Science.gov

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. Inner ear malformations: a practical diagnostic approach.

    PubMed

    Mazón, M; Pont, E; Montoya-Filardi, A; Carreres-Polo, J; Más-Estellés, F

    2016-12-29

    Pediatric sensorineural hearing loss is a major cause of disability; although inner ear malformations account for only 20-40% of all cases, recognition and characterization will be vital for the proper management of these patients. In this article relevant anatomy and development of inner ear are surveyed. The role of neuroimaging in pediatric sensorineural hearing loss and cochlear preimplantation study are assessed. The need for a universal system of classification of inner ear malformations with therapeutic and prognostic implications is highlighted. And finally, the radiological findings of each type of malformation are concisely described and depicted. Computed tomography and magnetic resonance imaging play a crucial role in the characterization of inner ear malformations and allow the assessment of the anatomical structures that enable the selection of appropriate treatment and surgical approach.

  3. [Recurrent meningitis in inner ear malformations].

    PubMed

    Claros, Pedro; Matusialk, Monika

    2008-01-01

    Authors present two cases of children with reccurent meningitis and unilateral deafness. Implemented diagnostics (CT, NMR, ABR) revealed one side inner ear congenital malformation in one case and anterior fossa bony defect accompanied by labirynthine deformation in the other case. The presence of perilymphatic fistulae in oval and round windows and cerebrospinal fluid leakage has been confirmed in both cases during surgery. Carefull obliteration of the Eustachian tube and both windows has been performed. Non- complicated postoperative course (2 months and 6 years - respectively) has prooved the effectiveness of applied treatment.

  4. [The progress of inner ear malformation in radiological research].

    PubMed

    Kong, Dehua; Fu, Kuang; Zhao, Hui

    2016-01-01

    Inner ear malformations are anomalies linking to development insults at different periods of embryogenesis,which are common causes of congenital sensorineural hearing loss. The evaluation of pediatric sensorineural hearing loss mostly depends on high-resolution computed tomography and magnetic resonance imaging, which can excellently depict the temporal bones and inner ear malformations.

  5. Otosclerosis associated with type B-1 inner ear malformation.

    PubMed

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

    2010-06-01

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

  6. [Cochlear implant for malformations of the inner ear].

    PubMed

    Aschendorff, A; Laszig, R; Maier, W; Beck, R; Schild, C; Birkenhäger, R; Wesarg, T; Kröger, S; Arndt, S

    2009-06-01

    The radiologic evaluation of the temporal bone in cochlear implant candidates can detect malformations of the inner ear in up to 20% of cases. The aim of our study was to analyze and classify malformations of the inner ear in patients with cochlear implants carried out from 2001 to 2009. Malformations of the inner ear, including malformations of the internal auditory canal were detected in 12.7% of children and 3.4% of adults. Mondini dysplasia was most common and occurred in 45% of cases. The surgical procedure had to be adapted according to the individual malformation. Modification of surgical access, management of intraoperative CSF gusher, choice of electrode array, intraoperative imaging and the use of navigation were the most important factors. Rehabilitation results were generally very positive and corresponded to the expectation depending on the duration of deafness, if no additional handicaps were present.

  7. Congenital inner ear malformations without sensorineural hearing loss in children.

    PubMed

    Ozeki, Michio; Kato, Zenichiro; Sasai, Hideo; Kubota, Kazuo; Funato, Michinori; Orii, Kenji; Kaneko, Hideo; Fukao, Toshiyuki; Kondo, Naomi

    2009-10-01

    Inner ear malformations are frequently found in patients with congenital hearing loss. It has been reported that normal hearing is rare in patients with severe inner ear vestibular malformations. A 9-year-old boy had had complained of recurrent dizziness and disequilibrium for 2 months. Clinical and neuro-otological examinations showed peripheral involvement of the vestibular system, while audiological investigation was normal. High-resolution magnetic resonance imaging, with three-dimensional reconstruction, showed dysplasia of the bilateral lateral semicircular canals (LSCCs). Isolated vestibular malformation might not be as rare as previously thought, and should be examined by imaging of the temporal bone.

  8. 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

  9. 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.

  10. Congenital inner ear malformations without sensorineural hearing loss.

    PubMed

    Yukawa, Kumiko; Horiguchi, Satoshi; Suzuki, Mamoru

    2008-03-01

    It has been reported that normal hearing is rare in patients with severe inner ear vestibular malformations [Kokai H, Oohashi M, Ishikawa K, Harada K, Hiratsuka H, Ogasawara M et al. Clinical review of inner ear malformation. J Otolaryngol Jpn 2003;106(10):1038-44; Schuknecht HF. Mondini dysplasia. A clinical pathological study. Ann Otol Rhinol Laryngol 1980;89(Suppl. 65):1-23; Jackler RK, Luxford WM, House WF. Congenital malformations of the inner ear: a classification based on embryogenesis. Laryngoscope 1987;97:2-14; Phelps PD. Congenital lesions of the inner ear, demonstrated by tomography. Arch Otolaryngol 1974;100:11-8]. A 37-year-old woman had combined dysplasia of the posterior and lateral semicircular canals (PSCC, LSCC) with normal cochlear development and normal hearing in both ears. She had complained of dizziness for 8 months. High resolution computed tomography (CT) showed hypogenesis of the bony labyrinth in both ears. Bilateral PSCC and LSCC dysplasia and dilatation of the vestibule were detected. Magnetic resonant imaging (MRI) revealed that the deformity of the PSCC was more severe than the LSCC. Although the caloric test of the left ear elicited no nystagmus and there was reduced response in the right ear, the horizontal vestibulo-occular reflex (VOR) was present. Her dizzy sensation disappeared within 3 months without special treatment. The dizziness attack might have been caused by a temporary breakdown of her peripheral vestibular system.

  11. Cochlear implantation in the Mondini inner ear malformation.

    PubMed

    Miyamoto, R T; Robbins, A J; Myres, W A; Pope, M L

    1986-07-01

    We report the case of a profoundly deaf 4-year-old boy with congenital deafness as a result of Mondini's dysplasia. The Mondini inner ear malformation is the result of arrested labyrinthine development during embryogenesis and is characterized by both bony and membranous anomalies of the inner ear. The dysplastic cochlear anatomy does not preclude successful cochlear implantation, and electrical threshold measurements are similar to those recorded in pediatric subjects deafened as a result of other causes.

  12. Surgical management of major congenital malformations of the ear.

    PubMed

    Chiossone, E

    1985-05-01

    Surgical management of major congenital malformations of the ear is a difficult and complex procedure. The risk of damaging middle and inner ear structures because of their frequently aberrant location, the failure to keep a patent ear canal over the long term, and the difficulty in achieving a good hearing result all challenge the otologic surgeon. The purpose of this study was to analyze the results obtained with a surgical technique developed to prevent postoperative stenosis or total closure of the newly formed auditory meatus and to achieve at least serviceable hearing in the majority of cases. Emphasis is placed on the selection of surgical cases based on a classification that evaluates, through x-ray tomograms, the anatomic relation of the glenoid fossa of the temporomandibular joint to the middle ear space. Anatomic and functional results are presented. Comments are made in regard to different possibilities of reconstruction of the sound conducting mechanism.

  13. Cochlear implantation in inner ear malformations--a review article.

    PubMed

    Sennaroglu, Levent

    2010-03-01

    Inner ear malformations constitute about 20% of congenital sensorineural hearing loss. In this review article an updated classification of cochlear malformations is provided. Incomplete partition and cochlear hypoplasia cases are each divided further into three groups. There are two main difficulties in the surgery of inner ear malformations; gusher and facial nerve abnormalities. Radiological features of malformations necessary to identify these problems preoperatively are discussed. Facial nerve abnormalities that may occur are described. Two different types of cerebrospinal fluid leakage are defined and necessary measures to prevent leakage are described. Standard and modified surgical approaches to overcome the described problems are described with literature findings. Finally meningitis which may occur with and without cochlear implantation in this special group of patients is emphasized. This is common in incomplete partition type I patients and is usually due to a fistula in one of the windows (usually oval window) which occurs as a result of cerebrospinal fluid pressure. This is a medical emergency leading to potential meningitis and measures that should be taken to stop the leak as soon as possible are described.

  14. Malformations of the middle and inner ear on CT imaging in 22q11 deletion syndrome.

    PubMed

    Loos, Elke; Verhaert, Nicolas; Willaert, Annelore; Devriendt, Koenraad; Swillen, Ann; Hermans, Robert; Op de Beeck, Katya; Hens, Greet

    2016-11-01

    The 22q11 deletion syndrome (22q11DS), the most frequent microdeletion syndrome in humans, presents with a large variety of abnormalities. A common abnormality is hearing impairment. The exact pathophysiological explanation of the observed hearing loss remains largely unknown. The aim of this study was to analyze the middle and inner ear malformations as seen on computer tomographic imaging in patients with 22q11DS. We retrospectively reviewed the charts of 11 22q11DS patients who had undergone a CT of the temporal bone in the past. Of the 22 examined ears, two showed an abnormal malleus and incus, 10 presented with a dense stapes superstructure, and three ears had an abnormal orientation of the stapes. With regard to the inner ear, 12 ears showed an incomplete partition type II with a normal vestibular aqueduct. In four ears the vestibule and lateral semicircular canal were composed of a single cavity, in 14 ears the vestibule was too wide, and three ears had a broadened lateral semicircular canal. These findings suggest that malformations of the stapes, cochlea, vestibule, and lateral semicircular canal are frequent in 22q11DS. To our knowledge, the current study involves the largest case series describing middle and inner ear malformations in 22q11DS. © 2016 Wiley Periodicals, Inc.

  15. Auditory maturity and hearing performance in inner ear malformations: a histological and electrical stimulation approach.

    PubMed

    Sainz, Manuel; Garcia-Valdecasas, Juan; Fernandez, Elena; Pascual, Maria Teresa; Roda, Olga

    2012-06-01

    The objective of this study was to assess the auditory performance of the neural structures in response to controlled electrical stimulation period. A prospective cohort study focused on the intracochlear electrical stimulation parameters and hearing performance of patients suffering different cochlear malformations who were treated by cochlear implants constituted the study design. The study sample constituted 16 patients, suffering profound prelingual hearing impairment, diagnosed on the basis of radiological criteria as having an inner ear malformation, and who underwent cochlear implantation and were followed for 24 months. Patients with common cavities, characterized by fewer nerve structures involved, less epithelial penetration, and deficient cochlear tonotopy distribution showed have higher thresholds and electrical charges than patients with cochlear hypoplasia, who in turn have higher thresholds than patients with minor malformations (p < 0.05). Furthermore, word perception was severely compromised in patients with a common cavity malformation and was also poor in patients with cochlear hypoplasia, who were unable to discriminate more than 50% of the words and relied on visual cues as a necessary aid to communication. Better results were reached by minor malformed inner ears. To conclude, the number of nerve structures involved, epithelial penetration and deficient cochlear tonotopy are responsible of inner ear functionality.

  16. 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.

  17. Cochlear Implantation in Inner Ear Malformations: Systematic Review of Speech Perception Outcomes and Intraoperative Findings.

    PubMed

    Farhood, Zachary; Nguyen, Shaun A; Miller, Stephen C; Holcomb, Meredith A; Meyer, Ted A; Rizk, And Habib G

    2017-03-01

    Objective (1) To analyze reported speech perception outcomes in patients with inner ear malformations who undergo cochlear implantation, (2) to review the surgical complications and findings, and (3) to compare the 2 classification systems of Jackler and Sennaroglu. Data Sources PubMed, Scopus (including Embase), Medline, and CINAHL Plus. Review Methods Fifty-nine articles were included that contained speech perception and/or intraoperative data. Cases were differentiated depending on whether the Jackler or Sennaroglu malformation classification was used. A meta-analysis of proportions examined incidences of complete insertion, gusher, and facial nerve aberrancy. For speech perception data, weighted means and standard deviations were calculated for all malformations for short-, medium-, and long-term follow-up. Speech tests were grouped into 3 categories-closed-set words, open-set words, and open-set sentences-and then compared through a comparison-of-means t test. Results Complete insertion was seen in 81.8% of all inner ear malformations (95% CI: 72.6-89.5); gusher was reported in 39.1% of cases (95% CI: 30.3-48.2); and facial nerve anomalies were encountered in 34.4% (95% CI: 20.1-50.3). Significant improvements in average performance were seen for closed- and open-set tests across all malformation types at 12 months postoperatively. Conclusions Cochlear implantation outcomes are favorable for those with inner ear malformations from a surgical and speech outcome standpoint. Accurate classification of anatomic malformations, as well as standardization of postimplantation speech outcomes, is necessary to improve understanding of the impact of implantation in this difficult patient population.

  18. 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.

  19. 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.

  20. 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

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

    PubMed

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

    2014-11-01

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

  2. 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

  3. Congenital malformations of the ear and cochlear implantation in children: review and temporal bone report of common cavity.

    PubMed

    Graham, J M; Phelps, P D; Michaels, L

    2000-01-01

    The objective of this review is to analyze aspects of congenital malformation of the ear in relation to cochlear implantation in children. Having briefly described the in utero development of the ear and the classification of types of external, middle and inner ear malformation, five practical aspects of these malformations are discussed. It seems likely that the combination of bilateral profound sensorineural deafness with bilateral microtia severe enough to make a surgical approach to the cochlea difficult will be extremely uncommon. No such cases have been reported, although Klippel-Feil deformity seems the syndrome most likely to produce this set of circumstances. Abnormalities in the intratympanic course of the facial nerve have been associated with cochlear malformation, emphasizing the benefit of intra-operative facial nerve monitoring, and a technique suggested for safely avoiding an abnormally placed nerve. Fistulae of cerebrospinal fluid (CSF) and perilymph can complicate surgery and are relatively common in common cavity and Mondini malformations. Strategies for facilitating surgery in the presence of 'gushers', for measuring the pressure of a gusher and for placement of the cochlear implant electrode array are reviewed, with reports of fluctuating levels of electric current when implants lie in dysplastic cochleas. The relationship of implant performance to VIIIth nerve tissue in malformed cochleas is discussed, with a description of the histological findings in a common cavity cochlea. Techniques for identifying the absence of the cochlear nerve are reviewed. Stimulation of the facial nerve by cochlear implants has been described in cases of congenital malformation of the labyrinth but is relatively uncommon. Case reports of the benefit received by implanted children with congenital cochlear malformation have appeared since 1988. Most cases reported have not yet been followed for long enough to establish a clear picture of the outcome following

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

    PubMed

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

    2014-01-01

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

  5. Middle and inner ear malformations in mutation-proven branchio-oculo-facial (BOF) syndrome: case series and review of the literature.

    PubMed

    Carter, Melissa T; Blaser, Susan; Papsin, Blake; Meschino, Wendy; Reardon, Willie; Klatt, Regan; Babul-Hirji, Riyana; Milunsky, Jeff; Chitayat, David

    2012-08-01

    Hearing impairment is common in individuals with branchio-oculo-facial (BOF) syndrome. The majority of described individuals have conductive hearing impairment due to malformed ossicles and/or external canal stenosis or atresia, although a sensorineural component to the hearing impairment in BOF syndrome is increasingly being reported. Sophisticated computed tomography (CT) of the temporal bone has revealed middle and inner ear malformations in three previous reports. We present middle and inner ear abnormalities in three additional individuals with mutation-proven BOF syndrome. We suggest that temporal bone CT imaging be included in the medical workup of a child with BOF syndrome, in order to guide management.

  6. Severe Klippel-Feil syndrome with Mondini malformation of inner ear

    PubMed Central

    Alaqeel, Aqeel Abdullah

    2014-01-01

    Klippel-Feil syndrome is defined as the fusion of cervical vertebra with associated congenital anomalies but was rarely reported to be associated with Mondini Malformation. We report a newborn girl with severe neck extension, computed tomography (CT) of the neck after birth showed fusion of the fifth, sixth, and seventh cervical vertebrae, compatible with Klippel-Feil Syndrome and CT temporal bone showed choclear dysplasia with incomplete number of turns that is compatible with Mondini Malformation. PMID:25852797

  7. Severe Klippel-Feil syndrome with Mondini malformation of inner ear.

    PubMed

    Alaqeel, Aqeel Abdullah

    2014-01-01

    Klippel-Feil syndrome is defined as the fusion of cervical vertebra with associated congenital anomalies but was rarely reported to be associated with Mondini Malformation. We report a newborn girl with severe neck extension, computed tomography (CT) of the neck after birth showed fusion of the fifth, sixth, and seventh cervical vertebrae, compatible with Klippel-Feil Syndrome and CT temporal bone showed choclear dysplasia with incomplete number of turns that is compatible with Mondini Malformation.

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

    PubMed

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

    2015-07-01

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

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

    PubMed

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

    2012-11-01

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

  10. Ear Tumors

    MedlinePlus

    ... Outer Ear Ear Blockages Ear Tumors External Otitis (Swimmer's Ear) Malignant External Otitis Perichondritis Tumors of the ... Outer Ear Ear Blockages Ear Tumors External Otitis (Swimmer's Ear) Malignant External Otitis Perichondritis NOTE: This is ...

  11. Ear Infection (Middle Ear)

    MedlinePlus

    Ear infection (middle ear) Overview By Mayo Clinic Staff An ear infection (acute otitis media) is most often a bacterial or viral infection that affects the middle ear, the air-filled space behind the eardrum that ...

  12. Pierced Ears

    MedlinePlus

    ... Room? What Happens in the Operating Room? Pierced Ears KidsHealth > For Kids > Pierced Ears A A A ... cool, but infected ears do not! Getting Your Ears Pierced It's important to get your ears pierced ...

  13. Swimmer's Ear

    MedlinePlus

    ... de los dientes Video: Getting an X-ray Swimmer's Ear KidsHealth > For Kids > Swimmer's Ear Print A ... continue How Do I Know if I Have Swimmer's Ear? Swimmer's ear may start with some itching, ...

  14. Ear Tubes

    MedlinePlus

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

  15. Ear Disorders

    MedlinePlus

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

  16. Your Ears

    MedlinePlus

    ... gross and useful. continue The Middle Ear: Good Vibrations After sound waves enter the outer ear, they ... take those sound waves and turn them into vibrations that are delivered to the inner ear. To ...

  17. Ear emergencies

    MedlinePlus

    ... an ear injury, avoid nose blowing and getting water in the injured ear. Treat ear infections right ... FDR Medical Services/Millard Fillmore Suburban Hospital, Buffalo, NY. Also reviewed by David Zieve, MD, MHA, ...

  18. Ear barotrauma

    MedlinePlus

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

  19. 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…

  20. Congenital malformations of the temporal bone.

    PubMed

    Mukerji, Shraddha S; Parmar, Hemant A; Ibrahim, Mohannad; Mukherji, Suresh K

    2011-08-01

    Congenital ear or temporal bone malformations are a diagnostic challenge to radiologists and surgeons alike. Newer imaging techniques can detect subtle changes in middle ear and cochlear anatomy. This information is invaluable with increasing use of hearing restoration surgeries and/or cochlear implants in such patients. This article discusses the embryogenesis, classification system, and salient imaging findings of congenital outer, middle ear, and inner ear anomalies in children. Both high-resolution computerized tomography and magnetic resonance imaging scans of the temporal bones are described.

  1. Ear trauma.

    PubMed

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

    2013-04-01

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

  2. 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, ...

  3. Cochlear Implantation in Children with Cochlear Malformation.

    PubMed

    Saikawa, Etsuko; Takano, Kenichi; Ogasawara, Noriko; Tsubomatsu, Chieko; Takahashi, Nozomi; Shirasaki, Hideaki; Himi, Tetsuo

    2016-01-01

    Cochlear implantation (CI) has proven to be an effective treatment for severe bilateral sensorineural hearing loss (SNHL). Inner ear malformation is a rare anomaly and occurs in approximately 20% of cases with congenital SNHL. In cases with cochlear malformation, CI can be successfully performed in nearly all patients, the exceptions being those with complete labyrinthine and cochlear aplasia. It is important to evaluate the severity of inner ear deformity and other associated anomalies during the preimplantation radiological assessment in order to identify any complication that may potentially occur during the surgery and subsequent patient management.

  4. Ear Infections

    MedlinePlus

    ... surgery. An ENT surgically inserts tubes inside your child’s middle ear. The tubes relieve the pressure and allow ... the risks of surgically inserting tubes inside my child's middle ear? What are the risks of not?Should ...

  5. 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? ...

  6. Ear tag

    MedlinePlus

    ... the opening of the ear are common in newborn infants. In most cases, these are normal. However, they ... M. Editorial team. Related MedlinePlus Health Topics Common Infant and Newborn Problems Ear Disorders Skin Conditions Browse the Encyclopedia ...

  7. Ear examination

    MedlinePlus

    ... page: //medlineplus.gov/ency/article/003340.htm Ear examination To use the sharing features on this page, ... ear References King EF, Couch ME. History, physical examination, and the preoperative evaluation. In: Flint PW, Haughey ...

  8. Ear wax

    MedlinePlus

    ... wax plug. Tip your head to allow the water to drain. You may need to repeat irrigation several times. To avoid damaging your ear or causing an infection: Never irrigate the ear if the eardrum may have a hole in it. Do not irrigate the ear with ...

  9. Cauliflower Ear

    MedlinePlus

    ... Room? What Happens in the Operating Room? What's Cauliflower Ear? KidsHealth > For Kids > What's Cauliflower Ear? A A A Have you ever seen ... looks bumpy and lumpy? The person might have cauliflower ear. That sure is a funny name. Let's ...

  10. Cauliflower Ear

    MedlinePlus

    ... los dientes Video: Getting an X-ray What's Cauliflower Ear? KidsHealth > For Kids > What's Cauliflower Ear? Print A A A Have you ever ... looks bumpy and lumpy? The person might have cauliflower ear. That sure is a funny name. Let's ...

  11. Congenital Auricular Malformations: Description of Anomalies and Syndromes.

    PubMed

    Bartel-Friedrich, Sylva

    2015-12-01

    Half of the malformations in the ear, nose, and throat region affect the ear. Malformations of the external ear (pinna or auricle with external auditory canal [EAC]) are collectively termed microtia. Microtia is a congenital anomaly that ranges in severity from mild structural abnormalities to complete absence of the external ear (anotia). Microtia occurs more frequently in males (∼2 or 3:1), is predominantly unilateral (∼70-90%), and more often involves the right ear (∼60%). The reported prevalence varies geographically from 0.83 to 17.4 per 10,000 births. Microtia may be genetic (with family history, spontaneous mutations) or acquired. Malformations of the external ear can also involve the middle ear and/or inner ear. Microtia may be an isolated birth defect, but associated anomalies or syndromes are described in 20 to 60% of cases, depending on study design. These generally fit within the oculo-auriculo-vertebral spectrum; defects are located most frequently in the facial skeleton, facial soft tissues, heart, and vertebral column, or comprise a syndrome (e.g., Treacher Collins syndrome). Diagnostic investigation of microtia includes clinical examination, audiologic testing, genetic analysis and, especially in higher grade malformations with EAC deformities, computed tomography (CT) or cone-beam CT for the planning of surgery and rehabilitation procedures, including implantation of hearing aids.

  12. Glomuvenous malformations.

    PubMed

    Henning, J Scott; Kovich, Olympia I; Schaffer, Julie V

    2007-01-27

    A 9-year-old girl presented with a congenital, blue-purple, partially compressible plaque with a cobblestone surface on the left lateral foot and ankle. Similar, solitary, blue nodules later appeared elsewhere on the extremities. The lesions were tender to palpation and were associated with spontaneous paroxysms of pain and paresthesias. Histopathologic evaluation of a skin biopsy specimen showed rows of glomus cells that surrounded thin-walled vascular channels, which confirmed the diagnosis of glomuvenous malformations. This autosomal dominant condition, which is due to mutations in the GLMN gene, presents with clinical findings that are distinct from those of familial, multiple, cutaneous and mucosal venous malformations. Treatment options include excision, sclerotherapy, and laser therapy (ablative or pulsed dye).

  13. Ear Problems

    MedlinePlus

    ... YesNoDo you have thick pus-filled or bloody drainage from the ear canal that started after a ... bone behind the ear, or from an ENLARGED LYMPH NODE.Self CareURGENTSEE YOUR DOCTOR RIGHT AWAY.Start ...

  14. Swimmer's ear

    MedlinePlus

    ... or a respiratory infection such as a cold. Swimming in unclean water can lead to swimmer's ear. ... very well after it has gotten wet. Avoid swimming in polluted water. Use earplugs when swimming. Try ...

  15. Pierced Ears

    MedlinePlus

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

  16. CT and MR imaging of congential abnormalities of the inner ear and internal auditory canal.

    PubMed

    Casselman, J W; Offeciers, E F; De Foer, B; Govaerts, P; Kuhweide, R; Somers, T

    2001-11-01

    The embryology of the inner ear must be known as many of the inner ear malformations present as a result of the arrest during the various stages of embryology. These malformations are described in this "embryologic" perspective and specific names for certain malformations are no longer used. Both CT and MR can be used to look at inner ear malformations but often both techniques are complementary. However, CT is preferred when associated middle- or external ear malformations must be excluded. Magnetic resonance is preferred when subtle changes in the membranous labyrinth or abnormalities of the nerves in the internal auditory canal must be visualised. The CT and MR technique must however be adapted as more and more subtle congenital malformations can only be seen when the right technique is used. The heavily T2-weighted gradient-echo or fast spin-echo MR techniques are mandatory if malformations of the inner ear must be excluded. The purpose of this paper is to describe the techniques used to study these patients and to give an overview of the most frequent and important congenital malformations which can be found in the inner ear and internal auditory canal/cerebellopontine angle.

  17. Anorectal malformations

    PubMed Central

    Levitt, Marc A; Peña, Alberto

    2007-01-01

    Anorectal malformations comprise a wide spectrum of diseases, which can affect boys and girls, and involve the distal anus and rectum as well as the urinary and genital tracts. They occur in approximately 1 in 5000 live births. Defects range from the very minor and easily treated with an excellent functional prognosis, to those that are complex, difficult to manage, are often associated with other anomalies, and have a poor functional prognosis. The surgical approach to repairing these defects changed dramatically in 1980 with the introduction of the posterior sagittal approach, which allowed surgeons to view the anatomy of these defects clearly, to repair them under direct vision, and to learn about the complex anatomic arrangement of the junction of rectum and genitourinary tract. Better imaging techniques, and a better knowledge of the anatomy and physiology of the pelvic structures at birth have refined diagnosis and initial management, and the analysis of large series of patients allows better prediction of associated anomalies and functional prognosis. The main concerns for the surgeon in correcting these anomalies are bowel control, urinary control, and sexual function. With early diagnosis, management of associated anomalies and efficient meticulous surgical repair, patients have the best chance for a good functional outcome. Fecal and urinary incontinence can occur even with an excellent anatomic repair, due mainly to associated problems such as a poorly developed sacrum, deficient nerve supply, and spinal cord anomalies. For these patients, an effective bowel management program, including enema and dietary restrictions has been devised to improve their quality of life. PMID:17651510

  18. 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 ...

  19. [Diagnosis and management of Mondini malformation].

    PubMed

    Yang, W; Fang, Y; Yang, S

    1997-02-01

    Owing to the development of imaging technology and audiology, some of the sensorineural hearing loss cases that were previcusly considered to be of unknown cause have been found to be inner ear malformation. Five cases of Mondini malformation are reviewed in this paper. CSF otorhinorrhea occurred in four cases, Klippel-Feil syndrome in three, and concurrent otosclerosis in one patient. In discussion, the authors point out that: 1) CT scanning is an supplement to audiologic tests for such patients; 2) perilymph fistula in Mondini malformation is often found at the oval window and its vicinity, the foot plate and the round window; 3) the fistula can be treated by plugging with fascial tissue via tympanoplastic approach with endaural incision. The mucosa around the fistula should be stripped away and the plugging tissue should be of dumb-bell shape.

  20. Meningitis after cochlear implantation in Mondini malformation.

    PubMed

    Page, E L; Eby, T L

    1997-01-01

    Although the potential for CSF leakage and subsequent meningitis after cochlear implantation in the malformed cochlea has been recognized, this complication has not been previously reported. We report a case of CSF otorhinorrhea and meningitis after minor head trauma developing 2 years after cochlear implantation in a child with Mondini malformation. Leakage of CSF was identified from the cochleostomy around the electrode of the implant, and this leak was sealed with a temporalis fascia and muscle plug. Although this complication appears to be rare, care must be taken to seal the cochleostomy in children with inner ear malformations at the initial surgery, and any episode of meningitis after surgery must be thoroughly investigated to rule out CSF leakage from the labyrinth.

  1. 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.

  2. Ear tube insertion - slideshow

    MedlinePlus

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

  3. What Is an Ear Infection?

    MedlinePlus

    ... the germs bother your outer ear, it's called swimmer's ear. The middle ear is a small pocket ... What's Hearing Loss? Taking Care of Your Ears Swimmer's Ear Perforated Eardrum What's Earwax? Contact Us Print ...

