Clinical implications of magnetic resonance imaging in temporomandibular disorders patients presenting ear fullness.

PubMed

Lee, Sang Yeon; Park, Ji Woon; Park, Seo Eun; Nam, Dong Woo; Lim, Hyun Jung; Kim, Young Ho

2017-12-14

The aim of this study was to investigate whether findings detected by temporomandibular joint magnetic resonance imaging (TMJ-MRI) can provide pathognomonic evidence of temporomandibular disorders (TMD) in patients with nonspecific ear fullness (EF). The association of nonspecific EF with clinical characteristics of TMD based on TMJ-MRI findings was examined. Retrospective analysis. Thirty-four subjects (42 ears) who had no detectable otologic problems as a cause of EF were enrolled in this study. Each subject underwent TMJ-MRI to identify pathology of the TMJ as a possible cause of nonspecific EF. All subjects participated in the re-evaluation process following TMD treatment. Anatomical abnormalities in TMJ-MRI, irrespective of TMD signs, were observed in 34 of the 42 ears (80.9%), such as degenerative change of the TMJ (16 ears), articular disc displacement (11 ears), and joint effusion (seven ears). Specific abnormalities of the TMJ were associated with nonspecific EF, and this symptom showed improvement following individualized TMD treatment in those with internal derangement and/or effusion of the TMJ. However, abnormal TMJ-MRI findings were also observed in seven of nine ears with no TMD signs, and there was no significant association between the presence of TMD signs and abnormal TMJ-MRI findings (χ 2 = 0.075, P = .784). Patients presenting with nonspecific EF may have TMD, which can be effectively diagnosed using TMJ-MRI. The present study revealed the causal relationship between nonspecific EF and abnormal TMJ findings based on MRI. Individualized TMD treatments based on TMJ-MRI led to improved treatment outcomes with special regard to nonspecific EF LEVEL OF EVIDENCE: 4 Laryngoscope, 2017. © 2017 The American Laryngological, Rhinological and Otological Society, Inc.

Congenital malformations of the inner ear and the vestibulocochlear nerve in children with sensorineural hearing loss: evaluation with CT and MRI.

PubMed

Westerhof, J P; Rademaker, J; Weber, B P; Becker, H

2001-01-01

The purpose of this work was to study the diagnostic value of CT and MRI in children with sensorineural hearing loss and to analyze anatomic abnormalities of the inner ear and the vestibulocochlear nerve in this patient group. We evaluated 42 inner ears in 21 children with congenital deafness who had congenital inner ear malformations and who were candidates for cochlear implants. All patients were studied with high resolution MR and helical CT examinations. The MR study included a T2-weighted 3D fast SE sequence. We describe and tabulate the anatomic abnormalities. Special attention was given to abnormalities of the vestibulocochlear nerve. The field of view in the plane according to the length axis of the internal auditory canal (IAC) was 4 cm. Additional continuous parasagittal reformations perpendicular to the length axis of the IAC were studied with a field of view of 3 cm. CT and MRI allowed accurate identification of malformations of the inner ear in children with congenital deafness. We identified 99 malformations, with a majority of patients demonstrating multiple abnormalities. Common imaging findings were Mondini abnormality and Mondini variants (12/42) and fusion of the lateral or superior semicircular canal with the vestibule (12/42). MRI demonstrated in 9 of 21 patients a rudimentary or absent vestibulocochlear nerve in the auditory canal. CT and MRI are important modalities to analyze the inner ear in children who are candidates for cochlear implants. MRI with an extremely small field of view should be used to study possible abnormalities of the vestibulocochlear nerves. This may alter clinical care and allow cochlear implant placement in patients whose electrodiagnostic studies suggest that the implant should not be performed. The detailed analysis of abnormalities of the inner ear might establish prognostic factors.

Vitamin D receptor deficiency impairs inner ear development in zebrafish

DOE Office of Scientific and Technical Information (OSTI.GOV)

Kwon, Hye-Joo; Biology Department, Princess Nourah University, Riyadh 11671

The biological actions of vitamin D are largely mediated through binding to the vitamin D receptor (VDR), a member of the nuclear hormone receptor family, which regulates gene expression in a wide variety of tissues and cells. Mutations in VDR gene have been implicated in ear disorders (hearing loss and balance disorder) but the mechanisms are not well established. In this study, to investigate the role of VDR in inner ear development, morpholino-mediated gene knockdown approaches were used in zebrafish model system. Two paralogs for VDR, vdra and vdrb, have been identified in zebrafish. Knockdown of vdra had no effectmore » on ear development, whereas knockdown of vdrb displayed morphological ear defects including smaller otic vesicles with malformed semicircular canals and abnormal otoliths. Loss-of-vdrb resulted in down-regulation of pre-otic markers, pax8 and pax2a, indicating impairment of otic induction. Furthermore, zebrafish embryos lacking vdrb produced fewer sensory hair cells in the ears and showed disruption of balance and motor coordination. These data reveal that VDR signaling plays an important role in ear development. - Highlights: • VDR signaling is involved in ear development. • Knockdown of vdrb causes inner ear malformations during embryogenesis. • Knockdown of vdrb affects otic placode induction. • Knockdown of vdrb reduces the number of sensory hair cells in the inner ear. • Knockdown of vdrb disrupts balance and motor coordination.« less

Middle ear abnormalities in Van Maldergem syndrome.

PubMed

Verheij, Emmy; Thomeer, Henricus G X M; Pameijer, Frank A; Topsakal, Vedat

2017-01-01

Van Maldergem syndrome (VMS) is a very rare syndrome that was first described in 1992. The main features of this syndrome comprise intellectual disability, blepharo-naso-facial malformation, and hand anomalies. Almost all nine described patients have been shown to be affected by conductive hearing impairment attributed to microtia, and atresia of the outer ear canal. Here, we present a VMS patient with congenital malformations of the middle ear as the main reason for severe conductive bilateral hearing impairment. To our knowledge, this is the first report to describe middle ear abnormalities in VMS. These malformations were seen on high resolution Computed Tomography scanning and during an exploratory tympanotomy. Due to the severity of the middle ear abnormalities and the risk for facial nerve damage, the patient was not offered an ossicular chain reconstruction but a bone conduction device after this exploratory tympanotomy. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

Spectacle fitting with ear, nose and face deformities or abnormalities.

PubMed

Eng, Helen; Chiu, Roger Sin Fai

2002-11-01

Spectacle frame selection and dispensing remain significant components of optometry. Occasionally, we encounter patients who are unable to wear conventional spectacles due to abnormalities or deformities following injury and/or surgery to their nose, ears or head. In these cases, spectacle frame fitting may be more complex and customized frame adjustments may be required to account for the anatomical variations. A patient with a microtia (hypoplastic pinna) was fitted with a tailored spectacle frame. The details are presented together with a summary of different frames and modifications available for ears, nose and face abnormalities.

Sonographic Measurement of Fetal Ear Length in Turkish Women with a Normal Pregnancy

PubMed Central

Özdemir, Mucize Eriç; Uzun, Işıl; Karahasanoğlu, Ayşe; Aygün, Mehmet; Akın, Hale; Yazıcıoğlu, Fehmi

2014-01-01

Background: Abnormal fetal ear length is a feature of chromosomal disorders. Fetal ear length measurement is a simple measurement that can be obtained during ultrasonographic examinations. Aims: To develop a nomogram for fetal ear length measurements in our population and investigate the correlation between fetal ear length, gestational age, and other standard fetal biometric measurements. Study Design: Cohort study. Methods: Ear lengths of the fetuses were measured in normal singleton pregnancies. The relationship between gestational age and fetal ear length in millimetres was analysed by simple linear regression. In addition, the correlation of fetal ear length measurements with biparietal diameter, head circumference, abdominal circumference, and femur length were evaluated.Ear length measurements were obtained from fetuses in 389 normal singleton pregnancies ranging between 16 and 28 weeks of gestation. Results: A nomogram was developed by linear regression analysis of the parameters ear length and gestational age. Fetal ear length (mm) = y = (1.348 X gestational age)−12.265), where gestational ages is in weeks. A high correlation was found between fetal ear length and gestational age, and a significant correlation was also found between fetal ear length and the biparietal diameter (r=0.962; p<0.001). Similar correlations were found between fetal ear length and head circumference, and fetal ear length and femur length. Conclusion: The results of this study provide a nomogram for fetal ear length. The study also demonstrates the relationship between ear length and other biometric measurements. PMID:25667783

Ear Tubes

MedlinePlus

... of the ear drum or eustachian tube, Down Syndrome, cleft palate, and barotrauma (injury to the middle ear caused by a reduction of air pressure, ... specialist) may be warranted if you or your child has experienced repeated ... fluid in the middle ear, barotrauma, or have an anatomic abnormality that ...

Ephrin-B2 governs morphogenesis of endolymphatic sac and duct epithelia in the mouse inner ear.

PubMed

Raft, Steven; Andrade, Leonardo R; Shao, Dongmei; Akiyama, Haruhiko; Henkemeyer, Mark; Wu, Doris K

2014-06-01

Control over ionic composition and volume of the inner ear luminal fluid endolymph is essential for normal hearing and balance. Mice deficient in either the EphB2 receptor tyrosine kinase or the cognate transmembrane ligand ephrin-B2 (Efnb2) exhibit background strain-specific vestibular-behavioral dysfunction and signs of abnormal endolymph homeostasis. Using various loss-of-function mouse models, we found that Efnb2 is required for growth and morphogenesis of the embryonic endolymphatic epithelium, a precursor of the endolymphatic sac (ES) and duct (ED), which mediate endolymph homeostasis. Conditional inactivation of Efnb2 in early-stage embryonic ear tissues disrupted cell proliferation, cell survival, and epithelial folding at the origin of the endolymphatic epithelium. This correlated with apparent absence of an ED, mis-localization of ES ion transport cells relative to inner ear sensory organs, dysplasia of the endolymph fluid space, and abnormally formed otoconia (extracellular calcite-protein composites) at later stages of embryonic development. A comparison of Efnb2 and Notch signaling-deficient mutant phenotypes indicated that these two signaling systems have distinct and non-overlapping roles in ES/ED development. Homozygous deletion of the Efnb2 C-terminus caused abnormalities similar to those found in the conditional Efnb2 null homozygote. Analyses of fetal Efnb2 C-terminus deletion heterozygotes found mis-localized ES ion transport cells only in the genetic background exhibiting vestibular dysfunction. We propose that developmental dysplasias described here are a gene dose-sensitive cause of the vestibular dysfunction observed in EphB-Efnb2 signaling-deficient mice. Published by Elsevier Inc.

Ephrin-B2 governs morphogenesis of endolymphatic sac and duct epithelia in the mouse inner ear

PubMed Central

Raft, Steven; Andrade, Leonardo R.; Shao, Dongmei; Akiyama, Haruhiko; Henkemeyer, Mark; Wu, Doris K.

2014-01-01

Control over ionic composition and volume of the inner ear luminal fluid endolymph is essential for normal hearing and balance. Mice deficient in either the EphB2 receptor tyrosine kinase or the cognate transmembrane ligand ephrin-B2 (Efnb2) exhibit background strain-specific vestibular behavioral dysfunction and signs of abnormal endolymph homeostasis. Using various loss-of-function mouse models, we found that Efnb2 is required for growth and morphogenesis of the embryonic endolymphatic epithelium, a precursor of the endolymphatic sac (ES) and duct (ED), which mediate endolymph homeostasis. Conditional inactivation of Efnb2 in early-stage embryonic ear tissues disrupted cell proliferation, cell survival, and epithelial folding at the origin of the endolymphatic epithelium. This correlated with apparent absence of an ED, mis-localization of ES ion transport cells relative to inner ear sensory organs, dysplasia of the endolymph fluid space, and abnormally formed otoconia (extracellular calcite protein composites) at later stages of embryonic development. A comparison of Efnb2 and Notch signaling deficient mutant phenotypes indicated that these two signaling systems have distinct and non overlapping roles in ES/ED development. Homozygous deletion of the Efnb2 C terminus caused abnormalities similar to those found in the conditional Efnb2 null homozygote. Analyses of fetal Efnb2 C-terminus deletion heterozygotes found mis-localized ES ion transport cells only in the genetic background exhibiting vestibular dysfunction. We propose that developmental dysplasias described here are a gene dose sensitive cause of the vestibular dysfunction observed in EphB-Efnb2 signaling-deficient mice. PMID:24583262

The Role of Zic Genes in Inner Ear Development in the Mouse: Exploring Mutant Mouse Phenotypes

PubMed Central

Chervenak, Andrew P.; Bank, Lisa M.; Thomsen, Nicole; Glanville-Jones, Hannah C; Skibo, Jonathan; Millen, Kathleen J.; Arkell, Ruth M.; Barald, Kate F.

2014-01-01

Background Murine Zic genes (Zic1-5) are expressed in the dorsal hindbrain and in periotic mesenchyme (POM) adjacent to the developing inner ear. Zic genes are involved in developmental signaling pathways in many organ systems, including the ear, although their exact roles haven't been fully elucidated. This report examines the role of Zic1, Zic2, and Zic4 during inner ear development in mouse mutants in which these Zic genes are affected Results Zic1/Zic4 double mutants don't exhibit any apparent defects in inner ear morphology. By contrast, inner ears from Zic2kd/kd and Zic2Ku/Ku mutants have severe but variable morphological defects in endolymphatic duct/sac and semicircular canal formation and in cochlear extension in the inner ear. Analysis of otocyst patterning in the Zic2Ku/Ku mutants by in situ hybridization showed changes in the expression patterns of Gbx2 and Pax2. Conclusions The experiments provide the first genetic evidence that the Zic genes are required for morphogenesis of the inner ear. Zic2 loss-of-function doesn't prevent initial otocyst patterning but leads to molecular abnormalities concomitant with morphogenesis of the endolymphatic duct. Functional hearing deficits often accompany inner ear dysmorphologies, making Zic2 a novel candidate gene for ongoing efforts to identify the genetic basis of human hearing loss. PMID:25178196

Brain stem and inner ear abnormalities in children with auditory neuropathy spectrum disorder and cochlear nerve deficiency.

PubMed

Huang, B Y; Roche, J P; Buchman, C A; Castillo, M

2010-11-01

Cranial abnormalities, including CND, are common in children with ANSD. The purpose of this study was to assess whether CND is associated with brain or inner ear abnormalities in a cohort of children with ANSD. Two neuroradiologists retrospectively reviewed cranial MR imaging examinations in 103 children with ANSD. Brain, cochlear nerve, and temporal bone abnormalities were described and tabulated. Findings were stratified on the basis of the presence and laterality of CND, and differences in the presence of associated inner ear or intracranial abnormalities were assessed by using 2-tailed Fisher exact tests. CND was identified in 33.0% of children and 26.9% of ears with ANSD. Significantly more patients with bilateral CND had intracranial abnormalities than those with unilateral CND (60.0% versus 15.8%; P = .012). Forty percent of patients with bilateral CND, 0% of patients with unilateral CND, and 10.1% of those without CND demonstrated hindbrain malformations. Patients with bilateral CND were more likely to demonstrate hindbrain malformations than patients with normal nerves (P = .01) or unilateral CND (P = .004). Labyrinthine abnormalities were significantly more common in patients with bilateral CND than in those without CND (P ≤ .001). Cochlear anomalies were more common in patients with bilateral versus unilateral CND (P = .01). IAC and cochlear aperture stenosis were more common in those with unilateral and bilateral CND than those without CND (both P < .001). Cochlear and hindbrain abnormalities are significantly more common among patients with ANSD with bilateral CND compared with those with at least 1 intact cochlear nerve.

Inner ear manifestations in CHARGE: Abnormalities, treatments, animal models, and progress toward treatments in auditory and vestibular structures.

PubMed

Choo, Daniel I; Tawfik, Kareem O; Martin, Donna M; Raphael, Yehoash

2017-12-01

The inner ear contains the sensory organs for hearing and balance. Both hearing and balance are commonly affected in individuals with CHARGE syndrome (CS), an autosomal dominant condition caused by heterozygous pathogenic variants in the CHD7 gene. Semicircular canal dysplasia or aplasia is the single most prevalent feature in individuals with CHARGE leading to deficient gross motor skills and ambulation. Identification of CHD7 as the major gene affected in CHARGE has enabled acceleration of research in this field. Great progress has been made in understanding the role of CHD7 in the development and function of the inner ear, as well as in related organs such as the middle ear and auditory and vestibular neural pathways. The goals of current research on CHD7 and CS are to (a) improve our understanding of the pathology caused by CHD7 pathogenic variants and (b) to provide better tools for prognosis and treatment. Current studies utilize cells and whole animals, from flies to mammals. The mouse is an excellent model for exploring mechanisms of Chd7 function in the ear, given the evolutionary conservation of ear structure, function, Chd7 expression, and similarity of mutant phenotypes between mice and humans. Newly recognized developmental functions for mouse Chd7 are shedding light on how abnormalities in CHD7 might lead to CS symptoms in humans. Here we review known human inner ear phenotypes associated with CHD7 pathogenic variants and CS, summarize progress toward diagnosis and treatment of inner ear-related pathologies, and explore new avenues for treatment based on basic science discoveries. © 2017 Wiley Periodicals, Inc.

Auricular hematoma and cauliflower deformation of the ear: from art to medicine.

PubMed

Mudry, Albert; Pirsig, Wolfgang

2009-01-01

Auricular hematoma and cauliflower deformation of the ear are unique in several respects. Knowledge about it began, in antiquity, through artists, particularly Greek and Roman, and then Japanese in the 18th century with their representation of cauliflower deformation of the ear on sculptures and paintings of pugilists and wrestlers. It is only in the 19th century that physicians began to make substantive progress in understanding this abnormality. It was first thought to be associated with mental disease, but by the beginning of the 20th century, its etiology was recognized as being caused by trauma and was then named auricular hematoma. The second step in the understanding of this affliction was the observation that auricular hematoma progresses toward cauliflower deformation of the ear, which was named cauliflower ear. Recognition of this evolution led to the development of therapies. During the second half of the 20th century, different treatments were developed. They included various hematoma drainage techniques with special bandages to prevent hematoma recurrence and ensuing progression to cauliflower ear. In summary, cauliflower deformation of the ear is an old artistic affliction that has only recently received medical attention.

Ick Ciliary Kinase Is Essential for Planar Cell Polarity Formation in Inner Ear Hair Cells and Hearing Function.

PubMed

Okamoto, Shio; Chaya, Taro; Omori, Yoshihiro; Kuwahara, Ryusuke; Kubo, Shun; Sakaguchi, Hirofumi; Furukawa, Takahisa

2017-02-22

Cellular asymmetries play crucial roles in development and organ function. The planar cell polarity (PCP) signaling pathway is involved in the establishment of cellular asymmetry within the plane of a cell sheet. Inner ear sensory hair cells (HCs), which have several rows of staircase-like stereocilia and one kinocilium located at the vertex of the stereocilia protruding from the apical surface of each HC, exhibit a typical form of PCP. Although connections between cilia and PCP signaling in vertebrate development have been reported, their precise nature is not well understood. During inner ear development, several ciliary proteins are known to play a role in PCP formation. In the current study, we investigated a functional role for intestinal cell kinase (Ick), which regulates intraflagellar transport (IFT) at the tip of cilia, in the mouse inner ear. A lack of Ick in the developing inner ear resulted in PCP defects in the cochlea, including misorientation or misshaping of stereocilia and aberrant localization of the kinocilium and basal body in the apical and middle turns, leading to auditory dysfunction. We also observed abnormal ciliary localization of Ift88 in both HCs and supporting cells. Together, our results show that Ick ciliary kinase is essential for PCP formation in inner ear HCs, suggesting that ciliary transport regulation is important for PCP signaling. SIGNIFICANCE STATEMENT The cochlea in the inner ear is the hearing organ. Planar cell polarity (PCP) in hair cells (HCs) in the cochlea is essential for mechanotransduction and refers to the asymmetric structure consisting of stereociliary bundles and the kinocilium on the apical surface of the cell body. We reported previously that a ciliary kinase, Ick, regulates intraflagellar transport (IFT). Here, we found that loss of Ick leads to abnormal localization of the IFT component in kinocilia, PCP defects in HCs, and hearing dysfunction. Our study defines the association of ciliary transport regulation with PCP formation in HCs and hearing function. Copyright © 2017 the authors 0270-6474/17/372073-13$15.00/0.

Ear-EEG detects ictal and interictal abnormalities in focal and generalized epilepsy - A comparison with scalp EEG monitoring.

PubMed

Zibrandtsen, I C; Kidmose, P; Christensen, C B; Kjaer, T W

2017-12-01

Ear-EEG is recording of electroencephalography from a small device in the ear. This is the first study to compare ictal and interictal abnormalities recorded with ear-EEG and simultaneous scalp-EEG in an epilepsy monitoring unit. We recorded and compared simultaneous ear-EEG and scalp-EEG from 15 patients with suspected temporal lobe epilepsy. EEGs were compared visually by independent neurophysiologists. Correlation and time-frequency analysis was used to quantify the similarity between ear and scalp electrodes. Spike-averages were used to assess similarity of interictal spikes. There were no differences in sensitivity or specificity for seizure detection. Mean correlation coefficient between ear-EEG and nearest scalp electrode was above 0.6 with a statistically significant decreasing trend with increasing distance away from the ear. Ictal morphology and frequency dynamics can be observed from visual inspection and time-frequency analysis. Spike averages derived from ear-EEG electrodes yield a recognizable spike appearance. Our results suggest that ear-EEG can reliably detect electroencephalographic patterns associated with focal temporal lobe seizures. Interictal spike morphology from sufficiently large temporal spike sources can be sampled using ear-EEG. Ear-EEG is likely to become an important tool in clinical epilepsy monitoring and diagnosis. Copyright © 2017 International Federation of Clinical Neurophysiology. Published by Elsevier B.V. All rights reserved.

Development of a novel clinical scoring system for on-farm diagnosis of bovine respiratory disease in pre-weaned dairy calves.

PubMed

Love, William J; Lehenbauer, Terry W; Kass, Philip H; Van Eenennaam, Alison L; Aly, Sharif S

2014-01-01

Several clinical scoring systems for diagnosis of bovine respiratory disease (BRD) in calves have been proposed. However, such systems were based on subjective judgment, rather than statistical methods, to weight scores. Data from a pair-matched case-control study on a California calf raising facility was used to develop three novel scoring systems to diagnose BRD in preweaned dairy calves. Disease status was assigned using both clinical signs and diagnostic test results for BRD-associated pathogens. Regression coefficients were used to weight score values. The systems presented use nasal and ocular discharge, rectal temperature, ear and head carriage, coughing, and respiratory quality as predictors. The systems developed in this research utilize fewer severity categories of clinical signs, require less calf handling, and had excellent agreement (Kappa > 0.8) when compared to an earlier scoring system. The first scoring system dichotomized all clinical predictors but required inducing a cough. The second scoring system removed induced cough as a clinical abnormality but required distinguishing between three levels of nasal discharge severity. The third system removed induced cough and forced a dichotomized variable for nasal discharge. The first system presented in this study used the following predictors and assigned values: coughing (induced or spontaneous coughing, 2 points), nasal discharge (any discharge, 3 points), ocular discharge (any discharge, 2 points), ear and head carriage (ear droop or head tilt, 5 points), fever (≥39.2°C or 102.5°F, 2 points), and respiratory quality (abnormal respiration, 2 points). Calves were categorized "BRD positive" if their total score was ≥4. This system correctly classified 95.4% cases and 88.6% controls. The second presented system categorized the predictors and assigned weights as follows: coughing (spontaneous only, 2 points), mild nasal discharge (unilateral, serous, or watery discharge, 3 points), moderate to severe nasal discharge (bilateral, cloudy, mucoid, mucopurlent, or copious discharge, 5 points), ocular discharge (any discharge, 1 point), ear and head carriage (ear droop or head tilt, 5 points), fever (≥39.2°C, 2 points), and respiratory quality (abnormal respiration, 2 points). Calves were categorized "BRD positive" if their total score was ≥4. This system correctly classified 89.3% cases and 92.8% controls. The third presented system used the following predictors and scores: coughing (spontaneous only, 2 points), nasal discharge (any, 4 points), ocular discharge (any, 2 points), ear and head carriage (ear droop or head tilt, 5 points), fever (≥39.2°C, 2 points), and respiratory quality (abnormal respiration, 2 points). Calves were categorized "BRD positive" if their total score was ≥5. This system correctly classified 89.4% cases and 90.8% controls. Each of the proposed systems offer few levels of clinical signs and data-based weights for on-farm diagnosis of BRD in dairy calves.

Development of a novel clinical scoring system for on-farm diagnosis of bovine respiratory disease in pre-weaned dairy calves

PubMed Central

Love, William J.; Lehenbauer, Terry W.; Kass, Philip H.; Van Eenennaam, Alison L.

2014-01-01

Several clinical scoring systems for diagnosis of bovine respiratory disease (BRD) in calves have been proposed. However, such systems were based on subjective judgment, rather than statistical methods, to weight scores. Data from a pair-matched case-control study on a California calf raising facility was used to develop three novel scoring systems to diagnose BRD in preweaned dairy calves. Disease status was assigned using both clinical signs and diagnostic test results for BRD-associated pathogens. Regression coefficients were used to weight score values. The systems presented use nasal and ocular discharge, rectal temperature, ear and head carriage, coughing, and respiratory quality as predictors. The systems developed in this research utilize fewer severity categories of clinical signs, require less calf handling, and had excellent agreement (Kappa > 0.8) when compared to an earlier scoring system. The first scoring system dichotomized all clinical predictors but required inducing a cough. The second scoring system removed induced cough as a clinical abnormality but required distinguishing between three levels of nasal discharge severity. The third system removed induced cough and forced a dichotomized variable for nasal discharge. The first system presented in this study used the following predictors and assigned values: coughing (induced or spontaneous coughing, 2 points), nasal discharge (any discharge, 3 points), ocular discharge (any discharge, 2 points), ear and head carriage (ear droop or head tilt, 5 points), fever (≥39.2°C or 102.5°F, 2 points), and respiratory quality (abnormal respiration, 2 points). Calves were categorized “BRD positive” if their total score was ≥4. This system correctly classified 95.4% cases and 88.6% controls. The second presented system categorized the predictors and assigned weights as follows: coughing (spontaneous only, 2 points), mild nasal discharge (unilateral, serous, or watery discharge, 3 points), moderate to severe nasal discharge (bilateral, cloudy, mucoid, mucopurlent, or copious discharge, 5 points), ocular discharge (any discharge, 1 point), ear and head carriage (ear droop or head tilt, 5 points), fever (≥39.2°C, 2 points), and respiratory quality (abnormal respiration, 2 points). Calves were categorized “BRD positive” if their total score was ≥4. This system correctly classified 89.3% cases and 92.8% controls. The third presented system used the following predictors and scores: coughing (spontaneous only, 2 points), nasal discharge (any, 4 points), ocular discharge (any, 2 points), ear and head carriage (ear droop or head tilt, 5 points), fever (≥39.2°C, 2 points), and respiratory quality (abnormal respiration, 2 points). Calves were categorized “BRD positive” if their total score was ≥5. This system correctly classified 89.4% cases and 90.8% controls. Each of the proposed systems offer few levels of clinical signs and data-based weights for on-farm diagnosis of BRD in dairy calves. PMID:24482759

Cochlear Implant Outcomes and Genetic Mutations in Children with Ear and Brain Anomalies

PubMed Central

Busi, Micol; Rosignoli, Monica; Minazzi, Federica; Trevisi, Patrizia; Aimoni, Claudia; Calzolari, Ferdinando; Martini, Alessandro

2015-01-01

Background. Specific clinical conditions could compromise cochlear implantation outcomes and drastically reduce the chance of an acceptable development of perceptual and linguistic capabilities. These conditions should certainly include the presence of inner ear malformations or brain abnormalities. The aims of this work were to study the diagnostic value of high resolution computed tomography (HRCT) and magnetic resonance imaging (MRI) in children with sensorineural hearing loss who were candidates for cochlear implants and to analyse the anatomic abnormalities of the ear and brain in patients who underwent cochlear implantation. We also analysed the effects of ear malformations and brain anomalies on the CI outcomes, speculating on their potential role in the management of language developmental disorders. Methods. The present study is a retrospective observational review of cochlear implant outcomes among hearing-impaired children who presented ear and/or brain anomalies at neuroimaging investigations with MRI and HRCT. Furthermore, genetic results from molecular genetic investigations (GJB2/GJB6 and, additionally, in selected cases, SLC26A4 or mitochondrial-DNA mutations) on this study group were herein described. Longitudinal and cross-sectional analysis was conducted using statistical tests. Results. Between January 1, 1996 and April 1, 2012, at the ENT-Audiology Department of the University Hospital of Ferrara, 620 cochlear implantations were performed. There were 426 implanted children at the time of the present study (who were <18 years). Among these, 143 patients (64 females and 79 males) presented ear and/or brain anomalies/lesions/malformations at neuroimaging investigations with MRI and HRCT. The age of the main study group (143 implanted children) ranged from 9 months and 16 years (average = 4.4; median = 3.0). Conclusions. Good outcomes with cochlear implants are possible in patients who present with inner ear or brain abnormalities, even if central nervous system anomalies represent a negative prognostic factor that is made worse by the concomitant presence of cochlear malformations. Common cavity and stenosis of the internal auditory canal (less than 2 mm) are negative prognostic factors even if brain lesions are absent. PMID:26236732

Cochlear Implant Outcomes and Genetic Mutations in Children with Ear and Brain Anomalies.

PubMed

Busi, Micol; Rosignoli, Monica; Castiglione, Alessandro; Minazzi, Federica; Trevisi, Patrizia; Aimoni, Claudia; Calzolari, Ferdinando; Granieri, Enrico; Martini, Alessandro

2015-01-01

Specific clinical conditions could compromise cochlear implantation outcomes and drastically reduce the chance of an acceptable development of perceptual and linguistic capabilities. These conditions should certainly include the presence of inner ear malformations or brain abnormalities. The aims of this work were to study the diagnostic value of high resolution computed tomography (HRCT) and magnetic resonance imaging (MRI) in children with sensorineural hearing loss who were candidates for cochlear implants and to analyse the anatomic abnormalities of the ear and brain in patients who underwent cochlear implantation. We also analysed the effects of ear malformations and brain anomalies on the CI outcomes, speculating on their potential role in the management of language developmental disorders. The present study is a retrospective observational review of cochlear implant outcomes among hearing-impaired children who presented ear and/or brain anomalies at neuroimaging investigations with MRI and HRCT. Furthermore, genetic results from molecular genetic investigations (GJB2/GJB6 and, additionally, in selected cases, SLC26A4 or mitochondrial-DNA mutations) on this study group were herein described. Longitudinal and cross-sectional analysis was conducted using statistical tests. Between January 1, 1996 and April 1, 2012, at the ENT-Audiology Department of the University Hospital of Ferrara, 620 cochlear implantations were performed. There were 426 implanted children at the time of the present study (who were <18 years). Among these, 143 patients (64 females and 79 males) presented ear and/or brain anomalies/lesions/malformations at neuroimaging investigations with MRI and HRCT. The age of the main study group (143 implanted children) ranged from 9 months and 16 years (average = 4.4; median = 3.0). Good outcomes with cochlear implants are possible in patients who present with inner ear or brain abnormalities, even if central nervous system anomalies represent a negative prognostic factor that is made worse by the concomitant presence of cochlear malformations. Common cavity and stenosis of the internal auditory canal (less than 2 mm) are negative prognostic factors even if brain lesions are absent.

Maternal characteristics and birth outcomes of pregnant women who had offspring with congenital ear abnormalities - a population-based case-control study.

PubMed

Paput, László; Bánhidy, Ferenc; Czeizel, Andrew E

2011-09-01

To describe the maternal characteristics and birth outcomes of newborn infants affected with isolated ear congenital abnormalities (IECA), mainly isolated anotia/microtia and unclassified multiple congenital abnormalities (CAs) including anotia/microtia (UMAM). Cases with IECA and UMAM were compared with their matched controls and all controls without any defect and malformed controls affected with other defects in the population-based large dataset of the Hungarian Case-Control Surveillance of Congenital Abnormalities. The mothers of 354 cases with IECA did not show significant difference in age, but their mean birth order was higher while their socio-economic status based on the maternal employment status was lower compared to the figures of their matched controls. There was a male excess among cases with microtia and mainly with UMAM. The evaluation of birth outcomes of newborns affected with IECA indicated intrauterine fetal growth retardation. Newborn infants with isolated microtia had intrauterine growth retardation and the association of this developmental defect localized for a small region of head with the general fetal development raises interesting theoretical question.

Eustachian tube function and middle ear barotrauma associated with extremes in atmospheric pressure.

PubMed

Miyazawa, T; Ueda, H; Yanagita, N

1996-11-01

Eustachian tube (ET) function was studied by means of sonotubometry and tubotympano-aerodynamography (TTAG) prior to and following exposure to hypobaric or hyperbaric conditions. Forty normal adults were subjected to hypobaric pressure. Fifty adults who underwent hyperbaric oxygen (HBO) therapy also were studied. Following hypobaric exposure, 14 of 80 ears (17.5%) exhibited middle ear barotrauma. Following hyperbaric exposure, 34 of 100 ears (34%) exhibited middle ear barotrauma. Dysfunction of the ET, characterized by altered active and passive opening capacity, was more prevalent following exposure to extremes in atmospheric pressure compared to baseline. The ET function, which was impaired after the first HBO treatment, improved gradually over the next 2 hours. Overall, however, ET function was worse after the seventh treatment. The patients who developed barotrauma exhibited worse ET function prior to hypobaric or hyperbaric exposure. Thus, abnormal ET function can be used to predict middle ear barotrauma prior to exposure to hypobaric or hyperbaric atmospheric pressure.

Hearing Loss in a Mouse Model of 22q11.2 Deletion Syndrome

PubMed Central

Fuchs, Jennifer C.; Zinnamon, Fhatarah A.; Taylor, Ruth R.; Ivins, Sarah; Scambler, Peter J.; Forge, Andrew; Tucker, Abigail S.; Linden, Jennifer F.

2013-01-01

22q11.2 Deletion Syndrome (22q11DS) arises from an interstitial chromosomal microdeletion encompassing at least 30 genes. This disorder is one of the most significant known cytogenetic risk factors for schizophrenia, and can also cause heart abnormalities, cognitive deficits, hearing difficulties, and a variety of other medical problems. The Df1/+ hemizygous knockout mouse, a model for human 22q11DS, recapitulates many of the deficits observed in the human syndrome including heart defects, impaired memory, and abnormal auditory sensorimotor gating. Here we show that Df1/+ mice, like human 22q11DS patients, have substantial rates of hearing loss arising from chronic middle ear infection. Auditory brainstem response (ABR) measurements revealed significant elevation of click-response thresholds in 48% of Df1/+ mice, often in only one ear. Anatomical and histological analysis of the middle ear demonstrated no gross structural abnormalities, but frequent signs of otitis media (OM, chronic inflammation of the middle ear), including excessive effusion and thickened mucosa. In mice for which both in vivo ABR thresholds and post mortem middle-ear histology were obtained, the severity of signs of OM correlated directly with the level of hearing impairment. These results suggest that abnormal auditory sensorimotor gating previously reported in mouse models of 22q11DS could arise from abnormalities in auditory processing. Furthermore, the findings indicate that Df1/+ mice are an excellent model for increased risk of OM in human 22q11DS patients. Given the frequently monaural nature of OM in Df1/+ mice, these animals could also be a powerful tool for investigating the interplay between genetic and environmental causes of OM. PMID:24244619

Hearing loss in a mouse model of 22q11.2 Deletion Syndrome.

PubMed

Fuchs, Jennifer C; Zinnamon, Fhatarah A; Taylor, Ruth R; Ivins, Sarah; Scambler, Peter J; Forge, Andrew; Tucker, Abigail S; Linden, Jennifer F

2013-01-01

22q11.2 Deletion Syndrome (22q11DS) arises from an interstitial chromosomal microdeletion encompassing at least 30 genes. This disorder is one of the most significant known cytogenetic risk factors for schizophrenia, and can also cause heart abnormalities, cognitive deficits, hearing difficulties, and a variety of other medical problems. The Df1/+ hemizygous knockout mouse, a model for human 22q11DS, recapitulates many of the deficits observed in the human syndrome including heart defects, impaired memory, and abnormal auditory sensorimotor gating. Here we show that Df1/+ mice, like human 22q11DS patients, have substantial rates of hearing loss arising from chronic middle ear infection. Auditory brainstem response (ABR) measurements revealed significant elevation of click-response thresholds in 48% of Df1/+ mice, often in only one ear. Anatomical and histological analysis of the middle ear demonstrated no gross structural abnormalities, but frequent signs of otitis media (OM, chronic inflammation of the middle ear), including excessive effusion and thickened mucosa. In mice for which both in vivo ABR thresholds and post mortem middle-ear histology were obtained, the severity of signs of OM correlated directly with the level of hearing impairment. These results suggest that abnormal auditory sensorimotor gating previously reported in mouse models of 22q11DS could arise from abnormalities in auditory processing. Furthermore, the findings indicate that Df1/+ mice are an excellent model for increased risk of OM in human 22q11DS patients. Given the frequently monaural nature of OM in Df1/+ mice, these animals could also be a powerful tool for investigating the interplay between genetic and environmental causes of OM.

Prevalence of clinical abnormalities in cats found to have nonneoplastic middle ear disease at necropsy: 59 cases (1991-2007).

PubMed

Schlicksup, Michael D; Van Winkle, Thomas J; Holt, David E

2009-10-01

To determine the prevalence of nonneoplastic middle ear disease among cats undergoing necropsy and the prevalence of clinical abnormalities in cats in which nonneoplastic middle ear disease was identified. Retrospective case series. 59 cats that underwent necropsy between January 1991 and August 2007. Medical records were searched to identify cats in which nonneoplastic middle ear disease was identified at necropsy. For cats included in the study, data that were recorded included signalment, initial complaint, whether the cat had any clinical signs of middle or external ear disease, whether the cat had upper respiratory tract disease, necropsy diagnosis, gross appearance of the bullae, and reason for euthanasia. Signs of middle ear disease that were considered included unilateral peripheral vestibular disease without motor deficits, Horner syndrome, and facial nerve paralysis. Of the 3,442 cats that underwent necropsy during the study period, 59 (1.7%) had nonneoplastic middle ear disease. Six of the 59 (10%) cats, including 1 cat that was affected bilaterally, had clinical signs of middle ear disease. Of these, 5 had signs of unilateral peripheral vestibular disease, and 1 had Horner syndrome. Results suggested that most cats with nonneoplastic middle ear disease did not have associated clinical signs. Findings may be of clinical relevance for cats in which middle ear disease is identified as an incidental finding during computed tomography or magnetic resonance imaging for unrelated diseases.

An in vitro model of murine middle ear epithelium.

PubMed

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

2016-11-01

Otitis media (OM), or middle ear inflammation, is the most common paediatric disease and leads to significant morbidity. Although understanding of underlying disease mechanisms is hampered by complex pathophysiology it is clear that epithelial abnormalities underpin the disease. There is currently a lack of a well-characterised in vitro model of the middle ear (ME) epithelium that replicates the complex cellular composition of the middle ear. Here, we report the development of a novel in vitro model of mouse middle ear epithelial cells (mMECs) at an air-liquid interface (ALI) that recapitulates the characteristics of the native murine ME epithelium. We demonstrate that mMECs undergo differentiation into the varied cell populations seen within the native middle ear. Proteomic analysis confirmed that the cultures secrete a multitude of innate defence proteins from their apical surface. We showed that the mMECs supported the growth of the otopathogen, nontypeable Haemophilus influenzae (NTHi), suggesting that the model can be successfully utilised to study host-pathogen interactions in the middle ear. Overall, our mMEC culture system can help to better understand the cell biology of the middle ear and improve our understanding of the pathophysiology of OM. The model also has the potential to serve as a platform for validation of treatments designed to reverse aspects of epithelial remodelling that underpin OM development. © 2016. Published by The Company of Biologists Ltd.

Guide to Understanding Pfeiffer Syndrome

MedlinePlus

... sockets (orbits). Approximately 50% of children with Pfeiffer syndrome have some form of hearing loss secondary to an abnormally small ear canal and middle ear. Dental problems are also common. Visual problems ...

Vestibular evoked myogenic potentials in patients with BPPV.

PubMed

Korres, Stavros; Gkoritsa, Eleni; Giannakakou-Razelou, Dimitra; Yiotakis, Ioannis; Riga, Maria; Nikolpoulos, Thomas P

2011-01-01

The probable cause of Benign Paroxysmal Positional Vertigo (BPPV) is a degeneration of the oto lithic organs (utricle and saccule). The aim of the study is to find possible alterations in Vestibular Evoked Myogenic Potentials (VEMP) recordings in BPPV patients, because the saccule is part of the VEMP pathway. 27 BPPV patients (24 unilateral and 3 bilateral) aged 20 to 70 years and 30 healthy age matched controls. BPPV was diagnosed by the upbeating geotropic nystagmus found in the supine position with the head overextended towards one side. The subjects were investigated with pure tone audiometry, bi-thermal caloric test with electronystagmographic (ENG) recording, and VEMP recording. P1 latency and N1 latency did not present any statistical difference between control ears and affected ears of the BPPV population. The percentage of abnormal VEMP in the BPPV population was statistically higher than in the control ears (p < 0.005). No significant relationship could be shown between the occurrence of Canal Paresis and abnormal VEMP. No relationship was found between the side (right or left ear) where BPPV appeared clinically and the side where abnormal VEMP was registered. BPPV is a clinical entity associated with increased occurrence of abnormal VEMP recordings, possibly due to degeneration of the saccular macula, which is part of the neural VEMP pathway.

Aural symptoms in patients with temporomandibular joint disorders: multiple frequency tympanometry provides objective evidence of changes in middle ear impedance.

PubMed

Riga, Maria; Xenellis, John; Peraki, Eleni; Ferekidou, Elisa; Korres, Stavros

2010-12-01

The association of temporomandibular joint (TMJ) disorders with aural symptoms, such as tinnitus, otic fullness, and subjective decrease of hearing acuity, is a well-established clinical observation. Although several hypotheses have been made about the otic-conductive origin of these complaints, conventional 226-Hz tympanometry has failed to demonstrate any middle ear abnormalities. The aim of this study was to evaluate patients with TMJ disorders with multiple frequency tympanometry (MFT). Prospective clinical study. Outpatient clinic. The population of this study consisted of 40 patients with unilateral TMJ disorders diagnosed for longer than 1 month. After verifying that there were no abnormal otoscopic findings, 226-Hz tympanometry, conventional pure-tone audiometry, brainstem auditory evoked potentials, and MFT were performed. Resonant frequency (RF) values. With the exception of MFT, no abnormal audiologic findings were revealed. The ear ipsilateral to the lesion demonstrated significantly higher (p = 0.002) RF values in comparison to the contralateral ear. The difference in RF values was more obvious in patients aged 45 years or younger. The results of this study imply an increase in the stiffness of the middle ear, which has not been detected by conventional tympanometry. This represents the first concrete documentation of minor alterations in the conductive properties of the middle ear and seems to support the various hypotheses on the middle-ear origin of aural complaints in patients with TMJ disorders. Further studies are needed before a clear insight on the presumably multifactorial pathophysiology of these complaints can finally be reached.

Isolated Sensorineural Hearing Loss as a Sequela after Lightning Strike

PubMed Central

Turan, Mahfuz; Kalkan, Ferhat; Bozan, Nazım; Özçalimli, İsa; Zeki Erdem, Mehmet; Yalınkılıç, Abdülaziz; Garca, Mehmet Fatih

2015-01-01

In most of the surviving patients after a lightning strike, audiovestibular abnormalities have been reported. The most frequently reported type of abnormalities is a tympanic membrane perforation with hearing loss and external ear canal burn. However a sensor neural hearing loss and mixed type hearing loss can also occur, but these occur rarely. A nineteen-year-old female patient had, after a lightning strike, serious burns on the left ear, behind the ear, and on the chest and neck. She also had in her left ear 108 dB hearing loss with irregular central perforation and in her right ear 52 dB sensorineural hearing loss. There was no hearing loss before the strike. A hearing aid was recommended for the right ear and good care and follow-up were recommended for the left ear. A lightning strike can cause serious audiological damage. Therefore, it is necessary to make a careful audiovestibular evaluation of the patients. Although there exist rarely healed cases from sensorineural hearing loss after lightning strike in literature, in our case hearing loss occurred bilaterally and then it healed unilaterally. This condition is quite rare in literature. PMID:26161278

Mirror ear: a reconstructive technique for substantial tragal anomalies or polyotia.

PubMed

Gore, Sinclair M; Myers, Simon R; Gault, David

2006-01-01

Polyotia (mirror ear) is an extremely rarely reported congenital anomaly of the external ear. The aetiology of this condition is unclear, and there are few descriptions of surgical techniques used. We aimed to review our experience with this condition by performing a retrospective review of the cases treated in our unit. Eight cases of polyotia treated at a referral centre for ear reconstruction in a 12 year period (1992-2004) were reviewed. Patient demographic data and associated syndromes were recorded. Operative techniques used in the cases were studied. There was an equal number of males and females. Four patients had abnormal contralateral ears and two patients were formally diagnosed as suffering from a congenital developmental syndrome. Five main components of surgical technique were found to be particularly relevant to these cases. These relate to deconstruction of the defect, management of extra cartilage, management of skin, proximity of the facial nerve and the timing of surgery. The authors conclude that a structured surgical approach can lead to successful reconstruction of these difficult abnormalities.

Refined localization of the Prieto-syndrome locus

DOE Office of Scientific and Technical Information (OSTI.GOV)

Martinez, F.; Prieto, F.; Gal, A.

PRS designates the locus for a syndromal form of X-linked mental retardation (Prieto syndrome) characterized by minor facial anomalies, ear malformation, abnormal growth of teeth, clinodactyly, sacral dimple, patellar luxation, malformation of lower limbs, abnormalities of the fundus of the eye, and subcortical cerebral atrophy. Linkage analysis localized the disease locus between DXS84 (Xp21.1) and DXS255. Here we present additional linkage data that provide further support and refinement of this localization. Individual III-18 gave birth to a male, currently aged 2 7/12 years, who clearly shows delayed psychomotor development. He began to walk at 23 months and his speech ismore » delayed. In addition, he shows the characteristic facial anomalies, {open_quotes}dysplastic{close_quotes} ears, sacral dimple, and clinodactyly, as do all other affected males in this family. 7 refs., 1 tab.« less

Pediatric tinnitus: Incidence of imaging anomalies and the impact of hearing loss.

PubMed

Kerr, Rhorie; Kang, Elise; Hopkins, Brandon; Anne, Samantha

2017-12-01

Guidelines exist for evaluation and management of tinnitus in adults; however lack of evidence in children limits applicability of these guidelines to pediatric patients. Objective of this study is to determine the incidence of inner ear anomalies detected on imaging studies within the pediatric population with tinnitus and evaluate if presence of hearing loss increases the rate of detection of anomalies in comparison to normal hearing patients. Retrospective review of all children with diagnosis of tinnitus from 2010 to 2015 ;at a tertiary care academic center. 102 pediatric patients with tinnitus were identified. Overall, 53 patients had imaging studies with 6 abnormal findings (11.3%). 51/102 patients had hearing loss of which 33 had imaging studies demonstrating 6 inner ear anomalies detected. This is an incidence of 18.2% for inner ear anomalies identified in patients with hearing loss (95% confidence interval (CI) of 7.0-35.5%). 4 of these 6 inner ear anomalies detected were vestibular aqueduct abnormalities. The other two anomalies were cochlear hypoplasia and bilateral semicircular canal dysmorphism. 51 patients had no hearing loss and of these patients, 20 had imaging studies with no inner ear abnormalities detected. There was no statistical difference in incidence of abnormal imaging findings in patients with and without hearing loss (Fisher's exact test, p ;= ;0.072.) CONCLUSION: There is a high incidence of anomalies detected in imaging studies done in pediatric patients with tinnitus, especially in the presence of hearing loss. Copyright © 2017 Elsevier B.V. All rights reserved.

Practical otic anatomy and physiology of the dog and cat.

PubMed

Njaa, Bradley L; Cole, Lynette K; Tabacca, Natalie

2012-11-01

Knowledge of the normal structure and function of the canine and feline ear is critical to be able to diagnose abnormalities that either involve the ear or originate within one or more of the ear compartments. In addition, a veterinarian must be aware of various structures within or associated with the ear so that they are not damaged or destroyed while treating an animal with otic disease. This article provides a brief discussion of the various anatomic features of the ear and normal physiology of portions of the ear. Copyright © 2012 Elsevier Inc. All rights reserved.

Vestibular evoked myogenic potentials in patients with BPPV

PubMed Central

Korres, Stavros; Gkoritsa, Eleni; Giannakakou-Razelou, Dimitra; Yiotakis, Ioannis; Riga, Maria; Nikolpoulos, Thomas P.

2011-01-01

Summary Background The probable cause of Benign Paroxysmal Positional Vertigo (BPPV) is a degeneration of the oto lithic organs (utricle and saccule). The aim of the study is to find possible alterations in Vestibular Evoked Myogenic Potentials (VEMP) recordings in BPPV patients, because the saccule is part of the VEMP pathway. Material/Methods 27 BPPV patients (24 unilateral and 3 bilateral) aged 20 to 70 years and 30 healthy age matched controls. BPPV was diagnosed by the upbeating geotropic nystagmus found in the supine position with the head overextended towards one side. The subjects were investigated with pure tone audiometry, bi-thermal caloric test with electronystagmographic (ENG) recording, and VEMP recording. Results P1 latency and N1 latency did not present any statistical difference between control ears and affected ears of the BPPV population. The percentage of abnormal VEMP in the BPPV population was statistically higher than in the control ears (p<0.005). No significant relationship could be shown between the occurrence of Canal Paresis and abnormal VEMP. No relationship was found between the side (right or left ear) where BPPV appeared clinically and the side where abnormal VEMP was registered. Conclusions BPPV is a clinical entity associated with increased occurrence of abnormal VEMP recordings, possibly due to degeneration of the saccular macula, which is part of the neural VEMP pathway. PMID:21169909

Anatomic Malformations of the Middle and Inner Ear in 22q11.2 Deletion Syndrome: Case Series and Literature Review.

PubMed

Verheij, E; Elden, L; Crowley, T B; Pameijer, F A; Zackai, E H; McDonald-McGinn, D M; Thomeer, H G X M

2018-05-01

The 22q11.2 deletion syndrome is characterized by a heterogenic phenotype, including hearing loss. The underlying cause of hearing loss, especially sensorineural hearing loss, is not yet clear. Therefore, our objective was to describe anatomic malformations in the middle and inner ear in patients with 22q11.2 deletion syndrome. A retrospective case series was conducted in 2 tertiary referral centers. All patients with 22q11.2 deletion syndrome who had undergone CT or MR imaging of the temporal bones were included. Radiologic images were evaluated on predetermined parameters, including abnormalities of the ossicular chain, cochlea, semicircular canals, and vestibule. There were 26 patients (52 ears) with a CT or MR imaging scan available. A dense stapes superstructure was found in 18 ears (36%), an incomplete partition type II was suspected in 12 cochleas (23%), the lateral semicircular canal was malformed with a small bony island in 17 ears (33%), and the lateral semicircular canal and vestibule were fused to a single cavity in 15 ears (29%). Middle and inner ear abnormalities were frequently encountered in our cohort, including malformations of the lateral semicircular canal. © 2018 by American Journal of Neuroradiology.

Hush puppy: a new mouse mutant with pinna, ossicle, and inner ear defects.

PubMed

Pau, Henry; Fuchs, Helmut; de Angelis, Martin Hrabé; Steel, Karen P

2005-01-01

Deafness can be associated with abnormalities of the pinna, ossicles, and cochlea. The authors studied a newly generated mouse mutant with pinna defects and asked whether these defects are associated with peripheral auditory or facial skeletal abnormalities, or both. Furthermore, the authors investigated where the mutation responsible for these defects was located in the mouse genome. The hearing of hush puppy mutants was assessed by Preyer reflex and electrophysiological measurement. The morphological features of their middle and inner ears were investigated by microdissection, paint-filling of the labyrinth, and scanning electron microscopy. Skeletal staining of skulls was performed to assess the craniofacial dimensions. Genome scanning was performed using microsatellite markers to localize the mutation to a chromosomal region. Some hush puppy mutants showed early onset of hearing impairment. They had small, bat-like pinnae and normal malleus but abnormal incus and stapes. Some mutants had asymmetrical defects and showed reduced penetrance of the ear abnormalities. Paint-filling of newborns' inner ears revealed no morphological abnormality, although half of the mice studied were expected to carry the mutation. Reduced numbers of outer hair cells were demonstrated in mutants' cochlea on scanning electron microscopy. Skeletal staining showed that the mutants have significantly shorter snouts and mandibles. Genome scan revealed that the mutation lies on chromosome 8 between markers D8Mit58 and D8Mit289. The study results indicate developmental problems of the first and second branchial arches and otocyst as a result of a single gene mutation. Similar defects are found in humans, and hush puppy provides a mouse model for investigation of such defects.

Wideband energy reflectance findings in presence of normal tympanogram in children with Down's syndrome.

PubMed

Kaf, Wafaa A

2011-02-01

The prevalence of middle ear disorders in children with Down syndrome is higher than normal children due to the associated craniofacial abnormalities. The goal of this study is to evaluate middle ear function using wideband energy reflectance at ambient pressure in 14 young children with Down syndrome and matched control group (2½-5 years old; N=19 ears per group) who each have a normal 226Hz tympanogram. All children underwent otoscopic examination, hearing screening using play audiometry (500-4000Hz), and middle ear testing using 226Hz tympanometry and wideband energy reflectance. The chirp signals for the wideband energy reflectance were presented to the child's ear at 65dB SPL stimulus level and the recording was done over 220-8000Hz range. The measured energy reflectance represents the ratio of the sound energy reflected from the tympanic membrane to the incident sound energy transmitted to the middle ear at a specific frequency. Paired Samples t-test was computed for the mean, 95th, 75th, 25th, and 5th percentile data of each frequency of the two groups. Despite the presence of normal tympanometric findings in both groups, results revealed abnormal wideband energy reflectance findings in 63% of the children with Down syndrome compared to the normal wideband energy reflectance findings in the control group. The mean energy reflectance ratio of the Down syndrome group was abnormally lower than that of the control at 5700-8000Hz (p<0.0005). The 5th and 95th percentile ratios of the Down syndrome group fell outside the 5th and 95th percentile of the control group (p<0.0005). Abnormally low energy reflectance ratios above 4000Hz in the presence of normal tympanograms in the Down syndrome group may suggest associated congenital middle ear anomalies in children with DS. The present findings suggest that wideband energy reflectance has the potential to be of more practical value in children with DS than tympanometry. Further research with a larger number of Down syndrome children will illuminate the potential of wideband energy reflectance in diagnosing middle ear disorders in children with Down syndrome. Copyright © 2010 Elsevier Ireland Ltd. All rights reserved.

A genome-wide association study identifies multiple loci for variation in human ear morphology.

PubMed

Adhikari, Kaustubh; Reales, Guillermo; Smith, Andrew J P; Konka, Esra; Palmen, Jutta; Quinto-Sanchez, Mirsha; Acuña-Alonzo, Victor; Jaramillo, Claudia; Arias, William; Fuentes, Macarena; Pizarro, María; Barquera Lozano, Rodrigo; Macín Pérez, Gastón; Gómez-Valdés, Jorge; Villamil-Ramírez, Hugo; Hunemeier, Tábita; Ramallo, Virginia; Silva de Cerqueira, Caio C; Hurtado, Malena; Villegas, Valeria; Granja, Vanessa; Gallo, Carla; Poletti, Giovanni; Schuler-Faccini, Lavinia; Salzano, Francisco M; Bortolini, Maria-Cátira; Canizales-Quinteros, Samuel; Rothhammer, Francisco; Bedoya, Gabriel; Calderón, Rosario; Rosique, Javier; Cheeseman, Michael; Bhutta, Mahmood F; Humphries, Steve E; Gonzalez-José, Rolando; Headon, Denis; Balding, David; Ruiz-Linares, Andrés

2015-06-24

Here we report a genome-wide association study for non-pathological pinna morphology in over 5,000 Latin Americans. We find genome-wide significant association at seven genomic regions affecting: lobe size and attachment, folding of antihelix, helix rolling, ear protrusion and antitragus size (linear regression P values 2 × 10(-8) to 3 × 10(-14)). Four traits are associated with a functional variant in the Ectodysplasin A receptor (EDAR) gene, a key regulator of embryonic skin appendage development. We confirm expression of Edar in the developing mouse ear and that Edar-deficient mice have an abnormally shaped pinna. Two traits are associated with SNPs in a region overlapping the T-Box Protein 15 (TBX15) gene, a major determinant of mouse skeletal development. Strongest association in this region is observed for SNP rs17023457 located in an evolutionarily conserved binding site for the transcription factor Cartilage paired-class homeoprotein 1 (CART1), and we confirm that rs17023457 alters in vitro binding of CART1.

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

PubMed

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

2003-07-01

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

Mice Haploinsufficient for Ets1 and Fli1 Display Middle Ear Abnormalities and Model Aspects of Jacobsen Syndrome.

PubMed

Carpinelli, Marina R; Kruse, Elizabeth A; Arhatari, Benedicta D; Debrincat, Marlyse A; Ogier, Jacqueline M; Bories, Jean-Christophe; Kile, Benjamin T; Burt, Rachel A

2015-07-01

E26 transformation-specific 1 (ETS1) and friend leukemia integration 1 (FLI1) are members of the ETS family of transcription factors, of which there are 28 in humans. Both genes are hemizygous in Jacobsen syndrome, an 11q contiguous gene deletion disorder involving thrombocytopenia, facial dysmorphism, growth and mental retardation, malformation of the heart and other organs, and hearing impairment associated with recurrent ear infections. To determine whether any of these defects are because of hemizygosity for ETS1 and FLI1, we characterized the phenotype of mice heterozygous for mutant alleles of Ets1 and Fli1. Fli1(+/-) mice displayed mild thrombocytopenia, as did Ets1(+/-)Fli1(+/-) animals. Fli1(+/-) and Ets1(+/-)Fli1(+/-) mice also displayed craniofacial abnormalities, including a small middle ear cavity, short nasal bone, and malformed interface between the nasal bone process and cartilaginous nasal septum. They exhibited hearing impairment, otitis media, fusions of ossicles to the middle ear wall, and deformed stapes. Hearing impairment was more penetrant and stapes malformations were more severe in Ets1(+/-)Fli1(+/-) mice than in Fli1(+/-) mice, indicating partial functional redundancy of these transcription factors during auditory development. Our findings indicate that the short nose, otitis media, and hearing impairment in Jacobsen syndrome are likely because of hemizygosity for ETS1 and FLI1. Copyright © 2015 American Society for Investigative Pathology. Published by Elsevier Inc. All rights reserved.

Wideband reflectance in Down syndrome.

PubMed

Soares, Jordana Costa; Urosas, Juliana Granja; Calarga, Karenina Santos; Pichelli, Tathiany Silva; Limongi, Suelly Cecília Olivan; Shahnaz, Navid; Carvallo, Renata Mota Mamede

2016-08-01

Children with Down syndrome (DS) have a high incidence of middle ear disorders and congenital abnormalities of the external, middle and inner ear. Energy reflectance (ER), a wideband acoustic immittance (WAI) measurement parameter, can measure the sound energy reflected or absorbed in the ear canal over a wider range of frequencies more efficiently and faster than conventional single-tone 226 Hz tympanometry. The aim of the present study was to compare the WAI measurements of children with DS with those of typically developing, normal-hearing children according to their tympanometric findings. Four groups of children with Down syndrome (age range: 2 years and 4 months to 16 years and 3 months; mean age: 8.5 yr) with normal tympanograms (19 ears), flat tympanograms (13 ears), mild negative pressure tympanograms (6 ears between -100 and -199 daPa at the admittance peak) and severe negative pressure tympanograms (4 ears at -200 daPa or lower at the admittance peak) were assessed. All findings were compared with data obtained from 21 ears of a healthy control group (age range: 3 years and 1 month to 13 years and 11 months; mean age: 7.9 yr). The subjects underwent tympanometry with a 226-Hz probe tone frequency and ER measurements along the 200-6,000 Hz range with a chirp stimulus using the Middle-Ear Power Analyzer (MEPA3 - HearID) by Mimosa Acoustics (Champaign, IL), software, version 3.3 [38]. Statistically significant differences were observed in the ER curves for some comparisons between the studied groups. There was also a negative correlation between the static acoustic admittance at the tympanic membrane level and ER measured with a chirp stimulus at 500 and 1,000 Hz. The discriminant analysis technique, which used a chirp stimulus at 1,000 and 1,600 Hz to classify the participants' data based on ER values, achieved a correct classification rate of 59.52% for participants with DS. While groups with abnormal middle ear status, as indicated by tympanometry, showed higher ER values compared to the DS tymp A group and the control group, similar reflectance curves were observed between control group and the DS tymp A group. WAI shows promise as a clinical diagnostic tool in investigating the impact of middle ear disorders in DS group. However, further research is required to investigate this issue in narrower age range group and a larger sample size. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

Craniofacial abnormalities among patients with Edwards Syndrome

PubMed Central

Rosa, Rafael Fabiano M.; Rosa, Rosana Cardoso M.; Lorenzen, Marina Boff; Zen, Paulo Ricardo G.; Graziadio, Carla; Paskulin, Giorgio Adriano

2013-01-01

OBJECTIVE To determine the frequency and types of craniofacial abnormalities observed in patients with trisomy 18 or Edwards syndrome (ES). METHODS This descriptive and retrospective study of a case series included all patients diagnosed with ES in a Clinical Genetics Service of a reference hospital in Southern Brazil from 1975 to 2008. The results of the karyotypic analysis, along with clinical data, were collected from medical records. RESULTS: The sample consisted of 50 patients, of which 66% were female. The median age at first evaluation was 14 days. Regarding the karyotypes, full trisomy of chromosome 18 was the main alteration (90%). Mosaicism was observed in 10%. The main craniofacial abnormalities were: microretrognathia (76%), abnormalities of the ear helix/dysplastic ears (70%), prominent occiput (52%), posteriorly rotated (46%) and low set ears (44%), and short palpebral fissures/blepharophimosis (46%). Other uncommon - but relevant - abnormalities included: microtia (18%), orofacial clefts (12%), preauricular tags (10%), facial palsy (4%), encephalocele (4%), absence of external auditory canal (2%) and asymmetric face (2%). One patient had an initial suspicion of oculo-auriculo-vertebral spectrum (OAVS) or Goldenhar syndrome. CONCLUSIONS: Despite the literature description of a characteristic clinical presentation for ES, craniofacial alterations may be variable among these patients. The OAVS findings in this sample are noteworthy. The association of ES with OAVS has been reported once in the literature. PMID:24142310

Stolbur Phytoplasma Transmission to Maize by Reptalus panzeri and the Disease Cycle of Maize Redness in Serbia

USDA-ARS?s Scientific Manuscript database

Maize redness (MR), induced by stolbur phytoplasma (Candidatus Phytoplasma solani, subgroup 16SrXII-A), is characterized by midrib, leaf and stalk reddening and abnormal ear development. MR has been reported from Serbia, Romania and Bulgaria for 50 years, and recent epiphytotics reduced yields by 4...

Abnormal vessel of the ear associated with congenital arteriovenous malformation in the postauricular region.

PubMed

Dixit, Shilpi Gupta; Dixit, Rakesh

2013-08-01

Variations in the branching pattern of the common, external, and internal carotid arteries can present as arteriovenous malformations, and their basis can be explained embryologically. Our case was a rare variation presenting as a congenital, very gradually increasing bluish painless swelling at the region of the left lobule of the ear arising from an abnormal vessel (from the postauricular artery) which was explored under general anesthesia through a postauricular curved incision. The abnormal vessel and other feeding vessels were ligated and a sclerosing agent injected. Anomalies of pharyngeal arch arteries like our case can be found resulting from the persistence of channels that normally disappear, and prior knowledge of these anomalies is essential before surgeries like mastoidectomy to prevent alarming hemorrhage.

[A new simple method for correcting the prominent ear caused by isolated ventroposition or hypertrophy of the auricle (author's transl)].

PubMed

Janneck, C

1980-04-01

A new operative procedure is described for bat ear abnormality, resulting from isolated hypertrophy of the concha or by a ventriposition of the whole ear. The procedure is described in detail and its advantages are stressed. It is a short operation which does not leave a visible scar and achieves reliable results. The post-operative findings in 11 patients are reported.

Role of skeletal muscle in ear development.

PubMed

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

2017-10-01

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

Computed tomographic findings in 205 dogs with clinical signs compatible with middle ear disease: a retrospective study.

PubMed

Belmudes, Audrey; Pressanti, Charline; Barthez, Paul Y; Castilla-Castaño, Eloy; Fabries, Lionel; Cadiergues, Marie C

2018-02-01

Computed tomography (CT) is considered to be the reference method to evaluate middle ear structures. To evaluate the presence and severity of CT changes in the middle ear and establish if any specific clinical presentations are associated with otitis media. Medical records of animals referred for CT with history and clinical signs consistent with middle ear disease. Retrospective evaluation of CT examinations of tympanic bullae performed over a six year period. Medical records were reviewed for signalment, clinical signs and cytological evaluation of the external ear canal. Dogs were divided into three clinical groups: chronic otitis externa (Group 1), peripheral vestibular disorder (Group 2) and other clinical presentations (Group 3). Group 1 - Of 214 ears, 87 (40.7%) had CT abnormalities: 38 of 87 (17.7%) had material-filled bullae, 42 of 87 (19.6%) had thickened bullae walls and seven of 87 (3.2%) had lysis of the bulla. Abnormalities were significantly more frequent in dogs with suppurative otitis than in erythemato-ceruminous otitis (57% and 23%, respectively; P = 0.003). Proliferative otitis, particularly in French bulldogs, was associated with severe otitis media. Group 2 - Of the 106 ears, 91 (85.8%) had normal tympanic bullae. Group 3 - Of the 26 ears from deaf dogs, 17 had filled bullae; all nine affected dogs were Cavalier King Charles spaniels. All dogs with Claude Bernard Horner syndrome or head tilt had normal tympanic bullae. CT is useful for canine chronic otitis externa, particularly in suppurative or proliferative otitis, even in the absence of associated neurological signs. © 2017 ESVD and ACVD.

Communication routes between intracranial spaces and inner ear: function, pathophysiologic importance and relations with inner ear diseases.

PubMed

Ciuman, Raphael R

2009-01-01

There exist 3 communication routes between the intracranial space and the inner ear, the vestibular aqueduct, the cochlear aqueduct, and the internal auditory canal. They possess a key role in inner ear pressure regulation and fluid homeostasis and are related to inner ear diseases. Relevant literature was reviewed, and the current knowledge of the anatomy, physiologic importance, and relations to inner ear diseases were described. Pathologic communication routes such as semicircular canal dehiscence syndrome were highlighted as well. Abnormalities in all 3 communication routes may predispose or be the cause of distinct inner ear pathologic condition and involved in other cochlear and vestibular syndromes, in which their role is not completely clear. The increasing knowledge of the underlying mechanisms encourages promising approaches for possible intervention in the future.

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

PubMed

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

2015-07-01

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

Surgical management of polyotia.

PubMed

Pan, Bo; Qie, Shuyan; Zhao, Yanyong; Tang, Xiaojun; Lin, Lin; Yang, Qinghua; Zhuang, Hongxing; Jiang, Haiyue

2010-08-01

Polyotia is an extremely rare type of congenital external ear malformation, which is defined as an accessory ear that is large enough to resemble an additional pinna. The terms 'mirror ear' or 'accessory ear' are sometime used. We present our methods in correcting this malformation and summarise the aetiology. The posterior part of the polyotia may presents with a normal ear, a constricted ear or a microtic ear. Free auricular composite tissue transplantation was used to correct the constricted ear. Ear reconstruction was applied in cases of microtia. The anterior auricle was mainly used to form the tragus. 7 cases polyotia were treated between 2004 and 2008. After free auricular composite tissue transplantation the size of the constricted ear and the contralateral ear was similar. In microtia cases the reconstructed ears were natural looking and had a satisfactory three-dimensional contour. The extra tissue of the anterior ear was excised and the tragus was reconstructed. Through operative intervention tailored to the individual case natural-looking and symmetric ears were acquired. The aetiology of polyotia probably relates to abnormal migration of neural crest cell. Copyright 2009 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.

A longitudinal study of hearing and middle ear status of individuals with cleft palate with and without additional malformations/syndromes.

PubMed

Flynn, Traci; Persson, Christina; Moller, Claes; Lohmander, Anette; Magnusson, Lennart

2014-09-01

Objective : To describe and compare the middle ear status and hearing sensitivity in adolescence with isolated cleft palate plus additional malformations and/or syndromes with those with only an isolated cleft palate. Design : Retrospective and longitudinal. Two groups of individuals with isolated cleft palate were compared. Participants : A cohort of individuals born over 4 years in the western region of Sweden. The cohort was divided into one group with isolated cleft palate (n = 31; ICP) and one group with isolated cleft palate plus additional malformations and/or syndromes (n = 37; ICP+). Methods : Middle ear status and hearing thresholds were collected from the medical records at 7, 10, 13, and 16 years of age, examined, and compared within and between groups over time. Results : The ICP+ group demonstrated a significantly higher prevalence of abnormal middle ear status and elevated hearing thresholds as compared with the ICP group. As the individuals aged, the prevalence of abnormal middle ear status decreased. The hearing levels in both groups decreased in the low to middle frequencies as individuals aged; however, the hearing in the high frequencies did not. Conclusions : Individuals with cleft palate need to be followed routinely for middle ear status and hearing thresholds to ensure optimal audiological rehabilitation, with particular attention to those with additional malformations and/or syndromes.

An experimental study of tissue reaction to hyaluronic acid (Restylane) and polymethylmethacrylate (Metacrill) in the mouse.

PubMed

Rosa, Simone C; Macedo, Jefferson L S; Magalhães, Albino V

2012-10-01

The aging skin is a challenge for medical science. Plastic surgeons and dermatologists are called every day to solve problems like filling wrinkles or folds. The material used must be biocompatible because abnormal reactions may cause catastrophic results. This study analyzes the biological behavior of polymethylmethacrylate (Metacrill) and hyaluronic acid (Restylane), using a histopathologic study in mice. A prospective study was performed using 40 mice for each substance: polymethylmethacrylate or hyaluronic acid was injected into the right ear, the left ear been used as a control. Histopathologic analyses of the right ear, liver, and kidney were performed at intervals during the study and revealed the development of a granulomatous reaction with fibrosis and absorption of spheres and signs of liver and kidney sistematization for polymethylmethacrylate. A discrete cellular reaction, with less formation of fibrosis, and no giant cells were seen in the mice injected with hyaluronic acid.

EYA1 mutations associated with the branchio-oto-renal syndrome result in defective otic development in Xenopus laevis

PubMed Central

Li, Youe; Manaligod, Jose M.; Weeks, Daniel L.

2009-01-01

Background information. The BOR (branchio-oto-renal) syndrome is a dominant disorder most commonly caused by mutations in the EYA1 (Eyes Absent 1) gene. Symptoms commonly include deafness and renal anomalies. Results. We have used the embryos of the frog Xenopus laevis as an animal model for early ear development to examine the effects of different EYA1 mutations. Four eya1 mRNAs encoding proteins correlated with congenital anomalies in human were injected into early stage embryos. We show that the expression of mutations associated with BOR, even in the presence of normal levels of endogenous eya1 mRNA, leads to morphologically abnormal ear development as measured by overall otic vesicle size, establishment of sensory tissue and otic innervation. The molecular consequences of mutant eya1 expression were assessed by QPCR (quantitative PCR) analysis and in situ hybridization. Embryos expressing mutant eya1 showed altered levels of multiple genes (six1, dach, neuroD, ngnr-1 and nt3) important for normal ear development. Conclusions. These studies lend support to the hypothesis that dominant-negative effects of EYA1 mutations may have a role in the pathogenesis of BOR. PMID:19951260

Vestibular-evoked myogenic potentials, clinical evaluation, and imaging findings in multiple sclerosis.

PubMed

Güven, Hayat; Bayır, Omer; Aytaç, Emrah; Ozdek, Ali; Comoğlu, Selim Selçuk; Korkmaz, Hakan

2014-02-01

Vestibular-evoked myogenic potentials (VEMP), short-latency electromyographic responses elicited by acoustic stimuli, evaluate the function of vestibulocollic reflex and may give information about brainstem function. The aim of the present study is to evaluate the potential contribution of VEMP to the diagnosis of multiple sclerosis (MS). Fifty patients with MS and 30 healthy control subjects were included in this study. The frequency of VEMP p1-n1 and n2-p2 waves; mean p1, n1, n2, and p2 latency; and mean p1-n1 and n2-p2 amplitude were determined. The relation between clinical and imaging findings and VEMP parameters was evaluated. The p1-n1 and n2-p2 waves were more frequently absent in MS than in control subjects [p1-n1 wave absent: MS, 25 (25 %) ears; control, 6 (10 %) ears; P ≤ 0.02] [n2-p2 wave absent: MS, 44 (44 %) ears; control, 7 (12 %) ears; P ≤ 0.001]. The mean p1-n1 amplitude was lower in MS than in control subjects (MS, 19.1 ± 7.2 μV; control, 23.3 ± 7.4 μV; P ≤ 0.002). A total of 24/50 (48 %) MS patients had VEMP abnormalities (absent responses and/or prolonged latencies). VEMP abnormalities were more frequent in patients with than without vestibular symptoms (P ≤ 0.02) and with brainstem functional system score (FSS) ≥ 1 than FSS = 0 (P ≤ 0.02). In patients with MS, absence of p1-n1 wave was more frequent in patients with than without vestibular symptoms [absence of p1-n1 wave: vestibular symptoms, 9 (45 %) ears; no vestibular symptoms, 16 (20 %) ears; P ≤ 0.03] and patients with Expanded Disability Status Scale (EDSS) score ≥ 5.5 [absence of p1-n1 wave: EDSS ≥ 5.5, 7 (70 %) ears; EDSS <5.5, 18 (20 %) ears; P ≤ 0.001]. Abnormal VEMP may be noted in MS patients, especially those with vestibular symptoms and greater disability. The VEMP test may complement other studies for diagnosis and follow-up of patients with MS.

Characteristics of vestibular evoked myogenic potentials in children with enlarged vestibular aqueduct.

PubMed

Zhou, Guangwei; Gopen, Quinton

2011-01-01

To explore the characteristics of vestibular evoked myogenic potential (VEMP) in children with enlarged vestibular aqueduct (EVA) and to determine the diagnostic value of VEMP testing for this particular inner ear structural anomaly. Retrospective cohort study in a pediatric tertiary care facility. A total of 25 pediatric cases (37 ears) of EVA were identified with complete records, including otologic evaluation, CT scan of the temporal bone, and audiologic assessment. Results of audiometry, tympanometry, and VEMP testing were analyzed. Hearing loss was found in 97% (36/37) of the ears with EVA. Airbone gaps (conductive components) were found in all hearing losses with normal middle ear pressure and mobility. Abnormally low threshold VEMP responses were found in 92% (34/37) of the ears with EVA. VEMP responses were absent unilaterally in three EVA patients who had vestibular complaints. No clear correlation was found between the size of EVA and the audiologic findings. The presence of airbone gaps in children with EVA was found without apparent middle ear pathology. Characteristics of VEMP in EVA were lower thresholds and higher amplitudes despite of the presence of airbone gaps. The abnormally low threshold VEMP responses suggested a "third" window effect in the pathologic condition of EVA. Unilateral absence of VEMP may implicate peripheral vestibular impairment. The findings from our study are helpful in clinical evaluation of young children who usually give limited and ambiguous input regarding their hearing and vestibular problems.

Electrical Stimulation of the Ear, Head, Cranial Nerve, or Cortex for the Treatment of Tinnitus: A Scoping Review

PubMed Central

Adjamian, Peyman

2016-01-01

Tinnitus is defined as the perception of sound in the absence of an external source. It is often associated with hearing loss and is thought to result from abnormal neural activity at some point or points in the auditory pathway, which is incorrectly interpreted by the brain as an actual sound. Neurostimulation therapies therefore, which interfere on some level with that abnormal activity, are a logical approach to treatment. For tinnitus, where the pathological neuronal activity might be associated with auditory and other areas of the brain, interventions using electromagnetic, electrical, or acoustic stimuli separately, or paired electrical and acoustic stimuli, have been proposed as treatments. Neurostimulation therapies should modulate neural activity to deliver a permanent reduction in tinnitus percept by driving the neuroplastic changes necessary to interrupt abnormal levels of oscillatory cortical activity and restore typical levels of activity. This change in activity should alter or interrupt the tinnitus percept (reduction or extinction) making it less bothersome. Here we review developments in therapies involving electrical stimulation of the ear, head, cranial nerve, or cortex in the treatment of tinnitus which demonstrably, or are hypothesised to, interrupt pathological neuronal activity in the cortex associated with tinnitus. PMID:27403346

Evaluation of the Normal Cochlear Second Interscalar Ridge Angle and Depth on 3D T2-Weighted Images: A Tool for the Diagnosis of Scala Communis and Incomplete Partition Type II.

PubMed

Booth, T N; Wick, C; Clarke, R; Kutz, J W; Medina, M; Gorsage, D; Xi, Y; Isaacson, B

2018-05-01

Cochlear malformations may be be subtle on imaging studies. The purpose of this study was to evaluate the angle and depth of the lateral second interscalar ridge or notch in ears without sensorineural hearing loss (normal ears) and compare them with ears that have a documented incomplete type II partition malformation. The second interscalar ridge notch angle and depth were measured on MR imaging in normal ears by a single experienced neuroradiologist. The images of normal and incomplete partition II malformation ears were then randomly mixed for 2 novice evaluators to measure both the second interscalar ridge notch angle and depth in a blinded manner. For the mixed group, interobserver agreement was calculated, normal and abnormal ear measurements were compared, and receiver operating characteristic curves were generated. The 94 normal ears had a mean second interscalar ridge angle of 80.86° ± 11.4° and depth of 0.54 ± 0.14 mm with the 98th percentile for an angle of 101° and a depth of 0.3 mm. In the mixed group, agreement between the 2 readers was excellent, with significant differences for angle and depth found between normal and incomplete partition type II ears for angle and depth on average ( P < .001). Receiver operating characteristic cutoffs for delineating normal from abnormal ears were similar for both readers (depth, 0.31/0.34 mm; angle, 114°/104°). A measured angle of >114° and a depth of the second interscalar ridge notch of ≤0.31 mm suggest the diagnosis of incomplete partition type II malformation and scala communis. These measurements can be accurately made by novice readers. © 2018 by American Journal of Neuroradiology.

