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.

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

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.

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.

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

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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.

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

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

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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.

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

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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.

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.

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.

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.

Chondroblastoma of temporal bone

Energy Technology Data Exchange (ETDEWEB)

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.

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.)

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

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)

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.

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.

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.

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.

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)

Osteoradionecrosis of the temporal bone

Energy Technology Data Exchange (ETDEWEB)

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)

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

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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.

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

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.

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.

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.

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.

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

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.

Massive Cerebrospinal Fluid Leak of the Temporal Bone

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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.

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)

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

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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.

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

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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.

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

A case of osteoradionecrosis of the temporal bone

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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)

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

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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.

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.).

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.)

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

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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.

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

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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.

IgG4-Related Disease of Bilateral Temporal Bones.

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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.

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

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

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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.

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

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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.

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

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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

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.

Cerebrospinal otorrhoea--a temporal bone report.

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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.

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

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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.

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)

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

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

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

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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.

Mondini defect in association with multiple congenital anomalies.

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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.

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

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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

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)

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...

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

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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.

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.

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.

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.

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.)

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.)

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, ...

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

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.

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.

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)

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)

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.

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

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)

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

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

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.

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...

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)

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.

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.

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)

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.

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.

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.

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

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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.

Fracture of the temporal bone in patients with traumatic brain injury

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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.

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

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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.

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

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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.

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

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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.

Skeletal changes in congenital fibrinogen abnormalities

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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.

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

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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)

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

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

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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

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

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...

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)

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

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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.

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.

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.)

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)

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

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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

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

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

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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.

Sclerosteosis involving the temporal bone: histopathologic aspects.

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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.

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

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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.

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

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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.

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

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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.

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.

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.)

Congenital Unilateral Hypoplasia of Depressor Anguli Oris

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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.

Mixed reality temporal bone surgical dissector: mechanical design.

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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.

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)

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

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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%.

[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.

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

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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

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.

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

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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

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

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.

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

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.

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

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.

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.

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

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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.

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.

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

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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.

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....

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.)

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

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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.

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.

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.

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

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.

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.

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.

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.

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.

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

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%)

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.

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...

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

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)

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

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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.

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.

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.

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

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

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.

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.

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

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)

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.

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)

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

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.)

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

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.)

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.

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.

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

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)

Pneumatization of the zygomatic process of temporal bone on computed tomograms

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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

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

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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

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

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

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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

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).

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.)

Repair of Temporal Bone Encephalocele following Canal Wall Down Mastoidectomy

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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.

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.

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

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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.

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.

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.

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.

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.

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

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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).

[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.

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)

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

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.

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

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

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.

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.

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.

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...

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.)

Dense bone - too much bone: Radiological considerations and differential diagnosis. Pt. 2

Energy Technology Data Exchange (ETDEWEB)

Jacobson, H.G.

1985-02-01

In conclusion, the attempt has been made to demonstrate that three major forms of new bone formation exist: reactive, neoplastic, and the newborn or relative skeletal sclerosis in congenital (developmental) disorders. A classification of skeletal disorders has been presented and four major groups have been selected from the nine categories in this classification. These are: congenital-developmental, metabolic and endocrine, benign neoplasms and malignant neoplasms. In all four categories a large group of entities which may present with new bone (sclerosis) are listed and are discussed in some, but limited, detail. A number of these entities in each of the four categories are illustrated. Some difficulty is encountered in considering the mechanisms for the production of bony sclerosis in the group of congenital-developmental disorders. In such entities as osteopetrosis, the overproduction of cartilage cords and subsequent excessive mineralization is known to be responsible for the dense bone. However, in various skeletal dysplasias (e.g. pyknodysostosis, van Bucherm disease), the exact mechanism for the development of the diffuse sclerotic process is not clearly understood. In the metabolic and endocrine category, the situation as to mechanism is less unclear in considering the reason for the development of bony sclerosis. Yet even in evaluating disorders such as renal osteodystrophy, the reactive bony sclerosis in the presence of secondary hyperparathyroidism and osteomalacia is a source of speculation with no definite proof, as yet.

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.

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.)

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.)

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.

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)

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) ...

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%)

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

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.

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.

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

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 ...

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)

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.

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

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.

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.

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.

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)

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.

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)

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.

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.

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.

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

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

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.

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.

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

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.

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.)

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.)

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 ...

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.

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.

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

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)

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.

Case report: unicameral bone cysts in a young patient with acquired generalized lipodystrophy.

Science.gov (United States)

Gregory, James M; Arkader, Alexandre; Bokhari, Aqiba; Bothari, Aqiba; Dormans, John P

2010-05-01

We report the case of a 13-year-old boy with bilateral distal femoral unicameral bone cysts (UBCs) associated with acquired generalized lipodystrophy. As opposed to congenital generalized lipodystrophy, cystic bone lesions in acquired generalized lipodystrophy are rare. After radiographic and histologic confirmation of the UBCs, we performed percutaneous intramedullary decompression, curettage, and grafting. UBCs can be an important manifestation of acquired generalized lipodystrophy. Cystic bone lesions appear to be less common in acquired generalized lipodystrophy than in congenital generalized lipodystrophy, and intramedullary adipose tissue loss may be a predisposing factor for the development of bone lesions in patients with acquired generalized lipodystrophy. When evaluating a patient with lipodystrophy, doctors should recognize the clinical course may include the development of UBCs.

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

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.

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.

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.

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)

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.

[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

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

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.

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...

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,

Congenital ossicular chain malformations with mobile stapes in children: Results in 17 cases.

Science.gov (United States)

Vincent, Robert; Wegner, Inge; Derks, Laura S M; Grolman, Wilko

2016-03-01

To prospectively evaluate surgical findings and hearing results in children undergoing surgery for congenital malformations of the ossicular chain with a mobile stapes footplate (Teunissen and Cremers class III malformations). A nonrandomized, nonblinded, prospective case series. Fifteen consecutive pediatric patients who underwent 17 surgical procedures for congenital ossicular malformations with a mobile stapes at a tertiary referral center were included. In 16 cases, postoperative pure-tone audiometry was available. The surgical technique for repair of the ossicular chain was dictated by the surgical findings at the time of surgery. The majority of the cases underwent ossiculoplasty. A Teflon piston, partial ossicular replacement prosthesis, or total ossicular replacement prosthesis was used in these cases. Associated surgical techniques included malleus relocation, Silastic banding, drilling out of a bony bridge, and a combination of these techniques. Hearing loss was evaluated using preoperative and postoperative four-frequency (0.5, 1, 2, and 4 kHz) audiometry. Air-conduction thresholds, bone-conduction thresholds, and air-bone gaps were measured. Postoperative audiometry was performed at 3, 6, 9, 12, 18, and 24 months after surgery and at a yearly interval thereafter. Overall, a postoperative air-bone gap closure to 10 dB or less was achieved in 63% of the included cases. A postoperative air-bone gap closure to 20 dB or less was achieved in 75%. Postoperative sensorineural hearing loss did not occur in any of the cases. Ossicular reconstruction is a feasible treatment option in children with congenital malformations of the ossicular chain with a mobile stapes footplate. 4. Laryngoscope, 126:682-688, 2016. © 2015 The American Laryngological, Rhinological and Otological Society, Inc.

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.

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.

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.

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.

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.

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

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.

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.)

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.

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...

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.

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.)

Conservative treatment of congenital false joint of shin in newborns and infants

Directory of Open Access Journals (Sweden)

O. V. Shchokin

2017-04-01

Full Text Available Congenital false joint of shin occurs in 1 case per 28 000 - 190 000 live births, but it is hard-to-treat and disabling disease. Objective: Improving treatment outcomes, reducing the number of surgical interventions for the treatment of congenital false joint of shin. Materials and Methods. The method is carried out using staged plaster casts with the gradual correction of deformities. When axis of the leg approximates to the normal one, axial load is added. First, it is done by tapping on the heel. When the child was 7-8 months old he was put on the legs and taught to walk. The plaster bandage is replaced by "Scotch cast" and "soft cast" bandage. The treatment lasted up to reaching of clinical effect – correction of shin deformation and absence of pathological mobility. Clinical effect must be confirmed by roentgenography which must demonstrate filling of false joint zone with bone tissue and restoration of intramedullary canal. During all the period of treating alternate courses of electrophoresis with calcium chloride, medical mud extracts and magnetic therapy are conducted. In the period from 1995 till 2015 in the Regional Zaporizhzhia Children Clinical Hospital 4 children (6 shins aged from 1 to 7 months with false joint of shin were treated using proposed method. Results and discussion. All 4 children (6 shins treated in clinic with proposed conservative method showed filling of false joint zone with bone tissue, restoration of intramedullary canal and significant extension of axis of the shin. The load on the leg in early terms results in compression of bone fragments (as in compression-distraction osteosynthesis, magnetic therapy, electrophoresis with calcium chloride and medical mud extracts promote active functioning of the muscles that improves regional blood supply and improves osteogenesis. Conclusions. Using the proposed method of treatment of congenital false joint of shins in newborns and infants can allow avoiding surgical

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

Science.gov (United States)

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

2013-05-01

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

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.

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.

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...

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

A Newborn with Congenital Mixed Phenotype Acute Leukemia After In Vitro Fertilization

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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.

Increased periodontal bone loss in temporarily B lymphocyte-deficient rats

DEFF Research Database (Denmark)

Klausen, B; Hougen, H P; Fiehn, N E

1989-01-01

In order to study the role of T lymphocytes and B lymphocytes in the development of marginal periodontitis, experiments were performed on specific-pathogen-free (SPF) rats with various immunologic profiles. The study comprised nude (congenitally T lymphocyte-deficient), thymus-grafted nude (T-lym......-lymphocyte deficiency did not interfere with the development of periodontal disease in this model, whereas a temporary and moderate reduction in B-lymphocyte numbers seemed to predispose for aggravation of periodontal bone loss.......In order to study the role of T lymphocytes and B lymphocytes in the development of marginal periodontitis, experiments were performed on specific-pathogen-free (SPF) rats with various immunologic profiles. The study comprised nude (congenitally T lymphocyte-deficient), thymus-grafted nude (T...... had significantly less periodontal bone support than controls. Anti-mu treated inoculated rats had significantly less periodontal bone support than nude and normal rats, whereas no difference was found between normal, nude, and thymus-grafted rats. It is concluded that permanent T...

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.)

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.

