Sample records for ear malformation cardiospondylocarpofacial

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

    PubMed

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

    2010-03-01

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

  2. COCHLEAR IMPLANTATION IN PATIENTS WITH INNER EAR MALFORMATIONS

    Microsoft Academic Search

    M. H. Khalessi; M. Motesaddi Zarandi; P. Borghei; S. Abdi

    Performing cochlear implantation in patients with inner ear malformation has always been a matter of dispute. This study was designed to analyze the operative findings, complications, and post- operative performance of patients with inner ear anomalies who underwent cochlear implantation. Six patients with inner ear malformations underwent implantation in our academic tertiary referral center from 1997 to 2002. The average

  3. Patterns of anomalies in children with malformed ears.

    PubMed

    Rapin, I; Ruben, R J

    1976-10-01

    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

  4. Ear malformation and hearing loss in patients with Treacher Collins syndrome.

    PubMed

    Pron, G; Galloway, C; Armstrong, D; Posnick, J

    1993-01-01

    Although the hearing loss of patients with Treacher Collins syndrome is well documented, few studies have reported jointly on their hearing loss and ear pathology. This paper reports on the hearing loss and computerized tomography (CT) assessments of ear malformations in a large pediatric series of patients with Treacher Collins. Of the 29 subjects assessed by the Craniofacial Program between 1986 and 1990, paired audiologic and complete CT assessments were available for 23 subjects. The external ear canal abnormalities were largely symmetric, either bilaterally stenotic or atretic. In most cases, the middle ear cavity was bilaterally hypoplastic and dysmorphic, and ossicles were symmetrically dysmorphic or missing. Inner ear structures were normal in all patients. The majority of patients had a unilateral or bilateral moderate or greater degree of hearing loss and almost half had an asymmetric hearing loss. The hearing loss of all subjects was conductive, except for three whose loss was bilateral mixed. Two types of bilaterally symmetric hearing loss configurations, flat and reverse sloping, were noted. Conductive hearing loss in patients with Treacher Collins is mainly attributable to their middle ear malformations, which are similar for those of patients with malformed or missing ossicles. PMID:8418881

  5. Computer assisted orthosis design system for malformed ears-automatic shape modification method for preventing excessive corrective force

    Microsoft Academic Search

    Akihiko Hanafusa; T. Isomura; Y. Sekiguchi; Hajime Takahashi; Takeyoshi Dohi

    2000-01-01

    Most malformed ears of neonates can be treated by mounting a suitably shaped orthosis. However, it needs much experience to make a orthosis that produces adequate corrective force. Especially, excessive force may cause a decubitus-like inflammation on the auricle. The authors are studying a computer assisted system to support the orthosis design. They have already developed a fundamental design and

  6. JS-X syndrome: A multiple congenital malformation with vocal cord paralysis, ear deformity, hearing loss, shoulder musculature underdevelopment, and X-linked recessive inheritance.

    PubMed

    Hoeve, Hans L J; Brooks, Alice S; Smit, Liesbeth S

    2015-07-01

    We report on a family with a not earlier described multiple congenital malformation. Several male family members suffer from laryngeal obstruction caused by bilateral vocal cord paralysis, outer and middle ear deformity with conductive and sensorineural hearing loss, facial dysmorphisms, and underdeveloped shoulder musculature. The affected female members only have middle ear deformity and hearing loss. The pedigree is suggestive of an X-linked recessive inheritance pattern. SNP-array revealed a deletion and duplication on Xq28 in the affected family members. A possible aetiology is a neurocristopathy with most symptoms expressed in structures derived from branchial arches. PMID:25998214

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

    PubMed

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

    2012-11-01

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

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

    PubMed Central

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

    2012-01-01

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

  9. Amphibian malformations

    USGS Publications Warehouse

    National Wildlife Health Center

    1998-01-01

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

  10. Ear Disorders

    MedlinePLUS

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

  11. Ear wax

    MedlinePLUS

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

  12. Elephant ear

    MedlinePLUS

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

  13. 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 ...

  14. Anorectal malformations

    PubMed Central

    Gangopadhyay, Ajay Narayan; Pandey, Vaibhav

    2015-01-01

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

  15. Study of the ear in anencephaly

    Microsoft Academic Search

    D. K. Banerjee; S. K. Basu; Asok Dandapath; B. M. Choddhury

    1980-01-01

    The ears of 8 anencephalics were studied. No gross macroscopic abnormality in the development of the external, middle and\\u000a internal ears were detected except that the internal auditory canals appeared more of a furrow with a bony ledge at its base.\\u000a There was a malformed long process of incus with abnormal disposition of the round and oval windows below and

  16. Ear wax

    PubMed Central

    2008-01-01

    Introduction Ear wax only becomes a problem if it causes a hearing impairment or other ear-related symptoms. Ear wax is more likely to accumulate and cause a hearing impairment when normal extrusion is prevented — for example, by the use of hearing aids, or by the use of cotton buds to clean the ears. Ear wax can visually obscure the ear drum, and may need to be removed for diagnostic purposes. Methods and outcomes We conducted a systematic review and aimed to answer the following clinical question: What are the effects of methods to remove ear wax? We searched: Medline, Embase, The Cochrane Library, and other important databases up to June 2007 (Clinical Evidence reviews are updated periodically; please check our website for the most up-to-date version of this review). We included harms alerts from relevant organisations such as the US Food and Drug Administration (FDA) and the UK Medicines and Healthcare products Regulatory Agency (MHRA). Results We found nine systematic reviews, RCTs, or observational studies that met our inclusion criteria. We performed a GRADE evaluation of the quality of evidence for interventions. Conclusions In this systematic review, we present information relating to the effectiveness and safety of the following interventions: ear syringing; manual removal (other than ear syringing); and wax softeners (alone or prior to syringing). PMID:19450340

  17. Renal ultrasonography not required in babies with isolated minor ear anomalies

    Microsoft Academic Search

    S A Deshpande; H Watson

    2006-01-01

    Aim: To determine whether infants with isolated minor anomalies of the external ear are at increased risk of renal malformations.Methods: Consecutive infants with isolated minor anomalies of the external ear (preauricular skin tags, preauricular sinuses, ear pits, and misshapen pinnae) were offered renal ultrasonography by experienced sonographers over a 41 month period. The prevalence of renal anomalies in such infants

  18. Genetics Home Reference: Capillary malformation-arteriovenous malformation syndrome

    MedlinePLUS

    ... literature OMIM Genetic disorder catalog Conditions > Capillary malformation-arteriovenous malformation syndrome (often shortened to CM-AVM ) On this ... August 2011 What is CM-AVM? Capillary malformation-arteriovenous malformation syndrome (CM-AVM) is a disorder of the ...

  19. Cerebral Cavernous Malformation

    MedlinePLUS

    ... medical institutions across the country. Much of this research focuses on finding better ways to prevent, treat, and ultimately cure CCM. NIH Patient Recruitment for Cavernous Malformation Clinical Trials At NIH Clinical Center Throughout the U.S. ...

  20. Management of Vascular Malformations

    PubMed Central

    Houbara, Seiji; Hirano, Akiyoshi

    2014-01-01

    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. PMID:25289321

  1. Congenital spinal lipomatous malformations

    Microsoft Academic Search

    Natarajan Muthukumar

    2009-01-01

    Background  To report this author’s experience with patients with a congenital spinal lipomatous malformation with special emphasis on\\u000a variations in clinical presentation, operative findings, and outcome based on the classification scheme proposed in the first\\u000a part of this two part article.\\u000a \\u000a \\u000a \\u000a Methods  From January 1995 to July 2005, 80 patients with a congenital spinal lipomatous malformation were treated. All patients underwent\\u000a routine

  2. Peripheral Vascular Malformations

    Microsoft Academic Search

    Peter Littler; Peter Rowlands

    \\u000a Peripheral vascular malformations are uncommon lesions and are thought to be due to a focal persistence of primitive vascular\\u000a elements.\\u000a \\u000a \\u000a Vascular malformations are not neoplastic. They are subdivided into high-flow and low-flow lesions. High-flow lesions are\\u000a arterial; low-flow lesions are venous, capillary, or lymphatic.\\u000a \\u000a \\u000a \\u000a It is important that these lesions are evaluated and treated by a specialist with experience in

  3. [Multiple intracranial arteriovenous malformation].

    PubMed

    Gelabert-González, Miguel; Santin-Amo, José María; Román-Pena, Paula; Vázquez Herrero, Fernando

    2015-01-01

    Multiple cerebral arteriovenous malformations (AVMs) are thought to be exceedingly rare lesions and have usually been reported as single cases. The incidence of multiple cerebral AVMs in major series ranges from 0.3% to 9% and, in the majority of cases, these malformations are associated with other vascular anomalies of the brain or soft tissues. We report a 62-year-old woman that presented with a left temporal haemorrhage. Angiography showed 3 AVMs located in the left temporal lobe, left cerebellar hemisphere and right temporal lobe. The lesions were treated with radiosurgery. PMID:25596643

  4. Chiari Malformation: Symptoms

    MedlinePLUS

    ... may cause tinnitus (ringing in the ears), depth perception, running into walls and tripping. 8 © 2012 C&S ... Of or pertaining to the mental processes of perception, memory, judgment, and reasoning 10 © 2012 C&S Patient ...

  5. Cardiovascular Malformations Among Preterm Infants

    Microsoft Academic Search

    Kirsty Tanner; Nilofer Sabrine; Christopher Wren

    2010-01-01

    Objective. Preterm birth and cardiovas- cular malformations are the 2 most common causes of neonatal and infant death, but there are no published population-based reports on the relationship between them. We undertook this study to determine the preva- lence and spectrum of cardiovascular malformations in a preterm population, the prevalence of prematurity among infants with cardiovascular malformations, and the influence

  6. Giant lymphatic malformation

    Microsoft Academic Search

    Sharon Tamir; Ronit Agid; Doron Halperin; Jean-Yves Sichel

    2006-01-01

    Lymphatic malformations (LM) are rare, often presenting as congenital abnormal growth of lymphatic vessels diagnosed most commonly in the pediatric population.We report a case of an adult women presenting with a giant LM of the neck and mediastinum reaching the upper part of the retroperitoneum, manifesting itself as bilateral supraclavicular neck masses accompanied with an abdominal mass.

  7. 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 ...

  8. Ear drainage culture

    MedlinePLUS

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

  9. Lymphatic malformations: diagnosis and management.

    PubMed

    Elluru, Ravindhra G; Balakrishnan, Karthik; Padua, Horacio M

    2014-08-01

    Lymphatic malformations are benign vascular lesions that arise from embryological disturbances in the development of the lymphatic system. They encompass a wide spectrum of related abnormalities, including cystic lymphatic lesions, angiokeratoma, lymphatic malformations that occur in bones (Gorham-Stout Syndrome), lymphatic and chylous leak conditions, and lymphedema. This article will focus only on lymphatic malformation mass lesions, whereas other related disease entities will be covered elsewhere in this journal issue. Lymphatic malformations occur frequently in lymphatic-rich areas such as the head and neck region, but they can also be found on any anatomical site in the body. In general, lymphatic malformations are categorized into macrocystic, microcystic, or combined depending on the size of the cysts contained within the lesion. Lymphatic malformations can cause both deformation of the anatomical site involved and functional deficits. The goal of this article is to discuss the etiology, epidemiology, treatment modalities, and comorbidities associated with lymphatic malformations. PMID:25241095

  10. Arteriovenous malformation management

    Microsoft Academic Search

    Wayne F. Yakes; Plinio Rossi; Henk Odink

    1996-01-01

    Arteriovenous malformations (AVM) are rare vascular lesions that can present with a myriad of clinical presentations. In our\\u000a institutions, initial workup consists of a clinical exam, color Doppler imaging, and magnetic resonance imaging. After the\\u000a initial nonivasive workup, arteriography, at times closed system venography, and ethanol endovascular repair of the AVM is\\u000a performed under general anesthesia. Depending on the size

  11. [Middle ear physiology].

    PubMed

    Ayerbe, I; Négrevergne, M; Ucelay, R; Sanchez Fernandez, J M

    1999-01-01

    The middle ear forms part of the sound transformer mechanism, together with the outer ear and the conducting system of the inner ear. An intermediate sensory organ, sensitive to acoustic vibration, and linked to the inner ear, the middle ear made its appearance during the period of adaptation of marine creatures to a terrestrial habitat; its presence is therefore a phylogenetic requirement. It is classical to ascribe three functions to the middle ear: the transmission of acoustic vibrations from the tympanic membrane to the cochlea, impedance matching between the air in the external auditary meatus and the labyrinthine fluids, and protection of the inner ear by means of the acoustic reflex. If the classical mechanical explanation has been able to explain its function, the conceptualization of its physiology in terms of energy allows an even better understanding, as well as providing and explanation for the paradoxes which arise in clinical practice when the classical model is used. PMID:10769562

  12. Arteriovenous Malformation Management

    SciTech Connect

    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

    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.

  13. Brainstem cavernous malformations.

    PubMed

    Petr, O; Lanzino, G

    2015-09-01

    Of all cavernous malformations (CMs), 4% to 35% are found in the brainstem accounting for 13% of vascular malformations of the posterior fossa. The annual risk of hemorrhage associated with a CM with no history of a previous hemorrhagic episode is very low ranging from 0.6% to 1.1% per year. However, the risk of recurrent hemorrhage after a presenting bleed is significantly higher. There is a correlation between the extent of persistent neurological deficits and the number of recurrent hemorrhages as rehemorrhage increases the rate and severity of neurological deficits. Neurological deficits often improve after a hemorrhagic event spontaneously and sometimes resolve completely. The indication for surgery in patients with brainstem CMs is controversial. Over the years, we have taken a more cautious stance and we often recommend observation in patients after a single symptomatic bleed as most patients return to a good level of functioning after a single bleed. Surgery is recommended for more aggressive lesions usually after a recurrent bleed. In general, given the very low risk of bleeding from truly asymptomatic lesions, surgery should not be considered in these patients. For symptomatic lesions which have presented with hemorrhage, the decision of whether or not to proceed with surgical resection is related to the risk of surgery, patient's disposition and perceived risk of rebleeding. Favorable outcome can be achieved through surgical resection after an appropriate selection of the patients and thorough preoperative surgical planning. PMID:25943871

  14. Anencephaly and its Associated Malformations

    PubMed Central

    Meshram, Pritee Madan; Hattangdi, Shanta Sunil

    2014-01-01

    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

  15. Middle ear promontory osteoma.

    PubMed

    Toro, Paula Cruz; Castillo, Angela Callejo; Moya Martínez, Rafael; Domènech Juan, Iván

    2014-01-01

    Osteomas are benign lesions of the lamellar bone that within the temporal region are common in the external ear canal. Osteomas of the middle ear are extremely rare and until now there are only 14 reported cases. They usually present with conductive hearing loss while others are asymptomatic and diagnosed incidentally. PMID:25087466

  16. Natural ear rings.

    PubMed

    Sabaretnam, Mayilvaganan; Virupakshaiah, Akash; Baruah, Rishiraj

    2015-05-01

    Ear lobe keloids are common following ear piercing and these lesions are conspicuous and cosmetically unappealing. Multiple methods including surgery, radiotherapy, anti mitotic agents, silicone sheet, pressure clips, and cryotherapy have been used. The Challenge is to have a good cosmetic outcome with minimal recurrence. PMID:25984317

  17. Natural ear rings

    PubMed Central

    Sabaretnam, Mayilvaganan; Virupakshaiah, Akash; Baruah, Rishiraj

    2015-01-01

    Key Clinical Message Ear lobe keloids are common following ear piercing and these lesions are conspicuous and cosmetically unappealing. Multiple methods including surgery, radiotherapy, anti mitotic agents, silicone sheet, pressure clips, and cryotherapy have been used. The Challenge is to have a good cosmetic outcome with minimal recurrence. PMID:25984317

  18. Ear tube insertion - series (image)

    MedlinePLUS

    Ear tube insertion is recommended for: fluid in the middle ear (present for more than 12 weeks) recurrent ear ... the accumulated fluid is suctioned out. A small tube is inserted through the incised eardrum in order ...

  19. Ear - blocked at high altitudes

    MedlinePLUS

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

  20. Controversies in Chiari I malformations

    PubMed Central

    Baisden, Jamie

    2012-01-01

    Background: The diagnosis and management of Chiari I malformations (CMI) remains controversial, particularly since it is often an incidental finding on cervical MR scans performed for neck pain and/or headaches. Recently, some surgeons “over-operated” on asymptomatic patients with Chiari I malformations, or even on those without the requisite radiographic diagnostic features for Chiari I malformations: unfortunately, only a subset were admonished for indiscriminate surgery. Nevertheless, when this hindbrain malformation is truly symptomatic, contributing to impaired cerebrospinal fluid (CSF) circulation, various valid surgical management strategies may be adopted. Methods: This review focuses on the current literature regarding the clinical presentation, diagnosis, and surgical management of Chiari I malformation. Variations in the surgical technique are also presented and critiqued. Results: The recommended treatment for Chiari I malformations I consists of decompressive suboccipital craniectomy and duroplasty when abnormal cine-flow MRI is observed preoperatively and blockage of CSF flow persists intraoperatively despite bony decompression. Conclusions: Controversy continues regarding the optimal surgical technique to address Chiari malformations I. Proper diagnostic studies and patient selection are needed to optimize patient outcomes, while avoiding unnecessary surgical procedures. PMID:22905329

  1. Imaging of congenital lung malformations.

    PubMed

    Chowdhury, Moti M; Chakraborty, Subhasis

    2015-08-01

    Congenital lung malformations are a heterogeneous group of anomalies that involve the lungs and tracheobronchial tree (congenital airway pulmonary malformation, bronchial atresia, bronchogenic cyst, congenital lobar overinflation, pulmonary cyst, hamartoma, pulmonary isomerism and azygous lobe), vascular abnormalities (arteriovenous malformations, anomalous pulmonary venous return, pulmonary artery sling, interrupted pulmonary artery, pulmonary varix, pulmonary vein stenosis and pulmonary lymphangiectasia), or frequently both entities (pulmonary sequestration, pulmonary maldevelopment and scimitar syndrome). Advances in diagnostic imaging (including sonography, multi-detector computer tomography, magnetic resonance imaging and angiography) have increased their detection during both antenatal and postnatal periods, and radiological characterisation, which in turn influence patient counselling and management stratification. An educational illustration of the clinical application in characterisation of these malformations is presented. PMID:26051049

  2. Ear infection - acute

    MedlinePLUS

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

  3. EARS STT Overview Phil Woodland

    E-print Network

    Hain, Thomas

    EARS STT Overview Phil Woodland February 4th 2004 Cambridge University Engineering Department EARS PI Meeting: Feb 4th 2004 #12;Woodland: STT Overview Outline · STT in EARS ­ Teams & the Super Team University Engineering Department EARS PI Meeting: Feb 4th 2004 1 #12;Woodland: STT Overview ­ Martigny

  4. EARS STT Overview Phil Woodland

    E-print Network

    Hain, Thomas

    EARS STT Overview Phil Woodland February 4th 2004 Cambridge University Engineering Department EARS PI Meeting: Feb 4th 2004 Woodland: STT Overview Outline . STT in EARS -- Teams & the Super Team University Engineering Department EARS PI Meeting: Feb 4th 2004 1 #12; Woodland: STT Overview -- Martigny

  5. Malformations of cortical development

    PubMed Central

    Pang, Trudy; Atefy, Ramin; Sheen, Volney

    2012-01-01

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

  6. Pulmonary Arteriovenous Malformations

    PubMed Central

    2014-01-01

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

  7. Pulmonary arteriovenous malformations.

    PubMed

    Shovlin, Claire L

    2014-12-01

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

  8. Intralesional radiofrequency in venous malformations.

    PubMed

    Garg, S; Kumar, S; Singh, Y B

    2015-03-01

    Venous malformations are usually asymptomatic and managed conservatively. Treatment, in the form of laser, sclerotherapy, or resection, is needed only if lesions present with symptoms or cosmetic deformity. The aim of this study was to find out how effective radiofrequency ablation was in patients with incomplete or unsatisfactory resolution of a venous malformation after an intralesional injection of bleomycin. During the 5 year period 2008-2012, we organised a prospective, clinical study at a tertiary care centre. Patients were selected from the outpatient department of the Lady Hardinge Medical College and associated hospitals, New Delhi, India. Five patients with venous malformations were treated by intralesional injection of bleomycin in a dose of 0.5U/kg body weight, which was repeated every 2 weeks for a total of 8 injections. They then had multiple intralesional radiofrequency ablation every 2 months until a satisfactory outcome was achieved. After the initial 8 doses the reduction in the size of the lesions was minimal (less than 50%). After 2-4 applications of radiofrequency ablation there was appreciable reduction in the size of the lesions (about 80%) with good functional and cosmetic outcomes. Radiofrequency ablation is an effective adjunct for patients with venous malformations of the head and neck that have not responded satisfactorily to intralesional injection of bleomycin. To our knowledge radiofrequency ablation after intralesional injection of bleomycin has not previously been described as a treatment for venous malformations. PMID:25554592

  9. Intracranial vascular malformations: MR and CT imaging

    SciTech Connect

    Kucharczyk, W.; Lemme-Pleghos, L.; Uske, A.; Brant-Zawadzki, M.; Dooms, G.; Norman, D.

    1985-08-01

    Twenty-four patients with 29 cerebrovascular malformations were evaluated with a combination of computed tomography (CT), angiography, and magnetic resonance (MR) imaging. Characteristics of the malformations on MR images were reviewed retrospectively, and a comparative evaluation of MR and CT images was made. Of 14 angiographically evident malformations, 13 intra-axial lesions were detected on both CT and MR images, and one dural malformation gave false-negative results on both modalities. The appearance of parenchymal lesions on MR images closely mirrored characteristic CT findings. Angiographically evident malformations have a highly characteristic appearance on MR images. MR may be more sensitive than CT in the detection of small hemorrhagic foci associated with cryptic arteriovenous malformations and may add specificity in the diagnosis of occult malformations in some cases, but MR is less sensitive than CT for the detection of small calcified malformations.

  10. [Surgical treatment of lymphatic malformations].

    PubMed

    Lavie, A; Desouches, C; Casanova, D; Bardot, J; Magalon, G

    2006-01-01

    Lymphatic malformations remain a therapeutic challenge. Many treatments by the past led to poor success. The wide variety of clinical presentations makes it difficult to outline specific management programs. Often, diagnosis or complication circumstances as infection, bleeding, airway obstruction, or handicap force acting. Thus, a specific follow-up joined to a multidisciplinary analysis are the key of well-planned surgery. Through our experience and a review of literature, we will describe principles of lymphatic malformations surgery and specific technics for specific locations. PMID:17007979

  11. Congenital malformations associated with anencephaly and iniencephaly

    Microsoft Academic Search

    T J David; A Nixon

    1976-01-01

    The necropsy reports of 294 cases of anencephaly and 50 cases of iniencephaly have been examined, and a tubulated list of associated malformations produced. Cases were divided by sex and the presence or absence of spina bifida. Forty-one per cent of the series had other malformations, and other malformations were more common in those cases with spina bifida than in

  12. The association of venous and cavernous malformations

    Microsoft Academic Search

    D. Rigamonti; R. F. Spetzler

    1988-01-01

    Summary Four cases of venous malformation associated with a cavernous malformation are reported. Because cavernous malformations are often angiographically occult and do not have a characteristic appearance on computed tomography (CT), they are seldom recognized preoperatively and may be missed if the surgical specimen is not carefully reviewed.

  13. The red ear syndrome

    PubMed Central

    2013-01-01

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

  14. Arteriovenous malformations in Cowden syndrome.

    PubMed

    Turnbull, M M; Humeniuk, V; Stein, B; Suthers, G K

    2005-08-01

    Cowden syndrome (OMIM No 158350) is a pleomorphic, autosomal dominant syndrome characterised by hamartomas in tissues derived from the endoderm, mesoderm, and ectoderm. It is caused by germline mutations in the PTEN gene and is allelic to the Bannayan-Riley-Ruvalcaba and Lhermitte-Duclos syndromes. The three syndromes are defined on clinical grounds but there is overlap in their definitions. The clinical features include trichilemmomas, verrucose lesions of the skin, macrocephaly, intellectual disability, cerebellar gangliocytoma, thyroid adenomas, fibroadenomas of the breast, and hamartomatous colonic polyps. Cutaneous haemangiomas are occasionally noted. Malignancies often arise in the affected tissues. Visceral arteriovenous malformations are a recognised component of the Bannayan-Riley-Ruvalcaba syndrome but have been reported rarely in Cowden syndrome. A family is described with a clinical diagnosis of Cowden syndrome, a familial frameshift mutation in the PTEN gene, and large visceral arteriovenous malformations. The association of these pleomorphic syndromes with arteriovenous malformations can be explained by the putative role of the PTEN gene in suppressing angiogenesis. Recognition of arteriovenous malformations as a clinical feature of Cowden syndrome has implications for the clinical management of patients with this disorder. PMID:16061556

  15. Ear preference in auditory perception

    Microsoft Academic Search

    M. P. Bryden

    1963-01-01

    In a dichotic listening experiment, normal adult Ss identified numbers presented to the right ear more accurately than numbers presented to the left ear and preferred to report the material from the right ear first. To evaluate the effect of order of report a further experiment was performed (N = 32) in which Ss were instructed to report each channel

  16. Ear Infections and Language Development.

    ERIC Educational Resources Information Center

    Roberts, Joanne E.; Zeisel, Susan A.

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

  17. Arteriovenous malformation of the oral cavity.

    PubMed

    Manjunath, S M; Shetty, Sujan; Moon, Ninad J; Sharma, Bhushan; Metta, Kiran Kumar; Gupta, Nitin; Goyal, Sandeep; Singh, Simranjit

    2014-01-01

    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

  18. Neuroimaging of cerebral cavernous malformations.

    PubMed

    Klostranec, J M; Krings, T

    2015-09-01

    Cerebral cavernous malformations (CCMs) are vascular malformations of the brain and brainstem that arise via a number of different mechanisms and can result in non-specific presentations. Therefore, medical imaging is essential in the diagnosis of these lesions and important to guide their clinical or surgical management. MRI is the modality of choice with newer protocols, such as susceptibility weighted imaging, playing an increasingly important role in the detection of CCMs. In this review we will discuss lesion structure, its proposed origins, and common lesion presentations and complications, before covering their expected appearances with different imaging modalities. We conclude with a discussion of insights about lesion behaviour acquired from advanced imaging techniques and provide a general approach to characterizing and diagnosing CCM lesions with neuroimaging. PMID:25968928

  19. Ear recognition: a complete system

    NASA Astrophysics Data System (ADS)

    Abaza, Ayman; Harrison, Mary Ann F.

    2013-05-01

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

  20. The Chiari II malformation: cause and impact

    Microsoft Academic Search

    David G. McLone; Mark S. Dias

    2003-01-01

    IntroductionIt is the Chiari II malformation and its effects that determine the quality of life of the individual born with spina bifida.DiscussionThe cause of this malformation has been a source of debate for many years. Understanding the cause enables strategies for the management of problems created by this malformation to be developed. An open neural tube defect allows fluid to

  1. Cryptic vascular malformations involving the brainstem

    SciTech Connect

    Yeates, A.; Enzmann, D.

    1983-01-01

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

  2. Bells in Your Ears

    NSDL National Science Digital Library

    Mid-continent Research for Education and Learning (McREL)

    2004-01-01

    Does sound travel better through solids or gases? This material is part of a series of hands-on science activities designed to arouse student interest. Here the student hangs a metal fork from a pencil using string, and then strikes the fork while the eraser end of the pencil is in his or her ear. The activity includes a description, a list of science process skills and complex reasoning strategies being used, and a compilation of applicable national science standards for grades K-12. Also provided are content topics, a list of necessary supplies, instructions to perform the activity, and presentation techniques. The activity's content is explained, and assessment suggestions are provided.

  3. Bionic ear imaging

    Microsoft Academic Search

    R. Cerini; N. Faccioli; M. Barillari; M. De Iorio; M. Carner; V. Colletti; R. Pozzi Mucelli

    2008-01-01

    Purpose  The aim of this study was to illustrate the different imaging features of middle and inner ear implants, brainstem implants\\u000a and inferior colliculus implants.\\u000a \\u000a \\u000a \\u000a Materials and methods  We retrospectively reviewed the computed tomography (CT) images of 468 patients with congenital or acquired transmissive or\\u000a neurosensory hearing loss who underwent surgery. The implants examined were: 22 Vibrant Soundbridge implants, 5 at the

  4. Chiari I malformation with holocord syrinx

    Microsoft Academic Search

    J Kumar; A Kumar; S Gupta

    2009-01-01

    We present the magnetic resonance imaging findings of a classical case of Chiari I malformation with holocord syringohydromyelia. The clinical presentation, imaging findings and treatment options are discussed.

  5. [Congenital vascular malformations (Hauert disease)].

    PubMed

    Hauert, J; Loose, D A; Dreyer, T; Obermayer, B; Deibele, A

    2012-06-01

    Patients with congenital vascular malformations often suffer from arthralgia, especially of the lower limbs. This orthopaedic disease pattern is defined as destructive, angiodysplatic arthritis or Hauert disease and leads to very early destruction of the joints. By presenting diagnostic and therapeutic algorithms, Hauert disease is emphasized as a possible differential diagnosis in order to minimize the risk of an incorrect diagnosis which might lead to under-, over-, or even incorrect treatment. A minimally invasive transathroscopic therapy in the early stages can lead to significant improvement of symptoms and prevention of progressive joint destruction. PMID:22699760

  6. Scalp arteriovenous malformations in young

    PubMed Central

    Gupta, Rakesh; Kayal, Akshat

    2014-01-01

    Scalp arteriovenous malformations are an exceptional group of vascular lesions with curious presentations and an elusive natural history. Their detection in the pediatric population is a rarer occurrence. We discuss our experience with five children suffering from this pathology and their surgical management carried at our institution from 2007 to 2013. The genesis in pediatric patients is, usually, spontaneous in contrast with the history of trauma seen in adults. Clinical symptoms, usually, range from an asymptomatic lesion, local discomfort, headaches to necrosis and massive hemorrhage. Selective angiography remains the cornerstone for investigation. Complete surgical excision, embolization or an approach combining the modalities is curative. PMID:25624933

  7. [Surgical treatment of arteriovenous malformations].

    PubMed

    Casanova, D; Bardot, J; Bartoli, J-M; Magalon, G

    2006-01-01

    MAV surgery remains still today one of most difficult in the surgery of vascular malformations. Embolisation the most selective possible follow-up of a surgical removal carrying the totality of the nidus is the only effective treatment. Usually, MAV of small size, quiescent, should not be operated, the surgery being reserved for the evolutionary or complicated forms. This surgical procedure must allow the complete eradication of the lesion without which the cure is not possible. The incomplete removal causes repetition, sometimes aggravation with, sometimes, life threatening problems. PMID:17007986

  8. Beware of dogs licking ears.

    PubMed

    Godey, B; Morandi, X; Bourdinière, J; Heurtin, C

    1999-10-01

    A patient with right-sided chronic purulent otorrhoea developed meningitis due to Pasteurella multocida transmitted by a dog that frequently licked his ear. We suggest that patients with a perforated tympanic membrane should avoid being licked on their ears by animals. PMID:10520644

  9. Congenital bronchopulmonary foregut malformations: concepts and controversies

    Microsoft Academic Search

    Beverley Newman

    2006-01-01

    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

  10. A Framework for Detecting Malformed SMS Attack

    Microsoft Academic Search

    M Zubair Rafique; Muhammad Khurram Khan; Khaled Alghathbar; Muddassar Farooq

    2011-01-01

    Malformed messages in different protocols pose a serious threat because they are used to remotely launch malicious activity. Furthermore, they are capable of crashing servers and end points, sometimes with a single message. Recently, it was shown that a malformed SMS can crash a mobile phone or gain unfettered access to it. In spite of this, little research has been

  11. Cerebral dysplastic vascular malformation: a developmental arrest

    SciTech Connect

    Wortzman, G.; Sima, A.A.F.; Morley, T.P.

    1983-08-01

    A cryptic malformation of the brain was found to represent an arrest in vascular development. Microscopy showed plump endothelium of blood vessels, which did not have a normal lumen and consisted of solid cords of cells. The microscopic, angiographic, and computed tomographic appearance of this anomaly are discussed and compared with cavernous angiomas, arteriovenous malformations, and venous angiomas.

  12. Cerebellar malformations: some pathogenetic considerations.

    PubMed

    Urich, H

    1979-01-01

    1) Destructive processes are responsible for most cases of cerebellar microgyria of the trabecular pattern. Erosion and subsequent fusion of the folia produce the disorganized pattern in which the various cellular elements retain their noraml relationship and are capable of normal maturation. Intrauterine infection is responsible for most cases; the evidence is conclusive in some cases, presumptive in others. 2) Faulty genetic coding, as illustrated by the trisomies, may lead to formation of heterotopias. The primitive cells aggregating around the dentate nucleus should be interpreted as matrix cells and not as cells of the external granular layer. Cortical heterotopias with attempted internal organisation also occur; their origin is obscure. The unusual, possibly unique, transposition of the internal granular and Purkinje cell layers observed in one case may be ascribed to faulty formation of the Bergmann glia by analogy with the weaver mouse. 3) It is impossible at present to disentangle the role of genetic and environmental factors in the pathogenesis of the hysraphic malformations. It is possible, however, that defective fusion of the intraventricular cerebellar primordium plays a part in the development of the Dandy-Walker malformation, of midine cerebellar clefts in some cases of occipital encephalocele, and of extra-axial ependymal cysts of the posterior fossa. PMID:233076

  13. Shape and Structural Feature Based Ear Recognition

    Microsoft Academic Search

    Zhichun Mu; Li Yuan; Zhengguang Xu; Dechun Xi; Shuai Qi

    2004-01-01

    \\u000a Application and research of ear recognition technology is a new subject in the field of biometric recognition. The earlier\\u000a research has shown that human ear is one of the representative human biometrics with uniqueness and stability. The paper discusses\\u000a the edge-based ear recognition method including ear edge detection, ear description and feature extraction, recognition method\\u000a and ear database construction. The

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

    PubMed

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

    2015-07-01

    Constricted ear combined with Stahl's ear is a rare ear deformity, which is a kind of complex congenital auricular deformity. From 1 January 2007 to 1 January 2014, 19 patients with constricted ear combined with Stahl's ear (Spock ear) were enrolled in this study, most of which were unilaterally deformed. To correct the deformity, a double Z-shaped skin incision was made on the posterior side of the auricle, with the entire layer of cartilage cut parallel to the helix traversing the third crus to form a fan-shaped cartilage flap. The superior crura of the antihelix were shaped by the folding cartilage rim. The cartilage of the abnormal third crus was made part of the new superior crura of antihelix, and the third crus was eliminated. The postoperative aesthetic assessment of the reshaped auricle was graded by both doctors and patients (or their parents). Out of the 19 patients, the number of satisfying cases of the symmetry, helix stretch, elimination of the third crus, the cranioauricular angle, and the substructure of the reshaped ears was 14 (nine excellent and five good), 16 (six excellent and 10 good), 17 (eight excellent and nine good), 15 (five excellent and 10 good), and 13 (two excellent and 11 good), respectively. With a maximum of a 90-month follow-up, no complication was observed. The results of the study suggested that this rare deformity could be corrected by appropriate surgical treatment, with a satisfied postoperative appearance. PMID:25921912

  15. A gene map of congenital malformations.

    PubMed Central

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

    1994-01-01

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

  16. Cephalic Pancreaticoduodenectomy for Bleeding Duodenal Arteriovenous Malformation

    PubMed Central

    Ortiz, Ruben; Dominguez, Eva; Barrena, S.; Martinez, Leopoldo; Prieto, Gerardo; Burgos, Emilio; Tovar, Juan Antonio

    2014-01-01

    Introduction?Treatment of recurrent severe gastrointestinal bleeding due to arteriovenous malformations may require complex resections. In some particular locations, extensive surgery is the only way out, as shown in this report. Case Report?A 2.5-year-old child suffered repeated episodes of upper gastrointestinal bleeding since the first month of life. After an extensive diagnostic workout, the diagnosis of duodenal arteriovenous malformation was established. Cephalic pancreaticoduodenectomy with pyloric preservation was performed and no further episodes of bleeding occurred in the ensuing 2 years. Conclusion?Bleeding malformations located in the pancreaticoduodenal area can be effectively treated in children by pylorus-preserving cephalic pancreaticoduodenectomy. PMID:25755960

  17. Hereditary intraosseous vascular malformation of the craniofacial region: imaging findings

    Microsoft Academic Search

    I Vargel; P KIRATLI; D AKINCI; Y ERK

    2004-01-01

    Benign vascular lesions can be classified into two categories depending on clinical behaviour and endothelial cell characteristics: neoplasms (haemangiomas) and vascular malformations. However, intraosseous vascular anomaly, previously called intraosseous haemangioma, is a very rare malformation. In our previous study, we described the first hereditary form of intraosseous vascular malformation of the craniofacial region, vascular malformation osseous (VMOS). Characteristic findings are

  18. Hearing, Ear Infections, and Deafness

    MedlinePLUS

    ... with hearing loss Ear Infections — facts for parents Communication Methods & Devices for People With Hearing Loss American ... Into Health ® National Institute on Deafness and Other Communication Disorders 31 Center Drive, MSC 2320, Bethesda, MD ...

