Help For Hearing Loss Sufferers

Hearing Sense Recovery

This product was created by Mark after he had gone through an experienceof getting his wife's hearing back. He was contacted by one of the most brilliant researchers on the topic of getting one's hearing back and he was able to conduct a research with him in the African tribes in Kenya, the superfoods they have used have made them become the country with the least hearing loss in the world. They have made an experiment on Mark's wife who was able to get her hearing back in merely three weeks. After that, Mark has decided to put the information out to the world so they can help their hearing and the hearing of their loved ones to finally experience life as it truly should. He put the information into an easy step by step guide that walks you through every single phase you have to go through to improve the hearing. The method is very easy to attain and it does not need any technical knowledge, you can easily get this product and you will be able to get your hearing back in just three weeks. The method that is used is also natural and safe and it definitely works no matter how bad the hearing condition is, it will heal and strengthen the cells responsible for hearing. Read more...

Hearing Sense Recovery Summary

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Effects of Congenital Deafness on Vision

Studies of visual functions after early genetic deafness indicate enhanced visual processing, at least for visual motion, visual attention, and peripheral vision. For example, deaf adults are better than hearing controls at detecting the onset or the direction of motion of a peripheral stimulus. They are also faster at switching visual attention toward a near-periphery target in the presence of distractors located at the fixation point. Electrophysiological recordings while subjects monitored moving stimuli have indicated larger visually evoked responses for deaf than hearing adults over occipital and temporal sites. These group differences were especially marked for peripheral stimuli. In an fMRI study, the effects of visual attention on motion The functions altered in the deaf, i.e., motion, visual attention, and peripheral processing, share the property of being mediated predominantly by the dorsal visual pathway that projects from V1 to the motion area (MT-MST) and the parietal...

Cortical Deafness

Corresponding observations in human cases of so-called cortical deafness were also made in the early 20th century patients with apparently complete loss of the ability to identify or discriminate sounds were described, but these individuals were usually not deficient in detecting the presence of sound. These dissociations have become much better understood in the past two decades. The concept of cortical deafness has been refined and usually refers to patients who suffer damage to auditory cortices in both hemispheres and who have profound difficulties in identifying sounds or recognizing speech. These patients usually have only mild or no threshold detection deficits, in accord with animal studies, and may also show no consistent disturbance in simple discrimination tasks for frequency or intensity. These phenomena indicate a functional dissociation since the inability to identify sounds is independent of the ability to detect the presence of sound. Thus, these cases support the idea...

Acquired deafness

Acquired deafness occurs with damage to the eighth nerve or its nuclei or sensory apparatus. In most cases vestibular signs are also seen as the vestibular branch of the nerve is also affected. Otitis media and interna can be accompanied by unilateral deafness, but auditory defects are less common. Tumours, trauma, degenerative disease and toxins (aminoglycoside antibotics) can all cause deafness, but acquired deafness due to a specific cause is rarely diag nosed. Assessment is also difficult, but measurement of brainstem auditory-evoked potentials can provide a quantitative measurement of hearing loss. However, for investigation subjective assessment by the owners is frequendy the only available assessment.

Ventricular Tachyarrhythmias

The long QT syndrome, in which the corrected QT interval is pathologically prolonged, is also associated with SCD. 10 Prolongation of the corrected QT interval probably represents dispersion in ventricular repolarization and can be congenital (with or without nerve deafness) or acquired (due to hypokalemia, hypomagnesemia, hypocalcemia, anorexia, ischemia, central nervous system pathology, terfenadine-ketoconazole combinations, or certain antipsychotic or antiarrhythmic drugs). The corrected QT interval can be calculated easily by the following formula

The Body of Difference

Physical difference and disability with great originality, by challenging conventional ideas about the body and about what being normal means. For example, Ablon (1984) has pointed out that the dwarf body is different but not disabled dwarfs experience their bodies as complete and normal. G. Frank (1986, 2000), in her phenomeno-logical life history study of a woman who was born without arms or legs, questions a view of this woman as missing body parts and describes how she asserts her fundamental normalcy. Other phenomenological work that addresses difference or disability include blindness (Ainlay, 1989), deafness (G. Becker, 1980 Preston, 1994), post-polio syndrome (Kaufert & Locker, 1990 Scheer & Luborsky, 1991), mobility impairments (Luborsky, 1995 Murphy, 1987), and disability more generally (Luborsky, 1994 Zola, 1982).

Introduction Clinical Setting

Classical Alport's syndrome is an X-linked disease and is the most common form of Alport's syndrome (90 of patients), with an overall incidence of Alport's syndrome in the United States of 1 5000 to 1 10,000 (1-4). Patients show hematuria in childhood with progressive hearing loss in one third, and ocular defects and progression to renal failure in 30 to 40 by early adulthood. Anterior lenticonus is the most common eye defect.

Vascular Disease Malformations and Flow Abnormalities

Kawasaki disease is a multisystem disorder characterized by vasculitis of small and medium arteries. Neurologic complications, including meningoencephalitis, monocyte-predominant pleocytosis in the CSF, facial nerve palsy, seizures, hemiplegia, and sensorineural hearing loss have been reported to occur in 1.1 to 3.7 of affected children.103105 Two papers detailing seven cases of SDH in children as young as 6 months have been reported.106 107 The SDH in these cases was hypothesized to be caused by vasculitis of dural vessels. All cases reported to date have been in living subjects with no fatalities or for whom postmortem findings had been reported.

Dysmorphology databases

Despite the problems outlined above, dysmorphology databases have a major clinical function in aiding doctors seeking a differential diagnosis for a patient presenting with a given array of signs. They can provide a list of all conditions with a combination, for example, of mental handicap, deafness and retinal dystrophy. The emphasis tends to be on a differential diagnosis, 'a manageable list of possible diagnoses', and although this might not seem as desirable as an exact diagnosis, searching a database for an exact diagnosis may lead to the correct condition being missed. This is because many unique features, or 'handles', will need to be entered in order to retrieve only one possibility, and for this to happen other possibilities will be excluded. In clinical dysmorphology too many syndromes overlap and it is better to view a short list of the possible diagnoses and then reject those that seem on inspection not to fit. Features combined with an inheritance pattern, e.g. any...

