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Sample records for congenital external auditory

  1. External auditory canal atresia of probable congenital origin in a dog.

    Science.gov (United States)

    Schmidt, K; Piaia, T; Bertolini, G; De Lorenzi, D

    2007-04-01

    A nine-month-old Labrador retriever was referred to the Clinica Veterinaria Privata San Marco because of frequent headshaking and downward turning of the right ear. Clinical examination revealed that there was no external acoustic meatus in the right ear. Computed tomography confirmed that the vertical part of the right auditory canal ended blindly, providing a diagnosis of external auditory canal atresia. Cytological examination and culture of fluid from the canal and the bulla revealed only aseptic cerumen; for this reason, it was assumed that the dog was probably affected by a congenital developmental deformity of the external auditory canal. Reconstructive surgery was performed using a "pull-through" technique. Four months after surgery the cosmetic and functional results were satisfactory.

  2. Congenital external auditory canal atresia and stenosis: temporal bone CT findings

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    Lee, Dong Hoon; Kim, Bum Soo; Jung, So Lyung; Kim, Young Joo; Chun, Ho Jong; Choi, Kyu Ho; Park, Shi Nae [College of Medicine, Catholic Univ. of Korea, Seoul (Korea, Republic of)

    2002-04-01

    To determine the computed tomographic (CT) findings of atresia and stenosis of the external auditory canal (EAC), and to describe associated abnormalities in surrounding structures. We retrospectively reviewed the axial and coronal CT images of the temporal bone in 15 patients (M:F=8:7;mean age, 15.8 years) with 16 cases of EAC atresia (unilateral n=11, bilateral n=1) and EAC stenosis (unilateral n=3). Associated abnormalities of the EAC, tympanic cavity, ossicles, mastoid air cells, eustachian tube, facial nerve course, mandibular condyle and condylar fossa, sigmoid sinus and jugular bulb, and the base of the middle cranial fossa were evaluated. Thirteen cases of bony EAC atresia (one bilateral), with an atretic bony plate, were noted, and one case of unilateral membranous atresia, in which a soft tissue the EAC. A unilateral lesion occurred more frequently on the right temporal bone (n=8, 73%). Associated abnormalities included a small tympanic cavity (n=8, 62%), decreased mastoid pneumatization (n=8, 62%), displacement of the mandibular condyle and the posterior wall of the condylar fossa (n=7, 54%), dilatation of the Eustachian tube (n=7, 54%), and inferior displacement of the temporal fossa base (n=8, 62%). Abnormalities of ossicles were noted in the malleolus (n=12, 92%), incus (n=10, 77%) and stapes (n=6, 46%). The course of the facial nerve was abnormal in four cases, and abnormality of the auditory canal was noted in one. Among three cases of EAC stenosis, ossicular aplasia was observed in one, and in another the location of the mandibular condyle and condylar fossa was abnormal. In the remaining case there was no associated abnormality. Atresia of the EAC is frequently accompanied by abnormalities of the middle ear cavity, ossicles, and adjacent structures other than the inner ear. For patients with atresia and stenosis of this canal, CT of the temporal bone is essentially helpful in evaluating these associated abnormalities.

  3. CT of the external auditory canal: Correlation with clinical otoscopy

    International Nuclear Information System (INIS)

    Shankar, L.; Hawke, M.; Leekam, R.N.

    1987-01-01

    CT is the modality of choice in the assessment of external auditory canal abnormalities. Disorders of the complex structures within the ear that may be difficult to define clinically are well visualized on high-resolution CT. This exhibit illustrates various external auditory canal abnormalities and correlates these with color illustrations from clinical otoscopy. Congenital lesions of the external auditory canal - microtia, temporo-bandibular joint herniation, and fistulas - and various acquired lesions - traumatic, inflammatory, and neoplastic - are reviewed in this exhibit

  4. Altered intrinsic connectivity of the auditory cortex in congenital amusia.

    Science.gov (United States)

    Leveque, Yohana; Fauvel, Baptiste; Groussard, Mathilde; Caclin, Anne; Albouy, Philippe; Platel, Hervé; Tillmann, Barbara

    2016-07-01

    Congenital amusia, a neurodevelopmental disorder of music perception and production, has been associated with abnormal anatomical and functional connectivity in a right frontotemporal pathway. To investigate whether spontaneous connectivity in brain networks involving the auditory cortex is altered in the amusic brain, we ran a seed-based connectivity analysis, contrasting at-rest functional MRI data of amusic and matched control participants. Our results reveal reduced frontotemporal connectivity in amusia during resting state, as well as an overconnectivity between the auditory cortex and the default mode network (DMN). The findings suggest that the auditory cortex is intrinsically more engaged toward internal processes and less available to external stimuli in amusics compared with controls. Beyond amusia, our findings provide new evidence for the link between cognitive deficits in pathology and abnormalities in the connectivity between sensory areas and the DMN at rest. Copyright © 2016 the American Physiological Society.

  5. External auditory canal carcinoma treatment

    International Nuclear Information System (INIS)

    Matsuda, Yoichi; Ueda, Yoshihisa; Kurita, Tomoyuki; Nakashima, Tadashi

    2010-01-01

    External auditory canal (EAC) carcinomas are relatively rare conditions lack on established treatment strategy. We analyzed a treatment modalities and outcome in 32 cases of EAC squamous cell carcinoma treated between 1980 and 2008. Subjects-17 men and 15 women ranging from 33 to 92 years old (average: 66) were divided by Arriaga's tumor staging into 12 T1, 5 T2, 6 T3, and 9 T4. Survival was calculated by the Kaplan-Meier method. Disease-specific 5-year survival was 100% for T1, T2, 44% for T3, and 33% for T4. In contrast to 100% 5-year survival for T1+T2 cancer, the 5-year survival for T3+T4 cancer was 37% with high recurrence due to positive surgical margins. The first 22 years of the 29 years surveyed, we performed surgery mainly, and irradiation or chemotherapy was selected for early disease or cases with positive surgical margins as postoperative therapy. During the 22-years, 5-year survival with T3+T4 cancer was 20%. After we started superselective intra-arterial (IA) rapid infusion chemotherapy combined with radiotherapy in 2003, we achieved negative surgical margins for advanced disease, and 5-year survival for T3+T4 cancer rise to 80%. (author)

  6. First branchial cleft sinus presenting with cholesteatoma and external auditory canal atresia.

    Science.gov (United States)

    Yalçin, Sinasi; Karlidağ, Turgut; Kaygusuz, Irfan; Demirbağ, Erhan

    2003-07-01

    First branchial cleft abnormalities are rare. They may involve the external auditory canal and middle ear. We describe a 6-year-old girl with congenital external auditory canal atresia, microtia, and cholesteatoma of mastoid and middle ear in addition to the first branchial cleft abnormalities. Clinical features of the patient are briefly described and the embryological relationship between first branchial cleft anomaly and external auditory canal atresia is discussed. The surgical management of these lesions may be performed, both the complete excision of the sinus and reconstructive otologic surgery.

  7. First Branchial Cleft Fistula Associated with External Auditory Canal Stenosis and Middle Ear Cholesteatoma

    Science.gov (United States)

    Abdollahi fakhim, Shahin; Naderpoor, Masoud; Mousaviagdas, Mehrnoosh

    2014-01-01

    Introduction: First branchial cleft anomalies manifest with duplication of the external auditory canal. Case Report: This report features a rare case of microtia and congenital middle ear and canal cholesteatoma with first branchial fistula. External auditory canal stenosis was complicated by middle ear and external canal cholesteatoma, but branchial fistula, opening in the zygomatic root and a sinus in the helical root, may explain this feature. A canal wall down mastoidectomy with canaloplasty and wide meatoplasty was performed. The branchial cleft was excised through parotidectomy and facial nerve dissection. Conclusion: It should be considered that canal stenosis in such cases can induce cholesteatoma formation in the auditory canal and middle ear. PMID:25320705

  8. First branchial cleft fistula associated with external auditory canal stenosis and middle ear cholesteatoma.

    Science.gov (United States)

    Abdollahi Fakhim, Shahin; Naderpoor, Masoud; Mousaviagdas, Mehrnoosh

    2014-10-01

    First branchial cleft anomalies manifest with duplication of the external auditory canal. This report features a rare case of microtia and congenital middle ear and canal cholesteatoma with first branchial fistula. External auditory canal stenosis was complicated by middle ear and external canal cholesteatoma, but branchial fistula, opening in the zygomatic root and a sinus in the helical root, may explain this feature. A canal wall down mastoidectomy with canaloplasty and wide meatoplasty was performed. The branchial cleft was excised through parotidectomy and facial nerve dissection. It should be considered that canal stenosis in such cases can induce cholesteatoma formation in the auditory canal and middle ear.

  9. CT analysis of 333 cases of congenital malformations of the external and middle ear

    International Nuclear Information System (INIS)

    Zou Xin; Li Qiang; Wang Zhenchang; Xian Junfang; Lan Baosen

    1997-01-01

    To analyze the different CT findings of congenital malformations of the external and middle ear, 333 cases including 404 ears with external and middle ear malformations diagnosed by high resolution CT (HRCT) were analysed according to the location and type of the malformation. In 404 ears, there were 364 ears with atresia of external auditory meatus, 40 ears with stenosis of external auditory meatus, 377 ears with malformation of the ossicles, 382 ears with stenosis of tympanum and 333 ears with anterior position of the mastoid segment of the facial canal. HRCT can show the location and type of external and middle ear malformation and provide valuable information for surgery

  10. Nodular Fasciitis of External Auditory Canal

    Directory of Open Access Journals (Sweden)

    Jihyun Ahn

    2016-09-01

    Full Text Available Nodular fasciitis is a pseudosarcomatous reactive process composed of fibroblasts and myofibroblasts, and it is most common in the upper extremities. Nodular fasciitis of the external auditory canal is rare. To the best of our knowledge, less than 20 cases have been reported to date. We present a case of nodular fasciitis arising in the cartilaginous part of the external auditory canal. A 19-year-old man complained of an auricular mass with pruritus. Computed tomography showed a 1.7 cm sized soft tissue mass in the right external auditory canal, and total excision was performed. Histologic examination revealed spindle or stellate cells proliferation in a fascicular and storiform pattern. Lymphoid cells and erythrocytes were intermixed with tumor cells. The stroma was myxoid to hyalinized with a few microcysts. The tumor cells were immunoreactive for smooth muscle actin, but not for desmin, caldesmon, CD34, S-100, anaplastic lymphoma kinase, and cytokeratin. The patient has been doing well during the 1 year follow-up period.

  11. Hearing after congenital deafness: central auditory plasticity and sensory deprivation.

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    Kral, A; Hartmann, R; Tillein, J; Heid, S; Klinke, R

    2002-08-01

    The congenitally deaf cat suffers from a degeneration of the inner ear. The organ of Corti bears no hair cells, yet the auditory afferents are preserved. Since these animals have no auditory experience, they were used as a model for congenital deafness. Kittens were equipped with a cochlear implant at different ages and electro-stimulated over a period of 2.0-5.5 months using a monopolar single-channel compressed analogue stimulation strategy (VIENNA-type signal processor). Following a period of auditory experience, we investigated cortical field potentials in response to electrical biphasic pulses applied by means of the cochlear implant. In comparison to naive unstimulated deaf cats and normal hearing cats, the chronically stimulated animals showed larger cortical regions producing middle-latency responses at or above 300 microV amplitude at the contralateral as well as the ipsilateral auditory cortex. The cortex ipsilateral to the chronically stimulated ear did not show any signs of reduced responsiveness when stimulating the 'untrained' ear through a second cochlear implant inserted in the final experiment. With comparable duration of auditory training, the activated cortical area was substantially smaller if implantation had been performed at an older age of 5-6 months. The data emphasize that young sensory systems in cats have a higher capacity for plasticity than older ones and that there is a sensitive period for the cat's auditory system.

  12. Early Visual Deprivation Severely Compromises the Auditory Sense of Space in Congenitally Blind Children

    Science.gov (United States)

    Vercillo, Tiziana; Burr, David; Gori, Monica

    2016-01-01

    A recent study has shown that congenitally blind adults, who have never had visual experience, are impaired on an auditory spatial bisection task (Gori, Sandini, Martinoli, & Burr, 2014). In this study we investigated how thresholds for auditory spatial bisection and auditory discrimination develop with age in sighted and congenitally blind…

  13. Congenital malformations of the external and middle ear: high-resolution CT findings of surgical import

    International Nuclear Information System (INIS)

    Swartz, J.D.; Faerber, E.N.

    1985-01-01

    The external auditory canal, middle ear, and bulk of the ossicular chain develop from the first branchial groove, first and second branchial arches, and first pharyngeal pouch. Embryologic development of these structures is complex and only rarely are two anomalies identical. This study includes 11 cases of unilateral external auditory canal atresia and two cases of bilateral atresia. Eight cases (four bilateral) of isolated congenital ossicular anomalies are also included. Emphasis is placed on findings of surgical import. All patients were studied with computed tomography only, because it was believed that the bony and soft-tissue detail achieved is superior to that with conventional multidirectional tomography

  14. Cancer of the external auditory canal

    DEFF Research Database (Denmark)

    Nyrop, Mette; Grøntved, Aksel

    2002-01-01

    OBJECTIVE: To evaluate the outcome of surgery for cancer of the external auditory canal and relate this to the Pittsburgh staging system used both on squamous cell carcinoma and non-squamous cell carcinoma. DESIGN: Retrospective case series of all patients who had surgery between 1979 and 2000....... Median follow-up was 47 months (range, 2-148 months). Data on age, sex, symptoms, TNM status, histopathological diagnosis, surgery, adjunctive therapy, sequelae, recurrence, and status at follow-up were obtained. SETTING: An ear, nose, and throat department in an ambulatory and hospitalized care center....... PATIENTS: Ten women and 10 men with previously untreated primary cancer. Median age at diagnosis was 67 years (range, 31-87 years). Survival data included 18 patients with at least 2 years of follow-up or recurrence. INTERVENTION: Local canal resection or partial temporal bone resection. MAIN OUTCOME...

  15. First Branchial Cleft Fistula Associated with External Auditory Canal Stenosis and Middle Ear Cholesteatoma

    Directory of Open Access Journals (Sweden)

    shahin abdollahi fakhim

    2014-10-01

    Full Text Available Introduction: First branchial cleft anomalies manifest with duplication of the external auditory canal.   Case Report: This report features a rare case of microtia and congenital middle ear and canal cholesteatoma with first branchial fistula. External auditory canal stenosis was complicated by middle ear and external canal cholesteatoma, but branchial fistula, opening in the zygomatic root and a sinus in the helical root, may explain this feature. A canal wall down mastoidectomy with canaloplasty and wide meatoplasty was performed. The branchial cleft was excised through parotidectomy and facial nerve dissection.   Conclusion:  It should be considered that canal stenosis in such cases can induce cholesteatoma formation in the auditory canal and middle ear.

  16. Masses and disease entities of the external auditory canal: Radiological and clinical correlation

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    White, R.D., E-mail: richard.white3@nhs.net [Department of Clinical Radiology, Ninewells Hospital and Medical School, Dundee (United Kingdom); Ananthakrishnan, G. [Department of Clinical Radiology, Ninewells Hospital and Medical School, Dundee (United Kingdom); McKean, S.A. [Department of Otolaryngology, Ninewells Hospital and Medical School, Dundee (United Kingdom); Brunton, J.N. [Department of Clinical Radiology, Ninewells Hospital and Medical School, Dundee (United Kingdom); Hussain, S.S.M. [Department of Otolaryngology, Ninewells Hospital and Medical School, Dundee (United Kingdom); Sudarshan, T.A. [Department of Clinical Radiology, Ninewells Hospital and Medical School, Dundee (United Kingdom)

    2012-02-15

    A wide spectrum of disease entities can affect the external auditory canal (EAC). This review describes the normal anatomy of the EAC. Congenital abnormalities, infections, neoplasms, and miscellaneous conditions, such as cholesteatoma and acquired stenosis, are shown with reference to clinical relevance and management. Cases have been histologically confirmed, where relevant. The EAC is frequently imaged - for example, on cross-sectional imaging of the brain - and this review should stimulate radiologists to include it as an important area for review.

  17. Preoperative diagnosis and surgical strategy in congenital auditory ossicular malformation of 26 ears

    International Nuclear Information System (INIS)

    Kanazawa, Yuji; Naito, Yasushi; Shinohara, Shogo; Fujiwara, Keizo; Kikuchi, Masahiro; Yamazaki, Hiroshi; Kurihara, Risa; Kishimoto, Ippei

    2012-01-01

    We retrospectively analyzed 26 ears of 21 subjects having auditory ossicular malformation and who had undergone auditory reconstruction between April 2004 and December 2010 at our clinic. We checked preoperative condition, pathological classification, surgical procedure, and hearing improvement. We could predict pathological conditions precisely from preoperative computed tomography (CT), including incudostapedial disconnection (9/12, 75%) and malleus and/or incus fixation (7/12, 58%), which tended to be present in external ear malformation, and stapes footplate fixation (0/12, 0%). We could not, however, predict complex malformation (0/8, 0%). Overall success was 90% (18/20) in the 20 ears observed for at least 1 year. In the 2 ears without improved hearing, the first had congenital cholesteatoma and no stapes superstructure, was treated with type IV tympanoplasty. The second had malleus, incus, and stapes fixation and discontinuity between the incus and stapes, and was treated with type III tympanoplasty and stapes mobilization. Preoperative diagnosis is difficult in mixed congenital auditory ossicular malformation, especially stapes footplate fixation, possibly requiring unexpected procedures, with a poor hearing outcome. Preoperative status must thus be evaluated precisely using hearing, tympanometry, acoustic reflex test, and CT. Temporal bone CT and external ear findings are useful in diagnosing middle-ear malformation. Subjects' informed consent should also be obtained due to the possible need for changing procedure based on findings during surgery. (author)

  18. Interactions of cognitive and auditory abilities in congenitally blind individuals.

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    Rokem, Ariel; Ahissar, Merav

    2009-02-01

    Congenitally blind individuals have been found to show superior performance in perceptual and memory tasks. In the present study, we asked whether superior stimulus encoding could account for performance in memory tasks. We characterized the performance of a group of congenitally blind individuals on a series of auditory, memory and executive cognitive tasks and compared their performance to that of sighted controls matched for age, education and musical training. As expected, we found superior verbal spans among congenitally blind individuals. Moreover, we found superior speech perception, measured by resilience to noise, and superior auditory frequency discrimination. However, when memory span was measured under conditions of equivalent speech perception, by adjusting the signal to noise ratio for each individual to the same level of perceptual difficulty (80% correct), the advantage in memory span was completely eliminated. Moreover, blind individuals did not possess any advantage in cognitive executive functions, such as manipulation of items in memory and math abilities. We propose that the short-term memory advantage of blind individuals results from better stimulus encoding, rather than from superiority at subsequent processing stages.

  19. Congenital Deafness Reduces, But Does Not Eliminate Auditory Responsiveness in Cat Extrastriate Visual Cortex.

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    Land, Rüdiger; Radecke, Jan-Ole; Kral, Andrej

    2018-04-01

    Congenital deafness not only affects the development of the auditory cortex, but also the interrelation between the visual and auditory system. For example, congenital deafness leads to visual modulation of the deaf auditory cortex in the form of cross-modal plasticity. Here we asked, whether congenital deafness additionally affects auditory modulation in the visual cortex. We demonstrate that auditory activity, which is normally present in the lateral suprasylvian visual areas in normal hearing cats, can also be elicited by electrical activation of the auditory system with cochlear implants. We then show that in adult congenitally deaf cats auditory activity in this region was reduced when tested with cochlear implant stimulation. However, the change in this area was small and auditory activity was not completely abolished despite years of congenital deafness. The results document that congenital deafness leads not only to changes in the auditory cortex but also affects auditory modulation of visual areas. However, the results further show a persistence of fundamental cortical sensory functional organization despite congenital deafness. Copyright © 2018 The Authors. Published by Elsevier Ltd.. All rights reserved.

  20. Development of Brainstem-Evoked Responses in Congenital Auditory Deprivation

    Directory of Open Access Journals (Sweden)

    J. Tillein

    2012-01-01

    Full Text Available To compare the development of the auditory system in hearing and completely acoustically deprived animals, naive congenitally deaf white cats (CDCs and hearing controls (HCs were investigated at different developmental stages from birth till adulthood. The CDCs had no hearing experience before the acute experiment. In both groups of animals, responses to cochlear implant stimulation were acutely assessed. Electrically evoked auditory brainstem responses (E-ABRs were recorded with monopolar stimulation at different current levels. CDCs demonstrated extensive development of E-ABRs, from first signs of responses at postnatal (p.n. day 3 through appearance of all waves of brainstem response at day 8 p.n. to mature responses around day 90 p.n.. Wave I of E-ABRs could not be distinguished from the artifact in majority of CDCs, whereas in HCs, it was clearly separated from the stimulus artifact. Waves II, III, and IV demonstrated higher thresholds in CDCs, whereas this difference was not found for wave V. Amplitudes of wave III were significantly higher in HCs, whereas wave V amplitudes were significantly higher in CDCs. No differences in latencies were observed between the animal groups. These data demonstrate significant postnatal subcortical development in absence of hearing, and also divergent effects of deafness on early waves II–IV and wave V of the E-ABR.

  1. CT and MR imaging of congenital abnormalities of the inner ear and internal auditory canal

    International Nuclear Information System (INIS)

    Casselman, Jan W.; Offeciers, Erwin F.; Foer, Bert de; Govaerts, Paul; Kuhweide, Rudy; Somers, Thomas

    2001-01-01

    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

  2. Decoding Visual Location From Neural Patterns in the Auditory Cortex of the Congenitally Deaf

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    Almeida, Jorge; He, Dongjun; Chen, Quanjing; Mahon, Bradford Z.; Zhang, Fan; Gonçalves, Óscar F.; Fang, Fang; Bi, Yanchao

    2016-01-01

    Sensory cortices of individuals who are congenitally deprived of a sense can exhibit considerable plasticity and be recruited to process information from the senses that remain intact. Here, we explored whether the auditory cortex of congenitally deaf individuals represents visual field location of a stimulus—a dimension that is represented in early visual areas. We used functional MRI to measure neural activity in auditory and visual cortices of congenitally deaf and hearing humans while they observed stimuli typically used for mapping visual field preferences in visual cortex. We found that the location of a visual stimulus can be successfully decoded from the patterns of neural activity in auditory cortex of congenitally deaf but not hearing individuals. This is particularly true for locations within the horizontal plane and within peripheral vision. These data show that the representations stored within neuroplastically changed auditory cortex can align with dimensions that are typically represented in visual cortex. PMID:26423461

  3. Epidermoid carcinoma of the external auditory canal

    International Nuclear Information System (INIS)

    Alfonso Rodriguez, Javier; Llerena Suarez, Jose Angel; Campis Cruz, Hipolito Amable

    2009-01-01

    A 43 years-old, urban, male patient assists the consultation of Otolaryngology with a long evolving otorrhea (around two years), receiving several treatments against the external otitis he suffered. With those antecedents and what we found when examining him, we decided to take a sample (biopsy) of the area, diagnosing an epidermoid carcinoma of the left external canal, an infrequent pathology in our settings

  4. The osseous external auditory canal : Surgery, shape, and sound

    NARCIS (Netherlands)

    van Spronsen, E.

    2018-01-01

    This thesis describes three aspects of the osseous external auditory canal (OEAC) which are related to each other: Surgery of the OEAC, the shape of the OEAC and the perceived sound quality due to the resonance function of the OEAC. Several aspects of the surgical intervention (Canalplasty) in

  5. Pneumocystis jirovecii infection of the external auditory canal

    African Journals Online (AJOL)

    nonspecific symptoms of otorrhoea, otalgia, hearing loss, vertigo and tinnitus. Clinical findings include a mass in the external auditory canal and occasional otitis media. Destruction of the ossicles and sclerosis of the mastoid air cells, extensive bony erosion, and extension into the middle cranial fossa are rare complications.

  6. External auditory canal leech: a rare case report of paediatric ...

    African Journals Online (AJOL)

    Leeches are blood sucking organism feed on human blood. While human bites are common, they rarely cause human internal infestation. We describe a rare case of a parasitic leech infestation of the External Auditory Canal (EAC). A two month old child presented to the Emergency department with a seven day history of ...

  7. Congenital malformations of the external and middle ear

    International Nuclear Information System (INIS)

    Koesling, S.; Omenzetter, M.; Bartel-Friedrich, S.

    2009-01-01

    With the focus on imaging, this paper gives a summarized view of the present knowledge on fields, which are necessary to know for a profound understanding of congenital malformations of the external and middle ear. Typical and less typical combinations of malformed parts of the ear can be derived from the embryogenesis. Clinical signs and audiometric findings lead to diagnosis in congenital aural atresia. Isolated middle ear malformations can be clinically mixed up especially with otosclerosis and tympanosclerosis. Imaging is needed for exact morphological information. In malformations of the external and middle ear, CT is the imaging modality of choice. Requirements on CT-technique as well as radiological findings including classification and pre-surgical rating are described. Morphological CT-correlates of congenital malformations and their differential diagnoses are enlisted and illustrated. The impact of CT-results on therapy is explained and actual therapeutic concepts are briefly presented

  8. Carcinoma of the external auditory canal and middle ear

    International Nuclear Information System (INIS)

    Pfreundner, Leo; Schwager, Konrad; Willner, Jochen; Baier, Kurt; Bratengeier, Klaus; Brunner, Franz Xaver; Flentje, Michael

    1999-01-01

    Purpose: To evaluate therapeutic modalities used at our institutions regarding local control, disease-free survival and actuarial survival in carcinoma of the external auditory canal and middle ear, in an attempt to provide guidelines for therapy. Methods and Materials: A series of 27 patients with carcinoma of the external auditory canal and middle ear treated between 1978 and 1997 in our institutions were analyzed with particular reference to tumor size and its relation to surrounding tissues, patterns of neck node involvement, surgical procedures, and radiation techniques employed. Clinical endpoints were freedom from local failure, overall survival, and disease-free survival. The median follow-up was 2.7 years (range 0.1-17.9 years). Results: Treatment by surgery and radiotherapy resulted in an overall 5-year survival rate of 61%. According to the Pittsburgh classification, the actuarial 5-year survival rate for early disease (T1 and T2 tumors) was 86%, for T3 tumors 50%, and T4 stages 41%. Patients with tumors limited to the external auditory canal had a 5-year survival rate of 100%, patients with tumor invasion of the temporal bone 63%, and patients with tumor infiltration beyond the temporal bone 38%. The rate of freedom from local recurrence was 50% at 5 years. Unresectability by dural and cerebral infiltration, and treatment factors such as complete resection or resection with tumor beyond surgical margins are of prognostic relevance. All patients with dural invasion died within 2.2 years. The actuarial 5-year survival rate of patients with complete tumor resection was 100%, but 66% in patients with tumor beyond surgical margins. 192 Iridium high-dose-rate (HDR) afterloading brachytherapy based on three-dimensional computed tomography (3D CT)-treatment planning was an effective tool in management of local recurrences following surgery and a full course of external beam radiotherapy. Conclusion: Surgical resection followed by radiotherapy adapted to stage

  9. Management of Acquired Atresia of the External Auditory Canal.

    Science.gov (United States)

    Bajin, Münir Demir; Yılmaz, Taner; Günaydın, Rıza Önder; Kuşçu, Oğuz; Sözen, Tevfik; Jafarov, Shamkal

    2015-08-01

    The aim was to evaluate surgical techniques and their relationship to postoperative success rate and hearing outcomes in acquired atresia of the external auditory canal. In this article, 24 patients with acquired atresia of the external auditory canal were retrospectively evaluated regarding their canal status, hearing, and postoperative success. Acquired stenosis occurs more commonly in males with a male: female ratio of 2-3:1; it seems to be a disorder affecting young adults. Previous ear surgery (13 patients, 54.2%) and external ear trauma (11 patients, 45.8%) were the main etiological factors of acquired ear canal stenosis. Mastoidectomy (12/13) and traffic accidents (8/11) comprise the majority of these etiological factors. Endaural incision is performed in 79.2% and postauricular incision for 20.8% of cases during the operation. As types of surgical approach, transcanal (70.8%), transmastoid (20.8%), and combined (8.4%) approaches are chosen. The atretic plate is generally located at the bony-cartilaginous junction (37.5%) and in the cartilaginous canal (33.3%); the bony canal is involved in a few cases only. Preserved healthy canal skin, split- or full-thickness skin grafts, or pre- or postauricular skin flaps are used to line the ear canal, but preserved healthy canal skin is preferred. The results of surgery are generally satisfactory, and complications are few if surgical principles are followed.

  10. Primary Synovial Sarcoma of External Auditory Canal: A Case Report.

    Science.gov (United States)

    Devi, Aarani; Jayakumar, Krishnannair L L

    2017-07-20

    Synovial sarcoma is a rare malignant tumor of mesenchymal origin. Primary synovial sarcoma of the ear is extremely rare and to date only two cases have been published in English medical literature. Though the tumor is reported to have an aggressive nature, early diagnosis and treatment may improve the outcome. Here, we report a rare case of synovial sarcoma of the external auditory canal in an 18-year-old male who was managed by chemotherapy and referred for palliation due to tumor progression.

  11. Impairments in musical abilities reflected in the auditory brainstem: evidence from congenital amusia.

    Science.gov (United States)

    Lehmann, Alexandre; Skoe, Erika; Moreau, Patricia; Peretz, Isabelle; Kraus, Nina

    2015-07-01

    Congenital amusia is a neurogenetic condition, characterized by a deficit in music perception and production, not explained by hearing loss, brain damage or lack of exposure to music. Despite inferior musical performance, amusics exhibit normal auditory cortical responses, with abnormal neural correlates suggested to lie beyond auditory cortices. Here we show, using auditory brainstem responses to complex sounds in humans, that fine-grained automatic processing of sounds is impoverished in amusia. Compared with matched non-musician controls, spectral amplitude was decreased in amusics for higher harmonic components of the auditory brainstem response. We also found a delayed response to the early transient aspects of the auditory stimulus in amusics. Neural measures of spectral amplitude and response timing correlated with participants' behavioral assessments of music processing. We demonstrate, for the first time, that amusia affects how complex acoustic signals are processed in the auditory brainstem. This neural signature of amusia mirrors what is observed in musicians, such that the aspects of the auditory brainstem responses that are enhanced in musicians are degraded in amusics. By showing that gradients of music abilities are reflected in the auditory brainstem, our findings have implications not only for current models of amusia but also for auditory functioning in general. © 2015 Federation of European Neuroscience Societies and John Wiley & Sons Ltd.

  12. Changes in auditory perceptions and cortex resulting from hearing recovery after extended congenital unilateral hearing loss

    Directory of Open Access Journals (Sweden)

    Jill B Firszt

    2013-12-01

    Full Text Available Monaural hearing induces auditory system reorganization. Imbalanced input also degrades time-intensity cues for sound localization and signal segregation for listening in noise. While there have been studies of bilateral auditory deprivation and later hearing restoration (e.g. cochlear implants, less is known about unilateral auditory deprivation and subsequent hearing improvement. We investigated effects of long-term congenital unilateral hearing loss on localization, speech understanding, and cortical organization following hearing recovery. Hearing in the congenitally affected ear of a 41 year old female improved significantly after stapedotomy and reconstruction. Pre-operative hearing threshold levels showed unilateral, mixed, moderately-severe to profound hearing loss. The contralateral ear had hearing threshold levels within normal limits. Testing was completed prior to, and three and nine months after surgery. Measurements were of sound localization with intensity-roved stimuli and speech recognition in various noise conditions. We also evoked magnetic resonance signals with monaural stimulation to the unaffected ear. Activation magnitudes were determined in core, belt, and parabelt auditory cortex regions via an interrupted single event design. Hearing improvement following 40 years of congenital unilateral hearing loss resulted in substantially improved sound localization and speech recognition in noise. Auditory cortex also reorganized. Contralateral auditory cortex responses were increased after hearing recovery and the extent of activated cortex was bilateral, including a greater portion of the posterior superior temporal plane. Thus, prolonged predominant monaural stimulation did not prevent auditory system changes consequent to restored binaural hearing. Results support future research of unilateral auditory deprivation effects and plasticity, with consideration for length of deprivation, age at hearing correction, degree and type

  13. Recruitment of the auditory cortex in congenitally deaf cats by long-term cochlear electrostimulation.

    Science.gov (United States)

    Klinke, R; Kral, A; Heid, S; Tillein, J; Hartmann, R

    1999-09-10

    In congenitally deaf cats, the central auditory system is deprived of acoustic input because of degeneration of the organ of Corti before the onset of hearing. Primary auditory afferents survive and can be stimulated electrically. By means of an intracochlear implant and an accompanying sound processor, congenitally deaf kittens were exposed to sounds and conditioned to respond to tones. After months of exposure to meaningful stimuli, the cortical activity in chronically implanted cats produced field potentials of higher amplitudes, expanded in area, developed long latency responses indicative of intracortical information processing, and showed more synaptic efficacy than in naïve, unstimulated deaf cats. The activity established by auditory experience resembles activity in hearing animals.

  14. A comparative study of simple auditory reaction time in blind (congenitally) and sighted subjects.

    Science.gov (United States)

    Gandhi, Pritesh Hariprasad; Gokhale, Pradnya A; Mehta, H B; Shah, C J

    2013-07-01

    Reaction time is the time interval between the application of a stimulus and the appearance of appropriate voluntary response by a subject. It involves stimulus processing, decision making, and response programming. Reaction time study has been popular due to their implication in sports physiology. Reaction time has been widely studied as its practical implications may be of great consequence e.g., a slower than normal reaction time while driving can have grave results. To study simple auditory reaction time in congenitally blind subjects and in age sex matched sighted subjects. To compare the simple auditory reaction time between congenitally blind subjects and healthy control subjects. STUDY HAD BEEN CARRIED OUT IN TWO GROUPS: The 1(st) of 50 congenitally blind subjects and 2(nd) group comprises of 50 healthy controls. It was carried out on Multiple Choice Reaction Time Apparatus, Inco Ambala Ltd. (Accuracy±0.001 s) in a sitting position at Government Medical College and Hospital, Bhavnagar and at a Blind School, PNR campus, Bhavnagar, Gujarat, India. Simple auditory reaction time response with four different type of sound (horn, bell, ring, and whistle) was recorded in both groups. According to our study, there is no significant different in reaction time between congenital blind and normal healthy persons. Blind individuals commonly utilize tactual and auditory cues for information and orientation and they reliance on touch and audition, together with more practice in using these modalities to guide behavior, is often reflected in better performance of blind relative to sighted participants in tactile or auditory discrimination tasks, but there is not any difference in reaction time between congenitally blind and sighted people.

  15. Early auditory processing in area V5/MT+ of the congenitally blind brain.

    Science.gov (United States)

    Watkins, Kate E; Shakespeare, Timothy J; O'Donoghue, M Clare; Alexander, Iona; Ragge, Nicola; Cowey, Alan; Bridge, Holly

    2013-11-13

    Previous imaging studies of congenital blindness have studied individuals with heterogeneous causes of blindness, which may influence the nature and extent of cross-modal plasticity. Here, we scanned a homogeneous group of blind people with bilateral congenital anophthalmia, a condition in which both eyes fail to develop, and, as a result, the visual pathway is not stimulated by either light or retinal waves. This model of congenital blindness presents an opportunity to investigate the effects of very early visual deafferentation on the functional organization of the brain. In anophthalmic animals, the occipital cortex receives direct subcortical auditory input. We hypothesized that this pattern of subcortical reorganization ought to result in a topographic mapping of auditory frequency information in the occipital cortex of anophthalmic people. Using functional MRI, we examined auditory-evoked activity to pure tones of high, medium, and low frequencies. Activity in the superior temporal cortex was significantly reduced in anophthalmic compared with sighted participants. In the occipital cortex, a region corresponding to the cytoarchitectural area V5/MT+ was activated in the anophthalmic participants but not in sighted controls. Whereas previous studies in the blind indicate that this cortical area is activated to auditory motion, our data show it is also active for trains of pure tone stimuli and in some anophthalmic participants shows a topographic mapping (tonotopy). Therefore, this region appears to be performing early sensory processing, possibly served by direct subcortical input from the pulvinar to V5/MT+.

  16. Dislocation of the incus into the external auditory canal after mountain-biking accident.

    Science.gov (United States)

    Saito, T; Kono, Y; Fukuoka, Y; Yamamoto, H; Saito, H

    2001-01-01

    We report a rare case of incus dislocation to the external auditory canal after a mountain-biking accident. Otoscopy showed ossicular protrusion in the upper part of the left external auditory canal. CT indicated the disappearance of the incus, and an incus-like bone was found in the left external auditory canal. There was another bony and board-like structure in the attic. During the surgery, a square-shaped bony plate (1 x 1 cm) was found in the attic. It was determined that the bony plate had fallen from the tegmen of the attic. The fracture line in the posterosuperior auditory canal extending to the fossa incudis was identified. According to these findings, it was considered that the incus was pushed into the external auditory canal by the impact of skull injury through the fractured posterosuperior auditory canal, which opened widely enough for incus dislocation. Copyright 2001 S. Karger AG, Basel

  17. Malignant transformation from benign papillomatosis of the external auditory canal.

    Science.gov (United States)

    Miah, Mohammed S; Crawford, Mairi; White, Sharon J; Hussain, Syed Shah Musheer

    2012-06-01

    Report a case of malignant transformation of benign ear canal papillomatosis to malignant squamous cell carcinoma (SCC) of the temporal bone. A 73-year-old with papillomata involving the posterior and inferior walls of the right external auditory canal (EAC), which subsequently transformed into SCC. Radical mastoidectomy and excision of the tumor and then radical radiotherapy. Loco-regional disease control. Recovery of facial nerve function. Approximately 20 months post-treatment, the patient remains disease free. No recovery of facial nerve function. Malignant transformation of a benign EAC papilloma to SCC of the temporal bone has not been reported previously. The association of human papillomavirus with temporal bone SCC has been reported in small number of studies with human papillomavirus subtypes 16 and 18 isolated in a high proportion of cases. With the increased availability in genotyping, the question over whether there should be further genetic analysis of benign lesions to assess their susceptibility to malignant transformation has merit.

  18. No auditory experience, no tinnitus: Lessons from subjects with congenital- and acquired single-sided deafness.

    Science.gov (United States)

    Lee, Sang-Yeon; Nam, Dong Woo; Koo, Ja-Won; De Ridder, Dirk; Vanneste, Sven; Song, Jae-Jin

    2017-10-01

    Recent studies have adopted the Bayesian brain model to explain the generation of tinnitus in subjects with auditory deafferentation. That is, as the human brain works in a Bayesian manner to reduce environmental uncertainty, missing auditory information due to hearing loss may cause auditory phantom percepts, i.e., tinnitus. This type of deafferentation-induced auditory phantom percept should be preceded by auditory experience because the fill-in phenomenon, namely tinnitus, is based upon auditory prediction and the resultant prediction error. For example, a recent animal study observed the absence of tinnitus in cats with congenital single-sided deafness (SSD; Eggermont and Kral, Hear Res 2016). However, no human studies have investigated the presence and characteristics of tinnitus in subjects with congenital SSD. Thus, the present study sought to reveal differences in the generation of tinnitus between subjects with congenital SSD and those with acquired SSD to evaluate the replicability of previous animal studies. This study enrolled 20 subjects with congenital SSD and 44 subjects with acquired SSD and examined the presence and characteristics of tinnitus in the groups. None of the 20 subjects with congenital SSD perceived tinnitus on the affected side, whereas 30 of 44 subjects with acquired SSD experienced tinnitus on the affected side. Additionally, there were significant positive correlations between tinnitus characteristics and the audiometric characteristics of the SSD. In accordance with the findings of the recent animal study, tinnitus was absent in subjects with congenital SSD, but relatively frequent in subjects with acquired SSD, which suggests that the development of tinnitus should be preceded by auditory experience. In other words, subjects with profound congenital peripheral deafferentation do not develop auditory phantom percepts because no auditory predictions are available from the Bayesian brain. Copyright © 2017 Elsevier B.V. All rights

  19. Congenital External Carotid-External Jugular Arteriovenous Fistula: Diagnosis With Contrast-Enhanced Computed Tomography

    International Nuclear Information System (INIS)

    Faghihi Langroudi, Taraneh; Arjmand Shabestari, Abbas; Pourghorban, Ramin; Khalili Pouya, Ensi

    2015-01-01

    Arteriovenous fistula (AVF) between the external carotid artery and external jugular vein is extremely rare, with only few cases reported in the literature so far. Most of these AVFs have been either iatrogenic or secondary to previous trauma. Herein, we report a 42-year-old woman with congenital AVF between the external carotid artery and external jugular vein, presenting with palpitation and dyspnea. The patient was suffering from mitral and tricuspid regurgitation. On physical examination, a thrill on the left side of the neck and an audible bruit over the left mandibular angle were detected. The possibility of abnormal AVF was considered and it was confirmed on contrast-enhanced computed tomography (CT), inferring that this modality is not only fast and non-invasive, but also accurate in detecting vascular abnormalities

  20. Duplication of the External Auditory Canal: Two Cases and a Review of the Literature

    Science.gov (United States)

    Goudakos, John K.; Blioskas, Sarantis; Psillas, George; Vital, Victor; Markou, Konstantinos

    2012-01-01

    The objective of the present paper is to describe the clinical presentation, diagnostic process, surgical treatment, and outcome of 2 patients with first branchial cleft anomaly. The first case was an 8-year-old girl presented with an elastic lesion located in the left infra-auricular area, in close relation with the lobule, duplicating the external auditory canal. The magnetic resonance imaging revealed a lesion, appearing as a rather well-circumscribed mass within the left parotid gland and duplicating the ear canal. A superficial parotidectomy was subsequently performed, with total excision of the cyst. The second patient was a 15-year-old girl presented with a congenital fistula of the right lateral neck. At superficial parotidectomy, a total excision of the fistula was performed. During the operation the tract was recorded to lay between the branches of the facial nerve, extending with a blind ending canal parallel to the external acoustic meatus. Conclusively, first branchial cleft anomalies are rare malformations with cervical, parotid, or auricular clinical manifestations. Diagnosis of first branchial cleft lesions is achieved mainly through careful physical examination. Complete surgical excision with wide exposure of the lesion is essential in order to achieve permanent cure and avoid recurrence. PMID:23213587

  1. Duplication of the External Auditory Canal: Two Cases and a Review of the Literature

    Directory of Open Access Journals (Sweden)

    John K. Goudakos

    2012-01-01

    Full Text Available The objective of the present paper is to describe the clinical presentation, diagnostic process, surgical treatment, and outcome of 2 patients with first branchial cleft anomaly. The first case was an 8-year-old girl presented with an elastic lesion located in the left infra-auricular area, in close relation with the lobule, duplicating the external auditory canal. The magnetic resonance imaging revealed a lesion, appearing as a rather well-circumscribed mass within the left parotid gland and duplicating the ear canal. A superficial parotidectomy was subsequently performed, with total excision of the cyst. The second patient was a 15-year-old girl presented with a congenital fistula of the right lateral neck. At superficial parotidectomy, a total excision of the fistula was performed. During the operation the tract was recorded to lay between the branches of the facial nerve, extending with a blind ending canal parallel to the external acoustic meatus. Conclusively, first branchial cleft anomalies are rare malformations with cervical, parotid, or auricular clinical manifestations. Diagnosis of first branchial cleft lesions is achieved mainly through careful physical examination. Complete surgical excision with wide exposure of the lesion is essential in order to achieve permanent cure and avoid recurrence.

  2. Carcinoma of the middle ear and external auditory canal

    International Nuclear Information System (INIS)

    Hahn, S.S.; Kim, J.A.; Goodchild, N.; Constable, W.C.

    1983-01-01

    Thirty-one patients with malignant tumors of the middle ear and external auditory canal (EAC) were observed at the University of Virginia Hospital from 1956 through 1980. Of 27 patients with carcinoma, 21 had squamous cell carcinoma, 4 had basal cell carcinoma and 2 had adenoid cystic carcinoma. The 27 patients with carcinoma are reviewed with regard to clinical presentation, treatment modality, results and complications. The majority (67%) of patients had a history of chronic ear drainage, 22% had a previous mastoidectomy or polypectomy and 7% had an associated cholesteatoma. Eighty percent of patients with carcinoma limited to EAC were alive and well at 5 years, compared to 43% of patients with involvement of the middle ear. Fifty-six percent of patients without invasion of the petrous bone were alive at 5 years compared to only 20% of patients with petrous bone involvement. The data strongly suggest that survival depends on the extent of disease. The corrected disease free 5 year survival rates were 14% for patients who had surgery alone and 50% for those who had surgery and radiotherapy. Of the three patients with advanced disease who received radiotherapy alone, none survived five years

  3. Transcanal endoscopic resection for leiomyoma of the external auditory canal and a review of the literature.

    Science.gov (United States)

    Ikeda, Ryoukichi; Tateda, Masaru; Okoshi, Akira; Morita, Shinkichi; Suzuki, Hiroyoshi; Hashimoto, Sho

    2016-02-01

    Leiomyoma usually originates from the uterus and alimentary tract, but in extremely rare cases leiomyoma can appear in the external auditory canal. Here we present a 37-year-old man with right auricular fullness. Preoperative findings suggested benign tumor or cholesteatoma in the right external auditory canal. We performed total resection using an endoauricular approach with transcanal endoscopic ear surgery. Histopathological and immunohistochemistry examination confirmed the diagnosis of leiomyoma of external auditory canal. Leiomyoma arising from soft tissue, including that in the external auditory canal, is classified into two types: that from the arrectores pilorum muscles and that from the muscle coats of blood vessels. Only four cases of leiomyoma of external auditor canal have been published in the English literature. The other four cases demonstrated vascular leiomyomas. This is the first report of leiomyoma of the EAC arising from arrectores pilorum muscles. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

  4. Ectopic external auditory canal and ossicular formation in the oculo-auriculo-vertebral spectrum

    Energy Technology Data Exchange (ETDEWEB)

    Supakul, Nucharin [Indiana University School of Medicine, Department of Radiology, Indianapolis, IN (United States); Ramathibodi Hospital, Mahidol University, Department of Diagnostic and Therapeutic Radiology, Bangkok (Thailand); Kralik, Stephen F. [Indiana University School of Medicine, Department of Radiology, Indianapolis, IN (United States); Ho, Chang Y. [Indiana University School of Medicine, Department of Radiology, Indianapolis, IN (United States); Riley Children' s Hospital, MRI Department, Indianapolis, IN (United States)

    2015-07-15

    Ear abnormalities in oculo-auricular-vertebral spectrum commonly present with varying degrees of external and middle ear atresias, usually in the expected locations of the temporal bone and associated soft tissues, without ectopia of the external auditory canal. We present the unique imaging of a 4-year-old girl with right hemifacial microsomia and ectopic location of an atretic external auditory canal, terminating in a hypoplastic temporomandibular joint containing bony structures with the appearance of auditory ossicles. This finding suggests an early embryological dysfunction involving Meckel's cartilage of the first branchial arch. (orig.)

  5. Impaired pitch perception and memory in congenital amusia: the deficit starts in the auditory cortex.

    Science.gov (United States)

    Albouy, Philippe; Mattout, Jérémie; Bouet, Romain; Maby, Emmanuel; Sanchez, Gaëtan; Aguera, Pierre-Emmanuel; Daligault, Sébastien; Delpuech, Claude; Bertrand, Olivier; Caclin, Anne; Tillmann, Barbara

    2013-05-01

    Congenital amusia is a lifelong disorder of music perception and production. The present study investigated the cerebral bases of impaired pitch perception and memory in congenital amusia using behavioural measures, magnetoencephalography and voxel-based morphometry. Congenital amusics and matched control subjects performed two melodic tasks (a melodic contour task and an easier transposition task); they had to indicate whether sequences of six tones (presented in pairs) were the same or different. Behavioural data indicated that in comparison with control participants, amusics' short-term memory was impaired for the melodic contour task, but not for the transposition task. The major finding was that pitch processing and short-term memory deficits can be traced down to amusics' early brain responses during encoding of the melodic information. Temporal and frontal generators of the N100m evoked by each note of the melody were abnormally recruited in the amusic brain. Dynamic causal modelling of the N100m further revealed decreased intrinsic connectivity in both auditory cortices, increased lateral connectivity between auditory cortices as well as a decreased right fronto-temporal backward connectivity in amusics relative to control subjects. Abnormal functioning of this fronto-temporal network was also shown during the retention interval and the retrieval of melodic information. In particular, induced gamma oscillations in right frontal areas were decreased in amusics during the retention interval. Using voxel-based morphometry, we confirmed morphological brain anomalies in terms of white and grey matter concentration in the right inferior frontal gyrus and the right superior temporal gyrus in the amusic brain. The convergence between functional and structural brain differences strengthens the hypothesis of abnormalities in the fronto-temporal pathway of the amusic brain. Our data provide first evidence of altered functioning of the auditory cortices during pitch

  6. The influence of visual information on auditory processing in individuals with congenital amusia: An ERP study.

    Science.gov (United States)

    Lu, Xuejing; Ho, Hao T; Sun, Yanan; Johnson, Blake W; Thompson, William F

    2016-07-15

    While most normal hearing individuals can readily use prosodic information in spoken language to interpret the moods and feelings of conversational partners, people with congenital amusia report that they often rely more on facial expressions and gestures, a strategy that may compensate for deficits in auditory processing. In this investigation, we used EEG to examine the extent to which individuals with congenital amusia draw upon visual information when making auditory or audio-visual judgments. Event-related potentials (ERP) were elicited by a change in pitch (up or down) between two sequential tones paired with a change in spatial position (up or down) between two visually presented dots. The change in dot position was either congruent or incongruent with the change in pitch. Participants were asked to judge (1) the direction of pitch change while ignoring the visual information (AV implicit task), and (2) whether the auditory and visual changes were congruent (AV explicit task). In the AV implicit task, amusic participants performed significantly worse in the incongruent condition than control participants. ERPs showed an enhanced N2-P3 response to incongruent AV pairings for control participants, but not for amusic participants. However when participants were explicitly directed to detect AV congruency, both groups exhibited enhanced N2-P3 responses to incongruent AV pairings. These findings indicate that amusics are capable of extracting information from both modalities in an AV task, but are biased to rely on visual information when it is available, presumably because they have learned that auditory information is unreliable. We conclude that amusic individuals implicitly draw upon visual information when judging auditory information, even though they have the capacity to explicitly recognize conflicts between these two sensory channels. Copyright © 2016 Elsevier Inc. All rights reserved.

  7. Clinical characteristics of keratosis obturans and external auditory canal cholesteatoma.

    Science.gov (United States)

    Park, So Young; Jung, Young Hoon; Oh, Jeong-Hoon

    2015-02-01

    Keratosis obturans (KO) and external auditory canal cholesteatoma (EACC) have been considered separate entities. While the disorders are distinct, they share many overlapping characteristics, making a correct diagnosis difficult. In the present study, we compared their clinical characteristics and radiological features to clarify the diagnostic criteria. Retrospective case series. Academic medical center. The clinical data of 23 cases of EACC and KO were retrospectively reviewed. The following clinical characteristics were compared between the 2 groups: sex, age, onset of symptoms, follow-up period, audiometric results, and imaging findings on temporal bone computed tomography including bilaterality, location, and the presence of extension to adjacent tissue. The mean age of the EACC group was significantly older than that of the KO group. All of the cases of EACC occurred unilaterally, and bilateral occurrences of KO were observed in 4 of 9 cases. All of the lesions in the KO group were circumferential, and no lesion in the EACC group invaded the superior canal wall. No significant differences in symptoms, such as acute otalgia, otorrhea, and hearing loss, were noted between the 2 groups. The incidence of conductive hearing impairment more than 10 dB was higher in the KO group than in the EACC group. Thus, KO and EACC are 2 distinct disease entities that share common features in clinical characteristics except for predominant age and bilaterality. Conservative treatment with meticulous cleaning of the lesion was successful in most cases with a long-term follow-up. © American Academy of Otolaryngology—Head and Neck Surgery Foundation 2014.

  8. A Narrow Internal Auditory Canal with Duplication in a Patient with Congenital Sensorineural Hearing Loss

    Energy Technology Data Exchange (ETDEWEB)

    Baik, Hye Won; Yu, Hye On; Kim, Kyung Soo; Kim, Gi Hyeon [Chung-Ang University Hospital, College of Medicine, Seoul (Korea, Republic of)

    2008-07-15

    A narrow internal auditory canal (IAC) with duplication is a rare anomaly of the temporal bone. It is associated with congenital sensorineural hearing loss. Aplasia or hypoplasia of the vestibulocochlear nerve may cause the hearing loss. We present an unusual case of an isolated narrow IAC with duplication that was detected by a CT scan. In this case, the IAC was divided by a bony septum into an empty stenotic inferoposterior portion and a large anterosuperior portion containing the facial nerve that was clearly delineated on MRI.

  9. Enhanced peripheral visual processing in congenitally deaf humans is supported by multiple brain regions, including primary auditory cortex

    OpenAIRE

    Scott, Gregory D.; Karns, Christina M.; Dow, Mark W.; Stevens, Courtney; Neville, Helen J.

    2014-01-01

    Brain reorganization associated with altered sensory experience clarifies the critical role of neuroplasticity in development. An example is enhanced peripheral visual processing associated with congenital deafness, but the neural systems supporting this have not been fully characterized. A gap in our understanding of deafness-enhanced peripheral vision is the contribution of primary auditory cortex. Previous studies of auditory cortex that use anatomical normalization across participants wer...

  10. [Constitute, imaging and auditory characteristics of pediatric patients with congenital malformations of inner ear in sensorineural hearing loss].

    Science.gov (United States)

    Li, Youjin; Yang, Jun; Li, Yun

    2011-01-01

    To analyze the constitute, imaging and auditory characteristics of pediatric patients with congenital malformations of inner ear in sensorineural hearing loss(SNHL). Imaging and auditory data of 125 cases (225 ears) in 860 pediatric patients with congenital SNHL who referred to ENT department of Shanghai Children Medical Center from February 2005 to January 2010 were retrospectively studied. Congenital malformations of inner ear accounted for 14.5% in 860 pediatric patients with congenital SNHL. Bilateral ear was involved in 98 cases (78.4%), unilateral in 27 cases (21.6%). One hundred and sixty-seven ears (74.2%) were identified as profound deafness, 36 ears (16%) severe deafness and 22 ears (9.8%) moderate deafness in 225 ears, respectively. In present group, large vestibular aqueduct (75.6%) was the most common and next was vestibular deformity (32%), then cochleovestibular deformity (23.1%). Mondini deformity (55.8%) was the most common and common cavity deformity (28.9%) next in cochleovestibular deformity. Profound deafness in cochlea involved congenital malformations of inner ear was significantly more than those in no cochlea involved malformations. The results are of importance for understanding the constitute of congenital malformations of inner ear in pediatric patients with congenital SNHL in China, for etiological diagnosis of congenital SNHL, for intervention including hearing aids or cochlear implant and prognosis.

  11. Adult-onset juvenile xanthogranuloma of the external auditory canal: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Hur, Joon Ho; Kim, Jae Kyun; Seo, Gi Young; Choi, Woo Sun; Byun, Jun Soo; Lee, Woong Jae; Lee, Tae Jin [Chung-Ang University College of Medicine, Chung-Ang University Hospital, Seoul (Korea, Republic of); Kim, Na Ra [Dept. of Radiology, Samsung Medical Center, Sungkyunkwan University College of Medicine, Seoul (Korea, Republic of)

    2016-05-15

    Juvenile xanthogranuloma (JXG) is a benign, spontaneously regressing lesion that usually occurs during the first year of life, but may also occur in adulthood. Although the most common presentation of JXG is the cutaneous lesion, it can also manifest in various visceral organs. JXG of the external auditory canal is extremely rare, and there have been only a few reports of those cases in the English literature. In this study, we present a case of pathologically proven JXG that occurred in the external auditory canal with a symptomatic clinical presentation.

  12. Movement Induces the Use of External Spatial Coordinates for Tactile Localization in Congenitally Blind Humans.

    Science.gov (United States)

    Heed, Tobias; Möller, Johanna; Röder, Brigitte

    2015-01-01

    To localize touch, the brain integrates spatial information coded in anatomically based and external spatial reference frames. Sighted humans, by default, use both reference frames in tactile localization. In contrast, congenitally blind individuals have been reported to rely exclusively on anatomical coordinates, suggesting a crucial role of the visual system for tactile spatial processing. We tested whether the use of external spatial information in touch can, alternatively, be induced by a movement context. Sighted and congenitally blind humans performed a tactile temporal order judgment task that indexes the use of external coordinates for tactile localization, while they executed bimanual arm movements with uncrossed and crossed start and end postures. In the sighted, start posture and planned end posture of the arm movement modulated tactile localization for stimuli presented before and during movement, indicating automatic, external recoding of touch. Contrary to previous findings, tactile localization of congenitally blind participants, too, was affected by external coordinates, though only for stimuli presented before movement start. Furthermore, only the movement's start posture, but not the planned end posture affected blind individuals' tactile performance. Thus, integration of external coordinates in touch is established without vision, though more selectively than when vision has developed normally, and possibly restricted to movement contexts. The lack of modulation by the planned posture in congenitally blind participants suggests that external coordinates in this group are not mediated by motor efference copy. Instead the task-related frequent posture changes, that is, movement consequences rather than planning, appear to have induced their use of external coordinates.

  13. Enhanced peripheral visual processing in congenitally deaf humans is supported by multiple brain regions, including primary auditory cortex

    Directory of Open Access Journals (Sweden)

    Gregory D. Scott

    2014-03-01

    Full Text Available Brain reorganization associated with altered sensory experience clarifies the critical role of neuroplasticity in development. An example is enhanced peripheral visual processing associated with congenital deafness, but the neural systems supporting this have not been fully characterized. A gap in our understanding of deafness-enhanced peripheral vision is the contribution of primary auditory cortex. Previous studies of auditory cortex that use anatomical normalization across participants were limited by inter-subject variability of Heschl’s gyrus. In addition to reorganized auditory cortex (cross-modal plasticity, a second gap in our understanding is the contribution of altered modality-specific cortices (visual intramodal plasticity in this case, as well as supramodal and multisensory cortices, especially when target detection is required across contrasts. Here we address these gaps by comparing fMRI signal change for peripheral versus perifoveal visual stimulation (11-15° vs. 2°-7° in congenitally deaf and hearing participants in a blocked experimental design with two analytical approaches: a Heschl’s gyrus region of interest analysis and a whole brain analysis. Our results using individually-defined primary auditory cortex (Heschl’s gyrus indicate that fMRI signal change for more peripheral stimuli was greater than perifoveal in deaf but not in hearing participants. Whole-brain analyses revealed differences between deaf and hearing participants for peripheral versus perifoveal visual processing in extrastriate visual cortex including primary auditory cortex, MT+/V5, superior-temporal auditory and multisensory and/or supramodal regions, such as posterior parietal cortex, frontal eye fields, anterior cingulate, and supplementary eye fields. Overall, these data demonstrate the contribution of neuroplasticity in multiple systems including primary auditory cortex, supramodal and multisensory regions, to altered visual processing in

  14. Auditory cues for orientation and postural control in sighted and congenitally blind people

    Science.gov (United States)

    Easton, R. D.; Greene, A. J.; DiZio, P.; Lackner, J. R.

    1998-01-01

    This study assessed whether stationary auditory information could affect body and head sway (as does visual and haptic information) in sighted and congenitally blind people. Two speakers, one placed adjacent to each ear, significantly stabilized center-of-foot-pressure sway in a tandem Romberg stance, while neither a single speaker in front of subjects nor a head-mounted sonar device reduced center-of-pressure sway. Center-of-pressure sway was reduced to the same level in the two-speaker condition for sighted and blind subjects. Both groups also evidenced reduced head sway in the two-speaker condition, although blind subjects' head sway was significantly larger than that of sighted subjects. The advantage of the two-speaker condition was probably attributable to the nature of distance compared with directional auditory information. The results rule out a deficit model of spatial hearing in blind people and are consistent with one version of a compensation model. Analysis of maximum cross-correlations between center-of-pressure and head sway, and associated time lags suggest that blind and sighted people may use different sensorimotor strategies to achieve stability.

  15. Squamous cell carcinoma of the external auditory canal: computed tomography findings in six cases

    International Nuclear Information System (INIS)

    Gonzalez, Fabio Mota; Paes Junior, Ademar Jose de Oliveira; Tornin, Olger de Souza; Souza, Ricardo Pires de

    2005-01-01

    Objective: to evaluate the role of computed tomography in the assessment of deep extension of squamous cell carcinoma of the external auditory canal. Materials and methods: in the period between May 1995 and December 2003 six patients with squamous cell carcinoma of the external auditory canal were submitted to computed tomography scan at 'Hospital Heliopolis', Sao Paulo, SP, Brazil, including axial and coronal slices with soft tissue and bone algorithms. Results: thickening of the soft tissue of the external auditory canal was seen in all patients, bone erosion and invasion of the middle ear in five (83.3%), invasion of the mastoid and parotid gland in four (66.7%), invasion of the temporomandibular joint in three (50%), and invasion of the middle cranial fossa, carotid canal and cervical lymph node enlargement in two (33.3%) patients. Conclusion: assessment of deep extension of squamous cell carcinoma of the external auditory canal using computed tomography is useful for clinical staging of the disease allowing better therapeutic planning for these patients. (author)

  16. Bilateral External Auditory Exostoses Causing Conductive Hearing Loss: A Case Report and Literature Review of the Surfer's Ear.

    Science.gov (United States)

    Barbon, Dennis A; Hegde, Rahul; Li, Shuo; Abdelbaki, Ahmed; Bajaj, Divyansh

    2017-10-30

    In patients with repeated exposure to cold water, such as cold water surfers and kayakers, the reactive exostoses can occur in the external auditory canal. The external auditory canal exostoses are multiple, benign bony growths. They can cause external auditory canal stenosis, leading to repeated otitis externa and potentially conductive hearing loss. It is vital to consider this entity in susceptible patients who report hearing loss, as timely intervention such as proper ear protection equipment can lower the risk of developing severe external auditory canal exostoses. We present a case of a 42-year-old male, cold water surfer with conductive hearing loss and bilateral external auditory canal (EAC) stenosis demonstrated on the computed tomography.

  17. Multislice CT of the auditory ossicles and ossicular ligaments. Delineation of normal anatomy and diagnosis of congenital anomaly

    International Nuclear Information System (INIS)

    Matsumoto, Shigeru; Tozaki, Hiromitu; Miyazaki, Hidemi

    2001-01-01

    By using four detector rows with 0.5 mm collimation, high resolution isotropic voxel data throughout the middle ear can be obtained with Multislice Helical CT (MSCT). The purpose of this study is to evaluate the usefulness of MSCT in demonstrating the auditory ossicles and ossicular ligaments and in the diagnosis of congenital ossicular anomalies. Thirty normal middle ears and 23 ear of 20 patients with suspicious congenital ossicular anomalies were examined. Axial images and multiplanar images were reconstructed. In the normal group, the images were evaluated based on scores for the visualization of the anatomical structure of the auditory ossicles and ossicular ligaments. In the group with anomalies, the findings suggesting ossicular anomalies were referenced and the prevalence was conjectured. Visualization of the auditory ossicles and ossicular ligaments was 98.3%-100% and 78.3%-100%, respectively. Congenital ossicular anomalies were detected in 20 ears (87.0%). MSCT is an accurate method for demonstrating minute and complicated 3D structures of the middle ear, and is found to be a technique of choice for diagnosis of ossicular anomalies. (author)

  18. Fibrous Dysplasia of the Temporal Bone with External Auditory Canal Stenosis and Secondary Cholesteatoma.

    Science.gov (United States)

    Liu, Yu-Hsi; Chang, Kuo-Ping

    2016-04-01

    Fibrous dysplasia is a slowly progressive benign fibro-osseous disease, rarely occurring in temporal bones. In these cases, most bony lesions developed from the bony part of the external auditory canals, causing otalgia, hearing impairment, otorrhea, and ear hygiene blockade and probably leading to secondary cholesteatoma. We presented the medical history of a 24-year-old woman with temporal monostotic fibrous dysplasia with secondary cholesteatoma. The initial presentation was unilateral conductive hearing loss. A hard external canal tumor contributing to canal stenosis and a near-absent tympanic membrane were found. Canaloplasty and type I tympanoplasty were performed, but the symptoms recurred after 5 years. She received canal wall down tympanomastoidectomy with ossciculoplasty at the second time, and secondary cholesteatoma in the middle ear was diagnosed. Fifteen years later, left otorrhea recurred again and transcanal endoscopic surgery was performed for middle ear clearance. Currently, revision surgeries provide a stable auditory condition, but her monostotic temporal fibrous dysplasia is still in place.

