing function of the Eustachian tube. So far, in most tube function tests pressures are applied far beyond the physiological range in order to open the tube or force it to open. New methods like sonotubometry with perfect sequences (PSEQ) or the application of pressure in the nasopharynx with the Estčve technique seem very promising. However, these measurements only provide snapshots of tube function. Presently, new tests are being developed for long-term measurements even in cases with perforated tympanic membranes. Attempts to improve impaired tube function have recently included laser surgery and balloon tuboplasty, yielding positive long-term results requiring verification in larger controlled studies. Eustachian tube dysfunction does not only mean blockage but can also include abnormal patencies of the Eustachian tube, for which new approaches are discussed here. In the case of suspected tube dysfunction, cartilage should be used to avoid early tympanic retraction or recurrent perforation; external ventilation using ventilation tubes should be considered. PMID:21909770

Pau, H W

2011-10-01

284

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

285

Venous malformations: Sclerotherapy with a mixture of ethanol and lipiodol  

SciTech Connect

Purpose. To evaluate the usefulness of a mixture of absolute ethanol and lipiodol in the management of venous malformations. Methods. Percutaneous sclerotherapy was performed with a mixture of absolute ethanol and lipiodol (9:1) in 17 patients with venous malformations, once in 12 patients, twice in 5. The therapeutic efficacy was evaluated by pain reduction. Conventional radiographs (n=15) and posttreatment magnetic resonance imaging (n=5) were obtained for the follow-up evaluation. Results. Sclerotherapy was successful in all but two patients. The therapeutic effect was excellent in two patients, good in seven, fair in five, and poor in one. Radiopacity of lipiodol was beneficial for monitoring the procedure rather than for follow-up evaluations. Areas with low signal-intensity strands were increased on T2-weighted images obtained after the sclerotherapy. Conclusion. Sclerotherapy with a mixture of ethanol and lipiodol is effective in treating venous malformations.

Suh, Jin-Suck [Yonsei University, College of Medicine, Department of Diagnostic Radiology (Korea, Republic of); Shin, Kyoo-Ho [Yonsei University, College of Medicine, Department of Orthopedic Surgery (Korea, Republic of); Na, Jae-Bum [Kyungsang University, College of Medicine, Department of Diagnostic Radiology (Korea, Republic of); Won, Jong-Yun [Yonsei University, College of Medicine, Department of Diagnostic Radiology (Korea, Republic of); Hahn, Soo-Bong [Yonsei University, College of Medicine, Department of Orthopedic Surgery (Korea, Republic of)

1997-07-15

286

Radiation therapy for arteriovenous malformations: a review.  

PubMed

There have been numerous case reports and series of patients treated with partial brain irradiation, linear accelerator-based radiosurgery, gamma knife radiosurgery, and Bragg peak therapy for inoperable arteriovenous malformations (AVMs). These cases are summarized and compared. There is convincing evidence that radiation therapy does have a role in obliterating carefully chosen inoperable lesions. The changes that occur in vessel walls after radiation are reviewed. Data about x-ray and gamma radiation are mostly historical and difficult to evaluate because of the techniques of partial brain irradiation. There is a lack of data about the volume of AVM treated and the minimum dose delivered to the AVM nidus. For gamma knife, heavy particle, and linear accelerator therapy, more complete data are available. The incidence of hemorrhage during the first 2 years after treatment, when radiation-induced vascular changes are proposed to occur, is approximately 2.6% per year for gamma knife therapy, 2% per year for proton beam therapy, 2.3% per year for helium beam therapy, and 2.3% per year for linear accelerator therapy. These rates are similar to the recurrence rate for hemorrhage of 2.2 to 3% per year expected based on the natural history of untreated AVMs. If AVM obliteration after therapy is not achieved, the incidence of recurrent hemorrhage remains between 2% per year after treatment with gamma knife therapy. The incidence of hemorrhage for all patients treated was reported as 0.15% per year in one study and 20% over 8 years in a follow-up study using proton beam therapy. Mortality from hemorrhage after treatment was 0.6% after gamma knife therapy, 2.3% after helium beam therapy, and 2 to 5% after proton beam therapy. These figures for mortality are all lower than the 11% observed for the natural history of untreated AVMs. Permanent neurological deficits experienced as a complication of radiation occurred in 2 to 3% of patients treated with gamma knife therapy, 4% of patients treated with helium beam therapy, 1.7% of patients treated with proton beam therapy, and 3% of patients treated with stereotactic linear accelerator therapy. Proton beam therapy has been used for both small and large lesions. The majority of lesions in patients treated with gamma knife, helium beam, and linear accelerator therapy have been small (usually less than 3.0 cm average diameter) lesions. In these patients with small inoperable lesions treated with accurately directed fields of isocentric radiation, the greatest incidence of AVM obliteration has been observed on follow-up angiograms.(ABSTRACT TRUNCATED AT 400 WORDS) PMID:2191236

Ogilvy, C S

1990-05-01

287

21 CFR 874.3620 - Ear, nose, and throat synthetic polymer material.  

Code of Federal Regulations, 2011 CFR

... Ear, nose, and throat synthetic polymer material. 874.3620 Section 874... Ear, nose, and throat synthetic polymer material. (a) Identification. Ear, nose, and throat synthetic polymer material is a device...

2011-04-01

288

21 CFR 874.3620 - Ear, nose, and throat synthetic polymer material.  

Code of Federal Regulations, 2010 CFR

... Ear, nose, and throat synthetic polymer material. 874.3620 Section 874... Ear, nose, and throat synthetic polymer material. (a) Identification. Ear, nose, and throat synthetic polymer material is a device...

2010-04-01

289

21 CFR 874.4250 - Ear, nose, and throat electric or pneumatic surgical drill.  

Code of Federal Regulations, 2010 CFR

... Ear, nose, and throat electric or pneumatic surgical drill. 874.4250 Section... Ear, nose, and throat electric or pneumatic surgical drill. (a) Identification. An ear, nose, and throat electric or pneumatic surgical drill is a rotating...

2010-04-01

290

21 CFR 874.4250 - Ear, nose, and throat electric or pneumatic surgical drill.  

Code of Federal Regulations, 2013 CFR

... Ear, nose, and throat electric or pneumatic surgical drill. 874.4250 Section... Ear, nose, and throat electric or pneumatic surgical drill. (a) Identification. An ear, nose, and throat electric or pneumatic surgical drill is a rotating...

2013-04-01

291

21 CFR 874.4250 - Ear, nose, and throat electric or pneumatic surgical drill.  

... Ear, nose, and throat electric or pneumatic surgical drill. 874.4250 Section... Ear, nose, and throat electric or pneumatic surgical drill. (a) Identification. An ear, nose, and throat electric or pneumatic surgical drill is a rotating...

2014-04-01

292

21 CFR 874.4250 - Ear, nose, and throat electric or pneumatic surgical drill.  

Code of Federal Regulations, 2012 CFR

... Ear, nose, and throat electric or pneumatic surgical drill. 874.4250 Section... Ear, nose, and throat electric or pneumatic surgical drill. (a) Identification. An ear, nose, and throat electric or pneumatic surgical drill is a rotating...

2012-04-01

293

21 CFR 874.4250 - Ear, nose, and throat electric or pneumatic surgical drill.  

Code of Federal Regulations, 2011 CFR

... Ear, nose, and throat electric or pneumatic surgical drill. 874.4250 Section... Ear, nose, and throat electric or pneumatic surgical drill. (a) Identification. An ear, nose, and throat electric or pneumatic surgical drill is a rotating...

2011-04-01

294

[Direct hearing aid provision by ENT specialists: Recommendations for prevention and treatment of complications after taking ear impressions].  

PubMed

In times of budgeting, rising costs and monetary losses, an increasing number of ENT specialists considers the hearing system sector due to the distinct undersupply and the expected demographic development to be a market of the future and appropriate to enlarge their field of competence. Exact prevalence of direct hearing aid provision by German otorhinolaryngologists as well as its complication rate are not well-known. Retrospectively, all patients were evaluated who presented at our department with complications in the context of outpatient direct hearing aid supply within the last 3 years. Inappropriate fitting contains the danger of malformed or displaced ear impressions with various injury patterns comprising the external auditory canal, tympanic membrane and ossicular chain. Practical advices for prevention of complications include the accurate modeling and placement of sufficient impression pads behind the second bend of the auditory canal, using an adequate type of impression material as well as performing the correct application procedures. Recommendations for diagnostic patterns and treatment of complications after taking ear impressions are presented, the differences between German "traditional" and "direct" hearing aid provision are critically discussed. PMID:19579285

Caffier, Ph P; Sedlmaier, B; Hölzl, M

2009-07-01

295

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 ...

296

Modeling of external ear acoustics for insert headphone usage  

Microsoft Academic Search

Although acoustics of the external ear has been studied extensively for auralization and hearing aids, the acoustic behavior with insert headphones is not as well known. Our research focused on the eects of outer ear physical dimensions, particularly to sound pressure at the eardrum. The main parameter was the length of the canal, but eardrum's damping of resonances was also

Marko Hiipakka; Miikka Tikander; Matti Karjalainen

297

The effects of pressure in the middle ear  

Microsoft Academic Search

The magnitude of cochlear responses in 14 ears (cat) was measured by a wave analyser while pressure in the middle ear was increased over a range of 50 mm. Hg. by means of an air system which included a sphygmomanometer gauge. Equal-response curves as a function of frequency depict decreased sensitivity occasioned by stepwise increase in pressure, the effect being

E. G. Wever; C. W. Bray; M. Lawrence

1942-01-01

298

21 CFR 344.12 - Ear drying aid active ingredient.  

...Drugs 5 2014-04-01 2014-04-01 false Ear drying aid active ingredient. 344.12 Section 344.12 Food and...OVER-THE-COUNTER HUMAN USE Active Ingredients § 344.12 Ear drying aid active ingredient. The active ingredient of the...

2014-04-01

299

Fgf19 expression patterns in the developing chick inner ear  

Microsoft Academic Search

The inner ear is a complex sensorial structure with hearing and balance functions. A key aim of developmental biology is to understand the molecular and cellular mechanisms involved in the induction, patterning and innervation of the vertebrate inner ear. These developmental events could be mediated by the expression of regulating genes, such as the members of the family of Fibroblast

Hortensia Sánchez-Calderón; Javier Francisco-Morcillo; Gervasio Martín-Partido; Matías Hidalgo-Sánchez

2007-01-01

300

CT of adenomas of the middle ear and mastoid cavity  

SciTech Connect

A case of mixed type adenoma of the middle ear and mastoid is presented in which CT showed complete opacification of the middle ear and mastoid air cells with bulging of the tympanic membrane but without ossicular or bony destruction. 7 refs., 1 figs.

Van Thong Ho [McGuire Veterans Affairs Medical Center, Richmond, VA (United States); Rao, V.J.; Mikaelian, D.O. [Thomas Jefferson Univ. Hospital and Jefferson Medical College, Philadelphia, PA (United States)

1996-03-01

301

Principles of Local Drug Delivery to the Inner Ear  

Microsoft Academic Search

As more and more substances have been shown in preclinical studies to be capable of preventing damage to the inner ear from exposure to noise, ototoxic drugs, ischemia, infection, inflammation, mechanical trauma and other insults, it is becoming very important to develop feasible and safe methods for the targeted delivery of drugs to specific regions in the inner ear. Recently

Alec N. Salt; Stefan K. Plontke

2009-01-01

302

Anthroposophic vs. conventional therapy of acute respiratory and ear infections  

Microsoft Academic Search

Summary CONTEXT: Acute respiratory and ear symptoms are frequently treated with antibiotics. Anthroposophic treatment of these symptoms relies primarily on anthroposophic medications. OBJECTIVE: To compare anthroposophic treatment to conventional treatment of acute respiratory and ear symptoms regarding clinical outcome, medication use and safety, and patient satisfaction. DESIGN: Prospective, non-randomised comparison of outcomes in patients self-selected to anthroposophic or conventional therapy

Harald J. Hamre; Michael Fischer; Marianne Heger; David Riley; Max Haidvogl; Erik Baars; Eileen Bristol; Michael Evans; Reinhard Schwarz; Helmut Kiene

2005-01-01

303

Medicament contact dermatitis in patients with chronic inflammatory ear disease.  

PubMed

Patch testing of 40 patients with chronic inflammatory ear disease demonstrated medicament allergic contact dermatitis in 35%. The most frequent sensitizers were neomycin, framycetin, clioquinol and gentamicin. Although allergic contact dermatitis to dewaxing ear drops was unusual, irritant reactions were common. PMID:6460100

Holmes, R C; Johns, A N; Wilkinson, J D; Black, M M; Rycroft, R J

1982-01-01

304

Purdue extensionGibberella Ear Rot Purdue extension  

E-print Network

for a pink to reddish mold that begins at the tip of the ear and develops toward the base (Figures 1 and 2 can affect the health of many monogastric animals, but swine are especially sensitive. If Gib ear rot is present, assume that the mycotoxins are also present. A test is needed to deter- mine the level

Holland, Jeffrey

305

Maternal diabetes and congenital malformations among live births in Hawaii  

Microsoft Academic Search

Summary  The frequency of congenital malformations, in 187,266 live births in Hawaii from 1956 through 1966, was found to be significantly\\u000a higher among offspring of diabetic mothers (0.0175) than in offspring of non-diabetic mothers (0.0086) or mothers with no\\u000a complications of pregnancy (0.0074). However, the frequency of malformations in offspring of diabetic mothers was not significantly\\u000a higher than in offspring of

Madeleine J. Goodman

1976-01-01

306

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

307

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

308

Imaging features of ductal plate malformations in adults.  

PubMed

Ductal plate malformations, also known as fibrocystic liver diseases, are a group of congenital disorders resulting from abnormal embryogenesis of the biliary ductal system. The abnormalities include choledochal cyst, Caroli's disease and Caroli's syndrome, adult autosomal dominant polycystic liver disease, and biliary hamartoma. The hepatic lesions can be associated with renal anomalies such as autosomal recessive polycystic kidney disease (ARPKD), medullary sponge kidney, and nephronophthisis. A clear knowledge of the embryology and pathogenesis of the ductal plate is central to the understanding of the characteristic imaging appearances of these complex disorders. Accurate diagnosis of ductal plate malformations is important to direct appropriate clinical management and prevent misdiagnosis. PMID:21840516

Venkatanarasimha, N; Thomas, R; Armstrong, E M; Shirley, J F; Fox, B M; Jackson, S A

2011-11-01

309

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

310

Split cervical spinal cord malformation and vertebral dysgenesis.  

PubMed

We report a case of vertebral malformation associated with diplomyelia believed to be a type II split cord malformation. Cervicothoracic level cases are exceptional. This article reports the case of an 11-year-old boy with no neurological symptoms who had not undergone surgery. The diagnosis was made during pregnancy by prenatal screening with ultrasound and MRI. Several embryological theories have been offered to provide an explanation for this syndrome. Close follow-up is mandatory. Surgery must only be considered if neurological deterioration occurs. PMID:19837020

Andro, C; Pecquery, R; De Vries, P; Forlodou, P; Fenoll, B

2009-11-01

311

[Homologous transplants in the middle ear (author's transl)].  