  19. Middle-Ear Pressure Under Basal Conditions

    E-print Network

    Allen, Jont

    Middle-Ear Pressure Under Basal Conditions Leif Hergils, MD, Bengt Magnuson, MD, PhD \\s=b\\Spontaneous pressure changes in the middle ear were measured under bas- al conditions in ten subjects with healthy ears. Results showed that the pressure in the majority of ears remained slightly above the atmo- spheric

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

    E-print Network

    Sim, Terence

    system [14] including automated segmentation of the ear in a pro- file view image and 3D shape matchingWonder Ears: Identification of Identical Twins from Ear Images Hossein Nejati , Li Zhang ,Terence explored automatic ear recognition for identical twin identification. Ear image recognition has been stud

  1. Update on the management of anorectal malformations.

    PubMed

    Bischoff, Andrea; Levitt, Marc A; Peña, Alberto

    2013-09-01

    Thirty-three years ago, on 10 August 1980, in Mexico City, the first patient with an anorectal malformation was operated on using the posterior sagittal approach. At that time it was not obvious that we were actually opening a "Pandora's box" that continues to give many positive surprises, a few disappointments, and the constant hope that each day we can learn more about how to improve the quality of life of children born with all different types of anorectal malformations. In November 2012, patient number 3000 in our database was operated in the city of Cochabamba, Bolivia; during one of our International Courses of Anorectal Malformations and Colorectal Problems in Children. The goal of this article is to give a brief update on the current management of patients with anorectal malformation, based on the multiple lessons learned during this period. PMID:23913263

  2. Genetics Home Reference: Cerebral cavernous malformation

    MedlinePLUS

    ... and they lack other support tissues, such as elastic fibers, which normally make them stretchy. As a ... cell ; central nervous system ; cerebral hemorrhage ; CNS ; cutaneous ; elastic ; familial ; family history ; gene ; hemangioma ; hemorrhage ; inheritance ; malformation ; ...

  3. Malformation and plastic surgery in childhood

    PubMed Central

    Siegert, Ralf; Magritz, Ralph

    2014-01-01

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

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

    PubMed Central

    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

    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

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

    PubMed

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

    2015-07-01

    E26 transformation-specific 1 (ETS1) and friend leukemia integration 1 (FLI1) are members of the ETS family of transcription factors, of which there are 28 in humans. Both genes are hemizygous in Jacobsen syndrome, an 11q contiguous gene deletion disorder involving thrombocytopenia, facial dysmorphism, growth and mental retardation, malformation of the heart and other organs, and hearing impairment associated with recurrent ear infections. To determine whether any of these defects are because of hemizygosity for ETS1 and FLI1, we characterized the phenotype of mice heterozygous for mutant alleles of Ets1 and Fli1. Fli1(+/-) mice displayed mild thrombocytopenia, as did Ets1(+/-)Fli1(+/-) animals. Fli1(+/-) and Ets1(+/-)Fli1(+/-) mice also displayed craniofacial abnormalities, including a small middle ear cavity, short nasal bone, and malformed interface between the nasal bone process and cartilaginous nasal septum. They exhibited hearing impairment, otitis media, fusions of ossicles to the middle ear wall, and deformed stapes. Hearing impairment was more penetrant and stapes malformations were more severe in Ets1(+/-)Fli1(+/-) mice than in Fli1(+/-) mice, indicating partial functional redundancy of these transcription factors during auditory development. Our findings indicate that the short nose, otitis media, and hearing impairment in Jacobsen syndrome are likely because of hemizygosity for ETS1 and FLI1. PMID:26093983

  6. Epidemiology of Fusarium Diseases and their Mycotoxins in Maize Ears

    Microsoft Academic Search

    Gary P. Munkvold

    2003-01-01

    Fusarium species cause two distinct diseases on ears of maize, Fusarium ear rot (or pink ear rot) and Gibberella ear rot (or red ear rot), both of which can result in mycotoxin contamination of maize grain. The primary causal agent for Fusarium ear rot is Fusarium verticillioides, but F. subglutinans and F. proliferatum are also important. Gibberella ear rot is

  7. Nature, frequency and natural history of intracranial cavernous malformations in adults 

    E-print Network

    Hall, Julie Maria

    2014-07-05

    Scottish Intracranial Vascular Malformation Study was the first prospective, population-based study of the major types of intracranial vascular malformations; arteriovenous, cavernous and venous malformations including ...

  8. 3D Printed Bionic Ears

    PubMed Central

    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

    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

  9. 3D printed bionic ears.

    PubMed

    Mannoor, Manu S; Jiang, Ziwen; James, Teena; Kong, Yong Lin; Malatesta, Karen A; Soboyejo, Winston O; Verma, Naveen; Gracias, David H; McAlpine, Michael C

    2013-06-12

    The ability to three-dimensionally interweave biological tissue with functional electronics could enable the creation of bionic organs possessing enhanced functionalities over their human counterparts. Conventional electronic devices are inherently two-dimensional, preventing seamless multidimensional integration with synthetic biology, as the processes and materials are very different. Here, we present a novel strategy for overcoming these difficulties via additive manufacturing of biological cells with structural and nanoparticle derived electronic elements. As a proof of concept, we generated a bionic ear via 3D printing of a cell-seeded hydrogel matrix in the anatomic geometry of a human ear, along with an intertwined conducting polymer consisting of infused silver nanoparticles. This allowed for in vitro culturing of cartilage tissue around an inductive coil antenna in the ear, which subsequently enables readout of inductively-coupled signals from cochlea-shaped electrodes. The printed ear exhibits enhanced auditory sensing for radio frequency reception, and complementary left and right ears can listen to stereo audio music. Overall, our approach suggests a means to intricately merge biologic and nanoelectronic functionalities via 3D printing. PMID:23635097

  10. A biomechanical ear model to evaluate middle-ear reconstruction.

    PubMed

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

    2009-12-01

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

  11. [The lymphatic malformations: clinical aspects and evolution].

    PubMed

    Salazard, B; Londner, J; Casanova, D; Bardot, J; Magalon, G

    2006-01-01

    Lymphatic malformations (LM) are the most frequent vascular malformations. There are three types of lesions involving lymphatic development that must be included in LM: vascular anomalies and knots (truncal malformations [TLM]); cystic anomalies, superficial or deep, uni- or multicystic (extratruncal malformations [ETLM]) and hemolymphatic anomalies which combine venous, arterial, or capillary malformations with LM. ETLM can be ubiquitously distributed but most are located in the cervical or axillary regions. Most ETLM are diagnosed at birth and in 80-90% of the cases before the age of 2. The clinical aspects are extremely variable: superficial ETLM (vesicular) and deep ETLM, localised or diffuse, mono- or multicystic. TLM are generally located on a lower limb with neonatal lymphatic oedema (often in a polymalformation context). All forms of the hemolymphatic combination can be identified. They are generally located on the limbs and are often unilateral. They are usually sporadic but can also be can be found in polymalformation syndromes (Klippel-Trénaunay, Parkes-Weber, Protée, Maffucci). ETLM generally tend to increase in volume and spread with age with stabilisation at puberty. They do not tend to spontaneously regress. Specific local complications can have serious consequences. They are linked to haemorrhaging, infections and compression phenomena. There can also be complications such as skeletal and soft tissue hypertrophy. PMID:17007978

  12. Neurosensory Development in the Zebrafish Inner Ear 

    E-print Network

    Vemaraju, Shruti

    2012-02-14

    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...

  13. Neurosensory Development in the Zebrafish Inner Ear

    E-print Network

    Vemaraju, Shruti

    2012-02-14

    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...

  14. Nonsurgical management of vascular malformation of masseter.

    PubMed

    Jayaraman, Venkatesh; Austin, Ravi David; Kannan, Saravanan

    2015-01-01

    Intramuscular vascular anomalies are rare congenital hamartomatous lesions. Less than 1% of these occur in skeletal muscle out of which 15% arise in head and neck musculature. In the head and neck region, masseter muscle is the most common site. It accounts for about 5% of intramuscular vascular malformations. They are present from birth but are clinically apparent during infancy and childhood and occasionally during adulthood. Due to its location it is often mistaken for a parotid swelling. The usual treatment of choice is surgical excision with a margin. This is associated with loss of motor function, hemorrhage, nerve damage. Intralesional sclerotherapy, embolization are nonsurgical alternatives for treatment of slow flow venous malformations. Sclerotherapy can be used solely in multiple sittings or as an adjunct to surgery. This article presents a case report of a 28-year-old male with recurrent intramuscular vascular malformation in the masseter muscle, which was successfully treated by ethanol sclerotherapy. PMID:25961625

  15. The caecilian ear: further observations.

    PubMed

    Wever, E G; Gans, C

    1976-10-01

    The structure of the ear is examined in two species of caecilians, Ichthyophis glutinosus and I. orthoplicatus, and the sensitivity to aerial sounds is assessed in terms of the electrical potentials of the cochlea. The results are in general agreement with previous reports on other caecilian species. PMID:1068485

  16. Tumors of the ear canal.

    PubMed

    Rogers, K S

    1988-07-01

    This discussion will focus on the clinical presentation, diagnostic evaluation, expected biologic behavior, and therapeutic options for tumours of the ear canal in the dog and cat. Particular emphasis will be placed on neoplasia of the ceruminous glands, squamous cell carcinoma, and non-neoplastic mass lesions. PMID:3264960

  17. Surgery of the ear and pinna.

    PubMed

    Lanz, Otto I; Wood, Brett C

    2004-03-01

    There are several disease processes of the ear and pinna that warrant surgical intervention. This article reviews surgical anatomy and common surgical procedures of the ear and pinna, including aural hematomas, lateral wall resection, vertical ear canal resection, total ear canal ablation and lateral bulla osteotomy, partial pinna resection, and feline inflammatory polyps. The clinical signs, diagnosis, and surgical treatment along with potential complications for each disease process are discussed. PMID:15062625

  18. Ear Recognition Based on Gabor Features and KFDA

    PubMed Central

    Mu, Zhichun

    2014-01-01

    We propose an ear recognition system based on 2D ear images which includes three stages: ear enrollment, feature extraction, and ear recognition. Ear enrollment includes ear detection and ear normalization. The ear detection approach based on improved Adaboost algorithm detects the ear part under complex background using two steps: offline cascaded classifier training and online ear detection. Then Active Shape Model is applied to segment the ear part and normalize all the ear images to the same size. For its eminent characteristics in spatial local feature extraction and orientation selection, Gabor filter based ear feature extraction is presented in this paper. Kernel Fisher Discriminant Analysis (KFDA) is then applied for dimension reduction of the high-dimensional Gabor features. Finally distance based classifier is applied for ear recognition. Experimental results of ear recognition on two datasets (USTB and UND datasets) and the performance of the ear authentication system show the feasibility and effectiveness of the proposed approach. PMID:24778595

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

    E-print Network

    Allen, Jont

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

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

    E-print Network

    Xin, Jack

    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 phenomenology of the inner ear (cochlea) form a class of nonlinear nonlocal dispersive partial differential

  1. The epidemiology of brain arteriovenous malformations in adults 

    E-print Network

    Al-Shahi, Rustam

    Arteriovenous malformations (AVMs) of the brain are part of the spectrum of intracranial vascular malformations (IVMs). They are the leading cause of intracerebral haemorrhage in young adults, they account for ~10% of non-traumatic subarachnoid...

  2. Immunologic Disorders of the Inner Ear.

    ERIC Educational Resources Information Center

    Kinney, William C.; Hughes, Gordon B.

    1997-01-01

    Immune inner ear disease represents a series of immune system mediated problems that can present with hearing loss, dizziness, or both. The etiology, presentation, testing, and treatment of primary immune inner ear disease is discussed. A review of secondary immune inner ear disease is presented for comparison. (Contains references.) (Author/CR)

  3. Ear Biometrics in Human Identification A Dissertation

    E-print Network

    Bowyer, Kevin W.

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

  4. EAR TO THE GROUND IN THIS ISSUE

    E-print Network

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

  5. Massive localised lymphoedema: a rare vascular malformation

    PubMed Central

    Williams, K J; Al-Sakkal, M N; Alsafi, A; Davies, A H

    2013-01-01

    Lymphatic malformations are a subset of congenital vascular malformations, and are caused by a defect in lymphatic development during embryogenesis. When lymphatic mesoderm development is prematurely arrested, it retains it proliferative potential. Stimulus in the future can cause the lesion to proliferate locally without coordination or regulation, resulting in the rare condition known as massive localised lymphoedema (MLL). We present a case report of MLL, a rare and ill-defined soft tissue mass reported in the morbidly obese, with reference to the existing literature. PMID:23761611

  6. Extrapyramidal dysfunction with cerebral arteriovenous malformations 1

    PubMed Central

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

    1974-01-01

    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

  7. Percutaneous Cryotherapy of Vascular Malformation: Initial Experience

    SciTech Connect

    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

    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.

  8. Genetics Home Reference: Microcephaly-capillary malformation syndrome

    MedlinePLUS

    ... with understanding microcephaly-capillary malformation syndrome? angiogenesis ; apoptosis ; autophagy ; autosomal ; autosomal recessive ; breakdown ; capillaries ; cell ; degrade ; developmental ...

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

    NASA Astrophysics Data System (ADS)

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

    2009-02-01

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

  10. A simple ear splint for microtia patients.

    PubMed

    Venkata Krishnan, C J; Balaji, S M; Jain, Ashish R

    2015-01-01

    Microtia is a congenital anomaly of the ear can occur as an isolated birth defect or as part of a spectrum of anomalies or as a syndrome. Microtia is often associated with impaired hearing and or total loss of hearing. Such patients typically require treatment for surgical ear reconstruction and for hearing impairment. Maintenance of ear projection and post auricular sulcus in staged ear reconstruction in microtia is a trying problem. So also is the maintenance of the patency of the external auditory meatus following recanalization and meatoplasty.This case report describes a simple effective way of fabrication of ear splint prosthesis. PMID:26096122

  11. Interventional treatment of pulmonary arteriovenous malformations.

    PubMed

    Andersen, Poul Erik; Kjeldsen, Anette Drøhse

    2010-09-28

    Pulmonary arteriovenous malformations (PAVM) are congenital vascular communications in the lungs. They act as right to left shunts so that the blood running through these malformations is not oxygenated or filtered. These patients are typically hypoxaemic with exercise intolerance and are at high risk of paradoxical emboli to the brain and other organs. These malformations are most commonly seen in hereditary haemorrhagic telangiectasia (HHT) (Mb. Osler-Weber-Rendu syndrome). Nowadays, the generally accepted treatment strategy of first choice is embolization of the afferent arteries to the arteriovenous malformations. It is a minimally invasive procedure and at the same time a lung preserving treatment with a very high technical success, high effectiveness and low morbidity and mortality. Embolization prevents cerebral stroke and abscess as well as pulmonary haemorrhage and further raises the functional level. Embolization is a well-established method of treating PAVM, with a significant effect on oxygenation of the blood. Screening for PAVM in patients at risk is recommended, especially in patients with HHT. PMID:21160695

  12. Traitement chirurgical des malformations lymphatiques

    Microsoft Academic Search

    A. Lavie; C. Desouches; D. Casanova; J. Bardot; G. Magalon

    2006-01-01

    Lymphatic malformations remain a therapeutic challenge. Many treatments by the past led to poor success. The wide variety of clinical presentations makes it difficult to outline specific management programs. Often, diagnosis or complication circumstances as infection, bleeding, airway obstruction, or handicap force acting. Thus, a specific follow-up joined to a multidisciplinary analysis are the key of well-planned surgery. Through our

  13. Inner ear deficits after chronic otitis media.

    PubMed

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

    2014-08-01

    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

  14. Congenital vascular malformations in scintigraphic evaluation

    PubMed Central

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

    2014-01-01

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

  15. The War of Jenkins’ Ear

    PubMed Central

    Graboyes, Evan M.; Hullar, Timothy E.

    2012-01-01

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

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

    NASA Astrophysics Data System (ADS)

    Delnavaz, Aidin; Voix, Jérémie

    2013-02-01

    Ear canal deformation caused by temporomandibular joint (jaw joint) activity, also known as "ear canal dynamic motion," is introduced in this paper as a candidate source of power to possibly recharge hearing aid batteries. The geometrical deformation of the ear canal is quantified in 3D by laser scanning of different custom ear moulds. An experimental setup is proposed to measure the amount of power potentially available from this source. The results show that 9 mW of power is available from a 15 mm3 dynamic change in the ear canal volume. Finally, the dynamic motion and power capability of the ear canal are investigated in a group of 12 subjects.

  17. Human ear recognition in 3D.

    PubMed

    Chen, Hui; Bhanu, Bir

    2007-04-01

    Human ear is a new class of relatively stable biometrics that has drawn researchers' attention recently. In this paper, we propose a complete human recognition system using 3D ear biometrics. The system consists of 3D ear detection, 3D ear identification, and 3D ear verification. For ear detection, we propose a new approach which uses a single reference 3D ear shape model and locates the ear helix and the antihelix parts in registered 2D color and 3D range images. For ear identification and verification using range images, two new representations are proposed. These include the ear helix/antihelix representation obtained from the detection algorithm and the local surface patch (LSP) representation computed at feature points. A local surface descriptor is characterized by a centroid, a local surface type, and a 2D histogram. The 2D histogram shows the frequency of occurrence of shape index values versus the angles between the normal of reference feature point and that of its neighbors. Both shape representations are used to estimate the initial rigid transformation between a gallery-probe pair. This transformation is applied to selected locations of ears in the gallery set and a modified Iterative Closest Point (ICP) algorithm is used to iteratively refine the transformation to bring the gallery ear and probe ear into the best alignment in the sense of the least root mean square error. The experimental results on the UCR data set of 155 subjects with 902 images under pose variations and the University of Notre Dame data set of 302 subjects with time-lapse gallery-probe pairs are presented to compare and demonstrate the effectiveness of the proposed algorithms and the system. PMID:17299227

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

    PubMed

    Tosi, B; Maestro, S; Marcheschi, M

    1995-10-01

    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

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

    NASA Astrophysics Data System (ADS)

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

    2013-06-01

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

  20. Hereditary Hemorrhagic Telangiectasia with Hepatic Vascular Malformations

    PubMed Central

    Nishioka, Yujiro; Akamatsu, Nobuhisa; Sugawara, Yasuhiko; Arita, Junichi; Sakamoto, Yoshihiro; Hasegawa, Kiyoshi; Kokudo, Norihiro

    2015-01-01

    Hereditary hemorrhagic telangiectasia (HHT) is a rare autosomal dominant hereditary disease. Early diagnosis is important to avoid complications from vascular lesions, but diagnosis is difficult in asymptomatic patients. A 69-year-old Japanese male patient was referred to our hospital for evaluation of hepatic vascular malformations. He had mild anemia with iron deficiency, and dynamic contrast-enhanced computed tomography revealed significant arteriovenous and arterioportal shunts throughout the liver. Telangiectasia from the pharynx to the duodenum was confirmed by gastrointestinal endoscopy. The patient history revealed episodes of epistaxis as well as a family history of epistaxis. He was diagnosed with HHT, although no other family member had been diagnosed with definite HHT. A diagnosis of HHT must be considered in patients with hepatic vascular malformations.

  1. Embolization and radiosurgery for arteriovenous malformations

    PubMed Central

    Plasencia, Andres R.; Santillan, Alejandro

    2012-01-01

    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

  2. Treatment of arteriovenous malformations of the brain

    Microsoft Academic Search

    Andreas Hartmann; Henning Mast; Jae H. Choi; Christian Stapf; Jay P. Mohr

    2007-01-01

    The treatment of ruptured and unruptured brain arteriovenous malformations (AVMs) is driven by the need to prevent incident\\u000a or recurrent intracranial hemorrhages. Improving feasibility of the rapidly developing endovascular, neurosurgical, and radiotherapeutic\\u000a procedures leads to invasive treatment of an increasing number of neurologically intact patients with accidentally diagnosed\\u000a AVMs. Recent data confirm that the natural history risk of unruptured AVMs

  3. Two patients with proatlas segmentation malformation

    Microsoft Academic Search

    Shangchen Xu; Qi Pang; Kaining Zhang; Hu Zhang

    2010-01-01

    A 58-year-old female and an 18-year-old male patient had progressive spastic quadriparesis of 10 years and 6 months duration, respectively. Proatlas segmentation malformation (PSM) was confirmed using three-dimensional (3D) reconstructive CT scans and MRI. Surgical procedures in one patient involved anterior decompression via a transoropharyngeal approach, cranial traction, and posterior occipital–cervical fixation and fusion. His postoperative neurological status had improved

  4. Complex malformation of the inferior vena cava.

    PubMed

    Lesanu, G; Balanescu, R; Pacurar, D; Iaru, O; Vlad, R M; Topor, L; Oraseanu, D

    2014-01-01

    Malformations of the inferior vena cava (IVC) are rare presentations, exceptional in children, and are usually asymptomatic.They are caused by disturbances in the embryological formation of the venous system or can develop as a result of perinatal venous thrombosis with secondary impairment of the venous development. We report the case of a 14 year old boy,admitted for pediatric evaluation before undergoing plastic surgery in order to remove superficial varicose veins of the lower abdomen. The patient presents with inequality in circumference and length of the legs. Laboratory investigations are normal and the abdominal ultrasound describes hypoplasia of the retrohepatic segment of the inferior vena cava. The diagnosis of complex malformation of the abdominal deep venous system (retrohepatic vena cava atresia, cavo-caval anastomosis through azygos veins, abnormal formation of the inferior vena cava with the absence of the left iliac vein) was established through a CT angiography. The presence of abdominal varicose dilations should indicate the necessity to closely look for malformations of the portal and/or caval venous systems. PMID:24742422

  5. Neuroembryology and brain malformations: an overview.

    PubMed

    Sarnat, Harvey B; Flores-Sarnat, Laura

    2013-01-01

    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

  6. The atrioventricular junctions in Ebstein malformation

    PubMed Central

    Ho, S; Goltz, D; McCarthy, K; Cook, A; Connell, M; Smith, A; Anderson, R

    2000-01-01

    OBJECTIVE—To review the anatomical structure of the right atrioventricular junction, including the specialised atrioventricular conduction system, in hearts with Ebstein's malformation, to identify potential substrates for the abnormalities in conduction.?METHODS—Five heart specimens representing the morphological spectrum of Ebstein malformation were examined grossly and histologically.?RESULTS—On the endocardial surface, the atrioventricular junction was marked by a faint line in two hearts, and by a small ridge in the other three. Analysis of the right parietal junction in four hearts revealed only two accessory muscular atrioventricular connections. A plane of fibrofatty tissue separated atrial from ventricular myocardium in the right parietal junction in all hearts. The compact atrioventricular node was closer to the coronary sinus than usual. Accessory nodoventricular connections were present in four hearts, while accessory fasciculo-ventricular connections were found in one. The right bundle branch was hypoplastic or absent in four hearts.?CONCLUSIONS—In this small series, the parietal atrioventricular junction was better developed than previously thought. Structural abnormalities of the atrioventricular conduction system, however, were present. These may account for some of the conduction abnormalities frequently observed with the Ebstein malformation.???Keywords: Ebstein's anomaly; atrioventricular node; bundle branch block; Wolff-Parkinson-White syndrome PMID:10722549

  7. An Effective 3D Ear Acquisition System

    PubMed Central

    Liu, Yahui; Lu, Guangming; Zhang, David

    2015-01-01

    The human ear is a new feature in biometrics that has several merits over the more common face, fingerprint and iris biometrics. It can be easily captured from a distance without a fully cooperative subject. Also, the ear has a relatively stable structure that does not change much with the age and facial expressions. In this paper, we present a novel method of 3D ear acquisition system by using triangulation imaging principle, and the experiment results show that this design is efficient and can be used for ear recognition. PMID:26061553

  8. An Effective 3D Ear Acquisition System.

    PubMed

    Liu, Yahui; Lu, Guangming; Zhang, David

    2015-01-01

    The human ear is a new feature in biometrics that has several merits over the more common face, fingerprint and iris biometrics. It can be easily captured from a distance without a fully cooperative subject. Also, the ear has a relatively stable structure that does not change much with the age and facial expressions. In this paper, we present a novel method of 3D ear acquisition system by using triangulation imaging principle, and the experiment results show that this design is efficient and can be used for ear recognition. PMID:26061553

  9. Adenomatous tumors of the middle ear.

    PubMed

    Pelosi, Stanley; Koss, Shira

    2015-04-01

    Adenomatous tumors are an uncommon cause of a middle ear mass. Clinical findings may be nonspecific, leading to difficulties in differentiation from other middle ear tumors. Controversy also exists whether to classify middle ear adenoma and carcinoid as separate neoplasms, or alternatively within a spectrum of the same pathologic entity. Most adenomatous middle ear tumors are indolent in behavior, with a benign histologic appearance and slowly progressive growth. The mainstay of treatment is complete surgical resection, which affords the greatest likelihood of cure. PMID:25769353

  10. Welfare implications of sheep ear tags.

    PubMed

    Edwards, D S; Johnston, A M

    1999-05-29

    The damaging effects of ear tags used to identify sheep were studied by examining the ears of sheep after slaughter in three different abattoirs and the ears of sheep on a farm. In total, 1040 ears with tags were examined. There were six types of ear tag: metal 'Ketchum' style loop tags; two-piece rigid plastic tags; 'Allflex' style flexible plastic tags with a male and female part; golf tee-shaped plastic ear tags; one-piece rigid plastic loop tags; and one-piece flexible plastic tags with a flap. The metal loop tags and plastic loop tags caused the most lesions, and the majority of the severe lesions. Ear tags placed near to the tip of the ear appeared to cause more damage. Some of the Ketchum style metal tags and two-piece rigid plastic tags appeared to be relatively new, as if recently fitted. These tags were more often associated with ear lesions, particularly moderate or severe lesions. The Allflex style flexible plastic tags caused the fewest problems, and the golf tee-shaped plastic tags also caused significantly fewer problems than the other tags. PMID:10390799

  11. Fear the EAR: Discovering and Mitigating Execution After Redirect Vulnerabilities

    E-print Network

    California at Santa Barbara, University of

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

  12. Extracranial vascular malformations (hemangiomas and vascular malformations) in children and adolescents - diagnosis, clinic, and therapy.

    PubMed

    Eivazi, Behfar; Werner, Jochen A

    2014-01-01

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

  13. Intraosseous mandibular artero-venous malformations: case report.

    PubMed

    Siniscalchi, Enrico Nastro; Minutoli, Fabio; Catalfamo, Luciano; Romano, Fabio; Longo, Marcello; De Ponte, Francesco Saverio

    2009-03-01

    Intraosseous mandibular artero-venous malformations (AVMs) are rare (5% of all vascular malformations) but of great clinical importance. They can lead to dental emergencies and may cause disfigurement, morbidity, and even death. We describe the radiological appearance and the endovascular treatment of a rare high-flow vascular malformation of the mandibular body resembling a dental cyst, embolized by Guglielmi's detachable coils (GDC). PMID:19027310

  14. Macrocephaly–capillary malformation syndrome: Three new cases

    Microsoft Academic Search

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

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

  15. Occlusive vascular disease associated with cerebral arteriovenous malformations

    SciTech Connect

    Mawad, M.E.; Hilal, S.K.; Michelsen, W.J.; Stein, B.; Ganti, S.R.

    1984-11-01

    Selective carotid angiography and computed tomography were used in a study of the association of occlusive vascular disease with cerebral arteriovenous malformations in 13 patients. The majority of the arterial occlusions were proximal to the vascular malformation. Some, however, extended distal to the major branch supplying the arteriovenous malformation (AVM). Selective angiography with subtraction techniques defines the distinct angioarchitecture of these AVMs and the associated stenoses and collateral telangiectases.

  16. Associated malformations among infants with neural tube defects.

    PubMed

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

    2011-03-01

    Infants with neural tube defects (NTDs) often have associated congenital anomalies. The reported frequency and types of associated malformations vary between different studies. The purpose of this investigation was to assess the frequency and types of associated malformations among infants with NTDs in a geographically well-defined population from 1979 to 2008 of 402,532 consecutive births. Of the 441 infants with NTDs born during this period, 20.4% had associated malformations. Infants with associated malformations were divided into those with recognizable conditions [11 (2.5%) infants with chromosomal and 23 (5.2%) with non-chromosomal conditions], and those without recognizable conditions [56 (12.7%) infants with multiple malformations]. Associated malformations were more frequent among infants with encephalocele (36.8%) than those with anencephaly (11.5%) or spina bifida (23.8%). Oral clefts and malformations in the musculoskeletal, renal and cardiovascular systems were the most commonly observed associated anomalies. The frequency of associated malformations in infants with NTDs emphasizes the need for a thorough investigation of these infants. Routine screening for other malformations, especially facial clefts and musculoskeletal, renal and cardiac anomalies, may need to be considered in infants with NTDs, and referral of these infants for genetics evaluation and counseling seems warranted. PMID:21337695

  17. Value of Ear Endoscopy in Cholesteatoma Surgery

    Microsoft Academic Search

    M. Badr-el-Dine

    2002-01-01

    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

  18. Primary carcinoid tumor of the ear

    Microsoft Academic Search

    S. Inoue; K. Tanaka; S. Kannae

    1982-01-01

    A very rare case of primary carcinoid tumor in the left ear of a 35-year-old woman is described. The argyrophilic property and uniformity of the size and shape of neurosecretory granules in the cytoplasm of tumor cells, correspond to the characteristics of carcinoid tumors derived from foregut endoderm. Clinical and light microscopic observations suggest this tumor originated from middle ear

  19. Review Article Mechanics of the frog ear

    E-print Network

    Allen, Jont

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

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

    MedlinePLUS

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

  1. Orf infection following ear tagging in goats.

    PubMed

    Housawi, F M; Abu Elzein, E M

    1991-01-01

    This communication describes the first record of orf infection in goats following ear-tagging. Typical orf lesions were observed in the affected goats, and the virus was reisolated and identified from them. The threat of orf infection following ear-tagging should be kept in mind in orf-endemic regions. PMID:1824135

  2. 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 ...

  3. [Cochlear damage caused by middle ear surgeries].

    PubMed

    Hüttenbrink, K B

    1991-02-01

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

  4. Gene therapy for the inner ear

    PubMed Central

    Fukui, Hideto; Raphael, Yehoash

    2012-01-01

    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

  5. Capillary Malformation–Arteriovenous Malformation, a New Clinical and Genetic Disorder Caused by RASA1 Mutations

    PubMed Central

    Eerola, Iiro; Boon, Laurence M.; Mulliken, John B.; Burrows, Patricia E.; Dompmartin, Anne; Watanabe, Shoji; Vanwijck, Romain; Vikkula, Miikka

    2003-01-01

    Capillary malformation (CM), or “port-wine stain,” is a common cutaneous vascular anomaly that initially appears as a red macular stain that darkens over years. CM also occurs in several combined vascular anomalies that exhibit hypertrophy, such as Sturge-Weber syndrome, Klippel-Trenaunay syndrome, and Parkes Weber syndrome. Occasional familial segregation of CM suggests that there is genetic susceptibility, underscored by the identification of a large locus, CMC1, on chromosome 5q. We used genetic fine mapping with polymorphic markers to reduce the size of the CMC1 locus. A positional candidate gene, RASA1, encoding p120-RasGAP, was screened for mutations in 17 families. Heterozygous inactivating RASA1 mutations were detected in six families manifesting atypical CMs that were multiple, small, round to oval in shape, and pinkish red in color. In addition to CM, either arteriovenous malformation, arteriovenous fistula, or Parkes Weber syndrome was documented in all the families with a mutation. We named this newly identified association caused by RASA1 mutations “CM-AVM,” for capillary malformation–arteriovenous malformation. The phenotypic variability can be explained by the involvement of p120-RasGAP in signaling for various growth factor receptors that control proliferation, migration, and survival of several cell types, including vascular endothelial cells. PMID:14639529

  6. BIOLOGY OF VASCULAR MALFORMATIONS OF THE BRAIN

    PubMed Central

    Leblanc, Gabrielle G.; Golanov, Eugene; Awad, Issam A.; Young, William L.

    2009-01-01

    Background and Purpose This review discusses recent research on the genetic, molecular, cellular, and developmental mechanisms underlying the etiology of vascular malformations of the brain (VMBs), including cerebral cavernous malformation (CCM), sporadic brain arteriovenous malformation (AVM), and the AVMs of hereditary hemorrhagic telangiectasia (HHT). Summary of Review The identification of gene mutations and genetic risk factors associated with CCM, HHT, and sporadic AVM has enabled the development of animal models for these diseases and provided new insights into their etiology. All of the genes associated with VMBs to date have known or plausible roles in angiogenesis and vascular remodeling. Recent work suggests that the angiogenic process most severely disrupted by VMB gene mutation is that of vascular stabilization, the process whereby vascular endothelial cells form capillary tubes, strengthen their intercellular junctions, and recruit smooth muscle cells to the vessel wall. In addition, there is now good evidence that in some cases CCM lesion formation involves a genetic two-hit mechanism, in which a germline mutation in one copy of a CCM gene is followed by a somatic mutation in the other copy. There is also increasing evidence that environmental second hits can produce lesions when there is a mutation to a single allele of a VMB gene. Conclusions Recent findings begin to explain how mutations in VMB genes render vessels vulnerable to rupture when challenged with other inauspicious genetic or environmental factors, and have suggested candidate therapeutics. Understanding of the cellular mechanisms of VMB formation and progression in humans has lagged behind that in animal models. New knowledge of lesion biology will spur new translational work. Several well-established clinical and genetic database efforts are already in place, and further progress will be facilitated by collaborative expansion and standardization of these. PMID:19834013

  7. Somatic Mutations in Cerebral Cortical Malformations

    PubMed Central

    Jamuar, Saumya S.; Lam, Anh-Thu N.; Kircher, Martin; D'Gama, Alissa M.; Wang, Jian; Barry, Brenda J.; Zhang, Xiaochang; Hill, Robert Sean; Partlow, Jennifer N.; Rozzo, Aldo; Servattalab, Sarah; Mehta, Bhaven K.; Topcu, Meral; Amrom, Dina; Andermann, Eva; Dan, Bernard; Parrini, Elena; Guerrini, Renzo; Scheffer, Ingrid E.; Berkovic, Samuel F.; Leventer, Richard J.; Shen, Yiping; Wu, Bai Lin; Barkovich, A. James; Sahin, Mustafa; Chang, Bernard S.; Bamshad, Michael; Nickerson, Deborah A.; Shendure, Jay; Poduri, Annapurna; Yu, Timothy W.; Walsh, Christopher A.

    2014-01-01

    BACKGROUND Although there is increasing recognition of the role of somatic mutations in genetic disorders, the prevalence of somatic mutations in neurodevelopmental disease and the optimal techniques to detect somatic mosaicism have not been systematically evaluated. METHODS Using a customized panel of known and candidate genes associated with brain malformations, we applied targeted high-coverage sequencing (depth, ?200×) to leukocyte-derived DNA samples from 158 persons with brain malformations, including the double-cortex syndrome (subcortical band heterotopia, 30 persons), polymicrogyria with megalencephaly (20), periventricular nodular heterotopia (61), and pachygyria (47). We validated candidate mutations with the use of Sanger sequencing and, for variants present at unequal read depths, subcloning followed by colony sequencing. RESULTS Validated, causal mutations were found in 27 persons (17%; range, 10 to 30% for each phenotype). Mutations were somatic in 8 of the 27 (30%), predominantly in persons with the double-cortex syndrome (in whom we found mutations in DCX and LIS1), persons with periventricular nodular heterotopia (FLNA), and persons with pachygyria (TUBB2B). Of the somatic mutations we detected, 5 (63%) were undetectable with the use of traditional Sanger sequencing but were validated through subcloning and subsequent sequencing of the subcloned DNA. We found potentially causal mutations in the candidate genes DYNC1H1, KIF5C, and other kinesin genes in persons with pachygyria. CONCLUSIONS Targeted sequencing was found to be useful for detecting somatic mutations in patients with brain malformations. High-coverage sequencing panels provide an important complement to whole-exome and whole-genome sequencing in the evaluation of somatic mutations in neuropsychiatric disease. (Funded by the National Institute of Neurological Disorders and Stroke and others.) PMID:25140959

  8. Surgical management of pediatric cerebral arteriovenous malformations

    Microsoft Academic Search

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

    2010-01-01

    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

  9. Management of unbled brain arteriovenous malformation study.

    PubMed

    Mohr, J P; Yaghi, Shadi

    2015-05-01

    In a recent trial limited to arteriovenous malformations discovered not to have bled, medical management was superior to medical management plus intervention. The trial was halted after 226 randomizations and a mean follow-up of 3.3 years owing to a disparity favoring the medical arm. Eligible patients were selected as suitable for lesion eradication. The initial sample size of 800 and follow-up plans for a mean of 7 years were lowered and shortened, respectively, by the outcome data. An application for extended follow-up was given poor priority scores owing to estimations that the disparities in outcomes would not change significantly. PMID:25907910

  10. Pediatric aneurysms and vein of Galen malformations

    PubMed Central

    Rao, V. R. K.; Mathuriya, S. N.

