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Sample records for congenital temporal bone

  1. 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.

  2. Temporal bone CT analysis of congenital ear anomalies

    International Nuclear Information System (INIS)

    Hwang, Jung Won; Moon, Min Joo; Sung, Kyu Bo

    1988-01-01

    Authors analysed the CT findings of the congenital ear anomalies of twenty-nine patients for 2 years and 3 months. The results were as follows: 1. Most of the patients were under the age of 20 (82.7%) and prevalent in male (72.4%). 2. Clinically, congenital ear anomalies were detected in 20 patients (68.9%), conductive hearing loss in 4, sensorineural hearing loss in 1, and the remained 4 patients were detected incidentally without clinical symptom. 3. In the cases of unilateral involvement of 20 patients, right ear was more common (12/20). Eight of 9 bilateral involvement showed similar degree. 4. The middle ear malformations were found in 22 patients (75.9%) and bilateral in 4 patients. 26 cases of middle ear malformations had been classified by Frey into 4 groups; Group I in 5, Group II in 9, Graoup III in 9 and Group IV in 3. 5. Incidentally found ear anomaly was lateral semicircular canal formed a single cavity with the vestibule in all patients (5 pts.). 6. Inner ear malformations accompanying sensorineural hearing loss were found in 3 patients with bilateral involvement and middle ear malformations were accompanied in 2 patients. The degree of involvement of labyrinth was variable.

  3. Three-dimensional assessment of the temporal bone and mandible deformations in patients with congenital aural atresia.

    Science.gov (United States)

    Fu, Yaoyao; Li, Chenlong; Dai, Peidong; Zhang, Tianyu

    2017-10-01

    To investigate the deformations of temporal bone and mandible combined with congenital aural atresia. A total of 158 patients with congenital aural atresia were included in the study. The raw CT data of the temporal bone was imported into MIMICS v 12 and threshold dissection, region growing and three-dimensional (3D) calculation were used to calculate 3D models. The 3D characteristics of the temporal bone and upper part of mandible were assessed. The tympanic part of the temporal bone was all undeveloped. Of all the patients included, 14 patients were found to have severe maxillofacial malformations. Among them, 2 cases have floating arch, 4 cases have interrupted arch, 5 cases have mandibular processes hypoplasia and 3 cases have interrupted arch combined with severe maxillary malformation. Ten of the 14 patients were suffered from dysplasia of the mastoid part of the temporal bone as well. Maxillofacial malformations may sometimes coexist with congenital aural atresia. Otolaryngologists should not neglect the coexisted maxillofacial malformations and give timely referral to maxillofacial surgeons. Copyright © 2017. Published by Elsevier B.V.

  4. 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.

  5. Otosclerosis: Temporal Bone Pathology.

    Science.gov (United States)

    Quesnel, Alicia M; Ishai, Reuven; McKenna, Michael J

    2018-04-01

    Otosclerosis is pathologically characterized by abnormal bony remodeling, which includes bone resorption, new bone deposition, and vascular proliferation in the temporal bone. Sensorineural hearing loss in otosclerosis is associated with extension of otosclerosis to the cochlear endosteum and deposition of collagen throughout the spiral ligament. Persistent or recurrent conductive hearing loss after stapedectomy has been associated with incomplete footplate fenestration, poor incus-prosthesis connection, and incus resorption in temporal bone specimens. Human temporal bone pathology has helped to define the role of computed tomography imaging for otosclerosis, confirming that computed tomography is highly sensitive for diagnosis, yet limited in assessing cochlear endosteal involvement. Copyright © 2017 Elsevier Inc. All rights reserved.

  6. SHEEP TEMPORAL BONE

    Directory of Open Access Journals (Sweden)

    Kesavan

    2016-03-01

    Full Text Available INTRODUCTION Human temporal bones are difficult to procure now a days due to various ethical issues. Sheep temporal bone is a good alternative due to morphological similarities, easy to procure and less cost. Many middle ear exercises can be done easily and handling of instruments is done in the procedures like myringoplasty, tympanoplasty, stapedotomy, facial nerve dissection and some middle ear implants. This is useful for resident training programme.

  7. Chondroblastoma of temporal bone

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    Tanohta, K.; Noda, M.; Katoh, H.; Okazaki, A.; Sugiyama, S.; Maehara, T.; Onishi, S.; Tanida, T.

    1986-07-01

    The case of a 55-year-old female with chondroblastoma arising from the left temporal bone is presented. Although 10 cases of temporal chondroblastoma have been reported, this is the first in which plain radiography, pluridirectional tomography, computed tomography (CT) and angiography were performed. We discuss the clinical and radiological aspects of this rare tumor.

  8. Chondroblastoma of temporal bone

    International Nuclear Information System (INIS)

    Tanohta, K.; Noda, M.; Katoh, H.; Okazaki, A.; Sugiyama, S.; Maehara, T.; Onishi, S.; Tanida, T.

    1986-01-01

    The case of a 55-year-old female with chondroblastoma arising from the left temporal bone is presented. Although 10 cases of temporal chondroblastoma have been reported, this is the first in which plain radiography, pluridirectional tomography, computed tomography (CT) and angiography were performed. We discuss the clinical and radiological aspects of this rare tumor. (orig.)

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

    Science.gov (United States)

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

    2000-01-01

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

  10. Thickened cortical bones in congenital neutropenia

    International Nuclear Information System (INIS)

    Boechat, M.I.; Gormley, L.S.; O'Laughlin, B.J.

    1987-01-01

    Congenital neutropenia is an uncommon entity which may be familial and has a wide spectrum of clinical expression. Three sisters with the severe form of the disease, that suffered from recurrent infections which lead to their demise are described. Review of their radiographs revealed the presence of cortical thickening of the bones. Although several syndroms with different bone abnormalities have been reported associated with neutropenia, the radiographic finding of thickened cortex in children with congenital neutropenia has not been previously described. (orig.)

  11. Thickened cortical bones in congenital neutropenia

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    Boechat, M.I.; Gormley, L.S.; O' Laughlin, B.J.

    1987-02-01

    Congenital neutropenia is an uncommon entity which may be familial and has a wide spectrum of clinical expression. Three sisters with the severe form of the disease, that suffered from recurrent infections which lead to their demise are described. Review of their radiographs revealed the presence of cortical thickening of the bones. Although several syndroms with different bone abnormalities have been reported associated with neutropenia, the radiographic finding of thickened cortex in children with congenital neutropenia has not been previously described.

  12. CT of temporal bone - IV. inner ear

    International Nuclear Information System (INIS)

    Kwon, Jae Yoon; Sung, Kyu Bo; Youn, Eun Kyoung; Park, Youn Kyeung; Lee, Young Uk

    1990-01-01

    Temporal bone CT was done in 697 patients from April 1985 to October 1989. The abnormal findings were seen in 453 patients, which were chronic otitis media in 355 patients, fracture in 49 patients and congenital anomaly in 44 patients, etc. The abnormal findings of inner ear were observed on 46 patients. The results were summarized as follows : 1. The incidence of inner ear involvement by chronic otitis media was 7.3% (26/355 : labyrinthine fistula in 17 patients, labyrinthitis ossificans in 9 patients). Labyrinthine fistula was most commonly located on lateral semicircular canal (15/17, 88.2%). 2. Fusion of vestibule with lateral semicircular canal and formation of common cavity was demonstrated incidentally in 5 patients (0.7% of total number of temporal bone CT), and bilateral in 3 patients. 3. The incidence of inner ear anomaly in congenital ear anomaly was 11.4% (5/44). All cases were bilateral and three patients showed associated middle ear anomaly. 4. The incidence of involvement of bony labyrinth in temporal bone fracture was 10.2% (5/49). Labyrinthine fracture was seen all patients of transverse(3) and mixed fracture(1). In longitudinal fracture, labyrinthine fracture was seen in 2.2% (1/45). 5. Others were traumatic labyrinthitis ossificans(1), intracanalicular acoustic neuroma(3) and facial nerve neuroma(1)

  13. Aneurysmal bone cyst of the temporal bone

    International Nuclear Information System (INIS)

    Buxi, Tarvinder; Sud Seema; Vohra, Rakesh; Sud, Aditi; Singh, Satnam

    2004-01-01

    Aneurysmal bone cyst (ABC) of the temporal bone is rare. The nature of the underlying disorder that converted into the ABC might, however, be difficult to ascertain on imaging as well as on histopathology. The unusual CT and MRI findings in a case of ABC of the temporal bone are presented. This had transdural intracerebral spread with a large component of solid enhancing matrix but no peripheral calcific rim. The patient was an adult of 45 years with a history of headache for more than 1 year Copyright (2004) Blackwell Publishing Asia Pty Ltd

  14. Temporal bone trauma and imaging

    International Nuclear Information System (INIS)

    Turetschek, K.; Czerny, C.; Wunderbaldinger, P.; Steiner, E.

    1997-01-01

    Fractures of the temporal bone result from direct trauma to the temporal bone or occur as one component of a severe craniocerebral injury. Complications of temporal trauma are hemotympanon, facial nerve paralysis, conductive or sensorineur hearing loss, and leakage of cerebrospinal fluid. Erly recognition and an appropiate therapy may improve or prevent permanent deficits related to such complications. Only 20-30% of temporal bone fractures can be visualized by plain films. CT has displaced plain radiography in the investigation of the otological trauma because subtle bony details are best evaluated by CT which even can be reformatted in multiple projections, regardless of the original plane of scanning. Associated epidural, subdural, and intracerebral hemorrhagic lesions are better defined by MRI. (orig.) [de

  15. Osteoradionecrosis of the temporal bone

    International Nuclear Information System (INIS)

    Fujimori, Masato; Koyama, Yukiko; Enomoto, Fuyuki; Ichikawa, Ginichiro

    2002-01-01

    We report a case of temporal bone necrosis that emerged after radiotherapy for epipharyngeal carcinoma performed 13 years ago. The patient was a 51-year-old male. His major complaint was left facial swelling. The patient underwent chemotherapy and radiotherapy (Co 60, 6120 rad), as the treatment of that period, for epipharyngeal carcinoma from September 30, 1986 to January 31, 1987. He also underwent lobectomy of the left temporal lobe in brain surgery for left temporal lobe necrosis in August, 1989. After that operation, we saw constriction in his left external acoustic meatus and continued the follow-up. On October 22, 1999 he felt a left facial swelling. We found skin defects and ulcer formation in the front part of his left ear. Although we administered an antiseptic and antibiotic to the diseased area, his condition did not improve. He was hospitalized for the purpose of undergoing medical treatment on January 6, 2000. We found extensive skin necrosis and defects in his left auricular area. The corrupted temporal bone reached the zygomatic, the bone department external acoustic meatus and the mastoid process was exposing. We performed debridement of the diseased area on January 19, 2000. On February 23, we performed reconstruction by left trapezius muscle flap after debridement once again. One year after the operation, the flap was completely incorporated. (author)

  16. Osteoradionecrosis of the temporal bone

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    Fujimori, Masato; Koyama, Yukiko; Enomoto, Fuyuki; Ichikawa, Ginichiro [Juntendo Univ., Tokyo (Japan). School of Medicine

    2002-08-01

    We report a case of temporal bone necrosis that emerged after radiotherapy for epipharyngeal carcinoma performed 13 years ago. The patient was a 51-year-old male. His major complaint was left facial swelling. The patient underwent chemotherapy and radiotherapy (Co 60, 6120 rad), as the treatment of that period, for epipharyngeal carcinoma from September 30, 1986 to January 31, 1987. He also underwent lobectomy of the left temporal lobe in brain surgery for left temporal lobe necrosis in August, 1989. After that operation, we saw constriction in his left external acoustic meatus and continued the follow-up. On October 22, 1999 he felt a left facial swelling. We found skin defects and ulcer formation in the front part of his left ear. Although we administered an antiseptic and antibiotic to the diseased area, his condition did not improve. He was hospitalized for the purpose of undergoing medical treatment on January 6, 2000. We found extensive skin necrosis and defects in his left auricular area. The corrupted temporal bone reached the zygomatic, the bone department external acoustic meatus and the mastoid process was exposing. We performed debridement of the diseased area on January 19, 2000. On February 23, we performed reconstruction by left trapezius muscle flap after debridement once again. One year after the operation, the flap was completely incorporated. (author)

  17. CT-scanning of ancient Greenlandic Inuit temporal bones

    International Nuclear Information System (INIS)

    Homoe, P.; Videbaek, H.

    1992-01-01

    Additional morphological evidence of former infectious middle ear disease (IMED) was found by CT-scanning in 5 of 6 Greenlandic Inuit crania strongly suspected for former IMED due to earlier examination revealing either bilateral hypocellularity or asymmetry of the pneumatized area of the temporal bones. The CT-scans showed sclerosing and obliteration of the air cells and even destruction of the cellular septae, and a high degree of irregularity of the cells. Sclerosing of the surrounding bone tissue was also found. The findings in one cranium were dubious and could both be regarded as a congenital malformation or an infection in infanthood. CT-scan confirms and even adds to the results of conventional X-ray of temporal bones making hypotheses of paleopathology more reliable. The findings also support the environmental theory of pneumatization of the air cell system in the temporal bones. (13 refs., 10 figs.)

  18. CT-scanning of ancient Greenlandic Inuit temporal bones

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    Homoe, P [Copenhagen Univ. (Denmark). Lab. of Biological Anthropology and Dept. of Otolaryngology, Head and Neck Surgery; Lynnerup, N [Copenhagen Univ., Lab. of Biological Anthropology and Univ. Inst. of Ferensic Medicine, Copenhagen (Denmark); Videbaek, H [Hvidovre Univ. Hospital, Copenhagen (Denmark). Dept. of Radiology

    1992-01-01

    Additional morphological evidence of former infectious middle ear disease (IMED) was found by CT-scanning in 5 of 6 Greenlandic Inuit crania strongly suspected for former IMED due to earlier examination revealing either bilateral hypocellularity or asymmetry of the pneumatized area of the temporal bones. The CT-scans showed sclerosing and obliteration of the air cells and even destruction of the cellular septae, and a high degree of irregularity of the cells. Sclerosing of the surrounding bone tissue was also found. The findings in one cranium were dubious and could both be regarded as a congenital malformation or an infection in infanthood. CT-scan confirms and even adds to the results of conventional X-ray of temporal bones making hypotheses of paleopathology more reliable. The findings also support the environmental theory of pneumatization of the air cell system in the temporal bones. (13 refs., 10 figs.).

  19. Chondroblastoma of the temporal bone

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    Kobayashi, Yuko; Murakami, Ryusuke; Toba, Masahiro; Ichikawa, Taro [Dept. of Radiology, Nippon Medical School, Tokyo (Japan); Kanazawa, Ryuzaburo; Sanno, Naoko; Shimura, Toshiro [Dept. of Neurosurgery, Nippon Medical School, Tokyo (Japan); Sawada, Namie; Hosone, Masaru [Dept. of Pathology, Nippon Medical School, Tokyo (Japan); Kumazaki, Tatsuo [Dept. of Radiology, Nippon Medical School, Tokyo (Japan)

    2001-12-01

    A rare case of chondroblastoma arising from the temporal bone that occurred in a 60-year-old woman is reported. The tumor appeared well demarcated and osteolytic on the radiographs. CT scan clearly depicted marginal and central calcification in the tumor. MR imaging demonstrated two components in the tumor: a solid component with predominantly low signal intensities on both T1- and T2-weighted sequences, and a multilocular cystic component with T1- and T2-elongation and fluid-fluid levels on the T2-weighted images. Postcontrast MR imaging revealed marked enhancement in the solid component and the septa of the cystic component. (orig.)

  20. The radiographic diagnosis of early attacking congenital syphilis of bone

    International Nuclear Information System (INIS)

    Ji Yaping; Zhuge Moyi

    2005-01-01

    Objective: To explore the method of radiological diagnosis of early attacking congenital syphilis. Methods: Seven cases of early attacking congenital syphilis of bone were retrospectively analyzed, diagnosed serologically, and were taken X-rays of the long bones. Results: Bone radiographs abnormalities were identified in 6 of 7 cases. Five cases suffered periotities, six cases metaphysitis, and three cases combined with diaphysitis. Seven cases had swollen soft tissue. The vertebraes, craniums and epiphysitis were not found abnormal in 7 cases. Diffusion, multiple and symmetric metaphysitis, periosteitis and osteitis were the radiological characters of congenital syphilis of bone. Conclusion: Radiography can affirm the diagnose of early attacking congenital syphilis and definite the arrange and depth. Radiographs of the extremities should be routinely taken in suspected infants. (authors)

  1. Schneiderian papilloma of the temporal bone

    NARCIS (Netherlands)

    van der Putten, L.; Bloemena, E.; Merkus, P.; Hensen, E.F.

    2013-01-01

    Temporal bone Schneiderian papilloma may present as a primary tumour originating from the middle ear and mastoid process, or an extension from sinonasal disease. Both forms are rare, this being only the 18th case of primary temporal bone Schneiderian papilloma described to date. Although the current

  2. Cerebrospinal otorrhoea--a temporal bone report.

    Science.gov (United States)

    Walby, A P

    1988-05-01

    Spontaneous cerebrospinal otorrhoea is a rare complication of a cholesteatoma. The histological findings in a temporal bone from such a case are reported. The cholesteatoma had eroded deeply through the vestibule into the internal auditory meatus.

  3. Giant osteoblastoma of temporal bone: case report

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    FIGUEIREDO EBERVAL GADELHA

    1998-01-01

    Full Text Available Benign osteoblastoma is an uncommon bone tumor accounting for approximately 1% of all bone tumors. There are only 35 cases of skull osteoblastoma reported in the literature. We describe the case of a 23 year old male with a giant osteoblastoma of temporal bone submitted to a total removal of the tumor after an effective embolization of all external carotid branches. The authors discuss diagnostic and management aspects of this uncommon skull tumor.

  4. High resolution CT of temporal bone trauma

    International Nuclear Information System (INIS)

    Youn, Eun Kyung

    1986-01-01

    Radiographic studies of the temporal bone following head trauma are indicated when there is cerebrospinal fluid otorrhea or rhinorrhoea, hearing loss, or facial nerve paralysis. Plain radiography displays only 17-30% of temporal bone fractures and pluridirectional tomography is both difficult to perform, particularly in the acutely ill patient, and less satisfactory for the demonstration of fine fractures. Consequently, high resolution CT is the imaging method of choice for the investigation of suspected temporal bone trauma and allows special resolution of fine bony detail comparable to that attainable by conventional tomography. Eight cases of temporal bone trauma examined at Korea General Hospital April 1985 through May 1986. The results were as follows: Seven patients (87%) suffered longitudinal fractures. In 6 patients who had purely conductive hearing loss, CT revealed various ossicular chain abnormality. In one patient who had neuro sensory hearing loss, CT demonstrated intract ossicular with a fracture nearing lateral wall of the lateral semicircular canal. In one patient who had mixed hearing loss, CT showed complex fracture.

  5. 3D Printed Pediatric Temporal Bone: A Novel Training Model.

    Science.gov (United States)

    Longfield, Evan A; Brickman, Todd M; Jeyakumar, Anita

    2015-06-01

    Temporal bone dissection is a fundamental element of otologic training. Cadaveric temporal bones (CTB) are the gold standard surgical training model; however, many institutions do not have ready access to them and their cost can be significant: $300 to $500. Furthermore, pediatric cadaveric temporal bones are not readily available. Our objective is to develop a pediatric temporal bone model. Temporal bone model. Tertiary Children's Hospital. Pediatric patient model. We describe the novel use of a 3D printer for the generation of a plaster training model from a pediatric high- resolution CT temporal bone scan of a normal pediatric temporal bone. Three models were produced and were evaluated. The models utilized multiple colors (white for bone, yellow for the facial nerve) and were of high quality. Two models were drilled as a proof of concept and found to be an acceptable facsimile of the patient's anatomy, rendering all necessary surgical landmarks accurately. The only negative comments pertaining to the 3D printed temporal bone as a training model were the lack of variation in hardness between cortical and cancellous bone, noting a tactile variation from cadaveric temporal bones. Our novel pediatric 3D temporal bone training model is a viable, low-cost training option for previously inaccessible pediatric temporal bone training. Our hope is that, as 3D printers become commonplace, these models could be rapidly reproduced, allowing for trainees to print models of patients before performing surgery on the living patient.

  6. Establishing a temporal bone laboratory: considerations for ENT specialist training.

    LENUS (Irish Health Repository)

    Fennessy, B G

    2012-02-01

    Cadaveric temporal bone dissection in a temporal bone laboratory is a vital component in training safe, competent otorhinolaryngologists. Recent controversies pertaining to organ retention have resulted in a more limited supply of temporal bones. Consequently, current trainees are dissecting far fewer bones than their consultants. We discuss the establishment of a temporal bone laboratory in the Department of Anatomy in the University College Cork, from the timely preparation and preservation of the tissue to its disposal. Comparisons are drawn between our experience and that of the United States training schemes. The temporal bone laboratory in Cork is the only one in existence in Ireland. The exposure and experience obtained by registrars rotating through Cork, has resulted in noticeable improvements in their operative abilities. The temporal bone laboratory remains a core component to training. It is hoped that this article may facilitate other units overcoming obstacles to establish a temporal bone laboratory.

  7. Temporal bone radiography using the orthopantomograph

    International Nuclear Information System (INIS)

    Tatezawa, T.

    1981-01-01

    Temporal bone radiographs obtained with an Orthopantomograph were compared with conventional radiographs. In acoustic neurinoma, cholesteatoma, otitis media, and middle fossa tumors, both methods demonstrated the abnormalities well. In two cases with lesions extending beyond the range of conventional projections, the broad orthopantomographic coverage was very valuable. Mastoid air cells, the mastoid process, petrous ridge, and internal auditory meatus were well demonstrated by both techniques. Orthopantomography was found to be superior in the demonstration of the petrous apex, while the superior semicircular canal was better demonstrated on the conventional views. Bilateral symmetry was particularly good and because of fewer films, radiation exposure was considerably less with orthopantomography. For many applications, orthopantomography is an adequate convenient substitute for conventional methods of examining the temporal bones

  8. CT-diagnosis of temporal bone trauma

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    Valavanis, A.; Stuckmann, G.; Antonucci, F.; Schubiger, O.

    1986-02-01

    73 patients with 78 fractures of the temporal bone were examined by high-resolution computed tomography (CT). Analysis of the CT-findings disclosed 55 longitudinal, 12 transverse, 8 combined and 3 atypical fractures. For determination of the fracture type, axial sections usually proved sufficient. However, for precise topographic analysis of the course of the fracture additional coronal sections were necessary in most of the cases. In the radiologic evaluation of temporal bone fractures detection of associated. Complications is clinically important since these can be surgically corrected. In this series 20 lesions of the ossicular chain were demonstrated by the combined performance of axial and coronal sections and sagittal reformations. High resolution CT demonstrated a lesion of the facial nerve canal in 79% of a patient group with traumatic facial nerve palsy. The most frequent site of injury of the facial nerve canal was the region of the geniculate ganglion. With the use of metrizaminde-CT-cisternography the site of cerebrospinal fluid leakage was demonstrated in 7 of 9 patients with liquorrhea. It is concluded that high-resolution CT is the radiologic method of choice for both topographic evaluation of temporal bone fractures and detection and precise localization of fracture-complications. (orig.).

  9. Ruptured Aneurysms of the Occipital Artery Associated with Congenital Occipital Bone Defect.

    Science.gov (United States)

    Kawasaki, Toshinari; Yoshida, Kazumichi; Kikuchi, Takayuki; Ishii, Akira; Takagi, Yasushi; Miyamoto, Susumu

    2017-01-01

    Traumatic aneurysms of the superficial temporal artery have been frequently reported in the literature, whereas traumatic aneurysms of the occipital artery (OA) are extremely rare. A 30-year-old man had been followed at another hospital for meningoencephalocele associated with his congenital occipital bone defect. He was admitted to our hospital with a chief complaint of neck swelling and pain during a football game. Computed tomography and magnetic resonance imaging showed a hematoma in his right neck along with the meningoencephalocele. In addition, it showed an atrophic cerebellum with a cyst protruding from his occipital bone defect. Digital subtraction angiography of the right OA showed 3 aneurysms responsible for the large hematoma in his neck. Endovascular embolization with 20% N-butyl-2-cyanoacrylate was performed for treatment of the ruptured aneurysms followed by emergent surgical evacuation of the hematoma. An occipital cranioplasty with titanium mesh was performed 10 months after the emergent intervention. In this patient, the congenital occipital bone defect with meningoencephalocele might have been the remote source of risk for traumatic pseudoaneurysms along the muscle branches of the OA. Copyright © 2016 Elsevier Inc. All rights reserved.

  10. Diagnosis of temporal bone diseases using three-dimensional images with multislice CT

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    Toyama, Yoshihiro; Togami, Taro; Murota, Makiko; Fukunaga, Kotaro; Hino, Ichiro; Sato, Katashi; Ohkawa, Motoomi [Kagawa Medical Univ., Miki (Japan)

    2001-08-01

    We evaluated the usefulness of three-dimensional images with multislice CT in the temporal bone diseases. Fifty-nine cases (26 with medial otitis, 8 choresteatoma, 10 congenital malformation, 3 high jugular bulb, 2 otosclerosis, and 10 others) were included in this study. In the ossicular and inner ear lesions, oblique multiplanar images of the long axis of each ossicle was useful the detection of abnormality. Structural deformity of ossicles and bony labyrinth were clearly delineated by surface rendering images. (author)

  11. CT diagnosis and differential diagnosis of otodystrophic lesions of the temporal bone

    International Nuclear Information System (INIS)

    D'Archambeau, O.; Parizel, P.M.; Schepper, A.M. De; Koekelkoren, E.; Van De Heyning, P.

    1990-01-01

    The purpose of this study was to assess the diagnostic and differential diagnostic value of high-resolution computed tomography in the evaluation of temporal-bone dystrophies. The study group included 55 patients with osseous abnormalities of the temporal bone in general, and the labyrinthine capsule in particular. In 27 patients the CT scan revealed evidence of otodystrophic lesions. The CT findings in patients with otosclerosis (21 patients), osteogenesis imperfecta (two patients), fibrous dysplasia (one patient). Paget's disease (one patient) and osteoporosis (two patients) are described. The CT scans of 17 patients revealed secondary osseous lesions due to metastasis (five patients), post-inflammatory changes (10 patients) or labyrinthitis ossificans (two patients). Normal variants and congenital mineralization defects were diagnosed in nine patients, Down's syndrome in two. Our results indicate the importance of high-resolution computed tomography as the primary imaging modality in evaluating osseous lesions of the temporal bone and labyrinth. (author). 14 refs.; 13 figs; 2 tabs

  12. Sclerosteosis involving the temporal bone: histopathologic aspects.

    Science.gov (United States)

    Nager, G T; Hamersma, H

    1986-01-01

    Sclerosteosis is a rare, potentially lethal, autosomal recessive, progressive craniotubular sclerosing bone dysplasia with characteristic facial and skeletal features. The temporal bone changes include a marked increase in overall size, extensive sclerosis, narrowing of the external auditory canal, and severe constriction of the internal auditory meatus, fallopian canal, eustachian tube, and middle ear cleft. Attenuation of the bony canals of the 9th, 10th, and 11th cranial nerves, reduction in size of the internal carotid artery, and severe obliteration of the sigmoid sinus and jugular bulb also occur. Loss of hearing, generally bilateral, is a frequent symptom. It often manifests in early childhood and initially is expressed as sound conduction impairment. Later, a sensorineural hearing loss and loss of vestibular nerve function often develop. Impairment of facial nerve function is another feature occasionally present at birth. In the beginning, a unilateral intermittent facial weakness may occur which eventually progresses to a bilateral permanent facial paresis. The histologic examination of the temporal bones from a patient with sclerosteosis explains the mechanisms involved in the progressive impairment of sound conduction and loss of cochlear, vestibular, and facial nerve function. There is a decrease of the arterial blood supply to the brain and an obstruction of the venous drainage from it. The histopathology reveals the obstacles to decompression of the middle ear cleft, ossicular chain, internal auditory and facial canals, and the risks, and in many instances the contraindications, to such procedures. On the other hand, decompression of the sigmoid sinus and jugular bulb should be considered as an additional life-saving procedure in conjunction with the prophylactic craniotomy recommended in all adult patients.

  13. Computed tomography of temporal bone fractures and temporal region anatomy in horses.

    Science.gov (United States)

    Pownder, S; Scrivani, P V; Bezuidenhout, A; Divers, T J; Ducharme, N G

    2010-01-01

    In people, specific classifications of temporal bone fractures are associated with clinical signs and prognosis. In horses, similar classifications have not been evaluated and might be useful establishing prognosis or understanding pathogenesis of certain types of trauma. We hypothesized associations between temporal bone fracture location and orientation in horses detected during computed tomography (CT) and frequency of facial nerve (CN7) deficit, vestibulocochlear nerve (CN8) deficit, or temporohyoid osteoarthropathy (THO). Complex temporal region anatomy may confound fracture identification, and consequently a description of normal anatomy was included. All horses undergoing temporal region CT at our hospital between July 1998 and May 2008. Data were collected retrospectively, examiners were blinded, and relationships were investigated among temporal bone fractures, ipsilateral THO, ipsilateral CN7, or ipsilateral CN8 deficits by Chi-square or Fischer's exact tests. Seventy-nine horses had CT examinations of the temporal region (158 temporal bones). Sixteen temporal bone fractures were detected in 14 horses. Cranial nerve deficits were seen with fractures in all parts of the temporal bone (petrosal, squamous, and temporal) and, temporal bone fractures were associated with CN7 and CN8 deficits and THO. No investigated fracture classification scheme, however, was associated with specific cranial nerve deficits. Without knowledge of the regional anatomy, normal structures may be mistaken for a temporal bone fracture or vice versa. Although no fracture classification scheme was associated with the assessed clinical signs, simple descriptive terminology (location and orientation) is recommended for reporting and facilitating future comparisons.

  14. European status on temporal bone training

    DEFF Research Database (Denmark)

    Frithioff, Andreas; Sørensen, Mads Sølvsten; Andersen, Steven Arild Wuyts

    2018-01-01

    laboratory facilities for training seems to be decreasing. Alternatives to traditional training can consist of drilling artificial models made of plaster or plastic but also virtual reality (VR) simulation. Nevertheless, the integration and availability of these alternatives into specialist training programs...... training modality. CONCLUSIONS: VR simulation and artificial models are reported to be used at many leading training departments already. Decreasing availability of cadavers, lower costs of VR simulation and artificial models, in addition to established evidence for a positive effect on the trainees......' competency, were reported as the main reasons. Most remaining departments expect to implement VR simulation and artificial models for temporal bone training into their residency programs in the near future....

  15. Chemodectomas arising in temporal bone structures

    International Nuclear Information System (INIS)

    Dickens, W.J.; Million, R.R.; Cassisi, N.J.; Singleton, G.T.

    1982-01-01

    Eighteen patients with chemodectomas arising in temporal bone structures were evaluated and treated at the University of Florida. Seventeen patients have each been followed a minimum of 3 years. Patients were retrospectively staged as having ''local'' or ''advanced'' disease, depending on the presence or absence of bone destruction and/or cranial nerve involvement. Fourteen of the patients received radiation therapy as all or part of their therapy; 6 patients were treated with radiation therapy alone, 3 patients were irradiated immediately postoperatively for residual disease, and 5 patients had radiation therapy for recurrence after operation. They were treated with cobalt-60 radiation with doses ranging from 3760 to 5640 rad. All irradiated patients demonstrated evidence of tumor regression, and none have had tumor recurrence with followup of 3-12 years. Of the 8 patients with cranial nerve paralysis prior to therapy, 5 had return of function of 1 or more cranial nerves. One of 6 patients treated initially with radiation therapy had a complication, while 6 of 8 patients irradiated postoperatively had complications. None of the complications were fatal. Three patients treated by operation for early disease limited to the hypotympanum had the disease controlled for 11-12 years. Guidelines for the selection of initial therapy are discussed

  16. Radiation injury to the temporal bone

    International Nuclear Information System (INIS)

    Guida, R.A.; Finn, D.G.; Buchalter, I.H.; Brookler, K.H.; Kimmelman, C.P.

    1990-01-01

    Osteoradionecrosis of the temporal bone is an unusual sequela of radiation therapy to the head and neck. Symptoms occur many years after the radiation is administered, and progression of the disease is insidious. Hearing loss (sensorineural, conductive, or mixed), otalgia, otorrhea, and even gross tissue extrusion herald this condition. Later, intracranial complications such as meningitis, temporal lobe or cerebellar abscess, and cranial neuropathies may occur. Reported here are five cases of this rare malady representing varying degrees of the disease process. They include a case of radiation-induced necrosis of the tympanic ring with persistent squamous debris in the external auditory canal and middle ear. Another case demonstrates the progression of radiation otitis media to mastoiditis with bony sequestration. Further progression of the disease process is seen in a third case that evolved into multiple cranial neuropathies from skull base destruction. Treatment includes systemic antibiotics, local wound care, and debridement in cases of localized tissue involvement. More extensive debridement with removal of sequestrations, abscess drainage, reconstruction with vascularized tissue from regional flaps, and mastoid obliteration may be warranted for severe cases. Hyperbaric oxygen therapy has provided limited benefit

  17. Chondrosarcoma of the temporal bone: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Park, Man Soo; Lee, Sang Youl; Chung, Jae Gul; Lee, Deok Hee; Jung, Seung Mun; Ryu, Dae Sik [Kang Nung Hospital, Ulsan Univ. Kangnung (Korea, Republic of)

    2001-07-01

    Chondrosarcoma of the temporal bone is a rare lesion. Clinically it has been confused with chordoma, glomus jugulare tumor and meningioma, among other conditions, and due to its anatomic location, cranial nerve palsy is frequently observed. We report a case involving a 50-year-old woman with chondrosarcoma of the temporal bone.

  18. Chondrosarcoma of the temporal bone: a case report

    International Nuclear Information System (INIS)

    Park, Man Soo; Lee, Sang Youl; Chung, Jae Gul; Lee, Deok Hee; Jung, Seung Mun; Ryu, Dae Sik

    2001-01-01

    Chondrosarcoma of the temporal bone is a rare lesion. Clinically it has been confused with chordoma, glomus jugulare tumor and meningioma, among other conditions, and due to its anatomic location, cranial nerve palsy is frequently observed. We report a case involving a 50-year-old woman with chondrosarcoma of the temporal bone

  19. Temporal Bone Fracture Causing Superior Semicircular Canal Dehiscence

    Directory of Open Access Journals (Sweden)

    Kevin A. Peng

    2014-01-01

    Full Text Available Importance. Superior semicircular canal dehiscence (SCD is a third window lesion of the inner ear causing symptoms of vertigo, autophony, tinnitus, and hearing loss. A “two-hit” hypothesis has traditionally been proposed, whereby thinly developed bone overlying the superior canal is disrupted by a sudden change in intracranial pressure. Although the symptoms of SCD may be precipitated by head injury, no previous reports have described a temporal bone fracture directly causing SCD. Observations. Two patients sustained temporal bone fractures after closed head trauma, and developed unilateral otologic symptoms consistent with SCD. In each instance, computed tomography imaging revealed fractures extending through the bony roof of the superior semicircular canal. Conclusions and Relevance. Temporal bone fractures, which are largely treated nonoperatively, have not previously been reported to cause SCD. As it is a potentially treatable entity, SCD resulting from temporal bone fracture must be recognized as a possibility and diagnosed promptly if present.

  20. Cadaveric Temporal Bone Dissection: Is It Obsolete Today?

    Directory of Open Access Journals (Sweden)

    Naik, Sulabha M.

    2014-01-01

    Full Text Available Introduction Traditionally, surgical training in otology, is imparted by dissecting harvested human cadaveric temporal bones. However, maintenance of a cadaveric temporal bone laboratory is expensive and carries risk of exposure to infection. In recent times, other modalities of training are gaining ground and are likely to eventually replace cadaveric temporal bone dissection altogether. Objectives Other alternative methods of training are emerging. New technology like simulation and virtual reality as high-fidelity, safer alternatives, are making rapid strides as teaching tools. Other options are the use of animal temporal bones as teaching tools. The advantages of these are compared. Data Synthesis None of these modalities can replicate the innumerable anatomical variations which are a characteristic feature of the human temporal bone. A novice surgeon not only needs exposure to surgical anatomy and it's variations but also needs to develop hand-eye coordination skills to gain expertise. Conclusion Deliberate practice on human cadaveric temporal bones only, will confer both mastery in anatomy and surgical technique. The human cadaveric temporal bone is ideal simulator for training in otology.

  1. CT features of fibrous dysplasia of the temporal bone

    International Nuclear Information System (INIS)

    Charrada-Ben Farhat, L.; Bourkhis, S.; Ben Yaacoub, I.; Dali, N.; Askri, A.; Hendaoui, L.

    2006-01-01

    Fibrous dysplasia is characterized by a progressive replacement of normal bone elements by fibrous tissue. The temporal bone is rarely involved. In this location, complications such as facial deformity, conductive hearing loss and facial peripheral neural involvement can occur. Positive diagnosis can be established with computerized tomography which also enables assessment of extension and detection of complications. We report a case of a 27-year-old man with extensive fibrous dysplasia of the right temporal bone presenting with conductive hearing loss secondary to progressive stenosis of the external auditory canal. Computerized tomography of the temporal region was performed. (authors)

  2. Bone scintigraphy in the diagnosis of fracture and infection of the temporal bone

    International Nuclear Information System (INIS)

    Djupesland, G.; Nakken, K.F.; Mueller, C.; Skjoerten, F.; Roehrt, T.; Eldevik, P.

    1983-01-01

    The sensivit of Tc99m-MDP-bone-scintiscanning in the diagnosis of temporal bone fracture was found to that of conventional radiography if the patients were examined 10 days after the trauma. Temporal bone osteomyelitis with concomitant moderate osteosclerosis was demonstrated by bone scintigraphy in 5 cases of mastoiditis with atypical symptoms. A case of apicitis was for the first time demonstrated by scintigraphy. A low sensivity of 67 Ga-scintigraphy was demonstrated by positive Tc99m-bone-scintigraphy and negative 67 Ga-scintigraphy in a patient with atypical mastoiditis. Tc99m-scintigraphy was negative in 5 cases of otitis media suppurative and in 3 cases of otitis media chronica cum cholesteatoma, all with slight degree of osteosclerosis in the mastoid. The sensitivity of Tc99m-bone-scintigraphy in fracture and osteomyelitis of the temporal bone seems to be a function of the amount of reactive new bone formed. (Authors)

  3. Temporal bone paragangliomas: 15 years experience

    Directory of Open Access Journals (Sweden)

    Mehmet Düzlü

    Full Text Available Abstract Introduction Temporal bone paragangliomas (TBPs are benign tumors arising from neural crest cells located along the jugular bulbus and the tympanic plexus. In general surgical excision, radiotherapy and wait-and-scan protocols are the main management modalities for TBPs. Objective In this paper we aim to present our clinical experience with TBPs and to review literature data. Methods The patients who were operated for tympanomastoid paraganglioma (TMP or tympanojugular paraganglioma (TJP in our clinic in the last 15 years were enrolled in the study. A detailed patient's charts review was performed retrospectively. Results There were 18 (52.9% cases with TMPs and 16 (47.1% cases with TJPs, a total of 34 patients operated for TBPs in this time period. The mean age was 50.3 ± 11.7 (range 25-71 years. The most common presenting symptoms were tinnitus and hearing loss for both TMPs and TJPs. Gross total tumor resection was achieved in 17 (94.4% and 10 (62.5% cases for TMPs and TJPs, respectively. Five patients (31.2% with TJP experienced facial palsy following the operation. For all the patients the mean follow-up period was 25.8 months (range 4-108 months. Conclusion In conclusion, based on our findings and literature review, total surgical excision alone or with preoperative embolization is the main treatment modality for TBPs. However radiotherapy, observation protocol and subtotal resection must be considered in cases of preoperative functioning cranial nerves, large tumors and advanced age.

  4. Temporal bone paragangliomas: 15 years experience.

    Science.gov (United States)

    Düzlü, Mehmet; Tutar, Hakan; Karamert, Recep; Karaloğlu, Furkan; Şahin, Muammer Melih; Göcek, Mehmet; Uğur, Mehmet Birol; Göksu, Nebil

    2016-12-08

    Temporal bone paragangliomas (TBPs) are benign tumors arising from neural crest cells located along the jugular bulbus and the tympanic plexus. In general surgical excision, radiotherapy and wait-and-scan protocols are the main management modalities for TBPs. In this paper we aim to present our clinical experience with TBPs and to review literature data. The patients who were operated for tympanomastoid paraganglioma (TMP) or tympanojugular paraganglioma (TJP) in our clinic in the last 15 years were enrolled in the study. A detailed patient's charts review was performed retrospectively. There were 18 (52.9%) cases with TMPs and 16 (47.1%) cases with TJPs, a total of 34 patients operated for TBPs in this time period. The mean age was 50.3± 11.7 (range 25-71 years). The most common presenting symptoms were tinnitus and hearing loss for both TMPs and TJPs. Gross total tumor resection was achieved in 17 (94.4%) and 10 (62.5%) cases for TMPs and TJPs, respectively. Five patients (31.2%) with TJP experienced facial palsy following the operation. For all the patients the mean follow-up period was 25.8 months (range 4-108 months). In conclusion, based on our findings and literature review, total surgical excision alone or with preoperative embolization is the main treatment modality for TBPs. However radiotherapy, observation protocol and subtotal resection must be considered in cases of preoperative functioning cranial nerves, large tumors and advanced age. Copyright © 2016 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

  5. Post traumatic facial nerve palsy without temporal bone fracture

    International Nuclear Information System (INIS)

    Scuotto, A.; Cappabianca, S.; Capasso, R.; Porto, A.; D'Oria, S.; Rotondo, M.

    2016-01-01

    Facial nerve injury following head trauma is a frequent event with or without temporal bone fractures. Computed tomography is the imaging modality of choice for assessing the possible bone disruption of the facial nerve canal. Magnetic resonance is helpful in presence of a facial nerve paralysis, unexplained by computed tomography findings. We present a case of delayed post-traumatic facial nerve palsy without radiological evidence of temporal bone fractures, in which magnetic resonance was crucial for diagnosing the nerve impairment. Radiological findings in accordance both with electrodiagnostic tests and clinical presentation suggested the successful conservative management. - Highlights: • Facial nerve is more prone to damage than any other cranial nerve after trauma. • Facial nerve trauma is usually associated with temporal bone fractures. • MRI is mandatory in case of no evidence of bone disruption at CT.

  6. Surgical anatomy of the temporal bone: an atlas

    International Nuclear Information System (INIS)

    Chan, L.L.; Manolidis, S.; Taber, K.H.; Hayman, L.A.

    2001-01-01

    This atlas demonstrates the usefulness of reconstructed high-resolution CT for planning temporal bone surgery. The first part focuses on a sagittal plane, the second on a rotated longitudinal plane, and the third on a rotated transverse plane. We believe knowledge of temporal bone anatomy in these planes facilitates surgical planning by showing anatomic relationships and providing a customized map for each patient. This decreases the likelihood of surgical mishap and improves teaching. (orig.)

  7. Case analysis of temporal bone lesions with facial paralysis as main manifestation and literature review.

    Science.gov (United States)

    Chen, Wen-Jing; Ye, Jing-Ying; Li, Xin; Xu, Jia; Yi, Hai-Jin

    2017-08-23

    This study aims to discuss clinical characteristics, image manifestation and treatment methods of temporal bone lesions with facial paralysis as the main manifestation for deepening the understanding of such type of lesions and reducing erroneous and missed diagnosis. The clinical data of 16 patients with temporal bone lesions and facial paralysis as main manifestation, who were diagnosed and treated from 2009 to 2016, were retrospectively analyzed. Among these patients, six patients had congenital petrous bone cholesteatoma (PBC), nine patients had facial nerve schwannoma, and one patient had facial nerve hemangioma. All the patients had an experience of long-term erroneous diagnosis. The lesions were completely excised by surgery. PBC and primary facial nerve tumors were pathologically confirmed. Facial-hypoglossal nerve anastomosis was performed on two patients. HB grade VI was recovered to HB grade V in one patient. The anastomosis failed due to severe facial nerve fibrosis in one patient. Hence, HB remained at grade VI. Postoperative recovery was good for all patients. No lesion recurrence was observed after 1-6 years of follow-up. For the patients with progressive or complete facial paralysis, imaging examination should be perfected in a timely manner. Furthermore, PBC, primary facial nerve tumors and other temporal bone space-occupying lesions should be eliminated. Lesions should be timely detected and proper intervention should be conducted, in order to reduce operation difficulty and complications, and increase the opportunity of facial nerve function reconstruction.

  8. IgG4-Related Disease of Bilateral Temporal Bones.

    Science.gov (United States)

    Li, Lilun; Ward, Bryan; Cocks, Margaret; Kheradmand, Amir; Francis, Howard W

    2017-03-01

    IgG4-related disease (IgG4-RD) is an idiopathic inflammatory condition that causes pseudotumor formation in single or multiple organs, including those of the head and neck. Temporal bone involvement is rare, with only 3 cases of unilateral temporal bone IgG4-RD described in the literature. We report the first known case of IgG4-RD of bilateral temporal bones and describe its clinical presentation, diagnosis, and treatment. The patient was a 52-year-old man with latent tuberculosis (TB) who presented with a 10-year history of bilateral profound hearing loss and vestibular dysfunction. Computed tomography and magnetic resonance imaging demonstrated bilateral labyrinthine destruction with invasion of the posterior fossa. Immunoglobulin level testing showed elevated total serum IgG levels with normal IgG4 levels. Bilateral mastoidectomies were performed, with biopsy samples demonstrating IgG4 staining with IgG4-positive plasma cells up to 40/HPF (high power field) on the right and 20/HPF on the left, consistent with bilateral IgG4-RD. IgG4-RD of bilateral temporal bones presents with chronic and progressive bilateral hearing loss and vestibular dysfunction. Clinical presentation and radiologic findings are nonspecific, and definitive diagnosis must be made with histopathology and immunostaining. Corticosteroids are therapeutic, but surgical resection may be necessary for temporal bone IgG4-RD to improve long-term remission.

  9. Anatomical study of the pigs temporal bone by microdissection.

    Science.gov (United States)

    Garcia, Leandro de Borborema; Andrade, José Santos Cruz de; Testa, José Ricardo Gurgel

    2014-01-01

    Initial study of the pig`s temporal bone anatomy in order to enable a new experimental model in ear surgery. Dissection of five temporal bones of Sus scrofa pigs obtained from UNIFESP - Surgical Skills Laboratory, removed with hole saw to avoid any injury and stored in formaldehyde 10% for better conservation. The microdissection in all five temporal bone had the following steps: inspection of the outer part, external canal and tympanic membrane microscopy, mastoidectomy, removal of external ear canal and tympanic membrane, inspection of ossicular chain and middle ear. Anatomically it is located at the same position than in humans. Some landmarks usually found in humans are missing. The tympanic membrane of the pig showed to be very similar to the human, separating the external and the middle ear. The middle ear`s appearance is very similar than in humans. The ossicular chain is almost exactly the same, as well as the facial nerve, showing the same relationship with the lateral semicircular canal. The temporal bone of the pigs can be used as an alternative for training in ear surgery, especially due the facility to find it and its similarity with temporal bone of the humans.

  10. Intra-temporal facial nerve centerline segmentation for navigated temporal bone surgery

    NARCIS (Netherlands)

    Voormolen, E.H.J.; Stralen, van M.; Woerdeman, P.A.; Pluim, J.P.W.; Noordmans, H.J.; Regli, L.; Berkelbach van der Sprenkel, J.W.; Viergever, M.A.; Wong, K.H.; Holmes III, D.R.

    2011-01-01

    Approaches through the temporal bone require surgeons to drill away bone to expose a target skull base lesion while evading vital structures contained within it, such as the sigmoid sinus, jugular bulb, and facial nerve. We hypothesize that an augmented neuronavigation system that continuously

  11. CT diagnosis and differential diagnosis of otodystrophic lesions of the temporal bone

    Energy Technology Data Exchange (ETDEWEB)

    D' Archambeau, O.; Parizel, P.M.; Schepper, A.M. De (Antwerp University Hospital (Belgium). Department of Radiology); Koekelkoren, E.; Van De Heyning, P. (Antwerp University Hospital (Belgium). Department of E.N.T.)

    The purpose of this study was to assess the diagnostic and differential diagnostic value of high-resolution computed tomography in the evaluation of temporal-bone dystrophies. The study group included 55 patients with osseous abnormalities of the temporal bone in general, and the labyrinthine capsule in particular. In 27 patients the CT scan revealed evidence of otodystrophic lesions. The CT findings in patients with otosclerosis (21 patients), osteogenesis imperfecta (two patients), fibrous dysplasia (one patient). Paget's disease (one patient) and osteoporosis (two patients) are described. The CT scans of 17 patients revealed secondary osseous lesions due to metastasis (five patients), post-inflammatory changes (10 patients) or labyrinthitis ossificans (two patients). Normal variants and congenital mineralization defects were diagnosed in nine patients, Down's syndrome in two. Our results indicate the importance of high-resolution computed tomography as the primary imaging modality in evaluating osseous lesions of the temporal bone and labyrinth. (author). 14 refs.; 13 figs; 2 tabs.

  12. A case of osteoradionecrosis of the temporal bone

    International Nuclear Information System (INIS)

    Okuno, Hideji; Saito, Yozo; Katori, Kimiaki; Hata, Yuko

    1983-01-01

    A case of osteoradionecrosis of the temporal bone was described. The patient had received radiotherapy for her nasopharyngeal cancer. Her symptoms of osteoradionecrosis of the temporal bone, otorrhea and earache, first appeared 5 years after her radiotherapy. Since then, conservative therapy was continued for 5 years, but her symptoms were not controlled with it. Ten years after her radiotherapy, the symptoms worsened rapidly, and the pain became intolerable, which necessitated mastoidectomy. After this surgery, she was free of symptoms until now. Based on this case, we discussed the pathologic, diagnostic and therapeutic aspects of osteoradionecrosis of the temporal bone. Emphasis was placed on the possibility of the occurence of this pathologic condition among all the patients who have received radiotherapy for malignant tumors of the head and neck or brain. (author)

  13. Massive Cerebrospinal Fluid Leak of the Temporal Bone

    Directory of Open Access Journals (Sweden)

    Giannicola Iannella

    2016-01-01

    Full Text Available Cerebrospinal fluid (CSF leakage of the temporal bone region is defined as abnormal communications between the subarachnoidal space and the air-containing spaces of the temporal bone. CSF leak remains one of the most frequent complications after VS surgery. Radiotherapy is considered a predisposing factor for development of temporal bone CSF leak because it may impair dural repair mechanisms, thus causing inadequate dural sealing. The authors describe the case of a 47-year-old man with a massive effusion of CSF which extended from the posterior and lateral skull base to the first cervical vertebrae; this complication appeared after a partial enucleation of a vestibular schwannoma (VS with subsequent radiation treatment and second operation with total VS resection.

  14. Conventional diagnostic imaging of the temporal bone. A historical review

    International Nuclear Information System (INIS)

    Canigiani, G.

    1997-01-01

    The Viennese Medical School played an important role in the development of radiological examinations and signs of the temporal bone with conventional X-rays. Famous pioneers include E.G. Mayer (1893-1969) and L. Psenner (1910-1986). Nowadays conventional X-rays and tomography have lost their important role in diagnostic radiology of the temporal bone, but the basic principles established in those early years of radiology are still used now. This statement is correct not only for conventional X-rays, but particularly for 'poly'-tomography in comparison with CT. (orig.) [de

  15. Aspects of temporal bone anatomy and pathology in conjunction with cochlear implant surgery

    Energy Technology Data Exchange (ETDEWEB)

    Stjernholm, Christina [Karolinska Inst., Stockholm (Sweden). Soedersjukhuset

    2003-07-01

    Cochlear implantation is a treatment for patients with severe sensorineural hearing loss/deafness, who get no help from ordinary hearing aids. The cochlear implant is surgically placed under the skin near the ear and a very thin electrode array is introduced into the cochlea of the inner ear, where it stimulates the remaining nerve fibers. The operation is complicated; it is performed with the aid of a microscope, and involves drilling very close to vital vessels and important nerves. High resolution computed tomography (CT) of the temporal bone is a part of the preoperative evaluation preceding cochlear implantation. It is a method for visualizing the bony structures of the middle and inner ear - to diagnose pathology and to describe the anatomy. The first work concerns CT of the temporal bone and cochlear implant surgery in children with CHARGE association. This is a rare condition with multiple congenital abnormalities, sometimes lethal. Children with CHARGE have different combinations of disabilities, of which impairments of vision and hearing, as well as balance problems and facial palsy can lead to developmental delay. There have been few reports of radiological temporal bone changes and none of cochlear implant surgery for this group. The work includes a report of the findings on preoperative CT and at surgery, as well as postimplant results in two children. A review of the latest diagnostic criteria of CHARGE and the temporal bone changes found in international literature is also included. The conclusion was that certain combinations of temporal bone changes in CHARGE are, if not specific, at least extremely rare in other materials. CT can visualize these changes and be used as a diagnostic tool. This is important, since some of the associated disabilities are not so obvious from the start. Early treatment is vital for the child's development. This work also shows that cochlear implantation may help some of these often very isolated children to

  16. Aspects of temporal bone anatomy and pathology in conjunction with cochlear implant surgery

    International Nuclear Information System (INIS)

    Stjernholm, Christina

    2003-01-01

    Cochlear implantation is a treatment for patients with severe sensorineural hearing loss/deafness, who get no help from ordinary hearing aids. The cochlear implant is surgically placed under the skin near the ear and a very thin electrode array is introduced into the cochlea of the inner ear, where it stimulates the remaining nerve fibers. The operation is complicated; it is performed with the aid of a microscope, and involves drilling very close to vital vessels and important nerves. High resolution computed tomography (CT) of the temporal bone is a part of the preoperative evaluation preceding cochlear implantation. It is a method for visualizing the bony structures of the middle and inner ear - to diagnose pathology and to describe the anatomy. The first work concerns CT of the temporal bone and cochlear implant surgery in children with CHARGE association. This is a rare condition with multiple congenital abnormalities, sometimes lethal. Children with CHARGE have different combinations of disabilities, of which impairments of vision and hearing, as well as balance problems and facial palsy can lead to developmental delay. There have been few reports of radiological temporal bone changes and none of cochlear implant surgery for this group. The work includes a report of the findings on preoperative CT and at surgery, as well as postimplant results in two children. A review of the latest diagnostic criteria of CHARGE and the temporal bone changes found in international literature is also included. The conclusion was that certain combinations of temporal bone changes in CHARGE are, if not specific, at least extremely rare in other materials. CT can visualize these changes and be used as a diagnostic tool. This is important, since some of the associated disabilities are not so obvious from the start. Early treatment is vital for the child's development. This work also shows that cochlear implantation may help some of these often very isolated children to communicate

  17. Bone Density Development of the Temporal Bone Assessed by Computed Tomography.

    Science.gov (United States)

    Takahashi, Kuniyuki; Morita, Yuka; Ohshima, Shinsuke; Izumi, Shuji; Kubota, Yamato; Horii, Arata

    2017-12-01

    The temporal bone shows regional differences in bone development. The spreading pattern of acute mastoiditis shows age-related differences. In infants, it spreads laterally and causes retroauricular swelling, whereas in older children, it tends to spread medially and causes intracranial complications. We hypothesized that bone maturation may influence the spreading pattern of acute mastoiditis. Eighty participants with normal hearing, aged 3 months to 42 years, participated in this study. Computed tomography (CT) values (Hounsfield unit [HU]) in various regions of the temporal bone, such as the otic capsule (OC), lateral surface of the mastoid cavity (LS), posterior cranial fossa (PCF), and middle cranial fossa (MCF), were measured as markers of bone density. Bone density development curves, wherein CT values were plotted against age, were created for each region. The age at which the CT value exceeded 1000 HU, which is used as an indicator of bone maturation, was calculated from the development curves and compared between the regions. The OC showed mature bone at birth, whereas the LS, PCF, and MCF showed rapid maturation in early childhood. However, there were significant regional differences in the ages of maturation: 1.7, 3.9, and 10.8 years for the LS, PCF, and MCF, respectively. To our knowledge, this is the first report to show regional differences in the maturation of temporal bone, which could partly account for the differences in the spreading pattern of acute mastoiditis in individuals of different ages.