Klippel-Feil syndrome and associated ear anomalies.

PubMed

Yildirim, Nadir; Arslanoğlu, Atilla; Mahiroğullari, Mahir; Sahan, Murat; Ozkan, Hüseyin

2008-01-01

Klippel-Feil syndrome (KFS) is a congenital segmentation anomaly of the cervical vertebrae that manifests as short neck, low hair line, and limited neck mobility. Various systemic malformations may also accompany the syndrome including wide variety of otopathologies affecting all 3 compartments of the ear (external, middle, and inner ear) as well as internal acoustic canal and vestibular aqueduct. We aimed to investigate these involvements and their clinical correlates in a group of patients with KFS. We present 20 KFS cases, of which 12 (% 60) displayed most of the reported ear abnormalities such as microtia, external ear canal stenosis, chronic ear inflammations and their sequels, anomalies of the tympanic cavity and ossicles, inner ear dysplasies, deformed internal acoustic canal, and wide vestibular aqueduct, which are demonstrated using the methods of otoscopy, audiologic testing, and temporal bone computed tomography. This series represents one of the highest reported rate of ear involvement in KFS. We found no correlation between the identified ear pathologies and the skeletal and extraskeletal malformations. The genetic nature of the syndrome was supported by the existence of affected family members in 4 (20%) of the cases.

Otologic manifestations of Fanconi anemia and other inherited bone marrow failure syndromes.

PubMed

Kalejaiye, Adedoyin; Giri, Neelam; Brewer, Carmen C; Zalewski, Christopher K; King, Kelly A; Adams, Charleen D; Rosenberg, Philip S; Kim, H Jeffrey; Alter, Blanche P

2016-12-01

The inherited bone marrow failure syndromes (IBMFSs) are diverse disorders with syndrome-specific features; their otologic and audiologic manifestations have not been well described. Our objective was to characterize these in patients with Fanconi anemia (FA), dyskeratosis congenita (DC), Diamond-Blackfan anemia (DBA), and Shwachman-Diamond syndrome (SDS), and to determine the association between physical findings and hearing loss. Patients with an IBMFS underwent comprehensive clinical and laboratory evaluations and testing for syndrome-specific gene mutations. Hearing loss was measured by pure tone audiometry and otologic abnormalities by otomicroscopy. Patients included 33 with FA, 37 with DC, 32 with DBA, and nine with SDS. Hearing loss was most frequent in patients with FA (45%) and DBA (14%). The most common type of hearing loss in FA was conductive (65%). Absent or hypoplastic radius, noted in 21% of the patients with FA, was associated with hearing loss in all cases. Otomicroscopy was abnormal in 66% of patients with FA. Characteristic ear abnormalities included small tympanic membrane (66%), malformed malleus (57%), aberrant tympanic bony island (48%), narrow external auditory canal (EAC) (32%), and abnormal course of chorda tympani (34%). Ear malformations were almost always associated with hearing loss. Hearing loss was rare in patients with DC and SDS. FA is the major IBMFS with associated hearing loss, which is most commonly conductive. Radial hypoplasia or aplasia and characteristic congenital ear malformations are associated with hearing loss in patients with FA. Recognition of these syndrome-specific abnormalities should lead to earlier management of hearing loss. © 2016 Wiley Periodicals, Inc.

Inner ear abnormalities in patients with Goldenhar syndrome.

PubMed

Bisdas, Sotirios; Lenarz, Minoo; Lenarz, Thomas; Becker, Hartmut

2005-05-01

The objective of this study is to investigate the inner ear malformations in patients with Goldenhar syndrome and to hypothesize the potential embryopathogenesis of these malformations. Retrospective case review. Tertiary referral center. Fourteen patients with Goldenhar syndrome. Each patient underwent hearing tests and high-resolution computed tomography (CT) of the temporal bone. In six patients, magnetic resonance imaging of the temporal bone also was performed. Among the 14 patients with Goldenhar syndrome, 13 had outer and middle ear anomalies and 5 (36%) had inner ear malformations, including one case of common cavity. Our observations regarding inner ear anomalies in Goldenhar syndrome correlate with the reported cases in the literature and may help to hypothesize the embryological origin of these malformations, which can caused by an early developmental arrest in the fourth gestational week. Specialists evaluating patients with Goldenhar syndrome should be aware of the possibility of inner ear malformations, which could be diagnosed earlier with appropriate imaging studies.

Neurogenin 1 Null Mutant Ears Develop Fewer, Morphologically Normal Hair Cells in Smaller Sensory Epithelia Devoid of Innervation

PubMed Central

Ma, Qiufu; Anderson, David J.

2000-01-01

The proneuronal gene neurogenin 1 (ngn1) is essential for development of the inner-ear sensory neurons that are completely absent in ngn1 null mutants. Neither afferent, efferent, nor autonomic nerve fibers were detected in the ears of ngn1 null mutants. We suggest that efferent and autonomic fibers are lost secondarily to the absence of afferents. In this article we show that ngn1 null mutants develop smaller sensory epithelia with morphologically normal hair cells. In particular, the saccule is reduced dramatically and forms only a small recess with few hair cells along a duct connecting the utricle with the cochlea. Hair cells of newborn ngn1 null mutants show no structural abnormalities, suggesting that embryonic development of hair cells is independent of innervation. However, the less regular pattern of dispersal within sensory epithelia may be caused by some effects of afferents or to the stunted growth of the sensory epithelia. Tracing of facial and stato-acoustic nerves in control and ngn1 null mutants showed that only the distal, epibranchial, placode-derived sensory neurons of the geniculate ganglion exist in mutants. Tracing further showed that these geniculate ganglion neurons project exclusively to the solitary tract. In addition to the normal complement of facial branchial and visceral motoneurons, ngn1 null mutants have some trigeminal motoneurons and contralateral inner-ear efferents projecting, at least temporarily, through the facial nerve. These data suggest that some neurons in the brainstem (e.g., inner-ear efferents, trigeminal motoneurons) require afferents to grow along and redirect to ectopic cranial nerve roots in the absence of their corresponding sensory roots. PMID:11545141

Classification and Current Management of Inner Ear Malformations.

PubMed

Sennaroğlu, Levent; Bajin, Münir Demir

2017-09-29

Morphologically congenital sensorineural hearing loss can be investigated under two categories. The majority of congenital hearing loss causes (80%) are membranous malformations. Here, the pathology involves inner ear hair cells. There is no gross bony abnormality and, therefore, in these cases high-resolution computerized tomography and magnetic resonance imaging of the temporal bone reveal normal findings. The remaining 20% have various malformations involving the bony labyrinth and, therefore, can be radiologically demonstrated by computerized tomography and magnetic resonance imaging. The latter group involves surgical challenges as well as problems in decision-making. Some cases may be managed by a hearing aid, others need cochlear implantation, and some cases are candidates for an auditory brainstem implantation (ABI). During cochlear implantation, there may be facial nerve abnormalities, cerebrospinal fluid leakage, electrode misplacement or difficulty in finding the cochlea itself. During surgery for inner ear malformations, the surgeon must be ready to modify the surgical approach or choose special electrodes for surgery. In the present review article, inner ear malformations are classified according to the differences observed in the cochlea. Hearing and language outcomes after various implantation methods are closely related to the status of the cochlear nerve, and a practical classification of the cochlear nerve deficiency is also provided.

Prenatal evaluation of the middle ear and diagnosis of middle ear hypoplasia using MRI.

PubMed

Katorza, Eldad; Nahama-Allouche, Catherine; Castaigne, Vanina; Gonzales, Marie; Galliani, Eva; Marlin, Sandrine; Jouannic, Jean-Marie; Rosenblatt, Jonathan; le Pointe, Hubert Ducou; Garel, Catherine

2011-05-01

Analysis of the middle ear with fetal MRI has not been previously reported. To show the contribution of fetal MRI to middle ear imaging. The tympanic cavity was evaluated in 108 fetal cerebral MRI examinations (facial and/or cerebral malformation excluded) and in two cases, one of Treacher Collins syndrome (case 1) and the other of oculo-auriculo-vertebral (OUV) spectrum (case 2) with middle ear hypoplasia identified by MRI at 27 and 36 weeks' gestation, respectively. In all 108 fetuses (mean gestational age 32.5 weeks), the tympanic cavity and T2 hypointensity related to the ossicles were well visualised on both sides. Case 1 had micro/retrognathia and bilateral external ear deformity and case 2 had retrognathism with a left low-set and deformed ear. MRI made it possible to recognize the marked hypoplasia of the tympanic cavity, which was bilateral in case 1 and unilateral in case 2. Both syndromes are characterized by craniofacial abnormalities including middle ear hypoplasia, which cannot be diagnosed with US. The middle ear cavity can be visualized with fetal MRI. We emphasize the use of this imaging modality in the diagnosis of middle ear hypoplasia.

[The experimental research of inner ear metabolism and electrical physiology of autoimmune sensorineural hearing loss].

PubMed

Tan, C; Cao, Y; Hu, P

1998-09-01

Inquire into the mechanism of inner ear pathological physiology in autoimmune sensorineural hearing loss (ASHL). With the auditory electric-physiological techniques and enzyme-histochemical method, the change of inner ear hearing function and enzyme activity were observed. These animals, which threshold of auditory nerve compound active potential (CAP) and cochlear microphonic potential(CM) heightening evidently, showed that the amplitude of endolymphatic potential(EP) (include-EP) bring down in various degrees, which was related to the change of the active of Na(+)-K(+)-ATPase and SDH in vascularis stria and endolymphatic sac. The abnormality of enzymes metabolism in inner ear tissues, which following autoimmune inflammation damage, is the pathological foundation of hearing dysfunction.

Histone deacetylase 1 is required for the development of the zebrafish inner ear

PubMed Central

He, Yingzi; Tang, Dongmei; Li, Wenyan; Chai, Renjie; Li, Huawei

2016-01-01

Histone deacetylase 1 (HDAC1) has been reported to be important for multiple aspects of normal embryonic development, but little is known about its function in the development of mechanosensory organs. Here, we first confirmed that HDAC1 is expressed in the developing otic vesicles of zebrafish by whole-mount in situ hybridization. Knockdown of HDAC1 using antisense morpholino oligonucleotides in zebrafish embryos induced smaller otic vesicles, abnormal otoliths, malformed or absent semicircular canals, and fewer sensory hair cells. HDAC1 loss of function also caused attenuated expression of a subset of key genes required for otic vesicle formation during development. Morpholino-mediated knockdown of HDAC1 resulted in decreased expression of members of the Fgf family in the otic vesicles, suggesting that HDAC1 is involved in the development of the inner ear through regulation of Fgf signaling pathways. Taken together, our results indicate that HDAC1 plays an important role in otic vesicle formation. PMID:26832938

Ear molding in newborn infants with auricular deformities.

PubMed

Byrd, H Steve; Langevin, Claude-Jean; Ghidoni, Lorraine A

2010-10-01

A review of a single physician's experience in managing over 831 infant ear deformities (488 patients) is presented. The authors' methods of molding have advanced from the use of various tapes, glues, and stents, to a comprehensive yet simple system that shapes the antihelix, the triangular fossa, the helical rim, and the overly prominent conchal-mastoid angle (EarWell Infant Ear Correction System). The types of deformities managed, and their relative occurrence, are as follows: (1) prominent/cup ear, 373 ears (45 percent); (2) lidding/lop ear, 224 ears (27 percent); (3) mixed ear deformities, 83 ears (10 percent) (all had associated conchal crus); (4) Stahl's ear, 66 ears (8 percent); (5) helical rim abnormalities, 58 ears (7 percent); (6) conchal crus, 25 ears (3 percent); and (7) cryptotia, two ears (0.2 percent). Bilateral deformities were present in 340 patients (70 percent), with unilateral deformities in 148 patients (30 percent). Fifty-eight infant ears (34 patients) were treated using the final version of the EarWell Infant Ear Correction System with a success rate exceeding 90 percent (good to excellent results). The system was found to be most successful when begun in the first week of the infant's life. When molding was initiated after 3 weeks from birth, only approximately half of the infants had a good response. Congenital ear deformities are common and only approximately 30 percent self-correct. These deformities can be corrected by initiating appropriate molding in the first week of life. Neonatal molding reduces the need for surgical correction with results that often exceed what can be achieved with the surgical alternative.

Floating-Harbor syndrome associated with middle ear abnormalities.

PubMed

Hendrickx, Jan-Jaap; Keymolen, Kathelijn; Desprechins, Brigitte; Casselman, Jan; Gordts, Frans

2010-01-01

Floating-Harbor syndrome is a rare syndrome of unknown etiology, which was first described in 1973. A triad of main features characterizes Floating-Harbor syndrome: short stature, characteristic face, and an expressive speech delay. We present a patient in whom the hearing thresholds improved insufficiently after placement of grommets. High-resolution CT scan of the temporal bone showed a prominent soft-tissue thickening suspected of causing fixation of the malleus, and fusion of the malleus head with the body of the incus. To our knowledge this is the first reported abnormal middle ear anatomy in a patient with Floating-Harbor syndrome. A conservative treatment with hearing aids was preferred as an initial treatment in favor of a surgical exploration.

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

PubMed

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.

Piercing Ear Keloid: Excision Using Loupe Magnification and Topical Liquid Silicone Gel as Adjuvant

PubMed Central

Ramesh, Bellam A.; Mohan, J.

2018-01-01

Background: Keloid is an abnormal growth of scar at the site of skin injury, which usually does not regress. It proliferates beyond the original scar. The ear keloid usually develops after piercing injury to wear ornaments. A patient usually asks for removal of keloid, as it is aesthetically unpleasant. Patient may sometimes complain of itching and pain. Aim: The study was conducted to analyze results following excision of keloid with its tract and topical silicone gel as the postsurgical adjuvant. Materials and Methods: Ear keloids measuring less than 0.5cm or more than 5cm in maximum dimension were excluded from the study. Nonpiercing causes such as burns, trauma, and recurrent keloid were excluded from the study. The study was carried out on 22 patients who had keloid because of piercing injury, including 4 cases with both ear keloids. Of 26 ear keloids, 19 had the tract or connecting tissue. The lesion was excised under anesthesia using magnification. For all the operated cases, topical liquid silicone gel was used as postsurgical adjuvant therapy. The method of application of topical silicone gel was taught to each patient and was considered significant. Result: The magnification helped in identification of tract in 73% of the cases in this study. Twenty patients had successfully responded to proposed treatment, and two patients developed recurrence while using topical silicone gel as the adjuvant. These two patients were managed with conventional triamcinolone injection. Conclusion: The topical silicone gel as postsurgical adjuvant therapy avoided the use of painful postsurgical injection or radiotherapy for the 1–3cm primary ear keloids. The advantages of magnification were better clearance of keloid tissue, easier identification of tract and removal of keloid pseudopods, meticulous suturing, and comfortable elevation of a small local flap. PMID:29731586

Acoustic Reflexes in Normal-Hearing Adults, Typically Developing Children, and Children with Suspected Auditory Processing Disorder: Thresholds, Real-Ear Corrections, and the Role of Static Compliance on Estimates.

PubMed

Saxena, Udit; Allan, Chris; Allen, Prudence

2017-06-01

Previous studies have suggested elevated reflex thresholds in children with auditory processing disorders (APDs). However, some aspects of the child's ear such as ear canal volume and static compliance of the middle ear could possibly affect the measurements of reflex thresholds and thus impact its interpretation. Sound levels used to elicit reflexes in a child's ear may be higher than predicted by calibration in a standard 2-cc coupler, and lower static compliance could make visualization of very small changes in impedance at threshold difficult. For this purpose, it is important to evaluate threshold data with consideration of differences between children and adults. A set of studies were conducted. The first compared reflex thresholds obtained using standard clinical procedures in children with suspected APD to that of typically developing children and adults to test the replicability of previous studies. The second study examined the impact of ear canal volume on estimates of reflex thresholds by applying real-ear corrections. Lastly, the relationship between static compliance and reflex threshold estimates was explored. The research is a set of case-control studies with a repeated measures design. The first study included data from 20 normal-hearing adults, 28 typically developing children, and 66 children suspected of having an APD. The second study included 28 normal-hearing adults and 30 typically developing children. In the first study, crossed and uncrossed reflex thresholds were measured in 5-dB step size. Reflex thresholds were analyzed using repeated measures analysis of variance (RM-ANOVA). In the second study, uncrossed reflex thresholds, real-ear correction, ear canal volume, and static compliance were measured. Reflex thresholds were measured using a 1-dB step size. The effect of real-ear correction and static compliance on reflex threshold was examined using RM-ANOVA and Pearson correlation coefficient, respectively. Study 1 replicated previous studies showing elevated reflex thresholds in many children with suspected APD when compared to data from adults using standard clinical procedures, especially in the crossed condition. The thresholds measured in children with suspected APD tended to be higher than those measured in the typically developing children. There were no significant differences between the typically developing children and adults. However, when real-ear calibrated stimulus levels were used, it was found that children's thresholds were elicited at higher levels than in the adults. A significant relationship between reflex thresholds and static compliance was found in the adult data, showing a trend for higher thresholds in ears with lower static compliance, but no such relationship was found in the data from the children. This study suggests that reflex measures in children should be adjusted for real-ear-to-coupler differences before interpretation. The data in children with suspected APD support previous studies suggesting abnormalities in reflex thresholds. The lack of correlation between threshold and static compliance estimates in children as was observed in the adults may suggest a nonmechanical explanation for age and clinically related effects. American Academy of Audiology

Rerouting the external auditory canal. A method of correcting congenital stenosis.

PubMed

Baron, S H

1975-04-01

An hourglass or funnel-shaped, stenosed, external auditory meatus with a normal tympanic membrane, middle and inner ear is one of the congenital anomalies that occasionally occurs. Such abnormality was present in both ears of a woman and caused chromic otitis externa and deafness. A routine meatoplasty on the right ear failed because of an unusual cephalad position of the drumhead in relation to a "downhill" position of the stenosed outer meatus. Rerouting the ear canal to a horizontal position by removing bone of the canal superiorly, posteriorly, and inferiorly, and grafting the now horizontal canal with skin taken from the postauricular fold produced a good result. This is a satisfactory procedure for a woman, but would be cosmetically unacceptable for a man.

FGF23 Deficiency Leads to Mixed Hearing Loss and Middle Ear Malformation in Mice

PubMed Central

Lysaght, Andrew C.; Yuan, Quan; Fan, Yi; Kalwani, Neil; Caruso, Paul; Cunnane, MaryBeth; Lanske, Beate; Stanković, Konstantina M.

2014-01-01

Fibroblast growth factor 23 (FGF23) is a circulating hormone important in phosphate homeostasis. Abnormal serum levels of FGF23 result in systemic pathologies in humans and mice, including renal phosphate wasting diseases and hyperphosphatemia. We sought to uncover the role FGF23 plays in the auditory system due to shared molecular mechanisms and genetic pathways between ear and kidney development, the critical roles multiple FGFs play in auditory development and the known hearing phenotype in mice deficient in klotho (KL), a critical co-factor for FGF23 signaling. Using functional assessments of hearing, we demonstrate that Fgf mice are profoundly deaf. Fgf mice have moderate hearing loss above 20 kHz, consistent with mixed conductive and sensorineural pathology of both middle and inner ear origin. Histology and high-voltage X-ray computed tomography of Fgf mice demonstrate dysplastic bulla and ossicles; Fgf mice have near-normal morphology. The cochleae of mutant mice appear nearly normal on gross and microscopic inspection. In wild type mice, FGF23 is ubiquitously expressed throughout the cochlea. Measurements from Fgf mice do not match the auditory phenotype of Kl −/− mice, suggesting that loss of FGF23 activity impacts the auditory system via mechanisms at least partially independent of KL. Given the extensive middle ear malformations and the overlap of initiation of FGF23 activity and Eustachian tube development, this work suggests a possible role for FGF23 in otitis media. PMID:25243481

Aging and the 4 kHz Air-bone Gap

PubMed Central

Nondahl, David M.; Tweed, Ted S.; Cruickshanks, Karen J.; Wiley, Terry L.; Dalton, Dayna S.

2011-01-01

Purpose To assess age- and gender-related patterns in the prevalence and 10-year incidence of 4 kHz air-bone gaps, and associated factors. Method Data were obtained as part of the longitudinal, population-based Epidemiology of Hearing Loss Study. An air-bone gap at 4 kHz was defined as an air-conduction threshold ≥15 dB higher than the bone-conduction threshold in the right ear. Results Among 3,553 participants aged 48 to 92 years at baseline (1993-1995), 3.4% had a 4 kHz air-bone gap in the right ear. The prevalence increased with age. Among the 120 participants with an air-bone gap, 60.0% did not have a flat tympanogram or an air-bone gap at .5 kHz. Ten years later we assessed 2093 participants who did not have a 4 kHz air-bone gap at baseline; 9.2% had developed a 4 kHz air-bone gap in the right ear. The incidence increased with age. Among the 192 participants who had developed an air-bone gap, 60.9% did not have a flat tympanogram or air-bone gaps at other frequencies. Conclusions These results suggest that a finding of a 4 kHz air-bone gap may reflect a combination of aging and other factors and not necessarily exclusively abnormal middle ear function. PMID:22232408

Congenital middle ear abnormalities with absence of the oval window: diagnosis, surgery, and audiometric outcomes.

PubMed

Su, Yu; Yuan, Hu; Song, Yue-shuai; Shen, Wei-dong; Han, Wei-ju; Liu, Jun; Han, Dong-yi; Dai, Pu

2014-08-01

Congenital absence of the oval window (CAOW) is a rare condition in which the stapes footplate fails to develop, resulting in a significant conductive hearing loss in the affected ear. The purpose of this study was to describe the surgical management and outcomes of patients with CAOW undergoing the oval window drill-out (OWD) procedure. A retrospective chart review of patients with CAOW between 1996 and 2011 was performed. Clinical data of patients who underwent OWD were collected. Seventy-nine patients (103 ears) were confirmed using exploratory tympanotomy as having congenital stapes anomalies and CAOW without any anomalies of the tympanic membrane and external auditory canal. Demographic data, CT findings, operative findings, complications, and preoperative/postoperative audiometry data of patients who underwent OWD were collected. The preoperative and postoperative audiologic findings were analyzed in 42 patients (56 ears) with complete data. Hearing restoration surgery was aborted for various reasons in 14 cases. Six patients underwent revision operations for worsening hearing after their first surgery. The average preoperative 4 tone air conduction threshold was 67 dB; the average 6-month postoperative four tone air conduction threshold was 49 dB, and the average postoperative hearing gain was 18 dB. For the 56 ears, the average 4 tone air conduction threshold 6 months after surgery was significantly lower than the preoperative threshold. The oval window drill-out procedure is a viable operation for patients with congenital absence of the oval window, and it is important for surgeons to develop personalized treatment programs to improve patients' hearing with minimal complications.

A comprehensive catalogue of the coding and non-coding transcripts of the human inner ear

PubMed Central

Corneveaux, Jason J.; Ohmen, Jeffrey; White, Cory; Allen, April N.; Lusis, Aldons J.; Van Camp, Guy; Huentelman, Matthew J.; Friedman, Rick A.

2015-01-01

The mammalian inner ear consists of the cochlea and the vestibular labyrinth (utricle, saccule, and semicircular canals), which participate in both hearing and balance. Proper development and life-long function of these structures involves a highly complex coordinated system of spatial and temporal gene expression. The characterization of the inner ear transcriptome is likely important for the functional study of auditory and vestibular components, yet, primarily due to tissue unavailability, detailed expression catalogues of the human inner ear remain largely incomplete. We report here, for the first time, comprehensive transcriptome characterization of the adult human cochlea, ampulla, saccule and utricle of the vestibule obtained from patients without hearing abnormalities. Using RNA-Seq, we measured the expression of >50,000 predicted genes corresponding to approximately 200,000 transcripts, in the adult inner ear and compared it to 32 other human tissues. First, we identified genes preferentially expressed in the inner ear, and unique either to the vestibule or cochlea. Next, we examined expression levels of specific groups of potentially interesting RNAs, such as genes implicated in hearing loss, long non-coding RNAs, pseudogenes and transcripts subject to nonsense mediated decay (NMD). We uncover the spatial specificity of expression of these RNAs in the hearing/balance system, and reveal evidence of tissue specific NMD. Lastly, we investigated the non-syndromic deafness loci to which no gene has been mapped, and narrow the list of potential candidates for each locus. These data represent the first high-resolution transcriptome catalogue of the adult human inner ear. A comprehensive identification of coding and non-coding RNAs in the inner ear will enable pathways of auditory and vestibular function to be further defined in the study of hearing and balance. Expression data are freely accessible at https://www.tgen.org/home/research/research-divisions/neurogenomics/supplementary-data/inner-ear-transcriptome.aspx PMID:26341477

Auditory Brainstem Responses in Young Males with Fragile X Syndrome

ERIC Educational Resources Information Center

Roberts, Joanne; Hennon, Elizabeth A.; Anderson, Kathleen; Roush, Jackson; Gravel, Judith; Skinner, Martie; Misenheimer, Jan; Reitz, Patricia

2005-01-01

Fragile X syndrome (FXS) is the most common inherited cause of mental retardation resulting in developmental delays in males. Atypical outer ear morphology is characteristic of FXS and may serve as a marker for abnormal auditory function. Despite this abnormality, studies of the hearing of young males with FXS are generally lacking. A few studies…

Passive smoking, salivary cotinine concentrations, and middle ear effusion in 7 year old children.

PubMed

Strachan, D P; Jarvis, M J; Feyerabend, C

1989-06-10

To assess the contribution of passive exposure to tobacco smoke to the development of middle ear underpressure and effusion. Cross sectional observational study. One third of the primary schools in Edinburgh. 892 Children aged 6 1/2 to 7 1/2 were examined, and satisfactory tympanograms were obtained in 872. Results of assay of salivary cotinine concentrations were available for 770 children, and satisfactory tympanograms were available for 736 of these. Correlation of the prevalence of middle ear underpressure and effusion with concentrations of the marker of nicotine, cotinine, in the saliva of the children. Middle ear pressure and compliance were measured in both ears by impedance tympanometry. Salivary cotinine concentrations were assayed by gas-liquid chromatography. Cotinine concentrations increased with the number of smokers in the household. Girls had higher concentrations than boys, and children living in rented housing had higher concentrations than those living in housing owned by their parents. There was a trend towards more abnormal tympanometric findings with increasing cotinine concentration, the odds ratio for a doubling of the cotinine concentration being 1.14 (95% confidence interval 1.03 to 1.27). After adjustment for the sex of the child and housing tenure the odds ratio for a doubling of the cotinine concentration was 1.13 (1.00 to 1.28). The results of this study are consistent with those of case-control studies of children attending for an operation to relieve middle ear effusion. They indicate that the disease should be added to the list of recognised hazards associated with passive smoking. About one third of the cases of middle ear effusion in this study were statistically attributable to exposure to tobacco smoke.

Leukoencephalopathy with thalamus and brainstem involvement and high lactate 'LTBL' caused by EARS2 mutations.

PubMed

Steenweg, Marjan E; Ghezzi, Daniele; Haack, Tobias; Abbink, Truus E M; Martinelli, Diego; van Berkel, Carola G M; Bley, Annette; Diogo, Luisa; Grillo, Eugenio; Te Water Naudé, Johann; Strom, Tim M; Bertini, Enrico; Prokisch, Holger; van der Knaap, Marjo S; Zeviani, Massimo

2012-05-01

In the large group of genetically undetermined infantile-onset mitochondrial encephalopathies, multiple defects of mitochondrial DNA-related respiratory-chain complexes constitute a frequent biochemical signature. In order to identify responsible genes, we used exome-next-generation sequencing in a selected cohort of patients with this biochemical signature. In an isolated patient, we found two mutant alleles for EARS2, the gene encoding mitochondrial glutamyl-tRNA synthetase. The brain magnetic resonance imaging of this patient was hallmarked by extensive symmetrical cerebral white matter abnormalities sparing the periventricular rim and symmetrical signal abnormalities of the thalami, midbrain, pons, medulla oblongata and cerebellar white matter. Proton magnetic resonance spectroscopy showed increased lactate. We matched this magnetic resonance imaging pattern with that of a cohort of 11 previously selected unrelated cases. We found mutations in the EARS2 gene in all. Subsequent detailed clinical and magnetic resonance imaging based phenotyping revealed two distinct groups: mild and severe. All 12 patients shared an infantile onset and rapidly progressive disease with severe magnetic resonance imaging abnormalities and increased lactate in body fluids and proton magnetic resonance spectroscopy. Patients in the 'mild' group partially recovered and regained milestones in the following years with striking magnetic resonance imaging improvement and declining lactate levels, whereas those of the 'severe' group were characterized by clinical stagnation, brain atrophy on magnetic resonance imaging and persistent lactate increases. This new neurological disease, early-onset leukoencephalopathy with thalamus and brainstem involvement and high lactate, is hallmarked by unique magnetic resonance imaging features, defined by a peculiar biphasic clinical course and caused by mutations in a single gene, EARS2, expanding the list of medically relevant defects of mitochondrial DNA translation.

Genetic Architecture of Ear Fasciation in Maize (Zea mays) under QTL Scrutiny

PubMed Central

Mendes-Moreira, Pedro; Alves, Mara L.; Satovic, Zlatko; dos Santos, João Pacheco; Santos, João Nina; Souza, João Cândido; Pêgo, Silas E.; Hallauer, Arnel R.; Vaz Patto, Maria Carlota

2015-01-01

Maize ear fasciation Knowledge of the genes affecting maize ear inflorescence may lead to better grain yield modeling. Maize ear fasciation, defined as abnormal flattened ears with high kernel row number, is a quantitative trait widely present in Portuguese maize landraces. Material and Methods Using a segregating population derived from an ear fasciation contrasting cross (consisting of 149 F2:3 families) we established a two location field trial using a complete randomized block design. Correlations and heritabilities for several ear fasciation-related traits and yield were determined. Quantitative Trait Loci (QTL) involved in the inheritance of those traits were identified and candidate genes for these QTL proposed. Results and Discussion Ear fasciation broad-sense heritability was 0.73. Highly significant correlations were found between ear fasciation and some ear and cob diameters and row number traits. For the 23 yield and ear fasciation-related traits, 65 QTL were identified, out of which 11 were detected in both environments, while for the three principal components, five to six QTL were detected per environment. Detected QTL were distributed across 17 genomic regions and explained individually, 8.7% to 22.4% of the individual traits or principal components phenotypic variance. Several candidate genes for these QTL regions were proposed, such as bearded-ear1, branched silkless1, compact plant1, ramosa2, ramosa3, tasselseed4 and terminal ear1. However, many QTL mapped to regions without known candidate genes, indicating potential chromosomal regions not yet targeted for maize ear traits selection. Conclusions Portuguese maize germplasm represents a valuable source of genes or allelic variants for yield improvement and elucidation of the genetic basis of ear fasciation traits. Future studies should focus on fine mapping of the identified genomic regions with the aim of map-based cloning. PMID:25923975

The Existence of a Natural Plica at the Anatomical Base of the Antihelix and its Surgical Importance to Address Protruding Ears: An Anatomicosurgical Study.

PubMed

Oliveira, Miguel Marques; Oliveira, Daniel Sousa Marques; Oliveira, Gustavo Sousa Marques

2017-04-01

Protruding ears represent the main abnormality of the external ear, which has required numerous anatomic and surgical studies. Most studies give attention to the absence of the antihelix as the anatomic defect responsible for the clinical deformity of the lateral aspect of the ear that leads to its anteversion. The reason for this study is the controversial origin of the fold of the antihelix within the auricle framework, a field of interest for aesthetic otoplasty. The current study examined the medial surface of the cartilaginous ear frame from cadaver specimens with right morphology to investigate the starting point of the fold of the antihelix. This allowed for verification of a natural plica at the anatomic base of this antihelical fold, which to date has not had its topography described morphologically. It is acknowledged that relevant literature makes no reference to this innominate natural plica at the origin of the antihelix, whose anatomic and surgical importance is related in this report. This study aimed to show that the existence of a natural plica at the base of the antihelix in ear framing represents a landmark between normal and protruding ear morphology. For 8 years, 118 ears were carefully investigated within rigid ethical principles based on a thorough review of the pertinent literature. The study investigated 16 selected cadaver specimens and 102 protruding ears dissected by the senior author including 49 bilateral cases (26 males and 23 females) and 4 unilateral cases (2 males and 2 females). Bifacial anthropometric measurements by calipers were used for documentation. A natural plica at the base of the antihelix was found in all cadaver ears selected with right morphology, whereas it was totally absent in every surgically treated protruding ear irrespective of color, gender, age, or ethnic origin. Ambilateral measures of the antihelix eminence certify the study object in normal specimens as well as its lack in abnormal ones. Technical and topographic knowledge that a natural plica exists at the anatomic base of the antihelix is a valuable key point in recognizing the normal external ear. In addition, the making of a natural plica is the first and most effective factor in the reconstruction of the antihelical fold and its absolute absence results in the pathologic condition for protruding ears. This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the A3 online Instructions to Authors. http://www.springer.com/00266 .

Chest MRI

MedlinePlus

... heart valves Heart defibrillator or pacemaker Inner ear (cochlear) implants Kidney disease or are on dialysis (you ... Diagnose abnormal growths in the chest Evaluate blood flow Show lymph nodes and blood vessels Show the ...

Potter syndrome

MedlinePlus

... Widely separated eyes with epicanthal folds , broad nasal bridge , low set ears , and receding chin Absence of ... Names Potter phenotype Images Amniotic fluid Broad nasal bridge References Copelovitch L, Kaplan BS. Developmental abnormalities of ...

A zebrafish model for Waardenburg syndrome type IV reveals diverse roles for Sox10 in the otic vesicle.

PubMed

Dutton, Kirsten; Abbas, Leila; Spencer, Joanne; Brannon, Claire; Mowbray, Catriona; Nikaido, Masataka; Kelsh, Robert N; Whitfield, Tanya T

2009-01-01

In humans, mutations in the SOX10 gene are a cause of the auditory-pigmentary disorder Waardenburg syndrome type IV (WS4) and related variants. SOX10 encodes an Sry-related HMG box protein essential for the development of the neural crest; deafness in WS4 and other Waardenburg syndromes is usually attributed to loss of neural-crest-derived melanocytes in the stria vascularis of the cochlea. However, SOX10 is strongly expressed in the developing otic vesicle and so direct roles for SOX10 in the otic epithelium might also be important. Here, we examine the otic phenotype of zebrafish sox10 mutants, a model for WS4. As a cochlea is not present in the fish ear, the severe otic phenotype in these mutants cannot be attributed to effects on this tissue. In zebrafish sox10 mutants, we see abnormalities in all otic placodal derivatives. Gene expression studies indicate deregulated expression of several otic genes, including fgf8, in sox10 mutants. Using a combination of mutant and morphant data, we show that the three sox genes belonging to group E (sox9a, sox9b and sox10) provide a link between otic induction pathways and subsequent otic patterning: they act redundantly to maintain sox10 expression throughout otic tissue and to restrict fgf8 expression to anterior macula regions. Single-cell labelling experiments indicate a small and transient neural crest contribution to the zebrafish ear during normal development, but this is unlikely to account for the strong defects seen in the sox10 mutant. We discuss the implication that the deafness in WS4 patients with SOX10 mutations might reflect a haploinsufficiency for SOX10 in the otic epithelium, resulting in patterning and functional abnormalities in the inner ear.

Ear Infection in Isolated Cleft Lip: Etiological Implications.

PubMed

Ruegg, Teresa A; Cooper, Margaret E; Leslie, Elizabeth J; Ford, Matthew D; Wehby, George L; Deleyiannis, Frederic W B; Czeizel, Andrew E; Hecht, Jacqueline T; Marazita, Mary L; Weinberg, Seth M

2017-03-01

  Chronic ear infections are a common occurrence in children with orofacial clefts involving the secondary palate. Less is known about the middle ear status of individuals with isolated clefts of the lip, although several studies have reported elevated rates of ear infection in this group. The purpose of this retrospective study was to test the hypothesis that chronic ear infections occur more frequently in isolated cleft lip cases (n = 94) compared with controls (n = 183).   A questionnaire was used to obtain information on history of chronic ear infection. The association between ear infection status (present/absent) and cleft lip status (cleft lip case/control) was tested using both chi-square and logistic regression.   The reported occurrence of chronic ear infection was significantly greater in cleft lip cases (31%) compared with unaffected controls (11%). After adjusting for age and sex, having a cleft lip increased the odds of being positive for ear infection by a factor greater than 3 (odds ratio = 3.698; 95% confidence interval = 1.91 to 7.14). Within cleft lip cases, there was no difference in the occurrence of ear infection by defect laterality or by the type of clefting present in the family history. Although velopharyngeal insufficiency was present in 18.4% of our cleft lip sample, there was no statistical association between ear infection and abnormal speech patterns. These results may have potential implications both for the clinical management of isolated cleft lip cases and for understanding the etiology of orofacial clefting.

Inner ear problems of Thai priest at Priest Hospital.

PubMed

Karnchanakas, Taweporn; Tantanavat, Are; Sinsakontavat, Jamjan

2008-01-01

The inner ear problems of Thai priest at Priest Hospital had never been reported previously, so Department of Ear Nose Throat try to correlate the metebotic disorder with inner ear problems. 1) To study the fasting blood sugar (FBS), total cholesterol (T. Chol), low density lipoprotein (LDL), and triglyceride (TG), the factors expected to involve in inner ear problems of priests at Priest Hospital. 2) To compare the FBS, T. Chol, HDL, LDL, and TG of priests with inner ear problems at Priest Hospital. 3) To find the percentage of abnormal from FBS, T. Chol, LDL, and TG. The study using 83 sampling of priests with inner ear problems and 107 priests as a controlled group. The research instruments used to collect data was the questionnaire which composed of general information, physical, ear-nose-throat and neurological examination, pure tone audiometry, brainstem evoke response audiometry (BERA) and the blood tests:FBS, T. Chol, TG, and LDL. The inner ear problems were composed of: 1) Dizziness 2) Hearing Loss 3) Tinnitus Aurium. The descriptive statistics were used to analyze the data from questionnaires and utilized frequency, percentage, standard deviation (S.D.) and t-test to achieve desired results. Priest at middle age and elderly with inner ear problems had greater FBS and TG than expected values of the control group. The middle age and elderly priests who had greater FBS and TG than expected values were sick with inner ear problems that causing dizziness, hearing loss and tinnitus aurium.

Ear Infection in Isolated Cleft Lip: Etiological Implications

PubMed Central

Ruegg, Teresa A.; Cooper, Margaret E.; Leslie, Elizabeth J.; Ford, Matthew D.; Wehby, George L.; Deleyiannis, Frederic W.B.; Czeizel, Andrew E.; Hecht, Jacqueline T.; Marazita, Mary L.; Weinberg, Seth M.

2015-01-01

Background and Hypothesis Chronic ear infections are a common occurrence in children with orofacial clefts involving the secondary palate. Less is known about the middle ear status of individuals with isolated clefts of the lip, although several studies have reported elevated rates of ear infection in this group. The purpose of this retrospective study was to test the hypothesis that chronic ear infections occur more frequently in isolated cleft lip cases (n=94) compared with controls (n=183). Methods A questionnaire was used to obtain information on history of chronic ear infection. The association between ear infection status (present/absent) and cleft lip status (cleft lip case/control) was tested using both chi-square and logistic regression. Results and Conclusions The reported occurrence of chronic ear infection was significantly greater in cleft lipcases (31%) compared to unaffected controls (11%). After adjusting for age and sex, having a cleft lip increased the odds of being positive for ear infection by a factor greater than three (OR=3.698; 95%CI=1.91–7.14). Within cleft lipcases, there was no difference in the occurrence of ear infection by defect laterality or by the type of clefting present in the family history. Although velopharyngeal insufficiency was present in 18.4% of our cleft lip sample, there was no statistical association between ear infection and abnormal speech patterns. These results may have potential implications both for the clinical management of isolated cleft lip cases and for understanding the etiology of orofacial clefting. PMID:26153759

Cloning of calves from various somatic cell types of male and female adult, newborn and fetal cows.

PubMed

Kato, Y; Tani, T; Tsunoda, Y

2000-11-01

Twenty-four calves were cloned from six somatic cell types of female and male adult, newborn and fetal cows. The clones were derived from female cumulus (n = 3), oviduct (n = 2) and uterine (n = 2) cells, female and male skin cells (n = 10), and male ear (n = 5) and liver (n = 2) cells. On the basis of the number of cloned embryos transferred (n = 172) to surrogate cows, the overall rate of success was 14%, but based on the number of surrogate mothers that became pregnant (n = 50), the success rate was 48%. Cell nuclei from uterus, ear and liver cells, which have not been tested previously, developed into newborn calves after nuclear transfer into enucleated oocytes. To date, seven female and six male calves have survived: six of the females were from adult cells (cumulus (n = 3), oviduct (n = 2) and skin (n = 1) cells) and one was from newborn skin cells, whereas the male calves were derived from adult ear cells (n = 3), newborn liver and skin cells (n = 2), and fetal cells (n = 1). Clones derived from adult cells frequently aborted in the later stages of pregnancy and calves developing to term showed a higher number of abnormalities than did those derived from newborn or fetal cells. The telomeric DNA lengths in the ear cells of three male calves cloned from the ear cells of a bull aged 10 years were similar to those of the original bull. However, the telomeric DNA lengths from the white blood cells of the clones, although similar to those in an age-matched control, were shorter than those of the original bull, which indicates that telomeric shortening varies among tissues.

Investigation of bacterial biofilm in the human middle ear using optical coherence tomography and acoustic measurements

PubMed Central

Nguyen, Cac T.; Robinson, Sarah R.; Jung, Woonggyu; Novak, Michael A.; Boppart, Stephen A.; Allen, Jont B.

2013-01-01

Children with chronic otitis media (OM) often have conductive hearing loss which results in communication difficulties and requires surgical treatment. Recent studies have provided clinical evidence that there is a one-to-one correspondence between chronic OM and the presence of a bacterial biofilm behind the tympanic membrane (TM). Here we investigate the acoustic effects of bacterial biofilms, confirmed using optical coherence tomography (OCT), in adult ears. Non-invasive OCT images are collected to visualize the cross-sectional structure of the middle ear, verifying the presence of a biofilm behind the TM. Wideband measurements of acoustic reflectance and impedance (0.2 to 6 [kHz]) are used to study the acoustic properties of ears with confirmed bacterial biofilms. Compared to known acoustic properties of normal middle ears, each of the ears with a bacterial biofilm has an elevated power reflectance in the 1 to 3 [kHz] range, corresponding to an abnormally small resistance (real part of the impedance). These results provide assistance for the clinical diagnosis of a bacterial biofilm, which could lead to improved treatment of chronic middle ear infection and further understanding of the impact of chronic OM on conductive hearing loss. PMID:23588039

Genetic Architecture of Ear Fasciation in Maize (Zea mays) under QTL Scrutiny.

PubMed

Mendes-Moreira, Pedro; Alves, Mara L; Satovic, Zlatko; Dos Santos, João Pacheco; Santos, João Nina; Souza, João Cândido; Pêgo, Silas E; Hallauer, Arnel R; Vaz Patto, Maria Carlota

2015-01-01

Knowledge of the genes affecting maize ear inflorescence may lead to better grain yield modeling. Maize ear fasciation, defined as abnormal flattened ears with high kernel row number, is a quantitative trait widely present in Portuguese maize landraces. Using a segregating population derived from an ear fasciation contrasting cross (consisting of 149 F2:3 families) we established a two location field trial using a complete randomized block design. Correlations and heritabilities for several ear fasciation-related traits and yield were determined. Quantitative Trait Loci (QTL) involved in the inheritance of those traits were identified and candidate genes for these QTL proposed. Ear fasciation broad-sense heritability was 0.73. Highly significant correlations were found between ear fasciation and some ear and cob diameters and row number traits. For the 23 yield and ear fasciation-related traits, 65 QTL were identified, out of which 11 were detected in both environments, while for the three principal components, five to six QTL were detected per environment. Detected QTL were distributed across 17 genomic regions and explained individually, 8.7% to 22.4% of the individual traits or principal components phenotypic variance. Several candidate genes for these QTL regions were proposed, such as bearded-ear1, branched silkless1, compact plant1, ramosa2, ramosa3, tasselseed4 and terminal ear1. However, many QTL mapped to regions without known candidate genes, indicating potential chromosomal regions not yet targeted for maize ear traits selection. Portuguese maize germplasm represents a valuable source of genes or allelic variants for yield improvement and elucidation of the genetic basis of ear fasciation traits. Future studies should focus on fine mapping of the identified genomic regions with the aim of map-based cloning.

Binaural Pitch Fusion in Bilateral Cochlear Implant Users.

PubMed

Reiss, Lina A J; Fowler, Jennifer R; Hartling, Curtis L; Oh, Yonghee

Binaural pitch fusion is the fusion of stimuli that evoke different pitches between the ears into a single auditory image. Individuals who use hearing aids or bimodal cochlear implants (CIs) experience abnormally broad binaural pitch fusion, such that sounds differing in pitch by as much as 3-4 octaves are fused across ears, leading to spectral averaging and speech perception interference. The goal of this study was to determine if adult bilateral CI users also experience broad binaural pitch fusion. Stimuli were pulse trains delivered to individual electrodes. Fusion ranges were measured using simultaneous, dichotic presentation of reference and comparison stimuli in opposite ears, and varying the comparison stimulus to find the range that fused with the reference stimulus. Bilateral CI listeners had binaural pitch fusion ranges varying from 0 to 12 mm (average 6.1 ± 3.9 mm), where 12 mm indicates fusion over all electrodes in the array. No significant correlations of fusion range were observed with any subject factors related to age, hearing loss history, or hearing device history, or with any electrode factors including interaural electrode pitch mismatch, pitch match bandwidth, or within-ear electrode discrimination abilities. Bilateral CI listeners have abnormally broad fusion, similar to hearing aid and bimodal CI listeners. This broad fusion may explain the variability of binaural benefits for speech perception in quiet and in noise in bilateral CI users.

Aronia melanocarpa Concentrate Ameliorates Pro-Inflammatory Responses in HaCaT Keratinocytes and 12-O-Tetradecanoylphorbol-13-Acetate-Induced Ear Edema in Mice.

PubMed

Goh, Ah Ra; Youn, Gi Soo; Yoo, Ki-Yeon; Won, Moo Ho; Han, Sang-Zin; Lim, Soon Sung; Lee, Keun Wook; Choi, Soo Young; Park, Jinseu

2016-07-01

Abnormal expression of pro-inflammatory mediators such as cell adhesion molecules and cytokines has been implicated in various inflammatory skin diseases, including atopic dermatitis. In this study, we investigated the anti-inflammatory activity of Aronia melanocarpa concentrate (AC) and its action mechanisms using in vivo and in vitro skin inflammation models. Topical application of AC on mouse ears significantly suppressed 12-O-tetradecanoylphorbol-13-acetate (TPA)-induced ear edema formation, as judged by measuring ear thickness and weight, and histological analysis. Topical administration of AC also reduced the expression of pro-inflammatory cytokines such as TNF-α, IL-1β, and IL-6 in TPA-stimulated mouse ears. Pretreatment with AC suppressed TNF-α-induced ICAM-I expression and subsequent monocyte adhesiveness in human keratinocyte cell line HaCaT. In addition, AC significantly decreased intracellular reactive oxygen species (ROS) generation as well as mitogen-activated protein kinase (MAPK) activation in TNF-α-stimulated HaCaT cells. AC and its constituent cyanidin 3-glucoside also attenuated TNF-α-induced IKK activation, IκB degradation, p65 phosphorylation/nuclear translocation, and p65 DNA binding activity in HaCaT cells. Overall, our results indicate that AC exerts anti-inflammatory activities by inhibiting expression of pro-inflammatory mediators in vitro and in vivo possibly through suppression of ROS-MAPK-NF-κB signaling pathways. Therefore, AC may be developed as a therapeutic agent to treat various inflammatory skin diseases.

Role of ocular VEMP test in assessing the occurrence of vertigo in otosclerosis patients.

PubMed

Lin, Kuei-You; Young, Yi-Ho

2015-01-01

This study adopted an inner ear test battery comprising audiometry, caloric test, ocular vestibular-evoked myogenic potential (oVEMP) test and cervical VEMP (cVEMP) test to find the factors related to the occurrence of vertigo in patients with otosclerosis. Fifty otosclerosis patients comprising 27 patients with vertigo (Group A) and 23 patients without vertigo/dizziness (Group B) were enrolled. Each patient underwent otoscopy, image study, audiometry, caloric test, and oVEMP and cVEMP tests via bone vibration stimuli. The sequence of inner ear deficits in Group A was in the order from oVEMP test (84%), cVEMP test (51%), caloric test (38%) and mean bone-conducted (BC) hearing threshold (14%), exhibiting a significantly declining trend (p<0.001). The rate of inner ear dysfunction in Group B also declined significantly but in a different order - cVEMP test (55%), oVEMP test (52%), mean BC hearing threshold (33%), and caloric test (18%). Comparison between the two groups revealed a significant difference in the oVEMP test results (p<0.01), but not in the results of the BC hearing threshold, caloric test or cVEMP test (p>0.05). Further, no significant differences existed between the BC hearing threshold and vestibular function test results. Otosclerosis patients with vertigo have more frequent abnormalities of oVEMPs to impulsive stimulation than do those without, consistent with more frequent abnormalities of the utricle. Abnormalities of oVEMPs and cVEMPs are more frequent than for caloric testing and BC hearing thresholds. The relative frequency of abnormalities may reflect the degree of pathological involvement of the utricle, saccule, semicircular canals and cochlea in otosclerosis patients with vertigo. Copyright © 2014 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.

Apert syndrome

MedlinePlus

... by ridging along sutures (craniosynostosis) Frequent ear infections Fusion or severe webbing of the 2nd, 3rd, and ... midface Skeletal (limb) abnormalities Short height Webbing or fusion of the toes Several other syndromes can lead ...

Question mark ears, temporo-mandibular joint malformation and hypotonia: auriculo-condylar syndrome or a distinct entity?

PubMed

Priolo, M; Lerone, M; Rosaia, L; Calcagno, E P; Sadeghi, A K; Ghezzi, F; Ravazzolo, R; Silengo, M

2000-10-01

We report a boy with prominent, peculiarly malformed ears, abnormality of the ramus of the mandible and hypotonia. An isolated peculiar bilateral ear deformity named 'question mark ear' has been delineated in plastic reconstruction surgery reviews [Cosman et al., 1970 Plast Reconstr Surg 46:454-457; Cosman (1984) Plast Reconstr Surg 73:572-576; Takato et al. (1989) Ann Plast Surg 22:69-73; Brodovsky (1997) Plast Reconstr Surg 100:1254-1257; Park (1998) Plast Reconstr Surg 101:1620-1623; Al-Quattan (1998) Plast Reconstr Surg 102:439-441] and a similar deformity of the ear and changes in the temporo-mandibular joint and condyle has been described by Jampol et al. [(1998) Am J Med Genet 75:449-452] and by Guion-Almeida et al. [(1999) Am J Med Genet 86:130-133]. The present case may be the third description of this malformation complex with additional clinical features characterized by hypotonia and mild developmental delay, or possibly a new distinct entity.

CT of the ear in Pendred syndrome.

PubMed

Goldfeld, Moshe; Glaser, Benjamin; Nassir, Elias; Gomori, John Moshe; Hazani, Elitsur; Bishara, Nassir

2005-05-01

To prospectively determine the structural anomalies of the inner ear by using thin-section computed tomography (CT) in an extended family with Pendred syndrome. Ethics committee approved the study, and informed consent was obtained from every patient or from parents of patients under legal age. Twelve patients (three females and nine males aged 7-47 years) with Pendred syndrome (all from the same ethnic isolate and with the same mutation in the PDS gene) were evaluated for inner-ear malformation at thin-section CT. Both ears were evaluated. Presence or absence of interscalar septum between upper and middle turns of the cochlea was evaluated, and vestibule and vestibular aqueduct were examined for enlargement. Modiolus was determined to be present or absent (modiolar deficiency). CT scans were evaluated in consensus by two radiologists (M.G., J.M.G.). All patients had inner ear malformation on both sides. Modiolus was absent and vestibule was enlarged on both sides in all 12 patients. Interscalar septum was absent in 18 (75%) of 24 ears. In eight patients, interscalar septum was absent in both ears, whereas in two patients, it was absent on only one side. Aqueduct was enlarged in 20 (80%) of 24 ears. In nine patients, both ears had enlarged aqueducts, while in two patients, only one side was abnormal. Inner ear malformation is an invariable finding in Pendred syndrome. Modiolus deficiency and vestibular enlargement were the most consistent anomalies in this population with Pendred syndrome. (c) RSNA, 2005.

Unknown syndrome: abnormal facies, hypothyroidism, postaxial polydactyly, and severe retardation: a third patient.

PubMed Central

Cavalcanti, D P

1989-01-01

Young and Simpson in 1987 and Fryns and Moerman in 1988 each reported a case of a new unknown syndrome with hypothyroidism, severe global retardation, and abnormal facies, including microcephaly, blepharophimosis, bulbous nose, thin upper lip, low set ears, and micrognathia. A male infant with a similar pattern of malformations and postaxial polydactyly is reported here. Images PMID:2614801

Genetics Home Reference: Alport syndrome

MedlinePlus

... particularly the organ of Corti, that transform sound waves into nerve impulses for the brain. Alterations in type IV collagen often result in abnormal inner ear function, which can lead to hearing loss. In the ...

Threshold changes of ABR results in toddlers and children.

PubMed

Louza, Julia; Polterauer, Daniel; Wittlinger, Natalie; Muzaini, Hanan Al; Scheckinger, Siiri; Hempel, Martin; Schuster, Maria

2016-06-01

Auditory brainstem response (ABR) is a clinically established method to identify the hearing threshold in young children and is regularly performed after hearing screening has failed. Some studies have shown that, after the first diagnosis of hearing impairment in ABR, further development takes place in a spectrum between progression of hearing loss and, surprisingly, hearing improvement. The aim of this study is to evaluate changes over time of auditory thresholds measured by ABR among young children. For this retrospective study, 459 auditory brainstem measurements were performed and analyzed between 2010 and 2014. Hearing loss was detected and assessed according to national guidelines. 104 right ears and 101 left ears of 116 children aged between 0 and 3 years with multiple ABR measurements were included. The auditory threshold was identified using click and/or NB-chirp-stimuli in natural sleep or in general anesthesia. The frequency of differences of at least more than 10dB between the measurements was identified. In 37 (35%) measurements of right ears and 38 (38%) of left ears there was an improvement of the auditory threshold of more than 10dB; in 27 of those measurements more than 20dB improvement was found. Deterioration was seen in 12% of the right ears and 10% of the left ears. Only half of the children had stable hearing thresholds in repeated measurements. The time between the measurements was on average 5 months (0 to 31 months). Hearing threshold changes are often seen in repeated ABR measurements. Therefore multiple measurements are necessary when ABR yields abnormal. Hearing threshold changes should be taken into account for hearing aid provision. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

Binaural integration: a challenge to overcome for children with hearing loss.

PubMed

Gordon, Karen A; Cushing, Sharon L; Easwar, Vijayalakshmi; Polonenko, Melissa J; Papsin, Blake C

2017-12-01

Access to bilateral hearing can be provided to children with hearing loss by fitting appropriate hearing devices to each affected ear. It is not clear, however, that bilateral input is properly integrated through hearing devices to promote binaural hearing. In the present review, we examine evidence indicating that abnormal binaural hearing continues to be a challenge for children with hearing loss despite early access to bilateral input. Behavioral responses and electrophysiological data in children, combined with data from developing animal models, reveal that deafness in early life disrupts binaural hearing and that present hearing devices are unable to reverse these changes and/or promote expected development. Possible limitations of hearing devices include mismatches in binaural place, level, and timing of stimulation. Such mismatches could be common in children with hearing loss. One potential solution is to modify present device fitting beyond providing audibility to each ear by implementing binaural fitting targets. Efforts to better integrate bilateral input could improve spatial hearing in children with hearing loss.

Effect of simulated bilateral cochlear distortion on speech discrimination in normal subjects.

PubMed

Hood, J D; Prasher, D K

1990-01-01

Bilateral sensorineural hearing loss may introduce grossly dissimilar cochlear distortion at the two ears, causing abnormal demands to be made upon the cortical analytical centres which normally receive congruent information. As a result, the prescription of binaural hearing aids may be a handicap rather than a help. In order to explore this possibility, 10 normal subjects were presented with simulated, dissimilar cochlear distortion at the two ears. Discrimination scores with binaural presentation were poorer than the best monaural score and there were clear indications that in the former, subjects selectively attended to one ear and neglected the other. In contrast, binaural presentation of the same simulated distortion resulted in a significant improvement, compared with the monaural discrimination score. Inability of the cortex to contend with discongruent speech input from the two ears may be a factor contributing to the rejection of binaural hearing aids in some individuals.

Cochlear pathology in chronic suppurative otitis media.

PubMed

Walby, A P; Barrera, A; Schuknecht, H F

1983-01-01

Chronic suppurative otitis media (COM) is reported to cause elevation of bone-conduction thresholds either by damage to cochlear sensorineural structures or by alteration in the mechanics of sound transmission in the ear. A retrospective study was made of the medical records of 87 patients with unilateral uncomplicated COM to document that abnormality in bone conduction does exist. In a separate study the cochlear pathology in 12 pairs of temporal bones with unilateral COM was studied by light microscopy. Infected ears showed higher than normal mean bone-conduction thresholds by amounts ranging from 1 dB at 500 Hz to 9.5 dB at 4,000 Hz. The temporal bones showed no greater loss of specialized sensorineural structures in infected ears than in normal control ears. Because there is no evidence that COM caused destruction of hair cells or cochlear neurons, alteration in the mechanics of sound transmission becomes a more plausible explanation for the hearing losses.

Methods for Teratogenic Screening of Air Force Chemicals

DTIC Science & Technology

1978-01-01

7 2 4 3 2.3 7 8 2 1 7 2.6 8 5 1 1 4 2.5 20 0 10 22 8 1 1 7 2.8 26 6 5 2 4 2.4 3 Gross Abnormalities Number of Fetuses with Abnormality Anophthalmia ...meningoencephalocoele), anophthalmia , cleft palate, cleft lip, misplaced ears, clubbed hind limbs, fused vertebrae, fused ribs, split centra and scoliosis.* In...experimentation with hydroxyurea in Fisher 344 rats, at 250 mg/kg on day nine of pregnancy, our results showed anophthalmia , brain abnormalities

Impedance and Otoscopy Screening of Multiply Handicapped Children in School.

ERIC Educational Resources Information Center

Bruns, Janet M.; And Others

1979-01-01

In order to examine the effectiveness of impedance and otoscopic screening in the determination of middle ear abnormalities, 79 physically handicapped, mentally retarded school children (mean age 8 years) were examined. (Author/PHR)

Congenital Auricular Malformations: Description of Anomalies and Syndromes.

PubMed

Bartel-Friedrich, Sylva

2015-12-01

Half of the malformations in the ear, nose, and throat region affect the ear. Malformations of the external ear (pinna or auricle with external auditory canal [EAC]) are collectively termed microtia. Microtia is a congenital anomaly that ranges in severity from mild structural abnormalities to complete absence of the external ear (anotia). Microtia occurs more frequently in males (∼2 or 3:1), is predominantly unilateral (∼70-90%), and more often involves the right ear (∼60%). The reported prevalence varies geographically from 0.83 to 17.4 per 10,000 births. Microtia may be genetic (with family history, spontaneous mutations) or acquired. Malformations of the external ear can also involve the middle ear and/or inner ear. Microtia may be an isolated birth defect, but associated anomalies or syndromes are described in 20 to 60% of cases, depending on study design. These generally fit within the oculo-auriculo-vertebral spectrum; defects are located most frequently in the facial skeleton, facial soft tissues, heart, and vertebral column, or comprise a syndrome (e.g., Treacher Collins syndrome). Diagnostic investigation of microtia includes clinical examination, audiologic testing, genetic analysis and, especially in higher grade malformations with EAC deformities, computed tomography (CT) or cone-beam CT for the planning of surgery and rehabilitation procedures, including implantation of hearing aids. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

The tympanic membrane displacement test and tinnitus: preliminary report on clinical observations, applications, and implications.

PubMed

Shulman, Abraham; Goldstein, Barbara; Marchbanks, Robert J

2012-01-01

The tympanic membrane displacement test (TMDT) is an attempt to record intracranial pressure (ICP) reflective of an intracranial pulse pressure amplitude wave (IPPA) transmitted to the inner ear and tympanic membrane with a probe placed into the external ear canal. Twelve tinnitus patients, divided into two groups, who were resistant to attempts to achieve tinnitus control or relief were selected for the TMDT. The group 1 TMDT recordings were obtained on one session test date, and group 2 (n = 6) recordings were obtained sequentially on different session test dates. Patient selection with the medical audiologic tinnitus patient protocol (MATPP) identified all to have a nonpulsatile, predominantly central-type severe disabling subjective idiopathic tinnitus (SIT) resistant to attempts for tinnitus relief with instrumentation or medication. Associated complaints in all selected SIT patients included persistent ear blockage in the SIT ear, normal middle-ear function, controlled secondary endolymphatic hydrops in the SIT ear, sensorineural hearing loss of high frequency, hyperacusis, occasional vertigo, and central nervous system complaints of headache, head pressure, and cognitive interference in memory and/or speech expression. Clinical concern is for the presence of an increased ICP reflecting an idiopathic intracranial hypertension (IIH) which, if not identified and treated, may be a factor influencing the clinical course of this particular cohort of SIT patients, highlighted by persistent ear blockage and associated complaints as described. We set out to accomplish a number of goals: (1) To identify abnormal intracranial pulse pressure (IPPA ICP) with the extracranial TMD in a preselected particular cohort of SIT patients clinically suspected (by use of the MATPP) to have an abnormal ICP (i.e., IIH); (2) to identify the abnormal IPPA ICP as a positive indicator for IIH and as a factor - not an etiology - influencing the clinical course of SIT in a preselected cohort of SIT patients; (3) to identify with the TMDT in SIT patients spontaneous nonevoked recordings of intra-aural pressure and test-retest reliability of the TMDT; (4) to identify with the TMDT levels of normal and abnormal IPPA ICP in real time in the clinical course of SIT (i.e., an objective diagnostic and treatment monitor function of the TMD targeting ICP and IIH before and after treatment); (5) to attempt to establish a correlation of treatment efficacy, targeting preand post-ICP as a manifestation of IIH, with SIT subjective tinnitus relief; (6) to identify the limitations and complications of the TMDT; and (7) to share with the reader the evolution of a new science of brain pulsatility and a technology having a clinical application for otology and neurotology complaints of hearing loss, tinnitus, ear blockage, and vertigo. The results reported in the literature complement and alter conventional medical teaching focusing on brain pulsation, absolute intracranial pressure, and brain disease. The Southampton Tympanic Membrane Displacement Analyzer was used to record spontaneous intra-aural pressure waves in 12 SIT patients. Patients selected for the TMDT were divided into two groups: Group 1 (n = 6) recordings were obtained on one session test date, and group 2 (n = 6) recordings were obtained sequentially on different session test dates. Multiple recordings were attempted in all patients to identify test-retest reliability in both groups. An attempt for treatment and control of an elevated ICP with or without reduced cerebral compliance (CC) was recommended in 4 patients. With single and multiple recordings using the TMDT, the IPPA (i.e., ICP) was demonstrated to be abnormal and to fluctuate in the clinical course of 10 of the 12 predominantly central-type tinnitus patients (SIT): abnormal IIPA with reduced CC in 8 of 12 patients and normal IPPA with reduced compliance in 2 of 12. Tinnitus treatment results targeting ICP as a manifestation of IIH with Diamox were positive in the short term in 2 patients and incomplete in 3. The SIT relief is reflective of fluctuation in the ICP and the overall issue of multifactorial brain pulsatility. (1) The TMDT demonstrated repeated and consistent spontaneous nonevoked recordings of displacement of the tympanic membrane, reflective of intra-aural pressure, abnormal IPPA ICP in a preselected particular cohort of SIT patients clinically suspected to have an abnormal ICP (i.e., IIH). (2) Test-retest reliability of the TMDT was positive. (3) The results of the TMDT application for identification of an elevated ICP and reduced CC were positive in 10 of 12 particular preselected patients with nonpulsatile, predominantly central-type SIT resistant to attempts for tinnitus relief with instrumentation or medication. These positive findings support clinical and basic science investigations previously reported in the literature. (4) The clinical significance of these preliminary results of an elevated ICP in a particular cohort of SIT patients supports the clinical impression of the presence of an IIH and its influence on the clinical course and overall treatment of SIT. (5) A final conclusion as to the clinical significance of an elevated ICP and reduced CC for IIH and the diagnosis and treatment of tinnitus remains to be established.

Diagnostic and surgical challenge: middle ear dermoid cyst in 12 month old with branchio-oto-renal syndrome and multiple middle-ear congenital anomalies.

PubMed

Johnston, D R; Whittemore, K; Poe, D; Robson, C D; Perez-Atayde, A R

2011-10-01

Described is the first case report, to our knowledge, of a middle-ear dermoid in a child with branchio-oto-renal (BOR) syndrome. Radiographic, pathologic, and intraoperative figures are shown. This was a diagnostic and surgical challenge as the presentation was similar to a congenital cholesteatoma and the child had numerous significant temporal bone abnormalities. After the intraoperative findings suggested a non-destructive process, the treatment strategy was altered. This case reiterates the need for a cautious, flexible operative approach in a syndromic child. Included is a relevant review of the literature and a detailed clinical analysis. Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.

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

PubMed

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

2012-08-01

Hearing impairment is common in individuals with branchio-oculo-facial (BOF) syndrome. The majority of described individuals have conductive hearing impairment due to malformed ossicles and/or external canal stenosis or atresia, although a sensorineural component to the hearing impairment in BOF syndrome is increasingly being reported. Sophisticated computed tomography (CT) of the temporal bone has revealed middle and inner ear malformations in three previous reports. We present middle and inner ear abnormalities in three additional individuals with mutation-proven BOF syndrome. We suggest that temporal bone CT imaging be included in the medical workup of a child with BOF syndrome, in order to guide management. Copyright © 2012 Wiley Periodicals, Inc.

Otoplasty: A graduated approach.

PubMed

Foda, H M

1999-01-01

Numerous otoplastic techniques have been described for the correction of protruding ears. Technique selection in otoplasty should be done only after careful analysis of the abnormal anatomy responsible for the protruding ear deformity. A graduated surgical approach is presented which is designed to address all contributing factors to the presenting auricular deformity. The approach starts with the more conservative cartilage-sparing suturing techniques, then proceeds to incorporate other more aggressive cartilage weakening maneuvers. Applying this approach resulted in better long-term results with less postoperative lateralization than that encountered on using the cartilage-sparing techniques alone.

Turner Syndrome

MedlinePlus

... skin. What health problems can occur with Turner syndrome? Girls and women with TS are at risk for congenital (present at birth) abnormalities of the heart and kidneys, high blood pressure, chronic or repeated middle ear infections, hearing loss, diabetes, underactive thyroid gland, bowel ...

Surgical correction of constricted ear combined with Stahl's ear.

PubMed

Bi, Ye; Lin, Lin; Yang, Qinhua; Pan, Bo; Zhao, Yanyong; He, Leren; Jiang, Haiyue

2015-07-01

Constricted ear combined with Stahl's ear is a rare ear deformity, which is a kind of complex congenital auricular deformity. From 1 January 2007 to 1 January 2014, 19 patients with constricted ear combined with Stahl's ear (Spock ear) were enrolled in this study, most of which were unilaterally deformed. To correct the deformity, a double Z-shaped skin incision was made on the posterior side of the auricle, with the entire layer of cartilage cut parallel to the helix traversing the third crus to form a fan-shaped cartilage flap. The superior crura of the antihelix were shaped by the folding cartilage rim. The cartilage of the abnormal third crus was made part of the new superior crura of antihelix, and the third crus was eliminated. The postoperative aesthetic assessment of the reshaped auricle was graded by both doctors and patients (or their parents). Out of the 19 patients, the number of satisfying cases of the symmetry, helix stretch, elimination of the third crus, the cranioauricular angle, and the substructure of the reshaped ears was 14 (nine excellent and five good), 16 (six excellent and 10 good), 17 (eight excellent and nine good), 15 (five excellent and 10 good), and 13 (two excellent and 11 good), respectively. With a maximum of a 90-month follow-up, no complication was observed. The results of the study suggested that this rare deformity could be corrected by appropriate surgical treatment, with a satisfied postoperative appearance. Copyright © 2015 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.

Multiethnic GWAS Reveals Polygenic Architecture of Earlobe Attachment.

PubMed

Shaffer, John R; Li, Jinxi; Lee, Myoung Keun; Roosenboom, Jasmien; Orlova, Ekaterina; Adhikari, Kaustabh; Gallo, Carla; Poletti, Giovanni; Schuler-Faccini, Lavinia; Bortolini, Maria-Cátira; Canizales-Quinteros, Samuel; Rothhammer, Francisco; Bedoya, Gabriel; González-José, Rolando; Pfeffer, Paige E; Wollenschlaeger, Christopher A; Hecht, Jacqueline T; Wehby, George L; Moreno, Lina M; Ding, Anan; Jin, Li; Yang, Yajun; Carlson, Jenna C; Leslie, Elizabeth J; Feingold, Eleanor; Marazita, Mary L; Hinds, David A; Cox, Timothy C; Wang, Sijia; Ruiz-Linares, Andrés; Weinberg, Seth M

2017-12-07

The genetic basis of earlobe attachment has been a matter of debate since the early 20 th century, such that geneticists argue both for and against polygenic inheritance. Recent genetic studies have identified a few loci associated with the trait, but large-scale analyses are still lacking. Here, we performed a genome-wide association study of lobe attachment in a multiethnic sample of 74,660 individuals from four cohorts (three with the trait scored by an expert rater and one with the trait self-reported). Meta-analysis of the three expert-rater-scored cohorts revealed six associated loci harboring numerous candidate genes, including EDAR, SP5, MRPS22, ADGRG6 (GPR126), KIAA1217, and PAX9. The large self-reported 23andMe cohort recapitulated each of these six loci. Moreover, meta-analysis across all four cohorts revealed a total of 49 significant (p < 5 × 10 -8 ) loci. Annotation and enrichment analyses of these 49 loci showed strong evidence of genes involved in ear development and syndromes with auricular phenotypes. RNA sequencing data from both human fetal ear and mouse second branchial arch tissue confirmed that genes located among associated loci showed evidence of expression. These results provide strong evidence for the polygenic nature of earlobe attachment and offer insights into the biological basis of normal and abnormal ear development. Copyright © 2017 The Authors. Published by Elsevier Inc. All rights reserved.

Human-induced contaminant impacts on migratory birds: lessons from the North American eared grebe (Podiceps nigricollis)

USGS Publications Warehouse

Sladky, Kurt K.; Quist, Charlotte; Ramirez, Pedro; Hill, David; Dein, F. Joshua

2003-01-01

The effects of aquatic contaminants generated by soda ash mining processes on the North American eared grebe (Podiceps nigricollis) population illustrates important issues associated with human-induced habitat degradation on the health of migratory species. Eared grebes have been extensively studied in their staging and breeding habitats, but little is known about their 2- to 3-day migratory periods. During migration, few bodies of water are available to the birds for refuge between freshwater breeding areas in Canada and hypersaline lakes (e.g., Great Salt Lake in Utah or Mono Lake in California) to which they migrate. One geographic refuge area includes a series of "tailings" ponds associated with soda ash mining operations in southwestern Wyoming. The ponds range from 100-1200 acres, with water containing high concentrations of sodium decahydrate (Na2CO3•10H2O). At cool temperatures (generally < 40°F) sodium decahydrate precipitates out of the water and crystallizes on solid objects in the ponds or on the water surface. Bird mortality on these ponds has been recognized since the early 1970's, and the mining companies have developed hazing strategies and rehabilitation programs in order to minimize mortality. In order to determine causes of grebe mortality and devise strategies to reduce mortality, a field epidemiologic investigation was developed with the following objectives: 1) to determine whether eared grebes have quantifiable physiologic abnormalities associated with exposure to soda ash mine pond water; 2) to evaluate physical effects of sodium decahydrate crystallization on grebe survival; 3) to establish cause of death based on necropsy of deceased grebes; 4) to determine long-term survivability of eared grebes after exposure to the pond water; and 5) to evaluate water quality and determine whether aquatic invertebrates are present in the ponds as a possible food source.

Correlation between caloric and ocular vestibular evoked myogenic potential test results.

PubMed

Huang, Chi-Hsuan; Wang, Shou-Jen; Young, Yi-Ho

2012-02-01

The ocular vestibular evoked myogenic potential (o-VEMP) test results correlate significantly with caloric test results for patients with acoustic neuroma (AN), but not for patients with Meniere's disease (MD), indicating that the o-VEMP test may replace the caloric test for evaluating the vestibular nerve from which the AN arises. Conversely, the caloric, o-VEMP, and cervical VEMP (c-VEMP) tests should be performed to map lesion sites in the vestibular labyrinth. This study performed caloric, o-VEMP, and c-VEMP tests on patients with central and peripheral vestibular disorders to investigate their relationships. In all, 66 patients comprising 16 with unilateral AN and 50 with unilateral definite MD were enrolled. All patients underwent caloric, o-VEMP, and c-VEMP tests. In the AN group, the caloric test identified canal paresis and caloric areflexia in 10 ears, while the o-VEMP and c-VEMP tests identified abnormal (absent or delayed) responses in 12 and 11 ears, respectively. A significant correlation existed between caloric and o-VEMP test results, but not between caloric and c-VEMP test results, or between o-VEMP and c-VEMP test results. For the MD group, abnormal caloric, o-VEMP, and c-VEMP test results were obtained for 24%, 44%, and 38% of hydropic ears, respectively. No correlation existed between any two test results.

Perilymphatic fistula in cabin attendants: an incapacitating consequence of flying with common cold.

PubMed

Klokker, Mads; Vesterhauge, Søren

2005-01-01

A perilymphatic fistula (PLF) is an abnormal communication between the inner ear and the middle ear that leaks perilymph. PLF is considered rare, but is known to occur during childbirth, straining, weightlifting, head trauma, and diving with middle ear equalizing problems. It has also, anecdotally, been described in connection with flying. The symptoms are uncharacteristic vertigo and, in some cases, hearing impairment and tinnitus. This study describes four cases of PLF during a period of 6 mo in a major Scandinavian airline company employing approximately 3000 cabin attendants (CAs). None of the cases were diagnosed at the primary health care level. All were referred to the Aviation Medical Center for investigation. The PLF diagnosis was based on the case history, Platform Pressure Test (a fistula test), and other vestibular tests. Only one CA has been able to return to flying duties. The article emphasizes the risk of flying with poor middle ear equalization and the necessity of reminding crews and airline companies to "never fly with a common cold".

Detailed assessment of the ear in Cornelia de Lange syndrome: comparison with a control sample using the new dysmorphology guidelines.

PubMed

Hunter, Alasdair G W; Collins, Julianne S; Deardorff, Matthew A; Krantz, Ian D

2009-10-01

The literature abounds with reports of malformation syndromes in which human external ears are variously described as dysplastic, abnormal, large/small, low set, typical, or in some way unusual. Rarely is the ear well illustrated or described in meaningful detail. With few exceptions, such as Down syndrome, there is no real understanding of the degree to which ear morphology is affected in a specific syndrome. This paper describes a retrospective attempt to apply the recently published Morphological Definitions of the ear to compare a control sample of convenience with a group of patients with Cornelia de Lange syndrome (CdLS) (all six papers in this issue are available online, open access at http://www3.interscience.wiley.com/journal/121641055/issue). Although this study has a number of limitations, it demonstrates that the method can be successfully applied and is capable of producing data that can be subjected to statistical analysis. The ears of the patients with CdLS were significantly different from the controls over a number of descriptors, the most significant of which included more frequent apparent posterior rotation, a shorter more serpiginous antihelical stem and sharper antihelical to inferior crus angle, a shorter crus helix, a more V-shaped incisura, and a smaller lobe.

Otitis Media and Its Sequelae in Kenyan Schoolchildren.

PubMed

Simões, Eric A F; Kiio, Francis; Carosone-Link, Phyllis J; Ndegwa, Serah N; Ayugi, John; Macharia, Isaac M

2016-12-01

The goal of this study was to obtain representative Kenyan data on the point prevalence of acute otitis media (AOM) and its sequelae (otitis media with effusion [OME] and chronic suppurative otitis media [CSOM]), a major cause of preventable hearing loss in children in developing countries. In Africa, there are limited studies on the prevalence of AOM and its sequelae in children. Study subjects were children aged 2 to 15 years and were enrolled from randomly selected preprimary and primary schools. After parental or guardian consent, subjects had a questionnaire administered, otoscopy and tympanometry were done, and audiometry was performed on those with ear problems detected on these examinations. A total of 9825 (75%) children was from rural schools. The prevalence of CSOM was 15 of 1000, OME was 15 of 1000, and AOM was 7 of 1000 children. Rural Rift Valley schoolchildren had the highest prevalence of CSOM (24 of 1000) compared with other regions (12 of 1000; P < .0001). Ear discharge occurred before 3.5 years in 50% of 901 children with ear discharge. A history of ear discharge was associated with abnormal tympanograms (odds ratio [OR], 11.9-19.2) and mild-to-severe hearing loss (OR, 21.6-38.6), even in children without ear disease (OR, 10.7-24.4). The burden of AOM sequelae in Kenyan preschool and schoolchildren is significant, and it occurs mostly in the first 4 years of life. By preventing early recurrent AOM, pneumococcal vaccination might partly avert nonreversible sequelae. © The Author 2015. Published by Oxford University Press on behalf of the Pediatric Infectious Diseases Society. All rights reserved. For Permissions, please e-mail: journals.permissions@oup.com.

