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Sample records for ear malformation cardiospondylocarpofacial

  1. Reconstruction of middle ear malformations

    PubMed Central

    Schwager, Konrad

    2008-01-01

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

  2. Classification and diagnosis of ear malformations

    PubMed Central

    Bartel-Friedrich, Sylva; Wulke, Cornelia

    2008-01-01

    In the ENT region 50% of the malformations affect the ear. Malformations of the outer and middle ear are predominantly unilateral (ca. 70-90%) and mostly involve the right ear. Inner ear malformations can be unilateral or bilateral. The incidence of ear malformations is approximately 1 in 3800 newborns. Ear malformations may be genetic (associated with syndromes or not, with family history, spontaneous mutations) or acquired in nature. Malformations can affect the outer ear (pinna and external auditory canal, EAC), middle ear and inner ear, not infrequently in combination. Formal classification is advisable in order to be able to predict the prognosis and compare treatment schedules. Various classifications have been proposed: pinna and EAC malformations according to Weerda [1], middle ear malformations according to Kösling [2], and inner ear malformations according to Jackler [3], [4], to Marangos [5] and to Sennaroglu [6]. Additionally, we describe Altmann’s classification of atresia auris congenita [7] and the Siegert-Mayer-Weerda score [8] for EAC and middle ear malformations, systems of great practicability that are in widespread clinical use. The diagnostic steps include clinical examination, audiological testing, genetic analysis and, especially, CT and MRI. These imaging methods are most usefully employed in combination. Precise description of the malformations by means of CT and MRI is indispensable for the planning and successful outcome of operative ear reconstruction and rehabilitation procedures, including cochlear implantation. PMID:22073081

  3. Revision Stapedectomy in a Female Patient with Inner Ear Malformation

    PubMed Central

    Patel, Tirth R.; Moberly, Aaron C.

    2016-01-01

    Objectives. We describe an unusual case of surgical management of congenital mixed hearing loss in a female patient with inner ear malformation. This report outlines the role of temporal bone imaging and previous surgical history in evaluating a patient's risk of perilymph gusher during stapes surgery. Methods. A 68-year-old female patient with a history of profound bilateral mixed hearing loss due to ossicular and cochlear malformation presented to our otology clinic. She had undergone multiple unsuccessful previous ear surgeries. Computed tomography revealed bilateral inner ear malformations. She elected to proceed with revision stapedectomy. Results. The patient received modest benefit to hearing, and no operative complications occurred. Conclusions. Although stapedectomy has been shown to improve hearing in patients with stapes fixation, there is risk of perilymph gusher in patients with inner ear abnormalities. Evaluation and counseling of the risk of gusher during stapes surgery should be done on a case-by-case basis. PMID:27144044

  4. Surgical and Technical Modalities for Hearing Restoration in Ear Malformations.

    PubMed

    Dazert, Stefan; Thomas, Jan Peter; Volkenstein, Stefan

    2015-12-01

    Malformations of the external and middle ear often go along with an aesthetic and functional handicap. Independent of additional aesthetic procedures, a successful functional hearing restoration leads to a tremendous gain in quality of life for affected patients. The introduction of implantable hearing systems (bone conduction and middle ear devices) offers new therapeutic options in this field. We focus on functional rehabilitation of patients with malformations, either by surgical reconstruction or the use of different implantable hearing devices, depending on the disease itself and the severity of malformation as well as hearing impairment. Patients with an open ear canal and minor malformations are good candidates for surgical hearing restoration of middle ear structures with passive titanium or autologous implants. In cases with complete fibrous or bony atresia of the ear canal, the most promising functional outcome and gain in quality of life can be expected with an active middle ear implant or a bone conduction device combined with a surgical aesthetic rehabilitation in a single or multi-step procedure. Although the surgical procedure for bone conduction devices is straightforward and safe, more sophisticated operations for active middle ear implants (e.g., Vibrant Soundbridge, MED-EL, Innsbruck, Austria) provide an improved speech discrimination in noise and the ability of sound localization compared with bone conduction devices where the stimulation reaches both cochleae. PMID:26667632

  5. Middle ear malformations in identical twins.

    PubMed

    Kidowaki, Naoko; Kamitani, Toru; Nakamura, Takashi; Taki, Masakatsu; Sakaguchi, Hirofumi; Suzuki, Toshihiro; Hisa, Yasuo

    2014-06-01

    The majority of the congenital anomalies of middle ear are solitary and a non-hereditary. We report cases of identical twins with congenital incudo-stapedial disconnection. Case 1 was an 8-year-old girl. Hearing impairment was identified at the age of three. She was referred to our university hospital in April 2005. Pure-tone audiogram showed conductive hearing impairments. Computed tomography (CT) revealed the incudo-stapedial disconnections in both ears. The exploratory tympanotomies on the right and left ears were performed in May and July 2005, respectively. The surgical findings showed absence of the long process and presence of the lenticular process of the incus in both ears. After the reconstructions of ossicular chain, the hearing of both ears improved. Case 2 was an 11-year-old girl. The hearing impairment of the right ear was identified in May 2008. She was referred to our university hospital three months later. Pure-tone audiogram showed the conductive hearing impairment in the right ear. CT revealed the incudo-stapedial disconnection in the right ear. The surgery showed the same findings as those of case 1. Anomalies of both cases suggest that the lenticular process of the incus and the stapes originate from a common primordium. PMID:24355584

  6. 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

  7. Cochlear implantation in patients with inner ear bone malformations with posterior labyrinth involvement: an exploratory study.

    PubMed

    Palomeque Vera, Juan Miguel; Platero Sánchez-Escribano, María; Gómez Hervás, Javier; Fernández Prada, María; González Ramírez, Amanda Rocío; Sainz Quevedo, Manuel

    2016-04-01

    Inner ear bone malformations are one cause of profound sensorineural hearing loss. This investigation focused on those affecting the posterior labyrinth, especially enlarged vestibular aqueduct syndrome, which is associated with fluctuating and progressive hearing loss. The objectives of this study were to analyze the behavior of the electrical stimulation, auditory functionality and linguistic development in patients with inner ear malformations involving the posterior labyrinth. The study included ten patients undergoing cochlear implantation (cases: five with enlarged vestibular aqueduct, two with vestibular aqueduct stenosis/aplasia, and three with semicircular canal disorders). Post-implantation, data were gathered on the electrical stimulation threshold and maximum comfort levels and on the number of functioning electrodes. Evaluation of Auditory Responses to Speech (EARS) subtests were used to assess auditory functionality and language acquisition at 6, 12, and 24 months post-implantation. Results were compared with findings in a control group of 28 cochlear implantation patients without these malformations. No significant differences were found between case and control groups in electrical stimulation parameters; auditory functionality subtest scores were lower in cases than controls, although the difference was only statistically significant for some subtests. In conclusion, cochlear implantation patients with posterior labyrinth bone malformations and profound hearing loss, including those with enlarged vestibular aqueduct syndrome, showed no significant difference in electrical stimulation threshold with controls. Although some auditory functionality test results were lower in cases than in controls, cochlear implantation appears to be beneficial for all patients with these malformations. PMID:25971996

  8. Congenital Malformations of the Inner Ear: Case Series and Review of the Literature.

    PubMed

    Piromchai, Patorn; Kasemsiri, Pornthep; Thanawirattananit, Panida; Yimtae, Kwanchanok

    2015-08-01

    Patients with craniofacial anomalies often present to doctors due to their noticeable disfigurement and are routinely assessed by otolaryngologists for hearing evaluation. However, small percentage of craniofacial anomaly patients may present with delayed speech though they may not have initial obvious external deformation. The objective of case series is to identify the congenital inner ear malformation. The series of clinical presentation, physical examination, investigations, treatments and follow-up results were demonstrated followed by the discussion. PMID:26742393

  9. Vibrant Soundbridge and Bone Conduction Hearing Aid in Patients with Bilateral Malformation of External Ear

    PubMed Central

    Mondelli, Maria Fernanda Capoani Garcia; Mariano, Thais Cristina Barbosa; Honório, Heitor Marques; Brito, Rubens Vuono de

    2015-01-01

    Introduction Hearing loss is the most common clinical finding in patients with malformation of the external ear canal. Among the possibilities of treatment, there is the adaptation of hearing aids by bone conduction and the adaptation of implantable hearing aids. Objective To assess speech perception with the use of Vibrant Soundbridge (VBS - MED-EL, Innsbruck, Austria) associated with additional amplification in patients with bilateral craniofacial malformation. Method We evaluated 11 patients with bilateral malformation over 12 years with mixed hearing loss or bilateral conductive. They were using the Softband (Oticon Medical, Sweden) and bone conduction hearing aid in the ear opposite the one with the VSB. We performed the evaluation of speech perception using the Hearing in Noise Test. Results Participants were eight men and three women with a mean of 19.5 years. The signal / noise ratio presented significant results in patients fitted with VSB and bone conduction hearing aid. Conclusion The results of speech perception were significantly better with use of VBS combined with bone conduction hearing aids. PMID:26722343

  10. Vibrant Soundbridge and Bone Conduction Hearing Aid in Patients with Bilateral Malformation of External Ear.

    PubMed

    Mondelli, Maria Fernanda Capoani Garcia; Mariano, Thais Cristina Barbosa; Honório, Heitor Marques; Brito, Rubens Vuono de

    2016-01-01

    Introduction Hearing loss is the most common clinical finding in patients with malformation of the external ear canal. Among the possibilities of treatment, there is the adaptation of hearing aids by bone conduction and the adaptation of implantable hearing aids. Objective To assess speech perception with the use of Vibrant Soundbridge (VBS - MED-EL, Innsbruck, Austria) associated with additional amplification in patients with bilateral craniofacial malformation. Method We evaluated 11 patients with bilateral malformation over 12 years with mixed hearing loss or bilateral conductive. They were using the Softband (Oticon Medical, Sweden) and bone conduction hearing aid in the ear opposite the one with the VSB. We performed the evaluation of speech perception using the Hearing in Noise Test. Results Participants were eight men and three women with a mean of 19.5 years. The signal / noise ratio presented significant results in patients fitted with VSB and bone conduction hearing aid. Conclusion The results of speech perception were significantly better with use of VBS combined with bone conduction hearing aids. PMID:26722343

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

    PubMed Central

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

    2014-01-01

    Fibroblast growth factor 23 (FGF23) is a circulating hormone important in phosphate homeostasis. Abnormal serum levels of FGF23 result in systemic pathologies in humans and mice, including renal phosphate wasting diseases and hyperphosphatemia. We sought to uncover the role FGF23 plays in the auditory system due to shared molecular mechanisms and genetic pathways between ear and kidney development, the critical roles multiple FGFs play in auditory development and the known hearing phenotype in mice deficient in klotho (KL), a critical co-factor for FGF23 signaling. Using functional assessments of hearing, we demonstrate that Fgf mice are profoundly deaf. Fgf mice have moderate hearing loss above 20 kHz, consistent with mixed conductive and sensorineural pathology of both middle and inner ear origin. Histology and high-voltage X-ray computed tomography of Fgf mice demonstrate dysplastic bulla and ossicles; Fgf mice have near-normal morphology. The cochleae of mutant mice appear nearly normal on gross and microscopic inspection. In wild type mice, FGF23 is ubiquitously expressed throughout the cochlea. Measurements from Fgf mice do not match the auditory phenotype of Kl−/− mice, suggesting that loss of FGF23 activity impacts the auditory system via mechanisms at least partially independent of KL. Given the extensive middle ear malformations and the overlap of initiation of FGF23 activity and Eustachian tube development, this work suggests a possible role for FGF23 in otitis media. PMID:25243481

  12. Histopathology of inner ear malformations: Do we have enough evidence to explain pathophysiology?

    PubMed

    Sennaroglu, Levent

    2016-01-01

    Objective To investigate the histopathology of inner ear malformations (IEMs) in order to explain their pathophysiology. Method Light microscopy was used to study 33 specimens exhibiting various IEMs in the collection of the Otopathology Laboratory at Harvard University's Massachusetts Eye and Ear Infirmary. Results The investigation found 18 incidences of cochlear hypoplasia (CH) (3 CH-I, 10 CH-II, 5 CH-III), 11 incomplete partitions (IPs) (5 IP-I, 6 IP-II), 2 vestibular dilatations, and 2 cases of cochlear nerve aplasia. The IP-I cases had characteristic defective endosteums, while the IP-II cases showed hydropic changes in the scala vestibuli. The CH cases were small in size externally, with normal or defective internal architecture. Conclusion In combination with embryological data, these findings suggest that cases of CH-III and CH-IV are most probably genetically predetermined to be small in size, and that development of the membranous labyrinth stops at a point earlier than normal, so that it is shorter. At the time of complete ossification, this results in a cochlea with small external dimensions and normal internal architecture. In CH-I and CH-II cases, there is arrested development of the internal architecture, in addition to a small cochlea; it is most likely that in these cases, there is a severely defective vascular supply from the internal auditory canal (IAC). IP-I may be the result of a defective vascular supply from the blood vessels of the IAC. In IP-II, an enlarged endolymphatic sac (EES) appears to be the genetic abnormality that causes the other abnormalities, as it allows high pressure to be transmitted into the cochlea and vestibule. In IP-III, the pathophysiology appears to be an abnormal vascular supply from the middle ear mucosa, caused by a genetic abnormality and resulting in a thinner otic capsule and the absence of the modiolus. PMID:26158591

  13. [One case of a pregnant femal taking realgar induceing congenital inner ear malformation].

    PubMed

    Diao, Mingfang; Sun, Jianjun; Liu, Yang

    2015-08-01

    A pregnant female taken realgar because of superstition, which caused the baby congenital deafness. Auditory test indicated that bilateral auditory brainstem response (ABR) hearing threshold level was greater than 90 dB nHL and auditory steady state response (ASSR) hearing level ranging from 0.5 kHz to 4 kHz was beyond 110 dB HL. Temporal bone CT showed that bilateral cochlear and semicircular canal malformations, with internal auditory canal broadened. PMID:26685413

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

    NASA Astrophysics Data System (ADS)

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

    2005-08-01

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

  15. Fusion of the ear bones

    MedlinePlus

    Fusion of the ear bones is the joining of the bones of the inner ear. These are the incus, malleus, and stapes bones. Related topics include: Chronic ear infection Otosclerosis Middle ear malformations

  16. [Results of the surgical management of congenital malformations of the external auditory canal and of the middle ear in children with special reference to vein xenografts].

    PubMed

    Makowski, A; Latkowski, B

    1999-01-01

    The paper deals with the possibility of surgical reconstruction of congenital malformations of the ear, the choice of a proper surgical method with the utilization of calf-vein xenografts, and with postoperative effects of the employed operative technique modified by the authors. The research material comprised 70 children between 3 and 17 years of age treated in years 1992-1995. Reconstructive surgery was performed in 24 children (29 ears). Calf-vein xenografts were used for myringoplasty and for the lining of the bony walls of the postoperative cavity (of the created external auditory canal and of the mastoid cavity). Hearing improvement was gained in 79% of cases, and in 55% of patients it was 24 dB or higher. Patent and epithelilized external auditory canals were obtained in 81% of the operated "atretic" ears. Failures referred to fibrodermal restenosis and lack of hearing improvement. Surgical treatment of bilateral congenital aural "atresia" can be performed in children at 4-6 years of age. Vein xenografts are a good reconstructive material recommended in congenital aural malformations for myringoplasty and canaloplasty. PMID:10581956

  17. 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

  18. Swimmer's Ear

    MedlinePlus

    ... Skiing, Snowboarding, Skating Crushes What's a Booger? Swimmer's Ear KidsHealth > For Kids > Swimmer's Ear Print A A ... How Do I Know if I Have Swimmer's Ear? Swimmer's ear may start with some itching, but ...

  19. Ear emergencies

    MedlinePlus

    Ear emergencies include objects in the ear canal and ruptured eardrums. ... Depending on the type of ear emergency, follow the steps below. OBJECT IN THE EAR Calm and reassure the person. If the object is sticking out and easy to ...

  20. Chiari Malformation

    MedlinePlus

    ... including those with Chiari I malformations. NIH Patient Recruitment for Chiari Malformation Clinical Trials At NIH Clinical Center Throughout the U.S. and Worldwide NINDS Clinical Trials Organizations Column1 Column2 March of Dimes 1275 Mamaroneck Avenue ...

  1. 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.

  2. Ear Disorders

    MedlinePlus

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

  3. Swimmer's ear

    MedlinePlus

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

  4. Cauliflower Ear

    MedlinePlus

    ... Snowboarding, Skating Crushes What's a Booger? What's Cauliflower Ear? KidsHealth > For Kids > What's Cauliflower Ear? Print A A A Text Size Have you ever seen someone whose ear looks bumpy and lumpy? The person might have ...

  5. Ear examination

    MedlinePlus

    An ear exam is performed when a health care provider looks inside your ear using an instrument called an otoscope. ... the head tilted toward the shoulder opposite the ear being examined. The provider will gently pull up, ...

  6. Ear Pieces

    ERIC Educational Resources Information Center

    DiJulio, Betsy

    2011-01-01

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

  7. Ear Pieces

    ERIC Educational Resources Information Center

    DiJulio, Betsy

    2011-01-01

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

  8. Arteriovenous Malformation

    MedlinePlus

    ... secondary menu Home Disorders A - Z Arteriovenous Malformation Information Page Publications Organizations News Research literature Organizations Professional Societies Government Resources NINDS Clinical Trials En Español Order Brochures ...

  9. Chiari Malformation

    MedlinePlus

    ... have cranial nerve compression. This can result in apnea (cessation of breathing), gagging, swallowing difficulties, facial numbness ... Chiari malformation with or without syringomyelia . A genetic prevalence has been identified in some families. Researchers continue ...

  10. Brain Malformations

    MedlinePlus

    Most brain malformations begin long before a baby is born. Something damages the developing nervous system or causes it ... medicines, infections, or radiation during pregnancy interferes with brain development. Parts of the brain may be missing, ...

  11. Ear wax

    MedlinePlus

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

  12. Ear Tubes

    MedlinePlus

    ... of recurrent ear infections and the need for repeat surgery in children four years and older. What happens ... soon (which is unpredictable), fluid may return and repeat surgery may be needed. Ear tubes that remain too ...

  13. Pierced Ears

    MedlinePlus

    ... clean your ears and put ear cleaning solution, rubbing alcohol, or antibiotic ointment on them. Whoever is doing ... Use a cotton ball or swab to apply rubbing alcohol or antibiotic ointment to the earlobe or lobes. ...

  14. Your Ears

    MedlinePlus

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

  15. Super Ears.

    ERIC Educational Resources Information Center

    Thompson, Stan

    1995-01-01

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

  16. 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 ...

  17. Ear barotrauma

    MedlinePlus

    Barotitis media; Barotrauma; Ear popping; Pressure-related ear pain; Eustachian tube dysfunction ... The air pressure in the middle ear is most often the same as the air pressure outside of the body. The Eustachian tube is a connection between the middle ...

  18. The ear: Diagnostic imaging

    SciTech Connect

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

    1986-01-01

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

  19. Chiari Malformation

    MedlinePlus

    ... of the back of the head or the neck and contains brain matter. The covering of the brain or spinal ... fusion of segments of the bones in the neck, and extra folds in the brain. How common are Chiari malformations? In the past, ...

  20. Dysmorphism of the middle ear: case report

    PubMed Central

    Solero, P; Ferrara, M; Musto, R; Pira, A; Di Lisi, D

    2005-01-01

    Summary Although there are numerous publications in the literature describing the wide range of diagnosis, classifications and treatment of malformations of the hearing apparatus, even more variations can be found in clinical practice. Indeed, each individual case is unique as far as concerns pathogenesis, clinical course and treatment. The case reported herein describes a 12-year-old boy affected by cranio-facial dysmorphism and monolateral conductive hearing loss in the right ear: followed from radiological diagnosis – carried out to study a malformation of the ear pinna – to surgical treatment. PMID:16602328

  1. Ear Infections

    MedlinePlus

    ... through the ear canal and hit the eardrum and cause it to vibrate. The vibration of the eardrum causes the tiny bones in ... psoriasis) that occur in other parts of the body can also occur in the ear canal and cause an infection. Bacteria from products you use ...

  2. Elephant ear

    MedlinePlus

    Elephant ear plants are indoor or outdoor plants with very large, arrow-shaped leaves. Poisoning may occur ... The harmful substances in elephant ear plants are: Oxalic acid Asparagine, a protein found in this plant Note: Leaves and stems are the most dangerous ...

  3. 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

  4. Cosmetic ear surgery

    MedlinePlus

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

  5. Outcome of cochlear implantation in children with cochlear malformations.

    PubMed

    Bille, Jesper; Fink-Jensen, Vibeke; Ovesen, Therese

    2015-03-01

    The objective of the study was the evaluation of outcomes of cochlear implantation (CI) in children with cochlear malformations. A retrospective case-control study was conducted in a tertiary referral centre. The patients were children with inner ear malformation judged by high-resolution computed tomography and magnetic resonance imaging treated with uni- or bilateral CI and a follow-up period of at least 3 years. They were matched with a control group of children operated for other reasons. The patients were operated by one of two surgeons using similar techniques including a standard perimodiolar electrode in all cases. The intervention was therapeutic and rehabilitative. The main outcome measures were category of auditory performance (CAP) and speech intelligibility rating (SIR). Eighteen children were diagnosed with cochlear malformations (12 % of children receiving CI). No statistical differences regarding CAP and SIR scores were found between the two groups. Only one child was diagnosed with a common cavity and performed below average. Children with auditory neuropathy performed beyond average. Children with cochlear malformations performed equally to children without malformation in the long term. Standard perimodiolar electrodes can be used despite cochlear malformations. The most important factors determining the outcome is the age of the child at the time of implantation and duration of hearing loss before CI. Awareness towards an increased risk of complications in case of inner ear malformations is recommended. PMID:24407715

  6. Ear defects.

    PubMed

    Shonka, David C; Park, Stephen S

    2009-08-01

    The projection and exposure of the auricle make it particularly susceptible to actinic injury and thus to cutaneous malignancies. Auricular reconstruction is challenging because of its unique surface anatomy and undulating topography. This article organizes auricular defects into different categories based on anatomic location and extent of tissue loss, including skin-only defects, small composite defects, full-thickness defects involving or sparing the upper third of the ear, and total auricular loss. The authors share an algorithm for repair of the array of auricular defects. PMID:19698921

  7. Ear Plastic Surgery

    MedlinePlus

    ... Find an ENT Doctor Near You Ear Plastic Surgery Ear Plastic Surgery Patient Health Information News media ... weight earrings. Does Insurance Pay for Cosmetic Ear Surgery? Insurance usually does not cover surgery solely for ...

  8. Ear tube insertion

    MedlinePlus

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

  9. Swimmer's Ear (External Otitis)

    MedlinePlus

    ... Can I Help a Friend Who Cuts? Swimmer's Ear (External Otitis) KidsHealth > For Teens > Swimmer's Ear (External Otitis) Print A A A Text Size ... Ryan awoke with a sharp pain in his ear — it really hurt! He remembered having ear infections ...

  10. Swimmer's Ear (For Parents)

    MedlinePlus

    ... for Your Child All About Food Allergies Swimmer's Ear (Otitis Externa) KidsHealth > For Parents > Swimmer's Ear (Otitis ... español Otitis del nadador (otitis externa) About Swimmer's Ear Otitis externa (OE) — commonly known as swimmer's ear — ...

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

    PubMed Central

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

    2014-01-01

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

  12. 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 ...

  13. Ear Infection and Vaccines

    MedlinePlus

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

  14. Ear infection - chronic

    MedlinePlus

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

  15. Travel Inside the Ear

    MedlinePlus Videos and Cool Tools

    ... Deafness Travel Inside the Ear Video When sound waves reach your ear, you know you've heard ... the sound through the hair cells to your brain. Last Updated Date: July 30, 2014 Languages Español ...

  16. Middle ear infection (image)

    MedlinePlus

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

  17. Flying and Your Child's Ears

    MedlinePlus

    ... All About Food Allergies Flying and Your Child's Ears KidsHealth > For Parents > Flying and Your Child's Ears ... a) cuando vuele en avión Flying's Effects on Ears Many of us have felt that weird ear- ...

  18. [The solitary ear].

    PubMed

    Garzón Calles, J A

    1996-01-01

    An unique ear refers to those cases of individuals with an air and/or bone conduction allowing a serviceable hearing in one ear, being the other one totally lost, owing to any disease. So it is relatively frequent patients presenting only one ear normal, being the other one deaf. The puzzle rises when the sole running good ear must be operated, for whichever pathology. It may be that these various circumstances were: double cholesteatoma, cholesteatoma with extreme loss of hearing, otosclerosis with total deafness and even patients with chronic middle ear otitis and progressive impairment of bilateral hearing. Before to take the final decision on behalf of surgery we must consider: the ear condition, the age and physical state, the job and the social activity and finally the desire and resolution of the patient himself as well. The paper explain our experience in dealing with 10 solitary ears seen in the last 6 years. PMID:8967558

  19. [Current strategy for the radiological exploration of the ear].

    PubMed

    Bourjat, P; Veillon, F

    1986-01-01

    CAT scan by thin slices offers the possibility of evaluation of the bone structures of the ear and their contents. The progressively increasing availability of the scanner should make access much more routine for study of the ear, with a concomitant reduction in tomographies, inadequate in a large number of circumstances. The 4 main indications are chronic otitis with cholesteatoma, trauma, acoustic neuromas and malformations. The respective performances of tomography and CAT scan in each of these situations are discussed and an investigation strategy is suggested. PMID:3010793

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

    MedlinePlus

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

  1. Pathology of the Ear

    PubMed Central

    Orengo, Ida; Robbins, Kerri; Marsch, Amanda

    2011-01-01

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

  2. [Radiosurgery for brain arteriovenous malformations].

    PubMed

    Latorzeff, I; Schlienger, M; Sabatier, J; Borius, P-Y; Bourdin, S; Menegalli, D; Cognard, C; Januel, A-C; Lotterie, J-A; Desal, H; Debono, B; Blond, S

    2012-06-01

    Radiosurgery as treatment for arteriovenous malformations has shown a good efficacy in reducing intracranial bleeding due to rupture. The choice of therapeutic modalities is based on evolutive risk and arteriovenous malformations volume, patient profile and risks stratification following therapeutic techniques (microsurgery, radiosurgery, embolization). Nidus size, arteriovenous malformations anatomical localization, prior embolization or bleeding, distributed dose are predictive factors for radiosurgery's good results and tolerance. This review article will highlight arteriovenous malformations radiosurgery indications and discuss recent irradiation alternatives for large arteriovenous malformation volumes. PMID:22721755

  3. 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 ...

  4. 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 ...

  5. Cerebral arteriovenous malformation

    MedlinePlus

    ... are: Confusion Ear noise/buzzing (also called pulsatile tinnitus) Headache in one or more parts of the ... Finding the best treatment for an AVM that is found on an imaging test but is not causing any symptoms can be difficult. ...

  6. Heterochrony and human malformation.

    PubMed

    Wilson, G N

    1988-02-01

    The role of altered developmental timing or heterochrony in morphologic evolution has intrigued classical and modern biologists. Analogous manifestations of developmental asynchrony occur in human dysmorphogenesis where they illustrate the residue and repertoire of phylogenetic change. Certain single malformations such as holoprosencephaly immediately suggest heterochrony by their resemblance to antecedent phylogenetic or embryologic structures. Multiple malformation syndromes of genetic, chromosomal, or teratogenic etiology may have altered developmental timing as an underlying theme. The persisting alpha-fetoprotein synthesis in ataxia-telangiectasia, the morphologic atavisms in Down or trisomy 13 syndromes, and the delayed growth or fetal to adult hemoglobin switch in diabetic embryopathy all exemplify developmental asynchrony. The perspective of heterochrony stresses the molecular history and hierarchy which is recapitulated with each pregnancy, and reconciles apparent discrepancies between the rates of molecular and morphologic evolution. Recognition of heterochrony places isolated anomalies in the context of pattern and suggests monitoring of teratogenesis through altered expression of ontogenetically regulated, phylogenetically relevant molecules. PMID:3281459

  7. Ear lobe reconstruction with a posterior ear flap. Case reports.

    PubMed

    Savaci, N; Tosun, Z; Hoşnuter, M

    2000-06-01

    We describe a technique for reconstruction of the ear lobe. Although there are many procedures that aim to reconstruct the ear lobe naturally, the aesthetic results of using a posterior ear flap are better because the scar tissue that remains behind the ear is invisible. PMID:10900635

  8. External Otitis (Swimmer's Ear)

    MedlinePlus

    ... Injuring the ear canal while cleaning it (using cotton-tipped swabs) or getting water or irritants, such ... these devices are not properly cleaned. Use of cotton-tipped swabs is a very common risk factor ...

  9. Ear Infections in Children

    MedlinePlus

    ... have identified colonies of antibiotic-resistant bacteria, called biofilms, that are present in the middle ears of ... infections. Understanding how to attack and kill these biofilms would be one way to successfully treat chronic ...

  10. External Otitis (Swimmer's Ear)

    MedlinePlus

    ... after swimming (as long as there is no hole [perforation] in the eardrum). Attempting to clean the ... is the most common cause of perforation (a hole) of the eardrum? Objects entering the ear Middle ...

  11. Ear infection - acute

    MedlinePlus

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

  12. Infrared ear thermometry.

    PubMed

    1991-11-01

    Many hospitals are considering using infrared (IR) ear thermometers (also called tympanic membrane thermometers) as an alternative to traditional temperature-measurement devices. IR technology allows users to quickly and noninvasively measure body temperature by inserting a directional probe into the ear canal. For infection control, ear thermometers offer the advantages of not contacting mucous membranes and eliminating the need for special handling procedures. For use on unconscious patients or those who are otherwise unwilling or unable to cooperate with traditional techniques, IR ear thermometers offer a more comfortable and less stressful method of temperature taking for both patients and nurses, especially where rectal temperatures are used. The recent entry into this market by major companies and distributors and the release of several new IR ear thermometers have helped to further stimulate interest in this technology. However, a complete transition to IR ear thermometers throughout the hospital may not be necessary and should be made only after these devices have been successfully used in selected clinical locations, such as emergency departments, critical care areas, recovery areas, and labor and delivery rooms. Clinical users must be familiar with the technique needed for temperature measurement with these instruments and understand the differences between temperatures taken in the ear canal and those obtained from traditional sites. In the following article, we first present and overview of clinical thermometry, describing traditional temperature-measurement sites and devices. We then discuss the advantages and disadvantages of using the ear canal, including the tympanic membrane (TM), as a site for temperature measurement.(ABSTRACT TRUNCATED AT 250 WORDS) PMID:1743976

  13. Treatment of congenital malformations.

    PubMed

    Brucker, Sara Yvonne; Rall, Katharina; Campo, Rudi; Oppelt, Peter; Isaacson, Keith

    2011-03-01

    The prevalence of müllerian malformations is 1 in 200, or 0.5%. A third of the anomalies are septate, a third bicornuate uteri, 10% arcuate uterus, 10% didelphis and unicornuate uterus, and < 5% uterine and vaginal aplasia. Correct diagnosis of the malformation is most important but often very difficult. Correct treatment can only be performed if the malformation is clear. Longitudinal vaginal septums have to be removed due to potential obstetric problems. Transverse vaginal septums can cause hematocolpos and pain and have to be incised crosswise and excised so as not to shorten the vagina at the same time. Congenital vaginal agenesis occurs in Mayer-Rokitansky-Kuster-Hauser syndrome patients and in androgen insensitivity syndrome. The first choice for surgical treatment should be the new laparoscopic-assisted creation of a neovagina. Septate uterus has to be distinguished from a bicornuate uterus. Even if it is not proven to be a cause for infertility, the chance of miscarriage can be diminished by performing hysteroscopic metroplasty. Repair of a uterine septum in infertility patients often improves pregnancy rates. In contrast, surgical repair of a bicornuate uterus requires an abdominal metroplasty. This should only be performed if the patient has recurrent fetal loss due to the uterine structural defect. In a unicornuate uterus it is most important to determine if there is a second uterine horn that can cause cyclic pain if it has functioning endometrium. The only surgical option in these cases is to remove the rudimentary uterus with endometrium and hematometra, respectively. PMID:21437824

  14. Arteriovenous Malformation Management

    SciTech Connect

    Yakes, Wayne F.; Rossi, Plinio; Odink, Henk

    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.

  15. Chiari-like Malformation.

    PubMed

    Loughin, Catherine A

    2016-03-01

    Chiari-like malformation is a condition of the craniocervical junction in which there is a mismatch of the structures of the caudal cranial fossa causing the cerebellum to herniate into the foramen magnum. This herniation can lead to fluid buildup in the spinal cord, also known as syringomyelia. Pain is the most common clinical sign followed by scratching. Other neurologic signs noted are facial nerve deficits, seizures, vestibular syndrome, ataxia, menace deficit, proprioceptive deficits, head tremor, temporal muscle atrophy, and multifocal central nervous system signs. MRI is the diagnostic of choice, but computed tomography can also be used. PMID:26631589

  16. Radiosurgery for arteriovenous malformations.

    PubMed

    Friedman, William A; Bova, Frank J

    2011-10-01

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

  17. 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

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

    PubMed Central

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

    2014-01-01

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

  19. What Is an Ear Infection?

    MedlinePlus

    ... Words En Español What Other Kids Are Reading Movie: Digestive System Winter Sports: Sledding, Skiing, Snowboarding, Skating ... For Kids For Parents MORE ON THIS TOPIC Movie: Ears Your Ears What's Hearing Loss? Taking Care ...

  20. Genetics Home Reference: capillary malformation-arteriovenous malformation syndrome

    MedlinePlus

    ... MALFORMATION Sources for This Page Boon LM, Mulliken JB, Vikkula M. RASA1: variable phenotype with capillary and ... 265-9. Review. Eerola I, Boon LM, Mulliken JB, Burrows PE, Dompmartin A, Watanabe S, Vanwijck R, Vikkula ...

  1. Reconstructive Middle Ear Surgery

    PubMed Central

    Ruby, R.R.F.; Ballagh, R.H.