    2011-01-01

    Pediatric aneurysms are different from adult aneurysms – they are more rare, are giant and in the posterior circulation more frequently than in adults and may be associated with congenital disorders. Infectious and traumatic aneursyms are also seen more frequently. Vein of Galen malformations are even rarer entities. They may be of choroidal or mural type. Based on the degree of AV shunting they may present with failure to thrive, with hydrocephalus or in severe cases with heart failure. The only possible treatment is by endovascular techniques – both transarterial and transvenous routes are employed. Rarely transtorcular approach is needed. These cases should be managed by an experienced neurointerventionist. PMID:22069420

  11. Longterm followup in 12 children with pulmonary arteriovenous malformations: confirmation of hereditary

    E-print Network

    Institut des Sciences Cognitives, CNRS

    Longterm followup in 12 children with pulmonary arteriovenous malformations: confirmation@yahoo.fr OBJECTIVE: To assess whether pulmonary arteriovenous malformation (PAVM) is associated with hereditary

  12. History, anatomic forms, and pathogenesis of Chiari I malformations

    Microsoft Academic Search

    Edgardo Schijman; C. Durand

    2004-01-01

    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

  13. Oculocerebrocutaneous syndrome: the brain malformation defines a core phenotype

    PubMed Central

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

    2005-01-01

    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

  14. Metabolic characteristics of cortical malformations causing epilepsy

    PubMed Central

    Mueller, Susanne G.; Barakos, Jerome A.; Cashdollar, Nathan; Flenniken, Derek L.; Vermathen, Peter; Matson, Gerald B.; Weiner, Michael W.

    2009-01-01

    Purpose Cortical malformations (CMs) are increasingly recognized as the epileptogenic substrate in patients with medically refractory neocortical epilepsy (NE). The aim of this study was to test the hypotheses that: 1. CMs are metabolically heterogeneous. 2. The structurally normal appearing perilesional zone is characterized by similar metabolic abnormalities as the CM. Methods Magnetic resonance spectroscopic imaging (MRSI) in combination with tissue segmentation was performed on eight patients with NE and CMs and 19 age-matched controls. In controls, NAA, Cr, Cho, NAA/Cr and NAA/Cho of all voxels of a given lobe were expressed as a function of white matter content and thresholds for pathological values determined by calculating the 95% prediction intervals. These thresholds were used to identify metabolically abnormal voxels within the CM and in the perilesional zone. Results 30% of all voxels in the CMs were abnormal, most frequently because of decreases of NAA or increases of Cho. Abnormal voxels tended to form metabolically heterogeneous clusters interspersed in metabolically normal regions. Furthermore, 15% of all voxels in the perilesional zone were abnormal, the most frequent being decreases of NAA and Cr. Conclusion In CMs metabolically normal regions are interspersed with metabolically heterogeneous abnormal regions. Metabolic abnormalities in the perilesional zone share several characteristics of CMs and might therefore represent areas with microscopic malformations and/or intrinsic epileptogenicity. PMID:15868069

  15. Mid-hindbrain malformations due to drugs taken during pregnancy.

    PubMed

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

    2014-04-01

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

  16. Pinna abnormalities and low-set ears

    MedlinePLUS

    ... Common abnormalities include cysts in the pinna or skin tags . Many children are born with ears that stick ... affect hearing. However, sometimes cosmetic surgery is recommended. Skin tags may be tied off, unless there is cartilage ...

  17. An analysis of the acoustic input impedance of the ear.

    PubMed

    Withnell, Robert H; Gowdy, Lauren E

    2013-10-01

    Ear canal acoustics was examined using a one-dimensional lossy transmission line with a distributed load impedance to model the ear. The acoustic input impedance of the ear was derived from sound pressure measurements in the ear canal of healthy human ears. A nonlinear least squares fit of the model to data generated estimates for ear canal radius, ear canal length, and quantified the resistance that would produce transmission losses. Derivation of ear canal radius has application to quantifying the impedance mismatch at the eardrum between the ear canal and the middle ear. The length of the ear canal was found, in general, to be longer than the length derived from the one-quarter wavelength standing wave frequency, consistent with the middle ear being mass-controlled at the standing wave frequency. Viscothermal losses in the ear canal, in some cases, may exceed that attributable to a smooth rigid wall. Resistance in the middle ear was found to contribute significantly to the total resistance. In effect, this analysis "reverse engineers" physical parameters of the ear from sound pressure measurements in the ear canal. PMID:23917695

  18. Purdue extensionDiplodia Ear Rot Purdue extension

    E-print Network

    Holland, Jeffrey

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

  19. PURDUE EXTENSIONArrested Ear Development in Hybrid Corn PURDUE EXTENSION

    E-print Network

    Holland, Jeffrey

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

  20. Short Papers___________________________________________________________________________________________________ Comparison and Combination of Ear and Face

    E-print Network

    Bowyer, Kevin W.

    ___________________________________________________________________________________________________ Comparison and Combination of Ear and Face Images in Appearance-Based Biometrics Kyong Chang, Kevin W. Bowyer that the ear may have advantages over the face for biometric recognition. Our previous experiments with ear performance using ear images. We report results of similar experiments on larger data sets that are more

  1. A Pain in the Ear: The Radiology of Otalgia

    Microsoft Academic Search

    Jane L. Weissman

    Otalgia is ear pain. Ear disease causes pri- mary otalgia. Secondary (referred) otalgia is referred to the ear from disease in structures remote from the ear. Otalgia, especially referred otalgia, can be a diagnostic challenge. The radiologic approach to a patient with otalgia relies on the physical examination. If the otoscopic findings are abnormal, the computed tomographic (CT) or magnetic

  2. Purdue extensionGibberella Ear Rot Purdue extension

    E-print Network

    Holland, Jeffrey

    1 Purdue extensionGibberella Ear Rot BP-77-W Purdue extension d i s e a s e s o f c o r n Gibberella Ear Rot Authors: Charles Woloshuk Kiersten Wise www.btny.purdue.edu Photos by Charles Woloshuk Gibberella ear rot, or Gib ear rot, is caused by the fungus, Gibberella zeae (Fusarium graminearum

  3. Development of the vertebrate inner ear.

    PubMed

    Rinkwitz, S; Bober, E; Baker, R

    2001-10-01

    The inner ear, also called the membranous labyrinth, contains the cochlea, which is responsible for the sense of hearing, and the vestibular apparatus, which is necessary for the sense of balance and gravity. The inner ear arises in the embryo from placodes, which are epithelial thickenings of the cranial ectoderm symmetrically located on either side of hindbrain rhombomeres 5 and 6. Placode formation in mice is first visible at the 12-somite stage and is controlled by surrounding tissues, the paraxial mesoderm and neural ectoderm. Diffusible molecules such as growth factors play an important role in this process. The activity of several genes confers the identity to the placodal cells. Subsequent cellular proliferation processes under influences from the adjacent hindbrain cause the inner ear epithelium to invaginate and form a vesicle called the otocyst. Combinatorial expression of several genes and diffusible factors secreted from the vesicle epithelium and hindbrain control specification of distinct inner ear compartments. Transplantation studies and inner ear in vitro cultures show that each of these compartments is already committed to develop unique inner ear structures. Later developmental periods are principally characterized by intrinsic differentiation processes. In particular, sensory patches differentiate into fully functional sensory epithelia, and the semicircular canals along with the cochlear duct are elaborated and ossified. PMID:11710453

  4. Transposition of external genitalia and associated malformations.

    PubMed

    Meguid, Nagwa Abdel; Temtamy, Samia A; Mazen, Inas

    2003-01-01

    We report three Egyptian boys from three different families born with penoscrotal transposition (PST). None of them fell into categories of known syndromes, associations, sequences or chromosome disorders. Other malformations observed included renal, genital, cardiovascular and limb defects. Although the occurrence of most reported cases of PST are sporadic, three families with affected sibs have been reported in the literature where parental consanguinity was not specified. Parental consanguinity is present in two of our three cases with young parental age suggesting the possibility of an autosomal recessive pattern of inheritance. A history of maternal hormonal treatment early in pregnancy in one of our cases may suggest a role of teratogenic agents. We conclude that PST may represent only a part of a wide spectrum of anomalies resulting from a major insult occurring during early embryonic development and is of heterogeneous etiology. PMID:12514368

  5. Two patients with proatlas segmentation malformation.

    PubMed

    Xu, Shangchen; Pang, Qi; Zhang, Kaining; Zhang, Hu

    2010-05-01

    A 58-year-old female and an 18-year-old male patient had progressive spastic quadriparesis of 10 years and 6 months duration, respectively. Proatlas segmentation malformation (PSM) was confirmed using three-dimensional (3D) reconstructive CT scans and MRI. Surgical procedures in one patient involved anterior decompression via a transoropharyngeal approach, cranial traction, and posterior occipital-cervical fixation and fusion. His postoperative neurological status had improved remarkably, with imaging showing good realignment of the occipito-atlanto-axial complex with comfortable decompression of the cervico-medulla junction and relief of syringomyelia. MRI and 3D-CT scans are the definitive diagnostic tools for PSM, and appropriate aggressive surgical intervention should be undertaken. PMID:20188566

  6. Computational analyses of arteriovenous malformations in neuroimaging.

    PubMed

    Di Ieva, Antonio; Boukadoum, Mounir; Lahmiri, Salim; Cusimano, Michael D

    2015-05-01

    Computational models have been investigated for the analysis of the physiopathology and morphology of arteriovenous malformation (AVM) in recent years. Special emphasis has been given to image fusion in multimodal imaging and 3-dimensional rendering of the AVM, with the aim to improve the visualization of the lesion (for diagnostic purposes) and the selection of the nidus (for therapeutic aims, like the selection of the region of interest for the gamma knife radiosurgery plan). Searching for new diagnostic and prognostic neuroimaging biomarkers, fractal-based computational models have been proposed for describing and quantifying the angioarchitecture of the nidus. Computational modeling in the AVM field offers promising tools of analysis and requires a strict collaboration among neurosurgeons, neuroradiologists, clinicians, computer scientists, and engineers. We present here some updated state-of-the-art exemplary cases in the field, focusing on recent neuroimaging computational modeling with clinical relevance, which might offer useful clinical tools for the management of AVMs in the future. PMID:25521662

  7. Multimodal device for assessment of skin malformations

    NASA Astrophysics Data System (ADS)

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

    2013-11-01

    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.

  8. Pulmonary arteriovenous malformations and their mimics.

    PubMed

    Gill, S S; Roddie, M E; Shovlin, C L; Jackson, J E

    2015-01-01

    Pulmonary arteriovenous malformations (PAVMs) are abnormal communications between the pulmonary arteries and veins, which result in a right-to-left (R-L) shunt with resultant hypoxemia, the severity of which will depend upon the size and number of lesions. Most PAVMs occur in individuals with hereditary haemorrhagic telangiectasia (HHT) and are a cause of serious morbidity and mortality largely related to cerebrovascular complications secondary to paradoxical embolization. The importance of their recognition and treatment by embolization, even in the absence of symptoms, is well known. Their appearances on chest radiographs are often, but not always, characteristic and the CT appearances are diagnostic; however, there are a number of both vascular and non-vascular diseases that can cause confusion. This review serves to highlight these PAVM "mimics". PMID:25443645

  9. [Surgical treatment of vascular malformations in children and adolescents].

    PubMed

    Haxhija, E Q; Spendel, S; Höllwarth, M E

    2009-04-01

    Vascular malformations are inborn errors of vasculogenesis in localised regions of the body. They are present at birth and grow proportionally with the child without ever showing any tendency to regress. This fact distinguishes them clearly from haemangiomas, which represent vascular tumours with high proliferation during the first year of life followed by a slow involution thereafter. The strategy for the treatment of vascular malformations differs substantially from the one for haemangiomas. According to the type of vascular malformation, the treatment may consist in laser therapy, sclerotherapy, selective embolisation, and/or surgical resection. Whereas systemic medication may save the life of children with life-threatening haemangiomas, such treatment would have no significant effect in children with vascular malformations. The aim of the surgical treatment is to perform a complete resection of the malformation in order to prevent its recurrence. However, since vascular malformations often have an infiltrative growth, frequently only subtotal resections can be performed to avoid unacceptable functional or cosmetic disfigurement of the body. Generally, an optimal management of children with vascular malformations includes a multidisciplinary approach with critical consideration of all potential treatment procedures. PMID:19085820

  10. Localization and treatment of lingual venous and arteriovenous malformations.

    PubMed

    Wiegand, Susanne; Tiburtius, Janna; Zimmermann, Annette P; Güldner, Christian; Eivazi, Behfar; Werner, Jochen A

    2014-02-01

    Venous and arteriovenous malformations of the tongue can cause haemorrhage, airway obstruction, difficulties in chewing and swallowing, speech problems as well as orthodontic abnormalities. The purpose of the present study was to evaluate their exact topography, clinical features, morphologic aspects and management. A retrospective review on all patients with venous and arteriovenous malformations of the tongue who presented between 1998 and 2010 was performed. Medical records were analysed with respect to age and sex distribution, exact localization, symptoms and clinical presentation, management and treatment outcome. Forty-four patients with tongue malformations were analysed. The malformations affected all areas of the tongue as well as the base of the tongue without predilection areas. Nd:YAG laser and CO2 laser therapy provided good results primarily in localized malformations, while in advanced malformations the management was multi-modal since a complete surgical excision was often impossible. The hypothesis that vascular malformations of the tongue occur more frequently along the course of the feeding vessels cannot be confirmed. The therapeutic approach is determined by the exact topography, haemodynamic properties, morphologic aspects and related clinical symptoms as well as patient-specific features. PMID:24558029

  11. Maternal Hypothyroidism in Early Pregnancy and Infant Structural Congenital Malformations

    PubMed Central

    Norstedt Wikner, Birgitta

    2014-01-01

    Background. The question is debated on whether maternal hypothyroidism or use of thyroxin in early pregnancy affects the risk for infant congenital malformations. Objectives. To expand the previously published study on maternal thyroxin use in early pregnancy and the risk for congenital malformations. Methods. Data from the Swedish Medical Birth Register were used for the years 1996–2011 and infant malformations were identified from national health registers. Women with preexisting diabetes or reporting the use of thyreostatics, anticonvulsants, or antihypertensives were excluded from analysis. Risk estimates were made as odds ratios (ORs) or risk ratios (RRs) after adjustment for year of delivery, maternal age, parity, smoking, and body mass index. Results. Among 23?259 infants whose mothers in early pregnancy used thyroxin, 730 had a major malformation; among all 1?567?736 infants, 48012 had such malformations. The adjusted OR was 1.06 (95% CI 0.98–1.14). For anal atresia the RR was 1.85 (95% CI 1.00–1.85) and for choanal atresia 3.14 (95% CI 1.26–6.47). The risk of some other malformations was also increased but statistical significance was not reached. Conclusions. Treated maternal hypothyroidism may be a weak risk factor for infant congenital malformations but an association with a few rare conditions is possible. PMID:24744955

  12. Contribution of Rare Copy Number Variants to Isolated Human Malformations

    PubMed Central

    Serra-Juhé, Clara; Rodríguez-Santiago, Benjamín; Cuscó, Ivon; Vendrell, Teresa; Camats, Núria; Torán, Núria; Pérez-Jurado, Luis A.

    2012-01-01

    Background Congenital malformations are present in approximately 2–3% of liveborn babies and 20% of stillborn fetuses. The mechanisms underlying the majority of sporadic and isolated congenital malformations are poorly understood, although it is hypothesized that the accumulation of rare genetic, genomic and epigenetic variants converge to deregulate developmental networks. Methodology/Principal Findings We selected samples from 95 fetuses with congenital malformations not ascribed to a specific syndrome (68 with isolated malformations, 27 with multiple malformations). Karyotyping and Multiplex Ligation-dependent Probe Amplification (MLPA) discarded recurrent genomic and cytogenetic rearrangements. DNA extracted from the affected tissue (46%) or from lung or liver (54%) was analyzed by molecular karyotyping. Validations and inheritance were obtained by MLPA. We identified 22 rare copy number variants (CNV) [>100 kb, either absent (n?=?7) or very uncommon (n?=?15, <1/2,000) in the control population] in 20/95 fetuses with congenital malformations (21%), including 11 deletions and 11 duplications. One of the 9 tested rearrangements was de novo while the remaining were inherited from a healthy parent. The highest frequency was observed in fetuses with heart hypoplasia (8/17, 62.5%), with two events previously related with the phenotype. Double events hitting candidate genes were detected in two samples with brain malformations. Globally, the burden of deletions was significantly higher in fetuses with malformations compared to controls. Conclusions/Significance Our data reveal a significant contribution of rare deletion-type CNV, mostly inherited but also de novo, to human congenital malformations, especially heart hypoplasia, and reinforce the hypothesis of a multifactorial etiology in most cases. PMID:23056206

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

    E-print Network

    Jacquemin, Christian

    An Eye for an Ear and an Ear for an Eye: Bidirectional Control in Virtual Multimedia Instrument. 1. Control, Mapping, and Interaction in Virtual Multimedia Instruments Recent trends in Human and Virtual Reality. It gives birth to new instruments applied to a wide range of human activities

  14. The relationship between hydrocephalus and Chiari type II malformation in the experimental rat fetuses with Arnold-Chiari malformation.

    PubMed

    Hung, C F

    1986-04-01

    Spina bifida, Chiari type II malformation, cerebral aqueduct stenosis and hydrocephalus are the most frequent association anomalies in the congenital malformation of the central nervous system (Warkany et al., 1958). They are potentially treatable and of clinical importance. But the relationship between hydrocephalus and Chiari type II malformation is still a controversial subject. A single oral dose of 240 mg/kg of ethylenethiourea (ETU) was given to Sprague Dawley (SD) rats on the 11th day of gestation. Fetuses were removed in the 20th day of gestation by cesarean sections; high incidence of spinal dysraphism associated with hindbrain crowding was found in these fetuses. They are similar to Arnold-Chiari malformation in humans. We used these experimental models to analyze the relationship between hydrocephalus and Chiari type II malformation. From the present investigation, no hydrocephalus or cerebral aqueduct stenosis was found in the experimental rat fetuses with the Arnold-Chiari malformation. So we do not consider the hydrodynamic theory that Chiari type II malformation was induced by increasing intracranial pressure in hydrocephalus. Hydrocephalus in the Arnold-Chiari malformation may not be the primary disorder but seems to be caused by plugging the foramen magnum in Chiari type II malformation. So the cerebrospinal fluid in the spinal subarachnoid space can not move upward to the cranial subarachnoid space for absorption to venous return. Cerebral aqueduct stenosis may be secondarily compressed by hydrocephalus and not be the primary development anomaly or acquired occlusion due to gliosis. This is in accord with the theory proposed by Russell and Donald (1935).(ABSTRACT TRUNCATED AT 250 WORDS) PMID:3749365

  15. Prolonged Radiant Exposure of the Middle Ear during Transcanal Endoscopic Ear Surgery.

    PubMed

    Shah, Parth V; Kozin, Elliott D; Remenschneider, Aaron K; Dedmon, Matthew M; Nakajima, Hideko Heidi; Cohen, Michael S; Lee, Daniel J

    2015-07-01

    Transcanal endoscopic ear surgery (EES) provides a high-resolution, wide-field view of the middle ear compared with the conventional operating microscope, reducing the need for a postauricular incision or mastoidectomy. Our group has shown in cadaveric human temporal bone studies that radiant energy from the endoscope tip can quickly elevate temperatures of the tympanic cavity. Elevated temperatures of the middle ear are associated with acute auditory brainstem response shifts in animal models. In EES, proposed methods to decrease middle ear temperature include frequent removal of the endoscope and the use of suction to rapidly dissipate heat; however, the routine application of such cooling techniques remains unknown. Herein, we aim to quantify the duration that the tympanic cavity is typically exposed to the endoscope during routine endoscopic middle ear surgery. We find that the tympanic cavity is exposed to the endoscope without a cooling mechanism for a prolonged period of time. PMID:25779471

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

    PubMed Central

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

    2014-01-01

    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

  17. Pulmonary vascular malformation complicating cryptococcal pneumonia in an immunocompetent patient

    PubMed Central

    Liu, Fang; Chen, Hanzhang; Zhu, Hailong; Li, Shuai; Gu, Pan; Fang, Xia; Wu, Yunjin; Zhang, Suxia; Zhang, Lanjing; Yi, Xianghua

    2014-01-01

    An immunocompetent 50-year-old male presented with slight cough and occasional lung congestion. The radiologic findings included diffuse, bilateral reticular and one nodular opacity at the upper lobe of right lung without clear margin. A wedge resection of the lesion showed disordered distribution of the medium-sized vessels and arterioles, several arterioles densely gathered including a few occlusive arterioles, or medium veins dilated with irregular and elongated cavity, indicating the existence of vascular malformation. Interestingly, near to the malformed vessels, a large area of necrosis with granulomatous inflammation was found. Of note, numerous intracytoplasmic organisms with a nucleus, a wall and a thick capsule, were free in the alveoli or located within the macrophages and polykaryocytes, suggesting cryptococci infection. This is to our best knowledge the first case showing concurrent vascular malformation and local pulmonary cryptococcosis, and vascular malformation was likely an important pathological predisposing factor for local pulmonary cryptococcosis infection. PMID:24696743

  18. MALLEABLE INSPECTION STATION WHERE WORKERS ELIMINATE MALFORMED CASTINGS AND SEPARATED ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    MALLEABLE INSPECTION STATION WHERE WORKERS ELIMINATE MALFORMED CASTINGS AND SEPARATED GOOD CASTINGS PRIOR TO ANNEALING. - Stockham Pipe & Fittings Company, Malleable Annealing Building, 4000 Tenth Avenue North, Birmingham, Jefferson County, AL

  19. Passive and active middle ear implants

    PubMed Central

    Beutner, Dirk; Hüttenbrink, Karl-Bernd

    2011-01-01

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

  20. Extracellular Melanin in Inflammatory Middle Ear Disease

    PubMed Central

    Fritz, Mark; Roehm, Pamela C.; Bannan, Michael A.; Lalwani, Anil K.

    2015-01-01

    Objective Melanin is a pigmented polymer with a known role in dermal solar protection. In vertebrates, melanogenesis has been reported in leukocyte population suggesting a potential role in innate immunity. In this study, we report the novel finding of melanin associated with chronic inflammation and speculate on its potential role in the middle ear and mastoid. Study Design Retrospective review of case series Methods Medical records of 6 patients who demonstrated melanin in the ear were reviewed. Results Six patients, ages from 1 to 63 were identified with extracellular melanin within middle ear and/or mastoid air cells at time of surgery. Intraoperative findings included cholesteatoma (n=3), chronic suppurative OM (n=2), and coalescent mastoiditis (n=1). Histologically, extracellular melanin was identified by Fontana-Masson stain; absence of melanocytes was confirmed by the absence of Melan-A and Prussian Blue stain. One patient had a positive stain for CD163 (a marker for macrophages). Conclusion This case series is the first demonstration of extracellular melanin within middle ear mucosa not associated with melanocytes or metastatic melanocytic lesions. The presence of melanin is either a variant of normal anatomy, a pathway of cholesteatoma formation, or a marker of the inflammatory immune response. Melanin's presence in the setting of inflammation suggests that there may be a heretofore unreported link between the pigmentary and immune systems in the ear. PMID:24999501

  1. Ear Acupuncture in European Traditional Medicine

    PubMed Central

    Firenzuoli, Fabio

    2007-01-01

    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

  2. Allelic and locus heterogeneity in inherited venous malformations

    Microsoft Academic Search

    Jennifer T. Calvert; J. Riney; Christopher D. Kontos; Eugene H. Cha; V ictor G. Prieto; Christopher R. Shea; Jonathan N. Berg; Norman C. Nevin; Sheila A. Simpson; Krystyna A. Pasyk; Marcy C. Speer; Kevin G. Peters; Douglas A. Marchuk

    1999-01-01

    Venous malformations are low-flow vascular lesions consisting of disorganized thin-walled vascular channels. These can occur sporadically but also as an autosomal dominant condition termed venous malformations, cutaneous and mucosal (VMCM; OMIM 600195). In two large unrelated kindreds map- ping to chromosome 9, the identical R849W mis- sense mutation was identified in the first kinase domain of Tie2, an endothelial cell-specific

  3. Split cord malformation type I distal to segmental myelomeningocele.

    PubMed

    Addas, Bassam M

    2014-12-01

    The coexistence of myelomeningocele (MMC) and split cord malformation (SCM) is a well-known phenomenon. The SCM is usually above or at the level of the MMC. Split cord malformation distal to the MMC is considered to be the rarest form of such a combination. We report a case of SCM (type I) distal to the MMC diagnosed pre-operatively. Repair of the MMC and the SCM were carried out in the same setting. PMID:25551117

  4. Occupational exposure to glycol ethers and human congenital malformations

    Microsoft Academic Search

    George Maldonado; Elizabeth Delzell; Rochelle W. Tyl; Lowell E. Sever

    2003-01-01

    ObjectivesThis commentary reviews toxicological information and critically evaluates epidemiological information on the relationship between glycol ethers and congenital malformations.MethodsThe authors identified and assessed toxicological and epidemiological research on glycol ethers used in occupational settings and congenital malformations. Sensitivity analyses evaluated the possible role of methodological problems in explaining the findings of the epidemiological studies.ResultsExposure to certain glycol ethers, including ethylene

  5. Aberrant Carotid Artery in the Middle Ear

    PubMed Central

    Yeti?er, Sertaç

    2015-01-01

    Background: Carotid artery abnormality in the middle ear is a rare clinical condition. Recognition of the problems related with this abnormality is important since it may mimic vascular tumors. Any intervention with incomplete evaluation can be fatal. Case Report: A 23-year-old girl with carotid abnormality and sensorineural hearing loss, unsteadiness and tinnitus is presented. She was followed for 2 months elsewhere assuming that she had Meniere’s disease and had previously received some medication for otitis media with effusion. Conclusion: Tomography and magnetic resonance imaging of the temporal bone are very helpful for visualization of an aberrant carotid in the middle ear. Radiological presentations are the reduced caliber of the aberrant carotid, an absence of cranial opening of carotid canal, tubular coursing along the medial wall of the middle ear in continuity with the horizontal carotid canal, dehiscence of the lateral carotid plate and enlargement of the tympanic canalicus. PMID:25759782

  6. Ear damage caused by leisure noise.

    PubMed

    Maassen, M.; Babisch, W.; Bachmann, K. D.; Ising, H.; Lehnert, G.; Plath, P.; Plinkert, P.; Rebentisch, E.; Schuschke, G.; Spreng, M.; Stange, G.; Struwe, V.; Zenner, H. P.

    2001-01-01

    Noise is a health risk. Recent findings suggest that leisure noise is a substantial danger especially to children, teenagers and young adults. Epidemiological studies of teenagers with no occupational noise exposure show an increasing number with a substantial and measurable irreversible inner ear damage. This is basically due to the wide spread exposition to very loud toys (pistols and squibs), crackers and exposure to electronically amplified music, e.g. from personal cassette players (PCP), at discos or concerts etc. Protection against irreversible ear damage by leisure noise has an important impact in preventive medical care. Therefore the general public must be informed that loud leisure activities may cause damage to the ear. In order to protect children, young people and adults, the legislature ought to set limits for sound levels in discos, concert halls and for music equipment and toys by establishing the necessary standards and regulations. PMID:12678931

  7. Precise individualized armature for ear reconstruction

    NASA Astrophysics Data System (ADS)

    Evenhouse, Raymond J.; Chen, Xiaoming

    1991-04-01

    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.

  8. Endoscopic anatomy of the pediatric middle ear.

    PubMed

    Isaacson, Glenn

    2014-01-01

    Traditionally, otologists have aimed to produce a clean, dry, safe ear with the best possible hearing result. More recently, "less invasively" has been added to this list of goals. The development of small-diameter, high-quality rigid endoscopes and high-definition video systems has made totally endoscopic, transcanal surgery a reality in adult otology and a possibility in pediatric otology. This article reviews the anatomy of the pediatric middle ear and its surrounding airspaces and structures based on the work of dozens of researchers over the past 50 years. It will focus on the developmental changes in ear anatomy from birth through the first decade, when structure and function change most rapidly. Understanding the limits and possibilities afforded by new endoscopic technologies, the pediatric otologist can strive for results matching or exceeding those achieved by more invasive surgical approaches. PMID:24154745

  9. Cerebral Arteriovenous Malformation Associated with Moyamoya Disease

    PubMed Central

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

    2014-01-01

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

  10. Surgical treatment of cerebral cavernous malformations.

    PubMed

    Davies, J M; Kim, H; Lawton, M T

    2015-09-01

    Cerebral cavernous malformations (CMs) are clusters of abnormally-formed, thin-walled blood vessels that tend to hemorrhage, resulting in focal neurological deficits, seizures, and even death, depending on the location of the lesion and extent of bleeding. Management of cerebral CMs can be reduced to the decision to observe or to surgically resect. The objective of the paper was to review options for surgical management of cerebral CMs. A university-based CM practice was examined for: 1) anatomical distribution of operatively managed CMs; and 2) surgical approaches to eloquent CMs. Although cerebral CMs can occur throughout the brain and can lead to significant neurological morbidity, even in highly eloquent locations, such as the brainstem, thalamus, and basal ganglia, experience demonstrates that the majority of CMs can be safely resected and that patients tend to experience long-term improvement in neurological function. The keys to good patient outcomes lie in appropriate patient selection and in thoughtful choice of a surgical approach that minimizes transgression of normal structures. PMID:25881653

  11. Middle ear cholesteatoma in 11 dogs

    PubMed Central

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

    2011-01-01

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

  12. Middle ear cholesteatoma in 11 dogs.

    PubMed

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

    2011-06-01

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

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

    PubMed

    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-Labrèze, Christine; Delrue, Marie-Ange; Bayliss, Susan; Martorell, Loreto; González-Enseñat, 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

    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

  14. 21 CFR 874.4140 - Ear, nose, and throat bur.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ...and throat bur. (a) Identification. An ear, nose, and throat bur is a device consisting of an interchangeable drill bit that is intended for use in an ear, nose, and throat electric or pneumatic surgical drill (§ 874.4250) for...

  15. 21 CFR 874.4140 - Ear, nose, and throat bur.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ...and throat bur. (a) Identification. An ear, nose, and throat bur is a device consisting of an interchangeable drill bit that is intended for use in an ear, nose, and throat electric or pneumatic surgical drill (§ 874.4250) for...

  16. 21 CFR 874.4140 - Ear, nose, and throat bur.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ...and throat bur. (a) Identification. An ear, nose, and throat bur is a device consisting of an interchangeable drill bit that is intended for use in an ear, nose, and throat electric or pneumatic surgical drill (§ 874.4250) for...

  17. Getting Teens to Read with Their Ears

    ERIC Educational Resources Information Center

    Fues, Marianne Cole

    2009-01-01

    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…

  18. Mule Ear Drapery in Jewel Cave

    USGS Multimedia Gallery

    Jewel Cave is currently the 3rd most extensive cave network in the world. It is believed to have formed completely underwater, thus leading to the extensive coating of calcite crystals. In the center of this image, a cave formation known as mule-ear drapery can be seen....

  19. Do Your Ears Pop in Space?

    Microsoft Academic Search

    Robert Lambourne

    1997-01-01

    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

  20. Repeat radiosurgery for cerebral arteriovenous malformations.

    PubMed

    Awad, Ahmed J; Walcott, Brian P; Stapleton, Christopher J; Ding, Dale; Lee, Cheng-Chia; Loeffler, Jay S

    2015-06-01

    We perform a systematic review of repeat radiosurgery for cerebral arteriovenous malformations (AVM) with an emphasis on lesion obliteration rates and complications. Radiosurgery is an accepted treatment modality for AVM located in eloquent cortex or deep brain structures. For residual or persistent lesions, repeat radiosurgery can be considered if sufficient time has passed to allow for a full appreciation of treatment effects, usually at least 3years. A systematic review was performed in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. References for this review were identified by searches of MEDLINE, Web of Science and Google Scholar databases. A total of 14 studies comprising 733 patients met the review criteria and were included. For series that reported target dose at both first and repeat treatments, the weighted means were 19.42Gy and 19.06Gy, respectively. The mean and median obliteration rate for the repeat radiosurgery treatments were 61% (95% confidence interval 51.9-71.7%) and 61.5%, respectively. The median follow up following radiosurgery ranged from 19.5 to 80months. Time to complete obliteration after the repeat treatment ranged from 21 to 40.8months. The most common complications of repeat radiosurgery for AVM included hemorrhage (7.6%) and radiation-induced changes (7.4%). Repeat radiosurgery can be used to treat incompletely obliterated AVM with an obliteration rate of 61%. Complications are related to treatment effect latency (hemorrhage risk) as well as radiation-induced changes. Repeat radiosurgery can be performed at 3 years following the initial treatment, allowing for full realization of effects from the initial treatment prior to commencing therapy. PMID:25913746

  1. Immune Response in Human Cerebral Cavernous Malformations

    PubMed Central

    Shi, Changbin; Shenkar, Robert; Du, Hongyan; Duckworth, Edward; Raja, Harish; Batjer, H. Hunt; Awad, Issam A.

    2009-01-01

    Background and Purpose Preliminary observations suggesting the presence of B and plasma cells and oligoclonality of immunoglobulin (Ig) G in cerebral cavernous malformations (CCMs) have motivated a systematic study correlating the infiltration of the immune cells with clinical activity and antigen-triggered immune response in surgically excised lesions. Methods Infiltration of plasma, B, T and HLA-DR expressing cells and macrophages within 23 excised CCMs was related to clinical activity. Relative amounts of Ig isotypes were determined. IgG clonality of mRNA from CCMs was assessed by spectratyping, cloning and sequencing. Results Infiltration of the immune cells ranged widely within CCM lesions and cells were generally co-expressed with each other. Immune cell infiltration did not associate with recent bleeding and lesion growth. Significantly more B lymphocytes in CCM lesions were associated with venous anomaly. More T cells were present in solitary lesions. More T cells and less macrophages were present in CCMs from younger subjects. IgG isotype was present in all CCM lesions. Most lesions also expressed IgM and IgA, with IgM predominance over IgA correlating with recent CCM growth. Oligoclonality was shown in IgG mRNA from CCMs, but not from peripheral blood lymphocytes, with only eight CDR3 sequences observed among 134 clones from two CCM lesions. Conclusions An antigen-directed oligoclonal IgG immune response is present within CCM lesions regardless of recent clinical activity. Apparent differences in immune response in younger patients and in lesions with recent growth will need confirmation in other series. The pathogenicity of oligoclonal immune response will require systematic hypothesis testing in recently available CCM murine models. PMID:19286587

  2. Magnetic Nanoparticles: Inner Ear Targeted Molecule Delivery and Middle Ear Implant

    Microsoft Academic Search

    Richard D. Kopke; Ronald A. Wassel; Fadee Mondalek; Brian Grady; Kejian Chen; Jianzhong Liu; Don Gibson; Kenneth J. Dormer

    2006-01-01

    Superparamagnetic iron oxide nanoparticles (SNP) composed of magnetite (Fe3O4) were studied preliminarily as vehicles for therapeutic molecule delivery to the inner ear and as a middle ear implant capable of producing biomechanically relevant forces for auditory function. Magnetite SNP were synthesized, then encapsulated in either silica or poly (D,L,-Lactide-co-glycolide) or obtained commercially with coatings of oleic acid or dextran. Permanent

  3. Ear and kidney syndromes: Molecular versus clinical approach

    Microsoft Academic Search

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

    2004-01-01

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

  4. Understanding Inner Ear Development with Gene Expression Profiling

    E-print Network

    Corey, David P.