  4. Swimmer's Ear (For Parents)

    MedlinePlus

    ... Feeding Your 1- to 2-Year-Old 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 — ...

  5. 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

  6. Pediatric neuroanesthesia. Arteriovenous malformations.

    PubMed

    Newfield, P; Hamid, R K

    2001-06-01

    Intracranial arteriovenous malformations can occur singly, multiply, and in conjunction with aneurysms and denovo, family, or in conjunction with connective-tissue disorders. Intracranial hemorrhage is the most common presentation, occurring in 20% to 50% of cases. In children, seizures are the second most common presentation occurring in 15% to 20% of cases. The modalities available treatment of arteriovenous malformations are microsurgery, embolization, and stereotactic radiosurgery with heavy particles, alpha knife, or linear accelerator. Induction, maintenance, and emergence from anesthesia are designed to prevent rupture of arteriovenous malformation and aneurysm and to improve intracranial compliance in the presence of an intracranial hematoma, during both diagnostic (CT, MR scanning) and therapeutic procedures.

  7. Pelvic Vascular Malformations

    PubMed Central

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

    2013-01-01

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

  8. CT and MR imaging of the inner ear and brain in children with congenital sensorineural hearing loss.

    PubMed

    Joshi, Varsha M; Navlekar, Shantanu K; Kishore, G Ravi; Reddy, K Jitender; Kumar, E C Vinay

    2012-01-01

    Imaging plays an important role in the evaluation of congenital sensorineural hearing loss. In children who are candidates for cochlear implantation surgery, it provides vital preoperative information about the inner ear, the vestibulocochlear nerve, and the brain. High-resolution computed tomography (CT) and magnetic resonance (MR) imaging provide excellent delineation of the intricate anatomy of the inner ear: CT depicts the minute details of osseous structures, and MR imaging allows visualization of the fluid-filled spaces and the vestibulocochlear nerve. Together, these complementary modalities can aid decision making about the best management strategy by facilitating the identification and characterization of inner ear malformations and any associated neurologic abnormalities. It is important that the radiologist be familiar with the key imaging features when interpreting CT and MR images obtained in this patient group. A broad spectrum of inner ear malformations have been described and linked to developmental insults at different stages of embryogenesis, and various systems have been proposed for classifying them. In this article, these malformations are described by using classification systems used by otolaryngologists for ease of interpretation. The relevant normal anatomy and development of the inner ear are briefly surveyed, standard imaging protocols for studying the inner ear are reviewed, and the imaging appearances of frequently observed inner ear malformations are described and illustrated. The impact of the identification of these malformations and commonly associated brain abnormalities on clinical management and prognosis also is discussed.

  9. Otoplasty (Cosmetic Ear Surgery)

    MedlinePlus

    ... is typically done on both ears to optimize symmetry. Otoplasty can be done at any age after ... your ears — including their placement, size, shape and symmetry. The doctor might also take pictures of your ...

  10. 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 ...

  11. Ear drainage culture

    MedlinePlus

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

  12. Swimmer's Ear (External Otitis)

    MedlinePlus

    ... ears. This is especially true if they use cotton swabs or dangerously sharp small objects, like hair ... all objects out of your ear canals — including cotton swabs — unless your doctor has told you it's ...

  13. 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 ...

  14. Swimmer's Ear (For Parents)

    MedlinePlus

    ... or inserting foreign objects like bobby pins or paper clips into the ear can all increase the ... discharge from the ear to help identify which bacteria or fungi are causing the infection. Over-the- ...

  15. Ear surgery - slideshow

    MedlinePlus

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

  16. Swimmer's Ear (External Otitis)

    MedlinePlus

    ... can be caused by many different types of bacteria or fungi. It usually develops in ears that are exposed to moisture. People who get OE often have been diving or swimming for long periods of time. This can bring infectious bacteria directly into the ear canal. Swimmer's ear occurs ...

  17. Measurements of normal inner ear on computed tomography in children with congenital sensorineural hearing loss.

    PubMed

    Lan, Ming-Ying; Shiao, Jiun-Yih; Ho, Ching-Yin; Hung, Hao-Chun

    2009-09-01

    The objective of this study is to use standardized measurements of the inner ear to see whether there are subtle bony malformations in children with congenital sensorineural hearing loss (SNHL) whose temporal bone computed tomography (CT) are grossly normal. The study includes 45 ears with congenital SNHL and grossly normal temporal bone CT scans and 45 ears with normal inner ear structures and normal hearing. Standardized measurements of the inner ear structures were made on axial temporal bone CT scans. Student's t test was performed to compare the measurements of the two groups. There were significant differences in the measurements of the bony island width of the superior semicircular canal, bony island width of the lateral semicircular canal and maximal height of cochlea between two groups (P < 0.05). In conclusion, standardized measurements of bony labyrinth of inner ear on temporal bone CT can identify subtle abnormalities of inner ear in patients with congenital SNHL having grossly normal radiological images.

  18. [Middle ear physiology].

    PubMed

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

    1999-01-01

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

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

    PubMed

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

    2006-07-01

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

  20. [Multiple intracranial arteriovenous malformation].

    PubMed

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

    2015-01-01

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

  1. Rare malformation of glans penis: arteriovenous malformation.

    PubMed

    Akin, Y; Sarac, M; Yucel, S

    2013-01-01

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

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

    MedlinePlus

    ... Year-Old 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 ...

  3. Pathology of the Ear

    PubMed Central

    Orengo, Ida; Robbins, Kerri; Marsch, Amanda

    2011-01-01

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

  4. Proteus Syndrome with Arteriovenous Malformation

    PubMed Central

    Asilian, Ali; Kamali, Atefeh Sadat; Riahi, Nabet Tajmir; Adibi, Neda; Mokhtari, Fatemeh

    2017-01-01

    Proteus syndrome is a rare sporadic disorder that appears with localized macrosomia, congenital lipomatosis, and slow flow vascular malformations, connective tissue nevus, and epidermal nevus. There are usually some manifestations at birth. The vascular abnormalities that have been reported in Proteus syndrome are capillary and slow flow venous malformation. We report a case of a 10-year-old boy with confirmed Proteus syndrome characterized by high flow vascular malformation (arteriovenous [AV] malformation) unlike the usual vascular malformations seen in this syndrome. This case adds a new perspective to the established clinical findings of the Proteus syndrome.

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

    MedlinePlus

    ... 2-Year-Old Middle Ear Infections and Ear Tube Surgery KidsHealth > For Parents > Middle Ear Infections and ... to 18 months or longer. previous continue Tympanostomy Tube Surgery If your child is old enough to ...

  6. Caring for Pierced Ears

    MedlinePlus

    ... close. Regularly wash your ears with soap and water. Carefully do this at least once a day to avoid infection. Twist the earrings a few times daily. This will help keep the pierced holes open. Put rubbing alcohol on your ears. Using ...

  7. Avoiding Infection After Ear Piercing

    MedlinePlus

    ... Text Size Email Print Share Avoiding Infection After Ear Piercing Page Content Article Body What is the best way to avoid infection after ear piercing? Ears may be pierced for cosmetic reasons ...

  8. Ear - blocked at high altitudes

    MedlinePlus

    ... and blocked ears; Flying and blocked ears; Eustachian tube dysfunction - high altitude ... The eustachian tube is a connection between the middle ear (the space deep to the eardrum) and the back of the ...

  9. Soft palate hypoplasia and concurrent middle ear pathology in six dogs.

    PubMed

    White, R N; Hawkins, H L; Alemi, V P; Warner, C

    2009-07-01

    This retrospective clinical study describes six consecutive cases of bilateral hypoplasia/malformation of the soft palate in dogs in which associated middle ear disease was investigated and the palatine defects were surgical repaired. Radiographic abnormalities of the tympanic bullae were seen in both ears of all six dogs (12 of 12). Negative tympanocentesis findings were recorded in 11 of 12 ears. A purulent otitis media was confirmed in one ear of one dog, and loss of hearing was also demonstrated in this ear on brainstem auditory evoked response hearing assessment. There was no evidence of hearing loss on brainstem auditory evoked response in any of the remaining ears. Surgical repair of the soft palate defect was undertaken in all six dogs. Long-term assessment of the clinical outcome was considered excellent in five dogs and reasonable in one dog (mean 18 months, range seven to 27 months). It would appear that surgical intervention for the treatment of bilateral palatine malformation/hypoplasia may be associated with a better prognosis than reported previously. The lack of middle ear effusion and associated hearing impairment suggests that the underlying aetiology of middle ear pathology in dogs suffering from congenital palatine defects may be different from that observed in human beings. The true nature of the radiographic bullae changes seen in dogs with soft palate defects remains unclear.

  10. Effectiveness of Ear Splint Therapy for Ear Deformities

    PubMed Central

    2017-01-01

    Objective To present our experience with ear splint therapy for babies with ear deformities, and thereby demonstrate that this therapy is an effective and safe intervention without significant complications. Methods This was a retrospective study of 54 babies (35 boys and 19 girls; 80 ears; age ≤3 months) with ear deformities who had received ear splint therapy at the Center for Torticollis, Department of Physical Medicine and Rehabilitation, Ajou University Hospital between December 2014 and February 2016. Before the initiation of ear splint therapy, ear deformities were classified with reference to the standard terminology. We compared the severity of ear deformity before and after ear splint therapy by using the physician's ratings. We also compared the physician's ratings and the caregiver's ratings on completion of ear splint therapy. Results Among these 54 babies, 41 children (58 ears, 72.5%) completed the ear splint therapy. The mean age at initiation of therapy was 52.91±18.26 days and the treatment duration was 44.27±32.06 days. Satyr ear, forward-facing ear lobe, Darwinian notch, overfolded ear, and cupped ear were the five most common ear deformities. At the completion of therapy, the final physician's ratings of ear deformities were significantly improved compared to the initial ratings (8.28±1.44 vs. 2.51±0.92; p<0.001). There was no significant difference between the physician's ratings and the caregiver's ratings at the completion of ear splint therapy (8.28±1.44 vs. 8.0±1.61; p=0.297). Conclusion We demonstrated that ear splint therapy significantly improved ear deformities in babies, as measured by quantitative rating scales. Ear splint therapy is an effective and safe intervention for babies with ear deformities. PMID:28289646

  11. Ear problems in swimmers.

    PubMed

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

    2005-08-01

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

  12. 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.

  13. 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.

  14. Ear Injuries (For Parents)

    MedlinePlus

    ... head, sports injuries, and even listening to loud music can cause ear damage, which can affect hearing ... But for kids and teens, listening to loud music (at concerts, in the car, through headphones) is ...

  15. Ear infection - chronic

    MedlinePlus

    ... Paralysis of the face Inflammation around the brain ( epidural abscess ) or in the brain Damage to the part ... pubmed/23818543 . Read More Cholesteatoma Ear infection - acute Epidural abscess Mastoiditis Otitis Review Date 4/21/2015 Updated ...

  16. Ear tube insertion

    MedlinePlus

    ... Ear tube surgery - what to ask your doctor Review Date 8/5/2015 Updated by: Sumana Jothi ... Otolaryngology, NCHCS VA, SFVA, San Francisco, CA. Internal review and update on 09/01/2016 by David ...

  17. Ear infection - acute

    MedlinePlus

    ... more than 6 children) Changes in altitude or climate Cold climate Exposure to smoke Family history of ear infections ... or fewer children. This can reduce your child's chances of getting a cold or other infection, and ...

  18. How to Use Ear Drops

    MedlinePlus

    ... the dropper tip down. Tilt the affected ear up or lie on your side. Pull the ear backward and upward (or if giving ... into the ear canal. Keep your ear tilted up for a few minutes or insert a soft ... from the Michigan Pharmacists Association's Patient Education Program.

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

    PubMed

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

    2014-04-01

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

  20. Inner ear disturbances related to middle ear inflammation

    PubMed Central

    Sone, Michihiko

    2017-01-01

    ABSTRACT The inner and middle ear are connected mainly through round and oval windows, and inflammation in the middle ear cavity can spread into the inner ear, which might induce a disturbance. In cases with intractable otitis media, attention should also be paid to symptoms related to the inner ear. In this paper, middle ear inflammation and related inner ear disturbances are reviewed with a focus on representative middle ear diseases (such as acute otitis media, chronic otitis media, otitis media with anti-neutrophil cytoplasmic antibody-associated vasculitis, eosinophilic otitis media, cholesteatoma with labyrinthine fistula, and reflux-related otitis media). Their clinical concerns are then discussed with reference to experimental studies. In these diseases, early diagnosis and adequate treatment are required to manage not only middle ear but also inner ear conditions. PMID:28303055

  1. Cardiovascular Malformations in CHARGE Syndrome with DiGeorge Phenotype: Two Case Reports.

    PubMed

    Yasuda, Kazushi; Morihana, Eiji; Fusazaki, Naoki; Ishikawa, Shiro

    2016-01-01

    Both CHARGE syndrome and DiGeorge anomaly are frequently accompanied by cardiovascular malformations. Some specific cardiovascular malformations such as interrupted aortic arch type B and truncus arteriosus are frequently associated with 22q11.2 deletion syndrome, while conotruncal defects and atrioventricular septal defects are overrepresented in patients with CHARGE syndrome. CHD7 gene mutation is identified in approximately two-thirds of patients with CHARGE syndrome, and chromosomal microdeletion at 22q11.2 is found in more than 95% of patients with 22q11.2 deletion syndrome. CHARGE syndrome is occasionally accompanied by DiGeorge phenotype. We report two patients with dysmorphic features of both CHARGE syndrome and 22q11.2 deletion syndrome. Although both of the two cases did not have 22q11.2 deletion, they had typical dysmorphic features of 22q11.2 deletion syndrome including cardiovascular malformations such as interrupted aortic arch type B. They also had characteristic features of CHARGE syndrome including ear malformation, genital hypoplasia, limb malformation, and endocrinological disorders. CHD7 gene mutation was confirmed in one of the two cases. When a patient with cardiovascular malformations frequently associated with 22q11.2 deletion syndrome does not have 22q11.2 deletion, we suggest that associated malformations characteristic of CHARGE syndrome should be searched for.

  2. Cardiovascular Malformations in CHARGE Syndrome with DiGeorge Phenotype: Two Case Reports

    PubMed Central

    Morihana, Eiji; Fusazaki, Naoki; Ishikawa, Shiro

    2016-01-01

    Both CHARGE syndrome and DiGeorge anomaly are frequently accompanied by cardiovascular malformations. Some specific cardiovascular malformations such as interrupted aortic arch type B and truncus arteriosus are frequently associated with 22q11.2 deletion syndrome, while conotruncal defects and atrioventricular septal defects are overrepresented in patients with CHARGE syndrome. CHD7 gene mutation is identified in approximately two-thirds of patients with CHARGE syndrome, and chromosomal microdeletion at 22q11.2 is found in more than 95% of patients with 22q11.2 deletion syndrome. CHARGE syndrome is occasionally accompanied by DiGeorge phenotype. We report two patients with dysmorphic features of both CHARGE syndrome and 22q11.2 deletion syndrome. Although both of the two cases did not have 22q11.2 deletion, they had typical dysmorphic features of 22q11.2 deletion syndrome including cardiovascular malformations such as interrupted aortic arch type B. They also had characteristic features of CHARGE syndrome including ear malformation, genital hypoplasia, limb malformation, and endocrinological disorders. CHD7 gene mutation was confirmed in one of the two cases. When a patient with cardiovascular malformations frequently associated with 22q11.2 deletion syndrome does not have 22q11.2 deletion, we suggest that associated malformations characteristic of CHARGE syndrome should be searched for. PMID:27957375

  3. [Inner Ear Hearing Loss].

    PubMed

    Hesse, G

    2016-06-01

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

  4. Listening to the Ear

    NASA Astrophysics Data System (ADS)

    Shera, Christopher Alan

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

  5. Listening to the ear

    NASA Astrophysics Data System (ADS)

    Shera, Christopher A.

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

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

    PubMed

    Nakajima, Yuji

    2015-02-01

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

  7. Fusion of the ear bones

    MedlinePlus

    ... Images Ear anatomy Medical findings based on ear anatomy References House JW, Cunningham CD. Otosclerosis. In: Flint PW, Haughey BH, Lund V, et al, eds. Cummings Otolaryngology: Head & Neck Surgery . 6th ed. Philadelphia, PA: Elsevier Saunders; ...

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

    MedlinePlus

    ... Work: Healthy Swimming Policy & Recommendations Fast Facts Healthy Water Sites Healthy Water Drinking Water Healthy Swimming Global ... painful. How is swimmer's ear spread at recreational water venues? Swimmer’s ear can occur when water stays ...

  9. [Diagnostics of genetic malformations in small ruminants].

    PubMed

    Ganter, M

    2013-01-01

    The epidemic occurrence of the Schmallenberg virus has induced numerous congenital malformations in small ruminants. Because of this high incidence of malformed lambs, an overview of the different causes of congenital malformations is provided. The most frequent infectious and physical causes as well as mineral and vitamin deficiencies and toxic agents which can induce congenital malformations are indicated. This list is supplemented by advice on sampling and laboratory diagnosis for an etiological diagnosis of the malformations.

  10. Ear Injuries (For Parents)

    MedlinePlus

    ... or it can be bought separately). Wear ear protection at concerts, especially when sitting near the stage or speakers (they'll still be able to hear with earplugs — it just won't be as deafening), mowing the lawn or using machinery (like in metal or wood shop at school), or playing a loud instrument ( ...

  11. The red ear syndrome

    PubMed Central

    2013-01-01

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

  12. 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…

  13. Taking Care of Your Ears

    MedlinePlus

    ... it to get better by itself. Tips for Swimmers Sometimes, swimming can lead to a case of swimmer's ear . That's when your outer ear gets infected, ... cause an infection. If you think you have swimmer's ear, your mom or dad needs to call ...

  14. 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…

  15. The contralateral ear in cholesteatoma.

    PubMed

    da Costa, Sady Selaimen; Teixeira, Adriane Ribeiro; Rosito, Letícia Petersen Schmidt

    2016-07-01

    Middle ear cholesteatoma has been extensively studied. Theories of cholesteatoma pathogenesis involving previous tympanic membrane retraction are the most widely accepted, but the contralateral ear in patients with cholesteatoma remains unstudied. This study aimed to investigate the contralateral ear in patients with cholesteatoma, and to determine whether the characteristics of it differ according to patient age and cholesteatoma growth patterns. This study was cross sectional. We evaluated 356 patients with middle ear cholesteatoma in at least one ear, and no history of surgery, between August 2000 and March 2013. Otoendoscopy was conducted on both the affected and the contralateral ear. They were classified as normal, tympanic membrane perforation, moderate to severe tympanic membrane retraction and cholesteatoma. The mean age of the patients was 32.77 years, and 53.1 % of the cohort were female. Only 34.8 % of the contralateral ears were normal. The most common abnormality was moderate to severe tympanic membrane retraction (41.6 %). Cholesteatoma was identified in 16 %. Children exhibited a greater frequency of tympanic membrane retractions, whereas adults exhibited a greater frequency of cholesteatoma. All of the contralateral ears in the anterior epitympanic group were normal, but otherwise there were no differences in the contralateral ear when we compared the cholesteatoma growth patterns. We conclude that patients diagnosed with acquired cholesteatoma of one ear are significantly more likely to exhibit abnormalities of the contralateral ear.

  16. Embolization of Arteriovenous Malformation

    PubMed Central

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

    2004-01-01

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

  17. 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

  18. Drug delivery to the ear.

    PubMed

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

    2013-01-01

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

  19. Prevalence of inner ear anomalies among cochlear implant candidates

    PubMed Central

    Aldhafeeri, Ahmad M.; Alsanosi, Abdulrahman A.

    2016-01-01

    Objectives: To determine the prevalence of inner ear anomalies and the frequency of different anomaly types among cochlear implant recipients. Methods: This study included a retrospective chart review of all patients who received cochlear implants between January 2009 and January 2013 in King Abdulaziz University Hospital cochlear implant program in Riyadh, Saudi Arabia. All subjects underwent thin-cut CT of the temporal bone and MRI. The collected data included age, gender, and CT and MRI findings regarding temporal bone anomalies. Patients with any identified congenital inner ear anomalies were included in the study. Results: In total, 316 patients’ cases were reviewed. Inner ear malformations were identified in 24 patients, which represented a prevalence of 7.5%. Among these 24 patients, 8 (33.3%) presented with a large vestibular aqueduct (LVA), 8 (33.3%) semicircular canal (SCC) dysplasia, 7 (29.1%) classical Mondini deformity, and one (4.1%) cochlear hypoplasia. Conclusion: The prevalence of inner ear anomalies among cochlear implant recipients was 7.5%. This result is consistent with findings worldwide. The most common anomalies were LVA and SCC hypoplasia; by contrast, in other regions, the most common anomaly is either the Mondini deformity, or LVA. PMID:27652360

  20. Imaging of congenital anomalies and acquired lesions of the inner ear.

    PubMed

    Krombach, Gabriele A; Honnef, Dagmar; Westhofen, Martin; Di Martino, Ercole; Günther, Rolf W

    2008-02-01

    Imaging of the temporal bone is under continous developement. In the recent decades the technical advances of magnetic resonance imaging and computed tomography have contributed to improved imaging quality in assessment of the temporal bone. Dedicated imaging protocols have been developed and are routinely employed in most institutions. However, imaging interpretation remains challenging, since the temporal bone is an anatomically highly complex region and most diseases of the inner ear occur with low incidence, so that even radiologists experienced in the field may be confronted with such entities for the first time. The current review gives an overview about symptoms and imaging appearance of malformations and acquired lesion of the inner ear.

  1. Cardiac arteriovenous malformation causing sudden death.

    PubMed

    Aguilera, Beatriz; Suárez-Mier, M Paz; Argente, Trinidad

    2004-01-01

    Cardiac vascular malformations are rare. We report a subendocardial arteriovenous malformation (AVM), associated with extensive myocardial fibrosis, causing sudden death in a 25-year-old woman. To our knowledge, this is the first autopsy case reported.

  2. Ear syringing: minimising the risks.

    PubMed

    Bird, Sara

    2008-05-01

    The patient, 61 years of age, saw the general practitioner for a repeat prescription for her blood pressure medication. During the consultation, the patient mentioned that she had some discomfort in her left ear. The GP examined the patient's ears and noted that both external auditory canals were blocked by wax. He recommended that the patient have her ears syringed and arranged for the practice nurse to perform the procedure. The GP did not see the patient again.

  3. [SURGICAL TREATMENT OF THE FACE CAPILLARY MALFORMATION].

    PubMed

    Galich, S P; Gindich, O A; Dabizha, A Yu; Ogorodnik, Ya P

    2015-08-01

    Results of surgical treatment of 37 patients for the head and neck capillary malformations were analyzed. Optimal surgical tactics, depending on the malformation form and localization, was proposed. Restitution of the tissues defect after excision of malformation, using the flaps transposition, have permitted to achieve good esthetic results.

  4. 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.

  5. Polysplenia with pulmonary arteriovenous malformations.

    PubMed

    Papagiannis, J; Kanter, R J; Effman, E L; Pratt, P C; Marcille, R; Browning, I B; Armstrong, B E

    1993-03-01

    A patient with polysplenia syndrome, dextrocardia, left atrial isomerism, normal great vessel relationships, and no intracardiac shunts developed progressive cyanosis and clubbing. Pulmonary arteriovenous malformations (PAVMs) were diagnosed by angiography and confirmed by lung biopsy. Superior mesenteric arteriogram revealed hypoplasia of the intrahepatic portal vein branches and a portosystemic shunt. The possible etiologies of PAVMs are discussed.

  6. Arteriovenous malformation of the uterus.

    PubMed

    Dodia, Nazera; George, Suku

    2015-09-17

    We present the case of a 54-year-old woman with intermittent right-sided abdominal pain. Ultrasound scans showed an unusual vascular appearance of the uterus with a thinned endometrium. Contrast CT led to a strong suspicion of an arteriovenous malformation of the uterus. The patient was successfully treated with a hysterectomy with salpingo-oophorectomy.

  7. Superior semicircular canal dehiscence associated with external, middle, and inner ear abnormalities.

    PubMed

    Wang, Jenny R; Parnes, Lorne S

    2010-02-01

    We report a case of bilateral superior semicircular canal dehiscence (SSCD) in a 38-year-old man who presented with congenital hearing loss without vestibular symptoms. This case is unusual due to the association of SSCD with multiple congenital anomalies of the external, middle, and inner ears, but without any other craniofacial or systemic developmental anomalies. Findings of multiple malformations of temporal bone structures in this case lend support to the theory that SSCD may have an underlying developmental or congenital etiology.

  8. Benign ear cyst or tumor

    MedlinePlus

    ... Bony tumor of the ear canal Images Ear anatomy References Nicolai P, Castelnuovo P. Benign tumors of the sinonasal tract. In: Flint PW, Haughey BH, Lund V, et al, eds. Cummings Otolaryngology: Head & Neck Surgery . 6th ed. Philadelphia, PA: Elsevier Saunders; ...

  9. 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 ...

  10. Wax blockage in the ear (image)

    MedlinePlus

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

  11. Factors leading to chronic middle ear disease.

    PubMed

    Canty, A A; Prestwood, U; Dugdale, A E; Lewis, A N

    1975-05-10

    In an Australian Aboriginal community, 65% of all people examined had clinical evidence of pathology in the ear drum or middle ear, but active ear disease was found mainly in children. In most people, both ears showed similar clinical changes. Clinical nutritional status and hygienic factors did not correlate with the presence of ear disease. Some families had significantly more ear disease than did others, suggesting that there is some as yet unidentified familial factor.

  12. Restudy of malformations of the internal auditory meatus, cochlear nerve canal and cochlear nerve.

    PubMed

    Li, Youjin; Yang, Jun; Liu, Jinfen; Wu, Hao

    2015-07-01

    The present study aims to restudy the correlation between the internal auditory meatus (IAM), the cochlear nerve canal (CNC), the cochlear nerve (CN) and inner ear malformations. In this retrospective study design, the abnormal diameter of the IAM, CNC and CN in patients with any kind of inner ear malformations was evaluated using multi-slice spiral computed tomography (MSCT) (37 patients) and magnetic resonance imaging (MRI) (18 patients). Of 37 MSCT-diagnosed patients, 2 had IAM atresia, 11 IAM stenosis, 22 enlarged IAM, and 2 normal IAM with an abnormal CN. MRI diagnoses of 18 patients revealed 8 cases of aplastic CN, 6 hypoplastic CN, and 4 normal CN. CNC stenosis was associated with CN hypoplasia (P < 0.001). Patients with absent or stenotic IAM had less CN development than those with normal or enlarged IAM (P = 0.001). We propose a modification of the existing classification systems with a view to distinguishing malformations of the IAM, CNC and CN.

  13. Outcomes in Endoscopic Ear Surgery.

    PubMed

    Kiringoda, Ruwan; Kozin, Elliott D; Lee, Daniel J

    2016-10-01

    Endoscopic ear surgery (EES) provides several advantages compared with traditional binocular microscopy, including a wide-field view, improved resolution with high magnification, and visual access to hidden corridors of the middle ear. Although binocular microscopic-assisted surgical techniques remain the gold standard for most otologists, EES is slowly emerging as a viable alternative for performing otologic surgery at several centers in the United States and abroad. In this review, we evaluate the current body of literature regarding EES outcomes, summarize our EES outcomes at the Massachusetts Eye and Ear Infirmary, and compare these results with data for microscopic-assisted otologic surgery.

  14. 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

  15. 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.

  16. Middle Ear Infections (For Parents)

    MedlinePlus

    ... up of invisible waves of energy, causes these vibrations. Every time you hear a sound, the various ... When the eardrum vibrates, the ossicles amplify these vibrations and carry them to the inner ear. The ...

  17. Deformity of Ears and Kidneys

    PubMed Central

    Taylor, W. C.

    1965-01-01

    Ten children with gross deformity of the external ear were observed. In six the facial bones were underdeveloped on the same side as the deformed ear. In all six there was a congenital abnormality of the kidney or upper urinary tract, usually on the same side as the deformed ear. In addition there were usually other associated congenital defects in each case. In the remaining four children the facial bones appeared normal, and pyelography showed no abnormality of the urinary tract. In these four children there were no other associated defects. These observations emphasize the importance of investigating the urinary tract in children with gross deformity of the external ear, especially where there is an associated underdevelopment of the facial bones. PMID:14317453

  18. Ototoxicity (Ear Poisoning) (For Parents)

    MedlinePlus

    ... part of the ear responsible for receiving/sending sounds and controlling balance. The degree of damage depends ... have trouble hearing certain things, from high-pitched sounds to talking if there's background noise. Or they ...