Outer and Middle Ears

The Nervous System The Ear

Ear is a fluid-filled space containing neural structures and their support. These inner ear structures offer significant impedance to the transmission of vibration from the air-filled outer ear to the inner ear. The resonance of the outer ear and the increases provided by the ossicular chain provide an impedance match between air and the fluids and structures of the inner ear so that over a significant portion of the audible range of hearing, changes in air pressure impinging on the auditory system are efficiently transmitted to the inner ear with no loss in sound level. Damage to the ossicular chain leads to significant hearing loss because ofthe loss ofthis crucial impedance matching function.

Adams Zone Theory See Color Vision Theorieslaws Of

The term adaptation derives from the Latin word adaptare, meaning to fit, and has a variety of meanings in science. In biology, adaptation refers to structural or behavioral changes of an organism, or part of an organism, that fit it more perfectly for the environmental conditions in which it lives where the changes have evolutionary survival value. In physiology, adaptation is the change or adjustment of a sense organ to some incoming stimulation, and the term sensory adaptation (also called negative adaptation) involves a decreased sensitivity to stimuli due to prolonged stimulation. In psychology, adaptation is the change in quality, clarity, or intensity of a sensory experience that occurs with continuous and unchanged stimulation. In psychology, in particular, adaptation may be discussed in a multitude of contexts, among which are auditory adaptation, visual adaptation, olfactory adaptation, pain adaptation, cutaneous adaptation, and gustatory...

The Evolution of Disease

In addition to recasting the ailments of old age as evolutionary byproducts, Darwinian medicine also interprets health problems of youth, for example, neonatal jaundice, through an adaptive framework. Neonatal jaundice involves excessive bilirubin levels in the blood, indicating inadequate clearance of red blood cells and other proteins and slowed elimination of waste products through the intestinal tract. Elevated bilirubin can be toxic in the brain, leading to disability, hearing loss, or death (Brett & Niermeyer, 1999, p. 8).

Ophthalmic Surgery Cataract Surgery

Cataracts may be congenital, traumatic, steroid- or radiation-induced, or degenerative. In degenerative cataracts there will also be other medical conditions of the ageing population. While diabetics have no more cataracts than the general population, they tend to present earlier and so there seems to be a preponderance of diabetic patients presenting for cataract surgery. Steroid induced cataracts present in patients taking long term steroids for other conditions, particularly eczema or asthma which should be taken into account. Cataract surgery demands a still eye with low intra-ocular pressure. This can usually be achieved by smooth anaesthesia with muscle relaxation and IPPV to achieve mild hypocapnia, whether via a tracheal tube or laryngeal mask, though the latter is preferable because of the lack of intubation pressor response or laryngeal spasm and coughing on extubation. There is a fashion for local anaesthesia for cataract surgery despite this having a higher failure rate,...

Hidden Problems in Stoma Care

As the proportion of people aged over 65 continues to grow, the proportion of elderly patients with stomas continues to grow (Ebersole and Hess, 1998). Currently, in the UK the average life expectancy is 83 years for men and 87 years for women. Certain areas of the UK, especially on the south coast of England, have high populations of people over the age of 70 years - as high as 27.9 . In 2000, 10.6 of the population was over the age of 80 (Black, 2000). For many of these patients, there are already other long-standing physical problems and cognitive problems which are going to be complicated further by stoma surgery. For those patients needing to learn self-care for their new stoma, individual assessment of the patient's psychological and cognitive function will be needed to assess how teaching strategies can be adapted. Fine and gross impairment in motor skills will complicate package opening, skin cleansing and appliance application. Visual and hearing impairments make it difficult...

Mumps And Other Viral Parotitis

Usually benign in children, mumps can be severe in adults. Although 25 percent of men suffer orchitis, only 5 percent of women manifest oophoritis. 19 Possible complications include mastitis, pancreatitis, myocarditis, meningoencephalitis, encephalomyelitis, and sensorineural deafness. 1 l8

Thiazides and Loop Diuretics

The toxicity associated with thiazides and loop diuretics involves two basic processes volume contraction and electrolyte derangements. Symptoms associated with intravascular volume depletion include hypotension, tachycardia, and altered mental status. Common electrolyte derangements include hyponatremia, hypokalemia, hypocalcemia, hypomagnesemia, and hypochloremic metabolic alkalosis. Other adverse reactions from thiazides and loop diuretics may include rash, pruritis, hearing loss, leukopenia, and thrombocytopenia.

Molecular Motors In The Nervous System

FIGURE 2.10 Examples of myosin motor proteins found in the mammalian nervous system. Myosin heavy chains contain the motor domain, whereas the light chains serve to regulate motor function. Myosin II was the first molecular motor characterized biochemically from skeletal muscle and brain. Biochemical and genetic approaches have now defined 11 classes of myosin, many of which can be found in brain. Myosin II is a classic two-headed myosin that forms thick filaments in nonmuscle cells. Myosin I motors have single motor domains, but may interact with actin microfilaments or membranes. Myosin V motors were initially identified as a mouse mutation that affected coat color and produced seizures. Myosin V has multiple binding sites for calmodulin that act as light chains. Mutations in other classes of myosin have been linked to deafness. Other myosins, including myosins I, II, and V, have been detected in growth cones as well as mature neurons. The specific roles of these various myosins in...

Libby Kumin PhD Cccslp

The most common risk factors that we see in infants and toddlers with Down syndrome that directly influence speech and language development are low muscle tone in the oral motor area, including the lips, tongue, and jaw (Kumin and Bahr, 1999), relative macroglossia (Desai, 1997), and otitis media with effusion (Roizen et al., 1992) resulting in fluctuating hearing loss. Because there is no one communication profile, there is no one treatment plan. Treatment should be individually designed to meet all of the communication needs of the child.

