Correction of prominent ears with a new splinting technique

Correction of prominent ears represents one of the main applications in plastic surgery. Apart from its cosmetic nature, this operation is important because of the psychological distress that the deformity causes patients. In this study, 40 patients who underwent treatment for prominent ears in our department were evaluated. The classical surgical technique described by Chongchet followed by a new splinting procedure was performed. The advantages of this technique are described in comparison with traditional methods

A retrospective study of patient outcomes and satisfaction following pinnaplasty

Introduction: Up to 5% of all children have prominent ears. Psychological distress and bullying adversely affect these children and can cause significant social exclusion. In times of austerity, cosmetic procedures such as surgical correction of prominent ears are felt to be an unnecessary cost to the health service. Materials and methods: A retrospective case note review of all patients undergoing pinnaplasty was undertaken. Postoperative outcomes were compared against the Royal College of Surgeons of England standards. The Glasgow Benefit Inventory, a validated post-intervention questionnaire, was then posted out to all patients. Results: A total of 72 patients were identified. Average age at procedure was 13 years. Eleven patients were above the age of 19 years. Twenty-eight patients were male and forty-four female. Sixty-two cases underwent bilateral pinnaplasty. No patients developed hematoma, and there were no readmissions within 30 days of surgery. Twenty-nine patients responded to the questionnaire (40%), of whom 27 reported a positive impact on their psychosocial well-being with a mean score of 36. Conclusion: Pinnaplasty offers patients an opportunity to alleviate the psychological distress of bullying and harassment secondary to the appearance of prominent ears

A novel KIF11 mutation in a Turkish patient with microcephaly, lymphedema, and chorioretinal dysplasia from a consanguineous family.

Microcephaly–lymphedema–chorioretinal dysplasia (MLCRD) syndrome is a rare syndrome that was first described in 1992. Characteristic craniofacial features include severe microcephaly, upslanting palpebral fissures, prominent ears, a broad nose, and a long philtrum with a pointed chin. Recently, mutations in KIF11 have been demonstrated to cause dominantly inherited MLCRD syndrome. Herein, we present a patient with MLCRD syndrome whose parents were first cousins. The parents are unaffected, and thus a recessive mode of inheritance for the disorder was considered likely. However, the propositus carries a novel, de novo nonsense mutationinexon2 of KIF11. The patient also had midline cleft tongue which has not previously been described in this syndrome

De novo variants disturbing the transactivation capacity of POU3F3 cause a characteristic neurodevelopmental disorder

POU3F3, also referred to as Brain-1, is a well-known transcription factor involved in the development of the central nervous system, but it has not previously been associated with a neurodevelopmental disorder. Here, we report the identification of 19 individuals with heterozygous POU3F3 disruptions, most of which are de novo variants. All individuals had developmental delays and/or intellectual disability and impairments in speech and language skills. Thirteen individuals had characteristic low-set, prominent, and/or cupped ears. Brain abnormalities were observed in seven of eleven MRI reports. POU3F3 is an intronless gene, insensitive to nonsense-mediated decay, and 13 individuals carried protein-truncating variants. All truncating variants that we tested in cellular models led to aberrant subcellular localization of the encoded protein. Luciferase assays demonstrated negative effects of these alleles on transcriptional activation of a reporter with a FOXP2-derived binding motif. In addition to the loss-of-function variants, five individuals had missense variants that clustered at specific positions within the functional domains, and one small in-frame deletion was identified. Two missense variants showed reduced transactivation capacity in our assays, whereas one variant displayed gain-of-function effects, suggesting a distinct pathophysiological mechanism. In bioluminescence resonance energy transfer (BRET) interaction assays, all the truncated POU3F3 versions that we tested had significantly impaired dimerization capacities, whereas all missense variants showed unaffected dimerization with wild-type POU3F3. Taken together, our identification and functional cell-based analyses of pathogenic variants in POU3F3, coupled with a clinical characterization, implicate disruptions of this gene in a characteristic neurodevelopmental disorder