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

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

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

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

Congenital amusia: a cognitive disorder limited to resolved harmonics and with no peripheral basis

Science.gov (United States)

Cousineaua, Marion; Oxenham, Andrew J.; Peretz, Isabelle

2015-01-01

Pitch plays a fundamental role in audition, from speech and music perception to auditory scene analysis. Congenital amusia is a neurogenetic disorder that appears to affect primarily pitch and melody perception. Pitch is normally conveyed by the spectro-temporal fine structure of low harmonics, but some pitch information is available in the temporal envelope produced by the interactions of higher harmonics. Using 10 amusic subjects and 10 matched controls, we tested the hypothesis that amusics suffer exclusively from impaired processing of spectro-temporal fine structure. We also tested whether the inability of amusics to process acoustic temporal fine structure extends beyond pitch by measuring sensitivity to interaural time differences, which also rely on temporal fine structure. Further tests were carried out on basic intensity and spectral resolution. As expected, pitch perception based on spectro-temporal fine structure was impaired in amusics; however, no significant deficits were observed in amusics' ability to perceive the pitch conveyed via temporal-envelope cues. Sensitivity to interaural time differences was also not significantly different between the amusic and control groups, ruling out deficits in the peripheral coding of temporal fine structure. Finally, no significant differences in intensity or spectral resolution were found between the amusic and control groups. The results demonstrate a pitch-specific deficit in fine spectro-temporal information processing in amusia that seems unrelated to temporal or spectral coding in the auditory periphery. These results are consistent with the view that there are distinct mechanisms dedicated to processing resolved and unresolved harmonics in the general population, the former being altered in congenital amusia while the latter is spared. PMID:25433224

Congenital amusia: a cognitive disorder limited to resolved harmonics and with no peripheral basis.

Science.gov (United States)

Cousineau, Marion; Oxenham, Andrew J; Peretz, Isabelle

2015-01-01

Pitch plays a fundamental role in audition, from speech and music perception to auditory scene analysis. Congenital amusia is a neurogenetic disorder that appears to affect primarily pitch and melody perception. Pitch is normally conveyed by the spectro-temporal fine structure of low harmonics, but some pitch information is available in the temporal envelope produced by the interactions of higher harmonics. Using 10 amusic subjects and 10 matched controls, we tested the hypothesis that amusics suffer exclusively from impaired processing of spectro-temporal fine structure. We also tested whether the inability of amusics to process acoustic temporal fine structure extends beyond pitch by measuring sensitivity to interaural time differences, which also rely on temporal fine structure. Further tests were carried out on basic intensity and spectral resolution. As expected, pitch perception based on spectro-temporal fine structure was impaired in amusics; however, no significant deficits were observed in amusics' ability to perceive the pitch conveyed via temporal-envelope cues. Sensitivity to interaural time differences was also not significantly different between the amusic and control groups, ruling out deficits in the peripheral coding of temporal fine structure. Finally, no significant differences in intensity or spectral resolution were found between the amusic and control groups. The results demonstrate a pitch-specific deficit in fine spectro-temporal information processing in amusia that seems unrelated to temporal or spectral coding in the auditory periphery. These results are consistent with the view that there are distinct mechanisms dedicated to processing resolved and unresolved harmonics in the general population, the former being altered in congenital amusia while the latter is spared. Copyright © 2014 Elsevier Ltd. All rights reserved.

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.

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.

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.

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.

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.

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.)

Bone formation induced in an infant by systemic prostaglandin-E2 administration

DEFF Research Database (Denmark)

Jørgensen, H R; Svanholm, H; Høst, A

1988-01-01

We report a case of long-term systemic administration of prostaglandin E2 (PGE2) to a newborn infant with ductus-dependent congenital heart disease. After 46 days of treatment, radiography showed cortical hyperostosis of the long bones. The child died 62 days after discontinuation of prostaglandin...... treatment. Histologic examination of tubular bones showed hyperostosis presumably due to prostaglandin-induced rapid formation of primitive bone. The additional finding of extensive resorption of the outer cortical surface and bone formation at the inner surface suggested a reversible phase after...

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.)

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.)

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.)

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

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.

ALCOHOL AND BONE GROWTH: A Literary Appraisal II

African Journals Online (AJOL)

user

the agent ii, Maternal-embryonic exchange and iii,. The genotype ... drug is administered to the pregnant animal .... experimentally induced congenital skeletal defects in rat were achieved with a nutrient deficient treatment. This discovery was rather accidental, because .... the stem cells in the growth plates of long bones are.

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.

Digital evaluation of orbital development in chinese children with congenital microphthalmia.

Science.gov (United States)

Yang, Guang; Wang, Jing; Chang, Qinglin; Wang, Zhenchang; Geng, Yulei; Li, Dongmei

2012-09-01

To evaluate the asymmetry of bilateral orbital development in Chinese children with congenital microphthalmia and to provide a criterion for tailoring treatment timing and therapy. Retrospective cohort study. By combining multisection helical computerized tomography imaging with a computer-aided design system, we measured 38 children between 0 and 6 years of age with congenital microphthalmia and 70 normal children of the same age group. Variables were measured, including orbital volume, depth, width, and height and eyeball volume. Displacement of the orbital rims was calculated by mirroring the unaffected orbit across the midsagittal plane of body. Significant differences were observed between the orbital volume, eyeball volume, orbital width, and orbital height of the affected and unaffected sides of children with congenital microphthalmia (P development is evident primarily in the inferior and lateral rims, correlating mostly with zygomatic and then maxilla and frontal bone. The growth of the affected orbit slows down or even stagnates by 3 years of age. Intervention therapy before 3 years of age was critical. Copyright © 2012 Elsevier Inc. All rights reserved.

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.

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.

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

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

Chondroblastoma of the sphenoid bone

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Patrocíni, Tomas Gomes

2008-12-01

Full Text Available Introduction: Chondroblastoma is an uncommon cartilaginous benign neoplasm, highly destructive, which specifically appears in the epiphysis of long bones in young patients. Its occurrence is extremely rare in the cranial base, normally occurring in the temporal bone. Objective: To describe a rare case in a patient presenting with a sphenoid bone chondroblastoma that invaded the middle cranial cavity, submitted to a successful surgical resection, without recurrence after 2 years. Case Report: W.J.S, 37 years old, male, forwarded to the otorhinolaryngology service with persistent and strong otalgia for 3 months. He had normal otoscopy and without visible tumorations. The computerized tomography confirmed tumor mass in the left infra-temporal cavity, invading the middle cranial cavity. The biopsy suggested giant cells tumor. After wide resection by frontal approach via orbitozygomatic osteotomy. During the surgery, we confirmed tomographic statements and didn't find temporal bone involvement. The histopathological exam confirmed chondroblastoma. After 18 months after the surgery, he doesn't present with complaints, without motor, sensitive deficits or of cranial nerves and without recurrence tomographic signals. Conclusion: The importance of differential diagnosis of chondroblastoma is remarkable in the cranial base lesions and its therapeutic approach, whose objective must always be the major possible resection with the maximum function conservation.

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...

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

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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.

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

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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.

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

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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.

Developing effective automated feedback in temporal bone surgery simulation.

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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.

A unique case of Shwachman-Diamond syndrome presenting with congenital hypopituitarism.

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Jivani, Nurin; Torrado-Jule, Carmen; Vaiselbuh, Sarah; Romanos-Sirakis, Eleny

2016-11-01

Shwachman-Diamond syndrome (SDS) is an autosomal recessive bone marrow failure syndrome typically characterized by neutropenia and pancreatic dysfunction, although phenotypic presentations vary, and the endocrine phenotype is not well-described. We report a unique case of a patient with SDS who initially presented with hypoglycemia and micropenis in the newborn period and was diagnosed with congenital hypopituitarism. We are not aware of any other cases of SDS documented with this combination of complex endocrinopathies.

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

Clinical, Chromosomal and Endocrine Studies for Congenital Adrenal Hyperplasia

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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.

Heterozygous M1V variant of ELA-2 gene mutation associated with G-CSF refractory severe congenital neutropenia.

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Setty, Bhuvana A; Yeager, Nicholas D; Bajwa, Rajinder P

2011-09-01

Severe congenital neutropenia is an autosomal recessive disorder characterized by maturation arrest at the promyelocyte/myelocyte phase in the bone marrow, absolute neutrophil count ELA-2 have been described. We report the case of a premature male infant with congenital neutropenia, associated with multiple infections, refractory to treatment with granulocyte colony stimulating factor who subsequently underwent matched sibling donor stem-cell transplant. He was found to be heterozygous for the M1V variant of the ELA-2 gene that we postulate to be causative for his severe neutropenia Copyright © 2011 Wiley-Liss, Inc.

Bioprinting and Organ-on-Chip Applications Towards Personalized Medicine for Bone Diseases.

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Arrigoni, Chiara; Gilardi, Mara; Bersini, Simone; Candrian, Christian; Moretti, Matteo

2017-06-01

The skeleton supports and confers structure to the whole body but several pathological and traumatic conditions affect the bone tissue. Most of those pathological conditions are specific and different among different patients, such as bone defects due to traumatic injuries or bone remodeling alterations due to congenital diseases. In this context, the development of personalized therapies would be highly desirable. In recent years the advent of innovative techniques like bioprinting and microfluidic organ-on-chip raised hopes of achieving key tools helping the application of personalized therapies for bone diseases. In this review we will illustrate the latest progresses in the bioprinting of personalized bone grafts and generation of patient-specific bone-on-chip devices, describing current approaches and limitations and possible future improvements for more effective personalized bone grafts and disease models.

Sickle Cell Disease with Cyanotic Congenital Heart Disease: Long-Term Outcomes in 5 Children.

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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.

Case Report: Congenital Erythroleukemia in a Premature Infant with Dysmorphic Features.

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Helin, Heidi; van der Walt, Jon; Holder, Muriel; George, Simi

2016-01-01

We present a case of pure erythroleukemia, diagnosed at autopsy, in a dysmorphic premature infant who died of multiorgan failure within 24 hours of birth. Dysmorphic features included facial and limb abnormalities with long philtrum, microagnathia, downturned mouth, short neck as well as abnormal and missing nails, missing distal phalanx from the second toe, and overlapping toes. Internal findings included gross hepatomegaly and patchy hemorrhages in the liver, splenomegaly, and cardiomegaly; and subdural, intracerebral, and intraventricular hemorrhages. Histology revealed infiltration of bone marrow, kidney, heart, liver, adrenal, lung, spleen, pancreas, thyroid, testis, thymus, and placenta by pure erythroleukemia. Only 6 cases of congenital erythroleukemia have been previously reported with autopsy findings similar to those of this case. The dysmorphic features, although not fitting any specific syndrome, make this case unique. Congenital erythroleukemia and possible syndromes suggested by the dysmorphic features are discussed.

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

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

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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

Anophthalmia, cleft lip/palate, absent vomer bone, nystagmus, and mental-motor retardation: a new syndrome or Fryns "anophthalmia-plus" syndrome?