    1992-01-01

    Conductive hearing loss is a common cause of deafness and disability, particularly in children and young adults. This article presents a brief overview of the various methods currently available for reconstructing the tympanic membrane and middle ear ossicular chain, including some comments as to their indications and limitations. Schematic diagrams showing these techniques illustrate the various types of repair described. PMID:21221356

  2. Travel Inside the Ear

    MedlinePlus Videos and Cool Tools

    ... form Search A–Z Index Español Menu Home Health Info Hearing, Ear Infections, and Deafness Balance Taste ... Voice, Speech, and Language Información en español Statistics Health Resources Clinical Studies Research Extramural Research Intramural Research ...

  3. From Ear to Brain

    ERIC Educational Resources Information Center

    Kimura, Doreen

    2011-01-01

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

  4. Combined and complex vascular malformations.

    PubMed

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

    2015-03-01

    The correct diagnosis of vascular malformations is obtainable by clinical assessment and patient history in the majority of cases. Nonetheless, confusion in nomenclature, existence of multiple classifications and rarity of these lesions leads to misdiagnosis and related wrong treatment. This is especially the case in combined or complex vascular malformations or vascular malformations that are part of syndromes as these have overlapping clinical and imaging features. New entities in the field of vascular anomalies have been described recently like fibro-adipose vascular anomaly or central conducting lymphatic anomalies. PMID:25698387

  5. 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…

  6. [Abdominal vascular malformations and Down syndrome].

    PubMed

    Nso Roca, A P; García Sánchez, P; Quero Jiménez, J

    2007-04-01

    Malformations of the abdominal venous system are rare vascular disorders. These entities are associated with other malformations and with chromosomal anomalies such as trisomy 21. Abdominal venous malformations are probably the most frequent congenital vascular malformations in Down syndrome. Prenatal diagnosis allows the early follow-up and treatment of complications. We present a case of Down syndrome associated with an abdominal venous malformation diagnosed at the first trimester of pregnancy. PMID:17430719

  7. Neurologic evaluation of the ear.

    PubMed

    Cook, Laurie B

    2004-03-01

    Diseases of the ear often cause signs of neurologic dysfunction because of damage of peripheral nervous system structures associated with the middle and inner ear. Vestibular dysfunction, facial paralysis, Horner's syndrome, and hearing deficits are the most common neurologic deficits that accompany middle and inner ear disease. Differentiating these signs from disease of the central nervous system is crucial for an accurate diagnosis and prognosis but can be difficult. Understanding the normal anatomy of the ear and its association with the brain is crucial to interpretation of the neurologic examination. This article reviews neurologic dysfunction commonly associated with diseases of the ear and differentiating these signs from central disease. PMID:15062617

  8. Pancreatic Arteriovenous Malformation

    PubMed Central

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

    2014-01-01

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

  9. 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

  10. 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

  11. Evolutionary origin of cardiac malformations.

    PubMed

    Taussig, H B

    1988-10-01

    The author has proposed in previous publications that isolated cardiac malformations have an evolutionary origin. This is partly supported by the fact that isolated cardiac malformations found in humans occur also in other placental mammals as well as in birds. External gross examination of the heart in just over 5,000 birds was carried out during a 3 year period. Anomalies included one instance of duplicate hearts, two specimens in which no heart could be identified and in a fourth, a yellow-rumped warbler, the heart lay in the neck outside of the thoracic cavity. Published reports of similar occurrences of an ectopically placed heart concern birds, cattle and humans. The fact that various species of both placental mammals and birds show evidence of heritability for heart defects, and that these species cannot interbreed, combined with the fact that birds and mammals have many similar malformations, points to either a common external causative factor or a common origin. Genes that code the malformed heart must be transmitted with that part of the genetic makeup common to all birds and mammals. Malformations caused by teratogens produce widespread organ injury to a potentially normal embryo whereas the evolutionary malformation is an organ-specific anomaly in an otherwise normal mammal or bird and occurs in widely separated species. The implications of this theory are important for parents of children with an isolated congenital heart defect who may have ingested one or another drug or chemical or have been exposed to toxins or infectious agents before or after conception of the affected offspring. PMID:3047192

  12. Arteriovenous malformation of nose-revision surgery.

    PubMed

    Srinivas, C V; Kailash, N; Kailas, Gayattre; Divya Jyothi, N

    2012-12-01

    Areteriovenous malformations are rare in the head and neck region and generally arise from intracranial vessels. We present one rare case with spontaneous arteriovenous malformations related to the nose. PMID:24294582

  13. Red Ear Syndrome.

    PubMed

    Raieli, V; Compagno, A; D'Amelio, M

    2016-03-01

    The Red ear syndrome (RES) is an intriguing syndrome originally described for the first time nearly 20 years ago. RES is characterized by unilateral/bilateral episodes of pain and burning sensation of the ear, associated with ipsilateral erythema. RES episodes are indeed isolated in some patients, but they can occur in association with primary headaches, including in particular migraine in the developmental age. Although the underlying pathophysiological mechanisms are still uncertain, in the recent years the described comorbidities have aroused increasing interest because of possible clinical implications. Moreover, RES seems to be more often associated with clinical features of migraine partially provoked by the involvement of the parasympathetic system. This clinical association has shed new light on the pathophysiology of RES, supporting the hypothesis of a shared pathophysiological background, for example, through the activation of the trigeminal autonomic reflex. Current therapies of RES will be also discussed. Finally, we will resume the more controversial aspects of this relatively new and probably underestimated neurological syndrome. PMID:26879877

  14. [MIDDLE EAR INFECTION].

    PubMed

    Mari?i?, Martina; Bonjak, Zrinka; Branica, Sre?ko

    2015-01-01

    Middle ear infection is one of the most common childhood infections and the leading reason for antibiotic prescriptions. Although the etiological diagnosis is rarely discovered, successful identification of pathogens depends on properly collected sample, chosen method and microbiological analysis made on time. The most common bacterial pathogen is Streptococcus pneumoniae. Others include Haemophilus influenzae, Moraxella catarrhalis and Pseudomonas aeruginosa, known as the most common bacterial pathogen of chronic inflamations. Viral or polimicrobial upper respiratory tract infections often precede this infection. Pneumococcal conjugate vaccines given during infancy decrease rates of acute middle ear inflammation. It is a self-limited disease with rare complications. The best treatment is watchful waiting for two days followed by amoxicillin during 7 days, only if it is necessary. If there is resistance, then combination of amoxicillin and beta lactamase inhibitor is second line. The best choice for patients allergic to penicillin are macrolides. Antibiotic treatment has contributed to frequent relapses and increase of multi-drug resistant pathogens by permitting their colonization, which eliminates protective nasopharyngeal flora. PMID:26749955

  15. Genetic causes of vascular malformations.

    PubMed

    Brouillard, Pascal; Vikkula, Miikka

    2007-10-15

    Vascular malformations are localized defects of vascular development. They usually affect a limited number of vessels in a restricted area of the body. Although most malformations are sporadic, inheritance is observed, enabling genetic analysis. Usually, sporadic forms present with a single lesion whereas multiple lesions are observed in familial cases. The last decade has seen unraveling of several causative genes and beginning of elucidation of the pathophysiological pathways involved in the inherited forms. In parallel, definition of the clinical phenotypes has improved and disorders such as Parkes-Weber syndrome (PKWS), first thought to be sporadic, is now known to be part of a more common inheritable phenotype. In addition, the concept of double-hit mechanism that we proposed earlier to explain the incomplete penetrance, variable expressivity and multifocality of lesions in inherited venous anomalies is now becoming confirmed, as some somatic mutations have been identified in venous, glomuvenous and cerebral cavernous malformations. It is thus tempting to suggest that familial forms of vascular malformations follow paradominant inheritance and that sporadic forms, the etiopathogenic causes of which are still unelucidated, are caused by somatic mutations in the same genes. PMID:17670762

  16. Congenital Pulmonary Malformation in Children

    PubMed Central

    Nadeem, Montasser; Elnazir, Basil; Greally, Peter

    2012-01-01

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

  17. Management of perinatal lung malformations.

    PubMed

    Macardle, C A; Kunisaki, S M

    2015-02-01

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

  18. Wax blockage in the ear (image)

    MedlinePlus

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

  19. Otoscopic exam of the ear (image)

    MedlinePlus

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

  20. Successful Bilateral Composite Ear Reattachment

    PubMed Central

    2014-01-01

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

  1. Reconstruction of partially amputated external ear with costal cartilage graft: case report.

    PubMed

    Brunelli, A; Bottini, D J; Cervelli, V; Cervelli, G; Grimaldi, M

    2004-06-01

    Many causes are responsible for secondary anomalies of the outer ear, such as: car accidents, sport- or work-related accidents, assaults, bites from animals or humans, benign or malignant tumours, burns and the effects of surgical interventions of the ear (plastic surgery on the ear or attempts at correction of primary malformations of the ear). The anatomical complexity of the ear makes its reconstruction particularly complicated with post-operative results that are often disappointing. The Authors describe their experience in the reconstruction of a partially amputated outer ear following a dog bite. The therapeutic protocol required various surgical stages. Initially, a cutaneous expander was applied at the level of the mastoid in order to ensure a sufficient quantity of local skin. The second stage was to remove cartilage from the ribs, followed by construction of a cartilaginous model of the ear and its insertion into the subcutaneous mastoid region after removal of the cutaneous expander and any residual ear cartilage. The last stage was to separate the neo-formed outer ear from the mastoid skin with the insertion of a cartilage graft to the posterior region of the reconstructed ear. This graft was covered by the occipital fascia rotated at 180 degrees and by a skin graft removed from the pubis. The postoperative result was satisfactory with recuperation of a good aesthetic appearance of the ear. Aim of the present report is to describe the surgical technique employed in the reconstruction of secondary anomalies of the ear and to highlight errors committed during this procedure. These considerations have allowed us to stress some fundamental elements in the reconstruction of the ear. In particular, the watershed was the awareness that we had to create a cartilaginous model that respected, as far as possible, the anatomy of the outer ear with all its ridges, trenches and cavities. This as well as ensuring a sufficient quantity of local skin in order to cover the cartilaginous graft and, therefore, reduce the risk of exposing the cartilage and subsequent infection, to guarantee an optimal end result. PMID:15584586

  2. Galenic arteriovenous malformation with precocious puberty.

    PubMed

    Ventureyra, E C; Badejo, A

    1984-01-01

    Pineal lesions may appear with precocious puberty. In this report, a patient with precocious puberty and macrogenitosomia caused by an arteriovenous malformation in the pineal region is presented. This vascular malformation was not visualized during investigations 3 years before the present series. It appears that the vascular malformation increased considerably in size within a 3-year period. This case suggests that some arteriovenous malformations may take a malignant course, increasing rapidly in size and behaving like tumors by causing destruction and compression of surrounding structures. This case seems to be unique because, to the best of our knowledge, an arteriovenous malformation associated with precocious puberty has never been described previously. PMID:6689808

  3. Arteriovenous Malformation of the Oral Cavity

    PubMed Central

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

    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

  4. AUTISM WITH OPHTHALMOLOGIC MALFORMATIONS: THE PLOT THICKENS

    PubMed Central

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

    2004-01-01

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

  5. Otoplasty for the Protruded Ear

    PubMed Central

    Nazarian, Ronen; Eshraghi, Adrien A.

    2011-01-01

    The prominent ear can produce significant social and psychological effects on an individual. Through the last century, many procedures have been described to correct this deformity. In this review, the authors navigate through the history of otoplasty for the protruded ear, and describe some of the breakthroughs in the procedure. Furthermore, they discuss key measurements that must be kept in mind in preoperative and intraoperative settings. They also describe in more detail some of the more common methods for correcting the protruded ear, as well as postoperative management and common complications faced after surgery. PMID:23115535

  6. 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

  7. Prenatal diagnosis of cloacal malformation.

    PubMed

    Peiro, Jose L; Scorletti, Federico; Sbragia, Lourenco

    2016-04-01

    Persistent cloaca malformation is the most severe type of anorectal and urogenital malformation. Decisions concerning the surgical treatment for this condition are taken during the first hours of life and may determine the quality of life of these patients. Thus, prenatal diagnosis becomes important for a prompt and efficient management of the fetus and newborn, and accurate counseling of the parents regarding its consequences and the future of the baby. Careful evaluation by ultrasonography, and further in-depth analysis with MRI, allow prenatal detection of characteristic findings, which can lead to diagnose or at least suspect this condition. We reviewed our experience and the literature in order to highlight the most important clues that can guide the physician in the differential diagnosis. PMID:26969229

  8. Cochlear Implant Outcomes and Genetic Mutations in Children with Ear and Brain Anomalies

    PubMed Central

    Busi, Micol; Rosignoli, Monica; Castiglione, Alessandro; Minazzi, Federica; Trevisi, Patrizia; Aimoni, Claudia; Calzolari, Ferdinando; Granieri, Enrico; Martini, Alessandro

    2015-01-01

    Background. Specific clinical conditions could compromise cochlear implantation outcomes and drastically reduce the chance of an acceptable development of perceptual and linguistic capabilities. These conditions should certainly include the presence of inner ear malformations or brain abnormalities. The aims of this work were to study the diagnostic value of high resolution computed tomography (HRCT) and magnetic resonance imaging (MRI) in children with sensorineural hearing loss who were candidates for cochlear implants and to analyse the anatomic abnormalities of the ear and brain in patients who underwent cochlear implantation. We also analysed the effects of ear malformations and brain anomalies on the CI outcomes, speculating on their potential role in the management of language developmental disorders. Methods. The present study is a retrospective observational review of cochlear implant outcomes among hearing-impaired children who presented ear and/or brain anomalies at neuroimaging investigations with MRI and HRCT. Furthermore, genetic results from molecular genetic investigations (GJB2/GJB6 and, additionally, in selected cases, SLC26A4 or mitochondrial-DNA mutations) on this study group were herein described. Longitudinal and cross-sectional analysis was conducted using statistical tests. Results. Between January 1, 1996 and April 1, 2012, at the ENT-Audiology Department of the University Hospital of Ferrara, 620 cochlear implantations were performed. There were 426 implanted children at the time of the present study (who were <18 years). Among these, 143 patients (64 females and 79 males) presented ear and/or brain anomalies/lesions/malformations at neuroimaging investigations with MRI and HRCT. The age of the main study group (143 implanted children) ranged from 9 months and 16 years (average = 4.4; median = 3.0). Conclusions. Good outcomes with cochlear implants are possible in patients who present with inner ear or brain abnormalities, even if central nervous system anomalies represent a negative prognostic factor that is made worse by the concomitant presence of cochlear malformations. Common cavity and stenosis of the internal auditory canal (less than 2 mm) are negative prognostic factors even if brain lesions are absent. PMID:26236732

  9. [Inner ear cochlear processes].

    PubMed

    Livshits, M S

    1995-01-01

    The processes accompanying the propagation across the basilar membrane (BM) of waves from a wide-band sound signal have been analyzed by physical methods. Particular emphasis in placed on the fact that BM is a low frequency filter. The characteristics of wave envelope from each of the sinusoidal components of the signal are given. The use of a simple mechanical model makes it possible to explain an initial increase in these envelopes. The steep slope of the envelope in its terminal part is explained in terms of the lighthill hydrodynamic model of cochlea. This provides a theoretical basis for adequate description of the periphery of the ear analyzer, with allowance for the active role of external auditory hair cells (EHC). Further research is needed to solve the problem completely. This is dictated by the complexity of the oscillatory system (BM in the region of the critical layer of cochlea plus EHC) and by its interaction with the autoregulation system in the air analyzer, which is described elsewhere. PMID:7662742

  10. Ototoxicity (Ear Poisoning) (For Parents)

    MedlinePlus

    ... Kids Up for Sports Pregnant? Your Baby's Growth Cerebral Palsy: Caring for Your Child All About Food Allergies ... Speech or Language Development Speech-Language Therapy Physical ... Loss? Can Loud Music Hurt My Ears? Going to the Audiologist Hearing ...

  11. Diseases of the ear pinna.

    PubMed

    Matousek, Jennifer L

    2004-03-01

    In conclusion, there are a wide variety of dermatologic diseases that can affect the ear pinnae. Most of these disorders are manifestations of a generalized dermatosis. Therefore, when pinnal lesions are present, a thorough diagnostic workup is indicated. PMID:15062622

  12. Autoimmune Inner Ear Disease (AIED)

    MedlinePlus

    ... Canal Dehiscence (SCD) Tinnitus Vestibular Hyperacusis Vision & Hearing Psychology Diet Other Topics Military Resources Infographics & Presentations Paid ... in women than men and less frequently in children and the elderly. When the ear is attacked, ...

  13. Taking Care of Your Ears

    MedlinePlus

    ... your hands before touching your pierced ears. Applying rubbing alcohol also can help keep germs away. With an adult's help, soak a cotton ball in rubbing alcohol and apply it to both sides of the ...

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

    MedlinePlus

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

  15. 21 CFR 870.2710 - Ear oximeter.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

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

  16. 21 CFR 870.2710 - Ear oximeter.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

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

  17. 21 CFR 870.2710 - Ear oximeter.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

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

  18. The visible ear surgery simulator.

    PubMed

    Trier, Peter; Noe, Karsten Østergaard; Sørensen, Mads Sølvsten; Mosegaard, Jesper

    2008-01-01

    This paper presents a real-time computer simulation of surgical procedures in the ear, in which a surgeon drills into the temporal bone to gain access to the middle or inner ear. The purpose of this simulator is to support development of anatomical insight and training of drilling skills for both medical students and experienced otologists. The key contributions in this application are the visualization and interaction models in the context of ear surgical simulation. The visualization is based on an existing data set, "The Visible Ear", containing a unique volume depicting the inner ear in natural colours. The applied visualization is based on GPU ray casting, allowing high quality and flexible volume rendering using modern graphics card. In connection with the visualization model, different methods for optimizing the GPU ray casting procedure are presented, along with a method for combining polygon based graphics with volume rendering. In addition, different light models are presented that contribute to a realistic rendering of the different parts of the inner ear. To achieve a physically plausible drilling experience, a Phantom Omni force feedback device is utilized. The applied interaction model facilitates a realistic user experience of the response forces from the drilling tool. PMID:18391361

  19. 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.

  20. Acquired Chiari malformation type I associated with a supratentorial fistulous arteriovenous malformation: a case report.

    PubMed

    Chen, Kuo-Wei; Kuo, Meng-Fai; Lee, Chung-Wei; Tu, Yong-Kwang

    2015-03-01

    A case of acquired Chiari malformation type I with frontal fistulous arteriovenous malformation (AVM) is presented, and the pathophysiology is discussed. The tonsillar herniation and hydrocephalus both resolved after AVM was excised. This case provides some insight into the complex hemodynamic change exerted by the fistulous AVM and the mechanism of the development of acquired Chiari malformation type I. PMID:25081218

  1. Tertiary Lymphoid Organs in Lymphatic Malformations

    PubMed Central

    Kirsh, Andrew L.; Cushing, Sharon L.; Chen, Eunice Y.; Schwartz, Stephen M.

    2011-01-01

    Abstract Background Examine lymphatic malformation lymphoid aggregates for the expression of tertiary lymphoid organ markers. Determine how lymphoid aggregate density relates to lymphatic malformation clinical features. Methods and Results Retrospective cohort study. Tissue and clinical data were reviewed from 29 patients in the Vascular Anomaly Database who represented the spectrum of head and neck lymphatic malformations and had >5 years of follow-up. Archived formalin-fixed, paraffin-embedded lymphatic malformation tissue was immunohistochemically stained with antibodies for tertiary lymphoid organ markers, which included follicular and mature myeloid dendritic cells, high endothelial venules, segregated B and T-cells, lymphatic endothelial cells, and lymphoid homing chemokines (CXCL13, CCL21). Lymphoid aggregate density (count/mm2) was quantified by 2 independent, blinded reviewers. Lymphoid aggregate density and lymphatic malformation clinical features were characterized using analysis of variance. Larger lymphatic malformation tissue lymphoid aggregates stained consistently for tertiary lymphoid organ markers. In oral cavity and neck specimens from the same patients (n = 9), there were more tertiary lymphoid organ in oral cavity than in neck specimens (p = 0.0235). In lymphatic malformation neck tissue, de Serres stage 4 lymphatic malformations displayed the highest tertiary lymphoid organ density. No significant association was seen between tertiary lymphoid organ density and other clinical features. Conclusion This study demonstrates that some lymphoid aggregates within lymphatic malformations represent tertiary lymphoid organs. There was an association between tertiary lymphoid organ density and lymphatic malformation location. Further study is required to define the role of lymphoid neogenesis and tertiary lymphoid organ formation in lymphatic malformation pathogenesis. PMID:21688977

  2. Ear problems and injuries in athletes.

    PubMed

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

    2014-01-01

    The ear is an unique organ--the principal structure involved in both hearing and balance. Although not common, problems with the ear may be encountered in specific sporting populations. Common conditions affecting the ear in the athlete include otitis externa, an infection of the external ear; external auditory canal exostoses, or abnormal bony growths in the canal; and otitis media, an infection of the middle ear. Given its position on the head, the ear is subject to trauma, often resulting in an auricular hematoma. Divers, due to pressure changes on descent and ascent, are subject to both ear barotrauma and ear decompression sickness. This article will discuss recognition, treatment, and prevention of these conditions affecting the ear in the athlete. PMID:24412886

  3. 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

  4. 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

  5. Fgf9 signaling regulates inner ear morphogenesis through epithelial-mesenchymal interactions.

    PubMed

    Pirvola, Ulla; Zhang, Xiuqin; Mantela, Johanna; Ornitz, David M; Ylikoski, Jukka

    2004-09-15

    The mammalian inner ear comprises the cochleovestibular labyrinth, derived from the ectodermal otic placode, and the encasing bony labyrinth of the temporal bone. Epithelial-mesenchymal interactions are thought to control inner ear development, but the modes and the molecules involved are largely unresolved. We show here that, during the precartilage and cartilage stages, Fgf9 is expressed in specific nonsensory domains of the otic epithelium and its receptors, Fgfr1(IIIc) and Fgfr2(IIIc), widely in the surrounding mesenchyme. To address the role of Fgf9 signaling, we analyzed the inner ears of mice homozygous for Fgf9 null alleles. Fgf9 inactivation leads to a hypoplastic vestibular component of the otic capsule and to the absence of the epithelial semicircular ducts. Reduced proliferation of the prechondrogenic mesenchyme was found to underlie capsular hypoplasticity. Semicircular duct development is blocked at the initial stages, since fusion plates do not form. Our results show that the mesenchyme directs fusion plate formation and they give direct evidence for the existence of reciprocal epithelial-mesenchymal interactions in the developing inner ear. In addition to the vestibule, in the cochlea, Fgf9 mutation caused defects in the interactions between the Reissner's membrane and the mesenchymal cells, leading to a malformed scala vestibuli. Together, these data show that Fgf9 signaling is required for inner ear morphogenesis. PMID:15328018

  6. Anesthesia and intracranial arteriovenous malformation.

    PubMed

    Sinha, Prabhat Kumar; Neema, Praveen Kumar; Rathod, Ramesh Chandra

    2004-06-01

    Anesthetic management of intracranial arteriovenous malformation (AVM) poses multiple challenges to the anesthesiologist in view of its complex and poorly understood pathophysiology and multiple modalities for its treatment involving different sub-specialties. The diagnosis of AVM is based on clinical presentation as well as radiological investigation. Pregnant patients with intracranial AVM and neonates with vein of Galen malformation may also pose a special challenge and require close attention. Despite technological advancement, reported morbidity or mortality after AVM treatment remains high and largely depends on age of the patient, recruitment of perforating vessels, its size, location in the brain, history of previous bleed and post-treatment hyperemic complication. Anesthetic management includes a thorough preoperative visit with meticulous planning based on different modalities of treatment including anesthesia for radiological investigation. Proper attention should be directed while transporting the patient for the procedure. Protection of the airway, adequate monitoring, and maintaining neurological and cardiovascular stability, and the patient's immobility during the radiological procedures, appreciation and management of various complications that can occur during and after the procedure and meticulous ICU management is essential. PMID:15269462

  7. Chiari I malformation presenting as chronic cough.

    PubMed

    Amos, Louella B; Quintero, Diana R

    2008-10-01

    We present a 9-month-old infant with persistent cough refractory to conventional asthma therapy. An extensive evaluation eventually revealed a Chiari I malformation with syringohydromyelia. His cough resolved one month after surgical decompression, suggesting that brainstem compression from the Chiari malformation directly caused his symptoms. PMID:18785260

  8. Complicated pneumothorax and congenital lung cystic malformation.

    PubMed

    Attou, Rachid; Reper, Pascal

    2014-04-01

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

  9. Tuning in the bullfrog ear.

    PubMed Central

    Lewis, E R

    1988-01-01

    When electrical resonances were observed in acoustic sensory cells of lower vertebrates, the hearing research community was presented with the exciting possibility that tuning in the ears of those animals might be explained directly in terms of familiar molecular devices. It is reported here that in the frog sacculus, where electrical resonances have been observed in isolated hair cells, the effects of those resonances are completely obscured in the tuning properties of the sacculus in the intact ear. This observation has important implications not only for students of the ear, but for reductionist biologists in general. All of the dynamic properties of a system of connected, bidirectional processes are consequences of all of those processes at once; in such a system, the properties of an experimentally isolated subsystem may be totally obscured in the operation of the system as a whole. PMID:3258166

  10. 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

  11. Mutations in KCTD1 Cause Scalp-Ear-Nipple Syndrome

    PubMed Central

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

    2013-01-01

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

  12. Inner Ear Drug Delivery for Auditory Applications

    PubMed Central

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

    2008-01-01

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

  13. Physiological functioning of the ear and masking

    NASA Technical Reports Server (NTRS)

    1984-01-01

    The physiological functions of the ear and the role masking plays in speech communication are examined. Topics under investigation include sound analysis of the ear, the aural reflex, and various types of noise masking.

  14. Middle Ear Infections (For Parents)

    MedlinePlus

    ... speak. Sound, which is made up of invisible waves of energy, causes these vibrations. Every time you hear a sound, the various structures of the ear have to work together to make sure ... Hearing begins when sound waves that travel through the air reach the outer ...

  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. New insights into craniofacial malformations.

    PubMed

    Twigg, Stephen R F; Wilkie, Andrew O M

    2015-10-15

    Development of the human skull and face is a highly orchestrated and complex three-dimensional morphogenetic process, involving hundreds of genes controlling the coordinated patterning, proliferation and differentiation of tissues having multiple embryological origins. Craniofacial malformations that occur because of abnormal development (including cleft lip and/or palate, craniosynostosis and facial dysostoses), comprise over one-third of all congenital birth defects. High-throughput sequencing has recently led to the identification of many new causative disease genes and functional studies have clarified their mechanisms of action. We present recent findings in craniofacial genetics and discuss how this information together with developmental studies in animal models is helping to increase understanding of normal craniofacial development. PMID:26085576

  17. Can Loud Music Hurt My Ears?

    MedlinePlus

    ... Snowboarding, Skating Crushes What's a Booger? Can Loud Music Hurt My Ears? KidsHealth > For Kids > Can Loud Music Hurt My Ears? Print A A A Text ... up? Oh! You want to know if loud music can hurt your ears . Are you asking because ...

  18. Handedness and Preferred Ear for Telephoning.

    ERIC Educational Resources Information Center

    Williams, Stephen M.

    1987-01-01

    Examined relationship between handedness and preferred ear for telephoning in 140 college students. Increased degree of sinistrality was associated with increased tendency to use left ear for telephoning. Found tendency to pick up telephone receiver with preferred hand and hold earpiece to ipsilateral ear. Results may relate to reports of reduced…

  19. 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)

  20. 21 CFR 878.3590 - Ear prosthesis.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ... 21 Food and Drugs 8 2012-04-01 2012-04-01 false Ear prosthesis. 878.3590 Section 878.3590 Food and... GENERAL AND PLASTIC SURGERY DEVICES Prosthetic Devices § 878.3590 Ear prosthesis. (a) Identification. An ear prosthesis is a silicone rubber solid device intended to be implanted to reconstruct the...

  1. 21 CFR 878.3590 - Ear prosthesis.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ... 21 Food and Drugs 8 2014-04-01 2014-04-01 false Ear prosthesis. 878.3590 Section 878.3590 Food and... GENERAL AND PLASTIC SURGERY DEVICES Prosthetic Devices § 878.3590 Ear prosthesis. (a) Identification. An ear prosthesis is a silicone rubber solid device intended to be implanted to reconstruct the...

  2. 21 CFR 878.3590 - Ear prosthesis.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ... 21 Food and Drugs 8 2013-04-01 2013-04-01 false Ear prosthesis. 878.3590 Section 878.3590 Food and... GENERAL AND PLASTIC SURGERY DEVICES Prosthetic Devices § 878.3590 Ear prosthesis. (a) Identification. An ear prosthesis is a silicone rubber solid device intended to be implanted to reconstruct the...

  3. Normative inner ear volumetric measurements.

    PubMed

    Teixido, Michael T; Kirkilas, Gary; Seymour, Peter; Sem, Kanik; Iaia, Alberto; Sabra, Omar; Isildak, Huseyin

    2015-01-01

    In the current study, we attempted to determine normative inner ear volumetric measurements generated from three-dimensional computed tomography (CT) images. In addition, we investigated a correlation between the axial length and the volume of the labyrinth and discussed clinical outcomes of this correlation. Amira 5.2.2 software was used to create three-dimensional isosurface images of the human labyrinth using two-dimensional CT images from 35 anatomically normal patients. With the three-dimensional labyrinths, complete dimensional analysis was performed to gain insight into both the volume and the greatest axial length of the inner ear. Paired t test and Pearson correlation were used. Our volume of the inner ear inquiry reported a mean volume of 221.5 with SD of 24.3 μL (0.228 μL for males and 0.218 μL for females). The length showed a mean of 1.713 cm with SD of 0.064 cm (1.753 cm for males and 1.695 cm for females). The length was used to estimate the volume, and the estimates were within 10% of the measured volume 74.3% of the time. Normative volumetric measurements of the inner ear can be obtained by using three-dimensional CT Imaging by Amira 5.2.2 software. There was a statistically significant positive correlation between the axial length of the labyrinth and the volume of the labyrinth. The axial length of the labyrinth could be used to estimate the volume of the labyrinth, which may be clinically important to estimate the concentration of the drug distributed in the inner ear. PMID:25490572

  4. Computed tomography of congenital brain malformations

    SciTech Connect

    Sarwar, M.

    1984-01-01

    This book is illustrated showing each condition. This book is designed to correlate the pathology of CNS malformations with their CT scan appearance, mainly on the axial images. The author has drawn upon his personal experience and the information gleaned from the literature dealing with the description of the CT scan findings of these malformations. The emphasis is on simplicity of description. Since a large degree of morphological variation exists in each entity, numerous illustrations (wherever applicable) are shown to depict those variations. When appropriate, deficiency of the CT scan in the evaluation of these CNS malformations also is indicated. A description of CNS embryology is included as well.

  5. Gbx2 is required for the morphogenesis of the mouse inner ear: a downstream candidate of hindbrain signaling.

    PubMed

    Lin, Zhengshi; Cantos, Raquel; Patente, Maria; Wu, Doris K

    2005-05-01

    Gbx2 is a homeobox-containing transcription factor that is related to unplugged in Drosophila. In mice, Gbx2 and Otx2 negatively regulate each other to establish the mid-hindbrain boundary in the neural tube. Here, we show that Gbx2 is required for the development of the mouse inner ear. Absence of the endolymphatic duct and swelling of the membranous labyrinth are common features in Gbx2-/- inner ears. More severe mutant phenotypes include absence of the anterior and posterior semicircular canals, and a malformed saccule and cochlear duct. However, formation of the lateral semicircular canal and its ampulla is usually unaffected. These inner ear phenotypes are remarkably similar to those reported in kreisler mice, which have inner ear defects attributed to defects in the hindbrain. Based on gene expression analyses, we propose that activation of Gbx2 expression within the inner ear is an important pathway whereby signals from the hindbrain regulate inner ear development. In addition, our results suggest that Gbx2 normally promotes dorsal fates such as the endolymphatic duct and semicircular canals by positively regulating genes such as Wnt2b and Dlx5. However, Gbx2 promotes ventral fates such as the saccule and cochlear duct, possibly by restricting Otx2 expression. PMID:15829521

  6. Postoperative ramifications of total ear replantation.

    PubMed

    Finical, S J; Keller, K M; Lovett, J E

    1998-12-01

    Complete avulsion of the external ear, much less common than other minor ear trauma, presents a complex and difficult problem. The authors present a 35-year-old male cigarette smoker who suffered traumatic avulsion of the right ear, and describe the replantation procedure and the patient's postoperative course. Aesthetic positioning of the replanted ear, treating the external auditory meatus, protecting the ear against exposure to the sun, and counseling the patient about issues related to sensation are important factors in the care of patients who have undergone such a procedure. PMID:9869143

  7. Autogenous and prosthetic reconstruction of the ear.

    PubMed

    Louis, Patrick J; Aponte-Wesson, Ruth A; Fernandes, Rui P; Clemow, Justin

    2013-05-01

    Injuries to the ear can result in partial or complete loss of the external ear. Resection of the external ear may be necessary secondary to malignant tumor or infection. This article discusses the diagnosis and management of acquired defects of the external ear. Because autogenous reconstruction is not always possible, both autogenous and prosthetic reconstruction are presented as well as the indications for both. This information should help guide the clinician in the decision-making process. In the hands of experienced clinicians, reconstruction of the external ear can result in an excellent outcome, with improved quality of life for the patient. PMID:23522966

  8. [Gas ventilation through middle ear mucosa].

