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This page shows you the most recent publications within this specialty of the MedWorm directory. This is page number 7.

Gene therapy for the inner ear.
We describe several gene vectors useful for inner ear gene therapy and the practical aspects of introducing these vectors into the ear. We then review the progress toward using gene transfer for therapies in both auditory and balance systems, and discuss the technological milestones needed to advance to clinical application of these methods. PMID: 23265411 [PubMed - as supplied by publisher]
Source: Hearing Research - December 18, 2012 Category: Audiology Authors: Fukui H, Raphael Y Tags: Hear Res Source Type: research

Noise-induced alterations in cochlear mechanics, electromotility, and cochlear amplification.
Abstract Loud sounds are a common cause of hearing loss. Very intense sounds may result in permanent hearing loss, but lower levels typically cause a transient decrease in auditory sensitivity. Studies have arrived at different conclusions as regards the physiological mechanisms underlying such temporary threshold shifts. Here, we investigated the effect of acoustic overstimulation on the mechanics of the low-frequency areas of the guinea pig cochlea. We demonstrate that brief loud sound exposure results in an increased phase lag and a paradoxical frequency-specific increase of sound-evoked displacement. Despite th...
Source: Pflugers Archiv : European Journal of Physiology - December 18, 2012 Category: Physiology Authors: Jacob S, Johansson C, Fridberger A Tags: Pflugers Arch Source Type: research

Postnatal corticosteroids and neurodevelopmental outcomes in extremely low birthweight or extremely preterm infants: 15-year experience in Victoria, Australia
Conclusions PCS use decreased in 2005 compared with earlier eras, and was accompanied by a rise in BPD, with no significant changes in mortality or neurological morbidity.
Source: Archives of Disease in Childhood - Fetal and Neonatal Edition - December 18, 2012 Category: Perinatology & Neonatology Authors: Cheong, J. L., Anderson, P., Roberts, G., Duff, J., Doyle, L. W., Victorian Infant Collaborative Study Group Tags: Eye Diseases, Bronchopulmonary dysplasia, Epidemiologic studies, Cerebral palsy, Ophthalmology, Child health, Infant health, Neonatal health, Disability, Ear, nose and throat/otolaryngology Original articles Source Type: research

Woman Implanted With Invisible Hearing Aid at UAMC, First in Southwest
Dr. Abraham Jacob, associate professor in the UA Department of Surgery Division of Otolaryngology, recently operated on the first person in the Southwest to receive a surgically implanted hearing device known as the Esteem. Jacob is the only surgeon in the Southwest – and one of only about 15 surgeons in the nation – approved to implant the device. The surgery was done at The University of Arizona Medical Center.
Source: The University of Arizona: Health - December 18, 2012 Category: Universities & Medical Training Authors: Alexis Blue Source Type: research

The human OPA1delTTAG mutation induces premature age-related systemic neurodegeneration in mouse
Dominant optic atrophy is a rare inherited optic nerve degeneration caused by mutations in the mitochondrial fusion gene OPA1. Recently, the clinical spectrum of dominant optic atrophy has been extended to frequent syndromic forms, exhibiting various degrees of neurological and muscle impairments frequently found in mitochondrial diseases. Although characterized by a specific loss of retinal ganglion cells, the pathophysiology of dominant optic atrophy is still poorly understood. We generated an Opa1 mouse model carrying the recurrent Opa1delTTAG mutation, which is found in 30% of all patients with dominant optic atrophy. ...
Source: Brain - December 18, 2012 Category: Neurology Authors: Sarzi, E., Angebault, C., Seveno, M., Gueguen, N., Chaix, B., Bielicki, G., Boddaert, N., Mausset-Bonnefont, A.-L., Cazevieille, C., Rigau, V., Renou, J.-P., Wang, J., Delettre, C., Brabet, P., Puel, J.-L., Hamel, C. P., Reynier, P., Lenaers, G. Tags: Original Articles Source Type: research

An In Vitro Model of Developmental Synaptogenesis Using Cocultures of Human Neural Progenitors and Cochlear Explants
Stem Cells and Development , Vol. 0, No. 0.
Source: Stem Cells and Development - December 16, 2012 Category: Stem Cells Tags: article Source Type: research

Dr. William F. House, Inventor of Cochlear Implant, Dies
Dr. House developed a surgical procedure for a debilitating form of vertigo that cured the astronaut Alan B. Shepard.
Source: NYT Health - December 15, 2012 Category: Consumer Health News Authors: By DOUGLAS MARTIN Tags: Shepard, Alan B Jr Ears and Hearing Deaths (Obituaries) 3M Company MMM NYSE House, William S. Cochlear Implants Source Type: news