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Ozçelik, Derya; Sağlam, Ibrahim; SIlan, Fatma; Sezen, Gülbin; Unveren, Toygar

2008-05-01

We report that a 4-year-old boy presented with right unilateral complete cleft lip and palate, right anophthalmos, left congenital nystagmus, absence of the vomer bone, mental-motor retardation, and normal lymphocyte karyotype (46, XY). For reconstruction of the deformities, we performed cleft lip repair by Millard's rotation-advancement technique and planned cleft palate repair. This combination of cleft lip and palate, anophthalmos, congenital nystagmus, absent vomer bone, and mental-motor retardation has not, to our knowledge, previously been described. We suggest that this represents either another case of the rare Fryns "anophthalmia-plus" syndrome or a new syndrome.

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...

Congenital Hypothyroidism

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... 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 ...

Clinical and morphological characteristics of malformations in infants with congenital cytomegalovirus infection and congenital toxoplasmosis

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L. Yu. Barycheva

2015-01-01

Full Text Available The results of following up infants with intrauterine infections and malformations were retrospectively analyzed. Infants with malformations were diagnosed as having congenital cytomegalovirus infection and congenital toxoplasmosis in 127 and 69 cases, respectively. The aim of the study was to characterize malformations in infants with congenital cytomegalovirus and congenital Toxoplasma infections. The infants with malformations in congenital cytomegalovirus infection were found to have higher mortality rates (61,4% than those with congenital toxoplasmosis (34,8%. Postmortem analysis indicated that there was a predominance of embryopathies in infants with congenital cytomegalovirus infection and that of fetopathies in those with congenital toxoplasmosis. The dead infants with congenital cytomegalovirus infection had more commonly developed visceral defects, including heart diseases, pneumopathies, gastrointestinal and genitourinary abnormalities; fetopathies of the central nervous system and eye were prevalent in congenital toxoplasmosis. The surviving children with congenital toxoplasmosis were more frequently observed to have disabling CNS and ocular sequels as obstructive hydrocephalus, infantile cerebral palsy, complete or partial blindness, and cerebrasthenic disorders than those with congenital cytomegalovirus infection. 

Mitochondrial disorders in congenital myopathies

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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.

[Surgery for intractable epilepsy in a patient with encephalocele of the temporal lobe: a case report].

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Semenov, M S; Belyakova-Bodina, A I; Murtazina, A F; Brutyan, A G; Golovteev, A L; Aziatskaya, G A; Samoylov, A S; Zabelin, M V; Udalov, Yu D

We describe a case of surgical treatment of intractable temporal epilepsy in a female patient with congenital middle cranial fossa encephalocele. We present clinical-anamnestic and neuroimaging data as well as the microscopic and macroscopic pictures of encephalocele. We analyze outcomes of surgery for this pathology, which have been reported in the literature. To date, there have been a few articles on this subject in the domestic literature. The development of neuroimaging techniques and a growing number of verified encephalocele cases promote the widespread use of surgery for treatment of intractable epilepsy. Congenital encephalocele should be considered in the differential diagnosis of intractable temporal epilepsy, and, if verified, surgical treatment is the method of choice in most cases.

Sphingolipids in Congenital Diaphragmatic Hernia; Results from an International Multicenter Study.

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Kitty G Snoek

Full Text Available Congenital diaphragmatic hernia is a severe congenital anomaly with significant mortality and morbidity, for instance chronic lung disease. Sphingolipids have shown to be involved in lung injury, but their role in the pathophysiology of chronic lung disease has not been explored. We hypothesized that sphingolipid profiles in tracheal aspirates could play a role in predicting the mortality/ development of chronic lung disease in congenital diaphragmatic hernia patients. Furthermore, we hypothesized that sphingolipid profiles differ between ventilation modes; conventional mechanical ventilation versus high-frequency oscillation.Sphingolipid levels in tracheal aspirates were determined at days 1, 3, 7 and 14 in 72 neonates with congenital diaphragmatic hernia, born after > 34 weeks gestation at four high-volume congenital diaphragmatic hernia centers. Data were collected within a multicenter trial of initial ventilation strategy (NTR 1310.36 patients (50.0% died or developed chronic lung disease, 34 patients (47.2% by stratification were initially ventilated by conventional mechanical ventilation and 38 patients (52.8% by high-frequency oscillation. Multivariable logistic regression analysis with correction for side of the defect, liver position and observed-to-expected lung-to-head ratio, showed that none of the changes in sphingolipid levels were significantly associated with mortality /development of chronic lung disease. At day 14, long-chain ceramides 18:1 and 24:0 were significantly elevated in patients initially ventilated by conventional mechanical ventilation compared to high-frequency oscillation.We could not detect significant differences in temporal sphingolipid levels in congenital diaphragmatic hernia infants with mortality/development of chronic lung disease versus survivors without development of CLD. Elevated levels of ceramides 18:1 and 24:0 in the conventional mechanical ventilation group when compared to high

Cytomegalovirus Congenital Cataract

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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.

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

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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

Epileptiform electroencephalogram abnormality in children with congenital sensorineural hearing loss.

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

2014-04-01

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

Multiple Bony Injuries on Bone Scan in a Case of Unsuspected Child Abuse

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Ya-Wen Chuang

2017-01-01

Full Text Available This case is described of an eleven-month-old infant with lower limbs swelling and the left elbow skeletal malformation following a fall. The radionuclide bone scan was performed to exclude bone infection or congenital skeletal anomaly. The images unexpectedly showed multiple increased radioactive foci throughout the whole body. It was a strong probability of child abuse. All lesions are readily apparent on the following plain film radiographs and MRI.

Petrous bone fracture: a virtual trauma analysis.

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Montava, Marion; Deveze, Arnaud; Arnoux, Pierre-Jean; Bidal, Samuel; Brunet, Christian; Lavieille, Jean-Pierre

2012-06-01

The temporal bone shields sensorineural, nervous, and vascular structures explaining the potential severity and complications of trauma related to road and sport accidents. So far, no clear data are available on the exact mechanisms involved for fracture processes. Modelization of structures helps to answer these concerns. Our objective was to design a finite element model of the petrous bone structure to modelize temporal bone fracture propagation in a scenario of lateral impact. A finite element model of the petrous bone structure was designed based on computed tomography data. A 7-m/s lateral impact was simulated to reproduce a typical lateral trauma. Results of model analysis was based on force recorded, stress level on bone structure up to induce a solution of continuity of the bony structure. Model simulation showed that bone fractures follow the main axes of the petrous bone and occurred in a 2-step process: first, a crush, and second, a massive fissuration of the petrous bone. The lines of fracture obtained by simulation of a lateral impact converge toward the middle ear region. This longitudinal fracture is located at the mastoid-petrous pyramid junction. Using this model, it was possible to map petrous bone fractures including fracture chronology and areas of fusion of the middle ear region. This technique may represent a first step to investigate the pathophysiology of the petrous bone fractures, aiming to define prognostic criteria for patients' care.

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

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.

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 ...

MR imaging of temporal lobe meningoencephalocele

International Nuclear Information System (INIS)

Tampieri, D.; Leblanc, R.; Melangon, D.; del-Carpio-O'Donovan, R.; Ethier, R.

1991-01-01

Basal meningoencephaloceles represent a rare entity, and they may be associated with a variety of midline cerebral abnormalities. The classification of basal meningoencephaloceles is related to their anatomic location. This paper reports experience in 3 patients, 2 who have temporal lobe epilepsy and a bone defect in the region of the foramen rotondum. In these 2 patients the encephalocele and its covering were protruding into the pterygopalatine fossa without any orbital involvement. The third patient presented with cerebrospinal fluid rhinorrhea caused by a transsphenoidal meningoencephalocele. MR imaging is the examination of choice for detecting these lesions since it allows for the visualization of the encephalocele and its meningeal covering as well as the bone defect and associated lesions in the temporal lobes

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.

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 ...

Altered retrieval of melodic information in congenital amusia: insights from dynamic causal modeling of MEG data.

Science.gov (United States)

Albouy, Philippe; Mattout, Jérémie; Sanchez, Gaëtan; Tillmann, Barbara; Caclin, Anne

2015-01-01

Congenital amusia is a neuro-developmental disorder that primarily manifests as a difficulty in the perception and memory of pitch-based materials, including music. Recent findings have shown that the amusic brain exhibits altered functioning of a fronto-temporal network during pitch perception and short-term memory. Within this network, during the encoding of melodies, a decreased right backward frontal-to-temporal connectivity was reported in amusia, along with an abnormal connectivity within and between auditory cortices. The present study investigated whether connectivity patterns between these regions were affected during the short-term memory retrieval of melodies. Amusics and controls had to indicate whether sequences of six tones that were presented in pairs were the same or different. When melodies were different only one tone changed in the second melody. Brain responses to the changed tone in "Different" trials and to its equivalent (original) tone in "Same" trials were compared between groups using Dynamic Causal Modeling (DCM). DCM results confirmed that congenital amusia is characterized by an altered effective connectivity within and between the two auditory cortices during sound processing. Furthermore, right temporal-to-frontal message passing was altered in comparison to controls, with notably an increase in "Same" trials. An additional analysis in control participants emphasized that the detection of an unexpected event in the typically functioning brain is supported by right fronto-temporal connections. The results can be interpreted in a predictive coding framework as reflecting an abnormal prediction error sent by temporal auditory regions towards frontal areas in the amusic brain.

Altered retrieval of melodic information in congenital amusia: Insights from Dynamic Causal Modeling of MEG data

Directory of Open Access Journals (Sweden)

Philippe eAlbouy

2015-02-01

Full Text Available Congenital amusia is a neuro-developmental disorder that primarily manifests as a difficulty in the perception and memory of pitch-based materials, including music. Recent findings have shown that the amusic brain exhibits altered functioning of a fronto-temporal network during pitch perception and memory. Within this network, during the encoding of melodies, a decreased right backward frontal-to-temporal connectivity was reported in amusia, along with an abnormal connectivity within and between auditory cortices. The present study investigated whether connectivity patterns between these regions were affected during the retrieval of melodies. Amusics and controls had to indicate whether sequences of six tones that were presented in pairs were the same or different. When melodies were different only one tone changed in the second melody. Brain responses to the changed tone in Different trials and to its equivalent (original tone in Same trials were compared between groups using Dynamic Causal Modeling (DCM. DCM results confirmed that congenital amusia is characterized by an altered effective connectivity within and between the two auditory cortices during sound processing. Furthermore, right temporal-to-frontal message passing was altered in comparison to controls, with an increase in Same trials and a decrease in Different trials. An additional analysis in control participants emphasized that the detection of an unexpected event in the typically functioning brain is supported by right fronto-temporal connections. The results can be interpreted in a predictive coding framework as reflecting an abnormal prediction error sent by temporal auditory regions towards frontal areas in the amusic brain.