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

    MedlinePlus

    ... previously covered by swimsuit > Pus-filled blisters around hair follicles Swimmer’s Ear (Otitis externa) > Pain when infected ear ... ear. You can find this product at your drug store. > Avoid putting objects in the ear (for ... levels drop, so testing your pool or hot tub’s disinfectant and pH ...

  20. 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... 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...

  1. 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...

  2. 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...

  3. 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...

  4. 21 CFR 870.2710 - Ear oximeter.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

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

  5. Multiple cavernous malformations with supravermian arachnoid cyst.

    PubMed

    Unalp, Aycan; Uran, Nedret

    2007-11-01

    Cerebral cavernous malformation are congenital vascular abnormalities that have been reported in 0.4% of the population; they represent 5-13% of all cerebrovascular malformations. Onset of cerebral cavernous malformations may be associated with seizures, intracranial hemorrhages, focal neurological deficit or migraine-type headaches. Some patients may require surgical intervention due to hemorrhage. Multiple cavernomas in childhood have been reported in the literature, but they are rare. This manuscript presents a 12-year-old girl with multiple cavernomas accompanied by supravermian arachnoid cyst detected by neuroimaging techniques. This is the first report that demonstrates a case of pediatric multiple cavernous malformation coexisting with arachnoid cyst of the supravermian cistern.

  6. Approaches to Inflight Ear Oximetry.

    DTIC Science & Technology

    1980-10-01

    of arterial oxygen saturation. For centri- fuge experiments the floe,! ett-Packard ear oximeter, Model 47201A, has been successfully used both at...These difficulties are perhaps even more significant with respect to inflight experimental use. The difficulties are: 1. The bloodless ear is not truly...available (9), and a number of papers on both the clini- cal use (10-17) and the experimental use (1, 2, 18) of this equipment have been published since its

  7. Novel Vascular Malformation in an Affected Newborn with Deletion Del(4)(q31.3)

    PubMed Central

    de León Ojeda, Norma Elena; Soriano-Torres, Michel; Cabrera, Mercedes J.; Benítez Ramos, Dunia Bárbara

    2012-01-01

    We report on a newborn male patient with a terminal deletion in the long arm of the chromosome 4 with a congenital heart defect unreported before in association with this syndrome. The patient had multiple congenital anomalies including a pointed duplicated fingernail, low set posteriorly rotated ears, large anterior fontanel, micrognathia, glabellar capillary vascular malformation, and Interrupted Aortic Arch type C. The patient died due to multiple congenital malformations; a peripheral chromosome analysis showed 46, XY, del(4)(q31.3) de novo. The only reported case with the same deletion was a male newborn that exhibited the pattern of minor anomalies of deletion 4q31 syndrome. The parents were cytogenetically normal. We compare clinical signs to other cases with a deletion in long arm of chromosome 4. PMID:23320208

  8. [Inner ear and subarachnoid spaces: relations and diseases].

    PubMed

    Marsot-Dupuch, K; Djouhri, H; Meyer, B; Pharaboz, C; Tran Ba Huy, P

    2001-06-01

    The purpose of this article is to describe, with 5 clinical cases, the physiological communications between the inner ear and the subarachnoid spaces (SAS) and present the imaging features with regard to. Therefore we briefly illustrate abnormal communications between SAS and perilymphatic fluids in certain cochlear and internal acoustic meatus (IAM) malformations and their consequences. Imaging features may depict diffusion pathway of bacterial meningitis to membranous labyrinth via the cochlear aqueduct or via the IAM. Rarely, in some patients referred for cochleovestibular symptoms, imaging features may display skull base tumors involving the area of cochlear or vestibular aqueduct aperture. Therefore, in patients referred for cochleovestibular symptoms, MR and CT study should carefully scrutinise not only the IAM but also the aperture of the cochlear and the vestibular aqueducts and the cerebellopontine meninges.

  9. 3D printed bionic ears.

    PubMed

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

    2013-06-12

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

  10. 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

  11. 3D finite element model of the chinchilla ear for characterizing middle ear functions.

    PubMed

    Wang, Xuelin; Gan, Rong Z

    2016-10-01

    Chinchilla is a commonly used animal model for research of sound transmission through the ear. Experimental measurements of the middle ear transfer function in chinchillas have shown that the middle ear cavity greatly affects the tympanic membrane (TM) and stapes footplate (FP) displacements. However, there is no finite element (FE) model of the chinchilla ear available in the literature to characterize the middle ear functions with the anatomical features of the chinchilla ear. This paper reports a recently completed 3D FE model of the chinchilla ear based on X-ray micro-computed tomography images of a chinchilla bulla. The model consisted of the ear canal, TM, middle ear ossicles and suspensory ligaments, and the middle ear cavity. Two boundary conditions of the middle ear cavity wall were simulated in the model as the rigid structure and the partially flexible surface, and the acoustic-mechanical coupled analysis was conducted with these two conditions to characterize the middle ear function. The model results were compared with experimental measurements reported in the literature including the TM and FP displacements and the middle ear input admittance in chinchilla ear. An application of this model was presented to identify the acoustic role of the middle ear septa-a unique feature of chinchilla middle ear cavity. This study provides the first 3D FE model of the chinchilla ear for characterizing the middle ear functions through the acoustic-mechanical coupled FE analysis.

  12. Development of the inner ear.

    PubMed

    Whitfield, Tanya T

    2015-06-01

    The vertebrate inner ear is a sensory organ of exquisite design and sensitivity. It responds to sound, gravity and movement, serving both auditory (hearing) and vestibular (balance) functions. Almost all cell types of the inner ear, including sensory hair cells, sensory neurons, secretory cells and supporting cells, derive from the otic placode, one of the several ectodermal thickenings that arise around the edge of the anterior neural plate in the early embryo. The developmental patterning mechanisms that underlie formation of the inner ear from the otic placode are varied and complex, involving the reiterative use of familiar signalling pathways, together with roles for transcription factors, transmembrane proteins, and extracellular matrix components. In this review, I have selected highlights that illustrate just a few of the many recent discoveries relating to the development of this fascinating organ system.

  13. 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

  14. Radiosurgery for brainstem arteriovenous malformation.

    PubMed

    Maruyama, Keisuke; Koga, Tomoyuki; Niranjan, Ajay; Kondziolka, Douglas; Flickinger, John C; Lunsford, L Dade

    2013-01-01

    The authors outlined the treatment result of arteriovenous malformations (AVMs) inside the brainstem by reviewing the 4 existing studies in detail. The majority of patients with brainstem AVMs had a history of hemorrhage, leading to neurological deficits at the time of treatment in 72-73% of patients. The most frequent location was the midbrain or the pons depending on studies, while the medulla oblongata was the least common location throughout the series. The obliteration rate after radiosurgery was 44-73%, which was generally lower than in other locations, while the complication rate was 5-14%, which was expectedly higher than in other locations. No objective evidence for size is known, and therefore, patient selection and treatment planning should be carefully performed after judicious assessment of treatment risks and benefits among limited treatment options.

  15. Pathogenesis of Brain Arteriovenous Malformations

    PubMed Central

    KOMIYAMA, Masaki

    2016-01-01

    Brain arteriovenous malformations (bAVMs) represent a high risk of intracranial hemorrhages, which are substantial causes of morbidity and mortality of bAVMs, especially in children and young adults. Although a variety of factors leading to hemorrhages of bAVMs are investigated extensively, their pathogenesis is still not well elucidated. The author has reviewed the updated data of genetic aspects of bAVMs, especially focusing on clinical and experimental knowledge from hereditary hemorrhagic telangiectasia, which is the representative genetic disease presenting with bAVMs caused by loss-of-function in one of the two genes: endoglin and activin receptor-like kinase 1. This knowledge may allow us to infer the pathogensis of sporadic bAVMs and in the development of new medical therapies for them. PMID:27076383

  16. New insights into craniofacial malformations

    PubMed Central

    Twigg, Stephen R.F.; Wilkie, Andrew O.M.

    2015-01-01

    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. Genetic Basis of Brain Malformations

    PubMed Central

    Parrini, Elena; Conti, Valerio; Dobyns, William B.; Guerrini, Renzo

    2016-01-01

    Malformations of cortical development (MCD) represent a major cause of developmental disabilities, severe epilepsy, and reproductive disadvantage. Genes that have been associated to MCD are mainly involved in cell proliferation and specification, neuronal migration, and late cortical organization. Lissencephaly-pachygyria-severe band heterotopia are diffuse neuronal migration disorders causing severe global neurological impairment. Abnormalities of the LIS1, DCX, ARX, RELN, VLDLR, ACTB, ACTG1, TUBG1, KIF5C, KIF2A, and CDK5 genes have been associated with these malformations. More recent studies have also established a relationship between lissencephaly, with or without associated microcephaly, corpus callosum dysgenesis as well as cerebellar hypoplasia, and at times, a morphological pattern consistent with polymicrogyria with mutations of several genes (TUBA1A, TUBA8, TUBB, TUBB2B, TUBB3, and DYNC1H1), regulating the synthesis and function of microtubule and centrosome key components and hence defined as tubulinopathies. MCD only affecting subsets of neurons, such as mild subcortical band heterotopia and periventricular heterotopia, have been associated with abnormalities of the DCX, FLN1A, and ARFGEF2 genes and cause neurological and cognitive impairment that vary from severe to mild deficits. Polymicrogyria results from abnormal late cortical organization and is inconstantly associated with abnormal neuronal migration. Localized polymicrogyria has been associated with anatomo-specific deficits, including disorders of language and higher cognition. Polymicrogyria is genetically heterogeneous, and only in a small minority of patients, a definite genetic cause has been identified. Megalencephaly with normal cortex or polymicrogyria by MRI imaging, hemimegalencephaly and focal cortical dysplasia can all result from mutations in genes of the PI3K-AKT-mTOR pathway. Postzygotic mutations have been described for most MCD and can be limited to the dysplastic tissue in the

  18. 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.

  19. Renal tract malformations: perspectives for nephrologists.

    PubMed

    Kerecuk, Larissa; Schreuder, Michiel F; Woolf, Adrian S

    2008-06-01

    Renal tract malformations are congenital anomalies of the kidneys and/or lower urinary tract. One challenging feature of these conditions is that they can present not only prenatally but also in childhood or adulthood. The most severe types of malformations, such as bilateral renal agenesis or dysplasia, although rare, lead to renal failure. With advances in dialysis and transplantation for young children, it is now possible to prevent the early death of at least some individuals with severe malformations. Other renal tract malformations, such as congenital pelviureteric junction obstruction and primary vesicoureteric reflux, are relatively common. Renal tract malformations are, collectively, the major cause of childhood end-stage renal disease. Their contribution to the number of adults on renal replacement therapy is less clear and has possibly been underestimated. Renal tract malformations can be familial, and specific mutations of genes involved in renal tract development can sometimes be found in affected individuals. These features provide information about the causes of malformations but also raise questions about whether to screen relatives. Whether prenatal decompression of obstructed renal tracts, or postnatal initiation of therapies such as prophylactic antibiotics or angiotensin blockade, improve long-term renal outcomes remains unclear.

  20. Mutations in KCTD1 cause scalp-ear-nipple syndrome.

    PubMed

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

    2013-04-04

    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.

  1. Vascular Malformations: Approach by an Interventional Radiologist

    PubMed Central

    Pimpalwar, Sheena

    2014-01-01

    Children with vascular malformations are best managed with a multidisciplinary team of specialists. Interventional radiology may deliver primary treatment such as staged sclerotherapy and embolization for malformations that are poor candidates for primary surgical resection or play a supportive role such as preoperative or intraoperative embolization. A thorough understanding of vascular morphology and flow dynamics is imperative to choosing the best treatment tool and technique. In this review, the author discusses the selection of techniques and tools used to treat vascular malformations based on their angiographic morphology. PMID:25045335

  2. Low-set ears and pinna abnormalities

    MedlinePlus

    Low-set ears; Microtia; "Lop" ear; Pinna abnormalities; Genetic defect-pinna; Congenital defect-pinna ... most cases, a health care provider finds pinna abnormalities during the first well-baby exam. This exam ...

  3. Physiological functioning of the ear and masking

    NASA Technical Reports Server (NTRS)

    1984-01-01

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

  4. Anterior commissure absence without callosal agenesis: a new brain malformation.

    PubMed

    Mitchell, T N; Stevens, J M; Free, S L; Sander, J W; Shorvon, S D; Sisodiya, S M

    2002-04-23

    The authors report a novel human brain malformation characterized by the absence of the anterior commissure without callosal agenesis, but associated with gross unilateral panhemispheric malformation incorporating subependymal heterotopia, subcortical heterotopia, and gyral abnormalities including temporal malformation and polymicrogyria. In contrast, a normal anterior commissure was found in 125 control subjects and in 113 other subjects with a range of brain malformations.

  5. Ear dominance and telephone sales.

    PubMed

    Furnham, A; Richardson, S; Miller, T

    1997-10-01

    In a field study, three equally sized sales teams used on of three head-sets--left, right, both ears--for a day's selling of insurance by telephone. This had no effect on sales. In a retrospective study of records, daily sales performance including the percentage conversion rate for sales divided by the number of calls and the number and duration of calls was related to preference for type of head-set. Sales were markedly influenced by the choice of head-set. People who chose to wear the left earpiece significantly out sold the others wearing right and stereohead-sets. Neither the number of incoming calls nor the time spent on the telephone were influenced by the choice of head-set. When sales are analysed in terms of individual differences in personal preference for type of head-set, those who chose the left ear had an advantage. Forced use of the left, versus right ear or both ears for one day had no effect.

  6. Middle Ear Infections (For Parents)

    MedlinePlus

    ... en español Infecciones del oído medio After the common cold , ear infections are the most frequently diagnosed childhood ... winter season, when lots of people get upper respiratory tract infections or colds. Signs and Symptoms The signs and ...

  7. Mechanics of the frog ear

    PubMed Central

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

    2010-01-01

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

  8. Adenomatous tumors of the middle ear.

    PubMed

    Pelosi, Stanley; Koss, Shira

    2015-04-01

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

  9. Ear recognition based on Gabor features and KFDA.

    PubMed

    Yuan, Li; Mu, Zhichun

    2014-01-01

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

  10. 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…

  11. Can Loud Music Hurt My Ears?

    MedlinePlus

    ... What Happens in the Operating Room? Can Loud Music Hurt My Ears? KidsHealth > For Kids > Can Loud Music Hurt My Ears? A A A en español ... up? Oh! You want to know if loud music can hurt your ears . Are you asking because ...

  12. 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)

  13. Can Loud Music Hurt My Ears?

    MedlinePlus

    ... dientes Video: Getting an X-ray Can Loud Music Hurt My Ears? KidsHealth > For Kids > Can Loud Music Hurt My Ears? Print A A A en ... up? Oh! You want to know if loud music can hurt your ears . Are you asking because ...

  14. Glomuvenous malformation: magnetic resonance imaging findings.

    PubMed

    Flors, Lucia; Norton, Patrick T; Hagspiel, Klaus D

    2015-02-01

    We report a case of a glomuvenous malformation involving the dorsal aspect of the right hand and distal forearm in an 11-year-old boy. He had a history of multiple vascular anomalies since birth and presented with increasing right hand pain. MRI played an important role in characterizing and determining the extent of the lesion. In particular, dynamic time-resolved contrast-enhanced MR angiography precisely defined its vascularity. The diagnosis was made histopathologically after partial resection of the lesion. Glomuvenous malformation is a rare developmental hamartoma that originates from the glomus body. Clinically they usually resemble a venous malformation but they are a different entity. In the appropriate clinical setting this rare condition must be included in the differential diagnosis of a vascular malformation, especially when subtle arterial enhancement, early venous shunting and progressive filling of dilated venous spaces are depicted on MRA.

  15. [Central nervous system malformations: neurosurgery correlates].

    PubMed

    Jiménez-León, Juan C; Betancourt-Fursow, Yaline M; Jiménez-Betancourt, Cristina S

    2013-09-06

    Congenital malformations of the central nervous system are related to alterations in neural tube formation, including most of the neurosurgical management entities, dysraphism and craniosynostosis; alterations of neuronal proliferation; megalencefaly and microcephaly; abnormal neuronal migration, lissencephaly, pachygyria, schizencephaly, agenesis of the corpus callosum, heterotopia and cortical dysplasia, spinal malformations and spinal dysraphism. We expose the classification of different central nervous system malformations that can be corrected by surgery in the shortest possible time and involving genesis mechanisms of these injuries getting better studied from neurogenic and neuroembryological fields, this involves connecting innovative knowledge areas where alteration mechanisms in dorsal induction (neural tube) and ventral induction (telencephalization) with the current way of correction, as well as the anomalies of cell proliferation and differentiation of neuronal migration and finally the complex malformations affecting the posterior fossa and current possibilities of correcting them.

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

    PubMed

    Reith, W; Yilmaz, U; Heumüller, I

    2014-04-01

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

  17. Venous malformations: clinical diagnosis and treatment

    PubMed Central

    Behravesh, Sasan; Yakes, Wayne; Gupta, Nikhil; Naidu, Sailendra; Chong, Brian W.; Khademhosseini, Ali

    2016-01-01

    Venous malformation (VM) is the most common type of congenital vascular malformation (CVM). They are present at birth and are often symptomatic, causing morbidity and pain. VMs can be challenging to diagnose and are often confused with hemangioma in terminology as well as with imaging. An accurate clinical history and cross-sectional imaging are critical for diagnosis and for devising management. This manuscript will review imaging approaches to diagnosing VMs and current treatment strategies. PMID:28123976

  18. 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

  19. Spontaneous thrombosis of vein of Galen malformation

    PubMed Central

    Kariyappa, Kalpana Devi; Krishnaswami, Murali; Gnanaprakasam, Francis; Ramachandran, Madan; Krishnaswamy, Visvanathan

    2016-01-01

    Vein of Galen malformation (VOGM) is a rare intracranial vascular malformation. Before the advent and advancement of various endovascular techniques, there was dismal prognosis. Rarely, this condition may spontaneously thrombose without the need for surgical or endovascular treatment with good prognosis. We report a case of an 8-month-old infant who had serial imaging, suggestive of VOGM and presented to us for further management, wherein the imaging revealed spontaneous thrombosis. PMID:27857804

  20. 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

  1. LDV measurement of bird ear vibrations to determine inner ear impedance and middle ear power flow

    NASA Astrophysics Data System (ADS)

    Muyshondt, Pieter G. G.; Pires, Felipe; Dirckx, Joris J. J.

    2016-06-01

    The mechanical behavior of the middle ear structures in birds and mammals is affected by the fluids in the inner ear (IE) that are present behind the oval window. In this study, the aim was to gather knowledge of the acoustic impedance of the IE in the ostrich, to be able to determine the effect on vibrations and power flow in the single-ossicle bird middle ear for future studies. To determine the IE impedance, vibrations of the ossicle were measured for both the quasi-static and acoustic stimulus frequencies. In the acoustic regime, vibrations were measured with a laser Doppler vibrometer and electromagnetic stimulation of the ossicle. The impedance of the inner ear could be determined by means of a simple RLC model in series, which resulted in a stiffness reactance of KIE = 0.20.1012 Pa/m3, an inertial impedance of MIE = 0.652.106 Pa s2/m3, and a resistance of RIE = 1.57.109 Pa s/m. The measured impedance is found to be considerably smaller than what is found for the human IE.

  2. 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

  3. [Intracranial arteriovenous malformations in Taiwan].

    PubMed

    Lin, L S; Shih, C J

    1993-12-01

    This paper analyzes the available literature on intracranial arteriovenous malformations (AVM) in Taiwan. The incidence and symptoms of the disease are studied with a view to assisting practitioners in its recognition. The incidence of intracranial AVM in patients who have suffered hemorrhagic stroke in Taiwan is 2.5% to 4.8%, with the male to female ratio being 1.5:1. The peak age at which bleeding from intracranial AVM occurred ranged from 10 to 40 years; bleeding showed no seasonal variation. Sudden headaches, vomiting, and disturbance of consciousness were the commonest presenting symptoms of AVM, similar to the rupture of intracranial aneurysms. However, the possibility of focal neurological deficit among patients with intracranial AVM was higher than in patients with intracranial aneurysms. Risk factors, such as hypertension, diabetes mellitus, heart disease, smoking and alcohol intake showed no close relationship to bleeding in intracranial AVM. Pregnancy is not a risk factor in female patients with intracranial AVM with no history of hemorrhage. Small intracranial AVM are more likely to bleed. Since 1961 the majority of Taiwan's intracranial AVM patients have been treated surgically, while before that date general medicine was the treatment of choice. In recent years, several developments such as operation microscope, microsurgical instruments and microsurgical techniques have enhanced the efficacy of surgical intervention in the treatment of AVM. When the mortality and morbidity rates resulting from the two forms of treatment are compared, surgical treatment shows a better prognosis for the treatment of intracranial AVM.

  4. 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.

  5. 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

  6. 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.

  7. Virus-induced congenital malformations in cattle.

    PubMed

    Agerholm, Jørgen S; Hewicker-Trautwein, Marion; Peperkamp, Klaas; Windsor, Peter A

    2015-09-24

    Diagnosing the cause of bovine congenital malformations (BCMs) is challenging for bovine veterinary practitioners and laboratory diagnosticians as many known as well as a large number of not-yet reported syndromes exist. Foetal infection with certain viruses, including bovine virus diarrhea virus (BVDV), Schmallenberg virus (SBV), blue tongue virus (BTV), Akabane virus (AKAV), or Aino virus (AV), is associated with a range of congenital malformations. It is tempting for veterinary practitioners to diagnose such infections based only on the morphology of the defective offspring. However, diagnosing a virus as a cause of BCMs usually requires laboratory examination and even in such cases, interpretation of findings may be challenging due to lack of experience regarding genetic defects causing similar lesions, even in cases where virus or congenital antibodies are present. Intrauterine infection of the foetus during the susceptible periods of development, i.e. around gestation days 60-180, by BVDV, SBV, BTV, AKAV and AV may cause malformations in the central nervous system, especially in the brain. Brain lesions typically consist of hydranencephaly, porencephaly, hydrocephalus and cerebellar hypoplasia, which in case of SBV, AKAV and AV infections may be associated by malformation of the axial and appendicular skeleton, e.g. arthrogryposis multiplex congenita. Doming of the calvarium is present in some, but not all, cases. None of these lesions are pathognomonic so diagnosing a viral cause based on gross lesions is uncertain. Several genetic defects share morphology with virus induced congenital malformations, so expert advice should be sought when BCMs are encountered.

  8. [Venous malformations: clinical characteristics and differential diagnosis].

    PubMed

    Casanova, D; Boon, L-M; Vikkula, M

    2006-01-01

    Venous malformations (VM) are localized defects of blood vessels that are due to vascular dysmorphogenesis. These slow-flow lesions can affect any tissue or organ. Clinically, a cutaneous VM is characterized by a bluish mass that is compressible on palpation. Phleboliths are commonly present. Symptoms depend on location and size. VM are often sporadic and isolated, however, they can be associated with other malformations and be part of a syndrome; Klippel-Trenaunay (capillary-lymphatico-venous malformation with limb hypertrophy) is the most common. Glomuvenous malformation (GVM) is another type of venous anomaly. In contrast to VM, GVM is often painful on palpation and not compressible. Clinical diagnosis of VM is often made in the presence of a bluish cutaneous lesion: however, other lesions can mimick VM. The most frequent anomalies are a blue naevus, a hemorrhagic lymphatic malformation, a sub-cutaneous hemangioma or even the presence of dilated superficial normal veins due to underlying venous stenoses. This chapter will detail the clinical characteristics of venous anomalies and their differential diagnosis.

  9. Ovine craniofacial malformation: a morphometrical study.

    PubMed

    Eriksen, T; Kuiper, H; Pielmeier, R; Ganter, M; Distl, O; Staszyk, C

    2012-12-01

    Craniofacial malformation in 64 sheep was phenotypically described as mandibular distoclusion. Digital radiographs were examined in order to determine the degree of morphological changes in certain bones of the skull. Therefore, laterolateral standardised digital radiographs were used to determine anatomic reference points. Subsequently, five reference lines were defined and 16 linear and seven angular measurements were determined to describe malformations in the bones of the skull. Statistical analysis revealed a significant shortening of the rostral part of the corpus mandibulae and of the ramus mandibulae. However, the molar part of the mandible remained unchanged. These morphological changes caused premolar and molar malocclusion. No further craniofacial abnormalities, such as an elongation of the maxilla or of the incisive bone, were identified. In conclusion, the phenotypically observed mandibular distoclusion is caused by a shortening of specific parts of the mandible. This form of ovine craniofacial malformation is therefore best described as brachygnathia inferior.

  10. Debendox in early pregnancy and fetal malformation.

    PubMed

    Fleming, D M; Knox, J D; Crombie, D L

    1981-07-11

    During the mid-1960s, 22 977 pregnant women in Scotland and England were followed up prospectively for the incidence of malformations in their infants evident at birth or within six weeks. During the first 13 weeks of gestation 620 of these women had been prescribed Debendox (dicyclomine-doxylamine-pyridoxine) and 743 other women agents other than Debendox containing pyridoxine. Of the 620 women given Debendox, 589 (95%) had a normal outcome of pregnancy, 8 (13%) delivered a malformed infant, and 23 (3.7%) had other outcomes. Of the 22 357 women who were given Debendox, 445 (2.0%) produced infants with malformation; and the rates for all abnormal outcomes among women given Debendox and those not given the drug were 5.0% and 5.4% respectively. These results support the hypothesis that Debendox is not teratogenic.

  11. Debendox in early pregnancy and fetal malformation.

    PubMed Central

    Fleming, D M; Knox, J D; Crombie, D L

    1981-01-01

    During the mid-1960s, 22 977 pregnant women in Scotland and England were followed up prospectively for the incidence of malformations in their infants evident at birth or within six weeks. During the first 13 weeks of gestation 620 of these women had been prescribed Debendox (dicyclomine-doxylamine-pyridoxine) and 743 other women agents other than Debendox containing pyridoxine. Of the 620 women given Debendox, 589 (95%) had a normal outcome of pregnancy, 8 (13%) delivered a malformed infant, and 23 (3.7%) had other outcomes. Of the 22 357 women who were given Debendox, 445 (2.0%) produced infants with malformation; and the rates for all abnormal outcomes among women given Debendox and those not given the drug were 5.0% and 5.4% respectively. These results support the hypothesis that Debendox is not teratogenic. PMID:6789952

  12. CT appearances of external ear canal cholesteatoma.

    PubMed

    Malcolm, P N; Francis, I S; Wareing, M J; Cox, T C

    1997-09-01

    External ear canal cholesteatoma (EECC) is rare in ear, nose and throat (ENT) practice. Two cases, one bilateral, are described. Computed tomography demonstrates the extent of bony involvement. Erosion of the external canal should not be overlooked when reviewing CT of the petrous bone in cases of discharge from the ear. EECC may necessitate surgery and delay in the diagnosis of EECC can result in progressive bony destruction.

  13. 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

  14. Atypical presentations in Chiari II malformation.

    PubMed

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

    2006-01-01

    Myelomeningocele with Chiari II malformation and hydrocephalus is a common association seen in infants with a congenital failure of neurulation. Here we report two cases of such an association presenting with different sets of problems. The first patient presented with severe inspiratory stridor due to bilateral abductor vocal cord paralysis, which was relieved completely within 24 h of definitive surgery. The second patient experienced intraoperative cardiac arrest. Definitive surgery was followed after successful cardiopulmonary resuscitation. The cause of the perioperative sequence of events in both cases is attributed to the associated pathologies of Chiari II malformation.

  15. 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.

  16. 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.

  17. Molecular Mechanisms of Inner Ear Development

    PubMed Central

    Wu, Doris K.; Kelley, Matthew W.

    2012-01-01

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

  18. An Effective 3D Ear Acquisition System.

    PubMed

    Liu, Yahui; Lu, Guangming; Zhang, David

    2015-01-01

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

  19. Development and Integration of the Ear.

    PubMed

    Fuchs, Jennifer C; Tucker, Abigail S

    2015-01-01

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

  20. 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.

  1. 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

  2. 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...

  3. Recurrent meningitis due to pneumococci and non-typable Haemophilus influenzae in a child with a Mondini malformation.

    PubMed

    Valmari, P; Palva, A

    1986-01-01

    An eight-year-old boy with a congenital inner ear malformation and recurrent otitis media had three episodes of bacteriologically confirmed meningitis within seven months. The first episode was caused by pneumococci, the other two by non-typable Haemophilus influenzae. All episodes were characterized by an abrupt onset. The CSF cultures were positive within 0.5 to 12 hours after the onset of symptoms. Despite misleading laboratory studies, surgical exploration revealed a CSF fistula associated to the inner ear anomaly. No further episodes occurred after the fistula was closed. Careful roentgenographic evaluation, including recently introduced special computed tomography (CT) methods, is indicated in recurrent meningitis. In addition, such evaluations should be considered even after the first episode, when special clinical features suggest a CSF fistula. Such features include an extremely rapid onset and detection of common non-invasive bacteria as causative agents, as illustrated by the present case.