Nigeria: U.S. Based Firm to Offer Free Medicare in Uvwie, Warri
[Vanguard]Warri -About 1000 Nigerians living in Uvwie and Warri South local government areas will benefit from a one-day free medical service sponsored by a US based medical firm, Boro Audiology Clinic.
Source: AllAfrica News: Health and Medicine - December 15, 2012 Category: African Health Source Type: news

Sensitivity of cochlear nucleus neurons to spatio-temporal changes in auditory nerve activity
The spatio-temporal pattern of auditory nerve (AN) activity, representing the relative timing of spikes across the tonotopic axis, contains cues to perceptual features of sounds such as pitch, loudness, timbre, and spatial location. These spatio-temporal cues may be extracted by neurons in the cochlear nucleus (CN) that are sensitive to relative timing of inputs from AN fibers innervating different cochlear regions. One possible mechanism for this extraction is "cross-frequency" coincidence detection (CD), in which a central neuron converts the degree of coincidence across the tonotopic axis into a rate code by preferentia...
Source: Journal of Neurophysiology - December 15, 2012 Category: Neurology Authors: Wang, G. I., Delgutte, B. Tags: Articles Source Type: research

Hearing Loss and Heavy Metal Toxicity in a Nicaraguan Mining Community: Audiological Results and Case Reports
Audiol Neurotol 2013;18:101–113 (DOI:10.1159/000345470)
Source: Audiology and Neurotology - December 14, 2012 Category: Audiology Source Type: research

Pre‐target axon sorting in the avian auditory brainstem
In this study we examine whether the decussating NM axons forming the crossed dorsal cochlear tract (XDCT) and innervating the contralateral NL are arranged in a systematic manner. We electroporated dye into cells in different frequency regions of NM to anterogradely label their axons in the XDCT. The placement of dye in NM was compared to the location of labeled axons in XDCT. Our results show that NM axons in XDCT are organized in a precise tonotopic manner along the rostrocaudal axis, spanning over the entire rostrocaudal extent of both the origin and target nuclei. We propose that in the avian auditory brainstem, this ...
Source: The Journal of Comparative Neurology - December 14, 2012 Category: Neurology Authors: Daniel T. Kashima, Edwin W Rubel, Armin H. Seidl Tags: Research Article Source Type: research

Acknowledgement to the Reviewers
Audiol Neurotol 2013;18:1–2 (DOI:10.1159/000342783)
Source: Audiology and Neurotology - December 13, 2012 Category: Audiology Source Type: research

Front & Back Matter
Audiol Neurotol 2013;18:X (DOI:10.1159/000346464)
Source: Audiology and Neurotology - December 13, 2012 Category: Audiology Source Type: research

Acknowledgement to the Reviewers
Audiol Neurotol 2013;18:1–2 (DOI:10.1159/000342783)
Source: Audiology and Neurotology - December 13, 2012 Category: Audiology Source Type: research

Front & Back Matter
Audiol Neurotol 2013;18:X (DOI:10.1159/000346464)
Source: Audiology and Neurotology - December 13, 2012 Category: Audiology Source Type: research

Researcher Finds Gender Differences In Seasonal Auditory Changes
Auditory systems differ between sexes in sparrows depending on the season, a Georgia State University neuroscientist has found. The work adds to our knowledge of how the parts of the nervous system, including that of humans, are able to change. Megan Gall, a post-doctoral researcher with Georgia State's Neuroscience Institute, tested the peripheral auditory systems of male and female house sparrows, comparing the hearing of each gender during non-breeding seasons and breeding seasons...
Source: Health News from Medical News Today - December 13, 2012 Category: Consumer Health News Tags: Hearing / Deafness Source Type: news

Vestibular and cochlear neuritis in patients with Ramsay Hunt syndrome: a Gd-enhanced MRI study.
Conclusion: It is suggested that vertigo in patients with Ramsay Hunt syndrome is mostly induced by superior vestibular neuritis consecutive to the reactivation of varicella-zoster virus (VZV) infection from the geniculate ganglion through the faciovestibular anastomosis. Refractory hearing loss in patients with Ramsay Hunt syndrome may be due to cochlear neuritis following the spread of VZV. Objectives: An attempt was made to selectively identify vestibulocochlear nerves in the internal auditory canal (IAC) on gadolinium (Gd)-enhanced MRI in patients with Ramsay Hunt syndrome. Methods: Fourteen patients with Ramsay Hunt s...
Source: Acta Oto-Laryngologica - December 13, 2012 Category: ENT & OMF Authors: Iwasaki H, Toda N, Takahashi M, Azuma T, Nakamura K, Takao SI, Harada M, Takeda N Tags: Acta Otolaryngol Source Type: research