PubMed

The original concept of tympanoplasty included besides the closure of the middle ear, the removal of pathological middle ear components leaving intact the functional and healthy components. The special conditions that prevail in the middle ear (aeration, absence of intrinsic trauma) allow the reconstruction of the middle ear mechanics to a great extent. Animal experiments and clinical experience reveal good transplantation properties of denatured and preserved ear ossicles. They are partly replaced by the body's own true tissue, and preserve the original form over a period of years. Denatured dear drums with malleus, incus, and parts of the stapes give astounding acoustic results. The posterior bony canal wall in old radical cavities can be replaced by the posterior wall of the human temporal bone which has been preserved in Cialit. PMID:123985

Plester, D

1975-03-01

312

Audiometric Predictions Using SFOAE and Middle-Ear Measurements  

PubMed Central

Objective The goals of the study are to determine how well stimulus-frequency otoacoustic emissions (SFOAEs) identify hearing loss, classify hearing loss as mild 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 function (ATF) measurements in the ear canal account for the variability in normal thresholds, and if the inclusion of ATFs improves the ability of SFOAEs to identify hearing loss and predict pure-tone thresholds. Design The total suppressed SFOAE signal and its corresponding noise were recorded in 85 ears (22 normal ears and 63 ears with sensorineural hearing loss) at octave frequencies from 0.5 – 8 kHz using a nonlinear residual method. SFOAEs were recorded a second time in three impaired ears to assess repeatability. Ambient-pressure ATFs were obtained in all but one of these 85 ears, and were also obtained from an additional 31 normal-hearing subjects in whom SFOAE data were not obtained. Pure-tone air-and bone-conduction thresholds and 226-Hz tympanograms were obtained on all subjects. Normal tympanometry and the absence of air-bone gaps were used to screen subjects for normal middle-ear function. Clinical decision theory was used to assess the performance of SFOAE and ATF predictors in classifying ears as normal or impaired, and linear regression analysis was used to test the ability of SFOAE and ATF variables to predict the air-conduction audiogram. Results The ability of SFOAEs to classify ears as normal or hearing impaired was significant at all test frequencies. The ability of SFOAEs to classify impaired ears as either mild or moderate-severe was significant at test frequencies from 0.5 to 4 kHz. SFOAEs were present in cases of severe hearing loss. SFOAEs were also significantly correlated with air-conduction thresholds from 0.5 to 8 kHz. The best performance occurred using the SFOAE signal-to-noise ratio (S/N) as the predictor, and the overall best performance was at 2 kHz. The SFOAE S/N measures were repeatable to within 3.5 dB in impaired ears. The ATF measures explained up to 25% of the variance in the normal audiogram; however, ATF measures did not improve SFOAEs predictors of hearing loss except at 4 kHz. Conclusions In common with other OAE types, SFOAEs are capable of identifying the presence of hearing loss. In particular, SFOAEs performed better than distortion-product and click-evoked OAEs in predicting auditory status at 0.5 kHz; SFOAE performance was similar to that of other OAE types at higher frequencies except for a slight performance reduction at 4 kHz. Because SFOAEs were detected in ears with mild to severe cases of hearing loss they may also provide an estimate of the classification of hearing loss. Although SFOAEs were significantly correlated with hearing threshold, they do not appear to have clinical utility in predicting a specific behavioral threshold. Information on middle-ear status as assessed by ATF measures offered minimal improvement in SFOAE predictions of auditory status in a population of normal and impaired ears with normal middle-ear function. However, ATF variables did explain a significant fraction of the variability in the audiograms of normal ears, suggesting that audiometric thresholds in normal ears are partially constrained by middle-ear function as assessed by ATF tests. PMID:16230898

Ellison, John C.; Keefe, Douglas H.

2006-01-01

313

Segmentation algorithms for ear image data towards biomechanical studies.  

PubMed

In recent years, the segmentation, i.e. the identification, of ear structures in video-otoscopy, computerised tomography (CT) and magnetic resonance (MR) image data, has gained significant importance in the medical imaging area, particularly those in CT and MR imaging. Segmentation is the fundamental step of any automated technique for supporting the medical diagnosis and, in particular, in biomechanics studies, for building realistic geometric models of ear structures. In this paper, a review of the algorithms used in ear segmentation is presented. The review includes an introduction to the usually biomechanical modelling approaches and also to the common imaging modalities. Afterwards, several segmentation algorithms for ear image data are described, and their specificities and difficulties as well as their advantages and disadvantages are identified and analysed using experimental examples. Finally, the conclusions are presented as well as a discussion about possible trends for future research concerning the ear segmentation. PMID:22994296

Ferreira, Ana; Gentil, Fernanda; Tavares, Joăo Manuel R S

2014-01-01

314

Inner ear symptoms and disease: Pathophysiological understanding and therapeutic options  

PubMed Central

In recent years, huge advances have taken place in understanding of inner ear pathophysiology causing sensorineural hearing loss, tinnitus, and vertigo. Advances in understanding comprise biochemical and physiological research of stimulus perception and conduction, inner ear homeostasis, and hereditary diseases with underlying genetics. This review describes and tabulates the various causes of inner ear disease and defines inner ear and non-inner ear causes of hearing loss, tinnitus, and vertigo. The aim of this review was to comprehensively breakdown this field of otorhinolaryngology for specialists and non-specialists and to discuss current therapeutic options in distinct diseases and promising research for future therapies, especially pharmaceutic, genetic, or stem cell therapy. PMID:24362017

Ciuman, Raphael R.

2013-01-01

315

Self-Ear-Cleaning Among Educated Young Adults in Nigeria  

PubMed Central

Context: Self-ear-cleaning has been reported to be common from several hospital-based studies and it has been associated with some diseases of the ear. Aims: To determine community-based prevalence of self-ear-cleaning and its sociodemographic correlates among educated young adults in Nigeria. Settings and Design: A cross-sectional survey conducted in a National Youth Service Corps camp in Nigeria. Subjects and Methods: Semistructured questionnaires were administered on a randomly selected sample of 1280 respondents. The outcome variable was self-ear-cleaning. Independent variables were sociodemographic variables, materials used and ear-cleaning habits. Statistical Analysis Used: Statistical Package for the Social Sciences (SPSS) version 15 was utilized for univariate, bivariate, and multiple logistic regression analysis. Results: There were 1012 respondents (M: F = 1.05:1). Mean age was 25.3 (standard deviation, 2.34). Prevalence of self-ear-cleaning was 93.4%. Mean age at first cleaning was 7.6 years. Cotton buds were the most frequently used objects (in 85.1%). Prevalence was high irrespective of sociodemographic class, significantly higher among females (?2 = 4.549, P = 0.033), those who believed the habit was beneficial (?2 = 114.185, P < 0.001) and those whose parents and siblings practiced the habit. Significant predictive factors were self-ear-cleaning in respondent's father [odds ratio (OR) P = 0.011) and owning cotton buds (OR = 0.192, P = 0.007). Conclusions: Self-ear-cleaning is almost universal. Most of the population is, therefore, at risk of possible harmful effects. Also, medical advice against self-ear-cleaning is not widely known. Rather, the erroneous perception that self-ear-cleaning is beneficial is common. Collaborative health education efforts targeted at families and schools and campaigns and advocacy for legislation regulating the sale of cotton buds are recommended. PMID:24791230

Olaosun, Adedayo Olugbenga

2014-01-01

316

Ear today gone tomorrow’: routine neonatal ear canal cultures are not useful predictors of early onset invasive bacterial sepsis  

Microsoft Academic Search

BackgroundCulture of body surfaces including the ear canal, throat and rectum are often routinely performed on new neonatal unit admissions. It has been postulated that in the early postnatal period the neonatal ear canal may still contain amniotic fluid and thus be superior to other sites for identifying maternally acquired bacteria responsible for early onset neonatal bacterial sepsis. However, the

A J Battersby; R Webster; T Neal; N Subhedar

2011-01-01

317

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

318

Binaural Hearing Relies on the differences in information reaching ears Relies on the differences in information reaching ears  

E-print Network

Binaural Hearing · Relies on the differences in information reaching ears· Relies environments · Fusion: "Two ears but one world" #12;The Major Binaural Cues Interaural Temporal Disparities different nature of input to the binaural processor Frequency Ampl Frequency Ampl Binaural ProcessorBinaural

Oliver, Douglas L.

319

A Registry-Based Assessment of Cerebral Palsy and Cerebral Malformations  

Microsoft Academic Search

Cerebral malformations are 1 of the many possible causes of cerebral palsy. In this study, a population-based comprehensive cerebral palsy registry was used to identify children whose cerebral palsy could be attributed to a cerebral malformation. The clinical features of these children were then compared with other children with cerebral palsy. Children with cerebral palsy and cerebral malformation did not

Lauren Self; Michael I. Shevell

2010-01-01

320

Notch-1 Signalling Is Activated in Brain Arteriovenous Malformations in Humans  

ERIC Educational Resources Information Center

A role for the Notch signalling pathway in the formation of arteriovenous malformations during development has been suggested. However, whether Notch signalling is involved in brain arteriovenous malformations in humans remains unclear. Here, we performed immunohistochemistry on surgically resected brain arteriovenous malformations and found that,…

ZhuGe, Qichuan; Zhong, Ming; Zheng, WeiMing; Yang, Guo-Yuan; Mao, XiaoOu; Xie, Lin; Chen, Gourong; Chen, Yongmei; Lawton, Michael T.; Young, William L.; Greenberg, David A.; Jin, Kunlin

2009-01-01

321

Squamous carcinoma of the external ear.  

PubMed

The medical records of 486 patients with pathologically proved squamous carcinoma of the skin of the external ear were analyzed. It is a disease of elderly white men, and the helix is the most common site of origin. Well-differentiated squamous carcinoma is the most frequent histologic variant. Ninety-five percent of our patients were treated surgically with above-clavical control in 87 percent and 28 percent survival. The low survival rate was related to the old age of the patients who frequently died of intercurrent disease and second cancers. A 12 percent incidence of nodal metastases is comparable with the incidence reported in other series. Aggressive surgical ablation and the selected use of adjunctive postoperative irradiation appear justified in those patients with locally invasive tumors, multiple nodal metastases, and extracapsular invasion. PMID:6625089

Byers, R; Kesler, K; Redmon, B; Medina, J; Schwarz, B

1983-10-01

322

Chiari Type I Malformation: Overview of Diagnosis and Treatment  

Microsoft Academic Search

Chiari Type I malformation (Chiari I) is a congenital hindbrain anomaly characterized by downward dis- placement of the cerebellar tonsils through the fora- men magnum. This can lead to compression of cere- bellar components, the lower brainstem, and the upper cervical spinal cord. In turn, a variety of neu- rological deficits and permanent nervous system damage may evolve. This review

John Nash; Joseph S. Cheng; Glenn A. Meyer; Bernd F. Remler

323

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

324

Pulmonary arteriovenous malformation in the newborn: A familial case  

Microsoft Academic Search

Pulmonary arteriovenous malformation (PAVM) is a rare cause of cyanosis in the newborn with nine previously reported cases. Typical signs at presentation include cyanosis, murmur, and congestive heart failure. Abnormality on chest x-ray is common with cardiomegaly, oligemia, and focal pulmonary density. There is a male predominance, unlike older children and adults with PAVM. Familial cases of PAVM have been

Steven W. Allen; Jonathan M. Whitfield; David R. Clarke; Eva Sujansky; James W. Wiggins

1993-01-01

325

Spontaneous Regression of Cerebral Arteriovenous Malformation in Hereditary Hemorrhagic Telangiectasia  

Microsoft Academic Search

Summary: Cerebral arteriovenous malformations (AVMs) are associated with hereditary hemorrhagic telangiectasia (HHT). I report a case of a patient with HHT with four cerebral AVMs in whom one AVM spontaneously regressed during a 5-year period. The spontaneous regression of this AVM in this patient with HHT supports the theory that AVMs associated with this syndrome have a different nat- ural

Harry J. Cloft

326

Fusarium species associated with waterberry (Syzygium cordatum) floral malformation  

E-print Network

or in swampy spots. This evergreen tree has white, fluffy flowers that bloom from August to November. Its fruit is an edible berry that turns dark-purple when ripe. In rural areas the tree is used as a remedy large numbers of flowers. With time these malformed inflorescences dry out without ever bearing fruit

327

Diencephalic-Mesencephalic Junction Dysplasia: A Novel Recessive Brain Malformation  

ERIC Educational Resources Information Center

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…

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

2012-01-01

328

Failed transarterial embolization of subserosal uterine arteriovenous malformation.  

PubMed

Uterine arteriovenous malformation (AVM) is rare but potentially life-threatening from excessive vaginal bleeding. All uterine AVMs reported to date have been found in the endometrial or myometrial layers. Here we present a patient with a subserosal type AVM on the fundus of uterus, which spontaneously ruptured. PMID:24328024

Seo, Kyung Jin; Kim, Jin; Sohn, In Sook; Kwon, Han Sung; Park, Sang Woo; Hwang, Han Sung

2013-09-01

329

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

330

Management of the fetus with a cystic adenomatoid malformation  

Microsoft Academic Search

Prenatal detection and serial sonographic study of fetuses with congenital cystic adenomatoid malformation (CCAM) now makes it possible to define the natural history of this lesion, determine the pathophysiologic features that affect clinical outcome, and formulate management based on prognosis. In a series of 22 prenatally diagnosed cases, the overall prognosis depended on the effects of the lung mass and

N. Scott Adzick; Michael R. Harrison

1993-01-01

331

Valproic Acid and Congenital MalformationsA Case Report  

Microsoft Academic Search

An infant exposed in utero to valproic acid was born with multiple congenital malformations, including duodenal atresia, lumbar vertebral fusion, thoracolumbar scoliosis, renal abnormalities, diastasis recti abdominis, depigmentation of the eye brows, and a large hemangioma. The child had neonatal hyperbilirubinemia and postnatal growth deficiency. This case report is an addition to the literature im plicating valproic acid as a

Maj Eric W. Bantz

1984-01-01

332

Valproic acid and congenital malformations. A case report.  

PubMed

An infant exposed in utero to valproic acid was born with multiple congenital malformations, including duodenal atresia, lumbar vertebral fusion, thoracolumbar scoliosis, renal abnormalities, diastasis recti abdominis, depigmentation of the eyebrows, and a large hemangioma. The child had neonatal hyperbilirubinemia and postnatal growth deficiency. This case report is an addition to the literature implicating valproic acid as a human teratogen. PMID:6426840

Bantz, E W

1984-06-01

333

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

334

Coiling of a vulvar arterio-venous malformation  

PubMed Central

The authors report the case of a 13-year-old girl with a painful vulvar swelling and abnormal vaginal bleeding, increasing in size after trauma. With MRI (GE Signa HDx 1.5 Tesla), it is diagnosed as an arterio-venous malformation arising from the left superior femoral artery. It is treated by embolisation using a coil. PMID:22674935

Van der Woude, Daisy Adriana Annejan; Stegeman, Marjan; Seelen, Jan L

2011-01-01

335

Maternal smoking and congenital malformations: an epidemiological study  

Microsoft Academic Search

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

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

1978-01-01

336

Development and malformations of the human pyramidal tract  

Microsoft Academic Search

The corticospinal tract develops over a rather long period of time, during which malformations involving this main central motor pathway may occur. In rodents, the spinal outgrowth of the corticospinal tract occurs entirely postnatally, but in primates largely prenatally. In mice, an increasing number of genes have been found to play a role during the development of the pyramidal tract.