  4. 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...

  5. Chiari I Malformation in Nephropathic Cystinosis

    PubMed Central

    Rao, Kavya I; Hesselink, John; Trauner, Doris A

    2015-01-01

    Objective To determine the relative incidence of Chiari I malformations in children with cystinosis compared with those in the general population. Study design Magnetic resonance imaging (MRI) scans were performed on 53 patients with nephropathic cystinosis and 120 controls, age range 3-18 years. Results Ten of 53 (18.9%) cystinosis patients had Chiari I or tonsillar ectopia, and only 2 of 120 controls (1.6%) had a similar finding. At least 2 of the patients had symptoms or signs thought to be related to the malformation, and one had surgical decompression. Two had an associated cervical syrinx. Conclusions Children with cystinosis have a 12-fold higher prevalence of Chiari I malformations than the general pediatric population. Chiari I malformations should be high on the differential diagnosis when individuals with cystinosis develop neurologic signs and symptoms, and MRI scans should be performed on children with cystinosis who present with new-onset headache, ataxia, incontinence, or other unexplained neurologic symptoms. PMID:26265281

  6. Angular craniometry in craniocervical junction malformation.

    PubMed

    Botelho, Ricardo Vieira; Ferreira, Edson Dener Zandonadi

    2013-10-01

    The craniometric linear dimensions of the posterior fossa have been relatively well studied, but angular craniometry has been poorly studied and may reveal differences in the several types of craniocervical junction malformation. The objectives of this study were to evaluate craniometric angles compared with normal subjects and elucidate the main angular differences among the types of craniocervical junction malformation and the correlation between craniocervical and cervical angles. Angular craniometries were studied using primary cranial angles (basal and Boogard's) and secondary craniocervical angles (clivus canal and cervical spine lordosis). Patients with basilar invagination had significantly wider basal angles, sharper clivus canal angles, larger Boogard's angles, and greater cervical lordosis than the Chiari malformation and control groups. The Chiari malformation group does not show significant differences when compared with normal controls. Platybasia occurred only in basilar invagination and is suggested to be more prevalent in type II than in type I. Platybasic patients have a more acute clivus canal angle and show greater cervical lordosis than non-platybasics. The Chiari group does not show significant differences when compared with the control, but the basilar invagination groups had craniometric variables significantly different from normal controls. Hyperlordosis observed in the basilar inavagination group was associated with craniocervical kyphosis conditioned by acute clivus canal angles.

  7. Congenital Malformations in River Buffalo (Bubalus bubalis).

    PubMed

    Albarella, Sara; Ciotola, Francesca; D'Anza, Emanuele; Coletta, Angelo; Zicarelli, Luigi; Peretti, Vincenzo

    2017-02-10

    The world buffalo population is about 168 million, and it is still growing, in India, China, Brazil, and Italy. In these countries, buffalo genetic breeding programs have been performed for many decades. The occurrence of congenital malformations has caused a slowing of the genetic progress and economic loss for the breeders, due to the death of animals, or damage to their reproductive ability or failing of milk production. Moreover, they cause animal welfare reduction because they can imply foetal dystocia and because the affected animals have a reduced fitness with little chances of survival. This review depicts, in the river buffalo (Bubalus bubalis) world population, the present status of the congenital malformations, due to genetic causes, to identify their frequency and distribution in order to develop genetic breeding plans able to improve the productive and reproductive performance, and avoid the spreading of detrimental gene variants. Congenital malformations most frequently reported in literature or signaled by breeders to the Department of Veterinary Medicine and Animal Production of the University Federico II (Naples, Italy) in river buffalo are: musculoskeletal defects (transverse hemimelia, arthrogryposis, umbilical hernia) and disorders of sexual development. In conclusion this review put in evidence that river buffalo have a great variety of malformations due to genetic causes, and TH and omphalocele are the most frequent and that several cases are still not reported, leading to an underestimation of the real weight of genetic diseases in this species.

  8. Differential Gene Expression in Human Cerebrovascular Malformations

    PubMed Central

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

    2009-01-01

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

  9. Congenital Malformations in River Buffalo (Bubalus bubalis)

    PubMed Central

    Albarella, Sara; Ciotola, Francesca; D’Anza, Emanuele; Coletta, Angelo; Zicarelli, Luigi; Peretti, Vincenzo

    2017-01-01

    Simple Summary Congenital malformations (due to genetic causes) represent a hidden danger for animal production, above all when genetic selection is undertaken for production improvements. These malformations are responsible for economic losses either because they reduce the productivity of the farm, or because their spread in the population would decrease the total productivity of that species/breed. River buffalo is a species of increasing interest all over the world for its production abilities, as proved by the buffalo genome project and the genetic selection plans that are currently performed in different countries. The aim of this review is to provide a general view of different models of congenital malformations in buffalo and their world distribution. This would be useful either for those who performed buffalo genetic selection or for researchers in genetic diseases, which would be an advantage to their studies with respect to the knowledge of gene mutations and interactions in this species. Abstract The world buffalo population is about 168 million, and it is still growing, in India, China, Brazil, and Italy. In these countries, buffalo genetic breeding programs have been performed for many decades. The occurrence of congenital malformations has caused a slowing of the genetic progress and economic loss for the breeders, due to the death of animals, or damage to their reproductive ability or failing of milk production. Moreover, they cause animal welfare reduction because they can imply foetal dystocia and because the affected animals have a reduced fitness with little chances of survival. This review depicts, in the river buffalo (Bubalus bubalis) world population, the present status of the congenital malformations, due to genetic causes, to identify their frequency and distribution in order to develop genetic breeding plans able to improve the productive and reproductive performance, and avoid the spreading of detrimental gene variants. Congenital

  10. The ability to listen with independent ears.

    PubMed

    Gallun, Frederick J; Mason, Christine R; Kidd, Gerald

    2007-11-01

    In three experiments, listeners identified speech processed into narrow bands and presented to the right ("target") ear. The ability of listeners to ignore (or even use) conflicting contralateral stimulation was examined by presenting various maskers to the target ear ("ipsilateral") and nontarget ear ("contralateral"). Theoretically, an absence of contralateral interference would imply selectively attending to only the target ear; the presence of interference from the contralateral stimulus would imply that listeners were unable to treat the stimuli at the two ears independently; and improved performance in the presence of informative contralateral stimulation would imply that listeners can process the signals at both ears and keep them separate rather than combining them. Experiments showed evidence of the ability to selectively process (or respond to) only the target ear in some, but not all, conditions. No evidence was found for improved performance due to contralateral stimulation. The pattern of interference found across experiments supports an interaction of stimulus-based factors (auditory grouping) and task-based factors (demand for processing resources) and suggests that listeners may not always be able to listen to the "better" ear even when it would be beneficial to do so.

  11. 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…

  12. 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....

  13. INNER EAR EMBRYOGENESIS: GENETIC AND ENVIRONMENTAL DETERMINANTS

    EPA Science Inventory

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

  14. Stem Cell Therapy for the Inner Ear

    PubMed Central

    Okano, Takayuki

    2012-01-01

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

  15. 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 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....

  16. 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....

  17. 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 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....

  18. 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 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....

  19. 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

  20. 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.

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

    PubMed Central

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

    2014-01-01

    Object Descriptions of temporal lobe arteriovenous malformations (AVMs) are inconsistent. To standardize reporting, the authors blended existing descriptions in the literature into an intuitive classification with 5 anatomical subtypes: lateral, medial, basal, sylvian, and ventricular. The authors’ surgical experience with temporal lobe AVMs was reviewed according to these subtypes. Methods Eighty-eight patients with temporal lobe AVMs were treated surgically. Results Lateral temporal lobe AVMs were the most common (58 AVMs, 66%). Thirteen AVMs (15%) were medial, 9 (10%) were basal, and 5 (6%) were sylvian. Ventricular AVMs were least common (3 AVMs, 3%). A temporal craniotomy based over the ear was used in 64%. Complete AVM resection was achieved in 82 patients (93%). Four patients (5%) died in the perioperative period (6 in all were lost to follow-up); 71 (87%) of the remaining 82 patients had good outcomes (modified Rankin Scale scores 0–2); and 68 (83%) were unchanged or improved after surgery. Conclusions Categorization of temporal AVMs into subtypes can assist with surgical planning and also standardize reporting. Lateral AVMs are the easiest to expose surgically, with circumferential access to feeding arteries and draining veins at the AVM margins. Basal AVMs require a subtemporal approach, often with some transcortical dissection through the inferior temporal gyrus. Medial AVMs are exposed tangentially with an orbitozygomatic craniotomy and transsylvian dissection of anterior choroidal artery and posterior cerebral artery feeders in the medial cisterns. Medial AVMs posterior to the cerebral peduncle require transcortical approaches through the temporooccipi tal gyrus. Sylvian AVMs require a wide sylvian fissure split and differentiation of normal arteries, terminal feeding arteries, and transit arteries. Ventricular AVMs require a transcortical approach through the inferior temporal gyrus that avoids the Meyer loop. Surgical results with temporal lobe

  2. [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. 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.

  4. Cytogenomic Aberrations in Congenital Cardiovascular Malformations

    PubMed Central

    Azamian, Mahshid; Lalani, Seema R.

    2016-01-01

    Congenital cardiovascular malformations are the most common birth defects, with a complex multifactorial etiology. Genetic factors play an important role, illuminated by numerous cytogenetically visible abnormalities, as well as submicroscopic genomic imbalances affecting critical genomic regions in the affected individuals. Study of rare families with Mendelian forms, as well as emerging next-generation sequencing technologies have uncovered a multitude of genes relevant for human congenital cardiac diseases. It is clear that the complex embryology of human cardiac development, with an orchestrated interplay of transcription factors, chromatin regulators, and signal transduction pathway molecules can be easily perturbed by genomic imbalances affecting dosage-sensitive regions. This review focuses on chromosomal abnormalities contributing to congenital heart diseases and underscores several genomic disorders linked to human cardiac malformations in the last few decades. PMID:27385961

  5. Occipitoatlantoaxial malformation in an adult goat.

    PubMed

    Seva, Juan I; Gómez, Serafin; Pallarés, Francisco J; Sánchez, Pedro; Bernabé, Antonio

    2008-09-01

    An occipitoatlantoaxial malformation was diagnosed in a 1-year-old Murciano-Granadina goat. At clinical examination, the head and cranial part of the neck were deviated to the right. Clinical signs of spinal cord or brain disease were not observed. At necropsy, morphological abnormalities were seen in the craniovertebral junction and cervical vertebrae, characterized by a firm attachment and incomplete articulation between the occipital bone and the atlas, and scoliosis in the cervical regions. The definitive diagnosis was bilateral asymmetrical occipitoatlantoaxial fusion with rotation of the atlas and atlantoaxial subluxation. To the authors' knowledge, this case report is the second occipitoatlantoaxial malformation described in a goat and the first description in an adult goat.

  6. Arteriovenous malformation in chronic gastrointestinal bleeding.

    PubMed Central

    Cavett, C M; Selby, J H; Hamilton, J L; Williamson, J W

    1977-01-01

    Arteriovenous malformations of the gastrointestinal tract are uncommon and treatment is problematic because routine barium contrast studies and endoscopy fail to demonstrate the lesion. Diagnosis is by selective mesenteric arteriography, demonstrating a characteristic vascular tuft and very early venous phase. Two cases of arteriovenous malformation are presented and 47 other reported cases are reviewed. Forty-five per cent were found in the cecum; 37, or 80%, involved the distal ileum, cecum ascending colon, or hepatic flexure. Seventy-five per cent of all patients fall into the 50--80 year age range. The literature reveals a recurring pattern of chronic gastrointestinal blood loss, anemia, and delay (even negative abdominal explorations) before the diagnosis is finally made. A more aggressive approach to chronic gastrointestinal bleeding is suggested through the use of selective mesenteric arteriography. Images Fig. 1. Fig. 2. Fig. 3. Fig. 4. Fig. 5. PMID:299801

  7. 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.

  8. 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

  9. 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.

  10. [Ear keloid and clinical research progress].

    PubMed

    Du, Guangyuan; Zhu, Jiang

    2014-04-01

    Keloid refers to the damaged skin due to excessive fibroblast proliferation. Ear is one predilection site. The pathogenesis of ear keloid is not very clear, and the treatment is also varied. Surgery, postoperative radiotherapy and laser treatment, steroid hormones, pressure therapy are the basic treatment methods. Integrated application of a variety of treatments, classification research and new materials using revealed the prospect for the treatment of the disease. This thesis reviews literature about ear keloid in recent 10 years, and introduces this disease and clinical research progress.

  11. 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.

  12. [Diabetic mother's newborn with Goldenhar syndrome and cerebral malformations. Case report].

    PubMed

    Kerckoff Villanueva, Hugo; Retamoza, Beatriz; Bautista, Armando

    2008-11-01

    Goldenhar syndrome, also known as oculo-auriculo-vertebral syndrome, is a rare illness with unknown etiology. It happens in 1 of 50,000 newborns and is characterized by first and second brachial arch changes, with variable grades of hemifacial hypoplasia. We report a clinical case of a 39-years-old woman with a BMI of 33, family history of diabetes mellitus decease, and two fetal losses with unknown etiology. We first met patient at 24 gestational weeks. Second level ultrasound findings were: fetus with tri-ventricular obstructive hydrocephalia, symmetrical macrocranium, and occipital encephalocele. As her capilar glycaemia level was 197 to 338 mg/dL, diagnosis was gestational diabetes. Patient was scheduled to cesarian section and we obtain a newborn with: 2,050 g of weight, 45 cm of height, 38 cm of cephalic diameter, 6/7 Apgar score, and 33 gestational weeks (Capurro). Obesity and 2-type diabetes are well-known fetal malformation etiologies, due to insulin resistance. Maternal hyperglycemia causes oxidative stress in embryo cells, it changes Pax3 gene path, and causes several chromosomopaties and malformations; main perinatal mortality etiology, among them, are neural tube alterations. All together enable to establish this Goldenhar diagnosis: palpebral coloboma, facial asymmetry, external ear, upper hemivertebra, and microphthalmy.

  13. Profound microcephaly, primordial dwarfism with developmental brain malformations: a new syndrome.

    PubMed

    Abdel-Salam, Ghada M H; Abdel-Hamid, Mohamed S; Saleem, Sahar N; Ahmed, Mahmoud K H; Issa, Mahmoud; Effat, Laila K; Kayed, Hisham F; Zaki, Maha S; Gaber, Khaled R

    2012-08-01

    We describe two sibs with a lethal form of profound congenital microcephaly, intrauterine and postnatal growth retardation, subtle skeletal changes, and poorly developed brain. The sibs had striking absent cranial vault with sloping of the forehead, large beaked nose, relatively large ears, and mandibular micro-retrognathia. Brain magnetic resonance imaging (MRI) revealed extremely simplified gyral pattern, large interhemispheric cyst and agenesis of corpus callosum, abnormally shaped hippocampus, and proportionately affected cerebellum and brainstem. In addition, fundus examination showed foveal hypoplasia with optic nerve atrophy. No abnormalities of the internal organs were found. This profound form of microcephaly was identified at 17 weeks gestation by ultrasound and fetal brain MRI helped in characterizing the developmental brain malformations in the second sib. Molecular analysis excluded mutations in potentially related genes such as RNU4ATAC, SLC25A19, and ASPM. These clinical and imaging findings are unlike that of any recognized severe forms of microcephaly which is believed to be a new microcephalic primordial dwarfism (MPD) with developmental brain malformations with most probably autosomal recessive inheritance based on consanguinity and similarly affected male and female sibs.

  14. Embolization and radiosurgery for arteriovenous malformations

    PubMed Central

    Plasencia, Andres R.; Santillan, Alejandro

    2012-01-01

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

  15. Arteriovenous malformations: epidemiology and clinical presentation.

    PubMed

    Laakso, Aki; Hernesniemi, Juha

    2012-01-01

    Arteriovenous malformations (AVMs) of the brain are relatively rare congenital developmental vascular lesions. They may cause hemorrhagic stroke, epilepsy, chronic headache, or focal neurologic deficits, and the incidence of asymptomatic AVMs is increasing due to widespread availability of noninvasive imaging methods. Since the most severe complication of an AVM is hemorrhagic stroke, most epidemiologic studies have concentrated on the hemorrhage risk and its risk factors. In this article, the authors discuss the epidemiology, presenting symptoms, and hemorrhage risk associated with brain AVMs.

  16. Debendox and congenital malformations in Northern Ireland.

    PubMed Central

    Harron, D W; Griffiths, K; Shanks, R G

    1980-01-01

    An investigation was carried out in Northern Ireland into the alleged association between fetal abnormalities and Debendox, an antiemetic drug used in pregnancy. During the period 1966-78 the total number of births each year and the overall incidence of congenital malformations per 10 000 births fell. The incidences of cleft lip, cleft palate, reduction deformities, and defects of the heart and great vessels fell from 1966 to 1976 but increased in 1977 and 1978. During the same period (1966-78) the number of prescriptions for Debendox issued by general practitioners increased more than fourfold. These observations suggest that there is no relation between congenital malformations and the use of Debendox. This conclusion, however, does not take into account other drug- or environmental-related factors that may have resulted in a reduction in the number of congenital malformations and would hence have masked an increase associated with greater usage of Debendox. In particular, the amount of Debendox sold direct to the public without a prescription and the use of the drug by patients who were not pregnant could not be established. The amount of drug used in these ways is probably small, and it is difficult to see how it might influence the conclusions reached. PMID:7437804

  17. Debendox and congenital malformations in Northern Ireland.

    PubMed

    Harron, D W; Griffiths, K; Shanks, R G

    1980-11-22

    An investigation was carried out in Northern Ireland into the alleged association between fetal abnormalities and Debendox, an antiemetic drug used in pregnancy. During the period 1966-78 the total number of births each year and the overall incidence of congenital malformations per 10 000 births fell. The incidences of cleft lip, cleft palate, reduction deformities, and defects of the heart and great vessels fell from 1966 to 1976 but increased in 1977 and 1978. During the same period (1966-78) the number of prescriptions for Debendox issued by general practitioners increased more than fourfold. These observations suggest that there is no relation between congenital malformations and the use of Debendox. This conclusion, however, does not take into account other drug- or environmental-related factors that may have resulted in a reduction in the number of congenital malformations and would hence have masked an increase associated with greater usage of Debendox. In particular, the amount of Debendox sold direct to the public without a prescription and the use of the drug by patients who were not pregnant could not be established. The amount of drug used in these ways is probably small, and it is difficult to see how it might influence the conclusions reached.

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

    PubMed

    Simán, M

    1997-01-01

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

  19. Evolution: Fossil Ears and Underwater Sonar.

    PubMed

    Lambert, Olivier

    2016-08-22

    A key innovation in the history of whales was the evolution of a sonar system together with high-frequency hearing. Fossils of an archaic toothed whale's inner ear bones provide clues for a stepwise emergence of underwater echolocation ability.

  20. Ear Infection Treatment: Do Alternative Therapies Work?

    MedlinePlus

    ... recommended for use in children — some have dangerous side effects or may interfere with conventional treatments. By Mayo Clinic Staff Alternative ear infection treatments abound on the internet and in books and magazines. They include chiropractic adjustments, homeopathy, herbal ...

  1. Diode Laser Ear Piercing: A Novel Technique.

    PubMed

    Suseela, Bibilash Babu; Babu, Preethitha; Chittoria, Ravi Kumar; Mohapatra, Devi Prasad

    2016-01-01

    Earlobe piercing is a common office room procedure done by a plastic surgeon. Various methods of ear piercing have been described. In this article, we describe a novel method of laser ear piercing using the diode laser. An 18-year-old female patient underwent an ear piercing using a diode laser with a power of 2.0 W in continuous mode after topical local anaesthetic and pre-cooling. The diode laser was fast, safe, easy to use and highly effective way of ear piercing. The advantages we noticed while using the diode laser over conventional methods were more precision, minimal trauma with less chances of hypertrophy and keloids, no bleeding with coagulation effect of laser, less time taken compared to conventional method and less chance of infection due to thermal heat effect of laser.

  2. Design Factors and Use of Ear Protection*

    PubMed Central

    Rice, C. G.; Coles, R. R. A.

    1966-01-01

    The problems of protecting the ear against hazardous noise are the subject of a general review, supported where relevant by data from the authors' own researches. Ear protectors are classified into two main types−plugs and muffs—and the general principles of their function and limitations are stated. Examples of representative ear protectors are given in more detail, with particular respect to their relative merits and pure-tone attenuation characteristics. The effects of earplugs on speech communication are considered and the relationships between pure-tone attenuation and protection against continuous noise are discussed in some detail. The results of temporary threshold shift (T.T.S.) reduction studies of the efficiency of V.51R and Selectone-K earplugs in protecting against reverberant and non-reverberant impulsive noises are presented. The design requirements of ear protectors and some of the problems created by them are also outlined. Images PMID:5946129

  3. Diode Laser Ear Piercing: A Novel Technique

    PubMed Central

    Suseela, Bibilash Babu; Babu, Preethitha; Chittoria, Ravi Kumar; Mohapatra, Devi Prasad

    2016-01-01

    Earlobe piercing is a common office room procedure done by a plastic surgeon. Various methods of ear piercing have been described. In this article, we describe a novel method of laser ear piercing using the diode laser. An 18-year-old female patient underwent an ear piercing using a diode laser with a power of 2.0 W in continuous mode after topical local anaesthetic and pre-cooling. The diode laser was fast, safe, easy to use and highly effective way of ear piercing. The advantages we noticed while using the diode laser over conventional methods were more precision, minimal trauma with less chances of hypertrophy and keloids, no bleeding with coagulation effect of laser, less time taken compared to conventional method and less chance of infection due to thermal heat effect of laser. PMID:28163460

  4. 21 CFR 874.4140 - Ear, nose, and throat bur.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ...) 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 is... 21 Food and Drugs 8 2010-04-01 2010-04-01 false Ear, nose, and throat bur. 874.4140 Section...

  5. 21 CFR 874.4140 - Ear, nose, and throat bur.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ...) 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 is... 21 Food and Drugs 8 2011-04-01 2011-04-01 false Ear, nose, and throat bur. 874.4140 Section...

  6. 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...

  7. 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...

  8. 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...

  9. 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...

  10. 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...

  11. 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...

  12. 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...

  13. Middle Ear Surgery in Only Hearing Ears and Postoperative Hearing Rehabilitation

    PubMed Central

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

    2014-01-01

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

  14. Inheritance of ear wax types, ear lobe attachment and tongue rolling ability.

    PubMed

    Cruz-Gonzalez, L; Lisker, R

    1982-01-01

    The mode of inheritance of ear wax type, ear lobe attachment and tongue rolling ability were studied in 77 families with a total of 293 children. The results clearly showed that the dry ear wax type and the attached ear lobe type represent the homozygous state for two pairs of autosomal recessive genes. The evidence for the same being true regarding the lack of ability to roll the tongue was less conclusive in our material, but this could be due to difficulties in communication between the examined individuals and the examiners.

  15. Malformations in neotropical viperids: qualitative and quantitative analysis.

    PubMed

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

    2013-11-01

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

  16. 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

  17. Binaural versus better-ear listening

    NASA Astrophysics Data System (ADS)

    Scarpaci, Jacob W.; Durlach, N. I.; Colburn, H. Steven

    2003-04-01

    Advantages of binaural over monaural hearing in noisy environments are reduced when the monaural stimulation is derived from the monaural signal with the better signal-to-noise ratio (better-ear listening). In the reported experiments, conducted in a soundproof room with two speakers and a custom-designed, noise-cancellation headset, speech intelligibility in the presence of interference was measured for both binaural and better-ear configurations. The headset, which incorporated two microphones (located at the two ears) and two insert earphones, was used to present binaural stimulation or better-ear (better-microphone) monaural stimulation. Although the results varied significantly with the locations of the target and interference sources, the advantage of binaural listening over better-ear listening was no more than a few dB. In addition to reporting the data obtained in these experiments, relations to previous work on better-ear listening and CROS hearing aids, as well as to current work on cochlear implants, are discussed. [Work supported by NIDCD (00100).

  18. 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

  19. Chiari Type I malformation presenting with bilateral hearing loss.

    PubMed

    Dolgun, Habibullah; Turkoglu, Erhan; Kertmen, Hayri; Yilmaz, Erdal R; Sekerci, Zeki

    2009-09-01

    Chiari Type I malformations can present with several clinical signs and symptoms. We describe a 44-year-old female patient presenting with bilateral hearing loss with hydrocephalus coexisting with Chiari Type I malformation and a unilateral arachnoid cyst. Thus, sensorineural hearing loss may be caused by hydrocephalus with Chiari Type I malformation. The placement of a ventriculoperitoneal shunt without a posterior fossa decompression is an effective treatment.

  20. [Diagnosis of fetal malformations with ultrasound--state of development].

    PubMed

    Fendel, M; Fendel, H

    1983-01-01

    Ultrasonography is of great importance for the prenatal diagnosis of fetal malformations and abnormalities. An early diagnosis in the second trimester is of great interest for an intrauterine or an extrauterine therapy planning (the choice of the time and mode of delivery). Defects of the neural tube including hydrocephalus, malformations of the extremities, the gastrointestinal tract, omphaloceles, the urogenital and cardiac system are described. Four cases of fetal malformations are presented: fetal myelomeningocele, hydrocephalus, bilateral hydronephrosis and lymphangioma with fetal ascites.

  1. Notch receptor expression in human brain arteriovenous malformations.

    PubMed

    Hill-Felberg, Sandra; Wu, Hope Hueizhi; Toms, Steven A; Dehdashti, Amir R

    2015-08-01

    The roles of the Notch pathway proteins in normal adult vascular physiology and the pathogenesis of brain arteriovenous malformations are not well-understood. Notch 1 and 4 have been detected in human and mutant mice vascular malformations respectively. Although mutations in the human Notch 3 gene caused a genetic form of vascular stroke and dementia, its role in arteriovenous malformations development has been unknown. In this study, we performed immunohistochemistry screening on tissue microarrays containing eight surgically resected human brain arteriovenous malformations and 10 control surgical epilepsy samples. The tissue microarrays were evaluated for Notch 1-4 expression. We have found that compared to normal brain vascular tissue Notch-3 was dramatically increased in brain arteriovenous malformations. Similarly, Notch 4 labelling was also increased in vascular malformations and was confirmed by western blot analysis. Notch 2 was not detectable in any of the human vessels analysed. Using both immunohistochemistry on microarrays and western blot analysis, we have found that Notch-1 expression was detectable in control vessels, and discovered a significant decrease of Notch 1 expression in vascular malformations. We have demonstrated that Notch 3 and 4, and not Notch 1, were highly increased in human arteriovenous malformations. Our findings suggested that Notch 4, and more importantly, Notch 3, may play a role in the development and pathobiology of human arteriovenous malformations.

  2. A case report: Pulmonary venous malformation complicated with pulmonary hemorrhage.

    PubMed

    Supakul, Nucharin; Fan, Rong; Karmazyn, Boaz

    2012-12-01

    Pulmonary venous malformation is extremely rare. We present imaging and clinical findings of a 17-year-old male with multifocal subcutaneous venous malformations and multiple cystic lesions in the liver and spleen, suggestive of slow flow vascular malformation. In the right lung, chest radiography followed by chest CT demonstrated large tortuous pulmonary veins and cystic emphysematous changes. Tc99m-MAA (pertechnetate-labeled macroaggregated albumin) lung perfusion scan demonstrated only 3% of normal perfusion to the right lung, with no evidence of arteriovenous shunting. The child had diffuse intraparenchymal hemorrhage throughout the right lower and middle lobes and underwent resection. Pathology confirmed the diagnosis of venous malformation complicated with bleeding.

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

    PubMed

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

    2014-03-01

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

  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. 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

  7. BIOLOGY OF VASCULAR MALFORMATIONS OF THE BRAIN

    PubMed Central

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

    2009-01-01

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

  8. 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.

  9. 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

  10. Oral vascular malformations: laser treatment and management

    NASA Astrophysics Data System (ADS)

    Romeo, U.; Rocchetti, F.; Gaimari, G.; Tenore, G.; Palaia, G.; Lo Giudice, G.

    2016-03-01

    Vascular malformations are a very heterogeneous group of circulatory system's diseases that can involve different kind of vessels: arterial, venous or lymphatic ones. Many treatments, such as conventional surgery, embolization, steroid therapy and laser therapy, are available for vascular lesions. The laser approach relies more therapeutic techniques: the transmucosal thermophotocoagulation, intralesional photocoagulation, the excisional biopsy. Today laser is demonstrated to be the gold standard technique to treat vascular lesions that allows a safe and efficient treatment and a lower post-operative healing time. The only disadvantage is the risk of carbonization that could be avoided by using the multiple-spot single pulsed wave technique.

  11. 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

  12. 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.