Measurements of three-dimensional shape and sound-induced motion of the chinchilla tympanic membrane.
This article is part of a Special Issue entitled "MEMRO 2012". Summary: A new method for simultaneously measuring the shape and sound-induced motion of the tympanic membrane is utilized to estimate the 3D motion on the membrane surface. PMID: 23247058 [PubMed - as supplied by publisher]
Source: Hearing Research - December 13, 2012 Category: Audiology Authors: Rosowski JJ, Dobrev I, Khaleghi M, Lu W, Cheng JT, Harrington E, Furlong C Tags: Hear Res Source Type: research

Listen, and Ye Shall Speak: Facilitating Spoken Language Development Through Auditory Training
Source: Journal of Deaf Studies and Deaf Education - December 13, 2012 Category: Audiology Authors: Sarant, J. Z. Tags: Book Reviews Source Type: research

Development and Initial Validation of a Questionnaire to Measure Hearing Parents' Perceptions of Health Care Professionals' Advice
This study reports the development of The Hearing Parents’ Perceptions of Health Professionals’ Advice Questionnaire (HPP/HPQ). This questionnaire was designed to investigate the impact of the advice and information that parents receive from health professionals during the time when their child’s hearing loss is identified and how parents, in turn, make initial decisions about services and interventions for their deaf child. Once developed, the HPP/HPQ was partially validated on 2 separate samples. Analysis of data from both samples supported a 14-item questionnaire, with all items loading onto a single c...
Source: Journal of Deaf Studies and Deaf Education - December 13, 2012 Category: Audiology Authors: Day, L. A., Brice, P. Tags: Empirical Manuscript Source Type: research

The British Sign Language Versions of the Patient Health Questionnaire, the Generalized Anxiety Disorder 7-Item Scale, and the Work and Social Adjustment Scale
The present study is aimed to translate 3 widely used clinical assessment measures into British Sign Language (BSL), to pilot the BSL versions, and to establish their validity and reliability. These were the Patient Health Questionnaire (PHQ-9), the Generalized Anxiety Disorder 7-item (GAD-7) scale, and the Work and Social Adjustment Scale (WSAS). The 3 assessment measures were translated into BSL and piloted with the Deaf signing population in the United Kingdom (n = 113). Participants completed the PHQ-9, GAD-7, WSAS, and Clinical Outcomes in Routine Evaluation–Outcome Measure (CORE-OM) online. The reliability and ...
Source: Journal of Deaf Studies and Deaf Education - December 13, 2012 Category: Audiology Authors: Rogers, K. D., Young, A., Lovell, K., Campbell, M., Scott, P. R., Kendal, S. Tags: Empirical Manuscript Source Type: research

Consonant Development in Pediatric Cochlear Implant Users Who Were Implanted Before 30 Months of Age
This study provided a yearly record of consonant development for the initial 4 years of cochlear implant (CI) use and established a precedent for using a standardized articulation test, the Goldman–Fristoe Test of Articulation—2 (Goldman, R., & Fristoe, M. [2000]. Goldman–Fristoe Test of Articulation—2. Circle Pines, MN: American Guidance Services). The study used CI age as a referent for 32 children who received their CI before 30 months of age. Consonants produced by 70% of the children were listed, as were the most common error types, which were consonant omissions and substitutions. Using co...
Source: Journal of Deaf Studies and Deaf Education - December 13, 2012 Category: Audiology Authors: Spencer, L. J., Guo, L.-Y. Tags: Empirical Manuscript Source Type: research

"We Communicated That Way for a Reason": Language Practices and Language Ideologies Among Hearing Adults Whose Parents Are Deaf
Families with deaf parents and hearing children are often bilingual and bimodal, with both a spoken language and a signed one in regular use among family members. When interviewed, 13 American hearing adults with deaf parents reported widely varying language practices, sign language abilities, and social affiliations with Deaf and Hearing communities. Despite this variation, the interviewees’ moral judgments of their own and others’ communicative behavior suggest that these adults share a language ideology concerning the obligation of all family members to expend effort to overcome potential communication barri...
Source: Journal of Deaf Studies and Deaf Education - December 13, 2012 Category: Audiology Authors: Pizer, G., Walters, K., Meier, R. P. Tags: Empirical Manuscript Source Type: research