  19. Rapidly Growing Chondroid Syringoma of the External Auditory Canal: Report of a Rare Case

    Science.gov (United States)

    Vasileiadis, Ioannis; Kapetanakis, Stylianos; Petousis, Aristotelis; Karakostas, Euthimios; Simantirakis, Christos

    2011-01-01

    Introduction. Chondroid syrinoma of the external auditory canal is an extremely rare benign neoplasm representing the cutaneous counterpart of pleomorphic adenoma of salivary glands. Less than 35 cases have been reported in the international literature. Case Presentation. We report a case of a 34-year-old male in whom a rapidly growing, well-circumscribed tumor arising from the external auditory canal was presented. Otoscopy revealed a smooth, nontender lesion covered by normal skin that almost obstructs the external auditory meatus. MRI was performed to define the extension of the lesion. It confirmed the presence of a 1.5 × 0.8 cm T2 high-signal intensity lesion in the superior and posterior wall of EAC without signs of bone erosion. The patient underwent complete resection of the tumor. The diagnosis was confirmed by histopathologic examination. Conclusion. Although chondroid syringoma is extremely rare, it should always be considered in the differential diagnosis of an aural polyp. Chondroid syringomas are usually asymptomatic, slow-growing, single benign tumors in subcutaneous or intradermal location. In our case, the new information is that this benign tumor could present also as a rapidly growing lesion, arising the suspicion for malignancy. PMID:21941560

  20. Rapidly Growing Chondroid Syringoma of the External Auditory Canal: Report of a Rare Case

    Directory of Open Access Journals (Sweden)

    Ioannis Vasileiadis

    2011-01-01

    Full Text Available Introduction. Chondroid syrinoma of the external auditory canal is an extremely rare benign neoplasm representing the cutaneous counterpart of pleomorphic adenoma of salivary glands. Less than 35 cases have been reported in the international literature. Case Presentation. We report a case of a 34-year-old male in whom a rapidly growing, well-circumscribed tumor arising from the external auditory canal was presented. Otoscopy revealed a smooth, nontender lesion covered by normal skin that almost obstructs the external auditory meatus. MRI was performed to define the extension of the lesion. It confirmed the presence of a 1.5×0.8 cm T2 high-signal intensity lesion in the superior and posterior wall of EAC without signs of bone erosion. The patient underwent complete resection of the tumor. The diagnosis was confirmed by histopathologic examination. Conclusion. Although chondroid syringoma is extremely rare, it should always be considered in the differential diagnosis of an aural polyp. Chondroid syringomas are usually asymptomatic, slow-growing, single benign tumors in subcutaneous or intradermal location. In our case, the new information is that this benign tumor could present also as a rapidly growing lesion, arising the suspicion for malignancy.

  1. External auditory exostoses and hearing loss in the Shanidar 1 Neandertal.

    Directory of Open Access Journals (Sweden)

    Erik Trinkaus

    Full Text Available The Late Pleistocene Shanidar 1 older adult male Neandertal is known for the crushing fracture of his left orbit with a probable reduction in vision, the loss of his right forearm and hand, and evidence of an abnormal gait, as well as probable diffuse idiopathic skeletal hyperostosis. He also exhibits advanced external auditory exostoses in his left auditory meatus and larger ones with complete bridging across the porus in the right meatus (both Grade 3. These growths indicate at least unilateral conductive hearing (CHL loss, a serious sensory deprivation for a Pleistocene hunter-gatherer. This condition joins the meatal atresia of the Middle Pleistocene Atapuerca-SH Cr.4 in providing evidence of survival with conductive hearing loss (and hence serious sensory deprivation among these Pleistocene humans. The presence of CHL in these fossils thereby reinforces the paleobiological and archeological evidence for supporting social matrices among these Pleistocene foraging peoples.

  2. Effects of incongruent auditory and visual room-related cues on sound externalization

    DEFF Research Database (Denmark)

    Carvajal, Juan Camilo Gil; Santurette, Sébastien; Cubick, Jens

    Sounds presented via headphones are typically perceived inside the head. However, the illusion of a sound source located out in space away from the listener’s head can be generated with binaural headphone-based auralization systems by convolving anechoic sound signals with a binaural room impulse...... response (BRIR) measured with miniature microphones placed in the listener’s ear canals. Sound externalization of such virtual sounds can be very convincing and robust but there have been reports that the illusion might break down when the listening environment differs from the room in which the BRIRs were...... recorded [1,2,3]. This may be due to incongruent auditory cues between the recording and playback room during sound reproduction [2]. Alternatively, an expectation effect caused by the visual impression of the room may affect the position of the perceived auditory image [3]. Here, we systematically...

  3. Impaired Pitch Perception and Memory in Congenital Amusia: The Deficit Starts in the Auditory Cortex

    Science.gov (United States)

    Albouy, Philippe; Mattout, Jeremie; Bouet, Romain; Maby, Emmanuel; Sanchez, Gaetan; Aguera, Pierre-Emmanuel; Daligault, Sebastien; Delpuech, Claude; Bertrand, Olivier; Caclin, Anne; Tillmann, Barbara

    2013-01-01

    Congenital amusia is a lifelong disorder of music perception and production. The present study investigated the cerebral bases of impaired pitch perception and memory in congenital amusia using behavioural measures, magnetoencephalography and voxel-based morphometry. Congenital amusics and matched control subjects performed two melodic tasks (a…

  4. [The application of vibrant sound bridge in microtia whose reconstructive external auditory canal occurred atresia].

    Science.gov (United States)

    Zhao, Shouqin; Gong, Shusheng; Han, Demin; Chen, Shubin; Li, Yi; Ma, Xiaobo; Liu, Haihong

    2012-05-01

    To investigate the effect of vibrant sound bridge implantation in microtia whose reconstructive external auditory canal occurred atresia. Three cases (2 males and 1 female) of microtia had underwent hearing reconstruction operation (Include the external ear canal reconstructive surgery and tympanoplasty). The age ranged from 15 to 18 years and the average age was 17 years. All the 3 cases suffered from conductive hearing loss with the air-bone gap ranging from 51.6 to 65.0 dB HL and the average value being 56. 3 dB HL. All the 3 cases underwent vibrant sound bridge implantation, including the floating mass transducer implanted in the head of stapes in 2 cases and in the niche of round window in 1 case. The postoperative hearing level improved from 21.6 to 52.5 dB HL with an average of 32.2 dB HL. There were no complications such as vertigo, tinnitus and facial paralysis. Through vibrant sound bridge implantation, the hearing level of microtia whose reconstructive external auditory canal occurred atresia was improved effectively.

  5. Needle in the external auditory canal: an unusual complication of inferior alveolar nerve block.

    Science.gov (United States)

    Ribeiro, Leandro; Ramalho, Sara; Gerós, Sandra; Ferreira, Edite Coimbra; Faria e Almeida, António; Condé, Artur

    2014-06-01

    Inferior alveolar nerve block is used to anesthetize the ipsilateral mandible. The most commonly used technique is one in which the anesthetic is injected directly into the pterygomandibular space, by an intraoral approach. The fracture of the needle, although uncommon, can lead to potentially serious complications. The needle is usually found in the pterygomandibular space, although it can migrate and damage adjacent structures, with variable consequences. The authors report an unusual case of a fractured needle, migrating to the external auditory canal, as a result of an inferior alveolar nerve block. Copyright © 2014 Elsevier Inc. All rights reserved.

  6. Internal versus External Auditory Hallucinations in Schizophrenia: Symptom and Course Correlates

    Science.gov (United States)

    Docherty, Nancy M.; Dinzeo, Thomas J.; McCleery, Amanda; Bell, Emily K.; Shakeel, Mohammed K.; Moe, Aubrey

    2015-01-01

    Introduction The auditory hallucinations associated with schizophrenia are phenomenologically diverse. “External” hallucinations classically have been considered to reflect more severe psychopathology than “internal” hallucinations, but empirical support has been equivocal. Methods We examined associations of “internal” v. “external” hallucinations with (a) other characteristics of the hallucinations, (b) severity of other symptoms, and (c) course of illness variables, in a sample of 97 stable outpatients with schizophrenia or schizoaffective disorder who experienced auditory hallucinations. Results Patients with internal hallucinations did not differ from those with external hallucinations on severity of other symptoms. However, they reported their hallucinations to be more emotionally negative, distressing, and long-lasting, less controllable, and less likely to remit over time. They also were more likely to experience voices commenting, conversing, or commanding. However, they also were more likely to have insight into the self-generated nature of their voices. Patients with internal hallucinations were not older, but had a later age of illness onset. Conclusions Differences in characteristics of auditory hallucinations are associated with differences in other characteristics of the disorder, and hence may be relevant to identifying subgroups of patients that are more homogeneous with respect to their underlying disease processes. PMID:25530157

  7. Ceruminous Adenoma of the External Auditory Canal: A Case Report with Imaging and Pathologic Findings

    Directory of Open Access Journals (Sweden)

    George Psillas

    2015-01-01

    Full Text Available Ceruminous adenomas are benign tumors that are rare in humans and present with a nonspecific symptomatology. The treatment of choice is surgical excision. We present an 87-year-old woman who presented with a reddish, tender, round, soft mass of the outer third of the inferior wall of the left external auditory canal, discharging a yellowish fluid upon pressure. Coincidentally, due to her poor general condition, this patient also showed symptoms consistent with chronic otitis media, parotitis, and cervical lymphadenopathy, such as otorrhea, through a ruptured tympanic membrane and swelling of the parotid gland and cervical lymph nodes. The external auditory canal lesion was surgically excised under general anesthesia, utilizing a transmeatal approach. The pathological diagnosis was ceruminous gland adenoma. The tumor was made of tubular and cystic structures and embedded in a fibrous, focally hyalinized stroma. Immunohistochemistry confirmed the presence of two distinct cell populations. The luminal cells expressed keratin 7, while peripheral (basal cells expressed keratins 5/6, S100 protein, and p63. The apocrine gland-related antigen GCDFP-15 was focally expressed by tumor cells. The postoperative course was uneventful and at the 2-year follow-up no recurrence of the ceruminous adenoma was noted.

  8. External auditory canal cholesteatoma and keratosis obturans: the role of imaging in preventing facial nerve injury.

    Science.gov (United States)

    McCoul, Edward D; Hanson, Matthew B

    2011-12-01

    We conducted a retrospective study to compare the clinical characteristics of external auditory canal cholesteatoma (EACC) with those of a similar entity, keratosis obturans (KO). We also sought to identify those aspects of each disease that may lead to complications. We identified 6 patients in each group. Imaging studies were reviewed for evidence of bony erosion and the proximity of disease to vital structures. All 6 patients in the EACC group had their diagnosis confirmed by computed tomography (CT), which demonstrated widening of the bony external auditory canal; 4 of these patients had critical erosion of bone adjacent to the facial nerve. Of the 6 patients with KO, only 2 had undergone CT, and neither exhibited any significant bony erosion or expansion; 1 of them developed osteomyelitis of the temporal bone and adjacent temporomandibular joint. Another patient manifested KO as part of a dermatophytid reaction. The essential component of treatment in all cases of EACC was microscopic debridement of the ear canal. We conclude that EACC may produce significant erosion of bone with exposure of vital structures, including the facial nerve. Because of the clinical similarity of EACC to KO, misdiagnosis is possible. Temporal bone imaging should be obtained prior to attempts at debridement of suspected EACC. Increased awareness of these uncommon conditions is warranted to prompt appropriate investigation and prevent iatrogenic complications such as facial nerve injury.

  9. A comparison of patterns of disease extension in keratosis obturans and external auditory canal cholesteatoma.

    Science.gov (United States)

    Shinnabe, Akihiro; Hara, Mariko; Hasegawa, Masayo; Matsuzawa, Shingo; Kanazawa, Hiromi; Yoshida, Naohiro; Iino, Yukiko

    2013-01-01

    To investigate the different pathways of progression to the middle ear in keratosis obturans (KO) and external auditory canal cholesteatoma (EACC). Retrospective case review. Referral hospital otolaryngology department. Patients with KO or EACC and middle ear disease who underwent surgical management were included. Four ears of 4 patients (mean age, 41.25 yr) were the KO group, and 5 ears of 4 patients (mean age, 49.5 yr) were the EACC group. Intraoperative findings of the middle ear cavity were investigated in KO and EACC groups. In the KO group, 3 patients had a perforated tympanic membrane and cholesteatoma in the tympanic cavity. The other patient had preoperative right facial palsy. Removal of the keratin plug revealed an adherent tympanic membrane. In intraoperative findings, the tympanic segment of the fallopian canal was found to be eroded because of inflammation. No case initially progressed to the mastoid cavity. Four patients had external auditory canal cholesteatoma with middle ear disease. In EACC group, all patients had initial progression to the mastoid cavity. KO tends to progress initially to the tympanic cavity via a diseased tympanic membrane. EACC tends to progress to the mastoid cavity via destruction of the posterior bony canal. This is the first report to investigate differences in pathway of progression to the middle ear cavity in these 2 diseases.

  10. Surgical Procedures for External Auditory Canal Carcinoma and the Preservation of Postoperative Hearing

    Directory of Open Access Journals (Sweden)

    Hiroshi Hoshikawa

    2012-01-01

    Full Text Available Carcinoma of the external auditory canal (EAC is an unusual head and neck malignancy. The pathophysiology of these tumors is different from other skin lesions because of their anatomical and functional characteristics. Early-stage carcinoma of the EAC can be generally cured by surgical treatment, and reconstruction of the EAC with a tympanoplasty can help to retain hearing, thus improving the patients’ quality of life. In this study, we present two cases of early-stage carcinoma of the EAC treated by canal reconstruction using skin grafts after lateral temporal bone resection. A rolled-up skin graft with a temporal muscle flap was useful for keeping the form and maintaining the postoperative hearing. An adequate size of the skin graft and blood supply to the graft bed are important for achieving a successful operation.

  11. The relationship between external auditory canal temperature and onset of estrus and ovulation in beef heifers.

    Science.gov (United States)

    Randi, Federico; McDonald, Michael; Duffy, Pat; Kelly, Alan K; Lonergan, Patrick

    2018-04-01

    The aim of this study was to evaluate the relationship of body temperature fluctuations, as measured by external auditory canal temperature, to the onset of estrus and ovulation. Beef heifers (n = 44, mean age 23.5 ± 0.4 months, mean weight 603.3 ± 5.7 kg) were fitted with a Boviminder ® ear tag 2 weeks before the start of the estrous synchronization protocol to allow acclimatization. The device recorded the temperature, accurate to 0.01° Fahrenheit, every 10 min and transmitted the data via a base station over the internet where it could be accessed remotely. The estrous cycles of all heifers were synchronized using an 8-day progesterone-based synchronization program; on day 0 a PRID was inserted in conjunction with an injection of GnRH, and PGF2α was administered the day before PRID removal. Heifers were checked for signs of estrus at 4-h intervals (i.e., 6 times per day) commencing 24 h after PRID withdrawal. Beginning 12 h after the onset of estrus, the ovaries were ultrasound scanned at 4-h intervals to determine the time of ovulation. Body temperature was recorded every 10 min and averaged to hourly means for the following 4 periods relative to the detected oestrus onset (=Time 0): Period I: -48 h to -7 h, Period II: -6 h to +6 h, Period III +7 h to ovulation, and Period IV: ovulation to 48 h post ovulation. Data were analysed using a Mixed Model ANOVA in SAS in a completely randomized design to observe effects of induced estrus on external auditory canal temperature. The mean (±SD) interval from removal of the PRID to onset of estrus activity was 46.6 ± 14.7 h. The mean duration of estrus was 16.0 ± 5.67 h and the mean interval from estrus onset to ovulation was 27.9 ± 7.68 h. Highest temperatures (100.95 ± 0.03 °F) were observed in Period II around estrus onset, whereas lowest temperatures were observed in the 48 h preceding estrus onset (100.28 ± 0.03 °F; Period I) and around ovulation (100.30

  12. Surfer's ear: external auditory exostoses are more prevalent in cold water surfers.

    Science.gov (United States)

    Kroon, David F; Lawson, M Louise; Derkay, Craig S; Hoffmann, Karen; McCook, Joe

    2002-05-01

    The study goal was to demonstrate the prevalence and severity of external auditory exostoses (EAEs) in a population of surfers and to examine the relationship between these lesions and the length of time surfed as well as water temperature in which the swimmers surfed. It was hypothesized that subjects who predominantly surfed in colder waters had more frequent and more severe exostoses. Two hundred two avid surfers (91% male and 9% female, median age 17 years) were included in the study. EAEs were graded based on the extent of external auditory canal patency; grades of normal (100% patency), mild (66% to 99% patency), and moderate-severe (surfing habits. There was a 38% overall prevalence of EAEs, with 69% of lesions graded as mild and 31% graded as moderate-severe. Professional surfers (odds ratio 3.8) and those subjects who surfed predominantly in colder waters (odds ratio 5.8) were found to be at a significantly increased risk for the development of EAEs. The number of years surfed was also found to be significant, increasing one's risk for developing an exostosis by 12% per year and for developing more severe lesions by 10% per year. Individuals who had moderate-severe EAEs were significantly more likely to be willing to surf in colder waters than were those who had mild EAEs (odds ratio 4.3). EAEs are more prevalent in cold water surfers, and additional years surfing increase one's risk not only for developing an EAE but also for developing more severe lesions.

  13. Spontaneous external auditory canal cholesteatoma in a young male: Imaging findings and differential diagnoses

    Directory of Open Access Journals (Sweden)

    Yashant Aswani

    2016-01-01

    Full Text Available A cholesteatoma is a non-neoplastic lesion of the petrous temporal bone commonly described as “skin in the wrong place.” It typically arises within the middle ear cavity, may drain externally via tympanic membrane (mural type, or may originate in the external auditory canal (EAC. The latter type is rarely encountered and typically affects the elderly. EAC cholesteatoma poses diagnostic challenges because it has numerous differential diagnoses. The present case describes a 19-year-old male who presented with gradually progressive diminution of hearing in a previously naïve right ear since 8 months. A soft tissue attenuation lesion confined to the right EAC with erosion of the canal on computed tomography prompted magnetic resonance imaging (MRI. The lesion showed restricted diffusion on MRI. Thus, a diagnosis of spontaneous EAC cholesteatoma was established. The case elucidates the rarity of spontaneous EAC cholesteatoma in a young male. In addition, it describes the role of imaging to detect, delineate the extent, and characterize lesions of petrous temporal bone. The case also discusses common differential diagnoses of EAC cholesteatoma, as well as the importance of diffusion weighted imaging in EAC cholesteatoma similar to its middle ear counterpart.

  14. Development of a method for estimating oesophageal temperature by multi-locational temperature measurement inside the external auditory canal

    Science.gov (United States)

    Nakada, Hirofumi; Horie, Seichi; Kawanami, Shoko; Inoue, Jinro; Iijima, Yoshinori; Sato, Kiyoharu; Abe, Takeshi

    2017-09-01

    We aimed to develop a practical method to estimate oesophageal temperature by measuring multi-locational auditory canal temperatures. This method can be applied to prevent heatstroke by simultaneously and continuously monitoring the core temperatures of people working under hot environments. We asked 11 healthy male volunteers to exercise, generating 80 W for 45 min in a climatic chamber set at 24, 32 and 40 °C, at 50% relative humidity. We also exposed the participants to radiation at 32 °C. We continuously measured temperatures at the oesophagus, rectum and three different locations along the external auditory canal. We developed equations for estimating oesophageal temperatures from auditory canal temperatures and compared their fitness and errors. The rectal temperature increased or decreased faster than oesophageal temperature at the start or end of exercise in all conditions. Estimated temperature showed good similarity with oesophageal temperature, and the square of the correlation coefficient of the best fitting model reached 0.904. We observed intermediate values between rectal and oesophageal temperatures during the rest phase. Even under the condition with radiation, estimated oesophageal temperature demonstrated concordant movement with oesophageal temperature at around 0.1 °C overestimation. Our method measured temperatures at three different locations along the external auditory canal. We confirmed that the approach can credibly estimate the oesophageal temperature from 24 to 40 °C for people performing exercise in the same place in a windless environment.

  15. White Matter Integrity Dissociates Verbal Memory and Auditory Attention Span in Emerging Adults with Congenital Heart Disease.

    Science.gov (United States)

    Brewster, Ryan C; King, Tricia Z; Burns, Thomas G; Drossner, David M; Mahle, William T

    2015-01-01

    White matter disruptions have been identified in individuals with congenital heart disease (CHD). However, no specific theory-driven relationships between microstructural white matter disruptions and cognition have been established in CHD. We conducted a two-part study. First, we identified significant differences in fractional anisotropy (FA) of emerging adults with CHD using Tract-Based Spatial Statistics (TBSS). TBSS analyses between 22 participants with CHD and 18 demographically similar controls identified five regions of normal appearing white matter with significantly lower FA in CHD, and two higher. Next, two regions of lower FA in CHD were selected to examine theory-driven differential relationships with cognition: voxels along the left uncinate fasciculus (UF; a tract theorized to contribute to verbal memory) and voxels along the right middle cerebellar peduncle (MCP; a tract previously linked to attention). In CHD, a significant positive correlation between UF FA and memory was found, r(20)=.42, p=.049 (uncorrected). There was no correlation between UF and auditory attention span. A positive correlation between MCP FA and auditory attention span was found, r(20)=.47, p=.027 (uncorrected). There was no correlation between MCP and memory. In controls, no significant relationships were identified. These results are consistent with previous literature demonstrating lower FA in younger CHD samples, and provide novel evidence for disrupted white matter integrity in emerging adults with CHD. Furthermore, a correlational double dissociation established distinct white matter circuitry (UF and MCP) and differential cognitive correlates (memory and attention span, respectively) in young adults with CHD.

  16. Imaging of congenital anomalies of the temporal bone.

    Science.gov (United States)

    Benton, C; Bellet, P S

    2000-02-01

    This article briefly presents the embryology of the ear and discusses the external auditory canal and middle ear, including first branchial cleft anomalies, external auditory canal atresia and stenoses, congenital cholesteatoma, and congenital teratoma. Topics related to the labyrinths include aplasia, the common-chamber anomaly, the pseudo-Mondini and Mondini malformations, semicircular canal dysplasia, and the large vestibular aqueduct. Vascular malformations and variations also are presented, including the absent and aberrant internal carotid artery, the persistent stapedial artery, and high jugular bulb; cerebrospinal fluid and perilymph fistulas are the subjects of the final section.

  17. Congenital cholesteatoma of the infratemporal fossa with congenital aural atresia and mastoiditis: a case report

    Directory of Open Access Journals (Sweden)

    Abdel-Aziz Mosaad

    2012-06-01

    Full Text Available Abstract Background Congenital cholesteatoma may be expected in abnormally developed ear, it may cause bony erosion of the middle ear cleft and extend to the infratemporal fossa. We present the first case of congenital cholesteatoma of the infratemporal fossa in a patient with congenital aural atresia that has been complicated with acute mastoiditis. Case presentation A sixteen year old Egyptian male patient presented with congenital cholesteatoma of the infratemporal fossa with congenital aural atresia complicated with acute mastoiditis. Two weeks earlier, the patient suffered pain necessitating hospital admission, magnetic resonance imaging revealed a soft tissue mass in the right infratemporal fossa. On presentation to our institute, Computerized tomography was done as a routine, it proved the diagnosis of mastoiditis, pure tone audiometry showed an air-bone gap of 60 dB. Cortical mastoidectomy was done for treatment of mastoiditis, removal of congenital cholesteatoma was carried out with reconstruction of external auditory canal. Follow-up of the patient for 2 years and 3 months showed a patent, infection free external auditory canal with an air-bone gap has been reduced to 35db. One year after the operation; MRI was done and it showed no residual or recurrent cholesteatoma. Conclusions Congenital cholesteatoma of the infratemporal fossa in cases of congenital aural atresia can be managed safely even if it was associated with mastoiditis. It is an original case report of interest to the speciality of otolaryngology.

  18. Vertically transmitted HPV-dependent squamous cell carcinoma of the external auditory canal. Case report of a child

    International Nuclear Information System (INIS)

    Snietura, Miroslaw; Pakulo, Slawomir; Kopec, Agnieszka; Piglowski, Wojciech; Stanek-Widera, Agata; Chelmecka-Wiktorczyk, Liliana; Drabik, Grazyna; Kosowski, Bogdan; Wyrobek, Lukasz; Balwierz, Walentyna

    2017-01-01

    There is much evidence that high-risk human papillomavirus (HPV) plays a causative role in a subset of head and neck squamous cell cancer (HNSCC) in adults. HPV-positive tumors behave differently even in their response to treatment and are therefore a distinct subset. Both HPV-positive and HPV-negative tumors of the head and neck region are usually in the domain of adults and cases in children are rare; thus when a 2-year-old child was diagnosed with this cancer in the external auditory canal, an in-depth assessment of the tumor was considered necessary. A 2-year-old girl was born to a HPV-positive mother who was diagnosed with cervical cancer during pregnancy. The child was delivered by caesarean section and the mother died of her cancer 7 months after delivery. After the diagnosis of locally invasive HPV-positive squamous cell cancer of the external auditory canal, the child was treated surgically, and with chemotherapy and radiotherapy. Full remission was obtained lasting up to 325 weeks since treatment was started, resulting in over 6 years of disease-free survival. This is the first case of advanced, HPV-related HNSCC in a 2-year-old child, in whom the tumor was located in the external auditory canal and who made a dramatic recovery after treatment with nonradical surgery, chemotherapy and radiotherapy. The child has currently been disease free for 6 years. This case supports the observation that HPV-related HNSCC tumors appear to respond favorably to treatment despite the patient's young age and the clinically advanced stage of the tumor. (orig.) [de

  19. The auditory dorsal stream plays a crucial role in projecting hallucinated voices into external space

    NARCIS (Netherlands)

    Looijestijn, Jasper; Diederen, Kelly M. J.; Goekoop, Rutger; Sommer, Iris E. C.; Daalman, Kirstin; Kahn, Rene S.; Hoek, Hans W.; Blom, Jan Dirk

    Introduction: Verbal auditory hallucinations (VAHs) are experienced as spoken voices which seem to originate in the extracorporeal environment or inside the head. Animal and human research has identified a 'where' pathway for sound processing comprising the planum temporale, the middle frontal gyrus

  20. Musical Brains. A study of evoked musical sensations without external auditory stimuli. Preliminary report of three cases

    International Nuclear Information System (INIS)

    Goycoolea, Marcos V; Mena, Ismael; Neubauer, Sonia G; Levy, Raquel G.; Fernandez Grez, Margarita; Berger, Claudia G

    2006-01-01

    Background: There are individuals, usually musicians, who are seemingly able to evoke musical sensations without external auditory stimuli. However, to date there is no available evidence to determine if it is feasible to have musical sensations without using external sensory receptors nor if there is a biological substrate to these sensations. Study design: Two single photon emission computerized tomography (SPECT) evaluations with [99mTc]-HMPAO were conducted in each of three female musicians. One was done under basal conditions (without evoking) and the other one while evoking these sensations. Results: In the NeuroSPECT studies of the musicians who were tested while evoking a musical composition, there was a significant increase in perfusion above the normal mean in the right and left hemispheres in Brodmann's areas 9 and 8 (frontal executive area) and in areas 40 on the left side (auditory center). However, under basal conditions there was no hyper perfusion of areas 9, 8, 39 and 40. In one case hyper perfusion was found under basal conditions in area 45, however it was less than when she was evoking. Conclusions: These findings are suggestive of a biological substrate to the process of evoking musical sensations (au)

  1. Selective attention reduces physiological noise in the external ear canals of humans. I: Auditory attention

    Science.gov (United States)

    Walsh, Kyle P.; Pasanen, Edward G.; McFadden, Dennis

    2014-01-01

    In this study, a nonlinear version of the stimulus-frequency OAE (SFOAE), called the nSFOAE, was used to measure cochlear responses from human subjects while they simultaneously performed behavioral tasks requiring, or not requiring, selective auditory attention. Appended to each stimulus presentation, and included in the calculation of each nSFOAE response, was a 30-ms silent period that was used to estimate the level of the inherent physiological noise in the ear canals of our subjects during each behavioral condition. Physiological-noise magnitudes were higher (noisier) for all subjects in the inattention task, and lower (quieter) in the selective auditory-attention tasks. These noise measures initially were made at the frequency of our nSFOAE probe tone (4.0 kHz), but the same attention effects also were observed across a wide range of frequencies. We attribute the observed differences in physiological-noise magnitudes between the inattention and attention conditions to different levels of efferent activation associated with the differing attentional demands of the behavioral tasks. One hypothesis is that when the attentional demand is relatively great, efferent activation is relatively high, and a decrease in the gain of the cochlear amplifier leads to lower-amplitude cochlear activity, and thus a smaller measure of noise from the ear. PMID:24732069

  2. Squamous cell carcinoma of the external auditory canal: computed tomography findings in six cases; Carcinoma espinocelular do conduto auditivo externo: estudo por tomografia computadorizada de seis casos

    Energy Technology Data Exchange (ETDEWEB)

    Gonzalez, Fabio Mota; Paes Junior, Ademar Jose de Oliveira; Tornin, Olger de Souza [Complexo Hospitalar Heliopolis, Sao Paulo, SP (Brazil). Servico de Diagnostico por Imagem]. E-mail: fmg961@yahoo.com.br; Souza, Ricardo Pires de [Complexo Hospitalar Heliopolis, Sao Paulo, SP (Brazil). Programa de Residencia Medica em Radiologia e Diagnostico por Imagem

    2005-05-15

    Objective: to evaluate the role of computed tomography in the assessment of deep extension of squamous cell carcinoma of the external auditory canal. Materials and methods: in the period between May 1995 and December 2003 six patients with squamous cell carcinoma of the external auditory canal were submitted to computed tomography scan at 'Hospital Heliopolis', Sao Paulo, SP, Brazil, including axial and coronal slices with soft tissue and bone algorithms. Results: thickening of the soft tissue of the external auditory canal was seen in all patients, bone erosion and invasion of the middle ear in five (83.3%), invasion of the mastoid and parotid gland in four (66.7%), invasion of the temporomandibular joint in three (50%), and invasion of the middle cranial fossa, carotid canal and cervical lymph node enlargement in two (33.3%) patients. Conclusion: assessment of deep extension of squamous cell carcinoma of the external auditory canal using computed tomography is useful for clinical staging of the disease allowing better therapeutic planning for these patients. (author)

  3. A Rare Case Report and Literature Review of External Auditory Canal Cholesteatoma with Circumferential Destruction of Canal Wall Exposing Facial Nerve

    Directory of Open Access Journals (Sweden)

    Leison Maharjan

    2017-01-01

    Full Text Available External auditory canal cholesteatoma (EACC is a rare condition with an estimated incidence of 1.2 per 1000 new otological patients. It is often mistaken with keratosis obturans. We discuss an extensive primary EACC with an aural polyp in a male which was managed by modified radical mastoidectomy.

  4. Congenital abnormality of the inner ear and internal auditory canal in a patient with deep sensorineural hearing loss: A case report

    International Nuclear Information System (INIS)

    Rivera, Diego; Saab, Said; Cordoba, Claudia; Montes, Guillermo; Barreto, Tatiana

    2010-01-01

    CT and MRI are complementary studies that have proven to be the best radiological tools in screening of children with unilateral or bilateral sensorineural hearing loss. Only about a 20% of the patients with congenital sensor neural hearing loss have manifestations in images. Due to the fact that most of these manifestations initiate in the bone, the CT is the first line of image study. MRI is indicated in the evaluation of suspected agenesis, neuropathy, aplasia or hypoplasia of the vestibulocochlear nerve, often associated with this type of hearing loss. We present a case of a 6 year old patient with deep bilateral sensor neural hearing loss, with radiological studies to determine a potential candidate for a cochlear or auditory brainstem implant as hearing rehabilitation.

  5. Helical CT of congenital ossicular anomalies

    International Nuclear Information System (INIS)

    Osada, Hisato; Machida, Kikuo; Honda, Norinari

    2001-01-01

    Since January 1996 to December 2000, 26 cases of congenital ossicular anomaly could be diagnosed with helical CT. All cases were unilateral. In 8 patients with malformation of the external ear, CT showed malleoincudal fixation (n=5), malleoincudal fixation and deformed incuts long process (n=1), deformed incus long process (n=1), and partial fusion of malleus neck and incus long process (n=1). In 18 patients with normal external ear, CT showed defect of the incus long process (n=5), defect of both the incus long process and stapes superstructure (n=8, 2 patients with congenital cholesteatoma, 1 with hypoplastic oval window), defect of the stapes superstructure (n=2, 1 patient with oval window absence), defect of the malleus manubrium (n=1), ossification of the stampede's tendon (n=1), and monopod stapes (n=1). Helical CT can evaluate the auditory ossicular chain in detail and is useful for diagnosing congenital ossicular anomaly. (author)

  6. External misattribution of internal thoughts and proneness to auditory hallucinations: the effect of emotional valence in the Deese–Roediger–McDermott paradigm

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    Mari eKanemoto

    2013-07-01

    Full Text Available Previous studies have suggested that a tendency to externalize internal thought is related to auditory hallucinations or even proneness to auditory hallucinations (AHp in the general population. However, although auditory hallucinations are related to emotional phenomena, few studies have investigated the effect of emotional valence on the aforementioned relationship. In addition, we do not know what component of psychotic phenomena relate to externalizing bias. The current study replicated our previous research, which suggested that individual differences in auditory hallucination-like experiences are strongly correlated with the external misattribution of internal thoughts, conceptualized in terms of false memory, using the Deese–Roediger–McDermott (DRM paradigm. We found a significant relationship between experimental performance and total scores on the Launay–Slade Hallucination Scale (LSHS. Among the LSHS factors, only vivid mental image, which is said to be a predictor of auditory hallucinations, was significantly related to experimental performance.We then investigated the potential effect of emotional valence using the DRM paradigm. The results indicate that participants with low scores on the LSHS (the low-AHp group in the current study showed an increased discriminability index (d' for positive words and a decreased d' for negative words. However, no effects of emotional valence were found for participants with high LSHS scores (high-AHp group. This study indicated that external misattribution of internal thoughts predicts AHp, and that the high-AHp group showed a smaller emotional valence effect in the DRM paradigm compared with the low-AHp group. We discuss this outcome from the perspective of the dual-process activation-monitoring framework in the DRM paradigm in regard to emotion-driven automatic thought in false memory.

  7. Vertically transmitted HPV-dependent squamous cell carcinoma of the external auditory canal. Case report of a child

    Energy Technology Data Exchange (ETDEWEB)

    Snietura, Miroslaw; Pakulo, Slawomir; Kopec, Agnieszka; Piglowski, Wojciech; Stanek-Widera, Agata [Maria Sklodowska-Curie Memorial Cancer Center and Institute of Oncology, Tumor Pathology Department, Gliwice (Poland); Chelmecka-Wiktorczyk, Liliana [University Children' s Hospital of Cracow, Department of Oncology and Hematology, Cracow (Poland); Drabik, Grazyna [University Children' s Hospital of Cracow, Pathology Department, Cracow (Poland); Kosowski, Bogdan [NZOZ Prosmed, Cracow (Poland); Wyrobek, Lukasz [University Children' s Hospital of Cracow, Department of Radiology, Cracow (Poland); Balwierz, Walentyna [University Children' s Hospital of Cracow, Department of Oncology and Hematology, Cracow (Poland); Jagiellonian University in Cracow, Clinic of Oncology and Hematology, Polish-American Pediatric Institute, Cracow (Poland)

    2017-02-15

    There is much evidence that high-risk human papillomavirus (HPV) plays a causative role in a subset of head and neck squamous cell cancer (HNSCC) in adults. HPV-positive tumors behave differently even in their response to treatment and are therefore a distinct subset. Both HPV-positive and HPV-negative tumors of the head and neck region are usually in the domain of adults and cases in children are rare; thus when a 2-year-old child was diagnosed with this cancer in the external auditory canal, an in-depth assessment of the tumor was considered necessary. A 2-year-old girl was born to a HPV-positive mother who was diagnosed with cervical cancer during pregnancy. The child was delivered by caesarean section and the mother died of her cancer 7 months after delivery. After the diagnosis of locally invasive HPV-positive squamous cell cancer of the external auditory canal, the child was treated surgically, and with chemotherapy and radiotherapy. Full remission was obtained lasting up to 325 weeks since treatment was started, resulting in over 6 years of disease-free survival. This is the first case of advanced, HPV-related HNSCC in a 2-year-old child, in whom the tumor was located in the external auditory canal and who made a dramatic recovery after treatment with nonradical surgery, chemotherapy and radiotherapy. The child has currently been disease free for 6 years. This case supports the observation that HPV-related HNSCC tumors appear to respond favorably to treatment despite the patient's young age and the clinically advanced stage of the tumor. (orig.) [German] Es gibt reichlich Belege dafuer, dass das menschliche Papillomavirus vom Hochrisikotyp (HR-HPV) in einigen Faellen von Plattenepithelkarzinomen des Kopf- und Halsbereichs (HNSCC) eine Schluesselrolle spielt. HPV-positive Tumoren verhalten sich anders, auch bezueglich des Ansprechens auf die Behandlung. Deswegen stellen sie eine separate biologische Gruppe dar. Sowohl HPV-positive als auch HPV

  8. Foreign bodies in the external auditory canal: Influence of age on incidence and outcomes in a Japanese population.

    Science.gov (United States)

    Nakao, Yoshio; Tanigawa, Tohru; Murotani, Kenta; Yamashita, Jun-Ichi

    2017-11-01

    Otolaryngologists occasionally observe foreign bodies (FB) in the external auditory canal (EAC), although relatively few studies have focused on the role of age in this condition. We retrospectively compared the incidences, outcomes and complications of FB in the EAC in different age groups. The patients at our center included 24 children (19%), 46 adults (37%) and 56 older adults (44%). Compared with adults, older adults were significantly more likely to have FB (peak age 75-79 years), be women (18/46 vs 34/56, P = 0.0461) and be unaware of their FB (18/46 vs 34/56, P = 0.0461). We observed that all EAC FB were more common during the summer, and biotic FB were not observed during the winter. Complications were more common in cases of biotic FB, compared with abiotic FB (5/27 vs 6/99, P = 0.0421). Our findings show that older adults are particularly susceptible to FB, are frequently unaware of their FB and can develop complications. These characteristics should be considered before treating FB in the EAC. Geriatr Gerontol Int 2017; 17: 2131-2135. © 2017 Japan Geriatrics Society.

  9. Prosthetic rehabilitation of intentionally malpositioned implants in a patient with squamous cell carcinoma of the external auditory canal: a clinical report.

    Science.gov (United States)

    Bagherpour, Reza; Mirfazaelian, Ali; Mangoli, Amir A; Alikhasi, Marzieh

    2009-08-01

    This clinical report describes the rehabilitation of a patient with a compromised temporal bone anatomy caused by resection of bone due to squamous cell carcinoma of external auditory canal. Tumor resection was followed by placement of three implants. The implants were intentionally malpositioned in the anterior-posterior plane due to the lack of temporal bone. Prosthodontic treatment included customized implant bar-supported auricular prosthesis.

  10. "Out-of-pitch" but still "in-time". An auditory psychophysical study in congenital amusic adults.

    Science.gov (United States)

    Hyde, Kristal L; Peretz, Isabelle

    2003-11-01

    Congenital amusia is a lifelong disability, commonly known as tone deafness, that prevents afflicted individuals from developing basic musical skills despite normal audiometry and above-average intellectual, memory, and language skills. Although it is estimated that 4% of the general population would be born with such a musical handicap, the underlying cause is presently unknown. Recently, we proposed that this disorder could be traced to a deficit in pitch perception on the basis of a single case. Here we provide psychophysical evidence for the existence of a generalized defect that is both fine grained and specific to pitch because time is unaffected.

  11. Event-related potential response to auditory social stimuli, parent-reported social communicative deficits and autism risk in school-aged children with congenital visual impairment

    Directory of Open Access Journals (Sweden)

    Joe Bathelt

    2017-10-01

    Full Text Available Communication with visual signals, like facial expression, is important in early social development, but the question if these signals are necessary for typical social development remains to be addressed. The potential impact on social development of being born with no or very low levels of vision is therefore of high theoretical and clinical interest. The current study investigated event-related potential responses to basic social stimuli in a rare group of school-aged children with congenital visual disorders of the anterior visual system (globe of the eye, retina, anterior optic nerve. Early-latency event-related potential responses showed no difference between the VI and control group, suggesting similar initial auditory processing. However, the mean amplitude over central and right frontal channels between 280 and 320 ms was reduced in response to own-name stimuli, but not control stimuli, in children with VI suggesting differences in social processing. Children with VI also showed an increased rate of autistic-related behaviours, pragmatic language deficits, as well as peer relationship and emotional problems on standard parent questionnaires. These findings suggest that vision may be necessary for the typical development of social processing across modalities.

  12. Event-related potential response to auditory social stimuli, parent-reported social communicative deficits and autism risk in school-aged children with congenital visual impairment.

    Science.gov (United States)

    Bathelt, Joe; Dale, Naomi; de Haan, Michelle

    2017-10-01

    Communication with visual signals, like facial expression, is important in early social development, but the question if these signals are necessary for typical social development remains to be addressed. The potential impact on social development of being born with no or very low levels of vision is therefore of high theoretical and clinical interest. The current study investigated event-related potential responses to basic social stimuli in a rare group of school-aged children with congenital visual disorders of the anterior visual system (globe of the eye, retina, anterior optic nerve). Early-latency event-related potential responses showed no difference between the VI and control group, suggesting similar initial auditory processing. However, the mean amplitude over central and right frontal channels between 280 and 320ms was reduced in response to own-name stimuli, but not control stimuli, in children with VI suggesting differences in social processing. Children with VI also showed an increased rate of autistic-related behaviours, pragmatic language deficits, as well as peer relationship and emotional problems on standard parent questionnaires. These findings suggest that vision may be necessary for the typical development of social processing across modalities. Copyright © 2017 The Authors. Published by Elsevier Ltd.. All rights reserved.

  13. Carcinoma espinocelular do conduto auditivo externo: estudo por tomografia computadorizada de seis casos Squamous cell carcinoma of the external auditory canal: computed tomography findings in six cases

    Directory of Open Access Journals (Sweden)

    Fábio Mota Gonzalez

    2005-06-01

    Full Text Available OBJETIVO: Analisar a importância da avaliação tomográfica da extensão profunda dos carcinomas espinocelulares do conduto auditivo externo. MATERIAIS E MÉTODOS: Foram realizados exames tomográficos com cortes axiais e coronais com janelas para partes moles e óssea em seis pacientes com carcinoma espinocelular do conduto auditivo externo, com idade variando entre 55 e 71 anos, internados no Hospital Heliópolis, no período entre maio de 1995 e dezembro de 2003. RESULTADOS: Dos seis pacientes, todos apresentavam aumento de partes moles no conduto auditivo externo, cinco (83,3% tinham erosão óssea e invasão da orelha média, quatro (66,7% possuíam comprometimento da mastóide e da glândula parótida, três (50% apresentavam invasão da articulação temporomandibular, dois (33,3% tinham invasão da fossa média, do canal carotídeo e linfonodomegalia júgulo-carotídea alta ipisilateral. CONCLUSÃO: A avaliação da extensão tumoral profunda fornecida pela tomografia computadorizada é importante no estadiamento clínico, possibilitando um planejamento terapêutico mais eficaz.OBJECTIVE: To evaluate the role of computed tomography in the assessment of deep extension of squamous cell carcinoma of the external auditory canal. MATERIALS AND METHODS: In the period between May 1995 and December 2003 six patients with squamous cell carcinoma of the external auditory canal were submitted to computed tomography scan at "Hospital Heliópolis", São Paulo, SP, Brazil, including axial and coronal slices with soft tissue and bone algorithms. RESULTS: Thickening of the soft tissue of the external auditory canal was seen in all patients, bone erosion and invasion of the middle ear in five (83.3%, invasion of the mastoid and parotid gland in four (66.7%, invasion of the temporomandibular joint in three (50%, and invasion of the middle cranial fossa, carotid canal and cervical lymph node enlargement in two (33.3% patients. CONCLUSION: Assessment of

  14. Longitudinal bracketed epiphysis of proximal phalanx of the great toe with congenital hallux varus managed simultaneously with monorail external fixator: a case report.

    Science.gov (United States)

    Verma, Vineet; Batra, Amit; Singla, Rohit; Gogna, Paritosh; Magu, Narender; Gupta, Rakesh

    2014-02-01

    Longitudinal bracketed epiphysis (delta phalanx) is a rare congenital anomaly that affects phalanges in the hand more commonly than toes. We present a rare case of congenital hallux varus with longitudinal bracketed epiphysis of proximal phalanx with bifid distal phalanx of the great toe, which was managed with monorail type of external fixator. To the best of our knowledge, this is the first report of its successful implementation in simultaneous treatment of longitudinal bracketed epiphysis of the proximal phalanx of the great toe and hallux varus. Apart from adding to the literature a case of rare subtype of delta phalanx with hallux varus, the present study highlights the role of a reliable alternative in its management.

  15. Congenital amusias.

    Science.gov (United States)

    Tillmann, B; Albouy, P; Caclin, A

    2015-01-01

    In contrast to the sophisticated music processing reported in the general population, individuals with congenital amusia show deficits in music perception and production. Congenital amusia occurs without brain damage, sensory or cognitive deficits, and has been suggested as a lifelong deficit with genetic origin. Even though recognized for a long time, this disorder has been systematically studied only relatively recently for its behavioral and neural correlates. The currently most investigated hypothesis about the underlying deficits concerns the pitch dimension, notably with impaired pitch discrimination and memory. Anatomic and functional investigations of pitch processing revealed that the amusic brain presents abnormalities in the auditory and inferior frontal cortices, associated with decreased connectivity between these structures. The deficit also impairs processing of pitch in speech material and processing of the time dimension in music for some of the amusic individuals, but does not seem to affect spatial processing. Some studies suggest at least partial dissociation in the disorder between perception and production. Recent studies revealed spared implicit pitch perception in congenital amusia, supporting the power of implicit cognition in the music domain. Current challenges consist in defining different subtypes of congenital amusia as well as developing rehabilitation programs for this "musical handicap." © 2015 Elsevier B.V. All rights reserved.

  16. Surgery With or Without Postoperative Radiation Therapy for Early-stage External Auditory Canal Squamous Cell Carcinoma: A Meta-analysis.

    Science.gov (United States)

    Oya, Ryohei; Takenaka, Yukinori; Takemura, Kazuya; Ashida, Naoki; Shimizu, Kotaro; Kitamura, Takahiro; Yamamoto, Yoshifumi; Uno, Atsuhiko

    2017-10-01

    External auditory canal squamous cell carcinoma (EACSCC) is a rare disease with no standard treatment supported by high-level evidence. The aim of this study was to investigate EACSCC prognoses according to treatment modality and thus determine the optimal intervention for early-stage disease. PubMed, Scopus, and Ichushi-Web searches of the English and Japanese-language literature published between January 1, 2006 and December 31, 2016 were performed using the key words "external auditory canal cancer" and "temporal bone cancer." Articles related to EACSCC that include the 5-year overall survival rate or individual patient data for histological types, follow-up periods, and final outcomes were enrolled. Sex, age, Moody's modified Pittsburgh stage, type of treatment modality, type of operation, follow-up period, and 5-year survival rates were extracted. Twenty articles were used for the aggregate meta-analysis using a random-effects model, and 18 articles that reported 99 patients with early-stage EACSCC were used for the individual patient data meta-analysis. The 5-year overall survival rate of early-stage EACSCC was 77%. Postoperative radiation therapy (PORT) was performed in 45% of stage I patients and 68% of stage II patients. Survival analysis of all patients showed no differences between the surgery-only and PORT groups; however, PORT exhibited a better prognosis than surgery alone among patients with stage I disease (p = 0.003, log-rank test). This result indicated that PORT can be the standard therapy for stages I and II EACSCC.

  17. Quantitative Electromyographic Analysis of Reaction Time to External Auditory Stimuli in Drug-Naïve Parkinson’s Disease

    Directory of Open Access Journals (Sweden)

    Do-Young Kwon

    2014-01-01

    Full Text Available Evaluation of motor symptoms in Parkinson’s disease (PD is still based on clinical rating scales by clinicians. Reaction time (RT is the time interval between a specific stimulus and the start of muscle response. The aim of this study was to identify the characteristics of RT responses in PD patients using electromyography (EMG and to elucidate the relationship between RT and clinical features of PD. The EMG activity of 31 PD patients was recorded during isometric muscle contraction. RT was defined as the time latency between an auditory beep and responsive EMG activity. PD patients demonstrated significant delays in both initiation and termination of muscle contraction compared with controls. Cardinal motor symptoms of PD were closely correlated with RT. RT was longer in more-affected side and in more-advanced PD stages. Frontal cognitive function, which is indicative of motor programming and movement regulation and perseveration, was also closely related with RT. In conclusion, greater RT is the characteristic motor features of PD and it could be used as a sensitive tool for motor function assessment in PD patients. Further investigations are required to clarify the clinical impact of the RT on the activity of daily living of patients with PD.

  18. Congenital high airway obstruction syndrome in the breech presentation managed by ex utero intrapartum treatment procedure after intraoperative external cephalic version.

    Science.gov (United States)

    Miwa, Ichiro; Sase, Masakatsu; Nakamura, Yasuhiko; Hasegawa, Keiko; Kawasaki, Masahiro; Ueda, Kazuyuki

    2012-05-01

    Congenital high airway obstruction syndrome (CHAOS) caused by laryngeal atresia was diagnosed by prenatal ultrasound in a male fetus at 26 weeks of gestation. Findings included massive ascites, subcutaneous edema, enlarged hyperechogenic lungs with diaphragmatic inversion, dilated trachea, polyhydramnios, and breech presentation. Those findings of CHAOS spontaneously returned to normal by 33 weeks of gestation. However, the placenta was localized to the anterior uterine wall. In addition, the fetal position had been breech until delivery. At 36 weeks of gestation, a planned ex utero intrapartum treatment (EXIT) procedure was performed following intraoperative external cephalic version (ECV) in which the fetus was approached from the posterior wall of the uterus. Laryngoscopy revealed the predicted laryngeal obstruction, and tracheostomy was placed. Intraoperative ECV may be a useful technique in breech presentation before EXIT procedure. © 2012 The Authors. Journal of Obstetrics and Gynaecology Research © 2012 Japan Society of Obstetrics and Gynecology.

  19. Congenital amusia.

    Science.gov (United States)

    Williamson, Victoria J; Stewart, Lauren

    2013-01-01

    For most people, music, like language, is acquired effortlessly in early life. But a few percent of the population have lifelong difficulties in the perception and production of music. In this chapter we discuss psycho-acoustic and behavioral studies that have attempted to delineate the nature of the auditory perceptual deficits in this group and consider whether these difficulties extend outside the musical domain. Finally, we review structural imaging studies in this group which point to subtle anomalies in temporal and frontal areas. We suggest that amusia can be considered a disorder of neural development, which has relatively specific consequences at the behavioral level. Studies of congenital amusia provide a unique window on the neurocognitive architecture of music processing. Copyright © 2013 Elsevier B.V. All rights reserved.

  20. Attending to auditory memory.

    Science.gov (United States)

    Zimmermann, Jacqueline F; Moscovitch, Morris; Alain, Claude

    2016-06-01

    Attention to memory describes the process of attending to memory traces when the object is no longer present. It has been studied primarily for representations of visual stimuli with only few studies examining attention to sound object representations in short-term memory. Here, we review the interplay of attention and auditory memory with an emphasis on 1) attending to auditory memory in the absence of related external stimuli (i.e., reflective attention) and 2) effects of existing memory on guiding attention. Attention to auditory memory is discussed in the context of change deafness, and we argue that failures to detect changes in our auditory environments are most likely the result of a faulty comparison system of incoming and stored information. Also, objects are the primary building blocks of auditory attention, but attention can also be directed to individual features (e.g., pitch). We review short-term and long-term memory guided modulation of attention based on characteristic features, location, and/or semantic properties of auditory objects, and propose that auditory attention to memory pathways emerge after sensory memory. A neural model for auditory attention to memory is developed, which comprises two separate pathways in the parietal cortex, one involved in attention to higher-order features and the other involved in attention to sensory information. This article is part of a Special Issue entitled SI: Auditory working memory. Copyright © 2015 Elsevier B.V. All rights reserved.

  1. Otalgia and eschar in the external auditory canal in scrub typhus complicated by acute respiratory distress syndrome and multiple organ failure

    Directory of Open Access Journals (Sweden)

    Hu Sung-Yuan

    2011-03-01

    Full Text Available Abstract Background Scrub typhus, a mite-transmitted zoonosis caused by Orientia tsutsugamushi, is an endemic disease in Taiwan and may be potentially fatal if diagnosis is delayed. Case presentations We encountered a 23-year-old previously healthy Taiwanese male soldier presenting with the right ear pain after training in the jungle and an eleven-day history of intermittent high fever up to 39°C. Amoxicillin/clavulanate was prescribed for otitis media at a local clinic. Skin rash over whole body and abdominal cramping pain with watery diarrhea appeared on the sixth day of fever. He was referred due to progressive dyspnea and cough for 4 days prior to admission in our institution. On physical examination, there were cardiopulmonary distress, icteric sclera, an eschar in the right external auditory canal and bilateral basal rales. Laboratory evaluation revealed thrombocytopenia, elevation of liver function and acute renal failure. Chest x-ray revealed bilateral diffuse infiltration. Doxycycline was prescribed for scrub typhus with acute respiratory distress syndrome and multiple organ failure. Fever subsided dramatically the next day and he was discharged on day 7 with oral tetracycline for 7 days. Conclusion Scrub typhus should be considered in acutely febrile patients with multiple organ involvement, particularly if there is an eschar or a history of environmental exposure in endemic areas. Rapid and accurate diagnosis, timely administration of antibiotics and intensive supportive care are necessary to decrease mortality of serious complications of scrub typhus.

  2. External auditory exostoses in the Xuchang and Xujiayao human remains: Patterns and implications among eastern Eurasian Middle and Late Pleistocene crania.

    Science.gov (United States)

    Trinkaus, Erik; Wu, Xiu-Jie

    2017-01-01

    In the context of Middle and Late Pleistocene eastern Eurasian human crania, the external auditory exostoses (EAE) of the late archaic Xuchang 1 and 2 and the Xujiayao 15 early Late Pleistocene human temporal bones are described. Xujiayao 15 has small EAE (Grade 1), Xuchang 1 presents bilateral medium EAE (Grade 2), and Xuchang 2 exhibits bilaterally large EAE (Grade 3), especially on the right side. These cranial remains join the other eastern Eurasian later Pleistocene humans in providing frequencies of 61% (N = 18) and 58% (N = 12) respectively for archaic and early modern human samples. These values are near the upper limits of recent human frequencies, and they imply frequent aquatic exposure among these Pleistocene humans. In addition, the medial extents of the Xuchang 1 and 2 EAE would have impinged on their tympanic membranes, and the large EAE of Xuchang 2 would have resulted in cerumen impaction. Both effects would have produced conductive hearing loss, a serious impairment in a Pleistocene foraging context.

  3. Postoperative Intensity-Modulated Radiotherapy for Squamous Cell Carcinoma of the External Auditory Canal and Middle Ear: Treatment Outcomes, Marginal Misses, and Perspective on Target Delineation

    Energy Technology Data Exchange (ETDEWEB)

    Chen, Wan-Yu [Division of Radiation Oncology, Department of Oncology, National Taiwan University Hospital, Taipei, Taiwan (China); Kuo, Sung-Hsin [Division of Radiation Oncology, Department of Oncology, National Taiwan University Hospital, Taipei, Taiwan (China); Graduate Institute of Clinical Medicine, National Taiwan University College of Medicine, Taipei, Taiwan (China); Chen, Yu-Hsuan; Lu, Szu-Huai; Tsai, Chiao-Ling [Division of Radiation Oncology, Department of Oncology, National Taiwan University Hospital, Taipei, Taiwan (China); Chia-Hsien Cheng, Jason [Division of Radiation Oncology, Department of Oncology, National Taiwan University Hospital, Taipei, Taiwan (China); Graduate Institute of Clinical Medicine, National Taiwan University College of Medicine, Taipei, Taiwan (China); Graduate Institute of Oncology, National Taiwan University College of Medicine, Taipei, Taiwan (China); Hong, Ruey-Long [Division of Medical Oncology, Department of Oncology, National Taiwan University Hospital, Taipei, Taiwan (China); Chen, Ya-Fang [Department of Medical Imaging, National Taiwan University Hospital, Taipei, Taiwan (China); Hsu, Chuan-Jen; Lin, Kai-Nan; Ko, Jenq-Yuh; Lou, Pei-Jen; Wang, Cheng-Ping [Department of Otolaryngology, National Taiwan University Hospital, Taipei, Taiwan (China); Chong, Fok-Ching [Graduate Institute of Electrical Engineering, National Taiwan University, Taipei, Taiwan (China); Wang, Chun-Wei, E-mail: cwwang@ntuh.gov.tw [Graduate Institute of Electrical Engineering, National Taiwan University, Taipei, Taiwan (China)

    2012-03-15

    Purpose: To report outcomes of the rare disease of squamous cell carcinoma (SCC) of the external auditory canal (EAC) and middle ear treated with surgery and postoperative intensity-modulated radiotherapy (IMRT). Failure patterns related to spatial dose distribution were also analyzed to provide insight into target delineation. Methods and Materials: A retrospective review was conducted of the records of 11 consecutive patients with SCC of the EAC and middle ear who were treated with curative surgery and postoperative IMRT at one institution between January 2007 and February 2010. The prescribed IMRT dose was 60 to 66 Gy at 2 Gy per fraction. Three patients also received concurrent cisplatin-based chemotherapy, and 1 patient received concurrent oral tegafur/uracil. The median follow-up time was 19 months (range, 6-33 months). Results: Four patients had locoregional recurrence, yielding an estimated 2-year locoregional control rate of 70.7%. Among them, 1 patient had persistent disease after treatment, and 3 had marginal recurrence. Distant metastasis occurred in 1 patient after extensive locoregional recurrence, yielding an estimated 2-year distant control rate of 85.7%. The estimated 2-year overall survival was 67.5%. The three cases of marginal recurrence were near the preauricular space and glenoid fossa of the temporomandibular joint, adjacent to the apex of the ear canal and glenoid fossa of the temporomandibular joint, and in the postauricular subcutaneous area and ipsilateral parotid nodes, respectively. Conclusions: Marginal misses should be recognized to improve target delineation. When treating SCC of the EAC and middle ear, care should be taken to cover the glenoid fossa of the temporomandibular joint and periauricular soft tissue. Elective ipsilateral parotid irradiation should be considered. The treatment planning procedure should also be refined to balance subcutaneous soft-tissue dosimetry and toxicity.

  4. Postoperative Intensity-Modulated Radiotherapy for Squamous Cell Carcinoma of the External Auditory Canal and Middle Ear: Treatment Outcomes, Marginal Misses, and Perspective on Target Delineation

    International Nuclear Information System (INIS)

    Chen, Wan-Yu; Kuo, Sung-Hsin; Chen, Yu-Hsuan; Lu, Szu-Huai; Tsai, Chiao-Ling; Chia-Hsien Cheng, Jason; Hong, Ruey-Long; Chen, Ya-Fang; Hsu, Chuan-Jen; Lin, Kai-Nan; Ko, Jenq-Yuh; Lou, Pei-Jen; Wang, Cheng-Ping; Chong, Fok-Ching; Wang, Chun-Wei

    2012-01-01

    Purpose: To report outcomes of the rare disease of squamous cell carcinoma (SCC) of the external auditory canal (EAC) and middle ear treated with surgery and postoperative intensity-modulated radiotherapy (IMRT). Failure patterns related to spatial dose distribution were also analyzed to provide insight into target delineation. Methods and Materials: A retrospective review was conducted of the records of 11 consecutive patients with SCC of the EAC and middle ear who were treated with curative surgery and postoperative IMRT at one institution between January 2007 and February 2010. The prescribed IMRT dose was 60 to 66 Gy at 2 Gy per fraction. Three patients also received concurrent cisplatin-based chemotherapy, and 1 patient received concurrent oral tegafur/uracil. The median follow-up time was 19 months (range, 6–33 months). Results: Four patients had locoregional recurrence, yielding an estimated 2-year locoregional control rate of 70.7%. Among them, 1 patient had persistent disease after treatment, and 3 had marginal recurrence. Distant metastasis occurred in 1 patient after extensive locoregional recurrence, yielding an estimated 2-year distant control rate of 85.7%. The estimated 2-year overall survival was 67.5%. The three cases of marginal recurrence were near the preauricular space and glenoid fossa of the temporomandibular joint, adjacent to the apex of the ear canal and glenoid fossa of the temporomandibular joint, and in the postauricular subcutaneous area and ipsilateral parotid nodes, respectively. Conclusions: Marginal misses should be recognized to improve target delineation. When treating SCC of the EAC and middle ear, care should be taken to cover the glenoid fossa of the temporomandibular joint and periauricular soft tissue. Elective ipsilateral parotid irradiation should be considered. The treatment planning procedure should also be refined to balance subcutaneous soft-tissue dosimetry and toxicity.

  5. DESCRIPTION OF BRAINSTEM AUDITORY EVOKED RESPONSES (AIR AND BONE CONDUCTION IN CHILDREN WITH NORMAL HEARING

    Directory of Open Access Journals (Sweden)

    A. V. Pashkov

    2014-01-01

    Full Text Available Diagnosis of hearing level in small children with conductive hearing loss associated with congenital craniofacial abnormalities, particularly with agenesis of external ear and external auditory meatus is a pressing issue. Conventional methods of assessing hearing in the first years of life, i. e. registration of brainstem auditory evoked responses to acoustic stimuli in the event of air conduction, does not give an indication of the auditory analyzer’s condition due to potential conductive hearing loss in these patients. This study was aimed at assessing potential of diagnosing the auditory analyzer’s function with registering brainstem auditory evoked responses (BAERs to acoustic stimuli transmitted by means of a bone vibrator. The study involved 17 children aged 3–10 years with normal hearing. We compared parameters of registering brainstem auditory evoked responses (peak V depending on the type of stimulus transmission (air/bone in children with normal hearing. The data on thresholds of the BAERs registered to acoustic stimuli in the event of air and bone conduction obtained in this study are comparable; hearing thresholds in the event of acoustic stimulation by means of a bone vibrator correlates with the results of the BAERs registered to the stimuli transmitted by means of air conduction earphones (r = 0.9. High correlation of thresholds of BAERs to the stimuli transmitted by means of a bone vibrator with thresholds of BAERs registered when air conduction earphones were used helps to assess auditory analyzer’s condition in patients with any form of conductive hearing loss.  