Genetic background has a major effect on the penetrance and severity of craniofacial defects in mice heterozygous for the gene encoding the nucleolar protein Treacle.

PubMed

Dixon, Jill; Dixon, Michael James

2004-04-01

Treacher Collins syndrome (TCS) is a craniofacial disorder that results from mutations in TCOF1, which encodes the nucleolar protein Treacle. The severity of the clinical features exhibits wide variation and includes hypoplasia of the mandible and maxilla, abnormalities of the external ears and middle ear ossicles, and cleft palate. To determine the in vivo function of Treacle, we previously generated Tcof1 heterozygous mice on a mixed C57BL/6 and 129 background. These mice exhibited a lethal phenotype, which included abnormal development of the maxilla, absence of the eyes and nasal passages, and neural tube defects. Here, we show that placing the mutation onto different genetic backgrounds has a major effect on the penetrance and severity of the craniofacial and other defects. The offspring exhibit markedly variable strain-dependent phenotypes that range from extremely severe and lethal in a mixed CBA/Ca and 129 background, to apparently normal and viable in a mixed BALB/c and 129 background. In the former case, in addition to a profoundly severe craniofacial phenotype, CBA-derived heterozygous mice also exhibited delayed ossification of the long bones, rib fusions, and digit anomalies. The results of our studies indicate that factors in the different genetic backgrounds contribute extensively to the Tcof1 phenotype. Copyright 2004 Wiley-Liss, Inc.

Molecular and phenotypic aspects of CHD7 mutation in CHARGE syndrome

PubMed Central

Zentner, Gabriel E.; Layman, Wanda S.; Martin, Donna M.; Scacheri, Peter C.

2010-01-01

CHARGE syndrome (Coloboma of the eye, Heart defects, Atresia of the choanae, Retardation of growth and/or development, Genital and/or urinary abnormalities, and Ear abnormalities (including deafness) is a genetic disorder characterized by a specific and a recognizable pattern of anomalies. De novo mutations in the gene encoding chromodomain helicase DNA binding protein 7 (CHD7) are the major cause of CHARGE syndrome. Here, we review the clinical features of 379 CHARGE patients who tested positive or negative for mutations in CHD7. We found that CHARGE individuals with CHD7 mutations more commonly have ocular colobomas, temporal bone anomalies (semicircular canal hypoplasia/dysplasia), and facial nerve paralysis compared with mutation negative individuals. We also highlight recent genetic and genomic studies that have provided functional insights into CHD7 and the pathogenesis of CHARGE syndrome. PMID:20186815

Growth hormone treatment does not affect incidences of middle ear disease or hearing loss in infants and toddlers with Turner syndrome.

PubMed

Davenport, Marsha L; Roush, Jackson; Liu, Chunhua; Zagar, Anthony J; Eugster, Erica; Travers, Sharon; Fechner, Patricia Y; Quigley, Charmian A

2010-01-01

No randomized, controlled, prospective study has evaluated the effect of growth hormone (GH) on the rates of middle ear (ME) disease and hearing loss in girls with Turner syndrome (TS). A 2-year, prospective, randomized, controlled, open-label, multicenter, clinical trial ('Toddler Turner Study'; August 1999 to August 2003) was carried out. The study was conducted at 11 US pediatric endocrine centers. Eighty-eight girls with TS, aged 9 months to 4 years, were enrolled. The interventions comprised recombinant GH (50 microg/kg/day, n = 45) or no treatment (n = 43) for 2 years. The outcome measures included occurrence rates of ear-related problems, otitis media (OM) and associated antibiotic treatments, tympanometric assessment of ME function and hearing assessment by audiology. At baseline, 57% of the girls (mean age = 1.98 +/- 1.00 years) had a history of recurrent OM, 33% had undergone tympanostomy tube (t-tube) insertion and 27% had abnormal hearing. There was no significant difference between the treatment groups for annual incidence of OM episodes (untreated control: 1.9 +/- 1.4; GH-treated: 1.5 +/- 1.6, p = 0.17). A quarter of the subjects underwent ear surgeries (mainly t-tube insertions) during the study. Recurrent or persistent abnormality of ME function on tympanometry was present in 28-45% of the girls without t-tubes at the 6 postbaseline visits. Hearing deficits were found in 19-32% of the girls at the annual postbaseline visits. Most of these were conductive deficits, however, 2 girls had findings consistent with sensorineural hearing loss, which was evident before 3 years of age. Ear and hearing problems are common in infants and toddlers with TS and are not significantly influenced by GH treatment. Girls with TS need early, regular and thorough ME monitoring by their primary care provider and/or otolaryngologist, and at least annual hearing evaluations by a pediatric audiologist. Copyright 2010 S. Karger AG, Basel.

Otosclerosis surgery: approaches, profits and complications.

PubMed

Japaridze, Sh; Lomidze, L; Jashi, M; Kekelidze, I; Gegenava, Kh

2009-05-01

A systematic analysis of stapedoplasty output in otosclerosis cases was carried out. The operations were done during the period of 2005-2008 years at the Department of Otorhinolaryngology of the Tbilisi State Medical University. From the overall number of 107 patients, 78 were females and 29 males, 72.9% and 27.1%, respectively. The ages ranged from 16 to 57 years. The mean age was 35 years. The conductive and mixed forms of hearing losses were diagnosed in 70 and 37 cases, 65.4% and 34.6%, respectively. Right ear was operated in 46 patients, left ear in 57, and both ears in four, 43.3%, 53.0%, and 3.7%, respectively. 111 ears have been cured thus in sum. Intumescences of external ear tube were observed during the operation in 22 patients, 20.6%. In 7, 6.5%, the facial nerve was located downward. Particularly abnormal placement of the facial nerve was the case in two patients, 1.9%. The endaural approach has been proved to own advantages over the transmeatal one. The data generally confirmed that stapedotomy, as compared to stapedectomy, is a better choice for the surgery output. In beneficial cases the air/bone gaps after the operation closed totally or nearly totally. Such a proper outcome was reached in 93 out of 111 ears operated, 83.8%. In most of remainder ears the gaps after the operation narrowed significantly but far not completely. The definite surgery failure happened in one case only, 0.9%. To balance the preserved middle-ear problems in non-perfect surgery cases, the hearing aids of bone-conduction types have been recommended. In mixed otosclerosis cases, conversely, the aids of air-conduction types were advised to overcome the coexisted inner-ear pathologies.

22q11.2 microduplication syndrome with congenital aural atresia: a family report.

PubMed

Boudewyns, An; van den Ende, Jenneke; Boiy, Tine; Van de Heyning, Paul; Declau, Frank

2012-06-01

22q11.2 microduplication syndrome is characterized by a large phenotypic variability including facial dysmorphism, developmental delay, and hearing loss. We describe a family in whom 5 of 11 children were affected by a unilateral or bilateral congenital aural atresia. Four of these 5 carried a 22q11.2 microduplication and had typical dysmorphic features. Computed tomography with 3-D reconstructions allowed for a detailed examination of the middle ear structures and classification of the atresia type. Audiometry revealed a moderately severe conductive hearing loss in accordance with the clinical and computed tomography findings. Detailed examination of the ear is warranted in patients with a 22q11.2 microduplication. When outer ear abnormalities are encountered, an additional workup including audiometry and computed tomography with 3-D reconstructions is required.

Phenotypic variability in Patau syndrome.

PubMed

Caba, Lavinia; Rusu, Cristina; Butnariu, Lacramioara; Panzaru, Monica; Braha, Elena; Volosciuc, M; Popescu, Roxana; Gramescu, Mihaela; Bujoran, C; Martiniuc, Violeta; Covic, M; Gorduza, E V

2013-01-01

Patau syndrome has an incidence of 1/10.000-20.000, the clinical diagnosis being suggested by the triad cleft lip and palate, microphthalmia/anophthalmia and postaxial polydactyly. Most frequent cytogenetic abnormality is free and homogeneous trisomy 13 (80.0%), rarely being detected trisomy mosaics or Robertsonian translocations. The objective of the study was to identify phenotypic features of trisomy 13. The retrospective study was conducted on a trial group of 14 cases diagnosed cytogenetically with trisomy 13 between January 2000 and December 2012 at lasi Medical Genetics Centre. Of the 14 cases, 3 were evaluated pathologically (two aborted foetuses and one stillborn), 8 cases were detected in the neonatal period, and 3 in infancy. Clinical diagnosis was supported by the identification of a model of abnormal development, mainly characterized by: maxillary cleft (lip and palate--5 cases; lip--1 case), ocular abnormalities (microphthalmia/anophthalmia--7 cases; cyclopia--1 case), postaxial polydactyly (7 cases), scalp defects (6 cases), congenital heart anomalies (10 cases, 6 patients with atrial septal defect), complete holoprosencephaly (4 cases), ear abnormalities (11 cases), broad nasal root (10 cases). An important issue in confirming the phenotypic variability of Patau syndrome is that the classic clinical triad was identified only in one case. Patau syndrome is a disease with variable expression and is characterized by a pattern of abnormal prenatal development characterized by facial dysmorphia, polydactyly and severe birth defects (heart, brain) that generate an increased in utero and perinatal mortality.

Congenital oval or round window anomaly with or without abnormal facial nerve course: surgical results for 15 ears.

PubMed

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.

Gene Therapy Restores Balance and Auditory Functions in a Mouse Model of Usher Syndrome.

PubMed

Isgrig, Kevin; Shteamer, Jack W; Belyantseva, Inna A; Drummond, Meghan C; Fitzgerald, Tracy S; Vijayakumar, Sarath; Jones, Sherri M; Griffith, Andrew J; Friedman, Thomas B; Cunningham, Lisa L; Chien, Wade W

2017-03-01

Dizziness and hearing loss are among the most common disabilities. Many forms of hereditary balance and hearing disorders are caused by abnormal development of stereocilia, mechanosensory organelles on the apical surface of hair cells in the inner ear. The deaf whirler mouse, a model of human Usher syndrome (manifested by hearing loss, dizziness, and blindness), has a recessive mutation in the whirlin gene, which renders hair cell stereocilia short and dysfunctional. In this study, wild-type whirlin cDNA was delivered to the inner ears of neonatal whirler mice using adeno-associated virus serotype 2/8 (AAV8-whirlin) by injection into the posterior semicircular canal. Unilateral whirlin gene therapy injection was able to restore balance function as well as improve hearing in whirler mice for at least 4 months. Our data indicate that gene therapy is likely to become a treatment option for hereditary disorders of balance and hearing. Copyright © 2017. Published by Elsevier Inc.

[Melorheostosis associated with arteriovenous malformation of the ear].

PubMed

Ingen-Housz-Oro, S; Chigot, V; Hamel-Teillac, D; Brunelle, F; De Prost, Y

2001-09-01

Melorheostosis is a rare bone dystrophy that may be associated with various vascular malformations. We report a case of arteriovenous fistulae of the ear associated with melorheostosis limited to the same side of the body. A 13 year-old boy presented a congenital port-wine nevus of the right side of the head complicated by an arteriovenous fistulae and angiomatous nodules of the ear. He was treated by laser, surgery of the nodules, arterial embolisations and sclerotherapy. In 1999, he had a benign trauma of the right hand. The X-ray showed hyperostosis resembling wax flowing down a candle reaching the carpus and some of the metacarpals and the phalanges of the right hand, typical of melorheostosis. The complete radiographic check-up showed the same characteristic appearance on the right side of the skull and the long bones of the right upper limb. Except a deformation of the right fingers, there were no others symptoms. Melorheostosis is a rare, sporadic and benign bone dysplasia that may be localized to a single limb or disseminated. The diagnosis is usually made in late childhood. Pain, stiffness, deformation of a limb are the main clinical manifestations. The skin may be erythematous and sclerotic. The radiographic appearance is characteristic with hyperostosis on one side of the bone resembling wax flowing down a candle. A vascular abnormality is present in 17 p. 100 of cases (hemangiomas, aneurysms, renal artery stenosis.). In these cases, melorheostosis is usually limited to the same side of the vascular lesion. We report the first case of arteriovenous fistulae of the ear associated with melorheostosis, on the same side of the body. The physiopathology of melorheostosis is still unknown but the association with a homolateral vascular abnormality suggests a localized defect in embryogenesis of the vascular and skeletal systems.

Facial Paralysis in Patients With Hemifacial Microsomia: Frequency, Distribution, and Association With Other OMENS Abnormalities.

PubMed

Li, Qiang; Zhou, Xu; Wang, Yue; Qian, Jin; Zhang, Qingguo

2018-05-15

Although facial paralysis is a fundamental feature of hemifacial microsomia, the frequency and distribution of nerve abnormalities in patients with hemifacial microsomia remain unclear. In this study, the authors classified 1125 cases with microtia (including 339 patients with hemifacial microsomia and 786 with isolated microtia) according to Orbital Distortion Mandibular Hypoplasia Ear Anomaly Nerve Involvement Soft Tissue Dependency (OMENS) scheme. Then, the authors performed an independent analysis to describe the distribution feature of nerve abnormalities and reveal the possible relationships between facial paralysis and the other 4 fundamental features in the OMENS system. Results revealed that facial paralysis is present 23.9% of patients with hemifacial microsomia. The frontal-temporal branch is the most vulnerable branch in the total 1125 cases with microtia. The occurrence of facial paralysis is positively correlated with mandibular hypoplasia and soft tissue deficiency both in the total 1125 cases and the hemifacial microsomia patients. Orbital asymmetry is related to facial paralysis only in the total microtia cases, and ear deformity is related to facial paralysis only in hemifacial microsomia patients. No significant association was found between the severity of facial paralysis and any of the other 4 OMENS anomalies. These data suggest that the occurrence of facial paralysis may be associated with other OMENS abnormalities. The presence of serious mandibular hypoplasia or soft tissue deficiency should alert the clinician to a high possibility but not a high severity of facial paralysis.

[Audiological characteristics of young children with otitis media with effusion].

PubMed

Wang, Qiu-ju; Shi, Wei; Lan, Lan; Wang, Da-yong; Zhang, Ya-mei

2008-12-01

To characterize the audiological features in the infants with otitis media with effusion (OME) and to investigate the utility of variety of objective audiometry methods in diagnosis and intervention on OME. Fifty six infants (40 males and 16 females) were investigated, who were referred to our clinic at the General Hospital of Chinese People's Liberation Army by the other hospitals from December 2004 to June 2007 when the infants were diagnosed or highly suspected of OME. The ages at the initial diagnosis ranged from 42 days to three years, with an average of five months. The infants, after receiving the conventional otolaryngological exams, were subjected to the tests of auditory brainstem response (ABR), otoacoustic emission (OAE), tympanometry (226 Hz and 1000 Hz) and behaviors audiometry. Among 56 affected infants, 87 ears were diagnosed with OME, of which 31 infants were affected bilateral and 25 with monaural. For the 49 infants who received hearing screening at birth, 36 infants were referred at the initial screening. For the 52 infants who received repeated screening, all subjects were referred. Six infants without receiving hearing screening came to clinic when their parents observed their kids' hearing impairment. Among the 52 cases (104 ears) who received tympanometry test, 20 subjects (28 ears) showed B or C type tympanometry curve. Thirty-nine cases (78 ears) were given tympanometry test at 1000 Hz, of which 38 cases (55 ears) showed abnormal hearing. Among 56 infants (112 ears) with ABR test, 49 subjects (74 ears) exhibited prolonged ABR type I curve. All 56 infants (112 ears) received OAE test, of which 55 subjects (81 ears) were referred. Four infants (8 ears) accepted the behavior test and all of them showed A-B Gap. The combined tympanometry test at both 226 Hz and 1000 Hz, ABR latency or threshold test, infant's behavior test and OAE, used jointly, enable characterizing better OME in infants, thus helping early diagnosis of this hearing disorder.

Diagnosis and medical treatment of otitis externa in the dog and cat.

PubMed

Jacobson, L S

2002-12-01

Otitis externa is no longer viewed as an isolated disease of the ear canal, but is a syndrome that is often a reflection of underlying dermatological disease. Causes are classified as predisposing (increase the risk of otitis); primary (directly induce otitis), secondary (contribute to otitis only in an abnormal ear or in conjunction with predisposing factors) and perpetuating (result from inflammation and pathology in ear, prevent resolution of otitis). Common primary causes include foreign bodies, hypersensitivity (particularly atopy and food allergy), keratinisation disorders (most commonly primary idiopathic seborrhoea and hypothyroidism) and earmites, particularly in cats. A systematic diagnostic procedure is required to identify causes and contributing factors. This should include history, clinical examination, otoscopy and cytology in all cases and culture and sensitivity as well as otitis media assessment and biopsy in severe and recurrent cases. Ancillary tests may be required depending on the underlying cause. Treatment consists of identifying and addressing predisposing and primary factors; cleaning the ear canal; topical therapy; systemic therapy where necessary; client education; follow-up; and preventive and maintenance therapy as required.

Whole-mount Confocal Microscopy for Adult Ear Skin: A Model System to Study Neuro-vascular Branching Morphogenesis and Immune Cell Distribution.

PubMed

Yamazaki, Tomoko; Li, Wenling; Mukouyama, Yoh-Suke

2018-03-29

Here, we present a protocol of a whole-mount adult ear skin imaging technique to study comprehensive three-dimensional neuro-vascular branching morphogenesis and patterning, as well as immune cell distribution at a cellular level. The analysis of peripheral nerve and blood vessel anatomical structures in adult tissues provides some insights into the understanding of functional neuro-vascular wiring and neuro-vascular degeneration in pathological conditions such as wound healing. As a highly informative model system, we have focused our studies on adult ear skin, which is readily accessible for dissection. Our simple and reproducible protocol provides an accurate depiction of the cellular components in the entire skin, such as peripheral nerves (sensory axons, sympathetic axons, and Schwann cells), blood vessels (endothelial cells and vascular smooth muscle cells), and inflammatory cells. We believe this protocol will pave the way to investigate morphological abnormalities in peripheral nerves and blood vessels as well as the inflammation in the adult ear skin under different pathological conditions.

The first case of Niikawa-Kuroki syndrome in Kazakhstan associated with café au lait spots.

PubMed

Al Mosawi, A J; Fewin, L

2009-10-01

Niikawa-Kuroki syndrome (Kabuki syndrome) is a multiple congenital anomaly syndrome of unknown etiology with a very wide spectrum of abnormalities and severity. The aim of this paper was to report the first case of the syndrome in Kazakhstan associated café au lait. Five year and half old boy from Kazakhstan (Uzbek-of Turk ethnicity) presented with dysmorphic facial features (long palpebral fissures, a broad and depressed nasal tip, large prominent earlobes, small head, epicanthic folds short stature, delayed language development, hypotonia, bilateral developmental dysplasia of the hip (DDH), large ears and triangular chin, café au lait spots. The clinical diagnosis was based on the triad of characteristic facial abnormalities (long palpebral fissures, a broad and depressed nasal tip, large prominent earlobes, small head), growth retardation, (DDH). In this paper the authors report the first case of Kabuki syndrome associated with café au lait spots.

Determinants of Change in Air-Bone Gap and Bone Conduction in Patients Operated on for Chronic Otitis Media.

PubMed

Wiatr, Maciej; Wiatr, Agnieszka; Składzień, Jacek; Stręk, Paweł

2015-08-11

Middle ear surgery aims to eliminate pathology from the middle ear, improve drainage and ventilation of the postoperative cavity, and reconstruct the tympanic membrane and ossicles. The aim of this work is to define the factors that affect ABG (air-bone gap) and bone conduction in the patients operated on due to chronic otitis media. A prospective analysis of patients operated on due to diseases of the middle ear during 2009-2012 was carried out. The cases of patients operated on for the first time due to chronic otitis media were analyzed. The analysis encompassed patients who had undergone middle ear surgery. The patients were divided into several groups taking into account the abnormalities of the middle ear mucous and damage of the ossicular chain observed during otosurgery. A significant hearing improvement was observed in patients with type 2 tympanoplasty in the course of chronic cholesteatoma otitis media and in patients with simple chronic inflammatory process in whom a PORP was used in the reconstruction. Granulation tissue was an unfavorable factor of hearing improvement following tympanoplasty. A significant improvement of bone conduction was observed in the patients with dry perforation without other lesions in the middle ear. The elimination of granulation lesions was a positive factor for the future improvement of the function of the inner ear. The presence of granuloma-related lesions in the middle ear spaces is likely to impede hearing improvement. Damage to the ossicular chain rules out the possibility of bone conduction improvement after surgery. The prognosis on tube-related simple chronic otitis media after myringoplasty, with the preserved continuity of the ossicular chain, consists of closing the ABG and leads to significant improvement of bone conduction.

Computed tomographic features of the osseous structures of the external acoustic meatus, tympanic cavity, and tympanic bulla of llamas (Lama glama).

PubMed

Concha-Albornoz, Ismael; Stieger-Vanegas, Susanne M; Cebra, Christopher K

2012-01-01

To evaluate the osseous structures of the external acoustic meatus, tympanic cavity, and tympanic bulla of llamas (Lama glama) by use of computed tomography (CT) and establish measurement values for use in detection of abnormalities associated with the external or middle ear in llamas. 10 adult llama heads without any evidence of ear disease. Heads of 10 healthy llamas euthanized by use of a captive bolt striking the dorsal aspect of the skull were collected. Transverse images of the heads were acquired with 1-mm slice thickness, and images were reconstructed in sagittal and dorsal planes. Measurements of the bony structures of the external and middle ear of each head were obtained. The osseous external acoustic meatus curved ventrally as it tracked medially. Its narrowest portion was located at the level of the tympanic annulus. The tympanic bulla conformation differed widely from the bubble-shaped tympanic bulla in dogs and cats. The bulla was divided by the stylohyoid fossa into a larger caudolateral and a smaller caudomedial process; its interior had a honeycombed structure with pneumatized cells similar to the honeycombed appearance of the human mastoid process. Results provided new information regarding the shape and dimensions of the osseous external and middle ear structures in adult llamas without ear disease. Specific landmarks for location of the external acoustic meatus, tympanic cavity, and tympanic bulla in relation to each other were identified. Knowledge of the CT appearance of normal structures will help clinicians to identify changes attributable to middle ear otitis, external ear canal stenosis, or congenital malformations of the ear in this species.

Mature middle and inner ears express Chd7 and exhibit distinctive pathologies in a mouse model of CHARGE syndrome.

PubMed

Hurd, Elizabeth A; Adams, Meredith E; Layman, Wanda S; Swiderski, Donald L; Beyer, Lisa A; Halsey, Karin E; Benson, Jennifer M; Gong, Tzy-Wen; Dolan, David F; Raphael, Yehoash; Martin, Donna M

2011-12-01

Heterozygous mutations in the gene encoding chromodomain-DNA-binding-protein 7 (CHD7) cause CHARGE syndrome, a multiple anomaly condition which includes vestibular dysfunction and hearing loss. Mice with heterozygous Chd7 mutations exhibit semicircular canal dysgenesis and abnormal inner ear neurogenesis, and are an excellent model of CHARGE syndrome. Here we characterized Chd7 expression in mature middle and inner ears, analyzed morphological features of mutant ears and tested whether Chd7 mutant mice have altered responses to noise exposure and correlated those responses to inner and middle ear structure. We found that Chd7 is highly expressed in mature inner and outer hair cells, spiral ganglion neurons, vestibular sensory epithelia and middle ear ossicles. There were no obvious defects in individual hair cell morphology by prestin immunostaining or scanning electron microscopy, and cochlear innervation appeared normal in Chd7(Gt)(/+) mice. Hearing thresholds by auditory brainstem response (ABR) testing were elevated at 4 and 16 kHz in Chd7(Gt)(/+) mice, and there were reduced distortion product otoacoustic emissions (DPOAE). Exposure of Chd7(Gt)(/+) mice to broadband noise resulted in variable degrees of hair cell loss which inversely correlated with severity of stapedial defects. The degrees of hair cell loss and threshold shifts after noise exposure were more severe in wild type mice than in mutants. Together, these data indicate that Chd7(Gt)(/+) mice have combined conductive and sensorineural hearing loss, correlating with changes in both middle and inner ears. Copyright © 2011 Elsevier B.V. All rights reserved.

Mature middle and inner ears express Chd7 and exhibit distinctive pathologies in a mouse model of CHARGE syndrome

PubMed Central

Hurd, Elizabeth A.; Adams, Meredith E.; Layman, Wanda S.; Swiderski, Donald L.; Beyer, Lisa A.; Halsey, Karin E.; Benson, Jennifer M.; Gong, Tzy-Wen; Dolan, David F.; Raphael, Yehoash; Martin, Donna M.

2011-01-01

Heterozygous mutations in the gene encoding chromodomain-DNA-binding-protein 7 (CHD7) cause CHARGE syndrome, a multiple anomaly condition which includes vestibular dysfunction and hearing loss. Mice with heterozygous Chd7 mutations exhibit semicircular canal dysgenesis and abnormal inner ear neurogenesis, and are an excellent model of CHARGE syndrome. Here we characterized Chd7 expression in mature middle and inner ears, analyzed morphological features of mutant ears and tested whether Chd7 mutant mice have altered responses to noise exposure and correlated those responses to inner and middle ear structure. We found that Chd7 is highly expressed in mature inner and outer hair cells, spiral ganglion neurons, vestibular sensory epithelia and middle ear ossicles. There were no obvious defects in individual hair cell morphology by Prestin immunostaining or scanning electron microscopy, and cochlear innervation appeared normal in Chd7Gt/+ mice. Hearing thresholds by auditory brainstem response (ABR) testing were elevated at 4 and 16 kHz in Chd7Gt/+ mice, and there were reduced distortion product otoacoustic emissions (DPOAE). Exposure of Chd7Gt/+ mice to broadband noise resulted in variable degrees of hair cell loss which inversely correlated with severity of stapedial defects. The degrees of hair cell loss and threshold shifts after noise exposure were more severe in wild type mice than in mutants. Together, these data indicate that Chd7Gt/+ mice have combined conductive and sensorineural hearing loss, correlating with changes in both middle and inner ears. PMID:21875659

Effects of Hatchery Rearing on the Structure and Function of Salmonid Mechanosensory Systems.

PubMed

Brown, Andrew D; Sisneros, Joseph A; Jurasin, Tyler; Coffin, Allison B

2016-01-01

This paper reviews recent studies on the effects of hatchery rearing on the auditory and lateral line systems of salmonid fishes. Major conclusions are that (1) hatchery-reared juveniles exhibit abnormal lateral line morphology (relative to wild-origin conspecifics), suggesting that the hatchery environment affects lateral line structure, perhaps due to differences in the hydrodynamic conditions of hatcheries versus natural rearing environments, and (2) hatchery-reared salmonids have a high proportion of abnormal otoliths, a condition associated with reduced auditory sensitivity and suggestive of inner ear dysfunction.

In Vivo Analysis of Lrig Genes Reveals Redundant and Independent Functions in the Inner Ear

PubMed Central

del Rio, Tony; Nishitani, Allison M.; Yu, Wei-Ming; Goodrich, Lisa V.

2013-01-01

Lrig proteins are conserved transmembrane proteins that modulate a variety of signaling pathways from worm to humans. In mammals, there are three family members – Lrig1, Lrig2, and Lrig3 – that are defined by closely related extracellular domains with a similar arrangement of leucine rich repeats and immunoglobulin domains. However, the intracellular domains show little homology. Lrig1 inhibits EGF signaling through internalization and degradation of ErbB receptors. Although Lrig3 can also bind ErbB receptors in vitro, it is unclear whether Lrig2 and Lrig3 exhibit similar functions to Lrig1. To gain insights into Lrig gene functions in vivo, we compared the expression and function of the Lrigs in the inner ear, which offers a sensitive system for detecting effects on morphogenesis and function. We find that all three family members are expressed in the inner ear throughout development, with Lrig1 and Lrig3 restricted to subsets of cells and Lrig2 expressed more broadly. Lrig1 and Lrig3 overlap prominently in the developing vestibular apparatus and simultaneous removal of both genes disrupts inner ear morphogenesis. This suggests that these two family members act redundantly in the otic epithelium. In contrast, although Lrig1 and Lrig2 are frequently co-expressed, Lrig1−/−;Lrig2−/− double mutant ears show no enhanced structural abnormalities. At later stages, Lrig1 expression is sustained in non-sensory tissues, whereas Lrig2 levels are enhanced in neurons and sensory epithelia. Consistent with these distinct expression patterns, Lrig1 and Lrig2 mutant mice exhibit different forms of impaired auditory responsiveness. Notably, Lrig1−/−;Lrig2−/− double mutant mice display vestibular deficits and suffer from a more severe auditory defect that is accompanied by a cochlear innervation phenotype not present in single mutants. Thus, Lrig genes appear to act both redundantly and independently, with Lrig2 emerging as the most functionally distinct family member. PMID:24086156

[Hearing capacity and speech production in 417 children with facial cleft abnormalities].

PubMed

Schönweiler, R; Schönweiler, B; Schmelzeisen, R

1994-11-01

Children with cleft palates often suffer from chronic conductive hearing losses, delayed language acquisition and speech disorders. This study presents results of speech and language outcomes in relation to hearing function and types of palatal malformations found. 417 children with cleft palates were examined during followup evaluations that extended over several years. Disorders were studied as they affected the ears, nose and throat, audiometry and speech and language pathology. Children with isolated cleft lips were excluded. Among the total group, 8% had normal speech and language development while 92% had speech or language disorders. 80% of these latter children had hearing problems that predominantly consisted of fluctuating conductive hearing losses caused by otitis media with effusion. 5% had sensorineural hearing losses. Fifty-eight children (14%) with rhinolalia aperta were not improved by speech therapy and required velopharyngoplasties, using a cranial-based pharyngeal flap. Language skills did not depend on the type of cleft palate presents but on the frequency and amount of hearing loss found. Otomicroscopy and audiometric follow-ups with insertions of ventilation tubes were considered to be most important for language development in those children with repeated middle ear infections. Speech or language therapy was necessary in 49% of the children.

Gestational lead exposure induces developmental abnormalities and up-regulates apoptosis of fetal cerebellar cells in rats.

PubMed

Mousa, Alyaa M; Al-Fadhli, Ameera S; Rao, Muddanna S; Kilarkaje, Narayana

2015-01-01

Lead (Pb), a known environmental toxicant, adversely affects almost all organ systems. In this study, we investigated the effects of maternal lead exposure on fetal rat cerebellum. Female Sprague-Dawley rats were given lead nitrate in drinking water (0, 0.5, and 1%) for two weeks before conception, and during pregnancy. Fetuses were collected by caesarian section on gestational day 21 and observed for developmental abnormalities. The fetal cerebellar sections from control and 1% lead group were stained with cresyl violet. Immunohistochemical expressions of p53, Bax, Bcl-2, and caspase 3 were quantified by AnalySIS image analyzer (Life Science, Germany). Lead exposure induced developmental abnormalities of eyes, ear, limbs, neck and ventral abdominal wall; however, these abnormalities were commonly seen in the 1% lead-treated group. In addition, lead also caused fetal mortality and reduced body growth in both dose groups and reduced brain weight in the 1% lead-treated group. The fetal cerebella from the 1% lead-treated group showed unorganized cerebellar cortical layers, and degenerative changes in granule and Purkinje cells such as the formation of clumps of Nissl granules. An increase in Bax and caspase 3, and a decrease in Bcl-2 (p < 0.05), but not in p53, showed apoptosis of the neurons. In conclusion, gestational lead exposure in rats induces fetal toxicity and developmental abnormalities. The lead exposure also impairs development of cerebellar layers, induces structural changes, and apoptosis in the fetal cerebellar cortex. These results suggest that lead exposure during gestation is extremely toxic to developing cerebellum in rats.

CHARGE association in a child with de novo inv dup (14)(q22{yields}q24.3)

DOE Office of Scientific and Technical Information (OSTI.GOV)

North, K.; Wu, B.L.; Whiteman, D.

1994-09-01

The CHARGE association is an increasingly recognized complex of multiple malformations, that include Coloboma, Heart defect, choanal Atresia, Retardation of mental and somatic development, hypoplastic Genitalia, and Ear abnormalities or deafness. It has been postulated that many of the defects result from abnormalities in the development, migration or interaction of cells of the cephalic neural crest. The majority of cases are sporadic. We report a case of an inverted duplication (14)(q22{yields}q24.3) associated with CHARGE association. The patient was a 4 {1/2}-year-old female and was the product of a normal pregnancy. Family history was unremarkable. The clinical manifestations included the combinationmore » of congenital anomalies (coloboma, ventricular septal defect, severe developmental delay and growth retardation, genital hypoplasia and sensorineural deafness) in association with soft tissue choanal atresia, dysphagia, and minor dysmorphic features (low set ears, upslanting palpebral fissures). High resolution cytogenetic studies revealed that the child has 46,XX,inv dup(14)(q22{yields}q24.3) and parents have normal chromosomes. FISH with a chromosome 14 paint probe confirmed that the duplicated region is entirely derived from chromosome 14. FISH with D22S75 probe for region 22q11.2 detected no deletion for this locus. Several duplications or deletions involving different chromosomes have been reported for patients with conditions resembling CHARGE association. This indicates that CHARGE is possible genetically heterogenous, parallelling the phenotypic heterogeneity of the disorder. Two published cases with unbalanced rearrengements involving 14q22 have some comparable features with our case, which suggests that the locus for a gene causing some of the features of CHARGE association may reside at 14q22 or 14q24.3.« less

A Histological Study of Scala Communis with Radiological Implications

PubMed Central

Makary, Chadi; Shin, Jennifer; Caruso, Paul; Curtin, Hugh; Merchant, Saumil

2010-01-01

Objectives Scala communis or interscalar septum (IS) defect is a developmental abnormality of the inner ear characterized by a dehiscence in the partition separating the turns of the cochlea. The goals of the present study were to (1) study this anomaly and describe its characteristics compared to control ears using a histological analysis of temporal bones, (2) discuss radiological implications regarding its diagnosis, and (3) describe its embryological derivation. Methods Out of 1775 temporal bones assessed, 22 specimens were found to have scala communis in cochleae containing all 3 turns (basal, middle and apical). These 22 ears were studied in detail by qualitative and quantitative methods using light microscopy. Results Scala communis occurred as an isolated inner ear anomaly, or in association with other congenital cochlear and/or vestibular anomalies. The defect occurred most often between the middle and apical turns of the cochlea. Compared to control ears, scala communis ears were found to have a smaller modiolar area (p < 0.0001) and flattening of the interscalar ridge (point of attachment of the IS to the inner lumen of the cochlea; p < 0.0001). Scala communis was compatible with normal hearing. Conclusions Flattening of the interscalar ridge has the potential to improve the diagnosis of scala communis in patients using CT scanning. The anomaly may result from a mesodermal defect such as excessive resorption of mesenchyme during the formation of the scalae, an error in the formation of bone, or both. PMID:20389062

Otologic Manifestations of Autosomal Recessive Congenital Ichthyosis in Children.

PubMed

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

2015-11-01

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

A histological study of scala communis with radiological implications.

PubMed

Makary, Chadi; Shin, Jennifer; Caruso, Paul; Curtin, Hugh; Merchant, Saumil

2010-01-01

Scala communis or interscalar septum (IS) defect is a developmental abnormality of the inner ear characterized by a dehiscence in the partition separating the turns of the cochlea. The goals of the present study were to (1) study this anomaly and describe its characteristics compared to control ears using a histological analysis of temporal bones, (2) discuss radiological implications regarding its diagnosis, and (3) describe its embryological derivation. Out of 1775 temporal bones assessed, 22 specimens were found to have scala communis in cochleae containing all 3 turns (basal, middle and apical). These 22 ears were studied in detail by qualitative and quantitative methods using light microscopy. Scala communis occurred as an isolated inner ear anomaly, or in association with other congenital cochlear and/or vestibular anomalies. The defect occurred most often between the middle and apical turns of the cochlea. Compared to control ears, scala communis ears were found to have a smaller modiolar area (p < 0.0001) and flattening of the interscalar ridge (point of attachment of the IS to the inner lumen of the cochlea; p < 0.0001). Scala communis was compatible with normal hearing. Flattening of the interscalar ridge has the potential to improve the diagnosis of scala communis in patients using CT scanning. The anomaly may result from a mesodermal defect such as excessive resorption of mesenchyme during the formation of the scalae, an error in the formation of bone, or both. Copyright © 2010 S. Karger AG, Basel.

Scanning Thin-Sheet Laser Imaging Microscopy Elucidates Details on Mouse Ear Development

PubMed Central

Kopecky, Benjamin; Johnson, Shane; Schmitz, Heather; Santi, Peter; Fritzsch, Bernd

2016-01-01

Background The mammalian inner ear is transformed from a flat placode into a three-dimensional (3D) structure with six sensory epithelia that allow for the perception of sound and both linear and angular acceleration. While hearing and balance problems are typically considered to be adult onset diseases, they may arise as a developmental perturbation to the developing ear. Future prevention of hearing or balance loss requires an understanding of how closely genetic mutations in model organisms reflect the human case, necessitating an objective multidimensional comparison of mouse ears with human ears that have comparable mutations in the same gene. Results Here, we present improved 3D analyses of normal murine ears during embryonic development using optical sections obtained through Thin-Sheet Laser Imaging Microscopy. We chronicle the transformation of an undifferentiated otic vesicle between mouse embryonic day 11.5 to a fully differentiated inner ear at postnatal day 15. Conclusions Our analysis of ear development provides new insights into ear development, enables unique perspectives into the complex development of the ear, and allows for the first full quantification of volumetric and linear aspects of ear growth. Our data provide the framework for future analysis of mutant phenotypes that are currently under-appreciated using only two dimensional renderings. PMID:22271591

Scanning thin-sheet laser imaging microscopy elucidates details on mouse ear development.

PubMed

Kopecky, Benjamin; Johnson, Shane; Schmitz, Heather; Santi, Peter; Fritzsch, Bernd

2012-03-01

The mammalian inner ear is transformed from a flat placode into a three-dimensional (3D) structure with six sensory epithelia that allow for the perception of sound and both linear and angular acceleration. While hearing and balance problems are typically considered to be adult onset diseases, they may arise as a developmental perturbation to the developing ear. Future prevention of hearing or balance loss requires an understanding of how closely genetic mutations in model organisms reflect the human case, necessitating an objective multidimensional comparison of mouse ears with human ears that have comparable mutations in the same gene. Here, we present improved 3D analyses of normal murine ears during embryonic development using optical sections obtained through Thin-Sheet Laser Imaging Microscopy. We chronicle the transformation of an undifferentiated otic vesicle between mouse embryonic day 11.5 to a fully differentiated inner ear at postnatal day 15. Our analysis of ear development provides new insights into ear development, enables unique perspectives into the complex development of the ear, and allows for the first full quantification of volumetric and linear aspects of ear growth. Our data provide the framework for future analysis of mutant phenotypes that are currently under-appreciated using only two dimensional renderings. Copyright © 2012 Wiley Periodicals, Inc.

Red ear syndrome precipitated by a dietary trigger: a case report

PubMed Central

2014-01-01

Introduction Red ear syndrome is a rare condition characterized by episodic attacks of erythema of the ear accompanied by burning ear pain. Symptoms are brought on by touch, exertion, heat or cold, stress, neck movements and washing or brushing of hair. Diagnosis and treatment of this condition are challenging. The case we report here involves a woman whose symptoms were brought on by a dietary trigger: orange juice as well as stress, causing significant physical and psychological morbidity. Avoidance of triggers resulted in symptomatic improvement. Case presentation A 22-year-old Caucasian woman who was a student presented twice to our department with evolving symptoms, the first time with hyperacusis (abnormal sound sensitivity arising from within the auditory system to sounds of moderate volume), intermittent right tinnitus and subjective hearing difficulties. She presented five years later with highly distressing episodes of erythematous ears, which were associated with burning pain around the ear and temporal areas, and intolerance to noise. After keeping a symptom diary, she identified orange juice and stress as triggers of her symptoms. No local head and neck pathology was present. Investigations and imaging were negative. Avoidance of triggers led to great symptomatic improvement. To the best of our knowledge, dietary triggers have not previously been reported as a trigger for this syndrome. This case shows a direct temporal link to a dietary trigger and supports a primary pathogenesis. Recognition and management of primary headache disorder and simple dietary and lifestyle changes brought about symptomatic relief. Conclusion Red ear syndrome is a little-known clinical syndrome of unknown etiology and management. To the best of our knowledge, our present case report is the first to describe primary red ear syndrome triggered by orange juice. Clinical benefit derived from avoidance of this trigger, which is already known to precipitate migraines, gives some insight into the pathogenesis of red ear syndrome. PMID:25303997

[The anatomical features of the middle ear exerting the influence on the formation of exudative otitis media in the breast-fed infants of different gestational age].

PubMed

Matroskin, A G; Rakhmanova, I V; Dreval', A A; Kislyakov, A N; Vladimirov, A I

The objective of the present study was to elucidate the anatomical features of the structure of the middle ear and eustachian tube in the breast-fed infants of different gestational age that may be responsible for the formation of exudates (fluids). We have examined 150 temporal bones obtained from the children's cadavers that were allocated to three groups as follows: 50 temporal bones obtained at weeks 26-30 weeks of gestation (group 1), 44 bones 31-36 weeks of gestation (group 2), and 37-40 weeks of gestation (full-term babies, group 3),The analysis of the data obtained on an individual bases revealed either increase or decreases in the selected characteristics of the eustachian tube in comparison with the respective average values as well as the well apparent predominance of a single change or a combination of alteration of several parameters in one case in 26-30 weeks and 31-36 weeks groups. No significant changes were found in group 1. It is concluded that the presence of a single change or a combination of two or three abnormal changes in the parameters of the bone structures of the eustachian tube can affect the development of the secretory process in the middle ear especially in the children born after 36 weeks of pregnancy.

Sudden Sensorineural Hearing Loss: The Question of Perilymph Fistula.

ERIC Educational Resources Information Center

Backous, Douglas D.; Niparko, John K.

1997-01-01

Perilymph fistula (PLF) is an abnormal communication between the fluid-containing spaces of the inner ear and the air-containing spaces of the temporal bone that can cause hearing loss, tinnitus, aural fullness, vertigo, and postural instability. Diagnosis of PLF and management of those with presumed PLF are discussed. (Contains extensive…

Abnormal cortical sensorimotor activity during “Target” sound detection in subjects with acute acoustic trauma sequelae: an fMRI study

PubMed Central

Job, Agnès; Pons, Yoann; Lamalle, Laurent; Jaillard, Assia; Buck, Karl; Segebarth, Christoph; Delon-Martin, Chantal

2012-01-01

The most common consequences of acute acoustic trauma (AAT) are hearing loss at frequencies above 3 kHz and tinnitus. In this study, we have used functional Magnetic Resonance Imaging (fMRI) to visualize neuronal activation patterns in military adults with AAT and various tinnitus sequelae during an auditory “oddball” attention task. AAT subjects displayed overactivities principally during reflex of target sound detection, in sensorimotor areas and in emotion-related areas such as the insula, anterior cingulate and prefrontal cortex, in premotor area, in cross-modal sensory associative areas, and, interestingly, in a region of the Rolandic operculum that has recently been shown to be involved in tympanic movements due to air pressure. We propose further investigations of this brain area and fine middle ear investigations, because our results might suggest a model in which AAT tinnitus may arise as a proprioceptive illusion caused by abnormal excitability of middle-ear muscle spindles possibly link with the acoustic reflex and associated with emotional and sensorimotor disturbances. PMID:22574285

Auditory processing deficits in individuals with primary open-angle glaucoma.

PubMed

Rance, Gary; O'Hare, Fleur; O'Leary, Stephen; Starr, Arnold; Ly, Anna; Cheng, Belinda; Tomlin, Dani; Graydon, Kelley; Chisari, Donella; Trounce, Ian; Crowston, Jonathan

2012-01-01

The high energy demand of the auditory and visual pathways render these sensory systems prone to diseases that impair mitochondrial function. Primary open-angle glaucoma, a neurodegenerative disease of the optic nerve, has recently been associated with a spectrum of mitochondrial abnormalities. This study sought to investigate auditory processing in individuals with open-angle glaucoma. DESIGN/STUDY SAMPLE: Twenty-seven subjects with open-angle glaucoma underwent electrophysiologic (auditory brainstem response), auditory temporal processing (amplitude modulation detection), and speech perception (monosyllabic words in quiet and background noise) assessment in each ear. A cohort of age, gender and hearing level matched control subjects was also tested. While the majority of glaucoma subjects in this study demonstrated normal auditory function, there were a significant number (6/27 subjects, 22%) who showed abnormal auditory brainstem responses and impaired auditory perception in one or both ears. The finding that a significant proportion of subjects with open-angle glaucoma presented with auditory dysfunction provides evidence of systemic neuronal susceptibility. Affected individuals may suffer significant communication difficulties in everyday listening situations.

Relationship Between Audio-Vestibular Functional Tests and Inner Ear MRI in Meniere's Disease.

PubMed

Quatre, Raphaële; Attyé, Arnaud; Karkas, Alexandre; Job, Agnès; Dumas, Georges; Schmerber, Sébastien

2018-04-25

Meniere's disease is an inner ear disorder generally attributed to an endolymphatic hydrops. Different electrophysiological tests and imaging techniques have been developed to improve endolymphatic hydrops diagnosis. The goal of our study was to compare the sensitivity and the specificity of delayed inner ear magnetic resonance imaging (MRI) after intravenous injection of gadolinium with extratympanic clicks electrocochleography (EcochG), phase shift of distortion product otoacoustic emissions (shift-DPOAEs), and cervical vestibular-evoked myogenic potentials (cVEMP) for the diagnosis of Meniere's disease. Forty-one patients, with a total of 50 affected ears, were included prospectively from April 2015 to April 2016 in our institution. Patients included had definite or possible Meniere's disease based on the latest American Academy of Otolaryngology-Head and Neck Surgery guidelines revised in 2015. All patients went through delayed inner ear MRI after intravenous injection of gadolinium (three dimension-fluid attenuated inversion recovery sequences), pure-tone audiometry, extratympanic clicks EcochG, shift-DPOAEs, and cVEMP on the same day. Endolymphatic hydrops was graded on MRI using the saccule to utricle ratio inversion defined as when the saccule appeared equal or larger than the utricle. Abnormal EcochG and shift-DPOAEs in patients with definite Meniere's disease (DMD) were found in 68 and 64.5%, respectively. The two methods were significantly associated in DMD group. In DMD group, 25.7% had a positive MRI. The correlation between MRI versus EcochG and MRI versus shift-DPOAEs was not significant. MRI hydrops detection was correlated with hearing loss. Finally, 22.9% of DMD group had positive cVEMP. EcochG and shift-DPOAEs were both well correlated with clinical criteria of Meniere's disease. Inner ear MRI showed hydrops when hearing loss was higher than 35 dB. The shift-DPOAEs presented the advantage of a rapid and easy measurement if DPOAEs could be recorded (i.e., hearing threshold <60dB). In contrast, EcochG can be performed regardless of hearing loss. In combination with shift-DPOAEs, it enhances the chances to confirm the diagnosis with a better confidence.

Intratympanic hemorrhage and concussion in a football offensive lineman.

PubMed

Waninger, Kevin N; Gloyeske, Brian M; Hauth, John M; Vanic, Keith A; Yen, David M

2014-03-01

A 26-year-old arena football lineman participating in a "bull in the ring" blocking and tackling drill was blindsided by an opposing teammate. He sustained a direct helmet-to-helmet blow to the right temporal area. This case describes a unique mechanism of ear barotrauma (intratympanic hemorrhage) and concussion caused by helmet-to-helmet contact in American football. Initial sideline evaluation revealed mild headache, confusion, dizziness, photophobia, and tinnitus, all consistent with a concussion diagnosis. Physical examination revealed a large postauricular contusion over the right mastoid process and diffuse right intratympanic hemorrhage on examination. No hemotympanum or effusion of the middle ear was noted. Abnormalities were seen on vestibular testing and visual accommodation and convergence testing. Computed tomography and magnetic resonance imaging were performed with attention to the temporal bones and were normal. Neuropsychological testing was abnormal compared to baseline. Audiology testing was within normal limits. Diagnosis was intratympanic hemorrhage secondary to barotrauma caused by helmet-to-helmet contact, with mastoid contusion and season-ending concussion. The tympanic membrane (TM) is a highly vascular membrane that is very sensitive to variations of atmospheric pressure. Overpressure can enter the external auditory canal, stretching and displacing the TM medially and causing injury to the tympanic membrane secondary to barotrauma. This concussed football player was never able to return to play due to his postconcussive symptoms. He had no residual hearing or ear problems. Copyright © 2014 Elsevier Inc. All rights reserved.

Oculoauriculovertebral spectrum with radial anomaly in child.

PubMed

Taksande, Amar; Vilhekar, Krishna

2013-01-01

Oculoauriculovertebral spectrum (OAVS) or Goldenhar syndrome is a wide spectrum of congenital anomalies that involves structures arising from the first and second branchial arches. It is characterized by a wide spectrum of symptoms and physical features. These abnormalities mainly involve the cheekbones, jaws, mouth, ears, eyes, or vertebrae. Other conditions with ear and/or radial involvement, such as, the Nager syndrome, Holt-Oram syndrome, Radial-renal syndrome, facioauriculoradial dysplasia, Fanconi anemia, and Vertebral, Anal atresia, Cardiac, Trachea, Esophageal, Renal, and Limb (VACTERL) association should be considered for differential diagnosis. Here we report a child who had facial asymmetry, microsomia, microtia, congenital facial nerve palsy, conductive hearing loss, skin tags, iris coloboma, and preaxial polydactyly.

Introversion and individual differences in middle ear acoustic reflex function.

PubMed

Bar-Haim, Yair

2002-10-01

A growing body of psychophysiological evidence points to the possibility that individual differences in early auditory processing may contribute to social withdrawal and introverted tendencies. The present study assessed the response characteristics of the acoustic reflex arc of introverted-withdrawn and extraverted-sociable individuals. Introverts displayed a greater incidence of abnormal middle ear acoustic reflexes and lower acoustic reflex amplitudes than extraverts. These findings were strongest for stimuli presented at a frequency of 2000 Hz. Results are discussed in light of the controversy concerning the anatomic loci (peripheral vs. central neuronal activity) of the individual differences between introverts and extraverts in early auditory processing. Copyright 2002 Elsevier Science B.V.

Better late than never: effective air-borne hearing of toads delayed by late maturation of the tympanic middle ear structures.

PubMed

Womack, Molly C; Christensen-Dalsgaard, Jakob; Hoke, Kim L

2016-10-15

Most vertebrates have evolved a tympanic middle ear that enables effective hearing of airborne sound on land. Although inner ears develop during the tadpole stages of toads, tympanic middle ear structures are not complete until months after metamorphosis, potentially limiting the sensitivity of post-metamorphic juveniles to sounds in their environment. We tested the hearing of five species of toads to determine how delayed ear development impairs airborne auditory sensitivity. We performed auditory brainstem recordings to test the hearing of the toads and used micro-computed tomography and histology to relate the development of ear structures to hearing ability. We found a large (14-27 dB) increase in hearing sensitivity from 900 to 2500 Hz over the course of ear development. Thickening of the tympanic annulus cartilage and full ossification of the middle ear bone are associated with increased hearing ability in the final stages of ear maturation. Thus, juvenile toads are at a hearing disadvantage, at least in the high-frequency range, throughout much of their development, because late-forming ear elements are critical to middle ear function at these frequencies. We discuss the potential fitness consequences of late hearing development, although research directly addressing selective pressures on hearing sensitivity across ontogeny is lacking. Given that most vertebrate sensory systems function very early in life, toad tympanic hearing may be a sensory development anomaly. © 2016. Published by The Company of Biologists Ltd.

Respiratory problems in children with Down syndrome.

PubMed

Pandit, Chetan; Fitzgerald, Dominic A

2012-03-01

Down syndrome is associated with a significant health burden, which is particularly apparent in young children who will frequently present with cardiac and respiratory problems. Respiratory presentations include problems related to structural abnormalities of the airways and lungs, glue ears, recurrent lower respiratory tract infections and obstructive sleep apnoea. These conditions are readily identifiable and able to be treated. An awareness of the breadth of respiratory problems and a plan to monitor patients with Down syndrome for their development has the potential to improve outcomes. © 2011 The Authors. Journal of Paediatrics and Child Health © 2011 Paediatrics and Child Health Division (Royal Australasian College of Physicians).

Evidence of hearing loss in a “normally-hearing” college-student population

PubMed Central

Le Prell, C. G.; Hensley, B.N.; Campbell, K. C. M.; Hall, J. W.; Guire, K.

2011-01-01

We report pure-tone hearing threshold findings in 56 college students. All subjects reported normal hearing during telephone interviews, yet not all subjects had normal sensitivity as defined by well-accepted criteria. At one or more test frequencies (0.25–8 kHz), 7% of ears had thresholds ≥25 dB HL and 12% had thresholds ≥20 dB HL. The proportion of ears with abnormal findings decreased when three-frequency pure-tone-averages were used. Low-frequency PTA hearing loss was detected in 2.7% of ears and high-frequency PTA hearing loss was detected in 7.1% of ears; however, there was little evidence for “notched” audiograms. There was a statistically reliable relationship in which personal music player use was correlated with decreased hearing status in male subjects. Routine screening and education regarding hearing loss risk factors are critical as college students do not always self-identify early changes in hearing. Large-scale systematic investigations of college students’ hearing status appear to be warranted; the current sample size was not adequate to precisely measure potential contributions of different sound sources to the elevated thresholds measured in some subjects. PMID:21288064

Hearing loss and the central auditory system: Implications for hearing aids

NASA Astrophysics Data System (ADS)

Frisina, Robert D.

2003-04-01

Hearing loss can result from disorders or damage to the ear (peripheral auditory system) or the brain (central auditory system). Here, the basic structure and function of the central auditory system will be highlighted as relevant to cases of permanent hearing loss where assistive devices (hearing aids) are called for. The parts of the brain used for hearing are altered in two basic ways in instances of hearing loss: (1) Damage to the ear can reduce the number and nature of input channels that the brainstem receives from the ear, causing plasticity of the central auditory system. This plasticity may partially compensate for the peripheral loss, or add new abnormalities such as distorted speech processing or tinnitus. (2) In some situations, damage to the brain can occur independently of the ear, as may occur in cases of head trauma, tumors or aging. Implications of deficits to the central auditory system for speech perception in noise, hearing aid use and future innovative circuit designs will be provided to set the stage for subsequent presentations in this special educational session. [Work supported by NIA-NIH Grant P01 AG09524 and the International Center for Hearing & Speech Research, Rochester, NY.

Wideband acoustic absorbance in children with Down syndrome.

PubMed

Durante, Alessandra Spada; Santos, Mayara; Roque, Nayara M C de F; Gameiro, Marcella S; Almeida, Katia de; Sousa Neto, Osmar Mesquita de

2018-01-10

Tympanometry is currently the most frequently used tool for assessing the status of the middle ear, commonly assessed using a single 226Hz tone. However, the use of the Acoustic Immittance Measures with a wideband stimulus is a promising high-resolution evaluation, especially in individuals known to have middle ear alterations, such as Down syndrome patients. The aim of this study was to analyze the acoustic absorbance measurements in children with Down syndrome. Cross-sectional study, approved by the institution's ethics committee. Data were collected from 30 children, with a mean age of 8.4 years, 15 with Down syndrome (DS-study group) and 15 children with typical development and no hearing complaints (control group). Energy absorbance was measured at frequencies of 226-8000Hz at ambient pressure and at peak pressure as a function of frequency using TITAN equipment. Statistical analysis was performed using the established level of statistical significance of 5%. With the 226Hz probe tone, 30 ears of the control group and 22 of the study group exhibited Type A tympanograms, whereas Type B was observed in eight children in the study group. The mean acoustic absorbance ratio of the study group was lower than that of the control group at frequencies centered at 2520Hz (p=0.008) for those with normal tympanometry results, and 226-4000Hz (p<0.03) for those with a Type B tympanometry curve. The low energy absorption in the presence of normal tympanograms in children with Down syndrome may suggest middle ear abnormalities. Copyright © 2018 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

How many breaks do we need to CATCH on 22q11?

DOE Office of Scientific and Technical Information (OSTI.GOV)

Dallapiccola, B.; Pizzuti, A.; Novelli, G.

1996-07-01

The major clinical manifestations of DiGeorge syndrome (DGS; MIM 188400), which reflect developmental abnormalities of the 3d and 4th pharyngeal pouch derivatives, include thymus- and parathyroid-gland aplasia or hypoplasia and conotruncal cardiac malformations. The additional dysmorphic facial features, such as hypertelorism, cleft lip and palate, bifid uvula, and small/low-set ears, which are also common, presumably reflect the same defect. The DGS phenotype has been associated with chromosome abnormalities and, sometimes, is the effect of teratogenic agents such as retinoic acid and alcohol. 53 refs., 1 fig.

Stimulation from Cochlear Implant Electrodes Assists with Recovery from Asymmetric Perceptual Tilt: Evidence from the Subjective Visual Vertical Test

PubMed Central

Gnanasegaram, Joshua J.; Parkes, William J.; Cushing, Sharon L.; McKnight, Carmen L.; Papsin, Blake C.; Gordon, Karen A.

2016-01-01

Vestibular end organ impairment is highly prevalent in children who have sensorineural hearing loss (SNHL) rehabilitated with cochlear implants (CIs). As a result, spatial perception is likely to be impacted in this population. Of particular interest is the perception of visual vertical because it reflects a perceptual tilt in the roll axis and is sensitive to an imbalance in otolith function. The objectives of the present study were thus to identify abnormalities in perception of the vertical plane in children with SNHL and determine whether such abnormalities could be resolved with stimulation from the CI. Participants included 53 children (15.2 ± 4.0 years of age) with SNHL and vestibular loss, confirmed with vestibular evoked myogenic potential (VEMP) testing. Testing protocol was validated in a sample of nine young adults with normal hearing (28.8 ± 7.7 years). Perception of visual vertical was assessed using the static Subjective Visual Vertical (SVV) test performed with and without stimulation in the participants with cochleovestibular loss. Trains of electrical pulses were delivered by an electrode in the left and/or right ear. Asymmetric spatial orientation deficits were found in nearly half of the participants with CIs (24/53 [45%]). The abnormal perception in this cohort was exacerbated by visual tilts in the direction of their deficit. Electric pulse trains delivered using the CI shifted this abnormal perception towards center (i.e., normal; p = 0.007). Importantly, this benefit was realized regardless of which ear was stimulated. These results suggest a role for CI stimulation beyond the auditory system, in particular, for improving vestibular/balance function. PMID:27679562

[Preliminary application of video head impulse test in the diagnosis of vertigo].

PubMed

Zhang, Yanmei; Chen, Siqi; Zhong, Zhen; Chen, Li; Wu, Yuanding; Zhao, Guiping; Liu, Yuhe

2015-06-01

To investigate clinical application of head impulse test with video recording eye movements in the diagnosis of vertigo. The video head impulse test(vHIT) was used to measure the eye saccades and velocity gain in 95 patients with vertigo which were divided into two groups, peripheral vertigo (47 cases) and central vertigo(48 cases); the characteristics of eye saccades and velocity gain of six semicircular canals in different patients with vertigo were analyzed, and were compared between the two groups. The vHIT result in patients with peripheral vertigo: in 22 patients (23 affected ears) with Meniere's disease, 21 ears were abnormal (91. 3%); the vHIT results in 4 patients with vestibular schwannoma, 2 patients with vestibular neuritis, 5 patients with delayed endolymphatic hydrops, 6 patients with sudden hearing loss accompanied vertigo, and 8 patients with vestibular dysfunction, were abnormal with correct saccades and/or lower velocity gain of vHIT. The abnormal vHIT results were also found in 35 of 48 patients (72. 9%) with central vertigo, which including posterior cerebral circulation ischemia(7 patients), cerebral infarction/stroke(6 patients), and dizziness with vertigo(17 patients) and others(18 patients). Abnormal rate of vHIT in patients with peripheral vertigo was 95. 7% (45/47), which was significantly higher than that (72. 9%) in patients with central vertigo. It is easy to perform the vHIT which without adverse reactions. We can record high-frequency characteristics of vestibular-ocular reflex among six semicircular canals through vHIT. The vHIT results which show the function of vestibular ocular reflex in different diseases with vertigo, can help discriminate peripheral vertigo from central vertigo, and it is a practical assessment method for vertigo.

Growth and development of infants with asymptomatic congenital cytomegalovirus infection.

PubMed

Shan, Ruobing; Wang, Xiaoliang; Fu, Ping

2009-10-31

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

MAD ointment ameliorates Imiquimod-induced psoriasiform dermatitis by inhibiting the IL-23/IL-17 axis in mice.

PubMed

OuYang, Qiong; Pan, YaQian; Luo, HanQiong; Xuan, ChunXiao; Liu, JinE; Liu, Jun

2016-10-01

Psoriasis is a chronic auto-immune inflammation disease with skin lesions and abnormal keratinocyte proliferation. The IL-23/IL-17 axis plays an important role in the pathogenesis of psoriasis. Madecassoside (MAD) was the most important constituents isolated from Centella asiatica, which has long been used in dermatology, and it is supposed that MAD may have effects on psoriasis. In the present study, the BALB/c mice ear and back skin received IMQ for 6 consecutive days to induce psoriasis-like dermatitis. MAD ointment was applied 6h later after IMQ treatment, and the IL-23/IL-17 pathway was investigated. The HE staining, BrdU and Psoriasis Area and Severity Index (PASI) were used to score the severity of keratinocyte proliferation and inflammation of the skin. Real-time PCR and Western Blot were used to detect the IL-23/IL-17 related cytokines. Flow Cytometry were applied to observe the numbers of Th17 cells. Daily application of IMQ for 6days on mouse ear skin and back skin induced psoriasis-like dermatitis. Real-time PCR showed that mRNA level of IL-23, IL-22, IL-17A were significantly decreased by MAD ointment treatment in ear skin. HE staining and BrdU incorporation implied that MAD ointment reduced keratinocyte proliferation. Flow Cytometry results showed MAD ointment decreased the numbers of Th17 cells. Thus, MAD ointment ameliorates Imiquimod-induced skin inflammation and abnormal keratinocyte through regulate the IL-23/IL-17 axis. Copyright © 2016 Elsevier B.V. All rights reserved.

Study of two patients with craniosynostosis and deletions of 11q: One with features of Saethre-Chotzen syndrome and the other with concomitant partial trisomy 4q

DOE Office of Scientific and Technical Information (OSTI.GOV)

Morsey, S.; Lewanda, A.F.; Reid, C.S.

1994-09-01

Partial monosomy 11q is associated with metopic craniosynostosis and trigonocephaly. Prominant features in the over 30 reported cases include downslanting palpebral fissures, epicanthal folds, hypertelorism, ptosis, wide/depressed nasal bridge, low set malformed ears, downturned mouth, micro/retrognathia, digital and cardiac anomalies and psychomotor retardation. We evaluated two patients referred for abnormal head shape. The first carried a diagnosis of Saethre-Chotzen syndrome due to brachycephaly, facial asymmetry, ptosis, cupped ears, sundactyly of 2nd and 3rd digits, developmental delay, and VSD. Karyotype revealed 46,XY,del(11)(q24.1{yields}qter). No abnormality was noted of chromosome 7p, where the Saethre-Chotzen syndrome locus has been mapped. This suggests genetic heterogeneitymore » for this condition. The second patient had no prior diagnosis. He had trigonocephaly, bilateral cryptorchidism and inguinal hernias. He also had hypotelorism, epicanthal folds, synophrys, posteriorly rotated ears, horizontal crease below his lower lip, unilateral single palmar crease, mild soft tissue syndactyly and a shawl scrotum. His karyotype of 46,XY,-11,+der(11)t(4;11)(q31.3;q25) revealed both partial 11q monosomy and partial 4q trisomy (the latter associated with cryptorchidism, horizontal chin crease and single palmar crease). Deletions of 11q appear to produce a wide spectrum of defects, which may even mimic other known craniosynostotic conditions. Study of these patients may lead to the identification of new genes involved in craniofacial morphogenesis.« less

Type II first branchial cleft anomaly.

PubMed

Al-Mahdi, Akmam H; Al-Khurri, Luay E; Atto, Ghada Z; Dhaher, Ameer

2013-01-01

First branchial cleft anomaly is a rare disease of the head and neck. It accounts for less than 8% of all branchial abnormalities. It is classified into type I, which is thought to arise from the duplication of the membranous external ear canal and are composed of ectoderm only, and type II that have ectoderm and mesoderm. Because of its rarity, first branchial cleft anomaly is often misdiagnosed and results in inappropriate management. A 9-year-old girl presented to us with fistula in the submandibular region and discharge in the external ear. Under general anesthesia, complete surgical excision of the fistula tract was done through step-ladder approach, and the histopathologic examination confirmed the diagnosis of type II first branchial cleft anomaly.

Two Cases of Partial Trisomy 4p and Partial Trisomy 14q

PubMed Central

Kim, Yeo-Hyang; Kim, Heung-Sik; Ryoo, Nam-Hee

2013-01-01

We present clinical and cytogenetic data on 2 cases of partial trisomy 4p and partial trisomy 14q. Both patients had an extra der(14)t(4;14)(p15.31;q12) chromosome due to a 3:1 segregation from a balanced translocation carrier mother. Array analyses indicated that their chromosomal breakpoints were similar, but there was no relationship between the 2 families. Both patients showed prominent growth retardation and psychomotor developmental delay. Other phenotypic manifestations were generally mild and variable; for example, patient 1 had a short palpebral fissure and low-set ears whereas patient 2 had a round face, asymmetric eyes, small ears, a short neck, finger/toe abnormalities, and behavioral problems. PMID:23301226

Ear Infections and Language Development.

ERIC Educational Resources Information Center

Roberts, Joanne E.; Zeisel, Susan A.

Ear infections in infants and preschoolers can cause mild or moderate temporary hearing loss, which may in turn affect a child's ability to understand and learn language. Noting that providing children with proper medical treatment for ear infections or middle ear fluid is important in preventing possible problems with language development, this…

Effect of Otitis Media with Effusion on Comodulation Masking Release in Children.

ERIC Educational Resources Information Center

Hall, Joseph W.; Grose, John H.

1994-01-01

This study investigated comodulation masking release (CMR) in 42 children (ages 5 to 10) with a history of otitis media with effusion (OME) and 19 control children with no history of ear disease. Results indicated that monotic CMRs were reduced in children with hearing loss due to OME and these CMRs remained abnormally small for several months.…

Non-mosaic tetrasomy 9p in a liveborn infant with multiple congenital anomalies: Case report and comparison with trisomy 9p

DOE Office of Scientific and Technical Information (OSTI.GOV)

Leichtman, L.G.; Zackowski, J.L.; Storto, P.D.

1996-06-14

Tetrasomy of the short(p) arm of chromosome 9 has been reported in few cases. Most of these children present with microbrachycephaly, wide forehead, hypertelorism, lowset, malformed ears, beaked noses, and micrognathia. Additional anomalies include short neck, congenital heart disease, genital abnormalities, multiple limb defects, hypotonia, and early death.

Molecular mechanisms of inner ear development.

PubMed

Wu, Doris K; Kelley, Matthew W

2012-08-01

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

Clinical use of vestibular evoked myogenic potentials in the evaluation of patients with air-bone gaps.

PubMed

Zhou, Guangwei; Poe, Dennis; Gopen, Quinton

2012-10-01

To determine the value of vestibular evoked myogenic potential (VEMP) test in clinical evaluation of air-bone gaps. Retrospective case review. Tertiary referral center. A total of 120 patients underwent VEMP testing during clinical investigation of significant air-bone gaps in their audiograms. Otologic examination and surgeries, high-resolution computerized tomography (CT), air and bone audiometry, tympanometry, acoustic reflex, and VEMP test. Imaging studies demonstrating structural anomalies in the temporal bone. Audiologic outcomes of air-bone gaps and VEMP thresholds. Surgical findings confirming imaging results. Middle ear pathologies, such as otosclerosis and chronic otitis media, were identified in 50 patients, and all of them had absent VEMP responses elicited by air-conduction stimuli. Moreover, 13 of them had successful middle ear surgeries with closures of the air-bone gaps. Abnormally low VEMP thresholds were found in 71 of 73 ears with inner ear anomalies, such as semicircular canal dehiscence and enlarged vestibular aqueduct. Seven patients with superior semicircular canal dehiscence underwent plugging procedure via middle fossa approach, and VEMP thresholds became normalized after the surgery in 3 of them. VEMP test failed to provide accurate diagnosis in only 3 cases. Air-bone gaps may be a result of various otologic pathologies, and the VEMP test is useful during clinical evaluation, better than tympanometry and acoustic reflexes. To avoid unnecessary middle ear surgery for air-bone gaps with unknown or unsure cause, VEMP test should be used in the differential diagnosis before an expensive imaging study.

Calcium oxalate stone formation in the inner ear as a result of an Slc26a4 mutation.

PubMed

Dror, Amiel A; Politi, Yael; Shahin, Hashem; Lenz, Danielle R; Dossena, Silvia; Nofziger, Charity; Fuchs, Helmut; Hrabé de Angelis, Martin; Paulmichl, Markus; Weiner, Steve; Avraham, Karen B

2010-07-09

Calcium oxalate stone formation occurs under pathological conditions and accounts for more than 80% of all types of kidney stones. In the current study, we show for the first time that calcium oxalate stones are formed in the mouse inner ear of a genetic model for hearing loss and vestibular dysfunction in humans. The vestibular system within the inner ear is dependent on extracellular tiny calcium carbonate minerals for proper function. Thousands of these biominerals, known as otoconia, are associated with the utricle and saccule sensory maculae and are vital for mechanical stimulation of the sensory hair cells. We show that a missense mutation within the Slc26a4 gene abolishes the transport activity of its encoded protein, pendrin. As a consequence, dramatic changes in mineral composition, size, and shape occur within the utricle and saccule in a differential manner. Although abnormal giant carbonate minerals reside in the utricle at all ages, in the saccule, a gradual change in mineral composition leads to a formation of calcium oxalate in adult mice. By combining imaging and spectroscopy tools, we determined the profile of mineral composition and morphology at different time points. We propose a novel mechanism for the accumulation and aggregation of oxalate crystals in the inner ear.

A comprehensive newborn exam: part I. General, head and neck, cardiopulmonary.

PubMed

Lewis, Mary L

2014-09-01

A comprehensive newborn examination involves a systematic inspection. A Ballard score uses physical and neurologic characteristics to assess gestational age. Craniosynostosis is caused by premature fusion of the sutures, and 20% of children with this condition have a genetic mutation or syndrome. The red reflex assessment is normal if there is symmetry in both eyes, without opacities, white spots, or dark spots. If the red reflex findings are abnormal or the patient has a family history of pertinent eye disorders, consultation with an ophthalmologist is warranted. Newborns with low-set ears should be evaluated for a genetic condition. Renal ultrasonography should be performed only in patients with isolated ear anomalies, such as preauricular pits or cup ears, if they are accompanied by other malformations or significant family history. If ankyloglossia is detected, a frenotomy may be considered if it impacts breastfeeding. The neck should be examined for full range of motion because uncorrected torticollis can lead to plagiocephaly and ear misalignment. Proper auscultation is crucial for evaluation of the bronchopulmonary circulation with close observation for signs of respiratory distress, including tachypnea, nasal flaring, grunting, retractions, and cyanosis. Benign murmurs are often present in the first hours of life. Pulse oximetry should be performed in a systematic fashion before discharge.

Otitis and autism spectrum disorders

PubMed Central

Tajima-Pozo, Kazuhiro; Zambrano-Enriquez, Diana; De Anta, Laura; Zelmanova, Julie; De Dios Vega, Jose Luis; Lopez-Ibor, Juan Jose

2010-01-01

The case of a 5-year-old child diagnosed as having pervasive developmental disorder (PDD), autistic type, from age 1 is reported. After surgery of vegetation in middle ear for repetitive otitis, the child presented an improvement in autistic behaviours, previously expressed as impaired social interactions, qualitative abnormalities in communication, a marked delay in language development, echolalia, stereotypies and self-aggressive behaviours. The aim of this paper is to bring attention to occurrences of misdiagnosis of PDD, which can occur when an adequate screening of the autistic syndrome is not realised. The result of the surgery was an improvement in autistic behaviours, despite the persistence of less severe autistic traits that may be more closely related to Asperger’s syndrome. PMID:22736729

Gfi1-Cre knock-in mouse line: A tool for inner ear hair cell-specific gene deletion

PubMed Central

Yang, Hua; Gan, Jean; Xie, Xiaoling; Deng, Min; Feng, Liang; Chen, Xiaowei; Gao, Zhiqiang; Gan, Lin

2010-01-01

Summary Gfi1encodes a zinc-finger transcription factor essential for the development and maintenance of haematopoiesis and the inner ear. In mouse inner ear, Gfi1 expression is confined to hair cells during development and in adulthood. To construct a genetic tool for inner ear hair cell-specific gene deletion, we generated a Gfi1-Cre mouse line by knocking-in Cre coding sequences into the Gfi1 locus and inactivating the endogenous Gfi1. The specificity and efficiency of Gfi1-Cre recombinase-mediated recombination in the developing inner ear was revealed through the expression of the conditional R26R-lacZ reporter gene. The onset of lacZ expression in the Gfi1Cre/+ inner ear was first detected at E13.5 in the vestibule and at E15.5 in the cochlea, coinciding with the generation of hair cells. Throughout inner ear development, lacZ expression was detected only in hair cells. Thus, Gfi1-Cre knock-in mouse line provides a useful tool for gene manipulations specifically in inner ear hair cells. PMID:20533399

Sources of DPOAEs revealed by suppression experiments, inverse fast Fourier transforms, and SFOAEs in impaired ears

NASA Astrophysics Data System (ADS)

Konrad-Martin, Dawn; Neely, Stephen T.; Keefe, Douglas H.; Dorn, Patricia A.; Cyr, Emily; Gorga, Michael P.

2002-04-01

DPOAE sources are modeled by intermodulation distortion generated near the f2 place and a reflection of this distortion near the DP place. In a previous paper, inverse fast Fourier transforms (IFFTs) of DPOAE filter functions in normal ears were consistent with this model [Konrad-Martin et al., J. Acoust. Soc. Am. 109, 2862-2879 (2001)]. In the present article, similar measurements were made in ears with specific hearing-loss configurations. It was hypothesized that hearing loss at f2 or DP frequencies would influence the relative contributions to the DPOAE from the corresponding basilar membrane places, and would affect the relative magnitudes of SFOAEs at frequencies equal to f2 and fDP. DPOAEs were measured with f2=4 kHz, f1 varied, and a suppressor near fDP. L2 was 25-55 dB SPL (L1=L2+10 dB). SFOAEs were measured at f2 and at 2.7 kHz (the average fDP produced by the f1 sweep) for stimulus levels of 20-60 dB SPL. SFOAE results supported predictions of the pattern of amplitude differences between SFOAEs at 4 and 2.7 kHz for sloping losses, but did not support predictions for the rising- and flat-loss categories. Unsuppressed IFFTs for rising losses typically had one peak. IFFTs for flat or sloping losses typically have two or more peaks; later peaks were more prominent in ears with sloping losses compared to normal ears. Specific predictions were unambiguously supported by the results for only four of ten cases, and were generally supported in two additional cases. Therefore, the relative contributions of the two DPOAE sources often were abnormal in impaired ears, but not always in the predicted manner.

Ectopic Mineralization in the Middle Ear and Chronic Otitis Media with Effusion Caused by RPL38 Deficiency in the Tail-short (Ts) Mouse*

PubMed Central

Noben-Trauth, Konrad; Latoche, Joseph R.

2011-01-01

Inflammation of the middle ear cavity (otitis media) and the abnormal deposition of bone at the otic capsule are common causes of conductive hearing impairment in children and adults. Although a host of environmental factors can contribute to these conditions, a genetic predisposition has an important role as well. Here, we analyze the Tail-short (Ts) mouse, which harbors a spontaneous semi-dominant mutation that causes skeletal defects and hearing loss. By genetic means, we show that the Ts phenotypes arise from an 18-kb deletion/insertion of the Rpl38 gene, encoding a ribosomal protein of the large subunit. We show that Ts mutants exhibit significantly elevated auditory-brain stem response thresholds and reduced distortion-product otoacoustic emissions, in the presence of normal endocochlear potentials and typical inner ear histology suggestive of a conductive hearing impairment. We locate the cause of the hearing impairment to the middle ear, demonstrating over-ossification at the round window ridge, ectopic deposition of cholesterol crystals in the middle ear cavity, enlarged Eustachian tube, and chronic otitis media with effusion all beginning at around 3 weeks after birth. Using specific antisera, we demonstrate that Rpl38 is an ∼8-kDa protein that is predominantly expressed in mature erythrocytes. Finally, using an Rpl38 cDNA transgene, we rescue the Ts phenotypes. Together, these data present a previously uncharacterized combination of interrelated middle ear pathologies and suggest Rpl38 deficiency as a model to dissect the causative relationships between neo-ossification, cholesterol crystal deposition, and Eustachian tubes in the etiology of otitis media. PMID:21062742

The molecular basis of neurosensory cell formation in ear development: a blueprint for hair cell and sensory neuron regeneration?