Central Auditory Pathways

Auditory Pathway Action Potential Nerves

Ipsilateral and contralateral superior olivary nuclei. Axons from cells in the superior olive travel along with other sensory fibers within the lateral lemniscus to reach the inferior colliculus. Collicular fibers synapse in the medial geniculate nucleus of the thalamus. Geniculate fibers represent the final projection to the primary auditory cortex located along the superior lip of the temporal lobe. Auditory fibers are represented bilaterally within the central pathway because of partial decussation of projections at several of the relay points within the brain stem. Thus, central auditory lesions rarely cause deafness in only one ear.

Teratogens and human malformations

Microcephaly Intrauterine growth retardation, skin discoloration Multiple malformations craniofacial, CNS, cardiac, thymic aplasia Deafness Tooth and bone discoloration Phocomelia, external ear defects, oesophageal and duodenal atresia, tetralogy of Fallot, renal agenesis Cleft palate and other craniofacial defects, cardiac defects

Temporal Lobe Lesions Impair Acoustic Processing

With these caveats, it is clear that apart from speech function, which strongly lateralizes to one (usually the left) hemisphere, basic auditory functions are subserved by both hemispheres. However, there does appear to be a degree of lateralization of musical processing to the right hemisphere. Bilateral lesions approximately confined to the core auditory areas are reported to result in transient auditory agnosia, which is a lack of awareness of auditory stimuli combined with abnormal pure tone thresholds in the absence of peripheral or brain stem damage (sometimes referred to as cortical deafness). With time, this condition may evolve into auditory agnosia for speech (pure word deafness), whereby patients regain near normal auditory acuity but remain impaired in their ability to interpret speech sounds. It is hypothesized that speech decoding requires more precise temporal analysis than is necessary for the detection and identification of most nonverbal sounds. This capacity to...

History of Food Folklore

Curative properties have also been ascribed to many foods for thousands of years. In ancient Rome, cabbage was considered the perfect medicinal plant and was prescribed frequently for a wide range of ailments including warts, deafness, and drunkenness. Apples, herbs, garlic, honey, milk, peppers, and many other foods were also highly regarded in ancient cultures for their therapeutic qualities. The prescription of foods as medicines was not necessarily based on scientific fact but instead was often based on early medical theories or magic. The ancient Greeks believed that the body was composed of four humors blood (hot and moist), phlegm (cold and moist), yellow bile (hot and dry), and black bile (cold and dry). Health was thought to result from a balance of the humors, and illness resulted from an imbalance. To counteract imbalances and restore health, physicians often prescribed specific foods, based on their perceived degree of 'heat' and 'moisture'. For example, fever, a 'hot'...

Manifestations of Lead Toxicity

Neurologic Full-blown lead encephalopathy, including delirium, truncal ataxia, hyperirritability, altered vision, lethargy, vomiting, and coma, is not common. Although peripheral nerve damage and paralysis may still be reported in adults, the most common toxicity observed is learning disability and an associated high-frequency hearing loss occurring in children with blood lead levels previously assumed to be safe. At low blood levels of lead (less than 10 mgdl-1), children may lose IQ points, possibly due to the interference of lead in normal calcium signaling in neurons and possibly by blocking the recently reported learning-induced activation of calcium phospholipid-dependent protein kinase C in the hippocampus. Lead has also produced necrosis of retinal photo-receptor cells and swelling of the endothelial lining of retinal blood vessels in rats. Lead may also damage the auditory nerves in rats, and it may be partially responsible for the high-frequency hearing loss observed in...

Chronic suppurative otitis media

Chronic suppurative otitis media (CSOM) is the commonest form of chronic otitis media. Clinically it is characterized by otorrhoea and conduction hearing loss of variable severity. Otoscopy reveals a perforated eardrum. The condition is classified into the safe (tubotympanic) and unsafe (atti-coantral) variety depending on the likelihood of coexisting cholesteatoma.

Central and Peripheral Nervous System

Age-related hearing loss is a feature of biological aging. It affects the cochlear neural structures and leads to loss of acuity, especially for higher pitched tones. It is speculated that apoptosis of the most vital neural cells drives this hearing loss, based on mutations in the mitochondria due to life-long free-radical stress.

Betweenmodality Plasticity

Whereas anecdotal evidence of better audition after early blindness or better vision after early deafness exists, the available data are quite mixed. The longheld belief that multisensory integration is a necessary step in optimal development led investigators to focus initially on the disabilities caused by early blindness or deafness. For example, a number of studies, mostly from the 1970s and the early 1980s, document deficient spatial abilities in the blind and deficient visual perception in the deaf. The realization of the adaptability of the brain led investigators to carefully review this issue. It is now evident that when the etiology and characteristics of the population tested are carefully controlled and the task is appropriately chosen not to rely on encoding strategies that are not available to the deprived subjects, convincing evidence of compensatory plasticity can be established.

Nasopharyngeal cancer

The first sign of NPC is often an enlarged metastatic cervical node in the posterior triangle. Common local signs and symptoms include nasal (blood-stained discharge, obstruction), aural (serous otitis media, tinnitis, conductive hearing loss) and neurological symptoms (diplopia due to abducen nerve paralysis). Diagnosis is by flexible fibreoptic nasopharyn-goscopy and biopsy. Elevated blood levels of antibodies to Epstein-Barr virus capsid antigen (IgA-VCA) and early antigen (IgA-EA) are often seen. CT and MRI are useful in staging the disease and in detection of recurrence. Radiation is the firstline treatment for NPC of all stages because of the radiosensi-tivity of undifferentiated carcinoma. For recurrent disease after radiotherapy, surgical resection of the nasopharynx by the transoropalatal approach, mandibular swing or maxilla swing approach are recently established surgical salvage procedures that are preferred over re-irradiation which is associated with complications...

Rubella Congenital Syndrome

The congenital syndrome of rubella is an illness of infancy resulting from rubella virus infection in utero. Infants present with signs and symptoms from the following categories (1) cataracts congenital glaucoma, congenital heart disease, hearing loss, and pigmentary retinopathy or (2) purpura, splenomegaly, jaundice, microcephaly, mental retardation, meningoencephalitis, and radiolucent bone disease. Laboratory detection is undertaken by any of the following methods (1) isolation of rubella virus, (2) demonstration of rubella-specific IgM, or (3) infant rubella antibody levels persistently high for longer than expected from passive maternal antibody transfer. Infant antibodies should decrease by a twofold dilution each month of life.