  6. Auditory agnosia.

    Science.gov (United States)

    Slevc, L Robert; Shell, Alison R

    2015-01-01

    Auditory agnosia refers to impairments in sound perception and identification despite intact hearing, cognitive functioning, and language abilities (reading, writing, and speaking). Auditory agnosia can be general, affecting all types of sound perception, or can be (relatively) specific to a particular domain. Verbal auditory agnosia (also known as (pure) word deafness) refers to deficits specific to speech processing, environmental sound agnosia refers to difficulties confined to non-speech environmental sounds, and amusia refers to deficits confined to music. These deficits can be apperceptive, affecting basic perceptual processes, or associative, affecting the relation of a perceived auditory object to its meaning. This chapter discusses what is known about the behavioral symptoms and lesion correlates of these different types of auditory agnosia (focusing especially on verbal auditory agnosia), evidence for the role of a rapid temporal processing deficit in some aspects of auditory agnosia, and the few attempts to treat the perceptual deficits associated with auditory agnosia. A clear picture of auditory agnosia has been slow to emerge, hampered by the considerable heterogeneity in behavioral deficits, associated brain damage, and variable assessments across cases. Despite this lack of clarity, these striking deficits in complex sound processing continue to inform our understanding of auditory perception and cognition. © 2015 Elsevier B.V. All rights reserved.

  7. Auditory Neural Prostheses – A Window to the Future

    Directory of Open Access Journals (Sweden)

    Mohan Kameshwaran

    2015-06-01

    Full Text Available Hearing loss is one of the commonest congenital anomalies to affect children world-over. The incidence of congenital hearing loss is more pronounced in developing countries like the Indian sub-continent, especially with the problems of consanguinity. Hearing loss is a double tragedy, as it leads to not only deafness but also language deprivation. However, hearing loss is the only truly remediable handicap, due to remarkable advances in biomedical engineering and surgical techniques. Auditory neural prostheses help to augment or restore hearing by integration of an external circuitry with the peripheral hearing apparatus and the central circuitry of the brain. A cochlear implant (CI is a surgically implantable device that helps restore hearing in patients with severe-profound hearing loss, unresponsive to amplification by conventional hearing aids. CIs are electronic devices designed to detect mechanical sound energy and convert it into electrical signals that can be delivered to the coch­lear nerve, bypassing the damaged hair cells of the coch­lea. The only true prerequisite is an intact auditory nerve. The emphasis is on implantation as early as possible to maximize speech understanding and perception. Bilateral CI has significant benefits which include improved speech perception in noisy environments and improved sound localization. Presently, the indications for CI have widened and these expanded indications for implantation are related to age, additional handicaps, residual hearing, and special etiologies of deafness. Combined electric and acoustic stimulation (EAS / hybrid device is designed for individuals with binaural low-frequency hearing and severe-to-profound high-frequency hearing loss. Auditory brainstem implantation (ABI is a safe and effective means of hearing rehabilitation in patients with retrocochlear disorders, such as neurofibromatosis type 2 (NF2 or congenital cochlear nerve aplasia, wherein the cochlear nerve is damaged

  8. Contribution of bioanthropology to the reconstruction of prehistoric productive processes. The external auditory exostoses in the prehispanic population of Gran Canaria

    Directory of Open Access Journals (Sweden)

    Velasco Vázquez, Javier

    2001-06-01

    Full Text Available The aim of this paper is an approach to the role of bioanthropological studies in the reconstruction of the productive processes of past societies. This objective is obtained starting from the survey and valuation of the prevalence of bone exostoses in the auditory canal among the prehistoric inhabitants of Gran Canaria. The auditory exostose is a bone wound well documented through clinical and experimental studies, closely related to the exposure of the auditory canal to cold water. The estimation of this bone anomaly among the analysed population, leads to the definition of outstanding territorial variations in the economic strategies of these human groups.

    En el presente trabajo se pretende abordar el papel de los estudios bioantropológicos en la reconstrucción de los procesos productivos de las sociedades del pasado. Esta finalidad es perseguida a partir del examen y valoración de la prevalencia de exostosis óseas en el canal auditivo en la población prehistórica de Gran Canaria. Las exostosis auditivas constituyen una lesión ósea, bien documentada en trabajos experimentales y clínicos, estrechamente relacionada con la exposición del canal auditivo al agua fría. La estimación de esta anormalidad ósea en el conjunto poblacional analizado permite la definición de importantes variaciones territoriales en las estrategias económicas emprendidas por estos grupos humanos.

  9. Congenital Hypothyroidism

    Science.gov (United States)

    ... Disease Featured Resource Find an Endocrinologist Search Congenital Hypothyroidism March 2012 Download PDFs English Espanol Editors Rosalind S. ... Resources MedlinePlus (NIH) Mayo Clinic What is congenital hypothyroidism? Newborn babies who are unable to make enough ...

  10. Narrow duplicated internal auditory canal: radiological findings and review of the literature

    International Nuclear Information System (INIS)

    Demir, Oezguen Ilhan; Cakmakci, Handan; Men, Sueleyman; Erdag, Taner Kemal

    2005-01-01

    Narrow duplicated internal auditory canal (IAC) is a rare malformation of the temporal bone that is associated with ipsilateral congenital sensorineural hearing loss. This may be an isolated finding or a part of a syndrome. Radiological examination should demonstrate aplasia or hypoplasia of the neural components of the narrow IAC, to guide the surgical approach. We report a 7-year-old boy with Klippel-Feil syndrome with a narrow double IAC with no sensorineural hearing loss but with conductive hearing loss. In this patient, the IAC consisted of two separate narrow bony canals clearly seen on 3D temporal bone CT and one nerve that was delineated on MRI. The contralateral external auditory canal was stenotic and the ossicles were dysplastic. (orig.)

  11. Antenatal diagnosis of congenital deafness.

    Science.gov (United States)

    Isaacson, G

    1988-01-01

    Advances in the field of antenatal diagnosis have made possible the detection of profound sensorineural hearing loss prior to birth. Fetal motion in response to sound and auditory evoked potential testing can determine the presence of fetal hearing in the third trimester of pregnancy. Imaging modalities including ultrasound, computed tomography, and magnetic resonance imaging hold promise for the diagnosis of some forms of congenital deafness in the second trimester fetus. The methods by which congenital deafness soon may be diagnosed and the implications for the otologist are discussed.

  12. Audiotactile integration is reduced in congenital blindness in a spatial ventriloquism task.

    Science.gov (United States)

    Occelli, Valeria; Bruns, Patrick; Zampini, Massimiliano; Röder, Brigitte

    2012-01-01

    In the ventriloquism effect, the presentation of spatially discrepant visual information biases the localization of simultaneously presented sounds. Recently, an analogous spatial influence of touch on audition has been observed. By manipulating hand posture, it has been demonstrated that this audiotactile ventriloquist effect predominantly operates in an external frame of reference. In the present study, we examined the contribution of developmental vision to audiotactile interactions as indicated by the ventriloquism effect. Congenitally blind, late blind and sighted adults were asked to report the perceived location of sounds presented from a left, a central or a right location. Auditory stimuli were either delivered alone or concurrently with touches at the left or the right hand. The hands were located to the right and to the left of the lateral speakers and participants either adopted an uncrossed or a crossed hand posture. While sighted controls and late blind participants similarly mislocalized auditory stimuli toward the concurrent tactile stimuli in bimodal trials, the congenitally blind showed a reduced ventriloquism effect. All groups showed a reduced audiotactile ventriloquism effect in the crossed hand condition. However, the magnitude of the reduction was significantly larger in the group of congenitally blind than in the group of sighted controls. These results suggest reduced audio-tactile interactions in spatial processing following a lack of visual input from birth. Copyright © 2011 Elsevier Ltd. All rights reserved.

  13. Auditory Neuropathy

    Science.gov (United States)

    ... children and adults with auditory neuropathy. Cochlear implants (electronic devices that compensate for damaged or nonworking parts ... and Drug Administration: Information on Cochlear Implants Telecommunications Relay Services Your Baby's Hearing Screening News Deaf health ...

  14. Auditory hallucinations.

    Science.gov (United States)

    Blom, Jan Dirk

    2015-01-01

    Auditory hallucinations constitute a phenomenologically rich group of endogenously mediated percepts which are associated with psychiatric, neurologic, otologic, and other medical conditions, but which are also experienced by 10-15% of all healthy individuals in the general population. The group of phenomena is probably best known for its verbal auditory subtype, but it also includes musical hallucinations, echo of reading, exploding-head syndrome, and many other types. The subgroup of verbal auditory hallucinations has been studied extensively with the aid of neuroimaging techniques, and from those studies emerges an outline of a functional as well as a structural network of widely distributed brain areas involved in their mediation. The present chapter provides an overview of the various types of auditory hallucination described in the literature, summarizes our current knowledge of the auditory networks involved in their mediation, and draws on ideas from the philosophy of science and network science to reconceptualize the auditory hallucinatory experience, and point out directions for future research into its neurobiologic substrates. In addition, it provides an overview of known associations with various clinical conditions and of the existing evidence for pharmacologic and non-pharmacologic treatments. © 2015 Elsevier B.V. All rights reserved.

  15. Congenital and perinatal cytomegalovirus infection

    Directory of Open Access Journals (Sweden)

    Chun Soo Kim

    2010-01-01

    Full Text Available Cytomegalovirus (CMV is currently the most common agent of congenital infection and the leading infectious cause of brain damage and hearing loss in children. Symptomatic congenital CMV infections usually result from maternal primary infection during early pregnancy. One half of symptomatic infants have cytomegalic inclusion disease (CID, which is characterized by involvement of multiple organs, in particular, the reticuloendothelial and central nervous system (CNS. Moreover, such involvement may or may not include ocular and auditory damage. Approximately 90% of infants with congenital infection are asymptomatic at birth. Preterm infants with perinatal CMV infection can have symptomatic diseases such as pneumonia, hepatitis, and thrombocytopenia. Microcephaly and abnormal neuroradiologic imaging are associated with a poor prognosis. Hearing loss may occur in both symptomatic and asymptomatic infants with congenital infection and may progress through childhood. Congenital infection is defined by the isolation of CMV from infants within the first 3 weeks of life. Ganciclovir therapy can be considered for infants with symptomatic congenital CMV infection involving the CNS. Pregnant women of seronegative state should be counseled on the importance of good hand washing and other control measures to prevent CMV infection. Heat treatment of infected breast milk at 72?#608;for 5 seconds can eliminate CMV completely.

  16. Neural correlates of olfactory processing in congenital blindness

    DEFF Research Database (Denmark)

    Kupers, R; Beaulieu-Lefebvre, M; Schneider, F C

    2011-01-01

    Adaptive neuroplastic changes have been well documented in congenitally blind individuals for the processing of tactile and auditory information. By contrast, very few studies have investigated olfactory processing in the absence of vision. There is ample evidence that the olfactory system...... magnetic resonance imaging to measure changes in the blood-oxygenation level-dependent signal in congenitally blind and blindfolded sighted control subjects during a simple odor detection task. We found several group differences in task-related activations. Compared to sighted controls, congenitally blind......, linking it also to olfactory processing in addition to tactile and auditory processing....

  17. Congenital tuberculosis

    African Journals Online (AJOL)

    Prof Ezechukwu

    2012-06-20

    Jun 20, 2012 ... Key words: Congenital tuberculo- sis, case report, miliary tuberculosis. Introduction. Congenital tuberculosis defines tuberculosis in infants of .... tary TB and otitis media, resulting in seizures, deafness, and death. It is therefore not surprising that the index case who presented at twelve weeks of age, had ...

  18. Congenital Abnormalities

    Science.gov (United States)

    ... tube defects. However, there is also a genetic influence to this type of congenital anomaly. Unknown Causes The vast majority of congenital abnormalities have no known cause. This is particularly troubling for parents who plan to have more children, because there is no way to predict if ...

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

    Science.gov (United States)

    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. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

  20. Congenital rubella

    Science.gov (United States)

    ... that usually closes shortly after birth remains open ( patent ductus arteriosus ) Narrowing of the large artery that ... prior to pregnancy can prevent congenital rubella. Pregnant women who have not had the vaccine should avoid ...

  1. Auditory agnosia due to long-term severe hydrocephalus caused by spina bifida - specific auditory pathway versus nonspecific auditory pathway.

    Science.gov (United States)

    Zhang, Qing; Kaga, Kimitaka; Hayashi, Akimasa

    2011-07-01

    A 27-year-old female showed auditory agnosia after long-term severe hydrocephalus due to congenital spina bifida. After years of hydrocephalus, she gradually suffered from hearing loss in her right ear at 19 years of age, followed by her left ear. During the time when she retained some ability to hear, she experienced severe difficulty in distinguishing verbal, environmental, and musical instrumental sounds. However, her auditory brainstem response and distortion product otoacoustic emissions were largely intact in the left ear. Her bilateral auditory cortices were preserved, as shown by neuroimaging, whereas her auditory radiations were severely damaged owing to progressive hydrocephalus. Although she had a complete bilateral hearing loss, she felt great pleasure when exposed to music. After years of self-training to read lips, she regained fluent ability to communicate. Clinical manifestations of this patient indicate that auditory agnosia can occur after long-term hydrocephalus due to spina bifida; the secondary auditory pathway may play a role in both auditory perception and hearing rehabilitation.

  2. The Effect of Early Visual Deprivation on the Neural Bases of Auditory Processing.

    Science.gov (United States)

    Guerreiro, Maria J S; Putzar, Lisa; Röder, Brigitte

    2016-02-03

    Transient congenital visual deprivation affects visual and multisensory processing. In contrast, the extent to which it affects auditory processing has not been investigated systematically. Research in permanently blind individuals has revealed brain reorganization during auditory processing, involving both intramodal and crossmodal plasticity. The present study investigated the effect of transient congenital visual deprivation on the neural bases of auditory processing in humans. Cataract-reversal individuals and normally sighted controls performed a speech-in-noise task while undergoing functional magnetic resonance imaging. Although there were no behavioral group differences, groups differed in auditory cortical responses: in the normally sighted group, auditory cortex activation increased with increasing noise level, whereas in the cataract-reversal group, no activation difference was observed across noise levels. An auditory activation of visual cortex was not observed at the group level in cataract-reversal individuals. The present data suggest prevailing auditory processing advantages after transient congenital visual deprivation, even many years after sight restoration. The present study demonstrates that people whose sight was restored after a transient period of congenital blindness show more efficient cortical processing of auditory stimuli (here speech), similarly to what has been observed in congenitally permanently blind individuals. These results underscore the importance of early sensory experience in permanently shaping brain function. Copyright © 2016 the authors 0270-6474/16/361620-11$15.00/0.

  3. Tactile feedback improves auditory spatial localization

    Directory of Open Access Journals (Sweden)

    Monica eGori

    2014-10-01

    Full Text Available Our recent studies suggest that congenitally blind adults have severely impaired thresholds in an auditory spatial-bisection task, pointing to the importance of vision in constructing complex auditory spatial maps (Gori et al., 2014. To explore strategies that may improve the auditory spatial sense in visually impaired people, we investigated the impact of tactile feedback on spatial auditory localization in 48 blindfolded sighted subjects. We measured auditory spatial bisection thresholds before and after training, either with tactile feedback, verbal feedback or no feedback. Audio thresholds were first measured with a spatial bisection task: subjects judged whether the second sound of a three sound sequence was spatially closer to the first or the third sound. The tactile-feedback group underwent two audio-tactile feedback sessions of 100 trials, where each auditory trial was followed by the same spatial sequence played on the subject’s forearm; auditory spatial bisection thresholds were evaluated after each session. In the verbal-feedback condition, the positions of the sounds were verbally reported to the subject after each feedback trial. The no-feedback group did the same sequence of trials, with no feedback. Performance improved significantly only after audio-tactile feedback. The results suggest that direct tactile feedback interacts with the auditory spatial localization system, possibly by a process of cross-sensory recalibration. Control tests with the subject rotated suggested that this effect occurs only when the tactile and acoustic sequences are spatially coherent. Our results suggest that the tactile system can be used to recalibrate the auditory sense of space. These results encourage the possibility of designing rehabilitation programs to help blind persons establish a robust auditory sense of space, through training with the tactile modality.

  4. Neurobiology of Congenital Amusia.

    Science.gov (United States)

    Peretz, Isabelle

    2016-11-01

    The past decade of research has provided compelling evidence that musical engagement is a fundamental human trait, and its biological basis is increasingly scrutinized. In this endeavor, the detailed study of individuals who have musical deficiencies is instructive because of likely neurogenetic underpinnings. Such individuals have 'congenital amusia', an umbrella term for lifelong musical disabilities that cannot be attributed to intellectual disability, lack of exposure, or brain damage after birth. Key points are reviewed here that have emerged during recent years regarding the neurobiology of the disorder, focusing on the importance of recurrent processing between the right inferior frontal cortex and the auditory cortex for conscious monitoring of musical pitch, and how this relates to developmental cognitive disorders in general. Copyright © 2016 Elsevier Ltd. All rights reserved.

  5. Congenital sensorineural hearing loss

    International Nuclear Information System (INIS)

    Mafee, M.F.; Selis, J.E.; Yannias, D.A.; Valvassori, G.E.; Pruzansky, S.; Applebaum, E.L.; Capek, V.

    1984-01-01

    The ears of 47 selected patients with congenital sensorineural hearing loss were examined with complex-motion tomography. The patients were divided into 3 general categories: those with a recognized syndrome, those with sensorineural hearing loss unrelated to any known syndrome, and those with microtia. A great variety of inner ear anomalies was detected, but rarely were these characteristic of a particular clinical entity. The most common finding was the Mondini malformation or one of its variants. Isolated dysplasia of the internal auditory canal or the vestibular aqueduct may be responsible for sensorineural hearing loss in some patients. Patients with microtia may also have severe inner ear abnormalities despite the fact that the outer and inner ears develop embryologically from completely separate systems

  6. Congenital sensorineural hearing loss

    Energy Technology Data Exchange (ETDEWEB)

    Mafee, M.F.; Selis, J.E.; Yannias, D.A.; Valvassori, G.E.; Pruzansky, S.; Applebaum, E.L.; Capek, V.

    1984-02-01

    The ears of 47 selected patients with congenital sensorineural hearing loss were examined with complex-motion tomography. The patients were divided into 3 general categories: those with a recognized syndrome, those with sensorineural hearing loss unrelated to any known syndrome, and those with microtia. A great variety of inner ear anomalies was detected, but rarely were these characteristic of a particular clinical entity. The most common finding was the Mondini malformation or one of its variants. Isolated dysplasia of the internal auditory canal or the vestibular aqueduct may be responsible for sensorineural hearing loss in some patients. Patients with microtia may also have severe inner ear abnormalities despite the fact that the outer and inner ears develop embryologically from completely separate systems.

  7. Congenital lymphoedema of the genitalia

    NARCIS (Netherlands)

    Bolt, R. J.; Peelen, W.; Nikkels, P. G.; de Jong, T. P.

    1998-01-01

    Isolated congenital lymphoedema of the external genitalia in boys is extremely rare. It can have major physical and emotional consequences for the children. Three male patients with primary lymphoedema of the penis and scrotum are described. The first case presented with lymphoedema of the prepuce

  8. Reduced taste sensitivity in congenital blindness

    DEFF Research Database (Denmark)

    Gagnon, Lea; Kupers, Ron; Ptito, Maurice

    2013-01-01

    behavioral results showed that compared with the normal sighted, blind subjects have increased thresholds for taste detection and taste identification. This finding is at odds with the superior performance of congenitally blind subjects in several tactile, auditory and olfactory tasks. Our psychometric data...... thresholds of the 5 basic tastants in 13 congenitally blind and 13 sighted control subjects. Participants also answered several eating habits questionnaires, including the Food Neophobia Scale, the Food Variety Seeking Tendency Scale, the Intuitive Eating Scale, and the Body Awareness Questionnaire. Our...

  9. Sound Localization in Patients With Congenital Unilateral Conductive Hearing Loss With a Transcutaneous Bone Conduction Implant.

    Science.gov (United States)

    Vyskocil, Erich; Liepins, Rudolfs; Kaider, Alexandra; Blineder, Michaela; Hamzavi, Sasan

    2017-03-01

    There is no consensus regarding the benefit of implantable hearing aids in congenital unilateral conductive hearing loss (UCHL). This study aimed to measure sound source localization performance in patients with congenital UCHL and contralateral normal hearing who received a new bone conduction implant. Evaluation of within-subject performance differences for sound source localization in a horizontal plane. Tertiary referral center. Five patients with atresia of the external auditory canal and contralateral normal hearing implanted with transcutaneous bone conduction implant at the Medical University of Vienna were tested. Activated/deactivated implant. Sound source localization test; localization performance quantified using the root mean square (RMS) error. Sound source localization ability was highly variable among individual subjects, with RMS errors ranging from 21 to 40 degrees. Horizontal plane localization performance in aided conditions showed statistically significant improvement compared with the unaided conditions, with RMS errors ranging from 17 to 27 degrees. The mean RMS error decreased by a factor of 0.71 (p conduction implant. Some patients with congenital UCHL might be capable of developing improved horizontal plane localization abilities with the binaural cues provided by this device.

  10. Congenital Hydrocephalus.

    Science.gov (United States)

    Estey, Chelsie M

    2016-03-01

    There are several types of hydrocephalus, which are characterized based on the location of the cerebrospinal fluid (CSF) accumulation. Physical features of animals with congenital hydrocephalus may include a dome-shaped skull, persistent fontanelle, and bilateral ventrolateral strabismus. Medical therapy involves decreasing the production of CSF. The most common surgical treatment is placement of a ventriculoperitoneal shunt. Postoperative complications may include infection, blockage, drainage abnormalities, and mechanical failure. Copyright © 2016 Elsevier Inc. All rights reserved.

  11. Congenital diplopodia

    International Nuclear Information System (INIS)

    Brower, Jason S.; Wootton-Gorges, Sandra L.; Costouros, John G.; Boakes, Jennette; Greenspan, Adam

    2003-01-01

    Diplopodia, or duplicated foot, is a rare congenital anomaly. It differs from polydactyly in that supernumerary metatarsal and tarsal bones are present as well as extra digits. Only a few cases of this anomaly have been reported in the literature to date. We present a newborn male without intrauterine teratogen exposure who was born with a duplicate foot of the left lower extremity and imperforate anus. (orig.)

  12. Congenital toxoplasmosis.

    Science.gov (United States)

    Kieffer, François; Wallon, Martine

    2013-01-01

    Congenital toxoplasmosis results from the transplacental transmission of the parasite Toxoplasma gondii after a maternal infection acquired in pregnancy. Prevalence of congenital infection ranges from 0.1 to 0.3 per 1000 live births. The maternal-fetal transmission rate increases with gestational age at maternal seroconversion, from less than 15% at 13 weeks of gestation to over 70% at 36 weeks. Conversely, the later the maternal infection, the lower the risk of symptomatic congenital infection (infections acquired during the third trimester are most often asymptomatic at birth). Prenatal diagnosis is currently performed by PCR analysis in amniotic fluid. Antenatal management and treatment vary considerably among countries. In some European countries, maternal infections are detected through serological screening allowing a prompt treatment with spiramycin, which is expected to reduce the risk of vertical transmission. If PCR analysis in amniotic fluid is positive or if maternal infection was acquired in the third trimester of pregnancy, a combination with pyrimethamine and sulphonamide is given until delivery. Benefits of antenatal treatments remain controversial. Infected newborns are prescribed pyrimethamine and sulphonamide for 12 months. Despite antenatal and postnatal treatment, chorioretinitis can occur at any age (prevalence>20% at 10 years of age): long-term ophthalmological follow-up remains necessary. Copyright © 2013 Elsevier B.V. All rights reserved.

  13. Congenital syphilis

    International Nuclear Information System (INIS)

    Lee, Sang Wook; Kim, Kyung Soo; Hur, Don

    1983-01-01

    In recent years, marked increase in incidence of congenital syphilis has occurred throughout the world due to changes in social norms and development of penicillin-resistant strains. Early diagnosis plays an important role in congenital syphilis as the clinical manifestations may simulate many other conditions in the paediatric age group. The authors analyzed 52 cases of congenital syphilis admitted to the department of paediatrics, Chosun University Hospital, clinically and radiologically. Among them, 18 cases were born in this hospital and 34 cases were admitted from OPD, during the period of 8 years from January, 1975 to December, 1982. The results obtained were as follows; 1. In 28 of 34 cases (82%), the first clinical manifestations were below the age of 3 months. 2. Among the 52 cases, a male predominance was observed with a male to female ratio of 2 : 1. 3. The serologic test (VDRL) of the 52 studied cases showed reactive response in 49 cases (94%), and that of syphilitic mothers except 6 cases, reactive in all studied cases. 4. The major manifestations of the 52 cases were bone tenderness (12%) and swelling of the joints (7%) in skeletal system, hepatosplenomegaly (79%) and skin lesions (73%) in extraskeletal one. 5. The radiological skeletal changes were detected in 45 of 52 cases (87%), and the commonest findings were detected in 45 of 52 cases (87%), and the commonest findings were metaphysitis (83%) and periostitis (81%). The most characteristic type of metaphysitis were transverse trophic line (74%) and zone of rarefaction (65%). 6. The commonest bones to be affected were growing metaphyses of the long bones, particulary about the wrist and the knee. The order of frequency were radius (80%), uina (80%), tibia (77%), femur (69%) and humerus (40%)

  14. Motion processing after sight restoration: No competition between visual recovery and auditory compensation.

    Science.gov (United States)

    Bottari, Davide; Kekunnaya, Ramesh; Hense, Marlene; Troje, Nikolaus F; Sourav, Suddha; Röder, Brigitte

    2018-02-15

    The present study tested whether or not functional adaptations following congenital blindness are maintained in humans after sight-restoration and whether they interfere with visual recovery. In permanently congenital blind individuals both intramodal plasticity (e.g. changes in auditory cortex) as well as crossmodal plasticity (e.g. an activation of visual cortex by auditory stimuli) have been observed. Both phenomena were hypothesized to contribute to improved auditory functions. For example, it has been shown that early permanently blind individuals outperform sighted controls in auditory motion processing and that auditory motion stimuli elicit activity in typical visual motion areas. Yet it is unknown what happens to these behavioral adaptations and cortical reorganizations when sight is restored, that is, whether compensatory auditory changes are lost and to which degree visual motion processing is reinstalled. Here we employed a combined behavioral-electrophysiological approach in a group of sight-recovery individuals with a history of a transient phase of congenital blindness lasting for several months to several years. They, as well as two control groups, one with visual impairments, one normally sighted, were tested in a visual and an auditory motion discrimination experiment. Task difficulty was manipulated by varying the visual motion coherence and the signal to noise ratio, respectively. The congenital cataract-reversal individuals showed lower performance in the visual global motion task than both control groups. At the same time, they outperformed both control groups in auditory motion processing suggesting that at least some compensatory behavioral adaptation as a consequence of a complete blindness from birth was maintained. Alpha oscillatory activity during the visual task was significantly lower in congenital cataract reversal individuals and they did not show ERPs modulated by visual motion coherence as observed in both control groups. In

  15. Predictive coding of visual-auditory and motor-auditory events: An electrophysiological study.

    Science.gov (United States)

    Stekelenburg, Jeroen J; Vroomen, Jean

    2015-11-11

    The amplitude of auditory components of the event-related potential (ERP) is attenuated when sounds are self-generated compared to externally generated sounds. This effect has been ascribed to internal forward modals predicting the sensory consequences of one's own motor actions. Auditory potentials are also attenuated when a sound is accompanied by a video of anticipatory visual motion that reliably predicts the sound. Here, we investigated whether the neural underpinnings of prediction of upcoming auditory stimuli are similar for motor-auditory (MA) and visual-auditory (VA) events using a stimulus omission paradigm. In the MA condition, a finger tap triggered the sound of a handclap whereas in the VA condition the same sound was accompanied by a video showing the handclap. In both conditions, the auditory stimulus was omitted in either 50% or 12% of the trials. These auditory omissions induced early and mid-latency ERP components (oN1 and oN2, presumably reflecting prediction and prediction error), and subsequent higher-order error evaluation processes. The oN1 and oN2 of MA and VA were alike in amplitude, topography, and neural sources despite that the origin of the prediction stems from different brain areas (motor versus visual cortex). This suggests that MA and VA predictions activate a sensory template of the sound in auditory cortex. This article is part of a Special Issue entitled SI: Prediction and Attention. Copyright © 2015 Elsevier B.V. All rights reserved.

  16. Auditory Perspective Taking

    National Research Council Canada - National Science Library

    Martinson, Eric; Brock, Derek

    2006-01-01

    .... From this knowledge of another's auditory perspective, a conversational partner can then adapt his or her auditory output to overcome a variety of environmental challenges and insure that what is said is intelligible...

  17. Psycho acoustical Measures in Individuals with Congenital Visual Impairment.

    Science.gov (United States)

    Kumar, Kaushlendra; Thomas, Teenu; Bhat, Jayashree S; Ranjan, Rajesh

    2017-12-01

    In congenital visual impaired individuals one modality is impaired (visual modality) this impairment is compensated by other sensory modalities. There is evidence that visual impaired performed better in different auditory task like localization, auditory memory, verbal memory, auditory attention, and other behavioural tasks when compare to normal sighted individuals. The current study was aimed to compare the temporal resolution, frequency resolution and speech perception in noise ability in individuals with congenital visual impaired and normal sighted. Temporal resolution, frequency resolution, and speech perception in noise were measured using MDT, GDT, DDT, SRDT, and SNR50 respectively. Twelve congenital visual impaired participants with age range of 18 to 40 years were taken and equal in number with normal sighted participants. All the participants had normal hearing sensitivity with normal middle ear functioning. Individual with visual impairment showed superior threshold in MDT, SRDT and SNR50 as compared to normal sighted individuals. This may be due to complexity of the tasks; MDT, SRDT and SNR50 are complex tasks than GDT and DDT. Visual impairment showed superior performance in auditory processing and speech perception with complex auditory perceptual tasks.

  18. Auditory midbrain processing is differentially modulated by auditory and visual cortices: An auditory fMRI study.

    Science.gov (United States)

    Gao, Patrick P; Zhang, Jevin W; Fan, Shu-Juan; Sanes, Dan H; Wu, Ed X

    2015-12-01

    The cortex contains extensive descending projections, yet the impact of cortical input on brainstem processing remains poorly understood. In the central auditory system, the auditory cortex contains direct and indirect pathways (via brainstem cholinergic cells) to nuclei of the auditory midbrain, called the inferior colliculus (IC). While these projections modulate auditory processing throughout the IC, single neuron recordings have samples from only a small fraction of cells during stimulation of the corticofugal pathway. Furthermore, assessments of cortical feedback have not been extended to sensory modalities other than audition. To address these issues, we devised blood-oxygen-level-dependent (BOLD) functional magnetic resonance imaging (fMRI) paradigms to measure the sound-evoked responses throughout the rat IC and investigated the effects of bilateral ablation of either auditory or visual cortices. Auditory cortex ablation increased the gain of IC responses to noise stimuli (primarily in the central nucleus of the IC) and decreased response selectivity to forward species-specific vocalizations (versus temporally reversed ones, most prominently in the external cortex of the IC). In contrast, visual cortex ablation decreased the gain and induced a much smaller effect on response selectivity. The results suggest that auditory cortical projections normally exert a large-scale and net suppressive influence on specific IC subnuclei, while visual cortical projections provide a facilitatory influence. Meanwhile, auditory cortical projections enhance the midbrain response selectivity to species-specific vocalizations. We also probed the role of the indirect cholinergic projections in the auditory system in the descending modulation process by pharmacologically blocking muscarinic cholinergic receptors. This manipulation did not affect the gain of IC responses but significantly reduced the response selectivity to vocalizations. The results imply that auditory cortical

  19. Otologic Manifestations of Autosomal Recessive Congenital Ichthyosis in Children.

    Science.gov (United States)

    Martín-Santiago, A; Rodríguez-Pascual, M; Knöpfel, N; Hernández-Martín, Á

    2015-11-01

    Few studies have investigated ear involvement in nonsyndromic autosomal recessive congenital ichthyosis (ARCI). To assess the type and frequency of otologic manifestations of ARCI in patients under follow-up at the pediatric dermatology department of our hospital. We prospectively studied the presence of ear pain, ear itching, tinnitus, otitis, cerumen impaction, accumulation of epithelial debris, and hearing loss. Daily hygiene measures, topical treatments, medical-surgical interventions, and frequency of visits to an ear, nose, and throat (ENT) specialist were noted in the patients' medical records. Ear examination and hearing tests were performed in all cases. Ten patients were studied: 2 had a self-healing collodion baby phenotype and 8 had ichthyosis. There was mention of otologic manifestations in the records of all 8 patients with ichthyosis (100%); 6 of these patients (75%) had abnormalities in the external auditory canal examination and 2 (25%) had conductive hearing loss. Our findings are limited by the small number of patients studied, all of whom were younger than 19 years. The involvement of both dermatologists and ENT specialists in the management of patients with ichthyosis is crucial to ensure the application of the best therapeutic and preventive measures. More studies are needed to assess the prevalence and impact on quality of life of ear involvement in patients with ichthyosis and to determine the optimal interval between ENT visits for these patients. Copyright © 2015 Elsevier España, S.L.U. and AEDV. All rights reserved.

  20. Congenital Unilateral Hypoplasia of Depressor Anguli Oris

    Directory of Open Access Journals (Sweden)

    Seckin O. Ulualp

    2012-01-01

    Full Text Available Objectives. Asymmetric facial appearance may originate from abnormalities of facial musculature or facial innervation. We describe clinical features of congenital hypoplasia of depressor anguli oris muscle in a child. Material and Methods. Chart of a 10-month-old female referred to a tertiary care pediatric hospital for assessment of facial paralysis was reviewed. Data included relevant history and physical examination, diagnostic work up, and management. Results. The child presented with asymmetric movement of lower lip since birth. Asymmetry of lower lip was more pronounced when she smiled and cried. Rest of the face movement was symmetric. On examination, the face appeared symmetric at rest. The child had inward deviation of right lower lip when she smiled. Facial nerve function, as determined by frowning/forehead, wrinkling, eye closure, nasolabial fold depth, and tearing, was symmetric. Magnetic resonance imaging of the temporal bones and internal auditory canals were within normal limits. Echocardiogram did not show cardiac abnormality. Auditory brainstem response showed no abnormality. Conclusions. Congenital hypoplasia of depressor anguli oris is a rare anomaly that causes asymmetric crying face. Pediatricians and otolaryngologists need to be cognizant of cardiac, head and neck, and central nervous system anomalies associated with congenital unilateral hypoplasia of depressor anguli oris.

  1. [Congenital hypothyroidism].

    Science.gov (United States)

    Castilla Peón, María Fernanda

    Congenital hypothyroidism (CH) is a cause of preventable mental retardation; therefore, timely diagnosis and treatment by the primary care physician is very important. CH screening must be performed between the second and fifth days of life with capillary blood done with a heel prick and must be confirmed by measurement of thyroid hormones in venous blood. The most common cause of CH is thyroid dysgenesis, which may be identified by a thyroid scan carried out before initiating treatment. Treatment should be with levothyroxine (10-15μg/kg/day) and should not be delayed or suspended during the first 3 years of life due to the deleterious effect on neurodevelopment in case of low thyroid hormones during this time. Preterm or sick infants or those with Down syndrome require special consideration. This article provides diagnostic and therapeutic algorithms for CH. Copyright © 2015 Hospital Infantil de México Federico Gómez. Publicado por Masson Doyma México S.A. All rights reserved.

  2. Are Patients with Spontaneous CSF Otorrhea and Superior Canal Dehiscence Congenitally Predisposed to Their Disorders?

    Science.gov (United States)

    Stevens, Shawn M; Hock, Kiefer; Samy, Ravi N; Pensak, Myles L

    2018-04-01

    Objectives (1) Compare lateral skull base (LSB) height/thickness in patients with spontaneous cerebrospinal fluid otorrhea (CSF), superior canal dehiscence (SCD), acoustic neuromas (AN), and otosclerosis (OTO). (2) Perform correlations between age, body mass index (BMI), sex, and LSB height/thickness. Study Design Case series with chart review. Setting Tertiary referral center. Subjects and Methods Patients with CSF, SCD, AN, and OTO diagnosed from 2006 to 2016 were included if they had high-definition temporal bone computed tomography (CT) and absence of trauma, radiation, chronic ear disease, and/or congenital anomaly. CT-based measurements included LSB height/thickness and pneumatization rates overlaying the external auditory canal (EAC), tegmen tympani (TgT), perigeniculate region (PG), and internal auditory canal (IAC). LSB height/thickness, age, sex, and BMI were statistically correlated. In total, 256 patients and 493 ears (109 CSF, 115 SCD, 269 AN/OTO) were measured. Results Patients with CSF had significantly higher BMIs than the other groups ( P CSF and SCD had similar radiographic LSB phenotypes at most measured locations. Both groups exhibited a significantly lower LSB height compared to the AN and OTO groups (mean, 3.9-4.2 mm vs 4.9-5.6 mm; P CSF and SCD also demonstrated significantly lower pneumatization rates, as low as 17% to 23% overlaying the PG and IAC ( P CSF and SCD exhibit similar radiographic LSB phenotypes. Age, sex, and BMI do not significantly correlate with LSB height/thickness. These data support the theory that CSF and SCD arise via similar congenital pathoetiologic mechanisms.

  3. Congenital platelet function defects

    Science.gov (United States)

    ... pool disorder; Glanzmann's thrombasthenia; Bernard-Soulier syndrome; Platelet function defects - congenital ... Congenital platelet function defects are bleeding disorders that cause reduced platelet function. Most of the time, people with these disorders have ...

  4. Congenital Heart Information Network

    Science.gov (United States)

    ... heart defects. Important Notice The Congenital Heart Information Network website is temporarily out of service. Please join ... and Uwe Baemayr for The Congenital Heart Information Network Exempt organization under Section 501(c)3. Copyright © ...

  5. Congenital heart disease

    Science.gov (United States)

    Congenital heart disease (CHD) is a problem with the heart's structure and function that is present at birth. ... Fraser CD, Kane LC. Congenital heart disease. In: Townsend CM Jr, ... Sabiston Textbook of Surgery: The Biological Basis of Modern ...

  6. Auditory Processing Disorder (For Parents)

    Science.gov (United States)

    ... role. Auditory cohesion problems: This is when higher-level listening tasks are difficult. Auditory cohesion skills — drawing inferences from conversations, understanding riddles, or comprehending verbal math problems — require heightened auditory processing and language levels. ...

  7. Regional Externalities

    NARCIS (Netherlands)

    Heijman, W.J.M.

    2007-01-01

    The book offers practical and theoretical insights in regional externalities. Regional externalities are a specific subset of externalities that can be defined as externalities where space plays a dominant role. This class of externalities can be divided into three categories: (1) externalities

  8. Congenital Intrahepatic Portosystemic Shunts

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Woong Hee; Kim, Young Tong; Jou, Sung Shick; Shin, Hyeong Cheol [Soonchunhyang University, Asan (Korea, Republic of)

    2008-12-15

    Intrahepatic portosystemic shunts are an anomalous connection between the portal vein and hepatic vein/IVC, which may be either congenital or acquired secondary to liver cirrhosis or portal hypertension. Cases of congenital intrahepatic shunts are usually encountered in children and may spontaneously resolve. We report 5 cases of congenital intrahepatic portosystemic shunts in neonates and an adult

  9. Malignant external otitis

    International Nuclear Information System (INIS)

    Dupuch, K.M.; Iryboz, T.; Firat, M.; Levy, C.; Tubiana, J.M.

    1991-01-01

    This paper illustrates the value of CT and MR in early diagnosis and spread of malignant external otitis. The authors retrospectively analyzed 15 patients with proved malignant external otitis examined with postcontrast high-resolution CT (15/15) and MR (6/15) (T1- and T2-weighting). Gallium studies were done in 6/15 patients. Early diagnosis was made when CT demonstrated a soft-tissue mass of the external auditory canal associated with scattered zones of cortical bone erosions (13/15). Spread of the disease was better delineated by MR than CT, especially skull base extension (6/15). Temporomandibular joint involvement with extension into parotid or/and masticator spaces 6/15 was as well detected with CT as with MR. If CT remains the first and best procedure for diagnosis, MR - despite its cost - appears a good procedure to depict exact anatomic spread, allowing therapeutic management

  10. Auditory-motor learning influences auditory memory for music.

    Science.gov (United States)

    Brown, Rachel M; Palmer, Caroline

    2012-05-01

    In two experiments, we investigated how auditory-motor learning influences performers' memory for music. Skilled pianists learned novel melodies in four conditions: auditory only (listening), motor only (performing without sound), strongly coupled auditory-motor (normal performance), and weakly coupled auditory-motor (performing along with auditory recordings). Pianists' recognition of the learned melodies was better following auditory-only or auditory-motor (weakly coupled and strongly coupled) learning than following motor-only learning, and better following strongly coupled auditory-motor learning than following auditory-only learning. Auditory and motor imagery abilities modulated the learning effects: Pianists with high auditory imagery scores had better recognition following motor-only learning, suggesting that auditory imagery compensated for missing auditory feedback at the learning stage. Experiment 2 replicated the findings of Experiment 1 with melodies that contained greater variation in acoustic features. Melodies that were slower and less variable in tempo and intensity were remembered better following weakly coupled auditory-motor learning. These findings suggest that motor learning can aid performers' auditory recognition of music beyond auditory learning alone, and that motor learning is influenced by individual abilities in mental imagery and by variation in acoustic features.

  11. Impaired encoding of rapid pitch information underlies perception and memory deficits in congenital amusia

    OpenAIRE

    Philippe Albouy; Marion Cousineau; Anne Caclin; Barbara Tillmann; Isabelle Peretz

    2016-01-01

    Recent theories suggest that the basis of neurodevelopmental auditory disorders such as dyslexia or specific language impairment might be a low-level sensory dysfunction. In the present study we test this hypothesis in congenital amusia, a neurodevelopmental disorder characterized by severe deficits in the processing of pitch-based material. We manipulated the temporal characteristics of auditory stimuli and investigated the influence of the time given to encode pitch information on participa...

  12. Auditory Integration Training

    Directory of Open Access Journals (Sweden)

    Zahra Jafari

    2002-07-01

    Full Text Available Auditory integration training (AIT is a hearing enhancement training process for sensory input anomalies found in individuals with autism, attention deficit hyperactive disorder, dyslexia, hyperactivity, learning disability, language impairments, pervasive developmental disorder, central auditory processing disorder, attention deficit disorder, depressin, and hyperacute hearing. AIT, recently introduced in the United States, and has received much notice of late following the release of The Sound of a Moracle, by Annabel Stehli. In her book, Mrs. Stehli describes before and after auditory integration training experiences with her daughter, who was diagnosed at age four as having autism.

  13. Review: Auditory Integration Training

    Directory of Open Access Journals (Sweden)

    Zahra Ja'fari

    2003-01-01

    Full Text Available Auditory integration training (AIT is a hearing enhancement training process for sensory input anomalies found in individuals with autism, attention deficit hyperactive disorder, dyslexia, hyperactivity, learning disability, language impairments, pervasive developmental disorder, central auditory processing disorder, attention deficit disorder, depression, and hyper acute hearing. AIT, recently introduced in the United States, and has received much notice of late following the release of the sound of a miracle, by Annabel Stehli. In her book, Mrs. Stehli describes before and after auditory integration training experiences with her daughter, who was diagnosed at age four as having autism.

  14. Anteverted internal auditory canal as an inner ear anomaly in patients with craniofacial microsomia.

    Science.gov (United States)

    L'Heureux-Lebeau, Bénédicte; Saliba, Issam

    2014-09-01

    Craniofacial microsomia involves structure of the first and second branchial arches. A wide range of ear anomalies, affecting external, middle and inner ear, has been described in association with this condition. We report three cases of anteverted internal auditory canal in patients presenting craniofacial microsomia. This unique internal auditory canal orientation was found on high-resolution computed tomography of the temporal bones. This internal auditory canal anomaly is yet unreported in craniofacial anomalies. Copyright © 2014. Published by Elsevier Ireland Ltd.

  15. Congenital orbital teratoma

    OpenAIRE

    Aiyub, Shereen; Chan, Weng Onn; Szetu, John; Sullivan, Laurence J; Pater, John; Cooper, Peter; Selva, Dinesh

    2013-01-01

    We present a case of mature congenital orbital teratoma managed with lid-sparing exenteration and dermis fat graft. This is a case report on the management of congenital orbital teratoma. A full-term baby was born in Fiji with prolapsed right globe which was surrounded by a nonpulsatile, cystic mass. Clinical and imaging features were consistent with congenital orbital teratoma. Due to limited surgical expertise, the patient was transferred to Adelaide, Australia for further management. The p...

  16. Cytomegalovirus Congenital Cataract

    Directory of Open Access Journals (Sweden)

    Ridha Wahyutomo

    2011-06-01

    Full Text Available Cytomegalovirus congenital infection is an infection caused by the the subfamily â Herpesviridae, during pregnancy. The incidence of infections among newborn infants is 1 %. One of the effects of congenitally acquired infection is the congenital cataract. A 6-year-old child complained to have a blurred vision diagnosed with cytomegalovirus congenital cataract. The diagnosis was confirmed by a positive serology testing for Ig M and Ig G CMV. The laboratory test using Giemsa staining to find inclusion bodies and a faster PCR could not be carried out (Sains Medika, 3(1:84-88.

  17. Genetics Home Reference: congenital hypothyroidism

    Science.gov (United States)

    ... Facebook Twitter Home Health Conditions Congenital hypothyroidism Congenital hypothyroidism Printable PDF Open All Close All Enable Javascript to view the expand/collapse boxes. Description Congenital hypothyroidism is a partial or complete loss of function ...

  18. Functionally Specific Oscillatory Activity Correlates between Visual and Auditory Cortex in the Blind

    Science.gov (United States)

    Schepers, Inga M.; Hipp, Joerg F.; Schneider, Till R.; Roder, Brigitte; Engel, Andreas K.

    2012-01-01

    Many studies have shown that the visual cortex of blind humans is activated in non-visual tasks. However, the electrophysiological signals underlying this cross-modal plasticity are largely unknown. Here, we characterize the neuronal population activity in the visual and auditory cortex of congenitally blind humans and sighted controls in a…

  19. Anatomic measurements of the posterior tympanum related to the round window vibroplasty in congenital aural atresia and stenosis patients.

    Science.gov (United States)

    Chen, Keguang; Yin, Dongming; Lyu, Huiying; Yang, Lin; Zhang, Tianyu; Dai, Peidong

    2016-01-01

    With the aggravation of the external auditory canal malformation, the size of extra-niche fossa became smaller, providing concrete data and valuable information for the better design, selecting and safer implantation of the transducer in the area of round window niche. Three-dimensional measurements and assessments before surgery might be helpful for a safer surgical approach and implantation of a vibrant soundbridge. The aim of this study was to investigate whether differences exist in the morphology of the posterior tympanum related to the round window vibroplasty among congenital aural atresia (CAA), congenital aural stenosis (CAS), and a normal control group, and to analyze its effect on the round window implantation of vibrant soundbridge. CT images of 10 normal subjects (20 ears), 27 CAS patients (30 ears), and 25 CAA patients (30 ears) were analyzed. The depth and the size of outside fossa of round window niche related to the round window vibroplasty (extra-niche fossa)and the distances between the center of round window niche and extra-niche fossa were calculated based on three-dimensional reconstruction using mimics software. Finally, the data were analyzed statistically. The size of extra-niche fossa in the atresia group was smaller than in the stenosis group (p < 0.05); furthermore, the size of extra-niche fossa in the stenosis group was smaller than that of the control group (p < 0.05). There was no statistically significant difference of the depth of extra-niche fossa among different groups.

  20. Auditory maturation and congenital hearing loss in NICU infants

    OpenAIRE

    Coenraad, Saskia

    2011-01-01

    textabstractThe number of preterm births has increased over the past decades as a result of increasing maternal age and in vitro fertilization (1). At the same time the survival of preterm infants has increased due to advances in perinatal and neonatal care. For example, antenatal corticosteroids for women with threatened preterm delivery, high-frequency oscillatory ventilation and inhaled nitric oxide have now become standard therapy (1). Unfortunately, these improvements sometimes come at a...

  1. Auditory maturation and congenital hearing loss in NICU infants

    NARCIS (Netherlands)

    S. Coenraad (Saskia)

    2011-01-01

    textabstractThe number of preterm births has increased over the past decades as a result of increasing maternal age and in vitro fertilization (1). At the same time the survival of preterm infants has increased due to advances in perinatal and neonatal care. For example, antenatal corticosteroids

  2. Auditory Spatial Layout

    Science.gov (United States)

    Wightman, Frederic L.; Jenison, Rick

    1995-01-01

    All auditory sensory information is packaged in a pair of acoustical pressure waveforms, one at each ear. While there is obvious structure in these waveforms, that structure (temporal and spectral patterns) bears no simple relationship to the structure of the environmental objects that produced them. The properties of auditory objects and their layout in space must be derived completely from higher level processing of the peripheral input. This chapter begins with a discussion of the peculiarities of acoustical stimuli and how they are received by the human auditory system. A distinction is made between the ambient sound field and the effective stimulus to differentiate the perceptual distinctions among various simple classes of sound sources (ambient field) from the known perceptual consequences of the linear transformations of the sound wave from source to receiver (effective stimulus). Next, the definition of an auditory object is dealt with, specifically the question of how the various components of a sound stream become segregated into distinct auditory objects. The remainder of the chapter focuses on issues related to the spatial layout of auditory objects, both stationary and moving.

  3. Mitochondrial disorders in congenital myopathies

    Directory of Open Access Journals (Sweden)

    D. A. Kharlamov

    2014-01-01

    Full Text Available The literature review gives data on the role of mitochondrial disorders in the pathogenesis of congenital myopathies: congenital muscular dystrophies and congenital structural myopathies. It describes changes in congenital muscular dystrophies with type VI collagen, in myodystrophy with giant mitochondria, in congenital central core myopathies, myotubular myopathy, etc. Clinical and experimental findings are presented. Approaches to therapy for energy disorders in congenital myopathies are depicted.

  4. Impaired encoding of rapid pitch information underlies perception and memory deficits in congenital amusia.

    Science.gov (United States)

    Albouy, Philippe; Cousineau, Marion; Caclin, Anne; Tillmann, Barbara; Peretz, Isabelle

    2016-01-06

    Recent theories suggest that the basis of neurodevelopmental auditory disorders such as dyslexia or specific language impairment might be a low-level sensory dysfunction. In the present study we test this hypothesis in congenital amusia, a neurodevelopmental disorder characterized by severe deficits in the processing of pitch-based material. We manipulated the temporal characteristics of auditory stimuli and investigated the influence of the time given to encode pitch information on participants' performance in discrimination and short-term memory. Our results show that amusics' performance in such tasks scales with the duration available to encode acoustic information. This suggests that in auditory neuro-developmental disorders, abnormalities in early steps of the auditory processing can underlie the high-level deficits (here musical disabilities). Observing that the slowing down of temporal dynamics improves amusics' pitch abilities allows considering this approach as a potential tool for remediation in developmental auditory disorders.

  5. Mondini dysplasia and congenital cytomegalovirus infection.

    Science.gov (United States)

    Bauman, N M; Kirby-Keyser, L J; Dolan, K D; Wexler, D; Gantz, B J; McCabe, B F; Bale, J F

    1994-01-01

    We report a case of bilateral temporal bone anomalies in a child with symptomatic congenital cytomegalovirus infection and severe, bilateral sensorineural hearing loss identified at 3 months of age. High-resolution temporal bone computed tomography (HRCT) revealed bilateral findings of a short, malformed cochlea lacking an interscalar septum, a short and wide internal auditory canal, and an enlarged vestibular aqueduct, features diagnostic of bilateral Mondini dysplasia. To determine the importance of this observation, we completed HRCT in five additional children between 7 months and 9 years of age who had evidence of symptomatic congenital cytomegalovirus infection. One child with profound sensorineural hearing loss had severe bilateral temporal bone dysplasia with a small cochlea lacking an interscalar septum, an abnormal vestibule, and a large cochlear aqueduct. Of the remaining four children, hearing thresholds ranged from normal to profoundly decreased, but their HRCT scans were normal to visual inspection. When inner ear dimensions of these temporal bones were compared with norms established by Pappas and coworkers, however, seven of the eight ears had short cochleas and narrow lateral semicircular canals, and three ears had short or narrow vestibules. These results indicate that congenital cytomegalovirus infection may cause anomalies or growth disturbances of the temporal bone.

  6. Congenital heat disease

    International Nuclear Information System (INIS)

    Higgins, C.B.; Silverman, N.H.; Kersting-Somerhoff, B.A.

    1990-01-01

    The book covers the tomographic anatomy of the normal and congenitally malformed heart and tomographic imaging of the normal heat. It then compares echocardiographic evaluation and the use of MR imaging in the diagnosis and evaluation of individual congenital cardiac malformations

  7. Key aspects congenital infection

    Directory of Open Access Journals (Sweden)

    Yu. V. Lobzin

    2014-01-01

    Full Text Available The key questions to solve the problem of congenital infection in the Russian Federation are: using in national practice over world accepted terminology adapted to the recommendations of the World Health Organization; representation of the modern concepts of an infectious process in the classification of congenital infections; scientific development and introducing in clinical practice the «standard case definitions», applied to different congenital infections; optimization of protocols and clinical guidelines for diagnosis, treatment and prevention of congenital infections; improvement a knowledge in the infectious disease for all  pecialists involved in the risk assessment of congenital infections, manage pregnancy and children. Based on our experience and analysis of publications, the authors suggest possible solutions.

  8. Integration of auditory and visual speech information

    NARCIS (Netherlands)

    Hall, M.; Smeele, P.M.T.; Kuhl, P.K.

    1998-01-01

    The integration of auditory and visual speech is observed when modes specify different places of articulation. Influences of auditory variation on integration were examined using consonant identifi-cation, plus quality and similarity ratings. Auditory identification predicted auditory-visual

  9. Abnormalities of the external genitalia and groins among primary ...

    African Journals Online (AJOL)

    Background: Abnormalities of the male external genitalia and groin, a set of lesions which may be congenital or acquired, are rather obscured to .... some have no gross functional or morphological import .... Disorders of the testis, scrotum and.

  10. Spatial Hearing with Incongruent Visual or Auditory Room Cues

    Science.gov (United States)

    Gil-Carvajal, Juan C.; Cubick, Jens; Santurette, Sébastien; Dau, Torsten

    2016-11-01

    In day-to-day life, humans usually perceive the location of sound sources as outside their heads. This externalized auditory spatial perception can be reproduced through headphones by recreating the sound pressure generated by the source at the listener’s eardrums. This requires the acoustical features of the recording environment and listener’s anatomy to be recorded at the listener’s ear canals. Although the resulting auditory images can be indistinguishable from real-world sources, their externalization may be less robust when the playback and recording environments differ. Here we tested whether a mismatch between playback and recording room reduces perceived distance, azimuthal direction, and compactness of the auditory image, and whether this is mostly due to incongruent auditory cues or to expectations generated from the visual impression of the room. Perceived distance ratings decreased significantly when collected in a more reverberant environment than the recording room, whereas azimuthal direction and compactness remained room independent. Moreover, modifying visual room-related cues had no effect on these three attributes, while incongruent auditory room-related cues between the recording and playback room did affect distance perception. Consequently, the external perception of virtual sounds depends on the degree of congruency between the acoustical features of the environment and the stimuli.

  11. Working memory training in congenitally blind individuals results in an integration of occipital cortex in functional networks.

    Science.gov (United States)

    Gudi-Mindermann, Helene; Rimmele, Johanna M; Nolte, Guido; Bruns, Patrick; Engel, Andreas K; Röder, Brigitte

    2018-08-01

    The functional relevance of crossmodal activation (e.g. auditory activation of occipital brain regions) in congenitally blind individuals is still not fully understood. The present study tested whether the occipital cortex of blind individuals is integrated into a challenged functional network. A working memory (WM) training over four sessions was implemented. Congenitally blind and matched sighted participants were adaptively trained with an n-back task employing either voices (auditory training) or tactile stimuli (tactile training). In addition, a minimally demanding 1-back task served as an active control condition. Power and functional connectivity of EEG activity evolving during the maintenance period of an auditory 2-back task were analyzed, run prior to and after the WM training. Modality-specific (following auditory training) and modality-independent WM training effects (following both auditory and tactile training) were assessed. Improvements in auditory WM were observed in all groups, and blind and sighted individuals did not differ in training gains. Auditory and tactile training of sighted participants led, relative to the active control group, to an increase in fronto-parietal theta-band power, suggesting a training-induced strengthening of the existing modality-independent WM network. No power effects were observed in the blind. Rather, after auditory training the blind showed a decrease in theta-band connectivity between central, parietal, and occipital electrodes compared to the blind tactile training and active control groups. Furthermore, in the blind auditory training increased beta-band connectivity between fronto-parietal, central and occipital electrodes. In the congenitally blind, these findings suggest a stronger integration of occipital areas into the auditory WM network. Copyright © 2018 Elsevier B.V. All rights reserved.

  12. Auditory and Visual Sensations

    CERN Document Server

    Ando, Yoichi

    2010-01-01

    Professor Yoichi Ando, acoustic architectural designer of the Kirishima International Concert Hall in Japan, presents a comprehensive rational-scientific approach to designing performance spaces. His theory is based on systematic psychoacoustical observations of spatial hearing and listener preferences, whose neuronal correlates are observed in the neurophysiology of the human brain. A correlation-based model of neuronal signal processing in the central auditory system is proposed in which temporal sensations (pitch, timbre, loudness, duration) are represented by an internal autocorrelation representation, and spatial sensations (sound location, size, diffuseness related to envelopment) are represented by an internal interaural crosscorrelation function. Together these two internal central auditory representations account for the basic auditory qualities that are relevant for listening to music and speech in indoor performance spaces. Observed psychological and neurophysiological commonalities between auditor...

  13. Modularity in Sensory Auditory Memory

    OpenAIRE

    Clement, Sylvain; Moroni, Christine; Samson, Séverine

    2004-01-01

    The goal of this paper was to review various experimental and neuropsychological studies that support the modular conception of auditory sensory memory or auditory short-term memory. Based on initial findings demonstrating that verbal sensory memory system can be dissociated from a general auditory memory store at the functional and anatomical levels. we reported a series of studies that provided evidence in favor of multiple auditory sensory stores specialized in retaining eit...

  14. Spatial Hearing with Incongruent Visual or Auditory Room Cues

    DEFF Research Database (Denmark)

    Gil Carvajal, Juan Camilo; Cubick, Jens; Santurette, Sébastien

    2016-01-01

    In day-to-day life, humans usually perceive the location of sound sources as outside their heads. This externalized auditory spatial perception can be reproduced through headphones by recreating the sound pressure generated by the source at the listener’s eardrums. This requires the acoustical...... the recording and playback room did affect distance perception. Consequently, the external perception of virtual sounds depends on the degree of congruency between the acoustical features of the environment and the stimuli....

  15. Congenital symmastia revisited

    DEFF Research Database (Denmark)

    Sillesen, Nanna H; Hölmich, Lisbeth R; Siersen, Hans Erik

    2012-01-01

    Symmastia is defined as medial confluence of the breast. The term 'symmastia' is modified from Greek (syn meaning 'together', and mastos meaning 'breast') and was first presented by Spence et al. in 1983. Two forms of symmastia exist: an iatrogenic and a congenital version. Congenital symmastia...... is a rare condition in which web-like soft tissue traverses the sternum to connect the breasts medially. The literature on congenital symmastia is limited, few cases have been published, and knowledge about ideal treatment is still insufficient....

  16. Neurofeedback-Based Enhancement of Single-Trial Auditory Evoked Potentials: Treatment of Auditory Verbal Hallucinations in Schizophrenia.

    Science.gov (United States)

    Rieger, Kathryn; Rarra, Marie-Helene; Diaz Hernandez, Laura; Hubl, Daniela; Koenig, Thomas

    2018-03-01

    Auditory verbal hallucinations depend on a broad neurobiological network ranging from the auditory system to language as well as memory-related processes. As part of this, the auditory N100 event-related potential (ERP) component is attenuated in patients with schizophrenia, with stronger attenuation occurring during auditory verbal hallucinations. Changes in the N100 component assumingly reflect disturbed responsiveness of the auditory system toward external stimuli in schizophrenia. With this premise, we investigated the therapeutic utility of neurofeedback training to modulate the auditory-evoked N100 component in patients with schizophrenia and associated auditory verbal hallucinations. Ten patients completed electroencephalography neurofeedback training for modulation of N100 (treatment condition) or another unrelated component, P200 (control condition). On a behavioral level, only the control group showed a tendency for symptom improvement in the Positive and Negative Syndrome Scale total score in a pre-/postcomparison ( t (4) = 2.71, P = .054); however, no significant differences were found in specific hallucination related symptoms ( t (7) = -0.53, P = .62). There was no significant overall effect of neurofeedback training on ERP components in our paradigm; however, we were able to identify different learning patterns, and found a correlation between learning and improvement in auditory verbal hallucination symptoms across training sessions ( r = 0.664, n = 9, P = .05). This effect results, with cautious interpretation due to the small sample size, primarily from the treatment group ( r = 0.97, n = 4, P = .03). In particular, a within-session learning parameter showed utility for predicting symptom improvement with neurofeedback training. In conclusion, patients with schizophrenia and associated auditory verbal hallucinations who exhibit a learning pattern more characterized by within-session aptitude may benefit from electroencephalography neurofeedback

  17. Early visual deprivation prompts the use of body-centered frames of reference for auditory localization.

    Science.gov (United States)

    Vercillo, Tiziana; Tonelli, Alessia; Gori, Monica

    2018-01-01

    The effects of early visual deprivation on auditory spatial processing are controversial. Results from recent psychophysical studies show that people who were born blind have a spatial impairment in localizing sound sources within specific auditory settings, while previous psychophysical studies revealed enhanced auditory spatial abilities in early blind compared to sighted individuals. An explanation of why an auditory spatial deficit is sometimes observed within blind populations and its task-dependency remains to be clarified. We investigated auditory spatial perception in early blind adults and demonstrated that the deficit derives from blind individual's reduced ability to remap sound locations using an external frame of reference. We found that performance in blind population was severely impaired when they were required to localize brief auditory stimuli with respect to external acoustic landmarks (external reference frame) or when they had to reproduce the spatial distance between two sounds. However, they performed similarly to sighted controls when had to localize sounds with respect to their own hand (body-centered reference frame), or to judge the distances of sounds from their finger. These results suggest that early visual deprivation and the lack of visual contextual cues during the critical period induce a preference for body-centered over external spatial auditory representations. Copyright © 2017 Elsevier B.V. All rights reserved.

  18. The usefulness of three-dimensional helical CT for the detection of abnormalities of the auditory ossicles

    International Nuclear Information System (INIS)

    Gong, Honghan; Hiraishi, Kumiko; Uesugi, Yasuo; Shimizu, Tadafumi; Narabayashi, Isamu

    1996-01-01

    To evaluate the usefulness of three-dimensional (3D) helical CT for the detection of abnormalities of the auditory ossicles, 3D helical CT of the middle ear was performed in seven patients with hearing disorder. It revealed that 4 patients had congenital deficiency of the auditory ossicles, 2 patients with chronic otitis media had shortening of the incus and one patient with head injury had doubtful fracture of the incus. This study indicated that 3D helical CT of the middle ear can represent the auditory ossicles objectively and can offer detailed diagnosis. (author)

  19. Congenital orbital teratoma.

    Science.gov (United States)

    Aiyub, Shereen; Chan, Wengonn; Szetu, John; Sullivan, Laurence J; Pater, John; Cooper, Peter; Selva, Dinesh

    2013-12-01

    We present a case of mature congenital orbital teratoma managed with lid-sparing exenteration and dermis fat graft. This is a case report on the management of congenital orbital teratoma. A full-term baby was born in Fiji with prolapsed right globe which was surrounded by a nonpulsatile, cystic mass. Clinical and imaging features were consistent with congenital orbital teratoma. Due to limited surgical expertise, the patient was transferred to Adelaide, Australia for further management. The patient underwent a lid-sparing exenteration with frozen section control of the apical margin. A dermis fat graft from the groin was placed beneath the lid skin to provide volume. Histopathology revealed mature tissues from each of the three germ cell layers which confirmed the diagnosis of mature teratoma. We describe the successful use of demis fat graft in socket reconstruction following lid-sparing exenteration for congenital orbital teratoma.