PubMed Central

Fritzsch, Bernd; Beisel, Kirk W.; Hansen, Laura

2014-01-01

Summary The inner ear of mammals uses neurosensory cells derived from the embryonic ear for mechanoelectric transduction of vestibular and auditory stimuli (the hair cells) and conducts this information to the brain via sensory neurons. As with most other neurons of mammals, lost hair cells and sensory neurons are not spontaneously replaced and result instead in age-dependent progressive hearing loss. We review the molecular basis of neurosensory development in the mouse ear to provide a blueprint for possible enhancement of therapeutically useful transformation of stem cells into lost neurosensory cells. We identify several readily available adult sources of stem cells that express, like the ectoderm-derived ear, genes known to be essential for ear development. Use of these stem cells combined with molecular insights into neurosensory cell specification and proliferation regulation of the ear, might allow for neurosensory regeneration of mammalian ears in the near future. PMID:17120192

Ear wax

MedlinePlus

See your provider if your ears are blocked with wax and you are unable to remove the wax. Also call if you have an ear wax blockage and you develop new symptoms, such as: Drainage from the ear Ear pain Fever Hearing loss that continues after you clean the wax

Molecular Mechanisms of Inner Ear Development

PubMed Central

Wu, Doris K.; Kelley, Matthew W.

2012-01-01

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

Cochlin-tomoprotein (CTP) detection test identified perilymph leakage preoperatively in revision stapes surgery.

PubMed

Kataoka, Yuko; Ikezono, Tetsuo; Fukushima, Kunihiro; Yuen, Koji; Maeda, Yukihide; Sugaya, Akiko; Nishizaki, Kazunori

2013-08-01

Perilymphatic fistula (PLF) is defined as an abnormal leakage between perilymph from the labyrinth to the middle ear. Symptoms include hearing loss, tinnitus, and vertigo. The standard mode of PLF detection is intraoperative visualization of perilymph leakage and fistula, which ostensibly confirms the existence of PLF. Other possible methods of diagnosis include confirmation of pneumolabyrinth via diagnostic imaging. Recently, a cochlin-tomoprotein (CTP) detection test has been developed that allows definitive diagnosis of PLF-related hearing loss. We report the case of a 45-year-old man who presented with right-sided tinnitus, hearing loss, and dizziness 30 years after stapes surgery. Middle ear lavage was performed after myringotomy. A preoperative diagnosis of PLF was reached using the CTP detection test. Intraoperative observations included a necrotic long process of the incus, displaced wire piston, and fibrous tissue in the oval window. Perilymph leakage was not evident. The oval window was closed with fascia, and vertigo disappeared within 2 weeks postoperatively. When PLF is suspected after stapes surgery, the CTP detection test can be a useful, highly sensitive, and less invasive method for preoperative diagnosis. Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.

Growth retardation, intellectual disability, facial anomalies, cataract, thoracic hypoplasia and skeletal abnormalities: a novel phenotype

PubMed Central

Shah, Hitesh; Bens, Susanne; Caliebe, Almuth; Graham, John M.; Girisha, Katta Mohan

2012-01-01

We report a fourteen year old adolescent girl with growth deficiency, microcephaly, intellectual disability, distinctive dysmorphic features (bulbous nose with wide nasal base, hypotelorism, deeply set eyes, protruding cupped ears and thick lips), cataract, pigmentary retinopathy, hypoplastic thorax, kyphoscoliosis and unusual skeletal changes but without chromosomal imbalances detected by array-CGH who probably represents a novel phenotype. PMID:22987502

From teosinte to maize: the catastrophic sexual transmutation.

PubMed

Iltis, H H

1983-11-25

An alternative to the theory that the ear of maize (Zea mays ssp. mays) evolved from a slender female ear of a Mexican annual teosinte holds that it was derived from the central spike of a male teosinte inflorescence (tassel) which terminates the primary lateral branches. This alternative hypothesis is more consistent with morphology and explains the anomalous lack of significant genetic and biochemical differences between these taxa. Maize, the only cereal with unisexual inflorescences, evolved through a sudden epigenetic sexual transmutation involving condensation of primary branches, which brought their tassels into the zone of female expression, leading to strong apical dominance and a catastrophic shift in nutrient allocation. Initially, this quantum change may have involved no new mutations, but rather genetic assimilation under human selection of an abnormality, perhaps environmentally triggered.

Temporal and spatial expression patterns of Hedgehog receptors in the developing inner and middle ear.

PubMed

Shin, Jeong-Oh; Ankamreddy, Harinarayana; Jakka, Naga Mahesh; Lee, Seokwon; Kim, Un-Kyung; Bok, Jinwoong

2017-01-01

The mammalian inner ear is a complex organ responsible for balance and hearing. Sonic hedgehog (Shh), a member of the Hedgehog (Hh) family of secreted proteins, has been shown to play important roles in several aspects of inner ear development, including dorsoventral axial specification, cochlear elongation, tonotopic patterning, and hair cell differentiation. Hh proteins initiate a downstream signaling cascade by binding to the Patched 1 (Ptch1) receptor. Recent studies have revealed that other types of co-receptors can also mediate Hh signaling, including growth arrest-specific 1 (Gas1), cell-adhesion molecules-related/down-regulated by oncogenes (Cdon), and biregional Cdon binding protein (Boc). However, little is known about the role of these Hh co-receptors in inner ear development. In this study, we examined the expression patterns of Gas1, Cdon, and Boc, as well as that of Ptch1, in the developing mouse inner ear from otocyst (embryonic day (E) 9.5) until birth and in the developing middle ear at E15.5. Ptch1, a readout of Hh signaling, was expressed in a graded pattern in response to Shh signaling throughout development. Expression patterns of Gas1, Cdon, and Boc differed from that of Ptch1, and each Hh co-receptor was expressed in specific cells and domains in the developing inner and middle ear. These unique and differential expression patterns of Hh co-receptors suggest their roles in mediating various time- and space-specific functions of Shh during ear development.

A multiple translocation event in a patient with hexadactyly, facial dysmorphism, mental retardation and behaviour disorder characterised comprehensively by molecular cytogenetics. Case report and review of the literature.

PubMed

Seidel, Jörg; Heller, Anita; Senger, Gabriele; Starke, Heike; Chudoba, Ilse; Kelbova, Christina; Tönnies, Holger; Neitzel, Heidemarie; Haase, Claudia; Beensen, Volkmar; Zintl, Felix; Claussen, Uwe; Liehr, Thomas

2003-09-01

We report a 13-year-old female patient with multiple congenital abnormalities (microcephaly, facial dysmorphism, anteverted dysplastic ears and postaxial hexadactyly), mental retardation, and adipose-gigantism. Ultrasonography revealed no signs of a heart defect or renal abnormalities. She showed no speech development and suffered from a behavioural disorder. CNS abnormalities were excluded by cerebral MRI. Initial cytogenetic studies by Giemsa banding revealed an aberrant karyotype involving three chromosomes, t(2;4;11). By high resolution banding and multicolour fluoresence in-situ hybridisation (M-FISH, MCB), chromosome 1 was also found to be involved in the complex chromosomal aberrations, confirming the karyotype 46,XX,t(2;11;4).ish t(1;4;2;11)(q43;q21.1;p12-p13.1;p14.1). To the best of our knowledge no patient has been previously described with such a complex translocation involving 4 chromosomes. This case demonstrates that conventional chromosome banding techniques such as Giemsa banding are not always sufficient to characterise complex chromosomal abnormalities. Only by the additional utilisation of molecular cytogenetic techniques could the complexity of the present chromosomal rearrangements and the origin of the involved chromosomal material be detected. Further molecular genetic studies will be performed to clarify the chromosomal breakpoints potentially responsible for the observed clinical symptoms. This report demonstrates that multicolour-fluorescence in-situ hybridisation studies should be performed in patients with congenital abnormalities and suspected aberrant karyotypes in addition to conventional Giemsa banding.

Responses of the ear to low frequency sounds, infrasound and wind turbines.

PubMed

Salt, Alec N; Hullar, Timothy E

2010-09-01

Infrasonic sounds are generated internally in the body (by respiration, heartbeat, coughing, etc) and by external sources, such as air conditioning systems, inside vehicles, some industrial processes and, now becoming increasingly prevalent, wind turbines. It is widely assumed that infrasound presented at an amplitude below what is audible has no influence on the ear. In this review, we consider possible ways that low frequency sounds, at levels that may or may not be heard, could influence the function of the ear. The inner ear has elaborate mechanisms to attenuate low frequency sound components before they are transmitted to the brain. The auditory portion of the ear, the cochlea, has two types of sensory cells, inner hair cells (IHC) and outer hair cells (OHC), of which the IHC are coupled to the afferent fibers that transmit "hearing" to the brain. The sensory stereocilia ("hairs") on the IHC are "fluid coupled" to mechanical stimuli, so their responses depend on stimulus velocity and their sensitivity decreases as sound frequency is lowered. In contrast, the OHC are directly coupled to mechanical stimuli, so their input remains greater than for IHC at low frequencies. At very low frequencies the OHC are stimulated by sounds at levels below those that are heard. Although the hair cells in other sensory structures such as the saccule may be tuned to infrasonic frequencies, auditory stimulus coupling to these structures is inefficient so that they are unlikely to be influenced by airborne infrasound. Structures that are involved in endolymph volume regulation are also known to be influenced by infrasound, but their sensitivity is also thought to be low. There are, however, abnormal states in which the ear becomes hypersensitive to infrasound. In most cases, the inner ear's responses to infrasound can be considered normal, but they could be associated with unfamiliar sensations or subtle changes in physiology. This raises the possibility that exposure to the infrasound component of wind turbine noise could influence the physiology of the ear. Copyright (c) 2010 Elsevier B.V. All rights reserved.

A comparison of tympanometry with 226 Hz and 1000 Hz probe tones in children with Down syndrome.

PubMed

Lewis, Meagan P; Bradford Bell, Elizabeth; Evans, Adele K

2011-12-01

For children with Down syndrome, the incidence of hearing loss may be as high as 78% [1], therefore the American Academy of Pediatrics recommends regular screening for the presence of hearing abnormalities. Tympanometry is used as an indication of middle ear pathology. In our experience, Down's patients' tympanograms do not always correlate with otoscopic findings. Down's patients have joint laxity, small ear canals, anterior tympanic membrane orientation and softer tissue composition, all factors thought to affect tympanogram results in infants. Because the use of the 1000 Hz tympanometry is widely recognized as standard procedure in the evaluation of infants aged 0-6 months, we propose it may have greater reliability in testing patients with Down syndrome. Compare the results of visual inspection of the tympanic membrane by a Pediatric Otolaryngologist to the results of tympanometry at traditional probe tone (226 Hz) and at the infant probe tone (1000 Hz). Institutional Review Board - approved prospective study of 26 subject-ears in patients with Down syndrome aged 6 months-18 years but recent stable middle ear/Eustachian tube function using physical examination and tympanometric probe tones at 226 Hz and 1000 Hz. Subject-ears were examined with record of "clear of effusion," showed the presence of "fluid," or were to be "excluded." Blinded to ear exam results, tympanometry was then completed with record of which Jerger classification tympanogram was found at each frequency. Although the sensitivity of each test was 1, the specificity of the 1000 Hz tympanometry (100%) in this study was markedly improved compared to the specificity of the 226 Hz tympanometry (71%) (p=0.016). This pilot study demonstrated evidence that tympanometry in children with Down syndrome may be more reliable at 1000 Hz than at 226 Hz in detecting the presence of middle ear effusion beyond infancy. Use of the 1000 Hz probe tone yielded fewer false positives for disease (type B tympanograms in the setting of absent middle ear disease). Further studies of a larger patient population are needed to corroborate these results. Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.

Abnormal placental development and early embryonic lethality in EpCAM-null mice.

PubMed

Nagao, Keisuke; Zhu, Jianjian; Heneghan, Mallorie B; Hanson, Jeffrey C; Morasso, Maria I; Tessarollo, Lino; Mackem, Susan; Udey, Mark C

2009-12-31

EpCAM (CD326) is encoded by the tacstd1 gene and expressed by a variety of normal and malignant epithelial cells and some leukocytes. Results of previous in vitro experiments suggested that EpCAM is an intercellular adhesion molecule. EpCAM has been extensively studied as a potential tumor marker and immunotherapy target, and more recent studies suggest that EpCAM expression may be characteristic of cancer stem cells. To gain insights into EpCAM function in vivo, we generated EpCAM -/- mice utilizing an embryonic stem cell line with a tacstd1 allele that had been disrupted. Gene trapping resulted in a protein comprised of the N-terminus of EpCAM encoded by 2 exons of the tacstd1 gene fused in frame to betageo. EpCAM +/- mice were viable and fertile and exhibited no obvious abnormalities. Examination of EpCAM +/- embryos revealed that betageo was expressed in several epithelial structures including developing ears (otocysts), eyes, branchial arches, gut, apical ectodermal ridges, lungs, pancreas, hair follicles and others. All EpCAM -/- mice died in utero by E12.5, and were small, developmentally delayed, and displayed prominent placental abnormalities. In developing placentas, EpCAM was expressed throughout the labyrinthine layer and by spongiotrophoblasts as well. Placentas of EpCAM -/- embryos were compact, with thin labyrinthine layers lacking prominent vascularity. Parietal trophoblast giant cells were also dramatically reduced in EpCAM -/- placentas. EpCAM was required for differentiation or survival of parietal trophoblast giant cells, normal development of the placental labyrinth and establishment of a competent maternal-fetal circulation. The findings in EpCAM-reporter mice suggest involvement of this molecule in development of vital organs including the gut, kidneys, pancreas, lungs, eyes, and limbs.

Timely diagnosis of dairy calf respiratory disease using a standardized scoring system.

PubMed

McGuirk, Sheila M; Peek, Simon F

2014-12-01

Respiratory disease of young dairy calves is a significant cause of morbidity, mortality, economic loss, and animal welfare concern but there is no gold standard diagnostic test for antemortem diagnosis. Clinical signs typically used to make a diagnosis of respiratory disease of calves are fever, cough, ocular or nasal discharge, abnormal breathing, and auscultation of abnormal lung sounds. Unfortunately, routine screening of calves for respiratory disease on the farm is rarely performed and until more comprehensive, practical and affordable respiratory disease-screening tools such as accelerometers, pedometers, appetite monitors, feed consumption detection systems, remote temperature recording devices, radiant heat detectors, electronic stethoscopes, and thoracic ultrasound are validated, timely diagnosis of respiratory disease can be facilitated using a standardized scoring system. We have developed a scoring system that attributes severity scores to each of four clinical parameters; rectal temperature, cough, nasal discharge, ocular discharge or ear position. A total respiratory score of five points or higher (provided that at least two abnormal parameters are observed) can be used to distinguish affected from unaffected calves. This can be applied as a screening tool twice-weekly to identify pre-weaned calves with respiratory disease thereby facilitating early detection. Coupled with effective treatment protocols, this scoring system will reduce post-weaning pneumonia, chronic pneumonia, and otitis media.

Anthropometric growth study of the ear in a Chinese population.

PubMed

Zhao, Shichun; Li, Dianguo; Liu, Zhenzhong; Wang, Yibiao; Liu, Lei; Jiang, Duyin; Pan, Bo

2018-04-01

A large number of anthropometric studies of the auricle have been reported in different nations, but little data were available in the Chinese population. The aim of this study was to analyze growth changes in the ear by measuring the width and length of ears in a Chinese population. A total of 480 participants were enrolled and classified into 1-, 3-, 5-, 7-, 9-, 12-, 14-, and 18-year groups (half were boys and half were girls in each group). Ear length, ear width, body weight, and body length were measured and recorded; ear index was calculated according to ear length and ear width. The growth of auricle and differences between genders were analyzed. Growth of ear in relation to body height and weight and the degree of emphasis on the length and width of the auricle were also analyzed. Ear length and width increased with age. Ear length achieved its mature size in both 14-year-old males and females. Ear width reached its mature size in males at 7 years and in females at 5 years. Different trends of ear index were shown between males and females. People in this population paid more attention to the length than the width of the auricle. The data indicated that ear development followed increase in age. There were gender and ethnic difference in the development of ear. These results may have potential implications for the diagnosis of congenital malformations, syndromes, and planning of ear reconstruction surgery. Copyright © 2017 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.

Non-surgical Management of Congenital Auricular Deformities

PubMed Central

Mohammadi, Ali Akbar; Imani, Mohammad Taghi; Kardeh, Sina; Karami, Mehrab Mohammad; Kherad, Masoomeh

2016-01-01

BACKGROUND Unlike congenital auricular malformations which are identified by underdevelopment of dermal and cartilaginous tissues, deformed ears are less sever congenital anomalies characterized only by a misshaped pinna structure and can be improved with acceptable cosmetic results and minimal cost through ear molding if treated in early neonatal period. In this study, authors present the first report of using splinting techniques for treatment of deformational auricular anomalies in Iranian children. METHODS Our case load consisted of a series of 29 patients (Male=16, Female=13) who were referred to Plastic Surgery Unit of Shiraz University of Medical Sciences from September 2011 to December 2014. Children aged more than 6 moths were excluded. Twenty-nine children affected by various deformities including prominent ears (n=11), lop ears (n=8) and constricted ears (n=10) were treated by splintage as a nonsurgical technique. The mean time of treatment was 13.33±2 weeks. RESULTS Eight (27.6%) patients did not complete the treatment. Splinting resulted in excellent or satisfactory results in 12 (57.14%) of treated cases. No improvement was observed at the end of the molding treatment in 9 patients. No complication was observed during the treatment in any of the patients. CONCLUSION The nonsurgical molding can be used as an effective approach for achieving natural outcomes and correcting cosmetic abnormalities. Rate of satisfaction is dependent on type of deformity, the neonatal age in which treatment started and also parents’ adherence to treatment methods and principals. Concerning the low rate of complications and high satisfactory results the method can be used instead of surgery in appropriate cases. PMID:27579269

Non-surgical Management of Congenital Auricular Deformities.

PubMed

Mohammadi, Ali Akbar; Imani, Mohammad Taghi; Kardeh, Sina; Karami, Mehrab Mohammad; Kherad, Masoomeh

2016-05-01

Unlike congenital auricular malformations which are identified by underdevelopment of dermal and cartilaginous tissues, deformed ears are less sever congenital anomalies characterized only by a misshaped pinna structure and can be improved with acceptable cosmetic results and minimal cost through ear molding if treated in early neonatal period. In this study, authors present the first report of using splinting techniques for treatment of deformational auricular anomalies in Iranian children. Our case load consisted of a series of 29 patients (Male=16, Female=13) who were referred to Plastic Surgery Unit of Shiraz University of Medical Sciences from September 2011 to December 2014. Children aged more than 6 moths were excluded. Twenty-nine children affected by various deformities including prominent ears (n=11), lop ears (n=8) and constricted ears (n=10) were treated by splintage as a nonsurgical technique. The mean time of treatment was 13.33±2 weeks. Eight (27.6%) patients did not complete the treatment. Splinting resulted in excellent or satisfactory results in 12 (57.14%) of treated cases. No improvement was observed at the end of the molding treatment in 9 patients. No complication was observed during the treatment in any of the patients. The nonsurgical molding can be used as an effective approach for achieving natural outcomes and correcting cosmetic abnormalities. Rate of satisfaction is dependent on type of deformity, the neonatal age in which treatment started and also parents' adherence to treatment methods and principals. Concerning the low rate of complications and high satisfactory results the method can be used instead of surgery in appropriate cases.

Intractable depression successfully treated with a combination of autogenic training and high-dose antidepressant in department of otorhinolaryngology: a case report

PubMed Central

2009-01-01

Introduction Patients suffering from ear discomfort are commonly encountered in the department of otolaryngology. If various clinical examinations do not reveal any objective findings, then the patients are referred to the department of internal medicine or psychiatry. Psychotherapy is recommended in some cases. This paper describes the successful administration of autogenic training in a patient suffering from ear discomfort due to major depression. Case presentation We present a case of intractable depression that was successfully treated with a combination of psychotherapy, administered by a clinical psychologist, and high-dose antidepressant. The patient was a 36-year-old female with hearing discomfort in her left ear. In 2003, she experienced insomnia and an appetite loss, and her condition was diagnosed as major depression along with an avoidant personality disorder. Her depression has not been improved with antidepressant treatment for 3 years in department of psychosomatic medicine. She was referred to our department because of ear discomfort in her left ear. There was no abnormality in her physical examinations. She wanted to be treated in department of otorhinolaryngology. We increased the dose of fluvoxamine maleate up to 200 mg/day, and introduced cognitive therapy and autogenic training by a clinical psychologist. Eventually, her depressive state as well as the hearing complaint was markedly alleviated. Conclusion Autogenic training can be a viable and acceptable treatment option for patients who fail to respond to other therapies. This case emphasizes the importance of autogenic training as a method to control physical symptom of depression. PMID:20184684

Intractable depression successfully treated with a combination of autogenic training and high-dose antidepressant in department of otorhinolaryngology: a case report.

PubMed

Goto, Fumiyuki; Nakai, Kimiko; Murakami, Masato; Ogawa, Kaoru

2009-08-14

Patients suffering from ear discomfort are commonly encountered in the department of otolaryngology. If various clinical examinations do not reveal any objective findings, then the patients are referred to the department of internal medicine or psychiatry. Psychotherapy is recommended in some cases. This paper describes the successful administration of autogenic training in a patient suffering from ear discomfort due to major depression. We present a case of intractable depression that was successfully treated with a combination of psychotherapy, administered by a clinical psychologist, and high-dose antidepressant. The patient was a 36-year-old female with hearing discomfort in her left ear. In 2003, she experienced insomnia and an appetite loss, and her condition was diagnosed as major depression along with an avoidant personality disorder. Her depression has not been improved with antidepressant treatment for 3 years in department of psychosomatic medicine. She was referred to our department because of ear discomfort in her left ear. There was no abnormality in her physical examinations. She wanted to be treated in department of otorhinolaryngology. We increased the dose of fluvoxamine maleate up to 200 mg/day, and introduced cognitive therapy and autogenic training by a clinical psychologist. Eventually, her depressive state as well as the hearing complaint was markedly alleviated. Autogenic training can be a viable and acceptable treatment option for patients who fail to respond to other therapies. This case emphasizes the importance of autogenic training as a method to control physical symptom of depression.

Predicting Lameness in Sheep Activity Using Tri-Axial Acceleration Signals

PubMed Central

Barwick, Jamie; Lamb, David; Dobos, Robin; Schneider, Derek; Welch, Mitchell; Trotter, Mark

2018-01-01

Simple Summary Monitoring livestock farmed under extensive conditions is challenging and this is particularly difficult when observing animal behaviour at an individual level. Lameness is a disease symptom that has traditionally relied on visual inspection to detect those animals with an abnormal walking pattern. More recently, accelerometer sensors have been used in other livestock industries to detect lame animals. These devices are able to record changes in activity intensity, allowing us to differentiate between a grazing, walking, and resting animal. Using these on-animal sensors, grazing, standing, walking, and lame walking were accurately detected from an ear attached sensor. With further development, this classification algorithm could be linked with an automatic livestock monitoring system to provide real time information on individual health status, something that is practically not possible under current extensive livestock production systems. Abstract Lameness is a clinical symptom associated with a number of sheep diseases around the world, having adverse effects on weight gain, fertility, and lamb birth weight, and increasing the risk of secondary diseases. Current methods to identify lame animals rely on labour intensive visual inspection. The aim of this current study was to determine the ability of a collar, leg, and ear attached tri-axial accelerometer to discriminate between sound and lame gait movement in sheep. Data were separated into 10 s mutually exclusive behaviour epochs and subjected to Quadratic Discriminant Analysis (QDA). Initial analysis showed the high misclassification of lame grazing events with sound grazing and standing from all deployment modes. The final classification model, which included lame walking and all sound activity classes, yielded a prediction accuracy for lame locomotion of 82%, 35%, and 87% for the ear, collar, and leg deployments, respectively. Misclassification of sound walking with lame walking within the leg accelerometer dataset highlights the superiority of an ear mode of attachment for the classification of lame gait characteristics based on time series accelerometer data. PMID:29324700

Development of an ear cap in chronic suppurative otitis media using additive manufacturing and TRIZ.

PubMed

Mawale, Mahesh B; Kuthe, Abhaykumar; Mawale, Anupama M; Dahake, Sandeep W

2018-06-01

The prevalence rate of chronic suppurative otitis media is high and its treatment continues to be a challenge for the otorhinolaryngologists. Due to middle ear infection, there may be pain, hearing loss and spontaneous rupture of the eardrum which results in perforation. Infections can cause a hole in the eardrum as a side effect of otitis media. The patients suffering from ear perforation or having a hole in eardrum require preventing entry of water in the ear. This article describes the development of ear cap using additive manufacturing and TRIZ (a collaborative tool) to prevent the entry of water in the ear during chronic otitis media.

Deflation opening pressure of the eustachian tube.

PubMed

Cohen, D

1989-03-01

Measurements derived from tests of the performance of the eustachian tube (ET) under a variety of normal and pathologic conditions are widely diffuse and overlap considerably. In this survey, the deflation opening pressure (DOP) of the ET was tested in 31 patients suffering either from recurrent otitis (these patients had ventilating tubes inserted) or from chronic otitis media. Oxygen was deflated from the external ear canal, through the middle ear to the pharyngeal end of the ET. The DOP was the pressure needed for the passing of the oxygen. This pressure was usually between 100 to 200 mm H2O. No difference was found in the DOP between infants and adults or between discharging ears and dry ones. A second measurement was obtained through measuring the deflation flow pressure (DFP) required for the continuous passage of oxygen through the ET. The DFP was less than the DOP by approximately 20 to 60 mm H2O, and again no difference was noted between age groups or between infected and noninfected ears. It was concluded that DOP and DFP measurements of the ET are similar in a variety of conditions and therefore cannot indicate whether the ET is normally or abnormally functioning. The existence of a linear connection between the health of the ET and its performance is not proven; hence the role of the ET in predicting the likely outcome of tympanoplasty should be assessed within a different context.

Characterization of tinnitus in Nigeria.

PubMed

Sogebi, Olusola Ayodele

2013-08-01

This study aimed to characterize tinnitus in middle aged and elderly out-patients attending a specialized clinic in a developing country. A cross sectional study of patients attending the ear, nose and throat (ENT) clinic of Olabisi Onabanjo University Teaching Hospital, OOUTH Sagamu, Nigeria. Data was collected with the use of a structured questionnaire. Data collected included socio demographics, medical history including experience of tinnitus, PTAs, BMI and BP. Data was analyzed using SPSS version 17.0. 79 patients had complaints of tinnitus thus making a crude prevalence of 14.5%, the prevalence increased steadily along the age groups. 51.9% of patients experienced tinnitus for a short period. 53.2% of the patients had symptoms referable to only one ear, while 54.4% had discrete as opposed to multiple types of tinnitus. Occurrence of intermittent symptoms was experienced by 75.9% of the patients and 70.9% were non-pulsatile in nature. Tinnitus was significantly associated with abnormal audiographic pattern, global increased hearing thresholds, high tone hearing loss, vertigo, hypertension and obesity. Tinnitus character was majorly short term, unilateral, discrete, intermittent, and non-pulsatile in nature, and it is associated with otological, audiological, anthropometric and cardiovascular anomalies. The characteristics of tinnitus in Nigerian patients were similar to those described in developed countries, but the major risk factors for tinnitus except hearing impairment, may be different from the latter. Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.

[Research progress of functional magnetic resonance imaging in mechanism studies of tinnitus].

PubMed

Ji, B B; Li, M; Zhang, J N

2018-02-07

Tinnitus is a subjective symptom of phantom sound in the ear or brain without sound or electrical stimulation in the environment. The mechanism of tinnitus is complicated and mostly unclear. Recent studies suggested that the abnormal peripheral auditory input lead to neuroplasticity changes in central nervous system followed by tinnitus. More research concerned on the tinnitus central mechanism. A rapid development of functional magnetic resonance imaging (fMRI) technique made it more widely used in tinnitus central mechanism research. fMRI brought new findings but also presented some shortages in technology and cognition in tinnitus study. This article summarized the outcomes of fMRI research on tinnitus in recent years, exploring its existing problems and application prospects.

Developmental evolutionary biology of the vertebrate ear: conserving mechanoelectric transduction and developmental pathways in diverging morphologies

NASA Technical Reports Server (NTRS)

Fritzsch, B.; Beisel, K. W.; Bermingham, N. A.

2000-01-01

This brief overview shows that a start has been made to molecularly dissect vertebrate ear development and its evolutionary conservation to the development of the insect hearing organ. However, neither the patterning process of the ear nor the patterning process of insect sensory organs is sufficiently known at the moment to provide more than a first glimpse. Moreover, hardly anything is known about otocyst development of the cephalopod molluscs, another triploblast lineage that evolved complex 'ears'. We hope that the apparent conserved functional and cellular components present in the ciliated sensory neurons/hair cells will also be found in the genes required for vertebrate ear and insect sensory organ morphogenesis (Fig. 3). Likewise, we expect that homologous pre-patterning genes will soon be identified for the non-sensory cell development, which is more than a blocking of neuronal development through the Delta/Notch signaling system. Generation of the apparently unique ear could thus represent a multiplication of non-sensory cells by asymmetric and symmetric divisions as well as modification of existing patterning process by implementing novel developmental modules. In the final analysis, the vertebrate ear may come about by increasing the level of gene interactions in an already existing and highly conserved interactive cascade of bHLH genes. Since this was apparently achieved in all three lineages of triploblasts independently (Fig. 3), we now need to understand how much of the morphogenetic cascades are equally conserved across phyla to generate complex ears. The existing mutations in humans and mice may be able to point the direction of future research to understand the development of specific cell types and morphologies in the formation of complex arthropod, cephalopod, and vertebrate 'ears'.

Development of a morphing technique for predicting the position and size of an artificial ear in hemifacial microsomia patients.

PubMed

Coward, Trevor J; Richards, Robin; Scott, Brendan J J

2014-01-01

People with hemifacial microsomia may be missing an ear on the affected side of the face. The principal aim of the study was to develop a morphing technique and to determine whether it could be used to appropriately position an artificial ear, as well as to give an indication of prosthesis size in comparison with the natural ear. Comparisons also were made between the artificial ears being worn by the patients with their natural ears. Data from stereophotogrammetry images of the faces of 10 people were converted into stereolithographic format. Anthropometric points on the face and ear of the unaffected side were plotted. By a process of scaling, the distance between facial landmarks on the unaffected side was estimated for the affected side so as to identify where the morphed ear would be positioned once generated. Generally, the morphed ears appeared to be in acceptable positions. There was a statistically significant difference between the position of the morphed and natural ears (P = .011), as well as the artificial and natural ears (P = .001), but this was unlikely to have any clinical implications. There were no significant differences among the sizes of the natural, morphed, and artificial ears (P = .072). Morphing appears to offer a more precise way of planning the positioning and construction of an artificial ear on patients with hemifacial microsomia than traditional methods. Differences in facial shape on either side of the face may impact on the process. This requires further study.

Balance disorders in the elderly and the benefit of balance exercise.

PubMed

Prasansuk, Suchitra; Siriyananda, Chana; Nakorn, Apinan Na; Atipas, Suvajana; Chongvisal, Samut

2004-10-01

Symptoms of balance disorders including 'unsteadiness', 'dizziness and vertigo' are common in the elderly and commonly found in general practice in medicine. There are many causes of balance disorders and vary from one person to another. Disorder of the internal ear or vestibular end-organ type is one cause. Unsteadiness of somato-sensory or proprioception is common in the elderly so is degenerative disorder of central control in brain. The elderly are prone to many chronic illnesses or disorders which are causes of balance disorder or give rise to more rapid degeneration of the central nervous system i.e. high blood pressure, diabetes mellitus, heart disease, proprioception and joint problems, arthritis and muscular weakness due to lack of good health and exercise. The objectives of this research study were to find the etiologies of balance disorders and how Balance Exercises and the 'National Health Service' can be of benefit in helping to prevent them. 1565 elderly inhabitants (age > or = 60 years) of 20 communities adjacent to Siriraj Hospital were selected for study. Among these, 625 persons had a history of balance disorders. Among those, 256 had symptoms during the week selected for examination. The average age was 66 years old, women outnumbered men with a ratio of 2.4:1. The common underlying causes were hypertension in 32.4%, diabetes mellitus 13.8%, arthritis 8.1%, and heart disease 4.4% respectively. All are still taking one or more types of drug. The subjects were randomly divided into two groups for the study purpose of effectiveness of balance exercise. Group 1 did not perform the head balance exercise and Group 2 performed the head balance exercise. Audiometric testing showed impaired hearing in 90% of the subjects. The majority slowed hearing loss in high frequencies. Testing of middle ear function found 75% of Group 1 to have normal middle ear function 77% of Group 2. Brainstem Electrical Response Audiometry (BERA) showed normal response latencies of in 96% of group 1 and 94% of Group 2. Poor morphology of waveform was found in 12% of Group 1 and 16% of Group 2. Doppler sonography for intra-cranial blood flow measurement showed abnormal flow of the ICA in 17.6% of group 1 and 20.16% of group 2. Basilar arterial abnormal flow was found in 77.6% of Group 1 and 80.6% of Group 2 respectively. The flow of ICA was improved after 8 weeks in both groups. The measurement of balance by Posturography showed 86.7% abnormality in Group 1 and 83.5% abnormality in Group 2 (and the majority due to inner ear problems but many cases had a mixture of joints and CNS problems too). Results of the self-evaluation (by questionaires) showed the elderly to have symptoms of light headedness in 51% and loss of balance in 29%, Vertigo with rotation occurred in 23.6%. 49% of the symptoms were intermittent, 56.4% experienced a 'fall". On questioning about the benefit and performance of Head and Neck Exercises, 82.8% found the exercises were easy to perform, 56.4% said the results were very beneficial.

The maize rachis affects Aspergillus flavus movement during ear development

USDA-ARS?s Scientific Manuscript database

Aspergillus flavus expressing green fluorescent protein (GFP) was used to follow infection in ears of maize hybrids resistant and susceptible to the fungus. Developing ears were needle-inoculated with GFP-transformed A. flavus 20 days after silk emergence, and GFP fluorescence in the pith was evalu...

Responses of the ear to low frequency sounds, infrasound and wind turbines

PubMed Central

Salt, Alec N.; Hullar, Timothy E.

2010-01-01

Infrasonic sounds are generated internally in the body (by respiration, heartbeat, coughing, etc) and by external sources, such as air conditioning systems, inside vehicles, some industrial processes and, now becoming increasingly prevalent, wind turbines. It is widely assumed that infrasound presented at an amplitude below what is audible has no influence on the ear. In this review, we consider possible ways that low frequency sounds, at levels that may or may not be heard, could influence the function of the ear. The inner ear has elaborate mechanisms to attenuate low frequency sound components before they are transmitted to the brain. The auditory portion of the ear, the cochlea, has two types of sensory cells, inner hair cells (IHC) and outer hair cells (OHC), of which the IHC are coupled to the afferent fibers that transmit “hearing” to the brain. The sensory stereocilia (“hairs”) on the IHC are “fluid coupled” to mechanical stimuli, so their responses depend on stimulus velocity and their sensitivity decreases as sound frequency is lowered. In contrast, the OHC are directly coupled to mechanical stimuli, so their input remains greater than for IHC at low frequencies. At very low frequencies the OHC are stimulated by sounds at levels below those that are heard. Although the hair cells in other sensory structures such as the saccule may be tuned to infrasonic frequencies, auditory stimulus coupling to these structures is inefficient so that they are unlikely to be influenced by airborne infrasound. Structures that are involved in endolymph volume regulation are also known to be influenced by infrasound, but their sensitivity is also thought to be low. There are, however, abnormal states in which the ear becomes hypersensitive to infrasound. In most cases, the inner ear’s responses to infrasound can be considered normal, but they could be associated with unfamiliar sensations or subtle changes in physiology. This raises the possibility that exposure to the infrasound component of wind turbine noise could influence the physiology of the ear. PMID:20561575

The Severity of Vestibular Dysfunction in Deafness as a Determinant of Comorbid Hyperactivity or Anxiety

PubMed Central

McKeehan, Nicholas

2017-01-01

Attention-deficit/hyperactivity disorder (ADHD) and anxiety-related disorders occur at rates 2–3 times higher in deaf compared with hearing children. Potential explanations for these elevated rates and the heterogeneity of behavioral disorders associated with deafness have usually focused on socio-environmental rather than biological effects. Children with the 22q11.2 deletion or duplication syndromes often display hearing loss and behavioral disorders, including ADHD and anxiety-related disorders. Here, we show that mouse mutants with either a gain or loss of function of the T-Box transcription factor gene, Tbx1, which lies within the 22q11.2 region and is responsible for most of the syndromic defects, exhibit inner ear defects and hyperactivity. Furthermore, we show that (1) inner ear dysfunction due to the tissue-specific loss of Tbx1 or Slc12a2, which encodes a sodium-potassium-chloride cotransporter and is also necessary for inner ear function, causes hyperactivity; (2) vestibular rather than auditory failure causes hyperactivity; and (3) the severity rather than the age of onset of vestibular dysfunction differentiates whether hyperactivity or anxiety co-occurs with inner ear dysfunction. Together, these findings highlight a biological link between inner ear dysfunction and behavioral disorders and how sensory abnormalities can contribute to the etiology of disorders traditionally considered of cerebral origin. SIGNIFICANCE STATEMENT This study examines the biological rather than socio-environmental reasons why hyperactivity and anxiety disorders occur at higher rates in deaf individuals. Using conditional genetic approaches in mice, the authors show that (1) inner ear dysfunction due to either Tbx1 or Slc12a2 mutations cause hyperactivity; (2) it is vestibular dysfunction, which frequently co-occurs with deafness but often remains undiagnosed, rather than auditory dysfunction that causes hyperactivity and anxiety-related symptoms; and (3) the severity of vestibular dysfunction can predict whether hyperactivity or anxiety coexist with inner ear dysfunction. These findings suggest a need to evaluate vestibular function in hearing impaired individuals, especially those who exhibit hyperactive and anxiety-related symptoms. PMID:28438970

The Severity of Vestibular Dysfunction in Deafness as a Determinant of Comorbid Hyperactivity or Anxiety.

PubMed

Antoine, Michelle W; Vijayakumar, Sarath; McKeehan, Nicholas; Jones, Sherri M; Hébert, Jean M

2017-05-17

Attention-deficit/hyperactivity disorder (ADHD) and anxiety-related disorders occur at rates 2-3 times higher in deaf compared with hearing children. Potential explanations for these elevated rates and the heterogeneity of behavioral disorders associated with deafness have usually focused on socio-environmental rather than biological effects. Children with the 22q11.2 deletion or duplication syndromes often display hearing loss and behavioral disorders, including ADHD and anxiety-related disorders. Here, we show that mouse mutants with either a gain or loss of function of the T-Box transcription factor gene, Tbx1 , which lies within the 22q11.2 region and is responsible for most of the syndromic defects, exhibit inner ear defects and hyperactivity. Furthermore, we show that (1) inner ear dysfunction due to the tissue-specific loss of Tbx1 or Slc12a2 , which encodes a sodium-potassium-chloride cotransporter and is also necessary for inner ear function, causes hyperactivity; (2) vestibular rather than auditory failure causes hyperactivity; and (3) the severity rather than the age of onset of vestibular dysfunction differentiates whether hyperactivity or anxiety co-occurs with inner ear dysfunction. Together, these findings highlight a biological link between inner ear dysfunction and behavioral disorders and how sensory abnormalities can contribute to the etiology of disorders traditionally considered of cerebral origin. SIGNIFICANCE STATEMENT This study examines the biological rather than socio-environmental reasons why hyperactivity and anxiety disorders occur at higher rates in deaf individuals. Using conditional genetic approaches in mice, the authors show that (1) inner ear dysfunction due to either Tbx1 or Slc12a2 mutations cause hyperactivity; (2) it is vestibular dysfunction, which frequently co-occurs with deafness but often remains undiagnosed, rather than auditory dysfunction that causes hyperactivity and anxiety-related symptoms; and (3) the severity of vestibular dysfunction can predict whether hyperactivity or anxiety coexist with inner ear dysfunction. These findings suggest a need to evaluate vestibular function in hearing impaired individuals, especially those who exhibit hyperactive and anxiety-related symptoms. Copyright © 2017 the authors 0270-6474/17/375144-11$15.00/0.

[Effects on hearing due to the occupational noise exposure of marble industry workers in the Federal District, Brazil].

PubMed

Harger, Marília Rabelo Holanda Camarano; Barbosa-Branco, Anadergh

2004-01-01

To evaluate the prevalence of hearing loss, its degree and type, among workers in the marble industry in the Brazilian Federal District (FD). Workers from eight marble industries in the FD were evaluated by means of a cross sectional epidemiological study. An audiometry screening test (air conduction) was performed. Workers with hearing loss were submitted to liminal tonal audiometry air & bone conduction and speech audiometry tests using an audiometer AD-28 (Interacoustics). All subjects studied were submitted to a visual inspection of the external acoustic meatus. One hundred and fifty two workers were examined; mean age was 32 years (SD = 8.6); average occupational noise exposure was of 8.3 years (SD = 6.8). Audiometries demonstrated that 48.0% (n = 73) had some type of hearing loss. Among the workers with hearing loss, 50.0% had results compatible with noise-induced hearing loss (NIHL); 41.0% with incipient noise induced hearing loss, 5.0% with sensorineural hearing loss (all except NIHL) and 4.0% with conductive and mixed hearing losses. Among workers with NIHL, 57.1% had bilateral involvement, 17.1% in the right ear and 25.7% in the left ear. Among those with incipient NIHL, 13.9% were bilateral, 19.4% were only in the right ear and 66.7% were only in the left ear. Abnormal audiograms were found in 48.0% of the sample. Among those with hearing loss, the predominant cause was NIHL, followed by those classified as having incipient noise induced hearing loss. Hearing loss usually started at 6 kHz, frequently in the left ear.

Fly-ear inspired acoustic sensors for gunshot localization

NASA Astrophysics Data System (ADS)

Liu, Haijun; Currano, Luke; Gee, Danny; Yang, Benjamin; Yu, Miao

2009-05-01

The supersensitive ears of the parasitoid fly Ormia ochracea have inspired researchers to develop bio-inspired directional microphone for sound localization. Although the fly ear is optimized for localizing the narrow-band calling song of crickets at 5 kHz, experiments and simulation have shown that it can amplify directional cues for a wide frequency range. In this article, a theoretical investigation is presented to study the use of fly-ear inspired directional microphones for gunshot localization. Using an equivalent 2-DOF model of the fly ear, the time responses of the fly ear structure to a typical shock wave are obtained and the associated time delay is estimated by using cross-correlation. Both near-field and far-field scenarios are considered. The simulation shows that the fly ear can greatly amplify the time delay by ~20 times, which indicates that with an interaural distance of only 1.2 mm the fly ear is able to generate a time delay comparable to that obtained by a conventional microphone pair with a separation as large as 24 mm. Since the parameters of the fly ear structure can also be tuned for muzzle blast and other impulse stimulus, fly-ear inspired acoustic sensors offers great potential for developing portable gunshot localization systems.

Stem Cell Therapy for the Inner Ear

PubMed Central

Okano, Takayuki

2012-01-01

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

Salivary glands abnormalities in oculo-auriculo-vertebral spectrum.

PubMed

Brotto, Davide; Manara, Renzo; Vio, Stefania; Ghiselli, Sara; Cantone, Elena; Mardari, Rodica; Toldo, Irene; Stritoni, Valentina; Castiglione, Alessandro; Lovo, Elisa; Trevisi, Patrizia; Bovo, Roberto; Martini, Alessandro

2018-01-01

Feeding and swallowing impairment are present in up to 80% of oculo-auriculo-vertebral spectrum (OAVS) patients. Salivary gland abnormalities have been reported in OAVS patients but their rate, features, and relationship with phenotype severity have yet to be defined. Parotid and submandibular salivary gland hypo/aplasia was evaluated on head MRI of 25 OAVS patients (16 with severe phenotype, Goldenhar syndrome) and 11 controls. All controls disclosed normal salivary glands. Abnormal parotid glands were found exclusively ipsilateral to facial microsomia in 21/25 OAVS patients (84%, aplasia in six patients) and showed no association with phenotype severity (14/16 patients with Goldenhar phenotype vs 7/9 patients with milder phenotype, p = 0.6). Submandibular salivary gland hypoplasia was detected in six OAVS patients, all with concomitant ipsilateral severe involvement of the parotid gland (p < 0.001). Submandibular salivary gland hypoplasia was associated to Goldenhar phenotype (p < 0.05). Parotid gland abnormalities were associated with ipsilateral fifth (p < 0.001) and seventh cranial nerve (p = 0.001) abnormalities. No association was found between parotid gland anomaly and ipsilateral internal carotid artery, inner ear, brain, eye, or spine abnormalities (p > 0.6). Salivary gland abnormalities are strikingly common in OAVS. Their detection might help the management of OAVS-associated swallowing and feeding impairment.

Do the angle and length of the eustachian tube influence the development of chronic otitis media?

PubMed

Dinç, Aykut Erdem; Damar, Murat; Uğur, Mehmet Birol; Öz, Ibrahim Ilker; Eliçora, Sultan Şevik; Bişkin, Sultan; Tutar, Hakan

2015-09-01

To compare the eustachian tube (ET) angle (ETa) and length (ETl) of ears with and without chronic otitis media (COM), and to determine the relationship between ET anatomy and the development of COM. A retrospective case-control study. The study group comprised 125 patients (age range, 8-79 years; 64 males and 61 females) with 124 normal ears and 126 diseased ears, including ears with chronic suppurative otitis media (CSOM) with central perforation, intratympanic tympanosclerosis (ITTS), cholesteatoma, and a tympanic membrane with retraction pockets (TMRP). ET angle and length were measured using computed tomography employing the multiplanar reconstruction technique. The ETa was significantly more horizontal in diseased versus normal ears of all study groups (P = .030), and there was no group difference in ETl (P = .160). ETl was shorter in CSOM versus ITTS ears and normal ears (P = .007 and P = .003, respectively) and in cholesteatoma versus TMRP ears (P = .014). In the unilateral COM group, there were no significant differences in the ETa or ETl of diseased versus contralateral normal ears (P = .155 and P = .710, respectively). The ETa was significantly more horizontal in childhood-onset diseased versus normal ears (P = .027), and there was no group difference in ETl (P = .732). The ETa (P = .002) and ETl (P < .001) were significantly greater in males than females. A more horizontal ETa and shorter ETl could be contributory (though not significantly) etiological factors in the development of COM. 3b. © 2015 The American Laryngological, Rhinological and Otological Society, Inc.

Effect of dietary inclusion of whole ear corn silage on stomach development and gastric mucosa integrity of heavy pigs at slaughter.

PubMed

Mason, Federico; Pascotto, Ernesto; Zanfi, Cristina; Spanghero, Mauro

2013-12-01

The effect of dietary inclusion of whole ear corn silage on stomach development and on the incidence of gastric lesions was studied in heavy pigs. Three groups of 14 castrated male pigs were fed a control cereal-based diet and two diets containing whole ear corn silage (15% or 30% DM) from 90 kg bodyweight to slaughter at 170 kg. The diets with whole ear corn silage increased the amount of neutral detergent fibre in the stomach contents, the weight of the organs and the area of the pyloric region. Follicular gastritis was significantly lower and gastritis less severe in pigs fed the whole ear corn silage diets than pigs fed the control diet. The inclusion of whole ear corn silage in the diet influenced the development of the stomach and reduced the incidence of gastritis in heavy pigs. Copyright © 2013 Elsevier Ltd. All rights reserved.

[Ear, nose and throat disease profile in children with Down syndrome].

PubMed

Paul D, María A; Bravo V, Alejandra; Beltrán M, Constanza; Cerda L, Jaime; Angulo M, Daniela; Lizama C, Macarena

2015-01-01

The children with Down syndrome (DS) are at increased risk of ear-nose-throat (ENT) disorders. International recommendations suggest early hearing screening and periodic specialist evaluation. Our goal was to characterize ENT disorders in children with DS, and propose recommendations for the Chilean population. Cross-sectional, descriptive study, of children with DS, between 6 months and 15 years of age. The data was obtained by a health interview to the parents and review of medical records. We analyzed 134 patients with an average age of 44.5 months. The 78.8% had ENT disorders, the most frequent ENT disorders was allergic rhinitis and otitis media with effusion. Hearing screening was abnormal in a quarter of the patients, 50% of children over 3 years of age had obstructive sleep apnea diagnosed by polysomnogram. Older children had a statistically higher frequency of ENT disorders. This series shows a high rate of ENT disorders in children with DS, which supports recommendations for hearing screening, high suspicion of obstructive sleep apnea and routine referral to an ENT specialist for prevention and aggressive therapy in order to reduce hearing loss and improve development of the child with DS. Copyright © 2015 Sociedad Chilena de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.

Development, survival and fitness performance of Helicoverpa zea (Lepidoptera: Noctuidae) in MON810 Bt field corn.

PubMed

Horner, T A; Dively, G P; Herbert, D A

2003-06-01

Helicoverpa zea (Boddie) development, survival, and feeding injury in MON810 transgenic ears of field corn (Zea mays L.) expressing Bacillus thuringiensis variety kurstaki (Bt) Cry1Ab endotoxins were compared with non-Bt ears at four geographic locations over two growing seasons. Expression of Cry1Ab endotoxin resulted in overall reductions in the percentage of damaged ears by 33% and in the amount of kernels consumed by 60%. Bt-induced effects varied significantly among locations, partly because of the overall level and timing of H. zea infestations, condition of silk tissue at the time of egg hatch, and the possible effects of plant stress. Larvae feeding on Bt ears produced scattered, discontinuous patches of partially consumed kernels, which were arranged more linearly than the compact feeding patterns in non-Bt ears. The feeding patterns suggest that larvae in Bt ears are moving about sampling kernels more frequently than larvae in non-Bt ears. Because not all kernels express the same level of endotoxin, the spatial heterogeneity of toxin distribution within Bt ears may provide an opportunity for development of behavioral responses in H. zea to avoid toxin. MON810 corn suppressed the establishment and development of H. zea to late instars by at least 75%. This level of control is considered a moderate dose, which may increase the risk of resistance development in areas where MON810 corn is widely adopted and H. zea overwinters successfully. Sublethal effects of MON810 corn resulted in prolonged larval and prepupal development, smaller pupae, and reduced fecundity of H. zea. The moderate dose effects and the spatial heterogeneity of toxin distribution among kernels could increase the additive genetic variance for both physiological and behavioral resistance in H. zea populations. Implications of localized population suppression are discussed.

[An ear thermometer based on infrared thermopiles sensor].

PubMed

Xie, Haiyuan; Qian, Mingli

2013-09-01

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

Partial duplication of chromosome 19 associated with syndromic duane retraction syndrome.

PubMed

Abu-Amero, Khaled K; Kondkar, Altaf A; Al Otaibi, Abdullah; Alorainy, Ibrahim A; Khan, Arif O; Hellani, Ali M; Oystreck, Darren T; Bosley, Thomas M

2015-03-01

To evaluate possible monogenic and chromosomal anomalies in a patient with unilateral Duane retraction syndrome, modest dysmorphism, cerebral white matter abnormalities, and normal cognitive function. Performing high-resolution array comparative genomic hybridization (array CGH) and sequencing of HOXA1, KIF21A, SALL4, and CHN1 genes. The proband had unilateral Duane retraction syndrome (DRS) type III on the right with low-set ears, prominent forehead, clinodactyly, and a history of frequent infections during early childhood. Motor development and cognitive function were normal. Parents were not related, and no other family member was similarly affected. MRI revealed multiple small areas of high signal on T2 weighted images in cerebral white matter oriented along white matter tracts. Sequencing of HOXA1, KIF21A, SALL4, and CHN1 did not reveal any mutation(s). Array CGH showed a 95 Kb de novo duplication on chromosome 19q13.4 encompassing four killer cell immunoglobulin-like receptor (KIR) genes. Conclusions. KIR genes have not previously been linked to a developmental syndrome, although they are known to be expressed in the human brain and brainstem and to be associated with certain infections and autoimmune diseases, including some affecting the nervous system. DRS and brain neuroimaging abnormalities may imply a central and peripheral oligodendrocyte abnormality related in some fashion to an immunomodulatory disturbance.

Analysis of Saccular Function With Vestibular Evoked Myogenic Potential Test in Meniere's Disease.

PubMed

Dabiri, Sasan; Yazdani, Nasrin; Esfahani, Mahdis; Tari, Niloufar; Adil, Susan; Mahvi, Zahra; Rezazadeh, Nima

2017-02-01

Meniere's disease is the disorder of inner ear characterized by vertigo, tinnitus and sensorineural hearing loss. The vestibular evoked myogenic potential (VEMP) test could be useful in the analysis of saccular function, and diagnosis of Meniere's disease. In this study, we've analyzed the saccular function, using VEMP test in different groups of Meniere's disease. Patients were categorized as possible, probable or definite Meniere's disease groups according to the guideline of American Academy of Otolaryngology-Head and Neck Surgery. The exclusion criteria were neuromuscular system diseases, diseases of central nervous system, inner ear disorders, conductive hearing loss, a history of ototoxic drug consumption, being a drug abuser and a positive history of inner ear surgery or manipulations. The VEMP test is the recording of positive and negative waves from sternocleidomastoid muscle that is made by an auditory click to the ear. From the total of 100 patients, the waves of VEMP test was seen in 59 patients which 19 patients had abnormal amplitude, and latency and 40 patients were with normally recorded waves. There was a significant relationship between the severity of hearing loss and a VEMP test without any recorded waves. Most of the cases with 'no wave recorded' VEMP test, were patients with severe hearing loss. However, there wasn't any relation between the pattern of hearing loss and 'no wave recorded' VEMP test. VEMP test could be a valuable diagnostic clue especially in patients with definite Meniere's disease.

Inner Ear Drug Delivery for Auditory Applications

PubMed Central

Swan, Erin E. Leary; Mescher, Mark J.; Sewell, William F.; Tao, Sarah L.; Borenstein, Jeffrey T.

2008-01-01

Many inner ear disorders cannot be adequately treated by systemic drug delivery. A blood-cochlear barrier exists, similar physiologically to the blood-brain barrier, which limits the concentration and size of molecules able to leave the circulation and gain access to the cells of the inner ear. However, research in novel therapeutics and delivery systems has led to significant progress in the development of local methods of drug delivery to the inner ear. Intratympanic approaches, which deliver therapeutics to the middle ear, rely on permeation through tissue for access to the structures of the inner ear, whereas intracochlear methods are able to directly insert drugs into the inner ear. Innovative drug delivery systems to treat various inner ear ailments such as ototoxicity, sudden sensorineural hearing loss, autoimmune inner ear disease, and for preserving neurons and regenerating sensory cells are being explored. PMID:18848590

Left and right reaction time differences to the sound intensity in normal and AD/HD children.

PubMed

Baghdadi, Golnaz; Towhidkhah, Farzad; Rostami, Reza

2017-06-01

Right hemisphere, which is attributed to the sound intensity discrimination, has abnormality in people with attention deficit/hyperactivity disorder (AD/HD). However, it is not studied whether the defect in the right hemisphere has influenced on the intensity sensation of AD/HD subjects or not. In this study, the sensitivity of normal and AD/HD children to the sound intensity was investigated. Nineteen normal and fourteen AD/HD children participated in the study and performed a simple auditory reaction time task. Using the regression analysis, the sensitivity of right and left ears to various sound intensity levels was examined. The statistical results showed that the sensitivity of AD/HD subjects to the intensity was lower than the normal group (p < 0.0001). Left and right pathways of the auditory system had the same pattern of response in AD/HD subjects (p > 0.05). However, in control group the left pathway was more sensitive to the sound intensity level than the right one (p = 0.0156). It can be probable that the deficit of the right hemisphere has influenced on the auditory sensitivity of AD/HD children. The possible existent deficits of other auditory system components such as middle ear, inner ear, or involved brain stem nucleuses may also lead to the observed results. The development of new biomarkers based on the sensitivity of the brain hemispheres to the sound intensity has been suggested to estimate the risk of AD/HD. Designing new technique to correct the auditory feedback has been also proposed in behavioral treatment sessions. Copyright © 2017. Published by Elsevier B.V.

Generation of inner ear organoids containing functional hair cells from human pluripotent stem cells.

PubMed

Koehler, Karl R; Nie, Jing; Longworth-Mills, Emma; Liu, Xiao-Ping; Lee, Jiyoon; Holt, Jeffrey R; Hashino, Eri

2017-06-01

The derivation of human inner ear tissue from pluripotent stem cells would enable in vitro screening of drug candidates for the treatment of hearing and balance dysfunction and may provide a source of cells for cell-based therapies of the inner ear. Here we report a method for differentiating human pluripotent stem cells to inner ear organoids that harbor functional hair cells. Using a three-dimensional culture system, we modulate TGF, BMP, FGF, and WNT signaling to generate multiple otic-vesicle-like structures from a single stem-cell aggregate. Over 2 months, the vesicles develop into inner ear organoids with sensory epithelia that are innervated by sensory neurons. Additionally, using CRISPR-Cas9, we generate an ATOH1-2A-eGFP cell line to detect hair cell induction and demonstrate that derived hair cells exhibit electrophysiological properties similar to those of native sensory hair cells. Our culture system should facilitate the study of human inner ear development and research on therapies for diseases of the inner ear.

Insights into inner ear-specific gene regulation: epigenetics and non-coding RNAs in inner ear development and regeneration

PubMed Central

Avraham, Karen B.

2016-01-01

The vertebrate inner ear houses highly specialized sensory organs, tuned to detect and encode sound, head motion and gravity. Gene expression programs under the control of transcription factors orchestrate the formation and specialization of the non-sensory inner ear labyrinth and its sensory constituents. More recently, epigenetic factors and non-coding RNAs emerged as an additional layer of gene regulation, both in inner ear development and disease. In this review, we provide an overview on how epigenetic modifications and non-coding RNAs, in particular microRNAs (miRNAs), influence gene expression and summarize recent discoveries that highlight their critical role in the proper formation of the inner ear labyrinth and its sensory organs. In contrast to non-mammalian vertebrates, adult mammals lack the ability to regenerate inner ear mechano-sensory hair cells. Finally, we discuss recent insights into how epigenetic factors and miRNAs may facilitate, or in the case of mammals, restrict sensory hair cell regeneration. PMID:27836639

Recurrent syncope and chronic ear pain

PubMed Central

Clegg, Andrew; Daverede, Luis; Wong, Winson; Loney, Elizabeth; Young, John

2010-01-01

An elderly gentleman presented to hospital with recurrent blackout episodes consistent with syncope and a 3-month history of right ear pain. Significant postural hypotension was recorded. White cell count and C reactive protein were elevated. MRI of the head and neck revealed a soft tissue abnormality in the right nasopharynx and base of skull. Tissue biopsies were obtained and microbiology specimens revealed a mixed growth of pseudomonas and diphtheroids. There was no histological evidence of malignancy. A diagnosis of skull base infection was made. Infective involvement of the carotid sinus was considered to be the cause of the recurrent syncope and postural hypotension. The patient responded well to a 12-week course of intravenous meropenem. Inflammatory markers returned to normal and a repeat MRI after 3 months of treatment showed significant resolution of infection. The syncopal episodes and orthostatic hypotension resolved in parallel with treatment of infection. PMID:22791782

A laboratory silicone for preclinical training in ear prosthesis.

PubMed

Anand, Vijay; Haribabu; Vimala; Gnanasamband, Vimala

2013-07-01

This article describes an industrial elastic silicone as a material for the laboratory fabrication of ear prosthesis. It has been tested for toxicity in lab animals by the SGS India Pvt. Ltd and approved as a material to pass the parameter of abnormal toxicity. This material therefore can be safely recommended for laboratory exercise to fabricate facial prosthesis. The high cost of the maxillo facial silicone materials prohibits their use for facial prosthesis in pre-clinical training of post-graduate students in maxillofacial prosthodontics. For this reason, pre-clinical laboratory exercise in facial prosthesis is inadequate. A few institutions use polymethyl methacrylate resins which are rigid and do not have elastic characteristics of silicone, which is used for facial defects. This cost-effective industrial silicone material which mimics the elastic and color characteristics of the conventional silicones can be recommended for preclinical exercises.

Proposal of a Classification System for the Assessment and Treatment of Prominent Ear Deformity.

PubMed

Lee, Youngdae; Kim, Young Seok; Lee, Won Jai; Rha, Dong Kyun; Kim, Jiye

2018-06-01

Prominent ear is the most common external ear deformity. To comprehensively treat prominent ear deformity, adequate comprehension of its pathophysiology is crucial. In this article, we analyze cases of prominent ear and suggest a simple classification system and treatment algorithm according to pathophysiology. We retrospectively reviewed a total of 205 Northeast Asian patients' clinical data who underwent an operation for prominent ear deformity. Follow-up assessments were conducted 3, 6, and 12 months after surgery. Prominent ear deformities were classified by diagnostic checkpoints. Class I (simple prominent ear) includes prominent ear that developed with the absence of the antihelix without conchal hypertrophy. Class II (mixed-type prominent ear) is defined as having not only a flat antihelix, but also conchal excess. Class III (conchal-type prominent ear) has an enlarged conchal bowl with a well-developed antihelix. Among the three types of prominent ear, class I was most frequent (162 patients, 81.6%). Class II was observed in 28 patients (13.6%) and class III in 10 patients (4.8%). We used the scaphomastoid suture method for correction of antihelical effacement, the anterior approach conchal resection for correction of conchal hypertrophy, and Bauer's squid incision for lobule prominence. The complication rate was 9.2% including early hematoma, hypersensitivity, and suture extrusion. Unfavorable results occurred in 4% including partial recurrence, overcorrection, and undercorrection. To reduce unfavorable results and avoid recurrence, we propose the use of a classification and treatment algorithm in preoperative evaluation of prominent ear. This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Heterozygous missense variants of LMX1A lead to nonsyndromic hearing impairment and vestibular dysfunction.

PubMed

Wesdorp, Mieke; de Koning Gans, Pia A M; Schraders, Margit; Oostrik, Jaap; Huynen, Martijn A; Venselaar, Hanka; Beynon, Andy J; van Gaalen, Judith; Piai, Vitória; Voermans, Nicol; van Rossum, Michelle M; Hartel, Bas P; Lelieveld, Stefan H; Wiel, Laurens; Verbist, Berit; Rotteveel, Liselotte J; van Dooren, Marieke F; Lichtner, Peter; Kunst, Henricus P M; Feenstra, Ilse; Admiraal, Ronald J C; Yntema, Helger G; Hoefsloot, Lies H; Pennings, Ronald J E; Kremer, Hannie

2018-05-12

Unraveling the causes and pathomechanisms of progressive disorders is essential for the development of therapeutic strategies. Here, we identified heterozygous pathogenic missense variants of LMX1A in two families of Dutch origin with progressive nonsyndromic hearing impairment (HI), using whole exome sequencing. One variant, c.721G > C (p.Val241Leu), occurred de novo and is predicted to affect the homeodomain of LMX1A, which is essential for DNA binding. The second variant, c.290G > C (p.Cys97Ser), predicted to affect a zinc-binding residue of the second LIM domain that is involved in protein-protein interactions. Bi-allelic deleterious variants of Lmx1a are associated with a complex phenotype in mice, including deafness and vestibular defects, due to arrest of inner ear development. Although Lmx1a mouse mutants demonstrate neurological, skeletal, pigmentation and reproductive system abnormalities, no syndromic features were present in the participating subjects of either family. LMX1A has previously been suggested as a candidate gene for intellectual disability, but our data do not support this, as affected subjects displayed normal cognition. Large variability was observed in the age of onset (a)symmetry, severity and progression rate of HI. About half of the affected individuals displayed vestibular dysfunction and experienced symptoms thereof. The late-onset progressive phenotype and the absence of cochleovestibular malformations on computed tomography scans indicate that heterozygous defects of LMX1A do not result in severe developmental abnormalities in humans. We propose that a single LMX1A wild-type copy is sufficient for normal development but insufficient for maintenance of cochleovestibular function. Alternatively, minor cochleovestibular developmental abnormalities could eventually lead to the progressive phenotype seen in the families.

Larval western bean cutworm feeding damage encourages the development of Gibberella ear rot on field corn.

PubMed

Parker, Nicole S; Anderson, Nolan R; Richmond, Douglas S; Long, Elizabeth Y; Wise, Kiersten A; Krupke, Christian H

2017-03-01

A 2 year study was conducted to determine whether western bean cutworm (Striacosta albicosta Smith) (WBC) larval feeding damage increases severity of the fungal disease Gibberella ear rot [Fusarium graminearum (Schwein.) Petch] in field corn (Zea mays L.). The effect of a quinone-outside inhibiting fungicide, pyraclostrobin, on Gibberella ear rot severity and mycotoxin production, both with and without WBC pressure, was also evaluated. The impact of each variable was assessed individually and in combination to determine the effect of each upon ear disease severity. There was a positive correlation between the presence of WBC larvae in field corn and Gibberella ear rot severity under inoculated conditions in the 2 years of the experiment. An application of pyraclostrobin did not impact Gibberella ear rot development when applied at corn growth stage R1 (silks first emerging). Feeding damage from WBC larvae significantly increases the development of F. graminearum in field corn. We conclude that an effective integrated management strategy for Gibberella ear rot should target the insect pest first, in an effort to limit disease severity and subsequent mycotoxin production by F. graminearum in kernels. © 2016 Society of Chemical Industry. © 2016 Society of Chemical Industry.

Postganglionic horner syndrome in three patients with coincident middle ear infection.

PubMed

Spector, Robert H

2008-09-01

Three patients developed a postganglionic Horner syndrome during the course of an ipsilateral uncomplicated middle ear infection. The mechanism may be an effect on the middle ear caroticotympanic sympathetic plexus, for which there is considerable anatomic and physiologic evidence. Why Horner syndrome does not occur more often after middle ear infection is a mystery.

Shaping sound in space: the regulation of inner ear patterning.

PubMed

Groves, Andrew K; Fekete, Donna M

2012-01-01

The inner ear is one of the most morphologically elaborate tissues in vertebrates, containing a group of mechanosensitive sensory organs that mediate hearing and balance. These organs are arranged precisely in space and contain intricately patterned sensory epithelia. Here, we review recent studies of inner ear development and patterning which reveal that multiple stages of ear development - ranging from its early induction from the embryonic ectoderm to the establishment of the three cardinal axes and the fine-grained arrangement of sensory cells - are orchestrated by gradients of signaling molecules.

Shaping sound in space: the regulation of inner ear patterning

PubMed Central

Groves, Andrew K.; Fekete, Donna M.

2012-01-01

The inner ear is one of the most morphologically elaborate tissues in vertebrates, containing a group of mechanosensitive sensory organs that mediate hearing and balance. These organs are arranged precisely in space and contain intricately patterned sensory epithelia. Here, we review recent studies of inner ear development and patterning which reveal that multiple stages of ear development – ranging from its early induction from the embryonic ectoderm to the establishment of the three cardinal axes and the fine-grained arrangement of sensory cells – are orchestrated by gradients of signaling molecules. PMID:22186725

Prevalence of Semicircular Canal Hypoplasia in Patients With CHARGE Syndrome: 3C Syndrome.

PubMed

Wineland, Andre; Menezes, Maithilee D; Shimony, Joshua S; Shinawi, Marwan S; Hullar, Timothy E; Hirose, Keiko

2017-02-01

CHARGE syndrome refers to a syndrome involving coloboma, heart defects, atresia choanae, retardation of growth and development, genitourinary disorders, and ear anomalies. However, Verloes revised the characteristics of CHARGE syndrome in 2005 to define this syndrome more broadly. Deficiency of the semicircular canals is now a major criterion for CHARGE syndrome. To characterize patients with CHARGE syndrome at our center using Verloes' criteria and to reevaluate the nomenclature for this condition. We performed a medical chart review of patients with CHARGE syndrome and reviewed their temporal bone imaging studies at a tertiary care children's hospital affiliated with Washington University in St Louis. Two authors independently reviewed each imaging study (A.W. and K.H.). Radiologic studies, physical findings, genetic tests, and other diagnostic tests were included. Patients with no temporal bone imaging studies were excluded. Eighteen children were included in this study; 13 children (72%) were male, and the mean (median; range) age of patients at the time of inner ear imaging studies was 2 years (4.5 years; 8 months to 8 years). Coloboma was present in 13 patients (72%) and choanal atresia in 5 (28%); semicircular canal anomalies were present in all patients. Additionally, 13 patients (72%) were diagnosed as having hindbrain anomalies, 17 (94%) as having endocrine disorders, 17 (94%) as having mediastinal organ malformations, and all as having middle or external ear abnormalities and development delay. Cleft lip and cleft palate were found in 6 of 14 patients (43%) who did not have choanal atresia. We tested 16 patients for mutations in the CHD7 gene; 10 were positive (63%) for mutations, 4 (25%) were negative, and 2 (13%) were inconclusive. Semicircular canal anomalies were the most consistent finding in our patients with CHARGE syndrome. Given the high prevalence of semicircular canal hypoplasia and importance of imaging for diagnosing CHARGE syndrome, we propose changing the term CHARGE syndrome to 3C syndrome to emphasize the importance of the semicircular canals and to recall the 3 major criteria for diagnosis: coloboma, choanal atresia, and semicircular canal anomaly. The nomenclature would also reference the 3 semicircular canals in each ear. This new name for CHARGE syndrome would provide a mnemonic and focus the disease on the most important clinical criteria for diagnosis.

EVALUATION AND DIAGNOSIS OF THE DYSMORPHIC INFANT

PubMed Central

Jones, Kelly L.; Adam, Margaret P.

2015-01-01

SYNOPSIS Neonatologists often have the unique opportunity to be the first to identify abnormalities in the neonate. In this review, multiple anomalies and physical features are discussed along with the potential associated genetic syndromes. The anomalies and physical features that are discussed include birth parameters, aplasia cutis congenita, holoprosencephaly, asymmetric crying facies, preauricular ear tags and pits, cleft lip with or without cleft palate, esophageal atresia/tracheoesophageal fistula, congenital heart defects, ventral wall defects, and polydactyly. PMID:26042903

[Advances in genetics of congenital malformation of external and middle ear].

PubMed

Wang, Dayong; Wang, Qiuju

2013-05-01

Congenital malformation of external and middle ear is a common disease in ENT department, and the incidence of this disease is second only to cleft lip and palate in the whole congenital malformations of the head and face. The external and middle ear malformations may occur separately, or as an important ear symptom of the systemic syndrome. We systematically review and analysis the genetic research progress of congenital malformation of external and middle ear, which would be helpful to understand the mechanism of external and middle ear development, and to provide clues for the further discovery of new virulence genes.

[The diversity analysis of spontaneous cerebrospinal fluid otorrhea between young children and adults].

PubMed

Liu, J; Mei, L Y; He, C F; Feng, Y

2016-11-01

Objective: To assess the diversity of spontaneous cerebrospinal fluid（CSF）otorrhear in clinical manifestation，CT，leakage sites and surgical operation between young children and adults． Method: We conducted a retrospective study of 6 consecutive patients who were all underwent the surgery through the transmastoid approach，including 4 adults patients and 2 children．In the 4 adults patients，two patients' bony defects lay on the tegmen mastoideum，one lay on the tegmen tympani，and another one lay on the sinus meningioma angle．None of the 4 adults patients had abnormal inner ear structures．Materials used in repair included free muscle graft，temporalis fascia，and fibrin glue of the 4 adults patients．The 2 children patients were diagnosed with congenital abnormalities of the lateral inner ear，who had bony defects of the foot plate or fenestra vestibule．Materials used in repair included free muscle graft，temporalis fascia,and musclein sequence. Result: No CSF leaks recurred after the operation except one adults patient，who's left ear recurred two times and experienced three operations. Conclusion: The clinical manifestations of spontaneous CSF otorrhea between young children and adults are different，the HRCT scan on temporal bone before the operation is very important．Especially foradults patients，making sure of theleakagesites and numbers isvaluable and significance for thesurgical procedure selection．. Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.

Asymmetric Hearing During Development: The Aural Preference Syndrome and Treatment Options.

PubMed

Gordon, Karen; Henkin, Yael; Kral, Andrej

2015-07-01

Deafness affects ∼2 in 1000 children and is one of the most common congenital impairments. Permanent hearing loss can be treated by fitting hearing aids. More severe to profound deafness is an indication for cochlear implantation. Although newborn hearing screening programs have increased the identification of asymmetric hearing loss, parents and caregivers of children with single-sided deafness are often hesitant to pursue therapy for the deaf ear. Delayed intervention has consequences for recovery of hearing. It has long been reported that asymmetric hearing loss/single-sided deafness compromises speech and language development and educational outcomes in children. Recent studies in animal models of deafness and in children consistently show evidence of an "aural preference syndrome" in which single-sided deafness in early childhood reorganizes the developing auditory pathways toward the hearing ear, with weaker central representation of the deaf ear. Delayed therapy consequently compromises benefit for the deaf ear, with slow rates of improvement measured over time. Therefore, asymmetric hearing needs early identification and intervention. Providing early effective stimulation in both ears through appropriate fitting of auditory prostheses, including hearing aids and cochlear implants, within a sensitive period in development has a cardinal role for securing the function of the impaired ear and for restoring binaural/spatial hearing. The impacts of asymmetric hearing loss on the developing auditory system and on spoken language development have often been underestimated. Thus, the traditional minimalist approach to clinical management aimed at 1 functional ear should be modified on the basis of current evidence. Copyright © 2015 by the American Academy of Pediatrics.

Various causes and clinical characteristics in vertigo in children with normal eardrums.

PubMed

Choung, Yun-Hoon; Park, Keehyun; Moon, Sung-Kyun; Kim, Chul-Ho; Ryu, Sang Jun

2003-08-01

The differential diagnosis of vertigo in children is extensive. Otitis media and middle ear effusion could be the most common causes of vertigo in children, but there are some problems in detecting the other causes for vertigo because they are one of most frequent diseases of childhood. The purpose of this study is to review the clinical characteristics and both the audiological and vestibular findings of vertigo in children with normal eardrums, who do not show otitis media or middle ear effusion, and to assist in making a differential diagnosis of vertigo. The fifty five children (< 16 years old) with vertigo, who visited the Department of Otolaryngology, Ajou University Hospital, Suwon, South Korea between January 1995 and December 2001 were selected for this study. These excluded the patients with abnormal eardrums/tympanograms or those that did not perform questionnaires, audiological, or vestibular evaluations. They were retrospectively analyzed for clinical symptoms, vestibular functions, and differential diagnosis. The most common causes for vertigo in children were migraine in 17 (30.9%) and benign paroxysmal vertigo of childhood (BPVC) in 14 (25.5%). Other less frequent causes included four cases of trauma, two cases each of Meniere's disease, delayed endolymphatic hydrops, benign positional vertigo, and one case only for cerebellopontine angle tumor, seizure, acute vestibular neuritis, juvenile rheumatoid arthritis, leaving ten cases (18.2%) as unclassified. Abnormal findings were noted in 13 (23.6%) in pure tone audiogram, 3 (5.5%) in positioning test, 6 (10.9%) in bithermal caloric test, and 36 (65.5%) in rotation chair test. The vertigo in children with normal eardrums, who did not show otitis media or middle ear effusion, was most commonly caused by migraine and BPVC. These findings have shown to be very different from those with adult vertigo. The evaluation of vertigo in children requires a questionnaire for extensive and complete history taking, audiograms and vestibular function tests. And in selected cases, electroencephalography, hematological evaluation, imaging of the brain or temporal bone should be performed.

The development of gastric cancer in a patient with polycythemia Vera, 3P deletion, and JAK2 V617F mutation.

PubMed

Ayvaz, Ozlem; Yavasoglu, Irfan; Kadikoylu, Gurhan; Meydan, Nezih; Barutca, Sabri; Bolaman, Zahit

2010-12-01

3p deletion which is frequently associated with solitary tumors and hematological malignancies is a chromosomal abnormality. Recently, Janus kinase-2 (JAK2) V617F mutation has an important role in the diagnosis of myeloproliferative disorders, especially in polycythemia vera (PV). We reported the development of gastric cancer in a 75-year-old patient with PV, 3p 12-14 deletion and JAK2 V617F mutation. PV was diagnosed according to the classification of World Health Organization. JAK2 V617F mutation with polymerase chain reaction and 3p12-14 deletion with cytogenetic examination of the bone marrow were detected. We investigated solitary tumors in the patient using computed tomographies of thorax, neck, ear, nose, and throat. However, they were normal. After 2 years, gastric cancer appeared in the patient. In conclusion, cytogenetic examination may be important in both the development and the diagnosis of hematological malignancies and solitary tumors. So the patients should be followed closely.

Development of a finite element model of the middle ear.

PubMed

Williams, K R; Blayney, A W; Rice, H J

1996-01-01

A representative finite element model of the healthy ear is developed commencing with a description of the decoupled isotropic tympanic membrane. This model was shown to vibrate in a manner similar to that found both numerically (1, 2) and experimentally (8). The introduction of a fibre system into the membrane matrix significantly altered the modes of vibration. The first mode "remains as a piston like movement as for the isotropic membrane. However, higher modes show a simpler vibration pattern similar to the second mode but with a varying axis of movement and lower amplitudes. The introduction of a malleus and incus does not change the natural frequencies or mode shapes of the membrane for certain support conditions. When constraints are imposed along the ossicular chain by simulation of a cochlear impedance term then significantly altered modes can occur. More recently a revised model of the ear has been developed by the inclusion of the outer ear canal. This discretisation uses geometries extracted from a Nuclear Magnetic resonance scan of a healthy subject and a crude inner ear model using stiffness parameters ultimately fixed through a parameter tuning process. The subsequently tuned model showed behaviour consistent with previous findings and should provide a good basis for subsequent modelling of diseased ears and assessment of the performance of middle ear prostheses.

The role of intracochlear drug delivery devices in the management of inner ear disease.

PubMed

Ayoob, Andrew M; Borenstein, Jeffrey T

2015-03-01

Diseases of the inner ear include those of the auditory and vestibular systems, and frequently result in disabling hearing loss or vertigo. Despite a rapidly expanding pipeline of potential cochlear therapeutics, the inner ear remains a challenging organ for targeted drug delivery, and new technologies are required to deliver these therapies in a safe and efficacious manner. In addition to traditional approaches for direct inner ear drug delivery, novel microfluidics-based systems are under development, promising improved control over pharmacokinetics over longer periods of delivery, ultimately with application towards hair cell regeneration in humans. Advances in the development of intracochlear drug delivery systems are reviewed, including passive systems, active microfluidic technologies and cochlear prosthesis-mediated delivery. This article provides a description of novel delivery systems and their potential future clinical applications in treating inner ear disease. Recent progresses in microfluidics and miniaturization technologies are enabling the development of wearable and ultimately implantable drug delivery microsystems. Progress in this field is being spurred by the convergence of advances in molecular biology, microfluidic flow control systems and models for drug transport in the inner ear. These advances will herald a new generation of devices, with near-term applications in preclinical models, and ultimately with human clinical use for a range of diseases of the inner ear.