H. J. ten Donkelaar; M. M. Y. Lammens; P. Wesseling; A. Hori; A. J. M. Keyser; J. J. Rotteveel

2004-01-01

337

Spitz nevus arising upon a congenital glomuvenous malformation.  

PubMed

There are several reports of the collision of vascular and pigmentary anomalies (e.g., phakomatosis pigmentovascularis) and the association between congenital melanocytic nevi and infantile hemangiomas. We report a case of Spitz nevus arising in skin overlying a congenital plaque-like glomuvenous malformation (GVM). This is the first report of a Spitz nevus arising in direct contiguity to a GVM. PMID:22304367

Arica, Deniz A; Arica, Ibrahim E; Yayli, Savas; Cobanoglu, Umit; Akay, Bengu N; Anadolu, Rana; Bahadir, Sevgi

2013-01-01

338

A syndrome including thumb malformations, microcephaly, short stature, and hypogonadism  

Microsoft Academic Search

We report on eight patients from seven different families affected with a syndrome which includes thumb defects, short stature, microcephaly, and mental retardation. Most of the patients had additional malformations, in particular amenorrhoea and azoospermia in the adults. There were no haematological manifestations and the chromosomes were normal without evidence of breakage even after stimulation. In five of the cases

J Zlotogora; J Dagan; A Ganen; M Abu-Libdeh; Z Ben-Neriah; T Cohen

1997-01-01

339

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...

340

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

341

The value of politzerization in the treatment of atelectatic ears.  

PubMed

The value of Politzerization as a method of treatment in atelectatic ears and secretory otitis media is controversial. In some places it has been used routinely for decades, in others it has been almost forgotten. A quantitative study of its therapeutic value has been difficult to find. The present study is an attempt to evaluate the therapeutic value of such an 'air douche' in atelectatic ears. Thirteen middle ears with atelectasis were Politzerized daily with air or N2 for up to five consecutive days. Once Politzerized, the atelectasis and retraction pockets disappeared in all the ears examined. However, continuous observation of the Politzerized ears with the surgical microscope revealed that all the ear drums returned swiftly to their retracted position. The time it took for a drum to return to its original place varied from 15 minutes up to a maximum of 335 minutes. Our observations show that even an increased number of Politzerizations did not alter the speed of reappearance of the atelectasis. It would therefore seem that the therapeutic value of Politzerization in atelectatic ears is doubtful. PMID:3171367

Luntz, M; Sadé, J

1988-09-01

342

Inverse solution of ear-canal area function from reflectance  

PubMed Central

A number of acoustical applications require the transformation of acoustical quantities, such as impedance and pressure that are measured at the entrance of the ear canal, to quantities at the eardrum. This transformation often requires knowledge of the shape of the ear canal. Previous attempts to measure ear-canal area functions were either invasive, non-reproducible, or could only measure the area function up to a point mid-way along the canal. A method to determine the area function of the ear canal from measurements of acoustic impedance at the entrance of the ear canal is described. The method is based on a solution to the inverse problem in which measurements of impedance are used to calculate reflectance, which is then used to determine the area function of the canal. The mean ear-canal area function determined using this method is similar to mean ear-canal area functions measured by other researchers using different techniques. The advantage of the proposed method over previous methods is that it is non- invasive, fast, and reproducible. PMID:22225043

Rasetshwane, Daniel M.; Neely, Stephen T.

2011-01-01

343

Combined Effect of Fluid and Pressure on Middle Ear Function  

PubMed Central

In our previous studies, the effects of effusion and pressure on sound transmission were investigated separately. The aim of this study is to investigate the combined effect of fluid and pressure on middle ear function. An otitis media with effusion model was created by injecting saline solution and air pressure simultaneously into the middle ear of human temporal bones. Tympanic membrane displacement in response to 90 dB SPL sound input was measured by a laser vibrometer and the compliance of the middle ear was measured by a tympanometer. The movement of the tympanic membrane at the umbo was reduced up to 17 dB by the combination of fluid and pressure in the middle ear over the auditory frequency range. The fluid and pressure effects on the umbo movement in the fluid-pressure combination are not additive. The combined effect of fluid and pressure on the umbo movement is different compared with that of only fluid or pressure change in the middle ear. Negative pressure in fluid-pressure combination had more effect on middle ear function than positive pressure. Tympanometry can detect the middle ear pressure of the fluid-pressure combination. This study provides quantitative information for analysis of the combined effect of fluid and pressure on tympanic membrane movement. PMID:18162348

Dai, Chenkai; Wood, Mark W.; Gan, Rong Z.

2008-01-01

344

Combined effect of fluid and pressure on middle ear function.  

PubMed

In our previous studies, the effects of effusion and pressure on sound transmission were investigated separately. The aim of this study is to investigate the combined effect of fluid and pressure on middle ear function. An otitis media with effusion model was created by injecting saline solution and air pressure simultaneously into the middle ear of human temporal bones. Tympanic membrane displacement in response to 90 dB SPL sound input was measured by a laser vibrometer and the compliance of the middle ear was measured by a tympanometer. The movement of the tympanic membrane at the umbo was reduced up to 17 dB by the combination of fluid and pressure in the middle ear over the auditory frequency range. The fluid and pressure effects on the umbo movement in the fluid-pressure combination are not additive. The combined effect of fluid and pressure on the umbo movement is different compared with that of only fluid or pressure change in the middle ear. Negative pressure in fluid-pressure combination had more effect on middle ear function than positive pressure. Tympanometry can detect the middle ear pressure of the fluid-pressure combination. This study provides quantitative information for analysis of the combined effect of fluid and pressure on tympanic membrane movement. PMID:18162348

Dai, Chenkai; Wood, Mark W; Gan, Rong Z

2008-02-01

345

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

346

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

PubMed Central

The development and evolution of mechanosensory cells and the vertebrate ear is reviewed with an emphasis on delineating the cellular, molecular and developmental basis of these changes. Outgroup comparisons suggests that mechanosensory cells are ancient features of multicellular organisms. Molecular evidence suggests that key genes involved in mechanosensory cell function and development are also conserved among metazoans. The divergent morphology of mechanosensory cells across phyla is interpreted here as ‘deep molecular homology’ that was in parallel shaped into different forms in each lineage. The vertebrate mechanosensory hair cell and its associated neuron are interpreted as uniquely derived features of vertebrates. It is proposed that the vertebrate otic placode presents a unique embryonic adaptation in which the diffusely distributed ancestral mechanosensory cells became concentrated to generate a large neurosensory precursor population. Morphogenesis of the inner ear is reviewed and shown to depend on genes expressed in and around the hindbrain that interact with the otic placode to define boundaries and polarities. These patterning genes affect downstream genes needed to maintain proliferation and to execute ear morphogenesis. We propose that fibroblast growth factors (FGFs) and their receptors (FGFRs) are a crucial central node to translate patterning into the complex morphology of the vertebrate ear. Unfortunately, the FGF and FGFR genes have not been fully analyzed in the many mutants with morphogenetic ear defects described thus far. Likewise, little information exists on the ear histogenesis and neurogenesis in many mutants. Nevertheless, a molecular mechanism is now emerging for the formation of the horizontal canal, an evolutionary novelty of the gnathostome ear. The existing general module mediating vertical canal growth and morphogenesis was modified by two sets of new genes: one set responsible for horizontal canal morphogenesis and another set for neurosensory formation of the horizontal crista and associated sensory neurons. The dramatic progress in deciphering the molecular basis of ear morphogenesis offers grounds for optimism for translational research toward intervention in human morphogenetic defects of the ear. PMID:16643865

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

2014-01-01

347

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

348

3D Ear Identification Based on Sparse Representation  

PubMed Central

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

349

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

350

Shaping sound in space: the regulation of inner ear patterning  

PubMed Central

The inner ear is one of the most morphologically elaborate tissues in vertebrates, containing a group of mechanosensitive sensory organs that mediate hearing and balance. These organs are arranged precisely in space and contain intricately patterned sensory epithelia. Here, we review recent studies of inner ear development and patterning which reveal that multiple stages of ear development – ranging from its early induction from the embryonic ectoderm to the establishment of the three cardinal axes and the fine-grained arrangement of sensory cells – are orchestrated by gradients of signaling molecules. PMID:22186725

Groves, Andrew K.; Fekete, Donna M.

2012-01-01

351

Tympanosclerosis of the ear drum in secretory otitis media.  

PubMed

Tympanosclerosis of the ear drum in children with SOM differs from normal ear drums in children with SOM. In tympanosclerosis the numbers of fibroblasts, fibrocytes and macrophages are increased in lamina propria and in the submucosa. The collagen microfibrils and procollagen filaments have increased in numbers with a partly disorganized pattern. Fibrolysis of microfibrils is conspicuous and hyaline degeneration appears in areas. Calcareous deposits are found scattered to a limited extent in lamina propria and in the submucosa. Profound calcification could only be found in children with tympanosclerosis coexisting with chronic perforation of the ear drum. PMID:6598264

Mřller, P

1984-01-01

352

[Functional examinations of the ear and auditory pathway].  

PubMed

Hearing is usually examined by means of pure tone and speech audiometry. Alternative examinations are required when the level of hearing defect needs to be more closely defined or hearing is being measured from a patient who is unable to give reliable feedback on a test sound. Neonatal hearing screening involves measuring of otoacoustic emissions generated in the cochlea of the inner ear or brain stem responses formed within the auditory pathway. Immittance measurements are used to evaluate the function of the middle ear. Most commonly used methods include tympanometry probing the mobility of the tympanic membrane and middle ear pressure, and acoustic reflex assessing the functionality of the ossicular chain. PMID:21568109

Laitakari, Jaakko; Kokkonen, Jukka

2011-01-01

353

Identification of synergistic signals initiating inner ear development.  

PubMed

Tissue manipulation experiments in amphibians more than 50 years ago showed that induction of the inner ear requires two signals: a mesodermal signal followed by a neural signal. However, the molecules mediating this process have remained elusive. We present evidence for mesodermal initiation of otic development in higher vertebrates and show that the mesoderm can direct terminal differentiation of the inner ear in rostral ectoderm. Furthermore, we demonstrate the synergistic interactions of the extracellular polypeptide ligands FGF-19 and Wnt-8c as mediators of mesodermal and neural signals, respectively, initiating inner ear development. PMID:11110663

Ladher, R K; Anakwe, K U; Gurney, A L; Schoenwolf, G C; Francis-West, P H

2000-12-01

354

Subgridding method for FDTD modeling in the inner ear  

NASA Astrophysics Data System (ADS)

A detailed dosimetry in the inner ear is performed using the FDTD algorithm with a subgridding method. A head model is exposed to a mobile phone radiation and the electromagnetic field in the region of the inner ear is computed with increased numerical resolution by factor of 3, 5 and 7. Results show that increase of the numerical resolution without increase of the geometrical resolution does not give more detailed SAR distribution in the inner ear. Therefore a new model of the cochlea with increasing the geometrical resolution from 1 mm to 1/7 mm and dosimetry in this model is presented.

Kopecky, Rudolf; Persson, Mikael

2004-04-01

355

Ear-canal standing waves and high-frequency sound calibration using otoacoustic emission probes  

E-print Network

-pressurelevels.The commonlyacceptedreferencefor the input to the middle ear is the sound-pressurelevel (re: 20 pPa) "at the ear- drum." Thisis(the ear- drum: Assumedto terminate the tube at a right angle) (Wiener and Ross, 1946; Gilman etal., 1981Ear-canal standing waves and high-frequency sound calibration using otoacoustic emission probes J

Allen, Jont

356

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

357

Ear-canal standing waves and high-frequency soundcalibration using otoacousticemissionprobes  

E-print Network

-pressurelevels.The commonlyacceptedreferencefor the input to the middle ear is the sound-pressurelevel (re: 20/zPa) "at the ear- drum canal:Analogousto a uni- formtube) terminatedby a compleximpedance(the ear- drum: Assumedto terminatetheEar-canal standing waves and high-frequency soundcalibration using otoacousticemissionprobes J. H

Allen, Jont

358

Non-acoustic Factors influencing Activity of Middle Ear Muscles in Waking Cats  

Microsoft Academic Search

THE intensity of sound transmitted from the ear drum to the inner ear can be attenuated by contraction of the middle ear muscles, the tensor tympani and stapedius. These muscles contract at the onset of sound, thereby reducing the size of cochlear microphonics as recorded at the round window. Previous explanations of middle ear muscle activity have only considered possible

Peter W. Carmel; Arnold Starr

1964-01-01

359

Laser interferometric vibration measurements of the middle ear in healthy humans  

Microsoft Academic Search

The use of spontaneous and evoked otacoustic emissions is now a standard clinical tool for diagnosis of the function of the inner ear. However, it is not possible to extract this information over the entire, functionally relevant frequency range because of imperfect coupling of: (1) stapedial to ear-drum vibrations through the ossicular chain of the middle ear and (2) ear-drum

J. Rodriguez Jorge; Werner Hemmert; C. Burkhardt; Hans-Peter Zenner; Anthony W. Gummer

1996-01-01

360

The contribution of ear photosynthesis to grain filling in bread wheat ( Triticum aestivum L.)  

Microsoft Academic Search

The contribution of ear photosynthesis to grain filling in wheat (Triticum aestivum L.) is not well known. The main objective of this work was to evaluate this contribution through three different experimental approaches: (1) ear photosynthesis was reduced by removing awns or shading the ears (in combination with a defoliation treatment), (2) grain weight per ear was compared in an

M. L. Maydup; M. Antonietta; J. J. Guiamet; C. Graciano; J. R. López; E. A. Tambussi

2010-01-01

361

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

362

Epizootic of ovine congenital malformations associated with Schmallenberg virus infection.  

PubMed

Epizootic outbreaks of congenital malformations in sheep are rare and have, to the best of our knowledge, never been reported before in Europe. This paper describes relevant preliminary findings from the first epizootic outbreak of ovine congenital malformations in the Netherlands. Between 25 November and 20 December 2011, congenital malformations in newborn lambs on sheep farms throughout the country were reported to the Animal Health Service in Deventer. Subsequently, small ruminant veterinary specialists visited these farms and collected relevant information from farmers by means of questionnaires. The deformities varied from mild to severe, and ewes were reported to have given birth to both normal and deformed lambs; both male and female lambs were affected. Most of the affected lambs were delivered at term. Besides malformed and normal lambs, dummy lambs, unable to suckle, were born also on these farms. None of the ewes had shown clinical signs during gestation or at parturition. Dystocia was common, because of the lambs' deformities. Lambs were submitted for post-mortem examination, and samples of brain tissue were collected for virus detection. The main macroscopic findings included arthrogryposis, torticollis, scoliosis and kyphosis, brachygnathia inferior, and mild-to-marked hypoplasia of the cerebrum, cerebellum and spinal cord. Preliminary data from the first ten affected farms suggest that nutritional deficiencies, intoxication, and genetic factors are not likely to have caused the malformations. Preliminary diagnostic analyses of precolostral serum samples excluded border disease virus, bovine viral diarrhoea virus, and bluetongue virus. In December 2011, samples of brain tissue from 54 lambs were sent to the Central Veterinary Institute of Wageningen University Research, Lelystad. Real-time PCR detected the presence of a virus, provisionally named the Schmallenberg virus, in brain tissue from 22 of the 54 lambs, which originated from seven of eight farms that had submitted lambs for post-mortem examination. This Schmallenberg virus was first reported in Germany and seems to be related to the Shamonda, Aino, and Akabane viruses, all of which belong to the Simbu serogroup of the genus Orthobunyavirus of the family Bunyaviridae. These preliminary findings suggest that the Schmallenberg virus is the most likely cause of this epizootic of ovine congenital malformations, which is the first such outbreak reported in Europe. PMID:22393844

van den Brom, R; Luttikholt, S J M; Lievaart-Peterson, K; Peperkamp, N H M T; Mars, M H; van der Poel, W H M; Vellema, P

2012-02-01

363

14 CFR 67.205 - Ear, nose, throat, and equilibrium.  

Code of Federal Regulations, 2012 CFR

...CONTINUED) AIRMEN MEDICAL STANDARDS AND CERTIFICATION Second-Class Airman Medical Certificate § 67.205...a second-class airman medical certificate are: ...in one ear or in a sound field environment....