Hydrogels That Allow and Facilitate Bone Repair, Remodeling, and Regeneration.

Science.gov (United States)

Short, Aaron R; Koralla, Deepthi; Deshmukh, Ameya; Wissel, Benjamin; Stocker, Benjamin; Calhoun, Mark; Dean, David; Winter, Jessica O

2015-10-28

Bone defects can originate from a variety of causes, including trauma, cancer, congenital deformity, and surgical reconstruction. Success of the current "gold standard" treatment (i.e., autologous bone grafts) is greatly influenced by insufficient or inappropriate bone stock. There is thus a critical need for the development of new, engineered materials for bone repair. This review describes the use of natural and synthetic hydrogels as scaffolds for bone tissue engineering. We discuss many of the advantages that hydrogels offer as bone repair materials, including their potential for osteoconductivity, biodegradability, controlled growth factor release, and cell encapsulation. We also discuss the use of hydrogels in composite devices with metals, ceramics, or polymers. These composites are useful because of the low mechanical moduli of hydrogels. Finally, the potential for thermosetting and photo-cross-linked hydrogels as three-dimensionally (3D) printed, patient-specific devices is highlighted. Three-dimensional printing enables controlled spatial distribution of scaffold materials, cells, and growth factors. Hydrogels, especially natural hydrogels present in bone matrix, have great potential to augment existing bone tissue engineering devices for the treatment of critical size bone defects.

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

Incidental detection of congenital Robertsonian translocation at diagnosis of Philadelphia chromosome-positive acute lymphocytic leukemia.

Science.gov (United States)

Yamaguchi, Tsukasa; Igarashi, Aiko; Kawamura, Machiko; Ozasa, Yuka; Yoshida, Masayuki; Kakihana, Kazuhiko; Sakamaki, Hisashi; Ohashi, Kazuteru

2015-05-01

A man in his early forties who had undergone 3 years of unsuccessful treatment for infertility due to oligospermia and asthenospermia developed fever and bone pain in December 20XX. He was subsequently diagnosed with acute lymphocytic leukemia. Conventional cytogenetic analysis revealed Robertsonian translocation (RT) with der(13;14)(q10;q10) in addition to the Philadelphia (Ph) chromosome. Dasatinib and prednisolone induced complete remission (CR) with disappearance of the Ph chromosome. However, RT persisted despite achieving CR. We speculate that RT is possibly congenital in our present case and might also have been responsible for the aforementioned infertility. Hematologists should be aware of the possibility that congenital chromosomal disorders might be found incidentally through diagnostic chromosome analysis for leukemia.

Constitutional bone impairment in Noonan syndrome.

Science.gov (United States)

Baldassarre, Giuseppina; Mussa, Alessandro; Carli, Diana; Molinatto, Cristina; Ferrero, Giovanni Battista

2017-03-01

Noonan syndrome (NS) is an autosomal dominant trait characterized by genotypic and phenotypic variability. It belongs to the Ras/MAPK pathway disorders collectively named Rasopathies or neurocardiofaciocutaneous syndromes. Phenotype is characterized by short stature, congenital heart defects, facial dysmorphisms, skeletal and ectodermal anomalies, cryptorchidism, mild to moderate developmental delay/learning disability, and tumor predisposition. Short stature and skeletal dysmorphisms are almost constant and several studies hypothesized a role for the RAS pathway in regulating bone metabolism. In this study, we investigated the bone quality assessed by phalangeal quantitative ultrasound (QUS) and the metabolic bone profiling in a group of patients with NS, to determine whether low bone mineralization is primary or secondary to NS characteristics. Thirty-five patients were enrolled, including 20 males (55.6%) and 15 females (44.5%) aged 1.0-17.8 years (mean 6.4 ± 4.5, median 4.9 years). Each patients was submitted to clinical examination, estimation of the bone age, laboratory assays, and QUS assessment. Twenty-five percent of the cohort shows reduced QUS values for their age based on bone transmission time. Bone measurement were adjusted for multiple factors frequently observed in NS patients, such as growth retardation, delayed bone age, retarded puberty, and reduced body mass index, potentially affecting bone quality or its appraisal. In spite of the correction attempts, QUS measurement indicates that bone impairment persists in nearly 15% of the cohort studied. Our results indicate that bone impairment in NS is likely primary and not secondary to any of the phenotypic traits of NS, nor consistent with metabolic disturbances. © 2017 Wiley Periodicals, Inc. © 2017 Wiley Periodicals, Inc.

The relationship between periodontal status and peripheral levels of neutrophils in two consanguineous siblings with severe congenital neutropenia: case reports.

Science.gov (United States)

Tözüm, Tolga Fikret; Berker, Ezel; Ersoy, Fügen; Tezcan, Iihan; Sanal, Ozden

2003-03-01

Congenital neutropenia is characterized by a severe reduction in absolute neutrophil counts, resulting in an almost total absence of neutrophils. It is well known that severe neutropenia affects periodontal status. Oral manifestations include ulcerations, gingival desquamation, gingival inflammation, attachment loss, and alveolar bone loss which may result in tooth loss. Treatment with granulocyte-colony stimulating factor (G-CSF) may improve this periodontal condition. This article reports the relationship between periodontal disease status and peripheral neutrophil levels in two consanguineous siblings with severe congenital neutropenia who did not receive routine G-CSF for 2 years prior to examination. Both siblings were given scaling, root planing, and periodontal prophylaxis in regular follow-up visits. This report demonstrates that periodontal therapy supported by adequate oral hygiene may result in restoration of neutrophil counts in siblings with congenital neutropenia.

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...

Comparing Life Quality Strategies and Emotion Regulation in People with Congenital and Non-Congenital Motor Disability

Directory of Open Access Journals (Sweden)

Seyedeh Zeynab Miraghaei

2017-10-01

Full Text Available Objective: The purpose of the present study was to compare emotion regulation strategies and life quality of people with congenital and non-congenital motor disabilities. Method: This study is a casual-comparative study and its population consisted of all people with congenital and non-congenital motor disability in Kahrizak Charity Foundation in Tehran in 2016. To conduct the study, available sampling method was used, and congenital and non-congenital disabled people were selected (200 people. To collect data, Cognitive Emotion Regulation Scale by Granovsky and life quality questionnaire were used. Research hypotheses were tested using multivariate analysis of variance. Results: The findings of this study showed that there is a significant difference between emotion regulation components in people with congenital and non-congenital disabilities (p<0.05. Also, according to the findings, a significant difference was observed between life quality dimensions (physical and mental health in people with congenital and non-congenital disabilities (p<0.05. Conclusion: According to the significant difference between two groups of subjects, necessary measures regarding consultation and psychotherapy should be taken into consideration to let people benefit from desirable mental health level.

Bilateral implant-retained auricular prosthesis for a patient with congenitally missing ears. A clinical report.

Science.gov (United States)

Kumar, Preeti Satheesh; Satheesh Kumar, K S; Savadi, Ravindra C

2012-06-01

Microtia is a major congenital anomaly of the external ear. It includes a spectrum of deformities from a grossly normal but small ear to the absence of the entire external ear. These deformities account for three in every 10,000 births, with bilaterally missing ears seen in fewer than 10% of all cases. Congenital abnormalities of the ear are unlikely to result in the complete absence of the ears, but the patient presented in this article had bilateral congenitally missing ears. There was loss of anatomic landmarks and alteration of normal bony architecture. Minimal tissue was available for retention; therefore, conventional techniques could not be used for achieving retention. A two-implant-supported auricular prosthesis was planned, but the patient was found to have deficient bone in the implant site. Hence the implants were placed posterior to these sites, and the superstructure was modified to accommodate for this change in position of the implant to ensure the esthetic positioning of the prosthesis. © 2012 by the American College of Prosthodontists.

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.

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

Altered topology of neural circuits in congenital prosopagnosia.

Science.gov (United States)

Rosenthal, Gideon; Tanzer, Michal; Simony, Erez; Hasson, Uri; Behrmann, Marlene; Avidan, Galia

2017-08-21

Using a novel, fMRI-based inter-subject functional correlation (ISFC) approach, which isolates stimulus-locked inter-regional correlation patterns, we compared the cortical topology of the neural circuit for face processing in participants with an impairment in face recognition, congenital prosopagnosia (CP), and matched controls. Whereas the anterior temporal lobe served as the major network hub for face processing in controls, this was not the case for the CPs. Instead, this group evinced hyper-connectivity in posterior regions of the visual cortex, mostly associated with the lateral occipital and the inferior temporal cortices. Moreover, the extent of this hyper-connectivity was correlated with the face recognition deficit. These results offer new insights into the perturbed cortical topology in CP, which may serve as the underlying neural basis of the behavioral deficits typical of this disorder. The approach adopted here has the potential to uncover altered topologies in other neurodevelopmental disorders, as well.

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....

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

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.

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

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.

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 ...

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 ...

[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

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.

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

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

Is Congenital Amusia a Disconnection Syndrome? A Study Combining Tract- and Network-Based Analysis

Directory of Open Access Journals (Sweden)

Jieqiong Wang

2017-09-01

Full Text Available Previous studies on congenital amusia mainly focused on the impaired fronto-temporal pathway. It is possible that neural pathways of amusia patients on a larger scale are affected. In this study, we investigated changes in structural connections by applying both tract-based and network-based analysis to DTI data of 12 subjects with congenital amusia and 20 demographic-matched normal controls. TBSS (tract-based spatial statistics was used to detect microstructural changes. The results showed that amusics had higher diffusivity indices in the corpus callosum, the right inferior/superior longitudinal fasciculus, and the right inferior frontal-occipital fasciculus (IFOF. The axial diffusivity values of the right IFOF were negatively correlated with musical scores in the amusia group. Network-based analysis showed that the efficiency of the brain network was reduced in amusics. The impairments of WM tracts were also found to be correlated with reduced network efficiency in amusics. This suggests that impaired WM tracts may lead to the reduced network efficiency seen in amusics. Our findings suggest that congenital amusia is a disconnection syndrome.

Is Congenital Amusia a Disconnection Syndrome? A Study Combining Tract- and Network-Based Analysis.

Science.gov (United States)

Wang, Jieqiong; Zhang, Caicai; Wan, Shibiao; Peng, Gang

2017-01-01

Previous studies on congenital amusia mainly focused on the impaired fronto-temporal pathway. It is possible that neural pathways of amusia patients on a larger scale are affected. In this study, we investigated changes in structural connections by applying both tract-based and network-based analysis to DTI data of 12 subjects with congenital amusia and 20 demographic-matched normal controls. TBSS (tract-based spatial statistics) was used to detect microstructural changes. The results showed that amusics had higher diffusivity indices in the corpus callosum, the right inferior/superior longitudinal fasciculus, and the right inferior frontal-occipital fasciculus (IFOF). The axial diffusivity values of the right IFOF were negatively correlated with musical scores in the amusia group. Network-based analysis showed that the efficiency of the brain network was reduced in amusics. The impairments of WM tracts were also found to be correlated with reduced network efficiency in amusics. This suggests that impaired WM tracts may lead to the reduced network efficiency seen in amusics. Our findings suggest that congenital amusia is a disconnection syndrome.