  20. Congenital orbital teratoma

    Directory of Open Access Journals (Sweden)

    Shereen Aiyub

    2013-01-01

    Full Text Available We present a case of mature congenital orbital teratoma managed with lid-sparing exenteration and dermis fat graft. This is a case report on the management of congenital orbital teratoma. A full-term baby was born in Fiji with prolapsed right globe which was surrounded by a nonpulsatile, cystic mass. Clinical and imaging features were consistent with congenital orbital teratoma. Due to limited surgical expertise, the patient was transferred to Adelaide, Australia for further management. The patient underwent a lid-sparing exenteration with frozen section control of the apical margin. A dermis fat graft from the groin was placed beneath the lid skin to provide volume. Histopathology revealed mature tissues from each of the three germ cell layers which confirmed the diagnosis of mature teratoma. We describe the successful use of demis fat graft in socket reconstruction following lid-sparing exenteration for congenital orbital teratoma.

  1. Congenital Constriction Band Syndrome

    OpenAIRE

    Rajesh Gupta, Fareed Malik, Rishabh Gupta, M.A.Basit, Dara Singh

    2008-01-01

    Congenital constriction bands are anomalous bands that encircle a digit or an extremity. Congenitalconstriction band syndrome is rare condition and is mostly associated with other musculoskeletaldisorders.We report such a rare experience.

  2. Congenital anterior urethral diverticulum

    International Nuclear Information System (INIS)

    Jung, Hyun Sub; Chung, Young Sun; Suh, Chee Jang; Won, Jong Jin

    1985-01-01

    Two cases of congenital anterior urethral diverticular which have occurred in a 4 year old and one month old boy are presented. Etiology, diagnostic procedures, and its clinical results are briefly reviewed

  3. Auditory Memory for Timbre

    Science.gov (United States)

    McKeown, Denis; Wellsted, David

    2009-01-01

    Psychophysical studies are reported examining how the context of recent auditory stimulation may modulate the processing of new sounds. The question posed is how recent tone stimulation may affect ongoing performance in a discrimination task. In the task, two complex sounds occurred in successive intervals. A single target component of one complex…

  4. Auditory evacuation beacons

    NARCIS (Netherlands)

    Wijngaarden, S.J. van; Bronkhorst, A.W.; Boer, L.C.

    2005-01-01

    Auditory evacuation beacons can be used to guide people to safe exits, even when vision is totally obscured by smoke. Conventional beacons make use of modulated noise signals. Controlled evacuation experiments show that such signals require explicit instructions and are often misunderstood. A new

  5. From sensation to percept: the neural signature of auditory event-related potentials.

    Science.gov (United States)

    Joos, Kathleen; Gilles, Annick; Van de Heyning, Paul; De Ridder, Dirk; Vanneste, Sven

    2014-05-01

    An external auditory stimulus induces an auditory sensation which may lead to a conscious auditory perception. Although the sensory aspect is well known, it is still a question how an auditory stimulus results in an individual's conscious percept. To unravel the uncertainties concerning the neural correlates of a conscious auditory percept, event-related potentials may serve as a useful tool. In the current review we mainly wanted to shed light on the perceptual aspects of auditory processing and therefore we mainly focused on the auditory late-latency responses. Moreover, there is increasing evidence that perception is an active process in which the brain searches for the information it expects to be present, suggesting that auditory perception requires the presence of both bottom-up, i.e. sensory and top-down, i.e. prediction-driven processing. Therefore, the auditory evoked potentials will be interpreted in the context of the Bayesian brain model, in which the brain predicts which information it expects and when this will happen. The internal representation of the auditory environment will be verified by sensation samples of the environment (P50, N100). When this incoming information violates the expectation, it will induce the emission of a prediction error signal (Mismatch Negativity), activating higher-order neural networks and inducing the update of prior internal representations of the environment (P300). Copyright © 2014 Elsevier Ltd. All rights reserved.

  6. Congenital chylous ascites

    International Nuclear Information System (INIS)

    Romanska-Kita, J.; Borszewska-Kornacka, M. K.; Rudzinska, I.; Wawrzoniak, T.; Dobrzanska, A.; Czech-Kowalska, J.

    2011-01-01

    Congenital chylous ascites is a rare entity, conditioned by numerous factors and with changing dynamics of the disease. Because of the lack of therapeutic and diagnostic standards, this disease constitutes to be a medical challenge. This article presents current knowledge on pathogenesis, diagnostics and management of this disease, as well as a case of a newborn with primary congenital chylous ascites in the abdominal cavity. (authors)

  7. Multifocal Congenital Hemangiopericytoma.

    Science.gov (United States)

    Robl, Renata; Carvalho, Vânia Oliveira; Abagge, Kerstin Taniguchi; Uber, Marjorie; Lichtvan, Leniza Costa Lima; Werner, Betina; Mehrdad Nadji, Mehrdad

    2017-01-01

    Congenital hemangiopericytoma (HPC) is a rare mesenchymal tumor with less aggressive behavior and a more favorable prognosis than similar tumors in adults. Multifocal presentation is even less common than isolated HPC and hence its clinical and histologic recognition may be challenging. A newborn infant with multifocal congenital HPC causing severe deformity but with a favorable outcome after chemotherapy and surgical removal is reported. © 2016 Wiley Periodicals, Inc.

  8. Neural Correlates of Realistic and Unrealistic Auditory Space Perception

    Directory of Open Access Journals (Sweden)

    Akiko Callan

    2011-10-01

    Full Text Available Binaural recordings can simulate externalized auditory space perception over headphones. However, if the orientation of the recorder's head and the orientation of the listener's head are incongruent, the simulated auditory space is not realistic. For example, if a person lying flat on a bed listens to an environmental sound that was recorded by microphones inserted in ears of a person who was in an upright position, the sound simulates an auditory space rotated 90 degrees to the real-world horizontal axis. Our question is whether brain activation patterns are different between the unrealistic auditory space (ie, the orientation of the listener's head and the orientation of the recorder's head are incongruent and the realistic auditory space (ie, the orientations are congruent. River sounds that were binaurally recorded either in a supine position or in an upright body position were served as auditory stimuli. During fMRI experiments, participants listen to the stimuli and pressed one of two buttons indicating the direction of the water flow (horizontal/vertical. Behavioral results indicated that participants could not differentiate between the congruent and the incongruent conditions. However, neuroimaging results showed that the congruent condition activated the planum temporale significantly more than the incongruent condition.

  9. Self-initiated actions result in suppressed auditory but amplified visual evoked components in healthy participants.

    Science.gov (United States)

    Mifsud, Nathan G; Oestreich, Lena K L; Jack, Bradley N; Ford, Judith M; Roach, Brian J; Mathalon, Daniel H; Whitford, Thomas J

    2016-05-01

    Self-suppression refers to the phenomenon that sensations initiated by our own movements are typically less salient, and elicit an attenuated neural response, compared to sensations resulting from changes in the external world. Evidence for self-suppression is provided by previous ERP studies in the auditory modality, which have found that healthy participants typically exhibit a reduced auditory N1 component when auditory stimuli are self-initiated as opposed to externally initiated. However, the literature investigating self-suppression in the visual modality is sparse, with mixed findings and experimental protocols. An EEG study was conducted to expand our understanding of self-suppression across different sensory modalities. Healthy participants experienced either an auditory (tone) or visual (pattern-reversal) stimulus following a willed button press (self-initiated), a random interval (externally initiated, unpredictable onset), or a visual countdown (externally initiated, predictable onset-to match the intrinsic predictability of self-initiated stimuli), while EEG was continuously recorded. Reduced N1 amplitudes for self- versus externally initiated tones indicated that self-suppression occurred in the auditory domain. In contrast, the visual N145 component was amplified for self- versus externally initiated pattern reversals. Externally initiated conditions did not differ as a function of their predictability. These findings highlight a difference in sensory processing of self-initiated stimuli across modalities, and may have implications for clinical disorders that are ostensibly associated with abnormal self-suppression. © 2016 Society for Psychophysiological Research.

  10. A Brief Period of Postnatal Visual Deprivation Alters the Balance between Auditory and Visual Attention.

    Science.gov (United States)

    de Heering, Adélaïde; Dormal, Giulia; Pelland, Maxime; Lewis, Terri; Maurer, Daphne; Collignon, Olivier

    2016-11-21

    Is a short and transient period of visual deprivation early in life sufficient to induce lifelong changes in how we attend to, and integrate, simple visual and auditory information [1, 2]? This question is of crucial importance given the recent demonstration in both animals and humans that a period of blindness early in life permanently affects the brain networks dedicated to visual, auditory, and multisensory processing [1-16]. To address this issue, we compared a group of adults who had been treated for congenital bilateral cataracts during early infancy with a group of normally sighted controls on a task requiring simple detection of lateralized visual and auditory targets, presented alone or in combination. Redundancy gains obtained from the audiovisual conditions were similar between groups and surpassed the reaction time distribution predicted by Miller's race model. However, in comparison to controls, cataract-reversal patients were faster at processing simple auditory targets and showed differences in how they shifted attention across modalities. Specifically, they were faster at switching attention from visual to auditory inputs than in the reverse situation, while an opposite pattern was observed for controls. Overall, these results reveal that the absence of visual input during the first months of life does not prevent the development of audiovisual integration but enhances the salience of simple auditory inputs, leading to a different crossmodal distribution of attentional resources between auditory and visual stimuli. Copyright © 2016 Elsevier Ltd. All rights reserved.

  11. CT virtual endoscopy of the auditory ossicular chain and its preliminary clinical application

    International Nuclear Information System (INIS)

    Wang Dong; Zhang Wanshi; Xiong Minghui; Xu Jiaxing; Yu Min; Xu Changyu

    2000-01-01

    Objective: To evaluate the ability of CT virtual endoscopy (CTVE) in visualization of auditory ossicular chain and its clinical application. Methods: CTVE of auditory ossicular chain was performed on GE HiSpeed CT/i with 1.0 mm slice thickness at pitch 1.0, bone algorithm, 9.6 cm FOV, and 0.1 mm reconstruction interval in 10 normal subjects and 21 patients with middle ear diseases, 14 of them were proved by operation. The threshold values for normal and abnormal auditory ossicular chain were -600--200 HU and 50-300 HU respectively. Results: CTVE could clearly demonstrate the shape, size, and relation of the normal auditory ossicular chain. A visualization rate of malleus, incus, and incudomalleal articulation was 100%, and was 32% for the stapedial foot plate. However, in only 21% could the anterior and posterior crura of stapes be distinguished. Cholesteatoma was found in 12 cases with chronic otitis media, in which CTVE demonstrated varying degrees of destruction of the auditory ossicle. In 1 case with congenital anomaly, ossicle dysplasia was seen. Conclusion: CTVE being a new, non-invasive method for demonstrating the three-dimensional image of auditory ossicular chain is useful in evaluating diseases of the ear, especially the auditory ossicles

  12. Development of the auditory system

    Science.gov (United States)

    Litovsky, Ruth

    2015-01-01

    Auditory development involves changes in the peripheral and central nervous system along the auditory pathways, and these occur naturally, and in response to stimulation. Human development occurs along a trajectory that can last decades, and is studied using behavioral psychophysics, as well as physiologic measurements with neural imaging. The auditory system constructs a perceptual space that takes information from objects and groups, segregates sounds, and provides meaning and access to communication tools such as language. Auditory signals are processed in a series of analysis stages, from peripheral to central. Coding of information has been studied for features of sound, including frequency, intensity, loudness, and location, in quiet and in the presence of maskers. In the latter case, the ability of the auditory system to perform an analysis of the scene becomes highly relevant. While some basic abilities are well developed at birth, there is a clear prolonged maturation of auditory development well into the teenage years. Maturation involves auditory pathways. However, non-auditory changes (attention, memory, cognition) play an important role in auditory development. The ability of the auditory system to adapt in response to novel stimuli is a key feature of development throughout the nervous system, known as neural plasticity. PMID:25726262

  13. Animal models for auditory streaming

    Science.gov (United States)

    Itatani, Naoya

    2017-01-01

    Sounds in the natural environment need to be assigned to acoustic sources to evaluate complex auditory scenes. Separating sources will affect the analysis of auditory features of sounds. As the benefits of assigning sounds to specific sources accrue to all species communicating acoustically, the ability for auditory scene analysis is widespread among different animals. Animal studies allow for a deeper insight into the neuronal mechanisms underlying auditory scene analysis. Here, we will review the paradigms applied in the study of auditory scene analysis and streaming of sequential sounds in animal models. We will compare the psychophysical results from the animal studies to the evidence obtained in human psychophysics of auditory streaming, i.e. in a task commonly used for measuring the capability for auditory scene analysis. Furthermore, the neuronal correlates of auditory streaming will be reviewed in different animal models and the observations of the neurons’ response measures will be related to perception. The across-species comparison will reveal whether similar demands in the analysis of acoustic scenes have resulted in similar perceptual and neuronal processing mechanisms in the wide range of species being capable of auditory scene analysis. This article is part of the themed issue ‘Auditory and visual scene analysis’. PMID:28044022

  14. The association of congenital neuroblastoma and congenital heart disease

    International Nuclear Information System (INIS)

    Bellah, R.; D'Andrea, A.; Children's Hospital, Boston, MA; Darillis, E.; Fellows, K.E.

    1989-01-01

    Several authors have reported an association between neuroblastoma and congenital heart disease; others contend that, unlike specific wellknown associations between malignancy and congenital defects (Wilm's tumor and aniridia, leukemia and Down's syndrome), no real relationship exists. We present three cases of cyanotic congenital heart disease in which subclinical neuroblastoma was found. We speculate that abnormal neural crest cell migration and development may be a common link between cardiac malformations and congenital neuroblastoma. (orig.)

  15. Congenital malaria in China.

    Directory of Open Access Journals (Sweden)

    Zhi-Yong Tao

    2014-03-01

    Full Text Available BACKGROUND: Congenital malaria, in which infants are directly infected with malaria parasites from their mother prior to or during birth, is a potentially life-threatening condition that occurs at relatively low rates in malaria-endemic regions. It is recognized as a serious problem in Plasmodium falciparum-endemic sub-Saharan Africa, where recent data suggests that it is more common than previously believed. In such regions where malaria transmission is high, neonates may be protected from disease caused by congenital malaria through the transfer of maternal antibodies against the parasite. However, in low P. vivax-endemic regions, immunity to vivax malaria is low; thus, there is the likelihood that congenital vivax malaria poses a more significant threat to newborn health. Malaria had previously been a major parasitic disease in China, and congenital malaria case reports in Chinese offer valuable information for understanding the risks posed by congenital malaria to neonatal health. As most of the literature documenting congenital malaria cases in China are written in Chinese and therefore are not easily accessible to the global malaria research community, we have undertaken an extensive review of the Chinese literature on this subject. METHODS/PRINCIPAL FINDINGS: Here, we reviewed congenital malaria cases from three major searchable Chinese journal databases, concentrating on data from 1915 through 2011. Following extensive screening, a total of 104 cases of congenital malaria were identified. These cases were distributed mainly in the eastern, central, and southern regions of China, as well as in the low-lying region of southwest China. The dominant species was P. vivax (92.50%, reflecting the malaria parasite species distribution in China. The leading clinical presentation was fever, and other clinical presentations were anaemia, jaundice, paleness, diarrhoea, vomiting, and general weakness. With the exception of two cases, all patients

  16. Congenital cytomegalovirus infection

    Directory of Open Access Journals (Sweden)

    Katarina Rednak-Paradiž

    2006-11-01

    Full Text Available Background: CMV is the most common agent that causes congenital virus infection. Only 10 % of infected children have symptomatic infection immediately after birth. Signs of central nervous system damage, neurosensory deafness and delayed psychomotor development may manifest as a result of asimptomatic congenital infection later in childhood. In the article we present basic properties of CMV; we describe clinical picture of the congenital infection and possibilities of diagnose and its treatment. We present five children with symptomatic congenital CMV infection that were hospitalized for the period 1992–2002 at the Neonatal department in the University Children’s Hospital in Ljubljana.Conclusions: Identification of infected neonates, especially those with asimptomatic congenital CMV infection, is difficult. Latest incidence of infection in Slovenia is unknown. With new investigations the efficiency of antiviral therapy was discovered but exact indications for therapy are not yet known. CMV vaccine, once available, may ultimately be the best control strategy for this important public health problem. Proper educating women in childbearing age about the risks of CMV and how to avoid disease transmission during pregnancy (hand washing, avoiding mouth-to-mouth contact with preschool children, usage of gloves especially when handling dipers or respiratory secretions are the only control strategies available.

  17. Prevalence of congenital amusia.

    Science.gov (United States)

    Peretz, Isabelle; Vuvan, Dominique T

    2017-05-01

    Congenital amusia (commonly known as tone deafness) is a lifelong musical disorder that affects 4% of the population according to a single estimate based on a single test from 1980. Here we present the first large-based measure of prevalence with a sample of 20 000 participants, which does not rely on self-referral. On the basis of three objective tests and a questionnaire, we show that (a) the prevalence of congenital amusia is only 1.5%, with slightly more females than males, unlike other developmental disorders where males often predominate; (b) self-disclosure is a reliable index of congenital amusia, which suggests that congenital amusia is hereditary, with 46% first-degree relatives similarly affected; (c) the deficit is not attenuated by musical training and (d) it emerges in relative isolation from other cognitive disorder, except for spatial orientation problems. Hence, we suggest that congenital amusia is likely to result from genetic variations that affect musical abilities specifically.

  18. Congenital amusia in childhood: a case study.

    Science.gov (United States)

    Lebrun, Marie-Andrée; Moreau, Patricia; McNally-Gagnon, Andréane; Mignault Goulet, Geneviève; Peretz, Isabelle

    2012-06-01

    Here we describe the first documented case of congenital amusia in childhood. AS is a 10-year-old girl who was referred to us by her choir director for persisting difficulties in singing. We tested her with the child version of the Montreal Battery for the Evaluation of Amusia (MBEA) which confirmed AS's severe problems with melodic and rhythmic discrimination and memory for melodies. The disorder appears to be limited to music since her audiometry as well as her intellectual and language skills are normal. Furthermore, the musical disorder is associated to a severe deficit in detecting small pitch changes. The electrical brain responses point to an anomaly in the early stages of auditory processing, such as reflected by an abnormal mismatch negativity (MMN) response to small pitch changes. In singing, AS makes more pitch than time errors. Thus, despite frequent and regular musical practice, AS's profile is similar to the adult form of congenital amusia. Copyright © 2011 Elsevier Srl. All rights reserved.

  19. Nitrofurantoin and congenital abnormalities

    DEFF Research Database (Denmark)

    Czeizel, A.E.; Rockenbauer, M.; Sørensen, Henrik Toft

    2001-01-01

    or fetuses with Down’s syndrome (patient controls), 23 (2.8%) pregnant women were treated with nitrofurantoin. The above differences between population controls and cases may be connected with recall bias, because the case-control pair analysis did not indicate a teratogenic potential of nitrofurantoin use......Objective: To study human teratogenic potential of oral nitrofurantoin treatment during pregnancy. Materials and Methods: Pair analysis of cases with congenital abnormalities and matched population controls in the population-based dataset of the Hungarian Case-Control Surveillance of Congenital...... during the second and the third months of gestation, i.e. in the critical period for major congenital abnormalities. Conclusion: Treatment with nitrofurantoin during pregnancy does not present detectable teratogenic risk to the fetus....

  20. Congenital syphilis: literature review

    Directory of Open Access Journals (Sweden)

    Eduardo Chaida Sonda

    2013-01-01

    Full Text Available Syphilis is an infectious disease caused by Treponema pallidum and has high rates of vertical transmission, which can reach 100% depending on the maternal disease and stage of pregnancy. The diagnosis of gestational syphilis is simple and its screening is required during the prenatal period. However, this disease still has a high prevalence, affecting two million pregnant women worldwide. The procedures performed in newborns with congenital syphilis represent costs that are three-fold higher than the ones spent with a baby without this infection. The treatment is generally carried out with penicillin and must be extended to sexual partners. Inadequate or lack of treatment of congenital syphilis can result in miscarriage, premature birth, acute complications and other fetal sequelae. KEYWORDS: Congenital syphilis. Treponema pallidum. Vertical transmission.

  1. Congenital cataract screening

    Directory of Open Access Journals (Sweden)

    Zhale Rajavi

    2016-01-01

    Full Text Available Congenital cataract is a leading cause of visual deprivation which can damage the developing visual system of a child; therefore early diagnosis, management and long-term follow-up are essential. It is recommended that all neonates be screened by red reflex examination at birth and suspected cases be referred to ophthalmic centers. Early surgery (1 year is highly recommended. After surgery, amblyopia treatment and periodic follow-up examinations should be started as soon as possible to achieve a satisfactory visual outcome. Practitioners should consider the possibility of posterior capsular opacity, elevated intraocular pressure and amblyopia during follow-up, especially in eyes with microphthalmia and/or associated congenital anomalies. All strabismic children should undergo slit lamp examination prior to strabismus surgery to rule out congenital lens opacities. From a social point of view, equal and fair medical care should be provided to all children regardless of gender.

  2. Auditory interfaces: The human perceiver

    Science.gov (United States)

    Colburn, H. Steven

    1991-01-01

    A brief introduction to the basic auditory abilities of the human perceiver with particular attention toward issues that may be important for the design of auditory interfaces is presented. The importance of appropriate auditory inputs to observers with normal hearing is probably related to the role of hearing as an omnidirectional, early warning system and to its role as the primary vehicle for communication of strong personal feelings.

  3. Auditory Perceptual Abilities Are Associated with Specific Auditory Experience

    Directory of Open Access Journals (Sweden)

    Yael Zaltz

    2017-11-01

    Full Text Available The extent to which auditory experience can shape general auditory perceptual abilities is still under constant debate. Some studies show that specific auditory expertise may have a general effect on auditory perceptual abilities, while others show a more limited influence, exhibited only in a relatively narrow range associated with the area of expertise. The current study addresses this issue by examining experience-dependent enhancement in perceptual abilities in the auditory domain. Three experiments were performed. In the first experiment, 12 pop and rock musicians and 15 non-musicians were tested in frequency discrimination (DLF, intensity discrimination, spectrum discrimination (DLS, and time discrimination (DLT. Results showed significant superiority of the musician group only for the DLF and DLT tasks, illuminating enhanced perceptual skills in the key features of pop music, in which miniscule changes in amplitude and spectrum are not critical to performance. The next two experiments attempted to differentiate between generalization and specificity in the influence of auditory experience, by comparing subgroups of specialists. First, seven guitar players and eight percussionists were tested in the DLF and DLT tasks that were found superior for musicians. Results showed superior abilities on the DLF task for guitar players, though no difference between the groups in DLT, demonstrating some dependency of auditory learning on the specific area of expertise. Subsequently, a third experiment was conducted, testing a possible influence of vowel density in native language on auditory perceptual abilities. Ten native speakers of German (a language characterized by a dense vowel system of 14 vowels, and 10 native speakers of Hebrew (characterized by a sparse vowel system of five vowels, were tested in a formant discrimination task. This is the linguistic equivalent of a DLS task. Results showed that German speakers had superior formant

  4. Congenital maxillary double lip

    Directory of Open Access Journals (Sweden)

    Dinesh Singh Chauhan

    2012-01-01

    Full Text Available Double lip, also referred to as "macrocheilia," is a rare anomaly which affects the upper lip more commonly than the lower lip. It consists of a fold of excess or redundant hypertrophic tissue on the mucosal side of the lip. The congenital double lip is believed to be present at birth and becomes more prominent after eruption of teeth. It affects esthetics and also interferes with speech and mastication. Simple surgical excision produces good functional and cosmetic results. We report a case of a non-syndromic congenital maxillary double lip in a 21-year-old male patient.

  5. Congenital hearing impairment

    Energy Technology Data Exchange (ETDEWEB)

    Robson, Caroline D. [Children' s Hospital and Harvard Medical School, Division of Neuroradiology, Department of Radiology, Boston, MA (United States)

    2006-04-15

    Establishing the etiology of congenital hearing impairment can significantly improve treatment for certain causes of hearing loss and facilitates genetic counseling. High-resolution CT and MRI have contributed to the evaluation and management of hearing impairment. In addition, with the identification of innumerable genetic loci and genetic defects involved in hearing loss, genetic testing has emerged as an invaluable tool in the assessment of hearing impairment. Some of the common forms of congenital hearing loss are reviewed and their imaging features illustrated. (orig.)

  6. Congenital spinal malformations

    International Nuclear Information System (INIS)

    Ertl-Wagner, B.B.; Reiser, M.F.

    2001-01-01

    Congenital spinal malformations form a complex and heterogeneous group of disorders whose pathogenesis is best explained embryologically. Radiologically, it is important to formulate a diagnosis when the disorder first becomes symptomatic. However, it is also crucial to detect complications of the disorder or of the respective therapeutic interventions in the further course of the disease such as hydromyelia or re-tethering after repair of a meningomyelocele. Moreover, once a congenital spinal malformation is diagnosed, associated malformations should be sought after. A possible syndromal classification such as in OEIS- or VACTERL-syndromes should also be considered. (orig.) [de

  7. Congenital hearing impairment

    International Nuclear Information System (INIS)

    Robson, Caroline D.

    2006-01-01

    Establishing the etiology of congenital hearing impairment can significantly improve treatment for certain causes of hearing loss and facilitates genetic counseling. High-resolution CT and MRI have contributed to the evaluation and management of hearing impairment. In addition, with the identification of innumerable genetic loci and genetic defects involved in hearing loss, genetic testing has emerged as an invaluable tool in the assessment of hearing impairment. Some of the common forms of congenital hearing loss are reviewed and their imaging features illustrated. (orig.)

  8. Congenital laryngeal anomalies,

    Directory of Open Access Journals (Sweden)

    Michael J. Rutter

    2014-12-01

    Full Text Available Introduction: It is essential for clinicians to understand issues relevant to the airway management of infants and to be cognizant of the fact that infants with congenital laryngeal anomalies are at particular risk for an unstable airway. Objectives: To familiarize clinicians with issues relevant to the airway management of infants and to present a succinct description of the diagnosis and management of an array of congenital laryngeal anomalies. Methods: Revision article, in which the main aspects concerning airway management of infants will be analyzed. Conclusions: It is critical for clinicians to understand issues relevant to the airway management of infants.

  9. Congenital Heart Defects and CCHD

    Science.gov (United States)

    ... and more. Stony Point, NY 10980 Close X Home > Complications & Loss > Birth defects & other health conditions > Congenital heart defects and ... in congenital heart defects. You have a family history of congenital heart ... syndrome or VCF. After birth Your baby may be tested for CCHD as ...

  10. An EMG Study of the Lip Muscles during Covert Auditory Verbal Hallucinations in Schizophrenia

    Science.gov (United States)

    Rapin, Lucile; Dohen, Marion; Polosan, Mircea; Perrier, Pascal; Loevenbruck, Hélène

    2013-01-01

    Purpose: "Auditory verbal hallucinations" (AVHs) are speech perceptions in the absence of external stimulation. According to an influential theoretical account of AVHs in schizophrenia, a deficit in inner-speech monitoring may cause the patients' verbal thoughts to be perceived as external voices. The account is based on a…

  11. The Central Auditory Processing Kit[TM]. Book 1: Auditory Memory [and] Book 2: Auditory Discrimination, Auditory Closure, and Auditory Synthesis [and] Book 3: Auditory Figure-Ground, Auditory Cohesion, Auditory Binaural Integration, and Compensatory Strategies.

    Science.gov (United States)

    Mokhemar, Mary Ann

    This kit for assessing central auditory processing disorders (CAPD), in children in grades 1 through 8 includes 3 books, 14 full-color cards with picture scenes, and a card depicting a phone key pad, all contained in a sturdy carrying case. The units in each of the three books correspond with auditory skill areas most commonly addressed in…

  12. Auditory Reserve and the Legacy of Auditory Experience

    Directory of Open Access Journals (Sweden)

    Erika Skoe

    2014-11-01

    Full Text Available Musical training during childhood has been linked to more robust encoding of sound later in life. We take this as evidence for an auditory reserve: a mechanism by which individuals capitalize on earlier life experiences to promote auditory processing. We assert that early auditory experiences guide how the reserve develops and is maintained over the lifetime. Experiences that occur after childhood, or which are limited in nature, are theorized to affect the reserve, although their influence on sensory processing may be less long-lasting and may potentially fade over time if not repeated. This auditory reserve may help to explain individual differences in how individuals cope with auditory impoverishment or loss of sensorineural function.

  13. Severe congenital neutropenia

    DEFF Research Database (Denmark)

    Borregaard, Niels

    2014-01-01

    In this issue of Blood, Tidwell et al1 demonstrate that mutations in the start codon (protein synthesis is initiated at the codon ATG) of neutrophil elastase (ELANE) result in the production of N-terminally truncated elastase, which mislocates to the nucleus and results in severe congenital neutr...... neutropenia (SCN)....

  14. Giant Congenital Melanocytic Nevus

    DEFF Research Database (Denmark)

    Rasmussen, Bo Sonnich; Henriksen, Trine Foged; Kølle, Stig-Frederik Trojahn

    2015-01-01

    Giant congenital melanocytic nevi (GCMN) occur in 1:20,000 livebirths and are associated with increased risk of malignant transformation. The treatment of GCMN from 1981 to 2010 in a tertiary referral center was reviewed evaluating the modalities used, cosmetic results, associated complications...

  15. Congenital neck masses.

    Science.gov (United States)

    Rosa, Peter A; Hirsch, David L; Dierks, Eric J

    2008-08-01

    Congenital neck lesions reflect abnormal embryogenesis in head and neck development. A thorough knowledge of embryology and anatomy is critical in the diagnosis and treatment of these lesions. The appropriate diagnosis of these lesions is necessary to provide appropriate treatment and long-term follow up, because some of these lesions may undergo malignant transformation or be harbingers of malignant disease.

  16. Congenital cystic eyeball

    Directory of Open Access Journals (Sweden)

    Gupta V

    1990-01-01

    Full Text Available A rare case of histopathologically proved case of congenital cystic eye in a one day old girl is described. It was an unusually large cystic mass bulging forwards stretching the upperlid. There was no rudimentary eyeball in the orbit. The cystic eye′s predilection for the left eye has been pointed out for the first time in this article.

  17. Congenital extracranial meningioma

    International Nuclear Information System (INIS)

    Wong, H.F.; Ng, S.H.; Wai, Y.Y.; Wan, Y.L.; Kong, M.S.

    1995-01-01

    The authors report a case of congenital meningioma in a newborn. This tumour is extremely rare and only six cases have been reported in the literature. Those reported cases were mainly intracranial. This is the first case of a neonatal extracranial meningioma that was evaluated preoperatively by computed tomography and magnetic resonance imaging. (orig.)

  18. Congenital cystic adenomatoid malformation

    International Nuclear Information System (INIS)

    Chaudhry, A.K.; Azam, M.; Maqsood, R.; Naz, B.; Salam, A.

    2003-01-01

    This case report presents the clinical picture, diagnostic methodology and surgical treatment of a female child who presented with chronic cough and dyspnoea due to congenital malformation of lung. A discussion of diagnosis and management is presented at the end. (author)

  19. Pseudoamblyopia in Congenital Cyclotropia

    Directory of Open Access Journals (Sweden)

    Antonio Frattolillo

    2017-01-01

    Full Text Available Purpose. To study the effect of surgery on amblyopia and suppression associated with congenital cyclovertical strabismus. Methods. The fixation pattern was investigated with microperimetry before and soon after surgery in ten consecutive children operated for congenital superior oblique palsy at the S. Martino Hospital, Belluno, Italy, between September 2014 and December 2015. Changes in visual performance in terms of best-corrected visual acuity (BCVA and stereopsis between the day before and one week after surgery were also evaluated. No other amblyopia treatment has been administered during the time study. Results. Surgical correction of the excyclodeviation in congenital SO palsy determined monocular and binocular sensory consequences: monocularly, in the cyclodeviated amblyopic eye, BCVA (0.46–0.03 LogMAR; p<0.0001 and the fixation pattern improved, as demonstrated by microperimetry examination. Binocularly, stereopsis improved or emerged while suppression at the Worth four-dot test disappeared. Conclusions. In the absence of further amblyopic factors such as coexisting constant vertical and/or horizontal deviation and anisometropia, the amblyopia encountered in congenital SO palsy may resolve soon after the surgical alignment. Therefore, it may be considered and defined “pseudoamblyopia.”

  20. Identification of congenital deafblindness

    DEFF Research Database (Denmark)

    Dammeyer, Jesper Herup

    2012-01-01

    . The study evaluated the assessment procedure of 190 children and adults found to be congenitally deafblind. Among the 190 individuals 76 percent were determined using functional assessment in addition to medical examination. A case example involving a 12-year-old child is also presented to illustrate...

  1. Auditory changes in acromegaly.

    Science.gov (United States)

    Tabur, S; Korkmaz, H; Baysal, E; Hatipoglu, E; Aytac, I; Akarsu, E

    2017-06-01

    The aim of this study is to determine the changes involving auditory system in cases with acromegaly. Otological examinations of 41 cases with acromegaly (uncontrolled n = 22, controlled n = 19) were compared with those of age and gender-matched 24 healthy subjects. Whereas the cases with acromegaly underwent examination with pure tone audiometry (PTA), speech audiometry for speech discrimination (SD), tympanometry, stapedius reflex evaluation and otoacoustic emission tests, the control group did only have otological examination and PTA. Additionally, previously performed paranasal sinus-computed tomography of all cases with acromegaly and control subjects were obtained to measure the length of internal acoustic canal (IAC). PTA values were higher (p acromegaly group was narrower compared to that in control group (p = 0.03 for right ears and p = 0.02 for left ears). When only cases with acromegaly were taken into consideration, PTA values in left ears had positive correlation with growth hormone and insulin-like growth factor-1 levels (r = 0.4, p = 0.02 and r = 0.3, p = 0.03). Of all cases with acromegaly 13 (32%) had hearing loss in at least one ear, 7 (54%) had sensorineural type and 6 (46%) had conductive type hearing loss. Acromegaly may cause certain changes in the auditory system in cases with acromegaly. The changes in the auditory system may be multifactorial causing both conductive and sensorioneural defects.

  2. First Branchial Cleft Malformation with Duplication of External Auditory Canal

    Science.gov (United States)

    Parida, Pradipta Kumar; Raja, Kalairasi; Surianarayanan, Gopalakrishnan; Ganeshan, Sivaraman

    2013-01-01

    First branchial cleft anomalies are uncommon, accounting for less than 10% of all branchial abnormalities. Their rare occurrence and varied presentation have frequently led to misdiagnosis and inadequate and inappropriate treatment of these conditions leading to repeated recurrences and secondary infection. In this paper, a case of 11-year girl with type 2 first branchial cleft defect is described. She first presented with a nonhealing ulcer of upper neck from childhood. Diagnosis had previously been missed and treated as tubercular ulcer. We confirmed the correct diagnosis by history and computerized tomography fistulogram. The lesion was completely excised with no further recurrence. PMID:24312740

  3. First Branchial Cleft Malformation with Duplication of External Auditory Canal

    Directory of Open Access Journals (Sweden)

    Pradipta Kumar Parida

    2013-01-01

    Full Text Available First branchial cleft anomalies are uncommon, accounting for less than 10% of all branchial abnormalities. Their rare occurrence and varied presentation have frequently led to misdiagnosis and inadequate and inappropriate treatment of these conditions leading to repeated recurrences and secondary infection. In this paper, a case of 11-year girl with type 2 first branchial cleft defect is described. She first presented with a nonhealing ulcer of upper neck from childhood. Diagnosis had previously been missed and treated as tubercular ulcer. We confirmed the correct diagnosis by history and computerized tomography fistulogram. The lesion was completely excised with no further recurrence.

  4. Partial Epilepsy with Auditory Features

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2004-07-01

    Full Text Available The clinical characteristics of 53 sporadic (S cases of idiopathic partial epilepsy with auditory features (IPEAF were analyzed and compared to previously reported familial (F cases of autosomal dominant partial epilepsy with auditory features (ADPEAF in a study at the University of Bologna, Italy.

  5. Word Recognition in Auditory Cortex

    Science.gov (United States)

    DeWitt, Iain D. J.

    2013-01-01

    Although spoken word recognition is more fundamental to human communication than text recognition, knowledge of word-processing in auditory cortex is comparatively impoverished. This dissertation synthesizes current models of auditory cortex, models of cortical pattern recognition, models of single-word reading, results in phonetics and results in…

  6. Peripheral Auditory Mechanisms

    CERN Document Server

    Hall, J; Hubbard, A; Neely, S; Tubis, A

    1986-01-01

    How weIl can we model experimental observations of the peripheral auditory system'? What theoretical predictions can we make that might be tested'? It was with these questions in mind that we organized the 1985 Mechanics of Hearing Workshop, to bring together auditory researchers to compare models with experimental observations. Tbe workshop forum was inspired by the very successful 1983 Mechanics of Hearing Workshop in Delft [1]. Boston University was chosen as the site of our meeting because of the Boston area's role as a center for hearing research in this country. We made a special effort at this meeting to attract students from around the world, because without students this field will not progress. Financial support for the workshop was provided in part by grant BNS- 8412878 from the National Science Foundation. Modeling is a traditional strategy in science and plays an important role in the scientific method. Models are the bridge between theory and experiment. Tbey test the assumptions made in experim...

  7. Temporal Organization of Sound Information in Auditory Memory

    OpenAIRE

    Song, Kun; Luo, Huan

    2017-01-01

    Memory is a constructive and organizational process. Instead of being stored with all the fine details, external information is reorganized and structured at certain spatiotemporal scales. It is well acknowledged that time plays a central role in audition by segmenting sound inputs into temporal chunks of appropriate length. However, it remains largely unknown whether critical temporal structures exist to mediate sound representation in auditory memory. To address the issue, here we designed ...

  8. Distractor Effect of Auditory Rhythms on Self-Paced Tapping in Chimpanzees and Humans

    Science.gov (United States)

    Hattori, Yuko; Tomonaga, Masaki; Matsuzawa, Tetsuro

    2015-01-01

    Humans tend to spontaneously align their movements in response to visual (e.g., swinging pendulum) and auditory rhythms (e.g., hearing music while walking). Particularly in the case of the response to auditory rhythms, neuroscientific research has indicated that motor resources are also recruited while perceiving an auditory rhythm (or regular pulse), suggesting a tight link between the auditory and motor systems in the human brain. However, the evolutionary origin of spontaneous responses to auditory rhythms is unclear. Here, we report that chimpanzees and humans show a similar distractor effect in perceiving isochronous rhythms during rhythmic movement. We used isochronous auditory rhythms as distractor stimuli during self-paced alternate tapping of two keys of an electronic keyboard by humans and chimpanzees. When the tempo was similar to their spontaneous motor tempo, tapping onset was influenced by intermittent entrainment to auditory rhythms. Although this effect itself is not an advanced rhythmic ability such as dancing or singing, our results suggest that, to some extent, the biological foundation for spontaneous responses to auditory rhythms was already deeply rooted in the common ancestor of chimpanzees and humans, 6 million years ago. This also suggests the possibility of a common attentional mechanism, as proposed by the dynamic attending theory, underlying the effect of perceiving external rhythms on motor movement. PMID:26132703

  9. Distractor Effect of Auditory Rhythms on Self-Paced Tapping in Chimpanzees and Humans.

    Directory of Open Access Journals (Sweden)

    Yuko Hattori

    Full Text Available Humans tend to spontaneously align their movements in response to visual (e.g., swinging pendulum and auditory rhythms (e.g., hearing music while walking. Particularly in the case of the response to auditory rhythms, neuroscientific research has indicated that motor resources are also recruited while perceiving an auditory rhythm (or regular pulse, suggesting a tight link between the auditory and motor systems in the human brain. However, the evolutionary origin of spontaneous responses to auditory rhythms is unclear. Here, we report that chimpanzees and humans show a similar distractor effect in perceiving isochronous rhythms during rhythmic movement. We used isochronous auditory rhythms as distractor stimuli during self-paced alternate tapping of two keys of an electronic keyboard by humans and chimpanzees. When the tempo was similar to their spontaneous motor tempo, tapping onset was influenced by intermittent entrainment to auditory rhythms. Although this effect itself is not an advanced rhythmic ability such as dancing or singing, our results suggest that, to some extent, the biological foundation for spontaneous responses to auditory rhythms was already deeply rooted in the common ancestor of chimpanzees and humans, 6 million years ago. This also suggests the possibility of a common attentional mechanism, as proposed by the dynamic attending theory, underlying the effect of perceiving external rhythms on motor movement.

  10. Distractor Effect of Auditory Rhythms on Self-Paced Tapping in Chimpanzees and Humans.

    Science.gov (United States)

    Hattori, Yuko; Tomonaga, Masaki; Matsuzawa, Tetsuro

    2015-01-01

    Humans tend to spontaneously align their movements in response to visual (e.g., swinging pendulum) and auditory rhythms (e.g., hearing music while walking). Particularly in the case of the response to auditory rhythms, neuroscientific research has indicated that motor resources are also recruited while perceiving an auditory rhythm (or regular pulse), suggesting a tight link between the auditory and motor systems in the human brain. However, the evolutionary origin of spontaneous responses to auditory rhythms is unclear. Here, we report that chimpanzees and humans show a similar distractor effect in perceiving isochronous rhythms during rhythmic movement. We used isochronous auditory rhythms as distractor stimuli during self-paced alternate tapping of two keys of an electronic keyboard by humans and chimpanzees. When the tempo was similar to their spontaneous motor tempo, tapping onset was influenced by intermittent entrainment to auditory rhythms. Although this effect itself is not an advanced rhythmic ability such as dancing or singing, our results suggest that, to some extent, the biological foundation for spontaneous responses to auditory rhythms was already deeply rooted in the common ancestor of chimpanzees and humans, 6 million years ago. This also suggests the possibility of a common attentional mechanism, as proposed by the dynamic attending theory, underlying the effect of perceiving external rhythms on motor movement.

  11. Congenital imprinting disorders

    DEFF Research Database (Denmark)

    Eggermann, Thomas; Netchine, Irène; Temple, I Karen

    2015-01-01

    Imprinting disorders (IDs) are a group of eight rare but probably underdiagnosed congenital diseases affecting growth, development and metabolism. They are caused by similar molecular changes affecting regulation, dosage or the genomic sequence of imprinted genes. Each ID is characterised...... by specific clinical features, and, as each appeared to be associated with specific imprinting defects, they have been widely regarded as separate entities. However, they share clinical characteristics and can show overlapping molecular alterations. Nevertheless, IDs are usually studied separately despite...... EUCID.net (European network of congenital imprinting disorders) now aims to promote better clinical care and scientific investigation of imprinting disorders by establishing a concerted multidisciplinary alliance of clinicians, researchers, patients and families. By encompassing all IDs and establishing...

  12. Congenital intestinal lymphangiectasia

    Directory of Open Access Journals (Sweden)

    Popović Dušan Đ.

    2011-01-01

    Full Text Available Background. Congenital intestinal lymphangiectasia is a disease which leads to protein losing enteropathy. Tortous, dilated lymphatic vessels in the intestinal wall and mesenterium are typical features of the disease. Clinical manifestations include malabsorption, diarrhea, steatorrhea, edema and effusions. Specific diet and medication are required for disease control. Case report. A 19-year old male patient was hospitalized due to diarrhea, abdominal swelling, weariness and fatigue. Physical examination revealed growth impairment, ascites, and lymphedema of the right hand and forearm. Laboratory assessment indicated iron deficiency anaemia, lymphopenia, malabsorption, inflammatory syndrome, and urinary infection. Enteroscopy and video capsule endoscopy demonstrated dilated lymphatic vessels in the small intestine. The diagnosis was confirmed by intestinal biopsy. The patient was put on high-protein diet containing medium-chain fatty acids, somatotropin and suportive therapy. Conclusion. Congenital intestinal lymphangiectasia is a rare disease, usually diagnosed in childhood. Early recognition of the disease and adequate treatment can prevent development of various complications.

  13. [Congenital intestinal lymphangiectasia].

    Science.gov (United States)

    Popović, Dugan D j; Spuran, Milan; Alempijević, Tamara; Krstić, Miodrag; Djuranović, Srdjan; Kovacević, Nada; Damnjanović, Svetozar; Micev, Marjan

    2011-03-01

    Congenital intestinal lymphangiectasia is a disease which leads to protein losing enteropathy. Tortuous, dilated lymphatic vessels in the intestinal wall and mesenterium are typical features of the disease. Clinical manifestations include malabsorption, diarrhea, steatorrhea, edema and effusions. Specific diet and medication are required for disease control. A 19-year old male patient was hospitalized due to diarrhea, abdominal swelling, weariness and fatigue. Physical examination revealed growth impairment, ascites, and lymphedema of the right hand and forearm. Laboratory assessment indicated iron deficiency anaemia, lymphopenia, malabsorption, inflammatory syndrome, and urinary infection. Enteroscopy and video capsule endoscopy demonstrated dilated lymphatic vessels in the small intestine. The diagnosis was confirmed by intestinal biopsy. The patient was put on high-protein diet containing medium-chain fatty acids, somatotropin and supportive therapy. Congenital intestinal lymphangiectasia is a rare disease, usually diagnosed in childhood. Early recognition of the disease and adequate treatment can prevent development of various complications.

  14. Congenital diaphramatic hernia

    Energy Technology Data Exchange (ETDEWEB)

    Kline-Fath, Beth M. [Cincinnati Children' s Hospital Medical Center, Department of Radiology, Fetal Care Center of Cincinnati, Cincinnati, OH (United States); Cincinnati Children' s Hospital Medical Center, Department of Radiology, MLC 5031, Cincinnati, OH (United States)

    2012-01-15

    Congenital diaphragmatic hernia, despite advances in therapy, remains a complex condition with significant morbidity and mortality. The etiology of the disorder is still incompletely understood, though the pulmonary hypoplasia and pulmonary hypertension that develop secondarily must be overcome to improve survival. Prenatal US and fetal MRI have helped in the development of a greater understanding of this disease. Also with these modalities, measurement techniques have been developed in an attempt to provide prognosticators for the development of pulmonary hypoplasia and pulmonary hypertension. There is a broad range of approaches for performing these measurements, and variability among imaging centers is noted. Despite inconsistent approaches, these techniques have become the foundation for counseling and prenatal and postnatal therapy. It is hoped that with further research with prenatal US and fetal MRI and the development of innovative medical and surgical therapies that the morbidity and mortality of children with congenital diaphragmatic hernias can be significantly reduced. (orig.)

  15. Congenital Hepatic Cyst

    Directory of Open Access Journals (Sweden)

    Aldo Recinos

    2017-04-01

    Full Text Available Congenital hepatic cyst is a rare and nonsymptomatic condition in infants and children. Its incidence is 2.5% in the postnatal life with a much lower incidence in the prenatal period. Incidental finding on antenatal imaging is the most common presentation. We present a case of a newborn in whom fetal ultrasound detected a cyst within the fetal liver. Postnatal imaging revealed a liver cyst in the right lobe of the liver, with no other intrahepatic structure affected. Liver function tests were abnormal, but the patient was asymptomatic. Posterior follow-up imaging showed a minor decrease in size. Management of congenital hepatic cyst is usually conservative, done with periodic ultrasound monitoring. However, surgical treatment is the mainstay of treatment when hydrops, progressive enlargement, hemorrhage, torsion, or compression of adjacent structures occurs. Malignant transformation can occur, but it is extremely rare. Partial or total removal of the cyst is the preferred treatment in neonates with a large lesion.

  16. Congenital diaphramatic hernia

    International Nuclear Information System (INIS)

    Kline-Fath, Beth M.

    2012-01-01

    Congenital diaphragmatic hernia, despite advances in therapy, remains a complex condition with significant morbidity and mortality. The etiology of the disorder is still incompletely understood, though the pulmonary hypoplasia and pulmonary hypertension that develop secondarily must be overcome to improve survival. Prenatal US and fetal MRI have helped in the development of a greater understanding of this disease. Also with these modalities, measurement techniques have been developed in an attempt to provide prognosticators for the development of pulmonary hypoplasia and pulmonary hypertension. There is a broad range of approaches for performing these measurements, and variability among imaging centers is noted. Despite inconsistent approaches, these techniques have become the foundation for counseling and prenatal and postnatal therapy. It is hoped that with further research with prenatal US and fetal MRI and the development of innovative medical and surgical therapies that the morbidity and mortality of children with congenital diaphragmatic hernias can be significantly reduced. (orig.)

  17. Developmental programming of auditory learning

    Directory of Open Access Journals (Sweden)

    Melania Puddu

    2012-10-01

    Full Text Available The basic structures involved in the development of auditory function and consequently in language acquisition are directed by genetic code, but the expression of individual genes may be altered by exposure to environmental factors, which if favorable, orient it in the proper direction, leading its development towards normality, if unfavorable, they deviate it from its physiological course. Early sensorial experience during the foetal period (i.e. intrauterine noise floor, sounds coming from the outside and attenuated by the uterine filter, particularly mother’s voice and modifications induced by it at the cochlear level represent the first example of programming in one of the earliest critical periods in development of the auditory system. This review will examine the factors that influence the developmental programming of auditory learning from the womb to the infancy. In particular it focuses on the following points: the prenatal auditory experience and the plastic phenomena presumably induced by it in the auditory system from the basilar membrane to the cortex;the involvement of these phenomena on language acquisition and on the perception of language communicative intention after birth;the consequences of auditory deprivation in critical periods of auditory development (i.e. premature interruption of foetal life.

  18. Characterizing Congenital Amusia

    OpenAIRE

    Stewart, Lauren

    2011-01-01

    The ability to make sense of the music in our environment involves sophisticated cognitive mechanisms that, for most people, are acquired effortlessly and in early life. A special population of individuals, with a disorder termed congenital amusia, report lifelong difficulties in this regard. Exploring the nature of this developmental disorder provides a window onto the cognitive architecture of typical musical processing, as well as allowing a study of the relationship between processing of ...

  19. The heart: Congenital disease

    International Nuclear Information System (INIS)

    Higgins, C.B.

    1987-01-01

    The most important diagnostic requirement in congenital heart disease (CHD) is definition of cardiovascular pathoanatomy. The considerable success in operative correction of even the most complex anomalies in recent years compels ever increasing precision in preoperative demonstration of these anomalies. Early experience with magnetic resonance imaging (MRI) at several institutions indicated that this modality is an effective noninvasive technique for evaluation of CHD. Indeed, MRI seems to have some advantage over other techniques, including angiography, for definitive diagnosis of congenital anomalies of the heart and great arteries and veins. The absence of ionizing radiation and contrast medium in MRI is an additional advantage; the former is particularly important for children, who, up to this time, have frequently been subjected to enormous radiation burdens from multiple cineangiograms during initial diagnosis and follow-up. This chapter describes the MRI appearance of cardiovascular anatomy im the segmental fashion proposed for analysis of complex CHD. Likewise, MRI demonstration of congenital cardiovascular lesions is organized into abnormalities situated at the four segmental cardiovascular levels: great vessels, atria, ventricles, and visceroatrial relationship. The role of MRI in evaluation of complex ventricular anomalies such as single ventricle and thoracic aortic abnormalities is specifically described

  20. Separate origins of the internal and external carotid arteries depicted on CT angiography: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Choi, Kwang Ho [Dept. of horacic and Cardiovascular Surgery, Pusan National University Yangsan Hospital, Yangsan (Korea, Republic of); Beak, Hye Jin; Jung, Hyun Kyung [Dept. of Radiology, Haeundae Paik Hospital, Inje University College of Medicine, Busan (Korea, Republic of)

    2015-08-15

    Agenesis of the common carotid artery (CCA) is a rare congenital anomaly. We presented a rare case of unilateral congenital absence of the right CCA with separate origins of the ipsilateral internal and external carotid arteries from the brachiocephalic artery. Further, we reviewed the embryological mechanism and clinical importance of this anomaly.

  1. Functional studies of the human auditory cortex, auditory memory and musical hallucinations

    International Nuclear Information System (INIS)

    Goycoolea, Marcos; Mena, Ismael; Neubauer, Sonia

    2004-01-01

    of Brodmann, more intense in the contralateral (right) side. There is activation of both frontal executive areas without lateralization. Simultaneously, while area 39 of Brodmann was being activated, the temporal lobe was being inhibited. This seemingly not previously reported functional observation is suggestive that also inhibitory and not only excitatory relays play a role in the auditory pathways. The central activity in our patient (without external auditory stimuli) -who was tested while having musical hallucinations- was a mirror image of that of our normal stimulated volunteers. It is suggested that the trigger role of the inner ear -if any- could conceivably be inhibitory, desinhibitory and not necessarily purely excitatory. Based on our observations the trigger effect in our patient, could occur via the left ear. Finally, our functional studies are suggestive that auditory memory for musical perceptions could be seemingly located in the right area 39 of Brodmann (Au)

  2. Impaired short-term memory for pitch in congenital amusia.

    Science.gov (United States)

    Tillmann, Barbara; Lévêque, Yohana; Fornoni, Lesly; Albouy, Philippe; Caclin, Anne

    2016-06-01

    Congenital amusia is a neuro-developmental disorder of music perception and production. The hypothesis is that the musical deficits arise from altered pitch processing, with impairments in pitch discrimination (i.e., pitch change detection, pitch direction discrimination and identification) and short-term memory. The present review article focuses on the deficit of short-term memory for pitch. Overall, the data discussed here suggest impairments at each level of processing in short-term memory tasks; starting with the encoding of the pitch information and the creation of the adequate memory trace, the retention of the pitch traces over time as well as the recollection and comparison of the stored information with newly incoming information. These impairments have been related to altered brain responses in a distributed fronto-temporal network, associated with decreased connectivity between these structures, as well as in abnormalities in the connectivity between the two auditory cortices. In contrast, amusic participants׳ short-term memory abilities for verbal material are preserved. These findings show that short-term memory deficits in congenital amusia are specific to pitch, suggesting a pitch-memory system that is, at least partly, separated from verbal memory. This article is part of a Special Issue entitled SI: Auditory working memory. Copyright © 2015 Elsevier B.V. All rights reserved.

  3. Murine model for congenital CMV infection and hearing impairment

    Directory of Open Access Journals (Sweden)

    Tao Liu

    2011-02-01

    Full Text Available Abstract Background Congenital cytomegalovirus (CMV infection is the leading cause of sensorineural hearing loss (SNHL, and SNHL is the most frequent sequela of congenital CMV infection. But the pathogenic mechanism remains unknown, and there is no ideal CMV intrauterine infection animal model to study the mechanisms by which SNHL develops. Methods We established the congenital murine cytomegalovirus (MCMV infection model by directly injecting the virus into the placenta on day 12.5 of gestation. Then, we observed the development and the MCMV congenital infection rate of the fetuses on the day they were born. Furthermore, we detected the auditory functions, the conditions of the MCMV infection, and the histological change of the inner ears of 28-day-old and 70-day-old offspring. Results Both the fetal loss rate and the teratism rate of offspring whose placentas were inoculated with MCMV increased, and their body length, head circumference, and weight decreased. The hearing level of offspring both decreased at both 28- and 70-days post birth; the 70-day-old mice developed lower hearing levels than did the 28-day old mice. No significant inflammatory changes in the cochleae of the mice were observed. MCMV DNA signals were mainly detected in the spiral ganglion neurons and the endolymph area, but not in the perilymph area. The number of neurons decreased, and their ultrastructures changed. Moreover, with age, the number of neurons dramatically decreased, and the ultrastructural lesions of neurons became much more severe. Conclusions The results suggest that the direct injection of MCMV into the placenta may efficiently cause fetal infection and disturb the intrauterine development of the fetus, and placental inoculation itself has no obvious adverse effects on offspring. The reduction in the number of spiral ganglion neurons and the ultrastructural lesions of the neurons may be the major cause of congenital CMV infection-induced progressive SNHL.

  4. Radiology of congenital heart disease

    International Nuclear Information System (INIS)

    Amplatz, K.

    1986-01-01

    This is a text on the radiologic diagnosis of congenital heart disease and its clinical manifestations. The main thrust of the book is the logical approach which allows an understanding of the complex theory of congenital heart disease. The atlas gives a concise overview of the entire field of congenital heart disease. Emphasis is placed on the understanding of the pathophysiology and its clinical and radiological consequences. Surgical treatment is included since it provides a different viewpoint of the anatomy

  5. Auditory short-term memory in the primate auditory cortex.

    Science.gov (United States)

    Scott, Brian H; Mishkin, Mortimer

    2016-06-01

    Sounds are fleeting, and assembling the sequence of inputs at the ear into a coherent percept requires auditory memory across various time scales. Auditory short-term memory comprises at least two components: an active ׳working memory' bolstered by rehearsal, and a sensory trace that may be passively retained. Working memory relies on representations recalled from long-term memory, and their rehearsal may require phonological mechanisms unique to humans. The sensory component, passive short-term memory (pSTM), is tractable to study in nonhuman primates, whose brain architecture and behavioral repertoire are comparable to our own. This review discusses recent advances in the behavioral and neurophysiological study of auditory memory with a focus on single-unit recordings from macaque monkeys performing delayed-match-to-sample (DMS) tasks. Monkeys appear to employ pSTM to solve these tasks, as evidenced by the impact of interfering stimuli on memory performance. In several regards, pSTM in monkeys resembles pitch memory in humans, and may engage similar neural mechanisms. Neural correlates of DMS performance have been observed throughout the auditory and prefrontal cortex, defining a network of areas supporting auditory STM with parallels to that supporting visual STM. These correlates include persistent neural firing, or a suppression of firing, during the delay period of the memory task, as well as suppression or (less commonly) enhancement of sensory responses when a sound is repeated as a ׳match' stimulus. Auditory STM is supported by a distributed temporo-frontal network in which sensitivity to stimulus history is an intrinsic feature of auditory processing. This article is part of a Special Issue entitled SI: Auditory working memory. Published by Elsevier B.V.

  6. Auditory short-term memory in the primate auditory cortex

    OpenAIRE

    Scott, Brian H.; Mishkin, Mortimer

    2015-01-01

    Sounds are fleeting, and assembling the sequence of inputs at the ear into a coherent percept requires auditory memory across various time scales. Auditory short-term memory comprises at least two components: an active ���working memory��� bolstered by rehearsal, and a sensory trace that may be passively retained. Working memory relies on representations recalled from long-term memory, and their rehearsal may require phonological mechanisms unique to humans. The sensory component, passive sho...

  7. Behaviour Problems in Children with Congenital Heart Disease

    Directory of Open Access Journals (Sweden)

    Beena Johnson

    2015-01-01

    Full Text Available As more children survive with congenital heart diseases, management of their behavioural problems are becoming increasingly important. In this article we aim to review the current status of knowledge on this aspect. Children with congenital heart diseases have more behavioural problems compared to children without chronic illnesses. Behavioural problems in children can be classified into externalizing behaviours and internalizing behaviours. Externalizing behaviours are marked by defiance, impulsivity, hyperactivity, disruptiveness, aggression and antisocial features. Internalizing behaviours are evidenced by withdrawal, dysphoria and anxiety. Boys with congenital heart diseases have more externalizing problems compared to girls. Preoperative hypoxia as well as peri and postoperative cardiocirculatory insufficiency can lead to internalizing and externalizing behavioral problems in future. High treatment intensity and palliative interventions are associated with poor behavioral outcomes. Children who underwent open heart surgery at very young age are prone to develop attention-deficit hyperactivity disorder on reaching school age. A comprehensive approach in this field is essential, so that effective early interventions and guidance can be planned.

  8. Early detection of congenital syphilis

    Directory of Open Access Journals (Sweden)

    Nagalakshmi Chowdhary

    2014-01-01

    Full Text Available Late congenital syphilis is a very rare clinical entity, and its early diagnosis and treatment is essential. Dental findings often provide valuable evidence for the diagnosis of late congenital syphilis. It occurs due to the transmission of the disease from an infected mother to her fetus through placenta. This long forgotten disease continues to effect pregnant women resulting in perinatal morbidity and mortality. Congenital syphilis is a preventable disease, and its presence reflects a failure of prenatal care delivery system, as well as syphilis control programs. We are reporting a case of late congenital syphilis with only Hutchinson′s teeth.

  9. Genetics Home Reference: congenital mirror movement disorder

    Science.gov (United States)

    ... Health Conditions Congenital mirror movement disorder Congenital mirror movement disorder Printable PDF Open All Close All Enable ... view the expand/collapse boxes. Description Congenital mirror movement disorder is a condition in which intentional movements ...

  10. Maps of the Auditory Cortex.

    Science.gov (United States)

    Brewer, Alyssa A; Barton, Brian

    2016-07-08

    One of the fundamental properties of the mammalian brain is that sensory regions of cortex are formed of multiple, functionally specialized cortical field maps (CFMs). Each CFM comprises two orthogonal topographical representations, reflecting two essential aspects of sensory space. In auditory cortex, auditory field maps (AFMs) are defined by the combination of tonotopic gradients, representing the spectral aspects of sound (i.e., tones), with orthogonal periodotopic gradients, representing the temporal aspects of sound (i.e., period or temporal envelope). Converging evidence from cytoarchitectural and neuroimaging measurements underlies the definition of 11 AFMs across core and belt regions of human auditory cortex, with likely homology to those of macaque. On a macrostructural level, AFMs are grouped into cloverleaf clusters, an organizational structure also seen in visual cortex. Future research can now use these AFMs to investigate specific stages of auditory processing, key for understanding behaviors such as speech perception and multimodal sensory integration.

  11. Demodulation Processes in Auditory Perception

    National Research Council Canada - National Science Library

    Feth, Lawrence

    1997-01-01

    The long range goal of this project was the understanding of human auditory processing of information conveyed by complex, time varying signals such as speech, music or important environmental sounds...

  12. Congenital candidiasis as a subject of research in medicine and human ecology.

    Science.gov (United States)

    Skoczylas, Michał M; Walat, Anna; Kordek, Agnieszka; Loniewska, Beata; Rudnicki, Jacek; Maleszka, Romuald; Torbé, Andrzej

    2014-01-01

    Congenital candidiasis is a severe complication of candidal vulvovaginitis. It occurs in two forms,congenital mucocutaneous candidiasis and congenital systemic candidiasis. Also newborns are in age group the most vulnerable to invasive candidiasis. Congenital candidiasis should be considered as an interdisciplinary problem including maternal and fetal condition (including antibiotic therapy during pregnancy), birth age and rare genetic predispositions as severe combined immunodeficiency or neutrophil-specific granule deficiency. Environmental factors are no less important to investigate in diagnosing, treatment and prevention. External factors (e.g., food) and microenvironment of human organism (microflora of the mouth, intestine and genitalia) are important for solving clinical problems connected to congenital candidiasis. Physician knowledge about microorganisms in a specific compartments of the microenvironment of human organism and in the course of defined disorders of homeostasis makes it easier to predict the course of the disease and allows the development of procedures that can be extremely helpful in individualized diagnostic and therapeutic process.

  13. Biomedical Simulation Models of Human Auditory Processes

    Science.gov (United States)

    Bicak, Mehmet M. A.

    2012-01-01

    Detailed acoustic engineering models that explore noise propagation mechanisms associated with noise attenuation and transmission paths created when using hearing protectors such as earplugs and headsets in high noise environments. Biomedical finite element (FE) models are developed based on volume Computed Tomography scan data which provides explicit external ear, ear canal, middle ear ossicular bones and cochlea geometry. Results from these studies have enabled a greater understanding of hearing protector to flesh dynamics as well as prioritizing noise propagation mechanisms. Prioritization of noise mechanisms can form an essential framework for exploration of new design principles and methods in both earplug and earcup applications. These models are currently being used in development of a novel hearing protection evaluation system that can provide experimentally correlated psychoacoustic noise attenuation. Moreover, these FE models can be used to simulate the effects of blast related impulse noise on human auditory mechanisms and brain tissue.