  18. A Novel Temporal Bone Simulation Model Using 3D Printing Techniques.

    Science.gov (United States)

    Mowry, Sarah E; Jammal, Hachem; Myer, Charles; Solares, Clementino Arturo; Weinberger, Paul

    2015-09-01

    An inexpensive temporal bone model for use in a temporal bone dissection laboratory setting can be made using a commercially available, consumer-grade 3D printer. Several models for a simulated temporal bone have been described but use commercial-grade printers and materials to produce these models. The goal of this project was to produce a plastic simulated temporal bone on an inexpensive 3D printer that recreates the visual and haptic experience associated with drilling a human temporal bone. Images from a high-resolution CT of a normal temporal bone were converted into stereolithography files via commercially available software, with image conversion and print settings adjusted to achieve optimal print quality. The temporal bone model was printed using acrylonitrile butadiene styrene (ABS) plastic filament on a MakerBot 2x 3D printer. Simulated temporal bones were drilled by seven expert temporal bone surgeons, assessing the fidelity of the model as compared with a human cadaveric temporal bone. Using a four-point scale, the simulated bones were assessed for haptic experience and recreation of the temporal bone anatomy. The created model was felt to be an accurate representation of a human temporal bone. All raters felt strongly this would be a good training model for junior residents or to simulate difficult surgical anatomy. Material cost for each model was $1.92. A realistic, inexpensive, and easily reproducible temporal bone model can be created on a consumer-grade desktop 3D printer.

  19. Papercraft temporal bone in the first step of anatomy education.

    Science.gov (United States)

    Hiraumi, Harukazu; Sato, Hiroaki; Ito, Juichi

    2017-06-01

    (1) To compare temporal bone anatomy comprehension taught to speech therapy students with or without a papercraft model. (2) To explore the effect of papercraft simulation on the understanding of surgical approaches in first-year residents. (1) One-hundred and ten speech therapy students were divided into three classes. The first class was taught with a lecture only. The students in the second class were given a lecture and a papercraft modeling task without instruction. The third class modeled a papercraft with instruction after the lecture. The students were tested on their understanding of temporal bone anatomy. (2) A questionnaire on the understanding of surgical approaches was completed by 10 residents before and after the papercraft modeling. The papercraft models were cut with scissors to simulate surgical approaches. (1) The average scores were 4.4/8 for the first class, 4.3/8 for the second class, and 6.3/8 for the third class. The third class had significantly better results than the other classes (ppapercraft modeling and cutting were 2.6/7 and 4.9/7, respectively. The numerical rating scale score significantly improved (ppapercraft temporal bone model is effective in the first step of learning temporal bone anatomy and surgical approaches. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  20. Craniofacial and temporal bone CT findings in cleidocranial dysplasia

    International Nuclear Information System (INIS)

    Gonzalez, Guido E.; Caruso, Paul A.; Curtin, Hugh D.; Small, Juan E.; Jyung, Robert W.; Troulis, Maria J.

    2008-01-01

    Cleidocranial dysplasia (CCD) is a multistructural polyostotic genetic disorder that results from mutation of the CBFA1 gene. Hearing loss is a frequent finding in CCD. We describe the CT craniofacial findings in CCD and provide a comprehensive discussion of the CT temporal bone findings in these patients. (orig.)

  1. [Progressive bone lengthening of the hand in congenital malformations. 41 cases].

    Science.gov (United States)

    Foucher, G; Pajardi, G; Lamas, C; Medina, J; Navarro, R

    2001-09-01

    We retrospectively reviewed the experience of two Hand Units with progressive bone distraction lengthening, collecting 41 cases of hand skeleton lengthening for congenital malformations. The Ilizarov callostasis method was used in 31 cases and in 10 cases bone union was reestablished at a second stage with an iliac graft (2 cases), vascularized metacarpal bone graft (one case), and vascularized (one case) or nonvascularized (3 cases) toe epiphysis. In the last three cases of index lengthening, the distal part was translocated to the tip of the third, deepening at the same stage the first web. The most frequently treated malformation was symbrachydactyly (22 cases). Mean lengthening was 2.3 cm (0.9 to 3.5) with a mean treatment duration of 3.8 months (1.5 - 8.2). The "lengthening index" was 0.59. There was a significant difference between phalanx and metacarpal lengthening, but the amount of lengthening or treatment duration were not affected by technique (Ilizarov vs bone grafting) or age. The complication rate was 32%. There were two complete failures, one extensor tendon tear, 3 pin tract infections (one requiring interruption of the lengthening), 2 cases of relevant pain, 2 delayed unions, 2 angulations and 1 callus fracture, 1 metacarpophalangeal dislocation and 1 joint stiffness. Despite advances in micorsurgical toe transfer, there are still indications for bone lengthening in congenital malformations. The apparent simplicity of the technique can mask a certain number of complications, emphasizing the need for surgical experience. Progressive bone lengthening in congenital deformity has the advantage of preserving sensitivity and avoiding bone resorption. Callostasis does not increase the duration of treatment compared to bone graft.

  2. Multiple congenital brachymetatarsia. A one-stage combined shortening and lengthening procedure without iliac bone graft.

    Science.gov (United States)

    Kim, J S; Baek, G H; Chung, M S; Yoon, P W

    2004-09-01

    We performed nine metatarsal and three proximal phalangeal lengthenings in five patients with congenital brachymetatarsia of the first and one or two other metatarsal bones, by a one-stage combined shortening and lengthening procedure using intercalcary autogenous bone grafts from adjacent shortened metatarsal bones. Instead of the isolated lengthening of the first and the other metatarsal bones, we shortened the adjacent normal metatarsal and used the excised bone to lengthen the short toes, except for the great toe, to restore the normal parabola. One skin incision was used. All the operations were performed bilaterally and the patients were followed up for a mean period of 69.5 months (29 to 107). They all regained a nearly normal parabola and were satisfied with the cosmetic results. Our technique is straightforward and produces good cosmetic results. Satisfactory, bony union is achieved, morbidity is low, and no additional surgery is required for the removal of metal implants.

  3. Temporal bone dissection simulator for training pediatric otolaryngology surgeons

    Science.gov (United States)

    Tabrizi, Pooneh R.; Sang, Hongqiang; Talari, Hadi F.; Preciado, Diego; Monfaredi, Reza; Reilly, Brian; Arikatla, Sreekanth; Enquobahrie, Andinet; Cleary, Kevin

    2017-03-01

    Cochlear implantation is the standard of care for infants born with severe hearing loss. Current guidelines approve the surgical placement of implants as early as 12 months of age. Implantation at a younger age poses a greater surgical challenge since the underdeveloped mastoid tip, along with thin calvarial bone, creates less room for surgical navigation and can result in increased surgical risk. We have been developing a temporal bone dissection simulator based on actual clinical cases for training otolaryngology fellows in this delicate procedure. The simulator system is based on pre-procedure CT (Computed Tomography) images from pediatric infant cases (hospital. The simulator includes: (1) simulation engine to provide the virtual reality of the temporal bone surgery environment, (2) a newly developed haptic interface for holding the surgical drill, (3) an Oculus Rift to provide a microscopic-like view of the temporal bone surgery, and (4) user interface to interact with the simulator through the Oculus Rift and the haptic device. To evaluate the system, we have collected 10 representative CT data sets and segmented the key structures: cochlea, round window, facial nerve, and ossicles. The simulator will present these key structures to the user and warn the user if needed by continuously calculating the distances between the tip of surgical drill and the key structures.

  4. Cholesterol granuloma of temporal bone: CT and MR findings

    International Nuclear Information System (INIS)

    Ruiz Jauriguizuria, J.C.; Ferrero Collado, A.; Ereno Ealo, M.J.; Grande Icaran, D.

    1994-01-01

    Three cases of cholesterol granuloma of the left temporal bone are presented, two located in the petrous apex and the other at the otomastoid level. One of the patients had a history of mastoid surgery and the other surgery of the bone ridge. The three cases were confirmed histologically following surgical resection. The etiopathogenesis of the lesion is discussed, as are the clinical, histological and radiological features, with a broad description of the computed tomography and magnetic resonance findings. The differential diagnosis is also dealt with. (Author)

  5. [A temporal bone CT study of the infants with hearing loss referred from universal newborn hearing screening].

    Science.gov (United States)

    Tao, Zheng; Li, Yun; Hou, Zheng; Cheng, Lan

    2007-02-01

    To explore the high resolution CT image of temporal bone in infants with hearing loss, and its value in evaluating the cause of hearing loss. In 2005, 0.12 million newborns have been included in the hearing screening system in Shanghai, and 1077 infants have failed to pass the hearing screening. One hundred and eight four infants were diagnosed as congenital hearing loss from mild to profound. A temporal bone HRCT scanning was performed to these infants. Among the 184 patients with congenital hearing loss, HRCT showed that 26 cases (14.1%) were associated with external ear malformation, and 21 cases (11.4%) were associated with middle ear malformation, 31 cases (16.8%) associated with inner ear malformation. The patients with inner ear malformation included 12 cases with Mondini malformation, 1 case with common cavity malformation, 6 cases with large vestibule malformation, 5 cases with internal auditory canal abnormalities, and 10 cases with vestibule, semicircular canals abnormalities. In addition, there were 20 cases (10.8%) with fluid in middle ear. HRCT image play an important role in the differential diagnosis and treatment of infants with congenital hearing loss.

  6. Intra-temporal facial nerve centerline segmentation for navigated temporal bone surgery

    Science.gov (United States)

    Voormolen, Eduard H. J.; van Stralen, Marijn; Woerdeman, Peter A.; Pluim, Josien P. W.; Noordmans, Herke J.; Regli, Luca; Berkelbach van der Sprenkel, Jan W.; Viergever, Max A.

    2011-03-01

    Approaches through the temporal bone require surgeons to drill away bone to expose a target skull base lesion while evading vital structures contained within it, such as the sigmoid sinus, jugular bulb, and facial nerve. We hypothesize that an augmented neuronavigation system that continuously calculates the distance to these structures and warns if the surgeon drills too close, will aid in making safe surgical approaches. Contemporary image guidance systems are lacking an automated method to segment the inhomogeneous and complexly curved facial nerve. Therefore, we developed a segmentation method to delineate the intra-temporal facial nerve centerline from clinically available temporal bone CT images semi-automatically. Our method requires the user to provide the start- and end-point of the facial nerve in a patient's CT scan, after which it iteratively matches an active appearance model based on the shape and texture of forty facial nerves. Its performance was evaluated on 20 patients by comparison to our gold standard: manually segmented facial nerve centerlines. Our segmentation method delineates facial nerve centerlines with a maximum error along its whole trajectory of 0.40+/-0.20 mm (mean+/-standard deviation). These results demonstrate that our model-based segmentation method can robustly segment facial nerve centerlines. Next, we can investigate whether integration of this automated facial nerve delineation with a distance calculating neuronavigation interface results in a system that can adequately warn surgeons during temporal bone drilling, and effectively diminishes risks of iatrogenic facial nerve palsy.

  7. Temporal bone trauma and complications: computed tomography findings

    Energy Technology Data Exchange (ETDEWEB)

    Costa, Ana Maria Doffemond; Gaiotti, Juliana Oggioni; Couto, Caroline Laurita Batista; Gomes, Natalia Delage; Diniz, Renata Lopes Furletti Caldeira; Motta, Emilia Guerra Pinto Coelho, E-mail: anadoffemond@yahoo.com.br [Hospital Mater Dei, Belo Horizonte, MG (Brazil). Unit of Radiology and Imaging Diagnosis

    2013-03-15

    Most temporal bone fractures result from high-energy blunt head trauma, and are frequently related to other skull fractures or to polytrauma. Fractures and displacements of ossicular chain in the middle ear represent some of the main complications of temporal bone injury, and hence they will be more deeply approached in the present article. Other types of injuries include labyrinthine fractures, dural fistula, facial nerve paralysis and extension into the carotid canal. Computed tomography plays a fundamental role in the initial evaluation of polytrauma patients, as it can help to identify important structural injuries that may lead to severe complications such as sensorineural hearing loss, conductive hearing loss, dizziness and balance dysfunction, perilymphatic fistulas, facial nerve paralysis, vascular injury and others. (author)

  8. Temporal bone trauma and complications: computed tomography findings

    International Nuclear Information System (INIS)

    Costa, Ana Maria Doffemond; Gaiotti, Juliana Oggioni; Couto, Caroline Laurita Batista; Gomes, Natalia Delage; Diniz, Renata Lopes Furletti Caldeira; Motta, Emilia Guerra Pinto Coelho

    2013-01-01

    Most temporal bone fractures result from high-energy blunt head trauma, and are frequently related to other skull fractures or to polytrauma. Fractures and displacements of ossicular chain in the middle ear represent some of the main complications of temporal bone injury, and hence they will be more deeply approached in the present article. Other types of injuries include labyrinthine fractures, dural fistula, facial nerve paralysis and extension into the carotid canal. Computed tomography plays a fundamental role in the initial evaluation of polytrauma patients, as it can help to identify important structural injuries that may lead to severe complications such as sensorineural hearing loss, conductive hearing loss, dizziness and balance dysfunction, perilymphatic fistulas, facial nerve paralysis, vascular injury and others. (author)

  9. X-ray diagnosis in temporal bone anomalies

    International Nuclear Information System (INIS)

    Schratter, M.; Canigiani, G.; Swoboda, H.; Brunner, E.

    1988-01-01

    The X-ray findings in temporal bone anomalies are reviewed. Radiological procedure and examination technique are presented, as are symptoms of important anomalies. The methods available are plain film X-ray of temporal bone, multi-directional tomography, and high-resolution CT. Although some of the abnormalities are visible even in plain films, consistent use of conventional tomography or CT is necessary for correct diagnosis. This procedure is indicated not only when an abnormality is clinically obvious, but also in all cases of unexplained hearing loss without evidence of acquired disease. The advantage of CT over conventional tomography is that soft tissue anomalies, such as primary cholesteatoma or tumor simulating vascular abnormalities, can be demonstrated. In these cases CT is obligatory. (orig.) [de

  10. Computer tomography in children and adolescents with suspected malformation of the petrous portion of the temporal bone

    International Nuclear Information System (INIS)

    Koesling, S.; Schneider-Moebius, C.; Koenig, E.; Meister, E.F.

    1997-01-01

    Purpose: To demonstrate HRCT findings and their therapeutic relevance in suspected congenital hearing disorders. Material and Methods: It was checked in 96 young patients if HRCT findings of the temporal bone could explain functional findings. Furthermore, the therapeutic consequences were noted. Results: Normal CT and normal functional findings were obtained in 49 temporal bones (TB). In conductive hearing loss (41 TB), dysplasias of the conducting apparatus (37 TB) and inflammatory changes (3 TB) were found. Combined hearing loss (18 TB) was clarified completely or partially in half the cases. There were 22 dysplasias of the inner ear, 3 dysplasias of the middle ear, 1 adandoned examination (2 TB), and 55 normal CT findings in senorineural hearing disorders (82 TB). 1 retardate had a malformation of the inner ear and, contralaterally, inflammatory middle ear. In cases of vestibular disorders (24 TB), 14 malformations of the inner ear were detected. (orig./AJ) [de

  11. Three cases of temporal bone osteoradionecrosis after nasopharyngeal carcinoma treatment

    International Nuclear Information System (INIS)

    Yamatodani, Takashi; Mizuta, Kunihiro; Nakanishi, Hiroshi; Takizawa, Yoshinori; Hosokawa, Kumiko; Hosokawa, Seiji; Mineta, Hiroyuki

    2012-01-01

    Osteoradionecrosis is most commonly caused by radiation-induced injury. We report on 3 cases of temporal bone necrosis that occurred after chemoradiotherapy for nasopharyngeal carcinoma performed more than 10 years previously. Case 1 was a 42-year-old woman who had nasopharyngeal carcinoma in 1991. The patient underwent chemoradiotherapy (70 Gy total) in 1991, and gamma knife irradiation (20 Gy) in 1998 for local recurrence. The bone in the posterior wall of the left external auditory canal began to be exposed in 2003. Otorrhea from the left ear increased and we found a skin defect and ulcer formation in the postauricular region. We performed radical mastoidectomy and debridement on April, 2010. The area of the defect was covered and filled in with a pedicle musculoperiosteal flap. The intra-aural skin became dry in 6 months, however, she lost consciousness due to a temporal lobe abscess and underwent an emergency operation on April, 2011. After operation, the patient recovered with no neurological symptoms and infections up to the present date. Case 2 was a 58-year-old man who had nasopharyngeal carcinoma in 2001. The patient underwent chemoradiotherapy (66 Gy total) in 2001, and X knife irradiation (15 Gy) 3 months later due to the remaining tumor. The left posterior ear canal wall collapsed and the tympanic membrane retracted with pooling epithelial debris appearing in 2007. Left facial nerve palsy was seen in December 2010. We performed a mastoidectomy on January, 2011. Cholesteatoma and necrotic granuloma with fragile bone filled the mastoid cavity, and a facial canal bone defect was seen. Bone necrosis with cholesteatoma and inflammatory granuloma was revealed by the pathological examination. The facial palsy improved after the operation. Case 3 was a 59-year-old man who had left abducens palsy with nasopharyngeal carcinoma invading the clivus. The patient underwent chemoradiotherapy (60 Gy total) in 2001, and X knife irradiation (24 Gy) 4 months later for

  12. A metastatic glomus jugulare tumor. A temporal bone report

    International Nuclear Information System (INIS)

    El Fiky, F.M.; Paparella, M.M.

    1984-01-01

    The clinicopathologic findings in the temporal bone of a patient with a highly malignant metastasizing glomus jugulare tumor are reported. The patient exhibited all the symptoms of primary malignant tumors of the ear, including facial paralysis, otorrhea, pain, hearing loss, tinnitus, dizziness, and vertigo. He was treated with cobalt irradiation followed by radium implant in the ear canal for a residual tumor; then a left-sided radical mastoidectomy was performed

  13. Congenital pial arteriovenous fistula in the temporal region draining into cavernous sinus: A case report

    International Nuclear Information System (INIS)

    Zhang, Ziyin; Wang, Chaohua; Zhang, Changwei; Xie, Xiaodong; Wang, Kun; Tang, Jianjian

    2013-01-01

    This report concerns a 4-month-old infant with progressive prominent and redness of his left eye since birth. This report concerns a 4-month-old infant with progressive prominent redness of his left eye since birth. Angiography revealed a congenital pial arteriovenous fistula between the temporal branch of the left posterior cerebral artery and left cavernous sinus through the sphenoparietal sinus, a condition not reported in the literature. The fistula was successfully occluded with two micro-coils by vertebrobasilar approach.

  14. Classification of temporal bone pneumatization based on sigmoid sinus using computed tomography

    Energy Technology Data Exchange (ETDEWEB)

    Han, S.-J. [Department of Otorhinolaryngology, National Health Insurance Corporation Ilsan Hospital, Seoul (Korea, Republic of); Song, M.H. [Department of Otorhinolaryngology, Yonsei University College of Medicine, Kang-nam Gu, Do-gok Dong, 146-92, Seoul, Republic of Korea 135-720 (Korea, Republic of); Kim, J. [Department of Radiology, Yonsei University College of Medicine, Seoul (Korea, Republic of); Lee, W.-S. [Department of Otorhinolaryngology, Yonsei University College of Medicine, Kang-nam Gu, Do-gok Dong, 146-92, Seoul, Republic of Korea 135-720 (Korea, Republic of); Lee, H.-K. [Department of Otorhinolaryngology, Yonsei University College of Medicine, Kang-nam Gu, Do-gok Dong, 146-92, Seoul, Republic of Korea 135-720 (Korea, Republic of)], E-mail: hoki@yuhs.ac

    2007-11-15

    Aim: To analyse several reference structures using axial computed tomography (CT) imaging of the temporal bone, which may reflect pneumatization of the entire temporal bone by statistical correlation to the actual volume of the temporal bone measured using three-dimensional reconstruction. Materials and methods: One hundred and sixteen temporal bones were studied, comprising 48 with normal findings and 68 sides showing chronic otitis media or temporal bone fracture. After measuring the volume of temporal bone air cells by the volume rendering technique using three-dimensional reconstruction images, classification of temporal bone pneumatization was performed using various reference structures on axial images to determine whether significant differences in the volume of temporal bone air cells could be found between the groups. Results: When the sigmoid sinus at the level of the malleoincudal complex was used in the classification, there were statistically significant differences between the groups that correlated with the entire volume of the temporal bone. Grouping based on the labyrinth and the ascending carotid artery showed insignificant differences in volume. Furthermore, there was no significant correlation between the cross-sectional area of the antrum and the entire volume of the temporal bone. Conclusion: The degree of pneumatization of temporal bone can be estimated easily by the evaluation of the air cells around the sigmoid sinus on axial CT images.

  15. Primary Ewing's Sarcoma of the temporal bone in an infant.

    Science.gov (United States)

    Goudarzipour, Kourosh; Shamsian, Shahin; Alavi, Samin; Nourbakhsh, Kazem; Aghakhani, Roxana; Eydian, Zahra; Arzanian, Mohammad Taghi

    2015-04-01

    Introduction : Ewing's sarcoma is the second most common primary malignant tumor of bone found in children after Osteosarcoma. It accounts for 4-9% of primary malignant bone tumors and it affects bones of the skull or face in only 1-4% of cases. Hence it rarely affects the head and neck. Subject and Method : In this case report, we describe a case of primary Ewing's sarcoma occurring in the temporal bone. The tumor was surgically excised, and the patient underwent chemotherapy for ten months. Results : Neither recurrence nor distant metastasis was noted in these 10 months after surgery but about 18 months after surgery our patient was expired. Conclusion : Although the prognosis of Ewing's sarcoma is generally poor because of early metastasis to the lungs and to other bones, a review of the article suggested that Ewing's sarcoma occurring in the skull can often be successfully managed by intensive therapy with radical excision and chemotherapy. This result was supported by the case reported here.

  16. 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.

  17. Development of a robot for surgery of temporal bone

    International Nuclear Information System (INIS)

    Komune, Shizuo

    2011-01-01

    Described was development of a robot for drill-outing the mastoid process, the first essential step purposing such otologic surgery of temporal bone as tympanoplasty, cochlear implantation and tumor resection of auditory nerve, etc. A model of the bone prototyped by CT 3D data (Ono and Co., Ltd., Tokyo) was used for getting the trace of the drill-outing procedure by an expert, and information of the trace and bone position was registered by STAMP (surface template-assisted marker positioning), which was then integrated with a navigation system 3D slicer (a free, open source software) with use of data from position sensors of optical Polaris (NDI, Canada) and magnetic Aurora (NDI) on the drill tip. The sensors were also usable for recording the trace after the surgery as a log by MRI. The robot system was made to have thus 3 parts of drill-outing, operative navigation and control unit based on anatomical information. The drill-outing mechanic was made to have 6 degrees of freedom. Comparison of logs of the procedure conducted in the phantom bone by the robot and by an otologic operator gave agreement within error of 0.9 mm. More mechanical preciseness was thought desirable for reproducible operation. (author)

  18. Mixed reality temporal bone surgical dissector: mechanical design.

    Science.gov (United States)

    Hochman, Jordan Brent; Sepehri, Nariman; Rampersad, Vivek; Kraut, Jay; Khazraee, Milad; Pisa, Justyn; Unger, Bertram

    2014-08-08

    The Development of a Novel Mixed Reality (MR) Simulation. An evolving training environment emphasizes the importance of simulation. Current haptic temporal bone simulators have difficulty representing realistic contact forces and while 3D printed models convincingly represent vibrational properties of bone, they cannot reproduce soft tissue. This paper introduces a mixed reality model, where the effective elements of both simulations are combined; haptic rendering of soft tissue directly interacts with a printed bone model. This paper addresses one aspect in a series of challenges, specifically the mechanical merger of a haptic device with an otic drill. This further necessitates gravity cancelation of the work assembly gripper mechanism. In this system, the haptic end-effector is replaced by a high-speed drill and the virtual contact forces need to be repositioned to the drill tip from the mid wand. Previous publications detail generation of both the requisite printed and haptic simulations. Custom software was developed to reposition the haptic interaction point to the drill tip. A custom fitting, to hold the otic drill, was developed and its weight was offset using the haptic device. The robustness of the system to disturbances and its stable performance during drilling were tested. The experiments were performed on a mixed reality model consisting of two drillable rapid-prototyped layers separated by a free-space. Within the free-space, a linear virtual force model is applied to simulate drill contact with soft tissue. Testing illustrated the effectiveness of gravity cancellation. Additionally, the system exhibited excellent performance given random inputs and during the drill's passage between real and virtual components of the model. No issues with registration at model boundaries were encountered. These tests provide a proof of concept for the initial stages in the development of a novel mixed-reality temporal bone simulator.

  19. Repair of Temporal Bone Encephalocele following Canal Wall Down Mastoidectomy

    Directory of Open Access Journals (Sweden)

    Sarantis Blioskas

    2014-01-01

    Full Text Available We report a rare case of a temporal bone encephalocele after a canal wall down mastoidectomy performed to treat chronic otitis media with cholesteatoma. The patient was treated successfully via an intracranial approach. An enhanced layer-by-layer repair of the encephalocele and skull base deficit was achieved from intradurally to extradurally, using temporalis fascia, nasal septum cartilage, and artificial dural graft. After a 22-month follow-up period the patient remains symptom free and no recurrence is noted.

  20. Transmastoid approach to temporal bone cerebrospinal fluid leaks.

    Science.gov (United States)

    Oliaei, Sepehr; Mahboubi, Hossein; Djalilian, Hamid R

    2012-01-01

    The aim of the study was to evaluate various presentations and treatment options for spontaneous cerebrospinal fluid (CSF) leakage originating in the temporal bone. Clinical data and imaging results for 18 ears (15 patients) presenting with spontaneous CSF leakage originating in the temporal bone were reviewed. Average follow-up period was 13.5 months. The main outcome measure was presence of persistent CSF leak postoperatively. A standard postauricular mastoidectomy was performed. Fifteen patients diagnosed with spontaneous CSF leakage over an 8-year period including 3 treated for bilateral disease were included in the study. The age ranged between 33 and 83 years. Presenting symptoms included serous otitis media (44%), persistent otorrhea after tympanostomy tube placement (28%), and meningitis (28%). Preoperative diagnosis was made using imaging studies and was substantiated by observation of CSF leakage and dural herniation intraoperatively. Treatment was eustachian tube plugging (5%), mastoidectomy with fat obliteration (61%), middle fossa approach with extradural (17%), intradural repair (5%), or combined middle fossa and transmastoid (TM) approach (11%). Successful treatment was obtained in 17 of the 18 cases. The last 9 patients in the series underwent TM approach alone for repair with no treatment failures. Repair of defects in tegmen mastoideum and posterior fossa can be successfully achieved on an outpatient basis without regard to size and multitude of defects via TM approach. This approach obviates the need for a craniotomy or lumbar drain. Copyright © 2012 Elsevier Inc. All rights reserved.

  1. Osteoradionecrosis of the temporal bone with cancer of the middle ear. A case report

    International Nuclear Information System (INIS)

    Ishida, Katsunori; Sakai, Makoto; Shinkawa, Atsushi

    1999-01-01

    Osteoradionecrosis of the temporal bone may occur as a result of radiation therapy for head and neck carcinomas or brain tumors. A 64-year-old female received radiation therapy for squamous cell carcinoma of the middle ear 20 years ago, and then she developed osteoradionecrosis of the temporal bone. The patient underwent extensive debridement with removal of sequestrations in the temporal bone and adjacent areas, and abscess drainage. Her postoperative course was satisfactory and there was no progression of the disease. (author)

  2. Nonvisual spatial navigation fMRI lateralizes mesial temporal lobe epilepsy in a patient with congenital blindness.

    Science.gov (United States)

    Toller, Gianina; Adhimoolam, Babu; Grunwald, Thomas; Huppertz, Hans-Jürgen; König, Kristina; Jokeit, Hennric

    2015-01-01

    Nonvisual spatial navigation functional magnetic resonance imaging (fMRI) may help clinicians determine memory lateralization in blind individuals with refractory mesial temporal lobe epilepsy (MTLE). We report on an exceptional case of a congenitally blind woman with late-onset left MTLE undergoing presurgical memory fMRI. To activate mesial temporal structures despite the lack of visual memory, the patient was requested to recall familiar routes using nonvisual multisensory and verbal cues. Our findings demonstrate the diagnostic value of a nonvisual fMRI task to lateralize MTLE despite congenital blindness and may therefore contribute to the risk assessment for postsurgical amnesia in rare cases with refractory MTLE and accompanying congenital blindness.

  3. [Computed tomography of the temporal bone in diagnosis of chronic exudative otitis media].

    Science.gov (United States)

    Zelikovich, E I

    2005-01-01

    Computed tomography (CT) of the temporal bone was made in 37 patients aged 2 to 55 years with chronic exudative otitis media (CEOM). In 21 of them the pathology was bilateral. The analysis of 58 CT images has identified CT signs of chronic exudative otitis media. They include partial (17 temporary bones) or complete (38 temporal bones) block of the bone opening of the auditory tube, pneumatic defects of the tympanic cavity (58 temporal bones), pneumatic defects of the mastoid process and antrum (47 temporal bones), pathologic retraction of the tympanic membrane. The examination of the temporal bone detected both CT-signs of CEOM and other causes of hearing disorders in 14 patients (26 temporal bones) with CEOM symptoms and inadequately high hypoacusis. Among these causes were malformation of the auditory ossicula (n=5), malformation of the labynthine window (n=2), malformation of the middle and internal ear (n=4), a wide aqueduct of the vestibule, labyrinthine anomaly of Mondini's type (n=1), cochlear hypoplasia (n=4), stenosis of the internal acoustic meatuses (n=2). Sclerotic fibrous dysplasia was suggested in 2 temporal bones (by CT data). CT was repeated after surgical treatment of 10 patients (14 temporal bones) and visual assessment of tympanostomy results was made.

  4. A case of a temporal bone meningioma presenting as a serous otitis media

    International Nuclear Information System (INIS)

    Nicolay, Simon; De Foer, Bert; Bernaerts, Anja; Van Dinther, Joost; Parizel, Paul M

    2014-01-01

    We report the imaging features of a case of a temporal bone meningioma extending into the middle ear cavity and clinically presenting as a serous otitis media. Temporal bone meningioma extending in the mastoid or the middle ear cavity, however, is very rare. In case of unexplained or therapy-resistant serous otitis media and a nasopharyngeal tumor being ruled out, a temporal bone computed tomography (CT) should be performed. If CT findings are suggestive of a temporal bone meningioma, a magnetic resonance imaging (MRI) examination with gadolinium will confirm diagnosis and show the exact extension of the lesion

  5. Multi-material 3D Models for Temporal Bone Surgical Simulation.

    Science.gov (United States)

    Rose, Austin S; Kimbell, Julia S; Webster, Caroline E; Harrysson, Ola L A; Formeister, Eric J; Buchman, Craig A

    2015-07-01

    A simulated, multicolor, multi-material temporal bone model can be created using 3-dimensional (3D) printing that will prove both safe and beneficial in training for actual temporal bone surgical cases. As the process of additive manufacturing, or 3D printing, has become more practical and affordable, a number of applications for the technology in the field of Otolaryngology-Head and Neck Surgery have been considered. One area of promise is temporal bone surgical simulation. Three-dimensional representations of human temporal bones were created from temporal bone computed tomography (CT) scans using biomedical image processing software. Multi-material models were then printed and dissected in a temporal bone laboratory by attending and resident otolaryngologists. A 5-point Likert scale was used to grade the models for their anatomical accuracy and suitability as a simulation of cadaveric and operative temporal bone drilling. The models produced for this study demonstrate significant anatomic detail and a likeness to human cadaver specimens for drilling and dissection. Simulated temporal bones created by this process have potential benefit in surgical training, preoperative simulation for challenging otologic cases, and the standardized testing of temporal bone surgical skills. © The Author(s) 2015.

  6. The role of bone conduction hearing aids in congenital unilateral hearing loss: A systematic review.

    Science.gov (United States)

    Liu, C Carrie; Livingstone, Devon; Yunker, Warren K

    2017-03-01

    To systematically review the literature on the audiological and/or quality of life benefits of a bone conduction hearing aid (BCHA) in children with congenital unilateral conductive or sensorineural deafness. A systematic search was performed according to the PRISMA guidelines using the PubMed, Medline, and Embase databases. Data were collected on the following outcomes of interest: speech reception threshold, speech discrimination, sound localization, and quality of life measures. Given the heterogeneity of the data for quantitative analysis, the results are qualitatively summarized. Eight studies were included in the review. Four studies examined the audiological outcomes associated with bone conduction hearing aid implantation. There was a consistent gain in speech reception thresholds and speech discrimination, especially in noisy environments. Results pertaining to sound localization was inconsistent. The studies that examined quality of life measures reported a high usage rate of BCHAs among children. Quality of life improvements are reported with suggested benefit in the subdomain of learning. Given the potential benefits of a BCHA, along with the fact that it can be safely trialed using a headband, it is reasonable to trial a BCHA in children with congenital unilateral deafness. Should the trial offer audiological and/or quality of life benefits for the individual child, then BCHA implantation can be considered. Copyright © 2017 Elsevier B.V. All rights reserved.

  7. Study of a temporal bone of Homo heildelbergensis.

    Science.gov (United States)

    Urquiza, Rafael; Botella, Miguel; Ciges, Miguel

    2005-05-01

    The characteristic features of the Hh specimen conformed to those of other Pleistocene human fossils, indicating strong cranial structures and a heavy mandible. The mastoid was large and suggested a powerful sternocleidomastoid muscle. The inner ear and tympanic cavities were similar in size and orientation, suggesting that their functions were probably similar. Our observations suggest that the left ear of this Hh specimen was healthy. The large canaliculo-fenestral angle confirms that this ancestor was bipedal. It also strongly suggests that Hh individuals were predisposed to develop certain pathologies of the labyrinth capsule associated with bipedalism, in particular otosclerosis. We studied a temporal bone of Homo heidelbergensis (Hh) in order to investigate the clinical and physiological implications of certain morphological features, especially those associated with the evolutionary reorganization of the inner ear. The bone, found in a breach of a cave near MAáaga in southern Spain, together with Middle Upper Pleistocene faunal remains, is >300000 years old. Four analytical methods were employed. A 3D high-resolution surface laser scan was used for anatomical measurements. For the sectional analysis of the middle and inner ears of Hh we used high-resolution CT, simultaneously studying a normal temporal bone from Homo sapiens sapiens (Hss). To study the middle and inner ear spaces we used 3D reconstruction CT preceded by an intra-bone air shielding technique. To examine the tympanic cavities and measure the canaliculo fenestral angle, we used a special minimally invasive endoscopic procedure. The surface, sectional and 3D CT examinations showed that the Hh specimen was generally more robust and larger than the Hss specimen. It had a large glenoid fossa. The external meatus was wide and deep. The middle ear, and especially the mastoid, was large and widely pneumatized. There were no appreciable differences in the position and size of the labyrinthine spaces

  8. Busulfan and total body irradiation as antihematopoietic stem cell agents in the preparation of patients with congenital bone marrow disorders for allogenic bone marrow transplantation

    International Nuclear Information System (INIS)

    Parkman, R.; Rappeport, J.M.; Hellman, S.; Lipton, J.; Smith, B.; Geha, R.; Nathan, D.G.

    1984-01-01

    The capacity of busulfan and total body irradiation to ablate hematopoietic stem cells as preparation for the allogeneic bone marrow transplantation of patients with congenital bone marrow disorders was studied. Fourteen patients received 18 transplants; busulfan was used in the preparatory regimen of eight transplants and total body irradiation in the regimens of six transplants. Sustained hematopoietic ablation was achieved in six of eight patients prepared with busulfan and in all six patients prepared with total body irradiation. Three patients prepared with total body irradiation died with idiopathic interstitial pneumonitis, whereas no patients receiving busulfan developed interstitial pneumonitis. The optimal antihematopoietic stem cell agent to be used for the preparation of patients with congenital bone marrow disorder for bone marrow transplantation is not certain

  9. Evaluation on temporal bone CT findings of cholesteatoma

    International Nuclear Information System (INIS)

    Lee, Kun Won; Lee, Nam Joon; Kang, Eun Young; Chung, Kyoo Byung; Suh, Won Hyuck

    1989-01-01

    Cholesteatomas are thought to result from ingrowth of keratinizing squamous epithelium from external ear to middle ear. The cholesteatomas are usually diagnosed by clinical symptoms and signs, otoscopy, and plain radiograms. But various view points are emphasized radiologically before operation, leading to examine by computed tomography (CT), especially in complicated cases. We retrospectively reviewed the CT findings of cholesteatomas in 25 surgically proven cases during the period from May, 1983 to Aug, 1988. The results were as follows: 1. Most cholesteatomas showed soft tissue mass and bony erosion of ossicles (88%), attic wall and mastoid antrum (84%) on temporal bone CT scan. 2. The CT members of cholesteatomas ranged from 25 to 50 HU (avg. 33 HU). 3. Involved sites were attic (16%), antrum (28%), and both attic and antrum (56%). Other sites were middle ear cavity and external auditory canal. 4. Extra-tympanomastoid extension of cholesteatoma was intracranial abscess (8%), exposure of dural sinus (8%), and extension along with neck (4%)

  10. Three-dimensional measurement of temporal bone by using personal computer

    International Nuclear Information System (INIS)

    Kimura, Hiroki; Murata, Kiyotaka; Isono, Michio; Azuma, Hiroji; Itou, Akihiko

    1996-01-01

    Measurement of anatomical indices in human temporal bone has been reported only sporadically using high resolution CT. We developed a method for measuring such indices by computer assisted processing of images obtained by high resolusion CT. Intensive measurement of distances between all anatomical points in the entire temporal bone structure became possible with this method. (author)

  11. Anatomic and Quantitative Temporal Bone CT for Preoperative Assessment of Branchio-Oto-Renal Syndrome.

    Science.gov (United States)

    Ginat, D T; Ferro, L; Gluth, M B

    2016-12-01

    We describe the temporal bone computed tomography (CT) findings of an unusual case of branchio-oto-renal syndrome with ectopic ossicles that are partially located in the middle cranial fossa. We also describe quantitative temporal bone CT assessment pertaining to cochlear implantation in the setting of anomalous cochlear anatomy associated with this syndrome.

  12. Statistical model estimating the occurrence of otitis media from temporal bone pneumatization

    DEFF Research Database (Denmark)

    Homøe, P; Lynnerup, N; Rasmussen, N

    1994-01-01

    In order to investigate the relationship between the pneumatization of temporal bones and the occurrence of otitis media in Greenlandic Inuit, 36 Greenlandic Inuit were examined by radiography of the temporal bones. The pneumatized cell area was measured planimetrically. All subjects answered...

  13. Histology of sheep temporal bone A histologia do osso temporal do ovino

    Directory of Open Access Journals (Sweden)

    Hormy Biavatti Soares

    2011-06-01

    Full Text Available Previous studies suggest that there is an excellent correlation between the morphology and dimensions of ear structures in sheep and human beings. AIM: To analyze and describe the histology of structures inside the temporal bone in sheep. MATERIAL AND METHODS: A total of 307 slides obtained from vertical and horizontal sections of the temporal bone of eight sheep were analyzed. Structures were classified as similar or not similar to human structures, based on cellularity and histological architecture parameters. STUDY DESIGN: Experimental. RESULTS: The study revealed similarities between sheep and humans in terms of type of epithelium, bone component, spaces in the auditory meatus, in addition to a marked histological resemblance of cellularity and that of the structures surrounding the ear. The main differences observed were the presence of an anatomic bulla, the absence of aeration in the mastoid and the inferior opening of the hypotympanum into the bulla in sheep. CONCLUSION: Based on these observations, it is possible to conclude that sheep represent an adequate option for training and research in otologic surgery.Resultados prévios apontam para uma ótima correlação entre a morfologia e as dimensões das estruturas anatômicas de ovelhas e seres humanos. OBJETIVO: Analisar e descrever a histologia das estruturas que compõem o osso temporal do ovino. MATERIAL E MÉTODO: Um total de 307 lâminas obtidas a partir de secções verticais e horizontais do osso temporal de oito ovelhas foi analisado. As estruturas foram caracterizadas como semelhantes ou não semelhantes às do ser humano, com base na celularidade e na arquitetura histológica das estruturas. DESENHO CIENTÍFICO: Experimental. RESULTADOS: Constatou-se semelhança quanto ao tipo de epitélio, componente ósseo, espaços da fenda auditiva e arquitetura, além da semelhança, em nível histológico, tanto dos componentes celulares como das estruturas contíguas ao ouvido. As

  14. Bone marrow morphology and disease progression in congenital thrombocytopenia: a detailed clinicopathologic and genetic study of eight cases.

    Science.gov (United States)

    Tsang, Hamilton C; Bussel, James B; Mathew, Susan; Liu, Yen-Chun; Imahiyerobo, Allison A; Orazi, Attilio; Geyer, Julia T

    2017-04-01

    Patients with congenital thrombocytopenia have an increased risk of developing myeloid neoplasms. In these cases, the morphologic distinction between disease at baseline and at progression is challenging. This report analyzes clinicopathologic features of congenital thrombocytopenia with long-term follow-up at one referral center. Records from the last 20 years were searched for cases of congenital thrombocytopenia with bone marrow biopsies and peripheral blood smears. The clinical, morphologic, immunophenotypic, and molecular features were analyzed. Six adult and two pediatric patients were identified (six male, two female). Age range at first biopsy was 1-47 (median, 31) years. Underlying diseases included thrombocytopenia-absent radius syndrome, congenital thrombocytopenia with radial-ulnar synostosis, MYH9-related disorder, shortened telomere syndrome, congenital thrombocytopenia with ANKRD26 mutation, and familial platelet disorder with predisposition to acute myeloid leukemia. Four patients had myelodysplastic/myeloproliferative neoplasm-like marrow changes such as hypercellularity, increased myeloid to erythroid ratio, numerous micromegakaryocytes (highlighted by CD42b), and marrow fibrosis. Two patients had marrow hypoplasia and two had unremarkable marrow morphology. Three patients-all in the myelodysplastic/myeloproliferative neoplasm-like group-developed disease progression characterized by erythroid and myeloid dysplasia, elevated bone marrow blasts, and new cytogenetic abnormalities. Unlike non-familial myeloid neoplasms, congenital thrombocytopenia patients in the myelodysplastic/myeloproliferative neoplasm-like group had a long and indolent clinical course (average age at disease progression, 47 years). In summary, three distinct morphologic types of congenital thrombocytopenia were identified: a hyperplastic myelodysplastic/myeloproliferative neoplasm-like group, a hypoplastic bone marrow failure-like group, and a group with relatively normal marrow

  15. Primary pericranial Ewing's sarcoma on the temporal bone: A case report.

    Science.gov (United States)

    Kawano, Hiroto; Nitta, Naoki; Ishida, Mitsuaki; Fukami, Tadateru; Nozaki, Kazuhiko

    2016-01-01

    Primary Ewing's sarcoma originating in the pericranium is an extremely rare disease entity. A 9-year-old female patient was admitted to our department due to a left temporal subcutaneous mass. The mass was localized under the left temporal muscle and attached to the surface of the temporal bone. Head computed tomography revealed a mass with bony spicule formation on the temporal bone, however, it did not show bone destruction or intracranial invasion. F-18 fluorodeoxyglucose positron emission tomography showed no lesions other than the mass on the temporal bone. Magnetic resonance imaging showed that the mass was located between the temporal bone and the pericranium. The mass was completely resected with the underlying temporal bone and the overlying deep layer of temporal muscle, and was diagnosed as primary Ewing's sarcoma. Because the tumor was located in the subpericranium, we created a new classification, "pericranial Ewing's sarcoma," and diagnosed the present tumor as pericranial Ewing's sarcoma. We herein present an extremely rare case of primary pericranial Ewing's sarcoma that developed on the temporal bone.

  16. [Temporal variation and geographical distribution: congenital heart defects in the Comunitat Valenciana].

    Science.gov (United States)

    Cavero Carbonell, C; Zurriaga, O; Pérez Panadés, J; Barona Vilar, C; Martos Jiménez, C

    2013-09-01

    The objective of this study is to estimate the prevalence of congenital heart defects (CHD) in the Comunitat Valenciana (CV) in children less than one year old and identify whether there are temporal and geographic variations within this prevalence. The minimum basic data set from hospital discharge reports was used to select patients, who were born between 1999-2008, were less than one year old, and who lived in the CV with at least one hospital admission in which the primary diagnosis and/or any of the events were coded as CHD (codes 745-747 of the International Classification of Diseases 9th Revision Clinical Modification). The first hospital discharge report with CHD was selected, using the health card number to detect duplication. The prevalence and 95% confidence intervals were calculated, and the prevalence ratio (PR) and smoothed PR was obtained for each municipality to identify geographic patterns. In the period 1999-2008 there were 6.377 patients younger than one year with some CHD, representing the 43.2% of cases of congenital anomalies. The prevalence was 134.3 per 10.000 live births (95% CI: 131.1-137.6). There was a significant increase in the prevalence, from 115.8 in the 1999-2003 period to 149.5 in the 2004-2008 period. A higher risk was identified in the north of the CV, and in some municipalities of the province of Alicante, in the south. The observed increase in CHD agrees with the findings in other countries and it can be explained, at least in part, by improved diagnostic techniques. The geographic pattern identified requires a more detailed analysis that could explain the geographic variations found. Copyright © 2012 Asociación Española de Pediatría. Published by Elsevier Espana. All rights reserved.

  17. Techniques for small-bone lengthening in congenital anomalies of the hand and foot.

    Science.gov (United States)

    Minguella, J; Cabrera, M; Escolá, J

    2001-10-01

    The purpose of this study is to analyse three different lengthening techniques used in 31 small bones for congenital malformations of the hand and foot: 15 metacarpals, 12 metatarsals, 1 foot stump and 3 spaces between a previously transplanted phalanx end of the carpus or the metacarpal. Progressive lengthening with an external fixator device was performed in 23 cases: the callus distraction (callotasis) technique was used in 15 cases, whereas in the other 8 cases the speed of lengthening was faster and the defect bridged with a bone graft as a second stage. In another eight cases, a one-stage lengthening was performed. In the callotasis group, the total length gained ranged from 9 mm to 30 mm and the percentage of lengthening obtained (compared with the initial bone length) averaged 53.4%; in the fast lengthening group, the length gained ranged from 8 mm to 15 mm, and the average percentage of lengthening was 53.1%; and in the one-stage group, the length gained ranged from 7 mm to 15 mm, and the average percentage of lengthening was 43%. The overall complication rate was 22.5%.

  18. Endolymphatic Sac Tumors and Papillary Adenocarcinoma of the Temporal Bone:Role of MRI and CT

    OpenAIRE

    Mahmood F. Mafee; Hemant Shah

    2003-01-01

    Adenomatous Tumors of the temporal bone are rare. Benign adenomatous neoplasms (adenoma) of the middle ear are a distinctive benign tumor based on histological and clinical observations. Papillary adenocarcinomas of the temporal bone are invasive tumors. Although, the exact site of origin of these neoplasms is not identified, owing to the local bone destruction (usually centered at posterior petromastoid plate), the general consensus favors the endolymphatic sac as being the origin of these t...

  19. Primary Ewing's Sarcoma of the Temporal Bone: A Rare Case Report and Literature Review.

    Science.gov (United States)

    Gupta, Divya; Gulati, Achal; Purnima

    2017-09-01

    Ewing's sarcoma is a malignant, round cell tumor arising from the bones and primarily affecting children and adolescent, accounting for 3 % of all childhood malignancies. Although the long bones and the trunk are typically affected, rare cases of it involving isolated bones throughout the body have been reported. Involvement of the skull bones is rare, constituting 1-6 % of the total Ewing's sarcoma cases but those affecting the cranial bones are rarer still, constituting only 1 %. We describe an 8 months old infant having Ewing sarcoma, of the petrous and mastoid parts of temporal bone along with the occipital bone, whose clinical presentation mimicked mastoiditis with facial nerve palsy. We discuss the clinical and therapeutic course of an extensive primary Ewing sarcoma of the temporal bone, which was treated without performing surgery and review this entity's literature in detail.

  20. Cranial base morphology and temporal bone pneumatization in Asian Homo erectus.

    Science.gov (United States)

    Balzeau, Antoine; Grimaud-Hervé, Dominique

    2006-10-01

    The external morphological features of the temporal bone are used frequently to determine taxonomic affinities of fossils of the genus Homo. Temporal bone pneumatization has been widely studied in great apes and in early hominids. However, this feature is rarely examined in the later hominids, particularly in Asian Homo erectus. We provide a comparative morphological and quantitative analysis of Asian Homo erectus from the sites of Ngandong, Sambungmacan, and Zhoukoudian, and of Neandertals and anatomically modern Homo sapiens in order to discuss causes and modalities of temporal bone pneumatization during hominid evolution. The evolution of temporal bone pneumatization in the genus Homo is more complex than previously described. Indeed, the Zhoukoudian fossils have a unique pattern of temporal bone pneumatization, whereas Ngandong and Sambungmacan fossils, as well as the Neandertals, more closely resemble the modern human pattern. Moreover, these Chinese fossils are characterized by a wide midvault and a relatively narrow occipital bone. Our results support the point of view that cell development does not play an active role in determining cranial base morphology. Instead, pneumatization is related to available space and to temporal bone morphology, and its development is related to correlated morphology and the relative disposition of the bones and cerebral lobes. Because variation in pneumatization is extensive within the same species, the phyletic implications of pneumatization are limited in the taxa considered here.

  1. Assessment of skills using a virtual reality temporal bone surgery simulator.

    Science.gov (United States)

    Linke, R; Leichtle, A; Sheikh, F; Schmidt, C; Frenzel, H; Graefe, H; Wollenberg, B; Meyer, J E

    2013-08-01

    Surgery on the temporal bone is technically challenging due to its complex anatomy. Precise anatomical dissection of the human temporal bone is essential and is fundamental for middle ear surgery. We assessed the possible application of a virtual reality temporal bone surgery simulator to the education of ear surgeons. Seventeen ENT physicians with different levels of surgical training and 20 medical students performed an antrotomy with a computer-based virtual temporal bone surgery simulator. The ease, accuracy and timing of the simulated temporal bone surgery were assessed using the automatic assessment software provided by the simulator device and additionally with a modified Final Product Analysis Scale. Trained ENT surgeons, physicians without temporal bone surgical training and medical students were all able to perform the antrotomy. However, the highly trained ENT surgeons were able to complete the surgery in approximately half the time, with better handling and accuracy as assessed by the significant reduction in injury to important middle ear structures. Trained ENT surgeons achieved significantly higher scores using both dissection analysis methods. Surprisingly, there were no significant differences in the results between medical students and physicians without experience in ear surgery. The virtual temporal bone training system can stratify users of known levels of experience. This system can be used not only to improve the surgical skills of trained ENT surgeons for more successful and injury-free surgeries, but also to train inexperienced physicians/medical students in developing their surgical skills for the ear.

  2. Facial nerve paralysis associated with temporal bone masses.

    Science.gov (United States)

    Nishijima, Hironobu; Kondo, Kenji; Kagoya, Ryoji; Iwamura, Hitoshi; Yasuhara, Kazuo; Yamasoba, Tatsuya

    2017-10-01

    To investigate the clinical and electrophysiological features of facial nerve paralysis (FNP) due to benign temporal bone masses (TBMs) and elucidate its differences as compared with Bell's palsy. FNP assessed by the House-Brackmann (HB) grading system and by electroneurography (ENoG) were compared retrospectively. We reviewed 914 patient records and identified 31 patients with FNP due to benign TBMs. Moderate FNP (HB Grades II-IV) was dominant for facial nerve schwannoma (FNS) (n=15), whereas severe FNP (Grades V and VI) was dominant for cholesteatomas (n=8) and hemangiomas (n=3). The average ENoG value was 19.8% for FNS, 15.6% for cholesteatoma, and 0% for hemangioma. Analysis of the correlation between HB grade and ENoG value for FNP due to TBMs and Bell's palsy revealed that given the same ENoG value, the corresponding HB grade was better for FNS, followed by cholesteatoma, and worst in Bell's palsy. Facial nerve damage caused by benign TBMs could depend on the underlying pathology. Facial movement and ENoG values did not correlate when comparing TBMs and Bell's palsy. When the HB grade is found to be unexpectedly better than the ENoG value, TBMs should be included in the differential diagnosis. Copyright © 2017 Elsevier B.V. All rights reserved.

  3. Differential Intracochlear Sound Pressure Measurements in Normal Human Temporal Bones

    Science.gov (United States)

    Nakajima, Hideko Heidi; Dong, Wei; Olson, Elizabeth S.; Merchant, Saumil N.; Ravicz, Michael E.; Rosowski, John J.

    2009-02-01

    We present the first simultaneous sound pressure measurements in scala vestibuli and scala tympani of the cochlea in human cadaveric temporal bones. Micro-scale fiberoptic pressure sensors enabled the study of differential sound pressure at the cochlear base. This differential pressure is the input to the cochlear partition, driving cochlear waves and auditory transduction. Results showed that: pressure of scala vestibuli was much greater than scala tympani except at low and high frequencies where scala tympani pressure affects the input to the cochlea; the differential pressure proved to be an excellent measure of normal ossicular transduction of sound (shown to decrease 30-50 dB with ossicular disarticulation, whereas the individual scala pressures were significantly affected by non-ossicular conduction of sound at high frequencies); the middle-ear gain and differential pressure were generally bandpass in frequency dependence; and the middle-ear delay in the human was over twice that of the gerbil. Concurrent stapes velocity measurements allowed determination of the differential impedance across the partition and round-window impedance. The differential impedance was generally resistive, while the round-window impedance was consistent with a compliance in conjunction with distributed inertia and damping. Our techniques can be used to study inner-ear conductive pathologies (e.g., semicircular dehiscence), as well as non-ossicular cochlear stimulation (e.g., round-window stimulation) - situations that cannot be completely quantified by measurements of stapes velocity or scala-vestibuli pressure by themselves.

  4. Developing effective automated feedback in temporal bone surgery simulation.

    Science.gov (United States)

    Wijewickrema, Sudanthi; Piromchai, Patorn; Zhou, Yun; Ioannou, Ioanna; Bailey, James; Kennedy, Gregor; O'Leary, Stephen

    2015-06-01

    We aim to test the effectiveness, accuracy, and usefulness of an automated feedback system in facilitating skill acquisition in virtual reality surgery. We evaluate the performance of the feedback system through a randomized controlled trial of 24 students allocated to feedback and nonfeedback groups. The feedback system was based on the Melbourne University temporal bone surgery simulator. The study was conducted at the simulation laboratory of the Royal Victorian Eye and Ear Hospital, Melbourne. The study participants were medical students from the University of Melbourne, who were asked to perform virtual cortical mastoidectomy on the simulator. The extent to which the drilling behavior of the feedback and nonfeedback groups differed was used to evaluate the effectiveness of the system. Its accuracy was determined through a postexperiment observational assessment of recordings made during the experiment by an expert surgeon. Its usability was evaluated using students' self-reports of their impressions of the system. A Friedman's test showed that there was a significant improvement in the drilling performance of the feedback group, χ(2)(1) = 14.450, P feedback (when trainee behavior was detected) 88.6% of the time and appropriate feedback (accurate advice) 84.2% of the time. Participants' opinions about the usefulness of the system were highly positive. The automated feedback system was observed to be effective in improving surgical technique, and the provided feedback was found to be accurate and useful. © American Academy of Otolaryngology—Head and Neck Surgery Foundation 2015.

  5. Computed tomography of the temporal bone and orbit

    International Nuclear Information System (INIS)

    Zonneveld, F.W.