    PubMed

    Ohno, F; Imakiire, T; Nobori, T; Ohyama, M

    1993-11-01

    The possible effects of gas ventilation via the middle ear mucosa on middle ear pressure changes with N2O inhalation were studied. Sixty-seven ears without otologic problems were selected from among cases undergoing ENT surgery under general anesthesia. Anesthesia was induced by isoflurane or sevoflurane, and inhalation with a gas mixture of 21/min O2 and 31/min N2O was started under controlled respiration via endotracheal tube. Middle ear pressure was measured each minute with tympanometry just before and during N2O inhalation. Changes in pressure were plotted against time on an X-Y plotter, and the rate of middle ear pressure change and pressure change in 10 minutes were calculated. The following results were obtained; 1. In all cases, the middle ear pressure rose with N2O inhalation. However, the same concentration of N2O created different middle ear pressure changes in different individuals varying from 1.6 mmH2O to 107.8 mmH2O per minute. 2. The rate of middle ear pressure elevation tended to be greater in younger subjects, especially in children aged 4 to 7. 3. There was a negative correlation between the middle ear pressure change and pneumatization of the middle ear. 4. Sex, pulmonary function, and the N2O expiratory flow concentration did not contribute to the variability in the rate of the middle ear pressure elevation. PMID:8283337

  9. 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

  10. Genetic analysis of malformations causing perinatal mortality.

    PubMed Central

    Young, I D; Rickett, A B; Clarke, M

    1986-01-01

    An analysis of congenital malformations, other than neural tube defects, which have contributed to perinatal mortality in Leicestershire is presented for the years 1976 to 1982 inclusive. Chromosomal, single gene, or polygenic inheritance accounted for 67% of cases. PMID:3950936

  11. Genetics Home Reference: Dandy-Walker malformation

    MedlinePlus

    ... from mild to severe, and those with normal intelligence may have learning disabilities. Children with Dandy-Walker ... Dandy-Walker Malformation: A Clinical and Surgical Outcome Analysis. J Coll Physicians Surg Pak. 2015 Jun;25( ...

  12. [Hemangiomas and vascular malformations. Review and update].

    PubMed

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

    1997-07-01

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

  13. EEG Recorded from the Ear: Characterizing the Ear-EEG Method

    PubMed Central

    Mikkelsen, Kaare B.; Kappel, Simon L.; Mandic, Danilo P.; Kidmose, Preben

    2015-01-01

    Highlights Auditory middle and late latency responses can be recorded reliably from ear-EEG.For sources close to the ear, ear-EEG has the same signal-to-noise-ratio as scalp.Ear-EEG is an excellent match for power spectrum-based analysis. A method for measuring electroencephalograms (EEG) from the outer ear, so-called ear-EEG, has recently been proposed. The method could potentially enable robust recording of EEG in natural environments. The objective of this study was to substantiate the ear-EEG method by using a larger population of subjects and several paradigms. For rigor, we considered simultaneous scalp and ear-EEG recordings with common reference. More precisely, 32 conventional scalp electrodes and 12 ear electrodes allowed a thorough comparison between conventional and ear electrodes, testing several different placements of references. The paradigms probed auditory onset response, mismatch negativity, auditory steady-state response and alpha power attenuation. By comparing event related potential (ERP) waveforms from the mismatch response paradigm, the signal measured from the ear electrodes was found to reflect the same cortical activity as that from nearby scalp electrodes. It was also found that referencing the ear-EEG electrodes to another within-ear electrode affects the time-domain recorded waveform (relative to scalp recordings), but not the timing of individual components. It was furthermore found that auditory steady-state responses and alpha-band modulation were measured reliably with the ear-EEG modality. Finally, our findings showed that the auditory mismatch response was difficult to monitor with the ear-EEG. We conclude that ear-EEG yields similar performance as conventional EEG for spectrogram-based analysis, similar timing of ERP components, and equal signal strength for sources close to the ear. Ear-EEG can reliably measure activity from regions of the cortex which are located close to the ears, especially in paradigms employing frequency-domain analyses. PMID:26635514

  14. The Microcephaly-Capillary Malformation Syndrome

    PubMed Central

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

    2012-01-01

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

  15. 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

  16. Venous Malformation: update on etiopathogenesis, diagnosis & management

    PubMed Central

    Dompmartin, Anne; Vikkula, Miikka; Boon, Laurence M

    2011-01-01

    The aim of this review was to discuss the current knowledge on etiopathogenesis, diagnosis and therapeutic management of venous malformations. Venous malformations (VMs) are slow-flow vascular anomalies. They are simple, sporadic or familial (cutaneo-mucosal venous malformation or glomuvenous malformations), combined (e.g. capillaro-venous, capillaro-lymphaticovenous malformations) or syndromic (Klippel-Trenaunay, Blue Rubber Bleb Naevus and Maffucci). Genetic studies have identified causes of familial forms and of 40% of sporadic VMs. Another diagnostic advancement is the identification of elevated D-dimer level as the first biomarker of venous malformations within vascular anomalies. Those associated with pain are often responsive to Low Molecular Weight Heparin which should also be used to avoid disseminated intravascular coagulopathy secondary to intervention, especially if fibrinogen level is low. Finally, development of a modified sclerosing agent, ethylcellulose–ethanol, has improved therapy. It is efficient and safe, and widens indications for sclerotherapy to sensitive and dangerous areas such as hands, feet and periocular area. PMID:20870869

  17. 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

  18. Role of hindbrain in inner ear morphogenesis: Analysis of Noggin knockout mice

    PubMed Central

    Bok, Jinwoong; Brunet, Lisa J.; Howard, Omar; Burton, Quianna; Wu, Doris K.

    2007-01-01

    Signaling from rhombomeres 5 and 6 of the hindbrain is thought to be important for inner ear patterning. In Noggin -/- embryos, the gross anatomy of the inner ear is distorted and malformed, with cochlear duct outgrowth and coiling most affected. We attributed these defects to a caudal shift of the rhombomeres caused by the shortened body axis and the kink in the neural tube. To test the hypothesis that a caudal shift of the rhombomeres affects inner ear development, we surgically generated chicken embryos in which rhombomeres 5 and 6 were similarly shifted relative to the position of the inner ears, as in Noggin mutants. All chicken embryos with shifted rhombomeres showed defects in cochlear duct formation indicating that signaling from rhombomeres 5 and 6 is important for cochlear duct patterning in both chicken and mice. In addition, the size of the otic capsule is increased in Noggin -/- mutants, which most likely is due to unopposed BMP signaling for chondrogenesis in the peri-otic mesenchyme. PMID:17900554

  19. Some Remarks on Imaging of the Inner Ear: Options and Limitations.

    PubMed

    Giesemann, A; Hofmann, E

    2015-10-01

    The temporal bone has a highly complex anatomical structure, in which the sensory organs of the cochlea and the vestibular system are contained within a small space together with the sound-conducting system of the middle ear. Detailed imaging is thus required in this anatomical area. There are a great many clinical aims for which the highest-possible spatial resolution is required. These include the localization of cerebrospinal fluid fistulas, the detection of malformations of the middle and inner ear and the vestibulocochlear nerve, an aberrant course of the facial nerve and anomalies of the arterial and venous structures, the confirmation of dehiscence of the semicircular canals and finally, the verification of endolymphatic hydrops in cases of Ménière's disease. However, the term 'high resolution' is very time dependent. Two milestones in this respect have been (in 1991) the 3D visualization of the inner ear by means of maximum-intensity projection (MIP) of a T2-weighted constructive interference in steady state (CISS) sequence of a 1.5-tesla magnetic resonance imaging (MRI) scanner (Tanioka et al., Radiology 178:141-144, 1991) and (in 1997) imaging of the vestibulocochlear nerve for the diagnosis of hypoplasia inside the internal auditory canal using the same sequence (Casselman et al., Radiology 202:773-781, 1997).The objective of this article is to highlight the options for, and the challenges of, contemporary imaging with regard to some clinical issues relating to the inner ear. PMID:26153464

  20. Curing arteriovenous malformations using embolization.

    PubMed

    Potts, Matthew B; Zumofen, Daniel W; Raz, Eytan; Nelson, Peter K; Riina, Howard A

    2014-09-01

    Endovascular embolization is typically reserved as an adjuvant therapy in the management of cerebral arteriovenous malformations (AVMs), either for preoperative devascularization or preradiosurgical volume reduction. Curative embolization plays a limited role in AVM treatment but several studies have shown that it is possible, especially with later-generation liquid embolic agents. Given the complexity of AVM anatomy and the recent controversies over the role of any intervention in AVM management, it is critical that the cerebrovascular community better define the indications of each treatment modality to provide quality AVM management. In this review, the authors evaluate the role of curative AVM embolization. Important considerations in the feasibility of curative AVM embolization include whether it can be performed reliably and safely, and whether it is a durable cure. Studies over the past 20 years have begun to define the anatomical factors that are amenable to complete endovascular occlusion, including size, feeding artery anatomy, AVM morphology, and endovascular accessibility. More recent studies have shown that highly selected patients with AVMs can be treated with curative intent, leading to occlusion rates as high as 100% of such prospectively identified lesions with minimal morbidity. Advances in endovascular technology and techniques that support the efficacy and safety of curative embolization are discussed, as is the importance of superselective diagnostic angiography. Finally, the durability of curative embolization is analyzed. Overall, while still unproven, endovascular embolization has the potential to be a safe, effective, and durable curative treatment for select AVMs, broadening the armamentarium with which one can treat this disease. PMID:25175438

  1. 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.

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

    PubMed Central

    Muteweye, Wilfred; Muguti, Godfrey I.

    2015-01-01

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

  3. Delayed presentation of anorectal malformations

    PubMed Central

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

    2008-01-01

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

  4. Bacteriology of the chronically discharging middle ear.

    PubMed

    Karma, P; Jokippi, L; Ojala, K; Jokipii, A M

    1978-01-01

    Suitable bacteriological techniques revealed anaerobic bacteria in 38 (33%) of 114 chronically discharging middle ears. The genus Bacteroides was cultured from 25 ears. Aerobic bacteriology showed the predominance of staphylococci, facultative enteric gramnegative rods, diphtheroid bacilli and Pseudomonas species. Anaerobic bacterial cultures were always mixed with aerobic bacteria. 12 ears were culture-negative, and 9 of the 108 Gram-stained smers revealed no bacteria. No significant difference in bacteriology was noted between ears with or without local antimicrobial treatment, or between profusely draining or only moist ears. The ears with postoperative recurrent infection or with clinical suspicion of cholesteatoma grew anaerobes significantly more often, and were seldom sterile. Because anaerobic bacteria are frequently associated with chronic otitis media, their characteristics with regard to susceptibility to antimicrobials and to air must be remembered in the choice of therapy. PMID:358732

  5. Local Inner Ear Drug Delivery and Pharmacokinetics

    PubMed Central

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

    2008-01-01

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

  6. Spectrum of urorectal septum malformation sequence.

    PubMed

    Shah, Krupa; Nayak, Shalini S; Shukla, Anju; Girisha, Katta M

    2016-05-01

    Urorectal septum malformation sequence (URSMS) is a rare spectrum of malformations involving various organ systems. Here, we present eight cases of URSMS, noted in autopsy, with different degrees of complexity, seven being the complete type and one being the partial type. All cases had gastrointestinal tract malformation in the form of the imperforate anus and indeterminate genitalia. Other gastrointestinal tract anomalies were anal agenesis in two cases, anorectal agenesis in two cases, and malformed lower intestinal tract in four cases. The associated renal abnormality was noted in five cases, which were unilateral renal agenesis, dysplastic kidney, hydronephrosis, horseshoe kidney, and unilateral hypoplastic ectopic kidney. External genital malformation, present in both male and female fetuses, included a knob-like structure at perineum in female fetuses, genital fold hypoplasia and penile aplasia or hypoplasia in male fetuses. Skeletal abnormalities included two cases of sacral agenesis and one case of lumbosacral dysraphism. Other anomalies included a case with alobar holoprosencephaly, truncus arteriosus with hypoplastic lungs in one case, and three cases with abdominal wall defects. It is our attempt to delineate a spectrum of abnormalities associated with URSMS. PMID:26663027

  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. Listening to Nature's orchestra with peculiar ears

    NASA Astrophysics Data System (ADS)

    Yager, David D.

    2003-04-01

    Insects use hearing for the crucial tasks of communicating with conspecifics and avoiding predators. Although all are based on the same acoustic principles, the diversity of insect ears is staggering and instructive. For instance, a South African grasshopper demonstrates that hearing conspecific calls is possible over distances 1 km with ears that do not have tympana. Actually, these creatures have six pairs of ears that play different roles in behavior. In numerical contrast, praying mantises have just a single ear in the ventral midline. The ear is very effective at detecting ultrasonic bat cries. However, the bioacoustics of sound transduction by two tympana facing each other in a deep, narrow slit is a puzzle. Tachinid flies demonstrate that directional hearing at 5 kHz is possible with a pair of ears fused together to give a total size of 1 mm. The ears are under the fly's chin. Hawk moths have their ears built into their mouthparts and the tympanum is more like a hollow ball than the usual membrane. As an apt last example, cicada ears are actually part of the orchestra: their tympana function both in sound reception and sound production.

  9. Management of Chronic Middle Ear Effusion

    PubMed Central

    Thomas, Gary L.

    1969-01-01

    Management of chronic middle ear effusion must center around the reestablishment of normal eustachian tube function. If fluid does not clear with medical management, aspiration becomes necessary. A prosthetic eustachian tube is placed in the tympanic membrane in order to artificially ventilate the middle ear space while primary etiologic factors are being corrected. Unless recurrent or chronic ear disease of this nature is diligently treated, progressive damage may bring about irreversible hearing loss and the potentially dangerous disease, cholesteatoma of the ear. ImagesFigure 1.Figure 2. PMID:5798499

  10. Milestones in the History of Ear Reconstruction.

    PubMed

    Berghaus, Alexander; Nicoló, Marion San

    2015-12-01

    The reconstruction of ear deformities has been challenging plastic surgeons since centuries. However, it is only in the 19th century that reports on partial and total ear reconstruction start increasing. In the quest for an aesthetically pleasing and natural-looking result, surgeons worked on the perfect framework and skin coverage. Different materials and flap techniques have evolved. Some were abandoned out of frustration, while others kept evolving over the years. In this article, we discuss the milestones in ear reconstruction-from ancient times to early attempts in Western civilization to the key chapters of ear reconstruction in the 20th century leading to the current techniques. PMID:26667630

  11. Endovascular treatment of spinal cord arteriovenous malformations.

    PubMed

    Veznedaroglu, Erol; Nelson, Peter K; Jabbour, Pascal M; Rosenwasser, Robert H

    2006-11-01

    Spinal cord arteriovenous malformations are rare lesions that represent one-tenth of the brain arteriovenous malformations. Depending on their location and relationship to the dura, these lesions are divided into four categories. Their clinical manifestations may vary from mild symptoms to severe motor deficits. Spinal angiography remains the "gold standard" for diagnosing spinal cord vascular lesions. Although the type of shunting remains difficult to determine by the magnetic resonance imaging, it is well analyzed by spinal angiography. The cure of the shunting is not by itself a therapeutic goal, but the objective is the creation of a new hemodynamic equilibrium between the lesion and the spinal cord to decrease the risk of hemorrhage and prevent the progression of the spinal cord ischemia. The endovascular tools seem to be a reasonable therapeutic option for the treatment of the majority of the spinal cord arteriovenous malformations. PMID:17053604

  12. Anorectal Malformation: Paediatric Problem Presenting in Adult

    PubMed Central

    Chavan, Rahulkumar N.; Chikkala, Bhargav; Das, Cinjini; Biswas, Somak; Sarkar, Diptendra Kumar; Pandey, Sushil Kumar

    2015-01-01

    This is a case report of 22-year-old girl admitted with abdominal distension, vomiting, and chronic constipation since birth. Abdomen was distended, and perineal examination revealed imperforate anus with vestibular fistula (ARM). So far worldwide very few cases have been reported about anorectal malformation presenting in adulthood, and thus extremely little data is available in the literature about an ideal management of anorectal malformation in adults. In our case in the treatment instead of conventional procedure of posterior sagittal anorectoplasty (PSARP) anal transposition was done and till two years after the definitive treatment during follow-up patient has been doing well with Kelly's score of six. Our experience suggests that anal transposition provides satisfactory outcome in adults presenting late with anorectal malformation. PMID:26539301

  13. Sex hormone exposure during pregnancy and malformations.

    PubMed

    Briggs, M H; Briggs, M

    1979-01-01

    This general review of the effects of exposure to sex hormones during pregnancy and subsequent fetal malformation presents summaries of animal studies, develops the data indicating virilization and feminization in humans, documents chromosome abnormalities, and presents data on the connection of steroid exposure in utero and somatic malformations. Fetal exposure can occur 3 different ways, through hormonal pregnancy test, via obstetrical use of hormones, or because of continued maternal use of oral contraceptives after conception. In the latter case, an ongoing prospective study indicates that accidental ingestion of oral contraceptives after conception is not harmful to the fetus if taken during early pregnancy. Tables present summaries of numerous large surveys and retrospective studies linking particular sex hormones (exogenous) to particular fetal malformations including neural tube defects and other constellations of developmental problems. The question of exogenous hormone effects on the personality of infants who were exposed in utero is addressed. PMID:400321

  14. Percutaneous Cryotherapy of Vascular Malformation: Initial Experience

    SciTech Connect

    Cornelis, F.; Neuville, A.; Labreze, C.; Kind, M.; Bui, B.; Midy, D.; Palussiere, J.; Grenier, N.

    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.

  15. Congenital Malformations Leading to Paradoxical Embolism.

    PubMed

    Bruckheimer, Elchanan

    2016-05-01

    The absolute separation of the right and left circulations and the filtration of blood by the pulmonary circulation are essential to prevent the passage of thrombotic material from the venous system into the systemic arterial circulation. Any breach of the intracardiac septae or circumvention of the pulmonary capillary network may cause a paradoxical embolus. The most common causes are atrial septal defects and pulmonary arteriovenous malformations. This article discusses unusual connections and pathways related to congenital malformations. Although anticoagulation is necessary to prevent paradoxical emboli, the hematologic disturbances and the most appropriate therapy in these patients warrant further investigation. PMID:27150173

  16. Vascular malformations: localized defects in vascular morphogenesis.

    PubMed

    Brouillard, P; Vikkula, M

    2003-05-01

    Vascular anomalies are localized defects of the vasculature, and usually affect a limited number of vessels in a restricted area of the body. They are subdivided into vascular malformations and vascular tumours. Most are sporadic, but Mendelian inheritance is observed in some families. By genetic analysis, several causative genes have been identified during the last 10 years. This has shed light into the pathophysiological pathways involved. Interestingly, in most cases, the primary defect seems to affect the characteristics of endothelial cells. Only mutations in the glomulin gene, responsible for hereditary glomuvenous malformations, are thought to directly affect vascular smooth-muscle cells. PMID:12752563

  17. Retroperitoneal vascular malformation mimicking incarcerated inguinal hernia.

    PubMed

    Dubey, Indu Bhushan; Sharma, Anuj; Singh, Ajay Kumar; Mohanty, Debajyoti

    2011-01-01

    A 30-year-old man presented to the Department of Surgery with a painful groin swelling on right side. Exploration revealed a reddish-blue hemangiomatous mass in the scrotum extending through inguinal canal into the retroperitoneum. On further dissection swelling was found to be originating from right external iliac vein. The swelling was excised after ligating all vascular connections. The histopathological examination of excised mass confirmed the diagnosis of venous variety of vascular malformation. This is the first reported case of vascular malformation arising from retroperitoneum and extending into inguinoscrotal region, presenting as incarcerated inguinal hernia. PMID:21633582

  18. Total knee arthroplasty in vascular malformation

    PubMed Central

    Bhende, Harish; Laud, Nanadkishore; Deore, Sandeep; Shashidhar, V

    2015-01-01

    In Klippel–Trenaunay syndrome, vascular malformations are not only in skin and superficial soft tissues but also in deep tissues like muscles bones and joints. It is well documemted that these recurrent intraarticular bleeds can cause early arthritis and joint pain. Performing arthroplasty in such patients is difficult and fraught with complications. We describe such a case where navigated total knee arthroplasty was performed with success to avoid the problems of intra medullary alignment used in the presence of intra medullary vascular malformations. We also suggest certain measures when knee arthroplasty is considered in such patients. PMID:26538765

  19. Congenital cystic adenomatoid malformation type I

    PubMed Central

    Ribeiro, Frederico Becker; Schultz, Regina

    2015-01-01

    Congenital cystic adenomatoid malformation (CCAM) is an hamartomatous congenital pulmonary airway malformation with incidence ranging between 1:10,000 and 1:35,000 newborns. Currently CCAM is classified into five groups according to clinical and pathological features. The clinical outcome varies depending on the subtype and the extent of involvement. The authors report the case of a premature male newborn with the prenatal diagnosis of CCAM Type 1 associated with cardiac right axis deviation, who died 67 hours after birth due to respiratory failure. In addition to the autopsy report of this rare entity, the authors present its classification and prognosis. PMID:26558243

  20. Neurodevelopmental malformations of the cerebellar vermis in genetically engineered rats

    EPA Science Inventory

    The cerebellar vermis is particularly vulnerable to neurodevelopmental malformations in humans and rodents. Sprague-Dawley, and Long-Evans rats exhibit spontaneous cerebellar malformations consisting of heterotopic neurons and glia in the molecular layer of the vermis. Malformati...

  1. [Lymphatic malformations in the head and neck area].

    PubMed

    Wiegand, S; Werner, J A

    2016-02-01

    Lymphatic malformations are congenital malformations of the lymphatic system. They are mainly located in the head and neck area, and grow proportional to the patients' body growth. Depending on the morphology, it can be distinguished between macrocystic, microcystic and mixed lymphatic malformations. Due to their infiltrative growth, microcystic lymphatic malformations are particularly difficult to treat. Therapeutic approaches include conventional surgical resection, laser therapy, sclerotherapy and systemic drug therapies. PMID:26820157

  2. Playing by Ear: Foundation or Frill?

    ERIC Educational Resources Information Center

    Woody, Robert H.

    2012-01-01

    Many people divide musicians into two types: those who can read music and those who play by ear. Formal music education tends to place great emphasis on producing musically literate performers but devotes much less attention to teaching students to make music without notation. Some would suggest that playing by ear is a specialized skill that is…

  3. 21 CFR 870.2710 - Ear oximeter.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 21 Food and Drugs 8 2011-04-01 2011-04-01 false Ear oximeter. 870.2710 Section 870.2710 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES CARDIOVASCULAR DEVICES Cardiovascular Monitoring Devices § 870.2710 Ear oximeter. (a) Identification. An...

  4. 21 CFR 870.2710 - Ear oximeter.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 21 Food and Drugs 8 2010-04-01 2010-04-01 false Ear oximeter. 870.2710 Section 870.2710 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES CARDIOVASCULAR DEVICES Cardiovascular Monitoring Devices § 870.2710 Ear oximeter. (a) Identification. An...

  5. 21 CFR 878.3590 - Ear prosthesis.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 21 Food and Drugs 8 2011-04-01 2011-04-01 false Ear prosthesis. 878.3590 Section 878.3590 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES GENERAL AND PLASTIC SURGERY DEVICES Prosthetic Devices § 878.3590 Ear prosthesis. (a) Identification....

  6. Cutaneous lesions of the external ear

    PubMed Central

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

    2008-01-01

    Skin diseases on the external aspect of the ear are seen in a variety of medical disciplines. Dermatologists, othorhinolaryngologists, general practitioners, general and plastic surgeons are regularly consulted regarding cutaneous lesions on the ear. This article will focus on those diseases wherefore surgery or laser therapy is considered as a possible treatment option or which are potentially subject to surgical evaluation. PMID:18261212

  7. INNER EAR EMBRYOGENESIS: GENETIC AND ENVIRONMENTAL DETERMINANTS

    EPA Science Inventory

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

  8. 21 CFR 878.3590 - Ear prosthesis.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 21 Food and Drugs 8 2010-04-01 2010-04-01 false Ear prosthesis. 878.3590 Section 878.3590 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES GENERAL AND PLASTIC SURGERY DEVICES Prosthetic Devices § 878.3590 Ear prosthesis. (a) Identification....

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

    PubMed

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

    2012-06-01

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

  10. Coexistent arteriovenous malformation and hippocampal sclerosis.

    PubMed

    Prayson, Richard A; O'Toole, Elizabeth E

    2016-06-01

    Cavernous angiomas or cavernomas have been occasionally described in patients presenting with medically intractable epilepsy. Reports of cavernomas associated with a second pathology potentially causative of seizures have rarely been documented; most commonly, the second pathology is focal cortical dysplasia or less frequently, hippocampal sclerosis. To our knowledge, cases of arteriovenous malformation arising in this clinical setting and associated with hippocampal sclerosis have not been previously described. We report a 56-year-old woman who initially presented at age 24years with staring spells. Imaging studies revealed an arteriovenous malformation in the right parietal lobe. At age 51years, she represented with signs and symptoms related to a hemorrhage from the malformation. The patient underwent Gamma Knife radiosurgery (Elekta AB, Stockholm, Sweden) of the lesion. She subsequently developed seizures, refractory to medical management. MRI studies showed atrophy in the right hippocampus. She underwent resection of the right parietal lobe and hippocampus. Histopathologic examination of the right parietal lesion revealed an arteriovenous malformation marked by focally prominent vascular sclerosis, calcification and adjacent hemosiderin deposition. The hippocampus was marked by prominent neuronal loss and gliosis in the CA1 region, consistent with CA1 sclerosis or hippocampal sclerosis International League Against Epilepsy type 2. PMID:26899356

  11. Mechanisms underlying epileptogenesis in cortical malformations.

    PubMed

    Jacobs, K M; Kharazia, V N; Prince, D A

    1999-09-01

    The presence of developmental cortical malformations is associated with epileptogenesis and other neurological disorders. In recent years, animal models specific to certain malformations have been developed to study the underlying epileptogenic mechanisms. Teratogens (chemical, thermal or radiation) applied during cortical neuroblast division and migration result in lissencephaly and focal cortical dysplasia. Animals with these malformations have a lowered seizure threshold as well as histopathologies typical of those found in human dysgenic brains. Alterations that may promote epileptogenesis have been identified in lissencephalic brains, such as increased numbers of bursting types of neurons, and abnormal connections between hippocampus, subcortical heterotopia, and neocortex. A distinct set of pathological properties is present in animal models of 4-layered microgyria, induced with cortical lesions made during late stages of cortical neuroblast migration. Hyperexcitability has been demonstrated in cortex adjacent to the microgyrus (paramicrogyral zone) in in vitro slice preparations. A number of observations suggest that cellular differentiation is delayed in microgyric brains. Other studies show increases in postsynaptic glutamate receptors and decreases in GABA(A) receptors in microgyric cortex. These alterations could promote epileptogenesis, depending on which cell types have the altered receptors. The microgyrus lacks thalamic afferents from sensory relay nuclei, that instead appear to project to the paramicrogyral region, thereby increasing excitatory connectivity within this epileptogenic zone. These studies have provided a necessary first step in understanding molecular and cellular mechanisms of epileptogenesis associated with cortical malformations. PMID:10515164

  12. Genetic basis of congenital cardiovascular malformations

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Cardiovascular malformations are a singularly important class of birth defects and due to dramatic improvements in medical and surgical care, there are now large numbers of adult survivors. The etiologies are complex, but there is strong evidence that genetic factors play a crucial role. Over the la...

  13. Arteriovenous malformation in hypomelanosis of Ito.

    PubMed

    Urgelles, E; Pascual-Castroviejo, I; Roche, C; Moneo, J L; Martinez, M A; Vega, A

    1996-01-01

    Hypomelanosis of Ito (HI) is a neurocutaneous syndrome with multisystemic involvement. Its most frequent neurological abnormalities are mental retardation and seizures. EEG, CT and MRI findings are not characteristic enough to be diagnostic. In this report, we describe a patient with typical cutaneous lesions of HI and an intracranial arteriovenous malformation (AVM), which has not been previously reported. PMID:8907350

  14. Numerical analysis of ossicular chain lesion of human ear

    NASA Astrophysics Data System (ADS)

    Liu, Yingxi; Li, Sheng; Sun, Xiuzhen

    2009-04-01

    Lesion of ossicular chain is a common ear disease impairing the sense of hearing. A comprehensive numerical model of human ear can provide better understanding of sound transmission. In this study, we propose a three-dimensional finite element model of human ear that incorporates the canal, tympanic membrane, ossicular bones, middle ear suspensory ligaments/muscles, middle ear cavity and inner ear fluid. Numerical analysis is conducted and employed to predict the effects of middle ear cavity, malleus handle defect, hypoplasia of the long process of incus, and stapedial crus defect on sound transmission. The present finite element model is shown to be reasonable in predicting the ossicular mechanics of human ear.

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

    PubMed Central

    Merchant, Gabrielle R.; Horton, Nicholas J.

    2011-01-01

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

  16. Ear cleaning: the UK and US perspective.

    PubMed

    Nuttall, Tim; Cole, Lynette K

    2004-04-01

    Ear cleaning helps maintain the normal otic environment and is important in the treatment of otitis. Over cleaning, however, may trigger otitis through maceration of the epidermal lining. Simple manual cleaning is useful for routine cleansing but doesn't remove tightly adherent debris. Bulb syringes are more vigorous but may damage the ear in inexperienced hands. Devices using mains water pressure or dental machines are also available. Thorough cleaning of the ear canals and middle ear cavity can only be achieved by retrograde flushing using specially adapted catheters, feeding tubes or video otoscopes under anaesthesia. Myringotomy, inspection and cleaning of the middle should be performed if the tympanic membrane appears abnormal. There are a wide variety of cleaning fluids available. Ceruminolytics soften and dissolve cerumen to facilitate cleaning. Surfactants emulsify debris, breaking it up and keeping it in solution. Astringents dry the ear canal surface, preventing maceration. Maintaining a low pH and incorporating antimicrobial agents can inhibit microbial proliferation and glucocorticoids can be used to reduce inflammation. Adverse effects and contraindications following ear cleaning can include maceration, contact reactions, otitis media, ear canal avulsion, vestibular syndrome, Horner's syndrome, facial nerve paralysis and deafness. Care should be exercised in selecting cleaning fluids if the tympanic membranes are ruptured. PMID:15030561

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

    PubMed Central

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

    2010-01-01

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

  18. Genetic animal models of malformations of cortical development and epilepsy.

    PubMed

    Wong, Michael; Roper, Steven N

    2016-02-15

    Malformations of cortical development constitute a variety of pathological brain abnormalities that commonly cause severe, medically-refractory epilepsy, including focal lesions, such as focal cortical dysplasia, heterotopias, and tubers of tuberous sclerosis complex, and diffuse malformations, such as lissencephaly. Although some cortical malformations result from environmental insults during cortical development in utero, genetic factors are increasingly recognized as primary pathogenic factors across the entire spectrum of malformations. Genes implicated in causing different cortical malformations are involved in a variety of physiological functions, but many are focused on regulation of cell proliferation, differentiation, and neuronal migration. Advances in molecular genetic methods have allowed the engineering of increasingly sophisticated animal models of cortical malformations and associated epilepsy. These animal models have identified some common mechanistic themes shared by a number of different cortical malformations, but also revealed the diversity and complexity of cellular and molecular mechanisms that lead to the development of the pathological lesions and resulting epileptogenesis. PMID:25911067

  19. Gouty tophi on the ear: a review.

    PubMed

    Chabra, Indy; Singh, Rajendra

    2013-10-01

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

  20. Surgical Management of Ear Diseases in Rabbits.

    PubMed

    Csomos, Rebecca; Bosscher, Georgia; Mans, Christoph; Hardie, Robert

    2016-01-01

    Otitis externa and media are frequently diagnosed disorders in rabbits and are particularly common in lop-eared breeds because of the specific anatomy of the ear canal. Medical management for otitis externa and media often provides only a temporary improvement in clinical signs. Surgery by means of partial or total ear canal ablation (PECA or TECA) combined with lateral bulla osteotomy (LBO) represents a feasible approach that is well tolerated and provides a good clinical outcome. Short-term complications associated with PECA/TECA-LBO include facial nerve paralysis and vestibular disease. PMID:26611929

  1. [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

  2. Directional Sensitivity of the Human Ear.

    ERIC Educational Resources Information Center

    Pitt, John D.; Bazley, Martin

    1985-01-01

    Presents a classroom experiment that demonstrates the directional sensitivity of the human ear. Outlines the activity's procedures and provides a diagrammatical view of the experimental arrangement. Also included is an analysis of the expected results. (ML)

  3. Ear, Nose & Throat Issues & Down Syndrome

    MedlinePlus

    ... Series Videos Webinar Series Health Care Associated Conditions ADHD & Down Syndrome Alzheimer's Disease & Down Syndrome Anesthesia & Down Syndrome Atlantoaxial Instability & Down Syndrome Blood Diseases & Down Syndrome Dental Issues & Down Syndrome Dual Diagnosis of Down Syndrome & Autism Ear, Nose & Throat Issues & ...

  4. Neonatal Ear Molding: Timing and Technique.

    PubMed

    Anstadt, Erin Elizabeth; Johns, Dana Nicole; Kwok, Alvin Chi-Ming; Siddiqi, Faizi; Gociman, Barbu

    2016-03-01

    The incidence of auricular deformities is believed to be ∼11.5 per 10 000 births, excluding children with microtia. Although not life-threatening, auricular deformities can cause undue distress for patients and their families. Although surgical procedures have traditionally been used to reconstruct congenital auricular deformities, ear molding has been gaining acceptance as an efficacious, noninvasive alternative for the treatment of newborns with ear deformations. We present the successful correction of bilateral Stahl's ear deformity in a newborn through a straightforward, nonsurgical method implemented on the first day of life. The aim of this report is to make pediatric practitioners aware of an effective and simple molding technique appropriate for correction of congenital auricular anomalies. In addition, it stresses the importance of very early initiation of ear cartilage molding for achieving the desired outcome. PMID:26908661

  5. [Middle ear effusion and allergy in childhood].