DOE Office of Scientific and Technical Information (OSTI.GOV)

Schimmenti, L.A.; Steinberger, J.; Mammel, M.C.

Partial deletion of the short arm of chromosome 9 (p24{r_arrow}pter) and partial duplication of the long arm of chromosome 5 (q32{r_arrow}qter) were observed in an abnormal boy who died at age 8 weeks of a complex cyanotic cardiac defect. He also had minor anomalies, sagittal craniosynostosis, triphalangeal thumbs, hypospadias, and a bifid scrotum. Two other infants with similar cytogenetic abnormalities were described previously. These patients had severe congenital heart defect, genitourinary anomalies, broad nasal bridge, low hairline, apparently low-set ears, short neck, and triphalangeal thumbs, in common with our patient. We suggest that combined monosomy 9q23,24{r_arrow}pter and trisomy 5q31,32{r_arrow}qter maymore » constitute a clinically recognizable syndrome. 13 refs., 2 figs., 2 tabs.« less

The Social Validity of Bug-in-Ear Coaching: Findings from Two Studies Implemented in Inclusive Early Childhood Environments

ERIC Educational Resources Information Center

Ottley, Jennifer Riggie; Coogle, Christan Grygas; Rahn, Naomi L.

2015-01-01

Coaching is a promising method for providing professional development, which takes many forms. One such form is real-time coaching through bug-in-ear technology. This study explored the social validity of bug-in-ear coaching when provided as a form of professional development with pre-service and in-service early childhood educators. Data from two…

The Social Validity of Bug-in-Ear Coaching: Findings from Two Studies Implemented in Inclusive Early Childhood Environments

ERIC Educational Resources Information Center

Ottley, Jennifer R.; Coogle, Christan Grygas; Rahn, Naomi L.

2015-01-01

Coaching is a promising method for providing professional development, which takes many forms. One such form is real-time coaching through bug-in-ear technology. This study explored the social validity of bug-in-ear coaching when provided as a form of professional development with preservice and in-service early childhood educators. Data from two…

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

PubMed

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

2012-08-01

We describe two sibs with a lethal form of profound congenital microcephaly, intrauterine and postnatal growth retardation, subtle skeletal changes, and poorly developed brain. The sibs had striking absent cranial vault with sloping of the forehead, large beaked nose, relatively large ears, and mandibular micro-retrognathia. Brain magnetic resonance imaging (MRI) revealed extremely simplified gyral pattern, large interhemispheric cyst and agenesis of corpus callosum, abnormally shaped hippocampus, and proportionately affected cerebellum and brainstem. In addition, fundus examination showed foveal hypoplasia with optic nerve atrophy. No abnormalities of the internal organs were found. This profound form of microcephaly was identified at 17 weeks gestation by ultrasound and fetal brain MRI helped in characterizing the developmental brain malformations in the second sib. Molecular analysis excluded mutations in potentially related genes such as RNU4ATAC, SLC25A19, and ASPM. These clinical and imaging findings are unlike that of any recognized severe forms of microcephaly which is believed to be a new microcephalic primordial dwarfism (MPD) with developmental brain malformations with most probably autosomal recessive inheritance based on consanguinity and similarly affected male and female sibs. Copyright © 2012 Wiley Periodicals, Inc.

A comparison of three methods to evaluate the position of an artificial ear on the deficient side of the face from a three-dimensional surface scan of patients with hemifacial microsomia.

PubMed

Coward, Trevor J; Watson, Roger M; Richards, Robin; Scott, Brendan J J

2012-01-01

Patients with hemifacial microsomia may have a missing ear on the deficient side of the face. The fabrication of an ear for such individuals usually has been accomplished by directly measuring the ear on the normal side to construct a prosthesis based on these dimensions, and the positioning has been, to a large extent, primarily operator-dependent. The aim of the present study was to compare three methods, developed from the identification of landmarks plotted on three-dimensional surface scans, to evaluate the position of an artificial ear on the deficient side of the face compared with the position of the natural ear on the normally developed side. Laser scans were undertaken of the faces of 14 subjects with hemifacial microsomia. Landmarks on the ear and face on the normal side were identified. Three methods of mirroring the normal ear on the deficient side of the face were investigated, which used either facial landmarks from the orbital area or a zero reference point generated from the intersection of three orthogonal planes on a frame of reference. To assess the methods, landmarks were identified on the ear situated on the normal side as well as on the face. These landmarks yielded paired dimensional measurements that could be compared between the normal and deficient sides. Mean differences and 95% confidence intervals were calculated. It was possible to mirror the normal ear image on to the deficient side of the face using all three methods. Generally only small differences between the dimensional measurements on the normal and deficient sides were observed. However, two-way analysis of variance revealed statistically significant differences between the three methods (P = .005). The method of mirroring using the outer canthi was found to result in the smallest dimensional differences between the anthropometric points on the ear and face between the normally developed and deficient sides. However, the effects of the deformity can result in limitations in relation to achieving a precise alignment of the ear to the facial tissues. This requires further study.

Ear asymmetries in middle-ear, cochlear, and brainstem responses in human infants

PubMed Central

Keefe, Douglas H.; Gorga, Michael P.; Jesteadt, Walt; Smith, Lynette M.

2008-01-01

In 2004, Sininger and Cone-Wesson examined asymmetries in the signal-to-noise ratio (SNR) of otoacoustic emissions (OAE) in infants, reporting that distortion-product (DP)OAE SNR was larger in the left ear, whereas transient-evoked (TE)OAE SNR was larger in the right. They proposed that cochlear and brainstem asymmetries facilitate development of brain-hemispheric specialization for sound processing. Similarly, in 2006 Sininger and Cone-Wesson described ear asymmetries mainly favoring the right ear in infant auditory brainstem responses (ABRs). The present study analyzed 2640 infant responses to further explore these effects. Ear differences in OAE SNR, signal, and noise were evaluated separately and across frequencies (1.5, 2, 3, and 4 kHz), and ABR asymmetries were compared with cochlear asymmetries. Analyses of ear-canal reflectance and admittance showed that asymmetries in middle-ear functioning did not explain cochlear and brainstem asymmetries. Current results are consistent with earlier studies showing right-ear dominance for TEOAE and ABR. Noise levels were higher in the right ear for OAEs and ABRs, causing ear asymmetries in SNR to differ from those in signal level. No left-ear dominance for DPOAE signal was observed. These results do not support a theory that ear asymmetries in cochlear processing mimic hemispheric brain specialization for auditory processing. PMID:18345839

A Ubiquitous Blood Pressure Sensor Worn at the Ear

NASA Astrophysics Data System (ADS)

Koizumi, Hiroshi; Shimada, Junichi; Uenishi, Yuji; Tochikubo, Osamu

2009-12-01

Blood pressure (BP) measurement and BP control are important for the prevention of lifestyle diseases, especially hypertension, which can lead to more serious conditions, such as cardiac infarction and cerebral apoplexy. The purpose of our study is to develop a ubiquitous blood pressure sensor that is more comfortable and less disruptive of users' daily activities than conventional blood pressure sensors. Our developed sensor is worn at an ear orifice and measures blood pressure at the tragus. This paper describes the concept, configuration, and the optical and electronic details of the developed ear-worn blood pressure sensor and presents preliminary evaluation results. The developed sensor causes almost no discomfort and produces signals whose quality is high enough for detecting BP at an ear, making it suitable for ubiquitous usage.

Headbobber: A Combined Morphogenetic and Cochleosaccular Mouse Model to Study 10qter Deletions in Human Deafness

PubMed Central

Buniello, Annalisa; Hardisty-Hughes, Rachel E.; Pass, Johanna C.; Bober, Eva; Smith, Richard J.; Steel, Karen P.

2013-01-01

The recessive mouse mutant headbobber (hb) displays the characteristic behavioural traits associated with vestibular defects including headbobbing, circling and deafness. This mutation was caused by the insertion of a transgene into distal chromosome 7 affecting expression of native genes. We show that the inner ear of hb/hb mutants lacks semicircular canals and cristae, and the saccule and utricle are fused together in a single utriculosaccular sac. Moreover, we detect severe abnormalities of the cochlear sensory hair cells, the stria vascularis looks severely disorganised, Reissner's membrane is collapsed and no endocochlear potential is detected. Myo7a and Kcnj10 expression analysis show a lack of the melanocyte-like intermediate cells in hb/hb stria vascularis, which can explain the absence of endocochlear potential. We use Trp2 as a marker of melanoblasts migrating from the neural crest at E12.5 and show that they do not interdigitate into the developing strial epithelium, associated with abnormal persistence of the basal lamina in the hb/hb cochlea. We perform array CGH, deep sequencing as well as an extensive expression analysis of candidate genes in the headbobber region of hb/hb and littermate controls, and conclude that the headbobber phenotype is caused by: 1) effect of a 648 kb deletion on distal Chr7, resulting in the loss of three protein coding genes (Gpr26, Cpmx2 and Chst15) with expression in the inner ear but unknown function; and 2) indirect, long range effect of the deletion on the expression of neighboring genes on Chr7, associated with downregulation of Hmx3, Hmx2 and Nkx1.2 homeobox transcription factors. Interestingly, deletions of the orthologous region in humans, affecting the same genes, have been reported in nineteen patients with common features including sensorineural hearing loss and vestibular problems. Therefore, we propose that headbobber is a useful model to gain insight into the mechanisms underlying deafness in human 10qter deletion syndrome. PMID:23457544

The burned ear; possibilities and challenges in framework reconstruction and coverage.

PubMed

Bos, E J; Doerga, P; Breugem, C C; van Zuijlen, P P

2016-11-01

Ear reconstruction remains a challenging procedure, especially in burn victims. The ear is particularly vulnerable to thermal injury because of its location and the thin integument. The thermal injury could subsequently include skin and the deeper located auricular cartilage framework. This type of injury could have long lasting mutilating effect not only because the ear's morphology is mainly related to this framework but also because it will not recover or regenerate once injured. Grafts of costal cartilage or synthetic materials might replace missing cartilage. However, the poor quality of the adjacent skin and subcutaneous tissues makes the reconstruction of a burned ear an even more daunting procedure than congenital or many oncologic indications. As such, regeneration of the skin will be the next step in reconstruction of the burned ear. There is still much development and research to be done, but encouraging results have been shown in tissue engineering of skin and cartilage. Furthermore, 3D (bio)printing of cartilage to facilitate reproduction of the ear's complex shape certainly has potential and might find an interesting role in ear reconstruction. In this review, different clinical challenges and options for ear reconstruction in burn patients are described. Subsequently, although still far from large scale clinical application, state of the art developments in the field of tissue engineering and 3D (bio)printing are also discussed. Copyright © 2016 Elsevier Ltd and ISBI. All rights reserved.

A Study to Design a System to Identify Handicapped Family Members of Active Duty Soldiers at Fort Benning, Georgia

DTIC Science & Technology

1982-05-01

words spoken. Under Age 7 % 30 decibel hearing loss without hearing aid in one or both ears. Epilepsy Parkinson’s Disease Cerebral Palsy Multiple... Intervention in the Care of the Physically Handicapped, Severely Retarded Child." Nursing Clinics of North America, Vol. 10, No. 2 (June 1975): 353...requires assistance to support the essentials of daily living. h. Cerebral Palsy . With: (1) IQ of 83 or less; (2) Abnormal behavior patterns, such as

Four cases of acoustic neuromas with normal hearing.

PubMed

Valente, M; Peterein, J; Goebel, J; Neely, J G

1995-05-01

In 95 percent of the cases, patients with acoustic neuromas will have some magnitude of hearing loss in the affected ear. This paper reports on four patients who had acoustic neuromas and normal hearing. Results from the case history, audiometric evaluation, auditory brainstem response (ABR), electroneurography (ENOG), and vestibular evaluation are reported for each patient. For all patients, the presence of unilateral tinnitus was the most common complaint. Audiologically, elevated or absent acoustic reflex thresholds and abnormal ABR findings were the most powerful diagnostic tools.

INNER EAR EMBRYOGENESIS: GENETIC AND ENVIRONMENTAL DETERMINANTS

EPA Science Inventory

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

Validity of the Hum Test, a Simple and Reliable Alternative to the Weber Test.

PubMed

Ahmed, Omar H; Gallant, Sara C; Ruiz, Ryan; Wang, Binhuan; Shapiro, William H; Voigt, Erich P

2018-06-01

To compare the diagnostic performance of the Hum Test against the Weber Test using pure tone audiometry (PTA) as the "gold standard" comparator. 29 participants with normal hearing of ages 18 to 35 without any history of hearing abnormalities or otologic conditions were enrolled. Subjects underwent three tests (Hum Test, Weber Test, and PTA) across two conditions: with an ear plug in one ear (side randomized) and without ear plugs. When examining the ability of the Hum Test to detect simulated conductive hearing loss (CHL), the test had a sensitivity of 89.7% and specificity of 100% with high pitched humming and 93.1% and 100%, respectively, with low pitched humming. The Weber Test had a sensitivity and specificity of 96.6% and 100%, respectively. McNemar's test demonstrated agreement between the Hum Test, performed with either high pitched ( P = .32) or low pitched ( P = .56) humming, and the Weber Test. Receiver operating characteristic (ROC) curves for the Hum Test (both high and low pitched) and Weber test were compared and demonstrated no statistically significant difference. The Hum Test is comparable to the Weber Test with regards to its sensitivity, specificity, and diagnostic accuracy in assessing new onset unilateral CHL in previously normal hearing subjects.

Effects of Carriers, Emulsifiers, and Biopesticides for Direct Silk Treatments on Caterpillar Feeding Damage and Ear Development in Sweet Corn.

PubMed

Westgate, P J; Schultz, B B; Hazzard, R V

2017-04-01

In the northeastern United States, control of Lepidopteran pests of sweet corn, particularly corn earworm [Helicoverpa zea (Boddie)], is difficult using organic methods. The direct application of corn oil and Bacillus thuringiensis (Bt) to corn silk has been shown to reduce ear damage from corn earworm in past studies; these studies sought to optimize this method by evaluating additional carrier and biopesticide mixtures that comply with the United States Federal Insecticide, Fungicide, and Rodenticide Act and National Organic Standards. Carriers, which are liquids used to dissolve the biopesticide and deliver it into the tip of the ear, may have phytotoxic or insecticidal properties. Experiments conducted from 2001 to 2005 evaluated caterpillar damage and ear development effects from carriers (vegetable and paraffinic oils and carrageenan), biopesticides (Bt, spinsosad, and neem), and three emulsifiers in various combinations when applied directly to the tips of the ears 5-7 d after silk initiation. There were no effects of emulsifiers on ear quality, except for slight reduction in caterpillar damage in one of the two years. There were no differences among corn, soy, canola, and safflower oils in corn earworm control or tip development. The carrageenan carrier had the least effect upon ear development as measured by the length of nonpollinated kernels at the tip, compared to corn oil or paraffinic oil (JMS Stylet Oil), which caused the greatest tip damage as well as an oily discoloration. The carrier-pesticide combinations with the best ear quality overall were spinosad in carrageenan or corn oil, and Bt in carrageenan. © The Authors 2017. Published by Oxford University Press on behalf of Entomological Society of America. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

Identification of Adeno-Associated Viral Vectors That Target Neonatal and Adult Mammalian Inner Ear Cell Subtypes

PubMed Central

Shu, Yilai; Tao, Yong; Wang, Zhengmin; Tang, Yong; Li, Huawei; Dai, Pu; Gao, Guangping; Chen, Zheng-Yi

2016-01-01

The mammalian inner ear consists of diverse cell types with important functions. Gene mutations in these diverse cell types have been found to underlie different forms of genetic hearing loss. Targeting these mutations for gene therapy development represents a future therapeutic strategy to treat hearing loss. Adeno-associated viral (AAV) vectors have become the vector of choice for gene delivery in animal models in vivo. To identify AAV vectors that target inner ear cell subtypes, we systemically screened 12 AAV vectors with different serotypes (AAV1, 2, 5, 6, 6.2, 7, 8, 9, rh.8, rh.10, rh.39, and rh.43) that carry a reporter gene GFP in neonatal and adult mice by microinjection in vivo. We found that most AAVs infect both neonatal and adult inner ear, with different specificities and expression levels. The inner ear cochlear sensory epithelial region, which includes auditory hair cells and supporting cells, is most frequently targeted for gene delivery. Expression of the transgene is sustained, and neonatal inner ear delivery does not adversely affect hearing. Adult inner ear injection of AAV has a similar infection pattern as the younger inner ear, with the exception that outer hair cell death caused by the injection procedure can lead to hearing loss. In the adult, more so than in the neonatal mice, cell types infected and efficiency of infection are correlated with the site of injection. Most infected cells survive in neonatal and adult inner ears. The study adds to the list of AAV vectors that transduce the mammalian inner ear efficiently, providing the tools that are important to study inner ear gene function and for the development of gene therapy to treat hearing loss. PMID:27342665

Computational Modeling of Blast Wave Transmission Through Human Ear.

PubMed

Leckness, Kegan; Nakmali, Don; Gan, Rong Z

2018-03-01

Hearing loss has become the most common disability among veterans. Understanding how blast waves propagate through the human ear is a necessary step in the development of effective hearing protection devices (HPDs). This article presents the first 3D finite element (FE) model of the human ear to simulate blast wave transmission through the ear. The 3D FE model of the human ear consisting of the ear canal, tympanic membrane, ossicular chain, and middle ear cavity was imported into ANSYS Workbench for coupled fluid-structure interaction analysis in the time domain. Blast pressure waveforms recorded external to the ear in human cadaver temporal bone tests were applied at the entrance of the ear canal in the model. The pressure waveforms near the tympanic membrane (TM) in the canal (P1) and behind the TM in the middle ear cavity (P2) were calculated. The model-predicted results were then compared with measured P1 and P2 waveforms recorded in human cadaver ears during blast tests. Results show that the model-derived P1 waveforms were in an agreement with the experimentally recorded waveforms with statistic Kurtosis analysis. The FE model will be used for the evaluation of HPDs in future studies.

Evolution and development of the vertebrate ear

NASA Technical Reports Server (NTRS)

Fritzsch, B.; Beisel, K. W.

2001-01-01

This review outlines major aspects of development and evolution of the ear, specifically addressing issues of cell fate commitment and the emerging molecular governance of these decisions. Available data support the notion of homology of subsets of mechanosensors across phyla (proprioreceptive mechanosensory neurons in insects, hair cells in vertebrates). It is argued that this conservation is primarily related to the specific transducing environment needed to achieve mechanosensation. Achieving this requires highly conserved transcription factors that regulate the expression of the relevant structural genes for mechanosensory transduction. While conserved at the level of some cell fate assignment genes (atonal and its mammalian homologue), the ear has also radically reorganized its development by implementing genes used for cell fate assignment in other parts of the developing nervous systems (e.g., neurogenin 1) and by evolving novel sets of genes specifically associated with the novel formation of sensory neurons that contact hair cells (neurotrophins and their receptors). Numerous genes have been identified that regulate morphogenesis, but there is only one common feature that emerges at the moment: the ear appears to have co-opted genes from a large variety of other parts of the developing body (forebrain, limbs, kidneys) and establishes, in combination with existing transcription factors, an environment in which those genes govern novel, ear-related morphogenetic aspects. The ear thus represents a unique mix of highly conserved developmental elements combined with co-opted and newly evolved developmental elements.

Handheld tympanometer measurements in conscious dogs for the evaluation of the middle ear and auditory tube.

PubMed

Strain, George M; Fernandes, Asia J

2015-06-01

Otitis externa is frequently accompanied by otitis media, yet it can be difficult to evaluate the tympanum, middle ear and auditory tube without the use of advanced radiographic imaging. The objective was to develop techniques for tympanometry testing in conscious dogs and to present normative data for clinical use of this equipment to enable assessment of the tympanum, middle ear and auditory tube. Sixteen hounds (14 female) from a school teaching colony. Dogs were gently restrained in a standing position. After cleaning of the ear canal, a tympanometer probe tip extension was placed in the vertical canal and automated testing performed using a handheld device. Both ears were tested in all dogs. Acceptable recordings were obtained from both ears of 13 dogs, from one ear in each of two dogs and from neither ear of one dog, resulting in data from 28 of 32 (88%) ears. Otoscopic examination confirmed the absence of inflammation or any other obvious explanation for the noncompliant dogs. No significant differences were seen between ears for any measure. Normative data are reported for peak compliance, peak compliance pressure, gradient and ear canal volume. Tympanograms can be recorded in conscious dogs to assist in the evaluation of the middle ear structures. © 2015 ESVD and ACVD.

Effects of model definitions and parameter values in finite element modeling of human middle ear mechanics.

PubMed

De Greef, Daniel; Pires, Felipe; Dirckx, Joris J J

2017-02-01

Despite continuing advances in finite element software, the realistic simulation of middle ear response under acoustic stimulation continues to be challenging. One reason for this is the wide range of possible choices that can be made during the definition of a model. Therefore, an explorative study of the relative influences of some of these choices is potentially very helpful. Three finite element models of the human middle ear were constructed, based on high-resolution micro-computed tomography scans from three different human temporal bones. Interesting variations in modeling definitions and parameter values were selected and their influences on middle ear transmission were evaluated. The models were compared against different experimental validation criteria, both from the literature and from our own measurements. Simulation conditions were restricted to the frequency range 0.1-10 kHz. Modeling the three geometries with the same modeling definitions and parameters produces stapes footplate response curves that exhibit similar shapes, but quantitative differences of 4 dB in the lower frequencies and up to 6 dB around the resonance peaks. The model properties with the largest influences on our model outcomes are the tympanic membrane (TM) damping and stiffness and the cochlear load. Model changes with a small to negligible influence include the isotropy or orthotropy of the TM, the geometry of the connection between the TM and the malleus, the microstructure of the incudostapedial joint, and the length of the tensor tympani tendon. The presented results provide insights into the importance of different features in middle ear finite element modeling. The application of three different individual middle ear geometries in a single study reduces the possibility that the conclusions are strongly affected by geometrical abnormalities. Some modeling variations that were hypothesized to be influential turned out to be of minor importance. Furthermore, it could be confirmed that different geometries, simulated using the same parameters and definitions, can produce significantly different responses. Copyright © 2016 Elsevier B.V. All rights reserved.

Further insight into the phenotype associated with a mutation in the ORC6 gene, causing Meier-Gorlin syndrome 3.

PubMed

Shalev, Stavit Allon; Khayat, Morad; Etty, Daniel-Spiegl; Elpeleg, Orly

2015-03-01

Mutations in genes encoding the origin recognition complex subunits cause Meier-Gorlin syndrome. The disease manifests a triad of short stature, small ears, and small and/or absent patellae with variable expressivity. We report on the identification of a homozygous deleterious mutation in the ORC6 gene in previously described fetuses at the severe end of the Meier-Gorlin spectrum. The phenotype included severe intrauterine growth retardation, dislocation of knees, gracile bones, clubfeet, and small mandible and chest. To date, the clinical presentation of ORC6-associated Meier-Gorlin syndrome has been mild compared to other the phenotype associated with other loci. The present report expands the clinical phenotype associated with ORC6 mutations to include severely abnormal embryological development suggesting a possible genotype-phenotype correlation. © 2015 Wiley Periodicals, Inc.

High-throughput method for ear phenotyping and kernel weight estimation in maize using ear digital imaging.

PubMed

Makanza, R; Zaman-Allah, M; Cairns, J E; Eyre, J; Burgueño, J; Pacheco, Ángela; Diepenbrock, C; Magorokosho, C; Tarekegne, A; Olsen, M; Prasanna, B M

2018-01-01

Grain yield, ear and kernel attributes can assist to understand the performance of maize plant under different environmental conditions and can be used in the variety development process to address farmer's preferences. These parameters are however still laborious and expensive to measure. A low-cost ear digital imaging method was developed that provides estimates of ear and kernel attributes i.e., ear number and size, kernel number and size as well as kernel weight from photos of ears harvested from field trial plots. The image processing method uses a script that runs in a batch mode on ImageJ; an open source software. Kernel weight was estimated using the total kernel number derived from the number of kernels visible on the image and the average kernel size. Data showed a good agreement in terms of accuracy and precision between ground truth measurements and data generated through image processing. Broad-sense heritability of the estimated parameters was in the range or higher than that for measured grain weight. Limitation of the method for kernel weight estimation is discussed. The method developed in this work provides an opportunity to significantly reduce the cost of selection in the breeding process, especially for resource constrained crop improvement programs and can be used to learn more about the genetic bases of grain yield determinants.

Suppression and restoration of male fertility using a transcription factor.

PubMed

Li, Song Feng; Iacuone, Sylvana; Parish, Roger W

2007-03-01

The Arabidopsis AtMYB103 gene codes for an R2R3 MYB domain protein whose expression is restricted to the tapetum of developing anthers and to trichomes. Down-regulation of expression using anti-sense leads to abnormal tapetum and pollen development, although seed setting still occurs (Higginson, T., Li, S.F. and Parish, R.W. (2003) AtMYB103 regulates tapetum and trichome development in Arabidopsis thaliana. Plant J. 35, 177-192). In this study, we show that blocking the function of the AtMYB103 gene, employing either an insertion mutant or an AtMYB103EAR chimeric repressor construct under the control of the AtMYB103 promoter, results in complete male sterility and failure to set seed. These plants exhibit similar abnormalities in tapetum and pollen development, with the tapetum becoming highly vacuolated at early stages and degenerating prematurely. No exine is deposited on to the pollen wall. The degeneration of pollen grains commences prior to pollen mitosis, the pollen collapsing and largely lacking cytoplasmic content. A restorer containing the AtMYB103 gene under the control of a stronger anther-specific promoter was introduced into pollen donor plants and crossed into the male sterile plants transgenic for the repressor. The male fertility of F1 plants was restored. The chimeric repressor and the restorer constitute a reversible male sterility system which could be adapted for hybrid seed production. This is the first reversible male sterility system targeting a transcription factor essential for pollen development. Strategies for generating inducible male sterility and maintainable male sterility for the production of hybrid crops are discussed.

Hearing loss and speech perception in noise difficulties in Fanconi anemia.

PubMed

Verheij, Emmy; Oomen, Karin P Q; Smetsers, Stephanie E; van Zanten, Gijsbert A; Speleman, Lucienne

2017-10-01

Fanconi anemia is a hereditary chromosomal instability disorder. Hearing loss and ear abnormalities are among the many manifestations reported in this disorder. In addition, Fanconi anemia patients often complain about hearing difficulties in situations with background noise (speech perception in noise difficulties). Our study aimed to describe the prevalence of hearing loss and speech perception in noise difficulties in Dutch Fanconi anemia patients. Retrospective chart review. A retrospective chart review was conducted at a Dutch tertiary care center. All patients with Fanconi anemia at clinical follow-up in our hospital were included. Medical files were reviewed to collect data on hearing loss and speech perception in noise difficulties. In total, 49 Fanconi anemia patients were included. Audiograms were available in 29 patients and showed hearing loss in 16 patients (55%). Conductive hearing loss was present in 24.1%, sensorineural in 20.7%, and mixed in 10.3%. A speech in noise test was performed in 17 patients; speech perception in noise was subnormal in nine patients (52.9%) and abnormal in two patients (11.7%). Hearing loss and speech perception in noise abnormalities are common in Fanconi anemia. Therefore, pure tone audiograms and speech in noise tests should be performed, preferably already at a young age, because hearing aids or assistive listening devices could be very valuable in developing language and communication skills. 4. Laryngoscope, 127:2358-2361, 2017. © 2016 The American Laryngological, Rhinological and Otological Society, Inc.

Continuous 24-hour measurement of middle ear pressure.

PubMed

Tideholm, B; Jönsson, S; Carlborg, B; Welinder, R; Grenner, J

1996-07-01

A new method was developed for continuous measurement of the middle ear pressure during a 24-h period. The equipment consisted of a piezo-electric pressure device and a digital memory. To allow continuous pressure recordings during normal every-day activities the equipment was made light and portable. The measurement accuracy of the equipment as well as the base-line and temperature stability were tested and found to meet to our requirements satisfactorily. In 4 volunteers with different middle ear conditions, a small perforation was made through the tympanic membrane. A rubber stopper containing a small polyethylene tube was fitted into the external ear canal. Tubal function tests were made to establish the equipment's ability to monitor fast pressure changes. The tests were well in accordance with other methods of direct pressure measurements. The equipment was carried by the volunteers for 24 h to monitor any slow or rapid dynamic pressure changes in the middle ear. Four continuous 24-h measurements are presented. The method was found to be suitable for valid measurements of dynamic pressure changes in the middle ear during normal every-day activities. It may become a useful instrument in the search for a better understanding of the development of chronic middle ear disease.

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

PubMed

Abdel-Aziz, Mosaad

2012-06-25

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. 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. 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.

The 7q11.23 Microduplication Syndrome: A Clinical Report with Review of Literature

PubMed Central

Abbas, Elham; Cox, Devin M.; Smith, Teri; Butler, Merlin G.

2016-01-01

We report a 14-year-old adolescent girl with selective mutism (SM) and a 7q11.23 microduplication detected by chromosomal microarray (CMA) analysis and reviewed the literature from 18 published clinical reports. Our patient had specific phobias, SM, extreme anxiety, obesity, cutis marmorata, and a round appearing face with a short neck and over folded ears. We reviewed the published clinical, cognitive, behavioral, and cytogenetic findings grouped by speech and language delay, growth and development, craniofacial, clinical, and behavior and cognitive features due to the 7q11.23 microduplication. This microduplication syndrome is characterized by speech delay (91%), social anxiety (42%), attention deficit hyperactivity disorder (ADHD, 37%), autism spectrum disorder (29%), and separation anxiety (13%). Other findings include abnormal brain imaging (80%), congenital heart and vascular defects (54%), and mild intellectual disability (38%). We then compared the phenotype with Williams–Beuren syndrome (WBS) which is due to a deletion of the same chromosome region. Both syndromes have abnormal brain imaging, hypotonia, delayed motor development, joint laxity, mild intellectual disability, ADHD, autism, and poor visuospatial skills but opposite or dissimilar findings regarding speech and behavioral patterns, cardiovascular problems, and social interaction. Those with WBS are prone to have hyperverbal speech, lack of stranger anxiety, and supravalvular aortic stenosis while those with the 7q11.23 microduplication have speech delay, SM, social anxiety, and are prone to aortic dilatation. PMID:27617154

The 7q11.23 Microduplication Syndrome: A Clinical Report with Review of Literature.

PubMed

Abbas, Elham; Cox, Devin M; Smith, Teri; Butler, Merlin G

2016-09-01

We report a 14-year-old adolescent girl with selective mutism (SM) and a 7q11.23 microduplication detected by chromosomal microarray (CMA) analysis and reviewed the literature from 18 published clinical reports. Our patient had specific phobias, SM, extreme anxiety, obesity, cutis marmorata, and a round appearing face with a short neck and over folded ears. We reviewed the published clinical, cognitive, behavioral, and cytogenetic findings grouped by speech and language delay, growth and development, craniofacial, clinical, and behavior and cognitive features due to the 7q11.23 microduplication. This microduplication syndrome is characterized by speech delay (91%), social anxiety (42%), attention deficit hyperactivity disorder (ADHD, 37%), autism spectrum disorder (29%), and separation anxiety (13%). Other findings include abnormal brain imaging (80%), congenital heart and vascular defects (54%), and mild intellectual disability (38%). We then compared the phenotype with Williams-Beuren syndrome (WBS) which is due to a deletion of the same chromosome region. Both syndromes have abnormal brain imaging, hypotonia, delayed motor development, joint laxity, mild intellectual disability, ADHD, autism, and poor visuospatial skills but opposite or dissimilar findings regarding speech and behavioral patterns, cardiovascular problems, and social interaction. Those with WBS are prone to have hyperverbal speech, lack of stranger anxiety, and supravalvular aortic stenosis while those with the 7q11.23 microduplication have speech delay, SM, social anxiety, and are prone to aortic dilatation.

On the differential diagnosis of Ménière's disease using low-frequency acoustic biasing of the 2f1-f2 DPOAE.

PubMed

Brown, Daniel J; Gibson, William P R

2011-12-01

We have cyclically suppressed the 2f1-f2 distortion product otoacoustic emission (DPOAE) with low-frequency tones (17-97 Hz) as a way of differentially diagnosing the endolymphatic hydrops assumed to be associated with Ménière's syndrome. Round-window electrocochleography (ECochG) was performed in subjects with sensorineural hearing loss (SNHL) on the day of DPOAE testing, and from which the amplitude of the summating potential (SP) was measured, to support the diagnosis of Ménière's syndrome based on symptoms. To summarize and compare the cyclic patterns of DPOAE modulation in these groups we have used the simplest model of DPOAE generation and modulation, by assuming that the DPOAEs were generated by a 1st-order Boltzmann nonlinearity so that the magnitude of the 2f1-f2 DPOAE resembled the 3rd derivative of the Boltzmann function. We have also assumed that the modulation of the DPOAEs by the low-frequency tones was simply due to a sinusoidal change in the operating point on the Boltzmann nonlinearity. We have found the cyclic DPOAE modulation to be different in subjects with Ménière's syndrome (n = 16) when compared to the patterns in normal subjects (n = 16) and in other control subjects with non-Ménière's SNHL and/or vestibular disorders (n = 13). The DPOAEs of normal and non-Ménière's ears were suppressed more during negative ear canal pressure than during positive ear canal pressure. By contrast, DPOAE modulation in Ménière's ears with abnormal ECochG was greatest during positive ear canal pressures. This test may provide a tool for diagnosing Ménière's in the early stages, and might be used to investigate the pathological mechanism underlying the hearing symptoms of this syndrome. Crown Copyright © 2011. Published by Elsevier B.V. All rights reserved.

Ectopic Mineralization and Conductive Hearing Loss in Enpp1asj Mutant Mice, a New Model for Otitis Media and Tympanosclerosis.

PubMed

Tian, Cong; Harris, Belinda S; Johnson, Kenneth R

2016-01-01

Otitis media (OM), inflammation of the middle ear, is a common cause of hearing loss in children and in patients with many different syndromic diseases. Studies of the human population and mouse models have revealed that OM is a multifactorial disease with many environmental and genetic contributing factors. Here, we report on otitis media-related hearing loss in asj (ages with stiffened joints) mutant mice, which bear a point mutation in the Enpp1 gene. Auditory-evoked brainstem response (ABR) measurements revealed that around 90% of the mutant mice (Enpp1asj/asj) tested had moderate to severe hearing impairment in at least one ear. The ABR thresholds were variable and generally elevated with age. We found otitis media with effusion (OME) in all of the hearing-impaired Enpp1asj/asj mice by anatomic and histological examinations. The volume and inflammatory cell content of the effusion varied among the asj mutant mice, but all mutants exhibited a thickened middle ear epithelium with fibrous polyps and more mucin-secreting goblet cells than controls. Other abnormalities observed in the Enpp1 mutant mice include over-ossification at the round window ridge, thickened and over-calcified stapedial artery, fusion of malleus and incus, and white patches on the inside of tympanic membrane, some of which are typical symptoms of tympanosclerosis. An excessive yellow discharge was detected in the outer ear canal of older asj mutant mice, with 100% penetrance by 5 months of age, and contributes to the progressive nature of the hearing loss. This is the first report of hearing loss and ear pathology associated with an Enpp1 mutation in mice. The Enpp1asj mutant mouse provides a new animal model for studying tympanosclerotic otitis and otitis media with effusion, and also provides a specific model for the hearing loss recently reported to be associated with human ENPP1 mutations causing generalized arterial calcification of infancy and hypophosphatemic rickets.

Ectopic Mineralization and Conductive Hearing Loss in Enpp1asj Mutant Mice, a New Model for Otitis Media and Tympanosclerosis

PubMed Central

Tian, Cong; Harris, Belinda S.; Johnson, Kenneth R.

2016-01-01

Otitis media (OM), inflammation of the middle ear, is a common cause of hearing loss in children and in patients with many different syndromic diseases. Studies of the human population and mouse models have revealed that OM is a multifactorial disease with many environmental and genetic contributing factors. Here, we report on otitis media-related hearing loss in asj (ages with stiffened joints) mutant mice, which bear a point mutation in the Enpp1 gene. Auditory-evoked brainstem response (ABR) measurements revealed that around 90% of the mutant mice (Enpp1asj/asj) tested had moderate to severe hearing impairment in at least one ear. The ABR thresholds were variable and generally elevated with age. We found otitis media with effusion (OME) in all of the hearing-impaired Enpp1asj/asj mice by anatomic and histological examinations. The volume and inflammatory cell content of the effusion varied among the asj mutant mice, but all mutants exhibited a thickened middle ear epithelium with fibrous polyps and more mucin-secreting goblet cells than controls. Other abnormalities observed in the Enpp1 mutant mice include over-ossification at the round window ridge, thickened and over-calcified stapedial artery, fusion of malleus and incus, and white patches on the inside of tympanic membrane, some of which are typical symptoms of tympanosclerosis. An excessive yellow discharge was detected in the outer ear canal of older asj mutant mice, with 100% penetrance by 5 months of age, and contributes to the progressive nature of the hearing loss. This is the first report of hearing loss and ear pathology associated with an Enpp1 mutation in mice. The Enpp1asj mutant mouse provides a new animal model for studying tympanosclerotic otitis and otitis media with effusion, and also provides a specific model for the hearing loss recently reported to be associated with human ENPP1 mutations causing generalized arterial calcification of infancy and hypophosphatemic rickets. PMID:27959908

Age- and Gender-Specific Normative Information from Children Assessed with a Dichotic Words Test.

PubMed

Moncrieff, Deborah

2015-01-01

The most widely used assessment in the clinical auditory processing disorder (APD) battery is the dichotic listening test. New tests with normative information are helpful for assessment and cross-check of results for reliable diagnosis. The Dichotic Words Test was developed for use in the clinical test battery for diagnosis of APD. The test stimuli were common single syllable words matched for average root-mean-square amplitude and each pair was temporally aligned at both onset and offset. The study was conducted to collect performance results from typically developing children to create normative information for the test. The study follows a cross-sectional design. Typically developing children (n = 416) between the ages of 5 and 12 yr were recruited from schools in the community. There were 217 males and 199 females in the study sample. Only children who passed a hearing screening were eligible to participate. Scores for each ear were recorded during administration of the first free recall version of the test. Ear advantages based on results recorded for left and right ears were used to measure prevalence of right, left, and no ear advantages. Results for each listener's dominant and non-dominant ears and the absolute difference between them were put into the data analysis. Results were analyzed for normality and because no results were normally distributed, all further analyses were done with nonparametric statistical tests. Normative data for dominant and non-dominant ear scores and ear advantages were determined at the 95% confidence interval through bootstrapping methods with 1,000 samples. Children were divided into four age groups based on results in their dominant ears. Females generally performed better than males and the prevalence of a right-ear advantage was ∼60% across all children tested. Normative lower-bound cut-off scores were established for males and females within each age group for dominant and non-dominant ear scores. Normative upper-bound cut-off scores were established for males and females within each age group for ear advantage scores. Normative information specific to age group and gender will be useful in clinical assessment for APD. Prevalence of left-ear advantage results in the sample may have been partly due to uncontrolled influences of voice-onset time in arranging the dichotic pairs. American Academy of Audiology.

A mouse model with postnatal endolymphatic hydrops and hearing loss

PubMed Central

Megerian, Cliff A.; Semaan, Maroun T.; Aftab, Saba; Kisley, Lauren B.; Zheng, Qing Yin; Pawlowski, Karen S.; Wright, Charles G.; Alagramam, Kumar N.

2010-01-01

Endolymphatic hydrops (ELH), hearing loss and neuronal degeneration occur together in a variety of clinically significant disorders, including Meniere’s disease (MD). However, the sequence of these pathological changes and their relationship to each other are not well understood. In this regard, an animal model that spontaneously develops these features postnatally would be useful for research purposes. A search for such a model led us to the PhexHyp-Duk mouse, a mutant allele of the Phex gene causing X-linked hypophosphatemic rickets. The hemizygous male (PhexHyp-Duk/Y) was previously reported to exhibit various abnormalities during adulthood, including thickening of bone, ELH and hearing loss. The reported inner-ear phenotype was suggestive of progressive pathology and spontaneous development of ELH postnatally, but not conclusive. The main focuses of this report are to further characterize the inner ear phenotype in PhexHyp-Duk/Y mice and to test the hypotheses that (a) the PhexHyp-Duk/Y mouse develops ELH and hearing loss postnatally, and (b) the development of ELH in the PhexHyp-Duk/Y mouse is associated with obstruction of the endolymphatic duct (ED) due to thickening of the surrounding bone. Auditory brainstem response (ABR) recordings at various times points and histological analysis of representative temporal bones reveal that PhexHyp-Duk/Y mice typically develop adult onset, asymmetric, progressive hearing loss closely followed by the onset of ELH. ABR and histological data show that functional degeneration precedes structural degeneration. The major degenerative correlate of hearing loss and ELH in the mutants is the primary loss of spiral ganglion cells. Further, PhexHyp-Duk/Y mice develop ELH without evidence of ED obstruction, supporting the idea that ELH can be induced by a mechanism other than the blockade of longitudinal flow of endolymphatic fluid, and occlusion of ED is not a prerequisite for the development of ELH in patients. PMID:18289812

Incorporating anthropometry into design of ear-related products.

PubMed

Liu, Bor-Shong

2008-01-01

To achieve mass customization and collaborative product design, human factors and ergonomics should play a key development role. The purpose of this study was to provide product designers with the anthropometic dimensions of outer ears for different demographic data, including gender and age. The second purpose was to compare the dimensions of various ear-related products (i.e., earphone, bluetooth earphone and ear-cup earphone) with the anthropometic database and recommend appropriate solutions for design. Two hundred subjects aged 20-59 was selected for this study and divided into four age stratifications. Further, three different dimensions of the outer ear (i.e., the earhole length, the ear connection length and the length of the pinna) were measured by superimposed grid photographic technique. The analysis of variance (ANOVA) was used to investigate the effects of gender, and age on ear dimensions. The results showed that all ear dimensions had significant gender effects. A comparison between the anthropometric dimensions and those of current products revealed that most current ear-related products need to be redesigned using anthropometric data. The shapes of earhole and pinna are not circular. Consequently, ear products need to be elongated so that users may feel more comfortably and not have the product slip off easily.

Structure and function of the mammalian middle ear. I: Large middle ears in small desert mammals.

PubMed

Mason, Matthew J

2016-02-01

Many species of small desert mammals are known to have expanded auditory bullae. The ears of gerbils and heteromyids have been well described, but much less is known about the middle ear anatomy of other desert mammals. In this study, the middle ears of three gerbils (Meriones, Desmodillus and Gerbillurus), two jerboas (Jaculus) and two sengis (elephant-shrews: Macroscelides and Elephantulus) were examined and compared, using micro-computed tomography and light microscopy. Middle ear cavity expansion has occurred in members of all three groups, apparently in association with an essentially 'freely mobile' ossicular morphology and the development of bony tubes for the middle ear arteries. Cavity expansion can occur in different ways, resulting in different subcavity patterns even between different species of gerbils. Having enlarged middle ear cavities aids low-frequency audition, and several adaptive advantages of low-frequency hearing to small desert mammals have been proposed. However, while Macroscelides was found here to have middle ear cavities so large that together they exceed brain volume, the bullae of Elephantulus are considerably smaller. Why middle ear cavities are enlarged in some desert species but not others remains unclear, but it may relate to microhabitat. © 2015 Anatomical Society.

Major evolutionary transitions and innovations: the tympanic middle ear

PubMed Central

2017-01-01

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

Modeling Analysis of Biomechanical Changes of Middle Ear and Cochlea in Otitis Media

NASA Astrophysics Data System (ADS)

Gan, Rong Z.; Zhang, Xiangming; Guan, Xiying

2011-11-01

A comprehensive finite element (FE) model of the human ear including the ear canal, middle ear, and spiral cochlea was developed using histological sections of human temporal bone. The cochlea was modeled with three chambers separated by the basilar membrane and Reissner's membrane and filled with perilymphatic fluid. The viscoelastic material behavior was applied to middle ear soft tissues based on dynamic measurements of tissues in our lab. The model was validated using the experimental data obtained in human temporal bones and then used to simulate various stages of otitis media (OM) including the changes of morphology, mechanical properties, pressure, and fluid level in the middle ear. Function alterations of the middle ear and cochlea in OM were derived from the model and compared with the measurements from temporal bones. This study indicates that OM can be simulated in the FE model to predict the hearing loss induced by biomechanical changes of the middle ear and cochlea.

THE EFFECTS OF RADIATION THERAPY ON THE EAR WITH PARTICULAR REFERENCE TO RADIATION OTITIS MEDIA

DOE Office of Scientific and Technical Information (OSTI.GOV)

Borsanyi, S.J.

Between l957 and 1961 over l00 patients who were treated by a Co/sup 60/ teletherapy unit for malignant tumors of the head and neck were observed. The ears were included in the field of irradiation (4000 to 6000 r to the region of the inner ear). Between 50 and 60% of the patients developed ear symptoms during or shortly after completion of treatment. The most common symptoms were a sensation of fullness in the ear, some loss of hearing, earache, and tinnitus. Examination of ears revealed mild to moderate hyperemia of ear drums, with slight retraction in eariy stages andmore » bulging at iater stages. There was a moderate conductive hearing loss also. This disease entity is termed radiation otitis media and its pathophysiologic mechanism is similar to that of serous otitis media. Sterile fluid fills the middle ear, containing also some desquamated epithelial cells. Radiation otitis media usually clears up in a few weeks after the completion of treatment. In the management of this condition, vasoconstrictors, mild analgesics, and gentie politzeration were sufficient. However, in a few cases bacterial invasion of the sterile fluid occurred, resulting in purulent otitis media which required the use of antibiotics. Hearing of 20 patients was tested at weekly intervals during and after the completion of radiation. Cut of the 40 ears, 16 showed a conductive hearing loss, averaging 20 db. Six ears showed a worsening of the original loss of preceptive hearing. However, this was also primarily due to the development of a conductive component. There were no microscopicaiiy detectable immediate changes in the cochlea or labyrinth exposed to radiation in cancerocidal doses. (H.H.D.)« less

Development and standardization of Arabic words in noise test in Egyptian children.

PubMed

Abdel Rahman, Tayseer Taha

2018-05-01

To develop and establish norms of Arabic Words in Noise test in Egyptian children. Total number of participants was 152 with normal hearing and ranging in age from 5 to 12 years. They are subdivided into two main groups (standardization group) which comprised 120 children with normal scholastic achievement and (application group) which comprised 32 children with different types of central auditory processing disorders. Arabic version of both Speech perception in noise (SPIN) and Words in Noise (WIN) tests were presented in each ear at zero signal to-noise ratio (SNR) using ipsilateral Cafeteria noise fixed at 50 dB sensation level (dBSL). The least performance in WIN test occurred between 5 and 7 years and highest scores from 9 to 12 years. However, no statistically significant difference was found among the three standardization age groups. Moreover, no statistically significant difference was found between the right and left ears scores or among the three lists. When the WIN test was compared to SPIN test in children with and without abnormal SPIN scores it showed highly consistent results except in children suffering from memory deficit reflecting that WIN test is more accurate than SPIN in this group of children. The Arabic WIN test can be used in children as young as 5 years. Also, it can be a good cross check test with SPIN test or used to follow up children after rehabilitation program in hearing impaired children or follow up after central auditory remediation of children with selective auditory attention deficit. Copyright © 2017. Published by Elsevier B.V.

Electrocardiogram Screening in Children with Congenital Sensorineural Hearing Loss: Prevalence and Follow-up of Abnormalities.

PubMed

Farzal, Zainab; Walsh, Jonathan; Ahmad, Faisal I; Roberts, Jason; Ferns, Sunita J; Zdanski, Carlton J

2018-03-01

Objective The purpose is to determine the prevalence of electrocardiogram (ECG) abnormalities, including borderline and prolonged QT, among screened children with sensorineural hearing loss (SNHL) and to analyze their subsequent medical workup. Study Design Institutional Review Board-approved case series with chart review. Setting Tertiary academic center. Subjects and Methods Cases from 1996 to 2014 involving pediatric patients (N = 1994) with SNHL were analyzed. Abnormal ECGs were categorized as borderline/prolonged QT or other. A board-certified pediatric cardiologist retrospectively determined the clinical significance of ECG changes. For follow-up analysis, children with heart disease, known syndromes, or inaccessible records were excluded. Results Among 772 children who had ECGs, 215 (27.8%) had abnormal results: 35 (4.5%) with QT abnormalities and 180 (23.3%) with other abnormalities. For children with QT abnormalities meeting inclusion criteria (n = 30), follow-up measures included cardiology referral (46.6%), repeat ECG by ear, nose, and throat (ENT) specialist (20%), clearance by ENT specialist with clinical correlation and/or comparison with old ECGs (20%), and pediatrician follow-up (6.7%). Documentation of further workup by ENT or referral was absent for 6.7%. For children with other ECG changes meeting inclusion criteria (n = 136), abnormalities were documented for 57 (41.9%); normal QT without other abnormality was documented for 18 (13.2%). The most common follow-up referrals were to pediatricians (16.9%) and cardiologists (10.3%). Among patients with clinically significant non-QT abnormalities mandating further evaluation (n = 122), 38 (31.1%) had documented follow-up in medical records. Conclusion There is a high prevalence of ECG abnormalities among children with congenital SNHL. If findings are confirmed by future studies, screening should be considered for congenital unilateral or bilateral SNHL, regardless of severity. We describe a standardized protocol for ECG screening/follow-up.

Programmed cell senescence during mammalian embryonic development.

PubMed

Muñoz-Espín, Daniel; Cañamero, Marta; Maraver, Antonio; Gómez-López, Gonzalo; Contreras, Julio; Murillo-Cuesta, Silvia; Rodríguez-Baeza, Alfonso; Varela-Nieto, Isabel; Ruberte, Jesús; Collado, Manuel; Serrano, Manuel

2013-11-21

Cellular senescence disables proliferation in damaged cells, and it is relevant for cancer and aging. Here, we show that senescence occurs during mammalian embryonic development at multiple locations, including the mesonephros and the endolymphatic sac of the inner ear, which we have analyzed in detail. Mechanistically, senescence in both structures is strictly dependent on p21, but independent of DNA damage, p53, or other cell-cycle inhibitors, and it is regulated by the TGF-β/SMAD and PI3K/FOXO pathways. Developmentally programmed senescence is followed by macrophage infiltration, clearance of senescent cells, and tissue remodeling. Loss of senescence due to the absence of p21 is partially compensated by apoptosis but still results in detectable developmental abnormalities. Importantly, the mesonephros and endolymphatic sac of human embryos also show evidence of senescence. We conclude that the role of developmentally programmed senescence is to promote tissue remodeling and propose that this is the evolutionary origin of damage-induced senescence. Copyright © 2013 Elsevier Inc. All rights reserved.

Nest success, cause-specific nest failure, and hatchability of aquatic birds at selenium-contaminated Kesterson Reservoir and a reference site

USGS Publications Warehouse

Ohlendorf, Harry M.; Hothem, Roger L.; Welsh, Daniel

1989-01-01

During 1983-1985, we studied the reproductive success of several species of aquatic birds (coots, ducks, shorebirds, and grebes) nesting at two sites in Merced County, California: a selenium-contaminated site (Kesterson Reservoir) and a nearby reference site (Volta Wildlife Area). We used a computer program (MICROMORT) developed for the analysis of radiotelemetry data (Heisey and Fuller 1985) to estimate nest success and cause-specific failure rates, and then compared these parameters and hatchability between sites and among years. Nest success and causes of failure varied by species, site, and year. The most important causes of nest failure were usually predation, desertion, and water-level changes. However, embryotoxicosis (mortality, deformity, and lack of embryonic development) was the most important cause of nest failure in Eared Grebes (Podiceps nigricollis) at Kesterson Reservoir. Embryotoxicosis also reduced the hatchability of eggs of all other species at Kesterson in one or more years; embryonic mortality occurred rarely at Volta, and abnormalities were not observed.

Otophyma: a case report and review of the literature of lymphedema (elephantiasis) of the ear.

PubMed

Carlson, J Andrew; Mazza, Jill; Kircher, Kenneth; Tran, Tien Anh

2008-02-01

Phymas (swellings, masses, or bulbs) are considered the end-stage of rosacea and mostly affect the nose (rhinophyma), and rarely involve the chin (gnatophyma), the cheek (metophyma), eyelids (blepharophyma), or ears (otophyma). Herein, we report the case of a 57-year-old man who developed unilateral enlargement of his left ear over 2 years. Biopsy revealed changes of rosaceous lymphedema associated with Demodex infestation. Corticosteroid and minocycline therapies resulted in partial reduction of the ear enlargement. Literature review examining for cases of lymphedema (elephantiasis) of the ear revealed that chronic inflammatory disorders (rosacea (most frequent), psoriasis, eczema), bacterial cellulitis (erysipelas), pediculosis, trauma, and primary (congenital) lymphedema can all lead to localized, lymphedematous enlargement of the ear. Depending on the severity, medical treatment directed at the inflammatory condition for mild, diffuse enlargement to surgical debulking for extensive diffuse enlargement or tumor formation can improve the signs and symptoms of otophyma. Decreased immune surveillance secondary to rosaceous lymphedema may explain why Demodex infestation is common in rosacea and support the suspicion that phymatous skin is predisposed to skin cancer development.

Ear biometrics for patient identification in global health: a cross-sectional study to test the feasibility of a simplified algorithm.

PubMed

Ragan, Elizabeth J; Johnson, Courtney; Milton, Jacqueline N; Gill, Christopher J

2016-11-02

One of the greatest public health challenges in low- and middle-income countries (LMICs) is identifying people over time and space. Recent years have seen an explosion of interest in developing electronic approaches to addressing this problem, with mobile technology at the forefront of these efforts. We investigate the possibility of biometrics as a simple, cost-efficient, and portable solution. Common biometrics approaches include fingerprinting, iris scanning and facial recognition, but all are less than ideal due to complexity, infringement on privacy, cost, or portability. Ear biometrics, however, proved to be a unique and viable solution. We developed an identification algorithm then conducted a cross sectional study in which we photographed left and right ears from 25 consenting adults. We then conducted re-identification and statistical analyses to identify the accuracy and replicability of our approach. Through principal component analysis, we found the curve of the ear helix to be the most reliable anatomical structure and the basis for re-identification. Although an individual ear allowed for high re-identification rate (88.3%), when both left and right ears were paired together, our rate of re-identification amidst the pool of potential matches was 100%. The results of this study have implications on future efforts towards building a biometrics solution for patient identification in LMICs. We provide a conceptual platform for further investigation into the development of an ear biometrics identification mobile application.

Head circumference - a useful single parameter for skull volume development in cranial growth analysis?

PubMed

Martini, Markus; Klausing, Anne; Lüchters, Guido; Heim, Nils; Messing-Jünger, Martina

2018-01-10

The measurement of maximal head circumference is a standard procedure in the examination of childrens' cranial growth and brain development. The objective of the study was to evaluate the validity of maximal head circumference to cranial volume in the first year of life using a new method which includes ear-to-ear over the head distance and maximal cranial length measurement. 3D surface scans for cranial volume assessment were conducted in this method comparison study of 44 healthy Caucasian children (29 male, 15 female) at the ages of 4 and 12 months. Cranial volume increased from measurements made at 4 months to 12 months of age by an average of 1174 ± 106 to 1579 ± 79 ml. Maximal cranial circumference increased from 43.4 ± 9 cm to 46.9 ± 7 cm and the ear-to ear measurement increased from 26.3 ± 21 cm to 31.6 ± 18 cm at the same time points. There was a monotone association between maximal head circumference (HC) and increase in volume, yet a backwards inference from maximal circumference to the volume had a predictive value of only 78% (adjusted R 2 ). Including the additional measurement of distance from ear to ear strengthened the ability of the model to predict the true value attained to 90%. The addition of the parameter skull length appeared to be negligible. The results demonstrate that for a distinct improvement in the evaluation of a physiological cranial volume development, the additional measurement of the ear-to ear distance using a measuring tape is expedient, and, especially for cases with pathological skull changes, such as craniosynostosis, ought to be conducted.

Estimation of outer-middle ear transmission using DPOAEs and fractional-order modeling of human middle ear

NASA Astrophysics Data System (ADS)

Naghibolhosseini, Maryam

Our ability to hear depends primarily on sound waves traveling through the outer and middle ear toward the inner ear. Hence, the characteristics of the outer and middle ear affect sound transmission to/from the inner ear. The role of the middle and outer ear in sound transmission is particularly important for otoacoustic emissions (OAEs), which are sound signals generated in a healthy cochlea, and recorded by a sensitive microphone placed in the ear canal. OAEs are used to evaluate the health and function of the cochlea; however, they are also affected by outer and middle ear characteristics. To better assess cochlear health using OAEs, it is critical to quantify the impact of the outer and middle ear on sound transmission. The reported research introduces a noninvasive approach to estimate outer-middle ear transmission using distortion product otoacoustic emissions (DPOAEs). In addition, the role of the outer and middle ear on sound transmission was investigated by developing a physical/mathematical model, which employed fractional-order lumped elements to include the viscoelastic characteristics of biological tissues. Impedance estimations from wideband refectance measurements were used for parameter fitting of the model. The model was validated comparing its estimates of the outer-middle ear sound transmission with those given by DPOAEs. The outer-middle ear transmission by the model was defined as the sum of forward and reverse outer-middle ear transmissions. To estimate the reverse transmission by the model, the probe-microphone impedance was calculated through estimating the Thevenin-equivalent circuit of the probe-microphone. The Thevenin-equivalent circuit was calculated using measurements in a number of test cavities. Such modeling enhances our understanding of the roles of different parts of the outer and middle ear and how they work together to determine their function. In addition, the model would be potentially helpful in diagnosing pathologies of cochlear or middle ear origin.

Facial palsy following trauma to the external ear: 3 case reports.

PubMed

Vögelin, E; Jones, B M

1997-12-01

We report two children and a young adult who developed unilateral facial palsy shortly after injury to the external ear. In two instances the paralysis followed a prominent ear correction and in the other a laceration to the concha. The trauma-triggered facial palsy was most likely idiopathic although the anatomy of the facial nerve near the ear leads one to speculate on a possible pathway of a virally induced palsy (Bell's palsy). Each patient recovered over a period of 6 months.

Bone-conduction circuit model for chinchilla part I: Defining parameters by fitting to air-conduction data

NASA Astrophysics Data System (ADS)

Bowers, Peter; Rosowski, John J.

2018-05-01

An air-conduction circuit model that will serve as the basis for a model of bone-conduction hearing is developed for chinchilla. The lumped-element model is based on the classic Zwislocki model of the human middle ear. Model parameters are fit to various measurements of chinchilla middle-ear transfer functions and impedances. The model is in agreement with studies of the effects of middle-ear cavity holes in experiments that require access to the middle-ear air space.

The zinc finger transcription factor Gfi1, implicated in lymphomagenesis, is required for inner ear hair cell differentiation and survival

NASA Technical Reports Server (NTRS)

Wallis, Deeann; Hamblen, Melanie; Zhou, Yi; Venken, Koen J T.; Schumacher, Armin; Grimes, H. Leighton; Zoghbi, Huda Y.; Orkin, Stuart H.; Bellen, Hugo J.

2003-01-01

Gfi1 was first identified as causing interleukin 2-independent growth in T cells and lymphomagenesis in mice. Much work has shown that Gfi1 and Gfi1b, a second mouse homolog, play pivotal roles in blood cell lineage differentiation. However, neither Gfi1 nor Gfi1b has been implicated in nervous system development, even though their invertebrate homologues, senseless in Drosophila and pag-3 in C. elegans are expressed and required in the nervous system. We show that Gfi1 mRNA is expressed in many areas that give rise to neuronal cells during embryonic development in mouse, and that Gfi1 protein has a more restricted expression pattern. By E12.5 Gfi1 mRNA is expressed in both the CNS and PNS as well as in many sensory epithelia including the developing inner ear epithelia. At later developmental stages, Gfi1 expression in the ear is refined to the hair cells and neurons throughout the inner ear. Gfi1 protein is expressed in a more restricted pattern in specialized sensory cells of the PNS, including the eye, presumptive Merkel cells, the lung and hair cells of the inner ear. Gfi1 mutant mice display behavioral defects that are consistent with inner ear anomalies, as they are ataxic, circle, display head tilting behavior and do not respond to noise. They have a unique inner ear phenotype in that the vestibular and cochlear hair cells are differentially affected. Although Gfi1-deficient mice initially specify inner ear hair cells, these hair cells are disorganized in both the vestibule and cochlea. The outer hair cells of the cochlea are improperly innervated and express neuronal markers that are not normally expressed in these cells. Furthermore, Gfi1 mutant mice lose all cochlear hair cells just prior to and soon after birth through apoptosis. Finally, by five months of age there is also a dramatic reduction in the number of cochlear neurons. Hence, Gfi1 is expressed in the developing nervous system, is required for inner ear hair cell differentiation, and its loss causes programmed cell death.

High temperature effects on photosynthate partitioning and sugar metabolism during ear expansion in maize (Zea mays L.) genotypes.

PubMed

Suwa, Ryuichi; Hakata, Hiroaki; Hara, Hiromichi; El-Shemy, Hany A; Adu-Gyamfi, Joseph J; Nguyen, Nguyen Tran; Kanai, Synsuke; Lightfoot, David A; Mohapatra, Pravat K; Fujita, Kounosuke

2010-01-01

Short hot and dry spells before, or during, silking have an inordinately large effect on maize (Zea mays L.; corn) grain yield. New high yielding genotypes could be developed if the mechanism of yield loss were more fully understood and new assays developed. The aim here was to determine the effects of high temperature (35/27 degrees C) compared to cooler (25/18 degrees C) temperatures (day/night). Stress was applied for a 14 d-period during reproductive stages prior to silking. Effects on whole plant biomass, ear development, photosynthesis and carbohydrate metabolism were measured in both dent and sweet corn genotypes. Results showed that the whole plant biomass was increased by the high temperature. However, the response varied among plant parts; in leaves and culms weights were slightly increased or stable; cob weights decreased; and other ear parts of dent corn also decreased by high temperature. Photosynthetic activity was not affected by the treatments. The (13)C export rate from an ear leaf was decreased by the high temperature treatment. The amount of (13)C partitioning to the ears decreased more than to other plant parts by the high temperature. Within the ear decreases were greatest in the cob than the shank within an ear. Sugar concentrations in both hemicellulose and cellulose fractions of cobs in sweet corn were decreased by high temperature, and the hemicellulose fraction in the shank also decreased. In dent corn there was no reduction of sugar concentration except in the in cellulose fraction, suggesting that synthesis of cell-wall components is impaired by high temperatures. The high temperature treatment promoted the growth of vegetative plant parts but reduced ear expansion, particularly suppression of cob extensibility by impairing hemicellulose and cellulose synthesis through reduction of photosynthate supply. Therefore, plant biomass production was enhanced and grain yield reduced by the high temperature treatment due to effects on sink activity rather than source activity. Heat resistant ear development can be targeted for genetic improvement. Copyright 2010 Elsevier Masson SAS. All rights reserved.

Traumatic neuroma: a late complication of otoplasty.

PubMed

Lwin, Christine T-T; Birch, Jeremy; Giele, Henk

2007-01-01

We report on the development of traumatic ear lobe neuromas as a late complication of bilateral otoplasty performed 16 years previously. To our knowledge, this is the first and only described case of traumatic neuromas arising as a late complication of otoplasty in the English literature. Traumatic neuroma should be considered in the differential diagnosis of any nodular lesions that develop within the external ear in patients who have had otoplasty or any other form of external ear surgery or trauma.

Macrophage migration inhibitory factor acts as a neurotrophin in the developing inner ear.

PubMed

Bank, Lisa M; Bianchi, Lynne M; Ebisu, Fumi; Lerman-Sinkoff, Dov; Smiley, Elizabeth C; Shen, Yu-chi; Ramamurthy, Poornapriya; Thompson, Deborah L; Roth, Therese M; Beck, Christine R; Flynn, Matthew; Teller, Ryan S; Feng, Luming; Llewellyn, G Nicholas; Holmes, Brandon; Sharples, Cyrrene; Coutinho-Budd, Jaeda; Linn, Stephanie A; Chervenak, Andrew P; Dolan, David F; Benson, Jennifer; Kanicki, Ariane; Martin, Catherine A; Altschuler, Richard; Koch, Alisa E; Koch, Alicia E; Jewett, Ethan M; Germiller, John A; Barald, Kate F

2012-12-01

This study is the first to demonstrate that macrophage migration inhibitory factor (MIF), an immune system 'inflammatory' cytokine that is released by the developing otocyst, plays a role in regulating early innervation of the mouse and chick inner ear. We demonstrate that MIF is a major bioactive component of the previously uncharacterized otocyst-derived factor, which directs initial neurite outgrowth from the statoacoustic ganglion (SAG) to the developing inner ear. Recombinant MIF acts as a neurotrophin in promoting both SAG directional neurite outgrowth and neuronal survival and is expressed in both the developing and mature inner ear of chick and mouse. A MIF receptor, CD74, is found on both embryonic SAG neurons and adult mouse spiral ganglion neurons. Mif knockout mice are hearing impaired and demonstrate altered innervation to the organ of Corti, as well as fewer sensory hair cells. Furthermore, mouse embryonic stem cells become neuron-like when exposed to picomolar levels of MIF, suggesting the general importance of this cytokine in neural development.

Recent advances in therapeutics and drug delivery for the treatment of inner ear diseases: a patent review (2011-2015).

PubMed

Nguyen, Kim; Kempfle, Judith S; Jung, David H; McKenna, Charles E

2017-02-01

Inner ear disorders such as hearing loss, tinnitus, and Ménière's disease significantly impact the quality of life of affected individuals. Treatment of such disorders is an ongoing challenge. Current clinical approaches relieve symptoms but do not fully restore hearing, and the search for more effective therapeutic methods represents an area of urgent current interest. Areas covered: Thirty four patents and patent applications published from 2011 to 2015 were selected from the database of the U.S. Patent and Trademark Office (USPTO) and World Intellectual Property Organization (WIPO), covering new approaches for the treatment of inner ear disorders described in the patent literature: 1) identification of new therapeutic agents, 2) development of sustained release formulations, and 3) medical devices that facilitate delivery of such agents to the inner ear. Expert opinion: The search for effective treatments of inner ear disorders is ongoing. Increased understanding of the molecular mechanisms of hearing loss, Ménière's disease, and tinnitus is driving development of new therapeutic agents. However, delivery of these agents to the inner ear is a continuing challenge. At present, combination of a suitable drug with an appropriate mode of drug delivery is the key focus of innovative research to cure inner ear disorders.

miR-124 promotes the neuronal differentiation of mouse inner ear neural stem cells

PubMed Central

Jiang, Di; Du, Jintao; Zhang, Xuemei; Zhou, Wei; Zong, Lin; Dong, Chang; Chen, Kaitian; Chen, Yu; Chen, Xihui; Jiang, Hongyan

2016-01-01

MicroRNAs (miRNAs or miRs) act as key regulators in neuronal development, synaptic morphogenesis and plasticity. However, their role in the neuronal differentiation of inner ear neural stem cells (NSCs) remains unclear. In this study, 6 miRNAs were selected and their expression patterns during the neuronal differentiation of inner ear NSCs were examined by RT-qPCR. We demonstrated that the culture of spiral ganglion stem cells present in the inner ears of newborn mice gave rise to neurons in vitro. The expression patterns of miR-124, miR-132, miR-134, miR-20a, miR-17-5p and miR-30a-5p were examined during a 14-day neuronal differentiation period. We found that miR-124 promoted the neuronal differentiation of and neurite outgrowth in mouse inner ear NSCs, and that the changes in the expression of tropomyosin receptor kinase B (TrkB) and cell division control protein 42 homolog (Cdc42) during inner ear NSC differentiation were associated with miR-124 expression. Our findings indicate that miR-124 plays a role in the neuronal differentiation of inner ear NSCs. This finding may lead to the development of novel strategies for restoring hearing in neurodegenerative diseases. PMID:28025992

Evolution of vertebrate mechanosensory hair cells and inner ears: toward identifying stimuli that select mutation driven altered morphologies

PubMed Central

Fritzsch, Bernd; Straka, Hans

2014-01-01

Among the major distance senses of vertebrates, the ear is unique in its complex morphological changes during evolution. Conceivably, these changes enable the ear to adapt toward sensing various physically well-characterized stimuli. This review develops a scenario that integrates sensory cell with organ evolution. We propose that molecular and cellular evolution of the vertebrate hair cells occurred prior to the formation of the vertebrate ear. We previously proposed that the genes driving hair cell differentiation, were aggregated in the otic region through developmental re-patterning that generated a unique vertebrate embryonic structure, the otic placode. In agreement with the presence of graviceptive receptors in many vertebrate outgroups, it is likely that the vertebrate ear originally functioned as a simple gravity-sensing organ. Based on the rare occurrence of angular acceleration receptors in vertebrate outgroups, we further propose that the canal system evolved with a more sophisticated ear morphogenesis. This evolving morphogenesis obviously turned the initial otocyst into a complex set of canals and recesses, harboring multiple sensory epithelia each adapted to the acquisition of a specific aspect of a given physical stimulus. As support for this evolutionary progression, we provide several details of the molecular basis of ear development. PMID:24281353

A short-wave infrared otoscope for middle ear disease diagnostics (Conference Presentation)

NASA Astrophysics Data System (ADS)

Carr, Jessica A.; Valdez, Tulio; Bruns, Oliver; Bawendi, Moungi

2016-02-01

Otitis media, a range of inflammatory conditions of the middle ear, is the second most common illness diagnosed in children. However, the diagnosis can be challenging, particularly in pediatric patients. Otitis media is commonly over-diagnosed and over-treated and has been identified as one of the primary factors in increased antibiotic resistance. We describe the development of a short-wave infrared (SWIR) otoscope for objective middle ear effusion diagnosis. The SWIR otoscope can unambiguously detect the presence of middle ear fluid based on its strong light absorption in the SWIR. This absorption causes a stark, visual contrast between the presence and absence of fluid behind the tympanic membrane. Additionally, when there is no middle ear fluid, the deeper tissue penetration of SWIR light allows the SWIR otoscope to better visualize middle ear anatomy through the tympanic membrane than is possible with visible light. We demonstrate that in healthy, adult human ears, SWIR otoscopy can image a range of middle ear anatomy, including landmarks of the entire ossicular chain, the promontory, the round window niche, and the chorda tympani. We suggest that SWIR otoscopy can provide valuable diagnostic information complementary to that provided by visible pneumotoscopy in the diagnosis of middle ear effusions, otitis media, and other maladies of the middle ear.

Deriving habitat models for northern long-eared bats from historical detection data: a case study using the Fernow Experimental Forest

Treesearch

W. Mark Ford; Alexander Silvis; Jane L. Rodrigue; Andrew B. Kniowski; Joshua B. Johnson

2016-01-01

The listing of the northern long-eared bat (Myotis septentrionalis) as federally threatened under the Endangered Species Act following severe population declines from white-nose syndrome presents considerable challenges to natural resource managers. Because the northern long-eared bat is a forest habitat generalist, development of effective...

Selective forces on origin, adaptation and reduction of tympanal ears in insects.

PubMed

Strauß, Johannes; Stumpner, Andreas

2015-01-01

Insect ears evolved many times independently. As a consequence, a striking diversity exists in the location, construction and behavioural implementation of ears. In this review, we first summarise what is known about the evolutionary origin of ears and the presumed precursor organs in the various insect groups. Thereafter, we focus on selective forces for making and keeping an ear: we discuss detecting and localising predators and conspecifics, including establishing new "private" channels for intraspecific communication. More advanced aspects involve judging the distance of conspecifics, or assessing individual quality from songs which makes auditory processing a means for exerting sexual selection on mating partners. We try to identify negative selective forces, mainly in the context of energy expenditure for developing and keeping an ear, but also in conjunction with acoustic communication, which incorporates risks like eavesdropping by predators and parasitoids. We then discuss balancing pressures, which might oppose optimising an ear for a specific task (when it serves different functions, for example). Subsequently, we describe various scenarios that might have led to a reduction or complete loss of ears in evolution. Finally, we describe cases of sex differences in ears and potential reasons for their appearance.

Otologic manifestations of Larsen syndrome.

PubMed

Marques, Letícia Helena de Sousa; Martins, Daniela Vieira; Juares, Gabriel Liria; Lorenzetti, Fábio Tadeu Moura; Monsanto, Rafael da Costa

2017-10-01

To describe and discuss otologic manifestations of Larsen syndrome, based on a case report and a systematic review of the literature. We performed a PubMED database search, and we selected studies reporting otolaryngologic manifestations secondary to Larsen syndrome. The selected articles were read in full, and three researchers independently extracted data from the studies. In parallel, we report the case of a 14-year-old patient who had hearing loss secondary to Larsen syndrome. Fifteen studies met our selection criteria. Seven studies reported hearing loss in patients with Larsen syndrome (4 had conductive hearing loss and 3 had mixed hearing loss). The conductive hearing loss may be secondary to ossicular malformations and/or middle ear effusions. Other causes for conductive hearing loss are mesenchymal remnants in the middle ear, Eustachian tube dysfunction, and cleft palate. Surgical management of the hearing loss is possible in selected cases, although the surgical and anesthetic risks should be considered. Hearing aids seem to be safe and effective treatment options for the hearing loss secondary to Larsen syndrome. Although rare, patients with Larsen syndrome may have hearing loss. The most frequent type of deficit is conductive, either secondary to malformation of the ossicles or middle ear effusion. Possible surgical correction of these abnormalities should be weighed against the anesthetic risks of these patients. Copyright © 2017 Elsevier B.V. All rights reserved.

Possible association of first and high birth order of pregnant women with the risk of isolated congenital abnormalities in Hungary - a population-based case-matched control study.

PubMed

Csermely, Gyula; Susánszky, Éva; Czeizel, Andrew E; Veszprémi, Béla

2014-08-01

In epidemiological studies at the estimation of risk factors in the origin of specified congenital abnormalities in general birth order (parity) is considered as confounder. The aim of this study was to analyze the possible association of first and high (four or more) birth order with the risk of congenital abnormalities in a population-based case-matched control data set. The large dataset of the Hungarian Case-Control Surveillance of Congenital Abnormalities included 21,494 cases with different isolated congenital abnormality and their 34,311 matched controls. First the distribution of birth order was compared of 24 congenital abnormality groups and their matched controls. In the second step the possible association of first and high birth order with the risk of congenital abnormalities was estimated. Finally some subgroups of neural-tube defects, congenital heart defects and abdominal wall's defects were evaluated separately. A higher risk of spina bifida aperta/cystica, esophageal atresia/stenosis and clubfoot was observed in the offspring of primiparous mothers. Of 24 congenital abnormality groups, 14 had mothers with larger proportion of high birth order. Ear defects, congenital heart defects, cleft lip± palate and obstructive defects of urinary tract had a linear trend from a lower proportion of first born cases to the larger proportion of high birth order. Birth order showed U-shaped distribution of neural-tube defects and clubfoot, i.e. both first and high birth order had a larger proportion in cases than in their matched controls. Birth order is a contributing factor in the origin of some isolated congenital abnormalities. The higher risk of certain congenital abnormalities in pregnant women with first or high birth order is worth considering in the clinical practice, e.g. ultrasound scanning. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

Acceleration induced water removal from ear canals.

NASA Astrophysics Data System (ADS)

Kang, Hosung; Averett, Katelee; Jung, Sunghwan

2017-11-01

Children and adults commonly experience having water trapped in the ear canals after swimming. To remove the water, individuals will shake their head sideways. Since a child's ear canal has a smaller diameter, it requires more acceleration of the head to remove the trapped water. In this study, we theoretically and experimentally investigated the acceleration required to break the surface meniscus of the water in artificial ear canals and hydrophobic-coated glass tubes. In experiments, ear canal models were 3D-printed from a CT-scanned human head. Also, glass tubes were coated with silane to match the hydrophobicity in ear canals. Then, using a linear stage, we measured the acceleration values required to forcefully eject the water from the artificial ear canals and glass tubes. A theoretical model was developed to predict the critical acceleration at a given tube diameter and water volume by using a modified Rayleigh-Taylor instability. Furthermore, this research can shed light on the potential of long-term brain injury and damage by shaking the head to push the water out of the ear canal. This research was supported by National Science Foundation Grant CBET-1604424.

Crystallographic texture and earing behavior analysis for different second cold reductions of double-reduction tinplate

NASA Astrophysics Data System (ADS)

Liao, Lu-hai; Zheng, Xiao-fei; Kang, Yong-lin; Liu, Wei; Yan, Yan; Mo, Zhi-ying

2018-06-01

Since the production of tinplate with non-earing properties is difficult, especially when it is produced via the double-reduction process, the optimal degree of second cold reduction is particularly important for achieving desirable drawing properties. The evolution of texture and the earing propensity of double-reduction tinplate with different extents of second reduction were investigated in this study. Optical microscopy and scanning electron microscopy were used to observe the changes in the microstructure at various extents of reduction. Two common testing methods, X-ray diffraction (XRD) and electron backscatter diffraction, were used to investigate the texture of the specimens, which revealed the effects of deformation percentage on the final texture development and the change in the grain boundary. The earing rate was determined via earing tests involving measurement of the height of any ear. The results obtained from both XRD analyses and earing tests revealed the same ideal value for the second cold reduction on the basis of the relationship between crystallographic texture and the degree of earing.