    1987-01-01

    The basis for this dissertation is the combination of the best set of high-resolution CT scanning parameters, on the one hand, and the technique of scanning perpendicular to the tissue interface, or parallel to an elongated anatomical structure (direct multiplanar CT technique) on the other. Although this technique yields better visualization of a number of anatomical details, the problem remains that the radiologist is as yet unfamiliar with these alternative cross-sectional planes. For this reason, a technique for cryosectioning fresh frozen specimens was selected and improved to create cross-sectional images that can be correlated with the direct multiplanar CT scans. The selection of special scan planes, the positioning, preparation and examination of the patient, and the CT and correlative anatomy are discussed separately for the temporal bone and the orbit. A few clinical applications are discussed. In the orbit, the value of high-resolution CT is demonstrated in the establishment of the relationship between space-occupying lesions and the optic nerve, and in the management of fractures of the orbital floor. 548 refs.; 253 figs.; 24 tabs

  6. Utility of 3D printed temporal bones in pre-surgical planning for complex BoneBridge cases.

    Science.gov (United States)

    Mukherjee, Payal; Cheng, Kai; Flanagan, Sean; Greenberg, Simon

    2017-08-01

    With the advent of single-sided hearing loss increasingly being treated with cochlear implantation, bone conduction implants are reserved for cases of conductive and mixed hearing loss with greater complexity. The BoneBridge (BB, MED-EL, Innsbruck, Austria) is an active fully implantable device with no attenuation of sound energy through soft tissue. However, the floating mass transducer (FMT) part of the device is very bulky, which limits insertion in complicated ears. In this study, 3D printed temporal bones of patients were used to study its utility in preoperative planning on complicated cases. Computed tomography (CT) scans of 16 ears were used to 3D print their temporal bones. Three otologists graded the use of routine preoperative planning provided by MED-EL and that of operating on the 3D printed bone of the patient. Data were collated to assess the advantage and disadvantage of the technology. There was a statistically significant benefit in using 3D printed temporal bones to plan surgery for difficult cases of BoneBridge surgery compared to the current standard. Surgeons preferred to have the printed bones in theatre to plan their drill sites and make the transition of the planning to the patient's operation more precise. 3D printing is an innovative use of technology in the use of preoperative planning for complex ear surgery. Surgical planning can be done on the patient's own anatomy which may help to decrease operating time, reduce cost, increase surgical precision and thus reduce complications.

  7. Congenital adrenal hyperplasia: a case report with premature teeth exfoliation and bone resorption.

    Science.gov (United States)

    Angelopoulou, Matina V; Kontogiorgos, Elias; Emmanouil, Dimitris

    2015-06-01

    Congenital adrenal hyperplasia (CAH) is an inherited autosomal recessive disorder characterized by insufficient production of cortisol. The aim of this case report was to present a child with CAH, premature exfoliation of primary teeth and accelerated eruption of his permanent teeth related to bone resorption. A 4.5-year-old Caucasian boy with CAH and long-term administration of glucocorticoids was referred for dental restoration. Clinical examination revealed primary molars with worn stainless steel crowns, severe attrition of the upper canines, and absence of the upper incisors. Before the completion of treatment, abnormal mobility of the first upper primary molars and the lower incisors was detected, and a few days later the teeth exfoliated prematurely. Histologic examination revealed normal tooth structure. Alkaline phosphatase and blood cells values were normal. Eruption of the permanent dentition was also accelerated. Tooth mobility was noticed in the permanent teeth as soon as they erupted, along with bone destruction. Examination revealed an elevated level of receptor activator of nuclear factor-κB ligand and lower-than-normal osteoprotegerin and vitamin D levels. The patient was treated with vitamin D supplements, and his teeth have been stable ever since. CAH is a serious chronic disorder appearing in children with accelerated dental development and possibly premature loss of primary teeth. Copyright © 2015 by the American Academy of Pediatrics.

  8. Pre-operative simulation of pediatric mastoid surgery with 3D-printed temporal bone models.

    Science.gov (United States)

    Rose, Austin S; Webster, Caroline E; Harrysson, Ola L A; Formeister, Eric J; Rawal, Rounak B; Iseli, Claire E

    2015-05-01

    As the process of additive manufacturing, or three-dimensional (3D) printing, has become more practical and affordable, a number of applications for the technology in the field of pediatric otolaryngology have been considered. One area of promise is temporal bone surgical simulation. Having previously developed a model for temporal bone surgical training using 3D printing, we sought to produce a patient-specific model for pre-operative simulation in pediatric otologic surgery. Our hypothesis was that the creation and pre-operative dissection of such a model was possible, and would demonstrate potential benefits in cases of abnormal temporal bone anatomy. In the case presented, an 11-year-old boy underwent a planned canal-wall-down (CWD) tympano-mastoidectomy for recurrent cholesteatoma preceded by a pre-operative surgical simulation using 3D-printed models of the temporal bone. The models were based on the child's pre-operative clinical CT scan and printed using multiple materials to simulate both bone and soft tissue structures. To help confirm the models as accurate representations of the child's anatomy, distances between various anatomic landmarks were measured and compared to the temporal bone CT scan and the 3D model. The simulation allowed the surgical team to appreciate the child's unusual temporal bone anatomy as well as any challenges that might arise in the safety of the temporal bone laboratory, prior to actual surgery in the operating room (OR). There was minimal variability, in terms of absolute distance (mm) and relative distance (%), in measurements between anatomic landmarks obtained from the patient intra-operatively, the pre-operative CT scan and the 3D-printed models. Accurate 3D temporal bone models can be rapidly produced based on clinical CT scans for pre-operative simulation of specific challenging otologic cases in children, potentially reducing medical errors and improving patient safety. Copyright © 2015 Elsevier Ireland Ltd. All rights

  9. The effect of life-long thyroxine treatment and physical activity on bone mineral density in young adult women with congenital hypothyroidism

    NARCIS (Netherlands)

    Kempers, M. J. E.; Vulsma, T.; Wiedijk, B. M.; de Vijlder, J. J. M.; van Eck-Smit, B. L. F.; Verberne, H. J.

    2006-01-01

    OBJECTIVE: Normalization of plasma thyrotropin in T4-supplemented patients with thyroidal congenital hypothyroidism (CH) requires elevated plasma FT4-concentrations compared to patients with acquired thyroidal hypothyroidism. We investigated bone mineral density (BMD) in patients with CH. PATIENTS

  10. Evaluation of CT-scanning of the temporal bone in the diagnosis of ear diseases

    International Nuclear Information System (INIS)

    Murata, Kiyotaka; Isono, Michio; Nishimae, Tadahide; Tamaki, Katsuhiko; Hosoi, Hiroshi; Ohta, Fumihiko

    1983-01-01

    CT-scanning of 96 temporal bones was carried out to reveal the extension of cholesteatoma, periossicular drainage, fracture lines, enlarged internal acoustic meatus and anomalies of labyrinthine capsules and ossicles. The clinical aspects of CT-scanning of the temporal bone (CTTB) were as follows: 1) Inner ear anomalies were observed in 17 temporal bones of unilateral deafness, high tone loss from unknown origin and fluctuant hearing loss. CTTB may explain the pathology of deafness from unknown origin. 2) Inner ear anomalies may be classified into more detailed groups than before. 3) The extension of cholesteatoma, localization and size of labyrinthine fistula can be estimated prior to surgery. 4) Cholesteatoma in a mastoidectomy cavity may be detected. 5) The malleus and incus may be visualized, although the stapes can hardly be found. 6) Fracture lines of a temporal bone, destruction of the internal acoustic meatus may be clearly detected. (author)

  11. Locating the scala media in the fixed human temporal bone for therapeutic access: a preliminary study.

    Science.gov (United States)

    Pau, H; Fagan, P; Oleskevich, S

    2006-11-01

    To investigate the location of the scala media in relation to the round window niche in human temporal bones. Ten human temporal bones were investigated by radical mastoidectomy and promontory drill-out. Temporal bone laboratory. The distance from the scala media to the anterior edge of the round window niche, measured by Fisch's stapedectomy measuring cylinders. The scala media was identified at the transection point of a vertical line 1.6 to 2.2 mm (mean=1.8 mm; standard deviation=0.2) anterior to the anterior edge of the round window niche and a horizontal line 0.2 mm inferior to the lower border of the oval window. This report demonstrates the point of entry into the scala media via the promontory in fixed temporal bone models, which may provide a site of entry for stem cells and gene therapy insertion.

  12. High-resolution computed tomography of the temporal bone. Part 2.: pathology

    International Nuclear Information System (INIS)

    Grzegorzewski, M.; Boron, Z.; Burzynska-Makuch, M.

    1995-01-01

    Selected cases of the temporal bone pathology on high resolution CT were demonstrated. Transverse and coronal sections of the cases were selected from 68 patients with various otological diseases. (author)

  13. Congenital Deficiency of Distal Ulna and Dislocation of the Radial Head Treated by Single Bone Forearm Procedure

    Directory of Open Access Journals (Sweden)

    Paragjyoti Gogoi

    2014-01-01

    Full Text Available Congenital deficiency of part of distal ulna affecting the distal radio-ulnar joint is a rare disorder. It is even rarer to find the association of proximal radio-ulnar joint dislocation along with distal ulnar deficiency. This type of congenital forearm anomaly is difficult to treat. Conversion to a single bone forearm in the expense of pronation-supination movement is a viable option. By doing so the elbow and wrist can be stabilized; however movement is possible in only one plane. We are describing here a girl of 8 years having proximal radio-ulnar joint dislocation along with deficiency of distal ulna treated by converting into a single bone forearm.

  14. la dysplasie fibreuse du rocher fibrous dysplasia of the temporal bone

    African Journals Online (AJOL)

    view of the disease and its manifestations in the temporal bone. Ann. OtolRhinolLaryngol 1982;92(Suppl.): 1–52. 5- Papadakis CE, Skoulakis CE, Propakapis EP, et al. Fibrous dyspla- sia of the temporal bone: report of a case and review of its characteris- tics. Ear Nose Throat J, 2000;79:52–57. 6- Yang H, Chen S, Zheng Y, ...

  15. Inflammatory Pseudotumor of the Temporal Bone: Three Cases and a Review of the Literature

    Directory of Open Access Journals (Sweden)

    Huiqin Tian

    2013-01-01

    Full Text Available Inflammatory pseudotumor (IP is a clinically aggressive but histologically benign condition of unknown cause. Its appearance in the temporal bone is uncommon. We present clinical, radiological, and histopathologic findings of three cases originating in the temporal bone. In the first case, a simultaneous IP of the temporal bone and parotid gland was found with histopathologic confirmation. In the second case, an enlarged cervical node, which was also believed to be related to IP, was observed accompanied with the temporal lesion. While the third case presented with chronic suppurative otitis media. Two of them were treated by surgery alone with complete resolve of the diseases. Another one underwent tympanomastoidectomy in combination with oral steroids, radiation, and chemotherapy, but the IP still recurred. A comprehensive review of the literature on clinical features of the temporal pseudotumor was conducted.

  16. A review of simulation platforms in surgery of the temporal bone.

    Science.gov (United States)

    Bhutta, M F

    2016-10-01

    Surgery of the temporal bone is a high-risk activity in an anatomically complex area. Simulation enables rehearsal of such surgery. The traditional simulation platform is the cadaveric temporal bone, but in recent years other simulation platforms have been created, including plastic and virtual reality platforms. To undertake a review of simulation platforms for temporal bone surgery, specifically assessing their educational value in terms of validity and in enabling transition to surgery. Systematic qualitative review. Search of the Pubmed, CINAHL, BEI and ERIC databases. Assessment of reported outcomes in terms of educational value. A total of 49 articles were included, covering cadaveric, animal, plastic and virtual simulation platforms. Cadaveric simulation is highly rated as an educational tool, but there may be a ceiling effect on educational outcomes after drilling 8-10 temporal bones. Animal models show significant anatomical variation from man. Plastic temporal bone models offer much potential, but at present lack sufficient anatomical or haptic validity. Similarly, virtual reality platforms lack sufficient anatomical or haptic validity, but with technological improvements they are advancing rapidly. At present, cadaveric simulation remains the best platform for training in temporal bone surgery. Technological advances enabling improved materials or modelling mean that in the future plastic or virtual platforms may become comparable to cadaveric platforms, and also offer additional functionality including patient-specific simulation from CT data. © 2015 John Wiley & Sons Ltd.

  17. Human multipotent mesenchymal stromal cells in the treatment of postoperative temporal bone defect: an animal model

    Czech Academy of Sciences Publication Activity Database

    Školoudík, L.; Chrobok, V.; Kalfert, D.; Kočí, Zuzana; Syková, Eva; Chumak, Tetyana; Popelář, Jiří; Syka, Josef; Laco, J.; Dědková, J.; Dayanithi, Govindan; Filip, S.

    2016-01-01

    Roč. 25, č. 7 (2016), s. 1405-1414 ISSN 0963-6897 R&D Projects: GA MŠk(CZ) LO1309 Institutional support: RVO:68378041 Keywords : Human bone marrow * Human mesenchymal stromal cells (hMSCs) * Middle ear surgery * Temporal bone Subject RIV: FP - Other Medical Disciplines Impact factor: 3.006, year: 2016

  18. Bone mineral density in young adult women with congenital adrenal hyperplasia

    Directory of Open Access Journals (Sweden)

    Nishant Raizada

    2016-01-01

    Full Text Available Background: There is equipoise regarding the status of bone mineral density (BMD in patients with congenital adrenal hyperplasia (CAH, where patients need to be on long-term low-dose steroids. Objective: We aimed to evaluate BMD at the hip, spine and forearm in women with CAH and compare it to healthy young adult women of the same age range. Subjects and Methods: Fifteen adult women with CAH with age ranging from 18 to 40 years (mean ± standard deviation = 27.5 ± 6.2 years underwent dual-energy X-ray absorptiometry along with laboratory evaluation. BMD at lumbar spine, hip, forearm along with T-scores were measured. Serum total calcium, phosphate, alkaline phosphatase, 25 hydroxy Vitamin D, intact parathyroid hormone, total testosterone, and dehydroepiandrosterone were assayed. History of any fractures in the past was taken. Fifteen healthy women in the same age range were taken as controls for comparison. Results: The BMD at hip (0.85 ± 0.02 g/cm2 in CAH was significantly lower as compared with controls (0.92 ± 0.03 g/cm2, P = 0.029. BMD at lumbar spine was also reduced (0.96 ± 0.02 vs. 1.03 ± 0.03, P = 0.057. The BMD at forearm was not significantly different between CAH and controls. The mean Vitamin D was 9.8 ng/ml (deficient range. There was no history of fractures in CAH. Conclusion: Young adult CAH women had lower BMD at spine and hip than healthy young adult women of the same age range. The forearm BMD was not different from controls. No change in fracture frequency was present. Patients with CAH being treated with steroids are at increased risk of osteopenia, and their bone health needs to be monitored.

  19. Content Validity of Temporal Bone Models Printed Via Inexpensive Methods and Materials.

    Science.gov (United States)

    Bone, T Michael; Mowry, Sarah E

    2016-09-01

    Computed tomographic (CT) scans of the 3-D printed temporal bone models will be within 15% accuracy of the CT scans of the cadaveric temporal bones. Previous studies have evaluated the face validity of 3-D-printed temporal bone models designed to train otolaryngology residents. The purpose of the study was to determine the content validity of temporal bone models printed using inexpensive printers and materials. Four cadaveric temporal bones were randomly selected and clinical temporal bone CT scans were obtained. Models were generated using previously described methods in acrylonitrile butadiene styrene (ABS) plastic using the Makerbot Replicator 2× and Hyrel printers. Models were radiographically scanned using the same protocol as the cadaveric bones. Four images from each cadaveric CT series and four corresponding images from the model CT series were selected, and voxel values were normalized to black or white. Scan slices were compared using PixelDiff software. Gross anatomic structures were evaluated in the model scans by four board certified otolaryngologists on a 4-point scale. Mean pixel difference between the cadaver and model scans was 14.25 ± 2.30% at the four selected CT slices. Mean cortical bone width difference and mean external auditory canal width difference were 0.58 ± 0.66 mm and 0.55 ± 0.46 mm, respectively. Expert raters felt the mastoid air cells were well represented (2.5 ± 0.5), while middle ear and otic capsule structures were not accurately rendered (all averaged bones for training residents in cortical mastoidectomies, but less effective for middle ear procedures.

  20. Correlations of External Landmarks With Internal Structures of the Temporal Bone.

    Science.gov (United States)

    Piromchai, Patorn; Wijewickrema, Sudanthi; Smeds, Henrik; Kennedy, Gregor; O'Leary, Stephen

    2015-09-01

    The internal anatomy of a temporal bone could be inferred from external landmarks. Mastoid surgery is an important skill that ENT surgeons need to acquire. Surgeons commonly use CT scans as a guide to understanding anatomical variations before surgery. Conversely, in cases where CT scans are not available, or in the temporal bone laboratory where residents are usually not provided with CT scans, it would be beneficial if the internal anatomy of a temporal bone could be inferred from external landmarks. We explored correlations between internal anatomical variations and metrics established to quantify the position of external landmarks that are commonly exposed in the operating room, or the temporal bone laboratory, before commencement of drilling. Mathematical models were developed to predict internal anatomy based on external structures. From an operating room view, the distances between the following external landmarks were observed to have statistically significant correlations with the internal anatomy of a temporal bone: temporal line, external auditory canal, mastoid tip, occipitomastoid suture, and Henle's spine. These structures can be used to infer a low lying dura mater (p = 0.002), an anteriorly located sigmoid sinus (p = 0.006), and a more lateral course of the facial nerve (p external landmarks. The distances between these two landmarks and the operating view external structures were able to further infer the laterality of the facial nerve (p internal structures with a high level of accuracy: the distance from the sigmoid sinus to the posterior external auditory canal (p external landmarks found on the temporal bone. These relationships could be used as a guideline to predict challenges during drilling and choosing appropriate temporal bones for dissection.

  1. Anatomia do osso temporal de ovelhas sob aspectos didáticos Lambs' temporal bone anatomy under didactic aspects

    Directory of Open Access Journals (Sweden)

    André Gurr

    2011-02-01

    Full Text Available É difícil encontrar ossos temporais humanos para o ensino de cirurgia otológica. Ossos temporais de ovelhas podem representar uma possível alternativa. MATERIAIS E MÉTODOS: Os ossos temporais de ovelhas foram dissecados em um programa convencional de dissecação de osso temporal no laboratório. Incluímos mastoidectomia, abordagens endaurais, mas também analisamos a aparência externa, o meato acústico externo e o hipotímpano. Algumas etapas são diferentes das preparações de ossos humanos. Os resultados morfométricos foram comparados à anatomia conhecida de humanos para verificar se o osso temporal de ovelhas seria utilizável para o ensino de cirurgia otológica. RESULTADOS: O osso temporal de ovelhas parece menor do que o humano. Encontramos uma área bolhosa se estendendo ao hipotímpano abaixo do meato acústico externo. A membrana timpânica é muito semelhante à humana. O meato acústico externo é menor e mais curto. A cadeia ossicular exibe analogias para com a humana. DISCUSSÃO: Esse estudo mostra que especificamente o ouvido médio, a membrana timpânica e o conduto auditivo externo são morfologicamente semelhantes às suas contrapartidas encontradas nos ossos temporais humanos. A ovelha parece ser um modelo viável para o ensino da anatomia do ouvido. A menor escala de algumas estruturas, especialmente dos componentes externos do osso temporal representa uma desvantagem. CONCLUSÕES: A ovelha parece representar uma alternativa viável no ensino de cirurgia otológica.Human temporal bones in teaching ear surgery are rare. The lamb's temporal bone might be a possible alternative. MATERIAL AND METHODS: Temporal bones of the lamb were dissected with a typical temporal bone lab drilling program. We included a mastoidectomy, endaural approaches, but also analyzed the outer appearance, the external ear canal and the hypotympanon. Some steps differed from preparation done in humans. The morphometric results were compared to

  2. Aggressive osteoblastoma in mastoid process of temporal bone with facial palsy

    Directory of Open Access Journals (Sweden)

    Manoj Jain

    2013-01-01

    Full Text Available Osteoblastoma is an uncommon primary bone tumor with a predilection for posterior elements of spine. Its occurrence in temporal bone and middle ear is extremely rare. Clinical symptoms are non-specific and cranial nerve involvement is uncommon. The cytomorphological features of osteoblastoma are not very well defined and the experience is limited to only few reports. We report an interesting and rare case of aggressive osteoblastoma, with progressive hearing loss and facial palsy, involving the mastoid process of temporal bone and middle ear along with the description of cyto-morphological features.

  3. 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.

  4. Tympanic plate fractures in temporal bone trauma: prevalence and associated injuries.

    Science.gov (United States)

    Wood, C P; Hunt, C H; Bergen, D C; Carlson, M L; Diehn, F E; Schwartz, K M; McKenzie, G A; Morreale, R F; Lane, J I

    2014-01-01

    The prevalence of tympanic plate fractures, which are associated with an increased risk of external auditory canal stenosis following temporal bone trauma, is unknown. A review of posttraumatic high-resolution CT temporal bone examinations was performed to determine the prevalence of tympanic plate fractures and to identify any associated temporal bone injuries. A retrospective review was performed to evaluate patients with head trauma who underwent emergent high-resolution CT examinations of the temporal bone from July 2006 to March 2012. Fractures were identified and assessed for orientation; involvement of the tympanic plate, scutum, bony labyrinth, facial nerve canal, and temporomandibular joint; and ossicular chain disruption. Thirty-nine patients (41.3 ± 17.2 years of age) had a total of 46 temporal bone fractures (7 bilateral). Tympanic plate fractures were identified in 27 (58.7%) of these 46 fractures. Ossicular disruption occurred in 17 (37.0%). Fractures involving the scutum occurred in 25 (54.4%). None of the 46 fractured temporal bones had a mandibular condyle dislocation or fracture. Of the 27 cases of tympanic plate fractures, 14 (51.8%) had ossicular disruption (P = .016) and 18 (66.6%) had a fracture of the scutum (P = .044). Temporomandibular joint gas was seen in 15 (33%) but was not statistically associated with tympanic plate fracture (P = .21). Tympanic plate fractures are commonly seen on high-resolution CT performed for evaluation of temporal bone trauma. It is important to recognize these fractures to avoid the preventable complication of external auditory canal stenosis and the potential for conductive hearing loss due to a fracture involving the scutum or ossicular chain.

  5. Face and content validation of a virtual reality temporal bone simulator.

    Science.gov (United States)

    Arora, Asit; Khemani, Sam; Tolley, Neil; Singh, Arvind; Budge, James; Varela, David A Diaz Voss; Francis, Howard W; Darzi, Ara; Bhatti, Nasir I

    2012-03-01

    To validate the VOXEL-MAN TempoSurg simulator for temporal bone dissection. Prospective international study. Otolaryngology departments of 2 academic health care institutions in the United Kingdom and United States. Eighty-five subjects were recruited consisting of an experienced and referent group. Participants performed a standardized familiarization session and temporal bone dissection task. Realism, training effectiveness, and global impressions were evaluated across 21 domains using a 5-point Likert-type scale. A score of 4 was the minimum threshold for acceptability. The experienced group comprised 25 otolaryngology trainers who had performed 150 mastoid operations. The referent group comprised 60 trainees (mean otolaryngology experience of 2.9 years). Familiarization took longer in the experienced group (P = .01). User-friendliness was positively rated (mean score 4.1). Seventy percent of participants rated anatomical appearance as acceptable. Trainers rated drill ergonomics worse than did trainees (P = .01). Simulation temporal bone training scored highly (mean score 4.3). Surgical anatomy, drill navigation, and hand-eye coordination accounted for this. Trainees were more likely to recommend temporal bone simulation to a colleague than were trainers (P = .01). Transferability of skills to the operating room was undecided (mean score 3.5). Realism of the VOXEL-MAN virtual reality temporal bone simulator is suboptimal in its current version. Nonetheless, it represents a useful adjunct to existing training methods and is particularly beneficial for novice surgeons before performing cadaveric temporal bone dissection. Improvements in realism, specifically drill ergonomics and visual-spatial perception during deeper temporal bone dissection, are warranted.

  6. TEMPORAL MODELING OF DNA DEGRADATION IN BONE REMAINS

    Directory of Open Access Journals (Sweden)

    Andrei Stefan

    2012-06-01

    Full Text Available The aim of this study is to follow the changes that occur, in time, at DNA level and to establish an efficient and reliable protocol for ancestral DNA extraction from bones found in archaeological sites. To test whether the protocol is efficient and capable of yielding good quality DNA, extraction was first performed on fresh bones. The material consists of fresh pig (Sus scrofa and cow (Bos taurus bones that were grounded by using a drill operating at low speed. The bone powder was then incubated in lysis buffer in the presence of proteinase K. DNA isolation and purification were done by using the phenol:chloroform protocol and DNA was precipitated with absolute ethanol stored at -20oC. The extractions were carried out once every month for a total of four extractions

  7. Transparent model of temporal bone and vestibulocochlear organ made by 3D printing.

    Science.gov (United States)

    Suzuki, Ryoji; Taniguchi, Naoto; Uchida, Fujio; Ishizawa, Akimitsu; Kanatsu, Yoshinori; Zhou, Ming; Funakoshi, Kodai; Akashi, Hideo; Abe, Hiroshi

    2018-01-01

    The vestibulocochlear organ is composed of tiny complex structures embedded in the petrous part of the temporal bone. Landmarks on the temporal bone surface provide the only orientation guide for dissection, but these need to be removed during the course of dissection, making it difficult to grasp the underlying three-dimensional structures, especially for beginners during gross anatomy classes. We report herein an attempt to produce a transparent three-dimensional-printed model of the human ear. En bloc samples of the temporal bone from donated cadavers were subjected to computed tomography (CT) scanning, and on the basis of the data, the surface temporal bone was reconstructed with transparent resin and the vestibulocochlear organ with white resin to create a 1:1.5 scale model. The carotid canal was stuffed with red cotton, and the sigmoid sinus and internal jugular vein were filled with blue clay. In the inner ear, the internal acoustic meatus, cochlea, and semicircular canals were well reconstructed in detail with white resin. The three-dimensional relationships of the semicircular canals, spiral turns of the cochlea, and internal acoustic meatus were well recognizable from every direction through the transparent surface resin. The anterior semicircular canal was obvious immediately beneath the arcuate eminence, and the topographical relationships of the vestibulocochlear organ and adjacent great vessels were easily discernible. We consider that this transparent temporal bone model will be a very useful aid for better understanding of the gross anatomy of the vestibulocochlear organ.

  8. Temporal Bone Osteomyelitis: The Relationship with Malignant Otitis Externa, the Diagnostic Dilemma, and Changing Trends

    Directory of Open Access Journals (Sweden)

    Jia-Cheng Chen

    2014-01-01

    Full Text Available Fifty-five patients hospitalized for osteomyelitis of the temporal bone between 1990 and 2011 were divided into two study groups: group 1 was patients collected from 1990 to 2001 and group 2 was composed of patients between 2002 and 2011. Clinical diagnostic criteria and epidemiologic data were analyzed to illustrate the altering features of osteomyelitis of the temporal bone. Group 1 patients were characterized by high prevalence of diabetes and more commonly suffered from otalgia, otitis externa and granulation tissue in the external auditory canal and higher positive culture for Pseudomonas aeruginosa. Noticeable changing trends were found between both groups, including declining prevalence of diabetes, fewer patients complaining of pain or presenting with otitis externa, and canal granulation, and increased variety of pathogens in group 2. We should highlight the index of clinical suspicion for osteomyelitis of the temporal bone, even in nondiabetic or immunocompetent patients. Painless otorrhea patients were also at risk of osteomyelitis of the temporal bone, especially patients with previous otologic operation. Increased multiplicity of pathogens amplified the difficulty of diagnosis for osteomyelitis of the temporal bone.

  9. Evaluation of a haptics-based virtual reality temporal bone simulator for anatomy and surgery training.

    Science.gov (United States)

    Fang, Te-Yung; Wang, Pa-Chun; Liu, Chih-Hsien; Su, Mu-Chun; Yeh, Shih-Ching

    2014-02-01

    Virtual reality simulation training may improve knowledge of anatomy and surgical skills. We evaluated a 3-dimensional, haptic, virtual reality temporal bone simulator for dissection training. The subjects were 7 otolaryngology residents (3 training sessions each) and 7 medical students (1 training session each). The virtual reality temporal bone simulation station included a computer with software that was linked to a force-feedback hand stylus, and the system recorded performance and collisions with vital anatomic structures. Subjects performed virtual reality dissections and completed questionnaires after the training sessions. Residents and students had favorable responses to most questions of the technology acceptance model (TAM) questionnaire. The average TAM scores were above neutral for residents and medical students in all domains, and the average TAM score for residents was significantly higher for the usefulness domain and lower for the playful domain than students. The average satisfaction questionnaire for residents showed that residents had greater overall satisfaction with cadaver temporal bone dissection training than training with the virtual reality simulator or plastic temporal bone. For medical students, the average comprehension score was significantly increased from before to after training for all anatomic structures. Medical students had significantly more collisions with the dura than residents. The residents had similar mean performance scores after the first and third training sessions for all dissection procedures. The virtual reality temporal bone simulator provided satisfactory training for otolaryngology residents and medical students. Copyright © 2013. Published by Elsevier Ireland Ltd.

  10. Evaluating the Effect of Virtual Reality Temporal Bone Simulation on Mastoidectomy Performance: A Meta-analysis.

    Science.gov (United States)

    Lui, Justin T; Hoy, Monica Y

    2017-06-01

    Background The increasing prevalence of virtual reality simulation in temporal bone surgery warrants an investigation to assess training effectiveness. Objectives To determine if temporal bone simulator use improves mastoidectomy performance. Data Sources Ovid Medline, Embase, and PubMed databases were systematically searched per the PRISMA guidelines. Review Methods Inclusion criteria were peer-reviewed publications that utilized quantitative data of mastoidectomy performance following the use of a temporal bone simulator. The search was restricted to human studies published in English. Studies were excluded if they were in non-peer-reviewed format, were descriptive in nature, or failed to provide surgical performance outcomes. Meta-analysis calculations were then performed. Results A meta-analysis based on the random-effects model revealed an improvement in overall mastoidectomy performance following training on the temporal bone simulator. A standardized mean difference of 0.87 (95% CI, 0.38-1.35) was generated in the setting of a heterogeneous study population ( I 2 = 64.3%, P virtual reality simulation temporal bone surgery studies, meta-analysis calculations demonstrate an improvement in trainee mastoidectomy performance with virtual simulation training.

  11. Recruitment of the middle temporal area by tactile motion in congenital blindness

    DEFF Research Database (Denmark)

    Ptito, Maurice; Matteau, Isabelle; Gjedde, Albert

    2009-01-01

    We used positron emission tomography to investigate whether tactile motion discrimination activates the dorsal visual stream in congenitally blind (CB) participants compared with sighted controls. The tactile stimuli consisted of either static dots, dots moving coherently in one of two possible...

  12. Unbiased Stereologic Estimation of the Spatial Distribution of Paget’s Disease in the Human Temporal Bone

    DEFF Research Database (Denmark)

    Bloch, Sune Land; Sørensen, Mads Sølvsten

    2014-01-01

    remodeling around the inner ear space and to compare it with that of otosclerosis in a contemporary context of temporal bone dynamics. MATERIALS AND METHODS: From the temporal bone collection of Massachusetts Eye and Ear Infirmary, 15 of 29 temporal bones with Paget's disease were selected to obtain...... an independent sample. All volume distributions were obtained along the normal axis of capsular bone remodeling activity by the use of vector-based stereology. RESULTS: Pagetic bone remodeling was distributed centrifugally around the inner ear space at the individual and the general level. This pattern...

  13. Bilateral Facial Paralysis Caused by Bilateral Temporal Bone Fracture: A Case Report and a Literature Review

    Directory of Open Access Journals (Sweden)

    Sultan Şevik Eliçora

    2015-01-01

    Full Text Available Bilateral facial paralysis caused by bilateral temporal bone fracture is a rare clinical entity, with seven cases reported in the literature to date. In this paper, we describe a 40-year-old male patient with bilateral facial paralysis and hearing loss that developed after an occupational accident. On physical examination, House-Brackmann (HB facial paralysis of grade 6 was observed on the right side and HB grade 5 paralysis on the left. Upon temporal bone computed tomography (CT examination, a fracture line exhibiting transverse progression was observed in both petrous temporal bones. Our patient underwent transmastoid facial decompression surgery of the right ear. The patient refused a left-side operation. Such patients require extensive monitoring in intensive care units because the presence of multiple injuries means that facial functions are often very difficult to evaluate. Therefore, delays may ensue in both diagnosis and treatment of bilateral facial paralysis.

  14. Cetuximab with radiotherapy as an alternative treatment for advanced squamous cell carcinoma of the temporal bone.

    Science.gov (United States)

    Ebisumoto, Koji; Okami, Kenji; Hamada, Masashi; Maki, Daisuke; Sakai, Akihiro; Saito, Kosuke; Shimizu, Fukuko; Kaneda, Shoji; Iida, Masahiro

    2018-06-01

    The prognosis of advanced temporal bone cancer is poor, because complete surgical resection is difficult to achieve. Chemoradiotherapy is one of the available curative treatment options; however, its systemic effects on the patient restrict the use of this treatment. A 69-year-old female (who needed peritoneal dialysis) presented at our clinic with T4 left external auditory canal cancer and was treated with cetuximab plus radiotherapy (RT). The primary lesion showed complete response. The patient is currently alive with no evidence of disease two years after completion of the treatment and does not show any late toxicity. This is the first advanced temporal bone cancer patient treated with RT plus cetuximab. Cetuximab plus RT might be a treatment alternative for patients with advanced temporal bone cancer. Copyright © 2017 Elsevier B.V. All rights reserved.

  15. Spatial and temporal variations of the callus mechanical properties during bone transport

    Energy Technology Data Exchange (ETDEWEB)

    Mora-Macias, J.; Reina-Romo, E.; Pajares, A.; Miranda, P.; Dominguez, J.

    2016-07-01

    Nanoindentation allows obtaining the elastic modulus and the hardness of materials point by point. This technique has been used to assess the mechanical propeties of the callus during fracture healing. However, as fas as the authors know, the evaluation of mechanical properties by this technique of the distraction and the docking-site calluses generated during bone transport have not been reported yet. Therefore, the aim of this work is using nanoindentation to assess the spatial and temporal variation of the elastic modulus of the woven bone generated during bone transport. Nanoindentation measurements were carried out using 6 samples from sheep sacrificed at different stages of the bone transport experiments. The results obtained show an important heterogeneity of the elastic modulus of the woven bone without spatial trends. In the case of temporal variation, a clear increase of the mean elastic modulus with time after surgery was observed (from 7±2GPa 35 days after surgery to 14±2GPa 525 days after surgery in the distraction callus and a similar increase in the docking site callus). Comparison with the evolution of the elastic modulus in the woven bone generated during fracture healing shows that mechanical properties increase slower in the case of the woven bone generated during bone transport. (Author)

  16. Facial nerve palsy associated with a cystic lesion of the temporal bone.

    Science.gov (United States)

    Kim, Na Hyun; Shin, Seung-Ho

    2014-03-01

    Facial nerve palsy results in the loss of facial expression and is most commonly caused by a benign, self-limiting inflammatory condition known as Bell palsy. However, there are other conditions that may cause facial paralysis, such as neoplastic conditions of the facial nerve, traumatic nerve injury, and temporal bone lesions. We present a case of facial nerve palsy concurrent with a benign cystic lesion of the temporal bone, adjacent to the tympanic segment of the facial nerve. The patient's symptoms subsided after facial nerve decompression via a transmastoid approach.

  17. CT-scanning of ancient Greenlandic Inuit temporal bones

    DEFF Research Database (Denmark)

    Homøe, P; Lynnerup, N; Videbaek, H

    1992-01-01

    Additional morphological evidence of former infectious middle ear disease (IMED) was found by CT-scanning in 5 of 6 Greenlandic Inuit crania strongly suspected for former IMED due to earlier examination revealing either bilateral hypocellularity or asymmetry of the pneumatized area of the temporal...

  18. Temporal bone fracture following blunt trauma caused by a flying fish.

    Science.gov (United States)

    Goldenberg, D; Karam, M; Danino, J; Flax-Goldenberg, R; Joachims, H Z

    1998-10-01

    Blunt trauma to the temporal region can cause fracture of the skull base, loss of hearing, vestibular symptoms and otorrhoea. The most common causes of blunt trauma to the ear and surrounding area are motor vehicle accidents, violent encounters, and sports-related accidents. We present an obscure case of a man who was struck in the ear by a flying fish while wading in the sea with resulting temporal bone fracture, sudden deafness, vertigo, cerebrospinal fluid otorrhoea, and pneumocephalus.

  19. Validation of exposure visualization and audible distance emission for navigated temporal bone drilling in phantoms.

    Directory of Open Access Journals (Sweden)

    Eduard H J Voormolen

    Full Text Available BACKGROUND: A neuronavigation interface with extended function as compared with current systems was developed to aid during temporal bone surgery. The interface, named EVADE, updates the prior anatomical image and visualizes the bone drilling process virtually in real-time without need for intra-operative imaging. Furthermore, EVADE continuously calculates the distance from the drill tip to segmented temporal bone critical structures (e.g. the sigmoid sinus and facial nerve and produces audiovisual warnings if the surgeon drills in too close vicinity. The aim of this study was to evaluate the accuracy and surgical utility of EVADE in physical phantoms. METHODOLOGY/PRINCIPAL FINDINGS: We performed 228 measurements assessing the position accuracy of tracking a navigated drill in the operating theatre. A mean target registration error of 1.33±0.61 mm with a maximum error of 3.04 mm was found. Five neurosurgeons each drilled two temporal bone phantoms, once using EVADE, and once using a standard neuronavigation interface. While using standard neuronavigation the surgeons damaged three modeled temporal bone critical structures. No structure was hit by surgeons utilizing EVADE. Surgeons felt better orientated and thought they had improved tumor exposure with EVADE. Furthermore, we compared the distances between surface meshes of the virtual drill cavities created by EVADE to actual drill cavities: average maximum errors of 2.54±0.49 mm and -2.70±0.48 mm were found. CONCLUSIONS/SIGNIFICANCE: These results demonstrate that EVADE gives accurate feedback which reduces risks of harming modeled critical structures compared to a standard neuronavigation interface during temporal bone phantom drilling.

  20. Displasia fibrosa do osso temporal: relato de dois casos Fibrous dysplasia of the temporal bone: report of two cases

    Directory of Open Access Journals (Sweden)

    Roberto Claudio B. Oliveira

    2004-10-01

    Full Text Available A displasia fibrosa do osso temporal é uma doença de etiologia ainda controversa, manifestando-se principalmente por estenose progressiva do conduto auditivo externo e pela perda condutiva da audição. Outras manifestações incluem abaulamento na região temporal ou retroauricular, otorréia, otalgia e disacusia sensório-neural. A incidência é maior no sexo masculino e acomete principalmente a raça branca. O exame radiológico característico demonstra um aspecto de "vidro-fosco" homogêneo envolvido por uma concha de tecido cortical denso, embora existam outros padrões radiológicos desta enfermidade. O exame microscópico demonstra um trabeculado ósseo semelhante aos caracteres chineses. Este estudo relata dois casos de displasia fibrosa do osso temporal que se destacam, pois ultrapassaram o osso temporal, acometendo a região zigomática, sendo que no segundo caso houve também comprometimento do osso esfenóide e o pterigóide. Os pacientes foram submetidos à mastoidectomia radical modificada e tiveram boa evolução.Fibrous dysplasia of the temporal bone (FDTB is a disorder which etiology is still controversial. Its main clinical feature is a progressive narrowing of the external auditory canal following by conductive hearing loss. Temporal or retroauricular enlargement, ear discharge, otalgia, and sensorineural hearing loss are additional findings. Women and Caucasians are more affected. The prominent finding is a homogeneous radiodense "grounded glass" like image shell surrounded by dense cortical tissue. However, other radiological patterns of this disease may be displayed. Microscopically, a trabecular of bone in "Chinese letter" configuration is found. The two cases of FDTB herein reported are particularly special for a far beyond temporal commitment reaching the zygomatic area in the first case and sphenoid and pterygoid bones in the second one. This infrequent clinical feature with unusual radiological findings made these

  1. Face and content validation of a novel three-dimensional printed temporal bone for surgical skills development.

    Science.gov (United States)

    Da Cruz, M J; Francis, H W

    2015-07-01

    To assess the face and content validity of a novel synthetic, three-dimensional printed temporal bone for surgical skills development and training. A synthetic temporal bone was printed using composite materials and three-dimensional printing technology. Surgical trainees were asked to complete three structured temporal bone dissection exercises. Attitudes and impressions were then assessed using a semi-structured questionnaire. Previous cadaver and real operating experiences were used as a reference. Trainees' experiences of the synthetic temporal bone were analysed in terms of four domains: anatomical realism, usefulness as a training tool, task-based usefulness and overall reactions. Responses across all domains indicated a high degree of acceptance, suggesting that the three-dimensional printed temporal bone was a useful tool in skills development. A sophisticated three-dimensional printed temporal bone that demonstrates face and content validity was developed. The efficiency in cost savings coupled with low associated biohazards make it likely that the printed temporal bone will be incorporated into traditional temporal bone skills development programmes in the near future.

  2. Pneumatization of the Temporal Bones in a Greenlandic Inuit Anthropological Material

    DEFF Research Database (Denmark)

    Homøe, P; Lynnerup, N

    1991-01-01

    The degree of pneumatization of the temporal bones correlates with exposure during childhood and adolescence to infectious middle ear diseases (IMED), both acute and chronic. The pneumatized area as seen on cranial X-rays can be measured. This was applied to an anthropological material in order...

  3. Incidental internal carotid artery calcifications on temporal bone CT in children

    Energy Technology Data Exchange (ETDEWEB)

    Koch, Bernadette; Jones, Blaise [Cincinnati Children' s Hospital Medical Center, Department of Radiology, Cincinnati, OH (United States); Blackham, Aaron [University of Cincinnati College of Medicine, Cincinnati, OH (United States)

    2007-02-15

    Incidental internal carotid artery (ICA) calcifications are occasionally noted on CT images of the brain and temporal bone. In adults, incidental calcifications have been correlated with increased incidence of hypercholesterolemia, cardiac disease, diabetes and carotid stenosis. To determine the incidence of incidental calcifications of the carotid siphon on temporal bone CT in children. We retrospectively reviewed 24 months of consecutive temporal bone CT examinations in children aged 18 years and younger. CT examinations on 663 patients were reviewed and the presence or absence of ICA calcifications was ranked as absent, questionable or definitive. In patients in whom definitive calcifications were identified, hospital charts were reviewed for evidence of diabetes mellitus, hypercholesterolemia, hypertriglyceridemia, hyperlipidemia and chronic renal disease as potential causes of early atherosclerosis. Of the 663 patients, 25% had definitive calcifications within the wall of the ICA: 6% of children younger than 2 years and 28% of children 12-19 years of age. Incidentally noted ICA calcifications are a common finding on temporal bone CT in children, most likely a physiologic response to turbulent flow at natural bends in the artery rather than secondary to underlying disease predisposing to early atherosclerotic calcification. (orig.)

  4. Anatomic variations of the cochlea and relations to other temporal bone structures

    Energy Technology Data Exchange (ETDEWEB)

    Dimopoulos, P.; Muren, C. (Akademiska Sjukhuset, Uppsala (Sweden). Dept. of Diagnostic Radiology Sabbatsberg' s Sjukhus, Stockholm (Sweden). Dept. of Diagnostic Radiology)

    1990-09-01

    The size and shape of the human cochlea and the normal ranges of variation of its dimensions were evaluated in 95 plastic casts, prepared from temporal bone specimens. The normal range of variation is fairly small, and is not age-dependent. Obvious digression from this range, associated with pertinent clinical symptoms, indicates an abnormality. (orig./MG).

  5. High-resolution CT of temporal bone trauma: review of 38 cases

    International Nuclear Information System (INIS)

    Hiroual, M.R.; Zougarhi, A.; Cherif Idrissi El Ganouni, N.; Essadki, O.; Ousehal, A.; Tijani Adil, O.; Maliki, O.; Aderdour, L.; Raji, A.

    2010-01-01

    Purpose Temporal bone trauma is frequent but difficult to assess due to the diversity of clinical presentations and complex anatomy. We have sought to assess the different types of fractures and complications on high-resolution CT. Materials and methods Descriptive retrospective study over a 24 month period performed in the ENT radiology section of the Mohammed 6 university medical center in Marrakech. A total of 38 cases of temporal bone trauma were reviewed. All patients underwent ENT evaluation and high-resolution CT of the temporal bone using 1 mm axial and coronal sections. Results Mean patient age was 33 years (range: 14-55 years) with male predominance (sex ratio: 36/2). Clinical symptoms were mainly otorrhagia and conductive hearing loss. Oblique extra-labyrinthine fractures were most frequent. Two cases of pneumo-labyrinth were noted. Management was conservative in most cases with deafness in 3 cases. Conclusion High-resolution CT of the temporal bone provides accurate depiction of lesions explaining the clinical symptoms and helps guide management. MRI is complimentary to further assess the labyrinth and VII-VIII nerve complex. (author)

  6. Interactive atlas using web browser: CT and MRI of the temporal bone

    International Nuclear Information System (INIS)

    Chung, Eun Chul; Youn, Eun Kyung; Lee, Young Uk

    2000-01-01

    The purposes of this study were to construct an interactive atlas of the temporal bone using a web-browser and to provide a template for web-based teaching files, using free and shared applets and scripts on the internet. HRCT and MR images of the temporal bone including its normal anatomy, tumors, trauma, inflammation, anomalies and vascular diseases were used in this study. Acquired radiologic images were transformed to GIF/JPG formats and to achieve appropriate image quality, were retouched. Text and image files of normal anatomy and diseases were written by HTML. JavaScript and applets were inserted in the HTML files for the interactive display of images and texts. In order to review anatomic features and diseases, a search index was also attached to the last part of the file. Using interactive images and text, temporal bone anatomy and disorders were displayed. Scripts and applets were also useful for indicating specific points of interest when a mouse was placed over the anatomic sites. The atlas may be viewed in the form of a CD-ROM, or via the internet using any computer platform or web-browser. This web-based teaching file of the temporal bone offers dynamic and interactive education. It can be usefully employed as a template for the production of interactive educational materials, offering JavaScript and providing suitable input for classes. It can replace texts and imaging contents. (author)

  7. Incidental internal carotid artery calcifications on temporal bone CT in children

    International Nuclear Information System (INIS)

    Koch, Bernadette; Jones, Blaise; Blackham, Aaron

    2007-01-01

    Incidental internal carotid artery (ICA) calcifications are occasionally noted on CT images of the brain and temporal bone. In adults, incidental calcifications have been correlated with increased incidence of hypercholesterolemia, cardiac disease, diabetes and carotid stenosis. To determine the incidence of incidental calcifications of the carotid siphon on temporal bone CT in children. We retrospectively reviewed 24 months of consecutive temporal bone CT examinations in children aged 18 years and younger. CT examinations on 663 patients were reviewed and the presence or absence of ICA calcifications was ranked as absent, questionable or definitive. In patients in whom definitive calcifications were identified, hospital charts were reviewed for evidence of diabetes mellitus, hypercholesterolemia, hypertriglyceridemia, hyperlipidemia and chronic renal disease as potential causes of early atherosclerosis. Of the 663 patients, 25% had definitive calcifications within the wall of the ICA: 6% of children younger than 2 years and 28% of children 12-19 years of age. Incidentally noted ICA calcifications are a common finding on temporal bone CT in children, most likely a physiologic response to turbulent flow at natural bends in the artery rather than secondary to underlying disease predisposing to early atherosclerotic calcification. (orig.)

  8. Determination of a facial nerve safety zone for navigated temporal bone surgery

    NARCIS (Netherlands)

    Voormolen, E.H.J.; Stralen, van M.; Woerdeman, P.A.; Pluim, J.P.W.; Noordmans, H.J.; Viergever, M.A.; Regli, L.; Berkelbach van der Sprenkel, J.W.

    2012-01-01

    Transtemporal approaches require surgeons to drill the temporal bone to expose target lesions while avoiding the critical structures within it, such as the facial nerve and other neurovascular structures. We envision a novel protective neuronavigation system that continuously calculates the drill

  9. High-resolution computed tomography of the temporal bone. Part 1.: normal anatomy

    International Nuclear Information System (INIS)

    Grzegorzewski, M.; Boron, Z.; Burzynska-Makuch, M.

    1995-01-01

    Normal anatomy of the temporal bone in transverse and coronal sections was presented. CT studies were performed using high-resolution program. The images of an asymptomatic ear of 2 patients were selected from 68 cases examined on account of various otological diseases. All the sections showed as many as 68 anatomic structures. (author)

  10. Transverse axial plane anatomy of the temporal bone employing high spatial resolution computed tomography

    International Nuclear Information System (INIS)

    Russell, E.J.; Koslow, M.; Lasjaunias, P.; Bergeron, R.T.; Chase, N.

    1982-01-01

    Anatomical relationships of temporal bone structures are demonstrated by thin section edge detection computed tomography. Many otic structures are best appreciated in axial view, but reorientation to anatomy as seen in this plane is needed for optimal diagnosis. A level by level review of key structure is presented toward this end. The limitations and advantages of computed tomography are discussed. (orig.)

  11. Benign osteoblastoma of the temporal bone: case report and literature review

    Directory of Open Access Journals (Sweden)

    Yoo, Hea Jung

    2008-09-01

    Full Text Available Introduction: Osteoblastoma is defined as a lesion of bone, which is rich in osteoblasts, well-vascularized, and affects mainly the spinal cord. Although it is benign, it presents malignant features and simulates osteosarcoma. It affects mainly young adults between 20 and 40 years old. It rarely compromises the temporal bone. Objective: To present a rare case in the temporal bone with clinical malignant features whose the anatomopathological study has revealed to be a benign tumor. Method: The patient presented a tumor which affected the middle ear cleft, the mastoid, and the right middle fossa. The patient underwent a surgery and, in association with otorhinolaryngology, the tumor was completely dried out from the middle fossa and the middle ear. Fascia lata was used to repair the dural impairment and an acrylic plate was used to cover the bone impairment. Result: Post-surgery evolved positively, however the acrylic plate has moved itself and has stenosed the right external acoustic meato, which had to be removed 3 years later. The patient has had a good followup, which is still being carried through. Conclusion: Osteoblastoma is a multiform tumor that might affect the temporal bone with malignant features, which simulates osteosarcoma, but, in histological terms, it does not present any malignant signals. However, there is a need for a long post-surgery follow-up.

  12. Creation of a 3D printed temporal bone model from clinical CT data.

    Science.gov (United States)

    Cohen, Joss; Reyes, Samuel A

    2015-01-01

    Generate and describe the process of creating a 3D printed, rapid prototype temporal bone model from clinical quality CT images. We describe a technique to create an accurate, alterable, and reproducible rapid prototype temporal bone model using freely available software to segment clinical CT data and generate three different 3D models composed of ABS plastic. Each model was evaluated based on the appearance and size of anatomical structures and response to surgical drilling. Mastoid air cells had retained scaffolding material in the initial versions. This required modifying the model to allow drainage of the scaffolding material. External auditory canal dimensions were similar to those measured from the clinical data. Malleus, incus, oval window, round window, promontory, horizontal semicircular canal, and mastoid segment of the facial nerve canal were identified in all models. The stapes was only partially formed in two models and absent in the third. Qualitative feel of the ABS plastic was softer than bone. The pate produced by drilling was similar to bone dust when appropriate irrigation was used. We present a rapid prototype temporal bone model made based on clinical CT data using 3D printing technology. The model can be made quickly and inexpensively enough to have potential applications for educational training. Copyright © 2015 Elsevier Inc. All rights reserved.

  13. Theoretical analysis of the spatio-temporal structure of bone multicellular units

    International Nuclear Information System (INIS)

    Buenzli, P R; Pivonka, P; Gardiner, B S; Smith, D W; Dunstan, C R; Mundy, G R

    2010-01-01

    Bone multicellular units (BMUs) maintain the viability of the skeletal tissue by coordinating locally the sequence of bone resorption and bone formation performed by cells of the osteoclastic and osteoblastic lineage. Understanding the emergence and the net bone balance of such structured microsystems out of the complex network of biochemical interactions between bone cells is fundamental for many bone-related diseases and the evaluation of fracture risk. Based on current experimental knowledge, we propose a spatio-temporal continuum model describing the interactions of osteoblastic and osteoclastic cells. We show that this model admits travelling-wave-like solutions with well-confined cell profiles upon specifying external conditions mimicking the environment encountered in cortical bone remodelling. The shapes of the various cell concentration profiles within this travelling structure are intrinsically linked to the parameters of the model such as differentiation, proliferation, and apoptosis rates of bone cells. The internal structure of BMUs is reproduced, allowing for experimental calibration. The spatial distribution of the key regulatory factors can also be exhibited, which in diseased states could give hints as to the biochemical agent most accountable for the disorder.

  14. Temporal Bone Fractures and its Classification: Retrospective Study of Incidence, Causes, Clinical Features, Complications and Outcome

    Directory of Open Access Journals (Sweden)

    Umamaheshwari Basavaraju

    2017-10-01

    Full Text Available Introduction: Temporal bone fracture is usually associated with high energy head injury and can cause potentially severe complications. Immediate detection of temporal bone fracture and its complications helps in providing early and effective treatment, which if left untreated can have drastic consequences. Aim: The main objective of the study is to document the frequency and most prevalent type of temporal bone fracture, co-existing complications and to establish association between them. Materials and Methods: One year (2015-2016 retrospective study of head injured patients presented to the Emergency Department, Mysore Medical College And Research Institute was conducted. Age and gender distribution, cause of injury, radiological findings, otorhinolaryngological clinical presentations and treatment given were analyzed. The results were tabulated and were evaluated by Microsoft Excel 2013. Results: Out of 1450 patients evaluated for head injury 154 patients were positive for temporal bone fracture. Incidence of the study was 10.6%. Majority of the patients were male (66.2% and were between 30 to 40 years (50.1%. The major cause of injury was motor vehicle accidents (84.48%. Right side was involved (58.4% more than the left side (41.5%. Most common clinical presentation was otorrhea 68.8%, followed by otalgia (35.04% and otorhinorrhea (24.67%. Longitudinal type fracture was most frequent 56.25%. Otic capsule involvement was present in 35.93%. Most of the fractures were managed conservatively whereas surgery was required in 12 patients (7.7%. Conclusion: Temporal bone fractures were frequently associated with severe traumatic brain injury leading to serious long term morbidity and sequelae. CT-scan is of utmost importance in detection of fractures and its complications.

  15. Successful Function-Preserving Therapy for Chondroblastoma of the Temporal Bone Involving the Temporomandibular Joint

    Directory of Open Access Journals (Sweden)

    Junkichi Yokoyama

    2011-02-01

    Full Text Available We present a case involving a late diagnosis of chondroblastoma of the temporal skull base involving the temporomandibular joint (TMJ. Following an initial misdiagnosis and unsuccessful treatment over a period of 5 years, the patient was referred to our department for further evaluation and possible surgical intervention for occlusal abnormalities, trismus, clicking of the TMJ, and hearing impairment. Based on preoperative immunochemical studies showing positive reaction of multinucleated giant cells for S-100 protein, the final diagnosis was chondroblastoma. The surgical approach – postauricular incision and total parotidectomy, with complete removal of the temporal bone, including the TMJ via the extended middle fossa – was successful in preserving facial nerves and diminishing clinical manifestations. This study highlights a misdiagnosed case in an effort to underline the importance of medical examinations and accurate differential diagnosis in cases involving any tumor mass in the temporal bone.