2012-01-01

364

14 CFR 67.205 - Ear, nose, throat, and equilibrium.  

Code of Federal Regulations, 2013 CFR

...CONTINUED) AIRMEN MEDICAL STANDARDS AND CERTIFICATION Second-Class Airman Medical Certificate § 67.205...a second-class airman medical certificate are: ...in one ear or in a sound field environment....

2013-01-01

365

14 CFR 67.305 - Ear, nose, throat, and equilibrium.  

Code of Federal Regulations, 2013 CFR

...CONTINUED) AIRMEN MEDICAL STANDARDS AND CERTIFICATION Third-Class Airman Medical Certificate § 67.305...for a third-class airman medical certificate are: ...in one ear or in a sound field environment....

2013-01-01

366

14 CFR 67.105 - Ear, nose, throat, and equilibrium.  

Code of Federal Regulations, 2010 CFR

...CONTINUED) AIRMEN MEDICAL STANDARDS AND CERTIFICATION First-Class Airman Medical Certificate § 67.105...for a first-class airman medical certificate are: ...in one ear or in a sound field environment....

2010-01-01

367

14 CFR 67.205 - Ear, nose, throat, and equilibrium.  

Code of Federal Regulations, 2011 CFR

...CONTINUED) AIRMEN MEDICAL STANDARDS AND CERTIFICATION Second-Class Airman Medical Certificate § 67.205...a second-class airman medical certificate are: ...in one ear or in a sound field environment....

2011-01-01

368

14 CFR 67.105 - Ear, nose, throat, and equilibrium.  

Code of Federal Regulations, 2011 CFR

...CONTINUED) AIRMEN MEDICAL STANDARDS AND CERTIFICATION First-Class Airman Medical Certificate § 67.105...for a first-class airman medical certificate are: ...in one ear or in a sound field environment....

2011-01-01

369

14 CFR 67.305 - Ear, nose, throat, and equilibrium.  

Code of Federal Regulations, 2011 CFR

...CONTINUED) AIRMEN MEDICAL STANDARDS AND CERTIFICATION Third-Class Airman Medical Certificate § 67.305...for a third-class airman medical certificate are: ...in one ear or in a sound field environment....

2011-01-01

370

14 CFR 67.205 - Ear, nose, throat, and equilibrium.  

...CONTINUED) AIRMEN MEDICAL STANDARDS AND CERTIFICATION Second-Class Airman Medical Certificate § 67.205...a second-class airman medical certificate are: ...in one ear or in a sound field environment....

2014-01-01

371

14 CFR 67.205 - Ear, nose, throat, and equilibrium.  

Code of Federal Regulations, 2010 CFR

...CONTINUED) AIRMEN MEDICAL STANDARDS AND CERTIFICATION Second-Class Airman Medical Certificate § 67.205...a second-class airman medical certificate are: ...in one ear or in a sound field environment....

2010-01-01

372

14 CFR 67.105 - Ear, nose, throat, and equilibrium.  

Code of Federal Regulations, 2012 CFR

...CONTINUED) AIRMEN MEDICAL STANDARDS AND CERTIFICATION First-Class Airman Medical Certificate § 67.105...for a first-class airman medical certificate are: ...in one ear or in a sound field environment....

2012-01-01

373

14 CFR 67.305 - Ear, nose, throat, and equilibrium.  

...CONTINUED) AIRMEN MEDICAL STANDARDS AND CERTIFICATION Third-Class Airman Medical Certificate § 67.305...for a third-class airman medical certificate are: ...in one ear or in a sound field environment....

2014-01-01

374

14 CFR 67.105 - Ear, nose, throat, and equilibrium.  

Code of Federal Regulations, 2013 CFR

...CONTINUED) AIRMEN MEDICAL STANDARDS AND CERTIFICATION First-Class Airman Medical Certificate § 67.105...for a first-class airman medical certificate are: ...in one ear or in a sound field environment....

2013-01-01

375

14 CFR 67.305 - Ear, nose, throat, and equilibrium.  

Code of Federal Regulations, 2012 CFR

...CONTINUED) AIRMEN MEDICAL STANDARDS AND CERTIFICATION Third-Class Airman Medical Certificate § 67.305...for a third-class airman medical certificate are: ...in one ear or in a sound field environment....

2012-01-01

376

Functional measurements of ear pathology in patients and cadaveric preparations  

E-print Network

This work investigated the utility of reflectance (R), a measure of middle-ear mobility, in the differential diagnosis of pathologies responsible for conductive hearing loss (CHL). Current clinical practice cannot distinguish ...

Merchant, Gabrielle Ryan

2014-01-01

377

Ear Problems Approved by the UHS Patient Education Committee  

E-print Network

Impaction occurs when earwax builds up in the canal. It can cause decreased hearing, ear pain, a plugged. Occasionally some individuals produce excess wax and OTC earwax softeners/removal products such as Debrox can

378

40 CFR 211.206-1 - Real ear method.  

Code of Federal Regulations, 2010 CFR

...PROGRAMS PRODUCT NOISE LABELING Hearing Protective Devices § 211...method. (a) The value of sound attenuation to be used in...Measurement of Real-Ear Protection of Hearing Protectors and Physical...in this section. (1) The sound field characteristics...

2010-07-01

379

40 CFR 211.206-1 - Real ear method.  

Code of Federal Regulations, 2011 CFR

...PROGRAMS PRODUCT NOISE LABELING Hearing Protective Devices § 211...method. (a) The value of sound attenuation to be used in...Measurement of Real-Ear Protection of Hearing Protectors and Physical...in this section. (1) The sound field characteristics...

2011-07-01

380

Barotrauma of the ears and sinuses after scuba diving.  

PubMed

The pathophysiology, differential diagnosis, and currently available management of barotrauma affecting the ears and sinuses after scuba diving are reviewed, along with medical standards for resuming scuba diving after barotrauma has resolved. PMID:11407445

Becker, G D; Parell, G J

2001-05-01

381

Forgotten T-tube in the middle ear.  

PubMed

Retention within the middle ear cleft is an unusual complication of T-tube insertion. A 40-year-old woman with Kartagener's Syndrome presented with hearing impairment in the right ear. She was found to have a previously inserted Goode T-tube lying within the middle ear behind an intact drum. She underwent successful removal of the T-tube via a myringotomy, and a new tube was re-inserted. Migration of a T-tube into the middle ear cleft should always be kept in mind in patients who present with otological symptoms and have a history of T-tube insertion, even in the presence of an intact drum. PMID:22538046

Shakeel, Muhammad; Trinidade, Aaron; Khan, Imran; Ah-See, Kim Wong

2012-05-01

382

Tympanostomy Tubes: A Rational Clinical Treatment for Middle Ear Disease.  

ERIC Educational Resources Information Center

The use of tympanostomy tubes to treat middle ear disease including otitis media is discussed with sections on the eustachian tube; acute otitis media; persistent effusion; changes in the tympanic membrane; special populations; and complications. (DB)

Roland, Peter S.; Brown, Orval

1990-01-01

383

Ear decomposition of 3-regular polyhedral links with applications.  

PubMed

In this paper, we introduce a notion of ear decomposition of 3-regular polyhedral links based on the ear decomposition of the 3-regular polyhedral graphs. As a result, we obtain an upper bound for the braid index of 3-regular polyhedral links. Our results may be used to characterize and analyze the structure and complexity of protein polyhedra and entanglement in biopolymers. PMID:24952099

Cheng, Xiao-Sheng; Zhang, Heping; Jin, Xian'an; Qiu, Wen-Yuan

2014-10-21

384

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

385

Efficiency of ear protectors in laboratory and real life tests  

Microsoft Academic Search

The effectiveness of ten different ear-protectors (6 types of earmuffs and 4 types of earplugs) has been tested under laboratory conditions and in the real occupational environment. Three methods were used: (1) physical, utilizing a dummy head; (2) subjective, real-ear, executed on trained human subjects; (3) subjective, measuring TTS2 resulting from occupational, one-workday exposure. It could be shown that the

K. Pawlas; J. Grzesik

1990-01-01

386

Melanoacanthoma of External Ear: Report of Two Cases  

PubMed Central

Melanoacanthoma is a rare lesion. Melanoacanthoma of external ear is still rarer. We present two cases of melanoacanthoma of external ear in adults which presented as pigmented growths and clinically were suspected as malignant lesions. Histopathology was diagnostic as it demonstrated the characteristic elevated lesion with abundant melanin pigment. No recurrence of the lesion was reported after four years of initial diagnosis. These cases have been presented because of their uncommon location, highlighting the differential diagnoses. PMID:24014974

Patnayak, Rashmi; Jena, Amitabh; Chowhan, Amit Kumar; Rukmangadha, Nandyala; Reddy, Mandyam Kumaraswamy

2013-01-01

387

Intratympanic Steroids for Inner Ear Disorders: A Review  

Microsoft Academic Search

Background\\/Aim: The use of intratympanic steroids (ITS) has proliferated over the past 10–15 years to include treatments for inner ear disorders, like Méničre’s Disease (MD) and sudden sensorineural hearing loss (SSNHL). The aim of this study was to review the clinical trials of ITS for inner ear disorders. Methods: PubMed and Ovid Medline databases were searched from 1966 to present

Amanda Hu; Lorne S. Parnes

2009-01-01

388

Mechanics and materials in middle ear reconstruction.  

PubMed

The normal anatomy and physiology of the middle ear is not reproduced in ossiculoplasty and an artificial mechanism for the transmission of sound results. This is true for all types of graft, be they of natural or man-made material. There are, therefore, 2 areas for consideration when looking at the problems encountered in such reconstructions: first, the materials' biocompatability and, secondly, the mechanical effects of the positioning of the graft in the reconstructed ossicular chain. The present work examines these mechanical effects using the finite element method to determine stress and displacement levels in the reconstructed ossicular chain. It is found that the stress levels at the implant-stapedial joint increases as the implant is gradually moved down the malleus. In contrast there is thought to be an increase in sound transmission as the implant is moved down the malleus. Changes in rigidity and hardness of the implant appear to make only modest stress attenuations at the implant-stapes interface. PMID:2032354

Lesser, T H; Williams, K R; Blayney, A W

1991-02-01

389

Experimental evidence against middle ear oxygen absorption.  

PubMed

The present theory of eustachian tube (ET) function and middle ear (ME) ventilation posits that oxygen absorbed by the ME mucosa causes negative ME pressure which is relieved by periodic opening of the ET during swallowing and yawning. After developing a method to cannulate the ET of mongrel dogs we connected the cannulas hermetically to manometers. This system excluded ET function and tested the oxygen absorption capacity of the ME. When we controlled respiration and maintained blood gas PO2 and PCO2 at normal levels, we were unable to find any manometric evidence of negative pressure of gas absorption in the dog ME. Lowering the PCO2 and raising the PO2 of the blood by hyperventilation caused negative ME pressure which could be measured manometrically. We confirmed these findings with the tympanometer. Raising the PCO2 and lowering the PO2 by hypoventilation caused positive pressure in the ME. There is no evidence in these experiments that O2 absorption occurs or causes negative ME pressure in the dog. To the contrary there is evidence that elevated blood levels of the more diffusible CO2 cause an increase in the ME pressure and lowered CO2 level causes a negative ME pressure. PMID:3920459

Buckingham, R A; Stuart, D R; Geick, M R; Girgis, S J; McGee, T J

1985-04-01

390

Lumped parametric model of the human ear for sound transmission.  

PubMed

A lumped parametric model of the human auditoria peripherals consisting of six masses suspended with six springs and ten dashpots was proposed. This model will provide the quantitative basis for the construction of a physical model of the human middle ear. The lumped model parameters were first identified using published anatomical data, and then determined through a parameter optimization process. The transfer function of the middle ear obtained from human temporal bone experiments with laser Doppler interferometers was used for creating the target function during the optimization process. It was found that, among 14 spring and dashpot parameters, there were five parameters which had pronounced effects on the dynamic behaviors of the model. The detailed discussion on the sensitivity of those parameters was provided with appropriate applications for sound transmission in the ear. We expect that the methods for characterizing the lumped model of the human ear and the model parameters will be useful for theoretical modeling of the ear function and construction of the ear physical model. PMID:15300453

Feng, Bin; Gan, Rong Z

2004-09-01

391

Evidence of Inner Ear Contribution in Bone Conduction in Chinchilla  

PubMed Central

We investigated the contribution of the middle ear to the physiological response to bone conduction stimuli in chinchilla. We measured intracochlear sound pressure in response to air conduction (AC) and bone conduction (BC) stimuli before and after interruption of the ossicular chain at the incudo-stapedial joint. Interruption of the chain effectively decouples the external and middle ear from the inner ear and significantly reduces the contributions of the outer ear and middle ear to the bone conduction response. With AC stimulation, both the scala vestibuli Psv and scala tympani Pst sound pressures drop by 30 to 40 dB after the interruption. In BC stimulation, Psv decreases after interruption by about 10 to 20 dB, but Pst is little affected. This difference in the sensitivity of the BC induced Psv and Pst to ossicular interruption is not consistent with a BC response to ossicular motion, but instead suggests a significant contribution of an inner-ear drive (e.g. cochlear fluid inertia or compressibility) to the BC response. PMID:23211609

Chhan, David; Roosli, Christof; McKinnon, Melissa L.; Rosowski, John J.

2013-01-01

392

Minnesota wolf ear lengths as possible indicators of taxonomic differences  

USGS Publications Warehouse

Genetic findings suggest that 2 types of wolves, Canis lupus (Gray Wolf) and C. lycaon (Eastern Wolf), and/or their hybrids occupy Minnesota (MN), and this study examines adult wolf ear lengths as a possible distinguisher between these two. Photographic evidence suggested that the Eastern Wolf possesses proportionately longer ears than Gray Wolves. Ear lengths from 22 northwestern MN wolves from the early 1970s and 22 Alaskan wolves were used to represent Gray Wolves, and the greatest length of the sample (12.8 cm) was used as the least length to demarcate Eastern Wolf from Gray Wolf influence in the samples. Twenty-three percent of 112 adult wolves from Algonquin Park in eastern Ontario and 30% of 106 recent adult wolves in northeastern MN possessed ears >12.8 cm. The northeastern MN sample differed significantly from that of current and past northwestern MN wolves. Ear-lengths of wolves in the eastern half of the northeastern MN wolf population were significantly longer than those in the western half of that study area, even though the mean distance between the 2 areas was only 40 km, and the mean length of my 2004–2009 sample was significantly longer than that of 1999–2003. These findings support the hypothesis that Eastern Wolves tend to possess longer ears than do Gray Wolves and suggest a dynamic hybridization process is still underway in MN.