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

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.

The Pelvic Digit: A Rare Congenital Anomaly as a Cause of Hip Pain

OpenAIRE

Moreta-Su?rez, Jes?s; de Ugarte-Sobr?n, Oskar S?ez; S?nchez-Sobrino, Alberto; Mart?nez-De Los Mozos, Jos? Luis

2012-01-01

Introduction: The pelvic digit or pelvic rib is an unusual congenital anomaly with a finger or rib like bone formation in soft tissues around normal pelvic skeleton. This is a benign lesion and mostly an Incidental finding on radiographs. Most reported cases are asymptomatic and do not require intervention. We report a case of symptomatic pelvic rib that required surgical excision. Case Report: A 57-year-old man presented with a long history of pain and functional limitation in his right hip....

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.

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.

Burden and impact of congenital syndromes and comorbidities among adults with congenital heart disease.

Science.gov (United States)

Bracher, Isabelle; Padrutt, Maria; Bonassin, Francesca; Santos Lopes, Bruno; Gruner, Christiane; Stämpfli, Simon F; Oxenius, Angela; De Pasquale, Gabriella; Seeliger, Theresa; Lüscher, Thomas F; Attenhofer Jost, Christine; Greutmann, Matthias

2017-08-01

Our aim was to assess the overall burden of congenital syndromes and non-cardiac comorbidities among adults with congenital heart disease and to assess their impact on circumstances of living and outcomes. Within a cohort of 1725 adults with congenital heart defects (65% defects of moderate or great complexity) followed at a single tertiary care center, congenital syndromes and comorbidities were identified by chart review. Their association with arrhythmias, circumstances of living and survival was analyzed. Within the study cohort, 232 patients (13%) had a genetic syndrome, 51% at least one comorbidity and 23% ≥2 comorbidities. Most prevalent comorbidities were systemic arterial hypertension (11%), thyroid dysfunction (9%), psychiatric disorders (9%), neurologic disorders (7%), chronic lung disease (7%), and previous stroke (6%). In contrast to higher congenital heart defect complexity, the presence of comorbidities had no impact on living circumstances but patients with comorbidities were less likely to work full-time. Atrial arrhythmias were more common among patients with moderate/great disease complexity and those with comorbidities but were less common among patients with congenital syndromes (pCongenital syndromes and comorbidities are highly prevalent in adults with congenital heart disease followed at specialist centers and add to the overall complexity of care. The presence of these additional factors has an impact on living circumstances, is associated with arrhythmias and needs to be further explored as prognostic markers. Copyright © 2017 Elsevier B.V. All rights reserved.

Congenital cytomegalovirus infection

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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.

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.

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)

Prevalence of congenital anomalies in newborns with congenital heart disease diagnosis

International Nuclear Information System (INIS)

Egbe, Alexander; Lee, Simon; Ho, Deborah; Uppu, Santosh; Srivastava, Shubhika

2014-01-01

There is a known association between congenital heart disease (CHD) and other congenital anomalies (CA). These associations have been altered by changes in prenatal factors in recent time. We reviewed the largest database of inpatient hospitalization information and analyzed the current association between common CHD diagnoses and other congenital anomalies. Case-control study design. We reviewed the Nationwide Inpatient Sample (NIS) database from 1998 to 2008 and identified all live births with CHD diagnosis (case) and live births without CHD diagnosis (control). We compared prevalence of associated congenital anomalies between the case and control groups. Our cohort consisted of 97,154 and 12,078,482 subjects in the case and control groups, respectively. In the CHD population, prevalence of non-syndromic congenital anomaly (NSCA), genetic syndrome (GS), and overall extra-cardiac congenital anomaly (CA) were 11.4, 2.2, and 13.6%, respectively. In the control group, prevalence of NSCA, GS, and CA were 6.7, 0.3, and 7.0%, respectively. NSCA (odds ratio (OR): 1.88, confidence interval (CI): 1.73-1.94), GS (OR 2.52, CI 2.44-2.61), and overall CA (OR: 2.01, CI: 1.97-2.14) were strongly associated with CHD. Prevalence of GS and multiple organ-system CA decreased significantly over the study period. This is the largest and most comprehensive population-based study evaluating association between CHD and extra-cardiac malformation (ECM) in newborns. There was significant decrease in prevalence of GS and multiple CA over the study period

Congenital heart defects and extracardiac malformations.

Science.gov (United States)

Rosa, Rosana Cardoso M; Rosa, Rafael Fabiano M; Zen, Paulo Ricardo G; Paskulin, Giorgio Adriano

2013-06-01

To review the association between congenital heart defects and extracardiac malformations. Scientific articles were searched in the Medline, Lilacs, and SciELO databases, using the descriptors "congenital heart disease," "congenital heart defects," "congenital cardiac malformations," "extracardiac defects," and "extracardiac malformations." All case series that specifically explored the association between congenital heart defects and extracardiac malformations were included. Congenital heart diseases are responsible for about 40% of birth defects, being one of the most common and severe malformations. Extracardiac malformations are observed in 7 to 50% of the patients with congenital heart disease, bringing a greater risk of comorbidity and mortality and increasing the risks related to heart surgery. Different studies have attempted to assess the presence of extracardiac abnormalities in patients with congenital heart disease. Among the changes described, those of the urinary tract are more often reported. However, no study has evaluated all patients in the same way. Extracardiac abnormalities are frequent among patients with congenital heart disease, and patients with these alterations may present an increased risk of morbimortality. Therefore, some authors have been discussing the importance and cost-effectiveness of screening these children for other malformations by complementary exams.

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 ...

Heart morphogenesis gene regulatory networks revealed by temporal expression analysis.

Science.gov (United States)

Hill, Jonathon T; Demarest, Bradley; Gorsi, Bushra; Smith, Megan; Yost, H Joseph

2017-10-01

During embryogenesis the heart forms as a linear tube that then undergoes multiple simultaneous morphogenetic events to obtain its mature shape. To understand the gene regulatory networks (GRNs) driving this phase of heart development, during which many congenital heart disease malformations likely arise, we conducted an RNA-seq timecourse in zebrafish from 30 hpf to 72 hpf and identified 5861 genes with altered expression. We clustered the genes by temporal expression pattern, identified transcription factor binding motifs enriched in each cluster, and generated a model GRN for the major gene batteries in heart morphogenesis. This approach predicted hundreds of regulatory interactions and found batteries enriched in specific cell and tissue types, indicating that the approach can be used to narrow the search for novel genetic markers and regulatory interactions. Subsequent analyses confirmed the GRN using two mutants, Tbx5 and nkx2-5 , and identified sets of duplicated zebrafish genes that do not show temporal subfunctionalization. This dataset provides an essential resource for future studies on the genetic/epigenetic pathways implicated in congenital heart defects and the mechanisms of cardiac transcriptional regulation. © 2017. Published by The Company of Biologists Ltd.

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.

Congenital and acquired foot disorders and their roentgenographic examination

International Nuclear Information System (INIS)

Weber, M.

1986-01-01

In addition to general radiologic aspects there are special orthopedic considerations have in interpretation of X-rays of the foot. This is especially important for the diagnosis of orthopedic foot diseases. In clubfoot X-rays are useful for therapeutic planning and control. Even in the first months of life radiographs can show important disturbances of growth of the foot and displacement of the bones of the tarsus. In other congenital foot deformities X-rays are important for diagnostic reasons: they prove luxations or skeletal deformities. The most important acquired foot disease is the pronating foot. X-rays do not only show the amount of joint damage and structural changes of bones but also allow to draw conclusions to be drawn about the causes of static and dynamic changes of the foot skeleton. Functional diagnostic radiological investigation is of decisive importance for evaluation of infantile pronating foot. X-rays allow the differentiation to be made between physiological and pathologic changes. Subtle radiographic investigation is essential while planning operative treatment in childhood, as in adults. (orig.) [de

Dynamics in prevalence of Down syndrome in children with congenital heart disease.

Science.gov (United States)

Pfitzer, Constanze; Helm, Paul C; Rosenthal, Lisa-Maria; Berger, Felix; Bauer, Ulrike M M; Schmitt, Katharina Rl

2018-01-01

We assessed the dynamics in the prevalence of children with congenital heart disease (CHD) and Down syndrome in Germany with regard to phenotype, severity, and gender. Data from patients with CHD and Down syndrome born between 1980 and 2014 were analyzed, who are registered with the German National Register for Congenital Heart Defects. One thousand six hundred eighteen CHD patients with Down syndrome were identified. The prevalence of children born with both Down syndrome and CHD was constant from 2005 to 2009 but increased from 2010 to 2014. Regarding CHD groups, complex and simple lesions have become more equal since 2005. The number of simple lesions with shunt has a peak prevalence in the period of 2010-2014. Atrioventricular septal defect was the most common CHD phenotype, but temporal changes were found within the group of CHD phenotypes over the observation period. Our findings suggest a growing number of CHD and Down syndrome, which may be the result of improved medical management and progress in educational, social, and financial support. This development is noteworthy as it adds new aspects to present discussions in the media and political settings. What is known: • Congenital heart disease is regarded to be the most important clinical phenomenon in children with Down syndrome, due to its significant impact on morbidity and mortality. • New developments in prenatal diagnostic and therapy management of congenital heart disease continue to influence the number of patients diagnosed with congenital heart disease and Down syndrome. What is New: • This study provides essential data giving the first overview of the dynamics in the prevalence of congenital heart disease and Down syndrome over an extended length of time up to 2015 in a large patient cohort, taking recent developments into account. • Our data suggest a growing prevalence of congenital heart disease and Down syndrome, which may be the result of improved medical management for Down syndrome

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

OpenAIRE

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

2016-01-01

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

The bone scan in tarsal coalition: a case report

International Nuclear Information System (INIS)

De Lima, R.T.; Mishkin, F.S.