In vivo imaging of middle-ear and inner-ear microstructures of a mouse guided by SD-OCT combined with a surgical microscope

PubMed Central

Cho, Nam Hyun; Jang, Jeong Hun; Jung, Woonggyu; Kim, Jeehyun

2014-01-01

We developed an augmented-reality system that combines optical coherence tomography (OCT) with a surgical microscope. By sharing the common optical path in the microscope and OCT, we could simultaneously acquire OCT and microscope views. The system was tested to identify the middle-ear and inner-ear microstructures of a mouse. Considering the probability of clinical application including otorhinolaryngology, diseases such as middle-ear effusion were visualized using in vivo mouse and OCT images simultaneously acquired through the eyepiece of the surgical microscope during surgical manipulation using the proposed system. This system is expected to realize a new practical area of OCT application. PMID:24787787

Investigation of middle ear anatomy and function with combined video otoscopy-phase sensitive OCT

PubMed Central

Park, Jesung; Cheng, Jeffrey T.; Ferguson, Daniel; Maguluri, Gopi; Chang, Ernest W.; Clancy, Caitlin; Lee, Daniel J.; Iftimia, Nicusor

2016-01-01

We report the development of a novel otoscopy probe for assessing middle ear anatomy and function. Video imaging and phase-sensitive optical coherence tomography are combined within the same optical path. A sound stimuli channel is incorporated as well to study middle ear function. Thus, besides visualizing the morphology of the middle ear, the vibration amplitude and frequency of the eardrum and ossicles are retrieved as well. Preliminary testing on cadaveric human temporal bone models has demonstrated the capability of this instrument for retrieving middle ear anatomy with micron scale resolution, as well as the vibration of the tympanic membrane and ossicles with sub-nm resolution. PMID:26977336

Usher syndrome: hearing loss, retinal degeneration and associated abnormalities

PubMed Central

Mathur, Pranav; Yang, Jun

2014-01-01

Usher syndrome (USH), clinically and genetically heterogeneous, is the leading genetic cause of combined hearing and vision loss. USH is classified into three types, based on the hearing and vestibular symptoms observed in patients. Sixteen loci have been reported to be involved in the occurrence of USH and atypical USH. Among them, twelve have been identified as causative genes and one as a modifier gene. Studies on the proteins encoded by these USH genes suggest that USH proteins interact among one another and function in multiprotein complexes in vivo. Although their exact functions remain enigmatic in the retina, USH proteins are required for the development, maintenance and function of hair bundles, which are the primary mechanosensitive structure of inner ear hair cells. Despite the unavailability of a cure, progress has been made to develop effective treatments for this disease. In this review, we focus on the most recent discoveries in the field with an emphasis on USH genes, protein complexes and functions in various tissues as well as progress toward therapeutic development for USH. PMID:25481835

Usher syndrome: Hearing loss, retinal degeneration and associated abnormalities.

PubMed

Mathur, Pranav; Yang, Jun

2015-03-01

Usher syndrome (USH), clinically and genetically heterogeneous, is the leading genetic cause of combined hearing and vision loss. USH is classified into three types, based on the hearing and vestibular symptoms observed in patients. Sixteen loci have been reported to be involved in the occurrence of USH and atypical USH. Among them, twelve have been identified as causative genes and one as a modifier gene. Studies on the proteins encoded by these USH genes suggest that USH proteins interact among one another and function in multiprotein complexes in vivo. Although their exact functions remain enigmatic in the retina, USH proteins are required for the development, maintenance and function of hair bundles, which are the primary mechanosensitive structure of inner ear hair cells. Despite the unavailability of a cure, progress has been made to develop effective treatments for this disease. In this review, we focus on the most recent discoveries in the field with an emphasis on USH genes, protein complexes and functions in various tissues as well as progress toward therapeutic development for USH. Copyright © 2014 Elsevier B.V. All rights reserved.

Apoplastic infusion of sucrose into stem internodes during female flowering does not increase grain yield in maize plants grown under nitrogen-limiting conditions.

PubMed

Peng, Yunfeng; Li, Chunjian; Fritschi, Felix B

2013-08-01

Nitrogen (N) limitation reduces leaf growth and photosynthetic rates of maize (Zea mays), and constrains photosynthate translocation to developing ears. Additionally, the period from about 1 week before to 2 weeks after silking is critical for establishing the reproductive sink capacity necessary to attain maximum yield. To investigate the influence of carbohydrate availability in plants of differing N status, a greenhouse study was performed in which exogenous sucrose (Suc) was infused around the time of silking into maize stems grown under different N regimes. N deficiency significantly reduced leaf area, leaf longevity, leaf chlorophyll content and photosynthetic rate. High N-delayed leaf senescence, particularly of the six uppermost leaves, compared to the other two N treatments. While N application increased ear leaf soluble protein concentration, it did not influence glucose and suc concentrations. Interestingly, ear leaf starch concentration decreased with increasing N application. Infusion of exogenous suc tended to increase non-structural carbohydrate concentrations in the developing ears of all N treatments at silking and 6 days after silking. However, leaf photosynthetic rates were not affected by suc infusion, and suc infusion failed to increase grain yield in any N treatment. The lack of an effect of suc infusion on ear growth and the high ear leaf starch concentration of N-deficient maize, suggest that yield reduction under N deficiency may not be due to insufficient photosynthate availability to the developing ear during silking, and that yield reduction under N deficiency may be determined at an earlier growth stage. Copyright © Physiologia Plantarum 2012.

Evidence for autosomal dominant inheritance of ablepharon-macrostomia syndrome.

PubMed

Rohena, Luis; Kuehn, Devon; Marchegiani, Shannon; Higginson, Jason D

2011-04-01

Ablepharon-macrostomia syndrome (AMS) is characterized by absent or short eyelids, macrostomia, ear anomalies, absent lanugo and hair, redundant skin, abnormal genitalia, and developmental delay in two-thirds of the reported patients. Additional anomalies include dry skin, growth retardation, hearing loss, camptodactyly, hypertelorism, absent zygomatic arches, and umbilical abnormalities. We present the second familial case of ablepharon-macrostomia syndrome in a newborn female and her 22-year-old father making autosomal dominant inheritance more likely than the previously proposed autosomal recessive transmission for this disorder. These cases likely represent the 16th and 17th reported cases of AMS and the first case suspected on prenatal ultrasound. Additionally, the child shows more prominent features of the disorder when compared to her father documenting variable expression and possible anticipation. This article is a US Government work and, as such, is in the public domain in the United States of America. Published 2011 Wiley-Liss, Inc.

Otic drug delivery systems: formulation principles and recent developments.

PubMed

Liu, Xu; Li, Mingshuang; Smyth, Hugh; Zhang, Feng

2018-04-25

Disorders of the ear severely impact the quality of life of millions of people, but the treatment of these disorders is an ongoing, but often overlooked challenge particularly in terms of formulation design and product development. The prevalence of ear disorders has spurred significant efforts to develop new therapeutic agents, but perhaps less innovation has been applied to new drug delivery systems to improve the efficacy of ear disease treatments. This review provides a brief overview of physiology, major diseases, and current therapies used via the otic route of administration. The primary focuses are on the various administration routes and their formulation principles. The article also presents recent advances in otic drug deliveries as well as potential limitations. Otic drug delivery technology will likely evolve in the next decade and more efficient or specific treatments for ear disease will arise from the development of less invasive drug delivery methods, safe and highly controlled drug delivery systems, and biotechnology targeting therapies.

Evaluation of plasma fibrinogen concentration as a diagnostic indicator of inflammation in red-eared sliders (Trachemys scripta elegans).

PubMed

Moore, A Russell; Allender, Matthew C; Mitchell, Mark A; MacNeill, Amy L

2015-01-15

To critically evaluate plasma fibrinogen concentration as a diagnostic indicator of inflammation in red-eared sliders (Trachemys scripta elegans). Prospective induced-disease model and prospective cross-sectional study. Plasma samples from 12 purpose-bred red-eared sliders and 153 farm-raised red-eared sliders. A modification of the Jacobsson method was developed to measure fibrinogen concentration in platelet-poor plasma from red-eared sliders. Purpose-bred turtles had been inoculated with a ranavirus (n = 4) or sterile PBS solution (8) as part of another study. Farm-raised red-eared sliders were categorized as healthy (n = 138) or overtly ill (15) on the basis of physical examination findings at the time of blood sample collection. Samples from 124 of the 138 healthy red-eared sliders were used to establish a fibrinogen concentration reference interval as measured by the modified Jacobsson method. Fibrinogen concentrations in ranavirus-infected and physically ill turtles were compared with those of healthy turtles to determine whether fibrinogen concentration would be a useful diagnostic indicator of inflammation in red-eared sliders. The modified Jacobsson method was reliably used to measure fibrinogen concentration. The fibrinogen concentration reference interval from healthy reproductively active female red-eared sliders was right skewed. Fibrinogen concentration did not differ significantly between healthy red-eared sliders and ranavirus-infected or overtly ill red-eared sliders. A reference interval for red-eared slider plasma fibrinogen concentration was established and partitioned by sex to account for considerable right skewing observed for females. Fibrinogen concentration was not a useful indicator of inflammation in red-eared sliders with ranavirus infection or other overt illnesses.

Comparison of Microbiological Flora in the External Auditory Canal of Normal Ear and an Ear with Acute Otitis Externa.

PubMed

Ghanpur, Asheesh Dora; Nayak, Dipak Ranjan; Chawla, Kiran; Shashidhar, V; Singh, Rohit

2017-09-01

Acute Otitis Externa (AOE) is also known as swimmer's ear. Investigations initiated during World War II firmly established the role of bacteria in the aetiology of Acute Otitis Externa. To culture the microbiological flora of the normal ear and compare it with the flora causing AOE and to know the role of normal ear canal flora and anaerobes in the aetiology. A prospective observational study was conducted on 64 patients clinically diagnosed with unilateral AOE. Ear swabs were taken from both the ears. Microbiological flora was studied considering diseased ear as test ear and the normal ear as the control. Aerobic and anaerobic cultures were done. Severity of the disease was assessed by subjective and objective scores. Effect of topical treatment with ichthammol glycerine pack was assessed after 48 hours and scores were calculated again. Patients with scores < 4 after pack removal were started on systemic antibiotics and were assessed after seven days of antibiotics course. Data was analysed using Paired t-test, Wilcoxon signed ranks test and Chi-square test. A p-value < 0.05 was considered significant. Pseudomonas aeruginosa (33%) was the most common bacteria cultured from the ear followed by Methicillin Resistant Staphylococcus aureus (MRSA) (18%). Patients with anaerobic organism in the test ear had severe symptoms and needed systemic antibiotic therapy. Most of the cases may respond to empirical antibiotic therapy. In cases with severe symptoms and the ones refractory to empirical treatment, a culture from the ear canal will not be a tax on the patient. This helps in giving a better understanding about the disease, causative organisms and helps in avoiding the use of inappropriate antibiotics that usually result in developing resistant strains of bacteria.

Not All Inner Ears are the Same: Otolith Matrix Proteins in the Inner Ear of Sub-Adult Cichlid Fish, Oreochromis Mossambicus, Reveal Insights Into the Biomineralization Process.

PubMed

Weigele, Jochen; Franz-Odendaal, Tamara A; Hilbig, Reinhard

2016-02-01

The fish ear stones (otoliths) consist mainly of calcium carbonate and have lower amounts of a proteinous matrix. This matrix consists of macromolecules, which directly control the biomineralization process. We analyzed the composition of this proteinous matrix by mass spectrometry in a shotgun approach. For this purpose, an enhanced protein purification technique was developed that excludes any potential contamination of proteins from body fluids. Using this method we identified eight proteins in the inner ear of Oreochromis mossambicus. These include the common otolith matrix proteins (OMP-1, otolin-1, neuroserpin, SPARC and otoconin), and three proteins (alpha tectorin, otogelin and transferrin) not previously localized to the otoliths. Moreover, we were able to exclude the occurrence of two matrix proteins (starmaker and pre-cerebellin-like protein) known from other fish species. In further analyses, we show that the absence of the OMP starmaker corresponds to calcitic otoliths and that pre-cerebellin-like protein is not present at any stage during the development of the otoliths of the inner ear. This study shows O. mossambicus does not have all of the known otolith proteins indicating that the matrix proteins in the inner ear of fish are not the same across species. Further functional studies of the novel proteins we identified during otolith development are required. © 2015 Wiley Periodicals, Inc.

Tuberculous otitis media with mastoiditis and central nervous system involvement.

PubMed

Mongkolrattanothai, Kanokporn; Oram, Ronda; Redleaf, Miriam; Bova, Judy; Englund, Janet A

2003-05-01

Tuberculosis of the middle ear and mastoid is currently a rare disease in developed countries, but this disease still occurs and may cause serious consequences. We report a case of disseminated tuberculosis involving the middle ear, mastoid, lung and central nervous system. Tuberculosis should be considered in the differential diagnosis of chronic ear drainage, especially in young children.

New HRCT-based measurement of the human outer ear canal as a basis for acoustical methods.

PubMed

Grewe, Johanna; Thiele, Cornelia; Mojallal, Hamidreza; Raab, Peter; Sankowsky-Rothe, Tobias; Lenarz, Thomas; Blau, Matthias; Teschner, Magnus

2013-06-01

As the form and size of the external auditory canal determine its transmitting function and hence the sound pressure in front of the eardrum, it is important to understand its anatomy in order to develop, optimize, and compare acoustical methods. High-resolution computed tomography (HRCT) data were measured retrospectively for 100 patients who had received a cochlear implant. In order to visualize the anatomy of the auditory canal, its length, radius, and the angle at which it runs were determined for the patients’ right and left ears. The canal’s volume was calculated, and a radius function was created. The determined length of the auditory canal averaged 23.6 mm for the right ear and 23.5 mm for the left ear. The calculated auditory canal volume (Vtotal) was 0.7 ml for the right ear and 0.69 ml for the left ear. The auditory canal was found to be significantly longer in men than in women, and the volume greater. The values obtained can be employed to develop a method that represents the shape of the auditory canal as accurately as possible to allow the best possible outcomes for hearing aid fitting.

Tissue-specific roles of Tbx1 in the development of the outer, middle and inner ear, defective in 22q11DS patients

PubMed Central

Arnold, Jelena S.; Braunstein, Evan M.; Ohyama, Takahiro; Groves, Andrew K.; Adams, Joe C.; Brown, M. Christian; Morrow, Bernice E.

2007-01-01

Most 22q11.2 deletion syndrome (22q11DS) patients have middle and outer ear anomalies, whereas some have inner ear malformations. Tbx1, a gene hemizygously deleted in 22q11DS patients and required for ear development, is expressed in multiple tissues during embryogenesis. To determine the role of Tbx1 in the first pharyngeal pouch (PPI) in forming outer and middle ears, we tissue-specifically inactivated the gene using Foxg1-Cre. In the conditional mutants, PPI failed to outgrow, preventing the middle ear bone condensations from forming. Tbx1 was also inactivated in the otic vesicle (OV), resulting in the failure of inner ear sensory organ formation, and in duplication of the cochleovestibular ganglion (CVG). Consistent with the anatomical defects, the sensory genes, Otx1 and Bmp4 were downregulated, whereas the CVG genes, Fgf3 and NeuroD, were upregulated. To delineate Tbx1 cell-autonomous roles, a more selective ablation, exclusively in the OV, was performed using Pax2-Cre. In contrast to the Foxg1-Cre mutants, Pax2-Cre conditional mutant mice survived to adulthood and had normal outer and middle ears but had the same inner ear defects as the Tbx1 null mice, with the same gene expression changes. These results demonstrate that Tbx1 has non-cell autonomous roles in PPI in the formation of outer and middle ears and cell-autonomous roles in the OV. Periotic mesenchymal markers, Prx2 and Brn4 were normal in both conditional mutants, whereas they were diminished in Tbx1−/− embryos. Thus, Tbx1 in the surrounding mesenchyme in both sets of conditional mutants cannot suppress the defects in the OV that occur in the null mutants. PMID:16600992

Segregating neural and mechanosensory fates in the developing ear: patterning, signaling, and transcriptional control

PubMed Central

Raft, Steven; Groves, Andrew K.

2014-01-01

The vertebrate inner ear is composed of multiple sensory receptor epithelia, each of which is specialized for detection of sound, gravity or angular acceleration. Each receptor epithelium contains mechanosensitive hair cells, which are connected to the brainstem by bipolar sensory neurons. Hair cells and their associated neurons are derived from the embryonic rudiment of the inner ear epithelium, but the precise spatial and temporal patterns of their generation, as well as the signals that coordinate these events, have only recently begun to be understood. Gene expression, lineage tracing, and mutant analyses suggest that both neurons and hair cells are generated from a common domain of neural and sensory competence in the embryonic inner ear rudiment. Members of the Shh, Wnt and FGF families, together with retinoic acid signals, regulate transcription factor genes within the inner ear rudiment to establish the axial identity of the ear and regionalize neurogenic activity. Close-range signaling, such as that of the Notch pathway, specifies the fate of sensory regions and individual cell types. We also describe positive and negative interactions between basic helix-loop-helix and SoxB family transcription factors that specify either neuronal or sensory fates in a context-dependent manner. Finally, we review recent work on inner ear development in zebrafish, which demonstrates that the relative timing of neurogenesis and sensory epithelial formation is not phylogenetically constrained. PMID:24902666

Perforated Eardrum

MedlinePlus

... the loss of hearing can be great and tinnitus (ringing in the ear) may occur. Chronic infection ... could cause ear infection), improved hearing, and lessened tinnitus. It also may prevent the development of cholesteatoma ( ...

New vascular classification of port-wine stains: improving prediction of Sturge-Weber risk.

PubMed

Waelchli, R; Aylett, S E; Robinson, K; Chong, W K; Martinez, A E; Kinsler, V A

2014-10-01

Facial port-wine stains (PWSs) are usually isolated findings; however, when associated with cerebral and ocular vascular malformations they form part of the classical triad of Sturge-Weber syndrome (SWS). To evaluate the associations between the phenotype of facial PWS and the diagnosis of SWS in a cohort with a high rate of SWS. Records were reviewed of all 192 children with a facial PWS seen in 2011-13. Adverse outcome measures were clinical (seizures, abnormal neurodevelopment, glaucoma) and radiological [abnormal magnetic resonance imaging (MRI)], modelled by multivariate logistic regression. The best predictor of adverse outcomes was a PWS involving any part of the forehead, delineated at its inferior border by a line joining the outer canthus of the eye to the top of the ear, and including the upper eyelid. This involves all three divisions of the trigeminal nerve, but corresponds well to the embryonic vascular development of the face. Bilateral distribution was not an independently significant phenotypic feature. Abnormal MRI was a better predictor of all clinical adverse outcome measures than PWS distribution; however, for practical reasons guidelines based on clinical phenotype are proposed. Facial PWS distribution appears to follow the embryonic vasculature of the face, rather than the trigeminal nerve. We propose that children with a PWS on any part of the 'forehead' should have an urgent ophthalmology review and a brain MRI. A prospective study has been established to test the validity of these guidelines. © The Authors. British Journal of Dermatology published by John Wiley & Sons Ltd on behalf of British Association of Dermatologists.

Congenital hearing loss

PubMed Central

Korver, Anna M. H.; Smith, Richard J. H.; Van Camp, Guy; Schleiss, Mark R.; Bitner-Glindzicz, Maria A. K.; Lustig, Lawrence R.; Usami, Shin-ichi; Boudewyns, An N.

2017-01-01

Congenital hearing loss (hearing loss present at birth) is one of the most prevalent chronic conditions in children. In the majority of developed countries, neonatal hearing-screening programmes enable early detection; early intervention will prevent delays in speech and language development and have long-lasting beneficial effects on social and emotional development and quality of life. A hearing loss diagnosis is usually followed by a search for an underlying aetiology. Congenital hearing loss might be attributed to environmental and prenatal factors, which prevail in low-income settings; congenital infections, particularly cytomegalovirus, are also a common risk factor for hearing loss. Genetic causes probably account for the majority of cases in developed countries; mutations can affect any component of the hearing pathway, in particular inner ear homeostasis (endolymph production and maintenance) and mechano-electrical transduction (conversion of a mechanical stimulus into electrochemical activity). Once the underlying cause of hearing loss is established, it might direct therapeutic decision-making and guide prevention and (genetic) counseling. Management options include specific antimicrobial therapies, surgical treatment of cranio-facial abnormalities and hearing aids. An improved understanding of the pathophysiology and molecular mechanisms underlying hearing loss and increased awareness of recent advances in genetic testing will promote the development of new treatment and screening strategies. PMID:28079113

Epidermal growth factor receptor as a novel molecular target for aggressive papillary tumors in the middle ear and temporal bone

PubMed Central

Kawabata, Shigeru; Christine Hollander, M; Munasinghe, Jeeva P.; Brinster, Lauren R.; Mercado-Matos, José R.; Li, Jie; Regales, Lucia; Pao, William; Jänne, Pasi A.; Wong, Kwok-Kin; Butman, John A.; Lonser, Russell R.; Hansen, Marlan R.; Gurgel, Richard K.; Vortmeyer, Alexander O.; Dennis, Phillip A.

2015-01-01

Adenomatous tumors in the middle ear and temporal bone are rare but highly morbid because they are difficult to detect prior to the development of audiovestibular dysfunction. Complete resection is often disfiguring and difficult because of location and the late stage at diagnosis, so identification of molecular targets and effective therapies is needed. Here, we describe a new mouse model of aggressive papillary ear tumor that was serendipitously discovered during the generation of a mouse model for mutant EGFR-driven lung cancer. Although these mice did not develop lung tumors, 43% developed head tilt and circling behavior. Magnetic resonance imaging (MRI) scans showed bilateral ear tumors located in the tympanic cavity. These tumors expressed mutant EGFR as well as active downstream targets such as Akt, mTOR and ERK1/2. EGFR-directed therapies were highly effective in eradicating the tumors and correcting the vestibular defects, suggesting these tumors are addicted to EGFR. EGFR activation was also observed in human ear neoplasms, which provides clinical relevance for this mouse model and rationale to test EGFR-targeted therapies in these rare neoplasms. PMID:26027747

Epidermal growth factor receptor as a novel molecular target for aggressive papillary tumors in the middle ear and temporal bone.

PubMed

Kawabata, Shigeru; Hollander, M Christine; Munasinghe, Jeeva P; Brinster, Lauren R; Mercado-Matos, José R; Li, Jie; Regales, Lucia; Pao, William; Jänne, Pasi A; Wong, Kwok-Kin; Butman, John A; Lonser, Russell R; Hansen, Marlan R; Gurgel, Richard K; Vortmeyer, Alexander O; Dennis, Phillip A

2015-05-10

Adenomatous tumors in the middle ear and temporal bone are rare but highly morbid because they are difficult to detect prior to the development of audiovestibular dysfunction. Complete resection is often disfiguring and difficult because of location and the late stage at diagnosis, so identification of molecular targets and effective therapies is needed. Here, we describe a new mouse model of aggressive papillary ear tumor that was serendipitously discovered during the generation of a mouse model for mutant EGFR-driven lung cancer. Although these mice did not develop lung tumors, 43% developed head tilt and circling behavior. Magnetic resonance imaging (MRI) scans showed bilateral ear tumors located in the tympanic cavity. These tumors expressed mutant EGFR as well as active downstream targets such as Akt, mTOR and ERK1/2. EGFR-directed therapies were highly effective in eradicating the tumors and correcting the vestibular defects, suggesting these tumors are addicted to EGFR. EGFR activation was also observed in human ear neoplasms, which provides clinical relevance for this mouse model and rationale to test EGFR-targeted therapies in these rare neoplasms.

Influenza Virus Induces Bacterial and Nonbacterial Otitis Media

PubMed Central

Diavatopoulos, Dimitri A.; Thornton, Ruth; Pedersen, John; Strugnell, Richard A.; Wise, Andrew K.; Reading, Patrick C.; Wijburg, Odilia L.

2011-01-01

Otitis media (OM) is one of the most common childhood diseases. OM can arise when a viral infection enables bacteria to disseminate from the nasopharynx to the middle ear. Here, we provide the first infant murine model for disease. Mice coinfected with Streptococcus pneumoniae and influenza virus had high bacterial load in the middle ear, middle ear inflammation, and hearing loss. In contrast, mice colonized with S. pneumoniae alone had significantly less bacteria in the ear, minimal hearing loss, and no inflammation. Of interest, infection with influenza virus alone also caused some middle ear inflammation and hearing loss. Overall, this study provides a clinically relevant and easily accessible animal model to study the pathogenesis and prevention of OM. Moreover, we provide, to our knowledge, the first evidence that influenza virus alone causes middle ear inflammation in infant mice. This inflammation may then play an important role in the development of bacterial OM. PMID:21930608

Requirements for FGF3 and FGF10 during inner ear formation.

PubMed

Alvarez, Yolanda; Alonso, Maria Teresa; Vendrell, Victor; Zelarayan, Laura Cecilia; Chamero, Pablo; Theil, Thomas; Bösl, Michael R; Kato, Shigeaki; Maconochie, Mark; Riethmacher, Dieter; Schimmang, Thomas

2003-12-01

Members of the fibroblast growth factor (FGF) gene family control formation of the body plan and organogenesis in vertebrates. FGF3 is expressed in the developing hindbrain and has been shown to be involved in inner ear development of different vertebrate species, including zebrafish, Xenopus, chick and mouse. In the mouse, insertion of a neomycin resistance gene into the Fgf3 gene via homologous recombination results in severe developmental defects during differentiation of the otic vesicle. We have addressed the precise roles of FGF3 and other FGF family members during formation of the murine inner ear using both loss- and gain-of-function experiments. We generated a new mutant allele lacking the entire FGF3-coding region but surprisingly found no evidence for severe defects either during inner ear development or in the mature sensory organ, suggesting the functional involvement of other FGF family members during its formation. Ectopic expression of FGF10 in the developing hindbrain of transgenic mice leads to the formation of ectopic vesicles, expressing some otic marker genes and thus indicating a role for FGF10 during otic vesicle formation. Expression analysis of FGF10 during mouse embryogenesis reveals a highly dynamic pattern of expression in the developing hindbrain, partially overlapping with FGF3 expression and coinciding with formation of the inner ear. However, FGF10 mutant mice have been reported to display only mild defects during inner ear differentiation. We thus created double mutant mice for FGF3 and FGF10, which form severely reduced otic vesicles, suggesting redundant roles of these FGFs, acting in combination as neural signals for otic vesicle formation.

15 CFR 744.6 - Restrictions on certain activities of U.S. persons.

Code of Federal Regulations, 2011 CFR

2011-01-01

.... 1 to part 740 of the EAR). (B) Will be used in the design, development, production, or use of missiles in or by a country listed in Country Group D:4 (see supplement no. 1 to part 740 of the EAR); or... 740 of the EAR); or (ii) Perform any contract, service, or employment that the U.S. person knows will...

Multiple enhancers located in a 1-Mb region upstream of POU3F4 promote expression during inner ear development and may be required for hearing

PubMed Central

Naranjo, Silvia; Voesenek, Krysta; de la Calle-Mustienes, Elisa; Robert-Moreno, Alex; Kokotas, Haris; Grigoriadou, Maria; Economides, John; Van Camp, Guy; Hilgert, Nele; Moreno, Felipe; Alsina, Berta; Petersen, Michael B.; Kremer, Hannie

2010-01-01

POU3F4 encodes a POU-domain transcription factor required for inner ear development. Defects in POU3F4 function are associated with X-linked deafness type 3 (DFN3). Multiple deletions affecting up to ~900-kb upstream of POU3F4 are found in DFN3 patients, suggesting the presence of essential POU3F4 enhancers in this region. Recently, an inner ear enhancer was reported that is absent in most DFN3 patients with upstream deletions. However, two indications suggest that additional enhancers in the POU3F4 upstream region are required for POU3F4 function during inner ear development. First, there is at least one DFN3 deletion that does not eliminate the reported enhancer. Second, the expression pattern driven by this enhancer does not fully recapitulate Pou3f4 expression in the inner ear. Here, we screened a 1-Mb region upstream of the POU3F4 gene for additional cis-regulatory elements and searched for novel DFN3 mutations in the identified POU3F4 enhancers. We found several novel enhancers for otic vesicle expression. Some of these also drive expression in kidney, pancreas and brain, tissues that are known to express Pou3f4. In addition, we report a new and smallest deletion identified so far in a DFN3 family which eliminates 3.9 kb, comprising almost exclusively the previous reported inner ear enhancer. We suggest that multiple enhancers control the expression of Pou3f4 in the inner ear and these may contribute to the phenotype observed in DFN3 patients. In addition, the novel deletion demonstrates that the previous reported enhancer, although not sufficient, is essential for POU3F4 function during inner ear development. Electronic supplementary material The online version of this article (doi:10.1007/s00439-010-0864-x) contains supplementary material, which is available to authorized users. PMID:20668882

Finite-Element Modelling of the Acoustic Input Admittance of the Newborn Ear Canal and Middle Ear.

PubMed

Motallebzadeh, Hamid; Maftoon, Nima; Pitaro, Jacob; Funnell, W Robert J; Daniel, Sam J

2017-02-01

Admittance measurement is a promising tool for evaluating the status of the middle ear in newborns. However, the newborn ear is anatomically very different from the adult one, and the acoustic input admittance is different than in adults. To aid in understanding the differences, a finite-element model of the newborn ear canal and middle ear was developed and its behaviour was studied for frequencies up to 2000 Hz. Material properties were taken from previous measurements and estimates. The simulation results were within the range of clinical admittance measurements made in newborns. Sensitivity analyses of the material properties show that in the canal model, the maximum admittance and the frequency at which that maximum admittance occurs are affected mainly by the stiffness parameter; in the middle-ear model, the damping is as important as the stiffness in influencing the maximum admittance magnitude but its effect on the corresponding frequency is negligible. Scaling up the geometries increases the admittance magnitude and shifts the resonances to lower frequencies. The results suggest that admittance measurements can provide more information about the condition of the middle ear when made at multiple frequencies around its resonance.

Proteomic Analysis Revealed Nitrogen-mediated Metabolic, Developmental, and Hormonal Regulation of Maize (Zea mays L.) Ear Growth

PubMed Central

Li, Chunjian; Li, Xuexian

2012-01-01

Optimal nitrogen (N) supply is critical for achieving high grain yield of maize. It is well established that N deficiency significantly reduces grain yield and N oversupply reduces N use efficiency without significant yield increase. However, the underlying proteomic mechanism remains poorly understood. The present field study showed that N deficiency significantly reduced ear size and dry matter accumulation in the cob and grain, directly resulting in a significant decrease in grain yield. The N content, biomass accumulation, and proteomic variations were further analysed in young ears at the silking stage under different N regimes. N deficiency significantly reduced N content and biomass accumulation in young ears of maize plants. Proteomic analysis identified 47 proteins with significant differential accumulation in young ears under different N treatments. Eighteen proteins also responded to other abiotic and biotic stresses, suggesting that N nutritional imbalance triggered a general stress response. Importantly, 24 proteins are involved in regulation of hormonal metabolism and functions, ear development, and C/N metabolism in young ears, indicating profound impacts of N nutrition on ear growth and grain yield at the proteomic level. PMID:22936831

An ear punch model for studying the effect of radiation on wound healing.

PubMed

Deoliveira, Divino; Jiao, Yiqun; Ross, Joel R; Corbin, Kayla; Xiao, Qizhen; Toncheva, Greta; Anderson-Evans, Colin; Yoshizumi, Terry T; Chen, Benny J; Chao, Nelson J

2011-08-01

Radiation and wound combined injury represents a major clinical challenge because of the synergistic interactions that lead to higher morbidity and mortality than either insult would produce singly. The purpose of this study was to develop a mouse ear punch model to study the physiological mechanisms underlying radiation effects on healing wounds. Surgical wounds were induced by a 2 mm surgical punch in the ear pinnae of MRL/MpJ mice. Photographs of the wounds were taken and the sizes of the ear punch wounds were quantified by image analysis. Local radiation to the ear was delivered by orthovoltage X-ray irradiator using a specially constructed jig that shields the other parts of body. Using this model, we demonstrated that local radiation to the wound area significantly delayed the healing of ear punch wounds in a dose-dependent fashion. The addition of sublethal whole body irradiation (7 Gy) further delayed the healing of ear punch wounds. These results were replicated in C57BL/6 mice; however, wound healing in MRL/MpJ mice was accelerated. These data indicate that the mouse ear punch model is a valuable model to study radiation and wound combined injury.

Comparison of Middle Ear Visualization With Endoscopy and Microscopy.

PubMed

Bennett, Marc L; Zhang, Dongqing; Labadie, Robert F; Noble, Jack H

2016-04-01

The primary goal of chronic ear surgery is the creation of a safe, clean dry ear. For cholesteatomas, complete removal of disease is dependent on visualization. Conventional microscopy is adequate for most dissection, but various subregions of the middle ear are better visualized with endoscopy. The purpose of the present study was to quantitatively assess the improved visualization that endoscopes afford as compared with operating microscopes. Microscopic and endoscopic views were simulated using a three-dimensional model developed from temporal bone scans. Surface renderings of the ear canal and middle ear subsegments were defined and the percentage of visualization of each middle ear subsegment, both with and without ossicles, was then determined for the microscope as well as for 0-, 30-, and 45-degree endoscopes. Using this information, we analyzed which mode of visualization is best suited for dissection within a particular anatomical region. Using a 0-degree scope provides significantly more visualization of every subregion, except the antrum, compared with a microscope. In addition, angled scopes permit visualizing significantly more surface area of every subregion of the middle ear than straight scopes or microscopes. Endoscopes offer advantages for cholesteatoma dissection in difficult-to-visualize areas including the sinus tympani and epitympanum.

Radiological differences between HIV-positive and HIV-negative children with cholesteatoma.

PubMed

McGuire, J K; Fagan, J J; Wojno, M; Manning, K; Harris, T

2018-07-01

HIV-positive children are possibly more prone to developing cholesteatoma. Chronic inflammation of the middle ear cleft may be more common in patients with HIV and this may predispose HIV-positive children to developing cholesteatoma. There are no studies that describe the radiological morphology of the middle ear cleft in HIV-positive compared to HIV-negative children with cholesteatoma. Compare the radiological differences of the middle ear cleft in HIV-positive and HIV-negative children with cholesteatoma. A retrospective, cross-sectional, observational analytical review of patients with cholesteatoma at our institute over a 6 year period. Forty patients were included in the study, 11 of whom had bilateral cholesteatoma and therefore 51 ears were eligible for our evaluation. HIV-positive patients had smaller (p=0.02) mastoid air cell systems (MACS). Forty percent of HIV-positive patients had sclerotic mastoids, whereas the rate was 3% in HIV-negative ears (p<0.02). Eighty-two percent of the HIV-positive patients had bilateral cholesteatoma compared to 7% of the control group (p<0.02). There was no difference between the 2 groups with regards to opacification of the middle ear cleft, bony erosion of middle ear structures, Eustachian tube obstruction or soft tissue occlusion of the post-nasal space. HIV-positive paediatric patients with cholesteatoma are more likely to have smaller, sclerotic mastoids compared to HIV-negative patients. They are significantly more likely to have bilateral cholesteatoma. This may have implications in terms of surveillance of HIV-positive children, as well as, an approach to management, recurrence and follow-up. HIV infection should be flagged as a risk factor for developing cholesteatoma. Copyright © 2018. Published by Elsevier B.V.

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

PubMed

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

2013-01-01

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

Pro-inflammatory interleukins in middle ear effusions from atopic and non-atopic children with chronic otitis media with effusion.

PubMed

Zielnik-Jurkiewicz, Beata; Stankiewicz-Szymczak, Wanda

2016-06-01

Chronic otitis media with effusion (OME) is associated with irreversible changes in the middle ear, sometimes leading to hearing loss and abnormal language development in children. While the pathogenesis of OME is not fully understood, inflammatory and allergic factors are thought to be involved. The study aimed to investigate the role of cytokines in the local development of chronic OME, and assess differences in the cytokine profiles between atopic and non-atopic children. 84 atopic and non-atopic children with chronic OME (mean age of 6 years 7 months) were studied. Age-matched children with hypertrophy of the adenoids and Eustachian tube dysfunction served as the control group. The number of past acute otitis media (AOM) episodes, their age, and the type of effusion were recorded for all children. Pro-inflammatory cytokine concentrations (TNF-α, IL-1β, IL-6 and IL-8) were determined and the presence of pathogenic bacteria in the patients' effusions was examined. High concentrations of TNF-α, IL-1β, IL-6 and IL-8 were found in the effusions in all children with chronic OME, with the highest levels observed in the non-atopic group. The atopic group showed persistently high IL-1β levels, while in the non-atopic children, IL-1β and TNF-α levels positively correlated with the patient's age and the number of past AOM episodes. Pathogenic bacteria were more frequently isolated from effusions in non-atopic children. In both atopic and non-atopic children, pro-inflammatory cytokines are found at high concentrations. This argues in favor of instituting anti-inflammatory management for treating OME, regardless of atopy.

Auditory Alterations in Children Infected by Human Immunodeficiency Virus Verified Through Auditory Processing Test

PubMed Central

Romero, Ana Carla Leite; Alfaya, Lívia Marangoni; Gonçales, Alina Sanches; Frizzo, Ana Claudia Figueiredo; Isaac, Myriam de Lima

2016-01-01

Introduction The auditory system of HIV-positive children may have deficits at various levels, such as the high incidence of problems in the middle ear that can cause hearing loss. Objective The objective of this study is to characterize the development of children infected by the Human Immunodeficiency Virus (HIV) in the Simplified Auditory Processing Test (SAPT) and the Staggered Spondaic Word Test. Methods We performed behavioral tests composed of the Simplified Auditory Processing Test and the Portuguese version of the Staggered Spondaic Word Test (SSW). The participants were 15 children infected by HIV, all using antiretroviral medication. Results The children had abnormal auditory processing verified by Simplified Auditory Processing Test and the Portuguese version of SSW. In the Simplified Auditory Processing Test, 60% of the children presented hearing impairment. In the SAPT, the memory test for verbal sounds showed more errors (53.33%); whereas in SSW, 86.67% of the children showed deficiencies indicating deficit in figure-ground, attention, and memory auditory skills. Furthermore, there are more errors in conditions of background noise in both age groups, where most errors were in the left ear in the Group of 8-year-olds, with similar results for the group aged 9 years. Conclusion The high incidence of hearing loss in children with HIV and comorbidity with several biological and environmental factors indicate the need for: 1) familiar and professional awareness of the impact on auditory alteration on the developing and learning of the children with HIV, and 2) access to educational plans and follow-up with multidisciplinary teams as early as possible to minimize the damage caused by auditory deficits. PMID:28050213

Preventing Cauliflower Ear With a Modified Tie-Through Technique.

PubMed

Dimeff, R J; Hough, D O

1989-03-01

In brief: Hematoma following trauma to the external ear is a common problem among wrestlers and boxers. If the hematoma is not treated promptly, infection, fibrosis, scarring, and calcification may develop, leading to the gross deformity known as cauliflower ear or wrestler's ear. Evacuation of the hematoma followed by compression of the auricle is commonly regarded as the treatment of choice. However, fluid frequently reaccumulates after this procedure. The authors describe a tie-through suture technique in which a collodion packing is secured to the auricle with two buttons. Multiple treatments for fluid reaccumulation are rarely necessary when this technique is used.

The role of Foxi family transcription factors in otic placode and neural crest cell development

PubMed Central

Edlund, Renée K.; Birol, Onur; Groves, Andrew K.

2015-01-01

The mammalian outer, middle and inner ears have different embryonic origins and evolved at different times in the vertebrate lineage. The outer ear is derived from first and second branchial arch ectoderm and mesoderm, the middle ear ossicles are derived from neural crest mesenchymal cells that invade the first and second branchial arches, whereas the inner ear and its associated vestibule-acoustic (VIIIth) ganglion are derived from the otic placode. In this review, we discuss recent findings in the development of these structures and describe the contributions of members of a Forkhead transcription factor family, the Foxi family to their formation. Foxi transcription factors are critical for formation of the otic placode, survival of the branchial arch neural crest, and developmental remodeling of the branchial arch ectoderm. PMID:25662269

Alternative splicing of inner-ear-expressed genes.

PubMed

Wang, Yanfei; Liu, Yueyue; Nie, Hongyun; Ma, Xin; Xu, Zhigang

2016-09-01

Alternative splicing plays a fundamental role in the development and physiological function of the inner ear. Inner-ear-specific gene splicing is necessary to establish the identity and maintain the function of the inner ear. For example, exon 68 of Cadherin 23 (Cdh23) gene is subject to inner-ear-specific alternative splicing, and as a result, Cdh23(+ 68) is only expressed in inner ear hair cells. Alternative splicing along the tonotopic axis of the cochlea contributes to frequency tuning, particularly in lower vertebrates, such as chickens and turtles. Differential splicing of Kcnma1, which encodes for the α subunit of the Ca(2+)-activated K(+) channel (BK channel), has been suggested to affect the channel gating properties and is important for frequency tuning. Consequently, deficits in alternative splicing have been shown to cause hearing loss, as we can observe in Bronx Waltzer (bv) mice and Sfswap mutant mice. Despite the advances in this field, the regulation of alternative splicing in the inner ear remains elusive. Further investigation is also needed to clarify the mechanism of hearing loss caused by alternative splicing deficits.

Finite element modeling of sound transmission with perforations of tympanic membrane

PubMed Central

Gan, Rong Z.; Cheng, Tao; Dai, Chenkai; Yang, Fan; Wood, Mark W.

2009-01-01

A three-dimensional finite element (FE) model of human ear with structures of the external ear canal, middle ear, and cochlea has been developed recently. In this paper, the FE model was used to predict the effect of tympanic membrane (TM) perforations on sound transmission through the middle ear. Two perforations were made in the posterior-inferior quadrant and inferior site of the TM in the model with areas of 1.33 and 0.82 mm2, respectively. These perforations were also created in human temporal bones with the same size and location. The vibrations of the TM (umbo) and stapes footplate were calculated from the model and measured from the temporal bones using laser Doppler vibrometers. The sound pressure in the middle ear cavity was derived from the model and measured from the bones. The results demonstrate that the TM perforations can be simulated in the FE model with geometrical visualization. The FE model provides reasonable predictions on effects of perforation size and location on middle ear transfer function. The middle ear structure-function relationship can be revealed with multi-field coupled FE analysis. PMID:19603881

KGFR as a possible therapeutic target in middle ear cholesteatoma.

PubMed

Yamamoto-Fukuda, Tomomi; Akiyama, Naotaro; Shibata, Yasuaki; Takahashi, Haruo; Ikeda, Tohru; Kohno, Michiaki; Koji, Takehiko

2014-11-01

We demonstrated that repression of keratinocyte growth factor (KGF) receptor (KGFR) could be a potentially useful strategy in the conservative treatment of middle ear cholesteatoma. Recently, the use of a selective inhibitor of the KGFR, SU5402, in an in vitro experiment resulted in the inhibition of the differentiation and proliferation of epithelial cells through KGF secretion by fibroblasts isolated from the cholesteatoma. In this study, we investigated the effects of the KGFR inhibitor during middle ear cholesteatoma formation in vivo. Based on the role of KGF in the development of cholesteatoma, Flag-hKGF cDNA driven by CMV14 promoter was transfected through electroporation into the external auditory canal of rats five times on every fourth day. Ears transfected with empty vector were used as controls. KGFR selective inhibitor (SU5402) or MEK inhibitor (PD0325901) was administered in the right ear of five rats after vector transfection. In the control, 2% DMSO in PBS was administered in the other ears after vector transfection. The use of a selective KGFR inhibitor, SU5402, completely prevented middle ear cholesteatoma formation in the rats.

Long-term consequences of Sox9 depletion on inner ear development

PubMed Central

Park, Byung-Yong; Saint-Jeannet, Jean-Pierre

2010-01-01

The transcription factor Sox9 has been implicated in inner ear formation in several species. To investigate the long-term consequences of Sox9 depletion on inner ear development we analyzed the inner ear architecture of Sox9-depleted Xenopus tadpoles generated by injection of increasing amounts of Sox9 morpholino antisense oligonucleotides. We found that Sox9-depletion resulted in major defects in the development of vestibular structures, semicircular canals and utricle, while the ventrally located saccule was less severely affected in these embryos. Consistent with this phenotype we observed a specific loss of the dorsal expression of Wnt3a expression in the otic vesicle of Sox9 morphants, associated with an increase in cell death and a reduction in cell proliferation in the region of the presumptive otic epithelium. We propose that in addition to its early role in placode specification, Sox9 is also required for the maintenance of progenitors in the otic epithelium. PMID:20201105

Optical coherence tomography for the diagnosis of human otitis media

NASA Astrophysics Data System (ADS)

Cho, Nam Hyun; Jung, Unsang; Jang, Jeong Hun; Jung, Woonggyu; Kim, Jeehyun; Lee, Sang Heun; Boppart, Stephen A.

2013-05-01

We report the application of Optical Coherence Tomography (OCT) to various types of human cases of otitis media (OM). Whereas conventional diagnostic modalities for OM, including standard and pneumatic otoscopy, are limited to visualizing the surface information of the tympanic membrane (TM), OCT is able to effectively reveal the depth-resolved microstructural below the TM with a very high spatial resolution. With the potential advantage of using OCT for diagnosing different types of OM, we examined in-vivo the use of 840 nm wavelength, and OCT spectral domain OCT (SDOCT) techniques, in several human cases including normal ears, and ears with adhesive and effusion types of OM. Peculiar positions were identified in two-dimensional OCT images of abnormal TMs compared to images of a normal TM. Analysis of A-scan (axial depth-scans) data from these positions could successfully identify unique patterns for different constituents within effusions. These OCT images may not only be used for constructing a database for the diagnosis and classification of OM, but they may also demonstrate the feasibility and advantages for upgrading the current otoscopy techniques.

Caloric Analysis of Patients with Benign Paroxysmal Positional Vertigo.

PubMed

Yetişer, Sertaç; İnce, Dilay

2017-12-01

The aim of this study is to compare nystagmus characteristics after caloric irrigation in patients with lateral canal (LC) and posterior canal (PC) benign paroxysmal positional vertigo (BPPV) and to analyze the role of symptom duration. A prospective study was conducted in 65 patients with BPPV (20 LC and 45 PC) who were subjected to caloric testing. Average slow-phase velocity and nystagmus duration were analyzed. Caloric hypo-excitability was 20.4%. It was more evident in patients with apogeotropic-type LC-BPPV. The comparison of average slow-phase velocity of the nystagmus and nystagmus duration between selected types of BPPV for pathologic, non-pathologic, and the control ears after warm and cold stimulation was not statistically significant (p>0.05). No correlation was found between caloric results and symptom duration (p>0.05). Some patients presented caloric hypo-excitability. Reliability of caloric testing to differentiate the ear with normal and abnormal vestibular function in different types of BPPV was low. No difference was found in the analysis of the impact of symptom duration. Caloric testing is not an ideal tool to study BPPV.

The preauricular sinus: a review of its clinical presentation, treatment, and associations.

PubMed

Scheinfeld, Noah S; Silverberg, Nanette B; Weinberg, Jeffrey M; Nozad, Valerie

2004-01-01

Preauricular sinuses (ear pits) are common congenital abnormalities. Usually asymptomatic, they manifest as small dells adjacent to the external ear near the anterior margin of the ascending limb of the helix, most frequently on the right side. Preauricular sinuses can be either inherited or sporadic. When inherited, they show an incomplete autosomal dominant pattern with reduced penetrance and variable expression. They may be bilateral, increasing the likelihood of being inherited, in 25-50% of cases. Preauricular sinuses are features of other conditions or syndromes in 3-10% of cases, primarily in association with deafness and branchio-oto-renal (BOR) syndrome. When other congenital anomalies coexist with these sinuses, auditory testing and renal ultrasound should be considered. Sinuses may become infected, most commonly with gram-positive bacteria, in which case their exudates should be cultured and appropriate antibiotics administered. Recurrent infection is a clear indication for complete excision and provides the only definitive cure. Recurrence rates after surgery range from 9% to 42%. Meticulous excision by an experienced head and neck surgeon minimizes the risk of recurrence.

Efficacy and toxicity of Samen-ista emulsion on treatment of cutaneous and mucosal bleeding.

PubMed

Hosseini, Mousalreza; Pourakbar, Ali; Forouzanfar, Fatemeh; Arian, Amirali; Ghaffarzadegan, Kamran; Salehi, Maryam; Esfandiari, Samaneh; Rakhshandeh, Hassan

2016-10-01

Despite new treatment methods, upper gastrointestinal bleeding remains challenging. Samen-ista emulsion is a new agent based on traditional medicine with coagulant properties. The efficacy and safety of Samen-ista were assessed in cutaneous and mucosal bleeding animal models. Coagulant properties of Samen-ista were evaluated using mice tail bleeding assay, marginal ear vein and upper gastrointestinal mucosal bleeding times in rabbits. After 7 days, clinical signs, mortality and end-organ (kidney, liver, lung, brain and gastric mucosa) histopathological changes were also examined. Samen-ista dose-dependently decreased mean cutaneous tail (128 vs. 14 s) and marginal ear vein (396 vs. 84 s) bleeding times. Rabbit's upper gastrointestinal bleeding time was also significantly decreased (214 vs. 15.8 s) upon Samen-ista local endoscopic application. Treatment with Samen-ista for 7 days did not cause any mortality, abnormal signs of bleeding, changes in appetite or significant histopathologicl changes. Samen-ista emulsion is well tolerated and highly effective in achieving hemostasis in cutaneous and mucosal bleeding animal models.

Estimation of sex from the anthropometric ear measurements of a Sudanese population.

PubMed

Ahmed, Altayeb Abdalla; Omer, Nosyba

2015-09-01

The external ear and its prints have multifaceted roles in medico-legal practice, e.g., identification and facial reconstruction. Furthermore, its norms are essential in the diagnosis of congenital anomalies and the design of hearing aids. Body part dimensions vary in different ethnic groups, so the most accurate statistical estimations of biological attributes are developed using population-specific standards. Sudan lacks comprehensive data about ear norms; moreover, there is a universal rarity in assessing the possibility of sex estimation from ear dimensions using robust statistical techniques. Therefore, this study attempts to establish data for normal adult Sudanese Arabs, assessing the existence of asymmetry and developing a population-specific equation for sex estimation. The study sample comprised 200 healthy Sudanese Arab volunteers (100 males and 100 females) in the age range of 18-30years. The physiognomic ear length and width, lobule length and width, and conchal length and width measurements were obtained by direct anthropometry, using a digital sliding caliper. Moreover, indices and asymmetry were assessed. Data were analyzed using basic descriptive statistics and discriminant function analyses employing jackknife validations of classification results. All linear dimensions used were sexually dimorphic except lobular lengths. Some of the variables and indices show asymmetry. Ear dimensions showed cross-validated sex classification accuracy ranging between 60.5% and 72%. Hence, the ear measurements cannot be used as an effective tool in the estimation of sex. However, in the absence of other more reliable means, it still can be considered a supportive trait in sex estimation. Further, asymmetry should be considered in identification from the ear measurements. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

Sheep as a large animal ear model: Middle-ear ossicular velocities and intracochlear sound pressure.

PubMed

Péus, Dominik; Dobrev, Ivo; Prochazka, Lukas; Thoele, Konrad; Dalbert, Adrian; Boss, Andreas; Newcomb, Nicolas; Probst, Rudolf; Röösli, Christof; Sim, Jae Hoon; Huber, Alexander; Pfiffner, Flurin

2017-08-01

Animals are frequently used for the development and testing of new hearing devices. Dimensions of the middle ear and cochlea differ significantly between humans and commonly used animals, such as rodents or cats. The sheep cochlea is anatomically more like the human cochlea in size and number of turns. This study investigated the middle-ear ossicular velocities and intracochlear sound pressure (ICSP) in sheep temporal bones, with the aim of characterizing the sheep as an experimental model for implantable hearing devices. Measurements were made on fresh sheep temporal bones. Velocity responses of the middle ear ossicles at the umbo, long process of the incus and stapes footplate were measured in the frequency range of 0.25-8 kHz using a laser Doppler vibrometer system. Results were normalized by the corresponding sound pressure level in the external ear canal (P EC ). Sequentially, ICSPs at the scala vestibuli and tympani were then recorded with custom MEMS-based hydrophones, while presenting identical acoustic stimuli. The sheep middle ear transmitted most effectively around 4.8 kHz, with a maximum stapes velocity of 0.2 mm/s/Pa. At the same frequency, the ICSP measurements in the scala vestibuli and tympani showed the maximum gain relative to the P EC (24 dB and 5 dB, respectively). The greatest pressure difference across the cochlear partition occurred between 4 and 6 kHz. A comparison between the results of this study and human reference data showed middle-ear resonance and best cochlear sensitivity at higher frequencies in sheep. In summary, sheep can be an appropriate large animal model for research and development of implantable hearing devices. Copyright © 2017 Elsevier B.V. All rights reserved.

Development and Validation of the Musical Ear Training Assessment (META)

ERIC Educational Resources Information Center

Wolf, Anna; Kopiez, Reinhard

2018-01-01

In the following study, we have developed an assessment instrument for the practice-dependent skill of analytical hearing following a strict test theoretical validation, resulting in the Musical Ear Training Assessment (META). By means of three pilot studies, a developmental study, and a validation study, we verified a one-dimensional test model…

Gata2 is required for the development of inner ear semicircular ducts and the surrounding perilymphatic space.

PubMed

Haugas, Maarja; Lilleväli, Kersti; Hakanen, Janne; Salminen, Marjo

2010-09-01

Gata2 has essential roles in the development of many organs. During mouse inner ear morphogenesis, it is expressed in otic vesicle and the surrounding periotic mesenchyme from early on, but no defects in the ear development of Gata2 null mice have been observed before lethality at embryonic day (E) 10.5. Here, we used conditional gene targeting to reveal the role of Gata2 at later stages of inner ear development. We show that Gata2 is critically required from E14.5-E15.5 onward for vestibular morphogenesis. Without Gata2 the semicircular ducts fail to grow to their normal size and the surrounding mesenchymal cells are not removed properly to generate the perilymphatic space. Gata2 is the first factor known to control the clearing of the vestibular perilymphatic mesenchyme, but interestingly, it is not required for the formation of the cochlear perilymphatic areas, suggesting distinct molecular control for these processes. Developmental Dynamics 239:2452-2469, 2010. © 2010 Wiley-Liss, Inc.

A distinct type of hidrotic ectodermal dysplasia.

PubMed

Halal, F; Setton, N; Wang, N S

1991-03-15

Four individuals from 2 generations of a family had a hidrotic type of ectodermal dysplasia (ED). Males and females were similarly affected. They had trichodysplasia, with absent eyebrows and eyelashes; normal teeth, onychodysplasia; normal sweating; mild retrognathia; abnormal dermatoglyphics; and mental retardation. Additional variable manifestations included irregular menses, high implanted or prominent ears, café-au-lait spot, keratosis pilaris, supernumerary nipple, and mild hearing loss. Their previously undescribed condition could be classified as an ED of 1-3 (trichoonychial) subgroup of group A according to Freire-Maia's classification and is inherited as an autosomal recessive trait.

Defects in middle ear cavitation cause conductive hearing loss in the Tcof1 mutant mouse.

PubMed

Richter, Carol A; Amin, Susan; Linden, Jennifer; Dixon, Jill; Dixon, Michael J; Tucker, Abigail S

2010-04-15

Conductive hearing loss (CHL) is one of the most common forms of human deafness. Despite this observation, a surprising gap in our understanding of the mechanisms underlying CHL remains, particularly with respect to the molecular mechanisms underlying middle ear development and disease. Treacher Collins syndrome (TCS) is an autosomal dominant disorder of facial development that results from mutations in the gene TCOF1. CHL is a common feature of TCS but the causes of the hearing defect have not been studied. In this study, we have utilized Tcof1 mutant mice to dissect the developmental mechanisms underlying CHL. Our results demonstrate that effective cavitation of the middle ear is intimately linked to growth of the auditory bulla, the neural crest cell-derived structure that encapsulates all middle ear components, and that defects in these processes have a profoundly detrimental effect on hearing. This research provides important insights into a poorly characterized cause of human deafness, and provides the first mouse model for the study of middle ear cavity defects, while also being of direct relevance to a human genetic disorder.

Missing links in some curious auditory phenomena: a tale from the middle ear.

PubMed

Carpenter, Michelle S; Cacace, Anthony T; Mahoney, Marty J

2012-02-01

Broadband middle ear power reflectance (BMEPR) is an emerging noninvasive electroacoustic measure that evaluates transmission/reflection properties of the middle ear in high resolution. It is applicable over the entire age continuum and is rapid to perform. However, it remains to be determined if BMEPR is just an incremental step in the evolution of middle ear assessment or a major advance in the way middle ear function can be evaluated. To evaluate effects of age, gender, ear, and frequency on BMEPR measurements in adults without a history of middle ear disease and to assess whether these factors require consideration in test development; to review how these data may influence active physiologic process within the inner ear; to consider how they reconcile with previously published results; and to suggest applications for future research. Prospective, cross-sectional, multivariate analysis to evaluate the effects of age, gender, ear, and frequency on BMEPR in humans without a history of middle ear disease and no air-bone gaps exceeding 10 dB for any frequency. Fifty-six adults in two age groups (Group 1: 18-25 yr, n = 28; Group 2: ≥50 and <66 yr, n = 28). Each age group was stratified by ear and gender in a balanced design. Pure tone air conduction and bone-conduction audiometry was conducted in a commercial sound booth, using a clinical audiometer with standard earphones enclosed in supra-aural ear cushions, and a standard bone-conduction oscillator and headband to evaluate for air-bone gaps. Broadband middle ear power reflectance was measured using a calibrated, commercially available computer-controlled system that incorporated a high quality probe assembly to transduce stimuli and record acoustic responses from the ear canal. Data were analyzed with a four-way (2 × 2 × 2 × 16) repeated measures analysis-of-variance (ANOVA) to evaluate the effects of age group (young vs. old), gender (male vs. female), ear (left vs. right), and frequency (258 to 5040 Hz) on BMEPR. The ANOVA revealed a significant main effect of frequency. There were also gender × ear, gender × frequency, and age × gender × ear interactions. The three-way, age × gender × ear interaction captured the essence of results and revealed lowest power reflectance values in younger females and for right ears. This trend partially reversed in the older age group where higher power reflectance values were observed only in right ears of older females. The significant effects of age, gender, ear, and frequency on BMEPR parallel ear- and gender-related differences in hearing sensitivity, ear, and gender differences in the prevalence of spontaneous otoacoustic emissions (OAEs), gender differences in the magnitude of transient evoked OAEs, and ear differences in transient evoked contralateral OAE suppression effects reported in the literature. While original discussions of these aforementioned effects focused primarily on endocochlear and olivocochlear mechanisms, the BMEPR measurements reported herein suggest that middle ear transmission characteristics may also play a role. American Academy of Audiology.

The Modified Rambo Transcanal Approach for Cochlear Implantation in CHARGE Syndrome.

PubMed

Wick, Cameron C; Moore, Amy M; Killeen, Daniel E; Isaacson, Brandon

2017-10-01

CHARGE syndrome is associated with a variety of temporal bone anomalies and deafness. The lack of surgical landmarks and facial nerve irregularities make cochlear implantation in this population a challenging endeavor. This study aims to describe a safe and efficacious transcanal approach for cochlear implantation that obviates the need to perform a mastoidectomy and facial recess. Three children with profound hearing loss secondary to CHARGE syndrome. Transcanal cochlear implantation with closure of the ear canal via a modified Rambo meatoplasty. Retrospective chart review of temporal bone anomalies associated with CHARGE syndrome, technical nuances of this transcanal approach, and cochlear implant outcomes. The mean patient age was 2.5 years (range 1.5-3.8 yr). Two were male and two were left ears. All patients had a hypoplastic mastoid, semicircular canal aplasia, and had some degree of cochlear dysplasia. A full cochlear implant insertion was achieved in all cases, even in the presence of grossly abnormal middle ear and facial nerve anatomy. There were no intraoperative or postoperative complications. The mean follow-up was 12.4 months (range, 3.9-25.2 mo). All three patients use their device daily. Their guardians report improved vocalization and environmental awareness. The modified Rambo transcanal approach provides a safe corridor for cochlear implantation in patients with CHARGE syndrome. This approach minimizes the anatomical variations associated with the syndrome and may reduce the risk of electrode extrusion. Implant outcomes in this patient population remain highly variable based on the patient's global cognitive capacity.

Genetics Home Reference: Ménière disease

MedlinePlus

... dizziness (vertigo), a roaring sound in the ears (tinnitus), a feeling of pressure or fullness in the ... many affected individuals develop ongoing problems with unsteadiness, tinnitus, and a feeling of fullness in the ears. ...

Wideband acoustic immittance for assessing middle ear functioning for preterm neonates in the neonatal intensive care unit.

PubMed

Gouws, Nandel; Swanepoel, De Wet; De Jager, Leigh Biagio

2017-06-28

The primary aim of newborn hearing screening is to detect permanent hearing loss. Because otoacoustic emissions (OAEs) and automated auditory brainstem response (AABR) are sensitive to hearing loss, they are often used as screening tools. On the other hand, false-positive results are most often because of transient outer- and middle ear conditions. Wideband acoustic immittance (WAI), which includes physical measures known as reflectance and absorbance, has shown potential for accurate assessment of middle ear function in young infants. The main objective of this study was to determine the feasibility of WAI as a diagnostic tool for assessing middle ear functioning in preterm neonates in the neonatal intensive care unit (NICU) designed for premature and ill neonates. A further objective was to indicate the difference between the reflectance values of tones and click stimuli. Fifty-six at-risk neonates (30 male and 26 female), with a mean age at testing of 35.6 weeks (range: 32-37 weeks) and a standard deviation of 1.6 from three private hospitals, who passed both the distortion product otoacoustic emission (DPOAE) and AABR tests, were evaluated prior to discharge from the NICU. Neonates who presented with abnormal DPOAE and AABR results were excluded from the study. WAI was measured by using chirp and tone stimuli. In addition to reflectance, the reflectance area index (RAI) values were calculated. Both tone and chirp stimuli indicated high-power reflectance values below a frequency of 1.5 kHz. Median reflectance reached a minimum of 0.67 at 1 kHz - 2 kHz but increased to 0.7 below 1 kHz and 0.72 above 2 kHz for the tone stimuli. For chirp stimuli, the median reflectance reached a minimum of 0.51 at 1 kHz - 2 kHz but increased to 0.68 below 1 kHz and decreased to 0.5 above 2 kHz. A comparison between the present study and previous studies on WAI indicated a substantial variability across all frequency ranges. These WAI measurements conducted on at-risk preterm NICU neonates (mean age at testing: 35.6 weeks, range: 32-37 weeks) identified WAI patterns not previously reported in the literature. High reflective values were obtained across all frequency ranges. The age of the neonates when tested might have influenced the results. The neonates included in the present study were very young preterm neonates compared to the ages of neonates in previous studies. WAI measured in at-risk preterm neonates in the NICU was variable with environmental and internal noise influences. Transient conditions affecting the sound-conduction pathway might have influenced the results. Additional research is required to investigate WAI testing in ears with and without middle ear dysfunction. The findings of the current study imply that in preterm neonates it was not possible to determine the feasibility of WAI as a diagnostic tool to differentiate between ears with and without middle ear pathology.

Deriving habitat models for northern long-eared bats from historical detection data: A case study using the Fernow Experimental Forest

USGS Publications Warehouse

Ford, W. Mark; Silvis, Alexander; Rodrigue, Jane L.; Kniowski, Andrew B.; Johnson, Joshua B.

2016-01-01

The listing of the northern long-eared bat (Myotis septentrionalis) as federally threatened under the Endangered Species Act following severe population declines from white-nose syndrome presents considerable challenges to natural resource managers. Because the northern long-eared bat is a forest habitat generalist, development of effective conservation measures will depend on appropriate understanding of its habitat relationships at individual locations. However, severely reduced population sizes make gathering data for such models difficult. As a result, historical data may be essential in development of habitat models. To date, there has been little evaluation of how effective historical bat presence data, such as data derived from mist-net captures, acoustic detection, and day-roost locations, may be in developing habitat models, nor is it clear how models created using different data sources may differ. We explored this issue by creating presence probability models for the northern long-eared bat on the Fernow Experimental Forest in the central Appalachian Mountains of West Virginia using a historical, presence-only data set. Each presence data type produced outputs that were dissimilar but that still corresponded with known traits of the northern long-eared bat or are easily explained in the context of the particular data collection protocol. However, our results also highlight potential limitations of individual data types. For example, models from mist-net capture data only showed high probability of presence along the dendritic network of riparian areas, an obvious artifact of sampling methodology. Development of ecological niche and presence models for northern long-eared bat populations could be highly valuable for resource managers going forward with this species. We caution, however, that efforts to create such models should consider the substantial limitations of models derived from historical data, and address model assumptions.

Development, Translation and Validation of Enhanced Asian Rome III Questionnaires for Diagnosis of Functional Bowel Diseases in Major Asian Languages: A Rome Foundation-Asian Neurogastroenterology and Motility Association Working Team Report.

PubMed

Ghoshal, Uday C; Gwee, Kok-Ann; Chen, Minhu; Gong, Xiao R; Pratap, Nitesh; Hou, Xiaohua; Syam, Ari F; Abdullah, Murdani; Bak, Young-Tae; Choi, Myung-Gyu; Gonlachanvit, Sutep; Chua, Andrew S B; Chong, Kuck-Meng; Siah, Kewin T H; Lu, Ching-Liang; Xiong, Lishou; Whitehead, William E

2015-01-01

The development-processes by regional socio-cultural adaptation of an Enhanced Asian Rome III questionnaire (EAR3Q), a cultural adaptation of the Rome III diagnostic questionnaire (R3DQ), and its translation-validation in Asian languages are presented. As English is not the first language for most Asians, translation-validation of EAR3Q is essential. Hence, we aimed to culturally adapt the R3DQ to develop EAR3Q and linguistically validate it to show that the EAR3Q is able to allocate diagnosis according to Rome III criteria. After EAR3Q was developed by Asian experts by consensus, it was translated into Chinese, Hindi-Telugu, Indonesian, Korean and Thai, following Rome Foundation guidelines; these were then validated on native subjects (healthy [n = 60], and patients with irritable bowel syndrome [n = 59], functional dyspepsia [n = 53] and functional constipation [n = 61]) diagnosed by clinicians using Rome III criteria, negative alarm features and investigations. Experts noted words for constipation, bloating, fullness and heartburn, posed difficulty. The English back-translated questionnaires demonstrated concordance with the original EAR3Q. Sensitivity and specificity of the questionnaires were high enough to diagnose respective functional gastrointestinal disorders (gold standard: clinical diagnoses) in most except Korean and Indonesian languages. Questionnaires often uncovered overlapping functional gastrointestinal disorders. Test-retest agreement (kappa) values of the translated questionnaires were high (0.700-1.000) except in Korean (0.300-0.500) and Indonesian (0.100-0.400) languages at the initial and 2-week follow-up visit. Though Chinese, Hindi and Telugu translations were performed well, Korean and Indonesian versions were not. Questionnaires often uncovered overlapping FGIDs, which were quite common.

Using the Real-Ear-to-Coupler Difference within the American Academy of Audiology Pediatric Amplification Guideline: Protocols for Applying and Predicting Earmold RECDs.

PubMed

Moodie, Sheila; Pietrobon, Jonathan; Rall, Eileen; Lindley, George; Eiten, Leisha; Gordey, Dave; Davidson, Lisa; Moodie, K Shane; Bagatto, Marlene; Haluschak, Meredith Magathan; Folkeard, Paula; Scollie, Susan

2016-03-01

Real-ear-to-coupler difference (RECD) measurements are used for the purposes of estimating degree and configuration of hearing loss (in dB SPL ear canal) and predicting hearing aid output from coupler-based measures. Accurate measurements of hearing threshold, derivation of hearing aid fitting targets, and predictions of hearing aid output in the ear canal assume consistent matching of RECD coupling procedure (i.e., foam tip or earmold) with that used during assessment and in verification of the hearing aid fitting. When there is a mismatch between these coupling procedures, errors are introduced. The goal of this study was to quantify the systematic difference in measured RECD values obtained when using a foam tip versus an earmold with various tube lengths. Assuming that systematic errors exist, the second goal was to investigate the use of a foam tip to earmold correction for the purposes of improving fitting accuracy when mismatched RECD coupling conditions occur (e.g., foam tip at assessment, earmold at verification). Eighteen adults and 17 children (age range: 3-127 mo) participated in this study. Data were obtained using simulated ears of various volumes and earmold tubing lengths and from patients using their own earmolds. Derived RECD values based on simulated ear measurements were compared with RECD values obtained for adult and pediatric ears for foam tip and earmold coupling. Results indicate that differences between foam tip and earmold RECDs are consistent across test ears for adults and children which support the development of a correction between foam tip and earmold couplings for RECDs that can be applied across individuals. The foam tip to earmold correction values developed in this study can be used to provide improved estimations of earmold RECDs. This may support better accuracy in acoustic transforms related to transforming thresholds and/or hearing aid coupler responses to ear canal sound pressure level for the purposes of fitting behind-the-ear hearing aids. American Academy of Audiology.

Physiological artifacts in scalp EEG and ear-EEG.

PubMed

Kappel, Simon L; Looney, David; Mandic, Danilo P; Kidmose, Preben

2017-08-11

A problem inherent to recording EEG is the interference arising from noise and artifacts. While in a laboratory environment, artifacts and interference can, to a large extent, be avoided or controlled, in real-life scenarios this is a challenge. Ear-EEG is a concept where EEG is acquired from electrodes in the ear. We present a characterization of physiological artifacts generated in a controlled environment for nine subjects. The influence of the artifacts was quantified in terms of the signal-to-noise ratio (SNR) deterioration of the auditory steady-state response. Alpha band modulation was also studied in an open/closed eyes paradigm. Artifacts related to jaw muscle contractions were present all over the scalp and in the ear, with the highest SNR deteriorations in the gamma band. The SNR deterioration for jaw artifacts were in general higher in the ear compared to the scalp. Whereas eye-blinking did not influence the SNR in the ear, it was significant for all groups of scalps electrodes in the delta and theta bands. Eye movements resulted in statistical significant SNR deterioration in both frontal, temporal and ear electrodes. Recordings of alpha band modulation showed increased power and coherence of the EEG for ear and scalp electrodes in the closed-eyes periods. Ear-EEG is a method developed for unobtrusive and discreet recording over long periods of time and in real-life environments. This study investigated the influence of the most important types of physiological artifacts, and demonstrated that spontaneous activity, in terms of alpha band oscillations, could be recorded from the ear-EEG platform. In its present form ear-EEG was more prone to jaw related artifacts and less prone to eye-blinking artifacts compared to state-of-the-art scalp based systems.

QTL Mapping of Kernel Number-Related Traits and Validation of One Major QTL for Ear Length in Maize.

PubMed

Huo, Dongao; Ning, Qiang; Shen, Xiaomeng; Liu, Lei; Zhang, Zuxin

2016-01-01

The kernel number is a grain yield component and an important maize breeding goal. Ear length, kernel number per row and ear row number are highly correlated with the kernel number per ear, which eventually determines the ear weight and grain yield. In this study, two sets of F2:3 families developed from two bi-parental crosses sharing one inbred line were used to identify quantitative trait loci (QTL) for four kernel number-related traits: ear length, kernel number per row, ear row number and ear weight. A total of 39 QTLs for the four traits were identified in the two populations. The phenotypic variance explained by a single QTL ranged from 0.4% to 29.5%. Additionally, 14 overlapping QTLs formed 5 QTL clusters on chromosomes 1, 4, 5, 7, and 10. Intriguingly, six QTLs for ear length and kernel number per row overlapped in a region on chromosome 1. This region was designated qEL1.10 and was validated as being simultaneously responsible for ear length, kernel number per row and ear weight in a near isogenic line-derived population, suggesting that qEL1.10 was a pleiotropic QTL with large effects. Furthermore, the performance of hybrids generated by crossing 6 elite inbred lines with two near isogenic lines at qEL1.10 showed the breeding value of qEL1.10 for the improvement of the kernel number and grain yield of maize hybrids. This study provides a basis for further fine mapping, molecular marker-aided breeding and functional studies of kernel number-related traits in maize.

Numerical evaluation of implantable hearing devices using a finite element model of human ear considering viscoelastic properties.

PubMed

Zhang, Jing; Tian, Jiabin; Ta, Na; Huang, Xinsheng; Rao, Zhushi

2016-08-01

Finite element method was employed in this study to analyze the change in performance of implantable hearing devices due to the consideration of soft tissues' viscoelasticity. An integrated finite element model of human ear including the external ear, middle ear and inner ear was first developed via reverse engineering and analyzed by acoustic-structure-fluid coupling. Viscoelastic properties of soft tissues in the middle ear were taken into consideration in this model. The model-derived dynamic responses including middle ear and cochlea functions showed a better agreement with experimental data at high frequencies above 3000 Hz than the Rayleigh-type damping. On this basis, a coupled finite element model consisting of the human ear and a piezoelectric actuator attached to the long process of incus was further constructed. Based on the electromechanical coupling analysis, equivalent sound pressure and power consumption of the actuator corresponding to viscoelasticity and Rayleigh damping were calculated using this model. The analytical results showed that the implant performance of the actuator evaluated using a finite element model considering viscoelastic properties gives a lower output above about 3 kHz than does Rayleigh damping model. Finite element model considering viscoelastic properties was more accurate to numerically evaluate implantable hearing devices. © IMechE 2016.

An ear punch model for studying the effect of radiation on wound healing

PubMed Central

DeOLIVEIRA, DIVINO; JIAO, YIQUN; ROSS, JOEL R.; CORBIN, KAYLA; XIAO, QIZHEN; TONCHEVA, GRETA; ANDERSON-EVANS, COLIN; YOSHIZUMI, TERRY T.; CHEN, BENNY J.; CHAO, NELSON J.

2011-01-01

Purpose Radiation and wound combined injury represents a major clinical challenge because of the synergistic interactions that lead to higher morbidity and mortality than either insult would produce singly. The purpose of this study was to develop a mouse ear punch model to study the physiological mechanisms underlying radiation effects on healing wounds. Materials and methods Surgical wounds were induced by a 2 mm surgical punch in the ear pinnae of MRL/MpJ mice. Photographs of the wounds were taken and the sizes of the ear punch wounds were quantified by image analysis. Local radiation to the ear was delivered by orthovoltage X-ray irradiator using a specially constructed jig that shields the other parts of body. Results Using this model, we demonstrated that local radiation to the wound area significantly delayed the healing of ear punch wounds in a dose-dependent fashion. The addition of sublethal whole body irradiation (7 Gy) further delayed the healing of ear punch wounds. These results were replicated in C57BL/6 mice; however, wound healing in MRL/MpJ mice was accelerated. Conclusions These data indicate that the mouse ear punch model is a valuable model to study radiation and wound combined injury. PMID:21480768

Metal casts showing the three-dimensional structure of the human inner ear were converted into jewelry.

PubMed

Heywood, Peter

2015-06-01

This article describes a straightforward method for making metal casts of the human inner ear developed in 1937 by M. Wharton Young of Howard University College of Medicine. These casts were used to study anatomy, but there do not appear to be any published photographs of the casts. Inner ear casts converted into jewelry provide the only known images of this work. Later, Young studied the inner ear in living rhesus monkeys by injecting mercury into their membranous labyrinths. Young's investigations indicated a blind-ending perilymphatic sac that was not in continuity with the subarachnoid space.

The diagnostic value of earlier and later components of Vestibular Evoked Myogenic Potentials (VEMP) in multiple sclerosis.

PubMed

Eleftheriadou, A; Deftereos, S N; Zarikas, V; Panagopoulos, G; Sfetsos, S; Karageorgiou, C L; Ferekidou, E; Kandiloros, D; Korres, S

2009-01-01

To evaluate the ability of VEMP to disclose spatial dissemination of Multiple Sclerosis. Forty-six MS patients with auditory and/or vestibular symptoms were studied. Patients were divided in two groups. Group 1 included 24 patients with brainstem MRI findings, and Group 2 included 22 patients without MRI findings. VEMP and BAEP have been recorded and assessed. Abnormal p13n23 wave was observed in 50%, while unilateral absence or bilateral delay of the n34p44 in 43% of the patients. The overall diagnostic value considering abnormal cases suggested by both first and second VEMP waves was increased to 71%. Statistically significant differences revealed between patients and controls for p13 latency (p=0.018). The p13n23 was abnormal in 7 patients, although MRI scanning did not reveal brainstem lesions. In 9 out of 18 MS patients suffering from unilateral hearing loss, n34p44 was present in the unaffected ears and absent in the affected side, although p13n23 was normal. Abnormal VEMP imply the presence of lesions undetected by MRI neuroimaging, which verifies the diagnostic value of the method. Unilateral absence of n34p44 complex was related with sensorineural hearing loss, supporting the hypothesis that n34p44 is of cochlear origin.

The comparative anatomy of the pig middle ear cavity: a model for middle ear inflammation in the human?

PubMed Central

PRACY, J. P.; WHITE, A.; MUSTAFA, Y.; SMITH, D.; PERRY, M. E.