Rethinking Education of Deaf Children in Zimbabwe: Challenges and Opportunities for Teacher Education
The study explores the communication challenges faced by teacher trainees in teaching deaf learners and the opportunities that they present. A critical disabilities study approach within the qualitative paradigm was employed to collect interview data from 14 trainee teachers (6 were men and 8 women) and 5 of their specialist mentors (all of them were women) at 3 special schools in Zimbabwe. The trainees were aged 28–45. Data were analyzed using theme identification methods. Results showed that all the mentors and trainees without deaf assistants tended to teach using spoken language and even though they had no prior ...
Source: Journal of Deaf Studies and Deaf Education - December 13, 2012 Category: Audiology Authors: Musengi, M., Ndofirepi, A., Shumba, A. Tags: Empirical Manuscript Source Type: research

School Placement and Perceived Quality of Life in Youth Who Are Deaf or Hard of Hearing
This study examined the relationship between quality of life and educational placement that include and do not include other DHH youth. Participants included 221 DHH youth, ages 11–18 with bilateral hearing loss. Results showed that there were few differences in quality of life related to school placement (with age, gender, depression symptoms, and hearing level as covariates). For both participation and perceived stigma, there was an interaction between school placement and parent hearing status, with no single school placement showing the best results. DHH youth with hearing parents in schools specifically for DHH ...
Source: Journal of Deaf Studies and Deaf Education - December 13, 2012 Category: Audiology Authors: Schick, B., Skalicky, A., Edwards, T., Kushalnagar, P., Topolski, T., Patrick, D. Tags: Empirical Manuscript Source Type: research

An Intervention for Early Mathematical Success: Outcomes from the Hybrid Version of the Building Math Readiness Parents as Partners (MRPP) Project
The Building Math Readiness in Young Deaf/Hard-of- Hearing Children: Parents as Partners (MRPP) Project works with parents to increase the understanding of foundational mathematics concepts in their preschool deaf/hard-of-hearing (d/hh) children in preparation for formal mathematics education. A multiple-case/single-unit case study incorporating descriptive statistics and grounded theory analysis was conducted on the hybrid version of the intervention. Results showed productive changes in parental behaviors indicating a possible positive effect on parent knowledge, recognition, and mediation of early ma.thematics concepts ...
Source: Journal of Deaf Studies and Deaf Education - December 13, 2012 Category: Audiology Authors: Kritzer, K. L., Pagliaro, C. M. Tags: Empirical Manuscript Source Type: research

Deaf Children Attending Different School Environments: Sign Language Abilities and Theory of Mind
The present study examined whether full access to sign language as a medium for instruction could influence performance in Theory of Mind (ToM) tasks. Three groups of Italian participants (age range: 6–14 years) participated in the study: Two groups of deaf signing children and one group of hearing–speaking children. The two groups of deaf children differed only in their school environment: One group attended a school with a teaching assistant (TA; Sign Language is offered only by the TA to a single deaf child), and the other group attended a bilingual program (Italian Sign Language and Italian). Linguistic abi...
Source: Journal of Deaf Studies and Deaf Education - December 13, 2012 Category: Audiology Authors: Tomasuolo, E., Valeri, G., Di Renzo, A., Pasqualetti, P., Volterra, V. Tags: Empirical Manuscript Source Type: research

Bimodal Bilingualism and the Frequency-Lag Hypothesis
The frequency-lag hypothesis proposes that bilinguals have slowed lexical retrieval relative to monolinguals and in their nondominant language relative to their dominant language, particularly for low-frequency words. These effects arise because bilinguals divide their language use between 2 languages and use their nondominant language less frequently. We conducted a picture-naming study with hearing American Sign Language (ASL)–English bilinguals (bimodal bilinguals), deaf signers, and English-speaking monolinguals. As predicted by the frequency-lag hypothesis, bimodal bilinguals were slower, less accurate, and exhi...
Source: Journal of Deaf Studies and Deaf Education - December 13, 2012 Category: Audiology Authors: Emmorey, K., Petrich, J. A. F., Gollan, T. H. Tags: Empirical Manuscript Source Type: research

Table_of_Contents
Source: Journal of Deaf Studies and Deaf Education - December 13, 2012 Category: Audiology Tags: Cover / Standing Material Source Type: research

