Prevalence of Peripheral Neuropathy Among Patients With Auditory Neuropathy
Auditory Neuropathy Spectrum Disorder,Peripheral NeuropathyAuditory neuropathy/ dys-synchrony/ peri-synaptic audiopathy (AN) are terms used to describe a specific hearing disorder with a disturbed auditory neural response in presence of normal cochlear function. Initially reported as early as the 1970s by ( Hinchcliffe et al) after observation of the patients with normal hearing thresholds, who had difficulty to detect the sounds especially in presence of background noise . Auditory Neuropathy is characterized by five integral features that distinguish it from other types of hearing loss. These are: Variable audiometric results anywhere from normal hearing to Sensorineural hearing loss of any degree up to profound hearing loss showing unusual audiometric pattern , Preserved outer hair cells (OHCs) responses such as otoacoustic emissions (OAEs) and/or cochlear microphonics (CM) , Altered neural processing such abnormal auditory brainstem responses (ABRs) with a reduced or absent wave V , Poor speech perception with poor speech recognition score that seems out of proportion compared to their pure-tone detection thresholds. , absent stapedial reflexes to the ipsilateral and contralateral tone at a 110-dB hearing level . In 2008 it was found that AN does not represent a single disease entity but it is a heterogeneous disease category with a wide range of hearing loss types ,etiologies , age and clinical manifestations hence the adoption of the term auditory neuropathy spectrum disorder (ANSD) by an International Newborn Hearing Screening Conference held in Italy following a comprehensive study of newborn hearing test results. The prevalence of ANSD is variable in published studies from (0.23 to 15%) among hearing impaired individuals . Regarding the underlying etiological factors, auditory neuropathy can be congenital or acquired . AN without the involvement of the neurological system has been termed as primary auditory neuropathy (PAN). Peripheral neuropathy is not a constant finding in all patients with this hearing disorder , AN may occur in the afferent pathway of acoustic nerve, probably accompanied by the pathological changes of efferent nerve in the olivocochlear system inside the brainstem Our study is conducted to detect the prevalence of peripheral neuropathy among patients with auditory neuropathy spectrum disorder .
Noninvasive Diagnostic Techniques in Determination of Site of Lesion of Auditory Neuropathy Spectrum...
Auditory Neuropathycross-sectional study of population who have ANSD
Rare Disease Patient Registry & Natural History Study - Coordination of Rare Diseases at Sanford...
Rare DisordersUndiagnosed Disorders316 moreCoRDS, or the Coordination of Rare Diseases at Sanford, is based at Sanford Research in Sioux Falls, South Dakota. It provides researchers with a centralized, international patient registry for all rare diseases. This program allows patients and researchers to connect as easily as possible to help advance treatments and cures for rare diseases. The CoRDS team works with patient advocacy groups, individuals and researchers to help in the advancement of research in over 7,000 rare diseases. The registry is free for patients to enroll and researchers to access. Visit sanfordresearch.org/CoRDS to enroll.
Assessment of Neuropathic Pain
Spinal Cord Injury + Peripheral Nerve DiseasesDevelopment of neuropathic pain is one of the most disabling sequels after spinal cord injury (SCI) and in peripheral nerve diseases. The functionality of the pain pathway in humans as well as its plastic changes following SCI can be assessed in vivo by surface electrophysiological recordings and functional magnetic resonance imaging after noxious heat stimulation of the skin. Aims: a) establishing a clinically applicable assessment of the pain pathway and characterizing its changes as a consequence of SCI and in peripheral nerve diseases in an objective manner, b) characterizing plastic changes in the pain pathway in SCI patients with neuropathic pain and in patients with peripheral nerve diseases and relating them to the development of neuropathic pain syndromes.
Depolarising Electrical Skin Stimulation in Neuropathic and Postoperative Pain
Neuropathy;PeripheralThe aim of the study is to evaluate voltage gated sodium channel (NaV1.7) dependent pain phenomena in neuropathic pain and chronic postoperative pain.
Diabetes, Falls, and Fractures
Diabetic Peripheral NeuropathyDiabetes7 moreComparing severity of diabetic peripheral neuropathy (small and large fibers including autonomic neuroapthy) to postural control and vestibular measurements
Diagnostic Accuracy of Quantitative Neuropad Test for Diabetic Peripheral Neuropathy
Diabetic Peripheral NeuropathyThis study aimed to evaluate the diagnostic vaule of quantitative Neuropad for diabetic peripheral neuropathy
Immunoglobulin M (IgM)-Anti-myelin-associated-glycoprotein(MAG) Peripheral Neuropathy Study
Immunoglobin M Anti-myelin-associated-glycoprotein Peripheral NeuropathyThere is no international consensus regarding how to assess and treat patients with immunoglobulin M (IgM)-anti-myelin-associated-glycoprotein (MAG) monoclonal gammopathy associated peripheral neuropathy. The purpose of the IMAGiNe study-Lyon/Bicêtre is to prospectively collect standardized clinical data of 25 patients with IgM-anti-MAG monoclonal gammopathy associated peripheral neuropathy in order to participate in the international IMAGiNe Study. The main objective is to describe in detail the various clinical subtypes, clinical course, treatment responses, antibody titers of IgM-anti-MAG monoclonal gammopathy associated peripheral neuropathy using a variety of outcome measures. The obtained observational data will allow the construction of an IgM monoclonal gammopathy associated peripheral neuropathy-specific Rasch-built overall Disability Scale (RODS) that should fulfill all modern clinimetric requirements, including cross-cultural validity. The coordinating centers for the IgM-RODS construction will be the Maastricht University Medical Center and University Medical Center Utrecht, both in the Netherlands.
Physiologic Measure of VIPN
Chemotherapy-induced Peripheral NeuropathyThe purpose of this study is the development of a physiologic endpoint using a novel technology that would provide an objective, easy to use and more sensitive assessment of VIPN in children and adolescents. The ability to more easily detect and monitor VIPN, even before it is clinically evident, would facilitate optimizing the dosing of vincristine for maximal disease response while minimizing the risk of lifelong functional deficits affecting quality of life. This approach would also enable the development of specific therapies to minimize or eliminate the occurrence of VIPN in children and adolescents. This is a single site study that aims to develop a novel device to evaluate and characterize vincristine-induced neuropathic pain. The investigators will enroll patients with ALL following the Delayed Intensification (DI) phase of treatment. At each study visit, the investigators will evaluate the nPRD as well as the TNS-PV. The nPRD will inform the neuropathy index which will be used to compare to the TNS-PV. We anticipate a correlation between the two.
Risk Prediction of Taxane Chemotherapy-Induced Peripheral Neuropathy
Breast CancerChemotherapy-induced Peripheral Neuropathy1 moreThis is an observational study to discover risk factors of chemotherapy-induced peripheral neuropathy (CIPN) in 350 patients with early stage breast cancer undergoing taxane-based chemotherapy at two main sites (University of North Carolina at Chapel Hill (UNC) Hospital, including Rex Hospital, and the University of Alabama at Birmingham (UAB) Hospital). The primary purpose of this study to explore patient- and procedure-based variables that identify patients at risk for developing CIPN during chemotherapy.