  16. Management of a Congenitally Missing Lateral Incisor with Orthodontics, Bone Grafting (a New Method and Single-Tooth Implant: A Case Report

    Directory of Open Access Journals (Sweden)

    Hamid Reza Arab

    2014-03-01

    Full Text Available Careful treatment planning, space management, augmentation of bone and attention to the details of implant surgical and prosthetic techniques are important factors when treating anterior maxilla specially replacement of missing teeth. This case report addresses a chair-side ridge augmentation procedure using autograft bone harvested with trephine drills and placed without using screws and the fundamental considerations related to replacement of a congenitally missing lateral incisor by a team approach

  17. Large capillary hemangioma of the temporal bone with a dural tail sign: A case report

    KAUST Repository

    YANG, GUANG

    2014-05-13

    The present study reports a rare case of large capillary hemangioma of the temporal bone with a dural tail sign. A 57-year-old female presented with pulsatile tinnitus and episodic vertigo associated with a ten-year history of an intermittent faint headache. Magnetic resonance imaging revealed a mass in the right petrous bone, which was hypointense on T1-weighted images and heterogeneously hyperintense on T2-weighted images, and showed a dural tail sign following gadolinium administration. Pre-operatively, this tumor was believed to be a meningioma. During surgery, the vascular tumor was removed by a modified pterional approach. A histopathological examination indicated that the tumor was a capillary hemangioma. Although intraosseous capillary hemangiomas are rare, they most frequently affect the temporal bone. Hemangiomas of the temporal bone may mimic other more common basal tumors. The diagnosis is most often made during surgical resection. The dural tail sign is not specific for meningioma, as it also occurs in other intracranial or extracranial tumors. The treatment of intratemporal hemangiomas is complete surgical excision, with radiotherapy used for unresectable lesions. To the best of our knowledge, the present study is the fourth case of intraosseous intracranial capillary hemangioma, but the largest intratemporal hemangioma to be reported in the literature to date.

  18. Large capillary hemangioma of the temporal bone with a dural tail sign: A case report

    KAUST Repository

    YANG, GUANG; LI, CHENGUANG; CHEN, XIN; LIU, YAOHUA; HAN, DAYONG; Gao, Xin; KAWAMOTO, KEIJI; ZHAO, SHIGUANG

    2014-01-01

    The present study reports a rare case of large capillary hemangioma of the temporal bone with a dural tail sign. A 57-year-old female presented with pulsatile tinnitus and episodic vertigo associated with a ten-year history of an intermittent faint headache. Magnetic resonance imaging revealed a mass in the right petrous bone, which was hypointense on T1-weighted images and heterogeneously hyperintense on T2-weighted images, and showed a dural tail sign following gadolinium administration. Pre-operatively, this tumor was believed to be a meningioma. During surgery, the vascular tumor was removed by a modified pterional approach. A histopathological examination indicated that the tumor was a capillary hemangioma. Although intraosseous capillary hemangiomas are rare, they most frequently affect the temporal bone. Hemangiomas of the temporal bone may mimic other more common basal tumors. The diagnosis is most often made during surgical resection. The dural tail sign is not specific for meningioma, as it also occurs in other intracranial or extracranial tumors. The treatment of intratemporal hemangiomas is complete surgical excision, with radiotherapy used for unresectable lesions. To the best of our knowledge, the present study is the fourth case of intraosseous intracranial capillary hemangioma, but the largest intratemporal hemangioma to be reported in the literature to date.

  19. The clinical significance of temporal bone CT with regard to hearing preservation in acoustic neurinoma removal

    International Nuclear Information System (INIS)

    Umezu, Hiromichi; Seki, Yojirou; Aiba, Tadashi

    1988-01-01

    Since Rand and Kurze discussed the possibility of the anatomic preservation of the cochlear nerve via the posterior fossa transmeatal approach for the acoustic neurinoma, there have been increasingly many reports on the preservation of cochlear nerve function. With recent advances in diagnostic tools, today's neurosurgeon is able to make an earlier and more precise diagnosis of acoustic neurinomas even when the patient has good hearing. If useful hearing is to be kept, the cochlear nerve and blood supply of the labyrinth have to be preserved. In addition, surgical entry into the labyrinth, upon the removal of the posterior wall of the internal auditory canal, must be avoided, since it is likely to result in permanent hearing loss. Because of its superior contrast and spatial resolution, thin-section and high-resolution computed tomography of the temporal bone has the great advantage of demonstrating the exact relationship of the internal auditory canal to the posterior semicircular canal, the vestibule, and the common crus. In this study, thin-section, high-resolution computed tomography of the temporal bone was performed in 11 cases of acoustic neurinoma pre- and postoperatively. At operation, the lateral limit of the bone removal of the posterior internal auditory canal was determined on the basis of preoperative computed tomography of the temporal bone. As a result, inadvertent entry into the labyrinth was avoided, and useful hearing was preserved in 8 cases. In order to preserve useful hearing following total tumor removal, it is essential to plan the operative strategy meticulously on the basis of the findings of the preoperative investigation, Including temporal bone CT. (author)

  20. Clinical significance of temporal bone CT with regard to hearing preservation in acoustic neurinoma removal

    Energy Technology Data Exchange (ETDEWEB)

    Umezu, Hiromichi; Seki, Yojirou; Aiba, Tadashi

    1988-02-01

    Since Rand and Kurze discussed the possibility of the anatomic preservation of the cochlear nerve via the posterior fossa transmeatal approach for the acoustic neurinoma, there have been increasingly many reports on the preservation of cochlear nerve function. With recent advances in diagnostic tools, today's neurosurgeon is able to make an earlier and more precise diagnosis of acoustic neurinomas even when the patient has good hearing. If useful hearing is to be kept, the cochlear nerve and blood supply of the labyrinth have to be preserved. In addition, surgical entry into the labyrinth, upon the removal of the posterior wall of the internal auditory canal, must be avoided, since it is likely to result in permanent hearing loss. Because of its superior contrast and spatial resolution, thin-section and high-resolution computed tomography of the temporal bone has the great advantage of demonstrating the exact relationship of the internal auditory canal to the posterior semicircular canal, the vestibule, and the common crus. In this study, thin-section, high-resolution computed tomography of the temporal bone was performed in 11 cases of acoustic neurinoma pre- and postoperatively. At operation, the lateral limit of the bone removal of the posterior internal auditory canal was determined on the basis of preoperative computed tomography of the temporal bone. As a result, inadvertent entry into the labyrinth was avoided, and useful hearing was preserved in 8 cases. In order to preserve useful hearing following total tumor removal, it is essential to plan the operative strategy meticulously on the basis of the findings of the preoperative investigation, Including temporal bone CT.

  1. CT and MRI characteristica of tumours of the temporal bone and the cerebello-pontine angle

    International Nuclear Information System (INIS)

    Imhof, H.; Henk, C.B.; Dirisamer, A.; Czerny, C.; Gstoettner, W.

    2003-01-01

    Tumours lesions of the temporal bone and of the cerebello-pontine angle are rare.This tumours can be separated into benign and malignant lesions. In this paper the CT and MRI characteristica of tumours of the temporal bone and the cerebello-pontane angle will be demonstrated. High resolution CT (HRCT) as usually performed in the axial plane are using a high resolution bone window level setting, coronal planes are the reconstructed from the axial data set or will be obtained directly. With the MRI FLAIR sequence in the axial plane the whole brain will be scanned either to depict or exclude a tumour invasion into the brain. After this,T2-weighted fast spin echo sequences or fatsuppressed inversion recovery sequences in high resolution technique in the axial plane will be obtained from the temporal bone and axial T1-weighted spinecho sequences before and after the intravenous application of contrast material will be obtained of this region. Finally T1-weighted spinecho sequences in high resolution technique with fatsuppression after the intravenous application of contrast material will be performed in the coronal plane. HRCT and MRI are both used to depict the most exact tumorous borders. HRCT excellently depicts the osseous changes for example exostosis of the external auditory canal, while also with HRCT osseous changes maybe characterized into more benign or malignant types. MRI has a very high soft tissue contrast and may therefore either characterize vascular space-occupying lesions for example glomus jugulare tumours or may differentiate between more benign or malignant lesions. In conclusion HRCT and MRI of the temporal bone are excellent methods to depict and mostly characterize tumour lesions and can help to differentiate between benign and malignant lesion. These imaging methods shall be used complementary and may have a great impact for the therapeutic planning. (orig.) [de

  2. Fracture of the temporal bone in patients with traumatic brain injury

    Directory of Open Access Journals (Sweden)

    Secchi, Myrian Marajó Dal

    2012-01-01

    Full Text Available Introduction: The fractures in the temporal bone are lesions that are observed in patients with traumatic brain injury (TBI. The computed tomography of high-resolution (CT allows evaluating the fracture and the complications. Objective: Evaluate patients with TBI and temporal bone fracture. Way of study: Retrospective study. Method: Were evaluated 28 patients interned by TBI with clinical evidence and/or radiologic from temporal bone fractures. Results: The age ranged from 3 to 75 years. The most affected side was the right side 50% (n=14, left side 36% (n=10 and both sides 14% (n=4. The etiology of the trauma was the falling 25% (n=7, accidents with motorcycles and bicycles 21% (n=6, physical aggression 14% (n=4, running over 11% (n=3, fall of object 4% (n=1 and other causes 25% (n=7. The clinical signs were: Otorrhagia 78%, otalgia 11% (n=3, otorrhea 7% (n=2, facial paralysis 7% (n=2 and hearing loss 7% (n=2. The otoscopic findings: otorrhagia 57% (n=16, laceration of external auditory canal 36% (n=10, hemotympanum 11% (n=3, normal 7% (n=2 and Battle signal 7% (n=2. The findings for CT of skull were: with no alterations 54% (n=15 and temporal fracture 7% (n=2 and the CT of temporal bones were: line of fracture 71% (n=20, opacification of the mastoid 25% (n=7, glenoid cavity air 14% (n=1, dislocation of the ossicular chain 7% (n=2 and veiling of the middle ear 4% (n=1. Conclusion: Patients with TBI must be submitted to the otorhinolaryngological evaluation and imaging, for the early diagnosis of the complications and treatment.

  3. CT-MR image data fusion for computer assisted navigated neurosurgery of temporal bone tumors

    International Nuclear Information System (INIS)

    Nemec, Stefan Franz; Donat, Markus Alexander; Mehrain, Sheida; Friedrich, Klaus; Krestan, Christian; Matula, Christian; Imhof, Herwig; Czerny, Christian

    2007-01-01

    Purpose: To demonstrate the value of multi detector computed tomography (MDCT) and magnetic resonance imaging (MRI) in the preoperative work up of temporal bone tumors and to present, especially, CT and MR image fusion for surgical planning and performance in computer assisted navigated neurosurgery of temporal bone tumors. Materials and methods: Fifteen patients with temporal bone tumors underwent MDCT and MRI. MDCT was performed in high-resolution bone window level setting in axial plane. The reconstructed MDCT slice thickness was 0.8 mm. MRI was performed in axial and coronal plane with T2-weighted fast spin-echo (FSE) sequences, un-enhanced and contrast-enhanced T1-weighted spin-echo (SE) sequences, and coronal T1-weighted SE sequences with fat suppression and with 3D T1-weighted gradient-echo (GE) contrast-enhanced sequences in axial plane. The 3D T1-weighted GE sequence had a slice thickness of 1 mm. Image data sets of CT and 3D T1-weighted GE sequences were merged utilizing a workstation to create CT-MR fusion images. MDCT and MR images were separately used to depict and characterize lesions. The fusion images were utilized for interventional planning and intraoperative image guidance. The intraoperative accuracy of the navigation unit was measured, defined as the deviation between the same landmark in the navigation image and the patient. Results: Tumorous lesions of bone and soft tissue were well delineated and characterized by CT and MR images. The images played a crucial role in the differentiation of benign and malignant pathologies, which consisted of 13 benign and 2 malignant tumors. The CT-MR fusion images supported the surgeon in preoperative planning and improved surgical performance. The mean intraoperative accuracy of the navigation system was 1.25 mm. Conclusion: CT and MRI are essential in the preoperative work up of temporal bone tumors. CT-MR image data fusion presents an accurate tool for planning the correct surgical procedure and is a

  4. Semicircular canal dehiscence: Frequency and distribution on temporal bone CT and its relationship with the clinical outcomes

    International Nuclear Information System (INIS)

    Elmali, Muzaffer; Polat, Ahmet Veysel; Kucuk, Harun; Atmaca, Sinan; Aksoy, Ahmet

    2013-01-01

    Purpose: In this study, we aimed to investigate the frequency of SCD and its distribution and relationship with clinical outcomes on thin-section CT of the temporal bone. Materials and methods: Digital temporal bone CT images of 850 consecutive patients (1700 temporal bone CTs, 5100 SCs) who presented with a range of complaints such as vertigo, deafness, ear pain, fullness, and discharge between January 2008 and December 2011 were re-evaluated. Axial and oblique coronal reconstruction images of the temporal bone were made with a reconstruction thickness of 0.5 mm. Additionally, superior SC was evaluated in two perpendicular planes. Results: Out of 850 patients, 70 had completely normal temporal bone CT. Ninety-three patients had at least one SCD. In the temporal bone-based evaluation, 119 (26 bilateral, 67 unilateral) of 1700 temporal bones (7%) showed dehiscence. The SC-based evaluation revealed 125 SCD (2.5%) in 5100 SCs. The total number and rates of SCD were as follows: superior 103 (82.4%), posterior 13 (10.4%), and lateral nine (7.2%). Twenty of the 93 patients with SCD (21.5%) revealed no other findings on their temporal bone CTs. We determined a significant correlation between vestibular complaints, conductive hearing loss and SCD but there was no correlation between mixed, sensorineural hearing loss and SCD. Conclusion: We determined the frequency of SCD in 11% of patients and 7% of temporal bones. With regards to the distribution, the superior SC showed the highest dehiscence rate (82.4%). We found a significant correlation between vestibular symptoms, conductive hearing loss and SCD

  5. Semicircular canal dehiscence: Frequency and distribution on temporal bone CT and its relationship with the clinical outcomes

    Energy Technology Data Exchange (ETDEWEB)

    Elmali, Muzaffer, E-mail: muzafel@yahoo.com.tr [Department of Radiology, Ondokuz Mayis University, Faculty of Medicine, Samsun (Turkey); Polat, Ahmet Veysel, E-mail: veyselp@hotmail.com [Department of Radiology, Ondokuz Mayis University, Faculty of Medicine, Samsun (Turkey); Kucuk, Harun, E-mail: hardrmd@yahoo.com [Department of Otorhinolaryngology, Ondokuz Mayis University, Faculty of Medicine, Samsun (Turkey); Atmaca, Sinan, E-mail: sinanatmaca@yahoo.com [Department of Otorhinolaryngology, Ondokuz Mayis University, Faculty of Medicine, Samsun (Turkey); Aksoy, Ahmet, E-mail: toxocara47@hotmail.com [Department of Otorhinolaryngology, Ondokuz Mayis University, Faculty of Medicine, Samsun (Turkey)

    2013-10-01

    Purpose: In this study, we aimed to investigate the frequency of SCD and its distribution and relationship with clinical outcomes on thin-section CT of the temporal bone. Materials and methods: Digital temporal bone CT images of 850 consecutive patients (1700 temporal bone CTs, 5100 SCs) who presented with a range of complaints such as vertigo, deafness, ear pain, fullness, and discharge between January 2008 and December 2011 were re-evaluated. Axial and oblique coronal reconstruction images of the temporal bone were made with a reconstruction thickness of 0.5 mm. Additionally, superior SC was evaluated in two perpendicular planes. Results: Out of 850 patients, 70 had completely normal temporal bone CT. Ninety-three patients had at least one SCD. In the temporal bone-based evaluation, 119 (26 bilateral, 67 unilateral) of 1700 temporal bones (7%) showed dehiscence. The SC-based evaluation revealed 125 SCD (2.5%) in 5100 SCs. The total number and rates of SCD were as follows: superior 103 (82.4%), posterior 13 (10.4%), and lateral nine (7.2%). Twenty of the 93 patients with SCD (21.5%) revealed no other findings on their temporal bone CTs. We determined a significant correlation between vestibular complaints, conductive hearing loss and SCD but there was no correlation between mixed, sensorineural hearing loss and SCD. Conclusion: We determined the frequency of SCD in 11% of patients and 7% of temporal bones. With regards to the distribution, the superior SC showed the highest dehiscence rate (82.4%). We found a significant correlation between vestibular symptoms, conductive hearing loss and SCD.

  6. Neutrophil Extracellular Traps and Fibrin in Otitis Media: Analysis of Human and Chinchilla Temporal Bones.

    Science.gov (United States)

    Schachern, Patricia A; Kwon, Geeyoun; Briles, David E; Ferrieri, Patricia; Juhn, Steven; Cureoglu, Sebahattin; Paparella, Michael M; Tsuprun, Vladimir

    2017-10-01

    Bacterial resistance in acute otitis can result in bacterial persistence and biofilm formation, triggering chronic and recurrent infections. To investigate the middle ear inflammatory response to bacterial infection in human and chinchilla temporal bones. Six chinchillas underwent intrabullar inoculations with 0.5 mL of 106 colony-forming units (CFUs) of Streptococcus pneumoniae, serotype 2. Two days later, we counted bacteria in middle ear effusions postmortem. One ear from each chinchilla was processed in paraffin and sectioned at 5 µm. The opposite ear was embedded in epoxy resin, sectioned at a thickness of 1 µm, and stained with toluidine blue. In addition, we examined human temporal bones from 2 deceased donors with clinical histories of otitis media (1 with acute onset otitis media, 1 with recurrent infection). Temporal bones had been previously removed at autopsy, processed, embedded in celloidin, and cut at a thickness of 20 µm. Sections of temporal bones from both chinchillas and humans were stained with hematoxylin-eosin and immunolabeled with antifibrin and antihistone H4 antibodies. Histopatological and imminohistochemical changes owing to otitis media. Bacterial counts in chinchilla middle ear effusions 2 days after inoculation were approximately 2 logs above initial inoculum counts. Both human and chinchilla middle ear effusions contained bacteria embedded in a fibrous matrix. Some fibers in the matrix showed positive staining with antifibrin antibody, others with antihistone H4 antibody. In acute and recurrent otitis media, fibrin and neutrophil extracellular traps (NETs) are part of the host inflammatory response to bacterial infection. In the early stages of otitis media the host defense system uses fibrin to entrap bacteria, and NETs function to eliminate bacteria. In chronic otitis media, fibrin and NETs appear to persist.

  7. Does microtia predict severity of temporal bone CT abnormalities in children with persistent conductive hearing loss?

    Science.gov (United States)

    Tekes, Aylin; Ishman, Stacey L; Baugher, Katherine M; Brown, David J; Lin, Sandra Y; Tunkel, David E; Unalp-Arida, Aynur; Huisman, Thierry A G M

    2013-07-01

    This study aimed to determine the spectrum of temporal bone computed tomography (CT) abnormalities in children with conductive hearing loss (CHL) with and without microtia. From 1993 to 2008, a total of 3396 pediatric records including CHL were reviewed at our institution and revealed 180 cases of persistent CHL, 46 of whom had diagnostic temporal bone CT examinations. All of these examinations were systematically reviewed by two pediatric neuroradiologists, working in consensus, who had 5 and 18 years, respectively, of dedicated pediatric neuroradiology experience. Of the 46 children, 16 were boys and 30 were girls (age: 0.2-16 years; mean: 5 years). Also, 21 (46%) children had microtia and 25 (54%) children did not, as determined by clinical evaluation. External auditory canal atresia/stenosis (EAC-A/S) was the most common anomaly in both microtia and non-microtia groups. Two or more anomalies were observed in 18/21 children with microtia. The frequency of EAC-A/S was greater in children with microtia versus those without it (86% versus 32%, respectively; P = 0.0003). Syndromic diagnoses were also significantly more frequently made in children with microtia versus those without microtia (76% versus 20%, respectively; P = 0.0001). Temporal bone CT scans were normal in 10 children (22%) with persistent CHL. Microtia is an important finding in children with CHL. EAC and middle ear/ossicle anomalies were significantly more frequently seen in children with microtia, and multiple anomalies and bilateral microtia were more common in children with syndromic associations. These findings highlight the importance of understanding the embryological development of the temporal bone. The presence of one anomaly should raise suspicion of the possibility of other anomalies, especially in the setting of microtia. Bilateral microtia and multiple anomalies should also raise suspicion of genetic syndromes. Copyright © 2012 Elsevier Masson SAS. All rights reserved.

  8. Visualization of subtle temporal bone structures. Comparison of cone beam CT and MDCT

    International Nuclear Information System (INIS)

    Pein, M.K.; Plontke, S.K.; Brandt, S.; Koesling, S.

    2014-01-01

    The purpose of this study was to compare the visualization of subtle, non-pathological temporal bone structures on cone beam computed tomography (CBCT) and multi-detector computed tomography (MDCT) in vivo. Temporal bone studies of images from 38 patients archived in the picture archiving and communication system (PACS) were analyzed (slice thickness MDCT 0.6 mm and CBCT 0.125 mm) of which 23 were imaged by MDCT and 15 by CBCT using optimized standard protocols. Inclusion criteria were normal radiological findings, absence of previous surgery and anatomical variants. Images were evaluated blind by three trained observers. Using a five-point scale the visualization of ten subtle structures of the temporal bone was analyzed. Subtle middle ear structures showed a tendency to be more easily distinguishable by CBCT with significantly better visualization of the tendon of the stapedius muscle and the crura of the stapes on CBCT (p = 0.003 and p = 0.033, respectively). In contrast, inner ear components, such as the osseus spiral lamina and the modiolus tended to be better detectable on MDCT, showing significant differences for the osseous spiral lamina (p = 0.001). The interrater reliability was 0.73 (Cohen's kappa coefficient) and intraobserver reliability was 0.89. The use of CBCT and MDCT allows equivalent and excellent imaging results if optimized protocols are chosen. With both imaging techniques subtle temporal bone structures could be visualized with a similar degree of definition. In vivo differences do not seem to be as large as suggested in several previous studies. (orig.) [de

  9. Inflammation and tumors of the temporal bone; Entzuendungen und Tumoren des Schlaefenbeins

    Energy Technology Data Exchange (ETDEWEB)

    Burian, M. [Universitaetsklinik fuer Hals-, Nasen- und Ohrenkrankheiten, Allgemeines Krankenhaus, Wien (Austria)

    1997-12-01

    The term `inflammation of the middle ear` covers a couple of deseases which range from the acute otitis media to the middle ear cholesteatoma. However, a clear characterization of a certain pathology is essential for any further treatment. Therefore this article presents a short overview about the different types of infections and their clinical manifestation. The tumors of the temporal bone show a great variety in their incidence. Even if tumors like the acoustic neurinoma or the paraganglioma are compareable common, the chondroblastoma of the temporal bone is absolutely rare. In spite of these differences the individual temporal bone neoplasias are shortly mentioned herein. (orig.) [Deutsch] Der Begriff Mittelohrentzuendung umfasst ein weites Spektrum von Krankheiten welches von der akuten Mittelohrentzuendung bis hin zum Cholesteatom reicht. Es soll in diesem Artikel eine kurze Uebersicht ueber die verschiedenen Entzuendungen gegeben werden, wobei vor allem auf eine klare Begriffsdefinition der einzelnen Entzuendungsformen und deren klinisches Erscheinungsbild geachtet wurde. Bei den Tumoren des Schlaefenbeins ist ein grosser Unterschied in der Inzidenz der einzelnen Tumoren gegeben. Waehrend Neubildungen wie das Akustikusneurinom oder das Paragangliom vergleichsweise haeufig im klinischen Alltag zu sehen sind, stellen Veraenderungen wie das Chondroblastom eine Raritaet dar. Trotz dieses Unterschieds im Vorkommen der verschiedenen Tumoren, wurde versucht, einen kurzen Gesamtueberblick ueber die Tumore des Mittel- und Innenohres zu geben. (orig.)

  10. Quantifying temporal bone morphology of great apes and humans: an approach using geometric morphometrics

    Science.gov (United States)

    Lockwood, Charles A; Lynch, John M; Kimbel, William H

    2002-01-01

    The hominid temporal bone offers a complex array of morphology that is linked to several different functional systems. Its frequent preservation in the fossil record gives the temporal bone added significance in the study of human evolution, but its morphology has proven difficult to quantify. In this study we use techniques of 3D geometric morphometrics to quantify differences among humans and great apes and discuss the results in a phylogenetic context. Twenty-three landmarks on the ectocranial surface of the temporal bone provide a high level of anatomical detail. Generalized Procrustes analysis (GPA) is used to register (adjust for position, orientation and scale) landmark data from 405 adults representing Homo, Pan, Gorilla and Pongo. Principal components analysis of residuals from the GPA shows that the major source of variation is between humans and apes. Human characteristics such as a coronally orientated petrous axis, a deep mandibular fossa, a projecting mastoid process, and reduced lateral extension of the tympanic element strongly impact the analysis. In phenetic cluster analyses, gorillas and orangutans group together with respect to chimpanzees, and all apes group together with respect to humans. Thus, the analysis contradicts depictions of African apes as a single morphotype. Gorillas and orangutans lack the extensive preglenoid surface of chimpanzees, and their mastoid processes are less medially inflected. These and other characters shared by gorillas and orangutans are probably primitive for the African hominid clade. PMID:12489757

  11. Bilateral Non-Hodgkin’s Lymphoma of the Temporal Bone: A Rare and Unusual Presentation

    Directory of Open Access Journals (Sweden)

    Sanjay Vaid

    2016-01-01

    Full Text Available Primary lymphoma of the temporal bone is an unusual finding in clinical practice and bilateral affection is even more rare. To the best of our knowledge, there are no reports of bilateral primary temporal bone lymphoma without middle ear involvement in the English medical literature so far. We report, for the first time, a case of primary lymphoma involving both temporal bones which presented with left-sided infranuclear facial palsy. A combination of contrast enhanced magnetic resonance imaging (MRI and high resolution computed tomography (HRCT was used to characterize and to map the extent of the lesion, as well as to identify the exact site of facial nerve affection. An excision biopsy and immunohistochemistry revealed diffuse large B-cell non-Hodgkin’s lymphoma (DLBCL. Whole body fluorodeoxyglucose (FDG positron emission tomography-computed tomography study (PET-CT was performed to stage the disease. The patient was treated with chemotherapy and radiation therapy and is now on regular follow-up. The patient is alive and asymptomatic without disease progression for the last twenty months after initial diagnosis.

  12. Morphometrical Study of the Temporal Bone and Auditory Ossicles in Guinea Pig

    Directory of Open Access Journals (Sweden)

    Ahmadali Mohammadpour

    2011-03-01

    Full Text Available In this research, anatomical descriptions of the structure of the temporal bone and auditory ossicles have been performed based on dissection of ten guinea pigs. The results showed that, in guinea pig temporal bone was similar to other animals and had three parts; squamous, tympanic and petrous .The tympanic part was much better developed and consisted of oval shaped tympanic bulla with many recesses in tympanic cavity. The auditory ossicles of guinea pig concluded of three small bones; malleus, incus and stapes but the head of the malleus and the body of incus were fused and forming a malleoincudal complex. The average of morphometric parameters showed that the malleus was 3.53 ± 0.22 mm in total length. In addition to head and handle, the malleus had two distinct process; lateral and muscular. The incus had a total length 1.23 ± 0.02mm. It had long and short crus although the long crus was developed better than short crus. The lenticular bone was a round bone that articulated with the long crus of incus. The stapes had a total length 1.38 ± 0.04mm. The anterior crus(0.86 ± 0.08mm was larger than posterior crus (0.76 ± 0.08mm. It is concluded that, in the guinea pig, the malleus and the incus are fused, forming a junction called incus-malleus, while in the other animals these are separate bones. The stapes is larger and has a triangular shape and the anterior and posterior crus are thicker than other rodents. Therefore, for otological studies, the guinea pig is a good lab animal.

  13. Unusual facies, arthrogryposis, advanced skeletal maturation and unique bone changes. A new congenital malformation syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Jequier, S.; Kozlowski, K.

    1987-07-01

    Two strikingly similar infant siblings showed the following pattern of anomalies: unusual cranio-facial appearance, arthrogryposis, advanced bone age of the hips and unique skeletal X-ray abnormalities. They represent a previously unrecognised, fatal malformation syndrome.

  14. 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.

  15. Familial congenital bowing with short thick bones and metaphyseal changes, a distinct entity

    International Nuclear Information System (INIS)

    Rezza, E.; Lendvai, D.; Iannaccone, G.

    1984-01-01

    The authors describe two siblings, a male and a female, with disproportionate short stature, rhizomelic-mesomelic shortening of the limb bones, marked bowing of the femora, moderate bowing of the humeri, radii and ulnae, straight tibiae and fibulae, normal hands, flared cupped metaphyses of the tibiae, ulnae, raddi and ribs, and narrow chest. There was some improvement of the bone changes with advancing age. These two patients are similar to five other cases from the literature and strongly support Hall and Spranger's view that this pseudocampomelic condition most likely represents a distinct familial bowing syndrome. The differential diagnosis and the hereditary aspects in the two patients, are also briefly discussed. (orig.)

  16. Long-term Compliance and Satisfaction With Percutaneous Bone Conduction Devices in Patients With Congenital Unilateral Conductive Hearing Loss.

    Science.gov (United States)

    Nelissen, Rik C; Mylanus, Emmanuel A M; Cremers, Cor W R J; Hol, Myrthe K S; Snik, Ad F M

    2015-06-01

    Patients with congenital unilateral conductive hearing loss (UCHL) can either be watchful monitored or treated surgically through the fitting of a percutaneous bone conduction device (BCD) or, in some cases, atresia repair. The current study evaluated the long-term compliance and satisfaction with a percutaneous BCD in this specific population. Fifty-three consecutive patients with congenital UCHL treated with a percutaneous BCD in our tertiary referral center between 1998 and 2011 were identified. Clinical and audiological data were retrospectively gathered from the patients' files. The patients were interviewed by telephone about their current device usage status and were asked to complete the Speech, Spatial and Qualities of Hearing Scale (SSQ). Compliance with the BCD was 56.6% after a mean follow-up of 7 years. The mean age at implantation of the users (22 years) was significantly higher than that of the nonusers (10 years). The mean time of device usage before the patients stopped using the BCD was 5 years. The primary reasons mentioned for quitting the BCD were experiencing excess background noise and/or subjectively not receiving enough benefit. Objectively measured features of binaural processing affected by the BCD were found to correlate with long-term BCD usage. The SSQ revealed significant improvement in the aided condition compared with the nonaided condition in the users, in contrast to the nonusers. The current disappointing long-term compliance figures indicate the need for an even more careful and individualized approach with life-long follow-up when fitting BCDs in this specific population, especially in children.

  17. Direct radiocarbon dating and DNA analysis of the Darra-i-Kur (Afghanistan) human temporal bone.

    Science.gov (United States)

    Douka, Katerina; Slon, Viviane; Stringer, Chris; Potts, Richard; Hübner, Alexander; Meyer, Matthias; Spoor, Fred; Pääbo, Svante; Higham, Tom

    2017-06-01

    The temporal bone discovered in the 1960s from the Darra-i-Kur cave in Afghanistan is often cited as one of the very few Pleistocene human fossils from Central Asia. Here we report the first direct radiocarbon date for the specimen and the genetic analyses of DNA extracted and sequenced from two areas of the bone. The new radiocarbon determination places the find to ∼4500 cal BP (∼2500 BCE) contradicting an assumed Palaeolithic age of ∼30,000 years, as originally suggested. The DNA retrieved from the specimen originates from a male individual who carried mitochondrial DNA of the modern human type. The petrous part yielded more endogenous ancient DNA molecules than the squamous part of the same bone. Molecular dating of the Darra-i-Kur mitochondrial DNA sequence corroborates the radiocarbon date and suggests that the specimen is younger than previously thought. Taken together, the results consolidate the fact that the human bone is not associated with the Pleistocene-age deposits of Darra-i-Kur; instead it is intrusive, possibly re-deposited from upper levels dating to much later periods (Neolithic). Despite its Holocene age, the Darra-i-Kur specimen is, so far, the first and only ancient human from Afghanistan whose DNA has been sequenced. Copyright © 2017 Elsevier Ltd. All rights reserved.

  18. The temporal bones from Sima de los Huesos Middle Pleistocene site (Sierra de Atapuerca, Spain). A phylogenetic approach.

    Science.gov (United States)

    Martínez, I; Arsuaga, J L

    1997-01-01

    Three well-preserved crania and 22 temporal bones were recovered from the Sima de los Huesos Middle Pleistocene site up to and including the 1994 field season. This is the largest sample of hominid temporal bones known from a single Middle Pleistocene site and it offers the chance to characterize the temporal bone morphology of an European Middle Pleistocene population and to study the phylogenetic relationships of the SH sample with other Upper and Middle Pleistocene hominids. We have carried out a cladistic analysis based on nine traits commonly used in phylogenetic analysis of Middle and Late Pleistocene hominids: shape of the temporal squama superior border, articular eminence morphology, contribution of the sphenoid bone to the median glenoid wall, postglenoid process projection, tympanic plate orientation, presence of the styloid process, mastoid process projection, digastric groove morphology and anterior mastoid tubercle. We have found two autapomorphies on the Home erectus temporal bone: strong reduction of the postglenoid process and absence of the styloid process. Modern humans, Neandertals and the Middle Pleistocene fossils from Europe and Africa constitute a clade characterized by a convex superior border of the temporal squama. The European Middle Pleistocene fossils from Sima de los Huesos, Petralona, Steinheim, Bilzingsleben and Castel di Guido share a Neandertal apomorphy: a relatively flat articular eminence. The fossils from Ehringsdorf, La Chaise Suardi and Biache-Saint-Vaast also display another Neandertal derived trait: an anteriorly obliterated digastric groove. Modern humans and the African Middle Pleistocene fossils share a synapomorphy: a sagittally orientated tympanic plate.

  19. Intraoperative cone-beam computed tomography and multi-slice computed tomography in temporal bone imaging for surgical treatment.

    Science.gov (United States)

    Erovic, Boban M; Chan, Harley H L; Daly, Michael J; Pothier, David D; Yu, Eugene; Coulson, Chris; Lai, Philip; Irish, Jonathan C

    2014-01-01

    Conventional computed tomography (CT) imaging is the standard imaging technique for temporal bone diseases, whereas cone-beam CT (CBCT) imaging is a very fast imaging tool with a significant less radiation dose compared with conventional CT. We hypothesize that a system for intraoperative cone-beam CT provides comparable image quality to diagnostic CT for identifying temporal bone anatomical landmarks in cadaveric specimens. Cross-sectional study. University tertiary care facility. Twenty cadaveric temporal bones were affixed into a head phantom and scanned with both a prototype cone-beam CT C-arm and multislice helical CT. Imaging performance was evaluated by 3 otologic surgeons and 1 head and neck radiologist. Participants were presented images in a randomized order and completed landmark identification questionnaires covering 21 structures. CBCT and multislice CT have comparable performance in identifying temporal structures. Three otologic surgeons indicated that CBCT provided statistically equivalent performance for 19 of 21 landmarks, with CBCT superior to CT for the chorda tympani and inferior for the crura of the stapes. Subgroup analysis showed that CBCT performed superiorly for temporal bone structures compared with CT. The radiologist rated CBCT and CT as statistically equivalent for 18 of 21 landmarks, with CT superior to CBCT for the crura of stapes, chorda tympani, and sigmoid sinus. CBCT provides comparable image quality to conventional CT for temporal bone anatomical sites in cadaveric specimens. Clinical applications of low-dose CBCT imaging in surgical planning, intraoperative guidance, and postoperative assessment are promising but require further investigation.

  20. Three dimensional CT of stapes. Stapedial imagings in dry temporal bone and clinical applications

    Energy Technology Data Exchange (ETDEWEB)

    Edamatsu, Hideo; Kubota, Osamu; Yamashita, Koichi [Kanazawa Medical Univ., Ishikawa (Japan)

    1995-03-01

    This study was performed to evaluate the usefulness and limitations of three dimensional (3-D) imagings of stapes in the middle ear by high speed helical CT. One dissected human temporal bone, ten normal and diseased ears were scanned with a slice of 1.0 mm and reconstructed in a thickness of 0.2-0.5 mm. Every specimen of 3-D can be observed in any plane and from any direction. Ossicular imagings of the temporal bone in 3-D were reconstructed as if the malleus, incus and stapes were observed under microscope. The whole structure of stapes was impossible to be represented by two dimensional CT heretofore in use, but 3-D in our study showed the head, crus and foot plate of the stapes in detail. Stapedial imagings of 3-D CT in normal ears showed the same findings as those recorded in temporal bone. Preoperative diagnostic findings of ossicles in the affected ears were very useful. Especially in ossicular anomalies, 3-D CT was positive in diagnosis and its accuracies were confirmed with operative observation. For the postoperative evaluation concerning the ossicular reconstruction, i.e. TORP and PORP, 3-D CT was also important method. It could present an anatomical relation between those prosthesis and the oval window. High speed helical CT can scan an object more quickly and clearly than formerly used CT, and its biological damage for human is less than that of the others. 3-D CT can be more clearly reconstructed with helical CT than former CT. (author).

  1. Accuracy of linear drilling in temporal bone using drill press system for minimally invasive cochlear implantation.

    Science.gov (United States)

    Dillon, Neal P; Balachandran, Ramya; Labadie, Robert F

    2016-03-01

    A minimally invasive approach for cochlear implantation involves drilling a narrow linear path through the temporal bone from the skull surface directly to the cochlea for insertion of the electrode array without the need for an invasive mastoidectomy. Potential drill positioning errors must be accounted for to predict the effectiveness and safety of the procedure. The drilling accuracy of a system used for this procedure was evaluated in bone surrogate material under a range of clinically relevant parameters. Additional experiments were performed to isolate the error at various points along the path to better understand why deflections occur. An experimental setup to precisely position the drill press over a target was used. Custom bone surrogate test blocks were manufactured to resemble the mastoid region of the temporal bone. The drilling error was measured by creating divots in plastic sheets before and after drilling and using a microscope to localize the divots. The drilling error was within the tolerance needed to avoid vital structures and ensure accurate placement of the electrode; however, some parameter sets yielded errors that may impact the effectiveness of the procedure when combined with other error sources. The error increases when the lateral stage of the path terminates in an air cell and when the guide bushings are positioned further from the skull surface. At contact points due to air cells along the trajectory, higher errors were found for impact angles of [Formula: see text] and higher as well as longer cantilevered drill lengths. The results of these experiments can be used to define more accurate and safe drill trajectories for this minimally invasive surgical procedure.

  2. Mucous retention cyst of temporal bone: a mimic of cholesteatoma on DW-MRI.

    Science.gov (United States)

    Karandikar, Amit; Goh, Julian; Loke, Siu Cheng; Yeo, Seng Beng; Tan, Tiong Yong

    2013-01-01

    Non-EPI DW imaging is increasingly being used as a sensitive sequence in detecting cholesteatomas especially if CT findings are not confirmatory. Cholesteatoma appears as a hyperintense focus on DWI. We present two cases of mucous retention cysts in the mastoid temporal bone/middle ear cavity, which present as hyperintense on non-EPI DWI and potentially may mimic cholesteatomas. Differentiating between the two conditions is important, as surgery can be avoided in mucous retention cysts. We have also discussed ways to differentiate between these two conditions on MRI. To our knowledge, this entity is not reported previously. © 2013 Elsevier Inc. All rights reserved.

  3. Destructive, granulating lesion in the temporal bone after elevated plasma homocysteine

    DEFF Research Database (Denmark)

    Bonding, Per; Skriver, Elisabeth; Helin, Pekka

    2004-01-01

    lesion in the left temporal bone was discovered; repeated histologic examination only showed simple granulation tissue. After 6 months, a part of the bony cochlea was extruded. With approximately 8 months' delay and after the patient had had postoperative lung embolism, plasma homocysteine was found...... to be significantly elevated, a condition known as an independent risk factor for thromboembolic lesions. In the acquired form, it is most often caused by nutritional deficiency of vitamin B cofactors. Accordingly, the patient was treated with folic acid, which rapidly normalized plasma homocysteine. Subsequently...

  4. Bilateral cochlear implantation in a patient with bilateral temporal bone fractures.

    Science.gov (United States)

    Chung, Jae Ho; Shin, Myung Chul; Min, Hyun Jung; Park, Chul Won; Lee, Seung Hwan

    2011-01-01

    With the emphasis on bilateral hearing nowadays, bilateral cochlear implantation has been tried out for bilateral aural rehabilitation. Bilateral sensorineural hearing loss caused by head trauma can get help from cochlear implantation. We present the case of a 44-year-old man with bilateral otic capsule violating temporal bone fractures due to head trauma. The patient demonstrated much improved audiometric and psychoacoustic performance after bilateral cochlear implantation. We believe bilateral cochlear implantation in such patient can be a very effective tool for rehabilitation. Copyright © 2011 Elsevier Inc. All rights reserved.

  5. Modifications to a 3D-printed temporal bone model for augmented stapes fixation surgery teaching.

    Science.gov (United States)

    Nguyen, Yann; Mamelle, Elisabeth; De Seta, Daniele; Sterkers, Olivier; Bernardeschi, Daniele; Torres, Renato

    2017-07-01

    Functional outcomes and complications in otosclerosis surgery are governed by the surgeon's experience. Thus, teaching the procedure to residents to guide them through the learning process as quickly as possible is challenging. Artificial 3D-printed temporal bones are replacing cadaver specimens in many institutions to learn mastoidectomy, but these are not suitable for middle ear surgery training. The goal of this work was to adapt such an artificial temporal bone to aid the teaching of otosclerosis surgery and to evaluate this tool. We have modified a commercially available 3D-printed temporal bone by replacing the incus and stapes of the model with in-house 3D-printed ossicles. The incus could be attached to a 6-axis force sensor. The stapes footplate was fenestrated and attached to a 1-axis force sensor. Six junior surgeons (residents) and seven senior surgeons (fellows or consultants) were enrolled to perform piston prosthesis placement and crimping as performed during otosclerosis surgery. The time required to perform the tasks and the forces applied to the incus and stapes were collected and analyzed. No statistically significant differences were observed between the junior and senior groups for time taken to perform the tasks and the forces applied to the incus during crimping and placement of the prosthesis. However, significantly lower forces were applied to the stapes by the senior surgeons in comparison with the junior surgeons during prosthesis placement (junior vs senior group, 328 ± 202.9 vs 80 ± 99.6 mN, p = 0.008) and during prosthesis crimping (junior vs senior group, 565 ± 233 vs 66 ± 48.6 mN, p = 0.02). We have described a new teaching tool for otosclerosis surgery based on the modification of a 3D-printed temporal bone to implement force sensors on the incus and stapes. This tool could be used as a training tool to help the residents to self-evaluate their progress with recording of objective measurements.

  6. Petrified ears in a patient with Keutel syndrome: temporal bone CT findings

    International Nuclear Information System (INIS)

    Parmar, Hemant; Blaser, Susan; Yoo, Shi-Joon; Unger, Sheila; Papsin, Blake

    2006-01-01

    We present unusual imaging findings of petrified ears in a 9-year-old girl with Keutel syndrome. The patient presented for a temporal bone study for hearing loss. CT scan showed middle and inner ear abnormalities along with extensive and unsuspected calcification of the external ears and ossicular ligaments. On further investigation, the patient was found to have diffuse cartilage calcification in the larynx and tracheobronchial tree, brachytelephalangism and peripheral pulmonary stenosis suggestive of Keutel syndrome. Confirmation was obtained by mutation analysis. (orig.)

  7. Primary chondroid chordoma arising from the petrous temporal bone: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Young Uk; Youn, Eun Kyung [Koryo General Hospital, Seoul (Korea, Republic of)

    1991-01-15

    Chordomas are uncommon tumors which arise from remnants of the primitive notochord. They are situated chiefly in the anterior spinal axis with a predilection for the sacrococcygeal region and the basiocciput. About 50% of chordomas are sacrococcygeal, 35% are intracranial, and 15% arise from a vertebral body. As a histologic variant of chordoma, /chondroid chordoma' was first described by Heffelfinger et al. We present a rare case of primary chondroid chordoma arising from the petrous temporal bone. To our knowledge, only two other cases of this type have been reported earlier.

  8. Primary chondroid chordoma arising from the petrous temporal bone: a case report

    International Nuclear Information System (INIS)

    Lee, Young Uk; Youn, Eun Kyung

    1991-01-01

    Chordomas are uncommon tumors which arise from remnants of the primitive notochord. They are situated chiefly in the anterior spinal axis with a predilection for the sacrococcygeal region and the basiocciput. About 50% of chordomas are sacrococcygeal, 35% are intracranial, and 15% arise from a vertebral body. As a histologic variant of chordoma, /chondroid chordoma' was first described by Heffelfinger et al. We present a rare case of primary chondroid chordoma arising from the petrous temporal bone. To our knowledge, only two other cases of this type have been reported earlier

  9. Comparison of cadaveric and isomorphic three-dimensional printed models in temporal bone education.

    Science.gov (United States)

    Hochman, Jordan B; Rhodes, Charlotte; Wong, Dana; Kraut, Jay; Pisa, Justyn; Unger, Bertram

    2015-10-01

    Current three-dimensional (3D) printed simulations are complicated by insufficient void spaces and inconsistent density. We describe a novel simulation with focus on internal anatomic fidelity and evaluate against template/identical cadaveric education. Research ethics board-approved prospective cohort study. Generation of a 3D printed temporal bone was performed using a proprietary algorithm that deconstructs the digital model into slices prior to printing. This supplemental process facilitates removal of residual material from air-containing spaces and permits requisite infiltrative access to the all regions of the model. Ten otolaryngology trainees dissected a cadaveric temporal bone (CTB) followed by a matched/isomorphic 3D printed bone model (PBM), based on derivative micro-computed tomography data. Participants rated 1) physical characteristics, 2) specific anatomic constructs, 3) usefulness in skill development, and 4) perceived educational value. The survey instrument employed a seven-point Likert scale. Trainees felt physical characteristics of the PBM were quite similar to CTB, with highly ranked cortical (5.5 ± 1.5) and trabecular (5.2 ± 1.3) bone drill quality. The overall model was considered comparable to CTB (5.9 ± 0.74), with respectable air cell reproduction (6.1 ± 1.1). Internal constructs were rated as satisfactory (range, 4.9-6.2). The simulation was considered a beneficial training tool for all types of mastoidectomy (range, 5.9-6.6), posterior tympanotomy (6.5 ± 0.71), and skull base approaches (range, 6-6.5). Participants believed the model to be an effective training instrument (6.7 ± 0.68), which should be incorporated into the temporal bone lab (7.0 ± 0.0). The PBM was thought to improve confidence (6.7 ± 0.68) and operative performance (6.7 ± 0.48). Study participants found the PBM to be an effective platform that compared favorably to CTB. The model was considered a valuable adjunctive

  10. Differential diagnosis between chronic otitis media with and without cholesteatoma by temporal bone CT: focus on bone change and mass effect

    International Nuclear Information System (INIS)

    Jung, Cheol Kyu; Park, Dong Woo; Seong, Jin Yong; Lee, Kak Soo; Park Choong Ki; Lee, Seung Ro; Hahm, Chang Kok

    2000-01-01

    In order to determine specific differences, we compared the temporal bone CT findings of chronic otitis media (COM) with and without cholesteatoma, focusing on bone change. Between 1997 and 1998, 82 patients (84 cases) underwent temporal bone CT and were shown to have COM, with or without cholesteatoma after mastoidectomy and tympanoplasty. There were 36 cases of COM with cholesteatoma (26 patients, M:F =3D 11:15; age range, 16-61 (mean, 36,2) years), and 58 cases without chlesteatoma (56 patients, M:F =3D 25:31, age range, 15-61 (mean, 36.2) years). The findings of temporal bone CT were analysed at the point of bony changes including erosion and medial displacement of ossicles (malleus, incus, and stapes), erosion or destruction of the scutum, tegmen, facial canal, and lateral semicircular canal, and ballooning of the tympanic cavity and mastoid antrum. In addition, the soft tissue changes seen on temporal bone CT were analyzed at the site of lateral bulging of soft tissue in Prussak's space, perforation of the pars flaccida, tympanic membrane retraction, and tympanosclerosis. We retrospectively compared the findings of temporal bone CT with the surgical findings, and to assess statistical significance, the Chi-square test was used. Bone erosion or destruction was seen in 36.2% of COM cases without cholesteatoma, and in 96.2% of cases with cholesteatoma. Comparing COM with and without cholesteatoma, the erosion of ossicles including the malleus (81%, 24%), incus (88%, 14%), stapes (58%, 10%), scutum (88%, 10%), facial canal (8%, 0%), and lateral semicircular canal (8%, 0%), was more common in COM with cholesteatoma (p-value less than 0.05), with the exception of erosion of the tegmen (8%, 3%). Other bony changes including medial displacement of ossicles (27%, 3%), ballooning of tympanic cavity and mastoid antrum (96%, 16%), and the soft tissue changes including lateral bulging of soft tissue in Prussak's space (58%, 14%) and perforation of the pars flaccida (35

  11. Differential diagnosis between chronic otitis media with and without cholesteatoma by temporal bone CT: focus on bone change and mass effect

    Energy Technology Data Exchange (ETDEWEB)

    Jung, Cheol Kyu; Park, Dong Woo; Seong, Jin Yong; Lee, Kak Soo; Park Choong Ki; Lee, Seung Ro; Hahm, Chang Kok [College of Medicine, Hanyang University, Seoul (Korea, Republic of)

    2000-01-01

    In order to determine specific differences, we compared the temporal bone CT findings of chronic otitis media (COM) with and without cholesteatoma, focusing on bone change. Between 1997 and 1998, 82 patients (84 cases) underwent temporal bone CT and were shown to have COM, with or without cholesteatoma after mastoidectomy and tympanoplasty. There were 36 cases of COM with cholesteatoma (26 patients, M:F =3D 11:15; age range, 16-61 (mean, 36,2) years), and 58 cases without chlesteatoma (56 patients, M:F =3D 25:31, age range, 15-61 (mean, 36.2) years). The findings of temporal bone CT were analysed at the point of bony changes including erosion and medial displacement of ossicles (malleus, incus, and stapes), erosion or destruction of the scutum, tegmen, facial canal, and lateral semicircular canal, and ballooning of the tympanic cavity and mastoid antrum. In addition, the soft tissue changes seen on temporal bone CT were analyzed at the site of lateral bulging of soft tissue in Prussak's space, perforation of the pars flaccida, tympanic membrane retraction, and tympanosclerosis. We retrospectively compared the findings of temporal bone CT with the surgical findings, and to assess statistical significance, the Chi-square test was used. Bone erosion or destruction was seen in 36.2% of COM cases without cholesteatoma, and in 96.2% of cases with cholesteatoma. Comparing COM with and without cholesteatoma, the erosion of ossicles including the malleus (81%, 24%), incus (88%, 14%), stapes (58%, 10%), scutum (88%, 10%), facial canal (8%, 0%), and lateral semicircular canal (8%, 0%), was more common in COM with cholesteatoma (p-value less than 0.05), with the exception of erosion of the tegmen (8%, 3%). Other bony changes including medial displacement of ossicles (27%, 3%), ballooning of tympanic cavity and mastoid antrum (96%, 16%), and the soft tissue changes including lateral bulging of soft tissue in Prussak's space (58%, 14%) and perforation of the pars

  12. Impact of congenital calcitonin deficiency due to dysgenetic hypothyroidism on bone mineral density

    Directory of Open Access Journals (Sweden)

    Daripa M.

    2004-01-01

    Full Text Available The objective of the present study was to determine the effect of chronic calcitonin deficiency on bone mass development. The results of 11 patients with thyroid dysgenesis (TD were compared to those of 17 normal individuals (C and of 9 patients with other forms of hypothyroidism (OH: 4 with hypothyroidism due to inborn errors of thyroid hormone synthesis and 5 with Hashimoto's thyroiditis. The subjects received an intravenous calcium stimulus and blood was collected for the determination of ionized calcium (Ca2+, calcitonin, and intact parathyroid hormone. Bone mineral density (BMD was determined by dual-energy X-ray absorptiometry. After calcium administration the levels of Ca2+ in the two groups of hypothyroidism were significantly higher than in the normal control group (10 min after starting calcium infusion: C = 1.29 ± 0.08 vs TD = 1.34 ± 0.03 vs OH = 1.34 ± 0.02 mmol/l; P < 0.05, and only the TD group showed no calcitonin response (5 min after starting calcium infusion: C = 27.9 ± 5.8 vs TD = 6.6 ± 0.3 vs OH = 43.0 ± 13.4 ng/l. BMD values did not differ significantly between groups (L2-L4: C = 1.116 ± 0.02 vs TD = 1.109 ± 0.03 vs OH = 1.050 ± 0.04 g/cm². These results indicate that early deficiency of calcitonin secretion has no detrimental effect on bone mass development. Furthermore, the increased calcitonin secretion observed in patients with inborn errors of thyroid hormone biosynthesis does not confer any advantage in terms of BMD.

  13. 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.)

  14. Spatial and temporal expression of glucocorticoid, retinoid, and thyroid hormone receptors is not altered in lungs of congenital diaphragmatic hernia

    NARCIS (Netherlands)

    Rajatapiti, Prapapan; Keijzer, Richard; Blommaart, Pietjan E.; Lamers, Wouter H.; de Krijger, Ronald R.; Visser, Theo J.; Tibboel, Dick; Rottier, Robbert

    2006-01-01

    The degree of associated pulmonary hypoplasia and persistent pulmonary hypertension are major determination factors for survival in congenital diaphragmatic hernia (CDH) patients. Glucocorticoids, thyroid hormone, and vitamin A have been shown to be involved in human lung development. To determine

  15. Insertion forces and intracochlear trauma in temporal bone specimens implanted with a straight atraumatic electrode array.

    Science.gov (United States)

    Mirsalehi, Marjan; Rau, Thomas S; Harbach, Lenka; Hügl, Silke; Mohebbi, Saleh; Lenarz, Thomas; Majdani, Omid

    2017-05-01

    The aim of the study was to evaluate insertion forces during manual insertion of a straight atraumatic electrode in human temporal bones, and post-implantation histologic evaluation of the samples to determine whether violation of intracochlear structures is related to insertion forces. In order to minimize intracochlear trauma and preserve residual hearing during cochlear implantation, knowledge of the insertion forces is necessary. Ten fresh frozen human temporal bones were prepared with canal wall down mastoidectomy. All samples were mounted on a one-axis force sensor. Insertion of a 16-mm straight atraumatic electrode was performed from different angles to induce "traumatic" insertion. Histologic evaluation was performed in order to evaluate intracochlear trauma. In 4 of 10 samples, dislocation of the electrode into scala vestibuli was observed. The mean insertion force for all 10 procedures was 0.003 ± 0.005 N. Insertion forces measured around the site of dislocation to scala vestibuli in 3 of 4 samples were significantly higher than insertion forces at the same location of the cochleae measured in samples without trauma (p straight atraumatic electrode is lower than reported by other studies using longer electrodes. Based on our study, insertion forces leading to basilar membrane trauma may be lower than the previously reported direct rupture forces.

  16. Creating an Optimal 3D Printed Model for Temporal Bone Dissection Training.

    Science.gov (United States)

    Takahashi, Kuniyuki; Morita, Yuka; Ohshima, Shinsuke; Izumi, Shuji; Kubota, Yamato; Yamamoto, Yutaka; Takahashi, Sugata; Horii, Arata

    2017-07-01

    Making a 3-dimensional (3D) temporal bone model is simple using a plaster powder bed and an inkjet printer. However, it is difficult to reproduce air-containing spaces and precise middle ear structures. The objective of this study was to overcome these problems and create a temporal bone model that would be useful both as a training tool and for preoperative simulation. Drainage holes were made to remove excess materials from air-containing spaces, ossicle ligaments were manually changed to bony structures, and small and/or soft tissue structures were colored differently while designing the 3D models. The outcomes were evaluated by 3 procedures: macroscopic and endoscopic inspection of the model, comparison of computed tomography (CT) images of the model to the original CT, and assessment of tactile sensation and reproducibility by 20 surgeons performing surgery on the model. Macroscopic and endoscopic inspection, CT images, and assessment by surgeons were in agreement in terms of reproducibility of model structures. Most structures could be reproduced, but the stapes, tympanic sinus, and mastoid air cells were unsatisfactory. Perioperative tactile sensation of the model was excellent. Although this model still does not embody perfect reproducibility, it proved sufficiently practical for use in surgical training.