Subscription
Source: Journal of Deaf Studies and Deaf Education - December 13, 2012 Category: Audiology Tags: Cover / Standing Material Source Type: research

Editorial_Board
Source: Journal of Deaf Studies and Deaf Education - December 13, 2012 Category: Audiology Tags: Cover / Standing Material Source Type: research

Cover
Source: Journal of Deaf Studies and Deaf Education - December 13, 2012 Category: Audiology Tags: Cover / Standing Material Source Type: research

Dr. William F. House dies at 89; championed cochlear implant
Dr. William F. House also developed a successful surgery for an ear disease that had prevented astronaut Alan Shepard from returning to space.Dr. William F. House, a dentist-turned-ear specialist who 50 years ago defied the medical establishment and many advocates for the hearing impaired to champion an implantable device, now widely accepted, that made everyday sounds audible to the profoundly deaf, has died. He was 89.
Source: L.A. Times - Health - December 12, 2012 Category: Consumer Health News Source Type: news

Cleft palate children need regular hearing check-ups
Children with cleft palate require regular audiologic and otologic follow-up to ensure their management is appropriate and timely, say researchers.
Source: MedWire News - Pediatrics - December 12, 2012 Category: Pediatrics Source Type: news

Assessing Literacy in Deaf Individuals
Neurocognitive Measurement and PredictorsHumans’ development of literacy has been a recent focus of intense research across the reading, cognitive, and neuroscience fields. But for individuals who are deaf—who rely greatly on their visual skills for language and learning—the findings don’t necessarily apply, leaving theoretical and practical gaps in approaches to their education.Assessing Literacy in Deaf Individuals: Neurocognitive ...
Source: Springer Psychology titles - December 12, 2012 Category: Psychiatry & Psychology Tags: Child and School Psychology Source Type: news

Risk of Sudden Sensorineural Hearing Loss in Patients with Systemic Lupus Erythematosus: A Population-Based Cohort Study
Audiol Neurotol 2013;18:95–100 (DOI:10.1159/000345512)
Source: Audiology and Neurotology - December 12, 2012 Category: Audiology Source Type: research

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Source: National Association of the Deaf - December 12, 2012 Category: Audiology Authors: shane.feldman Source Type: news

Deadline Extended: NAD Seeks Director of Communications
UPDATE: The deadline for submission has been extended to January 15th, 2013. Apply for this position by contacting us at jobs@nad.org. The National Association of the Deaf (NAD) seeks a Director of Communications to work at its Silver Spring office and oversee the organization's communications as well as engage in marketing efforts. The Director will ensure that all communications are timely, compelling, effective, and representative of the organization's mission, vision, and values. read more
Source: National Association of the Deaf - December 12, 2012 Category: Audiology Authors: admin Source Type: news

NAD Seeks Director of Communications
The National Association of the Deaf (NAD) seeks a Director of Communications to work at its Silver Spring office and oversee the organization's communications as well as engage in marketing efforts. The Director will ensure that all communications are timely, compelling, effective, and representative of the organization's mission, vision, and values. The Director leads the development and implementation of the NAD communications and brand strategies in order to strengthen the organization's engagement with its members, stakeholders, and the general public. read more
Source: National Association of the Deaf - December 12, 2012 Category: Audiology Authors: admin Source Type: news

Both Teens And Tweens Should Be Tested For Hearing Loss
Just yelling "turn it down" isn't enough when young people are blasting music directly into their ears via earbuds and headphones, parents say. A new poll from the University of Michigan shows parents are strongly in favor of required hearing screenings for kids all the way up to age 17. The University of Michigan Mott Children's Hospital National Poll on Children's Health recently asked a nationwide sample of parents of children 0-17 years old about whether they'd support requirements for hearing screening and where they'd prefer to have the screening done...
Source: Health News from Medical News Today - December 12, 2012 Category: Consumer Health News Tags: Hearing / Deafness Source Type: news

Influence of the middle ear anatomy on the performance of a membrane sensor in the incudostapedial joint gap.
This study investigates the implantable microphone sensor and its implantation concept. It intends to quantify the influence of the sensor's insertion position on the achievable microphone sensitivity. The investigation considers anatomical and pathological variations of the middle ear geometry and its space limitations. Temporal bone experiments on a laboratory model show that anatomical and pathological variations of the middle ear geometry can prevent the sensor from being placed optimally within the incudostapedial joint. Beyond scattering of transfer functions due to anatomic variations of individual middle ears there...
Source: Hearing Research - December 12, 2012 Category: Audiology Authors: Koch M, Seidler H, Hellmuth A, Bornitz M, Lasurashvili N, Zahnert T Tags: Hear Res Source Type: research