  13. Histology and synchrotron radiation-based microtomography of the inner ear in a molecularly confirmed case of CHARGE syndrome.

    PubMed

    Glueckert, Rudolf; Rask-Andersen, Helge; Sergi, Consolato; Schmutzhard, Joachim; Mueller, Bert; Beckmann, Felix; Rittinger, Olaf; Hoefsloot, Lies H; Schrott-Fischer, Anneliese; Janecke, Andreas R

    2010-03-01

    CHARGE (Coloboma of the iris or retina, heart defects, atresia of the choanae, retardation of growth and/or development, genital anomalies, ear anomalies) syndrome (OMIM #214800) affects about 1 in 10,000 children and is most often caused by chromodomain helicase DNA-binding protein-7 (CHD7) mutations. Inner ear defects and vestibular abnormalities are particularly common. Specifically, semicircular canal (SCC) hypoplasia/aplasia and the presence of a Mondini malformation can be considered pathognomonic in the context of congenital malformations of the CHARGE syndrome. We obtained a temporal bone (TB) of a patient with CHARGE syndrome who died from bacteremia at 3 months of age. The clinical diagnosis was confirmed in the patient by direct DNA sequencing and the detection of a de novo, truncating CHD7 mutation, c.6169dup (p.R2057fs). We assessed changes of the TB and the degree of neural preservation, which may influence the potential benefit of cochlear implantation. The TB was analyzed using synchrotron radiation-based micro computed tomography, and by light microscopy. The vestibular partition consisted of a rudimentary vestibule with agenesis of the SCCs. The cochlea was hypoplastic with poor or deficient interscaling and shortened (Mondini dysplasia). The organ of Corti had near normal structure and innervation. Modiolus and Rosenthal's canal were hypoplastic with perikarya displaced along the axon bundles into the internal acoustic meatus, which may be explained by the arrest or limited migration and translocation of the cell nuclei into the cochlear tube during development.

  14. 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

  15. 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.

  16. 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

  17. Stenogyria - not only in Chiari II malformation.

    PubMed

    Bekiesinska-Figatowska, Monika; Duczkowska, Agnieszka; Brągoszewska, Hanna; Duczkowski, Marek; Mierzewska, Hanna

    2014-12-15

    Stenogyria, meaning multiple small compacted gyri separated by shallow sulci, is reported in the literature in association with Chiari II malformation (CM II) which in turn is reported in association with myelomeningocele (MMC). The authors present five cases of stenogyria (and other abnormalities found in CM II, like callosal hypoplasia/dysplasia, agenesis of the anterior commissure, hypoplasia of the falx cerebri) in children without the history of MMC or any other form of open spinal dysraphism. In these cases stenogyria was associated with Chiari I malformation, rhombencephalosynapsis and spina bifida. Stenogyria, which is not a true neuronal migration disorder, should not be mistaken for polymicrogyria which is also present in CM II. It is histologically different from polymicrogyria because the cortex is normally organized. Also on MRI, the general sulcal pattern is preserved in stenogyria, while it is completely distorted in polymicrogyria. The authors conclude that features traditionally attributed to CM II, like stenogyria, occur not only in the population of patients with MMC as opposed to the widely accepted theory.

  18. 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.

  19. Sirenomelia and caudal malformations in two families.

    PubMed

    Gerard, Marion; Layet, Valérie; Costa, Teresa; Roumazeilles, Yves; Chenal, Pierre; Cailliez, Daniel; Gerard, Bénédicte

    2012-07-01

    We report on two families with co-occurrence of sirenomelia and caudal malformations. In the first family, the mother had undergone surgery for a short form of imperforate anus. Her first pregnancy was terminated because of bilateral renal agenesis with oligohydramnios. Her second pregnancy was interrupted because of sirenomelia. The second family was referred to us because of caudal malformation in their two children. The parents' spinal radiographs were normal. The first pregnancy resulted in a girl with imperforate anus, absence of S3-S5 and coccyx, abnormal pelvic floor, and an almost bifid anteriorly located bladder. The second pregnancy resulted in a baby girl with sirenomelia. No diabetes was present during the pregnancies in either of these two families. These families confirm the hypothesis that major genes are responsible for the embryogenesis of the caudal part of the embryo, with variable expression, as has been already described in sirenomelia mouse models (CYP26A1, BMP7/tsg). Molecular studies are underway in these families and in sporadic cases in our laboratory to explore the genetic basis of sirenomelia in humans.

  20. Pathogenesis and Cerebrospinal Fluid Hydrodynamics of the Chiari I Malformation.

    PubMed

    Buell, Thomas J; Heiss, John D; Oldfield, Edward H

    2015-10-01

    This article summarizes the current understanding of the pathophysiology of the Chiari I malformation that is based on observations of the anatomy visualized by modern imaging with MRI and prospective studies of the physiology of patients before and after surgery. The pathogenesis of a Chiari I malformation of the cerebellar tonsils is grouped into 4 general mechanisms.

  1. Glomuvenous Malformation: A Rare Periorbital Lesion of the Thermoregulatory Apparatus.

    PubMed

    Whipple, Katherine M; Godfrey, Kyle J; Solomon, James P; Lin, Jonathan H; Korn, Bobby S; Kikkawa, Don O

    Glomuvenous malformations (GVMs), previously referred to as glomus tumors or glomangiomas, are benign, mesenchymal venous malformations arising from glomus bodies. Glomus bodies are modified smooth muscle neuromyoarterial structures involved in temperature regulation via blood shunting. These classically occur in the digits but can occur in other locations. The authors present a case of a periorbital GVM presented following blunt trauma to the area.

  2. Congenital plaque-type glomuvenous malformation associated with chylous ascites.

    PubMed

    Tejedor, Maria; Martín-Santiago, Ana; Gómez, Cristina; Fiol, Miquel; Benítez-Segura, Ignacio

    2010-01-01

    Congenital plaque-type glomuvenous malformation (GVM) is caused by loss of function mutations in glomulin gene. We report a newborn with this rare vascular disorder associated with chylous ascites. The common mesenchymal origin of GVM and lymphatic vessels as well as the glomulin expression in vascular smooth muscle cells in utero could help explain this unusual prenatal complication of glomuvenous malformations.

  3. Klippel-Feil syndrome and Dandy-Walker malformation.

    PubMed

    Karaman, A; Kahveci, H

    2011-01-01

    The Klippel-Feil deformity is a complex of osseous and visceral anomalies, which include low hairline, platybasia, fused cervical vertebrae with a short neck, and deafness. Associated central nervous system abnormalities include occipital cephalocele, Chiari I malformation, syrinx, microcephaly, and hydrocephalus. Herein, we report a case with Klippel-Feil syndrome and Dandy-Walker malformation.

  4. 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.

  5. 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.

  6. Endoscopic anatomy of the pediatric middle ear.

    PubMed

    Isaacson, Glenn

    2014-01-01

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

  7. Malformations of cortical development: genetic mechanisms and diagnostic approach

    PubMed Central

    2017-01-01

    Malformations of cortical development are rare congenital anomalies of the cerebral cortex, wherein patients present with intractable epilepsy and various degrees of developmental delay. Cases show a spectrum of anomalous cortical formations with diverse anatomic and morphological abnormalities, a variety of genetic causes, and different clinical presentations. Brain magnetic resonance imaging has been of great help in determining the exact morphologies of cortical malformations. The hypothetical mechanisms of malformation include interruptions during the formation of cerebral cortex in the form of viral infection, genetic causes, and vascular events. Recent remarkable developments in genetic analysis methods have improved our understanding of these pathological mechanisms. The present review will discuss normal cortical development, the current proposed malformation classifications, and the diagnostic approach for malformations of cortical development. PMID:28203254

  8. Venous malformations: classification, development, diagnosis, and interventional radiologic management.

    PubMed

    Legiehn, Gerald M; Heran, Manraj K S

    2008-05-01

    Venous malformations are categorized as low-flow vascular malformations within the domain of vascular anomalies and are the most common vascular malformation encountered clinically. Venous malformations are by definition present at birth, undergo pari passu growth, and present clinically because of symptoms related to mass effect or stasis. Although diagnosis can usually be made by clinical history and examination, differentiation from other vascular and nonvascular entities often requires an imaging work-up that includes ultrasound, CT, MR imaging, and diagnostic phlebography. All decisions regarding imaging work-up and decision to treat must be coordinated though referral and discussions with a multidisciplinary team and be based on clearly defined clinical indications. Percutaneous image-guided sclerotherapy has become the mainstay of treatment for venous malformations and involves the introduction of any one of a number of endothelial-cidal sclerosants into the vascular spaces of the lesion, with each sclerosant possessing its own unique spectrum of advantages and disadvantages.

  9. 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

  10. Systemic antibiotic prophylaxis and reconstructive ear surgery.

    PubMed

    Carlin, W V; Lesser, T H; John, D G; Fielder, C; Carrick, D G; Thomas, P L; Hill, S

    1987-12-01

    This paper reports a multicentre, controlled, blind, prospective, randomized study into the use of prophylactic systemic antibiotics in myringoplasty surgery. Seventy-one individuals were clinically and bacteriologically assessed both preoperatively, and for a period of 8 weeks postoperatively. The results showed that antibiotic prophylaxis did not eradicate bacterial pathogens already present in preoperative ears, nor did it prevent their development during the postoperative period. The observation that an ear was wet or dry gave no indication of the actual presence or absence of pathogenic organisms.

  11. Modeling of sound transmission from ear canal to cochlea.

    PubMed

    Gan, Rong Z; Reeves, Brian P; Wang, Xuelin

    2007-12-01

    A 3-D finite element (FE) model of the human ear consisting of the external ear canal, middle ear, and cochlea is reported in this paper. The acoustic-structure-fluid coupled FE analysis was conducted on the model which included the air in the ear canal and middle ear cavity, the fluid in the cochlea, and the middle ear and cochlea structures (i.e., bones and soft tissues). The middle ear transfer function such as the movements of tympanic membrane, stapes footplate, and round window, the sound pressure gain across the middle ear, and the cochlear input impedance in response to sound stimulus applied in the ear canal were derived and compared with the published experimental measurements in human temporal bones. The frequency sensitivity of the basilar membrane motion and intracochlear pressure induced by sound pressure in the ear canal was predicted along the length of the basilar membrane from the basal turn to the apex. The satisfactory agreements between the model and experimental data in the literature indicate that the middle ear function was well simulated by the model and the simplified cochlea was able to correlate sound stimulus in the ear canal with vibration of the basilar membrane and pressure variation of the cochlear fluid. This study is the first step toward the development of a comprehensive FE model of the entire human ear for acoustic-mechanical analysis.

  12. Acoustics of the human middle-ear air space.

    PubMed

    Stepp, Cara E; Voss, Susan E

    2005-08-01

    The impedance of the middle-ear air space was measured on three human cadaver ears with complete mastoid air-cell systems. Below 500 Hz, the impedance is approximately compliance-like, and at higher frequencies (500-6000 Hz) the impedance magnitude has several (five to nine) extrema. Mechanisms for these extrema are identified and described through circuit models of the middle-ear air space. The measurements demonstrate that the middle-ear air space impedance can affect the middle-ear impedance at the tympanic membrane by as much as 10 dB at frequencies greater than 1000 Hz. Thus, variations in the middle-ear air space impedance that result from variations in anatomy of the middle-ear air space can contribute to inter-ear variations in both impedance measurements and otoacoustic emissions, when measured at the tympanic membrane.

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

    PubMed

    Xie, Haiyuan; Qian, Mingli

    2013-09-01

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

  14. Is Ear Candling a Safe Way to Remove Earwax?

    MedlinePlus

    Healthy Lifestyle Consumer health Is ear candling a safe way to remove earwax? Answers from Charles W. Beatty, ... 05, 2015 Original article: http://www.mayoclinic.org/healthy-lifestyle/consumer-health/expert-answers/ear-candling/faq-20058212 . ...

  15. 38 CFR 4.87 - Schedule of ratings-ear.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... recurrent tinnitus, whether the sound is perceived in one ear, both ears, or in the head. Note (3): Do not evaluate objective tinnitus (in which the sound is audible to other people and has a definable cause...

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

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... recurrent tinnitus, whether the sound is perceived in one ear, both ears, or in the head. Note (3): Do not evaluate objective tinnitus (in which the sound is audible to other people and has a definable cause...

  17. 38 CFR 4.87 - Schedule of ratings-ear.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... recurrent tinnitus, whether the sound is perceived in one ear, both ears, or in the head. Note (3): Do not evaluate objective tinnitus (in which the sound is audible to other people and has a definable cause...

  18. 38 CFR 4.87 - Schedule of ratings-ear.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... recurrent tinnitus, whether the sound is perceived in one ear, both ears, or in the head. Note (3): Do not evaluate objective tinnitus (in which the sound is audible to other people and has a definable cause...

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

    MedlinePlus

    ... Although there is no proven medical link between middle ear infections and pediatric obesity there may be a behavioral association between the two conditions. Some studies have found that when a child is rubbing or massaging the infected ear the ...

  20. Diagnosis and management of pulmonary arteriovenous malformations

    PubMed Central

    Papagiannis, J; Apostolopoulou, S; Sarris, GE; Rammos, S

    2002-01-01

    Pulmonary arteriovenous malformation is a rare anomaly that presents in several different ways. It can present as an isolated finding, or more often in the context of hereditary haemorrhagic telangiectasia. It can also complicate palliative surgery such as the Glenn operation for complex congenital heart disease with single ventricle physiology. Its management includes transcatheter embolization, which is the preferred mode of therapy, surgery (including resection of the affected lobe, segment, or the fistula itself), or rarely, medical therapy. Complications of the disease itself and of various modes of treatment are relatively common, and patients require close surveillance for possible recurrence, or development of new fistulas. In cases related to the Glenn operation, redirection of hepatic venous flow or heart transplantation may cure the problem. PMID:22368610

  1. Congenital cardiovascular malformations and the fetal circulation.

    PubMed

    Rudolph, A M

    2010-03-01

    After birth, gas exchange is achieved in the lung, whereas prenatally it occurs in the placenta. This is associated with differences in blood flow patterns in the fetus as compared with the postnatal circulation. Congenital cardiovascular malformations are associated with haemodynamic changes in the fetus, which differ from those occurring postnatally. Obstruction to cardiac outflow may alter myocardial development, resulting in progressive ventricular hypoplasia. Alteration of oxygen content may profoundly influence pulmonary vascular and ductus arteriosus responses. Interference in blood flow and oxygen content may affect cerebral development as a result of inadequate oxygen or energy substrate supply. The circulatory effects may be gestational dependent, related to maturation of vascular responses in different organs. These prenatal influences of congenital cardiac defects may severely affect immediate, as well as longterm, postnatal prognosis and survival. This has stimulated the development of techniques for palliation of disturbed circulation during fetal life.

  2. Small Rho-GTPases and cortical malformations

    PubMed Central

    2013-01-01

    Rho-GTPases have been found to be crucial for cytoskeleton remodelling and cell polarity, as well as key players in directed cell migration in various tissues and organs, therefore becoming good candidates for involvement in neuronal migration disorders. We recently found that genetic deletion of the small GTPase RhoA in the developing mouse cerebral cortex results in three distinct cortical malformations: a defect in the proliferation of progenitor cells during development that leads to a bigger cerebral cortex in the adult mouse, a change in the morphology of radial glial cells that results in the formation of a subcortical band heterotopia (SBH, also called Double Cortex) and an increase in the speed of migrating newborn neurons. The latter, together with the aberrant radial glial shape, is likely to be the cause of cobblestone lissencephaly, where neurons protrude beyond layer I at the pial surface of the brain. PMID:23524873

  3. Multimodal device for assessment of skin malformations

    NASA Astrophysics Data System (ADS)

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

    2013-11-01

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

  4. Congenital malformations of the brain and spine.

    PubMed

    Shankar, Prashant; Zamora, Carlos; Castillo, Mauricio

    2016-01-01

    In this chapter we briefly address the most common congenital brain and spinal anomalies as well as their most salient imaging, especially magnetic resonance, findings. Some of them, such as Chiari II, and open spinal defects, have become relatively rare due to their detection in utero and repair of the spinal malformation. Regardless of the type of brain anomaly, the most common clinical symptoms are mental retardation, hydrocephalus, and seizure; the latter two may need to be surgically and medically addressed. The most commonly found spinal congenital anomalies include the filum terminale lipoma which is generally asymptomatic and incidental and the caudal regression syndrome for which no primary treatment exists. Any spinal congenital anomaly may present in adulthood as a consequence of spinal cord tethering and/or development of syringomyelia.

  5. 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

  6. Cerebral cavernous malformation proteins at a glance.

    PubMed

    Draheim, Kyle M; Fisher, Oriana S; Boggon, Titus J; Calderwood, David A

    2014-02-15

    Loss-of-function mutations in genes encoding KRIT1 (also known as CCM1), CCM2 (also known as OSM and malcavernin) or PDCD10 (also known as CCM3) cause cerebral cavernous malformations (CCMs). These abnormalities are characterized by dilated leaky blood vessels, especially in the neurovasculature, that result in increased risk of stroke, focal neurological defects and seizures. The three CCM proteins can exist in a trimeric complex, and each of these essential multi-domain adaptor proteins also interacts with a range of signaling, cytoskeletal and adaptor proteins, presumably accounting for their roles in a range of basic cellular processes including cell adhesion, migration, polarity and apoptosis. In this Cell Science at a Glance article and the accompanying poster, we provide an overview of current models of CCM protein function focusing on how known protein-protein interactions might contribute to cellular phenotypes and highlighting gaps in our current understanding.

  7. 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

  8. Genetic Screening of Pediatric Cavernous Malformations.

    PubMed

    Merello, Elisa; Pavanello, Marco; Consales, Alessandro; Mascelli, Samantha; Raso, Alessandro; Accogli, Andrea; Cama, Armando; Valeria, Capra; De Marco, Patrizia

    2016-10-01

    Cerebral cavernous malformations (CCMs) are vascular malformations mostly located within the central nervous system. Heterozygous loss of function mutations in CCM1/KRIT1, CCM2/MGC4607, and CCM3/PDCD10 genes are identified in about 90 % of familial cases of CCMs and two thirds of sporadic cases with multiple lesions. In this study, we performed genetic screening of a cohort of 31 patients, mainly pediatric. We analyzed the CCM1, CCM2, and CCM3 genes by multiplex ligation-dependent probe amplification (MLPA) and direct sequencing of exons and intronic boundaries. A total of 9 typical pathogenic loss-of-function mutations were identified in 10 out 31 patients (32 %). The 75 % of familial cases were mutated and the percentage reached to 85 % when we consider only pediatric cases. Detection rate in sporadic cases with multiple lesions was considerably lower (16 %). We identified a novel variant of CCM3, the c.130-131insT (p.R45Efs*8), in 1 pediatric sporadic case with multiple lesions that introduced a premature termination codon into the messenger RNA (mRNA), most likely leading to mRNA decay. Similar to other CCM pediatric series, the main symptoms associated to clinical debut consisted of cerebral hemorrhage. In conclusion, the penetrance of CCM mutations in familial pediatric cases is high (85 %). The genetic workup could improve clinical and genetic counseling in CCM patients. Moreover, we confirmed the high risk of hemorrhage in children with CCMs.

  9. Middle Ear Implantable Hearing Devices: An Overview

    PubMed Central

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

    2009-01-01

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

  10. 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…

  11. 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…

  12. Keep Your Ear-Lids Open.

    ERIC Educational Resources Information Center

    Ferrington, Gary

    1994-01-01

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

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

    PubMed

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

    2008-06-01

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

  14. 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

  15. Optimum management of the discharging ear.

    PubMed

    Ruddy, J; Bickerton, R C

    1992-02-01

    Discharge from the ear can be the result of many disease processes. The ear may discharge blood, pus, cerebrospinal fluid (CSF) or wax. Keratosis obturans, stenosis of the external meatus and benign tumours of the external meatus all lead to wax build-up, which may cause recurrent attacks of otitis externa. Malignant tumours, such as basal cell carcinoma, squamous cell carcinoma and tumours of ceruminous gland origin may also present with discharge. Tumours should be excluded by submitting all material removed from the external canal for histological examination. Single or multiple abscesses (known as furuncles) may occur in the hair follicles in the skin of the external acoustic meatus (EAM). Compulsive scratching, hearing aids and foreign bodies placed in the ear predispose to otitis externa, which is also often associated with infection by Pseudomonas aeruginosa, Staphylococcus aureus and faecal organisms. Management may be with aluminium acetate 14%, topical antibiotic/steroid drops, a gauze wick soaked with icthammol 10% in glycerin or polymyxin B sulphate--neomycin sulphate--hydrocortisone acetate cream placed into the EAM and replaced every 24 to 48 hours, or systemic antibiotics according to severity. Malignant (necrotising) otitis externa causes progressive destruction of the temporal bone, and cranial nerve palsies (usually facial first). Treatment is limited debridement of infected bone, accompanied by intravenous aminoglycosides, and local antibiotic treatment and aural cleanout or oral ciprofloxacin. Middle ear conditions causing discharge include acute otitis media, infected grommets, traumatic perforations and chronic suppurative otitis media, as well as tumours of the ear canal skin and middle ear, radiation-induced otitis externa and osteoradionecrosis of the temporal bone, tuberculosis, Langerhans cell histiocytosis, spontaneous or post-traumatic CSF leaks, Wegeners granulomatosis and immune deficiency states. Topical application of aminoglycoside

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

    PubMed

    Anthwal, Neal; Thompson, Hannah

    2016-02-01

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

  17. 21 CFR 874.3430 - Middle ear mold.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 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. A middle ear mold is a preformed device that is intended to be implanted to reconstruct the middle...

  18. 21 CFR 874.3430 - Middle ear mold.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 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. A middle ear mold is a preformed device that is intended to be implanted to reconstruct the middle...

  19. 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...

  20. 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...

  1. 15 CFR 734.2 - Important EAR terms and principles.

    Code of Federal Regulations, 2012 CFR

    2012-01-01

    ... 15 Commerce and Foreign Trade 2 2012-01-01 2012-01-01 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...

  2. 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...

  3. 15 CFR 734.2 - Important EAR terms and principles.

    Code of Federal Regulations, 2013 CFR

    2013-01-01

    ... 15 Commerce and Foreign Trade 2 2013-01-01 2013-01-01 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...

  4. 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...

  5. Ear tube surgery - what to ask your doctor

    MedlinePlus

    What to ask your doctor about ear tube surgery; Tympanostomy - what to ask your doctor; Myringotomy - what to ask your doctor ... Why does my child need ear tubes? Can we try other treatments? ... the surgery? Is it safe to wait before getting ear tubes? Will ...

  6. Role of skeletal muscle in ear development.

    PubMed

    Rot, Irena; Baguma-Nibasheka, Mark; Costain, Willard J; Hong, Paul; Tafra, Robert; Mardesic-Brakus, Snjezana; Mrduljas-Djujic, Natasa; Saraga-Babic, Mirna; Kablar, Boris

    2017-03-08

    The current paper is a continuation of our work described in Rot and Kablar, 2010. Here, we show lists of 10 up- and 87 down-regulated genes obtained by a cDNA microarray analysis that compared developing Myf5-/-:Myod-/- (and Mrf4-/-) petrous part of the temporal bone, containing middle and inner ear, to the control, at embryonic day 18.5. Myf5-/-:Myod-/- fetuses entirely lack skeletal myoblasts and muscles. They are unable to move their head, which interferes with the perception of angular acceleration. Previously, we showed that the inner ear areas most affected in Myf5-/-:Myod-/- fetuses were the vestibular cristae ampullaris, sensitive to angular acceleration. Our finding that the type I hair cells were absent in the mutants' cristae was further used here to identify a profile of genes specific to the lacking cell type. Microarrays followed by a detailed consultation of web-accessible mouse databases allowed us to identify 6 candidate genes with a possible role in the development of the inner ear sensory organs: Actc1, Pgam2, Ldb3, Eno3, Hspb7 and Smpx. Additionally, we searched for human homologues of the candidate genes since a number of syndromes in humans have associated inner ear abnormalities. Mutations in one of our candidate genes, Smpx, have been reported as the cause of X-linked deafness in humans. Our current study suggests an epigenetic role that mechanical, and potentially other, stimuli originating from muscle, play in organogenesis, and offers an approach to finding novel genes responsible for altered inner ear phenotypes.

  7. Standardized measurements of the sound transmission of middle ear implants using a mechanical middle ear model.

    PubMed

    Meister, H; Walger, M; Mickenhagen, A; von Wedel, H; Stennert, E

    1999-01-01

    Several ways to evaluate the sound transmission properties of middle ear implants are now established. Besides computer-based simulations using acoustic and electrical analog circuits or finite element analysis, measurements can be performed with temporal bone preparations. Experiments with these preparations consider various anatomical properties, but a large number of parameters influence the outcome of measurements. To facilitate standardized measurements, a mechanical middle ear model was developed that allows comparison of the transfer function of middle ear implants on defined conditions. The model approximates the impedances of the tympanic membrane and inner ear with the aid of thin, flexible membranes. The implants are fit between the membranes, and displacement at an artificial stapes foot-plate is measured with an optical probe. Fundamental influences on the sound transmission properties of nine different middle ear implants (total ossicular replacement prostheses) were examined. Although the material and shape were different, some of the prostheses revealed very similar transfer functions. The mass of the implant showed the largest influence on sound conduction. With a higher mass, the frequency area above approximately 1 kHz was found to be significantly deteriorated. The lightest implant used was 4 mg and showed the best overall results. These findings show that middle ear prostheses should be as light as possible for optimum high-frequency transmission.

  8. Anthelmintic induced congenital malformations in sheep embryos using netobimin.

    PubMed

    Navarro, M; Cristofol, C; Carretero, A; Arboix, M; Ruberte, J

    1998-01-24

    Benzimidazole compounds have teratogenic effects in domestic and experimental animals. In this study, 14 Manchega ewes were treated orally, under controlled conditions, with 20 mg netobimin (a prodrug of a benzimidazole compound) per/kg bodyweight on the 17th day of pregnancy. Congenital malformations and abortions affected 60 per cent of the lambs. The main malformations were skeletal and renal, but vascular malformations were observed for the first time. The abnormalities were investigated using radiological, dissection and vascular injection techniques, and associations among them were recorded. The anomalies are discussed in terms of embryological considerations.

  9. Congenital malformations of the spinal cord without early symptoms.

    PubMed

    Moffie, D; Stefanko, S Z; Makkink, B

    1986-01-01

    Description of 11 patients with congenital malformations of the spinal cord. Six of them were males, five females and the age varied from 7 to 70 years. Most of these cases produced clinical neurological signs indicating spinal cord disease in later life during an intercurrent disease. It was thought that changes in the bloodvessels and/or perfusion of the area of the spinal cord malformation was the ultimate cause of the neurological symptoms. An exact explanation of the origin of these developmental disturbances of the spinal cord remains unknown. Different hypotheses proposed in the literature, concerning these malformations, are not satisfactory.

  10. [Arteriovenous malformation-glioma association: study of four cases].

    PubMed

    Borges, Lia Raquel R; Malheiros, Suzana M F; Pelaez, Maria Paula; Stávale, João Norberto; Santos, Adrialdo J; Carrete, Henrique; Nogueira, Roberto Gomes; Ferraz, Fernando A P; Gabbai, Alberto A

    2003-06-01

    We reviewed the clinical presentation, imaging and histopathologic findings in 4 patients with the diagnosis of arteriovenous malformation associated with glioma that were operated on from 1991 to 2000 in our institution. Four patients (2 males; age between 15 and 52 years) presented with progressive headache with clinical evidence of intracranial hypertension (in 3) and partial seizures (in 1). CT scan showed a brain tumor without any detectable pathologic vessels. Histologic examination revealed astrocytic tumors associated with arteriovenous malformation. No patient presented the vascular component intermixed with the tumor. The arteriovenous-glioma association is rare and must be identified by a clear demarcation between the malformation and the tumor.

  11. CHD7 deficiency in "Looper", a new mouse model of CHARGE syndrome, results in ossicle malformation, otosclerosis and hearing impairment.

    PubMed

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

    2014-01-01

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

  12. Ear candles: a triumph of ignorance over science.

    PubMed

    Ernst, E

    2004-01-01

    Ear candles are hollow tubes coated in wax which are inserted into patients' ears and then lit at the far end. The procedure is used as a complementary therapy for a wide range of conditions. A critical assessment of the evidence shows that its mode of action is implausible and demonstrably wrong. There are no data to suggest that it is effective for any condition. Furthermore, ear candles have been associated with ear injuries. The inescapable conclusion is that ear candles do more harm than good. Their use should be discouraged.

  13. Identification of a second HOXA2 nonsense mutation in a family with autosomal dominant non-syndromic microtia and distinctive ear morphology.