Mech, L. David

2011-01-01

393

Developmental origin and fate of middle ear structures.  

PubMed

Results from developmental and phylogenetic studies have converged to facilitate insight into two important steps in vertebrate evolution: (1) the ontogenetic origin of articulating elements of the buccal skeleton, i.e., jaws, and (2) the later origins of middle ear impedance-matching systems that convey air-borne sound to the inner ear fluids. Middle ear ossicles and other skeletal elements of the viscerocranium (i.e., gill suspensory arches and jaw bones) share a common origin both phylogenetically and ontogenetically. The intention of this brief overview of middle-ear development is to emphasize the intimate connection between evolution and embryogenesis. Examples of developmental situations are discussed in which cells of different provenance, such as neural crest, mesoderm or endoderm, gather together and reciprocal interactions finally determine cell fate. Effects of targeted mutagenesis on middle ear development are described to illustrate how the alteration of molecularly-controlled morphogenetic programs led to phylogenetic modifications of skeletal development. Ontogenetic plasticity has enabled the diversification of jaw elements as well as middle ear structures during evolution. This article is part of a special issue entitled "MEMRO 2012". PMID:23396272

Sienknecht, Ulrike J

2013-07-01

394

Arteriovenous malformation of the mandible and parotid gland  

PubMed Central

Arteriovenous malformations (AVMs) of the jaws are relatively rare, with fewer than 200 cases reported in the literature. Their real importance lies in their potential to result in exsanguination, which usually follows an unrelated treatment, such as tooth extraction, surgical intervention, puncture wound or blunt injury in involved areas, with the dentist unaware of the existence of the AVM. The present case illustrates an AVM in an 18-year-old female with swelling on the right side of the face. This case report is unique because although there was no history of bleeding episodes, thorough examination and investigation diagnosed it as high-flow vascular malformation. We ascertain the importance of dentists' awareness of the fatal outcome of these lesions and emphasize that, prior to performing any procedure, necessary investigations should always be done. PMID:22282511

Shailaja, S R; Manika; Manjula, M; Kumar, L V

2012-01-01

395

[Chiari type I malformation in a patient with Poland's syndrome].  

PubMed

Chiari malformation type I and Poland's syndrome are two rare diseases and their simultaneous presentation had not been previously described in the literature. We report the case of a 27 year old male with history of Poland's syndrome, who referred headache and motor impairment of the intrinsic muscles of the left hand. In a cervical spine MR a Chiari I malformation with syringomyelia from C1 to T2 was found, which was treated by foramen magnum decompression, dural plasty and removal of the posterior arch of the atlas. A discussion of the embryological mechanisms that might be involved in the coexistence of these two entities is presented, emphasizing the role of para-axial mesoderm. PMID:22167290

Apellido, Nombre; Alcalá-Cerra, G; Sabogal-Barrios, R; Nińo-Hernández, L M; Gutiérrez-Paternina, J J; Moscote-Salazar, L R; Preciado-Mesa, E

2011-12-01

396

Arnold-Chiari malformation in a captive African lion cub.  

PubMed

Progressive ataxia, delayed growth, dementia and tremors were noted in a female African lion (Panthera leo) cub at the Tel-Aviv Ramat-Gan Zoological Center (Israel). The lioness was 3-mo-old when clinical signs were first noticed. Repeated neurological evaluations and blood tests were conducted in an attempt to establish a diagnosis. A congenital abnormality was suspected and the lioness died 6 molater. Post mortem examination revealed an Arnold-Chiari malformation. The abnormality was classified as a Chiari type 2 malformation, based on the herniation of the cerebellar vermis and paravermis and the slight caudal displacement of the medulla, combined with lack of displacement in other parts of the brainstem. PMID:9706583

Shamir, M H; Horowitz, I H; Yakobson, B; Ofri, R

1998-07-01

397

Congenital malformations of the vertebral column in ancient amphibians.  

PubMed

Temnospondyls, the largest group of Palaeozoic and Mesozoic amphibians, primitively possess rhachitomous vertebrae with multipartite centra (consisting of one horse-shoe-shaped inter- and paired pleurocentra). In a group of temnospondyls, the stereospondyls, the intercentra became pronounced and disc-like, whereas the pleurocentra were reduced. We report the presence of congenital vertebral malformations (hemi, wedge and block vertebrae) in Permian and Triassic temnospondyls, showing that defects of formation and segmentation in the tetrapod vertebral column represent a fundamental failure of somitogenesis that can be followed throughout tetrapod evolution. This is irrespective of the type of affected vertebra, that is, rhachitomous or stereospondylous, and all components of the vertebra can be involved (intercentrum, pleurocentrum and neural arch), either together or independently on their own. This is the oldest known occurrence of wedge vertebra and congenital block vertebra described in fossil tetrapods. The frequency of vertebral congenital malformations in amphibians appears unchanged from the Holocene. PMID:23551141

Witzmann, F; Rothschild, B M; Hampe, O; Sobral, G; Gubin, Y M; Asbach, P

2014-04-01

398

Resection of pontine cavernous malformation through the pontomedullary sulcus.  

PubMed

In this video, we illustrate a right far-lateral craniotomy for resection of a 13-mm cavernous malformation of the pons in a healthy 53-year-old female patient presenting with diplopia and right 6th nerve palsy. The cavernous malformation was surrounded by normal pons, but was within 1 mm of the pontomedullary sulcus. The lesion was exposed from below through a far lateral craniotomy and accessed through the vasoaccessory triangle, superior to olivary nucleus and 12th cranial nerve. The alternative retrosigmoid craniotomy would have involved significant transgression of the middle cerebellar peduncle. The patient had gross-total resection and some temporary increase in her abducens nerve palsy without any complication. The video can be found here: http://youtu.be/8nOnrnTk3Tg . PMID:24380516

Abla, Adib Adnan; Clark, Aaron J; Lawton, Michael L

2014-01-01

399

Cloacal malformation: embryology, anatomy, and prenatal imaging features.  

PubMed

Cloacal malformation is a rare but important anomaly. Prenatal diagnosis is possible with knowledge of the distinctive imaging features. The purpose of this case series is to illustrate characteristic prenatal sonographic and magnetic resonance imaging features of cloacal malformation using imaging from 6 cases seen at a single academic center to augment published data. The imaging feature common to all cases was a central cystic pelvic mass containing a characteristic fluid-fluid level. Additional anomalies include uterine and vaginal duplication, hydronephrosis, and lumbosacral anomalies. Prenatal magnetic resonance imaging showed the absence of the normal T1-hyperintense meconium-filled rectum in all cases. Prenatal diagnosis may affect immediate neonatal care (eg, immediate drainage of hydrocolpos) with an ultimate improved outcome. PMID:23091258

Winkler, Nicole S; Kennedy, Anne M; Woodward, Paula J

2012-11-01

400

[Facial venous malformation presented with an unusual course].  

PubMed

Venous malformations are constitutionally-dependent vascular anomalies. In contrast to haemangiomas, they show no spontaneous regression. We describe here the case of a 21-year-old woman with a very severe facial disfigurement caused by a large venous malformation. After intensive consultation, advice and planning, the patient decided to undergo therapy and was admitted to hospital for treatment by an interdisciplinary team, consisting of a radiologist and a plastic surgeon. Under general anaesthesia, percutaneous sclerosis was performed twice. This enabled thrombosing and subsequent extensive resection of the monstrously large tumour. The well-planned and efficiently performed surgery helped the patient to gain a significant improvement in her quality of life. PMID:19085823

Piza-Katzer, H; Waldenberger, P

2009-04-01

401

Juvenile Pilocytic Astrocytoma in Association with Arteriovenous Malformation  

PubMed Central

Summary Pilocytic astrocytomas are highly vascular, relatively common primary brain tumors in the pediatric population, but their association with a true arteriovenous malformation (AVM) is extremely rare. We describe an eight-year-old girl with a right supratentorial juvenile pilocytic astrocytoma (WHO grade I) with an angiographically documented AVM entangled in the tumor mass who presented with intracranial hemorrhage due to a ruptured anterior choroidal artery pseudoaneurysm encased in the lesion. The AVM nidus as well as the hemorrhage site was embolized with Onyx. A literature review revealed only one previous report of a true intermixture of these two lesions. We hypothesize whether the association of vascular malformations and primary brain tumors are merely coincidental or if they point to the existence of a distinct entity and/or a common etiologic factor. PMID:22681727

Soltanolkotabi, M.; Schoeneman, S.E.; Dipatri, A.J.; Hurley, M.C.; Ansari, S.A.; Rajaram, V.; Tomita, T.; Shaibani, A.

2012-01-01

402

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

403

The evaluation of ear canal, middle ear, temporal bone, and cerebellopontine angle masses in infants, children, and adolescents.  

PubMed

Ear canal, middle ear, temporal bone, and CPA angle masses (except for cholesteatomas) are rare in the pediatric population. The physician needs to have a high degree of suspicion for such lesions if a child presents with ear pain unrelated to infection or otorrhea that fails to improve after treatment. A precise diagnosis needs to be made in these children and also in those with hearing loss, vertigo, and facial paralysis. The most useful imaging procedures for ear, temporal bone, and CPA masses are CT and MR imaging. With a suspected vascular lesion, a definitive diagnosis usually can be made by an imaging procedure or angiography. In all cases of mass lesions, except for some aneurysms and infections, a tissue diagnosis must be secured. PMID:1442313

Bellet, P S; Benton, C; Matt, B H; Myer, C M

1992-01-01

404

Novel Image-Guided Management of a Uterine Arteriovenous Malformation  

SciTech Connect

The investigators present a novel image-guided embolization, not previously described, of a uterine arteriovenous malformation (AVM) resistant to endovascular management. The uterus was exposed surgically, and Histoacryl (Braun, Fulda, Germany) was injected directly into the nidus using ultrasound guidance and fluoroscopy. The patient had a successful full-term pregnancy after this procedure. This technique may be a useful alternative management strategy in patients with uterine AVM who fail traditional endovascular embolization and who still desire fertility.

Przybojewski, Stefan J., E-mail: drstefanp@hotmail.com; Sadler, David J. [University of Calgary, Diagnostic Imaging Department, Foothills Hospital (Canada)

2011-02-15

405

Pediatric stroke: the importance of cerebral arteriopathy and vascular malformations  

Microsoft Academic Search

Purpose  Population-based estimates of the annual incidence of childhood stroke range from 2 to 13 per 100,000 person-years. More than\\u000a half of children who have had a stroke have long-term neurological sequelae. The goal of this article is to review recent\\u000a literature on both hemorrhagic and ischemic stroke in children with a focus on cerebral arteriopathy and vascular malformations\\u000a as stroke

Lauren A. Beslow; Lori C. Jordan

2010-01-01

406

Arteriovenous Malformation of the Pancreas: Report of a Case  

Microsoft Academic Search

Arteriovenous malformation (AVM) of the pancreas (AVMP) is uncommon and generally asymptomatic; therefore, few cases have\\u000a so far been reported. The symptoms of AVMP include gastrointestinal bleeding, pain, and portal hypertension. Definitive diagnosis\\u000a is confirmed by angiographic study, and surgery is the only effective treatment. We report a case of AVMP confirmed by computed\\u000a tomography, magnetic resonance imaging, and angiographic

Jean M. Butte; Francisco Pacheco; Antonieta Solar; Fernando J. Crovari; Nicolás P. Jarufe

2007-01-01

407

Epidemiology, diagnostics and treatment of vascular tumours and malformations.  

PubMed

Vascular tumours and vascular malformations are common vasculose anomalies characteristic for dissimilar clinical course, specific biological as well as immune cytological and histological properties. Vascular lesions classification system and their detailed division into groups and subgroups were elaborated and implemented in Rome, in 1996, during meeting of the International Society for the Study of Vascular Anomalies (ISSVA). It was based on modification of an earlier going division by Mullikien and G?owacki from 1982. Infantile hemangiomas are the most numerous group of benign tumours of mesenchymal origin. Vascular malformations appear definitely less often. They are composed of normal endothelium lined displastic vessels which originate from vascular tissue abnormal morphogenesis. In contrast, in hemangiomas, at the proliferation stage, increased, multiplication of endothelial cells is observed as well as of fibroblasts, mastocytes and macrophages. Infantile hemangiomas are usually not present at the moment of birth and white chloasma with superficial teleangiectasis appears which increases within 3-4 weeks and gets bright red colour and reveal very characteristic clinical course basing on intensive growth period and involution long process. Vascular malformations are observed most often at the delivery moment or they may appear at an early childhood. They enlarge proportionally along with the child's growth and their sudden expansion may be triggered by an infection, hormonal changes or trauma. Contrary to hemangiomas, they do not subside spontaneously and their abrupt increase may result in impairment or deformation of important anatomical structures. Infantile hemangiomas and vascular malformations require different and individual treatments which are often multi-stage procedures carried on in specialistic centres of plastic surgery, vascular surgery or maxillofacial surgery. PMID:24979522

Wójcicki, Piotr; Wójcicka, Karolina

2014-01-01

408

Venous thoracic outlet syndrome caused by a congenital rib malformation  

PubMed Central

Summary Venous thoracic outlet syndrome (VTOS) represents a rare disorder. Hypertrophy of the anterior scalene musculature is the cause of the compression syndrome in most cases. To our knowledge, we describe the first reported case worldwide of a venous compression syndrome caused by a congenital malformation of the 1st and 2nd ribs. Treatment by transaxillary partial rib resection was necessary and a very good postoperative result was achieved. PMID:22544354

Kirschbaum, Andreas; Palade, Emanuel; Csatari, Zoltan; Passlick, Bernward

2012-01-01

409

Surgical treatment of symptomatic cavernous malformations of the brainstem  

Microsoft Academic Search

Summary  \\u000a Introduction and objectives. Cavernous malformations (CM) at the level of the brainstem, continue to present a challenge in therapeutic terms and are\\u000a an important source of controversy. Here we present our experience and the results obtained by adopting surgical treatment.\\u000a \\u000a \\u000a Materials and methods. The results of a consecutive series of 17 patients were studied. The surgical intervention was designed

R. G. Sola; P. Pulido; J. Pastor; M. Ochoa; J. Castedo

2007-01-01

410

Gated magnetic resonance imaging of congenital cardiac malformations  

SciTech Connect

Magnetic resonance (MR) images of a variety of cardiac malformations in 19 patients aged 1 week to 33 years were obtained using pulse plethysmographic- or ECG-gated spin echo pulse sequences. Coronal, axial, and sagittal images displaying intracardiac structures with excellent spatial and contrast resolution were acquired during systole or diastole. It is concluded that MR will be a valuable noninvasive method of diagnosing congenital heart disease.

Fletcher, B.D.; Jocobstein, M.D.; Nelson, A.D.; Riemenschneider, T.A.; Alfidi, R.J.