1996-01-01

Tarsal coalition is abnormal fusion of two or more tarsal bones. The union may be fibrous, cartilaginous, or osseous and can be congenital or acquired in response to infection, articular disorders, trauma, or surgery. We report a case of fibrous talocalcaneal coalition in a 15-year-old boy in whom bone scintigraphy employing pinhole lateral views confirmed the clinical diagnosis when plain radiographs showed minimal changes and computed tomography was equivocal. The diagnosis of symptomatic tarsal coalition is important in that it is a common remediable cause of peroneal spastic flat foot, a frequently encountered condition. Scintigraphy provides important information about the presence and localization of this condition. (orig.). With 3 figs

The bone scan in tarsal coalition: a case report

Energy Technology Data Exchange (ETDEWEB)

De Lima, R.T. [Department of Radiology, Division of Nuclear Medicine, Harbor-UCLA Medical Center, Torrance, CA (United States); Mishkin, F.S. [Department of Radiology, Division of Nuclear Medicine, Harbor-UCLA Medical Center, Torrance, CA (United States)

1996-10-01

Tarsal coalition is abnormal fusion of two or more tarsal bones. The union may be fibrous, cartilaginous, or osseous and can be congenital or acquired in response to infection, articular disorders, trauma, or surgery. We report a case of fibrous talocalcaneal coalition in a 15-year-old boy in whom bone scintigraphy employing pinhole lateral views confirmed the clinical diagnosis when plain radiographs showed minimal changes and computed tomography was equivocal. The diagnosis of symptomatic tarsal coalition is important in that it is a common remediable cause of peroneal spastic flat foot, a frequently encountered condition. Scintigraphy provides important information about the presence and localization of this condition. (orig.). With 3 figs.

Congenital transverse deficiency of the tibia and fibula: a report of two cases

International Nuclear Information System (INIS)

Yasui, N.; Nakase, T.; Sahara, W.; Ochi, T.; Okumura, E.; Sato, M.

2000-01-01

We report two similar, but unrelated, patients with congenital bilateral partial deficiencies of the tibia and fibula associated with intact feet. In both patients, the tibia and fibula were absent on initial radiographs, while the femur and the tarsal bones were well developed and there was bilateral teratologic dislocation of the hips. Ultrasound and magnetic resonance imaging (MRI) studies suggested the presence of cartilaginous remnants of the tibia and fibula. There were multidirectional instabilities in the knees and ankles. The clinical and radiological features of these cases are distinct from those of congenital longitudinal deficiency of the tibia, in which the fibula is always preserved, and from longitudinal deficiency of the fibula, in which the tibia is present and the foot is usually involved. We suggest that the bilateral partial deficiencies of the tibia and fibula associated with the intact foot and teratologic dislocation of the hips is a single-entity disorder, possibly categorized as an intercalary transverse deficiency of the lower limb. (orig.)

Giant cell reparative granuloma of the occipital bone

International Nuclear Information System (INIS)

Santos-Briz, A.; Ricoy, J.R.; Martinez-Tello, F.J.; Lobato, R.D.; Ramos, A.; Millan, J.M.

2003-01-01

Giant cell reparative granuloma (GCRG) is a non-neoplastic fibrous lesion with unevenly distributed multinucleated giant cells, areas of osseous metaplasia and hemorrhage. The small bones of the hands and feet are the most common sites, followed by the vertebral bodies and craniofacial bones. In the craniofacial bones GCRG has been reported in the temporal bone, in the frontal bone and paranasal sinus. However, to the best of our knowledge no case has been reported in the occipital bone. We report on the imaging findings and pathological features of a GCRG of the occipital bone and discuss the differential diagnosis of this entity in this particular location, especially with giant cell tumor because of the therapeutic and prognostic implications. (orig.)

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

[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.

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 ...

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.

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

Congenital Ewing's Sarcoma/Peripheral Primitive Neuroectodermal Tumor: A Case Report and Review of the Literature

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Shu-Guang Jin

2016-10-01

Full Text Available Ewing's sarcoma (EWS and peripheral primitive neuroectodermal tumor (pPNET are small round cell malignancies that develop in soft tissue and bone. They very rarely affect newborns. A diagnosis of EWS/pPNET depends mainly on immunohistochemistry and molecular/genetic assays. Since these tumors are highly aggressive, patient prognosis is typically very poor, and treatment remains a challenge. Here, we report a 13-day-old newborn diagnosed with congenital EWS/pPNET and describe its treatment.

Fractionating the musical mind: insights from congenital amusia.

Science.gov (United States)

Stewart, Lauren

2008-04-01

Music, like language, is acquired effortlessly in early life and fulfils a multitude of social, cultural and emotional functions. However, those with a disorder recently termed 'congenital amusia' (CA) fail to recognise common tunes from their culture, do not hear when notes are 'out of tune' and sometimes report that music sounds like a 'din' or 'banging'. The core deficit appears to be a problem in discriminating pitch direction, a building block for the representation of melodic contour. Familial studies suggest the disorder is heritable and associated with structural differences in temporal and frontal cortices. The disorder provides a window onto the neuro-cognitive architecture of musical processing, and the possible etiologies of disordered development.

Left-Sided Congenital Diaphragmatic Hernia with Multiple Congenital Cardiac Anomalies, Hernia Sac, and Microscopic Hepatic Heterotopia: A Case Report

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Maria Arafah

2011-01-01

Full Text Available Congenital diaphragmatic hernia is a common congenital anomaly of uncertain etiology. Its association with multiple congenital anomalies in various organs is well recognized and antenatal radiological evidence of congenital diaphragmatic hernia warrants thorough evaluation to detect other anomalies, some of which can be life threatening. Rarely, heterotopic hepatic tissue is identified in the hernia, a rare pathological finding, exhibiting more than one macroscopic and microscopic characteristics, and always associated with cardiac congenital anomalies. Herein, we report a case of left-sided microscopic heterotopic hepatic tissue in a congenital diaphragmatic hernia in an infant with multiple cardiac congenital anomalies, but with preserved pericardium.

Congenital Heart Information Network

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... 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 © ...

Partial recovery of visual-spatial remapping of touch after restoring vision in a congenitally blind man.

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Ley, Pia; Bottari, Davide; Shenoy, Bhamy H; Kekunnaya, Ramesh; Röder, Brigitte

2013-05-01

In an initial processing step, sensory events are encoded in modality specific representations in the brain but seem to be automatically remapped into a supra-modal, presumably visual-external frame of reference. To test whether there is a sensitive phase in the first years of life during which visual input is crucial for the acquisition of this remapping process, we tested a single case of a congenitally blind man whose sight was restored after the age of two years. HS performed a tactile temporal order judgment task (TOJ) which required judging the temporal order of two tactile stimuli, one presented to each index finger. In addition, a visual-tactile cross-modal congruency task was run, in which spatially congruent and spatially incongruent visual distractor stimuli were presented together with tactile stimuli. The tactile stimuli had to be localized. Both tasks were performed with an uncrossed and a crossed hand posture. Similar to congenitally blind individuals HS did not show a crossing effect in the tactile TOJ task suggesting an anatomical rather than visual-external coding of touch. In the visual-tactile task, however, external remapping of touch was observed though incomplete compared to sighted controls. These data support the hypothesis of a sensitive phase for the acquisition of an automatic use of visual-spatial representations for coding tactile input. Nonetheless, these representations seem to be acquired to some extent after the end of congenital blindness but seem to be recruited only in the context of visual stimuli and are used with a reduced efficiency. Copyright © 2013 Elsevier Ltd. All rights reserved.

Early detection of congenital syphilis

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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.

Clinical review of inner ear malformation

International Nuclear Information System (INIS)

Kokai, Hiromi; Oohashi, Masami; Ishikawa, Kazuo; Harada, Kouji; Hiratsuka, Hitoshi; Ogasawara, Makoto; Miyashita, Souji; Terayama, Yoshihiko

2003-01-01

We had 126 patients with inner ear malformation diagnosed with temporal bone computed tomography (CT) scans at Azabu Triology Hospital between 1996 and 2002. We classified cases of inner ear malformation according to Jackler et al. The incidence of inner ear malformation in our series was as follows; labyrinthine anomalies 61% (isolated lateral semicircular canal dysplasia 56%, compound semicircular canal dysplasia 4%, semicircular canal aplasia 1%), cochlear anomalies 24%, enlargement of the vestibular aqueduct 12%, narrow internal auditory canal 2%, complete labyrinthine aplasia 1%, enlargement of the cochlear aqueduct 0%. The most frequent anomaly was isolated lateral semicircular canal dysplasia. We did not detect any significant clinical features in this anomaly. There were 2 patients with cochlear anomalies who had past histories of meningitis. Some patients with enlargement of the vestibular aqueduct had frequent attacks of fluctuating hearing. Clinically it is important to detect patients with inner ear malformation such as cochlear anomalies and enlargement of the vestibular aqueduct usually accompanied by congenital sensorineural hearing loss. For patients with congenital sensorineural hearing loss, we recommend temporal bone CT scan. (author)

Genetics Home Reference: critical congenital heart disease

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... 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 ...

Congenital Anorectal Malformation Severity Does Not Predict Severity of Congenital Heart Defects

NARCIS (Netherlands)

Jonker, Jara E.; Liem, Eryn T.; Elzenga, Nynke J.; Molenbuur, Bouwe; Trzpis, Monika; Broens, Paul M. A.

2016-01-01

Objective To determine the prevalence of congenital heart defects (CHDs) in patients with mild or severe congenital anorectal malformations (CARMs), and whether all patients with CARM need pediatric cardiology screening. Study design We included 129 patients with CARM born between 2004 and 2013, and

Congenital pseudarthrosis of lower leg treated by almost outdated method: Case report

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Vukašinović Zoran

2014-01-01

Full Text Available Introduction. Congenital pseudarthrosis of tibia is a rare congenital deformity with progressive evolution. Treatment is vague and difficult, and many methods have been used - from once mandatory early amputation to contemporary operative (Ilizarov method, free microvascular fibular graft and adjuvant methods (electrostimulation, biphosphonates, bone morphogenetic protein. We present the usage of once popular method of homologous graft insertion and intramedullary fixation. Case Outline. This is a case report of male patient with pseudarthrosis involving both crural bones (Boyd type 5, diagnosed in neonatal age. Early conservative treatment was unsuccessful, so child never initiated gait. At the age of three and a half years, operative treatment was applied: resection of pseudarthrosis on both tibia and fibula, and osteoplasty of tibia using cylindric homologous graft and intramedullary fixation with transtarsal Steinman pin, followed by long leg cast immobilization. Pin was removed after ten months, and physical therapy was initiated 1.5 year after surgery, with initial to partial weight bearing and short leg cast throughout another year. Two and a half years after surgery complete union of graft was documented, and then full weight bearing was allowed. At final visit, five years and three months after surgery, shin axis was correct, leg lengths were equal, and child had normal walk with full range of motion. X-ray showed complete union of both tibia and fibula. Conclusion. Despite bad prognostic factors (young age, severe deformity, utilization of obsolete and almost forgotten treatment methods can provide excellent result. [Projekat Ministarstva nauke Republike Srbije, br. III 41004

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

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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.