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

  5. Ear growth, developmental stages and yield in winter wheat

    E-print Network

    Paris-Sud XI, Université de

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

  6. A Survey on Ear Biometrics AYMAN ABAZA, WVHTC Foundation

    E-print Network

    Ross, Arun Abraham

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

  7. History of Studies on Mammalian Middle Ear Evolution: A

    E-print Network

    Sullivan, Jack

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

  8. Purdue extensionAspergillus Ear Rot Purdue extension

    E-print Network

    Holland, Jeffrey

    1 Purdue extensionAspergillus Ear Rot BP-83-W Purdue extension d i s e a s e s o f c o r n Aspergillus Ear Rot Authors: Charles Woloshuk Kiersten Wise www.btny.purdue.edu The fungus Aspergillus flavus causes Aspergillus ear rot, one of the most important diseases in corn. The fungus pro- duces a mycotoxin

  9. Performance analysis of the Ormia ochracea's coupled ears Murat Akcakayaa

    E-print Network

    Nehorai, Arye

    Performance analysis of the Ormia ochracea's coupled ears Murat Akcakayaa and Arye Nehoraib. This phenomenon has been explained by the mechanical coupling between the ears. In this paper, it is first shown that the coupling enhances the differences in times of arrival and frequency responses of the ears to the incoming

  10. Gross congenital malformation at birth in a government hospital.

    PubMed

    Sachdeva, Sandeep; Nanda, Smiti; Bhalla, Kapil; Sachdeva, Ruchi

    2014-01-01

    A hospital-based cross-sectional study was undertaken to determine proportion of gross congenital malformation (GCMF) occurring at intramural births. Rate of GCMF was found to be 16.4/1000 consecutive singleton births (>28 weeks) with three leading malformation as anencephaly (44.68%), talipes equinovarus (17.02%) and meningomyelocele (10.63%). Higher risk of malformed births were noticed amongst un-booked (2.07%) in-comparison to booked (1.01%) mothers; women with low level of education (up to 8 years [2.14%] vs. at least 9 years of schooling [0.82%]); gravida status of at least 3 (2.69%) followed by 1 (1.43%) and 2 (1.0%) respectively; pre-term (5.13%) vs. term (0.66%); cesarean section (4.36%) versus vaginal delivery (0.62%). Mortality was significantly higher among congenitally malformed (17.35%) than normal (0.34%) newborns. With-in study limitation, emergence of neural tube defect as the single largest category of congenital malformation indicates maternal malnutrition (especially folic acid) that needs appropriate attention and management. PMID:24748359

  11. Defining anural malformations in the context of a developmental problem

    USGS Publications Warehouse

    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

    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.

  12. Local Model of Arteriovenous Malformation of the Human Brain

    NASA Astrophysics Data System (ADS)

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

    2013-02-01

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

  13. An Unusual Cause of Dysphagia: A Large Expectorated Arteriovenous Malformation

    PubMed Central

    Griffin, Aaron S.; Gunasena, Rivindi; Schaefer, Nathan R.; Kennedy, Edmund

    2015-01-01

    Background Vascular malformations are generally detected in childhood or adolescence with first presentations in adulthood being rare. Case Report We report the case of a 52-year-old female with threatened compromise of her airway after expectorating a massive arteriovenous malformation anchored at the supraglottis. The only preceding symptom was dysphagia. The lesion was resected, the patient had a quick recovery, and she has shown no evidence of recurrence. Conclusion Although uncommon, vascular malformations of the supraglottis or hypopharynx should be considered in the differential diagnosis of a patient presenting with dysphagia because of the potential to cause disastrous airway compromise. Although a lesion presenting acutely mandates a definitive airway plan, when clinically possible, computed tomography scan and indirect laryngoscopy can provide useful information for the airway and operative teams.

  14. Venous malformations: Sclerotherapy with a mixture of ethanol and lipiodol

    SciTech Connect

    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

    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.

  15. Better-ear glimpsing in hearing-impaired listeners.

    PubMed

    Best, Virginia; Mason, Christine R; Kidd, Gerald; Iyer, Nandini; Brungart, Douglas S

    2015-02-01

    When competing speech sounds are spatially separated, listeners can make use of the ear with the better target-to-masker ratio. Recent studies showed that listeners with normal hearing are able to efficiently make use of this "better-ear," even when it alternates between left and right ears at different times in different frequency bands, which may contribute to the ability to listen in spatialized speech mixtures. In the present study, better-ear glimpsing in listeners with bilateral sensorineural hearing impairment, who perform poorly in spatialized speech mixtures, was investigated. The results suggest that this deficit is not related to better-ear glimpsing. PMID:25698053

  16. 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 ...

  17. A rare newly described overgrowth syndrome with vascular malformations-Cloves syndrome

    PubMed Central

    Gopal, Balaji; Keshava, Shyamkumar N; Selvaraj, Deepak

    2015-01-01

    There are many overgrowth syndromes described in the literature. Few are associated with vascular malformations. We describe a rare, newly described syndrome with features of overgrowth and vascular malformations. PMID:25709171

  18. Balloon-Occluded Retrograde Transvenous Embolization of a Pelvic Arteriovenous Malformation

    SciTech Connect

    Mitsuzaki, Katsuhiko; Yamashita, Yasuyuki; Utsunomiya, Daisuke; Sumi, Seiya; Ogata, Ichiro; Takahashi, Mutsumasa [Department of Radiology, Kumamoto University School of Medicine, 1-1-1 Honjo, Kumamoto 860 (Japan); Kawakami, Shigeo; Ueda, Shohichi [Department of Urology, Kumamoto University School of Medicine, 1-1-1 Honjo, Kumamoto 860 (Japan)

    1999-11-15

    We successfully performed embolization therapy for a pelvic arteriovenous malformation by the retrograde transvenous approach using a liquid embolic material. This malformation was unique in that it had a single draining vein, which allowed this technique employing an occlusion balloon.

  19. Constitutively active Notch4 receptor elicits brain arteriovenous malformations through enlargement of

    E-print Network

    Schaffer, Chris B.

    Constitutively active Notch4 receptor elicits brain arteriovenous malformations through enlargement the arteriovenous (AV) interface are critical for tissue function. AV malformation (AVM) is a pathological, MA, and approved November 11, 2014 (received for review August 13, 2014) Arteriovenous (AV

  20. Embolization in high-flow arteriovenous malformations of the face.

    PubMed

    Komiyama, M; Khosla, V K; Yamamoto, Y; Tazaki, H; Toyota, N

    1992-06-01

    Five patients with arteriovenous malformations of the face (4 males and 1 woman; age range, 11-38 years) were treated by selective embolization through the feeding arteries with polyvinyl alcohol particles. Immediate gross angiographical obliteration was obtained in 4 patients, with pronounced reduction of the arteriovenous shunt in the fifth. Clinical symptoms including bleeding, swelling, pulsations, bruit, and disfigurement improved in all the patients followed up for a period of 6 to 21 months. Polyvinyl alcohol particle embolization, without surgical resection, though palliative could be useful in select patients. The classification and diagnosis of congenital vascular malformations is briefly reviewed and treatment discussed. PMID:1622040

  1. Incorporating Amphibian Malformation into Inquiry-Based Learning

    NSDL National Science Digital Library

    Brooke L. Talley

    2007-01-01

    Since the first discovery of malformed frogs by an adventurous middle school teacher and her after-school science club in 1995, outreach programs like A Thousand Friends of Frogs (see Resources) have been created to connect students and teachers with scientists so that they can better understand frogs and their habitats. Many of the amphibian-malformation activities published in education-practitioner journals approach this subject through internet investigations (Webster 2002), hypothetical case studies (Murphy and Fortner 2001), or reading with discussion (Davidson, Matthews, and Patrick 2001). These activities can be supplemented with inquiry-based labs designed to instruct and engage students about conservation biology, as described in this article.

  2. Pulmonary arteriovenous malformations presenting as refractory heart failure

    PubMed Central

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

    2014-01-01

    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

  3. Regression of a Large Congenital Hepatic Arteriovenous Malformation

    PubMed Central

    Zakaria, Rania; Mostafa, Fatma Alzahraa; Hamza, Hala

    2015-01-01

    Congenital hepatic arteriovenous malformations are rarely seen in association with persistent neonatal pulmonary hypertension. We report the case of a full-term female newborn who presented with heart failure and respiratory distress soon after birth. Echocardiographic investigation revealed severe persistent pulmonary hypertension and patent ductus arteriosus. Here we report spontaneous regression in size of both the feeder vessel and the vascular bed of the congenital hepatic arteriovenous malformation. We postulate that our conservative use of oral heart failure therapy, in the form of diuretic agents and captopril, decreased the congestion and diameter of the affected vessels. PMID:25873838

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

    PubMed Central

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

    2013-01-01

    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

  5. Arteriovenous malformation within an isocitrate dehydrogenase 1 mutated anaplastic oligodendroglioma

    PubMed Central

    Lai, Grace; Muller, Karra A.; Carter, Bob S.; Chen, Clark C.

    2015-01-01

    Background: The co-occurrence of intracranial arteriovenous malformations (AVMs) and cerebral neoplasms is exceedingly rare but may harbor implications pertaining to the molecular medicine of brain cancer pathogenesis. Case Description: Here, we present a case of de novo AVM within an isocitrate dehydrogenase 1 mutated anaplastic oligodendroglioma (WHO Grade III) and review the potential contribution of this mutation to aberrant angiogenesis as an interesting case study in molecular medicine. Conclusion: The co-occurrence of an IDH1 mutated neoplasm and AVM supports the hypothesis that IDH1 mutations may contribute to aberrant angiogenesis and vascular malformation.

  6. A new method for assessment of craniofacial malformations.

    PubMed

    Pelo, Sandro; Tassiello, Stefano; Boniello, Roberto; Gasparini, Giulio; Longobardi, Gianluigi

    2006-11-01

    Many assessments of craniofacial malformations are generally undertaken to assist in surgical intervention including physical examination, cephalometric radiographs in anteroposterior and lateral views, stereolithographic models, and anthropometric measurements integrated with three-dimensional computed tomography (3-D CT) reconstructions to quantify skeletal deformities. In the present report, the use of 3-D Malformation Analysis, a three-dimensional methodology for planning craniofacial operative procedures, is presented. In addition to cephalometric and anthropometric databases, the measurements from 3-D surface reconstructions from CT were used intraoperatively to establish the correct position of skeletal segments. PMID:17119401

  7. [Diseases of the middle ear in childhood].

    PubMed

    Minovi, A; Dazert, S

    2014-03-01

    Middle ear diseases in childhood play an important role in daily ENT practice due to their high incidence. Some of these like acute otitis media or otitis media with effusion have been studied extensively within the last decades. In this article, we present a selection of important childhood middle ear diseases and discuss the actual literature concerning their treatment, management of complications and outcome. Another main topic of this paper deals with the possibilities of surgical hearing rehabilitation in childhood. The bone-anchored hearing aid BAHA® and the active partially implantable device Vibrant Soundbridge® could successfully be applied for children. In this manuscript, we discuss the actual literature concerning clinical outcomes of -these implantable hearing aids. PMID:24710778

  8. Diseases of the middle ear in childhood.

    PubMed

    Minovi, Amir; Dazert, Stefan

    2014-01-01

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

  9. Diseases of the middle ear in childhood

    PubMed Central

    Minovi, Amir; Dazert, Stefan

    2014-01-01

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

  10. Ear tag induced Staphylococcus infection in mice.

    PubMed

    Cover, C E; Keenan, C M; Bettinger, G E

    1989-07-01

    Mice used in a 2-year oral toxicity study developed a progressive, moist dermatitis. The initial lesions were seen around the ears in which metal identification tags had been placed and usually progressed to include the skin of the neck and shoulder. Clinically, the mice were pruritic, lost weight, had rough coats, and became moribund. The predominant finding at necropsy was pale brown kidneys with irregular granular surfaces. Histologically, there was inflammation and focal-to-diffuse necrosis in the visceral organs and affected skin. The predominant organism isolated from the skin, kidneys and heart blood was Staphylococcus aureus. This bacterium is a common inhabitant of the skin of conventionally housed mice and its isolation from the kidneys and blood suggested that the portal of entry was the wound caused by the insertion of the metal ear tag. PMID:2761227

  11. Wide band bunny-ear radiating element

    Microsoft Academic Search

    J. J. Lee; S. Livingston

    1993-01-01

    A low-cost wideband radiating element for EW (electronic warfare) and radar array antenna applications is reported. This printed end-fire bunny-ear element is fed by a balanced slot line, and it can operate over 0.5 to 18 GHz with very low loss in an isolated environment. The input transition, the feed line, and the launching section of this element are essentially

  12. An investigation of ear necrosis in pigs

    PubMed Central

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

    2013-01-01

    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

  13. Ear Recognition from One Sample Per Person

    PubMed Central

    Chen, Long; Mu, Zhichun; Zhang, Baoqing; Zhang, Yi

    2015-01-01

    Biometrics has the advantages of efficiency and convenience in identity authentication. As one of the most promising biometric-based methods, ear recognition has received broad attention and research. Previous studies have achieved remarkable performance with multiple samples per person (MSPP) in the gallery. However, most conventional methods are insufficient when there is only one sample per person (OSPP) available in the gallery. To solve the OSPP problem by maximizing the use of a single sample, this paper proposes a hybrid multi-keypoint descriptor sparse representation-based classification (MKD-SRC) ear recognition approach based on 2D and 3D information. Because most 3D sensors capture 3D data accessorizing the corresponding 2D data, it is sensible to use both types of information. First, the ear region is extracted from the profile. Second, keypoints are detected and described for both the 2D texture image and 3D range image. Then, the hybrid MKD-SRC algorithm is used to complete the recognition with only OSPP in the gallery. Experimental results on a benchmark dataset have demonstrated the feasibility and effectiveness of the proposed method in resolving the OSPP problem. A Rank-one recognition rate of 96.4% is achieved for a gallery of 415 subjects, and the time involved in the computation is satisfactory compared to conventional methods. PMID:26024226

  14. Incidence of anencephaly and other major malformations when oestriol excretion is very low

    Microsoft Academic Search

    L. DEAN; D. A. ABELL; N. A. BEISCHER

    1977-01-01

    A study of 533 women with very low urinary oestriol excretion during the third trimester of pregnancy showed an incidence of major fetal malformations among their infants of 7-1% and a perinatal mortality rate of 14-6%. Thirteen of the malformations were cases of anencephaly, and 26 of the 78 perinatal deaths were due to or associated with major fetal malformations.

  15. Size Estimation and Magnification Error in Radiographic Imaging: Implications for Classification of Arteriovenous Malformations

    E-print Network

    Cunningham, Ian

    of Arteriovenous Malformations Kost Elisevich, Ian A. Cunningham, and Leo Assis PURPOSE: To assess magnification error in digital subtraction angiography as it pertains to arteriovenous malformation (AVM) size schemes for AVMs have been hampered by this technical error. Index terms: Arteriovenous malformations

  16. Visualization and Analysis of Cerebral Arteriovenous Malformation combining 3D and 4D MR Image Sequences

    E-print Network

    Lübeck, Universität zu

    1 Visualization and Analysis of Cerebral Arteriovenous Malformation combining 3D and 4D MR Image-Eppendorf Abstract. In this paper methods for visualization and analysis of cerebral arteriovenous malformations (AVM angiography; cerebral arteriovenous malformation; maximum intensity projection; mutual information; image

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

    ERIC Educational Resources Information Center

    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

    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,…

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

    PubMed Central

    Ravicz, Michael E.; Rosowski, John J.

    2013-01-01

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

  19. Cecal vascular malformation mimicking appendicitis in a child

    Microsoft Academic Search

    Sherif Emil; Siamak Milanchi; Adrian Goldstein

    2005-01-01

    A 5-year-old girl presented with abdominal pain suggestive of appendicitis. Intraoperatively, a solid cecal mass was identified\\u000a along with mesenteric adenopathy. A right hemicolectomy was performed. Pathologic examination revealed a vascular malformation\\u000a with evidence of recent hemorrhage.

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

    PubMed

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

    1992-02-01

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

  1. Sequelae and support after termination of pregnancy for fetal malformation

    Microsoft Academic Search

    J Lloyd; K M Laurence

    1985-01-01

    A retrospective study examined the reactions to the termination of pregnancy for fetal malformation and the follow up services that were available. Women resident in Mid Glamorgan who had had a termination between 1977 and 1981 because of positive findings after midtrimester prenatal diagnostic tests for neural tube defect or chromosome abnormalities were interviewed at home using a semistructured interview

  2. Spitz nevus arising upon a congenital glomuvenous malformation.

    PubMed

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

    2013-01-01

    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

  3. Macrocephaly-capillary malformation syndrome: three new cases.

    PubMed

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

    2012-02-15

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

  4. The Chiari II malformation: Part IV. The hindbrain deformity

    Microsoft Academic Search

    T. P. Naidich; D. G. McLone; K. H. Fulling

    1983-01-01

    Computed tomography successfully delineates the multiple components of the Chiari II malformation at the craniocervical junction, the hindbrain, and the cervical spinal cord. These include wide foramen magnum and upper cervical spinal canal; incomplete fusions of the posterior arches of C1 and lower cervical vertebrae; cascading protrusions of vermis, fourth ventricle, medulla, and cervical cord into the spinal canal; cervicomedullary

  5. Surgical Management of Chiari Malformation: Analysis of 128 Cases

    Microsoft Academic Search

    Fuyou Guo; Meiyun Wang; Jiang Long; Huaili Wang; Hongwei Sun; Bo Yang; Laijun Song

    2007-01-01

    Objective: A variety of surgical interventions have been recommended for patients with Chiari malformations (CMs). In this study, we have evaluated the intraoperative findings and clinical outcome in different-aged patients with CMs undergoing posterior fossa decompression. Methods: Sixteen pediatric and 112 adult cases with CMs underwent suboccipital craniectomy and wide duraplasty as well as autogenous bone grafting in selected cases.

  6. Neurodevelopmental Outcomes in Children with Cerebellar Malformations: A Systematic Review

    ERIC Educational Resources Information Center

    Bolduc, Marie-Eve; Limperopoulos, Catherine

    2009-01-01

    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…

  7. Paraspinal arteriovenous malformation Onyx embolization via an Ascent balloon.

    PubMed

    Martínez-Galdámez, Mario; Rodriguez-Arias, Carlos A; Utiel, Elena; Arreba, Emilio; Gonzalo, Miguel; Arenillas, Juan F

    2013-01-01

    Purely extradural lumbar spinal arteriovenous malformations (AVMs) are rare lesions that have diverse presentations and imaging features. The treatment of a symptomatic high flow paraspinal AVM with multiple feeders remains a challenge. We report the first use of an Ascent balloon (dual lumen balloon catheter) to deliver Onyx with excellent penetration to a paraspinal AVM. PMID:23524491

  8. Paraspinal arteriovenous malformation Onyx embolization via an Ascent balloon.

    PubMed

    Martínez-Galdámez, Mario; Rodriguez-Arias, Carlos A; Utiel, Elena; Arreba, Emilio; Gonzalo, Miguel; Arenillas, Juan F

    2014-04-01

    Purely extradural lumbar spinal arteriovenous malformations (AVMs) are rare lesions that have diverse presentations and imaging features. The treatment of a symptomatic high flow paraspinal AVM with multiple feeders remains a challenge. We report the first use of an Ascent balloon (dual lumen balloon catheter) to deliver Onyx with excellent penetration to a paraspinal AVM. PMID:23543731

  9. Proximity to Pollution Sources and Risk of Amphibian Limb Malformation

    PubMed Central

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

    2005-01-01

    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

  10. Arteriovenous malformations of the brain: natural history in unoperated patients

    Microsoft Academic Search

    P M Crawford; C R West; D W Chadwick; M D Shaw

    1986-01-01

    Two-hundred and seventeen patients from a total population of 343 patients with arteriovenous malformations, were managed without surgery. Follow up was for a mean of 10.4 years. Using life survival analyses, there was a 42% risk of haemorrhage, 29% risk of death, 18% risk of epilepsy and a 27% risk of having a neurological handicap by 20 years after diagnosis

  11. A proposed angiographic classification of intracranial arteriovenous fistulae and malformations

    Microsoft Academic Search

    E. Houdart; Y. P. Gobin; A. Casasco; A. Aymard; D. Herbreteau; J. J. Merland

    1993-01-01

    We propose an angioarchitectural classification of intracranial vascular lesions as arteriovenous, arteriolovenous and arteriolovenulous fistulae. In order to validate this classification, 99 intracranial arteriovenous lesions were reviewed in 98 patients. Arteriolovenulous fistulae included 39 isolated brain arteriovenous malformations (AVMs) and 1 AVM associated with a giant arteriovenous fistula (AVF). Arteriovenous fistulae included 8 giant AVFs of the brain, 6 vein

  12. Diagnosis and Nonsurgical Management of Uterine Arteriovenous Malformation

    SciTech Connect

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

    2007-11-15

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

  13. Stereotactic radiosurgery for arteriovenous malformations of the brain

    Microsoft Academic Search

    L. Dade Lunsford; Douglas Kondziolka; John C. Flickinger; David J. Bissonette; Charles A. Jungreis; Ann H. Maitz; Joseph A. Horton; Robert J. Coffey

    1991-01-01

    Stereotactic radiosurgery successfully obliterates carefully selected arteriovenous malformations (AVM's) of the brain. In an initial 3-year experience using the 201-source cobalt-60 gamma knife at the University of Pittsburgh, 227 patients with AVM's were treated. Symptoms at presentation included prior hemorrhage in 143 patients (63%), headache in 104 (46%), and seizures in 70 (31%). Neurological deficits were present in 102 patients

  14. Brain Arteriovenous Malformations: Assessment with Dynamic MR Digital Subtraction Angiography

    Microsoft Academic Search

    Paul D. Griffiths; Nigel Hoggard; Daniel J. Warren; Iain D. Wilkinson; Bob Anderson; Charles A. Romanowski

    BACKGROUND AND PURPOSE: Conventional catheter angiography (CCA) is the current reference standard for the diagnosis, assessment, and management of pial brain arteriovenous malformations (AVMs). The purpose of this study was to develop an MR angiographic tech- nique that produces dynamic images comparable to those provided by CCA and to apply the technique to the investigation of pial brain AVMs. METHODS:

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

    PubMed

    Bolduc, Marie-Eve; Limperopoulos, Catherine

    2009-04-01

    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

  16. The North American Reporting Center for Amphibian Malformations

    NSDL National Science Digital Library

    Fowle, Suzanne C.

    The Northern Prairie Wildlife Research Center (NPWRC) has posted several more resources online. This article introduces readers to The North American Reporting Center for Amphibian Malformations and discusses availability and uses of the Center's databases and resources. The resource may be browsed online or downloaded as a .zip file.

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

    PubMed Central

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

    2014-01-01

    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

  18. [Successful management of an acquired uterine arteriovenous malformation by selective ligation of the internal iliac artery].

    PubMed

    Raherinantenaina, F; Rajaonanahary, T M A; Randriamandrato, T A V; Rakoto Ratsimba, H N

    2015-05-01

    Uterine arteriovenous malformations can be congenital or acquired. When acquired, they result from abnormal arteriovenous communication between one or more uterine arteries and a myometrial and/or endometrial venous plexus, without the interposition of a vascular nidus. Arteriovenous malformations are composed of a tortuous net of fragile low-resistant arteriovenous shunts. Uterine arteriovenous malformations create a rare and potentially life-threatening condition. The method of treatment is determined by symptoms, desire for future fertility, extent, and location of the malformation. The first treatment option for uterine arteriovenous malformation is hysterectomy, and the second option is uterine artery embolization. Selective ligation of the vessels supplying the malformation is an effective treatment option when conservative methods have failed. The present report describes a patient whose uterine arteriovenous malformation was successfully managed by selective ligation of the internal iliac artery. PMID:25778842

  19. Microsurgical replantation of a partial ear, with leech therapy.

    PubMed

    Cho, B H; Ahn, H B

    1999-10-01

    Ear reconstruction is very difficult to perform and often results in a devastating deformity. The use of microsurgical replantation techniques has allowed very favorable aesthetic results. The authors report a case of partial ear replantation without venous repair with the use of medicinal leeches to decompress the acute venous congestion that occurred during postoperative care. Medicinal leech therapy can be very useful in partial ear replantation in cases with no venous repair. PMID:10517472

  20. The middle ear mass: a rare but important diagnosis

    PubMed Central

    Pankhania, Miran; Rourke, Thomas; Draper, Mark R

    2011-01-01

    The authors report a rare case of primary intracranial meningioma presenting as a middle ear mass with conductive hearing loss. The authors aim to highlight the importance of diagnosing a middle ear mass, which although rare, may have a substantial impact on ongoing patient management. A discussion of other middle ear pathologies is made in order to demonstrate the subtle differences in presentation. PMID:22669530

  1. Image of the ear auricle in brain scintigrams

    Microsoft Academic Search

    Dag M. Solheim; Jens O. Andersen

    1977-01-01

    In a prospective study covering a 2-year period, the ear auricle was distinctly imaged in lateral 99mTc-pertechnetate brain scintiphotos\\/scintiscans in 25 of 2500 patients submitted to routine brain scintigraphy. In 6 patients with initially unremarkable scintiphotos, ear rubbing resulted in scintigraphic ear imaging in the lateral projection, indicating auricular hyperemia as a significant cause of the phenomenon. The evaluation of

  2. On hearing with more than one ear: lessons from evolution

    PubMed Central

    Schnupp, Jan W H; Carr, Catherine E

    2011-01-01

    Although ears capable of detecting airborne sound have arisen repeatedly and independently in different species, most animals that are capable of hearing have a pair of ears. We review the advantages that arise from having two ears and discuss recent research on the similarities and differences in the binaural processing strategies adopted by birds and mammals. We also ask how these different adaptations for binaural and spatial hearing might inform and inspire the development of techniques for future auditory prosthetic devices. PMID:19471267

  3. Computed Tomography Staging of Middle Ear Cholesteatoma

    PubMed Central

    Razek, Ahmed Abdel Khalek Abdel; Ghonim, Mohamed Rashad; Ashraf, Bassem

    2015-01-01

    Summary Background To establish computed tomography (CT) staging of middle ear cholesteatoma and assess its impact on the selection of the surgical procedure. Material/Methods Prospective study was conducted on 61 consecutive patients (mean age 26.8 years) with middle ear cholesteatoma. CT scan of the temporal bone and surgery were performed in all patients. CT staging classified cholesteatoma according to its location in the tympanic cavity (T); extension into the mastoid (M); and associated complications (C). Cholesteatoma was staged as stage I (T1, T2), stage II (T3, M1, M2, C1), and stage III (C2). Results The overall sensitivity of CT staging of cholesteatoma compared to surgery was 88% with excellent agreement and correlation between CT findings and intra-operative findings (K=0.863, r=0.86, P=0.001). There was excellent agreement and correlation of CT staging with surgical findings for T location (K=0.811, r=0.89, P=0.001), good for M extension (K=0.734, r=0.88, P=0.001), and excellent for associated C complications (K=1.00, r=1.0, P=0.001). Atticotympanotomy was carried out in stage I (n=14), intact canal wall surgery was performed in stage II (n=38), and canal wall down surgery was done in stage III (n=5) and stage II (n=4). Conclusions We established CT staging of middle ear cholesteatoma that helps surgeons to select an appropriate surgery.

  4. Immunosuppressive therapy for autoimmune inner ear disease

    PubMed Central

    Buniel, Maria C; Geelan-Hansen, Katie; Weber, Peter C

    2009-01-01

    Autoimmune inner ear disease (AIED) is a rare disease that is diagnosed after clinical suspicion and response to corticosteroids. AIED manifests as progressive, bilateral, although often asynchronous, sensorineural hearing loss and can be associated with vestibular symptoms. Since its description as a defined disease entity in 1979, the initial mainstay of treatment remains high-dose corticosteroids. Several animal models have been developed to assist in determining efficacy of immunosuppression in AIED, and several clinical studies have also investigated the role of both steroid and steroid-sparing treatments. Here we discuss the basic science and clinical research surrounding the history of immunosuppressive therapy in AIED. PMID:19885385

  5. The windows of the inner ear.

    PubMed

    Thomson, S; Madani, G

    2014-03-01

    The oval and round windows of the inner ear are important structures for the transmission of sound and may be affected by a variety of disease entities. The anatomy of this small area is one that often causes the radiology trainee some difficulty, but there are certain disease states that can be easily diagnosed when knowing where and how to look. As this area is very important to the otologist in a variety of preoperative settings, accurate assessment of the windows and recognition of important and potentially complex intra-operative anomalies, will greatly aid our surgical colleagues. PMID:24365668

  6. Fly-ear inspired acoustic sensors for gunshot localization

    NASA Astrophysics Data System (ADS)

    Liu, Haijun; Currano, Luke; Gee, Danny; Yang, Benjamin; Yu, Miao

    2009-05-01

    The supersensitive ears of the parasitoid fly Ormia ochracea have inspired researchers to develop bio-inspired directional microphone for sound localization. Although the fly ear is optimized for localizing the narrow-band calling song of crickets at 5 kHz, experiments and simulation have shown that it can amplify directional cues for a wide frequency range. In this article, a theoretical investigation is presented to study the use of fly-ear inspired directional microphones for gunshot localization. Using an equivalent 2-DOF model of the fly ear, the time responses of the fly ear structure to a typical shock wave are obtained and the associated time delay is estimated by using cross-correlation. Both near-field and far-field scenarios are considered. The simulation shows that the fly ear can greatly amplify the time delay by ~20 times, which indicates that with an interaural distance of only 1.2 mm the fly ear is able to generate a time delay comparable to that obtained by a conventional microphone pair with a separation as large as 24 mm. Since the parameters of the fly ear structure can also be tuned for muzzle blast and other impulse stimulus, fly-ear inspired acoustic sensors offers great potential for developing portable gunshot localization systems.

  7. Magnetically driven middle ear ossicles for optical measurement of vibrations in an ear with opened eardrum

    NASA Astrophysics Data System (ADS)

    Peacock, John; Von Unge, Magnus; Dirckx, Joris

    2012-06-01

    Vibrations of the middle ear ossicles are easily measured by means of laser vibrometry. However, exposing the ossicles requires the removal of the eardrum, with the result that the ossicles can no longer be stimulated acoustically. To overcome this we devised a new set up in which the ossicles can be driven magnetically. After measuring the response of the eardrum to an acoustic signal, we then remove the eardrum and attach a small magnet to the exposed manubrium (the part of the first auditory ossicle, the malleus, which is normally attached to the eardrum). An electromagnetic excitation coil is then used to drive the magnet, and the output to the coil adjusted until the vibration of the manubrium, as measured by the vibrometer, matches that measured in response to the acoustic signal. Such a set-up has uses in research on middle ear mechanics, such as the measurement of non-linearities in their response, as well as applications in the diagnosis of middle ear conditions such as the fixation of the ossicles by otosclerosis, or in chronic otitis media. We describe our set up in which the vibrometer unit is attached to a surgical microscope, offering accurate positioning of the laser beam. We discuss the viability of our method and its future potential by presenting some measurements on artificially fixated ears.

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

    E-print Network

    Bowyer, Kevin W.

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

  9. Vibrations in the human middle ear

    PubMed Central

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

    2011-01-01

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

  10. Congenital umbilical arterio-venous malformation: a word of caution.

    PubMed

    Gozar, Horea; Gozar, Liliana; Badiu, Catalin Constantin; Suciu, Horatiu

    2014-05-01

    We report on an 18-month old girl who presented in good clinical shape with a pulsatile tumour in the umbilical area which had a shape and localization similar to that of an umbilical bowel hernia. The Doppler ultrasound of the umbilical tumour revealed a large arterio-venous vascular malformation with a haemodynamically significant blood shunting. Furthermore, the inferior caval vein and the hepatic veins were dilated. Computed tomography angiography revealed permeable umbilical veins and arteries communicating within a large dilated arterio-venous fistula. The growing tumour was excised without any perioperative complications. Further postoperative recovery was uneventful and the baby was discharged 10 days after surgery. We advocate careful antenatal ultrasound evaluation of these vascular malformations. Early surgical removal in newborns is vital in order to avoid severe complications. PMID:24550232

  11. Giant mesenteric lymphatic malformation presenting as small bowel volvulus

    PubMed Central

    Cauley, Christy E.; Spencer, Philip J.; Sagar, Pallavi; Goldstein, Allan M.

    2013-01-01

    Abdominal pain with bilious emesis is an ominous clinical presentation with many possible causes. We describe a previously healthy 4-year-old boy who presented with these symptoms and ultrasound findings of fluid throughout most of the abdominal cavity. Computed tomography imaging revealed a large cystic mass (21-by-13 cm) associated with a small bowel obstruction due to volvulus. A laparoscopic exploration was undertaken, revealing a large mass arising from the small intestinal mesentery and causing a segmental volvulus of the small bowel. Conversion to mini-laparotomy allowed reduction of the volvulus and segmental resection of the small bowel associated with a giant mesenteric lymphatic malformation. This case describes a rare cause of intestinal volvulus due to a mesenteric lymphatic malformation. PMID:24963906

  12. Congenital malformations of the vertebral column in ancient amphibians.

    PubMed

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

    2014-04-01

    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

  13. Management of Cerebral Cavernous Malformations: From Diagnosis to Treatment

    PubMed Central

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

    2015-01-01

    Cerebral cavernous malformations are the most common vascular malformations and can be found in many locations in the brain. If left untreated, cavernomas may lead to intracerebral hemorrhage, seizures, focal neurological deficits, or headaches. As they are angiographically occult, their diagnosis relies on various MR imaging techniques, which detect different characteristics of the lesions as well as aiding in planning the surgical treatment. The clinical presentation and the location of the lesion are the most important factors involved in determining the optimal course of treatment of cavernomas. We concisely review the literature and discuss the advantages and limitations of each of the three available methods of treatment—microsurgical resection, stereotactic radiosurgery, and conservative management—depending on the lesion characteristics. PMID:25629087

  14. Pulmonary arteriovenous malformations. Case reports and literature review.

    PubMed

    Grahmann, P R; Bonnet, R; Swoboda, L; Nöldge, G; Hasse, J

    1994-05-01

    Pulmonary arteriovenous malformations (PAVM) represent an uncommon disease with only 500 reported cases. To emphasize the resectional surgical standard, 2 patients with PAVM and pulmonary right-to-left shunt are presented and the optional treatments discussed. One patient had suffered from a cerebrovascular accident. The other patient's diagnosis resulted from a coincidental finding in connection with an unrelated illness. Because of the risk of acutely developing complications, especially disabling or fatal cerebral ischemia, therapy is generally recommended even in asymptomatic patients. The 2 patients presented here were treated by resection. Surgical treatment with a very low risk and parenchyma-sparing technique remains the golden standard for large isolated malformations. In addition to the established and reliable operative therapy, since 1978 catheter embolization is becoming the method of choice with an increasing range of indications in those centers experienced with this technique. PMID:8172388

  15. [Port wine stains or capillary malformations: surgical treatment].

    PubMed

    Berwald, C; Salazard, B; Bardot, J; Casanova, D; Magalon, G

    2006-01-01

    Capillary malformations do not demand mostly any therapeutics. For aesthetic reasons, family or child can demand a treatment to ease even to remove the unsightly character of the lesion. In this context, the means employees must be simple and not engender aftereffects more unaesthetic than the lesion. The pulsed dye laser fulfils perfectly this conditions by improving the color of the lesion without touching the texture of the skin. However it's a treatment requiring many sessions over 2-3 years. Surgery keeps an interest for the treatment of capillary malformations resistant to laser (in particular on the limbs) or to treat soft tissues hyperplasia met in certain cervicofacial locations. The surgery uses the whole techniques of plastic surgery classified from the most simple to the most complicated: excision-suture in one time or iterative, excision and coverage by a skin graft, use of skin expansion techniques with local flaps. PMID:17005311

  16. Management of cerebral cavernous malformations: from diagnosis to treatment.

    PubMed

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

    2015-01-01

    Cerebral cavernous malformations are the most common vascular malformations and can be found in many locations in the brain. If left untreated, cavernomas may lead to intracerebral hemorrhage, seizures, focal neurological deficits, or headaches. As they are angiographically occult, their diagnosis relies on various MR imaging techniques, which detect different characteristics of the lesions as well as aiding in planning the surgical treatment. The clinical presentation and the location of the lesion are the most important factors involved in determining the optimal course of treatment of cavernomas. We concisely review the literature and discuss the advantages and limitations of each of the three available methods of treatment--microsurgical resection, stereotactic radiosurgery, and conservative management--depending on the lesion characteristics. PMID:25629087

  17. Seizure control after radiosurgery on cerebral arteriovenous malformations

    Microsoft Academic Search

    Yoshihisa Kida; Tatsuya Kobayashi; Takayuki Tanaka; Yoshimasa Mori; Tosinori Hasegawa; Toshiki Kondoh

    2000-01-01

    Among 462 cases of cerebral arteriovenous malformation (AVM) treated with gamma-radiosurgery, the initial presentations were haemorrhage in 68%, epilepsy in 12.8%, neurological deficits in 3.2%, minor symptoms in 7.6% and asymptomatic in 4.5% respectively. There were 79 cases (17.1%) who had had a convulsive seizure before radiosurgery and they were classified into two groups: 58 cases presented with seizure as

  18. Congenital cystoid adenomatoid malformation: Surgery in a young child.

    PubMed

    Hasnain Fatimi, Saulat; Asif Ali, Taimur; Majid, Zain; Deedar Ali, Ranish

    2015-07-01

    We present the case of a 3.5-year-old child who presented with recurrent chest infections and fever since birth. Antenatal ultrasonography had shown that she had a congenital cystic malformation of the left lower lobe of her lung. She was initially managed conservatively, and after a couple of years, underwent an uneventful left lower lobectomy via a posterolateral thoracotomy. She did very well after the procedure and her symptoms resolved significantly. PMID:25505008

  19. Present State of Microneurosurgery of Cerebral Arteriovenous Malformations

    Microsoft Academic Search

    Juha Hernesniemi; Rossana Romani; Martin Lehecka; Puchong Isarakul; Reza Dashti; Özgür Celik; Ondrej Navratil; Mika Niemelä; Aki Laakso

    Microneurosurgical excision is known to be the definitive treatment for brain arteriovenous malformation (AVMs). The most\\u000a important factors governing the operability of an AVM are location, size, age of the patient, and the neurosurgeon’s and team’s\\u000a experience. We present in this review the surgical experience of the senior author (JH) in microneurosurgical treatment of\\u000a brain AVMs. This consists of the

  20. Coexistence of an intracranial meningioma and an arteriovenous malformation

    PubMed Central

    Honeybul, Stephen

    2015-01-01

    The occurrence of a primary brain tumour in association with a cerebral arteriovenous malformation (AVM) is a recognized but rarely reported finding. A 56-year-old female presented following a single tonic clonic seizure. Radiological investigations revealed a left posterior frontal parafalcine meningioma and a left parietal AVM. Both were uneventfully resected. Whether there is a causal relationship is unproven, however, this case report might lend some support to this hypothesis given the relatively close proximity of the two lesions.