    PubMed

    Piceci, F; Morlino, S; Castori, M; Buffone, E; De Luca, A; Grammatico, P; Guida, V

    2016-08-09

    Microtia is a congenital defect affecting external ears, which appear smaller and sometimes malformed. Here we describe a five-generation family with isolated bilateral microtia segregating as an autosomal dominant trait. Similar features have been previously observed in an autosomal dominant family with non-syndromic microtia and hearing loss segregating with a HOXA2 nonsense variant. HOXA2 biallelic mutations were also described in an inbreed family with autosomal recessive microtia, hearing impairment and incomplete cleft palate. In our family, sequence analysis detected a heterozygous protein truncating nonsense variant [c.670G>T, p.(Glu224*)] segregating in all affected individuals and absent in public databases. This study confirms the role of HOXA2 gene in dominant isolated microtia and contribute to further define the dysmorphogenetic effect of this gene on ear development.

  14. Type I Chiari malformation presenting central sleep apnea.

    PubMed

    Kitamura, Takuro; Miyazaki, Soichiro; Kadotani, Hiroshi; Kanemura, Takashi; Okawa, Masako; Tanaka, Toshihiko; Komada, Ichiro; Hatano, Taketo; Suzuki, Hideaki

    2014-04-01

    Sleep apnea is a rare but a well-known clinical feature of type I Chiari malformation. It may be obstructive or central in nature. Sleep apnea in patients with type I Chiari malformation rarely presents without accompanying neurological signs or symptoms. We here report a case of a 10-year-old girl who presented with central sleep apnea without any other neurological signs but was ultimately diagnosed with type I Chiari malformation. The patient initially showed mild improvement in symptoms after administration of an acetazolamide. Finally, posterior fossa decompression dramatically improved her respiratory status during sleep, both clinically and on polysomnography. This case suggests that type I Chiari malformation should be considered in the differential diagnoses of central apneas in children, even if there are no other neurological signs and symptoms. Furthermore, sagittal craniocervical magnetic resonance imaging may be necessary for a definitive diagnosis.

  15. Dural arteriovenous malformation: a rare cause of epilepsy in childhood.

    PubMed

    Caksen, H; Unal, O; Tombul, T; Cesur, Y; Abuhandan, M

    2001-09-01

    A 3 year and 6 month old girl with epilepsy associated with dural arteriovenous malformation (DAVM), diagnosed on the MRI, is presented to emphasise the importance of DAVM in the aetiology of childhood epilepsy.

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

    PubMed

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

    2011-04-01

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

  17. Glioma coexisting with angiographically occult cerebrovascular malformation: A case report

    PubMed Central

    Chen, Junhui; Chen, Lei; Zhang, Chunlei; He, Jianqing; Li, Peipei; Zhou, Jingxu; Zhu, Jun; Wang, Yuhai

    2016-01-01

    Angiographically occult cerebrovascular malformation (AOVM) is a type of complex cerebrovascular malformation that is not visible on digital subtraction angiography (DSA). Vascular malformation coexisting with glioma is clinically rare, and glioma coexisting with AOVM is even more rare. To the best of our knowledge, the present study is the first to report glioma coexisting with AOVM in the literature. The present study reports a rare case of glioma coexisting with AOVM in a 30-year-old male patient. Computed tomography (CT) scan revealed calcification, hemorrhage and edema in the right frontal lobe. CT angiography revealed a vascular malformation in the right frontal lobe, which was not observed on DSA. Finally, glioma coexisting with AOVM was confirmed by 2.0T magnetic resonance imaging and postoperative pathological examination. The present patient had a positive outcome and no neurological dysfunctions during the 6-month follow-up subsequent to surgery. PMID:27698825

  18. Chiari-I malformation in two fighter pilots.

    PubMed

    Akin, Ahmet; Canakci, Zafer; Sen, Ahmet; Tore, Hasan F

    2003-07-01

    This report describes two cases of Chiari Malformation Type I (Chiari-I) in fighter pilots of the Turkish Air Force. Chiari-I is a congenital malformation characterized by herniation of cerebellar tonsils through the foramen magnum. Patients have symptoms and signs related to dysfunction of the brainstem, spinal cord, and cerebellum. They generally are symptomatic in the earlier years of life. However, asymptomatic cases can eventually become symptomatic in later years. Symptoms can be provoked by increasing intracranial pressure (Valsalva or straining). We report on two pilots with Chiari-I malformation who had no symptoms or signs in their daily activities. Furthermore, these pilots had successfully completed physiological training, including centrifuge training, without any symptoms. However, they suffered from headache, neck spasms, and/or disequilibrium under +Gz during flight training sorties. The clinical presentation, diagnosis, treatment, possibility of acquired cases, and aeromedical disposition of Chiari malformations are discussed.

  19. Optic chiasmal cavernous angioma: A rare suprasellar vascular malformation

    PubMed Central

    Abou-Al-Shaar, Hussam; Bahatheq, Ayman; Takroni, Radwan; Al-Thubaiti, Ibrahim

    2016-01-01

    Background: Suprasellar cavernous malformation in the optic pathway is not commonly encountered. To date, there are only few reports present in the literature. Case Description: The authors report a rare case of suprasellar optic pathway cavernous malformation in a 33-year-old female who presented with progressive visual loss. Her imaging revealed a large heterogeneous, hyperintense, hemorrhagic right suprasellar extra-axial complex cystic structure, causing mass effect on the adjacent hypothalamus and third ventricle displacing these structures. Gross total resection of the lesion was achieved utilizing a right frontal craniotomy approach. Histopathological examination confirmed the diagnosis of suprasellar chiasmal cavernous malformation. Conclusion: Although visual pathway cavernous malformation is a rare event, it should be included in the differential diagnosis of lesions occurring suprasellarly in the visual pathway and hypothalamus. PMID:27583178

  20. Prolonged expiratory apnoea with cyanosis in Arnold Chiari II malformation

    PubMed Central

    Hussain, Nahin

    2017-01-01

    Apnoea associated with Arnold Chiari malformation is a known entity and can be obstructive or central. Differentiating between two types is vital to deciding management pathway and prognosticating disease process. PMID:28321315

  1. 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

  2. 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.

  3. Glomuvenous Malformation: A Rare Periorbital Lesion of the Thermoregulatory Apparatus

    PubMed Central

    Whipple, Katherine M.; Godfrey, Kyle J; Solomon, James P.; Lin, Jonathan H.; Korn, Bobby S.; Kikkawa, Don O.

    2016-01-01

    Glomuvenous malformations (GVMs), previously referred to as glomus tumors or glomangiomas, are benign, mesenchymal venous malformations arising from glomus bodies. Glomus bodies are modified smooth muscle neuromyoarterial structures involved in temperature regulation via blood shunting. These classically occur in the digits but can occur in other locations. The authors present a case of a periorbital GVM presented following blunt trauma to the area. PMID:27065433

  4. 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.

  5. Supernumerary nipples and renal malformations: a family study.

    PubMed

    Brown, Justin; Schwartz, Robert A

    2004-01-01

    We describe a family with supernumerary nipples and kidney and urinary tract malformations. The proband is a 59-year-old man with a supernumerary nipple and recently identified hypoplastic kidney, bladder diverticulum, and hypotonic bladder. His mother also has a supernumerary nipple and a recently identified hypoplastic kidney. Of his three children, all male, one has a supernumerary nipple but has not been evaluated for a kidney or urinary tract malformation. This familial disorder is discussed.

  6. Diffuse pulmonary arteriovenous malformation in a child with polysplenia syndrome.

    PubMed

    Kapur, S; Rome, J; Chandra, R S

    1995-01-01

    Pulmonary arteriovenous malformation (PAVM) is usually seen as a well-circumscribed cystic mass. In this communication we describe a diffuse arteriovenous malformation in a 5-month-old infant. The lesion was seen only at the microscopic level and was associated with abdominal heterotaxy, atrial situs solitus, polysplenia, interrupted inferior vena cava, atrial septal defect, and atrioventricular canal. This uncommon association may be within the spectrum of polysplenia syndrome.

  7. Split cord malformation type I distal to segmental myelomeningocele

    PubMed Central

    Addas, Bassam M.

    2014-01-01

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

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

    PubMed Central

    Lowe, C. R.

    1972-01-01

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

  9. Diseases of the middle ear in childhood

    PubMed Central

    Minovi, Amir; Dazert, Stefan

    2014-01-01

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

  10. 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

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

    PubMed Central

    Slavotinek, A; Tifft, C

    2002-01-01

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

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

    PubMed

    Slavotinek, A M; Tifft, C J

    2002-09-01

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

  13. Scaling of the mammalian middle ear.

    PubMed

    Nummela, S

    1995-05-01

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

  14. Reconstruction of the burned nose and ear.

    PubMed

    Bernard, S L

    2000-01-01

    Patients who have survived thermal injuries to the face suffer from severe disfigurement. When the nose and ear are involved, the resulting deformities are immediately obvious to all who see the patient. This level of injury results in a self-imposed confinement; the patients never leave their homes. It is therefore important that we plastic surgeons know, understand, and use all options available to improve our patients' appearance and ultimately their mental and physical well being.

  15. Ear Infections - Multiple Languages: MedlinePlus

    MedlinePlus

    ... gov/languages/earinfections.html Other topics A-Z A B C D E F G H I J K L M N O P Q R S T U V W XYZ List of All Topics All Ear Infections - Multiple Languages To use the sharing features on this page, please enable JavaScript. French (français) Japanese (日本語) Korean (한국어) Russian (Русский) Somali ( ...

  16. Local anesthesia for middle ear surgery.

    PubMed

    Caner, Gül; Olgun, Levent; Gültekin, Gürol; Aydar, Levent

    2005-08-01

    The adequacy of anesthesia and comfort during surgery was assessed for 100 consecutive patients undergoing middle ear surgery using local anesthesia, both by the patients themselves and by the surgeon. The possibility of inducing an iatrogenic facial weakness was also evaluated. Both the surgeon and the majority of patients were pleased with the quality of anesthesia and little adverse effects occurred as a consequence of local anesthesia itself.

  17. Prevention and avoidance of congenital malformations.

    PubMed

    Nevin, N C

    1988-06-15

    Many congenital abnormalities do not have either a Mendelian pattern of inheritance or an identifiable chromosome abnormality and are described as 'multifactorial' as it is assumed they are determined by several genes, each with added effects and modified to a greater or lesser extent by environmental factors. They include spina bifida and anencephaly, cleft lip or cleft palate or both, congenital heart defect and congenital dislocation of the hip, and they constitute a major community health problem. Developments in genetics, biochemistry and cytogenetics have presented new approaches to the prevention and avoidance of congenital abnormalities. The approaches available for the avoidance of congenital malformations include the avoidance of harmful environmental factors, the screening of the newborn and early treatment, genetic counselling and antenatal monitoring with selective termination. The prevention of neural-tube defects in 'high risk' mothers can be achieved by periconceptional vitamin supplementation. In Northern Ireland, of 438 fully supplemented women, only 4 (0.98%) infants or fetuses among 407 infants and fetuses examined had a neural-tube defect, whereas of 356 unsupplemented women, 16 (4.7%) infants or fetuses among 337 infants or fetuses examined had a neural-tube defect.

  18. Cerebral Arteriovenous Malformation Associated with Moyamoya Disease

    PubMed Central

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

    2014-01-01

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

  19. Cerebral arteriovenous malformation associated with moyamoya disease.

    PubMed

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

    2014-10-01

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

  20. [Intracranial arteriovenous malformations in pregnant women].

    PubMed

    Perquin, D A; Kloet, A; Tans, J T; Witte, G N; Dörr, P J

    1999-03-06

    Three women, aged 27, 32 and 30 years, respectively, suffered from headache, nausea and neurological abnormalities and were found to have an intracranial arteriovenous malformation (AVM). One of them after diagnosis had two pregnancies, both ended by caesarean section with good results. Another woman was 32 weeks pregnant when the AVM manifested itself with a haemorrhage; she recovered well and was delivered by caesarean section. After the AVM proved radiologically to have been obliterated, she delivered after her subsequent pregnancy by the vaginal route with vacuum extraction. The third woman was 15 weeks pregnant when major abnormalities developed. There was a large intracerebral haematoma with break-through to the ventricular system; this patient died. Intracranial haemorrhage during pregnancy is rate. It can result in maternal and foetal morbidity and mortality. It appears that pregnancy does not increase the rate of first cerebral haemorrhage from an AVM. The management of AVM rupture during pregnancy should be based primarily on neurosurgical rather than on obstetric considerations. Close collaboration with a team of neurologists, neurosurgeons, obstetricians and anaesthesiologists is mandatory.

  1. 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

  2. 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.

  3. Genetic and Developmental Basis of Cardiovascular Malformations

    PubMed Central

    Azhar, Mohamad; Ware, Stephanie M.

    2015-01-01

    Cardiovascular malformations (CVMs) are the most common birth defect, occurring in 1–5% of all live births. Genetic, epigenetic, and environmental factors all influence the development of CVMs, and an improved understanding of causation of CVMs is a prerequisite for prevention. Cardiac development is a complex, multi-step process of morphogenesis that is under genetic regulation. Multiple developmental pathways act independently or in combination to effect proper cardiac lineage specification, differentiation, and structure. Because of this complexity, there are numerous potential mechanisms by which genetic variation can impact both fetal cardiac development and latent cardiac disease. Although the genetic contribution to CVMs is well recognized, the genetic causes of human CVMs are still identified relatively infrequently. Mouse models are important tools to investigate the molecular mechanisms underpinning cardiac development as well as the complex genetics that characterize human CVMs. In this review we provide an overview of the key genetic concepts characterizing human CVMs, review their developmental basis, and provide examples to illustrate the critical developmental and genetic concepts underlying the pathogenesis of CVMs. PMID:26876120

  4. Carotid ultrasound for pulmonary arteriovenous malformation screening

    PubMed Central

    Schneider, Günther; Maßmann, Alexander; Gräber, Stefan; Geisthoff, Urban W.

    2015-01-01

    Objective In patients with hereditary hemorrhagic telangiectasia (HHT), pulmonary arteriovenous malformations (PAVMs) can cause serious neurological complications. Our aim was to evaluate the potential of contrast-enhanced Doppler ultrasound (CE-US) of the common carotid artery as a screening test for detection of PAVMs. Methods A total of 124 consecutive patients with HHT or a positive family history underwent screening for PAVMs with CE-US and thoracic contrast-enhanced magnetic resonance angiography (CE-MRA). CE-US was performed after receiving (D)-galactose microparticulate, and CE-MRA with gadobenate dimeglumine. Twenty-five patients with confirmed PAVMs were referred to conventional pulmonary catheter angiography (PA). Findings on CE-US and CE-MRA were evaluated using contingency tables and McNemar’s test. Results Using CE-MRA as the reference test, CE-US had a sensitivity of 100%, a specificity of 87%, and a negative predictive value of 100%. In 25 patients who underwent PA, PAVMs that had been diagnosed on CE-US and CE-MRA were confirmed. Of the PAVMs detected by CE-MRA, 24% were not identified on PA. Conclusion CE-US is a simple, minimally invasive screening method that can easily be performed in different settings. CE-US can predict PAVMs with high probability of success. CE-US may be a simple alternative to transthoracic echocardiography in the assessment of PAVMs in certain HHT-patients. PMID:28352707

  5. Psychosocial adjustment and craniofacial malformations in childhood.

    PubMed

    Pertschuk, M J; Whitaker, L A

    1985-02-01

    Forty-three children between the ages of 6 and 13 years with congenital facial anomalies underwent psychosocial evaluation prior to surgery. Also evaluated were healthy children matched to the craniofacial subjects by sex, age, intelligence, and economic background. Relative to this comparison group, the craniofacial children were found to have poorer self-concept, greater anxiety at the time of evaluation, and more introversion. Parents of the craniofacial children noted more frequent negative social encounters for their children and more hyperactive behavior at home. Teachers reported more problematic classroom behavior. Examination of these results revealed craniofacial malformations to be associated with psychosocial limitations rather than marked deficits. These children tended to function less well than the comparison children, but with few exceptions, they were not functioning in a psychosocially deviant range. Explanations for the observed circumscribed impact of facial deformity include the use of denial as a coping mechanism, possible diminished significance of appearance for younger children, and the restricted environment experienced by most of the subjects. It can be predicted that time would render these protective influences ineffective, so that adolescent and young adult patients could be at far greater psychosocial risk.

  6. 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.

  7. Vascular permeability in cerebral cavernous malformations

    PubMed Central

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

    2015-01-01

    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

  8. 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.

  9. 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.

  10. Facio-auriculo-vertebral sequence in association with DiGeorge sequence, Rokitansky sequence, and Dandy-Walker malformation: case report.

    PubMed

    Pillay, Komala; Matthews, Louise S; Wainwright, Helen C

    2003-01-01

    Extreme variability of expression is characteristic of the facio-auriculo-vertebral sequence. Sporadic and familial cases have been reported with obvious etiologic heterogeneity. Most reports in the literature are of clinical cases. The purpose of this paper is to present a fetal autopsy case report of the facio-auriculo-vertebral sequence in association with DiGeorge sequence, Rokitansky sequence, and Dandy-Walker malformation. A standard neonatal autopsy was performed on a macerated female fetus, gestational age 29 wk. External examination of the fetus revealed hypoplastic right face, low-set microtic right ear, and macrostomia. Internal examination showed hypoplastic thymus and lungs, a type I truncus arteriosus, and ventricular septal defect. Both kidneys showed evidence of pelvi-ureteric junction obstruction. The ovaries and fallopian tubes were present with an absent uterus and vagina (Rokitansky sequence). In addition, Dandy-Walker malformation was identified. Microscopically, a single hypoplastic parathyroid gland was noted and there was cystic renal dysplasia. We report the sixth case of the facio-auriculo-vertebral sequence in association with Rokitansky sequence and the first case of this sequence in association with Dandy-Walker malformation. In addition, features of DiGeorge sequence were present.

  11. Recurrent Streptococcus Pneumoniae Meningitis in a Child with Split Hand and Foot Malformation and Undiagnosed Mondini Dysplasia.

    PubMed

    Katarzyna, Mazur-Melewska; Jarosław, Szydłowski; Katarzyna, Jończyk-Potoczna; Wojciech, Służewski; Magdalena, Figlerowicz

    Recurrent bacterial meningitis is a life-threatening infection of the central nervous system that is mostly connected with anatomical abnormalities of the skull, chronic parameningeal infections and immunodeficiencies. It's rarely seen, but when it occurs an extensive investigation should be carried out to discover the responsible factor, so that further episodes can be prevented. We report on a child with split hand and foot (SHFM), confirmed incorrect karyotype 46, XY, t(7:12)(q21.2;q21.3) and a fourth episode of fulminant meningitis caused by penicillin-resistant Streptococcus pneumoniae. After a broad evaluation of factors predisposing to recurrent meningitis, the undiagnosed malformation of his inner and middle ears - Mondini dysplasia was found. We suggest examining all children with SHFM for hearing impairment before they develop recurrent meningitis. The time when the radiological procedure for searching for inner ear dysplasia should be performed could be a controversial issue: before or after the first episode of meningitis. From the epidemiological point of view, high-resolution computer tomography scanning of the temporal bones should be considered after the diagnosis of deafness in a child with SHFM related to 7q21 deletion.

  12. Detecting congenital malformations - Lessons learned from the Mpepu study, Botswana

    PubMed Central

    Zash, Rebecca; Shapiro, Roger L.; Batlang, Oganne; Botebele, Kerapetse; Bennett, Kara; Chilisa, Florence; von Widenfelt, Erik; Makhema, Joseph; Lockman, Shahin; Holmes, Lewis B.; Powis, Kathleen M

    2017-01-01

    Introduction A large and increasing number of HIV-infected women are conceiving on antiretroviral treatment (ART). While most antiretrovirals are considered safe in pregnancy, monitoring for rare pregnancy and infant adverse outcomes is warranted. Methods We conducted a retrospective secondary analysis nested within a clinical trial of infant cotrimoxazole vs. placebo prophylaxis in Botswana (the Mpepu Study). Infants were examined at birth, and at least every 3 months through 18 months of age. Abnormal physical findings and diagnostic testing revealing malformations were documented. Post hoc, a geneticist classified all reported malformations based on available documentation. Structural malformations with surgical, medical or cosmetic importance were classified as major malformations. We present a descriptive analysis of identified malformations. Results Between 2011 and 2014, 2,933 HIV-infected women who enrolled in the Mpepu study delivered 2,971 live-born infants. Study staff conducted 2,944 (99%) newborn exams. One thousand eighty-eight (38%) women were taking ART at conception; 1,147 (40%) started ART during pregnancy; 442 (15%) received zidovudine monotherapy; and 223 (7%) received no antiretroviral during pregnancy. Of 33 reported anomalies, 25 (76%) met congenital malformations criteria, 10 (30%) were classified as major malformations, 4 (40%) of which were identified after the birth exam. Discussion Our results highlight the importance of staff training on identification of congenital malformations, programmatic monitoring beyond the birth examination and the value of geneticist involvement in the malformations classification process in resource-limited settings. These elements will be important to fully define antiretroviral drug safety in pregnancy. Significance Surveillance systems for monitoring the safety of antiretroviral use during pregnancy among HIV-infected women in resource-limited setting are lacking. The World Health Organization

  13. 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

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

    PubMed Central

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

    2014-01-01

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

  15. Repeat radiosurgery for cerebral arteriovenous malformations

    PubMed Central

    Stapleton, Christopher J.; Ding, Dale; Leed, Cheng-Chia; Loeffler, Jay S.

    2015-01-01

    We perform a systematic review of repeated 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, repeated radiosurgery can be considered if sufficient time has passed to allow for a full appreciation of treatment effects, usually at least 3 years. A systematic review was performed in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses 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 19.42 Gy and 19.06 Gy, 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 80 months. Time to complete obliteration after the repeat treatment ranged from 21 to 40.8 months. The most common complications of repeated 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 three years following the initial treatment, allowing for full realization of effects from the initial treatment prior to commencing therapy. PMID:25913746

  16. Immune Response in Human Cerebral Cavernous Malformations

    PubMed Central

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

    2009-01-01

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

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

    PubMed

    Ravicz, Michael E; Rosowski, John J

    2012-10-01

    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 Y(TM,) the ME input admittance at the TM, from the admittance measured relatively far from the TM. Y(TM) appears valid at frequencies as high as 17 kHz, a much higher frequency than previously reported. The real part of Y(TM) 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.

  18. Electrical ear acupuncture reduces histamine-induced itch (alloknesis).

    PubMed

    Kesting, Marco Rainer; Thurmüller, Petra; Hölzle, Frank; Wolff, Klaus-Dietrich; Holland-Letz, Tim; Stücker, Markus

    2006-01-01

    In order to assess an objective measure for the outcome of ear acupuncture, we evaluated the effect of electrical ear acupuncture on areas of histamine-induced alloknesis in 32 healthy volunteers. In a first assessment 5 min after histamine application on both volar forearms, 16 subjects received right ear and 16 left ear acupuncture. Immediately before and 5 min after acupuncture, alloknesis areas on both forearms were planimetrically evaluated. A second assessment was carried out 4 weeks later with the same patients. They underwent histamine application once more, but received no acupuncture. Alloknesis areas were then compared with reference to time, assessment and therapy side. Forearms relating to ipsilateral acupuncture showed significantly reduced or even no alloknesis areas after therapy. On the contralateral sides and during the "non-acupuncture" assessment 4 weeks later, alloknesis areas were significantly enlarged compared with sides ipsilateral to right and left ear acupuncture. Hence, results verify the effects of electrical ear acupuncture by objective measures.

  19. Review of congenital inner ear abnormalities on CT temporal bone.

    PubMed

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

    2011-09-01

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

  20. 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.

  1. Sporadic Multifocal Venous Malformations of the Head and Neck

    PubMed Central

    Amato, Michael V.; Patel, Neha A.; Hu, Shirley; Pantelides, Harry

    2015-01-01

    Objective. To report a case of unusually widespread sporadic venous malformations of the head and neck associated with normal D-dimer levels and, due to the protean clinical manifestations and increased risk of coagulopathy of these lesions, to review their diagnosis and clinical management. Case Report. A 25-year-old man presented with a one-year history of intermittent right-sided neck swelling and tongue swelling. Physical exam revealed additional lesions present throughout the head and neck. There was no family history suggestive of heritable vascular malformations. Radiographic imaging demonstrated 15 lesions located in various tissue layers consistent with venous malformations. A coagulation screen showed a normal prothrombin time, activated partial thromboplastin time, international normalized ratio, D-dimer level, and fibrinogen level. It was determined that the patient was not at increased risk for intraoperative coagulopathy and preoperative heparin administration would not be necessary. The patient's buccal and tongue lesions were subsequently excised with no complications. The patient also underwent sclerotherapy evaluation for his neck mass. Conclusion. This case describes a unique presentation of sporadic multifocal venous malformations. It also emphasizes the importance of prompt diagnosis and workup when multiple venous malformations are present to prevent morbidity during surgical excision secondary to intravascular coagulopathy. PMID:26483982

  2. Neurodevelopmental Malformations of the Cerebellar Vermis in Genetically Engineered Rats.

    PubMed

    Ramos, Raddy L; Van Dine, Sarah E; Gilbert, Mary E; Leheste, Joerg R; Torres, German

    2015-12-01

    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. Malformations are almost exclusively found along the primary fissure and are indicative of deficits of neuronal migration during cerebellar development. In the present report, we test the prediction that genetically engineered rats on Sprague-Dawley or Long-Evans backgrounds will also exhibit the same cerebellar malformations. Consistent with our hypothesis, we found that three different transgenic lines on two different backgrounds had cerebellar malformations. Heterotopia in transgenic rats had identical cytoarchitecture as that observed in wild-type rats including altered morphology of Bergmann glia. In light of the possibility that heterotopia could affect results from behavioral studies, these data suggest that histological analyses be performed in studies of cerebellar function or development when using genetically engineered rats on these backgrounds in order to have more careful interpretation of experimental findings.

  3. 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.

  4. Pathobiology of human cerebrovascular malformations: basic mechanisms and clinical relevance.

    PubMed

    Gault, Judith; Sarin, Hemant; Awadallah, Nabil A; Shenkar, Robert; Awad, Issam A

    2004-07-01

    Cerebrovascular malformations affect more than 3% of the population, exposing them to a lifetime risk of hemorrhagic stroke, seizures, and focal neurological deficits. Cerebral cavernous malformations (CCMs) exhibit an immature vessel wall, a brittle hemorrhagic tendency, and epileptogenesis, whereas arteriovenous malformations (AVMs) lack capillary beds and manifest apoplectic bleeding under high-flow conditions. There are also more benign venous anomalies, capillary malformations, and lesions with mixed and transitional features. Advances have been made toward understanding the natural history, radiological and pathological correlates, and clinical management. Yet, mechanisms of lesion genesis and clinical manifestations remain largely unknown, and the clinical behavior in individual patients is highly unpredictable. Lesion pathogenesis likely involves abnormal assembly or maintenance of blood vessels, resulting in dysmorphic vessel phenotypes. Familial CCM disease is in part caused by mutations in a cytoskeletal-related protein that is likely integral to interendothelial cell connectivity and maturation of the vascular wall. Rare familial forms of AVM disease have been correlated with two different transforming growth factor-beta receptor components, possibly causing disturbance in signaling during vascular assembly. Relevance of these mechanisms to the more common and otherwise identical sporadic CCM and AVM lesions is being explored. In this report, basic mechanisms of vasculogenesis and angiogenesis and how they possibly relate to the common cerebrovascular malformation lesions are reviewed. Novel concepts are discussed related to the cellular, molecular, and genetic substrates in CCM and AVM as well as to how this knowledge can be applied to predict, explain, and possibly modify clinical disease manifestations.

  5. 3D ear identification based on sparse representation.

    PubMed

    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.