Modeling SNR G1.9+0.3 as a Supernova Inside a Planetary Nebula

Using 3D numerical hydrodynamical simulations we show that a type Ia supernova (SN Ia) explosion inside a planetary nebula (PN) can explain the observed shape of the G1.9+0.3 supernova remnant (SNR) and its X-ray morphology. The SNR G1.9+0.3 morphology can be generally described as a sphere with two small and incomplete lobes protruding on opposite sides of the SNR, termed "ears", a structure resembling many elliptical PNe. Observations show the synchrotron X-ray emission to be much stronger inside the two ears than in the rest of the SNR. We numerically show that a spherical SN Ia explosion into a circumstellar matter (CSM) with the structure of an elliptical PN with ears and clumps embedded in the ears can explain the X-ray properties of SNR G1.9+0.3. While the ejecta has already collided with the PN shell in most of the SNR and its forward shock has been slowed down, the ejecta is still advancing inside the ears. The fast forward shock inside the ears explains the stronger X-ray emission there. SN Ia inside PNe (SNIPs) seem to comprise a non-negligible fraction of resolved SN Ia remnants.Comment: Revised version. 19 pages, 8 figures. Accepted to MNRA

Oral characteristics of Trisomy 8 and monosomy 18: a case report

Several reports described various mosaic chromosomal syndromes characterized by alterations originated by either an excess or deficit in the number of chromosomes. A case of mosaic trisomy 8 and monosomy 18 with significant involvement of the oral cavity is described, both in terms of general medicine and from a dental-oral perspective, and the treatment plan was planned and discussed. Regular follow-up visits enabled to verify significant improvement in all parameters of the patient’s oral health, which urged us to press on with our quest to protect the right to health of patients affected by disabilities

On Shape-Mediated Enrolment in Ear Biometrics

Ears are a new biometric with major advantage in that they appear to maintain their shape with increased age. Any automatic biometric system needs enrolment to extract the target area from the background. In ear biometrics the inputs are often human head profile images. Furthermore ear biometrics is concerned with the effects of partial occlusion mostly caused by hair and earrings. We propose an ear enrolment algorithm based on finding the elliptical shape of the ear using a Hough Transform (HT) accruing tolerance to noise and occlusion. Robustness is improved further by enforcing some prior knowledge. We assess our enrolment on two face profile datasets; as well as synthetic occlusion

XMM-Newton observations of the eastern jet of SS433

The radio supernova remnant W50 hosts at its center the peculiar galactic X-ray binary SS 433. It shows a central spherical structure with two `ears' which are supposed to be formed by the interaction of the precessing jets of SS 433 with the supernova shell. In two pointings in September/October 2004 for 30 ks each the eastern jet of SS 433 was observed with XMM-Newton to study the outermost parts of the `ear' and the X-ray bright emission region about 35 arcmin from SS 433. The spectra consist of two components: a non-thermal power law with photon index \Gamma ~ 2.17+/-0.02 and a thermal component at a typical temperature of kT ~ 0.3 keV. The X-ray emission seems to fill the whole interior region of the radio remnant W50. The jet terminates in the eastern `ear' in a ring-like terminal shock which indicates a flow with a kind of hollow-cone morphology. The spatial coincidence of X-ray and radio emission suggests physical conditions similar to those found at the outer shocks of ordinary supernova remnants. The bright emission region closer to SS 433 radiates non-thermally in a spatially well confined geometry at higher X-ray energies. At soft X-rays the shape of the region gets blurred, centered on the hard lenticular emission. The shape of this region and the bend in the jet propagation direction might be caused by the interaction of a re-collimated jet with the outer, non homogeneous interstellar matter distribution. The physical conditions leading to the re-collimation of the jet and the peculiar emission morphology are far from being understood and require deeper observations as well as a detailed modeling of the interaction of a jet with its surroundings.Comment: 10 pages, 8 figures, to appear in A&

Human Auditory cortical processing of changes in interaural correlation

Sensitivity to the similarity of the acoustic waveforms at the two ears, and specifically to changes in similarity, is crucial to auditory scene analysis and extraction of objects from background. Here, we use the high temporal resolution of magnetoencephalography to investigate the dynamics of cortical processing of changes in interaural correlation, a measure of interaural similarity, and compare them with behavior. Stimuli are interaurally correlated or uncorrelated wideband noise, immediately followed by the same noise with intermediate degrees of interaural correlation. Behaviorally, listeners' sensitivity to changes in interaural correlation is asymmetrical. Listeners are faster and better at detecting transitions from correlated noise than transitions from uncorrelated noise. The cortical response to the change in correlation is characterized by an activation sequence starting from ∼50 ms after change. The strength of this response parallels behavioral performance: auditory cortical mechanisms are much less sensitive to transitions from uncorrelated noise than from correlated noise. In each case, sensitivity increases with interaural correlation difference. Brain responses to transitions from uncorrelated noise lag those from correlated noise by ∼80 ms, which may be the neural correlate of the observed behavioral response time differences. Importantly, we demonstrate differences in location and time course of neural processing: transitions from correlated noise are processed by a distinct neural population, and with greater speed, than transitions from uncorrelated noise