  14. Boosting pitch encoding with audiovisual interactions in congenital amusia.

    Science.gov (United States)

    Albouy, Philippe; Lévêque, Yohana; Hyde, Krista L; Bouchet, Patrick; Tillmann, Barbara; Caclin, Anne

    2015-01-01

    The combination of information across senses can enhance perception, as revealed for example by decreased reaction times or improved stimulus detection. Interestingly, these facilitatory effects have been shown to be maximal when responses to unisensory modalities are weak. The present study investigated whether audiovisual facilitation can be observed in congenital amusia, a music-specific disorder primarily ascribed to impairments of pitch processing. Amusic individuals and their matched controls performed two tasks. In Task 1, they were required to detect auditory, visual, or audiovisual stimuli as rapidly as possible. In Task 2, they were required to detect as accurately and as rapidly as possible a pitch change within an otherwise monotonic 5-tone sequence that was presented either only auditorily (A condition), or simultaneously with a temporally congruent, but otherwise uninformative visual stimulus (AV condition). Results of Task 1 showed that amusics exhibit typical auditory and visual detection, and typical audiovisual integration capacities: both amusics and controls exhibited shorter response times for audiovisual stimuli than for either auditory stimuli or visual stimuli. Results of Task 2 revealed that both groups benefited from simultaneous uninformative visual stimuli to detect pitch changes: accuracy was higher and response times shorter in the AV condition than in the A condition. The audiovisual improvements of response times were observed for different pitch interval sizes depending on the group. These results suggest that both typical listeners and amusic individuals can benefit from multisensory integration to improve their pitch processing abilities and that this benefit varies as a function of task difficulty. These findings constitute the first step towards the perspective to exploit multisensory paradigms to reduce pitch-related deficits in congenital amusia, notably by suggesting that audiovisual paradigms are effective in an appropriate

  15. Molecular and Genetic Studies of Congenital Myopathies

    Science.gov (United States)

    2018-03-21

    Central Core Disease; Centronuclear Myopathy; Congenital Fiber Type Disproportion; Multiminicore Disease; Myotubular Myopathy; Nemaline Myopathy; Rigid Spine Muscular Dystrophy; Undefined Congenital Myopathy

  16. Genetics Home Reference: Fukuyama congenital muscular dystrophy

    Science.gov (United States)

    ... with mental retardation Muscular dystrophy, congenital, Fukuyama type Muscular dystrophy, congenital, with central nervous system involvement Polymicrogyria with muscular dystrophy Related Information How ...

  17. Growth and development of infants with asymptomatic congenital cytomegalovirus infection.

    Science.gov (United States)

    Shan, Ruobing; Wang, Xiaoliang; Fu, Ping

    2009-10-31

    To observe changes in audiology, intellectual development, behavior development, and physical growth during systematic follow-up of infants with asymptomatic congenital human cytomegalovirus (HCMV) infection. Fifty-two infants diagnosed with asymptomatic congenital HCMV infection from July 2003 to July 2007 served as the infection group, and 21 healthy infants served as the control group. All infants were confirmed to have HCMV infection by Fluorescent Quantative polymerase chain reaction (FQ-PCR). In both the infection and control groups, the neonates and infants at 3 months, 6 months, and 1 year of age underwent examinations. 1) 20 items of National Black Nurses Association (NBNA) scores of neonates 12-14 days after birth in 2 groups were 38.3 +/- 1.95 and 38.5 +/- 2.29, without significant differences. 2) Auditory test: 50 ears of 25 cases in the infection group showed abnormal auditory thresholds in V waves with an abnormal rate of 14%, while no abnormalities were found in 21 cases in the control group. 3) Mental and psychomotor development index scores in the control group (107.49 +/- 11.31 and 107.19 +/- 10.98) were compared with those in 41 asymptomatically infected infants at 1 year of age (107.21 +/- 9.96 and 108.31 +/- 11.25), and no statistically significant difference was noted. 1) An elevated threshold in the V wave was present in asymptomatically infected infants, but could not be detected through otoacoustic emission (OAE) screening. 2) Either in the neonatal or infant periods, asymptomatic congenital HCMV infection did not have a significant influence on nervous behavior or on physical and intellectual development.

  18. Task demands affect spatial reference frame weighting during tactile localization in sighted and congenitally blind adults

    OpenAIRE

    Heed, Tobias; Roeder, Brigitte; Badde, Stephanie; Schubert, Jonathan

    2017-01-01

    Task demands modulate tactile localization in sighted humans, presumably through weight adjustments in the spatial integration of anatomical, skin-based, and external, posture-based information. In contrast, previous studies have suggested that congenitally blind humans, by default, refrain from automatic spatial integration and localize touch using only skin-based information. Here, sighted and congenitally blind participants localized tactile targets on the palm or back of one hand, while i...

  19. Diminished auditory sensory gating during active auditory verbal hallucinations.

    Science.gov (United States)

    Thoma, Robert J; Meier, Andrew; Houck, Jon; Clark, Vincent P; Lewine, Jeffrey D; Turner, Jessica; Calhoun, Vince; Stephen, Julia

    2017-10-01

    Auditory sensory gating, assessed in a paired-click paradigm, indicates the extent to which incoming stimuli are filtered, or "gated", in auditory cortex. Gating is typically computed as the ratio of the peak amplitude of the event related potential (ERP) to a second click (S2) divided by the peak amplitude of the ERP to a first click (S1). Higher gating ratios are purportedly indicative of incomplete suppression of S2 and considered to represent sensory processing dysfunction. In schizophrenia, hallucination severity is positively correlated with gating ratios, and it was hypothesized that a failure of sensory control processes early in auditory sensation (gating) may represent a larger system failure within the auditory data stream; resulting in auditory verbal hallucinations (AVH). EEG data were collected while patients (N=12) with treatment-resistant AVH pressed a button to indicate the beginning (AVH-on) and end (AVH-off) of each AVH during a paired click protocol. For each participant, separate gating ratios were computed for the P50, N100, and P200 components for each of the AVH-off and AVH-on states. AVH trait severity was assessed using the Psychotic Symptoms Rating Scales AVH Total score (PSYRATS). The results of a mixed model ANOVA revealed an overall effect for AVH state, such that gating ratios were significantly higher during the AVH-on state than during AVH-off for all three components. PSYRATS score was significantly and negatively correlated with N100 gating ratio only in the AVH-off state. These findings link onset of AVH with a failure of an empirically-defined auditory inhibition system, auditory sensory gating, and pave the way for a sensory gating model of AVH. Copyright © 2017 Elsevier B.V. All rights reserved.

  20. Prevalence of Congenital Malformations

    Directory of Open Access Journals (Sweden)

    Akhavan Karbasi Sedighah

    2009-05-01

    Full Text Available Congenital malformation (CM will begin to emerge as one of the major childhood health problems .Treatment and rehabilitation of children with congenital malformations are costly and complete recovery is usually impossible. The aim of this study was to determine frequency of CM in Yazd central city of the Islamic Republic of Iran to find out if there has been any difference in the rate and types of CM in this area. This descriptive-observational study carried on 4800 births delivered at all maternity hospitals in Yazd from October 2003 to June 2004. Prevalence of CM was 2.83% (2.86 % in male and 2.68 % in female out of the 136 cases 69(51.88% were males and 64 (48.12% were females and 3 with ambiguous genitalia. Positive family history of CM in sibling was in only 6 cases (4.41%.Overall, musculoskeletal (0.83%, central nerv-ous system (0.47% and genital system (0.37% were accounted as the most common. Frequency of CM was more seen in still birth (12.5% as in comparison to live birth (2.71%. There was not statistical difference be-tween prevalence of CM and neonatal's gender, gestational age, birth order and mother's age, drug ingestion, illness and parental consanguinity. In this study the overall prevalence of congenital malformation among the newborn was higher than those previous reported in Iran and determining the causes of this difference needs more extensive studies.

  1. Auditory Hallucinations as Translational Psychiatry: Evidence from Magnetic Resonance Imaging.

    Science.gov (United States)

    Hugdahl, Kenneth

    2017-12-01

    In this invited review article, I present a translational perspective and overview of our research on auditory hallucinations in schizophrenia at the University of Bergen, Norway, with a focus on the neuronal mechanisms underlying the phenomenology of experiencing "hearing voices". An auditory verbal hallucination (i.e. hearing a voice) is defined as a sensory experience in the absence of a corresponding external sensory source that could explain the phenomenological experience. I suggest a general frame or scheme for the study of auditory verbal hallucinations, called Levels of Explanation. Using a Levels of Explanation approach, mental phenomena can be described and explained at different levels (cultural, clinical, cognitive, brain-imaging, cellular and molecular). Another way of saying this is that, to advance knowledge in a research field, it is not only necessary to replicate findings, but also to show how evidence obtained with one method, and at one level of explanation, converges with evidence obtained with another method at another level. To achieve breakthroughs in our understanding of auditory verbal hallucinations, we have to advance vertically through the various levels, rather than the more common approach of staying at our favourite level and advancing horizontally (e.g., more advanced techniques and data acquisition analyses). The horizontal expansion will, however, not advance a deeper understanding of how an auditory verbal hallucination spontaneously starts and stops. Finally, I present data from the clinical, cognitive, brain-imaging, and cellular levels, where data from one level validate and support data at another level, called converging of evidence. Using a translational approach, the current status of auditory verbal hallucinations is that they implicate speech perception areas in the left temporal lobe, impairing perception of and attention to external sounds. Preliminary results also show that amygdala is implicated in the emotional

  2. Auditory Hallucinations as Translational Psychiatry: Evidence from Magnetic Resonance Imaging

    Directory of Open Access Journals (Sweden)

    Kenneth Hugdahl

    2017-12-01

    Full Text Available In this invited review article, I present a translational perspective and overview of our research on auditory hallucinations in schizophrenia at the University of Bergen, Norway, with a focus on the neuronal mechanisms underlying the phenomenology of experiencing "hearing voices". An auditory verbal hallucination (i.e. hearing a voice is defined as a sensory experience in the absence of a corresponding external sensory source that could explain the phenomenological experience. I suggest a general frame or scheme for the study of auditory verbal hallucinations, called Levels of Explanation. Using a Levels of Explanation approach, mental phenomena can be described and explained at different levels (cultural, clinical, cognitive, brain-imaging, cellular and molecular. Another way of saying this is that, to advance knowledge in a research field, it is not only necessary to replicate findings, but also to show how evidence obtained with one method, and at one level of explanation, converges with evidence obtained with another method at another level. To achieve breakthroughs in our understanding of auditory verbal hallucinations, we have to advance vertically through the various levels, rather than the more common approach of staying at our favourite level and advancing horizontally (e.g., more advanced techniques and data acquisition analyses. The horizontal expansion will, however, not advance a deeper understanding of how an auditory verbal hallucination spontaneously starts and stops. Finally, I present data from the clinical, cognitive, brain-imaging, and cellular levels, where data from one level validate and support data at another level, called converging of evidence. Using a translational approach, the current status of auditory verbal hallucinations is that they implicate speech perception areas in the left temporal lobe, impairing perception of and attention to external sounds. Preliminary results also show that amygdala is implicated in

  3. Congenital Midureteric Stricture

    Directory of Open Access Journals (Sweden)

    Singh Shalinder

    2001-01-01

    Full Text Available Congenital midureteric obstruction is a rare entity which can be caused by either ureteric valves or strictures. We report our experience with four patients with midureteric obstruction due to stricture over a six-year period. The condition needs to be differentiated from obstruction of the pelviureteric and vesicoureteric junctions. Obstruction can be initially screened by ultrasound and confirmed by a radionuclide scan with furosemide challenge. Retrograde ureteropyelography and/or intravenous urography may be required to define the level of the stricture.

  4. Nonclassic Congenital Adrenal Hyperplasia

    Directory of Open Access Journals (Sweden)

    Selma Feldman Witchel

    2010-01-01

    Full Text Available Nonclassic congenital adrenal hyperplasia (NCAH due to P450c21 (21-hydroxylase deficiency is a common autosomal recessive disorder. This disorder is due to mutations in the CYP21A2 gene which is located at chromosome 6p21. The clinical features predominantly reflect androgen excess rather than adrenal insufficiency leading to an ascertainment bias favoring diagnosis in females. Treatment goals include normal linear growth velocity and “on-time” puberty in affected children. For adolescent and adult women, treatment goals include regularization of menses, prevention of progression of hirsutism, and fertility. This paper will review key aspects regarding pathophysiology, diagnosis, and treatment of NCAH.

  5. Effects of vocal training in a musicophile with congenital amusia.

    Science.gov (United States)

    Wilbiks, Jonathan M P; Vuvan, Dominique T; Girard, Pier-Yves; Peretz, Isabelle; Russo, Frank A

    2016-12-01

    Congenital amusia is a condition in which an individual suffers from a deficit of musical pitch perception and production. Individuals suffering from congenital amusia generally tend to abstain from musical activities. Here, we present the unique case of Tim Falconer, a self-described musicophile who also suffers from congenital amusia. We describe and assess Tim's attempts to train himself out of amusia through a self-imposed 18-month program of formal vocal training and practice. We tested Tim with respect to music perception and vocal production across seven sessions including pre- and post-training assessments. We also obtained diffusion-weighted images of his brain to assess connectivity between auditory and motor planning areas via the arcuate fasciculus (AF). Tim's behavioral and brain data were compared to that of normal and amusic controls. While Tim showed temporary gains in his singing ability, he did not reach normal levels, and these gains faded when he was not engaged in regular lessons and practice. Tim did show some sustained gains with respect to the perception of musical rhythm and meter. We propose that Tim's lack of improvement in pitch perception and production tasks is due to long-standing and likely irreversible reduction in connectivity along the AF fiber tract.

  6. Multislice spiral computed tomography imaging in congenital inner ear malformations.

    Science.gov (United States)

    Ma, Hui; Han, Ping; Liang, Bo; Tian, Zhi-liang; Lei, Zi-qiao; Kong, Wei-jia; Feng, Gan-sheng

    2008-01-01

    The purpose of this study is to evaluate the usefulness of multislice spiral computed tomography (CT) in the diagnosis of congenital inner ear malformations. Forty-four patients with sensorineural hearing loss were examined on a Somatom Sensation 16 (Siemens) CT scanner. The 3-dimensional reconstructions and multiplanar reformation (MPR) were performed using the volume-rendering technique (VRT) on the workstation. Of the 44 patients examined for this study, 25 patients were found to be normal and 19 patients (36 ears) were diagnosed with congenital inner ear malformations. Of the malformations, the axial, MPR, and VRT images can all display the site and degree in 33 of the ears. Volume-rendering technique images were superior to the axial images in displaying the malformations in 3 ears with small lateral semicircular canal malformations. The common malformations were Michel deformity (1 ear), common cavity deformity (3 ears), incomplete partition I (3 ears), incomplete partition II (Mondini deformity) (5 ears), vestibular and semicircular canal malformations (14 ears), enlarged vestibular aqueduct (16 ears, 6 of which had other malformations), and internal auditory canal malformation (8 ears, all accompanied by other malformations). Multislice spiral CT allows a comprehensively assessment of various congenital inner ear malformations through high-quality MPR and VRT reconstructions. Volume-rendering technique images can display the site and degree of the malformation 3-dimensionally and intuitionisticly. This is very useful to the cochlear implantation.

  7. Congenital Syphilis Masquerading as Leukemia

    OpenAIRE

    Lee, Tiffany; Bell, Stephanie; Scimeme, Jason; Maraqa, Nizar

    2017-01-01

    As of late, the incidence of congenital syphilis in the United States is increasing. Each new case represents a failure of preventing, diagnosing, and treating syphilis in pregnant women. Pediatricians should confirm that all women have received adequate screening for and management of syphilis during pregnancy. Congenital syphilis is easily treatable but may be a diagnostic challenge with high morbidity and mortality.

  8. What Are Congenital Heart Defects?

    Science.gov (United States)

    ... a baby with a congenital heart defect. Family history and genetics Congenital heart disease is not usually passed along ... you or your child to a specialist in genetic testing. Cardiac MRI to diagnose a ... Factors to review family history, smoking, and medicines that increase your risk of ...

  9. Temporal Organization of Sound Information in Auditory Memory

    Directory of Open Access Journals (Sweden)

    Kun Song

    2017-06-01

    Full Text Available Memory is a constructive and organizational process. Instead of being stored with all the fine details, external information is reorganized and structured at certain spatiotemporal scales. It is well acknowledged that time plays a central role in audition by segmenting sound inputs into temporal chunks of appropriate length. However, it remains largely unknown whether critical temporal structures exist to mediate sound representation in auditory memory. To address the issue, here we designed an auditory memory transferring study, by combining a previously developed unsupervised white noise memory paradigm with a reversed sound manipulation method. Specifically, we systematically measured the memory transferring from a random white noise sound to its locally temporal reversed version on various temporal scales in seven experiments. We demonstrate a U-shape memory-transferring pattern with the minimum value around temporal scale of 200 ms. Furthermore, neither auditory perceptual similarity nor physical similarity as a function of the manipulating temporal scale can account for the memory-transferring results. Our results suggest that sounds are not stored with all the fine spectrotemporal details but are organized and structured at discrete temporal chunks in long-term auditory memory representation.

  10. Temporal Organization of Sound Information in Auditory Memory.

    Science.gov (United States)

    Song, Kun; Luo, Huan

    2017-01-01

    Memory is a constructive and organizational process. Instead of being stored with all the fine details, external information is reorganized and structured at certain spatiotemporal scales. It is well acknowledged that time plays a central role in audition by segmenting sound inputs into temporal chunks of appropriate length. However, it remains largely unknown whether critical temporal structures exist to mediate sound representation in auditory memory. To address the issue, here we designed an auditory memory transferring study, by combining a previously developed unsupervised white noise memory paradigm with a reversed sound manipulation method. Specifically, we systematically measured the memory transferring from a random white noise sound to its locally temporal reversed version on various temporal scales in seven experiments. We demonstrate a U-shape memory-transferring pattern with the minimum value around temporal scale of 200 ms. Furthermore, neither auditory perceptual similarity nor physical similarity as a function of the manipulating temporal scale can account for the memory-transferring results. Our results suggest that sounds are not stored with all the fine spectrotemporal details but are organized and structured at discrete temporal chunks in long-term auditory memory representation.

  11. [Neonatal tumours and congenital malformations].

    Science.gov (United States)

    Berbel Tornero, O; Ortega García, J A; Ferrís i Tortajada, J; García Castell, J; Donat i Colomer, J; Soldin, O P; Fuster Soler, J L

    2008-06-01

    The association between pediatric cancer and congenital abnormalities is well known but, there is no exclusive data on the neonatal period and the underlying etiopathogenic mechanisms are unknown. First, to analyze the frequency of neonatal tumours associated with congenital abnormalities; and second, to comment on the likely etiopathogenic hypotheses of a relationship between neonatal tumours and congenital abnormalities. Historical series of neonatal tumours from La Fe University Children's Hospital in Valencia (Spain), from January 1990 to December 1999. Histological varieties of neonatal tumours and associated congenital abnormalities were described. A systematic review of the last 25 years was carried out using Medline, Cancerlit, Index Citation Science and Embase. The search profile used was the combination of "neonatal/congenital-tumors/cancer/neoplasms" and "congenital malformations/birth defects". 72 neonatal tumours were identified (2.8% of all pediatric cancers diagnosed in our hospital) and in 15 cases (20.8%) there was some associated malformation, disease or syndrome. The association between congenital abnormalities and neonatal tumours were: a) angiomas in three patients: two patients with congenital heart disease with a choanal stenosis, laryngomalacia; b) neuroblastomas in two patients: horseshoe kidney with vertebral anomalies and other with congenital heart disease; c) teratomas in two patients: one with cleft palate with vertebral anomalies and other with metatarsal varus; d) one tumour of the central nervous system with Bochdaleck hernia; e) heart tumours in four patients with tuberous sclerosis; f) acute leukaemia in one patient with Down syndrome and congenital heart disease; g) kidney tumour in one case with triventricular hydrocephaly, and h) adrenocortical tumour: hemihypertrophy. The publications included the tumours diagnosed in different pediatric periods and without unified criteria to classify the congenital abnormalities. Little data

  12. Congenital nystagmus and negative electroretinography

    Directory of Open Access Journals (Sweden)

    Roussi M

    2011-04-01

    Full Text Available Mirella Roussi, Hélène Dalens, Jean Jacques Marcellier, Franck BacinDepartment of Ophthalmology, Clermont-Ferrand University, Clermont-Ferrand, FranceAbstract: Congenital nystagmus is a pathologic oculomotor state appearing at about three to four months of age. The precise diagnosis requires detailed clinical examination and electrophysiological findings. This case report presents two male patients with congenital nystagmus examined longitudinally from the age of six months until 17-18 years of age. Clinical and electrophysiological protocols were detailed. The first results showed electronegative electroretinography in the two cases and examination combined with electroretinographic findings helped us to make the diagnosis of Congenital Night Stationary Blindness (CSNB. This diagnosis was confirmed by genetic studies. CSNB is interesting to study because through electrophysiological findings, it enables a better understanding of the physiology of neural transmission in the outer part of the retina.Keywords: Congenital nystagmus, negative electroretinography, congenital night stationary blindness

  13. Congenital Leukemia in Down's syndrome

    International Nuclear Information System (INIS)

    Iqbal, W.; Khan, F.; Muzaffar, M.; Khan, U. A.; Rehman, M. U.; Khan, M. A.; Bari, A.

    2006-01-01

    Congenital Leukemia is a condition and often associated with fatal outcome/sup 1/. Most of the neonatal cases reported have acute non-lymphoblastic leukemia, in contrast to the predominance of acute lymphoblastic leukemia found in later childhood. congenital leukemia is occasionally associated with number of congenital anomalies and with chromosomal disorders such as Down's syndrome. Subtle cytogenetic abnormalities may occur more commonly in the affected infants and their parents, when studied with newer cytogenetic techniques/sup 2/. Inherent unstable hematopoieses resulting from chromosomal aberration in children with Downs's syndrome can present with transient myeloproliferative disorder, mimicking leukemia which undergoes spontaneous recovery/sup 3/. Only few cases of congenital leukemia with Downs syndrome, presented as congenital leukemia. (author)

  14. Middle ear implant in conductive and mixed congenital hearing loss in children.

    Science.gov (United States)

    Roman, Stéphane; Denoyelle, Françoise; Farinetti, Anne; Garabedian, Erea-Noel; Triglia, Jean-Michel

    2012-12-01

    Active middle ear implant can be used in children and adolescents with congenital hearing loss. The authors report their experience with the semi implantable Medel Vibrant Soundbridge(®) (VSB) in the audiologic rehabilitation of such patients. In this retrospective study, audiological and surgical data of 10 children (10.5±4 years) implanted with 12 VSB in 2 tertiary cares ENT Departments were analysed. Two children with bilateral external auditory canal (EAC) atresia and mixed hearing loss (mean air conduction (AC) thresholds=65dB HL) were bilaterally implanted. Eight children presented with microtia associated with EAC atresia bilaterally (n=3) and unilaterally (n=5). All of them had a conductive hearing loss in the implanted ear (mean (AC) thresholds were 58.75dB HL preoperatively). The Floating Mass Transducer was crimped on the long process of the incus (n=8) or on the suprastructure of the stapes (n=4). There were no intra- or postoperative surgical complications. All the children wore their implants after 5 weeks. Postoperative mean bone conduction (BC) thresholds were unchanged. The mean aided thresholds with VSB (four frequencies warble tones at 0.5, 1, 2 and 4 kHz) were 28dB HL (± 10). Word discrimination threshold in quiet conditions in free field with the VSB unilaterally activated was 50% at 38dB SPL (± 9). The results indicate that satisfaction of the children and their parents is very encouraging but surgeons should be cautious with this new approach in relation to the pinna reconstruction and to possible risks to inner ear and facial nerve. Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.

  15. Congenital nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    Claudia Fanni

    2014-06-01

    Full Text Available CNS (Congenital nephrotic syndrome is a disorder characterized by the presence of a nephrotic syndrome in the first three months of life. Different pathologies can cause this syndrome. In general, we can distinguish primary forms (sporadic and hereditary and secondary forms (acquired and associated with other syndromes. The most common form is the Finnish CNS (CNF, congenital nephrotic syndrome of the Finnish type, a hereditary form whose name derives from the fact that the highest incidence is described in that country (1.2:10,000. The pathogenesis, the clinical picture, the diagnostic criteria, the therapy and the outcome are described in details.  Proceedings of the International Course on Perinatal Pathology (part of the 10th International Workshop on Neonatology · October 22nd-25th, 2014 · Cagliari (Italy · October 25th, 2014 · The role of the clinical pathological dialogue in problem solving Guest Editors: Gavino Faa, Vassilios Fanos, Peter Van Eyken

  16. Congenital extrahepatic portosystemic shunts

    Energy Technology Data Exchange (ETDEWEB)

    Murray, Conor P.; Yoo, Shi-Joon; Babyn, Paul S. [Department of Diagnostic Imaging, Hospital for Sick Children, 555 University Avenue, M5G 1X8, Toronto, Ontario (Canada)

    2003-09-01

    A congenital extrahepatic portosystemic shunt (CEPS) is uncommon. A type 1 CEPS exists where there is absence of intrahepatic portal venous supply and a type 2 CEPS where this supply is preserved. The diagnosis of congenital portosystemic shunt is important because it may cause hepatic encephalopathy. To describe the clinical and imaging features of three children with CEPS and to review the cases in the published literature. The diagnostic imaging and medical records for three children with CEPS were retrieved and evaluated. An extensive literature search was performed. Including our cases, there are 61 reported cases of CEPS, 39 type 1 and 22 type 2. Type 1 occurs predominantly in females, while type 2 shows no significant sexual preponderance. The age at diagnosis ranges from 31 weeks of intrauterine life to 76 years. Both types of CEPS have a number of associations, the most common being nodular lesions of the liver (n=25), cardiac anomalies (n=19), portosystemic encephalopathy (n=10), polysplenia (n=9), biliary atresia (n=7), skeletal anomalies (n=5), and renal tract anomalies (n=4). MRI is recommended as an important means of diagnosing and classifying cases of CEPS and examining the associated cardiovascular and hepatic abnormalities. Screening for CEPS in patients born with polysplenia is suggested. (orig.)

  17. Congenital extrahepatic portosystemic shunts

    International Nuclear Information System (INIS)

    Murray, Conor P.; Yoo, Shi-Joon; Babyn, Paul S.

    2003-01-01

    A congenital extrahepatic portosystemic shunt (CEPS) is uncommon. A type 1 CEPS exists where there is absence of intrahepatic portal venous supply and a type 2 CEPS where this supply is preserved. The diagnosis of congenital portosystemic shunt is important because it may cause hepatic encephalopathy. To describe the clinical and imaging features of three children with CEPS and to review the cases in the published literature. The diagnostic imaging and medical records for three children with CEPS were retrieved and evaluated. An extensive literature search was performed. Including our cases, there are 61 reported cases of CEPS, 39 type 1 and 22 type 2. Type 1 occurs predominantly in females, while type 2 shows no significant sexual preponderance. The age at diagnosis ranges from 31 weeks of intrauterine life to 76 years. Both types of CEPS have a number of associations, the most common being nodular lesions of the liver (n=25), cardiac anomalies (n=19), portosystemic encephalopathy (n=10), polysplenia (n=9), biliary atresia (n=7), skeletal anomalies (n=5), and renal tract anomalies (n=4). MRI is recommended as an important means of diagnosing and classifying cases of CEPS and examining the associated cardiovascular and hepatic abnormalities. Screening for CEPS in patients born with polysplenia is suggested. (orig.)

  18. External and internal limitations in amplitude-modulation processing

    DEFF Research Database (Denmark)

    Ewert, Stephan; Dau, Torsten

    2004-01-01

    Three experiments are presented to explore the relative role of "external" signal variability and "internal" resolution limitations of the auditory system in the detection and discrimination of amplitude modulations (AM). In the first experiment, AM-depth discrimination performance was determined......-filterbank models. The predictions revealed that AM-depth discrimination and AM detection are limited by a combination of the external signal variability and an internal "Weber-fraction" noise process....

  19. Human Immunodeficiency virus and invasive external otitis- A case ...

    African Journals Online (AJOL)

    Acute invasive external otitis is an uncommon life-threatening infection of the exteernal auditory canal (EAC), most often affecting the elderly diabetic patients. Although few reports have been made in HIV-positive/Aids patients among the caucasians. We present here a 25 year old nursing mother with a month history of ...

  20. Brainstem auditory evoked responses in an equine patient population: part I--adult horses.

    Science.gov (United States)

    Aleman, M; Holliday, T A; Nieto, J E; Williams, D C

    2014-01-01

    Brainstem auditory evoked response has been an underused diagnostic modality in horses as evidenced by few reports on the subject. To describe BAER findings, common clinical signs, and causes of hearing loss in adult horses. Study group, 76 horses; control group, 8 horses. Retrospective. BAER records from the Clinical Neurophysiology Laboratory were reviewed from the years of 1982 to 2013. Peak latencies, amplitudes, and interpeak intervals were measured when visible. Horses were grouped under disease categories. Descriptive statistics and a posthoc Bonferroni test were performed. Fifty-seven of 76 horses had BAER deficits. There was no breed or sex predisposition, with the exception of American Paint horses diagnosed with congenital sensorineural deafness. Eighty-six percent (n = 49/57) of the horses were younger than 16 years of age. The most common causes of BAER abnormalities were temporohyoid osteoarthropathy (THO, n = 20/20; abnormalities/total), congenital sensorineural deafness in Paint horses (17/17), multifocal brain disease (13/16), and otitis media/interna (4/4). Auditory loss was bilateral and unilateral in 74% (n = 42/57) and 26% (n = 15/57) of the horses, respectively. The most common causes of bilateral auditory loss were sensorineural deafness, THO, and multifocal brain disease whereas THO and otitis were the most common causes of unilateral deficits. Auditory deficits should be investigated in horses with altered behavior, THO, multifocal brain disease, otitis, and in horses with certain coat and eye color patterns. BAER testing is an objective and noninvasive diagnostic modality to assess auditory function in horses. Copyright © 2014 by the American College of Veterinary Internal Medicine.

  1. Fetal chromosome abnormalities and congenital malformations: an ...

    African Journals Online (AJOL)

    The results also showed that Multiple congenital anomalies (MCA) represented among 42.2%, congenital malformation of CNS represents 26.6%, congenital malformation of the skeletal system 20%, congenital polycystic kidney 8.8% and pyloric stenosis in 2.2%. Among the 21 women with abnormal karyotype of amniotic ...

  2. Genetics Home Reference: critical congenital heart disease

    Science.gov (United States)

    ... Facebook Twitter Home Health Conditions Critical congenital heart disease Critical congenital heart disease Printable PDF Open All Close All ... for Disease Control and Prevention: Congenital Heart Defects Disease InfoSearch: Congenital Heart Defects KidsHealth from Nemours Lucile Packard Children's ...

  3. General Concepts in Adult Congenital Heart Disease.

    Science.gov (United States)

    Mutluer, Ferit Onur; Çeliker, Alpay

    2018-01-20

    Congenital heart disease in adults (adult congenital heart disease) is a growing burden for healthcare systems. While infant mortality due to congenital heart disease in the last four decades decreased by almost 3-fold, adult congenital heart disease prevalence increased by more than 2-fold in United States. Adult congenital heart disease prevalence is expected to increase steadily until 2050 in projections. Adult congenital heart disease is a multifaceted problem with many dimensions. This manuscript aims to provide an overview of the common adult congenital heart diseases and summarize important points in management of these diseases with possible problems and complications that the patients and the physicians face.

  4. General Concepts in Adult Congenital Heart Disease

    Directory of Open Access Journals (Sweden)

    Ferit Onur Mutluer

    2018-02-01

    Full Text Available Congenital heart disease in adults (adult congenital heart disease is a growing burden for healthcare systems. While infant mortality due to congenital heart disease in the last four decades decreased by almost 3-fold, adult congenital heart disease prevalence increased by more than 2-fold in United States. Adult congenital heart disease prevalence is expected to increase steadily until 2050 in projections. Adult congenital heart disease is a multifaceted problem with many dimensions. This manuscript aims to provide an overview of the common adult congenital heart diseases and summarize important points in management of these diseases with possible problems and complications that the patients and the physicians face

  5. Auditory Hallucinations in Acute Stroke

    Directory of Open Access Journals (Sweden)

    Yair Lampl

    2005-01-01

    Full Text Available Auditory hallucinations are uncommon phenomena which can be directly caused by acute stroke, mostly described after lesions of the brain stem, very rarely reported after cortical strokes. The purpose of this study is to determine the frequency of this phenomenon. In a cross sectional study, 641 stroke patients were followed in the period between 1996–2000. Each patient underwent comprehensive investigation and follow-up. Four patients were found to have post cortical stroke auditory hallucinations. All of them occurred after an ischemic lesion of the right temporal lobe. After no more than four months, all patients were symptom-free and without therapy. The fact the auditory hallucinations may be of cortical origin must be taken into consideration in the treatment of stroke patients. The phenomenon may be completely reversible after a couple of months.

  6. Profiles in congenital heart disease

    International Nuclear Information System (INIS)

    Freed, M.D.; Keane, J.F.

    1986-01-01

    Pediatric cardiology has made great strides in the diagnosis, management, and correction of complex congenital malformations in the past two decades. The foundation of these advances is a more precise understanding of the physiology and anatomy of complex lesions that has been obtained from cardiac catheterization and angiography. The techniques for catheterization of infants and children have been discussed in another paper. This chapter focuses on brief profiles of some of the more important congenital abnormalities. The incidence cited in the discussion of each abnormality pertains to a population comprises of children and adults referred to The Children's Hospital Medical Center and Peter Bent Brigham Hospital, respectively, for evaluation of congenital heart disease

  7. Congenital pyriform aperture stenosis

    International Nuclear Information System (INIS)

    Osovsky, Micky; Aizer-Danon, Anat; Horev, Gadi; Sirota, Lea

    2007-01-01

    Nasal airway obstruction is a potentially life-threatening condition in the newborn. Neonates are obligatory nasal breathers. The pyriform aperture is the narrowest, most anterior bony portion of the nasal airway, and a decrease in its cross-sectional area will significantly increase nasal airway resistance. Congenital nasal pyriform aperture stenosis (CNPAS) is a rare, unusual form of nasal obstruction. It should be considered in the differential diagnosis of any neonate or infant with signs and symptoms of upper airway compromise. It is important to differentiate this level of obstruction from the more common posterior choanal stenosis or atresia. CNPAS presents with symptoms of nasal airway obstruction, which are often characterized by episodic apnea and cyclical cyanosis. (orig.)

  8. Characterizing congenital amusia.

    Science.gov (United States)

    Stewart, Lauren

    2011-04-01

    The ability to make sense of the music in our environment involves sophisticated cognitive mechanisms that, for most people, are acquired effortlessly and in early life. A special population of individuals, with a disorder termed congenital amusia, report lifelong difficulties in this regard. Exploring the nature of this developmental disorder provides a window onto the cognitive architecture of typical musical processing, as well as allowing a study of the relationship between processing of music and other domains, such as language. The present article considers findings concerning pitch discrimination, pitch memory, contour processing, experiential aspects of music listening in amusia, and emerging evidence concerning the neurobiology of the disorder. A simplified model of melodic processing is outlined, and possible loci of the cognitive deficit are discussed.

  9. From ear to body: the auditory-motor loop in spatial cognition

    Directory of Open Access Journals (Sweden)

    Isabelle eViaud-Delmon

    2014-09-01

    Full Text Available Spatial memory is mainly studied through the visual sensory modality: navigation tasks in humans rarely integrate dynamic and spatial auditory information. In order to study how a spatial scene can be memorized on the basis of auditory and idiothetic cues only, we constructed an auditory equivalent of the Morris water maze, a task widely used to assess spatial learning and memory in rodents. Participants were equipped with wireless headphones, which delivered a soundscape updated in real time according to their movements in 3D space. A wireless tracking system (video infrared with passive markers was used to send the coordinates of the subject’s head to the sound rendering system. The rendering system used advanced HRTF-based synthesis of directional cues and room acoustic simulation for the auralization of a realistic acoustic environment. Participants were guided blindfolded in an experimental room. Their task was to explore a delimitated area in order to find a hidden auditory target, i.e. a sound that was only triggered when walking on a precise location of the area. The position of this target could be coded in relationship to auditory landmarks constantly rendered during the exploration of the area. The task was composed of a practice trial, 6 acquisition trials during which they had to memorise the localisation of the target, and 4 test trials in which some aspects of the auditory scene were modified. The task ended with a probe trial in which the auditory target was removed.The configuration of searching paths allowed observing how auditory information was coded to memorise the position of the target. They suggested that space can be efficiently coded without visual information in normal sighted subjects. In conclusion, space representation can be based on sensorimotor and auditory cues only, providing another argument in favour of the hypothesis that the brain has access to a modality-invariant representation of external space.

  10. From ear to body: the auditory-motor loop in spatial cognition.

    Science.gov (United States)

    Viaud-Delmon, Isabelle; Warusfel, Olivier

    2014-01-01

    SPATIAL MEMORY IS MAINLY STUDIED THROUGH THE VISUAL SENSORY MODALITY: navigation tasks in humans rarely integrate dynamic and spatial auditory information. In order to study how a spatial scene can be memorized on the basis of auditory and idiothetic cues only, we constructed an auditory equivalent of the Morris water maze, a task widely used to assess spatial learning and memory in rodents. Participants were equipped with wireless headphones, which delivered a soundscape updated in real time according to their movements in 3D space. A wireless tracking system (video infrared with passive markers) was used to send the coordinates of the subject's head to the sound rendering system. The rendering system used advanced HRTF-based synthesis of directional cues and room acoustic simulation for the auralization of a realistic acoustic environment. Participants were guided blindfolded in an experimental room. Their task was to explore a delimitated area in order to find a hidden auditory target, i.e., a sound that was only triggered when walking on a precise location of the area. The position of this target could be coded in relationship to auditory landmarks constantly rendered during the exploration of the area. The task was composed of a practice trial, 6 acquisition trials during which they had to memorize the localization of the target, and 4 test trials in which some aspects of the auditory scene were modified. The task ended with a probe trial in which the auditory target was removed. The configuration of searching paths allowed observing how auditory information was coded to memorize the position of the target. They suggested that space can be efficiently coded without visual information in normal sighted subjects. In conclusion, space representation can be based on sensorimotor and auditory cues only, providing another argument in favor of the hypothesis that the brain has access to a modality-invariant representation of external space.

  11. Surgical outcomes of early congenital cholesteatoma: minimally invasive transcanal approach.

    Science.gov (United States)

    Lee, Sang H; Jang, Jeong H; Lee, Dongjun; Lee, Hye-Ryung; Lee, Kyu-Yup

    2014-03-01

    To introduce a simple and alternative surgical technique, minimally invasive transcanal myringotomy (MITM), for early stage congenital cholesteatoma in children and to evaluate the feasibility and results of MITM for management of early stage congenital cholesteatoma with respect to its effectiveness and safety. Retrospective review. Between August 2008 and September 2012, a total of 36 patients with congenital cholesteatoma met the inclusion criteria and were analyzed. Patient medical records, including demographic characteristics, intraoperative findings, and follow-up records, were reviewed. Subjects consisted of 23 males (64%) and 13 females (36%), and the age at operation ranged from 12 months to 6 years (mean age = 3 years and 6 months). The number of congenital cholesteatoma was as follows: 26 patients at stage I and 10 patients at stage II. The follow-up duration was between 12 and 56 months, with an average of 30 months. There were no postoperative complications such as tympanic membrane perforation, dizziness, or secondary middle ear infection. Among 36 patients who had undergone the MITM approach for the treatment of congenital cholesteatoma, five (13.8%) showed recurrence and underwent a second-look operation. On the basis of our data, the MITM approach is a useful surgical technique for early stage congenital cholesteatoma in children. It has many advantages, in that there is no external wound and it is a simple surgical technique that involves easy postoperative care, a short operation time and hospitalization period, avoidance of serious complications, and easy repeatability for recurrence. © 2013 The American Laryngological, Rhinological and Otological Society, Inc.

  12. Pre-Attentive Auditory Processing of Lexicality

    Science.gov (United States)

    Jacobsen, Thomas; Horvath, Janos; Schroger, Erich; Lattner, Sonja; Widmann, Andreas; Winkler, Istvan

    2004-01-01

    The effects of lexicality on auditory change detection based on auditory sensory memory representations were investigated by presenting oddball sequences of repeatedly presented stimuli, while participants ignored the auditory stimuli. In a cross-linguistic study of Hungarian and German participants, stimulus sequences were composed of words that…

  13. Feature Assignment in Perception of Auditory Figure

    Science.gov (United States)

    Gregg, Melissa K.; Samuel, Arthur G.

    2012-01-01

    Because the environment often includes multiple sounds that overlap in time, listeners must segregate a sound of interest (the auditory figure) from other co-occurring sounds (the unattended auditory ground). We conducted a series of experiments to clarify the principles governing the extraction of auditory figures. We distinguish between auditory…

  14. [Congenital sensorineural deafness and associated syndromes].

    Science.gov (United States)

    Moatti, L; Garabedian, E N; Lacombe, H; Spir-Jacob, C

    1990-01-01

    The etiology of perceptive deafness, especially the congenital variety, requires investigation. The presence of a variety of signs associated with deafness constitutes an "associated syndrome" and helps to define a possible genetic origin. These syndromes only represent a small percentage of overall causes of deafness in children, since at most they account for only 10% of cases. Certain syndromes are encountered more often or are well known, others are extremely rare or have only been described recently. The authors report six of these very rare syndromes discovered among their patients: a KID syndrome, a Leopard syndrome, a Norrie syndrome, a Jervell and Lange Nielsen syndrome, a recently described entity called CEE with deafness and an External Neuro-Cochleo-Pancreatic syndrome which would not appear to have been previously described.

  15. Hypersensitivity to pain in congenital blindness

    DEFF Research Database (Denmark)

    Slimani, Hocine; Danti, Sabrina; Ricciardi, Emiliano

    2013-01-01

    Vision is important for avoiding encounters with objects in the environment that may imperil physical integrity. We tested whether, in the absence of vision, a lower pain threshold would arise from an adaptive shift to other sensory channels. We therefore measured heat and cold pain thresholds an...... that blind subjects are more attentive to signals of external threats. These findings indicate that the absence of vision from birth induces a hypersensitivity to painful stimuli, lending new support to a model of sensory integration of vision and pain processing......., congenitally blind subjects have lower heat pain thresholds, rate suprathreshold heat pain stimuli as more painful, and have increased sensitivity for cold pain stimuli. Thresholds for nonpainful thermal stimulation did not differ between groups. The results of the pain questionnaires further indicated...

  16. Altered Brain Functional Activity in Infants with Congenital Bilateral Severe Sensorineural Hearing Loss: A Resting-State Functional MRI Study under Sedation

    Directory of Open Access Journals (Sweden)

    Shuang Xia

    2017-01-01

    Full Text Available Early hearing deprivation could affect the development of auditory, language, and vision ability. Insufficient or no stimulation of the auditory cortex during the sensitive periods of plasticity could affect the function of hearing, language, and vision development. Twenty-three infants with congenital severe sensorineural hearing loss (CSSHL and 17 age and sex matched normal hearing subjects were recruited. The amplitude of low frequency fluctuations (ALFF and regional homogeneity (ReHo of the auditory, language, and vision related brain areas were compared between deaf infants and normal subjects. Compared with normal hearing subjects, decreased ALFF and ReHo were observed in auditory and language-related cortex. Increased ALFF and ReHo were observed in vision related cortex, which suggest that hearing and language function were impaired and vision function was enhanced due to the loss of hearing. ALFF of left Brodmann area 45 (BA45 was negatively correlated with deaf duration in infants with CSSHL. ALFF of right BA39 was positively correlated with deaf duration in infants with CSSHL. In conclusion, ALFF and ReHo can reflect the abnormal brain function in language, auditory, and visual information processing in infants with CSSHL. This demonstrates that the development of auditory, language, and vision processing function has been affected by congenital severe sensorineural hearing loss before 4 years of age.

  17. Audio-Tactile Integration in Congenitally and Late Deaf Cochlear Implant Users

    Science.gov (United States)

    Nava, Elena; Bottari, Davide; Villwock, Agnes; Fengler, Ineke; Büchner, Andreas; Lenarz, Thomas; Röder, Brigitte

    2014-01-01

    Several studies conducted in mammals and humans have shown that multisensory processing may be impaired following congenital sensory loss and in particular if no experience is achieved within specific early developmental time windows known as sensitive periods. In this study we investigated whether basic multisensory abilities are impaired in hearing-restored individuals with deafness acquired at different stages of development. To this aim, we tested congenitally and late deaf cochlear implant (CI) recipients, age-matched with two groups of hearing controls, on an audio-tactile redundancy paradigm, in which reaction times to unimodal and crossmodal redundant signals were measured. Our results showed that both congenitally and late deaf CI recipients were able to integrate audio-tactile stimuli, suggesting that congenital and acquired deafness does not prevent the development and recovery of basic multisensory processing. However, we found that congenitally deaf CI recipients had a lower multisensory gain compared to their matched controls, which may be explained by their faster responses to tactile stimuli. We discuss this finding in the context of reorganisation of the sensory systems following sensory loss and the possibility that these changes cannot be “rewired” through auditory reafferentation. PMID:24918766

  18. Audio-tactile integration in congenitally and late deaf cochlear implant users.

    Directory of Open Access Journals (Sweden)

    Elena Nava

    Full Text Available Several studies conducted in mammals and humans have shown that multisensory processing may be impaired following congenital sensory loss and in particular if no experience is achieved within specific early developmental time windows known as sensitive periods. In this study we investigated whether basic multisensory abilities are impaired in hearing-restored individuals with deafness acquired at different stages of development. To this aim, we tested congenitally and late deaf cochlear implant (CI recipients, age-matched with two groups of hearing controls, on an audio-tactile redundancy paradigm, in which reaction times to unimodal and crossmodal redundant signals were measured. Our results showed that both congenitally and late deaf CI recipients were able to integrate audio-tactile stimuli, suggesting that congenital and acquired deafness does not prevent the development and recovery of basic multisensory processing. However, we found that congenitally deaf CI recipients had a lower multisensory gain compared to their matched controls, which may be explained by their faster responses to tactile stimuli. We discuss this finding in the context of reorganisation of the sensory systems following sensory loss and the possibility that these changes cannot be "rewired" through auditory reafferentation.

  19. Epileptiform electroencephalogram abnormality in children with congenital sensorineural hearing loss.

    Science.gov (United States)

    El-Badry, Mohamed Mohamed; Hamdy, Nermin Aly; Sobhy, Sayed; Gamal, Reham

    2014-04-01

    This work was designed to study electroencephalogram findings in children with congenital sensorineural hearing loss and correlate these findings with the SNHL parameters as duration, etiology, severity, and type. Ninety children with bilateral congenital sensorineural hearing loss served as the study group. They were free from any neurological disorders or symptoms that are commonly associated with abnormal electroencephalogram as convulsions or loss of consciousness. Twenty children having normal hearing with no history of otological or neurological disorders served as the control group. All children participating in the study were subjected to full medical and audiological history, otological examination, neurological examination, audiological evaluation and electroencephalogram recording. Mean age of the children in the control group was 3.56 ± 2.1 years and mean age of the children in the study group was 3.8 ± 2.2 years. While none of the control children had abnormal electroencephalogram, 38 (42.2%) of children with congenital SNHL had epileptiform electroencephalogram abnormality. The epileptiform abnormality was generalized in 14 children (36.8%), focal temporal in 17 children (44.7%) and focal other than temporal in 7 children (18.4%). According to the hemispheric side affected, the abnormality was right in 14 children (36.8%), left in 10 children (26.3%) and bilateral in 14 children (36.8%). No statistically significant predominance of specific site or side of the epileptiform abnormality was found. Similarly, no statistical significant prevalent of the epileptiform abnormality was found in relation to the age or sex of children, duration of hearing loss or etiology of hearing loss (i.e., genetic vs. neonatal insults). On the other hand, the epileptiform abnormality was statistically prevalent in children with moderate degree of hearing loss, and in children with auditory neuropathy spectrum disorder. The epileptiform electroencephalogram abnormality is

  20. Congenital broncho-oesophageal fistula

    African Journals Online (AJOL)

    1983-04-09

    Apr 9, 1983 ... Rigid bronchoscopy performed under general anaesthesia .... Blackburn WR, Armour)' RA. Congenital esophago-pulmonary fistulas without esophageal atresia: an analysis of 260 fistulas in infants, children and adults.

  1. Congenital heart defect corrective surgeries

    Science.gov (United States)

    ... make it bigger with a patch made of Gore-tex, a man-made (synthetic) material. Another way ... 434. Bhatt AB, Foster E, Kuehl K, et al; American Heart Association Council on Clinical Cardiology. Congenital ...

  2. Congenital Heart Defects (For Parents)

    Science.gov (United States)

    ... to be associated with genetic disorders, such as Down syndrome . But the cause of most congenital heart defects isn't known. While they can't be prevented, many treatments are available for the defects and related health ...

  3. Genetics Home Reference: congenital hyperinsulinism

    Science.gov (United States)

    ... Topic: Hypoglycemia Health Topic: Metabolic Disorders Genetic and Rare Diseases Information Center (1 link) Congenital hyperinsulinism Educational Resources (7 links) Boston Children's Hospital: Hypoglycemia and Low Blood Sugar in Children Cook Children's Hospital (PDF) Disease InfoSearch: ...

  4. CHRNE Mutation and Congenital Myasthenia

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2009-01-01

    Full Text Available The CHRNE e1293insG mutation was identified in 14 (60% of 23 North African families with an early onset form of congenital myasthenic syndrome studied at centers in France, Tunisia, Algeria, and UK.

  5. Auditory-visual integration in fields of the auditory cortex.

    Science.gov (United States)

    Kubota, Michinori; Sugimoto, Shunji; Hosokawa, Yutaka; Ojima, Hisayuki; Horikawa, Junsei

    2017-03-01

    While multimodal interactions have been known to exist in the early sensory cortices, the response properties and spatiotemporal organization of these interactions are poorly understood. To elucidate the characteristics of multimodal sensory interactions in the cerebral cortex, neuronal responses to visual stimuli with or without auditory stimuli were investigated in core and belt fields of guinea pig auditory cortex using real-time optical imaging with a voltage-sensitive dye. On average, visual responses consisted of short excitation followed by long inhibition. Although visual responses were observed in core and belt fields, there were regional and temporal differences in responses. The most salient visual responses were observed in the caudal belt fields, especially posterior (P) and dorsocaudal belt (DCB) fields. Visual responses emerged first in fields P and DCB and then spread rostroventrally to core and ventrocaudal belt (VCB) fields. Absolute values of positive and negative peak amplitudes of visual responses were both larger in fields P and DCB than in core and VCB fields. When combined visual and auditory stimuli were applied, fields P and DCB were more inhibited than core and VCB fields beginning approximately 110 ms after stimuli. Correspondingly, differences between responses to auditory stimuli alone and combined audiovisual stimuli became larger in fields P and DCB than in core and VCB fields after approximately 110 ms after stimuli. These data indicate that visual influences are most salient in fields P and DCB, which manifest mainly as inhibition, and that they enhance differences in auditory responses among fields. Copyright © 2017 Elsevier B.V. All rights reserved.

  6. Auditory and audio-visual processing in patients with cochlear, auditory brainstem, and auditory midbrain implants: An EEG study.

    Science.gov (United States)

    Schierholz, Irina; Finke, Mareike; Kral, Andrej; Büchner, Andreas; Rach, Stefan; Lenarz, Thomas; Dengler, Reinhard; Sandmann, Pascale

    2017-04-01

    There is substantial variability in speech recognition ability across patients with cochlear implants (CIs), auditory brainstem implants (ABIs), and auditory midbrain implants (AMIs). To better understand how this variability is related to central processing differences, the current electroencephalography (EEG) study compared hearing abilities and auditory-cortex activation in patients with electrical stimulation at different sites of the auditory pathway. Three different groups of patients with auditory implants (Hannover Medical School; ABI: n = 6, CI: n = 6; AMI: n = 2) performed a speeded response task and a speech recognition test with auditory, visual, and audio-visual stimuli. Behavioral performance and cortical processing of auditory and audio-visual stimuli were compared between groups. ABI and AMI patients showed prolonged response times on auditory and audio-visual stimuli compared with NH listeners and CI patients. This was confirmed by prolonged N1 latencies and reduced N1 amplitudes in ABI and AMI patients. However, patients with central auditory implants showed a remarkable gain in performance when visual and auditory input was combined, in both speech and non-speech conditions, which was reflected by a strong visual modulation of auditory-cortex activation in these individuals. In sum, the results suggest that the behavioral improvement for audio-visual conditions in central auditory implant patients is based on enhanced audio-visual interactions in the auditory cortex. Their findings may provide important implications for the optimization of electrical stimulation and rehabilitation strategies in patients with central auditory prostheses. Hum Brain Mapp 38:2206-2225, 2017. © 2017 Wiley Periodicals, Inc. © 2017 Wiley Periodicals, Inc.

  7. Delayed Auditory Feedback and Movement

    Science.gov (United States)

    Pfordresher, Peter Q.; Dalla Bella, Simone

    2011-01-01

    It is well known that timing of rhythm production is disrupted by delayed auditory feedback (DAF), and that disruption varies with delay length. We tested the hypothesis that disruption depends on the state of the movement trajectory at the onset of DAF. Participants tapped isochronous rhythms at a rate specified by a metronome while hearing DAF…

  8. Molecular approach of auditory neuropathy.

    Science.gov (United States)

    Silva, Magali Aparecida Orate Menezes da; Piatto, Vânia Belintani; Maniglia, Jose Victor

    2015-01-01

    Mutations in the otoferlin gene are responsible for auditory neuropathy. To investigate the prevalence of mutations in the mutations in the otoferlin gene in patients with and without auditory neuropathy. This original cross-sectional case study evaluated 16 index cases with auditory neuropathy, 13 patients with sensorineural hearing loss, and 20 normal-hearing subjects. DNA was extracted from peripheral blood leukocytes, and the mutations in the otoferlin gene sites were amplified by polymerase chain reaction/restriction fragment length polymorphism. The 16 index cases included nine (56%) females and seven (44%) males. The 13 deaf patients comprised seven (54%) males and six (46%) females. Among the 20 normal-hearing subjects, 13 (65%) were males and seven were (35%) females. Thirteen (81%) index cases had wild-type genotype (AA) and three (19%) had the heterozygous AG genotype for IVS8-2A-G (intron 8) mutation. The 5473C-G (exon 44) mutation was found in a heterozygous state (CG) in seven (44%) index cases and nine (56%) had the wild-type allele (CC). Of these mutants, two (25%) were compound heterozygotes for the mutations found in intron 8 and exon 44. All patients with sensorineural hearing loss and normal-hearing individuals did not have mutations (100%). There are differences at the molecular level in patients with and without auditory neuropathy. Copyright © 2015 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

  9. Dynamics of auditory working memory

    Directory of Open Access Journals (Sweden)

    Jochen eKaiser

    2015-05-01

    Full Text Available Working memory denotes the ability to retain stimuli in mind that are no longer physically present and to perform mental operations on them. Electro- and magnetoencephalography allow investigating the short-term maintenance of acoustic stimuli at a high temporal resolution. Studies investigating working memory for non-spatial and spatial auditory information have suggested differential roles of regions along the putative auditory ventral and dorsal streams, respectively, in the processing of the different sound properties. Analyses of event-related potentials have shown sustained, memory load-dependent deflections over the retention periods. The topography of these waves suggested an involvement of modality-specific sensory storage regions. Spectral analysis has yielded information about the temporal dynamics of auditory working memory processing of individual stimuli, showing activation peaks during the delay phase whose timing was related to task performance. Coherence at different frequencies was enhanced between frontal and sensory cortex. In summary, auditory working memory seems to rely on the dynamic interplay between frontal executive systems and sensory representation regions.

  10. Congenital blindness limits allocentric to egocentric switching ability.

    Science.gov (United States)

    Ruggiero, Gennaro; Ruotolo, Francesco; Iachini, Tina

    2018-03-01

    Many everyday spatial activities require the cooperation or switching between egocentric (subject-to-object) and allocentric (object-to-object) spatial representations. The literature on blind people has reported that the lack of vision (congenital blindness) may limit the capacity to represent allocentric spatial information. However, research has mainly focused on the selective involvement of egocentric or allocentric representations, not the switching between them. Here we investigated the effect of visual deprivation on the ability to switch between spatial frames of reference. To this aim, congenitally blind (long-term visual deprivation), blindfolded sighted (temporary visual deprivation) and sighted (full visual availability) participants were compared on the Ego-Allo switching task. This task assessed the capacity to verbally judge the relative distances between memorized stimuli in switching (from egocentric-to-allocentric: Ego-Allo; from allocentric-to-egocentric: Allo-Ego) and non-switching (only-egocentric: Ego-Ego; only-allocentric: Allo-Allo) conditions. Results showed a difficulty in congenitally blind participants when switching from allocentric to egocentric representations, not when the first anchor point was egocentric. In line with previous results, a deficit in processing allocentric representations in non-switching conditions also emerged. These findings suggest that the allocentric deficit in congenital blindness may determine a difficulty in simultaneously maintaining and combining different spatial representations. This deficit alters the capacity to switch between reference frames specifically when the first anchor point is external and not body-centered.

  11. Lexical References to Sensory Modalities in Verbal Descriptions of People and Objects by Congenitally Blind, Late Blind and Sighted Adults

    OpenAIRE

    Chauvey, Valérie; Hatwell, Yvette; Verine, Bertrand; Kaminski, Gwenael; Gentaz, Edouard

    2012-01-01

    Background: Some previous studies have revealed that while congenitally blind people have a tendency to refer to visual attributes (‘verbalism'), references to auditory and tactile attributes are scarcer. However, this statement may be challenged by current theories claiming that cognition is linked to the perceptions and actions from which it derives. Verbal productions by the blind could therefore differ from those of the sighted because of their specific perceptual experience. The relative...

  12. Congenital non-central nervous system malformations in cerebral palsy: a distinct subset?

    Science.gov (United States)

    Self, Lauren; Dagenais, Lynn; Shevell, Michael

    2012-08-01

    The aim of this article was to identify and contrast the subset of children with cerebral palsy (CP) and non-central nervous system (CNS) congenital malformations with children with CP but no coexisting non-CNS congenital malformations. A population-based regional comprehensive CP registry was used to identify children with CP who had non-CNS congenital malformations (n = 34; 19 males, 15 females; 22 classified as Gross Motor Function Classification System [GMFCS] levels I-III, 12 as GMFCS level IV or V). Their clinical features were then compared with other children with CP without non-CNS congenital malformations (n = 207; 115 males, 92 females; 138 classified as GMFCS levels I-III, 69 as GMFCS level IV or V). Children with CP and non-CNS congenital malformations did not differ from those without in terms of neurological subtype distribution or functional severity, as measured by the GMFCS. Also, there was no association with previous maternal infections (i.e. toxoplasmosis, rubella, cytomegalovirus, herpes simplex virus 2 [TORCH]), maternal fever, use of illicit substances, asphyxia, neonatal encephalopathy, intraventricular haemorrhage, or septicaemia. The incidence of comorbidities such as convulsions, communication difficulties, gavage feeding, cortical blindness, and auditory impairment was not higher in this subgroup. The incidence of congenital non-CNS malformations among children with CP is appreciable. Children with these non-CNS malformations do not appear to differ from other children with CP regarding neurological subtype, functional severity, and comorbidities, or maternal or obstetrical factors. Thus, the specific presence of a non-CNS congenital malformation does not appear to assist the practitioner in the management or understanding of a child's CP. © The Authors. Developmental Medicine & Child Neurology © 2012 Mac Keith Press.

  13. Congenital amusia: a disorder of fine-grained pitch discrimination.

    Science.gov (United States)

    Peretz, Isabelle; Ayotte, Julie; Zatorre, Robert J; Mehler, Jacques; Ahad, Pierre; Penhune, Virginia B; Jutras, Benoît

    2002-01-17

    We report the first documented case of congenital amusia. This disorder refers to a musical disability that cannot be explained by prior brain lesion, hearing loss, cognitive deficits, socioaffective disturbance, or lack of environmental stimulation. This musical impairment is diagnosed in a middle-aged woman, hereafter referred to as Monica, who lacks most basic musical abilities, including melodic discrimination and recognition, despite normal audiometry and above-average intellectual, memory, and language skills. The results of psychophysical tests show that Monica has severe difficulties with detecting pitch changes. The data suggest that music-processing difficulties may result from problems in fine-grained discrimination of pitch, much in the same way as many language-processing difficulties arise from deficiencies in auditory temporal resolution.

  14. Pre-operative computed tomography evaluation of congenital aural atresia in children; Bilan tomodensitometrique pre-operatoire des aplasies majeures de l`oreille chez l`enfant

    Energy Technology Data Exchange (ETDEWEB)

    Lumbroso, C.; Sebag, G.; Argyropoulou, M.; Manach, Y.; Lallemand, D. [Hopital Necker-Enfants-Malades, 75 - Paris (France)

    1995-04-01

    To describe computed tomography (CT) findings in congenital aural atresia (CAA) and to illustrate the impact of these findings in the preoperative evaluation. A narrow bony external auditory canal (EAC) was present in 24% of the cases. In one of these cases, the EAC contained a cholesteatoma and was consequently a clear indication of surgery. An hyperpneumatized mastoid (22%), a posterior position of the temporo mandibular joint (16%), and a bulging medial temporal fossa (12%) made the operation much more difficult. The tympanic cavity was small in 68% of the cases, normal in 28% and absent in 4% of the cases without any detectable ossicular chain. Ossicular chain anomalies were present in 91% of the cases. This consisted most frequently of a fusion of the malleus and the incus (76%) with or without fusion of the tympanic wall (33%). Lateral and anterior displacement of the descending portion of the facial nerve was present in 62%. Oval and round windows were normal in 86% of the cases. A soft tissue opacity (33%) in the tympanic cavity made it difficult to evaluate the stapes, the incudo stapedial articulation, and the facial nerve. Simultaneous abnormalities of inner ear were exceptional. (authors). 9 refs., 8 figs.

  15. Short-term plasticity in auditory cognition.

    Science.gov (United States)

    Jääskeläinen, Iiro P; Ahveninen, Jyrki; Belliveau, John W; Raij, Tommi; Sams, Mikko

    2007-12-01

    Converging lines of evidence suggest that auditory system short-term plasticity can enable several perceptual and cognitive functions that have been previously considered as relatively distinct phenomena. Here we review recent findings suggesting that auditory stimulation, auditory selective attention and cross-modal effects of visual stimulation each cause transient excitatory and (surround) inhibitory modulations in the auditory cortex. These modulations might adaptively tune hierarchically organized sound feature maps of the auditory cortex (e.g. tonotopy), thus filtering relevant sounds during rapidly changing environmental and task demands. This could support auditory sensory memory, pre-attentive detection of sound novelty, enhanced perception during selective attention, influence of visual processing on auditory perception and longer-term plastic changes associated with perceptual learning.

  16. Executive abilities in children with congenital visual impairment in mid-childhood.

    Science.gov (United States)

    Bathelt, Joe; de Haan, Michelle; Salt, Alison; Dale, Naomi Jane

    2018-02-01

    The role of vision and vision deprivation in the development of executive function (EF) abilities in childhood is little understood; aspects of EF such as initiative, attention orienting, inhibition, planning and performance monitoring are often measured through visual tasks. Studying the development and integrity of EF abilities in children with congenital visual impairment (VI) may provide important insights into the development of EF and also its possible relationship with vision and non-visual senses. The current study investigates non-visual EF abilities in 18 school-age children of average verbal intelligence with VI of differing levels of severity arising from congenital disorders affecting the eye, retina, or anterior optic nerve. Standard auditory neuropsychological assessments of sustained and divided attention, phonemic, semantic and switching verbal fluency, verbal working memory, and ratings of everyday executive abilities by parents were undertaken. Executive skills were compared to age-matched typically-sighted (TS) typically-developing children and across levels of vision (mild to moderate VI [MVI] or severe to profound VI [SPVI]). The results do not indicate significant differences or deficits on direct assessments of verbal and auditory EF between the groups. However, parent ratings suggest difficulties with everyday executive abilities, with the greatest difficulties in those with SPVI. The findings are discussed as possibly reflecting increased demands of behavioral executive skills for children with VI in everyday situations despite auditory and verbal EF abilities in the typical range for their age. These findings have potential implications for clinical and educational practices.