    PubMed

    Bachert, C; Keilmann, A; Ganzer, U

    1989-04-01

    Besides mechanical or infectious factors in the genesis of secretory otitis media IgE-mediated hypersensitivity has often been discussed. In order to estimate the importance of an allergic reaction in the development of middle ear disease, we examined 27 children having adenoids and a secretory otitis media. We documented the patients' history of allergy and determined the IgE-levels in the serum, the middle ear effusions and the adenoid tissue. 5 out of 27 children suffered from an allergy of the upper respiratory tract, but only in one child we found an elevated level of IgE in middle ear effusion. On the basis of our investigations and recent literature, we suggest that an allergic rhinitis might cause an Eustachian tube dysfunction in a few patients. There is no evidence of a local allergic reaction of the middle ear mucosa. PMID:2742640

  6. 14 CFR 67.105 - Ear, nose, throat, and equilibrium.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... (CONTINUED) AIRMEN MEDICAL STANDARDS AND CERTIFICATION First-Class Airman Medical Certificate § 67.105 Ear, nose, throat, and equilibrium. Ear, nose, throat, and equilibrium standards for a first-class airman... Better ear (Db) 35 30 30 40 Poorer ear (Db) 35 50 50 60 (b) No disease or condition of the middle...

  7. 21 CFR 874.3430 - Middle ear mold.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 21 Food and Drugs 8 2010-04-01 2010-04-01 false Middle ear mold. 874.3430 Section 874.3430 Food... DEVICES EAR, NOSE, AND THROAT DEVICES Prosthetic Devices § 874.3430 Middle ear mold. (a) Identification. A middle ear mold is a preformed device that is intended to be implanted to reconstruct the middle...

  8. 21 CFR 874.4140 - Ear, nose, and throat bur.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ... 21 Food and Drugs 8 2014-04-01 2014-04-01 false Ear, nose, and throat bur. 874.4140 Section 874...) MEDICAL DEVICES EAR, NOSE, AND THROAT DEVICES Surgical Devices § 874.4140 Ear, nose, and throat bur. (a) Identification. An ear, nose, and throat bur is a device consisting of an interchangeable drill bit that...

  9. 38 CFR 4.87 - Schedule of ratings-ear.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 1 2013-07-01 2013-07-01 false Schedule of ratings-ear...—ear. Diseases of the Ear Rating 6200Chronic suppurative otitis media, mastoiditis, or cholesteatoma... of the substance 10 6208Malignant neoplasm of the ear (other than skin only) 100 Note: A rating...

  10. 38 CFR 4.87 - Schedule of ratings-ear.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 1 2011-07-01 2011-07-01 false Schedule of ratings-ear...—ear. Diseases of the Ear Rating 6200Chronic suppurative otitis media, mastoiditis, or cholesteatoma... of the substance 10 6208Malignant neoplasm of the ear (other than skin only) 100 Note: A rating...

  11. 38 CFR 4.87 - Schedule of ratings-ear.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 1 2010-07-01 2010-07-01 false Schedule of ratings-ear...—ear. Diseases of the Ear Rating 6200Chronic suppurative otitis media, mastoiditis, or cholesteatoma... of the substance 10 6208Malignant neoplasm of the ear (other than skin only) 100 Note: A rating...

  12. 21 CFR 874.4140 - Ear, nose, and throat bur.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ... 21 Food and Drugs 8 2012-04-01 2012-04-01 false Ear, nose, and throat bur. 874.4140 Section 874...) MEDICAL DEVICES EAR, NOSE, AND THROAT DEVICES Surgical Devices § 874.4140 Ear, nose, and throat bur. (a) Identification. An ear, nose, and throat bur is a device consisting of an interchangeable drill bit that...

  13. 21 CFR 874.3430 - Middle ear mold.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ... 21 Food and Drugs 8 2013-04-01 2013-04-01 false Middle ear mold. 874.3430 Section 874.3430 Food... DEVICES EAR, NOSE, AND THROAT DEVICES Prosthetic Devices § 874.3430 Middle ear mold. (a) Identification. A middle ear mold is a preformed device that is intended to be implanted to reconstruct the middle...

  14. 38 CFR 4.87 - Schedule of ratings-ear.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 1 2012-07-01 2012-07-01 false Schedule of ratings-ear...—ear. Diseases of the Ear Rating 6200Chronic suppurative otitis media, mastoiditis, or cholesteatoma... of the substance 10 6208Malignant neoplasm of the ear (other than skin only) 100 Note: A rating...

  15. 21 CFR 874.3430 - Middle ear mold.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ... 21 Food and Drugs 8 2012-04-01 2012-04-01 false Middle ear mold. 874.3430 Section 874.3430 Food... DEVICES EAR, NOSE, AND THROAT DEVICES Prosthetic Devices § 874.3430 Middle ear mold. (a) Identification. A middle ear mold is a preformed device that is intended to be implanted to reconstruct the middle...

  16. 38 CFR 4.87 - Schedule of ratings-ear.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 1 2014-07-01 2014-07-01 false Schedule of ratings-ear...—ear. Diseases of the Ear Rating 6200Chronic suppurative otitis media, mastoiditis, or cholesteatoma... of the substance 10 6208Malignant neoplasm of the ear (other than skin only) 100 Note: A rating...

  17. 21 CFR 874.3430 - Middle ear mold.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ... 21 Food and Drugs 8 2014-04-01 2014-04-01 false Middle ear mold. 874.3430 Section 874.3430 Food... DEVICES EAR, NOSE, AND THROAT DEVICES Prosthetic Devices § 874.3430 Middle ear mold. (a) Identification. A middle ear mold is a preformed device that is intended to be implanted to reconstruct the middle...

  18. 21 CFR 874.4140 - Ear, nose, and throat bur.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ... 21 Food and Drugs 8 2013-04-01 2013-04-01 false Ear, nose, and throat bur. 874.4140 Section 874...) MEDICAL DEVICES EAR, NOSE, AND THROAT DEVICES Surgical Devices § 874.4140 Ear, nose, and throat bur. (a) Identification. An ear, nose, and throat bur is a device consisting of an interchangeable drill bit that...

  19. FTO variant associated with malformation syndrome.

    PubMed

    Rohena, Luis; Lawson, Michelle; Guzman, Edwin; Ganapathi, Mythily; Cho, Megan T; Haverfield, Eden; Anyane-Yeboa, Kwame

    2016-04-01

    Common FTO variants are associated with obesity. However, it has recently been shown that homozygous FTO c.947G>A variant, which predicts p.R316Q, and c.956C>T, which predicts p.S319F, are associated with a malformation syndrome inherited in an autosomal recessive pattern. We present a similar homozygous FTO c.965G>A variant that predicts p.R322Q, associated with a lethal malformation syndrome in a consanguineous Yemeni family. Functional studies showed that the p.R316Q, p.S219F, and p.R322Q variants render the FTO protein inactive. We further expand on the phenotype of homozygous FTO loss-of-function mutations to include eye abnormalities, gingival overgrowth, craniosynostosis, and cutaneous photosensitivity. © 2015 Wiley Periodicals, Inc. PMID:26697951

  20. Animal Models in Studying Cerebral Arteriovenous Malformation

    PubMed Central

    Xu, Ming; Xu, Hongzhi; Qin, Zhiyong

    2015-01-01

    Brain arteriovenous malformation (AVM) is an important cause of hemorrhagic stroke. The etiology is largely unknown and the therapeutics are controversial. A review of AVM-associated animal models may be helpful in order to understand the up-to-date knowledge and promote further research about the disease. We searched PubMed till December 31, 2014, with the term “arteriovenous malformation,” limiting results to animals and English language. Publications that described creations of AVM animal models or investigated AVM-related mechanisms and treatments using these models were reviewed. More than 100 articles fulfilling our inclusion criteria were identified, and from them eight different types of the original models were summarized. The backgrounds and procedures of these models, their applications, and research findings were demonstrated. Animal models are useful in studying the pathogenesis of AVM formation, growth, and rupture, as well as in developing and testing new treatments. Creations of preferable models are expected. PMID:26649296

  1. Animal Models in Studying Cerebral Arteriovenous Malformation.

    PubMed

    Xu, Ming; Xu, Hongzhi; Qin, Zhiyong

    2015-01-01

    Brain arteriovenous malformation (AVM) is an important cause of hemorrhagic stroke. The etiology is largely unknown and the therapeutics are controversial. A review of AVM-associated animal models may be helpful in order to understand the up-to-date knowledge and promote further research about the disease. We searched PubMed till December 31, 2014, with the term "arteriovenous malformation," limiting results to animals and English language. Publications that described creations of AVM animal models or investigated AVM-related mechanisms and treatments using these models were reviewed. More than 100 articles fulfilling our inclusion criteria were identified, and from them eight different types of the original models were summarized. The backgrounds and procedures of these models, their applications, and research findings were demonstrated. Animal models are useful in studying the pathogenesis of AVM formation, growth, and rupture, as well as in developing and testing new treatments. Creations of preferable models are expected. PMID:26649296

  2. Arteriovenous malformation of the uterine cervix.

    PubMed

    Val-Bernal, José-Fernando; Hermana, Sandra

    2016-03-01

    A uterine arteriovenous malformation (AVM) is an uncommon cause of uterine bleeding. Location of this lesion in the uterine cervix is exceptional. We report a case of a 34-year-old woman who presented with chronic menorrhagias and hypochromic anemia. A sonographic study revealed a 10-cm, fundal, intramural, uterine well-circumscribed mass that distorted the endometrial cavity. The patient underwent hysterectomy for a large uterine leiomyoma. The pathological study revealed an incidental AVM of the posterior half of the cervix measuring 5.5cm in major diameter. We suggest that in our case cervical AVM might have occurred due to a large corporal leiomyoma distorting the uterine circulation. Differential diagnosis includes capillary hemangioma, venous malformation, or arteriovenous fistula. PMID:26810780

  3. Congenital cystic adenomatoid malformation of the lung

    PubMed Central

    Moncreieff, M. W.; Cameron, A. H.; Astley, R.; Roberts, K. D.; Abrams, L. D.; Mann, J. R.

    1969-01-01

    Nine cases of congenital cystic adenomatoid malformation of the lung are described. One was stillborn: two presented in the newborn period (one of them surviving after a lobectomy), and the remaining six were older children all of whom survived after lobectomy. There have been only three cases previously reported in children outside the newborn period. The pathological, clinical, and radiological features are discussed and compared with previously reported cases. Images PMID:5815941

  4. Magnetic Resonance Imaging of Malformations of Midbrain-Hindbrain.

    PubMed

    Abdel Razek, Ahmed Abdel Khalek; Castillo, Mauricio

    2016-01-01

    We aim to review the magnetic resonance imaging appearance of malformations of midbrain and hindbrain. These can be classified as predominantly cerebellar malformations, combined cerebellar and brain stem malformations, and predominantly brain stem malformations. The diagnostic criteria for the majority of these morphological malformations are based on neuroimaging findings. The predominantly cerebellar malformations include predominantly vermian hypoplasia seen in Dandy-Walker malformation and rhombencephalosynapsis, global cerebellar hypoplasia reported in lissencephaly and microlissencephaly, and unilateral cerebellar hypoplasia seen in PHACES, vanishing cerebellum, and cerebellar cleft. Cerebellar dysplasias are seen in Chudley-McCullough syndrome, associated with LAMA1 mutations and GPR56 mutations; Lhermitte-Duclos disease; and focal cerebellar dysplasias. Cerebellar hyperplasias are seen in megalencephaly-related syndromes and hemimegalencephaly with ipsilateral cerebellomegaly. Cerebellar and brain stem malformations include tubulinopathies, Joubert syndrome, cobblestone malformations, pontocerebellar hypoplasias, and congenital disorders of glycosylation type Ia. Predominantly brain stem malformations include congenital innervation dysgenesis syndrome, pontine tegmental cap dysplasia, diencephalic-mesencephalic junction dysplasia, disconnection syndrome, and pontine clefts. PMID:26599961

  5. Middle ear muscles of the frog.

    PubMed Central

    Wever, E G

    1979-01-01

    The anuran middle ear in its complete form includes two skeletal elements, the columella and operculum, each occupying a portion of the oval window of the otic capsule and each provided with a middle ear muscle. The two elements have an interlocking arrangement of a form that makes it possible for these muscles to exercise a high degree of control of sound transmission from tympanic membrane to inner ear receptors. From the anatomical relations it is inferred that the two muscles operate as antagonists so that contraction of the opercular muscle and relaxation of the columellar muscle leave the columella free to move in and out of the oval window in response to sound vibrations, whereas a contraction of the columellar muscle and relaxation of the opercular muscle tend to immobilize the columella and reduce the transmission inward. The frog thus achieves a degree of control of sound reception that probably is unmatched among vertebrate ears. The purpose of the middle ear mechanism is no doubt the protection of the inner ear receptors (the amphibian and basilar papillae) from overstimulation by sounds, including the animal's own cries and the intense clamor produced by a group of frogs calling in chorus. Images PMID:288086

  6. Combined Lymphedema and Capillary Malformation of the Lower Extremity

    PubMed Central

    Maclellan, Reid A.; Chaudry, Gulraiz

    2016-01-01

    Background: Primary lymphedema and capillary malformation are independent vascular malformations that can cause overgrowth of the lower extremity. We report a series of patients who had both types of malformations affecting the same leg. The condition is unique but may be confused with other types of vascular malformation overgrowth conditions (eg, Klippel–Trenaunay and Parkes Weber). Methods: Our Vascular Anomalies Center and Lymphedema Program databases were searched for patients with both capillary malformation and lymphedema. Diagnosis of lymphedema–capillary malformation was made by history, physical examination, and imaging studies. Because lymphedema–capillary malformation has phenotypical overlap with other conditions, only patients who had imaging confirming their diagnosis were included in the analysis. Clinical and radiological features, morbidity, and treatment were recorded. Results: Eight patients (4 females and 4 males) had confirmed lymphedema–capillary malformation. Referring diagnosis was Klippel–Trenaunay syndrome (n = 4), diffuse capillary malformation with overgrowth (n = 3), or lymphatic malformation (n = 1). The condition was unilateral (n = 6) or bilateral (n = 2). Morbidity included infection (n = 6), difficulty fitting clothes (n = 6), bleeding or leaking vesicles (n = 5), leg length discrepancy (n = 4), and difficulty ambulating (n = 3). All patients were managed with compression regimens. Operative management was liposuction (n = 3), treatment of phlebectatic veins (n = 3), staged skin/subcutaneous excision (n = 1), and/or epiphysiodesis (n = 1). Conclusions: Lymphedema and capillary malformation can occur together in the same extremity. Both conditions independently cause limb overgrowth primarily because of subcutaneous adipose deposition. Compression garments and suction-assisted lipectomy can improve the condition. Lymphedema–capillary malformation should not be confused with other vascular malformation overgrowth diseases that have different morbidities and treatments. PMID:27014547

  7. Congenital lung malformations: an ongoing controversy

    PubMed Central

    Burge, DM; Marven, SS

    2013-01-01

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

  8. Predictions of middle-ear and passive cochlear mechanics using a finite element model of the pediatric ear.

    PubMed

    Wang, Xuelin; Keefe, Douglas H; Gan, Rong Z

    2016-04-01

    A finite element (FE) model was developed based on histological sections of a temporal bone of a 4-year-old child to simulate middle-ear and cochlear function in ears with normal hearing and otitis media. This pediatric model of the normal ear, consisting of an ear canal, middle ear, and spiral cochlea, was first validated with published energy absorbance (EA) measurements in young children with normal ears. The model was used to simulate EA in an ear with middle-ear effusion, whose results were compared to clinical EA measurements. The spiral cochlea component of the model was constructed under the assumption that the mechanics were passive. The FE model predicted middle-ear transfer functions between the ear canal and cochlea. Effects of ear structure and mechanical properties of soft tissues were compared in model predictions for the pediatric and adult ears. EA responses are predicted to differ between adult and pediatric ears due to differences in the stiffness and damping of soft tissues within the ear, and any residual geometrical differences between the adult ear and pediatric ear at age 4 years. The results have significance for predicting effects of otitis media in children. PMID:27106321

  9. Evaluation and management of peripheral venous and lymphatic malformations.

    PubMed

    Nassiri, Naiem; Thomas, Jones; Cirillo-Penn, Nolan C

    2016-04-01

    The International Society for Study of Vascular Anomalies (ISSVA) broadly categorizes vascular anomalies as vascular tumors or vascular malformations. The latter are congenital lesions that are further categorized by their flow properties and include high-flow arteriovenous malformations, slow-flow venous and lymphatic malformations, and congenital mixed syndromes, which can include a combination of malformations. Unlike vascular tumors, vascular malformations never regress and can persist and grow for the duration of the patient's lifespan. As our understanding of the natural history, hemodynamics, and treatment outcomes of these lesions has expanded and evolved over the last few decades, certain fundamental diagnostic and therapeutic principles have been established and are considered standard of care. These overarching principles are crucial to adhere to in the overall management of these lesions and are highlighted and expanded on in this report, which focuses exclusively on peripheral slow-flow venous and lymphatic malformations. PMID:26993876

  10. 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

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

    PubMed Central

    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

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

    PubMed

    Mason, Matthew J

    2016-02-01

    Many species of small desert mammals are known to have expanded auditory bullae. The ears of gerbils and heteromyids have been well described, but much less is known about the middle ear anatomy of other desert mammals. In this study, the middle ears of three gerbils (Meriones, Desmodillus and Gerbillurus), two jerboas (Jaculus) and two sengis (elephant-shrews: Macroscelides and Elephantulus) were examined and compared, using micro-computed tomography and light microscopy. Middle ear cavity expansion has occurred in members of all three groups, apparently in association with an essentially 'freely mobile' ossicular morphology and the development of bony tubes for the middle ear arteries. Cavity expansion can occur in different ways, resulting in different subcavity patterns even between different species of gerbils. Having enlarged middle ear cavities aids low-frequency audition, and several adaptive advantages of low-frequency hearing to small desert mammals have been proposed. However, while Macroscelides was found here to have middle ear cavities so large that together they exceed brain volume, the bullae of Elephantulus are considerably smaller. Why middle ear cavities are enlarged in some desert species but not others remains unclear, but it may relate to microhabitat. PMID:26104342

  13. Comparison of ear tattoo, ear notching and microtattoo in rats undergoing cardiovascular telemetry.

    PubMed

    Kasanen, I H E; Voipio, H-M; Leskinen, H; Luodonpää, M; Nevalainen, T O

    2011-07-01

    Individual and permanent identification of experimental animals is a common and often essential research practice. There is little information available on the short-term effects of these procedures on the animals. In this study, seven rats were implanted with telemetric devices. The effects of three different identification methods (ear tattoo, ear notching and microtattoo) were compared. Cardiovascular data were collected for 24 h after the procedures. Time periods of 0-1, 1-4, 4-16 h (dark) and 16-24 h after the procedure were analysed separately. The most pronounced differences in measured parameters were observed during the first hour after the procedures were performed. Mean arterial pressure (MAP) was significantly higher (P < 0.012) following the ear tattoo than the microtattoo procedure by a difference of approximately 5 mmHg. Heart rate (HR) was significantly elevated (P < 0.001) after ear tattoo compared with both ear notching (Δ = 31 beats per minute [bpm]) and microtattoo (Δ = 44 bpm). During the 1-4 h period and the following dark period, the MAP was highest in the ear notching group, but no differences were observed in the HRs. During the following dark period (4-16 h) and the next day (16-24 h) differences in MAP and HR were minor. In conclusion, microtattoo appears to cause the mildest changes in HR and blood pressure. Based on these results, ear tattoo and ear notching should be replaced by microtattoo whenever possible. PMID:21504993

  14. Hemangiomas and Vascular Malformations: Current Theory and Management

    PubMed Central

    Richter, Gresham T.; Friedman, Adva B.

    2012-01-01

    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 tumor. Lymphatic, capillary, venous, and arteriovenous malformations make up the majority of vascular malformations. This paper reviews current theory and practice in the etiology, diagnosis, and treatment of these more common vascular anomalies. PMID:22611412

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

    PubMed

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

    2016-01-01

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

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

    PubMed Central

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

    2016-01-01

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

  17. 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

  18. 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

  19. Sciaenid inner ears: a study in diversity.

    PubMed

    Ramcharitar, J; Higgs, D M; Popper, A N

    2001-01-01

    Sciaenid fishes (Family Sciaenidae) could potentially serve as models for understanding the relationship between structure and function in the teleost auditory system, as they show a broad range of variation in not only the structure of the ear but also in the relationship between the ear and swim bladder. In this study, scanning electron microscopy (SEM) was used to investigate inner ear ultrastructure of the Atlantic croaker (Micropogonias undulatus), spotted seatrout (Cynoscion nebulosus), kingfish (Menticirrhus americanus) and spot (Leiostomus xanthurus). These species reflect the diversity of otolith and swim bladder morphology in sciaenids. The distribution of different hair cell bundle types, as well as hair cell orientation patterns on the saccular and lagenar maculae of these fishes were similar to one another. The rostral ends of the saccular sensory epithelia (maculae) were highly expanded in a dorsal-ventral direction in the Atlantic croaker and spotted seatrout as compared to the kingfish and spot. Also, ciliary bundles of the saccular maculae contained more stereocilia in the Atlantic croaker and spotted seatrout as compared with kingfish and spot. The shapes of the lagenar maculae were similar in all four species. In the Atlantic croaker and spotted seatrout lagenar maculae, the number of stereocilia per bundle was greater than those for the kingfish and spot. Given that saccular macula shape and numbers of stereocilia per bundle correlate with swim bladder proximity to the ear in the studied species, it is possible that inner ear ultrastructure could be indicative of auditory ability in fishes. PMID:11910172

  20. Assessment of Ear Disorders Using Power Reflectance

    PubMed Central

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

    2013-01-01

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

  1. 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

  2. Evolution and development of the vertebrate ear

    NASA Technical Reports Server (NTRS)

    Fritzsch, B.; Beisel, K. W.

    2001-01-01

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

  3. Malformations of the tooth root in humans

    PubMed Central

    Luder, Hans U.

    2015-01-01

    The most common root malformations in humans arise from either developmental disorders of the root alone or disorders of radicular development as part of a general tooth dysplasia. The aim of this review is to relate the characteristics of these root malformations to potentially disrupted processes involved in radicular morphogenesis. Radicular morphogenesis proceeds under the control of Hertwig's epithelial root sheath (HERS) which determines the number, length, and shape of the root, induces the formation of radicular dentin, and participates in the development of root cementum. Formation of HERS at the transition from crown to root development appears to be very insensitive to adverse effects, with the result that rootless teeth are extremely rare. In contrast, shortened roots as a consequence of impaired or prematurely halted apical growth of HERS constitute the most prevalent radicular dysplasia which occurs due to trauma and unknown reasons as well as in association with dentin disorders. While odontoblast differentiation inevitably stops when growth of HERS is arrested, it seems to be unaffected even in cases of severe dentin dysplasias such as regional odontodysplasia and dentin dysplasia type I. As a result radicular dentin formation is at least initiated and progresses for a limited time. The only condition affecting cementogenesis is hypophosphatasia which disrupts the formation of acellular cementum through an inhibition of mineralization. A process particularly susceptible to adverse effects appears to be the formation of the furcation in multirooted teeth. Impairment or disruption of this process entails taurodontism, single-rooted posterior teeth, and misshapen furcations. Thus, even though many characteristics of human root malformations can be related to disorders of specific processes involved in radicular morphogenesis, precise inferences as to the pathogenesis of these dysplasias are hampered by the still limited knowledge on root formation. PMID:26578979

  4. 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

  5. Laser treatment of oral vascular malformations

    NASA Astrophysics Data System (ADS)

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

    2014-01-01

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

  6. Capillary MalformationArteriovenous 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 malformationarteriovenous 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

  7. [Early stage of a cloverleaf skull malformation].

    PubMed

    Fischer, G; Hori, A; Ulbrich, R; Rath, W

    1982-12-01

    Cloverleaf skull anomaly was diagnosed sonographically and in the fetogram, together with concomitant chondrodystrophy. This resulted in an indication for intentional abortion in the 29th week. Consequently, this rare form of skull monstrosity could be examined pathologico-anatomically for the first time in a very early stage of foetal development. Contrary to the widely held opinion that the reason for such hideous malformation is a hydrocephalus internus due to a deformation of the skull base, we found a practically negligible hydrocephalus, although the cloverleaf skull had already developed in a very marked manner. Hence, this case contradicts the generally adopted formal pathogenetic interpretation of cloverleaf skull monstrosity. PMID:7178767

  8. 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

  9. Intrapulmonary arteriovenous malformation causing recurrent strokes

    PubMed Central

    Abed, Kareem; Premachandra, Lalith; Vankawala, Viren; Sun, Qi

    2015-01-01

    This case reveals a left pulmonary arteriovenous malformation (PAVM) as a cause of recurrent cerebral and cerebellar emboli. Extensive workup excluded other etiologies of emboli formation, and the patient was transferred to a tertiary care center for percutaneous embolotherapy. In the absence of a clear etiology, PAVM should be considered as a potential cause of recurrent cerebral emboli, especially in the absence of carotid disease, intracardiac thrombus, atrial septal defect, and patent foramen ovale. Diagnostic work-up for the PAVM can be cost effective and expedited by utilization of agitated saline contrast echocardiography, as noted in our case. PMID:26486114

  10. Toward postnatal reversal of ocular congenital malformations

    PubMed Central

    Sahel, José-Alain; Marazova, Katia

    2013-01-01

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

  11. Spinal arteriovenous malformation masquerating zoster sine herpete.

    PubMed

    Lee, Ji Young; Ok, Se Jin; Oh, Chang Keun; Park, Sun Kyung; Kim, Do Wan; Yang, Jong Yeun

    2013-01-01

    Zoster sine herpete (ZSH) is difficult to diagnosis during an acute period due to the absence of the characteristic zosteriform dermatomal rash; therefore, progression to postherpetic neuralgia is more common than typical zoster. In addition, misdiagnosis of other neuropathic pain as ZSH is common in clinical situations. Here, we report a case of spinal arteriovenous malformation that mimics ZSH. This is a rare condition; therefore, high clinical suspicion for a correct diagnosis and proper examination are not easy. However, early diagnosis and definitive treatment are essential to prevent neurologic deficit and mortality. PMID:23342212

  12. Spinal Arteriovenous Malformation Masquerating Zoster Sine Herpete

    PubMed Central

    Lee, Ji Young; Ok, Se Jin; Oh, Chang Keun; Park, Sun Kyung; Kim, Do Wan

    2013-01-01

    Zoster sine herpete (ZSH) is difficult to diagnosis during an acute period due to the absence of the characteristic zosteriform dermatomal rash; therefore, progression to postherpetic neuralgia is more common than typical zoster. In addition, misdiagnosis of other neuropathic pain as ZSH is common in clinical situations. Here, we report a case of spinal arteriovenous malformation that mimics ZSH. This is a rare condition; therefore, high clinical suspicion for a correct diagnosis and proper examination are not easy. However, early diagnosis and definitive treatment are essential to prevent neurologic deficit and mortality. PMID:23342212

  13. Genetic and Developmental Basis of Cardiovascular Malformations.

    PubMed

    Azhar, Mohamad; Ware, Stephanie M

    2016-03-01

    Cardiovascular malformations (CVMs) are the most common birth defect, occurring in 1% to 5% of all live births. Genetic, epigenetic, and environmental factors all influence the development of CVMs, and an improved understanding of the causation of CVMs is a prerequisite for prevention. Cardiac development is a complex, multistep process of morphogenesis that is under genetic regulation. Although the genetic contribution to CVMs is well recognized, the genetic causes of human CVMs are still identified infrequently. This article discusses the key genetic concepts characterizing human CVMs, their developmental basis, and the critical developmental and genetic concepts underlying their pathogenesis. PMID:26876120

  14. "Petrified Ears" in Secondary Adrenal Insufficiency.

    PubMed

    Goswami, Soumik; Chakraborty, Partha Pratim; Ray, Sayantan; Ghosh, Sujoy; Chowdhury, Subhankar

    2016-02-01

    Petrification of the auricle, a rarely encountered clinical entity usually results from ectopic calcification of the auricular cartilages and manifests as rigid ear. The underlying pathogenesis remains ambiguous with several proposed hypotheses till date. Auricular calcification may be the sole cutaneous marker of underlying endocrinopathy at times. Adrenal insufficiency is the most common endocrinological disorder to be associated with such stiff ears and it has been described in both primary as well as secondary forms of the disease. We present here a 30-year-old man whose clinical condition deteriorated following levothyroxine supplementation and the presence of "petrified ears" ultimately provided a clue to the diagnosis of associated secondary adrenal insufficiency. PMID:27042511

  15. [Bone Conduction and Active Middle Ear Implants].

    PubMed

    Volkenstein, S; Thomas, J P; Dazert, S

    2016-05-01

    The majority of patients with moderate to severe hearing loss can be supplied with conventional hearing aids depending on severity and cause for hearing loss in a satisfying way. However, some patients either do not benefit enough from conventional hearing aids or cannot wear them due to inflammatory reactions and chronic infections of the external auditory canal or due to anatomical reasons. For these patients there are fully- and semi-implantable middle ear and bone conduction implants available. These devices either directly stimulate the skull (bone conduction devices), middle ear structures (active middle ear implants) or the cochlea itself (direct acoustic stimulation). Patients who failed surgical hearing rehabilitation or do not benefit from conventional hearing aids may achieve a significant better speech understanding and tremendous improvement in quality of life by implantable hearing devices with careful attention to the audiological and anatomical indication criteria. PMID:27135430

  16. Middle ear gas exchange in isobaric counterdiffusion.

    PubMed

    Dueker, C W; Lambertsen, C J; Rosowski, J J; Saunders, J C

    1979-12-01

    Nitrous oxide entry into the middle ear gas space was studied in cats in relation to anesthesia and the vestibular dysfunction caused by isobaric inert gas counter-diffusion in diving. A catheter implanted in the auditory bulla was used for direct gas sampling and pressure measurements. Experiments were designed to evaluate the participation of the eustachian tube, mucosal blood vessels, and tympanic membrane in middle ear gas exchange. The eustachian tube did not contribute to N2O entry and the mucosal blood supply only contributed about one-third of the total N2O accumulation. Diffusion across the tympanic membrane accounted for most of the N2O entering the middle ear from ambient and respiratory environments containing N2O. PMID:536296

  17. 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.

  18. High intensity anthropogenic sound damages fish ears

    NASA Astrophysics Data System (ADS)

    McCauley, Robert D.; Fewtrell, Jane; Popper, Arthur N.

    2003-01-01

    Marine petroleum exploration involves the repetitive use of high-energy noise sources, air-guns, that produce a short, sharp, low-frequency sound. Despite reports of behavioral responses of fishes and marine mammals to such noise, it is not known whether exposure to air-guns has the potential to damage the ears of aquatic vertebrates. It is shown here that the ears of fish exposed to an operating air-gun sustained extensive damage to their sensory epithelia that was apparent as ablated hair cells. The damage was regionally severe, with no evidence of repair or replacement of damaged sensory cells up to 58 days after air-gun exposure.

  19. Middle ear cholesteatoma in 11 dogs

    PubMed Central

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

    2011-01-01

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

  20. Genetic Basis of Congenital Cardiovascular Malformations

    PubMed Central

    Lalani, Seema R.; Belmont, John W.

    2014-01-01

    Cardiovascular malformations are a singularly important class of birth defects and, due to dramatic improvements in medical and surgical care, there are now large numbers of adult survivors. The etiologies are complex, but there is strong evidence that genetic factors play a crucial role. Over the last 15 years there has been enormous progress in the discovery of causative genes for syndromic heart malformations and in rare families with Mendelian forms. The rapid characterization of genomic disorders as major contributors to congenital heart defects is also notable. The genes identified encode many transcription factors, chromatin regulators, growth factors and signal transduction pathways– all unified by their required roles in normal cardiac development. Genome-wide sequencing of the coding regions promises to elucidate genetic causation in several disorders affecting cardiac development. Such comprehensive studies evaluating both common and rare variants would be essential in characterizing gene-gene interactions, as well as in understanding the gene-environment interactions that increase the susceptibility to congenital heart defects. PMID:24793338

  1. Twin pregnancy in the congenital malformed uterus.

    PubMed

    Heinonen, Pentti K

    2016-07-01

    The frequency and outcome of twin pregnancies in women with uterine malformation were studied. The cohort comprised 13 (4.9%) women with twin pregnancy found among 263 women. They had 483 deliveries, 13 of them twins (2.7%; 95% CI 1.6-4.6%). Among 38 patients with unicornuate uterus 5 (6.8%) out of 74 deliveries were twins, 39 women with didelphic uterus 2 (3.2%) out of 62 deliveries and 147 women with septate or subseptate uterus 6 (2.3%) out of 264 deliveries were twins. The mean duration of gestation was 249 days (range 190-268 days), 5 (38%) out of 13 deliveries were premature, 25 out of 26 newborns were alive. Mean durations of gestation and mean weights of newborns did not differ when 7 cases with unicornuate or didelphic uterus were compared to 6 cases with complete or partial uterine septum. A congenital malformed uterus can bear twin pregnancy without severe complications apart from prematurity. PMID:26757792

  2. Variations of some elements in cadmium-induced malformed fish

    SciTech Connect

    Muramoto, S.

    1981-08-01

    Reports of malformation induced by cadmium such as the appearance of vertebral anomalies in carp have been described by the present author. In this paper, the appearance of such malformed fish by exposure to cadmium was confirmed in a repeat experiment. Decalcification of the fish was studied from spinal x-ray photographs and the results of some elements analysis.

  3. Middle Ear Infection (Chronic Otitis Media) and Hearing Loss

    MedlinePlus

    ... Doctor Near You Middle Ear Infection (Chronic Otitis Media) and Hearing Loss Middle Ear Infection (Chronic Otitis ... staff at newsroom@entnet.org . What is otitis media? Otitis media refers to inflammation of the middle ...

  4. Pediatric Obesity and Ear, Nose, and Throat Disorders

    MedlinePlus

    ... Calendar Find an ENT Doctor Near You Pediatric Obesity and Ear, Nose, and Throat Disorders Pediatric Obesity ... self-esteem, and isolation from their peers. Pediatric obesity and otolaryngic problems Otolaryngologists, or ear, nose, and ...