Food Borne Infections during Pregnancy

Toxoplasma gondii is a parasite that can be transmitted to the fetus in utero through transpla-cental transmission, causing stillbirth, miscarriage, or mental retardation. The parasite has been found in raw, inadequately cooked or cured meat, cat feces, and unwashed raw fruit and vegetables. It has also occasionally been reported in unpasteur-ized goat milk. In the United Kingdom, toxoplasmosis occurs in approximately 2.5-5.5 in 1000 pregnant women (1750-2850 cases per year), generally causing flu-like symptoms, swollen lymph glands, or muscle aches and pains that last for a few days to several weeks. If a pregnant woman contracts the infection, there is an approximately 30-40 chance of fetal infection (congenital toxo-plasmosis). Infants who became infected before birth may develop growth problems, vision and hearing loss, hydrocephalus, brain damage, epilepsy, and other problems. In Europe, congenital toxoplasmosis affects between 1 and 10 in 10 000 newborns, of whom 1 or 2 develop...

Hyperviscosity Syndrome

Fatigue, headache, anorexia, and somnolence are early nonspecific symptoms. As blood flow slows, microthromboses may occur, with the advent of local symptoms such as deafness, visual disturbances, and jacksonian or generalized seizures. The diagnosis of hyperviscosity must be considered in the emergency department when patients with unexplained stupor or coma are found to have anemia, with rouleau formation on the peripheral blood smear. The most readily appreciated physical findings are in the ocular fundi and include sausage-linked retinal vessels, hemorrhages, and exudates. Laboratory evaluation should include coagulation, renal, and electrolyte profiles. Hypercalcemia can coincide, and when M-component protein concentrations are high, factitious hyponatremia may also be present. A clue to the presence of hyperviscosity may be the laboratory's inability to perform chemical tests because of the serum stasis in the analyzers, undoubtedly due to too thick blood. Serum viscosity and...

Prolonged QT Syndrome

This is searched for more than any other etiology as a cause of syncope in young healthy children. In nearly all cases, the syndrome is inherited and is associated with deafness in about 75 percent of cases. Secondary, noncongenital cases result from anorexia, bulimia, or chronic ingestion of antidysrhythmic medications. Undiagnosed, untreated, or undertreated congenital prolonged QT syndrome results in a mortality rate in excess of 90 percent.

Otitis media with effusion

Otitis media with effusion (OME) is a condition with complex etiologies including anatomical variations, allergy, infections and inflammation. The interplay of these factors lead finally to structural and or functional abnormality of the Eustachian tube resulting in OME. The more horizontal lie of the Eustachian tube and frequent attacks of URTI contribute to the high prevalence of OME in infants and young children of any race. The reported cumulative incidence of first episode of OME reaches almost 100 by the age of 3 years. The incidence drops sharply after the age of 7 so much so that the condition is uncommon amongst teenagers and rare in adults. However, in places where nasopharyngeal carcinoma (NPC) is endemic, deafness associated with OME is a common presenting symptom of the disease. In these areas, NPC should be excluded in any adult with unilateral OME.

Prevalence And Clinical Diversity

Sclerae, joint laxity, dentinogenesis imperfecta, easy bruising, and hearing loss. OI represents extreme variation in severity from lethal perinatal to barely detectable. These clinical features, along with radiological and genetic criteria, were used to separate four major clinical types of OI. 1 The patients affected with the mildest (type I) OI have blue sclerae, osteopenia, slight growth retardation or normal height, and premature deafness dentinogenesis imperfecta is less common. Fractures are not commonly observed at birth, but begin with am-bulation and decrease following puberty. Type II is the lethal perinatal form infants exhibit multiple intra-uterine fractures of the ribs and long bones. They have a relatively large and soft cranium, short limbs, and a narrow thoracic cavity death usually results from pulmonary insufficiency. Type III is the progressively deforming form with multiple fractures, short stature, and severe dentinogenesis imperfecta. Children with type III may...

Etiology Pathogenesis

Each Alport kindred reported thus far has presented its own unique mutation. More than 300 mutations in the COL4A5 gene have been identified (4). The rate of progression to end stage and deafness in hemizygous affected males are mutation dependent. Large deletions, nonsense mutations, or mutations that changed the reading frame were associated with 90 risk of end-stage renal disease before age 30 in affected males with X-linked Alport's, with only 50 risk for patients with missense and 70 risk for those with splice site mutations. Risk for hearing loss before age 30 was 60 in patients with missense mutations, versus 90 risk for all other mutations (16). Ultrastructural features do not strictly correlate with type of mutation, in that some patients with major gene rearrangements had no significant lesions, and varying ultrastructural abnormalities were present even within the same kindred (2).

Neurological Substrate Of The Disorder

The damage responsible for these disorders is usually caused by cerebrovascular disease, usually embolic stroke, that involves the midportion of the first temporal gyrus bilaterally (at least one case has been reportedly caused by a hemorrhage in an auditory structure in the brain stem, the inferior colliculi). If caused by unilateral damage, the lesion is usually deep in the posterior temporal lobe of the hemisphere dominant for speech (usually the left hemisphere). In such cases, there may be signs that any residual abilities that are present depend on right hemispheric linguistic abilities. For example, the person may report hearing a word that is semantically related to the actual word spoken (horse for pony). Some authors have suggested that damage to the primary auditory cortex produces pure word deafness (agnosia for spoken language only), whereas damage to the auditory association cortex produces difficulties with nonspeech sounds. Damage to both regions would produce global...

Other Mutations In The 12S rRna Gene

The T1095C mutation was found in two Italian families. 19,32 In one family, the proband had Parkinson's disease, neuropathy, and a history of AID. 32 The second family with matrilineal inheritance of hearing loss included two maternal relatives of the proband who had histories of AID. 19 More data are needed to draw a firm conclusion about the role of this mutation in AID.