  6. Neuroendocrine Adenoma of the Middle Ear: A Rare Histopathological Diagnosis

    PubMed Central

    McGinness, Sam; Coleman, Hedley; Varikatt, Winny; da Cruz, Melville

    2016-01-01

    Neuroendocrine tumours occur throughout the body but are rare in the head and neck region and particularly rare in the middle ear. Clinical findings are often nonspecific and therefore pose a diagnostic challenge. Furthermore, the nomenclature of neuroendocrine tumours of the middle ear is historically controversial. Herein a case is presented of a middle ear adenoma in a 33-year-old patient who presented with otalgia, hearing loss, and facial nerve palsy. A brief discussion is included regarding the histopathological features of middle ear adenomas and seeks to clarify the correct nomenclature for these tumours. PMID:27429819

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

    NASA Astrophysics Data System (ADS)

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

    2012-06-01

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

  8. Management of patients with brain arteriovenous malformations.

    PubMed

    Söderman, Michael; Andersson, Tommy; Karlsson, Bengt; Wallace, M Christopher; Edner, Göran

    2003-06-01

    Arteriovenous malformations (AVMs) of the brain, which are probably genetically determined, are errors in the development of the vasculature that, together with the effects of blood flow, may lead to a focal arteriovenous shunt. Clinically, the adult patient may present with acute or chronic neurological symptoms-fixed or unstable-such as deficits, seizures or headache. Sometimes the lesion is an incidental finding. In about half of the patients, the revealing event is an intracranial haemorrhage. The prevalence of AVM in the western world is probably <0.01% and the detection rate is about one per 100,000 person-years. Most AVMs are revealed in patients 20-40 years of age. Therefore, the risk of developing neurological symptoms from an AVM, usually because of haemorrhage, increases with patient age. In the young adult population, AVMs are significant risk factors for hemorrhagic stroke. This risk increases with AVM volume and is higher in centrally located AVMs. Almost all patients with AVM are subjected to treatment, either by surgery, radiosurgery or embolisation, with the functional aim of reducing the risk of haemorrhage or to alleviate neurological symptoms with an acceptable treatment risk. Few neurocentres have physicians highly skilled in all treatment modalities. Therefore, the prescribed treatment may not be defined from an objective assessment of what is optimal for each individual patient, but rather from local expertise. In this context, more and better data about the natural history and the outcome of different treatments, as well as predictive models, would be valuable to help to optimise the management. Management strategies obviously differ according to local preferences, but results presented in the literature suggest the following strategy: (I) cortically located AVMs with a nidus volume <10 ml could be operated, with or without presurgical embolisation, unless there is a single feeder that can easily be catheterised and embolised for

  9. 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

  10. 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.

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

    PubMed

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

    2015-11-05

    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.

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

    PubMed

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

    2010-11-01

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

  13. Fetal Magnetic Resonance Imaging of Malformations Associated with Heterotaxy

    PubMed Central

    Shah, Parinda H; Anderson, Robert H

    2015-01-01

    Magnetic resonance imaging (MRI) is increasingly used as an investigation during fetal life, particularly for assessment of intracranial masses, congenital diaphragmatic hernia, myelomeningocele, and abdominal masses. As the number of scans increases, so is the variety of congenital malformations being recognized. It is axiomatic that interpretation of the findings is enhanced when attention is paid to the likely findings in the setting of known syndromes, this information then dictating the need for additional acquisition of images. One such syndrome is so-called “visceral heterotaxy”, in which there is typically an isomeric, rather than a lateralized, arrangement of the thoracic and abdominal organs. Typically associated with complex congenital cardiac malformations, heterotaxy can also involve the central nervous system, and produce pulmonary, gastrointestinal, immunologic, and genitourinary malformations. In this review, we discuss how these findings can be demonstrated using fetal MRI.  PMID:26180693

  14. Arteriovenous Malformation Underlying a Plexiform Neurofibroma: An Unusual Presentation

    PubMed Central

    Abbas, Zaheer; Khani, Sepideh; Zare, Javad

    2017-01-01

    Vascular abnormalities associated with neurofibromatosis type 1 are well described in the literature, however, arteriovenous malformation is a very rare finding in neurofibromatosis type 1. We report the case of an 11-year-old girl who presented with a soft mass on the right flank. Provisional diagnosis of plexiform neurofibroma was made on the basis of clinical and histological observations. Because the lesion was warm on palpation, imaging studies were performed to evaluate further and arteriovenous malformation was detected underlying the plexiform neurofibroma. This report emphasizes the importance of careful examination and proper investigations of the plexiform neurofibroma prior to treatment strategies to avoid future complications. The rarity of plexiform neurofibroma in association with arteriovenous malformation at the same site was also highlighted in this report.

  15. Endovascular embolization of life threatening intracranial arterio-venous malformation.

    PubMed

    Khan, S U; Rahman, K M; Siddiqui, M R; Hoque, M A; Mondol, B A; Hussain, S; Mohammad, Q D

    2010-07-01

    Haemorrhagic stroke from cerebral arteriovenous malformations (AVMs) represents 2% of all hemorrhagic strokes. A clear understanding of the diagnostic and treatment algorithms of cerebral AVM management is very important, because AVMs are a cause of hemorrhage in young adults. Surgery, endovascular therapy, and radiosurgery can be used alone or in combination to treat an AVM. We reported a 40 years old man of cerebral arteriovenous malformation (AVM), complicated with intracerebral hemorrhage (ICH). Digital subtraction angiogram was done for diagnosis and endovascular embolization for treatment of the case. This is the first successful cerebral arteriovenous malformations (AVMs) embolization in any government hospital of Bangladesh. The aim of this case report is to inform about this new technologies and emerging treatment strategies in these areas.

  16. Brainstem cavernous malformations: Natural history versus surgical management.

    PubMed

    Walcott, Brian P; Choudhri, Omar; Lawton, Michael T

    2016-10-01

    While brainstem cavernous malformations were once considered inoperable, improvements in patient selection, surgical exposures, intraoperative MRI-guidance, MR tractography, and neurophysiologic monitoring have resulted in good outcomes in the majority of operated patients. In a consecutive series of 104 patients with brainstem cavernous malformations, only 14% of patients experienced cranial nerve or motor dysfunction that was worse at late follow-up, relative to their preoperative condition. Outcomes were predicted by several factors, including larger lesion size, lesions that crossed the midline, the presence of a developmental venous anomaly, older age, and greater time interval from lesion hemorrhage to surgery. The 14% of patients who experienced a persistent neurological deficit as a result of surgery, while substantial from any perspective, compares favorably with the risks of observation based on a recent meta-analysis. Curative resection is a safe and effective treatment for brainstem cavernous malformations that will prevent re-hemorrhage in symptomatic patients.

  17. Antidepressant use during pregnancy and the risk of major congenital malformations in a cohort of depressed pregnant women: an updated analysis of the Quebec Pregnancy Cohort

    PubMed Central

    Bérard, Anick; Zhao, Jin-Ping; Sheehy, Odile

    2017-01-01

    Objective Antidepressant use during gestation has been associated with risk of major congenital malformations but estimates can lack statistical power or be confounded by maternal depression. We aimed to determine the association between first-trimester exposure to antidepressants and the risk of major congenital malformations in a cohort of depressed/anxious women. Setting and participants Data were obtained from the Quebec Pregnancy Cohort (QPC). All pregnancies with a diagnosis of depression or anxiety, or exposed to antidepressants in the 12 months before pregnancy, and ending with a live-born singleton were included. Outcome measures Antidepressant classes (selective serotonin reuptake inhibitors (SSRI), serotonin–norepinephrine reuptake inhibitors (SNRI), tricyclic antidepressants (TCA) and other antidepressants) and types were individually compared with non-exposure during the first trimester (depressed untreated). Major congenital malformations overall and organ-specific malformations in the first year of life were identified. Results 18 487 pregnant women were included. When looking at the specific types of antidepressant used during the first trimester, only citalopram was increasing the risk of major congenital malformations (adjusted OR, (aOR) 1.36, 95% CI 1.08 to 1.73; 88 exposed cases), although there was a trend towards increased risk for the most frequently used antidepressants. Antidepressants with serotonin reuptake inhibition effect (SSRI, SNRI, amitriptyline (the most used TCA)) increased the risk of certain organ-specific defects: paroxetine increased the risk of cardiac defects (aOR 1.45, 95% CI 1.12 to 1.88), and ventricular/atrial septal defects (aOR 1.39, 95% CI 1.00 to 1.93); citalopram increased the risk of musculoskeletal defects (aOR 1.92, 95% CI 1.40 to 2.62), and craniosynostosis (aOR 3.95, 95% CI 2.08 to 7.52); TCA was associated with eye, ear, face and neck defects (aOR 2.45, 95% CI 1.05 to 5.72), and digestive defects (aOR 2

  18. Topical antibiotic ear drops: are they safe?

    PubMed

    Pappas, S; Nikolopoulos, T P; Korres, S; Papacharalampous, G; Tzangarulakis, A; Ferekidis, E

    2006-09-01

    A discharging ear is a very common condition for the general practitioner and the ENT surgeon. Oral and intravenous antibiotics have potential complications, are costly, and exhibit increasing resistance. This study explores the advantages and disadvantages of all common otic preparations and compares effectiveness, safety, cost and complication rates. In chronic otitis media (chronic discharging ears), topical antibiotics seem to be the treatment of choice by comparison with oral or intravenous antibiotics. Cultures and antibiograms do not correspond directly to clinical efficacy, as laboratory determination of resistance does not take into account the high concentration of antibiotics in local preparations. It is safer to use quinolone drops as a first-line treatment, but it is still possible to use short courses of other drops if quinolones are either unavailable or contraindicated (e.g. allergy), or when bacteria are resistant to them. However, in such a situation, a round window membrane involved in an established inflammatory process and therefore less permeable to the passage of topical preparations is the preferred setting, as ototoxicity is a potential complication, especially in the case of gentamicin, in which case patients should be warned accordingly.

  19. [European Portuguese EARS test battery adaptation].

    PubMed

    Alves, Marisa; Ramos, Daniela; Oliveira, Graça; Alves, Helena; Anderson, Ilona; Magalhães, Isabel; Martins, Jorge H; Simões, Margarida; Ferreira, Raquel; Fonseca, Rita; Andrade, Susana; Silva, Luís; Ribeiro, Carlos; Ferreira, Pedro Lopes

    2014-01-01

    Introdução: A utilização de instrumentos de avaliação em saúde adequados é fundamental na gestão da prestação de cuidados. A escassez, em Portugal, de instrumentos específicos para a avaliação do desempenho de crianças utilizadoras de implantes cocleares motivou o trabalho de tradução e de adaptação da bateria de testes EARS (Evaluation of Auditory Responses to Speech) para o português europeu. Esta bateria de testes é hoje um dos instrumentos mais comummente utilizados por equipas de (re)habilitação de crianças surdas com implantes cocleares em todo o mundo. O objetivo a atingir com a validação do EARS foi fornecer às equipas de (re)habilitação um instrumento que permita: (i) monitorizar a evolução individual da reabilitação; (ii) gerir um programa de (re)habilitação de acordo com resultados objetivos, comparáveis entre diferentes equipas de (re)habilitação; (iii) obter dados comparáveis comequipas internacionais; e (iv) melhorar a adesão e a motivação da família e restantes profissionais no ambulatório.Material e Métodos: No processo de tradução e de adaptação da bateria de testes, os procedimentos adotados foram os seguintes: (i) tradução da versão inglesa para português europeu por um tradutor profissional; (ii) revisão dessa tradução realizada por um painel de especialistas constituído por otorrinolaringologistas, terapeutas da fala e técnicos de audiologia; (iii) adaptação dos estímulos de teste pela equipa de terapeutas da fala; e (iv) nova revisão por parte do painel de especialistas.Resultados: São apresentados, para cada um dos instrumentos que compõem a bateria EARS, as adaptações introduzidas, conciliando as características e os objetivos originais dos instrumentos com as particularidades linguísticas e culturais da população portuguesa.Discussão: São discutidas as dificuldades encontradas durante o processo de tradução e de adaptação e as soluções adotadas. São feitas

  20. [Urinary tract abnormalities with anorrectal malformations (author's transl)].

    PubMed

    Nogués, A; Ceres, M L; Olagüe, R; Andrés, V; Lanuza, A

    1978-01-01

    Thirty five patients with anorrectal malformations are reviewed. These are divided in high and low anomalies according to some simple clinical data, better than the drawing of reference lines to determinate the height of puborrectalis muscle. Malformations were associated in 13 cases with urinary tract estructural anomalies and in four cases with isolated vesico-ureteral reflux. Diagnosis of urinary tract infection was made in 14 patients, 12 of them with recto-urinary fistula. A point is made about the complete and early exploration of all these patients to prevent irreparable renal damage that could be developed.

  1. Antenatal diagnosis of congenital renal malformations using ultrasound.

    PubMed

    Sanghvi, K P; Merchant, R H; Gondhalekar, A; Lulla, C P; Mehta, A A; Mehta, K P

    1998-08-01

    Our objectives were to determine the accuracy of antenatal sonography for the detection of congenital renal malformations and to characterize the type of malformations, seen in a 3-year prospective study at a university-affiliated maternity hospital. Participants were 31,217 pregnant women, during the study period, and subjects were 65 fetuses in whom renal malformations were detected on antenatal ultrasound. Pelvic ultrasound scans were performed at least once between 20 and 37 weeks' gestation on all pregnant women attending the antenatal clinic of the hospital for the detection of renal malformations. Fetal urinary sampling, diversion procedures, or termination of pregnancy were carried out as required in those detected to have renal anomalies. Postnatal diagnosis was confirmed by sonography or autopsy. Diagnostic procedures and renal surgery were performed postnatally if indicated. Sixty-five fetuses (0.2 per cent) were diagnosed to have congenital renal malformation antenatally at a mean gestational age of 28.4 weeks. A dilated urinary system was seen in 39, cystic renal disease in 15, agenesis/hypoplasia in six, combined lesions in four, and a horseshoe kidney in one. Oligohydramnios was noted in 20 (31 per cent) pregnancies. Multiple congenital malformations associated with renal anomalies were detected in 12 pregnancies. Termination was carried out at 20 weeks in two pregnancies for lethal malformations; fetal urinary sampling was done in two fetuses with obstructed uropathy, and a vesicoamniotic shunt inserted in one. Postnatal ultrasound confirmed a dilated urinary system in 32, cystic renal dysplasia in 15, renal aplasia/hypoplasia in five, combined lesions in six, and a horseshoe and an ectopic kidney in one each. Five infants were found to be normal. There were seven stillbirths and seven neonatal deaths. Radionuclide scans showed obstruction in nine, decreased renal function in six, and absent renal functions in 10 infants. Micturating

  2. Influence of maternal distress during pregnancy on fetal malformations.

    PubMed

    Blomberg, S

    1980-10-01

    The aim of the study was to investigate whether emotional stress in a pregnant woman might have an adverse effect in the form of malformations on fetal development. The children of 1,263 women whose applications for legal abortion in 1960 had been refused were compared with the next children born in the same delivery wards and paired matched controls were thereby obtained. Results. 1) The incidence of malformations according to the criteria established by the Swedish Register of Malformations was 1.8% in the proband series as against 1.1% in the control series. 2) The incidence of malformation increased with higher age and lower social class in the proband series, whereas no such connection was found in the control series. 3) The incidence of malformations for children of abortion applicants 25 years of age and above from social class III was 3% in the proband series as against 0.6% in the control series (P = 0.017). 4) One malformation, cleft palate, occurred at a significantly (P less than 0.01) higher incidence than in the country as a whole, four cases against the expected value of 0.69. 5) Etiological analysis showed that three of the cases were chromosomal aberrations, all of them Down's syndrome, in two cases there was a genetic background and two cases were thalidomide-induced phocomelia. In three proband children there may have been a connection with the abuse of alcohol by the mother. 6) When the chromosomal, genetic and thalidomide cases are excluded, the preponderance of malformations in the proband children of mothers 25 years of age and above belonging to social class III still remains (2.2% vs. 0.3%, P = 0.017). Conclusion. The results may be seen as support for the hypothesis that emotional stress in a pregnant woman, operationally defined by the factor unwanted pregnancy, may interfere with fetal development and result in a higher incidence of malformations. The interference may occur directly via psycho-endocrinal or autonomous mechanisms or

  3. Split cervical spinal cord malformation and vertebral dysgenesis.

    PubMed

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

    2009-11-01

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

  4. Vascular malformations: an update on imaging and management.

    PubMed

    Sierre, Sergio; Teplisky, Darío; Lipsich, José

    2016-04-01

    Vascular malformations comprise a broad and heterogeneous range of lesions that often represent a diagnostic and therapeutic challenge for the pediatrician. For a long time, the use of an inaccurate nomenclature has led to confusion. Since management depends on the specific vascular malformation, a proper classification and identification is critical. The objective of this article is to provide the necessary information about the current classification and terminology of vascular anomalies, including basic concepts about available imaging diagnostic and therapeutic tools for the management of such complex condition.

  5. [Type I Chiari malformation associated with cerebellar atrophy. Case report].

    PubMed

    Moscote-Salazar, Luis Rafael; Calderón-Miranda, Willem Guillermo; Alvis-Miranda, Hernando Raphael; Lee-Aguirre, Ángel; Alcalá-Cerra, Gabriel

    2017-01-01

    Chiari malformation is characterized by caudal displacement of the cerebellar tonsils that penetrate into the spinal canal through the foramen magnum, achieving reach the atlas or axis. trunk and any drop of the fourth ventricle is observed. Typically is seen in young adults. In some cases scoliosis and Syringomyelic cavities may occur. The authors present (as far as they know) the first case in the literature with long term follow-up, of a caucasian woman with an unusual form of cerebellar atrophy and Chiari Type I malformation, suffering from weakness in his upper and lower extremities with rapidly progression. The patient was successfully treated with suboccipital decompression and C1 laminectomy.

  6. Diffusion imaging and tractography of congenital brain malformations.

    PubMed

    Wahl, Michael; Barkovich, A James; Mukherjee, Pratik

    2010-01-01

    Diffusion imaging is an MRI modality that measures the microscopic molecular motion of water in order to investigate white matter microstructure. The modality has been used extensively in recent years to investigate the neuroanatomical basis of congenital brain malformations. We review the basic principles of diffusion imaging and of specific techniques, including diffusion tensor imaging (DTI) and high angular resolution diffusion imaging (HARDI). We show how DTI and HARDI, and their application to fiber tractography, has elucidated the aberrant connectivity underlying a number of congenital brain malformations. Finally, we discuss potential uses for diffusion imaging of developmental disorders in the clinical and research realms.

  7. Nursery care of the newborn with malformation syndrome.

    PubMed

    Pluchinotta, Francesca Romana; Memo, Luigi

    2009-10-01

    Congenital malformations are not so rare, and represent a relevant challenge to health care providers. We delineate a methodology for the clinical approach to the malformed newborn, that takes into account the needs of the patient as single person and as part of a familiar and social context. We consider first the medical problem that the neonatologists face in the nursery and neonatal intensive care unit regarding clinical assistance, diagnosis, parent counselling and hospital discharge. Then we focus on the periodic follow-up, and the role of the primary care physician and the specialist in the assistance of this patients.

  8. 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

  9. Diagnosis and management of extensive vascular malformations of the lower limb: part I. Clinical diagnosis.

    PubMed

    Redondo, Pedro; Aguado, Leyre; Martínez-Cuesta, Antonio

    2011-11-01

    There is significant confusion in the literature when describing vascular anomalies, and vascular malformations are often misnamed or incorrectly classified. Part I of this two-part series on the diagnosis and management of extensive vascular malformations of the lower limbs will discuss the dermatologist's role in the diagnosis of these lesions. At least nine types of vascular malformations with specific clinical and radiologic characteristics must be distinguished in the lower limbs: Klippel-Trénaunay syndrome, port-wine stain with or without hypertrophy, cutis marmorata telangiectatica congenita, macrocephaly-capillary malformation, Parkes Weber syndrome, Stewart-Bluefarb syndrome, venous malformation, glomuvenous malformation, and lymphatic malformation. This article highlights the differences in clinical appearance and discusses the differential diagnosis of extensive vascular malformations in an attempt to ensure earlier diagnosis and better outcomes for these patients.

  10. Consensus conference on Chiari: a malformation or an anomaly? Scoliosis and others orthopaedic deformities related to Chiari 1 malformation.

    PubMed

    Colombo, Luca F; Motta, Francesco

    2011-12-01

    In this article, we analysed the orthopaedic malformation in patients affected by Chiari I malformations as well as conservative or surgical treatment. The most common deformity in these patients is scoliosis. Different studies suggest a causal relation between syringomyelia and spinal deformities that differ by the type of deformities: asymptomatic scoliosis is characterized by a higher incidence of a single curve and convexity to the left, while symptomatic scoliosis is characterized by a double thoracolumbar curve. The conservative treatment with brace in these patients is not effective and scoliosis is typically evolutive. The evidence of the international data is that in patients without myelomeningocele or congenital scoliosis, but with Arnold Chiari I malformation and syringomyelia, suboccipital craniectomy gives the best chance for syrinx reduction and scoliosis improvement, particulary in children younger than 10 years and below a Cobb angle of 30°. The orthopaedic treatment in late decompression or in progressive curve is spine arthrodesis.

  11. Diffusion-weighted imaging in fetuses with unilateral cortical malformations and callosal agenesis.

    PubMed

    Glenn, O A; Quiroz, E M; Berman, J I; Studholme, C; Xu, D

    2010-06-01

    DWI was performed in fetuses with callosal agenesis and unilateral cortical malformations. ADC values were retrospectively measured in the developing white matter underlying the cortical malformation and compared with the corresponding contralateral white matter. In all 3 patients, ADC values were lower under the areas of cortical malformation compared with the normal contralateral side. Our findings suggest that there are structural differences in the developing white matter underlying areas of cortical malformation.

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

    PubMed

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

    2000-12-01

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

  13. Ear length and kidney function decline after kidney donation.

    PubMed

    Katavetin, Pisut; Watanatorn, Salin; Townamchai, Natavudh; Avihingsanon, Yingyos; Praditpornsilpa, Kearkiat

    2016-11-01

    The preservation of kidney function after kidney donation depends on the kidney reserve - the potential of the remaining kidney to boost their function after loss of the other kidney. In Traditional Chinese Medicine, size and shape of the external ears are examined to evaluate the person's kidney health. We hypothesized that ear size might be a practical yet overlooked marker of kidney reserve. Fifty kidney transplantation donors were participated in this study. The length and width of both ears of all participants were measured during one of the post-donation visits. Pre-donation serum creatinine and post-donation serum creatinine as well as other relevant parameters (age, sex, weight, height, etc.) of the participants were extracted from medical records. The estimated GFR was calculated from serum creatinine, age and sex using the CKD-EPI equation. Ear length negatively associated with %GFR decline after kidney donation. For every 1 cm increase in ear length, it was associated with 5.7% less GFR decline after kidney donation (95% Confidence Interval 0.2 to 11.3, P = 0.04). Ear width, as well as age, sex, body weight, height, body mass index, and pre-donation eGFR did not significantly associate with the GFR decline. Our findings support the notion of Traditional Chinese Medicine that ear morphology may be associated with kidney health and suggest that ear length might be a useful predictor of kidney function decline after kidney donation.

  14. 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)

  15. Alternative Ear-Canal Measures Related to Absorbance

    PubMed Central

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

    2013-01-01

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

  16. Major evolutionary transitions and innovations: the tympanic middle ear

    PubMed Central

    2017-01-01

    One of the most amazing transitions and innovations during the evolution of mammals was the formation of a novel jaw joint and the incorporation of the original jaw joint into the middle ear to create the unique mammalian three bone/ossicle ear. In this review, we look at the key steps that led to this change and other unusual features of the middle ear and how developmental biology has been providing an understanding of the mechanisms involved. This starts with an overview of the tympanic (air-filled) middle ear, and how the ear drum (tympanic membrane) and the cavity itself form during development in amniotes. This is followed by an investigation of how the ear is connected to the pharynx and the relationship of the ear to the bony bulla in which it sits. Finally, the novel mammalian jaw joint and versatile dentary bone will be discussed with respect to evolution of the mammalian middle ear. This article is part of the themed issue ‘Evo-devo in the genomics era, and the origins of morphological diversity’. PMID:27994124

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

    Code of Federal Regulations, 2014 CFR

    2014-01-01

    ... activities over which BIS exercises regulatory jurisdiction under the EAR. Conversely, items and activities... programs administered by other agencies. Items and activities subject to the EAR are not necessarily exempted from the control programs of other agencies. Although BIS and other agencies that...

  18. 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...

  19. 21 CFR 344.12 - Ear drying aid active ingredient.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 21 Food and Drugs 5 2011-04-01 2011-04-01 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...

  20. 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...

  1. 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...

  2. Pseudocarcinomatous hyperplasia involving the ear from gout: a diagnostic pitfall.

    PubMed

    Morrissey, Kelly A; Garcia-Albea, Victoria; Goldberg, Lynne J

    2014-01-01

    The ear is a characteristic location for deposition of uric acid in patients with gout. Pseudocarcinomatous hyperplasia has not been described in this location. We report three patients with tophaceous gout on the ear whose biopsies exhibited epidermal hyperplasia mimicking squamous cell carcinoma, in order to call attention to this potential diagnostic pitfall.

  3. 21 CFR 344.12 - Ear drying aid active ingredient.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 21 Food and Drugs 5 2010-04-01 2010-04-01 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...

  4. 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.

  5. 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..., nose, throat, and equilibrium. Ear, nose, throat, and equilibrium standards for a third-class airman... by, or that may reasonably be expected to be manifested by, vertigo or a disturbance of equilibrium....

  6. 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..., nose, throat, and equilibrium. Ear, nose, throat, and equilibrium standards for a second-class airman..., vertigo or a disturbance of equilibrium....

  7. 14 CFR 67.305 - Ear, nose, throat, and equilibrium.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... 14 Aeronautics and Space 2 2010-01-01 2010-01-01 false Ear, nose, throat, and equilibrium. 67.305..., nose, throat, and equilibrium. Ear, nose, throat, and equilibrium standards for a third-class airman... by, or that may reasonably be expected to be manifested by, vertigo or a disturbance of equilibrium....

  8. 14 CFR 67.105 - Ear, nose, throat, and equilibrium.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... 14 Aeronautics and Space 2 2010-01-01 2010-01-01 false Ear, nose, throat, and equilibrium. 67.105..., nose, throat, and equilibrium. Ear, nose, throat, and equilibrium standards for a first-class airman... may reasonably be expected to be manifested by, vertigo or a disturbance of equilibrium....

  9. 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..., nose, throat, and equilibrium. Ear, nose, throat, and equilibrium standards for a second-class airman..., vertigo or a disturbance of equilibrium....

  10. 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..., nose, throat, and equilibrium. Ear, nose, throat, and equilibrium standards for a first-class airman... may reasonably be expected to be manifested by, vertigo or a disturbance of equilibrium....

  11. 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..., nose, throat, and equilibrium. Ear, nose, throat, and equilibrium standards for a third-class airman... by, or that may reasonably be expected to be manifested by, vertigo or a disturbance of equilibrium....

  12. 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..., nose, throat, and equilibrium. Ear, nose, throat, and equilibrium standards for a third-class airman... by, or that may reasonably be expected to be manifested by, vertigo or a disturbance of equilibrium....

  13. 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..., nose, throat, and equilibrium. Ear, nose, throat, and equilibrium standards for a first-class airman... may reasonably be expected to be manifested by, vertigo or a disturbance of equilibrium....

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

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... 14 Aeronautics and Space 2 2010-01-01 2010-01-01 false Ear, nose, throat, and equilibrium. 67.205..., nose, throat, and equilibrium. Ear, nose, throat, and equilibrium standards for a second-class airman..., vertigo or a disturbance of equilibrium....

  15. 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..., nose, throat, and equilibrium. Ear, nose, throat, and equilibrium standards for a first-class airman... may reasonably be expected to be manifested by, vertigo or a disturbance of equilibrium....

  16. 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..., nose, throat, and equilibrium. Ear, nose, throat, and equilibrium standards for a third-class airman... by, or that may reasonably be expected to be manifested by, vertigo or a disturbance of equilibrium....

  17. 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..., nose, throat, and equilibrium. Ear, nose, throat, and equilibrium standards for a second-class airman..., vertigo or a disturbance of equilibrium....

  18. 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..., nose, throat, and equilibrium. Ear, nose, throat, and equilibrium standards for a second-class airman..., vertigo or a disturbance of equilibrium....

  19. 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..., nose, throat, and equilibrium. Ear, nose, throat, and equilibrium standards for a first-class airman... may reasonably be expected to be manifested by, vertigo or a disturbance of equilibrium....

  20. 21 CFR 874.4420 - Ear, nose, and throat manual surgical instrument.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ... device includes the esophageal dilator; tracheal bistour (a long, narrow surgical knife); tracheal dilator; tracheal hook; laryngeal injection set; laryngeal knife; laryngeal saw; laryngeal trocar...; wire ear loop; microrule; mirror; mobilizer; ear, nose, and throat punch; ear, nose and throat...

  1. 21 CFR 874.4420 - Ear, nose, and throat manual surgical instrument.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ... device includes the esophageal dilator; tracheal bistour (a long, narrow surgical knife); tracheal dilator; tracheal hook; laryngeal injection set; laryngeal knife; laryngeal saw; laryngeal trocar...; wire ear loop; microrule; mirror; mobilizer; ear, nose, and throat punch; 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

    ... device includes the esophageal dilator; tracheal bistour (a long, narrow surgical knife); tracheal dilator; tracheal hook; laryngeal injection set; laryngeal knife; laryngeal saw; laryngeal trocar...; wire ear loop; microrule; mirror; mobilizer; ear, nose, and throat punch; ear, nose and throat...

  3. Common Post-Op Ear Drops Tied to Eardrum Perforations in Kids

    MedlinePlus

    ... gov/news/fullstory_164372.html Common Post-Op Ear Drops Tied to Eardrum Perforations in Kids Rate ... 2017 (HealthDay News) -- Children who suffer through multiple ear infections are often candidates for ear tube surgery. ...