  5. Ear biometric recognition using local texture descriptors

    NASA Astrophysics Data System (ADS)

    Benzaoui, Amir; Hadid, Abdenour; Boukrouche, Abdelhani

    2014-09-01

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

  6. 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…

  7. Play It by Ear. Hearing Conservation Curriculum.

    ERIC Educational Resources Information Center

    Olson, Dianne R.

    This curriculum was designed to help teachers teach their fourth-grade students about hearing and the effects of loud noises on hearing. The program describes the human ear and how it works, explains the health effects of noise, and offers ways for students to protect their hearing from unsafe noise levels. Students are taught how hearing is…

  8. Lewis Thomas and droopy rabbit ears

    PubMed Central

    Bashyam, Hema

    2007-01-01

    In 1956, Lewis Thomas discovered that injecting rabbits with a plant protease called papain caused their ears to collapse. This experiment led to the identification of the endogenous proteases that cause the tissue destruction seen in diseases such as rheumatoid arthritis.

  9. "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 ...

  10. Mule Ear Drapery in Jewel Cave

    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....

  11. Ear Infections - Multiple Languages: MedlinePlus

    MedlinePlus

    ... on this page, please enable JavaScript. French (français) Japanese (日本語) Korean (한국어) Russian (Русский) Somali (af Soomaali) ... moyenne - français (French) Bilingual PDF Health Information Translations Japanese (日本語) Middle Ear Infection 中耳炎 - 日本語 (Japanese) Bilingual ...

  12. The formation and management of middle ear granulation tissue in chronic ear disease.

    PubMed

    Roland, Peter S

    2004-01-01

    Granulation tissue is an important pathogenic feature of all types of chronic ear disease, and it can be controlled and treated with good aural toilet, appropriate antibiotic therapy, topical steroids, and chemical cautery or surgical debridement. PMID:14986396

  13. Diving injuries to the inner ear.

    PubMed

    Farmer, J C

    1977-01-01

    Most of the previous literature concerning otologic problems in compressed gas environments has emphasized middle ear barotrauma. With recent increases in commercial, military, and sport diving to deeper depths, inner ear disturbances during these exposures have been noted more frequently. Studies of inner ear physiology and pathology during diving indicate that the causes and treatment of these problems differ depending upon the phase and type of diving. Humans exposed to simulated depths of up to 305 meters without barotrauma or decompression sickness develop transient, conductive hearing losses with no audiometric evidence of cochlear dysfunction. Transient vertigo and nystagmus during diving have been noted with caloric stimulation, resulting from the unequal entry of cold water into the external auditory canals, and with asymmetric middle ear pressure equilibration during ascent and descent (alternobaric vertigo). Equilibrium disturbances noted with nitrogen narcosis, oxygen toxicity, hypercarbia, or hypoxia appear primarily related to the effects of these conditions upon the central nervous system and not to specific vestibular end-organ dysfunction. Compression of humans in helium-oxygen at depths greater than 152.4 meters results in transient symptoms of tremor, dizziness, and nausea plus decrements in postural equilibrium and psychomotor performance, the high pressure nervous syndrome. Vestibular function studies during these conditions indicate that these problems are due to central dysfunction and not to vestibular end-organ dysfunction. Persistent inner ear injuries have been noted during several phases of diving: 1) Such injuries during compression (inner ear barotrauma) have been related to round window ruptures occurring with straining, or a Valsalva's maneuver during inadequate middle ear pressure equilibration. Divers who develop cochlear and/or vestibular symptoms during shallow diving in which decompression sickness is unlikely or during compression in deeper diving, should be placed on bed rest with head elevation and avoidance of maneuvers which result in increased cerebrospinal fluid and intralabyrinthine pressure. With no improvement in symptoms after 48 hours, exploratory tympanotomy and repair of a possible labyrinthine window fistula should be considered. Recompression therapy is contraindicated in these cases... PMID:402882

  14. 15 CFR 734.2 - Important EAR terms and principles.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... 15 Commerce and Foreign Trade 2 2010-01-01 2010-01-01 false Important EAR terms and principles... OF THE EXPORT ADMINISTRATION REGULATIONS § 734.2 Important EAR terms and principles. (a) Subject to the EAR—Definition. (1) “Subject to the EAR” is a term used in the EAR to describe those items...

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

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 21 Food and Drugs 8 2010-04-01 2010-04-01 false Ear, nose, and throat bur. 874.4140 Section 874.4140 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES EAR, NOSE, AND THROAT DEVICES Surgical Devices § 874.4140 Ear, nose, and throat bur....

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

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 21 Food and Drugs 8 2011-04-01 2011-04-01 false Ear, nose, and throat bur. 874.4140 Section 874.4140 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES EAR, NOSE, AND THROAT DEVICES Surgical Devices § 874.4140 Ear, nose, and throat bur....

  17. 15 CFR 734.2 - Important EAR terms and principles.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ... 15 Commerce and Foreign Trade 2 2011-01-01 2011-01-01 false Important EAR terms and principles... OF THE EXPORT ADMINISTRATION REGULATIONS § 734.2 Important EAR terms and principles. (a) Subject to the EAR—Definition. (1) “Subject to the EAR” is a term used in the EAR to describe those items...

  18. 21 CFR 874.3430 - Middle ear mold.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 21 Food and Drugs 8 2011-04-01 2011-04-01 false Middle ear mold. 874.3430 Section 874.3430 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES EAR, NOSE, AND THROAT DEVICES Prosthetic Devices § 874.3430 Middle ear mold. (a) Identification....

  19. 14 CFR 67.205 - Ear, nose, throat, and equilibrium.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... (CONTINUED) AIRMEN MEDICAL STANDARDS AND CERTIFICATION Second-Class Airman Medical Certificate § 67.205 Ear, nose, throat, and equilibrium. Ear, nose, throat, and equilibrium standards for a second-class airman...) 35 50 50 60 (b) No disease or condition of the middle or internal ear, nose, oral cavity, pharynx,...

  20. 14 CFR 67.305 - Ear, nose, throat, and equilibrium.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... (CONTINUED) AIRMEN MEDICAL STANDARDS AND CERTIFICATION Third-Class Airman Medical Certificate § 67.305 Ear, nose, throat, and equilibrium. Ear, nose, throat, and equilibrium standards for a third-class airman...) 35 50 50 60 (b) No disease or condition of the middle or internal ear, nose, oral cavity, pharynx,...

  1. 14 CFR 67.305 - Ear, nose, throat, and equilibrium.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ... 14 Aeronautics and Space 2 2011-01-01 2011-01-01 false Ear, nose, throat, and equilibrium. 67.305... (CONTINUED) AIRMEN MEDICAL STANDARDS AND CERTIFICATION Third-Class Airman Medical Certificate § 67.305 Ear, nose, throat, and equilibrium. Ear, nose, throat, and equilibrium standards for a third-class...

  2. 14 CFR 67.205 - Ear, nose, throat, and equilibrium.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ... 14 Aeronautics and Space 2 2011-01-01 2011-01-01 false Ear, nose, throat, and equilibrium. 67.205... (CONTINUED) AIRMEN MEDICAL STANDARDS AND CERTIFICATION Second-Class Airman Medical Certificate § 67.205 Ear, nose, throat, and equilibrium. Ear, nose, throat, and equilibrium standards for a second-class...

  3. 14 CFR 67.105 - Ear, nose, throat, and equilibrium.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ... 14 Aeronautics and Space 2 2011-01-01 2011-01-01 false Ear, nose, throat, and equilibrium. 67.105... (CONTINUED) AIRMEN MEDICAL STANDARDS AND CERTIFICATION First-Class Airman Medical Certificate § 67.105 Ear, nose, throat, and equilibrium. Ear, nose, throat, and equilibrium standards for a first-class...

  4. 14 CFR 67.105 - Ear, nose, throat, and equilibrium.

    Code of Federal Regulations, 2014 CFR

    2014-01-01

    ... 14 Aeronautics and Space 2 2014-01-01 2014-01-01 false Ear, nose, throat, and equilibrium. 67.105... (CONTINUED) AIRMEN MEDICAL STANDARDS AND CERTIFICATION First-Class Airman Medical Certificate § 67.105 Ear, nose, throat, and equilibrium. Ear, nose, throat, and equilibrium standards for a first-class...

  5. 14 CFR 67.105 - Ear, nose, throat, and equilibrium.

    Code of Federal Regulations, 2013 CFR

    2013-01-01

    ... 14 Aeronautics and Space 2 2013-01-01 2013-01-01 false Ear, nose, throat, and equilibrium. 67.105... (CONTINUED) AIRMEN MEDICAL STANDARDS AND CERTIFICATION First-Class Airman Medical Certificate § 67.105 Ear, nose, throat, and equilibrium. Ear, nose, throat, and equilibrium standards for a first-class...

  6. 40 CFR 211.206-1 - Real ear method.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 40 Protection of Environment 25 2011-07-01 2011-07-01 false Real ear method. 211.206-1 Section 211... PRODUCT NOISE LABELING Hearing Protective Devices § 211.206-1 Real ear method. (a) The value of sound... “Method for the Measurement of Real-Ear Protection of Hearing Protectors and Physical Attenuation...

  7. 14 CFR 67.205 - Ear, nose, throat, and equilibrium.

    Code of Federal Regulations, 2014 CFR

    2014-01-01

    ... 14 Aeronautics and Space 2 2014-01-01 2014-01-01 false Ear, nose, throat, and equilibrium. 67.205... (CONTINUED) AIRMEN MEDICAL STANDARDS AND CERTIFICATION Second-Class Airman Medical Certificate § 67.205 Ear, nose, throat, and equilibrium. Ear, nose, throat, and equilibrium standards for a second-class...

  8. 14 CFR 67.205 - Ear, nose, throat, and equilibrium.

    Code of Federal Regulations, 2013 CFR

    2013-01-01

    ... 14 Aeronautics and Space 2 2013-01-01 2013-01-01 false Ear, nose, throat, and equilibrium. 67.205... (CONTINUED) AIRMEN MEDICAL STANDARDS AND CERTIFICATION Second-Class Airman Medical Certificate § 67.205 Ear, nose, throat, and equilibrium. Ear, nose, throat, and equilibrium standards for a second-class...

  9. 40 CFR 211.206-1 - Real ear method.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... 40 Protection of Environment 26 2013-07-01 2013-07-01 false Real ear method. 211.206-1 Section 211... PRODUCT NOISE LABELING Hearing Protective Devices § 211.206-1 Real ear method. (a) The value of sound... “Method for the Measurement of Real-Ear Protection of Hearing Protectors and Physical Attenuation...

  10. 14 CFR 67.105 - Ear, nose, throat, and equilibrium.

    Code of Federal Regulations, 2012 CFR

    2012-01-01

    ... 14 Aeronautics and Space 2 2012-01-01 2012-01-01 false Ear, nose, throat, and equilibrium. 67.105... (CONTINUED) AIRMEN MEDICAL STANDARDS AND CERTIFICATION First-Class Airman Medical Certificate § 67.105 Ear, nose, throat, and equilibrium. Ear, nose, throat, and equilibrium standards for a first-class...

  11. 40 CFR 211.206-1 - Real ear method.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... 40 Protection of Environment 26 2012-07-01 2011-07-01 true Real ear method. 211.206-1 Section 211... PRODUCT NOISE LABELING Hearing Protective Devices § 211.206-1 Real ear method. (a) The value of sound... “Method for the Measurement of Real-Ear Protection of Hearing Protectors and Physical Attenuation...

  12. 14 CFR 67.305 - Ear, nose, throat, and equilibrium.

    Code of Federal Regulations, 2014 CFR

    2014-01-01

    ... 14 Aeronautics and Space 2 2014-01-01 2014-01-01 false Ear, nose, throat, and equilibrium. 67.305... (CONTINUED) AIRMEN MEDICAL STANDARDS AND CERTIFICATION Third-Class Airman Medical Certificate § 67.305 Ear, nose, throat, and equilibrium. Ear, nose, throat, and equilibrium standards for a third-class...

  13. 14 CFR 67.305 - Ear, nose, throat, and equilibrium.

    Code of Federal Regulations, 2013 CFR

    2013-01-01

    ... 14 Aeronautics and Space 2 2013-01-01 2013-01-01 false Ear, nose, throat, and equilibrium. 67.305... (CONTINUED) AIRMEN MEDICAL STANDARDS AND CERTIFICATION Third-Class Airman Medical Certificate § 67.305 Ear, nose, throat, and equilibrium. Ear, nose, throat, and equilibrium standards for a third-class...

  14. 40 CFR 211.206-1 - Real ear method.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... 40 Protection of Environment 25 2014-07-01 2014-07-01 false Real ear method. 211.206-1 Section 211... PRODUCT NOISE LABELING Hearing Protective Devices § 211.206-1 Real ear method. (a) The value of sound... “Method for the Measurement of Real-Ear Protection of Hearing Protectors and Physical Attenuation...

  15. 40 CFR 211.206-1 - Real ear method.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... 40 Protection of Environment 24 2010-07-01 2010-07-01 false Real ear method. 211.206-1 Section 211... PRODUCT NOISE LABELING Hearing Protective Devices § 211.206-1 Real ear method. (a) The value of sound... “Method for the Measurement of Real-Ear Protection of Hearing Protectors and Physical Attenuation...

  16. 14 CFR 67.205 - Ear, nose, throat, and equilibrium.

    Code of Federal Regulations, 2012 CFR

    2012-01-01

    ... 14 Aeronautics and Space 2 2012-01-01 2012-01-01 false Ear, nose, throat, and equilibrium. 67.205... (CONTINUED) AIRMEN MEDICAL STANDARDS AND CERTIFICATION Second-Class Airman Medical Certificate § 67.205 Ear, nose, throat, and equilibrium. Ear, nose, throat, and equilibrium standards for a second-class...

  17. 14 CFR 67.305 - Ear, nose, throat, and equilibrium.

    Code of Federal Regulations, 2012 CFR

    2012-01-01

    ... 14 Aeronautics and Space 2 2012-01-01 2012-01-01 false Ear, nose, throat, and equilibrium. 67.305... (CONTINUED) AIRMEN MEDICAL STANDARDS AND CERTIFICATION Third-Class Airman Medical Certificate § 67.305 Ear, nose, throat, and equilibrium. Ear, nose, throat, and equilibrium standards for a third-class...

  18. Malformations and the Manx Syndrome in Cats

    PubMed Central

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

    1979-01-01

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

  19. Computational analyses of arteriovenous malformations in neuroimaging.

    PubMed

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

    2015-01-01

    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

  20. Birth malformations in Babylon and Assyria.

    PubMed

    Pangas, J C

    2000-04-10

    In this paper, we intend to study the oldest systematic descriptions of birth malformations in the history of medicine, as they appear in cuneiform texts written four millennia ago in the land between the two rivers, Mesopotamia, the cradle of civilization, the land of Assyro-Babylonian culture. The core of our work will be an omen text, which was published in 1970 and, since then, has remained confined to the field of Assyriological studies; thus, the history of medicine and disease have not taken advantage of its knowledge. This text is known in Assyriology by its Akkadian name: "shumma izbu" (Izbu), "if an anomaly" [Leichty, 1970: The Omen series shumma izbu, Texts from Cuneiform Sources IV]. PMID:10766990

  1. Coil embolization of pulmonary arteriovenous malformations.

    PubMed

    Jackson, J E; Whyte, M K; Allison, D J; Hughes, J M

    1990-01-01

    Sixteen patients with pulmonary arteriovenous malformations (PAVM) have been treated by percutaneous transvenous coil embolization. In the ten patients who have had all their angiographically demonstrable PAVM's embolised there has been a reduction of right to left shunting from a mean of 28.1% to 13% and an improvement in mean arterial oxygen saturation from 87.4% to 92.4%. Eight of these ten patients now have oxygen saturations of more than 90%. All patients have shown symptomatic improvement. There have been three complications relating to the embolizations, none of which has been serious. Coil embolization of PAVM's is an effective, safe and well tolerated procedure. Embolization should be performed in all cases of PAVM's to prevent paradoxical embolization. PMID:2209021

  2. Multimodality imaging of pancreatic arteriovenous malformation.

    PubMed

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

    2015-01-01

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

  3. [Rhythm disorders and cardiac crypto-malformations].

    PubMed

    Davy, J M; Raczka, F; Cung, T T; Combes, N; Bortone, A; Gaty, D

    2005-12-01

    Faced with a cardiac arrhythmia occuring in an apparently healthy heart, it is necessary to perform an anatomical investigation to detect any unsuspected anomalies. Congenital cardiopathy must certainly be excluded, as this is often responsible for rhythm disorders and/or cardiac conduction defects. Similarly, any acquired conditions, cardiomyopathy, or cardiac tumour must be sought. However, the possibility should always be considered of a minimal congenital malformation, which could be repsonsible for: any type of cardiac arrhythmia: rhythm disorder or conduction defect at the atrial, junctional or ventricular level, with a benign or serious prognosis. Unexpected therapeutic difficulties during radiofrequency ablation procedures or at implantation of pacemakers or defibrillators. Together with rhythm studies, the investigation of choice is high quality imaging, either the classic left or right angiography or the more modern cardiac CT or intracardiac mapping. PMID:16433240

  4. [Congenital cystic adenomatoid malformation of the lung or congenital pulmonary airway malformation].

    PubMed

    Sousa, Vítor; Carvalho, Lina

    2003-01-01

    The cystic adenomatoid malformation of the lung is an hamartomatous lesion, easily identifiable by its morphology through the application of Stocker's et al (1977) classification (type 1, 2 and 3) and also following the criteria of Yousem, to understand the five types dependent on the level of malformation in the airway and lung. The three morphological types described by Stocker were identified in 6 cases of the archive of the Department of Pathology of Coimbra's University Hospital, studied morphologically by the use of Movat's pentachromic stain and the application of the antibody anti-CK7 and anti-body anti-TTF1. In the three morphological types the elastic alveolar net is absent. The CK7 identifies the epithelial distribution and is useful to evaluate the extension of the inflammatory lesion. The antibody anti-TTF1, apparently absent in type 3 cases, is easily identified in type 1 and 2 cases and overexpressed in inflammatory areas. It seems that the absence of cells identified by the antibody anti-TTF1 prevents overdiagnosing of type 4 in Yousem's classification of congenital pulmonary airway malformation (CPAM). PMID:14685634

  5. Temporary umbilical loop colostomy for anorectal malformations.

    PubMed

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

    2012-11-01

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

  6. Amplatzer vascular plugs in congenital cardiovascular malformations

    PubMed Central

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

    2013-01-01

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

  7. Knowledge, Attitude and Practice of Ear Care in Coastal Karnataka

    PubMed Central

    Ganapathi, Keerthan; Kanthila, Jayashree

    2015-01-01

    Introduction Ear as an organ is necessary for the perception of sound and body balance. Ear infection, diabetes mellitus, hypertension and excessive use of mobile phone for listening to music at high volume all can reduce hearing. No earlier study was available in the costal Karnataka population, regarding the practice of ear care. The study objective was to ascertain the level of knowledge of the community regarding ear care, to find out whether some of the common conditions affecting hearing are known and to find out the common practices involved in maintaining ear hygiene. Materials and Methods This cross-sectional study was conducted on 500 subjects in two tertiary care hospitals by convenient sampling, using self-administered questionnaire. Knowledge, Attitude and Practice across the age groups, religion & education background were studied. Results Across different education groups, 66.7%-90% did not know that ‘cold’ can cause ear infection and 46.7%-75.0% did not know that diabetes and hypertension can reduce hearing. When there is ear pain or discharge, people put ear drops available at home in 48.3%-75.0% across 3 age groups; 58.5%-61.5% across 3 religions and 44.8%-67.9% across 5 education groups. No statistically significant difference was found in the practice of pouring oil into ears across religions. A total of 58.6%-100% daily clean inside the ear and 70-100% use cotton buds. Conclusion General perception of the people is that ear is necessary only for hearing. Majority did not know that nasal infection can affect the ear and that DM and hypertension can cause hearing loss. When there is ear pain and discharge, most of the adults put drops that are available at home. Pouring oil into the ears and cleaning inside the ear canals is routinely practiced in costal Karnataka. PMID:26816922

  8. A developmental and genetic classification for midbrain-hindbrain malformations

    PubMed Central

    Millen, Kathleen J.; Dobyns, William B.

    2009-01-01

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

  9. 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

  10. 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

  11. Treatment of Prominent Ears and Otoplasty: A Contemporary Review.

    PubMed

    Pawar, Sachin S; Koch, Cody A; Murakami, Craig

    2015-01-01

    Prominent ears affect approximately 5% of the population and can have a significant psychological impact on patients. A wide variety of otoplasty techniques have been described, all sharing the goal of re-creating the normal appearance of the ear and achieving symmetry between the 2 sides. Recent trends in otoplasty techniques have consistently moved toward less invasive options, ranging from nonsurgical newborn ear molding to cartilage-sparing surgical techniques and even incisionless, office-based procedures. Herein, we review anatomy of the external ear, patient evaluation, the evolution of nonsurgical and surgical otoplasty techniques, otoplasty outcomes, and future trends for treatment of prominent ears. PMID:26158729

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

    NASA Astrophysics Data System (ADS)

    Naghibolhosseini, Maryam

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

  13. RASA1 somatic mutation and variable expressivity in capillary malformation/arteriovenous malformation (CM/AVM) syndrome.

    PubMed

    Macmurdo, Colleen F; Wooderchak-Donahue, Whitney; Bayrak-Toydemir, Pinar; Le, Jenny; Wallenstein, Matthew B; Milla, Carlos; Teng, Joyce M C; Bernstein, Jonathan A; Stevenson, David A

    2016-06-01

    Germline mutations in RASA1 are associated with capillary malformation-arteriovenous malformation (CM-AVM) syndrome. CM-AVM syndrome is characterized by multi-focal capillary malformations and arteriovenous malformations. Lymphatic anomalies have been proposed as part of the phenotype. Intrafamilial variability has been reported, suggesting modifiers and somatic events. The objective of the study was to identify somatic RASA1 "second hits" from vascular malformations associated with CM-AVM syndrome, and describe phenotypic variability. Participants were examined and phenotyped. Genomic DNA was extracted from peripheral blood on all participants. Whole-exome sequencing was performed on the proband. Using Sanger sequencing, RASA1 exon 8 was PCR-amplified to track the c.1248T>G, p.Tyr416X germline variant through the family. A skin biopsy of a capillary malformation from the proband's mother was also obtained, and next-generation sequencing was performed on DNA from the affected tissue. A familial germline heterozygous novel pathogenic RASA1 variant, c.1248T>G (p.Tyr416X), was identified in the proband and her mother. The proband had capillary malformations, chylothorax, lymphedema, and overgrowth, while her affected mother had only isolated capillary malformations. Sequence analysis of DNA extracted from a skin biopsy of a capillary malformation of the affected mother showed a second RASA1 somatic mutation (c.2245C>T, p.Arg749X). These results and the extreme variable expressivity support the hypothesis that somatic "second hits" are required for the development of vascular anomalies associated with CM-AVM syndrome. In addition, the phenotypes of the affected individuals further clarify that lymphatic manifestations are also part of the phenotypic spectrum of RASA1-related disorders. © 2016 Wiley Periodicals, Inc. PMID:26969842

  14. Gene expression profiling of the inner ear.

    PubMed

    Schimmang, Thomas; Maconochie, Mark

    2016-02-01

    The identification of transcriptional differences has served as an important starting point in understanding the molecular mechanisms behind biological processes and systems. The developmental biology of the inner ear, the biology of hearing and of course the pathology of deafness are all processes that warrant a molecular description if we are to improve human health. To this end, technological innovation has meant that larger scale analysis of gene transcription has been possible for a number of years now, extending our molecular analysis of genes to beyond those that are currently in vogue for a given system. In this review, some of the contributions gene profiling has made to understanding developmental, pathological and physiological processes in the inner ear are highlighted. PMID:26403558

  15. 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

  16. The inner ear and the neurologist

    PubMed Central

    Agrup, Charlotte; Gleeson, Michael; Rudge, Peter

    2007-01-01

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

  17. Ear replantation after human bite avulsion injury.

    PubMed

    Horta, Ricardo; Costa-Ferreira, António; Costa, Joana; Silva, Pedro; Amarante, José Manuel; Silva, Alvaro; Filipe, Rita

    2011-07-01

    Traumatic auricular amputation due to human bite is not a common event, but it constitutes a difficult challenge for the reconstructive surgeon. Microsurgery can be performed in some cases, but replantation of a severed ear without microsurgery can be a safe alternative. We present a case of a 44-year-old male individual who was involved in a fight and sustained a human bite, resulting in almost a complete amputation of his right ear. The Baudet technique has been used successfully. It is a simple technique and very reliable because it allows a great surface of contact between the graft and the vascular bed, substantially increasing its odds of survival in cases presenting with high risk of infection such as human bite injury. It also produces excellent aesthetic results. PMID:21772155

  18. An Ear-Worn Vital Signs Monitor.

    PubMed

    He, David Da; Winokur, Eric S; Sodini, Charles G

    2015-11-01

    This paper presents a wearable vital signs monitor at the ear. The monitor measures the electrocardiogram (ECG), ballistocardiogram (BCG), and photoplethysmogram (PPG) to obtain pre-ejection period (PEP), stroke volume (SV), cardiac output (CO), and pulse transit time (PTT). The ear is demonstrated as a natural anchoring point for the integrated sensing of physiological signals. All three signals measured can be used to obtain heart rate (HR). Combining the ECG and BCG allows for the estimation of the PEP, while combining the BCG and PPG allows for the measurement of PTT. Additionally, the J-wave amplitude of the BCG is correlated with the SV and, when combined with HR, yields CO. Results from a clinical human study on 13 subjects demonstrate this proof-of-concept device. PMID:26208264

  19. Ringing ears: the neuroscience of tinnitus.

    PubMed

    Roberts, Larry E; Eggermont, Jos J; Caspary, Donald M; Shore, Susan E; Melcher, Jennifer R; Kaltenbach, James A

    2010-11-10

    Tinnitus is a phantom sound (ringing of the ears) that affects quality of life for millions around the world and is associated in most cases with hearing impairment. This symposium will consider evidence that deafferentation of tonotopically organized central auditory structures leads to increased neuron spontaneous firing rates and neural synchrony in the hearing loss region. This region covers the frequency spectrum of tinnitus sounds, which are optimally suppressed following exposure to band-limited noise covering the same frequencies. Cross-modal compensations in subcortical structures may contribute to tinnitus and its modulation by jaw-clenching and eye movements. Yet many older individuals with impaired hearing do not have tinnitus, possibly because age-related changes in inhibitory circuits are better preserved. A brain network involving limbic and other nonauditory regions is active in tinnitus and may be driven when spectrotemporal information conveyed by the damaged ear does not match that predicted by central auditory processing. PMID:21068300

  20. Why Do Elephants Flap Their Ears?

    NASA Astrophysics Data System (ADS)

    Koffi, Moise; Jiji, Latif; Andreopoulos, Yiannis

    2009-11-01

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

  1. 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

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

    PubMed

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

    2007-03-01

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

  3. [Cystic adenomatoid malformation of the lung (author's transl)].

    PubMed

    Cobos Barroso, N; Boix Ochoa, J; Corominas Casaramona, J M; Liñán Cortés, S; Lucaya Layret, J; Torán Fuentes, N

    1979-01-01

    Four cases of congenital cystic adenomatoid malformation are presented. The age of the patients ranged from 1 day to 7 years. All cases were treated surgically and 3 of them survived. The only death in the group corresponded to the neonatal case. The clinical, radiological and patological features of congenital cystic adenomatoid malformation are also reviewed. Occasionally the clinical manifestations will be hardly noticeable or non existent and the malformation may be diagnosed beyond the neonatal period in the course of a radiological examination performed as routine or because of repeated respiratory infections. Surgery is the treatment of choice. In the group of neonates where the cystic adenomatoid malformation doesn't cause respiratory difficulty the authors feel that it might be advisable to postpone surgery until a later date. PMID:484942

  4. Contracted foal syndrome associated with multiple malformations in two foals.

    PubMed

    Binanti, D; Zani, D D; De Zani, D; Turci, T; Zavaglia, G; Riccaboni, P

    2014-02-01

    Congenital anomalies in horses are very rare, and contracted foal syndrome is one of the most commonly reported. This malformation is characterized by contraction of the joints of the forelimbs and/or hindlimbs. In addition, the syndrome can be characterized by vertebral column malformations, such as scoliosis or torticollis, and cranial deformity. The present report describes the radiological and necroscopical findings of multiple rare malformations in two foals. Both foals showed skeletal abnormalities and fenestration of the abdominal cavity. Other pathological findings include a interventricular septal defect in one and a unilateral hydronephrosis and partial hydroureter in the other foal. Although in this report a specific aetiology could not be provided, insecticides treatment provided during the second month of pregnancy might play a role in the pathogenesis of these malformations. PMID:23406278

  5. Prenatal Diagnosis of Unusual Fetal Pial Arteriovenous Malformation

    PubMed Central

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

    2003-01-01

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

  6. Management strategy after diagnosis of Abernethy malformation: a case report

    PubMed Central

    2012-01-01

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

  7. Variants in CUL4B are associated with cerebral malformations.

    PubMed

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

    2015-01-01

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

  8. Variants in CUL4B are Associated with Cerebral Malformations

    PubMed Central

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

    2015-01-01

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

  9. The Chiari malformations: a review with emphasis on anatomical traits.

    PubMed

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

    2015-03-01

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

  10. Mesenteric lymphatic malformation associated with acute appendicitis: a case report

    PubMed Central

    2009-01-01

    Introduction Mesenteric lymphatic malformations are rare, benign tumors that are most commonly found in children. The presentation of these tumors is variable and may either be innocuous or life threatening. It has been suggested that mesenteric lymphatic malformations are congenital; however, there is evidence that their growth may be stimulated by local trauma. Case presentation We describe the first case of a mesenteric lymphatic malformation associated with acute appendicitis in a 13-year-old Caucasian boy. The patient is well six months after surgical excision of the tumor. Conclusion The reader should be aware that growth and/or development of mesenteric lymphatic malformations may be associated with trauma and other pro-inflammatory processes. PMID:19918284

  11. Current concepts of the etiology of central nervous system malformations.

    PubMed

    Melnick, M

    1979-01-01

    We have seen that what must be applied to dysmorphology is the doctrine of multifactorial causality, ie dysmorphogenetic events have both genetic and nongenetic etiologic components to varying degrees. Complicating matters is the extent to which there is etiologic and/or mechanistic heterogeneity (Fig. 1). This is nicely illustrated by the holoprosencephaly anomaly. In addition, there are numerous CNS malformations that have major single gene, chromosomal, or environmental initiating agents of malformation mechanisms. Still a mystery is the common neural tube malformations. It is now clear that the "multifactorial/threshold" model is an inadequate explanation of the observed data and until the etiologic heterogeneity of these malformations is clearly defined, our knowledge remains primarily empiric. A potential area of fruitful investigation is likely to be the identification of maternal genotypes which do not allow detoxification of potential environmental teratogens. PMID:380680

  12. Oral encapsulated vascular malformation: An undescribed presentation in the mouth

    PubMed Central

    Dias, Márcio-Américo; Dias, Pedro-de Souza; Martínez-Martínez, Marisol; Sena-Filho, Marcondes; de Almeida, Oslei-Paes

    2016-01-01

    Vascular lesions have been classified in two broad categories, hemangiomas and malformations. Encapsulated vascular lesions have not been reported in the oral cavity, but they were described in other sites, mainly in the orbit. Herein, we present a case of an oral encapsulated vascular lesion located in the right buccal mucosa of a 69-year-old male, including histological and immunohistochemical description and a literature review. Key words:Buccal mucosa, hemangioma, vascular malformation, oral cavity. PMID:26855712

  13. Multiple medullary venous malformations decreasing cerebral blood flow: Case report

    SciTech Connect

    Tomura, N.; Inugami, A.; Uemura, K.; Hadeishi, H.; Yasui, N. )

    1991-02-01

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

  14. [Anorectal and external genitalia malformation associated with perineal hemangioma].

    PubMed

    Peláez Mata, D J; García Crespo, J M; Fernández Toral, J

    2001-10-01

    We describe a girl with anorectal and genitourinary malformation with perineal hemangioma. Posterior sagittal anorectoplasty was performed at 21 months of age with good functional results. The hemangioma required no treatment and involuted spontaneously when the patient was 6 years old. Although anorectal malformations are frequently associated with multiple congenital anomalies, the association described herein is uncommon and we found only three similar cases reported in the medical literature. PMID:11578543

  15. Antioxidant properties of methanolic extracts from several ear mushrooms.

    PubMed

    Mau, J L; Chao, G R; Wu, K T

    2001-11-01

    Five kinds of ear mushrooms are commercially available in Taiwan, including black, red, jin, snow, and silver ears. Methanolic extracts were prepared from these ear mushrooms, and their antioxidant properties were studied. For all methanolic extracts from ear mushrooms, the antioxidant activities in the 1,3-diethyl-2-thiobarbituric acid method were moderate (38.6 approximately 74.6%) at 1.0-5.0 mg/mL. Methanolic extracts from red, jin, and snow ears showed excellent antioxidant activities in the conjugated diene method at 5.0 mg/mL. At 5.0 mg/mL, reducing powers of methanolic extracts were in the descending order of snow > black approximately red approximately jin > silver ears. The scavenging effect of methanolic extracts from ear mushrooms on 1,1-diphenyl-2-picrylhydrazyl radicals was excellent except for that from silver ears. Ear mushroom extracts were not good scavengers for hydroxyl free radicals but were good chelators for ferrous ions. Naturally occurring antioxidants, including ascorbic acid, tocopherols, and total phenols, were found in the methanolic extracts. However, beta-carotene was not detected. Total antioxidant components were 15.69, 30.09, 27.83, 49.17, and 31.70 mg/g for black, red, jin, snow, and silver ears, respectively. PMID:11714344

  16. Limb malformations and abnormal sex hormone concentrations in frogs.

    PubMed Central

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

    2000-01-01

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

  17. Congenital brain abnormalities: an update on malformations of cortical development and infratentorial malformations.

    PubMed

    Poretti, Andrea; Boltshauser, Eugen; Huisman, Thierry A G M

    2014-07-01

    In the past two decades, significant progress in neuroimaging and genetic techniques has allowed for advances in the correct definition/classification of congenital brain abnormalities, which have resulted in a better understanding of their pathogenesis. In addition, new groups of diseases, such as axonal guidance disorders or tubulinopathies, are increasingly reported. Well-defined neuroimaging diagnostic criteria have been suggested for the majority of congenital brain abnormalities. Accurate diagnoses of these complex abnormalities, including distinction between malformations and disruptions, are of paramount significance for management, prognosis, and family counseling. In the next decade, these advances will hopefully be translated into deeper understanding of these disorders and more specific treatments. PMID:25192502

  18. Implications of an Incidental Pulmonary Arteriovenous Malformation

    PubMed Central

    Holden, Van K.; Shah, Nirav G.; Verceles, Avelino C.