Plasticity And Reorganization

In cases of complete deafness, on the other hand, there is evidence from both human and animal studies that auditory cortical regions may respond to visual cues, although the nature ofthe processes involved has not been worked out. However, it has been shown in some animal species that when optic nerve inputs are rerouted to the medial geniculate nucleus during early phases of development, auditory cortical areas begin to show some of the structural and functional characteristics of visual cortex, implying that these features are driven by the nature of the stimulation that is received rather than by epigenetic factors exclusively.

Treatment Options and Continuing Research

Blood transfusions have been a common therapy for severe thalassemia, but transfusions do not cure the disease, and frequent transfusions can cause iron overload, an illness caused by excessively high levels of iron. A drug, called an iron chelator, may be given to bind with the excess iron. Iron chelators can produce additional side effects, such as hearing loss and reduced growth.

Convergent Evolution

Legitimately or validly from the given premises. In the contexts of Freudian analysis, clinical psychology, and psychotherapy, the notion of conversion (e.g., conversion hysteria) refers to the transformation or translation of psychic conflicts or psychological problems into physical symptoms such as apparent paralysis, blindness, deafness, or anaesthesia. See also FREUD'S THEORY OF PERSONALITY GESTALT THEORY LAWS MIND MEN-TAL SET, LAW OF NULL HYPOTHESIS PERCEPTION (II. COMPARATIVE APPRAISAL), THEORIES OF. REFERENCES

Wheatstonepanum Limiting Case See Panum Phenomenoneffect

Named after the French physician Marie-Pierre Jean Flourens (1794-1867), states that thinking depends on the functioning of the cerebrum as a whole the laws of thought - refer to the three logical principles of identity, contradiction, and excluded middle, and are considered to be the basic principles of all reasoning and the law of participation - is a principle of human primitive thinking, which asserts that things that are similar are considered to be identical . According to Watson, what psychologists call thought is nothing but talking to oneself (cf., the motor theory of speech perception propounded by A. M. Liberman, which holds that speech is assumed to be perceived by an implicit, covert system that maps the acoustic properties of the input against a set of deep motor representations of idealized articulation). However, Watson's extreme behaviorist view that thinking or thought depends only on the implicit muscle movements of speech has proven to be inadequate cf., central...

Autosomal Dominant Inheritance

There are hundreds of medical conditions due to autosomal dominant alleles, most of them very rare. They include neurodegenerative disorders such as Huntington's disease, a variety of deafness syndromes, and metabolic disorders such as familial hypercholesterolemia (affecting blood cholesterol levels) and variegate porphyria (affecting the oxygen-carrying porphyrin molecule). Table 1 lists some other examples.

Membranedamaging toxins

SH-activated cytolysins A family of thiol-activated cytolysins exists produced by at least 17 bacterial species belonging to several genera. Sterol is the primary target in susceptible membranes to which such proteins, initially secreted as water-soluble entities, bind. Arc ring complexes are formed in membranes which do not contain cholesterol, are extract-able, and comprise conformationally altered proteins which will bind nonspecifically to other membrane lipids sterol is only a primary anchor. Gross amino acid compositions of these proteins differ widely but serological cross-reactivity indicates at least some conserved epitopes. The majority are known not to be primary determinants of virulence. However, streptolysin-O (Streptococcus pyogenes) and listeri-olysin (Listeria monocytogenes) are definitely produced in vivo and damage kill neutrophils, causing their granules to discharge their degradative enzymes to kill the pathogen (or harm the host). Listeriolysin is also important...

Clinical Features

The clinical effects of mercury poisoning depend on the form and, in some cases, the route of administration. In general, the neurologic, gastrointestinal, and renal systems are predominantly affected. The short-chained alkyl compounds, methyl, dimethyl, and ethyl mercury, have the most devastating effects on the CNS, 24 followed by elemental mercury, whose primary toxicity is neurologic. Both forms of mercury produce erethism, a constellation of neuropsychiatric abnormalities including anxiety, depression, irritability, mania, sleep disturbances, excessive shyness, and memory loss. Tremor, either intention or nonintention, is a common physical finding.25 The short-chained alkyls produce paresthesias (early sign), ataxia, muscular rigidity or spasticity, and visual and hearing impairment and induce CNS teratogenic effects. Gastrointestinal effects of both elemental and short-chained alkyl compounds are mild. In cases of severe, chronic poisoning with elemental mercury, stomatitis,...

Synergistic Interactions

Brummett has pointed out the synergistic interaction between loop diuretics and aminoglycosides. This combination often produces permanent deafness, especially in patients with renal failure. The acute loss of hearing may occur within 30 min to 2 hr after intravenous administration of diuretics, in a patient on aminoglycosides. This interaction may be specific of loop diuretics and does not hold for other diuretics such as mannitol, hydrochlorothiazide, or mercurials. There is also a synergistic toxicity when loop diuretics are given with cis-platinum. This is especially important because loop diuretics are recommended to produce a diuresis to prevent cis-platinum nephrotoxicity.

Mechanical Motion in the Inner Ear in Response to Sound

Which feeds energy back into the organ of Corti and alters the mechanical properties of the cochlear partition and possibly the transduction process. This active process may be controlled, in part, by feedback via olivocochlear axons originating in the auditory brain stem and profusely terminating at the base of OHCs. Apparently as the result of an active process, the organ of Corti acts not only to receive sound but also to generate it, as an otoacoustic emission (OAC) recorded by a microphone in the ear canal. There are several categories of OACs, reflecting perhaps more than one nonlinear active process in the cochlea. OACs are proving useful as an objective tool for diagnosing sensorineural hearing loss.

Nuclear Genes in Mitochondrial Function and Biogenesis

A growing list of human pathologies has been related to mitochondrial defects and the responsible mutations mapped to RNA and protein-coding genes of mtDNA. Such mutations manifest a wide range of clinical pheno-types including encephalomyopathies, cardiomyopathy, deafness, diabetes, optic neuropathy, and dystonia. Similar presentations are elicited by mutations in nuclear DNA that affect mitochondrial metabolism either directly through lesions in the structural genes for enzymes of the TCA cycle, fatty acid oxidation, and mtDNA integrity or indirectly by interfering with metabolite and protein import, assembly of respiratory chain complexes, metal homeostasis, and protein turnover. In view of their central role in oxidative metabolism, the list of currently known mitochondrially related diseases will undoubtedly be increased in the future.