1984-01-01

411

Vascular Malformations of the Spine and Spinal Cord  

Microsoft Academic Search

Spinal vascular malformations are rare diseases with a wide variety of neurologic presentations. Their classification depends\\u000a on the differentiation of shunting versus nonshunting lesions, the latter being the spinal cord cavernomas. In the shunting\\u000a lesions, the next step in the proposed classification scheme is related to the feeding artery which can subdivide the dural\\u000a vascular shunts from the pial vascular

Timo Krings

2010-01-01

412

Cerebral arteriovenous malformation: prenatal and postnatal central blood flow dynamics  

Microsoft Academic Search

Using the Doppler technique, this study compared the prenatal and postnatal flow patterns of an infant with cerebral arteriovenous (AV) malformation. Fetal right ventricular end-diastolic dimension was 1.7 cm with right ventricular ejection equaling 66% of the combined cardiac output. Diastolic flow was reversed in the fetal aortic isthmus but forward-moving in the descending aorta, resulting in a watershed phenomenon.

D. J. Patton; J-C Fouron

1995-01-01

413

Volume measurement of cerebral arteriovenous malformations from angiography.  

PubMed

We designed software for measuring the volume of cerebral arteriovenous malformations from angiography and validated it against prescription volumes in radiosurgery. We aimed to create a model for the risk for complications as a function of volume, based on established outcome prediction models for Gamma Knife radiosurgery, but without the need for dose planning. We created an application for computing the volume of cerebral arteriovenous malformations from the intersection of two X-ray cones in stereotactic space. Volume measurements were compared with prescription volumes from dose planning, in phantoms and in patients treated with Gamma Knife radiosurgery for cerebral arteriovenous malformations. Previous studies of 1128 treated patients were used to calculate the risk for complication as a function of the nidus volume. In 63 patients volumes measured with either method correlated, R2 = 0.85. Volume as measured with the intersecting cone model (ICM) correlated with predicted Gamma Knife radiosurgery complication rate, R2 = 0.84. The ICM can thus be used for measurement of AVM volumes less than 10 cm3 from angiography. Outcome models from Gamma Knife radiosurgery may be applied, but with reduced exactness. Standardised AVM volume measurement is valuable for comparing outcome and for quantification of volume reduction after therapy, notably embolisation. Thus the optimal management plan may be selected in conjunction with diagnostic or therapeutic angiography. PMID:11071447

Söderman, M; Karlsson, B; Launnay, L; Thuresson, B; Ericson, K

2000-09-01

414

Environmental monitoring using malformed embryos of the amphipod Monoporeia affinis  

SciTech Connect

Reproduction variables of Monoporeia affinis, such as embryonic malformation were confirmed as the most sensitive variable, when soft bottom microcosms were exposed to metals such as cadmium and lead, arsenic, organic compounds such as 4,5,6 trichloroguaiacol, contaminated sediment from areas impacted by heavy metals and pulp mill effluents. The effects were demonstrated also in low concentrations that did not significantly affect the meiofauna community. The microcosm test-system with high ecological realism could offer a possibility to translate laboratory results to the natural environments. Field surveys outside different types of pulp mills and metal works on the coast of the Gulf of Bothnia have confirmed the laboratory results. Significantly higher levels of malformed embryos of Monoporeia affinis were demonstrated in the impacted areas in comparison with reference areas. The reproduction variables of Monoporeia affinis have been used in the national environmental monitoring program during two years and results indicated possibilities to distinguish between effects of xenobiotica and secondary eutrophication effects, such as unsaturated oxygen condition and occurrence of sulfides, which resulted in increased frequencies of dead eggs but not affected the frequencies of malformed eggs and embryos.

Sundelin, B.; Eriksson, A.K. [Stockholm Univ. (Sweden). Inst. of Applied Environmental Research

1995-12-31

415

Sonographic markers for early diagnosis of fetal malformations  

PubMed Central

Fetal malformations are very frequent in industrialized countries. Although advanced maternal age may affect pregnancy outcome adversely, 80%-90% of fetal malformations occur in the absence of a specific risk factor for parents. The only effective approach for prenatal screening is currently represented by an ultrasound scan. However, ultrasound methods present two important limitations: the substantial absence of quantitative parameters and the dependence on the sonographer experience. In recent years, together with the improvement in transducer technology, quantitative and objective sonographic markers highly predictive of fetal malformations have been developed. These markers can be detected at early gestation (11-14 wk) and generally are not pathological in themselves but have an increased incidence in abnormal fetuses. Thus, prenatal ultrasonography during the second trimester of gestation provides a “genetic sonogram”, including, for instance, nuchal translucency, short humeral length, echogenic bowel, echogenic intracardiac focus and choroid plexus cyst, that is used to identify morphological features of fetal Down’s syndrome with a potential sensitivity of more than 90%. Other specific and sensitive markers can be seen in the case of cardiac defects and skeletal anomalies. In the future, sonographic markers could limit even more the use of invasive and dangerous techniques of prenatal diagnosis (amniocentesis, etc.). PMID:24179631

Renna, Maria Daniela; Pisani, Paola; Conversano, Francesco; Perrone, Emanuele; Casciaro, Ernesto; Renzo, Gian Carlo Di; Paola, Marco Di; Perrone, Antonio; Casciaro, Sergio

2013-01-01

416

[Complications in the evolution of haemangiomas and vascular malformations].  

PubMed

The differentiation of haemangiomas and vascular malformations is histological, clinical and prognostic. Although the majority of haemangiomas evolve towards spontaneous resolution, as many as 10% of cases can develop complications with ulceration, pain and haemorrhaging. Besides, the localisation of haemangiomas in the head and neck, next to vital structures, can compromise their functions. Hence, compression of the airway might be a vital emergency. Periorbital haemangiomas can give rise to amblyopia due to sensory deprivation or due to a restrictive strabismus. Lumbosacral haemangiomas must be studied with Nuclear Magnetic Resonance because of their frequent association with alterations in the midline at the level of the spine, anus, genitals or kidneys. Amongst visceral haemangiomas, hepatic haemangiomas are the most serious due to their association with congestive cardiac insufficiency. The association of extensive facial haemangiomas with anomalies of the central nervous system, vascular, cardiac, ocular and sternal anomalies, is denominated PHACE syndrome and is frequently complicated by mental deficiency, convulsions or ictus. Vascular malformations of trigeminal localisation are associated in up to 15% of cases with glaucoma or choroidal or leptomeningeal haemangiomas (Sturge-Weber syndrome). Combined vascular malformations localised in the extremities can become complicated with thrombophlebitis, regional osteolysis and even distant thromboembolisms (Klippel-Treneaunay Syndrome). On the other hand, there is a coagulopathy due to consumption (Kassabach-Merrit Syndrome) that can complicate some vascular tumours such as the Kaposiform haemangioendothelioma and the tufted angioma. Finally, the complications of the treatments employed are reviewed. PMID:15148512

Belzunce, A; Casellas, M

2004-01-01

417

Congenital malformations of the central nervous system in spontaneous abortions.  

PubMed Central

A study of 2620 pregnancies ending in spontaneous abortion revealed a CNS defect in 3.6% of embryos and fetuses, and 3% of all complete conceptuses. The type of malformation observed varied with the gestational age at expulsion, encephaloceles being predominant in earlier specimens, while more typical anencephalus and spina bifida were more common among later abortions. Chromosome abnormalities were found in 40% of abortuses with CNS defects, but were almost entirely confined to those which were still at the embryonic stage of development. 53% of the latter were chromosomally abnormal, which is the same as the proportion found among embryos without a CNS malformation. Using published life-tables of recognized pregnancies it was estimated that the prevalence of anencephalus, spina bifida, or related malformation (other than hydrocephalus), without a chromosome anomaly, is 5.3 per thousand conceptuses at the beginning of the eighth week of gestation. By comparing this with the prevalence in total births, it was further estimated that only 24% of these are born alive, with 54% aborting spontaneously and 22% being stillborn. PMID:775092

Creasy, M R; Alberman, E D

1976-01-01

418

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

419

OBESITY AND THE RISK AND DETECTION OF FETAL MALFORMATIONS  

PubMed Central

The incidence of obesity in pregnancy has increased over the past two decades, with nearly 50% of U.S. women aged 15–49 are classified as overweight or obese. Obesity (independent of diabetes) among gravidae poses unique risks which extend towards the fetus, with several large population-based analyses demonstrating independent increased risks for fetal malformations including neural tube defects, cardiac anomalies, and orofacial clefts as well as stillbirth and macrosomia. Unfortunately, several lines of evidence also suggest that the quality of the prenatal fetal anatomic survey and certain aspects of prenatal diagnostic screening programs are significantly limited. The net effect is that among obese gravidae, the increased risk of fetal anomalies is further offset by a concomitant diminished ability to sonographically detect such malformation in the prenatal interval. The purpose of this summary review is to systematically examine the evidence suggesting an increased risk of fetal malformations in obese gravidae, the contributing role of diabetes, and the limitations of prenatal diagnostic and sonographic screening among this at-risk population. PMID:22713503

RACUSIN, Diana; STEVENS, Blair; CAMPBELL, Genevieve; AAGAARD-TILLERY, Kjersti

2012-01-01

420

What is otitis media? Otitis media is an inflammation of the middle ear that usually occurs as the result of a middle ear  

E-print Network

into the tympanic membrane (ear drum). The tube allows any fluid to drain out and helps keeps the air pressure· What is otitis media? Otitis media is an inflammation of the middle ear that usually occurs as the result of a middle ear infection. Common symptoms can include fever, pain, hearing problems, ruptured

O'Toole, Alice J.

421

The genetics of auricular development and malformation: new findings in model systems driving future directions for microtia research.  

PubMed

Microtia is a term used to describe a wide array of phenotypic presentations of the outer ear. Although the majority of the cases are isolated in nature, much of our understanding of the causes of microtia has been driven by the identification of genes underlying syndromic forms where the anomaly co-presents with various other craniofacial and extra-craniofacial structural defects. In this review we discuss recent findings in mice deficient in Hoxa2, a key regulator of branchial arch patterning, which has necessitated a revision to the canonical model of pinna morphogenesis. The revised model will likely impact current classification schemes for microtia and, as we argue in this review, the interpretation of the developmental basis for various auricular malformations. In addition, we highlight recent studies in other mammalian species that are providing the first clues as to possible causes of at least some isolated anomalies and thus should now accelerate the search for the more elusive genetic contributions to the many isolated and non-syndromic cases of microtia. These findings, together with the application of new genome-level sequencing technologies and more thorough quantitative assessment of available mutant mouse resources, promise an exciting future for genetic studies in microtia. PMID:24880027

Cox, Timothy C; Camci, Esra D; Vora, Siddharth; Luquetti, Daniela V; Turner, Eric E

2014-08-01

422

The ear converts the pressure amplitude variations of sound waves into sensations that we can perceive. The central point to be gleaned from this description is that the ear  

E-print Network

push on the eardrum. Because the other side of the ear drum (known as the middle ear) is held at a fairly constant pressure the sound causes the eardrum to vibrate. 2. The vibrations of the ear drumThe Ear The ear converts the pressure amplitude variations of sound waves into sensations that we

Robertson, William

423

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

424

Finite element modeling of energy absorbance in normal and disordered human ears.  

PubMed

The finite element (FE) model of the human ear has been developed to analyze the middle ear and cochlea function in relation to the ear structures. However, the energy absorbance or energy reflectance used in the research and clinical audiology test has not been reported in the FE model. The relationship between the middle ear structure and the energy absorbance (EA) needs to be identified using the FE model. In this study, a FE model of the human ear, including the ear canal, the middle ear and the spiral cochlea constructed from the histological sections of a human temporal bone, was used to calculate EA. The viscoelastic material properties were applied to the middle ear soft tissues. Three middle ear disorders were simulated in the FE model: otitis media, otosclerosis, and ossicular chain disarticulation. Multi-physics (acoustic, structure, and fluid) coupled analysis was conducted in the model. The FE model was first validated with the published experimental data on the middle ear input impedance and EA of the normal ear. The EA in three disordered ears was obtained from the model and compared with the published results measured in the clinics and the temporal bone experiments. The consistence of the model-derived EA with the published data demonstrates that the FE model is feasible to analyze EA. The effects of middle ear pressure, middle ear effusion, and mechanical properties of soft tissues on EA were estimated and discussed. This article is part of a special issue entitled "MEMRO 2012". PMID:23274858

Zhang, Xiangming; Gan, Rong Z

2013-07-01

425

Thoracolumbar spinal vascular malformation as a rare cause of isolated intraventricular hemorrhage.  

PubMed

Spinal vascular malformations are rare vascular lesions that most frequently present with back pain, radiculopathy, and/or myelopathy. Neurological decline is typically secondary to progressive radiculopathy, myelopathy, venous thrombosis, and stroke. Few case reports have described thoracolumbar spinal vascular malformations that present with both subarachnoid and intraventricular hemorrhage. This is the first reported case of a thoracolumbar spinal vascular malformation presenting with isolated intraventricular hemorrhage on initial imaging followed by acute and fatal rehemorrhage. PMID:24784978

Marlin, Evan S; Entwistle, John J; Arnold, Michael A; Pierson, Christopher R; Governale, Lance S

2014-07-01

426

Combined laparoscopic and cystoscopic injection sclerotherapy for bladder venous malformation: a novel technique.  

PubMed

Treatment of vascular malformations of the urinary bladder can be challenging. We report a case of bladder venous malformation treated with sodium tetradecyl sulphate (STS 3%) sclerotherapy, using a combined cystoscopic and percutaneous transperitoneal laparoscopy guided approach. When cystoscopic views are poor, the laparoscopic approach is a useful adjunct to aid sclerotherapy of bladder venous malformation. This technique has not been previously described. PMID:22841403

Sinha, C K; Barnacle, A; Mushtaq, I; Cherian, A

2013-02-01

427

Papillary endothelial hyperplasia in association with vascular malformation of the hand.  

PubMed

Vascular malformations are uncommonly encountered in the hand. This case report involves the unique case of a rapidly enlarging mass associated with a known vascular malformation. Final pathology revealed dense reactive fibrous tissue, with prominent blood vessels, consistent with a venous malformation and associated papillary endothelial hyperplasia. The intraoperative finding of two separately identifiable masses, one fibrotic and one vascular, has not previously been reported. PMID:24875342

Alves, Kristin; Bauer, Andrea; Jupiter, Jesse

2014-01-01

428

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

429

Emerging role of contrast-enhanced MRI in diagnosing vascular malformations.  

PubMed

Vascular malformations comprise a diverse and rare group of lesions which generally pose a formidable treatment challenge. Requisite for optimal surgical planning are imaging modalities capable of delineating involved anatomy and malformation flow characteristics. In this regard, we and others have purported the advantages of contrast-enhanced MRI. Here, we review the current body of literature regarding the emerging of role of contrast enhanced MRI for the management of vascular malformations. PMID:25301311

Turley, Ryan S; Lidsky, Michael E; Markovic, Jovan N; Shortell, Cynthia K

2014-07-01

430

Cervical spine malformation in cornelia de lange syndrome: a report of three patients.  