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

Contribution of G.A. Ilizarov to bone reconstruction: historical achievements and state of the art

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Alexander V. Gubin

2016-07-01

Full Text Available Abstract Methodological solutions of Prof. G.A. Ilizarov are the core stone of the contemporary bone lengthening and reconstruction surgery. They have been acknowledged in the orthopaedic world as one of the greatest contributions to treating bone pathologies. The Ilizarov method of transosseous compression–distraction osteosynthesis has been widely used for managing bone non-union and defects, bone infection, congenital and posttraumatic limb length discrepancies, hand and foot disorders. The optimal conditions for implementing distraction and compression osteogenesis were proven by numerous experimental studies that Prof. G.A. Ilizarov organized and supervised at a large orthopaedic research institute in Kurgan. The tension stress effect on regeneration and growth of tissues was thoroughly investigated with radiographic, histological and biochemical methods. The impact of the Ilizarov method on the progress of bone lengthening and reconstruction surgery could be called revolutionary.

Adult congenital heart disease imaging with second-generation dual-source computed tomography: initial experiences and findings.

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Ghoshhajra, Brian B; Sidhu, Manavjot S; El-Sherief, Ahmed; Rojas, Carlos; Yeh, Doreen Defaria; Engel, Leif-Christopher; Liberthson, Richard; Abbara, Suhny; Bhatt, Ami

2012-01-01

Adult congenital heart disease patients present a unique challenge to the cardiac imager. Patients may present with both acute and chronic manifestations of their complex congenital heart disease and also require surveillance for sequelae of their medical and surgical interventions. Multimodality imaging is often required to clarify their anatomy and physiology. Radiation dose is of particular concern in these patients with lifelong imaging needs for their chronic disease. The second-generation dual-source scanner is a recently available advanced clinical cardiac computed tomography (CT) scanner. It offers a combination of the high-spatial resolution of modern CT, the high-temporal resolution of dual-source technology, and the wide z-axis coverage of modern cone-beam geometry CT scanners. These advances in technology allow novel protocols that markedly reduce scan time, significantly reduce radiation exposure, and expand the physiologic imaging capabilities of cardiac CT. We present a case series of complicated adult congenital heart disease patients imaged by the second-generation dual-source CT scanner with extremely low-radiation doses and excellent image quality. © 2012 Wiley Periodicals, Inc.

A neonatal screening of congenital hypothyroidism covered 266 401 cases in Tianjin area

International Nuclear Information System (INIS)

Fang Peihua; Lv Mei; Huang Guangyu; Yuan Chengyun; Xu Jing; Yang Qingyan; Wang Xiuying; Ma Xiancheng; Liu Geli; Chen Kunming; Zhang Moling

2003-01-01

Objective: To observe the incidence, the curative rate and the long term prognosis of congenital hypothyroidism (CH) newborns detected by screening program in Tianjin area initiated at the beginning of 1982. Methods: Primarily, the T 4 and thyroid-stimulating hormone (TSH) radioimmunoassay (RIA) of dried blood sample on filter paper developed in our laboratory were used. The TSH RIA was replaced by a time-resolved fluorescence immunoassay (Tr-FIA) in 1998. The primary T 4 RIA was replaced by a commercial kit for T 4 RIA in 1999. SPECT imaging on thyroid was performed after intravenous administration of 99 Tc m O 4 - 11.1-18.5 MBq. Results: A total of 266 401 neonates was screened for CH in our laboratory in Tianjin area. 36 cases of permanent CH were confirmed in the program. The incidence of CH was 0.014%; 22 cases of CH here were kept in treatment . Of the 22 cases, 19 cases were recalled in 2000, 18 of them (94.7%) showed currently with normal growth and development in the check-up. Imaging on thyroid ( 99 Tc m ): among 19 patients with CH, 1 case was found with normal gland, 1 with a hypogenetic thyroid, 3 cases with enlarged thyroid, 7 with ectopic gland, and the remaining 7 cases didn't show any image of thyroid. The bone age of 20 CH children was evaluated with the X ray radiography. In 6 cases of them, the bone age was normal, and 7 cases had progressed from development delay to normal. So far, retarded bone age of the remaining 7 CH patients didn't show any renewing yet. The bone age renewal was found in 3 younger children of them, but for the other 3 cases (9-12 years old) of CH patients with thyroid gland absence, the renewal of bone age was slower. Intelligence quotient (IQ) in 16 cases was measured. The scores of IQ in 13 cases of them (81.3%) were 80-119, 2 cases 72-77, 1 case 60. IQs of 6/16 cases of CH patients with thyroid absence were lower. May be it related to that, their hypothyroidism during fetal life was severer. Conclusions: Neonatal

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...

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 ...

Neural bases of congenital amusia in tonal language speakers.

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Zhang, Caicai; Peng, Gang; Shao, Jing; Wang, William S-Y

2017-03-01

Congenital amusia is a lifelong neurodevelopmental disorder of fine-grained pitch processing. In this fMRI study, we examined the neural bases of congenial amusia in speakers of a tonal language - Cantonese. Previous studies on non-tonal language speakers suggest that the neural deficits of congenital amusia lie in the music-selective neural circuitry in the right inferior frontal gyrus (IFG). However, it is unclear whether this finding can generalize to congenital amusics in tonal languages. Tonal language experience has been reported to shape the neural processing of pitch, which raises the question of how tonal language experience affects the neural bases of congenital amusia. To investigate this question, we examined the neural circuitries sub-serving the processing of relative pitch interval in pitch-matched Cantonese level tone and musical stimuli in 11 Cantonese-speaking amusics and 11 musically intact controls. Cantonese-speaking amusics exhibited abnormal brain activities in a widely distributed neural network during the processing of lexical tone and musical stimuli. Whereas the controls exhibited significant activation in the right superior temporal gyrus (STG) in the lexical tone condition and in the cerebellum regardless of the lexical tone and music conditions, no activation was found in the amusics in those regions, which likely reflects a dysfunctional neural mechanism of relative pitch processing in the amusics. Furthermore, the amusics showed abnormally strong activation of the right middle frontal gyrus and precuneus when the pitch stimuli were repeated, which presumably reflect deficits of attending to repeated pitch stimuli or encoding them into working memory. No significant group difference was found in the right IFG in either the whole-brain analysis or region-of-interest analysis. These findings imply that the neural deficits in tonal language speakers might differ from those in non-tonal language speakers, and overlap partly with the

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

NARCIS (Netherlands)

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

2003-01-01

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

Congenital syphilis in the skeleton of a child from Poland (Radom, 18th–19th century AD

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Tomczyk Jacek

2015-03-01

Full Text Available An incomplete skeleton of a 3-year-old child with suspected congenital syphilis was found in the Radom area of Poland. Squama frontalis and zygomatic bones are characterized by significant bone loss. Radiographic pictures show a geographic destructive lesion of a serpiginous shape surrounded by a zone of reactive osteosclerosis in the squama frontalis. The radiographic findings included a slight widening and contour irregularities of the distal humeral metaphyses. The appearance of teeth did not suggest Hutchinson teeth, but the examination of the permanent molars showed signs of mulberry molars. Two teeth were tested for the presence of mercury. Chemical analysis did not indicate mercury accumulation (enamel: 0.07 μg/g, dentine: 0.14 μg/g, bone: 0.11 μg/g. Mercury values obtained for the examined samples were similar to those that are typical of healthy teeth in today’s individuals.

[Analyses of the clinical characteristics of unilateral conductive hearing loss with intact tympanic membrane].

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Tang, Chaoying; Zhang, Jishuai; Han, Weiju; Shen, Weidong; Liu, Jun; Hou, Zhaohui; Dai, Pu; Yang, Shiming; Han, Dongyi

2016-05-01

To analyze the clinical characteristics of unilateral conductive hearing loss with intact tympanic membrane, and summarize the key diagnostic points, differential diagnosis and observe the effects of surgical treatment. We reviewed data from 82 patients with unilateral conductive hearing loss with intact tympanic membranes who accepted the exploratory tympanotomy from April 2011 to September 2013. There were 41 males and 41 females, aged from 7 to 66( averaged 26.5±13.7)years, with a history of one month to 50 years. The history, clinical symptoms, audiological evaluation, high resolution temporal bone CT, the results of surgical exploration and hearing reconstruction were analyzed. The exploratory tympanotomy revealed 43 cases of congenital middle ear malformations (52.4%), 22 cases of otosclerosis (26.8%), eight cases of congenital cholesteatoma (9.8%), six cases of trauma induced conductive hearing loss (7.3%), three cases of congenital ossicular malformations with congenital cholesteatoma (3.7%). Progressive hearing loss was common in patients with otosclerosis and congenital cholesteatoma, and patients with congenital middle ear malformations described their hearing loss since childhood. High resolution temporal bone CT of congenital middle ear malformation, trauma induced conductive hearing loss, congenital cholesteatoma diagnosis rate was 40.0%, 50.0%, and 83.3% respectively. The preoperative air-conductive threshold of patients with absence of the oval window were increased to (66.9±1.1)dBHL, the preoperative bone-conductive threshold achieved (28.3±10.4)dBHL at 2 000 Hz. While patients with stapes fixation and that with ossicular chain discontinuity were (27.2±9.7)dBHL and (17.8±8.8)dBHL(P=0.000)respectively. Through the tympanic exploration with endaural incision under the microscope, different hearing reconstruction were applied according to different lesions. After the operation, the hearing level of 52 patients with return visit were improved, the

Future of bone pathology, bone grafting, and osseointegration in oral and maxillofacial surgery: how applying optical advancements can help both fields

Science.gov (United States)

Tandon, Rahul; Herford, Alan S.

2013-03-01

Introduction: In recent years, advances in technology are propelling the field of oral and maxillofacial surgery into new realms. With a relatively thin alveolar mucosa overlying the underlying bone, significant diagnostic and therapeutic advantages are present. However, there remains an enormous gap between advancements in physics, in particular optics, and oral and maxillofacial surgery. Bone Pathology: Improvements in diagnosis, classification, and treatment of the various bone pathologies are still being sought after as advancements in technology continue to progress. Combining the clinical, histological, and pathological characteristics with these advancements, patients with debilitating pathologies may have more promising treatment options and prognosis. Bone Grafting: Defects in the facial bones, in particular the jaws, may be due to a number of reasons: pathology, trauma, infections, congenital deformities, or simply due to atrophy. Bone grafting is commonly employed to correct such defects, and allows new bone formation through tissue regeneration. Osseointegration: Growing use of dental implants has focused attention on osseointegration and its process. Osseointegration refers to the actual process of the direct contact between bone and implant, without an intervening soft tissue layer. The theories proposed regarding this process are many, yet there lacks a clear, unified stance on the actual process and its mechanisms. Further investigation using optical probes could provide that unifying answer. Conclusion: The primary goal of this lecture is to introduce pioneers in the field of optics to the field of oral and maxillofacial surgery. With a brief introduction into the procedures and techniques, we are hopeful to bridge the ever-widening gap between the clinical science and the basic sciences.