  1. Fetal Facial Defects: Associated Malformations and Chromosomal Abnormalities

    Microsoft Academic Search

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

    1993-01-01

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

  2. Embolotherapy of Large Pulmonary Arteriovenous Malformations: Long-Term Results

    Microsoft Academic Search

    Daniel W. Lee; Robert I. White; Thomas K. Egglin; Jeffrey S. Pollak; Pierre B. Fayad; Joel A. Wirth; Melvin M. Rosenblatt; Kevin W. Dickey; Catherine M. Burdge

    1997-01-01

    Background. The purpose of this study was to document the long-term results of transcatheter embolotherapy of large pulmonary arteriovenous malformations (PAVMs).Methods. From a data base of 221 consecutive patients with PAVMs treated by embolotherapy between 1978 and 1995, 45 patients with 52 PAVMs, supplied by feeding arteries 8 mm in diameter or larger, were selected for a retrospective investigation.Results. Of

  3. Coexistence of an intracranial meningioma and an arteriovenous malformation.

    PubMed

    Honeybul, Stephen

    2015-01-01

    The occurrence of a primary brain tumour in association with a cerebral arteriovenous malformation (AVM) is a recognized but rarely reported finding. A 56-year-old female presented following a single tonic clonic seizure. Radiological investigations revealed a left posterior frontal parafalcine meningioma and a left parietal AVM. Both were uneventfully resected. Whether there is a causal relationship is unproven, however, this case report might lend some support to this hypothesis given the relatively close proximity of the two lesions. PMID:26060239

  4. Pediatric stroke: the importance of cerebral arteriopathy and vascular malformations

    Microsoft Academic Search

    Lauren A. Beslow; Lori C. Jordan

    2010-01-01

    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

  5. Cerebral arteriovenous malformation: prenatal and postnatal central blood flow dynamics

    Microsoft Academic Search

    D. J. Patton; J-C Fouron

    1995-01-01

    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.

  6. Thoracoscopic anatomical resection of congenital lung malformations in adults

    PubMed Central

    Macias, Lidia; Ojanguren, Amaia; Dahdah, Julien

    2015-01-01

    Congenital lung malformations (CLM) are a heterogeneous group of disorders that may require surgical resection to prevent complications. Thoracoscopic resection of CLM has been reported in infants. Our goal was to state whether it can also be a viable option in adults. Between 2007 and 2014, 11 patients had a thoracoscopic resection of a CLM (six lobectomies and five anatomic segmentectomies) with satisfactory results. Although being more challenging in adults due to infectious sequellae, this approach is safe. PMID:25922729

  7. Congestive hepatopathy secondary to large renal arteriovenous malformation

    PubMed Central

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

    2013-01-01

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

  8. Congestive hepatopathy secondary to large renal arteriovenous malformation.

    PubMed

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

    2013-01-01

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

  9. Transcranial colour-coded duplex sonography of cerebral arteriovenous malformations

    Microsoft Academic Search

    R. W. Baumgartner; H. P. Mattle; G. Schroth

    1996-01-01

    A transcranial colour-coded duplex sonography (TCCD) study was performed to evaluate the sensitivity of detection and the\\u000a feasibility of visualising details of cerebral arteriovenous malformations (AVMs). We prospectively examined 15 patients with\\u000a 2 large (> 4 cm), 7 medium-size (2–4 cm) and 6 small (< 2 cm) radiologically proven supratentorial AVMs of the brain using\\u000a TCCD. A feeding vessel was

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

    E-print Network

    Allen, Jont

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

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

    E-print Network

    2006-01-01

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

  12. Heterozygous Mutations of OTX2 Cause Severe Ocular Malformations

    PubMed Central

    Ragge, Nicola K.; Brown, Alison G.; Poloschek, Charlotte M.; Lorenz, Birgit; Henderson, R. Alex; Clarke, Michael P.; Russell-Eggitt, Isabelle; Fielder, Alistair; Gerrelli, Dianne; Martinez-Barbera, Juan Pedro; Ruddle, Piers; Hurst, Jane; Collin, J. Richard O.; Salt, Alison; Cooper, Simon T.; Thompson, Pamela J.; Sisodiya, Sanjay M.; Williamson, Kathleen A.; FitzPatrick, David R.; Heyningen, Veronica van; Hanson, Isabel M.

    2005-01-01

    Major malformations of the human eye, including microphthalmia and anophthalmia, are examples of phenotypes that recur in families yet often show no clear Mendelian inheritance pattern. Defining loci by mapping is therefore rarely feasible. Using a candidate-gene approach, we have identified heterozygous coding-region changes in the homeobox gene OTX2 in eight families with ocular malformations. The expression pattern of OTX2 in human embryos is consistent with the eye phenotypes observed in the patients, which range from bilateral anophthalmia to retinal defects resembling Leber congenital amaurosis and pigmentary retinopathy. Magnetic resonance imaging scans revealed defects of the optic nerve, optic chiasm, and, in some cases, brain. In two families, the mutations appear to have occurred de novo in severely affected offspring, and, in two other families, the mutations have been inherited from a gonosomal mosaic parent. Data from these four families support a simple model in which OTX2 heterozygous loss-of-function mutations cause ocular malformations. Four additional families display complex inheritance patterns, suggesting that OTX2 mutations alone may not lead to consistent phenotypes. The high incidence of mosaicism and the reduced penetrance have implications for genetic counseling. PMID:15846561

  13. Environmental monitoring using malformed embryos of the amphipod Monoporeia affinis

    SciTech Connect

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

    1995-12-31

    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.

  14. Multimodality intraoperative neurophysiological monitoring during Onyx embolization of cerebrovascular malformations.

    PubMed

    Deshaies, Eric M; Singla, Amit; Allott, Geoffrey; Villwock, Mark R; Li, Fenghua; Gorji, Reza

    2015-03-01

    General anesthesia prohibits neurological examination during embolization of cerebrovascular malformations when provocative testing prior to pedicle occlusion is needed. Intraoperative neurophysiological monitoring (IONM) has the potential to fill this gap but remains relatively unexplored. We conduct a retrospective review of consecutive patients with cerebrovascular malformations treated with Onyx (ethylene vinyl alcohol copolymer, dissolved in dimethyl sulfoxide) embolization under general anesthesia with IONM from 2009 to 2012. Somatosensory evoked potentials (SSEPs), transcranial motor evoked potentials (TcMEPs), visual evoked potential (VEPs), auditory brainstem response (ABR), and electroencephalography (EEG) were used selectively in all patients depending on the location of the malformation. Provocative testing combined with IONM was performed in 28 patients over 75 sessions. Three patients demonstrated changes in TcMEPs or ABR during provocative testing, which halted the planned embolization. Two patients demonstrated changes in baseline SSEPs after embolization, despite normal IONM during provocative testing, correlating with postprocedural contralateral weakness. Six patients developed visual deficits after arterial occlusion despite unchanged VEPs and occipital EEG during provocative testing and embolization. We therefore conclude that the sensitivity of TcMEPs and SSEPs is preferable to EEG, and we strongly caution against relying on occipital recorded VEPs to predict visual deficits. PMID:26036117

  15. OBESITY AND THE RISK AND DETECTION OF FETAL MALFORMATIONS

    PubMed Central

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

    2012-01-01

    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

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

    Technology Transfer Automated Retrieval System (TEKTRAN)

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

  17. Replantation of an avulsed ear, using a single arterial anastamosis.

    PubMed

    O'Toole, G; Bhatti, K; Masood, S

    2008-01-01

    Avulsion of the ear is relatively uncommon and replantation a technical challenge. A case in which an avulsed ear was successfully replanted using a single arterial anastamosis is described. The surgical difficulties encountered, the pharmaceutical approach to postoperative care and the problems which resulted from the lack of venous drainage are discussed. PMID:18061545

  18. Sound direction estimation using an artificial ear for robots

    Microsoft Academic Search

    Sungmok Hwang; Youngjin Park; Youn-sik Park

    2011-01-01

    We propose a novel design of an artificial robot ear for sound direction estimation using two measured outputs only. The spectral features in the interaural transfer functions (ITFs) of the proposed artificial ears are distinctive and move monotonically according to the sound direction. Thus, these features provide effective sound cues to estimate sound direction using the measured two output signals.

  19. Artificial Robot Ear Design for Sound Direction Estimation

    Microsoft Academic Search

    Youngjin Park; Sungmok Hwang

    2007-01-01

    We propose a novel design of artificial robot ear for sound direction estimation using measured two outputs only. The spectral features in head-related transfer functions and in interaural transfer functions (ITFs) are distinctive in the voice frequency band. Thus, these features provide effective sound cues to estimate the sound direction using two measured ear outputs only without input information. Especially,

  20. Mechanism and Rate of Middle Ear Fluid Absorption

    Microsoft Academic Search

    Petia Petrova; Sharon Freeman; Haim Sohmer

    2007-01-01

    Several mechanisms have been suggested to explain the clearance of fluids from the middle ear. These include a pumping action through the eustachian tube, mucociliary beating through the tube, outflow of water to the blood due to osmotic gradients and an active Na+ transport driving water absorption. In order to assess these mechanisms, the middle ear cavity of paralyzed, ventilated

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

    PubMed Central

    Chapman, Susan Caroline

    2010-01-01

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

  2. Rhabdomyosarcoma in middle ear of an adult: a rare presentation.

    PubMed

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

    2012-01-01

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

  3. The correlation of middle ear aeration with mastoid pneumatization

    Microsoft Academic Search

    J. Sadé

    1992-01-01

    Atelectatic ears, which by definition are poorly aerated, are also usually associated with poor mastoid pneumatization. On the other hand, otosclerotic patients, whose middle ears are usually exceptionally well aerated, also have excellent mastoid pneumatization. Three unusual cases are presented, in which partial atelectasis developed in stapedectomized patients. In each case the mastoid was later found to be nonpneumatized, and

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

    PubMed

    Röhm, E; Adam, E

    1986-01-01

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

  5. Alternative Ear-Canal Measures Related to Absorbance

    PubMed Central

    Neely, Stephen T.; Stenfelt, Stefan; Schairer, Kim S.

    2013-01-01

    Several alternative ear-canal measures are similar to absorbance in their requirement for prior determination of a Thévenin-equivalent sound source. Examples are (1) sound intensity level (SIL), (2) forward-pressure level (FPL), (3) time-domain ear-canal reflectance (TDR), and (4) cochlear reflectance (CR). These four related measures are similar to absorbance in their utilization of wide-band stimuli and their focus on recording ear-canal sound pressure. The related measures differ from absorbance in how the ear-canal pressure is analyzed and in the type of information that is extracted from the recorded response. SIL and FPL have both been shown to be better as measures of sound level in the ear canal compared to sound pressure level (SPL) because they reduced calibration errors due to standing waves in studies of behavioral thresholds and otoacoustic emissions. TDR may be used to estimate ear-canal geometry and may have the potential to assess middle-ear pathology. CR reveals information about the inner ear that is similar to what is provided by other types of otoacoustic emissions and may have theoretical advantages that strengthen its interpretation. PMID:23900185

  6. Pedigree selection for Gibberella ear rot resistance in maize

    Microsoft Academic Search

    Daniel A. Presello; Lana M. Reid; Gail Butler; Diane E. Mather

    2005-01-01

    The pedigree method is often used for developing inbred lines in maize (Zea mays L.). This study was conducted to assess the effectiveness of pedigree selection for improving resistance to Gibberella ear rot in four maize populations. Selection was based on the severity of ear rot symptoms after inoculation with macroconidial suspensions of Fusarium graminearum (Schwabe) into the silk channel

  7. Middle Ear Resonance and Acoustic Immittance Measures in Children.

    ERIC Educational Resources Information Center

    Hanks, Wendy D.; Rose, Katie J.

    1993-01-01

    This study established a normal middle ear resonance estimated from sweep frequency tympanometry, established normal equivalent ear canal volume, static acoustic admittance, and tympanometric peak pressure at 226 hertz in 90 children with normal hearing and 68 children with deafness, ages 6-15. No significant intergroup or age differences were…

  8. Preventing Cauliflower Ear with a Modified Tie-Through Technique.

    ERIC Educational Resources Information Center

    Dimeff, Robert J.; Hough, David O.

    1989-01-01

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

  9. Acute respiratory arrest. A complication of malformation of the shunt in children with myelomeningocele and Arnold-Chiari malformation.

    PubMed

    Tomita, T; McLone, D G

    1983-02-01

    Three children had hydrocephalus associated with myelomeningocele and the Arnold-Chiari malformation. They all experienced neck pain and opisthotonos, followed by the sudden onset of respiratory arrest as a result of the malfunction of a CSF shunt. There were no changes in pupillary response, and each patient remained alert until shortly before the respiratory arrest. Prompt surgical revision of the CSF shunt was carried out, and all three patients recovered. The mechanism of acute respiratory arrest seems to be acute compression of the brainstem as a result of hydrocephalus. The supratentorial pressure is direct on the posterior fossa structures through the enlarged tentorial opening, which is one of the characteristics of the brain in the Arnold-Chiari malformation. Sudden respiratory arrest, a life-threatening complication, is a result of a malfunction of the CSF shunt in children with myelomeningocele and requires prompt surgical decompression. PMID:6849301

  10. Ear Infection and Hearing Loss Amongst Headphone Users

    PubMed Central

    Mazlan, R.; Saim, L.; Thomas, A.; Said, R.; Liyab, B.

    2002-01-01

    The use of headphone has been thought to cause infection in the ear canal and contribute to hearing loss. In this study, we examined 136 Customer Service Representative from Celcom (Malaysia) Sdn. Bhd. who use headphone throughout their working hours. The purpose of this study was to determine the prevalence of ear canal infection and other related diseases of the ear, nose and throat. Their hearing thresholds were also determined using the Amplaid 309 Clinical Audiometer. We found no incidence of infection of the external ear canal amongst the subjects. There were 4 cases of chronic middle ear infection and 4 cases of impacted wax. Hearing impairment was found in 25 subjects (21.2%). However, there was no significant association between hearing loss and the exposure to sound from headphone usage because the high frequencies were not predominantly affected. There was also no association between hearing loss and duration of service. PMID:22844220

  11. Recurrent syncope and chronic ear pain.

    PubMed

    Clegg, Andrew; Daverede, Luis; Wong, Winson; Loney, Elizabeth; Young, John

    2010-01-01

    An elderly gentleman presented to hospital with recurrent blackout episodes consistent with syncope and a 3-month history of right ear pain. Significant postural hypotension was recorded. White cell count and C reactive protein were elevated. MRI of the head and neck revealed a soft tissue abnormality in the right nasopharynx and base of skull. Tissue biopsies were obtained and microbiology specimens revealed a mixed growth of pseudomonas and diphtheroids. There was no histological evidence of malignancy. A diagnosis of skull base infection was made. Infective involvement of the carotid sinus was considered to be the cause of the recurrent syncope and postural hypotension. The patient responded well to a 12-week course of intravenous meropenem. Inflammatory markers returned to normal and a repeat MRI after 3 months of treatment showed significant resolution of infection. The syncopal episodes and orthostatic hypotension resolved in parallel with treatment of infection. PMID:22791782

  12. Clinical treatment of vascular inner ear diseases.

    PubMed

    Hultcrantz, E

    1988-01-01

    A multitude of general disorders of the vascular system may also affect the blood circulation of the cochlea and cause symptoms such as fluctuating or permanent hearing loss. Such is the case for arteriosclerosis combined with hypertension or hypotension, collagenosis, and diabetes. Blood disorders, like leukemia, sickle cell anemia, and polycythemia, and infectious diseases involving the blood vessels, such as lues, may also present their primary symptoms in the ear. The otorhinolaryngologist must be able to establish the correct diagnosis and refer patients requiring more general treatment to other specialists. The use of specific vasoactive treatment should be continued to those patients with symptoms of acute or fluctuating hearing loss, vertigo, or tinnitus who exhibit no other signs. Modern techniques for cochlear blood flow measurements have verified that several of the treatment protocols in use, which have a sound theoretical background, do indeed increase cochlear blood flow. PMID:3067594

  13. Analysis of Earing in Deep Drawn Cups

    SciTech Connect

    Aretz, Holger; Aegerter, Johannes; Engler, Olaf [Hydro Aluminium Deutschland GmbH, Research and Development, Georg-von-Boeselager-Str. 21, D-53117 Bonn (Germany)

    2010-06-15

    The cup-drawing of a strongly anisotropic sheet metal is simulated using a commercial finite element software along with a user material subroutine. In order to accurately describe the plastic anisotropy of the material the well-known recent yield function 'Yld2004-18p' is extended. Regarding the experimental characterization of the considered material the occurrence of dynamic strain aging lead to an oscillating signal of the width change of the tensile samples, which prevented a reliable determination of plastic strain ratios (r-values). Thus, an improved measurement concept was developed that leads to a very robust and reproducible determination of r-values. Furthermore, a novel plane-strain tensile test sample is presented which is used for the characterization of the plastic anisotropy in biaxial loading states. A quantitative comparison with measured earing profiles of deep drawn cups illustrates the predictive capabilities of the numerical simulation.

  14. Relapsing Polychondritis: Inflamed Joints and Ears

    PubMed Central

    Meliko?lu, Meltem Alkan; ?enel, Kaz?m

    2015-01-01

    Background: Relapsing polychondritis (RP) is an episodic and progressive inflammatory disease of the cartilaginous structures, including elastic cartilage of the ear and nose, hyaline cartilage of the peripheral joints, fibrocartilage at axial sites, and cartilage of the tracheo-bronchial tree. The spectrum of its presentations may vary from intermittent mild episodes of chondritis to occasional organ involvement or even life-threatening manifestations. Case Report: We presented a 64 year-old male patient with bilaterally knee arthritis and discoloration of pinna. Conclusion: There is lack of awareness about this disease due to its rarity. With this case presentation, our goal was to draw attention to this disease, which could be delayed for the diagnosis. PMID:25759785

  15. Tuberculosis of the ear, a professional disease?

    PubMed

    Sens, Patrícia Maria; Almeida, Clemente I R; Valle, Lupércio O do; Costa, Luís H C; Angeli, Miguel L S

    2008-01-01

    Tuberculosis is a rare cause of chronic suppurative otitis media and mastoiditis; the predisposing factors of this association, however, are not commonly described. There has been an alarming increase in the incidence of tuberculosis in Brazil, including tuberculous otitis media. These patients typically present multiple perforations of the tympanic membrane, an ear discharge, and progressive hearing loss. This diagnosis should be taken into account in patients that do not respond to routine therapy for fungal external otitis or bacterial otitis media. In this retrospective study, the authors describe four cases of patients with tuberculous otitis media. This sample consisted of two physicians, a chemical engineer and an underage child in whose family there were cases of active tuberculosis. Predisposing factors for tuberculous otitis were contact with family members that had tuberculosis, professional contact with patients and exposure to pathogenic microorganisms in airways. PMID:18852993

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

    SciTech Connect

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

    1999-12-01

    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.

  17. Sensitivity of patients with familial cerebral cavernous malformations to therapeutic radiation.

    PubMed

    Golden, Michael; Saeidi, Saba; Liem, Benny; Marchand, Eric; Morrison, Leslie; Hart, Blaine

    2015-02-01

    Familial cerebral cavernous malformations are autosomal dominant conditions that can result in significant morbidity. A two-hit mechanism is accepted as likely responsible for formation of these malformations. We present two patients with this disease who received therapeutic radiation and developed very high numbers of malformations within the radiation ports, supporting radiation as an accelerator of lesion formation and suggesting implications for risks of radiation in this disease. PMID:25565562

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

    PubMed Central

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

    2014-01-01

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

  19. Trigeminal neuralgia secondary to Chiari's malformation—treatment with ventriculoperitoneal shunt

    Microsoft Academic Search

    Kanna Gnanalingham; Shabin Man Joshi; Ben Lopez; Habib Ellamushi; Peter Hamlyn

    2005-01-01

    BackgroundTrigeminal neuralgia (TN) is most commonly related to vascular compression of the trigeminal nerve. Trigeminal neuralgia associated with Chiari's malformation and associated hydrocephalus are rare.

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

    PubMed

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

    2014-08-01

    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

  1. Combined Effect of Fluid and Pressure on Middle Ear Function

    PubMed Central

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

    2008-01-01

    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

  2. Middle ear transmission in the grass frog, Rana temporaria.

    PubMed

    Jørgensen, M B; Kanneworff, M

    1998-01-01

    The anuran middle ear serves to transmit eardrum vibrations to the inner ear. In order to do this efficiently, the eardrum and middle ear must operate as an impedance transformer matching the low impedance of air to the higher impedance of the fluid-filled inner ear. In amniotes, one of the mechanisms used to achieve impedance transformation is to have the middle ear work as a force-amplifying lever system. Here, we present evidence that the grass frog middle ear also implements a lever system. The columellar footplate, which sits in the oval window, is firmly connected to the otic capsule along its ventral edge. Therefore, simple in-out movement of the columella is prevented while a rotational movement around the footplate's ventral edge is possible. The latter movement pattern was confirmed by laser vibrometry measurements of eardrum and footplate vibrations. The results showed that the footplate vibrations were 20-30 dB weaker than those of the eardrum and that the two structures vibrated 180 degrees out of phase (at low frequencies). The lever ratio was approximately 6, i.e. somewhat higher than lever ratios reported for amniotes. Hence, the middle ear lever probably makes a significant contribution to impedance matching in frogs. PMID:9447714

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

    PubMed Central

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

    2014-01-01

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

  4. Microsurgical replantation of a partial helix of the ear.

    PubMed

    Kim, Kwang Seog; Kim, Eui Sik; Hwang, Jae Ha; Lee, Sam Yong

    2009-01-01

    Microsurgical ear replantation is a significant challenge because vessel sizes are diminutive. Furthermore, as ear vessels are larger in the medial portion than in the lateral portion, microsurgical replantation of a replant including only the helix is extremely difficult. The authors replanted a partial helix of the ear using a single arterial anastomosis. As no suitable veins could be identified, medicinal leech therapy and systemic heparinization were used to achieve venous drainage. The replanted helix survived completely and the cosmetic result was excellent. Microsurgical replantation should be considered the treatment of choice in helix amputation cases, even though amount of replant is small. (c) 2009 Wiley-Liss, Inc. Microsurgery 2009. PMID:19306389

  5. Shaping sound in space: the regulation of inner ear patterning

    PubMed Central

    Groves, Andrew K.; Fekete, Donna M.

    2012-01-01

    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

  6. Vasospasm after intraventricular hemorrhage caused by arteriovenous malformation

    PubMed Central

    Tseng, Wei-Lung; Tsai, Yi-Hsin

    2015-01-01

    Intraventricular hemorrhage (IVH) induced vasospasm is rare in clinical practice. We report a case with vasospasm 10 days after IVH due to thalamic bleeding from an arteriovenous malformation. The vasospasm was so severe that transarterial embolization failed. Follow-up angiography could not reveal the vascular lesion and spontaneous thrombosis was considered. The discussion includes literature review and possible mechanism of such phenomenon. IVH-related vasospasm is rare but should always be kept in mind. Early detection with proper treatment provides better clinical outcome in such cases. PMID:25972942

  7. Convergence nystagmus associated with Arnold-Chiari malformation.

    PubMed

    Mossman, S S; Bronstein, A M; Gresty, M A; Kendall, B; Rudge, P

    1990-03-01

    A case of convergence nystagmus associated with an Arnold-Chiari type I malformation is presented. The nystagmus appeared in the absence of fixation, was provoked during Valsalva's maneuver and neck flexion and extension, and attenuated on deep inspiration. Sagittal magnetic resonance images showed that the diameter of the cerebral aqueduct increased with the neck in full flexion and in full extension. Surgical foramen magnum decompression considerably reduced the nystagmus and abolished the postural variation of aqueduct diameter. It is postulated that this nystagmus was due to a combination of mechanical distortion and abnormal transmission of cerebrospinal fluid pressure to the aqueductal region. PMID:2310320

  8. Brain Arteriovenous Malformation Modeling, Pathogenesis and Novel Therapeutic Targets

    PubMed Central

    Chen, Wanqiu; Choi, Eun-Jung; McDougall, Cameron M.; Su, Hua

    2014-01-01

    Patients harboring brain arteriovenous malformation (bAVM) are at life-threatening risk of rupture and intracranial hemorrhage (ICH). The pathogenesis of bAVM has not been completely understood. Current treatment options are invasive and ? 20% of patients are not offered interventional therapy because of excessive treatment risk. There are no specific medical therapies to treat bAVMs. The lack of validated animal models has been an obstacle for testing hypotheses of bAVM pathogenesis and testing new therapies. In this review, we summarize bAVM model development; and bAVM pathogenesis and potential therapeutic targets that have been identified during model development. PMID:24723256

  9. Onyx embolization for the treatment of brain arteriovenous malformations

    Microsoft Academic Search

    Feng Xu; Wei Ni; Yujun Liao; Yuxiang Gu; Bin Xu; Bing Leng; Donglei Song

    2011-01-01

    Background  Onyx has emerged in recent years for the endovascular treatment of brain arteriovenous malformations (AVMs). However, the\\u000a role of Onyx embolization is still under discussion. We report our initial experiences in the treatment of brain AVMs with\\u000a Onyx embolization.\\u000a \\u000a \\u000a \\u000a \\u000a Methods  Between January 2004 and December 2007, 86 patients with brain AVMs were embolized with Onyx. Clinical presentation included\\u000a intracerebral hemorrhage in

  10. Cesarean scar pregnancy: a rare cause of uterine arteriovenous malformation.

    PubMed

    Akbayir, Ozgur; Gedikbasi, Ali; Akyol, Alpaslan; Ucar, Adem; Saygi-Ozyurt, Sezin; Gulkilik, Ahmet

    2011-01-01

    A 38-year-old gravida 4, para 2 woman with a history of two Cesarean sections and one curettage was referred to our hospital, because of painless vaginal bleeding and 6 weeks + 2 days of amenorrhea. The first diagnosis was Cesarean scar pregnancy, managed with methotrexate. Subsequently, an arteriovenous malformation developed, which was diagnosed with color Doppler imaging. The diagnosis was confirmed with angiography. Successful bilateral uterine artery embolization was performed with ethylene vinyl alcohol copolymer (Onyx), n-butyl-2-cyanoacrylate (Histoacryl), and gelfoam. PMID:21647920

  11. Ethnic differences in congenital malformations in the Netherlands: analyses of a 5-year birth cohort.

    PubMed

    Anthony, S; Kateman, H; Brand, R; den Ouden, A L; Dorrepaal, C A; van der Pal-de Bruin, K M; Buitendijk, S E

    2005-03-01

    Congenital malformations are among the major causes of perinatal mortality and morbidity at present. Research into the ethnic diversity of congenital malformations can form a basis both for aetiological studies and for health care advice and planning. This study compared the overall prevalence of congenital malformations, the prevalence in different organ systems and of several specific malformations between different maternal ethnic groups in the Netherlands using a 5-year national birth cohort (1996-2000) containing 881 800 births. Maternal ethnic groups considered were Dutch; Mediterranean (Moroccan/Turkish); other European; Black; Hindu and Asian. Mediterranean women had a 20% higher risk of having a child with a congenital malformation than Dutch women (age-adjusted OR = 1.21 [95% CI 1.16, 1.27]). They showed an increased risk of malformations in several organ systems such as the central nervous system and sensory organs, the urogenital system and skin and abdominal wall. Further, they had an increased risk of the group of chromosomal malformations/multiple malformations/syndromes. For the specific group of multiple malformations the maternal age adjusted OR was 1.80 [95% CI 1.47, 2.20]. The Black group showed a significantly increased risk of skeletal and muscular malformations (age adjusted OR = 1.76 [95% CI 1.53, 2.02]) with a sixfold increased risk of polydactyly compared with the Dutch group. For Mediterranean women, the largest and fastest growing group of immigrants in the Netherlands, this study demonstrated an increased risk of congenital malformations. PMID:15787888

  12. Green laser light activates the inner ear.

    PubMed

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

    2009-01-01

    The hearing performance with conventional hearing aids and cochlear implants is dramatically reduced in noisy environments and for sounds more complex than speech (e. g. music), partially due to the lack of localized sensorineural activation across different frequency regions with these devices. Laser light can be focused in a controlled manner and may provide more localized activation of the inner ear, the cochlea. We sought to assess whether visible light with parameters that could induce an optoacoustic effect (532 nm, 10-ns pulses) would activate the cochlea. Auditory brainstem responses (ABRs) were recorded preoperatively in anesthetized guinea pigs to confirm normal hearing. After opening the bulla, a 50-microm core-diameter optical fiber was positioned in the round window niche and directed toward the basilar membrane. Optically induced ABRs (OABRs), similar in shape to those of acoustic stimulation, were elicited with single pulses. The OABR peaks increased with energy level (0.6 to 23 microJ/pulse) and remained consistent even after 30 minutes of continuous stimulation at 13 microJ, indicating minimal or no stimulation-induced damage within the cochlea. Our findings demonstrate that visible light can effectively and reliably activate the cochlea without any apparent damage. Further studies are in progress to investigate the frequency-specific nature and mechanism of green light cochlear activation. PMID:19725719

  13. Why Internally Coupled Ears (ICE) Work Well

    NASA Astrophysics Data System (ADS)

    van Hemmen, J. Leo

    2014-03-01

    Many vertebrates, such as frogs and lizards, have an air-filled cavity between left and right eardrum, i.e., internally coupled ears (ICE). Depending on source direction, internal time (iTD) and level (iLD) difference as experienced by the animal's auditory system may greatly exceed [C. Vossen et al., JASA 128 (2010) 909-918] the external, or interaural, time and level difference (ITD and ILD). Sensory processing only encodes iTD and iLD. We present an extension of ICE theory so as to elucidate the underlying physics. First, the membrane properties of the eardrum explain why for low frequencies iTD dominates whereas iLD does so for higher frequencies. Second, the plateau of iTD = ? ITD for constant 1 < ? < 5 and variable input frequency

  14. Image analysis of the human inner ear.

    PubMed

    Kubo, T; Anniko, M; Hsu, W J

    1998-01-01

    The KS 300 is a multifunctional software image analysis system using an object-oriented programming environment. The possibility of its application for the inner ear was studied by using specimens from humans and squirrel monkeys, immunostained for the brain-derived calcium-binding protein, S-100 protein. Grey images were used for measurements. The cell borders were outlined by hand, using a digitizer. The absolute grey values of the pixels changed when the brightness of the images or other conditions changed. By contrast, the relative grey values, i.e. the absolute grey values correlated to the mean grey values of the histoimage, remained constant. By utilizing these relative grey values, it was possible to compare cells both between different specimens and between different areas within the same specimen. The different grey values of spiral ganglion cells stained for S-100 protein are objective quantitative measurements and are believed to reflect differences in their function. In some regions of both human and squirrel monkey specimens, relatively intensely stained cells predominated, whereas in other regions, relatively weakly stained cells were mainly observed. Thus, our image analysis system using the relative grey values has proved suitable for quantitative analysis of immunostained specimens in order to compare them and to assess cell function. PMID:9504164

  15. How minute sooglossid frogs hear without a middle ear.

    PubMed

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

    2013-09-17

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

  16. Making an Effort to Listen: Mechanical Amplification in the Ear

    PubMed Central

    Hudspeth, A. J.

    2009-01-01

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

  17. What have lizard ears taught us about auditory physiology?

    PubMed

    Manley, Geoffrey A; Köppl, Christine

    2008-04-01

    The structure of the basilar papilla of the inner ear of lizards is the most diverse among all vertebrates. Research on a variety of lizard ears, animals that are remarkably robust under laboratory conditions, has provided the field of auditory research with valuable information, particularly on the minimum structural requirements for sensitive, selective hearing and on the importance of the tectorial membrane and active processes in this regard. Despite the absence of a tuned basilar membrane, lizard ears produce highly frequency selective hearing through micromechanical tuning of small, resonant hair-cell-tectorial units or of free-standing hair bundles. These units are driven by an active process that also underlies spontaneous and other otoacoustic emissions. Lizard ears provided the first in vivo evidence that the active process is calcium-sensitive and lies within the stereovillar bundles of the hair cells. PMID:17983712

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

    E-print Network

    Allen, Jont

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

  19. Plant physiology Role of awns in ear water-use efficiency

    E-print Network

    Paris-Sud XI, Université de

    Plant physiology Role of awns in ear water-use efficiency and grain weight in barley J Bort net photosynthesis and water-use efficiency (WUE: net photosynthesis/transpiration) of ears and flag photosynthesis of awned ears was markedly higher than that of awnless ears, until 3 weeks after anthesis

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

    E-print Network

    Paris-Sud XI, Université de

    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

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

    E-print Network

    Allen, Jont

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

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

    E-print Network

    Szigeti, Zoltán

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

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

    E-print Network

    O'Toole, Alice J.

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

  4. Wideband energy reflectance measurements: Effects of negative middle ear pressure and application of a pressure compensation

    E-print Network

    Allen, Jont

    Wideband energy reflectance measurements: Effects of negative middle ear pressure and application become popular as a tool for evaluating mid- dle ear function. Negative middle ear pressure (MEP) is a prevalent form of middle ear dysfunction, which may impact application of ER measurements in differential

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

    PubMed Central

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

    2014-01-01

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

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

    PubMed Central

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

    2009-01-01

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

  7. Mapping a Gene Causing Cerebral Cavernous Malformation to 7q11.2-q21

    Microsoft Academic Search

    Murat Gunel; Issam A. Awad; John Anson; Richard P. Lifton

    1995-01-01

    Cerebral cavernous malformation is a common disease of the brain vasculature of unknown cause characterized by dilated thin-walled sinusoidal vessels (caverns); these lesions cause varying clinical presentations which include headache, seizure, and hemorrhagic stroke. This disorder is frequently familial, with autosomal dominant inheritance. Using a general linkage approach in two extended cavernous malformation kindreds, we have identified linkage of this

  8. Combined spatial-temporal analysis of malformation rates in Bavaria after the Chernobyl accident

    Microsoft Academic Search

    Helmut Küchenhoff; Astrid Engelhardt; Alfred Körblein

    Malformation rates in the German state of Bavaria, as a whole, did not increase in 1987, the year following the Chernobyl accident. Also an analysis of the monthly data does not show any association between radiation exposure and malformation rates seven month later. But in a detailed analysis on the level of districts taking the spatial structure into account we

  9. Multiple de novo vascular malformations in relation to diffuse venous occlusive disease: a case report

    Microsoft Academic Search

    H. A. Desal; S. K. Lee; B. S. Kim; S. Raoul; M. Tymianski; K. G. TerBrugge

    2005-01-01

    Brain vascular malformations are dynamic disorders. Although mostly considered to be of congenital origin, the improvement of clinical imaging and vasculogenesis knowledge has shown that they might also result from a biological dysfunction of the remodeling process after birth. Venous occlusive disease and ishemia may represent powerful revealing triggers and support the capillary venous origin of some vascular malformations. We

  10. Clinical syndromes of arteriovenous malformations of the transverse-sigmoid sinus

    Microsoft Academic Search

    S Obrador; M Soto; J Silvela

    1975-01-01

    Arteriovenous malformations or fistulae shunting arterial blood from branches of the external and internal carotid and vertebral arteries into the transverse-sigmoid sinus may produce different clinical syndromes. The literature is reviewed with 96 patients including six personal cases. Usually these malformations have a congenital origin and only in 4% of the series was there a previous history of a severe

  11. Regional Ependymal Upregulation of Vimentin in Chiari II Malformation, Aqueductal Stenosis, and Hydromyelia

    Microsoft Academic Search

    Harvey B. Sarnat

    2004-01-01

    Vimentin, glial fibrillary acidic protein (GFAP) and S-100 ? protein were studied by immunocytochemistry in the ependyma of patients with Chiari II malformations, congenital aqueductal stenosis, and hydromyelia. Paraffin sections of brains and spinal cords of 16 patients were examined, 14 with Chiari II malformations, most with aqueductal stenosis and\\/or hydromyelia as associated features, and 2 patients with congenital aqueductal

  12. Vascular malformations as underlying cause of chronic thromboembolism and pulmonary hypertension

    Microsoft Academic Search

    Charlene E. U. Oduber; Victor E. A. Gerdes; Chantal M. A. M. van der Horst; Paul Bresser

    2009-01-01

    We report four patients with chronic thromboembolic pulmonary hypertension (CTEPH) presumably due to recurrent pulmonary embolism from low-flow vascular malformations, and give a review of the literature. Venous malformations, such as those observed in Klippel- Trenaunay syndrome (KTS) can be associated with hypercoagulability, thrombosis and recurrent pulmonary embolism and ultimately CTEPH. Since many physicians appear unfamiliar with these potential complications,

  13. Transcatheter Embolization of Extremity Vascular Malformations: The Long-term Success of Multiple Interventions

    Microsoft Academic Search

    Caron B. Rockman; Robert J. Rosen; Glenn R. Jacobowitz; Jonathan Weiswasser; Dirk J. Hofstee; B. Fioole; Patrick J. Lamparello; Mark A. Adelman; Paul J. Gagne; Thomas S. Riles

    2003-01-01

    Vascular malformations of the extremities present a difficult therapeutic challenge. Ligation of feeding vessels may lead to tissue necrosis and limb loss and can make subsequent attempts at transcatheter therapy impossible. The purpose of this study was to review our results with transcatheter embolization therapy in symptomatic vascular malformations in the upper and lower extremities in 50 patients. A retrospective

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

    Microsoft Academic Search

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

    1992-01-01

    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

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

    SciTech Connect

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

    1995-07-03

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

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

    Microsoft Academic Search

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

    2006-01-01

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

  17. Review Article: Chiari Type I Malformation with or Without Syringomyelia: Prevalence and Genetics

    Microsoft Academic Search

    Marcy C. Speer; David S. Enterline; Lorraine Mehltretter; Preston Hammock; Judith Joseph; Margaret Dickerson; Richard G. Ellenbogen; Thomas H. Milhorat; Michael A. Hauser; Timothy M. George

    2003-01-01

    Chiari type I malformation has traditionally been defined as a downward herniation of the cerebellar tonsils of =5 mm through the foramen magnum and it is likely associated with a volumetrically reduced posterior fossa. Syringomyelia is commonly associated with Chiari type I malformation. We estimate the prevalence of these two conditions and determine that they are more common than previously

  18. Diagnosis and Management of a Vein of Galen Malformation in a Neonate

    Microsoft Academic Search

    Stacy L. French

    2010-01-01

    Vein of Galen arterial malformation (VGAM) is an arteriovenous malformation between cerebral vessels of the circle of Willis and the vein of Galen. VGAM is a direct communication between an artery and a vein resulting in increased cardiac output due to arterial blood flowing directly into a vein and returning directly to the heart, without traversing the full vascular system.