  17. Simplified Summative Temporal Bone Dissection Scale Demonstrates Equivalence to Existing Measures.

    Science.gov (United States)

    Pisa, Justyn; Gousseau, Michael; Mowat, Stephanie; Westerberg, Brian; Unger, Bert; Hochman, Jordan B

    2018-01-01

    Emphasis on patient safety has created the need for quality assessment of fundamental surgical skills. Existing temporal bone rating scales are laborious, subject to evaluator fatigue, and contain inconsistencies when conferring points. To address these deficiencies, a novel binary assessment tool was designed and validated against a well-established rating scale. Residents completed a mastoidectomy with posterior tympanotomy on identical 3D-printed temporal bone models. Four neurotologists evaluated each specimen using a validated scale (Welling) and a newly developed "CanadaWest" scale, with scoring repeated after a 4-week interval. Nineteen participants were clustered into junior, intermediate, and senior cohorts. An ANOVA found significant differences between performance of the junior-intermediate and junior-senior cohorts for both Welling and CanadaWest scales ( P .05). Cohen's kappa found strong intrarater reliability (0.711) with a high degree of interrater reliability of (0.858) for the CanadaWest scale, similar to scores on the Welling scale of (0.713) and (0.917), respectively. The CanadaWest scale was facile and delineated performance by experience level with strong intrarater reliability. Comparable to the validated Welling Scale, it distinguished junior from senior trainees but was challenged in differentiating intermediate and senior trainee performance.

  18. Middle cranial fossa approach to repair tegmen defects assisted by three-dimensionally printed temporal bone models.

    Science.gov (United States)

    Ahmed, Sameer; VanKoevering, Kyle K; Kline, Stephanie; Green, Glenn E; Arts, H Alexander

    2017-10-01

    To explore the perioperative utility of three-dimensionally (3D)-printed temporal bone models of patients undergoing repair of lateral skull base defects and spontaneous cerebrospinal fluid leaks with the middle cranial fossa approach. Case series. 3D-printed temporal bone models-based on patient-specific, high-resolution computed tomographic imaging-were constructed using inexpensive polymer materials. Preoperatively, the models demonstrated the extent of temporal lobe retraction necessary to visualize the proposed defects in the lateral skull base. Also preoperatively, Silastic sheeting was arranged across the modeled tegmen, marked, and cut to cover all of the proposed defect sites. The Silastic sheeting was then sterilized and subsequently served as a precise intraoperative template for a synthetic dural replacement graft. Of note, these grafts were customized without needing to retract the temporal lobe. Five patients underwent the middle cranial fossa approach assisted by 3D-printed temporal bone models to repair tegmen defects and spontaneous cerebrospinal fluid leaks. No complications were encountered. The prefabricated dural repair grafts were easily placed and fit precisely onto the middle fossa floor without any additional modifications. All defects were covered as predicted by the 3D temporal bone models. At their postoperative visits, all five patients maintained resolution of their spontaneous cerebrospinal fluid leaks. Inexpensive 3D-printed temporal bone models of tegmen defects can serve as beneficial adjuncts during lateral skull base repair. The models provide a panoramic preoperative view of all tegmen defects and allow for custom templating of dural grafts without temporal lobe retraction. 4 Laryngoscope, 127:2347-2351, 2017. © 2016 The American Laryngological, Rhinological and Otological Society, Inc.

  19. CT and MRI findings of temporal bone anomaly in patients with tinnitus

    International Nuclear Information System (INIS)

    Wang Bing; Xian Junfang; Wang Zhenchang; Liu Zhaohui

    2011-01-01

    Objective: To study high resolution CT (HRCT) and MRI findings of temporal hone anomaly in patients with tinnitus and identify the optimal examination method in the detection of the anomaly. Methods: The HRCT and MRI data were analyzed retrospectively in 1015 patients including 145 patients with pulsatile tinnitus (PT) and 870 patients with nonpulsatile tinnitus (NPT). The positive rates of HRCT and MRI in the identification of temporal bone anomaly were analyzed and the efficiency of various examination methods was compared in revealing the anomaly. Data were tested by Chi-square test analysis. Results: Among 1015 patients, anomaly was seen in 767 cases (75.57%). High jugular bulb was found in 414 patients, accounting for 40.79%. Sigmoid sinus anomaly was detected in 387 patients (38.13%), while otitis media was found in 148 cases (14.58%), and low middle cranial fossa in 70 cases (6.90%). The positive rate of HRCT in the detection of high jugular bulb was 54.89% (365/665), which was significantly higher than those of other methods (P 2 =56.537, P<0.01). The fast imaging employing steady-state acquisition (FIESTA) sequence was the best examination method in displaying the vessel within the internal auditory canal (42/42,100%). Conclusions: High jugular bulb and sigmoid sinus anomaly were the most frequent abnormal findings of temporal bone in patients with tinnitus. Enhanced HRCT was the choice of modality in patients with PT. Plain HRCT was recommended for NPT. FIESTA sequence was the best in the evaluation of the vessel within the internal auditory canal. (authors)

  20. Evaluation of temporal bone pneumatization on high resolution CT (HRCT) measurements of the temporal bone in normal and otitis media group and their correlation to measurements of internal auditory meatus, vestibular or cochlear aqueduct

    International Nuclear Information System (INIS)

    Nakamura, Miyako

    1988-01-01

    High resolution CT axial scans were made at the three levels of the temoral bone 91 cases. These cases consisted of 109 sides of normal pneumatization (NR group) and 73 of poor pneumatization resulted by chronic otitis (OM group). NR group included sensorineural hearing loss cases and/or sudden deafness on the side. Three levels of continuous slicing were chosen at the internal auditory meatus, the vestibular and the cochlear aqueduct, respectively. In each slice two sagittal and two horizontal measurements were done on the outer contour of the temporal bone. At the proper level, diameter as well as length of the internal acoustic meatus, the vestibular or the cochlear aqueduct were measured. Measurements of the temporal bone showed statistically significant difference between NR and OM groups. Correlation of both diameter and length of the internal auditory meatus to the temporal bone measurements were statistically significant. Neither of measurements on the vestibular or the cochlear aqueduct showed any significant correlation to that of the temporal bone. (author)

  1. [Congenital hemihypertrophy associated with cutaneous pigmento-vascular, cerebral, visceral and bone abnormalities].

    Science.gov (United States)

    Hidano, A; Arai, Y

    1987-01-01

    A case of hemihypertrophy associated with multiple anomalies of the skin, bone and visceral organs is presented. A 31-year-old female was admitted for evaluation of her skin conditions. Her family history is noncontributory, while her past history discloses operations for syndactyly of the right foot, tonsillar hypertrophy, anal prolapse and ovarial cyst. Erythemas of the face and the left upper extremity were noticed during the neonatal period and hypertrophy of the right side of the body started at age 2 months. On admission, hemihypertrophy was observed in the face, trunk and extremities. Multiple faint nevi flammei were seen on the right half of the face and on the left side of the trunk and extremities. Telangiectasis and nevus anemicus were seen in the upper chest. The left upper extremity showed diffuse brown patches that was histologically basal pigmentation with some giant melanosomes. Visceral anomalies consisted of fibromatous tumors of the tip of the tongue and mitral prolapse. Angiography and computed tomography revealed a possible arteriovenous malformation of the right occipital region, small hemangiomas around the patella, dilation of the lateral ventricle, and calcification of the choroid plexus. Tortuous superficial veins were noted in the right leg. She had no seizure, but her IQ was 68. The bone disorders consisted of scoliosis, short forth metacarpus, hypoplastic mandible and peroneal exostosis. Examination revealed a slight diminution of urinary corticosteroid, but no other endocrinological disorders were found. The hemihypertrophy in this case is at least partially due to an arteriovenous shunt, suggested by elevated oxygen saturation of the blood obtained from the internal saphenous vein.(ABSTRACT TRUNCATED AT 250 WORDS)

  2. Three-year experience with the Sophono in children with congenital conductive unilateral hearing loss: tolerability, audiometry, and sound localization compared to a bone-anchored hearing aid.

    Science.gov (United States)

    Nelissen, Rik C; Agterberg, Martijn J H; Hol, Myrthe K S; Snik, Ad F M

    2016-10-01

    Bone conduction devices (BCDs) are advocated as an amplification option for patients with congenital conductive unilateral hearing loss (UHL), while other treatment options could also be considered. The current study compared a transcutaneous BCD (Sophono) with a percutaneous BCD (bone-anchored hearing aid, BAHA) in 12 children with congenital conductive UHL. Tolerability, audiometry, and sound localization abilities with both types of BCD were studied retrospectively. The mean follow-up was 3.6 years for the Sophono users (n = 6) and 4.7 years for the BAHA users (n = 6). In each group, two patients had stopped using their BCD. Tolerability was favorable for the Sophono. Aided thresholds with the Sophono were unsatisfactory, as they did not reach under a mean pure tone average of 30 dB HL. Sound localization generally improved with both the Sophono and the BAHA, although localization abilities did not reach the level of normal hearing children. These findings, together with previously reported outcomes, are important to take into account when counseling patients and their caretakers. The selection of a suitable amplification option should always be made deliberately and on individual basis for each patient in this diverse group of children with congenital conductive UHL.

  3. Pneumatization of the zygomatic process of temporal bone on computed tomograms

    Directory of Open Access Journals (Sweden)

    Friedrich, Reinhard E.

    2016-06-01

    Full Text Available Purpose: Zygomatic air cells (ZAC are a variant of temporal bone pneumatization that needs no treatment. However, ZAC can have an impact on surgical procedures in the temporo-mandibular joint region. Recent reports suggest that computed tomography will disclose more ZAC than can be diagnosed on panoramic radiography. The aim of this study was to analyze ZAC prevalence on CT in a population that was not pre-selected by admission to a dental clinic. Furthermore, an extensive literature review was performed to assess the prevalence of ZAC and to address the impact of imaging technique on the definition of the item.Material and methods: Digitalized cranial CTs of 2007 patients were retrospectively analyzed. The Frankfort horizontal was used to define a ZAC on sagittal CTs. Results: In this study group, 806 were female (40.16% and 1,201 were male (59.84%. Mean age was 49.96 years in the whole group (female: 55.83 years, male: 46.01 years. A ZAC was diagnosed in 152 patients (female: 66, male: 86. Unilateral ZAC surpasses bilateral findings (115 vs. 37 patients. ZAC were diagnosed in children 5 years of age and older. Sectional imaging techniques show a better visualization of the region of interest. However, presently an increase of ZAC prevalence attributable to imaging technique cannot conclusively be derived from the current literature. The normal finding of a ZAC on radiograms is a sharply defined homogenous transparent lesion restricted to the zygomatic process of the temporal bone that has no volume effect on the shape of the process.Conclusion: ZAC is an anatomical variant of the temporal bone that has come into focus of maxillofacial radiology due to its noticeable aspect on panoramic radiograms. The harmless variant can be expected in about one in thirteen individuals undergoing facial radiology. Panoramic radiograms appear to be sufficient to present ZAC of relevant size. However, in preparation for surgical procedures affecting the

  4. Evaluation of the utility of temporal subtraction images in successive whole-body bone scans: a prospective clinical study

    International Nuclear Information System (INIS)

    Shiraishi, J.; Appelbaum, D.; Pu, Y.; Engelmann, R.; Li Qiang; Doi, K.

    2007-01-01

    We have begun a prospective clinical study for evaluating the clinical utility of temporal subtraction images in successive whole-body bone scans. The computerized temporal subtraction technique has been developed in order to highlight interval changes of abnormal lesions due to skeletal metastases, primary bone tumors, osteomyelitis, and fractures. In our initial preliminary results of the prospective study which was started on November 22, 2006 in our hospital, radiologists reported some interval changes which were not recognized in the initial standard readings, but were obvious when temporal subtraction images were viewed. The usefulness of the temporal subtraction images will be investigated in terms of its clinical utility by the prospective clinical study. (orig.)

  5. Decolonizing Straight Temporality Through Genre Trouble in Edwidge Danticat's The Farming of Bones

    Directory of Open Access Journals (Sweden)

    Eliana de Souza Ávila

    2014-12-01

    Full Text Available http://dx.doi.org/10.5007/2175-8026.2014n67p21 Framing genre trouble (McKenzie 2006 as a decolonial methodology, this paper considers the relevance of Edwidge Danticat’s The Farming of Bones (1998 for reading migrant texts against the grain of straight temporality which sustains the coloniality of power (Lugones 2007. Scrutinizing historiographic suppression, Danticat’s migrant text interrupts the chrononormative portrayal of the Trujillo genocide of Haitian workers in the Dominican Republic as a reality pertaining to an obsolete past and to the geocultural margins alone. Read in the aftermath of the testimonio controversy, it may thus decenter the ongoing deflection of attention from Rigoberta Menchú’s impact on the geocultural structures that sanction ongoing military intervention and genocide by refocusing on historiography as a terrain of relentless decolonial contestation rather than prescriptive narrative closure.

  6. Chondrosarcoma of the temporal bone. Diagnosis and treatment of 13 cases and review of the literature

    International Nuclear Information System (INIS)

    Coltrera, M.D.; Googe, P.B.; Harrist, T.J.; Hyams, V.J.; Schiller, A.L.; Goodman, M.L.

    1986-01-01

    Chondrosarcoma of the temporal bone is a rare lesion. Clinically it has been confused with multiple sclerosis, glomus jugulare tumors, meningioma, and chordomas. The cranial nerve palsies frequently observed with the tumors are related to the anatomic locations of the tumors. Thirteen patients with this entity are presented and the eleven other cases in the literature are reviewed. Histologically the tumors are low grade and exhibit myxoid features. The myxoid features must be differentiated from chordoma and chondroid chordoma. The tumor locations preclude surgical excision and conventional radiation therapy can cause unacceptable neurologic sequelae. Proton beam therapy has been effective in short-term results and appears capable of avoiding serious neurologic side effects

  7. Temporal bone trauma: correlative study between CT findings and clinical manifestations

    International Nuclear Information System (INIS)

    Kim, Jung Hee; Kim, Hyung Jin; Kim, Jae Hyoung

    1994-01-01

    To assess how accurately computed tomography (CT) can demonstrate the abnormal findings which are believed to cause the clinical signs and symptoms of hearing loss (HL), vertigo and facial paralysis (FP) in patients with temporal bone trauma. The authors studied CT scans of 39 ears in 35 patients with temporal bone trauma. CT scans were performed with 1-1.5 mm slice thickness and table incrementation. Both axial and coronal scans were obtained in 32 patients and in three patients only axial scans were obtained. We analyzed CT with special reference to the structural abnormalities of the external auditory canal, middle ear cavity, bony labyrinth, and facial nerve canal, and correlated these findings with the actual clinical signs and symptoms. As to hearing loss, we evaluated 32 ears in which pure tone audiometry or brainstem evoked response audiometry had been performed. With respect to the specific types of HL, CT accurately showed the abnormalities in 84% (16/19) in conductive HL, 100% (2/2) in sensorineural HL, and 25% (2/8) for mixed HL. When we categorized HL simply as conductive and sensorineural, assuming that mixed be the result of combined conductive and sensorineural HL, CT demonstrated the abnormalities in 89% (24/27) for conductive HL and 50% (5/10) for sensorineural HL. Concerning vertigo and FP, CT demonstrated abnormalities in 67%(4/6), and 29% (4/14), respectively. Except for conductive HL, CT seems to have a variable degree of limitation for the demonstration of the structural abnormalities resulting sensorineural HL, vertigo or facial paralysis. It is imperative to correlate the CT findings with the signs and symptoms in those clinical settings

  8. Temporal bone trauma: correlative study between CT findings and clinical manifestations

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Jung Hee; Kim, Hyung Jin; Kim, Jae Hyoung [College of Medicine, Gyeongsang National University, Jinju (Korea, Republic of)

    1994-11-15

    To assess how accurately computed tomography (CT) can demonstrate the abnormal findings which are believed to cause the clinical signs and symptoms of hearing loss (HL), vertigo and facial paralysis (FP) in patients with temporal bone trauma. The authors studied CT scans of 39 ears in 35 patients with temporal bone trauma. CT scans were performed with 1-1.5 mm slice thickness and table incrementation. Both axial and coronal scans were obtained in 32 patients and in three patients only axial scans were obtained. We analyzed CT with special reference to the structural abnormalities of the external auditory canal, middle ear cavity, bony labyrinth, and facial nerve canal, and correlated these findings with the actual clinical signs and symptoms. As to hearing loss, we evaluated 32 ears in which pure tone audiometry or brainstem evoked response audiometry had been performed. With respect to the specific types of HL, CT accurately showed the abnormalities in 84% (16/19) in conductive HL, 100% (2/2) in sensorineural HL, and 25% (2/8) for mixed HL. When we categorized HL simply as conductive and sensorineural, assuming that mixed be the result of combined conductive and sensorineural HL, CT demonstrated the abnormalities in 89% (24/27) for conductive HL and 50% (5/10) for sensorineural HL. Concerning vertigo and FP, CT demonstrated abnormalities in 67%(4/6), and 29% (4/14), respectively. Except for conductive HL, CT seems to have a variable degree of limitation for the demonstration of the structural abnormalities resulting sensorineural HL, vertigo or facial paralysis. It is imperative to correlate the CT findings with the signs and symptoms in those clinical settings.

  9. Differentiating levels of surgical experience on a virtual reality temporal bone simulator.

    Science.gov (United States)

    Zhao, Yi C; Kennedy, Gregor; Hall, Richard; O'Leary, Stephen

    2010-11-01

    Virtual reality simulation is increasingly being incorporated into surgical training and may have a role in temporal bone surgical education. Here we test whether metrics generated by a virtual reality surgical simulation can differentiate between three levels of experience, namely novices, otolaryngology residents, and experienced qualified surgeons. Cohort study. Royal Victorian Eye and Ear Hospital. Twenty-seven participants were recruited. There were 12 experts, six residents, and nine novices. After orientation, participants were asked to perform a modified radical mastoidectomy on the simulator. Comparisons of time taken, injury to structures, and forces exerted were made between the groups to determine which specific metrics would discriminate experience levels. Experts completed the simulated task in significantly shorter time than the other two groups (experts 22 minutes, residents 36 minutes, and novices 46 minutes; P = 0.001). Novices exerted significantly higher average forces when dissecting close to vital structures compared with experts (0.24 Newton [N] vs 0.13 N, P = 0.002). Novices were also more likely to injure structures such as dura compared to experts (23 injuries vs 3 injuries, P = 0.001). Compared with residents, the experts modulated their force between initial cortex dissection and dissection close to vital structures. Using the combination of these metrics, we were able to correctly classify the participants' level of experience 90 percent of the time. This preliminary study shows that measurements of performance obtained from within a virtual reality simulator can differentiate between levels of users' experience. These results suggest that simulator training may have a role in temporal bone training beyond foundational training. Copyright © 2010 American Academy of Otolaryngology–Head and Neck Surgery Foundation. Published by Mosby, Inc. All rights reserved.

  10. 3D-CT of the temporal bone area with high-speed processing

    International Nuclear Information System (INIS)

    Hattori, Taku

    1994-01-01

    Three-dimentional (3D)-CT was introduced to represent abnormal findings in the temporal bone area utilizing a SOMATOM DRH CT scanner with accessory 3D reconstruction software and an exclusive high-speed 3D processing system, VOXEL FLINGER. In a patient with eosinophilic granuloma, a defect in the squamous part of the temporal bone was demonstrated suggesting exposure of the dura mater during surgery. In a patient with a normal ear, well-developed mastoid cavity, a part of the handle and the head of the malleus, the incudomalleal joint, the short limb, body and a part of the long limb of the incus and the round window niche were demonstrated. In a case of chronic otitis media, poorly developed mastoid cavity and a possible defect of the tip of the long limb of the incus were demonstrated, in contrast to the patient with the normal ear. 3D-CT yields objective and solid images which are useful for diagnosis, treatment planning and explanation of the pathology to patients and their family. To obtain convincing 3D images, physicians themselves have to choose exact rotation angles. It is not adequate to reconstruct original CT data using a CT computer with accessory 3D software whose processing capability is not good enough for this purpose. The conclusion is as follows: 1) it is necessary and effective to transfer original CT data into the memory of the exclusive high-speed 3D processing system and 2) process the data by the voxel memory method to establish a clinically valuable 3D-CT imaging system. (author)

  11. Ferrochelatase deficiency of the bone marrow in a syndrome of congenital microcytic anaemia with iron overload of the liver and hyperferraemia

    International Nuclear Information System (INIS)

    Stavem, P.; Hovig, T.; Rootwelt, K.; Emblem, R.; Romslo, I.

    1985-01-01

    By far the most common mechanisms for hypochromic anaemias are either iron deficiency with a limited production of haem or the thalassaemias with a limited production of peptide chains. Some extremely rare congenital hypochromic anaemias have also been reported, in which iron deficiency or thalassaemia is not the cause. One of them is atransferrinaemia. In another rare type of hereditary, congenital hypochromic anaemia, the patients have hyperferraemia with a near fully saturated total iron binding capacity. In spite of heavy haemosiderin deposits in the liver, the bone marrow haemosiderin is reduced. In our studies which where reported in 1983, we found normal transferrin, Hb electrophoresis was normal, and there were no findings indicating thalassaemia minor or lead intoxication. We suggested that the most likely explanation of the condition was a defect in the iron transport mechanism from transferrin into the erythroid cells in the bone marrow, but at that time we had no method for studying this. During the last few years, more reliable methods have become available for assaying ferrochelatase, the enzyme largely responsible for the incorporation of iron into haem. We have therefore repeated our previous studies (with essentially the same results as reported in 1973), and have also assayed ferrochelatase activity of the bone marrow. (author)

  12. Primary giant myxoma of the temporal bone with major intracranial extension: presenting with hearing impairment and ear polyp

    Directory of Open Access Journals (Sweden)

    Satyarthee Guru Dutta

    2016-12-01

    Full Text Available Myxomas are mesenchymal origin, benign tumor, constituting approximately half of the benign cardiac tumors. Occasionally, it may also occurs at other locations, though the intracranial location of a myxoma is considered exceptionally rare. Only isolated few cases of intracranial myxoma are reported in the literature, almost all were locally confined within the originating bone. The extensive Pubmed and Medline search yielded only eight cases of primary myxoma arising in the temporal bone with extension into intracranial compartment. However intracranial extension is limited as early detection, however, Osterdock et al reported a case also arising from temporal bone with extensive intracranial extension. Author report an interesting case of intracranial myxoma in 27- year- old- male, involving the temporal bone associated with extensive bony erosion and also extending into infratemporal fossa, mastoid, and frontoparietal region and a polypoidal mass protruding into external ear. To the best of knowledge of authors, temporal myxoma presenting with external ear polypoidal mass, which underwent successful surgical excision is not reported and represent first case in the world literature.

  13. Analysis of Vibrant Soundbridge placement against the round window membrane in a human cadaveric temporal bone model.

    NARCIS (Netherlands)

    Pennings, R.J.E.; Ho, A.; Brown, J.; Wijhe, R.G. van; Bance, M.

    2010-01-01

    OBJECTIVE: To evaluate optimal placement of the Floating Mass Transducer of the Vibrant Soundbridge (Med-El, Innsbruck, Austria) against the round window membrane, particularly the impact of interposed coupling fascia and of covering materials. METHOD: : Six fresh human cadaveric temporal bones were

  14. Dual-time-point FDG-PET/CT Imaging of Temporal Bone Chondroblastoma: A Report of Two Cases

    Directory of Open Access Journals (Sweden)

    Akira Toriihara

    2015-07-01

    Full Text Available Temporal bone chondroblastoma is an extremely rare benign bone tumor. We encountered two cases showing similar imaging findings on computed tomography (CT, magnetic resonance imaging (MRI, and dual-time-point 18F-fluorodeoxyglucose (18F-FDG positron emission tomography (PET/CT. In both cases, CT images revealed temporal bone defects and sclerotic changes around the tumor. Most parts of the tumor showed low signal intensity on T2- weighted MRI images and non-uniform enhancement on gadolinium contrast-enhanced T1-weighted images. No increase in signal intensity was noted in diffusion-weighted images. Dual-time-point PET/CT showed markedly elevated 18F-FDG uptake, which increased from the early to delayed phase. Nevertheless, immunohistochemical analysis of the resected tumor tissue revealed weak expression of glucose transporter-1 and hexokinase II in both tumors. Temporal bone tumors, showing markedly elevated 18F-FDG uptake, which increases from the early to delayed phase on PET/CT images, may be diagnosed as malignant bone tumors. Therefore, the differential diagnosis should include chondroblastoma in combination with its characteristic findings on CT and MRI.

  15. Variation and diversity in Homo erectus: a 3D geometric morphometric analysis of the temporal bone.

    Science.gov (United States)

    Terhune, Claire E; Kimbel, William H; Lockwood, Charles A

    2007-07-01

    Although the level of taxonomic diversity within the fossil hominin species Homo erectus (sensu lato) is continually debated, there have been relatively few studies aiming to quantify the morphology of this species. Instead, most researchers have relied on qualitative descriptions or the evaluation of nonmetric characters, which in many cases display continuous variation. Also, only a few studies have used quantitative data to formally test hypotheses regarding the taxonomic composition of the "erectus" hypodigm. Despite these previous analyses, however, and perhaps in part due to these varied approaches for assessing variation within specimens typically referred to H. erectus (sensu lato) and the general lack of rigorous statistical testing of how variation within this taxon is partitioned, there is currently little consensus regarding whether this group is a single species, or whether it should instead be split into separate temporal or geographically delimited taxa. In order to evaluate possible explanations for variation within H. erectus, we tested the general hypothesis that variation within the temporal bone morphology of H. erectus is consistent with that of a single species, using great apes and humans as comparative taxa. Eighteen three-dimensional (3D) landmarks of the temporal bone were digitized on a total of 520 extant and fossil hominid crania. Landmarks were registered by Generalized Procrustes Analysis, and Procrustes distances were calculated for comparisons of individuals within and between the extant taxa. Distances between fossil specimens and between a priori groupings of fossils were then compared to the distances calculated within the extant taxa to assess the variation within the H. erectus sample relative to that of known species, subspecies, and populations. Results of these analyses indicate that shape variation within the entire H. erectus sample is generally higher than extant hominid intraspecific variation, and putative H. ergaster

  16. CT and MRI findings of temporal bone anomaly in patients with tinnitus

    Energy Technology Data Exchange (ETDEWEB)

    Bing, Wang; Junfang, Xian; Zhenchang, Wang; Zhaohui, Liu [Department of Radiology, Beijing Tongren Hospital, Capital Medical University (China)

    2011-03-15

    Objective: To study high resolution CT (HRCT) and MRI findings of temporal hone anomaly in patients with tinnitus and identify the optimal examination method in the detection of the anomaly. Methods: The HRCT and MRI data were analyzed retrospectively in 1015 patients including 145 patients with pulsatile tinnitus (PT) and 870 patients with nonpulsatile tinnitus (NPT). The positive rates of HRCT and MRI in the identification of temporal bone anomaly were analyzed and the efficiency of various examination methods was compared in revealing the anomaly. Data were tested by Chi-square test analysis. Results: Among 1015 patients, anomaly was seen in 767 cases (75.57%). High jugular bulb was found in 414 patients, accounting for 40.79%. Sigmoid sinus anomaly was detected in 387 patients (38.13%), while otitis media was found in 148 cases (14.58%), and low middle cranial fossa in 70 cases (6.90%). The positive rate of HRCT in the detection of high jugular bulb was 54.89% (365/665), which was significantly higher than those of other methods (P<0.05). The positive rate of enhanced HRCT in showing sigmoid sinus anomaly was 73.68% (56/76), which was significantly higher than those of other methods (P<0.05). Sigmoid sinus anomaly was the most frequent finding in patients with PT, accounting for 66.21% (96/145). The incidence of sigmoid sinus anomaly was higher in PT than in NPT (291/870, 33.45%; χ{sup 2}=56.537, P<0.01). The fast imaging employing steady-state acquisition (FIESTA) sequence was the best examination method in displaying the vessel within the internal auditory canal (42/42,100%). Conclusions: High jugular bulb and sigmoid sinus anomaly were the most frequent abnormal findings of temporal bone in patients with tinnitus. Enhanced HRCT was the choice of modality in patients with PT. Plain HRCT was recommended for NPT. FIESTA sequence was the best in the evaluation of the vessel within the internal auditory canal. (authors)

  17. 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%)

  18. Microsurgical and Endoscopic Anatomy for Intradural Temporal Bone Drilling and Applications of the Electromagnetic Navigation System: Various Extensions of the Retrosigmoid Approach.

    Science.gov (United States)

    Matsushima, Ken; Komune, Noritaka; Matsuo, Satoshi; Kohno, Michihiro

    2017-07-01

    The use of the retrosigmoid approach has recently been expanded by several modifications, including the suprameatal, transmeatal, suprajugular, and inframeatal extensions. Intradural temporal bone drilling without damaging vital structures inside or beside the bone, such as the internal carotid artery and jugular bulb, is a key step for these extensions. This study aimed to examine the microsurgical and endoscopic anatomy of the extensions of the retrosigmoid approach and to evaluate the clinical feasibility of an electromagnetic navigation system during intradural temporal bone drilling. Five temporal bones and 8 cadaveric cerebellopontine angles were examined to clarify the anatomy of retrosigmoid intradural temporal bone drilling. Twenty additional cerebellopontine angles were dissected in a clinical setting with an electromagnetic navigation system while measuring the target registration errors at 8 surgical landmarks on and inside the temporal bone. Retrosigmoid intradural temporal bone drilling expanded the surgical exposure to allow access to the petroclival and parasellar regions (suprameatal), internal acoustic meatus (transmeatal), upper jugular foramen (suprajugular), and petrous apex (inframeatal). The electromagnetic navigation continuously guided the drilling without line of sight limitation, and its small devices were easily manipulated in the deep and narrow surgical field in the posterior fossa. Mean target registration error was less than 0.50 mm during these procedures. The combination of endoscopic and microsurgical techniques aids in achieving optimal exposure for retrosigmoid intradural temporal bone drilling. The electromagnetic navigation system had clear advantages with acceptable accuracy including the usability of small devices without line of sight limitation. Copyright © 2017 Elsevier Inc. All rights reserved.

  19. Identification of photoacoustic transients during pulsed laser ablation of the human temporal bone: an experimental model.

    Science.gov (United States)

    Wong, B J; Dickinson, M R; Berns, M W; Neev, J

    1996-12-01

    Laser ablation of hard tissues during neurotologic operations has been accomplished with continuous-wave (CW) lasers in the visible and midinfrared spectrum. The mechanism of ablation at these wavelengths is secondary to photothermal-induced tissue destruction. As a result, significant thermal damage to surrounding tissue may occur. Pulsed ultraviolet (UV) lasers have been suggested as an alternative to the argon, KTP-532, and CO2 lasers currently used in clinical practice. The pulse length of Excimer lasers are considerably shorter than the thermal diffusion time of bone tissue, and as a consequence thermal injury is minimal. This makes pulsed lasers an attractive tool for tissue ablation in the ear: in essence a "cold knife." However, the short pulse width of Excimer lasers (typically 10-150 ns) can create large thermoelastic stresses in the ablation specimen. This study identifies the presence of these photoacoustic waves during the Excimer laser treatment of the cadaveric human temporal bone. A XeCl (lambda = 308 nm, tau p = 12 ns) excimer laser was used to ablate hard tissue surrounding the oval window and facial ridge with energies of 75, 45, 25, and 12 mJ/pulse. Spot size was estimated to be 0.5 mm2. Custom high-frequency polyvinyldifluoride (PVDF) piezoelectric film transducers were fabricated and attached to the promontory, round window niche, and facial ridges. The signals were amplified using a low-noise preamplifier and recorded on a digitizing oscilloscope. Photoacoustic waves were clearly identified. Notably, large acoustic waves were measured on the promontory and on both sides of the facial ridge. The implications and clinical relevance of these findings is discussed and compared to findings obtained from a model system.

  20. Temporal variation and lack of host specificity among bacterial endosymbionts of Osedax bone worms (Polychaeta: Siboglinidae

    Directory of Open Access Journals (Sweden)

    Salathé Rahel M

    2012-09-01

    Full Text Available Abstract Background Osedax worms use a proliferative root system to extract nutrients from the bones of sunken vertebrate carcasses. The roots contain bacterial endosymbionts that contribute to the nutrition of these mouthless and gutless worms. The worms acquire these essential endosymbionts locally from the environment in which their larvae settle. Here we report on the temporal dynamics of endosymbiont diversity hosted by nine Osedax species sampled during a three-year investigation of an experimental whale fall at 1820-m depth in the Monterey Bay, California. The host species were identified by their unique mitochondrial COI haplotypes. The endosymbionts were identified by ribotyping with PCR primers specifically designed to target Oceanospirillales. Results Thirty-two endosymbiont ribotypes associated with these worms clustered into two distinct bacterial ribospecies that together comprise a monophyletic group, mostly restricted to deep waters (>1000 m. Statistical analyses confirmed significant changes in the relative abundances of host species and the two dominant endosymbiont ribospecies during the three-year sampling period. Bone type (whale vs. cow also had a significant effect on host species, but not on the two dominant symbiont ribospecies. No statistically significant association existed between the host species and endosymbiont ribospecies. Conclusions Standard PCR and direct sequencing proved to be an efficient method for ribotyping the numerically dominant endosymbiont strains infecting a large sample of host individuals; however, this method did not adequately represent the frequency of mixed infections, which appears to be the rule rather than an exception for Osedax individuals. Through cloning and the use of experimental dilution series, we determined that minority ribotypes constituting less than 30% of a mixture would not likely be detected, leading to underestimates of the frequency of multiple infections in host

  1. Temporal variation and lack of host specificity among bacterial endosymbionts of Osedax bone worms (Polychaeta: Siboglinidae)

    Science.gov (United States)

    2012-01-01

    Background Osedax worms use a proliferative root system to extract nutrients from the bones of sunken vertebrate carcasses. The roots contain bacterial endosymbionts that contribute to the nutrition of these mouthless and gutless worms. The worms acquire these essential endosymbionts locally from the environment in which their larvae settle. Here we report on the temporal dynamics of endosymbiont diversity hosted by nine Osedax species sampled during a three-year investigation of an experimental whale fall at 1820-m depth in the Monterey Bay, California. The host species were identified by their unique mitochondrial COI haplotypes. The endosymbionts were identified by ribotyping with PCR primers specifically designed to target Oceanospirillales. Results Thirty-two endosymbiont ribotypes associated with these worms clustered into two distinct bacterial ribospecies that together comprise a monophyletic group, mostly restricted to deep waters (>1000 m). Statistical analyses confirmed significant changes in the relative abundances of host species and the two dominant endosymbiont ribospecies during the three-year sampling period. Bone type (whale vs. cow) also had a significant effect on host species, but not on the two dominant symbiont ribospecies. No statistically significant association existed between the host species and endosymbiont ribospecies. Conclusions Standard PCR and direct sequencing proved to be an efficient method for ribotyping the numerically dominant endosymbiont strains infecting a large sample of host individuals; however, this method did not adequately represent the frequency of mixed infections, which appears to be the rule rather than an exception for Osedax individuals. Through cloning and the use of experimental dilution series, we determined that minority ribotypes constituting less than 30% of a mixture would not likely be detected, leading to underestimates of the frequency of multiple infections in host individuals. PMID:23006795

  2. Spectrum of temporal bone abnormalities in patients with Waardenburg syndrome and SOX10 mutations.

    Science.gov (United States)

    Elmaleh-Bergès, M; Baumann, C; Noël-Pétroff, N; Sekkal, A; Couloigner, V; Devriendt, K; Wilson, M; Marlin, S; Sebag, G; Pingault, V

    2013-01-01

    Waardenburg syndrome, characterized by deafness and pigmentation abnormalities, is clinically and genetically heterogeneous, consisting of 4 distinct subtypes and involving several genes. SOX10 mutations have been found both in types 2 and 4 Waardenburg syndrome and neurologic variants. The purpose of this study was to evaluate both the full spectrum and relative frequencies of inner ear malformations in these patients. Fifteen patients with Waardenburg syndrome and different SOX10 mutations were studied retrospectively. Imaging was performed between February 2000 and March 2010 for cochlear implant work-up, diagnosis of hearing loss, and/or evaluation of neurologic impairment. Eleven patients had both CT and MR imaging examinations, 3 had MR imaging only, and 1 had CT only. Temporal bone abnormalities were bilateral. The most frequent pattern associated agenesis or hypoplasia of ≥1 semicircular canal, an enlarged vestibule, and a cochlea with a reduced size and occasionally an abnormal shape, but with normal partition in the 13/15 cases that could be analyzed. Three patients lacked a cochlear nerve, bilaterally in 2 patients. In addition, associated abnormalities were found when adequate MR imaging sequences were available: agenesis of the olfactory bulbs (7/8), hypoplastic or absent lacrimal glands (11/14), hypoplastic parotid glands (12/14), and white matter signal anomalies (7/13). In the appropriate clinical context, bilateral agenesis or hypoplasia of the semicircular canals or both, associated with an enlarged vestibule and a cochlear deformity, strongly suggests a diagnosis of Waardenburg syndrome linked to a SOX10 mutation.

  3. The new mid-scala electrode array: a radiologic and histologic study in human temporal bones.

    Science.gov (United States)

    Hassepass, Frederike; Bulla, Stefan; Maier, Wolfgang; Laszig, Roland; Arndt, Susan; Beck, Rainer; Traser, Lousia; Aschendorff, Antje

    2014-09-01

    To analyze the quality of insertion of the newly developed midscala (MS) electrode, which targets a midscalar electrode position to reduce the risk of trauma to the lateral wall and the modiolus. Modern cochlear implant surgery aims for a safe intracochlear placement of electrode arrays with an ongoing debate regarding cochleostomy or round window (RW) insertion and the use of lateral wall or perimodiolar electrode placement. Intracochlear trauma after insertion of different electrodes depends on insertion mode and electrode design and may result in trauma to the delicate structures of the cochlear. We performed a temporal bone (TB) trial with insertion of the MS electrode in n = 20 TB's after a mastoidectomy and posterior tympanotomy. Insertion was performed either via the RW or a cochleostomy. Electrode positioning, length of insertion, and angle of insertion were analyzed with rotational tomography (RT). TBs were histologically analyzed. Results of RT and histology were compared. Scala tympani (ST) insertion could be accomplished reliably by both RW and via a cochleostomy approach. In 20 TBs, 1 scala vestibuli insertion, 1 incomplete (ST), and 1 elevation of basilar membrane were depicted. No trauma was found in 94.7% of all ST insertions. RT allowed determination of the intracochlear electrode position, which was specified by histologic sectioning. The new MS electrode seems to fulfill reliable atraumatic intracochlear placement via RW and cochleostomy approaches. RT is available for evaluation of intracochlear electrode position, serving as a potential quality control instrument in human implantation.

  4. Usefulness of temporal bone prototype for drilling training: A prospective study.

    Science.gov (United States)

    Aussedat, C; Venail, F; Nguyen, Y; Lescanne, E; Marx, M; Bakhos, D

    2017-12-01

    Dissection of cadaveric temporal bones (TBs) is considered the gold standard for surgical training in otology. For many reasons, access to the anatomical laboratory and cadaveric TBs is difficult for some facilities. The aim of this prospective and comparative study was to evaluate the usefulness of a physical TB prototype for drilling training in residency. Prospective study. Tertiary referral centre. Thirty-four residents were included. Seventeen residents (mean age 26.7±1.6) drilled on only cadaveric TBs ("traditional" group), in the traditional training method, while seventeen residents (mean age 26.5±1.7) drilled first on a prototype and then on a cadaveric TB ("prototype" group). Drilling performance was assessed using a validated scale. Residents completed a mastoid image before and after each drilling to enable evaluation of mental representations of the mastoidectomy. No differences were observed between the groups with respect to age, drilling experience and level of residency. Regarding drilling performance, we found a significant difference across the groups, with a better score in the prototype group (P=.0007). For mental representation, the score was statistically improved (P=.0003) after drilling in both groups, suggesting that TB drilling improves the mental representation of the mastoidectomy whether prototype or cadaveric TB is used. The TB prototype improves the drilling performance and mental representation of the mastoidectomy in the young resident population. A drilling simulation with virtual or physical systems seems to be a beneficial tool to improve TB drilling. © 2017 John Wiley & Sons Ltd.

  5. Operative findings of conductive hearing loss with intact tympanic membrane and normal temporal bone computed tomography.

    Science.gov (United States)

    Kim, Se-Hyung; Cho, Yang-Sun; Kim, Hye Jeong; Kim, Hyung-Jin

    2014-06-01

    Despite recent technological advances in diagnostic methods including imaging technology, it is often difficult to establish a preoperative diagnosis of conductive hearing loss (CHL) in patients with an intact tympanic membrane (TM). Especially, in patients with a normal temporal bone computed tomography (TBCT), preoperative diagnosis is more difficult. We investigated middle ear disorders encountered in patients with CHL involving an intact TM and normal TBCT. We also analyzed the surgical results with special reference to the pathology. We reviewed the medical records of 365 patients with intact TM, who underwent exploratory tympanotomy for CHL. Fifty nine patients (67 ears, eight bilateral surgeries) had a normal preoperative TBCT findings reported by neuro-radiologists. Demographic data, otologic history, TM findings, preoperative imaging findings, intraoperative findings, and pre- and postoperative audiologic data were obtained and analyzed. Exploration was performed most frequently in the second and fifth decades. The most common postoperative diagnosis was stapedial fixation with non-progressive hearing loss. The most commonly performed hearing-restoring procedure was stapedotomy with piston wire prosthesis insertion. Various types of hearing-restoring procedures during exploration resulted in effective hearing improvement, especially with better outcome in the ossicular chain fixation group. In patients with CHL who have intact TM and normal TBCT, we should consider an exploratory tympanotomy for exact diagnosis and hearing improvement. Information of the common operative findings from this study may help in preoperative counseling.

  6. Stria vascularis and cochlear hair cell changes in syphilis: A human temporal bone study.

    Science.gov (United States)

    Hızlı, Ömer; Kaya, Serdar; Hızlı, Pelin; Paparella, Michael M; Cureoglu, Sebahattin

    2016-12-01

    To observe any changes in stria vascularis and cochlear hair cells in patients with syphilis. We examined 13 human temporal bone samples from 8 patients with syphilis (our syphilis group), as well as 12 histopathologically normal samples from 9 age-matched patients without syphilis (our control group). We compared, between the two groups, the mean area of the stria vascularis (measured with conventional light microscopy connected to a personal computer) and the mean percentage of cochlear hair cell loss (obtained from cytocochleograms). In our syphilis group, only 1 (7.7%) of the 13 samples had precipitate in the endolymphatic or perilymphatic spaces; 8 (61.5%) of the samples revealed the presence of endolymphatic hydrops (4 cochlear, 4 saccular). The mean area of the stria vascularis did not significantly differ, in any turn of the cochlea, between the 2 groups (P>0.1). However, we did find significant differences between the 2 groups in the mean percentage of outer hair cells in the apical turn (Psyphilis group, we observed either complete loss of the organ of Corti or a flattened organ of Corti without any cells in addition to the absence of both outer and inner hair cells. In this study, syphilis led either to complete loss of the organ of Corti or to significant loss of cochlear hair cells, in addition to cochleosaccular hydrops. But the area of the stria vascularis did not change. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  7. Differential Intracochlear Sound Pressure Measurements in Human Temporal Bones with an Off-the-Shelf Sensor

    Directory of Open Access Journals (Sweden)

    Martin Grossöhmichen

    2016-01-01

    Full Text Available The standard method to determine the output level of acoustic and mechanical stimulation to the inner ear is measurement of vibration response of the stapes in human cadaveric temporal bones (TBs by laser Doppler vibrometry. However, this method is reliable only if the intact ossicular chain is stimulated. For other stimulation modes an alternative method is needed. The differential intracochlear sound pressure between scala vestibuli (SV and scala tympani (ST is assumed to correlate with excitation. Using a custom-made pressure sensor it has been successfully measured and used to determine the output level of acoustic and mechanical stimulation. To make this method generally accessible, an off-the-shelf pressure sensor (Samba Preclin 420 LP, Samba Sensors was tested here for intracochlear sound pressure measurements. During acoustic stimulation, intracochlear sound pressures were simultaneously measurable in SV and ST between 0.1 and 8 kHz with sufficient signal-to-noise ratios with this sensor. The pressure differences were comparable to results obtained with custom-made sensors. Our results demonstrated that the pressure sensor Samba Preclin 420 LP is usable for measurements of intracochlear sound pressures in SV and ST and for the determination of differential intracochlear sound pressures.

  8. High-fidelity haptic and visual rendering for patient-specific simulation of temporal bone surgery.

    Science.gov (United States)

    Chan, Sonny; Li, Peter; Locketz, Garrett; Salisbury, Kenneth; Blevins, Nikolas H

    2016-12-01

    Medical imaging techniques provide a wealth of information for surgical preparation, but it is still often the case that surgeons are examining three-dimensional pre-operative image data as a series of two-dimensional images. With recent advances in visual computing and interactive technologies, there is much opportunity to provide surgeons an ability to actively manipulate and interpret digital image data in a surgically meaningful way. This article describes the design and initial evaluation of a virtual surgical environment that supports patient-specific simulation of temporal bone surgery using pre-operative medical image data. Computational methods are presented that enable six degree-of-freedom haptic feedback during manipulation, and that simulate virtual dissection according to the mechanical principles of orthogonal cutting and abrasive wear. A highly efficient direct volume renderer simultaneously provides high-fidelity visual feedback during surgical manipulation of the virtual anatomy. The resulting virtual surgical environment was assessed by evaluating its ability to replicate findings in the operating room, using pre-operative imaging of the same patient. Correspondences between surgical exposure, anatomical features, and the locations of pathology were readily observed when comparing intra-operative video with the simulation, indicating the predictive ability of the virtual surgical environment.

  9. Application of digital tomosynthesis to radiographic diagnosis of the temporal bone. Studies on visualization in normal subjects

    International Nuclear Information System (INIS)

    Kawai, Takashi

    1995-01-01

    To examine the usefulness of digital tomosynthesis for conducting radiographic diagnosis of the temporal bone, visualization of various aural structures such as the semicircular canals, cochlea, vestibular apparatus, ossicles of the ear and facial nerve canal was examined in 18 volunteers. The visualization of temporal bone specimens by digital tomosynthesis and CT images (slice thickness: 1.5 mm) was compared. The results showed that this system (Digital Tomosynthesis) produced clear images of bony labyrinthine structures such as the semicircular canals, cochlea, and vestibular apparatus. Visualization of the ossicles was also clear, and their continuity could be comprehended better than on CT images. This system also provided good visualization of the labyrinthine and tympanic parts of the facial nerve canal, although CT images had greater sharpness. Visualization of the lower half of the mastoid part was poor with this system. (author)

  10. Temporal mechanically-induced signaling events in bone and dorsal root ganglion neurons after in vivo bone loading.

    Directory of Open Access Journals (Sweden)

    Jason A Bleedorn

    Full Text Available Mechanical signals play an integral role in the regulation of bone mass and functional adaptation to bone loading. The osteocyte has long been considered the principle mechanosensory cell type in bone, although recent evidence suggests the sensory nervous system may play a role in mechanosensing. The specific signaling pathways responsible for functional adaptation of the skeleton through modeling and remodeling are not clearly defined. In vitro studies suggest involvement of intracellular signaling through mitogen-activated protein kinase (MAPK, phosphatidylinositol 3-kinase (PI3K/protein kinase B (Akt, and mammalian target of rapamycin (mTOR. However, anabolic signaling responses to bone loading using a whole animal in vivo model have not been studied in detail. Therefore, we examined mechanically-induced signaling events at five time points from 0 to 24 hours after loading using the rat in vivo ulna end-loading model. Western blot analysis of bone for MAPK's, PI3K/Akt, and mTOR signaling, and quantitative reverse transcription polymerase chain reaction (qRT-PCR to estimate gene expression of calcitonin gene-related protein alpha (CGRP-α, brain-derived neurotrophic factor (BDNF, nerve growth factor (NGF, c-jun, and c-fos in dorsal root ganglion (DRG of the brachial intumescence were performed. There was a significant increase in signaling through MAPK's including extracellular signal-related kinase (ERK and c-Jun N-terminal kinase (JNK in loaded limbs at 15 minutes after mechanical loading. Ulna loading did not significantly influence expression of the genes of interest in DRG neurons. Bone signaling and DRG gene expression from the loaded and contralateral limbs was correlated (SR>0.40, P<0.05. However, bone signaling did not correlate with expression of the genes of interest in DRG neurons. These results suggest that signaling through the MAPK pathway may be involved in load-induced bone formation in vivo. Further characterization of the

  11. Improving temporal bone dissection using self-directed virtual reality simulation: results of a randomized blinded control trial.

    Science.gov (United States)

    Zhao, Yi Chen; Kennedy, Gregor; Yukawa, Kumiko; Pyman, Brian; O'Leary, Stephen

    2011-03-01

    A significant benefit of virtual reality (VR) simulation is the ability to provide self-direct learning for trainees. This study aims to determine whether there are any differences in performance of cadaver temporal bone dissections between novices who received traditional teaching methods and those who received unsupervised self-directed learning in a VR temporal bone simulator. Randomized blinded control trial. Royal Victorian Eye and Ear Hospital. Twenty novice trainees. After receiving an hour lecture, participants were randomized into 2 groups to receive an additional 2 hours of training via traditional teaching methods or self-directed learning using a VR simulator with automated guidance. The simulation environment presented participants with structured training tasks, which were accompanied by real-time computer-generated feedback as well as real operative videos and photos. After the training, trainees were asked to perform a cortical mastoidectomy on a cadaveric temporal bone. The dissection was videotaped and assessed by 3 otologists blinded to participants' teaching group. The overall performance scores of the simulator-based training group were significantly higher than those of the traditional training group (67% vs 29%; P < .001), with an intraclass correlation coefficient of 0.93, indicating excellent interrater reliability. Using other assessments of performance, such as injury size, the VR simulator-based training group also performed better than the traditional group. This study indicates that self-directed learning on VR simulators can be used to improve performance on cadaver dissection in novice trainees compared with traditional teaching methods alone.

  12. New total ossicular replacement prostheses with a resilient joint: experimental data from human temporal bones.

    Science.gov (United States)

    Arechvo, Irina; Bornitz, Matthias; Lasurashvili, Nikoloz; Zahnert, Thomas; Beleites, Thomas

    2012-01-01

    New flexible total ossicular prostheses with an integrated microjoint can compensate for large static displacements in the reconstructed ossicular chain. When properly designed, they can mimic the function of the joints of the intact chain and ensure good vibration transfer in both straight and bent conditions. Prosthesis dislocations and extrusions are frequently observed after middle ear surgery. They are mainly related to the altered distance between the coupling points because of large static eardrum displacements. The new prostheses consist of 2 titanium shafts, which are incorporated into a silicone body. The sound transfer function and stapes footplate displacement at static loads were evaluated in human temporal bones after ossicular reconstruction using prostheses with 2 different silicones with different hardness values. The stiffness and bending characteristics of the prostheses were investigated with a quasi-static load. The sound transfer properties of the middle ears with the prostheses inserted under uncompressed conditions were comparable with those of ears with intact ossicular chains. The implant with the soft silicone had improved acoustic transfer characteristics over the implant with the hard silicone in a compressed state. In the quasi-static experiments, the minimum medial footplate displacement was found with the same implant. The bending characteristics depended on the silicone stiffness and correlated closely with the point and angle of the load incidence. The titanium prostheses with a resilient joint that were investigated in this study had good sound transfer characteristics under optimal conditions as well as in a compressed state. As a result of joint bending, the implants compensate for the small changes in length of the ossicular chain that occur under varying middle ear pressure. The implants require a stable support at the stapes footplate to function properly.

  13. Spatial and temporal changes of subchondral bone proceed to articular cartilage degeneration in rats subjected to knee immobilization.

    Science.gov (United States)

    Xu, Lei; Li, Zhe; Lei, Lei; Zhou, Yue-Zhu; Deng, Song-Yun; He, Yong-Bin; Ni, Guo-Xin

    2016-03-01

    This study was aimed to investigate the spatial and temporal changes of subchondral bone and its overlying articular cartilage in rats following knee immobilization. A total of 36 male Wistar rats (11-13 months old) were assigned randomly and evenly into 3 groups. For each group, knee joints in 6 rats were immobilized unilaterally for 1, 4, or 8 weeks, respectively, while the remaining rats were allowed free activity and served as external control groups. For each animal, femurs at both sides were dissected after sacrificed. The distal part of femur was examined by micro-CT. Subsequently, femoral condyles were collected for further histological observation and analysis. For articular cartilage, significant changes were observed only at 4 and 8 weeks of immobilization. The thickness of articular cartilage and chondrocytes numbers decreased with time. However, significant changes in subchondral bone were defined by micro-CT following immobilization in a time-dependent manner. Immobilization led to a thinner and more porous subchondral bone plate, as well as a reduction in trabecular thickness and separation with a more rod-like architecture. Changes in subchondral bone occurred earlier than in articular cartilage. More importantly, immobilization-induced changes in subchondral bone may contribute, at least partially, to changes in its overlying articular cartilage. © 2016 Wiley Periodicals, Inc.

  14. High temporal versus high spatial resolution in MR quantitative pulmonary perfusion imaging of two-year old children after congenital diaphragmatic hernia repair

    Energy Technology Data Exchange (ETDEWEB)

    Weidner, M.; Hagelstein, C.; Schoenberg, S.O.; Neff, K.W. [University Medical Center Mannheim, Medical Faculty Mannheim, Heidelberg University, Institute of Clinical Radiology and Nuclear Medicine, Mannheim (Germany); Zoellner, F.G.; Schad, L.R. [Heidelberg University, Computer Assisted Clinical Medicine, Medical Faculty Mannheim, Mannheim (Germany); Zahn, K. [University Medical Center Mannheim, Medical Faculty Mannheim, University of Heidelberg, Department of Pediatric Surgery, Mannheim (Germany); Schaible, T. [University Medical Center Mannheim, Medical Faculty Mannheim, Heidelberg University, Department of Pediatrics, Mannheim (Germany)

    2014-10-15

    Congenital diaphragmatic hernia (CDH) leads to lung hypoplasia. Using dynamic contrast-enhanced (DCE) MR imaging, lung perfusion can be quantified. As MR perfusion values depend on temporal resolution, we compared two protocols to investigate whether ipsilateral lung perfusion is impaired after CDH, whether there are protocol-dependent differences, and which protocol is preferred. DCE-MRI was performed in 36 2-year old children after CDH on a 3 T MRI system; protocol A (n = 18) based on a high spatial (3.0 s; voxel: 1.25 mm{sup 3}) and protocol B (n = 18) on a high temporal resolution (1.5 s; voxel: 2 mm{sup 3}). Pulmonary blood flow (PBF), pulmonary blood volume (PBV), mean transit time (MTT), and peak-contrast-to-noise-ratio (PCNR) were quantified. PBF was reduced ipsilaterally, with ipsilateral PBF of 45 ± 26 ml/100 ml/min to contralateral PBF of 63 ± 28 ml/100 ml/min (p = 0.0016) for protocol A; and for protocol B, side differences were equivalent (ipsilateral PBF = 62 ± 24 vs. contralateral PBF = 85 ± 30 ml/100 ml/min; p = 0.0034). PCNR was higher for protocol B (30 ± 18 vs. 20 ± 9; p = 0.0294). Protocol B showed higher values of PBF in comparison to protocol A (p always <0.05). Ipsilateral lung perfusion is reduced in 2-year old children following CDH repair. Higher temporal resolution and increased voxel size show a gain in PCNR and lead to higher perfusion values. Protocol B is therefore preferred. (orig.)