    2016-01-01

    Introduction. Pulmonary arteriovenous malformations (PAVMs) have been associated with life-threatening complications, such as stroke and massive hemoptysis, thus posing significant morbidity if left untreated. We report a case of an incidental finding of a PAVM in a trauma patient newly recognized to have suspected hereditary hemorrhagic telangiectasia (HHT). Case Description. A 34-year-old man with a history of recurrent epistaxis presented with a sudden fall associated with seizure-like activity. Trauma imaging showed a large subdural hematoma and, incidentally, a serpiginous focus within the right upper lobe with a prominent feeding artery consistent with a PAVM. The patient was diagnosed with a simple PAVM related to possible or suspected HHT, an autosomal dominant trait with age-related penetrance. He underwent a pulmonary arteriography of the right upper and lower lobe with the use of a microcatheter system; however, the PAVM could not be visualized. Thus, he was managed medically. The patient was educated on the need for prophylactic antibiotics prior to dental procedures and surveillance imaging. Discussion. Our case highlights the importance of obtaining a complete past medical and family history in young patients with a history of recurrent epistaxis to elicit features of HHT. The diagnosis can be made clinically and directly affects family members, who would otherwise not receive appropriate screening. PMID:27027094

  19. Gastroesophageal reflux and congenital gastrointestinal malformations.

    PubMed

    Marseglia, Lucia; Manti, Sara; D'Angelo, Gabriella; Gitto, Eloisa; Salpietro, Carmelo; Centorrino, Antonio; Scalfari, Gianfranco; Santoro, Giuseppe; Impellizzeri, Pietro; Romeo, Carmelo

    2015-07-28

    Although the outcome of newborns with surgical congenital diseases (e.g., diaphragmatic hernia; esophageal atresia; omphalocele; gastroschisis) has improved rapidly with recent advances in perinatal intensive care and surgery, infant survivors often require intensive treatment after birth, have prolonged hospitalizations, and, after discharge, may have long-term sequelae including gastro-intestinal comorbidities, above all, gastroesophageal reflux (GER). This condition involves the involuntary retrograde passage of gastric contents into the esophagus, with or without regurgitation or vomiting. It is a well-recognized condition, typical of infants, with an incidence of 85%, which usually resolves after physiological maturation of the lower esophageal sphincter and lengthening of the intra-abdominal esophagus, in the first few months after birth. Although the exact cause of abnormal esophageal function in congenital defects is not clearly understood, it has been hypothesized that common (increased intra-abdominal pressure after closure of the abdominal defect) and/or specific (e.g., motility disturbance of the upper gastrointestinal tract, damage of esophageal peristaltic pump) pathological mechanisms may play a role in the etiology of GER in patients with birth defects. Improvement of knowledge could positively impact the long-term prognosis of patients with surgical congenital diseases. The present manuscript provides a literature review focused on pathological and clinical characteristics of GER in patients who have undergone surgical treatment for congenital abdominal malformations. PMID:26229394

  20. Vascular permeability in cerebral cavernous malformations.

    PubMed

    Mikati, Abdul G; Khanna, Omaditya; Zhang, Lingjiao; Girard, Romuald; Shenkar, Robert; Guo, Xiaodong; Shah, Akash; Larsson, Henrik B W; Tan, Huan; Li, Luying; Wishnoff, Matthew S; Shi, Changbin; Christoforidis, Gregory A; Awad, Issam A

    2015-10-01

    Patients with the familial form of cerebral cavernous malformations (CCMs) are haploinsufficient for the CCM1, CCM2, or CCM3 gene. Loss of corresponding CCM proteins increases RhoA kinase-mediated endothelial permeability in vitro, and in mouse brains in vivo. A prospective case-controlled observational study investigated whether the brains of human subjects with familial CCM show vascular hyperpermeability by dynamic contrast-enhanced quantitative perfusion magnetic resonance imaging, in comparison with CCM cases without familial disease, and whether lesional or brain vascular permeability correlates with CCM disease activity. Permeability in white matter far (WMF) from lesions was significantly greater in familial than in sporadic cases, but was similar in CCM lesions. Permeability in WMF increased with age in sporadic patients, but not in familial cases. Patients with more aggressive familial CCM disease had greater WMF permeability compared to those with milder disease phenotype, but similar lesion permeability. Subjects receiving statin medications for routine cardiovascular indications had a trend of lower WMF, but not lesion, permeability. This is the first demonstration of brain vascular hyperpermeability in humans with an autosomal dominant disease, as predicted mechanistically. Brain permeability, more than lesion permeability, may serve as a biomarker of CCM disease activity, and help calibrate potential drug therapy. PMID:25966944

  1. The management of asymptomatic congenital lung malformations.

    TOXLINE Toxicology Bibliographic Information

    Laberge JM; Bratu I; Flageole H

    2004-01-01

    Congenital Pulmonary Airway Malformation (CPAM) and lung sequestration are often discovered on routine prenatal sonography. While some lesions may be large and cause serious complications in the fetus or newborn, many will be asymptomatic at birth. Some authors advocate simple observation because of the lack of data on the incidence of long-term complications. However, there are very few described cases where CPAM and intralobar sequestration (ILS) have remained asymptomatic throughout life; complications eventually develop in virtually all patients. The most common complication is pneumonia, which may respond poorly to medical treatment. Other complications include the development of malignancies (carcinomas and pleuropulmonary blastomas), pneumothorax and hemoptysis or hemothorax. Since lung resection will be required sooner or later for CPAM and ILS, it is best not to wait for complications to occur. We recommend surgery at 3 to 6 months of life at the latest, to allow compensatory lung growth to occur. At this age the postoperative course is usually smooth and long-term follow- up has shown normal respiratory function. The management of small non- communicating extralobar sequestrations is more controversial; it is known that these can remain asymptomatic throughout life but complications may develop and they are sometimes difficult to differentiate from neuroblastoma.

  2. The management of asymptomatic congenital lung malformations.

    PubMed

    Laberge, Jean-Martin; Bratu, Ioana; Flageole, Hélène

    2004-01-01

    Congenital Pulmonary Airway Malformation (CPAM) and lung sequestration are often discovered on routine prenatal sonography. While some lesions may be large and cause serious complications in the fetus or newborn, many will be asymptomatic at birth. Some authors advocate simple observation because of the lack of data on the incidence of long-term complications. However, there are very few described cases where CPAM and intralobar sequestration (ILS) have remained asymptomatic throughout life; complications eventually develop in virtually all patients. The most common complication is pneumonia, which may respond poorly to medical treatment. Other complications include the development of malignancies (carcinomas and pleuropulmonary blastomas), pneumothorax and hemoptysis or hemothorax. Since lung resection will be required sooner or later for CPAM and ILS, it is best not to wait for complications to occur. We recommend surgery at 3 to 6 months of life at the latest, to allow compensatory lung growth to occur. At this age the postoperative course is usually smooth and long-term follow- up has shown normal respiratory function. The management of small non- communicating extralobar sequestrations is more controversial; it is known that these can remain asymptomatic throughout life but complications may develop and they are sometimes difficult to differentiate from neuroblastoma. PMID:14980288

  3. Gastroesophageal reflux and congenital gastrointestinal malformations

    PubMed Central

    Marseglia, Lucia; Manti, Sara; D’Angelo, Gabriella; Gitto, Eloisa; Salpietro, Carmelo; Centorrino, Antonio; Scalfari, Gianfranco; Santoro, Giuseppe; Impellizzeri, Pietro; Romeo, Carmelo

    2015-01-01

    Although the outcome of newborns with surgical congenital diseases (e.g., diaphragmatic hernia; esophageal atresia; omphalocele; gastroschisis) has improved rapidly with recent advances in perinatal intensive care and surgery, infant survivors often require intensive treatment after birth, have prolonged hospitalizations, and, after discharge, may have long-term sequelae including gastro-intestinal comorbidities, above all, gastroesophageal reflux (GER). This condition involves the involuntary retrograde passage of gastric contents into the esophagus, with or without regurgitation or vomiting. It is a well-recognized condition, typical of infants, with an incidence of 85%, which usually resolves after physiological maturation of the lower esophageal sphincter and lengthening of the intra-abdominal esophagus, in the first few months after birth. Although the exact cause of abnormal esophageal function in congenital defects is not clearly understood, it has been hypothesized that common (increased intra-abdominal pressure after closure of the abdominal defect) and/or specific (e.g., motility disturbance of the upper gastrointestinal tract, damage of esophageal peristaltic pump) pathological mechanisms may play a role in the etiology of GER in patients with birth defects. Improvement of knowledge could positively impact the long-term prognosis of patients with surgical congenital diseases. The present manuscript provides a literature review focused on pathological and clinical characteristics of GER in patients who have undergone surgical treatment for congenital abdominal malformations. PMID:26229394

  4. Sports participation with Chiari I malformation.

    PubMed

    Strahle, Jennifer; Geh, Ndi; Selzer, Béla J; Bower, Regina; Himedan, Mai; Strahle, MaryKathryn; Wetjen, Nicholas M; Muraszko, Karin M; Garton, Hugh J L; Maher, Cormac O

    2016-04-01

    OBJECT There is currently no consensus on the safety of sports participation for patients with Chiari I malformation (CM-I). The authors' goal was to define the risk of sports participation for children with the imaging finding of CM-I. METHODS A prospective survey was administered to 503 CM-I patients at 2 sites over a 46-month period. Data were gathered on imaging characteristics, treatment, sports participation, and any sport-related injuries. Additionally, 81 patients completed at least 1 subsequent survey following their initial entry into the registry and were included in a prospective group, with a mean prospective follow-up period of 11 months. RESULTS Of the 503 CM-I patients, 328 participated in sports for a cumulative duration of 4641 seasons; 205 of these patients participated in contact sports. There were no serious or catastrophic neurological injuries. One patient had temporary extremity paresthesias that resolved within hours, and this was not definitely considered to be related to the CM-I. In the prospective cohort, there were no permanent neurological injuries. CONCLUSIONS No permanent or catastrophic neurological injuries were observed in CM-I patients participating in athletic activities. The authors believe that the risk of such injuries is low and that, in most cases, sports participation by children with CM-I is safe. PMID:26636249

  5. Sham Control Methods Used in Ear-Acupuncture/Ear-Acupressure Randomized Controlled Trials: A Systematic Review

    PubMed Central

    Zhang, Claire Shuiqing; Yang, Angela Weihong; Zhang, Anthony Lin; May, Brian H.

    2014-01-01

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

  6. Chinchilla middle-ear admittance and sound power: High-frequency estimates and effects of inner-ear modifications

    PubMed Central

    Ravicz, Michael E.; Rosowski, John J.

    2012-01-01

    The middle-ear input admittance relates sound power into the middle ear (ME) and sound pressure at the tympanic membrane (TM). ME input admittance was measured in the chinchilla ear canal as part of a larger study of sound power transmission through the ME into the inner ear. The middle ear was open, and the inner ear was intact or modified with small sensors inserted into the vestibule near the cochlear base. A simple model of the chinchilla ear canal, based on ear canal sound pressure measurements at two points along the canal and an assumption of plane-wave propagation, enables reliable estimates of YTM, the ME input admittance at the TM, from the admittance measured relatively far from the TM. YTM appears valid at frequencies as high as 17 kHz, a much higher frequency than previously reported. The real part of YTM decreases with frequency above 2 kHz. Effects of the inner-ear sensors (necessary for inner ear power computation) were small and generally limited to frequencies below 3 kHz. Computed power reflectance was ∼0.1 below 3.5 kHz, lower than with an intact ME below 2.5 kHz, and nearly 1 above 16 kHz. PMID:23039439

  7. A preliminary theoretical model of hydrodynamics in the inner ear.

    PubMed

    Yu, Vivian M; Strykowski, Paul J; Odland, Rick M

    2010-04-01

    Head movement should create a transient pressure imbalance across the membranous inner ear. We used basic concepts of fluid dynamics to develop a theoretical model of the inner ear. According to this model, two contiguous fluidic systems-the perilymphatic system and the endolymphatic system-are in hydrostatic equilibrium across a compliant membrane. Our model demonstrates that changes in resistance or compliance in one system results in a transient distortion of the membranous inner ear until equilibrium between the two systems is restored. The concept of hydrodynamic pressure changes in the inner ear has received little attention, but it may represent a new approach to understanding the inner ear and treating inner ear diseases. PMID:20397144

  8. 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. PMID:26171086

  9. Congenital malformations in twins in England and Wales.

    PubMed Central

    Doyle, P E; Beral, V; Botting, B; Wale, C J

    1991-01-01

    STUDY OBJECTIVE--The aim was to compare congenital malformation rates in twin births with those in singleton births. DESIGN--The study was an analysis of malformation rates in singleton and twin births using data from the Office of Population Censuses and Survey's Congenital Malformation Notification Scheme. SETTING--This was a national survey of births in England and Wales in 1979-1980 and 1982-1985. PARTICIPANTS--The data comprised 95,510 reported malformations in 3.7 million singleton births, and 1925 reported malformations in 76,000 twin births. MEASUREMENTS AND MAIN RESULTS--Twin malformation ratios were calculated using maternal age specific singleton rates as standard. In comparison with singleton births, twins have significantly higher reported frequencies of indeterminate sex and pseudohermaphroditism; anencephaly; patent ductus arteriosis; exomphalos; hydrocephalus; anomalies of the umbilical vessels; atresia or stenosis of the large intestine or anus; and tracheo-oesophageal fistula, atresia or stenosis. Twins also have significant reported deficits of polydactyly and syndactyly; congenital dislocation of the hip; anomalies of the tongue, branchial cleft and auricular sinus; post-anal dimple; and Down's syndrome. CONCLUSIONS--Several major malformations were significantly more common in twins than in singletons. The excess of indeterminate sex and pseudohermaphroditism has not been described before and may be analogous to freemartinism in cattle. Most of the conditions less common in twins are minor, and the reported deficits may be due to underascertainment of the less serious conditions in twins. Down's syndrome is an exception, and the deficit may well be real. PMID:2045744

  10. EARS, MARS combined radio observations - 2014

    NASA Astrophysics Data System (ADS)

    Tomezzoli, Giancarlo

    2014-02-01

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

  11. Sebaceous Hyperplasia Mimicking Linear Wart over Ear

    PubMed Central

    Nair, Pragya A; Diwan, Nilofar G

    2015-01-01

    Sebaceous hyperplasia (SH), a common, benign condition of sebaceous gland, presents most commonly over face. Lesions are usually described as asymptomatic, soft, discrete, and yellow with a surface that ranges from smooth to slightly verrucous. The pathogenesis is not fully understood. It does not require treatment, but many time lesions can be cosmetically unfavorable than, need to be destroyed or excised. A case of 23-year-old male presenting with the skin colored to hyperpigmented linear hyperkeratotic plaque mimicking a wart, over the right ear is presented here, which was diagnosed as SH. PMID:26903746

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

    PubMed

    Liu, Bor-Shong

    2008-01-01

    To achieve mass customization and collaborative product design, human factors and ergonomics should play a key development role. The purpose of this study was to provide product designers with the anthropometic dimensions of outer ears for different demographic data, including gender and age. The second purpose was to compare the dimensions of various ear-related products (i.e., earphone, bluetooth earphone and ear-cup earphone) with the anthropometic database and recommend appropriate solutions for design. Two hundred subjects aged 20-59 was selected for this study and divided into four age stratifications. Further, three different dimensions of the outer ear (i.e., the earhole length, the ear connection length and the length of the pinna) were measured by superimposed grid photographic technique. The analysis of variance (ANOVA) was used to investigate the effects of gender, and age on ear dimensions. The results showed that all ear dimensions had significant gender effects. A comparison between the anthropometric dimensions and those of current products revealed that most current ear-related products need to be redesigned using anthropometric data. The shapes of earhole and pinna are not circular. Consequently, ear products need to be elongated so that users may feel more comfortably and not have the product slip off easily. PMID:17374520

  13. 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.

  14. 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

  15. Surgical Strategies for Acutely Ruptured Arteriovenous Malformations.

    PubMed

    Martinez, Jaime L; Macdonald, R Loch

    2015-01-01

    Brain arteriovenous malformations (AVMs) are focal neurovascular lesions consisting of abnormal fistulous connections between the arterial and venous systems with no interposed capillaries. This arrangement creates a high-flow circulatory shunt with hemorrhagic risk and hemodynamic abnormalities. While most AVMs are asymptomatic, they may cause severe neurological complications and death. Each AVM carries an annual rupture risk of 2-4%. Intracranial hemorrhage due to AVM rupture is the most common initial manifestation (up to 70% of presentations), and it carries significant morbidity and mortality. This complication is particularly important in the young and otherwise healthy population, in whom AVMs cause up to one-third of all hemorrhagic strokes. A previous rupture is the single most important independent predictor of future hemorrhage. Current treatment modalities for AVM are microsurgery, endovascular embolization, and radiosurgery. In acutely ruptured AVMs, early microsurgical excision is usually avoided. The standard is to wait at least 4 weeks to allow for patient recovery, hematoma liquefaction, and inflammatory reactions to subside. Exceptions to this rule are small, superficial, low-grade AVMs with elucidated angioarchitecture, for which early simultaneous hematoma evacuation and AVM excision is feasible. Emergent hematoma evacuation with delayed AVM excision (unless, as mentioned, the AVM is low grade) is recommended in patients with a decreased level of consciousness due to intracranial hemorrhage, posterior fossa or temporal lobe hematoma of >30 ml, or hemispheric hematoma of >60 ml. The applicability of endovascular techniques for acutely ruptured AVMs is not clear, but feasible options, until a definitive treatment is determined, include occluding intranidal and distal flow-related aneurysms and 'sealing' any rupture site or focal angioarchitectural weakness when one can be clearly identified and safely accessed. Radiosurgery is not performed in acutely ruptured AVMs because its therapeutic effects occur in a delayed fashion. PMID:26587641

  16. Effect of the middle ear reflex on sound transmission to the inner ear of rat.

    PubMed

    Pilz, P K; Ostwald, J; Kreiter, A; Schnitzler, H U

    1997-03-01

    The effect of the acoustic middle ear reflex (MER) was quantified using electrodes chronically implanted in the middle ears of rats. Cochlear microphonics (CM) and middle ear muscle EMG were measured under light Ketamin anesthesia after stimulation with tone pulses of 5-20 kHz ranging between 75 and 120 dB SPL. With increasing intensity, the CM measured before the onset of the MER increased to a maximum amplitude and then decreased with higher SPLs. At 10 kHz this maximum was reached at 95 dB SPL, for other stimulus frequencies at higher SPLs. After a latency of 10-20 ms, CM to 10 kHz stimuli of 80-95 dB SPL were decreased by the attenuating action of the MER. The lowest threshold of the MER was also measured at 10 kHz (77 dB SPL in the mean). To stimuli greater than 100 dB SPL after a latency of 6-10 ms, the CM amplitude was increased. That this CM increase to intense stimuli is caused by the action of the MER was confirmed by control experiments such as cutting the tendons of the middle ear muscles. The CM decrease to stimuli below 100 dB SPL, as well as the increase to very intense stimuli, can be explained by sound attenuation caused by the MER, together with the nonlinear dependence of CM amplitude on stimulus level. The observed shift of the maxima of the CM input-output function by the MER to higher stimulus levels probably indicates an increase of the dynamic range of the ear. PMID:9083814

  17. 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.

  18. A familial venous malformation locus is on chromosome 9p

    SciTech Connect

    Boon, L.M.; Mulliken, J.B.; Vikkula, M.

    1994-09-01

    Venous malformation is the most common vascular malformation affecting 0.2% of the population. Depending upon size and location, these slow-flow lesions may cause pain, anatomic distortion and threaten life. Most venous malformations occur sporadically and present as solitary lesions. For this reason, determining their pathogenic bases has proven elusive. However, venous malformations also occur in several rare syndromes, some of which demonstrate Mendelian inheritance. As a first step towards identifying the pathogenic bases for these lesions, we have mapped a locus for an autosomal dominant disorder in a three generation family that manifests as multiple cutaneous and mucosal venous malformations. This locus lies within a 24.5 cM interval on chromosome 9p, defined by the markers D9S157 and D9S163. A maximum LOD score of 4.11 at {theta} = 0.05 is obtained with several markers within the interval. The interferon gene cluster, which has previously been implicated in angiogenesis, and the multiple tumor suppressor gene, responsible for several types of malignant tumors, also lie within this interval and are potential candidates.

  19. 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.

  20. Enhanced visualization of inner ear structures

    NASA Astrophysics Data System (ADS)

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

    2004-07-01

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

  1. Probing the Xenopus laevis inner ear transcriptome for biological function

    PubMed Central

    2012-01-01

    Background The senses of hearing and balance depend upon mechanoreception, a process that originates in the inner ear and shares features across species. Amphibians have been widely used for physiological studies of mechanotransduction by sensory hair cells. In contrast, much less is known of the genetic basis of auditory and vestibular function in this class of animals. Among amphibians, the genus Xenopus is a well-characterized genetic and developmental model that offers unique opportunities for inner ear research because of the amphibian capacity for tissue and organ regeneration. For these reasons, we implemented a functional genomics approach as a means to undertake a large-scale analysis of the Xenopus laevis inner ear transcriptome through microarray analysis. Results Microarray analysis uncovered genes within the X. laevis inner ear transcriptome associated with inner ear function and impairment in other organisms, thereby supporting the inclusion of Xenopus in cross-species genetic studies of the inner ear. The use of gene categories (inner ear tissue; deafness; ion channels; ion transporters; transcription factors) facilitated the assignment of functional significance to probe set identifiers. We enhanced the biological relevance of our microarray data by using a variety of curation approaches to increase the annotation of the Affymetrix GeneChip® Xenopus laevis Genome array. In addition, annotation analysis revealed the prevalence of inner ear transcripts represented by probe set identifiers that lack functional characterization. Conclusions We identified an abundance of targets for genetic analysis of auditory and vestibular function. The orthologues to human genes with known inner ear function and the highly expressed transcripts that lack annotation are particularly interesting candidates for future analyses. We used informatics approaches to impart biologically relevant information to the Xenopus inner ear transcriptome, thereby addressing the impediment imposed by insufficient gene annotation. These findings heighten the relevance of Xenopus as a model organism for genetic investigations of inner ear organogenesis, morphogenesis, and regeneration. PMID:22676585

  2. Evaluation of fungal flora in normal and diseased canine ears.

    PubMed

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

    2010-12-01

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

  3. Spinal arteriovenous malformations: Is surgery indicated?

    PubMed Central

    Singh, Bikramjit; Behari, Sanjay; Jaiswal, Awadhesh K.; Sahu, Rabi Narayan; Mehrotra, Anant; Mohan, B. Madan; Phadke, Rajendra V.

    2016-01-01

    Purpose: To identify clinico-radiological distinguishing features in various types of spinal arteriovenous malformations (AVM) with an aim to define the role of surgical intervention. Materials and Methods: Hero's modified Di Chiro classification differentiated four types of spinal AVMs on digital subtraction angiogram (DSA) in 74 patients: I. Dural arteriovenous fistulae (n = 35, 47.3%); II. Glomus/intramedullary (n = 13, 17.6%); III. Juvenile/metameric (n = 4, 5.4%); and, IV. Ventral perimedullary fistula (n = 21, 28.4%). A patient with extradural AVM remained unclassified. Demographic profiles, DSA features and reason for surgical referral were recorded. Statistical comparison of discrete variables like gender, spinal cord level, presentation and outcome was made using Chi-square test; and, continuous variables like age, feeder number, duration of symptoms and number of staged embolizations by one way analysis of variance with Boneferoni post hoc comparison. Embolization alone (n = 39, 52.7%), surgery alone (n = 16, 21.6%), and combined approach (n = 4, 5.4%) were the treatments offered (15 were treated elsewhere). Results: Type I-AVM occurred in significantly older population than other types (P = 0.01). Mean duration of symptoms was 13.18 ± 12.8 months. Thoracic cord involvement predominated in type-I and III AVMs (P = 0.01). Number of feeding arteries were 1 in 59.7%; 2 in 29.0%; and, multiple in 11.3% patients, respectively. Staged embolization procedures in type-III AVM were significant (P < 0.01). Surgical referral was required due to: Vessel tortuosity/insufficient parent vessel caliber (n = 7); residual AVM (n = 4); low flow AVM (n = 3); and, multiple feeders (n = 2). Check DSA (n = 34) revealed complete AVM obliteration in 26 and minor residual lesion in eight patients. Neurological status improved in 26 and stabilized in 25 patients. Conclusions: Differentiating between Type I-IV AVMs has a significant bearing on their management. Surgical intervention should be considered as an important adjunct/alternative to therapeutic embolization. PMID:27057219

  4. Genes and brain malformations associated with abnormal neuron positioning.

    PubMed

    Moffat, Jeffrey J; Ka, Minhan; Jung, Eui-Man; Kim, Woo-Yang

    2015-01-01

    Neuronal positioning is a fundamental process during brain development. Abnormalities in this process cause several types of brain malformations and are linked to neurodevelopmental disorders such as autism, intellectual disability, epilepsy, and schizophrenia. Little is known about the pathogenesis of developmental brain malformations associated with abnormal neuron positioning, which has hindered research into potential treatments. However, recent advances in neurogenetics provide clues to the pathogenesis of aberrant neuronal positioning by identifying causative genes. This may help us form a foundation upon which therapeutic tools can be developed. In this review, we first provide a brief overview of neural development and migration, as they relate to defects in neuronal positioning. We then discuss recent progress in identifying genes and brain malformations associated with aberrant neuronal positioning during human brain development. PMID:26541977

  5. Baller-Gerold syndrome associated with congenital portal venous malformation.

    PubMed Central

    Savarirayan, R; Tomlinson, P; Thompson, E

    1998-01-01

    We report a 4 year old boy in whom the clinical features of craniosynostosis and bilateral absent radii led to a diagnosis of Baller-Gerold syndrome. Additional congenital abnormalities included midface hypoplasia, atrial and ventricular septal defects, right hydronephrosis, partial sacral agenesis, and anterior ectopic anus. Evidence of portal venous hypertension was present from 8 months and a congenital portal venous malformation was discovered at 2 years. This is the first reported case of Baller-Gerold syndrome associated with a congenital portal venous malformation. We discuss the diagnostic confusion between this syndrome and other overlapping malformation syndromes and propose optimal evaluation strategies aimed at clarifying the nosology of these syndromes. Images PMID:9733037

  6. Electroencephalography in congenital malformations of the central nervous system.

    PubMed

    Campos, P; Cruz, G; Lizarraga, R; Bancalari, E; Guillen, D; Castañeda, C

    1994-12-01

    We studied clinical and EEG features of 36 cases with congenital malformations of the CNS. Patients were followed at the outpatient clinic of Hospital Cayetano Heredia and of Hogar Clinica San Juan de Dios in Lima-Peru, from January 1984 to June 1992. Eighty percent of the patients had convulsive syndromes and mental retardation. The most frequent malformation was agenesis of corpus callosum, and it was not possible to find a "typical" EEG pattern. The second were porencephalic cysts, with a good clinical-EEG correlation. There were two typical cases of schizencephaly, one of hemimegalencephaly with good prognosis, and one of holoprosencephaly. The results are compared to those obtained for a series we previously reported. Data discussed take into account reports on the subject registered in the literature. It is concluded that EEG is an useful method to evaluate possible CNS malformations in developing countries. PMID:7611945

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

    SciTech Connect

    Suh, Jin-Suck; Shin, Kyoo-Ho; Na, Jae-Bum; Won, Jong-Yun; Hahn, Soo-Bong

    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.

  8. Sleep-Related Breathing Disorders and the Chiari 1 Malformation.

    PubMed

    Leu, Roberta M

    2015-11-01

    The Chiari 1 malformation is characterized by > 5-mm herniation of the cerebellar tonsils through the foramen magnum. Consequent compression of the brain stem and nearby neuronal structures involved in respiratory control and maintenance of pharyngeal wall muscle tone may result in respiratory changes during sleep. These changes include respiratory failure and arrest, as well as sleep-related breathing disorders (ie, OSA and central sleep apnea). Although data have accrued on the significance of sleep-related breathing disorders in patients with the Chiari 1 malformation, many management questions remain unanswered. This article reviews the available literature on prevalence and management of sleep-related breathing disorders in patients with the Chiari 1 malformation. PMID:26158759

  9. 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)

  10. [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

  11. 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

  12. 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…

  13. 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...

  14. 21 CFR 344.12 - Ear drying aid active ingredient.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ... 21 Food and Drugs 5 2014-04-01 2014-04-01 false Ear drying aid active ingredient. 344.12 Section 344.12 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED....12 Ear drying aid active ingredient. The active ingredient of the product consists of...

  15. 21 CFR 344.12 - Ear drying aid active ingredient.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ... 21 Food and Drugs 5 2012-04-01 2012-04-01 false Ear drying aid active ingredient. 344.12 Section 344.12 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED....12 Ear drying aid active ingredient. The active ingredient of the product consists of...

  16. CT of adenomas of the middle ear and mastoid cavity

    SciTech Connect

    Van Thong Ho; Rao, V.J.; Mikaelian, D.O.

    1996-03-01

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

  17. 21 CFR 344.12 - Ear drying aid active ingredient.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ... 21 Food and Drugs 5 2013-04-01 2013-04-01 false Ear drying aid active ingredient. 344.12 Section 344.12 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED....12 Ear drying aid active ingredient. The active ingredient of the product consists of...

  18. Models of cortical malformation-Chemical and physical.

    PubMed

    Luhmann, Heiko J

    2016-02-15

    Pharmaco-resistant epilepsies, and also some neuropsychiatric disorders, are often associated with malformations in hippocampal and neocortical structures. The mechanisms leading to these cortical malformations causing an imbalance between the excitatory and inhibitory system are largely unknown. Animal models using chemical or physical manipulations reproduce different human pathologies by interfering with cell generation and neuronal migration. The model of in utero injection of methylazoxymethanol (MAM) acetate mimics periventricular nodular heterotopia. The freeze lesion model reproduces (poly)microgyria, focal heterotopia and schizencephaly. The in utero irradiation model causes microgyria and heterotopia. Intraperitoneal injections of carmustine 1-3-bis-chloroethyl-nitrosurea (BCNU) to pregnant rats produces laminar disorganization, heterotopias and cytomegalic neurons. The ibotenic acid model induces focal cortical malformations, which resemble human microgyria and ulegyria. Cortical dysplasia can be also observed following prenatal exposure to ethanol, cocaine or antiepileptic drugs. All these models of cortical malformations are characterized by a pronounced hyperexcitability, few of them also produce spontaneous epileptic seizures. This dysfunction results from an impairment in GABAergic inhibition and/or an increase in glutamatergic synaptic transmission. The cortical region initiating or contributing to this hyperexcitability may not necessarily correspond to the site of the focal malformation. In some models wide-spread molecular and functional changes can be observed in remote regions of the brain, where they cause pathophysiological activities. This paper gives an overview on different animal models of cortical malformations, which are mostly used in rodents and which mimic the pathology and to some extent the pathophysiology of neuronal migration disorders associated with epilepsy in humans. PMID:25850077

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

    PubMed

    Caffier, Ph P; Sedlmaier, B; Hölzl, M

    2009-07-01

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

  20. LKB1 Is Required for the Development and Maintenance of Stereocilia in Inner Ear Hair Cells in Mice

    PubMed Central

    Men, Yuqin; Zhang, Aizhen; Li, Haixiang; Zhang, Tingting; Jin, Yecheng; Li, Huashun

    2015-01-01

    The LKB1 gene, which encodes a serine/threonine kinase, was discovered to play crucial roles in cell differentiation, proliferation, and the establishment of cell polarity. In our study, LKB1 conditional knockout mice (Atoh1-LKB1-/- mice) were generated to investigate LKB1 function in the inner ear. Tests of auditory brainstem response and distortion product otoacoustic emissions revealed significant decreases in the hearing sensitivities of the Atoh1-LKB1-/- mice. In Atoh1-LKB1-/- mice, malformations of hair cell stereocilliary bundles were present as early as postnatal day 1 (P1), a time long before the maturation of the hair cell bundles. In addition, we also observed outer hair cell (OHC) loss starting at P14. The impaired stereocilliary bundles occurred long before the presence of hair cell loss. Stereociliary cytoskeletal structure depends on the core actin-based cytoskeleton and several actin-binding proteins. By Western blot, we examined actin-binding proteins, specifically ERM (ezrin/radixin/moesin) proteins involved in the regulation of the actin cytoskeleton of hair cell stereocilia. Our results revealed that the phosphorylation of ERM proteins (pERM) was significantly decreased in mutant mice. Thus, we propose that the decreased pERM may be a key factor for the impaired stereocillia function, and the damaged stereocillia may induce hair cell loss and hearing impairments. Taken together, our data indicates that LKB1 is required for the development and maintenance of stereocilia in the inner ear. PMID:26274331

  1. 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

  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. 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. PMID:26167373

  4. Cerebral arteriovenous malformation presenting as visual deterioration in a child.

    PubMed

    Kaye, L C; Kaye, S B; Lagnado, R; Boothroyd, A; Morton, C; May, P

    2000-10-01

    A rare case of visual loss as the presenting feature of a central arteriovenous malformation involving the vein of Galen is reported. A 5-year-old girl with a history of deteriorating vision for the past 6 months was examined. Ocular examination showed a left hemianopia, left optic atrophy, and dilated vessels of the right optic disc. MRI revealed a massive deep-seated central arteriovenous malformation involving the vein of Galen. The mechanism of visual loss is likely to be a combination of ischaemic optic atrophy associated with a steal phenomenon and direct compression of the right optic radiation. PMID:11085301

  5. Microvascular salvage of a thrombosed total ear replant.

    PubMed

    Senchenkov, Alex; Jacobson, Steven R

    2013-07-01

    Microvascular replantation, when possible, is the treatment of choice for total ear amputations. Both arterial and venous reconstruction should be attempted. The present case report describes a successful total ear replantation in a 45-year-old woman whose ear was amputated due to a horse accident. Venous thrombosis subsequently occurred and was managed with anticoagulation and leech therapy. Eighty hours after the replantation, arterial thrombosis took place. The posterior auricular artery thrombosed anastomosis was resected and reconstructed with an interposition vein graft. This report illustrates the feasibility of the successful microvascular salvage of a thrombosed total ear replant. It suggests the need for close clinical monitoring of the replanted ear and prompt microvascular reexploration in an event of the loss of arterial flow. PMID:23640855

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

    PubMed

    Liebau, A; Plontke, S K

    2015-06-01

    The indications for local drug therapy of inner ear hearing loss include sudden sensorineural hearing loss, Menière's disease, autoimmune-associated hearing loss, ototoxicity as a side effect of other therapies, acute acoustic trauma and improvement of the safety and performance of cochlear implants. Various drugs are currently being used and tested for local treatment of inner ear hearing loss, including glucocorticoids, growth factors, apoptosis inhibitors, antioxidants, TNF-α inhibitors and antibodies. To further a better understanding of pharmacokinetics and the development of rational pharmacotherapy of the inner ear, the"liberation, absorption, distribution, metabolism, elimination" (LADME) principle can be applied to local therapy of the inner ear. Local application strategies can be differentiated into intratympanic applications to the middle ear cavity and direct intralabyrinthine or intracochlear applications. PMID:25008276

  7. Inner ear symptoms and disease: Pathophysiological understanding and therapeutic options

    PubMed Central

    Ciuman, Raphael R.