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)

Congenital adrenal hyperplasia: Treatment and outcomes

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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.

[Petrous bone fracture. Our experience: 1999-2004].

Science.gov (United States)

Ramírez Sabio, J B; de Paula Vernetta, C; García Sanchís, J M; Callejo García, F J; Cortés Andrés, O; Quilis Quesada, V; Dualde Beltrán, D; Marco Algarra, J

2006-12-01

To review the petrous bone fractures during the last five years (1999-2004) in our hospital, its manage, control, and analysis onf the associated factors. To analyse the managing protocoles and current bibliography. We review 266 temporal bone fractures, 74 with petrous bone association. We analyse these fractures by sex distribution, injurie severity, otorhinolaryngological clinical findings, production mechanism and radiological findings. The cases are discussed and compared with current bibliography. Petrous bone fractures must be always suspected in patients with head trauma, specially if it associates severity and otorrhagia. It is necessary a deep colaboration between neurosurgeons, radiologists and otorhinolaryngologists to obtain a good management, control and follow up of the patients.

Congenital nystagmus and negative electroretinography

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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

In vivo analysis of biocompatibility and vascularization of the synthetic bone grafting substitute NanoBone.

Science.gov (United States)

Abshagen, K; Schrodi, I; Gerber, T; Vollmar, B

2009-11-01

One of the major challenges in the application of bone substitutes is adequate vascularization and biocompatibility of the implant. Thus, the temporal course of neovascularization and the microvascular inflammatory response of implants of NanoBone (fully synthetic nanocrystalline bone grafting material) were studied in vivo by using the mouse dorsal skinfold chamber model. Angiogenesis, microhemodynamics, and leukocyte-endothelial cell interaction were analyzed repetitively after implantation in the center and in the border zone of the implant up to 15 days. Both NanoBone granules and plates exhibited high biocompatibility comparable to that of cancellous bone, as indicated by a lack of venular leukocyte activation after implantation. In both synthetic NanoBone groups, signs of angiogenesis could be observed even at day 5 after implantation, whereas granules showed higher functional vessel density compared with NanoBone plates. The angiogenic response of the cancellous bone was markedly accelerated in the center of the implant tissue. Histologically, implant tissue showed an ingrowth of vascularized fibrous tissue into the material combined with an increased number of foreign-body giant cells. In conclusion, NanoBone, particularly in granular form, showed high biocompatibility and high angiogenic response, thus improving the healing of bone defects. Our results underline that, beside the composition and nanostructure, the macrostructure is also of importance for the incorporation of the biomaterial by the host tissue. (c) 2008 Wiley Periodicals, Inc.

Bone age detection via carpogram analysis using convolutional neural networks

Science.gov (United States)

Torres, Felipe; Bravo, María. Alejandra; Salinas, Emmanuel; Triana, Gustavo; Arbeláez, Pablo

2017-11-01

Bone age assessment is a critical factor for determining delayed development in children, which can be a sign of pathologies such as endocrine diseases, growth abnormalities, chromosomal, neurological and congenital disorders among others. In this paper we present BoneNet, a methodology to assess automatically the skeletal maturity state in pediatric patients based on Convolutional Neural Networks. We train and evaluate our algorithm on a database of X-Ray images provided by the hospital Fundacion Santa Fe de Bogot ´ a with around 1500 images of patients between the ages 1 to 18. ´ We compare two different architectures to classify the given data in order to explore the generality of our method. To accomplish this, we define multiple binary age assessment problems, dividing the data by bone age and differentiating the patients by their gender. Thus, exploring several parameters, we develop BoneNet. Our approach is holistic, efficient, and modular, since it is possible for the specialists to use all the networks combined to determine how is the skeletal maturity of a patient. BoneNet achieves over 90% accuracy for most of the critical age thresholds, when differentiating the images between over or under a given age.

The shortened cochlea: its classification and histopathologic features.

Science.gov (United States)

Zheng, Yiqing; Schachern, Patricia A; Cureoglu, Sebahattin; Mutlu, Cemil; Dijalilian, Hamid; Paparella, Michael M

2002-03-15

The term 'Mondini dysplasia' has been used to describe virtually any congenital abnormality of the osseous labyrinth resulting in confusion and seemingly contradictory observations and conclusions about this type of deformity. The purpose of this study is to histopathologically classify and describe temporal bones whose cochleas have less than 2.5 turns. Of the 1800 temporal bones in our collection, 21 from 12 cases were found to have cochleas with less than 2.5 cochlear turns. Ages ranged from stillborn to 50 years. Temporal bones were harvested at autopsy, processed and embedded in celloidin. Sections were cut at a thickness of 20 microm and every 10th section stained with hematoxylin-eosin and examined using light microscopy. The number of turns, length of cochlea, integrity of cochlear base, length of modiolus, abnormalities of the semicircular canals and vestibule, enlargement of the vestibular aqueduct and middle ears were documented. Twenty-one temporal bones from age-matched patients without cochlear deformities were used as controls for modiolar length measurements. Malformation of the shortened cochlea was histopathologically classified into three groups as follows: (1) Common cavity, cochlear dysplasia (one ear)--severe dysplasia of the cochlea without a complete basal turn; (2) Mondini dysplasia (11 ears)--1.5 cochlear turns, a complete basal turn, an incomplete or absent interscalar septum and a complete bone at the base of the modiolus; and (3) Mondini-like dysplasia type A (five ears)--2 turns to the cochlea including a complete basal turn and complete bone at the base of the modiolus; and type B (four ears)--1.5-2 turns to the cochlea, hypoplasia of or a missing bone at the base of the modiolus (either with or without a communication between the internal auditory canal and the cochlea) and a complete basal turn. The range of congenital malformations in short cochlea is highly variable. Fundamental to the accurate evaluation of a labyrinthine anomaly

Congenital and perinatal cytomegalovirus infection

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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.

Multifocal Congenital Hemangiopericytoma.

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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.

Wise regulates bone deposition through genetic interactions with Lrp5.

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Ellies, Debra L; Economou, Androulla; Viviano, Beth; Rey, Jean-Philippe; Paine-Saunders, Stephenie; Krumlauf, Robb; Saunders, Scott

2014-01-01

In this study using genetic approaches in mouse we demonstrate that the secreted protein Wise plays essential roles in regulating early bone formation through its ability to modulate Wnt signaling via interactions with the Lrp5 co-receptor. In Wise-/- mutant mice we find an increase in the rate of osteoblast proliferation and a transient increase in bone mineral density. This change in proliferation is dependent upon Lrp5, as Wise;Lrp5 double mutants have normal bone mass. This suggests that Wise serves as a negative modulator of Wnt signaling in active osteoblasts. Wise and the closely related protein Sclerostin (Sost) are expressed in osteoblast cells during temporally distinct early and late phases in a manner consistent with the temporal onset of their respective increased bone density phenotypes. These data suggest that Wise and Sost may have common roles in regulating bone development through their ability to control the balance of Wnt signaling. We find that Wise is also required to potentiate proliferation in chondrocytes, serving as a potential positive modulator of Wnt activity. Our analyses demonstrate that Wise plays a key role in processes that control the number of osteoblasts and chondrocytes during bone homeostasis and provide important insight into mechanisms regulating the Wnt pathway during skeletal development.

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

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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.)

Congenital Ewing's Sarcoma/Peripheral Primitive Neuroectodermal Tumor: A Case Report and Review of the Literature.

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Jin, Shu-Guang; Jiang, Xiao-Ping; Zhong, Lin

2016-10-01

Ewing's sarcoma (EWS) and peripheral primitive neuroectodermal tumor (pPNET) are small round cell malignancies that develop in soft tissue and bone. They very rarely affect newborns. A diagnosis of EWS/pPNET depends mainly on immunohistochemistry and molecular/genetic assays. Since these tumors are highly aggressive, patient prognosis is typically very poor, and treatment remains a challenge. Here, we report a 13-day-old newborn diagnosed with congenital EWS/pPNET and describe its treatment. Copyright © 2014. Published by Elsevier B.V.

Educational series in congenital heart disease:Congenital left-sided heart obstruction

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Carr, Michelle; Curtis, Stephanie; Marek, Jan

2018-01-01

Congenital obstruction of the left ventricular outflow tract remains a significant problem and multilevel obstruction can often coexist. Obstruction can take several morphological forms and may involve the subvalvar, valvar or supravalvar portion of the aortic valve complex. Congenital valvar stenosis presenting in the neonatal period represents a spectrum of disorders ranging from the hypoplastic left heart syndrome to almost normal hearts. Treatment options vary dependent on the severity of...

Impaired short-term memory for pitch in congenital amusia.

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

2016-06-01

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

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

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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.

Genetics Home Reference: congenital mirror movement disorder

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... 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 ...

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

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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.

Radiology of congenital heart disease

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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

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

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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.

Congenital obstructive posterior urethral membranes and recurrent urinary tract infection: a rare case of congenital hypertrophy of the verumontanum

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Diana Bancin

2015-03-01

Full Text Available Congenital obstructive posterior urethral membranes (COPUM is a complex disease closely related to several pathological changes in kidney development and function, as a result of urinary reflux since in utero. This congenital anomaly of urinary tract potentially causes hydroureteronephrosis that is often associated with recurrent urinary tract infections and, ultimately, one of the most common causes of end-stage renal disease in children.1,2 Congenital hypertrophy of the verumontanum as part of COPUM is very rare. Only a few reports have been written on congenital hypertrophy of the vermontanum causing congenital obstructive uropathy.3-6

Congenital neutropenia in the era of genomics: classification, diagnosis, and natural history.

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Donadieu, Jean; Beaupain, Blandine; Fenneteau, Odile; Bellanné-Chantelot, Christine

2017-11-01

This review focuses on the classification, diagnosis and natural history of congenital neutropenia (CN). CN encompasses a number of genetic disorders with chronic neutropenia and, for some, affecting other organ systems, such as the pancreas, central nervous system, heart, bone and skin. To date, 24 distinct genes have been associated with CN. The number of genes involved makes gene screening difficult. This can be solved by next-generation sequencing (NGS) of targeted gene panels. One of the major complications of CN is spontaneous leukaemia, which is preceded by clonal somatic evolution, and can be screened by a targeted NGS panel focused on somatic events. © 2017 John Wiley & Sons Ltd.

Congenital cataract screening

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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.