  15. Mutations in Subunits of the Activating Signal Cointegrator 1 Complex Are Associated with Prenatal Spinal Muscular Atrophy and Congenital Bone Fractures

    Science.gov (United States)

    Knierim, Ellen; Hirata, Hiromi; Wolf, Nicole I.; Morales-Gonzalez, Susanne; Schottmann, Gudrun; Tanaka, Yu; Rudnik-Schöneborn, Sabine; Orgeur, Mickael; Zerres, Klaus; Vogt, Stefanie; van Riesen, Anne; Gill, Esther; Seifert, Franziska; Zwirner, Angelika; Kirschner, Janbernd; Goebel, Hans Hilmar; Hübner, Christoph; Stricker, Sigmar; Meierhofer, David; Stenzel, Werner; Schuelke, Markus

    2016-01-01

    Transcriptional signal cointegrators associate with transcription factors or nuclear receptors and coregulate tissue-specific gene transcription. We report on recessive loss-of-function mutations in two genes (TRIP4 and ASCC1) that encode subunits of the nuclear activating signal cointegrator 1 (ASC-1) complex. We used autozygosity mapping and whole-exome sequencing to search for pathogenic mutations in four families. Affected individuals presented with prenatal-onset spinal muscular atrophy (SMA), multiple congenital contractures (arthrogryposis multiplex congenita), respiratory distress, and congenital bone fractures. We identified homozygous and compound-heterozygous nonsense and frameshift TRIP4 and ASCC1 mutations that led to a truncation or the entire absence of the respective proteins and cosegregated with the disease phenotype. Trip4 and Ascc1 have identical expression patterns in 17.5-day-old mouse embryos with high expression levels in the spinal cord, brain, paraspinal ganglia, thyroid, and submandibular glands. Antisense morpholino-mediated knockdown of either trip4 or ascc1 in zebrafish disrupted the highly patterned and coordinated process of α-motoneuron outgrowth and formation of myotomes and neuromuscular junctions and led to a swimming defect in the larvae. Immunoprecipitation of the ASC-1 complex consistently copurified cysteine and glycine rich protein 1 (CSRP1), a transcriptional cofactor, which is known to be involved in spinal cord regeneration upon injury in adult zebrafish. ASCC1 mutant fibroblasts downregulated genes associated with neurogenesis, neuronal migration, and pathfinding (SERPINF1, DAB1, SEMA3D, SEMA3A), as well as with bone development (TNFRSF11B, RASSF2, STC1). Our findings indicate that the dysfunction of a transcriptional coactivator complex can result in a clinical syndrome affecting the neuromuscular system. PMID:26924529

  16. Threshold levels of 25-hydroxyvitamin D and parathyroid hormone for impaired bone health in children with congenital ichthyosis and type IV and V skin.

    Science.gov (United States)

    Sethuraman, G; Sreenivas, V; Yenamandra, V K; Gupta, N; Sharma, V K; Marwaha, R K; Bhari, N; Irshad, M; Kabra, M; Thulkar, S

    2015-01-01

    Patients with congenital ichthyosis, especially those with darker skin types, are at increased risk of developing vitamin D deficiency and rickets. The relationships between 25-hydroxyvitamin D [25(OH)D], parathyroid hormone (PTH) and bone health have not been studied previously, in ichthyosis. To determine the threshold levels of 25(OH)D and PTH for impaired bone health in children with congenital ichthyosis. In this cross-sectional study, 119 children with ichthyosis and 168 controls were recruited. Serum 25(OH)D, PTH, calcium, phosphate and alkaline phosphatase (ALP) were measured. Radiological screening for rickets was carried out only in children with ichthyosis. Forty-seven children with ichthyosis had either clinical or radiological evidence of rickets. The correlation between serum 25(OH)D and PTH showed that a serum level of 25(OH)D 8 ng mL(-1) was associated with a significant increase in PTH. The correlation between PTH and ALP showed that a serum PTH level of 75 pg mL(-1) was associated with a significant increase in ALP levels. Of the different clinical phenotypes of ichthyosis, both autosomal recessive congenital ichthyosis (ARCI) and epidermolytic ichthyosis (EI) were found to have significantly increased PTH, ALP and radiological rickets scores compared with common ichthyosis. Serum levels of 25(OH)D ≤ 8 ng mL(-1) and PTH ≥ 75 pg mL(-1) significantly increases the risk for development of rickets [odds ratio (OR) 2·8; 95% confidence interval (CI) 1·05-7·40; P = 0·04] in ichthyosis. Among the different types, patients with ARCI (OR 4·83; 95% CI 1·74-13·45; P < 0·01) and EI (OR 5·71; 95% CI 1·74-18·79; P < 0·01) are at an increased risk of developing rickets. © 2014 British Association of Dermatologists.

  17. Mastoiditis and facial paralysis as initial manifestations of temporal bone systemic diseases - the significance of the histopathological examination.

    Science.gov (United States)

    Maniu, Alma Aurelia; Harabagiu, Oana; Damian, Laura Otilia; Ştefănescu, Eugen HoraŢiu; FănuŢă, Bogdan Marius; Cătană, Andreea; Mogoantă, Carmen Aurelia

    2016-01-01

    Several systemic diseases, including granulomatous and infectious processes, tumors, bone disorders, collagen-vascular and other autoimmune diseases may involve the middle ear and temporal bone. These diseases are difficult to diagnose when symptoms mimic acute otomastoiditis. The present report describes our experience with three such cases initially misdiagnosed. Their predominating symptoms were otological with mastoiditis, hearing loss, and subsequently facial nerve palsy. The cases were considered an emergency and the patients underwent tympanomastoidectomy, under the suspicion of otitis media with cholesteatoma, in order to remove a possible abscess and to decompress the facial nerve. The common features were the presence of severe granulation tissue filling the mastoid cavity and middle ear during surgery, without cholesteatoma. The definitive diagnoses was made by means of biopsy of the granulation tissue from the middle ear, revealing granulomatosis with polyangiitis (formerly known as Wegener's granulomatosis) in one case, middle ear tuberculosis and diffuse large B-cell lymphoma respectively. After specific associated therapy facial nerve functions improved, and atypical inflammatory states of the ear resolved. As a group, systemic diseases of the middle ear and temporal bone are uncommon, but aggressive lesions. After analyzing these cases and reviewing the literature, we would like to stress upon the importance of microscopic examination of the affected tissue, required for an accurate diagnosis and effective treatment.

  18. Temporal bone CT findings of tuberculous otitis media : comparison with chronic otitis media

    International Nuclear Information System (INIS)

    Chang, Jeong A; Rho, Myung Ho; Kim, Young Min; Lee, Ho Seung; Choi, Pil Yeob; Seong, Young Soon; Kwon, Jae Soo; Lee, Sang Wook; Jung, Keon Sik

    1999-01-01

    To compare the differential findings of tuberculous otitis media(TOM) with those of chronic sup purative otitis media with or without cholesteatoma, as seen on high resolution temporal bone CT. We retrospectively reviewed 14 cases of TOM, 30 cases of chronic suppurative otitis media(CSOM), and 30 cases of chronic otitis media with cholesteatoma(Chole). All had been pathologically confirmed. We evaluated the preservation of mastoid cells without sclerotic change, the location and extension of soft tissue to the external auditary canal, and erosion of ossicles, the tegmen tympani, scutum, bony labyrinth, facial nerve canal and sigmoid sinus, and the presence of intracranial complications. Soft tissue in the mastoid antrum was seen in all cases of TOM(100%), 29 cases of CSOM(96.7%), and 26 cases of Chole(86.7%). In contrast, the soft tissue in the entire middle ear cavity was noted in 13 cases of TOM(92.8%), 7 cases of CSOM(23.3%), and 12 cases of Chole(40%). Soft tissue extended to the superior aspect of the external auditory canal in 4 cases of TOM (28.6%) and 5 cases of Chole (16.7%). Mastoid air cells were seen in 9 cases of TOM (64.3%), 4 cases of CSOM (13.3%), and 3 cases of Chole(10%). Ossicular erosion was noted in 6 cases of TOM (42.9%), 12 cases of CSOM (40%), and 26 cases of Chole(86.7%), while in one case of TOM (7.1%), 5 cases of CSOM (16.7%), and 15 cases of Chole(50%) there was erosion of the scutum. In one case of TOM, follow-up CT study after 9 months of antituberculous medication without surgery revealed complete clearing of previously noted soft tissue in the middle ear cavity. Specific CT findings of TOM were not seen, but if there were findings of soft tissue in the entire middle ear cavity, soft tissue extension to the external auditory canal, preservation of mastoid air cells without sclerotic change, and intact scutum, TOM may be differentiated from other chronic otitis media

  19. Temporal bone CT findings of tuberculous otitis media : comparison with chronic otitis media

    Energy Technology Data Exchange (ETDEWEB)

    Chang, Jeong A; Rho, Myung Ho; Kim, Young Min; Lee, Ho Seung; Choi, Pil Yeob; Seong, Young Soon; Kwon, Jae Soo; Lee, Sang Wook [Masan Samsung Hospital, Sungkyunkwan Univ. College of Medicine, Seoul (Korea, Republic of); Jung, Keon Sik [Pohang Sunrin Hospital, Pohang (Korea, Republic of)

    1999-06-01

    To compare the differential findings of tuberculous otitis media(TOM) with those of chronic sup purative otitis media with or without cholesteatoma, as seen on high resolution temporal bone CT. We retrospectively reviewed 14 cases of TOM, 30 cases of chronic suppurative otitis media(CSOM), and 30 cases of chronic otitis media with cholesteatoma(Chole). All had been pathologically confirmed. We evaluated the preservation of mastoid cells without sclerotic change, the location and extension of soft tissue to the external auditary canal, and erosion of ossicles, the tegmen tympani, scutum, bony labyrinth, facial nerve canal and sigmoid sinus, and the presence of intracranial complications. Soft tissue in the mastoid antrum was seen in all cases of TOM(100%), 29 cases of CSOM(96.7%), and 26 cases of Chole(86.7%). In contrast, the soft tissue in the entire middle ear cavity was noted in 13 cases of TOM(92.8%), 7 cases of CSOM(23.3%), and 12 cases of Chole(40%). Soft tissue extended to the superior aspect of the external auditory canal in 4 cases of TOM (28.6%) and 5 cases of Chole (16.7%). Mastoid air cells were seen in 9 cases of TOM (64.3%), 4 cases of CSOM (13.3%), and 3 cases of Chole(10%). Ossicular erosion was noted in 6 cases of TOM (42.9%), 12 cases of CSOM (40%), and 26 cases of Chole(86.7%), while in one case of TOM (7.1%), 5 cases of CSOM (16.7%), and 15 cases of Chole(50%) there was erosion of the scutum. In one case of TOM, follow-up CT study after 9 months of antituberculous medication without surgery revealed complete clearing of previously noted soft tissue in the middle ear cavity. Specific CT findings of TOM were not seen, but if there were findings of soft tissue in the entire middle ear cavity, soft tissue extension to the external auditory canal, preservation of mastoid air cells without sclerotic change, and intact scutum, TOM may be differentiated from other chronic otitis media.

  20. The temporal expression of estrogen receptor alpha-36 and runx2 in human bone marrow derived stromal cells during osteogenesis

    International Nuclear Information System (INIS)

    Francis, W.R.; Owens, S.E.; Wilde, C.; Pallister, I.; Kanamarlapudi, V.; Zou, W.; Xia, Z.

    2014-01-01

    Highlights: • ERα36 is the predominant ERα isoform involved in bone regulation in human BMSC. • ERα36 mRNA is significantly upregulated during the process of osteogenesis. • The pattern of ERα36 and runx2 mRNA expression is similar during osteogenesis. • ERα36 appears to be co-localised with runx2 during osteogenesis. - Abstract: During bone maintenance in vivo, estrogen signals through estrogen receptor (ER)-α. The objectives of this study were to investigate the temporal expression of ERα36 and ascertain its functional relevance during osteogenesis in human bone marrow derived stromal cells (BMSC). This was assessed in relation to runt-related transcription factor-2 (runx2), a main modulatory protein involved in bone formation. ERα36 and runx2 subcellular localisation was assessed using immunocytochemistry, and their mRNA expression levels by real time PCR throughout the process of osteogenesis. The osteogenically induced BMSCs demonstrated a rise in ERα36 mRNA during proliferation followed by a decline in expression at day 10, which represents a change in dynamics within the culture between the proliferative stage and the differentiative stage. The mRNA expression profile of runx2 mirrored that of ERα36 and showed a degree subcellular co-localisation with ERα36. This study suggests that ERα36 is involved in the process of osteogenesis in BMSCs, which has implications in estrogen deficient environments

  1. Does colon cancer ever metastasize to bone first? a temporal analysis of colorectal cancer progression

    International Nuclear Information System (INIS)

    Roth, Eira S; Fetzer, David T; Barron, Bruce J; Joseph, Usha A; Gayed, Isis W; Wan, David Q

    2009-01-01

    It is well recognized that colorectal cancer does not frequently metastasize to bone. The aim of this retrospective study was to establish whether colorectal cancer ever bypasses other organs and metastasizes directly to bone and whether the presence of lung lesions is superior to liver as a better predictor of the likelihood and timing of bone metastasis. We performed a retrospective analysis on patients with a clinical diagnosis of colon cancer referred for staging using whole-body 18 F-FDG PET and CT or PET/CT. We combined PET and CT reports from 252 individuals with information concerning patient history, other imaging modalities, and treatments to analyze disease progression. No patient had isolated osseous metastasis at the time of diagnosis, and none developed isolated bone metastasis without other organ involvement during our survey period. It took significantly longer for colorectal cancer patients to develop metastasis to the lungs (23.3 months) or to bone (21.2 months) than to the liver (9.8 months). Conclusion: Metastasis only to bone without other organ involvement in colorectal cancer patients is extremely rare, perhaps more rare than we previously thought. Our findings suggest that resistant metastasis to the lungs predicts potential disease progression to bone in the colorectal cancer population better than liver metastasis does

  2. The 'temporal effect' in hominids: Reinvestigating the nature of support for a chimp-human clade in bone morphology.

    Science.gov (United States)

    Pearson, Alannah; Groves, Colin; Cardini, Andrea

    2015-11-01

    In 2004, an analysis by Lockwood and colleagues of hard-tissue morphology, using geometric morphometrics on the temporal bone, succeeded in recovering the correct phylogeny of living hominids without resorting to potentially problematic methods for transforming continuous shape variables into meristic characters. That work has increased hope that by using modern analytical methods and phylogenetically informative anatomical data we might one day be able to accurately infer the relationships of hominins, including the closest extinct relatives of modern humans. In the present study, using 3D virtually generated models of the hominid temporal bone and a larger suite of geometric morphometric and comparative techniques, we have re-examined the evidence for a Pan-Homo clade. Despite differences in samples, as well as the type of raw data, the effect of measurement error (and especially landmark digitization by a different operator), but also a broader perspective brought in by our diverse set of approaches, our reanalysis largely supports Lockwood and colleagues' original results. However, by focusing not only mainly on shape (as in the original 2004 analysis) but also on size and 'size-corrected' (non-allometric) shape, we demonstrate that the strong phylogenetic signal in the temporal bone is largely related to similarities in size. Thus, with this study, we are not suggesting the use of a single 'character', such as size, for phylogenetic inference, but we do challenge the common view that shape, with its highly complex and multivariate nature, is necessarily more phylogenetically informative than size and that actually size and size-related shape variation (i.e., allometry) confound phylogenetic inference based on morphology. This perspective may in fact be less generalizable than often believed. Thus, while we confirm the original findings by Lockwood et al., we provide a deep reinterpretation of their nature and potential implications for hominid phylogenetics

  3. Radiation dose optimization in pediatric temporal bone computed tomography: influence of tube tension on image contrast and image quality.

    Science.gov (United States)

    Nauer, Claude Bertrand; Zubler, Christoph; Weisstanner, Christian; Stieger, Christof; Senn, Pascal; Arnold, Andreas

    2012-03-01

    The purpose of this experimental study was to investigate the effect of tube tension reduction on image contrast and image quality in pediatric temporal bone computed tomography (CT). Seven lamb heads with infant-equivalent sizes were scanned repeatedly, using four tube tensions from 140 to 80 kV while the CT-Dose Index (CTDI) was held constant. Scanning was repeated with four CTDI values from 30 to 3 mGy. Image contrast was calculated for the middle ear as the Hounsfield unit (HU) difference between bone and air and for the inner ear as the HU difference between bone and fluid. The influence of tube tension on high-contrast detail delineation was evaluated using a phantom. The subjective image quality of eight middle and inner ear structures was assessed using a 4-point scale (scores 1-2 = insufficient; scores 3-4 = sufficient). Middle and inner ear contrast showed a near linear increase with tube tension reduction (r = -0.94/-0.88) and was highest at 80 kV. Tube tension had no influence on spatial resolution. Subjective image quality analysis showed significantly better scoring at lower tube tensions, with highest image quality at 80 kV. However, image quality improvement was most relevant for low-dose scans. Image contrast in the temporal bone is significantly higher at low tube tensions, leading to a better subjective image quality. Highest contrast and best quality were found at 80 kV. This image quality improvement might be utilized to further reduce the radiation dose in pediatric low-dose CT protocols.

  4. Radiation dose optimization in pediatric temporal bone computed tomography: influence of tube tension on image contrast and image quality

    International Nuclear Information System (INIS)

    Nauer, Claude Bertrand; Zubler, Christoph; Weisstanner, Christian; Stieger, Christof; Senn, Pascal; Arnold, Andreas

    2012-01-01

    The purpose of this experimental study was to investigate the effect of tube tension reduction on image contrast and image quality in pediatric temporal bone computed tomography (CT). Seven lamb heads with infant-equivalent sizes were scanned repeatedly, using four tube tensions from 140 to 80 kV while the CT-Dose Index (CTDI) was held constant. Scanning was repeated with four CTDI values from 30 to 3 mGy. Image contrast was calculated for the middle ear as the Hounsfield unit (HU) difference between bone and air and for the inner ear as the HU difference between bone and fluid. The influence of tube tension on high-contrast detail delineation was evaluated using a phantom. The subjective image quality of eight middle and inner ear structures was assessed using a 4-point scale (scores 1-2 = insufficient; scores 3-4 = sufficient). Middle and inner ear contrast showed a near linear increase with tube tension reduction (r = -0.94/-0.88) and was highest at 80 kV. Tube tension had no influence on spatial resolution. Subjective image quality analysis showed significantly better scoring at lower tube tensions, with highest image quality at 80 kV. However, image quality improvement was most relevant for low-dose scans. Image contrast in the temporal bone is significantly higher at low tube tensions, leading to a better subjective image quality. Highest contrast and best quality were found at 80 kV. This image quality improvement might be utilized to further reduce the radiation dose in pediatric low-dose CT protocols. (orig.)

  5. CT examination of the temporal bones for cochlear implantation; Badanie CT kosci skroniowych u osob przygotowanych do operacji wszczepu slimakowego

    Energy Technology Data Exchange (ETDEWEB)

    Szyfter, W.; Szymiec, E.; Pruszewicz, A.; Szmeja, Z.; Paprzycki, W.; Sekula, A.; Przybylska, J. [Klinika Otolaryngologii, Akademia Medyczna, Poznan (Poland)

    1995-12-31

    CT examinations of temporal bones were performed in 31 deaf patients from qualify diagnostic stage prepared for cochlear implants. Essential for surgical treatment anatomic details were estimated, especially canalis spiralis cochleae was paid to attention. In our group, 8 patients had some CT changes (abnormal findings) which corresponded to different stages of obliteration the cochlea. The majority of patients lost the hearing because of meningitis. In 6 implanted patients CT scan was compared with surgical findings. During the operation 1 patient with patent cochlea on CT scan had partially ossified basal turn. (author) 16 refs, 4 figs, 2 tabs

  6. Temporal bone extramedullary hematopoiesis as a causeof pediatric bilateral conductive hearing loss:Case report and review of the literature.

    Science.gov (United States)

    Lanigan, Alexander; Fordham, M Taylor

    2017-06-01

    Extramedullary hematopoiesis occurs in children with hemoglobinopathy and chronic anemia. The liver and spleen are often affected first, but other foci can develop to support erythrocyte demand. We report a case of a nine-year-old with beta thalassemia and temporal bone extramedullary hematopoiesis causing ossicular fixation and bilateral conductive hearing loss. There is only one case in the literature describing this phenomenon in pediatric patients, and this is the first case report of bilateral hearing loss from this physiologic phenomenon. Otolaryngologists should consider this etiology in patients with chronic anemia and conductive hearing loss in the absence of otitis media. Published by Elsevier B.V.

  7. 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.

  8. High strength, biodegradable and cytocompatible alpha tricalcium phosphate-iron composites for temporal reduction of bone fractures.

    Science.gov (United States)

    Montufar, E B; Casas-Luna, M; Horynová, M; Tkachenko, S; Fohlerová, Z; Diaz-de-la-Torre, S; Dvořák, K; Čelko, L; Kaiser, J

    2018-04-01

    In this work alpha tricalcium phosphate (α-TCP)/iron (Fe) composites were developed as a new family of biodegradable, load-bearing and cytocompatible materials. The composites with composition from pure ceramic to pure metallic samples were consolidated by pulsed electric current assisted sintering to minimise processing time and temperature while improving their mechanical performance. The mechanical strength of the composites was increased and controlled with the Fe content, passing from brittle to ductile failure. In particular, the addition of 25 vol% of Fe produced a ceramic matrix composite with elastic modulus much closer to cortical bone than that of titanium or biodegradable magnesium alloys and specific compressive strength above that of stainless steel, chromium-cobalt alloys and pure titanium, currently used in clinic for internal fracture fixation. All the composites studied exhibited higher degradation rate than their individual components, presenting values around 200 μm/year, but also their compressive strength did not show a significant reduction in the period required for bone fracture consolidation. Composites showed preferential degradation of α-TCP areas rather than β-TCP areas, suggesting that α-TCP can produce composites with higher degradation rate. The composites were cytocompatible both in indirect and direct contact with bone cells. Osteoblast-like cells attached and spread on the surface of the composites, presenting proliferation rate similar to cells on tissue culture-grade polystyrene and they showed alkaline phosphatase activity. Therefore, this new family of composites is a potential alternative to produce implants for temporal reduction of bone fractures. Biodegradable alpha-tricalcium phosphate/iron (α-TCP/Fe) composites are promising candidates for the fabrication of temporal osteosynthesis devices. Similar to biodegradable metals, these composites can avoid implant removal after bone fracture healing, particularly in

  9. Two developmentally temporal quantitative trait loci underlie convergent evolution of increased branchial bone length in sticklebacks

    Science.gov (United States)

    Erickson, Priscilla A.; Glazer, Andrew M.; Cleves, Phillip A.; Smith, Alyson S.; Miller, Craig T.

    2014-01-01

    In convergent evolution, similar phenotypes evolve repeatedly in independent populations, often reflecting adaptation to similar environments. Understanding whether convergent evolution proceeds via similar or different genetic and developmental mechanisms offers insight towards the repeatability and predictability of evolution. Oceanic populations of threespine stickleback fish, Gasterosteus aculeatus, have repeatedly colonized countless freshwater lakes and streams, where new diets lead to morphological adaptations related to feeding. Here, we show that heritable increases in branchial bone length have convergently evolved in two independently derived freshwater stickleback populations. In both populations, an increased bone growth rate in juveniles underlies the convergent adult phenotype, and one population also has a longer cartilage template. Using F2 crosses from these two freshwater populations, we show that two quantitative trait loci (QTL) control branchial bone length at distinct points in development. In both populations, a QTL on chromosome 21 controls bone length throughout juvenile development, and a QTL on chromosome 4 controls bone length only in adults. In addition to these similar developmental profiles, these QTL show similar chromosomal locations in both populations. Our results suggest that sticklebacks have convergently evolved longer branchial bones using similar genetic and developmental programmes in two independently derived populations. PMID:24966315

  10. Temporal trends in obesity, osteoporosis treatment, bone mineral density, and fracture rates: a population-based historical cohort study.

    Science.gov (United States)

    Leslie, William D; Lix, Lisa M; Yogendran, Marina S; Morin, Suzanne N; Metge, Colleen J; Majumdar, Sumit R

    2014-04-01

    Diverging international trends in fracture rates have been observed, with most reports showing that fracture rates have stabilized or decreased in North American and many European populations. We studied two complementary population-based historical cohorts from the Province of Manitoba, Canada (1996-2006) to determine whether declining osteoporotic fracture rates in Canada are attributable to trends in obesity, osteoporosis treatment, or bone mineral density (BMD). The Population Fracture Registry included women aged 50 years and older with major osteoporotic fractures, and was used to assess impact of changes in osteoporosis treatment. The BMD Registry included all women aged 50 years and older undergoing BMD tests, and was used to assess impact of changes in obesity and BMD. Model-based estimates of temporal changes in fracture rates (Fracture Registry) were calculated. Temporal changes in obesity and BMD and their association with fracture rates (BMD Registry) were estimated. In the Fracture Registry (n=27,341), fracture rates declined 1.6% per year (95% confidence interval [CI], 1.3% to 2.0%). Although osteoporosis treatment increased from 5.6% to 17.4%, the decline in fractures was independent of osteoporosis treatment. In the BMD Registry (n=36,587), obesity increased from 12.7% to 27.4%. Femoral neck BMD increased 0.52% per year and lumbar spine BMD increased 0.32% per year after covariate adjustment (pobesity or osteoporosis treatment. © 2014 American Society for Bone and Mineral Research.

  11. Congenital stapes malformation: Rare conductive hearing loss in a patient with Waardenburg syndrome.

    Science.gov (United States)

    Melzer, Jonathan M; Eliason, Michael; Conley, George S

    2016-04-01

    Waardenburg syndrome is a known autosomal dominant cause of congenital hearing loss. It is characterized by a distinctive phenotypic appearance and often involves sensorineural hearing loss. Temporal bone abnormalities and inner ear dysmorphisms have been described in association with the disease. However, middle ear abnormalities as causes of conductive hearing loss are not typically seen in Waardenburg syndrome. We discuss a case of an 8-year-old female who meets diagnostic criteria for Waardenburg syndrome type 3 and who presented with a bilateral conductive hearing loss associated with congenital stapes fixation. We discuss management strategy in this previously unreported phenotype. © 2015 The American Laryngological, Rhinological and Otological Society, Inc.

  12. Sigmoid plate dehiscence: Congenital or acquired condition?

    Energy Technology Data Exchange (ETDEWEB)

    Liu, Zhaohui, E-mail: lzhtrhos@163.com [Capital Medical University, Beijing Tongren Hospital, No 1 Dong Jiao Min Street, Dongcheng District, Beijing 100730 (China); Li, Jing, E-mail: lijingxbh@yahoo.com.cn [Capital Medical University, Beijing Tongren Hospital, No 1 Dong Jiao Min Street, Dongcheng District, Beijing 100730 (China); Zhao, Pengfei, E-mail: zhaopengf05@163.com [Capital Medical University, Beijing Friendship Hospital, No 95 Yongan Road, Xicheng District, Beijing 100050 (China); Lv, Han, E-mail: chrislvhan@126.com [Capital Medical University, Beijing Friendship Hospital, No 95 Yongan Road, Xicheng District, Beijing 100050 (China); Dong, Cheng, E-mail: derc007@sina.com [Capital Medical University, Beijing Friendship Hospital, No 95 Yongan Road, Xicheng District, Beijing 100050 (China); Liu, Wenjuan, E-mail: wenjuanliu@163.com [Jining No. 1 People' s Hospital, No. 6 Health Street, Jining 272100 (China); Wang, Zhenchang, E-mail: cjr.wzhch@vip.163.com [Capital Medical University, Beijing Friendship Hospital, No 95 Yongan Road, Xicheng District, Beijing 100050 (China)

    2015-05-15

    Highlights: • CT with multiplanar reformations can accurately display the sigmoid platet dehiscence. • The prevalence of sigmoid plate dehiscence was no significant difference among different age groups. • The size of sigmoid plate bony defects were not statistically different among different age groups. • The sigmoid plate dehiscence is more commonly a congenital than an acquired condition. - Abstract: Background and purpose: The imaging features of sigmoid plate dehiscence-induced pulsatile tinnitus have been presented. The origin of the sigmoid plate dehiscence, however, remains unclear. Our aim was to assess the prevalence and extent of sigmoid plate dehiscence on computed tomography (CT) images in multiple age groups to determine whether this condition is more likely to be congenital or acquired. Materials and methods: We retrospectively reviewed contrast-enhanced CT images of sigmoid plates of temporal bones in 504 patients. Each temporal bone was characterized as normal or dehiscent. Patients were then subcategorized into four age groups, and the prevalence and extent of dehiscent sigmoid plates in each group were calculated and compared. Results: Overall, 80 patients had sigmoid plate dehiscence, nine of whom had it bilaterally. In successively older age groups, the prevalences of sigmoid plate dehiscence were 18.9%, 20.1%, 14.5%, and 12.7%, respectively. Respective average anteroposterior bony defect diameters were 3.7 ± 1.7, 3.0 ± 1.3, 3.1 ± 1.5, and 3.0 ± 1.1 mm. Respective average vertical bony defect diameters were 3.6 ± 2.3, 2.6 ± 1.2, 3.2 ± 1.5, and 3.0 ± 1.7 mm. The prevalence and extent of sigmoid plate dehiscence were not statistically different among the four age groups. Conclusions: The similar radiologic prevalence and extent of dehiscent sigmoid plates among the age groups suggest that the dehiscence is more commonly a congenital than an acquired condition.

  13. Sigmoid plate dehiscence: Congenital or acquired condition?

    International Nuclear Information System (INIS)

    Liu, Zhaohui; Li, Jing; Zhao, Pengfei; Lv, Han; Dong, Cheng; Liu, Wenjuan; Wang, Zhenchang

    2015-01-01

    Highlights: • CT with multiplanar reformations can accurately display the sigmoid platet dehiscence. • The prevalence of sigmoid plate dehiscence was no significant difference among different age groups. • The size of sigmoid plate bony defects were not statistically different among different age groups. • The sigmoid plate dehiscence is more commonly a congenital than an acquired condition. - Abstract: Background and purpose: The imaging features of sigmoid plate dehiscence-induced pulsatile tinnitus have been presented. The origin of the sigmoid plate dehiscence, however, remains unclear. Our aim was to assess the prevalence and extent of sigmoid plate dehiscence on computed tomography (CT) images in multiple age groups to determine whether this condition is more likely to be congenital or acquired. Materials and methods: We retrospectively reviewed contrast-enhanced CT images of sigmoid plates of temporal bones in 504 patients. Each temporal bone was characterized as normal or dehiscent. Patients were then subcategorized into four age groups, and the prevalence and extent of dehiscent sigmoid plates in each group were calculated and compared. Results: Overall, 80 patients had sigmoid plate dehiscence, nine of whom had it bilaterally. In successively older age groups, the prevalences of sigmoid plate dehiscence were 18.9%, 20.1%, 14.5%, and 12.7%, respectively. Respective average anteroposterior bony defect diameters were 3.7 ± 1.7, 3.0 ± 1.3, 3.1 ± 1.5, and 3.0 ± 1.1 mm. Respective average vertical bony defect diameters were 3.6 ± 2.3, 2.6 ± 1.2, 3.2 ± 1.5, and 3.0 ± 1.7 mm. The prevalence and extent of sigmoid plate dehiscence were not statistically different among the four age groups. Conclusions: The similar radiologic prevalence and extent of dehiscent sigmoid plates among the age groups suggest that the dehiscence is more commonly a congenital than an acquired condition

  14. 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 ...

  15. Hypoglossal-facial nerve "side"-to-side neurorrhaphy for facial paralysis resulting from closed temporal bone fractures.

    Science.gov (United States)

    Su, Diya; Li, Dezhi; Wang, Shiwei; Qiao, Hui; Li, Ping; Wang, Binbin; Wan, Hong; Schumacher, Michael; Liu, Song

    2018-06-06

    Closed temporal bone fractures due to cranial trauma often result in facial nerve injury, frequently inducing incomplete facial paralysis. Conventional hypoglossal-facial nerve end-to-end neurorrhaphy may not be suitable for these injuries because sacrifice of the lesioned facial nerve for neurorrhaphy destroys the remnant axons and/or potential spontaneous innervation. we modified the classical method by hypoglossal-facial nerve "side"-to-side neurorrhaphy using an interpositional predegenerated nerve graft to treat these injuries. Five patients who experienced facial paralysis resulting from closed temporal bone fractures due to cranial trauma were treated with the "side"-to-side neurorrhaphy. An additional 4 patients did not receive the neurorrhaphy and served as controls. Before treatment, all patients had suffered House-Brackmann (H-B) grade V or VI facial paralysis for a mean of 5 months. During the 12-30 months of follow-up period, no further detectable deficits were observed, but an improvement in facial nerve function was evidenced over time in the 5 neurorrhaphy-treated patients. At the end of follow-up, the improved facial function reached H-B grade II in 3, grade III in 1 and grade IV in 1 of the 5 patients, consistent with the electrophysiological examinations. In the control group, two patients showed slightly spontaneous innervation with facial function improved from H-B grade VI to V, and the other patients remained unchanged at H-B grade V or VI. We concluded that the hypoglossal-facial nerve "side"-to-side neurorrhaphy can preserve the injured facial nerve and is suitable for treating significant incomplete facial paralysis resulting from closed temporal bone fractures, providing an evident beneficial effect. Moreover, this treatment may be performed earlier after the onset of facial paralysis in order to reduce the unfavorable changes to the injured facial nerve and atrophy of its target muscles due to long-term denervation and allow axonal

  16. Nerve canals at the fundus of the internal auditory canal on high-resolution temporal bone CT

    International Nuclear Information System (INIS)

    Ji, Yoon Ha; Youn, Eun Kyung; Kim, Seung Chul

    2001-01-01

    To identify and evaluate the normal anatomy of nerve canals in the fundus of the internal auditory canal which can be visualized on high-resolution temporal bone CT. We retrospectively reviewed high-resolution (1 mm thickness and interval contiguous scan) temporal bone CT images of 253 ears in 150 patients who had not suffered trauma or undergone surgery. Those with a history of uncomplicated inflammatory disease were included, but those with symptoms of vertigo, sensorineural hearing loss, or facial nerve palsy were excluded. Three radiologists determined the detectability and location of canals for the labyrinthine segment of the facial, superior vestibular and cochlear nerve, and the saccular branch and posterior ampullary nerve of the inferior vestibular nerve. Five bony canals in the fundus of the internal auditory canal were identified as nerve canals. Four canals were identified on axial CT images in 100% of cases; the so-called singular canal was identified in only 68%. On coronal CT images, canals for the labyrinthine segment of the facial and superior vestibular nerve were seen in 100% of cases, but those for the cochlear nerve, the saccular branch of the inferior vestibular nerve, and the singular canal were seen in 90.1%, 87.4% and 78% of cases, respectiveIy. In all detectable cases, the canal for the labyrinthine segment of the facial nerve was revealed as one which traversed anterolateralIy, from the anterosuperior portion of the fundus of the internal auditory canal. The canal for the cochlear nerve was located just below that for the labyrinthine segment of the facial nerve, while that canal for the superior vestibular nerve was seen at the posterior aspect of these two canals. The canal for the saccular branch of the inferior vestibular nerve was located just below the canal for the superior vestibular nerve, and that for the posterior ampullary nerve, the so-called singular canal, ran laterally or posteolateralIy from the posteroinferior aspect of

  17. Nerve canals at the fundus of the internal auditory canal on high-resolution temporal bone CT

    Energy Technology Data Exchange (ETDEWEB)

    Ji, Yoon Ha; Youn, Eun Kyung; Kim, Seung Chul [Sungkyunkwan Univ., School of Medicine, Seoul (Korea, Republic of)

    2001-12-01

    To identify and evaluate the normal anatomy of nerve canals in the fundus of the internal auditory canal which can be visualized on high-resolution temporal bone CT. We retrospectively reviewed high-resolution (1 mm thickness and interval contiguous scan) temporal bone CT images of 253 ears in 150 patients who had not suffered trauma or undergone surgery. Those with a history of uncomplicated inflammatory disease were included, but those with symptoms of vertigo, sensorineural hearing loss, or facial nerve palsy were excluded. Three radiologists determined the detectability and location of canals for the labyrinthine segment of the facial, superior vestibular and cochlear nerve, and the saccular branch and posterior ampullary nerve of the inferior vestibular nerve. Five bony canals in the fundus of the internal auditory canal were identified as nerve canals. Four canals were identified on axial CT images in 100% of cases; the so-called singular canal was identified in only 68%. On coronal CT images, canals for the labyrinthine segment of the facial and superior vestibular nerve were seen in 100% of cases, but those for the cochlear nerve, the saccular branch of the inferior vestibular nerve, and the singular canal were seen in 90.1%, 87.4% and 78% of cases, respectiveIy. In all detectable cases, the canal for the labyrinthine segment of the facial nerve was revealed as one which traversed anterolateralIy, from the anterosuperior portion of the fundus of the internal auditory canal. The canal for the cochlear nerve was located just below that for the labyrinthine segment of the facial nerve, while that canal for the superior vestibular nerve was seen at the posterior aspect of these two canals. The canal for the saccular branch of the inferior vestibular nerve was located just below the canal for the superior vestibular nerve, and that for the posterior ampullary nerve, the so-called singular canal, ran laterally or posteolateralIy from the posteroinferior aspect of

  18. 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.

  19. The usefulness of MR imaging of the temporal bone in the evaluation of patients with facial and audiovestibular dysfunction

    Energy Technology Data Exchange (ETDEWEB)

    Park, Sang Uk; Kim, Hyung Jin; Cho, Young Kuk; Lim, Myung Kwan; Kim, Won Hong; Suh, Chang Hae; Lee, Seung Chul [Inha University College of Medicine, Incheon (Korea, Republic of)

    2002-01-01

    To evaluate the clinical utility of MR imaging of the temporal bone in patients with facial and audiovestibular dysfunction with particular emphasis on the importance of contrast enhancement. We retrospectively reviewed the MR images of 179 patients [72 men, 107 women; average age, 44 (range, 1-77) years] who presented with peripheral facial palsy (n=15), audiometrically proven sensorineural hearing loss (n=104), vertigo (n=109), or tinnitus (n=92). Positive MR imaging findings possibly responsible for the patients' clinical manifestations were categorized according to the anatomic sites and presumed etiologies of the lesions. We also assessed the utility of contrast-enhanced MR imaging by analyzing its contribution to the demonstration of lesions which would otherwise not have been apparent. All MR images were interpreted by two neuroradiologists, who reached their conclusions by consensus. MR images demonstrated positive findings, thought to account for the presenting symptoms, in 78 (44%) of 179 patients, including 15 (100%) of 15 with peripheral facial palsy, 43 (41%) of 104 with sensorineural hearing loss, 40 (37%) of 109 with vertigo, and 39 (42%) of 92 with tinnitus. Thirty (38%) of those 78 patients had lesions that could be confidently recognized only at contrast-enhanced MR imaging. Even though its use led to positive findings in less than half of these patients, MR imaging of the temporal bone is a useful diagnostic procedure in the evaluation of those with facial and audiovestibular dysfunction. Because it was only at contrast-enhanced MR imaging that a significant number of patients showed positive imaging findings which explained their clinical manifestations, the use of contrast material is highly recommended.

  20. Anatomy-Specific Virtual Reality Simulation in Temporal Bone Dissection: Perceived Utility and Impact on Surgeon Confidence.

    Science.gov (United States)

    Locketz, Garrett D; Lui, Justin T; Chan, Sonny; Salisbury, Kenneth; Dort, Joseph C; Youngblood, Patricia; Blevins, Nikolas H

    2017-06-01

    Objective To evaluate the effect of anatomy-specific virtual reality (VR) surgical rehearsal on surgeon confidence and temporal bone dissection performance. Study Design Prospective pre- and poststudy of a novel virtual surgical rehearsal platform. Setting Academic otolaryngology-head and neck surgery residency training programs. Subjects and Methods Sixteen otolaryngology-head and neck surgery residents from 2 North American training institutions were recruited. Surveys were administered to assess subjects' baseline confidence in performing 12 subtasks of cortical mastoidectomy with facial recess. A cadaver temporal bone was randomly assigned to each subject. Cadaver specimens were scanned with a clinical computed tomography protocol, allowing the creation of anatomy-specific models for use in a VR surgical rehearsal platform. Subjects then rehearsed a virtual mastoidectomy on data sets derived from their specimens. Surgical confidence surveys were administered again. Subjects then dissected assigned cadaver specimens, which were blindly graded with a modified Welling scale. A final survey assessed the perceived utility of rehearsal on dissection performance. Results Of 16 subjects, 14 (87.5%) reported a significant increase in overall confidence after conducting an anatomy-specific VR rehearsal. A significant correlation existed between perceived utility of rehearsal and confidence improvement. The effect of rehearsal on confidence was dependent on trainee experience and the inherent difficulty of the surgical subtask. Postrehearsal confidence correlated strongly with graded dissection performance. Subjects rated anatomy-specific rehearsal as having a moderate to high contribution to their dissection performance. Conclusion Anatomy-specific virtual rehearsal improves surgeon confidence in performing mastoid dissection, dependent on surgeon experience and task difficulty. The subjective confidence gained through rehearsal correlates positively with subsequent

  1. The usefulness of MR imaging of the temporal bone in the evaluation of patients with facial and audiovestibular dysfunction

    International Nuclear Information System (INIS)

    Park, Sang Uk; Kim, Hyung Jin; Cho, Young Kuk; Lim, Myung Kwan; Kim, Won Hong; Suh, Chang Hae; Lee, Seung Chul

    2002-01-01

    To evaluate the clinical utility of MR imaging of the temporal bone in patients with facial and audiovestibular dysfunction with particular emphasis on the importance of contrast enhancement. We retrospectively reviewed the MR images of 179 patients [72 men, 107 women; average age, 44 (range, 1-77) years] who presented with peripheral facial palsy (n=15), audiometrically proven sensorineural hearing loss (n=104), vertigo (n=109), or tinnitus (n=92). Positive MR imaging findings possibly responsible for the patients' clinical manifestations were categorized according to the anatomic sites and presumed etiologies of the lesions. We also assessed the utility of contrast-enhanced MR imaging by analyzing its contribution to the demonstration of lesions which would otherwise not have been apparent. All MR images were interpreted by two neuroradiologists, who reached their conclusions by consensus. MR images demonstrated positive findings, thought to account for the presenting symptoms, in 78 (44%) of 179 patients, including 15 (100%) of 15 with peripheral facial palsy, 43 (41%) of 104 with sensorineural hearing loss, 40 (37%) of 109 with vertigo, and 39 (42%) of 92 with tinnitus. Thirty (38%) of those 78 patients had lesions that could be confidently recognized only at contrast-enhanced MR imaging. Even though its use led to positive findings in less than half of these patients, MR imaging of the temporal bone is a useful diagnostic procedure in the evaluation of those with facial and audiovestibular dysfunction. Because it was only at contrast-enhanced MR imaging that a significant number of patients showed positive imaging findings which explained their clinical manifestations, the use of contrast material is highly recommended

  2. The usefulness of MR imaging of the temporal bone in the evaluation of patients with facial and audiovestibular dysfunction

    Energy Technology Data Exchange (ETDEWEB)

    Park, Sang Uk; Kim, Hyung Jin; Cho, Young Kuk; Lim, Myung Kwan; Kim, Won Hong; Suh, Chang Hae; Lee, Seung Chul [Inha University College of Medicine, Incheon (Korea, Republic of)

    2002-01-01

    To evaluate the clinical utility of MR imaging of the temporal bone in patients with facial and audiovestibular dysfunction with particular emphasis on the importance of contrast enhancement. We retrospectively reviewed the MR images of 179 patients [72 men, 107 women; average age, 44 (range, 1-77) years] who presented with peripheral facial palsy (n=15), audiometrically proven sensorineural hearing loss (n=104), vertigo (n=109), or tinnitus (n=92). Positive MR imaging findings possibly responsible for the patients' clinical manifestations were categorized according to the anatomic sites and presumed etiologies of the lesions. We also assessed the utility of contrast-enhanced MR imaging by analyzing its contribution to the demonstration of lesions which would otherwise not have been apparent. All MR images were interpreted by two neuroradiologists, who reached their conclusions by consensus. MR images demonstrated positive findings, thought to account for the presenting symptoms, in 78 (44%) of 179 patients, including 15 (100%) of 15 with peripheral facial palsy, 43 (41%) of 104 with sensorineural hearing loss, 40 (37%) of 109 with vertigo, and 39 (42%) of 92 with tinnitus. Thirty (38%) of those 78 patients had lesions that could be confidently recognized only at contrast-enhanced MR imaging. Even though its use led to positive findings in less than half of these patients, MR imaging of the temporal bone is a useful diagnostic procedure in the evaluation of those with facial and audiovestibular dysfunction. Because it was only at contrast-enhanced MR imaging that a significant number of patients showed positive imaging findings which explained their clinical manifestations, the use of contrast material is highly recommended.

  3. A novel framework for the temporal analysis of bone mineral density in metastatic lesions using CT images of the femur

    Science.gov (United States)

    Knoop, Tom H.; Derikx, Loes C.; Verdonschot, Nico; Slump, Cornelis H.

    2015-03-01

    In the progressive stages of cancer, metastatic lesions in often develop in the femur. The accompanying pain and risk of fracture dramatically affect the quality of life of the patient. Radiotherapy is often administered as palliative treatment to relieve pain and restore the bone around the lesion. It is thought to affect the bone mineralization of the treated region, but the quantitative relation between radiation dose and femur remineralization remains unclear. A new framework for the longitudinal analysis of CT-scans of patients receiving radiotherapy is presented to investigate this relationship. The implemented framework is capable of automatic calibration of Hounsfield Units to calcium equivalent values and the estimation of a prediction interval per scan. Other features of the framework are temporal registration of femurs using elastix, transformation of arbitrary Regions Of Interests (ROI), and extraction of metrics for analysis. Build in Matlab, the modular approach aids easy adaptation to the pertinent questions in the explorative phase of the research. For validation purposes, an in-vitro model consisting of a human cadaver femur with a milled hole in the intertrochanteric region was used, representing a femur with a metastatic lesion. The hole was incrementally stacked with plates of PMMA bone cement with variable radiopaqueness. Using a Kolmogorov-Smirnov (KS) test, changes in density distribution due to an increase of the calcium concentration could be discriminated. In a 21 cm3 ROI, changes in 8% of the volume from 888 ± 57mg • ml-1 to 1000 ± 80mg • ml-1 could be statistically proven using the proposed framework. In conclusion, the newly developed framework proved to be a useful and flexible tool for the analysis of longitudinal CT data.

  4. [Clinical analysis of 102 patients with congenital inner ear malformation].

    Science.gov (United States)

    Zhu, X; Lian, N; Cai, Z

    1995-01-01

    Hearing loss and CT findings of 200 ears from 102 cases with congenital malformation of inner ear were included in our study. Hearing loss was typically bilateral severe, or total deafness. 75 percent of them were found deaf within one-year-old. In addition, 47 patients' (46%) mothers were noted to have caught a cold in first trimester of pregnancy. Temporal bone abnormalties were described as five types: 1. Michel malformation, 2. Mondini malformation, 3. enlargement of the vestibular aqueducts, 4. developmental deformity of cochlear aqueduct, 5. developmental deformity of internal acoustic meatus. Most cases showed malformations of vestibule or vestibular aqueducts.

  5. Prenatal diagnosis of congenital heart disease using four-dimensional spatio-temporal image correlation (STIC) telemedicine via an Internet link: a pilot study.

    Science.gov (United States)

    Viñals, F; Mandujano, L; Vargas, G; Giuliano, A

    2005-01-01

    To assess whether the spatio-temporal image correlation (STIC) acquisition technique can be taught to a general obstetrician by e-mail; whether STIC volume datasets can be transmitted over the Internet; and whether STIC volume datasets analyzed offline at a remote setting can be used to confirm or exclude major cardiac defects (TELE-STIC). This was a prospective study involving 50 pregnant women with gestational ages ranging between 20 and 36 weeks. These patients were selected by two general obstetricians (operators) working in geographically remote areas of Chile. Although both obstetricians were users of equipment capable of four-dimensional (4D) ultrasound with STIC, they lacked skill in the performance of fetal cardiac examination. A dedicated web disk was created to upload the acquired volume datasets using an Internet broadband connection. Offline analysis was performed by a single investigator experienced in fetal echocardiography (the administrator). A telemedicine link via the Internet was possible in all cases. Seventy-seven volume datasets were sent to the web server. A complete cardiac examination according to set criteria was achieved by the administrator in 86% of the cases scanned by one operator and 95% of the cases scanned by the other operator. Three patients had cardiac defects confirmed postnatally, two fetuses had extracardiac anomalies and one fetus had a suspected cardiac defect unconfirmed by second-opinion TELE-STIC. There were two isolated major congenital heart defects. Both patients were given advice by e-mail and teleconference using a web camera about the likely outcome and benefits of scheduling in utero transport to a tertiary care center. STIC volumes can be obtained by operators inexperienced in fetal echocardiography, transmitted via the Internet, and their analysis enables recognition of most of the structures and views necessary to assess fetal cardiac anatomy. The preliminary use of TELE-STIC allowed us to demonstrate that

  6. Presbycusis: a human temporal bone study of individuals with flat audiometric patterns of hearing loss using a new method to quantify stria vascularis volume.

    Science.gov (United States)

    Nelson, Erik G; Hinojosa, Raul

    2003-10-01

    The purpose of this study was to determine the prevalence of stria vascularis atrophy in individuals with presbycusis and flat audiometric patterns of hearing loss. Individuals with presbycusis have historically been categorized by the shape of their audiograms, and flat audiometric thresholds have been reported to be associated with atrophy of the stria vascularis. Stria vascularis volume was not measured in these studies. Retrospective case review. Archival human temporal bones from individuals with presbycusis were selected on the basis of strict audiometric criteria for flat audiometric thresholds. Six temporal bones that met these criteria were identified and compared with 10 temporal bones in individuals with normal hearing. A unique quantitative method was developed to measure the stria vascularis volume in these temporal bones. The hair cell and spiral ganglion cell populations also were quantitatively evaluated. Only one of the six individuals with presbycusis and flat audiometric thresholds had significant atrophy of the stria vascularis. This individual with stria vascularis atrophy also had reduced inner hair cell, outer hair cell, and ganglion cell populations. Three of the individuals with presbycusis had spiral ganglion cell loss, three individuals had inner hair cell loss, and all six individuals had outer hair cell loss. The results of this investigation suggest that individuals with presbycusis and flat audiometric patterns of hearing loss infrequently have stria vascularis atrophy. Outer hair cell loss alone or in combination with inner hair cell or ganglion cell loss may be the cause of flat audiometric thresholds in individuals with presbycusis.

  7. Mondini defect in association with multiple congenital anomalies.

    Science.gov (United States)

    Sekhar, H K; Sachs, M

    1976-01-01

    A case of bilaterally symmetrical genetic aplasia conforming to Mondini type of congenital deformity in a 12-day-old child is presented with the help of temporal bone sections. Cochlear changes include a stunted modiolus, deficient interscalar septum between the middle and upper coils forming a scala communis cochleae, a degenerated organ of Corti and reduced spiral ganglion cells and dendrites. The vestibule is malformed, with membranous labyrinth being deficient. The utricle and semicircular canals are absent. There is no oval window or stapedial footplate, and the facial nerve is hypoplastic. An interesting feature is the unusual association of bilateral bony choanal atresia, atrial septal defect, cleft lip, absence of olfactory bulbs in the brain, and congenital ophthalmic anomalies.

  8. Radiation exposure to the lens of the eye in temporal bone radiography

    International Nuclear Information System (INIS)

    Paix, D.

    1980-01-01

    Both the sensitivity of the eye to radiation cataract and the doses delivered to it in petrous bone examination are ill-defined. However, the following guidelines are suggested: (1) radiation cataract is unlikely to be a contraindication in patients over 50; (2) lens exposure per film when using an AP projection should be about 5m Gy; this may be reduced tenfold when tomographic movements permit the use of eye shields containing 1mm of lead; (3) projections which do not include the eyes in the direct beam should entail lens doses of 0.2m Gy or less; (4) steps should be taken to prevent the accumulated dose to the patient's lens from reaching 300m Gy per year or 15 Gy in all

  9. Skull base, orbits, temporal bone, and cranial nerves: anatomy on MR imaging.

    Science.gov (United States)

    Morani, Ajaykumar C; Ramani, Nisha S; Wesolowski, Jeffrey R

    2011-08-01

    Accurate delineation, diagnosis, and treatment planning of skull base lesions require knowledge of the complex anatomy of the skull base. Because the skull base cannot be directly evaluated, imaging is critical for the diagnosis and management of skull base diseases. Although computed tomography (CT) is excellent for outlining the bony detail, magnetic resonance (MR) imaging provides better soft tissue detail and is helpful for evaluating the adjacent meninges, brain parenchyma, and bone marrow of the skull base. Thus, CT and MR imaging are often used together for evaluating skull base lesions. This article focuses on the radiologic anatomy of the skull base pertinent to MR imaging evaluation. Copyright © 2011 Elsevier Inc. All rights reserved.

  10. Mineralización ósea en niños y adolescentes con hipotiroidismo congénito Bone mineralization in children and adolescents presenting with a congenital hypothyroidism

    Directory of Open Access Journals (Sweden)

    Kesia Granela Cortiñas

    2011-08-01

    Full Text Available Introducción: en el hipotiroidismo congénito la mineralización ósea puede afectarse por la enfermedad o por los efectos del tratamiento con hormonas tiroideas. Objetivo: determinar en pacientes con hipotiroidismo congénito la mineralización ósea. Métodos: se realizó estudio descriptivo transversal pareado, en niños cubanos con hipotiroidismo congénito (n=67 y un grupo control (n=67. Se analizó la mineralización ósea por densitometría, y se tuvo en cuenta la edad, el sexo, la maduración ósea y sexual, el diagnóstico hormonal y el tratamiento. Se aplicaron intervalos de confianza (95 %, análisis de varianza y correlación con significación. Resultados: la densidad, el contenido mineral óseo y el z-score en niños cubanos con hipotiroidismo congénito, y el grupo control no fue diferente significativamente (p=0,466; 0,155; 0,416 respectivamente. Estimaciones de asociación de mineralización ósea por edad mostró diferencia significativa en la densidad mineral ósea y contenido mineral óseo (p=0,000 para ambos grupos, y el z-score solo para los enfermos. La mineralización ósea fue significativa en la dosis promedio con levotiroxina en la segunda y tercera fase ósea, y en el tiempo de tratamiento. En el hipotiroidismo congénito permanente hubo correlación con el diagnóstico hormonal y el tiempo de tratamiento (p=0,000. Conclusiones: la mineralización ósea es homogénea en ambos grupos. La densidad y el contenido mineral óseo en niños cubanos con hipotiroidismo congénito y el grupo control se asocian con la edad, y son independientes del sexo. El z-score en pacientes con hipotiroidismo congénito sufre variación con la edad, y es independiente al sexo en ambos grupos. La densidad y el contenido mineral óseo varían en niños con hipotiroidismo congénito.Introduction: in the case of the congenital hypothyroidism the bone mineralization may be affected by disease or by the effects of the thyroid hormones treatment

  11. 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 ...

  12. 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 ...

  13. Initial results of a new generation dual source CT system using only an in-plane comb filter for ultra-high resolution temporal bone imaging.

    Science.gov (United States)

    Meyer, Mathias; Haubenreisser, Holger; Raupach, Rainer; Schmidt, Bernhard; Lietzmann, Florian; Leidecker, Christianne; Allmendinger, Thomas; Flohr, Thomas; Schad, Lothar R; Schoenberg, Stefan O; Henzler, Thomas

    2015-01-01

    To prospectively evaluate radiation dose and image quality of a third generation dual-source CT (DSCT) without z-axis filter behind the patient for temporal bone CT. Forty-five patients were either examined on a first, second, or third generation DSCT in an ultra-high-resolution (UHR) temporal bone-imaging mode. On the third generation DSCT system, the tighter focal spot of 0.2 mm(2) removes the necessity for an additional z-axis-filter, leading to an improved z-axis radiation dose efficiency. Images of 0.4 mm were reconstructed using standard filtered-back-projection or iterative reconstruction (IR) technique for previous generations of DSCT and a novel IR algorithm for the third generation DSCT. Radiation dose and image quality were compared between the three DSCT systems. The statistically significantly highest subjective and objective image quality was evaluated for the third generation DSCT when compared to the first or second generation DSCT systems (all p generation examination as compared to the first and second generation DSCT. Temporal bone imaging without z-axis-UHR-filter and a novel third generation IR algorithm allows for significantly higher image quality while lowering effective dose when compared to the first two generations of DSCTs. • Omitting the z-axis-filter allows a reduction in radiation dose of 50% • A smaller focal spot of 0.2 mm (2) significantly improves spatial resolution • Ultra-high-resolution temporal-bone-CT helps to gain diagnostic information of the middle/inner ear.