Symptomatic lipid storage in carriers for the PNPLA2 gene
mermann & Eva Morava
Source: European Journal of Human Genetics - December 12, 2012 Category: Genetics & Stem Cells Authors: Mirian C H JanssenBaziel van EngelenLivia KapustaMartin LammensMartin van DijkJudith FischerMarinette van der GraafRon A WeversManuela FahrleitnerRobert ZimmermannEva Morava Tags: symptomatic carriers deafness lipid myopathy neutral lipid storage insulin resistance implantable cardioverter-defibrillator Source Type: research

Gene expression profiling in genetic animal models of provide elements to unveil the molecular mechanisms underlying epileptogenesis in rodents
CONCLUSION: This study may help to clarify the underlying molecular mechanism that leads to the predisposition to seizures in these animals. Our results indicate the activation of distinct molecular pathways in both models.
Source: Journal of Epilepsy and Clinical Neurophysiology - December 11, 2012 Category: Neurology Source Type: research

Scientists Identify Molecules In The Ear That Convert Sound Into Brain Signals
For scientists who study the genetics of hearing and deafness, finding the exact genetic machinery in the inner ear that responds to sound waves and converts them into electrical impulses, the language of the brain, has been something of a holy grail. Now this quest has come to fruition. Scientists at The Scripps Research Institute (TSRI) in La Jolla, CA, have identified a critical component of this ear-to-brain conversion - a protein called TMHS...
Source: Health News from Medical News Today - December 11, 2012 Category: Consumer Health News Tags: Hearing / Deafness Source Type: news

The rarity of silence - or - what can we hear from history
The present article gives a short analysis of emerging of historical knowledge and its relations to human senses. People are re-constructing history using different cognitive strategies and different approaches to how to use our ability to see, to hear and to touch. People are historically deaf and disabled – we cannot hear an enormous part of our history, and in terms of understanding the world we may learn from really disabled persons – who are fully capable to create images of the surrounding reality accurately and adequately. During the past decades, due to the great development of technology, we have becom...
Source: Culture - December 11, 2012 Category: Psychiatry & Psychology Authors: Maslov, K. S. Tags: History, Sound & amp;amp; Psychology: Source Type: research

Uganda: Plan for the Blind, Deaf On HIV Prevention
[New Vision]JULIE (not real name) is a 14-year-old girl with hearing impairment. Initially she was raped and later repeatedly coerced into sex with gifts and threats by a businessman.
Source: AllAfrica News: Health and Medicine - December 10, 2012 Category: African Health Source Type: news

Clinical Reasoning: An unusual cause of multiple cranial nerve impairment
In January 2009, an 89-year-old Caucasian man was referred to our hospital for progressive multiple cranial neuropathies. His past medical history included arterial hypertension, acute pancreatitis, heart failure, and in situ cutaneous squamous cell carcinoma (SCC) in the left temporal scalp, which was treated with total local excision (negative margin) in 2001. Two years later (2003), a right temporal SCC was diagnosed and treated with surgery and local radiotherapy (54 gray). In June 2006, a recurrence occurred on the same side, and pathology from the surgical excision revealed negative margins. In September 2007, he was...
Source: Neurology - December 10, 2012 Category: Neurology Authors: Roubeau, V., Diard-Detoeuf, C., Moriniere, S., Cottier, J.-P., Limousin, N., de Toffol, B., Hommet, C., Mondon, K. Tags: MRI, All Clinical Neurology, Nerve tumor RESIDENT AND FELLOW SECTION Source Type: research

Early presentation of gait impairment in Wolfram Syndrome
Background: Classically characterized by early onset insulin-dependent diabetes mellitus, optic atrophy, deafness, diabetes insipidus, and neurological abnormalities, Wolfram syndrome (WFS) is also associated with atypical brainstem and cerebellar findings in the first decade of life. As such, we hypothesized that gait differences between individuals with WFS and typically developing (TD) individuals may be detectable across the course of the disease. Methods: Gait was assessed for 13 individuals with WFS (min 6.4 yrs, max 25.8 yrs) and 29 age-matched, typically developing individuals (min 5.6 yrs, max 28.5 yrs) using a GA...
Source: Orphanet Journal of Rare Diseases - December 8, 2012 Category: Internal Medicine Authors: Kristen PickettRyan DuncanJames HoekelBess MarshallTamara HersheyGammon Earhart Source Type: research