  4. Anatomy of the Human Ear/Questions to Ask your Hearing Professional

    MedlinePlus

    ... Section: Focus on Communication Anatomy of the Human Ear/ Questions to Ask your Hearing Professional Past Issues / ... A feeling of fullness or fluid in the ear Ringing in your ears (called tinnitus) Causes Aging ...

  5. Chiari type 1 malformation in a pseudotumour cerebri patient: is it an acquired or congenital Chiari malformation?

    PubMed

    Istek, Seref

    2014-06-04

    Chiari malformation type 1 (CM1) is a developmental abnormality of the cerebellar tonsils. Patients with CM1 commonly present with headache. Papilloedema is rarely seen in CM1. However, a 52-year-old woman presented to the hospital with a headache and her ophthalmological examination revealed bilateral papilloedema. Her cranial MRI was compatible with borderline CM1. Bilateral papilloedema and headache suggested idiopathic intracranial hypertension (IIH) as the preliminary diagnosis. IIH is a rare case in CM1. This article argues about this association and discusses as to whether it is an acquired or congenital Chiari malformation.

  6. 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,…

  7. 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...

  8. Impedance matching, optimum velocity, and ideal middle ears.

    PubMed

    Peake, W T; Rosowski, J J

    1991-05-01

    One way to assess an ear's performance as a receiver of acoustic power is to consider impedance matching at the tympanic membrane. Assumptions about some of the impedances involved have lead to the idea of an optimum velocity magnitude (per unit pressure), which has been used as a test of middle-ear performance. We show that this approach is not a realistic way to assess effectiveness of power absorption at the tympanic membrane. More generally, we suggest that, if the performance of the combined external-and-middle ear in collecting acoustic power and delivering it to the inner ear is considered, the external- and middle-ear power-transfer efficiencies, as well as impedance matching, are involved in relating performance to an ideal.

  9. 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

  10. Radiology of external ear: indications, normal anatomy, and pathological processes.

    PubMed

    Mazón, M; Pont, E; Montesinos, P; Carreres-Polo, J; Más-Estellés, F

    2016-01-01

    The external ear is accessible to direct examination; the clinical history and otoscopy are sufficient to diagnose and treat most diseases of the external ear. We aim to describe the normal anatomy of the external ear, specify the indications for imaging tests, and review the clinical and radiological manifestations of the most common diseases affecting the external ear. We classify these diseases according to their origin into congenital, inflammatory, infectious, or traumatic disease or benign bone tumors or malignant tumors. Imaging does not play an important role in diseases of the external ear, but in certain clinical scenarios it can be crucial for reaching a concrete diagnosis and establishing the best treatment. Computed tomography is the first-choice technique for most diseases. Magnetic resonance imaging complements computed tomography and makes it possible to differentiate among different tissue types and to evaluate the extension of disease accurately.

  11. Segmentation algorithms for ear image data towards biomechanical studies.

    PubMed

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

    2014-01-01

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

  12. Bilateral external ear canal osteomas - discussion on a clinical case.

    PubMed

    Gheorghe, D C; Stanciu, A E; Ulici, A; Zamfir-Chiru-Anton, A

    2016-01-01

    Osteomas of the external ear are uncommon benign tumors that need to be differentiated from the external ear canal exostoses, bony proliferations that are linked mainly to cold-water exposure. Clinical manifestations vary from no symptoms to recurrent local infections and external ear cholesteatoma. Objective: presenting a rare case that we did not find described in the published literature. A patient with multiple long-term asymptomatic osteomas of both external ear canals presented to our department. Material: Data recorded from the patient's medical record was reviewed and analyzed. Surgery was performed and histology confirmed the presumptive diagnosis. Results: There was a discrepancy between the local severity of the disease, with a complete obstruction of his ear canals, and the long-term disease-free status of the patient. Conclusion: We hypothesized about the etiology of these multiple bilateral osteomas of the EAC, in light of the clinical and surgical findings.

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

    PubMed

    Dutta, Mainak; Ghatak, Soumya; Biswas, Gautam

    2013-08-01

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

  14. Dichotic ear preferences of stuttering children and adults.

    PubMed

    Sommers, R K; Brady, W A; Moore, W H

    1975-12-01

    39 stutterers and 39 normal speakers indicated their ear preferences for dichotically presented words and digits. A single response mode for both dichotic words and digits was selected to study speech perception. Stutterers showed significantly less of the normal right-ear preference for dichotic words and digits than non-stutterers. The proportion of stutterers who failed to demonstrate a right-ear preference for dichotic words was significantly greater than for non-stutterers. 18% of the stutterers and none of the non-stutterers showed reversed or a left-ear preference for dichotic digits. Although non-stuttering children and adults performed alike on the dichotic tasks, the right-ear dichotic-words scores of stuttering children were significantly smaller than those of adult stutterers. The results are related to an early notion that stuttering may be related to mixed dominance and recent evidence showing that large percentages of older stuttering children show spontaneous remission of stuttering.

  15. Inner ear insult suppresses the respiratory response to carbon dioxide.

    PubMed

    Allen, T; Juric-Sekhar, G; Campbell, S; Mussar, K E; Seidel, K; Tan, J; Zyphur, M; Villagracia, L; Stephanian, D; Koch, H; Ramirez, J M; Rubens, D D

    2011-02-23

    Compensated respiratory acidosis has been observed in a significant number of patients with active vestibular disease. We therefore hypothesized that the inner ear may play an unrecognized integral role in respiratory control. To test this premise, we investigated whether mice with induced inner ear injury demonstrated any alteration in their respiratory response to inhaled carbon dioxide (CO(2)). Experimental mice and control mice were included in two separate experiments. Intra-tympanic gentamycin injections were administered to induce inner ear damage in experimental animals. Hearing loss and vestibular dysfunction were tested 1-week after injections to confirm presence of inner ear insult, following which the animal's respiratory response to inhalation of 8% CO(2) was examined. Mice with inner ear injury (n=60) displayed a significantly diminished hypercapnic ventilatory response (HCVR). This contrasted with the normal HCVR seen in control mice that had not undergone tympanic injections (n=30), controls that received tympanic injections with saline (n=5), and controls that had gentamicin administered systemically (n=5). In response to inspired CO(2), the mean respiratory frequency of control mice increased by an average of 50% over their baseline values for both parts of the experiment. In contrast, the ear-damaged experimental group mean values increased by only three breaths per minute (bpm) (2%) in the first experiment and by 28 bpm (11%) in the second experiment. Inner ear damage significantly reduces the respiratory response to CO(2) inhalation. In addition to the established role of the inner ear organ in hearing and balance, this alludes to an unidentified function of the inner ear and its interconnecting neuronal pathways in respiratory regulation. This finding may offer valuable new clues for disease states with abnormal respiratory control where inner ear dysfunction may be present.

  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…

  17. Congenital subclavian arteriovenous malformation causing cardiac failure in an adult.

    PubMed

    Anoop, T M; Sreejith, P; Thomas, Joby K; Gailin, B; Jabbar, P K; Ittycheria, Cherian C; George, Raju

    2009-07-01

    Congenital arteriovenous malformations (AVMs) of the thoracic region are rarely reported in adults. The authors report an unusual case of a 30-year-old man who presented with a large congenital AVM and heart failure. The diagnosis was made using transthoracic Doppler echocardiography and computed tomography. Embolization followed by surgical resection of the AVM resulted in the prompt relief of heart failure.

  18. Spitz nevus arising upon a congenital glomuvenous malformation.

    PubMed

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

    2013-01-01

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

  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. 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…

  1. "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…

  2. Cardiac output determinations with ear piece densitometry.

    PubMed

    Hedenstierna, G; Schildt, B

    1975-01-01

    The results of cardiac output determinations by a dye dilution technique were compared using (a) a dichromatic earpiece which was calibrated as a flow-through cuvette, but also permitted automatic computing by virtue of a pressure capsule, and (b) an ordinary flow-through densitometer. Eleven subjects, some with cardio-pulmonary disease, were investigated. Cardiac outputs were systematically overestimated when automatically computed. The results obtained by manual calculation with the ear-piece corresponded more nearly with those derived from the flow-through cuvette, but still with a deviation from the identity line and with a residual standard deviation of 0.8 l/min. Double determinations had a residual standard deviation of 0.7 l/min. Despite its ease of handling, an earpiece densitometer seems to be too unreliable to be suitable for routine use.

  3. Analysis of Earing in Deep Drawn Cups

    SciTech Connect

    Aretz, Holger; Aegerter, Johannes; Engler, Olaf

    2010-06-15

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

  4. Ear reconstruction with porous polyethylene implants.

    PubMed

    Berghaus, Alexander; Stelter, Klaus; Naumann, Andreas; Hempel, John Martin

    2010-01-01

    This article describes a surgical technique using porous polyethylene as the framework material for ear reconstruction. In comparison to the use of rib cartilage, porous polyethylene - first described by Berghaus in 1982 - provides better definition and projection as well as congruency with the opposite side. Hospitalization time is significantly shorter. There are less surgical interventions than with traditional microtia operations that use rib cartilage, and the patient is spared the additional procedure needed to remove the rib cartilage, with all the associated complications as well as the resulting thorax scar. Also, reconstruction can take place at an earlier age, which is advantageous for those concerned. Using porous polyethylene as the frame material, a temporoparietal flap and full-thickness skin cover, we have been able to achieve very convincing results over recent years.

  5. Clinical Characteristics of Patients Who Underwent Surgery for Genital Tract Malformations at Peking Union Medical College Hospital across 31 Years

    PubMed Central

    Wang, Guang-Han; Zhu, Lan; Liu, Ai-Ming; Xu, Tao; Lang, Jing-He

    2016-01-01

    Background: Female genital malformations represent miscellaneous deviations from normal anatomy. This study aimed to explore the clinical characteristics of patients who underwent surgery for genital tract malformations at Peking Union Medical College Hospital (PUMCH) during a 31-year period. Methods: We retrospectively reviewed surgical cases of congenital malformation of the female genital tract at PUMCH for a 31-year period, analyzed the clinical characteristics of 1634 hospitalized patients, and investigated their general condition, diagnosis, and treatment process. Results: The average patient age was 27.6 ± 9.9 years. The average ages of patients who underwent surgery for uterine malformation and vaginal malformation were 31.9 ± 8.8 years and 24.7 ± 9.0 years, respectively; these ages differed significantly (P < 0.01). Among patients with genital tract malformation, the percentages of vaginal malformation, uterine malformation, vulva malformation, cervical malformation, and other malformations were 43.9%, 43.5%, 7.4%, 2.3%, and 2.8%, respectively. Among patients with uterine malformation, 34.5% underwent surgery for the genital tract malformation, whereas in patients with vaginal malformation, the proportion is 70.6%; the difference between the two groups was statistically significant (P < 0.01). The percentage of complications of the urinary system in patients with vaginal malformations was 10.2%, which was statistically significantly higher than that (5.3%) in patients with uterine malformations (P < 0.01). Conclusions: Compared to patients with uterine malformations, patients with vaginal malformations displayed more severe clinical symptoms, a younger surgical age, and a greater need for attention, early diagnosis, and treatment. Patients with genital tract malformations, particularly vaginal malformations, tend to have more complications of the urinary system and other malformations than patients with uterine malformations. PMID:27748336

  6. Passage of albumin from the middle ear to the inner ear in otitis media in the chinchilla

    SciTech Connect

    Goldberg, B.; Goycoolea, M.V.; Schleivert, P.M.; Shea, D.; Schachern, P.; Paparella, M.M.; Carpenter, A.M.

    1981-08-01

    A study of the permeability of the middle ear-inner ear interface for macromolecules was carried out in chinchillas with open and obstructed eustachian tubes utilizing tritiated human serum albumin and immunoelectrophoresis. Tritiated albumin was placed in the round window niche area or normal animals and animals in which the eustachian tubes had been obstructed for 24 hours or 14 days. The tritiated albumin was allowed to remain in the middle ear cavity for 24 hours, Samples of middle ear effusion, perilymph, blood and cerebrospinal fluid were collected and measured for radioactivity. Radioactivity was demonstrated in the perilymph. Samples of middle ear effusions and perilymph were also studied by immunoelectrophoresis with goat antihuman albumin. Albumin placed in the round window niche of an experimental animal could be recovered unchanged in the perilymph. The results suggest a pathophysiologic explanation for the association of otitis media and sensorineural hearing loss or endolymphatic hydrops.

  7. Abernethy malformation: one of the etiologies of hepatopulmonary syndrome.

    PubMed

    Alvarez, Alfonso E; Ribeiro, Antônio F; Hessel, Gabriel; Baracat, Jamal; Ribeiro, José D

    2002-11-01

    Hepatopulmonary syndrome (HPS) is the clinical relationship between hepatic disease and the existence of pulmonary vascular dilatations, which can result in a range of arterial oxygenation abnormalities. It is probably caused by an alteration in the synthesis or metabolism of vasoactive pulmonary substances at a hepatic level, leading to vasodilatation of pulmonary vessels and diffusion perfusion defects. The Abernethy malformation is characterized by the congenital diversion of portal blood away from the liver, by either end-to-side or side-to-side shunt. Here, we report on a 5-year-and-11-month-old-boy who had started cyanosis at age 4 years and 11 months, and did not have any other pulmonary or cardiac signs or symptoms. In the investigation, arterial blood gases revealed a PaO(2) of 41.4 mm Hg. The chest x-ray film and echo Doppler cardiography were normal. Nuclear scanning with Technetium 99m-labeled macroaggregated albumin showed the presence of arteriovenous shunt, at 47%. Abdominal echography revealed Abernethy malformation with an absence of portal vein. We concluded that the patient had HPS caused by Abernethy malformation. The possible mechanism is that in this malformation, there is a deviation in the blood that comes from the spleen to the vena cava without passing through the liver, so there is no metabolism of some substances which can be responsible for the imbalance between the vasodilatation and the vasoconstriction of the pulmonary circulation. Abernethy malformation must be included as one of the etiologies of hepatopulmonary syndrome. This is the first case described in the literature with this form of presentation.

  8. 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.

  9. 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.

  10. [Proteus syndrome: Case report of bladder vascular malformation causing massive hematuria].

    PubMed

    Abbo, O; Bouali, O; Galinier, P; Moscovici, J

    2012-02-01

    Proteus syndrome is a rare, sporadic disorder consisting of disproportionate overgrowth of multiple tissues, vascular malformations, and connective tissue or epidermal nevi. Due to mosaic pattern of distribution, the phenotypes are variable and diverse. Vascular malformations are part of the major criteria used to define and diagnose this syndrome. It can involve the gastrointestinal tract, spleen, or the urinary tract but bladder malformations are rare. We report here a case of bladder vascular malformation in a 12-year-old boy known to have Proteus syndrome and review the literature on bladder malformations or tumors in this syndrome.

  11. 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

  12. 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.

  13. An in vitro model of murine middle ear epithelium

    PubMed Central

    Mulay, Apoorva; Akram, Khondoker M.; Williams, Debbie; Armes, Hannah; Russell, Catherine; Hood, Derek; Armstrong, Stuart; Stewart, James P.; Brown, Steve D. M.; Bingle, Lynne

    2016-01-01

    ABSTRACT Otitis media (OM), or middle ear inflammation, is the most common paediatric disease and leads to significant morbidity. Although understanding of underlying disease mechanisms is hampered by complex pathophysiology it is clear that epithelial abnormalities underpin the disease. There is currently a lack of a well-characterised in vitro model of the middle ear (ME) epithelium that replicates the complex cellular composition of the middle ear. Here, we report the development of a novel in vitro model of mouse middle ear epithelial cells (mMECs) at an air–liquid interface (ALI) that recapitulates the characteristics of the native murine ME epithelium. We demonstrate that mMECs undergo differentiation into the varied cell populations seen within the native middle ear. Proteomic analysis confirmed that the cultures secrete a multitude of innate defence proteins from their apical surface. We showed that the mMECs supported the growth of the otopathogen, nontypeable Haemophilus influenzae (NTHi), suggesting that the model can be successfully utilised to study host–pathogen interactions in the middle ear. Overall, our mMEC culture system can help to better understand the cell biology of the middle ear and improve our understanding of the pathophysiology of OM. The model also has the potential to serve as a platform for validation of treatments designed to reverse aspects of epithelial remodelling that underpin OM development. PMID:27660200

  14. Inverse solution of ear-canal area function from reflectance.

    PubMed

    Rasetshwane, Daniel M; Neely, Stephen T

    2011-12-01

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

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

    PubMed Central

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

    2008-01-01

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

  16. 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

  17. 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

  18. Decrease in Middle Ear Resonance Frequency During Pregnancy.

    PubMed

    Dag, Emine Kutlu; Gulumser, Cagri; Erbek, Seyra

    2016-04-20

    Many physiological changes occur during pregnancy. The aim of the study was to reveal whether there is a change in middle ear resonance frequency during pregnancy. A prospective case-control study was designed at a tertiary referral center. The study included 46 pregnant women at the third trimester (27-40 weeks) and 43 nonpregnant voluntary women. All the study subjects underwent pure-tone audiometry and multifrequency tympanometry. Pure-tone hearing levels at frequencies of 250 to 8000 Hz and resonance frequency values were compared between pregnant and nonpregnant women. Impact of age, side of the tested ear, and weight gained in pregnancy on resonance frequency were evaluated. Air conduction threshold values at frequencies of 250 Hz and 500 Hz were significantly higher in pregnant women than in the control group (P<0.001). Middle ear resonance frequency values of both ears in pregnant women were found to be significantly lower than those in control group (P<0.001). There was no statistically significant relation of middle ear resonance frequency values to age or side of the tested ear in both groups (P>0.05). A negative correlation between weight gained in pregnancy and middle ear resonance frequency values was determined for the left ear (correlation coefficient for left ears: -0.348, P=0.018). The results of this study suggest that resonance frequency may be decreased during the pregnancy. More comprehensive studies in which many pregnant women followed regularly before and after pregnancy are needed to have more certain links.

  19. Decrease in Middle Ear Resonance Frequency During Pregnancy

    PubMed Central

    Dag, Emine Kutlu; Gulumser, Cagri; Erbek, Seyra

    2016-01-01

    Many physiological changes occur during pregnancy. The aim of the study was to reveal whether there is a change in middle ear resonance frequency during pregnancy. A prospective case-control study was designed at a tertiary referral center. The study included 46 pregnant women at the third trimester (27-40 weeks) and 43 nonpregnant voluntary women. All the study subjects underwent pure-tone audiometry and multifrequency tympanometry. Pure-tone hearing levels at frequencies of 250 to 8000 Hz and resonance frequency values were compared between pregnant and nonpregnant women. Impact of age, side of the tested ear, and weight gained in pregnancy on resonance frequency were evaluated. Air conduction threshold values at frequencies of 250 Hz and 500 Hz were significantly higher in pregnant women than in the control group (P<0.001). Middle ear resonance frequency values of both ears in pregnant women were found to be significantly lower than those in control group (P<0.001). There was no statistically significant relation of middle ear resonance frequency values to age or side of the tested ear in both groups (P>0.05). A negative correlation between weight gained in pregnancy and middle ear resonance frequency values was determined for the left ear (correlation coefficient for left ears: –0.348, P=0.018). The results of this study suggest that resonance frequency may be decreased during the pregnancy. More comprehensive studies in which many pregnant women followed regularly before and after pregnancy are needed to have more certain links. PMID:27588163

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

    PubMed Central

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

    2014-01-01

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

  1. [Lop ear - knife, tape, or nothing at all?].

    PubMed

    Klockars, Tuomas

    2013-01-01

    More than 200 different surgical techniques of correction of lop ear have been published. The operation is usually recommended to be performed at the age of six years or after. In addition, lop ear surgery involves risks, the most common complications being bleeding, infections, sensory alterations and scarring problems. Surgical preference and decision should always be based on realistic expectations of the patient or the parents, and prior to the decision they should have adequate information about the nature of the procedure and potential complications. Splint therapy of lop ear is possible for infants.

  2. A Mini-Atlas of Ear-drum Pathology

    PubMed Central

    Hawke, Michael; Kwok, Peter

    1987-01-01

    The authors provide a number of ear-drum pictures and identify and discuss diseases affecting the external ear canal, the tympanic membrane and middle ear. They also deal with the removal of foreign bodies from the external canal, perforation of the tympanic membrane, and the use of an artificial ventilation tube. ImagesFig. 1Fig. 2Fig. 3Fig. 4Fig. 5Fig. 6Fig. 7Fig. 8Fig. 9Fig. 10Fig. 11Fig. 12Fig. 13Fig. 14Fig. 15Fig. 16Fig. 17Fig. 18 PMID:21263886

  3. [A case of angiosarcoma of the outer ear].

    PubMed

    Issara, Karima; Boughaleb, Zouhour; Tawfiq, Nezha; Bouchbika, Zineb; Benchakroun, Nadia; Jouhadi, Hassan; Sahraoui, Souha; Benider, Abdellatif

    2016-01-01

    Angiosarcoma of the ear is a very rare and locally aggressive malignant tumor. It accounts for 4-5% of cutaneous sarcomas and less than 1% of all sarcomas. Our study aims to describe and discuss diagnostic and therapeutic modalities of this malignancy. We report the clinical case of a 31 year old patient with bulging mass of the pavilion of the left ear. CT scan was in favor of tumor of the pavilion of the ear invading ipsilateral parotid. Histological diagnosis was in favor of angiosarcoma. The treatment consisted of complete surgical resection followed by adjuvant radiotherapy. She is in complete remission with a follow-up period of 1 year.

  4. Epizootic of ovine congenital malformations associated with Schmallenberg virus infection.

    PubMed

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

    2012-02-01

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

  5. Middle ear mucosal regeneration with three-dimensionally tissue-engineered autologous middle ear cell sheets in rabbit model.

    PubMed

    Yaguchi, Yuichiro; Murakami, Daisuke; Yamato, Masayuki; Hama, Takanori; Yamamoto, Kazuhisa; Kojima, Hiromi; Moriyama, Hiroshi; Okano, Teruo

    2016-03-01

    The likelihood of recurrent retraction and adhesion of newly formed tympanic membrane is high when middle ear mucosa is extensively lost during cholesteatoma and adhesive otitis media surgery. If rapid postoperative regeneration of the mucosa on the exposed bone surface can be achieved, prevention of recurrent eardrum adhesion and cholesteatoma formation, for which there has been no definitive treatment, can be expected. Suture-less transplantation of tissue-engineered mucosal cell sheets was examined immediately after the operation of otitis media surgery in order to quickly regenerate middle ear mucosa lost during surgery in a rabbit model. Transplantable middle ear mucosal cell sheets with a three-dimensional tissue architecture very similar to native middle ear mucosa were fabricated from middle ear mucosal tissue fragments obtained in an autologous manner from middle ear bulla on temperature-responsive culture surfaces. Immediately after the mucosa was resected from middle ear bone bulla inner cavity, mucosal cell sheets were grafted at the resected site. Both bone hyperplasia and granulation tissue formation were inhibited and early mucosal regeneration was observed in the cell sheet-grafted group, compared with the control group in which only mucosal removal was carried out and the bone surface exposed. This result indicates that tissue engineered mucosal cell sheets would be useful to minimize complications after the surgical operation on otitis media and future clinical application is expected.

  6. 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

  7. EARS : Repositioning data management near data acquisition.

    NASA Astrophysics Data System (ADS)

    Sinquin, Jean-Marc; Sorribas, Jordi; Diviacco, Paolo; Vandenberghe, Thomas; Munoz, Raquel; Garcia, Oscar

    2016-04-01

    The EU FP7 Projects Eurofleets and Eurofleets2 are an European wide alliance of marine research centers that aim to share their research vessels, to improve information sharing on planned, current and completed cruises, on details of ocean-going research vessels and specialized equipment, and to durably improve cost-effectiveness of cruises. Within this context logging of information on how, when and where anything happens on board of the vessel is crucial information for data users in a later stage. This forms a primordial step in the process of data quality control as it could assist in the understanding of anomalies and unexpected trends recorded in the acquired data sets. In this way completeness of the metadata is improved as it is recorded accurately at the origin of the measurement. The collection of this crucial information has been done in very different ways, using different procedures, formats and pieces of software in the context of the European Research Fleet. At the time that the Eurofleets project started, every institution and country had adopted different strategies and approaches, which complicated the task of users that need to log general purpose information and events on-board whenever they access a different platform loosing the opportunity to produce this valuable metadata on-board. Among the many goals the Eurofleets project has, a very important task is the development of an "event log software" called EARS (Eurofleets Automatic Reporting System) that enables scientists and operators to record what happens during a survey. EARS will allow users to fill, in a standardized way, the gap existing at the moment in metadata description that only very seldom links data with its history. Events generated automatically by acquisition instruments will also be handled, enhancing the granularity and precision of the event annotation. The adoption of a common procedure to log survey events and a common terminology to describe them is crucial to provide

  8. Experimental evidence against middle ear oxygen absorption.

    PubMed

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

    1985-04-01

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

  9. 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

  10. 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.

  11. 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.

  12. Multiple disseminated glomuvenous malformations: do we know enough?

    PubMed

    Solovan, C; Chiticariu, Elena; Beinsan, Doina; Zurac, Sabina; Baderca, Flavia

    2012-01-01

    Multiple glomuvenous malformations (GVMs), also known as glomangiomas, are uncommon entities with histological features of both glomus cells proliferation and venous malformation. A 14-year-old boy was admitted to our clinic with multiple dermal blue nodules, disseminated in different segments of the body. The patient's family history was positive for similar lesions; his mother and maternal grandmother had some asymptomatic blue nodules on their body. Histological examination showed a tumor composed of multiple caveronous vessels surrounded by glomus cells, positive for alpha smooth muscle actin, HHF35 (pan-actin), and h-caldesmon. This is a case of multiple GVMs, a rare disease caused by mutations in glomulin gene, with an autosomal dominant pattern of inheritance. The clinical and histopathological features are briefly discussed.

  13. 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.

  14. Linear accelerator radiosurgery for arteriovenous malformations: Updated literature review.

    PubMed

    Yahya, S; Heyes, G; Nightingale, P; Lamin, S; Chavda, S; Geh, I; Spooner, D; Cruickshank, G; Sanghera, P

    2017-04-01

    Arteriovenous malformations (AVMs) are the leading causing of intra-cerebral haemorrhage. Stereotactic radiosurgery (SRS) is an established treatment for arteriovenous malformations (AVM) and commonly delivered using Gamma Knife within dedicated radiosurgery units. Linear accelerator (LINAC) SRS is increasingly available however debate remains over whether it offers an equivalent outcome. The aim of this project is to evaluate the outcomes using LINAC SRS for AVMs used within a UK neurosciences unit and review the literature to aid decision making across various SRS platforms. Results have shown comparability across platforms and strongly supports that an adapted LINAC based SRS facility within a dynamic regional neuro-oncology department delivers similar outcomes (in terms of obliteration and toxicity) to any other dedicated radio-surgical platform. Locally available facilities can facilitate discussion between options however throughput will inevitably be lower than centrally based dedicated national radiosurgery units.

  15. 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

  16. Use of tramadol in early pregnancy and congenital malformation risk.

    PubMed

    Källén, Bengt; Reis, Margareta

    2015-12-01

    Only few studies exist regarding the risk of a teratogenic effect of tramadol when used in early pregnancy. Using the Swedish Medical Birth Register, women (deliveries in 1997-2013) who had reported the use of tramadol in early pregnancy were identified. Maternal characteristics and concomitant drug use were analyzed. Among 1,682,846 women (1,797,678 infants), 1751 (1776 infants) had used tramadol, 96 of the infants had a congenital malformation and 70 of them were relatively severe. The adjusted odds ratio for a relatively severe malformation was 1.33 (95% CI 1.05-1.70). The odds ratios for cardiovascular defects (1.56, 95% CI 1.04-2.29) and for pes equinovarus (3.63, 95% CI 1.61-6.89) were significantly increased. The study suggests a teratogenic effect of tramadol but the risk increase is moderate.

  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.

  18. 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.

  19. [The newborn infant with a suspected malformation syndrome].

    PubMed

    Memo, L

    2010-06-01

    Congenital malformations occur in 2-3% of live births, and often represent a special diagnostic and management challenge. Few clinical guidelines exist to assist the neonatologist in the approach to diagnosis and initial management of the malformed newborn. To provide optimal care for these children, one must employ a systematic approach to identify the likely pathogenic mechanism leading to the birth defects present. Determining how distinct anomalies relate to one another may lead to elucidation of a specific genetic etiology for the patient's condition. Genetic testing is increasingly available to allow for diagnostic confirmation. Using this systematic approach to a child with congenital anomalies permits accurate prognostic and recurrence risk counseling, informed management decisions, and the appropriate allocation of social support and medical resources.

  20. 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