  14. Initial results of a new generation dual source CT system using only an in-plane comb filter for ultra-high resolution temporal bone imaging

    International Nuclear Information System (INIS)

    Meyer, Mathias; Haubenreisser, Holger; Schoenberg, Stefan O.; Henzler, Thomas; Raupach, Rainer; Schmidt, Bernhard; Leidecker, Christianne; Allmendinger, Thomas; Flohr, Thomas; Lietzmann, Florian; Schad, Lothar R.

    2015-01-01

    To prospectively evaluate radiation dose and image quality of a third generation dual-source CT (DSCT) without z-axis filter behind the patient for temporal bone CT. Forty-five patients were either examined on a first, second, or third generation DSCT in an ultra-high-resolution (UHR) temporal bone-imaging mode. On the third generation DSCT system, the tighter focal spot of 0.2 mm 2 removesthe necessity for an additional z-axis-filter, leading to an improved z-axis radiation dose efficiency. Images of 0.4 mm were reconstructed using standard filtered-back-projection or iterative reconstruction (IR) technique for previous generations of DSCT and a novel IR algorithm for the third generation DSCT. Radiation dose and image quality were compared between the three DSCT systems. The statistically significantly highest subjective and objective image quality was evaluated for the third generation DSCT when compared to the first or second generation DSCT systems (all p < 0.05). Total effective dose was 63 %/39 % lower for the third generation examination as compared to the first and second generation DSCT. Temporal bone imaging without z-axis-UHR-filter and a novel third generation IR algorithm allows for significantly higher image quality while lowering effective dose when compared to the first two generations of DSCTs. (orig.)

  15. Freely-available, true-color volume rendering software and cryohistology data sets for virtual exploration of the temporal bone anatomy.

    Science.gov (United States)

    Kahrs, Lüder Alexander; Labadie, Robert Frederick

    2013-01-01

    Cadaveric dissection of temporal bone anatomy is not always possible or feasible in certain educational environments. Volume rendering using CT and/or MRI helps understanding spatial relationships, but they suffer in nonrealistic depictions especially regarding color of anatomical structures. Freely available, nonstained histological data sets and software which are able to render such data sets in realistic color could overcome this limitation and be a very effective teaching tool. With recent availability of specialized public-domain software, volume rendering of true-color, histological data sets is now possible. We present both feasibility as well as step-by-step instructions to allow processing of publicly available data sets (Visible Female Human and Visible Ear) into easily navigable 3-dimensional models using free software. Example renderings are shown to demonstrate the utility of these free methods in virtual exploration of the complex anatomy of the temporal bone. After exploring the data sets, the Visible Ear appears more natural than the Visible Human. We provide directions for an easy-to-use, open-source software in conjunction with freely available histological data sets. This work facilitates self-education of spatial relationships of anatomical structures inside the human temporal bone as well as it allows exploration of surgical approaches prior to cadaveric testing and/or clinical implementation. Copyright © 2013 S. Karger AG, Basel.

  16. Prevalence of radiographic semicircular canal dehiscence in very young children: an evaluation using high-resolution computed tomography of the temporal bones

    International Nuclear Information System (INIS)

    Hagiwara, Mari; Fatterpekar, Girish; Shaikh, Jamil A.; Fang, Yixin; Roehm, Pamela C.

    2012-01-01

    Previous studies suggest that semicircular canal dehiscences (SCDs) have a developmental origin. We hypothesized that if SCDs originate during development, incidence of radiographic SCDs in young children will be higher than in adults. Thirty-four temporal bone HRCTs of children younger than 2 years and 40 temporal bone HRCTs of patients older than 18 years were reformatted and re-evaluated for presence of SCD or canal thinning. Results were compared with indications for HRCT and clinical information. SCDs were detected in 27.3% of children younger than 2 years of age (superior, 13.8%; posterior, 20%) and in 3% of adults (P < 0.004). Of children with one radiographic dehiscence, 55.6% had multiple and 44% had bilateral SCDs on HRCT. No lateral canal SCDs were present. Thinning of bone overlying the semicircular canals was found in 44% of children younger than 2 years and 2.5% of adults (P < 0.0001). SCDs are more common on HRCTs of very young children. This supports the hypothesis that SCDs originate from discontinuation of bone deposition/maturation. However, SCDs on imaging do not necessarily correlate with canal dehiscence syndrome and should therefore be interpreted carefully. (orig.)

  17. Prevalence of radiographic semicircular canal dehiscence in very young children: an evaluation using high-resolution computed tomography of the temporal bones

    Energy Technology Data Exchange (ETDEWEB)

    Hagiwara, Mari; Fatterpekar, Girish [New York University School of Medicine, Department of Radiology, New York, NY (United States); Shaikh, Jamil A. [New York University School of Medicine, Department of Otolaryngology, New York, NY (United States); Fang, Yixin [New York University School of Medicine, Department of Otolaryngology, New York, NY (United States); New York University School of Medicine, Division of Biostatistics, Department of Environmental Medicine, New York, NY (United States); Roehm, Pamela C. [New York University School of Medicine, Department of Otolaryngology, New York, NY (United States); New York University School of Medicine, Department of Otolaryngology, Division of Otology/Neurotology, New York, NY (United States)

    2012-12-15

    Previous studies suggest that semicircular canal dehiscences (SCDs) have a developmental origin. We hypothesized that if SCDs originate during development, incidence of radiographic SCDs in young children will be higher than in adults. Thirty-four temporal bone HRCTs of children younger than 2 years and 40 temporal bone HRCTs of patients older than 18 years were reformatted and re-evaluated for presence of SCD or canal thinning. Results were compared with indications for HRCT and clinical information. SCDs were detected in 27.3% of children younger than 2 years of age (superior, 13.8%; posterior, 20%) and in 3% of adults (P < 0.004). Of children with one radiographic dehiscence, 55.6% had multiple and 44% had bilateral SCDs on HRCT. No lateral canal SCDs were present. Thinning of bone overlying the semicircular canals was found in 44% of children younger than 2 years and 2.5% of adults (P < 0.0001). SCDs are more common on HRCTs of very young children. This supports the hypothesis that SCDs originate from discontinuation of bone deposition/maturation. However, SCDs on imaging do not necessarily correlate with canal dehiscence syndrome and should therefore be interpreted carefully. (orig.)

  18. 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 ...

  19. The Effect of Scala Tympani Morphology on Basilar Membrane Contact With a Straight Electrode Array: A Human Temporal Bone Study.

    Science.gov (United States)

    Verberne, Juul; Risi, Frank; Campbell, Luke; Chambers, Scott; O'Leary, Stephen

    2017-01-01

    Scala tympani morphology influences the insertion dynamics and intra-scalar position of straight electrode arrays. Hearing preservation is the goal of cochlear implantation with current thin straight electrode arrays. These hug the lateral wall, facilitating full, atraumatic insertions. However, most studies still report some postoperative hearing loss. This study explores the influence of scala tympani morphology on array position relative to the basilar membrane and its possible contribution to postoperative hearing loss. Twenty-six fresh-frozen human temporal bones implanted with a straight electrode array were three-dimensionally reconstructed from micro-photographic histological sections. Insertion depth and the proximity between the array and basilar membrane were recorded. Lateral wall shape was quantified as a curvature ratio. Insertion depths ranged from 233 to 470 degrees. The mean first point of contact between the array and basilar membrane was 185 degrees; arrays tended to remain in contact with the membrane after first contacting it. Eighty-nine and 93% of arrays that reached the upper basal (>240-360 degrees) and second (>360-720 degrees) turns respectively contacted the basilar membrane in these regions. Scalar wall curvature ratio decreased significantly (the wall became steeper) from the basal to second turns. This shift correlated with a reduced distance between the array and basilar membrane. Scala tympani morphology influences the insertion dynamics and intra-scalar position of a straight electrode array. In addition to gross trauma of cochlear structures, contact between the array and basilar membrane and how this impacts membrane function should be considered in hearing preservation cases.

  20. Diagnostic criteria for enlarged vestibular aqueduct (EVA) on CT of the temporal bones. Borderline cases of EVA

    International Nuclear Information System (INIS)

    Matsumoto, Mariko; Hoshino, Tomoyuki; Kikura, Mikino; Kikawada, Keiko; Kikawada, Toru

    2005-01-01

    The objective of this study was to examine measurements of the vestibular aqueduct on axial CT figures and to examine the cases with borderline EVA. The width of the vestibular aqueduct was measured in two places, the midpoint of the duct and the external aperture in the posterior cranial fossa. Criteria was as follow: Enlargement; ≥1.5 mm at the midpoint, ≥2 mm at the aperture, Borderline; 1-1.4 mm at the midpoint, 1.5-1.9 mm at the aperture. Three hundred forty-five cases with CT scans of the temporal bones taken during July 2003 to June 2004 in the secondary ENT referral center. Those patients include sensorinearal, mixed or conductive deafness, vertigo, ear infections and other ear diseases. Enlarged vestibular aqueduct was found in 10 ears with sensorineural deafness (SD) and 2 ears without SD. Borderline measurements were found in 19 ears with SD and 33 ears without SD. The enlarged midpoint measurement was not seen in the cases without SD. More than 1.5 mm of the definition for the enlargement at the midpoint of the vestibular aqueduct seemed to be appropriate in the clinical situation. The measurement at the midpoint of the duct is more reliable than at the external aperture. The conductive component in EVA Syndrome with mixed hearing loss is present only at the lower frequencies (250, 500 Hz), not at the middle and higer frequencies. Long-term follow-up of hearing should be done in the borderline cases with check-up of PDS gene anomaly if necessary. (author)

  1. High-resolution T1-weighted 3D real IR imaging of the temporal bone using triple-dose contrast material

    Energy Technology Data Exchange (ETDEWEB)

    Naganawa, Shinji; Koshikawa, Tokiko; Nakamura, Tatsuya; Fukatsu, Hiroshi; Ishigaki, Takeo [Department of Radiology, Nagoya University School of Medicine, 65 Tsurumai-cho, Shouwa-ku, 466-8550, Nagoya (Japan); Aoki, Ikuo [Medical System Company, Toshiba Corporation, Tokyo (Japan)

    2003-12-01

    The small structures in the temporal bone are surrounded by bone and air. The objectives of this study were (a) to compare contrast-enhanced T1-weighted images acquired by fast spin-echo-based three-dimensional real inversion recovery (3D rIR) against those acquired by gradient echo-based 3D SPGR in the visualization of the enhancement of small structures in the temporal bone, and (b) to determine whether either 3D rIR or 3D SPGR is useful for visualizing enhancement of the cochlear lymph fluid. Seven healthy men (age range 27-46 years) volunteered to participate in this study. All MR imaging was performed using a dedicated bilateral quadrature surface phased-array coil for temporal bone imaging at 1.5 T (Visart EX, Toshiba, Tokyo, Japan). The 3D rIR images (TR/TE/TI: 1800 ms/10 ms/500 ms) and flow-compensated 3D SPGR images (TR/TE/FA: 23 ms/10 ms/25 ) were obtained with a reconstructed voxel size of 0.6 x 0.7 x 0.8 mm{sup 3}. Images were acquired before and 1, 90, 180, and 270 min after the administration of triple-dose Gd-DTPA-BMA (0.3 mmol/kg). In post-contrast MR images, the degree of enhancement of the cochlear aqueduct, endolymphatic sac, subarcuate artery, geniculate ganglion of the facial nerve, and cochlear lymph fluid space was assessed by two radiologists. The degree of enhancement was scored as follows: 0 (no enhancement); 1 (slight enhancement); 2 (intermediate between 1 and 3); and 3 (enhancement similar to that of vessels). Enhancement scores for the endolymphatic sac, subarcuate artery, and geniculate ganglion were higher in 3D rIR than in 3D SPGR. Washout of enhancement in the endolymphatic sac appeared to be delayed compared with that in the subarcuate artery, suggesting that the enhancement in the endolymphatic sac may have been due in part to non-vascular tissue enhancement. Enhancement of the cochlear lymph space was not observed in any of the subjects in 3D rIR and 3D SPGR. The 3D rIR sequence may be more sensitive than the 3D SPGR sequence in

  2. Muscle and bone follow similar temporal patterns of recovery from muscle-induced disuse due to botulinum toxin injection.

    Science.gov (United States)

    Manske, Sarah L; Boyd, Steven K; Zernicke, Ronald F

    2010-01-01

    If muscle force is a primary source for triggering bone adaptation, with disuse and reloading, bone changes should follow muscle changes. We examined the timing and magnitude of changes in muscle cross-sectional area (MCSA) and bone architecture in response to muscle inactivity following botulinum toxin (BTX) injection. We hypothesized that MCSA would return to baseline levels sooner than bone properties following BTX injection. Female BALB mice (15 weeks old) were injected with 20 muL of BTX (1 U/100 g body mass, n=18) or saline (SAL, n=18) into the posterior calf musculature of one limb. The contralateral limb (CON) served as an internal control. MCSA and bone properties were assessed at baseline, 2, 4, 8, 12, and 16 weeks post-injection using in vivo micro-CT at the tibia proximal metaphysis (bone only) and diaphysis. Muscles were dissected and weighed after sacrifice. Significant GroupxLegxTime interactions indicated that the maximal decrease in MCSA (56%), proximal metaphyseal BV/TV (38%) and proximal diaphyseal Ct.Ar (7%) occurred 4 weeks after injection. There was no delay prior to bone recovery as both muscle and bone properties began to recover after this time, but MCSA and BV/TV remained 15% and 20% lower, respectively, in the BTX-injected leg than the BTX-CON leg 16 weeks post-injection. Gastrocnemius mass (primarily fast-twitch) was 14% lower in the BTX-injected leg than the SAL-injected leg, while soleus mass (primarily slow-twitch) was 15% greater in the BTX group than the SAL group. Our finding that muscle size and bone began to recover at similar times after BTX injection was unexpected. This suggested that partial weight-bearing and/or return of slow-twitch muscle activity in the BTX leg may have been sufficient to stimulate bone recovery. Alternatively, muscle function may have recovered sooner than MCSA. Our results indicated that muscle cross-sectional area, while important, may not be the primary factor associated with bone loss and recovery

  3. 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.

  4. Presbycusis: a human temporal bone study of individuals with downward sloping audiometric patterns of hearing loss and review of the literature.

    Science.gov (United States)

    Nelson, Erik G; Hinojosa, Raul

    2006-09-01

    The purpose of this retrospective case review was to identify patterns of cochlear element degeneration in individuals with presbycusis exhibiting downward sloping audiometric patterns of hearing loss and to correlate these findings with those reported in the literature to clarify conflicting concepts regarding the association between hearing loss and morphologic abnormalities. Archival human temporal bones from individuals with presbycusis were selected on the basis of strict audiometric criteria for downward-sloping audiometric thresholds. Twenty-one temporal bones that met these criteria were identified and compared with 10 temporal bones from individuals with normal hearing. The stria vascularis volumes, spiral ganglion cell populations, inner hair cells, and outer hair cells were quantitatively evaluated. The relationship between the severity of hearing loss and the degeneration of cochlear elements was analyzed using univariate linear regression models. Outer hair cell loss and ganglion cell loss was observed in all individuals with presbycusis. Inner hair cell loss was observed in 18 of the 21 individuals with presbycusis and stria vascularis loss was observed in 10 of the 21 individuals with presbycusis. The extent of degeneration of all four of the cochlear elements evaluated was highly associated with the severity of hearing loss based on audiometric thresholds at 8,000 Hz and the pure-tone average at 500, 1,000, and 2,000 Hz. The extent of ganglion cell degeneration was associated with the slope of the audiogram. Individuals with downward-sloping audiometric patterns of presbycusis exhibit degeneration of the stria vascularis, spiral ganglion cells, inner hair cells, and outer hair cells that is associated with the severity of hearing loss. This association has not been previously reported in studies that did not use quantitative methodologies for evaluating the cochlear elements and strict audiometric criteria for selecting cases.

  5. RNA analysis of inner ear cells from formalin fixed paraffin embedded (FFPE) archival human temporal bone section using laser microdissection--a technical report.

    Science.gov (United States)

    Kimura, Yurika; Kubo, Sachiho; Koda, Hiroko; Shigemoto, Kazuhiro; Sawabe, Motoji; Kitamura, Ken

    2013-08-01

    Molecular analysis using archival human inner ear specimens is challenging because of the anatomical complexity, long-term fixation, and decalcification. However, this method may provide great benefit for elucidation of otological diseases. Here, we extracted mRNA for RT-PCR from tissues dissected from archival FFPE human inner ears by laser microdissection. Three human temporal bones obtained at autopsy were fixed in formalin, decalcified by EDTA, and embedded in paraffin. The samples were isolated into spiral ligaments, outer hair cells, spiral ganglion cells, and stria vascularis by laser microdissection. RNA was extracted and heat-treated in 10 mM citrate buffer to remove the formalin-derived modification. To identify the sites where COCH and SLC26A5 mRNA were expressed, semi-nested RT-PCR was performed. We also examined how long COCH mRNA could be amplified by semi-nested RT-PCR in archival temporal bone. COCH was expressed in the spiral ligament and stria vascularis. However, SLC26A5 was expressed only in outer hair cells. The maximum base length of COCH mRNA amplified by RT-PCR was 98 bp in 1 case and 123 bp in 2 cases. We detected COCH and SLC26A5 mRNA in specific structures and cells of the inner ear from archival human temporal bone. Our innovative method using laser microdissection and semi-nested RT-PCR should advance future RNA study of human inner ear diseases. Copyright © 2013 Elsevier B.V. All rights reserved.

  6. 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.

  7. Right-to-left-shunt detected by c-TCD using the orbital window in comparison with temporal bone windows.

    Science.gov (United States)

    Kobayashi, Kazuto; Kimura, Kazumi; Iguchi, Yasuyuki; Sakai, Kenichirou; Aoki, Junya; Iwanaga, Takeshi; Shibazaki, Kensaku

    2012-01-01

    There have been some reports on right-to-left shunt as a cause of cryptogenic stroke. Although contrast transcranial Doppler (c-TCD) can detect RLS, an insufficient temporal window has occasionally restricted its applicability. Thus, we compared the rates of detecting RLS among temporal windows for the middle cerebral arteries (MCAs) and the orbital window for the internal carotid artery (ICA) on c-TCD. We used c-TCD to detect RLS in patients with suspected ischemic stroke. We enrolled patients who had both sufficient bilateral temporal windows for MCAs and a right orbital window for ICA and performed c-TCD using all three windows simultaneously. We enrolled 106 consecutive patients and identified microembolic signals (MES) in 30 (28%) of them. Among these 30 patients, 15 had MES from all 3 windows. When these 30 patients were defined as being positive for RLS, the rates of detection were 67%, 73%, and 80% from the right temporal, left temporal, and right orbital windows, respectively (P= .795). The right orbital window as well as the temporal window for c-TCD could detect RLS. Insonation from the orbital window should be useful for patients who lack temporal windows. Copyright © 2010 by the American Society of Neuroimaging.

  8. 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.

  9. 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.

  10. Insertion characteristics and placement of the Mid-Scala electrode array in human temporal bones using detailed cone beam computed tomography.

    Science.gov (United States)

    Dietz, Aarno; Gazibegovic, Dzemal; Tervaniemi, Jyrki; Vartiainen, Veli-Matti; Löppönen, Heikki

    2016-12-01

    The aim of this study was to evaluate the insertion results and placement of the new Advanced Bionics HiFocus Mid-Scala (HFms) electrode array, inserted through the round window membrane, in eight fresh human temporal bones using cone beam computed tomography (CBCT). Pre- and post-insertion CBCT scans were registered to create a 3D reconstruction of the cochlea with the array inserted. With an image fusion technique both the bony edges of the cochlea and the electrode array in situ could accurately be determined, thus enabling to identify the exact position of the electrode array within the scala tympani. Vertical and horizontal scalar location was measured at four points along the cochlea base at an angular insertion depth of 90°, 180° and 270° and at electrode 16, the most basal electrode. Smooth insertion through the round window membrane was possible in all temporal bones. The imaging results showed that there were no dislocations from the scala tympani into the scala vestibule. The HFms electrode was positioned in the middle of the scala along the whole electrode array in three out of the eight bones and in 62 % of the individual locations measured along the base of the cochlea. In only one cochlea a close proximity of the electrode with the basilar membrane was observed, indicating possible contact with the basilar membrane. The results and assessments presented in this study appear to be highly accurate. Although a further validation including histopathology is needed, the image fusion technique described in this study represents currently the most accurate method for intracochlear electrode assessment obtainable with CBCT.

  11. 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.

  12. The temporal course of mucoperiosteal flap revascularization at guided bone regeneration treated implant sites: a pilot study

    NARCIS (Netherlands)

    Milstein, D.M.J.; Mathura, K.R.; Lindeboom, J.A.H.; Ramsoekh, D.; Lindeboom, R.; Ince, C.

    2009-01-01

    Aims: To investigate post-operative capillary density regeneration in healing mucoperiosteal flaps at guided bone regeneration-treated implant sites. Material and Methods: A non-invasive post-operative investigation was performed in 10 patients using orthogonal polarization spectral (OPS) imaging

  13. The temporal course of mucoperiosteal flap revascularization at guided bone regeneration-treated implant sites: a pilot study

    NARCIS (Netherlands)

    Milstein, Dan M. J.; Mathura, Keshen R.; Lindeboom, Jerôme A. H.; Ramsoekh, Dewkoemar; Lindeboom, Robert; Ince, Can

    2009-01-01

    P>Aims To investigate post-operative capillary density regeneration in healing mucoperiosteal flaps at guided bone regeneration-treated implant sites. Material and Methods A non-invasive post-operative investigation was performed in 10 patients using orthogonal polarization spectral (OPS) imaging

  14. Genetics Home Reference: congenital bile acid synthesis defect type 1

    Science.gov (United States)

    ... result in softening and weakening of the bones ( rickets ) in some individuals. If left untreated, congenital bile ... Encyclopedia: Cholestasis Health Topic: Liver Diseases Health Topic: Rickets Genetic and Rare Diseases Information Center (1 link) ...

  15. Mutations in the thrombopoietin receptor, Mpl, in children with congenital amegakaryocytic thrombocytopenia

    NARCIS (Netherlands)

    van den Oudenrijn, S.; Bruin, M.; Folman, C. C.; Peters, M.; Faulkner, L. B.; de Haas, M.; von dem Borne, A. E.

    2000-01-01

    Congenital amegakaryocytic thrombocytopenia (CAMT) is a rare disorder of undefined aetiology. The disease presents with severe thrombocytopenia and absence of megakaryocytes in the bone marrow. Furthermore, CAMT patients may develop bone marrow aplasia. To obtain more insight into the mechanism

  16. Trauma dos ossos temporais e suas complicações: aspectos na tomografia computadorizada Temporal bone trauma and complications: computed tomography findings

    Directory of Open Access Journals (Sweden)

    Ana Maria Doffémond Costa

    2013-04-01

    Full Text Available A maioria das fraturas dos ossos temporais resulta de traumas cranianos bruscos, de alta energia, estando muitas vezes relacionadas a outras fraturas cranianas ou a politraumatismo. As fraturas e os deslocamentos da cadeia ossicular, na orelha média, representam umas das principais complicações das injúrias nos ossos temporais e, por isso, serão abordadas de maneira mais profunda neste artigo. Os outros tipos de injúrias englobam as fraturas labirínticas, fístula dural, paralisia facial e extensão da linha de fratura ao canal carotídeo. A tomografia computadorizada tem papel fundamental na avaliação inicial dos pacientes politraumatizados, pois é capaz de identificar injúrias em importantes estruturas que podem causar graves complicações, como perda auditiva de condução ou neurossensorial, tonturas e disfunções do equilíbrio, fístulas perilinfáticas, paralisia do nervo facial, lesões vasculares, entre outras.Most temporal bone fractures result from high-energy blunt head trauma, and are frequently related to other skull fractures or to polytrauma. Fractures and displacements of ossicular chain in the middle ear represent some of the main complications of temporal bone injury, and hence they will be more deeply approached in the present article. Other types of injuries include labyrinthine fractures, dural fistula, facial nerve paralysis and extension into the carotid canal. Computed tomography plays a fundamental role in the initial evaluation of polytrauma patients, as it can help to identify important structural injuries that may lead to severe complications such as sensorineural hearing loss, conductive hearing loss, dizziness and balance dysfunction, perilymphatic fistulas, facial nerve paralysis, vascular injury and others.

  17. Development of Composite Scaffolds for Load Bearing Segmental Bone Defects

    Science.gov (United States)

    2013-07-01

    osteoarthritis [24], generic infections, congenital deformity corrections [25], pathological degenerative bone destruction and other degenerative diseases [20...resection and reconstruction), osteoporosis, osteoarthritis , generic infections, congenital deformity corrections, pathological degenerative bone...the construct. Pore size/ Porosity: Quality of a bone scaffold to have pore size and porosity percent similar to established guidelines . Ideal

  18. Bone X-Ray (Radiography)

    Medline Plus

    Full Text Available ... taken of the unaffected limb, or of a child's growth plate (where new bone is forming), for ... around joints, and in evaluating the hips of children with congenital problems. top of page This page ...

  19. Four novel ELANE mutations in patients with congenital neutropenia.

    Science.gov (United States)

    Kurnikova, Maria; Maschan, Michael; Dinova, Evgeniya; Shagina, Irina; Finogenova, Natalia; Mamedova, Elena; Polovtseva, Tatyana; Shagin, Dmitry; Shcherbina, Anna

    2011-08-01

    Congenital neutropenia is a heterogeneous bone marrow failure syndrome characterized by a maturation arrest of myelopoesis at the promyelocyte/myelocyte stage. Cyclic neutropenia (CyN) and severe congenital neutropenia (SCN) are two main forms of congenital neutropenia. Genetic analysis has shown that heterozygous mutations in the ELANE gene encoding the neutrophil elastase are the major cause of these disorders. We investigated the prevalence of ELANE mutations in a group of 16 patients from 14 families with congenital neutropenia. Five patients had typical manifestations of CyN, and 11 patients had SCN. Seven different heterozygous ELANE mutations were found, including four novel mutations. Copyright © 2011 Wiley-Liss, Inc.

  20. Congenital rickets presenting as refractory respiratory distress at birth.

    Science.gov (United States)

    Tiwari, Soumya; Kumar, Rajesh; Singla, Shilpy; Dudeja, Ajay; Nangia, Sushma; Saili, Arvind

    2014-08-01

    Congenital rickets is a very rare entity in the spectrum of metabolic bone disease in children. The authors report an as yet unreported case of congenital rickets presenting with respiratory distress at birth. The radiographs of long bones and wrist showed generalized osteopenia with cupping and fraying of epiphyseal ends in the second week of life. The patient was managed with very high doses of vitamin D which led to clinico-radiological and biochemical improvement. More than being interesting for its extreme rarity, this report assumes importance as it brings forth the possibility of congenital rickets being a differential diagnosis for a newborn with respiratory distress.

  1. Congenital syphilitic hepatitis: a radionuclide study

    International Nuclear Information System (INIS)

    Gates, G.F.; Stanley, P.; Gwinn, J.L.; Miller, J.H.

    1978-01-01

    A two-month-old girl with congenital syphilitic hepatitis had bizarre liver scintigraphic features showing diminished hepatic uptake of a radiocolloid with accentuated pulmonary and bone marrow accumulation. These features were reversible following penicillin therapy and to our knowledge are previously undescribed manifestations of this multisystemic disease

  2. [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.

  3. A comparison of high resolution CT scan of temporal bone and operative findings in middle ear cholesteatoma

    Energy Technology Data Exchange (ETDEWEB)

    Kweon, Tae Beom; Seong, Hun; Cheon, Mal Soon; Kim, Hack Jin; Jang, Keung Jae; Chun, Byung Hee [Dae Dong General Hospital, Busan (Korea, Republic of)

    1993-09-15

    To evaluate the value of HRCT imaging in middle ear cholesteatoma, we prospectively analysed the CT images in 28 surgically proven cases with cholesteatomas regarding main site of lesion, ossicular change, facial nerve exposure and fistula formation. The most common main site of lesion was the epitympanum (92.8%). The results of sensitivity, positive predictability, and accuracy by CT imagings were as follows: for ossicular involvement, 94.1%, 88.8%, and 86.2% in malleus, 96.0%, 88.8%, and 85.7% in incus, 81.2%, 81.2%, and 78.5% in stapes; for facial nerve exposure, 66.6%, 57.1%, and 81.2%: for fistula formation, 100%, 75.0%, and 96.4%, respectively. In conclusion, the temoral bone HRCT imaging is an accurate preoperative method in detecting main lesion site, ossicular involvement, fistula formation. Because of the low sensitivity and positive predictability in detecting facial nerve exposure, it is necessary to correlate the HRCT images with the clinical status.

  4. Bone grafting: An overview

    Directory of Open Access Journals (Sweden)

    D. O. Joshi

    2010-08-01

    Full Text Available Bone grafting is the process by which bone is transferred from a source (donor to site (recipient. Due to trauma from accidents by speedy vehicles, falling down from height or gunshot injury particularly in human being, acquired or developmental diseases like rickets, congenital defects like abnormal bone development, wearing out because of age and overuse; lead to bone loss and to replace the loss we need the bone grafting. Osteogenesis, osteoinduction, osteoconduction, mechanical supports are the four basic mechanisms of bone graft. Bone graft can be harvested from the iliac crest, proximal tibia, proximal humerus, proximal femur, ribs and sternum. An ideal bone graft material is biologically inert, source of osteogenic, act as a mechanical support, readily available, easily adaptable in terms of size, shape, length and replaced by the host bone. Except blood, bone is grafted with greater frequency. Bone graft indicated for variety of orthopedic abnormalities, comminuted fractures, delayed unions, non-unions, arthrodesis and osteomyelitis. Bone graft can be harvested from the iliac crest, proximal tibia, proximal humerus, proximal femur, ribs and sternum. By adopting different procedure of graft preservation its antigenicity can be minimized. The concept of bone banking for obtaining bone grafts and implants is very useful for clinical application. Absolute stability require for successful incorporation. Ideal bone graft must possess osteogenic, osteoinductive and osteocon-ductive properties. Cancellous bone graft is superior to cortical bone graft. Usually autologous cancellous bone graft are used as fresh grafts where as allografts are employed as an alloimplant. None of the available type of bone grafts possesses all these properties therefore, a single type of graft cannot be recomm-ended for all types of orthopedic abnormalities. Bone grafts and implants can be selected as per clinical problems, the equipments available and preference of

  5. 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 ...

  6. 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 © ...

  7. 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 ...

  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. Comparison of capacity for diagnosis and visuality of auditory ossicles at different scanning angles in the computed tomography of temporal bone

    International Nuclear Information System (INIS)

    Ogura, Akio; Nakayama, Yoshiki

    1992-01-01

    Computed tomographic (CT) scanning has made significant contributions to the diagnosis and evaluation of temporal bone lesions by the thin-section, high-resolution techniques. However, these techniques involve greater radiation exposure to the lens of patients. A mean was thus sought for reducing the radiation exposure at different scanning angles such as +15 degrees and -10 degrees to the Reid's base line. Purposes of this study were to measure radiation exposure to the lens using the two tomographic planes and to compare the ability to visualize auditory ossicles and labyrinthine structures. Visual evaluation of tomographic images on auditory ossicles was made by blinded methods using four rankings by six radiologists. The statistical significance of the intergroup difference in the visualization of tomographic planes was assessed for a significance level of 0.01. Thermoluminescent dosimeter chips were placed on the cornea of tissue equivalent to the skull phantom to evaluate radiation exposure for two separate tomographic planes. As the result, tomographic plane at an angle of -10 degrees to Reid's base line allowed better visualization than the other plane for the malleus, incus, facial nerve canal, and tuba auditiva (p<0.01). Scannings at an angle of -10 degrees to Reid's base line reduced radiation exposure to approximately one-fiftieth (1/50) that with the scans at the other angle. (author)

  10. The prevalence of superior semicircular canal dehiscence in conductive and mixed hearing loss in the absence of other pathology using submillimetric temporal bone computed tomography.

    Science.gov (United States)

    Lee, Young Hen; Rivas-Rodriguez, Francisco; Song, Jae-Jun; Yang, Kyung-Sook; Mukherji, Suresh K

    2014-01-01

    The objective of this study was to assess the relationship between superior semicircular canal dehiscence (SSCD) and hearing impairment. We retrospectively compared the prevalence of SSCD in the ears classified as conductive hearing loss (CHL), mixed hearing loss (MHL), and normal hearing status using submillimetric temporal bone computed tomography (TBCT) on the basis of coronal and additional reformatted planes dedicated to SSCD. From the patients with CHL (n = 127) and MHL (n = 45), the overall prevalence of SSCD in the ears classified as CHL, MHL, and normal hearing status were 6.6%, 7.2%, and 3.0%, respectively. Furthermore, the odds ratio for SSCD in the absence of any cause of hearing loss (eg, dysfunction of the tympanic membrane or middle ear, TBCT abnormalities, otosclerosis, trauma, surgery) was 5.35 in MHL (4/27; P = 0.037, 95% confidence interval, 1.1-25.81) and 3.31 in CHL (5/61; P = 0.115, 95% confidence interval, 0.75-14.63), compared with normal hearing status. Bony covering of the SSC should be specifically evaluated in patients with hearing impairment using submillimetric TBCT.

  11. 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

  12. Congenital Amegakaryocytic Thrombocytopenic Purpura (CAMT)

    International Nuclear Information System (INIS)

    Ghauri, R. I.; Naveed, M.; Mannan, J.

    2014-01-01

    Congenital amegakaryocytic thrombocytopenia (CAMT) is a rare, autosomal recessive disorder induced by mutations of the gene coding for thrombopoietin (TPO) receptor (c-MPL) despite high levels of serum TPO. Patients initially present with isolated thrombocytopenia that subsequently progresses into pancytopenia. Although the mechanisms leading to aplasia are unknown, the age of onset has been reported to depend on the severity of the c-MPL functional defect. The primary treatment for CAMT is bone marrow transplantation. This report describes a newborn girl who presented to us with symptoms of sepsis but septic profile came negative except thrombocytopenia. Bone marrow biopsy was done for thrombocytopenia which revealed amegakaryocytic thrombocytopenia. She was given prednisolone. (author)

  13. Skeletal changes in congenital fibrinogen abnormalities

    Energy Technology Data Exchange (ETDEWEB)

    Lagier, R.; Bouvier, C.A.; van Strijthem, N.

    1980-01-01

    We report anatomico-radiologic study of humerus, femur, and tibia from a case of total congenital afibrinogenemia. Juxtatrabecular hemorrhages occur mainly in metaphyses and seem to be related to normal lines of stress. They may lead to the formation of intraosseous cysts and to a remodelling of bone trabeculae. The radiologic lesions in a second case, diagnosed as congenital dysfibrinogenemia, are similar to those found in Case 1 (femoral trabeculae remodelling) but also resemble some alterations described in hemophilia (pseudotumor of the right iliac bone). Anatomic study of the lesions in Case 2 was not possible. The significance of these observations could be better defined by a more extended skeletal study (radiologic and when feasible anatomic) of patients with congenital clotting defects and especially with inherited disorders of the fibrinogen molecule. It would also be worthwhile investigating manifest or latent hemostatic disorders (particularly at the fibrinogen level) in patients with solitary or aneurysmal bone cysts, and even with bone infarct or unexplained trabecular remodelling.

  14. Osteodistrofias do Osso Temporal: Revisão dos Conceitos Atuais, Manifestações Clínicas e Opções Terapêuticas Osteodysplasia of the Temporal Bone: Up-date Concepts, Clinical Presentations and Therapeutic Options

    Directory of Open Access Journals (Sweden)

    Oswaldo Laércio M. Cruz

    Full Text Available Sob a designação de osteodistrofias do osso temporal, podemos encontrar uma série de doenças que apresentam em comum a desorganização da arquitetura ou da composição do tecido ósseo. A otospongiose é, com larga margem, a osteodistrofia mais comum nessa localização e suas alterações, repercussões clínicas e tratamentos são amplamente discutidos na literatura. Entretanto, formas menos freqüentes, como a displasia fibrosa e a osteogênese imperfeita, não são entidades raras e merecem atenção. Este artigo tem como objetivo discutir essas formas menos comuns de osteodistrofia do temporal através de uma revisão sobre os conceitos atuais dessas entidades, da apresentação de três exemplos clínicos e a discussão sobre opções de tratamento.Osteodysplasia of the temporal bone included a significant amount of osseous diseases sharing bone matrix structural and composition damage. Otospongiosis is, by far, the most frequent form of this involvement in the temporal bone. Nevertheless, fibrous dysplasia and osteogenesis imperfecta are not rare and deserve attention. In this article, the authors present a discussion about the recent concepts of those less frequent forms of osteodysplasia of temporal bone, its options of treatment, illustrated with three clinical cases.

  15. On exposure to anorexia nervosa, the temporal variation in axial and appendicular skeletal development predisposes to site-specific deficits in bone size and density: a cross-sectional study.

    Science.gov (United States)

    Seeman, E; Karlsson, M K; Duan, Y

    2000-11-01

    Skeletal development is heterogeneous. Throughout growth, bone size is more maturationally advanced than the mineral being accrued within its periosteal envelope; before puberty, appendicular growth is more rapid than axial growth; during puberty, appendicular growth slows and axial growth accelerates. We studied women with differing age of onset of anorexia nervosa to determine whether this temporal heterogeneity in growth predisposed to the development of deficits in bone size and volumetric bone mineral density (vBMD), which varied by site and severity depending on the age at which anorexia nervosa occurred. Bone size and vBMD of the third lumbar vertebra and femoral neck were measured using dual-energy X-ray absorptiometry in 210 women aged 21 years (range, 12-40 years) with anorexia nervosa. Results were expressed as age-specific SDs (mean +/- SEM). Bone width depended on the age of onset of anorexia nervosa; when the onset of anorexia nervosa occurred (1) before 15 years of age, deficits in vertebral body and femoral neck width did not differ (-0.77+/-0.27 SD and -0.55+/-0.17 SD, respectively); (2) between 15 and 19 years of age, deficits in vertebral body width (-0.95+/-0.16 SD) were three times the deficits in femoral neck width (-0.28+/-0.14 SD; p anorexia nervosa. No deficit in bone width was observed at the femoral neck. Deficits in vBMD at the vertebra and femoral neck were independent of the age of onset of anorexia nervosa but increased as the duration of anorexia nervosa increased, being about 0.5 SD lower at the vertebra than femoral neck. We infer that the maturational development of a region at the time of exposure to disease, and disease duration, determine the site, magnitude, and type of trait deficit in anorexia nervosa. Bone fragility due to reduced bone size and reduced vBMD in adulthood is partly established during growth.

  16. Temporal bone encephalocele and cerebrospinal fluid fistula repair utilizing the middle cranial fossa or combined mastoid-middle cranial fossa approach.

    Science.gov (United States)

    Carlson, Matthew L; Copeland, William R; Driscoll, Colin L; Link, Michael J; Haynes, David S; Thompson, Reid C; Weaver, Kyle D; Wanna, George B

    2013-11-01

    The goals of this study were to report the clinical presentation, radiographic findings, operative strategy, and outcomes among patients with temporal bone encephaloceles and cerebrospinal fluid fistulas (CSFFs) and to identify clinical variables associated with surgical outcome. A retrospective case series including all patients who underwent a middle fossa craniotomy or combined mastoid-middle cranial fossa repair of encephalocele and/or CSFF between 2000 and 2012 was accrued from 2 tertiary academic referral centers. Eighty-nine consecutive surgeries (86 patients, 59.3% women) were included. The mean age at time of surgery was 52.3 years, and the left side was affected in 53.9% of cases. The mean delay between symptom onset and diagnosis was 35.4 months, and the most common presenting symptoms were hearing loss (92.1%) and persistent ipsilateral otorrhea (73.0%). Few reported a history of intracranial infection (6.7%) or seizures (2.2%). Thirteen (14.6%) of 89 cases had a history of major head trauma, 23 (25.8%) were associated with chronic ear disease without prior operation, 17 (19.1%) occurred following tympanomastoidectomy, and 1 (1.1%) developed in a patient with a cerebral aqueduct cyst resulting in obstructive hydrocephalus. The remaining 35 cases (39.3%) were considered spontaneous. Among all patients, the mean body mass index (BMI) was 35.3 kg/m(2), and 46.4% exhibited empty sella syndrome. Patients with spontaneous lesions were statistically significantly older (p = 0.007) and were more commonly female (p = 0.048) compared with those with nonspontaneous pathology. Additionally, those with spontaneous lesions had a greater BMI than those with nonspontaneous disease (p = 0.102), although this difference did not achieve statistical significance. Thirty-two surgeries (36.0%) involved a middle fossa craniotomy alone, whereas 57 (64.0%) involved a combined mastoid-middle fossa repair. There were 7 recurrences (7.9%); 2 patients with recurrence developed

  17. Temporal growth factor release from platelet-rich plasma, trehalose lyophilized platelets, and bone marrow aspirate and their effect on tendon and ligament gene expression.

    Science.gov (United States)

    McCarrel, Taralyn; Fortier, Lisa

    2009-08-01

    Platelet-rich plasma (PRP) has generated substantial interest for tendon and ligament regeneration because of the high concentrations of growth factors in platelet alpha-granules. This study compared the temporal release of growth factors from bone marrow aspirate (BMA), PRP, and lyophilized platelet product (PP), and measured their effects on tendon and ligament gene expression. Blood and BMA were collected and processed to yield PRP and plasma. Flexor digitorum superficialis tendon (FDS) and suspensory ligament (SL) explants were cultured in 10% plasma in DMEM (control), BMA, PRP, or PP. TGF-beta1 and PDGF-BB concentrations were determined at 0, 24, and 96 h of culture using ELISA. Quantitative RT-PCR for collagen types I and III (COL1A1, COL3A1), cartilage oligomeric matrix protein (COMP), decorin, and matrix metalloproteinases-3 and 13 (MMP-3, MMP-13) was performed. TGF-beta1 and PDGF-BB concentrations were highest in PRP and PP. Growth factor quantity was unchanged in BMA, increased in PRP, and decreased in PP over 4 days. TGF-beta1 and platelet concentrations were positively correlated. Lyophilized PP and PRP resulted in increased COL1A1:COL3A1 ratio, increased COMP, and decreased MMP-13 expression. BMA resulted in decreased COMP and increased MMP-3 and MMP-13 gene expression. Platelet concentration was positively correlated with COL1A1, ratio of COL1A1:COL3A1, and COMP, and negatively correlated with COL3A1, MMP-13, and MMP-3. White blood cell concentration was positively correlated with COL3A1, MMP3, and MMP13, and negatively correlated with a ratio of COL1A1:COL3A1, COMP, and decorin. These findings support further in vivo investigation of PRP and PP for treatment of tendonitis and desmitis. Copyright 2009 Orthopaedic Research Society. Published by Wiley Periodicals, Inc.

  18. Congenital chondromyxoid fibroma of the ethmoid: case report

    International Nuclear Information System (INIS)

    Mendoza, M.; Gonzalez, I.; Aperribay, M.; Nogues, A.; Hermosa, J.R.

    1998-01-01

    This report describes a congenital case of chondromyxoid fibroma (CMF) arising from the ethmoid bone. We believe it to be the second case of congenital CMF that has been documented, and the third case of CMF arising in the ethmoid. We describe the radiographic features of this rare entity and indicate the necessity for careful correlation between radiographic and histological findings to distinguish CMF from chondrosarcoma. (orig.)

  19. Congenital anophthalmia: current concepts in management.

    Science.gov (United States)

    Quaranta-Leoni, Francesco M

    2011-09-01

    The introduction of hydrogel socket and orbital expanders has modified the approach towards the rehabilitation of congenital anophthalmia. This study highlights the most recent advances for the treatment of congenital anophthalmia based on personal experience and the review of recent literature. Hydrogel socket expanders may be positioned as an out-patient procedure with topical anaesthesia, using cyanoacrylate glue as opposed to temporary tarsorraphy. Increased orbital volume has been confirmed by computed tomography (CT) scan or magnetic resonance imaging (MRI) following early dermis-fat graft in children with congenital anophthalmia. An orbital tissue expander made of an inflatable silicone globe sliding on a titanium T-plate and secured to the lateral orbital rim appears to be effective to stimulate orbital bone growth and development. Congenital anophthalmia has a complex cause with both genetic and environmental factors involved. The ideal treatment is simultaneous expansion of the eyelids, socket and orbital bones, and it should begin after birth as soon as possible. Socket expansion with self-inflating expanders is a useful technique, although custom-made conformers may produce similar results. Dermis-fat grafts are another reasonable option as an orbital implant, following adequate lid and socket expansion.

  20. 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

  1. Spontaneous subconjunctival abscess in congenital lamellar ichthyosis

    Directory of Open Access Journals (Sweden)

    Shivanand C Bubanale

    2018-01-01

    Full Text Available Congenital lamellar ichthyosis is an autosomal recessive, heterogeneous disorder presenting at birth with generalized skin involvement. The most common ophthalmic manifestation noted is bilateral ectropion of the lower eyelids. A 1-month-old female neonate, the second born of a nonconsanguineous marriage, presented with 4 days' history of redness, discharge, and swelling in the right eye. There was severe right upper eyelid ectropion, conjunctival injection, chemosis, a subconjunctival mass on the temporal bulbar conjunctiva spontaneously draining pus and corneal haze. The anterior chamber, iris, lens and fundus appeared normal. Congenital lamellar ichthyosis was suspected because of scaling and excessive dryness of the entire body. The occurrence of a spontaneous subconjunctival abscess is not known in lamellar ichthyosis. We thus report the management of a rare case of unilateral upper eyelid ectropion, subconjunctival abscess with orbital cellulitis in congenital lamellar ichthyosis.

  2. 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...

  3. 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.

  4. 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 ...

  5. 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.

  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. Bone tumor

    Science.gov (United States)

    Tumor - bone; Bone cancer; Primary bone tumor; Secondary bone tumor; Bone tumor - benign ... The cause of bone tumors is unknown. They often occur in areas of the bone that grow rapidly. Possible causes include: Genetic defects ...

  9. Application of in vivo micro-computed tomography in the temporal characterisation of subchondral bone architecture in a rat model of low-dose monosodium iodoacetate-induced osteoarthritis

    Science.gov (United States)

    2011-01-01

    Introduction Osteoarthritis (OA) is a complex, multifactorial joint disease affecting both the cartilage and the subchondral bone. Animal models of OA aid in the understanding of the pathogenesis of OA and testing suitable drugs for OA treatment. In this study we characterized the temporal changes in the tibial subchondral bone architecture in a rat model of low-dose monosodium iodoacetate (MIA)-induced OA using in vivo micro-computed tomography (CT). Methods Male Wistar rats received a single intra-articular injection of low-dose MIA (0.2 mg) in the right knee joint and sterile saline in the left knee joint. The animals were scanned in vivo by micro-CT at two, six, and ten weeks post-injection, analogous to early, intermediate, and advanced stages of OA, to assess architectural changes in the tibial subchondral bone. The articular cartilage changes in the tibiae were assessed macroscopically and histologically at ten weeks post-injection. Results Interestingly, tibiae of the MIA-injected knees showed significant bone loss at two weeks, followed by increased trabecular thickness and separation at six and ten weeks. The trabecular number was decreased at all time points compared to control tibiae. The tibial subchondral plate thickness of the MIA-injected knee was increased at two and six weeks and the plate porosity was increased at all time points compared to control. At ten weeks, histology revealed loss of proteoglycans, chondrocyte necrosis, chondrocyte clusters, cartilage fibrillation, and delamination in the MIA-injected tibiae, whereas the control tibiae showed no changes. Micro-CT images and histology showed the presence of subchondral bone sclerosis, cysts, and osteophytes. Conclusions These findings demonstrate that the low-dose MIA rat model closely mimics the pathological features of progressive human OA. The low-dose MIA rat model is therefore suitable to study the effect of therapeutic drugs on cartilage and bone in a non-trauma model of OA. In vivo

  10. Congenitally transmitted visceral leishmaniasis: report of two brazilian human cases

    Directory of Open Access Journals (Sweden)

    Myrlena Regina Machado Mescouto-Borges

    2013-04-01

    Full Text Available Visceral leishmaniasis is a relevant public health problem worldwide. Most of the reported cases in Latin America are from Brazil. Herein we report two human cases of congenitally transmitted visceral leishmaniasis in two patients who developed symptoms during pregnancy. The diagnosis was made by visual examination of Leishmania parasites in bone marrow aspirates of the mothers and by detecting parasite kDNA in bone marrow samples of the newborn children using polymerase chain reaction.

  11. 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....

  12. 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.

  13. 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.

  14. 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.

  15. 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

  16. The facial nerve in the temporal bone as visualised via thin-layer paratransversal and sagittal MR tomographic images by means of T1 spin-echo and FLASH sequences

    International Nuclear Information System (INIS)

    Mueller-Lisse, U.; Jaeger, L.J.E.; Bruegel, F.J.; Grevers, G.; Reiser, M.F.

    1995-01-01

    It is difficult to effect visualization and delineation of the facial nerve and its neighbouring structures in the temporal bone with conventional MRI examination protocols. We tested temporal bone MRI with 2 mm slices and compared T 1 -weighted FLASH (T R =400 ms, T E =10 ms, 90 flip angle) and spin-echo (T R =540 ms, T E =15 ms) sequences. 5 volunteers and 14 patients were examined with the head coil of a 1.0 T whole body MRI scanner (Impact, Siemens, Erlangen) with para-transversal images orientated parallel to the inferior outline of the clivus and sagittal images orientated along the brainstem. The facial nerve and its neighbouring structures could be reliably visualized and differentiated along its entire course. The FLASH sequence was superior to the spin-echo sequence. 8 of 11 patients with peripheral facial nerve palsy showed contrast enhancement. In two patients, local swelling of the affected facial nerve was evident. (orig./MG) [de

  17. 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)

  18. 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.

  19. 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)

  20. Open-type congenital cholesteatoma: differential diagnosis for conductive hearing loss with a normal tympanic membrane.

    Science.gov (United States)

    Kim, Se-Hyung; Cho, Yang-Sun; Chu, Ho-Suk; Jang, Jeon-Yeob; Chung, Won-Ho; Hong, Sung Hwa

    2012-06-01

    In patients with progressive conductive hearing loss and a normal tympanic membrane (TM), and with soft tissue density in the middle ear cavity (MEC) on temporal bone computed tomography (TBCT) scan, open-type congenital cholesteatoma (OCC) should be highly suspected and a proper surgical plan that includes mastoid exploration and second-stage operation is required. The clinical presentation of OCC is very similar to congenital ossicular anomaly (COA) presenting with a conductive hearing loss with intact TM. Therefore, it is challenging to make a correct preoperative diagnosis in patients with OCC. We evaluated the clinical characteristics of OCC compared with those of COA to find diagnostic clues useful in diagnosis of OCC. The medical records of 12 patients with surgically proven OCC and 14 patients with surgically proven COA were reviewed for demographic data, otologic history, preoperative TBCT findings, intraoperative findings, and pre- and postoperative audiologic data. There was no difference between OCC and COA based on demographic data, preoperative hearing, and ossicular status on TBCT. However, the presence of progressive hearing loss, soft tissue density in the MEC on TBCT scan, and the need for mastoid surgery and second-stage operation were significantly more frequent in OCC patients.

  1. Role of CT in Congenital Heart Disease.

    Science.gov (United States)

    Rajiah, Prabhakar; Saboo, Sachin S; Abbara, Suhny

    2017-01-01

    Congenital heart diseases (CHD) are being increasingly encountered in cardiac imaging due to improved outcomes from surgical and interventional techniques. Imaging plays an important role in the evaluation of CHD, both prior to and after surgeries and interventions. Computed tomography (CT) has several advantages in the evaluation of these disorders, particularly its high spatial resolution, multi-planar reconstruction capabilities at sub-millimeter isotropic resolution, good temporal resolution, wide field of view, and rapid turnaround time, which minimizes the need for sedation and anesthesia in young children or children with disabilities. With modern scanners, images can be acquired as fast as within one heartbeat. Although there is a risk of ionizing radiation, the radiation dose can be minimized by using several dose reduction strategies. There is a risk of contrast nephrotoxicity in patients with renal dysfunction. In this article, we will review the role of CT in the evaluation of several congenital heart diseases, both in children and adults.

  2. 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.)

  3. 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

  4. 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.

  5. 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.

  6. Familial severe congenital neutropenia associated with infantile osteoporosis: a new entity.

    Science.gov (United States)

    Elhasid, R; Hofbauer, L C; Ish-Shalom, S; Ben-Arush, M; Koc, O; Rowe, J M; Etzioni, A

    2003-01-01

    A new entity manifested by severe congenital neutropenia associated with osteoporosis and recurrent bone fracture is described in a family. A possible role for a new recognized cytokine system involved in bone remodeling, the osteoprotegerin/receptor activator of nuclear factor-kappa B ligand, is suggested. Copyright 2002 Wiley-Liss, Inc.

  7. 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

  8. 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....

  9. 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.

  10. 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.

  11. Bone tumors

    International Nuclear Information System (INIS)

    Unni, K.K.

    1988-01-01

    This book contains the proceedings on bone tumors. Topics covered include: Bone tumor imaging: Contribution of CT and MRI, staging of bone tumors, perind cell tumors of bone, and metastatic bone disease

  12. A dominant mutation in the gene encoding the erythroid transcription factor KLF1 causes a congenital dyserythropoietic anemia

    DEFF Research Database (Denmark)

    Arnaud, Lionel; Saison, Carole; Helias, Virginie

    2010-01-01

    The congenital dyserythropoietic anemias (CDAs) are inherited red blood cell disorders whose hallmarks are ineffective erythropoiesis, hemolysis, and morphological abnormalities of erythroblasts in bone marrow. We have identified a missense mutation in KLF1 of patients with a hitherto unclassified...

  13. 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.

  14. 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.)

  15. 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

  16. 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.)

  17. 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.

  18. 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 ...

  19. Congenital hypothyroidism and concurrent renal insufficiency in a kitten

    Directory of Open Access Journals (Sweden)

    Chee Kin Lim

    2014-11-01

    Full Text Available A 3-month-old male domestic short-hair kitten was presented with chronic constipation and disproportionate dwarfism. Radiographs of the long bones and spine revealed delayed epiphyseal ossification and epiphyseal dysgenesis. Diagnosis of congenital primary hypothyroidism was confirmed by low serum total thyroxine and high thyroid stimulating hormone concentrations. Appropriate supplementation of levothyroxine was instituted. The kitten subsequently developed mild renal azotaemia and renal proteinuria, possibly as a consequence of treatment or an unmasked congenital renal developmental abnormality. Early recognition, diagnosis and treatment are vital as alleviation of clinical signs may depend on the cat’s age at the time of diagnosis.

  20. Congenital hypothyroidism and concurrent renal insufficiency in a kitten.

    Science.gov (United States)

    Lim, Chee Kin; Rosa, Chantal T; de Witt, Yolanda; Schoeman, Johan P

    2014-11-14

    A 3-month-old male domestic short-hair kitten was presented with chronic constipation and disproportionate dwarfism. Radiographs of the long bones and spine revealed delayed epiphyseal ossification and epiphyseal dysgenesis. Diagnosis of congenital primary hypothyroidism was confirmed by low serum total thyroxine and high thyroid stimulating hormone concentrations. Appropriate supplementation of levothyroxine was instituted. The kitten subsequently developed mild renal azotaemia and renal proteinuria, possibly as a consequence of treatment or an unmasked congenital renal developmental abnormality. Early recognition, diagnosis and treatment are vital as alleviation of clinical signs may depend on the cat's age at the time of diagnosis.

  1. Cellular and molecular prerequisites for bone tissue engineering

    NARCIS (Netherlands)

    Siddappa, Ramakrishnaiah

    2007-01-01

    Recent advances in medicine and other biological disciplines have considerably enhanced the life expectancy of human and consequently, resulting in age related health problems including skeletal complications. In addition, bone substitute to regenerate fractures resulting from trauma, congenital and

  2. Sonocubic fine: new three-dimensional ultrasound software to the screening of congenital heart diseases

    Directory of Open Access Journals (Sweden)

    Edward Araujo Júnior

    2014-09-01

    Full Text Available Congenital heart disease is the most common fetal congenital malformations; however, the prenatal rate detection still is low. The two-dimensional echocardiography is the "gold standard" exam to screening and diagnosis of congenital heart disease during the prenatal; however, this exam is operator-depending and it is realized only in high risk pregnancies. Spatio-temporal image correlation is a three-dimensional ultrasound software that analyses the fetal heart and your connections in the multiplanar and rendering modes; however, spatio-temporal image correlation too is operator-depending and time-consuming. We presenting a new three-dimensional software named Sonocubic fine to the screening of congenital heart disease. This software applies intelligent navigation technology to spatio-temporal image correlation volume datasets to automatically generate nine fetal echocardiography standard views. Thus, this new software tends to be less operator-depending and time-consuming.