C1494T Mutation In The 12S rRna Gene

The homoplasmic C1494T mutation was recently identified in a large Chinese family with AID and maternal inheritance.1-14-1 Twenty of 39 matrilineal relatives, who are supposed to carry the mutation, exhibited hearing loss. Clinical data have shown that the treatment with amino-glycosides can induce or worsen deafness in matrilineal relatives. The mutation occurs at a position where the A at position 1555 can make a pair with the mutant base (Fig. 1D). Additional data indicated that the cell lines derived from carriers showed a lower rate of total oxygen consumption and a significant increase in doubling time with the high concentration of aminoglycosides. Nuclear background probably plays a role in the deafness phe-notype and AID with the C1494T mutation. 14

Functional Birth Defects

Another common functional genetic disorder is hearing loss, which can result from a defect in any one of more than fifty different genes. One gene, connexin 26, may be responsible for a large portion of inherited hearing loss. Some forms of congenital hearing loss may be due to prenatal exposure to infectious agents such as rubella. Genetic screening or screening for hearing loss at birth may be the most important test for hearing impairment yet to be developed, as early recognition and treatment can lead to dramatic improvements in hearing and, consequently, in the development of language in early childhood.

Transduction Process in Cochlear Hair Cells

Inner ear structures are easily damaged by intense sound, drugs, viruses, and bacteria, and there are genetic causes of inner ear malformation. The resulting hearing loss is called sensorineural, and in such cases no treatment has been found to fully restore normal inner ear function. Some functional hearing may be restored, however, by electrically stimulating surviving spiral ganglion neurons through a cochlear prosthesis.

Differential Diagnosis

There is increasing evidence that many adult-onset focal dystonias are genetically based. At this time, molecular descriptions of dystonic conditions have been reported with idiopathic torsion dystonia (DYT1), focal dystonias (DYT7), mixed dystonias (DYT6 and DYT13), dopa-responsive dystonia, myoclonic dysto-nia, rapid-onset dystonia parkinsonism, Fahr disease, Hallervorden-Spatz syndrome, X-linked dystonia parkinsonism, deafness-dystonia syndrome, mitochon-drial dystonias, myoclonic dystonia, neuroacanthocyto-sis, and the paroxysmal dystonias dyskinesias. In addition, focal dystonia may emerge in families exhibiting generalized dystonia and has also been related to the DYT1 allele (Table 4.1).

Otitis Externa

Acetic acid ear drops are the easiest and least expensive way to eliminate the infecting agent. A 2 solution is effective and available commercially in aqueous (Otic Domeboro) or propylene glycol (Vosol or Orlex) solutions. These drops should be used three to four times a day for at least 1 week. However, when OE is accompanied by a Tm perforation, burning or stinging will occur with the use of acid- or alcohol-containing medication thus, an antibiotic preparation containing neomycin, polymyxin B, and hydrocortisone (Cortisporin Otic suspension) is less irritating. Another option is the use of Cortisporin ophthalmic suspension, which is free of both acid and alcohol. Ophthalmic gentamicin or tobramycin are alternative drugs however, when these agents are administered systemically, they have ototoxic properties, although hearing loss due to their topical use has not been documented. 21 Another option is the use of ophthalmic quinolone drops (ciprofloxacin, ofloxacin, and norfloxacin,...

Bullous Myringitis

PATHOPHYSIOLOGY The blisters are believed to occur between the highly innervated outer epithelium and the inner fibrous layer of the TM, explaining the severe otalgia. The blisters may be blood-filled, serous, or serosanguinous. Middle ear effusions may be present, either sympathetic in origin or as a result of medial rupture of bullae. Otorrhea as a result of ruptured bullae is short-lived. A reversible hearing loss is commonly associated with the condition and may be conductive, sensorineural, or mixed. DIAGNOSIS Patients with bullous myringitis typically present with severe, throbbing otalgia, often with hearing loss. Otoscopy reveals multiple fluid-filled blisters, which may be yellow to red in color. There may be bloody otorrhea or hemotympanum.

Cytomegalovirus

CMV is one of the TORCH agents toxoplasmosis, other (viruses), rubella, CMV, and herpes (simplex viruses) and is capable of causing intrauterine infection. Fewer than 25 percent of neonates with intrauterine CMV will display symptoms. Those at highest risk are those whose mother acquires primary disease during the first half of the pregnancy. A seropositive mother's antibodies to CMV appear to provide the fetus some protection. Classic intrauterine CMV (congenital cytomegalic inclusion disease) involves multiple organs, including jaundice, hepatosplenomegaly, microcephaly, petechiae, and inner ear problems, as well as CNS defects. Children who are asymptomatic at birth may still have hearing loss that results in lower IQ scores and learning disabilities later in life.

Background

Figure 1 Mapofthe human mitochondrial genome. D-loop control region. Letters around the outside perimeter indicate cognate amino acids of the tRNA genes. Other gene symbols are defined in the text. Arrows followed by names of continents, and associated letters on the inside of the circle, indicate the position of polymorphisms that define region-specific mtDNA lineages (i.e. haplogroups). Arrows associated with abbreviations followed by numbers, around the outside of the circle, indicate representative pathogenic mutations, the number being the nucleotide position of the mutation. DEAF deafness MELAS mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes LHON Leber's hereditary optic neuropathy ADPD Alzheimer's and Parkinson's disease MERRF myoclonic epilepsy and ragged red fibre disease NARP neurogenic muscle weakness, ataxia, and retinitis pigmentosum LDYS LHON plus dystonia PC prostate cancer. (http www.mitomap.org). Figure 1 Mapofthe human mitochondrial genome....