  19. Ear decomposition of 3-regular polyhedral links with applications.

    PubMed

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

    2014-10-21

    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

  20. Surgical Management of Patients with Chiari I Malformation

    PubMed Central

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

    2012-01-01

    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

  1. Morphological and functional aspects of progenitors perturbed in cortical malformations

    PubMed Central

    Bizzotto, Sara; Francis, Fiona

    2015-01-01

    In this review, we discuss molecular and cellular mechanisms important for the function of neuronal progenitors during development, revealed by their perturbation in different cortical malformations. We focus on a class of neuronal progenitors, radial glial cells (RGCs), which are renowned for their unique morphological and behavioral characteristics, constituting a key element during the development of the mammalian cerebral cortex. We describe how the particular morphology of these cells is related to their roles in the orchestration of cortical development and their influence on other progenitor types and post-mitotic neurons. Important for disease mechanisms, we overview what is currently known about RGC cellular components, cytoskeletal mechanisms, signaling pathways and cell cycle characteristics, focusing on how defects lead to abnormal development and cortical malformation phenotypes. The multiple recent entry points from human genetics and animal models are contributing to our understanding of this important cell type. Combining data from phenotypes in the mouse reveals molecules which potentially act in common pathways. Going beyond this, we discuss future directions that may provide new data in this expanding area. PMID:25729350

  2. Treatment of cerebral arteriovenous malformations: combined neurosurgical and neuroradiologic approach.

    PubMed

    Cromwell, L D; Harris, A B

    1983-01-01

    Experience with 31 intraoperative embolizations performed on 23 patients is reported. The procedure involves the direct injection of embolic material into the main arterial feeders during craniotomy. The combined effort involves the exposure of the lesion at craniotomy, standard arteriotomy, and fluoroscopically monitored intravascular infusion of contrast material followed by the injection of bucrylate (IBCA, Ethicon, Somerville, NJ) mixed with tantalum or Pantopaque into the feeding vessels and into the interstices of the malformation. It was concluded that the procedure significantly diminishes operating time and blood loss for the lesions that were subsequently resected. Fluoroscopy coupled with high-quality stop-frame videotape recording allows a detailed study of the flow characteristics of the malformation so that polymerization time can be regulated appropriately. Follow-up varied from 3 months to 4 1/2 years. There were two deaths related to the procedure and one additional complication of cortical blindness that partially resolved. None of the survivors rebled. Most of the survivors improved. PMID:6410746

  3. Minnesota wolf ear lengths as possible indicators of taxonomic differences

    USGS Publications Warehouse

    Mech, L. David

    2011-01-01

    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.

  4. Inner ear morphological correlates of ultrasonic hearing in frogs.

    PubMed

    Arch, Victoria S; Simmons, Dwayne D; Quiñones, Patricia M; Feng, Albert S; Jiang, Jianping; Stuart, Bryan L; Shen, Jun-Xian; Blair, Chris; Narins, Peter M

    2012-01-01

    Three species of anuran amphibians (Odorrana tormota, Odorrana livida and Huia cavitympanum) have recently been found to detect ultrasounds. We employed immunohistochemistry and confocal microscopy to examine several morphometrics of the inner ear of these ultrasonically sensitive species. We compared morphological data collected from the ultrasound-detecting species with data from Rana pipiens, a frog with a typical anuran upper cut-off frequency of ?3 kHz. In addition, we examined the ears of two species of Lao torrent frogs, Odorrana chloronota and Amolops daorum, that live in an acoustic environment approximating those of ultrasonically sensitive frogs. Our results suggest that the three ultrasound-detecting species have converged on small-scale functional modifications of the basilar papilla (BP), the high-frequency hearing organ in the frog inner ear. These modifications include: 1. reduced BP chamber volume, 2. reduced tectorial membrane mass, 3. reduced hair bundle length, and 4. reduced hair cell soma length. While none of these factors on its own could account for the US sensitivity of the inner ears of these species, the combination of these factors appears to extend their hearing bandwidth, and facilitate high-frequency/ultrasound detection. These modifications are also seen in the ears of O. chloronota, suggesting that this species is a candidate for high-frequency hearing sensitivity. These data form the foundation for future functional work probing the physiological bases of ultrasound detection by a non-mammalian ear. PMID:22146424

  5. Computed tomography features of middle ear cholesteatoma in dogs.

    PubMed

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

    2010-01-01

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

  6. Retinoid Signaling in Inner Ear Development: a “Goldilocks” Phenomenon

    PubMed Central

    Frenz, Dorothy A.; Liu, Wei; Cvekl, Ales; Xie, Qing; Wassef, Lesley; Quadro, Loredana; Niederreither, Karen; Maconochie, Mark; Shanske, Alan

    2010-01-01

    Retinoic acid is a biologically active derivative of vitamin A that is indispensable for inner ear development. The normal function of retinoic acid is achieved only at optimal homeostatic concentrations, with an excess or deficiency in retinoic acid leading to inner ear dysmorphogenesis. We present an overview of the role of retinoic acid in the developing mammalian inner ear, discussing both how and when retinoic acid may act to critically control a program of inner ear development. Molecular mechanisms of otic teratogenicity involving two members of the fibroblast growth factor family, FGF3 and FGF10, and their downstream targets, Dlx5 and Dlx6, are examined under conditions of both retinoic acid excess and deficiency. We term the effect of too little or too much retinoic acid on FGF/Dlx signaling a Goldilocks phenomenon. We demonstrate that in each case (retinoic acid excess, retinoic acid deficiency), retinoic acid can directly affect FGF3/FGF10 signaling within the otic epithelium, leading to downregulated expression of these essential signaling molecules, which in turn, leads to diminution in Dlx5/Dlx6 expression. Non-cell autonomous affects of the otic epithelium subsequently occur, altering transforming growth factor beta (TGF?) expression in the neighboring periotic mesenchyme and serving as a putative explanation for retinoic acid-mediated otic capsule defects. We conclude that retinoic acid coordinates inner ear morphogenesis by controlling an FGF/Dlx signaling cascade, whose perturbation by deviations in local retinoid concentrations can lead to inner ear dysmorphogenesis. PMID:21108385

  7. Developmental origin and fate of middle ear structures.

    PubMed

    Sienknecht, Ulrike J

    2013-07-01

    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

  8. Evaluation of the association of zoonotic Ljungan virus with perinatal deaths and fetal malformation.

    PubMed

    Zheng, Lili; Wang, Fang; Huang, Jing; Xin, Hong

    2015-03-01

    More and more epidemiologic and experimental data support the notion that Ljungan virus (LV), originally isolated from some rodent populations in Sweden, Denmark, and the United States, plays an important role in stillbirth and fetal malformation. Mouse dams infected with LV may result in uterine resorption and perinatal deaths that may cross generations, and their offspring may suffer high rates of malformations including cranial, brain, and limb malformations. In humans, researches founded that LV infection is related to malformation, intrauterine fetal death, and even central nervous system malformation. Although molecularly characterized, little is known about the biophysical nature of LV. Consequently, the role of LV infections in sudden infant death syndrome is still confusing, and the mechanism of how LV infections cause diseases is not clear. More research is clearly necessary to explore the mechanisms of LV infection in human and animal diseases to bring improvement to the clinical outcomes. PMID:25789980

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

    PubMed Central

    2014-01-01

    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

  10. Mastoid obliteration combined with soft-wall reconstruction of posterior ear canal

    Microsoft Academic Search

    Haruo Takahashi; Tetsu Iwanaga; Satoru Kaieda; Tomomi Fukuda; Hidetaka Kumagami; Kenji Takasaki; Seishi Hasebe; Kazuo Funabiki

    2007-01-01

    To clarify the usefulness of modified soft-wall reconstruction method by combing with mastoid obliteration, 96 patients (98\\u000a ears) with their age ranging from 5 to 82 (average 51.3), including 62 ears with chronic otitis media (COM) with cholesteatoma,\\u000a 18 ears with non-cholesteatomatous COM, 14 ears with postoperative cavity problem, and 4 ears with adhesive-type COM, who\\u000a had soft-wall reconstruction of

  11. Acoustic–structural coupled finite element analysis for sound transmission in human ear—Pressure distributions

    Microsoft Academic Search

    Rong Z. Gan; Qunli Sun; Bin Feng; Mark W. Wood

    2006-01-01

    A three-dimensional (3D) finite element (FE) model of human ear with accurate structural geometry of the external ear canal, tympanic membrane (TM), ossicles, middle ear suspensory ligaments, and middle ear cavity has been recently reported by our group. In present study, this 3D FE model was modified to include acoustic–structural interfaces for coupled analysis from the ear canal through the

  12. Bacterial isolates and drug susceptibility patterns of ear discharge from patients with ear infection at Gondar University Hospital, Northwest Ethiopia

    PubMed Central

    2013-01-01

    Background Ear infection is a common problem for both children and adults especially in developing countries. However in Ethiopia particularly in the study area, there is no recent data that shows the magnitude of the problem. The aim of this study was to determine the bacterial isolates and their drug susceptibility patterns from patients who had ear infection. Method A retrospective study was conducted from September, 2009 to August, 2012 at Gondar University Hospital, Northwest Ethiopia. Ear discharge samples were cultured on MacConkey agar, blood agar and chocolate agar plates. A standard biochemical procedure was used for full identification of bacterial isolates. Antimicrobial susceptibility tests were done on Mueller-Hinton agar by using disk diffusion method. Data were entered and analyzed by using SPSS version 20 software and P-value of?ear discharge samples were tested for bacterial isolation and 204 (89.5%) cases were found to have bacterial isolates. From the total bacterial isolates, 115 (56.4%) were gram negative bacteria and the predominant isolate was proteus species (27.5%). Of individuals who had ear infection, 185 (90.7%) had single bacterial infection while 19 (9.3%) had mixed infections. Under five children were more affected by ear infection. The prevalence of ear infection was significantly high in males (63.7 vs 36.3%) (P?=?0.017). Of all bacterial isolates, 192 (94.1%) had multiple antibiotic resistant pattern. Non Lactose Fermenter Gram Negative Rods (46.0%), Klebsella species (47.7%) and Pseudomonas species (48.5%) were resistant against the commonly used antibiotics. Conclusion The prevalence of ear infection was very high in the study area. Majority of the bacterial isolates were resistant to multiple antibiotics. Hence antibiotics susceptibility test is mandatory before prescribing any antibiotics. PMID:23914777

  13. Complex Stapes Motions in Human Ears

    PubMed Central

    Chatzimichalis, Michail; Lauxmann, Michael; Röösli, Christof; Eiber, Albrecht; Huber, Alexander M.

    2010-01-01

    It has been reported that the physiological motion of the stapes in human and several animals in response to acoustic stimulation is mainly piston-like at low frequencies. At higher frequencies, the pattern includes rocking motions around the long and short axes of the footplate in human and animal ears. Measurements of such extended stapes motions are highly sensitive to the exact angulation of the stapes in relation to the measurement devices and to measurement errors. In this study, velocity in a specific direction was measured at multiple points on the footplates of human temporal bones using a Scanning Laser Doppler Vibrometer (SLDV) system, and the elementary components of the stapes motions, which were the piston-like motion and the rocking motions about the short and long axes of the footplate, were calculated from the measurements. The angular position of a laser beam with respect to the stapes and coordinates of the measurement points on the footplate plane were calculated by correlation between the SLDV measurement frame and the footplate-fixed frame, which was obtained from micro-CT images. The ratios of the rocking motions relative to the piston-like motion increased with frequency and reached a maximum around 7 kHz. A novel method for quantitatively assessing measurements of complex stapes motions and error boundaries of the motion components is presented. In the frequency range of 0.5 to 8 kHz, the magnitudes of the piston-like and two rocking motions were larger than estimated values of the corresponding upper error bounds. PMID:20165895

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

    E-print Network

    2006-01-01

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

  15. Auditory Location in the Irrelevant Sound Effect: The Effects of Presenting Auditory Stimuli to Either the Left Ear, Right Ear or Both Ears

    ERIC Educational Resources Information Center

    Hadlington, Lee; Bridges, Andrew M.; Darby, Richard J.

    2004-01-01

    Two experiments used both irrelevant speech and tones in order to assess the effect of manipulating the spatial location of irrelevant sound. Previous research in this area had produced inconclusive results (e.g., Colle, 1980). The current study demonstrated a novel finding, that sound presented to the left ear produces the greatest level of…

  16. Onyx® in the management of cranial arteriovenous malformations.

    PubMed

    Jalaly, Jalal; Dalfino, John; Mousa, Shaker A

    2013-07-01

    The ethylene vinyl alcohol copolymer, Onyx® (ev3, Inc., MN, USA), is an embolic agent used in the management of arteriovenous malformations (AVMs) and was approved by the US FDA in 2005. Use of Onyx has resulted in higher curative rates compared with previous embolic agents such as N-butyl-2-cyanoacrylate. Onyx has several advantages over previous embolic agents. For instance, Onyx is a copolymer and does not adhere to catheters but solidifies slowly owing to the diffusion of its solvent dimethyl sulfoxide. This review focuses on different results obtained from the experience of different neurointerventionalists with an emphasis on the aim to cure. The studies presented are compared in terms of curative rates, morbidity and mortality. Ideas are presented for future treatment options for AVM rupture using nanotargeted therapy with the guide of specific markers highly expressed on the surface of cells or tissues of interest. Such nanotargeted therapy might transform AVM treatment with new embolic agents. PMID:23895072

  17. A case of scrotal venous malformation mimicking a failed varicocelectomy.

    PubMed

    Yi, Chong K; Derosa, Raffaella; Sterbis, Joseph R; Ching, Brian H

    2014-01-01

    A 21-year-old man presented to interventional radiology department with several years of left scrotal pain, which exacerbated by prolonged standing and walking. The patient had undergone a left varicocelectomy at the age of 10, after which he had a persistent scrotal mass. As he grew older, the left scrotal mass continued to increase in size, and symptoms progressively worsened. Physical examination revealed a non-tender, firm left scrotal mass which remained unchanged in size after Valsalva manoeuvres. Initial ultrasound examination revealed an extratesticular, intrascrotal mass with minimal internal flow. MRI revealed a heterogeneously enhancing, low-flow venous malformation centred in the midscrotum involving multiple tissue planes. Two sessions of percutaneous sclerotherapy failed to relieve his pain. Three months after the second sclerotherapy, the patient underwent surgical resection. At the time of his postoperative visit, his left scrotal pain had significantly improved and pain with prolonged standing and walking had resolved completely. PMID:24654244

  18. Quantification of intracerebral steal in patients with arteriovenous malformation

    SciTech Connect

    Homan, R.W.; Devous, M.D. Sr.; Stokely, E.M.; Bonte, F.J.

    1986-08-01

    Eleven patients with angiographically and/or pathologically proved arteriovenous malformations (AVMs) were studied using dynamic, single-photon-emission computed tomography (DSPECT). Quantification of regional cerebral blood flow in structurally normal areas remote from the AVM disclosed areas of decreased flow compared with normal controls in eight of 11 patients examined. Areas of hypoperfusion correlated with altered function as manifested by epileptogenic foci and impaired cognitive function. Dynamic, single-photon-emission computed tomography provides a noninvasive technique to monitor quantitatively hemodynamic changes associated with AVMs. Our findings suggest that such changes are present in the majority of patients with AVMs and that they may be clinically significant. The potential application of regional cerebral blood flow imaging by DSPECT in the management of patients with AVMs is discussed.

  19. [Thrombosed scapular intramuscular venous malformation: A pediatric case report].

    PubMed

    Fekir, A; Balguerie, X; Bardoux, I; Dacher, J-N; Vivier, P-H

    2015-07-01

    Venous malformation (VM) is a frequent soft tissue mass in children that should not be confused with vascular tumors. We report the case of a 10-year-old boy with a bulky thrombosed VM of the shoulder with functional disability. D-dimer levels were high. Pathognomonic phleboliths were demonstrated on conventional x-rays. Echo-doppler confirmed the venous nature of the lesion and areas without flow suspicious for thrombosis. MRI provides the deep extension and can show non-calcified thrombi that are difficult to see with ultrasound. Localized intravascular coagulopathy is often associated with thrombosis phenomena and has to be confirmed by measurement of D-dimer levels in order to initiate anticoagulation quickly with low-molecular-weight heparin. Follow-up includes measurement of D-dimer levels and potentially MRI to evaluate the anticoagulation efficacy and tailor treatment duration. PMID:26047748

  20. Developmental outcomes of Down syndrome and Dandy-Walker malformation

    PubMed Central

    Love, Kaitlin; Huddleston, Lillie; Olney, Pat; Wrubel, David; Visootsak, Jeannie

    2012-01-01

    Dandy-Walker syndrome (DWS), or Dandy-Walker complex, is a congenital brain malformation of the posterior fossa, typically resulting in developmental delay and cognitive disability. The co-occurrence of Down syndrome (DS) and DWS is relatively uncommon; thus, its impact on developmental outcomes has not been fully elucidated. Herein, we report a case of a 37-month-old child with DS and DWS, who is functioning at the following age-equivalent: gross motor at a 9-mo level, fine motor 6 mo, expressive language 14 mo, receptive language 9 mo. As such, it is important to determine how the DWS influences developmental outcomes, and appreciate the importance of early interventional therapy. PMID:22866020

  1. Embolization of Arteriovenous Malformation with Diluted Mixture of NBCA

    PubMed Central

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

    2000-01-01

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

  2. Antibiotics potentially used in response to bioterrorism and the risk of major congenital malformations.

    PubMed

    Cooper, William O; Hernandez-Diaz, Sonia; Arbogast, Patrick G; Dudley, Judith A; Dyer, Shannon M; Gideon, Patricia S; Hall, Kathleen S; Kaltenbach, Lisa A; Ray, Wayne A

    2009-01-01

    This study was designed to assess the association between pregnancy-related exposures to antibiotics recommended for use in the event of a bioterrorism attack and major congenital malformations. A retrospective cohort study included 30 049 infants from Tennessee Medicaid born between 1985 and 2000 identified from computerised state databases. Infants with fetal exposures to ciprofloxacin, azithromycin, doxycycline and amoxicillin (antibiotics recommended for potential bioterrorism attacks) (n = 24 521) and erythromycin (included as a positive control) (n = 2128) were compared with infants with no fetal exposure to any antibiotics (n = 3400). Major congenital malformations identified from computerised records were confirmed through medical record review. Overall, 869 (2.9%) of infants in the cohort had a confirmed major congenital malformation, with major malformations ranging from 2.5% to 3.0% among the antibiotic-specific exposure groups. No increased risk was present in multivariable analyses for any malformations and for malformations of specific organ systems. In conclusion, these data suggest that ciprofloxacin, azithromycin, doxycycline or amoxicillin use by pregnant women should not result in a greater incidence of overall major congenital malformations in infants whose mothers take these medications, though a large increase in risk cannot be ruled out. PMID:19228311

  3. ANTIBIOTICS POTENTIALLY USED IN RESPONSE TO BIOTERRORISM AND THE RISK OF MAJOR CONGENITAL MALFORMATIONS

    PubMed Central

    Cooper, William O.; Hernandez-Diaz, Sonia; Arbogast, Patrick G.; Dudley, Judith A.; Dyer, Shannon M.; Gideon, Patricia S.; Hall, Kathleen S.; Kaltenbach, Lisa A.; Ray, Wayne A.

    2012-01-01

    SUMMARY This study was designed to assess the association between pregnancy-related exposures to antibiotics recommended for use in the event of a bioterrorism attack and major congenital malformations. A retrospective cohort study included 30,049 infants from Tennessee Medicaid born between 1985–2000 identified from computerized state databases. Infants with fetal exposures to ciprofloxacin, azithromycin, doxycycline, and amoxicillin (antibiotics recommended for potential bioterrorism attacks) and erythromycin (included as a positive control) were compared to infants with no fetal exposure to any antibiotics. Major congenital malformations identified from computerized records were confirmed through medical record review. Overall, 869 (2.9%) of infants in the cohort had a confirmed major congenital malformation, with major malformations ranging from 2.5%–3.0% among the antibiotic-specific exposure groups. No increased risk was present in multivariable analyses for any malformations and for malformations of specific organ systems. In conclusion, these data suggest that ciprofloxacin, azithromycin, doxycycline, or amoxicillin use by pregnant women should not result in a greater incidence of overall major congenital malformations in infants whose mothers take these medications, though a large increase in risk cannot be ruled out. PMID:19228311

  4. Management of cerebral cavernous malformations in the pediatric population: a literature review and case illustrations.

    PubMed

    Kosnik-Infinger, L; Carroll, C; Greiner, H; Leach, J; Mangano, F T

    2015-09-01

    Cavernous malformations (CM) are vascular malformations of the central nervous system that may occur in the brain and spinal cord. They are one of the four major types of vascular malformations that also includes developmental venous anomalies (DVA)s, arteriovenous malformations (AVMs), and capillary telangiectasias. CMs are a common vascular malformation, and 25% of them occur in the pediatric age group. They can present with acute or chronic symptoms including headache, neurologic deficits secondary to hemorrhage, mass effect, or epilepsy. This review will focus on the neurosurgical management of intracranial cavernous malformations in children. Pediatric CMs have special considerations different from CM that occur in the adult population which are highlighted throughout this review. Characteristics specific to pediatric CM epidemiology, genetics, presentation, pathology, location, size, epilepsy, and management will be discussed. Specific considerations must be entertained with the diagnosis of pediatric CM in that management needs to include consideration of the lifetime risk of hemorrhage, as well as the possibility of development of epilepsy. If in an accessible location, most cavernomas should be surgically removed in a timely fashion to provide lifelong cure for pediatric patients. The review closes with the discussion of two interesting cavernous malformation cases occurring in a 12-year old male and a 12-year old female that exhibit many of the important aspects specific to the management of a pediatric patient with CM, highlighting the importance of a multidisciplinary approach to treatment. PMID:25998208

  5. NPHP4 Variants are Associated with Pleiotropic Heart Malformations

    PubMed Central

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

    2014-01-01

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

  6. Stereotactic proton beam therapy for intracranial arteriovenous malformations

    SciTech Connect

    Vernimmen, Frederik J.A.I. [Department of Radiation Oncology, Tygerberg Hospital, Tygerberg (South Africa)]. E-mail: fv@sun.ac.za; Slabbert, Jacobus P. [Department of Radiation Biophysics, iThemba LABS, Somerset West (South Africa); Wilson, Jennifer A. [Department of Radiation Oncology, Little Company of Mary Hospital, Pretoria (South Africa); Fredericks, Shaheeda [Department of Radiography/Therapy, iThemba LABS, Somerset West (South Africa); Melvill, Roger [Department of Neurosurgery, Groote Schuur Hospital, Observatory (South Africa)

    2005-05-01

    Purpose: To investigate hypofractionated stereotactic proton therapy of predominantly large intracranial arteriovenous malformations (AVMs) by analyzing retrospectively the results from a cohort of patients. Methods and Materials: Since 1993, a total of 85 patients with vascular lesions have been treated. Of those, 64 patients fulfilled the criteria of having an arteriovenous malformation and sufficient follow-up. The AVMs were grouped by volume: <14 cc (26 patients) and {>=}14 cc (38 patients). Treatment was delivered with a fixed horizontal 200 MeV proton beam under stereotactic conditions, using a stereophotogrammetric positioning system. The majority of patients were hypofractionated (2 or 3 fractions), and the proton doses are presented as single-fraction equivalent cobalt Gray equivalent doses (SFEcGyE). The overall mean minimum target volume dose was 17.37 SFEcGyE, ranging from 10.38-22.05 SFEcGyE. Results: Analysis by volume group showed obliteration in 67% for volumes <14 cc and 43% for volumes {>=}14 cc. Grade IV acute complications were observed in 3% of patients. Transient delayed effects were seen in 15 patients (23%), becoming permanent in 3 patients. One patient also developed a cyst 8 years after therapy. Conclusions: Stereotactic proton beam therapy applied in a hypofractionated schedule allows for the safe treatment of large AVMs, with acceptable results. It is an alternative to other treatment strategies for large AVMs. AVMs are likely not static entities, but probably undergo vascular remodeling. Factors influencing angiogenesis could play a new role in a form of adjuvant therapy to improve on the radiosurgical results.

  7. Aborting a Malformed Fetus: A Debatable Issue in Saudi Arabia

    PubMed Central

    Al-Alaiyan, Saleh; AlFaleh, Khalid M.

    2012-01-01

    Congenital anomalies contribute a significant proportion of infant morbidity and mortality, as well as fetal mortality. They are generally grouped into three major categories: structural/metabolic, congenital infections, and other conditions. The most prevalent conditions include congenital heart defects, orofacial clefts, Down syndrome, and neural tube defects. Several prenatal diagnostic procedures have been introduced, both cytogenetic (such as chorion biopsy, amniocentesis and funiculocentesis) and biophysical (ultrasound 2-D, 3-D and 4-D, ultrasonography with Doppler, etc.). Insufficient data are currently available from Saudi Arabia on the epidemiology of the lethal congenital abnormalities which should be a priority due to high rate of consanguineous marriages among first cousins and their association with congenital anomalies. In terms of consanguinity and birth defects, a significant positive association has been consistently demonstrated between consanguinity and morbidity, and congenital defects with a complex etiology appear to be both more prevalent in consanguineous families and have a greater likelihood of recurrence. A debate regarding aborting a malformed fetus still exists among the senior Islamic scholars in many of the Islamic countries. The progressive interpretations of Islam have resulted in laws allowing for early abortion on request in two countries; six others permit abortion on health grounds and three more also allow abortion in cases of rape or fetal impairment. In Saudi Arabia, efforts to legalize abortion in certain circumstances have been recently discussed among Senior Religious Scholars and specialized physicians to permit abortions in certain circumstances. In this mini-review we discuss the current debate regarding aborting a malformed fetus in Saudi Arabia with a focus on the Islamic perspective. PMID:24027674

  8. Aborting a malformed fetus: a debatable issue in saudi arabia.

    PubMed

    Al-Alaiyan, Saleh; Alfaleh, Khalid M

    2012-01-01

    Congenital anomalies contribute a significant proportion of infant morbidity and mortality, as well as fetal mortality. They are generally grouped into three major categories: structural/metabolic, congenital infections, and other conditions. The most prevalent conditions include congenital heart defects, orofacial clefts, Down syndrome, and neural tube defects. Several prenatal diagnostic procedures have been introduced, both cytogenetic (such as chorion biopsy, amniocentesis and funiculocentesis) and biophysical (ultrasound 2-D, 3-D and 4-D, ultrasonography with Doppler, etc.). Insufficient data are currently available from Saudi Arabia on the epidemiology of the lethal congenital abnormalities which should be a priority due to high rate of consanguineous marriages among first cousins and their association with congenital anomalies. In terms of consanguinity and birth defects, a significant positive association has been consistently demonstrated between consanguinity and morbidity, and congenital defects with a complex etiology appear to be both more prevalent in consanguineous families and have a greater likelihood of recurrence. A debate regarding aborting a malformed fetus still exists among the senior Islamic scholars in many of the Islamic countries. The progressive interpretations of Islam have resulted in laws allowing for early abortion on request in two countries; six others permit abortion on health grounds and three more also allow abortion in cases of rape or fetal impairment. In Saudi Arabia, efforts to legalize abortion in certain circumstances have been recently discussed among Senior Religious Scholars and specialized physicians to permit abortions in certain circumstances. In this mini-review we discuss the current debate regarding aborting a malformed fetus in Saudi Arabia with a focus on the Islamic perspective. PMID:24027674

  9. Spag17 Deficiency Results in Skeletal Malformations and Bone Abnormalities

    PubMed Central

    Teves, Maria Eugenia; Sundaresan, Gobalakrishnan; Cohen, David J.; Hyzy, Sharon L.; Kajan, Illya; Maczis, Melissa; Zhang, Zhibing; Costanzo, Richard M.; Zweit, Jamal; Schwartz, Zvi; Boyan, Barbara D.; Strauss, Jerome F.

    2015-01-01

    Height is the result of many growth and development processes. Most of the genes associated with height are known to play a role in skeletal development. Single-nucleotide polymorphisms in the SPAG17 gene have been associated with human height. However, it is not clear how this gene influences linear growth. Here we show that a targeted mutation in Spag17 leads to skeletal malformations. Hind limb length in mutants was significantly shorter than in wild-type mice. Studies revealed differences in maturation of femur and tibia suggesting alterations in limb patterning. Morphometric studies showed increased bone formation evidenced by increased trabecular bone area and the ratio of bone area to total area, leading to reductions in the ratio of marrow area/total area in the femur. Micro-CTs and von Kossa staining demonstrated increased mineral in the femur. Moreover, osteocalcin and osterix were more highly expressed in mutant mice than in wild-type mice femurs. These data suggest that femur bone shortening may be due to premature ossification. On the other hand, tibias appear to be shorter due to a delay in cartilage and bone development. Morphometric studies showed reduction in growth plate and bone formation. These defects did not affect bone mineralization, although the volume of primary bone and levels of osteocalcin and osterix were higher. Other skeletal malformations were observed including fused sternebrae, reduced mineralization in the skull, medial and metacarpal phalanges. Primary cilia from chondrocytes, osteoblasts, and embryonic fibroblasts (MEFs) isolated from knockout mice were shorter and fewer cells had primary cilia in comparison to cells from wild-type mice. In addition, Spag17 knockdown in wild-type MEFs by Spag17 siRNA duplex reproduced the shorter primary cilia phenotype. Our findings disclosed unexpected functions for Spag17 in the regulation of skeletal growth and mineralization, perhaps because of its role in primary cilia of chondrocytes and osteoblasts. PMID:26017218

  10. Analysis of environmental-stress-related impairments of inner ear.

    PubMed

    Ohgami, Nobutaka; Iida, Machiko; Omata, Yasuhiro; Nakano, Chihiro; Wenting, Wu; Li, Xiang; Kato, Masashi

    2015-01-01

    Noise stress generated in industry is one of the environmental factors that physically affects the functions of the inner ear. Exposure to noise can cause hearing loss, resulting in serious problems in occupational and daily life. At present, however, there are very limited ways to prevent hearing impairments. The inner ear consists of the organ of Corti, vestibule and semicircular canal. Functional or morphological damage of these tissues in the inner ear caused by genetic factors, aging or environmental factors can result in hearing or balance impairments. In this review, we first introduce a deafness-related molecule found by our clinical research. Our experimental research using genetically engineered mice further demonstrated that impaired activity of the target molecule caused congenital and age-related hearing loss with neurodegeneration of spiral ganglion neurons in the inner ears. We also describe impaired balance in mice caused by exposure to low-frequency noise under experimental conditions with indoor environmental monitoring. We believe that our approaches to pursue both experimental research and fieldwork research complementarily are crucial for the development of a method for prevention of impairments of the inner ear. PMID:25994339

  11. An abbreviated history of the ear: from Renaissance to present.

    PubMed Central

    Hachmeister, Jorge E.

    2003-01-01

    In this article we discuss important discoveries in relation to the anatomy and physiology of the ear from Renaissance to present. Before the Renaissance, there was a paucity of knowledge of the anatomy of the ear, because of the relative inaccessibility of the temporal bone and the general perception that human dissections should not be conducted. It was not until the sixteenth century that the middle ear was described with detail. Further progress would be made between the sixteenth and eighteenth century in describing the inner ear. In the nineteenth century, technological advancement permitted a description of the cells and structures that constitute the cochlea. Von Helmholtz made further progress in hearing physiology when he postulated his resonance theory and later von Békésy when he observed a traveling wave in human cadavers within the cochlea. Brownell later made a major advance when he discovered that the ear has a mechanism for sound amplification, via outer hair cell electromotility. Images Figure 1 Figure 2 PMID:15369636

  12. Inner ear lesions in congenital cytomegalovirus infection of human fetuses.

    PubMed

    Teissier, Natacha; Delezoide, Anne-Lise; Mas, Anne-Elisabeth; Khung-Savatovsky, Suonavy; Bessières, Bettina; Nardelli, Jeannette; Vauloup-Fellous, Christelle; Picone, Olivier; Houhou, Nadira; Oury, Jean-François; Van Den Abbeele, Thierry; Gressens, Pierre; Adle-Biassette, Homa

    2011-12-01

    Congenital cytomegalovirus (CMV) infection is the leading cause of non-hereditary congenital sensorineural hearing loss (SNHL). The natural course and the pathophysiology of inner ear lesions during human fetal CMV infection have not yet been reported. Inner ear lesions were investigated in six CMV-infected fetuses aged 19-35 postconceptional weeks and correlated with central nervous system (CNS) lesions. All the fetuses had high viral loads in the amniotic fluid and severe visceral and CNS lesions visible by ultrasound. Diffuse lesions consisting of both cytomegalic cells containing inclusion bodies and inflammation were found within all studied structures including the inner ear, brain, other organs, and placenta, suggesting hematogenous dissemination. Cochlear infection was consistently present and predominated in the stria vascularis (5/6), whereas the supporting cells in the organ of Corti were less often involved (2/6). Vestibular infection, found in 4/6 cases, was florid; the non-sensory epithelia, including the dark cells, were extensively infected. The endolymphatic sac was infected in 1 of 3 cases. The severity of inner ear infection was correlated with the CNS lesions, confirming the neurotropism of CMV. This study documenting infection of the structures involved in endolymph secretion and potassium homeostasis in fetuses with high amniotic fluid viral loads suggests that potassium dysregulation in the endolymphatic compartment of the inner ear may lead to secondary degeneration of the sensory structures. In addition, the occurrence of SNHL depends on the intensity and duration of the viral infection and inflammation. PMID:22033878

  13. Ultrasound-guided endovenous diode laser in the treatment of congenital venous malformations: preliminary experience.

    PubMed

    Sidhu, Manrita K; Perkins, Jonathan A; Shaw, Dennis W W; Bittles, Mark A; Andrews, R Torrance

    2005-06-01

    The authors present their experience in treating congenital venous malformations with ultrasound (US)-guided endovenous diode laser. Six patients underwent treatment of eight venous malformations for complaints including pain, activity limitation, or cosmetic defect. At a mean follow-up interval of 14.5 months, all had either resolution of (five patients) or marked decrease in (one patient) pain, allowing them to resume previously limited activities. There were no instances of nerve damage or skin necrosis. One patient had a self-limited mucosal tongue base ulcer. In this small series of patients, endovenous laser treatment of venous malformations was effective during short-term follow-up. PMID:15947054

  14. [Management of a perineal arteriovenous malformation in a 5-year-old child].