PubMed

Cornelia de Lange syndrome (CdLS) is a complex genetic disease with skeletal involvement mostly related to upper limb malformations. We report on three males with clinical and molecular diagnoses of CdLS. Besides typical CdLS features, all showed different cervical spine malformations. To the best of our knowledge, this is an unusual malformation in the CdLS phenotypic spectrum. PMID:24668777

Bettini, Laura Rachele; Locatelli, Laura; Mariani, Milena; Cianci, Paola; Giussani, Carlo; Canonico, Francesco; Cereda, Anna; Russo, Silvia; Gervasini, Cristina; Biondi, Andrea; Selicorni, Angelo

2014-06-01

431

Human ear detection in the thermal infrared spectrum  

NASA Astrophysics Data System (ADS)

In this paper the problem of human ear detection in the thermal infrared (IR) spectrum is studied in order to illustrate the advantages and limitations of the most important steps of ear-based biometrics that can operate in day and night time environments. The main contributions of this work are two-fold: First, a dual-band database is assembled that consists of visible and thermal profile face images. The thermal data was collected using a high definition middle-wave infrared (3-5 microns) camera that is capable of acquiring thermal imprints of human skin. Second, a fully automated, thermal imaging based ear detection method is developed for real-time segmentation of human ears in either day or night time environments. The proposed method is based on Haar features forming a cascaded AdaBoost classifier (our modified version of the original Viola-Jones approach1 that was designed to be applied mainly in visible band images). The main advantage of the proposed method, applied on our profile face image data set collected in the thermal-band, is that it is designed to reduce the learning time required by the original Viola-Jones method from several weeks to several hours. Unlike other approaches reported in the literature, which have been tested but not designed to operate in the thermal band, our method yields a high detection accuracy that reaches ~ 91.5%. Further analysis on our data set yielded that: (a) photometric normalization techniques do not directly improve ear detection performance. However, when using a certain photometric normalization technique (CLAHE) on falsely detected images, the detection rate improved by ~ 4%; (b) the high detection accuracy of our method did not degrade when we lowered down the original spatial resolution of thermal ear images. For example, even after using one third of the original spatial resolution (i.e. ~ 20% of the original computational time) of the thermal profile face images, the high ear detection accuracy of our method remained unaffected. This resulted also in speeding up the detection time of an ear image from 265 to 17 milliseconds per image. To the best of our knowledge this is the first time that the problem of human ear detection in the thermal band is being investigated in the open literature.

Abaza, Ayman; Bourlai, Thirimachos

2012-06-01

432

Clinical Features: Goldberg-Shprintzen megacolon syndrome (GOSHS, OMIM #609460) is a multiple malformation disorder  

E-print Network

malformation disorder characterized by Hirschsprung megacolon, microcephaly, hypertelorism, submucous cleft:185-189. 2. Hurst, JA., et al. Unknown syndrome: Hirschsprung's disease, microcephaly, and iris coloboma

Ober, Carole

433

Complex thoracic malformations: is there an association between adolescent idiopathic scoliosis and pectus excavatum?.  

E-print Network

??Study Design: This is a retrospective review of 220 patients with adolescent idiopathic scoliosis (AIS).Objectives: When combined thoracic malformations occur scoliosis and pectus excavatum (PE)… (more)

Berdan, Elizabeth Ann

2013-01-01

434

Trematode infection causes malformations and population effects in a declining New Zealand fish.  

PubMed

1. Animal malformations engender wide public and scientific concern because of associated environmental health risks. This is highlighted by increased incidence of limb malformations in amphibians associated with trematode infections and disturbance. Malformations may signal new emerging disease threats, but whether the phenomenon is broadly applicable across taxa, or has population-scale impacts, is unknown. 2. Malformations are widely reported in fish and, until now, have been attributed mainly to contaminants. We tested whether the trematode Telogaster opisthorchis caused severe malformations, leading to population effects, in Galaxias anomalus, a threatened New Zealand freshwater fish. 3. Experimental infection of larval fish caused increasing spinal malformation and mortality with infection intensity that closely matched field patterns. Field malformation frequency peaked in January (65%), before declining sharply in February (25%) and remaining low thereafter. 4. The peak occurred during a 'critical window' of larval development, with the decline coincident with a population crash, indicating that malformation was causing mortality in the field. 5. The occurrence of such critical developmental windows may explain why this mechanism of population impact has been overlooked. With global environmental stressors predicted to enhance trematode infections, our results show that parasite-induced malformation, and its population-scale impacts, could be more widespread than previously considered. PMID:19886894

Kelly, David W; Thomas, Harriet; Thieltges, David W; Poulin, Robert; Tompkins, Daniel M

2010-03-01

435

Effect of ibudilast on microcirculation thrombosis in rat inner ear.  

PubMed

The effect of ibudilast (0.1, 0.3 mg/kg), which has cerebral vasodilating and antiplatelet effects, was evaluated in two models of rat inner ear microcirculation thrombosis by using the photochemical reaction between green light (wave length: 540 nm) and intravenous injection of rose bengal. Furthermore, the inner ear blood flow was measured by a laser-Doppler flowmeter. In the hearing disturbance model, under anesthesia, the compound action potential of the cochlear nerve (AP) was measured by an electrocochleogram. The sound stimulus was an 8-kHz sine wave at 80 dB SPL. The AP was calculated 128 times. In the controls, the AP disappeared about 4 min after the intravenous injection of rose bengal (20 mg/kg). The time required to completely suppress the AP in the animals treated with ibudilast (0.1, 0.3 mg/kg) was significantly prolonged as compared with that in the controls. In the equilibrium dysfunction model, ibudilast (0.1, 0.3 mg/kg) reduced the time of abnormal swimming in the swimming test 24 hr after the completion of photo-illumination. Ibudilast (0.3 mg/kg) increased the inner ear blood flow during the 10-min observation period as compared with the controls, while it did not affect the mean blood pressure. In conclusion, ibudilast increased the inner ear blood flow and was effective in two models of rat inner ear microcirculation thrombosis. PMID:8459653

Umemura, K; Asai, Y; Hirata, Y; Uematsu, T; Nakashima, M

1993-02-01

436

Anisotropic yield function capable of predicting eight ears  

NASA Astrophysics Data System (ADS)

Deep drawing of a cylindrical cup from a rolled sheet is one of the typical forming operations where the effect of this anisotropy is most evident. Indeed, it is well documented in the literature that the number of ears and the shape of the earing pattern correlate with the r-values profile. For the strongly textured aluminum alloy AA 5042 (Numisheet Benchmark 2011), the experimental r-value distribution has two minima between the rolling and transverse direction data provided for this show that the r-value along the transverse direction (TD) is five times larger than the value corresponding to the rolling direction. Therefore, it is expected that there are more that the earing profile has more than four ears. The main objective of this paper is to assess whether a new form of CPB06ex2 yield function (Plunkett et al. (2008)) tailored for metals with no tension-compression asymmetry is capable of predicting more than four ears for this material.

Yoon, J. H.; Cazacu, O.

2011-08-01

437

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

438

Tissue concentrations of ofloxacin in the middle ear.  

PubMed

Twenty patients with chronic otitis media underwent tympanoplasty and were given an oral dose (two 200-mg tablets) of a new antibiotic, ofloxacin, three to seven hours before surgery. The study aimed to demonstrate satisfactory concentrations of ofloxacin in the middle ear as the basis for that agent's well-known clinical efficacy. The main pathogens responsible for bacterial infections in the ear, nose, and throat, especially those in the ear, are Staphylococcus aureus, Staphylococcus epidermidis, Pseudomonas aeruginosa, and alpha-streptococci. The chemotherapeutic agent used must reach adequate concentrations in the bone, mucous membrane, and serum. Mucous membrane, bone, and serum samples were obtained from the middle ear and examined for ofloxacin levels. The medication was also administered for a minimum of five days postoperatively. The average serum level of ofloxacin was 2.1 micrograms/ml, and the concentrations in both the mucous membrane and bone (2.2 micrograms/gm) were above the serum levels. The extent to which the concentrations at the possible site of infection exceeded the minimal inhibitory concentrations (MICs) for the pathogens was investigated at our clinic in 190 isolates. The study showed that, with the leading pathogens such as staphylococci, tissue concentrations were about four times higher than the MICs and that even Pseudomonas organisms were effectively inhibited. All patients had a satisfactory clinical response to therapy. The authors thus recommend a dosage of 400 mg of ofloxacin three to five hours prior to surgery for middle ear infection. PMID:3478138

Thorn, V

1987-01-01

439

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

440

Influences on clinical practice: the case of glue ear  

PubMed Central

A case study of clinical practice in children with glue ear is presented. The case is part of a larger project, funded by the North Thames Research and Development Programme, that sought to explore the part played by clinicians in the implementation of research and development into practice in two areas: adult asthma and glue ear in children. What is striking about this case is the differences found in every area of the analysis. That is, diversity was found in views about diagnosis and treatment of glue ear; the organisation of related services; and in the reported practice of our interviewees, both between particular groupings of clinical staff and within these groupings. The challenge inherent in the case is to go beyond describing the complexity and differences that were found, and look for patterns in the accounts of practice and tease out why such patterns may occur. PMID:10557674

Dopson, S.; Miller, R.; Dawson, S.; Sutherland, K.

1999-01-01

441

A Ubiquitous Blood Pressure Sensor Worn at the Ear  

NASA Astrophysics Data System (ADS)

Blood pressure (BP) measurement and BP control are important for the prevention of lifestyle diseases, especially hypertension, which can lead to more serious conditions, such as cardiac infarction and cerebral apoplexy. The purpose of our study is to develop a ubiquitous blood pressure sensor that is more comfortable and less disruptive of users' daily activities than conventional blood pressure sensors. Our developed sensor is worn at an ear orifice and measures blood pressure at the tragus. This paper describes the concept, configuration, and the optical and electronic details of the developed ear-worn blood pressure sensor and presents preliminary evaluation results. The developed sensor causes almost no discomfort and produces signals whose quality is high enough for detecting BP at an ear, making it suitable for ubiquitous usage.

Koizumi, Hiroshi; Shimada, Junichi; Uenishi, Yuji; Tochikubo, Osamu

2009-12-01

442

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

443

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

2014-01-01

444

Murine middle ear inflammation and ion homeostasis gene expression  

PubMed Central

Hypothesis Ion homeostasis genes are responsible for movement of ions and water in the epithelium of the middle ear. Background It is not well known to what extent disruption of ion homeostasis is a factor in the accumulation of middle ear fluid during otitis media. Methods Balb/c mice were transtympanically injected with heat-killed Hemophilus influenza bacteria. Untreated and saline injected mice were used as controls. Mice were euthanized at 6, 24, 72 hours and one week after injection, the bullae harvested, and total RNA isolated from the middle ear tissues. Ion homeostasis genes were analyzed with real-time qRT-PCR from the following gene families: Na+,K+-ATPase, claudins, K+ transport channels, epithelial Na+ channels, gap junctions, and aquaporins. Inflammatory genes were also analyzed to document inflammation. Results All inflammatory genes analyzed were significantly upregulated, more at 6 hours than at 24 hours, with the exception of VEGF and Mapk8. Most middle ear ion homeostasis genes experienced downregulation due to inflammation. This was most prominent in the aquaporin and Na+, K+-ATPase genes. Significant upregulation was seen in several genes in response to inflammation and saline independently. Conclusion The innate immune response to bacteria in the middle ear induces expression of several inflammatory genes. Coinciding with this inflammation is the downregulation of numerous ion homeostasis genes that are involved in ion and water transport and maintenance of tight junctions. This may explain the fluid accumulation within the middle ear seen with both acute and chronic otitis media. PMID:21307808

MacArthur, Carol J.; Hausman, Frances; Kempton, J Beth; Trune, Dennis R

2011-01-01

445

The thermally injured ear: a systematic approach to reconstruction.  

PubMed

The ears are special and unique structures that ordinarily are ignored during our daily routines. A thermal injury of relatively moderate proportions can irreparably alter their shape and appearance. Many reconstructive techniques have been garnered to restore these delicate structures. Herculean efforts will consistently fall short of these goals if tissue preservation is not in the forefront of our treatment protocol. Iontophoresis coupled with topical antimicrobial agents have been shown to ameliorate cartilage loss, allowing for delayed operative intervention, when more consistent results may be obtained. Early radical resection of ear soft tissue or structural cartilage should be an endeavor of last resort in all but a few instances, such as unresponsive suppurative chondritis. Segmental restoration of the injured ear allows for dismantling of the various parts to recreate the whole. A facsimile of the original is possible if the major visible distinguishing landmarks are salvaged. Flap resurfacing of exposed cartilage yields closer tissue match, color, and texture, and it affords a greater proclivity for survival than does graft closure. Sacrifice of the helical lip relegates the ear to that of a flat, less-than-optimal appearance. Tissue expansion coupled with cutaneous flap closure will usually preclude this situation. Skin grafting is a valuable tool in our armamentarium but should be used judiciously in situations where graft coverage is either necessary or desired to produce enhanced results. In such instances, the thickness of the graft must be considered, ranging from an almost translucent quality for the antihelix to that of a much thicker graft for the helix. Application and direction of the graft will be determined by the underlying surface contours. Until the reconstruction has been completed, burn patients and their families usually do not view the injured ear that has been snatched from the fires of adversity. The unveiling frequently effects a felicitous atmosphere, because they perceive a relatively normal-looking ear. The final result is all that matters. PMID:1633672

Rosenthal, J S

1992-07-01

446

Treatment of vascular malformation of the gastrointestinal tract  

NASA Astrophysics Data System (ADS)

Vascular malformations of the gastrointestinal tract are rare phenomenon. They are generally manifested by upper or lower GI - bleeding and do not resolve spontaneously. Emergency intervention is necessary. This paper reports on 10 cases, treated in the Dept. of Pediatric surgery of the FU Berlin, recorded from 1981 to 1999. We use the Nd:YAG laser 1064 nm, Fibertom 5100, Dornier, Germany, with a 600nm barefiber. Reduction in size of the hemangiomas and stop of the GI-bleeding was achieved in all cases.

Waldschmidt, Juergen; Stroedter, L.; Doede, T.; Kischkel, A.

2000-06-01

447

Urothelial mucosal malformation: a rare cause for ureteropelvic junction obstruction.  