  17. Congenital amusia persists in the developing brain after daily music listening.

    Science.gov (United States)

    Mignault Goulet, Geneviève; Moreau, Patricia; Robitaille, Nicolas; Peretz, Isabelle

    2012-01-01

    Congenital amusia is a neurodevelopmental disorder that affects about 3% of the adult population. Adults experiencing this musical disorder in the absence of macroscopically visible brain injury are described as cases of congenital amusia under the assumption that the musical deficits have been present from birth. Here, we show that this disorder can be expressed in the developing brain. We found that (10-13 year-old) children exhibit a marked deficit in the detection of fine-grained pitch differences in both musical and acoustical context in comparison to their normally developing peers comparable in age and general intelligence. This behavioral deficit could be traced down to their abnormal P300 brain responses to the detection of subtle pitch changes. The altered pattern of electrical activity does not seem to arise from an anomalous functioning of the auditory cortex, because all early components of the brain potentials, the N100, the MMN, and the P200 appear normal. Rather, the brain and behavioral measures point to disrupted information propagation from the auditory cortex to other cortical regions. Furthermore, the behavioral and neural manifestations of the disorder remained unchanged after 4 weeks of daily musical listening. These results show that congenital amusia can be detected in childhood despite regular musical exposure and normal intellectual functioning.

  18. Congenital amusia persists in the developing brain after daily music listening.

    Directory of Open Access Journals (Sweden)

    Geneviève Mignault Goulet

    Full Text Available Congenital amusia is a neurodevelopmental disorder that affects about 3% of the adult population. Adults experiencing this musical disorder in the absence of macroscopically visible brain injury are described as cases of congenital amusia under the assumption that the musical deficits have been present from birth. Here, we show that this disorder can be expressed in the developing brain. We found that (10-13 year-old children exhibit a marked deficit in the detection of fine-grained pitch differences in both musical and acoustical context in comparison to their normally developing peers comparable in age and general intelligence. This behavioral deficit could be traced down to their abnormal P300 brain responses to the detection of subtle pitch changes. The altered pattern of electrical activity does not seem to arise from an anomalous functioning of the auditory cortex, because all early components of the brain potentials, the N100, the MMN, and the P200 appear normal. Rather, the brain and behavioral measures point to disrupted information propagation from the auditory cortex to other cortical regions. Furthermore, the behavioral and neural manifestations of the disorder remained unchanged after 4 weeks of daily musical listening. These results show that congenital amusia can be detected in childhood despite regular musical exposure and normal intellectual functioning.

  19. Comparative analysis of detection methods for congenital cytomegalovirus infection in a Guinea pig model.

    Science.gov (United States)

    Park, Albert H; Mann, David; Error, Marc E; Miller, Matthew; Firpo, Matthew A; Wang, Yong; Alder, Stephen C; Schleiss, Mark R

    2013-01-01

    To assess the validity of the guinea pig as a model for congenital cytomegalovirus (CMV) infection by comparing the effectiveness of detecting the virus by real-time polymerase chain reaction (PCR) in blood, urine, and saliva. Case-control study. Academic research. Eleven pregnant Hartley guinea pigs. Blood, urine, and saliva samples were collected from guinea pig pups delivered from pregnant dams inoculated with guinea pig CMV. These samples were then evaluated for the presence of guinea pig CMV by real-time PCR assuming 100% transmission. Thirty-one pups delivered from 9 inoculated pregnant dams and 8 uninfected control pups underwent testing for guinea pig CMV and for auditory brainstem response hearing loss. Repeated-measures analysis of variance demonstrated no statistically significantly lower weight for the infected pups compared with the noninfected control pups. Six infected pups demonstrated auditory brainstem response hearing loss. The sensitivity and specificity of the real-time PCR assay on saliva samples were 74.2% and 100.0%, respectively. The sensitivity of the real-time PCR on blood and urine samples was significantly lower than that on saliva samples. Real-time PCR assays of blood, urine, and saliva revealed that saliva samples show high sensitivity and specificity for detecting congenital CMV infection in guinea pigs. This finding is consistent with recent screening studies in human newborns. The guinea pig may be a good animal model in which to compare different diagnostic assays for congenital CMV infection.

  20. Congenital Short QT Syndrome

    Directory of Open Access Journals (Sweden)

    Charles Antzelevitch

    2004-04-01

    Full Text Available Long QT intervals in the ECG have long been associated with sudden cardiac death. The congenital long QT syndrome was first described in individuals with structurally normal hearts in 1957.1 Little was known about the significance of a short QT interval. In 1993, after analyzing 6693 consecutive Holter recordings Algra et al concluded that an increased risk of sudden death was present not only in patients with long QT interval, but also in patients with short QT interval (<400 ms.2 Because this was a retrospective analysis, further evaluation of the data was not possible. It was not until 2000 that a short-QT syndrome (SQTS was proposed as a new inherited clinical syndrome by Gussak et al.3 The initial report was of two siblings and their mother all of whom displayed persistently short QT interval. The youngest was a 17 year old female presenting with several episodes of paroxysmal atrial fibrillation requiring electrical cardioversion.3 Her QT interval measured 280 msec at a heart rate of 69. Her 21 year old brother displayed a QT interval of 272 msec at a heart rate of 58, whereas the 51 year old mother showed a QT of 260 msec at a heart rate of 74. The authors also noted similar ECG findings in another unrelated 37 year old patient associated with sudden cardiac death.

  1. Congenital hypothyroidism in neonates

    Directory of Open Access Journals (Sweden)

    Aneela Anjum

    2014-01-01

    Full Text Available Context: Congenital hypothyroidism (CH is one of the most common preventable causes of mental retardation in children and it occurs in approximately 1:2,000-1:4,000 newborns. Aims and Objectives: The aim of this study is to determine the frequency of CH in neonates. Settings and Design: This cross-sectional study was conducted in neonatal units of the Department of Pediatrics Unit-I, King Edward Medical University/Mayo Hospital, Lahore and Lady Willington Hospital Lahore in 6 months (January-June 2011. Materials and Methods: Sample was collected by non-probability purposive sampling. After consent, 550 newborn were registered for the study. Demographic data and relevant history was recorded. After aseptic measures, 2-3 ml venous blood analyzed for thyroid-stimulating hormone (TSH level by immunoradiometric assay. Treatment was started according to the individual merit as per protocol. Statistical Analysis Used: Data was analyzed by SPSS 17 and Chi-square test was applied to find out the association of CH with different variables. Results: The study population consisted of 550 newborns. Among 550 newborns, 4 (0.8% newborns had elevated TSH level. CH had statistically significant association with mother′s hypothyroidism (P value 0.000 and mother′s drug intake during the pregnancy period (P value 0.013. Conclusion: CH is 0.8% in neonates. It has statistically significant association with mother′s hypothyroidism and mother′s drug intake during pregnancy.

  2. Congenital coronary artery fistula

    International Nuclear Information System (INIS)

    Oh, Yeon Hee; Kim, Hong; Zeon, Seoc Kil; Suh, Soo Jhi

    1986-01-01

    Congenital coronary artery fistula (CCAF) is communication of a coronary artery or its main branch with one of the atria or ventricles, the coronary sinus, the superior vena cava, or the pulmonary artery. In Korean peoples, only 4 cases of the CCAF were reported as rare as worldwide and authors want to report another case of CCAF, confirmed by operation. 10-year-old girl shows a fistula between sinus node artery of the right coronary artery and right atrium on root aortogram with left-to-right shunt and Qp/Qs=1.58, in which simple ligation of the sinus node artery from right coronary artery was performed. All of the 5 Korean CCAF (4 were previously reported and 1 of authors) were originated from right coronary artery, and of which 4 were opening into right ventricle and 1 of authors were into right atrium. Associated cardiac anomaly was noted in only 1 case as single coronary artery. Ages were from 9 months of age to 10 years old and no adult left case were found. 3 were female and 2 were male patients.

  3. Congenital nephrotic syndrome.

    Science.gov (United States)

    Hamed, Radi Ma

    2003-01-01

    The congenital nephrotic syndrome (CNS) is an uncommon disorder with onset of the nephrotic syndrome usually in the first three months of life. Several different diseases may cause the syndrome. These may be inherited, sporadic, acquired or part of a general malformation syndrome. The clinical course is marked by failure to thrive, recurrent life threatening bacterial infections, and early death from sepsis and/or uremia. A characteristic phenotype may be seen in children with CNS. The majority of reported cases of CNS are of the Finnish type (CNF). Although the role of the glomerular basement membrane has been emphasized as the barrier for retaining plasma proteins, recent studies have clearly shown that the slit diaphragm is the structure most likely to be the barrier in the glomerular capillary wall. The gene (NPHS1) was shown to encode a novel protein that was termed nephrin, due to its specific location in the kidney filter barrier, where it seems to form a highly organized filter structure. Nephrin is a transmembrane protein that probably forms the main building block of an isoporous zipper-like slit diaphragm filter structure. Defects in nephrin lead to the abnormal or absent slit diaphragm resulting in massive proteinuria and renal failure.

  4. [Penile congenital abnormalities].

    Science.gov (United States)

    Boillot, B; Teklali, Y; Moog, R; Droupy, S

    2013-07-01

    Congenital abnormalities of the penis are usually diagnosed at birth and pose aesthetic and functional problems sometimes requiring surgical management. A literature review was conducted on Medline considering the articles listed until January 2012. Hypospadias is the most common malformation (1 in 250 boys. Familial forms: 7%). The causes remain hypothetical but the doubling of the incidence in 30 years could be linked to fetal exposure to endocrine disruptors "estrogen-like" used in the food industry in particular. Surgical treatment is usually intended to improve the aesthetic appearance but sometimes, in case of significant curvature or posterior meatus, necessary for normal sexual life and fertility. Other malformations (epispades, buried penis, transpositions, twists and preputial abnormalities) as well as management for functional or aesthetic consequences of these malformations in adulthood require complex surgical care in a specialized environment. The improvement of surgical techniques and pediatric anesthesia allows an early and effective specialized surgical approach of penile malformations. Management of sequelae in adulthood must be discussed and requires experience of surgical techniques on pediatric and adult penis. Copyright © 2013 Elsevier Masson SAS. All rights reserved.

  5. Congenital cystic lung malformations

    International Nuclear Information System (INIS)

    Stoever, B.; Scheer, I.; Bassir, C.; Chaoui, R.; Henrich, W.; Schwabe, M.; Wauer, R.

    2006-01-01

    Purpose: The aim of the study concerning congenital cystic lung malformations was to evaluate prenatal diagnoses postnatally to determine prognostic factors as well as to define optimized perinatal management. Materials and Methods: The study is based on 45 prenatal ultrasound examinations depicting fetal cystic lung lesions. 32 of the mothers had follow-up examinations. 5 pregnancies were terminated due to CCAM and additional malformations. Complete regression of the lesions was seen prenatally in 8 cases and postnatally in 5 children. Results: Surgical intervention due to respiratory insufficiency was necessary in 4 neonates. According to the imaging results, CCAM was present in 4 cases and sequestration in 7 patients. No correlation between the imaging findings and the surgical results was found in 3 children: One child suffered from rhadomyoid dysplasia, and in the case of the second child, a left-sided hernia of the diaphragm and additional sequestration were detected. The third child showed AV malformation. The cystic lesions of the 14 children operated upon were proven histologically. The degree of accuracy in the present study was high. Conclusion: Precise perinatal management is warranted in order to determine according to the clinical relevance surgical intervention and to prevent complications after the first year of life. This is performed during the neonatal period for respiratory insufficient neonates and within the first year of life for clinically stable children. (orig.)

  6. Congenital pulmonary lymphangiectasia

    Directory of Open Access Journals (Sweden)

    Campisi Corradino

    2006-10-01

    Full Text Available Abstract Congenital pulmonary lymphangiectasia (PL is a rare developmental disorder involving the lung, and characterized by pulmonary subpleural, interlobar, perivascular and peribronchial lymphatic dilatation. The prevalence is unknown. PL presents at birth with severe respiratory distress, tachypnea and cyanosis, with a very high mortality rate at or within a few hours of birth. Most reported cases are sporadic and the etiology is not completely understood. It has been suggested that PL lymphatic channels of the fetal lung do not undergo the normal regression process at 20 weeks of gestation. Secondary PL may be caused by a cardiac lesion. The diagnostic approach includes complete family and obstetric history, conventional radiologic studies, ultrasound and magnetic resonance studies, lymphoscintigraphy, lung functionality tests, lung biopsy, bronchoscopy, and pleural effusion examination. During the prenatal period, all causes leading to hydrops fetalis should be considered in the diagnosis of PL. Fetal ultrasound evaluation plays a key role in the antenatal diagnosis of PL. At birth, mechanical ventilation and pleural drainage are nearly always necessary to obtain a favorable outcome of respiratory distress. Home supplemental oxygen therapy and symptomatic treatment of recurrent cough and wheeze are often necessary during childhood, sometimes associated with prolonged pleural drainage. Recent advances in intensive neonatal care have changed the previously nearly fatal outcome of PL at birth. Patients affected by PL who survive infancy, present medical problems which are characteristic of chronic lung disease.

  7. Electrophysiological assessment of auditory processing disorder in children with non-syndromic cleft lip and/or palate.

    Science.gov (United States)

    Ma, Xiaoran; McPherson, Bradley; Ma, Lian

    2016-01-01

    Cleft lip and/or palate is a common congenital craniofacial malformation found worldwide. A frequently associated disorder is conductive hearing loss, and this disorder has been thoroughly investigated in children with non-syndromic cleft lip and/or palate (NSCL/P). However, analysis of auditory processing function is rarely reported for this population, although this issue should not be ignored since abnormal auditory cortical structures have been found in populations with cleft disorders. The present study utilized electrophysiological tests to assess the auditory status of a large group of children with NSCL/P, and investigated whether this group had less robust central auditory processing abilities compared to craniofacially normal children. 146 children with NSCL/P who had normal peripheral hearing thresholds, and 60 craniofacially normal children aged from 6 to 15 years, were recruited. Electrophysiological tests, including auditory brainstem response (ABR), P1-N1-P2 complex, and P300 component recording, were conducted. ABR and N1 wave latencies were significantly prolonged in children with NSCL/P. An atypical developmental trend was found for long latency potentials in children with cleft compared to control group children. Children with unilateral cleft lip and palate showed a greater level of abnormal results compared with other cleft subgroups, whereas the cleft lip subgroup had the most robust responses for all tests. Children with NSCL/P may have slower than normal neural transmission times between the peripheral auditory nerve and brainstem. Possible delayed development of myelination and synaptogenesis may also influence auditory processing function in this population. Present research outcomes were consistent with previous, smaller sample size, electrophysiological studies on infants and children with cleft lip/palate disorders. In view of the these findings, and reports of educational disadvantage associated with cleft disorders, further research

  8. Areas Recruited during Action Understanding Are Not Modulated by Auditory or Sign Language Experience.

    Science.gov (United States)

    Fang, Yuxing; Chen, Quanjing; Lingnau, Angelika; Han, Zaizhu; Bi, Yanchao

    2016-01-01

    The observation of other people's actions recruits a network of areas including the inferior frontal gyrus (IFG), the inferior parietal lobule (IPL), and posterior middle temporal gyrus (pMTG). These regions have been shown to be activated through both visual and auditory inputs. Intriguingly, previous studies found no engagement of IFG and IPL for deaf participants during non-linguistic action observation, leading to the proposal that auditory experience or sign language usage might shape the functionality of these areas. To understand which variables induce plastic changes in areas recruited during the processing of other people's actions, we examined the effects of tasks (action understanding and passive viewing) and effectors (arm actions vs. leg actions), as well as sign language experience in a group of 12 congenitally deaf signers and 13 hearing participants. In Experiment 1, we found a stronger activation during an action recognition task in comparison to a low-level visual control task in IFG, IPL and pMTG in both deaf signers and hearing individuals, but no effect of auditory or sign language experience. In Experiment 2, we replicated the results of the first experiment using a passive viewing task. Together, our results provide robust evidence demonstrating that the response obtained in IFG, IPL, and pMTG during action recognition and passive viewing is not affected by auditory or sign language experience, adding further support for the supra-modal nature of these regions.

  9. Developmental dysgraphia with profound hearing impairment: intervention by auditory methods enabled by cochlear implant.

    Science.gov (United States)

    Fukushima, Kunihiro; Kawasaki, Akihiro; Nagayasu, Rie; Kunisue, Kazuya; Maeda, Yukihide; Kariya, Shin; Kataoka, Yuko; Nishizaki, Kazunori

    2008-06-01

    Learning disability combined with hearing impairment (LDHI) is a poor prognostic factor for the language development of hearing impaired children after educational intervention. A typical example of a child with LDHI and effective interventions provided by cochlear implants are presented in this report. A case of congenital cytomegaloviral infection that showed dysgraphia as well as profound deafness was reported and an underlying visual processing problem diagnosed in the present case caused the patient's dysgraphia. The dysgraphia could be circumvented by the use of auditory memory fairly established by a cochlear implant.

  10. Biomimetic Sonar for Electrical Activation of the Auditory Pathway

    Directory of Open Access Journals (Sweden)

    D. Menniti

    2017-01-01

    Full Text Available Relying on the mechanism of bat’s echolocation system, a bioinspired electronic device has been developed to investigate the cortical activity of mammals in response to auditory sensorial stimuli. By means of implanted electrodes, acoustical information about the external environment generated by a biomimetic system and converted in electrical signals was delivered to anatomically selected structures of the auditory pathway. Electrocorticographic recordings showed that cerebral activity response is highly dependent on the information carried out by ultrasounds and is frequency-locked with the signal repetition rate. Frequency analysis reveals that delta and beta rhythm content increases, suggesting that sensorial information is successfully transferred and integrated. In addition, principal component analysis highlights how all the stimuli generate patterns of neural activity which can be clearly classified. The results show that brain response is modulated by echo signal features suggesting that spatial information sent by biomimetic sonar is efficiently interpreted and encoded by the auditory system. Consequently, these results give new perspective in artificial environmental perception, which could be used for developing new techniques useful in treating pathological conditions or influencing our perception of the surroundings.

  11. Rapid Auditory System Adaptation Using a Virtual Auditory Environment

    Directory of Open Access Journals (Sweden)

    Gaëtan Parseihian

    2011-10-01

    Full Text Available Various studies have highlighted plasticity of the auditory system from visual stimuli, limiting the trained field of perception. The aim of the present study is to investigate auditory system adaptation using an audio-kinesthetic platform. Participants were placed in a Virtual Auditory Environment allowing the association of the physical position of a virtual sound source with an alternate set of acoustic spectral cues or Head-Related Transfer Function (HRTF through the use of a tracked ball manipulated by the subject. This set-up has the advantage to be not being limited to the visual field while also offering a natural perception-action coupling through the constant awareness of one's hand position. Adaptation process to non-individualized HRTF was realized through a spatial search game application. A total of 25 subjects participated, consisting of subjects presented with modified cues using non-individualized HRTF and a control group using individual measured HRTFs to account for any learning effect due to the game itself. The training game lasted 12 minutes and was repeated over 3 consecutive days. Adaptation effects were measured with repeated localization tests. Results showed a significant performance improvement for vertical localization and a significant reduction in the front/back confusion rate after 3 sessions.

  12. Auditory Dysfunction in Patients with Cerebrovascular Disease

    Directory of Open Access Journals (Sweden)

    Sadaharu Tabuchi

    2014-01-01

    Full Text Available Auditory dysfunction is a common clinical symptom that can induce profound effects on the quality of life of those affected. Cerebrovascular disease (CVD is the most prevalent neurological disorder today, but it has generally been considered a rare cause of auditory dysfunction. However, a substantial proportion of patients with stroke might have auditory dysfunction that has been underestimated due to difficulties with evaluation. The present study reviews relationships between auditory dysfunction and types of CVD including cerebral infarction, intracerebral hemorrhage, subarachnoid hemorrhage, cerebrovascular malformation, moyamoya disease, and superficial siderosis. Recent advances in the etiology, anatomy, and strategies to diagnose and treat these conditions are described. The numbers of patients with CVD accompanied by auditory dysfunction will increase as the population ages. Cerebrovascular diseases often include the auditory system, resulting in various types of auditory dysfunctions, such as unilateral or bilateral deafness, cortical deafness, pure word deafness, auditory agnosia, and auditory hallucinations, some of which are subtle and can only be detected by precise psychoacoustic and electrophysiological testing. The contribution of CVD to auditory dysfunction needs to be understood because CVD can be fatal if overlooked.

  13. Adaptation in the auditory system: an overview

    Directory of Open Access Journals (Sweden)

    David ePérez-González

    2014-02-01

    Full Text Available The early stages of the auditory system need to preserve the timing information of sounds in order to extract the basic features of acoustic stimuli. At the same time, different processes of neuronal adaptation occur at several levels to further process the auditory information. For instance, auditory nerve fiber responses already experience adaptation of their firing rates, a type of response that can be found in many other auditory nuclei and may be useful for emphasizing the onset of the stimuli. However, it is at higher levels in the auditory hierarchy where more sophisticated types of neuronal processing take place. For example, stimulus-specific adaptation, where neurons show adaptation to frequent, repetitive stimuli, but maintain their responsiveness to stimuli with different physical characteristics, thus representing a distinct kind of processing that may play a role in change and deviance detection. In the auditory cortex, adaptation takes more elaborate forms, and contributes to the processing of complex sequences, auditory scene analysis and attention. Here we review the multiple types of adaptation that occur in the auditory system, which are part of the pool of resources that the neurons employ to process the auditory scene, and are critical to a proper understanding of the neuronal mechanisms that govern auditory perception.

  14. [Congenital cardiopathy and cerebral abscess].

    Science.gov (United States)

    Paixão, A; de Andrade, F F; Sampayo, F

    1989-01-01

    During 1986 the authors came across two cases of brain abscess among children with congenital heart disease followed at the Pediatric Cardiology Service and decided to evaluate their global experience on the subject. In a retrospective study of 860 infants and children with cyanotic congenital heart disease and final diagnosis, there were four cases complicated with brain abscess. The following items were evaluated: prevalence of the complication, type of congenital heart disease, date and age at the diagnosis of brain abscess, diagnostic methods, neurosurgical treatment and results. The main findings were: all patients were above two years of age and had noncorrected cyanotic congenital heart disease belonging to the classic high risk group; the first two cases had been treated in other institutions and only scanty information was available; two recent cases had early diagnosis on CAT scan followed by neurosurgical treatment. All children survived. brain abscess is a rare but severe complication occurring in patients with noncorrected cyanotic congenital heart disease above two years of age; whenever prevention turns impossible, early diagnosis and treatment provide good short term and long term results. A multidisciplinar approach with full cooperation is advocated.

  15. Dysrhythmia: a specific congenital rhythm perception deficit

    Directory of Open Access Journals (Sweden)

    Jacques eLaunay

    2014-02-01

    Full Text Available Why do some people have problems ‘feeling the beat’? Here we investigate participants with congenital impairments in musical rhythm perception and production. A web-based version of the Montreal Battery of Evaluation of Amusia (MBEA was used to screen for difficulties with rhythmic processing in a large sample and we identified three ‘dysrhythmic’ individuals who scored below cut-off for the rhythm subtest, but not the pitch-based subtests. Follow-up testing in the laboratory was conducted to characterize the nature of both rhythm perception and production deficits in these dysrhythmic individuals. We found that they differed from control participants when required to synchronize their tapping to an external stimulus with a metrical pulse, but not when required to tap spontaneously (with no external stimulus or to tap in time to an isochronous stimulus. Dysrhythmics exhibited a general tendency to tap at half the expected tempo when asked to synchronize to the beat of strongly metrical rhythms. These results suggest that the individuals studied here did not have motor production problems, but suffer from a selective rhythm perception deficit that influences the ability to entrain to metrical rhythms.

  16. Cyanotic congenital heart disease

    Energy Technology Data Exchange (ETDEWEB)

    Yeon, Kyung Mo; Yoo, Shi Joon; Han, Man Chung; Hong, Chang Yee; Lee, Yung Kyoon [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    1979-12-15

    Authors analyzed 265 cases of cyanotic congenital heart disease in which cardiac catheterization and angiocardiography were done at the Department of Radiology, Seoul National University Hospital between April 1973 and August 1979. The results are as follows; 1. Among 265 cases, 178 patients were male and 87 patients were female. 240 patients were below the age of 20 and none was over 35 year. 2. The incidence of individual lesions are as follows: tetralogy of Fallot-176; double outlet right ventricle-20; pentalogy-12; trilogy-11; corrected transposition of great arteries-10; complete transposition of great arteries-8; pulmonary atresia-7; single ventricle-6; Ebstein's anomaly-5; total anomalous pulmonary venous return-4; tricuspid atresia-3; double outlet left ventricle-1; truncus arteriosus-1; hypoplastic left ventricle-1. 3. Fallot's teralogy, pentalogy and trilogy were characteristic in their simple chest and angiocardiographic manifestations, but in a few cases of tetralogy and pentalogy it was difficult to differentiate them from double outlet right ventricle or pulmonary atresia. 4. In double outlet right ventricle and transposition of great arteries which are the pathologic spectrum resulting from abnormal conal growth, differential points were ventricular and great arterial loop patterns and their connections but it was very difficult to differentiate them from each other by single injection into one ventricle alone. 5. Ebstein's anomaly and total anomalous pulmonary venous return were so characteristic in angiocardiography was done ventriculography alone. 6. In 7 cases with double outlet right ventricle and transposition of great arteries, selective biventriculography was done and more accurate diagnosis could be made, which was quite difficult with one ventriculography alone. In 31 cases, cineangiocardiography was done and it gave more accurate information about the type and degree of pulmonary stenosis and overriding of aorta, the origin

  17. Cyanotic congenital heart disease

    International Nuclear Information System (INIS)

    Yeon, Kyung Mo; Yoo, Shi Joon; Han, Man Chung; Hong, Chang Yee; Lee, Yung Kyoon

    1979-01-01

    Authors analyzed 265 cases of cyanotic congenital heart disease in which cardiac catheterization and angiocardiography were done at the Department of Radiology, Seoul National University Hospital between April 1973 and August 1979. The results are as follows; 1. Among 265 cases, 178 patients were male and 87 patients were female. 240 patients were below the age of 20 and none was over 35 year. 2. The incidence of individual lesions are as follows: tetralogy of Fallot-176; double outlet right ventricle-20; pentalogy-12; trilogy-11; corrected transposition of great arteries-10; complete transposition of great arteries-8; pulmonary atresia-7; single ventricle-6; Ebstein's anomaly-5; total anomalous pulmonary venous return-4; tricuspid atresia-3; double outlet left ventricle-1; truncus arteriosus-1; hypoplastic left ventricle-1. 3. Fallot's teralogy, pentalogy and trilogy were characteristic in their simple chest and angiocardiographic manifestations, but in a few cases of tetralogy and pentalogy it was difficult to differentiate them from double outlet right ventricle or pulmonary atresia. 4. In double outlet right ventricle and transposition of great arteries which are the pathologic spectrum resulting from abnormal conal growth, differential points were ventricular and great arterial loop patterns and their connections but it was very difficult to differentiate them from each other by single injection into one ventricle alone. 5. Ebstein's anomaly and total anomalous pulmonary venous return were so characteristic in angiocardiography was done ventriculography alone. 6. In 7 cases with double outlet right ventricle and transposition of great arteries, selective biventriculography was done and more accurate diagnosis could be made, which was quite difficult with one ventriculography alone. In 31 cases, cineangiocardiography was done and it gave more accurate information about the type and degree of pulmonary stenosis and overriding of aorta, the origin of great

  18. Effect of delayed auditory feedback on stuttering with and without central auditory processing disorders.

    Science.gov (United States)

    Picoloto, Luana Altran; Cardoso, Ana Cláudia Vieira; Cerqueira, Amanda Venuti; Oliveira, Cristiane Moço Canhetti de

    2017-12-07

    To verify the effect of delayed auditory feedback on speech fluency of individuals who stutter with and without central auditory processing disorders. The participants were twenty individuals with stuttering from 7 to 17 years old and were divided into two groups: Stuttering Group with Auditory Processing Disorders (SGAPD): 10 individuals with central auditory processing disorders, and Stuttering Group (SG): 10 individuals without central auditory processing disorders. Procedures were: fluency assessment with non-altered auditory feedback (NAF) and delayed auditory feedback (DAF), assessment of the stuttering severity and central auditory processing (CAP). Phono Tools software was used to cause a delay of 100 milliseconds in the auditory feedback. The "Wilcoxon Signal Post" test was used in the intragroup analysis and "Mann-Whitney" test in the intergroup analysis. The DAF caused a statistically significant reduction in SG: in the frequency score of stuttering-like disfluencies in the analysis of the Stuttering Severity Instrument, in the amount of blocks and repetitions of monosyllabic words, and in the frequency of stuttering-like disfluencies of duration. Delayed auditory feedback did not cause statistically significant effects on SGAPD fluency, individuals with stuttering with auditory processing disorders. The effect of delayed auditory feedback in speech fluency of individuals who stutter was different in individuals of both groups, because there was an improvement in fluency only in individuals without auditory processing disorder.

  19. A new classification system for congenital laryngeal cysts.

    Science.gov (United States)

    Forte, Vito; Fuoco, Gabriel; James, Adrian

    2004-06-01

    A new classification system for congenital laryngeal cysts based on the extent of the cyst and on the embryologic tissue of origin is proposed. Retrospective chart review. The charts of 20 patients with either congenital or acquired laryngeal cysts that were treated surgically between 1987 and 2002 at the Hospital for Sick Children, Toronto were retrospectively reviewed. Clinical presentation, radiologic findings, surgical management, histopathology, and outcome were recorded. A new classification system is proposed to better appreciate the origin of these cysts and to guide in their successful surgical management. Fourteen of the supraglottic and subglottic simple mucous retention cysts posed no diagnostic or therapeutic challenge and were treated successfully by a single endoscopic excision or marsupialization. The remaining six patients with congenital cysts in the study were deemed more complex, and all required open surgical procedures for cure. On the basis of the analysis of the data of these patients, a new classification of congenital laryngeal cysts is proposed. Type I cysts are confined to the larynx, the cyst wall composed of endodermal elements only, and can be managed endoscopically. Type II cysts extend beyond the confines of the larynx and require an external approach. The Type II cysts are further subclassified histologically on the basis of the embryologic tissue of origin: IIa, composed of endoderm only and IIb, containing endodermal and mesodermal elements (epithelium and cartilage) in the wall of the cyst. A new classification system for congenital laryngeal cysts is proposed on the basis of the extent of the cyst and the embryologic tissue of origin. This classification can help guide the surgeon with initial management and help us better understand the origin of these cysts.

  20. Reality of auditory verbal hallucinations.

    Science.gov (United States)

    Raij, Tuukka T; Valkonen-Korhonen, Minna; Holi, Matti; Therman, Sebastian; Lehtonen, Johannes; Hari, Riitta

    2009-11-01

    Distortion of the sense of reality, actualized in delusions and hallucinations, is the key feature of psychosis but the underlying neuronal correlates remain largely unknown. We studied 11 highly functioning subjects with schizophrenia or schizoaffective disorder while they rated the reality of auditory verbal hallucinations (AVH) during functional magnetic resonance imaging (fMRI). The subjective reality of AVH correlated strongly and specifically with the hallucination-related activation strength of the inferior frontal gyri (IFG), including the Broca's language region. Furthermore, how real the hallucination that subjects experienced was depended on the hallucination-related coupling between the IFG, the ventral striatum, the auditory cortex, the right posterior temporal lobe, and the cingulate cortex. Our findings suggest that the subjective reality of AVH is related to motor mechanisms of speech comprehension, with contributions from sensory and salience-detection-related brain regions as well as circuitries related to self-monitoring and the experience of agency.

  1. Pitch-Responsive Cortical Regions in Congenital Amusia.

    Science.gov (United States)

    Norman-Haignere, Sam V; Albouy, Philippe; Caclin, Anne; McDermott, Josh H; Kanwisher, Nancy G; Tillmann, Barbara

    2016-03-09

    Congenital amusia is a lifelong deficit in music perception thought to reflect an underlying impairment in the perception and memory of pitch. The neural basis of amusic impairments is actively debated. Some prior studies have suggested that amusia stems from impaired connectivity between auditory and frontal cortex. However, it remains possible that impairments in pitch coding within auditory cortex also contribute to the disorder, in part because prior studies have not measured responses from the cortical regions most implicated in pitch perception in normal individuals. We addressed this question by measuring fMRI responses in 11 subjects with amusia and 11 age- and education-matched controls to a stimulus contrast that reliably identifies pitch-responsive regions in normal individuals: harmonic tones versus frequency-matched noise. Our findings demonstrate that amusic individuals with a substantial pitch perception deficit exhibit clusters of pitch-responsive voxels that are comparable in extent, selectivity, and anatomical location to those of control participants. We discuss possible explanations for why amusics might be impaired at perceiving pitch relations despite exhibiting normal fMRI responses to pitch in their auditory cortex: (1) individual neurons within the pitch-responsive region might exhibit abnormal tuning or temporal coding not detectable with fMRI, (2) anatomical tracts that link pitch-responsive regions to other brain areas (e.g., frontal cortex) might be altered, and (3) cortical regions outside of pitch-responsive cortex might be abnormal. The ability to identify pitch-responsive regions in individual amusic subjects will make it possible to ask more precise questions about their role in amusia in future work. Copyright © 2016 the authors 0270-6474/16/362986-09$15.00/0.

  2. Magnetic resonance imaging of congenital heart disease

    International Nuclear Information System (INIS)

    Fletcher, B.D.; Jacobstein, M.D.

    1988-01-01

    Focusing primarily on MR imaging of the heart, this book covers other diagnostic imaging modalities as well. The authors review new technologies and diagnostic procedures pertinent to congenital heat disease and present each congenital heat abnormality as a separate entity

  3. Genetic Counseling for Congenital Heart Defects

    Science.gov (United States)

    ... Artery Disease Venous Thromboembolism Aortic Aneurysm More Genetic Counseling for Congenital Heart Defects Updated:Jan 19,2018 ... with congenital heart disease considers having children. Genetic counseling can help answer these questions and address your ...

  4. CCT and sonographic findings in congenital craniopharyngioma

    Energy Technology Data Exchange (ETDEWEB)

    Helmke, K.; Hausdorf, G.; Moehrs, D.; Laas, R.

    1984-11-01

    In a case of congenital craniopharyngioma results of the sonographic and CCT examinations are reported and compared with postmortem macroscopic cuts. Comparison is made of clinical data from six cases with congenital craniopharyngioma as reported in the literature.

  5. CCT and sonographic findings in congenital craniopharyngioma

    International Nuclear Information System (INIS)

    Helmke, K.; Hausdorf, G.; Moehrs, D.; Laas, R.

    1984-01-01

    In a case of congenital craniopharyngioma results of the sonographic and CCT examinations are reported and compared with postmortem macroscopic cuts. Comparison is made of clinical data from six cases with congenital craniopharyngioma as reported in the literature. (orig.)

  6. Genetics Home Reference: severe congenital neutropenia

    Science.gov (United States)

    ... A characteristic of X-linked inheritance is that fathers cannot pass X-linked traits to their sons. ... Genetic Testing Registry: Severe congenital neutropenia 2, autosomal dominant Genetic Testing Registry: Severe congenital neutropenia 3, autosomal ...

  7. Genetics Home Reference: congenital dyserythropoietic anemia

    Science.gov (United States)

    ... Facebook Twitter Home Health Conditions CDA Congenital dyserythropoietic anemia Printable PDF Open All Close All Enable Javascript ... view the expand/collapse boxes. Description Congenital dyserythropoietic anemia ( CDA ) is an inherited blood disorder that affects ...

  8. Cerebral palsy and congenital malformations

    DEFF Research Database (Denmark)

    Garne, Ester; Dolk, Helen; Krägeloh-Mann, Inge

    2007-01-01

    AIM: To determine the proportion of children with cerebral palsy (CP) who have cerebral and non-cerebral congenital malformations. METHODS: Data from 11 CP registries contributing to the European Cerebral Palsy Database (SCPE), for children born in the period 1976-1996. The malformations were...... classified as recognized syndromes, chromosomal anomalies, cerebral malformations or non-cerebral malformations. Prevalence of malformations was compared to published data on livebirths from a European database of congenital malformations (EUROCAT). RESULTS: Overall 547 out of 4584 children (11.9%) with CP...... were reported to have a congenital malformation. The majority (8.6% of all children) were diagnosed with a cerebral malformation. The most frequent types of cerebral malformations were microcephaly and hydrocephaly. Non-cerebral malformations were present in 97 CP children and in further 14 CP children...

  9. Laterality of basic auditory perception.

    Science.gov (United States)

    Sininger, Yvonne S; Bhatara, Anjali

    2012-01-01

    Laterality (left-right ear differences) of auditory processing was assessed using basic auditory skills: (1) gap detection, (2) frequency discrimination, and (3) intensity discrimination. Stimuli included tones (500, 1000, and 4000 Hz) and wide-band noise presented monaurally to each ear of typical adult listeners. The hypothesis tested was that processing of tonal stimuli would be enhanced by left ear (LE) stimulation and noise by right ear (RE) presentations. To investigate the limits of laterality by (1) spectral width, a narrow-band noise (NBN) of 450-Hz bandwidth was evaluated using intensity discrimination, and (2) stimulus duration, 200, 500, and 1000 ms duration tones were evaluated using frequency discrimination. A left ear advantage (LEA) was demonstrated with tonal stimuli in all experiments, but an expected REA for noise stimuli was not found. The NBN stimulus demonstrated no LEA and was characterised as a noise. No change in laterality was found with changes in stimulus durations. The LEA for tonal stimuli is felt to be due to more direct connections between the left ear and the right auditory cortex, which has been shown to be primary for spectral analysis and tonal processing. The lack of a REA for noise stimuli is unexplained. Sex differences in laterality for noise stimuli were noted but were not statistically significant. This study did establish a subtle but clear pattern of LEA for processing of tonal stimuli.

  10. Auditory Motion Elicits a Visual Motion Aftereffect

    Directory of Open Access Journals (Sweden)

    Christopher C. Berger

    2016-12-01

    Full Text Available The visual motion aftereffect is a visual illusion in which exposure to continuous motion in one direction leads to a subsequent illusion of visual motion in the opposite direction. Previous findings have been mixed with regard to whether this visual illusion can be induced cross-modally by auditory stimuli. Based on research on multisensory perception demonstrating the profound influence auditory perception can have on the interpretation and perceived motion of visual stimuli, we hypothesized that exposure to auditory stimuli with strong directional motion cues should induce a visual motion aftereffect. Here, we demonstrate that horizontally moving auditory stimuli induced a significant visual motion aftereffect—an effect that was driven primarily by a change in visual motion perception following exposure to leftward moving auditory stimuli. This finding is consistent with the notion that visual and auditory motion perception rely on at least partially overlapping neural substrates.

  11. Auditory Motion Elicits a Visual Motion Aftereffect.

    Science.gov (United States)

    Berger, Christopher C; Ehrsson, H Henrik

    2016-01-01

    The visual motion aftereffect is a visual illusion in which exposure to continuous motion in one direction leads to a subsequent illusion of visual motion in the opposite direction. Previous findings have been mixed with regard to whether this visual illusion can be induced cross-modally by auditory stimuli. Based on research on multisensory perception demonstrating the profound influence auditory perception can have on the interpretation and perceived motion of visual stimuli, we hypothesized that exposure to auditory stimuli with strong directional motion cues should induce a visual motion aftereffect. Here, we demonstrate that horizontally moving auditory stimuli induced a significant visual motion aftereffect-an effect that was driven primarily by a change in visual motion perception following exposure to leftward moving auditory stimuli. This finding is consistent with the notion that visual and auditory motion perception rely on at least partially overlapping neural substrates.

  12. On diagnosis of congenital toxoplasmosis

    International Nuclear Information System (INIS)

    Denisova, L.B.; Vorontsova, S.V.; Shvedov, V.A.

    1999-01-01

    Chemical manifestations are considered and CT-semiotics of congenital toxoplasmosis, which can form intracranial calcinates, is described. Taking a certain case of congenital toxoplasmosis observation as an example the potentialities and significance of X-ray computerized tomography (CT) in the identification of brain pathological changes are demonstrated. It is shown that intracranial calcificates may be a sign of toxoplasmosis infection. In case of cytomegalovirus infection the calcificates on CT-scans have mostly periventricular localization. Equally with the revealing of brain status in case of chronic stage of neutrotoxoplasmosis the CT has also played a decisive role in diagnosis of pseudotumoroseus course of ischemic insult [ru

  13. MRI of congenital urethroperineal fistula

    Energy Technology Data Exchange (ETDEWEB)

    Ghadimi-Mahani, Maryam; Dillman, Jonathan R.; Pai, Deepa; DiPietro, Michael [C. S. Mott Children' s Hospital, Department of Radiology, Section of Pediatric Radiology, University of Michigan Health System, Ann Arbor, MI (United States); Park, John [C. S. Mott Children' s Hospital, Department of Pediatric Urology, University of Michigan Health System, Ann Arbor, MI (United States)

    2010-12-15

    We present the MRI features of a congenital urethroperineal fistula diagnosed in a 12-year-old boy being evaluated after a single urinary tract infection. This diagnosis was initially suggested by voiding cystourethrogram and confirmed by MRI. Imaging revealed an abnormal fluid-filled tract arising from the posterior urethra and tracking to the perineal skin surface that increased in size during micturition. Surgical resection and histopathological evaluation of the abnormal tract confirmed the diagnosis of congenital urethroperineal fistula. MRI played important roles in confirming the diagnosis and assisting surgical planning. (orig.)

  14. MRI of congenital urethroperineal fistula

    International Nuclear Information System (INIS)

    Ghadimi-Mahani, Maryam; Dillman, Jonathan R.; Pai, Deepa; DiPietro, Michael; Park, John

    2010-01-01

    We present the MRI features of a congenital urethroperineal fistula diagnosed in a 12-year-old boy being evaluated after a single urinary tract infection. This diagnosis was initially suggested by voiding cystourethrogram and confirmed by MRI. Imaging revealed an abnormal fluid-filled tract arising from the posterior urethra and tracking to the perineal skin surface that increased in size during micturition. Surgical resection and histopathological evaluation of the abnormal tract confirmed the diagnosis of congenital urethroperineal fistula. MRI played important roles in confirming the diagnosis and assisting surgical planning. (orig.)

  15. Congenital hypothyroidism: insights into pathogenesis and treatment

    OpenAIRE

    Cherella, Christine E.; Wassner, Ari J.

    2017-01-01

    Congenital hypothyroidism occurs in approximately 1 in 2000 newborns and can have devastating neurodevelopmental consequences if not detected and treated promptly. While newborn screening has virtually eradicated intellectual disability due to severe congenital hypothyroidism in the developed world, more stringent screening strategies have resulted in increased detection of mild congenital hypothyroidism. Recent studies provide conflicting evidence about the potential neurodevelopmental risks...

  16. The prevalence of congenital anomalies in Europe

    DEFF Research Database (Denmark)

    Dolk, Helen; Loane, Maria; Garne, Ester

    2010-01-01

    EUROCAT (European Surveillance of Congenital Anomalies) is the network of population-based registers of congenital anomaly in Europe, with a common protocol and data quality review, covering 1.5 million annual births in 22 countries. EUROCAT recorded a total prevalence of major congenital anomali...

  17. The Effect of Working Memory Training on Auditory Stream Segregation in Auditory Processing Disorders Children

    OpenAIRE

    Abdollah Moossavi; Saeideh Mehrkian; Yones Lotfi; Soghrat Faghih zadeh; Hamed Adjedi

    2015-01-01

    Objectives: This study investigated the efficacy of working memory training for improving working memory capacity and related auditory stream segregation in auditory processing disorders children. Methods: Fifteen subjects (9-11 years), clinically diagnosed with auditory processing disorder participated in this non-randomized case-controlled trial. Working memory abilities and auditory stream segregation were evaluated prior to beginning and six weeks after completing the training program...

  18. Functional mapping of the primate auditory system.

    Science.gov (United States)

    Poremba, Amy; Saunders, Richard C; Crane, Alison M; Cook, Michelle; Sokoloff, Louis; Mishkin, Mortimer

    2003-01-24

    Cerebral auditory areas were delineated in the awake, passively listening, rhesus monkey by comparing the rates of glucose utilization in an intact hemisphere and in an acoustically isolated contralateral hemisphere of the same animal. The auditory system defined in this way occupied large portions of cerebral tissue, an extent probably second only to that of the visual system. Cortically, the activated areas included the entire superior temporal gyrus and large portions of the parietal, prefrontal, and limbic lobes. Several auditory areas overlapped with previously identified visual areas, suggesting that the auditory system, like the visual system, contains separate pathways for processing stimulus quality, location, and motion.

  19. Auditory Modeling for Noisy Speech Recognition

    National Research Council Canada - National Science Library

    2000-01-01

    ... digital filtering for noise cancellation which interfaces to speech recognition software. It uses auditory features in speech recognition training, and provides applications to multilingual spoken language translation...

  20. Auditory prediction during speaking and listening.

    Science.gov (United States)

    Sato, Marc; Shiller, Douglas M

    2018-02-02

    In the present EEG study, the role of auditory prediction in speech was explored through the comparison of auditory cortical responses during active speaking and passive listening to the same acoustic speech signals. Two manipulations of sensory prediction accuracy were used during the speaking task: (1) a real-time change in vowel F1 feedback (reducing prediction accuracy relative to unaltered feedback) and (2) presenting a stable auditory target rather than a visual cue to speak (enhancing auditory prediction accuracy during baseline productions, and potentially enhancing the perturbing effect of altered feedback). While subjects compensated for the F1 manipulation, no difference between the auditory-cue and visual-cue conditions were found. Under visually-cued conditions, reduced N1/P2 amplitude was observed during speaking vs. listening, reflecting a motor-to-sensory prediction. In addition, a significant correlation was observed between the magnitude of behavioral compensatory F1 response and the magnitude of this speaking induced suppression (SIS) for P2 during the altered auditory feedback phase, where a stronger compensatory decrease in F1 was associated with a stronger the SIS effect. Finally, under the auditory-cued condition, an auditory repetition-suppression effect was observed in N1/P2 amplitude during the listening task but not active speaking, suggesting that auditory predictive processes during speaking and passive listening are functionally distinct. Copyright © 2018 Elsevier Inc. All rights reserved.

  1. Human Factors Military Lexicon: Auditory Displays

    National Research Council Canada - National Science Library

    Letowski, Tomasz

    2001-01-01

    .... In addition to definitions specific to auditory displays, speech communication, and audio technology, the lexicon includes several terms unique to military operational environments and human factors...

  2. Auditory, visual and auditory-visual memory and sequencing performance in typically developing children.

    Science.gov (United States)

    Pillai, Roshni; Yathiraj, Asha

    2017-09-01

    The study evaluated whether there exists a difference/relation in the way four different memory skills (memory score, sequencing score, memory span, & sequencing span) are processed through the auditory modality, visual modality and combined modalities. Four memory skills were evaluated on 30 typically developing children aged 7 years and 8 years across three modality conditions (auditory, visual, & auditory-visual). Analogous auditory and visual stimuli were presented to evaluate the three modality conditions across the two age groups. The children obtained significantly higher memory scores through the auditory modality compared to the visual modality. Likewise, their memory scores were significantly higher through the auditory-visual modality condition than through the visual modality. However, no effect of modality was observed on the sequencing scores as well as for the memory and the sequencing span. A good agreement was seen between the different modality conditions that were studied (auditory, visual, & auditory-visual) for the different memory skills measures (memory scores, sequencing scores, memory span, & sequencing span). A relatively lower agreement was noted only between the auditory and visual modalities as well as between the visual and auditory-visual modality conditions for the memory scores, measured using Bland-Altman plots. The study highlights the efficacy of using analogous stimuli to assess the auditory, visual as well as combined modalities. The study supports the view that the performance of children on different memory skills was better through the auditory modality compared to the visual modality. Copyright © 2017 Elsevier B.V. All rights reserved.

  3. Stuttering adults' lack of pre-speech auditory modulation normalizes when speaking with delayed auditory feedback.

    Science.gov (United States)

    Daliri, Ayoub; Max, Ludo

    2018-02-01

    Auditory modulation during speech movement planning is limited in adults who stutter (AWS), but the functional relevance of the phenomenon itself remains unknown. We investigated for AWS and adults who do not stutter (AWNS) (a) a potential relationship between pre-speech auditory modulation and auditory feedback contributions to speech motor learning and (b) the effect on pre-speech auditory modulation of real-time versus delayed auditory feedback. Experiment I used a sensorimotor adaptation paradigm to estimate auditory-motor speech learning. Using acoustic speech recordings, we quantified subjects' formant frequency adjustments across trials when continually exposed to formant-shifted auditory feedback. In Experiment II, we used electroencephalography to determine the same subjects' extent of pre-speech auditory modulation (reductions in auditory evoked potential N1 amplitude) when probe tones were delivered prior to speaking versus not speaking. To manipulate subjects' ability to monitor real-time feedback, we included speaking conditions with non-altered auditory feedback (NAF) and delayed auditory feedback (DAF). Experiment I showed that auditory-motor learning was limited for AWS versus AWNS, and the extent of learning was negatively correlated with stuttering frequency. Experiment II yielded several key findings: (a) our prior finding of limited pre-speech auditory modulation in AWS was replicated; (b) DAF caused a decrease in auditory modulation for most AWNS but an increase for most AWS; and (c) for AWS, the amount of auditory modulation when speaking with DAF was positively correlated with stuttering frequency. Lastly, AWNS showed no correlation between pre-speech auditory modulation (Experiment II) and extent of auditory-motor learning (Experiment I) whereas AWS showed a negative correlation between these measures. Thus, findings suggest that AWS show deficits in both pre-speech auditory modulation and auditory-motor learning; however, limited pre

  4. Radionuclide evaluation in malignant external otitis

    International Nuclear Information System (INIS)

    Wolosker, Sara; Penha Costa Marques, Marise da; Fonseca, Lea Miriam Barbosa da; Hospital Universitario Clementino Fraga Filho, Rio de Janeiro, RJ

    1996-01-01

    Malignant external otitis (MEO) is a severe infection of the external auditory canal that can result in osteomyelitis of the skull base. Twenty-eight patients with MEO (19 diabetics, 8 with AIDS and 1 with acute mielogenous leukemia) were evaluated using 99m Tc-MDP bone scintigraphy. 67 Ga citrate and computed tomography (CT) at the time of diagnosis for establishing the MEO extent. 67 Ga scans were obtained periodically in order to assess the evolution of the treatment. 99m Tc scans had shown high sensitivity and specificity in assessing the bone disease. Serial 67 Ga scans had shown to be valuable indicators of infection remission. CT scans provided poor diagnosis of early bone involvement. (author). 26 refs., 4 figs

  5. Tinnitus. I: Auditory mechanisms: a model for tinnitus and hearing impairment.

    Science.gov (United States)

    Hazell, J W; Jastreboff, P J

    1990-02-01

    A model is proposed for tinnitus and sensorineural hearing loss involving cochlear pathology. As tinnitus is defined as a cortical perception of sound in the absence of an appropriate external stimulus it must result from a generator in the auditory system which undergoes extensive auditory processing before it is perceived. The concept of spatial nonlinearity in the cochlea is presented as a cause of tinnitus generation controlled by the efferents. Various clinical presentations of tinnitus and the way in which they respond to changes in the environment are discussed with respect to this control mechanism. The concept of auditory retraining as part of the habituation process, and interaction with the prefrontal cortex and limbic system is presented as a central model which emphasizes the importance of the emotional significance and meaning of tinnitus.

  6. Congenital Aortic Stenosis and Aneurysms

    NARCIS (Netherlands)

    D. van der Linde (Denise)

    2013-01-01

    textabstractDue to improvements in pediatric cardio-thoracic surgery, anesthesia and diagnostics over the past decades, the number of adult patients with congenital heart disease (CHD) is growing. This causes an increasing demand in clinical practice for insight in long term outcome in both

  7. Severe congenital neutropenia (Kostmann Syndrome)

    African Journals Online (AJOL)

    Severe congenital neutropenia (SCN), Kostmann syndrome is a heterogenous disorder of myelopoiesis characterized by severe chronic ... erogenous hematological disorders, characterized by extremely low circu- lating neutrophils and ..... tic activation of STAT5 and stimulate. G-CSF-induced cell proliferation.26, 27.

  8. Angiocardiography in congenital heart malformations

    International Nuclear Information System (INIS)

    Soto, B.; Pacifico, A.D.

    1990-01-01

    The contents of this book are well organized, it deals with the technique of angiocardiography, and describes the angiocardiographic anatomy of the normal heart and offers an excellent sequential analysis of cardiac morphology. The rest of the book describes the angiocardiographic findings observed in individual congenital cardiac disorders

  9. Congenital Pulmonary Malformation in Children

    Directory of Open Access Journals (Sweden)

    Montasser Nadeem

    2012-01-01

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

  10. [Genetics of congenital heart diseases].

    Science.gov (United States)

    Bonnet, Damien

    2017-06-01

    Developmental genetics of congenital heart diseases has evolved from analysis of serial slices in embryos towards molecular genetics of cardiac morphogenesis with a dynamic view of cardiac development. Genetics of congenital heart diseases has also changed from formal genetic analysis of familial recurrences or population-based analysis to screening for mutations in candidates genes identified in animal models. Close cooperation between molecular embryologists, pathologists involved in heart development and pediatric cardiologists is crucial for further increase of knowledge in the field of cardiac morphogenesis and genetics of cardiac defects. The genetic model for congenital heart disease has to be revised to favor a polygenic origin rather than a monogenic one. The main mechanism is altered genic dosage that can account for heart diseases in chromosomal anomalies as well as in point mutations in syndromic and isolated congenital heart diseases. The use of big data grouping information from cardiac development, interactions between genes and proteins, epigenetic factors such as chromatin remodeling or DNA methylation is the current source for improving our knowledge in the field and to give clues for future therapies. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

  11. Genetics of Primary Congenital Hypothyroidism

    NARCIS (Netherlands)

    Zwaveling-Soonawala, Nitash; van Trotsenburg, Paul

    2018-01-01

    Congenital hypothyroidism (CH) is one of the most common preventable forms of mental retardation and since the implementation of neonatal screening programs in the mid-1970s, early detection and treatment have proven to be very successful in preventing brain damage. CH may be of thyroidal (=

  12. Congenital Pulmonary Malformation in Children

    OpenAIRE

    Nadeem, Montasser; Elnazir, Basil; Greally, Peter

    2012-01-01

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

  13. Neural Correlates of Congenital Amusia

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2006-10-01

    Full Text Available Magnetic resonance T1-weighted images were analyzed, using voxel-based morphometry (VBM, to detect brain anatomical differences in two independent groups of adults with congenital amusia (or tone-deafness compared to controls with normal pitch perception and no formal musical training.

  14. Congenital bronchobiliary fistula: MRI appearance

    International Nuclear Information System (INIS)

    Hourigan, Jon S.; Carr, Michael G.; Burton, Edward M.; Ledbetter, Joel C.

    2004-01-01

    Congenital bronchobiliary fistula (CBBF) is a rare anomaly. Twenty-three cases have been reported since the anomaly was first described in 1952. Most of these cases were diagnosed by bronchoscopy, cholangiography, or hepatobiliary nuclear imaging. Our case of a newborn with bilious emesis with CBBF was depicted by T1-weighted gradient-echo MRI sequences. (orig.)

  15. What's New in Congenital Scoliosis?

    Science.gov (United States)

    Pahys, Joshua M; Guille, James T

    2018-03-01

    Congenital scoliosis is a failure of vertebral formation, segmentation, or a combination of the 2 arising from abnormal vertebral development during weeks 4 to 6 of gestation. The associated spinal deformity can be of varying severity and result in a stable or progressive deformity based on the type and location of the anomalous vertebra(e). Bracing for congenital scoliosis is rarely indicated, while recent reports have demonstrated the utility of serial derotational casting for longer curves with multiple anomalous vertebrae as an effective "time buying strategy" to delay the need for surgery. Earlier hemivertebra excision and short-segment posterior spinal fusion have been advocated to prevent future curve progression of the deformity and/or the development of large compensatory curves. It has been shown in recent long-term follow-up studies that growth rates of the vertebral body and spinal canal are not as dramatically affected by pedicle screw instrumentation at a young age as once thought. Growth friendly surgery with either spine-based or rib-based anchors has demonstrated good results with curve correction while maintaining spinal growth. Rib-based anchors are typically more commonly indicated in the setting of chest wall abnormalities and/or when spinal anatomy precludes placement of spinal instrumentation. Recently, magnetically controlled growing rods have shown promising results in several studies that include a small subset of congenital scoliosis cases. A literature search was performed to identify existing studies related to the treatment of congenital scoliosis published from January 1, 2005 to June 1, 2016. Databases included PubMed, Medline, and the Cochrane Library. The search was limited to English articles and yielded 36 papers. This project was initiated by the Pediatric Orthopaedic Society of North America Publications Committee and was reviewed and approved by the Pediatric Orthopaedic Society of North America Presidential Line. A total of

  16. Congenital contractural arachnodactyly with neurogenic muscular atrophy: case report

    Directory of Open Access Journals (Sweden)

    Scola Rosana Herminia

    2001-01-01

    Full Text Available We report the case of a 3-1/2-year-old girl with hypotonia, multiple joint contractures, hip luxation, arachnodactyly, adducted thumbs, dolichostenomelia, and abnormal external ears suggesting the diagnosis of congenital contractural arachnodactyly (CCA. The serum muscle enzimes were normal and the needle electromyography showed active and chronic denervation. The muscle biopsy demonstrated active and chronic denervation compatible with spinal muscular atrophy. Analysis of exons 7 and 8 of survival motor neuron gene through polymerase chain reaction did not show deletions. Neurogenic muscular atrophy is a new abnormality associated with CCA, suggesting that CCA is clinically heterogeneous.

  17. Auditory Association Cortex Lesions Impair Auditory Short-Term Memory in Monkeys

    Science.gov (United States)

    Colombo, Michael; D'Amato, Michael R.; Rodman, Hillary R.; Gross, Charles G.

    1990-01-01

    Monkeys that were trained to perform auditory and visual short-term memory tasks (delayed matching-to-sample) received lesions of the auditory association cortex in the superior temporal gyrus. Although visual memory was completely unaffected by the lesions, auditory memory was severely impaired. Despite this impairment, all monkeys could discriminate sounds closer in frequency than those used in the auditory memory task. This result suggests that the superior temporal cortex plays a role in auditory processing and retention similar to the role the inferior temporal cortex plays in visual processing and retention.

  18. Congenital anomalies in Primorsky region.

    Science.gov (United States)

    Kiku, P; Voronin, S; Golokhvast, K

    2015-01-01

    According to WHO hereditary diseases and congenital malformations contribute significantly to the health of population. Thus, the problems of epidemiology, clinical presentation, diagnosis and treatment of congenital abnormalities are of interest for many researchers [2]. In addition, the dynamic accounting for the incidence of congenital malformations and hereditary diseases allows the researchers to assess the ecological situation in the region [1]. The occurrence of congenital anomalies in the world varies; it depends heavily on how carefully the data is collected [4]. Multifactorial or polygenic diseases develop under the influence of environmental factors in the presence of defective genes. They can constitute up to 90% of all chronic pathology [2-5]. To determine the incidence of congenital anomalies under the influence of environmental factors. The study used the methodology of system evaluation of congenital anomalies incidence in Primorsky region, depending on bio-climatic and environmental conditions. The authors used health statistics for the period from 2000 to 2014, F.12 class for congenital abnormalities in adolescents and children that were compared in geographical and temporal aspects with environmental factors of 33 settlements in Primorsky region. The environment is represented by nature and climate (6 factor modules) and sanitation (7 factor modules) blocks of factors. When formalizing the information database of the environment a specially developed 10-point assessment scale was used. Statistical processing of the information was carried out using Pearson's chi-squared test and multiple regression method from SSPS application program package. The study found that over the 15-year period the level of congenital abnormalities in children increased by 27.5% and in adolescents - by 35.1%, and in 2014 it amounted to 1687.6 and 839.3 per 100 000 people, respectively. The predictive model shows a steady further growth of this pathology. The incidence

  19. Early auditory change detection implicitly facilitated by ignored concurrent visual change during a Braille reading task.

    Science.gov (United States)

    Aoyama, Atsushi; Haruyama, Tomohiro; Kuriki, Shinya

    2013-09-01

    Unconscious monitoring of multimodal stimulus changes enables humans to effectively sense the external environment. Such automatic change detection is thought to be reflected in auditory and visual mismatch negativity (MMN) and mismatch negativity fields (MMFs). These are event-related potentials and magnetic fields, respectively, evoked by deviant stimuli within a sequence of standard stimuli, and both are typically studied during irrelevant visual tasks that cause the stimuli to be ignored. Due to the sensitivity of MMN/MMF to potential effects of explicit attention to vision, however, it is unclear whether multisensory co-occurring changes can purely facilitate early sensory change detection reciprocally across modalities. We adopted a tactile task involving the reading of Braille patterns as a neutral ignore condition, while measuring magnetoencephalographic responses to concurrent audiovisual stimuli that were infrequently deviated either in auditory, visual, or audiovisual dimensions; 1000-Hz standard tones were switched to 1050-Hz deviant tones and/or two-by-two standard check patterns displayed on both sides of visual fields were switched to deviant reversed patterns. The check patterns were set to be faint enough so that the reversals could be easily ignored even during Braille reading. While visual MMFs were virtually undetectable even for visual and audiovisual deviants, significant auditory MMFs were observed for auditory and audiovisual deviants, originating from bilateral supratemporal auditory areas. Notably, auditory MMFs were significantly enhanced for audiovisual deviants from about 100 ms post-stimulus, as compared with the summation responses for auditory and visual deviants or for each of the unisensory deviants recorded in separate sessions. Evidenced by high tactile task performance with unawareness of visual changes, we conclude that Braille reading can successfully suppress explicit attention and that simultaneous multisensory changes can

  20. Narrow, duplicated internal auditory canal

    Energy Technology Data Exchange (ETDEWEB)

    Ferreira, T. [Servico de Neurorradiologia, Hospital Garcia de Orta, Avenida Torrado da Silva, 2801-951, Almada (Portugal); Shayestehfar, B. [Department of Radiology, UCLA Oliveview School of Medicine, Los Angeles, California (United States); Lufkin, R. [Department of Radiology, UCLA School of Medicine, Los Angeles, California (United States)

    2003-05-01

    A narrow internal auditory canal (IAC) constitutes a relative contraindication to cochlear implantation because it is associated with aplasia or hypoplasia of the vestibulocochlear nerve or its cochlear branch. We report an unusual case of a narrow, duplicated IAC, divided by a bony septum into a superior relatively large portion and an inferior stenotic portion, in which we could identify only the facial nerve. This case adds support to the association between a narrow IAC and aplasia or hypoplasia of the vestibulocochlear nerve. The normal facial nerve argues against the hypothesis that the narrow IAC is the result of a primary bony defect which inhibits the growth of the vestibulocochlear nerve. (orig.)

  1. Effectiveness of auditory and tactile crossmodal cues in a dual-task visual and auditory scenario.

    Science.gov (United States)

    Hopkins, Kevin; Kass, Steven J; Blalock, Lisa Durrance; Brill, J Christopher

    2017-05-01

    In this study, we examined how spatially informative auditory and tactile cues affected participants' performance on a visual search task while they simultaneously performed a secondary auditory task. Visual search task performance was assessed via reaction time and accuracy. Tactile and auditory cues provided the approximate location of the visual target within the search display. The inclusion of tactile and auditory cues improved performance in comparison to the no-cue baseline conditions. In comparison to the no-cue conditions, both tactile and auditory cues resulted in faster response times in the visual search only (single task) and visual-auditory (dual-task) conditions. However, the effectiveness of auditory and tactile cueing for visual task accuracy was shown to be dependent on task-type condition. Crossmodal cueing remains a viable strategy for improving task performance without increasing attentional load within a singular sensory modality. Practitioner Summary: Crossmodal cueing with dual-task performance has not been widely explored, yet has practical applications. We examined the effects of auditory and tactile crossmodal cues on visual search performance, with and without a secondary auditory task. Tactile cues aided visual search accuracy when also engaged in a secondary auditory task, whereas auditory cues did not.