    PubMed

    Galinier, P; Philandrianos, C; Bouali, O; Petit, P; Bardot, J; Salazard, B

    2008-06-01

    Arteriovenous malformations are seldom in children but raise important therapeutic problems. Apart from intracranial arteriovenous malformations, few observations have been described in the literature. We report the case of a superficial perineal arteriovenous malformation in a 5-year-old child. Tailored embolization followed shortly by thorough surgery is the best attitude. Surgery must totally eradicate the lesion to allow hope for a cure. Surgery should be reserved for forms that are extensive or a source of complications. Even total resection does not ensure non-recurrence. PMID:18456473

  15. Utility of 99mTc RBC Scintigraphy in Diagnosing Parotid Venous Malformations.

    PubMed

    Patel, Rosan; Saad, Hannan; Srinivasan, Ashok; Brown, Richard K J

    2015-07-01

    Venous malformations of the parotid glands are uncommon and can be difficult to confirm on routine anatomical imaging alone because of overlap of imaging features with other more common parotid lesions. Tc RBC scans combined with SPECT/CT offer a noninvasive way to diagnose parotid venous malformations without the need for biopsy. Combined with anatomical findings of phleboliths (when present), the diagnosis can be reliably determined. We present a case where MRI and biopsy were inconclusive, but the SPECT/CT was used to confirm the suspicion of a benign venous malformation. PMID:26018710

  16. Congenital cystic adenomatoid malformation presenting as lung abscess in a child.

    PubMed

    Kumar, K Jagadish; Anilkumar, M G; Shivamurthy, Y L; M Kumar, Prashanth

    2012-01-01

    Congenital cystic adenomatoid malformation composes of hamartomatous cystic lung lesions characterised by the presence of abnormal bronchiolar structures of varying sizes or distribution. In most of the cases, respiratory distress occurs during the neonatal period, and in about 90% of patients, congenital cystic adenomatoid malformation is diagnosed before the age of two years due to respiratory infection. We describe a case of congenital cystic adenomatoid malformation presenting as lung abscess in a 3-year six months old male clinically mimicking empyema. He undergone lobectomy and doing well in the follow-up. PMID:23289472

  17. Soft tissue attenuation in middle ear on HRCT: Pictorial review

    PubMed Central

    Anbarasu, Arangasamy; Chandrasekaran, Kiruthika; Balakrishnan, Sivasubramanian

    2012-01-01

    Middle ear disease is a common clinical entity; imaging, especially High resolution Computed Tomography (HRCT), plays a crucial role in diagnosis and assessing the disease extent, helping to decide appropriate management. Temporal bone imaging is challenging and involves thorough understanding of the anatomy, especially in relation to HRCT imaging. Most of the middle ear pathologies appear as “soft tissue” on imaging. Careful analysis of the soft tissue on the HRCT is crucial in achieving the right diagnosis; clinical information is essential and the imaging findings need correlation with clinical presentation and otoscopic findings. The purpose of this pictorial essay is to enlist the pathologies that present as soft tissue in middle ear and to provide a structured and practical imaging approach that will serve as a guide for confident reporting in daily practice. PMID:23833422

  18. MicroRNA expression in the embryonic mouse inner ear.

    PubMed

    Wang, Xian-Ren; Zhang, Xue-Mei; Zhen, Jing; Zhang, Pen-Xing; Xu, Geng; Jiang, Hongyan

    2010-06-23

    Although microRNA (miRNA) is expressed extensively in the postnatal mouse inner ear, its expression in the sensory epithelium during embryogenesis has not been well characterized. We investigated miRNA expression at E13.5 and E16.5 by microarray analysis, quantitative real-time-PCR, and in-situ hybridization. MiRNA-182, miRNA-140, miRNA-200c, and others showed distinct temporal and spatial expression patterns. MiRNA-194, whose expression in zebrafish seems to play an important role in the differentiation of the intestinal epithelium, was also expressed in the spiral ganglia of the mouse inner ear, where it may play a similar role in neuronal differentiation. Our results indicate that miRNAs are widely expressed in the developing inner ear, with more species recruited as hair cells differentiate, suggesting an important developmental role. PMID:20467336

  19. Performance analysis of the Ormia ochracea's coupled ears.

    PubMed

    Akcakaya, Murat; Nehorai, Arye

    2008-10-01

    The Ormia ochracea is able to locate a cricket's mating call despite the small distance between its ears compared with the wavelength. This phenomenon has been explained by the mechanical coupling between the ears. In this paper, it is first shown that the coupling enhances the differences in times of arrival and frequency responses of the ears to the incoming source signals. Then, the accuracy of estimating directions of arrival (DOAs) by the O. ochracea is analyzed by computing the Cramér-Rao bound (CRB). The differential equations of the mechanical model are rewritten in state space and its frequency response is calculated. Using the spectral properties of the system, the CRB for multiple stochastic sources with unknown directions and spectra is asymptotically computed. Numerical examples compare the CRB for the coupled and the uncoupled cases, illustrating the effect of the coupling on reducing the errors in estimating the DOAs. PMID:19062851

  20. Human Action Recognition Using Wireless Wearable In-Ear Microphone

    NASA Astrophysics Data System (ADS)

    Nishimura, Jun; Kuroda, Tadahiro

    To realize the ubiquitous eating habits monitoring, we proposed the use of sounds sensed by an in-ear placed wireless wearable microphone. A prototype of wireless wearable in-ear microphone was developed by utilizing a common Bluetooth headset. We proposed a robust chewing action recognition algorithm which consists of two recognition stages: “chew-like” signal detection and chewing sound verification stages. We also provide empirical results on other action recognition using in-ear sound including swallowing, cough, belch, and etc. The average chewing number counting error rate of 1.93% is achieved. Lastly, chewing sound mapping is proposed as a new prototypical approach to provide an additional intuitive feedback on food groups to be able to infer the eating habits in their daily life context.

  1. Influences on clinical practice: the case of glue ear

    PubMed Central

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

    1999-01-01

    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

  2. Regeneration of the mammalian inner ear sensory epithelium

    PubMed Central

    Wei, Dongguang; Yamoah, Ebenezer N.

    2010-01-01

    Purpose of Review This review will focus on “self-repair” of mammalian inner ear sensory epithelium including (1) recruiting the in situ proliferation and differentiation of endogenous cells at the damaged site and (2) the autologous transplantation Recent findings Self-repair refers to a favorable structural and functional outcome of damaged inner ear sensory epithelium. Our advanced ability of manipulating the fate of inner ear sensory cells makes in situ proliferation a possible candidate of hearing restoration. A practical alternative of the unavoidable immune rejection is to introduce autologous cells. Ependymal cells, induced pluripotent stem (iPS) cells, and olfactory sheath cells have been recognized as promising sources, which will spur ongoing efforts to evaluate the new cell sources for cell replacement therapy. Summary Further exploration of the innate advantages of in situ proliferation and using novel cell sources for autologous transplantation may serve as rehearsals for clinical trials in the near future. PMID:19617827

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

    PubMed

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

    2013-12-01

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

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

    NASA Astrophysics Data System (ADS)

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

    2013-12-01

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

  5. Ion Channel Gene Expression in the Inner Ear

    PubMed Central

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

    2007-01-01

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

  6. Eustachian tube function in patients with inner ear disorders.

    PubMed

    Park, Jonas J-H; Luedeke, Inger; Luecke, Kerstin; Emmerling, Oliver; Westhofen, Martin

    2013-05-01

    The influence of Eustachian tube (ET) dysfunction on the inner ear fluid pressure and thus on the inner ear function in Meniere's disease has been discussed controversially. So far, most of the studies examining ET function in inner ear disorders indirectly analyzed ET function by tympanometric methods. The present study directly studied ET function in inner ear disorders by sonotubometry. Healthy subjects and patients with Meniere's disease, sudden sensorineural hearing loss, cholesteatoma and chronic suppurative otitis media were examined by sonotubometry. Mean increase of sound pressure intensity (dB) and mean duration of sound pressure increase (s) were analyzed. Highest mean increase of sound pressure intensity was seen in healthy subjects when using >5 dB peaks (11.6 ± 0.7 dB) and >0 dB peaks (9.6 ± 0.6 dB). Comparative analysis including bilateral ears showed decreased ET function in patients with cholesteatoma (p = 0.002) and in patients with Meniere's disease (p = 0.003) when using >0 dB peaks. Examination of each specific ET opening maneuver showed impaired ET function in pathological ears of patients with cholesteatoma and with Meniere's disease, during yawning (p = 0.001; p < 0.001), dry swallowing (p = 0.010; p = 0.049), Toynbee maneuver (p = 0.033; p = 0.032) and drinking (p = 0.044; p = 0.027). Mild ET dysfunction is detected in patients with Meniere's disease by direct sonotubometric assessment of ET function. PMID:22941437

  7. Brain malformations in a patient with deletion 2p16.1: A refinement of the phenotype to BCL11A.

    PubMed

    Balci, Tugce B; Sawyer, Sarah L; Davila, Jorge; Humphreys, Peter; Dyment, David A

    2015-01-01

    Microdeletions of 2p15-16.1 have been reported in 15 patients with a recognizable syndrome of dysmorphic features, intellectual disability and microcephaly. Facial features include telecanthus, short palpebral fissures, epicanthal folds, a broad nasal root, smooth and long philtrum and large ears. Brain malformations can be observed in this syndrome and include hypoplasia of the corpus callosum and a simplified cortical gyral pattern. Case reports have narrowed the critical region of the neurodevelopmental phenotype to a region that spans the B-cell CLL/lymphoma 11A (BCL11A) gene. Here we present a 3-year-old normocephalic girl with moderate development delay and dysmorphic features including a prominent forehead, telecanthus, depressed nasal bridge, thin upper vermilion and a small chin. Magnetic resonance imaging shows enlargement of the lateral, third and fourth ventricles and hypoplastic corpus callosum, cerebellar vermis and pons. Array CGH revealed a 0.875 Mb de novo deletion at 2p16.1 that includes only BCL11A. The moderate delays, hypoplastic and dysmorphic corpus callosum and hippocampi and the facial features are in keeping with the previously described 2p15-16.1 microdeletion syndrome. However, hypoplasia of the pons and cerebellum are not commonly recognized features and are reminiscent of the brain malformations observed in individuals with a mutation in CASK. CASK is known to interact with BCL11A in the normal growth of axons. This case report highlights the role of BCL11A in 2p15-16.1 microdeletion syndrome and the unique phenotype suggests a common pathway for BCL11A and other genes in neurodevelopment. PMID:25979662

  8. [Clinical experience with silastic middle-ear prostheses].

    PubMed

    Zelený, M; Voldrich, Z

    1989-01-01

    The authors made clinical tests of silastic prostheses of the middle ear, type PORP, TORP and piston. They used 29 X PORP, 26 X TORP and 21 pistons. They did not reveal any signs of tissue intolerance to Silastic MDX 44-516, which is used for making prostheses. They achieved satisfactory anatomical and functional results. They recorded an auditory gain in more than half the patients (early: PORP 97%, TORP 73%, piston 52%; plasty transplants of ossicles obtained from subjects who died accidentally? For preserfic Council of the Ministry of Health, Czech Socialist Republic, recommended, based on the clinical tests, the manufacture of silastic prostheses of the middle ear. PMID:2540918

  9. Inner ear supporting cells: Rethinking the silent majority

    PubMed Central

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

    2014-01-01

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

  10. Energy localization and frequency analysis in the locust ear.

    PubMed

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

    2014-01-01

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

  11. Pathologic conditions of the external ear and auditory canal.

    PubMed

    Ostrowski, V B; Wiet, R J

    1996-09-01

    Primary care physicians are influential in diagnosing and initiating treatment of most pathologic conditions in patients with a history of hearing loss, chronic ear infection, diabetes, immunosuppression, or otologic symptoms with excessive exposure to sunlight. Lesions of the external ear and the external auditory canal (external acoustic meatus) are significant and common. Patients with such a history should have a thorough basic examination, which can be done with simple tools. Symptoms of hearing loss, otalgia, otorrhea, tinnitus, aural fullness, vertigo, and facial weakness may warrant referral of the patient to an otolaryngologist. The crux of preventing worsening otologic sequelae is early detection and treatment. PMID:8795656

  12. Energy localization and frequency analysis in the locust ear

    PubMed Central

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

    2014-01-01

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

  13. Temporomandibular joint disorder and inner ear pruritus: resolution by eminectomy.

    PubMed

    Pentyala, Sahana; Mysore, Pooja; Moller, Daryn; Pentyala, Srinivas; Kardovich, Richard; Martino, Andrew; Proothi, Michael

    2014-09-01

    Recurrent dislocation of the temporomandibular joint (TMJ) disk is caused by many factors. Dislocation can result in an acute or chronic closed lock condition. Temporomandibular joint dysfunction is often presented with otalgia symptoms. Other aural symptoms such as deafness, tinnitus, pressure/blockage, and vertigo are also commonly presented together with TMJ dysfunction (Clin Otolaryngol Allied Sci. 1980;5:23-36). However, pruritus associated with TMJ dysfunction in the inner ear has never been reported in the literature. We report a case history of TMJ dysfunction and associated inner ear pruritus, which are both resolved by eminectomy. PMID:25072971

  14. Ethical language and decision-making for prenatally diagnosed lethal malformations.

    PubMed

    Wilkinson, Dominic; de Crespigny, Lachlan; Xafis, Vicki

    2014-10-01

    In clinical practice, and in the medical literature, severe congenital malformations such as trisomy 18, anencephaly, and renal agenesis are frequently referred to as 'lethal' or as 'incompatible with life'. However, there is no agreement about a definition of lethal malformations, nor which conditions should be included in this category. Review of outcomes for malformations commonly designated 'lethal' reveals that prolonged survival is possible, even if rare. This article analyses the concept of lethal malformations and compares it to the problematic concept of 'futility'. We recommend avoiding the term 'lethal' and suggest that counseling should focus on salient prognostic features instead. For conditions with a high chance of early death or profound impairment in survivors despite treatment, perinatal and neonatal palliative care would be ethical. However, active obstetric and neonatal management, if desired, may also sometimes be appropriate. PMID:25200733

  15. Intraosseous venous malformations of the zygoma: clarification of misconceptions regarding diagnosis and management.

    PubMed

    Defazio, Michael V; Kassira, Wrood; Camison, Liliana; Meshkov, Lauren; Robinson, Philip G; Kawamoto, Henry K; Thaller, Seth R

    2014-03-01

    Primary intraosseous venous malformations affecting the zygoma are rare vascular lesions, with only 35 cases reported in the surgical literature. Despite the establishment of the binary classification system, which serves to distinguish vascular tumors from malformations, inappropriate use of the term "hemangioma" to describe a variety of distinct vascular anomalies remains widespread. The authors present 3 cases of zygomatic intraosseous venous malformations and summarize the clinical, radiographic, and immunohistochemical features of these lesions. In each case, an insidious clinical course, combined with the pathognomonic finding of radiating trabeculae on computed tomography, suggests the diagnosis of intraosseous venous malformation. Negative glucose transporter isoform 1 immunoreactivity and histopathological analysis were used to reinforce this diagnosis in 1 patient. Management was individualized in each case, based on symptom complex and aesthetic concern. Given that the therapeutic approach to vascular anomalies is dependent on accurate diagnosis, resolution of semantic matters will optimize the management of these lesions. PMID:23241780

  16. Posttraumatic active bleeding of mediastinal mixed vascular malformation: a case report.

    PubMed

    Soudack, Michalle; Vladovski, Eugene; Guralnik, Ludmilla; Ben-Nun, Alon; Engel, Ahuva

    2006-07-01

    A case of an actively bleeding mediastinal mass in a 4-year-old boy who sustained multitrauma is described. A computed tomography (CT) scan of the chest upon admission demonstrated a mediastinal mass, which enlarged significantly as seen by repeat CT scan at 3 days with a concomitant drop in serum hemoglobin levels. The lesion was excised, and pathological examination established the diagnosis of a lymphatic-venous malformation containing bloody fluid. Lymphatic-venous malformations are rare vascular malformations and are usually found in the head and neck and less commonly at other sites. Spontaneous bleeding is a known complication, and there are few reports describing posttraumatic hemorrhage. Our case is the first description in the English medical literature of a posttraumatic bleeding mediastinal mixed vascular malformation and includes sequential CT scans illustrating the dynamic nature of hemorrhage. PMID:16818071

  17. Ethical language and decision-making for prenatally diagnosed lethal malformations

    PubMed Central

    Wilkinson, Dominic; de Crespigny, Lachlan; Xafis, Vicki

    2014-01-01

    Summary In clinical practice, and in the medical literature, severe congenital malformations such as trisomy 18, anencephaly, and renal agenesis are frequently referred to as ‘lethal’ or as ‘incompatible with life’. However, there is no agreement about a definition of lethal malformations, nor which conditions should be included in this category. Review of outcomes for malformations commonly designated ‘lethal’ reveals that prolonged survival is possible, even if rare. This article analyses the concept of lethal malformations and compares it to the problematic concept of ‘futility’. We recommend avoiding the term ‘lethal’ and suggest that counseling should focus on salient prognostic features instead. For conditions with a high chance of early death or profound impairment in survivors despite treatment, perinatal and neonatal palliative care would be ethical. However, active obstetric and neonatal management, if desired, may also sometimes be appropriate. PMID:25200733

  18. Constitutively active Notch4 receptor elicits brain arteriovenous malformations through enlargement of capillary-like vessels

    E-print Network

    Kim, Tyson N.

    Arteriovenous (AV) malformation (AVM) is a devastating condition characterized by focal lesions of enlarged, tangled vessels that shunt blood from arteries directly to veins. AVMs can form anywhere in the body and can cause ...

  19. [Potentialities of magnetic resonance imaging in the complex of prenatal radiation diagnosis of fetal malformations].

    PubMed

    Mikha?lov, M K; Akberov, R F; Anisimov, V I; Iusupov, K F

    2004-01-01

    The purpose of the study was to investigate the potentialities of magnetic resonance imaging (MRI) in the complex of prenatal radiation diagnosis of fetal malformations. Twenty-eight female patients with suspected fetal malformations were examined. Ultrasound study was supplemented by MRI according to a specially developed protocol. Various fetal CNS malformations were diagnosed. These included the Arnold-Chiari syndrome, the Dandy-Walker syndrome, occlusive hydrocephaly, lobular holoprosencephaly, porencephaly, diaphragmatic hernias, anomalies of the abdomen and retroperitoneal space, as well as anomalies of facial structures, including median clefts, and dacryocystocele. The use of MRI in the complex prenatal radiation diagnosis makes it possible to visualize fetal malformation more clearly, contributes to the more adequate prediction of the outcome of pregnancy and to the choice of a management policy for a female patient. PMID:15462048

  20. Craniocervical growth collision: another explanation of the Arnold-Chiari malformation and of basilar impression

    Microsoft Academic Search

    M. Roth

    1986-01-01

    Analysis of neuro-cranio-spinal development suggests a cranio-cervical growth conflict as the cause of the Arnold-Chiari malformation and of basilar impression. The ascending course and elongation of the upper cervical nerves associated with the Arnold-Chiari malformation reflects the abnormal, caudo-cranially proceeding growth of the cervical spine. This is the opposite of the normal cranio-caudal direction of growth (which includes the brain)

  1. Resolution of acute acquired comitant esotropia after suboccipital decompression for Chiari I malformation

    Microsoft Academic Search

    Sabine Defoort-Dhellemmes; Eric Denion; Carl F. Arndt; Isabelle Bouvet-Drumare; Jean-Claude Hache; Patrick Dhellemmes

    2002-01-01

    PURPOSE: To report a case of acute comitant esotropia successfully treated with suboccipital decompression in a 9-year-old male patient with Chiari I malformation.DESIGN: Interventional case report.METHODS: A 9-year-old male with Chiari I malformation had acute onset of diplopia, headache, and comitant esotropia.RESULTS: About 9 months after suboccipital decompression, diplopia resolved and there was near orthophoria on examination 15 months after

  2. Vascular Malformation and Common Keratinocytic Nevus of the Soft Type: Phacomatosis Pigmentovascularis Revisited

    PubMed Central

    Laureano, André; Carvalho, Rodrigo; Amaro, Cristina; Freitas, Isabel; Cardoso, Jorge

    2014-01-01

    Phacomatosis pigmentovascularis is a rare syndrome characterized by the coexistence of a pigmented nevus and a cutaneous vascular malformation. We report a 5-year-old boy with all the typical findings of phacomatosis pigmentovascularis type Ia. Although its existence according to the traditional classification has been questioned, this case represents a very rare association of a capillary vascular malformation and a common keratinocytic nevus of the soft type. PMID:25506441

  3. Cutaneous vascular anomalies. Part I. Hamartomas, malformations, and dilation of preexisting vessels.

    PubMed

    Requena, L; Sangueza, O P

    1997-10-01

    Classification of cutaneous vascular anomalies is difficult because conceptual confusion persists between vascular neoplasms and malformations. However, hemangiomas of the infancy fulfill criteria both for hyperplasia and neoplasm because they result from proliferation of endothelial cells, but often undergo complete regression. Despite these pitfalls we have classified cutaneous vascular anomalies into the following categories: hamartomas, malformations, dilatations of preexisting vessels, hyperplasias, benign neoplasms, and malignant neoplasms. In this first part of our clinicopathologic review of vascular anomalies, hamartomas, malformations, and dilatation of preexisting vessels are covered. Hamartomas include several combined vascular and melanocytic proliferations grouped as phakomatosis pigmentovascularis and the so-called eccrine angiomatous hamartoma that consists of proliferations of both eccrine glands and blood vessels. Vascular malformations result from anomalies of embryologic development, and in some of them the abnormalities of the involved vessels are more functional than anatomic, as is the case of nevus anemicus. In contrast, other cutaneous vascular malformations show striking morphologic abnormalities of the vascular structures. These anatomic vascular malformations are subdivided into the following groups: capillary, venous, arterial, lymphatic, and combined anomalies. Spider angioma, capillary aneurysm-venous lake, and telangiectases are not vascular proliferations at all, but dilations of preexisting vessels. In our opinion, most of the lesions described with the generic term of "angiokeratoma" are not authentic vascular neoplasms, but hyperkeratotic malformations of capillaries and venules or acquired telangiectases of preexisting blood vessels of the papillary dermis. Therefore the first group of these "angiokeratomas" are included in the vascular malformations section, and the second group are covered in the section of dilation of preexisting vessels. Lymphangiectases are considered the lymphatic counterpart of angiokeratomas because they result from ectasia of preexisting lymphatic vessels of the papillary dermis. PMID:9344191

  4. Laser treatment of mucosal venous malformations of the upper aerodigestive tract in 50 patients

    Microsoft Academic Search

    Behfar Eivazi; Susanne Wiegand; Afshin Teymoortash; Andreas Neff; Jochen A. Werner

    2010-01-01

    We examined the clinical appearance of mucosal venous malformations of the upper aerodigestive tract with regard to their\\u000a accessibility to laser therapy and further assessed the value of treatment of these lesions with the Nd:YAG and CO2 lasers. A retrospective study was performed in 50 patients who had undergone laser treatment for mucosal low-flow vascular\\u000a malformations of the upper aerodigestive

  5. Unusual Systemic Artery to Pulmonary Artery Malformation Without Evidence of Systemic Disease, Trauma or Surgery

    SciTech Connect

    Geyik, Serdar; Yavuz, Kivilcim; Keller, Frederick S. [Oregon Health and Sciences University, Dotter Interventional Institute (United States)], E-mail: kellerf@ohsu.edu

    2006-10-15

    Connections between the systemic and pulmonary arterial systems are rare conditions that can be due to either congenital or acquired diseases such as anomalous systemic arterial supply to normal lung, pulmonary sequestration, and systemic supply to pulmonary arteriovenous malformations. Herein, a unique case of systemic artery to pulmonary arterial malformation and its endovascular treatment in a patient with no history of the usual etiologies is reported.

  6. Embolisation of pulmonary arteriovenous malformations: results and follow up in 32 patients

    Microsoft Academic Search

    T J Haitjema; T T Overtoom; C J Westermann; J W Lammers

    1995-01-01

    BACKGROUND--Pulmonary arteriovenous malformations may cause a number of complications when left untreated. Embolisation of the feeding vessels is a relatively new approach and information concerning its efficacy and long term results is scarce. METHODS--Pulmonary arteriovenous malformations with feeding arteries of > 3 mm were treated by embolisation. Right to left shunt fraction and arterial oxygen pressure breathing air (PaO2) were

  7. Pregnancy-associated acquired arteriovenous malformation of the nail unit: a novel cause of localized erythronychia.

    PubMed

    Rutnin, Suthinee; Nguyen, Jennifer V; Miller, Christopher J; DeHoratius, Danielle; Rubin, Adam I

    2012-11-01

    Localized longitudinal erythronychia describes a solitary red streak limited to a single nail unit. Localized longitudinal erythronychia can be caused by both benign and malignant entities. Most commonly, the cause is a benign process, such as an onychopapilloma. We report a case of an acquired subungual arteriovenous malformation that presented as localized longitudinal erythronychia during pregnancy. To our knowledge, this is the first report of localized longitudinal erythronychia arising from a pregnancy-associated arteriovenous malformation of the nail unit. PMID:22882235

  8. Magnetic resonance appearance of slow flow vascular malformations of the brainstem

    Microsoft Academic Search

    C. Griffin; R. DeLaPaz; D. Enzmann

    1987-01-01

    Twelve patients with slow flow brain stem vascular malformations had magnetic resonance scans performed on a 1.5 Tesla scanner using T1 (TR=400–800 ms, TE=25 ms) and T2 (TR=2000 ms, TE=20, 25, 80 ms) weighted scans. Eight patients (70%) had solitary brainstem vascular malformations while 4 (30%) had multiple lesions both supra and infratentorially. Five patients had venous angiomas demonstrated by

  9. A case of Dandy-Walker malformation associated with occipital meningocele, microphthalamia, and cleft palate

    Microsoft Academic Search

    S. Nishimaki; H. Yoda; K. Seki; T. Kawakami; H. Akamatsu; Y. Iwasaki

    1990-01-01

    We present a case of Dandy-Walker malformation associated with occipital meningocele, microphthalmia, and cleft palate. Small numbers of cases of Dandy-Walker malformation with occipital meningocele have been described in the literature, but to our knowledge, non of these also had microphthalmia or cleft palate. This association suggests that time of intrauterine origin of Dandy-Walker syndrome was in the sixth or

  10. A symphony of inner ear developmental control genes

    PubMed Central

    2010-01-01

    The inner ear is one of the most complex and detailed organs in the vertebrate body and provides us with the priceless ability to hear and perceive linear and angular acceleration (hence maintain balance). The development and morphogenesis of the inner ear from an ectodermal thickening into distinct auditory and vestibular components depends upon precise temporally and spatially coordinated gene expression patterns and well orchestrated signaling cascades within the otic vesicle and upon cellular movements and interactions with surrounding tissues. Gene loss of function analysis in mice has identified homeobox genes along with other transcription and secreted factors as crucial regulators of inner ear morphogenesis and development. While otic induction seems dependent upon fibroblast growth factors, morphogenesis of the otic vesicle into the distinct vestibular and auditory components appears to be clearly dependent upon the activities of a number of homeobox transcription factors. The Pax2 paired-homeobox gene is crucial for the specification of the ventral otic vesicle derived auditory structures and the Dlx5 and Dlx6 homeobox genes play a major role in specification of the dorsally derived vestibular structures. Some Micro RNAs have also been recently identified which play a crucial role in the inner ear formation. PMID:20637105

  11. Characterization of bunny-ear antennas for wireless basestation applications

    Microsoft Academic Search

    Marc C. Greenberg; Kathleen L. Virga

    1998-01-01

    The bunny-ear antenna (BEA) which has use for wireless basestation applications, mobile satellite communications, and radar imaging is characterized in detail. Whether the BEA is used as an isolated antenna or in an array, it needs to be well understood. A design methodology for the BEA and examples are presented. To discuss the effects from removing metalization near the feed,

  12. A symphony of inner ear developmental control genes.

    PubMed

    Chatterjee, Sumantra; Kraus, Petra; Lufkin, Thomas

    2010-01-01

    The inner ear is one of the most complex and detailed organs in the vertebrate body and provides us with the priceless ability to hear and perceive linear and angular acceleration (hence maintain balance). The development and morphogenesis of the inner ear from an ectodermal thickening into distinct auditory and vestibular components depends upon precise temporally and spatially coordinated gene expression patterns and well orchestrated signaling cascades within the otic vesicle and upon cellular movements and interactions with surrounding tissues. Gene loss of function analysis in mice has identified homeobox genes along with other transcription and secreted factors as crucial regulators of inner ear morphogenesis and development. While otic induction seems dependent upon fibroblast growth factors, morphogenesis of the otic vesicle into the distinct vestibular and auditory components appears to be clearly dependent upon the activities of a number of homeobox transcription factors. The Pax2 paired-homeobox gene is crucial for the specification of the ventral otic vesicle derived auditory structures and the Dlx5 and Dlx6 homeobox genes play a major role in specification of the dorsally derived vestibular structures. Some Micro RNAs have also been recently identified which play a crucial role in the inner ear formation. PMID:20637105

  13. EARS: Toward Fast Analysis of 3D Human

    E-print Network

    Anthropometric Rating System (EARS), an integrated collection of tunable semi-automatic procedures to compute during the upcoming large scan anthropometric survey. Keywords: 3D Human Body Scan, Mesh Segmentation in Anthropometric research and studies. 3D scans fully preserve the body shape measurements, and are the data

  14. Research Project on Ear Infections Dramatizes Challenge of Conflicts.

    ERIC Educational Resources Information Center

    Cordes, Colleen

    1993-01-01

    An unusual conflict-of-interest case involving the federal government, a university researcher on ear infections, and the pharmaceutical industry has renewed the debate over what constitutes unacceptable conflict of interest, federal review of medical treatment, government protection against research bias, and disclosure of research project…

  15. Auditory Responses in Normal-Hearing, Noise-Exposed Ears

    E-print Network

    Stamper, Greta

    2013-12-31

    and a click stimulus. DPOAEs were assessed at 1, 2 and 4 kHz. Significantly smaller amplitudes were seen in wave I of the ABR in response to high-level (e.g., 70 to 90 dB nHL) click and 4 kHz tone bursts in ears with greater noise-exposure backgrounds...

  16. Acoustic Reflectometry versus Tympanometry in Pediatric Middle Ear Screenings.

    ERIC Educational Resources Information Center

    Holmes, Alice E.; And Others

    1989-01-01

    Use of acoustic reflectometry was compared with tympanometry in middle ear screenings for 357 children, aged 5 months to 19 years. Results were analyzed according to sex, age, and sensorineural hearing status. Intratest reliability was highly significant and positive predictive accuracy and specificity rates were excellent, but sensitivity rates…

  17. 3D Ear Print Authentication using 3D Radon Transform

    Microsoft Academic Search

    W. A. Mahmoud; M. R. Shaker

    2006-01-01

    This paper introduces a proposed method authentication based upon 3D Radon transform. It considers the three dimensional ear of human as a personal identification number. Next, it produces the required features using the 3D Radon transform. This transform is adapted from its 2D form to adequate this application. The neural network was used in the identification phase, the evaluation test

  18. Atlas of the developing inner ear in zebrafish.

    PubMed

    Bever, Michele Miller; Fekete, Donna M

    2002-04-01

    This report provides a description of the normal developing inner ear of the zebrafish, Danio rerio, with special focus on the pars inferior. Zebrafish specimens, ranging in age from 3 to 30 days postfertilization (dpf), were processed for standard histologic sections or with a paint-fill method to show three-dimensional morphogenesis of the membranous labyrinth. Adult zebrafish (age 2 years) were also processed for inner ear paint-fills. Although development of the semicircular canals occurs rapidly (by 3 dpf), the pars inferior develops more gradually during days 5-20 postfertilization. A rudimentary endolymphatic duct emerges by 8 dpf. Differentiated hair cells of the lagenar macula are evident by 15 dpf, in a chamber located lateral and posterior to the saccule. By 20 dpf, the saccule itself is separated from the utricle, but remains connected by means of the utriculosaccular foramen. The maculae neglectae, each with differentiated hair cells, lie on the floor of the utricle near this foramen. A medial connection between the sacculi of right and left ears, the transverse canal, is also complete by 20 dpf. A ridge of mesenchyme, previously undescribed, bisects the saccule in zebrafish fry at 20-30 dpf. The images in the paint-fill atlas should provide a baseline for future studies of mutant zebrafish ears. PMID:11921341

  19. Behaviour and cognitive outcomes from middle ear disease

    PubMed Central

    Bennett, K.; Haggard, M.

    1999-01-01

    OBJECTIVES—To resolve controversies over associations between a history of middle ear disease and psychosocial or cognitive/educational outcomes?DESIGN—Multipurpose longitudinal birth cohort study. Original cohort comprised all UK births between 5 and 11 April 1970; data were available for approximately 12 000 children at 5 years old and 9000 children at 10 years old.?METHODS—For 5 year old children, parent reported data were available on health, social, and behavioural factors, including data on two validated markers of middle ear disease. Cognitive tests were administered at 5 and 10 years of age, and behavioural problems rated at 10 years by the child's teacher.?RESULTS—After adjustment for social background and maternal malaise, the developmental sequelae of middle ear disease remained significant even at 10 years. The largest effects were observed in behaviour problems and language test data at age 5, but effect sizes were modest overall.?IMPLICATIONS—These results provide an epidemiological basis for policies that aim to minimise the sequelae of middle ear disease by awareness in parents and preschool teachers, early referral, and intervention for more serious or persistent cases.?? PMID:10325755

  20. MOUSE-EAR OF PECAN: II. INFLUENCE OF NUTRIENT APPLICATIONS

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Mouse-ear(ME) is a severe growth disorder affecting pecan [Carya illinoinensis (Wangenh.) K. Koch] trees in orchards within the Gulf Coast Coastal Plain of the southeastern U.S. Slight to moderate ME was corrected by foliar sprays of either Cu, Mn, or GA3 shortly after budbreak, but sprays were ine...

  1. MOUSE-EAR OF PECAN: I. SYMPTOMOLOGY AND OCCURRENCE

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Mouse-ear is a potentially severe anomalous growth disorder affecting young pecan [Carya illinoinensis (Wangenh.) K. Koch] trees in portions of the Gulf Coast Coastal Plain of the southeastern U.S. A survey of its incidence and severity found it to be commonly exhibited by replants on second-genera...

  2. Mechanics of a 'simple' ear: tympanal vibrations in noctuid moths.

    PubMed

    Windmill, J F C; Fullard, J H; Robert, D

    2007-08-01

    Anatomically, the ears of moths are considered to be among the simplest ears found in animals. Microscanning laser vibrometry was used to examine the surface vibrations of the entire tympanal region of the ears of the noctuid moths Agrotis exclamationis, Noctua pronuba, Xestia c-nigrum and Xestia triangulum. During stimulation with ultrasound at intensities known to activate receptor neurones, the tympanum vibrates with maximum deflection amplitudes at the location where the receptor cells attach. In the reportedly heterogeneous tympana of noctuid moths, this attachment site is an opaque zone that is surrounded by a transparent, thinner cuticular region. In response to sound pressure, this region moves relatively little compared with the opaque zone. Thus, the deflections of the moth tympanic membrane are not those of a simple circular drum. The acoustic sensitivity of the ear of N. pronuba, as measured on the attachment site, is 100+/-14 nm Pa(-1) (N=10), corresponding to tympanal motion of a mere 200 pm at sound pressure levels near the neural threshold. PMID:17644678

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

    USGS Publications Warehouse

    Jensen, W.I.; Cotter, S.E.

    1976-01-01

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

  4. Early Middle Ear Effusion and Language at Age Seven

    ERIC Educational Resources Information Center

    Johnson, Dale L.; McCormick, David P.; Baldwin, Constance D.

    2008-01-01

    This study examined the relation of middle ear effusion (MEE) in the first 3 years of life to language outcomes at age seven. It was hypothesized, on the basis of a literature review, that (1) a low, but positive relation between early MEE and language measures in general will be observed at age seven, and (2) major effects will be demonstrated…

  5. MicroRNAs in sensorineural diseases of the ear

    PubMed Central

    Ushakov, Kathy; Rudnicki, Anya; Avraham, Karen B.

    2013-01-01

    Non-coding microRNAs (miRNAs) have a fundamental role in gene regulation and expression in almost every multicellular organism. Only discovered in the last decade, miRNAs are already known to play a leading role in many aspects of disease. In the vertebrate inner ear, miRNAs are essential for controlling development and survival of hair cells. Moreover, dysregulation of miRNAs has been implicated in sensorineural hearing impairment, as well as in other ear diseases such as cholesteatomas, vestibular schwannomas, and otitis media. Due to the inaccessibility of the ear in humans, animal models have provided the optimal tools to study miRNA expression and function, in particular mice and zebrafish. A major focus of current research has been to discover the targets of the miRNAs expressed in the inner ear, in order to determine the regulatory pathways of the auditory and vestibular systems. The potential for miRNAs manipulation in development of therapeutic tools for hearing impairment is as yet unexplored, paving the way for future work in the field. PMID:24391537

  6. A refractory case of erythromelalgia involving the ears

    Microsoft Academic Search

    Claudia C. Ramirez; Robert S. Kirsner

    2004-01-01

    Erythromelalgia is a rare syndrome that is characterized by episodic attacks of burning pain, erythema, and increased temperature usually affecting the extremities, which is aggravated by warmth or exercise. We describe a patient with a 3-year history of refractory burning pain and red ears. A review of clinical features, disease classification, associated diseases, and treatment of this disease is presented.