1998-01-01

This study was undertaken to develop a functional model of otitis media with effusion (OME) in the pig (Sus scrofa), with the purpose of investigating the origin of lymphocytes populating the middle ear during the course of an inflammatory process. The relevance of the model to the human condition of OME is to a large extent dependent on the anatomical and physiological similarities between the middle ear cavity and the pharyngeal lymphoid tissue of the pig and man. Anatomical specimens were collected from 7 young Large White pigs to determine the gross anatomy of the middle ear cavity and the histological characteristics of the middle ear mucosa. It was found that the anatomy of the 3 parts of the middle ear cavity in man and in the pig is broadly similar, although some minor differences were observed. The porcine eustachian tube was seen to be cartilaginous throughout its length in contrast to the part osseous, part cartilaginous structure found in man; the porcine ossicles were slightly different in shape to those of man and the air cell system was situated inferior to the tympanic cavity in the pig as opposed to posteriorly in man. This paper describes the structure and morphology of the pig middle ear cavity and compares and contrasts it with that of man. The minor differences observed are of anatomical importance but do not diminish the usefulness of the pig middle ear cleft as a potential model for human middle ear disorders. PMID:9688502

Analysis of the Effect of Locally Applied Inhomogeneous Static Magnetic Field-Exposure on Mouse Ear Edema – A Double Blind Study

PubMed Central

Kiss, Balázs; László, János F.; Szalai, Andrea; Pórszász, Róbert

2015-01-01

The effect static magnetic field (SMF)-exposure may exert on edema development has been investigated. A 6 h long whole-body (WBSMF) or local (LSMF), continuous, inhomogeneous SMF-exposure was applied on anesthetized mice in an in vivo model of mustard oil (MO)-induced ear edema. LSMF was applied below the treated ear, below the lumbar spine, or below the mandible. Ear thickness (v) was checked 8 times during the exposure period (at 0, 0.25, 1, 2, 3, 4, 5, and 6 h). The effect size of the applied treatment (η) on ear thickness was calculated by the formula η = 100% × (1–v j/v i), where group i is the control group and j is the treated group. Results showed that MO treatment in itself induced a significant ear edema with an effect of 9% (p<0.001). WBSMF or LSMF on the spine in combination with MO treatment increased ear thickness even further resulting in an effect of η>11% in both cases compared to SMF-exposure alone (p<0.001). In these cases SMF-exposure alone without MO treatment reduced ear thickness significantly (p<0.05), but within estimated experimental error. In cases of LSMF-exposure on the head, a significant SMF-exposure induced ear thickness reduction was found (η = 5%, p<0.05). LSMF-exposure on the spine affected ear thickness with and without MO treatment almost identically, which provides evidence that the place of local SMF action may be in the lower spinal region. PMID:25695832

Naturalistic and Experimental Analyses of Word Frequency and Neighborhood Density Effects in Slips of the Ear*

PubMed Central

Vitevitch, Michael S.

2008-01-01

A comparison of the lexical characteristics of 88 auditory misperceptions (i.e., slips of the ear) showed no difference in word-frequency, neighborhood density, and neighborhood frequency between the actual and the perceived utterances. Another comparison of slip of the ear tokens (i.e., actual and perceived utterances) and words in general (i.e., randomly selected from the lexicon) showed that slip of the ear tokens had denser neighborhoods and higher neighborhood frequency than words in general, as predicted from laboratory studies. Contrary to prediction, slip of the ear tokens were higher in frequency of occurrence than words in general. Additional laboratory-based investigations examined the possible source of the contradictory word frequency finding, highlighting the importance of using naturalistic and experimental data to develop models of spoken language processing. PMID:12866911

Fetal development of the elastic-fiber-mediated enthesis in the human middle ear.

PubMed

Takanashi, Yoshitaka; Shibata, Shunichi; Katori, Yukio; Murakami, Gen; Abe, Shinichi; Rodríguez-Vázquez, Jose Francisco; Kawase, Tetsuaki

2013-10-01

In the human middle ear, the annular ligament of the incudostapedial joint and the insertions of the tensor tympani and stapedius muscles contain abundant elastic fibers; i.e., the elastic-fiber-mediated entheses. Hyaluronan also coexists with the elastic fibers. In the present study using immunohistochemistry, we demonstrated the distribution of elastin not only in the incudostapedial joint but also in the other two joints of the middle ear in adults and fetuses. In adults, the expression of elastin did not extend out of the annular ligament composed of mature elastic fibers but clearly overlapped with it. Electron microscopic observations of the annular ligament demonstrated a few microfibrils along the elastic fibers. Thus, in contrast to the vocal cord, the middle ear entheses seemed not to contain elaunin and oxytalan fibers. In mid-term fetuses (at approximately 15-16 weeks of gestation) before opening of the external acoustic meatus, the incudostapedial joint showed abundant elastic fibers, but the incudomalleolar and stapediovestibular joints did not. At this stage, hyaluronan was not colocalized, but distributed diffusely in loose mesenchymal tissues surrounding the ear ossicles. Therefore, fetal development of elastin and elastic fibers in the middle ear entheses is unlikely to require acoustic oscillation. In late-stage fetuses (25-30 weeks), whose ear ossicles were almost the same size as those in adults, we observed bundling and branching of elastic fibers. However, hyaluronan expression was not as strong as in adults. Colocalization between elastic fibers and hyaluronan appeared to be a result of postnatal maturation of the entheses. Copyright © 2013 Elsevier GmbH. All rights reserved.

Inner ear test battery in guinea pig models - a review.

PubMed

Young, Yi-Ho

2018-06-01

This study reviewed the development of the inner ear test battery comprising auditory brainstem response (ABR), and caloric, ocular vestibular-evoked myogenic potential (oVEMP), and cervical vestibular-evoked myogenic potential (cVEMP) tests in guinea pig models at our laboratory over the last 20 years. Detailed description of the methodology for testing the small animals is also included. Inner ear disorders, i.e. ototoxicity, noise exposure, or perilymph fistula were established in guinea pig models first. One to four weeks after operation, each animal underwent ABR, oVEMP, cVEMP, and caloric tests. Then, animals were sacrificed for morphological study in the temporal bones. Inner ear endorgans can be comprehensively evaluated in guinea pig models via an inner ear test battery, which provides thorough information on the cochlea, saccule, utricle, and semicircular canal function of guinea pigs. Coupled with morphological study in the temporal bones of the animals may help elucidate the mechanism of inner ear disorders in humans. The inner ear test battery in guinea pig models may encourage young researchers to perform basic study in animals and stimulate the progress of experimental otology which is in evolution.

Effect of Absence of Developing Grain on Carbohydrate Content and Senescence of Maize Leaves

PubMed Central

Allison, J. C. S.; Weinmann, H.

1970-01-01

In maize (Zea mays L.) grown under normal conditions in Rhodesia, prevention of pollination or removal of the ears after flowering caused premature senescence of the leaves above the ear, preceded by the appearance of a purplish red color. In plants from which the ears had been removed the concentration of sugars and starch increased markedly in both upper and lower leaves, the increase being greater in the upper leaves. PMID:16657481

Body dysmorphic disorder in the dermatology patient.

PubMed

Koblenzer, Caroline S

Body dysmorphic disorder is primarily a psychiatric disorder, in which the patient believes that some normal or very near normal aspect of his or her physical appearance is distorted or ugly. Should there be a minor abnormality, it is grossly exaggerated in the mind of the patient, causing feelings of shame and embarrassment and leading daily to spending hours at the mirror, or any reflecting surface, as the patient tries to conceal or remove the perceived abnormality through the development of ritualistic behavior. Although other organs can be involved-for example, the shape of the nose or a portion of an ear- the skin, hair, and nails are most commonly involved, while the patient constantly seeks reassurance about appearance from friends and family. There is a broad spectrum of severity in body dysmorphic disorder, ranging from obsessional worry to frank delusion, and the psychiatric comorbidities-anxiety, depression, and personality disorder-are prominent parts of the picture. Unfortunately, the psychiatric comorbidities and the negative impact on every aspect of the patient's life may not be recognized by dermatologists and other non-psychiatric physicians, so that effective treatment is often not instituted or appropriate referrals made. This paper describes the incidence, possible etiologies, and clinical picture of body dysmorphic disorder in dermatology patients and discusses interpersonal approaches that may permit appropriate treatment or referral to take place. Specific treatments and prognosis are also discussed. Copyright © 2017 Elsevier Inc. All rights reserved.

Abnormalities in auditory efferent activities in children with selective mutism.

PubMed

Muchnik, Chava; Ari-Even Roth, Daphne; Hildesheimer, Minka; Arie, Miri; Bar-Haim, Yair; Henkin, Yael

2013-01-01

Two efferent feedback pathways to the auditory periphery may play a role in monitoring self-vocalization: the middle-ear acoustic reflex (MEAR) and the medial olivocochlear bundle (MOCB) reflex. Since most studies regarding the role of auditory efferent activity during self-vocalization were conducted in animals, human data are scarce. The working premise of the current study was that selective mutism (SM), a rare psychiatric disorder characterized by consistent failure to speak in specific social situations despite the ability to speak normally in other situations, may serve as a human model for studying the potential involvement of auditory efferent activity during self-vocalization. For this purpose, auditory efferent function was assessed in a group of 31 children with SM and compared to that of a group of 31 normally developing control children (mean age 8.9 and 8.8 years, respectively). All children exhibited normal hearing thresholds and type A tympanograms. MEAR and MOCB functions were evaluated by means of acoustic reflex thresholds and decay functions and the suppression of transient-evoked otoacoustic emissions, respectively. Auditory afferent function was tested by means of auditory brainstem responses (ABR). Results indicated a significantly higher proportion of children with abnormal MEAR and MOCB function in the SM group (58.6 and 38%, respectively) compared to controls (9.7 and 8%, respectively). The prevalence of abnormal MEAR and/or MOCB function was significantly higher in the SM group (71%) compared to controls (16%). Intact afferent function manifested in normal absolute and interpeak latencies of ABR components in all children. The finding of aberrant efferent auditory function in a large proportion of children with SM provides further support for the notion that MEAR and MOCB may play a significant role in the process of self-vocalization. © 2013 S. Karger AG, Basel.

Changes in Properties of Auditory Nerve Synapses following Conductive Hearing Loss.

PubMed

Zhuang, Xiaowen; Sun, Wei; Xu-Friedman, Matthew A

2017-01-11

Auditory activity plays an important role in the development of the auditory system. Decreased activity can result from conductive hearing loss (CHL) associated with otitis media, which may lead to long-term perceptual deficits. The effects of CHL have been mainly studied at later stages of the auditory pathway, but early stages remain less examined. However, changes in early stages could be important because they would affect how information about sounds is conveyed to higher-order areas for further processing and localization. We examined the effects of CHL at auditory nerve synapses onto bushy cells in the mouse anteroventral cochlear nucleus following occlusion of the ear canal. These synapses, called endbulbs of Held, normally show strong depression in voltage-clamp recordings in brain slices. After 1 week of CHL, endbulbs showed even greater depression, reflecting higher release probability. We observed no differences in quantal size between control and occluded mice. We confirmed these observations using mean-variance analysis and the integration method, which also revealed that the number of release sites decreased after occlusion. Consistent with this, synaptic puncta immunopositive for VGLUT1 decreased in area after occlusion. The level of depression and number of release sites both showed recovery after returning to normal conditions. Finally, bushy cells fired fewer action potentials in response to evoked synaptic activity after occlusion, likely because of increased depression and decreased input resistance. These effects appear to reflect a homeostatic, adaptive response of auditory nerve synapses to reduced activity. These effects may have important implications for perceptual changes following CHL. Normal hearing is important to everyday life, but abnormal auditory experience during development can lead to processing disorders. For example, otitis media reduces sound to the ear, which can cause long-lasting deficits in language skills and verbal production, but the location of the problem is unknown. Here, we show that occluding the ear causes synapses at the very first stage of the auditory pathway to modify their properties, by decreasing in size and increasing the likelihood of releasing neurotransmitter. This causes synapses to deplete faster, which reduces fidelity at central targets of the auditory nerve, which could affect perception. Temporary hearing loss could cause similar changes at later stages of the auditory pathway, which could contribute to disorders in behavior. Copyright © 2017 the authors 0270-6474/17/370323-10$15.00/0.

Micro-endoscopic ear anatomy of guinea pig applied to experimental surgery.

PubMed

Barros, Bruno Borges de Carvalho; Andrade, José Santos Cruz de; Garcia, Leandro Borborema; Pifaia, Gustavo Ribeiro; Cruz, Oswaldo Laércio Mendonça; Onishi, Ektor Tsuneo; Penido, Norma de Oliveira

2014-01-01

To describe topographic and endoscopic anatomy of guinea pig ear for development of surgical approaches in experimental studies. Experimental study. Eight adult guinea pigs (Cavia porcellus) were used in this study. Four animals were described through endoscopic view and four animals were used to describe topographic anatomy. The main structures of middle ear were well identified through endoscopy view: oval and round window, ossicles and vascular structures. Temporal bone position, landmarks and its relations to skull are perceived with topographic description. Topographic anatomic description allowed exposition of temporal bone relations for external surgical approaches. Alternatively, grooves and middle ear structures were identified and may be used to transcanal accesses.

Exceeding Parents' Expectations in Ear-Nose-Throat Outpatient Facilities: The Development and Analysis of a Questionnaire

ERIC Educational Resources Information Center

Margaritis, Eleftherios; Katharaki, Maria; Katharakis, George

2012-01-01

The study attempts to develop an outpatient service quality scale by investigating the key dimensions which assess parental satisfaction and provides a recommendation on an improved health service delivery system. The survey was conducted in an Ear-Nose-Throat outpatient clinic of a Greek public pediatric hospital. A total of 127 parents in…

Impact of Bug-in-Ear Professional Development on Early Childhood Co-Teachers' Use of Communication Strategies

ERIC Educational Resources Information Center

Ottley, Jennifer R.; Grygas Coogle, Christan; Rahn, Naomi L.; Spear, Caitlin F.

2017-01-01

The goal of this study was to build the capacity of early childhood teachers to implement evidence-based strategies. We investigated the efficacy of professional development with bug-in-ear peer coaching in improving teachers' use of communication strategies, the teachers' maintenance of strategies post intervention, and the social validity of the…

Impact of Bug-in-Ear Professional Development on Early Childhood Co-Teachers' Use of Communication Strategies

ERIC Educational Resources Information Center

Ottley, Jennifer R.; Coogle, Christan G.; Rahn, Naomi L.; Spear, Caitlin F.

2016-01-01

The goal of this study was to build the capacity of early childhood teachers to implement evidence-based strategies. We investigated the efficacy of professional development with bug-in-ear peer coaching in improving teachers' use of communication strategies, the teachers' maintenance of strategies post intervention, and the social validity of the…

[A case of pycnodysostosis--observation of the skull by CT scan].

PubMed

Anegawa, S; Bekki, Y; Furukawa, Y; Yokota, S; Torigoe, R

1987-07-01

A 13-year-old boy was presented to the Department of Neurosurgery, Saiseikai Fukuoka General Hospital for further examinations concerning abnormal findings in the skull radiogram taken when he struck his head. His physical features showed some characteristics the same as those of pycnodysostosis as follows--proportionate dwarfism, prominent forehead, short spoon-shaped fingers, bilateral exophthalmos. A skull radiogram revealed widely open cranial sutures with no healing of the fracture and craniotomy which was performed for an acute epidural hematoma 6 years ago. Furthermore, the mandible was hypoplastic with a virtual loss of mandibular angle. CT of the soft tissues showed somewhat dilated cortical sulci and ventricles without any structural abnormalities in the brain. CT of bone algorithm revealed specific characteristics of this disease. The paranasal sinuses were quite hypoplastic. Especially in the maxillary sinuses, frontal sinuses and mastoid air cells, none of developments of sinuses were noted, even though the middle and internal ear seemed to be normal. Moreover, the ethmoid and sphenoid sinuses were noted, although their developments were poor. The appearance of skull base was normal, including the inlets and outlets of cranial nerves or vessels and synchondroses. However, the density of the skull base, especially in the diploe, was higher than normal in Hansfield number. Furthermore, detailed measurements of skull base demonstrated that the skull base itself was also dwarfism. Pycnodysostosis is a generalized skeletal disease whose cardinal features are moderate generalized osteosclerosis and dwarfism. However, the detailed observation on the cranium by CT has not been reported. In our study, the development of sinuses in bones with intramembranous ossification are worse than that with endochondral ossification.(ABSTRACT TRUNCATED AT 250 WORDS)

A review of gene delivery and stem cell based therapies for regenerating inner ear hair cells.

PubMed

Devarajan, Keerthana; Staecker, Hinrich; Detamore, Michael S

2011-09-13

Sensory neural hearing loss and vestibular dysfunction have become the most common forms of sensory defects, affecting millions of people worldwide. Developing effective therapies to restore hearing loss is challenging, owing to the limited regenerative capacity of the inner ear hair cells. With recent advances in understanding the developmental biology of mammalian and non-mammalian hair cells a variety of strategies have emerged to restore lost hair cells are being developed. Two predominant strategies have developed to restore hair cells: transfer of genes responsible for hair cell genesis and replacement of missing cells via transfer of stem cells. In this review article, we evaluate the use of several genes involved in hair cell regeneration, the advantages and disadvantages of the different viral vectors employed in inner ear gene delivery and the insights gained from the use of embryonic, adult and induced pluripotent stem cells in generating inner ear hair cells. Understanding the role of genes, vectors and stem cells in therapeutic strategies led us to explore potential solutions to overcome the limitations associated with their use in hair cell regeneration.

Formation of otoconia in the Japanese red-bellied newt, Cynops pyrrhogaster

NASA Technical Reports Server (NTRS)

Wiederhold, M. L.; Yamashita, M.; Larsen, K.; Asashima, M.

1994-01-01

Pre-mated adult female newts and fertilized eggs will be flown on the International Microgravity Laboratory-2 flight, in 1994. One objective of the flight will be to observe the influence of microgravity on the development of the gravity-sensing organs in the inner ear. These organs contain sensory hair cells covered by a layer of dense stones (otoconia). Gravity and linear acceleration exert forces on these masses, leading to excitation of the nerve fibers innervating the hair cells. If the production of the otoliths is regulated to reach an optimal weight, their development might be abnormal in microgravity. Ground-based control experiments are reported describing the developmental sequence in which both the otoliths and their associated sensory epithelium and the semicircular canals appear and develop. Three-dimensional reconstruction of serial sections through the otic vesicle of newt embryos at stages 31 through 58 demonstrate the first appearance, relative position and growth of the otoliths. Reports of experiments in which fertilized frog eggs were flown on a Russian Cosmos mission conclude that the utricular otolith is increased in volume, whereas the saccular otolith maintains normal size, suggesting that at least in the utricle, the weight of the otolith might be regulated.

Perspectives on the use of the baboon in embryology and teratology research.

PubMed

Hendrickx, A G; Peterson, P E

1997-01-01

This paper summarizes the developmental stages of the baboon during the period of organ formation and provides comparative information for other primates, including the human. Special attention is directed to the early development of the nervous system, eye, ear and nose/palate. The similarity in development of these structures with humans indicates that the baboon is a suitable model for studies of normal and abnormal neurological development. Spontaneous prenatal loss rates in the baboon (2.4-11.2%) are slightly lower than those reported in rhesus and cynomologus monkeys. The baboon, in addition to the cynomologus monkey and macaque, has been used as a model in teratology research to assess the potential risk of thalidomide, sex steroids, Bendectin and rubella virus, as well as to study the pathogenesis of malformations associated with the corticosteroid triamcinolone acetonide. The rate of spontaneous malformations (<1%) in baboons, similar to that reported for other commonly used primates, supports their continued use as a teratological model. In this regard, a sample protocol is provided for the safety evaluation of biotechnology products using nonhuman primates, which are the most appropriate model for those compounds which are bioactive in species closely related to humans.

A microbiome case-control study of recurrent acute otitis media identified potentially protective bacterial genera.

PubMed

Lappan, Rachael; Imbrogno, Kara; Sikazwe, Chisha; Anderson, Denise; Mok, Danny; Coates, Harvey; Vijayasekaran, Shyan; Bumbak, Paul; Blyth, Christopher C; Jamieson, Sarra E; Peacock, Christopher S

2018-02-20

Recurrent acute otitis media (rAOM, recurrent ear infection) is a common childhood disease caused by bacteria termed otopathogens, for which current treatments have limited effectiveness. Generic probiotic therapies have shown promise, but seem to lack specificity. We hypothesised that healthy children with no history of AOM carry protective commensal bacteria that could be translated into a specific probiotic therapy to break the cycle of re-infection. We characterised the nasopharyngeal microbiome of these children (controls) in comparison to children with rAOM (cases) to identify potentially protective bacteria. As some children with rAOM do not appear to carry any of the known otopathogens, we also hypothesised that characterisation of the middle ear microbiome could identify novel otopathogens, which may also guide the development of more effective therapies. Middle ear fluids, middle ear rinses and ear canal swabs from the cases and nasopharyngeal swabs from both groups underwent 16S rRNA gene sequencing. The nasopharyngeal microbiomes of cases and controls were distinct. We observed a significantly higher abundance of Corynebacterium and Dolosigranulum in the nasopharynx of controls. Alloiococcus, Staphylococcus and Turicella were abundant in the middle ear and ear canal of cases, but were uncommon in the nasopharynx of both groups. Gemella and Neisseria were characteristic of the case nasopharynx, but were not prevalent in the middle ear. Corynebacterium and Dolosigranulum are characteristic of a healthy nasopharyngeal microbiome. Alloiococcus, Staphylococcus and Turicella are possible novel otopathogens, though their rarity in the nasopharynx and prevalence in the ear canal means that their role as normal aural flora cannot be ruled out. Gemella and Neisseria are unlikely to be novel otopathogens as they do not appear to colonise the middle ear in children with rAOM.

The Electronically Activated Recorder (EAR): A Method for the Naturalistic Observation of Daily Social Behavior

PubMed Central

Mehl, Matthias R.

2016-01-01

This article reviews the Electronically Activated Recorder or EAR as an ambulatory ecological momentary assessment tool for the real-world observation of daily behavior. Technically, the EAR is an audio recorder that intermittently records snippets of ambient sounds while participants go about their lives. Conceptually, it is a naturalistic observation method that yields an acoustic log of a person’s day as it unfolds. The power of the EAR lies in unobtrusively collecting authentic real-life observational data. In preserving a high degree of naturalism at the level of the raw recordings, it resembles ethnographic methods; through its sampling and coding, it enables larger empirical studies. The article provides an overview of the EAR method, reviews its validity, utility, and limitations, and discusses it in the context of current developments in ambulatory assessment, specifically the emerging field of mobile sensing. PMID:28529411

The Electronically Activated Recorder (EAR): A Method for the Naturalistic Observation of Daily Social Behavior.

PubMed

Mehl, Matthias R

2017-04-01

This article reviews the Electronically Activated Recorder or EAR as an ambulatory ecological momentary assessment tool for the real-world observation of daily behavior. Technically, the EAR is an audio recorder that intermittently records snippets of ambient sounds while participants go about their lives. Conceptually, it is a naturalistic observation method that yields an acoustic log of a person's day as it unfolds. The power of the EAR lies in unobtrusively collecting authentic real-life observational data. In preserving a high degree of naturalism at the level of the raw recordings, it resembles ethnographic methods; through its sampling and coding, it enables larger empirical studies. The article provides an overview of the EAR method, reviews its validity, utility, and limitations, and discusses it in the context of current developments in ambulatory assessment, specifically the emerging field of mobile sensing.

Self-demodulation of amplitude-modulated signal components in amplitude-modulated bone-conducted ultrasonic hearing

NASA Astrophysics Data System (ADS)

Ito, Kazuhito; Nakagawa, Seiji

2015-07-01

A novel hearing aid system utilizing amplitude-modulated bone-conducted ultrasound (AM-BCU) is being developed for use by profoundly deaf people. However, there is a lack of research on the acoustic aspects of AM-BCU hearing. In this study, acoustic fields in the ear canal under AM-BCU stimulation were examined with respect to the self-demodulation effect of amplitude-modulated signal components generated in the ear canal. We found self-demodulated signals with an audible sound pressure level related to the amplitude-modulated signal components of bone-conducted ultrasonic stimulation. In addition, the increases in the self-demodulated signal levels at low frequencies in the ear canal after occluding the ear canal opening, i.e., the positive occlusion effect, indicate the existence of a pathway by which the self-demodulated signals pass through the aural cartilage and soft tissue, and radiate into the ear canal.

Whole exome sequencing identifies a POLRID mutation segregating in a father and two daughters with findings of Klippel-Feil and Treacher Collins syndromes.

PubMed

Giampietro, Philip F; Armstrong, Linlea; Stoddard, Alex; Blank, Robert D; Livingston, Janet; Raggio, Cathy L; Rasmussen, Kristen; Pickart, Michael; Lorier, Rachel; Turner, Amy; Sund, Sarah; Sobrera, Nara; Neptune, Enid; Sweetser, David; Santiago-Cornier, Alberto; Broeckel, Ulrich

2015-01-01

We report on a father and his two daughters diagnosed with Klippel-Feil syndrome (KFS) but with craniofacial differences (zygomatic and mandibular hypoplasia and cleft palate) and external ear abnormalities suggestive of Treacher Collins syndrome (TCS). The diagnosis of KFS was favored, given that the neck anomalies were the predominant manifestations, and that the diagnosis predated later recognition of the association between spinal segmentation abnormalities and TCS. Genetic heterogeneity and the rarity of large families with KFS have limited the ability to identify mutations by traditional methods. Whole exome sequencing identified a nonsynonymous mutation in POLR1D (subunit of RNA polymerase I and II): exon2:c.T332C:p.L111P. Mutations in POLR1D are present in about 5% of individuals diagnosed with TCS. We propose that this mutation is causal in this family, suggesting a pathogenetic link between KFS and TCS. © 2014 Wiley Periodicals, Inc.

Fetus with Casamassima-Morton-Nance Syndrome and Limb-Body Wall Defect: Presentation of a Novel Association and Review of the Phenotype.

PubMed

Salinas-Torres, Victor M

2016-01-01

In 1981, Casamassima and colleagues described an autosomal recessive syndrome of spondylocostal dysostosis associated with anal and urogenital anomalies. Here, I describe 1 new fetus who presented with limb-body wall defect as a novel association, compile 7 patients, and review the clinical phenotype of Casamassima-Morton-Nance syndrome. This appears to be the 1st Casamassima-Morton-Nance syndrome fetus with this complex malformation. In light of this manifestation, a detailed comparative phenotypic analysis of published patients revealed a heterogeneous syndrome with significant clinical variability. Accordingly, it is proposed that Casamassima-Morton-Nance syndrome should be considered in those patients with the combination of a short and asymmetric thorax with rib and vertebral anomalies and scoliosis (spondylocostal-like pattern), anal atresia, absent external genitalia, renal and urethral abnormalities (caudal dysgenesis complex), craniofacial dysmorphic features (mainly flat nose with anteverted nares, low-set/abnormal ears, and short neck), hydrops, oligohydramnios, and a poor clinical outcome.

Inheritance of a Balanced t(12;20)(q24.33;p12.2) and Unbalanced der(13)t(7;13)(p21.3;q33.2) from a Maternally Derived Double Balanced Translocation Carrier.

PubMed

Peterson, Jess F; Geddes, Gabrielle C; Basel, Donald G; Schippman, Dana; Grignon, John W; vanTuinen, Peter; Kappes, Ulrike P

2018-03-01

We report a 4-month-old male proband with a history of prominent forehead, hypertelorism, ear abnormalities, micrognathia, hypospadias, and multiple cardiac abnormalities. Initial microarray analysis detected a concurrent 7p21.3-p22.3 duplication and 13q33.2-q34 deletion indicating an unbalanced rearrangement. However, subsequent conventional cytogenetic studies only revealed what appeared to be a balanced t(12;20)(q24.33;p12.2). Fluorescence in situ hybridization (FISH) using chromosome-specific subtelomere probes confirmed the presence of an unbalanced der(13)t(7;13)(p21.3;q33.2) and balanced t(12;20)(q24.33;p12.2), both of maternal origin. In addition to our unique clinical findings, this case highlights the benefits and limitations of both conventional cytogenetic studies and microarray analysis and how FISH complements each methodology.

Double trisomy 48,XXX,+18 with multiple dysmorphic features.

PubMed

Jiang, Zi-Yan; Wu, Xiao-Hui; Zou, Chao-Chun

2015-02-01

Chromosomal abnormality is a common cause of congenital anomalies, psychiatric disorders, and mental retardation. However, the double trisomy 48,XXX,+18 is a rare chromosome abnormality. Case report and literature review. A 7-hour-old girl presented to our unit because of poor response after birth. She presented with multiple dysmorphic features, including small for gestational age infant, flat nasal bridge, widely-spaced eyes, the left thumb deformities, flat facial profile, raised sternum, ventricular septal defect, the third lateral brain ventricle enlargement, and small liver. This case expands the spectrum of malformations reported in association with the double trisomy 48,XXX,+18. The literature on 16 fetuses or infants with the 48,XXX,+18 were also reviewed. These data suggested that in patients with clinical features similar to trisomy 18, especially with anomalies of the ears and/or reproductive malformations, double trisomy (48,XXX,+18) should be considered and karyotyping should be performed although it is a rare disease.

Assessment of central auditory processing in a group of workers exposed to solvents.

PubMed

Fuente, Adrian; McPherson, Bradley; Muñoz, Verónica; Pablo Espina, Juan

2006-12-01

Despite having normal hearing thresholds and speech recognition thresholds, results for central auditory tests were abnormal in a group of workers exposed to solvents. Workers exposed to solvents may have difficulties in everyday listening situations that are not related to a decrement in hearing thresholds. A central auditory processing disorder may underlie these difficulties. To study central auditory processing abilities in a group of workers occupationally exposed to a mix of organic solvents. Ten workers exposed to a mix of organic solvents and 10 matched non-exposed workers were studied. The test battery comprised pure-tone audiometry, tympanometry, acoustic reflex measurement, acoustic reflex decay, dichotic digit, pitch pattern sequence, masking level difference, filtered speech, random gap detection and hearing-in-noise tests. All the workers presented normal hearing thresholds and no signs of middle ear abnormalities. Workers exposed to solvents had lower results in comparison with the control group and previously reported normative data, in the majority of the tests.

Dental and craniofacial characteristics in a patient with Dubowitz syndrome: a case report.

PubMed

Ballini, Andrea; Cantore, Stefania; Tullo, Domenica; Desiate, Apollonia

2011-01-27

Dubowitz syndrome is a very rare, autosomal recessive disease characterized by microcephaly, growth retardation, a high sloping forehead, facial asymmetry, blepharophimosis, sparse hair and eyebrows, low-set ears and mental retardation. Symptoms vary between patients, but other characteristics include a soft high-pitched voice, dental and craniofacial abnormalities, partial webbing of the fingers and toes, palate deformations, genital abnormalities, eczema, hyperactivity, preference for concrete over abstract thinking, language difficulties and an aversion to crowds. We describe the craniofacial and dental characteristics of a 12-year-old Caucasian Italian boy with both the typical and less common findings of Dubowitz syndrome. Diagnosis of Dubowitz syndrome is mainly based on the facial phenotype. Possible conditions for differential diagnosis include Bloom syndrome, Smith-Lemli-Opitz syndrome, and fetal alcohol syndrome. As there are few reports of this syndrome in the literature, we hope this case report will enable health professionals to recognize the phenotypic alterations of this syndrome, and allow early referral for the necessary multidisciplinary treatments.

Rugby injury-associated pseudocyst of the auricle: report and review of sports-associated dermatoses of the ear.

PubMed

Kallini, Joseph R; Cohen, Philip R

2013-02-15

To describe a man with pseudocyst of the auricle, summarize the salient features of this condition, and review other sports-associated dermatoses of the ear. Pseudocyst of the auricle is an intracartilaginous collection of viscous straw-colored fluid typically located in the triangular fossa of the upper half of the auricle. It is usually asymptomatic. It can result in a permanent auricular deformity. A 63-year-old man developed a pseudocyst of the auricle following a traumatic rugby-related injury to his left ear. The lesion has persisted for many years; chronic massage has slightly decreased its size. The etiology of pseudocyst of the auricle has been described as either traumatic or developmental. Our patient developed his pseudocyst after a rugby-related injury. This condition is usually unilateral. Aspirate of the content is usually sterile. Histology shows an intracartilaginous cyst devoid of an epithelial lining. Treatment involves either partial removal of the cartilage or chemical irritation to enhance adhesiveness. Subsequent compression (via button bolsters) minimizes recurrence. Pseudocyst of the auricle is a benign cystic dilatation, which is intracartilaginous, devoid of an epithelial lining, and may be traumatic or non-traumatic in origin. A man with rugby-associated trauma to his left ear developed this condition. Auricular pseudocyst can be added to the list of sports-associated dermatoses of the ear.

The Role of CHD7 Mutations in Patients with Idiopathic Hypogonadotropic Hypogonadism and Kallmann Syndrome

PubMed Central

Kim, Hyung-Goo; Layman, Lawrence C.

2013-01-01

Mutations in the chromodomain helicase DNA binding protein-7 (CHD7) cause CHARGE syndrome, which includes eye coloboma, heart malformations, atresia of the choanae, retardation of growth/development, genital anomalies, and ear abnormalities. CHARGE syndrome is usually sporadic, but is also autosomal dominant. CHD7 encodes a large protein that participates in chromatin remodeling and transcription. Findings from studies of mouse models employing ENU-mutagenesis or gene-trap methods recapitulate human CHARGE syndrome. CHARGE patients may manifest anosmia and/or hypogonadism, features that overlap with idiopathic hypogonadotropic hypogonadism (IHH) and Kallmann syndrome (KS). Similarly, IHH/KS patients may also display partial CHARGE features. Therefore, it has been hypothesized that IHH/KS represents a milder allelic variant of CHARGE syndrome, which has been supported by the identification of heterozygous CHD7 mutations in both normosmic IHH and KS. Developmental expression within the hypothalamus and the presence of human mutations indicate that CHD7 has an important role in puberty and reproduction. PMID:21856375

Oral features and computerized rehabilitation of a young patient with CHARGE syndrome using minimally invasive long-term interim CAD-CAM restorations.

PubMed

Liebermann, Anja; Rafael, Caroline Freitas; Edelhoff, Daniel; Ramberger, Marc; Schweiger, Josef; Maziero Volpato, Claudia Angela; Saeidi Pour, Reza

2017-04-01

Patients with CHARGE syndrome (where CHARGE stands for coloboma of the iris or retina, heart defects or cardiac malformations, atresia/stenosis of the choanae, retardation of growth and development, genital anomalies, and ear abnormalities) present several orofacial anomalies. Their treatment depends on the specific type of manifestation. To perform the complex oral rehabilitation and achieve a conservative, esthetic, and functional exploration of the definitive treatment goal, computer-aided design and computer-aided manufacturing (CAD-CAM) polymers can be used as long-term interim restorations. This article reports the treatment of a young patient with CHARGE syndrome combined with oral alterations. CAD-CAM polymers offer an intermediate treatment with satisfying esthetics and function at low biological cost until bone growth is completed. This period facilitates additional planning for the definitive restoration. Copyright © 2016 Editorial Council for the Journal of Prosthetic Dentistry. Published by Elsevier Inc. All rights reserved.

Otolith and Vertical Canal Contributions to Dynamic Postural Control

NASA Technical Reports Server (NTRS)

Black, F. Owen

1999-01-01

The objective of this project is to determine: 1) how do normal subjects adjust postural movements in response to changing or altered otolith input, for example, due to aging? and 2) how do patients adapt postural control after altered unilateral or bilateral vestibular sensory inputs such as ablative inner ear surgery or ototoxicity, respectively? The following hypotheses are under investigation: 1) selective alteration of otolith input or abnormalities of otolith receptor function will result in distinctive spatial, frequency, and temporal patterns of head movements and body postural sway dynamics. 2) subjects with reduced, altered, or absent vertical semicircular canal receptor sensitivity but normal otolith receptor function or vice versa, should show predictable alterations of body and head movement strategies essential for the control of postural sway and movement. The effect of altered postural movement control upon compensation and/or adaptation will be determined. These experiments provide data for the development of computational models of postural control in normals, vestibular deficient subjects and normal humans exposed to unusual force environments, including orbital space flight.

Keep Your Ear-Lids Open.

ERIC Educational Resources Information Center

Ferrington, Gary

1994-01-01

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

Listening to the ear

NASA Astrophysics Data System (ADS)

Shera, Christopher A.

Otoacoustic emissions demonstrate that the ear creates sound while listening to sound, offering a promising acoustic window on the mechanics of hearing in awake, listening human beings. That window is clouded, however, by an incomplete knowledge of wave reflection and transmission, both forth and back within the cochlea and through the middle ear. This thesis "does windows," addressing wave propagation and scattering on both sides of the middle ear. A summary of highlights follows. Measurements of the cochlear input impedance in cat are used to identify a new symmetry in cochlear mechanics-termed "tapering symmetry" after its geometric interpretation in simple models-that guarantees that the wavelength of the traveling wave changes slowly with position near the stapes. Waves therefore propagate without reflection through the basal turns of the cochlea. Analytic methods for solving the cochlear wave equations using a perturbative scattering series are given and used to demonstrate that, contrary to common belief, conventional cochlear models exhibit negligible internal reflection whether or not they accurately represent the tapering symmetries of the inner ear. Frameworks for the systematic "deconstruction" of eardrum and middle-ear transduction characteristics are developed and applied to the analysis of noninvasive measurements of middle-ear and cochlear mechanics. A simple phenomenological model of inner-ear compressibility that correctly predicts hearing thresholds in patients with missing or disarticulated middle-ear ossicles is developed and used to establish an upper bound on cochlear compressibility several orders of magnitude smaller than that provided by direct measurements. Accurate measurements of stimulus frequency evoked otoacoustic emissions are performed and used to determine the form and frequency variation of the cochlear traveling-wave ratio noninvasively. Those measurements are inverted to obtain the spatial distribution of mechanical inhomogeneities responsible for evoked emission. Although current models require that the periodicities found in emission spectra and threshold hearing curves originate in a corresponding corrugation in the mechanics of the cochlea, it is shown that the observed spectral periodicities can arise spontaneously through the dynamics of wave propagation and reflection and that the organ of Corti, as suggested by the anatomy, need manifest no particular translational symmetries.

Listening to the Ear

NASA Astrophysics Data System (ADS)

Shera, Christopher Alan

Otoacoustic emissions demonstrate that the ear creates sound while listening to sound, offering a promising acoustic window on the mechanics of hearing in awake, listening human beings. That window is clouded, however, by an incomplete knowledge of wave reflection and transmission, both forth and back within the cochlea and through the middle ear. This thesis "does windows," addressing wave propagation and scattering on both sides of the middle ear. A summary of highlights follows. Measurements of the cochlear input impedance in cat are used to identify a new symmetry in cochlear mechanics--termed "tapering symmetry" after its geometric interpretation in simple models--that guarantees that the wavelength of the traveling wave changes slowly with position near the stapes. Waves therefore propagate without reflection through the basal turns of the cochlea. Analytic methods for solving the cochlear wave equations using a perturbative scattering series are given and used to demonstrate that, contrary to common belief, conventional cochlear models exhibit negligible internal reflection whether or not they accurately represent the tapering symmetries of the inner ear. Frameworks for the systematic "deconstruction" of eardrum and middle-ear transduction characteristics are developed and applied to the analysis of noninvasive measurements of middle-ear and cochlear mechanics. A simple phenomenological model of inner-ear compressibility that correctly predicts hearing thresholds in patients with missing or disarticulated middle-ear ossicles is developed and used to establish an upper bound on cochlear compressibility several orders of magnitude smaller than that provided by direct measurements. Accurate measurements of stimulus -frequency evoked otoacoustic emissions are performed and used to determine the form and frequency variation of the cochlear traveling-wave ratio noninvasively. Those measurements are inverted to obtain the spatial distribution of mechanical inhomogeneities responsible for evoked emission. Although current models require that the periodicities found in emission spectra and threshold hearing curves originate in a corresponding corrugation in the mechanics of the cochlea, it is shown that the observed spectral periodicities can arise spontaneously through the dynamics of wave propagation and reflection and that the organ of Corti, as suggested by the anatomy, need manifest no particular translational symmetries.

Sox2 and Jagged1 Expression in Normal and Drug-Damaged Adult Mouse Inner Ear

PubMed Central

Campbell, Sean; Taylor, Ruth R.; Forge, Andrew; Hume, Clifford R.

2007-01-01

Inner ear hair cells detect environmental signals associated with hearing, balance, and body orientation. In humans and other mammals, significant hair cell loss leads to irreversible hearing and balance deficits, whereas hair cell loss in nonmammalian vertebrates is repaired by the spontaneous generation of replacement hair cells. Research in mammalian hair cell regeneration is hampered by the lack of in vivo damage models for the adult mouse inner ear and the paucity of cell-type-specific markers for non-sensory cells within the sensory receptor epithelia. The present study delineates a protocol to drug damage the adult mouse auditory epithelium (organ of Corti) in situ and uses this protocol to investigate Sox2 and Jagged1 expression in damaged inner ear sensory epithelia. In other tissues, the transcription factor Sox2 and a ligand member of the Notch signaling pathway, Jagged1, are involved in regenerative processes. Both are involved in early inner ear development and are expressed in developing support cells, but little is known about their expressions in the adult. We describe a nonsurgical technique for inducing hair cell damage in adult mouse organ of Corti by a single high-dose injection of the aminoglycoside kanamycin followed by a single injection of the loop diuretic furosemide. This drug combination causes the rapid death of outer hair cells throughout the cochlea. Using immunocytochemical techniques, Sox2 is shown to be expressed specifically in support cells in normal adult mouse inner ear and is not affected by drug damage. Sox2 is absent from auditory hair cells, but is expressed in a subset of vestibular hair cells. Double-labeling experiments with Sox2 and calbindin suggest Sox2-positive hair cells are Type II. Jagged1 is also expressed in support cells in the adult ear and is not affected by drug damage. Sox2 and Jagged1 may be involved in the maintenance of support cells in adult mouse inner ear. PMID:18157569

Active space of pheromone plume and its relationship to effective attraction radius in applied models.

PubMed

Byers, John A

2008-09-01

The release rate of a semiochemical lure that attracts flying insects has a specific effective attraction radius (EAR) that corresponds to the lure's orientation response strength. EAR is defined as the radius of a passive sphere that intercepts the same number of insects as a semiochemical-baited trap. It is estimated by calculating the ratio of trap catches in the field in baited and unbaited traps and the interception area of the unbaited trap. EAR serves as a standardized method for comparing the attractive strengths of lures that is independent of population density. In two-dimensional encounter rate models that are used to describe insect mass trapping and mating disruption, a circular EAR (EAR(c)) describes a key parameter that affects catch or influence by pheromone in the models. However, the spherical EAR, as measured in the field, should be transformed to an EAR(c) for appropriate predictions in such models. The EAR(c) is calculated as (pi/2EAR(2))/F (L), where F (L) is the effective thickness of the flight layer where the insect searches. F (L) was estimated from catches of insects (42 species in the orders Coleoptera, Lepidoptera, Diptera, Hemiptera, and Thysanoptera) on traps at various heights as reported in the literature. The EAR(c) was proposed further as a simple but equivalent alternative to simulations of highly complex active-space plumes with variable response surfaces that have proven exceedingly difficult to quantify in nature. This hypothesis was explored in simulations where flying insects, represented as coordinate points, moved about in a correlated random walk in an area that contained a pheromone plume, represented as a sector of active space composed of a capture probability surface of variable complexity. In this plume model, catch was monitored at a constant density of flying insects and then compared to simulations in which a circular EAR(c) was enlarged until an equivalent rate was caught. This demonstrated that there is a circular EAR(c), where all insects that enter are caught, which corresponds in catch effect to any plume. Thus, the EAR(c), based on the field-observed EAR, can be used in encounter rate models to develop effective control programs based on mass trapping and/or mating disruption.

Further development of LLNA:DAE method as stand-alone skin-sensitization testing method and applied for evaluation of relative skin-sensitizing potency between chemicals.

PubMed

Yamashita, Kunihiko; Shinoda, Shinsuke; Hagiwara, Saori; Itagaki, Hiroshi

2015-04-01

To date, there has been no well-established local lymph node assay (LLNA) that includes an elicitation phase. Therefore, we developed a modified local lymph node assay with an elicitation phase (LLNA:DAE) to discriminate true skin sensitizers from chemicals that gave borderline positive results and previously reported this assay. To develop the LLNA:DAE method as a useful stand-alone testing method, we investigated the complete procedure for the LLNA:DAE method using hexyl cinnamic aldehyde (HCA), isoeugenol, and 2,4-dinitrochlorobenzene (DNCB) as test compounds. We defined the LLNA:DAE procedure as follows: in the dose-finding test, four concentrations of chemical applied to dorsum of the right ear on days 1, 2, and 3 and dorsum of both ears on day 10. Ear thickness and skin irritation score were measured on days 1, 3, 5, 10, and 12. Local lymph nodes were excised and weighed on day 12. The test dose for the primary LLNA:DAE study was selected as the dose that gave the highest left ear lymph node weight in the dose-finding study, or the lowest dose that produced a left ear lymph node of over 4 mg. This procedure was validated using nine different chemicals. Furthermore, qualitative relationship was observed between the degree of elicitation response in the left ear lymph node and the skin sensitizing potency of 32 chemicals tested in this study and the previous study. These results indicated that LLNA:DAE method was as first LLNA method that was able to evaluate the skin sensitizing potential and potency in elicitation response.

Field screening of experimental corn hybrids and inbred lines for multiple ear-feeding insect resistance.

PubMed

Ni, Xinzhi; Xu, Wenwei; Krakowsky, Matthew D; Buntin, G David; Brown, Steve L; Lee, R Dewey; Coy, Anton E

2007-10-01

Identifying and using native insect resistance genes is the core of integrated pest management. In this study, 10 experimental corn, Zea mays L., hybrids and 10 inbred lines were screened for resistance to major ear-feeding insects in the southeastern Coastal Plain region of the United States during 2004 and 2005. Ear-feeding insect damage was assessed at harvest by visual damage rating for the corn earworm, Helicoverpa zea (Boddie), and by the percentage of kernels damaged by the maize weevil, Sitophilus zeamais Motschulsky, and stink bugs [combination of Euschistus servus (Say) and southern green stink bug, Nezara viridula (L.)]. Among the eight inbred lines and two control populations examined, C3S1B73-5b was resistant to corn earworm, maize weevil, and stink bugs. In contrast, C3S1B73-4 was resistant to corn earworm and stink bugs, but not to maize weevil. In a similar manner, the corn hybrid S1W*CML343 was resistant to all three ear-feeding insects, whereas hybrid C3S1B73-3*Tx205 was resistant to corn earworm and maize weevil in both growing seasons, but susceptible to stink bugs in 2005. The silk-feeding bioassay showed that corn earworm developed better on corn silk than did fall armyworm. Among all phenotypic traits examined (i.e., corn ear size, husk extension, and husk tightness), only corn ear size was negatively correlated to corn earworm damage in the inbred lines examined, whereas only husk extension (i.e., coverage) was negatively correlated to both corn earworm and maize weevil damage on the experimental hybrids examined. Such information could be used to establish a baseline for developing agronomically elite corn germplasm that confers multiple ear-feeding insect resistance.

Challenges and outcomes of cholesteatoma management in children with Down syndrome.

PubMed

Ghadersohi, Saied; Bhushan, Bharat; Billings, Kathleen R

2018-03-01

The high incidence of chronic otitis media with effusion and Eustachian tube dysfunction in children with Down syndrome (DS) may predispose them to cholesteatoma formation. Establishing the diagnosis, choosing the appropriate operative intervention, and post-operative care can be challenging. To describe management strategies for cholesteatoma diagnosis, surgical treatment, and post-operative management in children with Down syndrome. Retrospective case series of 14 patients (17 total ears) with Down syndrome diagnosed with cholesteatoma over a 9-year period. A total of 14 patients with cholesteatoma (3 with bilateral disease) were analyzed. Thirteen ears (76.5%) had ≥2 tympanostomy tubes insertions prior to cholesteatoma diagnosis, and otorrhea and hearing loss were the most common presenting symptoms. Common pre-operative CT scan findings included mastoid sclerosis and ossicular erosion. The average age at first surgery was 9.8 years, and the average follow-up was 4.3 years. For acquired cholesteatoma, most ears were managed with canal wall up (CWU) approaches, but ultimately 6/15 (40.0%) required canal wall down (CWD) approaches. Postoperatively, 3 (20.0%) ears developed new tympanic membrane retraction pockets, but no recurrent cholesteatoma. Four (26.7%) ears developed recurrent disease, and 3 (20.0%) had residual disease at secondary procedures. Ossiculoplasty was performed in 4 ears. Twelve (70.6%) ears were rehabilitated with hearing aids or FM systems. The diagnosis of cholesteatoma in Down syndrome was associated with otorrhea, hearing loss, and CT scan findings of ossicular erosion and mastoid sclerosis. Most cases were managed with CWU surgical approaches. Hearing aid use was common post-operatively. Copyright © 2018 Elsevier B.V. All rights reserved.

[History of the ear speculum. Images from the history of otorhinolaryngology, highlighted by instruments from the collection of the German Medical History Museum in Ingolstadt].

PubMed

Feldmann, H

1996-05-01

BACKGROUND. Inspection of or interventions in the external ear canal and the nostrils pose similar technical problems. This is the reason why early instruments devised for otoscopy and rhinoscopy were based on an identical principle. They were shaped like a pair of tongs, comparable to nasal specula of today. A similar type of instrument had been developed earlier by barber surgeons for inspecting narrow wound cavities. TONG-SHAPED SPECULA. The first description and illustration of an aural and nasal speculum was provided by Guy de Chauliac in Montpellier, France in 1363. Sophisticated models were presented by Fabricius Hildanus in Germany in 1646 and J.J. Perret in Paris in 1772, who offered them in his illustrated catalogue of surgical instruments at fixed prices. W. Kramer (1836) in Berlin improved this tong-shaped instrument and devised the ear speculum named after him that was generally favored during the first half of the 19th century. Further variations of this type were presented by Lincke and Schmalz (1846) in Germany. FUNNEL-SHAPED SPECULA. Ignaz Gruber in Vienna in 1838 devised the first tunnel-shaped ear specula made of metal. They had a simple conical shape, were not divided into separate jaws, and could not be spread. Gruber himself did not publish his invention, but he demonstrated his ear specula to W. R. Wilde from Dublin, who had paid a visit to his office in Vienna. Wilde reported on this in 1844, and subsequently systematically refined Gruber's specula. A. v. Tröltsch from Würzburg (Germany) had seen these instruments at Wilde's office and it was Wilde himself and v. Tröltsch who helped this type of ear speculum to gain acceptance on an international scale. A different type of bottle-shaped ear speculum was first used by Schmalz (1846) and Erhard (1859) in Germany, but it was only developed into a commercially available instrument by Josef Gruber in Vienna in 1870. The ear specula most in use today were first presented by A. Hartmann in Berlin in 1881. SUPPLEMENTARY INVENTIONS. An important supplementary invention was the pneumatic ear speculum by E. Siegle in Stuttgart (Germany) in 1864. It permitted not only inspecting the tympanic membrane but also examining its compliance and response to variations in air pressure in the ear canal. The importance of this instrument was recognized at once and has remained undisputed to this day. J. Bruton, an English military surgeon, presented his otoscope in 1862. It was the first device to incorporate interchangeable ear specula, illumination by a perforated mirror, and a magnifying lens into one handy instrument. It is the precursor of the modern diagnostic sets comprised of a battery-handle, various specula, and accessories for otoscopy, rhinoscopy, and ophthalmoscopy which started to come into use in the late twenties of this century. This historical development of the ear specula is described and illustrated in detail.

Finite element modelling of human auditory periphery including a feed-forward amplification of the cochlea.

PubMed

Wang, Xuelin; Wang, Liling; Zhou, Jianjun; Hu, Yujin

2014-08-01

A three-dimensional finite element model is developed for the simulation of the sound transmission through the human auditory periphery consisting of the external ear canal, middle ear and cochlea. The cochlea is modelled as a straight duct divided into two fluid-filled scalae by the basilar membrane (BM) having an orthotropic material property with dimensional variation along its length. In particular, an active feed-forward mechanism is added into the passive cochlear model to represent the activity of the outer hair cells (OHCs). An iterative procedure is proposed for calculating the nonlinear response resulting from the active cochlea in the frequency domain. Results on the middle-ear transfer function, BM steady-state frequency response and intracochlear pressure are derived. A good match of the model predictions with experimental data from the literatures demonstrates the validity of the ear model for simulating sound pressure gain of middle ear, frequency to place map, cochlear sensitivity and compressive output for large intensity input. The current model featuring an active cochlea is able to correlate directly the sound stimulus in the ear canal with the vibration of BM and provides a tool to explore the mechanisms by which sound pressure in the ear canal is converted to a stimulus for the OHCs.

Visual responses of corn silk flies (Diptera: Ulidiidae)

USDA-ARS?s Scientific Manuscript database

Corn silk flies are major pests impacting fresh market sweet corn production in Florida and Georgia. Control depends solely on well-times applications of insecticides to protect corn ear development. Surveillance depends on visual inspection of ears with no effective trapping methods currently ava...

Database Development for Ocean Impacts: Imaging, Outreach and Rapid Response

DTIC Science & Technology

2012-04-18

Edds-Walton, R R. Fay Specialisation for underwater hearing in the red-eared slider turtle, Trachemys scripta elegans Association for Research in...hearing in the red-eared slider turtle, Trachemys scripta elegans . International Society for Neuroethology 2010 Ketten, D. R., Arruda, ,J., S

Human Action Recognition Using Wireless Wearable In-Ear Microphone

NASA Astrophysics Data System (ADS)

Nishimura, Jun; Kuroda, Tadahiro

To realize the ubiquitous eating habits monitoring, we proposed the use of sounds sensed by an in-ear placed wireless wearable microphone. A prototype of wireless wearable in-ear microphone was developed by utilizing a common Bluetooth headset. We proposed a robust chewing action recognition algorithm which consists of two recognition stages: “chew-like” signal detection and chewing sound verification stages. We also provide empirical results on other action recognition using in-ear sound including swallowing, cough, belch, and etc. The average chewing number counting error rate of 1.93% is achieved. Lastly, chewing sound mapping is proposed as a new prototypical approach to provide an additional intuitive feedback on food groups to be able to infer the eating habits in their daily life context.

Overlapping and distinct pRb pathways in the mammalian auditory and vestibular organs

PubMed Central

Huang, Mingqian; Sage, Cyrille; Tang, Yong; Lee, Sang Goo; Petrillo, Marco; Hinds, Philip W

2011-01-01

Retinoblastoma gene (Rb1) is required for proper cell cycle exit in the developing mouse inner ear and its deletion in the embryo leads to proliferation of sensory progenitor cells that differentiate into hair cells and supporting cells. In a conditional hair cell Rb1 knockout mouse, Pou4f3-Cre-pRb™/™, pRb™/™ utricular hair cells differentiate and survive into adulthood whereas differentiation and survival of pRb™/™ cochlear hair cells are impaired. To comprehensively survey the pRb pathway in the mammalian inner ear, we performed microarray analysis of pRb™/™ cochlea and utricle. The comparative analysis shows that the core pathway shared between pRb™/™ cochlea and utricle is centered on e2F, the key pathway that mediates pRb function. A majority of differentially expressed genes and enriched pathways are not shared but uniquely associated with pRb™/™ cochlea or utricle. In pRb™/™ cochlea, pathways involved in early inner ear development such as Wnt/β-catenin and Notch were enriched, whereas pathways involved in proliferation and survival are enriched in pRb™/™ utricle. Clustering analysis showed that the pRb™/™ inner ear has characteristics of a younger control inner ear, an indication of delayed differentiation. We created a transgenic mouse model (ER-Cre-pRbflox/flox) in which Rb1 can be acutely deleted postnatally. Acute Rb1 deletion in the adult mouse fails to induce proliferation or cell death in inner ear, strongly indicating that Rb1 loss in these postmitotic tissues can be effectively compensated for, or that pRb-mediated changes in the postmitotic compartment result in events that are functionally irreversible once enacted. This study thus supports the concept that pRb-regulated pathways relevant to hair cell development, encompassing proliferation, differentiation and survival, act predominantly during early development. PMID:21239885

Development, Translation and Validation of Enhanced Asian Rome III Questionnaires for Diagnosis of Functional Bowel Diseases in Major Asian Languages: A Rome Foundation-Asian Neurogastroenterology and Motility Association Working Team Report

PubMed Central

Ghoshal, Uday C; Gwee, Kok-Ann; Chen, Minhu; Gong, Xiao R; Pratap, Nitesh; Hou, Xiaohua; Syam, Ari F; Abdullah, Murdani; Bak, Young-Tae; Choi, Myung-Gyu; Gonlachanvit, Sutep; Chua, Andrew S B; Chong, Kuck-Meng; Siah, Kewin T H; Lu, Ching-Liang; Xiong, Lishou; Whitehead, William E

2015-01-01

Background/Aims The development-processes by regional socio-cultural adaptation of an Enhanced Asian Rome III questionnaire (EAR3Q), a cultural adaptation of the Rome III diagnostic questionnaire (R3DQ), and its translation-validation in Asian languages are presented. As English is not the first language for most Asians, translation-validation of EAR3Q is essential. Hence, we aimed to culturally adapt the R3DQ to develop EAR3Q and linguistically validate it to show that the EAR3Q is able to allocate diagnosis according to Rome III criteria. Methods After EAR3Q was developed by Asian experts by consensus, it was translated into Chinese, Hindi-Telugu, Indonesian, Korean, and Thai, following Rome Foundation guidelines; these were then validated on native subjects (healthy [n = 60], and patients with irritable bowel syndrome [n = 59], functional dyspepsia [n = 53] and functional constipation [n = 61]) diagnosed by clinicians using Rome III criteria, negative alarm features and investigations. Results Experts noted words for constipation, bloating, fullness and heartburn, posed difficulty. The English back-translated questionnaires demonstrated concordance with the original EAR3Q. Sensitivity and specificity of the questionnaires were high enough to diagnose respective functional gastrointestinal disorders (gold standard: clinical diagnoses) in most except Korean and Indonesian languages. Questionnaires often uncovered overlapping functional gastrointestinal disorders. Test-retest agreement (kappa) values of the translated questionnaires were high (0.700–1.000) except in Korean (0.300–0.500) and Indonesian (0.100–0.400) languages at the initial and 2-week follow-up visit. Conclusions Though Chinese, Hindi and Telugu translations were performed well, Korean and Indonesian versions were not. Questionnaires often uncovered overlapping FGIDs, which were quite common. PMID:25537673

A compact structured light based otoscope for three dimensional imaging of the tympanic membrane

NASA Astrophysics Data System (ADS)

Das, Anshuman J.; Estrada, Julio C.; Ge, Zhifei; Dolcetti, Sara; Chen, Deborah; Raskar, Ramesh

2015-02-01

Three dimensional (3D) imaging of the tympanic membrane (TM) has been carried out using a traditional otoscope equipped with a high-definition webcam, a portable projector and a telecentric optical system. The device allows us to project fringe patterns on the TM and the magnified image is processed using phase shifting algorithms to arrive at a 3D description of the TM. Obtaining a 3D image of the TM can aid in the diagnosis of ear infections such as otitis media with effusion, which is essentially fluid build-up in the middle ear. The high resolution of this device makes it possible examine a computer generated 3D profile for abnormalities in the shape of the eardrum. This adds an additional dimension to the image that can be obtained from a traditional otoscope by allowing visualization of the TM from different perspectives. In this paper, we present the design and construction of this device and details of the imaging processing for recovering the 3D profile of the subject under test. The design of the otoscope is similar to that of the traditional device making it ergonomically compatible and easy to adopt in clinical practice.

Vestibular-Evoked Myogenic Potentials in Bilateral Vestibulopathy

PubMed Central

Rosengren, Sally M.; Welgampola, Miriam S.; Taylor, Rachael L.

2018-01-01

Bilateral vestibulopathy (BVP) is a chronic condition in which patients have a reduction or absence of vestibular function in both ears. BVP is characterized by bilateral reduction of horizontal canal responses; however, there is increasing evidence that otolith function can also be affected. Cervical and ocular vestibular-evoked myogenic potentials (cVEMPs/oVEMPs) are relatively new tests of otolith function that can be used to test the saccule and utricle of both ears independently. Studies to date show that cVEMPs and oVEMPs are often small or absent in BVP but are in the normal range in a significant proportion of patients. The variability in otolith function is partly due to the heterogeneous nature of BVP but is also due to false negative and positive responses that occur because of the large range of normal VEMP amplitudes. Due to their variability, VEMPs are not part of the diagnosis of BVP; however, they are helpful complementary tests that can provide information about the extent of disease within the labyrinth. This article is a review of the use of VEMPs in BVP, summarizing the available data on VEMP abnormalities in patients and discussing the limitations of VEMPs in diagnosing bilateral loss of otolith function. PMID:29719527

Empowering the deaf. Let the deaf be deaf

PubMed Central

Munoz-Baell, I.; Ruiz, M

2000-01-01

Deafness is often regarded as just a one and only phenomenon. Accordingly, deaf people are pictured as a unified body of people who share a single problem. From a medical point of view, we find it usual to work with a classification of deafness in which pathologies attributable to an inner ear disorder are segregated from pathologies attributable to an outer/middle ear disorder. Medical intervention is thus concerned more with the origin, degree, type of loss, onset, and structural pathology of deafness than with communicative disability and the implications there may be for the patient (mainly dependency, denial of abnormal hearing behaviour, low self esteem, rejection of the prosthetic help, and the breakdown of social relationships). In this paper, we argue that hearing loss is a very complex phenomenon, which has many and serious consequences for people and involves many factors and issues that should be carefully examined. The immediate consequence of deafness is a breakdown in communication whereby the communicative function needs to be either initiated or restored. In that sense, empowering strategies—aimed at promoting not only a more traditional psychological empowerment but also a community one—should primarily focus on the removal of communication barriers.


Keywords: deafness PMID:10692961

Imaging Characteristics of Children with Auditory Neuropathy Spectrum Disorder

PubMed Central

Roche, Joseph P.; Huang, Benjamin Y.; Castillo, Mauricio; Bassim, Marc K.; Adunka, Oliver F.; Buchman, Craig A.

2013-01-01

Objective To identify and define the imaging characteristics of children with auditory neuropathy spectrum disorder (ANSD). Design Retrospective medical records review and analysis of both temporal bone computed tomography (CT) and magnetic resonance images (MRI) in from children with the diagnosis of ANSD. Setting Tertiary referral center. Patients 118 children with the electrophysiological characteristics of ANSD with available imaging studies for review. Interventions Two neuroradiologists and a neurotologist reviewed each study and consensus descriptions were established. Main outcome measures The type and number of imaging findings were tabulated. Results Sixty-eight (64%) MRIs revealed at least one imaging abnormality while selective use of CT identified 23 (55%) with anomalies. The most prevalent MRI findings included cochlear nerve deficiency (n=51; 28% of 183 nerves), brain abnormalities (n=42; 40% of 106 brains) and prominent temporal horns (n=33, 16% of 212 temporal lobes). The most prevalent CT finding from selective use of CT was cochlear dysplasia (n=13; 31%). Conclusions MRI will identify many abnormalities in children with ANSD that are not readily discernable on CT. Specifically, both developmental and acquired abnormalities of the brain, posterior cranial fossa, and cochlear nerves are not uncommonly seen in this patient population. Inner ear anomalies are well delineated using either imaging modality. Since many of the central nervous system findings identified in this study using MRI can alter the treatment and prognosis for these children, we believe that MRI should be the initial imaging study of choice for children with ANSD. PMID:20593543

RADIATION MALFORMATIONS BELONGING TO THE CYCLOPIA-ARRHINENCEPHALIA- OTECEPHALIA GROUP IN THE MOUSE FETUS

DOE Office of Scientific and Technical Information (OSTI.GOV)

Marakami, U.; Kameyana, Y.; Majima, A.

1962-03-01

Pregnant mice on the 8.5th day of gestation were subjected to 200-r whole-body irradiation and the fetuses were examined on the l3th to l9th day of gestation. Incidence of fetal death (23.0%) was much higher than in the controls (8.2%1, and malformations of the nose, eyes, and ears cecurred in 32%. These malformations included alterations leading to a beaklike snout (19%), a univentricular telencephalon with cyclopia (6%), and forms transitional between these two (6%). These malformations were specific for irradiation on the 8.5th day of gestation; in previous studies they were not prominent when irradiation was carried out on themore » 8th, lOth, or llth day. During this most sensitive developmental stsge at 8.5 days, 3 to 5 brain vesicles are formed and the anterior neuropore is closed, which may account for the greater effect of teratogenic agents on the central nervous system at this time. Other abnormalities included exencephalia 12%), hydrocephalus (4%), microcephalia 10.7%), abnormally shaped head (5%), spinal cord anomalies (6%), eye anomalies (29%1, harelip and cleft palate (12%), tail abnormalities (6%), and thoraco- abdominal hernia (5%). (H.H.D.)« less

RADIATION MALFORMATIONS BELONGING TO THE CYCLOPIA-ARRHINECEPHALIA- OCTOCEPHALIA GROUP IN THE MOUSE FETUS

DOE Office of Scientific and Technical Information (OSTI.GOV)

Murakami, U.; Kameyama, Y.; Majima, A.

1962-03-01

Pregnant mice on the 8 1/2th day of gestation were subjected to 200 r whole-body irradiation and the fetuses were examined on the l3th to l9th day of gestation. Incidence of fetal death (23.0%) was much higher than in the controls (8.2%), and malformations of the nose, eyes, and ears occurred in 32%. These malformations included alterations leading to a beaklike snout (19%), a univentricular telencephalon with cyclopia (6%), and fomas transitional between these two (6%). These malformations were specific for irradiation on the 8 1/2th day of gestation. In previous studies they were not prominent when irradiation was carriedmore » out on the 8th, l0th, or llth day. During this most sensitive developmental stage at 8 1/2 days, 3 to 5 brain vesicles are formed and the anterior neuropore is closed, which may account for the greater effect of teratogenic agents on the central nervous system at this time. Other abnormalities included exencephalia (2%), hydrocephalus (4%), microcephalia (0.7%), abnormally shaped head (5%), spinal cord anomalies (6%), eye anomalies (29%), harelip and cleft palate (l2%), tail abnormalities (6%), and thoraco- abdominal hennia (5%). (H.H.D.)« less

Tcof1/Treacle is required for neural crest cell formation and proliferation deficiencies that cause craniofacial abnormalities.

PubMed

Dixon, Jill; Jones, Natalie C; Sandell, Lisa L; Jayasinghe, Sachintha M; Crane, Jennifer; Rey, Jean-Philippe; Dixon, Michael J; Trainor, Paul A

2006-09-05

Neural crest cells are a migratory cell population that give rise to the majority of the cartilage, bone, connective tissue, and sensory ganglia in the head. Abnormalities in the formation, proliferation, migration, and differentiation phases of the neural crest cell life cycle can lead to craniofacial malformations, which constitute one-third of all congenital birth defects. Treacher Collins syndrome (TCS) is characterized by hypoplasia of the facial bones, cleft palate, and middle and external ear defects. Although TCS results from autosomal dominant mutations of the gene TCOF1, the mechanistic origins of the abnormalities observed in this condition are unknown, and the function of Treacle, the protein encoded by TCOF1, remains poorly understood. To investigate the developmental basis of TCS we generated a mouse model through germ-line mutation of Tcof1. Haploinsufficiency of Tcof1 leads to a deficiency in migrating neural crest cells, which results in severe craniofacial malformations. We demonstrate that Tcof1/Treacle is required cell-autonomously for the formation and proliferation of neural crest cells. Tcof1/Treacle regulates proliferation by controlling the production of mature ribosomes. Therefore, Tcof1/Treacle is a unique spatiotemporal regulator of ribosome biogenesis, a deficiency that disrupts neural crest cell formation and proliferation, causing the hypoplasia characteristic of TCS craniofacial anomalies.

Intrapartum diagnostic of Roberts syndrome - case presentation.

PubMed

Socolov, Răzvan Vladimir; Andreescu, Nicoleta Ioana; Haliciu, Ana Maria; Gorduza, Eusebiu Vlad; Dumitrache, Florentin; Balan, Raluca Anca; Puiu, Maria; Dobrescu, Mihaela Amelia; Socolov, Demetra Gabriela

2015-01-01

Roberts syndrome is a rare disease, with multiple limb and skeletal abnormalities (called "pseudothalidomide disease"). There are only around 150 cases described in literature. We present a case of Roberts syndrome, diagnosed in moment of delivery, after a pregnancy without prenatal follow-up. The stillborn baby was naturally delivered by a 17-year-old primiparous woman at 38 weeks of amenorrhea. The pregnancy was not followed due to socioeconomic and family situation, and no prenatal ultrasound was performed. The male baby has 2650 g and presented several morphological abnormalities and tight double umbilical abdominal loop. The macroscopic evaluation showed: dolichocephaly, hypoplastic inferior maxilla with micrognathia, antimongoloid palpebral slant, pterygium colli, abnormal and lower implanted ears, superior limbs phocomelia, syndactyly at lower left limb and tetradactyly in all limbs, bilateral cryptorchidism, pancreatic aplasia. Roberts syndrome is a rare genetic disease with recessive autosomal transmission generated by mutations in ESCO2 gene, located on chromosome 8. The disease should be easy to diagnose by antenatal ultrasound examination, but in our case, the lack of prenatal follow-up determined the diagnostic at term. We believe consider this case is an argument towards introducing ultrasound-screening compulsory to all pregnancies. To identify a possible genetic mutation, further investigations of the parents are in progress, but classically the disease has a recessive autosomal transmission.

The Association Between Vestibular Physical Examination, Vertigo Questionnaires, and the Electronystagmography in Patients With Vestibular Symptoms.

PubMed

Gofrit, Shany G; Mayler, Yulia; Eliashar, Ron; Bdolah-Abram, Tali; Ilan, Ophir; Gross, Menachem

2017-04-01

Dizziness makes up a diagnostic and treatment challenge. The diagnostic accuracy of the medical history and vestibular physical examination in cases of vestibular symptoms is not clear. The aim of this study is to determine the association between vestibular physical examination, vestibular questionnaires, and electronystagmography (ENG) test in patients with vestibular symptoms. This is a prospective study of 135 adults with vestibular symptoms. The subjects underwent targeted physical examination and filled vestibular questionnaires, including the Dizziness Handicap Inventory (DHI), before ENG testing. The results of the physical examination and questionnaires were compared with the final ENG findings. Of patients who had normal ENG results, 32.1% (17/52) showed abnormal physical examination, and 48.8% (40/82) of the patient who had normal physical examination showed abnormal ENG results ( P = .46). Among patients with severe disability by DHI, 46.4% (13/28) had an abnormal ENG, and 42.9% (12/28) had a normal ENG ( P = .39). This study did not demonstrate association between vestibular physical examination, vestibular questionnaires, and ENG results. Although history (augmented by questionnaires) and physical examination are the initial steps in the evaluation of vertigo, the current study suggests that they should be complemented by objective testing for evaluation of inner ear origin of vertigo.

Comparison of Video Head Impulse Test and Caloric Reflex Test in advanced unilateral definite Menière's disease.

PubMed

Rubin, F; Simon, F; Verillaud, B; Herman, P; Kania, R; Hautefort, C

2018-06-01

There have been very few studies of the Video Head Impulse Test (VHIT) in patients with Menière's Disease (MD). Some reported 100% normal VHIT results, others not. These discrepancies may be due to differences in severity. The present study compared VHIT and caloric reflex test results in advanced unilateral definite MD. A prospective study included 37 consecutive patients, with a mean age of 56±12 years. Mean hearing loss was 59±18dB HL; 12 patients were subject to Tumarkin's otolithic crises. Abnormal caloric reflex was defined as ≥20% deficit, and abnormal VHIT as presence of saccades or <0.64 gain in vertical semicircular canals and <0.78 in horizontal canals. All patients had normal VHIT results, and 3 had normal caloric reflex; mean caloric reflex deficit was 45%. The present study is the only one to use the August 2015 updated definition of MD. The results showed that, outside of episodes of crisis, VHIT was normal during advanced unilateral definite MD, in contrast to abnormal caloric reflex. This feature could help distinguish MD from other inner ear diseases, and it would be interesting to try to confirm this hypothesis by studying MD patients. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

Generation and characterization of Atoh1-Cre knock-in mouse line

PubMed Central

Yang, Hua; Xie, Xiaoling; Deng, Min; Chen, Xiaowei; Gan, Lin

2010-01-01

Summary Atoh1 encodes a basic helix-loop-helix (bHLH) transcription factor required for the development of the inner ear sensory epithelia, the dorsal spinal cord, brainstem, cerebellum, and intestinal secretory cells. In this study to create a genetic tool for the research on gene function in the ear sensory organs, we generated an Atoh1-Cre knock-in mouse line by replacing the entire Atoh1 coding sequences with the Cre coding sequences. Atoh1Cre/+mice were viable, fertile, and displayed no visible defects whereas the Atoh1Cre/Cremice died perinatally. The spatiotemporal activities of Cre recombinase were examined by crossing Atoh1-Cre mice with the R26R-lacZ conditional reporter mice. Atoh1-Cre activities were detected in the developing inner ear, the hindbrain, the spinal cord, and the intestine. In the inner ear, Atoh1-Cre activities were confined to the sensory organs in which lacZ expression is detected in nearly all of the hair cells and in many supporting cells. Thus, Atoh1-Cre mouse line serves as a useful tool for the functional study of genes in the inner ear. In addition, our results demonstrate that Atoh1 is expressed in the common progenitors destined for both hair and supporting cells. PMID:20533400

Comparison between Scalp EEG and Behind-the-Ear EEG for Development of a Wearable Seizure Detection System for Patients with Focal Epilepsy

PubMed Central

Gu, Ying; Cleeren, Evy; Dan, Jonathan; Claes, Kasper; Hunyadi, Borbála

2017-01-01

A wearable electroencephalogram (EEG) device for continuous monitoring of patients suffering from epilepsy would provide valuable information for the management of the disease. Currently no EEG setup is small and unobtrusive enough to be used in daily life. Recording behind the ear could prove to be a solution to a wearable EEG setup. This article examines the feasibility of recording epileptic EEG from behind the ear. It is achieved by comparison with scalp EEG recordings. Traditional scalp EEG and behind-the-ear EEG were simultaneously acquired from 12 patients with temporal, parietal, or occipital lobe epilepsy. Behind-the-ear EEG consisted of cross-head channels and unilateral channels. The analysis on Electrooculography (EOG) artifacts resulting from eye blinking showed that EOG artifacts were absent on cross-head channels and had significantly small amplitudes on unilateral channels. Temporal waveform and frequency content during seizures from behind-the-ear EEG visually resembled that from scalp EEG. Further, coherence analysis confirmed that behind-the-ear EEG acquired meaningful epileptic discharges similarly to scalp EEG. Moreover, automatic seizure detection based on support vector machine (SVM) showed that comparable seizure detection performance can be achieved using these two recordings. With scalp EEG, detection had a median sensitivity of 100% and a false detection rate of 1.14 per hour, while, with behind-the-ear EEG, it had a median sensitivity of 94.5% and a false detection rate of 0.52 per hour. These findings demonstrate the feasibility of detecting seizures from EEG recordings behind the ear for patients with focal epilepsy. PMID:29295522

The transcriptional activator ZNF143 is essential for normal development in zebrafish

PubMed Central

2012-01-01

Background ZNF143 is a sequence-specific DNA-binding protein that stimulates transcription of both small RNA genes by RNA polymerase II or III, or protein-coding genes by RNA polymerase II, using separable activating domains. We describe phenotypic effects following knockdown of this protein in developing Danio rerio (zebrafish) embryos by injection of morpholino antisense oligonucleotides that target znf143 mRNA. Results The loss of function phenotype is pleiotropic and includes a broad array of abnormalities including defects in heart, blood, ear and midbrain hindbrain boundary. Defects are rescued by coinjection of synthetic mRNA encoding full-length ZNF143 protein, but not by protein lacking the amino-terminal activation domains. Accordingly, expression of several marker genes is affected following knockdown, including GATA-binding protein 1 (gata1), cardiac myosin light chain 2 (cmlc2) and paired box gene 2a (pax2a). The zebrafish pax2a gene proximal promoter contains two binding sites for ZNF143, and reporter gene transcription driven by this promoter in transfected cells is activated by this protein. Conclusions Normal development of zebrafish embryos requires ZNF143. Furthermore, the pax2a gene is probably one example of many protein-coding gene targets of ZNF143 during zebrafish development. PMID:22268977

The transcriptional activator ZNF143 is essential for normal development in zebrafish.

PubMed

Halbig, Kari M; Lekven, Arne C; Kunkel, Gary R

2012-01-23

ZNF143 is a sequence-specific DNA-binding protein that stimulates transcription of both small RNA genes by RNA polymerase II or III, or protein-coding genes by RNA polymerase II, using separable activating domains. We describe phenotypic effects following knockdown of this protein in developing Danio rerio (zebrafish) embryos by injection of morpholino antisense oligonucleotides that target znf143 mRNA. The loss of function phenotype is pleiotropic and includes a broad array of abnormalities including defects in heart, blood, ear and midbrain hindbrain boundary. Defects are rescued by coinjection of synthetic mRNA encoding full-length ZNF143 protein, but not by protein lacking the amino-terminal activation domains. Accordingly, expression of several marker genes is affected following knockdown, including GATA-binding protein 1 (gata1), cardiac myosin light chain 2 (cmlc2) and paired box gene 2a (pax2a). The zebrafish pax2a gene proximal promoter contains two binding sites for ZNF143, and reporter gene transcription driven by this promoter in transfected cells is activated by this protein. Normal development of zebrafish embryos requires ZNF143. Furthermore, the pax2a gene is probably one example of many protein-coding gene targets of ZNF143 during zebrafish development.

Future Approaches for Inner Ear Protection and Repair

ERIC Educational Resources Information Center

Shibata, Seiji B.; Raphael, Yehoash

2010-01-01

Health care professionals tending to patients with inner ear disease face inquiries about therapy options, including treatments that are being developed for future use but not yet available. The devastating outcome of sensorineural hearing loss, combined with the permanent nature of the symptoms, make these inquiries demanding and frequent. The…

THE TREATMENT OF AERO-OTITIS MEDIA BY REDECOMPRESSION,

DTIC Science & Technology

The precipitating event preceding the appearance of aero- otitis media is the development of a relative vacuum within the middle ear. The aim of...obtaining normal pressure relationships between the middle ear and the environment. In 27 of a group of 33 men with severe aero- otitis media , this

Interaction Between Allergy and Middle Ear Infection.

PubMed

Oh, Jeong-Hoon; Kim, Woo Jin

2016-09-01

Recent studies have attempted to identify interactions among the causes of otitis media with effusion (OME). This review discusses the interaction between allergy and infection with regard to host and environmental factors in terms of the development of OME. Protection of the upper airway against microbial invasion requires active interaction between the defense mechanisms of the respiratory epithelium, including innate and adaptive immunity, and mechanical factors. The impairment of these defenses due to allergy and/or increased bacterial resistance may lead to increased susceptibility to infectious organisms in the respiratory tract and middle ear mucosa. Recent genetic studies have provided valuable information about the association of Toll-like receptor signaling variations with clinical phenotypes and the risk of infection in the middle ear. Among the causal factors of OME, allergy not only induces an inflammatory reaction in the middle ear cavity but also facilitates the invasion of infectious pathogens. There is also evidence that allergy can affect the susceptibility of patients to infection of the upper respiratory tract, including the middle ear cavity.