  2. Neural dynamics underlying attentional orienting to auditory representations in short-term memory.

    Science.gov (United States)

    Backer, Kristina C; Binns, Malcolm A; Alain, Claude

    2015-01-21

    Sounds are ephemeral. Thus, coherent auditory perception depends on "hearing" back in time: retrospectively attending that which was lost externally but preserved in short-term memory (STM). Current theories of auditory attention assume that sound features are integrated into a perceptual object, that multiple objects can coexist in STM, and that attention can be deployed to an object in STM. Recording electroencephalography from humans, we tested these assumptions, elucidating feature-general and feature-specific neural correlates of auditory attention to STM. Alpha/beta oscillations and frontal and posterior event-related potentials indexed feature-general top-down attentional control to one of several coexisting auditory representations in STM. Particularly, task performance during attentional orienting was correlated with alpha/low-beta desynchronization (i.e., power suppression). However, attention to one feature could occur without simultaneous processing of the second feature of the representation. Therefore, auditory attention to memory relies on both feature-specific and feature-general neural dynamics. Copyright © 2015 the authors 0270-6474/15/351307-12$15.00/0.

  3. Relative contributions of visual and auditory spatial representations to tactile localization.

    Science.gov (United States)

    Noel, Jean-Paul; Wallace, Mark

    2016-02-01

    Spatial localization of touch is critically dependent upon coordinate transformation between different reference frames, which must ultimately allow for alignment between somatotopic and external representations of space. Although prior work has shown an important role for cues such as body posture in influencing the spatial localization of touch, the relative contributions of the different sensory systems to this process are unknown. In the current study, we had participants perform a tactile temporal order judgment (TOJ) under different body postures and conditions of sensory deprivation. Specifically, participants performed non-speeded judgments about the order of two tactile stimuli presented in rapid succession on their ankles during conditions in which their legs were either uncrossed or crossed (and thus bringing somatotopic and external reference frames into conflict). These judgments were made in the absence of 1) visual, 2) auditory, or 3) combined audio-visual spatial information by blindfolding and/or placing participants in an anechoic chamber. As expected, results revealed that tactile temporal acuity was poorer under crossed than uncrossed leg postures. Intriguingly, results also revealed that auditory and audio-visual deprivation exacerbated the difference in tactile temporal acuity between uncrossed to crossed leg postures, an effect not seen for visual-only deprivation. Furthermore, the effects under combined audio-visual deprivation were greater than those seen for auditory deprivation. Collectively, these results indicate that mechanisms governing the alignment between somatotopic and external reference frames extend beyond those imposed by body posture to include spatial features conveyed by the auditory and visual modalities - with a heavier weighting of auditory than visual spatial information. Thus, sensory modalities conveying exteroceptive spatial information contribute to judgments regarding the localization of touch. Copyright © 2016

  4. Further Evidence of Auditory Extinction in Aphasia

    Science.gov (United States)

    Marshall, Rebecca Shisler; Basilakos, Alexandra; Love-Myers, Kim

    2013-01-01

    Purpose: Preliminary research ( Shisler, 2005) suggests that auditory extinction in individuals with aphasia (IWA) may be connected to binding and attention. In this study, the authors expanded on previous findings on auditory extinction to determine the source of extinction deficits in IWA. Method: Seventeen IWA (M[subscript age] = 53.19 years)…

  5. Auditory and visual evoked potentials during hyperoxia

    Science.gov (United States)

    Smith, D. B. D.; Strawbridge, P. J.

    1974-01-01

    Experimental study of the auditory and visual averaged evoked potentials (AEPs) recorded during hyperoxia, and investigation of the effect of hyperoxia on the so-called contingent negative variation (CNV). No effect of hyperoxia was found on the auditory AEP, the visual AEP, or the CNV. Comparisons with previous studies are discussed.

  6. Auditory Processing Disorder and Foreign Language Acquisition

    Science.gov (United States)

    Veselovska, Ganna

    2015-01-01

    This article aims at exploring various strategies for coping with the auditory processing disorder in the light of foreign language acquisition. The techniques relevant to dealing with the auditory processing disorder can be attributed to environmental and compensatory approaches. The environmental one involves actions directed at creating a…

  7. Bilateral duplication of the internal auditory canal

    International Nuclear Information System (INIS)

    Weon, Young Cheol; Kim, Jae Hyoung; Choi, Sung Kyu; Koo, Ja-Won

    2007-01-01

    Duplication of the internal auditory canal is an extremely rare temporal bone anomaly that is believed to result from aplasia or hypoplasia of the vestibulocochlear nerve. We report bilateral duplication of the internal auditory canal in a 28-month-old boy with developmental delay and sensorineural hearing loss. (orig.)

  8. Neural circuits in auditory and audiovisual memory.

    Science.gov (United States)

    Plakke, B; Romanski, L M

    2016-06-01

    Working memory is the ability to employ recently seen or heard stimuli and apply them to changing cognitive context. Although much is known about language processing and visual working memory, the neurobiological basis of auditory working memory is less clear. Historically, part of the problem has been the difficulty in obtaining a robust animal model to study auditory short-term memory. In recent years there has been neurophysiological and lesion studies indicating a cortical network involving both temporal and frontal cortices. Studies specifically targeting the role of the prefrontal cortex (PFC) in auditory working memory have suggested that dorsal and ventral prefrontal regions perform different roles during the processing of auditory mnemonic information, with the dorsolateral PFC performing similar functions for both auditory and visual working memory. In contrast, the ventrolateral PFC (VLPFC), which contains cells that respond robustly to auditory stimuli and that process both face and vocal stimuli may be an essential locus for both auditory and audiovisual working memory. These findings suggest a critical role for the VLPFC in the processing, integrating, and retaining of communication information. This article is part of a Special Issue entitled SI: Auditory working memory. Copyright © 2015 Elsevier B.V. All rights reserved.

  9. Primary Auditory Cortex Regulates Threat Memory Specificity

    Science.gov (United States)

    Wigestrand, Mattis B.; Schiff, Hillary C.; Fyhn, Marianne; LeDoux, Joseph E.; Sears, Robert M.

    2017-01-01

    Distinguishing threatening from nonthreatening stimuli is essential for survival and stimulus generalization is a hallmark of anxiety disorders. While auditory threat learning produces long-lasting plasticity in primary auditory cortex (Au1), it is not clear whether such Au1 plasticity regulates memory specificity or generalization. We used…

  10. Congenital Midline Nasal Mass: Four Cases with Review of Literature

    Directory of Open Access Journals (Sweden)

    Sambhaji Govind Chintale

    2017-12-01

    Full Text Available Introduction Congenital midline nasal masses include nasal dermoids, gliomas, encephaloceles. Although rare, these disorders are clinically important because of their potential for connection to the central nervous system. Preoperative knowledge of an intracranial connection is a necessity to allow for neurosurgical consultation and possible planning for craniotomy. This study discusses the clinical presentation of congenital midline nasal mass and the role of imaging modalities like CT scan and MRI in diagnosis and the surgical management. Materials and Methods  This prospective study is carried from March 2014 to March 2016, during which 4 cases presented to the Otorhinolaryngology department. Pre-operative evaluation of the patients included endoscopic evaluation along with haematological investigations, CT Scan and MRI. The masses were removed with nasal endoscopic sinus surgery or by external approaches and neurosurgical intervention. Result The age of the patients ranged from 3 years to 25 years. Three of them were male and one female. There was one case of nasoethmoidal encephalocele and the other three were dermoids (intranasal dermoid cyst, nasal dermoid cyst and nasal dermoid sinus cyst. Conclusion Congenital midline nasal masses are rare. These disorders are clinically important because of their intracranial connection which require proper evaluation with radiological imaging like CT scan and/or MRI before FNAC and any surgical intervention.

  11. Neurofeedback-Based Enhancement of Single Trial Auditory Evoked Potentials: Feasibility in Healthy Subjects.

    Science.gov (United States)

    Rieger, Kathryn; Rarra, Marie-Helene; Moor, Nicolas; Diaz Hernandez, Laura; Baenninger, Anja; Razavi, Nadja; Dierks, Thomas; Hubl, Daniela; Koenig, Thomas

    2018-03-01

    Previous studies showed a global reduction of the event-related potential component N100 in patients with schizophrenia, a phenomenon that is even more pronounced during auditory verbal hallucinations. This reduction assumingly results from dysfunctional activation of the primary auditory cortex by inner speech, which reduces its responsiveness to external stimuli. With this study, we tested the feasibility of enhancing the responsiveness of the primary auditory cortex to external stimuli with an upregulation of the event-related potential component N100 in healthy control subjects. A total of 15 healthy subjects performed 8 double-sessions of EEG-neurofeedback training over 2 weeks. The results of the used linear mixed effect model showed a significant active learning effect within sessions ( t = 5.99, P < .001) against an unspecific habituation effect that lowered the N100 amplitude over time. Across sessions, a significant increase in the passive condition ( t = 2.42, P = .03), named as carry-over effect, was observed. Given that the carry-over effect is one of the ultimate aims of neurofeedback, it seems reasonable to apply this neurofeedback training protocol to influence the N100 amplitude in patients with schizophrenia. This intervention could provide an alternative treatment option for auditory verbal hallucinations in these patients.

  12. Experiences from Auditory Brainstem Implantation (ABIs) in four paediatric patients.

    Science.gov (United States)

    Lundin, Karin; Stillesjö, Fredrik; Nyberg, Gunnar; Rask-Andersen, Helge

    2016-01-01

    Indications for auditory brainstem implants (ABIs) have been widened from patients with neurofibromatosis type 2 (NF2) to paediatric patients with congenital cochlear malformations, cochlear nerve hypoplasia/aplasia, or cochlear ossification after meningitis. We present four ABI surgeries performed in children at Uppsala University Hospital in Sweden since 2009. Three children were implanted with implants from Cochlear Ltd. (Lane Cove, Australia) and one child with an implant from MedEl GMBH (Innsbruck, Austria). A boy with Goldenhar syndrome was implanted with a Cochlear Nucleus ABI24M at age 2 years (patient 1). Another boy with CHARGE syndrome was implanted with a Cochlear Nucleus ABI541 at age 2.5 years (patient 2). Another boy with post-ossification meningitis was implanted with a Cochlear Nucleus ABI24M at age 4 years (patient 3). A girl with cochlear aplasia was implanted with a MedEl Synchrony ABI at age 3 years (patient 4). In patients 1, 2, and 3, the trans-labyrinthine approach was used, and in patient 4 the retro-sigmoid approach was used. Three of the four children benefited from their ABIs and use it full time. Two of the full time users had categories of auditory performance (CAP) score of 4 at their last follow up visit (6 and 2.5 years postoperative) which means they can discriminate consistently any combination of two of Ling's sounds. One child has not been fully evaluated yet, but is a full time user and had CAP 2 (responds to speech sounds) after 3 months of ABI use. No severe side or unpleasant stimulation effects have been observed so far. There was one case of immediate electrode migration and one case of implant device failure after 6.5 years. ABI should be considered as an option in the rehabilitation of children with similar diagnoses.

  13. Regulating multiple externalities

    DEFF Research Database (Denmark)

    Waldo, Staffan; Jensen, Frank; Nielsen, Max

    2016-01-01

    Open access is a well-known externality problem in fisheries causing excess capacity and overfishing. Due to global warming, externality problems from CO2 emissions have gained increased interest. With two externality problems, a first-best optimum can be achieved by using two regulatory instrume......Open access is a well-known externality problem in fisheries causing excess capacity and overfishing. Due to global warming, externality problems from CO2 emissions have gained increased interest. With two externality problems, a first-best optimum can be achieved by using two regulatory...

  14. Temporal auditory processing in elders

    Directory of Open Access Journals (Sweden)

    Azzolini, Vanuza Conceição

    2010-03-01

    Full Text Available Introduction: In the trial of aging all the structures of the organism are modified, generating intercurrences in the quality of the hearing and of the comprehension. The hearing loss that occurs in consequence of this trial occasion a reduction of the communicative function, causing, also, a distance of the social relationship. Objective: Comparing the performance of the temporal auditory processing between elderly individuals with and without hearing loss. Method: The present study is characterized for to be a prospective, transversal and of diagnosis character field work. They were analyzed 21 elders (16 women and 5 men, with ages between 60 to 81 years divided in two groups, a group "without hearing loss"; (n = 13 with normal auditive thresholds or restricted hearing loss to the isolated frequencies and a group "with hearing loss" (n = 8 with neurosensory hearing loss of variable degree between light to moderately severe. Both the groups performed the tests of frequency (PPS and duration (DPS, for evaluate the ability of temporal sequencing, and the test Randon Gap Detection Test (RGDT, for evaluate the temporal resolution ability. Results: It had not difference statistically significant between the groups, evaluated by the tests DPS and RGDT. The ability of temporal sequencing was significantly major in the group without hearing loss, when evaluated by the test PPS in the condition "muttering". This result presented a growing one significant in parallel with the increase of the age group. Conclusion: It had not difference in the temporal auditory processing in the comparison between the groups.

  15. A Brain System for Auditory Working Memory.

    Science.gov (United States)

    Kumar, Sukhbinder; Joseph, Sabine; Gander, Phillip E; Barascud, Nicolas; Halpern, Andrea R; Griffiths, Timothy D

    2016-04-20

    The brain basis for auditory working memory, the process of actively maintaining sounds in memory over short periods of time, is controversial. Using functional magnetic resonance imaging in human participants, we demonstrate that the maintenance of single tones in memory is associated with activation in auditory cortex. In addition, sustained activation was observed in hippocampus and inferior frontal gyrus. Multivoxel pattern analysis showed that patterns of activity in auditory cortex and left inferior frontal gyrus distinguished the tone that was maintained in memory. Functional connectivity during maintenance was demonstrated between auditory cortex and both the hippocampus and inferior frontal cortex. The data support a system for auditory working memory based on the maintenance of sound-specific representations in auditory cortex by projections from higher-order areas, including the hippocampus and frontal cortex. In this work, we demonstrate a system for maintaining sound in working memory based on activity in auditory cortex, hippocampus, and frontal cortex, and functional connectivity among them. Specifically, our work makes three advances from the previous work. First, we robustly demonstrate hippocampal involvement in all phases of auditory working memory (encoding, maintenance, and retrieval): the role of hippocampus in working memory is controversial. Second, using a pattern classification technique, we show that activity in the auditory cortex and inferior frontal gyrus is specific to the maintained tones in working memory. Third, we show long-range connectivity of auditory cortex to hippocampus and frontal cortex, which may be responsible for keeping such representations active during working memory maintenance. Copyright © 2016 Kumar et al.

  16. [Identification of risk factors for congenital malformations].

    Science.gov (United States)

    Canals C, Andrea; Cavada C, Gabriel; Nazer H, Julio

    2014-11-01

    The relative importance of congenital malformations as a cause of death in the first year of life is increasing along with the control of preventable causes of perinatal mortality. To identify risk factors for congenital malformations. Retrospective case-control study of births registered in the database of The Latin American Collaborative Study of Congenital Malformations (ECLAMC), in the period 2001-2010. Birth weight and gestational age were significantly lower in cases than controls, behaving as risk factors and associated with a greater severity of congenital malformations. The risk and severity of congenital malformations increased along with mother's age. Fetal growth retardation, a history of congenital malformations in the family, physical factors and acute illnesses of the mother in the first trimester of pregnancy were also significant risk factors for congenital malformations and their severity. The educational level of the mother was a protective factor for congenital malformations and their severity. Variables previously identified as risk factors for congenital malformations, were significantly related with the occurrence of congenital malformations and their severity.

  17. ExternE National Implementation Finland

    Energy Technology Data Exchange (ETDEWEB)

    Pingoud, K; Maelkki, H; Wihersaari, M; Pirilae, P [VTT Energy, Espoo (Finland); Hongisto, M [Imatran Voima Oy, Vantaa (Finland); Siitonen, S [Ekono Energy Ltd, Espoo (Finland); Johansson, M [Finnish Environment Institute, Helsinki (Finland)

    1999-07-01

    ExternE National Implementation is a continuation of the ExternE Project, funded in part by the European Commission's Joule III Programme. This study is the result of the ExternE National Implementation Project for Finland. Three fuel cycles were selected for the Finnish study: coal, peat and wood-derived biomass, which together are responsible for about 40% of total electricity generation in Finland and about 75% of the non-nuclear fuel based generation. The estimated external costs or damages were dominated by the global warming (GW) impacts in the coal and peat fuel cycles, but knowledge of the true GW impacts is still uncertain. From among other impacts that were valued in monetary terms the human health damages due to airborne emissions dominated in all the three fuel cycles. Monetary valuation for ecosystem impacts is not possible using the ExternE methodology at present. The Meri-Pori power station representing the coal fuel cycle is one of the world's cleanest and most efficient coal-fired power plants with a condensing turbine. The coal is imported mainly from Poland. The estimated health damages were about 4 mECU/kWh, crop damages an order of magnitude lower and damages caused to building materials two orders of magnitude lower. The power stations of the peat and biomass fuel cycles are of CHP type, generating electricity and heat for the district heating systems of two cities. Their fuels are of domestic origin. The estimated health damages allocated to electricity generation were about 5 and 6 mECU/kWh, respectively. The estimates were case-specific and thus an generalisation of the results to the whole electricity generation in Finland is unrealistic. Despite the uncertainties and limitations of the methodology, it is a promising tool in the comparison of similar kinds of fuel cycles, new power plants and pollution abatement technologies and different plant locations with each other. (orig.)

  18. ExternE National Implementation Finland

    International Nuclear Information System (INIS)

    Pingoud, K.; Maelkki, H.; Wihersaari, M.; Pirilae, P.; Hongisto, M.; Siitonen, S.; Johansson, M.

    1999-01-01

    ExternE National Implementation is a continuation of the ExternE Project, funded in part by the European Commission's Joule III Programme. This study is the result of the ExternE National Implementation Project for Finland. Three fuel cycles were selected for the Finnish study: coal, peat and wood-derived biomass, which together are responsible for about 40% of total electricity generation in Finland and about 75% of the non-nuclear fuel based generation. The estimated external costs or damages were dominated by the global warming (GW) impacts in the coal and peat fuel cycles, but knowledge of the true GW impacts is still uncertain. From among other impacts that were valued in monetary terms the human health damages due to airborne emissions dominated in all the three fuel cycles. Monetary valuation for ecosystem impacts is not possible using the ExternE methodology at present. The Meri-Pori power station representing the coal fuel cycle is one of the world's cleanest and most efficient coal-fired power plants with a condensing turbine. The coal is imported mainly from Poland. The estimated health damages were about 4 mECU/kWh, crop damages an order of magnitude lower and damages caused to building materials two orders of magnitude lower. The power stations of the peat and biomass fuel cycles are of CHP type, generating electricity and heat for the district heating systems of two cities. Their fuels are of domestic origin. The estimated health damages allocated to electricity generation were about 5 and 6 mECU/kWh, respectively. The estimates were case-specific and thus an generalisation of the results to the whole electricity generation in Finland is unrealistic. Despite the uncertainties and limitations of the methodology, it is a promising tool in the comparison of similar kinds of fuel cycles, new power plants and pollution abatement technologies and different plant locations with each other. (orig.)

  19. ExternE National Implementation Finland

    Energy Technology Data Exchange (ETDEWEB)

    Pingoud, K.; Maelkki, H.; Wihersaari, M.; Pirilae, P. [VTT Energy, Espoo (Finland); Hongisto, M. [Imatran Voima Oy, Vantaa (Finland); Siitonen, S. [Ekono Energy Ltd, Espoo (Finland); Johansson, M. [Finnish Environment Institute, Helsinki (Finland)

    1999-07-01

    ExternE National Implementation is a continuation of the ExternE Project, funded in part by the European Commission's Joule III Programme. This study is the result of the ExternE National Implementation Project for Finland. Three fuel cycles were selected for the Finnish study: coal, peat and wood-derived biomass, which together are responsible for about 40% of total electricity generation in Finland and about 75% of the non-nuclear fuel based generation. The estimated external costs or damages were dominated by the global warming (GW) impacts in the coal and peat fuel cycles, but knowledge of the true GW impacts is still uncertain. From among other impacts that were valued in monetary terms the human health damages due to airborne emissions dominated in all the three fuel cycles. Monetary valuation for ecosystem impacts is not possible using the ExternE methodology at present. The Meri-Pori power station representing the coal fuel cycle is one of the world's cleanest and most efficient coal-fired power plants with a condensing turbine. The coal is imported mainly from Poland. The estimated health damages were about 4 mECU/kWh, crop damages an order of magnitude lower and damages caused to building materials two orders of magnitude lower. The power stations of the peat and biomass fuel cycles are of CHP type, generating electricity and heat for the district heating systems of two cities. Their fuels are of domestic origin. The estimated health damages allocated to electricity generation were about 5 and 6 mECU/kWh, respectively. The estimates were case-specific and thus an generalisation of the results to the whole electricity generation in Finland is unrealistic. Despite the uncertainties and limitations of the methodology, it is a promising tool in the comparison of similar kinds of fuel cycles, new power plants and pollution abatement technologies and different plant locations with each other. (orig.)

  20. Radiation-induced external ear canal cholesteatoma-like disease

    Energy Technology Data Exchange (ETDEWEB)

    Ishihara, Akiko; Okuno, Hideji; Noguchi, Keisuke; Komatsuzaki, Atsushi [Tokyo Medical and Dental Univ. (Japan). School of Medicine

    1999-06-01

    Three cases of cholesteatoma-like disease in the ear canals after radiation therapy for head and neck tumor were reported. Effect of irradiation on bone and soft tissue including skin brings about pathological reaction to the external ear canal as well. Two types of disease resembling cholesteatomas have been recognized: keratosis obturans (KO) and external auditory canal cholesteatoma (EACC). KO appears to be derived from disease of canal skin involved with keratinization, creating a widning of the canal. EACC, on the other hand, seems to develop in the disease of bony canal where a localized absorption of its bone with invasion of squamous epithelium takes place. (author)

  1. Prenatal education for congenital toxoplasmosis.

    Science.gov (United States)

    Di Mario, Simona; Basevi, Vittorio; Gagliotti, Carlo; Spettoli, Daniela; Gori, Gianfranco; D'Amico, Roberto; Magrini, Nicola

    2015-10-23

    Congenital toxoplasmosis is considered a rare but potentially severe infection. Prenatal education about congenital toxoplasmosis could be the most efficient and least harmful intervention, yet its effectiveness is uncertain. To assess the effects of prenatal education for preventing congenital toxoplasmosis. We searched the Cochrane Pregnancy and Childbirth Group's Trials Register (31 May 2015), and reference lists of relevant papers, reviews and websites. Randomized and quasi-randomized controlled trials of all types of prenatal education on toxoplasmosis infection during pregnancy. Cluster-randomized trials were eligible for inclusion. Two review authors independently assessed trials for inclusion and risk of bias, extracted data and checked them for accuracy. Two cluster-randomized controlled trials (RCTs) (involving a total of 5455 women) met the inclusion criteria. The two included trials measured the effectiveness of the intervention in different ways, which meant that meta-analysis of the results was not possible. The overall quality of the two studies, as assessed using the GRADE approach, was low, with high risk of detection and attrition bias in both included trials.One trial (432 women enrolled) conducted in Canada was judged of low methodological quality. This trial did not report on any of the review's pre-specified primary outcomes and the secondary outcomes reported results only as P values. Moreover, losses to follow-up were high (34%, 147 out of 432 women initially enrolled). The authors concluded that prenatal education can effectively change pregnant women's behavior as it increased pet, personal and food hygiene. The second trial conducted in France was also judged of low methodological quality. Losses to follow-up were also high (44.5%, 2233 out of 5023 women initially enrolled) and differential (40% in the intervention group and 52% in the control group). The authors concluded that prenatal education for congenital toxoplasmoses has a

  2. External Otitis (Swimmer's Ear)

    Science.gov (United States)

    ... otitis. Fungal external otitis (otomycosis), typically caused by Aspergillus niger or Candida albicans, is less common. Boils are ... in the ear. Fungal external otitis caused by Aspergillus niger usually causes grayish black or yellow dots (called ...

  3. ExternE: Externalities of energy Vol. 2. Methodology

    International Nuclear Information System (INIS)

    Berry, J.; Holland, M.; Watkiss, P.

    1995-01-01

    This report describes the methodology used by the ExternE Project of the European Commission (DGXII) JOULE Programme for assessment of the external costs of energy. It is one of a series of reports describing analysis of nuclear, fossil and renewable fuel cycles for assessment of the externalities associated with electricity generation. Part I of the report deals with analysis of impacts, and Part II with the economic valuation of those impacts. Analysis is conducted on a marginal basis, to allow the effect of an incremental investment in a given technology to be quantified. Attention has been paid to the specificity of results with respect to the location of fuel cycle activities, the precise technologies used, and the type and source of fuel. The main advantages of this detailed approach are as follows: It takes full and proper account of the variability of impacts that might result from different power projects; It is more transparent than analysis based on hypothetically 'representative' cases for each of the different fuel cycles; It provides a framework for consistent comparison between fuel cycles. A wide variety of impacts have been considered. These include the effects of air pollution on the natural and human environment, consequences of accidents in the workplace, impacts of noise and visual intrusion on amenity, and the effects of climate change arising from the release of greenhouse gases. Wherever possible we have used the 'impact pathway' or 'damage function' approach to follow the analysis from identification of burdens (e.g. emissions) through to impact assessment and then valuation in monetary terms. This has required a detailed knowledge of the technologies involved, pollutant dispersion, analysis of effects on human and environmental health, and economics. In view of this the project brought together a multi-disciplinary team with experts from many European countries and the USA. The spatial and temporal ranges considered in the analysis are

  4. Congenital adrenal hyperplasia: Case report.

    OpenAIRE

    Jaime Avaria E.; María José Vargas F.; Loreto Triviño F.; Andrea Gleisner E.

    2013-01-01

    INTRODUCTION: Congenital adrenal hyperplasia (CAH) is an autosomal recessive disease whose main cause is the deficiency of 21-hydroxylase, an enzyme involved in the synthesis of cortisol and aldosterone. There are two forms of CAH, a classical and nonclassical form, being the first objective of analysis in the clinical case. Its clinical manifestations vary in severity, depending on the level of hormone deficiency. Within the classic is described the salt-wasting form, whose consequences are ...

  5. Congenital hypopituitarism and renal failure

    Directory of Open Access Journals (Sweden)

    Gaurav Atreja

    2011-01-01

    Full Text Available Congenital hypopituitarism is potentially fatal in the newborn period but treatable if the diagnosis is made early. We report a neonate who presented with hypothermia and severe hypoglycemia. He also had undescended testis and micropenis. Initial screening revealed panhypopituitarism, which was corrected promptly. He developed renal failure due to initial cardiovascular compromise related to hypotension but recovered quickly with standard management. Magnetic resonance imaging revealed absent stalk of anterior pituitary.

  6. Congenital unilateral hydrocephalus - CT findings

    International Nuclear Information System (INIS)

    Schulman, H.; Landau, D.; Schulman, P.; Hertzanu, Y.

    2000-01-01

    Congenital unilateral hydrocephalus is extremely uncommon with 18 cases previously reported in the English literature. Two additional newborns with unilateral hydrocephalus are presented. The second baby also presented a mega cisterna magna. This unusual association between Dandy-Walker variant and unilateral hydrocephalus has not been previously reported. Following ventriculo-peritoneal shunt, the babies had a normal cognitive neurodevelopment. The role of cranial computed tomography (CT) in diagnosis and follow-up is emphasized

  7. ASH External Web Portal (External Portal) -

    Data.gov (United States)

    Department of Transportation — The ASH External Web Portal is a web-based portal that provides single sign-on functionality, making the web portal a single location from which to be authenticated...

  8. Bottom-up influences of voice continuity in focusing selective auditory attention

    OpenAIRE

    Bressler, Scott; Masud, Salwa; Bharadwaj, Hari; Shinn-Cunningham, Barbara

    2014-01-01

    Selective auditory attention causes a relative enhancement of the neural representation of important information and suppression of the neural representation of distracting sound, which enables a listener to analyze and interpret information of interest. Some studies suggest that in both vision and in audition, the “unit” on which attention operates is an object: an estimate of the information coming from a particular external source out in the world. In this view, which object ends up in the...

  9. Congenital uronephropathy pattern in children

    Directory of Open Access Journals (Sweden)

    Husein Alatas

    2001-10-01

    Full Text Available To obtain the basic data of congenital uronephropathy pattern and the affecting factors in children, we conducted a cross-sectional study at the Department of Child Health Cipto Mangunkusumo (CM Hospital Jakarta from 1995 to 1999 and 9 teaching hospitals throughout Indonesia. During the study period 134 patients were obtained, 116 patients from the CM Hospital and 18 patients from other teaching hospitals. Most patients (48.8% were below 1 year of age; male were affected more than female (2.4:1. The disorder was classified into two groups, i.e., congenital nephropathy and uropathy. There were 10 children with nephropathy, i.e., 4 with unilateral renal hypoplasia, 3 with polycystic kidney, and 3 with renal agenesis. In the uropathy group, 43 were with hypospadia, 22 with primary reflux vesicoureter, 18 with neurogenic bladder, and 17 with ureteropelvic junction obstruction. The complications found were urinary tract infection (71.2%, chronic renal failure (15.7%, hypertension (3.7%, and acute renal failure (1.5%. Consanguinity, familial disorders, maternal diseases, x-ray exposure and abortion efforts were found in a small proportion of patients. History of drug or herbs use in the first trimester of pregnancy was found in a large proportion of patients, mostly took analgesics (especially acetaminophen. In conclusion, uropathy disorders were much more common than congenital nephropathy. The most common complication was urinary tract infection, followed by chronic renal failure, hypertension, and acute renal failure.

  10. Hennekam syndrome presenting as nonimmune hydrops fetalis, congenital chylothorax, and congenital pulmonary lymphangiectasia

    NARCIS (Netherlands)

    Bellini, Carlo; Mazzella, Massimo; Arioni, Cesare; Campisi, Corradino; Taddei, Gioconda; Tomà, Paolo; Boccardo, Francesco; Hennekam, Raoul C.; Serra, Giovanni

    2003-01-01

    We report a female infant with congenital lymphedema, facial anomalies, intestinal lymphangiectasia consistent with a diagnosis of Hennekam syndrome. At birth the patient presented with severe respiratory distress due to nonimmune hydrops fetalis, a congenital chylothorax (CC), and pulmonary

  11. Congenital aplasia of the semicircular canals.

    Science.gov (United States)

    Satar, Bulent; Mukherji, Suresh K; Telian, Steven A

    2003-05-01

    To describe the underrecognized inner ear malformation characterized by complete aplasia of the labyrinthine semicircular canals associated with a relatively well-formed cochlea, to investigate its relationship with known syndromic forms of hearing loss, and to hypothesize regarding the potential embryopathogenesis of this anomaly. A retrospective case review consisting of cases of sensorineural hearing loss with radiographic evidence demonstrating agenesis of the semicircular canals associated with a cochlea that was either morphologically normal or sufficiently well developed to accommodate the full insertion of a cochlear implant electrode. Cases were identified by computerized tomography findings that identified the anomaly under study. Departments of otolaryngology and radiology in a tertiary referral center, with a large cochlear implant program serving over 800 patients, more than half of whom are children. Fifteen patients with the anomaly under study were identified. Each patient underwent a complete otologic examination, audiometric studies, and high resolution computerized tomography of the temporal bone in axial and coronal planes. The bony morphology of the cochlea, round and oval windows, vestibule, semicircular canals, and vestibular aqueduct, and the course of the facial nerve were examined. Auditory findings and otologic treatment are presented. Of the 15 identified patients, 4 were nonsyndromic, 9 had CHARGE association (Coloboma of the eye, congenital Heart defects, choanal Atresia, mental and/or growth Retardation, Genital hypoplasia, and Ear anomalies and/or deafness), 1 met criteria for Noonan's syndrome, and one had features of both these syndromes. Although the cochlea was present in all cases, the cochlear morphology was usually abnormal in the CHARGE association patients. Of the 20 ears in the CHARGE subjects, only 3 ears (15%) were seen to have completely normal development of the cochlea in both the basal and upper turns. The others

  12. Auditory memory function in expert chess players.

    Science.gov (United States)

    Fattahi, Fariba; Geshani, Ahmad; Jafari, Zahra; Jalaie, Shohreh; Salman Mahini, Mona

    2015-01-01

    Chess is a game that involves many aspects of high level cognition such as memory, attention, focus and problem solving. Long term practice of chess can improve cognition performances and behavioral skills. Auditory memory, as a kind of memory, can be influenced by strengthening processes following long term chess playing like other behavioral skills because of common processing pathways in the brain. The purpose of this study was to evaluate the auditory memory function of expert chess players using the Persian version of dichotic auditory-verbal memory test. The Persian version of dichotic auditory-verbal memory test was performed for 30 expert chess players aged 20-35 years and 30 non chess players who were matched by different conditions; the participants in both groups were randomly selected. The performance of the two groups was compared by independent samples t-test using SPSS version 21. The mean score of dichotic auditory-verbal memory test between the two groups, expert chess players and non-chess players, revealed a significant difference (p≤ 0.001). The difference between the ears scores for expert chess players (p= 0.023) and non-chess players (p= 0.013) was significant. Gender had no effect on the test results. Auditory memory function in expert chess players was significantly better compared to non-chess players. It seems that increased auditory memory function is related to strengthening cognitive performances due to playing chess for a long time.

  13. Auditory attention activates peripheral visual cortex.

    Directory of Open Access Journals (Sweden)

    Anthony D Cate

    Full Text Available BACKGROUND: Recent neuroimaging studies have revealed that putatively unimodal regions of visual cortex can be activated during auditory tasks in sighted as well as in blind subjects. However, the task determinants and functional significance of auditory occipital activations (AOAs remains unclear. METHODOLOGY/PRINCIPAL FINDINGS: We examined AOAs in an intermodal selective attention task to distinguish whether they were stimulus-bound or recruited by higher-level cognitive operations associated with auditory attention. Cortical surface mapping showed that auditory occipital activations were localized to retinotopic visual cortex subserving the far peripheral visual field. AOAs depended strictly on the sustained engagement of auditory attention and were enhanced in more difficult listening conditions. In contrast, unattended sounds produced no AOAs regardless of their intensity, spatial location, or frequency. CONCLUSIONS/SIGNIFICANCE: Auditory attention, but not passive exposure to sounds, routinely activated peripheral regions of visual cortex when subjects attended to sound sources outside the visual field. Functional connections between auditory cortex and visual cortex subserving the peripheral visual field appear to underlie the generation of AOAs, which may reflect the priming of visual regions to process soon-to-appear objects associated with unseen sound sources.

  14. Auditory conflict and congruence in frontotemporal dementia.

    Science.gov (United States)

    Clark, Camilla N; Nicholas, Jennifer M; Agustus, Jennifer L; Hardy, Christopher J D; Russell, Lucy L; Brotherhood, Emilie V; Dick, Katrina M; Marshall, Charles R; Mummery, Catherine J; Rohrer, Jonathan D; Warren, Jason D

    2017-09-01

    Impaired analysis of signal conflict and congruence may contribute to diverse socio-emotional symptoms in frontotemporal dementias, however the underlying mechanisms have not been defined. Here we addressed this issue in patients with behavioural variant frontotemporal dementia (bvFTD; n = 19) and semantic dementia (SD; n = 10) relative to healthy older individuals (n = 20). We created auditory scenes in which semantic and emotional congruity of constituent sounds were independently probed; associated tasks controlled for auditory perceptual similarity, scene parsing and semantic competence. Neuroanatomical correlates of auditory congruity processing were assessed using voxel-based morphometry. Relative to healthy controls, both the bvFTD and SD groups had impaired semantic and emotional congruity processing (after taking auditory control task performance into account) and reduced affective integration of sounds into scenes. Grey matter correlates of auditory semantic congruity processing were identified in distributed regions encompassing prefrontal, parieto-temporal and insular areas and correlates of auditory emotional congruity in partly overlapping temporal, insular and striatal regions. Our findings suggest that decoding of auditory signal relatedness may probe a generic cognitive mechanism and neural architecture underpinning frontotemporal dementia syndromes. Copyright © 2017 The Author(s). Published by Elsevier Ltd.. All rights reserved.

  15. RISK FACTORS IN CHILD CONGENITAL MALFORMATIONS

    OpenAIRE

    Alina-Costina LUCA; Mirabela SUBOTNICU

    2015-01-01

    Congenital heart malformations are among the most common congenital malformations. Congenital heart malformations occur due to genetic and environmental factors during embryonic morphogenesis period of the heart. About 25% of these malformations are severe, requiring intervention immediately after birth or in infancy. Abnormalities of structure and function of the heart and great vessels are the consequence of teratogenic factors occurring between day 19 and 45 of gestation. (Yagel et al...

  16. Hip dysplasia and congenital hip dislocation

    Energy Technology Data Exchange (ETDEWEB)

    Lingg, G.; Nebel, G.; von Torklus, D.

    1981-11-01

    In human genetics and orthopedics quite different answers have been given to the question of hereditary transmission and frequency of hip dysplasia in families of children with congenital hip dislocation. We therefore have made roentgenometric measurements of 110 parents of children with congenital hip dislocation. In 25% we found abnormal flat acetabulae, whereas 12% had pathologic deep hips. This may propose a new concept of morphology of congenital hip dysplasia.

  17. Congenital pseudoarthrosis associated with venous malformation

    International Nuclear Information System (INIS)

    Al-Hadidy, A.; Haroun, A.; Al-Ryalat, N.; Hamamy, H.; Al-Hadidi, S.

    2007-01-01

    Congenital pseudoarthrosis is a pathologic entity that may be isolated, or may be associated with neurofibromatosis. We report the case of a 3-year-old female with congenital pseudoarthrosis involving the right tibia and fibula. Magnetic resonance imaging (MRI) and complementary magnetic resonance angiogram (MRA) revealed a lobulated mass with vivid enhancement, which led to the diagnosis of venous malformation. This is the first report of congenital pseudoarthrosis caused by the presence of a vascular malformation. (orig.)

  18. Congenital pseudoarthrosis associated with venous malformation

    Energy Technology Data Exchange (ETDEWEB)

    Al-Hadidy, A.; Haroun, A.; Al-Ryalat, N. [Jordan University Hospital, Radiology Department, P.O. Box 340621, Amman (Jordan); Hamamy, H. [Endocrinology and Genetics, National Center for Diabetes, Amman (Jordan); Al-Hadidi, S. [Jordan University Hospital, Departments of Orthopedics, Amman (Jordan)

    2007-06-15

    Congenital pseudoarthrosis is a pathologic entity that may be isolated, or may be associated with neurofibromatosis. We report the case of a 3-year-old female with congenital pseudoarthrosis involving the right tibia and fibula. Magnetic resonance imaging (MRI) and complementary magnetic resonance angiogram (MRA) revealed a lobulated mass with vivid enhancement, which led to the diagnosis of venous malformation. This is the first report of congenital pseudoarthrosis caused by the presence of a vascular malformation. (orig.)

  19. Auditory Connections and Functions of Prefrontal Cortex

    Directory of Open Access Journals (Sweden)

    Bethany ePlakke

    2014-07-01

    Full Text Available The functional auditory system extends from the ears to the frontal lobes with successively more complex functions occurring as one ascends the hierarchy of the nervous system. Several areas of the frontal lobe receive afferents from both early and late auditory processing regions within the temporal lobe. Afferents from the early part of the cortical auditory system, the auditory belt cortex, which are presumed to carry information regarding auditory features of sounds, project to only a few prefrontal regions and are most dense in the ventrolateral prefrontal cortex (VLPFC. In contrast, projections from the parabelt and the rostral superior temporal gyrus (STG most likely convey more complex information and target a larger, widespread region of the prefrontal cortex. Neuronal responses reflect these anatomical projections as some prefrontal neurons exhibit responses to features in acoustic stimuli, while other neurons display task-related responses. For example, recording studies in non-human primates indicate that VLPFC is responsive to complex sounds including vocalizations and that VLPFC neurons in area 12/47 respond to sounds with similar acoustic morphology. In contrast, neuronal responses during auditory working memory involve a wider region of the prefrontal cortex. In humans, the frontal lobe is involved in auditory detection, discrimination, and working memory. Past research suggests that dorsal and ventral subregions of the prefrontal cortex process different types of information with dorsal cortex processing spatial/visual information and ventral cortex processing non-spatial/auditory information. While this is apparent in the non-human primate and in some neuroimaging studies, most research in humans indicates that specific task conditions, stimuli or previous experience may bias the recruitment of specific prefrontal regions, suggesting a more flexible role for the frontal lobe during auditory cognition.

  20. Auditory connections and functions of prefrontal cortex

    Science.gov (United States)

    Plakke, Bethany; Romanski, Lizabeth M.

    2014-01-01

    The functional auditory system extends from the ears to the frontal lobes with successively more complex functions occurring as one ascends the hierarchy of the nervous system. Several areas of the frontal lobe receive afferents from both early and late auditory processing regions within the temporal lobe. Afferents from the early part of the cortical auditory system, the auditory belt cortex, which are presumed to carry information regarding auditory features of sounds, project to only a few prefrontal regions and are most dense in the ventrolateral prefrontal cortex (VLPFC). In contrast, projections from the parabelt and the rostral superior temporal gyrus (STG) most likely convey more complex information and target a larger, widespread region of the prefrontal cortex. Neuronal responses reflect these anatomical projections as some prefrontal neurons exhibit responses to features in acoustic stimuli, while other neurons display task-related responses. For example, recording studies in non-human primates indicate that VLPFC is responsive to complex sounds including vocalizations and that VLPFC neurons in area 12/47 respond to sounds with similar acoustic morphology. In contrast, neuronal responses during auditory working memory involve a wider region of the prefrontal cortex. In humans, the frontal lobe is involved in auditory detection, discrimination, and working memory. Past research suggests that dorsal and ventral subregions of the prefrontal cortex process different types of information with dorsal cortex processing spatial/visual information and ventral cortex processing non-spatial/auditory information. While this is apparent in the non-human primate and in some neuroimaging studies, most research in humans indicates that specific task conditions, stimuli or previous experience may bias the recruitment of specific prefrontal regions, suggesting a more flexible role for the frontal lobe during auditory cognition. PMID:25100931

  1. Pitch contour impairment in congenital amusia: New insights from the Self-paced Audio-visual Contour Task (SACT.

    Directory of Open Access Journals (Sweden)

    Xuejing Lu

    Full Text Available Individuals with congenital amusia usually exhibit impairments in melodic contour processing when asked to compare pairs of melodies that may or may not be identical to one another. However, it is unclear whether the impairment observed in contour processing is caused by an impairment of pitch discrimination, or is a consequence of poor pitch memory. To help resolve this ambiguity, we designed a novel Self-paced Audio-visual Contour Task (SACT that evaluates sensitivity to contour while placing minimal burden on memory. In this task, participants control the pace of an auditory contour that is simultaneously accompanied by a visual contour, and they are asked to judge whether the two contours are congruent or incongruent. In Experiment 1, melodic contours varying in pitch were presented with a series of dots that varied in spatial height. Amusics exhibited reduced sensitivity to audio-visual congruency in comparison to control participants. To exclude the possibility that the impairment arises from a general deficit in cross-modal mapping, Experiment 2 examined sensitivity to cross-modal mapping for two other auditory dimensions: timbral brightness and loudness. Amusics and controls were significantly more sensitive to large than small contour changes, and to changes in loudness than changes in timbre. However, there were no group differences in cross-modal mapping, suggesting that individuals with congenital amusia can comprehend spatial representations of acoustic information. Taken together, the findings indicate that pitch contour processing in congenital amusia remains impaired even when pitch memory is relatively unburdened.

  2. Pitch contour impairment in congenital amusia: New insights from the Self-paced Audio-visual Contour Task (SACT).

    Science.gov (United States)

    Lu, Xuejing; Sun, Yanan; Ho, Hao Tam; Thompson, William Forde

    2017-01-01

    Individuals with congenital amusia usually exhibit impairments in melodic contour processing when asked to compare pairs of melodies that may or may not be identical to one another. However, it is unclear whether the impairment observed in contour processing is caused by an impairment of pitch discrimination, or is a consequence of poor pitch memory. To help resolve this ambiguity, we designed a novel Self-paced Audio-visual Contour Task (SACT) that evaluates sensitivity to contour while placing minimal burden on memory. In this task, participants control the pace of an auditory contour that is simultaneously accompanied by a visual contour, and they are asked to judge whether the two contours are congruent or incongruent. In Experiment 1, melodic contours varying in pitch were presented with a series of dots that varied in spatial height. Amusics exhibited reduced sensitivity to audio-visual congruency in comparison to control participants. To exclude the possibility that the impairment arises from a general deficit in cross-modal mapping, Experiment 2 examined sensitivity to cross-modal mapping for two other auditory dimensions: timbral brightness and loudness. Amusics and controls were significantly more sensitive to large than small contour changes, and to changes in loudness than changes in timbre. However, there were no group differences in cross-modal mapping, suggesting that individuals with congenital amusia can comprehend spatial representations of acoustic information. Taken together, the findings indicate that pitch contour processing in congenital amusia remains impaired even when pitch memory is relatively unburdened.

  3. Clinical and morphological characteristics of malformations in infants with congenital cytomegalovirus infection and congenital toxoplasmosis

    Directory of Open Access Journals (Sweden)

    L. Yu. Barycheva

    2015-01-01

    Full Text Available The results of following up infants with intrauterine infections and malformations were retrospectively analyzed. Infants with malformations were diagnosed as having congenital cytomegalovirus infection and congenital toxoplasmosis in 127 and 69 cases, respectively. The aim of the study was to characterize malformations in infants with congenital cytomegalovirus and congenital Toxoplasma infections. The infants with malformations in congenital cytomegalovirus infection were found to have higher mortality rates (61,4% than those with congenital toxoplasmosis (34,8%. Postmortem analysis indicated that there was a predominance of embryopathies in infants with congenital cytomegalovirus infection and that of fetopathies in those with congenital toxoplasmosis. The dead infants with congenital cytomegalovirus infection had more commonly developed visceral defects, including heart diseases, pneumopathies, gastrointestinal and genitourinary abnormalities; fetopathies of the central nervous system and eye were prevalent in congenital toxoplasmosis. The surviving children with congenital toxoplasmosis were more frequently observed to have disabling CNS and ocular sequels as obstructive hydrocephalus, infantile cerebral palsy, complete or partial blindness, and cerebrasthenic disorders than those with congenital cytomegalovirus infection. 

  4. Congenital heart defects and extracardiac malformations.

    Science.gov (United States)

    Rosa, Rosana Cardoso M; Rosa, Rafael Fabiano M; Zen, Paulo Ricardo G; Paskulin, Giorgio Adriano

    2013-06-01

    To review the association between congenital heart defects and extracardiac malformations. Scientific articles were searched in the Medline, Lilacs, and SciELO databases, using the descriptors "congenital heart disease," "congenital heart defects," "congenital cardiac malformations," "extracardiac defects," and "extracardiac malformations." All case series that specifically explored the association between congenital heart defects and extracardiac malformations were included. Congenital heart diseases are responsible for about 40% of birth defects, being one of the most common and severe malformations. Extracardiac malformations are observed in 7 to 50% of the patients with congenital heart disease, bringing a greater risk of comorbidity and mortality and increasing the risks related to heart surgery. Different studies have attempted to assess the presence of extracardiac abnormalities in patients with congenital heart disease. Among the changes described, those of the urinary tract are more often reported. However, no study has evaluated all patients in the same way. Extracardiac abnormalities are frequent among patients with congenital heart disease, and patients with these alterations may present an increased risk of morbimortality. Therefore, some authors have been discussing the importance and cost-effectiveness of screening these children for other malformations by complementary exams.

  5. Congenital hypothyroidism presenting with postpartum bradycardia

    International Nuclear Information System (INIS)

    Kara, S.; Tayman, C.; Tonbul, A.; Tatli, M.; Andiran, N.; Turkay, S.

    2013-01-01

    Congenital hypothyroidism is a clinical condition characterized by lack of thyroid hormone because of thyroid gland developmental and thyroid hormone biosynthesis disorders. The most common cause of permanent hypothyroidism is congenital factors. Prompt diagnosis is critical. However, overt signs of hypothyroidism are rarely present at birth, and 95% of affected babies are asymptomatic. Hypoxemia, apnea, acidosis, increased intracranial pressure, vagal stimulus and central nerve system abnormalities represent the most common causes of bradycardia in the neonate. Bradycardia associated with congenital hypothyroidism is very rare. In this paper, a case of severe congenital hypothyroidism, induced by maternal blocker antibodies, who presented with bradycardia, is reported. (author)

  6. Major congenital anomalies in a Danish region

    DEFF Research Database (Denmark)

    Garne, Ester; Hansen, Anne Vinkel; Birkelund, Anne Sofie

    2014-01-01

    INTRODUCTION: This study describes the prevalence of congenital anomalies and changes over time in birth outcome, mortality and chronic maternal diseases. MATERIAL AND METHODS: This study was based on population data from the EUROCAT registry covering the Funen County, Denmark, 1995...... mortality decreased significantly over time for cases with major congenital anomalies (p congenital anomaly cases, 8% had a registration of one of these chronic maternal diseases......: diabetes, epilepsy, mental disorder, thyroid disease, asthma, or inflammatory bowel disease. Medication for these conditions accounted for 46% of maternal drug use. CONCLUSION: Maternal morbidity and use of potentially teratogenic medication have increased among congenital anomaly cases. Foetal and infant...

  7. Congenital rubella syndrome and delayed manifestations

    DEFF Research Database (Denmark)

    Dammeyer, Jesper Herup

    2010-01-01

    Objective: Several hypotheses of different medical and psychological delayed manifestations among people who have congenital rubella syndrome (CRS) have been discussed. This study tests some of these hypotheses of delayed manifestations. Methods: Gathering information about 35 individuals who hav...... which people with CRS face must primarily be understood in relation to congenital deafblindness and dual sensory and communicative deprivation....... CRS and who are congenitally deafblind. Results: None of the hypotheses could be confirmed when individuals with CRS were compared to a control group of individuals who were congenital deafblind with different aetiology than CRS. Conclusions: This study concludes that those health related problems...

  8. Neuromechanistic Model of Auditory Bistability.

    Directory of Open Access Journals (Sweden)

    James Rankin

    2015-11-01

    Full Text Available Sequences of higher frequency A and lower frequency B tones repeating in an ABA- triplet pattern are widely used to study auditory streaming. One may experience either an integrated percept, a single ABA-ABA- stream, or a segregated percept, separate but simultaneous streams A-A-A-A- and -B---B--. During minutes-long presentations, subjects may report irregular alternations between these interpretations. We combine neuromechanistic modeling and psychoacoustic experiments to study these persistent alternations and to characterize the effects of manipulating stimulus parameters. Unlike many phenomenological models with abstract, percept-specific competition and fixed inputs, our network model comprises neuronal units with sensory feature dependent inputs that mimic the pulsatile-like A1 responses to tones in the ABA- triplets. It embodies a neuronal computation for percept competition thought to occur beyond primary auditory cortex (A1. Mutual inhibition, adaptation and noise are implemented. We include slow NDMA recurrent excitation for local temporal memory that enables linkage across sound gaps from one triplet to the next. Percepts in our model are identified in the firing patterns of the neuronal units. We predict with the model that manipulations of the frequency difference between tones A and B should affect the dominance durations of the stronger percept, the one dominant a larger fraction of time, more than those of the weaker percept-a property that has been previously established and generalized across several visual bistable paradigms. We confirm the qualitative prediction with our psychoacoustic experiments and use the behavioral data to further constrain and improve the model, achieving quantitative agreement between experimental and modeling results. Our work and model provide a platform that can be extended to consider other stimulus conditions, including the effects of context and volition.

  9. Severe diarrhea-dehydration in infancy permanently alters auditory function.

    Science.gov (United States)

    Todd, N Wendell

    2012-02-01

    Of the myriad etiologies of sensorineural hearing impairment, metabolic stress is rarely considered. I posit that severe dehydration in conjunction with hypoxia, at least during infancy, prompts permanent changes in the cochlea. In a population-based prospective study of otitis media, children without otitis were found to have at age 4-8 years, worse auditory thresholds if as an infant had been hospitalized for diarrhea-dehydration. What is more, stapedius reflex thresholds tended to be lower in children who had been hospitalized for diarrhea-dehydration: that is, less acoustic energy for arousal or to be frightening. The hypothesis that the transient metabolic stress of dehydration with hypoxia prompts permanent sensorineural hearing impairment with reduced uncomfortable loudness thresholds, is both (1) consistent in an evolutionary sense with a subsequent survival advantage, and (2) subject to verification both by descriptive studies of children undergoing ECMO (ExtraCorporeal Membrane Oxygenation) or care for congenital diaphragmatic hernia, and by animal studies. Copyright © 2011 Elsevier Ltd. All rights reserved.

  10. A rare combination: congenital factor VII deficiency with Chiari malformation.

    Science.gov (United States)

    Bay, Ali; Aktekin, Elif; Erkutlu, Ibrahim

    2015-12-01

    Congenital factor (VII) deficiency is a rare bleeding disorder. We present a patient with congenital FVII deficiency and congenital hydrocephalus who underwent a ventriculoperitoneal shunt operation and needed no prophylaxis after the procedure.

  11. Congenital hydrocephalus in clinical practice : A genetic diagnostic approach

    NARCIS (Netherlands)

    Verhagen, J. M. A.; Schrander-Stumpel, C. T. R. M.; Krapels, P. C.; de Die-Smulders, C. E. M.; van Lint, F. H. M.; Willekes, C.; Weber, J. W.; Gavilanes, A. W. D.; Macville, M. V. E.; Stegmann, A. P. A.; Engelen, J. J. M.; Bakker, J.; Vos, Y. J.; Frints, S. G. M.

    2011-01-01

    Congenital hydrocephalus is a common and often disabling disorder. The etiology is very heterogeneous. Little is known about the genetic causes of congenital hydrocephalus. A retrospective survey was performed including patients with primary congenital hydrocephalus referred to the Department of

  12. Assessing the aging effect on auditory-verbal memory by Persian version of dichotic auditory verbal memory test

    Directory of Open Access Journals (Sweden)

    Zahra Shahidipour

    2014-01-01

    Conclusion: Based on the obtained results, significant reduction in auditory memory was seen in aged group and the Persian version of dichotic auditory-verbal memory test, like many other auditory verbal memory tests, showed the aging effects on auditory verbal memory performance.

  13. Touching words is not enough: how visual experience influences haptic-auditory associations in the "Bouba-Kiki" effect.

    Science.gov (United States)

    Fryer, Louise; Freeman, Jonathan; Pring, Linda

    2014-08-01

    Since Köhler's experiments in the 1920s, researchers have demonstrated a correspondence between words and shapes. Dubbed the "Bouba-Kiki" effect, these auditory-visual associations extend across cultures and are thought to be universal. More recently the effect has been shown in other modalities including taste, suggesting the effect is independent of vision. The study presented here tested the "Bouba-Kiki" effect in the auditory-haptic modalities, using 2D cut-outs and 3D models based on Köhler's original drawings. Presented with shapes they could feel but not see, sighted participants showed a robust "Bouba-Kiki" effect. However, in a sample of people with a range of visual impairments, from congenital total blindness to partial sight, the effect was significantly less pronounced. The findings suggest that, in the absence of a direct visual stimulus, visual imagery plays a role in crossmodal integration. Copyright © 2014 Elsevier B.V. All rights reserved.

  14. Network and external perturbation induce burst synchronisation in cat cerebral cortex

    Science.gov (United States)

    Lameu, Ewandson L.; Borges, Fernando S.; Borges, Rafael R.; Batista, Antonio M.; Baptista, Murilo S.; Viana, Ricardo L.

    2016-05-01

    The brain of mammals are divided into different cortical areas that are anatomically connected forming larger networks which perform cognitive tasks. The cat cerebral cortex is composed of 65 areas organised into the visual, auditory, somatosensory-motor and frontolimbic cognitive regions. We have built a network of networks, in which networks are connected among themselves according to the connections observed in the cat cortical areas aiming to study how inputs drive the synchronous behaviour in this cat brain-like network. We show that without external perturbations it is possible to observe high level of bursting synchronisation between neurons within almost all areas, except for the auditory area. Bursting synchronisation appears between neurons in the auditory region when an external perturbation is applied in another cognitive area. This is a clear evidence that burst synchronisation and collective behaviour in the brain might be a process mediated by other brain areas under stimulation.

  15. Kidney transplantation in a patient with absent right common iliac artery and congenital renal abnormalities.

    Science.gov (United States)

    Tay, Clifton Ming; Siew, Edwin Poh Yiew; Ng, Tze-Kiat; Vathsala, Anantharanam; Tiong, Ho Yee

    2015-01-01

    Congenital atresia of the common and external iliac arteries is a rare vascular anomaly that may be associated with congenital renal or genitourinary malformations. In ESRD patients, its presence may pose potential problems during renal transplantation. We report a rare case of kidney transplantation in a patient with VACTERL syndrome who was found to have absent right common and external iliac arteries during pre-operative imaging. Vascular supply to the right lower limb is derived from an anomalous branch from the left internal iliac artery which takes on a convoluted course across the pelvis. Kidney transplantation was performed successfully with implantation performed on the left side. Isolated cases of congenital iliac artery atresia have been described in association with urological abnormalities but no clear association has yet been established. However, we feel that it may be useful to perform routine angiographic evaluation for ESRD patients with congenital genitourinary abnormalities being planned for kidney transplantation. While most cases of congenital iliac artery anomalies are symptomatic with claudication, some remain asymptomatic with normal physical examination findings. There is some evidence in literature suggesting the usefulness of routine pre-operative CT in a selective group of patients. Kidney transplantation in such cases is safe and we recommend routine pre-operative imaging of patients known to have congenital genitourniary abnormalities. The kidney should be implanted heterotopically to the contralateral side of the vascular anomaly and care must be taken to preserve vascular supply to the lower limbs. Copyright © 2015 The Authors. Published by Elsevier Ltd.. All rights reserved.

  16. Competition and convergence between auditory and cross-modal visual inputs to primary auditory cortical areas

    Science.gov (United States)

    Mao, Yu-Ting; Hua, Tian-Miao

    2011-01-01

    Sensory neocortex is capable of considerable plasticity after sensory deprivation or damage to input pathways, especially early in development. Although plasticity can often be restorative, sometimes novel, ectopic inputs invade the affected cortical area. Invading inputs from other sensory modalities may compromise the original function or even take over, imposing a new function and preventing recovery. Using ferrets whose retinal axons were rerouted into auditory thalamus at birth, we were able to examine the effect of varying the degree of ectopic, cross-modal input on reorganization of developing auditory cortex. In particular, we assayed whether the invading visual inputs and the existing auditory inputs competed for or shared postsynaptic targets and whether the convergence of input modalities would induce multisensory processing. We demonstrate that although the cross-modal inputs create new visual neurons in auditory cortex, some auditory processing remains. The degree of damage to auditory input to the medial geniculate nucleus was directly related to the proportion of visual neurons in auditory cortex, suggesting that the visual and residual auditory inputs compete for cortical territory. Visual neurons were not segregated from auditory neurons but shared target space even on individual target cells, substantially increasing the proportion of multisensory neurons. Thus spatial convergence of visual and auditory input modalities may be sufficient to expand multisensory representations. Together these findings argue that early, patterned visual activity does not drive segregation of visual and auditory afferents and suggest that auditory function might be compromised by converging visual inputs. These results indicate possible ways in which multisensory cortical areas may form during development and evolution. They also suggest that rehabilitative strategies designed to promote recovery of function after sensory deprivation or damage need to take into

  17. Brainstem auditory evoked potential testing in Dalmatian dogs in Brazil

    Directory of Open Access Journals (Sweden)

    M.I.P. Palumbo

    2014-04-01

    Full Text Available The brain stem auditory-evoked potential (BAEP is an electrophysiologic test that detects and records the electrical activity in the auditory system from cochlea to midbrain, generated after an acoustic stimulus applied to the external ear. The aim of this study is to obtain normative data for BAEP in Dalmatian dogs in order to apply this to the evaluation of deafness and other neurologic disorders. BAEP were recorded from 30 Dalmatian dogs for a normative Brazilian study. Mean latencies for waves I, III, and V were 1.14 (±0.09, 2.62 (±0.10, and 3.46 (±0.14 ms, respectively. Mean inter-peak latencies for I-III, III-V, and I-V intervals were 1.48 (±0.17, 0.84 (±0.12, and 2.31 (±0.18 ms, respectively. Unilateral abnormalities were found in 16.7% of animals and bilateral deafness was seen in one dog. The normative data obtained in this paper is compatible with other published data. As far as we know this is the first report of deafness occurrence in Dalmatian dogs in Brazil.

  18. Phantom auditory perception (tinnitus): mechanisms of generation and perception.

    Science.gov (United States)

    Jastreboff, P J

    1990-08-01

    Phantom auditory perception--tinnitus--is a symptom of many pathologies. Although there are a number of theories postulating certain mechanisms of its generation, none have been proven yet. This paper analyses the phenomenon of tinnitus from the point of view of general neurophysiology. Existing theories and their extrapolation are presented, together with some new potential mechanisms of tinnitus generation, encompassing the involvement of calcium and calcium channels in cochlear function, with implications for malfunction and aging of the auditory and vestibular systems. It is hypothesized that most tinnitus results from the perception of abnormal activity, defined as activity which cannot be induced by any combination of external sounds. Moreover, it is hypothesized that signal recognition and classification circuits, working on holographic or neuronal network-like representation, are involved in the perception of tinnitus and are subject to plastic modification. Furthermore, it is proposed that all levels of the nervous system, to varying degrees, are involved in tinnitus manifestation. These concepts are used to unravel the inexplicable, unique features of tinnitus and its masking. Some clinical implications of these theories are suggested.

  19. [Assessment of the efficiency of the auditory training in children with dyslalia and auditory processing disorders].

    Science.gov (United States)

    Włodarczyk, Elżbieta; Szkiełkowska, Agata; Skarżyński, Henryk; Piłka, Adam

    2011-01-01

    To assess effectiveness of the auditory training in children with dyslalia and central auditory processing disorders. Material consisted of 50 children aged 7-9-years-old. Children with articulation disorders stayed under long-term speech therapy care in the Auditory and Phoniatrics Clinic. All children were examined by a laryngologist and a phoniatrician. Assessment included tonal and impedance audiometry and speech therapists' and psychologist's consultations. Additionally, a set of electrophysiological examinations was performed - registration of N2, P2, N2, P2, P300 waves and psychoacoustic test of central auditory functions: FPT - frequency pattern test. Next children took part in the regular auditory training and attended speech therapy. Speech assessment followed treatment and therapy, again psychoacoustic tests were performed and P300 cortical potentials were recorded. After that statistical analyses were performed. Analyses revealed that application of auditory training in patients with dyslalia and other central auditory disorders is very efficient. Auditory training may be a very efficient therapy supporting speech therapy in children suffering from dyslalia coexisting with articulation and central auditory disorders and in children with educational problems of audiogenic origin. Copyright © 2011 Polish Otolaryngology Society. Published by Elsevier Urban & Partner (Poland). All rights reserved.

