Myotonic dystrophy (DM) is the most common form of muscular dystrophy and is caused by expansion of a CTG trinucleotide repeat on human chromosome 19. Patients with DM develop atrioventricular conduction disturbances, the principal cardiac manifestation of this disease. The etiology of the pathophysiological changes observed in DM has yet to be resolved. Haploinsufficiency of myotonic dystrophy...

There is much savings involved in the prevention of heat loss. Many structures exhibit such loss. Much can be done to improve or minimize the heat loss in a structure. These include interior and exterior modifications. It has been shown that heat can move by means of convection, conduction, and radiation. Problems with heat loss can be due to moisture, and poor construction techniques. There is...

Mutations in SCN5A, the gene encoding the pore-forming subunit of cardiac Na(+) channels, cause a spectrum of arrhythmic syndromes. Of these, sinoatrial node (SAN) dysfunction occurs in patients with both loss- and gain-of-function SCN5A mutations. We explored for corresponding alterations in SAN function and intracardiac conduction and clarified possible mechanisms underlying these in an estab...

Semi-implantable transcutaneous bone conduction devices are treatment options for conductive and mixed hearing loss (CHL/MHL). For counseling of patients, realistic simulation of the functional result is desirable. This study compared speech recognition in noise with a semi-implantable transcutaneous bone conduction device to external stimulation with a bone conduction device fixed by a headban...

Although measurements of nerve conduction velocity in man were first made nearly a hundred years ago, the procedure was not used clinically until 1948 when Hodes, Larrabee, and German reported reduced conduction velocity in regenerating motor nerves. Later, Hodes (1949) described slow conduction in surviving nerve fibres after poliomyelitis, and more recently it has been shown that conduction m...

Peripheral nervous system abnormalities, including neuropathy, have been reported in people with cystic fibrosis. These abnormalities have largely been attributed to secondary manifestations of the disease. We tested the hypothesis that disruption of the cystic fibrosis transmembrane conductance regulator (CFTR) gene directly influences nervous system function by studying newborn CFTR(-/-) pigs...

To gain insight into the mechanisms of reperfusion arrhythmias, we studied ventricular automaticity and conduction characteristics in 35 dogs during 10 minutes of left anterior descending coronary artery (LAD) occlusion and subsequent reperfusion. The frequency of ectopic activity reached a maximum within the first 6-8 minutes after LAD occlusion and then declined, leading to an ectopia-free qu...

BACKGROUND bone conduction auditory brainstem responses (ABR) in normal hearing individuals. AIM to evaluate the clinical applicability of bone conduction ABR, characterizing normality and determining an assessment protocol. METHOD participants of this study were 22 individuals with normal hearing (20dB NA), with ages between 20 and 30 years, 14 female and 8 male. All individuals were asses...

BACKGROUND Use/misuse of the opioid combination hydrocodone-acetaminophen has been associated with permanent hearing loss. Although reports have been rare, this potential effect can have significant detrimental effect on patients' overall quality of life. To date, the ototoxic effect of hydrocodone alone has not been systematically investigated. OBJECTIVE In this report, we aimed to evaluate ...

Multifocal acquired demyelinating sensory and motor (MADSAM) neuropathy is characterized by an asymmetric multifocal pattern of motor and sensory loss, and conduction block and other features of demyelination in nerve conduction studies. MADSAM neuropathy needs to be differentiated from chronic inflammatory demyelinating polyneuropathy (CIDP) and multifocal motor neuropathy (MMN). In classic CI...