Zebrafish models of human eye and inner ear diseases.

PubMed

Blanco-Sánchez, B; Clément, A; Phillips, J B; Westerfield, M

2017-01-01

Eye and inner ear diseases are the most common sensory impairments that greatly impact quality of life. Zebrafish have been intensively employed to understand the fundamental mechanisms underlying eye and inner ear development. The zebrafish visual and vestibulo-acoustic systems are very similar to these in humans, and although not yet mature, they are functional by 5days post-fertilization (dpf). In this chapter, we show how the zebrafish has significantly contributed to the field of biomedical research and how researchers, by establishing disease models and meticulously characterizing their phenotypes, have taken the first steps toward therapies. We review here models for (1) eye diseases, (2) ear diseases, and (3) syndromes affecting eye and/or ear. The use of new genome editing technologies and high-throughput screening systems should increase considerably the speed at which knowledge from zebrafish disease models is acquired, opening avenues for better diagnostics, treatments, and therapies. Copyright © 2017 Elsevier Inc. All rights reserved.

The pathogenesis of cauliflower ear. An experimental study in rabbits.

PubMed

Ohlsén, L; Skoog, T; Sohn, S A

1975-01-01

Appreciating an imcomplete understanding of the pathogenesis of cauliflower ear, an experimental study was designed to demonstrate the pathophysiology of this deformity. The investigation was conducted in 2-month-old rabbits. In one ear a collection of blood was placed under the raised perichondrium which was then sutured back in place and the skin closed. In the other ear an equal amount of blood was deposited between the intact perichondrium and skin. In the first study new cartilage developed under the perichondrium, but in the ear in which the blood was left above the surface of the perichondrium-covered cartilage, complete resorption of the clot occurred. The cauliflower ear was thus shown to be generating cartilage, arising from a layer of raised perichondrium which was further stimulated by a sero-sanguinous medium. The subperichondrial hematoma was extensively invaded by chondroblasts within 2 weeks, and over a period of 4 weeks the new tissue gradually changed into more mature cartilage. It was a consistent finding that the separated perichondrium retracted, thus causing the original cartilage to rise and buckle over the hamatoma, similar to the picture observed in the human pathology.

Current concepts in the pathogenesis and treatment of chronic suppurative otitis media

PubMed Central

Mittal, Rahul; Lisi, Christopher V.; Gerring, Robert; Mittal, Jeenu; Mathee, Kalai; Narasimhan, Giri; Azad, Rajeev K.; Yao, Qi; Grati, M'hamed; Yan, Denise; Eshraghi, Adrien A.; Angeli, Simon I.; Telischi, Fred F.

2015-01-01

Otitis media (OM) is an inflammation of the middle ear associated with infection. Despite appropriate therapy, acute OM (AOM) can progress to chronic suppurative OM (CSOM) associated with ear drum perforation and purulent discharge. The effusion prevents the middle ear ossicles from properly relaying sound vibrations from the ear drum to the oval window of the inner ear, causing conductive hearing loss. In addition, the inflammatory mediators generated during CSOM can penetrate into the inner ear through the round window. This can cause the loss of hair cells in the cochlea, leading to sensorineural hearing loss. Pseudomonas aeruginosa and Staphylococcus aureus are the most predominant pathogens that cause CSOM. Although the pathogenesis of AOM is well studied, very limited research is available in relation to CSOM. With the emergence of antibiotic resistance as well as the ototoxicity of antibiotics and the potential risks of surgery, there is an urgent need to develop effective therapeutic strategies against CSOM. This warrants understanding the role of host immunity in CSOM and how the bacteria evade these potent immune responses. Understanding the molecular mechanisms leading to CSOM will help in designing novel treatment modalities against the disease and hence preventing the hearing loss. PMID:26248613

A prediction of the minke whale (Balaenoptera acutorostrata) middle-ear transfer function.

PubMed

Tubelli, Andrew A; Zosuls, Aleks; Ketten, Darlene R; Yamato, Maya; Mountain, David C

2012-11-01

The lack of baleen whale (Cetacea Mysticeti) audiograms impedes the assessment of the impacts of anthropogenic noise on these animals. Estimates of audiograms, which are difficult to obtain behaviorally or electrophysiologically for baleen whales, can be made by simulating the audiogram as a series of components representing the outer, middle, and inner ear (Rosowski, 1991; Ruggero and Temchin, 2002). The middle-ear portion of the system can be represented by the middle-ear transfer function (METF), a measure of the transmission of acoustic energy from the external ear to the cochlea. An anatomically accurate finite element model of the minke whale (Balaenoptera acutorostrata) middle ear was developed to predict the METF for a mysticete species. The elastic moduli of the auditory ossicles were measured by using nanoindentation. Other mechanical properties were estimated from experimental stiffness measurements or from published values. The METF predicted a best frequency range between approximately 30 Hz and 7.5 kHz or between 100 Hz and 25 kHz depending on stimulation location. Parametric analysis found that the most sensitive parameters are the elastic moduli of the glove finger and joints and the Rayleigh damping stiffness coefficient β. The predicted hearing range matches well with the vocalization range.

Isolation of sphere-forming stem cells from the mouse inner ear.

PubMed

Oshima, Kazuo; Senn, Pascal; Heller, Stefan

2009-01-01

The mammalian inner ear has very limited ability to regenerate lost sensory hair cells. This deficiency becomes apparent when hair cell loss leads to hearing loss as a result of either ototoxic insult or the aging process. Coincidently, with this inability to regenerate lost hair cells, the adult cochlea does not appear to harbor cells with a proliferative capacity that could serve as progenitor cells for lost cells. In contrast, adult mammalian vestibular sensory epithelia display a limited ability for hair cell regeneration, and sphere-forming cells with stem cell features can be isolated from the adult murine vestibular system. The neonatal inner ear, however, does harbor sphere-forming stem cells residing in cochlear and vestibular tissues. Here, we provide protocols to isolate sphere-forming stem cells from neonatal vestibular and cochlear sensory epithelia as well as from the spiral ganglion. We further describe procedures for sphere propagation, cell differentiation, and characterization of inner ear cell types derived from spheres. Sphere-forming stem cells from the mouse inner ear are an important tool for the development of cellular replacement strategies of damaged inner ears and are a bona fide progenitor cell source for transplantation studies.

Topical ear drop self-medication practice among the Ear, Nose, and Throat patients in Ido Ekiti, Nigeria: A cross - sectional study.

PubMed

Olajide, Toye Gabriel; Aremu, Kayode Shuaib; Esan, Olaide T; Dosunmu, Adepeju Oluwatona; Raji, Mustapha Muhammad

2018-01-01

Self-medication is a common habit in our country; Nigeria, especially among patients with otorhinolaryngological disorders. Medication when taken wrongly may bring dire consequences to the individual, such as masking developing diseases and may cause many other undesirable effects. The aim of this study was to determine the prevalence and to analyze topical ear drop self-medication practices among respondents attending the Ear, Nose, and Throat Clinic of Federal Teaching Hospital Ido Ekiti, Nigeria. A 6-month hospital based cross-sectional study was conducted among patients who were seen in the Ear, Nose, and Throat facility of Federal Teaching Hospital, Ido Ekiti from July to December 2016 to determine topical ear drop self-medication practices. A pretested semi-structured questionnaire was used to obtained information from respondents. A total of 162 respondents out of 493 patients seen during the study had otological problems. Of which 107 (66%) respondents had engaged in self-medication with topical ear drops. Their ages ranged between 2 and 83 years with a mean age of 36.6 ± 19.1 years. There were 75 males and 87 females. The major reason for self-medication was that their ailments were minor in about 40.2% and the most common indication for self-medication was ear blockage with hearing impairment (33.6%). Pharmacy/chemist shops (42%) were major sources of information for those that self-medicated. Chloramphenicol and gentamycin were the major drugs that were used by the respondents. Majority of the respondents in this study practiced self-medication using different topical ear drops. Major source of information on the topical ear drops used was from pharmacy/chemist shops. There is a need for adequate public health education to create awareness among people on the danger of self-medication and to enact or enforce the law to reduce access to over the counter drugs. Healthcare should be made available and avoidable at primary health-care level.

Placental Histomorphology in a Case of Double Trisomy 48,XXX,+18.

PubMed

Shah, Sujal I; Dyer, Lisa; Stanek, Jerzy

2018-01-01

Approximately 50% of early spontaneous abortions are found to have chromosomal abnormalities. In these cases, certain histopathologic abnormalities are suggestive of, although not diagnostic for, the presence of chromosomal abnormalities. However, placental histomorphology in cases of complex chromosomal abnormalities, including double trisomies, is virtually unknown. We present the case of a 27-year-old G3P22002 female presenting at 19 weeks and 1 day of gestation by last menstrual period for scheduled prenatal visit. Ultrasound revealed a single fetus without heart tones and adequate amniotic fluid. Limited fetal measurements were consistent with estimated gestational age of 17 weeks. Labor was induced with misoprostol due to fetal demise. Autopsy revealed an immature female fetus with grade 1-2 maceration. The ears were low-set and posteriorly rotated. The fingers were short bilaterally, and the right foot showed absence of the second and third digits. Evaluation of the organs showed predominantly marked autolysis consistent with retained stillbirth. Placental examination revealed multiple findings, including focal pseudovillous papilliform trophoblastic proliferation of the undersurface of the chorionic plate and clustering of perpendicularly oriented sclerotic chorionic villi in the chorion laeve, which have not been previously reported in cases of chromosomal abnormalities. Karyotype of placental tissue revealed a 48,XXX,+18 karyotype and the same double trisomy of fetal thymic tissue by FISH. In addition to convoluted outlines of chorionic villi, villous trophoblastic pseudoinclusions, and clusters of villous cytotrophoblasts, the previously unreported focal pseudovillous papilliform trophoblastic proliferation of the undersurface of the chorionic plate and clustering of perpendicularly oriented sclerotic chorionic villi in the chorion laeve were observed in this double trisomy case. More cases have to be examined to show if the histology is specific for this double trisomy.

Placental Histomorphology in a Case of Double Trisomy 48,XXX,+18

PubMed Central

2018-01-01

Background Approximately 50% of early spontaneous abortions are found to have chromosomal abnormalities. In these cases, certain histopathologic abnormalities are suggestive of, although not diagnostic for, the presence of chromosomal abnormalities. However, placental histomorphology in cases of complex chromosomal abnormalities, including double trisomies, is virtually unknown. Case Report We present the case of a 27-year-old G3P22002 female presenting at 19 weeks and 1 day of gestation by last menstrual period for scheduled prenatal visit. Ultrasound revealed a single fetus without heart tones and adequate amniotic fluid. Limited fetal measurements were consistent with estimated gestational age of 17 weeks. Labor was induced with misoprostol due to fetal demise. Autopsy revealed an immature female fetus with grade 1-2 maceration. The ears were low-set and posteriorly rotated. The fingers were short bilaterally, and the right foot showed absence of the second and third digits. Evaluation of the organs showed predominantly marked autolysis consistent with retained stillbirth. Placental examination revealed multiple findings, including focal pseudovillous papilliform trophoblastic proliferation of the undersurface of the chorionic plate and clustering of perpendicularly oriented sclerotic chorionic villi in the chorion laeve, which have not been previously reported in cases of chromosomal abnormalities. Karyotype of placental tissue revealed a 48,XXX,+18 karyotype and the same double trisomy of fetal thymic tissue by FISH. Conclusion In addition to convoluted outlines of chorionic villi, villous trophoblastic pseudoinclusions, and clusters of villous cytotrophoblasts, the previously unreported focal pseudovillous papilliform trophoblastic proliferation of the undersurface of the chorionic plate and clustering of perpendicularly oriented sclerotic chorionic villi in the chorion laeve were observed in this double trisomy case. More cases have to be examined to show if the histology is specific for this double trisomy. PMID:29707399

What Can We Learn about Auditory Processing from Adult Hearing Questionnaires?

PubMed

Bamiou, Doris-Eva; Iliadou, Vasiliki Vivian; Zanchetta, Sthella; Spyridakou, Chrysa

2015-01-01

Questionnaires addressing auditory disability may identify and quantify specific symptoms in adult patients with listening difficulties. (1) To assess validity of the Speech, Spatial, and Qualities of Hearing Scale (SSQ), the (Modified) Amsterdam Inventory for Auditory Disability (mAIAD), and the Hyperacusis Questionnaire (HYP) in adult patients experiencing listening difficulties in the presence of a normal audiogram. (2) To examine which individual questionnaire items give the worse scores in clinical participants with an auditory processing disorder (APD). A prospective correlational analysis study. Clinical participants (N = 58) referred for assessment because of listening difficulties in the presence of normal audiometric thresholds to audiology/ear, nose, and throat or audiovestibular medicine clinics. Normal control participants (N = 30). The mAIAD, HYP, and the SSQ were administered to a clinical population of nonneurological adults who were referred for auditory processing (AP) assessment because of hearing complaints, in the presence of normal audiogram and cochlear function, and to a sample of age-matched normal-hearing controls, before the AP testing. Clinical participants with abnormal results in at least one ear and in at least two tests of AP (and at least one of these tests to be nonspeech) were classified as clinical APD (N = 39), and the remaining (16 of whom had a single test abnormality) as clinical non-APD (N = 19). The SSQ correlated strongly with the mAIAD and the HYP, and correlation was similar within the clinical group and the normal controls. All questionnaire total scores and subscores (except sound distinction of mAIAD) were significantly worse in the clinical APD versus the normal group, while questionnaire total scores and most subscores indicated greater listening difficulties for the clinical non-APD versus the normal subgroups. Overall, the clinical non-APD group tended to give better scores than the APD in all questionnaires administered. Correlation was strong for the worse-ear gaps-in-noise threshold with the SSQ, mAIAD, and HYP; strong to moderate for the speech in babble and left-ear dichotic digit test scores (at p < 0.01); and weak to moderate for the remaining AP tests except the frequency pattern test that did not correlate. The worse-scored items in all three questionnaires concerned speech-in-noise questions. This is similar to worse-scored items by hearing-impaired participants as reported in the literature. Worse-scored items of the clinical group also included quality aspects of listening questions from the SSQ, which most likely pertain to cognitive aspects of listening, such as ability to ignore other sounds and listening effort. Hearing questionnaires may help assess symptoms of adults with APD. The listening difficulties and needs of adults with APD to some extent overlap with those of hearing-impaired listeners, but there are significant differences. The correlation of the gaps-in-noise and duration pattern (but not frequency pattern) tests with the questionnaire scores indicates that temporal processing deficits may play an important role in clinical presentation. American Academy of Audiology.

New optical tomographic & topographic techniques for biomedical applications

NASA Astrophysics Data System (ADS)

Buytaert, Jan

The mammalian middle ear contains the eardrum and the three auditory ossicles, and forms an impedance match between sound in air and pressure waves in the fluid of the inner ear. Without this intermediate system, with its unsurpassed efficiency and dynamic range, we would be practically deaf. Physics-based modeling of this extremely complex mechanical system is necessary to help our basic understanding of the functioning of hearing. Highly realistic models will make it possible to predict the outcome of surgical interventions and to optimize design of ossicle prostheses and active middle ear implants. To obtain such models and with realistic output, basic input data is still missing. In this dissertation I developed and used two new optical techniques to obtain two essential sets of data: accurate three-dimensional morphology of the middle ear structures, and elasticity parameters of the eardrum. The first technique is a new method for optical tomography of macroscopic biomedical objects, which makes it possible to measure the three-dimensional geometry of the middle ear ossicles and soft tissues which are connecting and suspending them. I made a new and high-resolution version of this orthogonal-plane fluorescence optical sectioning method, to obtain micrometer resolution in macroscopic specimens. The result is thus a complete 3-D model of the middle (and inner) ear of gerbil in unprecedented quality. On top of high-resolution morphological models of the middle ear structures, I applied the technique in other fields of research as well. The second device works according to a new optical profilometry technique which allows to measure shape and deformations of the eardrum and other membranes or objects. The approach is called projection moire profilometry, and creates moire interference fringes which contain the height information. I developed a setup which uses liquid crystal panels for grid projection and optical demodulation. Hence no moving parts are present and the setup is entirely digitally controlled. This measurement method is developed to determine the elasticity parameters of the eardrum in-situ. Other surface shapes however can also be measured.

Association of Gel-Forming Mucins and Aquaporin Gene Expression With Hearing Loss, Effusion Viscosity, and Inflammation in Otitis Media With Effusion.

PubMed

Samuels, Tina L; Yan, Justin C; Khampang, Pawjai; Dettmar, Peter W; MacKinnon, Alexander; Hong, Wenzhou; Johnston, Nikki; Papsin, Blake C; Chun, Robert H; McCormick, Michael E; Kerschner, Joseph E

2017-08-01

Persistent, viscous middle ear effusion in pediatric otitis media (OM) contributes to increased likelihood of anesthesia and surgery, conductive hearing loss, and subsequent developmental delays. Biomarkers of effusion viscosity and hearing loss have not yet been identified despite the potential that such markers hold for targeted therapy and screening. To investigate the association of gel-forming mucins and aquaporin 5 (AQP5) gene expression with inflammation, effusion viscosity, and hearing loss in pediatric OM with effusion (OME). Case-control study of 31 pediatric patients (aged 6 months to 12 years) with OME undergoing tympanostomy tube placement and control individuals (aged 1 to 10 years) undergoing surgery for cochlear implantation from February 1, 2013, through November 30, 2014. Those with 1 or more episodes of OM in the previous 12 months, immunologic abnormality, anatomical or physiologic ear defect, OM-associated syndrome (ie, Down syndrome, cleft palate), chronic mastoiditis, or history of cholesteatoma were excluded from the study. All patients with OME and 1 control were recruited from Children's Hospital of Wisconsin, Milwaukee. The remainder of the controls were recruited from Sick Kids Hospital in Toronto, Ontario, Canada. Two to 3 middle ear biopsy specimens, effusions, and preoperative audiometric data (obtained <3 weeks before surgery) were collected from patients; only biopsy specimens were collected from controls. Expression of the mucin 2 (MUC2), mucin 5AC (MUC5AC), mucin 5B (MUC5B), and AQP5 genes were assayed in middle ear biopsy specimens by quantitative polymerase chain reaction. One middle ear biopsy specimen was sectioned for histopathologic analysis. Reduced specific viscosity of effusions was assayed using rheometry. Of the 31 study participants, 24 patients had OME (mean [SD] age, 50.4 [31.9] months; 15 [62.5%] male; 16 [66.7%] white) and 7 acted as controls (mean [SD] age, 32.6 [24.4] months; 2 [26.6%] male; 6 [85.7%] white). Mucins and AQP5 gene expression were significantly higher in patients with OME relative to controls (MUC2: ratio, 127.6 [95% CI, 33.7-482.7]; MUC5AC: ratio, 3748.8 [95% CI, 558.1-25 178.4]; MUC5B: ratio, 471.1 [95% CI, 130.7-1697.4]; AQP5: ratio, 2.4 [95% CI, 1.1-5.6]). A 2-fold increase in MUC5B correlated with increased hearing loss (air-bone gap: 7.45 dB [95% CI, 2.65-12.24 dB]; sound field: 6.66 dB [95% CI, 6.63-6.69 dB]), effusion viscosity (2.75 mL/mg; 95% CI, 0.89-4.62 mL/mg), middle ear epithelial thickness (3.5 μm; 95% CI, 1.96-5.13 μm), and neutrophil infiltration (odds ratio, 1.7; 95% CI, 1.07-2.72). A 2-fold increase in AQP5 correlated with increased effusion viscosity (1.94 mL/mg; 95% CI, 0.08-3.80 mL/mg). Further exploration of the role of MUC5B in the pathophysiology of OME holds promise for development of novel, targeted therapies to reduce effusion viscosity, facilitation of effusion clearance, and prevention of disease chronicity and hearing loss in patients with OME.

Effect of the Yield Stress and r-value Distribution on the Earing Profile of Cup Drawing with Yld2000-2d Yield Function

NASA Astrophysics Data System (ADS)

Lou, Yanshan; Bae, Gihyun; Lee, Changsoo; Huh, Hoon

2010-06-01

This paper deals with the effect of the yield stress and r-value distribution on the earing in the cup drawing. The anisotropic yield function, Yld2000-2d yield function, is selected to describe the anisotropy of two metal sheets, 719B and AA5182-O. The tool dimension is referred from the Benchmark problem of NUMISHEET'2002. The Downhill Simplex method is applied to identify the anisotropic coefficients in Yld2000-2d yield function. Simulations of the drawing process are performed to investigate the earing profile of two materials. The earing profiles obtained from simulations are compared with the analytical model developed by Hosford and Caddell. Simulations are conducted with respect to the change of the yield stress and r-value distribution, respectively. The correlation between the anisotropy and the earing tendency is investigated based on simulation data. Finally, the earing mechanism is analyzed through the deformation process of the blank during the cup deep drawing. It can be concluded that ears locate at angular positions with lower yield stress and higher r-value while the valleys appear at the angular position with higher yield stress and lower r-value. The effect of the yield stress distribution is more important for the cup height distribution than that of the r-value distribution.

[Atypical inflammation of the middle ear].

PubMed

Garov, E V; Kryukov, A I; Zelenkova, V N; Sidorina, N G; Kaloshina, A S

The objective of the present study was to characterize the patients presenting with atypical inflammation of the middle ear and consider the currently available methods for their examination. A total of 20 patients at the age from 16 to 66 years were admitted to the Department of Ear Microsurgery during the period from 2008 and 2016 for the treatment of atypical inflammation of the middle ear. Eleven of them (18 ears) were found to have tuberculous lesions (TL) of the middle ear while the remaining 9 patients (11 ears) suffered giant cell vasculitis (GCV). All the patients underwent the general clinical and otorhinolaryngological examination, computed tomography of the temporal bones and the thoracic cavity organs, cytological, bacteriological, pathomorphological, and molecular-genetic studies including PCR diagnostics, rheumatological tests, as well as counseling by a phthisiotherapist and rheumatologist. The primary localization of TL in the middle ear was documented in 6 patients including its association with lung lesions in 5 cases. The clinical picture of the disease in 5 patients was that of smoldering exudative pathology and in 6 ones was accompanied by suppurative perforative otitis media. According to the laboratory analyses, bacteriological diagnostics proved efficient in 9% of the patients, pathomorphological and cytological diagnostics in 18% and 27.3% of the cases respectively while the effectiveness of PCR diagnostics was estimated at 55%. The diagnosis in individual patients was established within the period from 1 month to 1.5 years after they first sought medical advice in connection with complaints of the ear disease. Tuberculosis of the middle ear began to develop as exudative middle otitis that acquired the form of bilateral pathology in 4 patients. Three patients had a concomitant pulmonary disease. In 4 patuents, the diagnois of middle ear tuberculosis was established based on the presence of the specific antibodies and in 5 ones based on the results of the pathomorphological study. All the studies were carried out in duplicate. The period between the beginning of the disease and the establishment of the definitive diagnosis varied from 1 month to 1 year.

Viability and virulence of pneumolysin, pneumococcal surface protein A, and pneumolysin/pneumococcal surface protein A mutants in the ear.

PubMed

Schachern, Patricia A; Tsuprun, Vladimir; Goetz, Sarah; Cureoglu, Sebahattin; Juhn, Steven K; Briles, David E; Paparella, Michael M; Ferrieri, Patricia

2013-09-01

Understanding how pneumococcal proteins affect the pathology of the middle ear and inner ear is important for the development of new approaches to prevent otitis media and its complications. To determine the viability and virulence of Streptococcus pneumoniae mutants deficient in pneumolysin (Ply-) and pneumococcal surface protein A (PspA-) in the chinchilla middle ear. Bullae of chinchillas were inoculated bilaterally with wild-type (Wt), Ply-, PspA-, and Ply-/PspA- strains. Bacterial colony-forming units (CFUs) in middle ear effusions were counted at 48 hours. The CFUs of the PspA- group were also counted at 6 to 36 hours after inoculation. Temporal bone histopathological results were compared. Twenty-seven chinchillas in an academic research laboratory. Chinchilla middle ears were inoculated with S pneumoniae to produce sufficient volumes of effusions and noticeable histopathological changes in the ears. The CFU counts in the middle ear effusions and histopathological changes were compared to determine the effect of pneumococcal protein mutations on chinchilla ears. At 48 hours, CFUs in middle ears were increased for the Wt and Ply-/PspA- strains, but Ply- remained near inoculum level. No bacteria were detected in the PspA- group. The CFUs of PspA- decreased over time to a low level at 30 to 36 hours. In vitro, PspA- in Todd-Hewitt broth showed an increase in bacterial growth of 2 logs at 43 hours, indicating PspA- susceptibility to host defenses in vivo. The PspA- and Ply- groups had fewer pathologic findings than the Wt or Ply-/PspA- groups. Histopathological analysis showed significant differences in the number of bacteria in the scala tympani in the Wt group compared with the Ply-, PspA-, and Ply-/PspA- groups. The PspA- strain was the least virulent. The PspA- mutant was much less viable and less virulent in the ear than the Wt, Ply-, and Ply-/PspA- strains. There was no significant attenuation in the viability and virulence of the Ply-/PspA- mutant compared with the Wt or single mutants. The viability and virulence of pneumococcal mutants seemed to be protein and organ specific.

Anatomical measurement of the ossicles in patients with congenital aural atresia and stenosis.

PubMed

Li, Jieying; Chen, Keguang; Li, Chenlong; Yin, Dongming; Zhang, Tianyu; Dai, Peidong

2017-10-01

Our aims were to measure and compare anatomical parameters of the ossicles in normal, congenital aural stenosis (CAS), and congenital aural atresia (CAA) ears. This retrospective study was performed using three-dimensional reconstructed images derived from computed tomography scans of 20 normal subjects, 20 CAS patients, and 20 CAA patients. The lengths of the malleus handle and long process of the incus were greater in normal ears than in CAS and CAA ears (all P < 0.05). The angles of the incudostapedial joint and between the short and long processes of the incus were smaller in normal ears than in CAS and CAA ears (all P < 0.05). There were no significant differences in the positions of the malleus head and incudomalleolar joint, the size of the malleus head, the length of the short process of the incus, or the angle of the incudomalleolar joint (P > 0.05). Anatomical parameters of the lower part, but not of the upper part, of the ossicular chain in CAS and CAA ears differed from those in normal ears. Different branchial arch origins of the upper and lower parts of the ossicular chain may explain these findings. Dysplasia of the second arch, which develops into the lower part of the ossicular chain, may contribute to ossicular malformation in CAA and CAS. Accurate radiographic measurement of malformed ossicles may be useful for reconstructive surgery of CAA and CAS using the patient's native ossicular chain and for choosing an appropriate place for active middle ear implants. Copyright © 2017. Published by Elsevier B.V.

A 3-D analysis of the protympanum in human temporal bones with chronic ear disease.

PubMed

Pauna, Henrique F; Monsanto, Rafael C; Schachern, Patricia; Paparella, Michael M; Cureoglu, Sebahattin

2017-03-01

Eustachian tube dysfunction is believed to be an important factor to cholesteatoma development and recurrence of disease after surgical treatment. Although many studies have described prognostic factors, evaluation methods, or surgical techniques for Eustachian tube dysfunction, they relied on the soft tissues of its structure; little is known about its bony structure-the protympanum-which connects the Eustachian tube to the tympanic cavity, and can also be affected by several inflammatory conditions, both from the middle ear or from the nasopharynx. We studied temporal bones from patients with cholesteatoma, chronic otitis media (with and without retraction pockets), purulent otitis media, and non-diseased ears, looking for differences between the volume of the protympanum, the diameter of the Eustachian tube isthmus, and the distance between the anterior tympanic annulus and the promontory. Light microscopy and 3-D reconstruction software were used for the measurements. We observed a decrease of volume in the lumen of the four middle ear diseased ears compared to the control group. We observed a significant decrease in the volume of the protympanic space in the cholesteatoma group compared to the chronic otitis media group. We also observed a decrease in the bony space (protympanum space) in cholesteatoma, chronic otitis media with retraction pockets, and purulent otitis media compared to the control group. We found a correlation in middle ear diseases and a decrease in the middle ear space. Our findings may suggest that a smaller bony volume in the protympanic area may trigger middle ear dysventilation problems.

Pressure transfer function and absorption cross section from the diffuse field to the human infant ear canal.

PubMed

Keefe, D H; Bulen, J C; Campbell, S L; Burns, E M

1994-01-01

The diffuse-field pressure transfer function from a reverberant field to the ear canal of human infants, ages 1, 3, 6, 12, and 24 months, has been measured from 125-10700 Hz. The source was a loudspeaker using pink noise, and the diffuse-field pressure and the ear-canal pressure were simultaneously measured using a spatial averaging technique in a reverberant room. The results in most subjects show a two-peak structure in the 2-6-kHz range, corresponding to the ear-canal and concha resonances. The ear-canal resonance frequency decreases from 4.4 kHz at age 1 month to 2.9 kHz at age 24 months. The concha resonance frequency decreases from 5.5 kHz at age 1 month to 4.5 kHz at age 24 months. Below 2 kHz, the diffuse-field transfer function shows effects due to the torsos of the infant and parent, and varies with how the infant is held. Comparisons are reported of the diffuse-field absorption cross section for infants relative to adults. This quantity is a measure of power absorbed by the middle ear from a diffuse sound field, and large differences are observed in infants relative to adults. The radiation efficiencies of the infant and the adult ear are small at low frequencies, near unity at midfrequencies, and decrease at higher frequencies. The process of ear-canal development is not yet complete at age 24 months. The results have implications for experiments on hearing in infants.

[2000-year history of the ear syringe and its relationship to the enema. Images from the history of otorhinolaryngology, represented by instruments from the collection of the Ingolstadt Medical History Museum].

PubMed

Feldmann, H

1999-08-01

SYRINGES WITH A PISTON AND THEIR USE AS "CLYSTER ORICULARIUS" IN THE ANCIENT ROME: Syringes with a piston were already known in ancient times and have been mentioned as a device for spraying rose water (Philon of Byzantium, about 230 BC), and for fighting fires (Heron of Alexandria about 110 AD). Celsus mentions it several times in his grand opus "De Medicina" (1st century AD) in the treatment of purulent discharge from the ear and for removing foreign bodies from the external ear canal. He always calls it "clyster oricularius", i.e. ear syringe, even when he describes its use in applications other than otological, such as in urology. Later the ear syringe fell from favor for a very long time and was reinvented only at the beginning of the 19th century. IRRIGATION OF THE BOWELS, FROM THE BAG SYRINGE TO THE PISTON SYRINGE: Irrigations of the bowels, clysters, were performed from ancient times to the 18th century with a baglike syringe using the bladder of animals as a pouch. Syringes with a piston were used by barbers and surgeons only for cleansing wounds or irrigating natural cavities of the body. Irrigation of the external ear canal had completely fallen from favor. In France in the 18th century large syringes with a piston made of tin oder brass came into use for enemas and replaced the old baglike devices. THE REINVENTION OF THE EAR SYRINGE AFTER THE PROTOTYPE OF THE ENEMA SYRINGE: Itard, an otologist in France in 1821, was the first to describe irrigating the ear with a syringe to remove hard wax. He recommended using an enema syringe ("seringue à lavement") for the procedure. Soon after his publication special ear syringes of appropriate size were developed and described by Beck in Freiburg, Germany, in 1827; by Fabrizi in Modena, Italy, 1839; and Schmalz in Dresden, Germany, 1846, who also introduced the kidney-shaped bowel for catching the water. Apart from that, small baglike syringes made of rubber were devised especially for use by the patient himself (Kramer, Berlin, 1860). This historical development is described in detail and illustrated by numerous figures.

Effectiveness of Ear Splint Therapy for Ear Deformities

PubMed Central

2017-01-01

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

Development and Current Status of the “Cambridge” Loudness Models

PubMed Central

2014-01-01

This article reviews the evolution of a series of models of loudness developed in Cambridge, UK. The first model, applicable to stationary sounds, was based on modifications of the model developed by Zwicker, including the introduction of a filter to allow for the effects of transfer of sound through the outer and middle ear prior to the calculation of an excitation pattern, and changes in the way that the excitation pattern was calculated. Later, modifications were introduced to the assumed middle-ear transfer function and to the way that specific loudness was calculated from excitation level. These modifications led to a finite calculated loudness at absolute threshold, which made it possible to predict accurately the absolute thresholds of broadband and narrowband sounds, based on the assumption that the absolute threshold corresponds to a fixed small loudness. The model was also modified to give predictions of partial loudness—the loudness of one sound in the presence of another. This allowed predictions of masked thresholds based on the assumption that the masked threshold corresponds to a fixed small partial loudness. Versions of the model for time-varying sounds were developed, which allowed prediction of the masked threshold of any sound in a background of any other sound. More recent extensions incorporate binaural processing to account for the summation of loudness across ears. In parallel, versions of the model for predicting loudness for hearing-impaired ears have been developed and have been applied to the development of methods for fitting multichannel compression hearing aids. PMID:25315375

An Examination of Construct Validity for the EARLI Numeracy Skill Measures

ERIC Educational Resources Information Center

Cheng, Weiyi; Lei, Pui-Wa; DiPerna, James C.

2017-01-01

The purpose of the current study was to examine dimensionality and concurrent validity evidence of the EARLI numeracy measures (DiPerna, Morgan, & Lei, 2007), which were developed to assess key skills such as number identification, counting, and basic arithmetic. Two methods (NOHARM with approximate chi-square test and DIMTEST with DETECT…

15 CFR 734.3 - Items subject to the EAR.

Code of Federal Regulations, 2011 CFR

2011-01-01

..., and foreign-made technology that is commingled with controlled U.S.-origin technology: (i) In any....S. origin technology or software, as described in § 736.2(b)(3) of the EAR. The term “direct product... technology or software; and Note to paragraph (a)(4): Certain foreign-manufactured items developed or...

15 CFR 734.3 - Items subject to the EAR.

Code of Federal Regulations, 2014 CFR

2014-01-01

..., and foreign-made technology that is commingled with controlled U.S.-origin technology: (i) In any....S. origin technology or software, as described in § 736.2(b)(3) of the EAR. The term “direct product... technology or software; and Note to paragraph (a)(4): Certain foreign-manufactured items developed or...

15 CFR 734.3 - Items subject to the EAR.

Code of Federal Regulations, 2013 CFR

2013-01-01

..., and foreign-made technology that is commingled with controlled U.S.-origin technology: (i) In any....S. origin technology or software, as described in § 736.2(b)(3) of the EAR. The term “direct product... technology or software; and Note to paragraph (a)(4): Certain foreign-manufactured items developed or...

15 CFR 734.3 - Items subject to the EAR.

Code of Federal Regulations, 2010 CFR

2010-01-01

..., and foreign-made technology that is commingled with controlled U.S.-origin technology: (i) In any....S. origin technology or software, as described in § 736.2(b)(3) of the EAR. The term “direct product... technology or software; and Note to paragraph (a)(4): Certain foreign-manufactured items developed or...

15 CFR 734.3 - Items subject to the EAR.

Code of Federal Regulations, 2012 CFR

2012-01-01

..., and foreign-made technology that is commingled with controlled U.S.-origin technology: (i) In any....S. origin technology or software, as described in § 736.2(b)(3) of the EAR. The term “direct product... technology or software; and Note to paragraph (a)(4): Certain foreign-manufactured items developed or...

Strategies for Coping with Educational and Social Consequences of Chronic Ear Infections in Rural Communities.

ERIC Educational Resources Information Center

Pillai, Patrick

2000-01-01

Children with chronic ear infections experience a lag time in understanding speech, which inhibits classroom participation and the ability to make friends, and ultimately reduces self-esteem. Difficulty in hearing affects speech and vocabulary development, reading and writing proficiency, and academic performance, and could lead to placement in…

Murine CMV-Induced Hearing Loss Is Associated with Inner Ear Inflammation and Loss of Spiral Ganglia Neurons

PubMed Central

Golemac, Mijo; Pugel, Ester Pernjak; Jonjic, Stipan; Britt, William J.

2015-01-01

Congenital human cytomegalovirus (HCMV) occurs in 0.5–1% of live births and approximately 10% of infected infants develop hearing loss. The mechanism(s) of hearing loss remain unknown. We developed a murine model of CMV induced hearing loss in which murine cytomegalovirus (MCMV) infection of newborn mice leads to hematogenous spread of virus to the inner ear, induction of inflammatory responses, and hearing loss. Characteristics of the hearing loss described in infants with congenital HCMV infection were observed including, delayed onset, progressive hearing loss, and unilateral hearing loss in this model and, these characteristics were viral inoculum dependent. Viral antigens were present in the inner ear as were CD3+ mononuclear cells in the spiral ganglion and stria vascularis. Spiral ganglion neuron density was decreased after infection, thus providing a mechanism for hearing loss. The lack of significant inner ear histopathology and persistence of inflammation in cochlea of mice with hearing loss raised the possibility that inflammation was a major component of the mechanism(s) of hearing loss in MCMV infected mice. PMID:25875183

Congenital aural atresia.

PubMed

Abdel-Aziz, Mosaad

2013-07-01

Congenital aural atresia is a spectrum of ear deformities present at birth that involves some degree of failure of the development of the external auditory canal. This malformation may be associated with other congenital anomalies; it occurs as a result of abnormal development of the first and second branchial arches and the first branchial cleft and most often occurs sporadically, although the disease may be manifested in different syndromes. Congenital aural atresia is considered one of the most difficult and challenging surgeries for the otologic surgeon. The goals of atresia surgery are to restore functional hearing, preferably without the requirement of a hearing aid, and to reconstruct a patent, infection-free external auditory canal. The repair is usually done at the age of 6 years, so children with bilateral atresia may need hearing amplification in the first few weeks of life until the age at surgery. To optimize the surgical outcome, careful audiological and radiological evaluation of the patient should be performed preoperatively. Also, postoperative frequent packing and regular follow-up are mandatory to avoid restenosis and infection of the newly created canal. With careful intraoperative dissection and regular follow-up, complications of surgery can be avoided.

Eustachian tube function in adults without middle ear disease.

PubMed

Swarts, J Douglas; Alper, Cuneyt M; Mandel, Ellen M; Villardo, Richard; Doyle, William J

2011-04-01

We sought to develop normative values for 5 eustachian tube function (ETF) test protocols in adults without otitis media (OM). Twenty adults (19 to 48 years of age) without a recent history of OM (5 had OM in childhood) underwent unilateral myringotomy and were evaluated for ETF by use of the forced response, inflation, deflation, forcible "sniff", and Valsalva test protocols. When possible, these tests were repeated on a second day. Normative values for the parameters of these protocols in adult subjects without a recent history of OM were developed. Between-day data for the forced response test were highly correlated. A percentage of these tests showed eustachian tube "constriction" during swallowing--an abnormal condition. The percent reduction in applied pressures for the inflation and deflation tests was high, indicative of good ETF. Few subjects had a positive "sniff" test, whereas most had a positive Valsalva test, and the results for both tests were effort-dependent. Results of ETF tests in adults with and without recent OM have not been published. Normative data are now available for comparison with ETF test results in adults with OM. These protocols will be used to evaluate the efficacy of surgical procedures designed to improve ETF.

Distinct cerebellar foliation anomalies in a CHD7 haploinsufficient mouse model of CHARGE syndrome

PubMed Central

Whittaker, Danielle E.; Kasah, Sahrunizam; Donovan, Alex P. A.; Ellegood, Jacob; Riegman, Kimberley L. H.; Volk, Holger A.; McGonnell, Imelda; Lerch, Jason P.

2017-01-01

Mutations in the gene encoding the ATP dependent chromatin‐remodeling factor, CHD7 are the major cause of CHARGE (Coloboma, Heart defects, Atresia of the choanae, Retarded growth and development, Genital‐urinary anomalies, and Ear defects) syndrome. Neurodevelopmental defects and a range of neurological signs have been identified in individuals with CHARGE syndrome, including developmental delay, lack of coordination, intellectual disability, and autistic traits. We previously identified cerebellar vermis hypoplasia and abnormal cerebellar foliation in individuals with CHARGE syndrome. Here, we report mild cerebellar hypoplasia and distinct cerebellar foliation anomalies in a Chd7 haploinsufficient mouse model. We describe specific alterations in the precise spatio‐temporal sequence of fissure formation during perinatal cerebellar development responsible for these foliation anomalies. The altered cerebellar foliation pattern in Chd7 haploinsufficient mice show some similarities to those reported in mice with altered Engrailed, Fgf8 or Zic1 gene expression and we propose that mutations or polymorphisms in these genes may modify the cerebellar phenotype in CHARGE syndrome. Our findings in a mouse model of CHARGE syndrome indicate that a careful analysis of cerebellar foliation may be warranted in patients with CHARGE syndrome, particularly in patients with cerebellar hypoplasia and developmental delay. PMID:29168327

Bilateral tympanokeratomas (cholesteatomas) with bilateral otitis media, unilateral otitis interna and acoustic neuritis in a dog.

PubMed

Østevik, Liv; Rudlang, Kathrine; Holt Jahr, Tuva; Valheim, Mette; Njaa, Bradley Lyndon

2018-05-22

An aural cholesteatoma, more appropriately named tympanokeratoma, is an epidermoid cyst of the middle ear described in several species, including dogs, humans and Mongolian gerbils. The cyst lining consists of stratified, keratinizing squamous epithelium with central accumulation of a keratin debris. This case report describes vestibular ganglioneuritis and perineuritis in a dog with chronic otitis, bilateral tympanokeratomas and presumed extension of otic infection to the central nervous system. An 11-year-old intact male Dalmatian dog with chronic bilateral otitis externa and sudden development of symptoms of vestibular disease was examined. Due to the dog's old age the owner opted for euthanasia without any further examination or treatment and the dog was submitted for necropsy. Transection of the ears revealed grey soft material in the external ear canals and pearly white, dry material consistent with keratin in the tympanic bullae bilaterally. The brain and meninges were grossly unremarkable. Microscopical findings included bilateral otitis externa and media, unilateral otitis interna, ganglioneuritis and perineuritis of the spiral ganglion of the vestibulocochlear nerve and multifocal to coalescing, purulent meningitis. A keratinizing squamous epithelial layer continuous with the external acoustic meatus lined the middle ear compartments, consistent with bilateral tympanokeratomas. Focal bony erosion of the petrous portion of the temporal bone and squamous epithelium and Gram-positive bacterial cocci were evident in the left cochlea. The findings suggest that meningitis developed secondary to erosion of the temporal bone and ganglioneuritis and/or perineuritis of the vestibulocochlear nerve. Middle ear tympanokeratoma is an important and potentially life-threatening otic condition in the dog. Once a tympanokeratoma has developed expansion of the cyst can lead to erosion of bone and extension of otic infection to the inner ear, vestibulocochlear ganglion and nerve potentially leading to bacterial infection of the central nervous system.

Probable Opitz trigonocephaly C syndrome with medulloblastoma

DOE Office of Scientific and Technical Information (OSTI.GOV)

Omran, H.; Hildebrandt, F.; Brandis, M.

1997-04-14

We report on a patient with trigonocephaly, biparietal widening as a result of metopic synostosis, strabismus, upslanted palpebral fissures, apparently low-set ears with abnormal helices, deeply furrowed palate, postaxial polysyndactyly of the feet, ankle flexion deformities, cryptorchidism, loose skin, and severe mental retardation, findings compatible with a diagnosis of the Opitz trigonocephaly C syndrome (OTS). At the age of 12 years this patient presented with symptoms of raised intracranial pressure. A biopsy showed findings diagnostic of a medulloblastoma WHO Grade IV, an unprecedented finding in OTS. The possibility of coincidence should not prevent continued surveillance of OTS patients in themore » future for the occurrence of malignancy. 33 refs., 4 figs., 1 tab.« less

Vascular defects and sensorineural deafness in a mouse model of Norrie disease.

PubMed

Rehm, Heidi L; Zhang, Duan-Sun; Brown, M Christian; Burgess, Barbara; Halpin, Chris; Berger, Wolfgang; Morton, Cynthia C; Corey, David P; Chen, Zheng-Yi

2002-06-01

Norrie disease is an X-linked recessive syndrome of blindness, deafness, and mental retardation. A knock-out mouse model with an Ndp gene disruption was studied. We examined the hearing phenotype, including audiological, histological, and vascular evaluations. As is seen in humans, the mice had progressive hearing loss leading to profound deafness. The primary lesion was localized to the stria vascularis, which houses the main vasculature of the cochlea. Fluorescent dyes showed an abnormal vasculature in this region and eventual loss of two-thirds of the vessels. We propose that one of the principal functions of norrin in the ear is to regulate the interaction of the cochlea with its vasculature.

Classification of Newborn Ear Malformations and their Treatment with the EarWell Infant Ear Correction System.

PubMed

Daniali, Lily N; Rezzadeh, Kameron; Shell, Cheryl; Trovato, Matthew; Ha, Richard; Byrd, H Steve

2017-03-01

A single practice's treatment protocol and outcomes following molding therapy on newborn ear deformations and malformations with the EarWell Infant Ear Correction System were reviewed. A classification system for grading the severity of constricted ear malformations was created on the basis of anatomical findings. A retrospective chart/photograph review of a consecutive series of infants treated with the EarWell System from 2011 to 2014 was undertaken. The infants were placed in either deformation or malformation groups. Three classes of malformation were identified. Data regarding treatment induction, duration of treatment, and quality of outcome were collected for all study patients. One hundred seventy-five infant ear malformations and 303 infant ear deformities were treated with the EarWell System. The average age at initiation of treatment was 12 days; the mean duration of treatment was 37 days. An average of six office visits was required. Treated malformations included constricted ears [172 ears (98 percent)] and cryptotia [three ears (2 percent)]. Cup ear (34 ears) was considered a constricted malformation, in contrast to the prominent ear deformity. Constricted ears were assigned to one of three classes, with each subsequent class indicating increasing severity: class I, 77 ears (45 percent); class II, 81 ears (47 percent); and class III, 14 ears (8 percent). Molding therapy with the EarWell System reduced the severity by an average of 1.2 points (p < 0.01). Complications included minor superficial excoriations and abrasions. The EarWell System was shown to be effective in eliminating or reducing the need for surgery in all but the most severe malformations. Therapeutic, IV.

Acute Otitis Media in Children.

PubMed

Leung, Alexander K C; Wong, Alex H C

2017-01-01

Acute otitis media is a common childhood infection. Prompt diagnosis and appropriate treatment are very important. To review in depth the epidemiology, pathophysiology, clinical manifestations, diagnosis, complications and particularly treatment of acute otitis media in children. A PubMed search was completed in Clinical Queries using the key term "acute otitis media". Patents were searched using the key term "acute otitis media" from www.google.com/patents, http://espacenet.com, and www.freepatentsonline.com. Acute otitis media affects over 80% of children before their third birthday and 30 to 45% of these children have suffered two or more episodes. Streptococcus pneumoniae, nontypable Haemophilus influenzae, and Moraxella catarrhalis are the most frequently isolated middle-ear pathogens. The diagnosis is based on acute onset of symptoms such as otalgia and fever, middle ear inflammation such as erythema of the tympanic membrane, and middle ear effusion. The choice of treatment method depends on the age of the child, laterality, and the severity of the disease. Recent patents related to the management of acute otitis media are also retrieved and discussed. Antimicrobial treatment is recommended for all children less than two years of age, as well as in children ≥ two years of age who have a temperature ≥ 39oC; are toxic looking; have otalgia > 48 hours; have bilateral otitis media or otorrhea; have craniofacial abnormalities; are immunocompromised; or have uncertain access to follow-up. Amoxicillin is the drug of choice. Observation without antibiotic is an option in immunocompetent children ≥ two years of age who have an acute uncomplicated otitis media and non-severe illness if appropriate follow-up can be arranged. Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.org.

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

PubMed Central

Wang, Xuelin; Gan, Rong Z.

2016-01-01

Chinchilla is a commonly used animal model for research of sound transmission through the ear. Experimental measurements of the middle ear transfer function in chinchillas have shown that the middle ear cavity greatly affects the tympanic membrane (TM) and stapes footplate (FP) displacements. However, there is no finite element (FE) model of the chinchilla ear available in the literature to characterize the middle ear functions with the anatomical features of the chinchilla ear. This paper reports a recently completed 3D FE model of the chinchilla ear based on X-ray micro-computed tomography images of a chinchilla bulla. The model consisted of the ear canal, TM, middle ear ossicles and suspensory ligaments, and the middle ear cavity. Two boundary conditions of the middle ear cavity wall were simulated in the model as the rigid structure and the partially flexible surface, and the acoustic-mechanical coupled analysis was conducted with these two conditions to characterize the middle ear function. The model results were compared with experimental measurements reported in the literature including the TM and FP displacements and the middle ear input admittance in chinchilla ear. An application of this model was presented to identify the acoustic role of the middle ear septa - a unique feature of chinchilla middle ear cavity. This study provides the first 3D FE model of the chinchilla ear for characterizing the middle ear functions through the acoustic-mechanical coupled FE analysis. PMID:26785845

Broadened population-level frequency tuning in the auditory cortex of tinnitus patients.

PubMed

Sekiya, Kenichi; Takahashi, Mariko; Murakami, Shingo; Kakigi, Ryusuke; Okamoto, Hidehiko

2017-03-01

Tinnitus is a phantom auditory perception without an external sound source and is one of the most common public health concerns that impair the quality of life of many individuals. However, its neural mechanisms remain unclear. We herein examined population-level frequency tuning in the auditory cortex of unilateral tinnitus patients with similar hearing levels in both ears using magnetoencephalography. We compared auditory-evoked neural activities elicited by a stimulation to the tinnitus and nontinnitus ears. Objective magnetoencephalographic data suggested that population-level frequency tuning corresponding to the tinnitus ear was significantly broader than that corresponding to the nontinnitus ear in the human auditory cortex. The results obtained support the hypothesis that pathological alterations in inhibitory neural networks play an important role in the perception of subjective tinnitus. NEW & NOTEWORTHY Although subjective tinnitus is one of the most common public health concerns that impair the quality of life of many individuals, no standard treatment or objective diagnostic method currently exists. We herein revealed that population-level frequency tuning was significantly broader in the tinnitus ear than in the nontinnitus ear. The results of the present study provide an insight into the development of an objective diagnostic method for subjective tinnitus. Copyright © 2017 the American Physiological Society.

Simulations and Measurements of Human Middle Ear Vibrations Using Multi-Body Systems and Laser-Doppler Vibrometry with the Floating Mass Transducer.

PubMed

Böhnke, Frank; Bretan, Theodor; Lehner, Stefan; Strenger, Tobias

2013-10-22

The transfer characteristic of the human middle ear with an applied middle ear implant (floating mass transducer) is examined computationally with a Multi-body System approach and compared with experimental results. For this purpose, the geometry of the middle ear was reconstructed from μ-computer tomography slice data and prepared for a Multi-body System simulation. The transfer function of the floating mass transducer, which is the ratio of the input voltage and the generated force, is derived based on a physical context. The numerical results obtained with the Multi-body System approach are compared with experimental results by Laser Doppler measurements of the stapes footplate velocities of five different specimens. Although slightly differing anatomical structures were used for the calculation and the measurement, a high correspondence with respect to the course of stapes footplate displacement along the frequency was found. Notably, a notch at frequencies just below 1 kHz occurred. Additionally, phase courses of stapes footplate displacements were determined computationally if possible and compared with experimental results. The examinations were undertaken to quantify stapes footplate displacements in the clinical practice of middle ear implants and, also, to develop fitting strategies on a physical basis for hearing impaired patients aided with middle ear implants.

A prediction of the minke whale (Balaenoptera acutorostrata) middle-ear transfer functiona)

PubMed Central

Tubelli, Andrew A.; Zosuls, Aleks; Ketten, Darlene R.; Yamato, Maya; Mountain, David C.

2012-01-01

The lack of baleen whale (Cetacea Mysticeti) audiograms impedes the assessment of the impacts of anthropogenic noise on these animals. Estimates of audiograms, which are difficult to obtain behaviorally or electrophysiologically for baleen whales, can be made by simulating the audiogram as a series of components representing the outer, middle, and inner ear (Rosowski, 1991; Ruggero and Temchin, 2002). The middle-ear portion of the system can be represented by the middle-ear transfer function (METF), a measure of the transmission of acoustic energy from the external ear to the cochlea. An anatomically accurate finite element model of the minke whale (Balaenoptera acutorostrata) middle ear was developed to predict the METF for a mysticete species. The elastic moduli of the auditory ossicles were measured by using nanoindentation. Other mechanical properties were estimated from experimental stiffness measurements or from published values. The METF predicted a best frequency range between approximately 30 Hz and 7.5 kHz or between 100 Hz and 25 kHz depending on stimulation location. Parametric analysis found that the most sensitive parameters are the elastic moduli of the glove finger and joints and the Rayleigh damping stiffness coefficient β. The predicted hearing range matches well with the vocalization range. PMID:23145610

A novel vehicle for local protein delivery to the inner ear: injectable and biodegradable thermosensitive hydrogel loaded with PLGA nanoparticles.

PubMed

Dai, Juan; Long, Wei; Liang, Zhongping; Wen, Lu; Yang, Fan; Chen, Gang

2018-01-01

Delivery of biomacromolecular drugs into the inner ear is challenging, mainly because of their inherent instability as well as physiological and anatomical barriers. Therefore, protein-friendly, hydrogel-based delivery systems following local administration are being developed for inner ear therapy. Herein, biodegradable poly(lactic-co-glycolic acid) (PLGA) nanoparticles (NPs) containing interferon α-2 b (IFN α-2 b) were loaded in chitosan/glycerophosphate (CS/GP)-based thermosensitive hydrogel for IFN delivery by intratympanic injection. The injectable hydrogel possessed a physiological pH and formed semi-solid gel at 37 °C, with good swelling and deswelling properties. The CS/GP hydrogel could slowly degrade as visualized by scanning electron microscopy (SEM). The presence of NPs in CS/GP gel largely influenced in vitro drug release. In the guinea pig cochlea, a 1.5- to 3-fold increase in the drug exposure time of NPs-CS/GP was found than those of the solution, NPs and IFN-loaded hydrogel. Most importantly, a prolonged residence time was attained without obvious histological changes in the inner ear. This biodegradable, injectable, and thermosensitive NPs-CS/GP system may allow longer delivery of protein drugs to the inner ear, thus may be a potential novel vehicle for inner ear therapy.

Ear keloids as a primary candidate for the application of mitomycin C after shave excision: in vivo and in vitro study.

PubMed

Chi, Seong Geun; Kim, Jun Young; Lee, Weon Ju; Lee, Seok-Jong; Kim, Do Won; Sohn, Mi Yeung; Kim, Gun Wook; Kim, Moon Bum; Kim, Byung Soo

2011-02-01

Although many methods have been developed to treat ear keloids, new therapeutic options are still needed. To determine the effects of topical mitomycin C (MC) on shave-removed wounds and fibroblasts of ear keloids. Fourteen ear keloids in 12 patients were shaved, and MC (1 mg/mL) was applied to the resected bed for 5 minutes. The application was repeated 3 weeks later. All patients were assessed 2, 4, and 6 months after the procedure to evaluate the cosmetic results, recurrence, and postsurgical complications. An in vitro study to determine the effects of MC on fibroblasts of the excised keloids was conducted using the 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide (MTT) assay, the measurement of total cell counts, and immunoassay of DNA synthesis. Only one recurrence occurred (on the ear helix), and the patients were satisfied with the cosmetic outcomes. The results of the MTT assay, total cell counts, and DNA synthesis immunoassay confirmed the suppressive effects of MC on the keloid fibroblasts. The application of topical MC to the resected bed of shave-removed ear keloids was successful in preventing recurrences and providing an acceptable cosmetic outcome. © 2011 by the American Society for Dermatologic Surgery, Inc.

Increased susceptibility to otitis media in a Splunc1-deficient mouse model

PubMed Central

Bartlett, Jennifer A.; Meyerholz, David K.; Wohlford-Lenane, Christine L.; Naumann, Paul W.; Salzman, Nita H.; McCray, Paul B.

2015-01-01

ABSTRACT Otitis media (inflammation of the middle ear) is one of the most common diseases of early childhood. Susceptibility to otitis is influenced by a number of factors, including the actions of innate immune molecules secreted by the epithelia lining the nasopharynx, middle ear and Eustachian tube. The SPLUNC1 (short palate, lung, nasal epithelial clone 1) protein is a highly abundant secretory product of the mammalian nasal, oral and respiratory mucosa that is thought to play a multifunctional role in host defense. In this study we investigated Splunc1 expression in the ear of the mouse, and examined whether this protein contributes to overall host defense in the middle ear and/or Eustachian tube. We found that Splunc1 is highly expressed in both the surface epithelium and in submucosal glands in these regions in wild-type mice. In mice lacking Splunc1, we noted histologically an increased frequency of otitis media, characterized by the accumulation of leukocytes (neutrophils with scattered macrophages), proteinaceous fluid and mucus in the middle ear lumens. Furthermore, many of these mice had extensive remodeling of the middle ear wall, suggesting a chronic course of disease. From these observations, we conclude that loss of Splunc1 predisposes mice to the development of otitis media. The Splunc1−/− mouse model should help investigators to better understand both the biological role of Splunc1 as well as host defense mechanisms in the middle ear. PMID:25765466

From labyrinthine aplasia to otocyst deformity.

PubMed

Giesemann, Anja Maria; Goetz, Friedrich; Neuburger, Jürgen; Lenarz, Thomas; Lanfermann, Heinrich

2010-02-01

Inner ear malformations (IEMs) are rare and it is unusual to encounter the rarest of them, namely labyrinthine aplasia (LA) and otocyst deformity. They do, however, provide useful pointers as to the early embryonic development of the ear. LA is characterised as a complete absence of inner ear structures. While some common findings do emerge, a clear definition of the otocyst deformity does not exist. It is often confused with the common cavity first described by Edward Cock. Our purpose was to radiologically characterise LA and otocyst deformity. Retrospective analysis of CT and MRI data from four patients with LA or otocyst deformity. Middle and inner ear findings were categorised by two neuroradiologists. The bony carotid canal was found to be absent in all patients. Posterior located cystic structures were found in association with LA and otocyst deformity. In the most severe cases, only soft tissue was present at the medial border of the middle ear cavity. The individuals with otocyst deformity also had hypoplasia of the petrous apex bone. These cases demonstrate gradual changes in the two most severe IEMs. Clarification of terms was necessary and, based on these findings, we propose defining otocyst deformity as a cystic structure in place of the inner ear, with the cochlea, IAC and carotid canal absent. This condition needs to be differentiated from the common cavity described by Edward Cook. A clear definition of inner ear malformations is essential if outcomes following cochlear implantation are to be compared.

Brief report: reconstruction of joint hyaline cartilage by autologous progenitor cells derived from ear elastic cartilage.

PubMed

Mizuno, Mitsuru; Kobayashi, Shinji; Takebe, Takanori; Kan, Hiroomi; Yabuki, Yuichiro; Matsuzaki, Takahisa; Yoshikawa, Hiroshi Y; Nakabayashi, Seiichiro; Ik, Lee Jeong; Maegawa, Jiro; Taniguchi, Hideki

2014-03-01

In healthy joints, hyaline cartilage covering the joint surfaces of bones provides cushioning due to its unique mechanical properties. However, because of its limited regenerative capacity, age- and sports-related injuries to this tissue may lead to degenerative arthropathies, prompting researchers to investigate a variety of cell sources. We recently succeeded in isolating human cartilage progenitor cells from ear elastic cartilage. Human cartilage progenitor cells have high chondrogenic and proliferative potential to form elastic cartilage with long-term tissue maintenance. However, it is unknown whether ear-derived cartilage progenitor cells can be used to reconstruct hyaline cartilage, which has different mechanical and histological properties from elastic cartilage. In our efforts to develop foundational technologies for joint hyaline cartilage repair and reconstruction, we conducted this study to obtain an answer to this question. We created an experimental canine model of knee joint cartilage damage, transplanted ear-derived autologous cartilage progenitor cells. The reconstructed cartilage was rich in proteoglycans and showed unique histological characteristics similar to joint hyaline cartilage. In addition, mechanical properties of the reconstructed tissues were higher than those of ear cartilage and equal to those of joint hyaline cartilage. This study suggested that joint hyaline cartilage was reconstructed from ear-derived cartilage progenitor cells. It also demonstrated that ear-derived cartilage progenitor cells, which can be harvested by a minimally invasive method, would be useful for reconstructing joint hyaline cartilage in patients with degenerative arthropathies. © AlphaMed Press.

Drosophila model of Meier-Gorlin syndrome based on the mutation in a conserved C-Terminal domain of Orc6.

PubMed

Balasov, Maxim; Akhmetova, Katarina; Chesnokov, Igor

2015-11-01

Meier-Gorlin syndrome (MGS) is an autosomal recessive disorder characterized by microtia, primordial dwarfism, small ears, and skeletal abnormalities. Patients with MGS often carry mutations in the genes encoding the components of the pre-replicative complex such as Origin Recognition Complex (ORC) subunits Orc1, Orc4, Orc6, and helicase loaders Cdt1 and Cdc6. Orc6 is an important component of ORC and has functions in both DNA replication and cytokinesis. Mutation in conserved C-terminal motif of Orc6 associated with MGS impedes the interaction of Orc6 with core ORC. In order to study the effects of MGS mutation in an animal model system we introduced MGS mutation in Orc6 and established Drosophila model of MGS. Mutant flies die at third instar larval stage with abnormal chromosomes and DNA replication defects. The lethality can be rescued by elevated expression of mutant Orc6 protein. Rescued MGS flies are unable to fly and display multiple planar cell polarity defects. © 2015 Wiley Periodicals, Inc. © 2015 Wiley Periodicals, Inc.

Vascular loop in the cerebellopontine angle causing pulsatile tinnitus and headache: a case report

PubMed Central

Ramly, NA; Roslenda, AR; Suraya, A; Asma, A

2014-01-01

Tinnitus is a common disorder, it can be classified as pulsatile and non-pulsatile or objective and subjective. Pulsatile tinnitus is less common than non-pulsatile and can be due to vascular tumour such as glomus or vascular abnormality. We presented an interesting case of a 30 year-old Malay lady with a two-year history of pulsatile tinnitus which was worsening in three months duration. It was associated with intermittent headache. Clinical examination and tuning fork test were unremarkable. Apart from mild hearing loss at high frequency on the left ear, the pure tone audiogram (PTA) was otherwise normal. In view of the patient’s young age with no risk factor for high frequency loss, a magnetic resonance imaging (MRI) was performed to look for any abnormality in the cerebellopontine angle. It revealed a single vessel looping around the left vestibulocochlear and facial nerves at the cisternal portion, likely a branch of the anteroinferior cerebellar artery (AICA). Literature review on the pathophysiology and treatment option in this condition is discussed. PMID:26417253

Unusual Turner syndrome mosaic with a triple x cell line (47,X/49,XXX) in a western lowland gorilla (Gorilla gorilla gorilla).

PubMed

Bradford, Carol M; Tupa, Lynn; Wiese, Debbie; Hurley, Timothy J; Zimmerman, Ralph

2013-12-01

A 29-yr-old female western lowland gorilla (Gorilla gorilla gorilla) was evaluated for low fertility and a midterm abortion. Laboratory testing included karyotyping, which revealed an unusual mosaicism for Turner syndrome with Triple X (47,X/49,XXX). This appears to be the first report of Turner syndrome in a great ape. In humans, Turner syndrome occurs in approximately 1 in 3,000 females, with half of those monosomic for the X chromosome. A small proportion is mosaic for a triple X cell line (3-4%). In humans, Turner syndrome is associated with characteristic phenotype including short stature, obesity, a broad chest with widely spaced nipples, webbing of the neck, and anteverted ears. This individual gorilla is significantly shorter in stature than conspecifics and is obese despite normal caloric intake. Individuals with Turner syndrome should also be screened for common health issues, including congenital heart defects, obesity, kidney abnormalities, hypertension, hypothyroidism, and diabetes mellitus. Animals with decreased fertility, multiple miscarriages, fetal losses, unusual phenotypes, or a combination of these symptoms should be evaluated for genetic abnormalities.

A Joint Time-Frequency and Matrix Decomposition Feature Extraction Methodology for Pathological Voice Classification

NASA Astrophysics Data System (ADS)

Ghoraani, Behnaz; Krishnan, Sridhar

2009-12-01

The number of people affected by speech problems is increasing as the modern world places increasing demands on the human voice via mobile telephones, voice recognition software, and interpersonal verbal communications. In this paper, we propose a novel methodology for automatic pattern classification of pathological voices. The main contribution of this paper is extraction of meaningful and unique features using Adaptive time-frequency distribution (TFD) and nonnegative matrix factorization (NMF). We construct Adaptive TFD as an effective signal analysis domain to dynamically track the nonstationarity in the speech and utilize NMF as a matrix decomposition (MD) technique to quantify the constructed TFD. The proposed method extracts meaningful and unique features from the joint TFD of the speech, and automatically identifies and measures the abnormality of the signal. Depending on the abnormality measure of each signal, we classify the signal into normal or pathological. The proposed method is applied on the Massachusetts Eye and Ear Infirmary (MEEI) voice disorders database which consists of 161 pathological and 51 normal speakers, and an overall classification accuracy of 98.6% was achieved.

Progression of growth in the external ear from birth to maturity: a 2-year follow-up study in India.

PubMed

Purkait, Ruma

2013-06-01

This study aimed to follow the growth dynamics of auricular dimensions from birth to the age of 18 years. The norms of dimensions at different ages, the peak growth period and the maturity age of the dimensions are essential information to Physicians for early clinical diagnosis or for deciding the optimal time for surgery to correct abnormalities. For this study, 2,147 children belonging to central Indian population were measured in at least three sequential sessions. Eight dimensions including the physiognomic length and width of the ear and its morphologic width; conchal length, width, and depth; and lobular length and width were measured using anthropometric technique. Three new dimensions (tragal length and height and maximum width of the antihelix) were introduced in the study. Three indices (auricular, conchal, and lobular) also were derived. Most dimensions exhibited very rapid growth during the first 3-6 months of infancy and thereafter proceeded at a slow pace until adulthood. The smaller dimensions (conchal depth, tragal height, and maximum width of the antihelix) increased continuously throughout the growth period. At birth, most of the dimensions were 52-76 % of their adult size, while tragal length and height were less than half their adult size. Unlike the other dimensions, the lobule length was smaller in males, probably due to the higher frequency of hypoplastic and bow-shaped lobules among them. The width dimensions matured earlier, at 5.6-11 years, whereas the maturity age of lengths varied from 12 to 16 years. The data generated in the current study will be useful to Physicians as a guideline in correcting auricular deformity and in constructing age progression charts of the external ear. Knowledge concerning the maturation age of the ear will help law enforcement authorities in deciding when to use it for establishing personal identification. This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Usefulness of 1000-Hz probe tone in tympanometry according to age in Korean infants.

PubMed

Park, Mina; Han, Kyu-Hee; Jung, Hyunseo; Kim, Mee-Hee; Chang, Hyun-Kyung; Kim, Shin Hye; Park, Moo Kyun; Lee, Jun Ho

2015-01-01

Numerous studies have shown the superiority of a 1000-Hz frequency probe tone for evaluating the middle ear status of infants. However, most of these studies examined Caucasian populations. This study validated the 1000-Hz probe tone and evaluated the age at which it should be used in Korean infants. Data from 83 infants (43 males, 40 females; mean age 9.2±6.2 (range 1-30) months, 165 ears) were analyzed. Tympanograms were classified according to Baldwin's modification of the method of Marchant et al. and correlated with results based on combined diagnostic tests, including an endoscopic examination of the tympanic membrane, myringotomy findings, and the air and bone conduction auditory brainstem response (ABR) thresholds. Data were analyzed in five age groups, each covering a 3-month range. The traces were measured for both 226- and 1000-Hz probe tones. The sensitivity and specificity for the different age groups were also determined. For the 226-Hz probe tone, the tympanograms showed normal traces for most ears with otitis media effusions in infants younger than 12 months. By contrast, the tympanograms using the 1000-Hz probe tone showed abnormal traces in most of the infants with otitis media effusions in all age groups. In infants with no otitis media effusion, the tympanograms using both 226- and 1000-Hz probe tones were interpreted as normal in most cases in all age groups. In infants younger than 12 months, the sensitivity of the 226-Hz probe tone was very low (0-6.6%), whereas that of the 1000-Hz probe tone was very high (90-100%). In infants older than 13 months, however, the sensitivities of the 226- and 1000-Hz probe tones were 76.2% and 85.7%, respectively. Regarding specificity, the difference between the two probe tones was not significant for any age group. This study confirmed the superiority of the 1000-Hz probe tone for evaluating the middle ear in infants. We recommend using a 1000-Hz probe tone at least up to the age of 12 months for Korean infants. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

Dynamic Properties of Human Tympanic Membrane Based on Frequency-Temperature Superposition

PubMed Central

Zhang, Xiangming; Gan, Rong Z.

2012-01-01

The human tympanic membrane (TM) transfers sound in the ear canal into the mechanical vibration of the ossicles in the middle ear. The dynamic properties of TM directly affect the middle ear transfer function. The static or quasi-static mechanical properties of TM were reported in the literature, but the dynamic properties of TM over the auditory frequency range are very limited. In this paper, a new method was developed to measure the dynamic properties of human TM using the Dynamic-Mechanical Analyzer (DMA). The test was conducted at the frequency range of 1 to 40 Hz at three different temperatures: 5°, 25° and 37°C. The frequency-temperature superposition was applied to extend the testing frequency range to a much higher level (at least 3800 Hz). The generalized linear solid model was employed to describe the constitutive relation of the TM. The storage modulus E’ and the loss modulus E” were obtained from 11 specimens. The mean storage modulus was 15.1 MPa at 1 Hz and 27.6 MPa at 3800 Hz. The mean loss modulus was 0.28 MPa at 1 Hz and 4.1 MPa at 3800 Hz. The results show that the frequency-temperature superposition is a feasible approach to study the dynamic properties of the ear soft tissues. The dynamic properties of human TM obtained in this study provide a better description of the damping behavior of ear tissues. The properties can be transferred into the finite element (FE) model of the human ear to replace the Rayleigh type damping. The data reported here contribute to the biomechanics of the middle ear and improve the accuracy of the FE model for the human ear. PMID:22820983

Wheat Ear Detection in Plots by Segmenting Mobile Laser Scanner Data

NASA Astrophysics Data System (ADS)

Velumani, K.; Oude Elberink, S.; Yang, M. Y.; Baret, F.

2017-09-01

The use of Light Detection and Ranging (LiDAR) to study agricultural crop traits is becoming popular. Wheat plant traits such as crop height, biomass fractions and plant population are of interest to agronomists and biologists for the assessment of a genotype's performance in the environment. Among these performance indicators, plant population in the field is still widely estimated through manual counting which is a tedious and labour intensive task. The goal of this study is to explore the suitability of LiDAR observations to automate the counting process by the individual detection of wheat ears in the agricultural field. However, this is a challenging task owing to the random cropping pattern and noisy returns present in the point cloud. The goal is achieved by first segmenting the 3D point cloud followed by the classification of segments into ears and non-ears. In this study, two segmentation techniques: a) voxel-based segmentation and b) mean shift segmentation were adapted to suit the segmentation of plant point clouds. An ear classification strategy was developed to distinguish the ear segments from leaves and stems. Finally, the ears extracted by the automatic methods were compared with reference ear segments prepared by manual segmentation. Both the methods had an average detection rate of 85 %, aggregated over different flowering stages. The voxel-based approach performed well for late flowering stages (wheat crops aged 210 days or more) with a mean percentage accuracy of 94 % and takes less than 20 seconds to process 50,000 points with an average point density of 16  points/cm2. Meanwhile, the mean shift approach showed comparatively better counting accuracy of 95% for early flowering stage (crops aged below 225 days) and takes approximately 4 minutes to process 50,000 points.

Alternative metrics for real-ear-to-coupler difference average values in children.

PubMed

Blumsack, Judith T; Clark-Lewis, Sandra; Watts, Kelli M; Wilson, Martha W; Ross, Margaret E; Soles, Lindsey; Ennis, Cydney

2014-10-01

Ideally, individual real-ear-to-coupler difference (RECD) measurements are obtained for pediatric hearing instrument-fitting purposes. When RECD measurements cannot be obtained, age-related average RECDs based on typically developing North American children are used. Evidence suggests that these values may not be appropriate for populations of children with retarded growth patterns. The purpose of this study was to determine if another metric, such as head circumference, height, or weight, can be used for prediction of RECDs in children. Design was a correlational study. For all participants, RECD values in both ears, head circumference, height, and weight were measured. The sample consisted of 68 North American children (ages 3-11 yr). Height, weight, head circumference, and RECDs were measured and were analyzed for both ears at 500, 750, 1000, 1500, 2000, 3000, 4000, and 6000 Hz. A backward elimination multiple-regression analysis was used to determine if age, height, weight, and/or head circumference are significant predictors of RECDs. For the left ear, head circumference was retained as the only statistically significant variable in the final model. For the right ear, head circumference was retained as the only statistically significant independent variable at all frequencies except at 2000 and 4000 Hz. At these latter frequencies, weight was retained as the only statistically significant independent variable after all other variables were eliminated. Head circumference can be considered as a metric for RECD prediction in children when individual measurements cannot be obtained. In developing countries where equipment is often unavailable and stunted growth can reduce the value of using age as a metric, head circumference can be considered as an alternative metric in the prediction of RECDs. American Academy of Audiology.

A clip-free eyeglasses-based wearable monitoring device for measuring photoplethysmograhic signals.

PubMed

Zheng, Yali; Leung, Billy; Sy, Stanley; Zhang, Yuanting; Poon, Carmen C Y

2012-01-01

An eyeglasses-based device has been developed in this work to acquire photoplethysmogram (PPG) from the nose bridge. This device is aimed to provide wearable physiological monitoring without uncomfortable clips frequently used in PPG measurement from finger and ear. Switching control is applied on the LED and photo detector for power saving. An experiment involving postural change and treadmill jogging among 10 healthy young subjects was carried out to evaluate the performance of the device. Electrocardiogram (ECG) and PPG from finger, ear and nose were simultaneously recorded, from which heart rate (HR) and pulse transit time (PTT) were calculated. The results show that PPG measured from nose and ear are more resistant to motion than signal from finger during exercise. In addition, the difference between PTT measured from ear and nose indicates that local vasomotor activities may exist on ear and/or nose channel, and suggests that PPG from different sites should be used for cuff-less PTT-based BP estimation. We conclude that this wearable device has great potential to be used in the healthcare management in the future.

Transgenic expression of a maize geranyl geranyl transferase gene sequence in maize callus increases resistance to ear rot pathogens

USDA-ARS?s Scientific Manuscript database

Determining the genes responsible for pest resistance in maize can allow breeders to develop varieties with lower losses and less contamination with undesirable toxins. A gene sequence coding for a geranyl geranyl transferase-like protein located in a fungal ear rot resistance quantitative trait loc...

Magnetic resonance imaging of the inner ear by using a hybrid radiofrequency coil at 7 T

NASA Astrophysics Data System (ADS)

Kim, Kyoung-Nam; Heo, Phil; Kim, Young-Bo; Han, Gyu-Cheol

2015-01-01

Visualization of the membranous structures of the inner ear has been limited to the detection of the normal fluid signal intensity within the bony labyrinth by using magnetic resonance imaging (MRI) equipped with a 1.5 Tesla (T) magnet. High-field (HF) MRI has been available for more than a decade, and numerous studies have documented its significant advantages over conventional MRI with regards to its use in basic scientific research and routine clinical assessments. No previous studies of the inner ear by using HF MRI have been reported, in part because high-quality resolution of mastoid pneumatization is challenging due to artifacts generated in the HF environment and insufficient performance of radiofrequency (RF) coils. Therefore, a hybrid RF coil with integrated circuitry was developed at 7 T and was targeted for anatomical imaging to achieve a high resolution image of the structure of the human inner ear, excluding the bony portion. The inner-ear's structure is composed of soft tissues containing hydrogen ions and includes the membranous labyrinth, endolymphatic space, perilymphatic space, and cochlear-vestibular nerves. Visualization of the inner-ear's anatomy was performed in-vivo with a custom-designed hybrid RF coil and a specific imaging protocol based on an interpolated breath-held examination sequence. The comparative signal intensity value at 30-mm away from the phantom side was 88% higher for the hybrid RF coil and 24% higher for the 8-channel transmit/receive (Tx/Rx) coil than for the commercial birdcage coil. The optimized MRI protocol employed a hybrid RF coil because it enabled high-resolution imaging of the inner-ear's anatomy and accurate mapping of structures including the cochlea and the semicircular canals. These results indicate that 7 T MRI achieves high spatial resolution visualization of the inner-ear's anatomy. Therefore, MRI imaging using a hybrid RF coil at 7 T could provide a powerful tool for clinical investigations of petrous pathologies of the inner ear.

[Effect size on resonance of the outer ear canal by simulation of middle ear lesions using a temporal bone preparation].

PubMed

Scheinpflug, L; Vorwerk, U; Begall, K

1995-01-01

By means of a model of the external and the middle ear it is possible to simulate various, exactly defined pathological conditions of the middle ear and to describe their influence on ear canal resonance. Starting point of the investigations are fresh postmortem preparations of 8 human temporal bones with an intact ear drum and a retained skin of the ear canal. The compliance of the middle ear does not significantly differ from the clinical data of probands with healthy ears. After antrotomy it is possible to simulate pathological conditions of the middle ear one after the other at the same temporal bone. The influence of the changed middle ear conditions on ear drum compliance, ear canal volume and on the resonance curve of the external ear canal was investigated. For example, the middle ear was filled with water to create approximately the same conditions as in acute serous otitis media. In this middle ear condition a significant increase of the sound pressure amplification was found, on an average by 4 decibels compared to the unchanged temporal bone model. A small increase in resonance frequency was also measured. The advantages of this model are the approximately physiological conditions and the constant dimensions of the external and middle ear.