Acute otitis media

Following an upper respiratory tract infection (URTI), as ascending infection through the Eustachian tube. The natural course of acute otitis media is best described in four stages hyperaemic, inflammatory, suppurative and resolution phases. In the hyperaemic phase, the patient has otalgia without hearing loss and otoscopy reveals a hyperaemic eardrum. The inflammatory phase that follows is characterized by increasing otalgia and hearing loss. Fever is usually present at this phase. Otoscopy reveals a hyperaemic eardrum and middle ear effusion. The disease reaches a climax at the suppurative phase. The patient often becomes irritable because of intense otalgia and hyperpyrexia is frequently present. Otoscopy reveals pus collecting behind a bulging and intensely hyperaemic eardrum. The eardrum is now under severe tension and may rupture spontaneously. Once the eardrum ruptures, the condition enters the resolution phase. All the symptoms especially otalgia resolves rapidly.

Resource Allocation

Many children do not receive basic healthcare or social services. In some cases, countries that can afford to provide those services allocate insufficient funds for them. For example, the main health problems of children in the United States arise from failure to provide such basic care for children's allergies, asthma, dental pathology, hearing loss, vision impairment, and chronic disorders (Starfield Newachecket et al.). Basic healthcare and social services promote children's well-being, enhancing their opportunities in fundamental ways and correcting some inequities caused by the natural and social lotteries. Children who are sick cannot compete as equals and thus lack equality of opportunity with other children. The more these conditions are easily correctable, as many of them are, the more unjust it is to leave children sick or disabled. Failure to provide children with basic healthcare and social services when a society has sufficient means is unjust on the basis of any of four...

Cerebral Palsy

Children with cerebral palsy may have associated medical problems as a direct effect or complication of motor dysfunction or underlying brain damage. Seizures, oral motor dysfunction, gastroesophageal reflux, constipation, urinary tract infections, pneumonia, wheezing, hearing loss, strabismus, visual impairments, scoliosis, contractures, and hip dislocation or subluxation are all seen with increased frequency in children with cerebral palsy. Children with less severe presentations have minimal associated medical problems (i.e., the child with spastic diplegia may have only issues related to spasticity). As the severity of impairment increases to spastic quadriplegia, the incidence and severity of associated problems and need for emergency medical treatment also increase.

Aminoglycosides

Animoglycosides are bactericidal agents that bind to the 30 S bacterial ribosome and inhibit protein synthesis. Agents include gentamicin, tobramycin, amikacin, kanamycin, streptomycin, spectinomycin, and netilmicin. Aminoglycosides have a low therapeutic to toxic ratio, with ototoxicity and nephrotoxicity being common adverse effects. All aminoglycosides have the potential to damage vestibular and cochlear sensory cells, but neomycin is by far the most ototoxic. The incidence of hearing loss related to aminoglycoside has been reported to be between 2 and 25 percent. Hearing loss correlates closely with high-dose or prolonged therapy. Nephrotoxicity results from damage to the proximal renal tubules and correlates with drug dose, therapy duration, volume status, and extremes of age. Mild renal insufficiency may result in 10 to 25 percent of patients using aminoglycosides, as reflected in elevations in serum creatine. Renal damage is largely reversible with cessation of therapy, but...

Otitis Media

Otitis media, or inflammation of the middle ear, is one of the most common pediatric diagnoses. Each year there are 24.5 million office visits and over 3.7 million emergency department visits, with direct and indirect costs of 5.7 billion a year.12 and3 Acute otitis media (AOM) (acute suppurative, purulent, or bacterial) is associated with signs and symptoms of inflammation of the middle ear, such as otalgia, otorrhea, fever, irritability, anorexia, or vomiting. 4 Otitis media with effusion (OME) (secretory, nonsuppurative, serous, or mucoid) is a relatively asymptomatic collection of fluid in the middle ear. The duration (not the severity) of OME can be divided into acute (< 3 weeks), subacute (3 weeks to 3 months), and chronic (> 3 months).5 The most important distinction between OME and AOM is that the signs and symptoms of acute infection (otalgia, otorrhea, and fever) are lacking in OME, but hearing loss may be present in both conditions. 5

Auditory Skills

Children with Down syndrome are at increased risk for hearing loss. It is essential to have the pediatrician and the audiologist monitor hearing on a regular basis and treat any hearing problems (Cohen et al., 1999, Roizen et al., 1994, Shott, 2000). Some kinds of tests can be used to test infants within the first week of life. Otitis media with effusion (OME), inflammation of the middle ear with fluid buildup behind the eardrum, is the most common problem related to hearing. The fluid interferes with sound transmission, and the result is a conductive hearing loss that is fluctuating. It is difficult for infants and toddlers to learn to listen and to attend to sounds when they sometimes can hear the sounds and other times cannot (Roberts and Medley, 1995).

Examination

Patients with perennial or seasonal rhinoconjunctivitis may have injected conjunctivae (visible small blood vessels), erythematous conjunctivae (reddened whites of eyes), puffy eyelids, and erythematous, oedematous nasal mucosa (the lining of the nose appearing swollen and red). Studies investigating any link between food allergy and otitis media with effusion (sometimes termed glue ear - long-standing fluid in the middle ear resulting in, albeit temporary, conductive hearing loss) have been poorly conducted.18 To date, there is no good evidence linking this condition with food allergy.

Gala

Habitual risk factors, coronary disease 16 haemodynamic instability 30, 35, 37 haemodynamics, mitral valve surgery 78 haemodynamic stability 39 Hancock porcine prosthesis 89, 90 HCM see hypertrophic cardiomyopathy hearing loss 141, 230 heart failure 51-4 antiarrhythmics 124 apoptosis in 215-16, 219 atrial fibrillation 126 cardiac transplantation 43-9 identification and management of

CNS infections

Clinically, meningitis manifests as fever, headache, neck stiffness, photophobia and a deteriorating level of consciousness. A transient petechial skin rash can occur in meningococcal meningitis. Seizures, cranial nerve signs including deafness can develop. Focal neurological deficits can occur usually from focal ischaemia or the development of an abscess.