  7. Analysis of chick (Gallus gallus) middle ear columella formation

    Microsoft Academic Search

    Jamie L Wood; Ami J Hughes; Kathryn J Mercer; Susan C Chapman

    2010-01-01

    BACKGROUND: The chick middle ear bone, the columella, provides an accessible model in which to study the tissue and molecular interactions necessary for induction and patterning of the columella, as well as associated multiple aspects of endochondral ossification. These include mesenchymal condensation, chondrogenesis, ossification of the medial footplate and shaft, and joint formation between the persistent cartilage of the extracolumella

  8. ACOUSTIC STIMULATION OF THE EAR OF THE GOLDFISH (CARASSIUS AURATUS)

    Microsoft Academic Search

    RICHARD R. FAY; ARTHUR N. POPPER

    1974-01-01

    SUMMARY Microphonic potentials were recorded from the ears of the goldfish during acoustic stimulation in a situation where sound pressure and particle dis- placement could be varied. Microphonic potentials from fishes with the swim bladder intact were proportional to sound pressure. After removal of the swim bladder, sound pressure sensitivity declined by 20-35 dB and the response was generated in

  9. Maize resistance to gibberella ear rot: symptoms, deoxynivalenol, and yield

    Microsoft Academic Search

    B. Vigier; L. M. Reid; L. M. Dwyer; D. W. Stewart; R. C. Sinha; J. T. Arnason; G. Butler

    2001-01-01

    To investigate the effect of different environments on maize resistance to gibberella ear rot, disease symptoms, deoxynivalenol (DON) concentration, and grain yield were measured in three maize (Zea mays L.) inbred lines and five hybrids, from 1994 to1996, at six locations in eastern Canada. At each location, all genotypes were inoculated with a three-isolate macroconidial mix of Fusarium graminearum Schwabe

  10. Evaluating commercial maize hybrids for resistance to gibberella ear rot

    Microsoft Academic Search

    A. W. Schaafsma; R. W. Nicol; L. M. Reid

    1997-01-01

    An integral component of breeding maize for resistance to Fusarium graminearum ear rot is the identification of resistant genotypes. Since natural infection is not consistent from year to year, maize researchers must use manual techniques to inoculate the plant material with fungal spores. Information is presented here on site resistance of commercial maize hybrids to F. graminearum over three years

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

    Microsoft Academic Search

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

    2000-01-01

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

  12. Cardiovascular malformations in Smith-Lemli-Opitz syndrome

    SciTech Connect

    Lin, A.E.; Ardinger, H.H.; Ardinger, R.H. Jr. [Univ. of Kansas, Kansas City, KS (United States)] [and others] [Univ. of Kansas, Kansas City, KS (United States); and others

    1997-01-31

    We reviewed 215 patients (59 new, 156 from the literature) with Smith-Lemli-Opitz syndrome (SLOS), and found that 95 (44%) had a cardiovascular malformation (CVM). Classifying CVMs by disordered embryonic mechanisms, there were 5 (5.3%) class I (ectomesenchymal tissue migration abnormalities), 56 (58.9%) class II (abnormal intracardiac blood flow), 25 (26.3%) class IV (abnormal extracellular matrix), and 5 (5.3%) class V (abnormal targeted growth). Comparing the frequencies of individual CVMs in this series with a control group (the Baltimore-Washington Infant Study), there were 6 individual CVMs which showed a significant difference from expected values. When frequencies of CVMs in SLOS were analyzed by mechanistic class, classes IV and V were significantly more frequent, and class I significantly less frequent, than the control group. Although CVMs in SLOS display mechanistic heterogeneity, with an overall predominance of class II CVMs, the developmental error appears to favor alteration of the cardiovascular developmental mechanisms underlying atrioventricular canal and anomalous pulmonary venous return. This information should assist the clinical geneticist evaluating a patient with possible SLOS, and should suggest research direction for the mechanisms responsible for the SLOS phenotype. 102 refs., 1 fig., 7 tabs.

  13. Antenatally diagnosed congenital cystic adenomatoid malformations (CCAM): Research Review

    PubMed Central

    Di Prima, Fosca Antonia Francesca; Bellia, Adriano; Inclimona, Genny; Grasso, Francesco; Teresa, Maria; Cassaro, Meli Nazario

    2012-01-01

    Summary Prenatal identification of lung abnormalities has increased with prenatal surveillance. With the advent of improved antenatal imaging over the past ten years, the diagnosis, assessment and management of congenital cystic lung abnormalities have changed. These were once considered the exclusive domain of the surgeon, who had the authority to operate on all congenital cystic lung abnormalities regardless of size or clinical signs in order to avoid the risk of cancer and improve lung growth in even asymptomatic infants. Clinicians are reconsidering this approach in the light of the spontaneous improvement and possible resolution that occurs over months to years with many of these lesions, thinking about the opportunity to take a more conservative approach in many minimally symptomatic or asymptomatic infants in the early months of life. The risks of subsequent cancer are poorly understood and probably overstated. Many centers advocate surgery only in cases of symptomatic or significant lesions, although there is little consensus as to what constitutes a significant lesion. This article will review current knowledge (classification, pathogenesis, genetics, prenatal evaluation, clinical implications) on congenital cystic adenomatoid malformations (CCAM) and discuss management options for young children with these lung abnormalities. PMID:22905308

  14. Cerebral cavernous malformations: natural history and clinical management.

    PubMed

    Gross, Bradley A; Du, Rose

    2015-07-01

    Cavernous malformations (CMs) are angiographically-occult clusters of dilated sinusoidal channels that may present clinically with seizures, focal neurological deficits and/or hemorrhage. Across natural history studies, the annual hemorrhage rate ranged from 1.6-3.1% per patient-year, decreasing to 0.08-0.2% per patient-year for incidental CMs and to 0.3-0.6% for the collective group of unruptured CMs. Prior hemorrhage is a significant risk factor for subsequent CM hemorrhage. Hemorrhage clustering, particularly within the first 2 years, is an established phenomenon that may confound results of natural history studies evaluating the rate of rehemorrhage. Indeed, rehemorrhage rates for hemorrhagic CMs range from 4.5-22.9% in the literature. Surgical resection is the gold standard treatment for surgically-accessible, symptomatic CMs. Incidental CMs or minimally symptomatic, surgically inaccessible eloquent lesions may be considered for observation. Stereotactic radiosurgery is a controversial treatment approach of consideration only for cases of highly aggressive, surgically inaccessible CMs. PMID:26098013

  15. An Infiltrative Angioarchitectural Variant of Arteriovenous Malformation of Temporalis

    PubMed Central

    Byatnal, Aditi Amit; Rakheja, Mahima; Byatnal, Amit Raghavendra; Narayanaswamy, Venkadasalapathy

    2014-01-01

    Vascular anomalies of the head and neck region pose a certain diagnostic and therapeutic paradox. Management of arteriovenous malformations (AVM) is a challenge owing to the presence of abnormal vascular communications and high recurrence. We report a case of a 19-year-old male patient, who presented with diffuse swelling in the right temporal region. Magnetic Resonance Angiography (MRA) suggested it to be an AVM in the temporalis muscle, having afferents in the ascending pharyngeal artery, with cavernous angioma. Surgical excision of the lesion was carried out under carotid control. Histopathology of the excised specimen utilizing special stains confirmed the presence of AVM. An absence of distinct nidus concomitant along with the exuberant proliferation of capillaries between the muscle fibres suggested it be an infiltrative angioarchitectural variant. The present case highlights significance of diagnosing AVM in temporalis muscle which is a rare occurrence in head and neck region. Also, the importance of ruling out other closely resembling vascular diathesis, both non neoplastic and malignant is discussed. PMID:25386534

  16. Upper limb malformations in chromosome 22q11 deletions

    SciTech Connect

    Shalev, S.A.; Dar, H.; Barel, H.; Borochowitz, Z. [Bnai Zion Medical Center, Haifa (Israel)

    1996-03-29

    We read with interest the report of Cormier-Daire et al. in a recent issue of the journal, describing upper limb malformations in DiGeorge syndrome. We observed a family with this group of rare clinical expression of chromosome 22q11 deletions. The proposita was examined in our clinic when she was 4 years old. She was mildly mentally retarded. Clinical evaluation showed normal growth, long thin nose with squared tip, nasal speech, and abundant scalp hair and no cardiac anomalies. The girl was accompanied by her mother. Facial similarities were noted between the two. The mother reported to be treated with oral calcium due to hypoparathyroidism, diagnosed several years ago. Clinical evaluation showed wide flat face, short stature, mild mental retardation, slight hypertelorism, peculiar nose similar to her daughter`s, and nasal speech. No cardiac anomalies were found. Recently, a brother was born. Clinical examination documented large ventriculo-septal defect, retrognathia, narrow palpebral fissures, and long thin nose with squared tip. 1 ref.

  17. Kabuki Syndrome and Anorectal Malformations: Implications for Diagnosis and Treatment

    PubMed Central

    Siminas, Sotirios; Baillie, Colin Tennant; Turnock, Richard

    2015-01-01

    Kabuki syndrome (KS) is a rare genetic condition characterized by a distinctive facies, intellectual disability, growth delay, and a variety of skeletal, visceral, and other anomalies, including anorectal malformations (ARMs). We present two cases of female patients with KS, diagnosed and successfully managed at our institution, one with a perineal fistula and one with a rectovestibular fistula. Our report, along with a literature review, shows that the syndrome is usually associated with “low” anomalies, with a potential for a good prognosis. Management of the anorectal anomaly in patients with KS is not essentially different from that in other nonsyndromic patients, taking into account the frequent association of the syndrome with serious congenital heart disease, which might affect the decision-making and timing of the stages of anorectal reconstruction. The frequent occurrence of learning and feeding difficulties makes establishment of toilet training and bowel management rather more challenging, requiring the expertise of a multidisciplinary team. The finding of ARMs in female patients with other characteristics of KS, although inconstant, could support the clinical suspicion for the syndrome until genetic confirmation is available, and should alert the physician for the potential of severe cardiac defects. PMID:26171318

  18. Gastrointestinal stromal tumor mimicking arteriovenous malformation of the jejunum.

    PubMed

    Shiozawa, Kazue; Watanabe, Manabu; Igarashi, Yoshinori; Ichimori, Mioe; Hirano, Naoki; Nakano, Shigeru; Maeda, Tetsuya; Yamazaki, Kunihiro; Okubo, Youichiro; Nemoto, Tetsuo; Shibuya, Kazutoshi; Sumino, Yasukiyo

    2011-09-01

    There have been case reports of small intestinal gastrointestinal stromal tumors (GISTs) complicated with arteriovenous malformation (AVM) and angiodysplasia and exhibiting intense tumor staining. Herein we report a GIST of the small intestine that showed tumor staining and early venous return on imaging studies, and so the patient was suspected to have AVM. A 62-year-old male presented with intermittent pain in the left abdominal region. Contrast-enhanced computed tomography revealed a 15-mm-long spindle-shaped mass showing intense tumor staining and early venous return through the jejunal vein. In the arterial phase, the attenuation value of the tumor was 250 Hounsfield units. Color Doppler ultrasonography simultaneously delineated vessels extending from the serosal side and turbulent signals showing a mosaic pattern in the tumor. On angiography, intense staining was observed in the peripheral part of the second branch of the jejunal artery. Although these findings suggested AVM, the tumor was diagnosed as a GIST based on pathological examination of the resected specimens. In this case, no AVM or change in vascular density was noted despite the careful examination of pathological specimens, and the cause of the tumor staining remained unknown. PMID:22110415

  19. Acute respiratory distress syndrome after onyx embolization of arteriovenous malformation.

    PubMed

    Tawil, Isaac; Carlson, Andrew P; Taylor, Christopher L

    2011-01-01

    Purpose. We report a case of a 60-year-old male who underwent sequential Onyx embolizations of a cerebral arteriovenous malformation (AVM) which we implicate as the most likely etiology of subsequent acute respiratory distress syndrome (ARDS). Methods. Case report and literature review. Results. Shortly after the second Onyx embolization procedure, the patient declined from respiratory failure secondary to pulmonary edema. Clinical entities typically responsible for pulmonary edema including cardiac failure, renal failure, iatrogenic volume overload, negative-pressure pulmonary edema, and infectious etiologies were evaluated and excluded. The patient required mechanical ventilatory support for several days, delaying operative resection. The patient met clinical and radiographic criteria for ARDS. After excluding other etiologies of ARDS, we postulate that ARDS developed as a result of Onyx administration. The Onyx copolymer is dissolved in dimethyl sulfoxide (DMSO), a solvent excreted through the lungs and has been implicated in transient pulmonary side effects. Additionally, a direct toxic effect of the Onyx copolymer is postulated. Conclusion. Onyx embolization and DMSO toxicity are implicated as the etiology of ARDS given the lack of other inciting factors and the close temporal relationship. A strong physiologic rationale provides further support. Clinicians should consider this uncommon but important complication. PMID:21687580

  20. Urinary tract anomalies in patients with anorectal malformation.

    PubMed

    Islam, M N; Hasina, K; Reza, M S; Hasanuzzaman, S M; Akter, T; Talukder, S A

    2015-04-01

    Urinary tract anomalies are common associated anomalies in anorectal malformation (ARM) and major contributory factor for high morbidity and mortality in ARM. In this study, urinary tract anomalies were evaluated in 72 patients of ARM (43 high varieties & 29 low varieties) that were admitted in Dhaka Medical College & Hospital (DMCH) during the period of January 2007 to April 2008. In all cases, evaluation was done by ultrasonogram (USG) and voiding cysto-urethrography (VCUG). Intravenous urography (IVU) was done in selected cases. Urinary tract anomalies were found in 20(27.85%) cases of ARM patients. These anomalies were significantly higher 16(37.2%) in high variety of ARM than 4(13.8%) those with low variety of ARM. The pattern of urological anomalies were vesicoureteral reflux (VUR - 35%), hydronephrosis due to PUJ obstruction (25%), posterior urethral valve (PUV - 15%), renal agenesis (10%), duplex ureter (10%) and bladder diverticulum (5%). Before definite management of ARM urological evaluation is essential to prevent renal damage and to reduce the morbidity and mortality. PMID:26007265

  1. Genetics of cerebral cavernous malformations: current status and future prospects.

    PubMed

    Choquet, H; Pawlikowska, L; Lawton, M T; Kim, H

    2015-09-01

    Cerebral cavernous malformations (CCM) are vascular lesions which affect up to 0.5% of the general population, predisposing to headaches, seizures, cerebral hemorrhages and focal neurological deficits. CCM occurs in both sporadic and familial forms; familial cases follow an autosomal-dominant mode of inheritance and are caused by mutations in CCM1 (KRIT1), CCM2 (MGC4607), or CCM3 (PDCD10). Somatic mutations within the three CCM genes have been identified in CCM lesions from both sporadic and familial patients. We reviewed articles published in PubMed in English prior to March 2015 and provide an update on CCM mutations and the screening strategies used to identify them. Further, we summarize the specific clinical features related to CCM genotypes. As 5% to 15% of familial CCM cases remain genetically unexplained, we also discuss future approaches to expand understanding of the genetic architecture of CCM. Finally, we discuss possible genetic modifiers of CCM disease severity and progression. Understanding the genetic architecture of CCM is essential for an earlier diagnosis of the disease, predictive testing of at-risk patients, and design of targeted medical therapies of which there are currently none available. PMID:25900426

  2. Microsurgical Treatment and Outcome of Pediatric Supratentorial Cerebral Cavernous Malformation

    PubMed Central

    Noh, Jung-Hoon; Cho, Kyung Rae; Yeon, Je Young; Seol, Ho Jun

    2014-01-01

    Objective The purpose of this study was to investigate the clinical features and outcomes of pediatric cavernous malformation (CM) in the central nervous system. Methods Twenty-nine pediatric patients with supratentorial CM underwent microsurgical excision. In selected cases, transparent tubular retractor system (TTRS) was used to reduce retraction injury and intraoperative neuromonitoring (IONM) was held to preserve functioning cortex. Patients' demographics and symptoms were reviewed and surgical outcomes were discussed. Results The main initial clinical manifestations included the following : seizures (n=13, 45%), headache (n=7, 24%), focal neurological deficits (n=3, 10%), and an incidental finding (n=6, 21%). Overt hemorrhage was detected in 7 patients (24%). There were 19 children (66%) with a single CM and 10 (34%) children with multiple CMs. In 7 cases with deep-seated CM, we used a TTRS to minimize retraction. In 9 cases which location of CM was at eloquent area, IONM was taken during surgery. There was no major morbidity or mortality after surgery. In the 29 operated children, the overall long-term results were satisfactory : 25 (86%) patients had no signs or symptoms associated with CMs, 3 had controllable seizures, and 1 had mild weakness. Conclusion With the assistance of neuronavigation systems, intraoperative neuromonitoring, and TTRS, CMs could be targeted more accurately and excised more safely. Based on the satisfactory seizure outcome achieved, complete microsurgical excision in children is recommended for CMs presenting with seizures but removal of hemosiderin-stained areas seems to be unnecessary. PMID:25368767

  3. Gastric foregut cystic developmental malformation: Case series and literature review

    PubMed Central

    Geng, Yan-Hua; Wang, Chang-Xing; Li, Jiang-Tao; Chen, Qing-Yu; Li, Xiu-Zhen; Pan, Hao

    2015-01-01

    Foregut cystic developmental malformation (FCDM) is a very rare lesion of the alimentary tract, especially in the stomach. We discuss the concepts of gastric duplication cyst, bronchogenic cysts, and FCDM. Nomenclature has been inconsistent and confusing, but, by some definitions, gastric duplication cysts involve gastric mucosa and submucosal glands, bronchogenic cysts involve respiratory mucosa with underlying cartilage and glands, and FCDM lacks gastric mucosa or underlying glands or cartilage but has pseudostratified ciliated columnar epithelium (PCCE). We searched our departmental case files from the past 15 years and identified 12 cases of FCDM in the alimentary tract. We summarize the features of these 12 cases including a report in detail on a 52-year-old man with a submucosal cyst lined with simple PCCE and irregular and stratified circular muscle layers that merged with gastric smooth muscle bundles near the lesser curvature of the gastric cardia. A literature review of cases with this histology yielded 25 cases. We propose the term gastric-FCDM for such cases. Our own series of 12 cases confirms that preoperative recognition of the entity is infrequent and problematic. The rarity of this developmental disorder, as well as a lack of understanding of its embryologic origins, may contribute to missing the diagnosis. Not appreciating the diagnosis preoperatively can lead to an inappropriate surgical approach. In contrast, presurgical recognition of the entity will contribute to a good outcome and reduced risk of complications. PMID:25593458

  4. Biology of cerebral arteriovenous malformations with a focus on inflammation.

    PubMed

    Mouchtouris, Nikolaos; Jabbour, Pascal M; Starke, Robert M; Hasan, David M; Zanaty, Mario; Theofanis, Thana; Ding, Dale; Tjoumakaris, Stavropoula I; Dumont, Aaron S; Ghobrial, George M; Kung, David; Rosenwasser, Robert H; Chalouhi, Nohra

    2015-02-01

    Cerebral arteriovenous malformations (AVMs) entail a significant risk of intracerebral hemorrhage owing to the direct shunting of arterial blood into the venous vasculature without the dissipation of the arterial blood pressure. The mechanisms involved in the growth, progression and rupture of AVMs are not clearly understood, but a number of studies point to inflammation as a major contributor to their pathogenesis. The upregulation of proinflammatory cytokines induces the overexpression of cell adhesion molecules in AVM endothelial cells, resulting in enhanced recruitment of leukocytes. The increased leukocyte-derived release of metalloproteinase-9 is known to damage AVM walls and lead to rupture. Inflammation is also involved in altering the AVM angioarchitecture via the upregulation of angiogenic factors that affect endothelial cell proliferation, migration and apoptosis. The effects of inflammation on AVM pathogenesis are potentiated by certain single-nucleotide polymorphisms in the genes of proinflammatory cytokines, increasing their protein levels in the AVM tissue. Furthermore, studies on metalloproteinase-9 inhibitors and on the involvement of Notch signaling in AVMs provide promising data for a potential basis for pharmacological treatment of AVMs. Potential therapeutic targets and areas requiring further investigation are highlighted. PMID:25407267

  5. Malformation of the human superior olive in autistic spectrum disorders.

    PubMed

    Kulesza, Randy J; Lukose, Richard; Stevens, Lisa Veith

    2011-01-01

    Autistic spectrum disorders (ASD) comprise a continuum of psychosocial disorders clinically characterized by social difficulties, impaired communication skills and repetitive behavioral patterns. Despite the prevalence of ASD, the neurobiology of this disorder is poorly understood. However, abnormalities in neuronal morphology, cell number and connectivity have been described throughout the autistic brain. Further, there is ample evidence that auditory dysfunction is a common feature of autism. Our preliminary investigation of neuronal morphology in the auditory brainstem of individuals with ASD focused on the medial superior olive (MSO) and revealed that neurons in this region were significantly smaller and rounder than in controls. In this report, we expand our investigation to examine all nuclei within the human superior olivary complex (SOC), an important auditory brainstem center. We examine neuronal morphology and neuronal number in four control (average age=15 years) and 9 autistic brains (average age=15 years). This detailed investigation supports our previous descriptions of the MSO, and also reveals significant dysmorphology in five other SOC nuclei. Moreover, we provide evidence of a consistent and significant decrease in the number of SOC neurons in the autistic brain. Our studies implicate an extensive malformation of the auditory brainstem in the hearing and language difficulties in individuals with ASD. The results from this investigation suggest that neonatal testing of auditory function may aid in the identification of individuals with ASD earlier than presently possible. PMID:20946889

  6. [Correlation between inner ear disorders and temporomandibular joint diseases].

    PubMed

    Kempf, H G; Roller, R; Mühlbradt, L

    1993-01-01

    Acute and chronic inner ear diseases involve many etiological factors, some as yet unknown. ENT-specific, orthopedic, hemorrheological, immunological and neurological disorders can affect the cochleovestibular system and induce hearing loss, vertigo and/ortinnitus. We performed a prospective study to analyze factors of the dentognathological system and of the temporomandibular joint that can influence acute and chronic inner ear dysfunctions. A total of 138 patients (49.3% female, 50.9% male) receiving clinical treatment for inner ear dysfunctions (12.3% chronic sensorineural hearing loss, 15.2% Ménière's disease, 52.2% sudden hearing loss, 13.8% isolated tinnitus, 6.5% recurrent hearing loss) underwent a prospective dental and gnathological examination. In particular, the patient's dental status and a functional investigation of the masticatory muscles and the temporomandibular joint were analyzed. In 20.3% patients the examination showed no pathology of the dentognathological system. In contrast, there were pathological findings in 110 patients (79.7%): in 43.5% a temporomandibular joint syndrome was diagnosed, in 29% parafunction of the occlusion, and in 35% a myopathy of the masticatory muscles. Additionally 32.6% patients showed dental disorders that required treatment; 11.65% had problems with dentures and 20.3% malpositioned wisdom teeth. In 16 patients the recommended dental treatment was followed up and improvement of otological symptoms was found in 56.6%. The present investigation shows that many patients with inner ear dysfunction suffer from dentognathological disorders. For a subgroup of patients there exists the possibility of improving otological symptoms by dental treatment. Therefore we recommend a dentognathological examination in patients with inner ear dysfunctions of unknown etiology. PMID:8449789

  7. Middle-ear function with tympanic-membrane perforations. II. A simple model

    E-print Network

    Allen, Jont

    Middle-ear function with tympanic-membrane perforations. II. A simple model Susan E. Vossa) Picker-Peabody Laboratory of Auditory Physiology and Department of Otolaryngology, Massachusetts Eye and Ear Infirmary and Department of Otolaryngology, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts 02114; Speech

  8. Building and Testing a Statistical Shape Model of the Human Ear Canal

    E-print Network

    Building and Testing a Statistical Shape Model of the Human Ear Canal Rasmus Paulsen1 , Rasmus Snekkersten, Denmark {cni, slu}@oticon.dk, http://www.oticon.com/ Abstract. Today the design of custom in-the-ear of the variation of the shape of the ear canal. In this paper it is described how a dense surface point

  9. Middle Ear Cavity Morphology Is Consistent with an Aquatic Origin for Testudines

    E-print Network

    Middle Ear Cavity Morphology Is Consistent with an Aquatic Origin for Testudines Katie L. Willis1 environments, we examined middle ear morphology and scaling in most extant families, as well as some extinct of the middle ear cavity, with the tympanic disk located on the rostrolateral edge of the cavity. Sea Turtles

  10. Identification with a recombinant antibody of an inner-ear cytokeratin, a marker for

    E-print Network

    Hudspeth, A. James

    Identification with a recombinant antibody of an inner-ear cytokeratin, a marker for hair by A. James Hudspeth, February 4, 2000 Extensive biochemical characterization of cells in the inner ear has been hampered by a lack of tools with which to identify inner-ear proteins. By using a single

  11. Interactive Direct Volume Rendering of the Inner Ear for the Planning of Neurosurgery

    E-print Network

    Blanz, Volker

    Interactive Direct Volume Rendering of the Inner Ear for the Planning of Neurosurgery P. Hastreiter and inner ear is presented. The segmen- tation of the spiral CT­data and the reconstruction was performed considerably diffi- cult with the small and complex structures of the middle and the inner ear which requires

  12. Connecting the ear to the brain: Molecular mechanisms of auditory circuit assembly

    E-print Network

    Goodrich, Lisa V.

    Connecting the ear to the brain: Molecular mechanisms of auditory circuit assembly Jessica M . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 493 3.2.1. Sox2 and the maintenance of precursors in the inner ear 2011 Keywords: Auditory system Inner ear Neural development Circuit assembly Spiral ganglion neuron A B

  13. Ontogenetic and Phylogenetic Transformations of the Ear Ossicles in Marsupial Mammals

    E-print Network

    Smith, Kathleen K.

    Ontogenetic and Phylogenetic Transformations of the Ear Ossicles in Marsupial Mammals Marcelo R. Sa a wide range of taxa and aims at addressing several issues con- cerning the evolution of the ear ossicles in marsupials. Three-dimensional reconstructions of the ear ossicles based on histological series were done

  14. In Vivo Analysis of Lrig Genes Reveals Redundant and Independent Functions in the Inner Ear

    E-print Network

    Goodrich, Lisa V.

    In Vivo Analysis of Lrig Genes Reveals Redundant and Independent Functions in the Inner Ear Tony compared the expression and function of the Lrigs in the inner ear, which offers a sensitive system in the inner ear throughout development, with Lrig1 and Lrig3 restricted to subsets of cells and Lrig2

  15. ARTICLE doi:10.1038/nature09921 Transitional mammalian middle ear from

    E-print Network

    Sullivan, Jack

    ARTICLE doi:10.1038/nature09921 Transitional mammalian middle ear from a new Cretaceous Jehol the dentary and the detached ossicles during mammalian evolution. This transitional mammalian middle ear narrows the morphological gap between the mandibular middle ear in basal mammaliaforms and the definitive

  16. Connecting the ear to the brain: Molecular mechanisms of auditory circuit assembly

    E-print Network

    Goodrich, Lisa V.

    Connecting the ear to the brain: Molecular mechanisms of auditory circuit assembly Jessica M . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 000 3.2.1. Sox2 and the maintenance of precursors in the inner ear 9 December 2010 Accepted 3 January 2011 Keywords: Auditory system Inner ear Neural development

  17. Measurements of Forward and Reverse Acoustics of the Human Middle Ear: Implications for Otoacoustic Emissions

    E-print Network

    Allen, Jont

    Measurements of Forward and Reverse Acoustics of the Human Middle Ear: Implications for Otoacoustic and California Ear Institute at Stanford, 801 Welch Road, Palo Alto, CA 94304 Email: puria@stanford.edu Running/Abbreviated Title: Forward and reverse middle-ear acoustics Corresponding Author: Sunil Puria, PhD Dept

  18. Session 1pPPa --1 --Evaluation of a physiological ear model for the simulation of

    E-print Network

    Session 1pPPa -- 1 -- Evaluation of a physiological ear model for the simulation of nonlinear] it was shown that the ear model is able to rebuild the level­dependency of spectral and temporal masking by the physiological ear model. Since the model represents a unified approach comprising the most important masking

  19. Multi-Modal Biometrics Involving the Human Ear Christopher Middendorff, Kevin W. Bowyer, Ping Yan

    E-print Network

    Zhu, Zhigang

    Multi-Modal Biometrics Involving the Human Ear Christopher Middendorff, Kevin W. Bowyer, Ping Yan and robustness against change over time, the ear has become an increasingly pop- ular biometric feature. It has of combi- nation and the recognition rates of each. 1. Introduction Ears have gained attention

  20. Analysis of Gentamicin Kinetics in Fluids of the Inner Ear with Round Window Administration

    E-print Network

    Salt, Alec N.

    Analysis of Gentamicin Kinetics in Fluids of the Inner Ear with Round Window Administration *Stefan for quantifying drug distri- bution in the inner ear with local applications can be established. Background: As methods of local drug delivery to the inner ear gain wider clinical acceptance it becomes important to es

  1. Does the infrasound from wind turbines affect the inner ear? Alec N. Salt1

    E-print Network

    Salt, Alec N.

    Does the infrasound from wind turbines affect the inner ear? Alec N. Salt1 1 Washington University that the response of the inner ear to infrasound is complex and needs to be understood in more detail before it can be concluded that the ear cannot be affected by wind turbine noise. This work was supported by research grant

  2. INTRODUCTION The mammalian inner ear consists of two sensory organs: the

    E-print Network

    Ryugo, David K.

    INTRODUCTION The mammalian inner ear consists of two sensory organs: the cochlea and vestibular of the inner ear is composed of saccule, utricle and semicircular canals, which together determine head-dating analyses have provided evidence that all inner ear hair cells are produced in a period spanning from

  3. Prediction of a Mysticete Audiogram via Finite Element Analysis of the Middle Ear

    E-print Network

    Prediction of a Mysticete Audiogram via Finite Element Analysis of the Middle Ear Andrew Tubelli published audiograms. Both the middle ear and the cochlea play an important role in shaping the audiogram of any mammal. The transfer function of the middle ear shapes the low-frequency portions of an audiogram

  4. Percutaneous inner-ear access via an image-guided industrial robot system

    E-print Network

    Webster III, Robert James

    Percutaneous inner-ear access via an image-guided industrial robot system S Baron1 , H Eilers1 , B-accuracy requirements and proximity to sensitive nerves and brain tissues, the adoption of robots in inner-ear surgery-guided industrial robot systems for accessing challenging inner-ear targets. Features of the systems include optical

  5. Measurements of human middle ear forward and reverse acoustics: Implications for otoacoustic emissions

    E-print Network

    Allen, Jont

    Measurements of human middle ear forward and reverse acoustics: Implications for otoacoustic and California Ear Institute at Stanford, 801 Welch Road, Palo Alto, California 94304 Received 11 July 2002; revised 1 February 2003; accepted 4 February 2003 Middle and inner ears from human cadaver temporal bones

  6. STRUCTURE AND FUNCTIONS OF THE EAR OF THE By G. H. Parker, Ph. D.

    E-print Network

    STRUCTURE AND FUNCTIONS OF THE EAR OF THE SQUETEAGUE By G. H. Parker, Ph. D. Professor of. S. A., September 22 to 26, 1908 I2II #12;Blank page retained for pagination #12;ANATOMY OF THE EAR. STRUCTURE AND FUNCTIONS OF THE EAR OF THE SQUETEAGUE. ..,c By G. H. PARKER, Ph. D., Professor of Zoology

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

    E-print Network

    Dasgupta, Dipankar

    Short Communication Functional ear (a)symmetry in brainstem neural activity relevant to encoding (FFR) Functional ear asymmetry Experience-dependent plasticity Subcortical a b s t r a c t Pitch areas. This experiment investigates whether ear asymmetries vary in brainstem representation of pitch

  8. Steps for Using the EAR Part 732-page 1 Export Administration Regulations

    E-print Network

    Bernstein, Daniel

    Steps for Using the EAR Part 732-page 1 Export Administration Regulations §732.1 STEPS OVERVIEW (a)(1) Introduction In this part, references to the EAR are references to 15 CFR chapter VII, subchapter C. This part is intended to help you determine your obligations under the EAR by listing logical

  9. Laminin and Fibronectin Modulate Inner Ear Spiral Ganglion Neurite Outgrowth in an In Vitro Alternate

    E-print Network

    Bhatia, Sangeeta

    Laminin and Fibronectin Modulate Inner Ear Spiral Ganglion Neurite Outgrowth in an In Vitro) and fibronectin (FN) presented in stripe micro-patterns can provide guidance cues to neonatal (P5) inner ear model for neurite guidance in the developing inner ear in vivo. LN, in the SG and osseus spiral lamina

  10. MicroRNAs and epigenetic regulation in the mammalian inner ear: implications for deafness

    E-print Network

    Avraham, Karen

    MicroRNAs and epigenetic regulation in the mammalian inner ear: implications for deafness Lilach M common sensory disorder in humans and derives, in most cases, from inner-ear defects or degeneration is expressed specifically in the inner-ear hair cells, were linked with progressive hearing loss in humans

  11. The two ear theorem on matching-covered graphs Zoltan Szigeti

    E-print Network

    Szigeti, Zoltán

    The two ear theorem on matching-covered graphs Zolt´an Szigeti Abstract We give a simple and short proof for the two ear theorem on matching- covered graphs which is a well-known result of Lov) has a perfect matching. A sequence of subgraphs of G, (G0, G1, ..., Gm) is a graded ear

  12. Effect of Negative Middle-Ear Pressure on Transient-Evoked Otoacoustic Emissions

    E-print Network

    Allen, Jont

    Effect of Negative Middle-Ear Pressure on Transient- Evoked Otoacoustic Emissions Lynne Marshall of negative middle-ear pressure (MEP) on both the stimulus and response of transient-evoked otoacoustic emissions (TEOAEs) and the effect of compensating for negative pressure in the middle ear by pneumatically

  13. Specification of absorbed-sound power in the ear canal: Application to suppression of stimulus frequency

    E-print Network

    Allen, Jont

    Specification of absorbed-sound power in the ear canal: Application to suppression of stimulus; accepted 12 November 2010) An insert ear-canal probe including sound source and microphone can deliver a calibrated sound power level to the ear. The aural power absorbed is proportional to the product of mean

  14. SHORT SCIENTIFIC COMMUNICATION Acoustic recordings in human ear canals to sounds

    E-print Network

    Oliver, Douglas L.

    SHORT SCIENTIFIC COMMUNICATION Acoustic recordings in human ear canals to sounds at different for different sound locations in an anechoic chamber. For humans, we embedded microphones in ear molds that were custom fitted to the subject's ear canal. For the ball, the microphones were flush with the surface at 90

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

    E-print Network

    Allen, Jont

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

  16. RNA Isolation from Xenopus Inner Ear Sensory Endorgans for Transcriptional Profiling and Molecular Cloning

    E-print Network

    Chapter 1 RNA Isolation from Xenopus Inner Ear Sensory Endorgans for Transcriptional Profiling systems mandate the ability to isolate intact RNA from inner ear tissue. Methods presented here facilitate preparation of high quality inner ear RNA from larval and post-metamorphic Xenopus spec- imens that can

  17. Mouse Models for Deafness: Lessons for the Human Inner Ear and Hearing Loss

    E-print Network

    Avraham, Karen

    Mouse Models for Deafness: Lessons for the Human Inner Ear and Hearing Loss Karen B. Avraham high-resolution com- puted tomography (CT) scanning or invasive sur- gery, most studies on the ear interventions can be developed that can treat the diseased inner ear before permanent damage has occurred

  18. Audiometric Predictions Using Stimulus-Frequency Otoacoustic Emissions and Middle Ear

    E-print Network

    Allen, Jont

    Audiometric Predictions Using Stimulus-Frequency Otoacoustic Emissions and Middle Ear Measurements or moderate-severe, and correlate with pure-tone thresholds in a population of adults with normal middle ear function. Other goals are to determine if middle ear function as assessed by wideband acoustic transfer

  19. Ear-Canal Reflectance, Umbo Velocity, and Tympanometry in Normal-Hearing Adults

    E-print Network

    Allen, Jont

    Ear-Canal Reflectance, Umbo Velocity, and Tympanometry in Normal-Hearing Adults John J. Rosowski,1. Halpin,4 and Saumil N. Merchant1,2,3 Objective: This study compares measurements of ear-canal reflectance (ECR) to other objective measurements of middle ear function including audiometry, umbo velocity (VU

  20. Biometric Recognition Using 3D Ear Shape Ping Yan and Kevin W. Bowyer, Fellow, IEEE

    E-print Network

    Bowyer, Kevin W.

    Biometric Recognition Using 3D Ear Shape Ping Yan and Kevin W. Bowyer, Fellow, IEEE Abstract--Previous works have shown that the ear is a promising candidate for biometric identification. However, in prior work, the preprocessing of ear images has had manual steps and algorithms have not necessarily handled