PubMed

Ureteropelvic junction obstruction (UPJO) constitutes a significant cause of morbidity in children and exists in a wide range of severity and clinical manifestations. The cause of UPJO remains largely unknown except for a small group, in which crossing vessels have been considered etiological. Herein we describe a unique case in which intraluminal occlusion was the result of mucosal malformation, characterized by invaginated and branching urothelial epithelium present in the lamina propria. We believe that the present case is the first such description of this type of alteration. PMID:16808629

Huang, Weei-Yuarn; Olumi, Aria F; Rosen, Seymour

2006-01-01

448

Management of palatal vascular malformation using absolute ethanol sclerotherapy  

PubMed Central

Arteriovenous malformation is treated by variety of techniques over the years. Sclerotherapy is considered an effective and conservative technique for the treatment of benign vascular lesions and replaced the traditional role of surgical therapy, especially for the venous lesions that are surgically difficult or at inaccessible areas. Absolute ethanol was adopted as a new sclerosant agent for this complex form of venous defects to improve overall treatment results with acceptable morbidity and recurrence rates. Sclerotherapy has the advantage of no external scaring, low cost, and few complications in comparison to the surgical treatment. PMID:24765386

El-Hakim, Ibrahim; Alyamani, Ahmed

2011-01-01

449

Optoacoustic monitoring of laser correction of the ear shape  

SciTech Connect

Acoustic monitoring of a plastic operation for reshaping the porcine ear using radiation from a Ho:YAG laser was performed to control a change in the elasticity of the ear cartilage. Variations in the cartilage elasticity were controlled by changes in the amplitude and shape of an acoustic wave during the laser action. It is shown that the optoacoustic signal amplitude exponentially decreases at least by a factor of 2-2.5 at the moment of the cartilage reshaping caused by the action of radiation pulses from a Ho:YAG laser. (laser applications and other topics in quantum electronics)

Omel'chenko, A I; Sobol', E N; Sviridov, A P [Institute of Laser and Information Technologies, Russian Academy of Sciences, Troitsk, Moscow Region (Russian Federation); Harding, S; Jumel, K; Walker, R [University of Nottingham, National Centre for Macromolecular Hydrodynamics, Sutton Bonington, Leics (United Kingdom); Jones, N [University of Nottingham, Division of Otorhinolaryngology, Queens Medical Centre, Nottingham (United Kingdom)

2000-11-30

450

The Association of Middle Ear Effusion and Auditory Learning Disabilities in Children.  

ERIC Educational Resources Information Center

The study investigated the prevalence of middle ear effusion (conductive hearing loss) in 32 school aged children found to have auditory learning disabilities. Results indicated a systematic relationship between auditory learning disability and middle ear effusion. (DB)

Glass, Rita

1981-01-01

451

77 FR 35310 - Revisions to the Export Administration Regulations (EAR): Control of Military Training Equipment...  

Federal Register 2010, 2011, 2012, 2013

...Administration Regulations (EAR): Control of Military Training Equipment and Related Items...longer warrant control under Category IX (Military Training Equipment and Training) of...controlling under the EAR and its CCL military training equipment and related...

2012-06-13

452

Atmospheric air vs. normal middle ear gas: Effects on in vitro growth and collagen synthesis in normal middle ear fibroblasts  

Microsoft Academic Search

Summary  The present study was undertaken to quantitate the effects of atmospheric air and normal middle ear gas on cultured fibroblasts\\u000a obtained from normal rabbit middle ear mucosa. The cells were exposed to three different gas compositions: 7% O2:5% CO2:88% N2, 21% O2:5% CO2:74% N2, and 75% O2:5% CO2:20% N2. The growth was monitored by measuring the total content of cell

T. Ovesen; M. Gaihede; P. Scousboe; T. Ledet

1994-01-01

453

Congenital malformations in offspring of women with hyperglycemia first detected during pregnancy  

Microsoft Academic Search

Objectives: Our aim was to determine risk factors for congenital malformations in offspring of women with hyperglycemia first detected during pregnancy (i.e., women with gestational diabetes). Study Design: A total of 3743 pregnancies complicated by gestational diabetes mellitus delivered at >20 weeks of gestation were reviewed for the presence of congenital malformations diagnosed before hospital discharge. Anomalies were categorized as

Ute M. Schaefer; Giulana Songster; Anny Xiang; Kathleen Berkowitz; Thomas A. Buchanan; Siri L. Kjos

1997-01-01

454

Screening for Systemic Manifestations of Vascular Malformations in Patients With Hereditary Haemorrhagic Telangiectasia (Osler Disease)  

Microsoft Academic Search

Hereditary haemorrhagic telangiectasia (Rendu-Osler-Weber syndrome) is a disease characterized by systemic vascular malformations. Typical clinical manifestations are recurrent epistaxis and telangiectases of the skin and the mucous membranes. The syndrome is furthermore characterized by its hereditary aspect. The disease seems to be much more complicated than previously thought, mainly because of the accompanying vascular malformations in vital organs, like the

Ana Cerra Pohl; Jochen Alfred Werner; Benedikt Josef Folz

455

A review of contemporary options for medical management of hemangiomas, other vascular tumors, and vascular malformations.  

PubMed

Vascular anomalies include vascular tumors and vascular malformations. With growing pharmacologic options and parallels to cancer treatment and biology, the hematologist-oncologist has assumed a more prominent role in clinical care and research relating to these diagnoses. This also is a growing area for targeted therapies and drug repositioning. We performed a review of contemporary options for medical management of these lesions. PubMed was searched for "vascular anomaly", "hemangioma", "vascular malformation", "arteriovenous malformation", "capillary malformation", "cerebral cavernous malformation", "lymphatic malformation", and "venous malformation", each with "drug treatment" as a modifier. Manuscripts were reviewed to verify diagnoses, indications for treatment, dose-schedules, evidence of effectiveness, toxicities, and mechanisms of action. ClinicalTrials.gov also was reviewed for relevant trials. More than 20 agents were identified which have been used to treat vascular anomalies. Rigorous studies are lacking for many of these. The rarity of these tumors has limited development of medical approaches to treatment. Cooperative group trials will be needed to prove the effectiveness of drugs which have shown promise in cases and small series. The observant clinician remains a powerful tool for identifying potential new treatments for vascular tumors and malformations. PMID:23665062

Blatt, Julie; McLean, Thomas W; Castellino, Sharon M; Burkhart, Craig N

2013-09-01

456

Exceptional Multiplicity of Cerebral Arteriovenous Malformations Associated with Hereditary Hemorrhagic Telangiectasia (Osler Weber-Rendu Syndrome)  

Microsoft Academic Search

PURPOSE: To describe the clinical and imaging features of seven patients with hereditary hem- orrhagic telangiectasia and an exceptional number of cerebral arteriovenous malformations (AVMs). METHODS: One hundred thirty-six patients from a dedicated hereditary hemorrhagic telangiectasia clinic were screened systematically for cerebral AVMs by means of MR imaging. Thirty-one were found to have abnormalities suggestive of a vascular malformation. Eighteen

Christopher M. Putman; John C. Chaloupka; Robert K. Fulbright; Issam A. Awad; Robert I. White; Pierre B. Fayad

457

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 Ĺberg

1999-01-01

458

Treatment of the Chiari malformation by Drs. H.E. James and A. Brant  

Microsoft Academic Search

the secondary distortion, atrophy, or fibrosis. A common anomaly in Chiari types I and II is hypoplasia of the posterior fossa, which is usually not accompanied by histological malformations in the brain. We suggest that the central nervous system anomalies in Chiari anomalies types I and II are not the primary CNS malformation but only an adaptation to the hypoplasia

Akira Hori; A. Brant

2002-01-01

459

A cloverleaf skull syndrome probably of Beare-Stevenson type associated with Chiari malformation  

Microsoft Academic Search

A case of cloverleaf skull (CLS) syndrome with Chiari malformation was reported. The patient developed congenital hydrocephalus, upper airway obstruction and breath holding spells. Ventriculo-peritoneal shunt improved the hydrocephalus, but the patient progressively developed apneic episodes in spite of intubation. Brain magnetic resonance imaging (MRI) disclosed severe Chiari malformation. Laminectomy of the cervical vertebrae and craniectomy at the occipital bone

Shuichi Ito; Kiyoshi Matsui; Etsuro Ohsaki; Akiko Goto; Kimiyo Takagi; Mitsuhiko Koresawa; Susumu Ito; Kenichi Sekido; Masami Suzuki; Katsuyuki Torikai; Noriko Aida

1996-01-01

460

The Effect of Surgery for Split Spinal Cord Malformation on Neurologic and Urologic Function  

Microsoft Academic Search

The split spinal cord malformation (SSCM) is an occult spinal dysraphism which causes tethering of the spinal cord. We performed a retrospective analysis of 15 patients who had split cord malformations (without associated open neural tube defect) who underwent both pre- and postoperative urodynamic studies (UDS) in order to determine if a significant percentage of these patients, even in the

Mark R. Proctor; Stuart B. Bauer; R. Michael Scott

2000-01-01

461

Clinical Features Microcephaly-Capillary Malformation syndrome [MICCAP, OMIM#614261] is characterized by severe progressive  

E-print Network

4/13 Clinical Features Microcephaly-Capillary Malformation syndrome [MICCAP, OMIM#614261] is characterized by severe progressive microcephaly, early-onset refractory epilepsy, profound developmental delay Analysis for Microcephaly-Capillary Malformation Syndrome #12;4/13 References: 1. Carter MT, Geraghty MT

Das, Soma

462

Radionuclide blood pool scintigraphy in a child with intestinal arteriovenous malformation (juvenile angiodysplasia)  

Microsoft Academic Search

Arteriovenous (AV) malformation or angiodysplasia of the gastrointestinal tract is a very rare cause of bleeding in children. These lesions are congenital anomalies and thought to be of hamartomatous origin. We report on a 4-year-old child with an AV malformation of the distal ileum treated by local resection. A brief review of the relevant literature is included. The importance of

I. Garty; L. Siplovich; J. Horowitz; D. Miron; A. Verstandig; M. Dharan

1991-01-01

463

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

464

Hepatic vascular malformations in hereditary hemorrhagic telangiectasia: Doppler sonographic screening in a large family  

Microsoft Academic Search

Background\\/Aims: The prevalence of hepatic vascular malformations in hereditary hemorrhagic telangiectasia has been estimated in the literature on clinical criteria, thus giving unreliable data. In our study the presence of hepatic vascular malformations in hereditary hemorrhagic telangiectasia was evaluated in a large Italian family by using Doppler sonography as screening technique. Doppler sonographic findings were compared to computed tomography and

Elisabetta Buscarini; Luigi Buscarini; Cesare Danesino; Mauro Piantanida; Giuseppe Civardi; Pietro Quaretti; Sandro Rossi; Michele Di Stasi; Matteo Silva

1997-01-01

465

Clinical, Genetic and Environmental Factors Associated with Congenital Vertebral Malformations  

PubMed Central

Congenital vertebral malformations (CVM) pose a significant health problem because they can be associated with spinal deformities, such as congenital scoliosis and kyphosis, in addition to various syndromes and other congenital malformations. Additional information remains to be learned regarding the natural history of congenital scoliosis and related health problems. Although significant progress has been made in understanding the process of somite formation, which gives rise to vertebral bodies, there is a wide gap in our understanding of how genetic factors contribute to CVM development. Maternal diabetes during pregnancy most commonly contributes to the occurrence of CVM, followed by other factors such as hypoxia and anticonvulsant medications. This review highlights several emerging clinical issues related to CVM, including pulmonary and orthopedic outcome in congenital scoliosis. Recent breakthroughs in genetics related to gene and environment interactions associated with CVM development are discussed. The Klippel-Feil syndrome which is associated with cervical segmentation abnormalities is illustrated as an example in which animal models, such as the zebrafish, can be utilized to provide functional evidence of pathogenicity of identified mutations. PMID:23653580

Giampietro, P.F.; Raggio, C.L.; Blank, R.D.; McCarty, C.; Broeckel, U.; Pickart, M.A.

2013-01-01

466

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

467

PDCD10 Gene Mutations in Multiple Cerebral Cavernous Malformations  

PubMed Central

Cerebral cavernous malformations (CCMs) are vascular abnormalities that may cause seizures, intracerebral haemorrhages, and focal neurological deficits. Familial form shows an autosomal dominant pattern of inheritance with incomplete penetrance and variable clinical expression. Three genes have been identified causing familial CCM: KRIT1/CCM1, MGC4607/CCM2, and PDCD10/CCM3. Aim of this study is to report additional PDCD10/CCM3 families poorly described so far which account for 10-15% of hereditary cerebral cavernous malformations. Our group investigated 87 consecutive Italian affected individuals (i.e. positive Magnetic Resonance Imaging) with multiple/familial CCM through direct sequencing and Multiplex Ligation-Dependent Probe Amplification (MLPA) analysis. We identified mutations in over 97.7% of cases, and PDCD10/CCM3 accounts for 13.1%. PDCD10/CCM3 molecular screening revealed four already known mutations and four novel ones. The mutated patients show an earlier onset of clinical manifestations as compared to CCM1/CCM2 mutated patients. The study of further families carrying mutations in PDCD10/CCM3 may help define a possible correlation between genotype and phenotype; an accurate clinical follow up of the subjects would help define more precisely whether mutations in PDCD10/CCM3 lead to a characteristic phenotype. PMID:25354366

Cigoli, Maria Sole; Avemaria, Francesca; De Benedetti, Stefano; Gesu, Giovanni P.; Accorsi, Lucio Giordano; Parmigiani, Stefano; Corona, Maria Franca; Capra, Valeria; Mosca, Andrea; Giovannini, Simona; Notturno, Francesca; Ciccocioppo, Fausta; Volpi, Lilia; Estienne, Margherita; De Michele, Giuseppe; Antenora, Antonella; Bilo, Leda; Tavoni, Antonietta; Zamponi, Nelia; Alfei, Enrico; Baranello, Giovanni; Riva, Daria; Penco, Silvana

2014-01-01

468

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

469

Surgical Management of Patients with Chiari I Malformation  

PubMed Central

Chiari malformations (CMs) constitute a variety of four mainly syndromes (I, II, III, and IV), which describe the protrusion of brain tissue into the spinal canal through the foramen magnum. These malformations frequently occur in combination with other pathological entities such as myelomeningocele, hydrocephalus, and/or hydrosyringomyelia. The recent improvement of imaging techniques has increased not only the rate of CM diagnosis but also the necessity for its early treatment. Several different surgical techniques have been employed in the treatment of patients with symptomatic CM-I. In our current study, a systematic and critical review of the pertinent literature was made for identifying the most commonly employed surgical procedures in the management of these patients. Emphasis was given in outlining the advantages and disadvantages of each surgical approach. Moreover, an attempt was made for defining those parameters that may be prognostic factors for their surgical outcome. There is a consensus that surgical treatment is reserved only for symptomatic patients with CM-I. It has also been postulated that early surgically intervention is usually associated with better outcome. Despite the large number of previously published clinical series, further clinical research with large-scale studies is necessary for defining surgical treatment guidelines in these patients. PMID:22811732

Siasios, John; Kapsalaki, Eftychia Z.; Fountas, Kostas N.

2012-01-01

470

The Alimentary Tract Malformations in the Rabbit Thalidomide Embryopathy  

PubMed Central

Among 906 thalidomide exposed young from 6 different rabbit strains, 141 major malformations involving the alimentary tract were found. All but 4 of the defects can confidently be regarded as a drug effect. The lesions occurred in the derivatives of the foregut, hindgut and umbilicus. Foregut anomalies (68 specimens) comprised hiatus hernia, megaoesophagus, prepyloric notching of the greater curvature of the stomach and deficient septum formation between oesophagus and trachea. In many rabbits all 4 lesions were present together. Hindgut malformations (61 specimens) were made up of rectal atresia and a small number of diverticula and/or saccular dilatations of the terminal colon, some in company with atresia. The 32 examples of atresia which were examined in detail had a coexistent fistula extending to the urethra or the anus. Omphalocoele (6 specimens in one strain) was the umbilical defect. It is suggested that some of the lesions result from impeded septum formation in the laryngotracheal grouve and the cloaca. ImagesFigs. 7-12Figs. 25-30Figs. 13-20Figs. 1-6Figs. 21-24 PMID:5429073

Vickers, T. H.

1970-01-01