Prenatal Infections

Prenatal cytomegalovirus (CMV) infection occurs through the first to third trimesters in symptomatic patients. CMV has an affinity for the rapidly proliferating subependymal cells lining the ventricles. Viral multiplication and subsequent calcium deposition result in brain disruption or dysgenesis with periventricular or diffuse calcification. Microcephaly (50 ), hydrocephalus, neuronal migration disorders, porencephaly, and polycystic encephalomalasia are observed. Mental retardation, visual disturbance, hearing loss, language disorders, and epilepsy can be observed.23,29,30

Figure 422

Following the initial identification of the original four POU factors, a number of other members of this family have been described both in mammals and other organisms such as Drosophila, Xenopus and zebra fish. Like the original factors, these novel POU proteins also play a critical role in the regulation of developmental gene expression. Thus, for example, the Drosophila POU protein drifter (CFla) has been shown to be of vital importance in the development of the nervous system (Anderson et al., 1995), while mutations in the gene encoding the Brn-4 factor appear to be the cause of the most common form of deafness in humans (de Kok et al., 1995). Moreover, all the novel POU domain-containing genes isolated by He et al. (1989) from the rat, on the basis of their containing a POU domain (see Chapter 2, section As with the POU proteins, Pax factors play a critical role in gene regulation during development particularly in the developing nervous system. Thus, for example, Pax6 has been...

Etiology

Today, multiple risk factors contributing to the diagnosis of CP have been identified. However, in most cases, the underlying mechanism remains unknown. Researchers have not been able to link the exact timing of risk factors to resultant lesions in the developing brain. Two etiologies that do produce homogeneous clinical patterns are maternal iron deficiency and Rh incompatibility. Mothers who suffer from an iron deficiency during pregnancy give birth to children who display spastic diplegia with deaf mutism, and infants born with Rh incompatibility experience choreoathes-tosis and deafness. However, the majority of risk factors that are identified as contributing to CP do not necessarily lead to the disorder.

Mercury

Disasters in Minamata, Japan, in the 1950s and in Iraq in 1971-1972 demonstrated that acute prenatal exposure may result in severe mental retardation, cerebral palsy, blindness, and deafness. However, whether exposure to lower chronic doses, which may occur if pregnant women consume large amounts of fish, can also lead to adverse neurodevelopmental consequences is less certain. Large, long-term prospective epidemio-logical studies of high fish-eating populations have not found a consistent pattern of association between exposure and neuropsychological outcomes. Although subtle neuropsychological changes were reported in a study of children in the Faroe Islands study, where exposure was mainly from whale consumption, a similar study in the Seychelles found no adverse effects from fish consumption alone.

Lab and Consults

Chromosomal karyotype genetic counseling hematocrit or complete blood count to investigate plethora (polycythemia) or thrombocytopenia (possible myeloproliferative disorders) thyroid function test check on results of state-mandated screening evaluation by a pediatric cardiologist including echocardiogram (even in the absence of a murmur) reinforce the need for subacute bacterial endocarditis (SBE) prophylaxis in susceptible children with cardiac disease refer for auditory brain stem response (ABR) or otoa-coustic emission (OAE) test to assess congenital sensorineural hearing loss at birth or by 3 months of age. Refer for a pediatric ophthalmological evaluation by 6 months of age for screening purposes. Refer immediately if there are any indications of nystagmus, strabismus, or poor vision. If feeding difficulties are noted, consultation with a feeding specialist (occupational therapist or lactation nurse) is advised.

Paranasal sinuses

Of paranasal sinus tumours, 80 occur in the maxillary sinus with the rest arising from the ethmoid, frontal and sphenoid sinuses. The maxillary sinuses are lined by ciliated columnar epithelium and the majority of the malignant epithelial neoplasms are squamous cell carcinoma. Oral signs and symptoms appear early while nasal obstruction and bloody nasal discharge are late symptoms. Invasion of the orbit is associated with ocular signs, including unilateral proptopsis and diplopia. Anterior extension leads to facial asymmetry and deformity. Extension of tumour posteriorly leads to destruction of the pterygoid plates and invasion of the infratemporal fossa. There may be unilateral deafness and facial palsy. Both axial and coronal CT scans are required to define the extent of the paranasal tumour since extension of tumour to adjacent sinuses and structures are common due to delay in presentation and in diagnosis.

Clinical Description

Peripheral pulmonary artery stenosis, butterfly-like vertebral arch defect, posterior embryotoxon, and a peculiar facies. 1 However, minor features such as renal abnormalities, skeletal defect, high-pitched voice, and mental and growth retardation were also described. In addition, some patients occasionally present with clinical features including other arterial stenosis, small bowel atresia, deafness, and diabetes. 2

Corticosteroids

Adjunctive corticosteroid therapy for bacterial meningitis remains controversial. Animal studies of meningitis have shown that bacterial lysis resulting from antimicrobial therapy leads to inflammation in the subarachnoid space which in turn may contribute to poor outcomes.32,33 These studies have also demonstrated that adjunctive corticosteroid therapy reduces cerebrospinal fluid inflammation and subsequent neurologic sequelae.32,33 A number of randomized controlled trials have examined the possible role of corticosteroid therapy in pediatric meningitis but have come to differing conclusions. A meta-analysis of these trials showed a beneficial effect of adjunctive dexamethasone therapy in reducing severe hearing loss in children with H. influenzae type b meningitis and further suggested a similar benefit reducing hearing loss in those with pneumococcal meningitis.34 Recently, de Gans et al. reported the results of a multicenter trial of 301 adults with bacterial meningitis randomized...

Long QT syndrome

Several mutations have also been noted in potassium channels, which reduce potassium flux through a loss of function.12'14 These mutations appear to have a dominant negative effect. Rarely, the QT syndrome is inherited in an autosomal recessive manner and may be associated with deafness, such as in the Jervell and Lang-Nielsen syndrome. This led to the recognition that the inward potassium current is necessary for endolymph production in the inner ear.15 There is extensive phenotypic variability among these various genes and mutations, and within the same family, in keeping with other genes, there are many modifiers yet to be recognized to properly interpret genotype phenotype correlations.

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