    2013-01-01

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

  8. The comparative anatomy of the pig middle ear cavity: a model for middle ear inflammation in the human?

    PubMed Central

    PRACY, J. P.; WHITE, A.; MUSTAFA, Y.; SMITH, D.; PERRY, M. E.

    1998-01-01

    This study was undertaken to develop a functional model of otitis media with effusion (OME) in the pig (Sus scrofa), with the purpose of investigating the origin of lymphocytes populating the middle ear during the course of an inflammatory process. The relevance of the model to the human condition of OME is to a large extent dependent on the anatomical and physiological similarities between the middle ear cavity and the pharyngeal lymphoid tissue of the pig and man. Anatomical specimens were collected from 7 young Large White pigs to determine the gross anatomy of the middle ear cavity and the histological characteristics of the middle ear mucosa. It was found that the anatomy of the 3 parts of the middle ear cavity in man and in the pig is broadly similar, although some minor differences were observed. The porcine eustachian tube was seen to be cartilaginous throughout its length in contrast to the part osseous, part cartilaginous structure found in man; the porcine ossicles were slightly different in shape to those of man and the air cell system was situated inferior to the tympanic cavity in the pig as opposed to posteriorly in man. This paper describes the structure and morphology of the pig middle ear cavity and compares and contrasts it with that of man. The minor differences observed are of anatomical importance but do not diminish the usefulness of the pig middle ear cleft as a potential model for human middle ear disorders. PMID:9688502

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

    ERIC Educational Resources Information Center

    Varvarigou, Maria

    2014-01-01

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

  10. Sclerotherapy using 1% sodium tetradecyl sulfate to treat a vascular malformation: a report of two cases.

    PubMed

    Min, Hong-Gi; Kim, Su-Gwan; Oh, Ji-Su; You, Jae-Seek

    2015-12-01

    Vascular malformations are the most common congenital and neonatal vascular anomalies in the head and neck region. The demand for simple and esthetic vascular malformation treatments have increased more recently. In this study, two patients that were diagnosed with venous malformations were treated with sodium tetradecyl sulfate as a sclerosing agent. Recurrence was not found one year after the surgery. This article gives a brief case report of sclerotherapy as an effective approach to treat vascular malformations in the oral cavity. PMID:26734559

  11. Sclerotherapy using 1% sodium tetradecyl sulfate to treat a vascular malformation: a report of two cases

    PubMed Central

    2015-01-01

    Vascular malformations are the most common congenital and neonatal vascular anomalies in the head and neck region. The demand for simple and esthetic vascular malformation treatments have increased more recently. In this study, two patients that were diagnosed with venous malformations were treated with sodium tetradecyl sulfate as a sclerosing agent. Recurrence was not found one year after the surgery. This article gives a brief case report of sclerotherapy as an effective approach to treat vascular malformations in the oral cavity. PMID:26734559

  12. 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,…

  13. 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,

  14. Mimosa tenuiflora as a Cause of Malformations in Ruminants in the Northeastern Brazilian Semiarid Rangelands

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Craniofacial anomalies, eye malformations, and permanent flexures of the forelimbs are common malformations seen in ruminants grazing semiarid rangelands of Northeastern Brazil. To investigate the cause of these malformations, we fed 2 suspected plants, Mimosa tenuiflora or Prosopis juliflora, to gr...

  15. Hydrostatic pressure in the inner ear fluid compartments and its effects on inner ear function.

    PubMed

    Bhmer, A

    1993-01-01

    The present study summarizes the experimental findings obtained on the pressure in the inner ear fluids and on the effects of pressure changes on cochlear function in the guinea pig. Two types of pressures have to be distinguished in the inner ear fluid compartments: (i) hydrostatic fluid pressure and (ii) superimposed hydrodynamic high frequency (> 100 Hz) sound pressure oscillations. Hydrostatic pressure in the inner ear fluids in guinea pigs is in the order of 200 Pa (2 cm H2O) and shows slow (< 5 Hz) respiratory and pulsatory oscillations as well as considerable physiological variations in the range of -100 to +700 Pa. In normal ears, hydrostatic pressure in the perilymph equals pressure in the endolymph, and pressure changes applied to one compartment are immediately transmitted to the other one. A high compliance of Reissner's membrane seems to be the cause of this endolymphatic-perilymphatic pressure equalization. In experimental endolymphatic hydrops, a unique animal model for Meniere's disease, endolymphatic pressure is higher (100 Pa and above) than perilymphatic pressure. These pressure gradients occur only in late stages of hydrops, probably when Reissner's membrane has lost its high compliance after long standing distension. Positive endolymphatic-perilymphatic pressure gradients are secondary to and not the primary cause of hydrops formation. Changes of hydrostatic pressure do not affect auditory function as long as they stay in the physiological range. This includes the sudden loss of positive inner ear pressure that occurs in perilymph fistulas. The rationale for surgical repair of perilymph fistulas in patients in order to restore the hearing function thus becomes questionable. Other aspects of surgical repair, however, as e.g. prevention of labyrinthitis due to permanently open fistula, could not be investigated in this model, because in guinea pigs even large fistulas heal spontaneously within a few days. In experimental endolymphatic hydrops, deterioration of auditory thresholds was partially correlated to the presence of positive endolymphatic-perilymphatic pressure gradients. A change in pressure, however, occurred later than the first deterioration in auditory function. Therefore positive endo-perilymphatic pressure gradients may contribute to, but are not the only cause of hearing impairment. PMID:8273452

  16. Diencephalic-Mesencephalic Junction Dysplasia: A Novel Recessive Brain Malformation

    ERIC Educational Resources Information Center

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

    2012-01-01

    We describe six cases from three unrelated consanguineous Egyptian families with a novel characteristic brain malformation at the level of the diencephalic-mesencephalic junction. Brain magnetic resonance imaging demonstrated a dysplasia of the diencephalic-mesencephalic junction with a characteristic "butterfly"-like contour of the midbrain on

  17. Polymicrogyria: a common and heterogeneous malformation of cortical development.

    PubMed

    Stutterd, Chloe A; Leventer, Richard J

    2014-06-01

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

  18. Major malformations after first trimester exposure to aspirin and NSAIDs.

    PubMed

    Nakhai-Pour, Hamid Reza; Bérard, Anick

    2008-09-01

    The use of aspirin and other NSAIDs during the first trimester of pregnancy is widespread, despite inconclusive evidence regarding the possible risks for the baby. We present an overview of the current evidence relating to the associations between aspirin or NSAID use during the first trimester of pregnancy and the risk of congenital malformations. We systematically searched Medline, Embase, the Cochrane Library and the reference lists of all relevant articles from 1966 to March 2008 that examined the association between aspirin and NSAID use during the first trimester of pregnancy and the risk of congenital malformations in humans. We analyzed 30 studies that met the predefined inclusion criteria: 22 case-control studies, seven cohort studies and one randomized, controlled trial. There are not enough human data available to assess the effect of high-dose aspirin and NSAIDs in pregnant women, such as those used in the treatment of rheumatoid arthritis, osteoarthritis and pain relief. This review suggests that the exposure to aspirin or NSAIDs during the first trimester of pregnancy is associated with an increased risk of gastroschisis (aspirin), cardiac malformations (NSAIDs) and orofacial malformations (naproxen). PMID:24422733

  19. Diagnosis and Nonsurgical Management of Uterine Arteriovenous Malformation

    SciTech Connect

    Rangarajan, R. D.; Moloney, J. C.; Anderson, H. J.

    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.

  20. 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

  1. 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

  2. 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…

  3. LIMITED PCB ANTAGONISM OF TCDD-INDUCED MALFORMATIONS IN MICE

    EPA Science Inventory

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

  4. Complex cardiac malformation in a case of trisomy 9.

    PubMed Central

    Williams, T; Zardawi, I; Quaife, R; Young, I D

    1985-01-01

    A case of trisomy 9 showing a complex cardiac malformation is presented with a review of other published cases. A distinct trisomy 9 syndrome can be recognised with intrauterine growth retardation, short survival, consistent facial dysmorphism, congenital heart disease, and abnormalities of the skeletal, genital, and renal systems. There is no evidence for a maternal age effect. Images PMID:4009647

  5. Complex cardiac malformation in a case of trisomy 9.

    PubMed

    Williams, T; Zardawi, I; Quaife, R; Young, I D

    1985-06-01

    A case of trisomy 9 showing a complex cardiac malformation is presented with a review of other published cases. A distinct trisomy 9 syndrome can be recognised with intrauterine growth retardation, short survival, consistent facial dysmorphism, congenital heart disease, and abnormalities of the skeletal, genital, and renal systems. There is no evidence for a maternal age effect. PMID:4009647

  6. Embryonic Expression of Cyclooxygenase-2 Causes Malformations in Axial Skeleton*

    PubMed Central

    Shim, Minsub; Foley, Julie; Anna, Colleen; Mishina, Yuji; Eling, Thomas

    2010-01-01

    Cyclooxygenases (COXs) have important functions in various physiological and pathological processes. COX-2 expression is highly induced by a variety of stimuli and is observed during certain periods of embryonic development. In this report, the direct effect of COX-2 expression on embryonic development is examined in a novel COX-2 transgenic mouse model that ubiquitously expresses human COX-2 from the early stages of embryonic development. COX-2 transgenic fetuses exhibit severe skeletal malformations and die shortly after birth. Skeletal malformations are localized along the entire vertebral column and rib cage and are linked to defective formation of cartilage anlagen. The cartilage anlagen of axial skeleton fail to properly develop in transgenic embryos because of impaired precartilaginous sclerotomal condensation, which results from the reduction of cell number in the sclerotome. Despite the ubiquitous expression of COX-2, the number of apoptotic cells is highly increased in the sclerotome of transgenic embryos but not in other tissues, suggesting that it is a tissue-specific response. Therefore, the loss of sclerotomal cells due to an increased apoptosis is probably responsible for axial skeletal malformations in transgenic fetuses. In addition, the sclerotomal accumulation of p53 protein is observed in transgenic embryos, suggesting that COX-2 may induce apoptosis via the up-regulation of p53. Our results demonstrate that the aberrant COX-2 signaling during embryonic development is teratogenic and suggest a possible association of COX-2 with fetal malformations of unknown etiology. PMID:20236942

  7. Diencephalic-Mesencephalic Junction Dysplasia: A Novel Recessive Brain Malformation

    ERIC Educational Resources Information Center

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

    2012-01-01

    We describe six cases from three unrelated consanguineous Egyptian families with a novel characteristic brain malformation at the level of the diencephalic-mesencephalic junction. Brain magnetic resonance imaging demonstrated a dysplasia of the diencephalic-mesencephalic junction with a characteristic "butterfly"-like contour of the midbrain on…

  8. Coiling of a vulvar arterio-venous malformation

    PubMed Central

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

    2011-01-01

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

  9. Zwislocki's model of the middle ear re-visited

    NASA Astrophysics Data System (ADS)

    Withnell, Robert H.; Fields, Taylor N.

    2015-12-01

    Zwislocki's circuit model of the middle ear [11] has been used, in original or modified form, in subsequent studies modeling the ear [4, 6]. The model includes two eardrum modes of vibration, a shunt for flexible coupling between the incus and stapes, and a single tuned oscillator for ossicular vibration. The contribution of each of these mechanisms was examined by fitting a model of the ear to acoustic input impedance data from healthy human ears. The circuit elements for a non-ossicular eardrum vibration and a flexible coupling between the incus and stapes were found to be detrimental or non-essential for the model-fit-to-data. A single mode of eardrum vibration for sound transmission to the middle ear is consistent with the eardrum acting as an impedance-matching device, with pars-tensa eardrum vibration coupled to the ossicles [1]. A single-tuned oscillator was insufficient to account for the bandwidth of the ear. The frequency response of the ear suggests multiple resonant modes of ossicular vibration.

  10. Human fetal inner ear involvement in congenital cytomegalovirus infection

    PubMed Central

    2013-01-01

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

  11. 3D Ear Identification Based on Sparse Representation

    PubMed Central

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

    2014-01-01

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

  12. 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

  13. 3D visualization of middle ear structures

    NASA Astrophysics Data System (ADS)

    Vogel, Uwe; Schmitt, Thomas

    1998-06-01

    The achievement of volume geometry data from middle ear structures and surrounding components performs a necessary supposition for the finite element simulation of the vibrational and transfer characteristics of the ossicular chain. So far those models base on generalized figures and size data from anatomy textbooks or particular manual and one- or two-dimensional distance measurements of single ossicles, mostly obtained by light microscopy, respectively. Therefore the goal of this study is to create a procedure for complete three-dimensional imaging of real middle ear structures (tympanic membrane, ossicles, ligaments) in vitro or even in vivo. The main problems are their microscopic size with relevant structures from 10 micrometer to 5 mm, representing various tissue properties (bone, soft tissue). Additionally, these structures are surrounded by the temporal bone, the most solid bone of the human body. Generally there exist several established diagnostic tools for medical imaging that could be used for geometry data acquisition, e.g., X-ray computed tomography and magnetic resonance imaging. Basically they image different tissue parameters, either bony structures (ossicles), or soft tissue (tympanic membrane, ligaments). But considering this application those standard techniques allow low spatial resolution only, usually in the 0.5 - 1mm range, at least in one spatial direction. Thus particular structures of the middle ear region could even be missed completely because of their spatial location. In vitro there is a way out by collecting three complete data sets, each distinguished by 90 degree rotation of a cube-shaped temporal bone specimen. That allows high-resolution imaging in three orthogonal planes, which essentially supports the three-dimensional interpolation of the unknown elements, starting from the regularly set elements of the cubic grid with an edge extension given by the original two-dimensional matrix. A different approach represents the application of a micro- tomographic imaging device. Therefore an X-ray beam focused down to few microns passes the object in a tomographic arrangement. Subsequently the slices become reconstructed. Generally spatial resolution down to 10 micrometer may be obtained by using this procedure. But there exist few devices only, it is not available as standard equipment. The best results concerning spatial resolution should be achieved by applying conventional histologic sectioning techniques. Of course the target will become destroyed during the procedure. It is cut into sections (e.g., 10 micrometer thick), every layer is stained, and the image acquired and stored by a digital still-camera with appropriate resolution (e.g., 2024 X 3036). Three-dimensional reconstruction is done with the computer. The staining allows visual selection of bones and soft tissues, resolutions down to 10 micrometer are possible without target segmentation. But there arise some practical problems. Mainly the geometric context of the layers is affected by the cutting procedure, especially if cutting bone. Another problem performs the adjustment of the -- possibly distorted -- slices to each other. Artificial markers are necessary, which could allow automatic adjustment too. But the introduction and imaging of the markers is difficult inside the temporal bone specimen, that is interspersed by several cavities. Of course the internal target structures must not be destroyed by the marker introduction. Furthermore the embedding compound could disturb the image acquisition, e.g., by optical scattering of paraffin. A related alternative is given by layered ablation/grinding and imaging of the top layer. This saves the geometric consistency, but requires very tricky and time-consuming embedding procedures. Both approaches require considerable expenditures. The possible approaches are evaluated in detail and first results are compared. So far none of the above-mentioned procedures has been established as a standard tool for three-dimensional geometry data acquisition of the middle ear. Otherwise the establishment of a high-resolution imaging technique for those structures, even in vivo, would be of high interest in diagnostics, anatomy and middle ear modeling and research at all.

  14. Congenital diaphragmatic hernia: influence of associated malformations on survival.

    PubMed Central

    Sweed, Y; Puri, P

    1993-01-01

    The medical records of 116 consecutive cases of congenital diaphragmatic hernia (CHD) among 368,772 live births at the three maternity hospitals in Dublin were examined and the incidence of associated malformations and their impact on survival analysed. The patients were divided into two groups: group I included 64 (55%) patients who died during resuscitation and stabilisation before surgery at a mean age of 11.2 hours and group II included 52 (45%) patients who were operated upon. All patients in group I underwent detailed postmortem examination as did the 45% patients who died in group II. The mean (SD) gestational age for group I patients (36.1 (4.5) weeks) was significantly lower than the mean gestational age of group II patients (39.0 (2.4) weeks). Similarly, the mean birth weight of group I patients (2415 (906) g) was significantly lower than that of group II patients (3140 (563) g). Of the newborns who died before surgery, 40 (62.5%) patients had 79 associated malformations. The major associated anomalies were: cardiac (n = 16), neural tube defects (n = 15), skeletal (n = 8), chromosomal (n = 5), urinary tract (n = 6), gastrointestinal (n = 3), omphalocele (n = 4), craniofacial (n = 5), pulmonary (n = 2), and syndromes (n = 2). Sixteen (40%) of these patients were found to have multiple anomalies. Of the 52 patients who were operated upon, only four (7.7%) had associated malformations. Our data shows that associated malformations in neonates with CDH is a major factor influencing outcome in this congenital malformation. PMID:8192736

  15. Brain Vascular Malformation Consortium: Overview, Progress and Future Directions

    PubMed Central

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

    2013-01-01

    Brain vascular malformations are resource-intensive to manage effectively, are associated with serious neurological morbidity, lack specific medical therapies, and have no validated biomarkers for disease severity and progression. Investigators have tended to work in research silos with suboptimal cross-communication. We present here a paradigm for interdisciplinary collaboration to facilitate rare disease research. The Brain Vascular Malformation Consortium (BVMC) is a multidisciplinary, inter-institutional group of investigators, one of 17 consortia in the Office of Rare Disease Research Rare Disease Clinical Research Network (RDCRN). The diseases under study are: familial Cerebral Cavernous Malformations type 1, common Hispanic mutation (CCM1-CHM); Sturge-Weber Syndrome (SWS); and brain arteriovenous malformation in hereditary hemorrhagic telangiectasia (HHT). Each project is developing biomarkers for disease progression and severity, and has established scalable, relational databases for observational and longitudinal studies that are stored centrally by the RDCRN Data Management and Coordinating Center. Patient Support Organizations (PSOs) are a key RDCRN component in the recruitment and support of participants. The BVMC PSOs include Angioma Alliance, Sturge Weber Foundation, and HHT Foundation International. Our networks of clinical centers of excellence in SWS and HHT, as well as our PSOs, have enhanced BVMC patient recruitment. The BVMC provides unique and valuable resources to the clinical neurovascular community, and recently reported findings are reviewed. Future planned studies will apply successful approaches and insights across the three projects to leverage the combined resources of the BVMC and RDCRN in advancing new biomarkers and treatment strategies for patients with vascular malformations. PMID:25221778

  16. Correlation between ultrasound diagnosis and autopsy findings of fetal malformations

    PubMed Central

    Vimercati, Antonella; Grasso, Silvana; Abruzzese, Marinella; Chincoli, Annarosa; de Gennaro, Alessandra; Miccolis, Angela; Serio, Gabriella; Selvaggi, Luigi; Fascilla, Fabiana Divina

    2012-01-01

    Summary Objective to compare ultrasound (US) and autopsy findings of fetal malformations in second trimester terminations of pregnancy to evaluate the degree of agreement between US and fetal autopsy. Methods in this study, all second trimester termination of pregnancy between 2003–2010 were considered. US and autopsy findings were compared and all cases were classified into five categories according to the degree of agreement between US and pathology (A1: full agreement between US and autopsy; A2: autopsy confirmed all US findings but revealed additional anomalies ‘rarely detectable’ prenatally; B: autopsy demonstrated all US findings but revealed additional anomalies ‘detectable’ prenatally; C: US findings were only partially demonstrated at fetal autopsy; D: total disagreement between US and autopsy). Results 144 cases were selected. In 49% of cases there was total agreement between US and autopsy diagnosis (A1). In 22% of cases additional information were about anomalies ‘not detectable’ by US (A2). In 12% of cases autopsy provided additional information about anomalies not observed but ‘detectable’ by US (B). In 13% of cases some anomalies revealed at US, such as valve insufficiencies, pericardial and pleural effusions, were not verified at autopsy (C). Total lack of agreement was noted only in 4% of cases (D). Main areas of disagreement concerned cardiovascular, CNS and complex malformations. The degree of agreement was higher if malformations were diagnosed in a tertiary center. Conclusions this study shows an overall high degree of agreement between definitive US and autopsy findings in second trimester termination of pregnancy for fetal malformations. Autopsy reveals to be the best tool to diagnose malformations and often showed other abnormalities of clinical importance not detected by US, but sometimes also US could provide additional information about functional anomalies because US is a dynamic examination. PMID:22905306

  17. Unilateral cerebellar hypoplasia and mesencephalic malformation in a Hanoverian foal.

    PubMed

    Schröder, S; Schmidt, M J; Preis, S; Klumpp, S; Köhler, K; Kuchelmeister, K; Herden, C

    2013-01-01

    Neurological cases, especially in foals, are rare in the daily practical work. The most common causes are traumata and infectious diseases of the central nervous system (CNS). This case report provides further insights into the wide spectrum of possible neuropathological lesions by detailing a complex malformation with unilateral neurological signs that occurred later post natum. Thus, clinicians should also be aware of malformations in case of respective neurological patients. A Hanoverian foal was presented with progressive ataxia. General and blood examination revealed no further alterations. By neurologic examination, a unilateral hypermetria was diagnosed and a cysternography of the head was performed. A cerebellar malformation was assumed and the foal was euthanized due to poor prognosis. At necropsy, a unilateral absence of a cerebellar hemisphere and vermis accompanied by contralateral malformation of the mesencephalon was diagnosed. The missing areas of the right cerebellar hemisphere were replaced by a cystic formation. The left part of the mesencephalic lamina quadrigemina was reduced in size and the corpus callosum was hypoplastic. Additional microscopical findings were most obvious near the cyst formation and included angiofibrosis in remaining cerebellar and mesencephalic parenchyma and leptomeninges, heterotopia of cerebellar neurons, sclerosis in cerebellar cortex, focal proliferation of meningeal cells and mild mononuclear perivascular infiltrates. Occassional irregular neuronal arrangement in the mesencephalon was also present. Infectious agents such as Borna disease virus, rabies virus, and equine herpesvirus were not detected. Therefore, the complex malformation in this foal might have been caused by a destructive, possibly ischemic event, or could represent a sequel of a primary retrocerebellar cyst with accompanying compression of adjacent parenchyma. PMID:23608891

  18. 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

  19. [Hernia of the external ear canal of uncommon origin].

    PubMed

    Llorente Arenas, E M; Fraile Rodrigo, J; Naya Gálvez, M J; Marín Garrido, C; Carmen Sampériz, L; Martínez Berganza, R; Damborenea Tajada, J

    2000-01-01

    The temporo-mandibular joint is directly related to the anterior wall of the external ear canal. Therefore, defects in this wall may lead to herniation of the soft tissues of the temporo-mandibular joint into the external ear canal. Such herniation usually is secondary to external injuries, iatrogenic trauma, neoplasms, and so on, but it may also be congenital. We report a well-documented case of temporo-mandibular joint herniation into the external ear canal of probable congenital origin. PMID:10799936

  20. Neonatal Hairy Ear Pinnae and Gestational Diabetes: Just a Coincidence?

    PubMed

    Valerio, Enrico; Riello, Laura; Chirico, Michela; Semenzato, Rossella; Cutrone, Mario

    2015-01-01

    A newborn girl of 36 weeks gestation was noted to have several anomalies, including bilateral low ear attachment with ear pinnae hypertrichosis, left preauricular pit, micrognathia, short lingual frenulum, and short neck. Pregnancy history revealed poorly controlled maternal gestational diabetes (GD). Localized hypertrichosis of the ear pinnae may represent a potential marker of GD and thereby alert physicians to suspect other potentially GD-associated conditions such as macrosomia, asphyxia, respiratory distress, hypoglycemia, hypocalcemia, hyperbilirubinemia, polycythemia, hypertrophic cardiomyopathy, and congenital anomalies, particularly those involving the central nervous system. PMID:26391439

  1. [Introduction to the surgery of the middle ear: general principles].

    PubMed

    Babighian, G; Domínguez, M J

    1993-01-01

    We study the basic guidelines of the middle ear surgery, having as target the recuperation of the anatomic functional integrity of ear. There are different important elements in the quality/quantity of the functional results as follows: The surgery technical method used. The actual pathology in the middle ear. The quality of eustachian tube function. The surgeon's experience and ability and several factors. We review the concept introduced by Wullstein called it tympanoplasty from 1952 till nowadays. Finally, we described the most frequent surgery procedures used in the daily practice. PMID:8129965

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

    SciTech Connect

    Lv, Ming-ming; Fan, Xin-dong; Su, Li-xin

    2013-10-15

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

  3. Stereotactic radiosurgery for deep intracranial arteriovenous malformations, part 2: Basal ganglia and thalamus arteriovenous malformations.

    PubMed

    Cohen-Inbar, Or; Ding, Dale; Sheehan, Jason P

    2016-02-01

    The aim of this review is to critically analyze the outcomes following stereotactic radiosurgery (SRS) for arteriovenous malformations (AVM) of the basal ganglia and thalamus. The management of these deep-seated lesions continues to challenge neurosurgeons. Basal ganglia and thalamic AVM show a higher risk of hemorrhage, and an associated devastating morbidity and mortality, as compared to AVM in more superficial locations. Any of the currently available treatment modalities may fail or result in iatrogenic neurologic deterioration. Recent evidence from A Randomized Trial of Unruptured Brain AVM (ARUBA) further deters aggressive approaches that carry a significant risk of treatment-related adverse events. Microsurgical resection, endovascular embolization and SRS all play a role in the treatment of AVM. SRS is an effective therapeutic option for AVM of the thalamus and basal ganglia that are deemed high risk for resection. SRS offers acceptable obliteration rates, with generally lower risks of hemorrhage occurring during the latency period compared to the AVM natural history. Considering that incompletely obliterated lesions still harbor the potential for rupture, additional treatments such as repeat SRS and microsurgical resection should be considered when complete obliteration is not achieved by an initial SRS procedure. Patients with AVM of the basal ganglia and thalamus require continued clinical and radiologic observation and follow-up after SRS, even after angiographic obliteration has been confirmed. PMID:26732284

  4. Stereotactic radiosurgery for deep intracranial arteriovenous malformations, part 1: Brainstem arteriovenous malformations.

    PubMed

    Cohen-Inbar, Or; Ding, Dale; Chen, Ching-Jen; Sheehan, Jason P

    2016-02-01

    The management of brainstem arteriovenous malformations (AVM) are one of the greatest challenges encountered by neurosurgeons. Brainstem AVM have a higher risk of hemorrhage compared to AVM in other locations, and rupture of these lesions commonly results in devastating neurological morbidity and mortality. The potential morbidity associated with currently available treatment modalities further compounds the complexity of decision making for affected patients. Stereotactic radiosurgery (SRS) has an important role in the management of brainstem AVM. SRS offers acceptable obliteration rates with lower risks of hemorrhage occurring during the latency period. Complex nidal architecture requires a multi-disciplinary treatment approach. Nidi partly involving subpial/epipial regions of the dorsal midbrain or cerebellopontine angle should be considered for a combination of endovascular embolization, micro-surgical resection and SRS. Considering the fact that incompletely obliterated lesions (even when reduced in size) could still cause lethal hemorrhages, additional treatment, including repeat SRS and surgical resection should be considered when complete obliteration is not achieved by first SRS. Patients with brainstem AVM require continued clinical and radiological observation and follow-up after SRS, well after angiographic obliteration has been confirmed. PMID:26740034

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

    PubMed Central

    VanderMeer, Julia E.; Ahituv, Nadav

    2011-01-01

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

  6. Congenital pulmonary malformations in pediatric patients: review and update on etiology, classification, and imaging findings.

    PubMed

    Lee, Edward Y; Dorkin, Henry; Vargas, Sara O

    2011-09-01

    Congenital pulmonary malformations represent a heterogeneous group of developmental disorders affecting the lung parenchyma, the arterial supply to the lung, and the lung's venous drainage. In both asymptomatic and symptomatic pediatric patients with congenital pulmonary malformations, the diagnosis of such malformations usually requires imaging evaluation, particularly in cases of surgical lesions for preoperative assessment. The goal of this article is to review the current imaging techniques for evaluating congenital pulmonary malformations and their characteristic imaging findings, which can allow differentiation among various congenital pulmonary malformations in pediatric patients. PMID:21889015

  7. Evolution of Gravity Receptors in the Ear

    NASA Technical Reports Server (NTRS)

    Popper, Arthur N. (Principal Investigator)

    1996-01-01

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

  8. Arnold-Chiari malformation and associated anomalies in a dicephalic newborn calf.

    PubMed

    Wakuri, H; Mori, T; Mutoh, K

    1990-12-01

    A full-term female newborn calf of the Japanese black short-horn breed which was two-headed (dicephalus) and had two incomplete necks was carefully dissected. The calf was one of twins, the other being a normal male. There was no record of reproductive problems with the cow. The left and right heads were of almost the same size and shape. Each head had a nose, mouth, eyeballs, and ears. The brains were very congested. That is to say, in both the left and right brains, the longitudinal fissure was unclear, and these was narrower pattern in the cerebral gyri. The surfaces of the two brains displayed poly- or microgyria-like features. The cerebellar gyri ran longitudinally among the occipital lobes of the cerebral hemispheres. The caudal half of the cerebrum, the cerebellum and the brain stem were distorted ventrally at an angle of approximately 80 degrees. Twisting of the brain stem was observed in the right brain. The end of the medulla oblongata and a tongue-like process of the cerebellum extended through the foramen magnum of the skull into each enlarged vertebral canal (Arnold-Chiari malformation). They extended under the arch of the each 2nd cervical vertebra. The 4th ventricle was also displaced and irregularly dilated. The two necks were conjoined in the single 3rd cervical vertebra at an angle of approximately 45 degrees to each other. The one trunk was slightly distorted. The ribs revealed the presence of a left 14th extra tib. In the lumbar region, spina bifida was apparent. The pelvis was distorted asymmetrically. The tail was short. The viscera appeared as one set, but the rectum and anus were duplicated. Although the calf displayed female features, the vagina was undeveloped. The four limbs appeared normal and the joints moved freely. PMID:2093159

  9. The Massachusetts Eye and Ear Infirmary, Boston.

    PubMed

    Mardell, A

    1995-09-01

    It was a wet and grey day in downtown Boston. It was reported on the six o'clock news that a record 2.7 inches of rain fell on the city that day. Much of it seemed to have fallen on me as I drove in from the suburbs. I had come all this way on a long anticipated holiday to see 'The Fall' in New England but there was something about the rain that was strangely reminiscent of our home grown variety in Wales. Should I perhaps have stayed at home with some videos and books? It would certainly have been cheaper. I had arranged to visit the 'Eye and Ear' some weeks before. The link being one of my consultant colleagues who had undergone some of his training as a resident there many years before, under the guidance of the famous Dr. Harold Schucneckt. I wanted to see what things were like in hospitals and operating theatres and get a taste of the American nursing and healthcare system. PMID:7663057

  10. Tricellular Tight Junctions in the Inner Ear

    PubMed Central

    2016-01-01

    Tight junctions (TJs) are structures that seal the space between the epithelial cell sheets. In the inner ear, the barrier function of TJs is indispensable for the separation of the endolymphatic and perilymphatic spaces, which is essential for the generation and maintenance of the endocochlear potential (EP). TJs are formed by the intercellular binding of membrane proteins, known as claudins, and mutations in these proteins cause deafness in humans and mice. Within the epithelial cell sheet, however, a bound structure is present at the site where the corners of three cells meet (tricellular tight junctions (tTJs)), and the maintenance of the barrier function at this location cannot be explained by the claudins alone. Tricellulin and the angulin family of proteins (angulin-1/LSR, angulin-2/ILDR1, and angulin-3/ILDR2) have been identified as tTJ-associated proteins. Tricellulin and ILDR1 are localized at the tTJ and alterations in these proteins have been reported to be involved in deafness. In this review, we will present the current state of knowledge for tTJs. PMID:27195292

  11. 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

  12. 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 = γ ITD for constant 1 < γ < 5 and variable input frequency <ν∘ follows; e.g., for the Tockay gecko ν∘ ~ 1 . 5 kHz. Third, we use a sectorial instead of circular membrane to quantify the effect of the extracolumella embedded in the tympanum and connecting with the cochlea. The main parameters can be adjusted so that the model is species independent. Work done in collaboration with A.P. Vedurmudi and J. Goulet; partially supported by BCCN-Munich.