  3. Clinicopathological features of transient myeloproliferative syndrome and congenital leukaemia

    International Nuclear Information System (INIS)

    Sajid, N.; Ahmed, N.; Mahmood, S.

    2010-01-01

    The objectives of the study were to determine the spectrum of the clinical and pathological findings, the management and prognosis of patients of transient myeloproliferative syndrome (TMS) and congenital leukaemia. Study Design: Case series. Place and Duration of Study: The study was conducted over a period of 8 years, from January 2000 to December 2007, at the Children's Hospital and the Institute of Child Health, Lahore. Methodology: Suspected patients presenting with fever, pallor, bruises and hepatosplenomegaly and diagnosed as either transient myeloproliferative disorder or congenital leukaemia were studied. The complete blood count, reticulocyte count, leukocyte alkaline phosphatase score, liver function tests, karyotyping studies and bone marrow aspiration biopsy were performed in all of those patients. Management and out come was noted. Results were described as frequency percentages. Results: Out of 10,000 patients presenting during this period, 24 patients were diagnosed as either of transient myeloproliferative syndrome or congenital leukaemia. Fifteen of these were diagnosed as patients of TMS and 9 as patients of congenital leukaemia. Down syndrome (DS) was diagnosed in 75% of these patients. TMS patients were put on supportive treatment and recovered spontaneously. One DS patient with congenital leukaemia went into spontaneous remission and 2 of DS patients with congenital leukaemia responded to chemotherapy while rest of them either died or lost to follow-up. Conclusion: TMS and congenital leukaemia were not very uncommon in the studied population. Majority had Down syndrome. It is important to differentiate their clinical and pathological presentations for proper management. TMS may resolve with supportive treatment while congenital leukaemia is a fatal condition requiring chemotherapy. (author)

  4. 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)....

  5. 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...

  6. 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.

  7. 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.

  8. 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.)

  9. 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)

  10. 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.”

  11. 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...

  12. Neuropatía sensitiva trigeminal secundaria a granuloma de colesterol de la punta del peñasco del temporal Trigeminal neuralgia secondary to cholesterol granuloma of the petrous bone apex

    Directory of Open Access Journals (Sweden)

    M.A. Pons García

    2009-10-01

    Full Text Available La neuropatía aislada de la rama sensitiva del trigémino es una entidad poco habitual. Los pacientes suelen referir hipoestesia y /o disestesia generalmente a nivel de la segunda y tercera rama del trigémino, mientras que la neuralgia es muy infrecuente.¹ Su asociación con enfermedades sistémicas del tejido conectivo es bien conocida.² Se ha descrito asociada a distintas lesiones del SNC sobre todo tumores de fosa posterior o base de cráneo, así como neoplasias mandibulares.3,4 Presentamos una paciente con hipoestesia en el territorio V2-V3 asociada a dolor hemifacial paroxístico secundario a una lesión del peñasco del temporal.Trigeminal Neuralgia is an uncommon entity. The patients report hypoesthesia and/or dysesthesia of the second and third ramus of trigeminal nerve, while neuralgia is very rare.¹ Its association with systemic diseases of connective tissue is well know.² It has been described as being associated with different lesions of the central nervous system, especially with the posterior cavity or cranial base tumors, as well as jaw neoplasias.3,4 We presented a patient with hypoesthesia V2-V3 and hemi facial paroxysmal pain secondary to lesion of petrous apex of temporal bone.

  13. Advances in cardiac magnetic resonance imaging of congenital heart disease

    Energy Technology Data Exchange (ETDEWEB)

    Driessen, Mieke M.P. [University of Utrecht, University Medical Center Utrecht, Department of Radiology, PO Box 85500, Utrecht (Netherlands); University of Utrecht, University Medical Center Utrecht, Department of Cardiology, PO Box 85500, Utrecht (Netherlands); The Interuniversity Cardiology Institute of the Netherlands (ICIN) - Netherlands Heart Institute, PO Box 19258, Utrecht (Netherlands); Breur, Johannes M.P.J. [Wilhelmina Children' s Hospital, University Medical Center Utrecht, Department of Pediatric Cardiology, PO Box 85500, Utrecht (Netherlands); Budde, Ricardo P.J.; Oorschot, Joep W.M. van; Leiner, Tim [University of Utrecht, University Medical Center Utrecht, Department of Radiology, PO Box 85500, Utrecht (Netherlands); Kimmenade, Roland R.J. van; Sieswerda, Gertjan Tj [University of Utrecht, University Medical Center Utrecht, Department of Cardiology, PO Box 85500, Utrecht (Netherlands); Meijboom, Folkert J. [University of Utrecht, University Medical Center Utrecht, Department of Cardiology, PO Box 85500, Utrecht (Netherlands); Wilhelmina Children' s Hospital, University Medical Center Utrecht, Department of Pediatric Cardiology, PO Box 85500, Utrecht (Netherlands)

    2015-01-01

    Due to advances in cardiac surgery, survival of patients with congenital heart disease has increased considerably during the past decades. Many of these patients require repeated cardiovascular magnetic resonance imaging to assess cardiac anatomy and function. In the past decade, technological advances have enabled faster and more robust cardiovascular magnetic resonance with improved image quality and spatial as well as temporal resolution. This review aims to provide an overview of advances in cardiovascular magnetic resonance hardware and acquisition techniques relevant to both pediatric and adult patients with congenital heart disease and discusses the techniques used to assess function, anatomy, flow and tissue characterization. (orig.)

  14. A case report of congenital sensory neuropathy with anhidrosis

    International Nuclear Information System (INIS)

    Lee, Won Hyong; Chang, Hae Soon; Han, Man Chung; Lee, Suck Hyun; Lee, Duk Yong

    1974-01-01

    Congenital sensory neuropathy with anhidrosis is rare disease and may be confused with other cause of pain insensitivity or indifference. Other cause of pain insensitivity include congenital indifference to pain, congenital sensory neuropathy, hereditary sensory radicular neuropathy, nonprogressive sensory radicular neuropathy, syringomyelia, and hysterical analgesia. It is hereditary disease which is transmitted with autosomal recessive trait. The patient is 8 years old Korean male with complaint of swelling and local heat on right knee joint. Generalized analgesia is noted on physical examination. The skin is dry and coarse with no evidence of sweating. Delayed motor development was noted on early children. Mental development is retarded. On past history, patient showed unpredictable rises of temperature, though the general condition remained good. Multiple painless fracture on right humerus and right metatasal bone was occurred. Rt.knee radiograms show marked swelling of soft tissue and periosteal calcification on distal femru,which are resemble with neurotrophic joint

  15. A case report of congenital sensory neuropathy with anhidrosis

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Won Hyong; Chang, Hae Soon; Han, Man Chung; Lee, Suck Hyun; Lee, Duk Yong [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    1974-10-15

    Congenital sensory neuropathy with anhidrosis is rare disease and may be confused with other cause of pain insensitivity or indifference. Other cause of pain insensitivity include congenital indifference to pain, congenital sensory neuropathy, hereditary sensory radicular neuropathy, nonprogressive sensory radicular neuropathy, syringomyelia, and hysterical analgesia. It is hereditary disease which is transmitted with autosomal recessive trait. The patient is 8 years old Korean male with complaint of swelling and local heat on right knee joint. Generalized analgesia is noted on physical examination. The skin is dry and coarse with no evidence of sweating. Delayed motor development was noted on early children. Mental development is retarded. On past history, patient showed unpredictable rises of temperature, though the general condition remained good. Multiple painless fracture on right humerus and right metatasal bone was occurred. Rt.knee radiograms show marked swelling of soft tissue and periosteal calcification on distal femru,which are resemble with neurotrophic joint.

  16. Bone marrow transplantation for correction of enzyme deficiency disease

    International Nuclear Information System (INIS)

    Hong, C.; Sutherland, D.E.R.; Matas, A.J.; Najarian, J.S.

    1979-01-01

    Mutant acatalasemic mice provide a prototype of congenital enzyme deficiency disease. Normal blood catalase levels were achieved permanently in congenitally acatalasemic mice by transplantation of bone marrow cells from congeneic normal catalasemic mice using relatively small numbers of cells following whole body irradiation. The increase in blood catalase activity was physiologically effective as demonstrated by the protection of the previously acatalasemic mice against the otherwise lethal effects of hydrogen peroxide injections. Bone marrow transplantation has the potential to provide a continuous source of some enzymes and may be applicable as treatment for certain congenital enzyme deficiency diseases

  17. 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...

  18. 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.

  19. [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.

  20. 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.)

  1. 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.

  2. 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.)

  3. 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 ...

  4. 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

  5. Congenital osteofibrous dysplasia associated with pseudoarthrosis of the tibia and fibula

    International Nuclear Information System (INIS)

    Teo, Harvey E.L.; Peh, Wilfred C.G.; Akhilesh, M.; Tan, S.B.; Ishida, T.

    2007-01-01

    The association between congenital pseudoarthrosis and osteofibrous dysplasia of the tibia and fibula is a rare entity that has been recently recognized. We report a male newborn who was found to have swelling and deformity of the left lower leg. Radiographs and magnetic resonance imaging showed an extensive destructive lesion of the tibial shaft, with dysplastic congenital pseudoarthrosis of the lower fibula. Histopathologial examination confirmed the diagnosis of congenital pseudoarthrosis of the tibia and fibula with underlying osteofibrous dysplasia involving both bones. Immunohistochemical stains showed cytokeratin positivity. (orig.)

  6. Congenital hypothyroidism and concurrent renal insufficiency in a kitten

    OpenAIRE

    Chee Kin Lim; Chantal T. Rosa; Yolanda de Witt; Johan P. Schoeman

    2014-01-01

    A 3-month-old male domestic short-hair kitten was presented with chronic constipation and disproportionate dwarfism. Radiographs of the long bones and spine revealed delayed epiphyseal ossification and epiphyseal dysgenesis. Diagnosis of congenital primary hypothyroidism was confirmed by low serum total thyroxine and high thyroid stimulating hormone concentrations. Appropriate supplementation of levothyroxine was instituted. The kitten subsequently developed mild renal azotaemia and renal pro...

  7. 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

  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. Searching for new features of intravitality of hanging based on macro- and microscopic evaluation of the proximal attachment of the sternocleidomastoid muscle and the mastoid process of the temporal bone.

    Science.gov (United States)

    Szleszkowski, Ł; Hałoń, A; Thannhäuser, A; Jurek, T

    2015-01-01

    Assessment of the usefulness of intravital lesions in the proximal attachment of the sternocleidomastoid muscle and the mastoid process of the temporal bone in medico-legal evaluation of death by hanging. The study material was obtained from the bodies of 35 people who died by hanging. The control group comprised specimens collected from 30 people who died of non-traumatic causes. The structures under study were examined macro- and microscopically. The basic change which could be recognized as a marker of intravitality of hanging was the presence of a macroscopically extensive blotchy area of abundant ecchymosis in the proximal muscle attachment, similar to that found in the distal attachment, and the presence of abundant diffuse intraosseous ecchymoses in the mastoid process. None of the cases revealed any ecchymoses in the proximal attachment of the muscle that would be similar to those present in the distal attachment. Discolourations within the mastoid processes, macroscopically suggestive of extensive intraosseous effusions arising from the mechanism of stretching, were not confirmed by microscopic evaluation and occurred at the same frequency as in the control group. Limitations of the study were related to the method which involved sample collection by means of bone chisels, decalcification and preparation of specimens, which had an effect, for example, on the measurable evaluation of the degree of congestion. The study has failed to provide convincing and unambiguous data on the usefulness of examining mastoid processes and proximal attachments of the sternocleidomastoid muscles during autopsy to determine the presence of intravitality features of hanging. A description of research methodology and its associated difficulties, e.g. with the interpretation of results, can also be useful for the planning of similar studies by other researchers.

  10. Searching for new features of intravitality of hanging based on macro- and microscopic evaluation of the proximal attachment of the sternocleidomastoid muscle and the mastoid process of the temporal bone

    Directory of Open Access Journals (Sweden)

    Łukasz Szleszkowski

    2016-03-01

    Full Text Available Aim of the study : Assessment of the usefulness of intravital lesions in the proximal attachment of the sternocleidomastoid muscle and the mastoid process of the temporal bone in medico-legal evaluation of death by hanging. Material and methods: The study material was obtained from the bodies of 35 people who died by hanging. The control group comprised specimens collected from 30 people who died of non-traumatic causes. The structures under study were examined macro- and microscopically. The basic change which could be recognized as a marker of intravitality of hanging was the presence of a macroscopically extensive blotchy area of abundant ecchymosis in the proximal muscle attachment, similar to that found in the distal attachment, and the presence of abundant diffuse intraosseous ecchymoses in the mastoid process. Results: None of the cases revealed any ecchymoses in the proximal attachment of the muscle that would be similar to those present in the distal attachment. Discolourations within the mastoid processes, macroscopically suggestive of extensive intraosseous effusions arising from the mechanism of stretching, were not confirmed by microscopic evaluation and occurred at the same frequency as in the control group. Limitations of the study were related to the method which involved sample collection by means of bone chisels, decalcification and preparation of specimens, which had an effect, for example, on the measurable evaluation of the degree of congestion. Conclusions : The study has failed to provide convincing and unambiguous data on the usefulness of examining mastoid processes and proximal attachments of the sternocleidomastoid muscles during autopsy to determine the presence of intravitality features of hanging. A description of research methodology and its associated difficulties, e.g. with the interpretation of results, can also be useful for the planning of similar studies by other researchers.

  11. 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 ...

  12. 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

  13. 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 ...

  14. Coronary artery anomalies and clinically important anatomy in patients with congenital heart disease: multislice CT findings

    International Nuclear Information System (INIS)

    Goo, Hyun Woo; Seo, Dong-Man; Yun, Tae-Jin; Park, Jeong-Jun; Park, In-Sook; Ko, Jae Kon; Kim, Young Hwee

    2009-01-01

    In patients with congenital heart disease, coronary artery anomalies are common and have different clinical importance from individuals with structurally normal hearts. Visibility of the coronary arteries by CT has markedly improved due to high temporal resolution and ECG-synchronized data acquisition. In this article we describe current multislice CT techniques for coronary artery imaging and illustrate coronary artery anomalies and clinically important coronary artery anatomy from the point of view of congenital heart disease. (orig.)

  15. Coronary artery anomalies and clinically important anatomy in patients with congenital heart disease: multislice CT findings

    Energy Technology Data Exchange (ETDEWEB)

    Goo, Hyun Woo [University of Ulsan College of Medicine, Department of Radiology and Research Institute of Radiology, Asan Medical Center, Seoul (Korea); Seo, Dong-Man; Yun, Tae-Jin; Park, Jeong-Jun [University of Ulsan College of Medicine, Department of Pediatric Cardiac Surgery, Asan Medical Center, Seoul (Korea); Park, In-Sook; Ko, Jae Kon; Kim, Young Hwee [University of Ulsan College of Medicine, Department of Pediatric Cardiology, Asan Medical Center, Seoul (Korea)

    2009-03-15

    In patients with congenital heart disease, coronary artery anomalies are common and have different clinical importance from individuals with structurally normal hearts. Visibility of the coronary arteries by CT has markedly improved due to high temporal resolution and ECG-synchronized data acquisition. In this article we describe current multislice CT techniques for coronary artery imaging and illustrate coronary artery anomalies and clinically important coronary artery anatomy from the point of view of congenital heart disease. (orig.)

  16. Functional analysis of single amino-acid mutations in the thrombopoietin-receptor Mpl underlying congenital amegakaryocytic thrombocytopenia

    NARCIS (Netherlands)

    Tijssen, Marloes R.; di Summa, Franca; van den Oudenrijn, Sonja; Zwaginga, Jaap Jan; van der Schoot, C. Ellen; Voermans, Carlijn; de Haas, Masja

    2008-01-01

    Congenital amegakaryocytic thrombocytopenia (CAMT) is a rare disorder that presents with severe thrombocytopenia and absence of megakaryocytes in the bone marrow. The disease may develop into bone marrow aplasia. Genetic defects in the gene encoding the thrombopoietin (Tpo) receptor, MPL, are the

  17. Suboccipital neuropathy after bone conduction device placement

    NARCIS (Netherlands)

    Faber, H.T.; Ru, J.A. de

    2013-01-01

    OBJECTIVE: To describe the clinical characteristics of a 70-year-old female with occipital neuropathy following bone conduction device surgery. DESCRIPTION: A 65-year-old woman underwent bone conduction device placement surgery on the left temporal bone. Postoperatively she progressively developed

  18. 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.

  19. Bone Gap Management Using Linear Rail System (LRS): Initial ...

    African Journals Online (AJOL)

    Background: Vascularized fibular grafting, free fibular graft, tibia profibula synostosis, amputation with a good prosthesis and Ilizarov technique are some of the suitable options for managing bone gaps that result from trauma or treatment of tumours, bone infection, congenital pseudoarthrosis and repeated failed ...

  20. 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.

  1. 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.

  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 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.

  4. 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 ...

  5. [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

  6. 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

  7. 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)

  8. 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

  9. 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.)

  10. 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.)

  11. Congenital adrenal hyperplasia: Treatment and outcomes

    Directory of Open Access Journals (Sweden)

    Mahdi Kamoun

    2013-01-01

    Full Text Available Congenital adrenal hyperplasia (CAH describes a group of autosomal recessive disorders where there is impairment of cortisol biosynthesis. CAH due to 21-hydroxylase deficiency accounts for 95% of cases and shows a wide range of clinical severity. Glucocorticoid and mineralocorticoid replacement therapies are the mainstays of treatment of CAH. The optimal treatment for adults with CAH continues to be a challenge. Important long-term health issues for adults with CAH affect both men and women. These issues may either be due to the disease or to steroid treatment and may affect final height, fertility, cardiometabolic risk, bone metabolism, neuro-cognitive development and the quality-of-life. Patients with CAH should be regularly followed-up from childhood to adulthood by multidisciplinary teams who have knowledge of CAH. Optimal replacement therapy, close clinical and laboratory monitoring, early life-style interventions, early and regular fertility assessment and continuous psychological management are needed to improve outcome.

  12. The Return of Congenital Rickets, Are We Missing Occult Cases?

    Science.gov (United States)

    Elidrissy, Abdelwahab T H

    2016-09-01

    Congenital rickets is the term given to fetus born with clinical features of rickets, but those born with biochemical evidence of rickets without obvious clinical features still can be considered occult congenital rickets. Some of the affected babies with this disease have the intrauterine rachitic environment, but a calcium trans-placental pump prevents the fetus from having clinical features of rickets. They may present with hypocalcemia few days after birth or later with more florid features of rickets. Congenital rickets cases born with florid features reported over the last 40 years are few and can be divided into two groups. The first due to severe maternal osteomalacia in which their bones were so decalcified to have enough calcium to be pumped to their fetus. Another group in which newborn babies were hypocalcemic due to other maternal diseases as malabsorption, celiac disease, pre-eclampsia, and prematurity. All inherited rickets cases per se, or as part of other syndromes can be considered congenital rickets. Most cases seen in our region are due to maternal vitamin D deficiency with symptoms becoming obvious when the infants are breastfed, or may present with hypocalcemic convulsions or craniotabes. This is a review of congenital rickets with the aim of shedding light on this potentially acute disease that needs more attention and awareness in the neonatal period to avoid rare serious complications as cardiomyopathy or myelofibrosis and the complications of hypocalcemic convulsions. Congenital rickets cases seen simulate a tip of an ice-burg and its prevention is an important issue, especially with the tremendous urbanization with tall buildings living in sun-deprived flats as the commonest type of residence leading to the increasing incidence of maternal osteomalacia and rickets.

  13. Bone Cancer

    Science.gov (United States)

    Cancer that starts in a bone is uncommon. Cancer that has spread to the bone from another ... more common. There are three types of bone cancer: Osteosarcoma - occurs most often between ages 10 and ...

  14. Bone Diseases

    Science.gov (United States)

    Your bones help you move, give you shape and support your body. They are living tissues that rebuild constantly ... childhood and your teens, your body adds new bone faster than it removes old bone. After about ...

  15. 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 ...

  16. 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 ...

  17. The petrous bone

    DEFF Research Database (Denmark)

    Jørkov, Marie Louise Schjellerup; Heinemeier, Jan; Lynnerup, Niels

    2009-01-01

    Intraskeletal variation in the composition of carbon (delta(13)C) and nitrogen (delta(15)N) stable isotopes measured in collagen is tested from various human bones and dentine. Samples were taken from the femur, rib, and petrous part of the temporal bone from well-preserved skeletons of both adults...... (n = 34) and subadults (n = 24). Additional samples of dentine from the root of 1st molars were taken from 16 individuals. The skeletal material is from a medieval cemetery (AD 1200-1573) in Holbaek, Denmark. Our results indicate that the petrous bone has an isotopic signal that differs significantly...... from that of femur and rib within the single skeleton (P bone and the 1st molar. The intraskeletal variation may reflect differences...

  18. 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.

  19. 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

  20. 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

  1. 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.)

  2. 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.

  3. 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.

  4. 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

  5. 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.

  6. A case of kwashiorkor in a child with congenital hypothyroidism

    Directory of Open Access Journals (Sweden)

    Helena Aneke Tangkilisan

    2001-12-01

    Full Text Available A 12-year-old Indonesian girl with the body weight of 9.8 kg and length 78 cm was admitted to the Department of Child Health, Manado General Hospital with kwashiorkor and congenital hypothyroidism. The main complaint was edema starting 3 weeks before admission. Physical examination showed body weight for age (BW/A 14.3%, body weight for body length (BW/BL 66.0% and body length for age (BL/A 49.1%. On admission she looked severely ill, apathetic, with hypothermia and hypotonia. Almost all signs and symptoms of kwashiorkor and congenital hypothyroidism were found accompanied bronchopneumonia and dermatologic problems. Laboratory findings showed severe anemia, leukocytosis, hypoproteinemia, hyponatremia, hypokalemia, elevated thyroid stimulating hormone and low level of Thyroxine-4. Bone age equaled to a newborn baby bone age. There was no thyroid tissue on thyroid ultrasound examination. The patient was treated for severe protein energy malnutrition and ThyraxR. Problems of kwashiorkor could been solved well but not with the congenital hypothyroidism. She was discharged from hospital after 2 months treatment and till now at 14th year of age with her developmental milestones equals to that of 8 month old baby.

  7. A Newborn with Congenital Mixed Phenotype Acute Leukemia After In Vitro Fertilization

    Directory of Open Access Journals (Sweden)

    Hacer Ergin

    2015-08-01

    Full Text Available Congenital leukemia is a rare disease. The majority of cases of this disease are acute myelogenous leukemia (AML. Congenital acute lymphoblastic leukemia (ALL is rare and most often is of B cell lineage. Rarely, some cases have been designated biphenotypic or mixed phenotype acute leukemia (MPAL. Herein, we report a preterm newborn referred to us as a result of the appearance of blue-violaceous dermal nodules on her body at birth. She was a twin and the product of an in vitro fertilization (IVF pregnancy. Physical examination showed jaundice, hepatosplenomegaly, and peripheral facial nerve palsy in addition to dermal nodules. Bone marrow aspiration showed 40% blasts of lymphoid lineage; skin biopsy and its immunohistochemistry revealed myeloblastic infiltration of the dermis. Cytogenetic analysis (46,XX, fluorescence in situ hybridization (FISH analysis, and cranial magnetic resonance were normal. The patient was diagnosed with congenital MPAL, and an association between IVF and congenital leukemia was suggested.

  8. Congenital Amegakaryocytic Thrombocytopenia Type II Presenting with Multiple Central Nervous System Anomalies

    NARCIS (Netherlands)

    Eshuis-Peters, Ellis; Versluys, Anne Brigitta; Stokman, Marijn Fijke; van der Crabben, Saskia Nanette; Nij Bijvank, Sebastiaan W A; van Wezel-Meijler, Gerda

    Congenital amegakaryocytic thrombocytopenia (CAMT) is a rare autosomal recessive bone marrow failure, caused by MPL gene mutations. The combination of CAMT and central nervous system abnormalities is uncommon. We describe a case with a homozygous missense MPL gene mutation and polymicrogyria,

  9. [Amyoplasia congenita: a serious congenital abnormality with a relatively favorable prognosis

    NARCIS (Netherlands)

    Petru, R.; Verrips, A.; Ravenswaaij-Arts, C.M.A. van

    2002-01-01

    After an uneventful pregnancy a girl was born with serious joint contractures and several fractures of the long bones. The family history was negative for congenital abnormalities. Based on the distinct clinical presentation the diagnosis was 'amyoplasia', which is a partial aplasia of skeletal

  10. Temporal networks

    CERN Document Server

    Saramäki, Jari

    2013-01-01

    The concept of temporal networks is an extension of complex networks as a modeling framework to include information on when interactions between nodes happen. Many studies of the last decade examine how the static network structure affect dynamic systems on the network. In this traditional approach  the temporal aspects are pre-encoded in the dynamic system model. Temporal-network methods, on the other hand, lift the temporal information from the level of system dynamics to the mathematical representation of the contact network itself. This framework becomes particularly useful for cases where there is a lot of structure and heterogeneity both in the timings of interaction events and the network topology. The advantage compared to common static network approaches is the ability to design more accurate models in order to explain and predict large-scale dynamic phenomena (such as, e.g., epidemic outbreaks and other spreading phenomena). On the other hand, temporal network methods are mathematically and concept...

  11. 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.

  12. 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 ...

  13. 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 ...

  14. 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: ...

  15. 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.

  16. 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.

  17. Orofacial manifestations of congenital hypothyroidism: Clinicoradiological case report

    Directory of Open Access Journals (Sweden)

    Gundareddy N Suma

    2014-01-01

    Full Text Available Thyroid disorders have a widespread effect on the ossification of cartilage, growth of teeth, facial contour, and overall body proportions. Both growth hormones and thyroid hormones show permissive action on growth stimulation. Deregulation in thyroid homeostasis can result in various orofacial and skeletal abnormalities. This report describes a case of congenital hypothyroidism in a 20-year-old female patient, presenting with a short stature, hypotonia, alopecia, euryprosopic face with puffiness, multiple retained deciduous teeth, delayed fusion of the intracranial sutures, and epiphysis and diaphysis of the long bones. Based on various biochemical and radiographic investigations, diagnosis of congenital hypothyroidism was established. Craniometry and hand-wrist radiographic evaluation of the growth pattern further aided in strengthening the diagnosis.

  18. Erosions of the Petrous Temporal Bone

    African Journals Online (AJOL)

    tumours of the parotid gland; tumours of the paranasal sinuses; and tumours of ... showed severe erosion of the anterior and posterior walls and floor of the external .... sphenoid sinus, the pituitary fossa, and the greater and lesser wings of the ...

  19. Spontaneous Bilateral Meningoencephalocoeles of the Temporal Bones

    Directory of Open Access Journals (Sweden)

    Oliver Rose

    2013-01-01

    Full Text Available Spontaneous tegmen tympani defects are rare with even rarer bilateral cases. The symptoms are nonspecific; hence, a high index of suspicion is required to prevent serious intracranial complications. We present a case of spontaneous bilateral tegmen tympani defects with associated meningoencephalocoeles in a 54-year-old male who presented with the signs and symptoms of severe meningitis. After careful workup which included a lumbar puncture, CT and MRI scans, both defects were repaired using a middle fossa approach. The patient made an uneventful recovery with complete cessation of otorrhoea and improvement in his hearing.

  20. 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.

  1. 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.

  2. 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.

  3. 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.

  4. 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.

  5. [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.

  6. 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.)

  7. 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.

  8. Project Temporalities

    DEFF Research Database (Denmark)

    Tryggestad, Kjell; Justesen, Lise; Mouritsen, Jan

    2013-01-01

    Purpose – The purpose of this paper is to explore how animals can become stakeholders in interaction with project management technologies and what happens with project temporalities when new and surprising stakeholders become part of a project and a recognized matter of concern to be taken...... into account. Design/methodology/approach – The paper is based on a qualitative case study of a project in the building industry. The authors use actor-network theory (ANT) to analyze the emergence of animal stakeholders, stakes and temporalities. Findings – The study shows how project temporalities can...... multiply in interaction with project management technologies and how conventional linear conceptions of project time may be contested with the emergence of new non-human stakeholders and temporalities. Research limitations/implications – The study draws on ANT to show how animals can become stakeholders...

  9. Bone marrow aspiration

    Science.gov (United States)

    Iliac crest tap; Sternal tap; Leukemia - bone marrow aspiration; Aplastic anemia - bone marrow aspiration; Myelodysplastic syndrome - bone marrow aspiration; Thrombocytopenia - bone marrow aspiration; Myelofibrosis - bone marrow aspiration

  10. Low Bone Density

    Science.gov (United States)

    ... Density Exam/Testing › Low Bone Density Low Bone Density Low bone density is when your bone density ... people with normal bone density. Detecting Low Bone Density A bone density test will determine whether you ...

  11. Amplificação por condução óssea em malformações congênitas: benefício e satisfação Amplification by bone conduction in congenital malformations: patient benefits and satisfaction

    Directory of Open Access Journals (Sweden)

    Elaine Cristina Moreto Paccola

    2013-06-01

    Full Text Available A deficiência auditiva é um dos achados clínicos mais comuns em sujeitos com malformações de orelha. O tratamento consiste em realizar a cirurgia e/ou adaptar o aparelho de amplificação sonora por via óssea (AASI VO. A intervenção precoce é fundamental para favorecer a estimulação auditiva e desenvolvimento da fala e linguagem. OBJETIVO: Caracterizar o perfil audiológico de sujeitos com malformação congênita de orelha externa e/ou média e avaliar o benefício e a satisfação destes com o uso de AASI VO. MÉTODO: Estudo descritivo, sujeitos com malformações congênitas bilaterais de orelha externa e/ou média, deficiência auditiva condutiva ou mista, moderada ou grave e usuários de AASI VO. Avaliação do benefício utilizando teste de reconhecimento de sentenças com ruído competitivo e medidas de ganho funcional e avaliação da satisfação utilizando questionário internacional QI - AASI. RESULTADOS: Foram avaliados 13 sujeitos, sendo 61% do sexo masculino e 80% com deficiência auditiva condutiva moderada ou grave. Houve melhor desempenho na avaliação proposta na condição com AASI, quando comparada à condição sem AASI. CONCLUSÃO: Os AASI VO retroauriculares apresentaram vantagens para a população estudada e devem ser considerados como uma opção para intervenção. A satisfação foi confirmada pelos escores elevados obtidos no QI - AASI.Hearing loss is one of the most common clinical findings in subjects with malformations of the ear. Treatment consists of surgery and/or adapt a hearing aid amplification by bone (HA VO. Early intervention is critical to auditory stimulation and development of speech and language. OBJECTIVE: To characterize the audiological profile of subjects with congenital malformation of the external ear and/or middle and evaluate the benefit and satisfaction of using HA VO. METHOD: A descriptive study, subjects with bilateral congenital malformations of the external ear and/or middle

  12. Congenital Unilateral Deafness Affects Cerebral Organization of Reading

    Directory of Open Access Journals (Sweden)

    Alice Mado Proverbio

    2013-06-01

    Full Text Available It is known that early sensory deprivation modifies brain functional structure and connectivity. The aim of the present study was to investigate the neuro-functional organization of reading in a patient with profound congenital unilateral deafness. Using event-related potentials (ERPs, we compared cortical networks supporting the processing of written words in patient RA (completely deaf in the right ear since birth and in a group of control volunteers. We found that congenital unilateral hearing deprivation modifies neural mechanisms of word reading. Indeed, while written word processing was left-lateralized in controls, we found a strong right lateralization of the fusiform and inferior occipital gyri activation in RA. This finding goes in the same direction of recent proposals that the ventral occipito-temporal activity in word reading seem to lateralize to the same hemisphere as the one involved in spoken language processing.

  13. Specifics of psychomotor development in group of congenital blind children

    Directory of Open Access Journals (Sweden)

    Zbyněk Janečka

    2011-03-01

    Full Text Available Ontogenesis of the psychomotor development in group of congenital blind children has its own specifics. Visual defect is influenced by many things. In the period from birth to two years of age occur in children, significant changes in cognitive, psychomotor and social development. Compared with the normal sighted population go the development of congenital blind children in all these areas slower. Visual deprivation also influenced on development of body posture. More important is whether the development proceeds in stages that correspond to the development of normal vision child. If development proceeds in the right direction is the temporal aspect criterion rather orientation. For blind children is also important to strengthen the ability to correctly identify their own body through somatognosy. Stereognosy in turn determines the degree of contact with the outer world and focus it in relation to the physical schema.

  14. Histone methylations in heart development, congenital and adult heart diseases.

    Science.gov (United States)

    Zhang, Qing-Jun; Liu, Zhi-Ping

    2015-01-01

    Heart development comprises myocyte specification, differentiation and cardiac morphogenesis. These processes are regulated by a group of core cardiac transcription factors in a coordinated temporal and spatial manner. Histone methylation is an emerging epigenetic mechanism for regulating gene transcription. Interplay among cardiac transcription factors and histone lysine modifiers plays important role in heart development. Aberrant expression and mutation of the histone lysine modifiers during development and in adult life can cause either embryonic lethality or congenital heart diseases, and influences the response of adult hearts to pathological stresses. In this review, we describe current body of literature on the role of several common histone methylations and their modifying enzymes in heart development, congenital and adult heart diseases.

  15. MR imaging in congenital lower limb deformities

    International Nuclear Information System (INIS)

    Laor, T.; Jaramillo, D.; Hoffer, F.A.; Kasser, J.R.

    1996-01-01

    Treatment for children with cogenital deformities of the lower extremities may vary, depending on the state of the unossified skeletal structures and surrounding soft tissues. The purpose of our study was to demonstrate the spectrum of the osteochondral and extrasosseous abnormalities as depicted with MR imaging. We retrospectively reviewed MR examinations of 13 limbs of ten children (aged 1 month-9 years, mean 2.1 years) with longitudinal and transverse deformities of the lower extremities. The lesions imaged were fibular hemimelia (n=5), tibial hemimelia (n=5), and congenital constriction bands (n=3). Each examination was assessed for abnormalities in the osteocartilaginous and extraosseous (articular or periarticular components such as ligaments, tendons, and menisci; the muscles and the arteries) structures. Abnormalities were seen in all patients. Osteocartilaginous abnormalities in the patients with longitudinal deformities included abnormal distal femoral epiphyses, abnormal proximal tribial physes, hypertrophied and dislocated proximal fibular epiphyses, unsuspected fibular and tibial remnants, and absence or coalition of the tarsal bones. No osteocartilaginous abnormalities were seen in the patients with congential constriction bands. Articular abormalities in patients with either form of hemimelia included absent cruciate ligaments and menisci, dislocated or absent cartilaginous patellae, absent patellar tendons, and abnormal collateral ligaments. All but one limb imaged had absent or attenuated muscle groups. Of the nine MR arteriograms performed at the level of the knee, eight were abnormal. The normal popliteal trifurcation was absent or in an abnormal location. We conclude that MR imaging of children with congenital lower extremity deformities shows many osteochondral and extraosseous abnormalities that are not depicted by conventional radiogrpahy. This information can help to plan early surgical intervention and prosthetic rehabilitation. (orig.)

  16. Congenital vascular malformations in scintigraphic evaluation

    International Nuclear Information System (INIS)

    Pilecki, Stanisław; Gierach, Marcin; Gierach, Joanna; Świętaszczyk, Cyprian; Junik, Roman; Lasek, Władysław

    2014-01-01

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

  17. [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.

  18. Reversibility of cortical hyperostosis following long-term prostaglandin E1 therapy in infants with ductus-dependent congenital heart disease

    DEFF Research Database (Denmark)

    Høst, A; Halken, S; Andersen, P E

    1988-01-01

    Two neonates with complex cyanotic congenital heart disease, receiving long-term prostaglandin E1 infusion, for 59 and 78 days respectively, demonstrated significant radiographic changes of symmetric cortical hyperostosis of the long bones. Bone biopsies from one of the patients elucidated...

  19. 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

  20. 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

  1. 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

  2. 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 ...

  3. 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.

  4. 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.)

  5. 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 ...

  6. 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 ...

  7. Dental signs attributed to congenital syphilis and its treatments in the Hamann-Todd Skeletal Collection

    Directory of Open Access Journals (Sweden)

    Ioannou Stella

    2017-12-01

    Full Text Available Syphilis in the United States during the 1800s and 1900s had a high prevalence rate causing great concern to health officials. Various measures were taken to control its spread. Mercuric treatments were used up until the introduction of penicillin. The aim of this paper is to determine whether dental abnormalities related to congenital syphilis in individuals who died of syphilis or syphilis-related causes, in the Hamman Todd Osteological Collection, occur and whether mercurial treatment was effective. Hutchinson, Moon and Fournier’s works were analyzed to determine dental abnormalities associated with congenital syphilis and its treatments and used as criteria. Hillson et al. (AJPA,107:25-40 standardized method of description of dental changes was used. In the Hamman Todd Osteological Collection in Cleveland, Ohio, 102 individuals had cause of death recorded in the catalogue as syphilis or lues, and 69 had causes of death relating to syphilis which included paresis (53, aortic insufficiency (15 and pericarditis (1. Thus altogether 171 individuals were studied. Dentition was examined to determine if dental abnormalities associated with congenital syphilis and its treatments were present in individuals not recorded as having congenital syphilis. Crania were examined for any osteological changes. One individual (2266 demonstrated dental malformations possibly related to the congenital disease itself, while three demonstrated dental abnormalities associated with mercuric treatments in childhood (2118, 2263 and 3097. No remarkable bone pathologies were evident on any skull. The use of pre-penicillin treatment of congenital syphilis may have been effective to maintain health into adulthood but not always in eradicating the infection. Effects of mercury on enamel formation and bone changes, need to be considered when making a differential diagnosis of syphilis/congenital syphilis.

  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. Bone tumors

    International Nuclear Information System (INIS)

    Moylan, D.J.; Yelovich, R.M.

    1991-01-01

    Primary bone malignancies are relatively rare with less than 4,000 new cases per year. Multiple myeloma (more correctly a hematologic malignancy) accounts for 40%; osteosarcomas, 28%; chondrosarcomas, 13%; fibrosarcomas arising in bone, 4%; and Ewing's sarcoma, 7%. The authors discuss various treatments for bone tumors, including radiotherapy, chemotherapy and surgery

  10. 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.

  11. 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

  12. 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.)

  13. 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.)

  14. 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...

  15. 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...

  16. Bone scanning in the child and young adult. Pt. 2

    Energy Technology Data Exchange (ETDEWEB)

    Murray, I P.C. [Prince of Wales Hospital, Randwick (Australia). Dept. of Nuclear Medicine

    1980-01-01

    The sensitivity of the radionuclide bone scan in identifying osteoblastic reaction in bone and in detecting local alterations in blood flow is valuable in many benign diseases involving bone, particularly those which are more common in children and young adults, and in which early detection may be critical to future health. Bone scanning offers a simple yet reliable means for establishing an early diagnosis, evaluating the extent of the disease, and assessing the therapeutic response in disorders resulting from infection, trauma, or vascular insult. Useful information may also be obtained in disturbances of growth and development, and in congenital lesions.

  17. Temporal networks

    Science.gov (United States)

    Holme, Petter; Saramäki, Jari

    2012-10-01

    A great variety of systems in nature, society and technology-from the web of sexual contacts to the Internet, from the nervous system to power grids-can be modeled as graphs of vertices coupled by edges. The network structure, describing how the graph is wired, helps us understand, predict and optimize the behavior of dynamical systems. In many cases, however, the edges are not continuously active. As an example, in networks of communication via e-mail, text messages, or phone calls, edges represent sequences of instantaneous or practically instantaneous contacts. In some cases, edges are active for non-negligible periods of time: e.g., the proximity patterns of inpatients at hospitals can be represented by a graph where an edge between two individuals is on throughout the time they are at the same ward. Like network topology, the temporal structure of edge activations can affect dynamics of systems interacting through the network, from disease contagion on the network of patients to information diffusion over an e-mail network. In this review, we present the emergent field of temporal networks, and discuss methods for analyzing topological and temporal structure and models for elucidating their relation to the behavior of dynamical systems. In the light of traditional network theory, one can see this framework as moving the information of when things happen from the dynamical system on the network, to the network itself. Since fundamental properties, such as the transitivity of edges, do not necessarily hold in temporal networks, many of these methods need to be quite different from those for static networks. The study of temporal networks is very interdisciplinary in nature. Reflecting this, even the object of study has many names-temporal graphs, evolving graphs, time-varying graphs, time-aggregated graphs, time-stamped graphs, dynamic networks, dynamic graphs, dynamical graphs, and so on. This review covers different fields where temporal graphs are considered

  18. Bone banking.

    Science.gov (United States)

    Howard, W

    1999-04-01

    The use of human organs and tissues for transplantation in Australia has increased significantly over the past 30 years. In 1997, the Australian Coordinating Committee on Organ Registries and Donation (ACCORD) reported a total number of 190 organ donors, 636 corneal donors and 1509 bone donors Australia wide. Of the 1509 bone donations, 143 came from cadaveric sources and 1366 were made by living donors. Bone transplantation is not as widely recognised as solid organ or corneal transplantation. Due to improved technology and surgical skills, the demand for bone transplantation has increased markedly. This Clinical Update will provide an overview of the physiological aspects of bone transplantation and explore bone banking, a key step in the complex and critical process of bone transplantation.

  19. 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

  20. 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.

  1. 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

  2. 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.

  3. [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.

  4. 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 (=

  5. 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.

  6. 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.

  7. 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.)

  8. 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

  9. Medico-legal aspects of congenital heart diseases in buying and selling of pets

    Directory of Open Access Journals (Sweden)

    Annamaria Passantino

    2017-01-01

    Full Text Available Aim: The veterinarian should be able to assess congenital and inherited malformations such as heart defects because they may be object of legal disputes. In this study, the authors report some cases of congenital heart defects in pets (dogs and cats to clarify whether or not they may be considered a redhibitory defect. Materials and Methods: A total of 28 medical records of pets referred with suspected congenital heart disease were examined. All patients aged between 3 and 24 months underwent clinical examination, chest X-ray examination, electrocardiogram, and echocardiography and angiocardiography when necessary. Results: Congenital heart diseases or associated cardiac malformations were confirmed. Considering the above congenital diseases as redhibitory defect and the rights of the owners from a strictly legal viewpoint, 9 owners demanded an estimatory action and 11 a redhibitory action; 1 owner decided to demand the reimbursement of veterinary expenses because the animal died; 7 owners took no legal action but requested surgical intervention. Conclusions: Until more appropriate and detailed legislation on the buying and selling of pet animals is put in place; the authors propose to include in the contract a temporal extension of the guarantee relating to congenital heart disease, which can often become evident later.

  10. 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

  11. Congenital cystic masses of the face and neck: CT evaluation

    International Nuclear Information System (INIS)

    Chung, Hae Gyeong; Kim, Hyung Jin; Kim, Jae Hyoung; Hwang, Eui Gee; Jeon, Sea Young; Kim, Sun Young; Chung, Sung Hoon

    1991-01-01

    Recognition of the congenital cystic masses of the face and neck is important because they are usually benign, and can be completely cured by surgical excision. We retrospective analyzed CT scan of 18 surgically proven congenital cystic masses of the face and neck. The cases included 5 thyroglossal duct cysts, 4 cystic hygromas, 5 dermoid cysts, 1 branchial cleft cyst, and 3 fissural cysts of the face. Of five cases of thyroglossal duct cysts, CT showed either a well-marginated, rim enhancing unilocular cystic mass (n=3), or a diffuse but heterogeneous highly enhancing soft tissue mass (n=2). The latter two cases were confirmed later as infected thyroglossal duct cysts. Four cases of cystic hygromas were seen as either an irregularly-marginated (n=3) or a well-marginated (n=1) rim enhancing multiseptated cystic mass with a fluid-fluid level. Five cases of dermoid cysts appeared as well-marginated rim enhancing unilocular ovoid masses. The content of each mass was predominantly fluid in four cases, of which additional solid components were found in two, and interspersed fat globules in one. One case was composed of a homogeneous fatty density. One case of branchial cleft cyst was in the anterior triangle near the left mandibular angle, and appeared as a well-marginated enhancing cystic mass with a thick rim. In this case displacement of the adjacent structures was noted also. All three cases of fissural cysts of the face were seen as a well-marginated, rim enhancing cystic mass, causing a smooth pressure erosion of the adjacent bones. We conclude that CT is useful for the evaluation of the congenital cystic masses of the face and neck, because it can differentiate various forms of the congenital lesions and is able to clearly reveal the relation of the mass to the adjacent structures

  12. Congenital cystic masses of the face and neck: CT evaluation

    Energy Technology Data Exchange (ETDEWEB)

    Chung, Hae Gyeong; Kim, Hyung Jin; Kim, Jae Hyoung; Hwang, Eui Gee; Jeon, Sea Young; Kim, Sun Young; Chung, Sung Hoon [Gyeongsang National University, Jinju (Korea, Republic of)

    1991-09-15

    Recognition of the congenital cystic masses of the face and neck is important because they are usually benign, and can be completely cured by surgical excision. We retrospective analyzed CT scan of 18 surgically proven congenital cystic masses of the face and neck. The cases included 5 thyroglossal duct cysts, 4 cystic hygromas, 5 dermoid cysts, 1 branchial cleft cyst, and 3 fissural cysts of the face. Of five cases of thyroglossal duct cysts, CT showed either a well-marginated, rim enhancing unilocular cystic mass (n=3), or a diffuse but heterogeneous highly enhancing soft tissue mass (n=2). The latter two cases were confirmed later as infected thyroglossal duct cysts. Four cases of cystic hygromas were seen as either an irregularly-marginated (n=3) or a well-marginated (n=1) rim enhancing multiseptated cystic mass with a fluid-fluid level. Five cases of dermoid cysts appeared as well-marginated rim enhancing unilocular ovoid masses. The content of each mass was predominantly fluid in four cases, of which additional solid components were found in two, and interspersed fat globules in one. One case was composed of a homogeneous fatty density. One case of branchial cleft cyst was in the anterior triangle near the left mandibular angle, and appeared as a well-marginated enhancing cystic mass with a thick rim. In this case displacement of the adjacent structures was noted also. All three cases of fissural cysts of the face were seen as a well-marginated, rim enhancing cystic mass, causing a smooth pressure erosion of the adjacent bones. We conclude that CT is useful for the evaluation of the congenital cystic masses of the face and neck, because it can differentiate various forms of the congenital lesions and is able to clearly reveal the relation of the mass to the adjacent structures.

  13. Clinical, chromosomal and endocrine studies for congenital adrenal hyperplasia

    International Nuclear Information System (INIS)

    Soliman, S.E.; Shousha, M.; Hafez, M.

    2006-01-01

    Severe forms of congenital adrenal hyperplasia are potentially fatal if unrecognized and untreated. The aim of this study was to clarify the clinical presentation together with the chromosomal and laboratory associations in this syndrome. Twenty four patients diagnosed as congenital adrenal hyperplasia were referred from Children's Hospital, Cairo University, Egypt, for hormonal and chromosomal workup. The age ranged from eight months to 19 years with mean age of 3.18 years. Twenty two patients were diagnosed as classic congenital adrenal hyperplasia (CAH) syndrome. Severe salt wasting form was present in ten patients whereas simple virilisation was the presenting manifestation in twelve patients. Two patients presented as late onset congenital adrenal hyperplasia (LOCAH). The sex of rearing was female in 18 cases and male in six cases. Genitography and sonography confirmed the presence of female internal organs in all cases. Advanced bone age was evident by radiographic studies. Although the karyotyping was 46,XX in all cases, the diagnosed correct sex was delayed in six cases. Serum concentrations of 17-hydroxyprogesterone (17.OH.P), dehydroepiandrosterone sulfate (DHEAS), delta, 4-androstenedione (D4A), testosterone (T) and 11-deoxycortisol were all elevated as compared to controls. It was found that the adrenal androgens DHEAS, D4A and T were more elevated in salt losers when compared to simple virilising patients. However, this difference was statistically non-significant. The present study demonstrates that the clinical examination and laboratory investigations are necessary for the early detection and treatment of these cases to avoid major medical and psychological problems for the patients and their parents

  14. Clinical, Chromosomal and Endocrine Studies for Congenital Adrenal Hyperplasia

    International Nuclear Information System (INIS)

    Shousha, M.A.; Somaya, E.T.; Attia, M.

    2007-01-01

    Several forms of congenital adrenal hyperplasia are potentially fatal if unrecognized and untreated. The aim of this study is to throw light on the clinical presentation together with chromosomal and laboratory associations in this syndrome. Twenty four patients diagnosed as congenital adrenal hyperplasia were referred from the Diabetic Endocrine Metabolic Pediatric Unit [DEMPU], Children's Hospital, Cairo University for hormonal and chromosomal workup. Twenty two patients were diagnosed as classic congenital adrenal hyperplasia (CAH) syndrome. Sever salt wasting form was present in ten patients whereas simple virilization was the presenting manifestation in twelve patients. Two patients presented as late onset congenital adrenal hyperplasia (LOCAH). The mean age was 3.18 years, ranging from eight months to 19 years. The sex of rearing was Female in 18 cases and male in six cases. Genitography and sonography confirmed the presence of female internal organs in all cases. Advanced bone age was evident by radiographic studies. Although the karyotyping was (46,XX) in all cases, the correct sex diagnosis was delayed in 6 cases. Serum concentrations of 17-hydroxyprogesterone (17.OH.P); Dehydroepiandrosterone sulfate (DHEAS); Delta,4-androstenedione (D 4 A); Testosterone and 11-deoxycortisol were all elevated in relation to controls. We found that the adrenal androgens DHEAS, delta 4A, and T were more elevated in salt losers when compared to simple virilizing patients. However, this difference was not of statistical significance. The present study demonstrates that clinical examination and laboratory investigations are necessary for early detection and treatment of hese cases to avoid major medical and psychological problems for the patients and their parents.

  15. One-stage metatarsal lengthening by allograft interposition: a novel approach for congenital brachymetatarsia.

    Science.gov (United States)

    Giannini, Sandro; Faldini, Cesare; Pagkrati, Stavroula; Miscione, Maria Teresa; Luciani, Deianira

    2010-07-01

    Congenital brachymetatarsia, a shortened metatarsal bone, can be corrected surgically by callus distraction or one-stage lengthening using bone graft. We asked whether one-stage metatarsal lengthening using metatarsal homologous bone graft could improve forefoot function, lead to metatarsal healing, restore metatarsal parabola, and improve cosmetic appearance. We retrospectively reviewed 29 patients (41 feet) in whom we lengthened 50 metatarsals. Surgery consisted of a transverse proximal osteotomy of the metatarsal shaft and interposition of a metatarsal homologous bone graft (average, 13 mm long) fixed with an intramedullary Kirschner wire. Minimum followup was 3 years (mean, 5 years; range, 3-11 years). Bone union was achieved in all cases. The mean preoperative American Orthopaedic Foot and Ankle Society score was 37 points (range, 28-53 points) and the mean postoperative score was 88 points (range, 74-96 points), with an average improvement of 51 points. Radiographically, the mean gain in length was 13 mm (range, 10-15 mm), and the mean percentage increase was 23%. One-stage metatarsal lengthening using interposition of metatarsal homologous bone graft to correct congenital brachymetatarsia has low morbidity for the patient, limited complications, short recovery times, and restores forefoot anatomy. Level IV, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence.

  16. Temporal naturalism

    Science.gov (United States)

    Smolin, Lee

    2015-11-01

    Two people may claim both to be naturalists, but have divergent conceptions of basic elements of the natural world which lead them to mean different things when they talk about laws of nature, or states, or the role of mathematics in physics. These disagreements do not much affect the ordinary practice of science which is about small subsystems of the universe, described or explained against a background, idealized to be fixed. But these issues become crucial when we consider including the whole universe within our system, for then there is no fixed background to reference observables to. I argue here that the key issue responsible for divergent versions of naturalism and divergent approaches to cosmology is the conception of time. One version, which I call temporal naturalism, holds that time, in the sense of the succession of present moments, is real, and that laws of nature evolve in that time. This is contrasted with timeless naturalism, which holds that laws are immutable and the present moment and its passage are illusions. I argue that temporal naturalism is empirically more adequate than the alternatives, because it offers testable explanations for puzzles its rivals cannot address, and is likely a better basis for solving major puzzles that presently face cosmology and physics. This essay also addresses the problem of qualia and experience within naturalism and argues that only temporal naturalism can make a place for qualia as intrinsic qualities of matter.

  17. Sickle Cell Disease with Cyanotic Congenital Heart Disease: Long-Term Outcomes in 5 Children.

    Science.gov (United States)

    Iannucci, Glen J; Adisa, Olufolake A; Oster, Matthew E; McConnell, Michael; Mahle, William T

    2016-12-01

    Sickle cell disease is a risk factor for cerebrovascular accidents in the pediatric population. This risk is compounded by hypoxemia. Cyanotic congenital heart disease can expose patients to prolonged hypoxemia. To our knowledge, the long-term outcome of patients who have combined sickle cell and cyanotic congenital heart disease has not been reported. We retrospectively reviewed patient records at our institution and identified 5 patients (3 girls and 2 boys) who had both conditions. Their outcomes were uniformly poor: 4 died (age range, 12 mo-17 yr); 3 had documented cerebrovascular accidents; and 3 developed ventricular dysfunction. The surviving patient had developmental delays. On the basis of this series, we suggest mitigating hypoxemia, and thus the risk of stroke, in patients who have sickle cell disease and cyanotic congenital heart disease. Potential therapies include chronic blood transfusions, hydroxyurea, earlier surgical correction to reduce the duration of hypoxemia, and heart or bone marrow transplantation.

  18. Bone development

    DEFF Research Database (Denmark)

    Tatara, M.R.; Tygesen, Malin Plumhoff; Sawa-Wojtanowicz, B.

    2007-01-01

    The objective of this study was to determine the long-term effect of alpha-ketoglutarate (AKG) administration during early neonatal life on skeletal development and function, with emphasis on bone exposed to regular stress and used to serve for systemic changes monitoring, the rib. Shropshire ram.......01). Furthermore, AKG administration induced significantly higher bone mineral density of the cortical bone by 7.1% (P

  19. Congenital oval or round window anomaly with or without abnormal facial nerve course: surgical results for 15 ears.

    Science.gov (United States)

    Thomeer, Henricus; Kunst, Henricus; Verbist, Berit; Cremers, Cor

    2012-07-01

    To describe the audiometric results in a consecutive series of patients with congenital ossicular aplasia (Class 4a) or dysplasia of the oval and/or round window (Class 4b), which might include a possible anomalous course of the facial nerve. Retrospective chart study. Tertiary referral center. A tertiary referral center study with a total of 14 patients with congenital minor ear anomalies as part of a consecutive series (n = 89) who underwent exploratory tympanotomies (15 ears). Audiometric results. In 8 of 15 ears, ossicular reconstruction was attempted. In the short term (1 mo), there was a serviceable hearing outcome (air-bone gap closure to within 25 dB) in 4 ears. However, the long-term results showed deterioration because of an increased air-bone gap in all but 1 ear. No facial nerve lesion was observed postoperatively. Congenital dysplasia or aplasia of the oval and/or round window is an uncommon congenital minor ear anomaly. Classical microsurgical opportunities are rare in this group of anomalies. Newer options for hearing rehabilitation, such as the osseointegrated passive bone conduction devices, have become viable alternatives for conventional air conduction hearing devices. In the near future, upcoming active bone conduction devices might become the most preferred surgical option. In cases in which the facial nerve is only partially overlying the oval window, a type of malleostapedotomy procedure might result in a serviceable postoperative hearing level.

  20. [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.