  20. Altered retrieval of melodic information in congenital amusia: insights from dynamic causal modeling of MEG data.

    Science.gov (United States)

    Albouy, Philippe; Mattout, Jérémie; Sanchez, Gaëtan; Tillmann, Barbara; Caclin, Anne

    2015-01-01

    Congenital amusia is a neuro-developmental disorder that primarily manifests as a difficulty in the perception and memory of pitch-based materials, including music. Recent findings have shown that the amusic brain exhibits altered functioning of a fronto-temporal network during pitch perception and short-term memory. Within this network, during the encoding of melodies, a decreased right backward frontal-to-temporal connectivity was reported in amusia, along with an abnormal connectivity within and between auditory cortices. The present study investigated whether connectivity patterns between these regions were affected during the short-term memory retrieval of melodies. Amusics and controls had to indicate whether sequences of six tones that were presented in pairs were the same or different. When melodies were different only one tone changed in the second melody. Brain responses to the changed tone in "Different" trials and to its equivalent (original) tone in "Same" trials were compared between groups using Dynamic Causal Modeling (DCM). DCM results confirmed that congenital amusia is characterized by an altered effective connectivity within and between the two auditory cortices during sound processing. Furthermore, right temporal-to-frontal message passing was altered in comparison to controls, with notably an increase in "Same" trials. An additional analysis in control participants emphasized that the detection of an unexpected event in the typically functioning brain is supported by right fronto-temporal connections. The results can be interpreted in a predictive coding framework as reflecting an abnormal prediction error sent by temporal auditory regions towards frontal areas in the amusic brain.

  1. Altered retrieval of melodic information in congenital amusia: Insights from Dynamic Causal Modeling of MEG data

    Directory of Open Access Journals (Sweden)

    Philippe eAlbouy

    2015-02-01

    Full Text Available Congenital amusia is a neuro-developmental disorder that primarily manifests as a difficulty in the perception and memory of pitch-based materials, including music. Recent findings have shown that the amusic brain exhibits altered functioning of a fronto-temporal network during pitch perception and memory. Within this network, during the encoding of melodies, a decreased right backward frontal-to-temporal connectivity was reported in amusia, along with an abnormal connectivity within and between auditory cortices. The present study investigated whether connectivity patterns between these regions were affected during the retrieval of melodies. Amusics and controls had to indicate whether sequences of six tones that were presented in pairs were the same or different. When melodies were different only one tone changed in the second melody. Brain responses to the changed tone in Different trials and to its equivalent (original tone in Same trials were compared between groups using Dynamic Causal Modeling (DCM. DCM results confirmed that congenital amusia is characterized by an altered effective connectivity within and between the two auditory cortices during sound processing. Furthermore, right temporal-to-frontal message passing was altered in comparison to controls, with an increase in Same trials and a decrease in Different trials. An additional analysis in control participants emphasized that the detection of an unexpected event in the typically functioning brain is supported by right fronto-temporal connections. The results can be interpreted in a predictive coding framework as reflecting an abnormal prediction error sent by temporal auditory regions towards frontal areas in the amusic brain.

  2. A Comparative Study of Simple Auditory Reaction Time in Blind (Congenitally) and Sighted Subjects

    OpenAIRE

    Gandhi, Pritesh Hariprasad; Gokhale, Pradnya A.; Mehta, H. B.; Shah, C. J.

    2013-01-01

    Background: Reaction time is the time interval between the application of a stimulus and the appearance of appropriate voluntary response by a subject. It involves stimulus processing, decision making, and response programming. Reaction time study has been popular due to their implication in sports physiology. Reaction time has been widely studied as its practical implications may be of great consequence e.g., a slower than normal reaction time while driving can have grave results. Objective:...

  3. Auditory Pattern Memory and Group Signal Detection

    National Research Council Canada - National Science Library

    Sorkin, Robert

    1997-01-01

    .... The experiments with temporally-coded auditory patterns showed how listeners' attention is influenced by the position and the amount of information carried by different segments of the pattern...

  4. Auditory Peripheral Processing of Degraded Speech

    National Research Council Canada - National Science Library

    Ghitza, Oded

    2003-01-01

    ...". The underlying thesis is that the auditory periphery contributes to the robust performance of humans in speech reception in noise through a concerted contribution of the efferent feedback system...

  5. Robotic Discovery of the Auditory Scene

    National Research Council Canada - National Science Library

    Martinson, E; Schultz, A

    2007-01-01

    .... Motivated by the large negative effect of ambient noise sources on robot audition, the long-term goal is to provide awareness of the auditory scene to a robot, so that it may more effectively act...

  6. Effect of omega-3 on auditory system

    Directory of Open Access Journals (Sweden)

    Vida Rahimi

    2014-01-01

    Full Text Available Background and Aim: Omega-3 fatty acid have structural and biological roles in the body 's various systems . Numerous studies have tried to research about it. Auditory system is affected a s well. The aim of this article was to review the researches about the effect of omega-3 on auditory system.Methods: We searched Medline , Google Scholar, PubMed, Cochrane Library and SID search engines with the "auditory" and "omega-3" keywords and read textbooks about this subject between 19 70 and 20 13.Conclusion: Both excess and deficient amounts of dietary omega-3 fatty acid can cause harmful effects on fetal and infant growth and development of brain and central nervous system esspesially auditory system. It is important to determine the adequate dosage of omega-3.

  7. Presbycusis and auditory brainstem responses: a review

    Directory of Open Access Journals (Sweden)

    Shilpa Khullar

    2011-06-01

    Full Text Available Age-related hearing loss or presbycusis is a complex phenomenon consisting of elevation of hearing levels as well as changes in the auditory processing. It is commonly classified into four categories depending on the cause. Auditory brainstem responses (ABRs are a type of early evoked potentials recorded within the first 10 ms of stimulation. They represent the synchronized activity of the auditory nerve and the brainstem. Some of the changes that occur in the aging auditory system may significantly influence the interpretation of the ABRs in comparison with the ABRs of the young adults. The waves of ABRs are described in terms of amplitude, latencies and interpeak latency of the different waves. There is a tendency of the amplitude to decrease and the absolute latencies to increase with advancing age but these trends are not always clear due to increase in threshold with advancing age that act a major confounding factor in the interpretation of ABRs.

  8. Auditory motion capturing ambiguous visual motion

    Directory of Open Access Journals (Sweden)

    Arjen eAlink

    2012-01-01

    Full Text Available In this study, it is demonstrated that moving sounds have an effect on the direction in which one sees visual stimuli move. During the main experiment sounds were presented consecutively at four speaker locations inducing left- or rightwards auditory apparent motion. On the path of auditory apparent motion, visual apparent motion stimuli were presented with a high degree of directional ambiguity. The main outcome of this experiment is that our participants perceived visual apparent motion stimuli that were ambiguous (equally likely to be perceived as moving left- or rightwards more often as moving in the same direction than in the opposite direction of auditory apparent motion. During the control experiment we replicated this finding and found no effect of sound motion direction on eye movements. This indicates that auditory motion can capture our visual motion percept when visual motion direction is insufficiently determinate without affecting eye movements.

  9. Auditory-vocal mirroring in songbirds.

    Science.gov (United States)

    Mooney, Richard

    2014-01-01

    Mirror neurons are theorized to serve as a neural substrate for spoken language in humans, but the existence and functions of auditory-vocal mirror neurons in the human brain remain largely matters of speculation. Songbirds resemble humans in their capacity for vocal learning and depend on their learned songs to facilitate courtship and individual recognition. Recent neurophysiological studies have detected putative auditory-vocal mirror neurons in a sensorimotor region of the songbird's brain that plays an important role in expressive and receptive aspects of vocal communication. This review discusses the auditory and motor-related properties of these cells, considers their potential role on song learning and communication in relation to classical studies of birdsong, and points to the circuit and developmental mechanisms that may give rise to auditory-vocal mirroring in the songbird's brain.

  10. Environment for Auditory Research Facility (EAR)

    Data.gov (United States)

    Federal Laboratory Consortium — EAR is an auditory perception and communication research center enabling state-of-the-art simulation of various indoor and outdoor acoustic environments. The heart...

  11. Deficits of congenital amusia beyond pitch: Evidence from impaired categorical perception of vowels in Cantonese-speaking congenital amusics.

    Science.gov (United States)

    Zhang, Caicai; Shao, Jing; Huang, Xunan

    2017-01-01

    Congenital amusia is a lifelong disorder of fine-grained pitch processing in music and speech. However, it remains unclear whether amusia is a pitch-specific deficit, or whether it affects frequency/spectral processing more broadly, such as the perception of formant frequency in vowels, apart from pitch. In this study, in order to illuminate the scope of the deficits, we compared the performance of 15 Cantonese-speaking amusics and 15 matched controls on the categorical perception of sound continua in four stimulus contexts: lexical tone, pure tone, vowel, and voice onset time (VOT). Whereas lexical tone, pure tone and vowel continua rely on frequency/spectral processing, the VOT continuum depends on duration/temporal processing. We found that the amusic participants performed similarly to controls in all stimulus contexts in the identification, in terms of the across-category boundary location and boundary width. However, the amusic participants performed systematically worse than controls in discriminating stimuli in those three contexts that depended on frequency/spectral processing (lexical tone, pure tone and vowel), whereas they performed normally when discriminating duration differences (VOT). These findings suggest that the deficit of amusia is probably not pitch specific, but affects frequency/spectral processing more broadly. Furthermore, there appeared to be differences in the impairment of frequency/spectral discrimination in speech and nonspeech contexts. The amusic participants exhibited less benefit in between-category discriminations than controls in speech contexts (lexical tone and vowel), suggesting reduced categorical perception; on the other hand, they performed inferiorly compared to controls across the board regardless of between- and within-category discriminations in nonspeech contexts (pure tone), suggesting impaired general auditory processing. These differences imply that the frequency/spectral-processing deficit might be manifested

  12. In search of an auditory engram

    OpenAIRE

    Fritz, Jonathan; Mishkin, Mortimer; Saunders, Richard C.

    2005-01-01

    Monkeys trained preoperatively on a task designed to assess auditory recognition memory were impaired after removal of either the rostral superior temporal gyrus or the medial temporal lobe but were unaffected by lesions of the rhinal cortex. Behavioral analysis indicated that this result occurred because the monkeys did not or could not use long-term auditory recognition, and so depended instead on short-term working memory, which is unaffected by rhinal lesions. The findings suggest that mo...

  13. Neural oscillations in auditory working memory

    OpenAIRE

    Wilsch, A.

    2015-01-01

    The present thesis investigated memory load and memory decay in auditory working memory. Alpha power as a marker for memory load served as the primary indicator for load and decay fluctuations hypothetically reflecting functional inhibition of irrelevant information. Memory load was induced by presenting auditory signals (syllables and pure-tone sequences) in noise because speech-in-noise has been shown before to increase memory load. The aim of the thesis was to assess with magnetoencephalog...

  14. Perceptual consequences of disrupted auditory nerve activity.

    Science.gov (United States)

    Zeng, Fan-Gang; Kong, Ying-Yee; Michalewski, Henry J; Starr, Arnold

    2005-06-01

    Perceptual consequences of disrupted auditory nerve activity were systematically studied in 21 subjects who had been clinically diagnosed with auditory neuropathy (AN), a recently defined disorder characterized by normal outer hair cell function but disrupted auditory nerve function. Neurological and electrophysical evidence suggests that disrupted auditory nerve activity is due to desynchronized or reduced neural activity or both. Psychophysical measures showed that the disrupted neural activity has minimal effects on intensity-related perception, such as loudness discrimination, pitch discrimination at high frequencies, and sound localization using interaural level differences. In contrast, the disrupted neural activity significantly impairs timing related perception, such as pitch discrimination at low frequencies, temporal integration, gap detection, temporal modulation detection, backward and forward masking, signal detection in noise, binaural beats, and sound localization using interaural time differences. These perceptual consequences are the opposite of what is typically observed in cochlear-impaired subjects who have impaired intensity perception but relatively normal temporal processing after taking their impaired intensity perception into account. These differences in perceptual consequences between auditory neuropathy and cochlear damage suggest the use of different neural codes in auditory perception: a suboptimal spike count code for intensity processing, a synchronized spike code for temporal processing, and a duplex code for frequency processing. We also proposed two underlying physiological models based on desynchronized and reduced discharge in the auditory nerve to successfully account for the observed neurological and behavioral data. These methods and measures cannot differentiate between these two AN models, but future studies using electric stimulation of the auditory nerve via a cochlear implant might. These results not only show the unique

  15. ExternE: Externalities of energy Vol. 1. Summary

    International Nuclear Information System (INIS)

    Holland, M.; Berry, J.

    1995-01-01

    There is a growing requirement for policy analysts to take account of the environment in their decision making and to undertake the specified cost-benefit analysis. Within the European Union this is reflected in the 5th Environmental Action Programme, and the Commission's White Paper entitled 'Growth, competitiveness, employment and the ways forward to the 21st century'. This has led to a need for evaluation of environmental externalities. The ExternE Project commenced in 1991 as the European part of a collaborative study between the European Commission and the US Department of Energy. It aims to be the first systematic approach to the evaluation of external costs of a wide range of different fuel cycles. The project will result in an operational accounting framework for the quantification and monetarisation of priority environmental and other externalities. This framework will allow the calculation of the marginal external costs and benefits for specific power plants, at specific sites using specified technologies. There are three major phases in the project. Phase 1 was undertaken in collaboration with the US Department of Energy. In this phase the teams jointly developed the conceptual approach and methodology and shared scientific information for application to a number of fuel cycles. On the European side work concentrated on the nuclear and coal fuel cycles which together were expected to raise many of the fundamental issues in fuel cycle analysis. The project is currently nearing completion of Phase 2. During this phase the methodology has been applied to a wide range of different fossil, nuclear and renewable fuel cycles for power generation and energy conservation options. Also a series of National Implementation Programmes is underway in which the methodology and accounting framework are being applied to reference sites throughout Europe. In addition the general methodology is being extended to address the evaluation of externalities associated with

  16. Externalities of fuel cycles 'ExternE' project. Summary report

    International Nuclear Information System (INIS)

    Holland, M.; Berry, J.

    1994-01-01

    There is a growing requirement for policy analysts to take account of the environment in their decision making and to undertake the specified cost-benefit analysis. Within the European Union this is reflected in the 5th Environmental Action Programme, and the Commission's White Paper entitled 'Growth, competitiveness, employment and the ways forward to the 21st century'. This has led to a need for evaluation of environmental externalities. The ExternE Project commenced in 1991 as the European part of a collaborative study between the European Commission and the US Department of Energy. It aims to be the first systematic approach to the evaluation of external costs of a wide range of different fuel cycles. The project will result in an operational accounting framework for the quantification and monetarisation of priority environmental and other externalities. This framework will allow the calculation of the marginal external costs and benefits for specific power plants, at specific sites using specified technologies. There are three major phases in the project. Phase I was undertaken in collaboration with the US Department of Energy. In this phase the teams jointly developed the conceptual approach and methodology and shared scientific information for application to a number of fuel cycles. On the European side work concentrated on the nuclear and coal fuel cycles which together were expected to raise many of the fundamental issues in fuel cycle analysis. The project is currently nearing completion of Phase 2. During this phase the methodology has been applied to a wide range of different fossil, nuclear and renewable fuel cycles for power generation and energy conservation options. Also a series of National Implementation Programmes are underway in which the methodology and accounting framework are being applied to reference sites throughout Europe. In addition the general methodology is being extended to address the evaluation of externalities associated with

  17. Adrenomyeloneuropathy associated with congenital cataract

    International Nuclear Information System (INIS)

    Komori, Tetsuo; Nagashima, Toshiko; Hirose, Kazuhiko; Tanabe, Hitoshi; Tsubaki, Tadao

    1988-01-01

    Two cases of adrenomyeloneuropathy (AMN) in a family and the results of their MRI study are reported. Case 1, a 24-year-old male proband, was admitted to our hospital because of gait disturbance for three years. Bilateral cataracts were pointed out at birth, which required left side lenticotomy at age four. Neurological examinations on admission revealed a marked spastic paraparesis with pathological reflexes and a mild hypesthesia in the distal part of the left leg. No abnormal findings were detected in X-ray studies on the spine and spinal cord, electromyography and nerve conduction test. Serum very long chain fatty acids (VLCFAs) levels were apparently elevated, and the serum cortisol response to ACTH was low. Case 2, a 53-year-old woman, is the mother of Case 1 having a complaint of gait disturbance since age 51. She presented a mild spastic paraparesis with localized hypesthesia in the distal part of the both legs. She also had bilateral congenital cataracts. Her serum VLCFAs analysis indicated the intermediate levels between that of AMN and the normal control. Adrenal functions were normal. Cranial MRI (TR 2,000 msec/TE 80 msec) study disclosed high signal intensity areas in bilateral internal capsules in Case 1. These findings, suggesting the pathological change of dysmyelination, seemed to be well compatible with the clinical pictures. With these clinical findings and the laboratory data, these two cases were diagnosed as AMN. In addition, association of congenital cataract with AMN in both cases was characteristic in this family, which hasn't been reported in the literature. On regarding the genetic background of these two disorders, AMN and congenital cataract, it was speculated that each gene could be closely located on the same or very adjacent locus, possibly on Xq. (author)

  18. Procedures for central auditory processing screening in schoolchildren.

    Science.gov (United States)

    Carvalho, Nádia Giulian de; Ubiali, Thalita; Amaral, Maria Isabel Ramos do; Santos, Maria Francisca Colella

    2018-03-22

    Central auditory processing screening in schoolchildren has led to debates in literature, both regarding the protocol to be used and the importance of actions aimed at prevention and promotion of auditory health. Defining effective screening procedures for central auditory processing is a challenge in Audiology. This study aimed to analyze the scientific research on central auditory processing screening and discuss the effectiveness of the procedures utilized. A search was performed in the SciELO and PUBMed databases by two researchers. The descriptors used in Portuguese and English were: auditory processing, screening, hearing, auditory perception, children, auditory tests and their respective terms in Portuguese. original articles involving schoolchildren, auditory screening of central auditory skills and articles in Portuguese or English. studies with adult and/or neonatal populations, peripheral auditory screening only, and duplicate articles. After applying the described criteria, 11 articles were included. At the international level, central auditory processing screening methods used were: screening test for auditory processing disorder and its revised version, screening test for auditory processing, scale of auditory behaviors, children's auditory performance scale and Feather Squadron. In the Brazilian scenario, the procedures used were the simplified auditory processing assessment and Zaidan's battery of tests. At the international level, the screening test for auditory processing and Feather Squadron batteries stand out as the most comprehensive evaluation of hearing skills. At the national level, there is a paucity of studies that use methods evaluating more than four skills, and are normalized by age group. The use of simplified auditory processing assessment and questionnaires can be complementary in the search for an easy access and low-cost alternative in the auditory screening of Brazilian schoolchildren. Interactive tools should be proposed, that

  19. Speech Evoked Auditory Brainstem Response in Stuttering

    Directory of Open Access Journals (Sweden)

    Ali Akbar Tahaei

    2014-01-01

    Full Text Available Auditory processing deficits have been hypothesized as an underlying mechanism for stuttering. Previous studies have demonstrated abnormal responses in subjects with persistent developmental stuttering (PDS at the higher level of the central auditory system using speech stimuli. Recently, the potential usefulness of speech evoked auditory brainstem responses in central auditory processing disorders has been emphasized. The current study used the speech evoked ABR to investigate the hypothesis that subjects with PDS have specific auditory perceptual dysfunction. Objectives. To determine whether brainstem responses to speech stimuli differ between PDS subjects and normal fluent speakers. Methods. Twenty-five subjects with PDS participated in this study. The speech-ABRs were elicited by the 5-formant synthesized syllable/da/, with duration of 40 ms. Results. There were significant group differences for the onset and offset transient peaks. Subjects with PDS had longer latencies for the onset and offset peaks relative to the control group. Conclusions. Subjects with PDS showed a deficient neural timing in the early stages of the auditory pathway consistent with temporal processing deficits and their abnormal timing may underlie to their disfluency.

  20. Surgery for congenital choanal atresia.

    Science.gov (United States)

    Cedin, Antonio C; Atallah, Alvaro N; Andriolo, Régis B; Cruz, Oswaldo L; Pignatari, Shirley N

    2012-02-15

    Congenital choanal atresia is a rare abnormality characterized by unilateral or bilateral lack of patency of the posterior end of the nasal cavity. With an incidence of 1:5000 to 1:8000 births, it is twice as prevalent in females as it is in males. Surgical procedures aim to provide adequate functional choanal patency and a low rate of restenosis, avoid harm to any structure in development, enable shorter surgery and hospitalization times, and minimize morbidity and mortality. To evaluate the effectiveness and safety of the available surgical techniques for the treatment of congenital choanal atresia in patients with unilateral and bilateral atresia. We searched the Cochrane Ear, Nose and Throat Disorders Group Trials Register; the Cochrane Central Register of Controlled Trials (CENTRAL); PubMed; EMBASE; CINAHL; Web of Science; BIOSIS Previews; Cambridge Scientific Abstracts; ISRCTN and additional sources for published and unpublished trials. The date of the search was 31 January 2011. We planned to include parallel randomized or quasi-randomized controlled trials testing surgical approaches for the treatment of congenital atresia (irrespective of gender and age) that evaluated normal/adequate respiratory function (self reported or preserved nasal airway) and restenosis as the main primary outcomes. We did not consider reoperation and non-congenital atresia (e.g. traumatic, iatrogenic atresias) for inclusion. Three review authors independently assessed the titles and abstracts of the identified articles to determine potential relevance. For dichotomous and continuous variables, we planned to calculate risk ratios (relative risks; RR) and mean differences (MD) with 95% confidence intervals (CI), respectively. We planned to use the random-effects model since we were expecting substantial clinical and methodological heterogeneity. No randomized controlled trials were identified. From the 120 reports retrieved using our search strategy, 46 primary studies had the

  1. Laboratory Diagnosis of Congenital Toxoplasmosis

    Science.gov (United States)

    Pomares, Christelle

    2016-01-01

    Recent studies have demonstrated that screening and treatment for toxoplasmosis during gestation result in a decrease of vertical transmission and clinical sequelae. Early treatment was associated with improved outcomes. Thus, laboratory methods should aim for early identification of infants with congenital toxoplasmosis (CT). Diagnostic approaches should include, at least, detection of Toxoplasma IgG, IgM, and IgA and a comprehensive review of maternal history, including the gestational age at which the mother was infected and treatment. Here, we review laboratory methods for the diagnosis of CT, with emphasis on serological tools. A diagnostic algorithm that takes into account maternal history is presented. PMID:27147724

  2. Schizencephaly/congenital cerebral clefts

    International Nuclear Information System (INIS)

    Friedman, H.; Naidich, T.P.

    1987-01-01

    Schizencephaly (from the Greek meaning ''split brain''), is a term developed in the 1940s to explain symmetric clefts in the brain seen at autopsy in children with histories of severe neurologic defects. Use of the term has been expanded to include a variety of cerebral clefts. A review of the experience at Children's Memorial Hospital as well as case materials made available to the authors are presented, including CT, MR imaging, and US findings. Theories of etiology and pathogenesis of these congenital clefts, associated anomalies, and the spectrum of appearance of these clefts are discussed

  3. Emergence of antiviral resistance during oral valganciclovir treatment of an infant with congenital cytomegalovirus (CMV) infection.

    Science.gov (United States)

    Choi, K Yeon; Sharon, B; Balfour, H H; Belani, K; Pozos, T C; Schleiss, M R

    2013-08-01

    Congenital infection with human cytomegalovirus (CMV) is a major cause of morbidity, including sensorineural hearing loss (SNHL), in newborns. Antiviral therapy with ganciclovir (GCV) and its oral prodrug, valganciclovir (VAL-GCV) are increasingly being administered to infected infants, toward the goal of improving neurodevelopmental and auditory outcomes. In this case report, we describe a symptomatic congenitally infected infant treated with VAL-GCV in whom GCV resistance was suspected, based on a 50-fold increase in viral load after 6 weeks of oral therapy. Analyses of CMV sequences from both blood and urine demonstrated populations of viruses with M460V and L595F mutations in the UL97 phosphotransferase gene. In contrast, analysis of viral DNA retrieved from the newborn dried blood spot demonstrated wild-type UL97 sequences. DNAemia resolved after the discontinuation of VAL-GCV. Long-term VAL-GCV therapy in congenitally infected infants can select for resistant viral variants, and anticipatory virological monitoring may be warranted. Copyright © 2013 Elsevier B.V. All rights reserved.

  4. Inner ear anomalies causing congenital sensorineural hearing loss: CT and MR imaging findings

    International Nuclear Information System (INIS)

    Hong, Hyun Sook; Paik, Sang Hyun; Cha, Jang Gyu; Park, Seong Jin; Joh, Joon Hee; Park, Jai Soung; Kim, Dae Ho; Lee, Hae Kyung; Kim, Shi Chan

    2005-01-01

    Many congenital dysplasias of the osseous labyrinth have been identified, and the differential diagnosis of these dysplasias is essential for delivering proper patient management. We retrospectively reviewed the computed tomography (CT) and magnetic resonance (MR) imaging findings of 20 children who had congenital sensorineural hearing loss. The children included cases of enlarged vestibular aqueduct and endolymphatic sac (n=8), aplasia of the semicircular canal (n=4), lateral semicircular canal-vestibule dysplasia (n=3), common cavity malformations with a large vestibule (n=1), cochlear hypoplasia (n=1), Mondini's dysplasia with large vestibular aqueduct (n=1), Mondini's dysplasia with a large vestibule (n=1), and small internal auditory canal (n=1). Six cases were unilateral. Nine cases had combined deformities, and nine cased had cochlear implants. CT was performed with a 1.0-mm thickness in the direct coronal and axial sections with using bone algorithms. MR was performed with a temporal 3D T2 FSE 10-mm scan and with routine brain images. We describe here the imaging features for the anomalies of the inner ear in patients suffering from congenital sensorineural hearing loss

  5. FREQUENCY OF MALASSEZIA SPP. IN DOGS PRESENTING EXTERNAL OTITIS

    Directory of Open Access Journals (Sweden)

    N. R. Magalhães

    2017-12-01

    Full Text Available The external otitis is defined as an inflammation of the dog external ear canal and it is considered a common disease in dogs. It is a disease of multifactorial etiology, where one of the main microrganisms associated to the illness is the Malassezia ssp. Therefore, this work aimed to determine the frequency of Malassezia spp. by auricular cytology in dogs that present clinical signs of external otitis. Were used 23 dogs attended in a veterinary clinic located in Sinop-MT. The material was collected using a dry swab, where each extremity was inserted in one of the auditory canal (right and left, which was rotated, removed from the ear and rolled on the glass slide. First the glass slide was microscopically observed (objective 4X, for viewing mites, and then, the sample was fixed by heat and stained with Panoptic. Once stained, the glass slide was examined (objective 40X and 100X. Among the 23 dogs evaluated, 60.9% were positive for Malassezia spp., 8.7% were positive for the Otodectes cynotis mite, and also 30,4% of the animals showed mixed infection with Malassezia ssp. and bacteria. With this study, it can be concluded that Malassezia ssp. was found more frequently in at the auditory canal of dogs with that presents external otitis, associated or acting alone, indicating the importance of this yeast in cases of otitis.

  6. Genetically caused congenital anomalies of reproductive system

    Directory of Open Access Journals (Sweden)

    L. F. Kurilo

    2013-01-01

    Full Text Available Classification of congenital disorders, their frequency of occurrence in populations, and some terminology questions discussed in the review. Genetically caused congenital anomalies of reproductive system are outlined. Full information about genetic syndromes is stated in the book: Kozlova S.I., Demikova N.S. Hereditary syndromes and genetic counseling. M., 2007.

  7. Congenital segmental dilatation of the colon

    African Journals Online (AJOL)

    Congenital segmental dilatation of the colon is a rare cause of intestinal obstruction in neonates. We report a case of congenital segmental dilatation of the colon and highlight the clinical, radiological, and histopathological features of this entity. Proper surgical treatment was initiated on the basis of preoperative radiological ...

  8. Congenital Heart Diseases associated with Identified Syndromes ...

    African Journals Online (AJOL)

    Recognised syndromes were seen in 69(68%) cases. Down syndrome with 54 children contributed 78.3% of those with known syndromes. Other identified syndromes and associations were Marfan's, Noonan's, Edwards, Prune Belly, Apert, Ellis-van creveld syndrome and congenital rubella syndrome. Congenital heart ...

  9. The genetic landscape of familial congenital hydrocephalus.

    Science.gov (United States)

    Shaheen, Ranad; Sebai, Mohammed Adeeb; Patel, Nisha; Ewida, Nour; Kurdi, Wesam; Altweijri, Ikhlass; Sogaty, Sameera; Almardawi, Elham; Seidahmed, Mohammed Zain; Alnemri, Abdulrahman; Madirevula, Sateesh; Ibrahim, Niema; Abdulwahab, Firdous; Hashem, Mais; Al-Sheddi, Tarfa; Alomar, Rana; Alobeid, Eman; Sallout, Bahauddin; AlBaqawi, Badi; AlAali, Wajeih; Ajaji, Nouf; Lesmana, Harry; Hopkin, Robert J; Dupuis, Lucie; Mendoza-Londono, Roberto; Al Rukban, Hadeel; Yoon, Grace; Faqeih, Eissa; Alkuraya, Fowzan S

    2017-06-01

    Congenital hydrocephalus is an important birth defect, the genetics of which remains incompletely understood. To date, only 4 genes are known to cause Mendelian diseases in which congenital hydrocephalus is the main or sole clinical feature, 2 X-linked (L1CAM and AP1S2) and 2 autosomal recessive (CCDC88C and MPDZ). In this study, we aimed to determine the genetic etiology of familial congenital hydrocephalus with the assumption that these cases represent Mendelian forms of the disease. Exome sequencing combined, where applicable, with positional mapping. We identified a likely causal mutation in the majority of these families (21 of 27, 78%), spanning 16 genes, none of which is X-linked. Ciliopathies and dystroglycanopathies were the most common etiologies of congenital hydrocephalus in our cohort (19% and 26%, respectively). In 1 family with 4 affected members, we identified a homozygous truncating variant in EML1, which we propose as a novel cause of congenital hydrocephalus in addition to its suggested role in cortical malformation. Similarly, we show that recessive mutations in WDR81, previously linked to cerebellar ataxia, mental retardation, and disequilibrium syndrome 2, cause severe congenital hydrocephalus. Furthermore, we confirm the previously reported candidacy of MPDZ by presenting a phenotypic spectrum of congenital hydrocephalus associated with 5 recessive alleles. Our study highlights the importance of recessive mutations in familial congenital hydrocephalus and expands the locus heterogeneity of this condition. Ann Neurol 2017;81:890-897. © 2017 American Neurological Association.

  10. Congenital lobar emphysema: Is surgery routinely necessary ...

    African Journals Online (AJOL)

    Congenital lobar emphysema (CLE) is a rare congenital abnormality characterised by overinflation of a pulmonary lobe. Its aetiology is unknown. The management of CLE has traditionally been surgical. A newborn boy with a birthweight of 2.5 kg was delivered at full-term by caesarian section due to food delivery.

  11. Congenital Diverticular Disease of the Entire Colon

    Directory of Open Access Journals (Sweden)

    A. Patel

    2013-01-01

    Full Text Available Congenital or true colonic diverticulosis is a rare condition typified by the preservation of the colonic wall architecture within the diverticular outpouching. Cases of multiple jejunal diverticula have been reported as well as cases of solitary giant diverticula of the colon. There have been no reports in the literature of pancolonic congenital diverticulosis.

  12. Congenital malformations in paediatric and neurosurgical practices ...

    African Journals Online (AJOL)

    Congenital malformations in paediatric and neurosurgical practices: problems and pattern (A preliminary report) ... Open Access DOWNLOAD FULL TEXT ... over a 5-year period (1998 to 2002) with congenital anomalies to the Paediatric Surgery and Neurosurgery units of the University Teaching Hospital, Ilorin, Nigeria.

  13. Cyanotic congenital heart disease and atherosclerosis

    DEFF Research Database (Denmark)

    Tarp, Julie Bjerre; Jensen, Annette Schophuus; Engstrøm, Thomas

    2017-01-01

    Improved treatment options in paediatric cardiology and congenital heart surgery have resulted in an ageing population of patients with cyanotic congenital heart disease (CCHD). The risk of acquired heart disease such as atherosclerosis increases with age.Previous studies have speculated whether...

  14. Genetics Home Reference: congenital central hypoventilation syndrome

    Science.gov (United States)

    ... Kravis EM, Zhou L, Rand CM, Weese-Mayer DE. Congenital central hypoventilation syndrome: PHOX2B mutations and phenotype. Am J Respir Crit ... BA, Leurgans SE, Berry-Kravis EM, Weese-Mayer DE. Congenital central hypoventilation syndrome: PHOX2B genotype determines risk for sudden death. Pediatr ...

  15. The changing epidemiology of congenital heart disease

    NARCIS (Netherlands)

    van der Bom, Teun; Zomer, A. Carla; Zwinderman, Aeilko H.; Meijboom, Folkert J.; Bouma, Berto J.; Mulder, Barbara J. M.

    2011-01-01

    Congenital heart disease is the most common congenital disorder in newborns. Advances in cardiovascular medicine and surgery have enabled most patients to reach adulthood. Unfortunately, prolonged survival has been achieved at a cost, as many patients suffer late complications, of which heart

  16. Health in adults with congenital heart disease

    NARCIS (Netherlands)

    Cuypers, Judith A. A. E.; Utens, Elisabeth M. W. J.; Roos-Hesselink, Jolien W.

    2016-01-01

    Since the introduction of cardiac surgery, the prospects for children born with a cardiac defect have improved spectacularly. Many reach adulthood and the population of adults with congenital heart disease is increasing and ageing. However, repair of congenital heart disease does not mean cure. Many

  17. External radiation surveillance

    International Nuclear Information System (INIS)

    Antonio, E.J.

    1995-01-01

    This section of the 1994 Hanford Site Environmental Report describes how external radiation was measured, how surveys were performed, and the results of these measurements and surveys. External radiation exposure rates were measured at locations on and off the Hanford Site using thermoluminescent dosimeters (TLD). External radiation and contamination surveys were also performed with portable radiation survey instruments at locations on and around the Hanford Site

  18. External radiation surveillance

    Energy Technology Data Exchange (ETDEWEB)

    Antonio, E.J.

    1995-06-01

    This section of the 1994 Hanford Site Environmental Report describes how external radiation was measured, how surveys were performed, and the results of these measurements and surveys. External radiation exposure rates were measured at locations on and off the Hanford Site using thermoluminescent dosimeters (TLD). External radiation and contamination surveys were also performed with portable radiation survey instruments at locations on and around the Hanford Site.

  19. Health in adults with congenital heart disease.

    Science.gov (United States)

    Cuypers, Judith A A E; Utens, Elisabeth M W J; Roos-Hesselink, Jolien W

    2016-09-01

    Since the introduction of cardiac surgery, the prospects for children born with a cardiac defect have improved spectacularly. Many reach adulthood and the population of adults with congenital heart disease is increasing and ageing. However, repair of congenital heart disease does not mean cure. Many adults with congenital heart disease encounter late complications. Late morbidity can be related to the congenital heart defect itself, but may also be the consequence of the surgical or medical treatment or longstanding alterations in hemodynamics, neurodevelopment and psychosocial development. This narrative review describes the cardiac and non-cardiac long-term morbidity in the adult population with congenital heart disease. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  20. Laboratory diagnosis of the rare anaemias: external quality assessment benefits patient care

    OpenAIRE

    Barbara De La Salle; Andrea Mosca; Renata Paleari; Vasileios Rapanakis; Keith Hyde

    2013-01-01

    Since its introduction in the 1960s, external quality assessment has developed to become an essential component of the quality management system of the diagnostic laboratory. External quality assessment provides a long term, retrospective view of laboratory performance, demonstrating the competence of the laboratory to others. The ENERCA project (the European Network for Rare and Congenital Anaemias) has established a list of core laboratory tests that are used in the diagnosis of rare and co...

  1. Effects of scanner acoustic noise on intrinsic brain activity during auditory stimulation.

    Science.gov (United States)

    Yakunina, Natalia; Kang, Eun Kyoung; Kim, Tae Su; Min, Ji-Hoon; Kim, Sam Soo; Nam, Eui-Cheol

    2015-10-01

    Although the effects of scanner background noise (SBN) during functional magnetic resonance imaging (fMRI) have been extensively investigated for the brain regions involved in auditory processing, its impact on other types of intrinsic brain activity has largely been neglected. The present study evaluated the influence of SBN on a number of intrinsic connectivity networks (ICNs) during auditory stimulation by comparing the results obtained using sparse temporal acquisition (STA) with those using continuous acquisition (CA). Fourteen healthy subjects were presented with classical music pieces in a block paradigm during two sessions of STA and CA. A volume-matched CA dataset (CAm) was generated by subsampling the CA dataset to temporally match it with the STA data. Independent component analysis was performed on the concatenated STA-CAm datasets, and voxel data, time courses, power spectra, and functional connectivity were compared. The ICA revealed 19 ICNs; the auditory, default mode, salience, and frontoparietal networks showed greater activity in the STA. The spectral peaks in 17 networks corresponded to the stimulation cycles in the STA, while only five networks displayed this correspondence in the CA. The dorsal default mode and salience networks exhibited stronger correlations with the stimulus waveform in the STA. SBN appeared to influence not only the areas of auditory response but also the majority of other ICNs, including attention and sensory networks. Therefore, SBN should be regarded as a serious nuisance factor during fMRI studies investigating intrinsic brain activity under external stimulation or task loads.

  2. Effects of scanner acoustic noise on intrinsic brain activity during auditory stimulation

    Energy Technology Data Exchange (ETDEWEB)

    Yakunina, Natalia [Kangwon National University, Institute of Medical Science, School of Medicine, Chuncheon (Korea, Republic of); Kangwon National University Hospital, Neuroscience Research Institute, Chuncheon (Korea, Republic of); Kang, Eun Kyoung [Kangwon National University Hospital, Department of Rehabilitation Medicine, Chuncheon (Korea, Republic of); Kim, Tae Su [Kangwon National University Hospital, Department of Otolaryngology, Chuncheon (Korea, Republic of); Kangwon National University, School of Medicine, Department of Otolaryngology, Chuncheon (Korea, Republic of); Min, Ji-Hoon [University of Michigan, Department of Biopsychology, Cognition, and Neuroscience, Ann Arbor, MI (United States); Kim, Sam Soo [Kangwon National University Hospital, Neuroscience Research Institute, Chuncheon (Korea, Republic of); Kangwon National University, School of Medicine, Department of Radiology, Chuncheon (Korea, Republic of); Nam, Eui-Cheol [Kangwon National University Hospital, Neuroscience Research Institute, Chuncheon (Korea, Republic of); Kangwon National University, School of Medicine, Department of Otolaryngology, Chuncheon (Korea, Republic of)

    2015-10-15

    Although the effects of scanner background noise (SBN) during functional magnetic resonance imaging (fMRI) have been extensively investigated for the brain regions involved in auditory processing, its impact on other types of intrinsic brain activity has largely been neglected. The present study evaluated the influence of SBN on a number of intrinsic connectivity networks (ICNs) during auditory stimulation by comparing the results obtained using sparse temporal acquisition (STA) with those using continuous acquisition (CA). Fourteen healthy subjects were presented with classical music pieces in a block paradigm during two sessions of STA and CA. A volume-matched CA dataset (CAm) was generated by subsampling the CA dataset to temporally match it with the STA data. Independent component analysis was performed on the concatenated STA-CAm datasets, and voxel data, time courses, power spectra, and functional connectivity were compared. The ICA revealed 19 ICNs; the auditory, default mode, salience, and frontoparietal networks showed greater activity in the STA. The spectral peaks in 17 networks corresponded to the stimulation cycles in the STA, while only five networks displayed this correspondence in the CA. The dorsal default mode and salience networks exhibited stronger correlations with the stimulus waveform in the STA. SBN appeared to influence not only the areas of auditory response but also the majority of other ICNs, including attention and sensory networks. Therefore, SBN should be regarded as a serious nuisance factor during fMRI studies investigating intrinsic brain activity under external stimulation or task loads. (orig.)

  3. Auditory and visual interactions between the superior and inferior colliculi in the ferret.

    Science.gov (United States)

    Stitt, Iain; Galindo-Leon, Edgar; Pieper, Florian; Hollensteiner, Karl J; Engler, Gerhard; Engel, Andreas K

    2015-05-01

    The integration of visual and auditory spatial information is important for building an accurate perception of the external world, but the fundamental mechanisms governing such audiovisual interaction have only partially been resolved. The earliest interface between auditory and visual processing pathways is in the midbrain, where the superior (SC) and inferior colliculi (IC) are reciprocally connected in an audiovisual loop. Here, we investigate the mechanisms of audiovisual interaction in the midbrain by recording neural signals from the SC and IC simultaneously in anesthetized ferrets. Visual stimuli reliably produced band-limited phase locking of IC local field potentials (LFPs) in two distinct frequency bands: 6-10 and 15-30 Hz. These visual LFP responses co-localized with robust auditory responses that were characteristic of the IC. Imaginary coherence analysis confirmed that visual responses in the IC were not volume-conducted signals from the neighboring SC. Visual responses in the IC occurred later than retinally driven superficial SC layers and earlier than deep SC layers that receive indirect visual inputs, suggesting that retinal inputs do not drive visually evoked responses in the IC. In addition, SC and IC recording sites with overlapping visual spatial receptive fields displayed stronger functional connectivity than sites with separate receptive fields, indicating that visual spatial maps are aligned across both midbrain structures. Reciprocal coupling between the IC and SC therefore probably serves the dynamic integration of visual and auditory representations of space. © 2015 Federation of European Neuroscience Societies and John Wiley & Sons Ltd.

  4. Effects of scanner acoustic noise on intrinsic brain activity during auditory stimulation

    International Nuclear Information System (INIS)

    Yakunina, Natalia; Kang, Eun Kyoung; Kim, Tae Su; Min, Ji-Hoon; Kim, Sam Soo; Nam, Eui-Cheol

    2015-01-01

    Although the effects of scanner background noise (SBN) during functional magnetic resonance imaging (fMRI) have been extensively investigated for the brain regions involved in auditory processing, its impact on other types of intrinsic brain activity has largely been neglected. The present study evaluated the influence of SBN on a number of intrinsic connectivity networks (ICNs) during auditory stimulation by comparing the results obtained using sparse temporal acquisition (STA) with those using continuous acquisition (CA). Fourteen healthy subjects were presented with classical music pieces in a block paradigm during two sessions of STA and CA. A volume-matched CA dataset (CAm) was generated by subsampling the CA dataset to temporally match it with the STA data. Independent component analysis was performed on the concatenated STA-CAm datasets, and voxel data, time courses, power spectra, and functional connectivity were compared. The ICA revealed 19 ICNs; the auditory, default mode, salience, and frontoparietal networks showed greater activity in the STA. The spectral peaks in 17 networks corresponded to the stimulation cycles in the STA, while only five networks displayed this correspondence in the CA. The dorsal default mode and salience networks exhibited stronger correlations with the stimulus waveform in the STA. SBN appeared to influence not only the areas of auditory response but also the majority of other ICNs, including attention and sensory networks. Therefore, SBN should be regarded as a serious nuisance factor during fMRI studies investigating intrinsic brain activity under external stimulation or task loads. (orig.)

  5. Amygdala and auditory cortex exhibit distinct sensitivity to relevant acoustic features of auditory emotions.

    Science.gov (United States)

    Pannese, Alessia; Grandjean, Didier; Frühholz, Sascha

    2016-12-01

    Discriminating between auditory signals of different affective value is critical to successful social interaction. It is commonly held that acoustic decoding of such signals occurs in the auditory system, whereas affective decoding occurs in the amygdala. However, given that the amygdala receives direct subcortical projections that bypass the auditory cortex, it is possible that some acoustic decoding occurs in the amygdala as well, when the acoustic features are relevant for affective discrimination. We tested this hypothesis by combining functional neuroimaging with the neurophysiological phenomena of repetition suppression (RS) and repetition enhancement (RE) in human listeners. Our results show that both amygdala and auditory cortex responded differentially to physical voice features, suggesting that the amygdala and auditory cortex decode the affective quality of the voice not only by processing the emotional content from previously processed acoustic features, but also by processing the acoustic features themselves, when these are relevant to the identification of the voice's affective value. Specifically, we found that the auditory cortex is sensitive to spectral high-frequency voice cues when discriminating vocal anger from vocal fear and joy, whereas the amygdala is sensitive to vocal pitch when discriminating between negative vocal emotions (i.e., anger and fear). Vocal pitch is an instantaneously recognized voice feature, which is potentially transferred to the amygdala by direct subcortical projections. These results together provide evidence that, besides the auditory cortex, the amygdala too processes acoustic information, when this is relevant to the discrimination of auditory emotions. Copyright © 2016 Elsevier Ltd. All rights reserved.

  6. Interaction of language, auditory and memory brain networks in auditory verbal hallucinations

    NARCIS (Netherlands)

    Curcic-Blake, Branislava; Ford, Judith M.; Hubl, Daniela; Orlov, Natasza D.; Sommer, Iris E.; Waters, Flavie; Allen, Paul; Jardri, Renaud; Woodruff, Peter W.; David, Olivier; Mulert, Christoph; Woodward, Todd S.; Aleman, Andre

    Auditory verbal hallucinations (AVH) occur in psychotic disorders, but also as a symptom of other conditions and even in healthy people. Several current theories on the origin of AVH converge, with neuroimaging studies suggesting that the language, auditory and memory/limbic networks are of

  7. MRI of congenital pituitary insufficiency

    International Nuclear Information System (INIS)

    Almeida Magalhaes, Alvaro C. de; Uehara, Karla C.; Iezzi, Denise

    1995-01-01

    We compare 1,5 T magnetic resonance (MR) image findings in 193 patients with congenital pituitary congenital insufficiency. One hundred and thirty nine of the MR studies were obtained in patients who had isolated growth hormone deficiency. Other fifth - four patients had multiple pituitary hormone deficiency. On MR images, normal anterior and posterior lobes of the pituitary glands can be clearly differentiated because the posterior lobe has a characteristic high intensity on TI-weighted images. In fifty-four patients, the high- intensity of the posterior lobe was not seen, but a similar high signal intensity was observed at the proximal stump in fifty-one patients. this high- intensity area is the newly formed ectopic posterior lobe, which also secrets anti-diuretic hormone just as the posterior lobe would. MR imaging can demonstrate the transection of the pituitary stalk and the formation of the ectopic lobe, revealing to be a useful diagnostic tool in the definition of the type of alteration in growth defects of endocrine origin. (author)

  8. Corpuscular radiation and congenital anomalies

    International Nuclear Information System (INIS)

    Sato, Shinji; Yajima, Akira

    1983-01-01

    Many explorations have been done by our antecessors to find out about the congenital anomalies which might be caused by X-ray or γ-ray and experimental teratological researches have also been done, with some results. However, there have been less systematic studies on corpuscular radiation. Neutron ray is a radioactive ray no electrically-charged equally as photon (X-ray, γ-ray). With an equal dosage of it as photon's, its localized energy is high so that it is different in RBE, OER, etc. In heavy charged particle (proton ray, He ion, Ne ion, π-meson ray), there is the characteristic that the energy increases at around the deepest spot within the range rather than at the point of injection into a system, which is called Bragg peak. The type and energy of this radiation reflected in the uniqueness in energy distribution, in ionization density and in LET makes it the most important radioactive biological parameter. At this paper, we shall review the types of radioactive rays and discuss the congenital anomalies (teratogenecity) including the experimental results obtained by application of our proton ray. (author)

  9. ExternE: Externalities of energy Vol. 5. Nuclear

    International Nuclear Information System (INIS)

    Dreicer, M.; Tort, V.; Manen, P.

    1995-01-01

    Since the early 1970s, there has been increased interest in the environmental impacts that are caused by the generation of electricity. The comparative risk assessment studies at that time used mainly deaths and injuries as impact indicators. By the end of the 1980s studies changed to the assessment of the costs imposed on society and the environment that were not included in the market price of the energy produced, the so-called external costs. The preliminary studies that were published set the conceptual basis, grounded in neo-classical economics, for the valuation of the health and environmental impacts that could be assessed. As a consequence of the many questions raised by the methodologies employed by these early studies, Directorate General XII (DG XII) of the Commission of the European Communities established a collaborative research programme with the United States Department of Energy to identify an appropriate methodology for this type of work. Following the completion of this collaboration, the DG XII programme has continued as the ExternE project. The main objective of the work carried out at CEPN was to develop an impact pathway methodology for the nuclear fuel cycle that would be consistent with the methodologies developed for other fuel cycles, without loosing the nuclear-specific techniques required for a proper evaluation. In this way, comparisons between the different fuel cycles would be possible. This report presents the methodology and demonstration of the results in the context of the French nuclear fuel cycle. The United States team at Oak Ridge National Laboratory has previously issued a draft report on the results of their assessment. The French fuel cycle was broken down into 8 separate stages. Reference sites and 1990s technology were chosen to represent the total nuclear fuel cycle, as it exists today. In addition, the transportation of material between the sites was considered. The facilities are assessed for routine operation, except

  10. Encoding of Sucrose's Palatability in the Nucleus Accumbens Shell and Its Modulation by Exteroceptive Auditory Cues

    Directory of Open Access Journals (Sweden)

    Miguel Villavicencio

    2018-05-01

    Full Text Available Although the palatability of sucrose is the primary reason for why it is over consumed, it is not well understood how it is encoded in the nucleus accumbens shell (NAcSh, a brain region involved in reward, feeding, and sensory/motor transformations. Similarly, untouched are issues regarding how an external auditory stimulus affects sucrose palatability and, in the NAcSh, the neuronal correlates of this behavior. To address these questions in behaving rats, we investigated how food-related auditory cues modulate sucrose's palatability. The goals are to determine whether NAcSh neuronal responses would track sucrose's palatability (as measured by the increase in hedonically positive oromotor responses lick rate, sucrose concentration, and how it processes auditory information. Using brief-access tests, we found that sucrose's palatability was enhanced by exteroceptive auditory cues that signal the start and the end of a reward epoch. With only the start cue the rejection of water was accelerated, and the sucrose/water ratio was enhanced, indicating greater palatability. However, the start cue also fragmented licking patterns and decreased caloric intake. In the presence of both start and stop cues, the animals fed continuously and increased their caloric intake. Analysis of the licking microstructure confirmed that auditory cues (either signaling the start alone or start/stop enhanced sucrose's oromotor-palatability responses. Recordings of extracellular single-unit activity identified several distinct populations of NAcSh responses that tracked either the sucrose palatability responses or the sucrose concentrations by increasing or decreasing their activity. Another neural population fired synchronously with licking and exhibited an enhancement in their coherence with increasing sucrose concentrations. The population of NAcSh's Palatability-related and Lick-Inactive neurons were the most important for decoding sucrose's palatability. Only the Lick

  11. Manipulation of Auditory Inputs as Rehabilitation Therapy for Maladaptive Auditory Cortical Reorganization

    Directory of Open Access Journals (Sweden)

    Hidehiko Okamoto

    2018-01-01

    Full Text Available Neurophysiological and neuroimaging data suggest that the brains of not only children but also adults are reorganized based on sensory inputs and behaviors. Plastic changes in the brain are generally beneficial; however, maladaptive cortical reorganization in the auditory cortex may lead to hearing disorders such as tinnitus and hyperacusis. Recent studies attempted to noninvasively visualize pathological neural activity in the living human brain and reverse maladaptive cortical reorganization by the suitable manipulation of auditory inputs in order to alleviate detrimental auditory symptoms. The effects of the manipulation of auditory inputs on maladaptively reorganized brain were reviewed herein. The findings obtained indicate that rehabilitation therapy based on the manipulation of auditory inputs is an effective and safe approach for hearing disorders. The appropriate manipulation of sensory inputs guided by the visualization of pathological brain activities using recent neuroimaging techniques may contribute to the establishment of new clinical applications for affected individuals.

  12. Improvement of auditory hallucinations and reduction of primary auditory area's activation following TMS

    International Nuclear Information System (INIS)

    Giesel, Frederik L.; Mehndiratta, Amit; Hempel, Albrecht; Hempel, Eckhard; Kress, Kai R.; Essig, Marco; Schröder, Johannes

    2012-01-01

    Background: In the present case study, improvement of auditory hallucinations following transcranial magnetic stimulation (TMS) therapy was investigated with respect to activation changes of the auditory cortices. Methods: Using functional magnetic resonance imaging (fMRI), activation of the auditory cortices was assessed prior to and after a 4-week TMS series of the left superior temporal gyrus in a schizophrenic patient with medication-resistant auditory hallucinations. Results: Hallucinations decreased slightly after the third and profoundly after the fourth week of TMS. Activation in the primary auditory area decreased, whereas activation in the operculum and insula remained stable. Conclusions: Combination of TMS and repetitive fMRI is promising to elucidate the physiological changes induced by TMS.

  13. The basis of musical consonance as revealed by congenital amusia.

    Science.gov (United States)

    Cousineau, Marion; McDermott, Josh H; Peretz, Isabelle

    2012-11-27

    Some combinations of musical notes sound pleasing and are termed "consonant," but others sound unpleasant and are termed "dissonant." The distinction between consonance and dissonance plays a central role in Western music, and its origins have posed one of the oldest and most debated problems in perception. In modern times, dissonance has been widely believed to be the product of "beating": interference between frequency components in the cochlea that has been believed to be more pronounced in dissonant than consonant sounds. However, harmonic frequency relations, a higher-order sound attribute closely related to pitch perception, has also been proposed to account for consonance. To tease apart theories of musical consonance, we tested sound preferences in individuals with congenital amusia, a neurogenetic disorder characterized by abnormal pitch perception. We assessed amusics' preferences for musical chords as well as for the isolated acoustic properties of beating and harmonicity. In contrast to control subjects, amusic listeners showed no preference for consonance, rating the pleasantness of consonant chords no higher than that of dissonant chords. Amusics also failed to exhibit the normally observed preference for harmonic over inharmonic tones, nor could they discriminate such tones from each other. Despite these abnormalities, amusics exhibited normal preferences and discrimination for stimuli with and without beating. This dissociation indicates that, contrary to classic theories, beating is unlikely to underlie consonance. Our results instead suggest the need to integrate harmonicity as a foundation of music preferences, and illustrate how amusia may be used to investigate normal auditory function.

  14. Congenital anophthalmia and microphthalmia: epidemiology and orbitofacial rehabilitation

    Directory of Open Access Journals (Sweden)

    Llorente-González S

    2011-12-01

    Full Text Available Sara Llorente-González1, J Peralta-Calvo2, JM Abelairas-Gómez21Ophthalmology Service of Hospital de Torrejón, Madrid, Spain; 2Ophthalmology Service of Hospital Universitario La Paz, Madrid, SpainObjective: To describe the prevalence of congenital anophthalmia and microphthalmia in Hospital Universitario La Paz, and to identify associated risk factors and evaluate cosmetic results in treated and nontreated patients.Methods: A retrospective, descriptive, cross-sectional study of patients treated with orbital expanding techniques (cases and nontreated patients (controls was carried out as a comparative case series study. A total of 36 patients with unilateral or bilateral anophthalmia or microphthalmia as main diagnosis were included; 52 epidemiological and management variables for each patient were analyzed. The study evaluated orbital growth and facial symmetry.Results: The overall cosmetic result in the study’s group of patients was satisfactory: 66.7% showed good or very good orbital growth, and 75% showed good or very good facial symmetry. Controls had better cosmetic outcome but showed more cataracts (P = 0.05, inferior colobomas (P = 0.026, and family history (P = 0.056 than the cases. Controls also showed significantly better orbital growth (P = 0.042 and facial symmetry (P = 0.014 than the cases.Conclusion: This study suggests that the mere presence of a globe (controls still provides better orbitofacial development than the artificial stimulation (cases currently available for patients with congenital anophthalmia and microphthalmia, who receive internal and external orbital rehabilitation.Keywords: cosmetic outcome, orbital rehabilitation, ocular malformation, congenital cataract, persistent fetal vasculature

  15. Auditory and motor imagery modulate learning in music performance.

    Science.gov (United States)

    Brown, Rachel M; Palmer, Caroline

    2013-01-01

    Skilled performers such as athletes or musicians can improve their performance by imagining the actions or sensory outcomes associated with their skill. Performers vary widely in their auditory and motor imagery abilities, and these individual differences influence sensorimotor learning. It is unknown whether imagery abilities influence both memory encoding and retrieval. We examined how auditory and motor imagery abilities influence musicians' encoding (during Learning, as they practiced novel melodies), and retrieval (during Recall of those melodies). Pianists learned melodies by listening without performing (auditory learning) or performing without sound (motor learning); following Learning, pianists performed the melodies from memory with auditory feedback (Recall). During either Learning (Experiment 1) or Recall (Experiment 2), pianists experienced either auditory interference, motor interference, or no interference. Pitch accuracy (percentage of correct pitches produced) and temporal regularity (variability of quarter-note interonset intervals) were measured at Recall. Independent tests measured auditory and motor imagery skills. Pianists' pitch accuracy was higher following auditory learning than following motor learning and lower in motor interference conditions (Experiments 1 and 2). Both auditory and motor imagery skills improved pitch accuracy overall. Auditory imagery skills modulated pitch accuracy encoding (Experiment 1): Higher auditory imagery skill corresponded to higher pitch accuracy following auditory learning with auditory or motor interference, and following motor learning with motor or no interference. These findings suggest that auditory imagery abilities decrease vulnerability to interference and compensate for missing auditory feedback at encoding. Auditory imagery skills also influenced temporal regularity at retrieval (Experiment 2): Higher auditory imagery skill predicted greater temporal regularity during Recall in the presence of

  16. Auditory and motor imagery modulate learning in music performance

    Science.gov (United States)

    Brown, Rachel M.; Palmer, Caroline

    2013-01-01

    Skilled performers such as athletes or musicians can improve their performance by imagining the actions or sensory outcomes associated with their skill. Performers vary widely in their auditory and motor imagery abilities, and these individual differences influence sensorimotor learning. It is unknown whether imagery abilities influence both memory encoding and retrieval. We examined how auditory and motor imagery abilities influence musicians' encoding (during Learning, as they practiced novel melodies), and retrieval (during Recall of those melodies). Pianists learned melodies by listening without performing (auditory learning) or performing without sound (motor learning); following Learning, pianists performed the melodies from memory with auditory feedback (Recall). During either Learning (Experiment 1) or Recall (Experiment 2), pianists experienced either auditory interference, motor interference, or no interference. Pitch accuracy (percentage of correct pitches produced) and temporal regularity (variability of quarter-note interonset intervals) were measured at Recall. Independent tests measured auditory and motor imagery skills. Pianists' pitch accuracy was higher following auditory learning than following motor learning and lower in motor interference conditions (Experiments 1 and 2). Both auditory and motor imagery skills improved pitch accuracy overall. Auditory imagery skills modulated pitch accuracy encoding (Experiment 1): Higher auditory imagery skill corresponded to higher pitch accuracy following auditory learning with auditory or motor interference, and following motor learning with motor or no interference. These findings suggest that auditory imagery abilities decrease vulnerability to interference and compensate for missing auditory feedback at encoding. Auditory imagery skills also influenced temporal regularity at retrieval (Experiment 2): Higher auditory imagery skill predicted greater temporal regularity during Recall in the presence of

  17. Experience and information loss in auditory and visual memory.

    Science.gov (United States)

    Gloede, Michele E; Paulauskas, Emily E; Gregg, Melissa K

    2017-07-01

    Recent studies show that recognition memory for sounds is inferior to memory for pictures. Four experiments were conducted to examine the nature of auditory and visual memory. Experiments 1-3 were conducted to evaluate the role of experience in auditory and visual memory. Participants received a study phase with pictures/sounds, followed by a recognition memory test. Participants then completed auditory training with each of the sounds, followed by a second memory test. Despite auditory training in Experiments 1 and 2, visual memory was superior to auditory memory. In Experiment 3, we found that it is possible to improve auditory memory, but only after 3 days of specific auditory training and 3 days of visual memory decay. We examined the time course of information loss in auditory and visual memory in Experiment 4 and found a trade-off between visual and auditory recognition memory: Visual memory appears to have a larger capacity, while auditory memory is more enduring. Our results indicate that visual and auditory memory are inherently different memory systems and that differences in visual and auditory recognition memory performance may be due to the different amounts of experience with visual and auditory information, as well as structurally different neural circuitry specialized for information retention.

  18. Perceptual Plasticity for Auditory Object Recognition

    Science.gov (United States)

    Heald, Shannon L. M.; Van Hedger, Stephen C.; Nusbaum, Howard C.

    2017-01-01

    In our auditory environment, we rarely experience the exact acoustic waveform twice. This is especially true for communicative signals that have meaning for listeners. In speech and music, the acoustic signal changes as a function of the talker (or instrument), speaking (or playing) rate, and room acoustics, to name a few factors. Yet, despite this acoustic variability, we are able to recognize a sentence or melody as the same across various kinds of acoustic inputs and determine meaning based on listening goals, expectations, context, and experience. The recognition process relates acoustic signals to prior experience despite variability in signal-relevant and signal-irrelevant acoustic properties, some of which could be considered as “noise” in service of a recognition goal. However, some acoustic variability, if systematic, is lawful and can be exploited by listeners to aid in recognition. Perceivable changes in systematic variability can herald a need for listeners to reorganize perception and reorient their attention to more immediately signal-relevant cues. This view is not incorporated currently in many extant theories of auditory perception, which traditionally reduce psychological or neural representations of perceptual objects and the processes that act on them to static entities. While this reduction is likely done for the sake of empirical tractability, such a reduction may seriously distort the perceptual process to be modeled. We argue that perceptual representations, as well as the processes underlying perception, are dynamically determined by an interaction between the uncertainty of the auditory signal and constraints of context. This suggests that the process of auditory recognition is highly context-dependent in that the identity of a given auditory object may be intrinsically tied to its preceding context. To argue for the flexible neural and psychological updating of sound-to-meaning mappings across speech and music, we draw upon examples

  19. Evolution of external genitalia: insights from reptilian development.

    Science.gov (United States)

    Gredler, Marissa L; Larkins, Christine E; Leal, Francisca; Lewis, A Kelsey; Herrera, Ana M; Perriton, Claire L; Sanger, Thomas J; Cohn, Martin J

    2014-01-01

    External genitalia are found in each of the major clades of amniotes. The phallus is an intromittent organ that functions to deliver sperm into the female reproductive tract for internal fertilization. The cellular and molecular genetic mechanisms of external genital development have begun to be elucidated from studies of the mouse genital tubercle, an embryonic appendage adjacent to the cloaca that is the precursor of the penis and clitoris. Progress in this area has improved our understanding of genitourinary malformations, which are among the most common birth defects in humans, and created new opportunities for comparative studies of other taxa. External genitalia evolve rapidly, which has led to a striking diversity of anatomical forms. Within the past year, studies of external genital development in non-mammalian amniotes, including birds, lizards, snakes, alligators, and turtles, have begun to shed light on the molecular and morphogenetic mechanisms underlying the diversification of phallus morphology. Here, we review recent progress in the comparative developmental biology of external genitalia and discuss the implications of this work for understanding external genital evolution. We address the question of the deep homology (shared common ancestry) of genital structures and of developmental mechanisms, and identify new areas of investigation that can be pursued by taking a comparative approach to studying development of the external genitalia. We propose an evolutionary interpretation of hypospadias, a congenital malformation of the urethra, and discuss how investigations of non-mammalian species can provide novel perspectives on human pathologies.

  20. Absence of auditory 'global interference' in autism.

    Science.gov (United States)

    Foxton, Jessica M; Stewart, Mary E; Barnard, Louise; Rodgers, Jacqui; Young, Allan H; O'Brien, Gregory; Griffiths, Timothy D

    2003-12-01

    There has been considerable recent interest in the cognitive style of individuals with Autism Spectrum Disorder (ASD). One theory, that of weak central coherence, concerns an inability to combine stimulus details into a coherent whole. Here we test this theory in the case of sound patterns, using a new definition of the details (local structure) and the coherent whole (global structure). Thirteen individuals with a diagnosis of autism or Asperger's syndrome and 15 control participants were administered auditory tests, where they were required to match local pitch direction changes between two auditory sequences. When the other local features of the sequence pairs were altered (the actual pitches and relative time points of pitch direction change), the control participants obtained lower scores compared with when these details were left unchanged. This can be attributed to interference from the global structure, defined as the combination of the local auditory details. In contrast, the participants with ASD did not obtain lower scores in the presence of such mismatches. This was attributed to the absence of interference from an auditory coherent whole. The results are consistent with the presence of abnormal interactions between local and global auditory perception in ASD.