  13. Green laser light activates the inner ear

    NASA Astrophysics Data System (ADS)

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

    2009-07-01

    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-μm 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 μJ/pulse) and remained consistent even after 30 minutes of continuous stimulation at 13 μJ, 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.

  14. NARSTO PAC2001 GOLDEN EARS GAS PM DATA

    Atmospheric Science Data Center

    2014-04-25

    NARSTO PAC2001 GOLDEN EARS GAS PM DATA Project Title:  NARSTO Discipline:  ... Station, Ground Station Instrument:  Pressure Sensor Temperature Probe Humidity Sensor Wind Sensor UV Ozone Detector Chemiluminescence DMA GC-MS ...

  15. How minute sooglossid frogs hear without a middle ear

    PubMed Central

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

    2013-01-01

    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. Forgotten T-tube in the middle ear.

    PubMed

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

    2012-05-01

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

  17. 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

  18. Ensemble training to improve recognition using 2D ear

    NASA Astrophysics Data System (ADS)

    Middendorff, Christopher; Bowyer, Kevin W.

    2009-05-01

    The ear has gained popularity as a biometric feature due to the robustness of the shape over time and across emotional expression. Popular methods of ear biometrics analyze the ear as a whole, leaving these methods vulnerable to error due to occlusion. Many researchers explore ear recognition using an ensemble, but none present a method for designing the individual parts that comprise the ensemble. In this work, we introduce a method of modifying the ensemble shapes to improve performance. We determine how different properties of an ensemble training system can affect overall performance. We show that ensembles built from small parts will outperform ensembles built with larger parts, and that incorporating a large number of parts improves the performance of the ensemble.

  19. [Aural polyp in chronic inflammatory middle ear disease].

    PubMed

    López Aguado, D; López Campos, D; Pérez Piñero, B; Campos Bañales, M E

    2003-03-01

    240 patients with chronic otitis media (COM) were studied: 166 ears termed as non cholesteatomatous otitis media and 74 with cholesteatoma. In 38 ears an aural polyp was found with no evidence of cholesteatoma in 19 ears (11.4%) whereas a cholesteatoma was present in the remaining 19 ears. The histology of the polyp and the characteristics of the chronic process were matched: a) The aural polyp is an infrequent complication in COM. b) After histological analysis was found to present two different pictures: The inflammatory reaction polyp, present in non cholesteatomatous COM; and the polyp with granulation tissue and foreign body reaction (keratina) usually found in cholesteatomatous COM. c) The finding of granulation tissue reaction and keratina in an aural polyp is a good predictor for the presence of a cholesteatoma. PMID:12825338

  20. 21 CFR 874.4500 - Ear, nose, and throat microsurgical carbon dioxide laser.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

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  1. 21 CFR 874.4250 - Ear, nose, and throat electric or pneumatic surgical drill.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

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  2. 21 CFR 874.4350 - Ear, nose, and throat fiberoptic light source and carrier.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

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  3. 21 CFR 874.4250 - Ear, nose, and throat electric or pneumatic surgical drill.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

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  4. 21 CFR 874.4500 - Ear, nose, and throat microsurgical carbon dioxide laser.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

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  5. 21 CFR 874.5300 - Ear, nose, and throat examination and treatment unit.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

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  6. 21 CFR 874.5300 - Ear, nose, and throat examination and treatment unit.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

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  7. 21 CFR 874.4350 - Ear, nose, and throat fiberoptic light source and carrier.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

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  8. 21 CFR 874.5300 - Ear, nose, and throat examination and treatment unit.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

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  9. 21 CFR 874.5220 - Ear, nose, and throat drug administration device.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

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  10. 21 CFR 874.4250 - Ear, nose, and throat electric or pneumatic surgical drill.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

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  11. 21 CFR 874.4350 - Ear, nose, and throat fiberoptic light source and carrier.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

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  12. 21 CFR 874.4350 - Ear, nose, and throat fiberoptic light source and carrier.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

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  13. 21 CFR 874.5300 - Ear, nose, and throat examination and treatment unit.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

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  14. 21 CFR 874.4350 - Ear, nose, and throat fiberoptic light source and carrier.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

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  15. 21 CFR 874.4250 - Ear, nose, and throat electric or pneumatic surgical drill.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

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  16. 21 CFR 874.4250 - Ear, nose, and throat electric or pneumatic surgical drill.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

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  17. 21 CFR 874.5300 - Ear, nose, and throat examination and treatment unit.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

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  18. 21 CFR 874.4420 - Ear, nose, and throat manual surgical instrument.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

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  19. 21 CFR 874.4420 - Ear, nose, and throat manual surgical instrument.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

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  20. 21 CFR 874.5220 - Ear, nose, and throat drug administration device.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

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  1. 21 CFR 874.5220 - Ear, nose, and throat drug administration device.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ... 21 Food and Drugs 8 2014-04-01 2014-04-01 false Ear, nose, and throat drug administration device... SERVICES (CONTINUED) MEDICAL DEVICES EAR, NOSE, AND THROAT DEVICES Therapeutic Devices § 874.5220 Ear, nose, and throat drug administration device. (a) Identification. An ear, nose, and throat...

  2. 21 CFR 874.4420 - Ear, nose, and throat manual surgical instrument.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

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  3. SHIELD: an integrative gene expression database for inner ear research

    PubMed Central

    Shen, Jun; Scheffer, Déborah I.; Kwan, Kelvin Y.; Corey, David P.

    2015-01-01

    The inner ear is a highly specialized mechanosensitive organ responsible for hearing and balance. Its small size and difficulty in harvesting sufficient tissue has hindered the progress of molecular studies. The protein components of mechanotransduction, the molecular biology of inner ear development and the genetic causes of many hereditary hearing and balance disorders remain largely unknown. Inner-ear gene expression data will help illuminate each of these areas. For over a decade, our laboratories and others have generated extensive sets of gene expression data for different cell types in the inner ear using various sample preparation methods and high-throughput genome-wide approaches. To facilitate the study of genes in the inner ear by efficient presentation of the accumulated data and to foster collaboration among investigators, we have developed the Shared Harvard Inner Ear Laboratory Database (SHIELD), an integrated resource that seeks to compile, organize and analyse the genomic, transcriptomic and proteomic knowledge of the inner ear. Five datasets are currently available. These datasets are combined in a relational database that integrates experimental data and annotations relevant to the inner ear. The SHIELD has a searchable web interface with two data retrieval options: viewing the gene pages online or downloading individual datasets as data tables. Each retrieved gene page shows the gene expression data and detailed gene information with hyperlinks to other online databases with up-to-date annotations. Downloadable data tables, for more convenient offline data analysis, are derived from publications and are current as of the time of publication. The SHIELD has made published and some unpublished data freely available to the public with the hope and expectation of accelerating discovery in the molecular biology of balance, hearing and deafness. Database URL: https://shield.hms.harvard.edu PMID:26209310

  4. Ear keloids: a review and update of treatment options

    PubMed Central

    SOBEC, RALUCA; DOBREANU, CODRIN; FODOR, LUCIAN; ŞOMCUTEAN, ANA; ŢICHIL, IOANA; COSGAREA, MARCEL

    2013-01-01

    Ear keloids are among the most challenging plastic surgery conditions and may have significant psychosocial impact for the patient. Their aesthetic considerations are serious and despite a variety of treatment options, they often proved to be recurrent. This paper reviews the management options of ear keloids available in literature up to date. Multiple therapeutic options are discussed, such as: surgical treatment, corticosteroid injections, laser therapy, cryotherapy, radiotherapy, pressure therapy, therapy with antitumor or immunosuppressive agents. PMID:26527968

  5. SHIELD: an integrative gene expression database for inner ear research.

    PubMed

    Shen, Jun; Scheffer, Déborah I; Kwan, Kelvin Y; Corey, David P

    2015-01-01

    The inner ear is a highly specialized mechanosensitive organ responsible for hearing and balance. Its small size and difficulty in harvesting sufficient tissue has hindered the progress of molecular studies. The protein components of mechanotransduction, the molecular biology of inner ear development and the genetic causes of many hereditary hearing and balance disorders remain largely unknown. Inner-ear gene expression data will help illuminate each of these areas. For over a decade, our laboratories and others have generated extensive sets of gene expression data for different cell types in the inner ear using various sample preparation methods and high-throughput genome-wide approaches. To facilitate the study of genes in the inner ear by efficient presentation of the accumulated data and to foster collaboration among investigators, we have developed the Shared Harvard Inner Ear Laboratory Database (SHIELD), an integrated resource that seeks to compile, organize and analyse the genomic, transcriptomic and proteomic knowledge of the inner ear. Five datasets are currently available. These datasets are combined in a relational database that integrates experimental data and annotations relevant to the inner ear. The SHIELD has a searchable web interface with two data retrieval options: viewing the gene pages online or downloading individual datasets as data tables. Each retrieved gene page shows the gene expression data and detailed gene information with hyperlinks to other online databases with up-to-date annotations. Downloadable data tables, for more convenient offline data analysis, are derived from publications and are current as of the time of publication. The SHIELD has made published and some unpublished data freely available to the public with the hope and expectation of accelerating discovery in the molecular biology of balance, hearing and deafness. PMID:26209310

  6. Temporal bone meningioma involving the middle ear: A case report

    PubMed Central

    RICCIARDIELLO, FILIPPO; FATTORE, LUCIA; LIGUORI, MARIA ESTER; OLIVA, FLAVIA; LUCE, AMALIA; ABATE, TERESA; CARAGLIA, MICHELE; PIANESE, ANNALISA; RAUCCI, ALDO FALCO

    2015-01-01

    Meningioma is a common intracranial tumor involving the meninges. The localization of this type of tumor is rarely extracranial due to its typically low invasive properties. Furthermore, invasion of the middle ear is exceptional. The present study reported a case of meningioma extending into the middle ear from the middle cranial fossa through the tegmen tympani. The clinical and pathological characteristics, as well as the outcome of the patient, were described. PMID:26622828

  7. Prevalence of middle ear disorders in coal miners

    SciTech Connect

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

    1981-06-01

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

  8. Lumped parametric model of the human ear for sound transmission.

    PubMed

    Feng, Bin; Gan, Rong Z

    2004-09-01

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

  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. Pediatric lymphatic malformations: evolving understanding and therapeutic options.

    PubMed

    Defnet, Ann M; Bagrodia, Naina; Hernandez, Sonia L; Gwilliam, Natalie; Kandel, Jessica J

    2016-05-01

    Multimodal treatment of lymphatic malformations continues to expand as new information about the biology and genetics of these lesions is discovered, along with knowledge gained from clinical practice. A patient-centered approach, ideally provided by a multidisciplinary medical and surgical team, should guide timing and modality of treatment. Current treatment options include observation, surgery, sclerotherapy, radiofrequency ablation, and laser therapy. New medical and surgical therapies are emerging, and include sildenafil, propranolol, sirolimus, and vascularized lymph node transfer. The primary focus of management is to support and optimize these patients' quality of life. Researchers continue to study lymphatic malformations with the goal of increasing therapeutic options and developing effective clinical pathways for these complicated lesions. PMID:26815877

  11. Renal-hepatic-pancreatic dysplasia: an autosomal recessive malformation.

    PubMed Central

    Torra, R; Alós, L; Ramos, J; Estivill, X

    1996-01-01

    We report two brothers with a cystic malformation of the kidneys, liver, and pancreas. In both cases the malformation was fatal and the children died shortly after birth. The pathological findings, consisting of multicystic dysplastic kidneys, dilated and dysgenetic bile ducts, dilated pancreatic ducts, and polysplenia, correspond to those reported by Ivemark as renal-hepatic-pancreatic dysplasia. Many polymalformation syndromes include cystic affectation of these three organs, so this syndrome could be an isolated entity or a final common pathway of response of these organs to a variety of developmental disturbances, which could also include splenic abnormalities. We propose an autosomal recessive pattern of inheritance for renal-hepatic-pancreatic dysplasia. Images PMID:8733053

  12. The diagnosis and interventional management of pulmonary arteriovenous malformations.

    PubMed

    Morgan, Gareth J; Qureshi, Shakeel A

    2016-05-17

    Pulmonary arteriovenous malformations (PAVM) describe a broad group of complex vascular malformations, often associated with multi-system diseases and with palliated complex congenital heart disease. They can have major clinical consequences, resulting in strokes, cerebral abscesses, cyanosis and, infrequently, rupture into the pleural space. The best approach to their investigation and interventional treatment is via a multidisciplinary pathway which should be focused in high-volume centres with on-site thoracic and cardiovascular surgical back-up. The availability of computed tomography (CT) and a broad cathlab inventory aid procedural planning and success. The results of interventional treatment are very encouraging and are applicable to an increasingly broad group of patients thanks to improvements in interventional techniques and a significant expansion of the inventory of vascular occlusion devices. PMID:27174107

  13. Congenital pulmonary airway malformation: A report of two cases

    PubMed Central

    Bolde, Saroj; Pudale, Smita; Pandit, Gopal; Ruikar, Kirti; Ingle, Sachin B

    2015-01-01

    Congenital pulmonary airway malformation (CPAM), previously known as congenital cystic adenomatoid malformation is a congenital disorder of the lung similar to bronchopulmonary sequestration. In CPAM, usually an entire lobe of lung is replaced by a non-working cystic piece of abnormal lung tissue. This abnormal tissue will never function as normal lung tissue. The underlying cause for CPAM is not known. It occurs in approximately 1 in every 30000 pregnancies. The association between CPAM and malignancy has been well documented. There is a small risk (0.7%) of malignant transformation within the cyst. So early diagnosis and surgical resection is important to prevent the grave complications. Herein, we are reporting two interesting cases of CPAM and one belonged to Type II and other belonged to Type III of Stocker’s classification. PMID:25984523

  14. Congenital pulmonary airway malformation: A report of two cases.

    PubMed

    Bolde, Saroj; Pudale, Smita; Pandit, Gopal; Ruikar, Kirti; Ingle, Sachin B

    2015-05-16

    Congenital pulmonary airway malformation (CPAM), previously known as congenital cystic adenomatoid malformation is a congenital disorder of the lung similar to bronchopulmonary sequestration. In CPAM, usually an entire lobe of lung is replaced by a non-working cystic piece of abnormal lung tissue. This abnormal tissue will never function as normal lung tissue. The underlying cause for CPAM is not known. It occurs in approximately 1 in every 30000 pregnancies. The association between CPAM and malignancy has been well documented. There is a small risk (0.7%) of malignant transformation within the cyst. So early diagnosis and surgical resection is important to prevent the grave complications. Herein, we are reporting two interesting cases of CPAM and one belonged to Type II and other belonged to Type III of Stocker's classification. PMID:25984523

  15. Stereotactic radiosurgery with the linear accelerator: treatment of arteriovenous malformations.

    PubMed

    Betti, O O; Munari, C; Rosler, R

    1989-03-01

    An original stereotactic radiosurgical approach coupling a) Talairach's stereotactic methodology, b) a specially devised mechanical system, and c) a linear accelerator is detailed. The authors present their preliminary results on 66 patients with nonsurgical intracranial arteriovenous malformations. The doses delivered for treatment varied from 20 to 70 Gy. Doses of no more than 40 Gy were used in 80% of patients. An angiographic study was performed when the computed tomographic scan controls showed relevant modifications of the lesion volume. Total obliteration was obtained in 27 of the 41 patients (65.8%) who were followed up for at least 24 months. The percentage of the cured patients is significantly higher when a) the entire malformation is included in the 75% isodose (96%) and b) the maximum diameter of the lesion is less than 12 mm (81%). Two patients died of rebleeding at 18 and 29 months after treatment. PMID:2648180

  16. Behavioral effects of congenital ventromedial prefrontal cortex malformation

    PubMed Central

    2011-01-01

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

  17. 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

  18. Spontaneous thrombosis of a vein of galen malformation

    PubMed Central

    Mohanty, Chandan B.; Srinivas, Dwarakanath; Sampath, Somanna

    2016-01-01

    Vein of Galen malformation (VOGM) is a rare congenital vascular anomaly, comprising about 1% of all intracranial vascular anomalies, predominantly affecting the children less than 1 year of age. A 6-month-old infant presented with complaints of increasing head size of 3 months duration and multiple episodes of vomiting associated with refusal to feed since 7 days. He was a known case of VOGM who had initially refused treatment. Investigations revealed a spontaneously thrombosed VOGM with obstructive hydrocephalous. Child improved uneventfully with ventriculoperitoneal shunt. Spontaneous thrombosis of a VOGM is a rare occurrence and carries a better prognosis. The relevant literature is discussed with emphasis on etiopathogenesis, mechanism, and management of spontaneous thrombosis of the malformation. PMID:26889287

  19. Real ear unaided gain and its relation with the equivalent volume of the external and middle ear

    PubMed Central

    Bastos, Bárbara Guimarães; Ferrari, Deborah Viviane; Blasc, Wanderléia Quinhoneiro

    2012-01-01

    Summary Introduction: Old age is associated with changes in the characteristics of the middle ear transmission system and in external ear resonance, and these carry implications for the hearing aid (HA) verification process for which targets and measures of the real ear insertion gain (REIG) are used. Aim: To compare the real ear unaided gain (REUG) and the equivalent volumes of the external ear (VeqEE) and the middle ear (VeqME) between elderly and adult patients. Methods: This is a retrospective study in which the medical records of 28 elderly patients (aged between 61 and 102 years, average hearing thresholds between 38.75 and 85 dB HL) and 23 adult patients (aged 20–59, mean hearing thresholds between 31.25 and 116.25 dB HL) with bilateral sensorineural hearing loss and no history of middle ear abnormalities were analyzed. Immittance measurements (VeqEE, VeqME, and pressure of the peak of maximum compliance) and the REUG (frequency and amplitude of the primary peak) were recovered for a total of 40 ears. These data were compared between elderly and adults as well as between men and women, using Student's t test. Correlations (Pearson) between immittance and REUG data were also verified. Results: No statistically significant differences (p < 0.01) were found for immittance and REUG data between elderly and adults, or between men and women. A negative and weak but significant correlation was observed between the REUG primary peak and VeqEE. Conclusion: Hearing aid verification can be performed with target and measures of the REIG in the elderly population. PMID:25991959

  20. Novel Image-Guided Management of a Uterine Arteriovenous Malformation

    SciTech Connect

    Przybojewski, Stefan J. Sadler, David J.

    2011-02-15

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

  1. [Anorectal malformations: their diagnosis and the initial decisions].

    PubMed

    de Espinosa, H

    1994-05-01

    The author presents the salient aspects of anorectal malformation which allow us to simplify their anatomical diagnosis. In many cases only clinical means are used while in others adequate use of technical studies are necessary. This enables us to reach therapeutic decisions in the simplest and most orderly fashion so that patients with these anomalies may be given the opportunity of attaining normal or near normal function. PMID:7991806

  2. A malformed newborn with 9p and 4q trisomy.

    PubMed

    Fryns, J P; Azou, M; Devliegher, H; Eggermont, E; van den Berghe, H

    1981-01-01

    A malformed male newborn with partial trisomy 9p (qter-9q13) and distal 4q trisomy (4qter-4q31), being the unbalanced product of a balanced reciprocal translocation in the mother karyotype: 46,XX,t(4;9)(q31;q13) is reported. Besides the typical craniofacial stigmata of pure 9q trisomy the child presented with poor neurological condition and failure to thrive. PMID:6971619

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

    PubMed Central

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

    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 in unoperated patients. PMID:3958721

  4. 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

  5. Pulmonary arteriovenous malformation presenting as a large aneurysm

    PubMed Central

    Kamath, S Ganesh; Vivek, G; Borkar, Shirish; Ramachandran, Padmakumar

    2012-01-01

    We report the case of a 49-year-old woman who presented with breathlessness on exertion and iron deficiency anaemia. Evaluation revealed a large mass in the right lung which on further investigation was determined to be a pulmonary arteriovenous malformation (PAVM) with a large aneurysmal sac. The patient subsequently underwent a lobectomy with an uneventful recovery. The pathophysiology of PAVM and its varied presentations are also discussed. PMID:23076692

  6. FT-IR Microspectroscopy of Rat Ear Cartilage

    PubMed Central

    Vidal, Benedicto de Campos; Mello, Maria Luiza S.

    2016-01-01

    Rat ear cartilage was studied using Fourier transform-infrared (FT-IR) microspectroscopy to expand the current knowledge which has been established for relatively more complex cartilage types. Comparison of the FT-IR spectra of the ear cartilage extracellular matrix (ECM) with published data on articular cartilage, collagen II and 4-chondroitin-sulfate standards, as well as of collagen type I-containing dermal collagen bundles (CBs) with collagen type II, was performed. Ear cartilage ECM glycosaminoglycans (GAGs) were revealed histochemically and as a reduction in ECM FT-IR spectral band heights (1140–820 cm-1) after testicular hyaluronidase digestion. Although ear cartilage is less complex than articular cartilage, it contains ECM components with a macromolecular orientation as revealed using polarization microscopy. Collagen type II and GAGs, which play a structural role in the stereo-arrangement of the ear cartilage, contribute to its FT-IR spectrum. Similar to articular cartilage, ear cartilage showed that proteoglycans add a contribution to the collagen amide I spectral region, a finding that does not recommend this region for collagen type II quantification purposes. In contrast to articular cartilage, the symmetric stretching vibration of –SO3- groups at 1064 cm-1 appeared under-represented in the FT-IR spectral profile of ear cartilage. Because the band corresponding to the asymmetric stretching vibration of –SO3- groups (1236–1225 cm-1) overlapped with that of amide III bands, it is not recommended for evaluation of the –SO3- contribution to the FT-IR spectrum of the ear cartilage ECM. Instead, a peak (or shoulder) at 1027–1016 cm-1 could be better considered for this intent. Amide I/amide II ratios as calculated here and data from the literature suggest that protein complexes of the ear cartilage ECM are arranged with a lower helical conformation compared to pure collagen II. The present results could motivate further studies on this tissue under pathological or experimental states involving ear cartilage. PMID:27015280

  7. FT-IR Microspectroscopy of Rat Ear Cartilage.

    PubMed

    Vidal, Benedicto de Campos; Mello, Maria Luiza S

    2016-01-01

    Rat ear cartilage was studied using Fourier transform-infrared (FT-IR) microspectroscopy to expand the current knowledge which has been established for relatively more complex cartilage types. Comparison of the FT-IR spectra of the ear cartilage extracellular matrix (ECM) with published data on articular cartilage, collagen II and 4-chondroitin-sulfate standards, as well as of collagen type I-containing dermal collagen bundles (CBs) with collagen type II, was performed. Ear cartilage ECM glycosaminoglycans (GAGs) were revealed histochemically and as a reduction in ECM FT-IR spectral band heights (1140-820 cm-1) after testicular hyaluronidase digestion. Although ear cartilage is less complex than articular cartilage, it contains ECM components with a macromolecular orientation as revealed using polarization microscopy. Collagen type II and GAGs, which play a structural role in the stereo-arrangement of the ear cartilage, contribute to its FT-IR spectrum. Similar to articular cartilage, ear cartilage showed that proteoglycans add a contribution to the collagen amide I spectral region, a finding that does not recommend this region for collagen type II quantification purposes. In contrast to articular cartilage, the symmetric stretching vibration of -SO3- groups at 1064 cm-1 appeared under-represented in the FT-IR spectral profile of ear cartilage. Because the band corresponding to the asymmetric stretching vibration of -SO3- groups (1236-1225 cm-1) overlapped with that of amide III bands, it is not recommended for evaluation of the -SO3- contribution to the FT-IR spectrum of the ear cartilage ECM. Instead, a peak (or shoulder) at 1027-1016 cm-1 could be better considered for this intent. Amide I/amide II ratios as calculated here and data from the literature suggest that protein complexes of the ear cartilage ECM are arranged with a lower helical conformation compared to pure collagen II. The present results could motivate further studies on this tissue under pathological or experimental states involving ear cartilage. PMID:27015280

  8. 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

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

    PubMed Central

    Lloyd, J; Laurence, K M

    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 schedule. Three retrospective internal comparison groups were formed from those women who had also had a spontaneous abortion, previous stillbirth, or neonatal death or previous termination for medicosocial reasons early in pregnancy. Of the 48 women interviewed, 37 (77%) experienced an acute grief reaction after the index pregnancy was ended. This reaction was akin to that documented after stillbirth or neonatal death. Twenty two women (46%) remained symptomatic six months after the pregnancy had been ended, some requiring psychiatric support, compared with no such reaction after spontaneous abortion or termination for medicosocial reasons. All the women who had previously had a stillbirth or neonatal death were visited at home either by the general practitioner or by the midwife after that event but such follow up was limited to only eight of the study group after termination for fetal malformation. The findings suggest that support is inadequate for these patients and that improved follow up and counselling services may lessen the adverse sequelae of termination for fetal malformation. PMID:3919839

  10. Pathophysiological analyses of cortical malformation using gyrencephalic mammals

    PubMed Central

    Masuda, Kosuke; Toda, Tomohisa; Shinmyo, Yohei; Ebisu, Haruka; Hoshiba, Yoshio; Wakimoto, Mayu; Ichikawa, Yoshie; Kawasaki, Hiroshi

    2015-01-01

    One of the most prominent features of the cerebral cortex of higher mammals is the presence of gyri. Because malformations of the cortical gyri are associated with severe disability in brain function, the mechanisms underlying malformations of the cortical gyri have been of great interest. Combining gyrencephalic carnivore ferrets and genetic manipulations using in utero electroporation, here we successfully recapitulated the cortical phenotypes of thanatophoric dysplasia (TD) by expressing fibroblast growth factor 8 in the ferret cerebral cortex. Strikingly, in contrast to TD mice, our TD ferret model showed not only megalencephaly but also polymicrogyria. We further uncovered that outer radial glial cells (oRGs) and intermediate progenitor cells (IPs) were markedly increased. Because it has been proposed that increased oRGs and/or IPs resulted in the appearance of cortical gyri during evolution, it seemed possible that increased oRGs and IPs underlie the pathogenesis of polymicrogyria. Our findings should help shed light on the molecular mechanisms underlying the formation and malformation of cortical gyri in higher mammals. PMID:26482531

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

    SciTech Connect

    Sundelin, B.; Eriksson, A.K.

    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.

  12. Computational modelling for the embolization of brain arteriovenous malformations.

    PubMed

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

    2012-09-01

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

  13. Congenital malformations of the central nervous system in spontaneous abortions.

    PubMed Central

    Creasy, M R; Alberman, E D

    1976-01-01

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

  14. Acute Porphyria in a Patient with Arnold Chiari Malformation

    PubMed Central

    Shen, Jianbin; O’Keefe, Kevin; Webb, Lisa B.; DeGirolamo, Angela

    2015-01-01

    Patient: Female, 33 Final Diagnosis: Acute porphyria Symptoms: Abdominal pain • alternating bowel habits Medication: Metronidazole • bactrim • oxybutynin Clinical Procedure: EMG • porhyria workup Specialty: Neurology Objective: Rare disease Background: Acute porphyria and Arnold Chiari malformation are both uncommon genetic disorders without known association. The insidious onset, non-specific clinical manifestations, and precipitating factors often cause diagnosis of acute porphyria to be missed, particularly in patients with comorbidities. Case Report: A women with Arnold Chiari malformation type II who was treated with oxybutynin and antibiotics, including Bactrim for neurogenic bladder and recurrent urinary tract infection, presented with non-specific abdominal pain, constipation, and diarrhea. After receiving Flagyl for C. difficile colitis, the patient developed psychosis, ascending paralysis, and metabolic derangements. She underwent extensive neurological workup due to her congenital neurological abnormalities, most of which were unremarkable. As a differential diagnosis of Guillain Barré syndrome, acute porphyria was then considered and ultimately proved to be the diagnosis. After hematin administration and intense rehabilitation, the patient slowly recovered from the full-blown acute porphyria attack. Conclusions: This case report, for the first time, documents acute porphyria attack as a result of a sequential combination of 3 common medications. This is the first case report of the concomitant presence of both acute porphyria and Arnold Chiari malformation, 2 genetic disorders with unclear association. PMID:25697467

  15. Sonographic markers for early diagnosis of fetal malformations

    PubMed Central

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

    2013-01-01

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

  16. Sulindac metabolites decrease cerebrovascular malformations in CCM3-knockout mice

    PubMed Central

    Bravi, Luca; Rudini, Noemi; Cuttano, Roberto; Giampietro, Costanza; Maddaluno, Luigi; Ferrarini, Luca; Adams, Ralf H.; Corada, Monica; Boulday, Gwenola; Tournier-Lasserve, Elizabeth; Dejana, Elisabetta; Lampugnani, Maria Grazia

    2015-01-01

    Cerebral cavernous malformation (CCM) is a disease of the central nervous system causing hemorrhage-prone multiple lumen vascular malformations and very severe neurological consequences. At present, the only recommended treatment of CCM is surgical. Because surgery is often not applicable, pharmacological treatment would be highly desirable. We describe here a murine model of the disease that develops after endothelial-cell–selective ablation of the CCM3 gene. We report an early, cell-autonomous, Wnt-receptor–independent stimulation of β-catenin transcription activity in CCM3-deficient endothelial cells both in vitro and in vivo and a triggering of a β-catenin–driven transcription program that leads to endothelial-to-mesenchymal transition. TGF-β/BMP signaling is then required for the progression of the disease. We also found that the anti-inflammatory drugs sulindac sulfide and sulindac sulfone, which attenuate β-catenin transcription activity, reduce vascular malformations in endothelial CCM3-deficient mice. This study opens previously unidentified perspectives for an effective pharmacological therapy of intracranial vascular cavernomas. PMID:26109568

  17. Advances in ultrasound imaging for congenital malformations during early gestation

    PubMed Central

    Rayburn, William F.; Jolley, Jennifer A.; Simpson, Lynn L.

    2015-01-01

    With refinement in ultrasound technology, detection of fetal structural abnormalities has improved and there have been detailed reports of the natural history and expected outcomes for many anomalies. The ability to either reassure a high-risk woman with normal intrauterine images or offer comprehensive counseling and offer options in cases of strongly suspected lethal or major malformations has shifted prenatal diagnoses to the earliest possible gestational age. When indicated, scans in early gestation are valuable in accurate gestational dating. Stricter sonographic criteria for early nonviability guard against unnecessary intervention. Most birth defects are without known risk factors, and detection of certain malformations is possible in the late first trimester. The best time for a standard complete fetal and placental scan is 18–20 weeks. In addition, certain soft anatomic markers provide clues to chromosomal aneuploidy risk. Maternal obesity and multifetal pregnancies are now more common and further limit early gestation visibility. Other advanced imaging techniques during early gestation in select cases of suspected malformations include fetal echocardiography and magnetic resonance imaging. PMID:25820190

  18. Pathophysiological analyses of cortical malformation using gyrencephalic mammals.

    PubMed

    Masuda, Kosuke; Toda, Tomohisa; Shinmyo, Yohei; Ebisu, Haruka; Hoshiba, Yoshio; Wakimoto, Mayu; Ichikawa, Yoshie; Kawasaki, Hiroshi

    2015-01-01

    One of the most prominent features of the cerebral cortex of higher mammals is the presence of gyri. Because malformations of the cortical gyri are associated with severe disability in brain function, the mechanisms underlying malformations of the cortical gyri have been of great interest. Combining gyrencephalic carnivore ferrets and genetic manipulations using in utero electroporation, here we successfully recapitulated the cortical phenotypes of thanatophoric dysplasia (TD) by expressing fibroblast growth factor 8 in the ferret cerebral cortex. Strikingly, in contrast to TD mice, our TD ferret model showed not only megalencephaly but also polymicrogyria. We further uncovered that outer radial glial cells (oRGs) and intermediate progenitor cells (IPs) were markedly increased. Because it has been proposed that increased oRGs and/or IPs resulted in the appearance of cortical gyri during evolution, it seemed possible that increased oRGs and IPs underlie the pathogenesis of polymicrogyria. Our findings should help shed light on the molecular mechanisms underlying the formation and malformation of cortical gyri in higher mammals. PMID:26482531

  19. 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

  20. Two stage ear/microtia reconstruction using costal cartilage

    PubMed Central

    Balaji, S. M.

    2015-01-01

    Background: Reconstruction of Grade III microtia is a challenging entity in maxillofacial esthetic rehabilitation. Several advocacies and philosophies exist in this field. The aim of the manuscript is to present a single South Indian Experience with Ear reconstruction among South Indian Population. Materials and Methods: Retrospective analysis of unilateral Grade III microtia reconstruction was performed. Using a set of predefined inclusion and exclusion criteria, the population was selected. Outcome measures in terms of the ear size, auriculocephalic angle, and the conchal depth were measured in the reconstructed and normal side. Descriptive statistics is presented. Results: Twenty-four patients formed the study group and had undergone the classical two-stage reconstruction in a similar fashion. The mean ear size in normal side was 65.8 ± 2.8 mm whereas on the reconstructed side, it was 61.3 ± 5.8 mm. The center's technique achieved above 75% similarity as that of the other normal ear. The mean auriculocephalic angle was 44.6 ± 5.2° whereas for the surgically reconstructed ear, it was 41.9 ± 2.6°. Overall, in these patients, we achieved a 79.94% similarity of auriculocephalic angle in the reconstructed ear as compared to the normal auricle. The conchal depth was 19.2 ± 2.1 mm and 16.6 ± 1.9 mm for normal and reconstructed ear, respectively. In terms of conchal depth, the present study group showed an achievement of 82.88% of accuracy even after a prolonged follow-up. Discussion and Conclusion: The center employs a classic two stage reconstruction with a customized prosthesis that helps to avoid the loss of projection geometry and minimizes adhesion, infection, and early loss of structural stability. PMID:26981464